0702055522

Practical General Practice
ALGrawany
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Practical General Practice
Guidelines for Effective Clinical Management
SEVENTH EDITION
Adam Staten, MA (Cantab), MBBS, MRCP (UK),

DRCOG, DMCC, PGCertCE, MRCGP
General Practitioner
Milton Keynes, UK
Paul Staten, MBBS, MA (Cantab), DRCOG, MRCGP

Milton Keynes, UK
For additional online content visit

ExpertConsult.com
Edinburgh London New York Oxford Philadelphia St Louis Sydney 2019
ALGrawany
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Notices
Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using
any information, methods, compounds or experiments described herein. Because of rapid advances in the
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Contents
Preface, vii 16 Contraception, Sexual Problems, and Sexually

The Structure of the Book, ix Transmitted Infections, 246
Acknowledgements, xi Lindsey Pope
List of Contributors, xiii
17 Infectious Diseases and Vaccination, 278
Neil Ritchie
Section 1: Principles and Practice of 18 Psychiatric Problems, 313
Primary Care Dominique Thompson
1 Principles and Practice of Primary Care, 1 19 Urinary and Renal Problems, 340
Adam Staten Lindsey Pope
2 Long-Term Conditions, 7 20 Ear, Nose, and Throat Problems, 354

Stewart W. Mercer, Harry Hao-Xiang Wang Adam Staten
3 Communication Skills, 11 21 Eye Problems, 371

Annemieke Bikker, Lynsay Crawford Suzannah Drummond
4 Ethics, 19 22 Skin Problems, 381

Al Dowie Kieran Dinwoodie, Anchal Goyal, Catriona Nisbett,
Jane Colgan
5 Disability, 26
Lynn Legg, Jane Tracy 23 Allergic Problems, 403
Aziz Sheikh
Section 2: Manual of Clinical Practice 24 Diabetes and Endocrinology, 409

Russell Drummond, Frances McManus, Kate Hughes,
6 Children’s Health, 37 Sharon Mackin, David Carty
Ruth Margaret Bland, Hilary Lockhart Pearce
25 Persistent Physical Symptoms and Symptoms
7 Cardiovascular Problems, 65 Without Apparent Disease, 426
David Nicholas Blane Christopher Burton
8 Respiratory Problems, 93 26 Palliative Care and Care of the

Hilary Pinnock Dying Patient, 431
Ben Dietsch
9 Gastroenterologic Problems, 109
John Paul Seenan, Heather Lafferty
10 Surgical Problems, 134 Section 3: Appendices

Iain Wilson
Appendix 1 Routine Schedule of
11 Musculoskeletal Problems, 142
Immunizations, 471
John MacLean
Appendix 2 Incubation Period and Infectivity of
12 Neurological Problems, 170
Common Diseases, 472
Declan Nugent
Appendix 3 A Suggested Table of
13 Women’s Health, 191
Immunizations for Travel, 474
Lindsey Pope
Appendix 4 Notification of Infectious
14 Obstetric Problems, 209
Diseases, 475
Lindsey Pope
Appendix 5 Child Health Promotion, 476
15 Older People’s Health, 229
Ian Reeves Appendix 6 Stages of Child Development, 478
ALGrawany v
vi Contents
Appendix 7 Stages of Puberty, 481 Appendix 19 Immunizations in Pregnancy, 503

Appendix 8 Predicted Normal Peak Flow Appendix 20 Edinburgh Postnatal Depression
Values in Children (Under 15 Years Scale, 504
of Age), 483
Appendix 21 Admission Procedures for Patients
Appendix 9 Peak Expiratory Flow in Normal With Mental Health Problems, 506
Subjects, 484
Appendix 22 The Early Warning Form for Use in
Appendix 10 FEV1/FVC Charts, 485 Psychotic Illness, 508
Appendix 11A Summary of Management of Appendix 23 AUDIT, 509
Asthma in Adults, 487
Appendix 24 International Prostate Symptom
Appendix 11B Summary of Management of Score (IPSS), 511
Asthma in Children, 488
Appendix 25 Body Mass Index, 513
Appendix 11C Management of Acute Severe
Appendix 26 Reference Ranges for Young
Asthma in Adults in General
Adults, 514
Practice, 489
Appendix 27 Anaphylaxis Algorithm, 516
Appendix 11D Management of Acute Severe
Asthma in Children in General Appendix 28 Problems Associated With Specific
Practice, 491 Causes of Disability, 517
Appendix 12 Care Pathway for Respiratory Tract Appendix 29 The Community Dependency
Infections, 493 Index, 521
Appendix 13 Guidance for DMARD Appendix 30 Nottingham Extended Activities
Prescribing, 495 of Daily Living Questionnaire
(EADL), 523
Appendix 14 Dermatomes and Myotomes, 497
Appendix 31 Drug Stabilities in Syringe
Appendix 15 Testing Peripheral Nerves, 498
Drivers, 524
Appendix 16 Drug Levels, 500
Appendix 32 Guidelines for the Urgent
Appendix 17 Checklist to Guide the Review Referral of Patients With Suspected
of a Patient With Multiple Cancer, 526
Sclerosis, 501
Appendix 33 Opioid Dose Conversion Chart, 531
Appendix 18 Medical Management of
Obesity, 502 Index, 532
Preface
It is 30 years since the publication of the first edition of this years from the guidance produced by the various esteemed
textbook and since then, a time during which five further medical institutions.
editions of the book have been published, a wealth of research As ever, this book should be seen as a guide and a template
and numerous guidelines have been produced as part of the from which general practitioners can derive their own ways
worldwide crusade to practise the evidence-based medicine, of working based on a logical, structured approach. The
for which this book was in some ways a forerunner. chapters of this book are designed to mirror the mental
This is the first edition not to be edited by Alex Khot processes of the doctor during the general practice consulta-
and Andrew Polmear, whose vision and passion for production and so help that doctor to synthesise rational and safe
ing straightforward, evidence-based, and above all practical treatment plans for his or her patients.
guidelines for general practitioners working at the coal face Whilst keeping pace with the changes in guidance has
of primary care originally brought this textbook into being. made the production of this book a challenge, the wealth
We hope this edition follows those principles and remains and breadth of research and guidance currently available has
a reliable desktop companion for GPs. also enabled our contributors to produce more robust guid-
Producing a guidelines-based book like this is much like ance than has ever previously been possible.
the endless work of Sisyphus pushing his boulder up a hill In recognition of the fact that previous editions of this book
in Tartarus for all eternity, only to watch it roll back down have attracted a readership outside of the United Kingdom, this
before reaching the summit. The pace of change in medicine, edition has deliberately been designed to be less UK focused
the rate at which guidelines are produced, and at which with guidance based on guidelines and opinion from around
consensus opinion changes, makes it almost inevitable that the globe. Where possible the details of patient support groups,
a book such as this is at risk of being out of date before the relevant to specific diseases in different countries, have also
ink is dry on the page. However, guidelines tend to change been included to reflect the more global outlook of this edition.
by evolution rather than revolution and the content of this
book, produced by experts working in the real world of daily Adam Staten
practice, is likely to differ only by nuance in the coming Paul Staten
ALGrawany vii
The Structure of the Book
Bullets Where the order is important we number the list:

1. Sit the patient up.
Different coloured bullet points have been used to provide 2. Give oxygen.
emphasis for different types of comment: 3. Give diamorphine …
• Black bullets are for general information or explanation
e.g. ‘Treatment can be expected to….’ Boxes
• Pink bullets are instructions for questions that should be
asked, examinations that should be performed, or inves- These are used to highlight information that might otherwise
tigations and treatments that should be undertaken e.g. get lost in the text: guidelines, a list of tests as a ‘work-up’
‘Ask the patient x, y and z’, ‘Examine for a, b and c’. for a patient with a particular condition, or patient organisa-
• Grey bullets are used where there is a subdivision of another tions for example.
heading.
References
Lists
Our aim is to reference every statement of fact. Where such
Where we present a list in no particular order we use: a statement is not accompanied by a reference, the reader
(a) chest pain; or can assume it is taken from the reference in a box at the
(b) hypotension; or start of that section.
(c) heart failure.
ALGrawany ix
Acknowledgements
Firstly we would like to thank Alex Khot and Andrew Polmear An enormous thank you must go to the team at Elsevier,
whose vision led to the production of the first six editions in particular, Carole McMurray, Pauline Graham, and Radjan
of this text book; we hope that this current edition lives up Lourde Selvanadin for whom the last few months and years
to the high standards they have set. We would, of course, must have felt like something of a cat herding exercise.
like to thank all our contributors who have worked to ever Adam would like to thank his wife Shiva and his daughters
tightening deadlines, to produce a set of great chapters, fre- Rose and Grace who have had to tolerate a husband and
quently swimming against the tide of new guidance and father who has spent many vacant hours tapping slowly at
research to keep this edition up to date as we have moved his laptop. Paul would like to thank his partner Erica for
through production. We would particularly like to thank her help and support.
Mr Iain Wilson, Mr Robert Hone, and Dr Naema Alam
who all stepped in at the last moment with invaluable advice
and contributions to help get this book to press.
ALGrawany xi
List of Contributors
The editor(s) would like to acknowledge and offer grateful thanks for the input of all previous editions’ contributors, without
whom this new edition would not have been possible.
Annemieke Bikker, MSc Lynsay Crawford, MBChB

Teaching Fellow Clinical University Teacher
University of Edinburgh School of Medicine, University of Glasgow
Usher Institute of Population Health Sciences and Deputy Director of Vocational Studies
Informatics School of Medicine, University of Glasgow
College of Medicine and Veterinary Medicine GP Partner
Edinburgh, Scotland Balmore Surgery
Possilpark Health and Care Centre
Ruth Margaret Bland, BSc, MBChB, MD, FRCPCH Glasgow
Consultant General Paediatrics
Royal Hospital for Children and Honorary Associate Ben Dietsch, MBChB
Clinical Professor Lead Specialty Doctor
Institute of Health and Wellbeing Willen Hospice
University of Glasgow Milton Keynes
Glasgow
Kieran Dinwoodie, MBChB, MRCGP, DRCOG, DTM&H,
David Nicholas Blane, BSc, MBChB, MPH Dip Derm
Clinical Academic Fellow General Practitioner Principal
General Practice and Primary Care Calderside Medical Practice
University of Glasgow Blantyre
Glasgow
Al Dowie, PhD
Christopher Burton, MD, FRCGP General Practice and Primary Care
Professor of Primary Medical Care University of Sheffield University of Glasgow
Sheffield Glasgow
David Carty, MBChB, PhD, FRCP Russell Drummond, MBChB

Consultant Endocrinologist Honorary Clinical Associate Professor
Department of Diabetes, Endocrinology and Clinical School of Medicine, Dentistry and Nursing
Pharmacology University of Glasgow
Glasgow Royal Infirmary Glasgow
Glasgow
Suzannah Drummond, MBBS, FRCOphth
Jane Colgan, MBChB, DTH&H, MRCP Consultant in Ophthalmic Surgery
Specialty Doctor Ophthalmology
Monklands Hosptial Tennent Institute of Ophthalmology
Lanarkshire Glasgow
Airdrie, Scotland
ALGrawany xiii
xiv List of Contributors
Anchal Goyal, MBBS, nMRCGP, DRCOG, DPD, GPwSI Catriona Nisbett, MBChB, MRCGP, MRCP, DRCOG, Dip
Dermatology Dermatology
NHS Lanarkshire General Practitioner
Monklands District General Hospital, Airdrie The Murray Surgery, East Kilbride
Kate Hughes, MBChB PhD MRCP (Diabetes and Declan Nugent, MB BCh, BAO, MRCGP
Endocrinology) General Practitioner
Consultant Physician and Diabetologist and Glasgow
Endocrinologist
Honorary Senior Lecturer Hilary Pinnock, MBChB, MD, MRCGP
School of Medicine, Dentistry and Nursing Reader
University of Glasgow Allergy and Respiratory Research Group, Centre for
Glasgow Population Health Sciences
University of Edinburgh
Heather Lafferty, MBChB, MRCP Edinburgh, Scotland
Consultant Physician and Gastroenterologist General Practitioner
Queen Elizabeth University Hospital Whitstable Medical Practice
Glasgow Whitstable
Lynn A. Legg, PhD, MPH Hilary Lockhart Pearce, MBChB, MRCPCH

Research Fellow Consultant General Paediatrics
Scottish Centre for Excellence in Rehabilitation Research Royal Hospital for Children
University of Strathclyde Glasgow
Glasgow
Lindsey Pope, MBChB, MRCGP, PGCertMedEd, FHEA
Sharon Mackin, MBChB (Hons), MRCP Clinical Senior University Teacher
Specialty Registrar Diabetes and Endocrinology General Practice and Primary Care
Department of Diabetes and Endocrinology University of Glasgow
Glasgow
Ian Reeves, BSc, BM
John MacLean, MBChB, MRCGP, FRCPS (Glas), FFSEM, Department of Medicine for the Elderly
DRCOG Southern General Hospital
General Practitioner Glasgow
Maryhill Health and Care Centre and
Sport and Exercise Medicine Doctor Neil Ritchie, MBChB, PhD, MRCP(UK) (Infectious
Hampden Sports Clinic and Scottish FA and Diseases)
Honorary Clinical Associate Professor Clinical Lecturer in Infectious Diseases
University of Glasgow Institute of Infection, Immunity and Inflammation
Glasgow University of Glasgow
Glasgow
Frances McManus, MBChB, BMedSci, PhD, MRCP
(Diabetes and Endocrinology) John Paul Seenan, MBChB, MD, MRCP
Consultant Physician Consultant
Department of Diabetes Department of Gastroenterology
Endocrinology and Clinical Pharmacology Queen Elizabeth University Hospital
Glasgow
Stewart W. Mercer, MD, PhD, FRCGP, FFPHM, FRCPE

Professor of Primary Care Research
University of Glasgow
Director of the Scottish School of Primary Care
Glasgow
List of Contributors xv
Aziz Sheikh, BSc, MBBS, MSc, MD Harry Hao-Xiang Wang, PhD

Chair of Primary Care Research and Development Associate Professor
University of Edinburgh School of Public Health
Usher Institute of Population Health Sciences and Sun Yat-Sen University
Informatics PR China
College of Medicine and Veterinary Medicine Honorary Senior Lecturer
Edinburgh General Practice and Primary Care
University of Glasgow
Dominique Thompson, MBChB, MRCGP (Dist) Glasgow
GP and Director
Buzz Consulting Iain Wilson, MBBS, BSc, MRCS
Bristol Surgical Trainee
Queen Alexandra Hospital
Jane Tracy, MBBS, DRACOG, GCHE Portsmouth
Director
Centre for Developmental Disability Health Victoria
Monash University
Melbourne, Victoria, Australia
ALGrawany
S E C T I ON 1 Principles and Practice of Primary Care
1
Principles and Practice of
Primary Care
ADAM STATEN
C H A P T E R CO N T E N T S
Challenges of Primary Care Telemedicine
Population Challenges Communications Technology
The Challenge of External Factors Models of Care
The Evolving Primary Care Team Telephone Triage
Nurse Practitioners Shared Medical Appointments
Physiotherapists The General Practitioner Consultant
Clinical Pharmacists The Virtual Ward
Physician Assistants/Associates Caring for the Doctor
Mental Health Professionals The Burnout Syndrome
Medical Assistants Finding Help and Treatment
Use of Technology
Electronic Medical Records
OBJECTIVES
• Primary care can be defined as any care that is delivered in holistic approach to patient care, and an approach to
the community as opposed to the inpatient setting. In patient care that is proactive rather than reactive.
wealthier countries primary care is usually considered to be • The WHO (2003) recognises the core principles of primary
the first level of care provision, whereas in poorer countries care to be:
it may be seen as a systemwide strategy to providing access 1. universal access to care and coverage based on need;
to healthcare (World Health Organization [WHO], 2003). 2. commitment to health equity as part of development
• It is widely recognised that building health services around oriented to social justice;
high-quality primary care results in better public health, 3. community participation in defining and implementing
fewer inequalities in healthcare by socioeconomic class, and health agenda;
lower rates of unnecessary hospital admissions (Kringos, 4. intersectoral approaches to health.
Boerma, van der Zee, & Groenewegen, 2013). • These principles are underpinned by the declaration of
• The structure of primary care varies widely from country to Alma-Ata, made in 1978 (WHO 1978) in which primary care
country but there are key similarities to treating patients in was defined as “essential health care based on practical,
the community that are true in all countries, and all scientifically sound and socially acceptable methods and
healthcare systems, including the interaction between technology made universally accessible.”
healthcare, social care, and third sector organisations, a
ALGrawany 1
2 se c t i o n 1 Principles and Practice of Primary Care
add to the pressures of working within primary care and

Challenges of Primary Care medicine in general.
Population Challenges
The Evolving Primary Care Team
• The provision of holistic care is at the heart of primary
care and providing this care is increasingly challenging • Whilst general practitioners (GPs) are usually considered
with a global population that is increasing in size, age, to be central to the provision of primary care services,
and multimorbidity. the primary care team includes all those professionals
• The increasing capability to diagnose and treat disease leads who contribute to the health and well-being of patients
to increasing patient demand and increasing resource cost in the community.
both in terms of time and finance. • With the increasing complexity of healthcare provision,
• Particularly in developed nations, the rise of illnesses and the increasing complexity of the patients who receive
related to lifestyle factors such as smoking, alcohol con- treatment in the community, any attempt for GPs to
sumption, and obesity create a burden to the healthcare practice in isolation without recourse to the wider
system and are a complicating factor to many other ill- primary healthcare team is likely to result in frustration
nesses. Globally, infectious diseases such human immu- for the GP and poor-quality, possibly dangerous care for
nodeficiency virus/acquired immunodeficiency syndrome patients.
(HIV/AIDS) contribute to the increasing burden on • The roles and responsibilities of the primary care team are
primary healthcare (and the wider healthcare system). to some extent limitless. It is characteristic of primary care
• An increasing emphasis in maintaining wellness rather that practitioners working in the community are expected
than simply treating ill health has put primary care at to deal to a greater or lesser extent with every problem that
the forefront of screening programmes, education pro- a patient may present. Often these problems are not simply
grammes, and primary preventative treatment. medical and they may be complicated by, or indeed may
• The increasing capabilities of modern medicine, the primarily be, psychological or social problems.
emphasis on keeping people well, and wider public • Many tasks in primary care are as well, and often better,
access to healthcare information (via the internet, for performed by members of the primary care team other
example) all contribute to rising patient expectations and than GPs.
managing these expectations in a resource limited envi- • The structure of primary care teams varies from country
ronment can prove very challenging. In the United to country—for example, in the United Kingdom den-
Kingdom the General Medical Council (GMC) found tists usually work separately from GPs, but in other
that this rise in expectations was a key contributing European countries it is common for doctors and den-
factor to the 100% increase in complaints made against tists to be colocated. Similarly, professionals such as
doctors between 2007 and 2012 (GMC, 2014). Rising social workers and mental health nurses are located
patient expectations is also frequently cited as a reason alongside GPs in many countries.
for doctors leaving their role in primary care (Leese, • As coordinating patient care becomes ever more complex
Young, & Sibbald, 2002). it is vital that the extended primary care team works
coherently to avoid patient neglect or duplication of
The Challenge of External Factors effort to deliver effective, rational care to patients.
• Workforce problems in primary care in many countries
• Healthcare is expensive and funding for primary care is have led to the innovation of new roles for established
not always adequate to meet the needs of the population healthcare professionals within primary care and the cre-
it serves. For example, in many developing countries ation of entirely new types of healthcare professionals.
funding is diverted away from the provision of compre- • The primary care team in any community should be
hensive primary care in favour of providing vertical care tailored to suit the healthcare needs of the local popula-
programmes targeting specific issues such as HIV/AIDS tion and it is therefore essential for anyone involved with
or childhood immunisations (Maeseneer et al., 2008). workforce planning to be familiar with the variety of
• The provision of healthcare can become highly politicised professionals that can contribute to providing primary
and interference in healthcare from politicians for politi- healthcare.
cal purposes, rather than to improve patient care, can be a • To deal with the demands of modern healthcare, doctors
source of real frustration and dissatisfaction for doctors. should see themselves as having a key role in driving
• Doctors now practice in the full glare of the media (and healthcare policy toward establishing the most effective
social media) spotlight. Not only can this be intimidat- primary care teams for their particular populations.
ing and exposing, but doctors working in general prac-
tice are often left to undo the damage done by inaccurate Nurse Practitioners
messages promulgated by the media.
• A worldwide tendency to increasing litigation and, in • Nurse practitioners, or advanced nurse practitioners, are
some circumstances, the criminalisation of medical error trained beyond the usual competences of registered
CHAPTER 1 Principles and Practice of Primary Care 3
nurses so that they are able to practice autonomously and • As polypharmacy in an ageing population becomes
assess and diagnose undifferentiated problems, to synthe- more common, expertise in medicine management will
sise treatment plans (Royal College of Nursing, 2012). be increasingly important and an increasing workload
Key to this is their ability to prescribe independently. burden for general practitioners.
• Nurse practitioners work in many different areas of • The role is perhaps best established in the United States
healthcare but within primary care they provide care where clinical pharmacists have been working and evolv-
both for acute illness (usually by providing consultations ing their role over a period of decades. In 1997 the
for minor illness) and chronic disease (such as perform- WHO published policy statements that envisaged an
ing routine reviews in respiratory illness or diabetes). expanded future role for pharmacists that would benefit
• They are well established in Anglophone countries where patients in healthcare systems globally. Since then the
they are seen as a key resource in helping to manage role has become increasingly recognised in the Anglo-
patient demand, but they are less well recognised in other sphere and across Europe. Clinical pharmacists are also
parts of the world. invaluable in bolstering the primary care teams in coun-
• Training to become an advanced nurse practitioner varies tries where doctor numbers are low.
from country to country and depends on the area of
healthcare in which the nurse is working, but in the Physician Assistants/Associates
United Kingdom the Royal College of Nursing provides
accredited training courses to upskill nurses and prepare • To train as a physician assistant (also known as a physi-
them for an advanced role. cian associate) the trainee must already have a degree in
• Evidence suggests that nurse practitioners provide good a life or healthcare science subject. Physician assistants
levels of patient satisfaction and good patient outcomes, then undergo an intense period of training in the medical
but the evidence of cost effectiveness remains equivocal model to enable them to interview, examine, and diag-
(Martin-Misener et al., 2015). nose patients; order and interpret tests; and perform
procedures according to competency. They may work in
Physiotherapists a variety of settings from surgery to emergency medicine,
but many work in primary care.
• Up to 30% of primary care consultations relate to • The physician assistant is a dependent medical practitio-
musculoskeletal problems, many of which are best dealt ner who works under the supervision of a physician. The
with by physiotherapists. However, direct access to phys- ability to prescribe is variable depending on the country
iotherapists for patients is not necessarily the norm (or US state) in which the individual works.
within primary care. • The physician assistant is a US invention; the role was
• Direct access is usually available to patients in Australia, established there over 50 years ago. Currently there are
absent in the United States, and patchy throughout the around 100,000 physician assistants practicing. They
European Union. This variability in access is despite the have been shown to be cost effective and acceptable to
fact that the majority of countries, particularly within patients, and in recent years several countries have shown
Europe, have the requisite legislation and train their interest in developing training programmes to produce
physiotherapists to have the requisite competencies to physician assistants to alleviate pressure on primary care
practice independently. Often the barriers to enabling doctors (Legler, Cawley, & Fenn, 2007).
direct access come from within the medical profession
itself, despite the potential reduction in workload that Mental Health Professionals
physiotherapists can provide (Chartered Society of Phys-
iotherapists, 2013). Where direct access is not available • Mental health problems are an enormous part of primary
patients must usually come via their primary care physi- care, either as the presenting problem or as a complicat-
cian to get access to physiotherapy. ing factor for other problems. Up to one third of all
• The provision of direct access physiotherapy has been general practice appointments are thought to involve a
shown to be both clinically and cost effective (Mallet mental health component.
et al., 2014). • Given this workload and the economic burden of mental
health in primary care, the WHO has produced policy
Clinical Pharmacists emphasising the importance of providing good-quality
primary mental healthcare. However, it remains unusual
• Clinical pharmacists have an extended role that involves for mental health nurses, or other mental health profes-
direct patient-facing activity with particular respect to sionals who are capable of delivering psychologic treat-
medicine management. Their key roles are in optimising ments, to be embedded within the primary care team.
medication and dosage regimes, de-conflicting medica- • Since 2014 in the Netherlands there has been a deliberate
tions that may interact, and ensuring the cost effective- shift in the provision of mental healthcare from second-
ness of medications. Many are also involved in the ary to primary care. This has been largely facilitated by
management of minor ailments and chronic disease. increasing the number of mental health nurses working
ALGrawany
alongside GPs such that between 2010 and 2014 the Telemedicine
proportion of practices in the Netherlands with a mental
health nurse increased from 20% to over 80%. This has • Telemedicine (or telehealth) relates to the remote moni-
not reduced GP workload but has increased the number toring of patients and the transfer of biometric data
of long appointments available in the community to from the patients’ home to their doctor. It has perhaps
patients with mental health problems (Magnée et al., been most utilised when dealing with cardiovascular or
2016). respiratory disease to enable early detection of decom-
• A Cochrane review of the effectiveness of counselling pensation of the monitored illness and proactive, early
provided within primary care found that it was clinically management.
more effective in the short term than usual care (although • As technology advances and equipment such as blood
not in the long term) and associated with similar costs pressure monitors and oxygen saturation probes become
to usual care (Bower et al., 2011). cheaper it is likely that this will be seen as a convenient
and cost-effective means of managing patients. It has the
Medical Assistants added advantage of engaging patients with their own
care and empowering them to take responsibility for
• Medical assistants primarily work within primary care managing their illness.
teams in the United States. They are allied health profes- • The cost effectiveness of telemedicine remains uncertain
sionals who work in both administrative and clinical (Henderson, 2013), but it is likely that increasing
roles. Their duties may include scheduling appointments, amounts of remote monitoring will become part and
handling correspondence, updating patient notes, as well parcel of future general practice; and as its use becomes
as performing clinical procedures such as ECGs and more common its cost effectiveness will improve.
blood draws, assisting the physician during procedures,
or preparing patients for examination. Communications Technology
• It is suggested that they are a key means by which doctors
can relieve themselves of their administrative workload • We have more ways to communicate with one another
and so enable themselves to focus more on direct patient than ever before—via telephone, email, text message, or
care (Sinsky et al., 2013). video phone. These technologies present the possibility
of interacting with our patients and our colleagues more
Use of Technology efficiently and more flexibly.
• Younger patients in particular are comfortable with com-
The use of technology within medicine has the potential to municating electronically. For example, the use of virtual
improve patient care and make the working life of primary clinics that employ email and text messaging to com-
healthcare professionals easier and less stressful. As tech- municate with young diabetic patients has dramatically
nologies develop it is important that those working within improved attendance rates (Mayor, 2016).
primary care stay alert to new ways in which this technology • Video phone applications (such as Skype) have been
can be applied to their own working environments. used in a variety of settings: providing remote care
for refugees, orthopaedic follow-up, and psychiatric
Electronic Medical Records consultation. GPs in the United Kingdom have
(Davies et al., 2016) experimented with using SkypeTM to review patients
in nursing homes.
• The use of electronic medical records (EMRs) is common • There are numerous email or phone-based systems that
but not ubiquitous. In New Zealand, Scandinavia, and can be employed to enable GPs to access specialist advice
the United Kingdom the use of medical records is almost rapidly, which may obviate the need for an acute admis-
universal, but this is not the case in other developed sion or a referral for specialist advice.
nations; for example, in Canada rates are below 80% and
in Switzerland they are below 60%. Models of Care
• Even where EMRs are used the capabilities of different
systems vary enormously with the most advanced allow- • As the burden of caring for enlarging and ageing popula-
ing the review of results, correspondence, production of tions increases, the way in which patients are seen in
patient summaries, transfer of electronic prescriptions to primary care will need to be adapted to increase capacity
pharmacies, and prompts and alerts for patient review. within the system.
This allows for more seamless care, reduction in duplica- • GPs need to adapt the ways in which they see their
tion of work, and the setting up of efficient and reliable patients to suit their particular patient populations.
patient recall systems for patient review and monitoring. Some of these varied models of seeing patients will
• Higher levels of doctor satisfaction with their EMRs be reliant on the technologies discussed earlier in the
have been shown to correlate with overall higher job chapter; others require a fresh approach to the traditional
satisfaction. medical consultation.
CHAPTER 1 Principles and Practice of Primary Care 5
Telephone Triage proactive, and multidisciplinary input. It is an elabora-

tion on the concept of the multidisciplinary team and
• Telephone triage is a means by which patient demand and may or may not make use of telehealth data.
flow can be managed. It has become popular, particularly • Versions of the virtual ward that have been trialled usually
in the United Kingdom, as a way to reduce the number of involve a team consisting of community nurses, GPs, ger-
patients that need to be seen face to face and involves iatricians, and possibly representatives from social ser-
patients speaking to a health professional (usually a doctor vices. This team meets at regular intervals to discuss a case
or a nurse) by phone to assess the need for a face-to-face load of complex patients.
review before the patient is offered an appointment. • By meeting regularly and having input from a
• Some who advocate the system estimate that up to 60% number of disciplines this approach aims to improve
of primary care problems can be resolved over the phone proactive care and so reduce the risk of an acute
and there is evidence suggesting that patients find this decompensation in illness requiring hospital admis-
means of interacting with their GP satisfactory. sion. It should also reduce duplication of effort by
• However, the ESTEEM trial was a large-scale trial of improving communication between all those involved
telephone triage which found that, although clinician in the patient’s care.
contact time on the day of the appointment request was
reduced, overall clinician contact time was no different
to usual care, which to some extent undermines its Caring for the Doctor
purpose (Holt et al., 2016). The Burnout Syndrome
Shared Medical Appointments • The world of general practice is without doubt stressful
and continues to become more so as a result of the chal-
• Shared medical appointments are part medical consulta- lenges detailed already in this chapter. A 2015 Com-
tion, part education session. Groups of patients with monwealth Fund survey of primary care in 10 developed
the same condition are seen together for an extended nations found that significant proportions of doctors
appointment and educated about their condition and in all 10 countries found their work in general practice
how it can be managed. This saves overall clinician time either very stressful or extremely stressful (Davies et al.,
whilst increasing the contact time the patient has with 2016).
the clinician. Other benefits include empowering patients • The phenomenon of physician burnout is well recog-
to self-manage and the creation of a peer support network nised but often not well handled. The three key features
for patients. of burnout are usually described as:
• They have been used in a range of settings including 1. emotional exhaustion;
diabetes, maternity, physiotherapy, and liver disease. 2. depersonalisation;
Patients report higher levels of satisfaction with shared 3. an absent sense of personal accomplishment.
medical appointment care than with usual care (Hey- • The burnout syndrome overlaps with, and is compli-
worth et al., 2014). cated by, anxiety and depression and shares key features
with those issues such as social withdrawal, absentee-
The General Practitioner Consultant ism from work, and problems with drug and alcohol
abuse.
• This is a model of care that relies on the GP having a • Doctors are at high risk of burnout as they are selected
team of varied allied health professionals at hand. based on personality traits such as perfectionism, high
• This model of care relies on central triage which directs achievement, a sense of responsibility, and competitive-
patients toward the relevant professional (e.g., physio- ness, which all put them at higher risk of burning out.
therapist, mental health nurse, physician associate). The • Work within medicine exposes people to extended
GP is not directly involved in the initial patient contact periods of extreme emotional stress (both their own and
but is called in to consult on cases that are beyond the that of other people), which contributes to burnout.
capability of the allied health professional. • A perceived stigma to mental illness amongst doctors
• Theoretically this frees up the GP to dedicate time to also means that doctors tend to seek help late by which
those most complex patients who require the most skilled point the damage may well be significant, including
input albeit at the expense of the regular and recurrent suicidality.
patient contacts that many would argue provide job sat-
isfaction in primary care. Finding Help and Treatment
The Virtual Ward • It is important that those working within general prac-
tice recognise the signs of stress and burnout both in
• The virtual ward is a concept designed to manage themselves and in their colleagues and feel able to seek
patients, often housebound patients, who require intense, help or suggest that their colleagues seek help.
ALGrawany
• Treatment for the burnout syndrome, or for depression or randomised controlled trial. British Medical Journal (Clinical Research
substance misuse problems in general, is along standard Ed.), 346. doi:https://doi.org/10.1136/bmj.f1035.
lines and includes cognitive behavioural therapy (CBT), Heyworth, L., et al. (2014). Influence of shared medical appoint-
medication, and counselling. These can be sought via the ments on patient satisfaction: A retrospective 3-year study. Annals
of Family Medicine, 12, 324–330.
doctors’ own GP although many are reluctant to seek help
Holt, T., et al. (2016). Telephone triage systems in UK general
in this way for themselves. Alternatively, many countries practice: Analysis of consultation duration during the index day
have mental health programmes specifically for medical in a pragmatic randomised controlled trial. The British Journal of
professionals that can operate on an anonymous basis. General Practice, 66, e214–e218.
• Self-help techniques such as mindfulness also have a Kringos, D. S., Boerma, W., van der Zee, J., & Groenewegen, P.
good evidence base amongst doctors working in primary (2013). Europe’s strong primary care systems are linked to better
care and many simple mindfulness techniques can be population health but also to higher health spending. Health
learnt via online apps. Affairs, 32, 686–694. doi:10.1377/hlthaff.2012.1242.
• GPs also have the opportunity to tackle the source of Leese, B., Young, R., & Sibbald, B. (2002). GP principals leaving
their distress either by changing the way in which they practice in the UK. The European Journal of General Practice, 8,
work or by changing the type of work that they do 62–68.
Legler, C. F., Cawley, J. F., & Fenn, W. H. (2007). Physician assis-
within the varied world of primary care.
tants: Education, practice and global interest. Medical Teacher, 29,
e22–e25.
Further Reading Maeseneer, J., van Weel, C., Egilman, D., et al. (2008). Funding for
primary health care in developing countries. BMJ (Clinical
Staten, A., & Lawson, E. (2017). GP wellbeing: Combatting burnout Research Ed.), 336, 518–519.
in general practice. London: CRC Press. Magnée, T., de Beurs, D. P., de Bakker, D. H., et al. (2016). Consulta-
tions in general practices with and without mental health nurses: An
observational study from 2010 to 2014. BMJ Open, 6, e011579.
References Mallett, R., et al. (2014). Is physiotherapy self-referral with telephone
triage viable, cost-effective and beneficial to musculoskeletal
Bower, P., Knowles, S., Coventry, P. A., et al. (2011). Counselling for outpatients in a primary care setting? Musculoskeletal Care, 12,
mental health and psychosocial problems in primary care. Cochrane 251–260.
Database of Systematic Reviews, (9), CD001025. Martin-Misener, R., et al. (2015). Cost effectiveness of nurse practi-
Chartered Society of Physiotherapists. (2013). Direct access and tioners. British Medical Journal Open, 5, e007167.
patient/client self-referral to physiotherapy: A review of contem- Mayor, S. (2016). Use texts, apps, and Skype to keep young people
porary practice within the European Union. Physiotherapy, 99, with diabetes engaged with services, says guidance. British Medical
285–291. Journal (Clinical Research Ed.), 352, i394.
Davies, E., et al. (2016). Under pressure: What the Commonwealth Royal College of Nursing. (2012). Advanced Nurse Practitioners: An
Fund’s 2015 international survey of general practitioners means for RCN Guide to advanced nursing practice, advanced nurse practition-
the UK. Retrieved from http://www.health.org.uk/publication/ ers and programme accreditation.
under-pressure#sthash.3qqLghqH.dpuf. Sinsky, C., et al. (2013). In search of joy in practice: A report of
General Medical Council. (2014). What’s behind the rise in complaints 23 high-functioning primary care practices. Annals of Family
about doctors from members of the public. Retrieved from https:// Medicine, 11, 272–278.
gmcuk.wordpress.com/2014/07/21/whats-behind-the-rise-in World Health Organisation. (1978). Declaration of International Con-
-complaints-about-doctors-from-members-of-the-public/. ference on Primary Health Care, Alma-Ata, USSR, 6–12 September.
Henderson, C. (2013). Cost effectiveness of telehealth for patients with World Health Organisation. (2003). The World Health Report: Shaping
long term conditions (Whole Systems Demonstrator telehealth ques- the future. Geneva: WHO Publishing.
tionnaire study): Nested economic evaluation in a pragmatic, cluster
2
Long-Term Conditions
STEWART W. MERCER, HARRY HAO-XIANG WANG
Prevalence of Long-Term Conditions Polypharmacy
Comorbidity and Multimorbidity Clinical Guidelines
Prevalence of MM Evidence-Based General Practice
Global Burden Management
Deprivation Effects Organisational
Effects of MM Inverse Care Law
Healthcare Utilisation What Do Patients With LTCs Need From General Practice?
Mental and Physical
OBJECTIVES
• A long-term condition (LTC) is commonly defined as chronic conditions seen in general practice include multiple
a condition that requires ongoing medical care, sclerosis, Parkinson disease, and muscular dystrophy.
limits what one can do, and is likely to last for a year • Conditions once considered terminal are now commonly
or more. seen, and regarded as long-term conditions, due to
• Common long-term conditions include diseases such as improved survival rates from treatments, and this includes
coronary heart disease, diabetes, asthma, and stroke. many cancers and infectious diseases such as human
Patients with such conditions are commonly seen and immunodeficiency virus (HIV) and acquired
managed in primary care in the long term. Less common immunodeficiency syndrome (AIDS).
Prevalence of Long-Term Conditions index condition (a condition of primary concern) is

• Prevalence rates of individual long-term conditions vary termed comorbidity. For example, a patient with diabetes
considerably between different countries and populations, and asthma, being cared for by a diabetologist, may be
though in most countries, including developing countries, considered by the specialist physician as a diabetic with
long-term conditions are increasing rapidly in the popula- comorbidity. It is a term mainly used by specialists reflect-
tion. This is true in all age groups, although certain condi- ing their own area of expertise.
tions affect certain age groups more than others. • In general practice, patients commonly have two or more
• It should be borne in mind that all prevalence estimates of long-term conditions without one being clearly an index
long-term conditions are based on data collection methods condition, and indeed the extent to which different con-
that have some flaws. Thus prevalence estimates will vary ditions affect patients often varies over time. Thus in
according to how the condition is defined and measured. primary care the term multimorbidity (MM) is preferred
to comorbidity.
Comorbidity and Multimorbidity
Prevalence of MM
• International studies have demonstrated that many people
living with chronic disorders have multiple chronic health • Multimorbidity is common and has been rising in preva-
problems simultaneously. The co-occurrence of one or lence over recent years. For example, a Canadian study of 21
more additional long-term conditions to a person with an family practices in Quebec reported a multimorbidity
7
prevalence of 69% in 18- to 44-year-olds, 93% in 45- to Healthcare Utilisation

64-year-olds, and 98% in those over age 65, with the
number of chronic conditions varying from 2.8 in the • Patients with LTCs and multimorbidity may have higher
youngest to 6.4 in the oldest (Fortin et al., 2005). In the overall vulnerability to diseases and less resistance to acute
United Kingdom, a large, nationally representative study in health threats (e.g., higher susceptibility to influenza).
Scotland found that over 40% of the whole population (all These interacting influences lead to a complex pattern in
ages included) had at least one long-term condition, and the demand and utilisation of health services.
almost 25% of the entire population had multimorbidity • Multimorbidity leads to an increased likelihood of referrals
(Barnett et al., 2012). between different providers of healthcare (often in a vertical
• The prevalence of multimorbidity increases substantially manner—i.e., general practitioner [GP] to several special-
with age and is present in most people aged 65 years or ists, but also between specialists, especially in centres of
older. However, the Scottish study also found that the excellence). Excessive use of specialist care leads to a rapid
absolute number of people with multimorbidity was rise in healthcare expenditure. Multimorbidity has become
higher in those younger than 65 years than those over one of the most salient influences on cost of healthcare due
65 years, thus long-term conditions and multimorbidity to the heavy burden on the healthcare utilisation.
should not be considered simply a problem of old age.
Mental and Physical
Global Burden
• LTCs span both mental and physical conditions, and com-
• Over recent decades, life expectancy has improved dra- monly patients have both. This relationship is bidirectional
matically and currently exceeds the age of 75 on average, in that patients with mental health problems commonly go
in nearly 60 countries. This is due to improved living on to develop physical health problems, and patients with
circumstances, greater access to universal education, and a wide range of LTCs are more likely to go on to develop
rapid advances in clinical medicine and public health. mental health problems than the general population.
• The ageing of the global population is regarded as the
most crucial driver of increases in the burden of chronic Polypharmacy
diseases. It is particularly evident in wealthier countries
where many people are living much longer now than • A common problem in patients with LTCs is polyphar-
ever, though not necessarily healthier in their extra years. macy, which is usually defined as being on five or more
It is estimated that by 2020, the incidence of long-term regular medications. In patients with multimorbidity, poly-
conditions will increase by approximately 30% to 40% pharmacy is even more common. This has serious implica-
as the population ages. tions for iatrogenesis. Indeed, a common reason for hospital
admission, especially in the elderly, is medication side effects
Deprivation Effects and interactions. Not only is this harmful to patients, but
it also infers a huge financial drain on healthcare systems.
• Health is seldom distributed evenly across populations • A second problem with polypharmacy is adherence to med-
and in most (if not all) countries of the world, the ication regimens. Research has shown that once patients get
poorest health is found in those living in situations of to five or more medications per day their adherence begins
poverty. This is also true of multimorbidity, which to decline. That’s not to say that patients stop taking all their
tends to be worse in those of the lowest socioeconomic tablets, but they do tend to be creative in developing their
status. The study in Scotland (discussed earlier) revealed own regimens, especially skipping tablets that have effects
an astonishingly precise relationship between multimor- that they don’t like such as loop diuretics.
bidity and deprivation. Multimorbidity in those living • Patients often have strong perceptions of which tablets may
in the most deprived areas also develops some 10 to 15 be giving them side effects (even if this is unlikely to be the
years younger than in the least deprived decile of the case), which can be influenced by a whole host of things
population. such as pill size, colour, and taste. It has been suggested that
• Many (though not all) studies have found that multi- polypills (combination pills with several ingredients—e.g.,
morbidity is more common in women than in men. for cardiovascular disease) may enhance adherence by
reducing the number of tablets required each day, though
Effects of MM at present there is little evidence to support this.
• Many LTCs are associated with increased mortality and/ Clinical Guidelines
or morbidity, and this is exacerbated by increasing levels
of multimorbidity. There is a clear linear relationship • A likely major driver of polypharmacy is guidelines. The
between levels of multimorbidity and death rate. development of clinical guidelines based on evidence
• Multimorbidity also increases hospital admission rates, collated from randomised controlled trials has been one
even for potentially avoidable admissions and has a of the major advances in the delivery of evidence-based
major negative impact on quality of life. medicine over the last 20 years. However, guidelines are
ALGrawany
CHAPTER 2 Long-Term Conditions 9
invariably single-disease focused. They give good advice the GPs, who remain the key clinicians in dealing with
on when to start medications in single LTCs, though patients with complex LTCs.
seldom give advice on when to stop them. This, com-
bined with the fact that most patients with LTCs have Organisational
multimorbidity, means that most patients rapidly accu-
mulate new prescriptions and thus polypharmacy. • The effective management of patients with LTCs in
primary care depends on a well-organised and strong
Evidence-Based General Practice primary care system. The work of the late Barbara Starfield,
a preeminent primary care clinical researcher, has shown
• The rise of evidence-based medicine (EBM) has been one that countries with strong primary care systems deliver
of the greatest achievements in medical research and higher quality of care and are more cost effective.
the implementation of EBM into general practice has • Thus a well-funded and well-developed primary care
resulted in huge improvements in the management of system is a basic requisite for the cost-effective manage-
LTCs. Statin prescribing for hypercholesterolaemia, anti- ment of the vast numbers of patients with LTCs.
hypertensive prescribing for hypertension, achievement • Many models exist with regard to how the management of
of glycaemic control in diabetes through insulin, or drug patients with LTCs is best organised, but one of the best
interventions are a few examples of EBM. The gold stan- known and most widely used is the chronic care model devel-
dard research method that underpins EBM is the ran- oped by Wagner and colleagues in the United States. The
domised controlled trial (RCT). chronic care model defines a range of important factors,
• It is such evidence of benefit from RCTs that under- which they suggest need to be addressed to promote effective
pins clinical guidelines. However, there is a danger of management of chronic disease. These factors include:
overextrapolating findings from RCTs on specific pop- a. clinical information systems (e.g., disease registers so
ulations (e.g., men <65 years) to a much wider popu- that patients with known LTCs can be identified and
lation (e.g., both sexes, elderly). It is also important to recalled);
realise that most RCTs on LTCs are on patients with b. delivery system design (e.g., annual review at a planned
single conditions, and most trials actively exclude visit, with members of the primary care team working
patients with comorbidity or multimorbidity from in a coordinated and complementary way);
taking part in such trials. A recent Cochrane review c. decision support (e.g., evidence-based practice guide-
of interventions specifically for patients with multi- lines, access to specialist advice);
morbidity found only a handful of RCTs published d. self-management support (e.g., by giving informa-
worldwide. tion, coaching, and motivation to support patients to
• Care must thus be taken not to blindly apply guidelines manage their own conditions better).
to all multimorbid patients without consideration of the • These four factors are thought to work together to improve
individual patient’s needs, circumstances, and priorities. patient’s functional and clinical outcomes as a result of pre-
Clinical judgment and shared decision making based on pared and proactive primary care staff having productive
informed choice are vital tools in the management of interactions with informed and activated patients.
patients with LTCs, alongside clinical guidelines. • Although the chronic care model is intuitively appealing
and gives a comprehensive overview of how best care may
Management be achieved, it should be noted that evidence for its effec-
tiveness outside the United States is limited and achieving
• The management of patients with LTCs is a large and certain aspects of it can be very difficult in practice.
important part of the work of general practice and • The model envisages self-management support as being
primary care. In the United Kingdom, about 80% of not just a function of the healthcare system but also a col-
patients who consult their GP or practice nurse have an laborative function of communities. Although this makes
LTC, and as we have seen, most of these patients are good sense in theory, as primary care is imbedded within
likely to have more than one LTC. communities, and communities often have a range of assets
• The majority of care for most patients with LTCs can that could help people with LTCs (e.g., charitable organisa-
safely be undertaken in primary care, especially once any tions, faith groups, lunch clubs, exercise facilities), effective
acute phase has been dealt with by secondary care (e.g., linkages between primary healthcare providers and local
myocardial infarction in patients with congenital heart community resources are hard to achieve in practice.
defect [CHD], initiation of insulin in newly diagnosed
type 1 diabetic) and the diagnosis is confirmed. Inverse Care Law
• In the United Kingdom practice nurses are increasingly
involved in the routine care of patients with LTCs (e.g., • An important issue compounding the effective manage-
in conducting annual reviews of patients with hyperten- ment of patients with LTCs in primary care is the con-
sion, asthma). These nurses require suitable training and tinuing existence of the inverse care law in many (if not
supervision and it is important to emphasise that they all) countries around the world. The term was first intro-
are not working autonomously but in partnership with duced in the 1970s by Julian Tudor Hart, a GP working
in a socioeconomically deprived population in Wales. The community based and community facing. Nowhere else in
inverse care law states that the availability of good medicine is whole-person care so possible on a population level
medical care tends to vary inversely with the need for it in and so needed. We have the opportunity to get to know our
the population served. In general terms, this means that patients and their families over time, and thus provide much
the poorest patients have the worst healthcare. needed continuity of care. Our care is comprehensive and coor-
• This is of course abundantly clear within healthcare dinated; delivered with compassion and caring. Empathy is
systems that are largely privately run. However, it is sadly important in all therapeutic relationships and empathic care is
also true of systems that have a national health service, supported by values of altruism. Empathy is especially valued by
such as the United Kingdom. The reason for this is that patients with multimorbidity. Empathy leads to higher patient
within the United Kingdom, primary care services (and and practitioner satisfaction, and better outcomes; research has
the number of GPs in an area) are not distributed accord- shown that patients never feel enabled in consultations without
ing to health need but according to population size. GP empathy. Empathy has also been linked to better adherence
• Although health need rises two- to threefold from the most with treatment regimens, reduction in symptom severity, and
affluent to the most deprived patients, the distribution of improved well-being. The effects of empathy can be direct and
GPs is flat across deprivation deciles. In practice, this means immediate or indirect and longer term.
that patients who live in poorer areas have worse access to (c) Generalism. GPs need to be expert generalists, which
high-quality care (longer waiting times to see a GP, shorter not only requires excellent technical clinical skills and
consultation length) with primary care practitioners in knowledge and effective communication skills, but also to
deprived areas being more stressed due to the greater need, be skilled in interpretive medicine, integrating multiple
demand, and clinical complexity of the patients. sources of knowledge (including biomedical, biographical,
• The inverse care law is of course not a law as such; it is a and professional) in a dynamic exploration and interpreta-
situation brought about by policy decisions through gov- tion of the individual illness experience. Practicing interpre-
ernments as the funders of healthcare. Such policies could tive medicine leads to decisions about what is wrong, and
be changed, and doctors themselves can advocate for such what is needed to intervene, which supports an outcome of
change. In Scotland, for example, the GPs working in the health as a resource for living, with the patient as an active
100 most deprived areas of the country formed an infor- partner in coproducing health.
mal group, GPs at the Deep End, which has been active
in vocalising the problems they and their patients face. Conclusions
What Do Patients With LTCs Need • As the population ages, the dramatic rise in the preva-
From General Practice? lence of LTCs is the major challenge facing the world
and most of its countries. It is also the major challenge
(a) Practice Organisation. Practices need to be well organ- facing general practice and primary care.
ised in caring for patients with LTCs, with effective means of • Multimorbidity is the norm not the exception, yet guide-
identifying patients (i.e., electronic disease registers) and lines and EBM are largely derived from research which
arranging proactive anticipatory care rather than simply react- excludes patients with multimorbidity.
ing to problems that patients present with in an unplanned • In managing patients with single or multiple LTCs,
way. The best way to organise such anticipatory care will general practice and primary care teams need to be well
depend on the practice resources and the patient population. organised to care for such patients and to be proactive
For example, in more affluent areas, patients with LTCs are and anticipatory rather than simply reactive. This requires
more likely to be proactive in their self-management by a strong primary care system with adequate resources.
attending booked reviews, etc. Patients in deprived areas more • Patients with multiple complex needs, must be at the
commonly have additional social and psychologic problems centre of care, not the round pegs in the square holes of
and thus may fail to attend booked appointments for reviews single disease–focused approaches. Holism lies at the
and so more anticipatory care may need to be done within the heart of good management, and empathic, patient-
reactive consultation. This of course has implications for con- centred care is a key requirement of the two facets of
sultation length, but some practices in deprived areas are able high-quality primary care for patients with LTCs—
to give longer consultations (e.g., 15–20 minutes) when it is generalism and interpretive medicine.
needed, by having spare time slots within booked sessions
which can be moved so that a 10-minute slot can be changed
immediately into a 20-minute slot. References
(b) Empathic, Person-Centred Care. The encounter Barnett, B., Mercer, S. W., Norbury, M., Watt, G., Wyke, S., &
between doctor and patient should never be reduced to a dry Guthrie, B. (2012). The epidemiology of multimorbidity—
tick box exercise where the GP blandly follows protocol. authors reply. Lancet, 380, 1383–1384.
General practice defines itself as a discipline that provides holis- Fortin, M., Bravo, G., Hudon, C., Vanasse, A., & Lapointe, L.
tic, generalist medicine. Holism means taking a biopsychosocial (2005). Prevalence of multimorbidity among adults seen in family
(and at times spiritual) approach to care. General practice is practice. Annals of Family Medicine, 3, 223–228.
3
Communication Skills
ANNEMIEKE BIKKER, LYNSAY CRAWFORD
Professional Requirement The Structure of the Consultation
Communication Skills The Consultation
Verbal Communication The Inner Consultation
Nonverbal Communication The Calgary Cambridge Method
Paralinguistics The CARE Approach
Rapport Overviews of Consultation Models
Ideas, Concerns, Effect, and Expectations How to React to a Complaint
Sharing Information Telephone Consultations
Shared Decision Making Communication Skills for Telephone Consultations
Informed Consent Confidentiality
Confidentiality Guidance on Remote Prescribing
Working With Interpreters Email Consultations

Confidentiality
Adherence and Compliance
Physical Arrangement of the Room
OBJECTIVES
• Good communication skills are central to general practice; or hidden agendas not revealed); poor concordance with
without them you cannot be an effective clinician. However advice or medication and therefore poorer outcomes; less
extensive your clinical knowledge, the inability to patient satisfaction; less patient enablement; and increased
communicate that information, or interact and engage with incidence of complaints.
patients, renders that knowledge ineffective. The • Good communication is crucial not just with patients but
consequences of ineffective or poor communication are with all other colleagues (nurses, receptions staff, relatives,
unsatisfactory consultations (patients’ concerns not elicited pharmacist, secondary care, etc.).
Professional Requirement e. listen to, and respond to, their concerns and preferences;
f. give patients the information they want or need in a
• The professional requirements related to communication way they can understand;
skills are outlined in the General Medical Council’s g. respect patients’ right to reach decisions with you
(GMC, 2013) Good Medical Practice as: about their treatment and care;
a. treat patients as individuals and respect their dignity; h. support patients in caring for themselves to improve
b. treat patients politely and considerately; and maintain their health;
c. respect patients’ rights to confidentiality; i. work with colleagues in the ways that best serve
d. work in partnership with patients; patients’ interests.
11
• The Royal College of General Practitioners (RCGP, with depression while a more rapid, loud voice suggests
2016) curriculum statement states that general practi- anger or excitement).
tioners (GPs) should display:
a. an understanding of the wider context in which the Rapport
consultation takes place;
b. an understanding of the structure of the consultation; • One of the most common mistakes in communication
c. commitment to an ethical and reflective attitude. is to talk too much and listen too little. In listening to
patients it is important to really listen and to let patients
know that you are listening to them. This establishes
Communication Skills trust and rapport.
Verbal Communication • Rapport can be established and maintained at four levels:
• During consultations it is important to use a mixture of Level 1: Nonverbal level—by matching body language;
question types: posture, gestures, facial expressions, and eye contact
a. Open questions Level 2: Paralinguistics/voice level—by matching breath-
b. Closed questions ing rate, tone, pitch, tempo
c. Focused questions Level 3: Language level—by matching or using another’s
d. Indirect questions words
• Question types to avoid: Level 4: Values level—by connecting with shared beliefs
a. Leading questions and values
b. Compound/double questions
• As well as questions, language is used for other pur- • Sensitivity to patient’s cues (Silverman et al., 2013):
poses: 1. Be alert to patient’s verbal cues (prompts, throw-away
a. Social exchanges (“Good morning” or comments on comments) and nonverbal cues (body language,
the weather) facial expression, vocal cues) to elicit emotional
b. Facilitations (“Go on” or “uh uh”) content of the illness, ideas, effect on daily living, and
c. Repetition/restatement (repeating back what has just expectations.
been said) 2. Clarify the emotions that the patient is hinting at
d. Confrontation (confronting with an observation: (e.g., by repeating or checking out a verbal cue: “You
“You look worried/sad/angry”) said that the cough worries you, especially at night.
e. Clarification/interpretation (clarifying what the What theories do you have about what it might be?”
patient has said: “So the tiredness started after your or a nonverbal cue: “Am I right in thinking that you
sleep pattern was disturbed?”) are puzzled by the information that I gave you?”).
f. Judgmental statements (responses that state the value 3. Explicitly acknowledge cues as appropriate.
judgment of the doctor: “Anyone who smokes ciga- • Demonstrating empathy to help develop rapport involves
rettes is foolish”) (Derksen et al., 2013):
g. Reassurance, explanation, instruction, or advice 1. understanding (or reconstruct and imagine) the
patient’s situation, perspective, and feelings;
Nonverbal Communication 2. verbally communicating that understanding to check
its accuracy (e.g., “I can see that you are very worried
• This includes: about the test results”);
a. dress and appearance; 3. acting on that understanding in a helpful therapeutic
b. facial expression; way.
c. gaze and eye contact; • Empathy does not require you to have experienced the
d. gestures; same problem as the patient or to like the patient.
e. posture; Research shows that patients consider empathy to be a
f. proximity—comfort zones; key component of quality of care and that empathy is
g. body contact and touch; linked to patient enablement (Derksen et al., 2013;
h. mirroring; Mercer et al., 2012).
i. pacing.
Ideas, Concerns, Effect, and Expectations
Paralinguistics
• This includes obtaining an understanding of how the patient
• This is the term given to those aspects of vocalization, sees the situation and experiences the illness, as well as
such as the speed, loudness, and pitch of the voice. These exploring the disease. Enquire about (Stewart et al., 2014):
may convey information about emotions, attitudes, or a. feelings that reflect the emotional content of the
personality (e.g., a soft, slow hesitant voice is associated illness (e.g., “Do you have any concerns about…?”);
CHAPTER 3 Communication Skills 13
b. ideas about what the patient thinks is the cause (e.g., TABLE
“What do you think is causing it?”); 3.1 Sharing Information
c. effect on the patient’s daily life (e.g., how illness limits
Type of Type of
daily activities and impairs their capacity to fulfil
Explanation Questions Purpose
certain responsibilities);
d. expectation of the consultation (e.g., “What were you Interpretive What To interpret or clarify
expecting from seeing me today?”). What is diabetes?
• By taking other factors into account (age, culture, the Descriptive How To describe a
physical environment, or people affected by the illness), concept or
the patient’s experience of the illness is put into the context process
of the person’s life (e.g., “Who is at home with you?”). How do my
kidneys work?
• This can be summarized as the ICEE framework, in
which the doctor explores: Giving Why To give reason
a. patient’s ideas (I) about what is wrong; reasons based on
principle, motives,
b. patient’s feelings/concerns (C) about the illness; or values
c. impact/effect (E) of the patient’s problems; Why did this
d. patient’s expectations (E) about what should be done. happen to me?
Sharing Information
• The GMC states that you must “give patients the infor-
mation they want or need to know in a way they can b. helping the patient to balance the risks and benefits
understand.” and make sure that their choice is based on fact rather
• Tailor the explanation to the patient by taking into than misconception.
account the patient’s needs and beliefs (e.g., “You men-
tioned depression and tiredness. I think tiredness is more Informed Consent
likely because…”).
• Observe the patient’s reactions to check if the explana- • The GMC (2013) states that it is the responsibility of
tion needs refinement. the person providing treatment or undertaking an inves-
• Find out what and how much the patient wants to tigation to obtain consent: Expressions of consent (from
know to match the amount and type of information GMC General Medical Council [available at www.gmc
to the patient’s needs and preferences (e.g., the diag- -uk.org/gmp]).
nosis, coping techniques or support available, the • According to the GMC (2013), you must give patients
causes of the illness, or side effects of treatment). the information they want or need about:
• Check what the patient already knows (e.g., “I don’t a. the diagnosis and prognosis;
know how much you know about high blood pres- b. any uncertainties, including options for further
sure, can you tell me?”). investigations;
• Avoid jargon or check the patient’s understanding of c. options for treating and managing the condition,
the technical term used. including the option not to treat;
• Back up verbal information with written information, d. the purpose of proposed treatments and what these
if appropriate, and ensure this is in the relevant will involve;
language. e. the potentials risks, burdens, and likelihood of success
• Check the patient’s understanding. of each option;
Depending on the patient, Thistlethwaite and Morris f. whether a proposed investigation or treatment is
(2006) suggest that the explanation is based on three experimental (part of research or innovative);
domains (Table 3.1). g. who is responsible for the treatment, the roles of those
involved, the involvement of students;
Shared Decision Making h. their right to refuse to take part in research or
teaching;
• “Shared decision making is an approach where clinicians i. their right to seek a second opinion;
and patients make decisions together using the best avail- j. any bills they will have to pay;
able evidence” (Elwyn et al., 2010). k. any conflicts of interest that you may have;
• The shared decision-making process includes (Thistle- l. information on any treatments with potential greater
waite & Morris, 2006): benefit than the ones offered by you or your organiza-
a. giving information to the patient on treatment tion.
options, possible risks, and benefits in a way that the • Before accepting patients’ consent, you must consider
patient understands; whether they have been given the information they want
ALGrawany
or need, and how well they understand the details and Adherence and Compliance
implications of what is proposed. This is more important
than how their consent is expressed or recorded. • Tate (2010) states several reasons why patients follow or
• Patients can give consent orally or in writing, or they do not follow the treatment:
may imply consent by complying with the proposed a. Some patients adhere because they are told by the
examination or treatment (e.g., by rolling up their sleeve doctor to do so.
to have their blood pressure taken). b. If the patient understands and believes the explana-
• In the case of minor or routine investigations or treat- tion given by the doctor then the patient is more
ments, if you are satisfied that the patient understands likely to adhere to treatment.
what you propose to do and why, it is usually enough to c. If the patient’s own understanding matches that of
have oral or implied consent. the doctor and the agenda is shared, then the patient
• In cases that involve higher risk, it is important that you is most likely to adhere.
get the patient’s written consent. This is so that everyone d. Shared decision making and linking the management
involved understands what was explained and agreed. plan with the patient’s beliefs are key to ensuring
• By law you must get written consent for certain treat- adherence and compliance.
ments, such as fertility treatment. You must follow the
laws and codes of practice that govern these situations. Physical Arrangement of the Room
Confidentiality • The physical arrangement of the room can facilitate or

hinder communication (Lloyd & Bor, 2004).
• The GMC, in Confidentiality (2009), states the princi- • Arrangement of seats
ples of confidentiality and respect for patients’ privacy. • Turning away and facing a computer can indicate
This includes: disinterest, so the patient may not give information
a. making sure that any personal information about critical to the consultation.
patients that you hold or control is effectively pro- • Arrangements such as sitting sideways or facing each
tected at all times against improper disclosure; other without a desk in the middle, and being at the
b. instances when personal information can be dis- same eye level, will facilitate communication.
closed, including if it is required by law, patients • Usually the patient’s chair is stable with four legs and
consent to this disclosure (either implicitly for the the doctor’s chair is often a swivel seat on wheels,
sake of their own care or expressly for other purposes), which helps to complete the various tasks and to face
or if it justified in the public interest. the patient and the computer at different times.
• Use of computer. Communication guides (Silverman
Working With Interpreters et al., 2013) suggest:
a. waiting until patients have finished their opening
• The GMC states you should make sure that arrange- statement before looking at the computer;
ments are made, wherever possible, to meet patients’ b. turning your attention back to patients if they start
language and communication needs. to speak whilst you are looking at the computer;
• Ask that the interpretation be in the first person without c. explaining to patients what you are doing so they
omissions, editing, polishing, or outside conversations. understand the process.
• Ask the interpreter to clarify (in his or her own words) any
misunderstandings that occur due to cultural differences. The Structure of the Consultation
• Position yourself so that you face and speak directly to
the patient rather than the interpreter. The ability to select from different consultation styles and skills
• Talk with the patient in the first person (using “I”). to navigate through the consultation facilitates the need to
• Maintain direct eye contact with the patient. meet patients with different expectations and preferences.
• Do not direct your questions or inquiries to the inter-
preter. The Consultation
• Ask the patient to repeat any instructions and explana-
tions given to ensure that they are understood. • Pendleton et al. (1984) defined seven tasks performed by
• Issues to be aware of (Lloyd & Bor, 2004): doctors, which form the aims for each consultation. It is
• Meanings can be altered in the translation process. not suggested that all tasks should be completed in every
• The patient can be embarrassed by the presence of an consultation, though they argue that continued omission of
interpreter due to the sensitive nature of the problem, one or more tasks will negatively impact on consultation
especially when the interpreter is of the same nation- outcome. Tasks 1 to 5 identify what the doctor needs to
ality. achieve. Tasks 6 and 7 relate to the entire consultation and
• The patient’s ideas can be reinterpreted by interpreter highlight the use of time and resources, and the develop-
or translated in a shortened version. ment of an effective doctor–patient relationship.
• The seven tasks: e. Housekeeping and taking care of yourself through stress
a. To define the reason for the patient’s attendance, prevention. Various options are given on what to do
including: about job stress during and outside the consultation.
1. the nature and history of the problem(s);
2. the aetiology (or cause) of the problem (i.e., the The Calgary Cambridge Method
interaction of the physical, psychologic, and social
factors); • The Calgary Cambridge Method (Kurtz et al., 2003)
3. the patient’s ideas, concerns, and expectations; integrates the tasks of the consultation and skills for
4. the effects of the problems (on daily living). communication.
b. To consider other problems that are present but not • Silverman et al. (2013) analysed 71 communication
presented by the patient, that is: skills, grouped under six headings. The skills needed
1. continuing problems (e.g., previous problems dis- depend on the context and the outcomes that the doctor
cussed at earlier consultations or social conditions and patient want to achieve.
relevant to the current problem); • These skills include:
2. modifiable risk factors (health promotion). a. initiating the session
c. To choose with the patient an appropriate action for • Establishing initial rapport
each problem. • Identifying reason(s) for attendance
d. To achieve a shared understanding of the problems b. gathering information
with the patient (including giving explanations that • Exploration of patient’s problems
relate to patient’s ideas about the problem). • Additional skills for understanding the patient’s
e. To involve the patient in the management and encour- perspective
age him or her to accept appropriate responsibility. The c. providing structure to the consultation
level of involvement that is appropriate will vary from • Making organization overt
patient to patient and from problem to problem. • Attending to flow
f. To use time and resources appropriately: d. building the relationship
1. in the consultation; • Using appropriate nonverbal behaviour
2. in the long term. • Developing the rapport
g. To establish or maintain a relationship with the patient • Involving the patient
that helps to achieve the other tasks (e.g., the doctor– e. explaining and planning
patient relationship encourages the sharing of decisions). • Providing the right amount and type of information
• Aiding accurate recall and understanding
The Inner Consultation • Achieving a shared understanding: incorporating
the patient’s perspective
• Neighbour (1987) uses five checkpoints (subgoals) in the • Planning: shared decision making
consultation alongside an awareness of minimal cues f. closing the session
(verbal and nonverbal) to help discover the unspoken • Forward planning
agenda. He emphasizes the importance of the start of the • Ensuring appropriate point of closure
consultation in which the patient conveys more informa-
tion then often is realized. Ideally, a checkpoint is reached The CARE Approach
before moving to the next.
• The five-stage model: • The CARE Approach (Bikker et al., 2014) aims to foster
a. Connecting—with the patient and developing rapport the achievement of empathic, patient-centred commu-
b. Summarizing—your understanding of the problem nication in health care encounters and is based on the
(events and emotional content) Consultation and Relational Empathy (CARE) measure,
c. Handing over—the management plan that is under- a patient-rated experience measure. It is a broad set of
stood, accepted, and agreed with the patient. Strate- guiding principles to be applied flexibly depending on
gies for handing over include: the situation and circumstance.
1. negotiating; • It consists of four interacting components that form an
2. influencing; integrated cyclical process:
3. gift wrapping. a. Connecting: Actively engaging with the patient to
d. Safety netting—and planning for the unexpected to create or deepen rapport and to facilitate open com-
manage uncertainty (e.g., using a specific time frame munication in a safe environment
such as, “Come back in 2 weeks if it doesn’t get better”). b. Assessing: Listening and taking a holistic approach
Neighbour points to three safety netting questions: to fully understand the patient’s situation, perspec-
1. If I am right, what do I expect to happen? tive, and feelings (and their attached meanings)
2. How will I know if I am wrong? c. Responding: Communicating your understanding
3. What would I do then? (and checking its accuracy) in a caring and
compassionate way, responding positively with clear Communication Skills for

explanations if appropriate Telephone Consultations
d. Empowering: Helping patients to feel more in control
according to their abilities, preferences, and values and • Car and Sheikh (2004) suggests using the same skills as
planning their treatment in partnership with them in face-to-face consultations, and being systematic in
covering the following:
Overviews of Consultation Models 1. Active listening (including verbal facilitation [e.g.,
“mm, I see…”, “tell me a bit more…”]) and increased
http://www.bradfordvts.co.uk/online-resources questioning/detailed history taking (e.g., “What does
http://www.skillscascade.com/models.htm the rash look like?”) to compensate for lack of visual
http://www.gp-training.net/training/communication_ cues
skills/consultation/index.htm 2. Frequent clarifying and paraphrasing (to ensure that
the messages have been sent and received in both
How to React to a Complaint directions)
3. Picking up verbal (red flag words/warning signs) and
• The General Medical Council (2013) states, “You must nonverbal cues (such as pace, pauses, change in voice
respond promptly, fully and honestly to complaints and intonation [e.g., “I can hear from your voice that you
apologize when appropriate.” are not sure about…”])
• MDDUS recommends to: 4. Offering opportunities to ask questions
a. ask colleagues for support; 5. Offering patient education
b. make sure someone else in the practice deals with the 6. Documentation
complaint if you are subject of the complaint;
c. keep good contemporaneous notes (this is absolutely Confidentiality
critical!);
d. be open to accepting that something may have gone • Telephone consulting is considered to have some addi-
wrong; tional risk with respect to confidentiality with the risk of
e. share learning from complaints with the whole a conversation being overheard as the area of most
practice; concern (McKinstry et al., 2009).
f. let the complainant know what the practice plans to • To minimize and manage breaches in confidentially in
do to put things right. telephone consultations and conversations McKinstry
et al. (2009) suggests the following:
Telephone Consultations • Reception areas should be organized so that telephone
calls are taken out of earshot of other patients.
• There is a limited but growing body of research with • Care should be taken not to identify patients in a way
regard to telephone consultations and guidance on how other patients can hear (e.g., rather than repeating
best to use this form of consultation (Bunn et al., 2007; names ask for date of birth).
Cochrane, 2009). The review by Car and Sheikh (2003) • Doctors should avoid taking calls in reception or
shows that patient satisfaction with telephone consulta- other staff areas, particularly in small communities.
tions is high. • Phone etiquette
• McKinstry et al. (2010) found that in comparison to • Check the provided number with the number on the
face-to-face consultations, telephone consultations tend patient’s record. If it does not match, additional care
to be shorter (4.6 versus 9.7 minutes), deal with fewer might be needed.
problems, and typically contain less information gather- • Always check when phoning patients that they are in
ing, counselling/advice, and rapport building. They also an environment where they can speak comfortably
found that telephone consultations were less likely to and confidentially.
include sufficient information to exclude important • Unless you recognize a patient’s voice always confirm
serious illnesses. They suggest that telephone consulta- identity by date of birth, or better still, last consulta-
tions may be more suited to structured follow-up and tion reason.
management of long-term conditions than for in-hours • Avoid getting involved with third party consulta-
acute management. tions; always ask to speak directly to the patient if
• In an exploratory study, McKinstry et al. (2011) found possible.
that the content of the consultations was equally well • If asking patients to provide details of a problem, recep-
remembered by patients, irrespective of whether these tionists should explain why they are asking (e.g., “so the
were conducted by telephone or face-to-face methods. In doctor can prioritize”) and explain that patients do not
both cases, recall was poorer in patients presenting multi- have to give any information if they do not wish to.
ple problems and with brain injury. • Practices should have caller identification switched off.
• If messages are left at all on an answering machine caregivers and healthcare professionals between patients/caregivers
they should be confined to confirmation that the and healthcare professionals. Cochrane Database of Systematic
clinician called and a request to call back. Review, (11), CD007978.
• If in doubt, ask to see the patient. Atherton, H., Brandt, H., Ziebland, S., Bikker, A., Campbell, J.,
Gibson, A., et al. (2018). Alternatives to the face-to-face consulta-
tion in general practice; focused ethnographic case study. British
Guidance on Remote Prescribing Journal of General Practice, 8(669).
Bikker, A. P., Cotton, P., & Mercer, S. W. (2014). Embracing Empathy
• The GMC (2013), in Good Practice in Prescribing and in Healthcare. A universal approach to person-centred, empathic
Managing Medicines and Devices, states that to prescribe healthcare encounters. London: Radcliffe.
“you must satisfy yourself that you can make an adequate Bunn, F., Byrne, G., & Kendall, S. (2007). The effects of telephone
assessment, establish a dialogue and obtain the patient’s consultation and triage on healthcare use and patient satisfaction: a
consent” and that you “may prescribe only when you systematic review. British Journal of General Practice, 57(542),
have adequate knowledge of the patient’s health, and are 714–722.
satisfied that the medicines serve the patient’s needs.” Car, J., & Sheikh, A. (2003). Telephone consultations. British Medical
• Nonsurgical cosmetic medicinal products cannot be pre- Journal, 326, 7396.
Car, J., & Sheikh, A. (2004). Email consultations in health care:
scribed remotely.
1—scope and effectiveness. British Medical Journal, 329, 435.
Derksen, F., Bensing, J., & Lagro-Janssen, A. (2013). Effectiveness of
Email Consultations empathy in general practice: a systematic review. British Journal
of General Practice, 606, e76–e84.
• There is inconclusive evidence on effects of email for clin- Elwyn, G., Laitner, S., Coulter, A., Walker, E., Watson, P., Thomson,
ical communication between patients’ health care pro- R., et al. (2010). Implementing shared decision making in the
fessionals on quality of care. There is no evidence-based NHS. BMJ, 341, c5146.
guidance on how email might best be used in clinical General Medical Council. (2009) Confidentiality: good practice in
practice (Atherton et al., 2012; Atherton et al., 2018). handling patient information. https://www.gmc-uk.org/ethical-
• There is little consensus about the rules of patient–provider guidance/ethical-guidance-for-doctors/confidentiality. (Accessed 8
online interactions and the important role that can be August 2018).
General Medical Council. (2013). Good medical practice. London,
played by staff in responding to certain types of messages.
England: GMC. Retrieved from www.gmc-uk.org/gmp.
• Based on a systematic review Car and Seikh (2004) con- Kurtz, S. M., Silverman, J. D., Benson, J., & Draper, J. (2003). Mar-
cluded that successful communication through emails rying Content and Process in Clinical Method Teaching: Enhanc-
depends on a shared understanding by doctor and patient ing the Calgary-Cambridge Guides. Academic Medicine, 78(8),
on its role, advantages, and limitations. They suggest 802–809.
introducing a standard protocol to inform patients on Lloyd, M., & Bor, R. (2004). Communication skills for medicine (2nd
how emails are dealt with in the practice. ed.). London: Harcourt.
McKinstry, B., Watson, P., Pinnock, H., Heaney, D., & Sheikh, A.
(2009). Telephone consulting in primary care: a triangulated quali-
Confidentiality tative study of patients and providers. British Journal of General
Topps (2006) suggests the following to increase patient Practice, 59(563), 433–440.
McKinstry, B., Hammersley, V., Burton, C., et al. (2010). The
confidentiality:
quality, safety and content of telephone and face-to-face con-
• Avoid specifics, especially in touchy subject areas. sultations: a comparative study. BMJ Quality and Safety, 19,
• Stick to logistic information, such as appointment 298–303.
availability. Mckinstry, B., Watson, P., Elton, R. A., Pinnock, H., Kidd, G.,
• Warn patients that anything they write or say may go Meyer, B., et al. (2011). Comparison of the accuracy of patients’
astray so they should be careful. recall of the content of telephone and face-to-face consultations:
• Warn users of email systems that employers/owners have an exploratory study. Postgraduate Medical Journal, 87(1028),
right of access. 394–399.
• Warn system users that they cannot assume confidentiality Mercer, S. W., Jani, B. D., Maxwell, M., Wong, S. Y. S., & Watt, G.
just because they are communicating with a doctor’s office. C. M. (2012). Patient enablement requires physician empathy: a
• Avoid confirming information given by the patient—it cross-sectional study of general practice consultations in areas of
high and low socioeconomic deprivation in Scotland. BMC Family
may be speculative by another party.
Practice, 13, 6.
• Never write or say anything that you would not be happy Neighbour, R. (1987). The inner consultation. Int: Kluwer Academic
seeing printed on a newspaper front page. Publishers.
Pendleton, D., Schofield, T., Tate, P., & Havelock, P. (1984). The
References consultation: an approach to learning and teaching. Oxford: Oxford
University Press.
Atherton, H., Sawmynaden, P., Sheikh, A., Majeed, A., & Car, J. Royal College of General Practitioners. (2016). The RCGP curriculum:
(2012). Email for clinical communication between patients/ Professional & clinical modules.
Silverman, J. D., Kurtz, S. M., & Draper, J. (2013). Skills for Com- Tate, P. (2010). The Doctor’s Communication Handbook. Oxon: Rad-
municating with Patients (3rd ed.). CRC Press. cliffe Publishing.
Stewart, M., Brown, J. B., Weston, W. W., McWhinney, I. R., Thistletwaite, M. (2006). The Patient-doctor Consultation in Primary
McWilliam, C. L., & Freeman, T. R. (2014). Patient-centered Care: Theory and Practice. Royal College of General Practitioners.
medicine: transforming the clinical method (3rd ed.). Oxon: Rad-
cliffe Medical Press.
4
Ethics
AL DOWIE
General Practice Ethics The Four Principles
Different Meanings of Ethics The Four Topics
Ethics, Law, and Professionalism Summary: Professional Ethics in General Practice
Ethical Reasoning
Benefits, Burdens, and Risks
OBJECTIVES
• The primary objective of this chapter is to offer general n. decisions against treatment to sustain life;
practitioners a practical aid to clinical ethical thinking o. death certification.
without feeling they need to be armed with a master’s • Clearly, then, general practice ethics has its own
degree in moral philosophy or medical law. While much of characteristics due to those features of clinical practice that
the ethics of patient care is centred on the three Cs of are particular to it as a specialty (Papanikitas and Spicer,
capacity, consent, and confidentiality, specific ethical 2018; Rogers and Braunack-Mayer, 2009).
territory that general practitioners commonly have to • In medical ethics generally, domains that tend to recur include
negotiate include the following: the following (Stirrat, Johnston, Gillon, & Boyd, 2010):
a. fit notes; a. foundations of medical ethics and law;
b. medical reports; b. professionalism: good medical practice;
c. fitness to drive; c. patients: their values, narratives, rights, and
d. confidentiality and disclosure; responsibilities;
e. young patients and consent; d. informed decision making and valid consent/refusal;
f. parental rights and responsibilities; e. capacity and incapacity;
g. child protection; f. confidentiality;
h. termination of pregnancy; g. justice and public health;
i. compulsory hospital admission and treatment; h. children and young people;
j. adult patients lacking capacity; i. mental health;
k. power of attorney; j. beginning of life;
l. advance decisions/advance directives; k. toward the end of life;
m. resuscitation decisions; l. medical research and audit.
General Practice Ethics f. Public health obligations

g. Rationing decisions in the management of individual
• Distinctive features of general practice ethics include: patients
a. Maintaining trust for longer-term clinical relationships h. Considerations of social responsibility and health
b. Accentuated importance of confidentiality in the inequality
context of local communities
c. Professional relationships with multiple patients from Different Meanings of Ethics
the same household
d. Complexity in care of patients with multimorbidity • The moment a patient enters the consulting room, the
e. Highly interprofessional context doctor is already in an ethical context. In that sense ethics
19
TABLE
4.1 Different Meanings of Ethics
Shorthand for Interpretation

Philosophical ethics Abstract, critical discussion of ethical concepts (metaethics)
Ethical theory Normative discussion of obligation in generalized terms
Ethical analysis Deliberation on ethical theory in specific circumstances (also known as applied or practical
Bioethics ethics)
Biomedical ethics Ethical analysis and/or governance and protocols with respect to biological and life sciences
Medical ethics Ethical analysis and/or governance and protocols with respect to scientific medicine
Health care ethics Ethical analysis and/or governance and protocols with respect to medical practice
Research ethics Ethical analysis and/or governance and protocols with respect to practice in the health professions
Public health ethics Governance and protocols with respect to research practices and research ethics committees
Population-based responsibilities, resource allocation, and policy in relation to health inequalities
Adapted from Dowie, A., & Martin, A. (2011). Ethic and law in the medical curriculum. AMEE Education Guide No. 53. Dundee: Association for Medical Educa-
tion in Europe.
TABLE
4.2 Normative Domains in Medicine
Ethical Criteria for self-directed practice that is not derived from the regulator or the state (though the three sets of
criteria may or may not coincide), whereby obligation is guided by principles, values, and responsibilities in
connection with relevant, ordinate interests.
Legal Criteria for state-sanctioned practice, whereby obligation is within a formal system of entitlements and duties
as specified in the law, which is subject to criteria of ethical and sociocultural standards.
Professional Criteria for validated practice, whereby obligation is according to the academic and clinical standards set by
accrediting and regulatory authorities (in the United Kingdom, the General Medical Council) as both a prior
condition and a continuing requirement for licensed practice through periodic appraisal, and in fulfilment of
the supplementary criteria for membership of learned societies and Royal Colleges.
tion in Europe.
in general practice is very much concerned with every- Ethics Law

day, ordinary, and routine aspects of patient care, even if
the presenting complaint is far from simple one.
• An issue is a controversy, and a dilemma is a difficult choice Normative
between alternatives. Ethical issues and dilemmas are not domains
the substance of most clinical practice, which is not usually
controversial and does not often entail awkward ethical
alternatives from which to choose. Sometimes the difficulty
lies in not yet possessing the relevant knowledge base or a
structured approach to ethical reasoning such as those illus-
trated later in the chapter.
Professionalism
• Because the single word ethics is used as a convenient short-
hand for widely differing domains, ambiguity surrounding • Fig. 4.1 Interplay of ethics, law, and professionalism.
the term frequently leads to misunderstanding wherever it
arises. Table 4.1 illustrates a range of various meanings.
strands. Table 4.2 teases out these strands as (1) self-
Ethics, Law, and Professionalism directed practice, (2) state-sanctioned practice, and (3)
validated practice.
• This chapter uses ethics as shorthand for yet another • Ethical, legal, and professional norms arise from separate
category in addition to those listed in Table 4.1, discourses with separate histories that nevertheless bear
namely the practice of professional ethics. This is a upon each other dynamically, as opposed to being simply
threefold nexus involving ethics, law, and professional- static zones with overlapping areas of intersection (Fig. 4.1).
ism. These normative domains in clinical practice, • Ethical norms answer to the requirements of law and to the
while distinct, combine with each other as intertwined standards set by the General Medical Council and other
CHAPTER 4 Ethics 21
• EXAMPLE 4.1 Referral Request for Termination doctor not to assist in the arrangements for a TOP, while
of Pregnancy also requiring Dr. Pal to direct Miss Thomas without
delay to another practitioner who can facilitate this if it
Miss Caitlin Thomas, a 20-year-old student who has recently is her intention to proceed.
registered at the practice, attends Dr. Pal’s morning surgery to
seek referral for an early medical termination of pregnancy
• Ethical. Example 4.2 is one of the exceptions to the
(TOP). As a matter of personal conscience, Dr. Pal is unable to general obligation of confidentiality for medical profes-
participate in TOP referrals. sionals not to disclose the personal information of
patients to other parties. As well as necessary disclosure
to other members of the health care team involved in the
clinical management of patients, any disclosure can be
• EXAMPLE 4.2 Patient Access to Medical made for which the patient gives explicit consent. This
Records can include, for example, medical reports for third parties
Mrs. Kezia Rosen is a retired manager who was discharged
such as employers or, as in this case, disclosure to another
from hospital last week following hip replacement surgery and is person whom the patient has identified. However, there
recovering at home. Her son has come to the practice with a is still the obligation to exercise care in doing so, for
signed letter from his mother requesting access to her medical example by making appropriate checks and safeguarding
records, which she wishes him to collect on her behalf. against unnecessary disclosure.
• Legal. The question of Mrs. Rosen gaining access to her
medical records is a legal one. In the United Kingdom,
professional bodies. It is possible for practitioners to take the Data Protection Act 2018 provides for the right of
an ethical stance personally that differs from what is sanc- access by persons (called data subjects) on whom records
tioned by the other two sources of norms, for example in are kept, whether paper or electronic, which includes
matters of conscience. When this is in conflict with the medical records. It also provides for access by others such
other domains, it is an external standard that prevails, since as Mrs. Rosen’s son with her explicit consent. The Act
the individual practitioner is ultimately accountable to the requires appropriate measures to preserve the security
law and to the profession. It can happen, though, that and integrity of records, which entails providing copies
external norms may shift toward personal ethical positions rather than originals. Prompt response is required, and
in line with contemporary society. compliance following receipt of the written subject access
• Legal norms in medicine are informed by professional request, as in Mrs. Rosen’s case, must be within 30 days.
codes and ethical reasoning. Standards that are set out in This must be provided free of charge, with the exception
legislation need to be interpreted to particular cases of requests that are excessive.
heard in the courts, so that the application of statutes • Professional. It is essential to maintain Mrs. Rosen’s trust
becomes clarified over time to take account of potential in responding to her request, which includes attention
injustices. Acts of Parliament are also subject to processes both to the duty of confidentiality and to the proper dis-
of updating and streamlining, as seen for example in closure that is sanctioned by law. Communication skills
progressive improvements to equality law. are central to this, for example in explaining the 30-day
• Ethical. In Example 4.1, Dr Pal’s ethical commitments compliance period, and the provision of copies rather
inform her clinical practice because they are central to than the original record. It is also appropriate to clarify
her sensibility as a person and formation as a profes- the details of Mrs. Rosen’s request, to confirm if it is for a
sional. This is important for every doctor, whatever part or the whole of her medical records (medical notes,
stance is taken, not only on this but on any area of ethical papers, correspondence, imaging, printouts of electronic
significance. It may be that the practitioner has thought files), and whether there might be a specific concern that
through a reasoned position on the subject, or equally it can be addressed directly.
could be a tacit view in terms of conscience.
• Legal. Miss Thomas is entitled to seek access to abortion ser- Ethical Reasoning
vices in the United Kingdom under the terms of the Abor-
tion Act 1967, as amended by the Human Fertilisation and • “What were your reasons for acting as you did, doctor?”
Embryology Act 1990, subject to the statutory conditions. This is a typical question that arises in practical examina-
• Professional. Professional norms are under-girded by the tions for college membership, or in hearings before a fitness
legal minimum and the imperatives of ethical responsi- to practice panel, or in litigation before a court when
bility, and as a safeguard may also go beyond what is counsel is leading evidence or cross-examining. The impli-
strictly required both ethically and legally. For example, cation is that for a practitioner’s actions to be defensible
a health authority may apply more stringent data protec- there has to be an underlying rationale that is coherent and
tion measures than are otherwise mandated by law to act can stand up to scrutiny. George Bernard Shaw’s wry com-
as a buffer against preventable breaches of confidential mentary on the professions being “a conspiracy against the
information held on patients in their health records. The laity” is countered by society’s insistence on ethical
General Medical Council recognizes the prerogative of a accountability as a safeguard against professional collusion.
• When thinking through our ethical decision making in The Four Principles
clinical practice, it is not necessary to be equipped with
a working knowledge of theories in philosophical ethics. • A merit of the benefits, burdens, and risks approach is
Instead there are practicable approaches to ethical analy- that it is highly patient centred, but on its own this can
sis that, while informed by a theoretical understanding entail the limitation of suspending wider concerns of
of ethics, are oriented toward making justifiable deci- ethical significance, such as considerations of steward-
sions rather than focusing on technical discussion. None ship, sustainability, and the rights of other stakeholders,
of these is capable of making ethical decisions on our including the practitioner’s institutional responsibilities
behalf; ultimately they all require the individual to make in leadership and management.
her or his own judgment. • The more nuanced framework from which it derives is the
• Three methods in ethical analysis are illustrated to clarify classic distillation of ethical theory in health care contexts
the process of ethical reasoning, out of which the prac- detailed by Beauchamp and Childress (2013). Their four
titioners can arrive at their own ethical conclusion: principles of (1) justice, (2) respect for autonomy,
a. Benefits, Burdens, and Risks (3) beneficence, and (4) nonmaleficence offer a widely
b. The Four Principles known but easily misunderstood set of ethical criteria that
c. The Four Topics have particular relevance to medical practice (Table 4.4).
• Again, these cannot do our reasoning for us, and indeed
Benefits, Burdens, and Risks ethical principles may so conflict with each other that we
have to exercise our judgment to arbitrate between them.
• Ethically uncertain situations may sometimes yield For example, there may be a new and more effective drug
straightforwardly to a basic analysis of benefits, burdens, therapy available for a particular illness, but due to the
and risks, which can be clarified during the course of number of patients potentially involved the additional cost
the clinical encounter through reflection-in-action to the practice would be at the expense of other prescribing
(Example 4.3). priorities. Rather, the four principles act as signposts to
• In Example 4.3, we can readily weigh the relative propor- help us identify the ethically significant aspects of a clinical
tions of risks, burdens, and benefits to see the clear lack situation on which to base our actions.
of ethical justification for screening (Table 4.3). There • Justice. In Example 4.4, fairness to Mr. Gordon involves
still remains the matter of responding to the father in an taking seriously the persistence of unpleasant symptoms,
appropriately professional fashion, but the ethical analy-
sis thought through during the course of the consulta-
tion will inform that discussion in terms of the father’s • EXAMPLE 4.4 Troubling Symptoms yet Normal
concern for his daughter’s best interests. Test Results
James Gordon is a 55-year-old patient who experiences sudden
attacks of nausea, sweating, and headache roughly once a month
• EXAMPLE 4.3 A Father Requests Genetic and sometimes more frequently. He is moderately hypotensive.
Testing for His Daughter Following referral to the local general hospital recently, there were
no abnormal findings from other investigations. As there has been
Following his wife’s death from breast cancer, Mr. Svoboda is no improvement in his symptoms, he is desperate for some
anxious about the possibility of a genetic cause that may have resolution and is determined to have further investigations
been passed on to his daughter, who is now 10 years old. He including a computed tomography (CT) scan, for which he is
wants her to be tested for inherited BRCA1 and BRCA2 willing to pay. He asks for referral to a different consultant at
mutations associated with the disease. another hospital as a private patient.
TABLE
4.3 A Father Requests Genetic Testing for His Daughter
Benefits Burdens Risks

None to the child, even in the Undergoing a screening process that Unpleasant experience for the daughter
event of an “all-clear,” since is not clinically indicated at her age. attending the clinic to have a sample taken.
it is the father who is seeking Harmful consequences to the child in Distress resulting from the explanation given to
reassurance here; in the the event of an untoward test the daughter (so as to gain her assent) of
event of an untoward result, result; introduction of a damaging why she is undergoing the screening process.
there are no risk-reduction anxiety unnecessarily early in her Possibility of generating a negative association
strategies relevant to life about the likelihood of future with medical services that may be harmful to
childhood. disease. the child’s trust and impact on how she
relates to health professionals and clinical
encounters in the future.
CHAPTER 4 Ethics 23
TABLE
4.4 The Four Principles
Interpretation Examples Not to Be Confused With

1. Justice
Fairness Avoiding statements in patient notes that are pejorative (e.g., Legal justice
poor historian). Retributive justice
A patient who is a frequent attender to your practice, and in your view One-size-fits-all style of
unnecessarily so, insists on a hospital referral. Your letter to the equal distribution; blind
consultant is careful not to suggest disparagingly that the patient disregard for significant
needlessly consumes clinical time and resources (e.g., “This patient differences in the needs
is never away from my surgery”). of patients.
2. Respect for Autonomy
Respect for A patient who presents with chronic obstructive pulmonary disease The idea that anything we
self-determination declines any help with smoking cessation. You focus instead on want to do is our own
achieving therapeutic concordance in view of the patient’s concern.
multimorbidity, and signal that you might come back to the smoking
another time.
An elderly patient with a stress-related illness is adamant that she will
continue to look after her husband at home, despite his advancing
dementia and the availability of a place in a local care home. Rather
than press the issue you suggest keeping this as an option and
arrange a timely follow-up with her.
3. Beneficence
Acting in the A patient fails to attend an appointment to discuss the results of a Doing good
patient’s best blood test, which shows abnormal liver function indicative of Benevolence
interests harmful alcohol dependency. You then have a letter sent out and ask
reception staff to contact the patient by telephone to arrange an
urgent appointment.
A Muslim patient has recently started a course of medication three
times daily, but is greatly concerned as it will not be possible for him
to sustain this regimen during Ramadan. You write him up instead
for a single daily dose which is not ideal for a steady concentration
but will provide him with sufficient cover during the fast.
4. Nonmaleficence
Weighing benefits of A patient with mild to moderate depressive illness had become Do no harm
treatment versus dependent on the medication and was stepped down on these Nonmalevolence
risk of harm leading to complete withdrawal. His symptoms have since returned, Nonmalfeasance
and because the previous treatment worked so effectively, he
requests the same prescription. You ask him instead to try cognitive
behavioural therapy in combination with a safer but less potent drug.
A patient is suffering poor quality of life directly as a result of her
experience of menopause. She is finding it impossible to fulfil her
obligations at work, thus exposing her to loss of income, and is also
unable to cope at home, with consequences for relationships with
her husband and teenage children. You offer her hormone
replacement therapy as the risks in the interim are not
disproportionate to the overall improvement she is likely to gain.
which interfere with his ability to work and to enjoy attention as a private patient. If Mr. Gordon insists on
social activities. It also involves offering some alternative going ahead then it is proper to respond with due coop-
to the pursuit of more tests as a private patient that either eration. Further, in so doing the ongoing professional
repeat those already done or are not clinically indicated relationship of trust and confidence is promoted.
in the absence of abnormal findings. Options might • Beneficence. With a view to Mr. Gordon’s best interests,
include exploring other approaches in primary care or it is appropriate to offer guidance on the usefulness of
offering a referral as previously for a second opinion. repeating tests that were performed only recently to no
• Respect for autonomy. It is a matter of Mr. Gordon’s self- avail, and the advisability of incurring the unwarranted
determination whether he should pursue medical personal expense he is contemplating. It is possible that
he is not fully aware of the scientific basis for being hesi- • Medical indications. Though not a severe case of acne,
tant about this and would benefit from an explanation. neither is it a transient flare-up, and Ken’s complexion is
• Nonmaleficence. Beyond the economic harm of paying for obviously a significant and continuing problem. It is
private consultations and unnecessary clinical workup, likely that the demands of work contribute to this, and
given Mr. Gordon’s impaired ability to work, and the health choices may also be a factor in promoting the
potential burdens of inconvenience and stress, there is condition of his skin in the long term.
also the reality that no investigations are entirely free of • Patient preferences. Ken is categorical that he would rather
risk. With regard to his intention to seek a CT scan, it not put up with the problem indefinitely and he would
may be that he does not appreciate the exposure to ioniz- like antibiotic therapy to clear it up quickly. That aside,
ing radiation entailed. Taken together, all the risks require it would be appropriate to alert him to lifestyle options
to be outweighed by the benefits, and this would be an that could help with this, including diet and exercise,
important area in communicating with Mr. Gordon. and possibly also practical considerations such as hand
hygiene in the workplace.
The Four Topics • Quality of life. In addition to the normal stress of his job,
the chronic unsightly appearance of his skin is substan-
• One of the difficulties with the four principles approach tially affecting his quality of life and the confidence to
is that its abstraction may not easily be amenable to some enjoy social contact. The benefit to Ken of an efficient
clinical situations in real life. As an alternative, Jonsen, and effective approach to managing his acne would be
Siegler, and Winslade (2015) set out their four-topic significant.
approach that is patient centred while also being cogni- • Contextual factors. Antimicrobial stewardship is a respon-
zant of governing obligations and values, thereby com- sibility in professional ethics because of the risks of
bining strengths of the two models discussed earlier. It unwarranted prescribing that impact both on the patient
provides a structure for comprehensive assessment of concerned and on the wider community. Decisions on a
ethical relevance by considering in sequence: case-by-case basis depend, in part, on the proportionality
1. Medical indications of likely benefit to the individual. In Ken’s situation, an
2. Patient preferences important contextual factor at his stage of adulthood is
3. Quality of life the opportunity to form relationships with the potential
4. Contextual features. for life partnership and family, which in this instance is
• These assessments, though separate, are kept together to preventably limited by his presenting complaint.
ensure attention to what is ethically salient in the round,
and in the light of that to help clarify what would be the
most appropriate way to proceed. The order of topics Summary: Professional Ethics in
denotes an expanding horizon of attention rather than a General Practice
hierarchy of ethical importance, and Example 4.5 illus-
trates how they might be used. • This chapter has clarified distinctive qualities of general
practice ethics; the nature of professional ethics compris-
ing the three intertwined domains of ethics, law, and
• EXAMPLE 4.5 A Patient Seeking Antibiotics
professionalism; and some practicable approaches to
Ken Porter is a 28-year-old science teacher who has been ethical analysis.
steadily troubled by unsightly acne over the past 15 months. He • To conclude, the mnemonic PROFESSIONAL ETHICS
comments that even throughout adolescence he never
can be formed from 18 key terms used in this chapter
experienced a skin problem so badly. Work is difficult enough
without feeling embarrassed in front of his classes, and it also (Table 4.5).
interferes with his personal life. Topical preparations purchased
over-the-counter have not proved useful, and this is his first visit
to the practice since the onset.
TABLE
4.5 Professional Ethics in General Practice
P Public trust Upholding the confidence of society in the profession

R Regulatory framework Normative criteria of practice in relation to maintaining registration
O Obligations Duty of care, responsibilities to colleagues, statutory requirements
F Formation Continuing professional development, reflective practice, values, professional identity
E Ethical framework Normative criteria of practice in relation to moral reasoning
CHAPTER 4 Ethics 25
TABLE
4.5 Professional Ethics in General Practice—cont’d
S Standards Performance benchmarks in clinical practice

S Social responsibility Attention to the effects of policy and practice on communities
I Institutional roles Leadership, management, participating in organizational structures
O Outcomes Attention to the efficacy of practice, clinical audit
N Non-collusion Not engaging jointly in pretence against or with patients
A Appraisal Accountability to clinical supervision
L Legal framework Normative criteria of practice in relation to the State
E Everyday Emphasis on the ordinary and routine in clinical situations
T Thought-through Ethical reasoning, principles of ethical practice
H Habits Character of the practitioner; habits of the head, hands, and heart (knowledge, skills, and
attitudes) that characterize the practice of medical professionals
I Intentions Attention to the goals of clinical practice in specific situations
C Consequences Attention to the scope for unintended outcomes that could result from actions that are being
considered
S Safety Acting in line with measures to avoid preventable harm
tion in Europe.
References Papanikitas, A., & Spicer, J. (Eds.), (2018). Handbook of primary care
ethics. Boca Raton, FL: CRC Press/Taylor & Francis Group.
Beauchamp, T. L., & Childress, J. F. (2013). Principles of biomedical Rogers, W. A., & Braunack-Mayer, A. J. (2009). Practical ethics for
ethics (7th ed.). Oxford: Oxford University Press. general practice (2nd ed.). Oxford: Oxford University Press.
Dowie, A., & Martin, A. (2011). Ethics and law in the medical cur- Stirrat, G. M., Johnston, C., Gillon, R., & Boyd, K. (2010). Medical
riculum. AMEE Education Guide No. 53. Dundee: Association ethics and law for doctors of tomorrow: The 1998 consensus state-
for Medical Education in Europe. ment updated. Journal of Medical Ethics, 36, 55–60.
Jonsen, A. R., Siegler, M., & Winslade, W. J. (2015). Clinical ethics:
A practical approach to ethical decisions in clinical medicine (8th
ed.). New York, NY: McGraw Hill.
5
Disability
LYNN LEGG, JANE TRACY
Effect of Impairment on Carrying Out Normal Day-to-Day Reasonable Adjustments
Activities Advocacy
Prevalence of Disability Principles of Practice
Human Rights and Disability Legal Issues of Consent and Capacity
Rehabilitation Comprehensive Health Care
Physical and Rehabilitation Medicine Mental Health Issues
Health and Social Care Professionals Down Syndrome
Assistive Technologies Health Care for Children With Down Syndrome
Rehabilitation Settings Health Care for Adolescents With Down Syndrome
Evidence-Based Rehabilitation Health Care for Adults With Down Syndrome
Intellectual Disability/Learning Disability/Developmental Health Care for Older People With Down Syndrome
Disability Carers
Terminology Caring for the Carers
The Importance of Language Resources for Carers
Health and Learning Disability: The Evidence Cycle of Good Health Care—A Shared Responsibility
Providing Primary Health Care
OBJECTIVES
• A disabled person is a person who has a disability. A person neurone disease, cancer, multiple sclerosis (MS),
is disabled if he or she has a sensory, physical, mental, or osteoarthritis, rheumatologic disorders
intellectual impairment and the impairment has a • Permanent impairments: personality disorder,
substantial adverse effect on his or her ability to carry out developmental disabilities, sensory impairment,
daily activities required to maintain health and well-being. traumatic brain injury, stroke
(Office for Disability Issues UK, 2010). • Transient or self-limiting impairments: head and neck pain,
• Impairments are not synonymous with underlying frozen shoulder, cellulitis, or flu
pathology, but rather the manifestation of that pathology. • Circadian rhythm impairments: rheumatoid arthritis,
• Disability can result from a wide spectrum of sensory, morning stiffness and functional disability in the early
physical, mental, or intellectual impairments ranging from morning
mild to serious: • Seasonal rhythm impairments: seasonal affective disorder
• Recurring episodic impairments: mental health conditions • Impairments following surgical or medical interventions:
(depression, bipolar affective disorders, schizophrenia), recovery from joint replacement surgery, reduced upper
asthma, and epilepsy limb strength and pain due to breast cancer treatment
• Progressive impairments: macular degeneration, chronic
obstructive pulmonary disease (COPD), dementia, motor
26
CHAPTER 5 Disability 27
Human Rights and Disability

Effect of Impairment on Carrying Out
Normal Day-to-Day Activities All people have equal rights to participate in their commu-
nities and to access community services, including health
• Complete or substantial dependence on another. services. People with disabilities experience inequalities and
• Time taken may be significantly longer (e.g., dressing social injustice, including rejection, isolation, discrimina-
and toileting) (Australian Bureau of Statistics, 2012a). tion, harassment, stigma, segregation, and institutionalisa-
• May affect a person’s ability to sustain an activity for long tion. They also currently experience barriers in accessing
periods of time or carry out the activity repeatedly. A education and employment opportunities, and timely and
person may be able to carry out an activity (such as appropriate health care and services.
walking) but suffer significant pain in doing so, which in
turn may limit the performance of that activity (Australian Rehabilitation
Bureau of Statistics, 2012a).
• An impairment may not have an adverse effect on a per- • The WHO (2011) defines rehabilitation as “a set of mea-
son’s ability to carry out a single daily living activity in sures that assist individuals who experience, or are likely to
isolation (such as getting out of bed). However, the effect experience, disability to achieve and maintain optimal func-
of an impairment on the person’s ability to carry out mul- tioning in interaction with their environments.”
tiple interconnected activities (such as getting out of bed, • The term rehabilitation is also a process aimed at enabling
toileting, showering, dressing, breakfasting to go to work) persons with a disability “to reach and maintain their
may be substantial. (Office for Disability Issues UK, 2010) optimal, physical, sensory intellectual, psychologi-
• Interruption of normal day-to-day activities such as a cal and social functional levels thus providing them
frequent need to go to the toilet. (Office for Disability with the tools they need to attain independence and
Issues UK, 2010) self-determination”.
• May have a circadian rhythm such as rheumatoid arthri- • The aims of rehabilitation are to:
tis (i.e., joint pain, morning stiffness, and functional a. prevent loss of function;
disability in the early morning hours). (Office for Dis- b. improve or restore function;
ability Issues UK, 2010) c. compensate for lost function;
• May affect a person’s ability to estimate or assess danger d. halt or slow decline in function;
(e.g., road safety, touching very hot things, or an inability e. maintain function. WHO. World report on Disabil-
to protect oneself from potential exploitation or violence). ity. 2011
(Office for Disability Issues UK, 2010)
• May cause a person to modify a behaviour (e.g., a person Physical and Rehabilitation Medicine
with rheumatoid arthritis may limit activities first thing in
the morning to avoid pain or to wait for prescribed medica- Physical and rehabilitation medicine doctors (physiatrists)
tions to take effect). Equally, a person may employ an are involved in the diagnosis of health conditions, assess-
avoidance strategy if there is a risk of considerable embar- ment of functioning, and prescription of medical and tech-
rassment in social situations such as faecal incontinence or nologic interventions that manage health conditions and
facial disfigurement. (Office for Disability Issues UK, 2010) optimize functional capacity.
• Physical impairments can cause mental effects and vice
versa (e.g., a person with fatigue or pain may experience Health and Social Care Professionals
difficulties in remembering or concentrating). Similarly,
mental impairments can have physical manifestations Health and social care professionals include occupational thera-
(e.g., a person with a mental impairment such as depres- pists, physiotherapists, orthotists and prosthetists, speech and
sion may experience difficulty in carrying out physical language therapists, rehabilitation nurses, audiologists, psychol-
activities). (Office for Disability Issues UK, 2010) ogists, social workers, and rehabilitation technologists.
• Interventions include:
Prevalence of Disability • Guided practice (such as practice walking and transfer-
ring after knee replacement)
Disability is common, and it becomes more so as popula- • Self-management strategies for people with long term
tions age. Approaches to measuring disability vary but it is health conditions
estimated that 15% of the world’s population have a dis- • Education and training
ability (WHO, 2011). The prevalence is 18.5% in Australia • Exercises
(Australian Bureau of Statistics, 2012b), 18% of the popula- • Manual therapy
tion of England and Wales report limitations in ability to • Use of specific techniques (such as reminiscence
perform day-to-day activities as a consequence of long term therapy, biofeedback, acupuncture, graded activity)
health problems or disability. (Reference: Census 2011, • Alternative or compensatory strategies (such as aug-
UK. Office for National Statistics). mentative and alternative communication)
• Environmental modification (internal and external EVIDENCE-BASED PRACTICE RESOURCES

physical adaptations to the home) FOR GENERAL PRACTITIONERS, PATIENTS,
• Provision of assistive technologies AND CARERS
• Advice and information 1. Cochrane systematic reviews are published online in The
• Work with unpaid carers Cochrane Library (http://www.cochrane.org)
• Support and counselling 2. The Royal College of Physicians London National Clinical
• Promotion of a healthy lifestyle and physical activity Guidelines for Stroke (http://www.rcplondon.ac.uk/
resources/stroke-guidelines)
3. Royal National Institute for the Blind (RNIB): http://www.
Assistive Technologies rnib.org.uk/Pages/Home.aspx
4. Action on Hearing Loss (RNID): http://www.
A product or service designed to enable persons with a dis- actiononhearingloss.org.uk/
ability to achieve and maintain optimal functioning in 5. Stroke Association: http://www.stroke.org.uk/
6. Chest Heart & Stroke Scotland: http://www.chss.org.uk/
interaction with their environments.
7. Parkinson’s UK: http://www.parkinsons.org.uk/
• Examples of assistive technologies: 8. Multiple Sclerosis Society UK: http://www.mssociety.org.uk/
• For people with mobility impairments: wheelchairs, 9. Alzheimer’s Society: http://www.alzheimers.org.uk/
prostheses, orthoses, scooters, bath boards and seats, 10. Arthritis Research UK: http://www.arthritisresearchuk.org/
ramps, grab rails, stair lifts 11. National Rheumatoid Arthritis Society UK: http://www.
nras.org.uk/
• For people with visual impairments: Braille-based type-
12. Motor Neurone Disease Association: http://www.
writers (braillers) and embossers, access technology mndassociation.org/
such as screen reader programs, high-contrast specta- 13. The British Heart Foundation: http://www.bhf.org.uk
cles, radio for people with visual impairment 14. Muscular Dystrophy Campaign: http://www.
• For people with hearing impairments: cochlear implants, muscular-dystrophy.org/
Intellectual Disability/Learning Disability
amplified telephones and mobiles, vibrating alarm clocks,
- Management Guidelines- Developmental Disability: https://
products to relieve the effect of tinnitus, hearing aids tgldcdp.tg.org.au/fulltext/quicklinks/management_guideline.
• For people with cognitive impairment: computer-based pdf
technology for recording of information, text to - Health care in People with Intellectual Disability Guidelines:
speech apps or programs that assist people with https://www.aci.health.nsw.gov.au/__data/assets/pdf_
file/0016/231514/Health_Care_in_People_with_Intellectual_
writing and reading difficulties, pictorial-based elec-
Disability_Guidelines.pdf
tronic timetables, GPS devices - Royal College of General Practitioners: https://www.rcgp.
• For people with speech impairments: speech synthesiz- org.uk/learningdisabilities/
ers, communication boards, modified typewriters, - Royal Australian College of General Practitioners: https://
text to voice software www.racgp.org.au/clinical-resources/clinical-guidelines/
guidelines-by-topic/view-all-guidelines-by-topic/disability/
• Equipment for activities of daily living: modified eating
positive-cardiometabolic-health
utensils, dressing aids, adapted personal hygiene equip-
ment, emergency call systems, dosette boxes for
medication
• Multidisciplinary biopsychosocial rehabilitation for neck
Rehabilitation Settings and shoulder pain among working age adults
• Multidisciplinary biopsychosocial rehabilitation for sub-
• Rehabilitation services can be delivered in a variety of acute low back pain among working age adults
settings including hospitals, clinics, GP surgeries, the • Pulmonary rehabilitation following exacerbations of
home, residential or nursing care homes, school, and chronic obstructive pulmonary disease
work (Australian Bureau of Statistics, 2012b). • Vocational rehabilitation for people with severe mental
illness
Evidence-Based Rehabilitation • Exercise-based cardiac rehabilitation for coronary heart
disease
Examples of systematic reviews in rehabilitation include: • Cognitive rehabilitation for people with schizophrenia
• Occupational therapy for adults with problems in activi- and related conditions
ties of daily living after stroke Source: The Cochrane Library (http://www.cochrane.org)
• Multidisciplinary rehabilitation for acquired brain injury
in adults of working age Intellectual Disability/Learning Disability/
• Home-based versus centre-based cardiac rehabilitation
• Physical rehabilitation for older people in long-term care Developmental Disability
• Multidisciplinary rehabilitation for older people with hip Terminology
fractures
• Multidisciplinary rehabilitation for adults with multiple • Learning disability is synonymous with intellectual dis-
sclerosis ability. Intellectual disability involves impairments of
intellectual functioning, acquired during the develop- • General Issues in Primary Care for Adults with Devel-
mental period, that impact on the persons ability to opmental Disability
manage the tasks of daily life in conceptual, social • Physical Health Guidelines for Adults with Develop-
and practical domains. Severity relates to the deficits mental Disability
in adaptive functioning. (American Psychiatric Asso- • Behavioural and Mental Health Guidelines for Adults
ciation, 2013). Developmental disability includes motor with Developmental Disability
impairments (cerebral palsy), social impairments • Care should be delivered to people with disabilities as
(autism spectrum disorders), sensory impairments part of a collaborative team.
(vision and hearing), and cognitive impairment (intel- • The aetiology of the disability should be established.
lectual disability). • Be aware of the communicative role of behaviour and the
• People with intellectual disability have a significantly behavioural manifestations of illness, pain or discomfort
reduced ability to understand new or complex informa- (physical and mental), and environmental stressors.
tion and to learn and apply new skills. This results in a • Disease prevention is essential.
reduced ability to cope independently (WHO, 2013). • Avoid the unnecessary or inappropriate use of medica-
• It is estimated that around 1% of the population have tions that may cause harm.
an intellectual disability (depending on definitions and • Be aware of the increased prevalence in people with dis-
methods of ascertainment) (McKenzie et al., 2016). abilities of:
• vision and hearing impairment;
The Importance of Language • dental disease;
• cardiac disorders (congenital and acquired);
Language reflects the attitudes of the speaker, and influences • musculoskeletal disorders including spasticity, scolio-
those of others. Inappropriate language can cause hurt and sis, osteopenia/osteoporosis, osteoarthritis;
offense to people with disabilities and their families and • respiratory disorders, particularly aspiration pneumo-
friends. People with disabilities are people first. Their disability nia, which are among the most common causes of
impacts on their life experience, but it does not define them. death for people with developmental disability;
Using ‘person-first’ language, such as ‘Nick is a man with • epilepsy, as seizures are a prominent cause of death;
intellectual disability’ focuses attention on the person, rather • gastrointestinal disease, particularly reflux and consti-
than the disability. pation, and the impact of these on quality of life and
behaviour (Helicobacter pylori is more common in
Health and Learning Disability: The Evidence people with developmental disability.);
• endocrine disorders such as thyroid disease, hypogo-
• People with learning disabilities experience: nadism, and diabetes;
• higher rates of morbidity and premature death than • mental health, as these disorders can be difficult to
their non–learning-disabled peers, a significant propor- identify when people have communication difficulties.
tion of which is avoidable (Emerson & Baines, 2010; Effective treatment depends on accurate diagnosis.
Heslop et al., 2013; Hollins & Tuffrey-Wijne, 2013;
World Health Organisation, 2018; Trollor et al., 2017 ); Providing Primary Health Care
• chronic and complex health and social needs;
• barriers to accessing health care; • Person-first approach. Think first of the person as a man
• fewer opportunities for preventive health and health or woman of a particular age, background, and physique.
promotion interventions; What care would you provide to this person if he or she
• more undiagnosed and un/undertreated health condi- did not have a disability? Is there any reason to modify
tions. (Lennox, Bain, & Rey-Conde, 2007; Emerson, that care? If so, why?
et al., 2011; Kavanagh, Krnjacki, & Kelly, 2012; • Then consider the person’s disability:
Trollor et al., 2017). • How does the intellectual disability affect health and
• Hospital care: People with intellectual disabilities encoun- health care? Consider the person’s life experiences and
ter particular barriers to high quality care while in hos- opportunities, communication and cognitive abilities,
pital (Iacono et al., 2014). ability to make considered decisions, and ways the
• Standardized health assessments are effective in the detec- person plans and anticipates consequences.
tion of previously unrecognized and/or unmet health • What is the cause of the disability and does that aetiologic
needs, including life-threatening conditions, and lead to diagnosis inform health care? For example, someone
targeted actions to address health needs (Lennox, Bain, with Down syndrome (DS) is at increased risk, for a
& Rey-Conde, 2007; Robertson, Roberts, Emerson, lifetime, of thyroid dysfunction, hearing and vision
Turner, & Greig, 2011; Sullivan et al., 2011). impairment, immune deficiency, and respiratory infec-
• Consensus guidelines in the primary care of adults with tion. In the teens, 20s, and 30s, these individuals are
developmental disabilities (Sullivan et al., 2011) provide at risk of depression and anxiety; and in their 40s and
evidence under these headings: beyond they are at risk of Alzheimer disease.
• Demonstrate respect: after using the strategies listed, acknowledge the impor-
• Address the person directly and use a tone of voice tance of the message by apologizing and expressing regret
consistent with his or her age. at not understanding.
• Ask the person’s permission before inviting the accompa- • All people communicate. People communicate through
nier into the consultation or asking questions of the facial expression, body language, and behaviour. It may be
accompanier, and always ensure the focus remains on clear that someone finds a sensation unpleasant from a
the person. grimace, is cold by shivering, or wants to leave when
• Support the person to understand. Many people with intel- becoming agitated and looking toward the door. Acknowl-
lectual and associated developmental disabilities have edging the person’s experience and communication dem-
communication difficulties: onstrates that you hear, value, and respect his or her wishes.
• Address the person by name, and use eye contact and/
or touch to get his or her attention. Reasonable Adjustments
• Assume competence when unsure of someone’s ability
to understand, and then adjust accordingly. A person’s • Reasonable adjustments are those required under the
ability to understand may be better than his or her antidiscrimination legislation to ensure services are as
ability to express oneself (and vice versa). accessible and effective for people with disabilities,
• Speak slowly, in short clear sentences. including intellectual disabilities, as they would be for
• Explain what will happen in the consultation to help people without disabilities (Turner & Robinson, 2011).
the person know what to expect. • Knowing a patient has an intellectual disability provides
• Ask one question at a time and provide adequate guidance to the general practitioner (GP) as to the rea-
time for the person to think about your question sonable adjustments to care that may be required to
and formulate a reply. Avoid leading questions ensure equity. A person may:
and check responses by asking again in a differ- • require more time for a consultation to enable him or
ent way. her to understand the information presented and have
• Avoid using abstract concepts such as time, as the a chance to ask questions;
person may find them more difficult to understand. • wish to have a family member, paid carer, or advocate
Use significant events such as meals, social or sporting present during the consultation;
events, birthdays or celebrations, rather than hours/ • have difficulty reading and writing and may need infor-
days/months when asking questions related to time. mation presented in easy English or in audio form;
• Use visual information (pictures, diagrams, signs, ges- • find it difficult to wait in a waiting room and it may
tures) to aid comprehension. be more appropriate to wait with his or her carer in
• Check understanding by asking the person to demon- comfortable surroundings nearby and be rung a few
strate or repeat what you have said in his or her own minutes before the appointment.
words.
• Some people may have limited literacy and thus may Advocacy
have difficulty reading patient information or appoint-
ment letters. The person may wish to involve support People with intellectual disabilities require their doctor to
workers or family members to assist with these tasks. be an advocate in negotiating the health system and making
• Support the person to express oneself. Cognitive impair- sure their rights to equity in health care are upheld.
ment makes identifying and verbalizing difficult and
physical condition may impact on speech. Principles of Practice
• If you do not know, ask the person how he or she commu-
nicates (i.e., to indicate yes/no, use communication aids). 1. Take a person-centred approach by seeking to under-
• If the person uses a communication device, ensure he stand the perspective, personality, experiences, and
or she has access to it, then read the directions (usually strengths of each person.
on or in the device or book) and use it together. 2. Support empowerment of the person to make as many
• Use visual cues such as objects, pictures, or diagrams. decisions as possible about his or her own life.
• If you can’t understand then ask for help. There may be times 3. Enable choice by providing information about choices
when you do not understand what the person is saying. In and support for the person to choose which suits him
this situation, it may be helpful to ask the person: or her best.
• to repeat a response; 4. Treat each person with dignity and respect, recognizing
• to say it another way (using different words, for instance); the inherent shared humanity in all people.
• to illustrate his or her method of saying “yes” and “no” and
then ask yes/no questions to identify what is being said; Legal Issues of Consent and Capacity
• if you could ask an accompanier to help you understand.
• Never pretend to understand when you do not as this deval- • For children, parents generally hold legal power to make
ues the person’s communication. If you can’t understand decisions on their behalf.
• For adults, it is presumed that they can make their own promotion (adequate exercise, healthy diet, not smo
decisions about health care. If this is in doubt it is up to king, etc.).
the health practitioner to establish the person’s legal
capacity and ability to: Mental Health Issues
1. understand the information provided;
2. believe and retain the information provided; • People with intellectual disabilities have a higher rate of
3. evaluate the relevant information; mental health issues.
4. express a choice. • Depression, anxiety, mania, and psychosis all occur in
• People who do not have capacity will require someone people with intellectual disabilities. When people have
to assist them or to make the decision on their behalf. cognitive and/or communication impairments they will
Whatever their capacity the person concerned should be express their symptoms through changes in their behav-
as actively involved in decision making as possible. iour. Depression may present as social withdrawal, irri-
tability, changes in appetite and sleep; mania as increased
Comprehensive Health Care activity, vocalization, and sleep disturbance; psychosis as
unexplained fear or appearing to respond to visual or
• Holistic health care is that in which the person’s physical auditory hallucinations.
and mental health are understood and supported in the • Treatment of disorders of mental health is the same as in
context of that person’s social context and life experiences. the general population. Effective treatment depends on
• All doctors should adopt a proactive approach to a per- accurate diagnosis. Vigilance by both the GP and
son’s health care. family/support staff with respect to response to medica-
• Comprehensive health assessments should always include tion and side effects is vital, as the patient is likely to
a systems review, a medication review, and, for those have difficulty reporting these.
in whom the cause of their intellectual disability is • Principles of medication use:
unknown, consideration of an aetiogic review every few 1. Effective treatment depends on accurate diagnosis.
years is recommended. Understanding of the genetic 2. Start at a low dosage and increase slowly (start low,
causes of disability is growing rapidly, and referral to go slow).
genetic services could be enlightening. 3. Always use the lowest effective dose.
• Comprehensive care includes a focus on disease preven- 4. Review medications regularly with particular regard
tion (immunization, cancer screening, etc.) and health to indications and side effects.
HEALTH CARE FOR PEOPLE WITH INTELLECTUAL DISABILITIES

Some Tips for Consultations
Challenge One Possible Solution

Prior to appointment Required appointment may not be made General practitioner or nurse initiates appointment and
reminds patient
Person/family/staff may not know what Contact person and/or carer and detail information
information is required required at appointment
Waiting time may be an issue Minimise waiting time
Arrange for person to wait nearby in comfortable
surroundings (park, coffee shop, car). Ring him or
her a few minutes before appointment
At consultation Person may feel anxious or frightened Greet warmly and establish rapport by chatting about
something of interest to person
Person may become irritated Speak to the person concerned. Remember he or she
is your patient, not the family member or paid carer
Person may get bored, become anxious, Include the person in the discussion, both verbally
or want to leave and nonverbally, no matter his or her capacity
Information given at consultation may not Write a summary of outcomes of consultation, the
be retained/passed on accurately to next steps in care, and who is responsible
those involved in care
After consultation Person may be anxious next time Discuss with patient/carer what went well, and how to
improve for next time
Record in notes
RESOURCES FOR HEALTH PROFESSIONALS • Dental/oral health. Dental review 3 to 6 monthly from
first teeth for monitoring development plus prevention
Understanding Health and Intellectual Disability: www.
and treatment of oral disease.
intellectualdisability.info
Therapeutic Guidelines Ltd. (2012). Management • Gastrointestinal. Monitor diet and weight. Consider gas-
guidelines: Developmental disability. Version 3. Melbourne: trooesophageal reflux disease (GORD), H. pylori, coeliac
Therapeutic Guidelines Ltd. https://tgldcdp.tg.org.au/fulltext/ disease, and constipation as these are all more common
quicklinks/management_guideline.pdf in children with DS.
NSW Agency for Clinical Innovation: www.aci.health.nsw.
• Atlantoaxial instability. Monitor for signs and symptoms
gov.au/resources/intellectual-disability/intellectual_disability_
training/id-training-videos of cord compression.
Management Guidelines: Developmental Disability (2012) • Haematologic/immunologic. Be alert to increased risk of
https://tgldcdp.tg.org.au/fulltext/quicklinks/management_ infections and leukaemia.
guideline.pdf
The Guide: Accessible Mental Health Services for People
with Intellectual Disability. A guide for providers. https:// Health Care for Adolescents With
tgldcdp.tg.org.au/fulltext/quicklinks/management_guideline.pdf Down Syndrome
• Sexuality. Girls will require education and support to
manage periods (see Resources for Carers). Everyone
Down Syndrome requires additional advice about sex and contraception.
Teenagers with DS are more vulnerable to sexual exploi-
• People with DS still experience significant barriers to the tation/abuse. They require education about appropri-
receipt of high-quality health care, and health outcomes ate behaviours (themselves and others), how to keep
compare unfavourably with those of the general population themselves safe, and how to get help if needed. For
(Therapeutic Guidelines—Developmental Disability 2012; those unable to advocate for themselves, adequate pro-
Tracy 2011; Torr et al., 2010). tection and supervision are required to ensure their
• They are at increased risk of a range of physical and safety.
mental health conditions, often live with multiple unrec- • Skin. Folliculitis/acne may affect self-esteem and interac-
ognized health issues, and have limited access to disease tions with others. Energetic treatment is required.
prevention and health promotion interventions. • Mental health. Biologic, psychologic, and social factors
contribute to increased risk of disorders of mental health,
Health Care for Children With especially anxiety and depression.
Down Syndrome • Transition from paediatric to adult services. Consider all ser-
vices used by the child and family: recreation, respite services,
• General care. As for all children, primary care has respon- school and postschool options (employment, education,
sibility for monitoring health and development. Health other), and mental health services. The GP has an important
promotion and disease prevention: diet, exercise, weight role in providing support and guidance through this time of
management, immunizations. change. Encourage the adolescent to take an increasingly
• Neonatal examination and investigation to identify con- active role in his or her health management.
genital anomalies, including cardiac and gastrointestinal
malformations. Health Care for Adults With Down Syndrome
• Nutrition. Dietitians can also offer information and support
(note: calcium, vitamin D for osteoporosis prevention). • Health monitoring. Risk factor identification (e.g., car-
• For families. Link to services and supports, including diovascular disease).
early intervention services, dental services, allied health • Health promotion and disease prevention. Diet, exercise, weight
services, and support services such as the Down Syn- management, cancer screening (may include smear test,
drome Association. mammography, bowel cancer screening), immunizations.
• Hearing. Auditory brainstem evoked response at 0 to 6 • Annual health assessments underpin proactive health care
months; audiology annually from 1 to 5 years; two yearly and enable early detection, health promotion, and disease
from age 5 to 18 years and at any time concern regarding prevention.
hearing loss is raised by parents, carers, or screening tests. • Behaviour change. A change in behaviour is a communi-
• Vision. Ophthalmologic examination at 0 to 6 months; cation—consider disorder of physical, dental, or mental
annually to age 5 years; 2 yearly to age 18 years and at health, and environmental causes.
any time concern regarding vision is raised by parents, • Dental and oral health. Dental review 6 monthly.
carers, or screening tests. • Cardiovascular. Mitral valve prolapse may develop during
• Endocrine. Check thyroid function at birth then annually adulthood (50% of adults)—regular cardiac examination
throughout childhood and again if suggestive symptoms and/or echocardiography is required.
or signs are noted. Assess specifically for undescended • Atlantoaxial instability. Cervical cord compression (~2%
testes and hypogonadism. of people with DS). Symptoms/signs include neck pain,
torticollis, limb weakness, increased reflexes, change in • Proactive assessment and investigation is required to
gait and/or bladder/bowel function, sensory changes. exclude treatable causes of functional decline.
• Hearing. Audiology 3 yearly and if suspicion of hearing
loss raised by carers or screening Carers
• Vision. Ophthalmological review at 30 years; every 5
years thereafter and if suspicion of visual loss raised by • In Australia, around 12% identify as carers (Australian
carers or screening. Bureau of Statistics, 2012a); 70% of primary carers are
• Thyroid function. Annually and whenever suggestive women and 83% of carers were caring for someone in
symptoms or signs are noted their household. Figures are similar in the United
• Mental health. Biologic, psychologic, social factors con- Kingdom (Carers Trust UK, 2012).
tribute to increased risk of disorders. Anxiety and depres- • When a person comes to his or her medical practitioner
sion more common. accompanied by a carer it is important to:
• Alzheimer disease. Average age of diagnosis early 50s; rare a. establish the relationship between the person and carer;
under 45 years; exclude physical/mental cause of func- b. clarify the carers role;
tional decline. c. ensure the person’s consent for the carer to be present;
• Gastrointestinal. Increased risk of GORD, coeliac disease, d. speak directly to the person, not to the carer. If the
and chronic constipation. person finds it difficult to express oneself, or if you
• Osteoporosis. Bone mineral density testing in early adulthood have trouble understanding the person, ask if you may
and repeat at menopause in women and ~40 years for hypo- ask questions of the carer. Always involve and include
gonadal men. Discuss and implement prevention strategies: the person in the consultation through verbal and
diet and exercise, and calcium and vitamin D supplementa- nonverbal communication.
tion if levels are low or the person is on an anticonvulsant. e. write down the key outcomes of the consultation, any
• Medication. Regular (3 monthly) review. Monitor response follow-up tasks that are required (investigations,
and side effects: reevaluate indications and efficacy (cease if further information, monitoring for response to treat-
ineffective); ensure lowest effective dose; educate patient and ment and side effects of medication, etc.), and
carers about expected response and side effects. Consider arrangements for review. This information must be
prepackaging of medication by pharmacy to ensure accurate given to the person and/or the carer to ensure it is
dosing and safety. Be aware of sensitivity to psychoactive relayed accurately to other carers involved.
medication (“Start low, go slow”) and be vigilant for side
effects (behaviour change may be an early symptom). Caring for the Carers
Health Care for Older People With • Family members who care for a relative tend to have lower
Down Syndrome rates of workforce participation, lower incomes, and reduced
connection with their social group and communities.
• The increasing lifespan of people with DS means more • They may find aspects of care difficult, and struggle to
people experience the conditions of ageing including make the time to address their own physical and mental
menopausal symptoms, arthritis, cardiovascular disease, health issues.
osteoporosis, sensory loss, and dementia. • It may therefore be helpful if the GP invites the carer to
• Alzheimer disease occurs earlier in people with DS with an come for his or her own consultation to discuss ways to
average age of diagnosis in the early to mid-50s. Symptoms acquire assistance with the caring role (services, equip-
may be difficult to differentiate from physical disease (e.g., ment, therapy, local activity groups for people requiring
hypothyroidism, anaemia); medication effects (e.g., nausea, care, respite, etc.).
confusion, dizziness); mental illness (e.g., depression, psy- • The consultation also provides an opportunity to review
chosis); and sensory deterioration (e.g., hearing and vision). the carer’s physical and mental health status and needs,
and to address current and potential issues.
RESOURCES FOR PARENTS AND Resources for Carers

PROFESSIONALS
• Carer organizations provide advice, peer support, and
Down Syndrome Association of Australia: http://www access to information and resources. Examples:
.downsyndrome.org.au
Down Syndrome Association UK; http://www.downs • Carers UK: http://www.carersuk.org/newsroom/stats-
-syndrome.org.uk and-facts
Down Syndrome Association Scotland: www.dsscotland • Carers Australia: http://www.carersaustralia.com.au
.org.uk • Local councils/authorities provide information about ser-
Down Syndrome Association Ireland: http://downsyndrome vices, resources, support, and activities.
.ie/campaigns-and-projects/person-first-language/
• Therapy services include occupational therapists, physio-
therapists, and speech therapists.
1. Person with disability

becomes unwell and
expresses this in
some way.
2. Person/carer/family/other
detects change in health
8. Health status or behaviour, appreciates
improved significance and arranges
appointment
3. Person attends
appropriate health
professional for
7. Person reviewed timely assessment
by health
professional-plan
revised if required.
4. Comprehensive
6. Management plan assessment
implemented by performed and
person and carers issues identified
5. Development
of Management
plan by GP in
collaboration
• Fig. 5.1 Cycle of good health care. GP, General practitioner.
World Health Organization. (1946). Preamble to the Constitution of the

Cycle of Good Health Care— World Health Organization as adopted by the International Health
Conference, New York, June 1946; signed July 1946 by the represen-
A Shared Responsibility tatives of 61 states (Official Records of the World Health Organiza-
tion, no. 2, p. 100) and entered into force on 7 April 1948.
• Optimal health care for someone with a disability
Turner, S. L., Nair, A., Sedki, I., Disler, P. B., & Wade, D. T. (2005).
requires a cycle (Fig. 5.1) (Tracy, 2013). At each step Multi-disciplinary rehabilitation for acquired brain injury in
there is a risk that the next may not occur. adults of working age. Cochrane Database of Systematic Reviews.
• GPs and support staff/family have a shared responsibility Taylor, R. S., Dalal, H., Jolly, K., Moxham, T., & Zawada, A. (2010).
to work collaboratively with the person with the intel- Home-based versus centre-based cardiac rehabilitation. Cochrane
lectual disability to ensure the steps of the cycle are worked Database of Systematic Reviews.
through and completed to ensure the person concerned Crocker, T., Forster, A., Young, J., Brown, L., Ozer, S., … Smith, J.
achieves and maintains optimal health and function. (2013). Physical rehabilitation for older people in long-term care.
Cochrane Database of Systematic Reviews.
Handoll-Helen, H. G., Cameron, I. D., Mak-Jenson, C. S., &
Further Reading Finnegan, T. P. (2009). Multidisciplinary rehabilitation for older
people with hip fractures. Cochrane Database of Systematic Reviews.
World Health Organization. (2013). ICF Online. Khan, F., Turner, S. L., Ng, L., Kilpatrick, T., & Amatya, B. (2007).
Office for National Statitistics. (2013). Disability in England and Multidisciplinary rehabilitation for adults with multiple sclerosis.
Wales, 2011 and comparison with 2001. Cochrane Database of Systematic Reviews.
United Nations. (2006). Convention on the rights of persons with dis- Hillier, S. L., & McDonnell, M. (2011). Vestibular rehabilitation for
abilities and optional protocol. unilateral peripheral vestibular dysfunction. Cochrane Database of
Equality and Human Rights Commission. (2013). The United Nations Systematic Reviews.
Convention on the rights of people with disabilites—What does it Karjalainen, K. A., Malmivaara, A., van-Tulder, M. W., Roine, R.,
mean for you? 2010. Equality and Human Rights Commission. Jauhiainen, M., … Hurri, H. (2003). Multidisciplinary
biopsychosocial rehabilitation for neck and shoulder pain among Emerson, E., & Baines, S. (2010). Health inequalities and people with
working age adults. Cochrane Database of Systematic Reviews. learning disabilities in the UK: 2010. Durham: Improving Health
Chung-Charlie, S. Y., Pollock, A., Campbell, T., Durward, B. R., & & Lives: Learning Disabilities Observatory.
Hagen, S. (2013). Cognitive rehabilitation for executive dysfunction Emerson, E., Madden, R., Graham, H., Llewellyn, G., Hatton, C.,
in adults with stroke or other adult non-progressive acquired brain & Robertson, J. (2011). The health of disabled people
damage. Cochrane Database of Systematic Review. and the social determinants of health. Public Health, 125(3),
Puhan, M. A., Gimeno, S. E., Scharplatz, M., Troosters, T., Walters, 145–147.
E. H., & Steurer, J. (2011). Pulmonary rehabilitation following Heslop, P., Blair, P., Fleming, P., Hoghton, M., Marriott, A., & Russ,
exacerbations of chronic obstructive pulmonary disease. Cochrane L. (2013). Confidential inquiry into premature deaths of people with
Database of Systematic Reviews. learning disabilities. Bristol: Norah Fry Research Centre, Univer-
Khan, F., Ng, L., Gonzalez, S., Hale, T., & Turner, S. L. (2008). sity of Bristol.
Multidisciplinary rehabilitation programmes following joint Hollins, S., & Tuffrey-Wijne, I. (2013). Meeting the needs of patients
replacement at the hip and knee in chronic arthropathy. Cochrane with learning disabilities. British Medical Journal, 346, doi: https://
Database of Systematic Reviews. doi.org/10.1136/bmj.f3421.
Khan, F., Amatya, B., Ng, L., Drummond, K., & Olver, J. (2013). Iacono, T., Bigby, C., Unsworth, C., Douglas, J., & Fitzpatrick, P.
Multidisciplinary rehabilitation after primary brain tumour treat- (2014). A systematic review of hospital experiences of people
ment. Cochrane Database of Systematic Reviews. with intellectual disability. BMC Health Services Research,
Thomson, L., Handoll-Helen, H. G., Cunningham, A. A., & Shaw, 14.
P. C. (2002). Physiotherapist-led programmes and interventions Kavanagh, A., Krnjacki, L., & Kelly, M. (2012). Disability and Health
for rehabilitation of anterior cruciate ligament, medial collateral Inequalities in Australia: Research Summary: Addressing the Social
ligament and meniscal injuries of the knee in adults. Cochrane and Economic Determinants of Mental and Physical Health. Victo-
Database of Systematic Reviews. rian Health Promotion Foundation, Carlton South, Vic. Available
Crowther, R., Marshall, M., Bond, G. R., & Huxley, P. (2001). at https://www.vichealth.vic.gov.au/media-and-resources/publica-
Vocational rehabilitation for people with severe mental illness. tions/disability-and- health-inequalities-in-australia.
Cochrane Database of Systematic Reviews. Lennox, N., Bain, C., & Rey-Conde, T. (2007). Effects of a compre-
Heran, B. S., Chen-Jenny, M. H., Ebrahim, S., Moxham, T., hensive health assessment programme for Australian adults with
Oldridge, N., … Rees, K. (2011). Exercise-based cardiac rehabili- intellectual disability: a cluster randomized trial. International
tation for coronary heart disease. Cochrane Database of Systematic Journal of Epidemiology, 36(1), 139–146.
Reviews. McKenzie, K., Milton, M., Smith, G., et al. Curr Dev Disord Rep
McGrath, J., & Hayes, R. L. (2000). Cognitive rehabilitation for (2016) 3: 104. https://doi.org/10.1007/s40474-016-0085-7.
people with schizophrenia and related conditions. Cochrane Data- Office for Disability Issues UK. (2010). Equality Act 2010 Guidance.
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Ostelo-Raymond, W. J. G., Costa-Leonardo, O. P., Maher, C. G., (2011). The impact of health checks for people with intellectual
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Reviews. 11.01436.x. [Epub 2011 Jul 5].
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Research Repository http://hdl.handle.net/1959.9/563533. Australian Family Physician, 40, 202–208.
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S E C TI ON 2 Manual of Clinical Practice
6
Children’s Health
RUTH MARGARET BLAND, HILARY LOCKHART PEARCE
The Child With a Fever What to Look for on Examination
What to Ask About the Child What to Do
What to Look for on Examination The Child Who Does Not Walk Aged 15 Months
Guideline What to Ask About the Child
What to Do What to Look for on Examination
Pyrexia of Unknown Origin What to Do
The Child With a Cough The Child With a Small Head
What to Ask About the Child What to Ask About the Child
What to Look for on Examination What to Look for on Examination
What to Do What to Do
The Child With Wheeze The Child With a Large Head
The Child With Constipation The Child With a Febrile Seizure
The Child With Abdominal Pain The Child With Seizures
The Child With Diarrhoea The Child With Headaches
The Child With a Murmur The Child With a Tic
The Child Who Collapses The Child With Dysuria and Abdominal Pain
What to Ask About the Child Guideline
What to Look for on Examination What to Ask About the Child
The Child With Chest Pain What to Do
What to Ask About the Child
37
38 se c t i o n 2 Manual of Clinical Practice
The Child With Bedwetting (Nocturnal Enuresis) What to Look for on Examination
What to Look for on Examination The Child Who Is Short
What to Do What to Ask About the Child
The Child With Protein in the Urine What to Look for on Examination
What to Look for on Examination The Child Who Is Tall
The Child With Blood in the Urine What to Look for on Examination
What to Look for on Examination The Child With Precocious Puberty
The Child With an Itchy Perineum What to Look for on Examination
What to Look for on Examination The Child With Excess Body Hair
The Child With Worms in the Stool What to Look for on Examination
What to Look for on Examination The Child With A Painful Limp
The Child With A Painful Penis What to Look for on Examination
What to Look for on Examination The Infant Who Vomits or Screams When Feeding
The Child With a Groin Swelling What to Look for on Examination
What to Look for on Examination The Infant Who Has Weight Faltering in the First
What to Do Few Weeks of Life
The Child With A Painful Scrotum What to Ask About the Child
What to Ask About the Child What to Look for on Examination
What to Look for on Examination What to Do
What to Do The Infant Who Has Weight Faltering After the First
The Child With a Non-blanching Rash Weeks of Life
The Child With Cervical Lymphadenopathy The Child Who Is Suspected of Having a Non-accidental Injury
The Child With Pica

The Child With a Fever • The challenge for the general practitioner (GP) is
identifying the child with a potentially serious bac-
• Fever is one of the most common reasons for seeking terial infection. If a child is acutely unwell then he
medical help. There are many causes of acute fever, and or she should be referred to hospital immediately,
it is important to take a thorough history and examine particularly if there is no obvious source for the
the child carefully. In most cases the fever is due to a viral fever, in which case the child will require a septic
illness that will be self-limiting. screen. Classic signs of particular infections, such as
CHAPTER 6 Children’s Health 39
the stiff neck and headache of meningitis, may not symptoms that may point to a cause for the fever, includ-
be present in infants who often present with fever, ing vomiting and diarrhoea (possible gastroenteritis),
pallor, and irritability, and so a GP needs to main- cough and coryzal symptoms (pneumonia or other respi-
tain a high index of suspicion for underlying serious ratory infection), pulling at an ear (possible otitis media).
infection. In an older child ask about pain passing urine (possible
urinary tract infection [UTI]), painful joint (possible
What to Ask About the Child septic arthritis), and seizures (possible meningitis or
herpes encephalitis).
• It is important to ask how long the fever has been present. Ask if other members of the family have been unwell and
A fever of more than 8 days duration, with no source, whether there has been any recent travel abroad.
suggests underlying pathology requiring further investi-
gation (see upcoming discussion). A parental history of
What to Look for on Examination
fever should always be taken seriously, even if a pyrexia
is not recorded by the examining doctor, as it is likely Guideline
that the child has been given paracetamol or a similar National Institute for Health and Care Excellence. (2013).
medication. Feverish illness in children. NICE clinical guideline 160.
• Ask about how long the fever has been present and Available from https://www.nice.org.uk/guidance/cg160/
whether the child is getting better or worse. resources/support-for-education-and-learning-educational
• In all cases of fever ask specifically about a rash—a -resource-traffic-light-table-pdf-189985789.
blanching rash is often present with viral infections but • The National Institute for Health and Care Excellence
a non-blanching rash may indicate an underlying serious (NICE) has produced a traffic light system for signs and
bacterial infection. symptoms that predict the risk of serious illness in chil-
• Explore how the child is feeding and whether he or she dren under 5 years of age which will be familiar to most
is refusing feeds or vomiting. Ask specifically about any UK general practitioners.
NICE Traffic light system for identifying the risk of serious illness in under 5s
High Risk: Refer Urgently Intermediate Risk: Refer Low Risk: Manage at Home
Colour: skin / Pale/mottled/ashen/blue Pallor reported by parent/carer Normal colour
lips/tongue
Activity No response to social cues Not responding normally to social Responds normally to social
Appears ill to health cues cues
professional No smile Content/smiles
Does not wake if roused Wakes only with prolonged Strong normal cry or not crying
Weak, high pitched or stimulation Stays awake or awakens
continuous cry Decreased activity quickly
Respiratory Grunting Nasal flaring
Respiratory rate (RR) >60 RR: 6–12 months >50 breaths/min;
breaths/min >12 months >40 breaths/min
Moderate/severe chest Oxygen saturation ≤95%
indrawing Crackles in chest
Circulation and Reduced skin turgor Tachycardia: <12 months: >160 beats/ Normal skin and eyes
hydration min; 12–24 months: >150 beats/ Moist mucous membranes
min; 2–5 years: >140 beats/min
Capillary return time ≥3 sec
Dry mucous membranes
Poor feeding in infants
Reduced urine output
Other ≤3 months: temp ≥38°C 3–6 months: temp ≥39°C None of the amber or red
Non-blanching rash Fever ≥5 days symptoms or signs
Bulging fontanelle Rigors
Neck stiffness Swelling of limb or joint
Status epilepticus Non–weight-bearing limb, not using
Focal neurologic signs an extremity
Focal seizure
• In addition to the clinical findings listed, examine the • Ask if there is a pattern to the coughing. Children with
child for specific signs that could indicate the source pertussis have paroxysmal spells of coughing but are
of fever, including examining for pus on the tonsils, generally well with no respiratory symptoms between
inflammation of the tympanic membranes, swelling and the episodes of spasmodic coughing. A child with asthma
inflammation of the joints, enlargement of the lymph may have exercise-induced, or nocturnal, cough. An
nodes, and tenderness in the abdomen. infant with gastro oesophageal reflux (GOR) may expe-
rience coughing after feeding or when prone, for example
What to Do when put into his or her cot to sleep, when the parents
might report a nighttime cough.
• The priority is to decide whether to refer the child for • Ask about the onset of the cough. If it started suddenly
further investigations, and whether this needs to be done with no prodromal symptoms, then ask about a history
urgently (see the NICE guidelines provided earlier). of choking and consider the possibility of an inhaled
• In older children who are not acutely unwell and in whom foreign body.
no source of acute fever can be found, the following inves- • Ask whether the cough sounds wet or dry. The cough
tigations are useful: urinalysis and urine culture, full blood associated with asthma sounds dry, while the cough asso-
count (FBC), C-reactive protein (CRP), stool culture. ciated with a respiratory infection sounds wet and may
• A normal white cell count and CRP, and a negative be productive of sputum. A barking cough associated
urinalysis, are reassuring; and it is unlikely that there is with stridor is typical of croup, while a harsh paroxysmal
any serious pathology underlying the fever. cough is suggestive of pertussis.
• It is not advisable to prescribe antibiotics to children • Check whether there are associated symptoms that
without a source for the fever. Most children who are might point to the aetiology of the cough (for
not acutely unwell with fever do not have underlying example, whether the child has fever, nasal discharge,
bacteraemia. increased work of breathing, reduced exercise toler-
• If an unwell child presents with a non-blanching rash and ance, anorexia, fatigue, or weight loss). If the child
fever, suspect meningococcal disease and give intramus- has a persistent cough associated with fever and night
cular (IM) antibiotics before urgent transfer to hospital. sweats, ask whether the child has been exposed to
tuberculosis (TB).
Pyrexia of Unknown Origin • For infants and young children, ask whether the caregiv-
ers have noted any episodes of apnoea, which may be
A pyrexia of unknown origin (PUO) is a fever of 8 days or associated with pertussis, bronchiolitis, and GOR.
more in a child in whom there is no source of fever after • Sinusitis may present with persistent cough and tender-
initial investigations have been done. These children should ness over the sinuses, and the child may present with
be referred for further investigation. The list of causes is vast, facial pain.
including infectious bacterial diseases (Lyme disease, salmo- • Ask about feeding, appetite, and weight gain. In infants
nella); viral illnesses (Epstein-Barr virus); fungal and para- vomiting and cough may suggest a diagnosis of gastroo-
sitic diseases; connective tissue diseases; Kawasaki disease esophageal reflux.
and malignancy. • In the family history, specifically ask about atopy, asthma,
or a TB contact.
The Child With a Cough • Take a child’s immunization status, in particular bacillus
Calmette-Guérin (BCG) and pertussis.
• Cough is a common symptom in children. It can be
acute (<3 weeks) or chronic (3–12 weeks). In infants What to Look for on Examination
think about pertussis and bronchiolitis. Most acute
coughs in older children are caused by viruses, but if the • Assess if the child is acutely unwell (refer to page 39), in
child is unwell with fever and tachypnoea then pneumo- particular looking for cyanosis or pallor; signs of respira-
nia must be excluded. tory distress include nasal flaring, grunting, chest indraw-
• Causes of chronic cough include asthma, pertussis, ing, or an increased respiratory rate (>50 breaths/min in
viruses including adenovirus, bacteria including myco- children 0–12 months; >40 breaths/min in children >12
plasma, and postnasal drip months of age). Refer urgently as appropriate if these
signs are present.
What to Ask About the Child • In young children, most information will be gained by
observation. However, it is useful to auscultate the chest
• Ask about the duration of the cough to find out if it is for crackles and wheeze if the child is not distressed and
acute or chronic. Most acute coughs are caused by viruses crying. If there are crackles, determine whether they are
and will be self-limiting. However, viruses such as adeno- generalized (e.g., in an infant with bronchiolitis) or local-
viruses can cause coughs that last up to 3 months. ized (e.g., in a child with lobar pneumonia).
• Examine the upper respiratory tract, including the or exercise-induced symptoms. A family, or personal,
tonsils, ears, and nose. history of atopy may also be helpful.
• Palpate the face over the region of the sinuses, as tender-
ness over these areas may suggest sinusitis. What to Ask About the Child
• Look for evidence of a chronic respiratory illness
including a chest deformity (e.g., sulci, increased anterior- • Ask about the pattern and frequency of episodes of
posterior diameter) and finger clubbing. wheeze. Is the wheeze frequent and episodic with defi-
• Weigh the child and plot this on an appropriate growth nite triggers, such as exercise or cold weather, suggestive
chart and compare with previous measurements to assess of asthma? Or does the wheeze only occur with upper
growth. Failure to thrive associated with a cough needs respiratory tract infections and is therefore more likely
further investigation. to be a viral-induced wheeze, particularly in a younger
child?
What to Do • Ask if the child has associated symptoms, including
a dry cough, respiratory symptoms with exercise, dis-
• Have a low threshold for referring infants with a clinical turbed sleep, or snoring. Children with asthma may have
diagnosis of a lower respiratory illness, bronchiolitis, or a nocturnal or early morning cough. A wet-sounding
pertussis to secondary care if they are ex-preterm (as they cough is less likely to be asthma.
can deteriorate quickly), have had apnoeic episodes with • Ask about family history. A diagnosis of asthma is more
this illness, or have been admitted to hospital previously likely if there is a family or personal history of atopy,
with a respiratory illness. For children over 1 year of age including eczema, hay fever, or other allergies.
with pneumonia, if they are not acutely unwell they • Ask if the wheeze has been present since birth; and if so,
may be managed at home with an oral antibiotic and consider a diagnosis of tracheobronchomalacia.
antipyretic. • Check if the child was born prematurely and if he or
• A child with a sudden onset of cough and a possible she was intubated and ventilated. Such children may be
history of choking should be referred urgently for inves- more likely to deteriorate quickly with respiratory ill-
tigation of an inhaled foreign body. These children may nesses and may need referral to secondary care more
also have stridor. promptly than children born at term with no neonatal
• If a child has a persistent nocturnal cough that you complications.
think may be asthma, give a trial of an inhaled steroid. • Always ask whether the child is exposed to cigarette
If the child does not respond to this, then refer to smoke and check his or her growth.
secondary care.
• Refer all children with a persistent cough for more than What to Look for on Examination
1 month who are not improving.
• Refer all children with a cough and failure to thrive • Assess if the child is acutely unwell, in particular looking
for further investigations, which may include imaging, for cyanosis or pallor, and signs of respiratory distress.
assessment of immune function, and bronchoscopy. • In a child with known asthma, look out for the so-called
• In infants with a possible diagnosis of gastrooesophageal silent chest with no wheeze. This is a dangerous sign as
reflux try an antireflux medication. it indicates little air movement.
• Be aware of children who are so breathless that they
The Child With Wheeze cannot talk in sentences. This is a dangerous sign indicat-
ing severe respiratory distress. Children who are agitated
• Wheeze within the context of a viral illness is common or exhausted should also be referred urgently.
in pre-school children. One third of children will have • Auscultate for wheeze. A generalized wheeze is present
at least one episode of wheeze before they are 3 years old. in asthma and in infants with bronchiolitis. Also check
The diagnosis of asthma, in children under the age of 5, for crackles, which may suggest lower respiratory tract
is a clinical one. In older children, asthma is diagnosed infection.
if more than one of the following symptoms is present: • If the child is old enough measure a peak expiratory flow
recurrent wheeze, cough, difficulty breathing, and chest and compare this to the reference charts and, if available,
tightness. The diagnosis should be supported with spi- to previous measurements of the child.
rometry in children old enough to comply with assess- • Look for evidence of a chronic respiratory condition,
ment (usually around the age of 5). including a chest deformity (e.g., sulci) and finger clubbing.
• A trial of a β-2 agonist may demonstrate reversibil- • Weigh the child, plot on an appropriate growth chart,
ity of bronchospasm, but in children less than 2 years and compare with previous measurements. Failure to
of age the response to bronchodilator therapy is not thrive associated with a cough or wheeze needs further
always consistent. The diagnosis should be considered investigation; also consider referring these children to
if there are other features, including nocturnal cough secondary care.
What to Do diarrhoea worse. However, overflow diarrhoea will not

resolve until any impacted faeces are passed.
• Management of acute wheeze in a child who is unwell • In the past history, ask whether the child passed meco-
includes oxygen therapy, β-2 agonist via a nebulizer or nium within the first 48 hours after delivery. Failure to
multidosing with an inhaler through a spacing device, pass meconium, or constipation beginning in the first
oral steroids, and referral to secondary care if indicated. few weeks of life, may indicate an underlying pathology
For longer term management of asthma, refer to guide- (e.g., Hirschsprung disease). However, it is important to
lines, including the British Guideline on the Manage- stress that most cases of constipation are idiopathic and
ment of Asthma, which provides a stepwise approach to the child will require no investigations.
the medical management of asthma. • As with all children, check that the child is thriving and
• For children with viral-induced wheeze who are well otherwise well.
between episodes, treatment with a β-2 agonist for
acute symptoms may be beneficial. However, β-2 ago- What to Look for on Examination
nists are often ineffective for children under the age of
1 year. • Assess if the child is acutely unwell. A distended abdomen,
• For all children it is useful to provide advice to the fami- especially in a child who is also vomiting, requires urgent
lies about related triggers, including avoidance of smoke, referral to secondary care. Palpate the abdomen for faecal
pollen, and animal dander where appropriate. masses, which are most often felt above the pelvic rim in
• Refer children to secondary care if they have wheeze and children with constipation. Check the child’s height and
are not growing well, have recurrent chest infections, or weight and plot on a chart, comparing with previous
have a chronic cough. measurements. A child who is failing to thrive with con-
stipation should be referred to secondary care for exclu-
The Child With Constipation sion of other disorders, including coeliac disease and
hypothyroidism.
• Constipation is a very common reason for referral to • There is no need to perform a rectal examination on a
secondary care. Parents are often concerned that the child with constipation in primary care. This will cause
child has underlying pathology and often come to hos- distress to the child and not help with the diagnosis.
pital expecting that investigations, including imaging, However, it is useful to check the appearance of the
will be carried out. anus externally. In a young infant, check that the anus
• More than 90% of cases of constipation are idiopathic is patent, as in rare cases an imperforate anus may have
(i.e., have no underlying anatomical or physiological been missed on neonatal examination. Check if there is
cause). It is important to provide a clear explanation to an anal fissure which may be the cause of bleeding per
the parent and child (where appropriate), implement a rectum, or multiple fissures or a fistula which may be
management plan, and arrange regular follow-up to suggestive of other pathology such as Crohn’s disease.
ensure that the child responds to treatment. Bruising around the anus may indicate possible child
abuse.
What to Ask About the Child • Neuromuscular causes of constipation are rare, but
check that there is no obvious deformity of the spine,
• Ask about the frequency and consistency of the stools (use to rule out a sacral dimple with no visible base, and
the Bristol stool chart to help children and caregivers to that the child does not have abnormal tone, power, gait,
identify the type of stools they are passing). Fewer than or reflexes. In a toddler check for delayed walking that
three formed stools per week, and stools that are hard or could indicate an underlying neuromuscular problem.
like rabbit droppings, are features of constipation.
• Explore whether the child has abdominal pain while What to Do
passing stool and between episodes of stooling. Children
with constipation often experience regular lower abdom- • Investigations are not normally required and idiopathic
inal pain and pain on defecation particularly if the stools constipation can usually be managed in primary care.
are large and hard. The key points of management are reassurance, a clear
• Ask if there has ever been blood coating the stool, sug- management plan, and follow-up to check response to
gesting a fissure caused by constipation. treatment.
• Ask about episodes of diarrhoea. Sometimes children with • It is important to explain that constipation is common
constipation present with diarrhoea, but this is overflow and that diet alone is unlikely to solve the problem,
diarrhoea, passed with no sensation and often offensive particularly in the short term. Explain the importance of
to smell. Caregivers will often report that the child is lazy starting a laxative to soften the stools, with the goal of
and failing to go to the toilet in time, and caregivers are passing a soft stool at least once per day. Explain that it
often concerned about treating the child with laxatives is preferable to have stools that are too loose rather than
(see upcoming discussion), as they feel it will make the too hard at this stage.
• Explain that if a child is soiling (overflow diarrhoea), this • Ask specifically about vomiting. Bilious vomiting usually
behaviour is not deliberate and should not be punished. has a surgical cause and children should be referred to
It is often a relief to caregivers and children to under- secondary care. Children with an acute surgical cause for
stand that overflow diarrhoea will stop once the constipa- their vomiting are usually unwell (e.g., a child with acute
tion resolves. appendicitis will usually present with listlessness and
• Treatment includes disimpaction if this is required anorexia in addition to abdominal pain).
and then regular treatment with appropriate laxatives. • Ask about the stools and whether defecation is associated
Paediatric Movicol (a macrogol) is the laxative of choice with pain. Diarrhoea, blood, or mucus per rectum may
for children. Clear guidance on doses for disimpaction indicate underlying pathology (e.g., inflammatory bowel
and maintenance are given in the British National For- disease). However, constipation with a fissure may also
mulary for Children and in the NICE guidelines. cause blood that coats the stools and is a common cause
• Give clear instructions on how to take the laxative, of abdominal pain.
explaining that it should not be stopped and started, and • Check whether the child has other symptoms that
that some abdominal pain and loose stools will be expe- may indicate underlying pathology. For example, mouth
rienced during the first days of administration. Caregiv- ulcers, joint pains, and anal skin tags are associated with
ers often stop giving laxatives as the child passes loose Crohn’s disease.
stools, but explain that this is to be expected in the first • Explore for factors in the social history that may be
few days. sources of anxiety, including problems at school, difficul-
• Children are often fearful of sitting on the toilet and ties with parents, parental separation or divorce.
attempting to pass stool. Once the stools are soft, encour- • Ask about dysuria, which may suggest UTI.
age the child to sit on the toilet for 5 to 10 minutes after
breakfast and evening meal. Giving a child balloons to What to Look for on Examination
blow up whilst on the toilet, and providing a foot stool
for comfort if unable to reach the floor, are strategies to • Examine for an acute abdomen or surgical cause for
encourage pushing. abdominal pain. Check for acute/rebound tenderness on
• Arrange follow-up for the child to check on response to palpation, a distended abdomen, fever, lethargy, and a
treatment and to suggest modifications in laxative doses furred tongue.
as required. If the child has faecal impaction then arrange • Functional abdominal pain is nonspecific. Children
follow-up after 1 week. Check adequate fluid intake. often vaguely point to their umbilicus when trying to
localize the pain and do not usually complain of tender-
The Child With Abdominal Pain ness when their abdomen is palpated.
• Measure and plot the child’s weight and height on a
• At least 10% of schoolchildren will experience abdomi- growth chart and compare to previous measurements. A
nal pain regularly. There are many causes and the chal- child with abdominal pain associated with weight falter-
lenge is to identify the child with underlying pathology ing needs further investigations to rule out conditions
and to avoid unnecessary tests on children with func- such as inflammatory bowel disease and coeliac disease.
tional abdominal pain who do not require them. • Check the anus externally for skin tags, fissures, fistulae,
and ulceration (there is no need to do a PR [rectal]
What to Ask About the Child exam). A fissure may be present with constipation. Skin
tags, multiple fissures, and fistulae may suggest inflam-
• Ask at what age the pain started. Take seriously abdomi- matory bowel disease, and should be further investigated.
nal pain commencing in young children (<5 years), in • Finger clubbing in a child with abdominal pain is sug-
whom constipation has been excluded. gestive of underlying pathology, including inflamma-
• Explore about the frequency and pattern of the pain, tory bowel disease. Similarly, mouth ulcers may indicate
bearing in mind that children with underlying pathology Crohn’s disease (but may also be an incidental finding).
will experience pain irrespective of play or other activities.
• Ask if the pain wakes the child at night, as functional What to Do
pain does not tend to cause nocturnal wakening.
• Explore if there is a relationship to eating, as Helicobacter • If you are concerned that there is underlying pathology
pylori disease may be associated with upper abdominal the following investigations may be helpful in distin-
and retrosternal pain on eating. guishing between functional and nonfunctional pain:
• Ask if any particular foods aggravate the pain. Pain can • Faecal calprotectin. Levels over 200 µg/g may be sug-
occur in coeliac disease after eating gluten. gestive of inflammatory bowel disease and these chil-
• Ask if the pain is associated with pallor, nausea, or a dren should be referred.
family history of migraine. Children can get abdominal • Blood tests: FBC, ferritin, inflammatory markers
migraine that presents with nonspecific abdominal pain (CRP, erythrocyte sedimentation rate [ESR]), liver
but not necessarily headache. function tests, coeliac antibody screen.
ALGrawany
• Faecal H. pylori antigen may be helpful in children with • Check whether the child has finger clubbing suggestive
upper abdominal/retrosternal pain and pain on eating, of underlying chronic pathology.
or those with a family history of H. pylori disease. • Check the anus externally for skin tags, fissures, fistulae,
• Urine culture to exclude UTI. and ulceration (there is no need to do a PR exam), par-
• Normal investigations are often reassuring to parents. ticularly if you are suspecting a chronic underlying cause.
• If the child is constipated manage appropriately. Drug A fissure may be present with constipation. Skin tags,
treatment may be helpful in the following conditions: multiple fissures, and fistulae may suggest inflammatory
serotonin antagonists in abdominal migraine and mebev- bowel disease and should be further investigated.
erine in irritable bowel syndrome. For debilitating func- • Check the mouth for ulcers, which may indicate under-
tional abdominal pain psychology input may be required. lying pathology (e.g., Crohn’s disease) but may also be
an incidental finding.
The Child With Diarrhoea
What to Do
• It is important to distinguish between acute (<2 weeks)
and chronic (>2 weeks) diarrhoea. • Acutely unwell children should be referred.
• Acute diarrhoea is often associated with vomiting and is • If the child can be managed at home, consider sending
usually caused by viruses, including rotavirus, adenovi- stools for culture if there is blood in the stool, or the
rus, and calicivirus. Rarely acute diarrhoea is caused by child has travelled abroad recently.
bacteria (Salmonella, Shigella, Yersinia, Campylobacter) or • For children with bloody diarrhoea it is wise to check
protozoa (Giardia and Cryptosporidium). the blood count, electrolytes, glucose and renal function.
Antibiotics may be required in the following specific
What to Ask About the Child circumstances to treat diarrhoea:
• If Giardia is isolated from the stool, give metronida-
• Ask about the duration of the diarrhoea and distinguish zole for 3 days. Asymptomatic patients do not require
between acute and chronic diarrhoea. treatment.
• Enquire whether the child has vomiting, as gastroenteri- • If Campylobacter is isolated from the stool, give eryth-
tis causes acute diarrhoea and vomiting. romycin if there is systemic upset or persistent blood
• Specifically ask whether the child has blood in the diar- in the stools, although many cases will resolve without
rhoea. This is found in infections (also see haemolytic antibiotic treatment (consult with your local micro-
uraemic syndrome [HUS]) and in inflammatory bowel biologist if in doubt).
disease if the diarrhoea is chronic. • If Salmonella or Shigella are isolated from the stool,
• Ask about recent travel abroad as this may indicate the consider referral to secondary care for treatment and
aetiology of the diarrhoea (e.g., Salmonella). management, particularly if the child is systemically
• Ask if the child lives on a farm or has had contact with unwell or is under 1 year old.
animals, and consider whether the child could have • If any of the following organisms are cultured from
Campylobacter. the stool, notify the environmental health department:
• Ask whether the child has ongoing abdominal pain Shigella, Salmonella, Giardia, Campylobacter, Cryptospo-
or other symptoms suggestive of inflammatory bowel ridium, Escherichia coli 0157. Discuss children with E.
disease or coeliac disease. If the child has constipation, coli 0157 with secondary care, due to its association
consider whether the diarrhoea is overflow diarrhoea. with HUS.
• Ask about the child’s diet, as fruit juices, diluting juice, • In cases of chronic diarrhoea and failure to thrive or
fizzy drinks, and sugar-free chewing gum may cause other symptoms, consider further investigations and/or
osmotic diarrhoea. referral. Inflammatory bowel disease and coeliac disease
may present with diarrhoea in children.
What to Look for on Examination • If a dietary cause for osmotic diarrhoea has been found,
suggest relevant dietary modifications.
• Assess whether the child is acutely unwell and in need • It is important to provide appropriate advice for feeding
of acute admission for treatment of severe dehydration a child during diarrhoea. Many caregivers may think that
or severe underlying infection. it is important to starve the child by providing only
• Check for petechiae, which may be present in HUS and water, but this is not helpful. Practical tips about feeding
sepsis. include the following:
• Examine the abdomen for tenderness, masses, and • For breastfed infants, encourage continued breast-
organomegaly. feeding which will provide both calories and
• Plot the child’s current weight and height on a growth chart water.
and compare with previous measurements. (Remember, if • For non-breastfeeding infants, it is rarely necessary to
the child is dehydrated his or her weight will be lower than withdraw feeds as this may delay recovery.
normal.) • It is rarely necessary to restrict lactose in the diet.
• Oral rehydration solution (ORS) may be prescribed • The most common murmur will be an innocent one. On
in moderate dehydration for replacement of fluids auscultation an innocent murmur will change with the
during the first 4 hours. After this, recommence child’s position (often being loudest when the child is
normal feeds, with ORS given after each loose stool lying down), have a short duration, will not radiate to
at a dose of 10 mL/kg body weight. Do not keep the precordium or back, have a soft quality often
children on ORS for prolonged periods of time, as described as “musical,” and be systolic with no associated
they will become ketotic due to low calorie intake and clicks or gallops.
may then experience abdominal pain and deteriorate
clinically. What to Do
The Child With a Murmur • Refer urgently all children with a murmur who have cya-
nosis or signs of heart failure (tachycardia, sweating, poor
• Most murmurs are innocent. feeding, tachypnoea, enlarged liver, or failure to thrive).
• The majority of pathological heart murmurs are picked • Also refer urgently all children noted to have a murmur
up in infancy or have been found on an antenatal scan. in the first 48 hours of life.
As many infants are now discharged from hospital a few • Refer children whose murmur is not consistent with
hours after birth, they may present to primary care in the findings of an innocent murmur, including a widely
the first few days of life with either heart failure or cya- radiating or particularly loud murmur.
nosis. These children require urgent referral to hospital • Refer those who have cardiac symptoms, including syncope,
for investigation and management. chest pain, or concerns regarding growth.
• Murmurs are often picked up at the routine 6-week baby • If you think the murmur is an innocent one:
check. These murmurs should also be referred to second- 1. document your findings;
ary care for assessment. 2. arrange to review the child again to reassess the
• The key features of an innocent murmur are the seven murmur (if the child is currently unwell, arrange
Ss (Bronzetti & Corzani, 2010): review once the acute illness has resolved);
1. Sensitive (change with position and respiration) 3. there is no need to refer to secondary care if the child
2. Short duration is well and the murmur has the characteristics of an
3. Single (no associated clicks or gallops) innocent murmur.
4. Small (nonradiating)
5. Soft (low amplitude) The Child Who Collapses
6. Sweet (not harsh, but musical)
7. Systolic • Syncope is a temporary loss of consciousness resulting in
collapse, caused by reduction in oxygenation to the brain.
What to Ask About the Child There are a number of causes, the most common of which
are vasovagal episodes (fainting) and reflex anoxic syncope
• Ask about cardiac symptoms, including chest pain, syncope, (RAS). RAS is most common in the toddler age group.
impaired exercise tolerance, colour changes, and cyanosis. • Rare cardiac causes of syncope include arrhythmias (long
• Ask whether the murmur has ever been noted previously. QT syndrome, Wolff-Parkinson-White syndrome) and
• Enquire about the child’s feeding, appetite, and growth. cardiac abnormalities (aortic or pulmonary stenosis). As
All children who are failing to thrive with a murmur syncope may result in abnormal movements, including
should be further investigated. stiffening of the body or limbs, convulsive jerking, or
• Check if there is a family history of cardiac disease. drowsiness following the event, syncope may sometimes
Children have an increased risk of congenital heart be confused for epilepsy.
disease if they have a first degree relative with a history • Breath holding attacks also occur in the toddler age
of congenital heart disease. group. During these events the child gets cross, starts to
cry, holds his or her breath, turns blue, and collapses.
What to Look for on Examination The cyanosis is due to the glottis being held closed. These
events are respiratory in origin and an electrocardiogram
• Assess if the child is acutely unwell. (ECG) taken at the time would not show asystole.
• Check for cyanosis and pallor and whether there are signs
or symptoms that could indicate heart failure, including What to Ask About the Child
tachycardia, an enlarged liver, sweating, and difficulties
with feeding (particularly in infants). • Obtain a detailed history of the event, including what
• Feel for the femoral pulses. Decreased or absent femoral happened before, during, and after the collapse. Try to
pulses and a short systolic murmur may indicate coarcta- obtain a history from a witness and, if old enough, the
tion of the aorta, a murmur that may not be picked up child. In vasovagal episodes the child will often have pro-
in early childhood. dromal features, including sweating, dizziness, and pallor.
• Ask about the frequency of episodes and, if the child • The most common cause is a musculoskeletal problem;
has had more than one episode, whether the episodes but also consider respiratory causes including asthma and
have similar features. Enquire whether there are specific pneumonia, gastrointestinal causes including oesophagi-
triggers to the events. Breath-holding attacks occur when tis, and anxiety associated with hyperventilation.
a child is crying; RAS occurs in response to a noxious
stimulus. Events during exercise or whilst supine are What to Ask About the Child
unusual in benign cases of syncope and children with
these features should be referred to secondary care. • Ask about the duration, frequency, location, and descrip-
• Check if there is a history of sudden death in a family tion of the pain.
member under 30 years of age or a family history of • Enquire whether the pain occurs at rest, on exertion, or
sudden infant death syndrome, and refer children with during the night.
a positive family history for further assessment. • Check whether there are associated symptoms, including
• If possible, ask the family to capture one of these events syncope, pallor, sweating, palpitations, all of which are
on video. concerning.
• Ask whether there are respiratory or gastrointestinal
What to Look for on Examination symptoms, including asthma or GOR.
• Check whether analgesia helps the pain. Antiinflamma-
Usually the child will present with a history of collapse, tory medication would help ease pain due to costochon-
rather than immediately after the collapse. In most cases the dritis or musculoskeletal problems.
child will have a normal examination. However: • If the child has been partaking in new exercises, lifting,
1. examine the cardiovascular system, including blood pres- or if they carry heavy books to school, consider a possible
sure (take whilst sitting and standing and look for a musculoskeletal cause.
postural drop in pressure, which may be the cause of a • Ask if there is a family history of sudden death in young
vasovagal episode); adults as there may be a history of hypertrophic obstruc-
2. examine the neurological system, looking for cranial nerve tive cardiomyopathy or long QT syndrome.
abnormalities, asymmetry of tone or power, or an abnor- • Check if the child has a history of Kawasaki disease,
mal or unstable gait suggestive of an underlying neuro- as this may result in later cardiac problems including a
logic condition, which would be a rare cause of collapse. coronary aneurysm.
In RAS, check the conjunctivae and palmar creases for
signs of iron deficiency anemia. What to Look for on Examination
What to Do • Assess for local tenderness found in costochondritis and

musculoskeletal problems.
• All children with syncope should have an ECG to rule out a • Examine the cardiovascular system. Check the peripheral
rare but potentially fatal cardiac problem. Ideally the ECG pulses and auscultate for a murmur. If possible check the
should be read by an experienced paediatrician. The excep- blood pressure.
tion is classic breath-holding attacks when the child is seen to • Examine the respiratory and gastrointestinal systems to
cry, hold his or her breath, and turn blue. An electroencepha- look for alternative causes of chest pain.
logram (EEG) is not usually required in cases of syncope.
• A clear explanation and reassurance is required for parents What to Do
and children. Prevention of vasovagal episodes includes
the following strategies: • Musculoskeletal causes should be managed with rest and
• Children can learn to recognize the prodromal symp- antiinflammatory medication.
toms and sit with the arms folded and legs crossed to • Indications for immediate referral to a paediatric cardi-
maintain their blood pressure and prevent syncope. ologist include:
• Suggest increasing fluids and dietary salt. • chest pain on exertion;
• It is difficult to prevent RAS or breath-holding attacks. • chest pain with palpitations;
Sometimes advising the parents to blow gently on the • chest pain with syncope;
face of a child about to have a breath holding attack may • a cardiac abnormality found on examination (e.g., a
abort the event. murmur);
• a family history of sudden death or cardiac problems
The Child With Chest Pain in a young adult.
• If features suggest a diagnosis of gastrooesophageal reflux,
• Chest pain is a common reason for 10- to 16-year-olds a trial of an acid-blocking drug may be worthwhile.
to attend the emergency department. It can cause signifi- • Treat respiratory conditions as appropriate.
cant concern to parents and children but in the majority • Manage anxiety-induced episodes with behavioural
of cases is noncardiac in origin. techniques.
• Microcephaly can be caused by a variety of genetic and

The Child Who Does Not Walk Aged environmental causes. It is important to distinguish
15 Months between primary microcephaly where an abnormal OFC
has been present since birth (corrected appropriately for
• Delays in walking may be due to a variety of causes gestational age and weight and length) and secondary or
ranging from simple bottom shuffling to cerebral palsy acquired microcephaly due to deceleration in the growth
(children have spasticity) and neuromuscular disorders of the brain after birth.
including Duchenne muscular dystrophy and spinal
muscular atrophy (children have weakness). What to Ask About the Child
What to Ask About the Child • Ask about the pregnancy and whether the mother was well,
whether she had any infections (e.g., cytomegalovirus,
• Ask about the birth history, including maternal health rubella, toxoplasmosis), and whether she took any drugs
during pregnancy and prematurity. Check the records for during pregnancy. Consider whether there could have
the Apgar scores. Cerebral palsy has many causes but the been a prenatal insult.
insult is usually prenatal. • Ask about the delivery, including the type of delivery and
• Ask if the child appears unwell or in pain and whether the Apgar scores, and consider whether there could have
there is a local cause for the delayed walking (e.g., a been an insult during delivery.
swollen or painful joint). • Enquire whether the parents are consanguineous and
• Assess whether the child has met other gross develop- consider whether there could be a metabolic or genetic
mental milestones: cause (microcephaly may be autosomal recessive).
• Was reaching/grasping delayed (>5 months)? • Ask whether the infant was unwell during the first 6
• Was sitting delayed (>7 months)? weeks of life (e.g., with a serious bacterial infection such
• Did the child dislike lying prone (more likely in as meningitis) and consider whether there has been a
bottom shufflers)? postnatal insult.
• Did the child crawl (bottom shufflers tend not to • Ask about appropriate development milestones: Does
crawl)? the child smile (at 6 weeks), reach/grasp (by 5 months),
• Ask if the child bottom shuffles and if there is a family sit (by 7 months)?
history of delayed walking or bottom shuffling.
• Take careful measurements of the weight, length, and
• Look for dysmorphic features and assess the general OFC and plot on appropriate charts.
development of the child, including vision, hearing, • Measure the OFC of the parents and any siblings and
social, and fine motor skills. plot on appropriate charts.
• Check the child’s truncal tone: Pick up the child under • Look for any dysmorphic features associated with micro-
the arms. If the child slides through your hands (like a cephaly, including primordial dwarfism and Dubowitz
rag doll), then he or she has truncal hypotonia. syndrome.
• Check for asymmetry in tone or power in the legs or • Check if the child is hypertonic or hypotonic.
arms (this may point to an underlying neuromuscular
problem). What to Do
• Check for tenderness on palpation of the legs or joints.
• All children with microcephaly should be referred to
What to Do secondary care for further assessment.
• For children who are bottom shufflers, reassure the The Child With a Large Head
parents that the child will walk eventually.
• If you have other concerns regarding the child’s neuro- • Macrocephaly is defined as an occipital-frontal circumfer-
logic examination or development then refer for further ence (OFC) more than 2 standard deviations above the
assessment. A physiotherapy assessment is often useful if mean.
you are not sure whether there is an underlying problem. • Macrocephaly does not always indicate a large brain
(megalencephaly) (e.g., children with hydrocephalus will
The Child With a Small Head have a large head but their brain in not enlarged).
• It is important to distinguish between an infant who has
• Microcephaly is defined as an occipital-frontal circumfer- always had a large head, whose OFC is tracking appro-
ence (OFC) more than 2 standard deviations below the priately without deviating upward away from previous
mean. This indicates a small brain, or microcephaly. measurements, and an infant whose OFC was on a lower
centile and is now accelerating and crossing centiles in secondary care for further management and investigation
an upward direction. of the source of the fever.
• A large head may be familial. Other causes include con- • First febrile seizures do not usually occur in those above
genital problems, infections, subdural bleeds, metabolic 6 years of age.
storage and degenerative diseases, and cranioskeletal • The incidence of first febrile seizures is around 1 in
dysplasias. 20 children; approximately one third of children who
have a febrile seizure will go on to have another febrile
What to Ask About the Child seizure.
• Most commonly the duration of febrile seizures ranges
• Ask about the child’s development and whether normal from a few seconds to 15 minutes.
milestones have been reached.
• Check if there are any signs of raised intracranial pressure What to Ask About the Child
including poor feeding or irritability.
• Explore whether other members of the family have large • Ask for a description of the seizure. Febrile seizures may
heads. involve a variety of abnormal movements or posturing.
• Enquire whether the child has had any serious illnesses Children with focal seizures should be referred to hospital.
(e.g., meningitis). • Ask about the length of the seizure and the recovery
• Ask if the parents have any concerns about the child. time. Any child who has not regained consciousness, or
whose conscious level is not clearly improving after 30
What to Look for on Examination minutes should be assumed to have increased intracranial
pressure and referred for further management.
• Take careful measurements of the child’s weight, length, • Ask about a family history of epilepsy or febrile seizures.
and OFC and plot on appropriate charts. If one or both parents have a history of febrile seizures the
• If possible measure the parents’ OFC and plot on an risk of recurrent febrile seizures for the child increases.
appropriate chart (plot at age 18 years on female and • Try to find a source of the fever by asking about respira-
male charts as appropriate). tory symptoms, vomiting, diarrhoea, rash, and illness in
• Assess the child’s development and whether this is age other family members.
appropriate. In familial macrocephaly the child’s devel-
opment will be normal. What to Look for on Examination
• Palpate the anterior and posterior fontanelles. The ante-
rior fontanelle usually closes by 12–18 months and the • Refer any child at high or intermediate risk of a serious
posterior fontanelle by 2 months. If they are open beyond illness (see NICE traffic light system for fever in children
this, consider whether the child has raised intracranial <5 years earlier in the chapter).
pressure. • Take the temperature and give an antipyretic (either
• Check whether the child’s anterior fontanelle is tense and orally or per rectum).
bulging, suggesting raised intracranial pressure. • Look for a source of the infection. Specifically examine
• Check whether the child has any depigmented or pig- the throat, ears, chest, abdomen, and skin.
mented patches, as neurofibromatosis and tuberous scle-
rosis are both causes of macrocephaly. What to Do
What to Do • The first priority is to manage the seizure: check airway,

breathing, and circulation. If the seizure has lasted for
• A normally developed child whose OFC is tracking par- more than 5 minutes give buccal midazolam or rectal
allel to the 99th centile without deviating in an upward diazepam (for doses, see the BNF for Children) and refer
direction, and with a family history of macrocephaly, does the child to hospital.
not need further investigations. Reassure the parents. • You will often be consulted after the seizure and your
• A child with signs suggesting raised intracranial pressure, priority is to determine the cause of, and treat where
or an OFC crossing centiles in an upward direction, appropriate, the underlying cause of the fever. See “The
should be referred immediately. Child with a Fever” to guide whether the child should
be referred to hospital.
The Child With a Febrile Seizure • For children being managed at home reassure the parents
that:
• Febrile seizures occur when there is a rapid increase in • febrile seizures are common;
the temperature of a young child, usually between the • most children will not have another one;
ages of 1 and 3 years. • longitudinal studies show that children with febrile
• Children who have a seizure below 6 months of age, even seizures have normal school achievement, comparable
if this is associated with fever, should be referred to with their siblings who have not had febrile seizures;
• the majority of children with febrile seizures do not • If the child has a fever or looks unwell, then assess for
go on to develop epilepsy. signs of meningitis or another cause of febrile illness that
• Refer the child to hospital if there are unusual features may have precipitated the seizure.
associated with the seizure, including a prolonged dura- • Examine the tone and power and check for symmetry.
tion (>15 minutes), asymmetrical movements, a long • Examine for signs of raised intracranial pressure.
time (>30 minutes) to return to normal conscious level.
What to Do
The Child With Seizures
• If the child is having a seizure, your first priority is
• There are numerous forms of epilepsy, including general- to manage the event. Check airway, breathing, and
ized seizures, focal seizures, absence seizures, infantile circulation. If the seizure has lasted for more than 5
spasms, and myoclonic seizures. minutes, give buccal midazolam or rectal diazepam (for
• In all children with a seizure, a detailed description of doses, see the BNF for Children) and refer the child to
the episode is critical to making a diagnosis. If possible, hospital.
ask the caregivers to capture the event on video. Provid- • You will often be consulted after the seizure, and your
ing a detailed description helps to classify the seizure and priority is to determine whether the child needs to be
determine the drugs to use. Approximately 40% of chil- urgently referred to hospital (e.g., child not regained
dren presenting with a first seizure have nonepileptic consciousness, signs of a serious bacterial infection,
events. These include syncope, migraine-related disor- seizure was associated with a fever with no obvious
ders, and self-gratification events (masturbation is normal cause, or signs of raised intracranial pressure) or whether
in infants, although sometimes shocking for the parents). the child can be referred non-urgently for a clinic
• Seizures may also be the first indication of raised intra- appointment.
cranial pressure and may occur in children with menin- • It is advisable not to start a discussion about epilepsy,
gitis or with a metabolic derangement, including low including the prognosis and consequences for lifestyle,
blood sugar. These children will be unwell. until the child has had further investigations (e.g., an
• Infants sometimes present with a history of myoclonic EEG) and a diagnosis has been made. Some children
jerks. These usually occur either when the child is asleep may not be started on medication immediately.
or when going into or out of sleep. The parents describe • Most neonatal seizures require urgent referral for inves-
rhythmic movements of the limbs. This is not a seizure, tigation, as they are often caused by an acute event,
does not require investigation or treatment, and the including hypoglycaemia, an electrolyte imbalance, or
parents should be reassured. infection.
• Myoclonic jerks are not seizures and do not require
What to Ask About the Child investigations or treatment.
• Document events preceding the episode(s) including at The Child With Headaches
what time of day/night the events occur, if there are any
obvious triggers, and what the child was doing before the Headaches are common in schoolchildren and are usually
seizure. benign. The challenge is to identify the very small group of
• Ask for a description of the seizure, from the events children who have serious underlying pathology, including
preceding the episode to the child recovering completely. brain tumours.
• Ask caregivers to describe any movements or sounds.
Explore whether the movements were symmetrical, and What to Ask About the Child
if there were any lateralizing signs including eye devia-
tion and tonic/clonic movements of one side of the body • Ask when the headaches first began. A long history
only. of intermittent headaches, and a child who is well in
• Ask about the length of the episode and whether the between, suggests a benign aetiology.
child had bladder or bowel incontinence during or after • Ask about the frequency of headaches and whether the
the episode. child is well in between episodes of headache. A head-
• Ask the child, if old enough about the events and what ache caused by meningeal stretching (i.e., raised intra-
he or she recalls. cranial pressure) is more likely to be constant rather than
• Ask how the child appeared and behaved after the intermittent.
episode. • Ask about the nature of the headaches:
• Throbbing headaches are typical of migraines.
What to Look for on Examination • Feeling as though there is a band around the scalp is
typical of tension headaches.
• Usually the patient will present to you after the seizure • Tenderness over the face may suggest inflammation
so you are unlikely to witness it. of the sinuses.
• Check whether there are any symptoms suggesting raised The Child With a Tic
intracranial pressure:
• Have there been recent changes in behaviour? The appearance of facial tics in children, although common,
• Is the headache made worse on coughing, sneezing, is a source of concern to parents.
bending over (e.g., to touch the toes), or squatting
down? What to Ask About the Child
• Is the headache worse in the mornings, and is it asso-
ciated with vomiting? • Ask if there were any triggers for the facial tics, including
• Does the headache wake the child from sleep? events that occurred prior to the start of the ticking.
• Enquire whether there have been problems with unsteadi- • Enquire whether the tics can be suppressed. Often chil-
ness of the gait, and ask whether the child has had sei- dren can suppress a tic if they are outside the home
zures. If the child wears glasses, enquire about recent eye environment, including at school.
tests or the need for one. • Ask if the tic is associated with motor or vocal tics sug-
• Explore the social history for any issues that may be gestive of Tourette’s syndrome which requires referral to
causing the child anxiety, including recent separation of secondary care.
parents, change in school, or bullying.
• If possible observe the tics or ask the parents to capture
• Assess whether the child looks well or unwell. A short them on video.
history of headaches in an unwell child with fever may • Observe for motor and vocal tics.
indicate an infective cause. • Conduct a neurological examination, particularly ensur-
• Consider checking the blood pressure. ing the cranial nerves are normal.
• Conduct a neurological examination. In particular examine
for unsteadiness of the gait and examine the cranial nerves. What to Do
Specifically assess whether the child can maintain an upward
gaze and whether there is sun setting of the eyes. • Reassurance is the most important management strategy
• Examine whether the child’s eyes move laterally in both for children with facial tics.
directions and whether there is diplopia. • If the tics are causing excessive distress, psychological
• Examine the optic fundi for papilloedema. It is often interventions may be helpful.
difficult to get a good view of the fundi in young • Suggest that the parents (and teachers) do not reprimand
children. the child, or try to stop the child from ticking, as this is
• Palpate the area over the sinuses to check for tenderness. likely to make the tics worse.
• Explain to the parents that the ticking often resolves,
What to Do although it is difficult to predict if and when the ticking
may get better.
• If the child is acutely unwell refer immediately, including
children with fever. The Child With Dysuria and
• If the child has abnormalities of the cranial nerves, gait,
or neurological examination then refer immediately as Abdominal Pain
these signs are suggestive of raised intracranial pressure. Guideline
• If the history and examination are not suggestive of
serious underlying pathology, then management will National Institute for Health and Care Excellence. Urinary
depend on the type of headache: tract infection in under 16s: diagnosis and management.
• Migraine: reassure, suggest simple analgesia, and if NICE clinical guideline 54. Available at https://www.nice
vomiting is a feature, then an antiemetic may be .org.uk/guidance/cg54.
useful. For children with frequent and debilitating
migraine, a trial of a migraine prophylaxis (pizotifen or What to Ask About the Child
propranolol) may be helpful. Mild hypoglycaemia is a
common trigger so encourage the child to eat regularly • In older children ask about fever, dysuria, abdominal and
and healthily. Children often do not drink enough, so loin pain, symptoms suggestive of UTI.
encourage an adequate intake of water daily. • Infants with UTI present with nonspecific symptoms,
• Tension headaches: reassure, offer advice about avoid- including poor feeding, failure to thrive, irritability, and
ance of triggers, and suggest simple analgesia and an unexplained fever.
adequate fluid intake. • In all children with a fever and no obvious source of
• Sinusitis: offer a trial of decongestants. infection, a clean catch urine sample should be obtained
• If headaches are debilitating, then refer to secondary care. for urinalysis, and culture if urinalysis is positive.
• Ask about a history of previous confirmed or uncon- • Management of a confirmed UTI:

firmed UTIs. • Refer all infants, less than 3 months of age, with either
• Explore the antenatal history and ask about any antena- upper or lower UTI, for parenteral antibiotics.
tal ultrasound scan results. • Refer all infants over 3 months of age for parenteral
• Ask about a history of constipation as children who are antibiotics if they are in the high/intermediate risk
constipated are prone to UTIs. group (see NICE guideline on page 50) or they are
• Ask about any family history of renal disease, including unable to tolerate oral fluids/medication.
vesicoureteric reflux. • Refer all children with recurrent UTIs (two upper
• Ask about the child’s development and if there are any UTIs or three lower UTIs).
concerns that the child has a neurological problem. • For infants over 3 months of age with an upper UTI
• Ask if the child has a history of dysfunctional voiding who are not unwell, manage at home with 10 days of
including poor urine flow or diurnal or nocturnal oral antibiotics. Have a low threshold for referral for
enuresis. parenteral antibiotics.
• Assess the child’s fluid intake and check for age-appropriate • For infants over 3 months of age with a lower UTI
consumption. who are not unwell, manage at home with oral anti-
• Ask about hygiene including how the child wipes his or biotics as per local guidelines (trimethoprim or a
her bottom (encourage children to wipe their bottoms cephalosporin).
from front to back). • Infants who are managed at home need an early
review to ensure they are responding to treatment.
What to Look for on Examination Review the choice of antibiotics once the urine culture
and sensitivity results are available.
• In most cases examination will be normal. • It is not necessary to repeat urine culture at the end
• Take the child’s temperature. of a course of antibiotics if the child responded well.
• Plot the child’s weight and height on a chart and compare • Further investigations for children under 3 years of age
to previous measurements to assess growth. with a proven UTI:
• Examine the abdomen for tenderness or an enlarged • Children under 3 years of age are most at risk of renal
bladder. damage following UTI. They are also more likely to
• Examine the spine and assess tone and power in the have underlying pathology leading to the UTI (e.g.,
lower limbs and observe the child’s gait. Rarely the child vesicoureteric reflux).
may have an underlying neurological condition. • These will depend on your local guidelines and refer-
• If appropriate size cuffs are available take the child’s ral hospital.
blood pressure. • Children under 6 months of age usually have a renal
ultrasound within 6 weeks. Further imaging depends
What to Do on local guidelines, whether the child responded to
treatment within the first 48 hours, and whether the
• Refer febrile children to secondary care if appropriate UTI was upper or lower guides further imaging.
according to the NICE guidelines (see above). Children, • For older children give advice about:
particularly infants, may need to be referred urgently • cleaning themselves after toileting;
before you are able to obtain a urine sample if they have • encouraging complete bladder emptying and a good
a fever and are unwell looking. fluid intake;
• Obtain a clean catch urine sample: • managing constipation where appropriate.
• Perform urinalysis looking for leukocyte esterase and • Prophylactic antibiotics are required only in special circum-
nitrites. The presence of nitrites with or without leu- stances (e.g., infants who are waiting for further imaging).
kocyte esterase is indicative of a UTI. • Most children who require prophylactic antibiotics will
• Send the urine for culture if urinalysis is positive. have been referred to secondary care, and the antibiotics
• Try to distinguish between: will have been started there.
• an upper UTI (acute pyelonephritis): bacteriuria, • Counsel parents on recognition of the symptoms asso
fever ≥38°C with or without loin pain; ciated with UTI, particularly in infants who may have
• a lower UTI: bacteriuria, with no systemic features. nonspecific symptoms.
• If the child can be managed at home, treat with antibiot-
ics if the urinalysis is positive for nitrites. The Child With Bedwetting
• If the urinalysis is negative for nitrites but positive for (Nocturnal Enuresis)
leukocyte esterase, only start the antibiotics if there are
clinical symptoms of UTI. • Nocturnal enuresis is a distressing condition with signifi-
• If urinalysis is negative for both nitrites and leukocyte cant impact on the lives of the child and the family.
esterase, then do not assume that this is a UTI but search • The prevalence improves with age: approximately 21%
for alternative causes to explain the symptoms. at 4 years decreasing to 1.5% at 9 years.
• It is important to establish whether the enuresis is • Manage a UTI appropriately.

primary (the child has never been dry at night) or sec- • General management for nocturnal enuresis includes the
ondary (there has been a recent loss of acquired night- following:
time control). • The child should never be penalized for wetting. A
supportive approach should be encouraged.
What to Ask About the Child • Optimize fluid intake and avoid caffeine.
• Manage constipation if present.
• Establish whether the child has primary or secondary • Waking or lifting the child to go to the toilet is a
enuresis. helpful practical measure in the short term but does
• If the child has secondary enuresis, consider systemic not influence long-term resolution.
illness (including UTIs and diabetes mellitus [DM]) and • Star charts may be helpful for all aspects of manage-
emotional issues. ment, including adequate fluid intake, regular toilet-
• Ask about the pattern of the wetting, including how ing, and engaging in practical steps (e.g., changing
many nights and how many times per night. The latter bed sheets).
might be difficult to ascertain if the child does not wake • If the child is under 5 years of age, a trial of 2 con-
up. Multiple episodes of wetting per night are suggestive secutive nights without wearing a nappy is worth-
of an overactive bladder. while to assess the success of any intervention.
• Enquire the time of night when wetting happens. Wetting • Alarm systems are often used in children with noctur-
often happens after 1 to 2 hours of sleep if the diagnosis nal enuresis. Alarms have a high long-term success rate
is enuresis. and may be useful if there has been no response to
• Ask if the child wakes on wetting. toileting and reward systems. They are less useful if the
• Ask about associated daytime symptoms: wetting is infrequent (one or two times per week). Assess
• Ask about daytime wetting, frequency of micturition the response after 1 month and, if successful, continue
(>7 times per day), or urgency of micturition, sugges- until the child has had 2 weeks of uninterrupted dry
tive of an irritable bladder. beds.
• Enquire whether the child has dysuria or other symp- • A trial of medication may be used in children from age
toms of a UTI. 5 years. Desmopressin may be used (1) for short-term
• Explore symptoms that would suggest DM including use (e.g., occasional sleepovers), (2) in conjunction with
thirst, polydipsia, polyuria, weight loss, abnormal an alarm system, and (3) if an alarm system is undesir-
breathing. able. Treat for 3 months and then withdraw for 1 week
• If DM is considered in a child, do an immediate capillary to check if dryness has been achieved. If used longer
blood glucose, and do not wait for a fasting blood glucose term, withdraw to assess success every 3 months.
or a urine sample.
• Check if the child is having an appropriate, or excessive, The Child With Protein in the Urine
fluid intake. Recommended intakes are:
• age 4 to 8 years: 1000 to 1400 mL/day; • Proteinuria may be an incidental finding in a well child
• age 9 to 13 years: 1200 to 2100 mL/day; but may also be a clue to underlying renal disease.
• age 14 to 18 years: up to 3200 mL/day. • A trace of protein is not usually significant; however, 1+
• Explore whether the child has learning difficulties, devel- of protein is the equivalent of 30 mg/dL and the urine
opmental delay, behavioural or emotional problems, or should be retested to check that the proteinuria has
a family history of DM. resolved.
• Persistent proteinuria is suggestive of a glomerular lesion
What to Look for on Examination and, if associated with haematuria, the likelihood of
underlying renal disease increases.
• In the majority of cases examination will be normal.
• Examine the abdomen for tenderness (possible UTI) and What to Ask About the Child
masses, and the spine for abnormalities.
• Observe the child’s gait as rarely enuresis may be second- • In a child with an incidental finding of proteinuria ask
ary to a neurological cause. if the child has had a recent illness and whether the child
• Plot the weight and compare against previous measure- has undertaken strenuous exercise. Both are triggers for
ments. A child with secondary enuresis and weight loss benign proteinuria.
loss must have DM excluded. • For a child with oedema and proteinuria ask if there has
What to Do been a previous history of oedema, suggesting that the
child has had previous episodes of proteinuria.
• Investigations are not normally required unless the child • Ask if there is a history of a recent upper respiratory
has symptoms of UTI (check urinalysis) or DM (check infection, as glomerulonephritis may follow a recent
capillary blood glucose immediately). infection.
• Check if the child is taking any medications, as some • Brown or cola coloured urine is suggestive of a glo-
drugs (e.g., penicillamine, gold, and ethosuximide) may merular source of bleeding (e.g., poststreptococcal glo-
be associated with nephrotic syndrome. merulonephritis, Henoch-Schönlein purpura [HSP],
• Ask about a family history of renal disease. or HUS).
• Ask if there are symptoms of a UTI (dysuria, frequency
What to Look for on Examination or urgency of micturition, fever, abdominal or loin pain).
• Enquire whether the child has had a recent upper respira-
• Check the child’s temperature, blood pressure (if an tory tract infection as glomerulonephritis may follow an
appropriate sized cuff is available), heart rate, and capil- infection.
lary return; assess if the child is haemodynamically stable • Ask if the child has had a rash, associated with HSP
or acutely unwell. and HUS.
• Assess for oedema in dependent sites, including the feet, • Ask about any medications, and whether the child is
lower back, and face when the child has been recumbent. taking rifampicin, which can cause pink urine.
• Examine the abdomen for pain. • Ask about the ingestion of specific foods including
• If possible, test for orthostatic proteinuria (i.e., check a beetroot.
urinalysis in the recumbent and standing positions). The • Check if there is a family history of deafness (Alport
standing sample will have two to four times more protein syndrome), renal disease or haematuria, and whether the
in it compared to the recumbent sample. Orthostatic child has had previous documented episodes of haema-
proteinuria may also be diagnosed if an early morning turia. Consider benign familial haematuria.
urine specimen is negative for protein but samples taken • Enquire whether the child has a history of easy bruising
later in the day are positive. or bleeding and consider a haematological cause.

• If there is an incidental finding of proteinuria (1+ or • There will be little to find in children with an incidental
more), repeat urinalysis three times over a period of 2 to finding of haematuria.
3 weeks, using an early morning urine sample. If pro- • Check for oedema suggesting proteinuria.
teinuria is persistent then obtain an early morning urine • Examine the abdomen for a mass (Wilms tumour pre-
sample and send to the laboratory for a protein to creati- sents with a mass in a young child) and tenderness (may
nine ratio. The normal range is below 20 mg/mmol. be present with UTI).
• If the child has intermittent proteinuria, reassess in 3 to • Examine for a rash, bruising, petechiae, or purpura,
6 months. If still present refer to secondary care. which is associated with HSP and HUS.
• If the child has orthostatic proteinuria, reassure and • Check the joints as swollen and painful joints may be
repeat urinalysis in 1 year. If the child has nonorthostatic present in HSP.
proteinuria then refer for further evaluation. • Take the temperature and if possible measure the blood
• If the child has associated haematuria or oedema, pressure, ensuring the correct size cuff is used.
or a raised protein to creatinine ratio, refer to second
ary care. What to Do
The Child With Blood in the Urine • In an asymptomatic child with isolated, microscopic hae-
maturia, repeat urinalysis with microscopy in 2 weeks. If
• Microscopic haematuria is not visible to the naked eye, the haematuria persists, check the blood pressure, send
but diagnosed on urinalysis and seeing red blood cells on urine for a protein–creatinine ratio, arrange to repeat
microscopy. urinalysis again, and ask family members to bring urine
• Macroscopic haematuria is visible to the naked eye. samples for urinalysis (considering a diagnosis of benign
• It is important to confirm the presence of red blood cells familial haematuria).
in the urine, as there are other reasons the urine might • If haematuria is present on three occasions, refer to sec-
appear red or brown in colour, including ingestion of ondary care for further investigations.
some food substances, urates, and the presence of myo- • If there are symptoms suggestive of UTI, manage
globin or haemoglobin. appropriately.
• Children with macroscopic haematuria and no obvious
What to Ask About the Child cause whould have a renal ultrasound performed to
exclude a Wilms tumour.
• Ask about the colour of the urine: • Refer any child with persistent haematuria to secondary
• Bright red or pink urine is suggestive of bleeding care for further assessment.
from the urinary tract (e.g., UTI, trauma, or a renal • Refer children with haematuria and proteinuria to sec-
calculus). ondary care, as this is suggestive of glomerulonephritis.
ALGrawany
The Child With an Itchy Perineum • Do not overwash the area.

• Avoid shampoo, soap, and bubble bath; soap substi-
• Inflammation or irritation of the vulva is common in tutes can be used.
young girls and improves at puberty. • Suggest showers rather than baths, particularly for
• It results from a lack of oestrogen and a thin vaginal/ washing the hair.
vulval lining that is easily irritated. • Avoid tight clothing and change wet clothing quickly.
• Girls have often had the symptoms for some time before • Use cotton underwear and change regularly.
presenting to medical staff. • Encourage regular toileting and complete emptying
of the bladder.
What to Ask About the Child • Use soft toilet paper and encourage good toilet
hygiene (wipe from front to back).
• Ask about the frequency and pattern of symptoms • Treat constipation.
(e.g., itch and discharge, both of which are features of • Avoid prolonged sitting if experiencing symptoms
vulvovaginitis). (e.g., horse riding or cycling).
• Ask about urinary symptoms, including burning or • Suggest strategies to manage an episode of vulvovaginitis:
stinging on passing urine. If these are present obtain a • Cool compress may soothe the area.
clean catch urine specimen for urinalysis and culture. • Barrier creams (particularly those used for napkin der-
Lichen sclerosis is an uncommon condition in children matitis) are usually effective.
that can present with itch and dysuria, but it has distinct • Some experts suggest urinating with the knees open
clinical findings (see the upcoming discussion). and rinsing with water afterwards.
• Ask about the following that might be triggers for • Avoid tight clothing and suggest wearing nightdresses
vulvovaginitis: rather than pyjamas to sleep. The exception would be
• Irritants, including soap, bubble bath, or shampoo a child with threadworms where pyjamas can decrease
• Toilet hygiene, including whether bottom is wiped the chance of eggs being spread to bedclothes or
from front to back fingers during the night.
• Wearing of tight clothing, including jeans and tights • Prescribing antibiotic or antifungal treatment is not
• Ask whether threadworms have been noted. Thread- necessary.
worms cause itch and scratching, which may be the cause • Management of lichen sclerosis:
of the symptoms, or may have exacerbated an episode of • This is treated in the same way as vulvovaginitis.
vulvovaginitis. • A steroid cream at night may be helpful.
• Explore carefully whether there are any concerns around • The condition will usually resolve naturally but may
child abuse, including whether the child has demon- cause scarring.
strated any change in behaviour, if there have been previ- • If there is doubt about the diagnosis, refer to second-
ous social work concerns, how the child is doing at ary care.
school, and who regularly looks after the child.
What to Look for on Examination The Child With Worms in the Stool
• Examine the external vulval/vaginal area for erythema, • Threadworms affect both pre-school and school-age chil-
discharge, skin changes, and labial adhesions. Vulvovagi- dren, with often more than one family member affected.
nitis presents with an erythematous vulva/vagina. Lichen • They are spread by the faecal-oral route and the eggs
sclerosis also presents with erythema and white patches can survive for up to 2 weeks, so repeat infection is
in a distinctive figure-8 pattern. common.
• Observe the external anal margin for threadworms. • Other worms may be present in the stools, particularly
• Examine for other skin conditions, including eczema. in children who have travelled to areas where other vari-
• Observe for any features of potential concern, including eties of worms are prevalent.
bruising around the perineal area or bleeding.
What to Do
• Ask about itch, particularly intense, nocturnal, perineal
• Investigations are not necessary or helpful. Vaginal itching that is common with threadworms.
thrush is very unusual in an immune-competent, prepu- • Ask about previous episodes.
bescent girl. • Ask about sleep disturbance and irritability at night.
• If the child has symptoms suggestive of a UTI then • For girls ask about symptoms of vulvovaginitis.
obtain a clean catch urine for urinalysis and, if positive, • Ask about recurrent abdominal pain that may be
a urine culture. present.
• Advice to prevent recurrence of vulvovaginitis: • Ask if other family members have been affected.
What to Look for on Examination • Ask an older child to retract the foreskin for you to assess
for phimosis.
• Look for small white worms on the perineum or in the • Assess for urinary flow if there is a history of problems,
stools. and ask about ballooning of the foreskin upon micturi-
• Examine for evidence of perineal or vulval erythema or tion due to a tight meatus.
irritation. • In severe phimosis, there may be obstruction to the
urinary flow.
What to Do
What to Do
• Investigation for threadworms is not usually necessary.
The worms are readily seen at night or in the early • If there are symptoms of a UTI, then obtain a clean catch
morning on the perineum or visible in the stools. urine for urinalysis and culture.
• If the diagnosis is unclear, the sellotape test can be con- • Refer for a urological assessment if the child has recur-
ducted: Tape a piece of sellotape over the perineum and rent episodes of balanitis or phimosis, as a circumcision
then remove. The worms will be seen on the tape. may be required.
• Management includes treating all family members, as • A subpreputial swab is only required if symptoms are
worms are readily spread within households. Prescribe severe or persistent.
mebendazole and be aware that a second course may be • General management of balanitis includes:
required. • avoidance of potential irritants, including soap,
• Ask all family members to shower; wash all bedclothes, bubble bath, and wipes;
towels, and soft toys. Vacuum all carpets and floors. • encouragement of good hygiene, including washing
• Ensure that fingernails are cut short, encourage good the area twice daily with warm water and patting (not
hand washing (including scrubbing of the nails), and rubbing) dry;
avoid thumb sucking. • thorough hand washing;
• in younger children, encouraging frequent nappy
The Child With A Painful Penis changes;
• if the foreskin is free, gently retract during washing,
• Balanitis affects prepubescent boys, mostly those who are but do not forcefully retract the foreskin of an infant.
pre-school age. • Specific management of balanitis will be related to the
• There is inflammation of the glans of the penis, which is underlying aetiology.
painful and sometimes associated with swelling. • Nonspecific dermatitis is treated with a topical hydro-
• The non-, or partially, retractile foreskin present in this cortisone with imidazole cream twice daily for 1 week
age results in poor hygiene and infection. only.
• Most boys will only experience a single episode, but • If there is no improvement after 1 week, stop the cream
balanitis can be chronic or recurrent. and send a swab from the affected area.
• Aetiology may be grouped into nonspecific dermatitis, • For allergic or irritant balanitis, treat with topical hydro-
infection, irritant, or allergic dermatological, including cortisone.
eczema, and due to manipulation of the foreskin. • If a swab has shown candida infection, treat with imid-
azole cream, but do not treat blindly with an antifungal
What to Ask About the Child cream.
• For bacterial infections, treat with a 1-week course of
• Ask about penile pain, itch, odour, and any penile dis- oral flucloxacillin +/- hydrocortisone cream depending
charge suggestive of an infective aetiology. on the level of discomfort.
• Enquire about penile swelling and difficulties with
urination and check for a normal urine stream. The Child With a Groin Swelling
• Ask about previous episodes.
• Explore hygiene practices and exposure to irritants, • The two most common causes of a groin swelling (apart
including bubble bath. from inguinal lymphadenopathy) are an inguinal hernia
• Ask about associated skin conditions, including eczema, and a hydrocele.
and check for symptoms of UTI (dysuria, abdominal/ • Inguinal hernias may occur in girls but are rare, and also
loin pain). present as a lump in the groin.
What to Look for on Examination What to Ask About the Child

• Observe for redness of the glans and foreskin with • Ask when the swelling was first noticed. Inguinal hernias
possible swelling. are usually noticed during the second or third month
• Observe for penile exudate and odour. of life.
• Enquire whether the swelling comes and goes. Often • meningococcal disease;
hernias appear during coughing or crying and reduce • idiopathic thrombocytopaenic purpura (ITP);
spontaneously between such times. • HSP;
• haematologic malignancy;
What to Look for on Examination • viral illnesses, streptococcal infections, connective tissues
disorders, and HUS.
• Transillumination will help to identify a hydrocele. • Any child with a non-blanching rash needs urgent assess-
However, testicular tumours also transilluminate so may ment and investigation.
be misdiagnosed for a hydrocele. • HSP affects the skin, joints (most commonly the knees
• Check for cellulitis of the scrotum and groin area, and ankles), abdomen, kidneys, and gastrointestinal tract
which may indicate an incarcerated hernia. If so, do (children may present with gastrointestinal bleeding).
not try to reduce the hernia but refer immediately to Around 1% of children develop end stage renal disease,
secondary care. but mostly the nephritis is self-limiting.
• ITP is an immune mediated thrombocytopenia that is
What to Do usually acute. Children are usually well and of pre-school
age.
• Refer all children with a groin swelling for a surgical • HUS presents with thrombocytopenia, anemia, and
opinion (except for a swelling due to enlarged inguinal renal failure. The child often has bloody diarrhoea, most
lymph nodes). commonly caused by E. coli, Shigella, or echovirus.
• Urgently refer children with a suspected incarcerated
hernia. What to Ask About the Child
The Child With A Painful Scrotum • Ask how long the rash has been present. A rapidly spread-
ing rash in an unwell child suggests meningococcal
• It is important not to miss a torsion of the testis in a disease and requires urgent management.
child presenting with a painful scrotum. • Check if the child has a fever, which would suggest an
• Other causes include epididymo-orchitis, idiopathic infectious cause, but may also be present in malignancy
scrotal oedema, trauma, tumour, and a varicocele. and connective tissue disorders.
• Enquire whether the child had a preceding upper respira-
What to Ask About the Child tory tract infection as this is often a prodrome to HSP
and ITP.
• Ask whether the pain is in the scrotum and testis or also • Ask if the child has bleeding from mucous membranes
in the abdomen. A torsion of the testis presents with a (e.g., the nose), as this may be present in malignancy
painful, swollen testis, and often with pain in the lower and ITP.
abdomen and groin. • Enquire whether there is a family history of bruising,
suggestive of a familial form of thrombocytopenia.
What to Look for on Examination • A history of joint pain, weight loss, or fever may indicate
a malignancy or connective tissue disease.
• A painful, swollen testis indicates a torsion of the testis • Abdominal pain and/or arthritis is a common presenta-
until proven otherwise. tion of HSP.
• Examine the scrotum whilst the child is standing up. Do • Check if the child has had bloody diarrhoea as abdomi-
the veins of the testis feel like a bag of worms? If so, the nal pain and bloody diarrhoea are often the prodrome
child may have a varicocele. to HUS.
• Check whether the scrotum and perineal area is red and
swollen, with a non tender testis. This may be due to What to Look for on Examination
idiopathic scrotal oedema.
• Assess if the child is acutely unwell. Measure the tem-
What to Do perature as a high fever is suggestive of a bacterial infec-
tion (e.g., meningococcal or streptococcal disease).
• Refer all cases of a painful scrotum for surgical review. • Check the pattern of petechiae, purpura, and/or
Torsion of the testis should be referred urgently to a bruising:
paediatric surgeon. • Meningococcal: no specific distribution. There is a
widespread, rapidly progressing rash in an ill child.
The Child With a Non-blanching Rash • HSP: typical distribution on the lateral malleoli,
ventral aspects of feet, buttocks, and extensor aspects
• The most common causes of a non-blanching rash (pete- of legs. The rash is a palpable, purpuric, rash some-
chiae and purpura) include: times with preceding urticaria. The child often has
associated joint swelling/inflammation and a tender • Enquire whether there has been any contact with TB
abdomen on palpation. or recent travel to a TB-endemic area, and if the child
• ITP: widespread petechiae and purpura in a child has night sweats. Night sweats are present with TB or
who is not acutely unwell (note: a child with menin- malignancy.
gococcal disease is acutely unwell). • Ask whether the child has been more lethargic than
• Check if the child has a petechial rash that is only usual, which may suggest TB or malignancy.
present in the distribution of the superior vena cava • Ask if the child has a chronic cough that may suggest TB.
(i.e., above the clavicles). In a child with vomiting • Ask about eczema, as this is a common cause of localized
or a cough, petechiae may be present in this lymphadenopathy.
distribution.
• Non-accidental injury may result in non-blanching What to Look for on Examination
lesions suggestive of the mechanism of injury (e.g.,
finger marks or bite marks). • Assess the pattern of the enlarged lymph nodes. Are they
• Check for lymphadenopathy, hepatomegaly, and spleno- only in the cervical region or also in the axillary and
megaly which may indicate a malignancy. inguinal regions?
• In an infant check for signs of a non-accidental injury, • Check the appearance of the nodes. Are they red, tender,
including unusual patterns of bruising and petechiae or fluctuant, suggesting infection and possibly an abscess?
which may be present in the upper body as a result of • Check for supraclavicular nodes. A supraclavicular node
shaking, a bulging fontanelle indicating raised intra- is not usually associated with a recent infection and is
cranial pressure, or inflammation of limbs suggestive of more suggestive of malignancy.
a fracture. • Nodes that are hard, matted together, or non mobile
(i.e., fixed to underlying structures) are more suggestive
What to Do of non benign lymphadenopathy.
• Examine for hepatosplenomegaly, which may be present
• Most children with a non-blanching rash should be referred in malignancies and also in some viral infections, includ-
for further investigation and management. Exceptions are ing Epstein-Barr virus (glandular fever).
when the child has a known respiratory illness, is not acutely • Measure the weight and compare against previous
unwell, and has petechiae in the distribution of the superior weights. Benign lymphadenopathy does not present with
vena cava. weight loss, and further investigation to rule out TB and
• If the child is acutely unwell, give IM antibiotics prior malignancy should be undertaken in a child with acute
to transfer. weight loss.
• In a child with suspected HSP, check the urine for blood • Examine the mouth for dental caries.
and protein and, if possible, check the blood pressure.
If HSP is confirmed, it is important to monitor the What to Do
blood pressure and urine protein at intervals, initially
weekly then monthly for 6 months, to detect any renal • If the child is well, with mobile, small lymph nodes and
damage. a recent infection, reassure the parents and arrange to
review the patient again.
The Child With Cervical Lymphadenopathy • If you are suspicious of TB or malignant disease, then
refer the patient for further investigations.
• Cervical lymphadenopathy is extremely common in chil-
dren, a cause of great concern to parents, and a common The Child With Pica
reason for referral to secondary care.
• The main causes are benign lymphadenopathy secondary • Pica (eating substances with no nutritive value) is a
to infections and, rarely, granulomatous disease (i.e., TB) common reason for referral to secondary care.
and malignancy. • The main cause of pica is iron deficiency or, rarely, lead
• The most common malignancies causing lymphadenopa poisoning.
thy include Hodgkin disease, non-Hodgkin lymphoma, • Iron deficiency anemia is common in children, and is
and leukaemia. usually due to a poor diet or a diet consisting largely of
cow’s milk.
• Ask if the child has had any recent infections, particu-
larly of the throat, ear, nose, and scalp. Benign lymph- • Take a good detailed history, specifically asking about
adenopathy is typically secondary to a recent infection. the amount of milk in the child’s diet and whether the
• Ask if any of the nodes have recently increased in size. Rapid child eats meat. Iron deficiency anemia is common in
enlargement of nodes may need further investigation. children whose diet consists mostly of cow’s milk or who
are vegetarian. Red meat and cereals are good sources of • The vast majority of those referred to secondary care
dietary iron. have common variations of normal physiological growth.
• Ask about the child’s gestational age. Preterm infants These include familial short stature and constitutional
require supplemental iron, as iron is transferred transpla- short stature (also known as constitutional delay).
centally during the last trimester of pregnancy and there • Pathological causes are unusual and include endocrine
is little iron in milk. The iron in breastmilk is more problems, Turner and Noonan syndromes, chronic disease,
available than that in formula milk. and malnutrition.
• Ask if there has been any history of blood loss (e.g.,
melena, blood in vomit, or menorrhagia in pubertal What to Ask About the Child
girls). This is to exclude other causes of iron deficiency,
particularly in a child with an apparently good diet. • Ask for the parents’ and siblings’ heights and ages of
• Enquire whether the family home is old and consider pubertal onset:
whether the child has developed lead poisoning from old • Children with familial short stature have short parents,
paint work. a normal growth velocity and pubertal onset, and no
• Check if there is a family history of β-thalassaemia, signs of physical disease. These children look short but
which also causes a low haemoglobin and low mean normal.
corpuscular volume. • Children with constitutional short stature have parents
with normal stature; however, one parent may have
What to Look for on Examination had a delay in growth and late puberty. These children
look short and normal, have a delayed puberty with
• Examine for pale conjunctivae and pale palmar creases. late growth spurt, but achieve a final height within
• Always examine for lymphadenopathy and hepato- the parental target range.
splenomegaly to ensure that the child does not have an • Ask about nutrition and assess whether the dietary intake
underlying pathology accounting for the iron deficiency. is adequate for growth.
• Examine for a systolic flow murmur often present in iron • Ask about symptoms of chronic diseases, including diar-
deficiency. rhoea, abdominal pain (inflammatory bowel disease,
• Examine the mouth for glossitis or angular cheilitis, both coeliac disease), and uncontrolled asthma.
suggesting iron deficiency. • Enquire about inhaled steroid therapy, and the length
and dose of steroid treatment which may affect
What to Do growth.
• Explore the social history and whether there are concerns
• It is often difficult to take blood tests in primary care, so about psychosocial deprivation.
children are often referred to secondary care. If blood • If relevant ask about signs of puberty.
testing is possible, take a full blood count and film, fer-
ritin level, and lead level. What to Look for on Examination
• In iron deficiency anemia there will be a low mean
corpuscular volume (MCV) and low haemoglobin. • Plot all weight and height measurements on an appropri-
• In lead poisoning the blood will be normochromic or ate chart.
slightly hypochromic with characteristic basophilic • If the child is less than 2 years of age, also plot the
stippling on the film. OFC.
• If relevant for ethnicity, also send blood for a haemoglo- • It is useful to measure the height at four monthly inter-
binopathy screen. vals to assess the height velocity.
• Usually iron deficiency is treated with oral iron sup- • Conduct a systemic examination for signs of chronic
plements after the diagnosis is confirmed. disease.
• Children with lead poisoning require referral to sec- • Assess if there are any dysmorphic signs (e.g., skeletal
ondary care and chelation therapy. disproportion).
• Premature infants should take oral iron supplements • Plot the parents’ heights on a growth chart at “age 18
until at least 6 months of age. years.” This is useful information to provide if you are
referring to secondary care, as both parents may not
The Child Who Is Short attend the hospital appointment.
• The mean expected adult height is approximately calcu-
• Short stature is defined as a height below the 0.4th centile lated as follows:
on a growth chart or less than 2 standard deviations • Boy: The mean of the parents’ heights plus 7 cm
below the mean for gender and age. • Girl: The mean of the parents’ heights minus 7 cm
• Short stature may be a source of emotional and social • Most growth charts explain how to calculate the midpa-
distress to both children and parents who often ask for rental height centile and the target range around this
referral to secondary care. mean value.
What to Do • Plot parental heights on a chart and calculate the expected

adult height.
• If you or the parents have concerns about the child then • Check for features of early puberty.
refer to secondary care. However, in most cases monitor-
ing the height velocity and reassurance will be the main- What to Do
stays of management.
• If referring to secondary care it is useful to provide a • If the cause is thought to be normal genetic tall stature
detailed history, including measurements of the child, or constitutional tall stature, monitor the growth over
their siblings, and parents. 6 to 12 months and reassure the parents and child.
• If the history and examination are not consistent with a
The Child Who Is Tall normal variant tall stature, refer to secondary care.
• If obesity is an associated issue, provide guidance on
• Tall stature, defined as a height greater than 2 standard weight management.
deviations above the mean for gender and age, is more
accepted by society than in previous generations.
• Concern is often expressed by parents about girls who The Child With Precocious Puberty
are tall, although most cases will not require investiga-
tion. However, repeated measurements to assess growth • The age of onset of puberty is subject to much variation.
over a period of 6 to 12 months are important. Definitions of precocious puberty vary in the literature,
• Rarely, tall stature is caused by endocrine problems but features of sexual development in a girl younger than
including hyperthyroidism or precocious puberty. 8 years and a boy younger than 9 years require assessment.
• The impact of early sexual development can be detrimen-
What to Ask About the Child tal to a child both physically and emotionally.
• Precocious puberty can be true (i.e., early puberty but
• Ask about parental heights and heights of any siblings. under normal hypothalamic control) or pseudo (i.e.,
Most growth charts explain how to calculate the midpa- independent of hypothalamic control).
rental height centile and the target range around this • True precocious puberty is common in girls, but rare in
mean value. Assess whether the child’s height is within boys. Therefore it is important to investigate and identify
the expected parental target range. The mean expected a cause for precocious puberty in all boys.
adult height is calculated as follows:
• Boy: The mean of the parents’ heights plus 7 cm What to Ask About the Child
• Girl: The mean of the parents’ heights minus 7 cm
• Ask when the parents noticed that the child was tall and • Ask about growth and pubertal onset in the parents and
try to assess whether the child has been growing con- any siblings.
stantly or if the growth has accelerated recently. • Ask specifically about different aspects of puberty:
• Ask about signs of puberty in the child and whether the • Breast enlargement
parents had early puberty: • Penile enlargement
• In normal genetic tall stature: one or both parents are • Development of pubic hair
tall, and the child looks normal and tall. • Vaginal bleeding
• In constitutional tall stature, the child will have early • Body odour
puberty and grow to his or her final height earlier than • Mood swings
peers. • Ask about a history of headaches, vomiting, visual dis-
• Ask about symptoms suggestive of hyperthyroidism and turbance, or polydipsia. These suggest a brain tumour in
whether the child has headaches or visual problems sug- the presence of precocious puberty.
gestive of a cranial lesion (such as a pituitary tumour in
acromegaly). What to Look for on Examination
What to Look for on Examination • Measure the child and plot weight and height on an
appropriate chart and compare to previous measurements.
• Measure and weigh the child, plot on an appropriate • If you are confident, perform Tanner staging on the child.
chart, and compare to previous measurements. • Examine the optic fundi and the cranial nerves, and
• If the child is less than 2 years, measure and plot the OFC. observe the gait (very rarely precocious puberty is caused
• Assess if there are features of hyperthyroidism: by a brain tumour).
• Weight loss • Neurofibromatosis and McCune-Albright syndrome are
• Goiter both associated with precocious puberty, so check the skin for
• Tachycardia cafe au lait spots (neurofibromatosis and McCune-Albright
• Exophthalmos syndrome) and axillary freckling (neurofibromatosis).
What to Do • If hair is present at 6 to 8 years and is sparse, refer for

assessment. The hair growth will be reviewed every 3
• Refer all girls under 8 years and boys under 9 years with to 4 months over a period of 1 year to ensure that it is
signs of sexual development to secondary care. appropriate and there is no pubertal development.
• If hair growth is excessive and causing emotional distress,
refer for assessment and advice about hair removal,
The Child With Excess including chemical and laser therapy.
Body Hair • If features are suggestive of polycystic ovary syndrome,
refer for assessment.
• Excessive or premature hair development can cause sig-
nificant anxiety and distress for children, particularly The Child With A Painful Limp
girls.
• It is important to establish whether there are associated • The most common cause of limp due to hip pain is
concerns regarding precocious puberty. transient synovitis, most common between the ages of 4
• Adrenarche, the adrenal stage of puberty, results in body and 10 years.
odour, greasy skin and hair, weight gain, and pubic and • About 90% of cases resolve in 7 days but, as it cannot
axillary hair development without breast development. be reliably distinguished from more serious causes of hip
It is usually seen from 8 years and is considered prema- pain, all children should be referred to secondary care.
ture if it occurs before 6 years of age. An exaggerated • The child who presents with a painful limp and com-
form can be seen between 6 and 8 years. plains of pain in the knee should be assumed to have a
• Hirsutism (androgen dependent areas) and hypertricho- problem in the hip until proved otherwise.
sis (generalized) are forms of excessive or inappropriate • An unwell child who is non–weight bearing is likely to
hair growth. They predominate in certain ethnic groups have a more serious underlying pathology, including
but can also be indicative of polycystic ovary syndrome. septic arthritis, osteomyelitis, or leukaemia.
• Ask about growth and pubertal onset in parents and any • Check the child’s age as many aetiologies occur in spe-
siblings. cific age ranges (e.g., synovitis tends to affect pre-school
• Ask about features of adrenal excess including greasy skin children).
and hair, and body odour. • Ask whether the onset was acute or chronic and whether
• Enquire about mood swings or behavioural disturbance. there was a prodromal illness. Transient synovitis typi-
• Explore whether the child has had a recent growth spurt cally presents after a viral illness.
or gain in weight. • Ask about a history of trauma and consider a Toddler
• Ask about other pubertal features, including breast and fracture in a young child.
penile enlargement; and in girls, features suggestive of • Enquire if there has been a history of systemic upset
polycystic ovary syndrome, including menstrual distur- including fever, lethargy, pallor, weight loss, or easy
bance and weight gain. bruising or bleeding, and think about diagnoses includ-
ing leukaemia, septic arthritis, and osteomyelitis.
What to Look for on Examination • If there is pain at rest or at night consider osteomyelitis,
septic arthritis, or malignancy.
• Measure and plot the height and weight and compare • If the child is unable to bear weight, has pain at rest,
to previous measurements. Check the height velocity. and is also febrile, then consider septic arthritis or
Is the child deviating away from his or her previous osteomyelitis.
centile line? • Take a careful history of the presentation of any injury.
• Look for tall stature. If the history is not consistent with the injury, consider
• Assess the amount and distribution of hair growth. a possible non-accidental injury.
• If you are confident, perform Tanner staging.
• Observe for greasy hair and skin. Is there body odour? What to Look for on Examination
• Examine the abdomen for masses.
• Check the optic fundi and perform a general neurologic • Assess whether the child looks well or unwell.
examination. • Take the temperature and observe the level of comfort
of the child. Children with septic arthritis are in pain
What to Do at rest.
• Examine for restricted movement, bony tenderness, joint
• If pubic or axillary hair is present before 6 years of age, swelling, tenderness, erythema, or deformity and assess
refer for investigation. the child’s gait and spine.
• Remember to examine the feet. Check if there is a local What to Look for on Examination
cause for the limp (e.g., a foreign body).
• Examine for a rash, as HSP may present with joint • Assess whether the infant is thriving. Plot the infant’s
swelling and pain. current weight on a chart and compare to previous
• Check for pallor, lymphadenopathy, bruising, or hepa- weights. Infants with GOR are usually thriving. If the
tosplenomegaly which may suggest a malignancy. infant is not thriving, then consider other diagnoses.
• Examine for pallor, cyanosis, and dehydration. Infants
What to Do with GOR are usually well looking, normally developed,
and thriving. They are miserable and often cry when
• Refer all children with a painful limp to secondary care being fed, but are not acutely unwell looking.
to exclude serious pathology. • Always try to observe a feed.
• Conduct a full systematic examination of the infant,
The Infant Who Vomits or Screams including auscultation of the heart and palpation of the
When Feeding abdomen.
• The complaint of distress or vomiting with feeds is very What to Do

common in general practice.
• Causes range from relatively benign to potentially life • If the infant has fever and is unwell, refer to secondary
threatening, but the cause can often be elicited through care (see page 39).
a careful history. • If you suspect that the infant has pyloric stenosis, refer
to secondary care.
What to Ask About the Child • If you think the infant has GOR (thriving, well looking,
vomiting or screaming with feeds), try the following:
• For breastfeeding infants, ask about: • Reduced volume but more frequent feeds
• duration and frequency of feeds (in the first few weeks • Thickened feeds in formula-fed babies (e.g., Instant
of life infants will feed at least eight times per 24 Carobel)
hours, and usually more frequently than this); • Gaviscon. This is easy to give to formula feeding
• whether the infant appears satisfied after feeding from infants where it is added to bottles. For breastfeeding
one breast. If infants are taken off one breast after a infants, it is not as easy, as it needs to be given before
few minutes and then offered the second breast, the feed, ideally mixed with breastmilk.
they will only receive foremilk (containing water and • A trial of an acid-blocking medication may be useful
lactose) and no hindmilk (containing more fat). These (ranitidine or omeprazole). Doses are given in the
infants may feel hungry again soon after feeding. British National Formulary for Children.
• For formula feeding infants ask about: • Children between 1 and 2 years can be considered for
• the number and volume of feeds per day; a 4-week trial of either omeprazole or ranitidine.
• calculating the volume of feeds the infant is receiv- • Arrange for someone to review the infant in a few days’
ing. If the total volume of feeds is above 150 mL/ time. GOR can be extremely upsetting for parents, even
kg/24 hours and the infant is thriving, suggest reduc- though they can see that their infant is thriving, and
ing the volume per feed, particularly if the infant is they usually need a lot of support. It is easy for a breast-
vomiting. feeding mother to become discouraged and to stop
• Ask about vomiting, including the frequency and timing breastfeeding.
of vomits in relation to feeds, and whether the vomit • If the vomiting and screaming does not settle, refer to
contains blood or bile. In gastrooesophageal reflux the secondary care. It is not sensible to switch formula milks
infant will vomit effortlessly during, after, and sometimes without a clear reason to do so:
between feeds. Feeding relaxes the lower gastrooesopha- • Cow’s milk protein intolerance presents as excessive
geal sphincter. The vomit in GOR will not contain bile, crying, vomiting, and sometimes diarrhoea and blood
and rarely contains blood. in the stool. Occasionally the infant may have consti-
• Ask if the vomiting is projectile and only occurs imme- pation. There is no diagnostic test, but other causes
diately after a feed. Consider pyloric stenosis and ask of crying should also be sought. A short trial of a
about a family history of pyloric stenosis. Infants with hydrolysed formula will result in a dramatic improve-
pyloric stenosis may have dehydration and failure to ment and reintroduction of cow’s milk in resumption
thrive, but if diagnosed early enough the only sign may of symptoms. Local guidelines are usually available
be projectile vomiting. about the brand of milk to prescribe and at what age
• Ask about the behaviour of the infant. Infants with GOR to rechallenge with cow’s milk.
will often display certain posturing, including arching True allergy to cow’s milk presents with an urticarial rash
of the back, straightening out of the legs, and becoming or anaphylaxis. There is often a personal or family history of
very distressed when put in a supine or prone position. atopy. It is appropriate to refer these children to secondary
care for advice. These children need a hydrolysed formula What to Do

and a milk-free diet for at least the first year of life.
• A well infant whom you think has a feeding problem can
The Infant Who Has Weight Faltering in be dealt with in primary care with support from an
experienced health visitor and, if relevant, breastfeeding
the First Few Weeks of Life expert.
What to Ask About the Child • If possible check for a UTI, which may present with
failure to thrive in an infant who has few other symptoms.
• Ask if the mother thinks the baby is unwell. A baby who • If there are concerns about the infant’s general health or
is failing to thrive may have sepsis. no obvious cause can be found for the failure to thrive
• Take a thorough feeding history: then refer to secondary care.
• For breastfed infants, ask about: • Regular review of infants is needed and their families
1. frequency of breastfeeds—a young infant who is need support and encouragement.
exclusively breastfed should be taking a minimum
of eight feeds per 24 hours; The Infant Who Has Weight Faltering After
2. duration of breastfeeds (Is the baby offered one the First Weeks of Life
breast until satisfied and comes off the breast inde-
pendently? Is the infant offered the second breast?); • Most cases of failure to thrive or weight faltering are due
3. whether the baby feeds overnight or sleeps through to poor oral intake and not to underlying pathology.
the night; However, sometimes the child has poor intake because
4. whether the baby is given additional fluids or feeds of an underlying condition (e.g., children with a UTI
(e.g., Is the infant given drinks of water [with may not feed well).
empty calories] between feeds?); • It is always important to take a detailed dietary history.
5. if breastfeeding feels comfortable for the mother.
• For formula fed infants, ask about: What to Ask About the Child
1. the frequency of feeds and volume of each feed
taken; • Take a detailed feeding history, starting from how
2. the type of milk and how the milk is prepared. If the child was fed in the first months of life (see The
milk is prepared incorrectly and made too dilute, Infant Who Has Weight Faltering in the First Weeks
this could be a reason for failure to gain weight. of Life).
• In all children ask about vomiting, diarrhoea, and leth- • Assess whether there were any problems with feeding in
argy. Young infants who were initially jaundiced and the first few weeks of life.
sleepy are sometimes slower to establish feeding routines. • Ask about the frequency of complementary feeds and
• Ask about the mother’s health in pregnancy, the birth, snacks in children over 6 months of age. By 10 months
and delivery. of age, children should be taking three meals and two
• Check the mother’s antenatal record and make sure that snacks per day.
she was screened for infections including HIV. Ask about • Ask about the consistency of the complementary feeds.
any infections in the family, including TB. Some children are offered very dilute complementary
feeds as their parents are concerned about them choking.
What to Look for on Examination • Ask about the quantity of feeds taken and whether they
are only taking a mouthful or managing to finish a small
• Check the infant thoroughly for symptoms and signs bowl of food.
suggestive of a serious infection. If you have any concerns • Find out what milk the child is taking. Ideally breastfeed-
about the infant’s general health refer to secondary care. ing should continue after the introduction of comple-
• Plot weights on an appropriate chart (if the infant was mentary feeds.
preterm adjust for gestational age). Use growth charts • Ask about other symptoms the child may have, including
based on the World Health Organization growth charts fever and dysuria, suggestive of a UTI.
(including the charts currently used in the United • Ask if the child has abdominal pain suggesting a possible
Kingdom) that show the growth of children who have gastrointestinal problem (e.g., coeliac disease).
been optimally fed from birth. • Ask about stool patterns and whether the child has con-
• Check the infant’s mouth for thrush and the napkin area stipation (children with constipation often have poor
for rashes. appetites) or diarrhoea.
• Check the infant’s tone. Infants who are floppy with • Explore whether the child has been drinking more than
poor tone should be referred to secondary care. usual or passing large quantities of urine, as diabetes
• Auscultate the heart and check for a heart murmur. mellitus (DM) may be the cause of weight loss.
• Check for signs of a respiratory problem including • Ask whether the child has a chronic cough (suggestive
indrawing, tachypnoea, and nasal flaring of a chronic respiratory condition) or if the child snores
at night with interruptions in breathing (suggestive of • If DM is suspected, check a capillary blood glucose
obstructive sleep apnoea). and refer/discuss immediately if above 8 mmol/l and/
• Check that the mother was tested for HIV in pregnancy. or symptomatic of DM.
• Explore whether there are concerns about the child’s • Treat constipation.
psychosocial well-being and if there have been previous • If you suspect the child has obstructive sleep apnoea,
social work concerns. then refer to an ENT (ears, nose, throat) surgeon.
• If you have concerns about neglect or non-accidental
What to Look for on Examination injury, refer to secondary care and contact the social
work department.
• Weigh and measure the child, including the OFC, plot • Refer children whom you think may have underlying
on an appropriate chart, and compare to previous mea- pathology for further investigations.
surements. Check if the child is tracking appropriately
along his or her centile line. If the child’s weight and
height have always been on the 0.4th centile since birth,
and the child is well, then there is no failure to thrive The Child Who Is Suspected of Having
and the child has always been small. a Non-accidental Injury
• When assessing a child’s weight, a rough rule of thumb is:
• a child doubles birth weight by 4 to 5 months; • Children can sustain a multitude of injuries during normal,
• a child triples birth weight by 12 to 13 months. active play. The challenge is to identify those who are at
• Examine for pale conjunctivae and palmar creases risk of non-accidental injury, and children whose clini-
(suggestive of iron deficiency). cal presentation or history is suggestive of non-accidental
• Examine for loose skin folds (suggestive of recent injury.
weight loss). • A thorough history and examination is essential.
• Assess whether the child looks neglected, if the child is • Information from other professionals, including health
dirty and ill-kempt, and if there is severe napkin dermatitis. visitors, social workers, and school personnel, who know
• Examine the abdomen for masses, including faecal the family is useful in identifying vulnerable children.
masses, and hepatosplenomegaly.
• Examine the respiratory system to assess if the child has What to Ask About the Child
chest signs suggestive of a chronic respiratory problem.
• Examine the tonsils and assess whether the child is a • Obtain a detailed history of the injury:
mouth breather. Children with obstructive sleep apnoea • Was it a witnessed fall or trauma?
are often poor eaters with weight faltering. • Is the history describing how the injury occurred con-
• Examine whether the child has any signs suggestive of sistent with the clinical findings?
non-accidental injury, including bruises, and assess how • When did the injury occur and has there been a delay
the parent/caregiver interacts with the child. Observe in presentation to medical services?
how the child behaves during the consultation (i.e., • Explore the past medical history, including the peri-
acting withdrawn or active) and how the caregiver con- natal history. Have there been previous injuries, ill-
trols the child. nesses, visits to the hospital emergency department?
• Does the child have a history of bleeding or easy
What to Do bruising and is there a family history of bleeding
disorders?
• If the problem is a dietary one then address this. • Take a detailed social history including who lives in
• If possible a home visit by a health visitor may be helpful. the house, who has contact with the child/children,
• Give clear counselling around the quantities and fre- and whether previous concerns have been raised about
quency of foods to be offered. the family from the social work department.
• Explain that the child should not be forced to feed but • Ask about the other siblings and how they are growing
should be fed responsively. and developing.
• All children over 1 year of age in the United Kingdom • Explore whether there are any recent events in the
should take a multivitamin supplement (e.g., Healthy family that could have caused stress, including finan-
Start Vitamins). cial difficulties or separation of the parents.
• Always encourage a mother who is breastfeeding to con-
tinue. It is easy for her to lose confidence in the value of What to Look for on Examination
her breastmilk if her child has weight faltering.
• Further management depends on the history and • Assess whether the child is thriving. Measure the child and
examination: plot his or her weight and height on appropriate charts.
• It is useful to obtain a clean catch urine for urinalysis • Observe whether the child is clean and appropriately
+/− culture to exclude a UTI or glycosuria (DM). dressed or unkempt, dirty, and inappropriately clad.
ALGrawany
• Examine for features of potential neglect, including Behrman, R., & Kliegman, R. (2002). Pediatric decision making strate-
untreated skin conditions, severe nappy rash, and lice. gies. Philadelphia: WB Saunders Company.
• Assess any injuries and document these carefully and Bronzetti, G., & Corzani, A. (2010). The seven “S” murmurs: an allit-
consider whether the injuries are in keeping with the eration about innocent murmurs in cardiac auscultation. Clinical
Pediatrics, 49(7), 713.
history of how they occurred.
Cramer, K., & Scherl, S. (2004). Orthopaedic surgery essential, pediat-
• Injuries of concern: rics. Philadelphia: Lippincott Williams and Wilkins.
• Bruises in the shape of fingers, grip marks, or possibly Raine, J., Donaldson, M., Gregory, J. W., & Van Vliet, G. (2011). Prac-
caused by an implement tical endocrinology and diabetes in children. Oxford: Wiley-Blackwell.
• Any injury in a non-mobile child, unless it was an
Guidelines
accident (e.g., a parent falling whilst holding the child)
British Guideline on the Management of Asthma. (2011). Quick refer-
• Burns or scalds which appear to have been caused by ence guide. Retrieved from http://www.sign.ac.uk/pdf/qrg101.pdf;
direct contact being applied to the skin, including http://www.brit-thoracic.org.uk.
immersion in hot water or a cigarette burn British Guidelines on the Management of Community Acquired
• A torn frenulum in the mouth of a young infant Pneumonia in Children. (2011). Retrieved from https://www.brit
• Injuries on non bony prominences of the head, neck, -thoracic.org.uk/standards-of-care/guidelines/bts-guidelines-for
back, or buttocks -the-management-of-community-acquired-pneumonia-in
• Multiple, unexplained lacerations, abrasions, or scars -children-update-2011/.
• In young infants, feel the anterior fontanelle. An infant National Institute for Health and Care Excellence. (2013). Clinical
who has been shaken may have a tense, bulging fonta- knowledge summary. Balanitis in children. Retrieved from http://
nelle and signs of raised intracranial pressure (irritability, cks.org.uk/balanitis.
National Institute for Health and Care Excellence. (2013). Diarrhoea
crying, vomiting, poor feeding).
and vomiting in children under 5. NICE clinical guideline 84.
• Assess the child’s emotional state and interaction with Retrieved from www.nice.org.uk.
the parents or caregivers. Is the child wary (frozen watch- National Institute for Health and Care Excellence. (2013). Feverish
fulness) or is parental hostility or detachment noted? illness in children. NICE clinical guideline 160. Retrieved from
www.nice.org.uk/guidance/cg160/resources/support-for
What to Do -education-and-learning-educational-resource-traffic-light-table
-pdf-189985789.
• Any child who presents with an unexplained injury, National Institute for Health and Care Excellence. (2013). Nocturnal
unexplained delay in presentation, or whose history is enuresis—the management of bedwetting in children and young
inconsistent with the presentation should be referred for people. NICE clinical guideline 111. Retrieved from www.nice.org.uk.
assessment. Local guidelines will dictate further manage- National Institute for Health and Care Excellence. (2013). Urinary
tract infection in under 16s: Diagnosis and management. NICE
ment, but usually this includes:
clinical guideline 54. Retrieved from https://www.nice.org.uk/
• an assessment by a senior paediatrician (including guidance/cgG54.
examination and history from the referrer and accom- National Institute for Health and Care Excellence. (2013). When to
panying adult); suspect child maltreatment. NICE clinical guideline 89. Retrieved
• consent for taking clinical photographs of any injuries; from www.nice.org.uk.
• investigations (blood tests, cranial imaging, x-rays); Royal Children’s Hospital. (2013). The management of vulvovaginitis.
• liaising with other professionals (social workers, Melbourne: Royal Children’s Hospital. http://222.rch.org.au.
police) as required on an individual case basis. Resources
• If you have concerns about any child whom you feel is The Enuresis Resource and Information Centre (ERIC). www.eric
being neglected, discuss the case with a social worker and .org.uk.
senior paediatrician. World Health Organization. (2009). Infant and young child feeding:
Model chapter for textbooks for medical students and allied health profes-
References sionals. Retrieved from www.who.int/maternal_child_adolescent/
documents/9789241597494/en/.
Books
Beattie, J., & Carachi, R. (Eds.), (2005). Practical paediatric problems.
A textbook for MRCPCH. London: Hodder Arnold.
7
Cardiovascular Problems
DAVID NICHOLAS BLANE
Hypertension Chronic Heart Failure (CHF) With Left Ventricular
Detection Dysfunction
Blood Pressure Targets ACE Inhibitors/Angiotensin-II Receptor Blockers
Beta-Blockers
Practicalities of Blood Pressure Measurement
Diuretics (Other Than Spironolactone)
Workup
Second Line Pharmacologic Management
Nondrug Treatment Mineralocorticoid Receptor Antagonists
Drug Management Ivabradine
Primary Prevention Digoxin
Antihypertensives Heart Failure With Preserved Ejection Fraction (HF-PEF)
Drug Treatment of Patients With Other Medical Problems Grounds for Admission
Other Points About the Drugs Referral for Specialist Review
Thiazides End-Stage Heart Failure
Calcium Channel Blockers Breathlessness
ACE Inhibitors and Related Drugs Weakness and Fatigue
Beta-Blockers Anorexia/Nausea
Alpha-Blockers Oedema
Poor Control
Acute Pulmonary Oedema
Resistant Hypertension
Right Heart Failure
Referral Acute Right Heart Failure
Chest Pain of Recent Onset Chronic Cor Pulmonale
Unstable Angina (Acute Coronary Syndrome) Palpitations and Arrhythmias
History
Myocardial Infarction (MI)
Examination
Contraindications to Thrombolysis
Investigations
Cardiac Rehabilitation Next Steps
Other Factual Advice Ectopic Beats in a Normal Heart
Stable Angina Atrial Fibrillation
Referral Identification of Atrial Fibrillation
Management Cardioversion Versus Rate Control
Drug Treatment of Stable Angina Risk Stratification for Anticoagulation
Cardiovascular Risk Reduction Assessment of Bleeding Risk
Selecting Drugs Anticoagulation
Revascularization Dabigatran
Noncardiac Chest Pain Rivaroxaban and Apixaban
Heart Failure Left Atrial Appendage Closure
Diagnosis Paroxysmal Atrial Fibrillation
Serum Natriuretic Peptides Catheter Ablation
How to Use B-Type Natriuretic Peptide Anticoagulation
Management of Confirmed Chronic Heart Failure Atrial Flutter
General Measures Paroxysmal Supraventricular Tachycardia
65
Ventricular Tachycardia Talking to the Patient About Absolute Risks When Taking a
Sick Sinus Syndrome Statin
Bradycardia Secondary Prevention
Prophylaxis of Infective Endocarditis Annual Workup at a Coronary Heart Disease Prevention
Clinic
The Prevention of Cardiovascular Disease
Familial Hypercholesterolaemia
Primary Prevention
Lifestyle Changes
Intensive Management When a 10-Year Cardiovascular
Disease Risk of At Least 10% Is Detected
Lipid Lowering
Blood Pressure Control
Hypertension • BOX 7.1 Definition of Hypertension

Stage 1 Hypertension
GUIDELINE
• Clinic BP ≥140/90 mm Hg and subsequent ABPM or HBPM
National Institute for Health and Care Excellence. (2011a). ≥135/85 mm Hg
Hypertension: Clinical management of primary hypertension
in adults (update). NICE clinical guideline 127. Available at Stage 2 Hypertension
http://guidance.nice.org.uk/CG127. • Clinic BP ≥160/100 mm Hg and subsequent ABPM or
HBPM ≥150/95 mm Hg
Severe Hypertension
• Clinic BP ≥180/110 mm Hg
• Hypertension is one of the most common conditions Note: Where a threshold or target level of blood pressure is given (e.g.,
treated in primary care in the United Kingdom and one 160/100 mm Hg), it means that action should be taken if the systolic is 160 or
of the most important preventable causes of death world- over OR the diastolic is 100 or over.
ABPM, Ambulatory blood pressure monitoring; HBPM, home blood pressure
wide (Krause et al., 2011). It is the main risk factor for monitoring.
development of stroke and ischaemic heart disease and Source: National Institute for Health and Care Excellence. (2011).
is strongly associated with the development of chronic Hypertension: Clinical management of primary hypertension in adults (update).
NICE clinical guideline 127. Available at http://guidance.nice.org.uk/CG127.
kidney disease and cognitive impairment.
• Hypertension, defined as the presence of persistently
raised blood pressure at or greater than 140/90 mm Hg
(Box 7.1), affects more than a quarter of all UK adults
and over half of those aged 65 years or more (Health and Detection
Social Care Information Centre, 2011). The adult preva-
lence of high blood pressure is even higher in other parts • All adults should have their blood pressure measured at
of the world, with over 40% of adults affected in Africa, least every 5 years up to the age of 80 and at least annu-
for example (World Health Organisation [WHO], n.d.). ally thereafter (Hodgkinson et al., 2011).
• Most people with hypertension have no symptoms or • The 2011 NICE guidelines recommended a major shift in
clinical findings on examination and their hypertension how blood pressure measurements are taken and hyper-
is identified incidentally or as a result of complications tension diagnosed, centering on the use of ambulatory
such as angina, myocardial infarction (MI), stroke, and (ABPM) and home (HBPM) blood pressure monitoring
arrhythmias. to complement clinic measurements (Box 7.2). This is in
• The rule of halves was described in the United States by part a response to the overtreatment of people with so-
Wilber and Barrow in 1972. They stated that half of hyper- called white coat hypertension.
tensives are not known to have a raised blood pressure • The guidelines recommend the following steps to diag-
(BP); of those with known hypertension, half are not on nose hypertension:
treatment and half of those on treatment are poorly con- 1. If a clinic BP is over 140/90 mm Hg, take a second
trolled. The figures for detection and treatment of hyper- reading in the consultation.
tension have improved in recent years, but this remains a 2. If the second reading is very different from the first,
useful reminder of the challenge posed by hypertension. take a third reading.
CHAPTER 7 Cardiovascular Problems 67
• BOX 7.2 Definitions of Ambulatory Blood • Note that in patients with type 2 diabetes, a stricter target
Pressure Monitoring and Home Blood of less than 140/80 mm Hg is the aim (<135/75 mm Hg
Pressure Monitoring in those with microalbuminuria).
• Three points should be made about these targets:
ABPM is a noninvasive method of measuring blood pressure in a 1. Any reduction in blood pressure carries benefit, even
patient’s own environment. At least two measurements per hour
should be taken during the patient’s usual waking hours (e.g.,
if the target is not reached (Czernichow et al., 2011).
between 8 am and 10 pm). The readings are taken automatically 2. The lower the blood pressure the greater the benefit
to minimize interference with everyday activities and sleep (Ettehad et al., 2016).
patterns. By taking regular readings throughout the day, a more 3. These BP targets may change in light of more recent
accurate estimate of blood pressure can be obtained. research recommending more intensive BP lower-
The average value of at least 14 measurements is needed to
confirm a diagnosis of hypertension. Patients are most commonly
ing for high risk patients (The SPRINT Research
referred to appropriately equipped hospital clinics, though it is Group, 2015)
possible for general practitioners to establish their own system of
ambulatory blood pressure monitoring.
Home blood pressure monitoring requires the patient to Practicalities of Blood Pressure Measurement
measure his or her own blood pressure using an automatic
blood pressure monitor, either supplied by the local health
1. The patient should be seated, but in older patients and
service or purchased privately. To confirm a diagnosis of in patients with diabetes check the blood pressure both
hypertension it is advised that: standing and sitting.
1. two consecutive measurements are taken at least 1 min 2. On the first occasion measure the BP in both arms. A
apart, twice daily (ideally in the morning and evening), with significant difference is found in 20% of hypertensives. If
the patient seated, for 4–7 days;
2. measurements taken on the first day should be discarded
there is a difference of more than 5 mm Hg, then use the
and the average value of all remaining measurements should arm with the higher reading for future measurements.
be used. 3. Measure the systolic and diastolic pressures to the nearest
2 mm Hg. If over 140/90 mm Hg, repeat the measure-
ment toward the end of the consultation. If markedly
different from each other, take at least one more. Take
the average. Repeated readings by a nurse give the most
3. Record the lowest reading; if it is over 140/90 mm Hg, reliable clinic results, occasional readings by a doctor the
offer 24-hour ABPM to confirm the diagnosis (Hodg- least (Little et al., 2002).
kinson et al., 2011). 4. Timing. In mild uncomplicated hypertension, do not
4. HBPM should be used as an alternative if ABPM start treatment until three readings have been taken over
is declined or not tolerated, or for practices that a 3-month period. About 25% of blood pressures will
lack ABPM equipment (Ritchie, Campbell, & settle in that time. Those that settle to below treatment
Murchie, 2011). levels need lifelong annual follow-up. If the initial dia-
• There is a growing evidence base for the use of self- stolic is over 200/110 mm Hg or there is evidence of
monitoring (and, indeed, self-management) of BP by end organ damage, cardiovascular disease, or diabetes,
patients (Uhlig, Patel, Ip, Kitsios, Balk, 2013). Guide- three readings over 2 weeks would be more appropriate.
lines may change to reflect this in the future. Consider immediate treatment if the pressure is over
• Other recent research has suggested that blood pres- 220/120 mm Hg.
sure should be measured in both arms and that subse- 5. Follow-up 6 monthly, once the patient is established on
quent BP monitoring should be done in the arm with treatment. It is as good as monthly.
the highest reading (Clark, Taylor, Shore, & Campbell,
2012; Clark, Taylor, Shore, Ukoumunne, & Campbell, Workup
2012). If a BP difference of over 10 mm Hg is found,
peripheral artery disease is likely and further evaluation 1. Check for a history of family and personal risk factors
(e.g., ankle-brachial pressure measurement) is warranted. for stroke or coronary heart disease. Check whether rel-
evant drugs (e.g., nonsteroidal antiinflammatory drugs
Blood Pressure Targets [NSAIDs]) or excess alcohol are taken.
2. Examination, including:
• The target of hypertension treatment is to reduce clinic • fundi (essential only in severe hypertension) (van den
blood pressure levels to below 140/90 mm Hg in people Born, Hulsman, Hoekstra, Schlingemann, & van Mont-
aged under 80, and below 150/90 mm Hg in people frans, 2005);
aged 80 and over. • femoral pulses;
• Previous guidance focused on treating those aged under • palpation of kidneys and auscultation for presence of
80, but more recent evidence has shown that treatment bruit;
is well tolerated and reduces total mortality and cardio- • signs of left ventricular hypertrophy.
vascular events (Beckett et al., 2008, 2011). 3. Urinalysis for protein and blood
4. Blood: 6. Dietary Approaches to Stop Hypertension (DASH) diet:

• Creatinine and electrolytes This is a diet rich in fruit, vegetables, and oily fish and
• Fasting blood sugar low in sodium and total and saturated fats. It has been
• Serum lipids shown in a number of trials to reduce BP by up to
5. Look for left ventricular hypertrophy using electrocar- 11/5 mm Hg (Appel et al., 1997). It is similar to a
diogram (ECG) and chest x-ray (CXR). Mediterranean-style diet (Sacks & Campos, 2010).
6. Calculate the patient’s 10-year cardiovascular disease 7. Coffee: Coffee is known to acutely raise BP, but a 2012
(CVD) risk using a recognized calculator (e.g., the systematic review and meta-analysis found no significant
QRISK®2 cardiovascular disease risk calculator, available effect on BP or the risk of hypertension (Steffen, Kuhle,
at http://www.qrisk.org/). A 20% 10-year CVD risk Hensrud, Erwin, & Murad, 2012). However, standard
means that the lower threshold for treatment applies and advice remains to discourage excessive coffee drinking.
that primary prevention of CVD is indicated (see section 8. Contraceptive pill: Consider stopping but not until other
below on Primary Prevention). adequate contraceptive measures are in place.
9. Stress: The relationship between stress and blood pressure
Nondrug Treatment is not well understood, yet anecdotally patients often
blame a stressful life for their hypertension. This is an
Nondrug treatment can lower the systolic pressure by 4 to area of ongoing research, with some promising results for
10 mm Hg (Stevens, Obarzanek, & Cook, 2001; Writing stress reduction interventions (Hughes et al., 2013).
Group of the PREMIER Collaborative Research Group,
2003). It lowers the risk of CVD and should be offered
Drug Management
to all with hypertension, whether or not drugs are being
prescribed. Primary Prevention
Consider the following: • Aspirin: The use of low-dose aspirin (75 mg daily) in
1. Smoking: Ask about smoking. If appropriate offer advice primary prevention is controversial, with ongoing debates
and refer to the smoking cessation services. Stopping will about benefits (reducing cardiovascular events) versus
not reduce the BP but it will lower the cardiovascular risk. risks (bleeding events) (Barnett, Burrill, & Iheanacho,
2. Exercise. Physical activity lowers the risk of developing 2010). At the time of writing, aspirin is not licensed for
hypertension and is an effective treatment for those with primary prevention in the United Kingdom and should
established hypertension. Brisk walking for 30 minutes not be routinely started, even in those with risk factors
every day is as beneficial as more vigorous exercise three such as hypertension and diabetes (Scottish Intercolle-
times a week. After only 2 weeks of aerobic exercise, the giate Guidelines Network, 2010).
mean fall in blood pressure is 5/4 mm Hg (Whelton, • Statins: Give statins if the patient has CVD or diabetes
Chin, Xin, He, 2002). or has a risk of CVD that is sufficiently high. SIGN
3. Weight: Encourage weight loss if overweight (body mass (2017) recommends a risk threshold of CVD of at or
index [BMI] >25 kg/m2). Overweight is a significant and greater than 20% in the next 10 years as an indication
independent predictor of the level of BP (Cox et al., for the introduction of statin therapy; however, the
1996). A 10-kg weight loss promotes a reduction of 5 to NICE guidance from 2014 suggests consideration of
20 mm Hg (Chobanian, 2003). primary prevention strategies in those with over a 10%
4. Alcohol: There is a direct dose–response relationship between risk in the next 10 years, if lifestyle measures have not
alcohol intake and risk of hypertension, particularly when proved effective.
alcohol intake exceeds two drinks per day (Xin et al., 2001).
Support patients to reduce excessive consumption. Antihypertensives
5. Salt: Reduce intake of salt to less than 5.8 g/day or less • About half of patients fail to take their antihypertensives
than 2.4 g sodium. A 2011 Cochrane review was unable as prescribed. Patients have many reservations about
to confirm whether reducing dietary salt had significant drug treatment (Benson & Britten, 2002). Getting them
effects on mortality or cardiovascular morbidity (Taylor, to voice those reservations gives clinicians a chance to
Ashton, Moxham, Hooper, & Ebrahim, 2011) but a alter any erroneous ideas they may have. A study of black
subsequent meta-analysis found a significant reduction Caribbean patients in London found these common
in cardiovascular events (He & MacGregor, 2011), sup- misconceptions (Connell, McKevitt, & Wolfe, 2005):
porting long-standing public health recommendations to • Once the BP was controlled, they were cured and
reduce salt consumption in the population. Common didn’t need the medication.
sources of salt are nuts, crisps, canned foods, table sauces, • They could sense when their BP was raised and so
and bread. Many processed foods are high in salt, so could judge when they needed to take the medication.
avoid those with over 1.5 g per 100 g food. Also note • Commonly used drugs produce a similar average fall of
that many labels use sodium rather than salt content: 1 g 9.1/5.5 mm Hg at standard doses (Law, Wald, Morris,
sodium = 2.5 g salt, so more than 0.6 g sodium per & Jordan, 2003). Most patients with hypertension there-
100 g food is high. fore need more than one antihypertensive drug.
• Combining two drugs from different classes reduces the A 2012 Cochrane review of Randomised Controlled
blood pressure five times more than doubling the dose Trials (RCTs) of treatment of mild hypertension in those
of one drug (Wald et al., 2009). without preexisting cardiovascular disease found that treat-
• The timing of medication may also be important. There ment did not reduce morbidity or mortality (Diao, Wright,
is a morning rise in blood pressure, in keeping with Cundiff, & Gueyffier, 2012).
circadian rhythms. A Cochrane review found that advis- • Offer step 1 treatment to people of any age with stage 2
ing patients to take their antihypertensives in the evening hypertension.
resulted in small gains in 24-hour BP reduction (mean • Offer people aged under 55 years an ACE inhibitor or a
of 1.7/1.4 mm Hg) (Zhao, Xu, Wan, & Wang, 2011). low-cost angiotensin receptor blocker (ARB). If an ACE
It is unclear, however, if this translates to a reduction in inhibitor is prescribed and not tolerated (e.g., due to
CV events. cough), offer a low-cost ARB.
• The choice of drugs should be influenced by age, comor- • Offer people aged over 55 years and black people of
bidity, adverse effects, possible synergistic effects between African or Caribbean family origin of any age a calcium
classes of drugs, and the individual’s response to each channel blocker (CCB). If a CCB is not suitable (e.g.,
drug. Ethnic origin also influences the choice of medica- due to oedema or intolerance), or there is evidence/risk
tion; younger white patients tend to have high levels of heart failure, offer a thiazide-like diuretic.
of renin and angiotensin II; older patients and those
of African origin tend to have low renin levels and Drug Treatment of Patients With Other
so respond less well to drugs that block the renin– Medical Problems
angiotensin system, although the differences are thought
to be small. Most individuals (approximately four of five individuals
• Follow the scheme in Table 7.1, but do not persevere in one study [Barnett et al., 2012]) with hypertension will
with a drug that has produced no benefit (i.e., a drop in have additional chronic diseases.
BP of <5 mm Hg). Instead, switch to a drug with a dif- 1. Angina: Use a beta-blocker or a calcium channel blocker.
ferent mode of action (e.g., from A to C or D). Persevere 2. Heart failure: Use a diuretic, an ACE inhibitor, a beta-
with a drug that shows some but inadequate benefit; it blocker, then an alpha-blocker.
may be synergistic with a drug from a different group. 3. Diabetes: Use an ACE inhibitor, a low-dose thiazide, an
• Divide drugs into those that suppress the renin system alpha-blocker, or calcium channel blocker.
(A [ACE inhibitors and angiotensin receptor blockers]) 4. Smokers: Do not use a beta-blocker.
and those that work independently of it (C [calcium 5. Migraine: Use a beta-blocker and/or calcium channel
channel blockers] and D [diuretics]) (Brown et al., blocker, then all other alternatives.
2003). The following steps are recommended, but tailor 6. Those active in sports: Avoid beta-blockers.
them to the needs of the individual patient. 7. Raynaud syndrome: Use a calcium channel blocker.
• Offer step 1 treatment to people aged under 80 8. Renal failure: Get specialist advice. ACE inhibitors may
with stage 1 hypertension and one or more of the improve renal function but will need to be given in
following: lower dosage.
• Target organ damage 9. Gout: Avoid thiazides.
• Established cardiovascular disease 10. Asthma: Avoid beta-blockers.
• Renal disease
• Diabetes
Other Points About the Drugs
• 10-year cardiovascular risk equivalent to 20%
or more Thiazides
• Indapamide (2.5 mg daily) or chlorthalidone (12.5–
25 mg daily) has been suggested by NICE to be prefer-
ential to bendroflumethiazide.
TABLE
Drug Management in Hypertension • Recheck creatinine and electrolytes 1 month after
7.1
starting a thiazide then repeat annually. Repeat more
Age <55 and frequently if the patient is unwell or is taking digoxin or
Non-Black Age ≥55 or Black another drug that might affect renal function. A rise of
Step 1 A C or D creatinine of up to 30% is acceptable provided it remains
less than 200 µmol/L (Martin & Coleman, 2006).
Step 2 A and C or D • Diabetes is not a contraindication. In the Systolic Hyper-
Step 3 A and C and D tension in the Elderly Program (SHEP) study, a thiazide
Step 4 Add spironolactone, or, if not tolerated, an was associated with the development of diabetes in an
alpha-blocker, or a beta-blocker, or extra 4.3% of patients over 4 years, but it protected those
another diuretic, or refer who developed diabetes against an increase in cardiovas-
cular mortality (Kostis et al., 2005).
• Ask specifically about erectile difficulties in men. The • Warn the patient not to stop a beta-blocker suddenly.
incidence in patients on thiazides is double that in those Even those without known coronary heart disease (CHD)
on placebo (17% versus 8%), but patients rarely volun- have a fourfold increased risk of myocardial infarction or
teer this information. angina in the subsequent 4 weeks.
Calcium Channel Blockers Alpha-Blockers

• Use a long-acting preparation (e.g., diltiazem 120–180 mg • Total cholesterol falls by an average of 4%, with
slow release twice a day or verapamil 120–240 mg twice a beneficial rise in high-density lipoprotein (HDL)
a day or 240–480 mg slow release daily). Their hypo- cholesterol.
tensive effect is the same as nifedipine, with fewer side • Start with a low dose (e.g., terazosin 1 mg or doxazosin
effects. Use brand names. Different generic products 1 mg), taken at night in case of first-dose hypotension.
have different bioavailabilities. • Use with caution in the elderly, who may experience
• Use a dihydropyridine calcium channel blocker (e.g., continued postural hypotension.
nifedipine slow release or amlodipine), if:
1. beta-blockers are also being given; Poor Control
2. there is peripheral vascular disease with skin isch- • Gently ask about adherence. A question such as, “How
aemia; it will not, however, help intermittent difficult do you find it to take all of your tablets?” is more
claudication; likely to elicit a truthful answer than “Do you ever forget
3. there is a risk of heart failure, which might be wors- to take them?”
ened by a non-dihydropyridine. • Consider the white-coat effect. If the BP seems to fluctu-
ate or the patient seems tense, arrange for home readings
ACE Inhibitors and Related Drugs (see Box 7.2).
• Starting them: • If neither of these applies, consider this to be resistant
1. The first dose should be taken at night. Even then, hypertension.
first-dose hypotension due to once-daily agents may
not occur until 6 to 8 hours after the first dose, and Resistant Hypertension
may last for 24 hours.
2. Recheck serum creatinine and electrolytes 1 week • Resistant hypertension is common, being found in
after starting the drug, and after any dose increase, approximately 10% of all treated patients.
then annually. • It is defined as hypertension not controlled by three
• A rise of serum creatinine of less than 30% is accept- drugs, at best tolerated doses, where the patient is
able provided it remains under 200 µmol/L (Martin & taking them and the BP is raised at home as well as at
Coleman, 2006). A greater rise suggests renal artery ste- the clinic.
nosis or chronic kidney disease. Above that, reduce or • It is important, as patients with resistant hypertension
stop the drug and recheck weekly till the creatinine has are very high risk: They are 50% more likely to experi-
returned to its previous level. Look for underlying renal ence an adverse cardiovascular event compared to other
disease. hypertensive patients (Myat, Redwood, Qureshi, Spertus,
• A rise of serum K+ to 5.5 to 5.9 mmol/L is acceptable & Williams, 2012).
but recheck more frequently. Stop the drug if the level • Before a diagnosis of resistant hypertension can be made,
reaches 6 mmol/L and refer (WHO, n.d.). check that this is true resistance, not poor adherence or
• Use them in patients with insulin-dependent diabetes; white-coat hypertension
they improve insulin resistance (whereas thiazides and • New evidence suggests adding spironolactone as the 4th
beta-blockers may worsen it). line medication (after A + C + D) in patients with an
• Avoid them in patients with peripheral vascular eGFR >45 ml/min (Williams et al., 2015)
disease. • Resistant hypertension is likely to be multifactorial.
• Use an angiotensin II receptor antagonist in patients who Consider the following:
need an ACE inhibitor but cannot tolerate it because of 1. Lifestyle factors: obesity, excees alcohol intake, excess
cough. dietary sodium, cocaine and amphetamines misuse; of
these, obesity is the most common feature of patients
Beta-Blockers with resistant hypertension. One study of over 45,000
• Perform a peak flow before and after starting treat- primary care patients in Germany found that obese
ment if the history suggests the possibility of chronic individuals (BMI >40 kg/m2) were more than five
obstructive pulmonary disease (COPD). If there is a times more likely to need three antihypertensive drugs
significant fall, stop the drug. However, cardioselective and three times more likely to require four antihyper-
beta-blockers may be tolerated in mild to moderate tensive drugs to achieve BP control compared with
reversible airway obstructions (Salpeter, Ormiston, & individuals with a normal BMI (≤25 kg/m2) (Sharma
Salpeter, 2005). et al., 2004).
2. Medication-related causes: NSAIDs, selective COX-2 Chest Pain of Recent Onset

inhibitors, steroids, sympathomimetics (e.g., decon-
gestants), oral contraceptives, and liquorice ingestion
in sweets or chewed tobacco. GUIDELINE
3. An underlying medical cause: Up to 10% of these National Institute for Health and Care Excellence. (2010a).
patients have a previously undiagnosed secondary Chest pain of recent onset. NICE clinical guideline 95.
Available at www.nice.org.uk.
cause:
• Renal disorders: the commonest overall cause
and least amenable to treatment. Includes dia-
betic kidney disease, glomerulonephritis, chronic • Chest pain is very common, accounting for about 1% of
pyelonephritis, obstructive uropathy, and polycys- all patient encounters in general practice, 5% of visits to
tic kidney disease. the emergency department, and 25% of all emergency
• Primary hyperaldosteronism: the commonest single hospital admissions (Goodacre, 2005). These encounters
cause of secondary hypertension, accounting for represent just a fraction of the number of episodes of
5% to 13% of all cases of hypertension (Grasko, chest pain experienced in the community, for which
Nguyen, & Glendenning, 2010). Suspect if there is medical attention is not always sought (Elliott, McAteer,
low potassium (K) and high sodium (Na), although & Hannaford, 2011).
in many patients these will be normal. The ratio • The challenge for GPs is to identify serious cardiac
of plasma aldosterone to renin will be raised and disease while also protecting patients from unnecessary
warrants referral to confirm the diagnosis and the investigations and hospital admissions.
underlying cause (Conn adenoma or idiopathic). • The 2010 NICE guidelines focus on the assessment and
Note that spironolactone, eplerenone, amiloride, diagnosis of recent onset chest pain or discomfort of
and dihydropyridine calcium channel blockers (e.g., suspected cardiac origin. They present two separate diag-
amlodipine) should be stopped before doing these nostic pathways: one for people with acute chest pain in
tests (Grasko et al., 2010). whom an acute coronary syndrome is suspected; the
• Obstructive sleep apnoea (OSA): ask about other for those with intermittent stable chest pain in
snoring, episodes of apnoea at night, and daytime whom stable angina is suspected (Cooper et al., 2010).
sleepiness. Medical history-taking and physical examination will
• Vascular disorders: coarctation of the aorta determine which of these pathways to follow, as will be
(suspect if significant interarm difference in blood described in the following sections.
pressure). Check for radio-radial or radio-femoral
delay; and renal artery stenosis (common in older Unstable Angina (Acute
hypertensives). Suspect if evidence of peripheral Coronary Syndrome)
vascular disease. Check for abdominal bruit.
• Thyroid diseases: hyperthyroidism usually increases
systolic blood pressure, whereas hypothyroidism GUIDELINES
usually increases diastolic blood pressure. Scottish Intercollegiate Guidelines Network. (2016). Acute
• Cushing disease: look for the typical clinical features coronary syndrome. SIGN publication 148. Edinburgh: SIGN.
(e.g., centripetal obesity, moon facies, abdominal Available from http://www.sign.ac.uk.
National Institute for Health and Care Excellence. (2010).
striae). Unstable angina and NSTEMI: The early management of
• Phaeochromocytoma: suggested by a history of unstable angina and non-ST-segment-elevation myocardial
episodic headaches, sweating, palpitations associ- infarction. NICE clinical guideline 94. Available from http://
ated with an often dramatic rise of blood pressure. www.nice.org.uk/guidance/CG94.
Check 24-hour urinary catecholamines.
• If a cause for resistance is found that can be managed in
primary care, continue management, even if it means • The definition of acute coronary syndrome (ACS), which
proceeding to step 4. Otherwise, refer. Hypertension includes unstable angina and myocardial infarction,
clinics are capable of controlling half of those referred depends on the specific characterstics of each element of
with resistant hypertension. the triad (SIGN, 2016):
• Clinical presentation (including a history of coronary
Referral artery disease)
• ECG changes
• Refer patients in whom there is reason to suspect second- • Biochemical cardiac markers
ary hypertension: onset age under 40, fluctuating BP • Symptoms and signs that may indicate an acute coronary
levels, evidence of renal disease, resistant hypertension. syndrome include the following:
• Refer urgently anyone with accelerated hypertension • Chest pain lasting more than 15 minutes (may be in
(grade IV retinopathy). arms, back, or jaw)
• BOX 7.3 Immediate Management of Suspected • Thrombolysis: make an initial judgment about the patient’s
Acute Coronary Syndrome suitability (see the upcoming contraindications). All
patients with a typical history of MI and ST segment
• Admit by emergency ambulance any patient with cardiac elevation or left bundle branch block (LBBB) should be
pain lasting >15 minutes despite GTN, with an abnormal or
unavailable ECG. Start management immediately but do not
considered for thrombolysis, regardless of age, if their
delay transfer to hospital. quality of life warrants it, and if local policy does not
• While awaiting admission, give: prefer acute percutaneous coronary intervention (PCI).
• pain relief: • Thrombolysis is of benefit in the 24 hours after the onset
1. GTN (e.g., three sprays over 15 minutes) if pain of symptoms, with more benefit the sooner it is given.
continues.
2. Consider IV opioid if available: diamorphine 1 mg/min
Thrombolysis involves hospital admission, whether or
(maximum 5 mg) or morphine 2 mg/min (maximum not it is given at home first.
10 mg). Administer with IV antiemetic such as • GPs need special training. It should be given outside
metoclopramide 10 mg or cyclizine 50 mg. hospital only if:
• aspirin 300 mg (unless allergic), written record of 1. there is strong clinical suspicion of acute myocardial
administration to go with patient;
• 12-lead ECG (fax ahead if possible), but do not do if it will
infarction;
delay hospital transfer; 2. chest pain, unrelieved by GTN spray, has been
• ACS if the ECG is normal; present for at least 20 minutes and for no more than
• pulse oximetry (offer oxygen only if O2 saturation is <94%; 12 hours;
aim for 94%–98%); 3. the ECG shows ST elevation or LBBB;
• patient monitoring until diagnosis.
4. a defibrillator is available, because of the small but
significant increase in risk of ventricular fibrillation
(VF) after thrombolysis;
• Chest pain with associated nausea and vomiting, 5. no contraindications exist.
sweating, breathlessness
• Chest pain associated with haemodynamic instability Contraindications to Thrombolysis
• New onset chest pain, or abrupt deterioration in pre- (Lip, Chin, & Prasad, 2002)
viously stable angina, with recurrent pain occurring Absolute
at rest or at significantly lower levels of activity, or that 1. Aortic dissection
the frequency, duration, or severity of the attacks has 2. Previous cerebral haemorrhage
substantially worsened 3. Known cerebral aneurysm or AV malformation
• Subsequent management depends on timing of presentation: 4. Intracranial neoplasm
• If the patient is presenting with current chest pain, 5. Any cerebrovascular accident in the last 6 months
proceed with immediate management as outlined in 6. Active internal bleeding (other than menstruation)
Box 7.3. 7. Streptokinase should not be given to a patient who has
• If the chest pain was in the last 12 hours, but the received it more than 4 days previously. The development
patient is currently pain free, do an ECG. Arrange of antibodies may reduce its effectiveness.
emergency admission if the ECG is abnormal or if
ECG is unavailable; if ECG is normal, arrange urgent Relative
same-day assessment. 1. Uncontrolled hypertension (BP >180/110 mm Hg) or
• If the chest pain was 12 to 72 hours ago, arrange chronic severe hypertension
urgent same-day assessment. 2. On anticoagulants or known bleeding diathesis
3. Trauma within past 2 to 4 weeks, including head injury
Myocardial Infarction (MI) and prolonged (>10 minutes) CPR
4. Aortic aneurysm
• Defibrillation: defibrillate a patient who develops ven- 5. Recent (within 3 weeks) major surgery, organ biopsy, or
tricular fibrillation while awaiting transfer to hospital. If puncture of a noncompressible vessel
no defibrillator is available, perform cardiopulmonary 6. Recent (within 6 months) GI, or GU, or other internal
resuscitation while waiting for one to arrive. Out-of- bleeding
hospital defibrillation has been shown to save lives. 7. Pregnancy
• Primary PCI: patients with an ST-segment-elevation 8. Active peptic ulcer
acute coronary syndrome should be treated immediately
with primary percutaneous coronary intervention (PCI) Cardiac Rehabilitation
(SIGN, 2016).
• When primary PCI cannot be provided within 120 minutes • Most patients following MI will now routinely be enrolled
of ECG diagnosis, patients with an ST-segment-elevation in a cardiac rehabilitation programme. This should be a
acute coronary syndrome should receive immediate (pre- comprehensive package of support, including exercise,
hospital or admission) thrombolytic therapy (SIGN, 2016). education, and psychologic support.
• Exercise. Encourage the continuation of exercise, which • Do not fly for 2 weeks, and then only if able to climb
will continue to improve cardiac performance and sur- one flight of stairs without difficulty. A more cautious
vival and reduce the risk of another infarction by 20%. policy is to advise against flying for 6 weeks.
Exercise can be tennis, jogging, cycling, swimming, or • Drivers should not drive for 1 month, but no longer are
circuit training. NICE (2013) recommends 20 to 30 required to notify the Driver and Vehicle Licensing Agency
minutes each day, increasing slowly so that it is suffi- (DVLA). They should inform their insurance company.
ciently vigorous to make the patient slightly breathless. Heavy Goods Vehicle (HGV) and Public Service Vehicle
• Smoking. Encourage a smoker to stop, with the news that (PSV) licence holders must notify the DVLA, and may only
stopping even at this late stage probably reduces mortal- continue vocational driving after individual assessment.
ity by 50%.
• Weight. Advise the obese to lose weight. Stable Angina
• Diet. Whether the patient needs to lose weight or not,
recommend a Mediterranean diet. The elements of the GUIDELINES
diet that seem to be associated with a lower mortality are
moderate alcohol, low meat intake, and high intake of National Institute for Health and Care Excellence. (2011b).
Management of stable angina. NICE clinical guideline 126.
vegetables, fruit, nuts, olive oil, and legumes. Available at www.nice.org.uk.
• Alcohol. Check that safe limits are not being exceeded. Scottish Intercollegiate Guidelines Network. (2018).
• Lipids. Arrange a test for serum lipids at 3 months Management of stable angina. National clinical guideline 151.
postinfarction, unless the lipids were assessed within 24 Available at www.sign.ac.uk.
hours of the onset of the infarct. Once blood has been
taken, start a statin regardless of baseline value and con-
tinue it long term. • Angina is the main symptom of myocardial ischaemia
• Diabetes. Unless known to be diabetic, or to have had an and is usually caused by atherosclerotic coronary artery
assessment of glucose metabolism in hospital, check the disease restricting blood flow (and therefore oxygen
fasting plasma glucose. delivery) to the heart muscle.
• Depression. Look again for depression. Even at 1 year, • Stable angina is common, affecting about 8% of men
25% are depressed. and 3% of women aged 55 to 64 years and about 14%
• Other drugs. of men and 8% of women aged 65 to 74 years in England
• Check that the patient continues to take an ACE (O’Flynn et al., 2011). The diagnosis is clinical, based
inhibitor (or an angiotensin-11 receptor blocker if not on the history.
tolerated). • The history is of pain or constricting discomfort that
• A beta-blocker should be taken for at least 12 months typically occurs in the front of the chest, but can radiate
post MI in people without left ventricular dysfunc- to the neck, shoulders, jaw, or arms (NICE, 2011). It
tion or heart failure, unless contraindicated. A beta- is usually brought on by physical exertion or emotional
blocker should be carried on indefinitely in those with stress, but some people can have atypical symptoms
left ventricular dysfunction. such as gastrointestinal discomfort, breathlessness, or
• Dual antiplatelet therapy is usually advised for up nausea.
to 12 months after an MI. This is usually aspirin • Stable angina is unlikely if the pain is:
plus a second antiplatelet drug such as clopidogrel, • continuous or very prolonged;
prasugrel, or ticagrelor. Aspirin is usually carried on • unrelated to activity;
lifelong, clopidogrel can be used for those intolerant • brought on by breathing in;
of aspirin. • associated with dizziness, palpitations, tingling, or dif-
• Recommend an annual influenza immunization. ficulty swallowing.
• Check that the patient has a cardiology follow-up In such cases, consider other causes of chest pain such
appointment for an assessment of the coronary arteries as gastrointestinal or musculoskeletal pain.
and suitability for invasive treatment (PCI), as well as for • The NICE guidelines present a table to help estimate the
an assessment of left ventricular function. The initial likelihood of coronary artery disease in someone present-
screening to assess coronary blood flow is likely to be an ing with symptoms of angina, but such tables are not yet
exercise (stress) ECG. Patients with a negative stress test routinely used in general practice. Put simply, the likeli-
are unlikely to require coronary artery surgery, and so an hood of angina increases with age and with the presence
unnecessary angiogram can be avoided. of additional cardiovascular risk factors:
• Smoking
Other Factual Advice • Hypertension
• Diabetes
• Sedentary workers may return to work at 4 to 6 weeks, • Family history of CHD (first-degree relative: male
light manual workers at 6 to 8 weeks, and heavy manual <55 years/female <65 years)
workers at 3 months following MI. • Raised cholesterol (>6.5 mmol/L) and other lipids
ALGrawany
• As well as these risk factors, also assess for: Drug Treatment of Stable Angina
1. BMI; (Bass & Mayou, 2002)
2. hypo/hyperthyroidism;
3. anaemia/polycythaemia; • Patients require optimal drug treatment with one or two
4. arrhythmias; antiangina drugs as necessary to treat symptoms plus
5. valvular disease (any aortic systolic murmur needs drugs for secondary prevention of cardiovascular disease.
assessment for aortic stenosis); • Explain the purpose of the drug treatment, why it is
6. depression and social isolation; important to take the drugs regularly, and how side
7. physical activity levels. effects of drug treatment might affect the person’s daily
activities.
WORKUP—INVESTIGATIONS • Titrate the drug dosage against the person’s symptoms
1. Haemoglobin up to the maximum tolerable dosage.
2. Thyroid function tests (TFTs) • Review the person’s response to treatment, including any
3. Fasting blood sugar side effects, 2 to 4 weeks after starting or changing drug
4. Fasting lipid profile treatment.
5. Resting ECG. A normal ECG does not exclude the
diagnosis but an abnormal ECG makes it more likely.
ECG features consistent with coronary artery disease Cardiovascular Risk Reduction
include:
• pathological Q waves; • Encourage lifestyle change (i.e., diet, exercise, smoking,
• LBBB; alcohol).
• ST segment and T wave abnormalities (e.g., flattening or
inversion).
• Consider aspirin 75 mg daily. Clopidogrel is preferred if
patient has peripheral arterial disease or if aspirin not
tolerated. Dual therapy is only recommended in stable
Referral angina after PCI for up to one year.
• Offer statin treatment (Atorvastatin 80 mg) and treat
• Patients with suspected angina should be referred to high blood pressure in line with NICE guidelines.
cardiology (e.g., fast track chest pain clinic) for definitive • Consider ACE inhibitors or low-cost ARB for people
diagnosis. with stable angina and diabetes, previous MI, CKD or
• Further investigations will depend on local arrangements LV systolic dysfunction.
but may include exercise ECG or other forms of nonin-
vasive functional testing. Selecting Drugs
Management 1. Offer either a beta-blocker (e.g., atenolol 100 mg, meto-

prolol 50–100 mg twice daily, or bisoprolol 5–20 mg)
• Information and support: or calcium channel blocker (use rate limiting CCB [e.g.,
• Explain stable angina and its long-term course and diltiazem], unless heart failure or heart block, in which
management, including factors that can provoke an case use amlodipine) as first line treatment.
attack (e.g., cold, exertion, stress, heavy meals). • If either is not tolerated, switch to the other.
• Explore fears and misconceptions about angina (e.g., • If either is ineffective, switch to the other or combine
around physical exertion, including sexual activity). both (NB: avoid verapamil in combination with
• Encourage self-management skills (e.g., pacing activi- beta-blocker).
ties and goal setting). 2. If the person cannot tolerate β-blockers or calcium
• Preventing and treating episiodes of angina: channel blockers or if both are contraindicated, consider
• Advise patients to use a short-acting nitrate (e.g., glyc- monotherapy with one of the following:
eryl trinitrate [GTN]) spray or buccal tablets during • A long-acting nitrate
episodes of angina and immediately before any • Ivabradine
planned exertion, including intercourse (except if the • Nicorandil
patient has just taken Viagra or similar). • Ranolazine
• Explain that side effects such as flushing, headache, and 3. If not satisfactorily controlled on two drugs, refer for
lightheadedness may occur. Patients should sit down or consideration of revascularization
hold onto something if they feel lightheaded or dizzy.
• Advise patients to repeat the dose after 5 minutes if Revascularization
the pain has not gone, and to call 999 if the pain
persists for another 5 minutes. • Coronary intervention such as percutaneous coronary
• Patients with stable angina should seek professional intervention (PCI) or coronary artery bypass graft (CA
help if they have a sudden worsening in the frequency BG) surgery should only be considered if symptoms are
or severity of their angina. not optimally controlled with medical treatment.
• Refer to cardiology for further assessment, which may • Refer to a psychologist or counsellor patients who accept
include noninvasive investigations and/or coronary the above explanation but who still find themselves both-
angiography. ered by the pain.
• Coronary angiography is the traditional investigation for • Consider a trial of a tricyclic antidepressant or SSRI
establishing the nature, anatomy, and severity of CHD. in a patient sufficiently troubled by pain, even in the
It is an invasive investigation and carries a mortality risk absence of depression.
of around 0.1% for elective procedures (SIGN, 2018).
• The main purpose of revascularization is to improve the Heart Failure
symptoms of stable angina:
• CABG surgery and PCI are effective in relieving GUIDELINE
symptoms, but repeat revascularization may be neces-
sary after either procedure, and the rate is lower after National Institute for Health and Care Excellence. (2010b).
Chronic heart failure: Management of chronic heart failure in
CABG surgery. adults in primary and secondary care. NICE clinical guideline
• Stroke is an uncommon complication of each of these 108. Available at www.nice.org.
two procedures (the incidence is similar). Scottish Intercollegiate Guidelines Network. (2016a).
• There is a potential survival advantage with CABG Management of chronic heart failure. SIGN guideline 147.
surgery for some people with multivessel disease. Available at www.sign.ac.uk.
• In those for whom both percutaneous and surgical
revascularization is feasible, percutaneous revasculari
zation is a more cost-effective approach. • Heart failure is a complex syndrome of symptoms and
signs, caused by structural or functional abnormalities of
Noncardiac Chest Pain the heart (NICE, 2010b). It is often divided into heart
failure due to left ventricular systolic dysfunction (LVSD,
• Less than half of patients referred to cardiac clinics and associated with a reduced left ventricular ejection frac-
accident and emergency (A&E) departments with chest tion) or heart failure with a preserved ejection fraction
pain are found to have coronary artery disease (Bass & (HF-PEF). Most of the evidence on treatment is for
Mayou, 2002). Many of these patients continue to suffer heart failure due to LVSD. The most common causes of
pain and long-term functional impairment. Their man- heart failure in the United Kingdom are coronary artery
agement is often inadequate once the diagnosis of cardiac disease and hypertension, and many patients have had a
disease is excluded. myocardial infarction in the past.
• A clinical prediction rule called the Marburg Heart Score • Both the incidence and prevalence of heart failure
(MHS) assists GPs in ruling out chest pain caused by increase steeply with age, and the prevalence is expected
CHD (Haasenritter et al., 2012). Based on five findings to rise in the future as the result of an ageing population,
from history and examination, the score has a high nega- improved survival of people with ischaemic heart disease,
tive predictive value, but further research is needed to and more effective treatments for heart failure (Owan
assess its use in routine practice. et al., 2006).
• Be clear from the history, investigations, and specialist • The prognosis remains poor, despite improvements in
assessment that the pain is noncardiac. Then resist outcomes in the past decade. In one community-based
requests for further cardiac referral. study, 10-year survival rates ranged from 12% in those
• Try to make a positive diagnosis of the cause of the with multiple-cause heart failure to around 31% in those
pain: musculoskeletal disorders, panic attacks, reflux, with LVSD (Taylor, Roalfe, Iles, & Hobbs, 2012).
depression.
• If a clear diagnosis is possible, treat accordingly (e.g., Diagnosis
with NSAIDs for musculoskeletal pain, a PPI for reflux,
antidepressants for depression). • Making the diagnosis of heart failure can be challenging.
• If no clear diagnosis is possible, attempt an explanation Patients may present with a range of symptoms, includ-
along the following lines: that the symptoms are real; ing fatigue, breathlessness, and ankle swelling; and they
that they do not arise from the heart or from any other may also have other conditions such as CHD, diabetes,
specific illness; that the brain normally disregards sensa- hypertension, and COPD, which can confuse the picture
tions that it receives from all over the body, but that for (SIGN, 2016a).
some reason, in this patient, it is sensitized to sensations • Careful history-taking and examination will help to
from the chest. This explanation might lead on to discus- guide further assessment. The following clinical signs will
sion of how such sensitization may have occurred. The add to the suspicion of heart failure, though none are
patient may volunteer the memory of some event that diagnostic in isolation:
seemed to trigger the chest pain. • Raised jugular venous pressure (JVP)
• Ask who else in the family is worried about the pain and • Presence of a third heart sound (S3)
try to see them as well. • Basal crepitations
• Tachycardia • If raised (BNP 100–400 pg/mL [29–116 pmol/L] OR

• Peripheral oedema NT-pro-BNP 400–2000 pg/mL [47–236 pmol/L]):
• Initial investigations should help to rule out other causes refer within 6 weeks
of symptoms and should include full blood count, fasting • If levels are normal (BNP <100 pg/mL [29 pmol/L]
glucose, thyroid function tests, serum urea and creati- OR NT-pro-BNP <400 pg/mL [47 pmol/L]), a diag-
nine, urinalysis, ECG, and chest x-ray. NICE and SIGN nosis of heart failure is unlikely in an untreated
guidelines also now recommend the widespread use of patient, and alternative diagnoses should be sought.
serum natriuretic peptides as an aid to diagnosis of heart
failure. Management of Confirmed Chronic
Serum Natriuretic Peptides Heart Failure
• B-type natriuretic peptide (BNP) and N-terminal-pro- Pharmacologic management of heart failure depends on
BNP (NT-pro-BNP) are peptide hormones produced whether the person has LVSD or preserved ejection fraction
in the heart. They are raised with increasing left ven- (most research has been done on the former), and each will
tricular volume and pressure and concentrations tend be considered in turn in this section. First, there are a number
to rise with NYHA class (Table 7.2). Very high levels of general measures that apply to both forms of heart failure.
indicate a worse prognosis and should prompt urgent
referral. General Measures
• If the test is negative, heart failure is very unlikely (i.e., • Information: Explain what is going on and what to do if
it has high sensitivity). False negatives may be caused by things get worse.
obesity and by drugs used to treat heart failure (i.e., • Weight: Encourage to reduce weight, if obese. Recom-
diuretics, ACE inhibitors, ARBs, and beta-blockers). mend daily weighing at roughly the same time and in
• If the test is positive, it does not diagnose heart failure similar clothes. Patients should report if they gain more
but prompts referral for echo (i.e., it has relatively low than 2 kg over 2 days. Use diuretics flexibly, guided by
specificity). False positives occur with cardiac ischaemia weight (Arroll, Doughty, & Andersen, 2010).
from any causes (e.g., LVH, tachycardia, hypoxia) and • Diet: Advise a diet with no added salt.
in some other chronic conditions (e.g., diabetes, CKD, • Fluid: Restrict fluid intake to 2 L/day, unless losing fluids
COPD, cirrhosis) (Al-Mohammad et al., 2010). from sweat, diarrhoea, or vomiting.
• Alcohol: Keep alcohol intake low. In alcoholic heart
How to Use B-Type Natriuretic Peptide disease recommend complete cessation.
• The first question to ask of someone presenting with • Smoking: Stop smoking; it causes vasoconstriction. Refer
possible heart failure is, “Have you previously had an to the smoking cessation services.
MI?” If so, refer urgently (within 2 weeks) for echo and • Exercise: Rest in the acute phase, but exercise once stable.
specialist assessment. There is no need to check BNP in Regular low intensity physical activity improves mortal-
this situation. ity and morbidity. Refer to the heart failure rehabilitation
• If, however, there is no history of MI, the NICE guide- service if this facility exists.
lines suggest checking BNP and referring to cardiology • Sexual activity: Be prepared to broach sensitive issues
if the level is raised: with patients, such as sexual activity, as these may not be
• If very high (BNP >400 pg/mL [116 pmol/L] OR raised by patients.
NT-pro-BNP >2000 pg/mL [236 pmol/L]): refer • Vaccination: Recommend influenza vaccination annually
urgently (to be seen within 2 weeks) and pneumococcal vaccination once.
• Air travel: Air travel will be possible for the majority of
patients with heart failure, depending on their clinical
TABLE New York Heart Association Classification of condition at the time of travel.
7.2 Heart Failure Symptoms • Driving regulations: Advise patients to check with the DVLA,
Class I—asymptomatic No limitations of ordinary activity particularly drivers of large goods vehicles (https://www.gov
.uk/government/organisations/driver-and-vehicle-licensing
Class II—mild Slight limitation of physical
activity but comfortable at -agency).
rest • Depression: Check for depression. It is present in one
third and should be treated as thoroughly as if it was
Class III—moderate Marked limitation of physical
activity. Comfortable at rest not associated with heart failure. Warn patients not to
but symptomatic on less than buy St. John wort over the counter (OTC) because of
ordinary physical activity its interaction with prescribed medication, specifically
Class IV—severe Inability to carry on any physical digoxin and warfarin.
activity without discomfort. • NSAIDs: Stop NSAIDs (including COX-2 inhibitors) if
Symptoms present at rest taken, unless they are essential. Warn the patient not to
buy them OTC.
• Carers: Identify the carer or carers and involve them in drops by more than 1 kg because of dehydration,
the management. the patient should stop the diuretic and drink more
fluid.
Chronic Heart Failure (CHF) With Beta-Blockers
Left Ventricular Dysfunction
• All patients with LVSD should be started on a licensed
Left ventricular systolic dysfunction, assessed by measuring beta-blocker (e.g., bisoprolol, carvedilol, nebivolol) once
the left ventricular ejection fraction by echocardiography, heart failure is controlled, provided there are no absolute
refers to impaired left ventricular pump (contractile) func- contraindications (e.g., heart block or asthma).
tion. Most evidence to guide management of heart failure • In patients already taking another beta-blocker (e.g.,
is in this group of patients, which make up around half atenolol for angina) who develop heart failure, advise
of all heart failure cases (Arroll, Doughty, & Andersen, switching to one of the above. A study in British general
2010). practice found that switching to bisoprolol or carvedilol,
• All patients should be offered both ACE inhibitors and mainly from atenolol, was the single most valuable
beta-blockers, unless there are contraindications. manoeuvre in patients with left ventricular dysfunction
(Mant et al., 2008).
ACE Inhibitors/Angiotensin-II Receptor Blockers • NICE guidelines suggest you should not be put off pre-
• ACE inhibitors increase the ability to exercise, improve scribing beta-blockers in individuals with what were pre-
well-being, and prolong life in all degrees of heart failure viously considered to be relative contraindications, such
(Dargie & McMurray, 1994). as older adults and those with peripheral vascular disease,
• ARBs (e.g., candesartan and valsartan) may be used in erectile dysfunction, diabetes mellitus, interstitial pul-
patients intolerant of ACE inhibitors (e.g., with ACEI- monary disease, and COPD without reversibility.
related cough). • Take a “start low, go slow” approach. Start at a low dose
• If there is a high probability of chronic heart failure (e.g., bisoprolol 1.25 mg daily or carvedilol 3.125 mg
(CHF), start treatment before waiting for echo, unless twice daily), and double the dose every 2 to 4 weeks until
valve disease is suspected, in which case wait. the target dose is reached (bisoprolol 10 mg daily,
• Exclude the absolute contraindications of allergy to ACE carvedilol 25 mg twice daily).
inhibitors and pregnancy. • Monitor heart rate, BP, and clinical status at each dose
• Start at a low dose and titrate upward at short intervals titration. If the pulse is less than 50 and symptoms have
(e.g., every 2 weeks) until the optimal tolerated or target worsened, halve the dose. Stop and seek specialist advice
dose is achieved. if symptoms have become severe.
• Measure serum urea, creatinine, electrolytes, and eGFR • If at any stage it is necessary to stop the beta-blocker,
when starting an ACE inhibitor and after each dose tail it off gradually to avoid rebound ischaemia and
increment. Also check blood pressure. arrhythmias.
• ACE inhibitors (and ARBs) should not normally be
started if the pretreatment serum potassium concen- Diuretics (Other Than Spironolactone)
tration is significantly above the normal reference • Diuretics should be routinely used for the relief of con-
range (typically >5.0 mmol/L). Stop ACEI/ARB gestive symptoms and fluid retention in patients with
therapy if the serum potassium concentration rises heart failure, and titrated (up and down) according to
to above 6.0 mmol/L and other drugs known to need following the initiation of first line heart failure
promote hyperkalaemia have been discontinued. therapies. Their only function is symptom relief.
• If there is a drop in eGFR at or over 25% or an • Avoid overtreatment, which can lead to dehydration and
increase in plasma creatinine at or over 30%, investi- renal dysfunction, particularly with loop diuretics. Hypovo-
gate other causes of a deterioration in renal function laemia is a common cause of dizziness and lightheadedness.
such as volume depletion or concurrent medication • Monitor blood pressure and renal function after 4 weeks
(e.g., NSAIDs). If no other cause is found, stop the and then 6 monthly.
ACEI/ARB or reduce dose to previously tolerated • Use a loop diuretic (e.g., furosemide or bumetanide)
level. in moderate to severe heart failure. They will cause less
• Advise the patient to take the first dose before going to hypokalaemia and impotence than a high-dose thiazide
bed to reduce the effect of first-dose hypotension. (e.g., bendroflumethiazide). Increase the dose gradually
• Once the dose is stable, repeat creatinine and electro- until diuresis occurs.
lytes at 3 months then 6 monthly or sooner if further • Once stable, reduce the dose to as low as possible without
dose titration is necessary or there is intercurrent oedema or breathlessness occurring. Alternatively, con-
infection. sider stopping or reducing to a thiazide diuretic.
• Warn the patient to avoid dehydration, for instance in • The addition of a thiazide diuretic or a potassium sparing
gastroenteritis or in hot weather, as this can cause a diuretic such as spironolactone (or both) to loop diuret-
sudden deterioration in renal function. If the weight ics can be useful if pulmonary or ankle oedema persists,
because the different classes of diuretic are thought to have fraction. It has the same symptoms and signs as heart
an additive effect. However, even in short-term combined failure with LVSD, and carries a similar mortality risk
use, the diuresis can be excessive and can cause hypokalae- but has a less clear evidence base for treatment (Jong,
mia. Check creatinine and electrolytes after 3 days. McKelvie, & Yusuf, 2010).
• Patients with HF-PEF are more likely than those with
Second Line Pharmacologic Management LVSD to be older, female, have hypertension, and atrial
fibrillation but are less likely to have coronary artery
• If a patient remains symptomatic despite optimal treatment disease.
with ACE inhibitor and beta-blocker, refer for specialist • NICE recommends the use of diuretics for symptomatic
advice on second line treatment which might include: relief.
• A mineralocorticoid receptor antagonist (MRA), such • ACE inhibitors and beta-blockers should be considered,
as spironolactone or eplerenone; particularly when there are other compelling indications
• an ARB; for their uses (such as coronary artery disease, hyperten-
• sacubitril/valsartan (angiotensin receptor neprilysin sion, and diabetes mellitus).
inhibitor) - stop ACE inhibitor and ARB, continue • If atrial fibrillation is present, digoxin may be added
beta-blocker and MRA; for rate control, and anticoagulation should be
• hydralazine plus nitrate (especially for a patient of considered.
African or Caribbean descent with moderate to severe
heart failure); Grounds for Admission
• ivabradine; 1. Severe symptoms (e.g., severe dyspnoea or hypotension)
• digoxin. 2. Acute myocardial infarction
3. Severe complicating medical illness (e.g., pneumonia)
Mineralocorticoid Receptor Antagonists 4. Inadequate social support
• Hyperkalaemia is a potentially fatal adverse effect and 5. Failure to respond to treatment
is most likely in those with impaired renal function. 6. Uncontrolled arrhythmia
Only give spironolactone if the creatinine is less than
220 µmol/L. Referral for Specialist Review
• Warn the patient to avoid salt substitutes with a high
potassium content. Warn males about gynaecomastia. • Refer patients to the specialist multidisciplinary heart
• If K+ reaches 5.5 to 5.9 mmol/L or creatinine rises to failure team for:
200 µmol/L, reduce the dose and monitor closely. If • the initial diagnosis of heart failure;
those levels are exceeded, stop and seek specialist advice. • the management of:
1. severe heart failure (NYHA class IV);
Ivabradine 2. heart failure that does not respond to treatment;
• Ivabradine was approved by NICE in November 2012 3. heart failure that can no longer be managed effec-
and is an option for patients: tively in the home setting.
• with symptomatic stable heart failure (NYHA class II to • Consider specialist review:
IV) with LVSD and an ejection fraction of 35% or less; • when renal function continues to deteriorate or dete-
• who are in sinus rhythm with a resting heart rate riorates rapidly;
above 75 bpm; • when there are concerns about low blood zpressure;
• who are also receiving standard therapy includ- • when the patient is pregnant or considering pregnancy.
ing beta-blockers, ACE inhibitors, and aldosterone
antagonists. End-Stage Heart Failure
• Ivabradine should be initiated by a heart failure specialist
(e.g., specialist nurse) with access to a multidisciplinary • Issues of sudden death and living with uncertainty are
heart failure team. pertinent to all patients with heart failure. The opportu-
nity to discuss these issues should be available at all stages
Digoxin of care.
• Consider for patients in sinus rhythm if still symptomatic • Deciding on prognosis in heart failure is difficult and
despite all the above. See the upcoming section on atrial cardiologic advice may be needed. The palliative needs
fibrillation for the use of digoxin in patients with AF. of patients and carers should be identified, assessed, and
managed at the earliest opportunity. There should be
Heart Failure With Preserved Ejection access to professionals with palliative care skills within
Fraction (HF-PEF) the heart failure team.
• Nonessential treatment should be stopped.
• As noted, it is thought that up to 50% of patients • The focus should be on symptom control (see the upcom-
with heart failure have a preserved ventricular ejection ing discussion).
• Note: Digoxin and aminophylline should not be used in

Breathlessness acute failure outside hospital.
• Morphine is the most effective palliation. Give a low
oral dose (2.5–5 mg 4-hourly), as peak plasma levels are
Right Heart Failure
higher in heart failure.
• If breathless at rest then consider oxygen therapy at home Acute Right Heart Failure
(40%–80% if there is no COPD). • The clinical picture of low output state, no pulmonary
• Refer for relaxation, breathing exercises, and anxiety oedema, and a raised JVP may be due to massive pul-
management if these are available. monary embolism or to acute right heart failure as in
a right ventricle myocardial infarction, or acute cor
Weakness and Fatigue pulmonale.
• This is usually secondary to a low cardiac output. • Admit urgently. Position the patient however they are
• Drug reduction. Consider reducing ACE inhibitors and the most comfortable. Do not give morphine; respi-
beta-blockers, and diuretics if there is dehydration. ratory depression and hypotension are very likely to
• Depression. Avoid tricyclics; SSRIs may be of value. follow. Do not give diuretics as they may precipitate
• Social factors. Increase social care if possible and ensure shock.
support for the carer(s).
Chronic Cor Pulmonale
Anorexia/Nausea • Treat the lung disease by considering:
• Digoxin. Check serum level and renal function. 1. antibiotics for exacerbations of infection;
• Consider increasing diuretics if there is hepatic congestion. 2. inhaled bronchodilators and steroids via a spacing
• Diet. Advise the patient to have small, appetizing meals device;
more frequently. Allow small amounts of alcohol before 3. physiotherapy;
meals. 4. long-term oxygen therapy;
• Antiemetics. Use levomepromazine. Avoid cyclizine. 5. nasal intermittent positive pressure ventilation for
those with thoracic deformities and obstructive sleep
Oedema apnoea.
• Mobilization is advisable in theory but rarely practicable • Admit readily anyone with an acute exacerbation.
at this stage. Oxygen will dilate the pulmonary vessels and so reduce
• Diuretics. Avoid increasing diuretics at this stage unless the pulmonary artery pressure. The danger of hyper-
doing so gives symptomatic relief. They are unlikely to capnoea makes this hazardous in the acute situation at
have much effect on the oedema. home.
• Be cautious about advising the patient to raise the feet. • Use diuretics carefully as they may reduce renal blood
This may increase the venous return to the heart and flow further. They are only required if oedema becomes
worsen the dyspnoea. troublesome.
• Compression stockings/bandages. These may increase tissue • Use digoxin only if the patient is in atrial fibrillation.
damage and are uncomfortable. • Do not start ACE inhibitors outside hospital.
Acute Pulmonary Oedema Palpitations and Arrhythmias

• When called urgently to the patient in extremis, get • Palpitations are a common presentation in general prac-
someone else to dial for an ambulance while you: tice and a frequent reason for cardiology referrals. They
1. sit the patient up with the legs down; often cause considerable distress and anxiety for the
2. give oxygen if available; patient; however, more often than not they are benign,
3. give a GTN tablet or spray sublingually (for its imme- with less than half of patients with palpitations suffering
diate vasodilator effect); from an arrhythmia and not every identified arrhyth-
4. give intravenous (IV) diamorphine 1 mg/min up to mia being of clinical or prognostic significance (Mayou,
5 mg, or IV morphine 2 mg/min up to 10 mg, mixed Sprigings, Birkhead, & Price, 2003).
with metoclopramide 10 mg IV; do not use cyclizine • Furthermore, not all arrhythmias present with palpita-
as it raises systemic arterial pressure; tions. Some may cause no symptoms, while in other cases
5. give a loop diuretic (IV furosemide 50 mg or IV symptoms include tachycardia, bradycardia, chest pain,
bumetanide 1 mg). breathlessness, lightheadedness, and fainting (syncope)
• Look for causes of the heart failure that may need or near fainting.
treatment in their own right, especially myocardial • As well as arrhythmias, palpitations can be caused
infarction. by structural heart diseases, psychosomatic disorders, sys-
• Admit once the patient is sufficiently stable, for fuller temic diseases, and effects of medical and recreational
assessment and for continuing treatment. drugs (Raviele et al., 2011).
• Careful history-taking and physical examination are • Patients with the following features should be referred
important, but further investigation is invariably required urgently to cardiology:
(Hoefman et al., 2007). • Palpitations with syncope/near syncope
• Palpitations during exercise
History • Family history of inheritable heart disease/sudden
• Ask what the patient means by palpitations. It may be arrhythmic death syndrome (SADS)
more like a pulsatile tinnitus or a chest discomfort. • High-risk structural heart disease
• Ask about the rate, frequency, and duration of palpi- • High-degree atrioventricular block
tations, as well as exacerbating and relieving factors. • Patients with the following features should be referred
The patient may be able to tap out the rate on the con- routinely to cardiology or to local palpitations assessment
sulting desk. service:
• Are there any associated symptoms, such as sweating, • Palpitations with associated symptoms
breathlessness, or chest pain? If any of these are present • Abnormal ECG
and the patient has palpitations at that time, refer to • History suggestive of recurrent tachyarrhythmia
hospital immediately by 999 ambulance. • Structural heart disease
• Ask about smoking, alcohol, caffeine consumption, and • Patients with the following features may not require
use of illicit substances such as cocaine, ecstasy, and referral:
amphetamines. • History in keeping with extrasystoles/ectopic beats
• Ask about general well-being. In particular, assess for any • Normal ECG
life stressors and ask about personal or family history of • No family history
anxiety or heart problems. • No evidence of IHD or structural heart disease
Examination
Ectopic Beats in a Normal Heart
• Assess for tremor, which may indicate thyrotoxico-
sis or anxiety. Ask the patient to hold the arms out- • Explain their benign nature to the patient.
stretched in front with the palms down and to spread the • Advise against caffeine, fatigue, smoking, and alcohol.
fingers. • Beta-blockers should be used only if the patient is unable
• Assess pulse rate and rhythm and check blood pressure. to tolerate the ectopics.
Is the pulse rate regularly irregular, suggesting ectopic • Frequent ventricular ectopics (>100/hour) may be grounds
beats, or irregularly irregular, suggesting AF or atrial for referral. Patients may have a prolapsing mitral valve,
flutter? or the older patient may have unsuspected ischaemic
• Examine the heart, assessing for evidence of structural heart disease.
heart disease (e.g., murmurs, abnormal heart sounds, or • For those still troubled by their symptoms, consider
signs of heart failure). referral for cognitive behavioural therapy (CBT) or
similar (Mayou, Sprigings, Birkhead, & Price, 2002).
Investigations
• Blood tests should include full blood count (to exclude Atrial Fibrillation
anaemia), urea and electrolytes, and thyroid function
tests.
• Arrange a 12-lead ECG. Abnormalities to look for GUIDELINE
include:
• atrial fibrillation; National Institute for Health and Care Excellence. (2014).
Atrial fibrillation: Management. NICE clinical guideline 180.
• second- and third-degree AV block; Available at www.nice.org.uk.
• signs of previous myocardial infarction;
• left ventricular hypertrophy and left ventricular strain
patterns;
• left bundle branch block; • Atrial fibrillation (AF) is the most common disorder of
• abnormal T wave inversion and ST segment changes; heart rhythm with a prevalence that increases with age
• signs of preexcitation (short PR interval and delta from about 6% in people aged 65 to 74, to 12% in
waves); people aged 75 to 84, and 16% in people aged 85 and
• abnormal QTc interval and T wave morphology. over (Fitzmaurice et al., 2007).
• It is associated with increased risk of stroke and heart failure,
Next Steps with higher mortality as a result.
• There is no validated risk stratification tool that is widely • AF also increases the risk of sudden cardiac death (Chen,
used in practice, although some authors have proposed Sotoodehnia, & Bůžková, 2013).
a traffic light system to guide further management (Wolff • Oral anticoagulation can reduce the risk of stroke by
& Cowan, 2009). two-thirds (Aguilar & Hart, 2005). However, current
evidence suggests that only about half of patients with Cardioversion Versus Rate Control
AF identified as being at high risk are receiving antico-
agulants (Holt et al., 2012). • Rate control is recommended as the treatment of choice in
• The older you are, the more dangerous AF becomes, with most patients. It has advantages over rhythm control: fewer
strokes being more common and more disabling. Yet unde- hospital admissions, fewer adverse drug reactions, possibly a
ruse of anticoagulation is highest in older people, who have lower mortality (Atrial Fibrillation Follow-up Investigation
most to gain from treatment (Hobbs et al., 2011). of Rhythm Management [AFFIRM], 2002), and it is more
• Primary care has considerable potential to further reduce cost effective (Hagens, Vermeulen, & TenVergert, 2004).
the risk of stroke in AF, by identifying high-risk patients • Referral for cardioversion is more appropriate for certain
and lowering the threshold for offering anticoagulation, patients, either because it is more likely to succeed than
as recommended in guidelines. in others (the first three categories) or because sinus
rhythm offers greater chance of clinical benefit than rate
Identification of Atrial Fibrillation control (the last two categories):
• Younger patients
• The SAFE study in 2005 showed that opportunistic • Those presenting for the first time with lone AF
screening (by pulse palpation followed by ECG if pulse • Those with AF secondary to a cause that has now been
irregular) of patients aged over 65 is cost effective for treated
detecting AF (Hobbs et al., 2005). Opportunistic screen- • Those who are symptomatic
ing is endorsed by the European Society of Cardiology • Those with congestive heart failure (restoration of
Guidelines. sinus rhythm may improve LV function)
• Some have gone further, suggesting that a national screen- • The decision as to whether to use chemical or electri-
ing programme should be introduced, using this approach, cal cardioversion will be made in discussion between
for all patients over 65 (James & Campbell, 2012). the patient and cardiologist. Vernakalant is a new
• Screening of such patients while attending annual intravenous antiarrhythmic agent approved for car-
influenza vaccination clinics has been piloted, but with dioversion of AF of 7 days duration or less. Other
mixed results (Gordon, Hickman, & Pentney, 2012; agents include ibutilide, flecainide, propafenone, and
Rhys, Azhar, & Foster, 2013). amiodarone.
• Rate control is more appropriate in those in whom car-
dioversion is less likely to succeed:
WORKUP OF ATRIAL FIBRILLATION • Those over 65 years old
• Those with structural heart disease or CHD
1. History of alcohol intake (either chronic or bingeing)
2. Blood pressure (half of all cases of AF are hypertensive) • Those whose AF is longstanding (e.g., >12 months)
3. TFTs, creatinine, and electrolytes • Those in whom previous attempts at cardioversion
4. Examine for heart failure, valvular heart disease, congenital have failed or been followed by relapse
heart disease, or acute pericarditis or myocarditis • Those in whom an underlying cause (e.g., thyrotoxi-
5. ECG (looking for ischaemic heart disease [IHD], left
cosis) has not yet been corrected
ventricular (LV) strain, and delta waves)
6. NICE recommend performing an echocardiogram in • Those with a contraindication to antiarrhythmic
patients for whom: drugs
• a baseline echocardiogram is important for long-term • Aim for lenient (HR <110 bpm) rather than strict (HR
management; <80 bpm) rate control, unless patient is symptomatic.
• a rhythm control strategy that includes cardioversion is
• Use:
being considered;
• there is a high risk or a suspicion of underlying 1. a beta-blocker first line (e.g., atenolol, bisoprolol,
structural/functional heart disease (such as heart failure metoprolol);
or heart murmur) that influences subsequent 2. a rate-limiting calcium channel blocker second line
management (e.g., choice of antiarrhythmic drug); (e.g., diltiazem or verapamil); avoid both in heart
• refinement of clinical risk stratification for
failure, and avoid the combination of verapamil and
antithrombotic therapy is needed.
a beta-blocker;
3. digoxin if still symptomatic. If digoxin is contraindi-
cated, add diltiazem to the beta-blocker. The usual
concern about causing bradycardia or AV block is less
• Admit if rapid AF is associated with: of a problem in AF since that is the aim of the
1. chest pain; treatment.
2. hypotension;
3. more than mild heart failure. Risk Stratification for Anticoagulation
• Refer urgently if seen within 48 hours of the onset of
AF and the patient is a candidate for cardioversion (see • A risk factor-based approach to stroke risk stratifica-
the upcoming discussion). tion has been promoted for many years (e.g., CHADS2
[Congested heart failure, Hypertension, Age ≥75, Diabe- Anticoagulation

tes, Stroke (doubled)]) (Gage et al., 2001), but recent evi-
dence has prompted a shift in focus toward identification • For more than half a century, warfarin has been the primary
of “truly low-risk” patients who do not need antithrom- medication used to reduce the risk of thromboembolic
botic therapy. events in patients with atrial fibrillation (Mega, 2011).
• The CHADS2 score has been replaced by the CHA2DS2- • Research has confirmed that warfarin is superior to anti-
VASc, which has been found to be more accurate (Table platelet therapy (e.g., aspirin) in the management of AF
7.3) (Olesen, 2011). in the community, even in older people (Mant et al.,
• Using CHA2DS2-VASc, all patients over 75 and all 2007). Aspirin should no longer be used for stroke pre-
women over 65 are automatically considered at high risk vention in AF; such patients should be reviewed.
and thus do not need further formal risk assessment, • Despite its clinical efficacy, warfarin has several limita-
other than consideration of bleeding risk. tions, including drug interactions and the need for
regular blood monitoring (INR should be controlled to
Assessment of Bleeding Risk between 2 and 3) and dose adjustments. See the accom-
panying table for contraindications to warfarin.
• Before starting anticoagulation, an assessment of bleed- • As a result, alternative anticoagulants that are at least as
ing risk should be undertaken. Major bleeding, especially efficacious but easier to administer have been developed
intracranial haemorrhage (ICH), is the most feared com- and widely promoted.
plication of anticoagulation therapy and confers a high • Four novel oral anticoagulant agents (NOACs) have
risk of death and disability (Connolly et al., 2011). been approved by NICE as alternatives to warfarin in
• Several different guidelines recommend the use of the nonvalvular AF. These are dabigatran, rivaroxaban, apix-
HAS-BLED score, which has been validated in many aban, and edoxaban.
cohort studies and correlates well with ICH risk (Table • Dabigatran is a direct thrombin inhibitor and the other
7.4) (Cairns, Connolly, McMurtry, Stephenson, & three are factor Xa inhibitors.
Talajic, 2001; Camm et al., 2010; Lip et al., 2011). • The main advantages of these agents are that they are
• A HAS-BLED score of 3 or more indicates a bleeding fixed dose, less susceptible to interactions, and do not
risk (ICH or requiring admission) on anticoagulation require monitoring.
over the next year sufficient to justify caution with
anticoagulation.
• A score of 3 or above does not exclude patients from TABLE The HAS-BLED Bleeding Risk Score
receiving anticoagulation, but would require extra 7.4 (Pisters et al., 2010)
caution to control bleeding risks (e.g., closer monitoring Letter Clinical Characteristic Points
of renal function and INR, better management of
hypertension). H Hypertension 1
A Abnormal renal and liver function 1 or 2
(1 point each)
S Stroke 1
TABLE
CHA2DS2-VASc B Bleeding 1
7.3
Risk Factor Score L Labile INRs 1
C Congestive heart failure 1 E Elderly (over 65) 1
H Hypertension 1 D Drugs or alcohol (1 point each) 1 or 2
A2 Age ≥75 2 TOTAL
D Diabetes 1 Hypertension is defined as systolic blood pressure >160 mm Hg.

Abnormal kidney function is defined as the presence of chronic dialysis
S2 CVA or TIA 2 or renal transplantation or serum creatinine ≥200 mmol/L.
Abnormal liver function is defined as chronic hepatic disease (e.g., cir-
V Vascular disease 1 rhosis) or biochemical evidence of significant hepatic derangement (e.g.,
bilirubin >2 × upper limit of normal, in association with aspartate amino-
A Age ≥65 1
transferase/alanine aminotransferase/alkaline phosphatase >3 × upper
Sc Sex category (i.e., female) 1 limit normal, etc.).
Bleeding refers to previous bleeding history and/or predisposition to
TOTAL SCORE bleeding (e.g., bleeding diathesis, anemia).
Labile INRs refers to unstable/high INRs or poor time in therapeutic range
0 = low risk (0.8% annual stroke rate) (e.g., <60%).
1 = moderate risk (1.75% annual stroke rate) Drugs/alcohol use refers to concomitant use of drugs, such as antiplate-
≥2 = high risk (>2.7% annual stroke rate) let agents, nonsteroidal antiinflammatory drugs, or alcohol abuse.
3 = 3.2%; 5 = 6.7%; 7 = 9.6% INR, International normalized ratio.
• Antidotes have recently been developed for both dabiga- CONTRAINDICATIONS TO WARFARIN IN
tran and the factor Xa inhibitors. ATRIAL FIBRILLATION (Man-Son-Hing &
• A number of studies have concluded that the NOACs Laupacis, 2003)
compare favourably to warfarin, in terms of both efficacy Absolute Contraindications
(reducing overall mortality and strokes) and safety profile • Pregnancy
(less intracranial bleeding) (Dentali, Riva, & Crowther, • Active peptic ulcer or other active source of GI bleeding
2012; Rasmussen, Larsen, Graungaard, Skjøth, & Lip, • Current major trauma or surgery
2012). • Uncontrolled hypertension
• All of the NOACs share these contraindications: sig- • A bleeding diathesis
• Bacterial endocarditis
nificant risk of major bleeding, pregnancy and breast • Alcoholism
feeding, additional anticoagulant therapy, and concomi- • Inability to control the INR
tant use of itraconazole, ketoconazole and HIV protease
inhibitors. Factors Which Increase the Risk of Bleeding
• For patients switching from warfarin to a NOAC, war- Slightly but Only Enough to Alter the Decision in
Cases Where the Benefit Is Already Borderline
farin should be stopped and the NOAC started as soon
• Current NSAID use (add gastroprotection or change to a
as the INR is below 2. COX-2 inhibitor)
• Activities that involve a high risk of injury
Dabigatran
• Dabigatran is recommended by NICE (2012a) as an Factors Often Erroneously Thought to
option for the prevention of stroke in nonvalvular AF in Contraindicate Warfarin
people with one or more of the following risk factors: • Old age. The INR is no harder to control in the elderly
than in younger patients. There is a slight increase in
• Heart failure: LVEF less than 40% or symptomatic bleeding risk in older patients but with increasing age the
with NYHA Class 2 or above benefit from warfarin rises more than the risk
• Previous CVA, TIA, or embolism • Past history of peptic ulcer or GI bleeding, now resolved
• Age 75 and older • Hypertension controlled (<160/90 mm Hg)
• Age 65 and older, with diabetes, coronary heart • Patients at risk of falling
• Previous stroke
disease, or high blood pressure • Alcohol intake of one to two drinks a day
• The recommended daily dose is 300 mg taken as one
150-mg capsule twice daily. Therapy is continued long
term.
• Patients aged 80 years or older, and those with renal
impairment (eGFR 30–49) should be treated with a Left Atrial Appendage Closure
daily dose of 220 mg taken as one 110-mg capsule twice • The left atrial appendage (LAA) is considered the main
daily because of the increased risk of bleeding in this (though not the only) site of thrombus formation leading
population. to ischaemic stroke in people with AF.
• It is contraindicated in people with severe renal impair- • Minimally invasive techniques have been developed to
ment (eGFR <30), and those at increased risk of bleed- occlude the LAA orifice and thereby reduce stroke risk,
ing, including previous GI ulceration, recent surgery, with results equivalent to warfarin (Holmes et al., 2009).
hepatic impairment, or liver disease. • At present, this procedure is only considered for patients
• Check renal function before starting. Do not start in any in whom oral anticoagulation is contraindicated.
patient with severe renal impairment (eGFR <30).
Rivaroxaban and Apixaban Paroxysmal Atrial Fibrillation

• NICE have approved rivaroxaban and apixaban as alter- • Paroxysmal atrial fibrillation (PAF) is defined as recurrent
natives to warfarin in people with nonvalvular AF and (two or more) episodes of AF that terminate spontane-
one or more of the following risk factors (NICE, 2012d, ously in less than 7 days, and usually less than 24 hours.
2013): • Tailor the treatment to the severity.
• Heart failure • The patient with infrequent attacks and no serious symp-
• Previous CVA or TIA toms, or attacks that can be averted by avoiding a precipitat-
• Hypertension ing cause (e.g., alcohol or caffeine), either give:
• Age over 75 years • no drug treatment, or
• Diabetes • a pill-in-the-pocket, to be taken at the onset of an
• The recommended daily dose of rivaroxaban is 20 mg attack, if the patient is suitable (see the accompanying
daily, reduced to 15 mg daily in people with renal impair- box).
ment (eGFR 30–49). It should be taken after food. • The patient with more frequent attacks or more severe
• The dose of apixaban is 5 mg twice daily, reduced to symptoms:
2.5 mg twice daily in renal impairment. • receives a standard beta-blocker.
ALGrawany
• If that is ineffective or not tolerated, refer for spe- regardless of the number and severity of paroxysms (Friberg,
cialist assessment. Other medications such as sotalol, Hammar, & Rosenqvist, 2010).
flecainide, propafenone, or amiodarone may be con-
sidered. Dronedarone, which is structurally related to Atrial Flutter
amiodarone, has been approved for the treatment of
paroxysmal or persistent AF, but only under special- • Atrial flutter should be approached in a similar way to
ist initiation and supervision. It should not be given atrial fibrillation, with a focus on:
to patients with moderate or severe heart failure, and 1. rate control;
should be avoided in patients with less severe heart 2. rhythm control;
failure, if appropriate alternatives exist. 3. prevention of thromboembolic complications.
• NICE warns of the danger of initiating drugs other • It is much less common than AF and usually less well
than standard beta-blockers for paroxysmal AF in tolerated, often presenting with palpitations.
general practice without specialist advice because of • Episodes of atrial flutter and AF can occur in the same
the risk that the drug will itself cause ventricular person.
arrhythmias. This is most likely in those with underly- • Refer all suspected cases for specialist assessment.
ing heart disease. In practice the patient will often • Radio-frequency catheter ablation is first line treatment
have been assessed already by a cardiologist and had (Sawhney & Feld, 2008).
previous experience of an antiarrhythmic drug without
adverse effects, obviating the need for re-referral. Paroxysmal Supraventricular Tachycardia
NICE RECOMMENDATIONS FOR THE • If the patient is seen during the attack, get the patient
SUITABILITY OF A PATIENT FOR A to perform a Valsalva manoeuvre. This can be described
“PILL-IN-THE-POCKET” to the patient as trying to breathe out forcefully while
1. Infrequent episodes (e.g., between once per month and keeping the mouth closed and nose pinched, or simulat-
once per year) ing straining on the toilet (Whinnett, Afzal Sohaib, &
2. Sufficiently reliable to use the treatment correctly Wyn Davies, 2012).
3. No structural heart disease, coronary heart disease, or left • Apply carotid sinus massage except where the patient:
ventricular dysfunction 1. is elderly;
4. Satisfactory baseline state: systolic blood pressure
>100 mm Hg, resting heart rate >70 bpm 2. has ischaemic heart disease;
3. is likely to be digoxin toxic;
4. has a carotid bruit;
5. has a history of transient ischaemic attacks.
• If a paroxysm becomes persistent (arbitrarily defined as Note: The British National Formulary recommends
lasting at least 7 days), refer for consideration of cardio- ECG monitoring during carotid sinus massage.
version. Refer immediately if the patient develops heart • Admit if the attack continues and there is no clear history
failure or hypotension. of previous attacks which have terminated themselves.
Even if there is such a history, keep the patient at the
Catheter Ablation surgery until the attack indeed terminates.
• The European Society of Cardiology (ESC) guidelines rec- • Record an ECG and give the patient a copy.
ommend that catheter ablation (by pulmonary vein isola- • If SVT is diagnosed from the patient’s history or if the
tion) be considered as a first line therapy for AF rhythm attack has terminated before admission was needed:
control in selected patients (i.e., those with PAF with a pref- • Refer for specialist confirmation and initiation of
erence for interventional treatment and a low-risk profile for treatment. Referral should be urgent if attacks are
procedural complications) (Kirchhof et al., 2016). associated with chest pain, dizziness, or breathlessness.
• Studies have shown promising results (Nielsen et al., • Catheter ablation is the first line definitive manage-
2012), but the procedure carries the risk of invasive ment option for SVT.
complications. In one study, complication rates were • Drug treatment is reserved for minimizing symptoms
0.6% for stroke, 1.3% for tamponade, 1.3% for periph- while awaiting catheter ablation or for those who
eral vascular complications, and around 2% for pericar- decline catheter ablation or in whom the procedure
ditis (Arbelo et al., 2012). carries an unacceptably high risk.
• Discuss with a cardiologist before starting an antiar-
Anticoagulation rhythmic drug (e.g., sotalol, flecainide, verapamil),
A patient with paroxysmal AF should be considered for while awaiting the cardiology appointment.
antithrombotic treatment according to the presence of risk • A baseline ECG, with a further ECG before each dose
factors (e.g., as assessed by CHA2DS2-VASc). The risk of increase, is recommended to look for prolongation of
stroke, although less well defined in paroxysmal AF, is con- the QT interval, an indicator that there is a risk of
sidered to be the same as in persistent or permanent AF, drug-induced torsade de pointes. A final decision on
the most appropriate drug will depend on the electro- • Do not offer antibiotics to prevent ineffective endocar-
pathology in the individual patient. ditis (IE) for any of the following procedures:
• Instruct the patient in the use of the Valsalva manoeu- • Any dental procedure
vre, and check that he or she is not smoking or misus- • An obstetric or gynaecologic procedure, or childbirth
ing alcohol or caffeine. • A procedure on the bladder or urinary tract
• A procedure on the oesophagus, stomach, or intestines
Ventricular Tachycardia • A procedure on the airways (including ear, nose, and
throat and bronchoscopy)
• VT associated with loss of consciousness or hypotension is • People with the following cardiac conditions should be
a medical emergency requiring immediate cardioversion. regarded as being at risk of developing IE:
• Call 999 emergency ambulance. • Acquired valvular heart disease with stenosis or
• Remember ABCs (airway, breathing, circulation), oxygen, regurgitation
ECG monitoring. • Valve replacement
• If the patient is conscious but in extremis, consider IV • Structural congenital heart disease, including surgi-
lidocaine 100 mg while waiting for the ambulance. cally corrected or palliated structural conditions, but
• After discharge, prophylaxis will be needed. If amioda- excluding isolated atrial septal defect, fully repaired
rone is chosen, 6-monthly TFTs and LFTs are necessary. ventricular septal defect or fully repaired patent
ductus arteriosus, and closure devices that are judged
Sick Sinus Syndrome to be endothelialized
• Hypertrophic cardiomyopathy
This requires admission for pacing. Drugs are likely to make • Previous infective endocarditis
symptoms worse because of the variability of the rhythms. • People at risk of IE should be offered clear and consistent
information about prevention, including:
Bradycardia • the benefits and risks of antibiotic prophylaxis, and
an explanation of why antibiotic prophylaxis is no
• Refer all patients with a bradycardia, other than sinus longer routinely recommended;
bradycardia, even if asymptomatic. A pacemaker is likely • the importance of maintaining good oral health;
to be needed and may be life saving. Untreated second • symptoms that may indicate IE and when to seek
degree and complete atrioventricular (AV) block have a expert advice;
mortality of 25% to 50% in the first year after diagnosis. • the risks of undergoing invasive procedures, including
For this reason, even asymptomatic patients with a rate nonmedical procedures such as body piercing or
of 40 or below should be paced. tattooing.
• Admit a patient in acute AV block with hypotension due • In people at risk of IE, it is important to investigate and
to the bradycardia. Give IV atropine if available while treat promptly any episodes of infection to reduce the
waiting for the ambulance. risk of endocarditis developing.
• If a person at risk of IE is receiving antimicrobial therapy
PATIENT SUPPORT GROUPS because of undergoing a gastrointestinal or genitourinary
procedure at a site where there is a suspected infection,
Arrhythmia Alliance: www.heartrhythmcharity.org.uk/
Atrial Fibrillation Association: www.atrialfibrillation.org.uk/
offer an antibiotic that covers organisms that cause infec-
Sudden Adult Death Trust: www.sadsuk.org/ tive endocarditis.
Cardiac Risk in the Young: www.c-r-y.org.uk/
PATIENT INFORMATION
There are a range of resources available through the British
Heart Foundation website at www.bhf.org.uk. Their helpline
Prophylaxis of Infective Endocarditis (0300 330 3311) is open Monday through Friday, 9 am to
5 pm.
GUIDELINE
Prophylaxis against infective endocarditis. NICE clinical The Prevention of Cardiovascular Disease
guideline 64. Available at www.nice.org.uk.
GUIDELINE
National Institute for Health and Care Excellence. (2014a).
• Antibiotic prophylaxis aims to reduce the incidence of Cardiovascular disease: Risk assessment and reduction,
infective endocarditis. including lipid modification. NICE clinical guideline 181.
Available at www.nice.org.uk.
• The NICE guidance represented a major shift in advice
on antibiotic prophylaxis.
• Cardiovascular disease (CVD) describes disease of the as the US Framingham heart study data (Anderson,
heart and blood vessels caused by the process of athero- Odell, Wilson, & Kannel, 1991), according to:
sclerosis and predominantly affects people older than 50 • age;
years (NICE, 2014a). There are significant gender differ- • sex;
ences in cardiovascular risk, including biologic differ- • lifetime smoking habit;
ences associated with pregnancy and menopause (Parikh, • blood pressure (if treated use pretreatment level);
2011), as well as differences in behavioural risk factors • serum lipids if known. If treated use pretreatment level.
(Huxley & Woodward, 2011). • There are a number of different validated resources available
• Worldwide, the two most important modifiable cardio- to calculate 10-year risk of cardiovascular disease, but NICE
vascular risk factors are smoking and abnormal lipids now recommends the use of QRISK2 assessment tool.
(SIGN, 2017). • Asymptomatic individuals should be considered at high
• The next most important are hypertension, diabetes, risk if they are assessed as having a 10% or greater risk of
psychosocial factors (including deprivation), and abdom- a first cardiovascular event over 10 years. Such individuals
inal obesity, but their relative effects vary in different warrant intervention with lifestyle changes and consider-
regions of the world. ation for drug therapy, to reduce their absolute risk.
• Risk factors tend to cluster and their effects are multipli- • NICE now recommends that the QRISK2 tool is used
cative, not additive. The presence of one or more of the up to and including age 84 years. Consider people aged
following increases the chance that other risk factors will 85 and older to be at increased risk of CVD because of
also be present: hypertension, raised cholesterol, inactiv- their age alone.
ity, obesity, smoking, and glucose intolerance (Perry, • A further consideration when assessing someone’s CVD
Wannamethee, & Walker, 1995). risk is the presence of xanthelasmata. A 2011 study
• Strategies for the prevention of cardiovascular disease can found that xanthelasmata predict an increased risk of CV
be divided into primary and secondary prevention. Primary events, particularly in women, even if serum lipid levels
prevention is concerned with preventing the occurrence are normal (Christoffersen et al., 2011). The authors
of cardiovascular disease in those currently unaffected. suggest xanthelasmata may be a sign of an increased
Secondary prevention relates to delaying or reversing the propensity to deposit lipid in soft tissues and is therefore
progression of disease in those already affected. While a cutaneous marker of atheroma. There is no such
targeting those at highest risk is known to be most cost increased risk with arcus corneae.
effective, most countries recognize the importance of a
multifaceted approach, adopting both population-based Lifestyle Changes
and more targeted interventions (Rose, 1981).
• Stop smoking. Someone who smokes 20 cigarettes a
Primary Prevention day or less and stops has a risk of CHD 10 years later
almost the same as in one who has never smoked. Recov-
• For the primary prevention of CVD in primary care, a ery is, however, less the longer the person has smoked
systematic strategy should be used to identify people who (Doll, Peto, Boreham, & Sutherland, 2004). Nicotine
are likely to be at high risk, now considered to be a 10%, replacement therapy increases the rate of quitting by
10-year risk after lifestyle modification.The NICE (2014) 50% to 70%, regardless of the setting and independent
guidelines remain controversial, reflecting uncertainty in of whether support, other than brief advice, is offered
the data that underpins them (Otto, 2016). (Stead, Perera, Bullen, Mant, & Lancaster, 2008).
• The role of GPs and practice nurses is, first and foremost, • Alcohol. Keep alcohol intake within safe limits. Current
to support patients to make and sustain lifestyle changes UK recommendations are 14 units of alcohol or less per
where appropriate and to help them make an informed week for both men and women.
decision regarding statins if they are at increased CVD • Take exercise.. Encourage the patient to incorporate exer-
risk. This should be based on an indvidualised assessment cise into his or her daily life, rather than rely on visiting
of the likely benefits and harms of statins. a gym or playing football at the weekend. Simple advice
• The following groups of people should be assumed to be from the GP is unlikely to change behaviour. A more
at high risk based on clinical history alone and do not supportive programme is needed.
require risk assessment with a scoring system: • Manage social isolation. Depression and lack of social
• People who have had a previous cardiovascular event support are associated with an increased risk of cardio-
(angina, MI, stroke, transient ischaemic attack, or vascular disease (Bunker et al., 2003), as is the combina-
peripheral arterial disease) tion of high workload and low autonomy at work
• People with diabetes (type 1 or 2) over the age of 40 years (Aboa-Eboule et al., 2007).
• People with familial hypercholesterolaemia • Control weight. Central obesity, rather than a raised BMI,
• CVD risk should be estimated using information already carries the greater cardiovascular risk and can be assessed
contained in the patient’s records, and using a chart or by measuring the waist circumference, with a single
computer programme based on epidemiologic data such cutoff point for each sex. Men with a waist circumference
over 94 cm and women over 80 cm are likely to have • Avoid itraconazole and ketoconazole (contraindicated
other risk factors for cardiovascular disease, and men with simvastatin).
with a waist circumference over 102 cm (women over • Reduce statin dose (max 20 mg simvastatin) with
88 cm) are 2.5 to 4.5 times as likely to have other major amlodipine, diltiazem, verapamil, and amiodarone.
cardiovascular risk factors (Han et al., 1995). Small • On subsequent visits, check that the patient is taking the
losses of weight are possible in primary care if advice statin. A study from Liverpool found that a quarter of
on diet and exercise is accompanied by a behavioural patients took their statin less than 80% of the time and
programme. had a higher mortality (Howell, Trotter, Mottram, &
• Diet. Encourage patients to: Rowe, 2004).
• reduce their meat and fat intake;
• eat oily fish at least twice a week; Blood Pressure Control
• eat bread, pasta, and potatoes as sources of carbohydrate; • Check blood pressure annually.
• eat at least five portions a day of fruit or vegetables; • Treat if levels are sustained at 140/90 mm Hg or above.
• use olive oil and rape seed margarine instead of • Aim for a level of below 140/90 mm Hg, or in those with
butter. type 2 diabetes below 140/80 mm Hg (and 130/80 mm Hg
if microalbuminuria or proteinuria is present).
Intensive Management When a 10-Year
Cardiovascular Disease Risk of At Least Talking to the Patient About Absolute Risks
10% Is Detected When Taking a Statin
Lipid Lowering NICE have produced guidance on the components of
• NICE now recommends offering atorvastatin 20 mg for good patient experience in adult NHS services, including
the primary prevention of CVD to people who have a recommendations on the communication of risk (NICE,
10% or greater 10-year risk of developing CVD 2014b). Shared decision making is particularly important
• Check TFTs, creatinine, LFTs, and fasting sugar. in the context of CVD prevention, as the benefits to indi-
Note that elevated liver enzymes are not a contrain- vidual patients (of taking a statin, for instance) may be
dication to the use of a statin. They are not associ- small and are not without the risk of harm. NICE have
ated with subsequent statin-induced toxicity (Chalasani developed a patient decision aid which provides absolute
et al., 2004). benefits and harms of taking a statin (NICE, 2014c). Ade-
• Check baseline lipids. NICE no longer recommends a quate time should be set aside for shared decision making
fasting test. using aids such as this. Discussions should be documented
• NICE recommends annual medication reviews for patients and can be re-visited in the future if patients change
and consideration of an annual, nonfasting, non-HDL their minds.
cholesterol blood test to inform the discussion. Aim for a
reduction of greater than 40% in non-HDL cholesterol Secondary Prevention
in both primary and secondary prevention.
• Stongly encourage physical activity in all those taking Consider the following measures in those with cardiovascu-
statins. In combination, mortality risk is greatly reduced lar disease, diabetes, chronic kidney disease, or primary
in those with dyslipidaemia (Kokkinos, Faselis, Myers, hyperlipidaemia.
Panagiotakos, & Doumas, 2013). • Recommend lifestyle changes as for primary prevention.
• Warn the patient to report muscle pain or weakness; if • Lipid-lowering measures:
present, check creatine kinase (CK) and TSH (Lasker & • Order the tests recommended under primary preven-
Chowdhury, 2012). tion and give the same warnings.
• If CK is 10 times or above upper limit of normal then • Offer a statin regardless of baseline cholesterol
STOP immediately. This is a risk of rhabdomyolysis. level.
• If CK is 5 times or below normal, this is rarely clini- • Start atorvastatin 80 mg in people with CVD. Use a
cally significant and is often related to exercise. lower dose if there are potential drug interections,
• If CK is not significantly raised, suggest rechallenging high risk of adverse effects or patient preference.
with a statin at a lower dose or switching statin (e.g., • Recheck the serum lipids after 3 months. If TC ≥
10 mg atorvastatin is equivalent to 40 mg simva- 4 mmol/L or LDL ≥2 mmol/L consider intensifying
statin). If myalgia recurs, try a nonstatin treatment treatment. If targets are still not reached, add ezeti-
(i.e., ezetimibe 10 mg). mibe. Other options are a fibrate (but with a warning
• Continue indefinitely unless adverse effects occur. about the increased risk of rhabdomyolysis), nicotinic
• Be aware of interactions: acid or an omega-3 fatty acid.
• When macrolides (e.g., erythromycin and clarithro- • If adverse effects occur consider reducing the statin
mycin) have to be prescribed, stop the statin and dose, or changing to another statin, or using ezeti-
restart 1 week after the macrolide is finished. mibe, a fibrate, or nicotinic acid.
• Blood pressure. Treat if levels are sustained at 140/90 mm Hg Familial Hypercholesterolaemia

or above. Aim for a level of below 140/90 mm Hg, or in
those with diabetes below 130/80 mm Hg.
• Antiplatelet therapy. Recommendations for antiplatelet GUIDELINE
therapy vary in different scenarios. These are now largely National Institute for Health and Care Excellence. (2008).
specialist-led decisions. Familial hypercholesterolaemia. NICE clinical guideline 71.
• ACE inhibition. Prescribe an ACE inhibitor to all patients Available at www.nice.org.uk.
with coronary heart disease. Titrate to maximum toler-
ated doses and continue long term.
• Prescribe a beta-blocker to all those who have had an MI. • Familial hypercholesterolaemia (FH) is important
Start them in patients whose MI was in the last 5 years because it carries a very high risk of premature CV mor-
and who therefore missed the opportunity to have them bidity and mortality. It is generally asymptomatic so
started in the acute stage. Patients at the highest risk is easily missed in general practice (Gill, Harnden, &
benefit most from beta-blockade (e.g., those aged >50 Karpe, 2012).
with angina, hypertension, or heart failure). • It is present in about 1 in 500 of the population in the
• Eplerenone. This aldosterone antagonist is now preferred United Kingdom and for men carries a 50% risk of a
to spironolactone in patients who have LV dysfunction major coronary event by the age of 50. In women the risk
following MI. is 30% by the age of 60. This risk is far higher than would
be predicted from the cholesterol level alone and requires
Annual Workup at a Coronary Heart Disease intensive therapy (high-dose statins and ezetimibe).
Prevention Clinic • Suspect FH as a possible diagnosis in adults with:
• a total cholesterol level greater than 7.5 mmol/l
• Check: and/or
1. fasting sugar; • a personal or family history of premature CHD (an
2. total cholesterol; event before 60 years in an index individual or first-
3. urine protein; degree relative).
4. BP; • NICE recommends systematically searching primary care
5. weight, height, and BMI. records for people:
• Action to be taken when a new abnormal result is • younger than 30 years, with a total cholesterol con-
found: centration greater than 7.5 mmol/l and
• Fasting sugar: • 30 years of older, with a total cholesterol concentra-
1. If fasting sugar is 7 mmol/L or higher and the tion greater than 9.0 mmol/l
patient has symptoms (thirst, polyuria, lethargy), • If suspicion of FH is raised:
this is diabetes. • Recheck the serum lipids with a fasting specimen.
2. If fasting sugar is 7 mmol/L or higher and the • Check for other causes of raised cholesterol (e.g.,
patient has no symptoms, repeat after 1 week. If hypothyroidism, excess alcohol consumption).
still 7 mmol/L or higher, diabetes is confirmed. • Ask about the family history across three generations.
3. If fasting sugar is between 6.1 and 6.9 mmol/L Accept that the patient may need to return with these
inclusive, check blood sugar 2 hours after a 75-g details after consultation with the family.
glucose drink (=394 mL of the new Lucozade for- • Ask about symptoms of CVD.
mulation which contains 73 kcal/100 mL): • Examine for evidence of CVD or skin or tendon
a. If greater than 11 mmol/L, diabetes is confirmed. manifestations of hyperlipidaemia.
b. If less than 7.8 mmol/L, this is impaired fasting • Make a clinical diagnosis of FH according to the Simon
glycaemia (IFG). Warn as for IGT (see the Broome criteria:
upcoming discussion). • FH exists if the total cholesterol is above 7.5 mmol/L
c. If between 7.8 and 11 mmol/L inclusive, this is or the LDL cholesterol is above 4.9 mmol/L AND
impaired glucose tolerance (IGT). Warn that it tendon xanthoma are present (in the patient or a first
carries an increased risk of diabetes, which can or second degree relative).
be reduced by exercise and diet. Recheck fasting • FH is possible if:
sugar annually. 1. the total cholesterol is above 7.5 mmol/L or the
• Proteinuria. If + or more, repeat after 1 week on the first LDL cholesterol is above 4.9 mmol/L AND there
morning specimen. If still + or more, send mid-stream is a first degree relative with a myocardial infarction
urine for culture and sensitivities, serum creatinine and before the age of 60 or a second degree relative with
electrolytes, and an albumin to creatinine ratio (ACR) on a myocardial infarction before the age of 50; or
a single urine sample. If the urine ACR is above 30 or there 2. the total cholesterol is above 7.5 mmol/L or the
is haematuria, or the creatinine is raised, this is significant LDL cholesterol is above 4.9 mmol/L in the patient
proteinuria (different thresholds apply in diabetes). AND also in a first or second degree relative (or
>6.7 mmol/L or >4.0 mmol/L, respectively, in a and rhythm control in patients with atrial fibrillation. The New
brother or sister under 16 years old). England Journal of Medicine, 347, 1825–1833.
• Referral is needed: Barnett, H., Burrill, P., & Iheanacho, I. (2010). Don’t use aspirin for
1. to a specialist with expertise in familial hypercholesterol- primary prevention of cardiovascular disease. British Medical
Journal (Clinical Research Ed.), 340, c1805.
aemia for all with a definite or possible clinical diag-
Barnett, K., Mercer, S. W., Norbury, M., et al. (2012). Epidemiology
nosis. Further investigation will include DNA testing of multimorbidity and implications for health care, research, and
for relevant mutations and family screening; medical education: A cross-sectional study. Lancet, 380, 37–43.
2. to a cardiologist if there are symptoms of CHD. Con- Bass, C., & Mayou, R. (2002). Chest pain. British Medical Journal
sider referral if the patient has no symptoms but there (Clinical Research Ed.), 325, 588–591.
is a family history of CHD in early adult life or the Beckett, N., Peters, R., Tuomilehto, J., et al. for the HYVET Study
patient has two or more other risk factors for CHD. Group. (2011). Immediate and late benefits of treating very elderly
• Cholesterol lowering in a patient with a confirmed diagnosis people with hypertension: Results from active treatment extension
of FH: to hypertension in the very elderly randomised controlled trial.
• Give a high-intensity statin (e.g., atorvastatin 40 mg British Medical Journal (Clinical Research Ed.), 344, d7541.
daily). Beckett, N. S., Peters, R., Fletcher, A. E., et al. for the HYVET Study
Group. (2008). Treatment of hypertension in patients 80 years of
• Aim for a LDL cholesterol over 50% below the pre-
age or older. The New England Journal of Medicine, 358, 1887–1898.
treated level. Benson, J., & Britten, N. (2002). Patients’ decisions about whether
• If the target is not met, increase the statin to the or not to take antihypertensive drugs: Qualitative study. British
maximum licensed dose, provided it is tolerated, and/ Medical Journal (Clinical Research Ed.), 325, 873–876.
or add ezetimibe. Brown, M., Cruickshank, J., Dominiczak, A., et al. (2003). Better
• Re-refer if the target is not met. blood pressure control: How to combine drugs. Journal of Human
• Review annually once stable. Stress that lifestyle Hypertension, 17, 81–86.
changes are even more important in someone with Bunker, S., Colquhoun, D., Esler, M., et al. (2003). Stress’ and coro-
FH than in the general population. nary heart disease: Psychosocial risk factors. The Medical Journal
of Australia, 178, 272–276.
Cairns, J. A., Connolly, S., McMurtry, S., Stephenson, M., & Talajic,
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8
Respiratory Problems
HILARY PINNOCK
Asthma Management of Patients With Lower Respiratory Tract
Diagnosis Symptoms With Focal Chest Signs or Where the Patient Is
Diagnosis of Asthma in Children at High Risk of Pneumonia or Is Systemically Ill
Diagnosis of Asthma in Infants CRB-65 Score
Management Vaccination
General Measures Tuberculosis
Therapeutic Management Chronic Obstructive Pulmonary Disease
Leukotriene Antagonists
Diagnosis
Other Therapeutic Options
Assessment of Severity
Management of Adults and Children 5 Years and Over Not
Controlled on a Moderate Dose of Inhaled Steroids and Management
Initial Add-on Therapy Drug Management
Devices Bronchodilators
Regular Review Inhaled Steroids
Personal Asthma Action Plans Combination Therapy
Indications for Outpatient Referral Mucolytic Therapy
Acute Asthma Severe and Very Severe Chronic Obstructive Pulmonary
Acute Asthma in Adults and Schoolchildren Disease
Acute Asthma in Preschool Children Indications for Referral for Specialist Advice
Lower Respiratory Tract Infection Acute Exacerbations of COPD
Clinical Assessment Assessment
Investigations Initial Treatment
Arrange Further Care
Management of Well Patients With Lower Respiratory Tract
Symptoms Without Focal Chest Signs
Asthma 2. Symptoms are episodic, often worse at night and are

commonly triggered by viral infections, exercise, and
Diagnosis (see Appendices) allergy.
3. An historical record of significantly lower peak flows
• The probability of asthma is assessed from a structured during symptomatic episodes provides objective evi-
clinical assessment, supported by lung function tests and dence of variability.
the diagnosis confirmed by response to treatment (BTS/ 4. A personal or family history of atopy supports the
SIGN, 2016). The process should be clearly documented diagnosis.
so that the evidence for the diagnosis is clear to future 5. Absence of symptoms, signs or clinical history to suggest
users of the clinical record. alternative diagnoses.
• Structured clinical assessment: • Lung function tests:
1. Variable symptoms of coughing, wheezing, chest tight- 1. Evidence of airflow obstruction. The presence and sever-
ness, and shortness of breath. ity of airflow obstruction can be assessed by spirometry,
93
GUIDELINES are raised in conditions that cause eosinophilic inflam-

mation, and therefore raised levels support, but do
BTS/SIGN, 2016 not prove, a diagnosis of asthma.
British Thoracic Society/Scottish Intercollegiate Guideline • The probability of asthma is based on a structured clinical
Network. (2016). British guideline on the management of
asthma. SIGN guideline 153. Available at www.brit-thoracic.
assessment supported by investigations:
org.uk; See https://www.sign.ac.uk/assets/sign153_figure1_ 1. High probability of asthma. Commence a carefully
diagnostic_algorithm.pdf. See also Appendices 11A–D. monitored initiation of treatment with a moderate
dose of inhaled steroids, and monitor the response,
GINA, 2018 both symptomatically and with repeated measures of
The Global Initiative on Asthma. (2018). Asthma prevention and lung function. A good response confirms a diagnosis
management: A practical guide for public health officials and
health care professionals. Available at www.ginasthma.com.
of asthma. A poor response, if inhaler technique and
compliance are good, should lead to reconsideration
NICE, 2017 of the diagnostic possibilities.
National Institute for Health and Clinical Excellence. (2017). 2. Low probability of asthma. Investigate and treat for the
Asthma: diagnosis, monitoring and chronic asthma more probable diagnosis, reconsidering asthma in those
management. Available from nice.org.uk/guidance/ng80. who do not respond.
ARIA, 2010 3. Intermediate probability of asthma. If the diagnosis is
Brożek, J. L., Bousquet, J., Baena-Cagnani, C. E., et al.
not clear, or the response to asthma treatment is poor,
(2010). Allergic Rhinitis and its Impact on Asthma (ARIA) arrange further investigations. Spirometry is the pivotal
guidelines: 2010 revision. Journal of Allergy and Clinical test as the differential diagnosis and approach to man-
Immunology, 126, 466–476. Available at http://www.euforea agement depends on whether the patient has airflow
.eu/assets/pdfs/aria/2010-ARIA-Report.pdf. obstruction. Consider referral for further tests, poten-
BSACI, 2017 tially including FeNO, if the diagnosis remains unclear.
Scadding, G. K., Kariyawasam, H. H., Scadding, G., et al.
(2017). BSACI guideline for the diagnosis and management of Diagnosis of Asthma in Children
allergic and non‐allergic rhinitis (Revised Edition 2017; 2007).
Clinical & Experimental Allergy, 47, 856–889. Available from The diagnosis of asthma in children follows a similar process
http://www.bsaci.org/Guidelines/rhinitis-2nd-edition-guideline. of establishing probabilities and observing the response to
BTS Emergency Oxygen Guidelines, 2017 a trial of treatment (BTS/SIGN, 2016). There are, however,
O’Driscoll, B. R., Howard, L. S., Earis, J., et al. (2017).
some specific caveats:
BTS guideline for oxygen use in adults in healthcare and • Clarify what parents mean when they use the word wheeze
emergency settings. Thorax, 72(Suppl 1), ii1–ii90. (Cane, Ranganathan, & McKenzie, 2000).
• Assess on the basis of symptoms supplemented by lung
function tests. Children under 7 cannot reliably use a
but because asthma is a variable condition, asymp- peak flow meter, though school-age children can usually
tomatic patients will commonly have normal lung perform spirometry if encouraged by a skilled technician.
function. This does not exclude asthma. • A period of watchful waiting may be appropriate for
2. Objective evidence of variability. Diurnal variation greater children who cannot perform spirometry and who are
than 20% may conveniently be demonstrated by record- minimally symptomatic.
ing morning and evening peak flows over 2 weeks and
demonstrating the typical sawtooth pattern. A fall in Diagnosis of Asthma in Infants
peak flow exceeding 20% when the patient next meets
his or her trigger, or an increase over 20% in response Wheezy infants present particular diagnostic difficulties, and
to treatment, may be noted as part of home charting. there is little evidence-based data to guide decisions.
Note that a normal peak flow at a consultation does • Exclude serious pathology (e.g., cystic fibrosis, congenital heart
not exclude the diagnosis of asthma. Asthma is a vari- defects, inhaled foreign body, oesophageal problems). Referral
able condition, and readings need to be taken over to a paediatrician is appropriate if the diagnosis is in doubt.
time to check for variability. • Distinguish between asthma and viral-associated wheeze
3. A formal reversibility test. If the presenting lung (Table 8.1). This can be a difficult distinction to make,
function is obstructive or the patient is symptomatic, and the diagnosis may only be clear in retrospect, as
it may be possible to demonstrate a significant response children with viral-associated wheeze will usually lose that
to a single dose of a β-2 agonist or a 6-week course tendency by the time they reach school age.
of inhaled steroids. An increase in forced expiratory 1. Consider risk factors and the clinical history.
volume in 1 second (FEV1) greater than 400 mL or 2. Record the presence of wheeze heard by a health
an increase in peak flow over 20% suggests asthma. professional.
4 Fractional exhaled nitric oxide (FeNO) can be measured 3. A carefully supervised trial of treatment (e.g., inhaled
if facilities are available. Levels of exhaled nitric oxide steroids for 8 weeks) may be helpful (Bush, 2000;
CHAPTER 8 Respiratory Problems 95
TABLE Distinguishing Between Asthma and Viral- • Inhaled steroids are are recommended as the first treatment
8.1 Associated Wheeze step for almost all people with symptomatic asthma (BTS/
SIGN, 2016). Regular treatment improves lung function,
Suggestive of Viral-
reduces symptoms, reduces use of rescue bronchodilation,
Suggestive of Asthma Associated Wheeze
and reduces the risk of exacerbations (Adams, Bestall, &
Family or personal history Prematurity, small for dates Jones, 1999; Adams, Bestall, Lasserson, Jones, & Cates,
of atopy 2008; Adams et al., 2005).
Maternal smoking • The correct dose of inhaled steroids is the lowest dose
Cough and wheeze not Cough and wheeze only compatible with asthma control. Many adults will be
associated with viral illness with viral infections controlled on a low dose: beclomethasone or budesonide
Good response to Poor response to 400 µg daily or fluticasone 250 µg daily. BTS/SIGN,
treatment and relapse on treatment 2016 has a useful table of comparative doses.
cessation of treatment • Discuss attitudes to regular treatment. Compliance with
inhaled steroid treatment is poor (van Staa, 2003) and may
be improved by strategies such as simplifying regimes, regular
supportive review, and reminders (Nieuwlaat, 2014).
Cochran, 1998). Doses up to beclomethasone 200 µg • Explain ways of avoiding local side effects. Candidiasis
daily, budesonide 200 µg daily, or fluticasone 100 µg may be reduced by rinsing the mouth after administration
daily via a spacer ± mask may be needed to overcome or using a spacer device; dysphonia may be improved by
practical problems with delivering inhaled therapy to reducing the dose.
very young children. • Reassure patients that important systemic side effects are
4. A poor response to therapy makes asthma unlikely, unlikely at moderate doses (beclomethasone or budesonide
and treatment should be stopped. <800 µg daily, fluticasone <500 µg daily). In children,
5. A good response to therapy should be confirmed by despite an initial reduction in growth velocity, final height
a stepped withdrawal of treatment to exclude coinci- is not significantly affected (Agertoft & Pedersen, 2000;
dental improvement. Childhood Asthma Management Program Research Group,
2000). High-dose inhaled steroids (beclomethasone or
budesonide >1500 µg daily, fluticasone >750 µg daily)
Management have an increased risk of adrenal suppression, cataracts,
General Measures reduced bone density, and bruising (Adams et al., 2008),
• Advise on the avoidance of triggers: and risks should be balanced against benefits (e.g., in
1. Asthma UK and Allergy UK produce useful informa- people using frequent/regular oral steroids to treat
tion for patients about allergen avoidance. exacerbations).
2. Common allergens include pets, especially cats, house • β-2 agonists provide symptomatic relief and should be
dust mite, and pollen. prescribed on an as required basis (BTS/SIGN, 2016;
3. Current chemical and physical measures for reducing Walters, Walters, Gibson, & Jones, 2003).
house dust mite allergen have not been shown to be • Patients requiring relief medication more than three times
effective (Gøtzsche & Johansen, 2008). a week, waking with asthma on more than one night a
4. Rhinitis is a common comorbidity and may be effec- week, or who have had an exacerbation in the last 2 years
tively treated with intranasal steroids (Scadding, 2017; should be offered regular prevention with inhaled
Taramarcaz & Gibson, 2003). steroids.
5. Adults in whom an occupational cause for their symp- • Exercise-induced asthma that persists despite good asthma
toms is suspected should be referred. control with inhaled steroids may be prevented by using
• Encourage smoking cessation. Both active and passive a β-2 agonist prior to exercise.
smoking status should be documented and support offered • Long-acting β-2 agonists are given regularly twice a day
to those wishing to quit. as add-on therapy to people whose asthma is not controlled
• Consider drug effects: on inhaled steroids (BTS/SIGN, 2016). The addition of
1. Beta-blockers (systemic or topical eye drops) are long-acting β-2 agonists improves lung function and
contraindicated. symptom control compared with increasing the dose of
2. Asthmatics sensitive to aspirin should not use nonster- inhaled steroids (Pauwels et al., 1997; Walters, Gibson,
oidal antiinflammatory drugs (NSAIDs). Lasserson, & Walters, 2007).
• In response to concerns about a very small increased
Therapeutic Management (see Appendices 11A risk in patients on long-acting β-2 agonists of asthma
and 11B) deaths (Nelson et al., 2006), and serious adverse events
• Patients should start treatment at the step most appropri- (Cates & Cates, 2008; Cates, Cates, & Lasserson, 2012;
ate to the severity at presentation (see Appendices 11A–C) Cates, Oleszczuk, Stovold, & Wieland, 2012), guidelines
(BTS/SIGN, 2016; GINA, 2018). emphasize the importance of maintaining an adequate
dose of inhaled steroids. Patients should be counselled Management of Adults and Children 5 Years
appropriately. and Over Not Controlled on a Moderate Dose
• Combination inhalers have the advantage of ensuring of Inhaled Steroids and Initial Add-on Therapy
that patients cannot take long-acting β-2 agonists without
taking inhaled steroids and simplify treatment regimes, • There is little evidence to guide treatment in patients
but with the disadvantage of that it is more difficult to poorly controlled on moderate doses of inhaled steroid/
adjust the dose of individual components. long-acting β-2 agonists. Options include a trial of a
• Flexible use of a budesonide/formoterol combination as second add-on treatment (e.g. a long acting muscarinic
a rescue medication in addition to regular use as a pre- receptor antagonist [NICE 2017; BTS/SIGN 2016) or
venter therapy is an effective treatment option for selected high dose inhaled steroid.
adult patients who are poorly controlled with inhaled • Consider referring patients requiring additional add-on
corticosteroids (ICS) and β-2 agonists. Careful self- therapy for specialist advice, especially if control remains
management education about this strategy is required. poor.
Leukotriene Antagonists Devices

• Leukotriene antagonists are mediator antagonists that The effectiveness of inhaled therapy depends on inhaler
are an option as an add-on therapy (BTS/SIGN, 2016; technique and the particle size produced by the device. Inhaler
Chauhan, 2017; NICE 2017). A review concluded that devices should be selected for individual patients, taking the
although they were less effective than long-acting β-2 following into consideration (Table 8.2):
agonists in relieving symptoms over 12 weeks, the reduc- 1. The patient’s ability to use a device. This should always be
tion in exacerbations over 2 years was similar, with some checked before an inhaler is prescribed and rechecked
studies showing an improved safety profile (Joos et al., regularly. Only a minority of patients using a metered
2008). dose inhaler have adequate inhaler technique, with slightly
• A trial in primary care demonstrated that, in real-life better performance for other devices (Brocklebank et al.,
practice, when factors such as patient preference for inhal- 2001). Technique should be observed for:
ers or tablets, ability to use inhalers, attitudes to steroids, • the patient’s inspirational flow rate compared to flow
and adherence may all influence effectiveness, there may rate recommended by the manufacturer for efficient
be little difference between long-acting β-2 agonist or actuation and inhalation;
leukotriene antagonist treatment options (Price et al., • hand/breath coordination;
2011). • ability to follow the instructions for correct use.
• Leukotriene antagonists are recommended as the first line 2. The drug and dosage required. High doses of inhaled steroid
add-on therapy for children under 5 years. should be given by a spacer device.
3. National Institute for Health and Care Excellence (NICE)
Other Therapeutic Options guidelines recommend the use of spacers (with masks for
• Sodium cromoglicate may have a small overall treatment infants) for children under the age of 5 (NICE, 2000).
effect but should not be first line treatment for the pre- Older children will need careful assessment of ability to
vention of asthma (van der Wouden et al., 2008). use inhalers, and more portable options will be appro-
• A short rescue course of oral steroids may be needed at propriate especially for bronchodilators (NICE, 2002).
any time and at any step, but frequent use should prompt 4. Patient preference and lifestyle issues may be relevant.
referral to a respiratory physician. 5. Cost of the device.
TABLE
8.2 Choice of Inhaler Device for Different Age Groups
Age Devices to Consider Comments

Preschool MDI + spacer (with a mask for infants) NICE (2000) recommendation
School-age MDI + spacer Recommended for inhaled steroids because it reduces oral deposition
(NICE, 2002)
Dry powder devices Once a child has adequate inspiration and can be relied on not to blow
by mistake
Breath-actuated devices Once inspiration is adequate
Adults MDI If technique is adequate. Should be used with a spacer to deliver high
doses of inhaled steroid
Breath-actuated devices
Dry powder devices
MDI, Metered dose inhaler.

2. Responding to the assessment by identifying reasons for

Regular Review poor control and adjusting the management strategy
Patients receiving treatment for asthma should be reviewed accordingly (Ryan, 2013):
regularly by a general practitioner (GP) or trained asthma • Systematically consider reasons for poor control
nurse. Reviews in primary care, conducted face to face or, (Haughney, 2008):
if a patient is well controlled, by telephone (Pinnock et al., • Review the diagnosis. Failure to respond to treatment
2003, 2007), should encompass the following three steps may indicate an incorrect diagnosis or the develop-
(Pinnock et al., 2010): ment of a comorbid cause for symptoms.
1. Assessing current symptom control and risk of future • Check and correct inhaler technique.
exacerbations • Assess and address adherence. Patients and their health-
• Specific morbidity questions. Patients underreport symp- care professionals both overestimate compliance with
toms (Haughney, Barnes, Partridge, & Cleland, 2004), regular medication. Nonjudgmental questioning and
and control should be assessed by asking standard achieving concordance about treatment goals may
morbidity questions at every asthma review consulta- improve compliance (Nieuwlaat, 2014).
tion. The Royal College of Physicians’ “three questions” • Ask about and treat rhinitis. Three quarters of people
(RCP3Qs) is an example of a suitable morbidity score with asthma have symptoms of rhinitis, which
(Table 8.3) (Pearson & Bucknall, 1999). A negative should be treated with nasal steroids (Brożek, 2010;
response to all three questions suggests good control; Scadding, 2017; Thomas & Price, 2008).
two or more positive answers indicate poor control • Assess smoking status, and offer cessation advice.
(Pinnock et al., 2012). Interpret responses in the light Smoking adversely affects asthma control. This may
of other indicators of control (such as exacerbations be because of a comorbid diagnosis of chronic
and use of reliever inhalers). obstructive pulmonary disease (COPD) or because
• Patient reported cutcomes: control questionnaires. Short smoking reduces the effectiveness of inhaled steroids.
questionnaires for measuring asthma control include Persistent smokers need relatively high doses of
the Asthma Control Questionnaire (Juniper, Bousquet, inhaled steroids.
Abetz, & Bateman, 2006) and the Asthma Control • Adjust therapy according to evidence-based guidelines.
Test (Nathan et al., 2004). They are validated for 3. Exploring patients’ ideas, concerns, and expectations
patient self-completion: completion with the assistance and supporting self-management to facilitate ongoing
of the clinician may influence the result (Honkoop control:
et al., 2013). • Supported self-management. Every asthma consultation
• Use of relief medication. Good asthma control is associ- is an opportunity to review, reinforce, and extend
ated with little or no need for short-acting β-2 agonists. asthma knowledge and skills (BTS/SIGN, 2016).
At the other extreme, use of 12 or more canisters a Education should aim to empower patients to be in
year is a marker for at-risk asthma (RCP, 2104). control of their asthma and should include the provi-
• Assessing risk of future attacks. A history of acute sion of written personalised asthma action plans
attacks, poor current symptom control, over-reliance of (Pinnock, 2015).
short-acting beta agonists are key predictors of future
risk (Buelo 2018). Personal Asthma Action Plans
Education involving self-monitoring and written action plans
reduces hospitalizations, emergency consultations, days off
work or school, and nocturnal asthma (Pinnock, 2017;
Pinnock, 2015).
• Offer all people with asthma a written asthma action
TABLE Royal College of Physicians Three Questions plan, which should (Gibson & Powell, 2004):
8.3 (Pearson & Bucknall, 1999) 1. be tailored to the individual patient, taking into account
In the past week (or month): his or her clinical condition and preference for
autonomy;
1 Have you had difficulty sleeping Yes/No
because of your asthma symptoms 2. involve self-monitoring, based on symptoms and/or
(including cough)? peak flows;
3. provide information about features that would indicate
2 Have you had your usual asthma Yes/No
symptoms during the day (cough, the patient’s asthma is worsening;
wheeze, tight chest, or 4. advise what action the patient should take, including
breathlessness)? information about increasing inhaled steroids and start-
3 Has your asthma interfered with your Yes/No ing oral steroids. A recent trial in adults shows that a
usual activities (housework, work or four fold increase in ICS at the onset of an attack
school, etc.)? reduces the risk of need a course of oral steroids by
20% (McKeever, 2018).
An example of a PAAP can be found on the Asthma UK N’Diaye, 2003). Early administration improves out-
website: www.asthma.org.uk. comes; prednisolone 40 to 50 mg for adults, 30 mg
for children should therefore be given at the consulta-
Indications for Outpatient Referral tion. The number needed to treat (NNT) to prevent
Referral to a respiratory physician or paediatrician should one admission in patients with severe asthma is five
be considered if: (95% confidence interval [CI] 4 to 7).
1. the diagnosis is not clear; 4. Assess response.
2. the patient is not controlled with moderate doses of inhaled Note: Infective triggers for acute asthma are usually viral.
steroids ± add-on therapy and inhaler technique is Routine prescribing of antibiotics is not appropriate.
satisfactory; • Arrange further care.
3. there is a possibility of occupational asthma; • Admit:
4. there is a history of life-threatening attacks or brittle 1. patients with life-threatening asthma;
asthma. 2. patients with severe asthma who do not respond
to emergency bronchodilation.
Other factors (e.g., time of day, social situation, previ-
Acute Asthma ous history of severe attacks) will influence the
Acute Asthma in Adults and Schoolchildren (See decision to admit.
Appendices 11C and D) • Instructions:
Delay is the most common factor identified as contributing 1. Bronchodilation may be repeated 3 to 4 hourly.
to asthma deaths, often related to a failure on the part Ideally, progress should be monitored by peak
of the patient or the healthcare professional to appreci- flow.
ate the severity of the attack (British Thoracic Associa- 2. Prednisolone 40 to 50 mg daily for at least 5 days
tion, 1982; RCP, 2014). The presence of psychosocial or until recovery (adults); 30 mg daily for 3 days
problems is an important risk factor for fatal asthma (children).
(Mohan et al., 1996). 3. Increase (or recommence) inhaled steroids until full
• Assess severity and record the following: control is regained.
1. General condition. Inability to speak, exhaustion, 4. Ensure the patient knows when and how to call
cyanosis, and a silent chest indicate life-threatening further medical help.
asthma. • Provide early follow-up. Arrange to assess progress within
2. Peak flow. Peak flow (PF) at presentation should be 24 hours (severe asthma) or 48 hours (uncontrolled
compared with the patient’s best (or predicted, if best asthma).
is not known): • Review. Arrange for a review of overall asthma control
• PF <75% of best = moderate asthma exacerbation; and revision of wriiten Personal Asthma Action Plan.
• PF <50% of best = acute severe asthma;
• PF <33% of best = life-threatening asthma.
3. Respiratory rate (RR). More than 25/min in adults, Acute Asthma in Preschool Children (See
more than 30/min in children over 5 years indicates Appendix 11D)
severe asthma. • Assess severity and record: exhaustion, agitation, drowsi-
4. Heart rate (HR). More than 110/min in adults, more ness, too breathless to feed, use of accessory muscles,
than 125/min in children over 5 years indicates severe RR over 40/min or HR over 140/min are signs of severe
asthma. asthma.
5. Oximetry. Oxygen saturation below 92% should alert • Act promptly to provide relief
the clinician to the severity of the attack. 1. Bronchodilation: multiple (10) doses of a β-2 agonist
• Act promptly to provide relief: through a large volume spacer ± mask or 2.5 mg sal-
1. Bronchodilation. Administer multiple (10) doses of a butamol or 5 mg terbutaline by nebulizer.
β-2 agonist through a large-volume spacer, or 5 mg 2. Oxygen should be given at high flow rates.
salbutamol/by oxygen-driven nebulizer (Cates, Welsh, 3. Systemic steroids: Consider soluble prednisolone
& Rowe, 2013). Nebulizers require flow rates of 6 to 20 mg.
8 L/min. Consider adding ipratropium in severe or 4. Assess response: RR, HR, and general condition.
life-threatening asthma. • Arrange further care. Admit:
2. Oxygen. Give at high flow rates for severe or life- 1. children with any features of severe or life-threatening
threatening asthma aiming to maintain oxygen asthma;
saturation between 94% and 98% (O’Driscoll, 2. children who do not respond to bronchodilation or
2017). who relapse within 3 hours.
3. Systemic steroids. Steroids reduce mortality, relapses, • Poor social circumstances or a previous history of a severe
subsequent hospital admission, and the requirement attack should lower the threshold for admission.
for β-2 agonist therapy (Rowe, Spooner, Ducharme, • Children not admitted should be given prednisolone 20 mg
Bretzlaff, & Bota, 2001; Smith, Iqbal, Rowe, & for 3 days and clear instructions for review.
PROFESSIONAL GROUPS • The majority of previously well patients who present to

their GP with acute respiratory symptoms have a self-
• Primary Care Respiratory Society (PCRS): http://www.
limiting illness (Macfarlane et al., 2001).
pcrs-uk.org. The PCRS is the UK-wide professional society
supporting primary care to deliver high value patient • Fewer than half have evidence of a bacterial infection
centred respiratory care. They are active in campaigning, (Macfarlane et al., 1993, 2001).
research, and education. Their website provides opinion • Without a chest x-ray (CXR), identification of the 6%
sheets, resources for practices. Their scientific journal is npj of patients with lower respiratory tract infection who
Primary Care Respiratory Medicine.
have community-acquired pneumonia is imprecise, with
• International Primary Care Respiratory Group (IPCRG):
http://www.theipcrg.org. The IPCRG is an organisation of no individual sign or symptom being reliably predictive
national primary care respiratory organisations and a (Macfarlane et al., 2001; Metlay, Kapoor, & Fine, 1997).
global community of practice. Its website includes
practical guidance and resources for primary care, and
links to websites of member countries. Clinical Assessment
• British Thoracic Society (BTS): http://www.brit-thoracic.
org.uk. The BTS exists to improve standards of care for The aim of the initial history and examination is to identify
people who have respiratory diseases and to support patients with, or at risk of developing, severe or complicated
and develop those who provide that care. BTS guidelines illness.
may be downloaded from the website. Also available • Identify high-risk groups. The risk of pneumonia increases
are information sheets for patients on a wide range of
with age and in institutionalized patients. Clinical features
respiratory problems.
are less reliable in the elderly.
• Ask about comorbidity. Premorbid conditions that increase
the risk of pneumonia include COPD (with increased
RESPIRATORY TRAINING risk in patients using inhaled steroids), congestive heart
Education for Health: http://www.educationforhealth.org/.
failure, neurologic disease, diabetes, alcoholism, and
chronic renal or hepatic failure (Farr et al., 2000).
• Examine the chest. The presence of localized chest signs
is positively correlated with radiographic evidence of
PATIENT GROUPS pneumonia, but their absence does not exclude the diag-
• Asthma UK: http://www.asthma.org.uk. 18 Mansell nosis (Macfarlane et al., 2001; Metlay et al., 1997).
Street, London, E1 8AA; tel. 0300 222 5800. Asthma UK • Assess severity. Fever above 40°C, RR over 30/min, BP
provides information leaflets about asthma for patients below 90/60, pleuritic chest pain, or confusion indicate
and resources such as asthma action plans which can be severe infection (Ewig, Schafer, & Torres, 2000; Fine et al.,
ordered or downloaded from the website.
1996) and should prompt referral.
• British Lung Foundation: www.blf.org.uk/. 73-75 Goswell
Road, London EC1V 7ER, tel. 030 555. The BLF produces • Assess oximetry. An oxygen saturation below 92% indicates
information for patients about a wide range of respiratory significant hypoxia and should prompt referral.
diseases which may be ordered or downloaded from the
website. It runs local Breathe Easy groups.
• Allergy UK: http://www.allergyuk.org/. Planwell House, Investigations
LEFA Business Park, Edgington Way, Sidcup, Kent, DA14
5BH. Helpline 01322 619 898. Allergy UK provides a Investigations are rarely needed in primary care, but consider:
dedicated helpline, support network, and online forum for 1. CXR may be indicated if there are focal chest signs and
those with allergy and intolerance. in high-risk patients or those with signs of severe disease
even in the absence of focal chest signs. Persistence of
systemic illness, the development of unexpected symptoms
(e.g., haemoptysis or pleuritic pain), or failure of respira-
Lower Respiratory Tract Infection tory symptoms to resolve should prompt investigation;
2. a raised C-reactive protein (CRP) (>50 mg/L) is positively
GUIDELINES
correlated with radiographic evidence of pneumonia, but
NICE (RTI), 2008 is of no value in assessing severity;
National Institute for Health and Clinical Excellence. (2008). 3. a raised blood urea (>11 mmol/L) and a leukocytosis
Prescribing of antibiotics for self-limiting respiratory tract (>20,000 white blood cells [WBC]/mL) or leukopenia
infections in adults and children in primary care. NICE clinical (<4000 WBC/mL) indicate severe infection and should
guideline 69. Available at www.nice.org.uk/cg69.
prompt urgent referral (Ewig et al., 2000; Fine et al., 1996).
BTS (CAP), 2009
Lim, W. S., Baudouin, S. V., George, R. C., et al. (2009). BTS Management of Well Patients With Lower
guidelines for the management of community acquired Respiratory Tract Symptoms Without Focal
pneumonia in adults: Update 2009. Thorax, 64, Siii1–55. [A
primary care summary is available at https://www.nature. Chest Signs
com/articles/pcrj201014.]
• Once patients with, or at risk of developing, severe or
complicated illness have been excluded, well patients with
lower respiratory tract infections can be managed with • Prescribe antibiotics. Antibiotic treatment should not be
explanation, self-management of symptoms, and safety delayed if a diagnosis of pneumonia is suspected:
netting. • Use amoxicillin 500 mg to 1 g three times a day for
• Discuss the natural history of lower respiratory tract illness. 5 to 7 days (doxycycline 200-mg loading dose then
The provision of leaflets explaining the nature of a cough 100 mg daily or clarithromycin 500 mg twice a day
and the expected duration (up to 3 weeks) can reduce are alternatives).
reconsultation rates (Macfarlane, Holmes, & Macfarlane, • Doxycycline or clarithromycin are first choice for
1997). Advise that failure to improve as expected, deterio- mycoplasma pneumonia.
ration, or development of new symptoms should prompt • Local microbiologists can provide information about
reconsultation. local resistance patterns.
• Provide advice on symptomatic treatment: • Consider the need for admission. Admission is recom-
• Paracetamol, extra fluids, hot lemon, and bed rest are mended in the presence of (Levy et al., 2010):
commonly recommended in the acute phase, although 1. signs of severe disease (fever >40°C, RR >30/min, BP
there are no studies to validate these common remedies. <90/60, pleuritic chest pain, confusion). These param-
• Zinc used within 24 hours of the onset of symptoms eters may be combined in the CRB-65 score to inform
may improve the resolution rate of upper respiratory the decision to admit (see upcoming discussion);
tract symptoms (Hemilä, 2011). 2. high-risk conditions (e.g., pneumonia associated
• The use of vitamin C supplements to prevent or treat with influenza, chickenpox);
respiratory infection is unproven (Hemilä, 2013). 3. suspected complications (e.g., pleural effusion,
• Cough medicines are often bought over the counter, malignancy);
but there is no evidence to support effectiveness (Chang, 4. diagnostic uncertainty.
Cheng, & Chang, 2014). • Factors that may contribute to the decision to admit are:
• Avoid prescribing antibiotics in well patients with no addi- 1. comorbidity (COPD, CCF, diabetes, alcoholism,
tional risk factors. Benefits from antibiotics are modest and, chronic renal or hepatic failure);
for most patients, will not outweigh the risk of adverse 2. unsatisfactory social circumstances;
effects, the costs, or the negative consequences of antibiotic 3. failure to respond to first line antibiotics.
resistance (Smith, Fahey, Smucny, & Becker, 2017). • Arrange follow-up. Consider investigating or admitting
• Antibiotics increase the resolution of symptoms by patients who fail to respond. Fever should settle within
about half a day. 2 days, but some symptoms such as cough may persist
• Patients who also have the typical symptoms of upper for a few weeks after the antibiotic course has finished.
respirtatory tract infection (URTI) and have been ill If symptoms persist and the initial CXR was abnormal,
for less than 1 week may be least likely to benefit from it should be repeated 6 weeks later to exclude underlying
antibiotics. malignancy, especially in patients who smoke.
• Declining the request for antibiotics and educating
patients on the limitations and disadvantages of treat- CRB-65 Score
ment is effective in reducing antibiotic use, does not
increase reattendance rates for the same episode, and CRB-65 is a validated score for assessing severity of pneu-
reduces the likelihood that the patient will consult monia (Bauer et al., 2006). Score 1 point for each of the
with a subsequent self-limiting respirary infection. following parameters: confusion, respiratory rate over 30/
• Use the opportunity to provide smoking cessation min, BP below 90/60, 65 years or older.
advice. • CRB-65 0: Low risk. Referral to hospital not indicated
for clinical reasons.
Management of Patients With Lower • CRB-65 1 or 2: Increased risk. Consider the need for
Respiratory Tract Symptoms With Focal Chest hospital assessment.
Signs or Where the Patient Is at High Risk of • CRB-65 3 or 4: High risk of death. Arrange urgent hos-
Pneumonia or Is Systemically Ill pital admission.
• Community-acquired pneumonia is a potentially serious Vaccination

condition with a mortality in the community of less than
1%, In people admitted with pneumonia, mortality rises • Influenza vaccine is recommended for the following groups
to between 5% and 14%. of patients:
• Consider the need for a CXR if the diagnosis is in doubt, • all children aged two to nine
there is concern about underlying pathology, or there is • those aged six months to under 65 years in clinical
a poor response to treatment (Levy et al., 2010). risk groups
• Assess oxygen saturation and consider referral if previously • pregnant women
healthy patients have an oxygen saturation below 94% • those aged 65 years and over
(Levy et al., 2010). • those in long-stay residential care homes
• carers languages are available on the British Lung Foundation

• frontline health and social care workers website (https://shop.blf.org.uk/collections/lung-health
Three forms of vaccine are available: -information/products/tuberculosis) and from TB Alert
• a quadrivalent live attenuated influenza vaccine (LAIV), (https://www.thetruthabouttb.org/professionals/patient
administered as a nasal spray for children (excluding -support/).
those with immunodeficiency or severe asthma) • Be prepared for concerned enquiries from contacts. Contact
• A trivalent inactivated flu vaccine for adults 65 years tracing is undertaken according to defined procedures usually
and over by a respiratory nurse specialist. Close contacts are defined
• A quadrivalent influenza vaccines for adults under as people from the same household, close friends, or fre-
65years. quent visitors to the household. Most other contacts are
For further details see Public Health England: Flu plan usually casual contacts and are at considerably lower risk
2017/18. of infection. In the event of a local outbreak, the local
• Pneumococcal vaccination is recommended for people Consultant in Communicable Disease can provide advice.
over 65 and those with comorbidities.
Chronic Obstructive Pulmonary Disease
Tuberculosis
GUIDELINES
GUIDELINES NICE (COPD), 2010
National Institute for Health and Care Excellence. (2016). National Institute for Health and Care Excellence. (2010).
Tuberculosis. NICE clinical guideline 33. Available at https:// Management of chronic obstructive pulmonary disease in
www.nice.org.uk/guidance/ng33. adults in primary and secondary care. NICE clinical guideline
101. Available at www.nice.org.uk/cg101.
GOLD, 2013
Patients with tuberculosis (TB) should be managed by physi- Global Initiative for Chronic Obstructive Lung Disease. (2018).
cians or paediatricians with specialist expertise in the man- Global strategy for the diagnosis, management and
prevention of chronic obstructive pulmonary disease.
agement of TB and supported by TB nurse specialists or Available at www.goldcopd.com.
health visitors. The role of primary care is to:
• Be alert to the possibility of TB. The incidence of TB is
particularly high among people born in countries with a Diagnosis
high TB burden, with almost two-thirds of TB cases in
migrants occurring within 6 years of entering the country • The aim is to make an objective diagnosis of COPD as
(Public Health England, 2017). New patient medicals early as possible in the course of the disease to encourage
may be an opportunity to check the bacillus Calmette- smoking cessation and prevent progression.
Guérin (BCG) status of immigrants (Griffiths et al., 2007). • Ask about smoking. Smoking history may be recorded as
Other high-risk groups include patients with impaired pack years (i.e., 1 pack year = 20 cigarettes a day for 1
immunity (including human immunodeficiency virus year). Note any occupational exposure (e.g., coal mining).
[HIV]), patients on steroids, the homeless, and those • Ask about symptoms of cough, sputum production, wheeze,
dependent on alcohol or drugs. and breathlessness and their impact on lifestyle. These
• Consider the diagnosis. Symptoms of fever and night sweats, symptoms have an insidious onset, usually after the age
cough, weight loss, and haemoptysis should prompt inves- of 35 years, and progress slowly with little variability.
tigation, though primary care clinicians should be alert Suspect bronchial carcinoma if there is haemoptysis.
to the wide range of nonspecific symtoms that occur in • Examine the chest. The chest will usually be clear in mild
people with TB (Metcalf, Davies, Wood, & Butler, 2007). COPD; rhonchi may develop during exacerbations and
A CXR should be arranged and three sputum samples as the disease progresses. Localized signs may indicate an
may be sent for microscopy, culture, and sensitivity and underlying carcinoma.
should be marked as “suspected TB.” • Record body mass index (BMI). Low BMI is associated
• Ensure prompt referral. Most patients can be treated as out- with a poor prognosis.
patients. Inpatient care may be considered because of clinical • Check for signs of chronic hypoxia. Cyanosis and confusion
severity or to ensure compliance with treatment regimes. can occur with the onset of respiratory failure; ankle
• Encourage compliance. Treatment regimes will involve a oedema suggests cor pulmonale.
combination of drugs and will continue for at least 6 • Arrange spirometry. The diagnosis should be confirmed
months. Supervision will be arranged by the hospital clinic by spirometry, which should be undertaken when the
in accordance with local policy. Directly observed therapy patient is stable (i.e., at least 6 weeks should have elapsed
may be needed to ensure compliance. since an exacerbation). Spirometry in COPD demonstrates
• Support the patient and family. Information for clinicians an obstructive pattern (forced expiratory volume in 1
and answers to frequently asked questions in a range of second/forced vital capacity [FEV1/FVC] <70%).
• Consider the need to exclude asthma. In most cases the • Composite indices. There is increasing interest in multi-
diagnosis of COPD will be suggested by the combination component assessments. For example, the DOSE index
of clinical history, signs, and spirometry that does not (D = dyspnoea, O = airflow obstruction, S = smoking
return to normal with drug therapy. In some cases, to status, E = exacerbation frequency) is designed for use
exclude asthma, it will be important to confirm the lack in primary care and predicts use of healthcare resources
of reversibility. This may be demonstrated by: (Jones et al., 2009). An alternative, more suited to use
1. a poor response (<400 mL increase in FEV1) to bron- in secondary care, is the BODE index (B = body mass
chodilators, inhaled, or oral steroids; index, O = airflow obstruction, D = dyspneoa, E = exercise
2. serial peak flow measurements showing less than 20% capacity measured by the 6-minute walk test) predicts
variability. This may be undertaken as a therapeutic mortality (Celli et al., 2004).
trial or as a formal reversibility test.
• Consider the need for other investigations: Management
1. A CXR should be considered at initial presentation
to exclude other pathology. • Treatment is aimed at providing the best possible relief
2. An oxygen saturation of less than 92%, the presence of symptoms, preventing exacerbations, and improving
of polycythaemia or peripheral oedema suggests chronic quality of life. As the disease progresses, treatment levels
hypoxia and should prompt referral for blood gas and professional support should be stepped up to provide
estimations. adequate palliation.
• Use every opportunity to encourage the patient to stop smoking.
Assessment of Severity This is the only intervention that can prevent the acceler-
ated decline in lung function that occurs in patients with
• Postbronchodilator FEV1 expressed as a percentage of pre- COPD (Anthonisen, 1994; Fletcher & Peto, 1977; Koha-
dicted may be used to classify the severity of airflow nsal et al., 2009). It is essential that patients understand
obstruction: the implications of continuing to smoke and the benefit
• Mild COPD is defined as FEV1 >80% of predicted of quitting—even with severe disease. Combine pharma-
in a symptomatic patient cotherapy with appropriate support to aid quit attempts
• Moderate COPD is defined as FEV1 <80% of predicted (NICE, 2006; van Schayck, Pinnock, Ostrem, Litt, &
• Severe COPD is defined as FEV1 <50% of predicted IPCRG, 2008).
• Very severe COPD is defined as FEV1 <30% of • Encourage exercise. Exercise is a key component of all
predicted pulmonary rehabilitation programmes which have been
• Use the MRC dyspnoea scale to grade the degree of breath- shown to improve exercise capacity and the quality of
lessness (Table 8.4) (Fletcher et al., 1959). life. Explain that breathlessness is uncomfortable but not
• Patient-reported outcomes. Short validated questionnaires dangerous.
for assessing the symptomatic burden of COPD and the • Stress the importance of good nutrition in all patients.
impact on quality of life include the COPD Assessment Refer patients with an abnormal BMI for dietetic advice.
Test (CAT; www.catestonline.org) (Jones et al., 2009) and Nutritional supplements may be recommended for under-
the Clinical COPD Questionnaire (CCQ; www/ccq/nl/) weight patients (Ferreira, Brooks, White, & Goldstein,
(van der Molen et al., 2003). 2012).
• Advise vaccination. Influenza vaccination reduces the
number of patients who experience an exacerbation (Poole,
TABLE Chacko, Wood-Baker, & Cates, 2006). Reactions to the
8.4 MRC Dyspnoea Scale (Fletcher et al., 1959) vaccine are mild and transient. COPD patients are at
Degree of Breathlessness Related
increased risk of pneumonia and pneumococcal vaccina-
Grade to Activities
tion is recommended.
• Provide information. The British Lung Foundation pub-
1 Not troubled by breathlessness except on lishes patient information about COPD (www.blf.org.uk).
strenuous exercise They also run Breathe Easy clubs in many areas.
2 Short of breath when hurrying or walking
up a slight hill
3 Walks slower than contemporaries on level Drug Management
ground because of breathlessness or has
to stop for breath when walking at own Bronchodilators
pace • These are the cornerstone of treatment to reduce symp-
4 Stops for breath after walking about 100 m toms. They reduce breathlessness, improve quality of life,
or after a few minutes on ground level and reduce need for oral steroids, but have a only small
5 Too breathless to leave the house, or effect on lung function.
breathless when dressing or undressing • Start with a short-acting β-2 agonist or antimuscarinic.
Both are effective in treating COPD, and there may be
a small additive effect (Appleton, Jones, et al., 2006). Severe and Very Severe Chronic Obstructive
They should be prescribed on an as required basis. Pulmonary Disease
• Introduce a new drug as a therapeutic trial, accepting
improved lung function or subjective improvement, such • Patients with COPD become progressively more disabled.
as reduced breathlessness, improved exercise capacity, or Regular monitoring of symptoms (e.g., with the MRC
activities of daily living as end points. dyspnoea score) and lung function should prompt increas-
• Add a long-acting bronchodilator if still symptomatic. Long- ingly aggressive therapy.
acting β-2 agonists or long-acting antimuscarinics can • Offer referral to pulmonary rehabilitation. All patients
produce a small increase in lung function and result in with functional disability (e.g., MRC dyspnoea score 3
a reduction in breathlessness and symptom scores, and or more) should be encouraged to attend pulmonary
improved quality of life (Appleton, Poole, et al., 2006; rehabilitation. Rehabilitation, which may be hospital or
Barr, Bourbeau, & Camargo, 2005; Chong, Karner, & community based, provides a multidisciplinary, holistic
Poole, 2012). Consider using a long-acting bronchodi- approach to the care of patients with COPD and has
lator in patients who have two or more exacerbations been shown to increase exercise tolerance, relieve breath-
a year. Both long-acting β-2 agonists and long-acting lessness, and improve quality of life (McCarthy, Casey,
antimuscarinics have been shown to reduce exacerbation Devane, Murphy, Murphy, & Lacasse, 2015).
rates (NNT for 1 year to prevent an exacerbation: with • Consider the need for a high-dose bronchodilator for
a long-acting β-2 agonist = 24; with tiotropium = 14) symptomatic relief. If this is not possible by multiple
(Appleton, Poole, et al., 2006; Barr et al., 2005; Chong doses through a spacer device, consider referral for assess-
et al., 2012). ment for a home nebulizer.
• Check inhaler technique before prescribing an inhaler, • Consider referral for assessment for long-term oxygen
choosing a device the patient is able to use (Brocklebank therapy. Patients with oxygen saturation at rest below
et al., 2001). 92%, and/or with polycythaemia or peripheral oedema,
• Consider using a theophylline if control is still poor or in should be referred for assessment for oxygen therapy. The
patients unable to use inhaled therapy. They have a modest criteria depend on lung function, blood gas estimations,
effect on lung function and a variable effect on symptoms and the presence of complications. In patients who are
and exercise capacity. Side effects are frequent, limiting chronically hypoxic, oxygen therapy for at least 15 hours
their value (Ram et al., 2002). a day has been shown to improve 5-year survival (Cran-
ston, Crockett, Moss, & Alpers, 2005). The evidence to
Inhaled Steroids support the use of short bursts of oxygen to relieve exercise-
• Consider whether inhaled steroids are indicated: induced breathlessness is less clear (Nonoyama, Brooks,
• Inhaled steroids do not consistently reduce the under- Lacasse, Guyatt, & Goldstein, 2007). Advice on air travel
lying rate of decline in lung function (Yang, Clarke, with oxygen is available from the European Lung Foun-
Sim, & Fong, 2012). dation (http://www.europeanlung.org/en/).
• Inhaled steroids have been shown to reduce the exac- • Look for depression. Depression is common in COPD.
erbation rate by 25% in patients with severe or very Psychological treatments (e.g. cognitive behavioural
severe COPD (FEV1 <50% predicted) and are therefore therapy) and/or lifestyle interventions that include an
of most benefit in patients who have two or more exercise component significantly improve symptoms (Cov-
exacerbations a year (Yang et al., 2012). entry et al., 2013). Some patients may require treatment
• Prescribe inhaled steroids in patients who have both with antidepressants.
asthma and COPD (GINA & GOLD, 2017). • Advise disabled patients that they may qualify for benefits.
• Bruising occurs more frequently in patients on inhaled Advice may be obtained from the Department of Work
steroids. There is a potential risk of osteoporosis in and Pensions.
patients using high-dose inhaled steroids. • Offer referral for practical help as disability increases.
• Inhaled steroids increase the risk of pneumonia with People with severe COPD are often silent about their
a 3-year number needed to harm (NNH) of 17 disease and do not ask for help (Habraken, 2008; Gia-
(Nannini, Lasserson, & Poole P, 2012). comini, DeJean, Simeonov, & Smith, 2012; Pinnock et al.,
2011). Provision of appliances such as walking aids, stair
Combination Therapy lifts, and bath aids may be appropriate. Support with
• Prescribe inhaled steroids in combination with long-acting domestic care may be needed. A wheelchair and a disabled
bronchodilators for patients in whom both drugs are parking permit may prevent the COPD patient becoming
indicated as these simplify the treatment regime and are housebound, and daycare may provide a break for both
generally cost effective. the patient and the carer.
• Even though accurate prognosis is difficult, consider the
Mucolytic Therapy need for holistic assessment and supportive care. Repeated
• Consider mucolytic therapy in patients with a chronic hospital admissions, comorbidity, chronic hypoxia,
productive cough. Mucolytic therapy can improve symptoms low BMI, and being housebound are all indicators of
and reduce exacerbations (Poole, Chong, & Cates, 2015). poor prognosis, though the slow deterioration (over years)
ALGrawany
may make it very difficult to identify the moment for inclu- 3. Peripheral oedema, right heart failure
sion on a palliative care register (Giacomini et al., 2012; 4. Low (<92%) oxygen saturation, or a reduction from
Pinnock et al., 2011). COPD patients, however, need the the patient’s normal levels
same attention to symptom control (especially relief of • Consider:
breathlessness) and support as patients dying of cancer. 1. the patient’s social circumstances;
• Consider therapeutic options for palliating symptoms: 2. the possibility of other respiratory pathology (e.g.,
• Morphine (initial dose: morphine 5 mg 4 hourly) carcinoma or TB);
relieves intractable breathlessness without increasing 3. the possibility of nonrespiratory comorbidity. In one
hypoxia (Jennings, Davies, Higgins, & Broadley, 2001). study, 40% of patients with an exacerbation of COPD
• Cool air (e.g., from a fan) sometimes reduces the sen- were found to have other conditions (e.g., hyperten-
sation of breathlessness. sion, CHD, diabetes); 14% had more than one condi-
• Benzodiazepines may relieve anxiety (though first tion (O’Brien et al., 2000).
remember that high doses of β-2 agonists can aggravate
anxiety). Initial Treatment
• Oxygen may reduce confusion due to hypoxia. • Bronchodilators to relieve breathlessness:
• Chlorpromazine or haloperidol may ease confusion 1. Increase or add a β-2 agonist and/or anticholinergic
and restlessness. drugs (McCrory & Brown, 2003).
• Offer the opportunity to discuss their prognosis and con- 2. Check inhaler technique (MDI + spacer may be easiest
sider their preferences in the event of future exacerbations. for the breathless patient).
3. Consider nebulized therapy for the severely breathless.
Indications for Referral for Specialist Advice • Antibiotics are only indicated if there is increased puru-
lence of sputum (Anthonisen et al., 1987; Ram et al.,
• For an objective diagnosis if the diagnosis is not clear, or 2006). Recommended antibiotic is amoxicillin, tetracy-
if spirometry is not available in primary care cline, or erythromycin, but prescribers should take into
• Aged under 40 or rapid deterioration in symptoms of account any guidance issued by local microbiologists.
lung function • Oral steroids increase the rate of lung function improve-
• Frequent infective exacerbations ment over the first 72 hours of an exacerbation (Niewoehner
• Onset of cor pulmonale et al., 1999; Walters, Tan, White, Gibson, Wood‐Baker,
• Assessment for long-term oxygen therapy or regular nebu- & Walters, 2014). In general practice, patients with mod-
lizer use erate or severe COPD (i.e., baseline FEV1 <50% predicted)
• Assessment for pulmonary rehabilitation who have a significant increase in breathlessness should
• The possibility of industrial disease (e.g., pneumoconiosis) be given prednisolone in a dose of 30 mg daily for 7 to
or an occupational cause for the COPD 14 days for severe exacerbations unless contraindicated.
• Diuretics may be given for peripheral oedema due to cor
Acute Exacerbations of COPD pulmonale.
Although commonly due to infection, worsening of previ- Arrange Further Care

ously stable COPD may be due to the development of other • Consider admission if there is:
pathology, including pneumonia, lung cancer, left ventricular 1. confusion, or deteriorating general condition; or
failure, pulmonary embolism, and pneumothorax. 2. cyanosis, SaO2 below 90%, or the patient is already
on long-term oxygen therapy; or
Assessment 3. severe breathlessness and poor response to treatment;
• In the history ask about: or
1. onset and duration of current exacerbation; 4. worsening peripheral oedema; or
2. increased volume and purulence of sputum; 5. social circumstances are poor or there is an inability
3. increased wheeze and shortness of breath; to cope at home.
4. increasing confusion or decreasing conscious level; • Arrange follow-up bearing in mind the following points:
5. condition when stable, especially the need for long- 1. Failure to make a recovery should prompt a CXR.
term oxygen; 2. Smoking cessation should be encouraged and lifestyle
6. severity of previous exacerbations and previous advice given.
admissions. 3. Regular treatment should be optimized.
• When examining the patient look for signs of a severe • Hospital at home. About one in four patients presenting
exacerbation: to hospital emergency departments with acute exacerba-
1. Cyanosis, confusion, exhaustion, decreased level of tions of COPD can be safely and successfully treated at
activity home with support from respiratory nurses (Jeppesen
2. Severity of breathlessness, use of accessory muscles, et al., 2012). Both patients and carers preferred this hos-
respiratory rate pital at home scheme to inpatient care.
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9
Gastroenterologic Problems
JOHN PAUL SEENAN, HEATHER LAFFERTY
Dyspepsia, Helicobacter Pylori, and Gastrooesophageal Reflux Lymphoma and Cancer Risk
Disease Noncoeliac Gluten Sensitivity
Alarm Symptoms Iron Deficiency Anemia
Initial Management in Primary Care Causes of Iron Deficiency Anemia
Investigation and Referral History
H. pylori Examination
Testing for H. pylori
Initial Investigation
Test and Treat
Eradicating H. pylori Further Investigation
Confirmation of Successful Eradication Treatment
Other Considerations and Controversies Weight Loss
Functional Dyspepsis History and Examination
Peptic Ulcer Disease Investigation
Gastrooesophageal Reflux Disease Endoscopy
Dysphagia Imaging
Biliary/Pancreatic Disease Further Management
Irritable Bowel Syndrome Treatment
Diagnosis and Referral Jaundice and Abnormal Liver Function Tests
Investigation Special Situations
IBS Subtypes History
Treatment History of the Presenting Complaint
Drug History
Inflammatory Bowel Disease Social History
Ulcerative Colitis Past Medical History
Crohn’s Disease Examination
Inflammatory Bowel Disease Pharmacologic Treatment Initial Investigations
Surgery
Specific Liver Diseases
Indications for Admission
Viral Hepatitis
Cancer Surveillance
Hepatitis B
Fertility and Pregnancy
Hepatitis C
Breastfeeding
Hepatitis A
Vaccinations
Hepatitis E
Chronic Diarrhoea Autoimmune Hepatitis (AIH)
Further Investigation Primary Sclerosing Cholangitis (PSC)
Coeliac Disease Primary Biliary Cholangitis (PBC)
Overlap Syndromes
Initial Testing and Referral
Nonalcoholic Fatty Liver Disease
Further Management Alcoholic Hepatitis
Ongoing Management Hereditary Haemochromatosis
Refractory or Recurrent Symptoms Wilson Disease
Refractory Coeliac Disease
109
Chronic Liver Disease Varices

Staging of Advanced Liver Disease TIPSS
Management of Patients With Cirrhosis Hepatocellular Carcinoma
Ascites Liver Transplant
Hepatic Encephalopathy
was inversely correlated with the finding of cancer in these

Dyspepsia, Helicobacter Pylori, and older patients (Kapoor, Bassi, Sturgess, & Bodgeret, 2005).
Gastrooesophageal Reflux Disease
Initial Management in Primary Care
GUIDELINE
• If a previous diagnosis has been made at endoscopy with no
Gastro-oesophageal reflux disease and dyspepsia in adults: new alarm symptoms, then manage as previously advised.
Investigation and management. NICE clinical guideline 184. • Encourage lifestyle modification with avoidance of
Retrieved from www.nice.org.uk/guidance/cg184. food triggers, alcohol reduction, smoking cessation, and
NICE Clinical Guideline. (2015). Suspected cancer: weight loss.
recognition and referral. Retrieved from www.nice.org.uk/
guidance/ng12.
• Check that the patient is not taking a drug that can cause
dyspepsia (e.g., aspirin or nonsteroidal antiinflammatory
drug [NSAID], calcium antagonist, nitrate, theophylline,
bisphosphonate, or steroid).
• Dyspepsia is not a diagnosis but rather describes a range • Offer to test and treat H. pylori infection.
of symptoms which should prompt doctors to consider • Consider a 4-week empirical trial of a proton pump
underlying disease of the upper gastrointestinal (GI) tract. inhibitor (PPI) such as omeprazole 20 mg once daily in
These symptoms may include upper abdominal/epigastric Helicobacter-negative patients. If symptoms recur after
pain, nausea, vomiting, belching, bloating, and borborygmi initial treatment, step down PPI to the lowest dose required
(NICE, 2014b). to control symptoms. Encourage self-management and
• Upper gastrointestinal symptoms are extremely common discuss using treatment on an as required basis.
in the general population and the vast majority of patients • Offer histamine-2 (H2) antagonists (e.g., ranitidine 150 mg
have benign disease (Maconi, Manes, & Porro, 2008). twice daily) in patients with inadequate response to a PPI.
• In Western coutries, one-third of the population has
symptoms of dyspepsia but of these only 25% seek medical Investigation and Referral
attention; 10% will be investigated by endoscopy with
only 2% of patients undergoing endoscopy being diag- • Patients presenting with dyspepsia and significant acute
nosed with gastric cancer (NICE, 2004). gastrointestinal bleeding require immediate (same day)
• In 90% of patients (including 75% of patients with Heli- specialist referral for endoscopy.
cobacter pylori infection) the final diagnosis is functional • Consider referral to exclude upper GI cancer in the fol-
dyspepsia and most disease is managed symptomatically lowing circumstances:
(Briggs et al., 1996). • Consider a suspected cancer pathway referral (for an
appointment within 2 weeks) for people with an upper
Alarm Symptoms abdominal mass consistent with gastric cancer.
• Offer urgent direct access upper GI endoscopy (to be
• Although benign disease predominates, the possibility of performed within 2 weeks) to assess for oesophago-
cancer is often a significant concern for patients, families, gastric cancer in people with dysphagia or aged 55
and treating clinicians. and older with weight loss and upper abdominal pain,
• Alarm features have been shown to be sensitive, but not reflux, or dyspepsia.
specific, in screening for upper GI carcinoma. In a UK • Consider nonurgent direct access upper GI endoscopy
study of 1852 patients referred to a rapid access upper to assess for oesophagogastric cancer in people with
GI cancer service, only dysphagia, weight loss, and age haematemesis.
over 55 were independent significant predictors of cancer. • Consider nonurgent direct access upper GI endoscopy
Furthermore, age over 55 was only a significant predictor to assess for oesophagogastric cancer in people with:
if at least one other alarm feature was present (anemia, • treatment-resistant dyspepsia, or
anorexia, vomiting, dysphagia, weight loss, or high-risk • upper abdominal pain with low haemoglobin
features). Simple dyspepsia, even if new or continuous, levels, or
CHAPTER 9 Gastroenterologic Problems 111
• raised platelet count with nausea, vomiting, weight a full dose PPI, with amoxicillin 1 g and either clarithro-
loss, reflux, dyspepsia, or upper abdominal pain, or mycin 500 mg, or (in the case of penicillin hypersensitiv-
• nausea/vomiting with weight loss, reflux, dyspepsia, ity) metronidazole 400 mg and clarithromycin 250 mg,
or upper abdominal pain. all given twice daily.
• Eradication is effective in 80% to 85% of patients on
H. pylori triple therapy using either antibiotic combination.
• Prescribing amoxicillin and tetracycline rarely results in
• Gram-negative bacterium identified in 1982 by Australian H. pylori resistance, whereas resistance occurs after limited
scientists Barry Marshall and Robin Warren (Marshall & exposure to clarithromycin and quinolones. Exposure to
Warren, 1984) and recognized to affect at least 50% of metronidazole also results in H. pylori resistance, but this
the population worldwide. has less of an impact on the effectiveness of treatment
• Infection usually occurs in the first few years of life and regimens.
tends to persist indefinitely unless treated. • First-line therapy. Choose the treatment regimen with the
• H. pylori infection is recognized as a risk factor for peptic lowest acquisition cost and consider previous exposure
ulcer disease (reported to develop in 1% to 10% of infected to clarithromycin or metronidazole (it is not necessary
patients) and gastric cancer (occurring in 0.1% to 3%) to consider previous metronidazole exposure in patients
(McColl, 2010). with penicillin allergy).
• Second-line therapy. Should use different antibiotics to
Testing for H. pylori first-line therapy.
• H. pylori serology testing is cheap, convenient, and widely • Seek advice from a gastroenterologist if eradication of
available. However, a meta-analysis of commercially avail- H. pylori is not successful with second line therapy.
able antibody assays demonstrates a sensitivity and speci- • Before prescribing further therapy with quadruple or
ficity of only 85% and 79%, respectively (Loy, Irwig, sequential therapy, it will be important to confirm that the
Katelaris, & Talley, 1996). A further limitation is that infection is still present and to consider whether additional
this test has little value in confirming eradication of the antimicrobial treatment is appropriate (e.g., patients with
infection because the antibodies persist for many months, confirmed peptic ulcer disease). This is likely to require
if not longer, after eradication. endoscopy with rapid-urease testing or gastric histology
• The urea breath test (UBT) involves drinking 13C- or used to confirm persistent infection. It may also allow tissue
14
C-labeled urea, which is converted to labelled carbon to be sent for culture and sensitivity to guide antibiotic
dioxide by the urease in H. pylori. The infection can also prescribing where appropriate (McColl, 2010).
be detected by identifying H. pylori–specific antigens in • However, patients with no endoscopic evidence of sig-
a stool sample. These tests have much better sensitivity nificant Helicobacter-related pathology are likely to have
(95%) and specificity (95%) than serologic testing (Gisbert functional dyspepsia and given the marginal benefit of
& Pajares, 2004; Vaira & Vakil, 2001). Helicobacter eradication in this group (NNT =15) symp-
For both the breath test and the faecal antigen test, the tomatic management with long-term acid suppression
patient should stop taking PPIs 2 weeks before testing, should may be more appropriate (McColl, 2010).
stop taking H2-receptor antagonists for 24 hours before
testing, and should avoid taking antimicrobial agents for 4 Confirmation of Successful Eradication
weeks before testing, since these medications may suppress • Not routinely required but should be performed in the
the infection and reduce the sensitivity of testing. following circumstances:
1. Ongoing or recurrent symptoms following eradication
Test and Treat therapy
• The rationale for test and treat is that Helicobacter eradica- 2. Partial resection for gastric cancer
tion can effectively treat undiagnosed peptic ulcer disease 3. Mucosa-associated lymphoid tissue (MALT) lymphoma
in uninvestigated dyspepsia without alarm symptoms. A 4. Complicated peptic ulcer disease (e.g., following per-
large randomized control study found this to be cost foration or ulcer bleed)
effective due to the reduction in unnecessary endoscopy • Wait 4 weeks after eradication before repeating UBT or
despite having a number needed to treat (NNT) of seven faecal antigen.
patients (Ford et al., 2005).
• H. pylori eradication is likely to be helpful in patients Other Considerations and Controversies
with duodenal ulcer (DU), gastric ulcer (GU), gastritis, • H. pylori and reflux. Chronic Helicobacter infection may
duodenitis, and (marginally) in functional dyspepsia (NNT result in atrophic gastritis and reduced acid secretion,
= 15) (McColl, 2010). which is protective against significant reflux disease. Most
epidemiologic studies have demonstrated a negative asso-
Eradicating H. pylori ciation between H. pylori infection and gastrooesophageal
• NICE recommends a 1-week triple therapy regimen as first reflux disease (GORD) or its complications with the
line eradication therapy. The optimum regimen consists of prevalence of H. pylori infection being lower in reflux
patients than in controls. Numerous studies have now cardiac arrhythmia and extrapyramidal side effects such
reported a strong negative association between H. pylori that they are largely restricted to use for short-term symp-
infection and the risk of adenocarcinoma of the oesophagus tomatic relief (Moayyedi et al., 2003b).
or gastrooesophageal junction.
• Population screening. Despite being identified as a car- Peptic Ulcer Disease
cinogen, there is no consensus that population screening
and eradication of asymptomatic individuals is benefi- • The vast majority of peptic ulcer disease is caused by H.
cial. Given the negative association of Helicobacter with pylori infection or use of NSAIDs.
oesophageal adenocarcinoma, it has been postulated that • H. pylori–positive patients should receive eradication
eradication might increase the risk of oesophageal adeno- therapy.
carcinoma due to increased acid secretion and reflux, thus • Offer H. pylori–negative patients who are not taking
potentially trading one cancer risk for another (Islami & NSAIDs a full dose PPI (e.g., omeprazole 20 mg daily)
Kamangar, 2008). or H2-receptor agonist for 4 to 8 weeks.
• Other people to test and treat. European guidelines rec- • Patients taking NSAIDs should stop where possible
ommend eradicating H. pylori infection in first-degree and be offered full dose PPI (e.g., omeprazole 20 mg
relatives of patients with gastric cancer and in patients daily) or H2-receptor agonist for 8 weeks.
with atrophic gastritis, unexplained iron deficiency • Patients continuing NSAIDs should be coprescribed
anemia, or chronic idiopathic thrombocytopenic purpura a PPI, counselled regarding the risk with the need for
(Malfertheiner et al., 2007). treatment reviewed regularly, and a switch to COX-2
• Reinfection. This rarely occurs and many reported cases selective NSAID considered.
may simply represent recrudescence (i.e., failure of • Patients with gastric ulcer should undergo repeat endos-
initial eradication) (van der, van der Hulst, Dankert, & copy after 6 to 8 weeks +/− biopsy to ensure healing
Tytgat, 1997). and exclude malignancy.
• Consider repeat testing to confirm successful eradica-
Functional Dyspepsis tion in H. pylori–positive patients with complicated
PUD (e.g., perforation or bleeding) and depending
• Functional dyspepsia (FD) is defined as the presence on size/extent of ulceration.
of symptoms thought to originate in the gastroduode-
nal region, in the absence of any organic, systemic, or Gastrooesophageal Reflux Disease
metabolic disease that is likely to explain the symptoms
(Tack et al., 2006). • Is the most common chronic disease in Western countries
• Significant overlap may occur with the symptoms of irri- with 25% experiencing monthly symptoms and 5% having
table bowel syndrome. FD should be diagnosed based on reflux daily (Moayyedi & Talley, 2006).
the ROME III diagnostic criteria* and must include: • Typically presents with heartburn and regurgitation.
1. One or more of the following: • Patients should be advised regarding lifestyle modification
a. Bothersome postprandial fullness with the avoidance of food triggers (e.g., chocolate, red
b. Early satiation wine, caffeine), smoking cessation, and weight loss while
c. Epigastric pain acid suppression is the mainstay of pharmacologic
d. Epigastric burning treatment.
AND • In general, the differences between available PPIs are small.
2. No evidence of structural disease (including at upper However, patients with an insufficient therapeutic response
endoscopy) that is likely to explain the symptoms to the standard dose of one PPI may benefit from one
• Treatment of FD begins with reassurance and explanation of the other PPIs, an increased dose of the same PPI, or
regarding the diagnosis. The evidence base in FD is limited a twice-a-day PPI regimen (Boeckxstaens, El Serag, Smout,
by high placebo response rates (20%–60%) and many & Kahrilas, 2014).
small, heterogenous studies, but acid suppression remains • Consider referral for laparoscopic fundoplication in patients
the first line management. Meta-analyses have shown PPIs who have confirmed reflux unresponsive to acid suppres-
and H2 antagonists to be effective with a NNT of 7 and sion or in whom long-term acid suppression is not toler-
8, respectively (Moayyedi et al. 2003b). However, some ated or desired.
of the benefit with these drugs may be from treating • Complications of reflux include oeosphagitis, peptic stric-
undiagnosed reflux. Helicobacter eradication provides ture formation, columnar metaplasia (Barrett oesophagus),
only marginal benefit (NNT = 17) (Moayyedi et al., and oesophageal adenocarcinoma.
2003a). Prokinetics (e.g., domperidone and metoclo- • Screening for Barrett oesophagus with endoscopy is not
pramide) appear efficacious but are limited by risk of feasible or justified for an unselected population with
reflux symptoms but can be considered in patients with
*Criteria fulfilled for the last 3 months with symptom onset at least 6 chronic GORD symptoms and multiple risk factors (at
months before diagnosis. least three of age ≥50 years, white race, male sex, obesity).
The threshold should be lowered if there is a family history ultrasound scan if CT is not available, to assess for pan-
of Barrett or oesophageal adenocarcinoma in one or more creatic cancer in people aged 60 and over with weight
first-degree relatives (Fitzgerald et al., 2014). loss and diarrhoea, back pain, abdominal pain, nausea,
• Due to the increased risk of oesophageal adenocarci- vomiting, constipation, or new-onset diabetes.
noma in patients with Barrett oesophagus, surveillance • If endoscopy is negative and symptoms do not respond
endoscopy is recommended (Fitzgerald et al., 2014). to H. pylori eradication or acid suppression, consider
High-grade and persistent low-grade dysplasia may now alternative investigations such as abdominal ultrasound,
be treated endoscopically with radio-frequency ablation amylase, and liver function tests (LFTs) which may point
(RFA), often avoiding the need for oesophagectomy to biliary or pancreatic disease.
(NICE, 2014a). • Cholecystitis typically presents with persistent pain (lasting
>6 hours) and fever. In contrast, the pain from biliary
Dysphagia colic is intermittent and lasts from a few minutes to 1
hour before subsiding after up to 6 hours. In both cases
• As discussed, urgent direct-access endoscopy should be pain classically occurs postprandially.
considered in all patients presenting with dysphagia. • Ultrasound is effective in detecting cholecystitis and/or
However, many will subsequently have a normal examina- gallstones (sensitivity >95%) (Shea et al., 1994) but inferior
tion or be found to have benign disease, so an approach to magnetic resonance cholangiopancreatography (MRCP)
to further investigation is required (Abdel Jalil, Katzka, for identifying biliary obstruction (e.g., common bile duct
& Castell, 2015). stones) (Singh, Mann, Thukral, & Singh, 2014).
• Patients with peptic oesophageal strictures may require • If biliary colic/cholecystitis is suspected or confirmed,
balloon dilatation (perforation risk of approximately 1%) then surgical referral should be considered. Endoscopic
and should be maintained on long-term PPI to prevent retrograde cholangiopancreatography (ERCP) +/− sphinc-
recurrence. terotomy is required for bile duct stones. Cholecystectomy
• Eosinophilic oesophagitis typically presents as food bolus should subsequently be performed and is also indicated
obstruction in young men with a history of atopy. Character- for chronic cholecystitis.
istic endoscopic appearances have been described but it should • Functional disorders can also affect the gallbladder (e.g.,
be considered in patients presenting with dysphagia even biliary dyskinesia) and biliary tree (sphincter of Oddi
with a normal endoscopy. Proximal and distal oesophageal dysfunction). Pharmacologic therapy seems to be inef-
biopsies (showing >20 eosinophils per high-powered field) fective, and ERCP with sphincterotomy may be required
may help to confirm or refute the diagnosis. Many patients (Sgouros & Pereira, 2006).
respond to acid suppression, but in others elimination diets
and/or topical steroids may be required. Irritable Bowel Syndrome
• Chest x-ray (CXR), should be considered in smokers to
exclude extrinsic compression from a bronchial neoplasm GUIDELINES
+/− mediastinal mass.
• Barium swallow may identify the presence of a pharyngeal
Irritable bowel syndrome in adults: Diagnosis and
pouch or oesophageal dysmotility in patients with per- management. NICE clinical guideline 61 (updated 2017).
sistent dysphagia. Retrieved from www.nice.org.uk/guidance/cg61.
• Oesophageal manometry (+/− pH monitoring to exclude Spiller, R., Aziz, Q., Creed, F., Emmanuel, A., Houghton,
significant reflux) helps to diagnose motor disorders L., Hungin, P., et al. (2007). Guidelines on the irritable bowel
syndrome: Mechanisms and practical management. Gut, 56,
of the oesophagus (e.g., diffuse oesophageal spasm or
1770–1798
achalasia).
• Oesophageal spasm often presents with chest pain, while
achalasia is characterized by weight loss and frequent
regurgitation. Heller myotomy, pneumohydralic balloon • Irritable bowel syndrome (IBS) is a common, chronic
dilatation, and Botox injection to the lower oesophageal gastrointestinal condition of unknown cause (Spiller
sphincter are therapeutic options that may be considered et al., 2007).
in confirmed achalasia. • Of the UK population, 10% to 20% are estimated to be
• NB: Patients with high dysphagia (i.e., above the level of affected with the condition; it is twice as common in
the suprasternal notch) should be considered for otorhi- women, and the peak incidence is between ages 20 and
nolaryngology referral and nasendoscopy particularly if 30 years (NICE, 2008).
current or ex-smokers. • It is characterized by abdominal pain or discomfort associ-
ated with defaecation, abdominal bloating, and bowel
Biliary/Pancreatic Disease dysfunction (constipation, diarrhoea, or both).
• IBS can also affect sleep and cause stress, anxiety, and
• Consider an urgent direct access computerized tomography lethargy. It is associated with reduced work productivity
(CT) scan (to be performed within 2 weeks), or an urgent and quality of life.
Diagnosis and Referral • Patient self-management should be encouraged. This should

include information on general lifestyle, physical activity,
• Consider IBS in patients presenting with 6 months or and diet (including limiting intake of insoluble fibre)
more of abdominal pain/discomfort, bloating, or change (NICE, 2008).
in bowel habit (think ABC). • More detailed dietary advice is provided in the British
• It is defined by the ROME III diagnostic criteria as recurrent Dietetic Association’s Food Facts for IBS Factsheet, available
abdominal pain or discomfort at least 3 days/month in the at www.bda.uk.com/foodfacts/IBSfoodfacts.pdf. Referral
last 3 months associated with two or more of the following: to a specialist dietitian for more detailed advice and/or
1. Improvement with defecation supervision of a low FODMAP diet may also be helpful.
2. Onset associated with a change in frequency of stool • The evidence for probiotics is weak, but they may help
3. Onset associated with a change in form (appearance) bloating and flatulence in some patients (Didari, Mozaf-
of stool fari, Nikfar, & Abdollahi, 2015; DuPont, 2014; Zhang
• Symptom onset should be at least 6 months prior to et al., 2016).
diagnosis. Refer to secondary care patients with any of • Consider prescribing antispasmodic agents (e.g., pepper-
the following red flags: mint oil) for patients with IBS (Ruepert et al., 2011).
1. Unintentional and unexplained weight loss • Laxatives should be considered for constipated patients
2. Rectal bleeding but lactulose avoided as it may exacerbate bloating and
3. Family history of bowel or ovarian cancer discomfort (NICE, 2008).
4. Change in bowel habit to looser and/or more frequent • Loperamide should be the first choice antimotility agent
stools for 6 or more weeks in patients over 60 years in IBS-D.
5. anaemia • Consider tricyclic antidepressants (TCAs) as second line
6. Abdominal/rectal masses treatment for patients with IBS if laxatives, loperamide,
7. Raised inflammatory markers or antispasmodics have not helped. Start at a low dose
(e.g., amitriptyline 10 mg nocte) to minimize sedation.
Investigation Increase dose if necessary and tolerated (but not usually
>30 mg). TCAs are effective (NNT = 3) and primarily
• Full blood count (FBC), erythrocyte sedimentation rate reduce pain but also have an antimotility effect with a
(ESR), C-reactive protein (CRP), and antibody testing suggestion that patients with IBS-D obtain the greatest
for coeliac disease (tissue transglutaminase or endomysial benefit (Ruepert et al., 2011).
antibodies) should be performed in all patients with sus- • Consider selective serotonin reuptake inhibitors (e.g.,
pected IBS (NICE, 2008). fluoxetine 20 mg daily) only if TCAs are ineffective
• Faecal calprotectin (FC) has been shown to be a useful (Ruepert et al., 2011).
screening test to differentiate between inflammatory bowel • Linaclotide (290 µg once daily) is a guanylate cylcase C
disease (IBD) and IBS. It is a calcium and zinc-binding agonist (GCCA) which elevates cyclic guanosine mono-
protein within the cytosol of neutrophils and a sensitive but phosphate (cGMP), accelerating transit in the GI tract
nonspecific marker of inflammation within the GI tract. through increased fluid transit and reducing visceral hyper-
• NICE (2013a) recommends using FC to support the sensitivity. It improves pain, bloating, and constipation in
diagnosis of IBS where cancer is not suspected. IBS-C and should be considered if optimal or maximum
• Some studies suggest the manufacturer’s FC cutoff values tolerated doses of laxatives from different classes have
are too low for utilization in clinical practice with levels less not helped in patients with constipation for 12 or more
than 200 µg/g rarely associated with any organic disease months (Chey et al., 2012; Quigley et al., 2013).
(Seenan, Thomson, Rankin, Smith, & Gaya, 2014). • Prucalopride (1–2 mg daily) is a highly selective serotonin
5-HT4 agonist that stimulates gut motility. It has been
IBS Subtypes shown to be effective in patients (85% women) suffering
from chronic constipation with an expected onset of effect
• Irritable bowel syndrome can be classified based on the within 4 weeks (Camilleri, Kerstens, Rykx, & Vande-
predominant symptoms into: plassche, 2008; Camilleri et al., 2010, 2016).
• constipation-predominant IBS (IBS-C);
• diarrhoea-predominant IBS (IBS-D);
• mixed-type IBS (IBS-M). Inflammatory Bowel Disease
• This allows treatment to be tailored to the most trouble- Ulcerative Colitis
some symptom (Longstreth et al., 2006).
• Ulcerative colitis (UC) is the most common form of
Treatment inflammatory bowel disease with an incidence of 10 per
100,000 and prevalence of 240 per 100,000. This means
• A positive diagnosis of IBS should be made with emphasis there are an estimated 146,000 patients with UC across
on reassurance and explanation. the United Kingdom.
GUIDELINES Oral Aminosalicylates

• Indications: Induction and maintenance of remission in
patients with mild to moderate or moderate to severe
Crohn’s disease: Management. NICE clinical guideline 152
(updated 2016). Retrieved from www.nice.org.uk/guidance/ (high dose) UC.
cg152. • Sulphasalazine and oral aminosalicylates are effective therapy
National Institute for Health and Care Excellence. (2013). for both inducing and maintaining remission in UC.
Ulcerative colitis: Management. NICE clinical guideline 166. • Oral aminosalicylates are preferred due to an improved
Retrieved from www.nice.org.uk/guidance/cg166.
side effect profile. Diarrhoea (3%), headache (2%),
nausea (2%), and rash (1%) are reported, but a systematic
review has shown adverse events are similar to placebo for
mesalazine.
• All aminosalicylates have been associated with nephro-
• Presentation peaks between the ages of 15 and 25 years, toxicity (including interstitial nephritis and nephrotic
with a smaller peak later in life from 55 to 65 years of age. syndrome). Patients receiving oral aminosalicylates should
• UC typically affects the colon continuously from the have renal function monitored at commencement and
rectum to a variable distance proximally. then annually (Mowat et al., 2011).
• It usually presents with bloody diarrhoea, urgency, and • Studies suggest that doses over 2 g are more effective at
abdominal pain. Weight loss and extraintestinal symptoms inducing remission in patients with moderate to severe
can also occur (NICE, 2013c). disease (Ford et al., 2011). Higher doses (4.8 g versus
2.4 g daily) are associated with increased rates of response
Crohn’s Disease and earlier mucosal healing (Hanauer et al., 2005, 2007;
Lichtenstein, Ramsey, & Rubin, 2011).
• Crohn’s disease affects an estimated 115,000 people in • Oral therapy can be combined with topical aminosalicy-
the United Kingdom. lates if required.
• One-third of cases present before the age of 21 years. • Except in patients with proctitis, treatment is contin-
• Unlike UC, it causes transmural inflammation and can ued indefinitely to reduce the risk of relapse (Feagan &
affect anywhere in the GI tract from mouth to anus. MacDonald, 2012) and mitigate colorectal cancer risk
• Historically 50% to 80% of patients require surgery due (van Staa, Card, Logan, & Leufkens, 2005). The thresh-
to complications such as perforation, obstruction due to old dose to reduce colorectal cancer risk appears to be
stricture formation, or fistulization. 1.2 g/day (Rubin, LoSavio, Yadron, Huo, & Hanauer,
• Smoking and genetic predisposition are potential aetio- 2006). Modified release preparations should be used to
logical factors (NICE, 2012). reduce the pill burden, minimize dosing, and improve
patient compliance.
Inflammatory Bowel Disease Pharmacologic • There is limited evidence to support the use of amino-
Treatment salicylates in active Crohn’s disease. However, they may
Topical Therapy have a role in reducing the risk of colorectal neoplasia in
• Indication. Induction and maintenance of remission in Crohn’s colitis (van Staa et al., 2005) and the prevention
patients with UC and Crohn’s colitis. of postoperative recurrence (Gordon, Naidoo, Thomas,
• Particularly effective in distal disease. Consider using sup- & Akobeng, 2011).
positories in proctitis and enemas in patients with distal • Typical doses. Mesalazine 2.4 to 4.8 g daily (active disease)
or left-sided colitis. Various enema preparations are avail- and 1.2 to 2.4 g (maintenance).
able including liquid, foam, and retention enemas. Choice • Recommended monitoring. Renal function annually.
of preparation depends on patient preference.
• Topical therapy can also provide additional benefit to Oral Steroids
oral therapy in patients with extensive colitis (inflamma- • Indication. Induction of remission in patients with both
tion that extends beyond splenic flexure). UC and Crohn’s disease.
• Studies suggest aminosalicylate preparations are more • Effective in inducing remission but maintenance therapy
effective than steroid suppositories or enemas (Marshall avoided due to side effects of long-term use (e.g., hyper-
& Irvine, 1997; Munkholm, Michetti, Probert, Elkjaer, tension, diabetes, osteoporosis, cataracts, and glaucoma).
& Marteau, 2010). • Patients receiving systemic steroids should be coprescribed
• Topical therapy may be used to treat acute exacerbations, calcium and vitamin D as bone protection.
as as required therapy in patients with limited disease • Budesonide can be used in both UC (Cortiment, Ferring,
(proctitis) or as regular maintenance therapy. Budesonide MMX, colonic release) and mild to moderate
• Typical doses. Mesalazine suppositories 0.75 to 1.5 g daily, ileocaecal Crohn’s disease (Entocort, Tillotts or Budenofalk,
mesalazine enemas 1 to 2 g daily, prednisolone rectal foam Dr Falk terminal ileal release). It may be preferred due
20 to 40 mg daily (for up to 4 weeks). to high first-pass metabolism, low systemic absorption,
• Monitoring. Nil required. and better side effect profile.
• Consider prescribing steroids in patient unresponsive or • Methotrexate (MTX) has been shown to be effective as both
intolerant to aminosalicylates. induction (Alfadhli, McDonald, & Feagan, 2003; McDonald,
• Typical doses: Wang, Tsoulis, MacDonald, & Feagan, 2014) and mainte-
a. Prednisolone 40 mg daily, reducing by 5 mg per week nance therapy for Crohn’s disease (Feagan et al., 2000).
over 8 weeks to stop • It is often used in patients who are intolerant to thiopurines.
b. Budesonide MMX (Cortiment) 9 mg for 8 weeks then • Coprescription of folic acid 5 mg (once a week, taken 3
stop days after MTX) limits GI side effects of nausea, vom-
c. Budesonide (Entocort, Budenofalk) 9 mg for 8 weeks iting, diarrhea, and stomatitis. Long-term concerns are
then 6 mg for 2 weeks and 3 mg for further 2 weeks hepatotoxicity, pneumonitis, and opportunistic infections.
• Monitoring. Consider risk of diabetes in symptomatic • MTX is teratogenic and should not be used in women
patients and screening for osteoporosis, hypertension, or men considering conception. It may persist in tissues
cataracts, and glaucoma with high cumulative doses. for long periods; therefore conception should be avoided
for 3 to 6 months after withdrawal of therapy (Mowat
Thiopurines et al., 2011).
• Indication: Maintenance of remission in moderate to severe • Monitoring. Please refer to local shared-care protocol.
UC and Crohn’s disease. Measurement of full blood count and liver function tests
• Thiopurines have demonstrated efficacy in maintaining are generally advisable before and within 4 weeks of start-
remission for both UC (Gisbert, Linares, McNicholl, ing therapy, then monthly.
Mate, & Gomollon, 2009) and Crohn’s disease (Chande,
Tsoulis, & MacDonald, 2013). They may also have a role Biologics
in preventing postoperative recurrence in Crohn’s disease • Indication. Induction and maintenance therapy in patients
(Gordon, Taylor, Akobeng, & Thomas, 2014). with moderate to severe UC and Crohn’s disease who are
• Check thiopurine methyltransferase (TPMT) activity prior unresponsive to, or intolerant of, or unsuitable for con-
to starting. One in 300 will have absent TPMT activity, ventional therapy.
putting them at risk of life-threatening myelosuppression • Infliximab, adalimumab (anti–tumor necrosis factor-alpha
(Mowat et al., 2011). [anti-TNFα]), and vedolizumab (antiadhesion) are now
• Other side effects (e.g., pancreatitis, hepatitis, myalgia, approved for use in both UC and Crohn’s disease. Goli-
nausea, vomiting) appear to be independent of TPMT mumab (anti-TNFα) is only approved for use in UC.
activity. Patients should also be advised regarding the • Prior to initiation patients are screened for tuberculosis
increased relative risk (approximately two- to threefold) (TB) based on history, CXR, and blood testing (interferon
of nonmelanoma skin cancer (NMSC) and lymphoma. gamma release assays [IGRAs]—e.g., T-SPOT or
Advice to reduce the risk of NMSC by avoiding excessive QuantiFERON-TB Gold). Human immunodeficiency
sun exposure should be offered (see www.bad.org.uk). virus (HIV) and hepatitis B and C serology should also
• Mercaptopurine may be used in patients with normal TPMT be performed.
activity who are intolerant of azathioprine without pan- • Anti-TNFs are avoided in patients with a history of con-
creatitis (Hindorf, Johansson, Eriksson, Kvifors, & Almer, firmed or suspected demyelination.
2009; Lees, Maan, Hansoti, Satsangi, & Arnott, 2008). • Monitoring: Please refer to local shared-care protocol.
• Thiopurine metabolite monitoring can identify noncompli-
ance and patients in whom coprescription of allopurinol to Surgery
low-dose azathioprine may be beneficial to improve efficacy Ulcerative Colitis
and prevent drug-induced hepatitis (Smith et al., 2012). • Indications:
• Given the potential side effects of long-term use, cessation 1. Severe colitis refractory to medical therapy—may be
should be considered after 4 years of remission with the emergency or semielective procedure
benefits and risks of continuing azathioprine discussed 2. Dysplasia/cancer
with individual patients. • Acutely patients will usually undergo subtotal colectomy.
• Typical doses: In some cases, an ileal pouch anal anastomosis (IPAA)
1. Azathioprine 2 to 2.5 mg/kg will be considered at a later stage (e.g., 6 months after
2. Mercaptopurine 1 to 1.5 mg/kg initial surgery).
• Monitoring. Please refer to local shared-care protocol but • In UC surgery can generally be considered curative and
typically requires Full Blood Count FBC Urea and Elec- removes both the risk of colorectal cancer and need for colo-
trolytes, U&Es, and Liver Function Tests LFTs weekly noscopic surveillance. Laparascopic surgery with enhanced
for 8 weeks then monthly for 3 months and 3 monthly recovery and early discharge is increasingly available.
thereafter. • However, there is a morbidity and mortality attached to
surgery. Furthermore, IPAA will not always be possible,
Methotrexate and some patients may require a permanent stoma. Pouch
• Indication. Induction and maintenance therapy in patients function may be variable and increased stool frequency/
with Crohn’s disease. volume is expected.
• After proctocolectomy with IPAA, the median stool fre- • Other indications for admission include:
quency is four to eight motions per day with a volume of • acute obstruction;
around 700 mL (compared to 200 mL in healthy subjects). • suspected intrabdominal or perianal sepsis;
• Pouchitis (nonspecific inflammation of the pouch) occurs • significant GI bleeding.
in up to 50% of patients after 10 years. Once confirmed
(endoscopically and histologically) antibiotics (metroni- Cancer Surveillance
dazole and/or ciprofloxacin) will often induce remission. • Patients with UC and Crohn’s disease are considered to
This can be maintained with the probiotic VSL#3 (Singh, have an equivalent increased risk of colorectal cancer
Stroud, Holubar, Sandborn, & Pardi, 2015). Other treat- (CRC) provided there is a similar extent and duration of
ments for refractory pouchitis include Infliximab (IFX), colonic inflammation.
steroids (e.g., budesonide), and removal of the pouch • Risk factors for CRC in patients with IBD include:
(van Assche et al., 2013). • duration and extent of disease;
• family history of CRC (patients with an affected first-
Crohn’s Disease degree relative have a twofold increased risk);
• Indications: • primary sclerosing cholangitis;
1. Severe colitis refractory to medical therapy—subtotal • young age at diagnosis;
colectomy or panproctocolectomy without IPAA • evidence suggesting persistent inflammation, which
2. Stricturing disease causing obstruction increases the risk of colorectal cancer; this is now con-
3. Crohn’s mass/abscess sidered when determining surveillance intervals. This
4. Fistulizing disease (including perianal disease with emphasizes the importance of compliance to treatment
associated sepsis) and good disease control.
5. Colonic dysplasia/cancer • All patients should undergo a repeat colonoscopy at 10
• Patients with perianal sepsis associated with Crohn’s disease years from initial diagnosis. This should ideally be per-
should undergo an examination under anaesthesia (EUA), formed as a dye-spray chromoendoscopy with the patient
ideally by a colorectal surgeon experienced in managing in clinical remission and will guide the need for further
IBD. This will often involve drainage of any abscess/ surveillance, which may be at 1, 3, or 5 years dependant
collection, laying open of fistula tracts +/− insertion of on the estimated risk.
a noncutting Seton suture to allow drainage and prevent
recurrent sepsis. Fertility and Pregnancy
• Perianal disease may also be treated with antibiotics (e.g., • Patients with inactive IBD can be expected to have normal
metronidazole or ciprofloxacin). However, long-term use fertility levels, but this is reduced by active disease.
of these drugs is limited by concerns regarding antibiotic • Both male and female patients undergoing surgery are at
resistance, antibiotic-associated diarrhoea, and other side risk of resultant infertility (via impotence/ejaculatory
effects (e.g., neuropathy with metronidazole and seizures problems and impaired tubular function, respectively).
with ciprofloxacin). This is of particular concern when pouch formation and/
or rectal excision is performed. Sulphasalazine (but not
Indications for Admission other aminosalicylates or thiopurines) cause a reversible
• Patients with acute severe colitis require hospital admis- and dose-dependent decrease in sperm motility and count.
sion for intravenous steroids +/− rescue medical therapy • Methotrexate is teratogenic and therefore should not be used
with infliximab (or, in cases where this is contraindicated, in females wishing to conceive (van Assche et al., 2010).
cyclosporin). The rate of colectomy (nonelective) for • Patients should be in clinical remission before conception
inpatients with UC remains relatively high at 10% to as this will be associated with better pregnancy outcomes.
15% (Royal College of Physicians, 2014). Acute severe • Folate supplementation (about 2 mg/day) is important
colitis is defined according to Truelove and Witts (1955) to prevent neural tube defects. This is particularly impor-
criteria as follows: tant when sulphasalazine is used since this may interfere
with folate absorption.
• IBD is a risk for preterm birth and low birth weight but
Bowel movements (no. ≥6 plus at least one of the not stillbirth or congenital malformations.
per day) features of systemic • If conception occurs when disease is quiescent, then the
upset (marked with an*) risk of relapse during pregnancy is the same as in
Blood in stools Visible blood nonpregnant women. However, in patients who conceive
Pyrexia (temperature Yes when the disease is active, the majority will continue
>37.8°C)* to have active disease during pregnancy (e.g., in
Pulse rate >90 bpm* Yes Crohn’s disease two-thirds of those with active disease
Anemia* Yes at conception continue to have active disease with the
Erythrocyte sedimentation >30 majority [two-thirds] deteriorating clinically during their
rate (mm/h)* pregnancy).
• Medical treatment (except MTX) should generally be and vaccination considered before initiation of steroids,
continued throughout pregnancy as the benefits of good immunosuppressants, or anti-TNF monoclonal antibody
disease control outweigh the risks of therapy. therapy in the nonimmune.
• Specific guidance: • Influenza, pneumococcal, and human papillomavirus
• Metronidazole should be used with caution and only (HPV) (females) vaccination is recommended for immu-
if there is no alternative due to the risk of prematu- nosuppressed adults and is best considered for all patients
rity. Ciprofloxacin or co-amoxiclav is preferable but with IBD, given the frequent need for steroid and immu-
to minimize risk, the shortest possible course should nosuppressive therapy. Booster vaccinations are appro-
always be used. priate for influenza (annually) and pneumococcus (after
• Suppositories and enemas are safe until the third 3 years).
trimester. • Live vaccines should be avoided in patients on immuno-
• Despite having an FDA rating D, which is extrapolated suppression or steroids (measles, mumps, rubella [MMR],
from animal studies, human studies suggest azathioprine oral polio, yellow fever, live typhoid, varicella, bacillus
and mercaptopurine are safe and well tolerated (Alstead, Calmette-Guérin [BCG]).
Ritchie, Lennard-Jones, Farthing, & Clark, 1990; • Immunoglobulin postexposure prophylaxis of nonimmune
Francella et al., 2003; Norgard, Pedersen, Fonager, individuals on high-dose steroid or immunosuppression
Rasmussen, & Sorensen, 2003; Zlatanic et al., 2003). should be considered after exposure to varicella or measles.
• Anti-TNF drugs are recognized to cross the placenta Acyclovir prophylaxis may also be used for varicella (van
in the late second and third trimesters. To minimize Assche et al., 2010).
foetal exposure and subsequent immunosuppression,
consideration may be given to discontinuing these Chronic Diarrhoea
drugs at 24 weeks of gestation if patients are in remis-
sion. If continued throughout pregnancy, a neonatolo- GUIDELINE
gist should be consulted and live vaccines avoided in
the foetus until at least 6 months old. Thomas, P.D., Forbes, A., Green, J., Howdle, P., Long, R.,
Playford, R., et al. (2003). Guidelines for the investigation of
• Flares should be treated aggressively and similar to non- chronic diarrhea (2nd ed.). Gut, 52, S1–15.
pregnant patients.
• Patients with uncomplicated IBD can deliver vaginally.
Caesarean section should be preferred in perianal Crohn’s
disease, active rectal disease, or when an ileoanal pouch • Chronic diarrhoea may be defined as the abnormal passage
anastomosis is present. Colectomy or ileostomy patients of three or more loose or liquid stools per day for more
can deliver vaginally. than 4 weeks and/or a daily stool weight greater than
• Close collaboration between gastroenterologists and 200 g/day.
obstetricians is encouraged and in complex cases, con-
sideration should be given to early referral via pregnancy Further Investigation
planning or medical obstetric clinics (van Assche et al.,
2010). • NB: Refer to secondary care for further investigation
patients over 60 years of age with a change in bowel habit
Breastfeeding to looser and/or more frequent stools.
• Sulphasalazine, other aminosalicylates, thiopurines, and • Stool culture (including analysis for ova, cysts, and para-
anti-TNF therapy are all considered safe in breastfeeding. sites) should always be considered.
• Metronidazole and ciprofloxacin should be avoided as • Coeliac disease occurs in approximately 1 in 100 patients
they are excreted into breast milk. in the United Kingdom. Therefore serologic testing for
• Steroids appear in low concentrations in breast milk. To coeliac disease should be performed in all patients pre-
minimize exposure, a 4-hour delay after oral dosing may senting with diarrhoea:
be considered (van Assche et al., 2010). • Immunoglobulin A (IgA) tissue transglutaminase (tTG)
antibody testing is most common. In patients with
Vaccinations IgA deficiency (2%–3% of coeliac patients) an IgG
• A vaccine and infection history, including TB exposure, antiendomysial antibody can be tested (both have >95%
chickenpox history, and risk of hepatitis B, should be sensitivity and specificity).
obtained at diagnosis in patients with IBD. • Serology testing and histologic confirmation with duo-
• Varicella zoster serology should be checked if there is no denal biopsy must be performed on a gluten-rich diet
history of infection and immunization considered before to avoid false negative results.
initiation of steroids/immunosuppressants. • Microscopic colitis (lymphocytic or collagenous colitis)
• Hepatitis B serology should be checked in high-risk patients is increasingly recognised as a cause of chronic watery
(please refer to Immunisation Against Infectious Diseases: diarrhoea. The aetiology is unclear and it may occur as
The Green Book, Department of Health, United Kingdom) a postinfectious phenomenon but is often drug induced.
Commonly implicated drugs include lansoprazole, NSAIDs, GUIDELINES

and selective serotonin reuptake inhibitors (SSRIs), which
should be withdrawn if possible. Budesonide (9 mg daily
Coeliac disease: Recognition, assessment and management.
for 6 weeks) is effective at controlling diarrhoea in approxi- NICE clinical guideline. Retrieved from www.nice.org.uk/
mately 80%, but symptoms often recur on discontinuation. guidance/ng20.
Other therapeutic options include antidiarrhoeals, bismuth, Ludvigsson, J.F., Bai, J. C., Biagi, F., Card, T. R., Ciacci,
prednisolone, and aminosalicylates +/− bile acid sequestrants. C., Ciclitira, P. J., et al. (2014). The diagnosis and
management of adults with coeliac disease: Guidance
However, microscopic colitis is distinct from conventional
from the British Society of Gastroenterology. Gut, 63,
IBD and, importantly, is not associated with any long-term 1210–1228.
complication, so it should be managed symptomatically
(Nguyen, Smalley, Vege, & Carrasco-Labra, 2016).
• Malabsorption is often accompanied by steatorrhoea and
the passage of bulky, malodorous, pale stools. It may be • Human leukocyte antigen (HLA) types DQ2 (95%) or
associated with biochemical or haematologic abnormalities DQ8 are present in over 99% of individuals affected by
suggesting nutritional deficinecy (e.g., electrolyte deficien- coeliac disease.
cies, hypocalcaemia, low vitamin D, elevated prothrombin • Histologic features on duodenal biopsy are subtotal villous
time suggesting vitamin K deficiency, macrocytosis, or atrophy associated with crypt hyperplasia and increase in
haematinic deficiencies). intraepithelial lymphocytes.
• In patients with a history or investigations suggestive of • Coeliac disease is associated with increased risk of non-
malabsorption, consider the following: Hodgkin lymphoma and small bowel adenocarcinoma
• Faecal elastase (normal >500, low if pancreatic exocrine but not an overall increase in malignant conditions
failure) +/− CT pancreas. (Mooney, Hadjivassiliou, & Sanders, 2014).
• Hydrogen breath test or empiric trial of antibiotics • The interest in gluten as a factor in other conditions has
for small bowel bacterial overgrowth (SBBO)—par- increased and many individuals without coeliac disease
ticularly in patients with increased risk of this, such may identify themselves as gluten sensitive. The mechanism
as those with gut dysmotility or altered anatomy (e.g., for gluten sensitivity is unclear (Aziz et al., 2014; Lebwohl,
blind loop) due to previous surgery. Ludvigsson, & Green, 2015).
• Small bowel imaging (e.g., CT enterography or MRI
small bowel) to exclude small bowel Crohn disease Initial Testing and Referral
(Headstrom & Surawicz, 2005; Schiller, 2004).
• Bile salt malabsorption may occur following terminal ileal • Many individuals with coeliac disease are asymptomatic
resection but has also been described following cholecystec- but presenting symptoms which should prompt testing
tomy and in postinfectious or idiopathic diarrhoea. A 75Se for coeliac disease include:
homocholic acid-taurine (75Se-HCAT) scan demonstrat- • In children:
ing poor retention (<5% at 7 days) or an elevated serum a. recurrent abdominal pain;
7-alpha cholestenone suggests the diagnosis. However, a b. failure to thrive or short stature;
therapeutic trial of bile acid sequestrant (e.g., cholestyr- c. diarrhoea (only in 10%).
amine 4 g twice a day or colesevelam 3.75 g daily) is • In adults:
often preferred. a. diarrhoea (<50% of patients);
• Other rare causes of diarrhoea include: b. nonspecific abdominal symptoms often mimicking
• carcinoid syndrome—may be associated with flushing IBS;
and diagnosed by demonstrating elevated urinary c. anemia (around 50% of patients at diagnosis may be
5-hydroxyindoleacetic acid (5-HIAA); iron deficient but B12 and folate may also be low);
• gastrointestinal neuroendocrine tumours (GI NETs)— d. elevated liver enzymes;
consider gut hormone analysis; e. osteoporosis;
• facticious diarrhoea (e.g., laxative abuse) (Thomas et al., f. dermatitis herpetiformis (itchy, blistering rash on
2003). trunk). May precede clinical evidence of intestinal
involvement, but duodenal biopsies are abnormal;
Coeliac Disease g. aphthous stomatitis;
h. unexplained neurologic symptoms (may relate to
• Coeliac disease is an autoimmune condition causing micronutrient deficiency);
inflammation of the proximal small bowel induced by i. subfertility and low birthweight offspring.
the ingestion of gluten. • In addition to the above patients with a clinical picture
• It affects around 1% of the world’s population but inci- suggesting possible coeliac disease, testing is recommended
dence varies geographically. in the following groups:
• The prevalence of coeliac disease has increased in the last • First-degree relatives of patients with coeliac disease
50 years but most patients are undiagnosed. (risk is 10%)
• Patients with type 1 diabetes (risk is 4%–7%) • assess diet and adherence to gluten-free diet and con-
• Patients with other autoimmune disease with unex- sider the need for specialist dietetic advice.
plained symptoms • Suggested investigations:
• Individuals with Down or Turner syndrome • Measure tTG titre.
• IgA tTG antibody has 98% specificity and 95% sensitivity • Measure FBC, ferritin, folate, B12.
and is the initial investigation of choice. • Check calcium levels, alkaline phosphatase, and vitamin
• Where IgA deficiency is present, IgG antiendomysial D levels (Ludvigsson et al., 2014).
antibody should be tested. • Bone densitometry scan should be offered to patients
• Adult patients with positive antibody screening should with two or more of these: BMI less then 20, weight loss
be referred for oesophagogastroduodenoscopy (OGD) over 10%, age older than 70 years, persistent symptoms,
and duodenal biopsy. poor adherence to gluten-free diet (British Society of
• Patients referred for biopsy should be told to continue Gastroenterology [BSG], 2007).
to take gluten in at least one meal per day in the interim. • If osteopenia, repeat after 3 years on gluten-free diet.
• In symptomatic children an IgA tTG titre more than 10 • Repeat at age 55 years for men or at menopause for
times the upper limit of normal may be sufficient for women if normal at baseline.
diagnosis without biopsy (Murch et al., 2013). • Patients with osteoporosis should be treated according
• HLA typing should only be carried out in specialist set- to current guidelines.
tings to exclude coeliac disease: • All patients should be encouraged to ensure adequate
• in patients who exclude gluten prior to biopsy and are calcium intake, taking supplements if necessary, to
not willing to reintroduce it; achieve this.
• to minimise future testing in high-risk individuals such • Other autoimmune conditions are common in individuals
as first-degree relatives (NICE, 2015a). with coeliac disease and these should be tested for where
there are symptoms.
Further Management
Refractory or Recurrent Symptoms
• Where coeliac disease is confirmed by duodenal biopsy,
patients should be advised on lifelong exclusion of gluten • Patients with refractory symptoms or recurrence of symp-
from their diet. toms should be referred to gastroenterology for assessment.
• Patients diagnosed with coeliac disease should be referred • Causes of ongoing diarrhoea include:
to a dietitian. • noncompliance with gluten-free diet or inadvertent
• Patients should be advised to avoid all wheat, rye, and barley. gluten ingestion;
• Guidance on prescription of gluten-free products can be • lymphocytic colitis;
found in the document “Gluten Free Foods: A Revised • small bowel bacterial overgrowth;
Prescribing Guide” online at www.coeliac.org.uk. Prescrib- • lactose intolerance;
ing of gluten-free products may be restricted according • hyperthyroidism;
to local policy. • pancreatic insufficiency;
• Oats should be avoided initially but certified gluten-free • refractory coeliac disease;
oats may be introduced after 6 to 12 months with follow- • small bowel lymphoma or adenocarcinoma (Mooney
up assessment and serolologic testing to assess tolerance et al., 2014).
(NICE, 2015a).
• Patients should have a repeat biopsy if tTG titre has not Refractory Coeliac Disease
fallen to normal range after 12 months on gluten-free
diet (Ludvigsson et al., 2014). • Refractory coeliac disease (RCD) is diagnosed when clini-
• Patients should be encouraged to join a national coeliac cal and histologic features of coeliac disease persist despite
support group (e.g., Coeliac UK). strict gluten exclusion for at least 6 to 12 months.
• Patients with coeliac disease should be vaccinated • The incidence is uncertain but rare.
against: • Type 1 RCD small bowel lymphocytes are polyclonal and
• pneumococcus; express typical CD3/CD8 positivity. Response to treat-
• meningitis C; ment with steroids and immunosuppression is good and
• Haemophilus influenza B; 5-year survival is over 90%.
• influenza annually. • Type 2 RCD have aberrant or clonal T-cell lines with loss
of CD3/CD8 positivity.
Ongoing Management • Response to treatment is poor and progression to lym-
phoma is common; 5-year survival is 40% to 60% (Al
• Patients should be seen on an annual basis to: Toma et al., 2007; Rubio-Tapia & Murray, 2010).
• measure weight and height; • There are no guidelines for treatment of this condition,
• assess symptoms; so patients should be referred to a tertiary centre.
Lymphoma and Cancer Risk Causes of Iron Deficiency Anemia

• Individuals with coeliac disease should be reassured that • Coeliac disease is the most common cause of iron defi-
life expectancy is not reduced. ciency from malabsorption (prevelance 4%–6%). Other
• Patients with coeliac disease have around a two- to fourfold causes including postgastrectomy, Helicobacter coloniza-
increased risk of non-Hodgkin lymphoma equating to tion, gut resection, and small bowel bacterial overgrowth
about 1 in 2000 per year (Mooney et al., 2014). are less common.
• Enteropathy associated T-cell lymphoma (type 1) • Benign sources of gastrointestinal blood loss include aspirin
• This is a rare form of lymphoma associated with coeliac and NSAID use, which account for 10% to 20% of patients.
disease particulary type 2 RCD. • Prevalence of GI tract cancers are in the region of:
• Prognosis is poor with 2-year survival less than 20% • colonic 5% to 10%;
(Al Toma et al., 2007). • gastric 5%;
• Highest rate of diagnosis is in first year after diagnosis, • oesophageal 2%;
and risk reduces with compliance to a gluten-free diet. • small bowel tumours 1% to 2%;
• Risk of small bowel adenocarcinoma is more than 30 • ampullary carcinoma <1% (Goddard et al., 2011)
times higher in patients with coeliac disease, although it • Non-GI sources of blood loss include menstruation (20%–
is still rare (Howdle, Jalal, Holmes, & Houlston, 2003; 30%), blood donation (5%), and haematuria (1%); if
Mooney et al., 2014). persistent warrants urology referral (Goddard et al., 2011).
• Gluten-free diet is thought to reduce the risk of lymphoma
and small bowel cancer. History
Noncoeliac Gluten Sensitivity • Should include dietary review, careful history for any
source of blood loss, and/or associated symptoms (e.g.,
• Many people without coeliac disease perceive that there dyspepsia, altered bowel habit, and weight loss).
are health benefits to excluding gluten from diet (Lebwohl • Ask about previous history of anemia and investigation.
et al., 2015). • Ask about family or personal history of haemoglobin-
• In a population study of 1002 people in the United opathies, telangiectasia, or other bleeding disorders.
Kingdom, 13% reported gluten sensitivity and 3.7% avoid • Enquire about family history of GI tract cancer, IBD, or
eating gluten (Aziz et al., 2014). coeliac disease.
• Some patients with IBS improve when gluten is restricted. • Check drug history (e.g., anticoagulants, antiplatelets,
• Some of these patients will have IBS and be sensitive to NSAIDs).
FODMAPs. • Ask about previous surgical history (e.g., gastrectomy).
• The biologic basis for noncoeliac gluten sensitivity is not
established, therefore a frank discussion with patients Examination
regarding risks and benefits of dietary restriction is
recommended. • Clinical signs of iron deficiency are unusual, but patients
should be examined to exclude an abdominal mass and
Iron Deficiency Anemia those with rectal bleeding or tenesmus should have a
digital rectal examination (Goddard et al., 2011).
GUIDELINE
Goddard, A.F., James, M.W., McIntyre, A.S., & Scott, B.B. Initial Investigation
(2011). Guidelines for the management of iron deficiency
anaemia. Gut, 60, 1309–1316. • Full blood count with mean cell volume, blood film,
ferritin level.
• Low serum ferritin is recognised as the best indicator
of iron deficiency.
• Iron deficiency is common affecting up to 2% to 5% of • Ferritin is elevated in inflammatory states, so iron defi-
men and postmenopausal women in developed countries ciency can exist in the presence of normal ferritin.
(Goddard, James, McIntyre, & Scott, 2011). • Iron studies may help to clarify iron deficiency but
• Causes of iron deficiency include dietary deficiency, lack can be difficult to interpret. The accompanying table
of absorption from the diet, and chronic loss of blood. may help interpretation.
• Endoscopic investigation of iron deficiency in the absence • IgA tTG antibody for coeliac disease.
of anemia rarely yields a diagnosis of malignancy. Risks • Urinalysis in all patients (Goddard et al., 2011).
and benefits of investigation should be discussed with • The 2015 NICE guidelines for recognition of lower GI
the individual patient and should only be considered in cancers suggest considering faecal occult blood (FOB)
patients aged over 50 or with GI symptoms (Goddard testing in those with iron deficiency anemia without rectal
et al., 2011). bleeding who are under the age of 60 years.
Iron Deficiency Anemia of then for 3 months to replenish iron stores (Goddard
Test Anemia Chronic Disease et al., 2011; Short & Domagalski, 2013).
• A haemoglobin rise of 1 g in 4 weeks indicates adequate
MCV/MCH Low Low/normal response to treatment.
Ferritin Low Normal/high • Lack of response should prompt assessment of compliance
and/or consideration of ongoing blood loss or problems
Iron Low Low
with absorption.
TF High Low • Parenteral iron can be used where oral preparations are not
TF saturation Low Normal tolerated or not absorbed adequately (Goddard et al., 2011).
MCV/MCH, Mean corpuscular volume/mean corpuscular haemoglobin;
TF, transferrin Weight Loss
GUIDELINES
Suspected cancer: Recognition and referral. NICE clinical
Further Investigation guideline 12. Retrieved from www.nice.org.uk/guidance/ng12.
• Menstruating women with no GI symptoms should be National Institute for Health and Care Excellence. (2006).
Nutrition support for adults: Oral nutrition support, enteral
screened for coeliac disease with IgA tTG antibody. tube feeding and parenteral nutrition. NICE clinical guideline
• More invasive gastrointestinal investigation is only war- 32 (updated 2017). Retrieved from www.nice.org.uk/
ranted where there are GI symptoms or other high-risk guidance/cg32.
factors (i.e., age >50 years or strong family history of GI
tract malignancy) (Goddard et al., 2011).
• Women who are not menstruating and not pregnant, and • Unintentional weight loss is common, particularly in elderly
men of any age should have both upper and lower GI patients with 15% to 20% of those aged over 65 years, but as
investigation (Goddard et al., 2011). many as 50% to 60% of those residing in nursing homes are
• Endoscopy is the best test for investigation of the upper affected (McMinn, Steel, & Bowman, 2011; NICE, 2006).
GI tract. • Weight loss of 5% of body weight over 6 to 12 months
• Distal duodenal biopsies should be taken to exclude coeliac is generally accepted as significant, but smaller losses in
disease unless tTG is negative. frailer patients may also be important.
• Colonoscopy is the optimal lower GI investigation because • Weight loss may be due to reduced calorie intake and
of high sensitivity and ability to biopsy or remove polyps. conditions causing malabsorption, but in other conditions
• CT virtual colonoscopy is (up to 90%) sensitive for lesions such as weight loss in malignancy the mechanism is less
larger than 1 cm but does not allow for biopsy and histologic well understood.
diagnosis. This investigation usually still involves full bowel • Weight loss is not exclusively a symptom of gastrointestinal
preparation but can be performed with minimal preparation disease, which is the cause in around one-third of cases,
and/or without contrast on request (Spada et al., 2014). so a holistic approach to the patient is important.
• Following CT colonoscopy, flexible sigmoidoscopy may • Exclusion of malignancy is often the primary concern for
be required for completion as views of the rectum can both patient and doctor. Referral via urgent suspicion of
be suboptimal. cancer pathway for assessment or investigation should be
• Small bowel investigation is not indicated in every patient offered (NICE, 2015b).
with iron deficiency anemia but should be considered in • No cause may be found in up to 25% of older adults.
those who become anemic again after iron replacement, Malignancy may be the cause in a similar proportion.
particularly if the patient is young or transfusion depen- Data for younger adults are lacking (Gaddey & Holder,
dent despite iron supplementation (Goddard et al., 2011; 2014; McMinn et al., 2011).
Sidhu, Sanders, Morris, & McAlindon, 2008). • The incidence of eating disorders is increasing. Both genders
• In such cases: can be affected and a wide range of age from childhood
• Capsule endoscopy has a high diagnostic yield but is upwards (Micali, Hagberg, Petersen, & Treasure, 2013).
not a therapeutic modality, so it may need to be fol- • Patients with eating disorders have significantly increased
lowed up by push or balloon enteroscopy. mortality rates (Arcelus, Mitchell, Wales, & Nielsen, 2011),
• MR or CT enterography is sensitive for diagnosing therefore it is important to have a high index of suspicion.
inflammation and mucosal thickening but may not
show small vascular abnormalities or flat lesions. History and Examination
Treatment • Many conditions and medications can lead to reduced

appetite. Careful history of what the Patient eats as well
• Elemental iron 120 mg/day (e.g., ferrous fumarate 210 mg as factors associated with their ability to access, prepare,
3 doses/day) is required until haemoglogin is normal, and consume food is important (Gaddey & Holder, 2014).
• Medication side effects including nausea and altered • The yield from further blind investigation is low and an
sense of taste may be important (Alibhai, Greenwood, approach of watchful waiting over 3 to 6 months may
& Payette, 2005). be appropriate at this stage particularly in elderly patients
• Thorough systematic review is key to elicit other symptoms (Alibhai et al., 2005; McMinn et al., 2011).
which can guide referral pathway. • In younger patients and where malignancy is still strongly
• It is essential to document the patient’s weight, as self- suspected, CT thorax abdomen and pelvis may be the
reporting of weight is often not reliable. next investigation of choice.
• Full examination of all systems and examination for
lymphadenopathy is indicated. Treatment
Investigation • Nutrition support is advised in patients with body mass

index below 18.5 kg/m2, loss of 10% body weight in 3
• There are no guidelines specifically for the investigation to 6 months, or BMI less than 20 and loss of over 5%
of weight loss in all patients but there are many recom- body weight in 3 to 6 months.
mendations for investigation of suspected cancer. Patients’ • Dietetic input is essential to ensure micronutrient needs
symptoms should guide initial investigation. are met as well as energy and protein needs (NICE, 2006).
• Laboratory investigation: • Social support, aids, and equipment may be just as impor-
• tTG antibody tant for some patients, so a full multidisciplinary approach
• FBC is required.
• Thyroid function tests (TFTs) • No pharmacologic treatments have a proven benefit in
• CRP improving weight gain.
• Glucose • In patients with depression mirtazapine may be appro-
• There is some evidence that in older patients elevated priate. Megestrol is sometimes used in patients with
serum LDH is predictive of organic cause (McMinn poor appetite, but the evidence base for this is poor and
et al., 2011). side effects may limit use (Alibhai et al., 2005; McMinn
• Tumour markers are generally unhelpful but CA125 et al., 2011).
should be tested in women with weight loss, loss of
appetite, or early satiety (NICE, 2015b).
• HIV test should be performed (British HIV Associa- Jaundice and Abnormal Liver
tion, 2008). Function Tests
• Faecal elastase if diarrhoea or risk factors for pancreatic
disease GUIDELINES AND REVIEWS
• Faecal calprotectin if diarrhoea
Williams, R., et al. (2014). Addressing liver disease in the UK:
Endoscopy A blueprint for attaining excellence in health care and
reducing premature mortality from lifestyle issues of excess
• Oesophagogastroscopy (OGD) in any patients with uninten- consumption of alcohol, obesity, and viral hepatitis. Lancet,
tional weight loss. Patients aged over 55 years with abdominal 384, 1953–1997.
pain, dyspepsia, reflux, or nausea and vomiting with weight Lilford, R., et al. What is the best strategy for investigating
loss should be referred for urgent direct access OGD. abnormal LFTs in primary care? BMJ Open, 3, e003099.
Dyson, J.K., et al. (2014). Non-alcoholic fatty liver disease:
• Patients with weight loss associated with altered bowel A practical approach to treatment. Frontline Gastroenterology,
habits, rectal bleeding, or iron deficiency anemia should 5, 277–286.
be referred for urgent assessment or direct access colon- National Institute for Health and Care Excellence. (2016).
oscopy (NICE, 2015b). Assessment and management of cirrhosis. NICE clinical
guideline 50. Retrieved from www.nice.org.uk/guidance/ng50.
Imaging
• Ultrasound scan pelvis in women over 40 years especially
if bloating. • Liver disease is increasing in prevalence and general
• CXR all ever smokers or any respiratory symptom. practitioners will see many patients with abnormal liver
• CT abdomen as urgent direct access in patients aged 60 or function tests.
over with diarrhoea, constipation, nausea, and vomiting or • In England and Wales, 600,000 people have some form
abdominal or back pain or new onset diabetes (NICE, 2015b). of liver disease, with around 60,000 with cirrhosis (Wil-
liams et al., 2014).
Further Management • Death from liver disease is increasing and is the third
most common cause of premature death in the United
• In elderly patients who have been investigated along the Kingdom.
lines above with negative results, malignant diagnosis is • General practitioners are best placed to identify patients
uncommon. at risk of liver disease and intervene to modify risk factors.
• Less than 5% of people with abnormal liver tests will d. Diet and weight loss supplements increasingly (Bjorns-
have a specific liver disease and less will require treatment son et al., 2013)
(Lilford et al., 2013a; Lilford, Bentham, Armstrong, Neu- (For more information on DILI, go to www.livertox.nih.gov.)
berger, & Girlinget, 2013b).
• Conversely many individuals will be developing Social History
liver fibrosis with normal liver function tests (Williams • Alcohol history (units per day or week):
et al., 2014). a. The UK Department of Health (2016) recommends
that to reduce the risk of harm from alcohol all adults
Special Situations drink no more than 14 units of alcohol per week spread
evenly over 3 days of the week.
• Patients with jaundice and fever with right upper quadrant b. Daily alcohol of less than 20 g for a woman or less
pain may have ascending cholangitis and require urgent than 30 g for a man are unlikely to be the cause of
admission for antibiotics and definitive management with liver disease.
ERCP. c. Patients with harmful drinking should be offered inter-
• All patients aged over 40 with jaundice should be ventions to reduce this (NICE, 2016a).
referred as urgent to be seen within 2 weeks by a specialist • Risk factors for bloodborne and sexually transmitted
(NICE, 2015b). infections
• Patients with evidence of impending liver failure such as • Travel history
altered mental status or in whom paracetamol overdose is • Family history (e.g., haemochromatosis, Gilbert syndrome)
suspected should be admitted as emergency (Bernal, Auz-
inger, Dhawan, & Wendon, 2010). Past Medical History
• Jaundice or abnormal LFT in the pregnant patient, par- • Comorbidities: diabetes and metabolic syndrome, auto-
ticularly in the third trimester, may indicate acute fatty immune diseases
liver of pregnancy, preeclampsia, or HELLP syndrome
and requires urgent admission to an obstetric unit Examination
(Hay, 2008).
• Decompensation of chronic liver disease with jaundice, • This may reveal evidence of underlying chronic liver disease,
encephalopathy, tense ascites, or evidence of bleeding is tender hepatomegaly in acute hepatitis, right upper quad-
likely to require emergency admission. rant tenderness, and Murphy sign in cholecystitis.
• Patients with isolated elevation of bilirubin are likely to • It is useful to document the patient’s weight and BMI.
have Gilbert syndrome and should have levels of conju-
gated and unconjugated bilirubin measured and be Initial Investigations
screened for evidence of haemolysis.
• Blood tests:
• Initial LFT panel. Alanine transaminase (ALT) and alkaline
History phosphatase are most useful to guide whether this is a
History of the Presenting Complaint hepatitis or cholestatic picture (Lilford et al., 2013b).
• May give the diagnosis (e.g., where right upper quadrant • Coagulation. Prolongation of prothombin time may indi-
pain is associated with dark urine and pale stools in cate liver failure or vitamin K deficiency in cholestasis).
choledocholithiasis). • Full blood count. Thrombocytopaenia may indicate
• Hepatitis may present with general malaise and nonspecific chronic liver disease and portal hypertension.
symptoms. • Initial liver screen:
• Many patients with abnormal LFTs are entirely asymp- • A prospective cohort study has shown that repeating
tomatic and are picked up incidentally. abnormal LFTs after a period of time is an inefficient
use of resources and suggests that individuals are instead
Drug History tested for significant liver diagnoses directly (Lilford
• Incidence of drug-induced liver injury (DILI) is in the et al., 2013b).
region of 20 per 100,000 per year (Bjornsson, Bergmann, • The following panel is suggested:
Bjornsson, Kvaran, & Olafsson, 2013). a. Hepatitis B and C serology (Hep A, E, Epstein-Barr
• Careful drug history should go back at least 6 months virus [EBV], and cytomegalovirus [CMV] in an
prior to the abnormality. acute hepatitis)
• Ask about nonprescribed drugs, Chinese, herbal, and other b. Immunoglobulin levels
complementary therapies and dietary supplements. c. Antinuclear antibody (ANA), antimitochon-
• Common causes of DILI: drial antibody (AMA), antismooth muscle anti-
a. Antibiotics; co-amoxiclav, nitrofurantoin body (SMA), antinucleolar cytoplasmic antibody
b. NSAID; diclofenac (ANCA), liver kidney microsomal antibody (LKM),
c. Immunomodulators; azathioprine, infliximab and soluble liver antigen
d. Ferritin and (if elevated) transferrin saturation and highly effective available treatments. Patients at risk should
HFE gene be encouraged to have testing (NICE, 2013b; NICE
e. caeruloplasmin 2016a; Williams et al., 2014).
f. Alpha-1 antitrypsin level
g. Tissue transglutaminase antibody Hepatitis B
h. HIV test (particularly where risk factors are • Hepatitis B has a prevalence of 0.1% to 0.5% in the United
identified) Kingdom. But this varies between individual communities.
• Imaging: Worldwide an estimated 350 million people are infected.
• Ultrasound scan of the liver and biliary system is often Transmission is predominantly vertical in developing coun-
a useful first line investigation, especially where biliary tries. The majority of patients diagnosed in the United
obstruction is suspected. MRCP is indicated for abnor- Kingdom have migrated from endemic areas.
mal biliary dilatation or persistent cholestatic LFTs. • All pregnant women in the United Kingdom should be
• ERCP is reserved for patients in whom intervention screened antenatally. Any patient with positive HBV
is planned and is no longer regarded as a diagnostic serology should be referred to a specialist for further
procedure. management.
• Ultrasound may also be useful as a screening tool to • Not all patients require treatment at diagnosis but should
identify steatosis. have monitoring by a specialist to determine if and when
• Transient elastography (fibroscan): treatment should be started.
• Gives a measurement of liver stiffness, which correlates • Treatment when required is with a finite course of 48
with severity of liver fibrosis, and can be a useful tool weeks of pegylated interferon monotherapy or with (usually
in staging liver disease and prognosticating. It is par- indefinite) treatment with antivirals, now usually tenofovir
ticularly well validated for viral hepatitis. It may be or entecavir (NICE, 2013b).
less accurate in patients with high BMI (Castera, 2010; • Patients should be given advice on prevention of trans-
Castera et al., 2010; Czul & Bhamidimarri, 2016). mission of the virus and testing, and vaccination should
• NICE recommends the use of transient elastography be offered to sexual partners and the patients’ children.
(TE) to screen for cirrhosis in high-risk groups includ- • Hepatitis B is associated with increased risk of hepatocel-
ing individuals with hepatitis B, hepatitis C, and those lular carcinoma (HCC) in certain groups even in the
who drink in excess of 50 units of alcohol per week. absence of cirrhosis and surveillance will be offered (Euro-
• Serum biomarkers of fibrosis: pean Association for the Study of the Liver [EASL], 2012).
• These include direct markers such as hyaluronic acid
or procollagen III propeptide (PIIINP), proprietary Hepatitis C
panels of tests such as enhanced liver fibrosis (ELF) • The number of people in the United Kingdom with chronic
or FibroTest, or scoring systems based on a range of HCV is in the region of 215,000.
laboratory results (e.g., aspartate aminotransferase to • Injecting drug use is the main risk factor but migration
platelet ratio index [APRI] score or fibrosis-4 [FIB-4]). from endemic areas and receipt of blood products prior
Again these are validated against biopsy scores and can to 1991 are also important risk factors.
help to stage liver disease without the need for biopsy • HCV rarely causes an acute hepatitis and 75% to 85%
(Castera, Vilgrain, & Angulo, 2013; Czul & Bhami- of patients develop chronic active hepatitis.
dimarri, 2016). • Treatment of HCV has advanced rapidly in recent years
• NICE has recommended ELF for identifying advanced with the advent of a range of direct acting antivirals. These
fibrosis in patients with nonalcoholic fatty liver disease used in combination, ‘are well tolerated and offer high cure
(NICE, 2016b). rates with short treatment durations of between 8 and 16
• Liver biopsy: weeks. Treatment regimens including pegylated interferon
• Mainly required when there is diagnostic uncertainty are no longer recommended’ (EASL, 2018)
rather than for staging of liver disease; however, it may • All patients with chronic hepatitis C infection should be
be carried out when surrogate markers for fibrosis are considered for treatment of the virus and should be referred
borderline. Liver biopsy carries significant risks of to specialist care. Hepatitis C is now seen as an inf.
haemorrhage (0.05%–0.1%) and death (0.1%–0.01%).
In patients with coagulopathy or ascites, transjugular Hepatitis A
approach may be undertaken which reduces risk but • This faecal-oral transmitted virus is a cause of acute hepa-
gives smaller samples. titis often in the returning traveller.
• Vaccination with 2 doses 6 months apart is effective for
prevention in high-risk groups including travellers to
Specific Liver Diseases endemic areas, individuals who work in high-risk occupa-
Viral Hepatitis tions, and men who have sex with men with high-risk
• Chronic viral hepatitis B and C are important long-term sexual behaviours. Intravenous drug users and their close
conditions with significant morbidity and mortality and contacts should also be considered high risk.
• The illness is usually self-limiting with mild symptoms

occasionally lasting up to 6 months. In a minority Primary Sclerosing Cholangitis (PSC)
(<1%) of cases fulminant hepatitis may occur (Rezende • Male predominance and strong association with inflam-
et al., 2003). matory bowel disease (in 80% of cases).
• Diagnosis usually made by MRCP characteristic strictur-
Hepatitis E ing and dilatation—typical beading appearance.
• Also transmitted through the faecal-oral route, HEV usually • No specific treatment shown to prevent progression. Some
causes a mild self-limiting acute hepatitis or subclinical studies report improvement in biochemistry with certain
infection. doses of ursodeoxycholic acid; however, evidence is insuf-
• It can present with fulminant hepatic failure in pregnant ficient to recommend universally, and American guidelines
women. state this treatment should not be used due to an excess
• HEV is linked to outbreaks in places with contaminated mortality in a study of higher doses (Chapman et al.,
water supply in the developing world. In the United 2010; EASL, 2009).
Kingdom more recently, incidence is rising and may be • PSC carries a significant increased risk of cholangiocar-
linked to a reservoir of infection among animal popula- cinoma which can be difficult to diagnose, colon cancer,
tions, including pigs. and gallbladder cancer.
• Chronic infection with rapid progression to cirrhosis has • Surveillance is recommended with:
been documented in immunosuppressed groups (e.g., post • annual colonoscopy (chromoendoscopy where possible)
liver transplant), and in these groups treatment using with biopsies;
ribavirin may be indicated. • annual abdominal ultrasound for gallbladder (chole-
• The virus has been detected in donated blood from cystectomy if any abnormality). Some clinicians may
asymptomatic donors; however, the risk of transmis- perform interval MRCP; however, the evidence base
sion to immunocompetent individuals is unclear and for this is lacking.
the role for screening of blood products has not been • If dominant stricture is identified, then ERCP with
established. brushings +/− intervention may be required.
• Patients may present with extrahepatic features including
up to 5% with neurologic syndromes such as Guillain- Primary Biliary Cholangitis (PBC)
Barré syndrome (Dalton, Hunter, & Bendall, 2013). • Female preponderance (3:1) and association with other
autoimmune conditions.
Autoimmune Hepatitis (AIH) • Symptoms of itch and fatigue are typical.
• Presentation of this condition can range from a minor • Cholestatic LFTs IgM elevated and positive antimito-
elevation of transaminases with or without symptoms of chondial antibody.
general malaise through acute hepatitis to subacute liver • Typical phenotype associated with positive serology is
failure, athough the latter is rare. A relapsing remitting sufficient for the diagnosis, and biopsy is not always nec-
course may mean LFTs fluctuate with repeat testing. essary (EASL, 2009; Lindor et al., 2009).
• One-third of patients will have cirrhosis at diagnosis. • Treatment with ursodeoxycholic acid at a dose of 13 to
• There is a female preponderance (2:1) and there may be 15 mg/kg/day has been shown to improve long-term
a history of other associated autoimmune conditions prognosis in patients who respond biochemically.
including rheumatoid arthritis, Graves disease, and coeliac • Treatment of itch initially is with antihistamines. Bile seques-
disease. trants can be used (patients must be advised not to take
• Diagnosis is usually based on typical biochemistry along ursodeoxycholic acid within 2–4 hours of taking these) and
with positive autoantibodies and elevated IgG. in some cases rifaxim or naltrexone (Lindor et al., 2009).
• Liver biopsy is regarded as a prerequisite to confirm the • Hyperlipidaemia occurs but may not reflect increased
diagnosis and for staging and prognosis. The validity cardiovascular risk.
of noninvasive fibrosis markers is not well stablished • Osteoporosis is increased and should be screened for by
(EASL, 2015). dual-energy x-ray absorptiometry (DEXA) (Lindor et al.,
• Treatment is with steroids initially which may be in the 2009).
form of prednisolone or budesonide before introducing
a steroid sparing agent, usually a thiopurine. Overlap Syndromes
• Occasionally second line immunosupressive agents are • Patients may have features of two or more of the above-
required, including mycophenolate mofetil or tacrolimus. mentioned conditions. Treatment often follows the pre-
• Withdrawl of treatment should be undertaken with dominant condition or patients may receive a combination
caution as up to 50% to 90% of patients relapse. Treat- of immunosupression and ursodeoxycholic acid.
ment should be for at least 3 years and at least 24 months
from normalization of LFTs. Biopsy should be repeated Nonalcoholic Fatty Liver Disease
prior to treatment withdrawal to exclude disease activity • This is the most common liver disease in the Western
(EASL, 2015). world and with increasing prevalence, possibly the biggest
challenge in liver disease in the developed world in the • Can present as fulminant hepatic failure typically associ-
next decades; 17% to 40% of adults have some degree ated with haemolytic anemia.
of nonalcoholic fatty liver disease. • Ceruloplasmin measurement is a useful screening test
• Spectrum from steatosis to steatohepatitis to fibrosis and (levels will be low) but diagnosis requires liver biopsy and
cirrhosis. urinary copper.
• Death in these patients is mostly from cardiovascular • Treatment with chelating agents (i.e., penicillamine or
disease and cancer, but there is significant risk of progres- trientene) (Ferenci, 2004).
sion to liver cirrhosis (Angulo et al., 2015).
• Fibrosis on biopsy is still the best predictor of prognosis Chronic Liver Disease
but in practice noninvasive tests are usually preferred
(Angulo et al., 2015; EASL, 2016). GUIDELINE
• No specific treatments are available, but patients should
be counselled on lifestyle and risk factors. National Institute for Health and Care Excellence. (2016).
Cirrhosis in over 16s: Assessment and management. NICE
• Weight loss of 7% to 10% of body weight has been shown clinical guideline 50. Retrieved from www.nice.org.uk/
to improve fibrosis but the optimum method has not guidance/ng50.
been proven.
• There is some evidence of benefit from pioglitazone,
vitamin E, or obeticholic acid; however, these should
only be used within specialist settings (NICE, 2016b; • Cirrhosis results from injury (due to a variety of mecha-
Rinella, 2015). nisms or disease processes) leading to necroinflammation
• Statins are generally safe in this patient group and may be and fibrinogenesis within the liver. Histologic features of
beneficial, therefore should not usually be discontinued. liver cirrhosis include diffuse nodular regeneration, fibrous
septa, collapse of the normal liver architecture, and dis-
Alcoholic Hepatitis tortion of vascular structures.
• Alcohol is a common cause of cirrhosis. Alcoholic hepatitis • Clinical examination findings in advanced liver disease
presents with jaundice and carries a very high mortality may include:
when severe. Diagnosis is usually based on the history of • palmar erythema, spider naevi (>5 considered abnormal);
excessive alcohol consumption within 4 weeks prior to • caput medusae;
presentation and typical biochemistry with bilirubin greater • loss of body hair in men;
than 80 and aspartate aminotransferase/alanine transami- • gynaecomastia;
nase (AST/ALT) typically less than 500. • testicular atrophy;
• Treatment with corticosteroids may be considered under • all or none of these signs.
specialist supervision (Thursz et al., 2015). • Patients at risk of cirrhosis include individuals with a
BMI over 30, those who misuse alcohol, patients with
Hereditary Haemochromatosis type 2 diabetes, and patients with hepatitis B or C infec-
• Autosomal recessive inherited condition with homozygos- tion, as well as patients with other forms of liver disease
ity of C282Y mutation most common. as previously discussed.
• H63D /C282Y compound heterozygote is associated with • NICE recommends testing for cirrhosis with transient
some risk of iron overload. elastography in all men drinking more than 50 units
• Associated with iron overload from the third or fourth of alcohol per week (women >35 units) or anyone
decade. Later in women due to menstrual blood loss. diagnosed with alcohol-related liver disease. (NICE,
• Patients may present with joint pain or fatigue (van Bok- 2016a)
hoven, van Deursen, & Swinkels, 2011).
• Genetic testing is not recommended as a screening Staging of Advanced Liver Disease
test due to low penetrance of the condition (EASL,
2010). • Liver cirrhosis or advanced liver disease can be thought
• Ferritin level below 1000 with normal LFTs has a good of as a spectrum of clinical states from early disease with
negative predictive value for cirrhosis. compensated disease and absence of oesophageal varices,
• Treatment with venesection until iron stores (ferritin) through the development of portal hypertension, to decom-
depleted to less than 50 and, as required, venesection pensated liver disease with complications of varices and
thereafter (target ferritin 50–100). ascites.
• In patients in whom cirrhosis is suspected biopsy should • Prognosis varies between these stages with 1-year mortality
be performed. of 1% per year for early stage to more than 50% for
decompensated disease (Tsochatzis, Bosch, & Burroughs,
Wilson Disease 2014).
• Rare genetic condition with associated neuropsychiatric • Child-Pugh classification (see accompanying table) can
features. be used to describe the stage or severity of liver cirrhosis.
The Model for End-stage Liver Disease (MELD) and • Rapid accumulation of ascites may be due to development
UK Model for End-stage Liver Disease (UKELD) of portal vein thrombosis or HCC and should prompt
scores are used in the transplant assessment process referral and consideration of imaging.
to risk-stratify patients and select those in whom risk • Spontaneous bacterial peritonitis (SBP) may present with
of transplant is outweighed by risk of death without decompensation without specific symptoms and should
transplant. prompt urgent admission. Signs of peritonism are usually
absent. In hospital, mortality from SBP is around 20%.
Child-Pugh Classification of Chronic Liver Disease • After one episode of SBP, patients should receive long-
term antibiotic prophylaxis. Antibiotic choice is usually
Measure 1 Point 2 Points 3 Points a quinolone or cotrimoxazole but will be guided by local
Total bilirubin <34 34–50 >50 protocols and sensitivities.
(µmol/L)
Prothrombin time <4.0 4.0–6.0 >6.0
prolongation(s) Hepatic Encephalopathy
Ascites None Mild Moderate to
severe
• Hepatic encephalopathy (HE) will affect 30% to 40% of
Hepatic None Grade I–II Grade III–IV patients with cirrhosis at some time in their clinical course.
encephalopathy (or suppressed (or refractory) • HE can be classified according to the West Haven Criteria.
with Minimal hepatic encephalopathy (MHE) may be apparent
medication) only on psychometric testing; however, it may still lead
Serum albumin <28 28–35 >35 to a reduction in quality of life for patients.
(g/L)
• Overt hepatic encephalopathy (OHE) classically presents
with deficit in attention, reduced visiospatial awareness,
Management of Patients With Cirrhosis disorientation, alteration of sleep-wake cycle, change in
personality, and in later stages reduced concious level.
• Patients with stable compensated cirrhosis will usually be OHE usually has a precipitant such as variceal bleeding
seen at a liver clinc every 6 to 12 months for monitoring or infection.
and to arrange surveillance for HCC: • It is important in acute confusion to consider other causes
• Currently recommended 6 monthly ultrasound of liver and to be aware that patients with advanced liver disease
as HCC surveillance (Bruix & Sherman, 2011). are at risk of intracranial bleeding. There should be a low
• Patients should receive appropriate treatment depend- threshold for referral for urgent assessment and imaging
ing on the underlying aetiology of liver disease (e.g., in new confusion (EASL & American Association for the
antiviral therapy or venesection in haemochromatosis). Study of Liver Diseases [AASLD], 2014).
• Patients should be advised on lifestyle (weight reduc- • Lactulose is usually given to treat and prevent encephalopa-
tion in obesity, management of type 2 diabetes, smoking thy and rifaxamin is added in as a second line treatment.
cessation, and abstinence from alcohol). Both treatments are usually continued indefinitely.
Ascites
Varices
• Half of patients with compensated cirrhosis will develop
ascites within a 10-year period. • All patients at risk of varices should have a screening
• The development of ascites marks progression of liver endoscopy. Until recently this included all patients with
disease and associated 2-year mortality of up to 50%. cirrhosis; however, evidence now suggests that those with
The development of ascites should prompt consideration lower fibrosis scores (i.e., TE with stiffness <20 with
of assessment for transplantation. platelet count >150) may not require screening (Bosch
• Treatment is with low sodium diet (<2000 mg/day), & Sauerbruch, 2016; Tripathi et al., 2015).
aldosterone antagonist (e.g., spironolactone 100 mg • Grade 1 varices without red spots do not require primary
increasing in increments of 100 mg to maximum 300 mg) prophylaxis but should have further endoscopy after 1
with the addition of loop diuretics (e.g., furosemide year.
40–120 mg) in some cases. • Patients with Grade 2 or 3 varices receive nonselective beta
• Fluid restriction is not indicated unless hyponatraemia. blocker (NSBB) as primary prophylaxis (e.g., carvedilol
• Hyponatraemia is common and may limit diuretic use. 6.25 mg daily increasing to 12.5 mg daily after 1 week
Diuretics should be reduced or stopped if serum sodium if tolerated or propanolol 40 mg twice daily increased to
is below 125 mmol/L (Moore & Aithal, 2006). maximum tolerated dose). Patients on NSBB for primary
• NSAIDs should be avoided in patients with ascites due prophylaxis do not require routine endoscopy.
to cirrhosis. • Where NSBB is contraindicated, patients should have
• Angiotensin converting enzyme inhibitors (ACEi) and primary prophylaxis by endoscopic band ligation (EBL).
angiotensin receptor blockers (ARBs) may be harmful in • Patients who have had a variceal bleed should undergo
patients with ascites and should be stopped. a program of EBL every 2 to 4 weeks until varices are
obliterated. They should also receive NSBB unless there • In patients who survive after 1 year from transplant, renal
is a contraindication. disease and cardiovascular disease are the main causes of
• Transjugular intrahepatic portosystemic shunt (TIPSS) may morbidity and malignancy; and cardiovascular disease
be required as a recue therapy in acute variceal bleeding. accounts for most deaths. Many patients undergoing
• Bleeding from gastric varices can be more difficult to manage transplant have multiple risk factors including obesity,
endoscopically and more commonly requires TIPSS. metabolic syndrome, and type 2 diabetes (AASLD &
• There is controversy regarding the treatment with NSBB AST, 2012).
in advanced decompensated liver disease, with some • Patients should be given the following lifestyle advice:
studies indicating loss of benefit or harm from NSBB in • Diet: Avoid unpasteurized dairy products and raw or
more advanced disease but others showing benefit. More undercooked meat or eggs.
research is needed in this area and the decision to • Avoid smoking.
stop or restart NSBB should be made by the specialist • Use high factor sun protection and stay out of strong
(Krag & Madsen, 2015). sunlight. Patients should also be counselled to self-
examine their skin for suspicious lesions (AASLD &
TIPSS AST, 2012).
• Cardiovascular risk factors should be addressed, including
• Indicated as a rescue therapy in acute variceal haemor- weight management, hypertension, and diabetes.
rhage or for high-risk gastric varices. • Bone density should be considered and appropriately
• May be indicated for refractory ascites in appropriate managed according to protocols.
patients.
• Main adverse effect is encephalopathy which occurs in References
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ALGrawany
10
Surgical Problems
IAIN WILSON
Gallstone Disease Haemorrhoids
Investigation Assessment
Management Investigation
Biliary Colic Management
Cholecystitis Indications for Referral
Choledocholithiasis Thrombosed External Haemorrhoid
Anal Fissure
Gallbladder Polyps
Assessment
Diverticular Disease Management
Definitions Indications for Referral
Complications
Inguinal Hernia
Diverticulosis Investigation
Diverticular Disease Management
Acute Mild Uncomplicated Diverticulitis Indications for Referral
Investigation Vascular Problems
Management
Varicose Veins
Further Management
Management
Rectal Bleeding Referral
Assessment Peripheral Arterial Disease (PAD)
Investigation Diagnosis
Management Management
Indications for Referral
Gallstone Disease • 80% of gallstones are asymptomatic; 2% to 4% of people

with asymptomatic gallstones will develop symptoms or
GUIDELINES complications each year (Gurusamy & Davidson, 2014).
European Association for the Study of the Liver (EASL) • Complications of gallstones include biliary colic, chole-
Clinical Practice Guidelines. (2016). The prevention, diagnosis cystitis, pancreatitis, choledocholithiasis, cholangitis,
and treatment of gallstones. https://easl.eu/publication/ obstructive jaundice, and gallbladder cancer.
prevention-diagnosis-and-treatment-of-gallstones/.
National Institute for Health and Care Excellence. (2014). Investigation
Gallstone disease. Diagnosis and management of
cholelithiasis, cholecystitis and choledocholithiasis. NICE • Take bloods, including full blood count (FBC) and liver
clinical guideline 188. https://www.nice.org.uk/guidance/ function tests (LFTs) (National Institute for Health and
cg188/evidence/cg188-gallstone-disease-full-guideline3 Care Excellence [NICE], 2014). Serum amylase is only
required if there is suspicion of pancreatitis.
• Arrange an abdominal ultrasound.
• Gallstone disease is the most common gastrointestinal • Consider referring for magnetic resonance cholangiopan-
disorder causing hospital admission in European countries creatography (MRCP) if there are dilated bile ducts and/
(Farthing et al., 2014). or abnormal liver function tests.
134
CHAPTER 10 Surgical Problems 135
• Consider referring for MRCP or endoscopic ultrasound GUIDELINE

(EUS) if there is a strong suspicion of gallstones with a
EASL Clinical Practice Guidelines. (2016). The prevention,
negative abdominal ultrasound (AUS).
diagnosis and treatment of gallstones. https://easl.eu/
publication/prevention-diagnosis-and-treatment-of-gallstones/.
Management
• Laparoscopic cholecystectomy is not recommended for
patients with asymptomatic gallbladder stones in a normal
gall bladder. • Most polypoid lesions are benign, but a small number
• Refer all patients with symptomatic gallstones for con- are malignant or premalignant. The likelihood of a pol-
sideration of laparoscopic cholecystectomy. ypoid lesion being malignant or premalignant increases
• Advise patients to avoid food and drink that triggers their with polyp size.
symptoms until they have had their gallbladder removed • Cholecystectomy should be performed in patients with
(NICE, 2014). gallbladder polyps 1 cm or larger.
• Cholecystectomy should also be considered in patients
Biliary Colic with asymptomatic gallbladder stones and gallbladder
polyps 6 to 10 mm and in case of growing polyps.
• Patients with biliary colic require analgesia with paracetamol • Cholecystectomy is not indicated in patients with asymp-
and, if appropriate, nonsteroidal antiinflammatory drugs tomatic gallbladder stones and gallbladder polyps 5 mm
(NSAIDs). or smaller.
• Patients with severe symptoms may benefit from opioid
analgesia and spasmolytics.
• Cholecystectomy should be performed as early as possible Diverticular Disease
for patients with uncomplicated biliary colic (European
GUIDELINES
Association for the Study of the Liver [EASL], 2016).
Association of Coloproctology of Great Britain and Ireland
(ACPGBI) Commissioning Guide. (2014). Colonic diverticular
Cholecystitis disease. https://www.acpgbi.org.uk//content/uploads/2017/
• Antibiotics should be commenced if there is evidence of 02/Commissioning-guide-colonic-diverticular-disease-RCS
-2014.pdf.
sepsis, cholangitis, abscess, or perforation (EASL, 2016). National Institute for Health and Care Excellence. (2017).
These patients should usually have been admitted emer- Suspected cancer: Recognition and referral. NICE clinical
gently. Note: Patients with cholecystitis are usually prescribed guideline 12. https://www.nice.org.uk/guidance/ng12.
antibiotics. There is however some evidence to suggest that National Institute for Health and Care Excellence (NICE)
not all patients benefit from antibiotics. A small randomized Clinical Knowledge Summary. (2017). Diverticular disease.
https://cks.nice.org.uk/diverticular-disease#!rightTopic.
controlled trial could not demonstrate that intravenous World Society of Emergency Surgery (WSES) Guidelines.
antibiotic treatment improved outcomes of patients with (2016). WSES guidelines for the management of acute left
mild acute cholecystitis (Mazeh et al., 2012). sided colonic diverticulitis in the emergency setting. https://
• Patients with acute cholecystitis should be offered early www.ncbi.nlm.nih.gov/pmc/articles/PMC4966807/.
laparoscopic cholecystectomy by the admitting surgical
team (to be carried out within 1 week of diagnosis) (NICE,
2016). A Cochrane review has shown that early laparo- Definitions
scopic cholecystectomy for acute cholecystitis shortens
the total length of hospital stay (Gurusamy, Davidson, • Diverticulosis: presence of diverticula that are asymp-
Gluud, & Davidson, 2013). tomatic. The presence of diverticula is rare in individuals
under the age of 40; 50% of the population aged over
Choledocholithiasis 50 years is affected, 70% aged over 80 years of age is
affected (Association of Coloproctology of Great Britain
• Common bile duct (CBD) stones should be suspected in and Ireland [ACPGBI], 2014).
patients with acute cholangitis, acute pancreatitis, deranged • Diverticular disease: diverticula that are symptomatic.
liver function tests (LFTs), or a dilated biliary tree. • Diverticulitis: diverticula that become inflamed and/or
• Bile duct clearance and laparoscopic cholecystectomy infected. Classic symptoms include left iliac fossa pain,
should be offered to people with symptomatic or asymp- fever, and a change in bowel habit.
tomatic common bile duct stones (NICE, 2014). • Uncomplicated diverticulitis refers to localised diverticular
inflammation.
Gallbladder Polyps Complications
• Polypoid lesions of the gallbladder are found in 4% to • Complications of diverticulitis include rectal bleeding,
7% of patients who undergo abdominal ultrasound (Chou, local and generalised peritonitis, perforation, abscess,
Chen, Shyr, & Wang, 2019). fistula, and stricture formation.
• About 4% of people with diverticulosis will develop acute • There is evidence to suggest that selected patients with
diverticulitis (Shahedi, Fuller, & Bolus, 2013). acute uncomplicated diverticulitis do not require antibiot-
• Approximately 15% of people will have rectal bleeding ics. A multicentre randomised trial comparing antibiotic
from their diverticula (ACPGBI, 2014). treatment versus no antibiotic treatment of computed
tomography (CT) proven acute uncomplicated diverticu-
Diverticulosis litis showed that antibiotics did not expedite recovery or
prevent complications (Chabok et al., 2012).
• Advise patients to maintain a healthy, balanced, high-fibre • Reassess the patient within 48 hours, or sooner if symp-
diet with adequate fluid intake (NICE, 2017). toms deteriorate, and thereafter depending on response
to treatment.
Diverticular Disease • An emergency surgical admission should be arranged in
the following circumstances:
• Advise patients to maintain a healthy, balanced, high-fibre • There is suspicion of perforation or abscess.
diet with adequate fluid intake. • Symptoms cannot be managed in primary care.
• Consider prescribing bulk-forming laxatives. • The patient is unable to take or tolerate oral fluids at
• Patients should be referred to secondary care in the fol- home.
lowing circumstances: • The patient is unable to tolerate oral antibiotics at home.
• To confirm a suspected diagnosis of diverticular disease • The patient is frail and/or has significant comorbidities
• If symptoms affect quality of life and/or is immunocompromised (NICE, 2017).
• If pain is not controlled by paracetamol
• If there are symptoms that are suspicious for a colorectal Further Management
cancer (see upcoming guidelines). • All patients require investigation of the colonic lumen
• Patients with the following symptoms should be referred by endoscopy, barium enema, or CT colonography
for an appointment within 2 weeks under the NICE after the acute episode of diverticulitis has resolved
suspected cancer: recognition and referral pathway: (ACPGBI, 2014).
• Aged 40 and over with unexplained weight loss and • Indications for referral to secondary care include:
abdominal pain • frequent or severe recurrent episodes of acute
• Aged under 50 with rectal bleeding and unexplained diverticulitis;
abdominal pain, change in bowel habit, weight loss, • symptoms that affect quality of life;
and/or iron-deficiency anaemia • pain that is not controlled by paracetamol; and
• Aged 50 and over with unexplained rectal bleeding • the presence of red flag symptoms (i.e., change in
• Aged 60 and over with iron-deficiency anaemia, changes bowel habit, weight loss, symptoms suggestive of
in bowel habit, or tests show occult blood in faeces anaemia) (ACPGBI, 2014).
• Finding of a rectal or abdominal mass • Patient-related factors and not the number of episodes
of diverticulitis should dictate if elective sigmoid resec-
tion in indicated (World Society of Emergency Surgery
Acute Mild Uncomplicated Diverticulitis [WSES], 2016).
Investigation • Patients were previously offered elective bowel resection fol-
• Consider checking blood for raised white cell count and lowing two episodes of diverticulitis. The risk or recurrent
C-reactive protein (CRP). CRP is a useful tool in the diverticulitis is lower than previously thought. A systematic
prediction of the clinical severity of acute diverticulitis. review has shown that complicated diverticulitis follow-
Patients with a CRP value higher than 150 mg/L have ing an attack of uncomplicated diverticulitis is rare (<5%)
a significant risk of complicated diverticulitis (Mäkelä, (Regenbogen, Hardiman, Hendren, & Morris, 2014).
Klintrup, Takala, & Rautio, 2015). • Clear indications for elective sigmoid resections are symp-
tomatic stenosis, fistulas, and recurrent diverticular bleeding.
Management
• Referral to hospital is not mandatory for all patients with Rectal Bleeding
acute uncomplicated diverticulitis (ACPGBI, 2014).
• In selected patients without significant comorbidities,
management includes: GUIDELINE
• a diet of clear liquids only. Reintroduce solid food as ACPGBI Commissioning Guide. 2017. Rectal bleeding.
symptoms improve over 2 to 3 days; https://www.acpgbi.org.uk//content/uploads/2014/03/
• regular paracetamol for pain. Avoid NSAIDs and opioid Commissioning-Guide-for-Rectal-Bleeding-Dec-2017.pdf.
analgesics;
• considering prescribing broad-spectrum oral antibiotics
that cover anaerobes and Gram-negative bacilli (e.g., • Rectal bleeding is a common problem. In the United
co-amoxiclav or ciprofloxacin and metronidazole). Kingdom the 1-year prevalence in adults is about 10%
Treatment should last for at least 7 days (NICE, 2017). (ACPGBI, 2017).
• Most rectal bleeding is due to benign conditions such as • Clinicians should consider an emergency referral in patients
haemorrhoids, anal fissure, and diverticular disease. who are elderly, on oral anticoagulation, and unable to
• Rectal bleeding can be a manifestation of more sinister monitor bleeding.
problems such as inflammatory bowel disease and colorectal • Direct access flexible sigmoidoscopy provides the best
cancer. Clinicians should take care not to miss proximal reassurance for patients with rectal bleeding who are pri-
colorectal disease in the presence of benign anorectal marily concerned about malignancy (ACPGBI, 2017).
pathology (ACPGBI, 2017). Rectal bleeding has a positive
predictive value (PPV) for colorectal malignancy of 8% Indications for Referral
in patients aged over 50 (Astin et al., 2011). • Patients with the following symptoms should be referred
for an appointment within 2 weeks under the NICE
Assessment suspected cancer: recognition and referral pathway:
• Ask about perianal symptoms (pain, lump, prolapse, itch, • Aged under 50 with rectal bleeding and any unexplained
leakage, incomplete evacuation, and discharge). abdominal pain, change in bowel habit, weight loss,
• Enquire about bleeding, including colour of the blood iron-deficiency anaemia
(bright red, dark red) and where blood was observed (on • Aged 50 and over with unexplained rectal bleeding
the paper, coating stool, or in the pan). • Patients with rectal bleeding who do not fulfil the above-
• Enquire about any family history of colorectal cancer and mentioned criteria should be considered for urgent referral
inflammatory bowel disease. if there is:
• Ask about red flag symptoms, including weight loss, symp- • a strong family history of colorectal malignancy;
toms suggestive of anaemia, and change in bowel habit. • anxiety about colorectal malignancy;
• Perform an abdominal examination to exclude an abdomi- • persistent rectal bleeding despite treatment for
nal mass. haemorrhoids;
• Examine the external anus. Spread the buttocks and look • rectal bleeding in patients with a past history of pelvic
for fissures, prolapsed mucosa, or haemorrhoids. radiotherapy; and
• Perform a digital rectal examination (DRE). DRE will • suspected inflammatory bowel disease (ACPGBI, 2017).
provide useful information about the presence of perianal
disease, the prostate, sphincter tone, and the presence of Haemorrhoids
any pelvic masses. The cervix can commonly be palpated
anteriorly in women. Haemorrhoids are vascular structures GUIDELINES
and cannot usually be palpated on DRE. Patients with
American Society of Colon and Rectal Surgeons (ASCRS)
a fissure may not tolerate a DRE. Clinical Practice Guidelines. (2018). Management of
• If experience and logistics permit, undertake proctoscopy. haemorrhoids. https://www.fascrs.org/sites/default/files/
downloads/publication/cpg_management_of_hemorrhoids.pdf.
Investigation NICE Clinical Knowledge Summary. (2016). Haemorrhoids.
• Blood tests are not routinely indicated in patients with https://cks.nice.org.uk/haemorrhoids.
rectal bleeding.
• Consider FBC if there are signs and symptoms of anaemia.
• Other bloods tests may be indicated if bleeding is associ- • Haemorrhoids arise when the circular venous plexuses in
ated with problems such as weight loss or change in bowel the anus become persistently dilated.
habit. • Haemorrhoids that originate above the dentate line are
• If inflammatory bowel disease is suspected in a young, internal haemorrhoids. Haemorrhoids that originate below
low-risk patient consider a test for faecal calprotectin. A the dentate line are external haemorrhoids.
positive faecal calprotectin result has a high positive pre- • Factors that contribute to the development of haemor-
dictive value for finding inflammatory bowel disease at rhoids include constipation, straining, ageing, heavy lifting,
colonoscopy. chronic cough, pregnancy, and childbirth (NICE, 2016).
• Tumour markers and faecal occult blood testing are not
indicated in patients with rectal bleeding (ACPGBI, 2017). Assessment
• It is important for the clinician to establish that haemor-
Management rhoids are the cause of a patient’s symptoms and exclude
• The management of specific conditions will be discussed the presence of more proximal and sinister colorectal
later in this chapter. disease.
• Most bleeding resolves spontaneously. It is reasonable to • See “Assessment” under “Rectal Bleeding.”
manage low-risk patients with rectal bleeding with a “watch • Bleeding classically presents as bright red blood on the
and wait” policy (ACPGBI, 2017). toilet paper. Blood can also be seen in the toilet bowl or
• Emergency surgical referral should be arranged in the coating the faeces.
presence of haemodynamic instability, significant blood • Other possible symptoms include itching or irritation, a
loss, and the potential requirement for blood transfusion feeling of rectal fullness, discomfort, incomplete evacu-
(ACPGBI, 2017). ation, and soiling.
• Ask about prolapse. Internal haemorrhoids can be clas- • large skin tags (surgical excision may be required)
sified according to their degree of prolapse: (NICE, 2016).
• Grade I: project into the anal canal only • Most patients with grade I, II, and selected patients with
• Grade II: prolapse on straining but reduce grade III internal hemorrhoidal disease who fail medical
spontaneously treatment can be effectively treated with office-based pro-
• Grade III: prolapse on straining but require manual cedures. Hemorrhoidal banding is typically the most
reduction effective option (ASCRS, 2018).
• Grade IV: persistently prolapsed and cannot be reduced
Investigation Thrombosed External Haemorrhoid
• See “Investigation” under “Rectal Bleeding.” • Patients are usually advised to manage symptoms with
oral analgesia, topical analgesia, and laxatives. Most patients
Management treated nonoperatively will experience eventual resolution
• Diet and lifestyle modification are the first line for all of their symptoms.
patients with symptomatic haemorrhoids (American • Selected patients with thrombosed external haemorrhoids
Society of Colon and Rectal Surgeons [ASCRS], 2018). may benefit from early surgical excision (ASCRS, 2018).
• Recommend a diet with enough fluid and fibre intake so • One paper has shown that patients who underwent surgi-
stools are soft and easy to pass. cal management of their thrombosed haemorrhoid achieved
• Advise that patients minimise time spent sitting on the resolution of symptoms faster than those managed con-
toilet and straining. servatively (3.9 days vs. 24 days) (Greenspon, Williams,
• The anal region should be kept clean and dry. Young, & Orkin, 2004).
• Consider prescribing a bulk-forming laxative if there is
constipation. Anal Fissure
• Consider prescribing a topical haemorrhoidal preparation
(NICE, 2016). GUIDELINES
ASCRS Clinical Practice Guidelines. (2017). Clinical practice
Topical Haemorrhoidal Preparations guideline for the management of anal fissures. https://www.
• A variety of topical treatments are available to manage fascrs.org/sites/default/files/downloads/publication/cpg_for_
the symptoms of haemorrhoids. These may contain one the_management_of_anal_fissures_jan_2017_dcr_issue.pdf.
or a combination of mild astringents, lubricants, local NICE Clinical Knowledge Summary. (2017). Anal fissure.
anaesthetic, or corticosteroids. https://cks.nice.org.uk/anal-fissure.
• There is no evidence to suggest that one topical prepara-
tion is more effective than another, although lidocaine is
the preferred topical anaesthetic agent. • An anal fissure is a longitudinal tear in the skin of the
• Generally, haemorrhoidal preparations should be used in anal canal.
the morning and night, and after a bowel movement. • Primary anal fissures are thought to arise due to spasm of
• Prolonged use of any agent is not recommended; the internal anal sphincter causing localised ischaemia.
anaesthetic-containing preparations may cause sensitiza-
tion of the anal skin. Corticosteroid-containing prepara- Assessment
tions should be used for no longer than 7 days because • Spread the buttocks and inspect the anus.
prolonged use may lead to skin atrophy, contact dermatitis, • Of anal fissures, 90% occur in the posterior midline.
and skin sensitisation (NICE, 2016). • Features of chronic fissures include a hypertrophied anal
papilla at the proximal aspect of the fissure, a sentinel
Indications for Referral skin tag at the distal aspect of the fissure, and exposed
• Consider an emergency admission with acutely thrombosed internal anal sphincter muscle within the base of the
external haemorrhoids (see upcoming discussion) and fissure (NICE, 2017).
incarcerated and thrombosed internal haemorrhoids. • DRE may not be possible in patients with a fissure owing
• Refer all patients for an urgent 2-week appointment if to pain.
anal or colorectal cancer is suspected.
• Indications for routine referral to secondary care include: Management
• patients who do not respond to conservative treatment; • Advise patients increase the amount of fibre and fluid in
• third-degree or fourth-degree haemorrhoids that are their diet.
too large for nonoperative measures (haemorrhoidec- • Consider prescribing a bulking agent.
tomy may be needed); • Advise taking paracetamol and ibuprofen for analgesia.
• combined internal and external haemorrhoids with • Avoid opiate analgesia.
severe symptoms (surgery may be required); • Consider prescribing a topical anaesthetic for a few days.
• the presence of chronic irritation or leakage; and Avoid prolonged use as it can desensitise the anal skin.
• Advise that sitting in a shallow, warm bath several times • Inguinal hernias are almost always symptomatic.
a day (if possible, particularly after a bowel movement) • Asymptomatic hernias tend to become symptomatic. One
may help relieve pain. study has shown that 70% of patients managed with a
• Recommend the anal region is kept clean and dry. “watch and wait” strategy will eventually require surgery
• Almost half of patients who have an acute anal fissure within 5 years (Fitzgibbons et al., 2013).
will resolve their symptoms with non-operative measures
(Stewart et al., 2017). Investigation
• The gold standard for hernia diagnosis is clinical
Topical Medications examination.
• Anal fissures may be treated with topical nitrates. • The British Hernia Society (BHS) advises that diagnostic
• Consider prescribing topical glyceryl trinitrate (GTN) imaging should not be arranged at primary care level.
0.4% ointment. Advise the patient to use twice a day for • Dynamic ultrasound scan is recommended as the first
6 to 8 weeks. Consider prescribing a second course if the line investigation for patients with groin pain or swelling
fissure remains unhealed but there has been an improve- of an unclear origin (BHS, 2016).
ment in symptoms (NICE, 2017).
• The main side effect of topical GTN is headaches, which Management
often leads to patients stopping treatment. Headaches • The only cure for groin hernia is surgery.
with GTN have been observed in up to 70% of patients
in one paper (Berry, Barish, & Bhandari, 2013). Indications for Referral
• Topical GTN is associated with healing in approximately • All patients with an overt or suspected primary or recur-
50% of chronic anal fissures (Berry et al., 2013). rent inguinal or femoral hernia should be offered routine
• Topical 2% diltiazem is an alternative treatment for anal referral to secondary care.
fissure but is unlicensed. • Patients with strangulated or obstructed groin hernia
• GTN and diltiazem have been shown to be equally effec- should be emergency referrals.
tive in treating fissures (Sanei, Mahmoodieh, & Masoud- • Irreducible and partially reducible inguinal hernias, and
pour, 2009), although diltiazem is associated with fewer all groin hernias in women, should be urgent referrals.
side effects. • Patients with bilateral groin hernias should be referred
to a surgeon who performs both open and laparoscopic
Indications for Referral repair.
• Refer all patients for an urgent 2-week appointment if • Patients with recurrent inguinal or femoral hernias meeting
anal or colorectal cancer is suspected. referral criteria should be referred to a surgeon who per-
• Consider referral for examination under anaesthesia if forms both open and laparoscopic repair and where pos-
the diagnosis is unclear or if spasm and pain make diag- sible to the named surgeon who performed the first repair
nosis impossible. (providing the patient does not request otherwise).
• Refer patients who remain symptomatic and/or have an • Patients with multiple recurrent (more than one recur-
unhealed fissure despite adherence to conservative measures rence) groin hernias should be referred to a named surgeon
and topical agents. who has subspecialty interest in hernia repair and performs
• Consider referring patients whom you are considering both open and laparoscopic repair.
starting topical diltiazem. • Patients with minimally symptomatic inguinal hernias
• Refer with the presence of a fissure in an elderly patient. who have significant comorbidity and do not want to
• Further options for the management of anal fissure in have surgical repair (after appropriate information has
secondary care include injection of botulinum toxin and been provided) do not need to be referred (BHS, 2016).
lateral internal sphincterotomy.
Inguinal Hernia Vascular Problems

Varicose Veins
GUIDELINES
GUIDELINE
British Hernia Society (BHS) Commissioning Guide. (2016).
Groin hernia. https://www.rcseng.ac.uk/standards-and- National Institute of Health and Care Excellence. (2013).
research/commissioning/commissioning-guides/topics/. Varicose veins: Diagnosis and management. NICE clinical
HerniaSurge Group. (2018). International guidelines for guideline 168. www.nice.org.uk.
groin hernia management. https://www.ncbi.nlm.nih.gov/
pubmed/29330835.
• Varicose veins occur when the valves within the veins

• Groin hernia repair is one of the most commonly per- become incompetent allowing blood to reflux, which
formed operations in the world. results in tortuous, dilated veins.
• For some patients varicose veins are primarily a cosmetic • PAD is associated with the same risk factors as other
concern, but they can be complicated by discomfort, cardiovascular disease, including increasing age, smoking,
thrombophlebitis, deep vein thrombosis, bleeding, and obesity, diabetes mellitus, and hypercholesterolaemia.
skin changes (including lipodermatosclerosis, venous • Limb ischaemia may be acute or chronic.
eczema, and ulceration).
• For management of skin changes or ulcers in the context Diagnosis
of varicose veins, see Chapter 22. • Suspect acute ischaemia in a limb that has become painful
over a period of minutes, hours, or days that is pallid
Management with a loss of distal pulses. The limb may also be cold,
• If bleeding, then apply first aid and admit urgently. Bleed- cyanotic, with a loss or alteration of sensation and muscle
ing from varicose veins can sometimes be life threatening. power. This is a threat to limb viability and is thus an
• If not bleeding offer reassurance that complications are emergency. Admit immediately.
rare but warn about signs and symptoms of thrombosis, • Suspect chronic limb ischaemia if the patient reports the
bleeding, and ulceration. cramp-like pain on exercise typical of intermittent clau-
• Give lifestyle advice, including: dication. Pain is usually in the calf if there is narrowing
• weight loss; of the femoral or popliteal arteries. It is less commonly
• light or moderate exercise; and in the hip, thigh, or buttock if the common iliac artery
• avoiding prolonged periods of sitting and standing. is affected. Chronic limb ischaemia may also be indicated
• If referral is not indicated (see upcoming discussion) or by non healing wounds on the leg (including arterial
if the patient declines referral, consider compression stock- ulcers) or distal pulses are difficult to palpate.
ings, although note that NICE advises that compression • Measure the ankle brachial pressure index if PAD is suspected:
hosiery should only be offered if interventional treatment • ABPI <0.9 suggests PAD.
is thought to be inappropriate. • ABPI <0.5 suggests the patient is at risk of critical
• If considering compression hosiery: limb ischaemia. Refer urgently.
• assess whether the patient is able to use the hosiery • A value between 0.9 and 1.3 makes PAD less likely.
correctly; • A value >1.3 suggests arterial stiffening particularly in
• explain that class 2 stockings may be more effective patients with diabetes mellitus or renal failure.
than class 1 but are less well tolerated (see Chapter 22 • Note: NICE advises not to exclude PAD in patients with
for more detail); and diabetes mellitus based on a normal or raised ABPI alone.
• exclude underlying arterial disease, which is a contra-
indication to compression, with ankle brachial pressure Management
index (ABPI). • Offer treatment for secondary prevention of cardiovascular
disease, including:
Referral • smoking cessation;
• Many areas have specific referral criteria for varicose veins • advice on diet and exercise;
but NICE recommends that referral be considered for: • lipid modification with a statin;
• symptomatic veins; • management of hypertension;
• thrombophlebitis; and • management of diabetes; and
• skin changes, including active or healed ulceration. • antiplatelet therapy (NICE recommends clopidogrel
• Treatment options in secondary care include endothermal 75 mg once daily as first line).
ablation, endovenous laser treatment, ultrasound-guided • If available, offer a supervised exercise programme for
foam sclerotherapy, or surgery. patients with intermittent claudication. This involves 2
hours of supervised exercise per week for 3 months.
Peripheral Arterial Disease (PAD) Patients should be encouraged to exercise to the point of
maximum pain.
GUIDELINES • Unsupervised exercise may be recommended in suitable
patients with advice to patients to exercise for 30 minutes,
National Institute of Health and Care Excellence. (2012, three to five times each week, exercising until the onset
updated 2018). Peripheral arterial disease: Diagnosis and
management. NICE clinical guideline 147. www.nice.org.uk.
of symptoms and then resting until recovered.
NICE Clinical Knowledge Summaries. (2015). Peripheral • See Chapter 22 for management of arterial ulcers.
arterial disease. cks.nice.org.uk. • Consider referral to a vascular surgeon if the above men-
tioned measures are ineffective.
• Refer urgently if ABPI is below 0.5 due to the risk of
critical limb ischaemia.
• Peripheral arterial disease results from the narrowing or • Consider naftidrofuryl oxalate if the patient declines referral
occlusion of peripheral arteries due to atherosclerotic or is not appropriate for angioplasty or bypass surgery. Treat
disease. for 3 to 6 months and discontinue if there is no benefit.
References Gurusamy, K., Davidson, C., Gluud, C., & Davidson, B. (2013).
Early versus delayed laparoscopic cholecystectomy for people with
Association of Coloproctology of Great Britain and Ireland acute cholecystitis. The Cochrane Database of Systematic Reviews,
(ACPGBI). (2014). Commissioning guide: Rectal bleeding. https:// (6), CD005440.
www.acpgbi.org.uk//content/uploads/2017/02/Commissioning- HerniaSurge Group. (2018). International guidelines for groin hernia
guide-colonic-diverticular-disease-RCS-2014.pdf. management. Hernia: The Journal of Hernias and Abdominal Wall
Association of Coloproctology of Great Britain and Ireland (ACPGBI). Surgery, 22, 1–65.
(2017). Commissioning guide: Colonic diverticular disease. https:// Mäkelä, J., Klintrup, K., Takala, H., & Rautio, T. (2015). The role of
www.acpgbi.org.uk//content/uploads/2014/03/Commissioning- C-reactive protein in prediction of the severity of acute diverticulitis
Guide-for-Rectal-Bleeding-Dec-2017.pdf. in an emergency unit. Scandinavian Journal of Gastroenterology,
Astin, M., Griffin, T., Neal, R., et al. (2011). The diagnostic value 50, 536–541.
of symptoms for colorectal cancer in primary care: A systematic Mazeh, H., Mizrahi, I., Dior, U., et al. (2012). Role of antibiotic
review. British Journal of General Practice, 61, e231–e243. therapy in mild acute calculus cholecystitis: A prospective random-
Berry, S., Barish, C., & Bhandari, R. (2013). Nitroglycerin 0.4% oint- ized controlled trial. World Journal of Surgery, 36, 1750–1759.
ment vs placebo in the treatment of pain resulting from chronic National Institute for Health and Care Excellence (NICE).
anal fissure: A randomized, double-blind, placebo-controlled study. (2014). Gallstone disease: Diagnosis and management of choleli-
BMC Gastroenterology, 13, 106. thiasis, cholecystitis and choledocholithiasis. NICE clinical guide-
British Hernia Society. (2016). Commissioning guide: Groin hernia. line 188. https://www.nice.org.uk/guidance/cg188/evidence/
https://www.rcseng.ac.uk/standards-and-research/commissioning/ cg188-gallstone-disease-full-guideline3.
commissioning-guides/topics/. National Institute for Health and Care Excellence Clinical Knowl-
Davis, B., Lee-Kong, S., Migaly, J., et al. (2018). The American Society edge Summaries. (2016). Haemorrhoids. https://cks.nice.org.uk/
of Colon and Rectal Surgeons clinical practice guidelines for the man- haemorrhoids.
agement of hemorrhoids. https://www.fascrs.org/sites/default/files/ National Institute for Health and Care Excellence Clinical Knowledge
downloads/publication/cpg_management_of_hemorrhoids.pdf. Summaries. (2017). Diverticular disease. https://cks.nice.org.uk/
Chabok, A., Påhlman, L., Hjern, F., et al. (2012). Randomized clini- diverticular-disease#!rightTopic.
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Journal of Surgery, 99, 532–539. edge Summaries. (2017). Diverticular disease: Anal fissure. https://
Chou, S., Chen, S., Shyr, Y., & Wang, S. (2019). Polypoid lesions of cks.nice.org.uk/anal-fissure.
the gallbladder: Analysis of 1204 patients with long-term follow- Regenbogen, S., Hardiman, K., Hendren, S., & Morris, A. (2014).
up. Surgical Endoscopy, 31, 2776–2782. Surgery for diverticulitis in the 21st century: A systematic review.
European Association for the Study of the Liver (EASL). (2016). EASL JAMA Surgery, 149, 292–303.
clinical practice guidelines on the prevention, diagnosis and treatment Sanei, B., Mahmoodieh, M., & Masoudpour, H. (2009). Compari-
of gallstones. Journal of Hepatology, 65, 146–181. https://easl.eu/ son of topical glyceryl trinitrate with diltiazem ointment for the
publication/prevention-diagnosis-and-treatment-of-gallstones/. treatment of chronic anal fissure: A randomized clinical trial. Acta
Farthing, M., Roberts, S., Samuel, D., et al. (2014). Survey of digestive Chirurgica Belgica, 109, 727–730.
health across Europe: Final report. Part 1: The burden of gastro- Sartelli, M., Catena, F., Ansaloni, L., et al. (2016). WSES guidelines
intestinal diseases and the organisation and delivery of gastroen- for the management of acute left sided colonic diverticulitis in the
terology services across Europe. United European Gastroenterology emergency setting. World Journal of Emergency Surgery, 11, https://
Journal, 2, 539–543. www.ncbi.nlm.nih.gov/pmc/articles/PMC4966807/.
Fitzgibbons, R., Ramanan, B., Arya, S., et al. (2013). Long-term results Shahedi, K., Fuller, G., & Bolus, R. (2013). Long-term risk of acute
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Annals of Surgery, 258, 505–508. 1609–1613.
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Gurusamy, K., & Davidson, B. (2014). Gallstones. British Medical of_anal_fissures_jan_2017_dcr_issue.pdf.
Journal, 348, g2669.
11
Musculoskeletal Problems
JOHN MACLEAN
Osteoarthritis Exercises for the Shoulder
Management Strategy Flexibility
Education and Self-Management Strengthening the Rotator Cuff
Relatively Safe Pharmaceutical Options Tennis Elbow (Lateral Epicondylitis)
Adjunctive Nonpharmacologic, Nonsurgical Management Carpal Tunnel Syndrome
Adjunctive Pharmacologic Management De Quervain Tenosynovitis
Local Injections Ganglion
Disability Problems With the Lower Limbs
X-Rays Acute Knee Injury
Referrals to a Specialist Anterior Knee Pain
Postoperative Care Where There Is a Past History of Injury
Recurrence of Pain Where There Is No History of Injury
Antibiotic Prophylaxis Patients Still in Pain After 8 Weeks (With or Without History
of Injury)
Low Back Pain General Advice for Patellofemoral Pain
Aims of Management Knee Exercises
Managing Acute Mechanical Low Back Pain Steroid Injections at the Knee
Psychosocial Blocks to Recovery (Yellow Flags) Acute Ankle Injury
Management of Chronic Low Back Pain Rehabilitation After Ankle Injuries
Nerve Root Compression Heel Pain
Plantar Fasciitis
Referrals
Plantar Stretching for Plantar Fasciitis
Failure to Progress in Acute Low Back Pain
Achilles Tendonopathy
Failure to Progress With Neurological Symptoms or Signs, Achilles Tendon Rupture
and Other Serious Situations
Problems With the Feet
X-Rays
Bunions
Possible Serious Spinal Pathology
Hallux Rigidus
The Elderly With Acute Back Pain
Metatarsalgia
Advice/Exercises for Low Back Pain
Steroid Injections in Soft Tissue Lesions
Neck Pain
Indications
Mechanical Pain
Good Practice
Daily Stretching and Strengthening Exercises
Postinjection Advice
Radiculopathy
Contraindications to Corticosteroid Injection in Soft Tissue
Red Flags Lesions
Problems With the Upper Limbs Oral Nonsteroidal Antiinflammatory Drugs
Acute Shoulder Pain Which Oral Nonsteroidal Antiinflammatory Drug?
Acute Onset With Injury
Adverse Drug Reactions
Acute Onset Without Injury
Chronic Shoulder Pain Contraindications and Cautions
Simplified Guide to Cover Most Cases of Shoulder Pain Prescribing Practice
142
CHAPTER 11 Musculoskeletal Problems 143
Managing Nonsteroidal Antiinflammatory Drug-Induced Spondyloarthropathies

Gastrointestinal Complications Aims of Management
Uninvestigated Dyspepsia Diagnosis of Ankylosing Spondylitis
Classes of Nonsteroidal Antiinflammatory Drugs Diagnosis of Other Spondyloarthropathies
Monitoring Patients Taking Disease Modifying Antirheumatic Management
Drugs Referrals
Biologic Therapy Monitoring To a Rheumatologist
Gout To Other Specialities
Aims of Management Connective Tissue Diseases
Treatment of the Acute Attack Diagnosis
Management After an Attack Management
Prevention of Recurrence and Complications Osteoporosis
Prophylactic Drugs Definitions of Osteopenia and Osteoporosis
Allopurinol General Principles
Febuxostat Procedures Proposed in the Investigation of Osteoporosis
Indications for Referral Approach to Prognosis and Intervention
Rheumatoid Arthritis Clinical Risk Factors for Osteoporosis
Aims of Management General Management
General Pharmacological Intervention
Model of Care The Practicalities of Prescribing Bisphosphonates
Patient Assessment Corticosteroid-Induced Osteoporosis
Referral Polymyalgia Rheumatica
Prereferral Investigations Management Objectives
Patients With Poor Prognosis Diagnosis
Shared Care: Pharmacologic Treatment Investigations
Pain Control Initial Management
Steroids Maintenance Steroid Therapy
Disease Modifying Antirheumatic Drugs
Referral
The General Practitioner’s Role
Methotrexate Patient Education
Antidepressants Temporal Arteritis
Nondrug Management Diagnostic Criteria: American College of Rheumatology
Education Initial Management
Exercise Maintenance Treatment
Transcutaneous Electrical Nerve Stimulation, Heat, and Cold Steroids
Applications Fibromyalgia
Surgery Management
Alternative Therapies/Diet
Disability
Osteoarthritis • prevent progression of joint damage;

• reduce disability and improve function.
GUIDELINE
National Institute for Health and Care Excellence. (2014). Management Strategy
Osteoarthritis: Care and management. NICE clinical guideline
177. Available at www.nice.org.uk/Guidance/CG177.
• Make a holistic assessment of the impact of osteoarthritis
on the patient in terms of:
• function, occupation, leisure activities;
• quality of life;
• The aims of management are to: • mood and sleep;
• empower the patient in self-management; • relationships;
• control symptoms (mainly pain and stiffness); • comorbidities.
144 se c t i o n 2 Manual of clinical practice
TABLE
11.1 Management of Osteoarthritis
Core treatment These should be considered for all patients Education

Exercise
Weight loss for the overweight
Relatively safe pharmaceutic These should be considered before adjunctive Paracetamol
options treatments Topical nonsteroidal antiinflammatory drugs
Adjunctive treatments This refers to treatments which have less proof Pharmaceutical
of efficacy or involve more risk to the patient • Nonsteroidal antiinflammatory drugs
• Opioids
• Intraarticular steroids
• Capsaicin
Self-management
• Local heat and cold
• Assistive devices
Other nonpharmacologic
• Supports and braces
• Shock-absorbing shoes and insoles
• Transcutaneous electrical nerve stimulation
• Manual therapy
• Surgery
• Elicit the patient’s understanding of the illness and • Encourage weight loss in the overweight. This improves
concerns. function, though whether it reduces pain is not certain.
• Discuss the risks and benefits of treatment options.
• Formulate a management plan with the patient based on Relatively Safe Pharmaceutical Options
the above and taking account of the patient’s values and
preferences. • Recommend paracetamol. Regular doses, namely 1 g four
• Screen for depression which is more common in patients times daily, might be better than taking a dose as required.
with long-term disability. • For some joints, such as the knee, recommend topical
Management can be divided into three domains nonsteroidal anti-inflammatory drugs (NSAIDs) before
(Table 11.1). progressing to adjunctive drugs with their associated risks.
Education and Self-Management Adjunctive Nonpharmacologic,

Nonsurgical Management
• Address concerns and counter any misconceptions (e.g.,
that the disease inevitably progresses to joint replace- • Consider the following for pain relief:
ment). Patient-centred consultations result in better out- • Local application of heat or cold
comes and better use of resources (such as fewer unhelpful • Referral for manipulation or stretching
investigations). • Transcutaneous electrical nerve stimulation (TENS)
• Explain the disease in appropriate language and back this • Refer to the most appropriate agency according to local
up with an offer of written information. policies (e.g., physiotherapy or occupational therapy) for:
• assessment for bracing, joint supports, or insoles in
those with biomechanical joint pain or instability;
PATIENT INFORMATION
• assistive devices such as walking sticks.
Arthritis Research UK. Osteoarthritis: An information booklet. • Advise on footwear. Shock-absorbing shoes (trainers)
Available from www.arthritisresearchuk.org. and insoles (which can be purchased from pharmacies)
Arthritis Care. Living with osteoarthritis (information
booklet). Available from www.arthritiscare.org.uk/do-i-have- may help weightbearing joints.
arthritis/publications/223-living-with-osteoarthritis. • Advise the patient to keep active and to pace oneself, take
planned rest periods, and avoid bursts of overactivity.
• Look out for depression. If present, treating it not only
improves mood but results in less pain and improved
• Encourage exercise: function at 12 months (Lin et al., 2003).
• Local muscle strengthening • Recommend tricyclic antidepressants if sleep or mood
• General aerobic exercise disturbance is present.
• Refer to physiotherapy for exercise according to local • Do not recommend electroacupuncture. Consider the
policies. use of acupuncture when there are few options left.
Adjunctive Pharmacologic Management • referral for joint replacement is being considered;

• there is knee pain with locking: x-ray may show radi-
• Prescribe either a weak opioid or an oral NSAID (see opaque loose bodies;
NSAID section) depending on patient preferences and • there is diagnostic doubt.
comorbidities.
• Educate the patient in the self-management of analgesics so Referrals to a Specialist
that he or she can step up or down the scale of treatment:
• Recommend that NSAIDs be used for the shortest • Consider referral for joint replacement surgery when a
period possible. patient experiences joint symptoms, whether pain, stiff-
• Explain that paracetamol or co-codamol can be used ness, or reduced function, that impact substantially on
with ibuprofen, but that co-codamol should never his or her quality of life and have not responded to core
be taken with paracetamol because it contains the and adjunctive treatments. Referral is best made before
same drug. prolonged functional limitation and severe pain become
• Ensure the patient is not on more than one NSAID, established.
including over-the-counter (OTC) preparations (e.g., • Follow local referral thresholds, which vary, but age,
aspirin). gender, smoking, obesity, and comorbidities should not
• Consider intraarticular steroid injections. be barriers to referral.
• Consider topical capsaicin.
• Do not prescribe glucosamine or chondroitin (Towheed Postoperative Care
et al., 2005).
Patients will occasionally ask their general practitioner (GP)
Local Injections about their return to normal activities:
• Advise the patient who has had a hip replacement not to
• Intraarticular corticosteroid injections provide short- cross the legs or drive for 6 weeks. The danger of disloca-
term pain relief and may give patients the opportunity tion is greatest in the first 6 weeks and occurs when the
to begin other interventions such as exercise. hip is flexed, especially if internally rotated and adducted.
• Intraarticular hyaluronic acid injections have been shown • Advise patients that they may resume activities, includ-
to have only a weak effect. ing walking, swimming, bicycling, and tennis, after 6
• Consider a steroid injection for moderate to severe pain weeks, provided they take care not to fall. Contact sports
and for flareups. and use of ladders are not advised.
• Do not refer for intraarticular hyaluronic acid injections
for knees. Recurrence of Pain
• Arrange for an x-ray, erythrocyte sedimentation rate
Disability (ESR), and white blood cell (WBC) count in any patient
with a hip prosthesis who develops pain. This may show
• Consider the patient’s need for aids (e.g., bath aids) and loosening, infection, stress fracture, or dislocation. Refer
refer as appropriate. any of these to the surgeon urgently. Refer anyway if the
• Consider eligibility for benefits (e.g., Attendance Allow- pain does not settle with rest. Any of the above may be
ance, a disabled driver’s badge) and refer to the appropri- present despite a normal x-ray.
ate agency for advice.
• Consider the need for support and rehabilitation. Refer, Antibiotic Prophylaxis
according to local circumstances, to social services, a reha- Patients with prosthetic joint implants including total hip
bilitation team, or hospital-based multidisciplinary team. replacements do not need antibiotic prophylaxis for dental
treatment (Uçkay, 2008).
X-Rays
Low Back Pain
• Plain x-rays are not routinely indicated. They cannot
confirm the diagnosis because degenerative changes in GUIDELINE
several joints (e.g., spine and knees) start in middle age
National Institute for Health and Care Excellence. (2016). Low
and x-ray appearances correlate poorly with the symp- back pain and sciatica in over 16s: Assessment and
toms (Royal College of Radiologists, 2003). Diagnosis is management. NICE clinical guideline 59. Available from www.
mainly clinical in many syndromes and plain x-rays do nice.org.uk/guidance/NG59.
not differentiate between disease and nondisease (e.g.,
shoulder impingement syndrome).
• Refer for x-ray when: • Low back pain (LBP) can be defined as:
• soft tissue calcification is suspected (e.g., shoulder) • acute LBP: duration of 6 weeks or less
and when the result might inform the choice to use • subacute LBP: duration of 6 to 12 weeks
an NSAID or steroid injection; • chronic LBP: duration of 3 months or more
• LBP is extremely common. At any one time, 15% of • If not improving, advise graded return to normal
adults have it and 60% will have it some time in their activity and set a return date to work.
lives (Mason, 1994). Back pain is the most common • If not improving, also consider referral to another
cause of long-term sickness: 52 million working days are health professional with expertise in LBP, usually a
lost each year from back pain. physiotherapist, or possibly a manipulative therapist.
• Mechanical LBP is self-limiting: 80% to 90% of patients • Reassess for red flags (to come).
recover spontaneously within 3 months. • Reassess for yellow flags (to come). If present, be posi-
• At presentation triage patients into one of the three fol- tive, schedule regular reviews, encourage a programme
lowing groups based on history and examination: of activity, and consider early referral.
• Mechanical back pain • If there has been a failure to progress at 6 weeks then
• Nerve root compression continue and intensify current management and con-
• Possible serious spinal pathology—look for red flags sider referral to specialist services.
(see upcoming discussion) and act accordingly
• Note any psychological or social influences that might Psychosocial Blocks to Recovery
retard recovery: so-called yellow flags. (Yellow Flags)
Aims of Management • Yellow flags include:
• a belief that back pain is harmful or potentially
• Reduce pain and the length of sickness in mechanical disabling;
back pain. • fear-avoidance behaviour (avoiding a movement or
• Identify early the small minority with serious pathology activity due to misplaced anticipation of pain) and
needing immediate or urgent attention. reduced activity levels;
• Prevent acute back pain becoming chronic. • a tendency to low mood and withdrawal from social
interaction; an expectation that passive treatments
Managing Acute Mechanical Low rather than active participation will help;
Back Pain • awaiting compensation;
• a history of absence from work for back pain or other
• Features of mechanical back pain include: problems;
• presentation at age 20 to 55; • poor job satisfaction, long hours, heavy work;
• pain felt over lumbosacral area, buttocks, or thighs; • an overprotective family or a lack of support at home.
• pain alters with posture and activity; • Note that these yellow flags should not be used pejoratively.
• the patient is well. They are a guide to those patients in whom early interven-
• Take time to listen and examine, explain, and reassure. tion and early return to work are especially important.
These have beneficial effect on pain and anxiety.
• Encourage continued activity, including work if appro- Management of Chronic Low Back Pain
priate. Bedrest worsens outcome and should only be
taken if pain is severe, and then for no longer than neces- • The prevalence of LBP is not increasing but the level of
sary (Waddell, Feder, & Lewis, 1997). disability and claims for long-term sickness benefits are.
• Prescribe analgesics or NSAIDs. The management of chronic LBP therefore encompasses
• Avoid prescribing muscle relaxants (e.g., diazepam). several approaches:
They control pain, but the abuse potential and the avail- • Appropriate referral for physical treatments (see
ability of other analgesic options means that it should upcoming discussion)
not be common practice. • Principles of chronic pain management
• Ensure the patient has a medium-firm mattress (5 on the • Attention to psychosocial issues
European Committee for Standardisation scale). This has • Recommend regular physical exercise. This should start as
been shown to be more effective than a firm one (Kovacs soon as possible in the course of the illness. A light mobi-
et al., 2003). lization programme for patients who have had back pain
• Demonstrate stretching exercises or give written instruc- for 8 to 12 weeks has been shown to improve outcomes
tions (e.g., “Back Pain” booklet from the Association for initially and at 3 years (Hagen, Eriksen, & Ursin, 2000).
Real Change [ARC] is available from www.arc.org.uk). • Treat underlying anxiety or depression if present.
Many exercises are available and some have fanatical • Consider prescribing a tricyclic antidepressant at low doses,
supporters, but there is no evidence that any specific type even if depression is not present (Staiger et al., 2003).
of exercise is best (Tulder et al., 2000). • Explore work-related issues. If return to former employ-
• Offer a follow-up consultation after 2 to 6 weeks: ment is unlikely, advise the patient to see the disability
• If activity and function are improving, encourage employment adviser at the job centre.
return to normal activities even if pain is still present. • Consider referral to a multidisciplinary team; this can be
Stress that activity will not damage the back. effective in resistant cases (Guzmán et al., 2001).
Nerve Root Compression • There is insufficient evidence for physical agents and
passive modalities (e.g., ice, heat, short-wave diathermy,
• Mechanical back pain can be referred down the leg so massage, ultrasound, TENS, acupuncture), but they
not all sciatica arises from a prolapsed disc; nerve root remain popular.
compression is more likely if pain extends down to the
foot. Conversely, not all prolapsed discs cause sciatica Failure to Progress With Neurological
since they can be demonstrated in 50% of asymptomatic Symptoms or Signs, and Other Serious
adults. Situations
• Sciatica arising from a prolapsed disc that causes signifi-
cant disability has a lifetime prevalence of 5%. • Evidence of benefit from discectomy, which may be long
• Recognizing nerve root compression is important because term, is accumulating for patients with disc herniation,
surgery leads to quicker recovery than natural resolution, spinal stenosis, and spondylolisthesis (Gibson, 2007).
although there may be no difference in long-term out- • Sciatica. The ideal timing of referral to an orthopaedic
comes (Peul et al., 2007). surgeon of patients with sciatica which is not improving is
• Chymopapain (discolysis) is also effective but not widely not clear. A British Medical Journal (BMJ) leading article
used in the United Kingdom. suggests 8 weeks (Fairbank, 2008); patients with severe pain
• At presentation, test for limitation of straight leg raising. will be referred earlier.
If straight leg raising is not impaired and pain does not • Cauda equina syndrome (see upcoming discussion). Refer
radiate below the knee then nerve root compression is immediately to an orthopaedic surgeon.
very unlikely (Vroomen, de Krom, & Knotterus, 1999). • Other red flags. Refer urgently where appropriate
• Look for neurological deficit from L5/S1 compression: and, if a malignancy is suspected, refer urgently accord-
loss of sensation over the lateral border of the lower leg ing to the recommendations of the Department of
and foot, weakness of dorsiflexion and plantarflexion of Health (DOH, 2000).
foot, and impairment of the ankle reflex. In L3/4 com-
pression the knee reflex may be impaired. X-Rays
• Manage sciatica as you would acute LBP.
• Refer patients whose pain is not settling (the timescale • Explain to patients with mechanical LBP why x-rays are
will depend on local circumstances) or where there is unhelpful: Degenerative changes are common; most dis-
increasing neurological deficit. orders are of soft tissue, which cannot be shown on x-ray;
and the dose of radiation is high (60 times that of a chest
Referrals x-ray) (Royal College of Radiologists, 2003).
• Consider x-rays in the presence of red flags, alongside
Whom to refer to will depend on local availability and agreed urgent referral.
care pathways: physiotherapist, pain clinic, multidisciplinary
team, orthopaedic physicians, or orthopaedic surgeons. Possible Serious Spinal Pathology
Failure to Progress in Acute Low Back Pain • Red flags include:

• cauda equina syndrome (features include urinary
• All guidelines are consistent in recommending early retention, bilateral neurological symptoms and signs,
referral (≤2 weeks) for physiotherapy or manipulation saddle anaesthesia—urgent referral is indicated);
to prevent acute LBP becoming chronic. However, there • significant trauma (risk of fracture);
is conflicting evidence about whether this makes a dif- • weight loss (suggestive of cancer);
ference to long-term outcome, although a large UK trial • history of cancer (suggestive of metastases);
shows evidence of modest benefit at 3 and 12 months • fever (suggestive of infection);
from manipulation (UK BEAM Trial Team, 2004). This • intravenous drug use (suggestive of infection);
held whether the manipulation was in National Health • steroid use (risk of osteoporotic collapse);
Service (NHS) or private facilities. Conversely, it is now • patient aged over 50 years (cancer unlikely below
clear that, even in sciatica, epidural corticosteroid injec- this age);
tion offers no long-term benefit, although there may be • severe, unremitting nighttime pain (suggestive of
transient benefit at 3 weeks (Arden et al., 2005). cancer);
• Be prepared (Hazard et al., 1997) to discuss manipula- • pain that gets worse when patient is lying down (sug-
tion; many patients take themselves to osteopaths or gestive of cancer).
chiropractors and many physiotherapists practice a
related treatment, mobilization. The evidence for manip- The Elderly With Acute Back Pain
ulation is of low quality but it may lead to quicker
recovery in the first few weeks without making any dif- These patients are a special case because osteoporotic col-
ference to long-term outcomes (Koes et al., 1996). lapse and malignancy are more likely and they need an early
x-ray. Half will have definite abnormality, and 10% will Neck Pain
have a malignancy (Frank, 1993).
• The principles of management of low back pain apply to
Advice/Exercises for Low Back Pain neck pain (Nachemson & Jonsson, 2000).
• Triage neck pain into mechanical, nerve root compres-
The following is taken from the BackCare leaflet “Exercise sion (often termed radiculopathy), and potential serious
for a Better Back” and from the ARC leaflet “Back Pain” pathology (red flags).
(see upcoming discussion). • In mechanical neck pain, keeping active is more effective
• Pay attention to sitting, lifting, and bending to avoid than immobilization.
aggravating pain and to prevent recurrence. • Similar medication may be prescribed.
• Sitting: Avoid prolonged sitting. A rolled towel in the • Psychosocial factors are important (see previous
small of the back may ease pain. discussion).
• Lifting: Always lift with a straight back and bent • Imaging is not routinely indicated.
knees. • Manipulation or mobilization may be effective in the
• Bending: Avoid spending too long at a task that short term (Gross et al., 2004).
requires bending (e.g., ironing). • Acupuncture is not effective (Trinh et al., 2006).
• Exercise regularly.
• Stretching exercises to maintain flexibility—daily. You Mechanical Pain
may start these early on in an attack and should keep
them up long term. • Recognize by:
• Muscle strengthening exercises—daily. You should • aged 18 to 55;
start these as your pain improves and keep them up • absence of signs of radiculopathy or red flags;
long term. • made worse by posture/activity.
• Take up aerobic exercise for general fitness (e.g., • Advise the patient to stay active (Aker et al., 1996).
swimming). Collars should only be worn for a limited period.
• Suitable exercises (there are many others): • Give regular analgesia.
• Starting position: on all fours with hands shoulder • Advise or refer for exercises (Kay et al., 2005).
width apart, arms and thighs vertical: • Treat hyperextension injuries (including whiplash inju-
1. Arch the back and look down. Then lower the ries) in the same way but avoid a collar as this prolongs
stomach toward the floor, hollowing the back. symptoms.
2. Slowly walk the hands around to one side,
back to the starting position, then around to the Daily Stretching and Strengthening Exercises
other side.
3. Raise one hand off the floor, reach underneath Advise the patient to hold the neck for 10 seconds in each
your body as far as you can. On the return, swing of the six positions (right and left lateral flexion and rota-
the arm out to the side as far as you can then return tion, flexion and extension) within the pain-free range.
to the starting position. Follow the moving hand Repeat 10 times. (Based on the ARC information leaflet
with the eyes. Repeat with the other arm. “Pain in the Neck.”)
4. Draw alternate knees to the opposite elbow.
5. Stretch one arm forward in front, at the same time Radiculopathy
stretching the opposite leg out behind. Repeat on
the other side. • Distinguish radiculopathy from neck pain that is referred
6. Swing the seat from side to side. to the shoulder and arm.
All exercises should be repeated 10 times. Exercises that • Look for the following signs, though their sensitivity and
hurt should be set aside and returned to with fewer repeti- specificity have not been adequately assessed:
tions on another occasion. • Diminished reflexes (triceps, biceps, supinator)
• Diminished power
• Diminished sensation along dermatomes
• Axial compression test: Extend the neck and rotate
PATIENT INFORMATION the head to the side of the pain, then apply pressure
to the head. Reproducing pain or paraesthesiae in the
ARC. Back pain. Available from www.arc.org.uk (search for
“Back pain”). arm signifies likely radiculopathy.
BackCare, 16 Elmtree Road, Teddington, Middlesex, • Treat as for mechanical neck pain but refer if there is no
TW11 8ST, tel. 020 8977 5474. Has several leaflets on improvement after 4 weeks. Refer earlier if there is loss
aspects of neck and back pain (e.g., surgery, exercises), of power and refer immediately if deterioration is rapid.
which are also available for download from www.backcare. However, warn the patient that surgery is no better than
org.uk.
conservative treatment except in severe cases (Kadanka
et al., 2002).
Red Flags Acute Onset Without Injury

• Red flags for malignancy, infection, and inflammation • If there is a sudden onset of desperate pain then refer to acci-
are as for LBP. dent and emergency (A&E) department. This may indicate
• A red flag specific for neck pain is evidence of cervical acute decalcification of an area of calcification in the rotator
myelopathy (the equivalent of the cauda equina syndrome). cuff which would need urgent decompression.
Neck pain may be absent but the syndrome should be • If the situation is less desperate then x-ray:
suspected when any of the following features are present: • If normal, give local anaesthetic and steroid injection
• Sensory disturbances in upper and lower limbs and advice on cuff strengthening exercises (or refer to
• Weakness in upper and lower limbs physiotherapy for this advice). If that fails, refer to
• Clumsiness and gait disturbance outpatient department (OPD). If it helps substan-
• Spasticity of lower limbs (upper limbs may be normal, tially but is followed by relapse give further injections
spastic, or flaccid) with a maximum of three per year.
• Increased tendon reflexes • If abnormal, refer according to diagnosis (see upcom-
• Lhermitte sign: paraesthesiae in limbs on neck flexion ing discussion).
indicates neck instability and warrants immediate
admission. Arthritis of Acromioclavicular Joint or
• Refer patients with one or more red flags urgently for a Glenohumeral Joint
specialist opinion. • If symptoms are mild then give analgesia, an NSAID,
heat, shoulder exercises, or refer to physiotherapy.
PATIENT INFORMATION • If symptoms are more severe then support as above and
Arthritis Research UK. Pain in the neck: An information refer to OPD.
booklet. Available at www.arthritisresearchuk.org.
Calcification in the Rotator Cuff
• Refer to OPD. They are more likely to need surgery at
Problems With the Upper Limbs some stage. They may benefit from a subacromial injec-
tion of local anaesthetic and steroid meanwhile.
Acute Shoulder Pain
Red Flags for Systemic Disease
REVIEW • Investigate further if the patient is systemically unwell
Mitchell, C., Adebajo, A., Hay, E., & Carr, A. (2005). Shoulder (e.g., with fever or weight loss), has a history of cancer,
pain: Diagnosis and management in primary care. British has arthritis elsewhere, has a mass or neurological symp-
Medical Journal, 331, 1124–1128.
toms or signs in that arm.
Chronic Shoulder Pain
Acute Onset With Injury Is It Acromioclavicular Joint Pain?
• Clinical fracture or dislocation: send to the emergency • Characteristics:
department. • Pain is localized to the acromioclavicular joint
• If there is no clinical fracture or dislocation then x-ray (ACJ).
and send to the emergency department if a fracture or • The joint may be swollen and tender with crepitus.
dislocation is shown. • Abduction is painful in the final 20 degrees.
• If the x-ray is normal: control pain for 3 weeks with • Inject local anaesthetic and steroid and give analgesia. If
analgesia and a sling. not better in 2 weeks, refer to physiotherapy. If still no
• If pain or loss of movement continues beyond 3 weeks better, check the diagnosis and refer.
consider performing the local anaesthetic (LA) test if you
feel confident to do so (see below). Is It a Rotator Cuff Lesion?
• If pain and movement are both improved refer for • Characteristics:
physiotherapy. • Pain is usually felt at the deltoid insertion.
• If pain is improved but not movement refer urgently • Any or all movements are painful.
to an upper limb specialist (fracture clinic) as sus- • Active movements are more painful than passive
pected rotator cuff tear. Repair, if needed, should be movements.
performed within 6 to 8 weeks of the injury. • Pain is felt on resisted active elevation (this is the
• The LA test helps to distinguish pain from a tear as the impingement sign).
cause of an inability to raise the arm. Instill 2 mL of local • If pain is mild then give analgesia and advice on cuff
anaesthetic (e.g., l% lidocaine) anterolaterally into the strengthening exercises. If no improvement after 3 weeks
subacromial bursa. Pain should be relieved within 5 refer to physiotherapy (Green, Buchbinder, & Hetrick,
minutes. If indicated, steroid (e.g., 40 to 80 mg Depo- 2003). If physiotherapy is unhelpful give a subacromial
Medrone) can be given through the same needle. injection of LA. Subacromial injection of steroid has
been shown to be no better than the same injection into • Note: Nerve root compression in the neck frequently pre
the buttock (Koes, 2009). sents as shoulder pain, coexists with it, and can cause it!
• If pain is moderate or severe consider performing the LA test. • Note: Young sports people with shoulder pain frequently
• If the pain is improved and power is full, refer to have occult instability; refer them readily.
physiotherapy. If still unresolved, refer.
• If pain is improved but there is weakness, refer Simplified Guide to Cover Most Cases of
urgently as a suspected rotator cuff tear. Shoulder Pain
• If pain is not improved, refer.
• If pain and power improve substantially but return, give • If the patient is unwell or has other disorders that could
further injections up to a maximum of three per year. cause shoulder pain, investigate further.
• If the pain is acute and the patient cannot raise the arm
Is It Chronic Capsulitis (Frozen Shoulder)? actively but you can raise it passively, refer urgently as a
• Characteristics: suspected rotator cuff tear.
• There is tenderness anteriorly between the coracoid • If there is a history of partial or complete dislocation,
process and the head of the humerus. refer for assessment of instability.
• All movements are painful and restricted, whether • Otherwise, decide between:
active or passive, but there is less pain on resisted • acromioclavicular joint pain (tender over the ACJ
active movement (when the joint does not move). with restriction of moving the arm across the front
• Characteristically, the loss of external rotation is the of the chest);
most marked, there is no crepitus, and no arthritis • GHJ pain (active and passive movements of the
elsewhere. shoulder painful and restricted).
• Explain the nature of the condition and the fact that • In both those conditions recommend:
spontaneous resolution can be expected, with a mean • analgesia and rest until the acute pain eases;
duration of 30 months. However, explain that some • exercises within the limits of pain to keep the joint
long-term restriction of movement is usual (Dias, Cutts, mobile;
& Massoud, 2005). • the ARC booklet (see upcoming discussion) and
• Order mobilization physiotherapy, designed to keep explain the likely outcome; and possibly steroid/local
the shoulder mobile within the limits of pain (Green anaesthetic injection into the appropriate joint.
et al., 2003).
• At the same time, give a single steroid injection into the
Exercises for the Shoulder
glenohumeral joint (GHJ). The earlier the injection is
given, the more effective it is (Dias et al., 2005). Flexibility
• Refer refractory cases that have not resolved after 30 • Demonstrate pendular exercises:
months for consideration of manipulation under anaes- 1. Stand up and lean forward with arm of affected side
thesia or arthroscopic release. hanging perpendicularly.
2. Sweep the arm around in a circle within the pain-free
Is It Glenohumeral Joint Arthrosis? range. Do this 20 to 30 times.
• The characteristics are the same as in chronic capsulitis, but 3. As time goes on, increase the range of the movement
crepitus may be felt and there may be arthritis elsewhere. and length of time.
• Confirm with an x-ray. Management depends on both
the degree of pain and functional loss: Strengthening the Rotator Cuff
• If mild, give analgesia and advice on exercises. 1. Hold your arm outstretched at shoulder height and out
• If moderate or severe, refer with a view to replacement to the side.
surgery. 2. Bring your arm forward then up, push to the back then
down to the starting position.
Is the Glenohumeral Joint Unstable? 3. Repeat 10 times. As time goes on, build up to doing
• Characteristics: three sets of 10 repetitions.
• There may be a history of subluxation or dislocation.
• There are two tests for this: PATIENT INFORMATION
1. Fix the shoulder girdle with one hand and, with Arthritis Research UK. Shoulder pain. Available from www.
the other, try to rock the head of the humerus arthritisresearchuk.org.
backward and forward in the glenoid fossa.
2. Hold the arm abducted to 90 degrees with the
upper arm pointing forward. Does external rota- Tennis Elbow (Lateral Epicondylitis)
tion cause apprehension and pain?
• Refer for consideration of specialist physiotherapy or • A randomized trial has now clarified the benefits of
surgery. therapy (Bisset et al., 2006).
• Recommend that the patient avoid or reduce the provok- • Consider aspiration with a wide bore needle under local
ing action. They should not be attempted to resume the anaesthesia.
activity until the pain has gone. • Refer the following if they are painful:
• Recommend an analgesic or NSAID either topically or • Dorsal wrist ganglia (over the scapholunate ligament).
orally. There is evidence for short-term but not long-term Recurrence after surgery is 5% to 10%.
benefit. • Palmar digital ganglia (from the flexor tendon sheath
• Explain that waiting for recovery is as effective as any other at the base of the finger). Recurrence after surgery
approach, with 89% of patients recovered within 1 year. is rare.
Refer for a brace if the pain is troublesome enough to • Try to avoid referring palmar wrist ganglia. Recurrence
warrant it. after surgery is 45%.
• Consider referral to physiotherapy for manipulation and
exercises: in the short term it is superior to a wait-and-see
approach although at 1 year it shows no benefit. Problems With the Lower Limbs
• Do not offer corticosteroid injection. Despite some benefit Acute Knee Injury
at 6 weeks the relapse rate is high (72%) and the results
at 1 year are worse than physiotherapy and worse even • The Ottawa Knee Rules reduce the need for an x-ray
than a wait-and-see approach. after acute knee injury by 26%. They are as reliable in
• Consider referral for surgery if the patient’s life is suffi- children as in adults (Bulloch et al., 2003). Patients with
ciently disturbed 1 year after onset. There is some evi- major trauma are not suitable candidates for the use of the
dence for benefit (Verhaar et al., 1993). decision rule.
• The economic impact of adopting this approach could
Carpal Tunnel Syndrome be a cost saving to the NHS of nearly £2 million (Nichols
et al., 1999).
PATIENT EDUCATION
• The rules state that a knee x-ray series is only required
for knee injury patients with any of these findings:
Ashworth, N. (2014). Carpal tunnel syndrome. Retrieved from • Age 55 or older
www.clinicalevidence.com (search “carpal tunnel”).
• Isolated tenderness of the patella (i.e., no bone tender-
ness of the knee other than the patella)
• Tenderness at the head of the fibula
• Advise the patient to rest the joint if possible and avoid • Inability to flex to 90 degrees
provoking activities. • Inability to bear weight both immediately and when
• Advise the patient to try a night splint (Futuro, Ace) to examined (they should be able to take four steps [i.e.,
reduce the flexion of the joint. take their weight on each leg twice even if they limp])
• Do not give diuretics. Although commonly prescribed,
there is no evidence that they are of value. If There Is No Fracture
• Refer for steroid injection or surgical treatment, if: • Mobilize as soon as pain permits.
• there is thenar wasting or constant sensory impair- • Encourage quadriceps exercises (see upcoming discussion).
ment; or • Provide suitable analgesia.
• symptoms are severe; or
• there has been no improvement after 3 months. Surgi-
Anterior Knee Pain
cal decompression is more effective than splinting
(Verdugo et al., 2003). Where There Is a Past History of Injury
• Consider oral steroids while waiting for the appoint- • Refer to the next fracture clinic if pain and effusion have
ment. There is evidence of benefit after 2 weeks. persisted for more than 2 weeks.
• If no grounds for immediate referral, arrange an
De Quervain Tenosynovitis x-ray. Request anteroposterior (AP), lateral, and skyline
views.
• Consider injection of steroid into the tendon sheath, • If the x-ray is abnormal, refer to the next fracture clinic.
although accurate placing of the needle is difficult. This applies whether the fracture is of the patella or a
flake of bone from an osteochondral fracture.
Ganglion • If the x-ray is normal, refer to orthopaedic outpatients if
there is a good history of patella dislocation or the patella
• Explain to the patient that the lesion is harmless, that 40% is unstable on examination.
resolve spontaneously, and that surgery has its problems: • If neither of these applies, give advice (see upcoming
• Excision does not always relieve pain. discussion) and review after 8 weeks. Most young patients
• Recurrence after surgery is common. with anterior knee pain will have improved spontane-
• The scar may be unsightly. ously in that time (Heintjes et al., 2003a).
TABLE Reliability of the Ottawa Ankle Rules for

Where There Is No History of Injury 11.2 Identifying Ankle and Foot Fractures or
• Examine for gross abnormalities that might warrant Avulsion Fractures
immediate attention.
Specificity
• Reassure the patient if it is Osgood-Schlatter disease (see
Patient Sensitivity (Interquartile
upcoming discussion). Otherwise advise as below.
Groups (95% CI) Range) LR
Patients Still in Pain After 8 Weeks (With or All patients 96% 26% 0.10
Without History of Injury) (94%–99%) (19%–34%)
• Arrange an x-ray, if not already done. Ankle injuries 98% 40% 0.08
• If abnormal, refer to OPD for assessment. (96%–99%) (30%–48%)
• If normal, refer for physiotherapy. If still no progress,
Midfoot injuries 99% 38% 0.08
refer to OPD for assessment. Advise the patient that (97%–100%) (25%–70%)
it may mean specialist physiotherapy or a brace rather
than surgery. LR, the likelihood ratio for a negative result.
General Advice for Patellofemoral Pain

1. Avoid provoking activities (e.g., stairs, walking up and
down hill, the breaststroke kick, skiing, cycling, exercise
bikes, and high impact aerobics). • An ankle x-ray is required if there is any pain in the mal-
2. Recommend quads exercises (see upcoming discussion) leolar zone and:
and hamstring stretching exercises. There is some evi- • there is bone tenderness at the posterior edge or tip
dence that exercise may reduce anterior knee pain of the lateral malleolus; or
(Heintjes et al., 2003b). • there is bone tenderness at the posterior edge of the
3. Recommend simple analgesics. NSAIDs may reduce medial malleolus; or
pain in the short term but not after 3 months. • the patient is unable to weight bear both at injury and
4. Recommend a support bandage. when seen.
• A foot x-ray is required if there is pain in the midfoot
Knee Exercises zone and:
• Stretching the hamstrings: • there is bone tenderness at the navicular; or
1. Stand up with the affected leg slightly in front. • there is bone tenderness at the base of the fifth meta-
2. Place both palms over the knee cap and push toward tarsal; or
the ground. Keep this position for 30 seconds. • the patient is unable to weight bear both at injury and
3. Repeat four times. As time goes on, try to increase the when seen.
stretch by pointing your toes upward. • The immediate treatment of an ankle sprain (as for any
• Strengthening the quadriceps: injured joint or limb) is rest, ice, compression, elevation
1. Lying down on your back, lift the leg with the knee (RICE). Of these, compression appears to be the most
straight to a position about 45 degrees off the horizontal. important and, with elevation, must be maintained for
2. Hold this position for a count of 10 seconds. at least 48 hours (Smith, 2003). Ice should be applied
3. Lower the leg, then rest. no more than 20 minutes at a time, three times a day;
4. Repeat 10 times. As time goes on, build up to doing and the skin should be separated from the ice by a wet
three sets of 10 repetitions. towel (Institute for Clinical Systems Improvement [ICSI],
2003). It is an approach based on experience rather than
Steroid Injections at the Knee evidence.
• Consider injections for: • Analgesia, support with mobilization, immobiliza-
• anserine bursitis; tion, and surgical repair are all used in inversion
• the knee joint; injuries of the ankle. There is no robust evidence to
• the medial ligament of the knee. guide the clinician in their use although the use of
support and early mobilization seems to result in faster
Acute Ankle Injury recovery and better long-term outcomes (Kerkhoffs
et al., 2002).
• The Ottawa Ankle Rule reduces the need for x-rays fol-
lowing ankle injury by 30% to 40% (Table 11.2) (Bach- Rehabilitation After Ankle Injuries
mann et al., 2003). Note that the rule is very good at • Recommend active mobilization to restore propriocep-
identifying patients who do not need an x-ray (high tion. This can be achieved by regular exercises:
sensitivity). It is poor at identifying those who have a • Imagine writing the alphabet with the foot, first capi-
fracture (low specificity). tals then small letters.
• Balance on the injured leg while moving the free leg

forward and backward and side to side; initially with Achilles Tendon Rupture
eyes open then with eyes shut. • There may be a history of a sharp snap felt in the tendon
• Use a wobble board. on exertion or on injury (e.g., slipping off a ladder). Up
to 20% of ruptures are missed (Maffulli, 1999).
• Consider the possibility of rupture in those with an acute
Heel Pain history (as above) and all those who have a longer standing
Plantar Fasciitis Achilles swelling or ankle injury that is slow to resolve.
• Characteristics include: • Diagnose rupture by lying the patient face down with
• isolated plantar heel pain on initiation of weight bearing the feet over the end of the couch. Squeeze the calf
either in the morning on rising or after a period of sitting; firmly. If the tendon is intact this will cause plantar
• pain tends to decrease after a while but increases as flexion of the foot. If the tendon is ruptured there will
time on the feet increases. be, at most, a small flicker of the foot (Thompson test).
• Examine to confirm heel tenderness and to check the • Refer any patient with a suspected rupture to be seen
range of movements. There may be associated tightness within 24 hours.
of the Achilles tendon.
• Do not x-ray. The presence of a calcaneal spur does not
alter treatment. Problems With the Feet
• Advise the patient about weight reduction, if appropri- Bunions
ate, and the use of cushioned shoes.
• Give analgesics. There is no evidence that NSAIDs are • Although a percentage of bunions are inherited by an
more effective than simple analgesics. autosomal dominant gene this is of variable penetrance
• Advise the patient about stretching exercises (plantar and hence not a reason for early referral or assessment.
fascia and Achilles tendon) although there is no evidence • Examine to exclude heel valgus deformity and flatfoot
of effectiveness (Digiovanni et al., 2003). and refer to a podiatrist if either is found. Orthoses may
• Refer to a podiatrist if there is no improvement after help reduce pain (Ferrari, 2003). While awaiting the
6 weeks. appointment teach the patient calf and foot exercises.
• The benefit of steroid injection is likely to be tem- • Refer only those whose lives are severely affected by the
porary, if any, and not worth the possible harms bunion. They should be aware that:
(Crawford, 2004). There is evidence of benefit from • after the operation they will not be able to wear a shoe
shock-wave application if it is available (Rompe for 8 weeks; and
et al., 2003). • recovery of function will take at least 6 months.
Plantar Stretching for Plantar Fasciitis Hallux Rigidus

1. Sit with the affected foot crossed over the other knee. • Advise the patient to wear a shoe with a rigid sole or to
2. Grasp the toes and pull toward the shin until the plantar insert a rigid insole into the shoe.
fascia is stretched. • Refer if pain is interfering with work or sleep. Advise the
3. Hold each stretch for a count of 10 and repeat 10 times, patient that surgery is similar to bunion surgery, as is the
three times a day, for 8 weeks. recovery time.
Achilles Tendonopathy
Metatarsalgia
• Characteristics of Achilles tendonpathy include:
• an insidious onset leading to chronic posterior heel • Examine for a high arch and the presence of corns or
pain and swelling; calluses beneath the metatarsal heads.
• worsened pain with activity and pressure from • Refer to a podiatrist.
shoes; • Advise the patient to wear a metatarsal pad on the foot (just
• swelling medially and laterally to the insertion of the behind the site of the pain) or an adhesive pad inserted into
Achilles tendon. the shoe to relieve the pressure on the metatarsal heads.
• There is insufficient evidence to determine which treat- • Refer those not responding and patients with severe lan-
ment is most appropriate. However, the following are cinating pain radiating down the cleft between two toes
commonly tried: (Morton neuroma).
• Open backed footwear
• Stretching exercises to lengthen the Achilles tendon Steroid Injections in Soft Tissue Lesions
• Simple analgesia. The condition is not one of inflam-
mation and there is no reason to prefer an NSAID • The poor quality of many studies means that evidence of
(Khan et al., 2002). long-term benefit of steroid injections is lacking.
However, it is used because clinical experience demon- Oral Nonsteroidal Antiinflammatory Drugs
strates at least short-term relief for which there is evi-
dence (Speed, 2001). • NSAIDs have good analgesic action but many patients
• The following potential harms are found in injections of with acute or chronic pain can be managed with
the shoulder (Speed & Hazelman, 2001) and similar paracetamol or weak opioids such as codeine.
figures will apply to injections in other sites: • NSAIDs taken regularly at full dosage have an antiin-
• Infection in 1 in 14,000 to 50,000 injections flammatory action which is beneficial in controlling
• Tendon rupture in less than 1% swelling and stiffness, as well as pain, in inflamma-
• Local scarring in less than 1% tory diseases such as rheumatoid arthritis (RA), spon-
• Discuss with the patient the benefits and harms. dyloarthropathies (SpA), and in some cases advanced
• Familiarize yourself with the common techniques osteoarthritis.
(Silver, 1999). • Unlike opioids, they do not cause constipation, drowsi-
ness, or dependence. Against this must be set serious
Indications cardiovascular and gastrointestinal risks, particularly in
the elderly.
• Intensive use of other approaches for at least 2 months
has failed. Which Oral Nonsteroidal
• Rehabilitation is inhibited by symptoms. Antiinflammatory Drug?
Good Practice • The clinical effects of NSAIDs vary from patient to
• Obtain the patient’s consent. patient. There are important differences between NSAIDs
• Check that you can define the local anatomy. in their relative cardiovascular and gastrointestinal
• Select the finest needle that will reach the lesion. toxicity.
• Clean your hands and the patient’s skin. • Selective cyclooxygenase-2 (coxibs) have a lower risk of
• Use a no-touch technique. serious gastrointestinal effects than traditional NSAIDs.
• Use short-acting or medium-acting corticosteroid prepa- However, this advantage may be lost if prophylactic low-
rations in most cases, with local anaesthetic. dose aspirin is also prescribed.
• Injection should be peritendinous; avoid injection into • Of the traditional NSAIDs, low-dose ibuprofen (1.2 g
tendon substance. daily) has the lowest gastrointestinal toxicity.
• Minimum interval between injections should be • Coxibs increase the risk of thrombotic events and are
6 weeks. therefore contraindicated in established cardiovascu-
• Use a maximum of three injections at one site. lar disease. Diclofenac has the same thrombotic risk
• Soluble preparations may be useful in those patients who as coxibs.
have had hypersensitivity/local reaction to a previous • Low-dose ibuprofen and naproxen 1000 mg daily have
injection. the lowest risk of thrombotic events.
• Record details of the injection. • The choice of NSAID, therefore, should take account of
• Do not repeat if two injections do not provide at least 4 the individual’s risk profile.
weeks of relief.
Adverse Drug Reactions
Postinjection Advice
• Upper gastrointestinal (GI) disease: peptic ulceration,
• Warn the patient of early postinjection local anaesthesia bleeding, nonulcer dyspepsia
and to avoid initial overuse. • Fluid retention, leading to aggravation of heart failure
• Advise resting for at least 2 weeks after injection and • Renal failure, precipitated in preexisting renal impairment
avoid heavy loading for 6 weeks. • Hypersensitivity
• The patient should inform the doctor if there is any sug- • Worsening of hypertension control
gestion of infection or other serious adverse event.
Contraindications and Cautions
Contraindications to Corticosteroid Injection
in Soft Tissue Lesions • Absolute contraindications to all NSAIDs:
• Active peptic ulceration
• If pain relief and anti-inflammatory effects can be • Hypersensitivity (rhinoconjunctivitis, bronchospasm,
achieved by other methods urticaria, angioedema, and laryngeal oedema) to
• Local or systemic infection aspirin or another NSAID; avoidance is essential to
• Coagulopathy prevent life-threatening reactions
• Tendon tear • Pregnancy—third trimester
• Young patients • Severe heart failure
• Absolute contraindications to coxibs: coprescribe a gastroprotective drug. Use a proton pump

• Ischaemic heart disease inhibitor (PPI) though side effects (colic and diarrhoea)
• Cerebrovascular disease may limit its use. Misoprostol is an alternative. Old
• Peripheral arterial disease age is one such high-risk factor. Indeed, NICE (2014)
• Moderate heart failure guidance on osteoarthritis recommends the routine pre-
• Cautions for all NSAIDs: scription of a PPI for all patients on oral NSAIDs or
• Breastfeeding—largely due to manufacturer warn- coxibs.
ings. The British National Formulary (BNF) states the • Review the patient’s continued need for NSAIDs
amounts in breast milk for most NSAIDs are insignifi- regularly.
cant. See individual NSAIDs in Appendix 3 of the BNF.
• Renal, cardiac, or liver failure—renal failure may Managing Nonsteroidal Antiinflammatory
worsen; monitor urea and electrolytes (U&Es). Drug-Induced Gastrointestinal Complications
• Asthma—worsening of asthma may be due to pre-
scribed or OTC NSAIDs.
(New Zealand Guideline Group, 2004)
• Coagulation defects—includes anticoagulation therapy. Uninvestigated Dyspepsia
• The elderly. • Review risk factors for NSAID-induced ulcer. These are:
• Previous peptic ulceration. • past history of proven peptic ulcer disease or serious
• Concomitant use of medications that increase GI risk GI complications;
(e.g., steroid therapy or anticoagulants) • on medications likely to increase GI complications
• Cautions for the use of coxibs: such as steroids or anticoagulants or bisphosphonates;
• Left ventricular dysfunction • patients with significant comorbidity, such as heart
• Hypertension failure;
• Oedema for any other reason • Helicobater pylori infection;
• Patients with risk factors for heart disease • previous NSAID gastropathy;
• high dose of NSAID or taking an NSAID plus aspirin.
Prescribing Practice • Consider as being at increased risk patients who are:
• aged over 65 years plus one risk factor;
• Before prescribing an oral NSAID, consider using a • aged under 65 years plus two risk factors.
topical NSAID. • If at increased risk then:
• Weigh the risks and benefits (Table 11.3) for the indi- • refer patients for endoscopy if risk factor for ulcer present;
vidual and involve the patient in the decision. stop the NSAID pending the outcome (NICE, 2004);
• Prescribe the lowest effective dose for the shortest period • test and treat for H. pylori if positive.
to control symptoms. • If not at increased risk then:
• If the patient has not responded after 3 weeks, consider • stop the NSAID if possible, or reduce the dose, or
changing to an NSAID from another class. change to a less toxic NSAID or to a coxib;
• Advise patients to take their tablets with meals and to • if there is a need to continue an NSAID, prescribe a
report dyspepsia immediately. PPI—misoprostol is an alternative;
• For patients at high risk of developing GI complications, • proceed to endoscopy and testing for H. pylori as
whether prescribing a traditional NSAID or a coxib, above only if there is no improvement.
TABLE Comparative Risks of Traditional Nonsteroidal Antiinflammatory Drugs and Coxibs

11.3 (National Prescribing Centre, 2007)
Traditional NSAID Coxibs
Thrombotic risk RRs compared to placebo are diclofenac 1.63* (equivalent RR 1.42
to a coxib), low-dose ibuprofen 1.51 (not statistically NNH compared to placebo = 300 per annum
significant), naproxen 0.92 (not statistically significant) The risk remains constant for the period of use
GI risk RRs compared to placebo (from RCTs) are diclofenac RR compared to traditional NSAIDs: 0.39;
1.7, ibuprofen 1.2, naproxen 1.8. Risk is highest however, coxibs still carry some GI risk,
during first week probably equivalent to low-dose ibuprofen
*Meaning that diclofenac increases the risk of a thrombotic event by 63%. The absolute increase in risk depends on the baseline risk.
GI, Gastrointestinal; NNH, number needed to harm (the number who need to take the drug for one of them to have an excess adverse effect); NSAID, nonsteroidal
antiinflammatory drug; RR, relative risk.
Proven Peptic Ulcer Gout

• If NSAID treatment needs to be continued after ulcer
healing: GUIDELINE
• coprescribe a PPI or misoprostol; and Hui, M., Carr, A., Cameron, S., et al. (2017). The British
• consider switching to a coxib. Society for Rheumatology guideline for the management of
gout. Rheumatology, 56(7), e1–20. https://academic.oup.
com/rheumatology/article-lookup/doi/10.1093/rheumatology/
kex156.
Classes of Nonsteroidal National Institute for Health and Care Excellence. (2015).
Antiinflammatory Drugs Clincial knowledge summaries: Gout. Available from https://
cks.nice.org.uk/gout.
• Salicylates: aspirin, diflunisal, benorylate
• Acetic acids: diclofenac, etodolac, indomethacin, sulin-
dac, tolmetin
• Propionic acids: fenbufen, fenoprofen, flurbiprofen, Aims of Management
ketoprofen, ibuprofen, naproxen, tiaprofenic acid
• Fenamic acids: flufenamic, mefenamic • To terminate an attack
• Enolic acids: piroxicam, tenoxicam • To prevent recurrent attacks
• Nonacidic acids: nabumetone • To prevent complications
• Selective COX-2 inhibitors: celecoxib, etoricoxib
Treatment of the Acute Attack
• Consider the possibility of septic arthritis and refer
Monitoring Patients Taking Disease urgently if this is suspected.
Modifying Antirheumatic Drugs
Nonpharmacological
See following discussion and Appendix 13. • Advise resting of the affected joint(s).
• Advise the application of a cold pack.
Biologic Therapy Monitoring Pharmacolocgical

• Prescribe a fast-acting NSAID at maximum dose provid-
• Patients receiving biologicals will be monitored in sec- ing there are no contraindications.
ondary care. Disease modifying antirheumatic drugs • Advise that the NSAID should be continued until symp-
(DMARDs) may be coprescribed; if so, monitoring toms subside and for 1 to 2 weeks thereafter.
should be in line with the recommendation for the • Prescribe additional analgesia in the form of opiates, if
DMARDs. needed for severe pain.
• Patients receiving biologicals are at increased risk of
serious infections. They also have an increased risk of Patients With Comorbidity
malignancies and possibly autoimmune diseases, includ- • For patients at increased risk of GI complications, follow
ing demyelinating and lupuslike syndromes. advice under NSAID section or consider an alternative.
• Therefore for GPs it is more important to be alert for the • For patients on a diuretic for hypertension, consider
appearance of symptoms suggestive of these complica- changing to another hypertensive.
tions than to rely on routine monitoring. • For patients with heart failure, do not stop the diuretic.
• Warn patients of the risk of infection and advise them • For patients with heart failure or renal failure, limit the
to report symptoms other than minor illnesses. use of NSAID or consider an alternative.
• Pay particular attention to the risk of tuberculosis. If the
patient develops a productive cough or haemoptysis, Alternatives to an Nonsteroidal
weight loss, and fever, stop treatment and send a sputum Antiinflammatory Drugs
sample to be tested for acid-fast bacilli. • Prescribe colchicine at a dose of 500 µg two to four times
• Warn patients who are not immune to varicella of the per day. Do not use higher doses because of the risk of
need to avoid contact with chickenpox and shingles, and adverse effects (vomiting, diarrhea, and abdominal
of the need to report any inadvertent contact. cramps). The drug should be stopped when the attack
• Ensure that the patient has had a single immuniza- abates or if side effects outweigh the benefit. Caution is
tion against pneumococcus and annual influenza urged in breastfeeding, the elderly, in hepatic and renal
vaccine. impairment, and in GI and cardiac disease.
• If the patient develops a lupuslike rash, take blood for • Prescribe prednisolone 35 mg daily until the attack is
antinuclear antibodies (ANA) and double stranded DNA settling, then reduce to zero so that the total course is 7
(dsDNA) binding and inform the rheumatologist. to 10 days (Janssens et al., 2008).
Patients Not Responding to the Above Consider drug prophylaxis according to the following
• Consider an intraarticular injection of methylprednisolone indications:
(40 or 10 mg for a small joint) as a single dose. 1. If a second or further attacks occur within a year
2. Polyarticular gout
Management After an Attack 3. The presence of tophi
4. Clinical or radiologic signs of chronic gouty arthritis
• Review the patient at 4 to 6 weeks. 5. Prophylaxis when cytotoxic therapy is given for
• Educate the patient regarding self-management. Advise haematologic malignancy
the patient to start treatment as soon as possible after the 6. Recurrent uric acid renal stones
onset of any future attacks and to have a standby supply • Asymptomatic hyperuricaemia is not considered an indi-
of NSAID or alternative. cation for drug prophylaxis.
• Assess all patients for conditions associated with • Wait until the acute attack has subsided for at least 2
hyperuricaemia: weeks before starting prophylaxis, otherwise the attack
• Obesity may be prolonged.
• Alcohol consumption
• Blood pressure
Prophylactic Drugs
• Hyperlipidaemia
• Diabetes Allopurinol
• Renal failure • Prescribe allopurinol as the first choice because it is effec-
tive and does not increase the risk of uric acid stone
Investigations formation (unlike uricosurics, see upcoming discussion).
• Uric acid—check this after the attack has settled because • Prescribe allopurinol starting at 50 to 100 mg daily, increas-
it may be lowered during an attack. A raised level does ing by 50 to 100 mg every few weeks until the serum uric
not prove the diagnosis nor does a low level exclude it. acid is below 300 µmol/L. The maximum dose is 900 mg/
The main value is in determining and monitoring day. Doses above 300 mg daily should be divided.
prevention. • Check uric acid and creatinine yearly.
• Blood tests—check renal function, serum glucose, and • If minor side effects occur (e.g., rash) stop allopurinol
cholesterol. and then reintroduce it at as low a dose as the patient
• Consider joint aspiration when the diagnosis is in doubt can cut from a 100-mg tablet (e.g., 10 mg). If this is
(e.g., knee monoarthritis when the differential diagnosis tolerated, increase the dose slowly.
may be gout or pseudogout [urate crystals and calcium • Do not restart if the rash was exfoliative dermatitis or if it
pyrophosphate dihydrate crystals seen respectively on was associated with fever or involvement of another organ.
microscopy]). Send the aspirate immediately in a plain glass • Give colchicine or an NSAID for the first 3 to 6 months;
bottle for polarizing microscopy (by prior arrangement). attacks may be precipitated in the early stages of allopu-
• X-rays are rarely helpful but can sometimes confirm pseu- rinol treatment. Use colchicine 500 µg twice a day for
dogout (chondrocalcinosis seen on plain films) or dem- the first 3 to 6 months or a standard dose of an NSAID
onstrate joint damage from chronic gout, which is more for up to 6 weeks.
likely to happen as an insidious process in the elderly. • If an attack occurs during prophylaxis, continue to use
allopurinol at the same dose and treat the attack in its
Prevention of Recurrence and Complications own right.
• Advise patients to: Febuxostat

• lose weight if obese but to avoid rapid weight loss, • Febuxostat is an alternative xanthine-oxidase inhibitor
which can precipitate an attack; that can be used in patients who cannot tolerate allopu-
• restrict alcohol to recommended limits, have three rinol or whose renal function is prohibitive to its use.
alcohol-free days each week, and avoid beer and forti- • Start with a dose of 80 mg daily and increase to 120 mg
fied wines; daily after 4 weeks if necessary.
• avoid excessive amounts of purine-rich foods (e.g., • When starting febuxostat use colchicine 500 µg twice a day
offal, red meat, yeast extract, oily fish, and shellfish); for at least 6 months or a standard dose of an NSAID for
• take skimmed milk, lowfat dairy products (e.g., yog- up to 6 weeks (and consider gastroprotection). A low dose
hourt, vegetable sources of protein such as beans and of oral prednisolone can be considered for 4 to 12 weeks if
cherries); both colchicine and NSAIDs are contraindicated.
• maintain a fluid intake over 2 litres/day;
• consider stopping drugs that reduce uric acid excre- If the Above Fails
tion or reduce them to the lowest effective dose. They • Check compliance and review lifestyle (e.g., alcohol
are aspirin (though not low-dose aspirin used in car- consumption).
diovascular protection), thiazides, and loop diuretics. • Consider referral for uricosuric drugs.
Indications for Referral (National Library for • Therefore, the GP has a vital role in referring patients as
Health, 2008) soon as the suspicion of RA arises, when they have undif-
ferentiated inflammatory arthritis and before the diagnosis
• Septic arthritis is suspected: referral is urgent. is confirmed; 12% to 15% of such patients will eventu-
• The diagnosis is uncertain. ally turn out to have RA.
• There is a suspicion of an underlying systemic illness • The GP has a continuing role in managing the patient
(e.g., rheumatoid arthritis or connective tissue disorder). in the long term, within protocols shared with secondary
• Gout occurs during pregnancy or in a young person (<25 care. The GP also has a role in managing the illnesses
years of age). that accompany RA.
• Allopurinol is contraindicated or not tolerated, or is • Patients with RA have an increased risk of cardiovascular
being started but NSAIDs and colchicine are contrain- disease and mortality.
dicated or not tolerated. • Patients with RA have an increased risk of osteopo
• Allopurinol at maximum dose has been ineffective. rosis.
• A person has persistent symptoms during an acute attack
despite maximum doses of antiinflammatory medication Model of Care
(alone or in combination).
• An intraarticular steroid injection is indicated but the • Early referral from primary care for suspected RA for
facilities or expertise are not available. early diagnosis
• Complications are present, including urate kidney stones • Early intervention with DMARDs in the window of
or urate nephropathy. opportunity before joint erosion occurs
• Intense initial secondary care management and patient
PATIENT EDUCATION education
• Patient access and primary care management when the
Arthritis Research UK. Gout: An information booklet. Available
from www.arthritisresearchuk.org. disease is stabilized
• Regular review by a specialist nurse in secondary care
Rheumatoid Arthritis Patient Assessment

• Assess the impact on the patient’s mental health, sleep,
GUIDELINES fatigue, acts of daily living, and social function.
Luqmani, R., Hennell, S., Estrach, C., et al. (2006). British • Screen for and treat cardiovascular risk factors.
Society for Rheumatology and British Health Professionals in • Check for extraarticular manifestations including consti-
Rheumatology guideline for the management of rheumatoid tutional upset.
arthritis (the first 2 years). Rheumatology, 45, 1167–1169.
Rheumatoid arthritis: The management of rheumatoid arthritis Referral
in adults. NICE clinical guideline 79, updated 2015. Available
from www.nice.org.uk. • Refer when any of the following are present for over
6 weeks (Scottish Intercollegiate Guidelines Network
[SIGN], 2000):
Aims of Management • Three or more joints are swollen.
• The metacarpophalangeal or metatarsophalangeal
• To suppress or minimize disease progression joints are involved.
• To control symptoms (pain and stiffness) • Early morning stiffness lasts longer than 30 minutes.
• To preserve function The secondary care service should provide:
• To minimize drug side effects • formal assessment of the severity of disease as baseline
• To promote independence and quality of life for monitoring;
• a range of responses from a multidisciplinary team of nurses,
General physiotherapists, occupational therapist, social workers,
dieticians, orthopaedic surgeons, and podiatrists;
• The impact of early intensive treatment with DMARDs • a specialist nurse to review patients and to provide a
has raised hopes that they may suppress rheumatoid telephone helpline. Secondary care may provide an edu-
disease. However, complete disease suppression is limited cational programme.
by side effects.
• Treatment needs to be started as soon as possible before Prereferral Investigations
joint damage occurs. This window of opportunity is
within 3 months of the onset of symptoms. Yet early • Follow local protocols and investigate in accordance with
diagnosis is difficult even for specialists. the availability of tests at the local laboratory.
• Consider ordering the following with results to accom- • If necessary, prescribe stronger opiates.
pany the referral: • Bear in mind the effects of pain and of side effects on
• ESR and C-reactive protein (CRP), as an indication the individual’s lifestyle.
of disease activity.
• Rheumatoid factor—do this because a positive result Steroids
early in the disease has prognostic significance.
However, the sensitivity and specificity are not suffi- • Consider using systemic steroids in the following situations:
ciently high to make a diagnosis. • Bridging disease control between different DMARD
• The anticyclic citrullinated peptide (anti-CCP) (Mad- therapies
dison & Huey, 2006). It is highly specific (>95%) for • To achieve rapid control of symptoms but only once
rheumatoid at all stages of the disease. This means that the diagnosis has been established
a positive finding makes the diagnosis almost certain. • Recommend local steroid injections for localized flareups.
However, the sensitivity is not sufficiently high in early • Caution is required in long-term steroid use. (See Ster-
disease (45%–60%) to rule out disease so, if it is nega- oids in sections on polymyalgia and temporal arteritis
tive, referral should still be made on clinical grounds. and section on osteoporosis.)
• Full blood count (FBC), because the anaemia of
chronic disease is common and to get a baseline because Disease Modifying Antirheumatic Drugs
some DMARDs and biologicals affect the blood • They include antimetabolites, antimalarials, and biologic
count. agents (Table 11.4).
• Liver function tests (LFTs), because some DMARDs • The choice of DMARD will be made by the patient and
and biologicals affect the liver. consultant.
• U&Es and dip testing of urine because some • Methotrexate has emerged as the most commonly pre-
DMARDs and biologicals affect renal function. scribed DMARD closely followed by sulfasalazine.
• Viral serology if recent illness suggests a viral • Cytokine modulators (anti–tumor necrosis factor [anti-
arthritis. TNF] drugs) are indicated in patients who have failed to
• Consider x-ray of affected joints and of the hands and respond to at least two DMARDs, including methotrex-
feet because rheumatologists usually request them as ate (unless contraindicated).
a baseline for future progression. However, x-rays are
unlikely to be helpful in diagnosis because erosive The General Practitioner’s Role
changes are not usually apparent early in the disease • Support patients during the initiation of therapy. There
(Royal College of Radiologists, 2003). may be no benefit for 2 to 6 months.
• Counsel patient to report potential adverse effects
Patients With Poor Prognosis promptly (see Table 11.4).
• Ensure patients know about the dangers to conception:
The following carry a poor prognosis if present early in the • Several DMARDs are contraindicated in pregnancy.
disease: • Both male and female patients may need to delay
• Persistent synovitis conception until a period has elapsed after stopping
• High ESR or CRP cytotoxics. The period depends on the DMARD (e.g.,
• Positive rheumatoid factor 3 months for methotrexate, 2 years for women on
• Male sex leflunomide).
• Follow local protocols for monitoring the adverse effect
of these drugs, as protocols vary between institutions. See
Shared Care: Pharmacologic Treatment
Appendix 13 for a proposed protocol.
Pain Control
• Recommend paracetamol as a first line analgesic. It is Methotrexate
effective and safe. • The National Patient Safety Agency reports errors in
• Weigh and discuss the risks and benefits of additional prescribing and dispensing.
strategies for controlling pain: NSAIDs (which are supe- • State clearly on prescriptions for methotrexate that the
rior to paracetamol in controlling stiffness and swelling) dose is to be taken weekly.
and opiates. • Prescribe only one strength (usually 2.5 mg).
• Choose an NSAID or coxib according to the patient’s • Prescribe folic acid 5 mg to be taken on a separate day
risk and comorbidities. It is customary to try a patient of the week to reduce nausea.
on one NSAID or coxib for a period of several weeks
and then to switch to an alternative if it is ineffective. Antidepressants
• If NSAIDs or coxibs are needed for the long term, pre- • Ask about sleep disturbance and fatigue.
scribe the lowest effective dose. • Prescribe an antidepressant to aid sleep and reduce pain
• Start with a weak opiate such as codeine. as well as to treat depression, if present.
TABLE Disease Modifying Antirheumatic Drugs, Usual Dose, and Selected Toxicity (See British National
11.4 Formulary for Full List of Adverse Effects)
Drug Usual Maintenance Dose Toxicity
Methotrexate 7.5–15 mg/week GI symptoms, stomatitis, rash, alopecia, infrequent
myelosuppression, hepatotoxicity, rare but serious (even
life-threatening) pulmonary toxicity
Sulfasalazine 1000 mg twice or three times daily Rash, myelosuppression (infrequent), GI intolerance
Leflunomide Maintenance, 10–20 mg once daily Hepatotoxicity, myelosuppression, GI symptoms, rashes including
Stevens-Johnson syndrome, toxic epidermal necrolysis
Cytokine modulators, Etanercerpt, weekly or fortnightly Infections, sometimes severe, including tuberculosis, septicaemia,
anti-TNF drugs subcutaneous injection and hepatitis B reactivation. Hypersensitivity reactions, including
(etanercerpt, Infliximab intravenous infusion every lupus erythematosus–like syndrome, pruritus, injection-site
adalimumab, 8 weeks reactions, and blood disorders, myelosuppression. Worsening
infliximab) Adalimumab fortnightly s.c. injection heart failure
Hydroxychloroquine 200 mg twice daily Rash (infrequent), diarrhoea, retinal toxicity (rare)
Injectable gold salts 25–50 mg intramuscular every 2–4 Rash, stomatitis, myelosuppression, thrombocytopenia, proteinuria
weeks
Oral gold 3 mg daily or twice daily Same as injectable gold but less frequent, plus frequent diarrhoea
Azathioprine 50–150 mg daily Myelosuppression, hepatotoxicity (infrequent), early flulike illness
with fever, GI symptoms, elevated liver function tests
Penicillamine 250–750 mg daily Rash, stomatitis, loss of taste, proteinuria, myelosuppression,
infrequent but serious autoimmune disease
GI, Gastrointestinal; TNF, tumor necrosis factor.
Nondrug Management PATIENT INFORMATION

National Rheumatoid Arthritis Society. About rheumatoid
• Education, physiotherapy, and related interventions arthritis. Available at www.rheumatoid.org.uk (choose “About
remain at the centre of care. rheumatoid arthritis”). Helpline 0800 298 7650
• Constantly review patients not under the care of the Arthritis Research UK. Rheumatoid arthritis. Available at
multidisciplinary hospital team for the need for referral www.arthritisresearchuk.org
to any of the following: Arthritis Care. Rheumatoid arthritis. Available at www.
arthritiscare.org.uk/AboutArthritis/Conditions/
• Physiotherapists—for education, exercise programmes, Rheumatoidarthritis.
and splints
• Occupational therapists—for mobility and daily
living aids
• Nurse specialists
• Orthotic and prosthetic departments • Advise the patient to pace activities to a realistic level.
• Chiropodists • Explain the benefits of the different forms of exercise:
• Orthopaedic surgeon • Range-of-movement or stretching exercises relieve
stiffness and maintain flexibility.
Education • Strengthening exercises maintain muscle strength,
needed for function and joint support.
• Education improves knowledge, symptom control, • Aerobic exercise improves cardiovascular risk and aids
adherence, and self-management. weight control and overall function.
• Consider every consultation an opportunity to educate
the patient. Transcutaneous Electrical Nerve Stimulation,
• Provide information leaflets (see box). Heat, and Cold Applications
Exercise • There is no evidence that they do harm, though cold
rather than heat application is usually recommended for
• Advise patients to keep active and exercise. This improves actively inflamed joints.
mood and encourages self-sufficiency. • There is evidence they may provide short-term relief.
Surgery Diagnosis of Ankylosing Spondylitis

Surgery can be highly effective in selected cases (Scott et al., • Early diagnosis is important to establish an exercise pro-
1998) and covers tendon transfers, arthroplasties (including gramme and to start effective pharmacotherapy to
upper limb and small joints), and arthrodeses. prevent deformity. The average delay in diagnosis is 5 to
7 years.
Alternative Therapies/Diet • A history suggestive of inflammatory back pain (see
upcoming discussion) has both a sensitivity and specific-
• Of RA patients, 60% have tried alternative therapies and ity of about 75% for AS.
diets. Be prepared to discuss alternative and complemen- • Features of inflammatory back pain (Sieper & Rudwal-
tary strategies. let, 2005):
• Complementary therapies that may help but require • Morning stiffness of longer than 30 minutes duration
further research: • Improvement in back pain with exercise
• Gamma-linolenic acid (GLA) containing plant oils, • Pain at night or on early wakening
such as evening primrose, borage, and blackcurrant. • Good response to NSAIDs
GLA may relieve pain and morning stiffness. Side
effects include flatulence, nausea, and diarrhoea. Diagnosis of Other Spondyloarthropathies
Some plant oils can cause liver damage or interact
with medications. • Search for clues in the personal and family histories and
• Fish oils containing eicosapentaenoic acid (EPA) or on examination for diseases of bowel and skin and sexu-
docosahexaenoic acid (DHA). EPA and DHA may ally transmitted disease.
reduce pain and stiffness. Side effects include flatu- • Investigations:
lence and nausea. Fish oil can interact with medica- • CRP and ESR are frequently raised but may be normal.
tion (Mayo Clinic, 2008). • HLA-B27 does not confirm the diagnosis since the
• Acupuncture. Electroacupuncture may relieve pain but background incidence is 6% to 8% in the general popu-
acupuncture has no effect on symptoms, function, or lation. However, a negative result makes the diagnosis
quality of life (Casimiro et al., 2005). unlikely because 95% of AS sufferers are positive.
• X-rays of the sacroiliac joints (SIJs) give a high dose
Disability of radiation with little benefit because diagnostic
changes take years to develop. MRIs are more accurate
• Historically, over 80% of patients would have been at than x-rays.
least moderately disabled after 20 years. This may change • Ask for CRP and ESR to assess inflammation.
with the use of DMARDs. • Check FBC for the anaemia of chronic disease.
• Manage the disability according to the guidance in the • Check U&Es and LFTs if referring as DMARD or anti-
section on disability. TNF therapy may be considered.
• Explain why a plain x-ray is unlikely to help early in the
Spondyloarthropathies disease. Consider x-ray of the sacroiliac joints if symp-
toms have been present for several years.
• The spondyloarthropathies (SpA) are a group of condi- • Consider referral for diagnosis.
tions characterized by inflammatory spinal pain and
enthesitis. They are associated with the HLA-B27 tissue Management
type. The category includes:
• ankylosing spondylitis (AS); • Prescribe an NSAID at full dose. Choose one with a long
• reactive arthritis (following an infection, usually half-life to ease morning stiffness (e.g., naproxen). Pre-
bowel or genitourinary, the latter often termed Reiter scribe an alternative NSAID if response after 4 to 6
syndrome); weeks is insufficient.
• psoriatic arthritis; • Prescribe or advise OTC analgesics for use as needed.
• inflammatory bowel disease–associated arthritis; • Explain the need for regular, daily exercise: It helps to
• undifferentiated spondyloarthropathy. control pain, prevents increasing stiffness, and prevents
deformity (Elyan & Khan 2008). Swimming is a good
Aims of Management all-round exercise, but specific daily exercises have been
developed for maintenance of posture.
• Control symptoms (pain, stiffness, and disability) • Refer to physiotherapy for exercises or to the local
• Prevent deformity and disability National Ankylosing Spondylitis Society (NASS) group
• Manage nonskeletal complications: fatigue, anterior (see accompanying box), or both.
uveitis, pulmonary and thoracic restriction (AS), aortic • During flareups, advise patients to continue exercises
incompetence (AS), and inflammatory bowel disease within the limits of pain.
• Advise patient to lie on the floor prone or supine for 20 • A group practice with 8000 patients may have 50 to 80
minutes each day. patients with Sjögren syndrome, 4 patients with SLE,
• Prescribe low-dose antidepressants for nocturnal pain and 4 to 8 with other CTDs. They are uncommon but
and fatigue (Koh et al., 1997). the GP has a special role in both diagnosis and treatment
• Advise the patient to stop smoking. Limitation of chest because of their multisystem and chronic nature.
expansion occurs over time. • Evidence-based guidelines are not available for CTDs in
• Prescribe or advise hypromellose eye drops for conjunc- general; however, many of the management issues overlap
tival disease (dry gritty eyes). with those of other inflammatory rheumatic conditions
• Watch out for and warn the patient of the symptoms of already mentioned.
acute iritis.
• Consider local steroid injections for peripheral joint Diagnosis
disease or enthesitis for short-term relief during flareups.
• Most present with either arthralgia or Raynaud
phenomenon.
Referrals
• Suspect a CTD when:
To a Rheumatologist • there is arthralgia but no evidence of clinical synovitis;
• Refer suspected new cases: • a rash is present (malar rash of SLE spares the naso-
• Age under 45 years at onset; and labial fold, the rash of dermatomyositis affects face
• Pain present for over 3 months; and and hands);
• Inflammatory back pain features or sacroiliitis shown • muscle weakness, rather than the stiffness of polymy-
on x-ray or MRI (Sieper & Rudwaleit, 2005). algia rheumatica (PMR), is present;
• Refer if flareups fail to settle. • symptoms suggest multiple system involvement. The
• Refer established patients if NSAIDs alone are insuffi- more suggestive features there are, the greater is the
cient. Options which can be initiated in secondary care likelihood of disease being present, especially if com-
include DMARDs (methotrexate and sulfasalazine) and bined with nonspecific features.
biologicals (adalimumab and etanercept). • Review the medical history for salient features which may
have been overlooked in the past (e.g., miscarriages).
To Other Specialities • Order the following tests:
• Refer immediately for an ophthalmologic opinion if you • FBC: leucopenia, lymphopenia, thrombocytopenia,
suspect acute iritis. or haemolysis suggest a CTD;
• Refer to a gastroenterologist in presence of symptoms of • Urine dipstick and U&Es to screen for renal disease;
inflammatory bowel disease (NICE, 2008b). • Creatine kinase;
• ESR/CRP: they are usually raised in a CTD (but note
PATIENT INFORMATION that the CRP is often normal in SLE in the absence
National Ankylosing Spondylitis Society. About AS. Available of infection);
at www.nass.co.uk. • Serum ANA: it is positive in over 90% of many
CTDs; however, it should not be relied on as a diag-
nostic test in the absence of clinical features as it is
Connective Tissue Diseases positive in 5% of the general population and over
30% of people with rheumatoid arthritis.
• The connective tissue diseases are:
• systemic lupus erythematosus (SLE); Management
• systemic sclerosis;
• polymyositis; • Refer suspected cases early to a rheumatologist for diag-
• dermatomyositis; nosis and a management plan.
• Sjögren syndrome; • Share care for monitoring progress and therapy with the
• Raynaud disease; hospital specialist (as for RA).
• mixed connective tissue disorder. • Screen for and manage other cardiovascular risk factors
• The term connective tissue diseases (CTDs) covers a group (e.g., hypertension) especially when renal disease is
of inflammatory diseases affecting multiple systems that present or high-dose steroid therapy is used.
have a variable presentation and course and whose fea- • Women’s health: consider the increased risk of thromboses
tures overlap. They are strongly associated with several and drug therapy (e.g., cytotoxics) for CTD in precon-
autoantibodies. ception counselling, contraception, pregnancy, and
• Complications can be serious and fatal (e.g., lung fibro- hormone replacement therapy (HRT).
sis, renal failure, gangrene). • Treat infections promptly in SLE as they may lead to
• Many features, especially early in the disease, are flareups. Consider the possibility of atypical organisms
nonspecific. in the immunocompromised.
• Maintain a high index of suspicion for malignancy which • the presence of significant clinical risk factors in post-
may underlie the disease, complicate the disease, or com- menopausal women or men over the age of 50.
plicate immunosuppressive treatment. • The risk of future fracture is used as a guide to further man-
agement. Clinical judgment still has a place. Calculators do
PATIENT INFORMATION not take account of a tendency to falls, the greater prognos-
The British Sjögren’s Syndrome Association, helpline 0121 tic risk attached to vertebral relative to other fractures, or
455 6549, www.bssa.uk.net the greater risk after more than one fragility fracture.
Lupus UK, St James House, www.lupusuk.org.uk • There is geographic variation in how services are orga-
Scelroderma and Raynaud UK, www.sruk.co.uk/ nized and which speciality leads them. The GP’s role,
referral routes, and prereferral workup will vary accord-
ingly and local protocols need to be observed.
Osteoporosis • Assessment in general practice includes the search for a
possible cause of secondary osteoporosis and a differen-
GUIDELINES tial diagnosis, according to the individual’s age, sex, and
clinical features.
National Osteoporosis Guideline Group. (2017). Clinical
guideline for the prevention and treatment of osteoporosis.
• Take a history, perform an examination, and order inves-
Available at www.shef.ac.uk/NOGG/downloads.html. tigations appropriate to the individual’s clinical picture
The NOGG guidelines have been used in this section (see box).
because: • Refer younger men with osteoporosis for further inves-
• they cover both men and women; tigation since there is a greater chance of secondary
• they include corticosteroid-induced osteoporosis;
• they give explicit guidance on risk calculation.
osteoporosis in this group.
Procedures Proposed in the Investigation of

Osteoporosis
• Osteoporosis is a disorder of diminished bone density
and degenerate microarchitecture leading to fragility and • Perform the following investigations:
increasing the risk of fracture. • Blood cell count, ESR or CRP, serum calcium,
• Osteoporosis causes over 200,000 fractures each year in albumin, creatinine, phosphate, alkaline phosphatase,
the United Kingdom. and liver transaminases
• Most fractures occur in the larger group with lesser degrees • Thyroid function tests
of demineralization (osteopenia) through interaction with • Bone densitometry (DXA)
other risk factors (see upcoming discussion). • Consider the following investigations if indicated by
• A fragility fracture occurs on minimal trauma (e.g., history and examination:
falling when standing on the ground) after the age • Lateral radiographs of lumbar and thoracic spine/
of 40, in a typical site, including the vertebral bodies, DXA-based vertebral imaging
distal radius, proximal femur, and the proximal humerus. • Protein immunoelectrophoresis and urinary Bence
Jones proteins
Definitions of Osteopenia and Osteoporosis • Serum testosterone, sex hormone–binding globulin
(SHBG), follicle stimulating hormone (FSH), lutein-
• These conditions are defined by the result of dual-energy izing hormone (LH) (in men)
x-ray absorptiometry (DXA) measurements of bone • Serum prolactin
mineral density (BMD): • Screening tests for coeliac
• T score below –2.5 = osteoporosis. The BMD is greater
than 2.5 standard deviations below the young Approach to Prognosis and Intervention
adult mean.
• T score between –1 and –2.5 = osteopenia. The BMD • Be alert to patients at risk with a previous fragility frac-
is between 1 and 2.5 standard deviations below the ture or significant clinical risk factors (see upcoming
young adult mean. discussion).
• T score above –1 = normal. • Consider pharmacological intervention in postmeno-
• Where more than one site is scanned, the lowest pausal women with a fragility fracture without perform-
score is used. ing a DXA scan (NICE recommendations differ,
suggesting this strategy for women over 75).
General Principles • For all other cases, use the FRAX tool to assess the
patient’s 10-year probability of a major osteoporotic frac-
• Diagnosis is based on case finding—namely, identifying ture (www.sheffield.ac.uk/FRAX/index.htm). The tool
those likely to be at risk because of either: will place the patient in one of three bands: low, inter-
• a previous fragility fracture; or mediate, or high risk.
• If the risk is intermediate, arrange for a DXA scan because • Prescribe alendronate as a first line agent because of a
the results may alter management. Recalculate the risk using good evidence base for the prevention of fractures at
the FRAX tool and the result of the DXA. The tool will several sites and its cost effectiveness. Risedronate is an
now place the patient in one of two bands: low or high risk. alternative. It is suitable in both men and women for the:
• Low risk: Give general advice (see upcoming discussion). • treatment of postmenopausal osteoporosis, 10 mg
• High risk: Offer pharmacological intervention (see daily or 70 mg once weekly;
upcoming discussion). • treatment of osteoporosis in men, 10 mg daily;
• prevention of postmenopausal osteoporosis, 5 mg daily;
Clinical Risk Factors for Osteoporosis • prevention and treatment of corticosteroid-induced
osteoporosis, 5 mg daily but for postmenopausal
• Age women not receiving hormone replacement therapy,
• Sex 10 mg daily.
• Low body mass index (≤19 kg/m2)
• Previous fragility fracture, particularly of the hip, wrist, The Practicalities of Prescribing Bisphosphonates
and spine including morphometric vertebral fracture • Advise the patient to swallow the tablets whole with
• Parental history of hip fracture plenty of water, half an hour before breakfast and to
• Current glucocorticoid treatment (any dose, by mouth remain upright for half an hour afterward.
for 3 months or more) • Correct disturbances of calcium and mineral metabo-
• Current smoking lism (e.g., vitamin D deficiency, hypocalcaemia) before
• Alcohol—dose dependent, risks start at daily intake of starting. Monitor serum calcium concentration during
three or more units treatment.
• Causes of secondary osteoporosis, including: • Advise the patient on good dental hygiene during and
• rheumatoid arthritis; after treatment to avoid the rare complication of osteo-
• untreated hypogonadism in men and women; necrosis of the jaw. If remedial dental treatment is
• prolonged immobility; needed, advise the patient to consider having it done
• organ transplantation; before starting alendronate.
• type I diabetes; • If alendronate is not tolerated or contraindicated, con-
• hyperthyroidism; sider an alternative bisphosphonate such as risedronate.
• gastrointestinal disease; If this is not tolerated consider intravenous bisphospho-
• chronic liver disease; nate or denosumab. Raloxifene is an alternative for post-
• chronic obstructive pulmonary disease. menopausal osteoporosis (advise the patient it does not
• Falls (not used in the FRAX calculation) reduce menopausal vasomotor symptoms).
• The bisphosphonates have similar contraindications and
General Management side effects. If GI side effects are prominent, a weekly
formulation (alendronate 70 mg or risedronate 35 mg)
• Assess the risk of falls and refer to local falls prevention may be tried.
services as appropriate. • Contradindications to bisphosphonates are:
• Encourage exercise: resistance exercises (lifting weights) • abnormalities of the oesophagus that delay emptying
and weight-bearing impact exercise (skipping, dancing) (alendronate, risedronate);
(SIGN, 2003). • inability to stand or sit upright for at least 30 minutes;
• Advise a diet that provides a daily intake of 1000 mg • hypocalcaemia;
calcium and 800 IU of vitamin D3. The recommended • pregnancy;
calcium intake can be achieved with 1 pint of semiskimmed • breastfeeding.
milk plus one of: 3 oz hard cheese, three slices white bread • Bisphosphonates should be used with caution if there is:
(or six of wholemeal bread), one small pot of yoghourt, or • other upper GI problems;
3 oz canned sardines. The National Osteoporosis Society • significant renal failure (creatinine clearance <30 mL/
has a list of calcium and vitamin D contents which can be min). Treatment should be reviewed after 3 years with
useful for vegans and religious observances. intravenous (IV) zoledronate and after 5 years of oral
• Give the same dietary advice to those receiving pharma- bisphosphonate. Continuing treatment beyond this
cological intervention. period is generally recommended for those with a
• Prescribe calcium and ergocalciferol 1 tablet twice daily history of hip or vertebral fracture, those who have
to the housebound elderly. had a fracture on treatment, those taking corticoster-
oids, and people over age 75.
Pharmacological Intervention
Corticosteroid-Induced Osteoporosis
• Prevention and treatment relate to the prevention of
osteoporosis and treatment of established osteoporosis, • Keep doses of oral corticosteroids as low as possible and
not the prevention and treatment of fractures. courses as short as possible.
• Consider the risk of osteoporosis in patients with cumu- • TFTs for hypothyroidism
lative doses from intermittent courses. Long-term use of • Creatinine kinase for polymyositis and statin-induced
high-dose inhaled corticosteroids may also carry a risk. myositis
• CXR, as malignancy, especially lung cancer, can cause
polymyalgia
PATIENT INFORMATION • Protein electrophoresis to exclude multiple myeloma
National Osteoporosis Society. www.nos.org.uk (click on the
“About Osteoporosis” tab and follow the links). Initial Management
• Check FBC; a normocytic normochromic anaemia is
common.
• Measure weight, blood pressure (BP), and check serum
Polymyalgia Rheumatica glucose before starting steroids.
Management Objectives • Prescribe prednisolone 15 mg daily. Higher starting
doses are rarely needed and are more likely to cause
• To control symptoms of stiffness and pain (plus asso- adverse effects (Kyle & Hazleman, 1989a).
ciated constitutional symptoms such as fatigue and • Review within a fortnight. Expect a dramatic response
weight loss) within days.
• To reduce the risk of the complication of temporal arte- • If there is no response to steroids, review alternative
ritis (TA) diagnoses as above plus:
• To minimize the risks of steroid therapy • cervical spondylosis (the most common differential
diagnosis);
Diagnosis • rheumatoid arthritis.
• Raise the dose of prednisolone to 30 mg daily if you have
• The patient is being committed to at least 2 years of confidently excluded other diagnoses.
steroid therapy. As no single test reliably confirms the
diagnosis, the history is important. Maintenance Steroid Therapy
• The typical history in PMR is of pain, stiffness, or both,
affecting the upper arms and upper legs with diurnal • The risk of relapse is highest early on in treatment and
variation, that is to say, worse in the mornings. This at doses below prednisolone 10 mg. Temporal arteritis is
history is present in 95% of cases (Bahlas, Ramos-Remos, more likely to develop at doses below 10 mg daily.
& Davis, 1998). • Treatment needs to be maintained for at least 2 years.
• Confidently diagnose PMR if three or more of the diag- Even then, only a quarter of patients can stop steroids
nostic criteria (see box) are present or if one criterion after this time (Kyle & Hazleman, 1993).
is associated with a clinical or pathologic abnormality • Over half of patients with PMR (and giant cell arte-
of the temporal artery. The sensitivity for this criterion- ritis [GCA]) will develop complications of steroids,
based test is 92% (i.e., it will correctly identify 92% of the most common being weight gain (Kyle &
all cases) and the specificity is 80% (i.e., it will correctly Hazleman, 1989b).
exclude 80% of normals) (Bird et al., 1979). • The principle: reduce the prednisolone dose to the lowest
possible to maintain control.
• A suggested routine is as follows (Clinical Knowledge Sum-
maries, 2009):
Features of Polymyalgia Rheumatica • Prescribe 15 mg daily for the first month. Check
• Bilateral shoulder pain or stiffness inflammatory markers after 2 to 6 weeks.
• Onset of illness <2 weeks duration • Reduce by 2.5 mg each fortnight until 10 mg daily is
• Initial ESR above 40 mm/h. reached, providing there is no relapse of symptoms or
• Age 65 years or older rise in inflammatory markers.
• Depression and/or weight loss
• Bilateral tenderness in the upper arms • Review the patient 1 week after each dose
reduction.
• Reduce the daily dose by 1 mg every 6 weeks until 5
to 7 mg daily is reached.
• Maintenance dose: 5 to 7 mg for 12 months provid-
Investigations ing there is no relapse of symptoms or inflammatory
markers.
• Check ESR or CRP. Note that the ESR may be normal • Final reduction: reduce the daily dose by 1 mg every 6
in 20% of cases. to 8 weeks, until 3 mg daily is reached, then by 1 mg
• Consider tests to rule out alternative diagnoses as every 12 weeks, providing symptoms and (less impor-
appropriate: tantly) inflammatory markers remain controlled.
• If symptoms suggest continuing disease in the presence Initial Management (Pountain &
of normal inflammatory markers, give more weight to Hazleman, 1995)
the former.
• Warn patients that symptoms may return and advise early • Likely clinical diagnosis according to ACR criteria with
consultation. A return of symptoms is a more reliable early visual loss: If vision is impaired, give prednisolone up to
indication of relapse than inflammatory markers. 80 mg immediately and make an urgent referral to an
ophthalmologist. If there is likely to be a delay, continue
Referral that dose daily.
• Likely clinical diagnosis according to American College of
• Refer patients when: Rheumatology criteria without visual loss:
• symptom control requires a high maintenance dose of • Take blood for an ESR, CRP, and FBC.
steroids or the patient develops significant adverse • Start prednisolone 40 mg daily.
steroid effects on lower doses (steroid sparing drugs • See the patient after 48 hours.
may be considered); • If there is no response, review the diagnosis.
• there is doubt about the diagnosis, particularly when • Suspected diagnosis: Check ESR or CRP, start steroids
there is synovitis and anaemia suggestive of rheuma- 40 mg daily, and arrange for the patient to be seen within
toid arthritis. 48 hours by a rheumatologist/ophthalmologist.
• Opinion varies on the use of routine temporal artery
Patient Education biopsy. Follow local guidance with regard to referral.
• Advise patients to seek urgent attention if they develop Maintenance Treatment

symptoms suggestive of GCA:
• Unilateral headache • Reduce the dose by 5 mg every 4 weeks down to 10 mg
• Tenderness in the scalp daily, then as for polymyalgia rheumatica.
• Jaw claudication (facial pain on chewing) • If long-term maintenance is needed, keep the dose as low
• Advise patients about the risks of steroids (see upcoming as possible (e.g., about 3 mg daily).
discussion) and precautions to take.
• Offer the ARC booklet, available at www.arc.org.uk Steroids
(search for “polymyalgia rheumatica”). Adverse effects will develop in up to 50% of patients. The
risk is positively associated with the dose.
• Educate the patient about the risks:
Temporal Arteritis • Weight gain
• Hypertension
• TA is the most common form of giant cell arteritis, but • Osteoporosis
other arteries may be involved. After headache or pain • Development of diabetes/worsening of diabetic control
in the temple, the next most common syndrome is jaw • Infection risk
claudication. • Risk of GI perforation in combination with NSAIDs
• It is almost exclusively a disease of white people. • Adrenal suppression
• A normal ESR, CRP, or negative temporal artery biopsy • Ensure the patient has an up-to-date steroid card.
does not rule out the disease. The chance of obtaining a • Follow guidance on steroid-induced osteoporosis.
positive biopsy after 1 week of steroid therapy falls to • Advise the patient not to stop steroids suddenly.
10% (Pountain & Hazleman, 1995). • Advise the patient that it may be necessary to increase
• Steroid therapy should not be delayed pending the results the dose of steroids during intercurrent illness and to
of investigations because untreated GCA carries a risk of seek medical advice.
visual loss in 40% of patients. • Warn the patient to avoid contagious contacts with
chickenpox and shingles (there is a risk of fatal dissemi-
Diagnostic Criteria: American College of nated chickenpox if not immune). Advise them to seek
Rheumatology urgent medical advice if they are exposed. Obtain spe-
cialist advice if such exposure occurs.
• The presence of three of the following has a sensitivity • Consider testing immune status for chickenpox when
and specificity greater than 90%: commencing steroid therapy.
• Age at onset over 50 years • Check weight, BP, and urine (or blood) for glucose every
• New type of headache 3 months.
• Clinically abnormal temporal artery; thickened, • Patients are likely to be on 7.5 mg or more prednisolone
tender, or nodular with decreased pulsation daily for 6 months so should be started on prophylaxis
• ESR greater than 50 mm/h against osteoporosis (see Osteoporosis earlier in chapter).
• Positive arterial biopsy • Recommend pneumococcal and flu vaccination.
Fibromyalgia include a multidisciplinary approach on the basis of

expert opinion; there is no evidence that it is better in
GUIDELINE the majority of cases (Karjalainen et al., 2000).
• Consider referral for cognitive behavioural therapy.
Macfarlane, G. J., Kronisch, C., Dean, L. E., et al. (2017). • Complementary and alternative remedies are often tried.
European League Against Rheumatism (EULAR) revised
recommendations for the management of fibromyalgia.
The one systematic review was not of sufficient quality
Annals of the Rheumatic Diseases, 76, 318–328. www.eular to draw conclusions (Holdcraft et al., 2003).
.org (choose “Recommendations”).
PATIENT INFORMATION
Arthritis Research UK. Fibromyalgia. Available at https://www.
arthritisresearchuk.org/arthritis-information/conditions/
• This is a distinct syndrome of widespread, chronic pain. fibromyalgia.aspx.
The mechanism for the pain is thought to be driven
Patient Support
primarily by central sensitization. Criteria for diagnosis
Fibromyalgia Association UK, PO Box 206, Stourbridge, DY9
(Wolfe et al., 1990): 8YL, helpline 0845 345 2322; benefits helpline 0845 345
1. A history of widespread pain: pain must be present in 2343, http://www.fmauk.org.
all four quadrants of the body and in the spine.
2. Pain should be elicited in at least 11 of 18 specified
tender point sites on digital palpation. These are:
• occiput: suboccipital muscle insertions;
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CD002946.pub2
12
Neurological Problems
DECLAN NUGENT
Headache Drug Treatment
Migraine (With or Without Aura) Treatment Options for Early Parkinson Disease
Once the Diagnosis Is Made Treatment Options in Later Parkinson Disease
Acute Treatment Nonmotor Complications of Parkinson Disease
Prevention Palliative Care of the Terminal Patient
Drug Prophylaxis Main Drugs to Avoid in Parkinson Disease
Botulinum Toxin Type A in Chronic Migraine
Stroke
Migraine in Women of Reproductive Age
Acute Stroke
Menstrual Migraine
Face Arm Speech Time
Migraine and Oral Oestrogens
Longer-Term Management
Migraine in Pregnancy and Lactation
Secondary Prevention
Cluster Headaches (Migrainous Neuralgia)
Transient Ischaemic Attack
Acute Treatment
Assess the Urgency of Referral Using ABCD
Prevention
Other Measures
Non-migrainous Headache Recurrent Transient Ischemic Attacks
Tension-Type Headache
Motor Neurone Disease
Medication-Overuse Headache
End-of-Life Care in Motor Neurone Disease
Chronic Daily Headache
Multiple Sclerosis
Chronic Paroxysmal Hemicrania
Prognosis
Exertional and Coital Headache
Management of Acute Relapses
Severe Headache of Sudden Onset
Disease Modifying Treatment
Raised Intracranial Pressure Regular Review
Epilepsy The Bladder
Management of a Major Fit Other Issues
Emergency Management of Prolonged or Repeated End-of-Life Care
Seizures, Including Status Epilepticus in the Community
Subsequent Management Huntington’s Disease
Initial Management Management
The Danger of Swimming Coping With the Cognitive Impairment
Drug Treatment Paraplegia
National Institute of Health and Care Excellence Spasticity
Recommendations for Treatment in Epilepsy
Urinary Tract Infection
Practical Points
Special Considerations for Women and Girls’ Epilepsy Autonomic Dysreflexia
Follow-Up Pressure Ulcers
Avoiding SUDEP Psychological Aspects
Stopping Treatment Essential Tremor
Parkinson Disease
Management
170
CHAPTER 12 Neurological Problems 171
Headache demonstrated that undertreatment is not cost effective.

Previously people with migraine would have been treated
Headache accounts for 4.4% of consultations in primary with a stepped-care approach; however, evidence now
care and 30% of neurology outpatient appointments. They suggests that combination therapy is the most effective
are a cause of pain and disability, a substantial societal first line treatment for migraine.
burden, and an important cause of absence from work and
school. Once the Diagnosis Is Made
For details of assessment of headaches refer to guidelines • Explain that the large majority of patients can be suc-
below or National Institute of Health and Care Excellence cessfully treated but finding the best treatment can
(NICE) Pathways. take time.
• Discuss lifestyle changes that might make a difference
(see upcoming discussion).
GUIDELINES • Discuss the possibility of nondrug therapies (see upcom-
British Association for the Study of Headache (2010). ing discussion).
Guidelines for all healthcare professionals in the diagnosis • Make a follow-up appointment at a time calculated to
and management of migraine, tension-type, cluster and give the patient a chance to have had three or four
medication-overuse headache. www.bash.org.uk. further attacks.
National Institute for Health and Care Excellence (2012).
Headaches in over 12s: Diagnosis and management. Clinical Acute Treatment
guideline, 150, updated 2015. www.nice.org.uk.
Scottish Intercollegiate Guidelines Network (2008). The • Of the various symptoms of migraine only the headache
diagnosis and management of headache in adults. Guideline, and nausea respond to drug treatment. Acute use of
107. www.sign.ac.uk. drugs has therefore nothing to offer the patient who is
most troubled by the aura.
NICE guidelines suggest:
• Offer combination therapy with an oral triptan and a
• Where a positive diagnosis of primary headache has been nonsteroidal anti-inflammatory drug (NSAID), or an
made, a patient-centred approach to treatment should be oral triptan and paracetamol, as first line treatment
adopted to include: taking into account person’s preference, comorbidities,
• explanation of diagnosis and reassurance that other and risk of adverse events.
pathology has been excluded; • For people aged 12 to 17 years consider a nasal triptan
• options for management; in preference to an oral triptan.
• recognition that headache is a valid medical disorder • For people who prefer to take only one drug, consider
that can have a significant impact on the person, his monotherapy with:
or her family, or carers; a. an oral triptan (see upcoming discussion);
• written and oral information about headache disorders, b. NSAID: British Association for the Study of Head-
including information about support organizations; ache (BASH) recommends ibuprofen 400 to 600 mg,
• explanation of the risk of medication overuse for up to four doses in 24 hours. Alternatives include
people using acute treatments for their headache tolfenamic acid rapid release, naproxen, and diclofe-
disorder. nac. Where nausea and vomiting exist the use of
• Do not refer people diagnosed with tension-type, diclofenac suppositories 100 mg can be considered;
migraine, or cluster headaches for neuroimaging solely c. aspirin (900 mg): Should not be offered to patients
for reassurance. under 16 years due to the association with Reye
• Consider use of a headache diary to record the frequency, syndrome;
duration, and severity of headache; to monitor the effec- d. paracetamol (1 g): There is little evidence for the use
tiveness of headache interventions; and as a basis of of paracetamol on its own.
discussion with the person about the headache disorder • Consider an antiemetic in addition to other acute treat-
and its impact. ment for migraine even in the absence of nausea and
vomiting. There is evidence that antiemetics also enhance
Migraine (With or Without Aura) the efficacy of simultaneously administered oral analge-
sics (Drug and Therapeutics Bulletin [DTB], 1998).
• Migraine occurs in 15% of the UK adult population and Scottish Intercollegiate Guidelines Network (SIGN) and
more than 100,000 people are absent from work or BASH suggest use of:
school as a result of migraine every working day. The • prochlorperazine 3 to 6 mg buccal tablets or dom-
current management of migraine in primary care is far peridone 10 mg orally or 30 mg rectally for nausea
from satisfactory. and vomiting;
• Correct treatment can relieve the symptoms of migraine • metoclopromide 10 mg and domperidone 20 mg are
and improve quality of life. Increasingly it is being also useful as a prokinetic to promote gastric emptying.
• Do not offer ergots or opioids for the acute treatment of • Stop combined oral contraceptives (COCs) in a woman
migraine. Ergots have been found to be less effective than whose migraine starts or worsens when taking them,
other treatments with a more concerning side effect especially if focal symptoms develop. Their use is associ-
profile. Opioids offer little benefit to headache pain and ated with an increased risk of cerebral thrombosis.
it is at the expense of increased side effects such as nausea, • Discuss non-drug therapies, even though most are not avail-
plus the risk of medication overuse headache. able on the NHS. Limited evidence exists for benefit from
• For people in whom oral preparations (or nasal prepara- psychological therapies, stress management, and manual
tions in those ages 12–17 years) for the acute treatment therapy such as chiropractic treatment. NICE highlights a
of migraine are ineffective or not tolerated: low risk of cervical artery dissection/stroke with neck
a. Offer a nonoral preparation of metoclopromide or manipulation, although evidence for this is poor at present.
prochlorperazine. • Evidence exists for benefit from acupuncture in chronic
b. Consider adding a non-oral NSAID or triptan if these migraine and NICE suggests considering this if first line
have not been tried. drug prophylaxis is unsuitable or ineffective.
• Advise people that riboflavin 400 mg/day may be effec-
Use of Triptans tive in reducing migraine frequency and intensity for
• Triptans vary in individual response and tolerability. some people.
NICE recommends to start with the triptan with lowest • Reconsider the diagnosis in a patient who uses rescue
cost, and if consistently ineffective try one or more alterna- medication more than once a week.
tive triptans. BASH adds that ideally each triptan should • Consider drug prophylaxis in anyone whose life is suf-
be tried in three attacks before being rejected. Different ficiently disturbed by attacks.
dosage and route of administration should be considered.
• Should be taken at the start of the headache phase. There Drug Prophylaxis
is evidence of greater efficacy if taken while pain is still Two or more attacks per month is the usual criterion for
mild, but triptans appear to be ineffective if administered prophylaxis, but a patient with severe attacks may choose
during aura. prophylaxis with fewer than that.
• Contraindicated in ischemic heart disease (IHD), previ- • Discuss the benefits and risks of prophylactic treatment,
ous myocardial infarction, uncontrolled/severe hyperten- taking into account the person’s preferences, comorbidi-
sion, coronary vasospasm, peripheral vascular disease ties, risk of adverse events, and impact of the headache
(PVD), and in those with previous cerebral vascular acci- on quality of life.
dent (CVA) or transient ischemic attack (TIA). • NICE recommends use of topiramate or propranolol as
• Not indicated for hemiplegic, basilar, or ophthalmople- first line prophylactic agents.
gic migraine. 1. Topiramate: Initially 25 mg at night for 1 week, then
• Warn patients regarding side effects (tingling, heat, increase in steps of 25 mg at weekly intervals. The
heaviness, pressure or tightness in any part of body usual dosage is 50 to 100 mg daily in two divided
including chest and throat); discontinue if intense, flush- doses and the maximum dosage is 200 mg daily. Not
ing, dizziness, vomiting. licensed for those under 18 years of age.
• Advise women and girls with childbearing poten-
Patients Who Respond and Then Relapse tial that topiramate is associated with fetal malfor-
• Repeat the medication that worked earlier provided that mations and can impair the effectiveness of
doing so does not exceed dosage limits. All triptans are hormonal contraception. Ensure women are on
associated with a return of symptoms within 48 hours in appropriate contraception.
20% to 50% of those who initially responded. • The drug should not be stopped rapidly. If the patient
• In those who can be predicted to relapse, give naproxen suffers from any visual problems reduce and stop the
500 mg or tolfenamic acid 200 mg, along with suma drug as rapidly as possible while seeking urgent advice
triptan 100 mg. from an ophthalmologist; acute myopia and second-
ary angle-closure glaucoma can occur.
Prevention 2. Propranolol: Initially 80 mg daily (either 40 mg twice
• Encourage the patient to discover the triggers of an a day or 80 mg MR once daily). The dose may be
attack and see how they can be altered (e.g., stress, excite- increased to 160 mg daily and subsequently to
ment, certain foods, alcohol, hunger, fatigue, exertion, 240 mg daily if necessary (either in divided doses or
oversleeping, menstruation). However, food may be once daily if MR). BASH however recommend the
unfairly blamed as a migraine trigger. Only accept the use of atenolol 25 to 100 mg twice a day or metopro-
suggestion if attacks occur within 6 hours of ingestion, lol 50 to 100 mg twice a day over propranolol.
the association is repeated, and avoiding that food • Titrate each drug up to an effective dose or to the
reduces the frequency of attacks. maximum recommended dose.
• Encourage physical fitness, provided exercise does not • Try each for 6 to 8 weeks at full dose before abandoning
trigger attacks. Fitness can reduce the frequency of attacks. it. Ask the patient to monitor attacks with a diary.
• Continue a drug that is effective for 6 months then at least two of three menstrual cycles. Use a headache
review as the migraine may be in remission. Withdrawal diary to diagnose.
is best achieved by tapering the dose over 2 to 3 weeks. • If standard acute treatment fails to give an adequate
• If both topiramate and propranolol are unsuitable or response, consider intermittent prophylaxis.
ineffective, NICE suggests considering a course of up to • Frovatriptan (2.5 mg twice a day) or zolmitriptan (2.5 mg
10 sessions of acupuncture over 5 to 8 weeks. twice or three times a day) on the days migraine is expected
• For people who are already having treatment with is recommended by NICE. Warn the patient that she may
another form of prophylaxis, such as amitriptyline, and experience rebound headache after stopping the triptan.
whose headache is well controlled, continue the current • An NSAID, traditionally mefenamic acid 500 mg three
treatment as required. times a day or naproxen starting 2 days before the
• Use only one prophylactic drug at a time, except as an migraine is expected and ending after the risk has passed,
extreme measure, although the combination of a beta usually 2 to 3 days after the onset of menses. Though
blocker and amitriptyline may be useful in a patient who widely used there is limited evidence for this.
also has tension-type headaches. • Oestrogen supplementation. Some evidence exists for the
• Alternative options: use of transdermal oestrogen in the form of patches or
1. Tricyclic antidepressants. The drug most studied is ami- gel in the perimenstrual period. This is limited and not
triptyline 10 to 150 mg at night and is recommended recommended within NICE/SIGN guidelines.
by NICE. They are likely to be especially helpful in • Women already on the COC. Consider the tricycle regimen
patients with a combination of migraine and tension to reduce the frequency of menstruation to once every
headaches. Venlafaxine 75 to 150 mg/day is an alter- 10 weeks (i.e., take the pill continuously for 9 weeks
native. To avoid misunderstanding, explain that you rather than 3, followed by the usual 7-day pill-free inter-
are not giving them for their antidepressant effect. val). If migraine with aura occurs while on the COC, it
2. Sodium valproate is helpful both in migraine and must be stopped.
tension-type headache. Initially 200 mg twice a day,
increased if necessary to 1.5 g in divided doses. Migraine and Oral Oestrogens
3. Pizotifen has been used for many years, particularly in • Migraine with aura. NICE advise not to use combined
children and young people but clinical trials show hormonal contraceptives for contraception in women
little evidence of efficacy. with migraine with aura. This supports the UK Medical
4. Methysergide still has a role where all other prophylaxes Eligibility Criteria (UKMEC) advice that this would
have failed, but it should be given under specialist super- present an unacceptable risk to the individual due to
vision for 4 months at a time with 1 month between increased risk of ischaemic stroke.
courses to reduce the risk of retroperitoneal fibrosis. • Migraine without aura. Use the COC up to the age of
5. Calcium channel blockers. Nifedipine, verapamil, and 35, provided there are no other risk factors for vascular
diltiazem are thought to be effective in conventional disease, and the patient is not using ergotamine.
doses, but there is a lack of evidence. • The menopause. Use hormone replacement therapy (HRT)
if indicated. There is no evidence that its use increases
Botulinum Toxin Type A in Chronic Migraine stroke risk, although the migraine itself may be exacer-
In separate guidance NICE (2012) has recommended the use bated. If so, consider changing HRT, and SIGN suggests if
of botox as an option for prophylaxis of headache in adults taking oral HRT to consider transdermal as an alternative.
with chronic migraine (defined as headaches on at least 15
days/month of which at least 8 days are with migraine): Migraine in Pregnancy and Lactation
• that has not responded to at least three prior pharmaco- • Most women find that their migraine improves in preg-
logic prophylaxis therapies; and nancy. In those still troubled by attacks, the treatment
• whose condition is appropriately managed for medica- options are limited.
tion overuse. • Offer paracetamol for acute treatment. Consider use of a
triptan or NSAID, after discussing the need for treatment
SELF-HELP GROUP and risks associated with medication use. Aspirin or an
NSAID are felt to be safe except in the third trimester. For
Migraine Action Association, 27 East Street, Leicester LE1
nausea, metoclopramide or domperidone are unlikely to
6NB, Tel. 0116 275 8317; www.migraine.org.uk.
cause harm throughout pregnancy and lactation.
• Triptans: limited evidence for use during pregnancy and
as such cannot be recommended as routine. British
Migraine in Women of Reproductive Age National Formulary (BNF) states avoid unless potential
benefit outweighs the risk.
Menstrual Migraine • Be wary of making a new diagnosis of migraine in a
• Suspect if migraine occurs predominantly between 2 pregnant woman. Cerebral venous thrombosis may
days before and 3 days after the start of menstruation in mimic migraine.
• In lactating women use paracetamol, ibuprofen, diclofe- sedimentation rate (ESR) may be needed in patients over
nac, and/or domperidone. Sumatriptan is probably safe. 50 to exclude giant cell arteritis. Cervical spondylosis,
• Seek specialist advice if prophylactic treatment is needed sinusitis, intracranial haemorrhage, and other conditions
during pregnancy. This is not commonly required. of which headache is a symptom will need treatment in
their own right.
• Analgesic headache may complicate management and is
Cluster Headaches (Migrainous Neuralgia)
most likely to occur in patients taking analgesics com-
Acute Treatment bined with benzodiazepines or opioids.
• Discuss the need for neuroimaging for people with a first
bout of cluster headache with a specialist. Tension-Type Headache
• Offer oxygen and/or subcutaneous or nasal triptan as
acute treatment. • Exclude conditions associated with tension-type head-
• A triptan, given parenterally (e.g., sumatriptan 6 mg sub- ache (e.g., cervical spondylosis, poor neck posture, tem-
cutaneously), relieves pain in 73% to 96% of people poromandibular [TM] joint dysfunction, sinus pain, eye
within 15 minutes. Intranasal zolmitriptan 5 to 10 mg muscle disorders).
has a delayed bioavailability but evidence of efficacy with • Explain that the condition is real and benign, and that treat-
relief at 30 minutes in 50% to 63% of people (2008). ment aims at reducing the frequency and severity of symp-
• 100% oxygen at a flow rate of at least 12 L/min through toms rather than cure. Reassurance is very important.
a non-rebreathing mask and reservoir bag for 10 to 20 • Explore the tensions in the patient’s life.
minutes aborts an attack in some people. If found to be • Advise exercise (tension-type headache is more com-
useful in the accident and emergency department (A&E), monly seen in sedentary people) and relaxation methods.
arrange provision of home and ambulatory oxygen. • Assess whether depression is present.
• Do not offer paracetamol, NSAIDs, opioids, ergots, or • Check whether medication overuse is contributing to the
oral triptans. headache.
• Consider aspirin, paracetamol, or an NSAID as acute
Prevention treatment, taking into account the person’s preference,
• Attacks are so devastating that prevention is needed in comorbidities, and risk of adverse events. Episodic tension
most cases. BASH recommends specialist referral, but headaches occurring more than 2 days/week should prompt
the GP is likely to be involved in initiating or changing consideration of prophylaxis rather than acute treatments.
treatment before or between specialist appointments. • Do not offer opioids in the acute treatment of tension-
• Prophylaxis should be continued for 2 weeks after the type headaches.
last attack then tailed off. • Consider prophylaxis in those whose attacks are suffi-
• Trial evidence for the treatments below is scanty; it is ciently severe and frequent:
probably best for verapamil. • NICE recommends considering a course of acupunc-
• Consider verapamil 80 mg three times a day increasing ture, up to 10 sessions over a period of 5 to 8 weeks.
as high as 320 mg three times a day for prophylaxis • NICE felt there was not sufficient evidence to recom-
during a bout of cluster headache. Seek specialist advice mend pharmacologic prophylaxis but SIGN has previ-
regarding dosage regimes and need for electrocardiogram ously recommended the use of tricyclic antidepressants
(ECG) monitoring (risk of atrioventricular [AV] block). (TCAs). This was supported by a systematic review
Beta blockers should not be given concomitantly. It will which found some evidence for their use in tension-
stop two-thirds of episodes once attacks begin. type headaches with a number needed to treat (NNT)
• Seek specialist advice for cluster headache that does not of approximately 10 (Jackson et al., 2010).
respond to verapamil. There is a lack of controlled evidence • Amitriptyline 25 to 150 mg at night. Low doses are
for other prophylactic agents but prednisolone, lithium car- effective. Explain that it is not being given for its
bonate, and methysergide have been used previously. antidepressant effect. A significant effect can be
expected in 25% to 50% of patients.
PATIENT ORGANISATION • Aim to tail off prophylactic treatment after 4 to 6 months
Organisation for the Understanding of Cluster Headache if remission has occurred.
(OUCH), OUCH (UK), Pyramid House, 956 High Road, • Naproxen 250 to 500 mg twice a day for 3 weeks to break
London, N12 9RX. Tel. 01646 651979; www.ouchuk.org. a pattern of particularly troublesome and frequent attacks
may be helpful. Should not be repeated in case of treat-
ment failure.
Non-migrainous Headache
Medication-Overuse Headache
• A history and simple examination (blood pressure [BP],
fundi, and a search for focal neurologic signs) permit a • Establish the diagnosis. Headache may be daily or at
clinical diagnosis in most patients. An erythrocyte least 15 days/month in people overusing acute relief
medication for an underlying headache disorder. This exclusion, having ruled out subarachnoid bleeding for
does not occur in people using analgesia for other painful example.
conditions such as arthritis. Consider it in a patient who
has taken simple analgesics on 15 or more days/month Severe Headache of Sudden Onset
for at least 3 months, or who has taken a triptan, ergota-
mine, opioids, or combination analgesics for 10 or more • Refer urgently if the first attack is sufficiently severe to
days/month for at least 3 months. present acutely. It may be a subarachnoid haemorrhage,
• Explain the diagnosis to the patient. Patients are presaging a more severe bleed.
often hard to convince, especially the minority whose
headache takes up to 2 months without analgesics to Raised Intracranial Pressure
resolve.
• Explain that it is treated by withdrawing overused medi- • This is rarely the cause of headache in a patient without
cations. Advise people to stop taking all overused acute vomiting, papilloedema, or neurological signs. However,
headache medications for at least 1 month and to stop exceptions occur.
abruptly rather than gradually. • Refer patients with a short history of headache (<4 months),
• Advise that headache symptoms are likely to get worse especially if it is localized, worse on waking, worse on
in the short term before they improve and that there may coughing or straining, and especially in the older patient.
be associated withdrawal symptoms. Offer close follow-
up and support. Epilepsy
• Consider specialist referral/advice if using strong opioids, or
have relevant comorbidities, or in whom repeated attempts GUIDELINES
at withdrawal have been unsuccessful. Relapse is common
National Institute for Health and Care Excellence (2012).
with 40% estimated to relapse within 1 year (DTB, 2010). Epilepsies: Diagnosis and management. NICE clinical
• Consider prophylactic treatment for the underlying guideline, 137, updated 2016. www.nice.org.uk.
primary headache disorder in addition to stopping over- Scottish Intercollegiate Guidelines Network. (2015)
used medication. Diagnosis and management of epilepsy in adults. SIGN
• Review the diagnosis of medication overuse headache guideline, 143. www.sign.ac.uk.
and further management at 4 to 8 weeks after the start
of withdrawal of overused medications.
• Withdrawal headaches can be managed with naproxen Management of a Major Fit
250 mg three times a day or 500 mg twice a day. Some
specialists recommend a prolonged period of this treat- 1. Protect them from injury.
ment for 3 to 4 weeks, and not repeated, but there are 2. Prevent onlookers from restraining the fitting patient or
no studies to support this. Where nausea and vomiting putting anything in his or her mouth.
are present, antiemetics can be used. 3. Do not give drugs initially. The fit is likely to have
stopped before the person can act. Give drugs if the fit
Chronic Daily Headache is continuing without signs of abating after 5 minutes or
if three seizures occur within 1 hour.
This is said to exist when headache lasts for at least 4 hours 4. When seizure stops, check the airway and put in the
and occurs at least 15 days a month. Most patients are recovery position.
suffering from one or more of the following: migraine, 5. Check the patient’s cardiovascular and neurologic status.
tension-type headache, and analgesic-overuse headache. 6. Admit any patient with a fit if:
Management is of the underlying cause. If analgesic-overuse • there is suspicion that the fit is secondary to an other
headache is present, tackle that first. illness;
• this is his or her first seizure;
Chronic Paroxysmal Hemicrania • the patient fails to recover completely after the fit
(other than feeling sleepy);
• These are repeated attacks of unilateral headache lasting • there is status epilepticus.
less than 45 minutes.
• Indomethacin 25 to 75 mg three times a day reducing Emergency Management of Prolonged or
to 25 mg daily should prevent attacks. Indeed, if it does Repeated Seizures, Including Status Epilepticus in
not, reconsider the diagnosis. the Community
• Initiate treatment if a seizure lasts longer than 5 minutes
Exertional and Coital Headache or there are more than three in one hour:
1. Check airway, breathing, circulation (ABC).
• Give an NSAID or propranolol before attacks once a 2. Provide high flow oxygen if available.
pattern is established. This should be a diagnosis of 3. Check the blood glucose with a test strip.
4. NICE recommends administering buccal midazolam • Once the diagnosis of epilepsy has been made and a
as first line treatment for children, young people, and decision taken about drug treatment, the role of the
adults in the community. Use rectal diazepam if pre- GP depends on who else is on the team. An epilepsy
ferred or if buccal midazolam is not available. nurse specialist may be best suited to act as key worker
• Adults: but the GP is best placed to see the epilepsy in the
a. Administer buccal midazolam 10 mg first line. context of the patient’s other medical needs, and will
b. Provide rectal diazepam as an alternative: 10 to be the only professional in the community available out
20 mg rectally, repeating 15 minutes later if of hours.
necessary. • Whoever undertakes it, follow-up must be struc-
• Children. Give buccal midazolam: 0.5 mg/kg, to a tured, with a review at least annually, and defaulters
maximum of 10 mg. The BNF for Children suggests: sought out.
a. 0 to 3 months: 0.3 mg (300 µg)/kg up to a • Repeat prescriptions of drugs and a policy of waiting
maximum of 2.5 mg until a patient complains of problems are inadequate.
b. 3 months to 1 year: 2.5 mg The NICE guideline recommends that the care plan be
c. 1 to 5 years: 5 mg in writing; that it should be agreed by professionals,
d. 5 to 10 years: 7.5 mg patient, and carers; and that it should include details of
e. 10 to 18 years: 10 mg how to access care, about drug treatment including ben-
• All doses can be repeated once after 10 minutes if efits and possible adverse effects, and lifestyle issues such
necessary. as employment, driving, and swimming.
• At the time of writing, midazolam oromucosal • Re-referral is needed where:
solution is not licenced for use in children under • control is poor or the drugs are causing side
3 months of age. effects;
5. Call an ambulance (this will depend on response to • seizures have continued for 5 years;
treatment, the individual’s situation, and any person- • there are pointers to a previously unsuspected cause
alized care plan) particularly if: for the fits;
• seizure is continuing 5 minutes after the emer- • concurrent illness complicates management;
gency medication has been given; • the patient needs preconceptual advice for withdrawal
• history of frequent episodes of serial seizures or of antiepileptic drugs (AEDs).
status epilepticus;
• this is first seizure; Initial Management
• there are difficulties monitoring person’s ABC. • Find out how much the patient and family/carers under-
• Convulsive status epilepticus exists where a convulsive stand about epilepsy and answer their questions. Issues
seizure continues for a prolonged period (>5 minutes) to include in discussions are:
or when convulsive seizures occur one after the • support: offer verbal and written information about
other with no recovery in between. This is a medical epilepsy and signpost to support groups (see box);
emergency. • risk management: first aid and safety at home/school/
work;
Subsequent Management • prognosis: in 80% of patients with epilepsy, fits can be
• A single fit is not necessarily epilepsy. Risk of a second controlled by drug treatment (Rugg-Gunn & Sander,
seizure occurring within 2 years is about 50%. After a 2012).
second seizure the risk of a third is about 70% (Rugg- • Acknowledge the distress and anger the patient and
Gunn & Sander, 2012). Initial screening will generally family feel at the disruption this diagnosis has brought
take place in A&E with onward referral for specialist to their lives.
assessment. Some patients may present to the general • Driving. Advise the patient of the need to notify the Driver
practitioner (GP) after the event. and Vehicle Licensing Agency (DVLA) and the insurance
• Urgently refer for neurological assessment to be seen, if company of any seizure, however minor. Document this
possible, within 2 weeks, because of the need to exclude an advice. A licence is likely to be withdrawn until:
underlying cause and because of the implications for work • free from fits for 1 year; or
and driving. Refer more urgently if there are multiple • if the patient’s fits in the last year have been while
seizures or focal neurologic signs. The only patient who asleep and the patient has had fits while asleep for
need not be referred is a child aged 18 months to 5 years more than 3 years with no fits while awake in that
who has recovered promptly from a febrile convulsion. time. Fits at the time of waking or falling asleep count
• The NICE guideline recommends that referral without as “daytime” fits.
drug treatment should be the norm. Initiation of anti- • Employment. Advise the patient not to work at heights
epileptic treatment should only be considered in excep- or near dangerous machinery. A heavy goods vehicle
tional circumstances following discussion with specialist (HGV) or public service vehicle (PSV) license will be
services. lost until fit-free for 10 years.
• Lifestyle. Stress the positive side of how few changes there

need to be. Swimming is possible provided someone else is National Institute of Health and Care Excellence
present who could life save if necessary, provided the patient Recommendations for Treatment in Epilepsy
is not in one of the higher risk categories (see upcoming • Tonic-clonic or generalized seizures:
discussion). Avoid bathing a baby alone. Cycling in traffic • First line treatment: sodium valproate; initially
is probably unwise. Counsel the patient about disclosing 300 mg twice a day, increased gradually (in steps of
the diagnosis to friends and employers. 150–300 mg) every 3 days. Usual maintenance 1 to
2 g, max 2.5 g with specialist advice.
• Lamotrigine: if sodium valproate not suitable, ini-
SELF-HELP GROUPS
tially 25 mg once a day, as monotherapy, increased
Epilepsy Action, New Anstey House, Gate Way Drive, fortnightly to 100 to 200 mg daily.
Yeadon, Leeds LS19 7XY, helpline 0808 800 5050, www.
• Adjunctive treatment options include clobazam,
epilepsy.org.uk.
Epilepsy Society, Chesham Lane, Chalfont St Peter, lamotrigine, levetiracetam, sodium valproate, or
Bucks SL9 ORJ, helpline 01494 601400 for details of local topiramate.
groups and also as an excellent source of information. www. • Focal seizures:
epilepsysociety.org.uk • First line is carbamazepine or lamotrigine.
The Joint Epilepsy Council website has links to all the UK
• Carbamazepine: initially 100 to 200 mg once or twice
and Irish patient organisations: http://www.
jointepilepsycouncil.org.uk. daily, increased slowly (100–200 mg every 2 weeks).
Usual dose 0.8 to 1.2 g daily in divided doses.
• Levetiracetam, oxcarbazepine, or sodium valproate can
be used if first line choices are unsuitable or not tolerated.
The Danger of Swimming • Adjunctive treatment options include carbamazepine,
clobazam, gabapentin, lamotrigine, levetiracetam,
• The danger of swimming for a patient with epilepsy oxcarbazepine, sodium valproate, and topiramate.
varies according to clinical situation, as follows, com- • Petit mal or absence seizures:
pared to the general population: • May require treatment with ethosuximide, lamotrig-
• All types of epilepsy: ×15 ine, or sodium valproate.
• Prevalent epilepsy: ×18 • Myoclonic seizures:
• Epilepsy and learning disability: ×26 • Sodium valproate
• Temporal lobe excision performed: ×41
• Epilepsy and institutional care: ×97 Practical Points
• Adverse effects. Before starting a new drug note the adverse
Drug Treatment effects listed, for instance, in the BNF and discuss them
with the patient.
• Epilepsy is not a single condition but an umbrella term • Compliance. Explain the importance of not missing doses
for a number of conditions. First line treatment and and, especially, of not stopping treatment abruptly. Poor
adjuvant therapy are outlined by NICE based on the compliance may be a sign that the patient does not fully
type of epilepsy diagnosed. This will be a specialist deci- accept the diagnosis.
sion following discussion with the patient and family/ • Alcohol. Explain that moderate drinking of 1 to 3.5 units/
carers to include risks and side effects. day twice a week has no effect on seizure control. One to
• Treatment with AEDs is generally started after the second 2 units a day is probably safe. Heavier drinking may
epileptic seizure and diagnosis is confirmed with a spe- induce a fit as well as interact with antiepileptic drugs.
cialist. Exceptions to this do occur. • Drugs bought over the counter
• Consistent use of the same manufacturer’s preparations • Aspirin. Warn patients taking sodium valproate
is recommended. against taking intermittent aspirin. It displaces valpro-
• NICE recommends monotherapy wherever possible. If ate from protein-binding sites and so potentiates it.
this fails, monotherapy with a different drug is advised. If regular aspirin is needed, the dose of valproate may
Only where control is not achieved with any first line need to be reduced to allow for this.
drug should an add-on drug be given as well. • St John’s wort. It reduces plasma concentrations of
• Antiepileptic treatment is associated with a small carbamazepine and phenytoin.
increased risk of suicidal thoughts and behaviour. Patients
and caregivers should be alert to signs of this throughout Special Considerations for Women and
treatment (Medicines and Healthcare Products Regula- Girls’ Epilepsy
tory Agency [MRHA], 2008). • Careful counselling, with their partners if appropriate,
• Be alert to the use of sodium valproate in women of about contraception, conception, pregnancy, and breast-
childbearing age because of the risk of congenital mal- feeding are required.
formation and neurodevelopmental effects. • Be aware of interactions with contraception.
• AEDs and risk to developing foetus—most notably d. person’s information needs. May involve signposting to
sodium valproate. websites (as discussed) or information sources such as
• Planning pregnancy and pregnancy: www.patient.co.uk where a range of information leaf-
• Early specialist referral is indicated, ideally preconcep- lets are available;
tion, for advice regarding the risks and benefits of e. carer skills. Review knowledge of first aid for people
medication adjustment. Risk associated with inade- having a seizure and any agreed treatment protocols
quate seizure control is considered more detrimental for the treatment of prolonged or recurrent seizures;
to the foetus than use of AEDs. For unplanned preg- f. other considerations. SIGN does not recommend
nancies patients should be advised not to stop any routine blood tests as part of review.
AEDs and be referred urgently.
• Offer all women on AEDs 5 mg folic acid daily. This Adverse Effects of Anticonvulsant Drugs
should be continued throughout the first trimester.
Acute: Rash (usually seen shortly after starting the drug). Tail off
• Risk of seizure during labour is low, but sufficient to
the drug. If combined with fever and lymphadenopathy the rash
recommend delivery in an obstetric led unit. may represent a severe hypersensitivity syndrome. Usually starts
• Vitamin K is indicated for the baby on delivery (1 mg between 1 and 8 weeks of exposure. Multiple organ failure can
intramuscular (IM)) due to risk of neonatal haemor- occur. If the patient is systemically ill, admit.
rhage associated with antiepileptics. Chronic: Weight gain and sedation are the adverse effects of
most common complaints. Check that the patient is taking the
• Most women on monotherapy should be encouraged
lowest effective dose of as few drugs as possible. There is some
to breastfeed. If on combination therapy or other risk evidence that the quality of life is better on the newer drugs but
factors, such as premature birth, seek specialist advice. the NICE review did not consider the evidence to be strong. What
• Contraception and AED therapy: is clear is that each drug has a different side effect profile and
• Enzyme-inducing AEDs may reduce the effect of individuals respond to a drug in different ways. A small increased
risk of suicidal thoughts and behaviour has been reported.
combined oral contraceptive pill (COCP), progestin-
Some AEDs are known to reduce bone mineral density and
only pill (POP), and progesterone implants. increase fracture risk with long-term use. SIGN recommends
• Enzyme-inducers have no effect on intrauterine devices dietary and lifestyle advice be given at review to minimize the
(IUDs), whether copper coil or Mirena, or depot risk of osteoporosis. Consider supplementation for those at
medroxyprogesterone injection. Possible impact to increased risk.
effectiveness of norethisterone enanthate (Noristerat)
injection.
• If using COC, increased doses of oestrogen are Avoiding SUDEP
required.
• Emergency contraception: IUD is the preferred • The deaths of 500 people a year in the United Kingdom
option. Otherwise use of levonorgestrel at double are attributed to (Sudden unexpected death in epilepsy)
dose (3 g as a single dose) as soon as possible within SUDEP.
the first 72 hours. • In adults 33% are thought to be avoidable and the result
• Refer to Faculty of Sexual and Reproductive Health- of inadequate treatment.
care (FSRH, 2017): Drug Interactions with Hor- • Failure to collect prescriptions for antiepileptic drugs has
monal Contraception for further details. been identified as a warning sign (Neligan, Bell, &
Sander, 2011).
Follow-Up • To reduce this risk, observe the following rules:
• Titrate a drug up to the maximum tolerated dose, or
• NICE recommends a structured routine review in general until seizures are controlled.
practice should take place once a year with specialist • When discontinuing a drug, titrate a new drug up to
review depending on individual circumstances. This an effective dose first and then tail the old drug off
should include: slowly. The only exception to this is the patient having
a. seizure control and adverse effects of treatment. a life-threatening reaction to a drug, in which case
Encourage the use of a seizure diary to record seizure abrupt cessation (with immediate introduction of a
frequency and severity. Seizure type for those who new drug) is justified.
have more than one type of seizure and any changes
in pattern since last review; Stopping Treatment
b. social or psychological issues related to epilepsy. This
may include driving, work, family planning, attitude • Specialist advice is wise before withdrawing drugs in a
to diagnosis, and depression; patient who has been free from fits for a number of years.
c. review drug compliance by checking the frequency of Ultimately it is the patient who must make the decision,
repeat prescriptions. Ensure person understands risks weighing the problems of drug-taking against the upset
of poor control of seizures and sudden unexpected of a further fit with its implications for driving, employ-
death in epilepsy (SUDEP), to be discussed; ment, and family distress.
• SIGN recommends this be discussed with patients who • Routine use of functional imaging is not recommended
have been seizure free for at least 2 years. A risk table is by SIGN, but may be considered by specialists in certain
found within the SIGN guideline to aid discussions. situations. Single-photon emission computed tomogra-
• In adults with epilepsy who have been seizure free for 2 phy (SPECT) can be considered as an aid to clinical
years, about 60% will have no further seizures when diagnosis in patients where there is uncertainty between
medication is withdrawn (DTB, 2003). PD and nondegenerative parkinsonism/tremor.
• There is an increased likelihood of recurrence if there • Acute dopamine challenge testing is not recommended
has been: in diagnosis of PD.
• epilepsy since childhood;
• the need for more than one medication to control Management
epilepsy;
• seizures while on medication; • The diagnosis of Parkinson’s disease has huge medical,
• myoclonic or tonic-clonic seizures; psychological, and social implications for the patient and
• an abnormal EEG in the last year (DTB, 2003). family. The medical aspects of care will be shared between
• Reduction should be gradual and SIGN recommends for specialist and GP. The role of primary care in manage-
those on carbamazepine, lamotrigine, phenytoin, sodium ment of confirmed PD has been outlined by NICE
valproate, or vigabatrin, the dose should be reduced by Clinical Knowledge Studies (CKS).
10% every 2 to 4 weeks. • GPs should enable appropriate access to:
• Driving when stopping medication. The DVLA recom- • a Parkinson’s disease specialist physician, generally a
mends that patients should be advised not to drive neurologist or a geriatrician;
during withdrawal and for 6 months after cessation of • a Parkinson’s disease specialist nurse if available;
treatment. Patients must be counselled regarding the • speech and language therapy, physiotherapy, occupa-
need to satisfy driving regulations before resuming tional therapy, social services, community nursing,
driving if seizure does occur. continence and urology specialists, palliative care spe-
cialists, and psychology and mental health services;
Parkinson Disease • the Parkinson’s Disease Society local branch and
regional support (see upcoming discussion).
GUIDELINES • Liaise with specialist services, particularly in relation to
changes in medication. Only start or alter antiparkinso-
Scottish Intercollegiate Guidelines Network (2010). Diagnosis nian medication on the advice of specialist, ensure that
and pharmacological management of Parkinson’s disease.
SIGN guideline, 113. www.sign.ac.uk. changes to repeat medications are made promptly, and
National Institute for Health and Care Excellence (2017). titrate therapy between specialist reviews according to
Parkinson’s disease in adults. NICE clinical guideline, 71. the recommendations of secondary care.
www.nice.org.uk. • Do not suddenly stop any antiparkinsonian medication
Clinical Knowledge Summaries (2016). Parkinson’s as this can precipitate acute akinesia or neuroleptic
disease. Available at www.cks.nice.org.uk/
parkinsons-disease. malignant syndrome.
• Identify worsening motor symptoms and motor compli-
cations (which may be caused by the disease itself or by
antiparkinsonian medication), and manage these appro-
priately. This will usually require specialist advice or an
• Parkinson’s disease (PD) is a progressive neurodegen- interim referral.
erative condition resulting from the loss of dopamine- • Identify and appropriately manage nonmotor symptoms
containing cells in the substantia nigra. The disease and complications, which may be caused by the disease
should be confirmed by a specialist in all cases and itself or by antiparkinsonian medication.
patients referred untreated. • Manage comorbidities. Avoid or use with caution any
• Parkinsonism is a clinical syndrome involving bradyki- drugs that could exacerbate parkinsonism or interact with
nesia plus one or more of tremor (4–6 Hz when at rest), antiparkinsonian medications. For example, avoid use of
rigidity, and postural instability. Parkinson’s disease is the metoclopramide, prochlorperazine, and antipsychotics.
most common form of parkinsonism. Diagnosis of PD • Advise care staff in nursing or residential homes of the
also involves the absence of atypical features, a slow clini- need for correct timing of antiparkinsonian medication.
cal progression, and response to drug treatment. • Offer a regular medication review, including adherence
• Other causes of parkinsonism include drug-induced and adverse effects.
cerebrovascular disease, other forms of dementia, multi- • Other considerations:
system atrophy, and supranuclear palsy. • Financial benefits. Many patients are eligible for ben-
• Clinical diagnosis has poor specificity in the early stages efits for the disabled.
of PD. Regular specialist review is needed to review • Carers. Assess, and reassess, the ability of the carer to
diagnosis and response to treatment. cope.
• Driving. Advise drivers to notify the DVLA and their tablets after food to avoid nausea. Be aware however
insurance company at the point of diagnosis. that a protein meal can compete with levodopa for
absorption. If an antiemetic is needed, use domperi-
SELF-HELP GROUP done. Check the standing blood pressure before and
The Parkinson’s Disease Society, 215 Vauxhall Bridge Road, after starting the drug.
London SW1V 1EJ, tel. 020 7931 8080, has information for • Over time, the response to treatment may decrease
patients and carers and organizes local groups. Helpline: and motor complications may occur in approximately
0808 800 0303; www.parkinsons.org.uk. 40% after 4 to 6 years, including motor fluctuations
such as unpredictable switching between on and off
states, wearing off between doses, and dose failures;
Drug Treatment or dyskinesia may occur such as athetosis (slow, writh-
ing motions of fingers and hands) and dystonia
• Choice of medication is a specialist decision and should (involuntary spasms of muscle contraction that cause
be made taking into account patient preferences. abnormal movements and postures).
• Drug therapy does not prevent disease progression but • There is some debate about use of levodopa as first
improves most patients’ quality of life. line and some specialists employ alternative first line
• When initiating treatment, patients should be advised treatments to delay starting levodopa and thereby
about its limitations and possible side effects. About reduce the onset of disabling dyskinesia.
5% to 10% of patients with PD respond poorly to • Dopamine agonists:
treatment. • Used as monotherapy as either oral or transdermal prep-
• Claims that certain drugs are neuroprotective and should arations. These are found to be slightly less effective than
be started before the development of disabling symptoms levodopa in terms of treating motor impairment and
are not supported by clinical evidence. disability but may delay motor complications.
• The old practice of drug holidays is contraindicated • Two groups exist:
because sudden cessation may give rise to acute akinesia 1. Nonergot-derived dopamine receptor agonists
or to neuroleptic malignant syndrome. (e.g., pramipexole, ropinirole, and rotigotine); can
• It is not possible to identify a universal first-choice be used as first line treatment
therapy for early PD or as adjuvant therapy for later PD 2. Ergot-derived dopamine receptor agonists (e.g.,
according to guidelines. bromocriptine, cabergoline, and pergolide). Both
• Early disease can be considered the point at which a NICE and SIGN advise these are not used as first
diagnosis of idiopathic PD has been made and a clinical line treatment due to the risk of fibrotic reactions
decision has been made to start treatment (based on a (pulmonary, retroperitoneal, and pericardial). Spe-
functional disability requiring symptomatic treatment). cific baseline investigations and follow-up are rec-
Possible first choice therapies include levodopa, nonergot- ommended if used, as outlined in the BNF and
derived dopamine agonists, or monoamine oxidase B SIGN guidance. Be alert for symptoms such as
(MAO-B) inhibitors. Most people will eventually require persistent cough, chest pain, cardiac failure, and
levodopa. abdominal pain.
• Later disease refers to people with PD and on levodopa • Patients should be warned that treatment with dopa-
who have developed motor complications. Possible mine agonists and levodopa is associated with:
first choice therapies are dopamine agonists, MAO-B 1. impulse control disorders (including pathological
inhibitors, and catechol-O-methyltransferase (COMT) gambling, binge eating, and hypersexuality); and
inhibitors. 2. excessive daytime somnolence; be informed of the
implications for driving/operating machinery.
Treatment Options for Early Parkinson Disease • Other side effects include hallucinations, especially in
• Levodopa: older people, and postural hypotension, often worse
• Given with a dopa decarboxylase inhibitor to reduce at the start of treatment.
peripheral availability of levodopa and reduce side • Dopamine agonists are very likely to cause initial
effects such as nausea, vomiting, and cardiovascular nausea or vomiting and domperidone may be needed.
effects (e.g., co-beneldopa and co-careldopa). Slow • MAO-B inhibitors:
release preparations may also reduce side effects. • Such as rasagiline and selegiline
• Use the lowest effective dose to maintain good func- • NICE and SIGN state may be considered as a first-
tion to reduce the development of motor compli- choice therapy. When used as a monotherapy has
cations. This may include individual realistic goal been found to:
setting with specialist team (e.g., being able to walk 1. improve motor symptoms, improve activities of
to the shop). daily living, and delay the need for levodopa. It is
• Warn patients about the immediate side effects (e.g., less clear whether they delay the onset of motor
nausea, postural hypotension, and sleepiness). Give the complications;
2. cause or worsen dopaminergic side effects such as thought to affect up to 50% of patients. There is
dyskinesia, hallucinations, or vivid dreaming; significant overlap in symptoms of depression, cogni-
3. demonstrate a levodopa sparing effect (i.e., a lower tive impairment, and PD which makes diagnosis dif-
dose is required). ficult. Screening using self-rating or clinician rating
• Other drugs available: scales may be of benefit. Information from relatives
• Anticholinergics. Occasionally used when tremor pre- and carers should also supplement any assessment.
dominates. It is not recommended as first choice • In general terms, treatment of depression is the same
treatment due to limited efficacy and the propensity as those without PD. There is limited evidence from
to neuropsychiatric side effects. trials but selective serotonin reuptake inhibitors
• Amantadine. SIGN found there to be insufficient evi- (SSRIs) are most frequently used.
dence to support its use in early PD. Relatively weak • Tricyclic antidepressants may be more effective than
efficacy and mechanism of action has not been SSRIs but use is limited due to adverse effects of the
established. medication.
• Both classes should be avoided if on MAO-B inhibi-
Treatment Options in Later Parkinson Disease tors and with cautions if using a COMT inhibitor.
• Most people with PD will develop, with time, motor Seek specialist advice if necessary.
complications and will eventually require levodopa. • Dementia is more common in people with PD.
Adjuvant drugs to take alongside levodopa have been • Treatable causes should be investigated and treated
developed with aim of reducing motor complications (e.g., acute infection and depression).
and improving quality of life. • Consider safely reducing or discontinuing (on special-
• There are three main strategies when managing motor ist advice if necessary) any drugs which may be causing
complications: or worsening symptoms. This includes antimuscarin-
• Manipulation of oral/topical drug therapy ics such as tricyclic antidepressants, tolterodine or
• More invasive drug treatments (such as apomorphine oxybutynin, H2 antagonists such as ranitidine, ben-
infusions or intraduodenal levodopa) zodiazepines, amantadine, and dopamine agonists.
• Neurosugery, most commonly deep brain stimulation • Refer for specialist assessment and management.
• Patients with complex and disabling motor complications
should be reviewed regularly by their specialist team. In PATIENT INFORMATION
the later stages of disease, as nonmotor complications
Parkinson’s Dementia Information Sheet. PDS UK 2013.
begin to dominate quality of life, the withdrawal of www.parkinsons.org.uk.
some drugs is often appropriate. These decisions should
be made by specialist in consultation with carers and
patients.
• SIGN recommend the following options may be consid- • Psychosis is a key neuropsychiatric feature of PD and
ered in people with advanced PD: associated with a high degree of disability. This includes
• MAO-B inhibitors (selegiline or rasagiline) hallucinations, delusions, and paranoid beliefs.
• Dopamine agonists (oral or transdermal); nonergot • Management should include treating any reversible
agonists (ropinirole, pramipexole, and rotigotine) are causes and reviewing medication.
preferable • Liaise with specialist services early.
• COMT inhibitors (entacapone or tolcapone); may • Mild symptoms that are well tolerated may not need
aid reduction of off time in patients who have motor treatment. More severe symptoms may require gradual
fluctuations. withdrawal of precipitating anti-parkinsonian medi-
• Intermittent subcutaneous apomorphine; may aid cations or the use of an atypical antipsychotic.
reduction of off time. • Evidence supports the use of clozapine, but this
• Subcutaneous apomorphine infusions; for severe requires weekly blood monitoring which may be dif-
motor complications in specialist units. ficult in some patients. There is some evidence of
benefit from quetiapine, but it is not licensed for
Nonmotor Complications of treatment of psychosis in PD.
Parkinson Disease • Excessive daytime somnolence is found in up to 54% of
PD patients. Aetiology is felt to be multifactorial and
• It is important to be alert to nonmotor symptoms at increases with age.
patient reviews and manage them appropriately. An • Management should centre around finding a revers-
extensive list of these is available in NICE CKS, which ible cause such as depression, poor sleep hygiene, and
will include postural hypotension, falls, and pain. drugs associated with altered sleep pattern.
• Depression is of particular importance: • Other conditions associated with PD that may affect
• May be part of the disease rather than a reaction to sleep include restless legs syndrome, periodic leg move-
the disease. Mood disorders including depression are ment of sleep, nocturnal akinesia, and nocturia.
Palliative Care of the Terminal Patient discussion) to diagnose stroke or TIA is supported by
NICE stroke quality standards. Those people with persis-
• A time will come when no further alteration in drugs tent neurological symptoms who screen positive using a
will be helpful, and the patient and family will need an validated tool, in whom hypoglycaemia has been excluded
increased amount of support. and who have a possible diagnosis of stroke, should be
• Check that the patient and family have been given a transferred to specialist stroke unit within 1 hour.
chance to state how they want this stage of the patient’s • Patients with acute stroke should receive brain imaging
life to be managed. within 1 hour of arrival in hospital if they meet the
• Adequate analgesia and sedation of the patient will be indications for immediate imaging. These include:
needed, as in any palliative situation. • those who are candidates for thrombolysis (i.e., they
are within 3 hours of the start of symptoms, although
Main Drugs to Avoid in Parkinson Disease SIGN highlights evidence for up to 4.5 hours) or
early anticoagulation;
• Antipsychotics (e.g., chlorpromazine, haloperidol, flupen- • patients on anticoagulants or with a bleeding tendency;
tixol). Atypical antipsychotics maybe less likely to cause • those with a depressed level of consciousness (Glasgow
problems. Use clozapine cautiously. Coma Score <13);
• Antiemetics (e.g., metoclopramide, prochlorperazine). • those with progressive or fluctuating symptoms;
Use domperidone. • those with signs of alternative pathology (neck stiff-
• Baclofen may cause agitation and confusion. Use with ness, papilloedema, fever);
caution. • those whose stroke begins with severe headache of
sudden onset.
Stroke Face Arm Speech Time
GUIDELINES This was initially developed to aid paramedics to recognize
people with stroke but has become a widely publicized part
National Institute for Health and Care Excellence (2008). of the Act FAST campaign, which was launched in the
Stroke and transient ischaemic attack in over 16s: Diagnosis
and initial management. NICE clinical guideline, 68, updated United Kingdom in 2009 to aid public awareness:
2017. www.nice.org.uk. • Face: ask patient to smile and note if there is a new droop
National Institute for Health and Care Excellence (2010). of the mouth or eye on one side
Clopidogrel and modified-release dipyridamole for the • Arm: a new inability to hold one arm out for 5 seconds
prevention of occlusive vascular events. NICE clinical compared to the other arm
guideline, 210. www.nice.org.uk/ta210.
National Institute for Health and Care Excellence (2017). • Speech: new slurred speech or new inability to under-
Stroke and TIA. NICE Clinical Knowledge Summaries. www. stand or say words
cks.nice.org.uk. • Time: time to dial 999 immediately if you see any of
Scottish Intercollegiate Guidelines Network (2008). these signs
Management of patients with stroke or TIA: Assessment,
investigation, immediate management and secondary Longer-Term Management
prevention. SIGN guideline, 108. www.sign.ac.uk/
guideline108. • Follow-up arrangements will be based on individual
need and response to treatment. It is recommended to
have a primary care review within 6 weeks of discharge,
again at 6 months, and then annually.
Stroke is a preventable and treatable disease. Stroke is the third • Management should include:
biggest cause of death in the United Kingdom and it is esti- • assess need for further specialist review, advice, infor-
mated that 10% of the world’s population dies from a stroke. mation, support, and rehabilitation;
• assess social care needs over time, including carer needs;
Acute Stroke • assess health care needs;
• check and optimize lifestyle measures and drug treat-
• NICE recommends all people with suspected stroke be ments for secondary prevention.
admitted directly to a specialist stroke unit, after initial • Ensure involvement of the community rehabilitation team,
assessment, either from the community or from A&E. In if they are not already involved. This team will manage the
certain circumstances this may be inappropriate, for instance rehabilitation process but may need support from the GP.
the patient is already in the terminal stage of another illness. • Check that a multidisciplinary assessment has been per-
• Admit by dialling 999, without seeing the patient if formed, including assessment of conscious level, swal-
necessary. This will trigger the local protocol for the lowing, speech, pressure sore risk, nutritional status,
management of acute stroke. cognitive impairment, movement, and handling needs.
• Assessment of the patient by paramedics using a validated • Depression. Monitor for depression with a screening ques-
tool such as face arm speech time (FAST) (see upcoming tionnaire. Mood disturbance is common post stroke, which
includes depression, anxiety, and emotional lability. Con- • Driving. Explain that the patient should not drive for at
sider a therapeutic trial of an SSRI if depression is present least 1 month after a stroke or TIA and that the DVLA
and continue for at least 6 months if a benefit is achieved. and the insurance company should be informed.
• Bowel and bladder problems. Disturbance of control of • Influenza. Arrange for annual immunization, as well as
excretion is common in the acute phase of a stroke and pneumococcal immunization in those aged 65 and over.
remains a problem for a significant minority of patients. • Check that the patient knows about the relevant statu-
Support and continence aids should have been put in tory and voluntary organizations.
place prior to discharge, including carer training, but this • Arrange relief admissions and other support for the
will require ongoing support in the community. carers (e.g., attendance allowance) in consultation with
• For further information on specific issues refer to NICE the stroke team.
CKS Stroke—Long-Term Care and Support.
Secondary Prevention Transient Ischaemic Attack

• Lifestyle changes. Assist smokers to stop and urge weight • A TIA is a sudden focal neurological disturbance lasting
reduction, dietary change to include lowfat diet with two less than 24 hours. Early recurrent stroke is common. It
portions of oily fish a week, increased fruit and vegetable is estimated that 10% to 15% have a second TIA/CVA
intake, and reduced salt intake if hypertensive. Reduc- in the first week, with a high proportion being in the
tion of alcohol intake to no more than 14 units of first 48 hours (Markus, 2007).
alcohol per week spread over at least 3 days. Encourage • The likelihood of a subsequent stroke must be assessed
daily physical activity. so that urgent investigation can be provided for those
• Antiplatelet therapy. Following an ischaemic stroke all most at risk.
patients should be given clopidogrel 75 mg daily for life. • Check that the symptoms are due to a TIA. Differentiate
This is based on evidence from the Clopidogrel versus TIA from:
Aspirin in Patients at Risk of Ischemic Events (CAPRIE) • transient cerebral symptoms caused by hypoperfusion
trial and outlined in updated guidelines from NICE in due to cardiac disease;
2010. If clopidogrel is not tolerated then aspirin and • cerebral tumour. Patients with sensory TIAs,
dipyridamole MR should be used in combination. If jerking TIAs, loss of consciousness, or speech arrest
either aspirin or dipyridamole MR is contraindicated or should be assumed to have a tumour until proved
not tolerated, the other agent should be given alone. otherwise;
• Blood pressure. Once the initial phase of the stroke is over • epilepsy. A careful history of the attacks from an
(usually about 2 weeks), control any hypertension to observer is important;
achieve a target blood pressure of less than 130/80. Treat- • migraine;
ment of hypertension in the immediate poststroke period • traumatic brain injury;
is thought to potentially cause extension of stroke. • subdural haematoma;
• Atrial fibrillation. People with disabling ischaemic strokes • subarachnoid haemorrhage.
should be treated with aspirin 300 mg daily for 2 weeks • Note that a patient with a TIA who seems to be in the
before considering anticoagulant treatment with warfa- vertebrobasilar distribution, as manifested for instance
rin or an alternative anticoagulant. by vertigo, bilateral visual loss, or diplopia, should be
• Cholesterol. Statin therapy is recommended for all people managed in the same way as one with a TIA in the
following an ischaemic stroke. This should be initiated carotid distribution, except that the ABCD (Tsivgoulis
48 hours after stroke symptoms onset. NICE recom- et al., 2006) score is likely to be lower.
mends atorvastatin 20 to 80 mg daily as first line statin.
• Carotid artery surgery. Check that the patient has been con- Assess the Urgency of Referral Using ABCD
sidered for carotid endarterectomy or angioplasty, if the (Tsivgoulis et al., 2006)
stroke was in the appropriate carotid artery territory, and a Score the patient’s risk of stroke after a TIA as follows:
good recovery from the stroke is likely. NICE recommends 1. Age 60 and older: 1 point
surgery should occur within 2 weeks of acute non-disabling 2. Blood pressure 140/90 or above: 1 point if either systolic
stroke or TIA, as benefit is greatest within this timeframe. or diastolic reaches those levels
3. Clinical features: 2 points if unilateral weakness; if not,
Other Routine Matters 1 point if speech affected
• Explain the prognosis if requested; 65% are likely to 4. Duration: 2 points if event lasted 60 minutes or more;
achieve independence but the GP can modify this figure 1 point if 10 to 59 minutes; 0 points if less than 10 minutes
according to the individual patient’s clinical state. Patients 5. Diabetes: 1 point
with a better prognosis are those who are continent, who
have regained power on the affected side within 1 month, Significance of the Score
and who are progressing toward walking within 6 weeks. A study of patients admitted to the Oxford Neurology
Improvement is likely to continue for some months. Department found, using the 6-point ABCD score (omitting
diabetes), an overall stroke risk at 30 days of 9.7%. None of PATIENT ORGANIZATION

the strokes occurred in those with a score of 2 or less. Above
The Stroke Association, Stroke House, 240 City Road,
this the stroke risks were as follows:
London EC1V 2PR. Helpline 0845 30 33 100, www.stroke.
• a score of 3: stroke risk 3.5% org.uk.
• a score of 4: stroke risk 7.6% Local Stroke Clubs can be accessed through the
• a score of 5: stroke risk 21.3% community rehabilitation team.
• a score of 6: stroke risk 31.3% (Tsivgoulis et al., 2006)
Referral
• Score of 4 or more: these patients are at high risk of Motor Neurone Disease
subsequent stroke. Refer to the rapid access TIA clinic
(or equivalent local facility) to be seen within 24 hours GUIDELINES
of the onset of symptoms. This may involve admission
Motor Neurone Disease Association. Evidence-based clinical
in some areas.
guidelines in MND. www.mndassociation.org/for_professionals/
• Score of less than 4 but other worrying features: for for_GPs.
example, crescendo TIAs with two or more TIAs occur-
ring in 1 week. Refer to the rapid access TIA clinic (or
equivalent local facility) to be seen within 24 hours of
the onset of symptoms. • Refer every patient for confirmation of the diagnosis.
• Score of less than 4: or the TIA occurred over a week • Explain what little is known about the disease. Offer
before presentation. Refer to the rapid access TIA clinic referral to the Motor Neurone Disease (MND) Associa-
(or equivalent local facility) to be seen within 7 days. tion regional care development adviser.
• Admission also required where uncontrolled atrial • Support the patient and family as you would in any
fibrillation (AF) is present or if a TIA has occurred in terminal illness. The median survival is only 3 to 4 years,
a patient on warfarin, as urgent computed tomogra- with older patients having the worst prognosis. Always
phy (CT) is required to rule out a bleed. make a further appointment rather than waiting for the
patient or family to contact you with a problem.
Other Measures • Ventilation. Discuss at an early stage with the patient and
• Aspirin. Give 300 mg daily to be started immediately family the fact that ventilation may become necessary.
unless contraindicated. Continue while awaiting special- Noninvasive ventilation (i.e., via facemask), at night
ist assessment. Consider use of a proton pump inhibitor only, in those with good bulbar function prolongs life by
if there is a history of dyspepsia associated with aspirin. a median of 7 months with improvement in the quality
If there is a history of intolerance to aspirin, then an of life (McDermott & Shaw, 2008). If bulbar function
alternative antiplatelet should be given, generally clopi- fails, tracheostomy or ventilation may become necessary.
dogrel 75 mg daily should be used, although this is an Discuss this well before it becomes necessary; there is
unlicensed use. often no time to discuss it with them during a crisis.
• Long-term antiplatelet therapy. Initiated in secondary care • Nutrition. In the early stages of dysphagia, refer to a
following diagnosis. Generally, clopidogrel 75 mg (unli- speech therapist and a dietician. Offer referral for con-
censed use). NICE recommends use of a combination of sideration of insertion of a gastrostomy tube or a naso-
aspirin 75 mg daily and dipyridamole MR 200 mg BD gastric tube before the patient becomes weak through
if clopidogrel is not tolerated. Aspirin 75 mg alone can malnutrition. Standard criteria for tube feeding are when
be used if clopidogrel and dipyridamole are both not over 10% of premorbid weight has been lost or when the
tolerated or contraindicated. patient finds eating an ordeal because of choking or
• Secondary prevention. Manage as for stroke. because it takes too long (McDermott & Shaw, 2008).
• Driving. Advise as for stroke. • Refer for physiotherapy, occupational therapy, speech
therapy for help with speech as well as eating, district
Recurrent Transient Ischemic Attacks nursing, and social work assistance as appropriate. In
each case do this pro-actively, not when a serious problem
• If the TIA occurred in a patient already taking aspirin 75 mg has developed.
daily, options would include change to clopidogrel. • Hospice care. Introduce this idea well before it is needed.
• Check that a treatable cardiac or carotid cause has been Short-term admission as respite care will establish a link
excluded. with the hospice and help the patient to make a decision
• Tighten control of other risk factors for stroke (BP, cho- about what he or she wants to happen in the terminal
lesterol, glucose, etc.). stages.
• Admit if the TIAs assume a crescendo pattern. • Drug treatment. Riluzole is recommended by NICE
• Refer if recurrent attacks continue despite the above (2001) for the amyotrophic lateral sclerosis (ALS) form of
measures. motor neurone disease. Treatment should be initiated by a
specialist, but it can then be supervised under a shared-care • The patient should be actively involved in all deci-
arrangement with GPs. Riluzole prolongs life in ALS by an sions. To do this the patient needs clear verbal and
average of 3 to 4 months (McDermott & Shaw, 2008). written information.
• The patient and any family or other carers need emo-
End-of-Life Care in Motor Neurone Disease tional as well as practical support from the medical
services.
• Musculoskeletal pain. Consider physiotherapy to relieve • Whenever the patient is assessed, attention should be
the stiffness associated with prolonged immobility. Treat paid to any hidden factors (e.g., emotional state,
with NSAIDs and opioids. Use opioids early rather than fatigue, bladder and bowel problems) as well as to the
late in patients with pain, as well as to ease the distress presenting symptom. The NICE guidance includes a
of terminal respiratory failure. Treat muscle spasms with useful checklist of issues to consider.
baclofen, tizanidine, or diazepam, although evidence for • The GP should be proactive in the prevention of
their use in MND is lacking. avoidable morbidity (e.g., contractures, inhalation,
• Dribbling. Treat dribbling with anticholinergics (e.g., pressure sores, renal infections).
sublingual hyoscine 0.3 mg three times a day or hyoscine • The MS Society booklet for GPs stresses the role of the
hydrobromide transdermal patches). GP as patient’s advocate. The GP may not know how to
• Dry mouth. Try pineapple chunks or apple or lemon juice manage every problem raised by this complex illness but
to stimulate saliva. he or she should know someone who does and should
• Consider referral for tracheostomy for sputum retention make the referral.
or stridor, or for recurrent aspiration of food or drink. • Even if the patient is already authorized to self-refer
• Choking spasms. Give sublingual lorazepam (0.5–2.5 mg) directly, the GP may be needed to expedite the appoint-
and leave a supply with the patient for future occasions. ment if the first offer of an appointment is not suffi-
They relieve the laryngeal spasm associated with inhala- ciently prompt.
tion of food, drink, or saliva.
• Respiratory failure. Consider referral for ventilatory Prognosis
support if the quality of life is otherwise sufficiently
good. Be prepared to ease the distress of more prolonged • MS follows an unpredictable course, but a better prog-
dyspnoea with oral morphine (start with 2.5 mg four to nosis is associated with:
six times a day and titrate up) while the patient is able • young age at onset;
to swallow and subcutaneous morphine in the terminal • female gender;
stage. More than 90% of patients die in their sleep as a • a relapsing and remitting course;
result of increasing hypercapnia. Choking to death is not • initial symptoms (sensory or optic neuritis);
seen in clinical practice (McDermott & Shaw, 2008). • first manifestations affecting only one CNS region;
• high degree of recovery from initial bout;
• longer interval between first relapses;
PATIENT ORGANIZATION • low number of relapses in the first 2 years;
Motor Neurone Disease Association, PO Box 246, Northampton • less disability at 5 years after onset.
NN1 2PR; 08457 626262; www.mndassociation.org. • Explain what is known about the disease and how good
the prognosis is; the average life expectancy is 25 years
after onset of the disease; 5 years after diagnosis approxi-
mately 70% of people are still employed and 50% need
some help with walking. Approximately 25% of patients
Multiple Sclerosis have a non-disabling form of MS but up to 15% of
patients are severely disabled within a short period (Con-
GUIDELINE favreux, Vukusic, Moreau, & Adeleine, 2000). Point out
National Institute for Health and Clinical Excellence (2014). the positive prognostic features (see box) if they apply.
Multiple sclerosis in adults: Management. NICE clinical
guideline, 186. www.nice.org.uk.
Management of Acute Relapses
• Diagnose a relapse if new symptoms develop or existing
symptoms worsen and these last for longer than 24 hours
• The NICE guidance sets out the following principles and occur after a stable period of longer than 1 month.
regarding multiple sclerosis (MS): • Prior to treatment, possible precipitants, particularly
• Diagnosis should be made rapidly by a specialist neu- infections, should be sought. Urine dipstick should be
rologist; and every patient, once diagnosed, should done on all patients.
have access to neurologist and specialist neurological • Liaise with the specialist team; frequency of relapse may
rehabilitation services as the need arises. influence decisions regarding disease modifying treatment.
• At the start of an acute disabling relapse give methyl- severe spasticity. Percutaneous venoplasty is being used
prednisolone 500 mg orally daily for 5 days, after discus- in some centres but the evidence for this is currently
sion with the patient of the risks and benefits. Intravenous lacking. NICE (2012) recommends that this procedure
treatment may be necessary if the relapse is severe enough should only be used in the context of research.
to warrant admission or the patient cannot tolerate oral
steroids. Regular Review
• Give gastric protection with ranitidine 150 mg twice a • Check that there is a key worker to whom the patient has
day or omeprazole 20 mg once daily. immediate access and who is coordinating all members
• Also consider what help the patient needs, in the way of of the multidisciplinary team. If there is no such person
care and equipment, because of the relapse and whether then, by default, the role falls to the GP.
referral to the specialist neurological rehabilitation service • Fatigue, which is common and not the same as sleepi-
is needed. ness. The fatigue may be physical or mental, so that the
• Giving the same dose intravenously is not feasible in patient can concentrate for only a short period of time.
primary care because the high-dose intravenous prepara- Check for an underlying cause (e.g., depression, chronic
tion should be given over 30 minutes. pain, disturbed sleep). The most useful manoeuvre is to
• Steroids can hasten recovery, but there is no evidence explain that the fatigue is real and that it is a part of the
that the long-term course is altered. syndrome.
• No more than three courses should be given in 1 year. • Spasticity. Treat any precipitating cause, such as infection
• Do not give patients a prescription for steroids to keep or pain. Otherwise management should be supervised by
on standby. the specialist rehabilitation service. The components of
a treatment programme are:
Disease Modifying Treatment • stretching exercises by physiotherapist, patient, or
First Line Treatments family;
• There are currently five first line drugs: the beta interfer- • a skeletal muscle relaxant: baclofen or gabapentin is
ons (Avonex, Betaferon, Extavia, and Rebif ) and glat- often used first line; or if these fail, tizanidine, diaz-
iramer acetate (Copaxone). These should be started and epam, and dantrolene are options. Start low and
supervised by a consultant neurologist, preferably one increase the dose slowly. Up to 100 mg/day of baclofen
with a specialist interest in MS. may be required. If stopping, reduce the dose over
• All drugs must be given by injection. several weeks. Abrupt withdrawal of baclofen may
• Prescribing decisions in the United Kingdom are result in hallucinations or seizures;
determined by the criteria for NHS funding. NICE • consider an evening dose of diazepam if spasms or
did not recommend the use of these drugs but in the clonus interfere with sleep;
United Kingdom, under the risk-sharing scheme set • refer if spasticity is still uncontrolled for consideration
out in Health Service Circular 2002/004 (Depart- of other treatments. This may include use of Sativex
ment of Health [DOH], 2002) they were available (cannabis extract) spray or botulinum toxin as an
for funding for patients who met the specified crite- intramuscular injection.
ria. The Association of British Neurologists (2009) • Weakness. Refer to the specialist rehabilitation service
updated their guidelines and supported their use, taking for training in exercises and techniques to maximize
into account some revisions in the diagnostic criteria strength.
for MS. • Contractures. These may develop around any joint whose
• Flulike symptoms are common with both beta-interferon muscles are weak or spastic. Contractures lead to further
and glatiramer acetate after treatment. These lessen over reductions in mobility and difficulties in handling.
time. Injection site reactions are also common. Prevent them by instructing the patient or carer in
• Any woman receiving disease modifying therapy (e.g., passive stretching of the joint. Refer to the specialist team
interferon) must stop treatment for at least 12 months if a contracture develops.
before trying to conceive. • Pressure sores. Check that all wheelchair users have been
assessed for pressure sore risk and appropriate preventive
Second Line Therapies measures are in place.
• NICE (2007) has recommended natalizumab as an • Depression and anxiety. A major depressive episode occurs
option only for the treatment of rapidly evolving severe in over 50% at some stage. Search for it and treat it as
relapsing-remitting MS. Fingolimod is the first oral actively as in a patient without MS.
therapy for MS and has also been approved by NICE as • Emotionalism. Consider a trial of an antidepressant (a
an option in the treatment of highly active relapsing- TCA or SSRI).
remitting MS (NICE, 2012). In expert centres other • Dysphagia. If the patient has bulbar signs (dysarthria,
treatments may be considered. ataxia, or abnormal eye movements), or if there has been
• Other treatment. Sativex oromucosal spray, a cannabis a chest infection, assess swallowing formally.
extract, is licenced for use in the United Kingdom on a • Dysarthria. If communication is affected refer to a speech
named patient basis, via specialists, for moderate to and language therapist.
• Pain may be neuropathic or musculoskeletal. The former pregnancy with a slight increase in the risk of relapse
needs a trial of carbamazepine, gabapentin, or amitrip- after delivery.
tyline; the latter needs physiotherapy and analgesics. • Immunisations. A patient should have all routine immu-
• Visual problems that are not corrected with glasses need nizations as well as an annual influenza vaccination.
assessment by an ophthalmologist. Optic neuritis is the There is no evidence that they precipitate relapse.
most common cause of visual loss. • Employment. Check that the patient knows about the
• Cognitive impairment, if suspected, should be formally assistance that is available from disability employment
assessed and the results used to inform the management advisors and the Access to Work scheme.
of every aspect of the patient’s care.
• Bladder problems (see upcoming discussion). End-of-Life Care
• Constipation. Can usually be managed with adequate
fluid, bulk laxatives, and stool softeners. More severe While MS is not often fatal in itself, life is shortened by an
constipation may require osmotic agents, bowel stimu- average of 6 to 11 years and it may complicate a death from
lants, anal stimulation, suppositories, or enemas. another condition. See Motor Neurone Disease for a discus-
• Sexual problems. The precise nature of the sexual dysfunc- sion of end-of-life care.
tion will determine the treatment. Physical difficulty from
spasticity may be alleviated by premedication with baclofen, PATIENTS’ ORGANIZATION
and a fast-acting anticholinergic such as oxybutynin may
calm urinary urgency. Sexual dysfunction should not be The Multiple Sclerosis (MS) Society, 372 Edgware Road,
London NW2 6ND. National helpline: 0808 800 8000; www.
automatically attributed to MS. It may be necessary to mssociety.org.uk.
investigate hormonal levels and to obtain urological or
gynaecological consultation. Manual lubrication with gel
is a ready solution to vaginal dryness. Erectile dysfunction
may be treated by sildenafil at NHS expense in the United Huntington’s Disease
Kingdom. If it fails, older treatments may succeed (vacuum
devices, intracavernous injections, or a penile implant). • Diagnosis should always be made by a specialist.
• The health and state of mind of any carers should also be • Genetic testing may be requested by people who are at
explored. risk because of their family history. The arguments in
favour and against are complex and the discussion is best
The Bladder handled by experienced staff at a genetics centre.
• Management should be in the hands of a specialist team;
• Urgency. See whether access to the toilet can be made but the GP may be the first person to detect a new
easier and give an anticholinergic (e.g., oxybutynin or problem and needs to understand the principles of
tolterodine). management.
• Minor incontinence. Consider desmopressin for night-
time incontinence. It works by reducing the volume of Management
urine produced. The same dose once in any 24-hour
period may be useful to tide a patient over a time when • Involuntary movements may be helped by three groups
there is no toilet within reach (e.g., on a journey). of drugs: neuroleptics, benzodiazepines, and dopamine
Padding may help a patient of either sex. depleting agents. They have the problems respectively of
• More severe incontinence, occurring more than once a parkinsonism and tardive dyskinesia; of drowsiness and
week: refer to a continence service. The patient needs ataxia; and of depression and sedation. Some patients can
ultrasound assessment for a residual urine and consider- be managed by ensuring an environment free from stress,
ation of intermittent or even long-term catheterization. with padding of chair and bed and weights on wrists and
Meanwhile, supply pads for a woman and a penile drain- ankles to reduce movements.
age device for a man. • The impairment of voluntary movements does not
• Urinary infection. Once treated, order an ultrasound scan for respond to medication, but useful improvement can be
residual urine, if not already performed. Residuals in excess achieved by behavioural methods. Dysphagia can
of 15 mL are abnormal. If the residual is above 50 mL, or improve with a change of food type, usually to a slightly
if there have been more than three confirmed infections more liquid food, and by developing a habit of eating
in a year, refer to a continence service. If catheterization is slowly. A speech therapist can help with speech and with
needed, intermittent self-catheterization is better than an dysphagia. An occupational therapist can make the home
indwelling catheter if the patient can manage it. safer for a patient at risk of falling.
• The problems posed by cognitive impairment can be
Other Issues reduced by training the family to communicate with the
patient in a simple way. Explain that a patient who seems
• Pregnancy does not appear to influence the course of the to be unaware of his or her disability may have a neuro-
disease overall. There may be fewer relapses during the logical basis for the unawareness and is not being difficult.
• The specific psychiatric disorders that are associated with Spasticity

Huntington’s disease (HD), namely depression, mania,
and obsessive-compulsive disorder, may respond to the • Four types of oral drugs are licenced for the treatment of
same treatment as would be appropriate for a patient spasticity in the United Kingdom. These are benzodiaze-
without HD. pines, baclofen, dantrolene, and tizanidine. The Drugs
and Therapeutic Bulletin (2000) reported that tizanidine
Coping With the Cognitive Impairment was slightly better tolerated than other oral drugs (baclofen
and diazepam) and caused less muscle weakness.
• Explain to the family, and to the patient if the impair- • Attempts to control troublesome spasticity should
ment is not too advanced, the principles of coping with include referral for consideration of intrathecal injection
cognitive impairment. of baclofen, botulinum toxin, or nerve blocks.
• Explain that HD has certain specific problems and
suggest solutions to them: Urinary Tract Infection
• Difficulty with initiating and organizing tasks: use
lists and prompt the patient to do things. • Always confirm suspected urinary tract infections with a
• Perseveration of thoughts or actions: gently ease the midstream specimen of urine (MSU).
patient on to something else. • Do not give antibiotics if the patient is catheterized
• Impulsivity and irritability: reduce stress with a regular unless there are systemic symptoms.
schedule, respond with calmness, and try to find out
what has prompted it. Autonomic Dysreflexia
• Difficulty with attention: do one thing at a time.
• Lack of insight: it is a feature of the disease, not a sign • This is reflex sympathetic overactivity, giving rise to vaso-
that the patient is being difficult. constriction resulting in hypertension, severe headache,
• Advise the patient of the need to notify the DVLA and visual disturbance, anxiety, and pallor. The parasympathetic
insurance company of the condition once it is diagnosed system slows the heart and causes flushing and sweating
(though not when a genetic diagnosis has been made in above the lesion. It only occurs in patients with lesions above
an asymptomatic patient). T6. This should be considered a medical emergency.
• Raise the question of an advance directive. It can be a • If this occurs do the following:
great help if the patient decides certain key issues, and a. Sit the patient up.
records the decision, when still competent to do so. b. Remove the cause (e.g., distended bladder, UTI, loaded
Some key issues are preferred place of terminal care when colon, or anal fissure). If catheterizing or disimpacting,
the need arises, whether to be given a gastrostomy tube allow the lidocaine jelly at least 2 minutes to act. Both
when no longer able to swallow, whether to be resusci- activities can exacerbate autonomic dysreflexia. If flush-
tated when in a terminal state. ing a catheter through, use fluid at body temperature.
• Check what the family has been told about who else is c. It is essential that prompt action is taken to reduce
at risk of developing the disease and what decision has blood pressure to avoid serious or life-threatening com-
been made about genetic testing. Record this in those plications. Do not ignore headaches. Give nifedipine
patients’ records. 10 mg sublingual. Get the patient to bite the capsule.
Alternatively, use glyceryl trinitrate (GTN) one to two
sprays sublingually. Note that a spinal injury patient
PATIENT GROUPS may normally have low blood pressure (e.g., 90/60 mm
Huntington’s Disease Association, Neurosupport Centre, Hg) and a rise to normal level of 120/80 mm Hg may
Liverpool L3 8LR. Tel. 0151 298 3298; www.hda.org.uk. represent a significant elevation.
Scottish Huntington’s Association, Suite 135, St James d. Monitor the BP at least every 5 minutes. It may fluc-
Business Centre, Linwood Road, Paisley PA3 3AT, Scotland.
tuate rapidly. If hypotension occurs, lie the patient
Tel. 0141 848 0308; www.hdscotland.org.
Huntington’s Disease Association, Northern Ireland, C/O down and raise the legs.
Dept of Medical Genetics, Belfast City Hospital Trust, Lisburn e. Admit urgently to hospital if not settling.
Road, Belfast BT9 7AB, Northern Ireland. www.hdani.org.uk. f. If it does settle, warn the patient that it may recur as
the medication wears off, and that he or she should
call for help at the first sign of recurrence (The Queen
Elizabeth National Spinal Injuries Unit, 1999).
Paraplegia
Pressure Ulcers
Every patient with paraplegia will be under the care of a
consultant. The GP however is likely to be the doctor to • Avoid prolonged (>2 hours) immobilization in the same
whom certain problems present. A proactive approach can position.
make a difference to the patient’s quality of life. • Identify pressure areas and protect them.
• Ensure that someone inspects at-risk areas daily (ischii, alprazolam, topiramate, or gabapentin although the evi-
sacrum, trochanters, heels). dence of efficacy is less convincing.
• Refer for an exercise programme to retain posture, • Severe cases uncontrolled by medication: Consider referral
muscle strength. for neurosurgery; deep brain stimulation has shown sig-
• Refer to a dietician to ensure adequate nutrition. nificant reductions in tremor but further studies are
• Treat sores or ulcers intensively and admit early if not required. Botulinum toxin injection has been used in
resolving (e.g., within 2–4 weeks). some studies, but limited evidence exists to support use.
Psychological Aspects References

• Be aware of the fact that patients may be suffering: Association of British Neurologists. (2009). Revised guidelines for pre-
• a severe grief response to the loss of function and scribing in multiple sclerosis. www.theabn.org.uk.
independence; Confavreux, C., Vukusic, M. D., Moreau, M. D., & Adeleine, P.
• a feeling of fear and vulnerability; (2000). Relapses and progression of disability in multiple sclerosis.
• difficulties with their changing relationships with NEJM, 343, 1430–1438.
those around them. Department of Health. (2002). Health Service Circular 2002/004:
• Be prepared to raise the issue of sexuality. Avoid the Cost Effective Provision of Disease Modifying Therapies for
People with Multiple Sclerosis.
temptation to think of the individual as asexual. Sexual
Drug and Therapeutics Bulletin. (1998). Managing migraine. DTB,
function and fertility are possible with appropriate 36(6), 41–44.
support. Recommend publications from the Spinal Inju- Drugs and Therapeutics Bulletin. (2000). The management of spastic-
ries Association (see box). ity. DTB, 38, 44–46.
Drugs and Therapeutics Bulletin. (2003). When and how to stop
antiepileptic drugs in adults. DTB, 41, 41–43.
ADVICE FOR PATIENTS AND PROFESSIONALS Drug and Therapeutics Bulletin. (2010). Management of medication
overuse headache. DTB, 48, 2–6.
Spinal Injuries Association, SIA House, 2 Trueman Place,
Faculty of Sexual and Reproductive Health. (2017). Clincial Guid-
Oldbrook, Milton Keynes MK6 2HH
Helpline: 0800 980 0501; www.spinal.co.uk. ance: Drug Interactions with Hormonal Contraception. Available
www.fsrh.org. Accessed: 2nd October 2018.
Jackson, J. L., Shimeall, W., Sessums, L., et al. (2010). Tricyclic anti-
depressants and headaches: Systematic review and meta-analysis.
Essential Tremor British Medical Journal (Clinical Research Ed.), 341, c5222.
Markus, H. (2007). Improving the outcomes of stroke. British Medical
Journal (Clinical Research Ed.), 335, 359–360.
SYSTEMATIC REVIEW McDermott, C. J., & Shaw, P. J. (2008). Diagnosis and management
Deuchl, G., Raethjen, J., et al. (2011). Treatment of patients of motor neurone disease. British Medical Journal (Clinical Research
with essential tremor. Lancet Neurology. Ed.), 336, 658–662.
Medicines and Healthcare Products Regulatory Agency. (2008). Anti-
epileptics: Risk of suicidal thoughts and behaviour. www.mrha.gov.uk.
National Institute for Health and Care Excellence. (2001). Guidance
• Ask what effect the tremor is having. A quarter of those on the use of riluzole for the treatment of motor neurone disease. NICE
technology appraisal guidance, 20. www.nice.org.uk.
who consult about tremor retire early or change jobs
National Institute for Health and Care Excellence. (2007). Natal-
because of it. izumab for the treatment of adults with highly active relapsing-
• Mild cases: Patients may choose not to take any medica- remitting multiple sclerosis. NICE technology appraisal guidance,
tion. Check that they are not exacerbating the tremor 127. www.nice.org.uk.
with, for instance, caffeine. They may have noticed the National Institute for Health and Care Excellence. (2012a). Botulinum
improvement given by alcohol that is seen in 50% to toxin type a for the prevention of headaches in adults with chronic
70% of patients. migraine. NICE technology appraisal guidance, 260. www.nice.org.uk.
• Cases where the patient is more bothered: Propranolol and National Institute for Health and Care Excellence. (2012b). Fingolimod
primidone have established efficacy and produce a mean for the treatment of highly active relapsing-remitting multiple sclerosis.
tremor reduction of about 50%. NICE technology appraisal guidance, 254. www.nice.org.uk.
• Use propranolol 30 to 60 mg/day, increased to 60 to National Institute for Health and Care Excellence. (2012c). Percutaneous
venoplasty for chronic cerebrospinal venous insufficiency for multiple scle-
240 mg three times a day if necessary.
rosis. NICE interventional procedure guidance, 420. www.nice.org.uk.
• Primidone 50 mg daily initially, increased gradually Neligan, A., Bell, G., & Sander, J. W. (2011). Sudden death in
over 2 to 3 weeks if necessary. Side effects are common epilepsy. British Medical Journal (Clinical Research Ed.), 343,
and frequently dose limiting (e.g., drowsiness, dizzi- d7303.
ness, or disequilibrium). Rugg-Gunn, F. J., & Sander, J. W. (2012). Management of chronic
• Cases not responding to propranolol/primidone or in whom epilepsy. British Medical Journal (Clinical Research Ed.), 345,
they are contraindicated: Consider one of atenolol, sotalol, e4576.
Scottish Intercollegiate Guidance Network. (2008). Diagnosis and Tsivgoulis, G., Spengos, K., Manta, P., et al. (2006). Validation
Management of Headache in Adults. SIGN Guidance 107. Avail- of the ABCD score in identifying individuals of high risk of
able www.sign.ac.uk. Accessed 2nd October 2018. early stroke after a transient ischaemic attack. A hospital based
The Queen Elizabeth National Spinal Injuries Unit. (1999). Management case series study. Stroke; a Journal of Cerebral Circulation, 37,
of autonomic dysreflexia. Retrieved from www.spinalunit.scot.nhs.uk. 2892–2897.
13
Women’s Health
LINDSEY POPE
Dysmenorrhoea Gestational Trophoblastic Hydatidiform Mole and
Primary Dysmenorrhoea Choriocarcinoma
Secondary Dysmenorrhoea The Menopause
Endometriosis Establishing the Diagnosis
Menorrhagia Initial Management
Clinical Assessment Hormone Replacement Therapy
Treatment Benefits and Risks
Flooding Benefits
Risks
Delaying a Period
Other Issues to Discuss
Irregular Periods Contraindications
Teenagers Initial Assessment
Women in Their Reproductive Years Treatment
Older Women Oestrogens
Postmenopausal Women Progestogens
Perimenopausal Women With an Intact Uterus
Intermenstrual Bleeding
Postmenopausal Women With an Intact Uterus
Amenorrhoea Alternative Modes of Delivery
Primary Amenorrhoea Managing the Side Effects of HRT
Girls Aged 14 to 16 Years Bleeding
Girls Aged 16 and Over Bleeding on Cyclic Combined Therapy
Secondary Amenorrhoea Bleeding on Continuous Combined Therapy or Tibolone
Polycystic Ovary Syndrome Oestrogen-Related Side Effects
Progestogen-Related Side Effects
Diagnosis
Follow-Up of Women on Hormone Replacement Therapy
Management
Management of Symptoms How Long to Continue?
Stopping Treatment
Vaginal Discharge
Hormone Replacement Therapy and Contraception
Management: Candida
When Has the Menopause Occurred When Masked by
Recurrent Candidiasis (Four or More Symptomatic Episodes Hormone Replacement Therapy or Combined Oral
per Year) Contraceptive?
Management: Bacterial Vaginosis Cervical Screening
Pelvic Inflammatory Disease Action According to the Smear Result
Diagnosis An Inadequate Smear
Admission Borderline or Mild Dyskaryosis ±Human Papillomavirus
Moderate or Severe Dyskaryosis
Follow-Up for All Patients
Follow-Up After Colposcopy and Treatment
Premenstrual Syndrome (PMS)
Follow-Up After Hysterectomy
Therapeutic Options
Patients Not Responding
Ovarian Cancer Screening
191
Dysmenorrhoea Secondary Dysmenorrhoea

GUIDELINE • Perform an abdominal, vaginal and pelvic examination.
Take a high vaginal swab (HVS) and swabs for Chlamydia.
Royal College of Obstetricians and Gynaecologists. (2012). Consider a pelvic ultrasound scan.
The initial management of chronic pelvic pain. ‘Green Top’
guideline, 41. www.rcog.org.uk. • Suspect a serious cause if there is persistent intermenstrual
bleeding, post coital bleeding or an abnormal looking
cervix.
• Refer for laparoscopy women in whom there is suspicion
of pelvic pathology.
Primary Dysmenorrhoea • Chronic pelvic pain. This affects about one in six women.
It is a symptom with a number of contributory factors
• Dysmenorrhoea is common. In about 20% of women it including gynaecological factors and non-gynaecological
is severe enough to interfere with daily activities. factors (e.g., irritable bowel syndrome, nerve entrapment)
• Dysmenorrhoea is more common in women with an early as well as psychologic and social factors.
age of menarche, longer duration of menstruation, and • Allow enough time for the woman to tell her story.
in those who smoke. • Recommend a pain diary and review.
• Reassure the patient that this is not a sign of disease. An • Refer women who also have dyspareunia and low-grade
examination of the abdomen is worthwhile as part of pain throughout the cycle; they may have subclini-
that reassurance as well as to exclude gross pelvic pathol- cal endometriosis, adenomyosis, or low-grade pelvic
ogy. The need for a vaginal examination will depend on inflammatory disease (PID) despite a normal pelvic
the individual circumstances. It should be performed if examination.
the patient has been sexually active. • Otherwise, treat symptomatically as for primary dysmen-
• Give a nonsteroidal antiinflammatory drug (NSAID) orrhoea, although symptom control is less likely to be
alone or in addition to an analgesic. These work by successful.
inhibiting the synthesis of prostaglandins, and if the • A levonorgestral releasing intrauterine system (IUS) may
cycle is regular they should be started the day before be beneficial, especially as up to 50% of women will
the onset of menstruation until pain subsides. NSAIDs be amenorrhoeic after 12 months. This option depends
are effective in up to 70% of cases. Examples are ibu- on the suitability for the patient (Vercellini, Cortesi, &
profen 1200 mg daily, mefenamic acid 750 to 1500 mg Crosignani, 1997).
daily, or naproxen, although adverse effects may be • Intrauterine contraceptive device (IUD). Consider removing
more common with the latter (Marjoribanks, Proctor, & an IUD, if present.
Farquhar, 2006). • Refer all women, if the pain persists, to a gynaecologist.
• Simple analgesics such as paracetamol can be helpful,
especially for patients in whom NSAIDs are contraindi- Endometriosis
cated (Zhang & Po, 1998).
• Consider a trial of the combined oral contraceptive (COC). GUIDELINES
It is commonly used despite the lack of evidence either
way about its benefit. National Institute of Health and Care Excellence. (2017).
Endometriosis: Diagnosis and Management. NICE Guideline
• Complementary and alternative therapy options: 73. Available: www.nice.org.uk.
• Non drug measures that may help include locally Royal College of Obstetricians and Gynaecologists. The
applied heat (e.g. a hot water bottle) and transcutane- investigation and management of endometriosis. “Green Top”
ous electrical nerve stimulation (TENS). clinical guideline, 24. www.rcog.org.uk/endometriosis.
• Alternative and complementary therapies such as
herbal remedies, dietary supplements and acupunc-
ture lack good quality evidence to support their use
(Khan et al, 2012). • In addition to dysmenorrhoea, consider the diagnosis
• Toki-shakuyaku-san, a herbal remedy, which may reduce particularly in those with deep dyspareunia, chronic pelvic
pain after 6 months. pain, ovulation pain, subfertility, and those with cyclical
• Acupressure, which may be as effective as ibuprofen symptoms without excessive bleeding.
and high-frequency transcutaneous electrical nerve • Refer for laparoscopy patients in whom you suspect the
stimulation (TENS). diagnosis as examination is likely to be normal. The inter-
• Lifestyle and self-help techniques which could be helpful pretation of the finding of endometriosis can, however,
include smoking cessation, warmth to the abdomen, lying be difficult. It is found in about half of women presenting
supine, tea, and a warm bath. with dysmenorrhoea. In some of these it may be coinci-
• Women who are still in pain. Consider referral for all those dental, in that it is present in 2% to 22% of women who
not responding. have no symptoms.
CHAPTER 13 Women’s Health 193
• Where endometriosis has been diagnosed, treatment should Menorrhagia

be guided by a number of factors: wishes of the woman,
severity and duration of symptoms, requirements for fer- GUIDELINES
tility, previous treatment, and any abnormalities identified
on pelvic ultrasound or clinical examination. National Institute for Health and Care Excellence. (2007).
Heavy menstrual bleeding. NICE clinical guideline, 44,
• Patients not wishing to become pregnant consider: updated 2016. www.nice.org.uk.
• a combined oral contraceptive. First line is usually a mono- National Institute for Health and Care Excellence. (2017).
phasic COC containing 30 to 35 µg ethinyloestradiol, CKS menorrhagia. cks.nice.org.uk/menorrhagia.
and either norethisterone or levonorgestrel. A 3-month
trail of this treatment is recommended. A further option
may be to use a tricycling regime to control endome-
trioisis (Moore, Kennedy, & Prentice, 2001);
• progestogens continuously for 6 months (e.g., norethis- • Menorrhagia is regular, excessive menses occurring over
terone 10–20 mg daily). Dydrogesterone appears to consecutive cycles in an otherwise normal menstrual cycle
be no better than placebo (Farquhar, 2001; Vercellini that interferes with the woman’s quality of life.
et al., 1997); • Two thirds of women who have a blood loss of more
• danazol, an androgen that is less commonly used now. than 80 mL per month have to limit normal activities
Most often initiated in secondary care, the patient and have anaemia.
should be maintained on the lowest effective dose for • Menorrhagia is suggested by a history of bleeding that
6 months. Warn the patient about the possible side cannot be controlled with tampons alone and by having
effects of acne, weight gain, muscle cramps, oedema, to get up during the night to change.
and irreversible voice changes (Drug Therapy Bulletin • The presence of other menstrual symptoms may influence
[DTB], 1999; Pattie et al., 1998). Nonhormonal con- a woman’s assessment of the severity of her blood loss;
traception is essential if the patient is sexually active; 50% of women referred for menorrhagia have depression
• gonadorelin analogues, but prescribed only under the or anxiety as their primary problem (DTB, 1994a).
supervision of a gynaecologist. These are available in • One in 20 women aged 30 to 55 years consults her general
a number of different preparations (e.g., goserelin sub- practitioner (GP) each year with menorrhagia (Royal
cutaneous [SC] injection every 28 days, buserelin College of Obstetricians and Gynaecologists [RCOG],
intranasal every day). Oral contraceptives should be 1999) with one-third of women quantifying their periods
stopped prior to starting treatment and a nonhormonal as heavy.
form of contraception should be recommended as • In 40% to 60% no underlying cause is found.
ovulation may occur if the treatment is interrupted at
any point. Usual treatment is a single course of up to Clinical Assessment
6-month duration, though there is some evidence for
repeated and shorter duration courses. It is important • Check that there is no intermenstrual or postcoital
to warn patients about the possibility of menopausal bleeding.
type symptoms with this treatment and these may • Check that there are no other menstrual symptoms, such
necessitate addback treatment in the form of tibolone as pain or pelvic pressure, which might suggest an under-
(licensed) or hormone replacement therapy (HRT; lying pathology.
continuous combined, off licence); • Check that the patient has had a recent smear as offered
• levonorgestrel-releasing intrauterine system (LNG-IUS). This by the current recall system.
may reduce pain when maintained for at least 3 years. • Ascertain the impact the bleeding is having on the
• Patients wishing to become pregnant should: patient’s life.
• consider using NSAIDs, as in primary dysmenorrhea; or • Perform a pelvic and abdominal examination particularly
• refer for consideration of surgical ablation or excision if the history suggests an underlying pathology, initial
of endometriosis. This is an effective alternative to treatment has proved ineffective, or an IUS is being
medical management (Sutton et al., 1994). considered.
• Check haemoglobin (Hb); two-thirds of patients will be
anaemic.
• Exclude hypothyroidism only if there are signs or
PATIENT ADVICE symptoms.
National Endometriosis Society, 50 Westminster Palace • Consider haematologic abnormalities (e.g., von Willebrand
Gardens, Artillery Row, London SW1P 1RL, tel. 020 7222 disease or thrombocytopenia), especially in women who
2781; helpline 0808 808 2227; www.endo.org.uk. have a family history of easy bleeding or who have bled
Royal College of Obstetricians and Gynaecologists. heavily since the menarche.
(2016). Endometriosis: Information for you. www.rcog.org.uk.
• Refer to a gynaecologist for assessment if:
• the patient is over 45; or
• there is suspicion of an organic cause (fibroids, pelvic • gonadotropin-releasing hormone (GnRH) analogues are
pain, dyspareunia); or effective at treating menorrhagia but have significant
• there is any postcoital, intermenstrual, or irregular side effects. These should not be initiated in primary
bleeding, or any sudden change in blood loss; or care and are time limited in their use.
• medical treatment is unsuccessful.
Flooding
Treatment
• Give norethisterone:
• LNG-IUS (Mirena). This appears to be the most effec- • 15 mg twice a day for 2 days, then 10 mg twice a
tive nonsurgical treatment (National Institute for Health day for 2 days, then 15 mg once daily for 2 days, fol-
and Care Excellence [NICE], 2007), is licensed for the lowed by 10 mg once daily for 14 days. The patient
treatment of menorrhagia, and is now suggested as the should expect a bleed 2 to 3 days after stopping the
preferred first-choice treatment. The ECLIPSE trial found norethisterone; or
that the LNG-IUS resulted in greater improvements in • 20 mg twice a day for 5 days.
quality of life than usual medical treatment after 2 years
but the difference at 5 years was no longer significant Delaying a Period
(Gupta et al, 2015).
• If the LNG-IUS is to be used, the patient should be • Give norethisterone 5 mg two or three times a day 3 days
warned that irregular bleeding may occur in the first prior to the expected period. If that fails, double the dose
6 months and that progestogen-type adverse effects are on the next occasion. Continue until it is convenient to
possible: breast tenderness, acne, and headaches. A pelvic have the period. The next period can be expected 2 to 3
examination is needed before insertion. days after stopping norethisterone.
• Tranexamic acid (TXA). Start on the first day of each
cycle and continue until heavy bleeding has ceased. TXA
1 g four times a day decreases bleeding by up to 70% Irregular Periods
(NICE, 2007). Teenagers
• NSAIDs. Give mefenamic acid 500 mg three times
a day or naproxen 500 mg twice daily. Taken shortly • Reassure the patient that irregular periods are common
before or at the start of menstruation and contin- after the menarche and that a regular cycle will probably
ued during heavy loss, they can decrease bleeding by establish itself without treatment.
20% to 50%. They are especially useful if there is • If the patient is sufficiently bothered. Give a progestogen
dysmenorrhoea. cyclically, from day 10 to 25 or day 1 to 21, for 3 months.
• The COC is commonly used but there is insufficient There is, however, no reason to think that this will speed
evidence at present to adequately assess its effectiveness up the establishment of normal periods.
(NICE, 2007). It is especially useful if there is also dys- • COC can be considered especially if contraception is
menorrhoea and if contraception is required. required or the patient has dysmenorrhoea.
• Progestogens. Give them in tablet form for 21 days of each • Very infrequent periods. These patients need assessment
cycle (days 5–26). Giving them for the second half of (see upcoming discussion).
each cycle only is no better than placebo (Lethaby, Irvine,
& Cameron, 2001). This is not an effective form of con- Women in Their Reproductive Years
traception. Alternatively, they can be given by injection
in the same way as for contraception. • Exclude pregnancy.
• IUD. If an IUD is in situ, either give an NSAID • Check whether irregular periods or amenorrhoea have
or an antifibrinolytic or change to a progestogen- always been a feature. They may have polycystic ovary
releasing IUD. syndrome (PCOS; see upcoming discussion).
• Women not responding. Consider referral for: • Check whether the irregularity is due to the progestogen-
• ultrasound and if inconclusive, hysteroscopy to exclude only pill (POP).
endometrial polyps and other pathology such as fibroids. • Look for weight loss, excessive exercise, recent stress, hir-
Also consider endometrial sampling in patients over sutism, galactorrhoea, or infertility.
40 years of age; • Take blood for thyroid function tests (TFTs), prolactin, free
• endometrial ablation or hysterectomy. Endometrial androgen index, luteinizing hormone (LH), and follicle-
ablation has a shorter hospital stay and less time off stimulating hormone (FSH) on day 2 of the cycle.
work, but a proportion of women will have further • If results are normal and the patient wishes to become
bleeding and will require further surgery (Gannon pregnant, consider referral for clomiphene.
et al., 1991). It is not suitable for women who may • If the results are normal and pregnancy is not desired,
wish to conceive in the future; COC or cyclical progestogens can be considered.
• If the results are abnormal, refer to an endocrinologist Give cyclical progestogens from day 12 to 26 (i.e., in the
or gynaecologist, as appropriate. luteal phase), to women sufficiently troubled by the
symptoms.
Older Women • Women on the COC: Check compliance. If the examina-
tion is normal and bleeding persists, consider changing
• Distinguish the irregularity due to ovarian failure from the pill (see section on COC).
the pathological pattern of intermenstrual bleeding (IMB), • Women with an IUD:
which should be referred. • Take endocervical swabs and a HVS.
• Consider cyclical progestogens if the patient is sufficiently • If there is no infection, remove the IUD but remember
bothered. to discuss alternative contraception.
• The COC will regulate bleeding and provide contracep- • Reassess in 3 months.
tion. The known risks of thrombosis and breast carcinoma
should be discussed with the patient and documented. Amenorrhoea
• HRT can be considered if there are other symptoms of
the menopause and the bleeding is not pathological. REVIEW
National Institute of Health and Care Excellence. (2009/2014).
Postmenopausal Women CKS: Amenorrhoea. https://cks.nice.org.uk.
GUIDELINES
National Institute of Health and Care Excellence. (2015).
guideline, 12. www.nice.org.uk.
Scottish Intercollegiate Guidelines Network. (2002). Primary Amenorrhoea
Investigation of postmenopausal bleeding. SIGN guideline, Girls Aged 14 to 16 Years
61. www.sign.ac.uk.
• Refer any girl aged 14 who has no secondary sexual
characteristics.
• Refer any girl whose breast development began more than
2 years ago and who has not yet menstruated.
• Postmenopausal bleeding is generally accepted as an episode
of bleeding 12 months or more after the last period. Girls Aged 16 and Over
• Refer (to be seen within 2 weeks) if: • Examine her for evidence of endocrine disorders, chronic
• there has been an episode of postmenopausal bleeding systemic illness, and eating disorders.
• the patient is on tamoxifen, especially for more than • Look at the external genitalia and secondary sexual char-
5 years. There is a fourfold increase in incidence in acteristics, and check for an imperforate hymen (associated
endometrial cancer. with cyclical pain).
• For the management of bleeding in women taking HRT, • Refer to a gynaecologist or endocrinologist as indicated
see the section on HRT. by likely diagnosis.
Intermenstrual Bleeding Secondary Amenorrhoea

• Examine: • Secondary amenorrhoea exists when a woman has not
• for anaemia; menstruated for 6 months, having had a previously estab-
• the pelvis for abnormalities with a bimanual exami lished cycle.
nation; • In the history, note particularly:
• the cervix for abnormalities, and take swabs; • any weight loss (if the patient’s weight is <45 kg
• the vulva and vagina. and she is of average height, she is unlikely to
• Check smear history and past results. menstruate);
• Take a pregnancy test, if appropriate. • contraception. Amenorrhoea may occur with use of
• Refer urgently (to be seen within 2 weeks) any the LNG-IUS system, with depot medroxyprogesterone,
woman with abnormalities on examination suspicious or following use of the COC;
for cancer. • excessive exercise;
• Consider urgent referral for any patient with persistent • recent stress;
IMB despite normal examination findings. • the character of the cycles prior to the amenorrhoea.
• Premenstrual spotting reassure the patient if the loss is • Exclude pregnancy.
light and premenstrual. It is probably due to failure of • Take blood for LH and FSH, free testosterone, prolactin,
the corpus luteum, and should settle within a few cycles. oestradiol, and TFTs.
• Refer to a gynaecologist or endocrinologist, as appro 1. Polycystic ovaries on ultrasound scan (USS) (either ≥12
priate, if: follicles in at least one ovary or increased ovarian volume
• the FSH and LH are persistently high and the patient [>10 mL]). The diagnosis can be made in the absence of
is under the age of 40. This indicates premature ovarian polycystic ovaries on USS; and the appearance of polycystic
failure; or ovaries on USS, without the other criteria, is not enough
• the free testosterone is raised. This suggests PCOS (see to make the diagnosis.
upcoming discussion); or 2. Oligoovulation or anovulation: as suggested by menstrual
• the prolactin is over 1000 mIU/L or 500 to 1000 cycles longer than 35 days or less than 10 periods a year.
mIU/L on two occasions. This suggests a pituitary 3. Clinical and/or biochemical signs of hyperandrogenism.
adenoma. Check for drugs that can cause hyperpro- Clinical signs include acne, hirsutism, and androgenic
lactinaemia (e.g., selective serotonin reuptake inhibitors alopecia. The biochemical confirmation required is a raised
[SSRIs]); or total or free testosterone. A raised LH/FSH ratio is no
• the oestradiol is low; or longer considered useful in the diagnosis because of its
• there is a recent history of uterine or cervical surgery. inconsistency.
This may indicate Asherman syndrome. • Exclude adrenal hyperplasia, hyperprolactinaemia, andro-
• Osteoporosis risk needs to be considered in patients with gen secreting tumours, and Cushing syndrome. If the latter
persistent amenorrhoea. Measurement of bone density is suspected on clinical grounds, refer to an endocrinologist.
may be required and if symptoms have persisted over 12 • Check TFTs, serum prolactin, and free testosterone. If
months then consideration should be given to treatment there is clinical evidence of hyperandrogenism and the
with cyclic combined HRT (off-label use). Advice on total testosterone is greater than 5 nmol/L, check the
adequate dietary intake of calcium should be given. level of 17-hydroxyprogesterone.
• If one of the previous two criteria is met but not the
Polycystic Ovary Syndrome other, order a pelvic USS for ovarian cysts.
GUIDELINES Management
National Institute for Health and Care Excellence. (2013). • Explain what little is known about the syndrome: that
CKS: Polycystic ovary syndrome. cks.nice.org.uk/
polycystic-ovary-syndrome. the pituitary produces excess LH, which stimulates the
Royal College of Obstetricians and Gynaecologists. (2014). ovary to produce excess testosterone which in turn causes
Long-term consequences of polycystic ovary syndrome. acne and hirsutism. Associated with this is a tendency to
“Green Top” clinical guideline, 33. www.rcog.org.uk. insulin resistance, manifested by overweight and the devel-
opment of diabetes.
• Cardiovascular disease (CVD) risk. Check the serum lipids,
blood pressure, waist circumference, and body mass index
• Polycystic ovaries are a common ultrasound finding affect- (BMI) regularly in women over 35. NICE recommends
ing up to 33% of women of reproductive age in the calculating the CVD risk score but it should be noted that
United Kingdom, only a third of whom have clinical or cardiovascular risk calculators have not been validated in
biochemical features of PCOS. PCOS. Hypertension should be treated, but lipid-lowering
• The characteristic features include: agents should only be introduced by a specialist. Advice
• truncal obesity; should be given on weight loss, diet, and exercise, as
• oligomenorrhoea/amenorrhoea/dysfunctional uterine appropriate.
bleeding; • Diabetes. Screen all women with PCOS for impaired
• hirsutism; glucose tolerance and type 2 diabetes mellitus (DM).
• acne; Initially offer an oral glucose tolerance test to all women.
• raised serum-free testosterone or free androgen index; Consider repeating this annually for women at higher
• male-pattern hair loss risk (impaired glucose tolerance, strong family history of
• Patients with PCOS are twice as likely to develop diabetes DM, BMI >30 kg/m2 or 25 kg/m2 in Asian women, and
(Wild et al., 2000). By the age of 40, up to 40% will those with a history of gestational diabetes). Those not
have type 2 diabetes (Lord, Flight, & Norman, 2003). offered an annual oral glucose tolerance test (OGT) should
They have a higher prevalence of features associated with be offered their fasting glucose checked annually or should
metabolic syndrome: hypertension, dyslipidaemia, visceral be offered a OGT every second year.
obesity, insulin resistance, and hyperinsulinaemia. • Snoring. Ask about snoring and sleep apnoea. PCOS
appears to be a risk factor independently of BMI in sleep
Diagnosis apnoea.
• Weight. Advise about weight loss through diet (especially
Consider the diagnosis if two of the following three criteria with a low gastrointestinal [GI] diet) and exercise. Loss
are met: of weight has been reported to result in resumption of
ovulation, improvement in fertility, increased sex hormone history raises the possibility of pelvic infection; swabs are
binding globulin (SHBG), and reduced risk of diabetes needed in those at risk of sexually transmitted infections
and cardiovascular disease. and in those in whom the clinical picture does not suggest
bacterial vaginosis or candida.
Management of Symptoms • In those who are at low risk of sexually transmitted infec-
• Acne. See section on acne, but consider using COC pill tion and who decline examination, treatment can be given
or co-cyprindiol (Dianette) (NICE CKS, 2013). based on clinical history.
• Hirsutism. Physical methods of hair removal (e.g., waxing, • Interpretation of findings:
intense pulsed light [IPL]) and topical treatment with • Smelly white discharge with pH above 4.5: bacterial
eflornithine may be helpful but will not treat the underly- vaginosis.
ing cause. Eflornithine has been reported as resulting in • White curdy discharge, usually with vulval soreness
marked improvement in 32% of women. Women who are and itch, erythema, possibly fissuring and satellite
overweight should be advised to lose weight. First line drug lesions; pH below 4.5: candidiasis.
treatment is with a combination of cyproterone acetate • Smelly yellow or green frothy discharge with pH above
and ethinylestradiol (as Dianette) although a desogestrel- 4.5, perhaps with dysuria: trichomonas.
containing COC may be as effective. Second line treatments • When the clinical picture is not typical of bacterial vagi-
include spironolactone, finasteride, GnRH analogues, and nosis or candidiasis, or the patient is under age 25 or has
metformin. Contraception is required while on spirono- had a new sexual partner in the last year, take swabs:
lactone as there is a theoretical risk of feminizing a male • HVS for Trichomonas, the clue cells of bacterial vagi-
foetus (Farquhar et al., 2000). Warn that benefit is unlikely nosis, and Candida.
before about 6 months of any of these treatments. • Endocervical swab for chlamydia and gonorrhoea testing
• Lack of regular periods. This may predispose to endome- with nucleic acid amplification test (NAAT).
trial hyperplasia and later carcinoma and so all women • Patients with sexually transmitted infections are best
who have been amenorrhoeic should have their endome- managed in the genitourinary medicine (GUM) clinic.
trial thickness assessed. RCOG (UK) recommends that
a withdrawal bleed be induced every 3 to 4 months with Management: Candida
progestogens (for at least 12 days) or the COC. Women
who do not have withdrawal bleeds should be referred GUIDELINE
to a gynaecologist.
• Lack of ovulation. Refer to a gynaecologist. Metformin Clinical Effectiveness Group (Association for Genitourinary
Medicine and Medical Society for the Study of Venereal
500 mg three times a day, although unlicensed for this Disease). (2007). Management of vulvovaginal candidiasis.
use, can induce ovulation and reduce fasting insulin con- www.bashh.org/guidelines.
centrations, blood pressure, and low density lipoprotein
(LDL) cholesterol. However, RCOG (UK) states “long
term use of insulin sensitizing agents cannot as yet be • Give general advice to avoid local irritants, and avoid
recommended.” Patients are increasingly aware and ask tight-fitting synthetic clothing (Bingham, 1999).
for metformin treatment but this is ahead of the evidence • Treat only if symptomatic.
and outside the product licence (Harborne et al., 2003). • Prescribe a topical imidazole. Cure rates are 80% to 95%.
• Infertility. Refer. Other treatments recommended by NICE Give it either as a single dose or as a short course at night,
are metformin and clomiphene in combination, ovarian usually for 3 nights. Oral imidazoles are no more effective
drilling and gonadotrophins (NICE, 2013). (Watson et al., 2002) but they have more side effects. In
addition, they are contraindicated in pregnancy.
• Male partners. There is no evidence to support the treat-
SELF-HELP GROUP
ment of asymptomatic male sexual partners (Bisschop
Verity. The polycystic self-help group; www.verity-pcos.org.uk. et al., 1986).
• COC. Do not stop the COC. Its use is not associated
with candidiasis.
• Pregnancy. Asymptomatic colonization is more common
Vaginal Discharge in pregnancy (30%–40%). Treat symptomatic patients
with topical azoles, but a longer course may be necessary.
• Ask about the type of discharge, the timing, whether it
smells, whether it itches, and whether there is pelvic pain. Recurrent Candidiasis (Four or More
• Ask the woman what she thinks it is and, when asking Symptomatic Episodes per Year)
whether it seems related to sexual activity, ask tactfully
about the number of sexual partners. • Check the urine for glucose approximately 2 hours after
• Examine the vulva and test the vaginal pH using narrow a meal or glucose load. Note that this would be inadequate
range pH paper. Bimanual examination is needed if the as a screening test for diabetes in any other situation.
• Exclude other risk factors: iron deficiency anaemia, dose: or intravaginal clindamycin cream (2%) once daily
thyroid disease, frequent antibiotic use, corticosteroid for 7 days. The cure rate is 70% to 80%.
use, immunodeficiency. • Pregnancy and lactation. Symptomatic women in pregnancy
• Screen for other vaginal infections. should be treated with metronidazole. Lactating women
• Give either: should be treated with intravaginal metronidazole.
• an imidazole pessary daily for 2 weeks then weekly for 6
months; or use a pessary only when an infection is most Pelvic Inflammatory Disease
likely (e.g., after intercourse or when taking a broad-
spectrum antibiotic). These treatment regimens are empiri- GUIDELINE
cal and are not based on randomized controlled trials; or
• fluconazole 150 mg orally every 3 days for three doses British Association for Sexual Health and HIV. (2011). UK
National Guideline for the management of pelvic inflammatory
then weekly for 6 months. Approximately 90% of women disease. www.bashh.org.
remain disease free for 1 year. If there is relapse between
doses, consider giving twice weekly fluconazole 150 mg.
• Consider giving cetirizine 10 mg daily for 6 months or
zafirlukast 20 mg twice a day for 6 months. Allergy may be • PID is usually the result of ascending infection from
an important component especially in women with atopy. the endocervix. Neisseria gonorrhoeae and Chlamydia tra-
• Candida of the gut. There is no evidence to suggest that chomatis have been identified as causative agents, whilst
eradication of candida from the gut is helpful. Mycoplasma genitalium, anaerobes, and other organisms
• Probiotics/Lactobacillus. Evidence does not support the commonly found in the vagina may also be implicated.
use of oral or topical Lactobacillus in prevention of vul- • PID has a high morbidity; about 20% of affected women
vovaginal candidiasis. become infertile, 20% develop chronic pelvic pain, and
• Alteration of vaginal pH. Suggest a trial of a pH-lowering 10% of those who conceive have an ectopic pregnancy
agent (e.g., AciGel). (Metters et al., 1998).
• Repeated episodes of PID are associated with a four- to
Management: Bacterial Vaginosis sixfold increase in the risk of permanent tubal damage
(Hills et al., 1997).
GUIDELINE • PID may be asymptomatic.
• Of women with PID, 10% to 20% will develop
British Association for Sexual Health and HIV (2012). UK perihepatitis.
National Guideline for the management of bacterial vaginosis.
www.bashh.org.uk/guidelines. • A delay of only a few days in receiving treatment mark-
edly increases the risk of long-term sequelae (Hills et al.,
1993). Because of this, and the lack of definitive diagnostic
criteria, a low threshold for the empirical treatment of
PID is recommended.
• Bacterial vaginosis is characterized by a reduction in lac-
tobacilli and an overgrowth of predominantly anaerobic Diagnosis
organisms (Gardnerella vaginalis, Mycoplasma hominis,
Prevotella species, Mobiluncus species). • Symptoms can include fever, lower abdominal pain, deep
• Approximately 50% of women with the condition are dyspareunia, abnormal bleeding, abnormal vaginal or
asymptomatic. cervical discharge. On examination there may be cervical
• It is not regarded as sexually transmitted. It can arise and excitation and adnexal tenderness.
remit spontaneously in women regardless of sexual activ- • Clinical diagnosis is correct in only 65% to 90% of
ity. However, it is more common in women liable to cases compared to laparoscopy. Even laparoscopy can
sexually transmitted infections. miss mild cases but it remains the most reliable investi-
• Partners. No evidence has been found supporting the gation. In the United Kingdom, it is not recommended
treatment of partners of women affected by bacterial in all cases but should be performed when there is diag-
vaginosis (Potter, 1999). nostic doubt.
• Treatment is indicated for: • If not referring, perform the following:
• symptomatic women; • Endocervical swabs for gonococcus and chlamydia.
• pregnant women with a history of recurrent miscarriage; Negative microbiological tests do not exclude a diag-
• women undergoing termination of pregnancy. They nosis of PID; in at least 50% of cases no specific organ-
are at greater risk of PID if the bacterial vaginosis is ism is identified. Screen for the same diseases in any
untreated. sexual partners.
• Give metronidazole 400 to 500 mg twice a day for 5 to • Urinalysis, pregnancy test, and mistream specimen of
7 days; or intravaginal metronidazole gel (0.75%) once urine (MSU) to look for other causes of lower abdomi-
daily for 5 days; or metronidazole 2 g as a single oral nal pain.
• Blood for erythrocyte sedimentation rate (ESR) or PATIENT INFORMATION

C-reactive protein (CRP). A raised level would support
Download the RCOG leaflet “Acute Pelvic Inflammatory
the diagnosis (Miettinen et al., 1993).
Disease”. Available at www.rcog.org.uk.
• Recommend rest and provide adequate analgesia.
• Recommend that unprotected intercourse be avoided until
the woman and her partner(s) have completed treatment
and follow-up. If it is not possible to screen for gonor-
rhoea and chlamydia in the sexual partner(s) give empirical Premenstrual Syndrome (PMS)
treatment for both gonorrhoea and chlamydia (Groom
et al., 2001; Haddon et al., 1998). GUIDELINE
• If an IUD is in situ, and this is the preferred method of National Institute for Health and Care Excellence. (2014).
contraception, leave it in place. Remove it only if the CKS: Premenstrual syndrome. cks.nice.org.uk/
condition fails to respond to treatment and other causes premenstrual-syndrome.
of pain have been excluded (Larsson & Wennergren, 1981; Royal College of Obstetricians and Gynaecologists.
Soderberg & Lingren, 1981; Teisala, 1989). (2016). Management of Premenstrual Syndrome. Green-top
Guidline No. 48.
• Drug treatment:
• Ofloxacin 400 mg twice a day plus metronidazole
400 mg twice a day for 14 days.
• In women at high risk of gonococcal infection: ceftri- • Ninety-five percent of women have symptoms related to
axone 500 mg intramuscularly as a single dose then their menstrual cycle; in 5% they are disabling. More
14 days of oral doxycycline 100 mg twice a day and than 150 symptoms have been reported.
metronidazole 400 mg twice a day. • Diagnosis depends not on the type of symptom but on
• Review daily in case admission becomes necessary. the timing of the symptoms and their cyclicity. Symptoms
present 1 to 14 days prior to menstruation and disappear
Admission at the onset or by the day of the heaviest flow. If behav-
ioural symptoms persist throughout the cycle, then con-
• Admit patients if: sider a psychological/psychiatric disorder.
• the illness is severe; • Ask the patient to keep a menstrual and symptom diary
• not responding after 3 days of oral therapy; for at least 2 months. She can score symptoms according
• unable to tolerate oral drugs; to their severity.
• a pelvic mass is present; • Listen sympathetically. It is a real entity. This alone can
• the patient is pregnant; be therapeutic.
• there is diagnostic uncertainty; • Give simple advice:
• the patient suffers from immunodeficiency; • Dietary advice. Explain that some women seem more
• the patient is above the usual age for PID. An alterna- sensitive to blood sugar changes at this time of the
tive diagnosis (e.g., ovarian carcinoma) is more likely. cycle, and this may be the cause of food cravings, panic
reactions, irritability, and aggression. Advise patients
Follow-Up for All Patients to reduce their refined sugar intake as well as their
caffeine intake. Small frequent carbohydrate snacks
• Review within 72 hours. Refer if there is little improvement. seem effective in about 30% of women.
• Review again in 4 weeks to: • Stress management. Explain that although it is not the
• ensure there is adequate clinical response; cause of the syndrome, stress is harder to cope with
• ensure there was compliance with the antibiotics; during the premenstrual period. Relaxation methods,
• trace, investigate, and treat the woman’s partner(s) yoga, meditation, and exercise can be of benefit.
if a sexually transmitted disease (STD) is diagnosed
(Robinson & Kell, 1995). A study found that 60% of Therapeutic Options
contacts had relevant infections, and that in most of
these it was asymptomatic (Kamwendo et al., 1993). • SSRI. Consider using an SSRI. There is good evidence
Consider referral to a GUM clinic for contact tracing; of their efficacy (Wyatt, Dimmock, & O’Brien, 2002)
• stress the significance of the disease and the sequelae. and they may be effective if used in the luteal phase alone.
• Discuss with the patient her need for safer sex. Side effects may limit their acceptability.
• Advise the patient to ask for antibiotic cover (e.g., doxy- • Breast tenderness, bloating, and irritability may be improved
cycline 200 mg stat or metronidazole 2 g stat) if she ever by taking spironolactone 100 mg daily from day 12 until
needs a termination of pregnancy (TOP) or a dilatation the onset of menstruation.
and curettage (D&C). • NSAIDs given for 7 days prior to menses and 4 days after
• Advise the patient to seek advice promptly if the symptoms the onset have been shown to improve a number of symp-
return. toms but not breast tenderness.
• Aerobic exercise. Low and moderate exercise (50%–70% TABLE

of maximum) three times a week for 45 minutes has been 13.1 Ovarian Cancer Risk
shown to reduce symptoms.
Number of First-Degree Lifetime Risk of
• Oestrogens may improve or in some cases worsen the situ-
Relatives With Ovarian Developing Ovarian
ation. The COC can prove helpful, especially in those
Cancer Cancer
women requiring contraception. Women who experience
symptoms during their pill-free week can take three con- 0 1%
secutive packets back to back. A lower dose of oestrogen, 1 5%
as HRT, may help some women but cyclical progestogens
must also be used in women with an intact uterus. 2 15%
• Danazol has been shown to be effective. Adverse effects are >2 50%
common (masculinization and weight gain) with continu-
ous use but may not occur with luteal phase use alone.
Danazol should normally be initiated by a specialist. a. two or more first-degree relatives with ovarian cancer; or
• Vitamin B6 (pyridoxine) 50 to 100 mg daily. This recom- b. one first-degree relative with ovarian cancer and one
mendation is based on limited information from poor first-degree relative with breast cancer diagnosed under
quality trials, but the evidence suggests that B6 may be 50 years of age; or
of benefit. c. one ovarian cancer and two breast cancers diagnosed
Breast tenderness. Consider bromocriptine 1.25 mg twice under 60 years in first-degree relatives;
a day, but adverse effects are common. • known BRCA-1 or BRCA-2 mutations in the family; or
Note: Progestogens have been found unhelpful in the treat- • three colorectal cancers, at least one diagnosed under
ment of PMS. 50 years of age, and one ovarian cancer, all first-degree
Note: Evening primrose oil. There is no evidence that it is relatives of each other;
helpful in PMS. • affected relatives with any of the above combinations
who are related by second degree through an unaffected
Patients Not Responding male and there is an affected sister (i.e., paternal trans-
mission is occurring).
• Refer to a gynaecologist for the consideration of suppres- Note: A first-degree relative is a parent, sibling, or child; a
sion of ovarian function. Treatment options include high- second-degree relative is a grand-parent, aunt or uncle, or cousin.
dose danazol, high-dose oestrogen, and GnRH analogues • Weak family history. Women with one close relative with
with addback HRT. epithelial ovarian cancer diagnosed before the age of 50
may also wish to be referred for advice but may not be
PATIENT SUPPORT offered regular screening.
National Association for Premenstrual Syndrome (NAPS),
www.pms.org.uk. PATIENT INFORMATION
Premenstrual Society, PO Box 429, Addlestone, Surrey
KT15 1DZ, helpline 01932 872560 (11 am–6 pm, weekdays Cancer Research UK. Ovarian cancer screening. www.
only). cancerresearchuk.org.
Ovarian Cancer Screening Gestational Trophoblastic Hydatidiform

• Many women will request screening for ovarian cancer
Mole and Choriocarcinoma
on the basis of their family history. Some useful figures
GUIDELINE
can be used to reassure many women (Table 13.1).
• There is currently no national screening programme for Royal College of Obstetricians and Gynaecologists. (2010).
ovarian cancer. Despite early promising results, the UK The management of gestational trophoblastic disease.
“Green Top” clinical guideline, 38. www.rcog.org.uk.
Collaborative Trial of Ovarian Cancer Screening
(UKTOCS) has so far failed to show clearcut evidence
of benefit from a screening programme.
• Women at higher risk may be eligible for screening and
genetic testing; and if they fall into one of the following • Gestational trophoblastic disease is uncommon in the
groups, may be referred to the UK Familial Ovarian Cancer United Kingdom, with a calculated incidence of 1/714
Screening Study (UKFOCSS) or for genetic testing by live births. The incidence is highest in women from Asia
the local genetics service. Women considered at higher (incidence 1/387) compared to non-Asian women (inci-
risk are those with: dence 1/752).
• In the United Kingdom, there is an effective registration • Consider taking serial FSH measurements in the follow-
and treatment programme with high cure rates. ing circumstances:
• Once the diagnosis of hydatidiform mole has been made, • Women with symptoms under the age of 40 because
follow-up will be needed for between 6 months and 2 years. of the implications of premature ovarian failure
The aim is to detect the occurrence of choriocarcinoma. • Women with symptoms who have had a hysterectomy
• Once treated, serum estimations of levels of human cho- with ovarian conservation, who thus are at risk of an
rionic gonadotrophin (HCG) should be performed accord- early menopause
ing to protocols from the regional expert units (London, • Take FSH levels:
Sheffield, and Dundee). • 4 to 8 weeks apart when the woman is not on HRT
• The COC pill and hormone replacement are safe to use or hormonal contraception. FSH levels of greater than
once HCG levels have reverted to normal. 30 IU/L are generally considered to be in the post-
• Women should be advised not to conceive until the HCG menopausal range;
level has been normal for 6 months or follow-up has been • Abnormal bleeding. Refer women, without starting HRT,
completed (whichever is the sooner). The risk of further if they give a history of abnormal bleeding (e.g., sudden
recurrence is 1 in 55. After any further pregnancy, urine change in menstrual pattern, intermenstrual bleeding,
and blood samples should be taken to exclude disease postcoital bleeding, or a postmenopausal bleed) (Hope
recurrence. et al., 1999; Korhonen et al., 1997).
• Women who undergo chemotherapy should not attempt
to conceive until a year after completion of treatment. Initial Management
• Refer all women with persistent bleeding:
• after the evacuation of retained products of conception; • Counsel women about the menopause. Ideally all peri-
• after normal pregnancy; menopausal women should be given the opportunity to
• after miscarriage. discuss the menopause, with particular reference to the
• Send for histology any products of conception passed at symptomatology, common misconceptions, and treatment
home. options.
• If no products of conception are available, check HCG • Take the opportunity to discuss lifestyle issues (smoking
4 weeks after the loss of the pregnancy. cessation, diet, and exercise). HRT is no longer recom-
mended as a general prophylactic measure because of the
increased risk of breast cancer, but individual women
PATIENT INFORMATION
may judge that, for them, the benefit outweighs the risks
The Hydatidiform Mole and Choriocarcinoma UK information (discussed later).
service, www.hmole-chorio.org.uk.
• Osteoporosis. Identify those patients at high risk of osteo-
porosis and investigate and treat, if appropriate. HRT is
no longer indicated for the prevention of osteoporosis
except in those with an early menopause.
SCREENING CENTRES • Vasomotor symptoms:
Trophoblastic Tumour Screening and Treatment Centre. • HRT is extremely effective in controlling these
Department of Oncology, Charing Cross Hospital, Fulham symptoms (MacLennan, Lester, & Moore, 2001).
Palace Road, London W6 8RF, tel. 020 8846 1409, fax 020 Because of the possible harms, use the lowest effective
8748 5665. dose for the shortest time possible (see upcoming
Trophoblastic Tumour Screening and Treatment Centre.
Weston Park Hospital, Whitham Road, Sheffield S10 2SJ,
discussion).
tel. 0114 226 5202, fax 0114 226 5511. • SSRIs and related drugs. A meta-analysis has found
Hydatidiform Mole Follow-Up (Scotland) Department of some evidence of benefit, with paroxetine perform-
Obstetrics and Gynaecology, Ninewells Hospital, Dundee ing best. However, even paroxetine reduced flushes by
DD1 9SY, tel. 01382 632748, fax 01382 632096. only one or two a day compared to placebo (Nelson
et al., 2006). Venlafaxine has also been shown to be
superior to placebo for treating vasomotor symptoms
(Caan et al, 2015).
The Menopause • Gabapentin. A trial of 12 weeks of 900 mg/day reduced
Establishing the Diagnosis the hot flash composite score by 54% against 31% with
placebo (Guttuso et al., 2003). The use of gabapentin
• No investigations are routinely indicated. FSH levels for menopause is currently restricted to specialist centres.
fluctuate markedly during the perimenopause and so are • Consider clonidine for those unable to take HRT or
of limited value in symptomatic women in whom a clini- an SSRI (Edington & Chagnon, 1980; Nagamani,
cal history can form the basis of diagnosis. There is little Kelver, & Smith, 1987).
place for LH, oestradiol, and progesterone estimation in • Black cohosh, with or without other natural remedies,
clinical practice (Hope, Rees, & Brockie, 1999). has been shown to be ineffective (Newton et al., 2006).
• Vaginal dryness. Treatment options include: TABLE Minimum Doses of Oestrogen Needed for
• HRT (see upcoming discussion); 13.2 Bone Conservation
• local oestrogens. These can be given per vaginum daily
Drug Dosage Frequency
until symptoms have ceased, then twice weekly for
3 to 6 months. Systemic progestogens do not need to Oestradiol 1–2 mg Daily*
be given alongside local oestrogens as there is no evi- Oral conjugated equine 0.625 mg Daily
dence that they cause endometrial proliferation; oestrogens
• vaginal lubricants. These include K-Y jelly, Replens
Transdermal oestradiol 50 mg Daily
(a nonhormonal aqueous moisturizer), and Senselle patch
(a water-based lubricant).
• Urinary symptoms: Oestradiol gel 1.5 g (two Daily
measures)
• Symptoms related to urogenital atrophy will respond
to oestrogen by any route. Maximum benefit will not Oestradiol implants 50 mg 6 monthly
be seen for at least the first month, and may take up *Although 1 or 2 mg oral oestradiol can be used for prevention of osteo-
to a year (Cardozo et al., 1998). porosis, the bone protective effect is dose related. Some products are
• Stress incontinence is unlikely to respond to oestrogen licensed for osteoporosis prevention at 2 mg only, while others are licensed
at 1 and 2 mg.
replacement therapy alone. Refer patients who are suf-
ficiently troubled to a gynaecologist.
• Psychological symptoms. Psychological symptoms may relate
to a woman’s hormonal status but, equally, the menopause
may become a scapegoat for patients with underlying months. Improvement in mental function is less certain.
emotional problems (Hunter, 1996). Counselling may The mental health of women with vasomotor symptoms
be helpful but many will improve when their physical seems to improve on HRT but those without vasomotor
symptoms improve (Gath & Iles, 1990). Depression may symptoms worsen, with poorer physical functioning and
need treatment in its own right. lower energy levels compared to those on placebo (Hlatky
et al., 2002). Overall, HRT does not improve the quality
of life (Hays et al., 2003).
Hormone Replacement Therapy • Osteoporosis. Combined HRT protects against hip fracture
in unselected postmenopausal women and reduces the
GUIDELINE risk of all fractures. This represents five fewer hip fractures
per 10,000 person-years, a number needed to treat (NNT)
National Institute for Health and Care Excellence. (2015). of 2000 for 1 year (Rossouw et al., 2002). In women at
Menopause: Diagnosis and management. NICE clinical
guideline, 23. www.nice.org.uk. high risk of fracture the absolute benefit will be greater.
The benefit is dose related (Table 13.2). However, the
benefit on fracture risk is lost within 3 years of stopping
HRT (Heiss et al., 2008).
• HRT may be indicated in the following: • Prevention of carcinoma of the colon. HRT appears to reduce
• Women with an early menopause (<45 years of age) the risk of developing colonic carcinoma by 20% (RR
mainly for the prevention of osteoporosis. It may also 0.80 [95% CI 0.74–0.86]) (Nelson et al., 2002).
give some protection in this young age group against
CVD. It is usually given until age 50. Risks
• Women under the age of 65 with severe vasomotor • CVD. HRT does not increase risk of CVD when given
and other symptoms of the menopause who understand to women under the age of 60 nor does it affect the risk
the risks and are prepared to take HRT for a limited of dying from CVD. If cardiovascular risk factors are well
period. Analysis of the Women’s Health Initiative results managed, then they are not a contraindication to using
has shown no increase in breast cancer, myocardial HRT. Oral oestrogen appears to be associated with a
infarction, or stroke in women under 60 years of age small increased risk of stroke but no increase is seen with
who use HRT for 5 years or less (Rossouw et al., 2007). the transdermal route.
• Record that the risks of HRT have been explained and • Breast cancer. The use of combined HRT in healthy post-
an informed decision taken by the patient. Review the menopausal women is associated with an increase in breast
decision at least annually. cancer risk (RR 1.24 [95% CI 1.01–1.54]) (Rossouw
et al., 2002) but this is not the case with oestrogen-only
treatment. The increase in risk is related to the duration
Benefits and Risks
of treatment and the risk decreases again after stopping
Benefits treatment.
• Symptom relief. Improvement in vasomotor symptoms • Endometrial cancer. Unopposed oestrogen is associated
occurs in a few weeks and in vaginal symptoms in 3 with an extra five cases per 1000 women over 5 years.
The use of combined preparations reduces but does not anticoagulation, though that has its own risks. A trans-
eliminate this risk, with an estimated risk of two extra dermal route may reduce the risk of VTE. A specialist
cases per 1000 women over 10 years. opinion would be wise in those with thrombophilia.
• Ovarian cancer. Long-term combined or oestrogen-only • Past history of endometrial cancer. Refer women who want
HRT is associated with a small increased risk of ovarian to consider HRT to the appropriate specialist. Although
cancer. This excess risk disappears within a few years of conventional advice is that oestrogens are contraindicated,
stopping HRT. small studies of endometrial cancer survivors have not
• Venous thromboembolism (VTE). Oral HRT increases the risk shown an adverse effect on survival.
of VTE but transdermal HRT does not increase risk above • Diabetes and gall bladder disease. Use a transdermal preparation.
the baseline population risk. Therefore consider the trans- • Liver disease. Refer to a specialist clinic.
dermal route for women with additional risk factors for VTE • Endometriosis. Refer to a specialist clinic.
e.g. women with a BMI over 30. Consider referring women • Fibroids. HRT may enlarge fibroids, causing heavy or
who are at high risk of VTE (e.g., due to family history of painful withdrawal bleeds. Warn patients to report pain
hereditary thrombophilia or VTE) to a haematologist for or pressure effects on the bladder or bowel.
assessment. • Migraine is not a contraindication to HRT. There is no
• Dementia. The affect of HRT on dementia risk is evidence that the risk of stroke is increased by the use of
unknown. HRT in women with migraine (Bousser et al., 2000).
• Hypertension. There is no evidence that HRT raises blood
Other Issues to Discuss pressure (Rees & Purdie, 1999).
• Explain that taking HRT means that cyclical bleeding Initial Assessment
will return if the woman has an intact uterus and a cycli-
cal preparation is used. Irregular bleeding is common • Take a history and assess the woman’s menopausal status.
with continuous preparations. • Check for contraindications to HRT therapy, especially
• Explain that evidence from randomized trials suggests a history of breast cancer or VTE.
that HRT does not cause extra weight gain in addition • Check blood pressure (BP), BMI, and serum lipids.
to that normally gained at the time of the menopause • Advise the woman about breast awareness. Check that
(Norman, Flight, & Rees, 2001). mammography screening is in place if over 50 years.
Mammography is not needed before commencing
Contraindications HRT unless the woman is at high risk of breast cancer
• Oestrogen replacement therapy is absolutely contraindi- (Hope et al., 1999).
cated in very few patients. Even in women in whom • Check that regular cervical screening is taking place.
treatment appears contraindicated, oestrogen therapy may • Give lifestyle advice, as discussed.
be prescribed under supervision of a specialist menopause
clinic if the woman’s symptoms are particularly severe
Treatment
(Rees & Purdie, 1999).
• Absolute contraindications include: Oestrogens
• acute-phase myocardial infarction, pulmonary embo- • Start at the lowest possible dose of oestrogen (especially
lism, or deep vein thrombosis (DVT); in older women who tend to get more oestrogenic side
• active endometrial or breast cancer; effects) and increase at 3-monthly intervals, if necessary,
• pregnancy; to achieve optimum symptom control.
• undiagnosed breast mass; • Give oestrogens continuously, and only give them without
• uninvestigated abnormal vaginal bleeding; progestogens if the woman has had a hysterectomy.
• severe active liver disease.
Note: Many contraindications given in prescribing data Progestogens
sheets are derived from high-dose combined oral contracep- • These must be added for endometrial protection in women
tives and are, in the view of most experts, not applicable to with a uterus. Most products contain either C-19 deriva-
HRT (Rees & Purdie, 1999). tives (norethisterone/levonorgestrel), which are more
androgenic, or C-21 derivatives (medroxyprogesterone
HRT Use in Women With Comorbidity acetate/dydrogesterone), which are less androgenic.
(Relative Contraindications) • Change to a less androgenic preparation if the patient is
• History of thromboembolic disease. Women with a personal troubled by progestogenic side effects.
or family history of thromboembolism should be offered
screening for thrombophilia before starting. Those with Perimenopausal Women With an Intact Uterus
a personal history of VTE should not take HRT unless • Use a cyclical regimen (monthly or 3-monthly). The
the woman decides that, for her, the benefits outweigh majority of women will have a bleed toward the end of
the risks (RCOG, 2004). She may choose prophylactic the progestogen phase.
• No bleeding whilst taking a cyclic regime reflects an atro-

Postmenopausal Women With an Intact Uterus phic endometrium and occurs in 5% of women, but
• Use: pregnancy needs to be excluded in perimenopausal women.
• a cyclical regimen; or • Refer if there is:
• a continuous combined regimen. Continuous regimens • a change in the pattern of withdrawal bleeds and break-
induce an atrophic endometrium and so do not produce through bleeding that persists for more than 3 months;
a withdrawal bleed, although irregular bleeding can occur • unexpected or prolonged bleeding that persists for more
in the first 4 to 6 months. Bleeding should be investigated than 4 weeks after stopping HRT: refer urgently.
if it persists for longer than 6 months, becomes heavier
rather than less, or occurs after amenorrhoea; or Bleeding on Continuous Combined Therapy or
• tibolone. This combines oestrogenic and progestogenic Tibolone
activities, and weak androgenic activity. It must only • Explain to patients that the risk of bleeding is 40% in
be started at least 1 year after the menopause. There the first 4 to 6 months.
is evidence that it improves libido (Kokcu et al., 2000). • Make sure the patient was at least 1 year postmenopausal
before she started the regimen.
Alternative Modes of Delivery • Bleeding beyond 6 months requires further investigation.
• Oestrogen implants. Repeat every 4 to 8 months. Occasion- • Bleeding that occurs after a period of amenorrhoea requires
ally vasomotor symptoms can return despite supraphysi- further investigation.
ologic plasma concentrations of oestradiol (tachyphylaxis).
Check that plasma oestradiol levels have returned to normal Oestrogen-Related Side Effects
(<1000 pmol/L) before inserting a new implant. • Oestrogenic side effects include fluid retention, bloating,
• Transdermal patches and gels. These avoid the first-pass metabo- breast tenderness and enlargement, nausea, headache, leg
lism in the liver and deliver a more constant level of hormone. cramps, and dyspepsia.
Patches come as either reservoir or matrix patches. Skin • Encourage the patient to persist with therapy for 12 weeks,
reactions are less common with the matrix patches. as most side effects resolve with time.
• IUS. The LNG-IUS is licensed for 4-year usage for the • For persistent side effects consider:
delivery of progestogen to protect the endometrium. A • reducing the dose; or
5-year follow-up concludes that the LNG-IUS effectively • changing the oestrogen type (swap between the two
protects against endometrial hyperplasia (Varila, Wahl- main forms of oestrogen: oestradiol and conjugated
strom, & Rauramo, 2001). It provides contraception, equine oestrogens); or
and it is the only way a nonbleed regimen may be achieved • changing the route of delivery.
in the perimenopause. • Nausea and gastric upset. Change the timing of the oes-
trogen dose (e.g., try taking it with food or at bedtime).
Managing the Side Effects of HRT Progestogen-Related Side Effects
Bleeding • Progestogen-related side effects tend to occur in a cyclic
Patients on HRT should only be referred urgently (in the pattern during the progestogenic phase of cyclical HRT.
United Kingdom under the 2-week rule) if the bleeding Continuous combined products contain lower doses of pro-
continues after the HRT has been stopped for 4 weeks. gestogens and side effects are less likely. Side effects include
fluid retention, breast tenderness, headaches, mood swings,
Bleeding on Cyclic Combined Therapy depression, acne, lower abdominal pain, and backache.
• Check when the bleeding occurs. These regimens should • Encourage the patient to persist with therapy for about
produce regular predictable bleeds starting toward or soon 12 weeks as some side effects will resolve.
after the end of the progestogenic phase. • Various changes in the progestogens may be helpful, but
• Consider poor compliance, drug interactions, or remember not to reduce the dose or duration below that
GI upset. which protects the endometrium. Options:
• Try stopping HRT to see if it is the cause of the a. Reduce the duration but not below 10 days per cycle.
bleeding. b. Reduce the dose of progestogen, or
• If bleeding problems are due to HRT, alter the progestogen: c. Change the progestogen type (either C-19 or C-21
• Heavy or prolonged bleeding: increase the dose or dura- derivatives; see earlier discussion), or
tion of progestogen or change the type of progestogen d. Change the route of progestogen (oral, transdermal,
to a more androgenic type (see previous discussion). vaginal, or intrauterine), or
• Bleeding early in the progestogenic phase: increase the e. Reduce the frequency of how often the progestogen
dose or change the type of progestogen. is taken by switching to a long-cycling regime in which
• Painful bleeding: change the type of progestogen. progestogens are administered for 14 days every 3
• Irregular bleeding: change the regimen or increase the months. This is only suitable for women with scant
dose of progestogen. periods or who are postmenopausal, or
f. Change to a continuous combined therapy, which often Hormone Replacement Therapy

reduces progestogenic side effects with established use; and Contraception
it is suitable only for postmenopausal women however.
• Perimenopausal women cannot be assumed to be infertile.
Follow-Up of Women on Hormone • Routine HRT preparations do not suppress ovulation
Replacement Therapy and are not contraceptive.
• Contraception should be continued for 1 year after the
• See after 3 months, then every 6 months. last menstrual period (LMP) for women over 50 years,
• Check for compliance, bleeding patterns, and side effects. or for 2 years after the LMP for women under the age
• Check that the patient is up to date with her cervical of 50 years.
smears and mammograms. • Perimenopausal women may use:
Note: There is no need to check the BP except as good a. barrier methods, IUD, or LND-IUS, as well as HRT; or
practice in any well-person screening. b. the low-dose COC instead of HRT; or
c. the POP, possibly combined with HRT, although there
How Long to Continue? are theoretical concerns that the oestrogen component
of HRT may interfere with the action of the POP on
• Symptomatic relief. Guidelines recommend that HRT the cervical mucus (Pitkin, 2000).
be given “for the shortest possible time” but there is
no way of predicting how long this is. Most authori- When Has the Menopause Occurred When
ties recommend that HRT given for symptom relief be Masked by Hormone Replacement Therapy or
stopped within 5 years. In the World Health Organisation Combined Oral Contraceptive?
(WHO) trial, HRT was stopped after 5.7 years. Half the
women with vasomotor symptoms at the start of the • It is important to know this so that a realistic idea of
trial reported moderate or severe symptoms after stopping how long the woman needs to remain on contraception
(Ockene et al., 2005). If troublesome symptoms recur on can be calculated (see previous discussion); 80% of women
withdrawal, HRT can be restarted for 6 to 12 months are postmenopausal by the age of 54 years (DTB, 1994b).
at a time. • Discontinue HRT or COC for 6 to 8 weeks, then check
• Osteoporosis prevention when no more appropriate alterna- FSH, and repeat after a further 4 to 8 weeks (Gebbie,
tives exist. Five-year treatment is the minimum period for 1998). If both FSH levels are above 30 IU/L, stop con-
which benefit has been shown, yet after this the risks, traception after a further year. If a spontaneous period
especially of breast cancer, rise. The fact that the benefit occurs, or FSH is less than 30 IU/L, continue contracep-
rapidly drops off after stopping HRT must be set against tion and repeat the test in 1 year.
the risks associated with longer term use. • Women on the POP. Check the FSH without stopping
the POP.
Stopping Treatment
PATIENT ORGANIZATIONS
• HRT may be stopped abruptly or gradually. There is no
evidence that either approach is better in terms of pre- British Menopause Society. 4-6 Eton Place, Marlow, Bucks
SL7 2QA, tel. 01628 890199; www.thebms.org.
venting the return of symptoms.
• For gradual reduction, reduce the strength of oestrogen
every 1 to 2 months, then take it on alternate days for 1
to 2 months, then stop. At this stage: Cervical Screening
• if using a calendar pack, take alternate tablets from
the pack so that the regimen includes progestogen for
GUIDELINES
the second half of the cycle;
• if using a patch, cut the patch in half once the lowest A summary of updated national guidelines within the cervical
dose has been reached. If using the half-patch cycli- screening programme is available at www.cancerscreening.
nhs.uk/cervical.
cally, use the progestogen-containing half-patch for National Institute for Health and Care Excellence. (2017).
the second half of the cycle. CKS: Cervical screening. cks.nice.org.uk/cervical-screening.
• Review after a few months. If troublesome symptoms
have recurred, consider restarting therapy at the lowest
possible dose and titrating up as necessary. If only local
symptoms persist, use a vaginal preparation. • The NHS screening programme requires that all women
• Stopping HRT prior to surgery. The RCOG (2004) does between the ages of 25 and 64 are offered a smear:
not consider that this is necessary but recommends pro- • Aged 25: first invitation
phylactic measures instead: antithromboembolic stockings • 25–49: 3 yearly
and low molecular weight heparin. • 50–64: 5 yearly
• 65+: only screen those who have not been screened TABLE Terminology Used During Cervical
since the age of 50 or have had a recent abnormal test. 13.3 Screening
• The advisory committee on cervical screening reviewed
Cytological terms Histological equivalent*
the policy on screening (June 2009) and concluded that
there should be no change in policy on age of starting Mild dyskaryosis CIN 1 (mild dysplasia) outer
screening and that the harms of screening under this age 1/3 of epithelium
outweigh the benefits. Moderate dyskaryosis CIN 2 (moderate dysplasia)
• Evidence for the programme: 1/3 to 2/3 of epithelium
• In 2000, there were 2424 new registrations for invasive Severe dyskaryosis CIN 3 (severe dysplasia or
cervical cancer in England (National Statistics, Cancer carcinoma in situ) full
registrations in England, 2000). thickness of the epithelium
• Cervical cancer incidence fell by 42% between 1988 with breakdown of structure
between basement
and 1997 (England and Wales). This fall was directly
membrane and epithelium
related to the cervical screening programme (National
Statistics, 2000). CIN, Cervical intraepithelial neoplasia.
*The correlation between status on smear and on histology is not close.
• In 1995, there were 10.4/100,000 newly diagnosed
cases, by 1999 this had fallen to 9.3/100,000 (National
Statistics, 1999).
• Cervical cancer screening saves approximately 4500 • Advise the woman to continue her contraception, including
lives in England and prevents 3900 cases per year the COC; pregnancy makes management more difficult.
in the United Kingdom (Peto et al., 2004; Sasieni
et al., 1996). Follow-Up After Colposcopy and Treatment
• The major risk groups are:
• women with many sexual partners or whose partners • Follow-up at 6 months (by gynaecologist).
have had many partners; • If this is normal, then a smear will be performed yearly
• smoking. It doubles a woman’s risk. for up to 10 years (GP or gynaecologist).
• Liquid-based cytology (LBC) has reduced the number • If normal, then revert to 3-yearly smears.
of inadequate samples from over 9% to 2.9% in 2008.
Results are received faster and there is less pressure on Follow-Up After Hysterectomy
the workforce. The changeover to LBC was completed
in October 2008. • Routine smears for 10 years prior to hysterectomy and
negative for cervical intraepithelial neoplasia (CIN) at
hysterectomy, vault cytology not required.
Action According to the Smear Result • Less than 10 years routine smears prior to hysterectomy
An Inadequate Smear and negative for CIN at hysterectomy, single vault smear
• Smears cannot be interpreted (inadequate) if the specimen required, then no further follow-up required.
is obscured by inflammatory cells/blood, does not contain • CIN present at hysterectomy but fully excised, vault smears
the right type of cells, or is incorrectly labelled. at 6 and 18 months, then no further follow-up, if negative.
• Repeat an inadequate smear after between 6 weeks and • CIN present at hysterectomy with incomplete or uncertain
3 months. Repeating the smear within 6 weeks does not excision, follow-up as if cervix still in situ.
allow adequate tissue regrowth. Refer if there are two
further inadequate smears, repeated at 3-monthly intervals References
(preferably midcycle).
ALTS Group. (2003). A randomized trial on the management of
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14
Obstetric Problems
LINDSEY POPE
Prepregnancy Care Suspected Miscarriage After 14 Weeks of Pregnancy
Genetic Disorders Abdominal Pain
Diseases in High-Risk Ethnic Groups Infection or Contact With Infectious Disease
Sickle Cell and Thalassaemia Rubella Contact
Jewish Population: Tay-Sachs Varicella (Chickenpox)
Previous Obstetric History Cytomegalovirus
Investigations to Consider Undertaking Prior To Referral for Toxoplasmosis
Recurrent Miscarriage Listeria
Medical History Group B Streptococcal Infection (RCOG, 2017)
Hypertension (Discussion to Come) Hepatitis A
Diabetes (Discussion to Come) Hepatitis B
Epilepsy Herpes Simplex
Asthma Human Immunodeficiency Virus
Other Medication Parvovirus B19 Infection (Fifth Disease/Slapped Cheek
Psychiatric History Syndrome) (Morgan-Capner & Crowcroft, 2002)
Infection Zika Virus
Pruritis
Prenatal and Antenatal Advice
Excessive Weight Gain
Lifestyle
Fundal Height
Nutrition
Anaemia Hypertension
Vitamin D Pre-eclampsia
Folic Acid Long-Term Follow-Up (NICE, 2010)
Vitamin A Eclampsia
Proteinuria
Exercise
Bacteriuria
Sexual Intercourse in Pregnancy Glycosuria
Car Travel Anaemia
Travel Overseas Anaemia Developing in Pregnancy
Routine Antenatal Care Rhesus-Negative Women
Booking—Prior to 12 Weeks Abnormal Lie
Follow-Up Appointments High Head
At Each Appointment Check Premature Rupture of Membranes
Prenatal Diagnosis and Screening Postmaturity
Screening Tests Specific Medical Conditions and Pregnancy
Structural Abnormalities Asthma
Chromosomal Abnormalities Diabetes (Types 1 and 2)
Problems in Pregnancy Thyroid Disorders
Nausea and Vomiting Epilepsy
Inflammatory Bowel Disease (Fergusson, Mahsu-Dornan, &
Heartburn
Patterson, 2008)
Pelvic Pain Depression
Vaginal Bleeding and Miscarriage Drug Misuse
Ectopic Pregnancy
Suspected Miscarriage Up to 14 Weeks Pregnancy
209
Postnatal Care Breast Engorgement

Mother Mastitis
Baby Suppression of Lactation
At 6 Weeks Vaginal Bleeding After 24 Hours
Breastfeeding Fever
Advantages of Breastfeeding Depression
Problems With Breastfeeding Management of Postnatal Depression
Sore Nipples Exhaustion
Skin Infection Common Physical Problems
Blocked Duct Neonatal Jaundice
Prepregnancy Care is increasing, and studies suggest that clinicians

across specialties are poor at appreciating which condi-
• Whilst prepregnancy counselling offers an opportunity tions have identifiable genetic components (Harris
to reinforce good health habits and identify potential et al., 1999).
problems for pregnancy early, there is no evidence from • If there is any doubt, contact the nearest department of
randomized controlled trials that women who attend such clinical genetics for advice. Children or adults with a
visits have better health or pregnancy outcomes. In one major abnormality or disease, even if not at present thought
large cohort study, only a small proportion of women to be genetic in origin, should have blood stored at a
planning pregnancy followed recommendations for nutri- regional genetics laboratory for later analysis.
tion and lifestyle (Inskip et al., 2009). • Those at higher risk include the following:
• General practitioners (GPs) may have the opportunity to 1. Couples with a personal or family history of an abnor-
identify risk factors before conception. This can be done mality that is presumed to be genetic in origin. They
either at a specific consultation or when the opportunity should be referred for genetic counselling or have blood
arises (e.g., as part of contraceptive care) or when seeing taken by the GP on the advice of the geneticist. Impor-
patients with diabetes or hypertension. tant examples are:
• If a woman presents for a prepregnancy visit this can be • cystic fibrosis;
used as a time to encourage her to: • Huntington chorea;
• stop smoking; • Duchenne and other muscular dystrophies;
• take 400 µg of folic acid a day for the first 12 weeks • polycystic kidneys;
of pregnancy (explain that folic acid supplements have • intellectual disability, which may be due to fragile-X
been shown to reduce the risk of neural tube defects syndrome.
by 50%–70%) (Rush, 1994); 2. Couples belonging to a high-risk ethnic group (discus-
• avoid alcohol or at most take 2 units a week; sion to come).
• take a healthy diet; particularly avoiding soft cheeses 3. Older women. The risk of having a baby with Down
and liver. syndrome is 1 : 400 at age 35, rising to 1 : 100 at age
• At the same time the doctor can: 40, and 1 : 30 at age 45. Around the age of 37, the
• consider the risk of inherited disorders; risk of chromosomal abnormality is greater than the
• assess medical conditions (e.g., hypertension, diabetes, risk of miscarriage after amniocentesis. Recommend
epilepsy, hyperthyroidism); the leaflet “Screening for ., Edwards’ and Patau’s Syn-
• assess whether the woman has an eating disorder or dromes” from www.nhs.uk/.
is overweight; 4. Consanguineous couples. First-degree cousins have a
• check that the patient is immune to rubella and has slight but significant increase in congenital malforma-
an up-to-date cervical smear; tions. This is likely to be higher if there is a family
• warn the patient to avoid contact with chickenpox or history of congenital disease. Caucasian first-degree
exposed shingles. In the United States and Australia, cousins should be offered screening for cystic fibrosis
varicella vaccination is offered to non-immune women. even when there is no family history.
• Consider offering flu vaccination.
Diseases in High-Risk Ethnic Groups
Genetic Disorders Sickle Cell and Thalassaemia
• All couples should be referred for genetic counselling • Women from areas where malaria has been common are
if they request it or there are risk factors that need at increased risk of carrying the sickle cell or thalassaemia
further investigation. The list of identifiable conditions traits: Africa, the Caribbean, the Middle East, the Indian
CHAPTER 14 Obstetric Problems 211
subcontinent, South America, South and Southeast Asia, • Recurrent miscarriages. Refer to a gynaecologist for assess-
and the Mediterranean. ment all women who have had three consecutive miscar-
• In the United Kingdom, all pregnant women are offered riages. Reassure them, however, that they still have a 60%
screening for thalassaemia using the mean corpuscular chance of a normal pregnancy and that it is common for
haemoglobin (MCH) measurement as part of the routine no cause to be identified. Consider earlier referral if:
blood count. • the woman requests it;
• Women, or potential fathers, from an ethnic group at • there is a family history of miscarriage or of congenital
risk are also offered a test for sickle cell and related hae- disease;
moglobin variants. • there is a relevant medical problem; or
• At a prenatal consultation, a couple may choose to • there is an urgency to achieve a live birth, as in the
undergo screening because of the ethnic group to which older woman.
they belong or because of a positive family history. When
assessing ethnic origin try to go back at least two genera- Investigations to Consider Undertaking Prior To
tions. Discuss the appropriate test with the laboratory Referral for Recurrent Miscarriage
and send the completed Family Origin Questionnaire • Pelvic ultrasound
with the blood samples. This is available from the sickle • Luteinizing hormone (LH) on day 7
cell and thalassaemia screening programme (www.gov.uk, • Blood group and antibodies
then search for “Family origin questionnaire”). • Anticardiolipin antibodies (IgM and IgG)
• Refer to the obstetrician all couples who are both het- • Lupus anticoagulant
erozygous for sickle cell or thalassaemia, or when the • Protein C, protein S, factor V Leiden
mother is and the father is unknown. • Karyotyping of both partners (and of foetal products, if
available)
• Family history or previous infant with neural tube defects:
PATIENT INFORMATION • All women in this category should receive folic acid
prior to conception.
Sickle Cell Society, 54 Station Road, London NW10 4UA, tel.
020 8961 7795, helpline 0800 001 5660, www. • Give 5 mg daily, starting 1 month prior to stopping
sicklecellsociety.org. contraception, and continue throughout the first
UK Thalassaemia Society, 19 The Broadway, Southgate trimester.
Circus, London N14 6PH, tel. 020 8882 0011, free adviceline • Women should also receive advice about antenatal
0800 731 109, www.ukts.org.
diagnosis (see upcoming discussion).
• Size, gestation, and maturity of previous infants:
• Previous baby less than 2500 g: Stress the importance
of not smoking and drinking alcohol and explain that
Jewish Population: Tay-Sachs she is at increased risk of intrauterine growth retarda-
• Tay-Sachs disease is an incurable neurodegenerative dis- tion. Careful symphysis–fundal measurements should
order that begins around 6 months of age. Babies born be performed at each visit, ideally by the same expe-
with it live for only a few years. People of Ashkenazi rienced practitioner. The woman should be referred
(Eastern European) Jewish descent are most at risk. early in pregnancy for assessment by a specialist
• Consider referral of both prospective parents to the regional obstetrician.
genetics centre for testing for Tay-Sachs trait. One in 25 • Large previous baby: Check that a modified glucose
of Jewish descent is positive. tolerance test has been performed.
Previous Obstetric History Medical History

• Down syndrome. Make certain that all women who have Hypertension (Discussion to Come)
had a previous pregnancy affected by Down syndrome • This should be assessed before a woman gets pregnant,
have received genetic counselling, regardless of the age at as it may be masked by the fall in blood pressure (BP)
which they conceived. in the first half of pregnancy.
• Previous miscarriages (see upcoming discussion). Inform • Discuss the most appropriate drug with the relevant spe-
all patients, if asked, that the overall incidence of mis- cialist before conception.
carriage is around 15%. However, women whose last
pregnancy was normal have a risk of miscarriage of only Diabetes (Discussion to Come)
5%, whereas women whose last pregnancy ended in miscar- • Explain that diabetes is associated with an increase in
riage have a 20% to 25% chance of a miscarriage (Regan congenital abnormalities (about double), but that this
et al., 1989). Women with three consecutive miscarriages can be decreased significantly by good control of the
have a 40% chance of the next pregnancy ending in blood sugar prior to and during pregnancy (Nachum
miscarriage. et al., 1999; Steel et al., 1990).
• The National Institute for Heath and Care Excellence

Epilepsy (NICE) guideline “Antenatal and Postnatal Mental Health:
• Women with epilepsy are more likely to have unplanned Clinical Management and Service Guidance” (available
pregnancies due to contraceptive failure and twice as likely at www.nice.org.uk) discusses the risks associated with
to have a foetal malformation. psychotropic medication.
• Not all malformations may be attributable to antiepileptic
drugs (Fairgrieve et al., 2000). Psychiatric History
• Sodium valproate increases the risk of neural tube Mental illness is a common indirect cause of maternal
defects 50 times, to about 1.5%. mortality. Seeking a history early in pregnancy and ensur-
• With carbamazepine, the rate is 1%. ing effective follow-up in late pregnancy and the early
• There are few data on the safety of newer drugs. postnatal period is essential. See Chapter 18, Psychiatric
• The evidence of safety of one drug over another is Problems, for management of mental health problems
limited (RCOG, 2016). during pregnancy.
• Discuss with the neurologist:
• whether to stop anticonvulsants in any woman who Infection
has been free of seizures for 2 years; but counsel the
patient about the risks of untreated epilepsy in preg- • Rubella. All women should have rubella antibodies tested
nancy. Seizures may lead to stillbirth; or before each pregnancy; immunity can be lost between
• whether to change anticonvulsants or reduce their dose one pregnancy and the next.
in those in whom they cannot be stopped. • Hepatitis B. All women should be offered screening for
• Make it clear that even on anticonvulsants there is a 90% hepatitis B early in pregnancy as selective screening has
chance of a normal outcome that prenatal diagnosis is been shown to miss cases of hepatitis B virus (HBV) due
effective in picking up neural tube defects (see upcoming to the time pressures of routine clinical practice.
discussion), and that many defects can be corrected after • Listeria. Advise women to avoid soft cheeses, chilled ready-
birth. to-eat foods (unless thoroughly reheated), and patés prior
• Ensure that all women with epilepsy are offered: to and during pregnancy. They should avoid contact with
• folic acid. Those continuing antiepileptic medication sheep at lambing time, and with silage. Advise women
should take folic acid 5 mg daily from 3 months before to reheat food to steaming hot and thaw frozen foods in
conception until the end of week 12. Those stopping a microwave or refrigerator. Listeriosis is rare but can
their drugs need only take 400 µg daily from 1 month occur in outbreaks.
before conception; • Toxoplasmosis. Women contemplating pregnancy should
• an anomaly ultrasound examination at 18/19 weeks; be warned about the risks of handling soil, cat faeces, or
and raw meat and of eating undercooked meat and unwashed
• There is insufficient evidence to recommend the mater- vegetables and fruit; 80% of British women are not
nal use of oral vitamin K to prevent haemorrhagic immune at the time of pregnancy. Routine screening is
disease of the newborn, but all babies born to mothers not recommended.
using anti-epileptic medication should be offered 1 mg • Human immunodeficiency virus (HIV). All women should
vitamin K IM (RCOG, 2016). be offered screening for HIV early in pregnancy by mid-
• Educate a woman’s partner about what to do should she wives or doctors trained in pretest and post test counselling
have a seizure, as epilepsy may be less predictable. (Brocklehurst, 2000).
• Genital herpes and warts. Management is only an issue in
Asthma the last weeks of pregnancy.
• Asthma is one of the most common chronic medical
conditions and around 5% of pregnant women will require
asthma medication during pregnancy (Olesen et al., 1999). Prenatal and Antenatal Advice
• Asthma increases the risk of intrauterine growth restric- Lifestyle
tion (IUGR) by 24% overall, and by 47% in those with
severe asthma (Bracken et al., 2003). • Alcohol. Counsel the woman to reduce her alcohol con-
• Women with asthma should continue on their asthma sumption to a minimum (at the most 1 or 2 units once
medication, including preventers, as the risk of uncon- or twice a week). Complete abstinence is now recom-
trolled asthma poses a greater risk to the fetus than con- mended by the Department of Health, the British Medical
tinuing preventive medication (Martel et al., 2005). Association (BMA), the Royal College of Obstetricians
and Gynaecologists (RCOG), and the World Health
Other Medication Organisation (WHO). However, the evidence relating
• A decision should be taken about the need to stop other to the possibility of foetal damage from very low levels
drugs that are associated with foetal abnormalities (e.g., of alcohol intake does not provide a clear answer about
warfarin and lithium). the risk. A more powerful argument in favour of total
abstinence is that women who drink a little in preg-

nancy may easily overstep the limits without realizing it Folic Acid
(O’Brien, 2007). • Recommend that all women take 400 µg of folic acid a
• Smoking. Rather than asking women, “Do you smoke?” day from before conception until the end of week 12. This
a better question is, “What best describes your smoking? will prevent 95% of neural tube defects (DTB, 1994b).
Daily, Every once in a while, or I don’t currently smoke?” • Higher dose folic acid supplementation (5 mg daily) should
All women who smoke should be offered a smoking ces- be offered to women who have one of the following:
sation intervention. Advise about the risks. Provide infor- • The woman or her partner is affected by spina bifida.
mation. Assess willingness to quit. Assist to quit using a • The woman has had a previous pregnancy affected by
cognitive behavioural intervention. Arrange for additional neural tube defects.
support if unsuccessful. • Women taking epilepsy medication (see earlier).
• Coffee. Heavy coffee drinking (8 or more cups a day) is • The woman suffers with diabetes or coeliac disease.
associated with a tripling of the risk of stillbirth (OR 3.0 • BMI above 30.
[95% CI 1.5–5.9]) (Wisborg et al., 2003). One study • Women who suffer from sickle cell disease or
found that caffeine intake as low as 200 mg (2 cups of thalassaemia.
coffee or 5 cups of tea or 5 cans of caffeinated fizzy drinks)
was associated with a doubling of the rate of miscarriage Vitamin A
and that lower intakes were not without risk (Weng, • Advise all women to limit intake of vitamin A to 700 µg/
Odouli, & Li, 2008). However, a Danish randomized day and not to eat liver or liver products (e.g., sausage
trial failed to show any effect on birth weight or length or paté) because of their high vitamin A content. High
of gestation from reducing caffeine intake, suggesting that levels of vitamin A may be teratogenic.
the association of caffeine with poor outcomes may be due
to one or more confounding factors (Bech et al., 2007). Exercise
• Other drugs. All women should be asked about their use
of prescription and non-prescription drugs. Women using • Advise women that beginning or continuing a moderate
non-prescription drugs should be referred to the appro- course of exercise during pregnancy is not associated with
priate drug service for assistance in quitting. adverse outcomes.
• Inform women about sports that have a danger: contact
Nutrition sports, high-impact sports, and vigorous racquet sports
that may involve the risk of abdominal trauma, falls, or
• Recommend a balanced diet low in fat with adequate excessive joint stress; and scuba diving, which may
iron, fresh fruits, and vegetables. Avoid soft cheeses (see result in foetal birth defects and foetal decompression
Listeria earlier). disease.
Anaemia
Sexual Intercourse in Pregnancy
• Check haemoglobin (Hb) in all women with a previous
history or at high risk of anaemia (e.g., vegans, multiple • Advise women that sexual intercourse in pregnancy is
pregnancies, and those with a short interval between not known to be associated with any adverse outcomes.
pregnancies).
• Check the serum ferritin in those women who have a Car Travel
history of iron deficiency anaemia, even if the Hb is normal.
• Advise about the correct use of seatbelts in pregnancy:
Vitamin D • Above and below the bump, not over it.
• Evidence for the importance of vitamin D in pregnancy • Use three-point seatbelts with the lap strap placed as
is conflicting but low levels may be implicated in pre- low as possible beneath the bump, lying across the
eclampsia, low birth weight, and impaired glucose toler- thighs with the diagonal shoulder strap above the bump
ance (RCOG, 2014). lying between the breasts.
• All women should be counselled about the importance • Adjust the fit to be as snug as comfortably possible.
of adequate vitamin D intake during pregnancy and whilst
breastfeeding. Travel Overseas
• It is recommended that all pregnant women take 400
units of vitamin D daily and high risk women (such as • See Chapter 17 for information about overseas travel.
those with dark skin, little sun exposure, or BMI >30) • For advice about immunization in pregnancy, see
should take at least 1000 units daily. Appendix 19.
• Deficiency should be treated over 4–6 weeks with either • Malaria. Pregnant women are advised to avoid travel to
cholecalciferol 20,000 international units (iu) per week malarious areas. However, advice is available for those
or ergocalciferol 10,000 iu twice weekly (RCOG, 2014). whose travel is essential: See the Health Protection Agency
document “Malaria Prevention Guidelines for Travellers • Risk factors for postnatal depression should be noted at
from the UK” available at www.gov.uk. this stage, to facilitate earlier detection should it occur.
There is little evidence that it can be prevented by inter-
Routine Antenatal Care vention in the antenatal period (NICE, 2014).
• Care providers should:
GUIDELINE • ensure that pregnancy care is woman-centred and that
they use effective communication skills to ensure that
National Institute for Health and Care Excellence. (2008). women make informed choices about their care;
Antenatal care for uncomplicated pregnancies. NICE clinical
guideline 62. Updated 2017. Retrieved from www.nice.org.uk.
• be aware that there is a huge amount of information
presented to women during pregnancy and they should
provide written information and resources in plain
language;
• ensure women are given an opportunity to discuss
• The woman should be the focus of maternity care. information at antenatal visits.
• Midwifery and GP-led models of maternity care are safe • Because of the increase in information to be processed
for low-risk women. by pregnant women, early antenatal appointments should
• Women with the following conditions may require spe- be scheduled to allow discussion time.
cialist care:
• Current medical problem (e.g., diabetes, epilepsy, Booking—Prior to 12 Weeks
hypertension)
• Psychiatric disorder (on medication) • Take a full medical and obstetric history if the patient is
• Drug use such as heroin, cocaine (including crack new to the practice.
cocaine), and ecstasy • Recommend folic acid 400 µg daily until week 12 if the
• HIV or HBV infected patient is not already taking it.
• Autoimmune disorder • Assess risk and discuss the model of maternity care appro-
• Obesity (BMI ≥35 at first contact) or underweight priate for the woman.
(BMI <18 at first contact) • Estimate end due date (EDD). Offer early ultrasound
• Women who are particularly vulnerable (e.g., teenag- (10–13 weeks) to determine gestation. Offer 18- to
ers) or who lack social support 20-week ultrasound for structural anomalies.
• Multiple pregnancies • Provide information on diet and lifestyle. Routine iron
• Women who have experienced any of the following in supplementation is not recommended. Advise about reduc-
previous pregnancies also need specialist care: ing the risk of listeriosis, toxoplasmosis, and salmonellosis.
• Recurrent miscarriage (three or more consecutive preg- • Offer a smoking cessation programme, if a smoker.
nancy losses) or a midtrimester loss • Check blood group and rhesus D (RhD) status.
• Severe preeclampsia, HELLP (hemolysis [H], elevated • Offer screening for anaemia, red-cell autoantibodies, HBV,
liver enzymes [EL], and low platelet count [LP]) syn- HIV, rubella, asymptomatic bacteriuria, and syphilis. Tests
drome, or eclampsia for sickle cell trait and thalassaemia, if at risk.
• Rhesus isoimmunisation or other significant blood • Offer screening for Down syndrome.
group antibodies • Measure BMI and BP and test urine for proteinuria.
• Uterine surgery including caesarean section, myomec- • Assess psychosocial well-being. Ask about feelings and
tomy, or cone biopsy fears. Ask about relationships and supports. Consider
• Antenatal or postpartum haemorrhage on two asking about intimate partner violence. Ask the two screen-
occasions ing questions for depression (see upcoming discussion)
• Retained placenta on two occasions and, if the answers are positive, ask if this is something
• Puerperal psychosis the patient would like help with (NICE, 2014).
• Grand multiparity (more than six pregnancies) • Offer Pertussis vaccination to protect the newborn prior
• Stillbirth or neonatal death to routine immunisation at 8 weeks.
• Small-for-gestational-age infant (<5th centile) • Offer influenza vaccination if the woman has not been
• Large-for-gestational-age infant (>95th centile) vaccinated in the current influenza season.
• Baby weighing less than 2500 g or more than 4500 g • Advise women about the Healthy Start Scheme if they
• Baby with a congenital anomaly (structural or chro are under 18 years old or over 18 and in receipt of certain
mosomal) benefits.
• Women at risk of gestational diabetes should be offered
an oral glucose tolerance test (OGTT) at 16 to 18 weeks Follow-Up Appointments
and at 28 weeks. Those at high risk are women with a
high BMI, previous gestational diabetes, a previous mac- • Reducing the number of visits is not associated with
rosomic baby, a family history of diabetes, and an ethnic worse outcomes, except that women may be less satisfied
origin with a high prevalence of diabetes. (Sikorski et al., 1996; Villar et al., 2001). Ten visits can
provide essential care (booking, and weeks 16, 18–20, • to be informed that diagnostic tests are invasive and
25, 28, 31, 34, 36, 38, 40). Visits should be long enough have a risk of miscarriage;
to allow women to have concerns voiced and addressed. • information about the performance of a screening test
Some women will require more visits. Multiparous women based on their age, as maternal age is a key component
may need as few as six visits (booking and 28, 34, 36, 38, of all screening tests: younger women (<25) having
41 weeks). screening tests will have a low screen positive rate (1%)
• Current evidence does NOT support the following as and a 30% detection rate whilst older women (>40)
routine: will have a high screen positive rate (50%) and a 90%
• Repeated maternal weighing detection rate (Three Centres Consensus Guidelines
• Breast examination on Antenatal Care Project, 2001);
• Pelvic examination • to be informed that most women having a screen posi-
• Antenatal screening to predict postnatal depression tive result will not have a pregnancy affected by Down
using Edinburgh Postnatal Depression Scale (EPDS) syndrome.
• Iron supplementation
• Screening for cytomegalovirus (CMV), hepatitis C Types of Screening Tests for Chromosomal
virus, group B streptococcus, toxoplasmosis, bacterial Abnormalities
vaginosis, gestational diabetes mellitus • A number of screening tests exist, yet availability varies
• Formal foetal movement counting between countries and centres. Check with your local
• Antenatal electronic cardiotocography maternity unit to confirm availability.
• Ultrasound scanning after 24 weeks • Tests with detection rates greater than 75% and false
• Umbilical artery Doppler ultrasound scan (USS) positive rates less than 3% are:
• Uterine artery Doppler USS to predict preeclampsia • combined first trimester screening (nuchal translucency
at 11.5–14 weeks, human chorionic gonadotropin
At Each Appointment Check [hCG], and pregnancy-associated plasma protein A
• Patient’s general health and psychosocial well-being [PAPP-A] at 8–12 weeks);
• BP • second trimester maternal serum screening—the quadru-
• Symphysis–fundal height, plotted in centimeters on a chart ple test (hCG, alpha-fetoprotein [AFP], unconjugated
• Foetal movements or foetal heart sounds estriol [uE3], inhibin A) performed between 14 and
• Presentation (after 34 weeks) 20 weeks;
• Check urine for proteinuria or signs of infection. • the integrated test (nuchal translucency, PAPP-A + hCG,
AFP, uE3, inhibin A);
• the serum integrated test (PAPP-A + hCG, AFP,
Prenatal Diagnosis and Screening uE3, inhibin A) can be performed between 11 and
Screening Tests 20 weeks.
• Maternal blood cell-free DNA testing at 10 weeks is
Pregnant women should be offered screening for structural currently not offered on the NHS in the UK. It has
anomalies and chromosomal abnormalities by appropriately a detection rate of >99% for trisomy 21 and one study
trained staff. If there is a family history of another genetic has shown it would be feasible for use as a screening
disorder, the woman should be referred to a specialist service tool (Gil et al., 2013).
for genetic counselling. • The first, third, and fourth methods involve tests at dif-
ferent stages of pregnancy. A result is only reported when
Structural Abnormalities all tests have been performed.
• Ultrasonography should be offered between 18 and 20
weeks. Routine ultrasonography can detect the more Diagnostic Tests for Chromosomal Abnormalities
obvious forms of congenital abnormality (e.g., cranial • Chorionic villus biopsy (CVB). This is usually performed
and neural tube defects, severe skeletal dysplasia, and after 11 weeks and allows women to consider termination
abnormalities of the heart, chest, and abdominal organs). of pregnancy in the first trimester, but has a miscarriage
Hydrocephalus may be detected later. As technology and rate of 1.2%. There is also a further 1% to 2% chance
training improve, the range of anomalies detectable before of needing an amniocentesis to establish the diagnosis.
birth continues to increase. CVB performed before 10 weeks may rarely cause limb
• Fetoscopy. Visualization of the foetus is necessary for the abnormalities (Lilford, 1991).
assessment of some external malformations. It is also used • Amniocentesis. This is usually performed at 15 to 16 weeks,
for laser treatment of twin-to-twin transfusion syndrome. which will allow women to consider termination by 20
weeks. It is associated with a miscarriage rate of 1%.
Chromosomal Abnormalities Polymerase chain reaction technology allows results for
• Women need: Down and Edward syndromes in 48 hours, usually backed
• to understand that screening is a risk assessment and by culture results for other chromosomal anomalies avail-
not a diagnostic test; able 2 weeks later.
Problems in Pregnancy Vaginal Bleeding and Miscarriage

GUIDELINE
Nausea and Vomiting
Stillbirth and Neonatal Death Society. (2016). Pregnancy loss
• Around 80% of pregnant women experience nausea during and the death of a baby: Guidelines for professionals (4th
the first trimester and around 50% experience vomiting. ed.). SANDS. Retrieved from www.uk-sands.org.
These problems can vary from being minor to severe and
disabling.
• Reassure the woman that most cases of nausea and vomit- • Rhesus status. If a rhesus-negative patient bleeds, anti-D
ing settle by 20 weeks and that it does not harm the immunization is recommended. The dose depends upon
foetus. the preparation used. Give it within 72 hours of the start
• Consider over-the-counter or prescribed treatment if the of bleeding, although if a longer period has elapsed it
vomiting has severe consequences on the quality of life may still give some protection.
(on day-to-day activities, including interfering with house-
hold activities, restricting interaction with children, greater Ectopic Pregnancy
use of healthcare resources, and time lost off work). Ensure
that women remain adequately hydrated, avoid exhaus- • Ectopic pregnancy is usually associated with mild vaginal
tion, and consider the following (Jewell & Young, 2003): bleeding as well as pain. A negative pregnancy test (i.e.,
• Acupressure at P6 (seabands) and ginger may be of an hCG level <50 IU/L) virtually excludes it.
benefit. • Intrauterine pregnancies should be visible on transvaginal
• Antihistamines (such as promethazine or cylcizine) or ultrasound when serum hCG exceeds 1000 IU/L.
prochlorperazine reduce nausea and vomiting but can • Admit any patient in whom the possibility of an ectopic
cause drowsiness. There is no evidence that they are is anything more than remote. It is the most common
teratogenic. NICE CKS advise metoclopramide or cause of death in the first trimester.
ondansetron as second line options but advise that
their use should not be continued beyond 5 days. Suspected Miscarriage Up to 14 Weeks Pregnancy
• If associated with reflux, advise: GUIDANCE
• a biscuit before getting up;
• small frequent meals; Ectopic pregnancy and miscarriage: Diagnosis and initial
• a low-sodium, low-sugar antacid for heartburn; management. NICE clinical guideline 154. Retrieved from
• stop iron, if it is making symptoms worse. www.nice.org.uk.
• Prescribe an H2 receptor antagonist (e.g. ranitidine 150 mg
twice a day) or a proton pump inhibitor to patients with
reflux not controlled by the above measures (RCOG, 2016). • If provided with appropriate counselling, most women
• In late pregnancy exclude urinary tract infection (UTI), who miscarry in the first trimester will choose expectant
preeclampsia, and surgical causes of vomiting. management. About 81% of these complete their miscar-
• Hyperemesis gravidarum. Admit if the weight loss is more riage without intervention (Luise et al., 2002).
than 5% with ketosis. Thiamine supplementation, either • Assess haemodynamic stability. Admit women who are
orally or intravenously should be given (RCOG, 2016). not haemodynamically stable.
• Perform a pregnancy test if pregnancy has not previously
Heartburn been confirmed.
• Perform an abdominal exam looking for tenderness; uni-
• Heartburn is common in pregnancy and becomes more lateral tenderness is suggestive of an ectopic pregnancy.
common as the pregnancy progresses. • Perform a pelvic exam for cervical motion tenderness or
• Suggest lifestyle modifications (see upcoming discussion). pelvic tenderness (suggestive of an ectopic pregnancy),
• Alginate antacids (e.g., Gaviscon). provided there is no abdominal pain or tenderness. Do
• H2 receptor antagonists or proton pump inhibitors can not palpate for adnexal masses as this may risk rupturing
be used as discussed above. an ectopic pregnancy.
• If they are over 6 weeks of gestation, arrange admission
Pelvic Pain to an early pregnancy assessment unit (EPAU); the urgency
of admission will depend on the clinical situation.
• Symphysis pubis dysfunction is a collection of signs and • If they are less than 6 weeks of gestation:
symptoms of discomfort and pain in the pelvic area, • Repeat a pregnancy test after 7 to 10 days to confirm
including pelvic pain radiating to the upper thighs and miscarriage if bleeding stops before 6 weeks of gestation.
perineum. It can be debilitating. • Arranged admission to the EPAU if bleeding continues
• There is no evidence that any treatment helps, but referral beyond 6 weeks of gestation or if the patient develops
to a physiotherapist and pelvic support may be of value. signs suggestive of an ectopic pregnancy.
• If miscarriage is confirmed but not completed, then PATIENT INFORMATION AND SUPPORT
management may be expectant, medical (with vaginal or
For parents whose baby is stillborn or dies soon after birth,
oral misoprostol), or surgical (by manual vacuum aspira-
the Stillbirth and Neonatal Death Society (SANDS), 28
tion under local anaesthetic, or surgically under general Portland Place, London W1B 1LY, helpline 020 7436 5881,
anaesthetic). administration 020 7436 7940, www.uk-sands.org.
• Women who have been managed medically should take
a pregnancy test 3 weeks later. If it is still positive, they
need to be reassessed for molar or ectopic pregnancy. Abdominal Pain
• Rhesus-negative women who have undergone surgical
management should be offered anti-D rhesus prophylaxis. • Abdominal pain may be due to a variety of causes, some
of which are serious and may present in an atypical way.
Follow-Up After Miscarriage Any cause that could occur outside pregnancy (e.g., renal
• Arrange to meet all patients who have miscarried about calculus) must be considered.
4 weeks afterwards. Be aware that the patient has suffered • In the first 20 weeks, consider:
a bereavement, and may need counselling; 50% are still • miscarriage;
likely to feel depressed (Friedman, 1989). • ectopic pregnancy;
• Attend to any physical issues and the possible need for • urinary infection;
contraception. The traditional advice to have two normal • appendicitis;
periods before trying to conceive again may be more • impaction of a retroverted uterus;
important to allow time for the grieving process than for • red degeneration of a fibroid (the maximum incidence
any physical reason. is 12–18 weeks, but it may occur at any time);
• Be aware that women are distressed by the fact that no • torsion of an ovary or tube;
reason for the miscarriage can usually be given. Many feel • haematoma of the round ligament;
unreasonable guilt and feel brushed off by being reassured • accident to an ovarian cyst.
that miscarriage is common (Wong et al., 2003). Explain • After 20 weeks, consider, in addition:
that early miscarriage is due either to an abnormality of • labour;
foetal development or to a failure of implantation. • abruptio placentae;
• Explain that one miscarriage is followed by a slight or • haematoma of the rectus abdominis;
even no increase in risk of subsequent miscarriages (Everett, • red degeneration of a fibroid;
1997). Investigations are unlikely to be fruitful unless • uterine rupture;
three or more pregnancies have miscarried. Having three • dehiscence of the pubic symphysis.
consecutive miscarriages gives a patient a subsequent risk
of 40%. Referral for investigation is then traditional, but
Infection or Contact With Infectious Disease
earlier referral may be justified as discussed earlier.
Rubella Contact
• If a pregnant woman is infected with rubella, the risk to
the foetus is greatest up to 11 weeks but 30% of foetuses
PATIENT INFORMATION AND SUPPORT
between 11 and 16 weeks are affected.
The Miscarriage Association, c/o Clayton Hospital, Northgate, • Women experiencing infection before 16 weeks should be
Wakefield, West Yorkshire, WF1 3JS, helpline 01924 200799, offered counselling regarding termination of pregnancy.
administration 01924 200795 for leaflets about all aspects of
miscarriage, ectopic pregnancy, and molar pregnancy; www. • If a pregnant woman is affected between 16 and 19 weeks,
miscarriageassociation.org.uk. the risk of foetal damage is less than 2%. Deafness is the
most likely problem.
• After 19 weeks, the risk appears very low (Jones, 1990).
• Accidental immunization in pregnancy has not been asso-
ciated with embryopathy.
Suspected Miscarriage After 14 Weeks of
Pregnancy Rubella Contact Before 16 Weeks
If the pregnancy is less than 16 weeks, regardless of whether
• The more advanced the pregnancy, the more advisable it the woman has had rubella or the vaccination or antibodies
is to admit the patient to hospital at the onset of bleed- were previously detected, do the following:
ing, regardless if pain is present. • Take blood for rubella antibodies:
• After 14 weeks, painless bleeding is due to placenta praevia • If IgG is present and the blood was taken within 12
until proved otherwise. days of contact, inform the woman that she is immune
• Bleeding with severe pain is likely to be due to abruptio and that there is little need to worry.
placentae. • If IgG is present but the blood was taken more than
• Do not do a vaginal examination in these patients. 12 days after contact, request IgM levels. If IgM shows
recent infection, discuss the risks of foetal abnormality • Ganciclovir is effective in the neonate but has not been
and the question of termination of pregnancy. proven to be of benefit, if given to the mother during
• If IgG is absent, repeat IgM 2 weeks later. If she has pregnancy.
seroconverted, discuss the question of termination of • Advice to a woman who seroconverts to CMV during
pregnancy as mentioned. The value of immunoglobulin pregnancy is difficult and such cases should be referred
in protecting the foetus is doubtful. for tertiary centre advice.
• Any IgG-negative woman should be immunized in the
puerperium. Toxoplasmosis
Note: If a woman contracts rubella and decides to continue • Toxoplasmosis causes fetal infection in about 15% of
the pregnancy, foetal blood sampling (from the umbilical cases in the first trimester, rising to 70% in the third.
vein) from 20 weeks onwards can indicate whether the baby The risk of serious fetal damage if the fetus is infected is,
has contracted it by assessing foetal IgM. however, greater in early pregnancy. Overall, up to 90% of
Note: IgM can persist for up to a year after rubella. Only infected babies escape long-term damage. Most maternal
act on an IgM result, if it is consistent with the clinical picture cases are asymptomatic. The incidence of children born
or with a reliable history of exposure (Best et al., 2002). with definite or probable toxoplasmosis is very low.
• Where there is concern, check serology as soon as possible
Varicella (Chickenpox) after conception and monthly thereafter. IgM may stay
GUIDELINE positive for months; timing infection in relation to preg-
nancy stage requires IgG avidity testing at the National
Royal College of Obstetricians and Gynaecologists. (2015).
Chickenpox in pregnancy. RCOG “Green Top” clinical
Reference Laboratory.
guideline 13. Retrieved from www.rcog.org.uk. • In the case of infection, the woman may be offered USS
and amniocentesis to detect foetal abnormality, and foetal
blood sampling between 20 and 24 weeks. Even if FBS
demonstrates foetal infection, it cannot indicate whether the
• A congenital varicella syndrome, including limb deformities foetus has been damaged (Robinson, 1991). Treatment with
and scarring, may occur in 1–2% of pregnancies where spiramycin in pregnancy is safe but of unproven benefit.
the mother contracts chickenpox (though not shingles)
up to, but not after, 20 weeks of gestation. Listeria
• Exposure to chickenpox after 20 weeks and before 36 • Maternal infection may be asymptomatic or associated
weeks does not appear to be associated with foetal with fever. The infection carries a risk of miscarriage,
abnormalities. stillbirth, or the birth of an infected baby.
• A woman who is not known to be immune and who is in • Symptoms in the mother are so nonspecific that the diag-
contact with chickenpox in the first trimester: Check her nosis is rarely made during the acute illness, especially
varicella antibodies urgently and give varicella zoster since the only useful diagnostic test is blood culture.
immune globulin (VZIG), if not immune. • Women with fever for 48 hours who are pregnant and
• Give acyclovir to a pregnant woman who develops chick- who have no obvious other source of infection should
enpox at a gestation of over 20 weeks, starting within 24 have a blood culture specifying listeria. Treatment is with
hours of the appearance of the rash. Monitor any pregnant intravenous ampicillin.
woman with chickenpox carefully, as 10% develop
pneumonia. Group B Streptococcal Infection (RCOG, 2017)
• Offer an ultrasound anomaly scan to women contracting • Group B streptococcal (GBS) is the most common severe
chickenpox before 20 weeks of gestation. infection in infants in the first 7 days of life.
• If a mother contracts chickenpox in the 5 days prior to • Routine screening for GBS is not recommended.
giving birth or within 2 days of giving birth, the baby • If performed (e.g., in women considered at high risk),
should be given VZIG. screening should be performed 3 to 5 weeks prior to the
expected delivery.
Cytomegalovirus • Situations in which neonatal GBS infection is more
• CMV infection is now the most common congenital likely are:
infection worldwide. The birth incidence is between • neonatal GBS infection in a previous baby;
0.2–2.5%, and 10% of these infections will produce an • vaginal, urinary, or intestinal GBS present in the woman
affected neonate. in this pregnancy;
• Consequences of infection can include deafness and devel- • preterm (<37 weeks) labour;
opmental disability; this is more common with primary • prolonged (>18 hours) rupture of membranes;
infection and occurs in up to 20% of cases. • fever during labour (>38°C).
• Two-thirds of congenital infections are thought to result • Consider women at risk, as above, for intravenous antibi-
from primary maternal infection and one-third from otics during labour, or the newborn baby for antibiotics
recurrent infection in the mother. until blood cultures show that there is no BGS present.
• Recommend to the woman the excellent leaflet available be greatest with the bowel inflammation secondary to
from Group B Strep Support on www.gbss.org.uk. artificial feeds.
• Pregnancy does not appear to hasten the onset of acquired
Hepatitis A immunodeficiency syndrome (AIDS) in women who are
Reassure the mother that the infant will not be harmed HIV positive.
although it may be infected if occurring shortly before term.
Parvovirus B19 Infection (Fifth Disease/
Hepatitis B Slapped Cheek Syndrome)
• Hepatitis B is not influenced by pregnancy, but if the (Morgan-Capner & Crowcroft, 2002)
mother is infected there is a risk of acute infection of the • This is common in schools and nurseries, especially in
foetus or neonate. April and May. If a woman is exposed in the first 20
weeks of pregnancy, there is an increased risk of intra-
Herpes Simplex uterine death (excess risk 9%) and if infection occurs
Genital Herpes (BASSH & RCOG, 2014) between 9 and 20 weeks, foetal anaemia leads to hydrops
• Primary infection at delivery gives a 20% to 50% risk of foetalis (3% of whom die [included in the excess 9%]).
neonatal herpes, which is frequently fatal. Recurrent herpes The consequences usually occur within 3 to 5 weeks of
at delivery gives a risk of 0% to 3%. Lower segment maternal infection.
caesarean section (LSCS) is therefore only recommended • Maternal asymptomatic infection is as likely to damage
if the patient is suffering her first attack during labour. the foetus as symptomatic infection.
• Viral shedding is less in subsequent attacks and maternal • Check antibodies in all women exposed as soon as pos-
IgG crosses the placenta to provide some foetal protec- sible, informing the lab of the clinical details.
tion. There is little value in taking viral swabs in the last • If specific IgG is detected and specific IgM not detected,
month of pregnancy in those with a history of infection the woman can be reassured that she has had past
but without active lesions. infection.
• Aciclovir (400 mg three times daily) in the last month • If specific IgM is detected but specific IgG is not, a
of pregnancy reduces viral shedding and vertical transmis- further sample should be tested immediately. Refer all
sion (Braig et al., 2001). women who are positive to an obstetrician.
• If specific IgG and IgM are not detected, a further
Labial Herpes sample should be tested after 1 month.
The mother, and indeed any family or friends with labial
herpes, should refrain from direct contact with the baby Zika Virus
(i.e., kissing) once born.
• Mosquito borne illness, linked to microcephaly, especially
Genital Warts in the first trimester.
Respiratory papillomatosis in the child is strongly related to • Advise women to avoid traveling to Zika areas until after
maternal genital warts during pregnancy. However, the risk pregnancy.
is small (6.9 cases per 1000 live births, against a risk of 0.03 • If travel is unavoidable take strict precautions to avoid
per 1000 live births with no such maternal history) and is mosquito bites.
not reduced by caesarean section (Silverberg et al., 2003). • Get specialist advice if potential infection.
• Zika is transmitted in semen. Use condoms for 28 days
Human Immunodeficiency Virus after return from Zika area if no symptoms and 6 months
• A mother who is HIV positive has a risk of foetal infec- if symptomatic or infection confirmed.
tion of 15% to 25%, but this can be substantially reduced
by caesarean section delivery and/or antiretroviral therapy. Pruritis
Vaginal delivery is recommended for those with a low
viral load. • Pruritis, without jaundice, is the most common manifes-
• All HIV positive women should be on anti-retroviral treat- tation of obstetric cholestasis, which in turn is associated
ment during pregnancy, either continuing their current with premature birth in 60%, foetal distress in up to 33%,
regime or starting it once they fall pregnant (BHIVA, 2018). and intrauterine death in up to 2% (Milkiewicz et al.,
• Screening should be offered to all women. Some babies 2002). Although most women with pruritis in pregnancy
will be HIV antibody positive at birth due to maternal do not have obstetric cholestasis, it should be considered
antibody, but will become negative as maternal antibody in every case because of its serious implications.
is cleared. • Check liver function tests (LFTs). Transaminases are raised
• Breastfeeding doubles the chance of vertical transmission in 60% of cases of obstetric cholestasis, though only 25%
and is not recommended. If a woman must breastfeed, have a raised bilirubin.
then exclusive breastfeeding rather than mixed feeding • Refer all affected patients. Most obstetricians will deliver
should be recommended as neonatal seroconversion may the baby at 37 weeks.
Excessive Weight Gain its nadir by 16 weeks, plateaus until 22 weeks, and then
increases toward term.
• A woman’s BMI should be calculated at the first booking • Pregnancy-induced hypertension (PIH): BP which rises to
appointment. exceed 90 mm Hg diastolic on more than one occasion,
• The average weight gain in pregnancy is 10 to 12.5 kg in the second half of pregnancy, resolves after delivery
(0.65 kg first quarter, 4 kg by midpregnancy, 8.5 kg by and is not complicated by proteinuria. The International
the third quarter, 12.5 kg by term). Over a quarter of Society for the Study of Hypertension in Pregnancy has
the total gain is due to fat deposition in the middle two chosen this definition, rather than one based on a relative
quarters of pregnancy. rise in diastolic pressure, because any rise in pressure varies
• The ideal weight gain depends on the pre-pregnancy BMI. according to when the baseline was taken and because a
A woman with a BMI that is low or normal has fewest relative rise seems to correlate less well with outcomes than
obstetric and neonatal adverse outcomes if her weight the use of an absolute cut off point.
gain is less than 10 kg. A woman with a BMI 25 to 30 • Pre-eclampsia: This is defined in the same way as above,
does best if her weight gain is less than 9 kg and an obese but is associated with proteinuria of more than 300 mg/24 h.
woman does best with a weight gain less than 6 kg It may be further complicated by the HELLP syndrome
(Cedergren, 2007). Excessive weight gain may be associ- in which there is haemolysis, elevated liver enzymes and
ated with an increased incidence of pre-eclampsia. Check low platelets. The terms PIH and pre-eclampsia should not
BP, urine, and the presence of oedema. be used interchangeably as the former is not associated
• Despite the above, there is no evidence that the regular with poor maternal or foetal outcome, whilst pre-eclampsia
weighing of patients is of benefit and it is not recommended. is a leading cause of maternal and foetal morbidity.
• Obesity. Obesity is associated with an increase in congenital • Antihypertensives do not lessen the risk of developing
malformations and first trimester abortions, gestational pregnancy-induced hypertension or alter its progression,
diabetes, hypertension, macrosomia, stillbirth, prolonged but protect against stroke and possibly placental abruption.
labour, and caesarean birth. All women with obesity should • Aspirin prophylaxis (75 mg daily) may prevent the devel-
be seen by an obstetrician and monitored closely for weight opment of pre-eclampsia and moderate the condition
gain, BP, and diabetes (Stotland, 2009). once it has started (Askie et al., 2007).
• Eating disorders are common in pregnancy; the disorder • Measuring the BP. Use phase 4 diastolic pressure and not
may worsen or improve as the pregnancy progresses (Ward, phase 5, which may be misleadingly low in pregnancy
2008). Women with a recognized eating disorder should (MacGillivray & Thomas, 1991).
be referred to an obstetrician and the eating disorder • Chronic hypertension predates pregnancy or appears prior
service, early in pregnancy. Suggested screening questions to 20 weeks of gestation. A BP of 140/90 and above
at each antenatal visit: before 20 weeks suggests pre-existing hypertension and
• What is your current eating pattern? Are you restrict- needs specialist assessment.
ing your dietary intake? Do you binge? Do you vomit
or take laxatives after eating? Pre-eclampsia
• How do you feel about your shape and weight? GUIDELINE
• What is your weight? Are you gaining weight appropriately?
National Institute of Health and Care Excellence. (2010).
• What is your mood like? Do you feel low or anxious?
Hypertension in Pregnancy: the management of hypertensive
• What exercise are you taking? Are you exercising disorders during pregnancy. NICE clinical guideline 107.
too much?
Fundal Height
• Women with any of the following should be referred for
• At 22 weeks, the fundus should have reached the umbi- specialist assessment before 20 weeks of gestation:
licus, and at 32 weeks the lower rib border. • Previous pre-eclampsia
• The symphysis-to-fundus height in centimetres should be • Multiple pregnancy
within two of the number of weeks gestation between 20 • Underlying medical conditions:
and 36 weeks; after 36 weeks it should be within ±3 cm. 1. Pre-existing hypertension or booking diastolic BP
• If the fundus is over 3 cm less than dates, refer for exclu- 90 mm Hg or above
sion of foetal IUGR, or oligohydramnios. 2. Pre-existing renal disease or booking proteinuria
• If the fundus is 3 cm more than dates, refer for assessment (increased on more than one occasion or
for possible multiple pregnancy or hydramnios. ≥300 mg/24 h)
3. Pre-existing diabetes
Hypertension 4. Presence of antiphospholipid antibodies
• Women with any two or more of the following should be
• BP falls early in the first trimester as the fall in peripheral referred for specialist assessment before 20 weeks of gestation
resistance exceeds the rise in cardiac output. BP reaches and offered aspirin 75 mg from 12 weeks:
• First pregnancy Proteinuria in the Absence of Raised Blood Pressure

• More than 10 years since last baby (NICE, 2010)
• Age more than 40 years • One plus (+). Arrange for a midstream urine for culture
• BMI 35 and above and microscopy and check the urinary spot protein to
• Family history of pre-eclampsia (mother or sister) creatinine ratio. Review in 1 week. If culture and
• Multiple pregnancy microscopy are negative but the protein to creatinine
• Any woman with suspected pre-eclampsia should be ratio is positive, check the serum creatinine, the 24-hour
managed in conjunction with specialist advice. This will urinary protein or spot protein : creatinine ratio, and refer.
usually be undertaken in a special day assessment centre. Continue to look for preeclampsia; 10% of patients who
• Action to be taken if new hypertension is discovered after develop eclampsia have proteinuria without a raised BP
20 weeks: (Douglas & Redman, 1994).
• BP 140/90 - 149/99: monitor blood pressure weekly • More than +. Seek specialist advice with a view to obstetric
and test for proteinuria at each visit. assessment.
• BP 140/90 - 149/99 plus a symptom associated with • At least + with a symptom associated with preeclampsia.
preeclampsia: refer for same-day assessment Refer for same-day assessment.
• BP 140/90 - 149/99 plus proteinuria: refer for same-
day assessment Using the Different Tests for Proteinuria
• BP 150/100 - 159/109: measure blood pressure at (Chappell & Shennan, 2008)
least twice weekly, check for proteinuria at each visit • The protein dipstick is prone to both false negatives and
and check renal function, liver function tests (includ- false positives. Although recommended by NICE for the
ing bilirubin) and full blood count. Treat using oral routine assessment of urinary protein in antenatal care,
labetolol as first line. it is the least useful of the available tests.
• BP 160/110 or higher: admit to hospital. • The urinary spot protein to creatinine ratio has few false
Note: Symptoms associated with preeclampsia include negatives and more false positives. It is therefore a good
headache, visual disturbance, nausea, vomiting, and epi- screening test (if it is negative, the patient almost certainly
gastric pain. does not have significant proteinuria) but a more accurate
Note: Management of hypertension in pregnancy as out- test is required to confirm a positive finding. A positive
lined above should be in conjunction with the obstetric team. result is considered to be at or above 30 mg/mmol.
• A 24 hour-urinary protein remains the most accurate test
Long-Term Follow-Up (NICE, 2010) available, with a cutoff of 300 mg/24 h. False negatives occur
• Women who have had pre-eclampsia are at higher long- when women forget to collect every sample; and different
term risk of cardiovascular disease (CVD) and also higher laboratories use different assays and so get different results.
risk that the disease may occur earlier.
• Inform them that their risk of CVD when young is still Bacteriuria
low but that they should concentrate on prevention. • Of pregnant women, 2% to 10% have asymptomatic
• Counsel all women who have had pre-eclampsia about a bacteriuria. If untreated, 30% will develop a urinary infec-
healthy lifestyle. tion. Antibiotics are very effective in preventing pyelo-
• Screen for the emergence of other risk factors for CVD nephritis (Smaill, 2001).
at an earlier stage than usual. • Treat with amoxicillin, cephalosporin, or nitrofurantoin
according to sensitivities.
Eclampsia • Repeat the MSU 2 weeks after treatment.
• This is a medical emergency with a high maternal mortal-
ity. The patient must be resuscitated and transferred to Glycosuria
hospital as fast as possible. The anticonvulsant of choice • This occurs in 70% of pregnant women.
to prevent further fits is magnesium sulphate. • If there is 2+ of glucose in urine or + on two occasions,
• Recurrence and follow-up: Many women will have posttrau- then arrange for a modified glucose tolerance test. If the
matic stress disorder when their normal physiological preg- sugar level is raised (fasting sugar ≥5.6 mmol/L or the
nancy has been taken from them. It is important to explain 2-hour sugar ≥7.8 mmol/L), refer the patient urgently.
what happened and the relatively low chance of recurrence
in future pregnancies (20%). Many units offer uterine artery Gestational Diabetes
Doppler as a screening test in subsequent pregnancies, with
the possibility that aspirin can reduce this rate further. GUIDELINE
Proteinuria Diabetes in pregnancy: Management from preconception to
A trace of protein is acceptable. It may be due to contamina- the postnatal period. NICE guideline 3. Retrieved from www.
nice.org.uk.
tion with vaginal secretions or to a delay since the urine was
passed.
• A patient should be managed intensively if her 2-hour • Offer routine anti-D immunization to all Rhesus-negative
sugar is over 7.6 mmol/L or her fasting glucose is over women at 28 and 34 weeks, if nonsensitized, except where
5.6 mmol/L. For most patients, however, diet will be the patient:
sufficient to control blood sugars. Active management • is certain she will not have another child after the
with diet, blood glucose monitoring, and, if necessary, present pregnancy; or
metformin or insulin has been shown to reduce the risk • is in a stable relationship with the father of the child
of serious perinatal complications (Crowther et al., 2005). and the father is known to be RhD negative (NICE,
Recheck fasting glucose 6 to 13 weeks postpartum to be 2002).
sure that it has returned to normal. • In addition to routine antenatal anti-D immunization,
• Warn the patient of her 20% to 30% chance of develop- offer it postnatally and if a sensitizing event occurs ante-
ing diabetes in the next 5 years. This risk can be decreased natally, namely:
by maintenance of a diabetic diet after pregnancy. • abdominal trauma including external cephalic version;
• chorionic villus biopsy and amniocentesis;
Anaemia • antepartum haemorrhage;
• ectopic pregnancy;
• As the plasma volume increases in pregnancy, the Hb • termination of pregnancy or endoscopic retrograde
falls. Only a Hb below 11 g/dL (WHO) in the first tri- cholangiopancreatography (ERCP);
mester, or below 10.5 g/dL at 28 weeks, is considered to • threatened or complete miscarriage after 12 weeks;
be anaemia. • intrauterine death.
• Routine iron supplementation is not recommended. • Use 250 IU up to 20 weeks of pregnancy. Give 500 IU
• A low serum ferritin is not a reliable guide to iron defi- thereafter, followed by a Kleihauer test for foetal Hb. If
ciency in pregnancy. It may reflect a shift of iron stores the foetomaternal haemorrhage exceeds 4 mL red cells,
to the increased red cell mass rather than a low total body a further dose will be needed.
iron. However, there is no better non-invasive test.
Anaemia Developing in Pregnancy Abnormal Lie
• Check full blood count, film, and serum ferritin. Check • Check the lie from 32 to 34 weeks onward. If a transverse
B12 and red cell folate if there is macrocytosis. lie is found at 32 weeks or later, then either:
• Start iron in treatment doses if the Hb is below 10 g/dL • refer; or
or mean corpuscular value (MCV) is below 82 fL (e.g., • arrange a scan and reassess at 36 weeks. Refer if still
give ferrous sulphate 200 mg three times a day and folic transverse.
acid 5 mg daily). The addition of folate can almost double Note: A breech presentation should be seen at the hospital
the rise in Hb regardless of the patient’s folate status by 37 to 38 weeks.
(Juarez-Vazquez, Bonizzoni, & Scotti, 2002).
• Repeat Hb in 2 weeks. It should rise by about 0.8 g/dL High Head
per week.
• If there has been no response: • A high head is one that lies completely out of the pelvis.
• exclude occult infection, especially urinary; and either Make sure that the bladder and rectum are empty before
• consider arranging for parenteral iron; or accepting that the head is high.
• if the serum ferritin is normal, check the serum B12 • Primipara: refer by 36 weeks.
and red cell folate (if not already checked) and continue • Multipara: refer if still high at 38 weeks.
combined iron and folate.
• If Hb remains low, seek expert advice. Consider an Hb Premature Rupture of Membranes
electrophoresis regardless of the apparent ethnic origin.
All women suspected of rupturing membranes should be
Rhesus-Negative Women referred for specialist assessment. After 35 weeks of gestation,
the specialist is likely to recommend induction of labour.
• Check antibodies at booking and in primagravida at Before 35 weeks, a short inpatient stay with antibiotic cover
28 and 36 weeks; in multigravida, monthly from 24 is more likely.
weeks. If the maternal anti-D levels are 0.5 to 10.0 IU,
antibody levels are needed every 2 weeks. A level above Postmaturity
10 means that foetal blood sampling is needed and refer-
ral to a foetal medicine unit is indicated. If gestation • If the expected date of delivery was established by USS
allows, delivery, rather than intrauterine transfusion, in early pregnancy, routine induction of labour at 41
may be the preferred option. It is important to realize weeks reduces perinatal mortality (Crowley, 2002). Routine
(and explain) that subsequent pregnancies may behave induction does not cause a rise in the rate of LSCS nor
similarly. in lower maternal satisfaction.
• Women who have not given birth by 41 weeks should • Hyperthyroidism. Refer all pregnant women with hyper-
be offered a pelvic examination and membrane sweep thyroidism to an endocrinologist as soon as they present.
and discussion and information about induction of It is important that they remain euthyroid throughout
labour. the pregnancy. Propylthiouracil is the preferred drug during
pregnancy, given at the lowest effective dose to reduce
the risk of foetal hypothyroidism and goitre (Marx, Amin,
Specific Medical Conditions and Pregnancy & Lazarus, 2008).
Asthma
• Deterioration occurring during pregnancy is usually due Postpartum Thyroiditis
to a reduction in therapy because of a fear that it will • Up to 10% of women develop transient autoimmune
harm the foetus. thyroiditis after delivery. This is usually between 1 and
• Explain that poorly controlled asthma has been linked 3 months and may present with features of hyperthyroid-
to IUGR. ism, but more commonly with those of hypothyroidism
• Explain that beta-sympathomimetics, inhaled steroids, (i.e., fatigue and lethargy), at a time when she is under-
and ipratropium are safe, while oral steroids and theoph- standably tired anyway. Thyroxine is necessary for 6
ylline have been linked to preterm delivery. months, followed by repeat TFTs. Antithyroid drugs are
not usually needed for the hyperthyroid state, but beta-
Diabetes (Types 1 and 2) blockers may be given to control symptoms.
• Optimum control of blood sugars should be achieved by • Follow the patient with yearly TFTs. Up to 20%
the time of conception to reduce the risk of congenital develop hypothyroidism in the subsequent 4 years
malformation. (DTB, 1995).
• The GP should emphasize the importance of good control
throughout pregnancy, even if the majority of the man- Epilepsy
agement will be done by the hospital. • 20% of patients have an increase of fits during pregnancy
• Because of the higher risk of neural tube defects, women due either to poor compliance or to a fall in serum levels
with diabetes should take 5 mg (instead of 400 µg) of because of the physiological changes of pregnancy. Fits
folate daily before conception until the end of week 12. are more common when tired.
• Patients should be referred to the diabetes clinic and seen • Do not stop anticonvulsants or reduce their dose if a
2-weekly before 28 weeks and then weekly. woman presents already pregnant. Any teratogenic effect
• Insulin dose may need to be doubled or tripled, and will will have already occurred. Liaise with her neurologist
need to be reduced immediately after delivery to the pre- to plan the rest of the pregnancy. In general, it is not
pregnant dose. necessary to monitor serum levels of anticonvulsant in
• Patients on oral hypoglycaemic drugs should be changed pregnancy, but be guided by the clinical condition, and
to insulin. only increase the dose of a drug if the frequency of fits
• Blood sugar profiles should show the majority of readings increases.
(pre-meals) below 6 mmol/L. • Encourage the patient to take her treatment correctly.
• Aim to keep the HbA1c at 6% (42 mmol/mol) or below. Poor compliance is the main reason for fits in pregnancy.
• Discontinue angiotensin converting enzyme I (ACE-I) • Ensure that an anomaly ultrasound is performed at 18
and angiotensin receptor blockers before conception or to 19 weeks.
as soon as pregnancy is confirmed. Substitute with a more • Recommend folic acid 5 mg daily to all epileptic women
suitable drug. until the end of week 12. This is particularly important
• Discontinue statins before pregnancy or as soon as preg- in patients taking sodium valproate or carbamazepine or
nancy is confirmed. who have a history of a previous baby with a neural tube
• Women should have serial USSs to exclude macrosomia defect.
and placental insufficiency. They should have examination • Reassure women that the majority have a normal delivery
of the foetal heart at 18 to 20 weeks. and that epilepsy is not an indication for elective caesarean
section.
Thyroid Disorders
Pre-existing Thyroid Disease Inflammatory Bowel Disease
• Hypothyroidism. Women should be advised to delay con- (Fergusson, Mahsu-Dornan, & Patterson, 2008)
ception until thyroid function is stable on levothyrox- • Inflammatory bowel disease (IBD) is not worsened by
ine. Thyroid function should be checked at booking, at pregnancy and may improve. If the disease is quiescent
least once in each trimester, and after any dose changes. at the time of conception, it remains so in two-thirds of
Thyroxine requirements may increase during pregnancy. patients. If the disease is active, two-thirds of patients
Liaise with an endocrinologist as soon as the patient will have ongoing active disease.
falls pregnant with regards to changes in thyroxine • Women with inactive disease have no increased risk of
doses. an adverse outcome. Women with active disease have up
to a 35% miscarriage rate. Crohn’s disease has an increased • Contraception. Hormonal methods should not be started
risk of low birth weight, preterm delivery, and adverse before 4 weeks or before bleeding has stopped.
perinatal outcome. • Rest. Stress the need for rest in the first week, but the
• Assessment of IBD in pregnancy is based on clinical factors mother should also be encouraged to mobilize gently as
(e.g., abdominal pain, stool frequency, and bleeding). soon as possible to prevent deep vein thrombosis (DVT).
Pregnancy affects Hb concentration, erythrocyte sedimen- • Pelvic exercises. Urge the patient to perform pelvic exercises
tation rate (ESR), and serum albumin. C-reactive protein from the first day.
is not altered by pregnancy. • Warn the patient that she will inevitably receive conflict-
• Reassure the woman that it is most important to achieve ing advice from professionals, family, and friends. Parents
the best control of the disease possible and that this seems need to develop their own solutions.
to give the best outcome. All women should be seen • Depression. Ensure there is time to talk about her emo-
urgently by the gastroenterology and obstetric team. tional well-being.
• For the safety of commonly used drugs for inflammatory
bowel disease, see Caprilli et al. (2006). Baby
Depression • Examine the baby, unless this has already been done in
GUIDELINE hospital.
• Vitamin K. If it has not already been given intramuscularly,
O’Keane, V., & Marsh, M. S. (2007). Depression during
pregnancy. BMJ (Clinical Research Ed.), 334, 1003–1005.
give it orally according to local guidelines. If the baby is
National Institute for Health and Care Excellence. (2014). breastfed, it will need to be repeated at 1 and 4 weeks.
Antenatal and postnatal mental health: Clinical management • Registration. Encourage early registration of the baby with
and service guidance. NICE clinical guideline 45. Retrieved the registrar; the start of Child Allowance is dependent
from www.nice.org.uk. on this. Register the baby with the practice.
At 6 Weeks
• Rates of depression are higher during pregnancy than at • Does she have any outstanding questions about what
any other point in a woman’s life. happened during labour or birth?
• About half of postnatal depression starts during pregnancy. • Is she getting enough sleep? Who is sharing in the work of
• Two-thirds of women with recurrent depression will relapse caring for the baby? How is she feeling in herself? Is she
during pregnancy if they stop their antidepressants after feeling depressed? How is she getting along with her partner
conception. (if she has one)? Is she getting any time away from the baby?
• Depression during pregnancy is associated with poorer • Ask about pain in her breasts, back, perineum.
outcomes, especially preterm delivery. • Ask about urinary and faecal incontinence, haemorrhoids,
• Women depressed during pregnancy are more likely to constipation.
drink alcohol and smoke and less likely to attend antenatal • Ask about sex. Around 90% of women will report having
appointments. sex by 10 weeks postpartum, while 2% report not attempt-
• For a discussion about drug treatment for depression ing sex by 1 year (Glazener, 1997). An explanation that
during pregnancy see Chapter 18, Psychiatric Problems. libido is often low in the first year may relieve a couple
who are finding that this is the case.
Drug Misuse • Perform a vaginal examination only if symptoms dictate
or if a smear is due.
• Liaise with local substance misuse services. • Offer contraceptive advice.
• Amphetamines and cocaine: stop the drugs immediately. • Prevention of sudden infant death syndrome. Reinforce the
• Benzodiazepines: withdraw over 4 weeks. importance of:
• Barbiturates: arrange admission. If there is any delay, • the sleeping position (on the back);
maintain the patient on phenobarbital. • not smoking;
• Opiates: arrange an urgent outpatient appointment. Main- • not giving the baby a duvet.
tain the patient on oral methadone meanwhile.
Breastfeeding
Postnatal Care Advantages of Breastfeeding
Mother • Advantages for the baby: less infection, less chance of atopy
or diabetes, better bonding.
• Examine fundal height, perineum, lochia, and breasts. • Advantages for the mother: a lower risk of breast and ovarian
• Feeding. If necessary, reinforce the midwife’s advice on cancer, weight loss, and less postpartum bleeding. It is
breastfeeding (see upcoming discussion). also cheaper.
• The baby’s position should be altered so that the lower

Problems With Breastfeeding lip is nearer the blocked duct.
The majority of problems centre round the infant’s attempts
to remove milk. The baby needs to be in a comfortable Breast Engorgement
position to allow jaw and tongue to drain the milk ducts • The pain of engorgement is one of the most common
under the areola. reasons for stopping breastfeeding in the first 2 weeks.
• Check that mother and baby are managing the following: Treatment is disappointing with cabbage leaves, ultrasound,
• Baby’s chest against the mother’s chest, with baby’s and cold packs being no better than placebo (Snowden,
chin to the breast Renfrew, & Woolridge, 2001).
• Mouth wide open • Give simple analgesia and support.
• Both lips curled back • Attempt to prevent engorgement by removing any obstacles
• Lower lip at the junction of the areola and breast to easy breastfeeding.
• Rhythmic movements of jaw muscles
Mastitis
Sore Nipples • Early mastitis is inflammatory, not infectious, and may
GUIDANCE respond to effective emptying of the breast plus a nonster-
oidal antiinflammatory drug (NSAID).
The Breastfeeding Network. (2009). Differential diagnosis of
• Reassess the feeding position of the baby to ensure that
nipple pain. Retrieved from www.breastfeedingnetwork.org.
uk. milk is removed completely.
• Prescribe an NSAID.
• Treat with flucloxacillin for 10 to 14 days if the patient
is unwell, febrile, or there is any suspicion of abscess
formation. Use erythromycin if the patient is allergic to
• These are usually due to the baby’s tongue rubbing the penicillin. Warn mothers that the milk will change in taste
nipple rather than the areola. and feeding may be harder initially, or that the baby may
• Check that the nipple is positioned in an upward direc- develop diarrhoea and need to feed more frequently.
tion toward the roof of the baby’s mouth. • Consider admission for any woman who is systemically
• Allow breast milk to dry on the nipple when not feeding unwell with mastitis.
or, if the skin is broken, use white soft paraffin. • Breast abscess. If an abscess forms which is large enough
to need draining, admit the patient for incision and
Skin Infection drainage.
GUIDANCE FOR PATIENTS AND HEALTH Suppression of Lactation
PROFESSIONALS
This usually takes 4 to 5 days after stopping breastfeeding.
The Breastfeeding Network. (2009). Thrush and breastfeeding.
It may be very painful and require analgesia and a supportive
Retrieved from www.breastfeedingnetwork.org.uk.
bra. It is usually not necessary to use drugs. However, after
stillbirth, or if there is another good reason to stop lactation,
use bromocriptine 2.5 mg at night for 2 nights, then 2.5 mg
twice a day for 3 weeks. If used for a shorter period, a
• This may be due to candida infection and may present number of patients relapse.
as localized soreness, as pain around the areola and nipple,
or as pain in the breast after a feed. Vaginal Bleeding After 24 Hours
• Treat the mother with miconazole cream and the baby
with miconazole oral gel, whether the baby has signs of • The traditional management of excessive bleeding more
infection or not. It is unnecessary for the mother to remove than 24 hours after delivery (secondary postpartum haem-
the cream before feeding. orrhage [PPH]) is surgical. However, ERCP yields placental
• Consider using oral fluconazole if local treatment alone tissue in less than 30% of these cases and so the majority
is not working. This use is not licensed but WHO rec- of patients do not benefit.
ognizes fluconazole as compatible with breastfeeding. • Mild bleeding. Give:
Download the leaflet “Thrush and Breastfeeding” for • ergometrine 0.5 mg intramuscularly, then 0.5 mg three
guidance on dosing. The unlicensed use must be discussed times a day orally for 4 days; and
with the mother and documented. • antibiotics (e.g., amoxicillin/erythromycin ± metroni-
dazole for 5 days).
Blocked Duct • Severe bleeding, or if the os still admits one finger after 7
• This presents as a hard lump anywhere in the breast. days: admit. Admit more readily if there is no one at
• Get the mother to massage the breast while feeding the home to look after the patient, if she is already anaemic,
baby from that breast. febrile, or otherwise unwell.
Fever the choice is between an SSRI which is present in breast

milk at low levels (e.g., sertraline but not fluoxetine or
• Endometritis. Patients with fever, pain, and foul-smelling citalopram) and a TCA which, with the exception of
lochia are likely to need admission, but early cases could doxepin, appears safe in lactation (Spigset & Martensson,
be treated at home with amoxicillin/erythromycin and 1999). Prescribing antidepressants for a lactating woman
metronidazole. is a case-specific, risk-benefit decision (Wisner, Perel, &
• Be aware that DVT and UTI can cause fever without Findling, 1996).
localized symptoms. • Consider referral if a multidisciplinary mental health team
is able to offer more than the primary healthcare team.
Depression Ideally, if the woman requires admission, this should be
to a mother and baby facility.
• Depression after birth is experienced by one in seven • Refer urgently, to be seen within 24 hours, any woman
women in the year after childbirth. It is distinct from the with ideas of suicide or of harming the baby.
transient blues of the first 10 days and from a puerperal
psychosis, which is likely to need admission. PATIENT INFORMATION AND SUPPORT
• For a discussion of the management of postnatal depres-
sion please see Chapter 18, Psychiatric Problems. Association for Postnatal Illness, 145 Dawes Road, London
SW6 EB, helpline 020 7386 0868, www.apni.org.
Management of Postnatal Depression
• Antenatal and postnatal interventions offered routinely
to all women in an effort to prevent postnatal depression Exhaustion
have been ineffective (Lumley, Austin, & Mitchell, 2004)
apart from the IMPACT programme consisting of a rede- • Around 70% of women will experience extreme tiredness
signed community midwifery model of care (MacArthur and exhaustion in the 6 months after giving birth (Watson
et al., 2002). et al., 1984).
• Antenatal and postnatal interventions offered only to • Tiredness is common, even among women who are not
those women perceived to be at a higher risk of devel- depressed. It is uncommon to find a specific cause, but
oping depression have been ineffective (Lumley et al., anaemia, iron deficiency, and thyroid problems should
2004). be considered.
• Interventions offered to women identified as experiencing • Lack of sleep is one of the most common causes. Taking
depression have been effective and there is strong evidence note of the baby’s sleep patterns, excluding depression,
that postnatal counselling (from active listening to cogni- offering time to talk, encouraging time-out from childcare,
tive behavioural therapy) will reduce depression with a and encouraging sharing the work of looking after the
number needed to treat of two to three (Lumley et al., baby are all simple support strategies.
2004). Simple nondirective counselling by health visitors,
weekly for 8 weeks, doubles the recovery rate (Holden, Common Physical Problems
Sagovsky, & Cox, 1989).
• Check for physical health problems. Women experiencing • Physical problems are common: around 44% of women
the common postnatal physical problems are more likely will experience backache, 26% sexual problems, 21%
to be depressed. Ask about: haemorrhoids, 21% perineal pain, 17% mastitis, 13%
• tiredness. Consider checking Hb and ferritin, and TFTs; bowel problems, 11% urinary incontinence, 6% faecal
• backache; incontinence (Brown & Lumley, 1998; MacArthur, Bick,
• sexual problems; & Keighley, 1997).
• perineal pain; • Unfortunately, there is little evidence to guide our man-
• mastitis and feeding problems; agement of postpartum incontinence or perineal pain.
• urinary and faecal incontinence; Three quarters of women with urinary incontinence at 3
• constipation and haemorrhoids. months postpartum still had the problem 6 years later,
• Offer counselling/psychotherapy. Cognitive behavioural despite conservative nurse-led pelvic floor and bladder
therapy is as effective as fluoxetine in reducing postnatal training management of urinary and faecal incontinence
depression. The choice of treatment can be made by the (Glazener et al., 2005).
woman herself (Appleby, Warner, Whitton, & Faragher,
1997). Neonatal Jaundice
• Offer an antidepressant to the 3% to 5% of women whose
depression is moderate or severe (Oates, 2003) and for • Jaundice within 24 hours of birth: admit.
whom an effective form of counselling/psychotherapy is • Deep jaundice developing after 24 hours:
not immediately available, or who chooses an antidepres- • Admit if the baby is unwell or is at risk because of
sant. An SSRI is recommended. If she is breastfeeding, prematurity, small for dates, or birth asphyxia.
• Otherwise arrange for a serum bilirubin. If the level Crowley, P. (2002). Interventions for preventing or improving the
is 290 mol/L or above, discuss with the paediatric team. outcome of delivery at or beyond term (Cochrane review). In The
• Jaundice still present at 2 weeks: test the urine for bilirubin Cochrane Library (Issue 1). Oxford: Update Software. Retrieved
and the serum for conjugated bilirubin. If either is posi- from www.nelh.nhs.uk/cochrane.asp.
Crowther, C. A., Hiller, J. E., Moss, J. R., et al. (2005). Effect of
tive, admit. The baby may have biliary atresia. The earlier
treatment of gestational diabetes mellitus on pregnancy outcomes.
surgery is performed, the more successful it is likely to The New England Journal of Medicine, 352, 2477–2486.
be (Mackinlay, 1993). Douglas, K. A., & Redman, C. W. G. (1994). Eclampsia in the
• Admit if jaundice is associated with any of the following: United Kingdom. British Medical Journal (Clinical Research Ed.),
• Pale fatty stools 309, 1395–1400.
• Dark yellow urine Drug Therapy Bulletin. (1994b). Folic acid to prevent neural tube
• Failure to thrive defects. Drug and Therapeutics Bulletin, 32, 31–32.
• Poor feeding Drug Therapy Bulletin. (1995). The practical management of thyroid
• Tendency to bleed or bruise disease in pregnancy. Drug and Therapeutics Bulletin, 33, 75–77.
• Enlarged liver or spleen, or ascites Everett, C. (1997). Incidence and outcome of bleeding before the 20th
week of pregnancy: Prospective study from general practice. British
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15
Older People’s Health
IAN REEVES
Keeping Older People Healthy Training the Carers
Screening and Prevention For Patients in Residential Care
Smoking Psychosocial Interventions
General Principles for Carers
Alcohol
Medications for Cognitive Symptoms
Exercise Medication for Behavioural Symptoms
Nutrition Teaching the Patient to Cope With Cognitive Impairment
Managing Frailty Memory Difficulties
Early Intervention Anxiety, Moodiness, and Irritability
Difficulty in Initiating Activities
Supporting Older People at Home Reading
Difficulty Maintaining Living Arrangements Problems in Social Situations
Isolation Financial and Legal Aspects (UK)
Carer Stress Falls
Functional Deterioration at Home Risk Factors
Poor Recovery After an Acute Episode Diagnosis
Suicide Specific Evaluation
Delirium, Acute Confusion, Behavioural and Psychological Management
Symptoms of Dementia (BPSD) Loss of Mobility Community-Dwelling Older People at High Risk of
Recurrent Falls
Confirmation of the Presence of an Acute Confusional
Older People in Residential Care and Assisted Living
State
Settings at High Risk of Recurrent Falls
Diagnosis of the Cause of the Confusion
Vestibular Rehabilitation Exercises
Specific Evaluation
Lower Leg Problems
Possible Investigations
Night Cramps
Management
Diagnosis
Delirium, Acute Confusion
Possible Investigations
Change in Behaviour, With Stress and Distress Behaviour
Management
Evident
Loss of Mobility Restless Legs Syndrome
Deterioration Over a Longer Period Without Acute Illness, Workup
Depression, or Dementia Postural Ankle Oedema
Diagnosis Elder Abuse
Specific Evaluation Sex and the Elderly
Management Sexual Activity in Residential and Nursing Homes
Dementia Legal Aspects
Diagnosis Power of Attorney
Specific Evaluation Detention of an Older Person
Management
General
229
little specific training and a lack of support (Wilson

Keeping Older People Healthy et al., 2011).
Screening and Prevention The National Institute for Health and Care Excellence (NICE)
has specific guidance on the prevention of disease and frailty
• In general, cancer screening programmes continue until in later life as a result of excessive alcohol consumption.
older age. There is little evidence that screening is no
longer worthwhile as people age and most programmes Exercise
recommend continuing to age 75 years, although in the
United Kingdom some routine screening ends at 65. • Sustained low-level activity (e.g., walking) for 30 to 60
However, the benefits and risks of screening older adults minutes on at least 3 days per week (Manley, 1996) will
should be assessed on an individual basis, taking into result in moderate health benefits such as reduced fatigue,
account the patient’s estimated remaining life expectancy, weight loss, increased socialization, improved control of
comorbidities, and the action to be taken as a result of type 2 diabetes, and less shortness of breath on exertion
any screening results. (Rooney, 1993).
• If older people can tolerate their medication, then treating • Older people who exercise develop disability at a quarter
those with high blood pressure and high cholesterol will of the rate of those who do not, even though the exercis-
prolong life expectancy and reduce the risk of heart disease ers have a higher rate of fractures and resultant short-term
and stroke. The decision to initiate in older adults, and disability. Even the very old benefit from exercise because
particularly those 80 years and older, should be individu- of improvements in muscle strength, gait velocity, and
alized based on comorbidities and recognition of problems the ability to climb stairs (Elon, 1996).
that may arise from polypharmacy in this population • Advise regular, safe, physical activity as part of daily activi-
(Gueyffier et al., 1993). ties. Simple advice delivered in primary care is effective
• Influenza and pneumococcal vaccination are recommended in increasing activity for older patients (Elley et al., 2003;
in the United Kingdom and elsewhere for all persons Kerse et al., 1999).
aged 65 years and over. More than 90% of deaths from • Recommend exercise that mimics the activities of daily
influenza are in the over-60 age group (Nichol et al., living (ADLs) such as repetitive sit to stand and walking.
1994). This type of exercise is more acceptable and more beneficial
• Herpes zoster vaccination is also now offered to those for older people.
aged 70 with a catchup programme for those aged 70 to • Referral to physiotherapy for specific strength and balance
79, based on the evidence of cost effectiveness and as this training will reduce falls by 40% in women over age 80
age group is likely to have the greatest benefit from vac- years (Campbell et al., 1997).
cination (Oxman et al., 2005). • Participation in group-based tai chi chuan for older people
is associated with better fitness outcomes, including improve-
Smoking ments in maximal oxygen uptake (VO2 max), muscular
strength and flexibility, and a reduced risk of falls.
• Advise smoking cessation for all older people. Stopping
reduces mortality by 50%, regardless of the age at which Nutrition
a person stops. Nicotine replacement has not been specifi-
cally studied in older adults but can be used (Curb et al., • The National Diet and Nutrition Survey (1989–2016)
1996). Simple advice from primary care doctors is effective in the United Kingdom indicated that in those over the
in smoking cessation (Abdullah & Simon, 2006). age of 65:
• average intake of vitamins and minerals was above
Alcohol recommended levels except for some in residential and
nursing homes;
• Although alcohol consumption decreases with age, 17% • vitamin D status was poor in some, particularly those
of men and 7% of older women exceed safe limits; 1% in residential and nursing homes;
to 5% of older people who drink more than occasionally • poor oral health, especially a lack of natural teeth, was
report that they are “problem drinkers,” males more so associated with poor diet and nutritional status;
than females (Department of Health and Social Security • average intake of sugars and saturated fatty acids
[DHSS], 1994). exceeded recommended levels;
• Simple advice from the general practitioner (GP) may be • average fibre intake was lower than recommended;
effective in reducing alcohol consumption (Anderson, • the diet of older adults is generally better than younger
1993). peers.
• Using brief interventions could reduce excessive drink- • Among independent older people, 3% of men and 6%
ing but there still appears to be a gap between actual of women are underweight, and in nursing and residential
practice and potential for preventive work relat- care, these figures rise to 16% and 15%, respectively
ing to alcohol problems: Primary care doctors report (Finch et al., 1998).
CHAPTER 15 Older People’s Health 231
• Poorer households consume: failure. As a consequence, the frail person is at increased

• less of the following: fruit and vegetables, salads, whole- risk of disability and death from minor external stresses”
meal bread, whole grain and high fibre cereals, and oily (Campbell & Buchner, 1997).
fish; • General practice, as part of the primary healthcare network,
• more of the following: white bread, full-fat milk, table is well placed to detect and treat emerging frailty and
sugar, and processed meat products which are often prevent further deterioration.
high in fat. • The Nottingham Extended Activities of Daily Living
• With regard to nutrition, a national survey of older people (EADL; see Appendix 30) scale may be useful in detecting
in private households (Department of Health, 1992) found unsuspected functional decline. Electronic indexes are
the following: being developed to identify individuals with frailty, but
• Half of the women and a quarter of the men aged 85 the effectiveness of this type of case finding has not been
years and over were not able to cook a main meal alone. proved.
• Only 1 in 10 received Meals-on-Wheels (daily hot • Comprehensive geriatric assessment is associated with
food or weekly frozen meal deliveries). improved outcomes for older people such as reducing
• Assess the risk of dietary deficiencies in older patients hospital admission and readmission as well as physical
especially those with chronic diseases, poor dentition, and cognitive functioning (Ellis et al., 2017).
poor mobility, on low incomes, or who are housebound. • One specific organ manifestation of frailty is the decline in
• Have a low threshold for checking the vitamin B12 status renal function with age—a decline which has implications
of older people especially if they develop neuropsychiatric for the prescribing of drugs that are renally excreted and
symptoms. for the management of conditions which, in themselves,
• Consider checking a patient’s vitamin D status. Vitamin impair renal function. A rise in serum creatinine is a late
D deficiency is common in older people as the skin sign of renal impairment; estimation of the glomerular
decreases in capacity to synthesize the provitamin calcidol, filtration rate (GFR) is more useful.
exacerbated by their low exposure to sunlight. Deficiencies
are more common in the elderly housebound. Poor muscle Early Intervention
strength and weakness is associated with vitamin D defi-
ciency and this weakness may contribute to the risk of • Preventive home visits by health professionals or laypeople
falls. trained in case-finding reduce mortality and admission
• Advise patients, no matter what age, to maintain a healthy to residential care and may promote independence and
diet including at least five portions of fruit and vegeta- improve quality of life for frail older people (Elkan et al.,
bles a day. There is indirect evidence that the modified 2001).
Mediterranean diet is associated with increased survival • Effective management of minor ailments, such as painful
among older people (Trichopoulou, 2005). Refer to a foot problems, may halt declining mobility and improve
dietician if there is concern about dietary intake. Ask long-term outcomes.
the patient to do a food diary for 1 week prior to being • Physical therapy aimed at reducing functional impairment
seen. in older people with moderate frailty will delay functional
• Advise isolated patients to contact social services and local decline (i.e., in ADL score) (Gill et al., 2002).
nongovernmental organizations (e.g., Age Concern). Eating • Maximizing the management of chronic problems and
with others may offer more interaction and reduce loneli- minimizing polypharmacy may halt functional decline.
ness, and there is evidence that family-style meals may The UK National Service Framework recommends an
improve nutrition in older people (Nijs et al., 2006). annual review of medication for people aged 75 and over,
• The involuntary loss of more than 5% to 10% of an older with a 6-monthly review for those taking four or more
person’s usual weight during 1 year is an important clini- medicines.
cal sign associated with increased risk of mortality. Weight
loss should thus be treated as a serious symptom and Supporting Older People at Home
prompt a search for the cause.
• Involuntary weight loss is generally related to one or a • There is considerable variation in the availability of services
combination of four conditions: inadequate dietary intake, for older people internationally and within countries,
appetite loss (anorexia), muscle atrophy (sarcopenia), or both in formal and informal provision. Generally there
inflammatory effects of disease (cachexia). is a multitude of services available aimed at staying healthy,
either maintaining function or providing rehabilitation.
Managing Frailty • Services can be accessed either by direct referral or through
specialist geriatric services.
• Frailty is best regarded as “a condition or syndrome which • A comprehensive assessment is recommended before refer-
results from a multi-system reduction in reserve capacity ring to services.
to the extent that a number of physiological systems are • In general, services are accessed in the United Kingdom
close to, or passed the threshold of symptomatic clinical through a social worker; in Australia by direct referral or
through geriatric assessment services; and in New Zealand Suicide (O’Connell et al., 2004)
through the needs assessment service coordinator.
• Late-life depression often goes undetected and has a sig-
Difficulty Maintaining Living Arrangements nificant adverse impact on quality of life, outcomes of
medical disease, healthcare utilization, morbidity, and
• Refer to an occupational therapist for assessment of ADL mortality.
function at home and provision of equipment. • Suicide rates are almost twice as high in the older adult
• Refer to a social worker to arrange a home carer for compared with the general population, with the rate
housework. highest for white men over 85 years of age.
• Refer to a social worker for Meals-on-Wheels where these • The ratio of parasuicide to completed suicide is much
are available. lower suggesting that suicidal behaviour in older people
• Refer to a podiatrist/chiropodist for foot care. has a much greater degree of intent.
• The main psychologic factor associated with suicide in
Isolation older people is recurrent major depression.
• Poor physical health and disability seem to be associated
• Refer to Age Concern for provision of a volunteer/ with the wish to die.
befriending service. • Consider identifying all those with a known history of
• Refer to a social worker for day centre placement. depressive disorder and question directly as to whether they
• Refer to Dial-a-Driver or social worker to access a sub- have considered suicide. Treat and/or refer as appropriate.
sidized taxi service.
Delirium, Acute Confusion, Behavioural
Carer Stress
and Psychological Symptoms of Dementia
• Ask carers specifically how they are coping. (BPSD) Loss of Mobility
• Ascertain the source of any stress.
• Refer for access to respite services, including day and • A change in mental or physical status of usually well,
night respite, and residential home placement for short independent older people should be taken seriously and
periods. treated urgently. Symptoms of serious illness are often
• Refer for home care for personal assistance. masked and pyrexia may be lower than expected, even
• Refer to an occupational therapist, for assessment of ADL in overwhelming infection.
function and equipment. • Acute change can be due to serious disease without overt
symptomatology related to that disease. Confusion can
Functional Deterioration at Home be as simple as “the patient has suddenly changed, some-
thing is wrong, the level of function has deteriorated.”
Refer to: • Behaviour change may occur and can be described as
• a geriatrician for comprehensive assessment; verbal, vocal, or motor activity that is not explained by
• a day hospital, for multidisciplinary assessment and needs. The cause of this may be subtle and may be due
treatment; to undiagnosed pain, acute illness, or simply the inability
• physiotherapy for musculoskeletal assessment and exercises to communicate needs.
(Gill et al., 2002); • A loss of mobility or taken to bed may be due to serious
• occupational therapy for ADL functional assessment, illness, particularly when the deterioration is rapid.
provision of equipment, and treatment; • Establishing the onset of deterioration and prior function
• a social worker for personal home care; is essential to identify delirium characterized by a fluctuat-
• the district nurses for a bathing service; ing level of consciousness, disorientation, and global cognitive
• a speech language therapist if the condition affects com- deficit. This is almost always due to an organic cause, usually
munication or swallowing; infection, which is hard to diagnose with confidence.
• a dietician.
Confirmation of the Presence of
Poor Recovery After an Acute Episode an Acute Confusional State
* Consider referral to: • A formal assessment is the Confusion Assessment Method
• the appropriate hospital service; (CAM), which states that the patient has delirium if:
• the district nursing service; • there was an acute onset with a fluctuating course;
• the occupational therapist, physiotherapist for functional • there is inattention; and
assessment, and provision of equipment; • there is either disorganized thinking or an altered level
• the social worker for temporary provision of personal of consciousness. Consciousness may be depressed or
care and housework. the patient may be hyperalert.
• The CAM has a sensitivity of at least 94% and a specific- • Chest x-ray (CXR) and other x-rays as suggested by the
ity of at least 90% for the diagnosis of delirium when examination
used by nonpsychiatrists (Inouye et al., 1990).
Management
Diagnosis of the Cause of the Confusion Delirium, Acute Confusion
A diagnosis of acute serious illness or medication toxicity should • Refer for assessment if the diagnosis cannot be clearly
be assumed until proven otherwise. There are many causes for identified and the patient cannot initially be managed at
acute confusion, including (O’Keefe & Sanson, 1998): home, or if a diagnosis is made (e.g., pneumonia or stroke),
• infection, most commonly pneumonia and urinary tract which requires admission.
infection; If treating the patient at home:
• cardiovascular disorder, especially myocardial infarction • Treat any infection with an appropriate antibiotic.
and congestive heart failure; • Stop medications with potential toxicity, especially sedative/
• neurologic disorder, most commonly stroke; hypnotics if they are suspected of causing acute confusion.
• medication interaction or toxicity, particularly psychotropic Check for new medication as a precipitant cause.
or cardiovascular medications; • Treat dehydration with fluids with attention to cardio-
• acute alcohol withdrawal; vascular function.
• acute psychiatric disorder, including psychosis; • Be alert to the possibility of an acute kidney injury.
• electrolyte disturbance, dehydration, hyponatraemia; • Deal with faecal impaction in the usual way, with attention
• endocrine disorder, thyroid disease, diabetes; to follow-up bowel function. Fluids, exercise, and fibre are
• acute change in hearing or vision; all needed for adequate bowel function in older people.
• faecal impaction or retention of urine;
• neoplasia; Change in Behaviour, With Stress and Distress
• acute surgical emergency, such as peritonitis from appen- Behaviour Evident
dicitis or gallbladder disease; • Behavioural management is useful for any agitated patient,
• undiagnosed fracture; including:
• hypothermia. • allowing the person to wander in a safe environment;
• relocating to alternative living arrangements if distress
Specific Evaluation becomes a constant problem and cannot be managed
in the current living arrangement;
A thorough history and physical examination are necessary, • encouraging participation in usual activities;
including: • avoiding confrontation if aggression is a feature;
• history of prior functional level, social support, medica- • avoiding physical restraint; it worsens agitation and
tions, and medical problems. Involving caregivers or family can cause injury.
members to get a history may be necessary; • Treat the cause as discussed, especially undiagnosed
• examination of cardiovascular and neurologic systems; pain.
• musculoskeletal examination, especially for those with • Refer for admission to geriatric or psychogeriatric
reduced mobility. Suspect undiagnosed fracture after assessment ward if the patient cannot be managed at
apparent minor injury with excessive disability; home.
• abdominal examination to exclude an acute abdomen; • Refer to a psychiatrist for evaluation if psychosis is suspected.
• rectal examination; • If no cause is found and the patient has underlying demen-
• investigations will be guided by the history and physical tia, see upcoming discussion.
examination. • Medication. Avoid medication to sedate, as this may
prolong delirium, or shift to a hypoactive delirium instead.
Possible Investigations Reserve antipsychotics, typical or atypical, for patients
who are so agitated that they pose a risk to themselves
• Full blood count (FBC) and erythrocyte sedimentation or to others. They are also associated with increased mor-
rate (ESR) tality, especially from stroke and sudden cardiac death.
• Creatinine, estimated GFR (eGFR), and electrolytes Haloperidol may be associated with less risk than the
• Liver function tests (LFTs) atypicals. Use the smallest dose, for the shortest duration
• Measurement of Vitamin B12 levels possible.
• Thyroid function tests (TFTs) • Avoid benzodiazepines. Even short-acting benzodiazepines
• Midstream urine (MSU) or dipstick urine test accumulate in the older person and worsen the confusion.
• Blood glucose They increase the risk of injury from falling, with patients
• Calcium on higher doses of certain drugs (e.g., oxazepam, fluraz-
• Blood cultures epam, and chlordiazepoxide) having the highest risk
• Electrocardiogram (ECG) (Tamblyn et al., 2005).
• Clock drawing is a useful, nonthreatening test of mental

Loss of Mobility function. Simply draw a circle and ask the patient to
• Treat acute illness as for acute confusion earlier. make it into a clock with the time at “10 til 2.” If
• If minor injury is the cause, and significant disability has numbers are not spread throughout all four quadrants,
resulted and there is no fracture: then the test is suggestive of dementia, particularly if
• give adequate pain relief; short-term recall is reduced for three words (Mini-Cog
• mobilize as soon as possible; Test).
• refer to community physiotherapy or outpatient depart- • A standardized mental test score, such as the Abbrevi-
ment or day hospital services for rehabilitation (Forster, ated Mental Test Score (see box), is only reliable if
Young, & Langhorne, 2002). used precisely (Holmes & Gilbody, 1996). A more well-
• Refer the patient to a day hospital, which can improve validated but time-consuming score is the Mini-Mental
overall functional outcome and reduce service utilization State Examination (MMSE; discussion to come).
when compared with no comprehensive care (Forster
et al., 2002). ABBREVIATED MENTAL TEST SCORE
• Refer for comprehensive inpatient geriatric assessment
Each question scores 1. A score of 6 or less suggests
and management if overall function has deteriorated dementia.
without apparent cause (Stuck et al., 1993). 1. Age (exact number of years)
2. Time (to the nearest hour)
Deterioration Over a Longer Period Without Acute 3. A simple address (e.g., 42 West Street, to be repeated
Illness, Depression, or Dementia by the patient at the end of the test)
4. Year (the current year)
• Deterioration in mental or physical status can be slow 5. The place (exact address or the name of the surgery or
and progressive and not apparently due to acute illness hospital)
(i.e., not delirium). 6. Recognition of two persons present (by name or role)
• If the onset is slow and workup has not shown a treatable 7. Date of birth (correct day and month)
acute cause, then the differential diagnoses of dementia 8. Year of the second World War (1939 or 1945 is enough)
9. Name of the monarch (must be current monarch)
and depression must be considered. 10. Count backwards from 20 to 1 (with no mistakes, or with
mistakes which the patient corrects without prompting)
AMT4 can be found at https://www.racgp.org.au/your-practice/
Diagnosis guidelines/silverbook/tools/abbreviated-mental-test-score/
• The diagnosis of depression is often missed in older people
as the prominence of physical symptoms compounds the
diagnosis. Management
• Dementia is of more insidious onset than depression.
• In depression, cognitive and physical deterioration is worse • Treatment of dementia is outlined in the next section.
in the mornings. • Refer for geriatric medical or geriatric psychiatric assess-
• In depression, insight is present; in dementia, it is rare. ment if the diagnosis is not clear.
• Orientation is poor in dementia and preserved in • Treat depression, if present, with:
depression. • an antidepressant (Wilson et al., 2002); and/or
• Memory loss is characteristically worse for recent events • psychologic therapy, including cognitive behaviour
in dementia but can be similar for recent and remote therapy. Poor access to publicly funded counselling
events in depression. services may limit its availability.
• Follow-up is essential to ensure recovery of function.
Specific Evaluation
Dementia
• Ask about a family and personal history of depression.
• Ask about recent significant events. Bereavement and
GUIDELINES
relocation predispose to social isolation and depression.
• Check functional status and independence in living National Institute for Health and Care Excellence. (2006).
activities. Dementia: Supporting people with dementia and their carers
in health and social care. NICE clinical guideline 42, updated
• Check the patient’s social and financial supports. 2016. Retrieved from www.nice.org.uk.
• Ask about driving. Specific driving assessment may be
necessary later.
• The five-question geriatric depression scale is useful in
diagnosing depression in those without severe dementia • Dementia affects 10% of those over age 65 years and
and works in a variety of settings (Rinaldi et al., 2003). 20% of those over age 80 years.
• Assess the patient’s cognitive state formally using one or • Absolute numbers of those with dementia will increase
both of the following tests or use the “test your memory” exponentially in the next two decades as the world popu-
(TYM) test: lation ages.
• New therapies recently available and currently under TYPES OF DEMENTIA LISTED IN ORDER OF
investigation mean that identification of early dementia PREVALENCE
may become important in the future.
Dementia—Alzheimer Type
• A UK parliamentary committee report (House of Commons,
• Accounts for the majority of dementia cases
2008) criticized the whole range of dementia care: • Insidious onset over several years
• Poor diagnosis: only a third receive a formal diagnosis • Global deficits
• Fragmented home support
• Untrained staff in care homes Vascular Dementia
• Failure to recognize and manage dementia in hospitals • Accounts for 10% of dementia cases, although 29%–41%
of dementia cases autopsied have some vascular pathology
• Stepwise progression
Diagnosis • Bilateral neurological signs
• A history of cardiovascular disease suggests vascular
• Suspect dementia when the patient has: dementia
• impairment in short- and long-term memory, abstract • Risk factors, or a history of risk factors for cardiovascular
thinking, judgment, other higher cortical function, or disease, make vascular dementia more likely
personality change; and Dementia With Lewy Bodies
• the disturbance is severe enough to interfere significantly Dementia plus the following:
with work, social activities, or relationships; and • Balance and gait disorder
• delirium is absent (American Psychiatric Association, • Prominent hallucinations and delusions
1987). • Sensitivity to antipsychotics
* Confirm it with a formal assessment. NICE mentions: • Fluctuations in alertness
• six-item cognitive impairment test; Frontotemporal Dementia
• the General Practitioner Assessment of Cognition • Early loss of personal awareness
(GPCOG); • Early loss of social awareness
• the 7-minute screen; • Hyperorality (putting inappropriate things in the mouth)
• MMSE. • Stereotyped, perseverative behaviours
• A good combination is the Abbreviated Mental Test Score
Prion Disease (Creutzfeldt–Jakob Disease
plus the clock drawing test (see earlier discussion). The [CJD])
former tests orientation and memory; the latter tests praxis • Rapidly progressive symptoms
and spatial perception. • Characteristic electroencephalogram (EEG) pattern of
• A newer test is the TYM test (Brown et al., 2009). It periodic sharp wave complexes
has three advantages over the tests currently in use. It • Pathologic brain tissue diagnosis
tests many different cognitive domains; it is performed • Cerebrospinal fluid (CSF) 14-3-3 protein (high sensitivity
and specificity for diagnosis of CJD).
by the patient alone, taking less than a minute of the
doctor’s or nurse’s time to score it; and it is sensitive for
the detection of early Alzheimer’s disease (AD). Using a
score of 42 or less out of 50 as positive, it is 93% sensi-
tive and 86% specific for AD. The original article and * Examine the patient’s mental state, cognition, neurological
the test itself are available on www.tymtest.com. system.
* Determine, from the history and examination, whether * Examine the cardiovascular system.
it is possible to decide on the cause of the dementia (see * Investigations:
upcoming discussion). This entails examination of the • Vitamin B12 level
patient’s mental state and cognition, a neurologic and • Thyroid function
cardiovascular examination, and a screen for depression. • FBC, creatinine, eGFR, electrolytes, LFTs, calcium,
However, many patients have atypical or nonspecific glucose, ESR
presentations. • CXR, MSU
* Exclude the few cases of reversible cognitive impairment • Syphilis screening may no longer be needed unless
as detailed later in the chapter. The box lists the clinical there is a specific risk factor or they are from certain
features of the types of dementia. parts of the United States.
• Imaging with computed tomography (CT) or magnetic
Specific Evaluation resonance imaging (MRI) scan. Availability will limit
* Take a history from a caregiver or a source other than access in some regions.
the patient. Objective assessment of change (e.g., from • Other options are not recommended or not appropriate
the carer) may help support the diagnosis. Use the for primary care. Imaging with single photon emission
Informant Questionnaire on Cognitive Decline in the computerized tomography (SPECT) or positron emission
Elderly (IQCODE) or the informant component of tomography (PET) is not recommended for routine use.
GPCOG. Genetic testing and genotyping is not currently recom-
* Screen for depression. mended. The CSF 14-3-3 protein may become available
and has overall sensitivity of 92% and a specificity of

80% in diagnosing CJD (2012 systematic review spon- For Patients in Residential Care
sored by the American Academy of Neurology). * Offer residential care staff education about the behavioural
management of patients with AD.
* Explain that a safe wandering space is essential for the
Management older person with dementia to avoid agitation and
General unnecessary restraint, although there is no direct evi-
• It is widely accepted that patients with dementia should dence of benefit (Price et al., 2002). This is especially
be assessed by a psychogeriatrician or geriatrician. useful for sundowning (increased agitation in the late
• Cognition, function, mood, and behaviour as well as afternoon).
general health should be reevaluated in patients with • Scheduled toileting and prompted voiding reduces urinary
dementia every 6 months. incontinence.
• Prognosis. If the family wishes to know, explain that a diag- • Music therapy, simulated natural sounds, and intensive mul-
nosis of AD reduces life expectancy in a way that depends timodality group training may improve overall function.
on the person’s age. At age 65, median survival with AD • Specific assessment and treatment of pain can reduce
is 8 years; at 90, at is 3 years (Brookmeyer et al., 2002). distress (Husebo et al., 2011).
• Driving. Advise patients to inform the Driver and Vehicle
Licensing Agency (DVLA) and their insurance company. Psychosocial Interventions
If there is reasonable concern about public safety, the GP • The main nonpharmacologic approaches that can play a
should inform the DVLA as well. Download and give to role are as follows (Gitlin, Kales, & Lyketsos, 2012):
the patient a leaflet such as “Driving and Dementia” (avail- • Reality orientation; this is based on the belief that
able from https://www.alzheimers.org.uk/sites/default/ continual, repetitive reminders will keep the patient
files/migrate/downloads/driving_and_dementia_factsheet stimulated and better orientated. There is some evidence
_439.pdf). Indicate that the maximum driving time allowed of improved cognition and behaviour in dementia suf-
may be 3 years (Breen et al., 2007). Be wary of being too ferers (Spector et al., 2000).
certain, as clinical assessment is poorly associated with • Memory enhancement strategies; these include setting
driving ability. There is no evidence-based information to shorter term goals, maintaining a social circle and family
guide physician assessment of medical fitness to drive role.
(Molnar, Byszewski, Marshall, & Man-Son-Hing, 2005). • Presenting dementia as a disability that can be accom-
modated and emphasizing continuing abilities.
Training the Carers
• Refer for intensive long-term education and support for General Principles for Carers
caregivers (if available) to delay time to residential care • Treat the patient with respect and dignity and address the
placement. A meta-analysis has shown that interventions patient as though you expect him or her to understand.
designed to support carers can improve their mental health • Recognize the patient’s level of capability but do not talk
and result in the patient with AD staying at home longer down or treat the person as a child.
(Spijker et al., 2008). • Encourage all attempts at personal care and activities of
• Explain the principles of reality orientation and how to daily living.
cope with a person exhibiting behavioural and psycho- • When talking, look directly at the person and maintain
logical symptoms of dementia. eye contact.
• If sleep is a problem, train the carers in sleep hygiene prac- • Use short sentences expressing one thing at a time.
tices: advice about exercise, sleep at regular times, and avoiding • Do not use implied messages; say exactly what is
naps. This has been shown to improve sleep patterns with meant.
daytime benefits as well. To achieve these benefits the carers • If not understood, repeat the message a different way.
will need considerable support (McCurry et al., 2003). Do not shout and do not rush the person.
• Refer for home support. • Mention names of familiar people, the date, week, and
• Consider referral for placement in dementia-specific long- time in all conversations.
term care. This may reduce carer strain. • Reward the patient’s attempts with a smile or compliment.
• Consider whether the patient might be in pain. Many Reality orientation in the patient’s home could include:
older patients suffer from painful conditions. A patient • a large clock and a calendar visible at all times;
with dementia may be unable to communicate that pain • wearing a watch with a date display;
is a problem. Look out for signs of distress (frowning, • having stimulating materials available (e.g., newspapers
looking frightened, aggression, agitation, or withdrawal) and magazines);
and give an analgesic if pain could be the cause (Scherder • a reality orientation board with a schedule of daily activi-
et al., 2005). Specific assessment and treatment of pain ties and reminders (e.g., appointments, at what time to
can reduce distress (Husebo, Ballard, Sandvik, Nilsen, & expect a carer that day, as well a reminder of the day and
Aarsland, 2011). date).
vitamin E nor memantine appears to affect cognitive

Medications for Cognitive Symptoms function or dementia severity;
• The pharmacologic treatment of symptoms is complex • Gingko biloba. There is inconsistent evidence of benefit
and rapidly changing. Each decision should be personal- in cognitive function from taking gingko biloba and
ized to the individual’s needs rather than an improvement no evidence of increased harm. Over-the-counter
on a score. preparations vary considerably in potency compared
to the pure product used in randomized controlled
Alzheimer’s Disease trials (RCTs). Relatives can be advised that the chance
• NICE (2018) has issued guidance on the use of donepezil, of benefit is small.
galantamine, rivastigmine, and memantine for treatment
of AD (available: https://www.nice.org.uk/guidance/ta217). Ischaemic Vascular Dementia and Mixed Dementia
• Cholinesterase inhibitors have been shown to improve • Evidence-based data supporting pharmacologic efficacy
cognition and global functioning in patients with AD. of agents to treat non-AD dementia are less strong than
The improvements are small and come at the cost of an for AD.
increase in adverse effects. The benefits are greatest for • Some research shows that patients with vascular dementia
those in whom the disease is moderate or moderately (VaD) benefit from galantamine (Erkinjuntti et al., 2002).
severe: Galantamine 16 to 24 mg/day is associated with improved
• Donepezil, galantamine, and rivastigmine are now activities of daily living and cognition scores.
recommended as options for managing mild as well • Donepezil 5 to 10 mg/day is associated with small improve-
as moderate AD. ments in cognitive function (Malouf & Birks, 2004).
• Memantine is now recommended as an option for * Offer all the measures appropriate for the secondary pre-
managing moderate AD for people who cannot take vention of cardiovascular disease if the patient’s condition
cholinesterase inhibitors and as an option for managing and life expectancy warrant it. Blood pressure control
severe AD. with perindopril and indapamide has been shown to slow
• Treatment should be continued only when it is considered cognitive decline and reduce disability (Fransen et al.,
to be having a worthwhile effect on cognitive, global, 2003). Blood pressure lowering strategies have not been
functional, or behavioural symptoms. specifically tested for treatment or prevention of VaD
• The severity of the condition may be assessed using the and this effect is likely to be due to further stroke
MMSE, where moderate to moderately severe are represented prevention.
by scores of 10 to 20 points (Table 15.1). However, the • Similarly, other interventions (e.g., aspirin) may not halt
MMSE should not be used if another condition makes cognitive decline, although they are likely to reduce the
it unreliable (e.g., learning difficulties, sensory impair- risk of other vascular disease (Rands et al., 2005).
ment, or linguistic problems). • Based on the observed prevalence of potentially modifiable
• The drug should be initiated by a specialist in the care risk factors (e.g., hypertension, diabetes, inactivity), combined
of patients with dementia. with their associated relative risk for dementia, it has been
• When assessing the severity of AD and the need for treat- estimated that risk factor reductions of 10% to 25% could
ment, healthcare professionals should not rely solely on prevent up to half of dementia cases, if treated early.
cognition scores.
• Families may ask their GP’s opinion of various drugs that Medication for Behavioural Symptoms
can be bought over the counter, such as: • The treatment of delirium, an acute change in mental
• vitamin E supplementation. This might slow functional status characterized by fluctuation in level of conscious-
decline in men with mild to moderate AD but neither ness, attention, and cognitive function, is more complex
than in those with the stable symptoms of dementia
(Britton & Russell, 2002).
• Look for an organic cause, most commonly undiagnosed
TABLE Grading of Severity in Alzheimer Disease pain, infection, and cardiovascular disorder as with well
15.1 According to the Mini-Mental State older people.
Examination • Use behavioural and environmental modification. Reality
Severity MMSE Score orientation (presenting orientation information based on
time, place, and person related) has been shown in one
Mild 21–26
systematic review of small RCTs to improve behaviour
Moderate 15–20 (Spector et al., 2000).
Moderately severe 10–14 • Treat stress and distress behaviour and symptoms of psy-
chosis as follows:
Severe <10
• Psychotropic medication should be cautiously used for
MMSE, Mini-mental state examination. treatment of severe agitation or psychosis with the potential
for harm (e.g., low-dose haloperidol [0.5–1.0 mg orally
or intramuscularly]). Use the smallest possible dose for • try to concentrate in a place free of distractions;
the shortest periods of time. In patients with a parkin- • try to motivate yourself to learn. Make sure you under-
sonian disorder, use an atypical antipsychotic instead. stand and remember all the information;
• Benzodiazepines should be avoided in patients with • improve retention by rehearsing/repeating information
delirium, except in withdrawal syndromes or when and by making associations to improve retention;
other drugs cannot be used. • learn one new thing at a time and try to avoid the
• Withdraw an antipsychotic from a patient with dementia confusion that comes with information overload;
unless the need for it is overwhelming. A UK RCT in • when learning something, study for short periods and
patients with dementia on antipsychotic treatment found frequently, rather than long periods;
that those whose antipsychotic medication was switched • use memory aids (e.g., dry wipe boards, sticky notes,
to placebo had a better chance of surviving over 12 months diaries, a calendar, alarms).
than those in whom the medication was continued (Ballard
et al., 2009). Anxiety, Moodiness, and Irritability
• Advise the patient to:
Teaching the Patient to Cope With • identify the sources of aggravation and try to come
Cognitive Impairment up with a simple solution;
• practice talking to oneself (e.g., stay calm, relax);
• In the early stages of cognitive impairment, the patient • leave the situation explaining that you will need to
can be taught how to minimize the functional disability. calm down and go back to it later;
A simple analogy, such as “If you had a limp you’d use • use relaxation techniques (e.g., tapes, therapy, counting
a stick—but since it’s your memory that’s not so good, to 10);
you need to learn ways to help it,” may help the patient • take regular exercise;
to accept the concept. Another phrase that is readily • get things off your chest by talking or writing.
understood is “Use it or lose it.”
• Family and carers should be involved in this process from Difficulty in Initiating Activities
the beginning. It will help to overcome their frustration • Advise the patient to:
at living with a cognitively impaired relative and, as the • improve your organizational skills (daily routines, use
patient becomes more impaired, they can take over some of a diary, avoid putting things off, priority lists, break
of the tasks. the task into smaller tasks, use simple methods to
• Cognitive impairment is embarrassing and frustrating. achieve things, ask for help or delegate, take time and
The natural tendency is to cover it up. The following not to rush, if necessary, involve others in decision
approach depends on the opposite: accepting it and adopt- making);
ing techniques to minimize its effect. • involve others so they can help motivate you;
• Advise the patient to: • reward yourself when steps are completed. Do this
• do things when most alert; frequently and make the goals reasonable;
• take rest; • if orientation is a problem, use maps and landmarks,
• use relaxation techniques to reduce stress; plan routes, and write directions.
• keep active (e.g., household jobs, visiting, reading)
• be involved in sports, music, and other activities that Reading
involve coordination; • Having to reread something several times is common.
• keep a regular routine; This may be a result of concentration or memory problems
• learn to live with pain and not expect all pain to be but may also result from a change in the brain’s ability
relieved but use analgesics when necessary, particularly to handle a lot of information.
for night pain; • Advise patients to:
• express feelings; • move a finger under each word at a comfortable pace;
• eat well-balanced meals; • block off the words underneath the sentence with a
• reduce alcohol intake; sheet of paper;
• consider counselling. • create a window, in card, that only allows one line to
be read at a time;
Memory Difficulties • take notes to help focus and concentrate on the impor-
• Memory involves learning, storage, and recall. There is tant parts;
little that can be done to improve the storage of memory, • read larger print;
but there are techniques to improve learning and recall. • test themselves on what they have read.
• Advise patients to:
• avoid undermining their confidence. Remember: No Problems in Social Situations
one’s memory is perfect! If you forget something, don’t • Dementia sufferers may have difficulties in understanding
get too upset about it; certain social situations and jump to conclusions that are
• reduce alcohol and avoid sedatives; inappropriate.
• Different patterns of cognitive impairment require dif- • Impaired daily functioning, or a low score on the Not-
ferent specific approaches. See entries under Alzheimer tingham EADL scale (see earlier discussion)
Disease, Parkinson Disease, and Huntington Disease. • Use of psychotropic medications
• Advise patients to: • Those with multiple risk factors are at a higher risk
• clarify what was meant; of falls.
• explain what they believe was said;
• get feedback from others; Diagnosis
• recognize problem situations or people and plan how
to respond beforehand; • Ask older people (aged 75 and older) routinely, once a
• communicate openly and honestly; above all respect year, “Have you had a fall in the last year?”
the others’ position; • Perform a simple gait assessment (to come).
• give the benefit of the doubt to others and assume • Distinguish between hot and cold falls.
they mean no harm. • Hot falls result from major medical conditions such as
stroke, myocardial infarction, or seizure. Treatment of
Financial and Legal Aspects (UK) the acute illness usually entails admission to hospital.
• Patients may be eligible for exemption from Council tax. • Cold falls occur in the absence of serious acute illness.
• Advise that it may be appropriate for someone else to be This part of the chapter deals with management of
given power of attorney. cold falls and those with high risk of falls.
• Refer anyone with a recent cold fall, with recurrent falls
SUPPORT GROUPS in the last year, or with an abnormality of gait or balance
for falls risk assessment.
Alzheimers New Zealand, www.alzheimers.org.nz.
• NICE recommends a multifactorial risk assessment of
Alzheimer’s Association Australia, www.alzheimers.org.au.
Alzheimer’s Society UK, www.alzheimers.org.uk. older people who present for medical attention because
Details of Alzheimer Associations in other countries can of a fall or who report recurrent falls in the past year.
be found on the website of the Alzheimer’s Disease
International, www.alz.co.uk. Specific Evaluation
Age Concern New Zealand, www.ageconcern.org.nz.
A falls risk assessment includes:
AGEUK, https://www.ageuk.org.uk/.
a. history of fall circumstances;
b. medication review;
c. review of chronic medical problems, including alcohol
misuse;
Falls • examination of:
• vision;
GUIDELINES • gait and balance (see upcoming discussion);
American Geriatrics Society/British Geriatrics Society (AGS/ • lower leg strength;
BGS). (2011). Clinical practice guideline on prevention of falls • neurologic system, especially proprioceptive and coor-
in older persons. Journal of the American Geriatrics Society, dination function and including mental status;
59(1), 148. • cardiovascular system, especially heart rate and rhythm,
Falls in older people: Assessing risk and prevention.
lying and standing blood pressure, and the murmurs
Retrieved from https://www.nice.org.uk/guidance/qs86. of valvular disease.
• the environment in which the falls occurred, with atten-
tion to:
• hazards such as loose mats, cords, and unstable
Falls are serious and common for those over age 75 years. furniture;
Risk factors have been identified and specific interventions • lighting levels.
proven to reduce falls. • assessment of the person’s fear of falling and the effect it
is having on functional ability;
Risk Factors • investigations including FBC and ECG.
• Age over 80 years

• Cognitive impairment
• History of fall and fall-related injury SIMPLE GAIT ASSESSMENT
• Arthritis Ask the patient to stand from a chair, without using the arms,
• Depression walk 3 m, turn around and return (the Get Up and Go test)
• Use of mobility aid (Mathias, Nayak, & Isaacs, 1986). Unsteadiness or difficulty
• Gait and balance impairment completing this in less than 30 sec shows a gait and balance
deficit and further evaluation is needed.
• Lower limb muscle weakness
• Visual deficit
Management Vestibular Rehabilitation Exercises

• Results of the evaluation will guide specific management. a. In bed, performed slowly initially then more rapidly:
Most patients needing intervention will have multiple • eye movements: up and down, side to side, focusing on
risk factors. a finger as it moves from 1 m away to 30 cm away
• Multifactorial intervention is more effective than single • head movements: moving head forward, then backward,
intervention in preventing future falls. and turning from side to side
• Refer all with a history of unexplained syncope to a physi- b. Sitting: rotating the head; bending down and standing
cian. Investigations such as a lying and standing blood up with eyes open and closed
pressure or multiday ECG may reveal a cause. Carotid c. Standing: throwing a small ball from hand to hand, turning
sinus hypersensitivity can be detected by carotid sinus through 360 degrees
massage in controlled conditions, including cardiac d. Moving about: walking across the room, up and down a
monitoring. slope, up and down stairs with eyes open and then closed
Community-Dwelling Older People at High Risk of

Recurrent Falls Lower Leg Problems
• Exercise may reduce falls in community-dwelling older Night Cramps (Butler, Mulkerrin, &
persons (Sherrington et al., 2008). O’Keeffe, 2002)
• Review medications and reduce psychotropics. Drugs
that are especially likely to cause falls are benzodiazepines, Nocturnal leg cramps are common occurrences among older,
tricyclic antidepressants, phenothiazines and butyrophe- generally healthy adults, 70% of whom experience them at
nones, antihypertensives, anticholinergics, and hypo- some time.
glycaemic agents (American Geriatrics Society [AGS],
2015). Diagnosis
• Assess vision and refer if necessary. Cataract surgery reduces Most are idiopathic but they are more common in certain
the risk of fall with hip fracture (Tseng, Yu, Lum, & conditions:
Coleman, 2012). • Peripheral vascular disease
• Home environment. Assess whether shoes and slippers fit • Renal failure
properly. Check for loose carpets, poor lighting, or general • Diabetes
cluttering of furniture, as these increase the risk. Refer • Thyroid disease
for modification of other environmental hazards according • Hypomagnesaemia
to the availability of occupational therapy. • Hypocalcaemia
• Review all medical conditions. Treat cardiovascular dis- • Hypokalaemia
orders, including postural hypotension and any cardiac
arrhythmia. Possible Investigations
• Osteoporosis. When patients can tolerate it, consider • FBC and ESR
giving vitamin D3 800 IU daily with calcium cosupple- • Creatinine, eGFR, and electrolytes
mentation to reduce the risk of fractures. The vitamin D • LFTs
may, in addition, increase muscle strength, particularly • Blood glucose
for those who are deficient in vitamin D (Kahwati et al.). • Calcium and magnesium
• TFTs
Older People in Residential Care and Assisted
Living Settings at High Risk of Recurrent Falls Management
• A systematic review of studies of fall prevention in hos- • Treat the underlying cause when possible.
pitals and long-term care facilities found inconclusive • Look for a drug cause: Diuretics, nifedipine, beta-agonists,
evidence for the effectiveness of most approaches. These steroids, morphine, cimetidine, penicillamine, statins, and
approaches appear less effective in the real world than in lithium have all been implicated.
research studies (Coussement et al., 2008). • Explain the technique to abort a cramp as it is starting:
• Refer to physiotherapy for gait and balance training and Forcibly stretch the muscle that is in spasm. Thus for
advice on use of assistive devices. This depends on the cramp in the calf, the patient should stand up with the
availability of physiotherapy services in long-term care. leg straight and forcibly dorsiflex the foot by pressing the
• Consider giving vitamin D3 to achieve over 30 ng/mL. ball of the foot on the floor.
• It is reasonable for clinicians to consider the use of hip • Recommend a trial of calf stretching exercises; they have
protectors in patients at high risk of hip fractures who been found helpful. The technique is to stand 3 feet from
are willing to comply with their use. They are only effec- a wall, rest against it with the arms outstretched, then
tive if actually worn. They do not work at an institutional tilt toward it, keeping the heels on the floor, until the
level (Parker, 2005). stretch is felt in the calves, holding the position for 10
seconds. Do this exercise three times a day, with three tricyclic antidepressants (TCAs), phenytoin, and H2
stretches each time. If after 3 days there is improvement, blockers.
continue it long term. • Explain what to do during an attack: walk about, stretch
• Drug treatment: the legs, have a bath, do something interesting as a
• Quinine sulphate is not recommended. Quinine carries distraction, or massage the legs.
the risk of a severe sensitivity reaction in 1 in 1000 • Nonpharmacologic therapy options for prevention
to 3500, of which the main manifestation is severe include avoidance of aggravating drugs and substances
thrombocytopenia. Hepatitis and haemolytic uraemic such as caffeine, mentally alerting activities, exercise,
syndrome have also been described. It also is very dan- leg massage, and applied heat. In patients with mild
gerous in overdose. Because of these risks the US Food and/or intermittent symptoms, these therapies may
and Drug Administration has banned its marketing be sufficient for symptom relief.
for cramp, but it remains licensed for cramp in the • Consider drug treatment for patients with symptoms
United Kingdom. that are sufficiently severe and frequent. Be wary of
• There is limited and inconsistent evidence that calcium treating mild and infrequent symptoms with indefinite
channel blockers, vitamins, minerals, or naftidrofuryl medication duration. Options include:
reduce the frequency of cramps (Blyton, Chuter et al., • a dopamine agonist. These classes of drugs have
2012). been shown to be effective compared with placebo,
but the diagnostic uncertainty and side effects often
Restless Legs Syndrome limit their use.
• a calcium channel ligand (e.g., gabapentin), which
REVIEWS appears to improve symptoms in patients with
moderate to severe restless legs syndrome (RLS)
European Federation of Neurological Societies/European
Neurological Society/European Sleep Research Society particularly if neuropathy is present.
(EFNS/ENS/ESRS). (2012). Guideline on management of
restless legs syndrome. European Journal of Neurology,
PATIENT SUPPORT AND INFORMATION
19(11), 1385.
Leschziner, G., & Gringras, P. (2012). Restless legs RLS-UK: www.rls-uk.org/.
syndrome. BMJ (Clinical Research Ed.), 344. Bandolier: www.bandolier.org.uk (search on “restless
legs”).
• The syndrome is characterized by creepy crawly sensations

in the lower limbs. These occur at rest in the evenings or Postural Ankle Oedema
at night and are temporarily relieved by moving the limbs.
The minimum diagnostic criteria proposed by the Inter- • Many patients are given diuretics inappropriately for
national Restless Legs Syndrome Study Group (IRLSSG) postural oedema. Diuretics can cause paradoxic oedema.
and National Institutes of Health (NIH) are: • Assess carefully to exclude a cardiac, renal, or hepatic
• urge to move legs often accompanied by an unpleasant cause.
feeling; • Treat postural oedema by advising the patient to:
• onset or worsening of symptoms when at rest; • keep active;
• partial or complete relief by movement for as long as • elevate legs when sitting; and
movement continues; • use support hosiery.
• circadian pattern of symptom expression with high • Give a diuretic only where the oedema is too severe to
frequency of occurrence in evening and at night (often permit the patient to pull on a support stocking; and
interferes with sleep). even then, only give it for a maximum of 3 weeks.
• Evidence is inconsistent for association with iron deficiency, • Consider a trial without diuretics for those already started
but screening is generally recommended by consensus on them. In one study, 85% of those suitable were suc-
panels. cessfully withdrawn from diuretics (de Jonge et al.,
1994). Be aware that even patients who do not need their
Workup diuretics are likely to suffer an initial increase in oedema
1. FBC as a rebound phenomenon, which may take 6 weeks
2. Serum ferritin to settle.
3. Folic acid
4. B12 Elder Abuse
5. Creatinine, eGFR, and electrolytes
• Examine to exclude peripheral neuropathy. • A UK study has shown the prevalence of abuse in the
• Review the patient’s drugs. Possible causes are neuro- patient’s own home to be significant, with physical
leptics, lithium, beta-blockers, calcium channel blockers, abuse in 2%, verbal in 5%, and financial in 2% (Ogg
& Bennett, 1992); 45% of carers of older people in respite PROFESSIONAL AND PATIENT INFORMATION
care admitted to some form of abuse (Homer & Gilliard,
Information leaflets are available from Action on Elder Abuse,
1990).
PO Box 60001, Streatham, London SW16 9BY, tel. 020 8835
• There are no statutory guidelines or legislation; however, abuse 9280; helpline 080 8808 8141, www.elderabuse.org.uk.
is a crime. Intervention should always be interdisciplinary. For advice on what to do when you suspect patients are
• Abuse may take the form of: being abused in residential or nursing homes, download the
• physical: hitting, slapping, pushing, kicking, misuse of Commission for Social Care Inspection leaflet “Abuse of
Older People—What You Can Do to Stop It,” available from
medication, restraint, inappropriate sanctions;
http://lx.iriss.org.uk/sites/default/files/resources/Abuse%20
• psychological: emotional abuse, threats of harm or aban- of%20older%20people.pdf.
donment, deprivation of contact, humiliation, blaming,
controlling, intimidation, coercion, harassment, verbal
abuse, isolation or withdrawal from services or sup-
portive networks;
• sexual: rape and sexual assault or sexual acts to which Sex and the Elderly
the vulnerable adult has not consented, could not
consent to, or was pressurized into consenting to; • Sexual activity is common in the elderly with studies
• financial or material abuse: theft, fraud, exploitation, indicating at least 50% of 60- to 90-year-olds remain
pressure in connection with wills, property or inheri- sexually active. Although coitus declines in frequency,
tance or financial transactions, or the misuse or misap- interest is maintained in different ways (e.g., masturba-
propriation of property, possession, or benefits; tion, oral sex). There is a shift from genital sex to
• neglect and acts of omission: ignoring medical or physical intimacy.
care needs, failure to provide access to appropriate • In a study looking at the secular trends in health, the
health, social care or educational services, the with- sexual activity of Swedish 70-year-olds in 1971 was com-
holding of the necessities of life such as medication, pared with 70-year-olds in 2001. Men and women from
adequate nutrition; the latter group reported higher satisfaction with sexuality,
• discriminatory abuse: racist, sexist, based on a person’s fewer sexual dysfunctions, and more positive attitudes to
disability and other forms of harassment. sexuality in later life (Beckman et al., 2008).
• Abuse can occur anywhere, including in: • Medication may cause changes in sexual function, par-
• someone’s own home; ticularly beta-blockers, antidepressants, and antipsychotics
• a carer’s home; (Montejo, 2015).
• day care; • Phosphodiesterase (PDE) 5 inhibitors are used in the
• a residential or nursing home; treatment of erectile dysfunction. They work in older
• hospital. men, but less than in younger men (Müller et al., 2007).
• Abuse may occur for many reasons: • For men, explain the normal changes associated with ageing
• In the home the causes include poor quality long-term (Masters & Johnson, 1970):
relationships, a carer’s inability to provide the level of • The urgency of sexual interest declines from the late
care needed, a carer with mental or physical health fourth decade.
problems. • Erection is less frequent.
• In other settings it may be a symptom of a poorly run • Erection needs more stimulation, especially tactile.
establishment, especially when staff are inadequately • Erection is more difficult to sustain.
trained and poorly supervised. • Turgidity of the erection diminishes.
• Suspect abuse when: • Ejaculation is less forceful.
• there is delay in seeking medical help; • Refractory period is longer.
• there are differing histories from patient and carer, • There may be periods of difficulty establishing an erec-
especially if explanations are implausible; tion and the frequency of these episodes increases over
• there are inconsistencies on examination; time. However, the pleasure derived from sex may not
• there are frequent calls for visits by the GP or accident be significantly altered.
and emergency (A&E) department attendances; • For women, explain the normal changes associated with
• the carer does not accompany the patient when that ageing (Masters & Johnson, 1966):
would be expected; • Arousal requires more stimulation.
• there is abnormal behaviour in the presence of the • Lining of the vaginal wall thins and vaginal lubrication
carer (e.g., fear or withdrawal). decreases.
• Discuss the situation with the patient, carer, and involved • There is less vaginal vasocongestion and tensing.
care agencies. Further action will depend on the wishes • Uterine contractions are fewer.
and competence of the patient and the nature and severity • Clitoral detumescence is rapid.
of the abuse. • The capacity to achieve orgasm remains into old age
• Discuss the case with Action on Elder Abuse (see box). but the length of time taken to achieve it increases.
• Consider the risk of sexually transmitted infection in Detention of an Older Person

older people embarking on new relationships and counsel
them on the importance of safe sex. GUIDELINE
• Be willing to discuss sexual issues with all, but particularly
UK: The Mental Capacity Act 2005. Retrieved from https://
with:
www.legislation.gov.uk/ukpga/2005/9/contents.
• women undergoing gynaecologic operations;
• men undergoing prostate treatment;
• patients who have severe arthritis (changing position
and timing of analgesia may be needed); • In 2005 an amendment was made to the Mental Capacity
• patients who have had a stroke or myocardial infarction. Act, known as the Deprivation of Liberty Safeguards,
which took effect in England and Wales. The use of these
Sexual Activity in Residential and safeguards should be considered when someone is subject
Nursing Homes to continuous supervision or control, and whether they
are free to leave a given environment.
• Patients in nursing or residential homes may continue to • These safeguards make provision for the use of restrictions
have an interest in sexual activity. Sexual behaviour is and restraint (including, for example, the use of locks to
often seen as a problem rather than an expression of a stop people going to different parts of a building or the
need for love and intimacy. Men with dementia are more use of bed rails). They apply only to hospitals and care
likely to exhibit inappropriate sexual behaviour. homes but the Court of Protection can authorise the
• Inappropriate behaviour: Advise staff about sexuality in deprivation of liberty in other settings.
the elderly and advise them against unintentionally giving • They are applicable to those who lack capacity to consent
cues that may be misinterpreted (e.g., when washing them). to the restrictions placed upon them.
In addition, consider: • An application for the deprivation of someone’s liberty
• behavioural approaches: for example, tell the patient should be made to the local authority who will then
that the behaviour is inappropriate, isolate the patient appoint an assessor to adjudicate as to whether the act
from residents of the sex being subjected to inappro- applies or whether other legislation (such as the Mental
priate behaviour, use clothing that opens at the back Health Act) may be more applicable.
for a male who exposes his genitals; • Under the act, those who lack capacity and who do not have
• drug therapy: there is little evidence but consider selec- the support of family or friends to make decisions are entitled
tive serotonin reuptake inhibitors (SSRIs) in very dif- to have access to an independent mental capacity advocate
ficult cases. (IMCA) who can be appointed by social services.
• In Australia and New Zealand, refer to the local geriatric
assessment team if there is a need for legal intervention
Legal Aspects to ensure patient safety and well-being.
Power of Attorney
• At an early stage of declining mental function, suggest References
to the family that they consult a solicitor about a lasting
power of attorney (LPA) before the patient is incapable American Geriatric Society. (2015). Updated beers criteria for poten-
tially inappropriate medication use in older adults. Journal of the
of signing the form.
American Geriatrics Society, 63(11), 2227–2246.
• To sign, the patient must be capable of understanding the American Psychiatric Association (1987). Diagnostic and statistical
implications of so doing, and may be capable of this even manual of mental disorder (3rd ed. rev.). Washington, DC: APA.
if incapable of managing his or her affairs. If it is left too Anderson, P. (1993). Effectiveness of general practice interventions for
late, it is then necessary to apply to the Court of Protection patients with harmful alcohol consumption. The British Journal of
to appoint a receiver, and this is much more cumbersome. General Practice: The Journal of the Royal College of General Prac-
• The LPA must be registered before it can be used. Note titioners, 43, 386–389.
that this is different from the registration of the older Ballard, C., Hanney, M. L., Theodoulou, M., et al. (2009). The dementia
power, the enduring power of attorney (EPA), which only antipsychotic withdrawal trial (DART-AD): Long-term follow-up of
becomes necessary when the person who signed it is, or a randomised placebo-controlled trial. Lancet Neurology, 8, 151–157.
is becoming, incapable of managing his or her own affairs. Bates, B., Lennox, A., Prentice, A., Bates, C., & Swan, G. (2013).
National Diet and Nutrition Survey. Department of Health and Food
standards agency, 1–79. Retrieved from https://assets.publishing.
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power of attorney, Court of Protection, and other relevant legal _data/file/551352/NDNS_Y5_6_UK_Main_Text.pdf
aspects in the England and Wales go to https://www.gov.uk/
Beckman, N., Waern, M., Gustafson, D., et al. (2008). Secular trends
government/organisations/office-of-the-public-guardian. For
Scotland, http://www.publicguardian-scotland.gov.uk/home. in self reported sexual activity and satisfaction in Swedish 70 year
olds; cross sectional survey of four populations, 1971–2001. British
Medical Journal (Clinical Research Ed.), 337, 151–154.
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16
Contraception, Sexual
Problems, and Sexually
Transmitted Infections
LINDSEY POPE
Hormonal Contraception Risks Usually Outweigh Benefits (UKMEC3)
Combined Oral Contraceptive Pill Adverse Effects
Effectiveness Timing of Insertion
Assessment of the Patient Removal or Refit
Contraindications Timing of Removal
Choosing a Combined Pill Prophylactic Antibiotics for Cardiac Disease
Taking the Pill: Procedure and Advice Follow-Up
Stopping the Combined Oral Contraceptive Intrauterine System
Advice Barrier Methods
Postponing or Avoiding Bleeds Male Condoms
Follow-Up Female Condom
Changing the Pill Because of Side Effects Diaphragms
Combined Transdermal Patch Cervical Caps
Combined Vaginal Ring Fertility Awareness
Progestogen-Only Pill Emergency Contraception
Patients for Whom the Progestogen-Only Pill Is Particularly Hormonal Preparations
Indicated Intrauterine Device Insertion
Unacceptable Health Risk (UKMEC4)
Regimen for Hormonal Emergency Contraception
Risks Usually Outweigh Benefits (UKMEC3)
Taking the Progestogen-Only Pill: Procedure and Advice Contraindications
Advice Special Situations
Side Effects Management
Injectable Progestogens Follow-Up
Preparations
Contraception in Special Cases
Indications
Unacceptable Health Risk (UKMEC4) Contraception and Young People
Risks Outweigh Benefits (WHO3) Combined Oral Contraceptive
Before Starting Contraception and Older Women
Initiating Treatment and Follow-Up Women on the Combined Oral Contraceptive
Etonogestrel-Releasing Implant: Nexplanon Women on the Progestogen-Only Pill
Unacceptable Health Risk (UKMEC4) Hormone Replacement Therapy
Risks Outweigh Benefits (UKMEC3) Contraception and Learning Disability
Monitoring Sterilization
Problems With the Implant Which Partner Should Be Sterilized?
Nonhormonal Methods of Contraception Reversal
Intrauterine Devices Termination of Pregnancy
Unacceptable Health Risk (UKMEC4) The Patient Under 16 Years Old
246
CHAPTER 16 Contraception, Sexual Problems, and Sexually Transmitted Infections 247
Infertility Specific Management of Sexually Transmitted Infections

Investigations Gonorrhoea
Assessment of the Woman Genital Gonorrhoea (Uncomplicated)
Assessment of the Man Chlamydia Infection (Uncomplicated)
Normal Values For Semen (World Health Organization) Screening for Chlamydia
Advice Nonspecific Urethritis
The Role of the General Practitioner After Referral Trichomonas vaginalis
Sexual Problems Anogenital Warts
Erectile Dysfunction Soft, Nonkeratinized Warts
Workup Keratinized Warts
ED as a Risk Factor for Cardiovascular Disease Herpes Genitalis
Treatment in General Practice Management of a First Symptomatic Episode
Non-responders Management of Recurrences
Referral Syphilis
Lack of Sexual Desire Human Immunodeficiency Virus and Acquired
Dyspareunia Immunodeficiency Syndrome
Vaginismus Prevention and Early Diagnosis
Disorders of Orgasm Advising on Safer Sex and Avoiding High-Risk Activities
Premature Ejaculation The Human Immunodeficiency Virus Antibody Test
Retarded Ejaculation Human Immunodeficiency Virus Testing
Orgasmic Problems in Women Managing the Human Immunodeficiency Virus-Positive
Patient in the Community
Sexually Transmitted Infections and Human Routine Care
Immunodeficiency Virus Symptoms Needing Special Consideration
Patients Requiring Investigation by the General Venepuncture
Practitioner Post-Exposure Prophylaxis for Healthcare Workers
Patients Requiring Management by the General Practitioner (Including Needlestick Injuries)
Contact Tracing (Partner Notification) Post-Exposure Prophylaxis Following Sexual or Other
Non-Occupational Exposure
Principles of Treatment
Notification and Surveillance
Workup for Cases to Be Managed in Primary Care
• Patients wanting to go to a family planning clinic or

CONTRACEPTION who are being referred from general practice can get
details of a convenient clinic from the FPA. Their website
INFORMATION allows a search on a map with details right down to
For Professionals street level.
Faculty of Sexual & Reproductive Healthcare, www.fsrh.org
For Patients Hormonal Contraception

Family Planning Association, 23–28 Penn Street, London, N1
5DL, tel. 020 7608 5240, www.fpa.org.uk.
Combined Oral Contraceptive Pill
Effectiveness
With perfect use of the combined oral contraceptive (COC)
failure rates are as low as 0.3% in the first year (Trussell,
• When giving contraceptive advice it is good practice if 2007). However, with typical use this rises to 8%.
this is backed up with appropriate and accessible written
information. The Family Planning Association (FPA) Assessment of the Patient (Clinical Effectiveness
provides a range of leaflets covering all methods, which Unit, 2006a)
is regularly revised and is available through the local Health The following are the essential components of the assessment
Promotion Unit or from the website (see box). before initiating the COC:
• These leaflets should be used when a patient is deciding • Menstrual and obstetric history
on a method, initiating a method, and from time to time • History of migraine—presence of aura
as a refresher. However, leaflets are not a substitute for • Past or current illnesses that might represent contraindi-
discussion (Jones, 2008). cations
• Family history of venous thromboembolism (VTE), myo- paraesthesiae, disturbance of speech, first ever migraine
cardial infarction (MI), cerebrovascular accident (CVA), attack after starting COC, status migrainosus. Migraine
hypertension, and breast cancer without aura is relatively safe (this includes blurred vision,
• Current drug therapy (prescribed and over the counter photophobia, phonophobia, and flashing lights affecting
[OTC]) the whole visual field) for those who did not first develop
• Allergies it while on the COC.
• Blood pressure (BP) measurement (defer starting the COC • Breast cancer: current
until 8 weeks after delivery in women who have had • Diabetes: nephropathy, retinopathy, neuropathy, or other
preeclampsia, even if BP is normal) vascular disease
• Baseline height/weight: body mass index (BMI) (impor- • Viral hepatitis: acute or flare
tant, as it is a risk factor for VTE) • Cirrhosis: severe (decompensated)
• No blood tests are necessary before first prescription of • Liver tumours: benign (hepatocellular adenoma) or malig-
a COC without specific clinical indication. nant (hepatoma)
• In asymptomatic women breast and pelvic examinations • Systemic lupus erythematosus (SLE): with positive or
are not recommended before first prescription of a COC unknown antiphospholipid antibodies
(Stewart et al., 2001).
Risks Usually Outweigh Benefits (UKMEC3)
Contraindications (Clinical Effectiveness Unit, 2016)
UK Medical Eligibility Criteria (UKMEC) give four categories • Breastfeeding: between 6 weeks and 6 months postpartum
of condition in which use of the COC is (or is not) and primarily breastfeeding
contraindicated: • Postpartum (in non-breastfeeding women): less than
• UKMEC1 is a condition for which there is no restriction 21 days
on use of the method. • Smoking: aged 35 years and older and smoking less than
• UKMEC2 is when the advantages of the method gener- 15 cigarettes/day; aged 35 years and older and stopped
ally outweigh the theoretical or proven risks. smoking less than 1 year ago
• UKMEC3 is when the theoretical or proven risks usually • BMI: 35 and above
outweigh the advantages. Provision of a method requires • Multiple risk factors for arterial cardiovascular disease
expert clinical judgment and/or referral to a specialist • Hypertension: on treatment with BP adequately con-
contraceptive provider. trolled; elevated BP 140 to 159 systolic or 90 to 94
• UKMEC4 is a condition that represents an unacceptable diastolic
health risk and the method should not be used. • Family history of VTE: in a first-degree relative aged 45
years and younger
Unacceptable Health Risk (UKMEC4) (Clinical • Immobility: wheelchair use, debilitating illness
Effectiveness Unit, 2016) • Hyperlipidaemia: familial hypercholesterolaemia, for
• Breast feeding: less than 6 weeks postpartum (Clinical example
Effectiveness Unit, 2009b) • Migraine: past history of migraine with aura 5 or more
• Smoking: aged over 35 years and smoking more than 15 years ago, at any age
cigarettes/day • Breast cancer: past history of and no evidence of recur-
• Cardiovascular disease: multiple risk factors for arterial rence for 5 years; carriers of known mutations associated
cardiovascular disease such as older age, smoking, diabetes, with breast cancer (e.g., BRCA1)
and hypertension. Also, atrial fibrillation and cardiomy- • Gallbladder disease: symptomatic medically treated or
opathy with impaired cardiac function. current
• Hypertension: high BP (≥160 systolic or ≥95 diastolic) • History of cholestasis: past COC related
• Vascular disease: peripheral vascular disease, hypertensive • Potent enzyme-inducing drugs: rifampicin, rifabutin, and
retinopathy, and transient ischemic attacks (TIAs) certain antiepileptic drugs
• Venous thromboembolism: current or past history • Organ transplant: complicated by graft failure
• Major surgery with prolonged immobilization
• Known thrombogenic mutations: such as factor V Leiden, Arterial and Venous Thrombosis
prothrombin variant G20210A, protein S, protein C, • The risk factors for VTE and arterial disease should be
and antithrombin III deficiencies assessed separately.
• Ischaemic heart disease: current or past history • Age is a risk factor common to both conditions. Many
• Stroke: history of CVA of the risk factors can be viewed as being on a sliding
• Valvular heart disease: complicated by pulmonary hyper- scale—for instance there is not suddenly a problem with
tension, atrial fibrillation, history of infective endocarditis age on the 35th birthday.
• Migraine, with aura (MacGregor, 2007): The following • Smoking status, weight, and immobility are the only
suggest transient ischaemia and preclude use of the COC: factors that may be changed in the future and the suit-
loss of a part of the visual field, unilateral weakness/ ability for the COC reviewed.
RECOMMENDATION
Choosing a Combined Pill
Recommend the information leaflet from the Family Planning
• Pill formulations contain one of eight progestogens.
Association at www.fpa.org.uk.
• The initial dose of oestrogen should normally be in the
range 20 to 35 µg combined with a low or standard dose
of progestogen.
• A monophasic COC containing 30 µg ethinylestradiol with
norethisterone or levonorgestrel is a suitable first pill.
• As from June 1999, desogestrel- and gestodene-containing Higher Doses of Oestrogen
pills were recommended again as first line COCs, fol- Formulations containing 50 µg of ethinylestradiol should
lowing the pill scare of October 1995 (Anon., 1999). not be used unless specific individual circumstances warrant
However, in view of the apparent increased risk of VTE a higher dose:
with these preparations (relative risk [RR] 1.7 compared • Long-term use of an enzyme-inducing drug. NICE recom-
to levonorgestrel or norethisterone pills [Kemmeren, Algra, mends that women taking enzyme-inducing antiepileptic
& Grobbee, 2001]) the slightly increased risk of VTE drugs are started on 50 µg; if breakthrough bleeding (BTB)
should be explained to the patient (Table 16.1) and these occurs, the dose may be increased to 75 or 100 µg daily
pills should not be used in those with a risk factor for (Stokes et al., 2004).
VTE. Norgestimate-containing pills have similar rates of • Persistent BTB on a standard-strength COC, provided
VTE to levonorgestrel pills (Jick, Kaye, Russmann, & no other cause is found.
Jick, 2006). • Past true COC method failure, suggesting unusually rapid
• For all COCs the risk of VTE is greatest in the first year metabolism or malabsorption (an alternative is tricycling
of use. For COCs containing levonorgestrel, for instance, and shortening the pill-free interval [PFI] to 4 days; see
the RR for VTE in the first year is 6.6 compared to upcoming discussion).
women not taking the pill, falling to 1.3 after 5 years of
use (Brechin & Penney, 2004). Special Cases
• Desogestrel- and gestodene-containing pills are probably • Recommend an alternative method when there has been
best avoided for young first-time users. Their risk of VTE a previous failure of COC or where pill efficacy may be
is 3.1 times the risk they would run with a levonorgestrel reduced because the patient:
or norethisterone preparation (Kemmeren et al., 2001). • is on long-term hepatic enzyme-inducing drugs (e.g.,
These brands may be useful, however, for those who have for fungal infection, tuberculosis [TB], epilepsy, daytime
side effects, for acne sufferers, or for those with cycle sleepiness, or human immunodeficiency virus [HIV])
control problems. or taking OTC drugs (e.g., St John wort [consult the
• Co-cyprindiol (ethinylestradiol with cyproterone acetate) is British National Formulary (BNF)]) (Clinical Effec-
licensed as an acne treatment but is an effective contracep- tiveness Unit, 2005a); or
tive, too. It should be used only in those with significant • has severe malabsorption.
acne. There is a higher risk of VTE than with levonorgestrel • If an alternative method is unacceptable, consider:
pills: RR 3.9 (Vasilakis-Scaramozza & Jick, 2001). • starting a high-dose pill or two low-dose pills to give
• Women react individually to the pill; if side effects are at least 50 µg of ethinylestradiol; or
experienced it is well worth trying at least one other brand • running three packs of pills together (the tricy-
before abandoning the method. cle regimen) plus reducing the 3-monthly PFI to
4 days.
• If BTB still occurs, try 75 or 100 µg per day (Stokes
et al., 2004).
TABLE Phased preparations:
16.1 Risks of Venous Thromboembolism
• give a better bleeding pattern for a lower monthly dose
Background rate of VTE in 5/100,000/year (has been shown for levonorgestrel preparations) (Rosen-
healthy nonpregnant women berg & Long, 1992);
not taking a COC • are more expensive than fixed-dose preparations, not least
Healthy women taking 15/100,000/year because these products attract two dispensing fees (bipha-
levonorgestrel or sics) or three dispensing fees (triphasics) in the United
norethisterone COCs Kingdom;
Healthy women taking gestodene 25/100,000/year • have a reduced margin for error, especially early in the
or desogestrel COCs packet;
• may cause premenstrual tension–like symptoms toward
Pregnancy 60/100,000/year
the end of the packet;
COC, Combined oral contraceptive; VTE, venous thromboembolism. • are less flexible when a patient wants to postpone
a period.
Taking the Pill: Procedure and Advice Stopping the Combined Oral Contraceptive
Starting the Pill (Clinical Effectiveness Unit, 2006a) Alternative methods of contraception are needed from the
• Days 1 to 5 of the cycle: If started on or before day 5, then day of stopping the COC, not the end of the PFI.
no other precautions are necessary.
• After day 5, other precautions should be used for the first Advice
7 days. Mode of Action
• Changing from a hormonal method: COC can be started The main action of the pill is to suppress the normal cycle
immediately if the previous method has been used con- so that ovulation does not occur; the periods while on the
sistently and correctly, or if it is reasonably certain she is pill are withdrawal bleeds. Within 7 days of use the ovaries
not pregnant (Table 16.2). There is no need to wait for are fully suppressed. Conversely, during the PFI there is no
the next period. significant follicular development unless the PFI is lengthened
• After childbirth: If starting at the end of the third beyond 7 days.
week postpartum, no other precautions are needed.
A later start necessitates extra precautions for 7 days. Risks
Note that the earliest recorded ovulation after delivery is • Venous thromboembolism. The risk of VTE while using
day 30 (Guillebaud, 1989), so waiting until a postnatal any COC is increased but is less than the risk of
examination is not an option unless a woman is exclu- VTE during pregnancy (see Table 16.1) (Brechin &
sively breastfeeding. Penney, 2004).
• After miscarriage or termination of pregnancy (TOP): Start • Myocardial infarction. The risk of MI on a COC is con-
within 7 days. If starting later, extra precautions are needed fined to smokers (RR 9.5 compared to nonsmokers not
for 7 days. The earliest recorded ovulation after TOP is on COC) (Khader, Rice, John, & Abueita, 2003) and
day 16 (Guillebaud, 1989). those with arterial risk factors. Women who do not smoke,
who have their BP checked, and who do not have hyper-
Changing the Pill tension or diabetes are at no increased risk of MI on a
• Same or higher strength oestrogen but the same proges- COC, regardless of their age.
togen. Start it after the 7-day break. No extra precautions • Ischaemic stroke. The risk of ischaemic stroke in COC
are necessary. users is increased (RR 2.7) (Chan et al., 2004). Among
• Lower strength oestrogen or a different progestogen. Omit women with no history of migraine, who do not smoke,
the 7-day break. If the break is not omitted, extra precau- have their BP checked, and who do not have hyperten-
tions are needed for 7 days. sion, the risk is less.
• Gallstones. COCs may accelerate the presentation of
Postponing a Bleed cholelithiasis in those who are predisposed. Their use
This is possible (see upcoming discussion). should be avoided in those with known gallbladder disease.
They can be used after cholecystectomy (UKMEC2)
but usually not after medical treatment for gallstones
TABLE How a Clinician Can Be Reasonably Certain a (UKMEC3).
16.2 Woman Is Not Pregnant (Anon., 2005; • Hypertension. COCs can induce hypertension, particularly
Clinical Effectiveness Unit, 2006a) in the early months of use (Poulter, 1996). About 1% of
• There should be no symptoms or signs of pregnancy
COC users become clinically hypertensive with modern
and any ONE of the following criteria should be met: formulations. Pill-induced hypertension should not be
• There has been no sex since the start of the last treated with antihypertensive drugs, but the pill should
normal menstrual period. be stopped and observation continued.
• She has been correctly and consistently using a • Cancer. Overall the balance of risks and benefits of the
reliable* method of contraception.
• She is within the first 7 days of her cycle.
COC on cancer is beneficial. There is an increased risk of
• She is within the first 7 days after an abortion or cancer of the cervix; this is mitigated by a large reduction
miscarriage. in risk of cancer of the ovary (RR 0.73) (Collaborative
• She is fully breastfeeding, amenorrhoeic, and less Group on Epidemiological Studies of Ovarian Cancer,
than 6 months postpartum. 2008) and the endometrium (Weiderpass et al., 1999)
• She is not breastfeeding and is less than 3 weeks
postpartum or has had no unprotected sex since
and by a reduction in the risk of cancer of the colon
delivery. (RR 0.82) (Fernandez et al., 2001). The COC probably
• A pregnancy test adds weight to the diagnosis, accelerates development of cancer of the cervix caused
but only if 3 weeks have elapsed since the date of by chronic infection with oncogenic human papilloma-
last sex. virus (HPV); with 5 years or more of use the RR is 1.90
*The author does not regard condoms, coitus interruptus, or fertility (International Collaboration of Epidemiological Studies
awareness as reliable enough to exclude the possibility of pregnancy. of Cervical Cancer, 2007). The literature on the COC
and breast cancer is conflicting but some good quality
studies show no increased risk (Clinical Effectiveness Unit, • However, it is known that diarrhoea has to be of dysenteric
2010b). Both UK cohort studies show no increased risk: proportions to reduce pill absorption.
RRs of 1.0 (Vessey & Painter, 2006) and 0.90 (Han-
naford et al., 2010), respectively. By the age of 40 to 44 Drugs for Infections (Clinical Effectiveness Unit,
however, COC use is associated with 30 extra cancers per 2005a, 2011)
100,000 women. • The Faculty of Sexual and Reproductive Healthcare
• Inflammatory bowel disease (IBD). IBD may be associated (FSRH) no longer recommends the need to use barrier
with VTE, hepatobiliary disease, and osteoporosis, all of methods of contraception for non–enzyme-inducing
which would need to be taken into consideration when antibiotics.
considering suitability for the COC (Clinical Effectiveness • Rifampicin, rifabutin, griseofulvin, and some antiretroviral
Unit, 2009b). Also the efficacy of the COC may be reduced drugs (notably ritonavir-boosted protease inhibitors)
in women with Crohn disease who have small bowel induce liver enzymes.
disease and malabsorption. • Oral antifungal agents have also been associated with
anecdotal reports of COC failure.
Side Effects • Extra precautions should be taken during the treatment
There are side effects but most wear off after the first few and for 7 days thereafter. If this runs into the PFI the
cycles, especially bloating, nausea, and breast tenderness. next packet should be started without a break. Rifampicin
Nausea may be reduced by taking the pill at night. and rifabutin are such powerful enzyme inducers that
extra precautions should be taken for 4 weeks even after
Noncontraceptive Benefits a 2-day course and elimination of PFIs during this time
• The following have been shown to be beneficial effects (Clinical Effectiveness Unit, 2006a).
of the COC:
• Lighter, shorter bleeding (less anaemia) Surgery
• Less dysmenorrhoea The COC should be stopped from 4 weeks before until 2
• Less premenstrual syndrome (PMS) symptoms weeks after any major surgery, varicose vein surgery or sclero-
• Less pelvic inflammatory disease (PID) therapy, or any operation likely to be followed by immobi-
• Fewer ectopic pregnancies lization (e.g., leg surgery) (Clinical Effectiveness Unit, 2009).
• Less benign breast disease It is otherwise not necessary to stop the COC.
• A bone sparing effect
• Fewer functional ovarian cysts Postponing or Avoiding Bleeds
• Less hospitalization for fibroids Monophasic Pills
• Less symptomatic endometriosis Patients on a fixed-dose combined pill can postpone the
• The COC tends to improve acne. There is little to choose next withdrawal bleed by starting the next packet immedi-
between COC formulations. COCs need to be taken for ately, omitting the PFI.
at least 6 months for their full effect on the skin to become
apparent. Evidence for the use of co-cyprindiol, if a COC Phased Preparations
fails to improve acne, is of poor quality (Arowojolu, 2009). • If on Synphase, another packet can be taken immediately
following the first.
Bleeding • If taking other phased preparations there would be an
• Withdrawal bleeds are usually lighter than periods. abrupt drop in progestogen levels if the above regimen
• BTB may occur during the first two or three cycles. were followed, leading to a risk of bleeding. Advise:
• BTB is not a reason for stopping the pill in • tablets from the last phase of a spare packet can be
midpacket. taken, to give 7 (TriNovum) or 10 (Logynon, Triadene)
or 14 (Binovum) days’ postponement; or
Missed Pills (Clinical Effectiveness Unit, 2006a) • a packet can be started of the next higher dose mono-
• The riskiest time for pills to be forgotten is on either side phasic pill, omitting the PFI. With Qlaira, the 17
of the pill-free week. highest dose tablets should be used.
• Forgetting in the middle of a packet is less likely to give • If postponement by a few days is needed (e.g., to avoid a
rise to breakthrough ovulation or pregnancy. bleed at weekends), the necessary number of pills from
• If more than 24 hours late, this is classed as missed pills a fresh pack should be taken and the rest of that pack
and the agreed rules are as in Fig. 16.1. thrown away. If on phased preparations, they should follow
the principles stated earlier.
Diarrhoea or Vomiting • Note: Every Day (ED) preparations. In all the above men-
• This requires extra precautions for the period of illness tioned advice, the seven inactive pills in ED preparations
and for 7 days afterwards, as discussed. should be discarded.
• If this would run into the PFI, the PFI should • Extended use of the COC has become widespread; running
be omitted. several or all packets together reduces bleeding days and
If ONE or TWO 30 – 35 µg If THREE or MORE 30 –35 µg EE

ethinylestradiol (EE) pills have been pills have been missed at any time
missed at any time OR
OR TWO or MORE 20 µg EE pill are
ONE 20 µg EE pill is missed missed
She should take the most recent She should take the most recent missed
missed pill as soon as she remembers. pill as soon as she remembers.
She should continue taking the She should continue taking the remaining
remaining pills daily at her usual time.* pills daily at her usual time.*
She does not require additional She should be advised to use condoms
contraceptive protection. or abstain from sex until she has taken
pills for 7 days in a row.
She does not require emergency
contraception. IN ADDITION
(because extending the pill-free
interval is risky)
If pills are missed in week 1 If pills are missed in week 3

(Days 1–7) (because the pill-free (Days 15–21) (to avoid extending
interval has been extended) the pill-free interval)
Emergency contraception She should finish the pills

should be considered if she had in her current pack and
unprotected sex in the pill-free start a new pack the next
interval or in week 1. day; thus omitting the pill-
free interval.
*Depending on when she remembers her missed pill she may take two pills on the same day
(one at the moment of remembering and the other at the regular time) or even at the same time.
• Fig. 16.1 Action to be taken if pills are missed. (Faculty of Family Planning and Reproductive Health
Care Clinical Effectiveness Unit. (2005). WHO selected practice recommendations for contraceptive use
update. “Missed pills”: New recommendations. Journal of Family Planning and Reproductive Health Care,
31, 153–155. Reproduced with permission.)
menstrual cycle–related symptoms (Archer, 2006). When • check BP (discontinue if BP increases to and remains
used continuously for 1 year, 18% of women achieve at 160/95 mm Hg). If the BP is satisfactory 2 years
amenorrhoea by 3 months of use and 88% by 10 months after commencement of the COC, BP checks can be
(Miller & Hughes, 2003). Extended use can be at the per- extended to annually in women without risk factors
sonal preference of the woman according to how often she or relevant diseases.
wishes to bleed or recommended on medical grounds (e.g.,
for low bone density, endometriosis, PMS, or withdrawal Changing the Pill Because of Side Effects
headaches). When changing a pill because of side effects, the choice lies
between changing oestrogen/progestogen dominance and
Follow-Up changing to a pill with a different progestogen (see upcom-
• The patient should be seen again at 3 months, or earlier ing discussion).
if side effects occur. Do not give repeat prescriptions
without someone seeing the patient and at least checking Breakthrough Bleeding
the blood pressure. • BTB occurs in up to 30% of users in the first few cycles
• Once established on the pill, see the patient 6-monthly and: but tends to settle by the third cycle. Unless prior warning
• assess whether there are any new risk factors, including of this is given, discontinuation rates will be high, espe-
migraine; cially in the young.
• check whether the patient is smoking; • If BTB occurs in the first 3 months, ask whether pills have
• ask about side effects; been missed; encourage the patient to persevere.
• If BTB continues beyond 3 months, exclude lesions of the should be applied as soon as remembered giving a new
cervix and problems with taking the pill (e.g., vomiting). day 1—additional nonhormonal methods of contracep-
Also encourage smokers to quit, as they are more likely to tion should be used for the first 7 days of the new cycle.
have BTB (Rosenberg, Waugh, & Stevens, 1996). Give a If intercourse has occurred during this extended patch-free
pill with a higher progestogen dose, change to a progesto- interval, a possibility of fertilization should be considered.
gen with better cycle control (e.g., gestodene), or change • If applications of a patch in the middle of the cycle is delayed
to a triphasic formulation of the same progestogen. If this (i.e., the patch is not changed on day 8 or day 15):
fails, consider increasing the oestrogen content as well. • for up to 48 hours, apply a new patch immediately;
• If BTB occurs when previous control had been good, exclude next patch change day remains the same and no addi-
lesions of the cervix, chlamydia infection, interacting drugs, tional precautions are required;
gastrointestinal disorder, and a change to a vegetarian diet. • for more than 48 hours, contraceptive protection may
• Absent withdrawal bleeds (WB): have been lost. Stop the current cycle and start a new
• Explain that this is not unsafe and does not signify 4-week cycle immediately by applying a new patch
overdosage. giving a new day 1. Additional precautions should be
• Missing one WB does not need any action. If two are used for the first 7 days of the new cycle.
missed, exclude pregnancy. • If the patch is not removed at the end of the cycle (day 22),
• If the patient is concerned despite reassurance, consider remove it as soon as possible and start the next cycle on
a switch to a triphasic pill. the usual change day, after day 28. No extra precautions
are required.
Oestrogen Withdrawal Headache During Pill-Free Interval
• Consider advising the patient to use three packets con- Combined Vaginal Ring (Clinical Effectiveness
secutively without a break, followed by a 7-day break Unit, 2009c)
(tricycle regimen).
• NuvaRing was launched in 2009. It releases 120 µg etono-
Combined Transdermal Patch gestrel and 15 µg ethinylestradiol/day from an ethylene
vinyl acetate copolymer ring with an outer diameter of
54 mm. The same contraindications and drug interactions
RECOMMENDATION
apply as for the COC.
Recommend the information leaflet from the Family Planning • Tampon use has no effect on the systemic absorption of
Association at www.fpa.org.uk. the hormones released from NuvaRing.
• A ring can be removed (e.g., for sex) for up to 3 hours.
• Prior to dispensing, NuvaRing is stored at 2° to 8°C.
Once dispensed, storage is at room temperature and the
• At present there is only one product in the UK, Evra. shelf life is 4 months.
This patch releases norelgestromin 150 µg/day and ethi- • Women use a ring for 3 weeks followed by a ring-free
nylestradiol 20 µg/day into the circulation. The patch is week during which time they have a withdrawal bleed.
applied weekly for 21 days and the fourth week is patch A new ring is needed for each 4-week cycle.
free. Suitable sites are the upper outer arm, upper torso • NuvaRing has high effectiveness no different from the com-
(excluding breast), buttock, or lower abdomen. bined pill, especially when adherence is good (Ahrendt et al.,
• Effectiveness is similar to the COC. The same contrain- 2006; Oddsson, Leifels-Fischer, de Melo, et al., 2005).
dications apply as for the COC. Also, the same drug • Cycle control is better than with the combined pill (Bjar-
interactions as for the COC must be presumed. BTB nadóttir, Tuppurainen, & Killick, 2002; Merki-Feld &
and spotting and breast tenderness are more common Hund, 2007; Oddson, Leifels-Fischer, Wiel-Masson, et al.,
than with the COC in the first two cycles. 2005; Roumen, op ten Berg, & Hoomans, 2006).
• If a patch is partly detached for less than 24 hours, it • The ring can cause leucorrhoea, vaginal discomfort, vagi-
should be reapplied to the same site or replaced with a nitis, and ring-related events comprising foreign-body
new patch immediately. No additional contraception is sensation, coital problems, and expulsion (Ahrendt et al.,
needed and the next patch should be applied on the usual 2006; Oddsson, Leifels-Fischer, de Melo, et al., 2005).
change day. • If the ring-free interval is extended beyond 7 days, the woman
• If a patch remains detached for more than 24 hours or if should insert a new ring as soon as she remembers. Extra
the user is not aware when the patch became detached precautions should be used for the next 7 days. If unpro-
she should stop the current contraceptive cycle and start tected sex took place during the ring-free interval, the
a new cycle by applying a new patch, giving a new day use of emergency contraception can be considered or a
1—additional precautions must be used concurrently for pregnancy test done after 3 weeks.
the first 7 days of the new cycle. • If the ring was temporarily outside the vagina, it should
• If application of a new patch at the start of a new cycle be rinsed in lukewarm water and reinserted. If the ring
is delayed, contraceptive protection is lost. A new patch was outside the vagina for less than 3 hours, no further
action is necessary. If the ring was outside the vagina for

more than 3 hours, extra precautions should be used for Taking the Progestogen-Only Pill: Procedure
the next 7 days. If the loss in continuity of use of the and Advice
ring for more than 3 hours occurs in the third week of
INFORMATION FOR PATIENTS
use, a new ring should be inserted without the ring-free
interval. Recommend the information leaflet from the Family Planning
• If the ring is left in place for more than 3 weeks, this is Association at www.fpa.org.uk.
acceptable up to a total of 4 weeks, even if there is then
a ring-free week. If the ring has been left in the vagina
for longer than 4 weeks, pregnancy should be excluded Starting the POP:
before another ring is inserted. • Days 1 to 5 of the cycle: If started on or before day 5 then
no other precautions need be taken.
Progestogen-Only Pill (Clinical Effectiveness • After day 5: Extra precautions should be taken for the
Unit, 2008a) first 2 days.
• Switch from COC: If changing from a COC, go straight
There are four traditional progestogen-only pills (POPs) on to the POP without a 7-day break. Extra precautions
available. A POP containing desogestrel (Cerazette) was are not necessary although it may be necessary to take
launched in 2002 (Clinical Effectiveness Unit, 2003b). This the pill at a different time of day.
is much more likely than traditional POPs to inhibit ovula- • Postpartum: Start at 3 weeks postpartum. Extra precau-
tion, but comparative data on efficacy are lacking. Available tions are only needed for the next 2 days if starting later
data show that effectiveness is similar to the COC (Trus- than that.
sell, 2007) but traditional POPs are probably less effective Note: Time of day. Traditional POPs must be taken regu-
than COCs. larly at the same time each day (ideally at least 4 hours before
the most frequent time of intercourse to ensure maximal
Patients for Whom the Progestogen-Only Pill Is mucous-thickening effect).
Particularly Indicated
• Older women: those over 45 years old without risk factors, Advice
and those over 35 years old who smoke • Efficacy. Efficacy is lowest in women with no alteration
• Those with medical contraindications to oestrogen, including in cycle activity and is greatest in those with complete
a personal history of VTE suppression of cycles and consequent amenorrhoea.
• Those with risk factors for arterial disease, including hyper- Roughly 40% of women continue to ovulate, in 40%
tension, diabetes mellitus, migraine with aura, and smokers there is variable interference with the follicular and luteal
aged 35 years and older phase, and in 20% ovulation is inhibited completely.
• Lactating mothers: progestogen in the breast milk has • Bleeding pattern. From efficacy, it follows that 40% have
no adverse effect on the baby (Clinical Effectiveness similar cycles to their normal pattern, 40% have shorter
Unit, 2009b) cycles (which may gradually lengthen toward normal over
• Those who choose it, especially those aged over 25 or 30 time) with episodes of spotting or BTB, and 20% have
years, and who accept that it may be less reliable than long cycles or amenorrhoea.
the COC • Safety. There is no evidence of increased cardiovascular
risk (Heinemann, Assmann, DoMinh, & Garbe, 1999).
Unacceptable Health Risk (UKMEC4) However, there is limited evidence of an increased risk of
(Clinical Effectiveness Unit, 2016) breast cancer for use in the previous 5 years (RR 1.17), but
• Breast cancer: current no increase 10 or more years after stopping (Collaborative
Group on Hormonal Factors in Breast Cancer, 1996).
Risks Usually Outweigh Benefits (UKMEC3) • Antibiotics. The POP is not affected by antibiotics, except
(Clinical Effectiveness Unit, 2016) rifampicin, rifabutin, and griseofulvin (see Enzyme-
• Ischaemic heart disease: current or past history, if occurred inducing drugs [last bullet of this list]).
when on POP • Missed pills. If one or more pills are missed or delayed
• Stroke: history of CVA, if occurred when on POP for more than 3 hours then take one pill as soon as
• Breast cancer: past and no evidence of current disease for remembered; take the next pill at the usual time (this
5 years may mean taking two pills in 1 day); continue taking
• Cirrhosis: severe (decompensated) pills, one daily; extra precautions need to be taken for 2
• Liver tumours: benign (hepatocellular adenoma) and days. For Cerazette the instructions are the same, except
malignant (hepatoma) the window is 12 hours instead of 3 hours. Emergency
• SLE: with positive or unknown antiphospholipid antibodies contraception can be considered if unprotected sex occurs
• Enzyme-inducing drugs. during this 2-day period.
• Diarrhoea or vomiting. If vomiting or diarrhoea occurs, Indications

continue to take the pill regularly but take extra precau- The indications are similar to those for the POP. Patients
tions during the attack and for the next 2 days. for whom injectable progestogens are especially indicated
• Obesity. There is no evidence that the efficacy of POPs is are women:
reduced in women weighing over 70 kg; the licensed use • likely to forget (or to worry about forgetting) to take daily
of one pill per day is recommended. pills;
• Enzyme-inducing drugs. Those on enzyme-inducing drugs • for whom higher dose, long-term progestogens are beneficial
are best advised not to rely on POPs (Clinical Effective- (e.g., those with fibroids or endometriosis);
ness Unit, 2005a, 2008a). • with sickle cell disease: DMPA improves the blood picture
and reduces the number of crises (Westhoff, 2003).
Side Effects
Non-Menstrual Unacceptable Health Risk (UKMEC4) (Clinical
These are the usual progestogenic ones such as headaches, Effectiveness Unit, 2016)
tender breasts, acne, depression, weight gain, and loss of • Breast cancer: current
sexual drive. But because the dose of progestogen is so
low, these side effects are not that common and often Risks Outweigh Benefits (WHO3) (Clinical
not severe. Effectiveness Unit, 2016)
• Vascular disease: ischaemic heart disease, peripheral vascular
Irregular Bleeding disease, hypertensive retinopathy, or TIAs (Injectables
• Examine to exclude a pathologic cause. have hypooestrogenic effects and reduce HDL levels.)
• Do not change to another POP. There is no suggestion • Stroke: history of CVA
that with traditional POPs changing brand will improve • Unexplained vaginal bleeding: before evaluation
menstrual or nonmenstrual side effects. • Breast cancer: past and no evidence of current disease for
• Suggest a change to another method. 5 years
• Diabetes: with nephropathy, retinopathy, neuropathy, other
Abdominal Pain of Gynaecologic Origin vascular disease
• Consider ectopic pregnancy. If it can be excluded, then • Cirrhosis: severe (decompensated)
refer for ultrasound. It may be due to a functional ovarian • Liver tumours: benign (hepatocellular adenoma) or malig-
cyst. Such a cyst usually resolves without treatment. nant (hepatoma)
• SLE: with positive or unknown antiphospholipid
Amenorrhoea When Cycles Have Been Present antibodies
• Exclude pregnancy before assuming that it is due to the
POP. Before Starting
• Encourage the woman to persevere with the POP. She is Discuss with the patient the following factors.
in the group least likely to become pregnant with it.
Advantages
Injectable Progestogens (Clinical Effectiveness • Injectables are effective and independent of coitus, with
Unit, 2008b) no oestrogenic side effects.
• They are invisible to others, which may be important to
some women (e.g., those in controlling or abusive
relationships).
Recommend the information leaflet from the Family Planning • They decrease the risk of endometrial cancer, pelvic inflam-
Association at www.fpa.org.uk. matory disease, ectopic pregnancy, fibroids, and iron
deficiency anaemia (Westhoff, 2003).
• They may enhance lactation and relieve premenstrual and
Preparations menstrual symptoms.
• Depot Medroxypogesterone acetate (DMPA) as Depo- • Seizure control has been reported to be improved in some
Provera (150 mg in 1 mL) or Sayana Press (104 mg in epileptics.
0.65 mL). Mix the pre-loaded syringe thoroughly before
administration. Effectiveness
• Norethisterone enantate (NE), or Noristerat: 200 mg in • Injectables are a very effective means of contraception:
1 mL. Warm the vial close to body temperature before perfect use failure rates are below 0.7% for DMPA in
administration. the first year and below 1.0% for NE. Typical use failure
Note: NE is not licensed for long-term use. When used rates are 3%.
for longer than 16 weeks, this use outside the licence must • Blood levels of DMPA are not affected by drugs
be discussed with the patient. including enzyme inducers; there is no justification
for increasing the dose or reducing injection intervals reduction in BMD occurs, it takes place over the first 2
for this reason (Clinical Effectiveness Unit, 2005a). or 3 years and then tends to level off. The reduction is
With NE, care is needed with concurrent antiretroviral generally less than 1 SD (i.e., not into the osteopenic
therapy, certain antiepileptics, and rifampicin/rifabutin range) (Curtis & Martins, 2006). After discontinuation
(UKMEC2). of DMPA, BMD consistently returns toward, or to, base-
line values in women of all ages (Kaunitz, Arias, &
Side Effects McClung, 2008). Patterns of BMD recovery are similar
• Erratic bleeding. This will occur in most women initially. to those seen after cessation of lactation. Available evidence
Prolonged episodes of bleeding may occur but are rarely does not justify the requirement of a limit to the duration
heavy and decrease over time (World Health Organisation of DMPA use, even in adolescents. Care is needed in
[WHO], 1987). high-risk groups: smokers, BMI less than 19, thyroid
• Examine (especially the cervix). disease, long-term antiepileptic therapy, and those with
• Give either: a family history of osteoporosis. Avoid in those on oral
a. the next injection early (but not earlier than 4 weeks steroids.
after the last one); or • Cancer. There is limited evidence of an increased risk of
b. a short course of oestrogen (e.g., the COC, if no breast cancer for use in the previous 5 years (RR 1.17),
contraindication); or but no increase 10 or more years after stopping (Col-
c. a course of mefenamic acid. laborative Group on Hormonal Factors in Breast Cancer,
• Refer if the above do not control the bleeding. 1996). DMPA has a strong protective effect against endo-
• Alternatively, the patient may prefer to discontinue metrial cancer (RR 0.21) (WHO, 1991).
the method.
• Amenorrhoea. This is likely, more so the longer the method Initiating Treatment and Follow-Up
is used. The amenorrhoea rate with DMPA is around Schedule for the Injections
70% at 12 months (Canto De Cetina, Canto, & Ordoñez • Give the first injection within 5 days of the onset of
Luna, 2001). With NE, episodes of bleeding are the menstruation, within 3 weeks of delivery, or 1 to 5 days
more usual pattern and the amenorrhoea rate at 12 months after miscarriage or termination. The patient should use
is 25%. Explain that these methods make the lining of additional precautions for 7 days after the first injection
the womb so thin that there is no monthly shedding. unless it was given as mentioned earlier.
Many women find the amenorrhoea a very acceptable • Use the upper outer quadrant of the buttock or the lateral
side effect. thigh. The deltoid is used when the patient is obese,
• Exclude pregnancy (beware of weight gain at successive as it is important the drug reaches muscle. Warn the
visits with nausea or breast symptoms). patient not to massage the site as this may speed up drug
• Discuss other concerns. There is concern that injectables release.
can lower ovarian oestradiol production; see Reduced • Give subsequent injections: Depo-Provera 150 mg
Bone Mineral Density (under Risks). There is a lack of Sayana Press 13-weekly; Noristerat 200 mg 8-weekly.
consensus on any action to be taken with continuous • If the patient returns late for the next injection of DMPA
amenorrhoea for over 5 years. Some authorities have or NE, proceed as in Table 16.3.
recommended checking serum oestradiol but this is
not a useful proxy indicator for bone mineral density Etonogestrel-Releasing Implant: Nexplanon
(BMD). Bone densitometry is the test of choice but (Clinical Effectiveness Unit, 2014)
is expensive and not always freely available.
• Weight gain. This is possible, through appetite stimulation,
although some studies show no effect on weight (Westhoff,
2003). The weight gain is not generally the result of fluid Recommend the information leaflet from the Family Planning
retention. Modification of diet and behaviour will tend
to counteract the weight gain. Any weight gain is less
likely with NE.
• Return of fertility. This may be delayed, with a mean time • Nexplanon releases a mean etonogestrel dose of 60–70 µg/
to ovulation of 18 weeks from the expiry of the last dose day when first inserted, decreasing to 25–30 µ/day by
of DMPA (maximum 49 weeks). Return of fertility is the end of year 3.
much faster with NE: mean of 4 weeks and maximum • Subdermal implants should only be inserted and removed
of 26 weeks (Fotherby & Howard, 1986). if specific practical training has been obtained and a
minimum number of implants are inserted and removed
Risks to keep up the necessary skills.
• Reduced BMD. DMPA reduces BMD in many women • Nexplanon has the advantage that it lasts for 3 years but
who use it. However, so far no studies have shown an is reversible should the need arise. It has a high effective-
increased risk of osteoporosis or fractures. When a ness, 0.05% in the first year, higher even than vasectomy.
TABLE
16.3 Management of Late Injections of Injectable Progestogens (Clinical Effectiveness Unit, 2008)
Can the Is Emergency Is Additional Should a

Timing of Has Unprotected Injection Contraception Contraception or Pregnancy Test
Injection Sex Occurred? Be Given? Indicated?a Abstinence Advised? Be Performed?
Up to 14 weeks Not applicable, as Yes No No No
since last DMPA long as next
injection or up to injection is given
10 weeks since 14 weeks since
last NET-EN last DMPA injection
injection or 10 weeks since
last NET-EN
injection or before
When an injection is No (abstained or Yes No Yes, for the next 7 days No, if abstained
overdue: 14 used barrier Yes, if used
weeks + 1 day or methods) barrier methods,
more since last but at least
DMPA injection; 21 days later
or 10 weeks + 1
day or more Yes, but only in the Yes Yes, should offer Yes, for the next 7 days Yes, at least
since last NET-EN last 3 daysb Levonelle 1500 21 days later
injection or a copper IUD
Yes, but only in the Yes Yes, should offer a No, if opts for copper Yes, at least
last 4–5 daysb copper IUD IUD 21 days later
Yes, more than 5 No No Yes, for 21 days until a Yes, at the initial
days agob pregnancy test is presentation
confirmed negative and at least
and for a further 7 21 days later
days after giving
injection
a
If EC is declined, decisions about ongoing use of DMPA/NET-EN should be tailored to the individual woman. Alternative methods if required should then be
considered.
b
Not applicable if unprotected sex occurred within 14 weeks of last DMPA injection or 10 weeks of last NET-EN injection.
DMPA, Depot medroxyprogesterone acetate; EC, emergency contraception; IUD, intrauterine device; NET-EN, norethisterone enanthate.
Reproduced with permission of the Faculty of Sexual and Reproductive Healthcare.
Pregnancies in women using a contraceptive implant are Risks Outweigh Benefits (UKMEC3)
generally due to noninsertion and drug interactions (Clinical Effectiveness Unit, 2016)
(Harrison-Woolrych & Hill, 2005). • Ischaemic heart disease: current or past history
• The return of fertility after removal of Nexplanon is imme- • Stroke: history of CVA, if occurred when using implant
diate; ovulation occurs mostly within 3 weeks of removal. • Unexplained vaginal bleeding: before evaluation
Progestogen only implants have a beneficial effect on • Breast cancer: past history and no evidence of current
dysmenorrhoea. Nexplanon is more cost effective than disease for 5 years
the COC. • Cirrhosis: severe (decompensated)
• Disadvantages are the discomfort of insertion and removal. • Liver tumours benign (hepatocellular adenoma) or malig-
• Practicalities. If it is implanted other than on days 1 to nant (hepatoma)
5 of the menstrual cycle, extra precautions are needed • SLE: with positive or unknown antiphospholipid antibodies
for the first 7 days, if there is no previous method provid-
ing cover. For postpartum women, including those who Monitoring
are breastfeeding, insert at up to 3 weeks postpartum. • There is no reason to check weight and blood pressure.
Following abortion or miscarriage, insert at up to 5 days. • There is no reason to do any routine follow-up.
The patient should use additional precautions for 7 days
after insertion unless timed as above. Problems With the Implant
Menstrual
Unacceptable Health Risk (UKMEC4) • Bleeding patterns are variable even in an individual over
(Clinical Effectiveness Unit, 2016) time. Frequent irregular bleeding, spotting, and prolonged
• Breast cancer: current bleeding are all possible; heavy bleeding is rare.
• Amenorrhoea occurs in 21% of users. • Gestational trophoblastic disease—with persistently ele-

• Those who discontinue tend to be from the groups with vated beta-human chorionic gonadotropin (β-hCG) levels
less favourable patterns: prolonged bleeding (17%) and or malignant disease
frequent bleeding (6%). • Cervical or endometrial cancer: current
• Problematic bleeding can be treated as per injectables (see page • STI (current purulent cervicitis, chlamydial infection, or
255). Some clinicians add a progestogen such as Cerazette gonorrhoea) or PID: current
or norethisterone; there are no data to support this. • Pelvic TB
Non-Menstrual Risks Usually Outweigh Benefits (UKMEC3)

Non-Menstrual problems include acne, mastalgia, headache, (Clinical Effectiveness Unit, 2016)
weight gain, abdominal pain, emotional lability, and depression. • Postpartum insertion between 48 hours and 4 weeks
postpartum—in women who are breastfeeding, not breast-
feeding, or post caesarean section.
Nonhormonal Methods of Contraception • Distortion of the uterine cavity (e.g., congenital malfor-
Intrauterine Devices (Clinical Effectiveness mation, submucous fibroids)
• Ovarian cancer
Unit, 2015)
Adverse Effects
INFORMATION FOR PATIENTS Heavier Periods, Intermenstrual Bleeding,
Recommend the information leaflet from the Family Planning Dysmenorrhoea
Association at www.fpa.org.uk. • Warn the patient that these are common in the first few
cycles after insertion.
• Examine for infection or malposition of the device.
• Typical use failure rates for copper intrauterine devices • Prescribe nonsteroidal antiinflammatory drugs (NSAIDs),
(IUDs) are 0.8% in the first year (Trussell, 2007). which may reduce pain and bleeding.
• IUDs should only be fitted if specific practical training • Remove the IUD if it appears to be associated with a
has been obtained and a minimum number of devices change in bleeding pattern. If the pattern does not return
are fitted to keep up the necessary skills. to normal, refer for gynaecologic assessment.
• All IUDs with frames have copper on the stem and/or
on the arms. GyneFix is a frameless device with six copper Pelvic Infection
cylinders on a thread. Modern copper-containing IUDs • Symptoms of infection require examination and endo-
are clinically effective and safe for at least 5 years. The cervical swabs for STIs.
TT380 Slimline and TCu380A QuickLoad are effective • Treat with antibiotics.
for 10 years. • If the symptoms settle, the IUD can be left in place.
• The risk of perforation associated with insertion of IUDs • Actinomyces-like organisms (ALOs) may be found on a
is 1 in 1000. cervical smear:
• IUDs do not offer any protection against sexually transmit- • If the patient is asymptomatic, leave the IUD in place.
ted infections (STIs) but previous studies that purported • If the patient has symptoms (i.e., pain or discharge)
to show an increased risk of pelvic inflammatory disease remove the IUD and investigate the patient for pelvic
(PID) have now been shown to be flawed (Grimes, 2000). pain/STIs, treat with antibiotics as appropriate, and
There is no evidence that prophylactic use of antibiot- consider referral to genitourinary medicine (GUM)
ics for IUD insertion is of significant benefit in healthy or gynaecology.
women (Grimes, Lopez, Schulz, & Stanwood, 2004).
• Currently available high-load IUDs protect against ectopic Pregnancy
pregnancy when compared to using no contraception. • If symptoms suggest an ectopic pregnancy, admit to hos-
• Cumulative expulsion rates at 5 years for copper IUDs pital; 6% of pregnancies that occur with an IUD in place
are around 5%. Expulsion may occur at any time after are ectopic.
insertion, but is most likely in the early cycles, especially • If a scan indicates that the pregnancy is intrauterine,
during menstruation. Patients who are older and of higher gently remove the IUD if the threads are visible and the
parity are less likely to expel their device. pregnancy is less than 12 weeks. This will halve the mis-
carriage rate. If the pregnancy is beyond 12 weeks or no
Unacceptable Health Risk (UKMEC4) (Clinical threads are seen, refer early for antenatal assessment.
Effectiveness Unit, 2016
• Pregnancy Lost Threads
• Puerperal sepsis • Teach the patient to feel for the threads after each period,
• Immediately after septic abortion or on the first of the month if there is amenorrhoea with
• Insertion before evaluation of unexplained vaginal bleeding the IUD.
• If the threads are not palpable, warn the patient tempo- • The steroid reservoir in the Mirena IUS contains 52 mg
rarily to use other precautions, and arrange for ultrasound levonorgestrel. It is licensed for contraception and
scan (USS). menorrhagia (for 5 years) and for endometrial protec-
• If USS confirms that the device is in situ, leave in place tion (4 years). It is more cost effective than the COC
until it is due to be changed. Repeat vaginal examination (National Collaborating Centre for Women’s and Chil-
yearly, and only repeat USS if there is reason to suspect dren’s Health, 2005).
expulsion. • The steroid reservoir in the Jaydess IUS contains 13.5 mg
levonorgestrel. It is licensed for contraception (for 3 years)
Timing of Insertion but not for menorrhagia although it does reduce menstrual
• Insertion can be at any time during the menstrual cycle bleeding. It is smaller in size than Mirena.
if it is reasonably certain a woman is not pregnant (see • Protection after insertion. The IUS takes 7 days to provide
Table 16.2). effective contraceptive protection. Unless the IUS is
• Do not insert before 4 weeks postpartum. inserted within the first 7 days of the cycle, extra precau-
• An IUD can be safely fitted immediately after a first trimester tions are advised for the next 7 days. If there has been a
abortion (spontaneous or therapeutic), although this carries risk of conception it would be inappropriate to insert an
an increased risk of expulsion (Grimes et al., 2004). IUS that cycle.
• The IUS in older women. Women who have the Mirena
Removal or Refit inserted at the age of 45 or over for contraception can
• Women aged 40 and over: An IUD fitted after the age of retain the device until the menopause is confirmed or
40 can be left as the only means of contraception until the until contraception is no longer required.
menopause. When there has been 6 months of amenor-
rhoea it should be removed; later removal may be difficult Advantages of the IUS Over IUDs
because of cervical stenosis. If a woman presents more than • They reduce blood loss instead of increasing it and
6 months after the menopause with a device in situ, attempt they are a form of therapy for menorrhagia (although
to remove it. If difficulty is encountered try again after a Jaydess is not licensed for this), even in the presence of
short course of treatment with topical oestrogen. fibroids.
• They can reduce dysmenorrhoea.
Timing of Removal • They are more reliable as a contraceptive, with a failure
• If pregnancy is desired, the IUD may be removed at any time. rate of 0.2% in the first year compared to 0.8% for copper
• If pregnancy is not desired, remove the IUD when the IUDs. The IUS approximate in effectiveness to female
patient is established on a hormonal method or when sterilization.
barrier methods have been used carefully since the last • It has a very low rate of ectopic pregnancy.
period.
• Emergency contraception may be necessary if intercourse Disadvantages of the IUS
has occurred within the last 5 days and removal of the • The initial cost is much more than IUDs.
IUD is urgent. • It is slightly more difficult to insert than a copper IUD
because of a wider insertion diameter (although this is
Prophylactic Antibiotics for Cardiac Disease less of an issue with Jaydess).
For women with previous endocarditis or with a pros- • The patient may have irregular slight bleeding in the first
thetic heart valve it is no longer recommended to give 3 months of use.
antibiotic prophylaxis during IUD insertion or removal • Progestogenic side effects (headache, breast tenderness,
(NICE, 2008). nausea, mood changes, acne) are possible, especially at
the start, as there is some systemic absorption; the rate
Follow-Up of occurrence is no different from IUDs after 5 years.
• Review after 6 weeks. Thereafter follow-up is not necessary.
• Ask about menstrual blood loss, pelvic pain, vaginal dis- Patients for Whom the IUS Is Particularly Indicated
charge, and discomfort to the partner. • As an alternative to sterilization when the family is
• Do a pelvic examination and check the presence of the complete
threads. • Those with menorrhagia
• Those in whom the COC is contraindicated
Intrauterine System (Clinical Effectiveness • Those with learning disabilities or physical disabilities
Unit, 2015) needing long-term contraception
Unacceptable Health Risk (UKMEC4) (Clinical
Recommend the information leaflet from the Family Planning Effectiveness Unit, 2016)
In addition to the conditions listed for the IUD:
• Breast cancer: current
Risks Usually Outweigh Benefits (UKMEC3) (Clinical • They are not as effective a protector as condoms against
Effectiveness Unit, 2016) STIs.
In addition to conditions listed for the IUD: • They have a failure rate of 6% to 16% in the
• Ischaemic heart disease: current or past history, if developed first year.
while IUS in situ • They come in sizes rising in steps of 5 mm and in the form
• Cirrhosis: severe (decompensated) of coiled spring, arcing spring, or flat spring. Coil spring
• Liver tumours: benign (hepatocellular adenoma) and and arcing diaphragms are also available in silicone. Correct
malignant (hepatoma) size and type are determined on vaginal examination.
• SLE: with positive or unknown antiphospholipid antibodies • Use:
• The diaphragm should be inserted before sex
Barrier Methods (Clinical Effectiveness Unit, and left in place for at least 6 hours after the last act
2007a, 2007b) of sex.
• A strip of spermicide about 5 cm long should be placed
on the upper side of the diaphragm before insertion.
If sex takes place more than 3 hours later, more sper-
Recommend the information leaflet from the Family Planning micide should be inserted, either as a pessary or as
Association at www.fpa.org.uk. cream with an applicator.
• The diaphragm must be washed in warm soapy water,
dried after use, and stored in a cool place in its con-
• These have the advantage of protecting against STIs and tainer to maintain its shape.
HPV, as well as pregnancy. • Check the fit and comfort after 1 week and discuss again
• However, efficacy is utterly dependent on consistent use the routine for its use.
before/during sex; there is considerable potential for user • See after 3 months and then annually, but more frequently
failure, especially among young people. if there are difficulties or if there is a weight change of
• Condoms and diaphragms made of latex rubber can be more than 3 kg.
damaged by oil-based products such as: • Prescribe a new diaphragm annually.
• baby oil/bath oil/body oil/Vaseline;
• suntan oil/massage oil; Cervical Caps
• cream/ice cream/salad cream; • FemCap is made of silicone. Failure rates are 10% to
• lipstick/hair conditioner; 18% in the first year. Before starting, sizing is needed by
• many antithrush preparations; a doctor or nurse. FemCap comes in three sizes and is
• progesterone pessaries. reusable.
• Diaphragms and caps should not be used if the woman
Male Condoms has a history of toxic shock syndrome (UKMEC3).
• Male condoms give a pregnancy rate of 2% to 15% in • Condoms, diaphragms, and caps should not be used in
the first year. Non-latex condoms are now available in people with latex allergy (UKMEC3).
addition to latex rubber products; they cannot be damaged
by oil-based products. Fertility Awareness
• Ensure the couple knows to use condoms bearing the CE
mark and preferably also the British Standards Institution • Fertility awareness or natural family planning (NFP) relies
Kitemark. on avoidance of intercourse around the time of ovulation.
• Advise the couple about use of emergency contraception • Failure rates can be as low as 2.6% in the first year when
in the event of a mishap. multiple indicators are used to define the beginning and
the end of the fertile period (European Natural Family
Female Condom Planning Study Groups, 1993), but this high efficacy is
• Femidom is a loose-fitting polyurethane sheath with two only achieved by highly motivated and well-trained couples.
flexible rings, which is inserted into the vagina. • Expert training is essential; advise the patient to contact
• It is prelubricated with dimeticone, an odourless, non- Fertility UK (see box) for information about local
spermicidal lubricant. classes. Computerized thermometers and saliva testing
• It lines the vagina and covers some of the vulva. It is kits are on sale but they are unevaluated and cannot be
disposable and comes in one size. Its failure rate is 5% recommended.
to 21% in the first year.
PATIENT INFORMATION
Diaphragms
• Diaphragms are more effective than the condom; they Fertility UK, Bury Knowle Health Centre, 207 London Road,
Headington, Oxford, OX3 9JA, www.fertilityuk.org.
have the advantage that they do not need to be inserted
and removed at the time of intercourse.
• Recognized indicators are: Contraindications

• waking (basal) body temperature changes;
• changes in cervical secretions; • Established pregnancy.
• cervical changes; • IUD. Contraindications to the insertion of an IUD apply
• calculation based on cycle length; except that, in an emergency, an IUD can be inserted
• minor changes (e.g., mittelschmerz [midcycle pain]) with antibiotic cover in a patient at risk of PID. Con-
• Combining indicators is best; the combination of tem- traindications to the long-term use of an IUD do not
perature and cervical secretions (symptothermal) is com- apply because the IUD can be removed after the next
monly used. period.
• Persona is a fertility monitor that measures estrone-3- • Ulipristal should not be used in those needing oral steroids
glucuronide and luteinizing hormone (LH) in the urine such as asthma or in those on drugs which increase gastric
and predicts fertile days for an individual by storing infor- pH (e.g., antacids, H2-receptor antagonists, or proton
mation on the woman’s biochemistry and cycle length. pump inhibitors).
It requires eight urine tests per cycle. A red light indi-
cates fertile days and a green light indicates non-fertile Special Situations
days. A yellow light indicates that a urine test is required.
It must be realized that when this device is used by the • Enzyme-inducing drugs. These may be prescribed or bought
non-NFP fraternity without combining indicators, failure OTC. Patients taking enzyme-inducing drugs should take
rates will be higher. Nevertheless, a failure rate of 6.2% two Levonelle 1500 pills instead of one. Ulipristal should
in the first year is predicted in such a population (Bonnar not be used in those taking enzyme-inducers.
et al., 1999). • Previous ectopic pregnancy. Patients should understand
that neither method will protect against a further ectopic
Emergency Contraception (Clinical pregnancy.
• The woman who wants the most reliable method or in
Effectiveness Unit, 2017) whom there has been multiple exposure; use an IUD.
Hormonal Preparations • Use of hormonal contraception is possible immediately
following administration of Levonelle, with use of condoms
• The progestogen-only method is available as levonorgestrel or abstinence for 7 days (2 days for POP). When ulip-
1500 µg: Levonelle 1500 on prescription and as Levonelle ristal is used, there is a possibility that it may reduce the
One Step as a pharmacy item. efficacy of hormonal methods for up to a week, so advise
• Ulipristal acetate 30 mg (ellaOne), a progesterone receptor condoms or abstinence for 2 weeks after administration.
modulator is on prescription only.
Management
Intrauterine Device Insertion
• The treatment of choice is usually Levonelle, even in
• Insertion of a copper-IUD can be considered if: low-risk situations, in view of patient anxiety and the
• the patient presents within 5 days of unprotected inter- safety of the method.
course; or • Ulipristal can be offered for those presenting between 72
• the patient presents within 5 days of the calculated and 120 hours after unprotected intercourse. But patients
time of ovulation if intercourse took place more than should be informed about the copper IUD method and
5 days before presentation. its greater effectiveness.
• Patients who have failed to take oral contraception • Establish from the consultation:
correctly: • date of LMP;
• COC. Either method can be considered for a patient • whether the LMP was normal;
who misses three or more pills (see Fig. 16.1). • the normal menstrual cycle length and probable date
• POP. Either method can be considered for a patient of ovulation;
who has unprotected sex during the 48-hour window • all the occasions of unprotected sexual intercourse
after regular pill taking has been re-established after (UPSI) in the last cycle;
missed pills. • number of hours since first episode of UPSI;
• the method of contraception normally used.
Regimen for Hormonal Emergency • Warn the woman that the maximum risk of pregnancy
Contraception after midcycle UPSI is about 9% (Wilcox et al., 2001),
but depends on the inherent fertility of the couple.
• One tablet of Levonelle 1500 taken within 72 hours of • Explain to the patient that:
(the first episode of ) unprotected intercourse. • the method is not guaranteed. The average risk of
• One tablet of ulipristal taken within 120 hours of (the pregnancy from a single episode of coitus is 3%
first episode of ) unprotected intercourse. (Wilcox et al., 2001). The progestogen-only method
and ulipristal reduce this risk by around 85%. The IUD • Young people continue to have concerns about the possibil-
is more effective, reducing the risk by 99% (Liying & ity of breaches of confidentiality. Improving young people’s
Bilian, 2001); trust in the confidentiality of their practice should help
• if the hormonal method is given, barrier methods must remove one of the main obstacles that deter some teenagers
be used until the next period, which may be a little from seeking early sexual health advice (Donovan et al.,
earlier or later than expected. The COC may then be 2000). Every practice should develop its own confidentiality
started; policy.
• she should reattend if the next period does not come, • Legal aspects. Inform the patient (and, if she gives her consent,
or she has any other worries; her parents) of the legal situation; it is an offence for a person
• if vomiting occurs within 2 hours of taking the dose to have sexual contact with a girl under the age of 16, even
she should reattend urgently. if the girl consents (Sexual Offences Act, 2003).
• Smoking. Discuss the importance of not smoking, espe-
Follow-Up cially if the COC is to be used.
• Discuss the risks of contracting STIs, especially chlamydia,
• If a normal period has not occurred, exclude pregnancy and how condoms protect against transmission.
and bear in mind the possibility of ectopic pregnancy. • Raise the advantages, both psychological and physical, of
• Discuss the long-term need for contraception. Use the not having sex at a young age.
opportunity to counsel the young and inexperienced.
Consider whether a supply of Levonelle 1500 in advance Combined Oral Contraceptive
might be appropriate for a woman who does not wish • Warn the patient that BTB can occur in the first few
planned contraception but who anticipates the need for packets, is not harmful, and to come in and discuss the
occasional emergency contraception after unplanned problem rather than stop the pill.
sex. In a US study it increased the use of emergency • Stress the value of using the condom as well to reduce
contraception without reducing the use of routine the risk of STIs, including HIV (dual protection). Young
contraception (Jackson, Bimla Schwarz, Freedman, & people should be shown how to use condoms.
Darney, 2003).
Contraception and Older Women
Contraception in Special Cases (Clinical Effectiveness Unit, 2010b)
Contraception and Young People • Women over 40 are more likely to have irregular cycles,
(Clinical Effectiveness Unit, 2010a) but ovulation still occurs in around 35% of these cycles
and there is still need for contraception.
INFORMATION AND ADVICE
• Current advice is for contraception to be continued in
women aged 40 to 50 until there has been 2 years of
Brook: Free confidential counselling service for young people, amenorrhoea, and in women over 50 until there has been
at www.brook.org.uk.
Sexwise: For under 18-year-olds with problems with sex,
1 year of amenorrhoea.
relationships, and contraception, at https://sexwise.fpa.org.uk.
Women on the Combined Oral Contraceptive
• Age. Healthy non-smokers without risk factors may con-
tinue until the age of 50.
• In England and Wales, provision of contraceptive advice • Advise women who have stopped the COC at 50 to use
and treatment by health professionals to those aged under the POP, barrier methods, or spermicide until they have
16 is governed by a health circular (Anon., 2004a). Advice had 1 year of amenorrhoea. If menstruating on the POP,
and treatment may be given, according to the Fraser guide- follow the advice given next.
lines, without parental knowledge when the young person:
• has sufficient maturity to understand the moral, social, Women on the Progestogen-Only Pill (Clinical
and emotional implications of the treatment; Effectiveness Unit, 2010b)
• cannot be persuaded to inform the parents or allow • When amenorrhoea occurs in those who have been having
them to be informed; periods, stop the POP but use other precautions for 1
• is very likely to begin, or to continue, sexual intercourse year if aged over 50, for 2 years if aged 50 or less.
with or without contraception; • Alternatively, check the follicle-stimulating hormone (FSH).
• would be likely to suffer in terms of physical or • Those women who have always had amenorrhoea on the
mental health if no contraceptive advice or treatment POP can have their FSH checked after age 50.
were given;
• has his or her best interests served by being given Hormone Replacement Therapy
contraceptive advice or treatment without parental • Hormone replacement therapy (HRT) is not contraceptive
consent. as the natural oestrogens do not necessarily inhibit ovulation.
• Women started on HRT before the menopause should continue • Vasectomy is a more minor operation.
contraception (barrier or IUD, possibly the POP) until there • Vasectomy is safer as it is usually performed under local
is reason to think that they have reached the menopause anaesthesia.
(e.g., at age 50). It is impossible to assess the timing of the • A man has a longer reproductive span to lose.
natural menopause in women using HRT. If a woman is • A man is more likely to feel threatened (fear of loss of
unwilling to discontinue HRT for 3 to 4 months to allow sexual drive and sexual prowess).
accurate assessment of FSH levels, then barrier contracep-
tion, an IUD, or the POP can be used until the age of 55, Reversal
at which time loss of natural fertility can be assumed. • If a patient wants a reversal of sterilization, patency rates
• Women on the COC do not need HRT until the COC is of around 90% can be achieved with microsurgical tech-
stopped. niques but fertility is lower: 31% to 92% in females and
9% to 82% in males (rates tend to be at the low end of
Contraception and Learning Disability this range when the vasectomy was performed more than
(Cooper, 2000) 10 years previously).
• When counselling a patient, be aware that risk factors
• Contraception is most likely to be considered in women for subsequent regret about sterilization include:
with learning disability who: • age younger than 30;
1. are entering a sexual relationship. There is a 40% chance • no children;
of learning disability in children born to a couple where • not in a relationship;
both parents have learning disability; • association in time with pregnancy (full term or
2. are in danger of being exploited. abortion);
• Consent. Consult a consultant psychiatrist if there • crisis in relationship or not in mutually faithful relationship;
is doubt about the ability to give consent. The • coercion by partner or health professional;
majority of patients with moderate and mild learn- • psychological or psychosocial issues.
ing disability are able to give consent.
• Sex education. Refer to the local community learning Termination of Pregnancy (Anon., 2004c)
disability team, social services department, educa-
tion department, or voluntary organization. In the UK, the Abortion Act of 1967, as amended by the
• Epilepsy. If patients are on enzyme-inducing anti- Human Fertilisation and Embryology Act of 1990, allows
epileptics, the dose of the COC needs to be higher. TOP if two doctors agree that:
Those on the POP should change the method. • the continuance of the pregnancy would involve risk to
the life of the pregnant woman greater than if the preg-
Sterilization (Anon., 2004b; Brechin and nancy were terminated; or
Brigg, 2006) • the termination is necessary to prevent grave permanent
injury to the physical or mental health of the pregnant
When counselling for sterilization the following should be woman; or
borne in mind: • the pregnancy has not exceeded its 24th week and
• Both partners should ideally be seen together. the continuance of the pregnancy would involve risk,
• Patients should be made aware of the high efficacy of greater than if the pregnancy were terminated, of
alternative long-acting reversible contraceptive methods injury to the physical or mental health of the pregnant
(National Collaborating Centre for Women’s and Chil- woman; or
dren’s Health, 2005). • the pregnancy has not exceeded its 24th week and the
• The possibility of death of partner or child should be continuance of the pregnancy would involve risk, greater
covered. than if the pregnancy were terminated, of injury to the
• The possibility of relationship breakdown should be discussed. physical or mental health of any existing child(ren) of
• The pros and cons of male versus female sterilization the family of the pregnant woman; or
should be explained. • there is a substantial risk that if the child were born it
would suffer from such physical or mental abnormalities
Which Partner Should Be Sterilized? as to be seriously handicapped.
Sometimes it is not the partner who presents who is subse- Note: There is no time limit for the first, second, and last
quently sterilized. Points to be raised here are: grounds.
• Vasectomy is more effective than tubal occlusion: 1 in • Mortality for a TOP is 0.2 per 100,000 compared to 6
2000 lifetime pregnancy risk (when postoperative semen per 100,000 for death in childbirth.
analysis is adhered to) compared with 1 in 200 for tubal • The risk of serious complications and death increases with
occlusion (all varieties of technique). Effectiveness of the advancing gestation, so do not be responsible for delays
Filshie clip is a little more favourable, but the data are in referral.
less robust. • TOP is not associated with an increase in breast cancer risk.
• There are no proven associations between TOP and sub- • Start investigations as soon as the couple voices anxiety
sequent ectopic pregnancy, placenta praevia, or infertility. about conceiving. Explain that in 30% of cases the
• TOP may be associated with a small increase in the risk problem lies with the male alone, in 30% with the
of subsequent miscarriage or preterm delivery. female alone, and that in the remainder it is mixed or
• Higher quality studies show no higher risk of mental unexplained.
health sequelae in those who have TOPs compared to • Aim to consider referral after 1 year, or sooner if there is
women with unintended pregnancies who continue good reason to predict difficulties (e.g., oligoamenorrhoea
(Charles, Polis, Sridhara, & Blum, 2008). or amenorrhoea, abnormal sperm count, a history of PID,
• Counsel the woman to help her come to her own informed or maternal age over 36). Refer the couple directly to a
decision about TOP that she will not regret and to lessen the tertiary centre holding the contract for the treatment of
risk of emotional disturbance whatever decision is reached. subfertility, not to a general gynaecology clinic.
• Ensure that she is aware of the alternative of continuing
the pregnancy and keeping the baby or giving it up for
Investigations
adoption.
• Check she is not under pressure from partner or parent. Assessment of the Woman
• Discuss the need for and if possible plan future contra- • History should include details of menstrual cycle, previous
ception, including information about emergency contra- pregnancies, pelvic infections, or operations. Regular
ception if not already known about. periods are a reliable guide to the fact that the woman is
• Arrange a follow-up appointment 2 weeks after the ovulating. Check that they are having intercourse at the
termination to check for medical and psychological woman’s most fertile time (as well as throughout the cycle).
sequelae. Do not bother with temperature charts.
• Medical abortions with mifepristone and misoprostol are • Check that the woman is rubella immune and taking
available up to 24 weeks. Up 9 weeks the two oral tablets folic acid.
are usually given 1–2 days apart. Between 10 and 24 • Screen for Chlamydia.
weeks the misoprostol is given as a vaginal pessary. Mul- • Examine for evidence of pelvic pathology.
tiple pessaries may be required, given every three hours, • Progesterone level. Take blood 7 days before menstruation
until the foetus is delivered. Feticide should be carried is due. A level of more than 16 nmol/L suggests ovula-
out from 22 weeks gestation. tion, and a level of more than 30 nmol/L confirms it.
Borderline levels may be due to a deficient luteal phase
The Patient Under 16 Years Old or to mistimed sampling. Levels less than 16 nmol/L
(Anon., 2004a) confirm that the cycle was anovulatory.
• Measure FSH and LH in a woman with irregular cycles
• Involve the parents or guardians, with the patient’s consent. in whom it is impossible to predict when to check the
They will be needed to sign the consent form as well as blood progesterone.
to support the patient before and after the termination. Note: There is no value in measuring TFTs or prolactin
• If the patient refuses to inform a parent or guardian, she in women with regular menses in the absence of galactor-
has the right, under the Family Law Reform Act of 1969, rhoea or symptoms of thyroid disease.
to consent to treatment herself, provided she understands
the nature of the procedure, including its risks and Assessment of the Man
complications. • Medical history should include operations on or infections
• If a competent child consents to treatment, a parent cannot of the testes and operations on the prostate.
override that consent. • Drug history: sulfasalazine, tetracyclines, allopurinol,
anabolic steroids, cannabis, and cocaine have all been
Infertility shown to interfere with male reproductive function.
• Examine him, including genitals and secondary sexual
characteristics.
GUIDELINE
• Screen for Chlamydia.
National Institute for Health and Care Excellence. (2013). • Arrange for semen analysis. Semen should be produced
Fertility problems: Assessment and treatment. NICE clinical by masturbation 3 days after the last ejaculation and
guideline 156. Retrieved from www.nice.org.uk.
examined within an hour.
• If the count is low, repeat after 3 months and refer to a
specialist fertility clinic if still low. However, if the count
• Reassure the couple that 75% of couples achieve concep- is severely low, refer after a single count.
tion within 6 months, and 84% by 12 months. If the • Warn the patient that even a normal sperm count
woman is aged 35 the success rate is 94% over 3 years. does not mean that there is not some sperm dysfunc-
If the woman is aged 38 the success rate is 77% over tion, which can only be detected on more sophisticated
3 years. tests.
Normal Values For Semen (World Health • Sexual problems may have a physical or psychological
Organization) origin, but often they are mixed.
• All patients with sexual problems develop performance
• Volume: ≥2 mL anxiety, where they are not relaxed and spontaneous in
• pH: ≥7.2 love making, are alert to further failure, and become a
• Concentration: ≥20 million/mL spectator of their own sexual performance.
• Total sperm number: ≥40 million/ejaculate • In about 30% of patients, the partner also has a problem.
• Motility: ≥50% with ≥25% showing progressive motility • In any consultation about sexual problems it is important
• Vitality: >75% live to ascertain the following:
• Morphology: >4% normal forms • What is the real complaint?
• White blood cells: <1 million/mL • Why is the problem being presented now?
• Is the desire for change real?
Advice • Who is really complaining, the patient or the partner?
• What are the expectations of the patient?
Advice to the couple should routinely be: • Consider the following psychological aspects in any patient
• both should stop smoking; presenting with a problem:
• the woman should drink no more than 1 or 2 units of • Ignorance and misunderstanding. This includes faulty
alcohol once or twice weekly while trying to conceive; sex education and technique, inability to commu-
• men who drink heavily should cut their drinking to no nicate sexual needs, and unrealistic expectations
more than 3 to 4 units in any day; due to stereotyped views of expected behaviour and
• women and men with a BMI greater than 30 should lose performance.
weight regardless if ovulation is a problem; • Anger and resentment. This often remains unresolved,
• men should avoid soaking in hot baths, wearing tight with the couple unable to communicate their feelings
underwear, and remaining seated for many hours at a time; to each other clearly. It often arises where the partners
• couples should have regular sex throughout the cycle, every are unable to express their feelings as children because
2 to 3 days; there is no evidence that use of temperature of excessive unresolved anger in their parents. It may
charts and LH detection tests to time sex improve preg- occur in response to relationship difficulties, financial
nancy rates. difficulties, children, in-laws, or stress at work.
Use of clomifene in general cannot be justified in general • Shame, embarrassment, and guilt. This may be due to
practice in view of the increased risk of multiple pregnancy and a negative sexual attitude laid down in childhood, where
ovarian cancer. An exception to this is if a woman who previ- the parents looked upon sexuality as bad. Traumatic
ously conceived using it presents again with anovulatory cycles. sexual experiences may add to these fears.
• Anxiety/fear about sex. Fear of closeness, vulnerability,
The Role of the General Practitioner letting go, and failure may lead to a self-perpetuating
After Referral cycle of anxiety.
• Administer the drugs for assisted conception according • Poor self-image may contribute to lack of interest in
to a protocol agreed with the specialist clinic. sex and sexual response. Changes in women may occur
• Support the couple throughout the drawn-out process of after operations, especially mastectomy or hysterectomy,
investigation and treatment. postmenopausally and after childbirth. Both sexes com-
monly suffer after redundancy and when depressed.
Infertility Network UK is a self-help organization that supplies INFORMATION FOR PATIENTS
literature and runs support networks. Tel. 01424 732361, For self-help material online, go to www.netdoctor.co.uk
www.infertilitynetworkuk.com. (search “sex and relationships”).
The Human Fertilization and Embryology Authority, 10
Spring Gardens, London, SW1A 2BU, tel. 020 7291 8200,
www.hfea.gov.uk, has books and videos about assisted
conception.
Erectile Dysfunction
• Erectile dysfunction (ED) occurs in 10% to 15% of men
Sexual Problems but varies with age, with some degree of dysfunction
being experienced by 40% of men at age 40 and by 70%
REVIEW at age 70. ED in an otherwise asymptomatic man may
be a marker for underlying cardiovascular disease. Although
Wylie, K. (Ed.), (2015). ABC of sexual health (3rd ed.). at least 50% of the patients referred to specialist clinics
Malden: Blackwell.
have an organic component, anxiety about the situation
will make things worse (Hackett et al., 2007).
• From the history, assess particularly whether: they have a slower onset of action than injected or trans-
• the ED has arisen suddenly, with a precipitating cause, urethral alprostadil (see later in chapter). Sildenafil and
and is variable, with erections occurring in the early vardenafil are relatively short-acting phosphodiesterase
morning but not during intercourse. These patients are type-5 (PDE5) inhibitors, having a half-life of about 4
likely to have a psychological cause for the problem; or hours (suitable for occasional use), whereas tadalafil has
• the onset was gradual, is constant, with partial or a longer half-life of 17.5 hours (suitable for longer periods
poorly sustained erections and no full early-morning such as over a weekend).
erections. These patients are likely to have a physical • In the United Kingdom, generic sildenafil is available on
cause, although a psychological component is frequently the National Health Service (NHS) for all men with
superimposed. erectile dysfunction and can now be bought over the
• Consider the psychological aspects discussed earlier under counter. Other PDE5 inhibitors can only be prescribed
Sexual Problems. at NHS expense to men who have not responded to
• Be alert to psychiatric problems, including generalized sildenafil and who have any of the conditions approved
anxiety states (excessive adrenergic constrictor tone), by the Department of Health (DoH; see the BNF),
depression, psychosis, body dysmorphic disorder, gender marking the prescription Selected List Scheme (SLS), and
identity problems, and alcoholism. giving enough (usually) for one treatment a week. A private
• Check for the cause being an adverse drug reaction to prescription can be issued to any patient who does not
beta-blockers, thiazides, spironolactone, cimetidine, anti- come within the DoH guidelines.
depressants, phenothiazine antipsychotics, and especially • Studies have shown that patients often take PDE5 inhibi-
alcohol. tors incorrectly and that almost half of previous nonre-
• Examine: sponders respond if the drugs are taken correctly.
• blood pressure, heart rate, waist circumference, and • Instruct the patient to:
weight; • take sildenafil or vardenafil 30 to 60 minutes before
• genitalia (including testicular size, fibrosis in the shaft intercourse and tadalafil several hours before intercourse;
of the penis, and retractibility of the foreskin). • avoid excessive food or alcohol although absorption
• Further examination may sometimes be indicated by age is less of a problem with tadalafil.
or findings in the history especially cardiovascular, neu- • Beware the drug interaction with nitrates and nicorandil
rological, endocrine, and urinary systems. in those with cardiovascular comorbidity.
Workup Non-responders
• Fasting blood glucose and lipid. • Patients should use the drug eight times at maximal dosage
• Testosterone (if history or examination suggests possible before being classified as a non-responder.
hypogonadism or if required to reassure patient); take • Recheck testosterone and prolactin levels in non-responders.
blood at 9 am and not within 3 months of serious illness, PDE5 inhibitors are less effective in those with low or
which can depress the testosterone level. low-normal testosterone levels and there is some evidence
of improved response with testosterone replacement.
ED as a Risk Factor for Cardiovascular Disease • Counselling may be successful in psychogenic impotence
• Observational studies show that factors associated with and is appropriate for couples who do not wish to be
ED are similar to those associated with cardiovascular referred:
disease (CVD; i.e., sedentary lifestyle, obesity, smoking, • See the couple together.
hypercholesterolaemia, and the metabolic syndrome). • Recommend a manual (e.g., The Relate Guide to Sex
• ED seems to carry an independent risk for CVD of in Loving Relationships [see previous box]).
1.46, making it as powerful a predictor of CVD as • Instruct the couple on the sensate focus programme.
smoking. Instructions can be downloaded from the web (e.g.,
• When a man presents with ED which appears to be vas- at https://counselling-matters.org.uk/sites/counselling-
cular in origin, assess his risk of CVD. matters/files/SensateFocus.pdf ).
• Set homework assignments for the couple; these can
Treatment in General Practice be tailored to meet specific needs.
• Exercise and weight loss. In the Massachusetts Male Aging
Study (MMAS) men who started exercise in midlife had Referral
a 70% reduced risk for ED later in life. There is some • Refer those not responding for sex therapy via the local
evidence that exercise and weight loss, in those with estab- family planning clinic or Relate. The outcome is successful
lished ED, leads to a significant reduction in ED. in 50% to 80% of cases.
• Refer for relationship counselling those whom you sense
Phosphodiesterase Type-5 Inhibitor have larger relationship or personality problems.
• These are facilitators rather than initiators of erections • Refer to a urologist those with physical causes, those who
and require sexual stimulation to facilitate an erection; have not responded to PDE5 inhibitors or who have
contraindications to it, and those with psychological causes • vaginismus, which usually becomes apparent at vaginal
that are proving intractable. Possible treatments are: examination (see upcoming discussion);
• hormone replacement therapy in those with • vulval or vaginal causes: infections, vulvar vestibulitis
hypogonadism; syndrome, lichen sclerosus, lichen planus, urethral
• alprostadil, as injections into the corpus cavernosum caruncle, postmenopausal vaginitis, post-episiotomy
or transurethrally; syndrome. Those for whom there is no specific treat-
• vacuum devices; ment may benefit from 5% lidocaine ointment applied
• penile prostheses. 20 minutes before sexual intercourse; they can also be
referred to specialist physiotherapists;
DETAILS OF THERAPISTS ARE • pelvic causes with tenderness on rocking the cervix or
AVAILABLE FROM palpating the fornices (e.g., endometriosis, pelvic infec-
tion, ovarian pathology); and
British Association for Sexual and Relationship Therapy, tel.
020 8543 2707, www.basrt.org.uk. • psychogenic causes, where vulval burning or pain is
Institute of Psychosexual Medicine, Building 3, Chiswick due to somatization of other difficulties.
Park, 566 Chiswick High Road, Chiswick, London, W4 5YA, • Where a physical cause is not found, cognitive behavioural
tel. 020 7580 0631, www.ipm.org.uk. therapy (CBT) and selective serotonin reuptake inhibitors
(SSRIs) have been found to be beneficial.
Lack of Sexual Desire (Basson, 2006) INFORMATION FOR PATIENTS AND

THEIR PARTNERS
• This is the most common presenting female sexual
dysfunction. Vulval Pain Society, PO Box 7804, Nottingham NG3 5ZQ,
www.vulvalpainsociety.org.
• A low level of sexual desire is usually accompanied by
low levels of arousal and sexual excitement and infre-
quent orgasms, and is frequently associated with sexual
dissatisfaction. Vaginismus
• Underlying psychological difficulties are frequent. These
may relate specifically to sex (e.g., previous sexual abuse) or • This consists of a phobia of penetration and involuntary
they may relate to a more widespread psychological disorder. spasm of the pubococcygeal and associated muscles sur-
Physical illness and drugs/substances are also possible causes. rounding the lower third of the vagina. Such women may
• Women specifically lose sexual desire: avoid having cervical smears and sometimes present with
• postpartum; infertility.
• because of pain; • Explore the root cause. It may be:
• where their partner’s performance repeatedly leads to • fear of the unknown or the patient’s ignorance of her
frustration, as in premature ejaculation or ED. own anatomy;
• In general, psychological interventions and sex therapy • a past history of unpleasant experiences such as rape,
are of greater benefit than drugs. sexual abuse or severe emotional trauma, or previous
• Check for the presence of depression and anxiety. dyspareunia;
• Reassure the patient that sexual desire varies at different • a defence mechanism against growing up and becom-
times of life and that loss of desire is not necessarily ing a woman. (A common pattern is for the patient
abnormal. to live close to her parents, remaining the daughter
• Enquire whether she wants to improve her sexual desire and marrying an unassertive man.)
or whether it is her partner’s wish. • Desensitize simple cases by encouraging the woman to
• Consider factors that may be contributing (see previous examine herself, and also encourage her partner to be
discussion). confident enough to insert a finger into the vagina. Some
• Recommend the British Association for Sexual and Rela- women may prefer to use vaginal trainers.
tionship Therapy website, at www.basrt.org.uk, for discus- • Refer to a sex therapist if this simple approach fails. Hyp-
sion on the problem of lack of desire. notherapy may be helpful (McGuire & Hawton, 2003).
• Offer counselling along the lines described under erectile
dysfunction. Disorders of Orgasm
Dyspareunia (Weijmar Schultz et al., 2005) Anxiety tends to delay a woman’s orgasm, but accelerates a man’s.
• This may be primary, or secondary to a physical cause, and Premature Ejaculation (Waldinger, 2007)
considerable skill may be needed to unravel the problem. • This is present when ejaculation occurs sooner than either
• Distinguish from the history and examination between: partner would wish, usually before penetration or soon
• lack of lubrication due to lack of interest; after. Interest in sex may be reduced in both partners.
• Advise the patient that, with practice, he can learn to Patients Requiring Investigation by the
delay ejaculation. The stop/start technique can be taught General Practitioner
by therapists or learnt from the book cited earlier. In
essence, when, during caressing or during intercourse, • Those who are eligible for a local or national screening
a man feels he is close to climax he should stop being programme (e.g., for chlamydia).
stimulated and relax for 30 seconds. Stimulation can then • Those who require treatment urgently and cannot attend
recommence until he is close to climax again, when the GUM immediately (e.g., symptomatic pelvic inflamma-
relaxation is repeated. If this fails, the woman should tory disease out of hours).
squeeze the penis at the base of the glans between finger • Those who are unwilling or unable to attend a GUM
and thumb during relaxation phases. clinic after discussion and recommendation, and require
• Clomipramine 25 mg daily or paroxetine 20 mg each testing for STIs or HIV.
evening are effective in delaying ejaculation. • Those presenting with a possible STI-related problem in
a primary care setting which has training and experience
Retarded Ejaculation in managing STIs, including partner notification (e.g.,
• This is usually a sign of longstanding sexual inhibition. through Locally Enhanced Service schemes or GPs with
Often the patient can ejaculate by masturbation, but not a special interest).
intravaginally. • See discussion later in the chapter for other circumstances
• Explore any feelings of anxiety and guilt. in which HIV testing should be considered.
• Start a sensate focus programme (see previous discussion).
• If home therapy fails, refer for psychosexual counselling. Patients Requiring Management by the
Orgasmic Problems in Women (Meston, Hull, General Practitioner
Levin, & Sipski, 2004) • Those who have tested positive for an STI at the surgery
• A woman who has never achieved an orgasm may have and wish to be managed in primary care or cannot attend
deep-seated psychological reasons for being afraid to let GUM immediately.
go. She may need permission to investigate her body’s • Those who present as contacts of an STI and decline to
own responses further, either by masturbation or vibra- attend GUM for testing, treatment, and partner notifica-
tor. When she has learnt how to relax, she should be tion advice, after discussion and recommendation of this
encouraged to tell her partner and incorporate caressing course of action.
into their usual lovemaking.
• Women who have lost the ability to achieve orgasm may Contact Tracing (Partner Notification)
need counselling, especially about their current relation-
ship or a recent loss of self-image. • This is usually best done by GUM, or at least with their
• Check that she is not taking drugs that inhibit orgasm support and advice.
(e.g., clonidine) and that her failure to achieve orgasm is • As a minimum, screening of all contacts of acute STIs
not due to neurological disease or pelvic surgery. Total over the last 3 months should be attempted. For syphilis
hysterectomy does not impair ability to achieve orgasm and HIV there is no arbitrary limit, and partner notifica-
(Farrell & Kieser, 2000). tion will depend on who is contactable and the earliest
likely time of infection.
• If the patient is diagnosed with nonspecific urethritis
Sexually Transmitted Infections and (NSU), chlamydia, syphilis, Trichomonas vaginalis, or
Human Immunodeficiency Virus gonorrhoea, the partner should be treated even if tests are
negative.
• Sexually transmitted infections (STIs) frequently coexist,
so patients diagnosed with one STI generally need further Principles of Treatment
investigation to exclude other infections.
• GUM or sexual health clinics provide full screening, • Give appropriate antibiotics, bearing in mind any recent
immediate microscopy, and assistance for patients in travel history.
the treating and informing of partners (partner notifica- • Advise complete abstinence from all sexual contact until
tion or contact tracing). This is important in limiting both patient and partner are treated.
the spread of disease, and patients diagnosed with an • Follow-up after completion of antibiotics for compliance
STI should be routinely referred to GUM for further and possibly retesting (which is indicated only in preg-
management. nancy or where symptoms persist).
• Late diagnosis of HIV is a major cause of avoidable mor-
tality in infected individuals and general practitioners Workup for Cases to Be Managed in Primary Care
(GPs) have an important role in making earlier diag- • Take a full sexual history, focusing on the last two partners
noses which improves prognosis and can limit ongoing (irrespective of time interval) or all partners in the last 3
transmission. months (if more than two).
• Current practice is moving toward testing asymptomatic • Gonorrhoea is strongly concentrated among individuals
individuals without examination and reserving examina- with high-risk sexual behaviours and their partners. Refer-
tion for those who are symptomatic. ral to or, at the least, advice from GUM is therefore
• Where examination is indicated, examine the genitalia strongly recommended, and screening for other STIs
for discharge, ulcers, or warts (including the vagina and should be routinely undertaken.
cervix in females); examine the mouth for ulcers; and • Antibiotic resistance patterns are changing rapidly; it is
perform proctoscopy if there are anal symptoms or a advisable to check with the GUM clinic for current
history of anal intercourse. recommendations.
• Ceftriaxone 500 mg intramuscularly as a single dose with
Diagnostic Tests in Women 1 g azithromycin as a single dose, or cefixime 400 mg
• Gonorrhoea: cervical, urethral swabs; also oropharyngeal oral as a single dose (if IM injection is contraindicated
and rectal swabs if symptomatic at those sites, if a partner or not tolerated), or spectinomycin 2 g intramuscularly
has gonorrhoea, or if suggested by the sexual history. as a single dose. (At the time of writing, resistance pat-
• Chlamydia: Vulvovaginal swab for nucleic acid amplifica- terns are changing rapidly.)
tion testing (NAAT) has been shown to be more sensitive • Treat all patients for chlamydia infection. The coinfection
than endocervical swabs (Schoeman et al, 2012). First rate is 40%.
catch urine can also be used. • Seek advice for all pregnant women.
• T. vaginalis, bacterial vaginosis, and candida: high vaginal • Trace and treat contacts over the previous 3 months.
swabs may detect candidal infection but are only useful in • Follow-up at least once to confirm compliance, resolution
the diagnosis of T. vaginalis if inoculated into a trichomo- of symptoms, and partner notification.
nal culture medium. Swabs without immediate microscopy
are of limited use for diagnosing bacterial vaginosis. Chlamydia Infection (Uncomplicated)
• Check that cervical smears are up to date.
GUIDELINE AND REVIEW
Diagnostic Tests in Men
• Chlamydia: Testing is by NAAT. First catch urine is the British Association for Sexual Health and HIV. (2015). National
guideline on the management of Chlamydia trachomatis genital
test of choice for men. If urethral swabs are taken then tract infections. Retrieved from www.bashh.org/guidelines.
insert 1–4 cm into the urethra and rotate once.
• Gonorrhoea: Usually diagnosed by culture via urethral/rectal
swab in order to identify resistant strains. It can also be
diagnosed by NAAT either from swabs or first catch urine, • Nucleic acid amplification tests (NAATs) are now standard
but NAAT positive patients should also be cultured. for swab or urine samples. Their sensitivity is over 95%.
• Treatment and partner notification should be undertaken
Tests to Be Done in All Patients Suspected of Having a on strong suspicion (e.g., mucopurulent cervicitis or pelvic
Sexually Transmitted Infection inflammatory disease) before the result is back. Endocervi-
• Check syphilis serology and repeat it 3 months after cal swabs should be rotated firmly and male urethral
exposure if there has been a significant risk. swabs should be rotated 1 to 4 cm inside the urethra.
• Check hepatitis B serology in patients who have been exposed • Give:
in countries with high incidence, if a sexual partner comes • azithromycin 1 g as a single oral dose; or
from an endemic area, in homosexual men, and where the • doxycycline 100 mg twice a day for 7 days; or
patient and partner have a history of injecting drug use. • erythromycin 500 mg twice a day for 14 days (if both
Consider the need for immediate hepatitis B immunization. of the above are contraindicated).
• Discuss and offer HIV testing to all patients. Where a • In pregnancy, give:
patient does not wish to attend a GUM clinic, consid- • erythromycin 500 mg twice a day for 14 days or 500 mg
eration should be given to testing in primary care. four times a day for 7 days; or
• amoxicillin 500 mg three times a day for 7 days; and
Specific Management of Sexually • a test of cure performed 3 weeks after completing therapy.
• Patients should be referred to GUM to discuss partner
Transmitted Infections notification with a trained health adviser. The UK chlamydia
pilots suggest that this is acceptable to patients diagnosed in
Gonorrhoea primary care. A cutoff for 4 weeks is used for symptomatic
Genital Gonorrhoea (Uncomplicated) men; for all others, the lookback should be for 6 months
or to the last partner (whichever is the longer).
GUIDELINE
British Association for Sexual Health and HIV. (2011). National Screening for Chlamydia
guideline on the management of gonorrhoea in adults. • A screening programme for genital chlamydia has been
Retrieved from www.bashh.org/guidelines.
implemented in England aimed at covering 17% of people
under 25 years of age every year (NHS, 2009).
• Screening is particularly important for: symptoms persist, in which case the patient should attend
• women seeking TOP and their partners; GUM. Alcohol should be avoided during and for 48
• sexually active women and men under the age of 25, hours after treatment.
especially teenagers; • Symptomatic disease in early pregnancy can be controlled
• men and women aged 25 and over with a new sexual with clotrimazole pessaries 100 mg daily for 7 days, or
partner, or two or more partners in a year. Aci-gel, before systemic treatment in the second trimester.
• Offer screening with a vulvovaginal swab because it is • Current partners should be screened for STIs.
more sensitive and stable, but agree to urine testing (where
available) by a NAAT if the woman prefers it. Anogenital Warts
• Repeat the screen if the woman changes her partner.
GUIDELINE AND REVIEW
Nonspecific Urethritis British Association for Sexual Health and HIV. (2015).
National guideline on the management of anogenital warts.
GUIDELINE AND REVIEW Retrieved from www.bashh.org/HPV_guideline.
British Association for Sexual Health and HIV. (2015). National
guideline on the management of non-gonococcal urethritis.
Retrieved from www.bashh.org/guidelines.
• Most anogenital warts cause minor irritation or are simply
cosmetic. However, they cause a good deal of psychologi-
cal distress. Patients should be given an explanation of
• Test all patients who have urethritis for gonorrhoea and their condition, emphasizing that the majority of ano-
chlamydia. genital warts are caused by HPV types 6 and 11, which
• First line treatment, if tests for chlamydia and gonorrhoea are not associated with cervical neoplasia.
are negative, is doxycycline 100 mg twice a day for 7 • Many carriers of HPV have no visible lesions.
days, or azithromycin 1 g orally stat. Follow-up at 2 weeks. • Condom use may prevent transmission of warts to unin-
For symptomatic patients, all partners during the past 4 fected partners. Their use with regular partners, however,
weeks should be treated, and treatment of the regular has not been shown to affect the outcome of treatment
partner is recommended in all cases. in patients with visible warts.
• Patients should abstain from sexual intercourse until they • Perianal warts are associated with anal sex (although they
and their sexual partner have completed treatment. can occur without) and should suggest the need for ano-
• Refer unresolving cases to GUM, and all cases where you rectal samples for other STIs, and GUM referral.
are unable to complete partner notification. Some men • None of the existing treatments is satisfactory, and all
have recurring urethritis due to non-infectious causes; in have high recurrence rates. Patients should be made aware
these cases specialist advice and diagnostics, followed by of this. The evidence base for distinguishing first and
reassurance, are important. second line treatments is weak.
• Pregnancy: pregnant women can be treated using cryo-
Trichomonas vaginalis therapy, trichloroacetic acid, or other ablative therapies
in a specialist setting. However, warts often worsen during
GUIDELINE AND REVIEW pregnancy and improve afterwards. The risk of transmit-
ting warts or laryngeal papillomata to the neonate is small,
British Association for Sexual Health and HIV. (2014). National
guideline on the management of Trichomonas vaginalis.
and caesarean section is indicated only in rare cases of
Retrieved from www.bashh.org/guidelines. giant vulval warts or gross cervical warts.
Soft, Nonkeratinized Warts

• Prescribe podophyllotoxin 0.5% (twice daily for 3 days,
• Trichomonas vaginalis (TV) infection is almost exclusively repeated weekly for up to 4 weeks). Patients using podo-
sexually transmitted through urethral or vaginal inocula- phyllotoxin at home must take great care to follow the
tion. It is sometimes diagnosed on cervical cytology, where instructions to avoid chemical burns. It should not be
there is a false positive rate of about 30%, so tests should used in pregnancy and is not licensed for extragenital
be repeated. lesions such as anal warts.
• Trichomonas infection is strongly associated with other • Imiquimod 5% cream is an immune response modifier
STIs, and full screening should be undertaken. which can be used three times weekly for up to 16 weeks.
• Metronidazole 2 g orally stat, or metronidazole 400 mg It should not be used in pregnancy.
twice a day for 5 to 7 days, will clear 95% of infections.
The stat dose is as effective as the 7-day course but is Keratinized Warts
more likely to be associated with adverse effects (Forna • Use ablative therapy such as cryotherapy which is avail-
& Gulmezoglu, 2000). Test of cure is required only if able at GUM clinics.
Herpes Genitalis • Admit patients with urinary retention, meningism, or

severe systemic symptoms.
GUIDELINE AND REVIEW • Offer counselling, including a discussion of the risk of
asymptomatic shedding and its implications for relation-
British Association of Sexual Health and HIV. (2014). ships, and the need to inform healthcare workers in the
National guideline on the management of genital herpes.
Retrieved from www.bashh.org/guidelines. case of later pregnancy. Health advisers at the GUM clinic
are experienced in this area.
Management of Recurrences
• New diagnoses of primary herpes may be due to herpes • Most recurrences cause minor symptoms of a few days
simplex virus (HSV) type 1 or type 2. After childhood, duration.
HSV1 is equally likely to be acquired in the genital or • Severe recurrences may be shortened by oral antiviral
oral areas (Langenberg et al., 1999). agents as discussed. There is no evidence that topical
• Much genital herpes is due to orogenital contact and can antiviral creams are effective. Saline bathing, petroleum
therefore occur in patients who may not consider them- jelly, or lidocaine gel may aid symptoms.
selves as sexually active. • Suppressive therapy. Depending on patient preference,
• Primary herpes is often asymptomatic, and patients often relationship status, and severity of symptoms, it may be
present many years later with symptoms which are due appropriate to offer systemic antiviral treatment to reduce
to recurrence. the duration and severity of attacks and frequency. This can
• Autoinoculation (e.g., from labial herpes) can only occur reduce anxiety, help in adjustment, and improve quality
during primary episodes. Patients who have developed of life, usually in patients with six or more recurrences
herpes for the first time but had no recent sexual contact a year. Suppressive therapy should be undertaken for a
should be referred to a consultant in GUM who will be defined period of time before review, and is best under-
familiar with such cases, and able to advise patients at a taken by or in collaboration with the GUM clinic.
time of considerable distress and uncertainty.
• Genital herpes can be acquired from asymptomatic part- PATIENT SUPPORT GROUP
ners, sometimes in a long-term relationship. Some patients The Herpes Viruses Association, 41 North Road, London N7
never develop symptoms, although those with HSV2 will 9DP, tel. 0845 123 2305, info@herpes.org.uk, www.herpes.
usually develop symptoms at some point (Langenberg org.uk.
et al., 1999).
• Initial episodes are typically far more severe, and of longer
duration, than recurrences. Recurrences tend to decrease Syphilis
over time but are more frequent with HSV2 (Benedetti,
Zeh, & Corey, 1999). • Like gonorrhoea, syphilis is concentrated among individu-
• Diagnosis is by culture or PCR testing and typing of the als at high behavioural risk and their partners, and
virus using a swab from the base of an ulcer, sent rapidly untreated syphilis also has major long-term sequelae.
to the laboratory in special transport media. • Suspected or diagnosed syphilis must be referred to the
• In primary episodes, patients can benefit from practical GUM clinic, where further investigation, treatment, and
counselling in relation to the natural history and issues partner notification will be undertaken.
in relationships (including risk to the partner). This can • Long-term partner notification is required, and these
be provided at the GUM clinic. Psychological stress does patients may be at very high risk for other STIs and
not increase recurrences (Green & Kocsis, 1997). bloodborne viruses.
• Partner notification is guided by the individual case and
history of symptoms in the partner. Health advisers in Human Immunodeficiency Virus and Acquired
the GUM clinic are experienced in this issue and can
help with this.
Immunodeficiency Syndrome
Prevention and Early Diagnosis
Management of a First Symptomatic Episode • Prevention of HIV is an essential part of contraceptive care,
• Oral antiviral therapy, commenced within 5 days of the travel care, and well-person clinics. Testing for HIV should
episode or while new lesions are forming, reduces the now be universally offered as part of antenatal care, so that
severity and duration of that first episode. Options are women avoid vertical transmission, and access services.
acyclovir 200 mg five times daily, valacyclovir 500 mg
twice a day, or famciclovir 125 mg twice a day with a Advising on Safer Sex and Avoiding
duration of 5 days. High-Risk Activities
• Offer analgesia and recommend saline bathing. Topical • Hugging, kissing, and mutual masturbation are safe.
anaesthetics can be used although they occasionally cause • HIV has been transmitted through oral sex, but this is
sensitization. It may be easier to pass urine in a tepid bath. rare. A condom can be used for receptive oral sex, to
improve protection. Condoms can be lubricated with KY • If HIV primary infection is suspected, P24 antigen and plasma
Jelly or similar lubricants but not oils or Vaseline, which proviral DNA or HIV RNA testing may be appropriate, as
reduce their strength by 95%. prognosis may be improved by antiretroviral therapy at this
• High-risk sexual activities include penetrative vaginal or time. GUM specialist advice should be sought.
anal sex without a condom. • A positive antibody test should be repeated to exclude
• Other high-risk activities include sharing needles or identification errors.
syringes for intravenous drug use.
• Sex with local residents in high prevalence areas of the Human Immunodeficiency Virus Testing
world (e.g., Africa, Thailand, parts of South America) or • HIV testing is now more accepted as part of good clinical
with people otherwise at high risk (e.g., men who have care, and in the United Kingdom is offered to all pregnant
sex with men [MSM]) increases the risks in the event of women and patients with TB. Primary care practitioners
condom breakage or unprotected sex. are now encouraged to undertake HIV testing, particularly
• By the end of 2015, an estimated 101,200 people were living where this is unlikely to occur in another setting.
with HIV in the United Kingdom, of whom 13% remained • Patients who do not wish to have the result recorded in
unaware of their infection. HIV incidence is increasing among their notes in primary care should be referred to the
MSM. In 2015, 2800 MSM were diagnosed with HIV in the GUM clinic.
United Kingdom, of whom most probably became infected • The patient should understand that having the test is
there. In 2015 there were 2360 cases of new heterosexually entirely voluntary.
acquired HIV and this was the first year since the 1990s • Written information about the test and its pros and cons
that the number of new heterosexual diagnoses of people is available at www.tht.org.uk (choose “HIV Testing”).
born within the United Kingdom exceeded the number of
those born outside the country. Pre-Test Discussion
• MSM, migrants from countries with high HIV incidence, • Patients should have an HIV test only with their informed
and injecting drug users remain the populations at greatest consent and normally need to come back in person for
risk of HIV infection. the result.
• A high proportion of deaths are now avoidable and attrib- • Issues to be covered in pretest discussion for HIV:
utable to late diagnosis, and early diagnosis is now rec- • Difference between HIV infection and acquired immu-
ognized as a mainstay both of transmission prevention nodeficiency syndrome (AIDS), how the virus is trans-
and of clinical care. mitted, whether the patient has any misapprehensions.
• HIV testing is now recommended on GP registration • Latency between transmission and seroconversion.
in high prevalence areas, and for a range of indicator Ensure that the patient is not in the 3-month window
diseases in which HIV is much more likely than in the period (occasionally longer). If so, discuss the need to
general population. These indicator diseases include all postpone or repeat the test.
sexually transmitted infections, as well as many other • Importance of screening for other STIs and of making
conditions likely to present to the GP, such as severe plans for safer sex whatever the test result.
psoriasis or seborrhoeic dermatitis, chronic diarrhoea, Advantages of the Test
unexplained weight loss or lymphadenopathy, bacterial 1. Effective therapies are available, which extend life expec-
pneumonia, recurrent herpes zoster, unexplained blood tancy to near normal.
dyscrasia, peripheral neuropathy, pyrexia of unknown 2. Knowing the result means that partners can be protected
origin, aseptic meningitis, mononucleosis–like syndrome, if it is positive or make decision to move to safer sex if
and dementia (British Association for HIV, British Asso- negative.
ciation for Sexual Health and HIV, British Infection 3. Decisions about pregnancy can be made.
Society, 2008). 4. Anxiety due to uncertainty about HIV status is ended.
Note regarding insurance reports: The British Medical Asso- 5. Informed decisions about medical issues, such as live vac-
ciation (BMA) and Royal College of General Practitioners cines, can be made.
(RCGP) advise doctors not to answer the lifestyle questions Other Considerations
on insurance reports, even with consent. These questions 1. If positive, the patient will need to cope with a difficult
allocate a person to an at-risk group, which is discriminatory, diagnosis. He or she will need to consider whom to tell.
while only high-risk behaviour is relevant. 2. A positive (but not a negative) result may affect insurance,
and possibly employment prospects.
The Human Immunodeficiency Virus
Antibody Test Post Test Discussion
• The HIV test is 99.9% sensitive and specific 3 months Negative Results
after exposure to the virus (Sloand, Pitt, Chiarello, & • Discuss any concerns that have given rise to the test and
Nemo, 1991), although the window period before becom- how the patient plans to protect him or herself in the
ing positive can occasionally be longer (e.g., after post- future. Discuss the window period and whether further
exposure antiretroviral prophylaxis). testing will be needed for full reassurance.
Positive Results when the CD4 count is <350 × 106/1). These are best
• Ensure that you have time and there will be no interrup- done through the specialist HIV services (usually GUM).
tions to the interview. Tell the patient early in the interview, • Syphilis serology, baseline hepatitis B and C serology,
to allow time for reflection and questions. FBC, and LFT should be repeated yearly, while cyto-
• Establish what the patient knows and expects to happen, megalovirus (CMV) antibodies and toxoplasma antibodies
and ensure that he or she understands the difference should be assessed at baseline.
between HIV infection and AIDS. Ensure the patient • Lipids, blood sugar, calcium/phosphate, and possibly other
understands that there are now many very effective thera- monitoring tests are needed if on therapy. Women should
pies that dramatically improve prognosis. have yearly cervical smears because of the increased inci-
• Explain that the virus is not passed on by normal domestic dence of carcinoma.
or work contact. • Advise patients which symptoms should be treated seri-
• Refer to a specialist clinic, which may be the GUM service ously, including fever, weight loss, diarrhoea, lymphade-
or the Infectious Diseases Unit, for specialist follow-up nopathy, shortness of breath or cough, paraesthesiae,
and support. Clinics will fit in a newly diagnosed patient headache, mouth ulceration, and visual disturbances.
urgently. • Avoid bacille Calmette-Guérin (BCG) and yellow fever
• Discuss and advise on concerns about transmission to vaccines; seek specialist advice for other live vaccines but
others (see box “Advising on Safer Sex and Avoiding High- otherwise vaccinate as usual. Hepatitis B vaccination should
Risk Activities”) and the need for protected sex. be offered if seronegative.
• Find out what the patient’s plans are for the rest of the • Advise the patient to avoid exposure to toxoplasmosis
day and ensure that he or she arranges to meet someone (uncooked meat and unwashed salads), and to cryp-
for support. tosporidium if severely immunosuppressed (unboiled
• Arrange to see the patient again in a few days; do not tap water).
overload with information and issues at this initial • Encourage patients with a CD4 count less than 200 to
interview. take prophylaxis against Pneumocystis carinii pneumonia
• Give information on patient helplines. (PCP) in the form of co-trimoxazole 960 mg daily or
thrice weekly, or dapsone 100 mg daily, or nebulized
pentamidine 300 mg every 4 weeks.
PATIENT INFORMATION • Ensure the patient has adequate psychologic support in
Terrence Higgins Trust, 314-320 Gray’s Inn Road, London, adjusting to the diagnosis.
WC1X 8DP, tel. 080 8802 1221, www.tht.org.uk. • The issue of pregnancy and contraception, together with
The National AIDS Helpline (NAH) on 0800 567 123 for safe sex, needs to be discussed with women on an ongoing
free and confidential advice and information about HIV/AIDS,
other sexually transmitted infections, or sexual health matters. basis. Effective interventions are now available which can
A 24-hour 7-days a week telephone service, NAH can also reduce the risk of transmission to the baby to under 1%.
give details about local services, including sexual health Specialist advice should be sought at an early stage.
clinics and support agencies for people with HIV/AIDS, their • Risk reduction in relation to sexual partners needs to be
partners, family, and friends. regularly reviewed. Transmission of HIV to uninfected
partners, or transmission of resistant strains, is possible
and safe sex must be practised.
Managing the Human Immunodeficiency Symptoms Needing Special Consideration
Virus-Positive Patient in the Community • Cough, especially dry cough with breathlessness in a patient
• In the last few years, communication between special- with minimal chest signs, and (initially) a normal chest
ist HIV clinicians and primary care has improved, and x-ray (CXR) can indicate PCP. This requires urgent special-
patients are often managed in collaboration. This allows ist care. Patients are also at increased risk of community
GPs to gain experience in the condition, while encouraging acquired pneumonia, TB, and bacterial chest infection.
patients to access care for unrelated conditions and to use • Headaches can suggest cerebral toxoplasmosis, non-Hodgkin
out of hours care and community nursing appropriately. lymphoma, tuberculoma, or, importantly, cryptococcal
• Following publication of the Standards for HIV Clinical meningitis, which may not be accompanied by neck stiff-
Care (BHIVA, RCP, BASHH, BIS, 2009) there has been ness or headache.
an emphasis on provision of HIV care within a managed • Diarrhoea can indicate opportunistic bowel infection, includ-
service network, which will be expected to support primary ing cryptosporidium or microsporidium, although often
care and others in providing nonspecialist HIV services. no cause is found and symptomatic treatment is required.
• Dysphagia can indicate oesophageal candidiasis, treatable
Routine Care with fluconazole 100 mg once a day for 14 to 21 days, or
• Regular estimates of viral load and CD4 measurements are (in the severely immunocompromised) CMV ulceration.
important in advising patients to start highly active anti- • Visual disturbances, including flashes and floaters, can
retroviral therapy (HAART) at the optimal time (usually suggest CMV retinitis. This should be referred urgently.
Drug starter packs are usually held at the local accident

Venepuncture and emergency department.
• The same universal precautions should be taken as with • Take blood for baseline HIV and hepatitis B and C
patients of unknown serostatus (gloves, avoid resheathing serology.
needles, place in appropriately sealed packaging, and • If the risk is assessed as potentially significant, antiretroviral
transport in safe containers). Avoidance of resheathing drugs should be commenced as soon as possible, prefer-
needles should be standard practice. ably within 1 hour. Medication should be continued for
• Some laboratories require high-risk stickers depending a total of 4 weeks if subsequent risk assessment confirms
on local policy. High risk does not require stating the this is appropriate. The contents of starter packs are under
diagnosis of HIV for transport purposes, which is unnec- constant review.
essary for routine tests and can generally be avoided in • Unless the source is already known to be negative for
other cases. This practice is an unnecessary risk to patient hepatitis B, or the contact known to be fully immunized,
confidentiality. give active immunization against hepatitis B. Passive
• Spills should be mopped up with hypochlorite (10 parts immunization with hepatitis B immunoglobulin (HBIG)
water to 1 part household bleach) or undiluted Milton. can be given within 48 hours, but may still be worthwhile
up to 7 days after exposure. However, HBIG is expensive,
Post-Exposure Prophylaxis for Healthcare Workers in short supply, and only available for immunocompromised/
(Including Needlestick Injuries) pregnant patients exposed to known hepatitis B. Patients
already immunized against hepatitis B need a booster of
GUIDELINE active immunization.
Department of Health (2008). HIV post-exposure prophylaxis: • If the source patient is of unknown status, he or she
guidance from the UK Chief Medical Officer’s expert advisory should routinely be approached (by someone other
group on AIDS (rev. ed.). London: Department of Health.
Retrieved from www.gov.uk/government/uploads/system/
than the exposed patient) to ask for consent for
uploads/attachment_data/file/203139/HIV_post-exposure_ testing.
prophylaxis.pdf. • Arrange follow-up by Occupational Health, the GUM
department, or another appropriate department for the
exposed worker, to consider testing for HIV and hepatitis
B and C in confidence at a later stage.
• The risk of acquiring HIV infection following percutane-
ous exposure to HIV-infected blood is on average around Summary of Post-Exposure Prophylaxis
3 per 1000, and less than 1 in 1000 after mucocutaneous 1. Clean the wound.
exposure. 2. Get advice. If significant risk exists, offer:
• The risk is increased in cases of deep injury, visible blood • antiretroviral drugs within 1 hour;
on the device causing the injury, a needle which had • active immunization with hepatitis B vaccine;
entered an artery or vein, or terminal illness in the source • passive immunization with hepatitis B immunoglobulin
patient. if donor is HBV positive and recipient immuno-
• An 80% reduction in the transmission rate can be achieved compromised or pregnant.
by appropriate prophylaxis, and all districts should have 3. Take blood for baseline serology of contact and source
an arrangement for 24-hour access to such supplies for (with consent).
exposed healthcare workers. 4. Arrange follow-up.
• The risk of acquiring hepatitis is, in most parts of the
world, greater than that of HIV. Post-Exposure Prophylaxis Following Sexual or
Other Non-Occupational Exposure
Management • Post-exposure prophylaxis for HIV may be required fol-
• After any exposure to blood (regardless if the source lowing sexual exposure. Examples may include some cases
patient is known to be positive for HIV or hepa- of sexual assault and condom failure between HIV dis-
titis B or C), wash the wound liberally with soap cordant couples or unprotected anal sex in men who have
and water but do not scrub. Antiseptics should not sex with men. In these circumstances, prophylaxis against
be used. hepatitis B should also be considered.
• Telephone for advice on local arrangements for 24-hour • Policies and regimens for HIV post-exposure prophylaxis,
access to HIV prophylaxis, and specialist advice on risk including indications for use after sexual exposure, are to
assessment, based on the nature of the exposure, the be found on the British Association for Sexual Health and
risk posed by the source, and the immune status of the HIV (BASHH) website (www.bashh.org/documents/58/
contact. The local consultant in Communicable Disease 58.pdf ). The consultant at the local GUM clinic should
Control (via the Public Health Department), consultant be able to give immediate advice and to assist in risk assess-
microbiologist/virologist, or consultant in GUM will be ment. Prophylaxis, if required, should be started as soon
able to advise if you do not have information to hand. as possible and certainly within 72 hours.
Brechin, S., & Bigrigg, A. (2006). Male and female sterilisation. Current
Notification and Surveillance Obstetrics & Gynaecology, 16, 39–46.
• Microbiology laboratories voluntarily notify positive HIV Brechin, S., & Penney, G. C. (2004). Venous thromboembolism and
results (confidentially and anonymously) to the Com- hormonal contraception. Guideline 40, 1–13. London: Royal College
of Obstetricians & Gynaecologists.
municable Disease Surveillance Centre (CDSC), Centre
British Association for HIV, British Association for Sexual Health and
for Infections, 61 Colindale Avenue, London NW9 5EQ, HIV, & British Infection Society. (2008). UK National Guidelines
or Health Protection Scotland (www.hps.scot.nhs.uk/). for HIV Testing.
These establishments then write to the clinician concerned Canto De Cetina, T. E., Canto, P., & Ordoñez Luna, M. (2001).
asking for further clinical details. Effect of counselling to improve compliance in Mexican women
• Doctors may also notify cases directly. If the patient has receiving depotmedroxyprogesterone acetate. Contraception, 63,
not been referred on for further specialist management, 143–146.
the GP who receives a positive test result may be invited Chan, W. S., Ray, J., Wai, E. K., et al. (2004). Risk of stroke in women
to provide information for the voluntary, confidential, exposed to low-dose oral contraceptives: A critical evaluation of the
anonymized database. evidence. Archives of Internal Medicine, 164, 741–747.
• Details of HIV and AIDS surveillance, together with Charles, V. E., Polis, C. B., Sridhara, S. K., & Blum, R. W. (2008).
Abortion and long-term mental health outcomes: A systematic
recent data, can be seen at www.gov.uk.
review of the evidence. Contraception, 78, 436–450.
Clinical Effectiveness Unit. (2003b). Desogestrel-only pill (Cerazette).
The Journal of Family Planning and Reproductive Health Care / Faculty
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17
Infectious Diseases and
Vaccination
NEIL RITCHIE
Notification of Infectious Diseases Lyme Disease
Vaccination: General Considerations Presentation
Organization of an Immunization Programme in Primary Care Diagnosis
Procedure When Giving an Immunization Treatment
Prevaccination Quality Control Checks Post-Lyme Syndrome
Contraindications Tick Bites
Adverse Reactions Measles, Mumps, and Rubella
Vaccine Damage Payments Clinical Presentation
Live Vaccines Vaccination
Special Considerations for Use of Live Vaccines Indications
Special Cases Contraindications
Specific Infectious Diseases and Immunizations Adverse Effects
Practicalities
Diphtheria, Tetanus, and Polio
Indications Meningococcal and Pneumococcal Infection
Contraindications Meningococcal Vaccines
Management Indications
Group A Streptococcal Infection Contraindications
Practicalities
Haemophilus influenzae Type B
Pneumococcal Vaccines
Indications Indications
Contraindications Contraindications
Hepatitis (Viral) Practicalities
Hepatitis A and E Pertussis
Hepatitis A Vaccination Management of Suspected Cases
Hepatitis B Indications for Vaccination
Serology Contraindications
Vaccination
Staphylococcus aureus, Including MRSA
Practicalities
Management of Methicillin-Resistant Staphylococcus aureus
Hepatitis C
in the Community
Human Papillomavirus
Tuberculosis
Indications
Vaccination
Contraindications
Indications
Influenza Contraindications
Indications Practicalities
Contraindications
Practicalities
Antivirals for Influenza
278
CHAPTER 17 Infectious Diseases and Vaccination 279
Varicella (Chickenpox) and Herpes Zoster Rickettsial Infection

Varicella Vaccine and Immunoglobulin Fever-Arthralgia-Rash Arboviruses
Confirming Susceptibility Viral Haemorrhagic Fever
Indications (Vaccine) Other Causes of Fever
Practicalities (Vaccine)
Diarrhoea
Contraindications (Vaccine)
Adverse Reactions Dermatologic Presentations
Indications (Immunoglobulin) Eosinophilia
Practicalities Screening of Immigrants and Long-Term Travellers
Shingles Vaccine Schistosomiasis
Indications
Strongyloides
Practicalities
Tuberculosis
Treatment of Varicella
Sexually Transmitted Infection
Treatment of Herpes Zoster
Sexually Transmitted Infection and HIV
Pre-travel Advice
General Approach
Taking Medicines Out of the United Kingdom
Patients Requiring Investigation by the General Practitioner
Travellers’ Diarrhoea Patients Requiring Management by the General Practitioner
Malaria Contact Tracing
Travel-Related Vaccination Principles of Treatment
Special Considerations for Vaccines on the UK Vaccination General Investigation in Primary Care
Schedule How to Take Samples
Cholera Management of Specific Sexually Transmitted Infection
Japanese Encephalitis Genital Gonorrhoea
Rabies Chlamydia Infection
Tickborne Encephalitis Lymphogranuloma Venereum
Typhoid Nonspecific Urethritis
Yellow Fever Trichomonas Vaginalis
Symptoms in Travellers Returning From Abroad Anogenital Warts
History
Keratinized Warts
Fever Herpes Genitalis
Malaria Syphilis
Enteric Fever Human Immunodeficiency Virus Infection
Notification of Infectious Diseases GUIDANCE

The Department of Health (DoH) publishes Immunisation Against
Infectious diseases requiring notification to public health authori- Infectious Disease (“The Green Book”), which contains guidance
ties are outlined in Table 17.1. Notification serves two important on the use of vaccines. The book is regularly updated and
purposes: It allows epidemiologic monitoring of infectious dis- presents detailed advice on the individual vaccines. It is an
eases and it allows a coordinated public health response when excellent reference for more complex vaccination questions
required. There is a long list of pathogens that the laboratory is (https://www.gov.uk/government/collections/immunisation
-against-infectious-disease-the-green-book).
required to notify to public health authorities. However, some Vaccine-specific advice contained in this chapter
of the infectious diseases require notification on reasonable clini- references each specific chapter of “The Green Book”;
cal suspicion by the treating medical practitioner. additional references will be specified.
Note that the list of notifiable diseases varies in Scotland Advice about vaccination should also be available through
and Northern Ireland. the local health protection team.
Vaccination: General Considerations

A routine schedule of immunization is provided in Table 17.2. • there is an effective system for the ordering and proper
care of vaccines;
Organization of an Immunization • there are dedicated immunization clinics at times that
Programme in Primary Care are feasible for the patients; and
• written material about the vaccines is available; and that
Ensure that: parents and patients have time to study them and discuss
• effective call and recall systems are in place; any concerns, before giving consent.
TABLE
Notifiable Diseases Procedure When Giving an Immunization
17.1
• Establish that the immunization is needed.
Acute encephalitis* Malaria*
Acute infectious hepatitis* Measles
• Check that the patient is fit and that there are no con-
Acute meningitis* Meningococcal traindications. Minor illness does not mean that the
Acute poliomyelitis septicaemia immunization need be postponed. More severe illness
Anthrax Mumps may, but only because of the difficulty of distinguishing
Botulism Plague subsequent symptoms of the illness from an adverse effect
Brucellosis Rabies
Cholera Rubella
of the immunization.
Diphtheria Severe acute respiratory • Check that consent has been obtained. In childhood
Enteric fever (typhoid or syndrome (SARS) immunizations this usually consists of:
paratyphoid) Scarlet fever* • written consent to the programme in general; and
Food poisoning* Smallpox • verbal consent to the specific immunization at the time.
Haemolytic uraemic Tetanus
syndrome Tuberculosis
Bringing a child to an immunization clinic has been
Infectious bloody diarrhoea Typhus* viewed as giving consent, but parents may not realize
(or suspected Viral haemorrhagic fever which vaccines are going to be given. To be considered
Escherichia coli O157 Whooping cough informed, parents must have been given information
in Scotland) Yellow fever about the benefits and possible adverse effects of the
Invasive group A
streptococcal disease*
vaccine and had a chance to discuss them.
Legionnaires disease • Explain how to treat the more likely adverse effects (e.g.,
Leprosy*,† paracetamol or ibuprofen for pain or fever).
• Explain that information about those who have been
*Not in Scotland, where notifiable diseases additionally include Haemophilus
influenzae type b, necrotizing fasciitis, tularemia, West Nile fever.
immunized will be kept and used to monitor the safety
†
Not in Northern Ireland, where notifiable diseases additionally include and efficacy of the vaccination programme. This is a
chickenpox and gastroenteritis (<2 years). requirement of current Data Protection guidance.
• Check that the vaccines are not out of date and that the
cold chain has not been interrupted. This means you are
satisfied the vaccines have not, at any stage, frozen or
warmed to more than 8°C.
• Check that facilities for resuscitation are available. This
includes a resuscitation pack containing adrenaline that
TABLE is up to date.
17.2 Routine Schedule of Immunizations • Record the date, vaccine, and batch number in each
patient’s medical record.
Age Immunization • Do not clean the limb unless it is obviously dirty.
2 months DTaP/IPV/Hib, rotavirus, MenB, • Use a long (25-mm, blue hub) needle (either 23 or 25
and PCV gauge). This reduces the incidence of adverse reactions,
3 months DTaP/IPV/Hib and rotavirus presumably because it is more likely to reach muscle than
the short (16-mm, orange hub) needle (Diggle, Deeks,
4 months DTaP/IPV/Hib, MenB, and PCV
& Pollard, 2006).
12 months Hib/MenC • If more than one injection is to be given, give them
13–15 months MMR, MenB, and PCV into different limbs. If the same limb must be used, give
injections at least 2.5 cm apart. Record the site of each
2–7 years Annual influenza vaccine
vaccine given.
3 years 4 months– DTaP/IPV or dTaP/IPV and • Give vaccines intramuscularly unless the patient has a
5 years (preschool) MMR bleeding disorder, when deep subcutaneous injection
12–13 (girls only) HPV should be used. Exceptions to this are Bacillus Calmette-
13–18 years Td/IPV and Men ACWY
Guérin (BCG), Japanese encephalitis (JE), varicella, yellow
fever (YF), and some influenza vaccines.
18–24 years Men C (one dose) if not • Use the deltoid or the anterolateral thigh. Using the
already given
buttock risks inadvertent injection into fat which may
65 onwards Flu annually and PCV once reduce the effect of the immunization.
70 years Shingles • Consider how to reduce the trauma of the event for the
child. Infants who were given 25% sucrose solution to
aP, acellular pertussis; D, diphtheria; d, low-dose diphtheria; flu, influenza; drink 2 minutes before the injection and who continue
Hib, H. influenzae b; HPV, human papillomavirus; IPV, inactivated polio
vaccine; MenC, meningococcal C; MMR, mumps, measles, and rubella; to suck on the bottle or on a pacifier while being held by
PCV, pneumococcal vaccine; T, tetanus. their parents while the injection was given cried significantly
less (Reis, Roth, Syphan, Tarbell, & Holubkov, 2003).
• Do not insist that the patient wait in the clinic for 20 minutes • where encephalopathy or encephalitis occurred within 7
after the immunization. There is no evidence in favour of days of the previous immunization, no cause was found,
this time-honoured custom. An exception to this might be and the child had not recovered after 7 days; or
the patient with a history of syncope after injections. • where seizures and fever occurred within 72 hours of
the previous immunization, no cause was found, and
Prevaccination Quality Control Checks the child had not recovered after 24 hours.
• This tightening of the grounds on which immunization
• Check that the vaccine is in its original packaging, so might be refused or deferred comes from the realization
that batch number and expiry date can be checked. that the danger of infection in an unimmunized child
• Check that the vaccine is not out of date. puts him or her at greater risk than repeating the immu-
• Check that the vaccines have been kept in a dedicated nization. Except in the case of anaphylaxis, repetition of
refrigerator with a max/min thermometer whose readings most other apparent adverse effects seems to occur no
have been recorded and have not gone below 2°C or more than would be expected by chance.
above 8°C.
• If transported in a cool box to the clinic, check that the Vaccine Damage Payments
box also has a max/min thermometer which shows the
correct range has been adhered to. Check that vaccine • Vaccine damage payments are for those suffering 60% or
has not frozen because it was packed too close to the ice. more disablement as a result of immunization with one
• Dispose of multidose vials at the end of the clinic. of the recommended childhood vaccines administered
in the United Kingdom under the age of 18. Payment
Contraindications can also be made for damage due to polio, rubella, and
meningococcal C vaccines given after the age of 18, and
It is important to be clear about the genuine contraindica- for damage due to vaccines given as part of the man-
tions to immunization (see under individual vaccines) and agement of an outbreak of infectious disease in the
not to be deflected from immunizing children, for instance, United Kingdom.
because of: • Claim forms are available from the Vaccine Damage Pay-
• eczema; ments Unit, Palatine House, Lancaster Road, Preston PR1
• a family history of allergy; 1HB, tel. 01772 899944, or downloaded from the gov-
• a personal history of allergy unrelated to the vaccine in ernment website, http://www.direct.gov.uk (search on
question; Vaccine Damage Payments).
• a personal history of epilepsy or febrile convulsions;
• a minor infection, without fever or systemic upset; or Live Vaccines
• a nonallergic reaction to a previous immunization.
Serious concomitant infection may be grounds for post- • There are particular issues around the use of live vaccines.
poning immunization but only because of the difficulty of This is because live vaccines contain attenuated pathogens
distinguishing adverse effects of the vaccination from symp- which can cause clinically significant infection in the
toms due to the infection. immunocompromised.
• Live vaccines currently on the UK vaccination schedule
Adverse Reactions are as follows:
• Measles, mumps, and rubella (MMR)
• Previous editions of “The Green Book” listed severe local • Rotavirus
or systemic reactions as relative contraindications to further • Live attenuated influenza vaccine (LAIV)
immunization with that vaccine. The 2006 edition reverses • BCG vaccine
this advice, pointing out that these reactions are not allergic • Shingles
and may not recur with subsequent doses. • Live vaccines in regular use but not given routinely to all
• There is some evidence that antipyretic drugs can reduce are as follows:
the effectiveness of vaccines if given prophylactically • Varicella
to prevent fever but do not prevent febrile convulsions • YF
(Prymula et al., 2009). However, there is no evidence that • Oral typhoid vaccine
paracetamol given once fever develops has any impact on • All polio vaccines currently in use in the United Kingdom
vaccine response. Therefore prophylactic paracetamol or are inactivated, but live vaccine may be in use elsewhere
ibuprofen is no longer suggested following vaccination. The in the world.
only exception is in the case of the 2- and 4-month doses
of 4CMenB, where postvaccination fever is very common. Special Considerations for Use of Live Vaccines
See the section on meningococcal vaccines for details. • Until recently, when using multiple live vaccines, it was
• Anaphylaxis is usually the only absolute contraindication. advised to give them on the same day or to separate
Two situations in which immunization should be deferred them by at least 4 weeks. However, the guidance has
until the child is stable are: recently changed to highlight specific instances where
vaccines are known to interact (Public Health England original dose and certainly lower than the risk of remain-
[PHE], 2015c): ing unprotected.
• YF and MMR: do not coadminister on the same day. • If the patient is certain but the course is incomplete,
Leave at least 4 weeks between the vaccines. If urgent restart where the course left off.
protection is required then give at any time but consider • Usually a course started with one vaccine may be com-
a further dose of MMR. pleted with another vaccine brand. Inactivated polio
• Varicella/zoster vaccines and MMR: give on the same vaccine can be used to complete a course begun with live
day or leave at least 4 weeks between vaccines. polio vaccine.
• Tuberculin skin testing and MMR: MMR increases • Note that for some vaccines, there is a different schedule
the likelihood of a false negative skin test. Wait until for older children. For example, four doses of Haemophilus
the skin test has been read before giving MMR and influenzae type B (Hib) vaccine are given when the course
do not commence a skin test until 4 weeks after MMR is started in infancy but children 1 year or older require
has been given. only a single dose.
• Other live vaccines can be given at any time before
and after each other. People Living With Human Immunodeficiency Virus
• Previous advice that live vaccines should not be given • Generally speaking, inactivated vaccines can be given
within 3 weeks of receiving normal human immu- to those with human immunodeficiency virus (HIV) as
noglobulin now applies only to MMR. Other live normal. However, many vaccines are less effective in HIV
vaccines can be given at any time with respect to positive individuals. Combination vaccines, including a
immunoglobulin. hepatitis A component (Hep A/B and Hep A/typhoid)
• Live vaccines should not be given to the following: should be avoided as there is evidence of decreased efficacy.
• Pregnant women: In most cases the risk is theoreti- • Some live vaccines, such as MMR, varicella, shingles, and
cal only. Women inadvertently vaccinated during YF, can be safely given if the CD4 count is high.
pregnancy should be reassured that the risk is thought • Where there is a choice between live vaccines and a non-
to be very low. replicating alternative (influenza, typhoid, polio), the
• The immunocompromised, including: nonreplicating vaccine should be selected.
a. patients who have received high-dose systemic • BCG is absolutely contraindicated in HIV.
steroids in the last 3 months. This means children Guidelines on the use of vaccines in HIV positive adults
who have received 2 mg/kg/day for at least 1 week (British HIV Association, 2015) is published by the British
or 1 mg/kg/day for at least 1 month, or adults HIV Association, available at http://www.bhiva.org (choose
who have received the equivalent of 40 mg pred- Guidelines).
nisolone per day for more than 1 week or 20 mg/
day for at least 1 month. Lower doses may sup- Other Immunosuppressed Patients
press immunity, especially if combined with cyto- • Ensure that the full course of routine immunization has
toxic drugs. Discuss each case with the specialist been given. If further immunization is needed, discuss
concerned; timing with the physician in charge. Time their admin-
b. patients on biological therapies (e.g., infliximab); istration when immunosuppression is least, in order to:
c. patients with malignancy of the reticuloendothelial • maximize the chance that immunity will develop; and
system; or • in the case of live vaccines, reduce the risk of fulminat-
d. patients who are immunosuppressed due to radio- ing infection.
therapy or chemotherapy in the last 6 months or • In addition, give influenza and pneumococcal vaccination
who are in some other way immunodeficient. and warn the patient to avoid contact with chickenpox
This list is abbreviated and incomplete. For detailed advice and exposed shingles. If exposure does occur, consider
on the use of live vaccines in the immunocompromised, see urgent immunoglobulin (see page 293).
“The Green Book.” Note that not all cases are clear cut and,
if there is doubt about whether vaccination is indicated, this Contacts of Immunosuppressed Patients
should be discussed with a specialist. • Check that the full course of routine immunizations has
been given. Make good any deficiencies.
Special Cases • Immunize against varicella (unless already immune) and
Incomplete or Uncertain Vaccination History influenza, to reduce the risk of catching the disease and
• A flowchart outlining action to be taken in the event passing it to the patient.
of a child presenting late for vaccines is available
from the Department of Health and regularly updated Patients Without an Active Spleen
(PHE, n.d.b). • Give Hib, influenza, meningococcal vaccines and pneu-
• If the patient is uncertain, treat as though unimmu- mococcal vaccine.
nized. With the exception of BCG, the adverse effects • Give oral phenoxymethylpenicillin prophylaxis against
of repeat doses of vaccine are usually lower than the pneumococcal and meningococcal infection.
• The lifetime risk of overwhelming infection postsplenec- • The vaccines to prevent these illnesses are no longer available
tomy is around 5% but the greatest risk is in the first as single component vaccines and therefore the combination
few years following loss of splenic function. Most cases vaccine should be used even if there is only an indication for
are preventable (Newland, Provan, & Myint, 2005). one of the conditions. Four- and five-component vaccines
with the addition of pertussis (and Hib) are available and
Patients With Cochlear Implants or are used routinely in childhood (see Table 17.2).
Cerebrospinal Fluid Leak
• Give pneumococcal vaccine. Indications
• As part of UK immunization schedule (five doses as shown
Patients on Haemodialysis in Table 17.2).
• Give hepatitis B vaccine at double the normal dose for • Travellers to countries with outbreaks/endemic diphtheria
four doses. or polio or travellers who may not be able to seek immedi-
• Patients should also receive influenza and pneumococcal ate medical attention for a tetanus-prone wound who have
vaccine. not received a dose of vaccine within the last 10 years.
• Contacts of a diphtheria case. A booster dose should be
Patients With Haemophilia given even if previously fully vaccinated.
• Give hepatitis A and B vaccines. • Healthcare staff at risk of contact (i.e. microbiology labo-
• Note that patients with bleeding disorders may receive ratory staff) should receive a booster every 10 years.
some vaccines by an alternative route to reduce the risk
of bleeding. Contraindications
• Anaphylaxis to the vaccine or a vaccine component
Patients With Chronic Medical Conditions: Diabetes or • Tetanus-prone wounds. A tetanus-prone wound is defined
Heart, Lung, Liver, or Renal Disease as a wound or burn:
• Give influenza and pneumococcal vaccines. • that requires surgical intervention which is delayed
more than 6 hours;
Clinical Staff • where there is a significant amount of devitalized tissue
• Check that they have documented evidence of two MMR or a puncture type injury, especially with soil/manure
doses or of antibodies to measles and rubella. contamination;
• Consider BCG if Mantoux negative and in close contact • where there is the presence of a foreign body;
with patients with tuberculosis. • with compound fracture; or
• Give hepatitis B vaccine if working with blood or body • in the presence of systemic sepsis.
fluids contaminated with blood, or at risk of sharps inju- High-risk wounds are those that have been heavily contami-
ries, or of being bitten by a patient. nated with material thought likely to contain tetanus spores
(i.e., soil) or wounds that contain extensive devitalized tissue.
Non-Clinical Staff Who Are in Contact With Patients
• Check that they are immunized with MMR, as for clinical Management
staff. • Clean the wound thoroughly.
• If no clear history of chickenpox or shingles, check for • Note that high-risk wounds (as defined) require treatment
varicella antibodies and immunize if negative. with human tetanus immunoglobulin even if the patient
• Give hepatitis B vaccine if they may come into contact is fully vaccinated.
with contaminated sharps. • For nonimmunized or incompletely immunized patients
with a tetanus-prone wound, a detailed summary of actions
Specific Infectious Diseases for incompletely immunized patients is available in the
DoH “Green Book.” Give:
and Immunizations • a tetanus toxoid booster (combined with low-dose
Diphtheria, Tetanus, and Polio diphtheria) or a primary course as appropriate; and
• tetanus immunoglobulin. This is available from Bio Prod-
• Diphtheria is very rare in the United Kingdom and ucts Laboratory (020 8258 2342), or from the Scottish
almost all cases are either imported or arise in the National Blood Transfusion Services (0131 536 5300).
contacts of imported cases. Tetanus is also rare but Give one vial (250 IU) intramuscularly or two vials if more
sporadic cases do occur, usually in elderly individuals than 24 hours have elapsed, the wound is heavily contami-
whose immunity has waned. Good wound care and nated or is a burn, or the patient weighs over 90 kg.
appropriate use of immunization is vital in preven- • In patients of unknown immune status, if patients are
tion of such cases. Polio is also extremely rare in the uncertain whether a primary course has been given, assume
United Kingdom and throughout most of the world but that they will have received primary immunization only
remains endemic in some areas, most notably Pakistan if they were born in the United Kingdom after 1960 or
and Afghanistan. were in the Armed Forces in or after 1938.
Group A Streptococcal Infection • If scarlet fever is suspected, the following actions should
be taken:
• A throat swab for culture although note that a negative
GUIDANCE swab does not exclude scarlet fever as the cause of the
Scottish Intercollegiate Guidelines Network. (2010). illness. The differential diagnosis includes measles, par-
Management of sore throat and indications for tonsillectomy. vovirus (slapped cheek), and infectious mononucleosis.
SIGN clinical guideline 117. http://www.sign.ac.uk/pdf/ • Do not wait for the result of the throat swab before
sign117.pdf.
Public Health England. (2014). Interim guidelines for the prescribing antibiotics: penicillin V 500 mg four times
public health management of scarlet fever outbreaks in per day for 10 days or the age-appropriate dose for
schools, nurseries and other childcare settings. https://www children. Use a macrolide in those unable to take
.gov.uk/government/uploads/system/uploads/attachment penicillin.
_data/file/322727/PHE_Interim_guidelines_for_scarlet_fever • Advise self-isolation for 24 hours after commencing
_outbreaks_in_schools_and_nurseriesFINAL2.pdf.
antibiotic therapy.
• Do not wait for the result of the throat swab before
notifying the local health protection team.
• Sore throat is a common presentation in general practice.
The majority of patients require reassurance and symptom Haemophilus influenzae Type B
management only. Discriminating this group from those
who would benefit from a therapeutic intervention is a Hib is a cause of meningitis and epiglottitis, principally in
significant challenge. children. Since the introduction of vaccine it has become
• Suspect epiglottitis in patients with stridor, respiratory rare in the United Kingdom.
difficulty, or severe systemic illness. Do not examine the
throat of such patients as it may precipitate airway Indications
obstruction. Such patients should be admitted to hospital
immediately. • As per national schedule (see Table 17.2). A child who
• Other indications for admission to hospital are dehydra- misses these doses should be immunized up to the age of
tion and progressive difficulty swallowing. 10. If diphtheria, tetanus, and polio have been given, give
• Recommend ibuprofen or paracetamol for pain relief. a single dose of Hib/meningococcal C (MenC) vaccine.
• Asymptomatic carriage of group A streptococci is common • Household contacts of invasive Hib disease if aged under
and there is little evidence that throat swabs or rapid antigen 10 and not already immunized.
tests are helpful and their routine use is not recommended. • Non-household contacts aged under 10 at a creche, nursery,
• For the majority of patients well enough to be managed or playgroup if not already immunized.
in the community use the Centor criteria to guide a • A patient with invasive Hib disease, regardless of age, unless
decision on the use of antibiotics. subsequent serology shows that immunity has been acquired.
• The Centor criteria suggest that patients with three or • High-risk patients: children and adults with a non-functioning
four of the following criteria are more likely to benefit spleen, or otherwise immunocompromised, should receive a
from antibiotics: tonsillar exudate, tender anterior cervical single booster of Hib/MenC provided they have been fully
lymph nodes, history of fever, and absence of cough. immunized against Hib. If not, the primary course should
• If antibiotics are prescribed, consider whether a delayed be completed if under 10 or, if aged 10 or over, two doses
prescribing strategy is appropriate. of Hib/MenC should be given 2 months apart.
• Penicillin V 500 mg four times per day for 10 days is
recommended for first line treatment. Macrolides are Contraindications
appropriate therapy for patients unable to take penicillin.
Avoid amoxicillin and co-amoxiclav since these often cause • Acute illness
a rash in patients with mononucleosis. • Confirmed anaphylactic reaction to a previous dose or
• There has been a recent increase in cases of scarlet fever to neomycin, streptomycin, or polymyxin B
in the United Kingdom. Scarlet fever is caused by toxin- • A deteriorating neurologic condition is grounds for defer-
producing strains of group A streptococci and presents ring immunization, but a severe reaction to a previous dose
with a history of current or recent pharyngitis and extensive without anaphylaxis (e.g., fever, convulsions, screaming, or a
erythematous rash. hypotonic–hyporesponsive episode) is not a contraindication.
• Consider scarlet fever in patients with a history of phar-
yngitis and rash. Features that increase the likelihood of
scarlet fever are the presence of a sandpaper texture to the Hepatitis (Viral)
rash and a strawberry tongue. Desquamation, particularly Hepatitis A and E
of the fingers and toes, often occurs in convalescence.
• Patients are at risk of complications such as disseminated • Hepatitis A and E are clinically indistinguishable infec-
infection, glomerulonephritis, and rheumatic fever. tions characterized by symptoms of acute hepatitis (fever,
jaundice, right upper quadrant pain, pale stools, and dark offering protection against hepatitis B or typhoid are also
urine). Historically hepatitis A was the principle agent available and can be used where indicated.
of acute viral hepatitis in the United Kingdom but the • Human normal immunoglobulin (HNIG) to provide
incidence of this infection has declined (around 400 cases passive protection is now limited to high-risk contacts of
per year in England and Wales) and hepatitis E has been those with hepatitis A. Supplies are scarce and it should
increasingly reported (around 800 cases per year in England be used only on expert advice.
and Wales). Hepatitis E should be considered in any
patient with evidence of acute hepatitis (Kamar, Dalton, Hepatitis B
Abravanel, & Izopet, 2014).
• Hepatitis A is readily preventable through vaccination • Hepatitis B is a common infection throughout much of
but there is no vaccine to prevent hepatitis E licensed in the world but is relatively uncommon in the United
the United Kingdom. Kingdom.
• Hepatitis A is spread by the faecal-oral route and con- • Due to the low risk, vaccination is directed at those
taminated foodstuffs such as shellfish. It does not cause at risk.
chronic infection and rarely causes death. • Hepatitis B can present as either an acute hepatitis clini-
• In the United Kingdom, hepatitis E is zoonosis spread cally indistinguishable from hepatitis A or as a chronic
principally through eating infected pork. Travel-associated infection with mildly abnormal (or entirely normal) liver
disease is usually contracted in a similar manner to hepa- function tests (LFTs).
titis A. Note that no vaccine for hepatitis E is available • Chronic hepatitis B infection predisposes to cirrhosis and
in the United Kingdom. primary hepatocellular carcinoma which can be present
at presentation.
Hepatitis A Vaccination
Indications Serology
• Laboratory staff working with the virus Hepatitis serology is confusing due to the wide range of
• Haemophiliacs and any others treated with coagulation laboratory tests used. In brief:
factors (give the injection subcutaneously) • Surface antigen (HBsAg) is the best marker of active
• Those with chronic liver disease, including hepatitis B or infection while surface antibody (anti-HBsAg) is the best
C, because of the severity of hepatitis A infection in an marker of immunity (acquired naturally or through vac-
already compromised liver cination). Core antibody (anti-HBc) is not acquired
• Travellers to areas of poor sanitation, especially if travel- through vaccination and therefore suggests previously
ling to areas of high or medium risk repeatedly or for cleared infection in HBsAg negative individuals.
more than 3 months • The presence of e antigen (HBeAg) usually implies high
• Individuals who are at risk because of their sexual behav- viral load and high infectivity while e antibody (anti-
iour, including men who have sex with men (MSM) HBeAg) usually implies low viral load and low infectivity
• Injecting drug users although there are some pitfalls.
• Immunization should be considered for: • Any patient who has active hepatitis B infection should
• close contacts of those with hepatitis A or in the context be referred to a specialist even if they have normal LFTs.
of outbreaks in schools, nurseries, and closed com-
munities. Take advice from the Consultant in Com- Vaccination
municable Disease Control or, in Scotland, from the Indications
Consultant in Public Health Medicine (CPHM); “The Green Book” lists a large number of indications
• residents of institutions for those with learning diffi for hepatitis B vaccine. The following summarize the
culties; and recommendations.
• workers at risk (e.g., sewage workers, laboratory workers Pre-exposure Prophylaxis
exposed to hepatitis A, and those working in institu- • All those who inject drugs, or those considered to be at
tions for those with severe learning disabilities). risk of injecting drugs in the future. Close contacts of
persons who inject drugs should also be offered
Contraindications vaccination
• Severe febrile illness • Those who change sexual partners frequently
• Anaphylactic reaction to the vaccine or, in the case of • Close contacts of those with hepatitis B infection
Epaxal only, to egg • Families adopting children from countries of high preva-
lence and those who may provide foster care at short
Practicalities notice for children with infection
• Give one dose of vaccine intramuscularly into the deltoid. This • Those who may be at increased risk of infection due to
gives immunity for 3 years. A second dose 6 to 12 months medical treatment, including repeated blood product
later gives immunity for 10 to 20 years. A further dose may be administration, and renal dialysis, including those who
given at 20 years if the risk continues. Combination vaccines may require such interventions in the future
• Patients with chronic liver disease • Alternative schedules are available for use in special patient
• Prisoners populations. Children aged 1 to 15 can be protected by
• Individuals in residential accommodation for those with two doses of adult strength vaccine at 0 and 6 months.
learning difficulties and other persons with learning dif- For those with an urgent need of protection (e.g., those
ficulties based on an individual risk assessment imminently travelling abroad), a very rapid schedule (0,
• People travelling to areas with intermediate or high prevalence 7, and 21 days) can be used with a further dose at 12
of hepatitis B based on an individualized risk assessment months.
• Those at occupational risk, including healthcare workers, • Patients with chronic renal failure respond poorly to
laboratory staff, those working in prisons, those working hepatitis B vaccine and a higher dose vaccine (40 µg) is
in residential accommodation for those with learning dif- recommended.
ficulties, and any other occupation based on an appropriate
risk assessment Monitoring of Vaccine Protection
Post-exposure Prophylaxis • All those who are still at risk of hepatitis B should receive
• Infants born to mothers with chronic hepatitis B or acute a final booster dose 5 years after initial vaccination.
hepatitis B during pregnancy (note that further manage- • Vaccine protection can be assessed by measuring anti-
ment, including administration of immunoglobulin [HBIG] HBsAg levels. There is no indication to do this except in
to the infant and treatment of the mother with antiviral patients with occupational exposure and those receiving
drugs may be indicated based on specialist assessment) renal dialysis. In the case of occupational exposure do
• Those who may have been exposed to hepatitis B sexually, the following:
including victims of sexual assault (HBIG may also be a. Check anti-HBsAg levels 1 to 4 months after the
indicated, seek advice) primary course.
• Persons who are accidentally inoculated with potentially b. Those with levels above 100 mIU/mL do not require
infected body fluids (HBIG may also be indicated, seek advice) further monitoring and should receive the booster at
• HBIG is in limited supply and should be used in post- 5 years only.
exposure prophylaxis in genuinely high-risk cases after c. Those with levels 10 to 100 mIU/mL should receive
discussion with a specialist. Hepatitis B vaccine is effective an immediate further dose and the booster at 5 years.
postexposure prophylaxis and is sufficient in cases in which They also do not require further monitoring.
the risk of hepatitis B is low. d. Those with levels below 10 mIU/mL should receive a
• Note that hepatitis B vaccine is frequently indicated for further primary course and further assessment of anti-
those who present for advice about post-exposure pro- body levels at the end of this course. If the levels
phylaxis even when it can be established that the exposure remain below 10 mIU/mL then they should be con-
does not present a risk of hepatitis B transmission (e.g., sidered unprotected and would have to receive HBIG
blood splashes onto intact skin) because the person has in the case of significant exposure to hepatitis B.
an indication for preexposure vaccination. • Patients with renal failure on dialysis require annual moni-
toring of antibody levels with booster doses when antibody
Contraindications levels drop below 10 mIU/mL.
• Anaphylaxis to a previous dose of hepatitis B vaccine or
a vaccine component Hepatitis C
Practicalities • Hepatitis C causes chronic hepatitis in a high proportion
• There are a wide variety of hepatitis B vaccines available of those who have been infected. No vaccine is available
and schedules vary. It is important to refer to the product and acute infection is usually relatively mild and com-
literature to ensure the appropriate vaccine and schedule monly does not come to medical attention.
are chosen. The standard schedule of hepatitis B vaccina- • At the time of writing, a revolution in the management
tion should be used infrequently since the accelerated of hepatitis C is in progress with treatment changing
schedule (doses at 0, 1, and 2 months) gives similar from regimes consisting of pegylated interferon-α and
responses and higher compliance. A further dose should ribavirin to those based on directly acting antivirals. Such
be given at 12 months if the risk is ongoing. regimes are highly effective and it is likely that within
• Where a course is interrupted and there is a high likeli- the lifetime of this book that such treatments will be
hood of future noncompliance, practitioners should take available to all patients with chronic hepatitis C.
advantage of any opportunity to offer additional doses • It is therefore important to identify patients with chronic
of vaccine with the aim of delivering three doses to persons hepatitis C (including patients previously identified but not
at risk. This is particularly important for injecting drug in specialist follow-up) so that they can be offered treatment.
users, who are at particularly high risk of infection. • Some patients do not attend clinics because they are con-
• Only where there is no urgency and an absence of factors cerned about possible side effects of treatment or previ-
suggesting compliance may be poor should the standard ously failed therapy. They should be encouraged to attend
schedule be used. for assessment.
Human Papillomavirus • Patients with chronic respiratory, heart, kidney, liver,

or neurologic disease
• Human papillomavirus (HPV) is a cause of warts and • Patients with diabetes mellitus
premalignant/malignant conditions at a variety of sites. • Patients with significant immunosuppression, including
More than 99% of cases of cervical cancer are associated asplenia
with the presence of HPV and prevention of infection is • Pregnant women
associated with a substantial reduction in the development • Those with a BMI greater than 40
of cervical cancer, particularly among young women. • Health and social care workers
• Several other cancers are also strongly associated with infec- • Residents in residential care homes
tion with HPV, including oral cancer and anogenital • In addition, children aged over 2 years now receive influ-
cancers. enza vaccine as part of the routine childhood immuniza-
• Many varieties of HPV exist but the majority of cancers tion programme.
are associated with types 16 and 18. The currently avail-
able vaccine protects against these types and also types 6 Contraindications
and 11, which are the principal cause of genital warts.
• Anaphylaxis to a previous dose of influenza vaccine or a
Indications vaccine component
Note that nasally administered influenza vaccines are live,
• The vaccine is offered to all young women aged between attenuated, and should not be given to those with significant
11 and 14 as part of the UK national vaccination pro- immunosuppression as defined in the section on live vac-
gramme. Two doses are given at 0 and 6 to 24 months. cines. In cases where live vaccine is contraindicated, inactivated
• Men are currently not offered vaccination on the UK vaccine can be given as an alternative.
routine vaccination schedule (RVS) but at the time of
writing there is availability of the vaccine on a pilot for Practicalities
use of vaccine in sexual health clinics for men who have
sex with men (PHE, n.d.a). Influenza vaccines are given as a single dose except where
children in clinical risk groups are receiving influenza vaccine
Contraindications for the first time. In such cases, a second dose 4 weeks later
may be required; refer to “The Green Book” for details.
• Anaphylaxis to HPV vaccine or a vaccine component
Antivirals for Influenza
Influenza
GUIDANCE
• That influenza is common should not reduce the importance Detailed advice on the use of antivirals in influenza is
of influenza vaccination as influenza is a common cause of available from Public Health England (2016c).
death in the winter months when seasonal infection occurs.
As well as direct mortality from respiratory tract infection,
influenza is also associated with increased cardiovascular mor- • Several drugs with antiviral efficacy against influenza are
tality (Clar, Oseni, Flowers, Keshtkar-Jahromi, & Rees, 1996). available but the only drugs in common clinical use are
• Two types of influenza vaccine are available: oseltamivir and zanamivir. These drugs are recommended
• Inactivated vaccine, which is usually given to adults for treatment and post-exposure prophylaxis in selected
and is administered intramuscularly (or occasionally patients. Oseltamivir is prescribed in tablet form whereas
intradermally) zanamivir is a dried powder inhaler.
• Live, attenuated vaccine, which is used in children • Generally speaking, antiviral therapy is not recommended
aged 2 years or older and is administered intranasally in patients who are previously fit and well and do not
• Both vaccines types, with the exception of specially pro- require admission to hospital. Patients with chronic ill-
duced vaccines for emerging strains of influenza A such nesses or are 65 years or older, who are therefore at
as the H1N1 pandemic in 2009, contain three or four increased risk of developing complicated influenza, should
strains of influenza, including both influenza A and B. be offered antivirals if they present within 48 hours of
The vaccine is reformulated annually based on the strains onset or later at the prescriber’s discretion. The risk factors
of influenza likely to be prevalent in the winter months. for complicated influenza are the same as those used to
indicate eligibility for annual vaccination. It is not neces-
Indications sary to wait until the diagnosis is confirmed before pre-
scribing antivirals.
• All adults aged over 65 years should be offered annual • Most patients should receive oseltamivir for 5 days. Patients
vaccination. In addition, all those aged 6 months and over with severe immunosuppression (defined as in the section
in the following risk groups should be offered vaccination: on live vaccines) may be better managed with zanamavir
because of concern about the development of resistance doxycycline is contraindicated, then amoxicillin 1g three
and such patients should be discussed with a specialist. times daily for 21 days is an alternative.
• NICE guidelines suggest that patients not responding to
Lyme Disease one course of antibiotics may be offered further treatment
with one further course of an antibiotic from a different
RESOURCES class (for example amoxicillin for patients previously treated
with doxycycline. Patients with suspected late or compli-
NICE has recently published guidelines on the investigations cated Lyme should be referred to a specialist.
and management of Lyme disease. These are available at:
https://www.nice.org.uk/guidance/NG95.
Lyme Disease Action is a patient group offering advice Post-Lyme Syndrome
and support, at http://www.lymediseaseaction.org.uk.
Some patients develop chronic, disabling symptoms follow-
ing treatment for Lyme disease. Management is controversial
Lyme disease is caused by Borrelia burgdorferi and presents and some physicians recommend intravenous or prolonged
a variety of clinical difficulties. It is transmitted by tick bites. antibiotic therapy. There is no evidence of a benefit of pro-
In its acute form it is relatively uncontroversial and usually longed antibiotics and such patients may require specialist
straightforwardly managed successfully with antibiotics. Cases assessment.
of late disease are much less common but can be far more
difficult to diagnose and manage. The disease has become Tick Bites
highly politicized, particularly in the United States, where
some states have legislated on issues around diagnosis and • Tick bites are common, and concern about Lyme disease
treatment. prompts an increasing number of patients to present for
assessment.
Presentation • When a patient presents with a tick attached, this should
be removed with the use of a pair of tweezers. The tick
• Lyme disease has a varied initial presentation. In classic should be gripped as close to the skin surface as possible
early Lyme, patients present with a slowly enlarging target and pulled gently and smoothly away from the skin
lesion known as erythema chronicum migrans. Occasionally without twisting.
there may be multiple lesions. Patients are often nonspe- • Antimicrobial prophylaxis of tick bites is not usually rec-
cifically unwell with low grade fever, arthralgia, and leth- ommended in the United Kingdom but can be considered
argy. The rash may be not seen, atypical, or absent and in immunocompromised patients.
there is often no reported history of tick bite. • The US Centers for Disease Control and Prevention (CDC)
• Early complications include painful radiculopathy, cranial suggests some circumstances in which antimicrobial pro-
nerve (especially facial nerve) palsies, meningoencephalitis, phylaxis can be recommended but these circumstances
and carditis (typically presenting with heart block). Late are unlikely to be encountered in the United Kingdom.
complications include arthritis, peripheral neuropathy,
and chronic encephalomyelitis. These complications are Measles, Mumps, and Rubella
uncommon.
These infections were rare in the United Kingdom. However,
Diagnosis following the MMR vaccine scare in the late 1990s, vaccina-
tion rates fell and were slow to recover. Thus there is a current
• Early Lyme infection is a clinical diagnosis. cohort of young adults who are at increased risk of infection
• Serologic testing is unhelpful since it is frequently negative and cases of measles, mumps, and rubella occur in the United
in early infection and seropositivity among asymptomatic Kingdom. Recognition is important since public health
controls in endemic areas is relatively common. Therefore measures are required to prevent outbreaks.
patients with a typical rash and history of a tick bite should
be treated on clinical grounds without serology. Clinical Presentation
• Patients with early disease who are appropriately treated
often do not develop antibodies and so convalescent serol- • Measles presents as a febrile, coryzal illness followed by
ogy is also unhelpful in these cases. florid, erythematous macular rash which usually covers
• Serology should be reserved for those where there is signifi- the whole body. Koplik spots may be seen prior to the
cant doubt regarding the diagnosis or those who have com- development of the rash but are rarely clinically detected
plicated disease, in which serology is likely to be positive. in view of the rarity of the diagnosis today. Complications
include pneumonia, meningitis, and the late, devastating
Treatment subacute sclerosing panencephalitis.
• Mumps presents with fever and bilateral, painful parotid
• First line treatment of early Lyme infection is doxycycline swelling. Complications include meningitis, pancreatitis,
100 mg twice daily or 200mg once daily for 21 days. If and orchitis.
• Rubella is a mild illness characterized by coryza and

macular rash. It is only significant because of the severe Meningococcal and
consequences of intrauterine infection. Rubella is detected Pneumococcal Infection
only very rarely in the United Kingdom.
• Encapsulated organisms continue to represent a major
source of morbidity and mortality. Meningococcal disease
Vaccination
is the principle cause of bacterial meningitis in the young
Indications while Streptococcus pneumoniae dominates in older patients.
• All previously unvaccinated individuals born after 1970 The pneumococcus is also the most common cause of
should be vaccinated unless they give a past history of bacterial pneumonia.
infection. Note that vaccinated individuals born in the • Meningitis represents a challenging diagnosis, particularly
1970s will have received measles vaccine only and those in the early stages. While a minority of patients present
born in the 1980s will have received a combined measles/ with the classic triad of fever, headache, and neck stiffness,
rubella vaccine. most present with less specific features. Particularly chal-
• Children are vaccinated at 13 to 15 months and receive lenging is diagnosis in those at the extremes of age, who
a preschool booster. often present very nonspecifically. Rash is usually absent
• Older children and adults should receive two doses of in pneumococcal meningitis and is absent or nonspecific
vaccine 1 month apart. in the early stages of meningococcal infection. However,
Other indications for vaccination include the following: a petechial rash in a patient with features of systemic illness
• Women of childbearing age who are seronegative for is a very specific sign of meningococcal infection and should
rubella. Note that this is commonly only detected in prompt immediate action.
pregnancy and, since live vaccines are contraindicated • A recent UK guideline on the management of meningitis
during pregnancy, should be deferred until after delivery and meningococcal sepsis (McGill et al., 2016) recom-
• Healthcare workers in regular contact with pregnant mends that:
women who are seronegative • patients with suspected meningitis or meningococcal
• Immunocompetent, previously unvaccinated individuals less sepsis should be referred to hospital for assessment;
than 3 days postcontact with a confirmed case of measles • if possible, the patient should arrive in hospital within
• Immunocompromised individuals and nonimmune preg- 1 hour of initial assessment;
nant women can receive human normal immunoglobulin • there should be clear documentation of clinical signs
prophylaxis following measles contact. Immunoglobulin of meningitis (headache, altered mental status, neck
is not recommended following contact with mumps or stiffness, fever), presence or absence of rash, and signs
rubella. of shock (hypotension, capillary refill time); and
• Kernig and Brudzinski signs are insensitive and should
Contraindications not be used to exclude bacterial meningitis.
• Anaphylaxis to MMR vaccine or its components, includ- • The evidence for use of antibiotics in the community is
ing gelatin. Note that a gelatin-free vaccine is available mixed. However, early antibiotics are strongly associated with
as an alternative. Egg allergy, even if severe, is not a con- survival in bacterial meningitis and the UK guidelines rec-
traindication to vaccination. ommend use of prehospital antibiotics in the following
• Note that MMR is a live vaccine and has the same con- circumstances:
traindications and special precautions as other live vaccines • Patients with signs of meningococcal disease (i.e., rash
(see section Live vaccines). and severe sepsis or meningism)
• Presence of features of severe sepsis (i.e., hypotension,
Adverse Effects poor capillary refill, altered mental state)
• Fever, malaise, and rash are common post vaccine and • Where bacterial meningitis is suspected and there will
usually occur in the first week but can occur several weeks be a delay of greater than 1 hour in getting to hospital
after vaccination. • Recommended antibiotics are benzylpenicillin or ceftri-
• Febrile seizures occur in around 1 : 1000. axone, which can both be given intramuscularly. Patients
• Immune thrombocytopenic purpura occurs rarely and with allergy to these antibiotics should not receive anti-
resolves spontaneously. biotics prehospital and hospital transfer should not be
• The putative association between MMR and autism or delayed by administration of antibiotics.
inflammatory bowel disease has not been found in a large
number of well-conducted studies and is not a reason to Meningococcal Vaccines
avoid vaccination.
• There is no evidence that giving individual vaccine com- There are now a variety of meningococcal vaccines in routine
ponents is safer and is not recommended. use in the United Kingdom and reflect the different serotypes
of meningococci. Children receive vaccines against serogroups
Practicalities B and C while teenagers are given a quadrivalent vaccine
• MMR is given by intramuscular injection. protecting against serogroups A, C, W, and Y. The serogroup
B vaccine was the most recently developed vaccine and at

the time of writing has only recently been introduced into Contraindications
the UK immunization schedule. • Anaphylaxis to a previous dose of a pneumococcal vaccine
or a vaccine component
Indications
• Infants, children, and teenagers as part of the national Practicalities
vaccination schedule. Pneumococcal vaccines are given by intramuscular injection.
• Students up to 25 years attending university for the first Infants receive three doses but adults and children over 1
time who have not previously received quadrivalent menin- year are protected with a single dose of vaccine. Booster
gococcal vaccine should receive MenACWY. doses are not usually required except in cases of asplenia/
• Adults and children with asplenia, functional asplenia, splenic dysfunction or chronic renal failure where a booster
and complement disorders, including pharmacologic should be given every 5 years.
complement inhibition, should receive additional doses
of MenC, MenACWY, and MenB (×2) on the schedule Pertussis
shown in “The Green Book.”
• Travellers to countries with high prevalence of meningo- Whooping cough is a toxin-mediated condition that causes
coccal infection should receive MenACWY. chronic cough often followed by a characteristic postcough
• Travellers to the Hajj pilgrimage should receive MenACWY whoop or posttussive vomiting. Infants are at particular risk
(this is an entry requirement for pilgrims entering Saudi of complications and the majority of deaths occur in this age
Arabia). group. Pertussis vaccines in use in the United Kingdom are
• Contacts of patients with meningococcal disease as part acellular and contain purified pertussis proteins.
of a coordinated public health response.
Contraindications Management of Suspected Cases

• Anaphylaxis to a previous dose of a meningococcal vaccine GUIDELINES
or a vaccine component
Public Health England (2016b) publishes “Guidelines for
Practicalities the Public Health Management of Pertussis in England”
which includes sections on diagnosis and management in
• All meningococcal vaccines are given by intramuscular primary care.
injection.
• Prophylactic paracetamol is suggested after the MenB
vaccine at 2 and 4 months due to a high risk of fever;
60-mg doses of paracetamol are suggested immediately • Pertussis should be suspected in any patient presenting
following vaccination and then two more at 4- to 6-hour with a prolonged acute cough (≥14 days) and paroxysms
interverals (PHE, 2015d). of coughing, whooping, or post-tussive vomiting. Apnoeic
episodes in infants are another concerning feature. There
Pneumococcal Vaccines should be increased vigilance in contacts of confirmed
cases or in the context of a known outbreak.
Two pneumococcal vaccines are available in the United • Children with suspected whooping cough should be excluded
Kingdom. Pneumococcal polysaccharide vaccine (PPV) pro- from school or nursery until they have completed 2 days of
vides protection against 23 common serotypes and is given to antibiotic therapy or 3 weeks from the onset of symptoms.
adults but ineffective in young children, while pneumococcal • There is limited evidence that antibiotics shorten the
conjugate vaccine (PCV) is given to infants and children and duration of cough unless given in the early stages of infec-
is effective against 13 common invasive serotypes. tion. However, antibiotics are recommended to eradicate
the organism and prevent onward transmission.
Indications • If antibiotics are given, newer macrolides such as clar-
• Infants receive three doses as part of the national immu- ithromycin or azithromycin are usually recommended.
nization schedule. See PHE guidelines for dosing and advice in infants and
• Those aged 65 and over should be offered vaccination on pregnant women.
the first occasion they attend for influenza vaccine. • Attempt to confirm the diagnosis in all patients. Anterior
• Those in clinical risks groups: nasal swabs and throat swabs have low sensitivity for
• Chronic respiratory, heart, kidney, or liver disease culture and so a posterior nasopharyngeal swab is preferred.
• Diabetes mellitus Using a swab designed for the purpose, pass the swab
• Immunosuppression, including asplenia or splenic along the floor of the nasal cavity into the nasopharynx
dysfunction and send urgently to the laboratory.
• Those with cochlear implants • Polymerase chain reaction (PCR) is now available from
• Those with cerebrospinal fluid leaks many laboratories and has higher sensitivity than culture.
Throat swabs are also useful in this group. Discuss with (PVL) toxin. PVL-producing S. aureus has also been asso-
your local laboratory. ciated with severe necrotizing pneumonias and can affect
• Detection of antipertussis toxin IgG in saliva (children) young, otherwise healthy people.
or blood (adults) is available for those who have had • No vaccine is currently available to protect against staphy-
symptoms for more than 2 weeks and have not been lococcal disease.
recently vaccinated. Discuss with public health.
• Prophylactic therapy may be appropriate for close contacts Management of Methicillin-Resistant
if any of the contacts are vulnerable or at high risk of Staphylococcus aureus in the Community
infecting vulnerable groups (i.e., those caring for newborn
babies). Public health will advise on this. • MRSA colonization is more common in patients with a
history of hospital treatment and those who are residents
Indications for Vaccination in care homes. MRSA colonization does not require sys-
temic antibiotic therapy, although decolonization may
• Pertussis vaccine is only available in combination with other be attempted using topical treatments but this is usually
vaccine products. It is given as part of the UK national attempted only in patients who are hospitalized or con-
vaccination schedule. Four doses are given starting at 2 sidering elective hospital treatment. Such treatment should
months and is completed with the preschool booster. be discussed with an infection specialist.
• Pregnant women are offered a booster, ideally between weeks • MRSA infection should also be suspected in patients with
16 and 32 of gestation although the vaccine can be given soft tissue infections that fail to respond to standard therapy.
later in pregnancy, even up to the time of delivery. The vaccine • Patients known to be colonized with MRSA should be
is given to promote passive immunity in the infant and intro- advised to:
duction of the antenatal booster has been associated with a • continue usual social activities, as there is no need to
significant reduction in infant pertussis. Since only combina- self-isolate;
tion vaccines are available, women should be offered a single • be particularly careful with hygiene;
dose of diphtheria, tetanus, polio, and pertussis vaccine. • exercise caution when in contact with people who may
be particularly susceptible to infection, including those
Contraindications with open wounds, in-dwelling vascular devices, and
dermatologic conditions. Close contacts should be
• Anaphylaxis to a previous dose of a pertussis vaccine or advised to keep wounds and sores covered;
a vaccine component. • avoid sharing personal items such as towels with
others; and
Staphylococcus aureus, Including MRSA • inform any treating healthcare professionals of the
history of MRSA colonization.
GUIDELINES • Patients known to have MRSA or at high risk should be
discussed with an infection specialist if treatment is
The British Society of Antimicrobial Chemotherapy publishes
guidelines on management of MRSA infection in the
required. MRSA is often resistant to multiple antibiotics
community (Nathwani et al., 2008). and a regime of treatment should be carefully chosen to
There are also guidelines from the Royal College of Nursing suit the patient’s needs:
(2010) which include a section on care in the community. • Oral antibiotic therapy usually consists of a combina-
The Department of Health has published an information tion of oral antibiotics to reduce the risk of further
sheet for patients in primary care affected by MRSA (http://
mrsaactionuk.net/pdfs/MRSA_Advice.pdf).
resistance developing.
• If rifampicin is used, be aware of the potential for
drug interactions and seek help from a pharmacist if
necessary. Rifampicin should always be used as part
• S. aureus is a common colonizing bacteria. However, it of a combination therapy.
can cause serious soft tissue infections, particularly in • Note that a culture positive for MRSA from a wound
patients with wounds or immune compromise. Complica- does not mandate treatment if the wound is not
tions include endocarditis and osteoarticular infection. showing clinical signs of infection.
While great strides have been made in the last 10 years • All doctors and other healthcare professionals have a
to reduce the burden of disease caused by methicillin responsibility to maintain good infection control practice
(flucloxacillin)–resistant S. aureus (MRSA), which is usually to protect patients from harm.
hospital acquired, there has been little impact on cases • Ensure you are aware of the dress code for your place
of community-acquired staphylococcal disease that are of work and adhere to it.
usually sensitive to flucloxacillin. • Wash your hands after every patient contact.
• Recently there has also been increased awareness of isolated • It is good practice to carry alcohol hand gel when
cases and outbreaks of cutaneous abscesses caused by conducting home visits so that the hands can be
S. aureus expressing the Panton–Valentine leukocidin cleansed if there is no soap and water available.
Tuberculosis known contact of a case of pulmonary TB. There are

intermittent shortages of BCG vaccination which mandate
prioritization of BCG vaccination. The highest priority
GUIDELINES
for vaccination is infants at high risk.
National Institute for Health and Care Excellence
(NICE, 2016) guidelines contain important information on the
diagnosis and public health response to tuberculosis as well Contraindications
as recommendations for specialist clinics.
• Significant immunosuppression or immunocompromised,
including HIV infection
• Previous BCG vaccination or a positive tuberculin skin
• The incidence of tuberculosis (TB) has been rising in the test/interferon gamma release assay (If there is an uncertain
United Kingdom since the 1980s, although this may now vaccination history then the vaccine should only be given
be reversing. This rise has been driven by a rising number after a negative tuberculin skin test.)
of cases among those born outside the United Kingdom. • A personal history of TB infection
• The highest incidence of tuberculosis is found in people • A history of anaphylaxis to BCG
born in India and Pakistan, and among black Africans. • Those with septic skin conditions and patches of eczema
Most patients who develop TB do so after they have lived
in the United Kingdom for years. Practicalities
• Other risk factors for TB include ethnic minorities born
in the United Kingdom; immunocompromise, including • BCG is given by strictly intradermal injection as deeper
HIV infection; and a family history of TB. inoculation causes significant risk of abscess formation,
• All tuberculosis in the United Kingdom should be managed keloid scarring, and other local complications. BCG should
in specialist clinics. All patients with suspected TB should only be given by staff who have been appropriately trained
be referred to a specialist for investigation. in its delivery.
• Suspect TB in any patient with a persistent productive cough, • Axillary lymphadenitis with or without ulceration usually
especially if there are risk factors and/or constitutional symp- resolves spontaneously. Evidence of dissemination beyond
toms such as fever, night sweats, and weight loss. the local lymph node is likely to imply immunosuppres-
• Many laboratories do not routinely perform microscopy sion and such patients should always be referred to a
and culture for TB on sputum so ensure the clinical sus- specialist.
picion of TB is indicated on the request. • After BCG is given, the arm must be avoided for further
vaccinations for 3 months.
Vaccination
Varicella (Chickenpox) and Herpes Zoster
BCG vaccine contains a live, attenuated strain of Mycobac-
terium bovis. Immunization is now targeted on those at highest • Chickenpox is one of the few vaccine preventable diseases
risk as the incidence in the general population has declined. that remains common in the United Kingdom. The Joint
BCG offers particular protection against extrapulmonary Committee on Vaccination and Immunisation (JCVI)
forms of disease but only modest protection against pulmo- has concluded that universal varicella vaccination would
nary disease. not be cost effective and there is the theoretical risk of
paradoxically increasing incidence of chickenpox and
Indications shingles in adults although such an effect is not demon-
strated in the United States where the vaccine has been
• All infants living in a part of the United Kingdom with in use for some time. However, the varicella vaccine is
an incidence of TB greater than 40/100,000 (mostly available for certain groups in whom protection is par-
inner-city areas with large immigrant populations) ticularly desirable.
• Infants and children under age 16 born in a country or • The herpes zoster vaccine is unusual in that it is given to
who have a parent or grandparent born in a country with boost naturally acquired immunity to varicella to reduce
an incidence of TB greater than 40/100,000 the risk of virus reactivation. It is particularly effective in
• Infants and children under age 16 who are previously unvac- reducing morbidity due to postherpetic neuralgia.
cinated, tuberculin negative contacts of a case of pulmonary • Both vaccines are live, attenuated vaccines. They differ in
TB; neonates should not receive BCG in this situation that the inoculum is much smaller in the varicella vaccine.
• Those considered to be at occupational risk of tuberculosis
• Those under age 16 planning to live or work with local
Varicella Vaccine and Immunoglobulin
people for more than 3 months in a country with an
incidence of TB greater than 40/100,000 Confirming Susceptibility
• Tuberculin skin testing is not usually performed before • Generally a clear personal history of chickenpox can be
vaccinating those under 6 years of age unless they are a considered a reliable indicator of immunity to varicella.
• Where there is doubt those in whom therapy is being pregnant woman; neonates from seronegative mothers,
considered can be tested for varicella zoster antibodies. including in cases of maternal chickenpox within 7 days
• In cases where a patient has been exposed to varicella and of delivery; and those with significant immunosuppression.
immunoglobulin therapy is being considered, there is • The contact with varicella is in the preceding 7 days (10
usually sufficient time to check serology. days in cases where the antibody level is already known
to be negative).
Indications (Vaccine)
• Susceptible individuals undertaking healthcare or labora- Practicalities
tory work in which exposure to infected patients or mate- • VZIG is given by intramuscular injection at an age-
rial is likely dependent dose.
• Susceptible close contacts of severely immunosuppressed • Patients with bleeding disorders who cannot receive
children in whom exposure would be likely in the event intramuscular injections can be given normal human
of varicella infection (i.e., siblings) immunoglobulin.
• Note that VZIG contains other antibodies as well and
Practicalities (Vaccine) so can interfere with the development of immunity to
• Two different vaccines are available. Varilrix is licensed live vaccines. Defer vaccination for 3 months or if vac-
only for use in those aged 13 and over while Varivax can cination cannot be avoided then repeat the vaccination
be used from 12 months. Both vaccines are given in two after 3 months.
doses 4 to 8 weeks apart.
• There is evidence that recent MMR can blunt varicella vaccine
Shingles Vaccine
immune responses. MMR should either be given simultane-
ously or separated by an interval of at least 4 weeks. Indications
• Those aged 70 to 79 who have not previously received a
Contraindications (Vaccine) dose of shingles vaccine
• Anaphylaxis to varicella vaccine or one of its components.
• Varicella vaccine is a live vaccine and so should not be Practicalities
given to patients who are pregnant or immunosuppressed • The vaccine is administered intramuscularly or subcutane-
(see the section on live vaccines). ously as a single dose.
• It can be given at the same time as influenza vaccine and
Adverse Reactions pneumococcal vaccine if these vaccines are also indicated.
Rash, which may be vesicular, is commonly associated with
the vaccine (5%–10%). Otherwise the vaccine is usually Treatment of Varicella
well tolerated.
• Healthy children with chickenpox do not usually require
Indications (Immunoglobulin) treatment.
Varicella zoster immunoglobulin (VZIG) is indicated only • Treatment with aciclovir can be considered for those aged
in cases where the following four criteria are met: 14 or older presenting with chickenpox within 48 hours
• The patient has no varicella antibodies—most adults who of the onset of rash as this group is at increased risk of
report no clinical history of chickenpox infection have severe disease (Tunbridge, Breuer, Jeffery, & British Infec-
detectable varicella antibodies. Therefore every effort should tion Society, 2008).
be made to test before giving VZIG; however, this should • Complications of chickenpox include pneumonitis, super-
not extend the wait for VZIG to more than 7 days. added bacterial infection, central nervous system involve-
• There is a history of significant exposure to varicella. This ment, and haemorrhagic complications.
can be difficult to assess: • Pneumonitis is particularly concerning since it can be
• Generally persons with varicella are infectious from 48 present with symptoms but no clinical signs on ausculta-
hours prior to the appearance of rash and those with shin- tion and can be rapidly progressive.
gles are infectious from the appearance of the rash. The • Risk factors for pneumonitis include smoking, preexisting
period of infectivity ends when the lesions are crusted over. lung disease, pregnancy, and immunosuppression.
• In shingles, usually contact with a person with exposed • All patients with complicated chickenpox should be admit-
lesions is required for transmission. Exceptions include ted to hospital. Patients with immunosuppression (as defined
immunocompromised patients with shingles who may in the section on live vaccines) and pregnant patients beyond
shed large amounts of virus. 36 weeks of gestation should also be seen at hospital.
• A significant exposure usually requires contact for a
significant period of time (e.g., sharing a room for 15 Treatment of Herpes Zoster
minutes).
• The patient at risk of acquiring varicella comes from a • Treatment is usually only indicated within 72 hours of
group at high risk of complications. Those at risk include the onset of rash.
• Those aged under 50 rarely have severe shingles and the cover the traveller at all if the main purpose of the trip
risk of postherpetic neuralgia is low. They should not be is to obtain treatment.
routinely treated with antivirals unless they have: • Check that the traveller has received all the routine immu-
• particularly severe pain or rash; nizations recommended for the United Kingdom. Consider
• non-truncal involvement; or giving the following boosters if the last immunization was
• immunocompromise (consider admitting to hospital over 10 years ago and the planned travel poses more risk
unless the patient is not severely immunocompromised, than is present in the United Kingdom: polio, tetanus, and
is systemically well, and the shingles rash is localized). diphtheria. The combined Td/IPV vaccine should be used
• Those over age 50 are at increased risk of severe pain and even if only one or two of the immunizations is needed.
postherpetic neuralgia. They should usually be treated with • Recommend that patients visit the Department of Health
antivirals if they present within 72 hours of the onset of rash. website to review current advice for travellers (http://
• There is no risk to the foetus/neonate from maternal www.nhs.uk/nhsengland/Healthcareabroad/pages/
shingles since maternal antibodies will be present. Treat- Healthcareabroad.aspx). The Foreign Office also publishes
ment in pregnancy is not usually recommended unless up-to-date information on a country-by-country basis
the risks outweigh the benefits of treatment and specialist with general advice about travel (https://www.gov.uk/
advice should be sought if there is concern. foreign-travel-advice).
• Antiviral treatment of shingles should consist of aciclovir, • In regard to prevention, stress the importance of preven-
valaciclovir, or famciclovir. There is some evidence that use tive measures against gastroenteritis, insect bites, sunburn,
of valaciclovir and famciclovir are more effective for early accidents, and sexually transmitted diseases. Travellers
pain but evidence that they are more effective at preventing should know that accidents are the greatest cause of mor-
postherpetic neuralgia is lacking (Chen et al., 1996). bidity and mortality, and that more become HIV positive
• Postherpetic neuralgia is common, particularly in than contract typhoid or malaria.
older patients with shingles and can be very painful. Be • Recommend the appropriate immunizations from an up-
prepared to intervene early with a drug effective against to-date source (see the beginning of this section). For a
neuropathic pain. suggested table of how the necessary immunizations can
• Recurrent shingles is a common reason for referral to be scheduled see Appendix 3.
infectious diseases and immunology clinics. Recurrent • Specialist travel clinics are available throughout the country
shingles in young patients is an important indicator of and are useful for patients with complex or unusual intin-
underlying immune dysfunction (e.g., HIV infection). eraries. Some infectious disease units run specialist travel
However, it is important to consider other diagnoses such clinics (although they will still charge for vaccines). In
as herpes simplex virus (HSV) and dermatologic condi- other parts of the country, specialist services may only
tions. If possible, confirm the diagnosis by swabbing the be available privately. Make sure you know where specialist
lesions for PCR. travel advice may be obtained in your area.
• Altitude sickness is common among travellers going above
Pre-travel Advice 3000 m and can occur above 2500 m. Risk factors include
cardiorespiratory disease. The best way to prevent altitude
• Discuss the itinerary in relation to the traveller’s existing sickness is to acclimatize properly and to limit the rate
health problems. Travellers often do not consider the of ascent. Inexperienced travellers are often exposed to a
impact of their travel plans on their health. Consider if significant risk of altitude illness by attempts to rapidly
patients are fit to travel and any changes to their treat- climb mountains such as Kilimanjaro. Climbers should
ment prior to travel. be advised to stop ascending if they experience headache
• Explain that most countries outside the European Economic while climbing and should descend immediately if they are
Area (EEA) do not have reciprocal health agreements with not feeling better after 6 hours of rest or develop short-
the United Kingdom. Travel insurance is needed. Within ness of breath or alteration of consciousness. A climber
the EEA and Switzerland treatment needed while abroad is who is feeling unwell should never be left alone.
covered by the use of the European Health Insurance Card • Prophylactic treatment with acetazolamide 125 mg twice
(EHIC), which should be obtained before travelling, from daily can be given to patients at high risk of altitude-
the website http://www.dh.gov.uk or by phoning 0300 330 related illness (Ritchie, Baggott, & Todd, 2012). It should
1350. Applicants will need to have their NHS or National be started 2 days prior to ascending above 3000 m and
Insurance number at hand. Application may also be made continued until the day after the descent is begun. Adverse
by post on a form obtainable from a post office but the effects are common but usually mild and include pares-
applicant should allow 21 days for the EHIC to arrive. thesia in the hands and feet, urinary frequency, and altera-
• The EHIC gives the cardholder the same right to state- tion in taste. Acetazolamide is not a licence to ignore the
provided treatment as though a resident of that country. advice about regulating the rate of ascent. Clinical trials
Note that this will not necessarily cover all the costs nor of acetazolamide involve very few cases of severe altitude
extra items, such as repatriation back to the United sickness and there is no evidence that acetazolamide is
Kingdom for which insurance is still needed. It will not effective in preventing life-threatening illness.
• The British Moutaineering Council has an information • Traveller’s diarrhoea is usually caused by enteroaggregative
book on travelling at high altitudes as well as informa- Escherichia coli and is a self-limiting, non-severe illness
tion sheets about common tourist climbs (https://www for most affected travellers.
.thebmc.co.uk/mountain-specific-altitude-advice-sheets). • Advise the traveller that:
• Advise skiers and snowboarders that the evidence is • travellers’ diarrhoea is usually mild and self-limiting;
now in favour of wearing helmets in the prevention of • rehydration is the most important treatment. Ordinary
head injuries, with a 60% reduction in risk (Sulheim water is perfectly acceptable for mild cases but in more
et al., 2006). severe and prolonged cases there is a benefit to oral
• In regard to deep vein thrombosis (DVT), warn those at rehydration solutions;
increased risk of DVT about the hazard posed by longhaul • loperamide can be useful to control symptoms but
travel (say, >8 hours) whether by plane, bus, train, or car. should not be used if there is bloody diarrhoea or high
Explain the need for exercises and avoidance of constrict- fever. It should also not be used in young children;
ing clothing. For those at greatest risk recommend class and
2 below-knee graduated compression stockings. Consider, • medical attention should be sought if there is persistent
in conjunction with a haematologist, the need for injec- blood in the stools, persistent fever, confusion, the
tions of low molecular weight heparin in those with a traveller is unable to get out of bed, or is unable to
previous DVT or pulmonary embolus. maintain hydration by the oral route.
• Antibiotics can be useful in shortening the duration of
Taking Medicines Out of the United Kingdom symptoms. Ciprofloxacin is commonly prescribed but
rising rates of ciprofloxacin resistance among campylo-
• Recommend that a patient who plans to take prescription bacter and salmonella, particularly in India and Southeast
drugs abroad should take them in their original, labelled Asia, is a concern and azithromycin is a useful (unlicensed)
bottles, as well as a copy of the prescription (or the slip alternative. A convenient, single dose of 1 g has been well
for ordering repeat prescriptions). In some countries it is studied and is effective. A recent American clinical practice
an offence to enter with drugs which the patient will guideline summarizes the antibiotic treatment options
consider harmless (e.g., codeine or a hypnotic) without for acute diarrhoea (Riddle, DuPont, & Connor, 2016).
such documentation. • Antibiotic resistance is a concern. One study found high
• Controlled drugs are a special case: rates of carriage of resistant organisms in the faeces of
• Advise the patient that there are limits on the quantity travellers who had taken antibiotics (Kantele et al., 2015).
of controlled drugs that can be taken out of the United Antibiotics should only be prescribed for those in whom
Kingdom. To find out what these limits are and for further a diarrhoeal illness would be particularly troublesome
information see the leaflet published by HM Revenue and and travellers should be counselled not to use them for
Customs, “Taking Medicines With You When You Go mild symptoms.
Abroad,” HMRC reference notice 4, available at http://
www.hmrc.gov.uk (search on Drugs Abroad). Malaria
• Provide a letter detailing the patient’s condition and
drugs taken. GUIDANCE
• Explain that if they are within the limit then there is
no need to declare these drugs to customs in the United Public Health England (2015a) publishes guidelines for
malaria prevention in travellers from the United Kingdom. The
Kingdom. guidelines contain detailed information about all aspects of
• Advise the patient who has to carry more than the malaria prevention. However, the country-specific information
accepted limit of controlled drugs to apply for a licence is not regularly updated.
from the Home Office at least 14 days before travel- For detailed, country-specific information on malaria risk,
ling. Details of how to apply can be found at http:// prevention, and antimalarial resistance, see the country-
specific pages and maps available through the National
www.hmrc.gov.uk. Travel Health Network & Centre (NaTHNaC) or Travax.
• Advise the patient to contact the relevant Embassy,
High Commission, or the Home Office Drugs Branch
(Room 239, 50 Queen Anne’s Gate, London SW1
9AH, tel. 020 7217 8457/8446) for information about • Malaria is prevalent throughout the tropics and is capable
an individual country’s restrictions. of causing severe disease.
• As with most travel-related infections, those visiting friends
Travellers’ Diarrhoea and relatives are at greatest risk of acquiring malaria and
are also the least likely to seek pre-travel advice.
• Travellers’ diarrhoea is one of the most common condi- • Those born in regions where malaria is common often
tions affecting those travelling abroad. do not recognize that their immunity wanes when they
• Among patients travelling to the tropics, a diarrhoeal move to a nontropical region. They are therefore at risk
illness will occur in 60% (Steffen, 2005). of developing severe malaria when they return to visit.
• All travellers to tropical regions should receive advice about • Proguanil is never taken as monotherapy but is used
malaria and some will require pharmacologic prophylaxis. to enhance the efficacy of chloroquine in areas with
• Malaria risk varies from country to country, and often low level resistance to chloroquine. It is taken daily
varies from region to region within countries. Specific and the dose for adults is 200 mg. Weight-adjusted
advice about the country to be visited will be available doses are available from birth.
from NaTHNaC or Travax. • Mefloquine is taken weekly and is highly effective malaria
• All travellers to tropical countries should be advised about prophylaxis in most areas, including those with quinine
bite avoidance. Travellers should be advised about bite resistance. There is some mefloquine resistance in
avoidance even if there is no risk of malaria in the region Southeast Asia, particularly on the Thai/Cambodian
they are visiting. This is important, since other mosquito- border and this drug is not recommended for these
borne diseases such as dengue, chikungunya, and Zika areas. The adult dose of mefloquine is 250 mg weekly.
are common throughout the tropics and can only be It can be used in children weighing over 5 kg. There
prevented by effective bite avoidance: is significant concern about neuropsychiatric side effects
• Wear appropriate clothing: loose fitting clothing cover- and while these are uncommon, they can be severe.
ing as much of the body as possible, preferably sleeves Mefloquine is contraindicated in those with a history
to wrists, long trousers, socks, and shoes. of psychiatric illness or seizures. Those planning to
• Be aware of the peak biting period of mosquitoes. take mefloquine should be advised to begin 3 weeks
While mosquitoes may bite throughout the day, mos- prior to travel so there is time to assess whether adverse
quitoes are most active at dawn and dusk and continue effects will occur and an alternative agent selected.
to bite through the night. Like chloroquine, it should be taken for 4 weeks after
• Ensure protection at night; if not sleeping in a properly leaving the malaria risk area.
screened room, use an insecticide-treated net. Check • Doxycycline is taken daily and is highly effective malaria
the net for tears before use. prophylaxis throughout all risk areas. Side effects include
• Use insect repellent throughout the time in the tropics; dyspepsia, vulvovaginal candidiasis, and skin photo-
50% DEET is most effective and is known to be safe sensitivity. The dose for those 12 years and older is
in pregnancy although it is not suitable for those 100 mg daily but it is not recommended for those
younger than 2 months. Apply after sunscreen to skin under 12 years. It can be commenced 2 days prior to
and reapply frequently. Permethrin treatment of clothes travel and should be continued for 4 weeks after leaving
is also known to be effective in reducing bites. the malaria risk area.
• An information sheet on bite avoidance is available • Atovaquone/proguanil is also taken daily. Adverse
(https://www.gov.uk/government/uploads/system/ effects include headache and gastrointestinal upset.
uploads/attachment_data/file/507350/Mosquito_advice Atovaquone/proguanil has the advantage that it only
_sheet_v2.pdf ). needs to be taken for 2 days prior to entering the area
• All travellers to areas with any risk of malaria should of malaria risk and 7 days after leaving it. This makes
be advised about the need to consider malaria and seek it particularly convenient for short periods of travel in
urgent medical attention if a fever develops up to 1 year risk areas. However, atovaquone/proguanil is the most
after travel. expensive of the commonly used prophylactic regimes
• Malaria prophylaxis is recommended for those visiting which limits its use for most longer term travellers.
areas with a high risk of malaria transmission: The adult dose of the fixed-dose combination is 1
• No malaria prophylaxis is 100% effective, but efficacy tablet daily and it is suitable for children weighing
is significantly reduced if it is not taken diligently. more than 5 kg.
• It is important to note that chloroquine resistance is • Pregnancy is a difficult issue. Pregnancy is a major risk
widely disseminated, and chloroquine is not recom- factor for severe malaria and pregnant women are at
mended for malaria prophylaxis in most areas. See generally increased risk from tropical infection. Pregnant
country-specific guidelines on NaTHNac or Travax women should be advised not to travel to regions with
for advice. malaria risk. If travel is unavoidable, malaria prophylaxis
• In some areas with low level resistance to chloroquine, should be carefully selected. Chloroquine/proguanil is
chloroquine + proguanil is suggested. thought to be safe in all trimesters but is only suitable for
• In other areas where there is high level resistance to a minority of destinations. It is advisable to seek expert
chloroquine, the recommended agents are doxycycline, advice about the prophylaxis of pregnant women travel-
atovaquone/proguanil, or mefloquine. ling to regions with significant chloroquine resistance.
• Chloroquine is taken weekly. It is generally well toler- • Occasionally it may be appropriate to prescribe standby
ated and suitable for long-term use. The dose for adults therapy for malaria for those who may have difficulty
is 310 mg weekly and weight-adjusted doses are avail- accessing prompt treatment for malaria. Atovaquone/
able from birth. Patients should begin treatment 1 proguanil is most commonly prescribed for this indication
week before arrival and continued for 4 weeks after since it is readily available in the United Kingdom. It is
leaving the malaria risk area. important to note that standby treatment is not a
substitute for medical assessment. It should only be pre- long immunity persists after this booster dose is given.
scribed for those who may genuinely struggle to obtain It is reasonable to consider further boosters for those at
prompt medical attention and those who begin treatment continuing high risk.
should seek medical advice as soon as possible.
Contraindications
• Anaphylaxis to cholera vaccine or one of its components
Travel-Related Vaccination
Special Considerations for Vaccines on the UK Japanese Encephalitis
Vaccination Schedule • JE is endemic throughout most of India, Southeast Asia,
• Ensure that travellers are up to date with standard vaccines and parts of China.
and offer boosters as appropriate. Remember that vaccine • Most cases of JE are asymptomatic but it occasionally
preventable diseases now rare in the United Kingdom causes a severe encephalitis with high mortality and fre-
may be common elsewhere in the world. quent neurologic sequelae.
• Only inactivated polio vaccine is in use in the United • Cases are rare in travellers and those visiting cities are
Kingdom. However, countries with active transmission unlikely to be at risk.
of polio often use live, attenuated polio vaccine. Travellers • Transmission is usually during the summer months in
without proof of their vaccination status are sometimes temperate areas and year-round in the tropics and sub-
subject to vaccination with live, attenuated polio vaccine tropics. Country-specific information on transmission is
when visiting such countries (Afghanistan, Pakistan, and available through NaTHNac and Travax.
Nigeria at the time of writing). Immunosuppressed travel- • Vaccine is usually recommended for those visiting endemic
lers should not receive live, attenuated polio vaccine and areas for more than 1 month if travelling during the
so should travel with proof of having received the vaccine transmission season, particularly if travellers are visiting
within the preceeding 10 years. agricultural areas.
• Vaccination with meningococcal ACWY is mandatory
for pilgrims entering Saudi Arabia for the Hajj. Practicalities
• The licensed JE vaccine (IXIARO) has now replaced the
Cholera older unlicensed vaccine (Green Cross) which is no longer
• Cholera is a disease of extremely poor living conditions recommended.
causing profuse watery diarrhoea which is rapidly disabling • Two intramuscular doses of vaccine are given on days 0
and commonly fatal if there is no access to treatment. If and 28. Only if necessary, the course can be accelerated
treated promptly, patients should recover fully. by 4 days.
• It is extremely rare in tourists and vaccination is only • A booster dose can be given to those at ongoing risk 1
recommended for those whose reason for travel (usually to 2 years following the primary course but the duration
humanitarian work) puts them at particular risk. of protection following this dose is uncertain.
• Those who have previously received a primary course of
Indications JE vaccine at any time are protected by a single dose of
• Travellers planning on undertaking disaster relief or aid vaccine (those who previously received Green Cross can
work who may be at risk. Note that most travellers under- receive IXIARO).
taking humanitarian work among local populations are
not at risk and do not require the vaccine Contraindications
• Travellers to areas with active reported transmission of • Anaphylaxis to a dose of JE vaccine or one of its components
cholera where there may be a delay in obtaining medical
assistance Rabies
• Travellers considered at significant risk of acquiring cholera • Rabies is a severe viral encephalitis that is almost univer-
for other reasons sally fatal. However, it can be effectively prevented by
• Laboratory staff who may be exposed to cholera for occu- appropriate use of pre- and postexposure prophylaxis.
pational reasons (usually limited to those in reference and • Terrestrial mammals have been the traditional reservoir
research laboratories) for rabies infection and, globally, most infections are spread
through bites and scratches from infected wild animals
Practicalities such as dogs, cats, raccoons, and monkeys.
• Cholera vaccine is an oral vaccine delivered in two doses • Rabies in terrestrial mammals has been eradicated from
(three doses for children 2–6 years) 1 to 6 weeks apart. the United Kingdom for more than 100 years. However,
It is not recommended for children under the age of 2. related viruses are occasionally present in bats and a man
• If an interval of more than 6 weeks elapses between doses, died from rabies caused by European bat lyssavirus 2
the primary course must be restarted. (EBLV-2) after exposure to a bat in 2002.
• A booster dose can be given to those at ongoing risk after • Thus there are two groups of individuals that require
2 years (6 months for those aged 2–6). It is unclear how protection from rabies:
• Travellers to countries where rabies is endemic • Those with a potential exposure should be carefully
• Those likely to be exposed to animals in the United assessed. Risk assessment guidance is available through
Kingdom that may carry rabies or associated viruses PHE (HPS in Scotland).
(usually for occupational reasons) • Management of individuals potentially exposed to rabies
• For most travellers making short duration itineraries to will include use of vaccine (including giving booster
endemic countries, pre-exposure vaccination will not be doses to those previously vaccinated) and in some cases
recommended because the risk is small and post-exposure where the risk is high, administration of human rabies
prophylaxis is likely to be readily available. immunoglobulin.
• Advice on rabies postexposure prophylaxis can be obtained
Rabies Vaccine through the duty doctor in Colindale (020 8200 4400).
Indications for Preexposure Prophylaxis For contact information in Scotland, Wales, and Northern
• Those continuously exposed to rabies for occupational Ireland see “The Green Book.”
reasons (e.g., working in a reference or research laboratory
handling rabies virus–infected materials) Tickborne Encephalitis
• Those with ongoing risk of exposure to rabies vaccine Tickborne encephalitis (TBE) is an important cause of
(e.g., bat handlers, those working with imported animals, meningoencephalitis across Central and Eastern Europe,
those undertaking veterinary or healthcare work in coun- Russia and the Caucasus, and Japan. It is spread by the bite
tries with endemic rabies) of infected Ixodes ticks and travellers at risk include those
• Those with infrequent periods of exposure risk (e.g., trav- travelling to endemic areas who plan to spend time in forested
ellers to endemic areas). Country-specific risk assessments areas during the tick season (spring to early autumn).
are maintained through PHE (Health Protection Scotland • Advise travellers about the risk from tick bites and suggest
[HPS] in Scotland). the following:
Practicalities • Tuck long trousers into socks and spray exposed skin
• Two rabies vaccines are available in the United with an insect repellent containing DEET and clothes
Kingdom (Rabies Vaccine BP and Rabipur). They are with one containing permethrin.
interchangeable. • Warn travellers to avoid unpasteurized milk; it can be
• Regardless of the indication, all groups should receive caught from the milk of an infected animal.
three doses of rabies vaccine on days 0, 7, and 28 (if • Inspect the skin for ticks daily. If found, ticks should be
necessary, the third dose can be given on day 21). pulled off (intact) by applying steady traction. Do not
• Those with continuous or ongoing risk should have their rupture the tick; mouth parts will be left in the skin.
protection monitored with serology and booster doses of • Offer vaccine to those planning to walk, camp, or
vaccine given to maintain antibody levels in the protective work in forested regions in the infected areas in spring
range. It is anticipated that this will usually be managed or summer. The vaccine is effective and safe. It has
by occupational health departments. been used in Austria as part of the national routine
• Those with infrequent risk of exposure do not require vaccination programme for over 25 years. However,
serology but may be offered a booster at 10 years if there there has never been a case of TBE reported in a travel-
is ongoing infrequent risk. ler returning to the United Kingdom and travellers
• Note that even in those who have been vaccinated, further may reasonably decide to rely on tick-avoidance mea-
action is still required in the event of a significant exposure sures instead of the vaccine.
(see the section on postexposure prophylaxis).
Contraindications Practicalities
• Anaphylaxis to rabies vaccine or one of its components • TBE vaccine is given in three doses at 0, 1, and 6 months.
• Note that when rabies vaccine is used for postexposure • A shortened course given at 0 and 2 weeks can be given
prophylaxis there are no absolute contraindications and if limited time is available but gives less effective
if the patient had a previous severe reaction then expert protection.
advice should be sought since the risk of rabies is usually • Booster doses can be given every 3 years if there is
higher than the risk of a further adverse reaction. ongoing risk.
Post-exposure Prophylaxis
• Rabies can be transmitted by bites and scratches from Contraindications
infected mammals. Infrequently, transmission has also • Anaphylaxis to TBE vaccine or a vaccine component
been reported following exposure of blood or saliva onto • Anaphylaxis to egg
broken skin or mucous membranes. The United Kingdom
has been free of rabies transmitted from the terrestrial Typhoid
mammal population for more than 100 years. • Enteric fever is caused by Salmonella typhi and S. paratyphi.
• Exposure to bats should be considered to carry a (small) • In contrast to other types of salmonella infection in
risk of transmission of rabies wherever in the world it humans, typhoid does not cause prominent gastrointestinal
occurs (including the United Kingdom). symptoms. Instead, patients present nonspecifically unwell
with fever. In severe cases, patients develop multiorgan travel itinerary (e.g., if they are crossing borders at which
failure. a YF vaccination certificate will be required).
• Typhoid is common in parts of the world with inadequate
sanitation and is spread principally through consumption Indications
of contaminated food and drink. • Laboratory staff who may handle infectious material
• Typhoid vaccine offers protection against S. typhi but is • Travellers to countries that require evidence of vaccination
not effective at preventing paratyphoid. on entry
• Travellers to areas with a risk of YF infection even if no
Indications certificate is required on entry
• Travellers visiting typhoid-endemic areas with plans indi-
cating higher risk Adverse Effects
• Travellers with frequent or prolonged exposure to poor • Mild adverse effects such as headache, myalgia, and fever
sanitation are common.
• Laboratory staff who may handle typhoid • Rarely serious adverse effects can occur which may be life
threatening, including YF vaccine–associated neurotropic
Practicalities disease which causes encephalitis and YF vaccine–associated
• Two typhoid vaccines are available in the United Kingdom: viscerotropic disease which causes multiorgan failure.
• Vi vaccine is given by intramuscular injection in a • Severe adverse reactions are rare—around four per
single dose. million doses—but are more common in those older than
• Ty21a vaccine is given as three oral capsules on days 60 years.
0, 2, and 4.
• Vi vaccine is an inactivated vaccine; Ty21a is a live, attenu- Contraindications
ated vaccine. • Those under 6 months of age
• Injectable typhoid vaccines can be given as a combined • Those with a history of anaphylaxis to YF vaccine, a
injection with hepatitis A. vaccine component, or egg
• Whatever preparation of typhoid vaccine is given, booster • Those who have a disorder of the thymus
doses should be given to those at continuing risk every • Immunosuppressed individuals
3 years.
Symptoms in Travellers Returning
Contraindications
• Typhoid Vi vaccine should not be given to those who From Abroad
have had anaphylaxis following a previous dose of Typhoid History
Vi vaccine.
• Ty21a is a live vaccine and should not be given to those The assessment of patients with a history of foreign travel
with immunosuppression. It should also not be given to can be complex. At a minimum, it requires a careful history
those with a history of anaphylaxis to Ty21a or enteric- of the travel, including:
coated capsules, as these capsules contain gelatin. • countries and areas visited: A particularly important
distinction is between urban and rural travel. Gener-
Yellow Fever ally, rural travel is more likely to be associated with
• YF is an arbovirus infection spread by Aedes mosqui- transmission of tropical disease;
toes. YF is present in many countries in Africa and • activities undertaken;
South America. There are also countries that have the • contact with animals;
mosquito vector present but do not report cases of YF • freshwater contact;
infection. • healthcare contact;
• YF vaccine is a live attenuated vaccine that is very effec- • sexual contact; and
tive in the prevention of YF. It should be administered • pretravel health assessment, vaccination history, whether
10 days prior to travel. antimalarial prophylaxis was taken.
• YF vaccine can only be given by registered centres and
these centres are authorized to issue YF vaccination cer- Fever
tificates that travellers can use to demonstrate previous
vaccination. GUIDANCE
• In a recent change, YF vaccination certificates are The British Infection Association published recommendations
now valid for life and booster doses of the vaccine are for the investigation of fever in the returning traveller (Dockrell
no longer recommended by the World Health Organisa- et al., 2009). However, it should be noted that acute fever in
tion (WHO). patients returned from the tropics will usually require
discussion with a specialist.
• It is important to note that travellers may require YF
vaccine even if they are not at risk of YF based on their
• Assessment of travellers with undifferentiated fever is the TABLE Most Common Diagnoses in Patients
mainstay of the inpatient work of any service dealing 17.3 Presenting to GeoSentinel Sites With
with returning travellers. Fever is a common presenting Fever Stratified by Region of Travel
symptom and requires prompt investigation since it is (Leder et al., 2013)
the usual presenting feature of a number of life-threatening
Sub-Saharan Latin America/ Southeast
conditions.
Africa Caribbean Asia
• The initial step in the assessment of any returning traveller
Plasmodium Dengue Dengue
with fever is to conduct a viral haemorrhagic fever (VHF) falciparum P. vivax P. falciparum
risk assessment. The large outbreak of Ebola virus disease Rickettsia, Enteric fever P. vivax
in West Africa in 2013 to 2015 raised the awareness of spotted fever P. falciparum Chikungunya
VHF but the concern is not limited to West Africa. In Dengue Hepatitis A Enteric fever
primary care, if all of the following conditions are met Plasmodium Leptospirosis
vivax Middle East and
then the patient should be discussed with the local infec- Enteric fever North Africa Northeast
tion specialist prior to admission to hospital: Hepatitis A Asia
• A history of fever South-Central P. falciparum Dengue
Asia Acute brucellosis Extrapulmonary
• A history of travel to a country known to have endemic Enteric fever
Enteric fever tuberculosis
VHFs Dengue
Dengue Hepatitis E
• The onset of fever was within 21 days of leaving the P. vivax Q-fever Hepatitis A
risk area Chikungunya Enteric fever
• Note that guidelines are regularly reviewed and that the most Extrapulmonary Rickettsia,
up-to-date advice, including the countries considered at risk, tuberculosis spotted fever
will be available through PHE (2016a) or the appropriate
public health body in the devolved administrations.
• The vast majority of those meeting this definition will • It is vital to remember that while antimalarial prophylaxis
not be considered high risk after discussion with a special- is highly effective, 100% adherence to prophylaxis is not
ist and will be able to be admitted to hospital in the usual sufficient grounds on which to exclude malaria.
way. For further information on VHFs see the respective • P. falciparum usually presents within 2 to 3 months of exposure
section in this chapter. but presentations after 6 to 12 months do occur and other
• GeoSentinel is an ongoing collaboration between many types of malaria can rarely present years after exposure.
travel centres across the world (principally in Europe and • Diagnosis of malaria is made on blood films. However,
North America) collecting information on returning trav- most nonexpert laboratories use rapid diagnostic tests
ellers presenting with illness. This study provides important (antigen tests) as an initial screening tool. These require
information on the prevalence of diseases in unwell return- much less training and experience than performing blood
ing travellers. There are important geographical differences film examination. It is important to note that no single
in the epidemiology of infections in returning travellers test excludes malaria and that three separate examinations
(Table 17.3). 12 to 24 hours apart are required to exclude the diagnosis.
• Note that surveillance of secondary care presentations • If malaria is considered then urgent blood tests are required.
will favour more serious diagnoses. It is likely that non- Unless there is a reliable service in primary care to ensure
tropical infection will predominate but high-quality data that same-day results will be made available, suspected
are not available. malaria should be referred to secondary care.
Malaria Enteric Fever

• Malaria represents a major risk to the health of returning • Approximately 300 cases of typhoid fever are confirmed
travellers. It is capable of rapid progression from nonspe- in England each year although this is probably an under-
cific and relatively mild symptoms to death within a few estimate since blood cultures are often negative.
hours and a common finding in deaths from malaria in • The majority of cases of enteric fever occur in travellers
returning travellers is delayed recognition of the signifi- who have returned from India, Pakistan, and Bangladesh.
cance of symptoms by medical practitioners. • A small number of cases report no travel history but this
• Around 1500 cases are imported into the United Kingdom is extremely rare.
each year. • Suspected cases should be referred to secondary care for
• Patients may present without fever. A history of fever is assessment.
all that is required to consider the diagnosis.
• While malaria caused by Plasmodium falciparum is usually Rickettsial Infection
the most severe, all types of malaria are capable of causing
severe disease and, in particular, severe disease caused by • Rickettsia are another cause of undifferentiated fever in
Plasmodium vivax is increasingly recognized. returning travellers. Rickettsia species cause a wide range of
diseases which are widely distributed throughout the world. • VHF initially presents with undifferentiated fever but a
Mortality varies widely depending on the causative organism. significant number of patients go on to develop haemor-
• Rickettsia are zoonoses spread by insects although the rhagic complications and mortality is high. Fortunately,
vector varies from ticks to mites to fleas. imported cases are rare although there have been a handful
• In some cases there will be an eschar (e.g., in African of cases presenting de novo to hospitals in the United
tick typhus) but in others this is variable or unlikely to Kingdom in the last 10 years.
be seen. • For information on VHF risk assessment see the intro-
• Common features include petechial rash and thrombo- duction to the section on fever.
cytopenia.
• Testing is by PCR and serology but empiric therapy with Other Causes of Fever
doxycycline is indicated for suspected cases who should
usually be referred to hospital. • Viral hepatitis: Hepatitis A is unusual now that most
travellers are vaccinated and acute hepatitis B is rela-
Fever-Arthralgia-Rash Arboviruses tively uncommon in travellers. However, hepatitis E has
become increasingly recognized as a cause of acute hepa-
• Arboviruses are the group of viruses spread by arthropods titis both in the United Kingdom and among travellers.
such as mosquitoes and ticks. They are not necessarily Presentation is with fever and right upper quadrant pain
closely related in other respects and can cause a variety preceding jaundice (which is not always a feature of the
of clinical syndromes. infection).
• One group of arboviruses causes a syndrome consisting • Epstein-Barr virus: Infectious mononucleosis is common
of fever with associated maculopapular (or petechial) rash in younger returning travellers. In those who have nega-
and prominent joint symptoms but only unusually cause tive investigations or are older and so likely to be EBV
serious disease. Until rather recently dengue fever was immune consider cytomegalovirus (CMV). Always con-
the only prominent member of this group but progressive sider an HIV test in patients with a mononucleosis-like
globalization has led to global dissemination of dengue illness. Toxoplasmosis is another cause of persistent
and emergence of previously localized viruses such as lymphadenopathy in travellers.
chikungunya and Zika. • Amoebic liver abscess: An abscess can present many months
• The fever-arthralgia-rash (FAR) group is not reliably clini- after travel without necessarily a history of diarrhoea.
cally distinguishable and is a common cause of presenta- Suspect liver abscess in patients with fever and right upper
tion to secondary care with fever following travel. quadrant pain.
• These viruses are rarely life threatening (although they • Influenza: Flu is common among travellers given the close
can be extremely unpleasant) and treatment is usually contact involved in air travel.
supportive. The most important diagnostic challenge is
usually distinguishing these infections from others that Diarrhoea
require urgent treatment.
• At the time of writing, there is global concern about the • Diarrhoea is very common in returning travellers. Usually
implications of Zika virus infection during pregnancy. it is mild and self-limiting. Specific treatment is usually
Recommendations about this issue are evolving rapidly not required.
and will not be addressed here. • Note that diarrhoea can be a nonspecific symptom of
severe sepsis and fever, with diarrhoea a common present-
Viral Haemorrhagic Fever ing feature of malaria.
• Common causes are similar to locally acquired gastroen-
• The four most prominent VHF–causing viruses are Ebola, teritis with salmonella and campylobacter predominating.
Lassa, Marburg, and Congo-Crimean haemorrhagic fever Note that antibiotic resistance is much more common
(CCHF). There are a number of other viruses that can in infections acquired abroad and varies depending on
also cause VHF with much more limited distributions. geographic location. Seek advice from microbiology/
• With the exception of very rare cases of healthcare infectious diseases if treatment is considered.
workers infected in non-endemic countries, Ebola, Lassa, • Malabsorptive diarrhoea is relatively common following
and Marburg are limited to parts of sub-Saharan Africa. travel. Patients present with nausea, abdominal pain and
However, CCHF has a much broader area of distribu- bloating, watery diarrhoea, and weight loss which may
tion, including Africa, Eastern Europe, Turkey, Russia, last for months:
and Central Asia. In 2016, cases of CCHF were acquired • A number of pathogens can be associated with this
in Spain representing the first cases acquired in Western presentation but Giardia lamblia is the most common
Europe. and can be acquired anywhere in the world.
• In 2013 to 2015 there was a large outbreak of Ebola in • Giardia can be difficult to detect on stool microscopy
West Africa which caused thousands of deaths. However, and serial faeces examinations are often required. If
most outbreaks of VHF are small and sporadic. the history is typical then empiric treatment is often
necessary. Some laboratories now offer giardia antigen TABLE Most Common Diagnoses in Patients
tests on faeces and this is more sensitive. 17.4 Presenting to GeoSentinel Sites With
• First line treatment for giardia is with metronidazole Dermatologic Complaints Stratified by
or tinidazole, although community pharmacies some- Region of Travel (Checkley et al., 2010)
times struggle to obtain tinidazole.
Sub-Saharan Latin America/ Southeast
• In recent years, there have been a series of large out-
Africa Caribbean Asia
breaks of cyclosporiasis centered on Mexico although
Cutaneous larva Cutaneous larva Rabies PEP
many cases have been seen in the United States thought migrans migrans Cutaneous larva
to be associated with imported fruit and vegetables. Rabies Cutaneous migrans
Travellers have been commonly affected. Diagnosis is postexposure leishmaniasis Scabies
through faeces microscopy although this is insensitive. prophylactics Rabies PEP Marine
It is sometimes detected on duodenal biopsy. Make (PEP) Myiasis envenomation
Myiasis
sure the lab is aware of any clinical suspicion and the Tungiasis Middle East and Northeast Asia
travel history. Treatment is with co-trimoxazole. Seek North Africa Rabies PEP
advice if alternative treatment is required. South-Central Rabies PEP Other diagnoses
Asia Cutaneous from this area
• Acute bloody diarrhoea may be caused by salmonella or
Rabies PEP leishmaniasis were very
campylobacter although the odds of other pathogens
Cutaneous Cutaneous larva unusual
increase significantly. leishmaniasis migrans
• Bloody diarrhoea in an afebrile patient (traveller or Scabies Marine
not) suggests E. coli O157 infection, particularly if Cutaneous larva envenomation
there is a risk factor such as livestock contact, camping migrans
on agricultural land, or eating poorly prepared red
meat. Such patients should usually not receive anti-
biotics because of a possible increased risk of devel-
oping haemolytic uraemic syndrome, particularly in
children. • Myiasis is caused by the intradermal inoculation of fly
• Acute bloody diarrhoea with mucus and fever suggests eggs. A painful swelling develops at the site of inoculation
dysentery. Possible causes include Shigella dysenteriae which can be distinguished from a furuncle by the pres-
and Entamoeba histolytica. Treatment is quite different ence of a central breathing pore. A variety of treatment
and so diagnosis is vital. Send faeces (fresh if possible) strategies can be tried but surgical removal is often neces-
to the lab for ova, cysts, parasites examination as well sary. Referral to an expert is usually appropriate.
as culture. • Tungiasis is caused by the invasion of the skin by a gravid
• Shigella is diagnosed on culture but amoebae are seen flea. A firm, pruritic swelling develops, usually in the feet.
on microscopy although some laboratories use antigen The lesions eventually resolve spontaneously but can be
testing as an adjuvant. Note that not all amoebae are surgically removed.
pathogenic and reference lab reports are not always • Cutaneous leishmaniasis is a relatively common condition
easy to understand for the nonexpert. Detection of affecting travellers with an extensive distribution across
Entamoeba dispar or Entamoeba coli is generally con- South/Central America, the Mediterranean, and Central/
sidered nonpathogenic; if there is any uncertainty then South Asia. It is spread by the bite of the sandfly. There are
seek advice. important geographic differences with Old World cutaneous
leishmaniasis rarely becoming complicated (although it
Dermatologic Presentations may cause scarring) but New World cutaneous leishmani-
asis can be life threatening and usually requires systemic
GeoSentinel collects data on dermatologic presentations to treatment. Patients usually present with slowly develop-
specialist travel medicine centres. The top diagnoses by region ing ulcerating lesion on an exposed area. There are often
of travel are shown in Table 17.4. multiple lesions. Diagnosis can be confirmed on biopsy
• Cutaneous larva migrans (CLM) is caused by intradermal but is often made clinically. If suspected, patients should
non-human hookworm larvae. The larvae are unable to be referred to an infectious disease/post travel specialist.
penetrate through the basement membrane and so migrate • Scabies is another common skin condition presenting in
with the skin causing an intense pruritic reaction with a travellers. Patients complain of pruritis and may have
tracking appearance. The infestation is self-limiting but extensive excoriation. The diagnosis is made by finding
is distressing, painful, and can precipitate bacterial infec- skin burrows, which are usually present in the finger webs.
tion. Mebendazole can be used but is inferior to other
unlicensed treatments such as topical thiabendazole, Eosinophilia
albendazole, or ivermectin. Therefore, patients should
usually be referred to a local infectious disease/post travel • Asymptomatic eosinophilia is common in those who have
service for treatment. travelled in the tropics. It may be present in up to 5%
GUIDANCE treatment. Returning travellers with intestinal parasites could

not be distinguished from those without on the basis of symp-
The British Infection Association publishes recommendations
toms in a questionnaire study (Forna & Gulmezoglu, 2000).
for the assessment of eosinophilia in returning travellers
(Soonawala et al., 2014). Most tropical intestinal helminths cannot complete their life
cycle in the developed world and so there is neither a clinical
nor a public health benefit to testing asymptomatic travellers.
of tropical travellers. However, significant eosinophilia, Schistosomiasis

with an eosinophil percentage greater than 15%, is likely
to be associated with an underlying diagnosis. Schistosomiasis is very common in travellers returning home
• Remember that a variety of non-infectious conditions after visiting Africa. In this context it is usually asymptomatic.
can be associated with eosinophilia. Consider whether Travellers are often advised to seek testing to check if they have
atopic conditions may be the cause; other conditions been infected and may attend their general practitioner (GP).
associated with eosinophilia include drugs, vasculitis, and • Symptoms of schistosomiasis include lower urinary symp-
haematologic malignancy. toms and haematuria.
• Eosinophilia in tropical travellers should always be inves- • Early infection with schistosomiasis sometimes causes a
tigated as some causes are associated with significant long- syndrome of acute fever, abdominal pain, splenomegaly,
term consequences. Common causes include: and marked eosinophila (known as Katayama fever).
• intestinal helminths. These are a diverse group and • In symptomatic cases, serology and urine/faeces microscopy
many carriers are asymptomatic. Passing a worm is should be undertaken. Note that these investigations are
often very distressing for patients. If possible it should usually negative in Katayama fever.
be retained and sent to a laboratory for identification; • In asymptomatic cases, serology alone is sufficient and
• strongyloides. This may be asymptomatic but patients should be delayed until 12 weeks after the last possible
often have nonspecific abdominal symptoms. Worms time of exposure to avoid false negatives.
migrating through the skin may be visible as a slowly • Treatment is with praziquantel, which is usually only
moving pruritic rash. If carries become immuno- available from specialist centres.
suppressed, it can cause hyperinfestation syndrome
which presents with paralytic ileus and sepsis, and is Strongyloides
often fatal;
• schistosomiasis. This is very commonly acquired by Strongyloides is unusual among intestinal nematodes in that
travellers and is covered in the next section; it can complete its life cycle within a single host. This allows
• toxocariasis. Hydatid is often asymptomatic but causes for lifelong infection within tropical travellers. Strongyloides
the development of cysts which can develop in most hyperinfestation syndrome is a risk for those who have lived
of the internal organs. If these become large, they can within the tropics who become immunosuppressed. However,
cause significant local symptoms; or strongyloides is relatively uncommon among short-term
• filariasis: There are a number of different types of filarial travellers. A pragmatic approach is to test asymptomatic
infection which cause a diverse range of symptoms, individuals with prolonged tropical travel if they have been
including dermatologic reactions, elephantiasis, and living in areas of particular deprivation or immunosuppres-
visual problems. sion is planned.
• A reasonable initial approach is to send faeces for
microscopy on three occasions and ask the lab to arrange Tuberculosis
serology for strongyloides (for all travellers who have visited
the tropics) and schistosomiasis (for those who have visited • As discussed in this chapter, the United Kingdom has a
Africa). Regardless if a cause is found, it is reasonable to significant burden of tuberculosis among immigrant popu-
refer all patients to a specialist clinic since it is unlikely lations. Most tuberculosis is diagnosed years after the
that treatment will be available outside specialist centres. initial entry to the United Kingdom.
• Note that serology can be difficult to interpret since there • All entrants to the United Kingdom from high incidence
is often cross reactivity between different parasite serologies. countries are screened for active pulmonary TB prior to
being issued with a long stay visa.
• Screening for latent TB in those aged 16 to 35 arriving
Screening of Immigrants and from high incidence countries is about to be introduced
Long-Term Travellers in England. Identification of selected individuals with
latent TB allows treatment to reduce the burden of symp-
Returning travellers from the tropics often request screening for tomatic TB infection in the future (NICE, 2016).
acquired infections, particularly parasitic infection. Unfortu- • Screening for latent TB involves the use of either tuberculin
nately, with only a couple of exceptions, such testing is usually skin testing (Mantoux) or interferon gamma release assays
unhelpful and may expose travellers to needless worry and (IGRA) blood tests. A positive test should prompt a search
for evidence of active TB. If no active disease is found, in managing STIs, including partner notification (e.g.,
selected groups may be offered treatment for latent TB. through either Locally Enhanced Service schemes or GPs
• Note that while immunologic testing can support a clini- with a special interest)
cal suspicion of active TB, negative testing does not exclude
the diagnosis and must not be interpreted in this way. Patients Requiring Management by the
Sexually Transmitted Infection • Those who have tested positive for an STI at the surgery
and wish to be managed in primary care or cannot attend
Travellers who have been sexually active should be offered GUM immediately
a sexual health screen (see next section). • Those who present as contacts of an STI and decline to
attend GUM for testing, treatment, and partner notifica-
Sexually Transmitted Infection and HIV tion advice after discussion and recommendation of this
course of action
GUIDANCE Contact Tracing
Guidelines on the diagnosis and management of sexually • This is usually best done by GUM, or at least with their
transmitted infection are available through the British
Association of Sexual Health and HIV (BASHH). Individual
support and advice.
guidelines are available for each disease mentioned over the • As a minimum, screening of all contacts of acute STIs over
next few pages. To see individual guidelines, visit https:// the last 3 months should be attempted. For syphilis and HIV
www.bashh.org/guidelines. there is no arbitrary limit, and partner notification will
depend on who is contactable, and the earliest likely time of
infection.
• If the patient is diagnosed with nonspecific urethritis (NSU),
General Approach chlamydia, syphilis, Trichomonas vaginalis, or gonorrhoea,
the partner should be treated even if tests are negative.
• Sexually transmitted infection is common and not limited
to conventional risk groups. It is important to consider Principles of Treatment
sexually transmitted infection (STI) in any patient and • Give appropriate antibiotics, bearing in mind any recent
not allow preconceived ideas about a patient’s lifestyle to travel history.
influence the approach. That is not to say that one should • Advise complete abstinence from all sexual contact until
not be aware of risk factors and recognize that certain both patient and partner are treated.
groups are at increased risk of infection. • Follow up after completion of antibiotics for compliance
• Questions should be open and not make unwarranted and possibly retesting (which is indicated only in preg-
assumptions about a person’s sexuality. Be self-critical in nancy or where symptoms persist).
ensuring that a nonjudgmental approach is taken at all times.
• Genitourinary medicine (GUM) clinics provide a variety of General Investigation in Primary Care
services, including screening, symptom-based assessment, • GUM clinics offer testing services for asymptomatic indi-
counselling, and contact tracing. Those with a diagnosis viduals. When an asymptomatic person presents to primary
of STI should be routinely referred to a GUM clinic for care seeking investigation, the local testing clinic should
assessment and management. be suggested. However, some may be unable to attend
• Testing for HIV should be a routine part of practice in GUM services and investigations should be offered in
primary care. Primary care studies have found a missed primary care.
opportunity for HIV testing in up to 60% of patients • Take a sexual history focusing on the last two partners
presenting with advanced HIV in the United Kingdom. and all partners in the last 3 months.
• Asymptomatic individuals do not require clinical examina-
Patients Requiring Investigation by the tion.
General Practitioner • A basic asymptomatic screen includes the following inves-
• Those who are eligible for a local or national screening tigations:
programme (e.g., for chlamydia) • An appropriate genital sample for chlamydia and gon-
• Those who require treatment urgently and cannot attend orrhoea
GUM immediately (e.g., symptomatic pelvic inflamma- Men: first pass urine for PCR
tory disease out of hours) Women: self-taken vulvovaginal swab for PCR
• Those who are unwilling or unable to attend a GUM Microscopy and culture are not required for asymp-
clinic after discussion/recommendation and require testing tomatic patients
for STIs or HIV • Serology for HIV and syphilis
• Those presenting with a possible STI-related problem in • Additional testing should be offered for those requiring
a primary care setting which has training and experience additional investigation:
• MSM and women reporting receptive oral intercourse Monotherapy treatment should not be given as it is asso-
should have a pharyngeal swab for chlamydia and ciated with the development of resistance, and quinolones
gonorrhoea PCR. should not be used.
• Those reporting receptive anal intercourse should have • Partner notification, testing, and treatment are important
a rectal swab for chlamydia and gonorrhoea PCR. to prevent reinfection and onward transmission.
• Consider testing for hepatitis B and C for those at risk. • Follow-up with test of cure is important in view of reports
of treatment failure.
How to Take Samples
Samples for chlamydia and gonorrhoea are usually analysed Chlamydia Infection
using PCR as these organisms are difficult to grow in routine • The majority of infections with Chlamydia trachomatis
culture. Specialist sample collection containers exist and are clinically inapparent. When symptoms do exist they
should be used for these samples. Most laboratories will not are often mild. However, infection can cause severe symp-
accept samples except in the recommended collection con- toms and impacts on female fertility. Therefore, it is
tainers. Ensure to check what is needed locally. important to diagnose and treat whenever possible.
• Of sexually active young people 3% to 7% are infected.
• Two-thirds of partners of cases will also be infected.
First pass • Ask patient to urinate into a plain • Symptoms when they occur:
urine collection bottle; this may need to • Men: dysuria and urethral discharge
be transferred into a PCR • Complications: reactive arthritis and epididymoorchitis
collection container depending on • Women: vaginal discharge, postcoital bleeding and
local laboratory requirements. dyspareunia, abdominal pain
Self-taken • Ask the patient to insert the swab • Complications: pelvis inflammatory disease (PID),
vulvovaginal about 2 inches (5 cm) into the infertility, reactive arthritis, and perihepatitis
vagina and gently rotate it for • Since patients are usually asymptomatic, screening is the
10–30 s. most important method of case acquisition. Rectal and pha-
Pharyngeal • Swab the tonsils and posterior ryngeal infection also occur and are usually asymptomatic.
swab pharynx and remove the swab • Screening should be conducted when patients present for
without touching the mouth. sexual health screening and annually or on change of partner
Rectal swab • Insert the swab about 2 inches for all sexually active people under the age of 25.
(5 cm) into the rectum. Rotate the • Urethral and endocervical swabs are not required for
swab at least three times against diagnosis of chlamydia. First pass urine in men and self-
the rectal wall before removing. taken vulvovaginal swabs in women are much more accept-
able to patients and just as sensitive.
• Treatment is with azithromycin 1 g stat or doxycycline
Management of Specific Sexually 100 mg twice daily for 7 days.
Transmitted Infection • Note that doxycycline is contraindicated in pregnancy.
Azithromycin will be the best option for the majority of
See also Chapter 13, Women’s Health. patients in pregnancy but off licence (consult guidelines).
• Seek expert advice for extragenital infection.
Genital Gonorrhoea • Refer to GUM for partner notification unless this can
• Gonorrhoea represents a major public health threat. Labo- be appropriately managed within primary care. Ensure
ratory diagnoses of gonorrhoea have increased markedly that an explanation of the importance of attendance at
over the last 10 years. This has been accompanied by a steep GUM is given to patients who receive treatment within
rise in antibiotic resistance; and 2016 marked the first primary care.
reported case of treatment failure with combined ceftriaxone
and azithromycin therapy due to resistance in a patient in Lymphogranuloma Venereum
the United Kingdom. This is particularly frightening since • Lymphogranuloma Venereum (LGV) has emerged as an
there is no clear alternative treatment strategy if such increasingly common infection affecting almost exclusively
multidrug-resistant organisms become widespread. MSM. It is caused by invasive serovars of Chlamydia tracho-
• Gonorrhoea presents with genital discharge and is an matis. It causes severe proctitis in those with rectal infection
important cause of pelvic inflammatory disease. which can scar and mimic inflammatory bowel disease. It is
• Pharyngeal colonization is rarely symptomatic but is more important to consider the diagnosis in those with risk factors.
difficult to treat and is an important reservoir of infection.
• PCR is the mainstay of diagnosis although microscopy Nonspecific Urethritis
and culture are available in specialist clinics. • Urethritis in the absence of chlamydia and gonorrhoea
• Current treatment recommendation is to use ceftriaxone is relatively common. It is best assessed in a GUM clinic
500 mg intramuscularly and azithromycin 1 g orally. where microscopy of a urethral swab can be carried out.
• Common causes of NSU include Mycoplasma genitalium, • Imiquimod 5% cream is an immune response modifier
Trichomonas vaginalis, Candida sp., HSV, adenovirus. which can be used three times weekly for up to 16 weeks.
• Consider treatment with azithromycin 1 g stat or doxy- It should not be used in pregnancy.
cycline 100 mg twice daily.
• Partner notification and treatment is required. Both patient Keratinized Warts
and partner should abstain from sex until treatment. • Use ablative therapy such as cryotherapy which is avail-
able at GUM clinics.
Trichomonas Vaginalis
• Trichomonas vaginalis (TV) infection is almost exclusively Herpes Genitalis
sexually transmitted, through urethral or vaginal inocula- • Genital herpes may be due to infection with either HSV-1
tion. It is sometimes diagnosed on cervical cytology, where or HSV-2.
there is a false positive rate of about 30%, so tests should • Many patients who carry HSV are asymptomatic but
be repeated. shedding of virus can result in infection of sexual partners
• Symptoms are of vaginal discharge in women and urethritis even in the absence of visible lesions.
in men. • A significant proportion of genital HSV is thought to be
• Diagnosis is usually made on culture or vaginal or urethral acquired through orogenital contact and so patients may
swabs although PCR is available in some areas. not consider themselves sexually active.
• Trichomonas infection is associated with other STIs, and • Autoinnoculation of the genital area from another site
full screening should be undertaken. (e.g., the lips) may also occur during primary episodes.
• Metronidazole 2 g orally stat, or metronidazole 400 mg • It is useful to confirm the diagnosis, even in clinically
twice daily for 5 to 7 days, will clear 95% of infections. The certain cases by sending a swab to virology for PCR. This
stat dose is as effective as the 7-day course but is more likely can also provide the HSV type causing infection which
to be associated with adverse effects (Smith et al., 2014). has implications for prognosis.
Test of cure is required only if symptoms persist, in which • Partner notification can be tricky, since HSV is very
case the patient should attend GUM. Alcohol should be common and may present many years after initial infec-
avoided during and for 48 hours after treatment. tion. Seek advice from GUM.
• Recent and current partners should be treated for TV
and screened for STIs. First Symptomatic Episode
• Note that the first symptomatic episode does not occur
Anogenital Warts at the time of primary infection in the majority of cases.
• The majority of patients with anogenital warts have mild This is important since source of infection is an important
symptoms or cosmetic concerns only. However, the psy- concern for many patients.
chological impact should not be underestimated. • Analgesia is the most important treatment modality. Topical
• Since 2012 the vaccine used to prevent HPV-associated local analgesics (5% lidocaine ointment) occasionally cause
cancers has included components that give protection sensitization but can be useful. Saline bathing is also
against the common viral causes of anogenital warts. useful for some, particularly if there is significant difficulty
However, this vaccine is usually only available to women. passing urine.
• Many carriers of HPV have no visible lesions. • Antiviral therapy with aciclovir 200 mg five times daily,
• Condom use may reduce transmission of warts to unin- valaciclovir 500 mg twice daily, or famciclovir 125 mg
fected partners. Their use with regular partners, however, twice daily for 5 days should be offered if:
has not been shown to affect the outcome of treatment • within 5 days of onset of symptoms; or
in patients with visible warts. • new lesions are continuing to form; or
• Perianal warts are associated with anal sex (although they • there are significant systemic symptoms.
can occur without) and should suggest the need for ano- • Patients with urinary retention, meningism, or severe
rectal samples for other STIs, and GUM referral. systemic symptoms should be admitted to hospital.
• None of the existing treatments is satisfactory, and all • Discuss with patients:
have high recurrence rates. Patients should be made aware • asymptomatic shedding and implications for relation-
of this. The evidence base for distinguishing first and ships; and
second line treatments is weak. • the importance of informing obstetric team in case of
future pregnancy.
• Prescribe podophyllotoxin 0.5% (twice daily for 3 days, Recurrences
repeated weekly for up to 4 weeks). Patients using podo- • In most patients recurrences cause minor, short-lived
phyllotoxin at home must take great care to follow the symptoms.
instructions, to avoid chemical burns. It should not be • Recurrences should be managed using one of three strategies:
used in pregnancy, and is not licensed for extragenital • Supportive: Use the symptom management strategies
lesions, such as anal warts. outlined in this chapter.
• Standby treatment: Prescribe patients antivirals to store at • Working with local HIV services to deliver care to
home and take as soon as possible after onset of symptoms people living with HIV (e.g., when a patient known to
(aciclovir 800 mg three times daily for 2 days, famciclovir have HIV attends primary care with a symptom that
1 g twice daily for 1 day, valaciclovir 500 mg twice daily would normally be dealt with within primary care)
for 3 days, note the shorter duration of therapy).
• Suppressive therapy: Usually considered when the Human Immunodeficiency Virus Testing
patient is experiencing six or more recurrences per
GUIDANCE
year. Treat with aciclovir 400 mg twice daily, aciclovir
200 mg four times daily, famciclovir 250 mg twice British HIV Association testing guidelines provide a
daily, or valaciclovir 500 mg once daily. Treatment comprehensive guide to HIV testing in the United Kingdom
(http://www.bhiva.org/HIV-testing-guidelines.aspx). Note,
should be discontinued after a year to reassess ongoing however, that recommendations on the window period
need for treatment. (PHE, 2015b) have been updated since these guidelines (also
see upcoming discussion).
Syphilis
• Like other STIs, syphilis is becoming increasingly common
in the United Kingdom. Cases have risen steadily over the
last 10 years principally among MSM although cases are • In 2015, around 103,700 people were estimated to be
also seen outside traditional risk groups. Early syphilis pre- living with HIV infection with 18,100 unaware of their
sents with a painless ulcer at the site of initial infection. infection (British HIV Association, 2008).
Secondary syphilis presents with rash while late stage syphilis • Among those already infected with HIV, those without
may be asymptomatic or present with a wide variety of apparent risk factors—especially men—are most likely
nonspecific symptoms which make diagnosis difficult. to be undiagnosed.
• Genital ulcers should be swabbed for syphilis PCR. • Early diagnosis of HIV has two principle benefits:
Ground-glass microscopy is a point-of-care test that can • Permitting early treatment, since early treatment of
immediately confirm the diagnosis but is limited to spe- asymptomatic patients has been associated with long-
cialist clinics. term health benefits and those who are diagnosed with
• All patients presenting for a sexual health screen should advanced disease are much more likely to die and suffer
have syphilis serology carried out. It should be noted that chronic ill health than those diagnosed with early
serology can take up to 3 months after infection to become stage disease.
positive and so it should be repeated if appropriate. • Preventing further infections, as people are much less
• Management of syphilis requires specialist expertise and likely to transmit infection to others once they are
should be managed through GUM clinics. aware of their infection.
• HIV testing should not be limited to specialists. All GPs
Human Immunodeficiency Virus Infection should feel comfortable offering an HIV test.
• Advances in the treatment of HIV have revolutionized prog- • Modern fourth generation HIV tests are able to detect
nosis and quality of life for patients over the last 20 years. both HIV antigen and antibody. This means that it is
• Highly active antiretroviral therapy (HAART), which rare for a patient with symptoms of HIV seroconversion
involves the use of drug combinations to control viral to have a negative HIV test although testing should be
replication, has proved effective in generating prolonged repeated after 7 days in those in whom HIV seroconver-
viral suppression in the vast majority of patients. Most sion is strongly suspected who test negative. However,
patients are now able to take one of a number of fixed-dose the window period is still important for asymptomatic
combinations involving one tablet taken once per day. patients who require repeat testing.
• Viral suppression allows recovery of the immune system • Antigen/antibody (fourth generation) testing should
and even patients who have presented with advanced be offered by all laboratories performing testing on
disease are usually returned to good health. whole blood samples.
• Life expectancy has dramatically improved and cause of death • Other tests (e.g., point-of-care tests or dried blood
in developed countries has evolved with acquired immu- spots) are not as sensitive and are therefore more likely
nodeficiency syndrome (AIDS) accounting for only one in to yield a false negative result in early HIV.
five deaths (BASHH/EAGA, 2014). Important causes of • If a fourth generation test is used then a negative test
non-AIDS deaths are similar to the general population with 4 weeks after the potential exposure is highly likely to
non-AIDS cancers and cardiovascular diseases dominating. exclude HIV infection. A further test at 8 weeks fol-
There remains a significant excess of liver disease–related lowing exposure is only required if the risk of HIV
deaths among people living with HIV. transmission is thought to be particularly high.
• There are two principle roles for primary care in the • Note that the window period quoted is shorter than
management of HIV infection: previously advised in the 2008 guidelines.
• Identifying people living with undiagnosed HIV infec- • Patients should not be asked to wait 4 weeks after an
tion through appropriate use of HIV testing exposure to test for HIV if they are concerned. Patients
should always be offered an HIV test if they are TABLE Conditions Encountered in Primary Care
concerned. 17.5 Which Should Prompt the Offer of a Human
• Note that this advice regarding the window period Immunodeficiency Virus Test (Whittle, 2008;
only applies to HIV. The window period in hepatitis DoH, 2008)
B and C is longer (6 months).
Respiratory Bacterial pneumonia
• HIV testing should be offered to the following people:
• Those attending specialist services in which universal Neurology Peripheral neuropathy
testing is appropriate (e.g., drug dependency services, Dementia
antenatal services, blood donors) Dermatology Severe or difficult to treat seborrheic
• Those registering with a GP in areas where the HIV dermatitis or psoriasis
prevalence in the local population is relatively high Severe or recurrent herpes
zoster
(>2/1000) as part of a locally arranged policy
• Asymptomatic individuals who have a risk factor for Gastroenterology Oral candida or hairy leukoplakia
HIV infection (people at ongoing risk should be tested Chronic, unexplained diarrhoea
Unexplained weight loss
annually or more frequently if appropriate): Salmonella, shigella, or
i. Those diagnosed with a STI campylobacter
ii. Sexual partners of those living with HIV Hepatitis B or C infection
iii. Men who have sex with men and female sexual Gynaecology Cervical intraepithelial neoplasia or
contacts of MSM invasive cancer
iv. People who inject drugs Vaginal intraepithelial neoplasia
v. Those from a country of high HIV prevalence Haematology Any unexplained blood dyscrasia
(>1%) and anyone reporting sexual contact with
a person from such a country. ENT Unexplained lymphadenopathy
Chronic parotitis
• Those with indicator illnesses, suggesting an increased
chance of HIV infection, including all those with Other Mononucleosis-like syndrome
symptoms which may be directly attributable to HIV Pyrexia of unknown cause
Unexplained lymphadenopathy
infection (Table 17.5) Any sexually transmitted infection
Practicalities of Testing Conditions highlighted in bold are those commonly identified as missed
opportunities for testing in patients who subsequently were diagnosed
• In the past, HIV testing has been regarded as different with advanced infection.
from other blood tests for serious medical conditions.
However, the prognosis in HIV infection is now very
much improved and testing should be normalized.
• Written consent is not required in the United Kingdom • A negative test has no implications for insurance since
and a prolonged pretest discussion is not necessary for the industry code of practice prohibits asking about this
the majority of patients. information. However, a positive test would have impli-
• Pretest discussion should involve three key features: cations for insurance like any other chronic medical
• An explanation of why the test is being offered (e.g., condition.
that HIV is a possible cause of unexplained lymph- • There have been a number of prosecutions in the United
adenopathy or that HIV is more common among Kingdom relating to the transmission of HIV and
people who inject drugs). some people are concerned about the possible forensic
• An explanation of the benefits of testing. This will usually implications of a positive test. Such concerns should
involve a brief discussion of the excellent prognosis with not be a barrier to testing but should be dealt with
modern treatment and advantages of early treatment. sensitively. In general, it is very unlikely that persons
• Discussion of how the test results will be delivered. This who have no reason to believe they were infected with
will depend on the clinical context and the outcome of HIV would be prosecuted for passing on the infection
the discussion with the patient. It is not always necessary to a sexual partner; however, the law is incredibly
to communicate the result face to face but this should be complex and varies depending on which UK legal
the preferred option if there is a high pretest probability. jurisdiction is in force. Unless such concerns can be
• Some patients may have concerns about the consequences readily dealt with, expert advice should be sought.
of HIV testing: • Post test discussion following a negative test should involve
• HIV testing should be entirely voluntary and those two principal objectives:
being tested should have a reasonable assurance of • Offering health promotion advice: For individuals at
confidentiality. While those testing positive may choose ongoing risk this is often best achieved by referral to
to disclose their diagnosis to others, they are under specialist services.
no obligation to do so. Counselling relating to these • Advising on the need for repeat testing in some cases
issues is undertaken sensitively in the HIV clinic. (e.g., those who remain within the window period
following a defined exposure [see discussion in this chronic manageable condition, there has been an increasing
chapter] or those who are ongoing risk who should role for primary care in the management of those with HIV
be retested periodically). infection. A small minority of patients now die of the com-
• Post test discussion following a positive test: plications of AIDS and the focus in stable, treated patients
• If unsure, speak to specialist services about the appro- has shifted to regarding HIV as a risk factor for illnesses
priate onward referral so that the plan is clear during such cardiovascular disease and non-AIDS related cancers.
the discussion. As life expectancy has improved, the HIV-infected cohort
• Approach the discussion like any other consultation has grown older and many patients now have HIV as one
involving the breaking of bad news. Avoid interrup- of a number of chronic medical conditions.
tions and give consideration to issues such as the need • The key clinical parameters for assessment of patients known
for a translator. to have HIV are the CD4+ lymphocyte count and HIV
• Non-specialists should acknowledge their lack of detailed viral load.
clinical knowledge and make clear that more detailed • CD4+ count is highly correlated with the risk of develop-
counselling will be provided in the specialist clinic. ing opportunistic infections. Patients with a CD4+ count
• Patients should not be pressured to disclose their status to greater than 250 are unlikely to develop opportunistic
sexual partners although clearly many will choose to do infections although some infections, such as pneumococcal
so at this stage. Partner notification requires experience disease and tuberculosis, are significantly increased regard-
and will be undertaken through specialist services. less of CD4+ count.
• Advice should be given about how HIV is transmitted • HIV viral load is an important parameter for those on
and patients should be advised to abstain from sex until antiretroviral therapy. Treatment guidelines now recom-
reviewed in specialist services mend considering treatment in all patients regardless of
• If a person does not attend to receive a positive result, CD4+ count and the goal of treatment is to suppress viral
attempts should be made to recall the patient. Where replication so that the viral load in blood becomes unde-
these attempts fail, local specialist services should be con- tectable (this is usually reported as a viral load less than
tacted for advice and support. the limit of detection of the assay—i.e., <40 copies/mL).
• Long-term follow-up studies have demonstrated that HIV
Primary Human Immunodeficiency Virus Infection does not progress in patients with a suppressed viral load
• HIV seroconversion illness usually occurs a few weeks and, indeed, the CD4+ count usually rises back toward
following infection and occurs in the majority of cases. the normal range on therapy.
Symptoms are nonspecific and therefore diagnosis is • It follows that a patient known to have HIV infection who
often not considered. However, detection at this stage attends primary care and is on treatment with a CD4+
allows early treatment and intervention to prevent onward count above 250 and an undetectable viral load is very
transmission. unlikely to be presenting with an HIV-related illness.
• Symptoms of HIV seroconversion illness include: • Antiretroviral therapy has improved significantly since
• fever and myalgia; the early days of HAART. Most patients are now managed
• pharyngitis and lymphadenopathy; with one fixed-dose combination tablet per day. However,
• oral ulceration; adverse effects occur and drug interactions remain a sig-
• headache; and nificant concern:
• maculopapular rash. • Some common drugs have critical interactions with
• Symptoms are often mild and may include a minority of some antiretrovirals. It is the responsibility of the pre-
the symptoms listed. They resolve spontaneously after a scriber of any new drug to check whether significant
few weeks. interactions exist.
• It is important that HIV is added to the differential diag- • Interactions can be life threatening and involve everyday
nosis of any mononucleosis-like illness and HIV testing drugs (e.g., the interaction of some corticosteroids,
considered. This is particularly important if the Monospot including inhaled corticosteroids, with protease inhibi-
is negative but cases of Monospot positive HIV serocon- tors resulting in iatrogenic Cushing syndrome).
version have been occasionally reported. • The University of Liverpool operates an online service
• Occasionally, a fourth generation HIV test is negative to check for drug interactions and is regularly updated
shortly after the onset of symptoms. If acute HIV infec- (http://www.hiv-druginteractions.org; a smartphone
tion is strongly suspected, this should be discussed with app is also available).
a specialist as repeat testing or testing for HIV RNA may • Most HIV clinics have a specialist pharmacist who
be considered. may be consulted for advice.
Management of Patients Living With Human Routine Care

Immunodeficiency Virus • Those with HIV should be encouraged to attend routine
• As the management of HIV has developed and the illness cancer screening. Cervical cancer screening should be
has evolved from one which dramatically shortens life to a offered annually.
• Inactivated vaccines can be given as usual, although vac- toxoplasmosis, tuberculosis, lymphoma, and cryptococ-
cines may be less effective when given to those with HIV, cal meningitis. Note that meningism is often absent
particularly if the CD4+ count is low. Hepatitis B vaccine in these cases.
is indicated for all with HIV. • Diarrhoea and weight loss are common and may occur
• Live vaccines, including YF, can often be given to those in the absence of infection. Possible causes include
with a high CD4+ count but this should only be done bacterial, protozoal, or viral infection. Send faeces for
following expert advice. culture and discuss with a specialist if not improving.
• Family planning should be actively undertaken and women
who seek advice about pregnancy should be advised to Possible Exposure to Bloodborne Viruses
discuss this with the HIV clinic prior to conception. There are two ways in which people may present concerned
Effective treatment has reduced the risk of mother to about having acquired HIV or other bloodborne viruses.
child transmission to less than 1%. • Following percutaneous or mucosal exposure to potentially
• Risk reduction in relation to sexual partners needs to be infected body fluids: Most such exposures are occupational
regularly reviewed. Transmission of HIV to uninfected but members of the public will occasionally be exposed
partners, or transmission of resistant strains, is possible (e.g., through a needlestick from a discarded needle)
and safe sex should be advised. However, the risk of trans- (British Association for Sexual Health and HIV, 2015)
mission is thought to be extremely low when on treatment but see the subsequent notifications online.
with a persistently suppressed HIV viral load. Some couples • After any exposure to blood (whether the source patient
may make an informed decision to have unprotected is known to be HIV or hepatitis B or C positive),
sex—they should be advised to seek specialist advice from wash the wound liberally with soap and water, but do
the HIV clinic. not scrub. Antiseptics should not be used.
• The Department of Health advises that all healthcare
Patients With Low CD4+ Counts staff, including those in primary care, should be edu-
• Patients with CD4+ counts below 250 cells/mm3 are cated on the risks from possible exposures and be made
at increased risk of opportunistic infections and AIDS- aware of how to report an injury. A local policy on
related cancers such as lymphoma, Kaposi sarcoma, and how to obtain advice will be available and will provide
cervical cancer. information on how to obtain advice.
• The risk of complications increases as the CD4+ falls • HIV post-exposure prophylaxis starter packs are usually
further and patients with a CD4+ count below 50 cells/ available through the emergency department.
mm3 are at very high risk. • Take baseline blood for storage from the injured party
• Patients with a CD4+ count below 200 cells/mm3 are unless immediate testing for bloodborne viruses is
offered prophylaxis against pneumocystis pneumonia indicated.
(PCP), usually with co-trimoxazole if tolerated. Those • If the risk is assessed as potentially significant, anti-
with a CD4+ count below 50 cells/mm3 are offered pro- retroviral drugs should be commenced as soon as pos-
phylaxis against Mycobacterium avium, usually with weekly sible, preferably within 1 hour. Medication should be
azithromycin. continued for a total of 4 weeks if subsequent risk
• Management of patients with a low CD4+ count is very assessment confirms this is appropriate. The contents
complex and a specialist should be consulted for advice of starter packs are under constant review.
on management. • See the section on hepatitis B for information on post-
• Particular symptoms to be aware of are as follows: exposure prophylaxis.
• Oral candida is common and may be accompanied by • If the source patient is of unknown status, he or she
oesophageal candida. Systemic antifungal therapy is should routinely be approached (by someone other
usually required and fluconazole is the usual first choice. than the exposed patient) to ask for consent for testing.
High doses of fluconazole (≥100 mg) may be required, • Arrange follow-up by Occupational Health, the GUM
particularly for oesophagitis. Patients not responding department, or another appropriate department, for
to treatment within a few days should have an oral the exposed party, to consider testing for HIV and
swab sent to the laboratory for resistance testing and hepatitis B and C in confidence at a later stage.
be discussed with a specialist. • Generally speaking, incidents involving members of
• Cough/shortness of breath: PCP is a particular concern the public injured by a discarded needle in a public
and may present with a clear chest and normal chest place should not receive HIV post-exposure prophylaxis
x-ray (CXR). Exercise desaturation is a helpful sign. or hepatitis B immunoglobulin as the risk of transmis-
Suspected PCP requires urgent specialist assessment. sion is extremely low. They should be offered hepatitis
Patients are also at increased risk of community acquired B vaccination and follow-up. It is, however, reasonable
pneumonia and tuberculosis. to discuss such cases with a specialist.
• Headaches or other neurologic symptoms: Immuno- • Following sexual exposure: it is important to be aware
suppressed patients with a low CD4+ count are at that postexposure prophylaxis is available to those who
risk of a range of neurologic complications, including may have been exposed through sexual contact. HIV
postexposure prophylaxis is available up to 72 hours Forna & Gulmezoglu (2000).

after exposure (British Association for Sexual Health and Kamar, N., Dalton, H. R., Abravanel, F., & Izopet, J. (2014). Hepa-
HIV, 2015). titis E virus infection. Clinical Microbiology Reviews, 27, 116–138.
• There should be clear local protocols to seek urgent Kantele, A., Lääveri, T., Mero, S., et al. (2015). Antimicrobials increase
travelers’ risk of colonization by extended-spectrum betalactamase-
assessment for those who have been sexually assaulted
producing Enterobacteriaceae. Clinical Infectious Diseases: An Official
and victims of sexual assault should receive specialist Publication of the Infectious Diseases Society of America, 60, 837–846.
assessment. Leder, K., Torresi, J., Libman, M. D., et al. (2013). GeoSentinel sur-
• Other cases should be discussed urgently with the local veillance of illness in returned travelers, 2007–2011. Annals of
sexual health clinic. Such cases should still be referred Internal Medicine, 158, 456–468.
to GUM even if there is no indication for post-exposure McGill, F., Heyderman, R. S., Michael, B. D., et al. (2016). The UK
prophylaxis as a sexual health screen and health pro- Joint Specialists Societies guideline on the diagnosis and management
motion intervention will be indicated. of acute meningitis and meningococcal sepsis in immunocompetent
• There is evidence that pre-exposure prophylaxis of adults. Journal of Infection, 72, 405–438.
individuals at risk with daily antiretrovirals reduces Nathwani, D., Morgan, M., Masterton, R. G., et al. (2008). Guidelines
the risk of HIV acquisition. At the time of writing, for UK practice for the diagnosis and management of methicillin-
resistant Staphylococcus aureus (MRSA) infections presenting in
such treatment is not available on the National Health
the community. The Journal of Antimicrobial Chemotherapy, 61,
Service, but some people obtain it privately. 976–994.
National Institute for Health and Care Excellence. (2016). Tuberculosis
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18
Psychiatric Problems
DOMINIQUE THOMPSON
Depression Self-Harm
Presenting Complaints Acute Psychotic Disorders
Differential Diagnosis
Initial Assessment
Management of Depression
When There Is a Risk of Violence
Referral
Specific Treatment of Minor Depression (or Mild Depression Drug Treatment
on the ICD-10 Criteria) Precautions When Visiting a Disturbed Patient at Home
Specific Treatment of Major Depression (or Moderate- Referral and Admission
Severe Depression on the ICD-10 Criteria)
Compulsory Admission
Factors That Will Influence the Choice of an Antidepressant
Management of a Poor Response to Drug Therapy Indications for Use of the Mental Health Act (England and
Stopping Maintenance Therapy and Managing Wales) 1983
Discontinuation Reactions The Assessment Under the Act
How to Distinguish an Antidepressant Discontinuation Which Section of the Act Should Be Used?
Reaction From a Return of the Underlying Disorder How Desperate Is the Situation?
Cognitive Behavioural Therapy/Counselling/Other Further Clarifications
Psychotherapies Other Sections of Value
Prevention of Recurrence
Regulations for Scotland and Northern Ireland
Postnatal Depression
If Depression Is Found Chronic Schizophrenia
Structured Care
Suicide and Suicidal Risk Referral
Management of Suicidal Patients General Management
Low Risk Drug Treatment
High Risk Relapse
Follow-Up After Attempted Suicide Medication Review
Helping Those Bereaved by Suicide Side Effects of Medication
Stress Reactions Psychotherapy
Management Annual Review
Posttraumatic Stress Disorder Carers’ Needs
Management Bipolar Affective Disorder
Insomnia General Management
Simple Rules to Reduce Insomnia During Episodes of Depression
Anxiety Disorders During Episodes of Mania or Hypomania
Generalized Anxiety Medication
Management of a Crisis Prevention of Relapse
Long-Term Treatment Dealing With Violence
Practical Details When Prescribing SSRI for Anxiety
Eating Disorders
Special Situations
Panic Disorder Assessment in Primary Care
First Aid for a Panic Attack—Instructions for the Patient Investigation and Monitoring
Social Anxiety/Social Phobia Medication
Phobias Referral
Specific Phobias
Obsessive-Compulsive Disorder
313
Depression • If the answer to either question is yes, confirm the diag-

nosis of depression using the DSM-V criteria as follows.
• About 25% to 40% of general practitioner (GP) consulta- Used by GPs this is highly specific (Van Weel-Baumgarten
tions have a significant psychological component (Goldberg et al., 2000). However, if the score conflicts with clinical
& Lecrubier, 1995). Of these only about 5% are referred judgment, treat it as a checklist only and act on your
to specialist services. clinical judgment.
• Recent research has shown how specific questions help • A major depressive illness exists if the patient has low mood
to elicit crucial information about the patient’s mental or diminished interest or pleasure for at least 2 weeks,
state. occurring most of the day or nearly every day, and a
• Qualitative research has shown the importance of the positive score on at least five of the following:
patient’s relationship with the GP in such situations. • Depressed mood
Patients value an empathetic, interested doctor with whom • Loss of interest or pleasure
they have a continuing relationship (Buszewicz et al, 2006). • Change in weight or altered appetite almost every day
• Disturbed sleep
• Agitation or slowing of movement or speech
GUIDELINES • Fatigue or loss of self-energy
National Institute for Health and Care Excellence. (2016). • Guilt or low esteem
Depression in adults: Recognition and management. NICE • Poor concentration
clincial guideline 90. www.nice.org.uk. • Recurrent suicidal thoughts or acts
Anderson, I. M., Ferrier, I. N., Baldwin, R. C., et al. (2008). • Minor depression is characterized by three or four of the
Evidence-based guidelines for treating depressive disorders
with antidepressants: A revision of the 2000 British
above. The evidence that it responds to antidepressant
Association for Psychopharmacology guidelines. Journal of drugs is poor, unless it lasts for at least 2 years, when it
Psychopharmacology / British Association for may be called dysthymia, which may respond to drugs
Psychopharmacology, 22, 343–396. (Barrett et al., 2001).
Cautions
• The scoring system cannot be used in patients reacting
• GPs fail to diagnose up to half of their patients with a appropriately to a life crisis or who are schizophrenic.
major depressive illness, and often fail to treat adequately • To score as positive, the symptoms should indicate sig-
those whom they do recognize (Anderson, Nutt, & Deakin, nificant distress or impairment in functioning.
2000; Freeling et al., 1985). • No scoring system can be totally accurate. Modify the
• Depression in people from the African–Caribbean, Asian, result in light of the patient’s circumstances. A past or
refugee, and asylum-seeking communities is often over- family history of major depression, for instance, would
looked, although the prevalence is 60% higher than in increase the chance of major depression in a patient whose
the white population (Department of Health, 1999). score does not reach that threshold.
People from black and minority ethnic communities are • The distinction between major and minor depression on
much less likely to be referred to psychological therapies the DSM-V criteria should not be confused with mild,
(Department of Health, 1999). moderate, and severe depression on the ICD-10 criteria
• Those patients whose depression is most likely to be missed (World Health Organisation [WHO], 2003), where the
are those with somatic complaints or with physical illness, scoring is out of 10, not 9, and includes an assessment
those with long-standing depression, those who do not of functioning rather than a more rigid count of symp-
look depressed, and those who do not realize they are toms. This leads to the imperfect correlation between the
depressed (Freeling et al., 1985; Gill & Hatcher, 2002). two scores shown in Table 18.1.
• Depression is often accompanied and masked by anxiety
(Goldberg & Bridges, 1987), yet treatment of the anxiety Differential Diagnosis
alone is insufficient and may appear to worsen the • Schizophrenia. Look for evidence of schizophrenia, which
depression. may present with depression. If delusions or hallucina-
tions are also present, depression should not be the initial
Presenting Complaints diagnosis.
• Screen for depression in patients with low mood, or with • Bipolar affective disorder. Ask about a past history of
a past history, or who are at risk because of physical a manic episode or hypomania. The management of
illness or other mental illnesses such as anxiety or demen- bipolar affective disorder is different (see page 333).
tia. Ask two specific questions: During the past month A study in US primary care looked at the prevalence
have you felt: of a current diagnosis of depression plus a positive
• low, depressed, or hopeless? screen for bipolar affective disorder. In the adult primary
• little interest or pleasure in doing things? (Whooley care population, 4% had both but the GPs who were
et al., 1997) managing the depression did not detect concurrent
CHAPTER 18 Psychiatric Problems 315
TABLE
18.1 Depression: Definitions and Scoring
ICD-10 Definition ICD-10 Scores Equivalent DSM-V Definition

Mild depression 2–3. The patient is distressed but able to continue functioning Minor depression
Moderate depression 4 or more. The patient is likely to have great difficulty in Minor or major depression
continuing with normal activities
Severe depression Several symptoms are marked and distressing, typically loss of Major depression
self-esteem and ideas of worthlessness and guilt. Suicidal
thoughts are common and a number of somatic symptoms
are usually present. Hallucinations or delusions also mean
that the episode is severe, regardless of other symptoms
bipolar affective disorder in any of these patients (Das • Follow-up: Explain the need for and frequency of
et al., 2005). follow-up.
• Iatrogenic cause. Check for drugs that can cause depression • Patient information: Provide one of the leaflets listed in
(e.g., antihypertensives, histamine type-2 [H2] blockers, the box on page 318 and give the patient details of sources
steroids, and beta-blockers). of information and support.
• Life events. Ask about recent life events such as recent
childbirth, bereavement, termination of pregnancy, loss Referral
of job, work stress, family illness, or divorce. They may Refer to a specialist mental health service if:
have triggered the depression. • psychotic features are present or the depression is severe;
• the history suggests a bipolar illness;
Management of Depression • there is a significant risk of suicide or severe neglect;
• The GP will manage 90% of depressed patients without • other forms of treatment are needed (e.g., cognitive behav-
referral to a specialized mental health service. The fol- ioural therapy) if they are not available in primary care;
lowing steps are common to almost all severities of • the patient is a child or adolescent with major depressive
depression. illness;
• Life events: Identify relevant life events which may have • there has been a poor response to an appropriate antide-
precipitated the illness and focus on small steps that might pressant in maximum dosage, with good compliance,
be taken to reduce their impact. taken for an adequate period of time;
• Explore the impact of the depression on behaviour and • there is social isolation, little family support, or poor
relationships at home and work. compliance.
• Explain the nature of the illness, its treatment. and good
prognosis. Specific Treatment of Minor Depression (or Mild
• Discuss the link between physical symptoms and mood. Depression on the ICD-10 Criteria)
• Encourage the patient not to make major or irreversible • Explain the possible options:
decisions about work or family until their mental state • Watchful waiting. The patient may choose to return
improves. in, say, 2 weeks without treatment other than the
• Exercise: Recommend exercise, to the same extent as for general measures described previously.
cardiovascular fitness, for at least 10 weeks, even (or espe- • Guided self-help. The patient works through a written
cially) on days when the patient feels like staying in bed or computer programme which is usually designed
(Dunn et al, 2005). High-dose exercise 3 days a week is along cognitive behavioural lines (see upcoming Infor-
likely to lead to remission in one in five depressed patients mation for Patients box). National Institute for Health
compared to controls. The depressed patient will have and Care Excellence (NICE) has approved a computer-
little motivation and will need supervision. Refer to an ized cognitive behavioural therapy (CBT) programme,
exercise referral scheme if one is available. “Beating the Blues,” for mild to moderate depression,
• Light therapy: Recommend it for seasonal affective disorder. suitable for those with no computer experience. If the
However, if using artificial lights, exposure to a bank of GP is unable to offer access to it using National Health
lights is needed for 1 hour a day, preferably in the morning. System (NHS) funding, the patient can access it, for
Similar benefit is seen with exposure to natural light, even a considerable fee, at www.beatingtheblues.co.uk.
if the sky is cloudy (Jorm, Christensen, Griffiths, & • Counselling or psychotherapy. There is evidence that
Rodgers, 2002). Exposure to daylight could usefully be brief CBT, problem-solving therapy, and other forms
combined with exercise. of counselling are beneficial.
• Discuss drug therapy and counselling (see upcoming • Explain that antidepressants are usually ineffective in minor
discussion). depression unless it has lasted for 2 years (i.e., dysthymia).
Any possible benefit is likely to be outweighed by the on an SSRI) and to check on compliance. A quarter of
adverse effects. However, a patient with a past history of patients either never cash in their prescription or only
major depression who presents with minor depression do so once (Boardman & Walters, 2009). If an SSRI is
may choose to take an antidepressant at this stage. causing agitation, a 2-week course of a benzodiazepine
is an alternative to stopping the drug. Slowly increase the
Specific Treatment of Major Depression time between consultations as the patient responds.
(or Moderate-Severe Depression on the • Response. Do not be disappointed by a partial response.
ICD-10 Criteria) Studies of published and unpublished studies combined
• Offer an antidepressant. The NICE guideline recommends show that the effect size of all antidepressants is small
a selective serotonin reuptake inhibitor (SSRI) because (number needed to treat [NNT] = 6) (Boardman &
they are as effective as tricyclics but with a 10% less Walters, 2009) and certainly less than the claims of manu-
chance of being discontinued because of adverse effects. facturers, based on published studies alone (Turner &
Cost-effectiveness studies, in which doctors’ time is costed Rosenthal, 2008).
as well as drug expenditure, favour SSRIs or are at least • Maintenance. Continue the drug for at least 6 months
neutral (Simon et al., 1999; Stewart, 1998). However, after remission. This reduces the relapse rate from 50%
both types of drug have considerable but different adverse to 20%. A reduction in dose should only be made if side
effects. Attempt to make a choice that is the best for that effects are a problem. Consider treatment for 1 to 2 years
patient (see upcoming discussion). if there has been a previous episode in the recent past or
• Explain to patients that: the patient is at high risk because of age, family history,
• even if their depression is a reaction to life events, they or other features (see upcoming discussion).
are as likely to benefit from an antidepressant as if it
was endogenous; Factors That Will Influence the Choice of
• they will not notice any improvement for the first 10 an Antidepressant
days to 3 weeks but that adverse effects are most likely • The elderly: Avoid highly anticholinergic drugs (e.g., ami-
to occur in this period and then lessen; triptyline, clomipramine, doxepin, imipramine, and
• antidepressants are not addictive. They will not develop maprotiline).
craving or tolerance, but there is the possibility of a • Young people: The Committee on Safety of Medicines (CSM)
discontinuation reaction especially if stopped abruptly; has concluded that the majority of SSRIs are contraindicated
• stopping the drug early increases the risk of relapse. in children and adolescents up to the age of 18 because of
All patients should take the drug for 6 months after poor efficacy and a possible increase in the risk of self-harm
recovery. Anyone with a recent previous episode of and suicide. Fluoxetine seems to be an exception to this.
major depression should take it for 2 years, as should The CSM also cautions that very young adults may be at
other people at high risk of relapse (e.g., the elderly) similar risk, even though such a risk has not been detected
(see upcoming discussion). in trials (CSM Expert Working Group, 2004).
• Prescribe a generic preparation. There is some evidence • Pregnancy: Treat only if the benefit outweighs the risks.
that venlafaxine is more effective than other antidepres- Consider seeking specialist advice before starting antide-
sants (Smith et al., 2002) but adverse effects may also be pressants or switching the antidepressant of a woman
greater (Cipriani, Geddes, & Barbui, 2007). One study who is already being treated for depression when she falls
suggests that the risk-benefit profile from 117 randomized pregnant. Evidence about the relative risks and benefits
controlled trials (RCTs) favours sertraline (Cipriani et al., of antidepressant use in pregnancy is inadequate making
2009). informed decision making difficult (McDonagh et al,
• Drug dosage: 2014). Between 3–8% of pregnant women in Europe
• If choosing a tricyclic, start at the equivalent of ami- currently take an antidepressant so experience of antide-
triptyline 75 mg daily and expect to increase to 150 mg pressant use in pregnancy is rapidly growing and untreated
daily. A much-criticized meta-analysis (Furukawa, depression is associated with the risks of preterm birth
McGuire, & Barbui, 2002) has suggested that the lower and low birth weight (Jarde et al, 2016). NICE clinical
doses may be adequate. Such studies, and their critics, knowledge summaries advise use of a tricyclic antidepres-
inevitably examine the mean response of a large number sant, SSRI, or SNRI after discussion of risks and benefits
of patients. In clinical practice what matters is the (NICE, 2018). Use tricyclics with caution; neonatal irri-
response of that individual. Assess the effect of the tability has been reported with imipramine.
lower dose and increase the dose monthly if the response • Prostatism or glaucoma: Avoid all anticholinergic drugs.
is inadequate. • Cardiac disease: Avoid tricyclics and venlafaxine; the best
• If choosing an SSRI start at the standard dose. Evidence evidence for use in ischaemic heart disease is for sertraline.
that subsequent dose increases are beneficial is poor • Suicide risk: There is no evidence that one class of drugs
(although higher doses are effective in other conditions). poses a greater risk of suicide (Cipriani, Barbui, & Geddes,
• Follow-up. See the patient 1 week after starting a drug, 2005) but intuitively doctors will avoid the more toxic
to discuss any adverse effects (e.g., agitation or akathisia tricyclics.
• Lethargy: Avoid sedative drugs (e.g., amitriptyline, clo- unclear. Estimates based on reports of adverse drug reac-
mipramine, dosulepin, doxepin, maprotiline, mianserin, tions suggest that it is uncommon (5% with paroxetine,
trazodone, and trimipramine). <1% with other SSRIs) (Price et al., 1996) but such reports
• Anxiety or insomnia: Use a sedative drug. rely on the reporting doctor making the diagnosis. A
• Obesity: Use an SSRI. retrospective analysis of patients discontinuing antidepres-
• Epilepsy: Avoid SSRIs. sants found that a reaction occurred in 31% who had
• Other drugs: All classes of antidepressants show consider- taken clomipramine, in 17% who had taken a short-acting
able interaction with other drugs, but any one interaction SSRI (paroxetine or fluvoxamine), and in only 1.5% who
rarely applies to all classes and may not be seen with all had taken a long-acting SSRI (fluoxetine or sertraline)
members of one class. Choose an antidepressant tailored (Coupland, Bell, & Potokar, 1996). Even if half of these
to the patient’s other medication. are due to a placebo effect (Haddad et al., 1998), the
study suggests that the reaction is more common with
Management of a Poor Response to Drug Therapy the shorter acting drugs.
• Check that the patient was taking the drug correctly. An • Explain that a discontinuation reaction is unlikely to feel
apparent relapse could be due to a discontinuation reac- like a recurrence of depression. Dizziness, paraesthesia,
tion in a patient who has omitted one or more doses. tremor, anxiety, nausea, and palpitations are the most
• Where there is evidence that an increased dose may be common symptoms. They occur within days of stopping
associated with an improved response, and there are no the drug and last for 10 days on average.
adverse effects, consider increasing the dose gradually, • If a discontinuation reaction occurs:
waiting 4 weeks each time before deciding that the response • A mild reaction: Explain what is happening and con-
is inadequate. tinue the planned reduction, if the patient agrees.
• If dosage increase is not a possibility, assess whether referral • A more severe reaction, either:
is needed or whether the patient’s condition allows the a. increase the dose to the last dose that gave no
trial of another drug. Make a choice, according to any reaction; or
adverse effects experienced with the first drug, between b. change the patient to a longer acting drug of the
another SSRI, a tricyclic (but not dosulepin), and a same class; and
member of a different class (e.g., moclobemide, mir- c. prepare to tail off over a longer period (e.g., 3–6
tazapine, or reboxetine). Because it is a Reversible Inhibi- months).
tor of Monoamine Oxidase A (RIMA), moclobemide • Review patients 4 weeks after finally stopping the drug
cannot be started until the previous antidepressant to ensure they remain well. Advise them to return at the
has washed out of the system. For a tricyclic this is 1 earliest sign of a recurrence.
week but for fluoxetine it is 5 weeks. The STAR* D
study found that putting non-responders through four How to Distinguish an Antidepressant
steps of antidepressant treatment (either increased dosage Discontinuation Reaction From a Return of
or a change of drug) increased the rate of remission from the Underlying Disorder
37% with step 1 to 67% by step 4, in those who adhered • Its onset is within days (against weeks for a recurrence
to treatment (Rush et al., 2006). Against these impressive- of depression).
sounding results is the fact that dropout rates were high • It resolves with 24 hours of restarting the antidepressant.
and the percentage remitting fell with each step. • Although the patient may report mood disturbance
• Consider combining psychotherapy with drug treatment. (low mood, irritability, anxiety) there are likely to be somatic
Response is greater than with either alone, even if this is symptoms as well (numbness, paraesthesia, dizziness, head-
just because patients who also receive psychotherapy are ache, myalgia, fatigue, insomnia with vivid dreams).
more likely to take their drugs (Pampallona, Bollini, &
Tibaldi, 2004). Cognitive Behavioural Therapy/Counselling/
Other Psychotherapies
Stopping Maintenance Therapy and Managing • In patients at the mild to moderate end of the spectrum
Discontinuation Reactions of major depression, cognitive and interpersonal therapies
• Tail off, over 4 weeks, any antidepressant that has been can be as effective as antidepressants and may prevent
given for 8 weeks or more, to reduce the risk of a dis- relapse (Gloaguen et al., 1998). In a meta-analysis, anti-
continuation reaction (Haddad, Lejoyeux, & Young, 1998). depressants and psychotherapy both resulted in remission
A long-acting drug (e.g., fluoxetine or sertraline) may be at a mean of 16 weeks in 46% of patients against 24%
given at the same dose but every other day, then every of controls (Casacalenda, Perry, & Looper, 2002).
third day. A short-acting drug (e.g., paroxetine or citalo- • More patients would choose counselling than drugs if
pram) should be given daily at decreasing dosage. Tablets given the choice (Chilvers et al., 2001).
may be cut in half or a liquid form prescribed. • In major depression, a combination of antidepressants
• Warn the patient that a discontinuation reaction is pos- and cognitive behavioural therapy can be more effective
sible on stopping an antidepressant. The incidence is than either alone (Timonen & Liukkonen, 2008). Other
psychotherapies have not shown a benefit from being Postnatal Depression

combined with drugs.
• Most of the evidence for the benefit of psychological
GUIDELINES
therapies in depression lies with certain techniques only,
mainly cognitive behavioural, interpersonal, and problem- Scottish Intercollegiate Guidelines Network. (2012).
solving ones (Mynors-Wallis et al., 2000) and cannot be Management of perinatal mood disorders. SIGN guideline
127, 2002. www.sign.ac.uk.
assumed to exist for all forms of counselling (Department National Institute of Health and Care Excellence. (2014).
of Health, 2001). Antenatal and postnatal mental health: Clinical management
• A 6-year follow-up study suggests that a course of cogni- and service guidance. NICE clincial guideline 192, updated
tive behavioural therapy, in addition to drug therapy, 2017. www.nice.org.uk.
leads to fewer subsequent relapses than drug therapy alone
(40% vs. 90% in a study of 40 patients) (Fava, Ruini, &
Rafanelli, 2004).
Prevention of Recurrence
• The continuing nature of the condition and the benefit • Postnatal depression occurs in 10% to 15% of women
to be gained from early intervention argue strongly for in the first year after delivery, usually in the first 6 months.
depression to be managed as a chronic disease, with a The symptoms are almost always present at 6 weeks. It
systematic approach to follow-up by a dedicated team is distinct from the transient blues of the first 10 days,
following agreed protocols (Scott, 2006). and from a puerperal psychosis which is likely to need
• Of patients with a first episode of major depression, 75% admission.
have a recurrence within the next 10 years (Angst, 1997). • Most at risk are those with:
A second episode during the next 4 years requires full • previous psychological disturbance in pregnancy;
initial treatment, followed by psychiatric referral to con- • poor social support;
sider prophylaxis with long-term antidepressant medica- • a poor marital relationship;
tion. In the first 3 years this will reduce the rate of • recent stressful events;
recurrence from 41% to 18%. Only slightly more patients • an episode of the baby blues.
withdraw from treatment on an antidepressant than on • The strongest risk factors for puerperal psychosis are a
placebo (odds ratio [OR] 1.3) (95% confidence interval personal or family history of an affective psychosis. A
[CI] 1.07–1.59) (Geddes et al., 2003). woman who has one episode of puerperal psychosis has
• Long-term prophylaxis after a single episode may be a 25% to 57% risk of a recurrence in a subsequent preg-
appropriate in the following because their risk of recur- nancy. The risk of non-puerperal affective psychosis at
rence is even higher: some stage is even greater.
• The elderly • Every practice should be aware of the need to identify
• Those with a first-degree relative with bipolar disorder at-risk patients during the antenatal period and should
or recurrent major depression ensure that they receive more intensive help after birth.
• As a minimum, consider a 3-monthly telephone review Risk factors should be identified during pregnancy and
by a doctor or nurse over the 2 years after treatment is the primary care team needs to decide:
stopped. In a US study it increased remission over 2 years • what extra care this group should receive; and
by 33% (95%CI 7–46) (Rost et al., 2002). • who is responsible for identifying this condition as
early as possible.
INFORMATION FOR PATIENTS • Patients often do not realize that they are depressed, and
doctors recognize it even less frequently. Women often
The Samaritans. Tel. 116 123; email: jo@samaritans.org; present with a feeling of not coping rather than with
website: www.samaritans.org
The Mental Health Foundation has fact sheets for patients classic symptoms of depression.
as well as other resources at www.mentalhealth.org.uk. • The Scottish Intercollegiate Guidelines Network (SIGN)
Royal College of Psychiatry leaflet “Help Is at Hand: guideline recommends that every woman should be
Depression” is available at www.rcpsych.ac.uk/ screened for depression at 6 weeks and 3 months post-
help_is_at_hand. partum. The Edinburgh Postnatal Depression Scale (EPDS)
Books (Cox, Holden, & Sagovsky, 1987) may be administered
Butler, G., & Hope, A. (2007). Managing your mind: The by a trained professional (see Appendix 20) as part of
mental fitness guide. Oxford: OUP. that screening process but with the understanding that
Rowe, D. (2003). Depression: The way out of your prison. it is a screening, not a diagnostic, tool. Clinical assessment
London: Brunner-Routledge. is needed to establish the diagnosis.
Burns, D. D. (2005). Feeling good: The new mood • If the EPDS is not available, ask four simple questions:
therapy. London: Avon Books.
• How are you feeling?
• How are you sleeping?
• How are you eating? • There is no evidence that SSRIs increase the risk of suicide
• Are you enjoying the baby? in adults, though they appear to do so in children and
adolescents. On the contrary, it seems likely that effective
If Depression Is Found treatment of depression offers the most hope of reducing
• Check thyroid function tests (TFTs) in those complaining the risk of suicide. Even in adolescents the risk of suicide
mainly of tiredness. is greater in the month before starting antidepressants
• Counsel. Simple, nondirective counselling by health visi- than in the first month on therapy (Brent, 2007).
tors, weekly for 8 weeks, doubles the recovery rate (Holden, • Suicide is uncommon even in major depressive illness.
Sagovsky, & Cox, 1989). One study found that only 4% of patients admitted
• Explain to the patient that she is ill: it is not her fault because of depression killed themselves (Bostwick & Pan-
and does not mean that she is a poor mother. kratz, 2000). Furthermore, that 4% is hard to detect,
• Literature. Recommend one of the sources of information though an attempt should be made.
in the box Patient Organisations or print the information • In all patients with depression, assess the risk. For instance,
for the patient. ask a series of questions, only stopping when the answer
• Antidepressants. Offer drug treatment on the same basis is no:
that you would offer it to a non-puerperal patient (i.e., • Have you thought how nice it would be if you did
if major depressive illness is present). SIGN recommends not wake up one morning?
that tricyclics (other than doxepin) or SSRIs (although • Have you thought of killing yourself?
avoid fluoxetine, escitalopram, and citalopram if possible) • Have you decided how to do it?
may be used and the patient may continue to breastfeed. • Have you decided when to do it?
This goes beyond the manufacturers’ advice and indeed • Judge the risk according to the following risk factors:
a mother should be told that the SSRIs, especially, will • History of previous attempts, especially if determined or
be excreted in the breast milk but that there is no evidence violent methods were used, or if a suicide note was left
that this is harmful to the baby. However, the baby may • Intense feelings of hopelessness and worthlessness
experience a discontinuation reaction. Use a long-acting • Major mental illness, including depression
drug and tail off breastfeeding (or the drug if still breast- • Suicidal ideation or evidence of planning (e.g., if the
feeding) slowly. patient has decided when to do it, and if a sudden
• Consider referral if a multidisciplinary mental health team and infallible method has been chosen) (Boardman &
is able to offer more than the primary healthcare team. Walters, 2009)
• Refer immediately for urgent psychiatric assessment any • Chronic physical illness or in much pain
woman with ideas of suicide or of harming the baby or • Recent bereavement or other significant loss, includ-
with symptoms of puerperal psychosis. ing job
• Males. The male to female ratio for suicide is 4 : 1
• Old age; the risk age over 75 is three times that of
ages 15 to 24
PATIENT ORGANIZATIONS • Alcohol or other substance misuse
Association for Post Natal Illness, 145 Dawes Road, Fulham, • Previous inpatient psychiatric treatment
London SW6 7EB, tel. 020 7386 0868, www.apni.org • Family history of mental illness, suicide, or alcoholism
Mental Health Foundation; www.mentalhealth.org.uk.
National Childbirth Trust, Alexandra House, Oldham
• Positive for aquired immunodeficiency syndrome
Terrace, London W3 6NH, tel. 0300 330 0700, www.nct.org (AIDS) or HIV
.uk (search on “depression”). • Unmarried, separated, or divorced, especially if living
alone
Management of Suicidal Patients

Suicide and Suicidal Risk Low Risk
• Manage at home patients who have thoughts of suicide
• Two-thirds of successful suicides are mentally ill (Owens, but who have:
Lloyd, & Campbell, 2004), mostly with undiagnosed depres- • no clear suicidal plans or past history of a serious
sion. In the elderly the prevalence of mental illness in suicides suicidal attempt;
rises to up to 95% (O’Connell, Chin, Cunningham, & • good rapport with their GP or local psychiatric services;
Lawlor, 2004). • 24-hour home support;
• The risk of completed suicide in the first 9 days of drug • a stable personality;
treatment is 38 times that of the risk in a patient who • no psychotic illness, chronic physical illness, or drug
has been on treatment for at least 3 months (Jick, Kaye, or alcohol misuse
& Jick, 2004). This is independent of the type of anti- • Drugs. If prescribing, give small quantities of medication,
depressant used. initially.
High Risk
Stress Reactions
• Refer urgently to the mental health team. Check that SUPPORT ORGANIZATIONS
the patient meets the previously agreed local criteria for
same-day referral. A domiciliary visit by a psychiatrist Survivors of Bereavement by Suicide (SOBS). National
helpline 0300 111 5065; www.uk-sobs.org.uk.
may be indicated (even if a patient refuses all offers of Winston’s Wish is specifically for children bereaved by
help). suicide. Helpline 08088 020 021; www.winstonswish.org.uk.
• If the GP and psychiatrist cannot visit together, make CRUSE offers support to all those who are bereaved. Day
sure that the GP is notified immediately of the outcome by Day helpline 0844 4779400, Young Person’s helpline
of the visit. 0808 808 1677; www.crusebereavementcare.org.uk.
The Compassionate Friends helps parents and siblings
• Ensure that the carers can provide observation for 24 who have lost a child, including from suicide. Helpline 0345
hours a day, until the risk diminishes. The family may 123 2304; www.tcf.org.uk.
need help to provide this level of care. If drug treatment
is started, a relative or carer ought to be asked to supervise
the medication. • Stress reactions may be:
• acute and brief;
Follow-Up After Attempted Suicide • an adjustment reaction which may last a few months;
Often the first time a GP hears about a suicidal patient is or
when a hospital discharge report is received. When this occurs, • posttraumatic stress disorder (PTSD), with a delay
the doctor should: between the stress and the onset of symptoms.
• review the records to see if the patient has recently • Stress may be a reaction to loss or trauma. Symptoms
attended; may be those of anxiety, depression, abnormal behaviour,
• discuss the management with the mental healthcare team, or inability to cope with normal events. Symptoms may
if the patient has a severe longstanding mental illness; have been present for a long time before help is sought.
• identify anything that might indicate the patient was a • Stressful events include:
suicide risk; • crime or accidents involving psychological and/or
• enter suicide attempt on the notes; physical trauma;
• ask the patient to make an appointment to see the GP • bereavement, including siblings in a family where a
for review; child has died;
• follow-up to: • termination of pregnancy, miscarriage;
• identify depression or other precipitating life events; • redundancy, loss of job, occupational stress;
• assess the need for treatment, including counselling; • divorce, separation, relationship difficulties;
• make a care plan to review outcome and prevent • housing or financial problems;
recurrence. • surviving a disaster;
• start treatment if indicated, as discussed next. • having to perform in front of an audience, or having
a work appraisal;
• seeking asylum;
• drug or alcohol withdrawal.
SUPPORT ORGANIZATIONS
Management
The Samaritans. Tel. 116 123; email: jo@samaritans.org;
website: www.samaritans.org. • Identify underlying precipitating events and the steps taken
PAPYRUS (prevention of young suicide). Helpline 08000 by the patient to modify or cope with the situation.
684141; www.papyrus-uk.org. • Exclude a physical or drug cause for the symptoms (drug
or alcohol withdrawal, sudden stopping of a beta-blocker,
hyperthyroidism).
• Identify others who might help (e.g., Relate, Citizens’
Advice Bureau, Victim Support, union representative,
Helping Those Bereaved by Suicide local police domestic violence unit).
• Review what support can be obtained from family, friends,
• Grief after suicide seems to be particularly intense and work colleagues, and sources of community support.
often associated with shame and guilt. On average six • Discuss coping strategies.
people have intense grief for every suicide (Hawton, 2003). • Assess the need for counselling.
Identify the people most likely to be affected and give • Decide if short-term time off work might be helpful.
them a chance to talk about their feelings. • Consider prescribing a beta-blocker if somatic symptoms
• If their grief is intense, refer for counselling or recom- (shaking and tachycardia) are prominent. Avoid anxiolytic
mend self-help literature or self-help groups, according drugs but if the symptoms are very severe give them for
to its severity. a maximum of 2 weeks.
Posttraumatic Stress Disorder from one-third (with placebo) to half, although the reduc-
tion in symptoms was small (Jick et al., 2004). It may
• The risk of developing PTSD varies according to the take 8 weeks before effects are seen; and high doses may
nature of the trauma and the susceptibility of the patient, be needed. If effective, continue for at least 6 months in
ranging from 10% following a road traffic accident, to total, or at least 12 months if the PTSD has lasted more
32% following a myocardial infarct (Jones et al., 2007), than 3 months, to avoid relapse. There is an impression
to 57% following rape (Hull, 2004). that patients do better if the drugs are started early in
• A study from the Netherlands suggests that non-traumatic the course of the condition. NICE recommends paroxetine
life events (e.g., divorce or unemployment) may be more and mirtazapine for general use and other antidepressants
frequently the cause of PTSD than trauma (Mol et al., for use by mental health specialists (NICE, 2005b).
2005). • Other drugs. Consider a beta-blocker for a patient disabled
• Presentation may be delayed for several months after the by startle and hyperarousal symptoms. The evidence for
event. In the United States the lifetime prevalence is 8% benzodiazepines is poor. However, consider a nighttime
(American Psychiatric Association, 2000a). An Israeli study short-acting benzodiazepine for a patient exhausted by
found that only 2.4% of patients with PTSD had been poor sleep. Explain the disadvantages (dependence and
detected by their GPs (Munro, Freeman, & Law, 2004). tolerance) and obtain the patient’s agreement that it will
• Look for PTSD specifically in patients who have had an only be used, at the most, twice a week while waiting for
extremely traumatic incident in their lives. The cues to the SSRI to take effect.
the diagnosis are when, at least 1 month after the event, • Inform the patient about the resources listed in the box.
the patient’s life is disturbed by:
• intrusive symptoms; memories, flashbacks, and
nightmares; PATIENT ORGANIZATIONS
• avoidance of thoughts, activities, situations associated Victim Support provides emotional and practical support for
with the event; emotional numbing; victims of crime. Support line: 0808 1689 111; email:
• symptoms of autonomic arousal (e.g., hypervigilance, support@victimsupport.org.uk; website: www.victimsupport.
org.uk.
insomnia, irritability, excessive anger, and impaired The Refugee Council. Tel. 020 7346 6700; www
concentration and/or memory). .refugeecouncil.org.uk.
• Check whether the patient has taken refuge in drug or Combat Stress (Ex-Services Mental Welfare Society)
alcohol misuse. supports ex-service people with PTSD. Tel. 0800 138 1619;
• In severe cases assess the risk of suicide. email: contactus@combatstress.org.uk; website: www
.combatstress.org.uk.
MIND has excellent information. Call the Mindinfoline 0845
Management 766 0163 or download it from www.mind.org.uk (search
• Debriefing. Do not routinely offer debriefing after a trau- “post-traumatic stress disorder”).
matic event. It appears to be useless and may be harmful
(Bisson, 2004). A follow-up appointment 1 to 2 months
after the event is more likely to be useful. Even then,
beware of overdiagnosing it in a patient who is recovering Insomnia
spontaneously from a traumatic event.
• Provide information for patient and family (see box). • Insomnia may be primary, or secondary to another mental
• Encourage discussion of the precipitator event once PTSD or physical disorder or to medication. Common causes
has developed. of insomnia are depression, anxiety, menopausal symptoms,
• Encourage the patient to discuss his or her feelings and restless legs syndrome, pain, and drugs. Half of patients
fears. with insomnia have a specific mental disorder and an
• Explain how avoiding things that remind the patient of overlapping half have a physical disorder (NICE, 2004a).
the trauma prolongs the syndrome. • Patients with secondary insomnia need treatment both
• Draw up a gradual plan to face avoided activities and of the underlying condition and of the insomnia. A study
situations. of patients with rheumatoid arthritis found that treating
• Alcohol. Warn about the need to avoid excess use of alcohol. insomnia with a short-acting benzodiazepine improved
If the patient is already misusing alcohol or other drugs, morning stiffness as well as sleep (Walsh et al., 1996).
treatment of the misuse must come before treatment of • Sedatives significantly increase sleep duration and quality
the PTSD. but at the risk of adverse effects, especially cognitive
• Psychological therapy. Refer for trauma-focused CBT if impairment next day and daytime sleepiness (Glass et al.,
symptoms are present 3 months after the event. Refer at 2005). They also have addictive potential.
1 month if symptoms are severe (NICE, 2005a). • Check that the insomnia is not due to an underlying
• Antidepressants. SSRIs reduce all symptoms of PTSD as disorder which needs treatment in its own right. Drugs
well as treating any associated anxiety and depression. which can cause insomnia include SSRIs and venlafaxine,
Sertraline has been shown to increase the response rate and high-dose steroids.
• Explain that failing to sleep is a natural part of a reaction bed. Refuse to discuss exciting or worrying things in bed;
to stress and not harmful; and that worrying about not keep the bed for sleep and sex.
sleeping makes sleep harder to achieve. • Make sure the room is dark and quiet. Use earplugs if
• Check that the patient is not using alcohol to sleep. In necessary.
non-dependent drinkers it does improve sleep in the first • Avoid caffeine from about 4 pm. This may include choco-
part of the night but at the expense of early-morning late and tea as well as coffee and cola.
rebound awakening. Dependent drinkers lose even this • If you find yourself worrying, write down what the problem
early benefit. is and when you are going to think about it properly.
• Explain that simple rules will help (see discussion to come) Worrying about something at night magnifies a problem
and offer a leaflet (e.g., www.patient.co.uk (search on that may seem perfectly solvable in the day.
“insomnia”). Behavioural techniques can be more • Distract yourself with thoughts of something pleasant:
effective than drug treatment, with longer-lasting gardening, sex, an imaginary dinner party in which you
benefit, but they take longer to administer (Sivertsen et al., light a candle in front of each of your friends and visualize
2006). their faces in detail. You need something more interesting
• Offer medication only if the patient’s functioning is affected than counting sheep.
by the lack of sleep and the reason for the insomnia • If you still can’t sleep, don’t lie there fretting. Get up, sit
is temporary (e.g., jet lag or changing shifts). Obtain somewhere warm, do something restful, and go back to
the patient’s agreement that the course will not extend bed when you feel sleepy.
beyond 14 days because of the dangers of dependence, • However tired you feel in the day, don’t nap. It will mean
tolerance, and difficulties on withdrawal. Use a short- you sleep even less well at night.
acting benzodiazepine. NICE (2004a) has assessed the • If you’ve slept badly, accept it and lead a full day. Most people
Z drugs (zaleplon, zolpidem, and zopiclone) and found can function on less sleep than they think they need.
insufficient evidence to recommend them over the cheaper
benzodiazepines. INFORMATION FOR PATIENTS
• Be aware that some specialists in sleep disorders think
The Royal College of Psychiatrists has leaflets and audio
that the approach set out in the British National Formulary tapes at www.rcpsych.ac.uk (search on “sleep problems”).
(BNF) and repeated earlier is too draconian; and that the
evidence of tolerance and dependence, when short-acting
benzodiazepines are used for true primary insomnia, is
poor (Wilson & Nutt, 2003). A compromise position is Anxiety Disorders
to prescribe a short-acting benzodiazepine for, say, 14
nights, to be repeated every 2 months, allowing the patient • Anxiety disorders (Table 18.2) are more common than
to use them when the need is greatest. depression, rarely present as pure anxiety, are even less
• Consider a tricyclic antidepressant in those for whom all frequently diagnosed, yet the majority of patients will
other approaches have failed or are inappropriate. Depen- respond to treatment: usually an SSRI or cognitive behav-
dence is not a problem but daytime drowsiness and other ioural therapy.
adverse effects are. Consider it an option of last resort. • Detection of an anxiety disorder is improved if the GP
An early resort to drugs will distract the patient from the uses a few screening questions (Katon & Roy-Byrne, 2007).
changes in lifestyle, and in their mental attitude to insom- • There are few conditions in which a positive, persistent,
nia, that are necessary. and supportive stance by the doctor is more important.
• Do not prescribe melatonin for most types of insomnia. The patient’s instincts will be to avoid facing up to situ-
A meta-analysis has shown that it is ineffective both in ations that provoke anxiety while treatment relies on facing
secondary insomnia and in jet lag (Buscemi et al., 2006). up to them.
It may be useful in people with delayed sleep phase syn- • Almost all anxiety disorders can be managed in primary
drome (in which the patient’s circadian rhythm is off care, especially if a member of the team is trained in
kilter) (Buscemi et al., 2004). cognitive behavioural therapy. However, any patient suf-
• Consider referral for cognitive behavioural therapy if the ficiently distressed by an anxiety disorder who has not
patient is sufficiently troubled by the problem. Even long- responded to primary care management should be offered
term users of hypnotics can benefit, with improved sleep, referral to a specialist mental health service.
improved quality of life, and reduction in use of hypnotics • Individual SSRIs and related drugs are licensed in the
(Morgan et al., 2003). United Kingdom for one or more types of anxiety but
not for all types. There is every reason to think that this
Simple Rules to Reduce Insomnia is a quirk of licensing and that the benefits are common
• Go to bed and get up at the same time, even on weekends. to all drugs of that class.
It helps set your internal clock. • Do not lead the patient to expect a cure; they are likely
• Prepare for sleep by doing something restful in the half to be disappointed. Most however can expect significant
hour before bed. Avoid exercise in the 3 hours before improvement with treatment.
TABLE Prevalences of the Anxiety Disorders in the NICE does not recommend this in primary care. Warn
18.2 General Population (American Psychiatric of the risks of dependence, sedation, industrial accidents,
Association, 2000b; Ballenger & Tylee 2003) and road traffic accidents (Gale & Oakley-Browne, 2004a).
Outside the United Kingdom a longer term role for ben-
Condition Lifetime Prevalence
zodiazepines is accepted, with the view that dependence
Generalized anxiety disorder 5% and tolerance is rare when prescribed for anxiety (Ballenger
PD 2%–3% & Tylee, 2003).
• Arrange an early appointment to begin definitive treat-
Panic attacks without 10% ment for the condition.
reaching the criteria for PD
Agoraphobia 6% Long-Term Treatment
Social anxiety 13% • Explain how anxiety causes physical symptoms and how
they in turn increase anxiety. Offer a leaflet (see Informa-
Specific phobias 7%–11%
tion for Patients box).
OCD 2%–3% • Assess the patient’s disability. How does it affect family
Obsessive or compulsive 8% relationships, sex, work, and physical and mental state?
symptoms without reaching Is a job at risk? Is alcohol or caffeine intake excessive?
the criteria for OCD • Explain the options:
Note that most patients suffer from more than one condition.
• Cognitive behavioural therapy (CBT). CBT has the stron-
Note that the prevalence in a primary care population will be higher, since gest evidence of benefit (Gale & Oakley-Browne, 2004b).
patients with anxiety present more frequently, usually with somatic A 1- to 2-hour weekly session will be needed for a total
complaints.
OCD, Obsessive-compulsive disorder; PD, panic disorder.
of 8 to 20 hours with a trained professional. Briefly, it
involves training the patient to act and think differently
from his or her usual manner until a new response to
life becomes natural. However, do not oversell CBT to
• Anxiety disorders rarely start in midlife or later. Such a the patient. Even in randomized controlled trials only
presentation suggests another underlying disorder, espe- half have recovered at 6-month follow-up (Fisher &
cially depression or alcohol misuse. Durham, 1999). Those who do show some response
continue to improve for at least 2 years after treatment
Generalized Anxiety has ended (Dugas, Ladouceur, & Leger, 2003). Anxiety
management, without cognitive restructuring, can achieve
GUIDELINE similar benefit (Gale & Davidson, 2007).
• An SSRI. Almost half of patients show significant improve-
National Institute for Health and Care Excellence. (2011). ment with follow-up periods of up to 6 months. However,
Generalised anxiety disorder and panic disorder in adults:
Management. NICE clincial guideline 113.
this yields an NNT of only 5 (because several improve
on placebo) (Kapczinski, Lima, & Souza, 2003). Although
this seems an easier option than CBT there are disad-
vantages: benefit may not be seen for some weeks and
• Check whether the patient also suffers from other mental maximum benefit not for 6 months; anxiety may worsen
conditions which need treatment in their own right (e.g., in the first few weeks; at least 6 months of treatment will
panic disorder, obsessive-compulsive disorder [OCD], be needed; and, while not addictive, there is the possibility
phobia, depression, PTSD, or the misuse of alcohol or that the patient will notice a discontinuation reaction,
drugs). especially if the drug is stopped suddenly. NICE recom-
• Check that there is no physical condition mimicking the mends sertraline first line. In patients who cannot take
symptoms of anxiety (e.g., thyrotoxicosis or asthma). an SSRI use a tricyclic antidepressant.
• Self-help, using a written approach with supportive
Management of a Crisis visits to the doctor or nurse. This is also CBT but
• It may take a crisis to bring the patient to seek help. without a personal therapist; the patient needs to find
Immediate treatment is needed to tide the patient over the motivation to succeed and be comfortable with
and gain the confidence of patient and family. reading and with acting on the written word.
• Give a brief but clear explanation of what is happening • Recommend a self-help group, whichever treatment the
and that effective help is available. patient chooses. Some can assist the patient in a CBT
• Consider prescribing anxiolytics for 2 to 4 weeks with approach, but all will provide support and help the patient
the clear understanding that this is crisis management realize that he or she is not the only one with this problem.
only. Use a benzodiazepine although, in a patient who • Follow-up. Unless the patient will be seeing a therapist
has misused a benzodiazepine in the past, a sedating anti- within 2 weeks, arrange for follow-up along the following
histamine or an antipsychotic may be preferred, though lines: at 2, 4, and 6 weeks; then at 3 and 6 months. This
applies whether the patient decides on an SSRI or self- INFORMATION FOR PATIENTS
help or is waiting to see a CBT therapist.
Self-Help Group
Practical Details When Prescribing SSRI No Panic is a charity that provides information, written
for Anxiety treatment programmes, and telephone support for those with
anxiety, phobias, and obsessive-compulsive disorders.
1. Start with a low dose to minimize the increase in anxiety Helpline 0844 967 4848; www.nopanic.org.uk.
seen with standard doses. For instance, start with parox-
etine 5 mg daily for 2 weeks and double the dose every Leaflets
fortnight until reaching 20 mg daily. This means cutting The Royal College of Psychiatrists leaflet “Help Is at Hand:
up tablets or prescribing it in liquid form. Anxiety, Panic and Phobias” is helpful. Available at www.
rcpsych.ac.uk.
2. If the response at 8 weeks is inadequate, increase slowly
to the maximum recommended dose. Books
3. Treat for a minimum of 6 months after a response is Butler, G., & Hope, T. (2007). Manage your mind: The mental
achieved. Patients who show some response at 2 months fitness guide. Oxford: OUP.
may continue to improve over the subsequent 6 months.
4. Decide, with the patient, how long to continue to prevent
relapse. Treatment for 1 to 2 years is likely to be needed
if the condition is longstanding.
5. With prolonged use, sexual dysfunction becomes the most
significant adverse effect. For men with erectile dysfunc- online by the patient. The patient needs a password to
tion, sildenafil and related drugs can be used. access the programme, for which Clinical Commissioning
6. When stopping the drug, tail it off in fortnightly steps Group (CCG) approval is needed.
over 1 to 2 months according to what dose was used. If • If there has been no response after 12 weeks of an SSRI,
a discontinuation reaction occurs, go back a step and tail consider a tricyclic antidepressant.
off more slowly. • The details in the box Information for Patients are the
same as for generalized anxiety.
Special Situations • Teach the patient first aid for a panic attack (see next
• The patient who fails to respond to an SSRI or in whom it section).
is contraindicated or not tolerated: Consider a tricyclic • Do not offer a benzodiazepine or another anxiolytic. They
antidepressant in the same doses as for depression. An are less effective than SSRIs and they are inappropriate
alternative is pregabalin, originally used in partial seizure in a disorder that usually lasts over 4 weeks and where
epilepsy. Give it twice daily (Pohl et al., 2005) with a dependence can occur. They do not treat the depression
maximum dose of 200 mg/day; further dosage increases that is often coexistent or which may emerge as the panic
are of no benefit. disorder is treated.
• The patient whose anxiety returns when the SSRI is stopped: • Consider a long-acting beta-blocker. It will abolish some
It can be hard to distinguish a discontinuation reaction of the somatic symptoms of panic and may help the
from the return of anxiety. The symptoms (dizziness, subjective terror as well (Mol et al., 2005). However, it
numbness, tingling, nausea, headache, sweating, insomnia, will not lead to a lasting cure and may distract the patient
and feeling anxious) are common to both. Sometimes from the need to enter more definitive therapy (i.e., CBT).
the patient is clear that the symptoms feel different from • Monitor progress with two questions:
the original anxiety. If in doubt, restart the SSRI and • How many panic attacks have you had since I last
taper the dose more slowly. saw you?
• The patient whose physical symptoms of anxiety are crippling: • How severe were they, on a scale of 1 to 5, where 1
Consider a beta-blocker, although there is a dearth of is very mild and 5 is extremely severe?
evidence (Gale & Oakley-Browne, 2004c). A long-acting • Prepare yourself, if not the patient, for a relatively poor
preparation will be needed. response to treatment. A Cochrane review (Furukawa,
Watanabe, & Churchill, 2007) found that 2 years after
Panic Disorder the start of treatment, 60% of patients had not achieved
• Follow the management outlined under Generalized a sustained response. Combining psychotherapy with an
Anxiety, but without the offer of a benzodiazepine. It is antidepressant was more likely to achieve a response in
even more important to explain the link between the the short term than either alone, with a NNT of 10. In
feeling of panic and the physical symptoms it produces, other words, if 10 patients are treated with both treat-
since patients often see it as a physical illness. ments one more will respond than if treated with one or
• Offer the same triad of behavioural therapy or CBT, an the other. However, follow-up of 1 or 2 years found that
SSRI, or self-help. In addition, NICE has approved the the combination of psychotherapy and antidepressant was
use of computer-assisted CBT (NICE, 2006). Fearfighter better than antidepressant alone (NNT 6) but no better
is an 8- to 12-week computer course of treatment accessed than psychotherapy alone.
surgery, anxiety, or depression. Failure to discover the

First Aid for a Panic Attack—Instructions for underlying phobia will result in a failure of treatment.
the Patient • Offer referral for CBT, an SSRI, or a self-help programme
• Panic always subsides, even if it can take an hour. along CBT lines (see Generalized Anxiety).
• If some situation has triggered the attack, try to stay in • In those patients who choose to be treated without refer-
the situation. Leaving can make it harder to go back later. ral, use the following programme, with or without treat-
• Breathe slowly but not deeply. Deep breathing can mean ment with an SSRI:
you hyperventilate and develop more symptoms. • Urge the patient not to avoid the phobic situation.
• Check your watch. What seems like an hour may only Avoidance increases the strength of the phobia.
be 5 minutes. • Teach the patient the rules of first aid during an attack
• Distract yourself (e.g., read every detail of the label on of panic (see previous section).
something on the supermarket shelf ). • Plan a programme of gently increasing exposure to
• Have something you say to yourself during the attack (e.g., the phobia. Do this with imaginary exposure if real
“It’s only a panic attack. I’m not going to faint or vomit exposure is not possible. Exposure must be daily, and
[or whatever you most fear])—and so what if I do?”). the patient must stay in the phobic situation until the
• Don’t use alcohol. Attacks are more likely as the alcohol panic has subsided. Ask the patient to keep a diary to
wears off. discuss at each appointment.
• Check whether caffeine has played a part in triggering • Ask what would be the worst possible scenario. Often
the attack. it turns out to be unpleasant but acceptable. Usually
it is that other people will see that the patient is being
Social Anxiety/Social Phobia phobic. Being prepared to own up to a phobia is often
• Patients with social anxiety often think they are excessively a great step forward.
shy, rather than suffering from a treatable mental • Recommend Manage Your Mind: The Mental Fitness Guide
disorder. by Gillian Butler and Tony Hope for details of strategies
• Take even more care than usual to explain the nature of that patients can use for themselves.
the condition. The concept of an overactive circuit deep • Drug treatment at the time of the phobic event: Avoid giving
in the brain may be acceptable to a patient who already benzodiazepines for a phobia that poses a problem more
understands the concept of, for instance, an overactive than occasionally. The patient is likely to become even
thyroid. less able to face the situation without drugs than before
• Follow the management outlined under generalized anxiety, treatment began. Consider a beta-blocker (e.g., propranolol
but without the offer of a benzodiazepine. An SSRI, 40 mg) 1 to 2 hours before exposure to the phobic situ-
exposure therapy, and a combination of the two are all ation in those who cannot tolerate the physical symptoms
effective during treatment. A large study suggests that of panic.
those treated with exposure therapy alone continue to
improve in the 6 months after treatment, whereas those Specific Phobias
treated with sertraline, with or without exposure, show
some loss of benefit (Haug et al., 2003). If the SSRI is • Fear of flying. Exposure therapy, either using a computer
continued long term, the benefit is sustained and a treat- stimulation or just sitting in an aircraft and imagining
ment course of a minimum of 6 months, following remis- that it is flying, shows marked short-term benefits with
sion, is needed (Schneier, 2003). over half of patients who previously refused to fly now
• Offer a beta-blocker to a patient with a circumscribed managing to fly. However, the effect does not last and is
social anxiety which is disabling and where the somatic almost completely lost after 6 months (Maltby et al.,
symptoms (tremor, palpitations) are preventing the patient 2002). It is therefore most suitable for someone who has
from functioning. Performing musicians are the prime to make a one-off flight. Several organizations offer expo-
example. Warn the patient that they will find it hard not sure therapy. For instance, Virgin Atlantic offers “Flying
to become psychologically reliant on the drug. However, Without Fear” courses (tel: 01423 714900; www.flying
they may prefer this to the loss of their chosen career. withoutfear.info/).
• Fear of medical or dental procedures. Cognitive behavioural
Phobias therapy is the most effective treatment, but most patients
• Phobias require energetic management if they present present too late, when the need for help is urgent. Diaz-
early, to prevent them from becoming fixed. Even those epam 10 mg 1 to 2 hours before the event has been shown
presenting late are amenable to treatment if the patient to reduce anxiety significantly more than placebo, although
is willing to undergo therapy. All patients with phobias the placebo effect is also marked (Wilner et al., 2002).
need treatment. • Patients with agoraphobia are likely to find themselves
• Many patients who are phobic do not present with the unable to attend a primary care clinic and even more
phobia but with a consequence of it: alcohol or drug likely to have difficulty attending a specialist centre. Treat-
misuse, truancy, failure to attend appointments at the ment in primary care, using an SSRI and a simple CBT
approach (see previous discussion) will be successful in refuse CBT at first because it sounds too threatening may
some and allow others to improve enough to participate accept it after partial remission with drug treatment. CBT
in more definitive therapy. appears to offer most hope for lasting remission.
• If the patient responds then relapses after discharge, refer
INFORMATION FOR PATIENTS back urgently before the pattern of behaviour becomes
firmly established.
Self-Help Group
Triumph over Phobia has details of local groups. Tel. 01225 PATIENT INFORMATION
571740; email: info@topuk.org; website: www.
triumphoverphobia.com. Pedrick, C., & Hyman, B. (2010). The OCD workbook: Your
guide to breaking free from obsessive-compulsive disorder. In
Leaflets A Harbinger self-help workbook (3rd ed.).
The Royal College of Psychiatrists leaflet “Help Is at Hand: National Institute for Health and Care Excellence. (2005).
Anxiety, Panic and Phobias” is available at www.rcpsych. Treating obsessive-compulsive disorder (OCD) and body
ac.uk. dysmorphic disorder in adults, children and young people.
Understanding NICE guidance—information for people with
Book OCD or BBD, their families and carers, and the public. NICE
clincial guideline 31. www.nice.org.uk.
Butler, G., & Hope, T. (2007). Manage your mind: The mental
fitness guide. Oxford: OUP. Patient Support
OCD Action at www.ocdaction.org.uk or via the helpline
0845 3906232. The website includes aids to self-diagnosis,
blogs by people with the condition, and information about
support groups.
Obsessive-Compulsive Disorder
• Be on the alert for the diagnosis, as patients are often reluctant
to admit to the disorder. In patients whose symptoms raise
the possibility of OCD ask specific questions: “Do you find Self-Harm
yourself doing things repeatedly in a way you can’t control?”
and “Do you find yourself bothered by certain thoughts GUIDELINE
that you can’t get out of your mind?” Conditions that raise National Institute for Health and Care Excellence. (2004).
the possibility of OCD include depression, anxiety, eating Self-harm: The short-term physical and psychological
disorders, fear of illness, fear of causing others harm, and management and secondary prevention of self-harm in
skin disorders that suggest repeated hand washing. primary and secondary care. NICE clincial guideline 16,
• Once OCD is diagnosed, check for depression. One-third updated 2014. www.nice.org.uk.
with OCD are currently depressed and two-thirds will
become depressed at some stage (Ballenger & Tylee, 2003).
• Assess the risk of self-harm and suicide.
• The NICE (2005b) guideline suggests a stepped-care • In the United Kingdom 1 in 10 teenagers deliberately
approach, with the starting point being the recognition self-harms. In 2015–2016 in England and Wales nearly
of the disorder and the offer of information about the 19,000 required admission to hospital (National Society
condition and the support groups that are available (see for the Prevention of Cruelty to Children [NSPCC],
box). Later steps involve the provision of self-help mate- 2016). Seven of eight are female.
rial, referral for CBT, and the offer of an SSRI (see upcom- • It is the strongest single predictor of successful suicide,
ing discussion). being found in 40% to 60% of suicides (Hawton, Zahl,
• Involve the family or carers in the process of finding out & Weatherall, 2003). However, that does not make it a
about the disease and its treatment. useful predictor of suicide. Of self-harmers in the United
• When functional impairment is at least moderate, offer Kingdom, only 0.7% kill themselves within a year and
an SSRI as described under Generalized Anxiety. Expect 3% in 15 years.
the response to take even longer (up to 12 weeks) than • Two of three self-harmers consult their GP in the subse-
with other forms of anxiety. Expect to continue it for at quent 3 months (Bennewith et al., 2002). One in six will
least 1 year. self-harm again within a year. However, attempting to
• If the patient cannot take an SSRI, consider clomipramine assess the risk of repeated self-harm is unlikely to be suc-
(Ballenger & Tylee, 2003). NICE advises against other cessful (Kapur et al., 2005).
tricyclic antidepressants. • A large study of an intervention in which patients were
• Offer CBT if it is available. It involves gradual exposure sent an invitation to consult following an episode of self-
of the patient to situations that provoke anxiety while harm failed to show any benefit in terms of reducing
not allowing the patient the relief of using a ritual to repeated episodes (Bennewith et al., 2002). Indeed, sys-
relieve that anxiety. At the same time the therapist retrains tematic review has failed to identify benefit from any
the patient in different patterns of thought. Patients who treatment (Soomro, 2004).
• Ask what the patient did, what precipitated it, and whether • If this is a relapse, review past management and outcomes.
the problem is still present. Assume it is evidence of serious • Decide if admission or referral is necessary.
emotional distress. • Explain to the family what you think is wrong and what
• Assess the current mental state and the suicidal risk. action is needed. If feasible, give the patient the same
• Look for other problems: medical illness, social problems, information and obtain consent.
past or present physical or sexual abuse.
• Manage according to what is found. The underlying When There Is a Risk of Violence
problem may be trivial (an impulsive act, immediately
regretted) or grave (a psychotic illness). Among those GUIDELINES
who are not mentally ill, being unclear about the reason National Institute of Heath and Care Excellence. (2015).
for the self-harm is a bad prognostic sign, as is male sex Violence and agression: Short-term management in mental
and being an older rather than a younger adolescent (Royal health, health and community settings. NICE clincial guideline
College of Psychiatrists, 1998). Self-harm in people over 10.
National Institute of Heath and Care Excellence. (2014).
65 years of age is especially associated with depression
Psychosis and schizophrenia in adults: Prevention and
and suicide (NICE, 2004b). management. NICE clincial guideline 178. www.nice.org.uk.
• Teach the patient ways of reducing the risk of serious
harm, while the underlying problem is being attended
to (Mental Health Foundation, 2004):
• Harm minimization: for example, make a small cut
rather than a large one, or take aspirin rather than • First, try to calm the patient (see previous guidance).
paracetamol and never more than four at once. • Appoint one member of staff to relate to the patient.
• Distraction: have a plan of something to do once the Others should move away.
urge to self-harm starts to build up (e.g., go for a run • Encourage the patient to move to a safe place.
or play music very loudly). • If there is a weapon, ask the patient to put it in a neutral
• Identify someone you can talk to when you feel in danger. place, not to hand it over.
• Explain to the patient throughout, in a calm manner,
what you are doing.
PATIENT INFORMATION • Ask the patient open questions (e.g., “Tell me what’s
The Basement Project. www.basementproject.co.uk. going on.”).
Royal College of Psychiatrists. Self harm. www.rcpsych.
ac.uk (search on “self harm”).
Drug Treatment
• NICE advises against starting antipsychotics in primary
care, particularly for a first episode. Treatment decisions
Acute Psychotic Disorders should be made in conjunction with local mental health
services.
The possibility of acute psychosis is raised by the following: • For relapses follow the treatment care plan, if one is avail-
• Hallucinations able, and refer to specialist mental health services for
• Delusions further management. Treatment decisions should be based
• Disorganized or strange speech on patient preference, previous response to medications,
• Agitation or bizarre behaviour and medical history.
• Extreme and labile emotional states • If a patient initially refuses medication that is urgently needed,
• Family concern about recent changes in personality, spending time, in a calm manner, can often win the
behaviour, or function patient’s confidence. Do not deny the patient’s delusions
or hallucinations but say something like: “I can see you
Initial Assessment are pretty upset about all this and I can help with that.”
• Rarely, an agitated or aggressive patient needs rapid tran-
• Obtain a careful history from family or friends of recent quilization. Tranquilization makes subsequent assessment
events and changes in the patient’s behaviour. difficult, it is traumatic for the patient, and sedated patients
• Ask about drug or alcohol misuse. need observation, which may not be possible. If there is
• Assess the mental state including the risk of suicide and no alternative to forcible tranquilization do it only when
of harm to others. sufficient police, or mental health staff, have arrived. For
• Make a differential diagnosis if this is a first episode. a strong patient, four male officers are likely to be needed.
Consider the possibilities of acute confusional state, drug- • Options for rapid tranquilization:
induced psychosis, and epilepsy. An episode that is related • lorazepam 2 to 4 mg orally or, preferably, sublingually;
to cannabis use probably signifies an underlying psychosis or
triggered by the cannabis. • haloperidol 5 to 10 mg orally; or, if refused,
• lorazepam 1 to 2 mg intramuscularly; have flumazenil • The patient is suffering from a mental disorder of a
available for use if oversedated; or haloperidol 5 to nature or degree that warrants detention in a hospital
10 mg intramuscularly combined with promethazine for assessment (or assessment followed by medical
25 mg intramuscularly. treatment) for at least a limited period.
If two drugs are needed combine lorazepam and halo- • The patient ought to be detained in the interests of
peridol (with promethazine). his or her own health or safety, or with a view to the
• After rapid tranquillization, check pulse, blood pressure, protection of others.
and respiratory rate while waiting for the ambulance to • There is no alternative management other than com-
arrive. Ask the crew to continue monitoring with pulse pulsory admission to hospital.
oximetry in the ambulance. • Mental disorders are mental illness, psychopathic disorder,
mental impairment, and severe mental impairment. The
Precautions When Visiting a Disturbed latter two disorders refer to patients with learning difficul-
Patient at Home ties associated with abnormally aggressive or seriously
irresponsible conduct.
• Ensure there is no past history of violence or delusions • Although often called by relatives or neighbours, the
focused on the GP. doctor’s role is to safeguard the patient’s welfare and not
• If necessary, organize support and do not go alone. that of the relatives or neighbours, unless they are in
• Try to ensure that a relative or friend is present. danger.
• Tell someone in the practice whom you are visiting and • A patient can be admitted compulsorily even when posing
when. no danger provided that he or she is sufficiently ill (e.g.,
• Arrange for the practice staff to phone you after a speci- acutely psychotic) to need compulsory admission, because
fied time. treatment is urgently needed for the sake of the patient’s
• Have an action plan if you do not respond to the phone call. health.
• An approved mental health professional (AMHP) is
Referral and Admission responsible for making the application for compulsory
admission, coordinating the medical assessment of the
• Refer all patients suffering from acute psychosis to the psy- patient, providing the forms, and arranging for the trans-
chiatrist or specialist mental health team. The specialist port of the patient to hospital.
services will usually decide whether the patient needs • Every practice, and CCG, should have agreed on a pro-
admission or can be managed at home. cedure for the management of psychiatric crises. Appendix
• Admission is usually required in acute psychosis especially 21 is an example.
if:
• it is the first episode; The Assessment Under the Act
• there is a significant risk of suicide, violence, or neglect;
• the patient is non-compliant or has serious side effects; • Obtain all available information, from the patient’s records
or and from available friends and family. Try to find out
• there is coexisting alcohol or drug misuse. who is the nearest relative (note that this is not necessarily
• Management at home can be considered in a patient who the same person as the next of kin): They have the right
is suffering from a relapse of a known mental illness with to be informed, to object to detention, and to insist that
a previous good response to treatment, where the patient a detained patient be discharged, unless that patient poses
agrees to restart treatment, is at low risk, where home a danger.
care is safe, and community support is available. • Telephone the AMHP and agree on a plan. This should
• Driving. A patient suffering from an acute psychosis or include whether to involve a second doctor or the police
relapse of bipolar affective disorder should not drive and at this stage.
the Driver and Vehicle Licensing Agency (DVLA) must • If admission looks likely, contact the duty psychiatrist to
be informed. discuss the availability of a bed and the need for his or
her attendance.
• If the decision is made not to admit the patient, work
Compulsory Admission out a care plan. Essential features are that family members
Indications for Use of the Mental Health Act know whom to call in an emergency and that intensive
(England and Wales) 1983 follow-up arrangements are put in place.
This account includes amendments made to the Mental Health

Which Section of the Act Should Be Used?
Act up to 2015. It focuses on sections of the act most relevant
to general practice. How Desperate Is the Situation?
• The Mental Health Act can only be used if the following • The patient is about to injure others or self. The doctor may
conditions are met: restrain the patient or give emergency treatment knowing
that under common law such action is defensible if done • Learning difficulty is not grounds for compulsory admis-
in good faith. Such action would normally be followed sion under Sections 3 or 4 unless associated with “abnor-
by an admission under the act using Section 2 (28 days) mally aggressive or seriously irresponsible conduct.”
although if the emergency continues Section 4 (72 hours) However, the other relevant sections, including Section
might be needed. 2, can be used.
• Admission is needed and is so urgent that: • Psychopathic disorder is defined as “a persistent disorder
• the doctor cannot leave the patient; and or disability of mind (whether or not including signifi-
• waiting for an approved clinician (usually an approved cant impairment of intelligence) that results in abnor-
psychiatrist) would cause undesirable delay. mally aggressive or seriously irresponsible conduct on
Use Section 4 (detention for 72 hours). This requires the part of the person concerned.” It is grounds for
assessment by a doctor and an AMHP. Admission must compulsory admission, regardless of whether it is treat-
take place within 24 hours of the assessment or the appli- able, provided that the other criteria are met. Only in
cation, whichever is earlier. The fact that attendance by using Section 3 is it necessary that the psychopathy
the approved clinician is inconvenient is not grounds for should be “treatable”; that is, treatment should be neces-
the use of this section. sary to alleviate or prevent deterioration in the patient’s
• Admission is needed but there is time to wait for an approved condition.
clinician. Use Section 2 (28 days). This is the preferred
section and will be used in almost all cases. It requires Other Sections of Value
assessment by a mental health professional approved under
Section 12 of the act (the “first” doctor) and by a doctor • Section 3 allows for detention for 6 months. It is occa-
with previous knowledge of the patient (the “second” sionally used in the community instead of Section 4 if
doctor) and an AMHP. Admission must take place within the patient is already known to the psychiatric services.
5 days or the application is void. Arrange to see the patient In practice it is almost always used once the patient is in
together if possible, although the act allows the two doctors hospital and before a Section 2 or 4 has expired.
to examine the patient up to 5 days apart. • Section 7 allows the appointment of a guardian for up to
6 months. The guardian has the power to insist that the
Further Clarifications patient live at a specified place and attend for work, train-
ing, or medical appointments.
• The second doctor should be one with previous knowledge • Section 136 allows the police to remove a person from a
of the patient. However, if no such doctor is available, public place to a hospital or police station, for a maximum
any doctor may sign provided he or she does not work period of 72 hours, if the person is:
in the same hospital as the first doctor. • suffering from a mental disorder; and
• In theory the application for compulsory admission may • in immediate need of care and control.
be made by the nearest relative. In practice this is rarely • Section 135 allows a magistrate to authorize a police officer
wise: An AMHP is needed to ensure that the formalities (with a doctor and AMHP) to enter any premises to
are observed, that a full assessment is made, and to avoid which access has been denied, and remove the patient to
later recriminations within the family if the nearest rela- a place of safety if there is reasonable cause to suspect
tive made such a decision. that the person is:
• It is occasionally possible to commit a patient to hospital • suffering from a mental disorder;
under the act even if he or she agrees to informal admis- • being ill-treated or neglected or not kept under proper
sion. A psychiatrist may recommend this course of control;
action if, from previous knowledge of the patient, a • unable to care for oneself and lives alone.
judgment is made that an apparent agreement to informal The GP can usually gain entry legally without using Section
admission is not likely to be sustained. If taking this 135, for instance by asking the neighbours to open the
line of action, the reasons for doing so must be stated door or by using his or her relationship with the patient
on the form. to get the door open.
• Once an application has been signed, decide how to escort • A Community Treatment Order (CTO) ensures that assess-
the patient into an ambulance. Given time and patience ment and treatment are carried out in the community
it is usually possible to achieve it without force. If force with the responsible clinician having the power to recall
is needed it is the role of the police rather than the ambu- the patient to hospital if necessary.
lance crew.
• Drug or alcohol dependency is not in itself grounds for Regulations for Scotland and
compulsory admission. This is only possible if the person Northern Ireland
also suffers from a mental disorder as defined in the act.
If a patient is under the influence of drugs or alcohol so These follow similar principles to those for England
that a proper assessment cannot be made, it should be and Wales, but the brief details of the acts are as follows
postponed until such assessment is possible. (Tables 18.3 and 18.4):
TABLE Mental Health (Care and Treatment) • The patient’s ability to make decisions about the provi-
18.3 (Scotland) Act 2003 (see www.nes.scot.nhs sion of medical treatment is significantly impaired
.uk/mha/) because of his or her mental disorder.
• The making of the compulsory treatment order is
Type of
necessary.
Compulsion Professionals Duration
A compulsory treatment order may authorize detention
Emergency GP, and MHO if 72 hours in hospital or impose certain requirements on the patient
detention practicable in the community (a community-based compulsory treat-
Short-term GP and MHO 28 days ment order).
detention • The act also allows for the removal to a place of safety of
Compulsory MHO, AMP, and 6 months a person who is exposed to ill treatment or neglect, or
treatment order patient’s GP or who is unable to look after oneself or property/financial
another AMP affairs. It further allows for a person to be removed from
Power of entry MHO obtains from Single event a public place to one of safety where it is in the interests
sheriff or JP of that person or where it is necessary to protect other
people (NHS Education for Scotland, 2004).
AMP, Approved medical practitioner; GP, general practitioner; MHO,
mental health officer.
ADVICE FOR PATIENTS AND PROFESSIONALS
MIND provides a leaflet on the act at www.mind.org.uk
(search on “Mental Health Act”).
TABLE
18.4 Mental Health (Northern Ireland) Order 1986 Royal College of Psychiatrists, www.rcpsych.ac.uk (search
on “Mental Health Act”).
Situation Section Professionals Duration
Emergency 4 GP and ASW or 7 days
relative
Chronic Schizophrenia
Power of 129 ASW obtains Single event
entry from JP,
GP and police GUIDELINE
enter
National Institute of Heath and Care Excellence. (2014).
AMP, Approved medical practitioner; ASW, approved social worker; GP, Psychosis and schizophrenia in adults: Prevention and
general practitioner; JP, justice of the peace; MHO, mental health officer. management. NICE clincial guideline 178. www.nice.org.uk.
For more details, see www.psychiatry.ox.ac.uk/cebmh/whoguidemhpcuk/
mha.html.
• About 1% of a practice population will suffer from schizo-

• For emergency detention the following must apply: phrenia at some time in their life, more in cities and in
• The person has a mental disorder that causes his or immigrant communities.
her decision making to be significantly impaired. • Structured care offers a chance of improving the manage-
• It is necessary as a matter of urgency to detain the ment of such patients.
person for assessment. • In the United Kingdom the average GP will look after
• The person’s health, safety, or welfare or the safety of 12 patients with schizophrenia, half of them without
another person would be at significant risk if he or current involvement of the specialist mental health services
she was not detained. (Picchioni & Murray, 2007).
• Making arrangements for the possible granting of a
short-term detention certificate (see below) would Structured Care
involve undesirable delay. This entails:
• For short-term detention the same four criteria must apply • establishing a register of patients with severe and enduring
except that it need not be a matter of urgency, and deten- mental illness. This can be done from existing clinical
tion is for assessment or treatment. codes, from repeat prescriptions for psychotropic drugs,
• For a compulsory treatment order, the following must apply: from addresses of hostels and homes catering for the
• The patient has a mental disorder. mentally ill, and opportunistically;
• Medical treatment is available which would be likely • using the register to ensure that patients are seen at least
to prevent the disorder worsening, or would be likely anually and actively seeking out patients who default
to alleviate the symptoms or effects of the disorder. from follow-up. See upcoming discussion for details of
• There would be a significant risk to the patient or to the annual review;
any other person if the patient was not provided with • developing a care plan that is individualized for patients
such treatment. taking into account psychiatric, medical, social, and
occupational or educational issues. Ideally this care plan • Help the patient to claim the disability living allowance
should be shared with carers; if eligible.
• working with local mental health teams to identify agreed
policies and guidelines for treatment, referral, and the Drug Treatment
management of relapse;
• adapting existing computer templates to record long-term • Early drug treatment seems to lead to better medium- and
follow-up and undertaking practice audit of these goals. long-term outcomes (Loebel et al., 1992).
• Without drugs, 60% of patients relapse in the 9 months
Referral after an acute attack. Maintenance with antipsychotics
• Arrange for urgent assessment of the following: reduces this by over one-half.
• Those presenting for the first time • Atypical antipsychotics are now first line drugs in the
• Those posing a risk to themselves or to others United Kingdom, although if a patient is successfully
• Those relapsing, or showing the prodrome of a relapse treated with a conventional antipsychotic it should not
• Those at risk of relapse because of: be changed. Atypical antipsychotics are chosen because
a. a deterioration in their home circumstances; of the lower incidence of extrapyramidal adverse effects
b. poor compliance; or and possibly greater effect on cognition and on negative
c. abuse of drugs or alcohol. symptoms. However, they are no more effective than typical
• Those suffering from side effects of medication or who antipsychotics and overall they have as many, but different,
are not taking it. adverse effects (Picchioni & Murray, 2007). The exceptions
Such an assessment is better done at home than in the to this are clozapine and olanzapine, which may be more
outpatient department if possible. effective than other drugs (Citrone & Stroup, 2006).
• Patients will also need to be referred, although not neces- • The choice of long-term medication should be made by
sarily urgently, if: the psychiatrist and patient together. It will be needed
• they are becoming increasingly disabled by their illness; for at least 1 to 2 years after a relapse and monitoring
• a care plan needs to be drawn up, as when a patient will be needed for 2 years after that.
moves to a new area; or • All antipsychotics are associated with an increased risk
• the patient or family request referral or the therapeutic of sudden cardiac death as well as of stroke. A baseline
relationship has broken down. electrocardiogram (ECG) is recommended if the manufac-
turer advises one is necessary, if the patient has a personal
General Management history of cardiovascular disease, if physical exam identifies
• Education. The patient and the family need to know: a cardiovascular risk factor (such as high blood pressure),
• that 85% to 90% recover from the first episode in the or if the patient is being admitted (NICE, 2014).
following 2 years (WHO, 1979). In the 5 years fol-
lowing the first episode, half do not relapse, or relapse Relapse
but recover completely between episodes; 20% will Ascertain whether the relapse is due to the patient discon-
never have another episode (Picchioni & Murray 2007); tinuing medication. If it is then:
• how to identify early signs of relapse. Looking for early • assess the reason for stopping treatment (e.g., the presence
warning signs can prevent relapse or reduce its severity of side effects, failure to obtain a prescription);
(Falloon, Laporta, Fadden, & Graham-Hole, 1993). • decide if restarting previous medication is appropriate
The Early Warning Signs Form (see Appendix 22) can and acceptable;
be used to help with this; • review the need for urgent psychiatric assessment;
• how to obtain early treatment; • if assessment is needed but will be delayed, and there is
• that avoiding extremes of expressed emotion by family concern about medication, phone for advice from the
members, whether hostility or overprotectiveness, can psychiatrist;
reduce the risk of relapse. • inform the patient’s care coordinator or the lead clinician
• Advance directives. Discuss the use of advance directives identified in the care plan if this is available.
with patient and family. These indicate what the patient
wants to happen if a relapse occurs. If one is drawn up, Medication Review
the patient, and primary and secondary care, all need • Patients need a medication review at least annually (NICE,
copies. 2014). The repeat prescription slip should indicate when
• Check that the patient has been offered the benefits of a medication review is due.
case management. In the United Kingdom this takes the • Someone in the practice needs to be responsible for ensur-
form of the care programme approach, in which a special- ing such patients have attended for this review, and to
ist team assists the patient with daily living, in the under- know what action to take if the patient defaults. The
standing of the condition and in its management. medication review will include:
• If fit for sheltered work, liaise with the mental health • assessment of mental state and compliance;
team or the disablement resettlement officer. • presence of adverse effects or drug interactions. Adverse
• Assess the patient’s housing needs. effects with conventional antipsychotics are likely to
be extrapyramidal; with atypical antipsychotics they • daily activities, employment, training, income, and dis-
are likely to be weight gain and hyperprolactinaemia; ability benefits;
• existence of drug or alcohol problems; • substance misuse, which is likely in half of patients;
• whether the patient has been admitted in the past 6 • accommodation needs;
months; • carers, relationships, dependants (children), home support;
• patients on clozapine need monitoring for agranulo- • assessment of physical health, including weight, blood pres-
cytosis, weekly for 6 months then monthly in the sure, cervical screening, smoking status, family planning
United Kingdom. Organizing this is the responsibility needs, fasting sugar, Hba1C, lipids, and assessment of
of the prescriber. If delegated to primary care a written cardiovascular disease (CVD) risk. Smoking cessation should
protocol is needed. be offered where appropriate and coexisting cardiovascular
disease and diabetes should be treated. In the United States
Side Effects of Medication an annual ECG is recommended in those on an antipsy-
Conventional Antipsychotics chotic because it can prolong the QT interval;
• Extrapyramidal adverse effects. If they occur, discuss man- • medication review and monitoring blood tests if
agement with the specialist. The main options are: necessary;
• reduce the dose of the antipsychotic; • whether care is GP only or shared with psychiatrist, com-
• add an anticholinergic drug (e.g., orphenadrine 50 mg munity psychiatric nurse (CPN), or social worker;
three times a day). Avoid procyclidine, which is a • assessment of carer’s needs.
stimulant and can be abused. Try to withdraw an anti-
cholinergic after 3 months without symptoms. Tetra- Carers’ Needs
benazine may help in tardive dyskinesia; • People who care for someone with severe and enduring
• change to an atypical antipsychotic. Clozapine is the mental illness should have:
treatment of choice. • a needs assessment at regular intervals;
• Neuroleptic malignant syndrome occurs in 0.5%. • a care plan which is reviewed annually;
• links with local carer support groups.
Atypical Antipsychotics • Carers need to be identified and this information should
• Weight gain be put on their summary card or computer problem list.
• Dizziness and postural hypotension in the early stages The practice should have a carers’ register and a practice
• Diabetes policy on whom to assess, when, and by whom. Social
• Extrapyramidal adverse effects, but they are uncommon services should record each carer’s needs and draw up an
and usually respond to dose reduction agreed care plan that includes:
• information about the mental health needs of the patient;
All Antipsychotics • how to identify a relapse and what action to take;
• Palpitations. Repeat the ECG. If the QT interval is pro- • advice on benefits, housing, and employment;
longed but is less than or 500 ms, reduce the dose. If it • arrangements for short-term breaks;
is over 500 ms, stop the drug (Medicine and Healthcare • social support including access to carers’ support groups;
Products Regulatory Agency [MHRA], 2006). • information about appeals or complaints procedures.
• The plan should be confirmed in writing and commu-
Psychotherapy nicated to the primary care team. The GP is the profes-
sional most likely to identify signs of stress or deteriorating
Good evidence exists for both cognitive behavioural therapy health of a carer. The plan will indicate who should
and family therapy and NICE recommends that both forms review the carer’s needs at such times, which the GP
of therapy be offered to patients and their families suffering can initiate.
with psychosis. A minimum course of 6 months of either
is needed. If they are available they should be offered as well
as medication.
PATIENT ORGANIZATIONS
Rethink Severe Mental Illness, 89 Albert Embankment,
Annual Review London SE1 7TP. General enquiries tel. 0121 522 7007;
national advice line 0300 5000 927; www.rethink.org.
The annual review is likely to be more comprehensive if a MIND, Mind Infoline 0300 123 3393; www.mind.org.uk.
checklist or computer template is used. Topics to cover are: SANE, Saneline tel. 0300 304 7000; email support via the
• current mental state, including the presence of depression, website: www.sane.org.uk.
delusional thoughts, anxiety, hallucinations, and signs of
Patient Information
self-neglect. Half of patients with schizophrenia develop
depression at some stage. It carries a higher risk of suicide The Royal College of Psychiatrists, www.rcpsych.ac.uk
(search on “schizophrenia”).
than depression in other patients (Jones & Buckley, 2003);
• problems, crises, or admissions since last seen;
Bipolar Affective Disorder • Assess the need for admission, including compulsory admis-
sion, or for referral to the community mental health team.
• Tell the family who to contact if the patient becomes
GUIDELINE
very agitated or severely disruptive.
National Institute of Health and Care Excellence. (2014). • Identify the carer’s needs. In chronic illness consider
Bipolar disorder: Assessment and management. NICE clincial
respite care.
guideline 185, updated 2016. www.nice.org.uk.
Medication
• Discuss the options with the patient’s specialist. They are
likely to involve:
General Management • an oral antipsychotic (usually haloperidol, olanzapine,
quetiapine, or risperidone);
• Check that the patient has had a recent assessment by a • increasing lithium while keeping the blood level within
specialist and that everyone is clear who the key worker the therapeutic range;
is, in case of difficulties. • short-term use of a benzodiazepine for night sedation;
• Explain the nature of the condition to the patient and • tailing off an antidepressant if one is being taken.
family. • Do not expect dramatic improvement. Even in the ideal setting
• Agree on what the warning signs of relapse are for that of clinical trials only about half the patients have a 50%
individual (see Appendix 22). improvement of symptoms over 3 to 4 weeks (Keck, 2003).
• Draw up a plan of action for when relapse occurs. This • Prepare to taper off the antipsychotic over at least 2 weeks
will depend on the form the relapse takes; in hypomania once in full remission. Continued use can precipitate
it may mean removing cheques, debit cards, and credit depression.
cards from the patient; in depression it may mean inten-
sifying social support. In mania it may involve supplying Prevention of Relapse
medication (an antipsychotic or a benzodiazepine) to be • The choice of medication to prevent relapse should be
taken at the first sign of relapse. based on patient preference and what has previously been
• Explore whether stressors seem to trigger a relapse: irregular effective.
hours, lack of sleep, use of alcohol or drugs. • Lithium is the first line drug as it is most effective.
• Enter the patient on the practice register of patients with • Alternatives are valproate (which can also be added to
severe mental illness. lithium if lithium alone is ineffective) or olanzapine.
• Ensure recall for annual reviews. Quetiapine can also be used especially if this has been
• Offer information about the local and national support effective in previous relapses.
that is available (see box).
Lithium
During Episodes of Depression The GP needs to:
• Arrange for specialist assessment and discuss any medica- • ensure clear agreement on who is responsible for monitor-
tion changes with the specialist prior to assessment if ing lithium treatment;
there will be a delay. • have an agreed protocol for monitoring blood levels,
• NICE recommends fluoxetine combined with olanzapine identifying side effects, and taking appropriate action.
as first line. Lamotrigine on its own is an alternative as Put the patient on the recall register for monitoring blood
are olanzapine or quetiapine which can both be used on levels;
their own. • provide the patient with a written description of drug
• If the patient is already on lithium then check lithium side effects and what to do if these develop (e.g., see
levels and titrate to the therapeutic range. Fluoxetine and Lithium: Your Medicine available at www.rcpsych.ac.uk);
olanzapine can be added to lithium and, if this fails, then • check the patient knows to maintain adequate fluid intake
lamotrigine can be added to lithium in place of fluoxetine and avoid dehydration and nonsteroidal antiinflammatory
with olanzapine. drugs (NSAIDs);
• Similarly, if the patient is on valproate then titrate this • check that the patient understands that the lithium
to the maximum tolerated dose and then fluoxetine with should not be stopped unless toxicity occurs, as relapse
olanzapine can be added. may occur.
• check lithium levels every 3 months. Take blood 12 hours
During Episodes of Mania or Hypomania after the last dose. Levels of 0.4 to 0.8 mmol/L are ade-
• Avoid unnecessary confrontation. quate for maintenance but in acute mania a level of 0.8
• Check if there was a trigger for the relapse and whether to 1 mmol/L is necessary;
it can be rectified. • check for change in dose. If the dose is changed, check
• Assess the risk of impulsive behaviour (e.g., financial or levels weekly for 4 weeks, then monthly for 3 months
business recklessness). and then 3-monthly.
• Check thyroid and renal function annually. Eating Disorders

• Warn women of reproductive age that lithium is an
enzyme inducer, which reduces the efficacy of the com- GUIDELINES
bined oral contraceptive, and that it carries a risk of
teratogenicity. National Institute for Health and Care Excellence. (2017).
Eating disorders: Recognition and treatment. NICE clincial
guideline 69. www.nice.org.uk.
Management of Suspected Lithium Toxicity King’s College London. (n.d.). A GP’s guide to eating
• Symptoms of toxicity include muscle weakness, shaking, disorders. www.kcl.ac.uk (search on “GP guide eating
trembling, fasciculation, nausea, vomiting or diarrhoea, disorders”).
ataxia, confusion, slurred speech, toxic psychosis, convul- King’s College London. (2009). Guide to the medical risk
assessment for eating disorders. www.kcl.ac.uk (search on
sions, coma, and cardiac arrhythmias. “eating disorders”).
• Check the lithium level and urea and electrolytes (U&Es).
• Stop lithium. Levels usually fall to safety within 24 hours.
If the lithium level is more than 1.5 mmol/L, or there is
diarrhoea and vomiting, stop lithium immediately and
seek urgent specialist advice. • Approximately 1.25 million people are thought to have
• Identify the cause of the toxicity before restarting lithium. eating disorders in the United Kingdom, according to
• If no cause is found, restart at a lower dose. B-eat, a national eating disorders charity.
• Monitor levels monthly for 3 months after toxicity for • It is estimated that 10% have anorexia nervosa, 40% have
which there was no obvious reason. bulimia nervosa, and the rest fall into the category of
other specified feeding and eating disorders (OSFED),
SUPPORT AND ADVICE FOR PATIENTS
including binge eating disorder (BED) (B-Eat).
AND FAMILIES • Although eating disorders can develop at any age, the
risk is highest for young men and women between 13
Bipolar UK. Tel. 0333 323 3880, www.bipolaruk.org.
The Mental Health Foundation. They do not offer advice
and 17 years of age.
or support and there is no helpline, but the website has • Of sufferers, 20% die prematurely from their illness, either
useful information: www.mentalhealth.org.uk. by suicide or from physical complications (Herzog et al.,
2000).
• Anorexia nervosa has the highest mortality of all the mental
health conditions, with one in five anorexia patients dying
Dealing With Violence by suicide (Arcelus, 2011).
• In 2007 it was estimated through the Adult Psychiatric
Every practice needs a policy for dealing with violent patients. Morbidity Survey that 6.4% of all adults (>16 years) in
Many of these patients have a past history of violent or England showed signs of an eating disorder, up to 25%
threatening behaviour. A typical policy would include: of whom were male (NHS Information Centre for Health
• a notice in the waiting room stating that physical or and Social Care, 2007).
verbal abuse, racism, threats, or violence to staff will result • Anorexia is characterized by low weight, a fear of gaining
in removal from the practice; weight, a desire to be thin, and a distorted body image;
• panic buttons in consulting rooms and a clear procedure patients believe themselves to be overweight when often
to follow if one is set off; they are underweight. BMI is usually lower end of normal
• clear guidelines on who should deal with an aggressive, or below normal.
abusive, or violent patient in the waiting room; • Bulimia is characterized by episodes of binge eating (described
• how risks will be assessed and what action will be as eating a significant amount at one time to the point of
taken; being uncomfortably overfull), followed by episodes of
• a protocol for the management of a patient whose delu- compensatory behaviour such as vomiting (purging) or
sions are focused on a specific doctor or nurse; excessive exercise, use of laxatives or diuretics. Body mass
• written indications on when to call the police and who index (BMI) may be normal or slightly above normal.
should do so; • Binge eating disorder is similar to bulimia without the
• guidance on how to deal with home visit requests to a compensatory behaviours, and BMI is usually high.
potentially violent patient;
• sympathetic support for staff exposed to violent situations; Assessment in Primary Care
• a policy of informing local GPs that a violent patient
who has been removed from the practice list may want • The following should be asked about, or noted, during
to register with them; history-taking for eating disorders, or when deciding how
• a record kept of all such incidents; urgently to refer people:
• a way to report these incidents as significant events so • An unusually low or high BMI or body weight for
that lessons can be learnt by others. their age
• Rapid weight loss Investigation and Monitoring

• Dieting or restrictive eating practices (such as dieting
when they are underweight) that are worrying them, • When first assessing a person with an eating disorder it
their family members or carers, or professionals is likely that blood monitoring will be required, at least
• Family members or carers reporting a change in eating once, but possibly more frequently if BMI is low or there
behaviour is ongoing purging, laxative or diuretic use. Recommended
• Social withdrawal, particularly from situations that tests include FBC, renal function, liver function, glucose,
involve food creatine kinase (and thyroid function and C-reactive
• Other mental health problems, including depression, protein [CRP] at the first appointment only, to rule out
anxiety, self-harm, obsessive-compulsive disorder, as other causes of weight change).
well as suicidal ideation • Other investigations should include blood pressure, pulse,
• The possibility of alcohol or substance misuse BMI, muscle strength using the sit up/squat test (see
• A disproportionate concern about their weight or shape box), and skin condition.
(e.g., concerns about weight gain as a side effect of
contraceptive medication)
• Problems managing a chronic illness that affects diet, TESTS FOR MUSCLE STRENGTH
such as diabetes or coeliac disease 1. Squat Test. The patient is asked to squat down on
• Menstrual or other endocrine disturbances, or unex- haunches and is asked to stand up without using the
plained gastrointestinal symptoms arms as levers if at all possible.
• Physical signs of: 2. Sit-Up Test. The patient lies flat on a firm surface such as
the floor and has to sit up without using the hands.
• malnutrition, including poor circulation, dizziness,
palpitations, fainting, or pallor;
• fractures;
• compensatory behaviours, including laxative or diet
pill misuse, vomiting, or excessive exercise. • The King’s College Guide to Medical Risk Assessment
• Abdominal pain that is associated with vomiting or also contains a handy summary of when to refer because
restrictions in diet, and that cannot be fully explained of concern (appropriate within 2 weeks) and when to alert
by a medical condition (red flag/same-day assessment likely required). For example,
• Unexplained electrolyte imbalance or hypoglycaemia if weight loss is below 0.5 kg a week, in a person with a
• Atypical dental wear (such as erosion) low BMI, that would be a concern, but more than 1 kg
• Whether they take part in activities associated with a a week would be an alert. Similar guidance is included
high risk of eating disorders (e.g., professional sport, for all blood results (Treasure J, 2018).
fashion, dance, modelling) • It is important to obtain a BMI, but this can be chal-
• Risk factors for boys and men include non-hetreosex- lenging as many patients are reluctant to be weighed. It
uality, previous dieting, previous obesity, and participa- may require building of trust before they will agree to
tion in sports that emphasize thinness, or body building stepping onto the scales, but a potential option can be
(Thompson, 2017). to ask them to step onto the scales backwards so that they
• Be aware that children and young people with an eating cannot see the result, but you can note it discreetly. With
disorder may also present with faltering growth (e.g., a time, they may then agree to step on forwards, as you
low weight or height for their age) or delayed puberty. will have demonstrated sensitivity to their concerns and
• Do not use single measures such as BMI or duration of anxieties.
illness to determine whether to offer referral or treatment • Assess whether ECG monitoring is needed in people with
for an eating disorder. an eating disorder, based on the following risk factors:
• Do not use screening tools (e.g., SCOFF) as the sole method • Rapid weight loss
to determine whether people have an eating disorder. • Excessive exercise
• Good questions to screen for eating disorders (Cotton, • Severe purging behaviours, such as laxative or diuretic
Ball, & Robinson, 2003) include: use or vomiting
• Do you worry that you have lost control over how • Bradycardia
much you eat? • Hypotension
• Do you make yourself sick when you feel uncomfort- • Excessive caffeine (including from energy drinks)
ably full? • Prescribed or nonprescribed medications
• Do you ever eat in secret? • Muscular weakness
• Does your weight affect the way you feel about • Electrolyte imbalance
yourself? • Previous abnormal heart rhythm
• Are you satisfied with your eating patterns? (If the • Advise those who are taking laxatives or diuretics that
answer is no and yes, respectively, to the latter two these will not help weight loss and should be stopped
questions, then an eating disorder is much less likely.) gradually.
• Advise those who are exercising excessively, or obsessively, physical health due to preexisting medical complications
to stop doing so. (e.g., hypokalaemia secondary to vomiting).
• Advise those who are vomiting regularly to have regular • Offer ECG monitoring for people with an eating disorder
dental checkups and avoid brushing their teeth immedi- who are taking medication that could compromise cardiac
ately after purging. functioning (including medication that could cause elec-
• GPs should offer a physical and mental health review at trolyte imbalance, bradycardia <40 beats/min, hypoka-
least annually to people with anorexia nervosa who are laemia, or a prolonged QT interval).
not receiving ongoing secondary care treatment for their
eating disorder. The review should include: Referral
• weight or BMI (adjusted for age if appropriate);
• blood pressure; • If an eating disorder is suspected after an initial assess-
• relevant blood tests; ment, refer immediately to a community-based, age-
• any problems with daily functioning; appropriate (specialist) eating disorder service for further
• assessment of risk (related to both physical and mental assessment or treatment.
health); • The aim of treatment is to reach a healthy weight/BMI
• an ECG, for people with purging behaviours and/or and psychological well-being.
significant weight changes; • It is vital not to delay referral because recent evidence
• a discussion of treatment options. shows that people have on average already had symptoms
• Monitor growth and development in children and young of an eating disorder for almost 3 years (B-eat, 2017)
people with anorexia nervosa who have not completed before they seek help, and then wait up to 6 months
puberty (e.g., not reached menarche or final height). between first GP visit and starting treatment. It takes on
• Consider a bone mineral density scan after: average 21 months for people to recognize that they have
• after 1 year of underweight in children and young people, an eating disorder, and then another year before they seek
or earlier if they have bone pain or recurrent fractures; help from a healthcare professional. This leads to a cycle
• after 2 years of underweight in adults, or earlier if they of relapse and recovery of, on average, 6 years in duration.
have bone pain or recurrent fractures. The same report showed that boys and men had to wait
• Do not routinely offer oral or transdermal oestrogen over twice as long as girls and women before being referred
therapy to treat low bone mineral density in children or by their GP. In other words, GPs are not yet recognizing
young people with anorexia nervosa. eating disorders rapidly enough in males.
• Seek specialist paediatric or endocrinologist advice before • It is important to take patient and family/carer concerns
starting any hormonal treatment for low bone mineral seriously and to avoid potentially dismissive comments
density. Coordinate any treatment with the eating dis- implying that the behaviour is a phase or that they will
orders team. grow out of it.
• Dietary advice (and dietician referral) should only be
Medication offered as part of a multidisciplinary approach.
• The mainstay of all eating disorders treatment is psycho-
• Medication should never be the sole treatment for eating logic therapy. This should be provided by a specialist
disorders but antidepressants or anti-anxiety medication trained in the appropriate evidence-based modalities.
may be helpful if there are comorbidities. • For those with an eating disorder who have another con-
• Encourage people with anorexia nervosa to take an age- dition such as diabetes, the secondary care teams will
appropriate oral multivitamin and multimineral supple- need to collaborate closely to minimize risk and optimize
ment until their diet includes enough to meet their dietary monitoring and treatment.
reference values. • Pregnant women with an eating disorder will require
• When prescribing medication for people with an eating coordinated care from both obstetric and eating disorders
disorder and comorbid mental or physical health con- specialist teams.
ditions, take into account the impact malnutrition • Admit people with an eating disorder whose physical
and compensatory behaviours (e.g., vomiting) can health is severely compromised to a medical inpatient or
have on medication effectiveness and the risk of side day patient service for medical stabilization and to initiate
effects. refeeding, if these cannot be done in an outpatient setting.
• When prescribing for people with an eating disorder and • For people with an eating disorder and acute mental
a comorbidity, assess how the eating disorder will affect health risk (such as significant suicide risk), consider psy-
medication adherence (e.g., for medication that can affect chiatric crisis care or psychiatric inpatient care.
body weight). Patients may decline to take any medica- • If people’s physical health is at serious risk due to an
tion they are concerned may cause them to gain weight, eating disorder, they do not consent to treatment, and
for example. they can only be treated safely in an inpatient setting,
• When prescribing for people with an eating disorder take follow the legal framework for compulsory treatment in
into account the risks of medication that can compromise the Mental Health Act of 1983.
RESOURCES FOR PATIENTS AND CARERS Buscemi, N., Vandermeer, B., Hooton, N., et al. (2006). Efficacy and
safety of exogenous melatonin for secondary sleep disorders and
Websites sleep disorders accompanying sleep restriction: Meta-analysis. British
B-eat Charity, www.beateatingdisorders.org.uk. Medical Journal (Clinical Research Ed.), 332, 385–388.
AnorexiaBulimiaCare Charity, www.anorexiabulimiacare. Buszewicz, M., Pistrang, N., Barker, C., et al. (2006). Patients experi-
org.uk. ences of GP consultations for psychological problems: A qualitative
Network-ED, www.network-ed.org.uk. study. The British Journal of General Practice: The Journal of the
Men Get Eating Disorders Too, https://mengetedstoo. Royal College of General Practitioners, 56, 496–503.
co.uk.
Casacalenda, N., Perry, J., & Looper, K. (2002). Remission in major
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19
Urinary and Renal Problems
LINDSEY POPE
Urinary Tract Infections Difficulty Voiding
Uncomplicated Lower Urinary Tract Infection in Bladder Outflow Problems in Men and Benign Prostatic
Nonpregnant Women of Childbearing Age Hypertrophy
Cystitis/Uncomplicated Lower Urinary Tract Infection History
Which Midstream Specimen of Urine Results Are Abnormal? Examination
Self-Help Investigations
Urethral Syndrome aka Abacterial Cystitis Immediate Management
The Management of Those Not Needing/Wanting Referral
Lower Urinary Tract Infection in Men
After Viewing Investigation Results
Initial Assessment and Management
Review Patients at 6 to 12 Weeks if on an Alpha-Blocker;
Further Management
Otherwise at 3 to 6 Months
Acute Prostatitis
Chronic Prostatitis Risk Factors for Progression of Bladder Outflow Problems
in Men
Lower Urinary Tract Infection in Other Situations
Children Carcinoma of the Prostate
Pregnancy PSA Counselling and Interpretation
Postmenopausal Women Risk Factors for Cancer of the Prostate
Catheterized Patients Counselling for the Patient Who Requests PSA Screening
PSA Interpretation
Acute Pyelonephritis
Treatment Options
Urinary Incontinence
Urinary Stones
History
Once the Patient Is Discharged From Follow-Up
Examination
Investigations Asymptomatic Proteinuria in Men and Non Pregnant Women
Management of Primarily Stress Incontinence (Men and Interpretation
Women) Investigative Strategy if a Dipstick Shows Persistent
Management of Primarily Urger Incontinence (Men and Proteinuria + or ++
Women) Management
Management of Primarily Overflow Incontinence Asymptomatic Haematuria
Management of Incontinence in the Frail Elderly or Visible Haematuria
Cognitively Impaired Non-Visible Haematuria
Coping With Incontinence: Conservative Containment Investigative Strategy if Persistent Unexplained Microscopic
Advice for All Haematuria
Bedding Protection
Pads and Appliances Chronic Kidney Disease
Urinary Drainage Bags Assessment and Management of Chronic Kidney Disease
Urinary Catheters and Problem Solving Patients at Risk of Developing Chronic Kidney Disease
Infection Action to Be Taken on Finding an eGFR Under 60 or a
Bypass Normal eGFR With Other Signs of Kidney Damage
Catheterizing a Patient in Chronic Retention Further Management
340
CHAPTER 19 Urinary and Renal Problems 341
Urinary Tract Infections when treating the elderly, men, pregnant women and
catheterized patients. A single dose is less effective than
Uncomplicated Lower Urinary Tract Infection a 3-day course but may be justified when compliance is
in Nonpregnant Women of Childbearing Age likely to be a problem. Follow local guidelines where
available. When not available, for the 3-day course use
GUIDELINES trimethoprim 200 mg twice a day, although resistance
can be above 25% in some areas. Nitrofurantoin 50 mg
Urinary tract infection (lower)—in women. NICE clinical
four times a day, or 100 mg modified release twice daily,
knowledge summaries. Available at http://cks.nice.org.uk/ is an alternative where renal function is normal.
urinary-tract-infection-lower-women#!topicsummary. • Patients with resistant organisms may need a change of
Health Protection Agency. (n.d.). Diagnosis of urinary tract antibiotics. Women who are infected with unusual organ-
infection—quick reference guide. Available at www.hpa.org. isms (e.g., Proteus or Pseudomonas) need a repeat MSU
uk/webc/hpawebfile/hpaweb_c/1194947404720.
Scottish Intercollegiate Guidelines Network. (2006).
and further investigation if still present.
Management of suspected bacterial urinary tract infection in • Explain to the patient that symptoms should clear within
adults. SIGN clinical guideline 88. Available at www.sign. 7 days (i.e., may persist after the 3 days of antibiotics).
ac.uk/guidelines/fulltext/88/index.html. Advise the patient to return if symptoms are still severe
after 3 days or persist after 7 days.
• Prevention. In women with recurrent infection offer self-
help advice, as discussed later in the chapter.
Cystitis/Uncomplicated Lower
Which Midstream Specimen of Urine Results
Urinary Tract Infection Are Abnormal?
• Half of women who present with frequency and dysuria • Bacterial counts of 105/mL are significant and reported
do not have bacterial infection. Half of those with bacte- on MSU results, but lower counts may be found with
rial infections resolve within 3 days without antibiotics organisms that are difficult to culture (e.g., Staphylococ-
(Brumfitt et al., 1994). The key to management is in cus saprophyticus, Chlamydia, Gardnerella). Counts of 103
distinguishing an uncomplicated from a complicated and 104/mL should be repeated if the symptoms suggest
urinary tract infection (UTI) (acute pyelonephritis, unusual infection.
organism, structural predisposition, refractory, recurrent, • Organisms without cells can be ignored, except in pregnant
or systemic symptoms) and in avoiding unnecessary anti- women and young children. Cells without organisms need
biotics in those without infection or in the elderly with further investigation, unless the MSU was taken after
asymptomatic bacteriuria. antibiotics had been started.
• The management of uncomplicated lower UTI in • Repeat the MSU and refer if cells are still present.
women can be as effective over the telephone as if the • Females aged over 40 years old with a refractory UTI
patients are seen in person, although this must be done with haematuria or recurrent UTIs with haematuria need
with caution, given General Medical Council (GMC) a referral to a urologist.
advice on prescribing antibiotics in this manner.
Women with moderate or severe typical cystitis symp- Recurrence of Symptoms
toms can be treated based on history alone but where • Repeat the MSU, or do one for the first time, and assess
there is doubt or if the symptoms are mild, refractory whether the patient is suffering from:
or relapse, then it is better to do a urine dipstick test • relapse—the organism is the same;
and consider the need for a midstream specimen of • reinfection—a different organism is present; or
urine (MSU) test. • the urethral syndrome (see upcoming discussion).
• If positive for nitrites and/or leukocytes, assume that this Relapse
is infection and treat with antibiotics without sending • Treat, with an antibiotic to which the original organism
an MSU. Where positive for leukocytes but not nitrites, was sensitive, for 7 days.
remember that the symptoms may represent urethral • Look for a reason for the failure to clear the original
syndrome (see upcoming discussion). infection. Consider stones and chronic retention. Repeat
• If positive for protein and/or blood only, then consider the dipstick test for protein and haematuria.
the diagnosis and differentials further. Reinfection(s)
• If negative for all four tests, send an MSU for microscopy • Consider using a different antibiotic; the new organisms
and culture without starting antibiotics; 95% will have are likely to be resistant to the one originally used.
a negative MSU and do not require antibiotics at this Frequent Reinfection
stage (MeReC, 1995). Urethral syndrome is still pos- • Investigations. After more than three infections in a short
sible with these results. period of time, consider an ultrasound scan (USS); an
• If treating, give antibiotics for 3 days. This is as effective abdominal x-ray of the urinary tract; and urea, electrolytes,
as a 7-day course. Seven days are, however, appropriate and creatinine to rule out underlying abnormalities. If
normal, there is no need for specialist referral, but consider • ensure that sexual intercourse is not traumatic because,
prophylaxis. for instance, of lack of lubrication.
• Prophylaxis. Consider low-dose prophylaxis in those with • Do not treat with antibiotics, as overgrowth with lacto-
at least four attacks per year. Use nitrofurantoin (imme- bacilli and candida may be encouraged.
diate-release) 50 to 100 mg at night or trimethoprim • If symptoms are disabling, refer to a urologist. Urethral
100 mg at night for 6 months in the first instance. They dilatation is now only used when true urethral stenosis
will more than halve the number of attacks. If an infec- is found due to little evidence for its effectiveness otherwise
tion occurs while on prophylaxis, use a different drug as and potential for dilatation to cause periurethral fibrosis
treatment. A Cochrane review in 2012 failed to show leading to strictures.
significant benefit from cranberry products for preventing
UTIs and therefore did not recommend its use (Jepson,
Lower Urinary Tract Infection in Men
Williams & Craig, 2012).
Infections Occurring After Intercourse Initial Assessment and Management
• Advise emptying the bladder after intercourse. • Consider possible causes: prostate problems, congenital
• Give a single dose of antibiotics to be taken within 2 hours urinary tract problems, phimosis, previous urinary tract
of intercourse (e.g., trimethoprim 200 mg, off-licence use). surgery, immunodeficiency, or anal intercourse.
Asymptomatic Bacteriuria • Examine the abdomen (for a palpable bladder), the testes
• Asymptomatic bacteriuria in the elderly requires no treat- and epididymis to assess the extent of infection, the pros-
ment but does in pregnancy. tate, and the urethral meatus for discharge.
• Exclude diabetes with a fasting glucose.
Self-Help • Arrange for an MSU before, and 7 to 14 days after finish-
• Recommend that patients: ing, antibiotics.
• increase fluids; • Treat with antibiotics for 7 days in the first instance (tri-
• increase the frequency of micturition (practise double methoprim or nitrofurantoin) and review the MSU results.
voiding, i.e., attempting to pass urine a second time • If ill, admit.
immediately after the first);
• empty the bladder before sleep and after sexual Further Management
intercourse; • Consider USS and an abdominal x-ray to look for abnor-
• wear loose-fitting cotton underwear and avoid tights; malities of the urinary tract. This has been shown to
• avoid external sanitary towels; detect all pathology that would have been detected on
• make sure a diaphragm, if worn, fits comfortably (or intravenous pyelogram (IVP) as well as some that would
change contraceptive method); have been missed.
• use a lubricant (e.g., K-Y jelly) if vaginal dryness makes • If infection recurs, refer and consider low-dose prophylaxis
intercourse painful; until seen by a urologist.
• wipe from front to back (should be routine) and wash • Consider the possibility of a urinary tract cancer in men
the vulva with soapy water. with recurrent or persistent UTIs.
Note: Most episodes of dysuria in young men will be due
Urethral Syndrome aka Abacterial Cystitis to urethritis. Consider referral to a genitourinary medicine
(GUM) clinic.
• The diagnosis will have been made on the basis of repeated
attacks of frequency and dysuria with repeatedly sterile Acute Prostatitis
MSUs. There are no clear diagnostic criteria and it overlaps • Send an MSU, plus urethral swabs if there is any sugges-
with other diagnoses such as interstitial cystitis. tion of a sexually transmitted infection.
• Examine to exclude: • Start treatment with a quinolone antibiotic (cipro-
• vaginal infection, especially Candida, Chlamydia, Tricho- floxacin 500 mg twice a day or ofloxacin 200 mg twice
monas, Gardnerella, or gonorrhoea; a day) for 28 days. If these cannot be taken then tri-
• urethral herpes or warts; methoprim 200 mg twice a day for 28 days is recom-
• significant anterior prolapse; mended.
• atrophic vaginitis. • Give analgesics (e.g., paracetamol) or a nonsteroidal anti-
• Ask the patient to keep a diary of input, output, and inflammatory drug (NSAID) for symptomatic relief.
symptoms for 1 week, and consider managing as for detru- • If defecation is painful, offer a stool softener such as
sor instability (see Urge Incontinence, to come). lactulose.
• Self-help. Patients should: • Reassess after 48 hours.
• alkalinize the urine (e.g., with potassium citrate mixture); • Admit patients who are ill or in whom rectal examination
• avoid coloured toilet paper, scented soaps, bubble baths, suggests a prostatic abscess.
douches, antiseptics, talcum powder, vaginal deodor- • Following recovery, refer for investigation to exclude
ants, and deodorized tampons; underlying urinary tract abnormality.
• Trimethoprim 200 mg twice a day (off-label use).

Chronic Prostatitis Give a folic acid supplement if first trimester and
GUIDELINE do not give if folate deficient, taking a folate antago-
nist, or have been treated with trimethoprim in the
National Institute for Health and Care Excellence. (2015). past year.
Prostatitis—chronic. NICE clinical knowledge summaries.
Available at https://cks.nice.org.uk/prostatitis-chronic.
• Cefalexin 500 mg twice a day or 250 mg four times
a day.
• Repeat an MSU 7 days after completion of treatment,
and then at every antenatal visit until delivery.
• Acute pyelonephritis: Admit.
• Patients present with at least a 3-month history of pain • Second UTI during pregnancy: Refer to an obstetrician.
in the perineum or pelvic floor and lower urinary tract
symptoms. This diagnosis is made once other conditions Postmenopausal Women
have been excluded (e.g., UTI; benign prostatic hyperplasia • Recurrent UTIs are common after the menopause, affect-
[BPH]; cancer of prostate, bladder, or colon; urethral ing more than 10% of women. This is in addition to
stricture; calculus; pudendal neuralgia). Only 5% to 10% symptoms due to urogenital atrophy.
of patients with this symptom complex (perineal pain, • Oestrogen therapy. Prescribe low-dose oestrogen therapy
lower urinary tract symptoms, variable dipstick, and MSU in those with no contraindications. It has been shown to
results) have bacterial infection. change the colonization of the vagina and decrease infec-
• Establish the diagnosis: Order microscopy and culture tions. Topical treatment may be sufficient.
on the first part of the stream of the first urine passed in • Culture-negative cystitis. Organize a further culture if
the morning. Threads of white cells suggest prostatitis. symptoms persist, asking specifically for Ureaplasma
Prostatic massage is not usually done in primary care. urealyticum and Mycoplasma hominis. Consider treating
• If culture is positive: Treat with a quinolone (e.g., cip- with tetracycline or erythromycin for 3 months, if
rofloxacin 500 mg twice a day for 4 to 8 weeks). Repeat present.
the MSU at 4 weeks and advise an early review if • Refer for urodynamic investigation women not respond-
symptoms return. If a sexual infection was implicated, ing to the above measures or unsuitable for oestrogen.
check that the partner has been adequately assessed • Interstitial cystitis. Treatment is palliative and should be
and treated. decided by the urologist. The options are:
• If culture is negative: No treatment has been clearly shown • oral therapy with, for example, a tricyclic antidepres-
to aid resolution. Offer a trial of NSAIDs for symptomatic sant; or
relief and referral to a urologist if symptoms are not resolv- • intravesicular with, for example, botulinum toxin
ing. The value of referral is to confirm the diagnosis and injections.
assist in explaining it to the patient.
• If there are significant lower urinary tract symptoms then PATIENT SUPPORT
consider a 4–6 week trial of an alpha blocker (e.g. Tam-
sulosin). NICE advise not to prescribe an alpha blocker The Cystitis and Overactive Bladder Foundation, www.
cobfoundation.org.
and an antibiotic concurrently (NICE, 2015).
Lower Urinary Tract Infection in

Other Situations Catheterized Patients
Children • Of patients with an indwelling catheter, 90% have bac-
See Chapter 6, Children’s Health. teriuria after 17 days (Drug Therapy Bulletin [DTB],
1998). Dipsticks are of little value when catheterized,
Pregnancy and asymptomatic bacteriuria does not usually need
• All women are screened for bacteriuria at the first antenatal treatment.
visit. • Infection cannot be prevented by topical antimicrobials
• If asymptomatic bacteriuria is found then a second urine applied to the meatus, nor by irrigation with antimicro-
sample should be sent for culture. bials or antiseptics. The best defence against infection is
• If the second sample confirms asymptomatic bacteriuria to open the closed drainage system as infrequently as
then treat for 7 days with an antibiotic to which the possible.
organism is sensitive. • Give antibiotics for 7 days only if the patient is clearly
• If local guidelines are not available, options when sensi- symptomatic, if the infection is systemic (i.e., the patient
tivities are known (in order of preference): is febrile), or if the causative organism is Proteus. Proteus
• Nitrofurantoin 50 mg four times a day or 100 mg may give rise to triple-phosphate stones and is worth
(modified release) twice a day eradicating with antibiotics.
• If giving antibiotics, remove the catheter if the patient can • Check for other underlying conditions. Constipation and
manage without it for a few days. Insert a new one once UTIs are leading causes of incontinence in the elderly.
the urine is sterile. Neurogenic incontinence may present with a mixture of
• Consider whether intermittent catheterization by the symptoms. Cauda equina syndrome is a surgical emergency,
patient, or a suprapubic catheter, might be a better option. where results are poor when surgery is undertaken more
• Wash the meatus daily with soap and water. than 48 hours after first presentation (Markham, 2004).
• Only give antibiotics prophylactically if they are needed
to prevent endocarditis or if patients do badly with Examination
UTIs (e.g., many patients catheterized with multiple • Examine the abdomen, the genitals, and prostate in men,
sclerosis). with a pelvic examination in women, and a rectal exami-
nation in both sexes for constipation and for the integrity
Acute Pyelonephritis of the anal reflex.
• Proceed to the BPH guidance for further evaluation (dis-
• Admit if too ill to take oral fluids and medication, if the cussion to come) in men where this is the suspected cause
patient shows signs of sepsis, or if the patient is pregnant. of the incontinence, as well as performing the upcoming
Otherwise: investigations.
• arrange an MSU;
• prescribe a broad spectrum antibiotic (e.g. ciprofloxacin Investigations
500 mg twice daily for 7 days, or co-amoxiclav 625 mg • Dip the urine for sugar and evidence of infection. Con-
three times daily for 14 days); sider an MSU.
• consider the need for analgesics; • Frequency/volume diary.
• review the patient and the MSU result and confirm • Consider the use of a validated questionnaire for incon-
that the antibiotic was appropriate; tinence severity and quality of life (such as the King’s
• repeat the MSU 7 days after the antibiotics are finished; Health Questionnaire).
• follow-up: Consider a USS and an abdominal x-ray of • Consider a urinary USS to assess residual volume, espe-
the urinary tract. cially if there is a possibility that overflow incontinence
is present or if the patient does not respond promptly
Urinary Incontinence to the upcoming measures. A residual volume of 200 mL
or more is abnormal. In men a residual volume above
100 mL is associated with other problems relating
GUIDELINE to BPH.
National Institute for Health and Care Excellence. (2013, • Consider referral for urodynamic studies if the type of
updated 2015). Urinary incontinence in women: incontinence is not clear or if a trial of treatment fails.
Management. NICE clinical guideline 71. www.nice.org.uk.
• Refer to a urologist or gynaecologist, as appropriate, at this
stage if there is:
• a severe vaginal or uterine prolapse;
• Urinary incontinence effects both men and women across • a pelvic mass (to be seen within 2 weeks in the United
the spectrum of ages and can have a significant physical Kingdom as a suspected cancer);
and psychological impact on sufferers. • a vesical fistula;
• Many patients will not volunteer the problem to their GP. • persistent or recurrent infection;
• evidence of bladder outflow obstruction;
History • evidence of a large residual urine.
• Be ready to ask anyone about incontinence if they have • Refer to a neurologist if there appears to be a neurological
a condition which puts them at risk. A question such as, cause. The possibility of cauda equina syndrome or
“Do you ever have trouble holding your water?” is less myelopathy needs urgent discussion with the neurosur-
threatening than using the word incontinence. geons (or orthopaedic surgeons).
• Check, from the history, for other urinary symptoms.
Incontinence is usually part of a larger problem. Consider Management of Primarily Stress Incontinence
benign prostatic hypertrophy (BPH) in older men and (Men and Women)
perform an International Prostate Symptom (IPS) score
if indicated (see Appendix 24). • Reduce intraabdominal pressure by weight loss, the avoidance
• Identify the type of incontinence from the history: stress, of constipation, and reducing cough by stopping smoking.
urge, overflow, or continuous, or a mixture. Many suf- • Increase external sphincter tone (87% cured or improved)
ferers have at least two types. (US Department of Health and Human Services, 1992):
• Ask about precipitating events: excess fluids or alcohol, • by pelvic floor exercises for men and women. Teach
behavioural or cognitive problems, and poor mobility or the patient to practice stopping the flow of urine
access to a toilet. momentarily midstream and then continue tensing
those same muscles for 4 seconds at a time with 4 • Oestrogen given orally does not help incontinence in post-
seconds rest, for a total of 1 hour a day in 10 tighten- menopausal women (see earlier discussion), in contrast
ing bursts. Short, repeated bouts of exercises are the to the conclusions of a Cochrane review based on smaller
most useful. Continue for 3 months before deciding earlier studies (Moehrer, Hextall, & Jackson, 2003). The
that exercises have not helped; case for local oestrogen is unclear.
a. by using intravaginal weighted cones for women • Referral. Refer to a urologist for consideration of aug-
(available through urologists or local chemists). The mentation cystoplasty for those still sufficiently troubled
patient inserts the lowest weight cone, with the by symptoms despite treatment and who might be can-
pointed end downwards, and learns to retain the cone. didates for surgery.
The weight of the cones is steadily increased to 100 g;
b. by referral to an appropriate physiotherapist for
electrical stimulation therapy. PATIENT SUPPORT
• Postmenopausal women with an atrophic vagina. The The Cystitis and Overactive Bladder Foundation, www
Women’s Health Initiative has shown that oral oestrogen, .cobfoundation.org.
with or without progestogen, significantly worsened con-
tinence, both in those troubled by incontinence at the
start of the trial and in those who had no incontinence
at the start of the trial (Hendrix et al., 2005). No clearcut Management of Primarily Overflow
evidence exists for local oestrogen. Incontinence
• Referral. Refer those with troublesome symptoms that do
not respond to the measures above. Surgery cures or • Refer for assessment by a urologist, neurologist, or other
improves 85% of those operated on. Operations include specialist, according to the cause.
the older open abdominal retropubic suspension proce-
dures as well as the newer and less invasive suburethral Management of Incontinence in
sling procedures (including those using transvaginal tape). the Frail Elderly or Cognitively Impaired
• Consider prescribing duloxetine in addition to pelvic floor
exercises. It has an NNT for reduction of stress inconti- • Assess:
nence by over 50% of 5.7 (95%CI 4.5–7.8). However, • is it due to infection? Older people often do not experi-
most women report adverse effects, with nausea the most ence the typical symptoms of urinary infection. Send
common, and one in five women in trials stop the drug an MSU where possible;
(Anon., 2004). • is it a symptom of another physical illness or of dementia?
• are drugs causing or exacerbating the situation? Diuretics,
Management of Primarily Urger Incontinence sedatives, for example;
(Men and Women) • are there issues of practicality and mobility? How easy
is it to get to the toilet? Can mobility be improved?
• Reduce excessive fluid intake and try avoiding caffeine. Can clothing be made easier to unfasten?
• Bladder retraining results in up to 87% cured or improved • Encourage the patient to pass urine regularly to try to
(US Department of Health and Human Services, 1992). pre-empt the incontinence.
• Instruct the patient to:
• keep a frequency/volume chart for 1 week; Coping With Incontinence: Conservative
• return for discussion of the pattern the diary reveals. Containment Advice for All
In addition, check that the total volume of urine passed
does not suggest that the patient is drinking too much; • Where the situation does not respond to any of the above
• practise holding the urine when the urge to pass it is measures, a continence adviser will be able to advise the
there; and patient on appliances, some of which are prescribable.
• slowly increase the interval between voiding up to 2 Others may be available through the social services (includ-
to 3 hours. ing bath and laundry allowance).
• Anticholinergic drugs are helpful in up to 83% (Malone-Lee
et al., 2001) but are limited by side effects. Undertake Bedding Protection
a trial for 6 weeks and, if working, review again after 6 • Plastic mattress covers and one-way sheets can be bought if
months. Use oxybutynin, tolterodine, trospium, or propiv- not available from the community nursing service. They
erine; the last three are more expensive than oxybutynin not only increase comfort but also reduce bedsores.
but may have fewer adverse effects (Anon, 2001). Sixty
percent show marked improvement or cure in the pres- Pads and Appliances
ence of detrusor instability (Haeusler et al., 2002). • Inco pads may be available from the community nursing
• Desmopressin given as a single evening dose can be considered service.
when nocturia is a particular problem (off label use). • Cellulose wadding can be prescribed.
• Body-worn pads and waterproof pants usually have to be • Check that the bag is lower than the bladder; change the
bought (e.g., Urocare, Kanga, and Kanga pouch). catheter and see if the old one was blocked; use a small
• Collecting devices such as commodes, pans, and urinals bulb size (e.g., 5 mL); exclude and, if necessary, treat
can be bought from pharmacists or hired from the Red bladder stones and infection; and if there is no improve-
Cross or may be obtained via Social Services in the UK. ment use anticholinergic drugs (e.g., oxybutynin) (Foster,
• Penile sheaths are available on prescription. Conveen pro- Upsdell, & O’Reilly, 1990).
vides a free sizing kit and sheaths from 17 to 35 mm.
Catheterizing a Patient in Chronic Retention
Urinary Drainage Bags • Haematuria is inevitable if the bladder has been distended
• Assess the patient’s requirements: the length of tubing for some time. Clamping to release the urine in stages
(short for wearing on the thigh or long on the calf ); the does not prevent this.
type of tap (selection should depend on the patient’s • Diuresis may occur after the obstruction is relieved. Diuretic
dexterity); the capacity needed (350, 500, 750 mL). doses may need to be reduced. Admit patients whose
• Night bags hold 2 L and should only be used for bed- diuresis is severe.
bound patients or overnight.
PATIENT INFORMATION
Urinary Catheters and Problem Solving The Bladder and Bowel Foundation provides a continence
nurse helpline for medical advice: 0800 031 5412; www.
GUIDELINE bladderandbowelfoundation.org.
Incontinence Advisory Service, Disabled Living
National Institute for Clinical Excellence. (2012). Healthcare- Foundation, 34 Chatfield Road, Wandsworth, London SW11
associated infections: Prevention and control in primary and 3SE, tel. 020 7289 6111; helpline 0300 999 004; www.dlf
community care. NICE clinical guideline 139, updated 2017. .org.uk.
www.nice.org.uk. ERIC (Enuresis Resource and Information Centre for
children and young adults). 36 Old School House, Britannia
Road, Kingswood, Bristol BS15 8DB; helpline 0845 970
8008; www.eric.org.uk.
• Infection makes catheterization an unattractive solution
but one that is likely to be necessary in those with reten-
tion or neurogenic bladder dysfunction, with severe pres-
sure ulcers, who are terminally ill, or who cannot cope Difficulty Voiding
with less invasive appliances.
• Choose intermittent catheterization over an indwelling
Bladder Outflow Problems in Men and Benign
catheter if it is feasible. Prostatic Hypertrophy
• Catheters for long-term use should always be silicone with
a 10-mL balloon. GUIDELINES
• Catheter insertion should be performed using a no-touch National Institute for Health and Care Excellence. (2010). Lower
technique and clean, but not necessarily sterile, gloves. urinary tract symptoms in men: Management. NICE clinical
• Catheter changes should only be done when there is mal- guideline 97, updated 2015. Available at: www.nice.org.uk.
function or contamination or according to the manufac- National Institute for Health and Care Excellence. (2015).
turers’ recommendations.
• Urine samples should be taken from a sampling port, not
by disconnecting the bag, nor from the bag drainage outlet.
• Debris may be reduced by acidification of the urine with
ascorbic acid. Encrustation ends up affecting half of all • BPH is the most common cause of bladder outflow prob-
long-term catheters and is associated with infection by lems in men and occurs with increasing age and with
Proteus. When a patient starts to develop encrustation, variable symptoms.
plan a regular catheter change before the encrustation • Once symptoms are present, the rule of thirds applies with
becomes troublesome. one-third deteriorating, one-third remaining stable, and one-
third improving over time (Ball, Feneley, & Abrams, 1981).
Infection
• See page 343. Inflammation around the urethral meatus History
may be related to encrustation of the catheter and/or • Take a history for relevant symptoms and score them (see
infection. Appendix 24). BPH is very common, with 41% of men
over 50 years old reporting moderate or severe symptoms.
Bypass • Consider differential diagnoses: UTI, diabetes, prostatitis,
• This is caused by obstruction of the catheter or by detru- medications, or lifestyle factors such as fluid and alcohol
sor instability, not by the catheter being too small. intake.
• Identify whether the patient has: • Refer, at a timescale appropriate to the individual, those
• voiding/obstructive symptoms (poor flow, hesitancy, with chronic kidney disease (CKD) or with refractory
terminal dribbling, and incomplete emptying); symptoms affecting the quality of life.
• filling/overactive bladder symptoms (frequency, urgency,
urge incontinence, small volume urinating, and The Management of Those Not
nocturia). Needing/Wanting Referral After Viewing
• Is there nocturia out of proportion to other lower urinary Investigation Results
tract symptoms (LUTS)? Nocturia three or more times • Offer the patient a choice from the following options
a night raises the possibility of nocturnal polyuria, which according to how bothered he is by his symptoms and
can be defined as a nocturnal urine volume greater than whether he is at increased risk of progression of the
35% of the total 24-hour urine volume (Marinkovic, obstruction. Explain that surgery is by no means inevitable.
Gillen, & Stanton, 2004). This suggests a disturbance of In one study only 7% required surgery over the next 3
the normal diurnal excretory rhythm. It can occur in years (Wasson et al., 1995).
chronic renal disease, diabetes mellitus, diabetes insipidus, 1. Lifestyle advice and consider watchful waiting. This is a
right-sided heart failure, or can be an adverse effect of key first line approach to management. Advice includes
phenytoin, digoxin, lithium, diuretics, and excess vitamin advising a fluid intake reduced to 2 liters per day and
D. Any of these would need treatment followed by reas- avoiding caffeine. Recommend bladder training to
sessment of the LUTS. In the absence of a treatable cause those with filling symptoms. Briefly, this means gradu-
it may respond to restriction of fluid at night, diuretics ally lengthening the time between passing urine. Explain
in the morning, compression stockings for those with that avoiding excess fluid or alcohol intake, and avoid-
oedema, or desmopressin at night. ing constipation, will help to reduce the risk of acute
• Are there filling symptoms with no evidence of obstruc- retention. Review medications that might predispose
tion? Manage as for urge incontinence (see page 344). to retention (especially anticholinergics).
2. Drugs:
Examination • Use an alpha-blocker in those with mild symptoms
• Examine the abdomen for signs of chronic retention and without risk factors for progression and where a
pelvic or renal masses. quick symptomatic response is required. They are
• Consider performing a digital rectal examination to generally the first line drugs for BPH. If there is
examine for prostate size, to exclude sinister features, and benefit, it is felt within 4 to 6 weeks and lasts for
as an aid to management decisions. at least 3 years. A trial in the individual patient is
therefore feasible, using the IPS score to follow a
Investigations trend (see Appendix 24). All alpha-blockers share
• Perform a dipstick urinalysis. this effect; a considerable saving can be made by
• Test the blood for urea, creatinine and electrolytes, fasting choosing the least expensive. A reduction in BP
glucose and consider (with informed consent) a PSA, from the less selective alpha-blockers such as doxa-
though men with BPH are no more likely than others zosin might be beneficial in hypertensive patients.
to have prostate cancer. • Use a 5-α reductase inhibitor (finasteride, dutasteride)
• Consider ordering an ultrasound scan (with post void in those at higher risk of progression. Given to men
residual volume) where there is a suspicion of chronic with a large prostate or raised PSA, it will reduce
retention or an obstructive uropathy (e.g., palpable bladder, the risk of acute retention and the likelihood of
raised creatinine, overflow incontinence); uroflowmetry prostatectomy by 50% to 60%. Use the other risk
may also be available locally. Refer if the postvoiding factors to sway the decision in cases of doubt (Lepor
residual volume is greater than 100 mL. et al., 1996). No symptomatic benefit may be seen
for 3 to 6 months. If there is benefit, it is sustained
Immediate Management while the drug is continued. Libido and erection
• Admit those with acute urinary retention or acute kidney problems occur in 9%, and there are risks of gyn-
failure. aecomastia, reduced ejaculate volume, and presence
• Refer to be seen urgently (in the United Kingdom within of the drug in semen (such that condoms are
2 weeks) those with macroscopic haematuria (in the absence advised). PSA rates are, on average, halved, to be
of infection, or persisting after treatment of infection), a remembered when performing future PSAs.
nodular or firm or irregular prostate, a raised PSA for the • Use a combination of the two in those more troubled
patient’s age, or abnormal urinary cytology, where done. by their symptoms. A study of 3047 men with at
• Refer to be seen urgently (in the United Kingdom within least moderately symptomatic BPH found that clini-
2 weeks) those over 45 with persistent microscopic haema- cal progression of symptoms over 4 years occurred
turia. in 17% on placebo, in 10% on either finasteride
• Consider a non-urgent referral for men over 60 with or doxazosin, and in only 5% on both drugs (McCo-
recurrent or persistent UTIs. nnell et al., 2003).
• Antimuscarinics: Where BPH has caused a predomi-

nantly overactive bladder presentation consider these PSA Counselling and Interpretation
drugs in addition to bladder training. There is little (Public Health England, 2016)
evidence to suggest that they can precipitate acute • Population screening for cancer of the prostate is not
urinary retention even in the presence of severe BPH. recommended in the United Kingdom, but should be
• Saw palmetto: has only weak and conflicting evidence available for a man who requests it and is able to give
for efficacy in treating BPH symptoms. informed consent.
3. Surgery (usually transurethral resection of prostate, • A number of high profile campaigns have raised public
TURP) is much more effective than drugs in improving awareness of prostate cancer and PSA testing and GPs
symptoms and flow rates, but only in two-thirds of should be prepared to discuss the risks and benefits of
those who undergo it. It is most likely to be successful testing.
in those most bothered by their symptoms (Wasson
et al., 1995). It does, however, carry not only the short- Risk Factors for Cancer of the Prostate
term risks of any operation but also, in the case of • Age. Half of men aged over 80 years have prostate cancer
TURP (Brookes et al., 2002), the risk of incontinence but most are asymptomatic and will die of other causes.
in one-third although only 6% are bothered by it. After Guidance suggests that PSA screening should not be offered
TURP, 9% need reoperation within 5 years. The rates to an asymptomatic man over 75 years with less than 10
for sexual dysfunction vary according to the type of years life expectancy.
surgery but the main techniques all worsen ejaculatory • Family history. A family history of cancers of the prostate,
dysfunction (Emberton et al., 1996). breast, ovary, bladder, and kidney increase the risk.
• Race. Men of black ethnic origin have a rate that is double
Review Patients at 6 to 12 Weeks if on that of whites. Asian and Oriental men have the lowest
an Alpha-Blocker; Otherwise at 3 to 6 Months rates.
• Consider whether medications are working and tolerated,
whether further medications need to be used in combina- Counselling for the Patient Who Requests
tion, whether lifestyle advice should be repeated, and PSA Screening
whether a referral to a urologist is now indicated. • Explain the following points:
• Check creatinine and PSA annually. • A rectal examination is considered as well as the
blood test.
Risk Factors for Progression of Bladder • If screening is positive, a biopsy may be needed. The
Outflow Problems in Men biopsy itself is uncomfortable, risks infection, and is
followed by haematuria or haematospermia for up to
• Large prostate (>30 cc) 3 weeks in a third of patients. The mortality is 1 : 10,000.
• PSA >1.4 ng/mL regardless of age • Only about 25% of men who have a positive PSA
• Age >70 years turn out to have cancer. About 15% of men with a
• IPS score >7 (at least moderate severity) normal PSA may have cancer. Even biopsy will miss
• A low flow rate (<12 mL/sec) up to 20% of cancers.
• A postvoiding residual volume >100 mL on ultrasound • It is not clear that early treatment saves lives or
improves other outcomes; and the treatments that
Carcinoma of the Prostate may be offered can have potentially severe adverse
effects including effects on sexual functioning and
continence. Studies are conflicting on the benefits of
GUIDELINES screening, and the chief medical officer (CMO) of
National Institute for Health and Care Excellence. (2015). England has advised to continue to screen asymp-
Suspected cancer: Recognition and referral. NICE clinical tomatic men if they ask for it and are appropriately
informed. Where the patient has symptoms or signs
Prostate cancer: Diagnosis and management. NICE clinical suggestive of prostate cancer (see NICE guidance on
guideline 175. www.nice.org.uk. referring suspected cancer, earlier), PSA testing is more
likely to be useful. Consider performing a digital rectal
examination (DRE) and a PSA where there is unex-
plained haematuria, lower urinary tract symptoms,
• Prostate cancer raises difficult issues relating to screening erectile dysfunction, bone pain, low back pain, or
and the question of the value of treatment in the asymp- weight loss. Refer urgently patients with an abnormal
tomatic man in whom carcinoma is discovered inciden- DRE or where the PSA is rising or above the age-
tally. Symptomatic patients will benefit from referral. specific thresholds (see upcoming discussion). Repeat
• Nearly 10,000 die annually in the United Kingdom from the PSA after 1 to 3 months if borderline and refer
prostate cancer. urgently if rising.
• The stress and anxiety of knowing that you have or GUIDELINE

might have cancer is considerable and will affect life
insurance applications.
Renal or Ureteric Colic - Acute. NICE CKS. Available https://
cks.nice.org.uk
PSA Interpretation
1. The upper limit of normal rises with age and was revised
in the BAUS BPH 2004 guideline (see earlier): • Consider: a family history of urinary stones, dehydration,
• Age <50: 2.5 ng/mL urinary infection (especially by Proteus), hypercalcaemia,
• Age 50–59: 3.0 ng/mL hyperuricaemia, and a chronic obstructive uropathy.
• Age 60–69: 4.0 ng/mL • Analgesia. Use an NSAID parenterally (e.g., diclofenac
• Age 70+: 5.0 ng/mL 75 mg intramuscularly). Opioids may be necessary if that
2. Probability of cancer at different levels of PSA: fails; use morphine not pethidine.
• PSA 4–10: 25% • Check for microscopic haematuria with a dipstick. The
• PSA ≥11: 66% presence of haematuria supports the diagnosis but its
• PSA >60: usually indicates metastatic prostate cancer absence does to exclude it (NICE, 2015). The presence
3. Repeat a borderline level in 2 weeks (or according to local of nitrites suggests possible infection. Check MSU and
guidance); the result can alter by up to 30%. serum urea, electrolytes, and creatinine.
4. Double the result if the patient has been taking finasteride • Arrange, usually by urgent referral to urology within 7
or dutasteride for 6 months or more. Conversely, an days, urgent computed tomography of kidneys, urethra,
enlarged prostate due to BPH can double the PSA level bladder (CT KUB) or USS according to local guidelines:
without indicating cancer. • to establish the diagnosis;
5. Rectal examination does not raise the PSA but more • to assess the size, position, and number of stones;
invasive manoeuvres (even catheterization) can, as can • to look for obstruction.
UTI, prostatitis, and benign prostatic hypertrophy. • Admit for:
• uncontrolled pain;
Treatment Options • inability to drink adequate liquids;
This remains controversial. NICE guidance has been issued • infection;
(see earlier, NICE CG175) and there are various options: • complete unilateral or bilateral obstruction on intra-
• Active surveillance. Especially appropriate for men whose venous urogram (IVU);
life expectancy is less than 10 years and those reluctant • known renal insufficiency;
to face radical treatment. • known to have a single kidney.
• Radical (potentially curative) therapy. Surgery carries a risk • Refer to outpatients all those not requiring admission.
of incontinence that is mild in 4% to 21% and total in up • Instruct the patient to save any stone passed by passing
to 7%. Erectile dysfunction varies between 20% and 80%. urine through a filter (e.g., a woman’s stocking). Send it
• Hormone therapy or orchidectomy (androgen depriva- for analysis.
tion) is indicated for noncurable disease that is not organ-
confined. Early treatment in the form of androgen Once the Patient Is Discharged From Follow-Up
deprivation prolongs survival in advanced local and in • If conservative management has been chosen, support
metastatic disease. Hormone escape disease refers to a the patient with the information that 90% of small stones
rising PSA despite hormonal measures. The life expectancy (<5 mm) pass spontaneously and that 50% of stones
is about 6 months. Drug options are: that are 5 to 10 mm in diameter also pass. Although
• LHRH analogues (e.g., goserelin and leuprorelin), which dehydration is a risk factor for developing stones, a
can improve quality of life but not life expectancy. They Cochrane review in 2012 found that no recommendations
may also cause hot flushes, reduced libido, erectile dys- could be made with regards to water intake and preven-
function, gynaecomastia, and an initial tumour flare; tion of urinary tract stones based on current evidence
• nonsteroidal antiandrogens (e.g., flutamide and bicalu- (Bao & Wei, 2012).
tamide), which can be used as adjunctive therapy for • Check that an attempt has been made to find a cause (i.e.,
total androgen blockade. serum calcium and uric acid and a 24-hour urinary
calcium). If there is a family history or the patient has
Urinary Stones recurrent stones, the following should also be checked:
• 24-hour urine for pH, oxalate, phosphate, and uric acid
REVIEW • random urine for cystine
Holdgate, A., & Pollock, T. (2004). Nonsteroidal anti- • If an abnormality is found:
inflammatory drugs (NSAIDs) versus opioids for acute renal • Treat hypercalciuria and calcium phosphate calculi with
colic. The Cochrane Database of Systematic Reviews, (2), a low calcium diet and consider the use of bendroflu-
CD004137.
methiazide or potassium citrate.
• Treat hyperuricaemia and urate calculi with allopurinol.
• Explain the importance of avoiding dehydration. Management

• Give dietary advice for oxalate stones (avoid chocolate,
tea, rhubarb, spinach) and calcium stones (reduce • Protein +++ or more: refer promptly that day (by telephone
amount of dairy products). discussion with the nephrologists). The protein loss is
likely to be heavy enough to lead to nephrotic syndrome
(proteinuria >3.5 g/day or urinary albumin/creatinine
Asymptomatic Proteinuria in Men and ratio >300 mg/mmol).
Non Pregnant Women • Protein + or ++: Take an MSU and confirm that there is
persisting proteinuria with a second and a third sample 1
week apart, at least one taken on waking.
GUIDELINE • Refer less urgently to nephrology outpatients those:
Renal Unit, Royal Infirmary of Edinburgh (EdREN). (2010). GP • with a protein excretion of more than 1 g/day (consider
info: Proteinuria. www.edren.org (search on “proteinuria”). sending a request-for-advice letter if proteinuria is
between 150 mg and 1 g/day), or an albumin/creatinine
ratio of greater than 120 mg/mmol, even if they have
a primary condition which is likely to be the cause
Protein + or more on dipstick is likely to be significant, but (e.g., diabetes);
false positives (40%) and negatives (<20%) for urinary tract • with a protein excretion of 250 mg/L or an albumin/
disease occur. False positives can result from a sample being creatinine ratio of over 30 mg/mmol if they also have
highly concentrated or alkaline, or taken after exercise, during a raised creatinine or hypertension. Refer with more
a fever, during menstruation, or in the presence of vaginal urgency if the creatinine is clearly rising over weeks;
or urethral discharge. False negatives occur in dilute or acidic • with a decreased creatinine clearance;
urine or in non-albuminuric proteinuria. • with any proteinuria and a family history of renal disease.
• If proteinuria is present but does not meet the above
Interpretation criteria for referral, repeat the investigations 6-monthly
until it resolves or meets those criteria. Continue to
• Proteinuria which is absent on waking suggests orthostatic check the BP, serum creatinine, and a dipstick for hae-
proteinuria (but beware of diagnosing this in patients maturia.
>30 years old).
• Proteinuria that disappears on repeat testing may have
been idiopathic transient proteinuria, which is found Asymptomatic Haematuria
particularly below the age of 20 years, or may have been Visible Haematuria
associated with another medical condition (e.g., UTI or
heart failure). Repeat after a further 6 months. • Check that it is not due to menstruation or other
• Proteinuria that is intermittent but not showing one of the bleeding.
patterns above: investigate as for persistent proteinuria and, • Check the urine with a dipstick, send MSU for culture
if no grounds for referral are found, repeat the dipstick test, and blood for urea, creatinine, and electrolytes.
blood pressure, and serum creatinine 6- to 12-monthly. • Where the MSU is negative for red blood cells consider
• Persistent proteinuria: investigation is needed. beeturia, obstructive jaundice, and (rarely) porphyria.
However, if these are not present and repeat dipstick is
Investigative Strategy if a Dipstick Shows still positive, believe the dipstick. Red cells may have
Persistent Proteinuria + or ++ lysed on the journey to the laboratory.
• Refer any patient with painless macroscopic haematuria
• History, including urinary tract symptoms, family history urgently (via the 2-week pathway in the United Kingdom)
of renal disease, and medications. to a urologist. The exception is haematuria during a proven
• Examination, including abdomen and loins, blood pres- UTI, which does not need further investigation provided
sure, weight, and oedema. a test for haematuria 7 days after completing antibiotics
• Investigations: is clear. However, women aged over 40 with recurrent
• MSU and check the dipstick for haematuria (1+ is haematuria with UTIs, or refractory haematuria despite
significant) treating a UTI successfully, need an urgent urology refer-
• Urine for albumin/creatinine ratio (ACR) to quantify ral, and men with a first UTI may also need referring
the degree of proteinuria (see Appendix 26) even if the haematuria resolves (National Collaborating
• Serum urea, creatinine and electrolytes, and estimated Centre for Primary Care, 2004).
glomerular filtration rate (eGFR)
• Fasting blood sugar Non-Visible Haematuria
• Serum protein electrophoresis and urinary Bence Jones
protein if other features (anaemia, bone pain) suggest • Screening is not routinely recommended (poor specificity).
myeloma or the patient is over 50 years old • Aspirin, warfarin, etc., are not an excuse for haematuria.
GUIDELINE Chronic Kidney Disease

National Institute of Health and Care Excellence (2015).
Suspected Cancer: Recognition and Referral. NICE Guideline GUIDELINES
12. Available: www.nice.org.uk
Chronic kidney disease in adults: Assessment and
management. NICE clinical guideline 182. www.nice.org.uk.
• The patient should collect an early-morning urine sample
in a plain white bottle for dipstick analysis. False positives
occur after exercise, during menstruation or a UTI.
• An MSU is usually not necessary in primary care to confirm • The incidence of CKD is rising due to an ageing popula-
the non-visible haematuria. tion and increases in the incidence of the main causes
• A finding of 1+ or more is significant when persistent in (e.g., diabetes). There is an accompanying growing inci-
two of three early-morning urines over 4 to 6 weeks. Almost dence of the need for renal replacement therapy (RRT)
half of these patients will have glomerular disease, the pro- for end-stage CKD.
portion rising in the younger patient (Topham et al., 1994). • Most patients with CKD are asymptomatic. Nearly half
• Common causes are: of all patients with CKD are in the stage 3A/B area, with
• urologic: BPH, prostatitis, urologic cancers (especially much smaller numbers for stages 4 and 5.
>40 years old) or calculi; • The principal causes of CKD in the United Kingdom are
• renal: glomerular disease is relatively more common (from common to uncommon): diabetes mellitus, hyper-
in those under 40 years old, especially IgA nephropathy; tension (these two cause three quarters of all CKD),
polycystic kidney disease. vasculitis and glomerulonephritis, pyelonephritis, reno-
vascular disease, and obstructive uropathies. Adult poly-
Investigative Strategy if Persistent cystic kidney disease (APKD), medications, and amyloidosis
Unexplained Microscopic Haematuria are less common. CKD, diabetes, and proteinuria are
interlinked and are strong, independent risk factors for
• Always consider the history and examination. Is the patient cardiovascular disease and increased mortality.
ill or have red flags for urologic cancer? Urologic symptoms • Management should be based on an eGFR. The serum
+ haematuria makes a urology referral to exclude underly- creatinine alone is an insensitive measurement of renal func-
ing pathology appropriate. tion, only becoming abnormal after considerable renal
• Consider age, BP, and the presence of significant proteinuria. function decline. A study in primary care showed that using
A renal cause is more likely if the patient is under 40 years the eGFR increased the detection of CKD from 22% to
old, is unwell, or there is proteinuria, a raised BP, or ankle 85% (Akbari et al., 2004). NICE guidance aims to increase
oedema. Quantify the proteinuria. An albumin:creatinine the detection of CKD in at-risk groups and thus assist in
ratio (ACR) over 30 mg/mmol or a protein:creatinine ratio reducing the rates of associated cardiovascular disease and
(PCR) over 50 mg/mmol is significant. mortality, and in identifying and referring those at risk of
• Assess renal function by measuring serum urea, electrolytes, progression to end-stage CKD requiring RRT.
creatinine, and an eGFR. • Calculating eGFRs (mL/min/1.73 m2):
• Referral: Use the modification of diet in renal disease (MDRD)
• If the patient is 60 years or older consider urgent referral formula based on values for creatinine, urea, and albumin;
to exclude cancer if there is persistent non-visible hae- and the patient’s age, race, and gender. UK labs are now
maturia and either dysuria or a raised white cell count routinely providing eGFRs.
on blood test. Age is never an excuse for a low eGFR. Consider the
• If cancer is excluded, consider nephrology referral if whole picture, trend, and comorbidity when considering
the eGFR is persistently below 60, or the ACR is further assessment and management.
30 mg/mmol or more, or the PCR is 50 mg/mmol or
more, or the BO is 140/90 mm Hg or more.
Assessment and Management of Chronic
• If the patient is under 40 years old, consider nephrol-
ogy referral with appropriate urgency if necessary if Kidney Disease
any of the factors listed are present. Patients at Risk of Developing Chronic
• Where no cause is found after referral, monitor annually Kidney Disease
with BP, creatinine, eGFR, and an albumin:creatinine • Screen those with predisposing factors for CKD (e.g.,
ratio (ACR) or protein:creatinine ratio (PCR) urine test. hypertension, diabetes, cardiovascular disease, persistent
Consider urology re-referral if haematuria becomes symp- haematuria or proteinuria, nephrotoxic drugs, structural
tomatic or macroscopic. Consider nephrology re-referral renal problems or calculi, BPH, a family history of CKD
if the ACR or PCR deteriorate as above, or if the eGFR stage 5, or multisystem disease [e.g., systemic lupus ery-
falls persistently to under 30 or falls by more than 5 per thematosus (SLE)]).
year or more than 10 per 5 years (NICE CKD clinical • Perform annual serum creatinine, eGFR, and a PCR or
guideline, reference ahead). ACR. NICE supports the ACR over PCR.
Action to Be Taken on Finding an eGFR • Consider renal ultrasound if there is a family history of
Under 60 or a Normal eGFR With Other Signs of APKD, or there are obstructive urinary symptoms, per-
Kidney Damage sistent haematuria, significant progression of eGFR decline,
• Consider if the patient is ill, raising the suspicion of or possibly in CKD stages 4/5.
glomerulonephritis. If so, act according to the clinical • Monitor blood and urine tests according to the CKD
picture, not just the laboratory results. stage identified and in discussion with the patient
• Consider a repeat creatinine and eGFR with the patient (Table 19.1). Plan future monitoring to identify progres-
fasted and well hydrated, off oral NSAIDs. Consider past sion early. Most complications occur at eGFRs less than
creatinine levels to assess the stability of the result. Note 60. Check urea, creatinine, electrolytes, eGFR, BP, and
that the eGFR may fall by 25% as a result of taking an ACR or PCR at least annually. Check the calcium and
angiotensin-converting enzyme (ACE) inhibitor. If giving phosphate in CKD stages 4/5; check the haemoglobin
an ACE inhibitor, monitor closely, stop if over 25% dete- (Hb) in CKD stages 3B/4/5.
rioration in eGFR or over 30% rise in creatinine. Such • Be aware that for many people CKD will not progress.
deterioration raises the possibility of renal artery stenosis.
• Beware of misinterpreting the eGFR because of the fact Further Management
that serum creatinine is dependent on muscle mass. In • Consider referral: CKD stages 4/5 (for consideration of
those with high muscle mass the eGFR will be underes- RRT), ACR over 70 mg/mmol or PCR over 100 mg/
timated whilst in those with low muscle mass (including mmol (heavy proteinuria suggests progression and/or
amputees and paraplegics) it will be overestimated. Equally, underlying disease), ACR over 30 mg/mmol or PCR over
significant oedema and pregnancy cause problems with 50 mg/mmol with persistent haematuria (1+ on dipstick),
using the eGFR. significant eGFR deterioration (>5/year, >10/5 years),
• Beware of overinterpreting eGFR changes where values suspected renal artery stenosis or genetic kidney disease,
are greater than 60. Look instead for a greater than 20% or uncontrolled hypertension despite four antihyperten-
rise in the creatinine. sives.
• Repeat a new finding of eGFR less than 60 in 2 weeks • Medications review: Consider drugs that may be causing
and ensure three eGFRs are assessed over 3 months. At a reduced eGFR, and those whose dosage is affected by
least two under 60 suggest a true reduced eGFR and reduced renal function.
CKD. Consider the rate of progression and whether this • Consider the need for smoking cessation and healthy
is likely to be a problem, based on the patient’s age and lifestyle advice.
comorbidity. Significant progression is more likely if there • Control hypertension to 120 to 139/<90 mm Hg (120–
is also significant proteinuria. 129/<80 mm Hg with diabetes or ACR >70 mg/mmol).
• NICE now recommends using a cystatin C-based estima- Use an ACE inhibitor first line.
tion of eGFR on those who have an eGFR persistently • Give an ACE inhibitor (or angiotensin-2 receptor antago-
between 45 and 59 who have no other markers of renal nist) to maximal dosage if the patient has diabetes and
disease. If there are no other markers of renal disease and significant microalbuminuria; or ACR over 70 mg/mmol;
eGFR cystatin C is over 60, do not diagnose CKD. or CKD and hypertension and ACR over 30 mg/mmol.
TABLE
19.1 Classification and Key Actions for Chronic Kidney Disease
Stage of Kidney Disease GFR (mL/min/1.73 m2) Action

Stage 1. Evidence of kidney damage* ≥90 Address CVD risk factors, treat comorbidity. Monitor
but normal GFR annually
Stage 2. Evidence of kidney damage* 60–89 As above. Monitor annually
and mildly reduced GFR
Stage 3A. Moderately reduced GFR 45–59 Closer monitoring 6-monthly
Stage 3B. Moderately reduced GFR 30–44 Check for complications; treat/refer. 6-monthly monitoring
Stage 4. Severely reduced GFR 15–29 Refer to a nephrologist to consider RRT. 3-monthly
monitoring
Stage 5. Established kidney failure <15 RRT possibly indicated (haemodialysis, peritoneal dialysis,
transplantation). 6-weekly monitoring
Cardiac risk can be calculated over 10 years using a validated tool such as QRisk.
*Kidney damage: persistent proteinuria, haematuria, anatomic abnormalities (e.g., polycystic or scarred), shrunken kidneys, biopsy-proven glomerulonephritis.
CVD, Chronic kidney disease; GFR, glomerular filtration rate; RRT, renal replacement therapy.
Adapted From NICE Guidance.
• Optimize the management of risk factors for CKD pro- Foster, M. C., Upsdell, S. M., & O’Reilly, P. H. (1990). Urologi-
gression: CVD, significant proteinuria, hypertension, cal myths. British Medical Journal (Clinical Research Ed.), 301,
diabetes, smoking, chronic use of oral NSAIDs, and 1421–1423.
urinary outflow obstruction. Asian and black ethnicity Haeusler, G., Leitich, H., van Trotsenburg, M., et al. (2002). Drug
are also risk factors for progression. therapy of urinary urge incontinence: A systematic review. Obstetrics
and Gynaecology, 100, 1003–1016.
• Anaemia (Hb <11 g/dL), acidosis, and metabolic bone
Hendrix, S. L., Cochrane, B. B., Nygaard, I. E., et al. (2005). Effects of
disease are all complications increasingly prevalent from
estrogen with and without progestin on urinary incontinence. JAMA:
CKD stage 3B downward and may require referral or The Journal of the American Medical Association, 293, 998–1001.
primary care-initiated assessment, dependent upon local Jepson, R.G., Williams, G, & Craig, J.C. (2012). Cranberries for Treat-
guidance. In CKD stages 3/4/5 iron deficiency anaemia ing Urinary Tract Infections. The Cochrane Database of Systematic
is likely at ferritin levels below 100 µg/L. NICE has pub- Reviews, (10), CD001321.
lished guidance on anaemia in CKD, and after excluding Lepor, H., Williford, W. O., Barry, M. J., et al. (1996). The efficacy
other causes iron can be given to get the ferritin to above of terazosin, finasteride, or both in benign prostatic hyperplasia.
200 µg/L. If this fails refer for consideration of erythro- Veterans Affairs Cooperative Studies Benign Prostatic Hyperpla-
poietin therapy aiming in adults for a Hb of 10.5 to sia Study Group. The New England Journal of Medicine, 335,
12.5 g/dL. 533–539.
Malone-Lee, J. G., Walsh, J. B., Maugourd, M. F., et al. (2001).
• If monitoring shows an ACR over 70 mg/mmol or PCR
Tolterodine: A safe and effective treatment for older patients with
over 100 mg/mmol no repeat is needed; this is heavy
overactive bladder. Journal of the American Geriatric Society, 49,
proteinuria requiring referral. However, an ACR 30 to 700–705.
70 mg/mmol needs to be repeated on the first-morning Marinkovic, S. P., Gillen, L. M., & Stanton, S. L. (2004). Manag-
sample to confirm the proteinuria. Haematuria is also ing nocturia. British Medical Journal (Clinical Research Ed.), 328,
grounds for referral when persistent. In diabetes an ACR 1063–1066.
over 2.5 mg/mmol in men and over 3.5 mg/mmol in Markham, D. E. (2004). Cauda equina syndrome: Diagnosis, delay
women is significant and needs repeat confirmation on and litigation risk. MDU, 20(1), 12–15.
an early-morning ACR. McConnell, J. D., Roehrborn, C. G., Bautista, O. M., et al. (2003).
Note: The above plan assumes that the patient is not The long-term effects of doxazosin, finasteride, and combination
suffering from another condition that makes an active therapy on clinical progression of benign prostatic hypertrophy.
The New England Journal of Medicine, 349, 2387–2398.
approach inappropriate.
MeReC. (1995). Urinary tract infection. MeReC Bulletin, 6(8), 29–32.
(See also the Health Protection Agency guideline referenced at the
References top of Urinary Tract Infections section).
Moehrer, B., Hextall, A., & Jackson, S. (2003). Oestrogens for urinary
Abrams, P. (1995). Managing lower urinary tract symptoms in incontinence in women. Cochrane Database of Systematic Reviews,
older men. British Medical Journal (Clinical Research Ed.), 310, (2), CD001405.
1113–1117. National Collaborating Centre for Primary Care. (2004). Referral guide-
Akbari, A., Swedko, P. J., Clark, H. D., et al. (2004). Detection of lines for suspected cancer; draft for second consultation. Retrieved from
chronic kidney disease with laboratory reporting of estimated www.nice.org.uk/pdf/RSC_2ndcons_Full_version.pdf.
glomerular filtration rate and an educational program. Archive of National Institute for Health and Care Excellence. (2015). Suspected
Internal Medicine, 164, 1788–1792. cancer: Recognition and referral. NICE clinical guideline 12. Retrieved
Anon. (2001). Managing incontinence due to detrusor instability. from www.nice.org.uk.
Drug and Therapeutics Bulletin, 39, 59–64. O’Brian, J., & Long, H. (1995). Urinary incontinence: Long-term
Anon. (2004). Duloxetine for female stress urinary incontinence. effectiveness of nursing intervention in primary care. British Medical
Bandolier, 129, 4–5. www.jr2.ox.ac.uk/bandolier. Journal (Clinical Research Ed.), 311, 1208.
Ball, A. J., Feneley, R. C., & Abrams, P. H. (1981). The natural Public Health England. (2016). PSA testing and prostate cancer: Advice
history of untreated “prostatism”. British Journal of Urology, 53, for well men aged 50 and over.
613–616. Stothers, L. (2002). A randomized trial to evaluate effectiveness and
Bao, Y., & Wei, Q. (2012). Water for preventing urinary stones. The cost effectiveness of naturopathic cranberry products as prophylaxis
Cochrane Database of Systematic Reviews, (6), CD004292. against urinary tract infection in women. The Canadian Journal of
Brookes, S. T., Donovan, J. L., Peters, T. J., et al. (2002). Sexual dys- Urology, 9, 1558–1562.
function in men after treatment for lower urinary tract symptoms: Topham, P. S., Harper, S. J., Furness, P. N., et al. (1994). Glomerular
Evidence from randomised controlled trial. British Medical Journal disease as a cause of isolated microscopic haematuria. The Quarterly
(Clinical Research Ed.), 324, 1059–1061. Journal of Medicine, 87, 329–335.
Brumfitt, W., & Hamilton-Miller, J. M. T. (1994). Consensus view- US Department of Health and Human Services (1992). Urinary incon-
point on management of urinary infections. Journal of Antimicrobial tinence in adults. Rockville, MF: Agency for Health Care and Policy
Chemotherapy, 33, S147–S153. Research (AHCPR).
Drug Therapy Bulletin. (1998). Managing urinary tract infection in Wasson, J. H., Reda, D. J., Bruskewitz, R. C., et al. (1995). A com-
women. Drug and Therapeutics Bulletin, 36, 30–32. parison of transurethral surgery with watchful waiting for moderate
Emberton, M., Neal, D. E., Black, N., et al. (1996). The effect of symptoms of benign prostatic hyperplasia. The Veterans Affairs
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Journal of Urology, 77, 233–247. The New England Journal of Medicine, 332, 75–79.
20
Ear, Nose, and Throat Problems
ADAM STATEN
The Ear Nasal Polyps
Pain in the Ear The Common Cold
Acute Otitis Media (AOM) Rhinitis
Mastoiditis Seasonal and Perennial Rhinitis
Acute Furunculosis of the External Canal Intermittent Mild Symptoms or Acute Response to Allergen
Bloody Discharge (Bullous Myringitis) Regular Symptoms
Herpes Zoster (Ramsay Hunt Syndrome) Nasal Blockage
Discharge From the Ear Severe Symptoms
Otitis Externa Snoring and Obstructive Sleep Apnoea (OSA)
Chronic Otitis Media (COM) Snoring Without Apnoea
Hearing Loss The Throat and Mouth
Otitis Media With Effusion (OME)
Sore Throat
Hearing Tests
Management
Dizziness Special Cases
Vertigo
Vincent’s Angina
Examination
Treatment Infectious Mononucleosis
Vestibular Rehabilitation Exercises Oral Thrush
Ménière’s Disease Sore Throat Due to Postnasal Drip
Refer to an ENT Consultant Tonsillectomy
Medical Treatment: Acute Indications for Referral
Prophylactic Dysphagia
Benign Paroxysmal Positional Vertigo (BPPV) Hoarseness
The Hallpike Test
Postlaryngectomy
Management of BPPV
Instructions for Performance of the Modified Epley The Feeling of a Lump in the Throat
Manoeuvre Sore Mouth
Unsteadiness Red or White Patches in the Mouth
Lightheadedness Ulcerated Sore Mouth
Tinnitus Aphthous Ulcers
Treatment Sore Mouth Without Ulceration
Facial Palsy (Lower Motor Neurone) Dental Problems
Toothache
Bell’s Palsy
Loss of a Tooth Through Trauma
Referral
Complications After Extraction
Management in Primary Care
Halitosis
The Nose
Rhinosinusitis
Management of Acute Rhinosinusitis
Management of Chronic Rhinosinusitis
354
CHAPTER 20 Ear, Nose, and Throat Problems 355
• Antibiotics should be considered in patients who are systemi-

The Ear cally unwell, those whose symptoms are not improving after
Pain in the Ear 4 days, children under 2 years of age with bilateral AOM,
and children with perforation and/or discharge in the canal.
• Examine the external canal and drum. If these are normal • In older children discuss with the parents the question
then the pain is very unlikely to be due to ear disease. of whether to prescribe an antibiotic. Explain that the
• Consider: evidence suggests that it is unlikely to be of benefit unless
• temporomandibular (TM) joint pain; the AOM is bilateral or there is otorrhoea. Recommend
• dental causes; watchful waiting or prescribe amoxicillin, suggesting that
• cervical lymphadenopathy; they only use it if symptoms persist after 3 days.
• sinus pain; • If a tympanostomy tube is in place, give antibiotics more
• mumps; readily. They can more than halve the duration of the
• cervical spine pain; illness (from 8 to 3 days) with an number needed to treat
• pharyngeal tumour, oral cancer, tonsil cancer. Any (NNT) of 3 for freedom from discharge at 1 week
unilateral ear pain with normal ear drum in a smoker (Ruohola et al., 2003). Use a topical drop such as cipro-
should be suspicious for tonsil carcinoma. floxacin first rather than oral antibiotics and if no help
use a topical drop and oral antibiotics for 1 week before
Acute Otitis Media (AOM) considering referral to ENT emergency clinic for review.
Diagnosis • Review in 4 to 6 weeks for deafness and/or effusion. Half of
• Much commoner in children, especially less than 10 years all children will have an effusion at 1 month and 10% will
of age. have an effusion at 3 months. Persistent fluid is not an indica-
• Usually presents with acute onset of otalgia. tion for further antibiotic treatment (van Buchem et al., 1981).
• Younger children may present with pyrexia, crying, irri-
tability, and rubbing the ear. Referral to ENT
• The tympanic membrane is red and bulging. • Children younger than 3 months with a temperature of
• There may be discharge if the tympanic membrane per- 38oC or more.
forates, when the pain becomes less. • Suspected complications such as mastoiditis or meningitis
• Acute otitis media (also known as serous otitis media) or facial nerve palsy.
should be distinguished otitis media with effusion and • The patient has five or more attacks of AOM in one
myringitis (rare). winter, or six or more in 1 year.
Management Acute Otitis Media Prevention

• Eighty-five percent of children with acute otitis media • Avoid cigarette smoke exposure (Strachan & Cook, 1998).
(AOM) and otalgia are free of pain within 24 hours, without • Encourage breastfeeding.
antibiotics (van Buchem, Dunk, & Van’t Hof, 1981). • Feed upright where possible.
• It can be caused by viruses or bacteria.
• Most patients do not need antibiotics as symptoms usually Mastoiditis
resolve within a few days. • Admit any patient with:
• Antibiotics reduce pain and fever at 3 to 7 days in infants • tenderness, redness, or oedema over the mastoid, or a
and children under 2 years old and in those with bilateral new onset of a protruding ear;
AOM (Rovers et al., 2006). They reduce the risk of devel- • vertigo, vomiting, increasing deafness, and nystagmus;
oping contralateral AOM but have no effect on pain in or
the first 2 days, and no effect on the incidence of deafness • acute otitis media with continuing fever despite
at 1 month. antibiotics.
• If an antibiotic is required, the recommended first line
is amoxicillin for 5 days. If there is penicillin allergy then Acute Furunculosis of the External Canal
use erythromycin or clarithromycin. • Early cases respond to antibiotic/corticosteroid drops.
• Writing a prescription for antibiotics but suggesting to • Surrounding cellulitis needs oral antibiotics as well (e.g.,
the parents that they only use it if the otitis media has flucloxacillin or erythromycin).
not resolved in 3 days can reduce the number taking • Exclude diabetes.
antibiotics to a quarter with only minor delay in the
resolution of symptoms. Parents given a delayed prescrip- Bloody Discharge (Bullous Myringitis)
tion are less likely to believe that they need to consult a The patient usually suffers severe pain followed by bleeding.
doctor in future episodes (Little et al., 2001). A number of serosanguinous bullae appear on the tympanic
• Pain and fever should be managed with paracetamol or membrane or surrounding skin.
a nonsteroidal anti-inflammatory drug (NSAID; e.g., • Prescribe adequate analgesia and advise the patient to
ibuprofen). keep the canal dry.
• Reserve broad-spectrum antibiotics for patients with middle topical antibiotics. The canal is filled with blackish spots
ear effusions (Marais & Dale, 1997). or brownish-cream deposits.
• Consider topical drops containing steroids to reduce • Swab for bacteria and fungi.
inflammation & therefore pain. • Consider the need for microsuction and referral to ENT.
• Refer if the patient suffers persisting pain or hearing loss • Treat with clotrimazole solution 1% instilled three times
(Biedlingmaier, 1994). a day and continued for 14 days after the disappearance
of obvious infection.
Herpes Zoster (Ramsay Hunt Syndrome)
As well as a vesicular eruption of the auricle or external Malignant Otitis Externa
auditory meatus, the patient may develop facial palsy or • This is an uncommon condition where infection spreads
hearing loss and vertigo. into the bone of the mastoid and base of skull.
• Treat as for shingles anywhere, with oral acyclovir and • Patients present with pain more severe than expected
adequate analgesia. from examination.
• Arrange urgent (on-call) review by ENT for consideration • It is most commonly presents in older diabetics or immu-
of IV anti-virals particularly if their is any VII or VIII nocomprimised, and pseudomonas is the most common
nerve dysfunction. pathogen.
• Suspected cases should be referred urgently as it is poten-
tially fatal and requires specialist management.
Discharge From the Ear
Otitis Externa Chronic Otitis Media (COM)
• Otitis externa is inflammation of the skin of the external • Chronic otitis media (COM) can be active or inactive:
auditory canal. Itch is a common presenting symptom. • Active COM has inflammation and mucopurulent
• Common predisposing factors are abuse of the ear with discharge.
cotton buds and any scaling skin disorder (e.g., eczema, • Inactive COM has a perforation or a retraction pocket
psoriasis). with no discharge.
• Discharge may be purulent but not mucopurulent. Muco- • There are two main types of chronic otitis media: mucosal
purulent discharge orignates in the middle ear, not the and squamous.
external ear.
• The principles of management: Mucosal Chronic Otitis Media
• Correct predisposing factors. • There is a perforation of the tympanic membrane.
• Topical steroids are effective in dermatitis from cotton • If active, ideally the discharge should be cleaned by micro-
bud abuse. Hydrocortisone cream applied by the tip suction or by mopping with cotton wool.
of a finger is often effective and can be reused inter- • Topical antibiotics are more effective than systemic anti-
mittently if symptoms recur. biotics in the management of active COM (Macfadyen,
• If there is profuse discharge, prescribe ear drops con- Acuin, & Gamble, 2006).
taining antibacterial agents and steroid (van Balen et al., • Although manufacturers advise against using topical prepa-
2003). If treatment is still needed after 1 week, change rations containing aminoglycosides in the presence of a
to steroid drops alone. perforation, the consensus opinion of ENT-UK is that
• Tell the patient that recurrence is likely if the ear canal antibiotics such as gentamicin can be used in active COM
is scratched, cotton buds are used, or water enters the but for a maximum of 2 weeks (Phillips et al., 2007).
canal. When contact with water is unavoidable (e.g., • Cases where otorrhoea does not settle and cases where
showering), the meatus should be plugged with cotton there is recurrence of otorrhoea should be referred to a
wool impregnated with petroleum jelly. specialist. Surgery to close the perforation (tympanoplasty)
• Make sure that patients prone to recurrent attacks is often very effective management.
have appropriate medication at home to use at the
onset of symptoms. Squamous Chronic Otitis Media—Cholesteatoma
• If the canal is severely swollen: • In squamous chronic otitis media there is a retraction
• Refer for the insertion of an antibiotic/corticosteroid pocket into the middle ear and mastoid with collection
wick or insert 0.5-inch ribbon gauze soaked in steroid of squamous debris. In simple terms, this is skin growing
cream. Change it every 24–48 hours. into the middle ear with the accumulation of dead skin
• Give oral antibiotics if there is cellulitis of the sur- cells. The debris gets infected, which causes inflammation
rounding tissues. and erosion of surrounding tissues.
• The potential complications are hearing loss, facial palsy,
Refractory Otitis Externa meningitis, and intracranial abscess.
• This is more likely if there is a history of eczema or sebor- • The only effective treatment for active squamous chronic
rhoeic dermatitis. It is usually due to undiagnosed Candida otitis media is surgery. This usually entails some form of
or Aspergillus infection and may follow prolonged use of mastoid surgery.
• All cases of suspected cholesteatoma should be referred • Syringe if the wax is soft.
to a specialist. • Disimpact hard wax with a wax hook, then syringe
the rest if necessary.
Hearing Loss • Instill drops (tap water is as effective as anything) and
syringe after waiting for at least 15 minutes (Browning,
• At least one-third of patients over 70 and half of patients 2005; Hand & Harvey, 2004).
over 80 have hearing loss severe enough to be helped by • Suggest that the patient instills ear drops into the ear
a hearing aid (i.e., roughly a 35-decibel [dB] hearing loss canal for 3 to 7 nights and then re-attends for syring-
at speech frequencies). ing. Instillation of either an oil-based or a water-based
• All hearing-impaired people, particularly the elderly, may solvent for 1 week will clear wax in 20% of patients
become isolated, depressed, and difficult to live with (Keane et al., 1995) and facilitate syringing in the
(Hickish, 1989). others. There is no evidence of the superiority of com-
• Hearing loss is: mercial drops over water or sodium bicarbonate (Burton
• sensorineural (lesion of the inner ear or of cranial nerve & Doree, 2009).
VIII); or • Refer all patients with conductive hearing loss not due
• conductive (middle or outer ear). to wax.
• The appearance of the ear is an effective guide to the type
of hearing loss. Otitis Media With Effusion (OME)
Fluid in the middle ear without signs or symptoms of inflam-
Sensorineural Hearing Loss mation can result in hearing loss.
• Examine the drum and remove any wax that may be
contributing. Children
• Refer patients with: • This is also known as secretory otitis media, or glue ear.
• unilateral hearing loss of acute onset. If the patient is • Half of affected children resolve spontaneously within
seen within 3 days of onset, arrange for outpatient 3 months (American Academy of Pediatrics, 1997).
assessment within 24 hours. All patients with sudden However, 2% to 3% of children under 7 have a more
sensorineural hearing loss should receive steroids (under prolonged hearing loss due to OME and need specialist
specialist guidance). assessment.
• unilateral hearing loss with a longer history. Referral is • A period of 3 months of watchful waiting is appropriate.
less urgent, but the patient may have a treatable cause • Even those having specialist assessment are unlikely
(e.g., acoustic neuroma). to be helped by surgery, the benefits of which are
• Be active in detecting hearing loss and encourage patients only temporary. Watchful waiting and immediate pre-
to consider a hearing aid. surgery testing may reduce inappropriate surgery (NHS
• All patients who have noticeable hearing difficulty during Centre for Reviews and Dissemination, 1994). For
consultation should be referred to audiology. instance, ventilation tubes (grommets) improve hearing
• The whispered voice test is useful for assessing the 6 months after insertion but very little after 12 months.
significance of hearing impairment, particularly in Adenoidectomy is recommended with the second set
those who think their hearing is fine (Swan & Browning, of ventilation tubes and has been shown to prolong
1985). the disease free time period before recurrence. Adenoid-
• Refer patients for a hearing aid early rather than late, and ectomy improves hearing, but for only up to 2 years.
encourage them to persevere with it once supplied. Tonsillectomy and/or myringotomy does not help
Hearing-impaired patients lose the ability to discriminate hearing.
between sounds and so have to relearn this skill. They • OME is more common in children whose parents smoke
may take some time to get the full benefit from an aid. in the same room.
One follow-up in the United Kingdom at between 8 and • Antibiotics. There may be some benefit from antibiotics
16 years found that fewer than half were still using an in the short term but the effect is small, not maintained,
aid (Gianopoulos, Stephens, & Davis, 2002). and outweighed by side effects (Scottish Intercollegiate
Guidelines Network [SIGN], 2003).
Conductive Hearing Loss • Antihistamines and decongestants, orally or topically,
• Assume that all conductive deafness is treatable. This is are of no value and may cause harm (Griffin & Flynn,
true whether the drum looks normal, as in otosclerosis, 2011).
or abnormal, as in middle ear effusion or COM. • Steroids. They are not recommended. There is evidence
• Examine the ear canal and tympanic membrane. that oral steroids lead to quicker resolution of OME in
• If wax is present and occluding the ear canal it may be the short term but there is no evidence of longer term
contributing to the hearing loss, although removal of wax benefit (Simpson et al., 2010). There is no evidence of
only raises the hearing threshold by more than 10 dB in benefit from topical intranasal steroids (Simpson et al.,
one-third (Memel et al., 2002): 2010).
• Refer to the community audiology service to determine: not necessary. It is important to distinguish between vertigo,
▪ severity; unsteadiness, and the sensation of lightheadedness.
▪ that the hearing loss is conductive;
▪ the overall disability. Vertigo
• Refer direct to ENT outpatients if hearing loss is: Vertigo is the illusion of movement of the subject or of their
• persistent (i.e., more than 3 months); or surroundings, most commonly rotatory.
• severe (i.e., a hearing loss >25 dB or the child is having
difficulties with hearing, speech, learning, or social Episodic
functioning because of it); and • Vertigo lasting only a few seconds or minutes indicates a
• associated with persistent pain. short-lived depression or stimulation of the labyrinth or
• If a child has other disabilities, refer if there is any degree central connections. It is present on sudden changes in
of hearing loss because normal hearing is crucial. posture commonly due to benign paroxysmal positional
vertigo (BPPV; see upcoming discussion).
Adults • Vertigo lasting a few minutes to a few hours indicates a
• OME may follow an upper respiratory tract infection physiological or metabolic disturbance of the labyrinth.
(URTI) or barotrauma. It occurs in Ménière disease.
• Prescribe ephedrine nose drops to be instilled into the
nasopharynx twice daily for 5 days and followed by auto- Prolonged
inflation with a Valsalva manoeuvre after 15 minutes. Vertigo lasting more than 24 hours. The clinical picture is of
• Prescribe a steroid nasal spray if there is generalized severe incapacitating vertigo associated with nausea and
rhinitis. vomiting, and is due to:
• Refer if the effusion persists for more than 6 weeks. A • peripheral vestibular pathology or deficit, usually vestibular
nasopharyngeal tumour must be excluded, especially in neuronitis, in which there is no hearing loss (Cooper,
Chinese patients. 1993) or Ménière’s;
• a central cause usually associated with other signs, as in
Ventilation Tubes (Grommets) multiple sclerosis, stroke, a posterior fossa tumour, or
• The majority of ventilation tubes are extruded in 3 to 9 secondary deposits in the brain stem.
months, and only need to be reinserted if hearing loss
recurs. Examination
• Discharge should be treated by aural toilet and antibiotic/ The following should be included when relevant:
corticosteroid drops (see mucosal chronic otitis media). • Taking the patient’s temperature and testing for neck
• Swimming and bathing pose no significant risk but diving stiffness in the acute attack
should be banned. • Inspection of the ear drums for acute or chronic otitis media
• Testing for (or at least enquiring about) hearing loss in
Hearing Tests acute vertigo or possible vestibular schwannoma
The Whispered Voice Test • Looking for nystagmus (In peripheral lesions the nys-
This is a good screening test and is easily performed (Eekhof tagmus will be horizontal with the fast phase toward
et al., 1996) with 90% to 100% sensitivity and 70% to the healthy side. Looking toward that side will
87% specificity in adults. In children the sensitivity is slightly increase the amplitude of the nystagmus. In central
less but the specificity is higher (Pirozzo, Papinczak, & lesions the nystagmus may be horizontal, vertical, or
Glasziou, 2003). rotatory.)
1. Cover one of the patient’s ears with a finger in the • Looking for other neurological signs, especially cerebellar
meatus. Move it gently to and fro to mask the whisper in and cranial nerve signs and papilloedema, as in vestibular
that ear. schwannoma or other central lesions. Rhomberg and
2. Stand at arms’ length with your head behind the patient’s Unterberger tests should be performed.
other ear.
3. Two correct responses are required to pass. Whisper, Treatment
then ask the patient to repeat what he or she has Acute Vertigo
heard: for children under 6 years old use familiar terms • Give prochlorperazine 12.5 mg intramuscularly followed
(e.g., “bread and butter” or “Father Christmas”); for by 25 mg suppositories 8-hourly until the vomiting stops.
children over 6 years old use multisyllabic numbers Continue with prochlorperazine 5 to 10 mg three times
(e.g., “362”). a day orally for a few days only. Longer term use delays
recovery in acute vertigo.
Dizziness
Recurrent Vertigo
Diagnosis in dizziness comes mainly from history. Examina- Treatment should be directed at encouraging central com
tion is mainly to support diagnosis. Investigations are usually pensation.
• Labyrinthine sedatives such as prochlorperazine delay central • Betahistine is commonly used but a Cochrane review
compensation so should only be used as required for concluded that there was no evidence that it is effective
symptom relief. or ineffective in patients with Ménière’s disease or syn-
• Compensation may occur naturally, especially in younger drome (James & Burton, 2001), and a more recent RCT
people, but can be accelerated by vestibular rehabilitation showed betahistine to be no better than placebo (Adrion
exercises. One study in primary care found that two et al., 2016).
30-minute sessions tripled the number of patients who • Acute attacks: Give a labyrinthine sedative (e.g., prochlor-
improved (Yardley et al., 1998). perazine 5 mg three times a day or cinnarizine 30 mg
three times a day). Buccastem is useful in patients with
Vestibular Rehabilitation Exercises vomiting.
• In bed, performed slowly initially then more rapidly: • Recurrent attacks: Betahistine 16 mg three times a day is
• eye movements—up and down, side to side, focusing often given but the evidence shows that it is no better
on a finger as it moves from 1 m away to 30 cm away; than placebo (Adrion et al., 2016).
• head movements—moving head forward then backward • Poor response: Consider referral to ENT. Treatment in
and turning from side to side. unresponsive cases is intratympanic therapy:
• Sitting: rotating the head; bending down and standing • intratympanic gentamicin: usually effective but risk to
up with eyes open and closed. remaining hearing (Pullens & van Benthem, 2011); or
• Standing: throwing a small ball from hand to hand, turning • intratympanic steroids: unproven but no risk to hearing.
through 360 degrees.
• Moving about: walking across the room, up and down Prophylactic
a slope, up and down stairs with eyes open and then • There is no evidence for prophylactic management of
closed. Ménière’s disease or syndrome (James & Thorp, 2001).
Reducing dietary salt and caffeine is often recommended;
PATIENT INFORMATION there is no scientific evidence, but it is sensible medical
Dizziness and Balance Problems is available from British
advice nowadays in the Western world. There is no evidence
Brain & Spine Foundation, Lincoln House, Kennington Park, that use of diuretics is effective (Burgess & Kundu, 2006).
1-3 Brixton Road, London SW9 6DE, helpline 0808 808
1000, www.brainandspine.org.uk. PATIENTS’ ORGANIZATION
The Ménière’s Society, The Rookery, Surrey Hills Business
Park, Wotton, Dorking, Surrey, RH5 6QT, UK, helpline
01306876883, www.menieres.org.uk.
Ménière’s Disease
• It is often confused with vertiginous migraine (which
should be treated as per normal migraine guidelines).
Patients with migraine have no hearing loss and the
Benign Paroxysmal Positional Vertigo (BPPV)
vertigo usually lasts hours. It is often associated with
other symptoms of migraine but these may be absent. GUIDELINE
• This is characterized by episodic vertigo associated with Strickland, C., & Russell, R. (2003). What is the best way to
hearing loss, tinnitus, and often a feeling of aural full- manage benign paroxysmal positional vertigo? Journal of
ness. Symptoms are usually unilateral and vary in sever- Family Practice, 52, 971–973.
ity. In the early years hearing improves between attacks,
but in the long term, hearing loss becomes permanent.
Some people develop bilateral disease. • The vertigo lasts 5 to 10 seconds, though patients com-
• A patient with the condition who drives must inform the monly think it lasts much longer.
Driver and Vehicle Licensing Agency (DVLA). Driving • The vertigo is brought on by head movement, typically
will be permitted once symptoms are controlled. turning to one side in bed.
• This condition is diagnosed from the history and by per-
Refer to an ENT Consultant forming the Hallpike test (Lempert, Gresty, & Bronstein,
• To confirm diagnosis 1995).
• To exclude a vestibular schwannoma in patients with • It is thought to be caused by debris in the semicircular
persistent unilateral symptoms canals, most commonly in the posterior canal (95%).
Medical Treatment: Acute The Hallpike Test

• No randomized controlled trials (RCTs) exist for Before performing the Hallpike Test check for back or neck
acute management with betahistine, benzodiazepines, or problems and advise that the patient should not drive home
anticholinergics. afterwards.
1. Sit the patient on a couch, hold the patient’s head in recovery after head injury, and in the elderly after stand-
both hands, and turn it 45 degrees toward the test ear. ing or turning rapidly.
Support the neck and, maintaining torsion along with • Unsteadiness lasting hours or days is due to temporary
fixed gaze, move head rapidly backward to a head-hanging impairment of the central connections or decompensation
position, 30 degrees (optimally) below the horizontal. of the vestibular system. It commonly occurs with alcohol
2. Maintain the head-hanging position for 30 seconds and or drug overdose, normal doses of tranquillizers or seda-
observe the patient’s eyes. Typically nystagmus is rotatory tives, and hyperventilation.
with the upper pole going to the downmost ear.
3. Return the patient to the upright position. Prolonged
4. Test the opposite side if no nystagmus is seen on the first Unsteadiness lasting weeks or months is usually due to central
side or when the patient has settled from the first test. or vestibular inadequacy, and is common in the elderly.
Note: The nystagmus may only last a few seconds and
will not occur at all in some patients, in whom the diagnosis Lightheadedness
must be made on the history alone. Note also that the Patients with lightheadedness often report a momentary
patient will experience vertigo at the same time as the sensation of spinning; this does not mean that the vestibular
nystagmus. system is the cause of their symptoms.
Management of BPPV
Tinnitus
• Do not give labyrinthine sedatives.
• Teach the patient to minimize the vertigo by sitting up
GUIDELINES
or lying down in stages.
• The Epley (canalith repositioning) manoeuvre is a safe Guidelines are available from the British Tinnitus Association,
and effective treatment for BPPV (Hilton & Pinder, 2004). www.tinnitus.org.uk.
It involves a brief series of exercises to reposition debris
out of the posterior canal.
• The Epley manoeuvre can be completed in less than 5 • Generally 1% to 2% of the population has tinnitus that
minutes and does not require specialist training. Explana- severely affects the quality of life.
tory videos are available on YouTube (e.g., www.youtube • Reassure the patient that 15% of people experience tin-
.com/watch?v=ZqokxZRbJfw). nitus. For most, it improves with time. Worry about it
• Explain that many patients (about 35%) have recurrence can lead to a vicious circle of increasing distress and more
of symptoms. intrusive tinnitus.
• Patients should not drive for 24 hours afterwards. • Exclude a drug cause (e.g., aspirin, NSAIDs, quinine,
loop diuretics, tricyclics, aminoglycosides).
Instructions for Performance of the Modified • Perform a hearing test. Hearing loss is often a cause of
Epley Manoeuvre tinnitus.
These instructions are for a patient whose left ear is affected. If • Refer patients with:
it is the right ear that is affected, for left read right and for • tinnitus associated with hearing loss. Hearing aids are
right read left. often helpful;
1. Sit up on the bed with your head turned 45 degrees to the • unilateral tinnitus, for exclusion of cerebellopontine
left, having placed a pillow on the bed so that it will be angle tumours, especially if there is hearing loss on
under your shoulders, not your head, when you lie down. the same side;
2. Lie back quickly, keeping your head turned as before. • neck and skull bruits (carotid artery stenosis or arterio-
Wait 30 seconds. venous [AV] fistula);
3. Turn your head to the other side. Wait 30 seconds. • tinnitus severe enough to need specialist help;
4. Roll on to your right side. Wait 30 seconds. • tinnitus associated with systemic or neurological disease.
5. Sit up without rolling over on to your back (i.e., still
facing right). Treatment
6. Repeat this three times a day until the symptoms have • Non-specific support and counselling are very helpful.
ceased. • Look for depression associated with tinnitus and treat it
in its own right. Suicide is known as a consequence of
Unsteadiness depression and tinnitus. There is no evidence that anti-
Episodic depressants are helpful in the treatment of tinnitus (Baldo
• Unsteadiness lasting a few seconds indicates a physiologi- et al., 2012).
cal overload of the vestibular or central systems. It occurs • Amplification: A hearing aid may be used to amplify ordi-
most frequently on rapid movement, associated with a nary background noise.
minor inadequacy of the proprioceptive or labyrinthine • Encourage the patient to mask the tinnitus with back-
systems. In young people it occurs in the later stages of ground noise such as TV, radio, music.
• Tinnitus retraining therapy (TRT): This combines directive • If the eyelid, when closed, does not cover the cornea.
counselling with sound therapy to reduce the attention Refer to an ophthalmic surgeon.
that the patient pays to the tinnitus. There is evidence • If recovery has not started at 6 weeks. Refer to an ENT
that this is more effective than tinnitus maskers (Phillips surgeon to look for an underlying cause.
& McFerran, 2010). • If recovery at 9 months is incomplete. The patient may
• Cognitive behavioural therapy (CBT) does not change the benefit from cosmetic surgery.
subjective loudness of tinnitus but has a significant improve-
ment in depression scores and quality of life (Martinez Management in Primary Care
Devesa, Perera, Theodoulou, & Waddell, 2010). Refer if • Protect the eye with a patch and/or artificial tears. Warn
this is available locally, with a careful explanation that the patient that the eye is not protected by reflex blinking
this does not mean you think the tinnitus is imaginary. and must be safeguarded. This is especially true in windy,
dry conditions and during sleep.
PATIENT ORGANIZATIONS • Give oral prednisolone. Early treatment with prednisolone
significantly improves the chance of complete recovery
British Tinnitus Association, Ground Floor, Unit 5, Acorn
Business Park, Woodseats Close, Sheffield S8 0TB, helpline at 3 and 9 months (Sullivan et al., 2007). Give 50 mg/
0800 018 0527, www.tinnitus.org.uk. day for 10 days provided it can be started within 72 hours
Action on Hearing Loss (the new name of RNID), www. of the onset of the palsy.
actiononhearingloss.org.uk. • Antiviral treatment: There is low-quality evidence that
antivirals used in addition to steroids might further
improve the recovery rate (Gagyor et al., 2015). Antivirals
alone are not helpful.
Facial Palsy (Lower Motor Neurone) • Physiotherapy has no benefit (Teixeira, Valbuza, & Prado,
2011).
Refer urgently if there is evidence of: • Surgical decompression of the nerve is not indicated. There
• middle ear disease, especially cholesteatoma; is insufficient evidence that it is beneficial (McAllister
• parotid tumour; et al., 2013).
• cerebellopontine angle tumour (i.e., hearing loss, loss
of facial sensation, diplopia, or cerebellar signs);
• trauma; The Nose
• Ramsay Hunt syndrome (look for herpetic vesicles on Rhinosinusitis
the pinna or in the external auditory canal).
GUIDELINES AND REVIEWS
Bell’s Palsy Fokkens, W. J., Lund, V. J., Mullol, J., Bachert, C., et al.
(2012). European Position Paper on rhinosinusitis and nasal
OVERVIEW polyps. Rhinology, 50 (23), 1–299.
Davenport, R.J., McKinstry, B., Morrison, J.M., et al. (2009).
Bell’s palsy: New evidence provides a definitive drug therapy
strategy. British Journal of General Practitioner, 59, 569–570.
• Diagnosis is usually clinical.
• X-rays are not recommended. CT is investigation of choice.
• Characterized by a unilateral facial weakness. The weak- Management of Acute Rhinosinusitis

ness may be associated with mild pain behind the ear. • Most cases are viral.
The onset is typically hours. It is more common in preg- • Decongestants (topical or oral) are used widely but there
nant women, in those with diabetes, and following a are no studies to support their use.
recent upper respiratory infection. • Antibiotics. A Cochrane review concluded that there is
• The majority recover without treatment. no place for antibiotics in uncomplicated acute rhinosi-
nusitis as the incidence of side effects of treatment out-
PATIENT ORGANIZATION weighed the minimal benefits (Lemiengre et al., 2012).
The Drug Therapy Bulletin (2009) recommends a policy
Bell’s Palsy Association, www.bellspalsy.org.uk.
of no routine antibiotics or of delayed prescribing. Imme-
diate antibiotics should be reserved for those who are
systemically very unwell or are at risk of complications.
Referral • Advise the patient to:
• take simple analgesics;
• If loss of power is complete, refer to an ophthalmic surgeon • use steam inhalations;
to be seen that day. Tarsorrhaphy is likely to be needed. • nasal douche;
• While there is no evidence for decongestants, they Malignancy

may aide symptomatic relief in short term. Only use A polyp that does not have the typical smooth, pale, or
for 7 days. slightly reddened appearance may be malignant, especially
• Consider an antibiotic in those with (Fokkens et al., 2012): if unilateral. Refer urgently.
• pyrexia above 38°C;
• severe local pain, mainly on one side; PATIENT LEAFLET
• worsening discomfort with purulent discharge. Nasal polyps. Available at www.patient.co.uk (search on
• Refer urgently: “nasal polyps”).
• patients with orbital or facial cellulitis;
• patients who are severely ill.
Management of Chronic Rhinosinusitis

The Common Cold
• Examine the anterior nasal cavity. Refer any unilateral
obstruction as suspicious for malignancy. • A consultation about the common cold is an opportunity
• Intranasal steroids give some improvement in the symp- for the education of the patient in self-treatment as well
toms in chronic rhinosinusitis (Chong et al., 2016). as discussion of any underlying issues.
• Start treatment with intranasal steroids. Treat any underly- • There is some evidence that oral over-the-counter medica-
ing cause or allergy. Maintain treatment for at least 2 tions containing antihistamines, decongestants, analgesics,
months and assess efficacy. or a combination help with recovery (De Sutter et al.,
• Consider referral in patients with recurrent symptoms. 2012).
• A single course of antibiotics may be warranted but • Oral first-generation antihistamines will slightly reduce
repeated courses of antibiotics are not indicated (Royal rhinorrhoea and sneezing in the first 1 to 2 days but with
College of Surgeons, 2016). anticholinergic adverse effects, especially sedation (De
Sutter, Saraswat, & van Driel, 2015).
Nasal Polyps • Ipratropium bromide nasal spray will probably reduce
rhinorrhoea (AlBalawi, Othman, & AlFaleh, 2013).
• Steam inhalations give subjective benefit but there is no
GUIDELINE
evidence of objective benefit (Singh & Singh, 2013).
Scadding, G. K., Durham, S. R., Mirakian, R., et al. (2008). • Regular use of oral vitamin C appears to reduce the dura-
British Society for Allergy and Clinical Immunology guidelines tion and severity of symptoms (Hemilä & Chalker, 2013).
for the management of rhinosinusitis and nasal polyposis.
Clinical & Experimental Allergy, 38, 260–275. • There is no good evidence that echinacea improves symp-
toms (Karsch-Völk, 2014).
• There is no evidence that antibiotics are of benefit for
purulent nasal discharge (Kenealy & Arroll, 2013).
• Nasal polyps may be associated with asthma and aspirin
sensitivity, but if they occur in the presence of rhinitis it Rhinitis
is probably coincidental.
• The initial treatment should be medical.
GUIDANCE
• Cystic fibrosis should be considered in any child under
16 with nasal polyps. Bousquet, J., Reid, J., van Weel, C., et al. (2008). Allergic
• Stop aspirin and consider stopping other NSAIDs; there rhinitis management pocket reference. Allergy, 63, 990–996.
Scadding, G. K., Durham, S. R., Mirakian, R., et al.
is some cross-reaction. (2008). British Society for Allergy and Clinical Immunology
• Steroids. Give betamethasone drops 0.1%, 2 drops per guidelines for the management of allergic and non-allergic
nostril three times a day using the head upside down rhinitis. Clinical and Experimental Allergy, 38, 19–42.
position (see patient leaflet), alone or with oral predniso-
lone (0.5 mg/kg for 5–10 days). Significant improvement
may occur within 48 hours. Some patients need indefinite
prophylaxis with intranasal mometasone or fluticasone, Seasonal and Perennial Rhinitis
which have lower bioavailability than betamethasone drops. • Affects 15% to 20% of the population; 3% of general
The evidence for oral steroids comes from a single small practitioner (GP) consultations are for allergic rhinitis.
trial of poor quality (Patiar & Reece, 2009). • Treatment is best tailored to the individual’s symptoms;
• Give antibiotics if nasal discharge is purulent. Thick, where possible identify the underlying cause.
green-brown secretions may indicate fungal infection. This • Allergens should be avoided where possible (e.g., by reduc-
is more likely to be detected on microscopy than by culture. ing the dust in the house or driving with windows
• The British Society for Allergy and Clinical Immunology and air vents closed). However, measures to reduce expo-
(BSACI) guidelines (above) recommend that all new pre- sure to the house dust mite have been disappointing
sentations be referred to ENT. (Terreehorst et al., 2003).
which was not otherwise visible. In addition, patients

Intermittent Mild Symptoms or Acute Response with nasal deformity may benefit from surgery.
to Allergen
• Antihistamines reduce the nasal itching, watery hyperse- Snoring and Obstructive Sleep Apnoea (OSA)
cretion, and sneezing but do little for nasal blockage
(Pearlman et al., 1995). Intermittent use may be enough. GUIDELINES
• Use an antihistamine nasal spray for rapid relief (Ratner
et al., 1998).
Management of obstructive sleep apnoea/hypopnoea
• Use an oral, minimally sedating H1-selective antihistamine syndrome in adults. SIGN guideline 73. www.sign.ac.uk.
with a rapid onset (e.g., loratadine) for nasal and non- National Institute for Health and Care Excellence. (2008).
nasal symptoms. Continuous positive airway pressure for the treatment of
obstructive sleep apnoea/hypopnoea syndrome. www.nice.
Regular Symptoms org.uk.
• Antihistamines may be taken regularly, and for some
people are all that is necessary.
• Intranasal steroid sprays are the most effective prophylactic • Of middle-age men, 25% are snorers; 10% of middle-
treatment. There is a delayed onset of action of at least aged women snore; 4% of middle-aged men and 2% of
1 week. Combining them with an antihistamine adds no middle-aged women suffer from OSA, with at least five
further benefit (Weiner, Abramson, & Puy, 1998). episodes of apnoea/hypopnoea an hour at night plus
• Instruct the patient on their proper use: daytime sleepiness (Young et al., 1993).
• Use once or twice a day, according to manufacturers’ • Men under 65 who admit to both snoring and daytime
instructions. sleepiness are twice as likely to die as men with just one
• In seasonal rhinitis, start 2 weeks before the beginning of these symptoms and men without either symptom.
of the season. The excess deaths are mainly due to cardiovascular disease
• They should be continued until the rhinitis is likely (Lindberg et al., 1998).
to have subsided. • Those with moderate to severe obstructive sleep apnoea
• Consider an ipratropium nasal spray, in addition to (OSA) hypopnoea syndrome (OSAHS) or with mild
steroids, where rhinorrhoea is dominant (Sheikh, Panesar, OSAHS and daytime sleepiness are likely to be offered
& Dhami, 2005). continuous positive airways pressure (CPAP) as therapy
• Use sodium cromoglycate eye drops regularly in patients of first choice.
with allergic conjunctivitis. • Assess daytime sleepiness with the Epworth Sleepiness
• Consider referral to an allergy specialist. Scale. Alternatively, patients can score themselves on the
British Snoring and Sleep Apnoea Association website
Nasal Blockage (see upcoming information) (Table 20.1).
• Use a topical decongestant (e.g., ephedrine nasal drops • Refer to a sleep study centre (or possibly directly to ENT)
or xylometazoline spray for 5 days) to open the airway patients:
enough for the steroid spray to penetrate. Longer use is • with excessive daytime sleepiness;
likely to lead to rebound congestion on stopping. • where a partner reports apnoeic or choking episodes
• Use betamethasone nasal drops 0.1%. For greatest effect or restless sleep;
the patient should use these in the head-down position (for • where loud snoring is causing relationship problems; or
more information see leaflet on nasal polyps, earlier). Insert • where curable pathology in the nose could be contrib-
2 drops two to three times a day for no more than 1 month. uting (e.g., polyps or a deviated septum).
More prolonged use can lead to systemic side effects. Do
not give more than six courses a year. Try to maintain TABLE Symptoms and Signs in Obstructive Sleep
improvement after each course with a steroid spray. 20.1 Apnoea
Severe Symptoms Symptoms Signs
• Oral steroids can be given if symptoms warrant them Loud snoring Obesity
(e.g., at the time of an examination), but courses should Restless sleep with recurrent Sinusitis
not be repeated frequently. Give oral prednisolone 5 wakening; nightmares
to 10 mg daily, or 30 to 40 mg daily for 1 week in Daytime fatigue or poor Nasal or
dire situations. Intramuscular depot injections of concentration; sleepiness; nasopharyngeal
steroids (e.g., Kenalog) last for 3 months, with the irritability obstruction
inevitable pituitary adrenal suppression, and are not Family history of OSA Large tonsils
recommended for use in rhinitis. Large tongue
• Referral is needed for continuing severe symptoms. Small chin
Nasal endoscopy may reveal pathology (e.g., a polyp),
• Be especially ready to refer urgently patients: The following evidence demonstrates the limited value of
• with obstructive sleep apnoea/hypopnoea and COPD; throat swabs and of antibiotics in the management of sore
• with evidence of ventilatory failure; throat:
• who are symptomatic and drive or operate machinery. • Approximately 20% of sore throats are bacterial, namely
beta-haemolytic streptococci group A (GABHS) and groups
Driving C and G.
• When OSA is only suspected, warn the patient not to • The incidence is dependent on age. Only 15% are bacte-
drive when feeling sleepy and not to embark on a drive rial under the age of 3, while 50% are bacterial between
if very prone to feeling sleepy. the ages of 4 and 13 (DTB, 1995) and 10% in adults
• If the diagnosis is confirmed, the patient should inform (Cooper et al., 2001).
the DVLA and insurance company. Driving will only be • The sensitivity and specificity of the throat swab are low,
permitted once a doctor has confirmed that the condition at most 30% and 80%, respectively. Up to 40% of adults
is controlled. are carriers of GABHS. A positive swab is therefore not
proof of infection.
Snoring Without Apnoea • Streptococcal tonsillitis cannot reliably be diagnosed clini-
• Advise patients to make the following lifestyle changes: cally, although it is more likely if the patient is under 11
• Lose weight if obese, especially if the neck circumfer- years old with:
ence is over 43 cm (17 inches). • myalgia;
• Avoid alcohol before bedtime. • tender or swollen cervical lymph glands;
• Stop smoking. • history of fever;
• Discontinue nighttime sedation. • tonsillar exudates; and
• Sleep on the side; a golf ball sewn into the back of • is less likely if there is cough or earache (Dobbs, 1996).
the pyjamas may help this. • Even if all 4 of the CENTOR criteria are met (tonsillar
• Lift the head of the bed up. exudate, history of fever, tender anterior chain cervical
• Dilate the nostrils using a Nozovent (available from lymph nodes, absence of cough) the likelihood of the
www.britishsnoring.co.uk). infection being due to GABHS is still only 25–86%.
• Examine for nasal obstruction and consider a trial of • Penicillin speeds up recovery only slightly in patients with
intranasal steroids. streptococcal tonsillitis (7 days after the start of treatment
• Consider whether the patient could be hypothyroid. penicillin shows no advantage over placebo in relief of symp-
• Recommend ear plugs for the partner. toms). It does not get patients back to school or work more
• Refer to ENT in dire cases. quickly (Dagnelie, van der Graaf, & De Melker, 1996).
• Antibiotics do not protect against the rare non-suppurative
PATIENT INFORMATION complications in patients with streptococcal tonsillitis,
namely rheumatic fever (Howie & Foggo, 1985) and
The British Snoring and Sleep Apnoea Association (BSSAA), acute glomerulonephritis (Glasziou & Del Mar, 2000;
Castle Court, 41 London Road, Reigate, RH2 9RJ, tel.
01737 245638, www.britishsnoring.co.uk. Taylor & Howie, 1983) or, if they do, the incidence of
SATA (The Sleep Apnoea Trust), PO Box 60, Chinnor, those complications in developed countries is so rare that
OX39 4XE, tel. 0800 025 3500, www.sleep-apnoea-trust.org. it should not influence management (Spinks, Glasziou,
& Del Mar, 2013).
• Antibiotics do protect against the suppurative complica-
tions of sore throat, reducing the incidence of acute otitis
media by two-thirds and of acute sinusitis by a half.
The Throat and Mouth However, these complications are uncommon. It would
Sore Throat be necessary to treat 200 cases to prevent one case of
acute otitis media (Spinks et al., 2013).
GUIDELINE
• Antibiotics have no effect on the incidence of URTI,
whether bacterial or viral, in the subsequent 6 months.
Scottish Intercollegiate Guidelines Network. (2010). The early use of antibiotics may even increase the chance
Management of sore throat and indications for tonsillectomy.
SIGN guideline 117. www.sign.ac.uk.
of recurrence (El-Daher et al., 1991).
• Patients given penicillin for immediate use are more likely
to reattend than those not given antibiotics or given a
prescription only to be used 3 days later if symptoms
SYSTEMATIC REVIEW have not resolved (Little, Gould, et al., 1997; Little, Wil-
liamson, et al., 1997).
Spinks, A., Glasziou, P. P., & Del Mar, C. B. (2013). Antibiotics • If penicillin is used, a 10-day course is more successful
for sore throat. Cochrane Database of Systematic Reviews, 11.
than a 5-day course in eradicating streptococci from
the throat. However, this seems to be of no clinical
importance, since it does not alter the number of sore 2. take blood for an ASO titre. The throat swab may
throats over the following 6 months (Zwart et al., 2003). be a false negative.
• Patients who are more satisfied with the consultation get • With persistent streptococcal infection:
better more quickly (Little, Williamson, et al., 1997). 1. give a cephalosporin or co-amoxiclav. Beta-lactamase–
producing organisms may be destroying the penicillin.
Management • Patients with a past history of rheumatic fever should already
The aim is to: be taking prophylactic penicillin V 250 mg twice a day. If
1. avoid unnecessarily widespread use of antibiotics, with they develop a sore throat they should be given a cephalo-
its disadvantages: the dependence on the GP that it encour- sporin or co-amoxiclav, in case they have beta-lactamase-
ages, the possibility of antibiotic resistance, and the adverse producing organisms in the pharynx that are destroying
effects of the drugs; the penicillin.
2. give the ill patient the possible benefit of early penicillin. • Patients on chemotherapy, immunosuppressive drugs, or car-
• Antibiotics should not be used for symptomatic relief bimazole: Check the white blood cell (WBC) count.
in sore throat (SIGN, 2010). • Patients with a past history of quinsy: Treat as ill patients
• Recommend analgesics and antipyretics (Snow et al., (earlier).
2001). Ibuprofen 400 mg three times a day is the drug
of choice (SIGN, 2010). Vincent’s Angina
• Check what concerns the patient. Explain the probable
viral nature of the condition, and how the patient can This is an ulcerative gingivostomatitis, which can spread to
self-manage subsequent attacks at home (Little, Wil- the tonsils. It is due to an infection with fusiform bacilli
liamson, et al., 1997). and spirochetes.
• If giving a prescription for antibiotics when the indication • Give metronidazole 400 mg twice a day for 3 days.
is arguable, consider recommending that it only be used
if there has been no improvement after 3 days. This can Infectious Mononucleosis
reduce antibiotic use by two-thirds (Arroll et al., 2003).
• Instruct patients to return in 1 week, if still unwell, • Consider prednisolone 40 mg daily orally for 5 days in
for a throat swab, full blood count (FBC), and glan- patients with severe tonsillitis, to enable them to swallow
dular fever antibodies. and avoid airway obstruction.
• Be alert to the possibility that the sore throat is really • Consider a high-dose NSAID to improve the general
an excuse to consult about some more difficult issue. well-being of patients at an important time of their lives
(e.g., when taking examinations).
Special Cases • Avoid ampicillin-based antibiotics, including co-amoxiclav.
The modest benefit of penicillin, even in proven GABHS, makes
a decision not to prescribe reasonable in almost all cases. Oral Thrush
• Ill patients (e.g., with high fever, dysphagia, marked cervical
lymphadenopathy, and severe pharyngitis): • Confirm with a mouth swab.
• Take a throat swab or a rapid antigen test. • Treat with an oral antifungal without waiting for the
• Give phenoxymethylpenicillin 500 mg four times a day swab result.
for 5 days; or consider giving a cephalosporin. Give • Consider a predisposing cause (e.g., diabetes, the use of
erythromycin if the patient is allergic to penicillin. broad spectrum antibiotics or inhaled steroids, human
• Instruct the patient to telephone for the result. immunodeficiency virus [HIV] infection, or other causes
• If pathogenic streptococci are grown, urge the patient of immunosuppression).
to complete the 10-day course.
• Sore throat associated with stridor or breathing difficulty: Sore Throat Due to Postnasal Drip
admit immediately (SIGN, 2010).
• Patients with sore throat who are members of a closed com- Treat with intranasal steroids.
munity where there is an outbreak of streptococcal phar-
yngitis. Treat as ill patients (earlier). Tonsillectomy
• Patients in severe pain: Consider giving oral prednisolone
60 mg daily for 2 days. It reduces pain at 12 and 24 hours • Not all sore throats are due to tonsillitis.
with 57% pain free at 2 days compared to 33% on placebo. • In children with recurrent severe tonsillitis, surgery
The benefit is more marked in sore throat due to streptococ- offers the chance of avoiding two moderate or severe
cal infection (Kiderman et al., 2005). throat infections in the next 2 years. Children with
• Patients with prolonged tonsillitis: a history that is less severe benefit less (Marshall,
• With negative investigations: 1998).
1. repeat glandular fever antibodies weekly for 2 more • There is no convincing evidence for tonsillectomy in
weeks; adults.
REVIEW
Indications for Referral
Gleeson, M., & Jani, P. (1994). Long-term care of patients
• Recurrent acute tonsillitis, five or more episodes a year.
who have had a laryngectomy. British Medical Journal, 308,
Attacks should be severe enough to interfere with the 1452–1453.
child’s normal functioning.
• Airway obstruction, especially if there is sleep apnoea.
• Chronic tonsillitis, for over 3 months, especially if associ-
ated with halitosis. • is attending speech therapy;
• Quinsy or peritonsillar cellulitis, after recovery, in patients • is eating enough. The sense of smell diminishes because
under age 25 with a history of recurrent tonsillitis. the nose is not being used to sniff. Patients can be
• Bacterial carriers who have not responded to antibiotics (e.g., taught to sniff by speech therapists;
the diphtheria carrier and the carrier of haemolytic strep- • has been referred to the National Association of Lar-
tococcus group A who has had rheumatic fever). yngectomee Clubs, Suite 16, Tempo House, 15 Falcon
• Unilateral tonsillar enlargement or ulceration suggestive of Road, London, SW11 2PJ, tel. 020 7730 8585,
a serious underlying disorder (e.g., malignancy). www.laryngectomy.org.uk. Information for laryngec-
• Guttate psoriasis that is exacerbated by recurrent throat tomees, carers, and professionals can be found at the
infections. website.
• Less severely affected children are unlikely to benefit. One
RCT found that in moderately affected children, the The Feeling of a Lump in the Throat
modest benefit was outweighed by the 7.9% who had
surgery-related complications (Paradise et al., 2002). When diagnostic clues are absent:
• treat as oesophagitis for 2 weeks with antacids or a PPI;
Dysphagia • refer non-responders to the ENT department to exclude
a physical cause;
• Check haemoglobin (Hb) and refer urgently to an ENT • counsel those with negative findings to try to uncover
or a general surgeon to exclude a local cause. the cause for their probable globus hystericus.
• If the appointment is not imminent, order a barium
swallow. This is the one situation where a barium study
Sore Mouth
should precede endoscopy.
Red or White Patches in the Mouth
Hoarseness * Refer all as erythroplakia or leukoplakia unless the typical
skin lesions show that it is lichen planus. The risk of
• Refer urgently anyone suffering from hoarseness for more malignancy over 10 years is 3% to 6% with leukoplakia,
than 3 weeks, especially smokers. Early tumours confined and far higher with erythroplakia.
to the vocal cord treated by radiotherapy have an 80%
to 90% chance of 5-year survival. Ulcerated Sore Mouth
• Consider re-referring a patient with persistent constant • Be aware that ulcers may be associated with skin, genital,
hoarseness, even after a normal laryngoscopy performed or eye lesions as part of a larger syndrome.
in the early stages. A previously undetected nodule or
carcinoma may now be visible. Primary Herpes Stomatitis or Labialis
• Do not diagnose chronic laryngitis in smokers without
laryngoscopy. It is they who are most at risk of laryngeal SYSTEMATIC REVIEW
carcinoma.
Chi, C. (2015). Herpes labialis. Systematic review 1704.
• If laryngoscopy is normal, refer to a speech therapist or Clinical Evidence. London: BMJ Publishing Group. http://
voice teacher. Voice abuse is common in teachers, mothers clinicalevidence.bmj.com.
of young children, and anyone who shouts.
• Distinguish hoarseness from functional dysphonia, in which
the patient adopts a whisper for psychological reasons. These
patients need laryngoscopy to exclude a pathological cause
before urgent referral to a speech therapist, psychiatrist, or • First attack: If seen within the first 48 hours give an oral
psychologist. antiviral agent (e.g., acyclovir), unless mild. It can halve
the time to healing, at least in children.
Post Laryngectomy • Recurrent attacks: Use an oral antiviral agent. There is a
lack of evidence of benefit for topical agents.
• Treat all respiratory infections vigorously and admit patients • Prevention of recurrent attacks:
early if they are failing to expectorate their secretions. • Sunblock will dramatically reduce the rate of recurrence
• Check that the patient: in those in whom sun is a factor.
• Prophylactic oral antivirals probably do reduce the • Prescribe an artificial saliva product. Do not encourage
number of attacks and the size and duration of the sucking of sweets unless they are sugar free. Caries is
lesions. Either in the prodromal stage or before a already a hazard for patients with dry mouth.
high-risk activity such as skiing, give oral acyclovir • Oral thrush in a patient wearing dentures: Sterilize the
for 4 days. dentures by soaking them overnight in dilute hypochlorite
solution, then apply miconazole oral gel four times a day
Aphthous Ulcers to the part of the denture in contact with the gum.
• Workup where diagnosis is not clear: Swab for candida and
GUIDANCE check FBC, B12, folate, and serum iron.
Scully, C., Gorsky, M., & Lozada-Nur, F. (2003). The diagnosis
and management of recurrent aphthous stomatitis: A
consensus approach. Journal of the American Dental
Dental Problems
Association, 134, 200–207. • Current guidance from the British Medical Association
Scully, C. (2006). Aphthous ulceration. New England
Journal of Medicine, 355, 165–172. is that GPs should not treat dental problems, as they are
not qualified to do so.
• Staff in GP practices are advised to be aware of local provi-
sion for emergency dental care both in and out of hours.
• GPs retain an ethical responsibility to treat patients in
• These may be associated with stress, menstruation, poor emergency situations but treatment for dental problems
overall health, or occasionally coeliac disease. Large single should only be given in exceptional circumstances.
ulcers can reach 1 to 2 cm in diameter and take 6 weeks
to heal, but at 3 weeks some improvement should already Toothache
be seen. • Sensitive to hot, cold, and sweet stimuli: This is probably
• Check FBC, iron, B12, and folate levels; 20% of patients due to exposed dentine. Advise the patient to see a dentist.
with recurrent ulcers have low iron, B12, or folate levels. • Severe pain, touch sensitivity, gingival swelling with or
Where ulceration is recurrent, check HIV status and without local lymphadenopathy, and fever: This is a dental
antibodies for coeliac disease. abscess. Refer for drainage. If there is likely to be delay,
• In milder cases give: prescribe:
• triamcinalone in cellulose paste (Adcortyl in Orabase), • penicillin V 500 mg four times a day, or erythromycin
thinly applied two to four times a day; 500 mg twice a day, or metronidazole 400 mg twice
• hydrocortisone pellets: dissolve one pellet slowly in a day; and
contact with the ulcer, three to four times a day; • adequate analgesia. Try an NSAID first.
• topical anaesthetic rinse or gel, or antimicrobial
mouthwash; Loss of a Tooth Through Trauma
• pressurized steroids (asthma inhalers) sprayed directly • Reimplantation of a secondary tooth within 12 hours is
onto ulcer (an unlicensed use). likely to succeed, and is worth attempting after an even
• In more severe cases consider oral steroids. longer time.
• In recurrent cases use chlorhexidine mouthwash to reduce 1. Pick the tooth up by its crown, not by the root.
the frequency of relapse (Porter & Scully, 2007). 2. If dirty, wash it in cold water.
• Refer if there are other symptoms (e.g., uveitis, genital 3. Push it back into its socket or under the patient’s
ulceration, arthritis) suggestive of a systemic disease. tongue, or store it in a cup of milk or in the patient’s
• Continuous aphthous ulceration or severe ulceration: Refer saliva.
to an oral medicine specialist. They may benefit from 4. Send the patient immediately to a dentist or accident
higher strength steroids or immunosuppressants, including and emergency department for reimplantation.
thalidomide (an unlicensed use).
Complications After Extraction
Traumatic Ulcers Haemorrhage
• Treat the cause. 1. Allow the patient to rinse the mouth out.
• Try a local anti-inflammatory agent, such as salicylate 2. Place a damp gauze swab over the socket and ask the
cream, applied three to four times a day. patient to bite hard for 10 to 15 minutes.
• Refer for biopsy any ulcer that has not healed within 3 3. If this fails, either suture across the socket with 3.0 silk
weeks after the removal of any local cause. It may be a or refer to a dentist or oral surgeon.
squamous cell carcinoma.
Painful Socket, Bad Taste, Halitosis
Sore Mouth Without Ulceration • This usually occurs 4 to 5 days after an extraction and is
• Dry mouth may be due to anxiety, drugs, dehydration, due to the clot not forming or being removed and the
or Sjögren syndrome. socket filling with debris that gets infected.
• Refer to a dentist. • Delusional halitosis may be the only complaint of a

• Give strong analgesics. patient with a hypochondriacal depression. Refer to a
• Advise the patient to wash the mouth out with warm psychiatrist.
saline.
• Start antibiotics: penicillin V 500 mg four times a day
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21
Eye Problems
SUZANNAH DRUMMOND
General Points Twitching
Visual Acuity Testing Watery Eye
Instilling Drops Into the Eye Acute Disturbance of Vision
The Gritty Irritable Red Eye Acute Painless Loss of Vision
Acute Infective Conjunctivitis Central Retinal Artery Occlusion
Allergic Conjunctivitis Central or Branch Retinal Vein Occlusion
Acute Decreased Vision With Pain
Chronic or Recurrent Allergic Eye Disease Acute Closed Angle Glaucoma
Blepharitis Optic Neuritis
Dry Eye Acute Distortion of Vision
Trauma Floaters and Flashes
Foreign Body Gradual Loss of Vision
Corneal Foreign Body Cataracts
Chemical Burns Problems After Cataract Surgery
Ultraviolet Light Burns Age-Related Macular Degeneration
Corneal Abrasion
Chronic Simple Glaucoma
Blunt Injuries
Screening and Referral
Problems With Contact Lenses Allergy to Drops
Conjunctivitis Laser Treatment
The Painful Red Eye That Is Not Gritty Surgical Treatment
Ophthalmic Herpes Zoster Visual Handicap
Lash, Lids, and Lacrimal Problems Certification and Registration
Benefits
Acute Dacrocystitis
Prescribing for Patients With Contact Lenses (Mitchell &
Chalazion Edwards, 2001)
Styes
Corrective Surgery for Myopia
General Points • The Snellen chart should be the recommended distance

from the patient for the size of chart used. The distance
PATIENT INFORMATION should be marked and the patient should stand behind
the mark.
Patient information leaflets for a number of eye conditions are
available from Moorfields Eye Hospital at www.moorfields.
• The chart should be illuminated with 480 lux (e.g., a
nhs.uk/listing/conditions. spotlight).
• One study found that few practices fulfilled these criteria
(Pandit, 1994). Mobile technology developments now
Visual Acuity Testing allow distance visual acuity testing with iPad/iPhone. These
show a high level of agreement of visual acuity results
• Tests of visual acuity have a reliability of 98% and provide with Snellen charts suggesting this technology can be
essential referral information but only if the following used with confidence in the primary care setting. (O’Neill
steps are taken: 2016)
371
Instilling Drops Into the Eye • If the discharge is purulent, take swabs for viral, bacterial,
and chlamydial infection and a smear for gonococci. Then
• Instilling drops: Pull the lower lid down with the patient start chloramphenicol eye drops hourly and refer for an
looking up. Squeeze one drop onto the lower fornix. ophthalmic opinion the same day.
• Instilling ointments or gels: As above but squeeze 1 cm • Admit for intensive antibiotic therapy if:
onto the inner surface of the lid. Warn the patient it will • herpes simplex, chlamydia, or gonococcus is grown;
blur the vision for a short while. or
• the clinical situation worsens, with a red eye, cloudy
cornea, blepharitis, marked preauricular lymphadenopathy,
The Gritty Irritable Red Eye or an unwell child.
Acute Infective Conjunctivitis • Notify as ophthalmia neonatorum.
In an adult or child:
Allergic Conjunctivitis
• the history and examination traditionally give grounds
for distinguishing between bacterial and viral infections Acute
but there is no evidence to support this (Rietveld et al., • Reassure the patient that the eye will not be harmed and
2003); that the problem will settle soon.
• even in proven bacterial conjunctivitis, antibiotics only • Advise patients not to rub their eyes.
modestly improve the rate of resolution. Over half will • Discontinue contact lens use while symptomatic.
resolve without treatment in 2 to 5 days (Sheikh & • Apply cool compresses +/− refrigerate artificial tears.
Hurwitz, 2005); • If patients require further short-term treatment, use anti-
• individual meta-analysis showed that acute conjunctivitis histamines +/− vasoconstrictors (e.g., olopatadine or
in primary care can be thought of as a self-limiting condi- antazoline with xylometazoline).
tion. Patients with a purulent discharge or a mild severity
of red eye may have a small benefit from antibiotics N.B. Vasoconstrictors cause rebound hyperaemia if taken for
(Au Jefferies, Perera, & Evert, 2011). over 2 weeks.
If the visual acuity is normal and the cornea is clear, advise the
patient to: Chronic or Recurrent Allergic Eye Disease
• wipe away discharge; • Establish, if possible, what the allergen is and reduce or
• wash hands after touching the eyes; avoid contact.
• use separate towels. • Preferred use is for an antihistamine with mast cell sta-
Inform patients they are highly infectious. bilizing properties (e.g., olopatadine), and instruct the
• It seems logical to recommend that children should stay patient to use for at least 2 weeks. If there is concurrent
away from school even though this is not the recom- rhinitis, consider using a steroid nasal spray (e.g., Nasonex
mendation of the Health Protection Agency (Guidelines spray twice a day) rather than an oral antihistamine.
on the Management of Communicable Diseases in Schools • Prophylaxis: Give sodium cromoglicate, nedocromil sodium,
and Nurseries, 2008). or lodoxamide drops four times a day. Benefit may not
• Treat only if purulent discharge with mild red eye. It may be seen for 2 weeks. Where symptoms are seasonal, start
be appropriate to advise the patient to return in 3 days treatment before the season starts.
to collect a prescription for antibiotic drops if symptoms • Consider giving an oral nonsedating antihistamine if
are not resolving (Everitt, Little, & Smith, 2006). Prescribe symptoms are still not controlled.
chloramphenicol eye drops 2-hourly for 2 days, then four
times a day for a further 5 days. Gentamicin is only N.B. Not in dry eye patients as this can exacerbate symptoms.
indicated if there is gram-negative infection.
• If symptoms are still present at 1 week but there are no • Severe cases not responding to the above: Refer to ophthal-
complications, stop all treatment and review weekly. mology for consideration of weak topical steroid (e.g.,
Adenoviral infection typically takes 2 to 3 weeks to resolve. prednisolone).
• Povidone-iodine is becoming increasingly used in the • If a patient worsens dramatically after starting to use
treatment of refractory conjunctivitis and including adeno- drops consider contact sensitivity. The usual cause is the
conjunctivitis. (Pepose, J, 2018). preservative in the drops.
• Refer to eye casualty at any stage if the visual acuity is
reduced or the cornea is involved. Blepharitis
• Orbital cellulitis: Admit immediately.
In a neonate: • The mainstay of treatment is patient education and
• The likelihood and the dangers of sexually transmissible counselling.
disease are greater. • Emphasize that this is a chronic disease with intermittent
• If the eye is mildly sticky, take a swab and review. exacerbations.
CHAPTER 21 Eye Problems 373
• Reassure the patient that the condition will not threaten ointments (e.g., Lacri-Lube) for use at night only, as they
sight. Explain that it results from either colonization of create a greasy tear film that is difficult to see through.
eyelashes or Meibomian gland dysfunction. Treatment is • Refer:
long term. • urgently if there is a sudden increase in discomfort,
• Lid hygiene comprises three stages: redness, or deterioration in vision. These patients are
1. Compresses: Heat the area with a hot washcloth (not at increased risk of microbial keratitis;
scalding) for 5 to 10 minutes. • routinely patients who are having to instill drops very
2. Massage: Directly after compresses, massage lids toward frequently. Many other treatments may be appropriate:
lid margin to empty ducts. topical glucocorticoids or cyclosporine, autologous
3. Wash: Using dilute (1:3) baby shampoo and a cotton serum, vitamin A or oral antioxidants, punctual plugs,
bud, gently scrub eyelid margins, initially twice a day or lid surgery.
then twice weekly thereafter.
• Treat patients as if they have dry eyes, as the tears evapo- Trauma
rate more rapidly. See upcoming discussion for treatment
regimen but do not continue if there is no benefit. • Check the visual acuity. Refer if this is diminished or has
• If there is no improvement, give chloramphenicol oint- decreased when next seen.
ment to be applied to the lid margins twice daily for 1
month. Foreign Body
• Consider topical azithromycin 1% solution for 4/52 • Suspect a foreign body in any unilateral red eye with or
(Luchs, 2008). without a history of “something going into the eye.”
• Treat those still not responding and those with acne rosacea • Always fully evert the upper eyelid when looking for a
with long courses (three months) of oral antibiotics (e.g., foreign body. If a foreign body is seen, it can usually be
oxytetracycline 250 mg twice daily or lymecycline 408 mg removed from the conjunctiva without difficulty.
twice daily for fewer gastrointestinal [GI] side effects). A • If there is a history of pain while hammering metal or of
short course of Azithromycin 500 mg OD for three days exposure to glass splinters, refer to exclude an intraocular
may also be tried. foreign body.
• Consider referral to an ophthalmologist for those:
• remaining symptomatic despite treatment for a few Corneal Foreign Body
months particularly if unilateral (masquerade syndrome 1. Insert local anaesthetic. Start with proxymetacaine and
of sebaceous cell carcinoma); when the stinging has stopped, deepen the anaesthesia
• who have associated skin disease; with amethocaine 1% drops. Continue until the drops
• where there is suspicion of corneal involvement. The feel cold but do not sting.
inflammation is secondary to hypersensitivity to the 2. Remove the foreign body with a cotton wool bud or the
cell wall protein of staphylococcus and may require tip of a sterile needle. Do not dig into the cornea; at its
topical steroid and antibiotic drops for a short period. centre it is only 1 mm thick.
Encourage the patient to continue lid hygiene. 3. After removal, prescribe antibiotic eye ointment twice a
day for 2 days as prophylaxis.
Dry Eye 4. Give a cycloplegic (e.g., cyclopentolate 1% drops four
times a day) if pain continues.
• Early detection and treatment may help prevent corneal 5. Do not apply an eye pad (Easty, 1993).
ulcers and scarring. 6. Examine after 24 hours:
• Treat any blepharitis (see earlier). • with fluorescein to be sure the epithelium has healed;
• Check that the patient is not taking any drugs that might • to exclude a rust ring if the foreign body was metallic.
exacerbate the condition (e.g., antihistamines). If present, refer to an ophthalmologist urgently for it
• Encourage the patient to alter his or her environment by to be removed.
minimizing air conditioning and heating and to try to
increase the humidity, if appropriate. Chemical Burns
• Advise the intake of omega 3/6 fatty acids. • These are acid or alkali burns (usually in the form of
• Lubrication is the mainstay of treatment. cement, lime, caustic soda, or ammonia).
• Artificial tears: Encourage the patient to use as often as • Acid burns cause coagulation necrosis whereas alkali burns
necessary. First line, start with Poly-vinyl Alcohol (PVA) are the more serious, causing saponification of phospholipid
or Carbomer. Start at four times a day and increase. If membranes leading to rapid epithelial cell death and caustic
using more than six times daily, drops should be preserva- penetration. Ammonia can cause severe injury in 2 minutes.
tive free. Second line, use Carmellose or Hyaluronate. • Irrigate with tap water or normal saline for 20 minutes
• If Meibomiam gland disease (MGD) is identified use or with alkali burns until the pH is normal (pH 6.5–7.5).
Systane balance or Optive Plus (preservative free) (Argrawal, • Examine the cornea with fluorescein and send to eye
A et al., 2018). Ointments. Prescribe paraffin-containing casualty if it is not clear.
• Cement. Look for cement adhering to the eye, under the

lids, or in the conjunctival fornices. If found, give local Blunt Injuries
anaesthetic drops (e.g., benoxinate) and remove it, however Refer all patients who receive blunt injuries (e.g., from a
distressing this is to the patient. Refer immediately fol- squash ball) as this may give rise to bleeding into the anterior
lowing this. chamber of the eye (hyphaema) and the pressure may rise.
• Detergent. If the conjunctiva shows punctate epithelial
loss (staining with fluorescein), treat with chloramphenicol
Problems With Contact Lenses
and review in 3 days.
Conjunctivitis
Ultraviolet Light Burns • Advise the patient to remove the lens and leave it out
• Arc eye is usually due to exposure to ultraviolet C (UVC) until recovered.
light through welding or using a sunbed without eye
protection. Patients are usually unaware until 6 to 12 N.B. Keep the lens and its container, in case it is needed for culture.
hours after exposure.
• Treatment is supportive with anticipation that damaged • Check that the patient is cleaning and disinfecting
epithelium will regenerate within 24 to 72 hours. the lenses every night (or using disposable single-use
• Give local anaesthetic drops (e.g., benoxinate) once, but lenses).
do not repeat them; they retard corneal healing. • Prescribe antibiotic eye drops.
• Treat with chloramphenicol ointment for lubrication and • Arrange for slit-lamp examination to exclude keratitis,
prevention of superinfection four times a day for 2 to 3 days. either by the lens provider or the local ophthalmology
• The worst of the two eyes can be padded if it offers some emergency department.
relief. • Corneal abrasion: see earlier.
• Give sufficient sedatives and analgesics to ensure a night’s • Microbial keratitis: A white infiltrate viewed with a penlight
sleep. Opioids are likely to be needed. Cessation of blink- needs to be referred to the ophthalmology emergency
ing during sleep enables the corneal epithelium to heal. department immediately. There is a risk of perforation
• Review patient in 2 to 3 days. and endophthalmitis.
• Other problems need slit-lamp examination to distinguish
Corneal Abrasion them. Advise the patient to leave the lenses out and
• Use topical anaesthetic once for examination purposes only. contact the lens prescriber or refer the patient to an
• Confirm the abrasion with fluorescein dye after examining ophthalmologist. National Health Service (NHS) facili-
the eye and excluding other injuries (e.g., hyphaema and ties are available to lens wearers if pathologic problems
open globe). have arisen.
• In a contact lens wearer, use a penlight to look for an
infiltrate. The Painful Red Eye That Is Not Gritty
• Treat with chloramphenicol ointment four times a day
for 24 hours and then twice a day for a further week. • Most need referral to eye casualty:
• For a large abrasion, give a cycloplegic (e.g., cyclopentolate • keratitis, ocular herpes simplex, corneal ulcer, scleritis,
1% drops) twice a day if pain continues. Warn patient and uveitis the same day;
about driving and difficulty reading. Consider oral nonster- • acute glaucoma immediately.
oidal antiinflammatory drugs (NSAIDs; e.g., ibuprofen) • The GP may treat:
or topical ophthalmic NSAID drops (e.g., diclofenac • Episcleritis with an oral NSAID if there is discom-
sodium) (Weaver & Terrell, 2003). fort (e.g., diclofenac or flurbiprofen for 2 weeks).
• Do not apply an eye pad (Easty, 1993). Topical NSAIDs (e.g., diclofenac four times a day)
• Give oral analgesia if the pain is severe. are also appropriate. Refer if not improved within
• Refer if: a month.
• there is a decrease in acuity of greater than two lines • Recurrent uveitis. Treatment with topical steroids can
of the Snellen chart; be started, provided:
• there is a large defect (>50% of the epithelium); 1. their use has been previously sanctioned by a
• there is a purulent discharge/white infiltrate; consultant;
• the epithelium has not healed after 3 days. 2. the visual acuity is normal;
• Recurrent abrasions (erosions). Recurrences tend to occur 3. a dendritic ulcer is excluded with fluorescein and
in the early morning during opening of that eye, when magnified examination of the cornea; but arrange
tear secretion is less. Give long-term (3/12) artificial tears for specialist assessment;
during the day and ointment at night (see Dry Eye). 4. Ophthalmic supervision is always necessary because
Explain that recurrences can continue for months or years. intensive topical steroids may be needed. Treat with
Refer to an ophthalmologist if not improving. Laser abla- topical antivirals if the history is suggestive of herpes,
tion may be needed. while awaiting assessment.
Ophthalmic Herpes Zoster Styes

• Give a high-dose antiviral drug at the first sign of zoster • Treat the infected eyelash follicle with topical antibiotics.
in the ophthalmic division of the trigeminal nerve (i.e., • If there is marked swelling of the lid consider oral
when the rash appears on the forehead, usually before antibiotics.
any involvement of the eye). Untreated, about half would
develop eye involvement. Twitching
• Refer to an ophthalmologist if there is:
• Hutchinson sign (involvement of the nasociliary nerve • Myokymia. Reassure the patient that twitching of a small
which supplies the side of the nose and the skin of area of the orbicularis muscle is common and is not sin-
the inner corner of the eye); ister. It is often exacerbated by tiredness.
• a red eye; or • Facial twitching. Refer to a neurologist or paediatrician.
• visual complaints (Opstelten & Zall, 2005). • Blepharospasm. Forcible contractions of the entire orbi-
This referral is for an early but not immediate appoint- cularis muscle should be referred to an ophthalmologist.
ment. The complications for which ophthalmic expertise is Further investigation may be necessary (e.g., magnetic
needed (e.g., keratitis or uveitis) tend to occur a week or resonance imaging [MRI] scan) and botulinum toxin
more after the onset of the rash. injections can give short-term relief.
• Be alert to the long-term complications of eye involvement:
• Loss of corneal sensation may make the eye vulnerable Watery Eye
to neuropathic ulceration or exposure keratopathy.
• Scarring of the eyelid by an extensive periorbital vesicu- • This may result from inflammation or stenosis of the
lar rash may lead to incomplete closure, putting the nasolacrimal duct or malposition of the lid.
cornea at risk. • Treat any infection with topical antibiotics.
Patients with either of these problems will need to • Consider a trial of artificial tears (e.g., carbomer). The
use lubricating eye ointments long term with or without patient’s natural basal secretion of tears may be deficient
surgery. causing reflexive overwatering as compensation.
• Refer patients who are bothered by it to an ophthalmolo-
gist. They may benefit from exploration of the nasolacrimal
duct or lid surgery. Massage of the lacrimal sac does not
Lash, Lids, and Lacrimal Problems help unless there is a mucocoele.
Acute Dacrocystitis
Acute Disturbance of Vision
• Treat aggressively for presumed staphylococcal or strep-
tococcal infection (e.g., with flucloxacillin and amoxicillin) • Establish that the loss is acute and not just sudden aware-
for 1 week. ness of a preexisting field loss or cataract.
• Review after 24 hours. If the symptoms are worse, refer • Distinguish transient from continuing visual losses and
for an ophthalmologic opinion that day. treat as appropriate. For migraine, see Chapter 12; for
• Refer once recovered for an ophthalmologic opinion and transient ischemic attack (TIA), see Chapter 12.
possible surgery.
Acute Painless Loss of Vision
Chalazion Central Retinal Artery Occlusion
• Advise the patient to use a warm compress (e.g., face • Central retinal artery occlusion is an ouclar emergency
flannel) over the lump and massage toward the lid margin. and is the ocular analogue of cerebral stroke. It results in
Antibiotic ointments or drops are traditionally given (onto profound, usually monocular vision loss and is associated
the eye, in the hope that they will penetrate the chalazion with significant functional morbidity. The risk factors for
through the conjunctival surface) despite the lack of evi- CRAO are the same atherosclerotic risk factors as for
dence of benefit. stroke and heart disease. As such, individuals with CRAO
• Treat secondary infection with oral antibiotics (see may be at risk of ischemic end organ damage such as
Dacrocystitis). cerebral stroke. Management is not only to restore vision
• Review the patient, once recovered, and treat any blepha- but at the same time to manage risk factors that may lead
ritis present (see earlier). to other vascular conditions (Cugati, S et al., 2013).
• Refer if a hard lump has failed to resolve after 3 If seen within 24 hours:
months or is particularly troublesome, for incision • Arrange for the patient to be seen immediately in eye
and curettage. casualty. Restoration of blood flow within 90 to 100
• Prevention of recurrence. Encourage lid hygiene (see minutes leads to no retinal injury. If any delay is antici-
above). pated then give aspirin 300 mg (unless contraindicated).
If seen after 24 hours: tablets orally and instill 2% pilocarpine, 0.5% timoptol,
• There is no treatment for the eye and the object is to try and 1% apraclonidine into the eye 1 minute apart.
to prevent retinal artery occlusion of the other eye. • Treat nausea and vomiting with prochlorperazine 12.5 mg
• Refer to an ophthalmologist but less urgently. intramuscularly.
• Give aspirin 75 mg daily. • Do not attempt to dilate the pupil of anyone with sus-
• Take blood for erythrocyte sedimentation rate (ESR). If pected acute angle glaucoma.
the clinical picture suggests temporal arteritis, give steroids
while awaiting the result. Optic Neuritis
• Look for evidence of more widespread cardiovascular • Progressive reduction in vision over a few days with the
disease, especially a carotid bruit. Check blood pressure, nadir at 1 to 2 weeks, with pain on eye movements.
pulse fasting blood sugar, and lipids. Colours appear washed out. Symptoms may be worse
• Ensure the patient has been referred to the stroke team after a hot shower or bath.
for full systemic workup. • A third of the patients have papillitis, the remainder have
• Ensure that referral has been made to the stroke team for retrobulbar inflammation.
definitive work up. • Improvement occurs within 2 weeks and full recovery is
• The patient should be followed up at 1–4 weeks by the usual within 3 months.
ophthalmology team for assessment and treatment of • Of individuals aged 20 to 40 years with retrobulbar neuritis
ischaemic neovascular changes. in the United Kingdom, 50% to 70% will develop multiple
sclerosis (MS), almost all of them within 5 years. A second
Central or Branch Retinal Vein Occlusion episode increases that risk fourfold (Kanski, 1989).
• Workup: • Refer urgently patients having their first attack to oph-
• Blood pressure (BP) thalmology. Gadolinium-enhanced MRI provides con-
• Full blood count (FBC) for polycythaemia and firmation of optic neuritis and aids prognosis and treatment
leukaemia decisions. Arrange for the patient to be seen again if
• Serum lipids symptoms are not improving after 2 weeks.
• Blood sugar • Refer patients with a second attack to a neurologist (if
• Refer to eye casualty. The patient may need photocoagula- not already done) because of the chance that this is mul-
tion for chronic macular oedema and ischaemia following tiple sclerosis.
a branch vein occlusion. Various treatment options now • In younger patients consider infectious/postinfectious
exist in the form of intravitreal antivascular endothelial causes of optic neuritis. In older patients (>50 years)
growth factor (anti-VEGF) injections for branch or central consider ischaemic optic neuropathy.
vein occlusion with macula oedema. Monitoring by an • Ophthalmology/neurology may advocate intravenous
ophthalmologist in the absence of macular oedema is steroids in severe/bilateral visual loss as they speed recovery
required for 2 years postocclusion to assess for neovascu- (and decrease short-term risk of MS development) but
larization. Complications can develop early; 50% develop they give no benefit to long-term visual outcome or MS
neovascular glaucoma within 3 months. development. Oral steroids are contraindicated as they
cause an increased rate of recurrence of optic neuritis.
Decreased Vision With Pain
Acute Closed Angle Glaucoma Acute Distortion of Vision
• Suspect it when: • Refer urgently. Older patients may have acute wet age-
• the patient is unwell, with vomiting and eye or head related macular degeneration (AMD), especially if that
pain; and diagnosis has already been made in the other eye. Typi-
• the eye is red and tender, the cornea hazy, and the cally, they report that straight lines appear crooked.
pupil semidilated; • If vision is still better than 6/96 they may benefit from
• there may be a past history of episodes of blurred intraocular injections of an anti-VEGF approved by
vision, associated with halos round lights, that have National Institute of Health and Care Excellence (NICE)
been aborted by exposure to a bright light or by going provided they fit the criteria (Royal College of Ophthal-
to sleep. In these situations, the pupil constricts, pulling mologists Guidelines, 2015).
the pupillary iris out of the angle.
• Refer suspected cases urgently to eye casualty or admit. Floaters and Flashes
If treatment is delayed irreversible damage to the optic
nerve may occur in addition to adhesions between the • Refer urgently patients who report:
iris and cornea requiring surgical drainage. • multiple floaters, especially if associated with flashing
• If it is not possible to get the patient to hospital immediately lights. They may have a posterior vitreous detachment,
give acetazolamide 500 mg intravenously or two 250-mg which carries a 5% chance of causing a retinal tear;
• the appearance of a single large blob, which then frag- • In a child, refer as a matter of urgency. Any opacity of
ments. They may have a vitreous haemorrhage. the ocular media can have a catastrophic effect on the
• Be readier to refer if: development of vision and should be diagnosed and treated
• there is a family history of retinal tear or detachment; promptly.
• the other eye has already suffered a detachment. Almost • In a young adult, refer but also investigate for diabetes or
a quarter of patients develop a detachment in the second any other systemic disease that a clinical history might
eye; lead you to suspect.
• the patient has high myopia; • In the elderly:
• there is a history of cataract surgery, which increases • exclude diabetes;
the risk of retinal detachment ninefold. • refer to an optometrist all patients who have not had a
• Refer immediately if there is a field loss or decrease in recent refraction, as a myopic shift often accompanies
acuity. These imply that retinal detachment has occurred. the onset of cataract and a change in spectacle lenses
It may be visible ophthalmoscopically. Instruct the patient can often postpone the need for cataract surgery;
to lie with the face on the side of the detachment (i.e., • they should be able to refer directly to an ophthalmolo-
the side opposite the field defect) on the pillow, while gist if no improvement in vision is possible with refrac-
waiting for the ambulance. tion and:
Note: Retinal tears are unlikely to be detected by conven- 1. the patient is having significant visual problems
tional ophthalmoscopy. A normal fundus is therefore no affecting quality of vision, or activities such as driving
grounds for inaction. or reading; and
2. the patient is willing to undergo surgery. There is
INFORMATION FOR PATIENTS WITH RETINAL little point in referring those that are happy with
DETACHMENT
their current level of vision and would not accept
Understanding Retinal Detachment. Available online from the an invitation for surgery.
Royal College of Ophthalmologists; www.rcophth.ac.uk; • There is no absolute Snellen acuity below which surgery
search on “information booklets.”
is indicated. It depends on the patient’s individual needs
Information for Doctors and circumstances. Many patients are content with vision
Kang, H. K., & Luff, A. J. (2008). Management of retinal that is 6/18 or better unless they drive or do fine work.
detachment: a guide for non-ophthalmologists. British • Some patients find their cataract is more disabling than
Medical Journal, 336, 1235–1240. would be predicted from the level of Snellen acuity. This
happens particularly in posterior subcapsular cataract when
localized opacities scatter light, causing difficulty with
Gradual Loss of Vision reading and with glare when driving. After surgery 85%
to 90% will achieve vision that is adequate for driving
• Refer to the optometrist who can ascertain whether there (Allen & Vasavada, 2006).
is refractive error (normally acuity will improve with a
pinhole) and provide spectacles or perform a more thorough Problems After Cataract Surgery
examination as to any other causes and refer appropriately. • A red eye following surgery in 0.1% to 0.5%, is due to
• Suggest the following to all patients with visual difficulty: postoperative endophthalmitis. It would therefore be
• Use adequate lighting (e.g., an angle-poise lamp), prudent to refer immediately any postoperative patient
especially for reading and close work. with a red eye especially if there is reduced vision and pain.
• Rearrange the room to sit with the main window behind More commonly however it is caused by an allergy to the
the patient and the television away from the window. antibiotic and steroid drops given postoperatively.
• If visual acuity is 6/18 or worse, refer routinely as they • More long-term deterioration of vision after surgery: Refer
may be eligible for partial sight registration. the patient back; 20% develop opacification of the pos-
• Recommend the patient information leaflets available terior capsule within 2 years of operation, requiring laser
from the Royal National Institute of Blind People (RNIB) treatment.
at www.rnib.org.uk (search for “leaflets”).
Age-Related Macular Degeneration
Cataracts
• This occurs in elderly patients who present with progres-
SYSTEMATIC REVIEWS sive loss of central vision and, frequently, distortion effects.
Snellingen, T., Evans, J. R., Ravilla, T., & Foster, A. (2004). When looking at straight lines they appear wavy. The
Surgical interventions for age-related cataract (Cochrane vision is best in dim light. The eye appears normal except
review). In The Cochrane Library (Issue 2). Chichester, UK: for the choroidoretinal changes.
John Wiley & Sons, Ltd.
• Refer all patients for optometric assessment unless there
is a sudden deterioration suggestive of wet AMD (see
above, section on Acute Distortion of Vision) to confirm PATIENT INFORMATION

diagnosis and to refer to ophthalmology if appropriate.
International Glaucoma Association. Woodcote House, 15
• Reassure patients that, although they have lost some of
Highpoint Business Village, Henwood, Ashford, Kent, TN24
their central reading vision, they will never be completely 8DH, tel. 01233 64 81 70, www.glaucoma-association.com.
blind and that further deterioration will be slow. The Understanding Glaucoma is available online from the
average time it takes for vision to deteriorate to below Royal College of Ophthalmologists at www.rcophth.ac.uk
3/60 (eligible for blind registration) is 5 to 10 years. (search on “information booklets”).
• Encourage the patient to stop smoking. Smokers carry a
three- to fourfold increased risk of age-related macular
degeneration. There is evidence that stopping smoking
reduces the incidence and progression of the disease (Kelly • Driving. Advise the patient to notify the Driver and Vehicle
et al., 2004). A diet rich in carotenoids (green leafy veg- Licensing Agency (DVLA) if there is significant bilateral
etables), regular exercise and control of blood pressure visual field loss (Potamitis et al., 1994).
and cholesterol are also beneficial in prevention. • Treatment is managed by the hospital eye department.
• Following diagnosis, there is now evidence to show that It is helpful to know that the aim is to keep the pressure
nutritional supplements, including antioxidant vitamins around 15 mm Hg, using:
and lutein and zeaxanthin, are beneficial in slowing the • prostaglandin analogues (latanoprost, travoprost, and
progression of AMD (AREDS2 Research Group, 2013). bimatoprost), which increase aqueous outflow through
the uveoscleral pathway and can reduce intraocular
PATIENT INFORMATION pressure by 30% to 35%. Systemic side effects are
Macular Disease Society. Crown Chambers, South St, minimal; eyelashes may lengthen and darken and light
Andover, Hampshire, SP10 2BN, tel. 0300 3030 111, www. irises may permanently darken;
macularsociety.org. • beta blockers (timolol, carteolol, betaxolol, levobunolol,
Understanding Age-Related Macular Degeneration. and metipranolol), which reduce the secretion of
Available online from the Royal College of Ophthalmologists
at www.rcophth.ac.uk (search on “information booklets”).
aqueous. They can cause systemic symptoms and may
unmask latent and previously undiagnosed heart failure
and airway obstruction (Kirwan et al., 2002). Systemic
Chronic Simple Glaucoma effects can be reduced by finger pressure on the caruncle
or by shutting the eyes for several minutes after instill-
• Treatment to lower intraocular pressure has been shown ing the drops. This approach may also increase ocular
to slow the progression of the disease (Wormald, 2003). absorption of the drug;
• Loss of visual acuity is a late sign of chronic glaucoma. • carbonic anhydrase inhibitors (dorzolamide, brinzol-
By this stage, disc cupping and field loss are likely to be amide, or oral acetazolamide), which reduce the secre-
advanced and irreversible. tion of aqueous humour. Oral acetazolamide is the
• The incidence rises with age. In the United Kingdom it most effective but has significant side effects. Topical
is 1 in 5000 at age 40 to 49 years, and only really rises forms have few side effects. Neither should be used in
over age 60. Over 85, it is 1 in 10. patients with sulphonamide allergy.
• The risk increases: • parasympathomimetic drugs (e.g., pilocarpine) are now
• with a family history (×10); used only infrequently. They constrict the pupil and
• in high myopes (×3); pull on the trabecular meshwork increasing the flow.
• in diabetics (×3); Pilocarpine eye drops frequently cause headaches, but
• in African Caribbeans; they tend to disappear after the first few weeks of use.
• in users of topical steroid drops for over 5 days. Pilocarpine constricts the pupil and may cause blurred
vision if central lens opacities are present.
Screening and Referral
• Eye tests are advised every 2 years or more frequently if Allergy to Drops
in a high-risk group. This may present with intense itching and irritation of the eyes
• Screening for glaucoma should be completed by the and eyelids, which is exacerbated by instillation of the drops.
optometrist who will perform tonometry (pressure mea- The allergy may be to the active drug or preservative (usually
surement), visual field analysis, and fundoscopy as well benzalkonium chloride). Stopping treatment (with monitoring
as possibly gonioscopy and pachymetry (angle and corneal of the pressure) should result in rapid improvement. Some
thickness analysis). topical agents are available in a preservative-free form.
• Testing is free in Scotland and elsewhere for children and
adults over 60 years of age. Many of those in the high- Laser Treatment
risk groups are also eligible for free testing, including: Trabeculoplasty or even ciliary body ablation may be appro-
• age over 40 with a family history of glaucoma; priate in certain circumstances—usually as an adjunct to
• having glaucoma or diabetes. drops:
• Trabeculoplasty: Argon or diode burns are applied to the

trabecular meshwork. It is used only where the drainage Benefits
angle is open and is effective for a short term; hence it • Registration means that a social worker will contact the
is used more for elderly patients. patient and provide:
• Ciliary body ablation: Usually done with a diode laser to • access to a mobility officer, a teacher of braille, and
burn the ciliary body and reduce production of aqueous daily living courses;
humour. The process may need to be repeated to keep • free radio and cassette aids;
the pressure low and patients normally need to continue drug • details of the RNIB talking-book service.
treatment. • In addition, the registered blind (but not necessarily the
Other laser methods (iridotomy and iridoplasty) are used partially sighted) are eligible for:
in angle closure glaucoma. • a slightly higher rate of income support;
• extra Housing Benefit, Income Support, and Council
Surgical Treatment Tax Benefit;
Trabeculectomy is the most effective glaucoma filtration pro- • increased income tax allowance;
cedure when medical or laser treatment has failed. A channel • reduced fares and a Blue Parking Badge;
is created to allow flow from the anterior chamber. Once it • free sight tests; and
has been performed, bacterial conjunctivitis can develop more • reduction in the TV licence fee.
readily into endophthalmitis.
The Royal National Institute for the Blind (RNIB) provides
Visual Handicap information, support, and advice for anyone with a serious
sight problem. Website: www.rnib.org.uk; helpline 0303 123
• Over two-thirds of people who are eligible are not regis- 9999 (interpreters are available); email: helpline@rnib.org.uk.
tered as blind or partially sighted. The majority of these
receive no social care services whatsoever.
• Two-thirds of all people with visual impairment have an Prescribing for Patients With Contact Lenses
additional disability or serious health problem. (Mitchell & Edwards, 2001)
• Over 50% of visually impaired people live alone yet few Topical Applications
have been offered any training in daily living skills. Almost Avoid:
half of all visually impaired people cannot cook for them- • eye ointments;
selves because of hazards in the kitchen (Royal National • eye drops containing preservatives (in soft lenses);
Institute for the Blind, 2002). • topical steroids;
• coloured drops: fluorescein and rose bengal;
Certification and Registration • sympathomimetics.
• Certification is by a consultant ophthalmologist on
form CV1. Systemic Drugs
• Blind certification is for those with a visual acuity (VA) Avoid drugs that:
below 3/60, or with a VA of up to and including 6/60 • reduce or alter tear secretion: oral contraceptives,
but with gross visual field restriction. hormone replacement therapy (HRT), drugs with
• Certification as partially sighted is not defined by statute. It anticholinergic activity (e.g., tricyclic antidepressants),
is appropriate for patients with VA of 6/60 or worse with beta blockers, diuretics, isotretinoin;
full visual fields, or those with a VA better than that but • reduce blinking: benzodiazepines;
with restriction of visual fields. A patient with a homony- • stain the lens: nitrofurantoin, rifampicin, sulfasalazine;
mous hemianopia, for instance, is likely to be eligible. • are concentrated in the lens and can irritate the eye:
• Registration is voluntary and would normally be offered aspirin.
at the time of certification. Registration entitles a person
to a range of benefits and concessions as well as help from PATIENT INFORMATION
some local voluntary groups. The receipt of a certificate
The British Contact Lens Association; www.bcla.org.uk
of vision impairment (CVI) by the social services depart- (search on “do’s and don’ts”).
ment entitles that person to have his or her name added
to the register. This also acts as a trigger for social services
to arrange an assessment of the person’s social care needs.
• Patients with low vision but not certified as blind or Corrective Surgery for Myopia
partially sighted should be encouraged to self-refer to
social services using the “Low Vision Leaflet” (LVL) avail- • This remains a service provided by the private sector that
able from high street opticians. They are also likely to is not regulated.
benefit from a low visual aid referral which is normally • While legally in the United Kingdom any registered
through the ophthalmology department. medical practitioner may perform refractive surgery, the
Royal College of Ophthalmologists recommends that it based cohort study. British Medical Journal (Clinical Research Ed.),
should only be performed by a fully trained ophthalmolo- 325, 1396–1397.
gist who has undergone additional specialist training in Luchs, J. (2008). Efficacy of topical azithromycin ophthalmic solution
refractive surgery. He or she may or may not also be an 1% in the treatment of posterior blepharitis. Advanced Therapy,
25(9), 858.
NHS consultant.
Mitchell, R., & Edwards, R. (2001). Prescribing for patients who wear
• Advise patients to download the leaflet “Refractive Surgery” contact lenses. Prescriber, 40–44.
from the Royal College of Ophthalmologists at www. O’Neill, S., & McAndrew, D. (2016). The validity of visual acuity
rcophth.ac.uk/ (search on “refractive surgery”). assessment using mobile technology devices in the primary care
setting. Australian Family Physician, 45, 212–215.
References Opstelten, W., & Zall, M. J. W. (2005). Managing ophthalmic herpes
zoster in primary care. British Medical Journal (Clinical Research
Allen, D., & Vasavada, A. (2006). Cataract and surgery for cataract. Ed.), 331, 147–151.
British Medical Journal, 333, 128–132. Pandit, J. C. (1994). Testing acuity of vision in general practice: Reach-
AREDS2 Research Group. (2013). Lutein/zeaxanthin and ing recommended standard. British Medical Journal (Clinical Research
omega-3 fatty acids for age-related macular degeneration. The Ed.), 309, 1408.
Age-Related Eye Disease Study 2 (AREDS2) controlled random- Pepose, J. S., Ahuja, A., Liu, W., Narvekar, A., & Haque, R. (2018).
ized clinical trial. JAMA. Randomiszed, controlled, phase 2 trial of Povidone-Iodine/
Argrawal, A., Bruce, G., Cowie, A., et al. (2018). Scottish Dry Eye Dexamethasone Ophthalmic Suspension for Treatment of Adenoviral
Guidelines version 1.1 (2018) NHS Scotland. Conjunctivitis. American Journal of Ophthalmology, 194, 7–15.
Au Jefferies, J., Perera, R., & Evert, H. (2011). Acute infective conjunc- Potamitis, T., Aggarwal, R. K., Tsaloumas, M., et al. (1994). Driving,
tivitis in primary care: Who needs antibiotics? Individual patient glaucoma and the law. British Medical Journal (Clinical Research
data meta-analysis. British Journal of General Practitioner, 61(590). Ed.), 309, 1057–1058.
Crick, R. P., & Tuck, M. W. (1995). How can we improve the detec- Rietveld, R. P., van Weert, H. C., ter Riet, G., et al. (2003). Diagnostic
tion of glaucoma? British Medical Journal (Clinical Research Ed.), impact of signs and symptoms in acute infectious conjunctivitis:
310, 546–547. Systematic literature search. British Medical Journal (Clinical Research
Cugati, S., et al. (2013). Treatment options for central retinal aftery Ed.), 327, 789.
occlusion. Current Treatment Options in Neurology, 15, 63–67. Royal College of Ophthalmologists Guidelines (2015). Age-related
Easty, D. L. (1993). Is an eye pad needed in cases of corneal abrasion? macular degeneration: Guidelines for management. London: RCOphth.
British Medical Journal (Clinical Research Ed.), 307, 1022. Royal National Institute for the Blind. (2002). Progress in sight: National
Everitt, H. A., Little, P. S., & Smith, P. W. F. (2006). A randomised standards of social care for visually impaired adults. www.rnib.org.uk/
controlled trial of management strategies for acute infective con- social_services.
junctivitis in general practice. British Medical Journal (Clinical Sheikh, A., & Hurwitz, B. (2005). Topical antibiotics for bacterial
Research Ed.), 333, 321–324. conjunctivitis: Cochrane systematic review and meta-analysis update.
Guidelines on the Management of Communicable Diseases in Schools British Journal of General Practitioners, 55, 962–964.
and Nurseries. (2008). Health Protection Agency. www.hpa.org.uk/ Weaver, C. S., & Terrell, K. M. (2003). Do ophthalmic nonsteroidal
schools. anti-inflammatory drugs reduce the pain associated with simple
Kanski, J. J. (1989). Clinical ophthalmology (2nd ed.). London: corneal abrasion without delaying healing? Annals of Emergency
Butterworths. Medicine, 41, 134–140.
Kelly, S., Thornton, J., Lyratzopoulos, G., et al. (2004). Smoking Wormald, R. (2003). Treatment of raised intraocular pressure and
and blindness. British Medical Journal (Clinical Research Ed.), 328, prevention of glaucoma. British Medical Journal (Clinical Research
537–538. Ed.), 326, 723–724.
Kirwan, J. F., Nightingale, J. A., Bunce, C., et al. (2002). β blockers
for glaucoma and excess risk of airways obstruction: Population
22
Skin Problems
KIERAN DINWOODIE, ANCHAL GOYAL, CATRIONA NISBETT,
JANE COLGAN
Atopic Eczema Pustular Psoriasis
Complications of Eczema Erythrodermic Psoriasis
General Advice Nail Psoriasis
General Advice for Bottle and Breastfed Infants Lichen Planus
Emollients Investigations
Steroids Management
Treatment of Mild Flares Referral
Treatment of Moderate Flares
Treatment of Severe Flares Infection and Infestation
Referral Viral Infections
Warts
Irritant and Allergic Contact Dermatitis (Including Hand Herpes Simplex
Dermatitis) Disseminated Hsv Can Be Life Threatening—Early
Diagnostic Investigations Recognition and Urgent Referral Is Required. Herpes Zoster
Management Bacterial Infection
Seborrheic Dermatitis Staphylococci and Streptococci
Management Necrotizing Fasciitis
Infants Erythrasma
Seborrheic Dermatitis in the Scalp and Beard Gram-Negative Bacilii
Seborrheic Dermatitis of the Face and Body Lyme Disease
Severe or Recalcitrant Symptoms Fungal Infections
Management of Ocular Symptoms Tinea
Candidiasis
Acne Vulgaris
Pityriasis Versicolour
Presentation
Parasitic Infections
Diagnosis Scabies
Managment Head Lice
Topical Treatment Pubic Lice
Systemic Therapy
Skin Lesions and Malignancies
Referral
Benign Skin Lesions
Acne Rosacea Seborrhoeic Keratosis
Management Dermatofibroma/Histiocytoma
Sebaceous Hyperplasia
Psoriasis
Keratoacanthoma
Management
Precancerous Skin Lesions
Chronic Plaque Psoriasis
Actinic (Solar) Keratosis
Scalp Psoriasis Bowen Disease (Squamous Cell Carcinoma in situ)
Genital, Facial, and Flexural Psoriasis Cutaneous Horn
Guttate Psoriasis Atypical/Dysplastic Naevus
381
Malignant Skin Lesions Generalized Hyperhidrosis

Basal Cell Carcinoma (Rodent Ulcer) Investigations
Squamous Cell Carcinoma Management
Malignant Melanoma Management of Generalized Hyperhidrosis
Venous/Varicose Eczema Hidradenitis Suppurativa
Leg Ulcers Urticaria
General Advice Alopecia
Management Treatment Alopecia Areata
Referral
Hyperhidrosis
Primary Hyperhidrosis
GUIDELINES • Atopic eczema accounts for 30% of all dermatological con-

sultations in primary care and the prevalence is increasing.
Clinical Knowledge Summaries (CKS) website covers most of
the topics in this chapter by subject. Home page: https://
• Eighty percent of cases occur before the age of 5 years.
cks.nice.org.uk. • The person’s age affects the distribution:
• Infancy: Eczema primarily involves the face, the scalp,
and the extensor surfaces of the limbs. The nappy area
is usually spared.
Atopic Eczema • Children: Localization to the flexures of the limbs is
more likely.
• Adults: Flexural involvement. Eczema on the hands
GUIDELINES
can be the primary manifestation.
Eczema—atopic. NICE Clinical Knowledge Summaries.
Retrieved from https://cks.nice.org.uk. Complications of Eczema
Primary Care Dermatology Society. (2017). Eczema—
atopic eczema. Clinical guidance. Retrieved from www.pcds. • Infection:
org.uk/clinical-guidance/atopic-eczema. • Bacterial infection with Staphylococcus aureus may
Scottish Intercollegiate Guidance Network. (2011). The present as typical impetigo or worsening of eczema
management of atopic eczema in primary care. SIGN with increased redness, oozing, and crusting.
guideline 125. Retrieved from www.sign.ac.uk.
• Herpes simplex infection presents with grouped vesicles
and punched out erosions.
• Superficial fungal infections are also more common.
• Psychological:
• Atopic eczema is a chronic, relapsing, itchy skin condi- • Causes considerable distress.
tion. If it’s not itchy it’s not eczema. • In preschool children it increases the rate of behavioural
• It is typically an episodic disease of flares and remissions. problems, fearfulness, and dependence on parents.
• There is a tendency for it to improve in adult life. • In schoolchildren there can be problems with time off
• There is some evidence that the prognosis of atopic eczema school, impaired performance, teasing, and bullying.
is worse when the onset is early and in children with • Sleep disturbance is an important problem for sufferers
associated asthma. and their families.
• There is no known single cause for atopic eczema; it • Erythroderma usually occurs in people with worsening or
occurs in genetically susceptible individuals when exposed unstable eczema.
to environmental irritants or allergens. • Eye abnormalities:
• It may be exacerbated but not caused by trigger factors • Conjunctival irritation can occur.
such as stress or hormonal changes (premenstrual flares • Cataracts can also occur in people with severe atopic
occur in 30% of women and pregnancy can adversely eczema aged 15 to 25 years.
affect eczema in up to 50%). • Retinal detachment is rarely associated with atopic eczema.
• A specific genetic cause has not been identified but it is • Failure to thrive and growth restriction can occur in chil-
present in 80% of children where both parents are affected dren with severe atopic eczema.
with eczema and in 60% where one parent is affected
with eczema. General Advice
• Environmental factors have an important role, particularly
exposure to pets, house dust mites, pollen, and food aller- • In children, reassure parents that eczema often improves
gens (particularly cow milk or egg). with time.
CHAPTER 22 Skin Problems 383
• In adults, explain that eczema is a chronic illness char- • Prescribe emollients in large quantities: 600 g/week for
acterized by flares that can usually be controlled with an adult; 250 g/week for a child.
appropriate treatment. • Emollients should be applied by smoothing them into
• Advise the person/carer to avoid scratching (keep nails the skin along the line of hair growth.
short: use baby scratch mitts).
• Advise the person to avoid trigger factors (e.g., clothing, Steroids
soap, detergents, animals, heat).
• Do not offer advice about complementary therapies other • Topical steroids are safe and effective antiinflammatory
than that they are generally not recommended. skin preparations that are used to control eczema (and
• Diet should not be altered unless there is a known allergy many other skin conditions).
to specific foods and exclusion diet has been recommended • They are available in different strengths.
by a health care professional. • Side effects are rare if used appropriately but they can
• Advise the person to bath daily, using a bath additive cause skin thinning, telangiectasia, stretch marks, easy
(e.g., Hydromol Bath or Dermol) for no longer than 10 bruising, and hypertrichosis.
minutes, to then pat dry and apply emollient. • Topical steroids should be used cautiously on eyelid skin
as excessive use over weeks to months can cause glaucoma.
General Advice for Bottle and • Fingertip units guide the amount of steroid to be applied
Breastfed Infants to a body site:
• One hand: 1 fingertip unit
• The mothers of breastfed infants in whom allergy is sus- • One arm: 3 fingertip units
pected to be the cause of moderate or severe eczema may • One foot: 3 fingertip units
require referral for dietary advice. • One leg: 6 fingertip units
• A 6- to 8-week trial of hydrolysed protein formula milk • Face and neck: 2.5 fingertip units
or amino acid formula milk may be tried in bottle-fed • Trunk (front and back): 14 fingertip units
infants less than 6 months of age in whom eczema is not • Entire body: ~40 fingertip units
controlled by emollients or mild topical steroids. • Table 22.1 details commonly prescribeable topical steroids.
• Children who then respond well to a change in their
formula milk will require referral to a dietician. Treatment of Mild Flares
• Children who do not respond should have cow milk • In addition to emollients consider prescribing a mild topical
restarted with monitoring of their eczema. hydrocortisone cream or ointment for areas of red skin.
• Treatment should continue for 48 hours after the flare
Emollients has been controlled.
• Emollients are the mainstay of treatment for patients Treatment of Moderate Flares
with eczema. • Prescribe intensive treatment until the flare is controlled.
• Most emollients are plain but some contain active ingre- • Prescribe a generous amount of emollient and advise fre-
dients. These are generally best avoided. Active ingredients quent and liberal use.
include urea (Balneum Plus and Eucerin Intensive) and • Prescribe a moderately potent topical steroid to be used
lanolin (E45 and Oilatum Bath Formula). on inflamed areas (e.g., betamethasone valerate 0.025%;
• Ointments are usually poorly tolerated compared to creams, clobetasone butyrate).
which affects their compliance.
• There is no evidence from controlled trials to support the
use of one emollient over another: The correct emollient
is the one the person will use. TABLE
22.1 Commonly used Topical Steroids
• It may be more convenient to use better tolerated products
(creams and lotions) during day and use ointments at Examples of Commonly Prescribed
night. Application of an emollient every 2 to 3 hours Potency Treatments Available
should be considered normal. Patients often underestimate
the amount they should be using. Mild Hydrocortisone (0.5%, 1%, 2.5%)
• Advise the patient that it is particularly important to use Moderate Clobetasone butyrate (Eumovate)
emollients during or after bathing (within 3 minutes). Betamethasone valerate 0.025%
• Advise the patient that soap can be very damaging to (Betnovate RD)
people with eczema and therefore a soap substitute should Potent Betamethasone valerate 0.1% (Betnovate)
also be used. Mometasone furoate (Elocon)
• Do not prescribe aqueous cream as it can cause skin reactions. Fluocinolone acetonide (Synalar)
• Where possible prescribe an emollient with a pump dis- Very potent Clobetasone proprionate (Dermovate)
penser to minimize risk of bacterial contamination.
• Treatment should be continued for 48 hours after the • Refer for routine dermatology appointment if:
flare has settled. • diagnosis has become uncertain;
• Steroids should be applied once a day and only increased • current management does not control eczema satis-
to twice a day if skin does not improve. factorily (person having one to two flares/month or
• For delicate areas such as the face and flexures con- person is reacting adversely to many emollients);
sider starting with a mild potency topical steroid and • facial eczema has not responded to appropriate treatment;
increase to moderately potent corticosteroid only if • contact allergic dermatitis is suspected;
necessary. • there is recurrent secondary infection;
• If there are areas of infected skin treat with a topical • eczema is associated with significant social or psycho-
antibiotic. These should not be used for longer than 1 logic problems.
week due to the risk of antibiotic resistance. • Refer people in whom food allergy is suspected to either
• Between flares encourage the frequent and liberal use of immunology or paediatrics (children <6 months who
emollients even when skin appears clear. have widespread eczema that has not responded to emol-
• Consider maintenance topical steroids to control areas lients or mild topical steroids).
of skin prone to frequent flares. Consider one of the
following maintenance regimens:
• Stepdown approach: prescribing the lowest potency and Irritant and Allergic Contact Dermatitis
amount of topical steroid to control the condition (Including Hand Dermatitis)
• Intermittent treatment: weekend therapy on Saturdays
and Sundays only (probably the preferred option based
GUIDELINES
on the evidence of risks vs benefits)
• Topical calcineurin inhibitors (tacrolimus and pimecro- National Institute for Health and Care Excellence. (2013).
limus) are a second line option. However, they should Dermatitis—contact. NICE clinical knowledge summaries.
Retrieved from https://cks.nice.org.uk.
only be prescribed by a specialist (including general Primary Care Dermatology Society. (2014). Eczema:
practitioners [GPs] with a specialist interest in derma- Contact allergic dermatitis. Clinical guidance. Retrieved from
tology). They have evidence similar to that of moder- www.pcds.org.uk/contact_dermatitis.
ately potent steroids. They do not cause skin atrophy,
but long-term side effects are unknown and there is a
theoretic long-term risk of malignancy.
• If there is severe itch consider prescribing a 1-month trial • Contact dermatitis is an inflammatory skin reaction that
of a nonsedating antihistamine. occurs in response to an external agent acting as an irritant
or allergen.
Treatment of Severe Flares • Allergic contact dermatitis is a type IV hypersensitivity
• Prescribe a generous amount of emollients and advise reaction that occurs after sensitization and subsequent
frequent and liberal use. reexposure to an allergen.
• Prescribe a potent topical steroid (e.g., betamethasone • Irritant contact dermatitis is an inflammatory response
valerate 0.1%). Aim for a maximum 5-day use. Again that occurs after damage to the skin, usually by chemicals.
steroids should be prescribed once a day and only increased • In practice the two often coexist.
to twice daily if skin does not improve. • Atopic eczema is strongly associated with irritant contact
• For delicate areas such as the face and flexures, use a dermatitis. The hands and face being affected in prefer-
moderately potent topical steroid (e.g., betamethasone ence to other body parts helps to distinguish contact
valerate 0.025%, clobetasone butyrate 0.05%). Aim for dermatitis from other forms of dermatitis.
a maximum 5-day use. • Pompholyx eczema is a specific type of hand and foot
• If itching is severe and affecting sleep prescribe a sedating eczema characterized by vesicles. It is thought to be mul-
antihistamine (e.g., chlorphenamine) for adults and chil- tifactorial and related to sweating as flares occur in hot
dren aged 6 months or over. weather/humid conditions. However, there is a strong
• If there is severe, extensive eczema causing psychologic association with irritant and allergic contact dermatitis.
distress consider a short course of oral corticosteroids • Physical conditions such as heat, cold, repeated frictional
(30 mg prednisolone for 7 days). exposure, and low humidity can also increase the likeli-
• If there are signs of infection consider an oral antibiotic. hood and severity of contact dermatitis.
• Consider maintenance steroid therapy as per treatment • Common irritants:
of moderate flares. • Water
• Detergents and soaps
Referral • Solvents and abrasives
• Machining oils
• Admit to hospital if eczema herpeticum or erythrodermic • Acids and alkalis, including cements
eczema. • Powders, dusts, and soils
• Common allergens: • Consider short-term use of a systemic corticosteroid if

• Cosmetics: particularly fragrances, hair dyes, and nail there is:
varnish • significant impairment of function (e.g., eczema in
• Metals (e.g., nickel/cobalt in jewelery) the hands);
• Topical medications, including rare allergy to topical • extensive acute dermatitis (>20% of body affected).
corticosteroids • Evidence would suggest antihistamines are not helpful
• Rubber additives in this condition although in practice they are commonly
• Textiles particularly from dyes and formaldehyde resins prescribed.
• Plants: composite group (chrysanthemum and sunflow- • You may need to consider referral to an occupational
ers), daffodils, tulips, primula the most common health physician.
• Some occupations particularly associated with contact
dermatitis are: Seborrheic Dermatitis
• agricultural workers;
• beauticians;
GUIDELINE
• chemical workers;
• cleaners; National Institute for Health and Care Excellence. (2013).
• construction workers; Seborrheic dermatitis. NICE Clinical Knowledge Summaries.
Retrieved from https://cks.nice.org.uk.
• cooks and caterers;
• electronic workers;
• hairdressers;
• health and social care workers;
• machine operators; • Seborrheic dermatitis is a common inflammation of the
• mechanics; skin, occurring in areas rich in sebaceous glands (scalp,
• metal workers. nasolabial folds, ears, eyebrows, and chest). Blepharitis is
also commonly associated.
Diagnostic Investigations • It is estimated to affect 3% to 5% of the population.
• It presents as erythematous plaques or patches that can
• A subgroup of people with contact dermatitis will need vary from mild dandruff to dense, diffuse adherent scale.
further investigations to identify the causative stimuli and Dandruff is the precursor of seborrheic dermatitis.
to obtain treatments not available in primary care. • The exact cause of seborrheic dermatitis is unknown. It
• The gold standard investigation is patch testing. This needs is thought to be associated with the presence of Malassezia
expert knowledge as interpretation is complex. yeasts.
• Consider referral to a dermatologist if: • It is more common in men than women and commonly
• the person has chronic, recurring dermatitis despite occurs in infants younger than 3 months of age and in
appropriate avoidance measures and appropriate adults 30 to 60 years of age. It is more common in people
strength corticosteroid treatment; with Parkinson disease than the general population and
• there is suspicion of contact dermatitis but no clear it has a greater prevalence in immunocompromised people
history of exposure; than in healthy adults. It has been established as a pos-
• there is a suspicion of occupational contact dermatitis sible marker for early human immunodeficiency virus
that does not respond to corticosteroid therapy. (HIV) infection and this should be considered particularly
where there is severe disease.
Management • Seborrheic dermatitis in infants (commonly called cradle
cap) usually appears within the first 6 weeks of life and
• Identify the stimulus with a full occupational and recre- most commonly affects the scalp. In infants it usually
ational history. Identification and subsequent avoidance gets better over a few weeks with only some cases persist-
of the stimulus is an essential part of management. ing past 8 to 9 months.
• There should be frequent and liberal use of emollient.
• A soap substitute should be used instead of normal soap.
Management
• Gloves should be used for all house/occupational work.
Patients can use either cotton gloves, vinyl gloves, or rubber Infants
gloves. Vinyl or rubber gloves should be used for wet work. • Parents should be reassured it is not a serious condition,
• Treat localized acute dermatitis with a topical steroid it does not bother the baby, and it spontaneously resolves
appropriate to the severity and location of the dermatitis over 6 to 12 months.
(more information available in the atopic eczema section). • Advise parents to regularly wash the scalp with a baby
It is worth remembering that affected skin on the hands shampoo and then use a gentle brush to loosen scales.
and feet may need a potent or very potent topical steroid • Softening the scales with baby oil first can help remove
(e.g., betnovate or dermovate). the scales; and if particularly thick, soaking them overnight
in olive oil and then washing off in the morning Acne Vulgaris
can help.
• If the above simple measures are not effective prescribe • Acne vulgaris is one of the most common skin disorders
a topical imidazole cream (clotrimazole or miconazole). affecting the adolescent population. It can cause great
Treat until symptoms resolve but ideally not for more psychological distress in teenagers and young people.
than 4 weeks. • It usually starts at puberty, peaks at around 16 to 20 years
of age, and tends to self-resolve in most people by the third
Seborrheic Dermatitis in the Scalp and Beard decade. In about 7% of patients it persists into the fourth
• Reassure the person that seborrheic dermatitis is not caused and fifth decades or may resolve and recur in later life.
by lack of cleanliness or excessive dryness of the skin and • There are four aetiological factors:
is not transferable. 1. Excessive sebum production, leading to seborrhoea; it
• Explain that treatment cannot cure seborrheic dermatitis is androgen mediated
but can control it. Symptoms often recur after treatment 2. Comedone (blackhead/whitehead) formation, due to
is stopped. excessive proliferation of keratinocytes in intrafollicular
• Remove thick crusts or scales on the scalp before using ducts
an antifungal shampoo. This can be done by applying 3. Ductal colonization with Propionibacterium acnes
warm olive oil to the scalp for several hours, then washing 4. Inflammation leading to papule, pustule, and nodule
with a coal tar shampoo. formation
• Prescribe ketoconazole 2% shampoo for adolescents and • Acne can be graded in various ways. The simplest way to
adults (selenium sulfide shampoo is an alternative). Sham- grade it is as follows:
poos should be used twice a week for at least 1 month. 1. Noninflammatory acne/comedonal acne
Once symptoms are under control their use can then be 2. Inflammatory acne subdivided into mild, moderate,
reduced to once a week. Shampoos can also be applied and severe acne
to the beard area.
• If the patient has severe itching of the scalp consider Presentation
coprescribing 4 weeks of treatment with a potent topical
steroid scalp application (e.g., Bettamousse or Elocon • It has usually been present for months. It tends to be worse
scalp). These are not appropriate for the beard area as in autumn and winter due to lack of sunlight. Patients
they may cause thinning of skin on the face. can present with painful lesions; however, most times it is
• Topical steroids are not appropriate for long-term use and the psychological distress caused by the lesions that brings
their use as maintenance treatment is not recommended. patients to seek medical advice. Lesions usually develop
• Consider dermatology referral if symptoms have not on the face, neck, upper limbs, chest, and upper back.
resolved after 4 weeks. • Mild inflammatory acne usually presents with inflamed
comedones (open or closed), papules, and pustules.
Seborrheic Dermatitis of the Face and Body • Moderate inflammatory acne usually presents with inflamed
• Prescribe ketoconazole 2% cream twice daily for 4 weeks papules and pustules.
(clotrimazole or miconazole can also be used). • Severe inflammatory acne usually presents with nodules
• Consider adding a mildly potent topical corticosteroid and cysts, which can be painful.
(e.g., hydrocortisone 1%) to help settle inflammation. • Some special subtypes of acne are:
Corticosteroids should only be used short term (1–2 • acne conglobata: severe nodulocystic acne which is more
weeks). prominent on the trunk than on the face and tends
• Ketoconazole shampoo can also be used as a body wash. to lead to ulceration;
• acne fulminans: acute presentation with cystic ulcerat-
Severe or Recalcitrant Symptoms ing acne, typically seen in teenage boys. Commonly
• A systemic itraconazole 100 mg/day for 14 days can be associated with systemic illness such as myalgia, arthral-
considered along with dermatology referral. gia, fever, fatigue, leukocytosis, and raised erythrocyte
• HIV testing should be considered. sedimentation rate (ESR);
• drug- or chemical-induced acne: usually presents in
Management of Ocular Symptoms atypical sites and in the wrong age group. Common
• Lid hygiene with cotton wool soaked in cool boiled water triggers are systemic steroids, isoniazid and adreno-
may be enough. corticotropic hormone (ACTH), chlorinated hydro-
• Artificial tears should be applied liberally if eyes are dry carbons (in insecticides, fungicides), and cosmetics;
or sore. • infantile acne: rare, seen in neonates and may be self-
• As with acne rosacea, systemic treatment with a tetracy- limiting and transient. Only seen in boys in the first
cline can be used for 6 to 8 weeks if symptoms are par- 2 years of life. Presentation is with comedones, papules,
ticularly troublesome. Erythromycin is an alternative if pustules. Treatment is usually required with oral eryth-
patient is unable to take tetracycline. romycin or co-trimoxazole.
Diagnosis Treatment should be reviewed for efficacy and tolerability

after 8 to 12 weeks.
• Diagnosis is clinical and does not usually require any • Hormonal contraceptives such as co-cyprindiol can be con-
investigations. sidered in women. It is not licensed as a contraceptive in
• Differential diagnoses include: the UK but used in moderate to severe acne in women.
• perioral dermatitis: perioral non comedonal erythe- Patients should be informed about side effects such as
matous papules that tend to worsen with topical steroids increased risk of venous thromboembolism.
but respond to oral tetracycline; • Oral retinoids should not be prescribed or managed in
• seborrhoeic eczema: dry, scaly, erythematous, itchy skin primary care.
on scalp and upper trunk;
• rosacea: non comedonal erythema or telangiectasia with Referral
flushing that is seen in older people. Truncal involve-
ment is rare. May be associated with rhinophyma. • Refer to a dermatologist:
• severe inflammatory acne;
Management • patients with any grade of acne with scarring;
• patients who have failed to respond to two or more oral
• Irrespective of treatment the following should be discussed antibiotic courses of at least 12 weeks duration each;
with all patients: • diagnostic uncertainty.
• Acne can be chronic and take a long time to respond • All patients who have had treatment for acne can be
to treatment. referred to plastic surgery for scar revision; however, they
• No response to treatment may be seen in the first 6 should be allowed a period of 12 to 18 months post
to 8 weeks. treatment to see if the skin remodels itself.
• Continuing treatment for at least 6 to 8 months if
responding to treatment is important. Acne Rosacea
• Stress-induced and premenstrual flare may be seen and
sunlight usually causes temporary improvement but
GUIDELINE
not recommended due to high risk of skin cancer.
• Treatment should be based on severity of acne and aetiol- Primary Care Dermatology Society. (2018). Rosacea. Clinical
ogy or pathogenesis. guidance. Retrieved from www.pcds.org.uk/rosacea.
Topical Treatment
• Indicated on its own in mild inflammatory acne, in con- • Clinical appearance is similar to acne vulgaris; however,
junction with oral treatment in moderate acne, and as there is marked erythema and telangiectasia. There are
maintenance after oral treatment. associated papules and pustules but no comedones (helps
• The choice of treatment can be broken down based on differentiate from acne vulgaris).
mode of action, as follows: • Usually presents on face, particularly on cheeks, nose,
• Comedolytic and anti inflammatory therapies: milder chin, forehead, and tip of nose and most commonly pre-
forms such as benzoyl peroxide and azelaic acid to sents in those aged over 40.
stronger treatments such as topical retinoids (e.g., • Complications include blepharitis, conjunctivitis, keratitis,
adapalene, all-trans retinoic acid, isotretinoin) lymphoedema of face, and rhinophyma.
• Antibiotics: clindamycin, erythromycin, tetracycline • Differential diagnoses include:
• Combination therapies: Duac, Isotrex, Zineryt, and Epiduo, • acne vulgaris: usually have comedones and earlier age
which include a comedolytic therapy and antibiotic of presentation;
• seborrhoeic eczema: usually scaly, around nasolabial areas
Systemic Therapy and scalp. Can be itchy. No pustules;
• Oral antibiotics should be considered in acne not respond- • perioral dermatitis: usually in younger people and
ing to topical treatment or where acne is difficult to reach restricted to around the mouth;
(e.g., on the back). Common regimes include: • systemic lupus erythematosus (SLE): usually differentiated
• oxytetracycline 500 mg twice a day. Not used in chil- by lack of papules and pustules and patients are often
dren under 12 years of age. Warn patients regarding systemically unwell.
photosensitivity;
• lymecycline 408 mg daily. Side effects are the same as Management
tetracycline;
• doxycycline 100 mg daily or, if not tolerated, 50 mg • Oral antibiotics are the mainstay of treatment. Usually
daily. Side effects include photosensitivity; oxytetracycline 250–500 mg twice daily for 2 to 3 months.
• erythromycin 500 mg twice daily. Common side effects If there is no response then a further course should be
are gastrointestinal (GI). prescribed. Sometimes long-term treatment may be
required. Alternative regimes include doxycycline 100 mg • HIV infection/acquired immunodeficiency syndrome
daily, erythromycin 250–500 mg twice daily, lymecycline (AIDS).
408 mg once daily, minocycline 100 mg daily. • Psoriasis is associated with several other conditions:
• Topical treatment with 0.75% metronidazole gel or cream • Psoriatic arthritis
(Rozex/Metrogel) twice a day. Consider topical ivermectin • Traditional cardiovascular (CV) risk factors (hyperten-
daily for 16 weeks. Repeat course if effective. Discontinue sion, hyperlipidaemia, diabetes)
if no response after 12 weeks. • Inflammatory bowel disease
• Treatment of telangiectasia includes brimonidine gel/ • Obesity
Mirvaso. Warn patient regarding hypopigmentation and • Venous thromboembolism (VTE)
the possible bleaching effect which is temporary and settles • Nonmelanoma skin cancer
once treatment is stopped.
Management
Psoriasis General points to consider:
• Be aware of the possibility of joint involvement and refer
to rheumatology if psoriatic arthropathy is affected.
GUIDELINES
• Psoriasis can be associated with mood disturbance and
Scottish Intercollegiate Guidance Network. (2010). Diagnosis therefore this should be enquired about at review.
and management of psoriasis and psoriatic arthritis in adults. • Psoriasis is often thought of as a nonitchy skin condition;
SIGN guideline 121. Retrieved from www.sign.ac.uk.
however, patients often do find itch a problem and anti-
Psoriasis: Assessment and management. NICE clinical histamines can help.
guideline 153, updated 2017. Retrieved from www.nice.org.uk. • All patients with psoriasis should have yearly follow-up
to address cardiovascular risk factors. Despite being associ-
ated with an increased risk of CV disease it is not included
• Psoriasis is a chronic inflammatory multisystem disease in most CV risk assessment tools.
with predominantly skin and joint manifestations. It is • Compliance with treatment is improved by good patient
characterized by scaly skin lesions which can be in the understanding of the condition. Signposting to online
form of patches, papules, or plaques. Although it is usually web resources and patient information leaflets should be
chronic, the guttate form typically resolves within 3 to considered.
4 months of onset.
• Forms of psoriasis include: Chronic Plaque Psoriasis
• chronic plaque psoriasis (80%–90%): this includes
flexural, scalp, and facial psoriasis; • Offer topical treatments first line. National Institute for
• localized pustular psoriasis of the palms and soles; Health and Care Excellence (NICE, 2012) advises prescrib-
• nail psoriasis; ing a potent topical steroid (e.g., betnovate 0.1% in
• guttate psoriasis; morning) and a topical vitamin D preparation separately
• erythrodermic psoriasis; (e.g., Dovonex) and avoiding using combination products
• generalized pustular psoriasis. (NICE, 2012). However, many general practitioners (GPs),
• Around 1% to 3% of the population has psoriasis; it is dermatologists, and general practitioners with a special
more common in white people and, although presenta- interest (GPwSIs) routinely use a combination product
tion can be at any age, psoriasis most commonly presents (e.g., Dovobet) to improve compliance and bring about
between 15 and 30 years. Men and women are equally rapid control of symptoms. The Primary Care Dermatology
affected. Society (PCDS) and British Association of Dermatologists
• Factors that may influence the onset or cause an exacerba- (BAD) recognize this variation and their guidelines differ
tion of preexisting psoriasis include: from NICE.
• streptococcal infection (strongly associated with guttate • Topical corticosteroids are only suitable for treating
psoriasis but association may also apply to plaque localized psoriasis and repeat prescriptions should be
psoriasis); monitored as there is a risk of destabilizing disease. They
• drugs (lithium, antimalarials, beta blockers, nonsteroidal should not be applied for more than 8 weeks. Treat-
antiinflammatory drugs [NSAIDs], angiotensin- ment can be restarted after a 4-week treatment break if
converting enzyme [ACE] inhibitors, trazodone); needed.
• sunlight (usually beneficial but may exacerbate psoriasis • Emollients should be offered to reduce scale and help
in small number of people); with itch.
• trauma (the Koebner phenomenon); • If scale is a particular problem, preparations containing
• postpartum hormonal change; salicylic acid may be useful.
• psychologic stress; • Review the patient after 4 weeks. Continue treatment
• excessive alcohol intake; until skin is clear/nearly clear (i.e., skin is smooth but
• smoking; can still be red/pink).
• Topical vitamin D preparations alone can be used as • Refer to dermatology if there is a poor response to topical
maintenance treatment for chronic plaque psoriasis. steroid or continuous topical treatment is required to
• If the patient has a poor response to 8 weeks of potent maintain disease control.
topical steroid advise the patient to stop this and apply
vitamin D alone for 12 weeks twice daily. Guttate Psoriasis
• If there is still a poor response after this try a coal tar
preparation (e.g., Exorex or Carbo-Dome) once or twice • If lesions are widespread (>10% body surface area) refer to
daily. dermatology urgently for consideration of phototherapy.
• For people with treatment-resistant psoriasis con- • If lesions are not widespread reassure the person that
sider dithranol. Evidence for dithranol is very good guttate psoriasis is self-limiting and usually resolves within
but its use is limited by patient acceptance (it causes 3 to 4 months.
stinging sensation; stains clothes, furniture, and skin). • No treatment may be an option.
It should only be used for large focal plaques of pso- • If topical treatment is required consider topical treatment
riasis. The lowest strength (0.1%) should be titrated as discussed for plaque psoriasis.
slowly and only if tolerated. It should be applied once • Do not use antibiotics to treat guttate psoriasis triggered
daily. by a sore throat.
• Refer to secondary care for consideration of light treat-
ment or systemic therapy if topical treatment does not Pustular Psoriasis
control disease, if disease is widespread at onset, or if
there is psychologic distress due to disease. • If the person has generalized pustular psoriasis refer for
same-day specialist assessment and treatment.
Scalp Psoriasis • Even pustular psoriasis that is localized to hands and feet
is difficult to control and usually requires systemic treat-
• Initial treatment should be with a potent topical cortiment. It is probably advisable to refer to dermatology at
costeroid in a scalp application form (e.g., Elocon scalp). presentation and obtain same-day advice on treatment
Advise the patient to stop the corticosteroid once the in interim.
skin is clear.
• Coal tar shampoos can be considered but should not be Erythrodermic Psoriasis
used alone for people with severe scalp psoriasis.
• Review the patient at 4 weeks. If there is poor response • Refer for same-day specialist assessment and treatment. Eryth-
check compliance and offer the topical steroid in a different roderma is associated with life-threatening complications.
form such as shampoo or mousse (e.g., Bettamouse) and
consider a scalp treatment (e.g., salicylic acid, olive oil, Nail Psoriasis
coconut oil) to remove scale before further corticosteroid.
• If there is still a poor response to treatment after another • Treatment of nail psoriasis is very difficult. There is no
4 weeks consider a combined topical steroid and vitamin evidence for topical or systemic treatments.
D preparation (Dovobet scalp). • Advise patients to keep nails short, avoid manicures which
• If the above treatments do not control scalp dermatitis can exacerbate onychomycosis, and use nail polish to
consider a very potent topical steroid twice a day for 2 disguise appearance.
weeks and referral to a dermatologist.
Lichen Planus
Genital, Facial, and Flexural Psoriasis
• Lichen planus is usually distributed in the flexural aspects
• Emollients should be offered to all patients. of wrists, ankles, and the lumbar region of the back.
• A short-term mild or moderate topical steroid preparation Mouth, hair, nails, and genital areas can also be affected.
should be used for up to 2 weeks. • It is recognizable by the “6Ps”: purple, polygonic, planar
• If there is a good response to treatment repeated short courses (flat-topped) papules, and pruritic plaques.
of topical corticosteroids may be used to maintain disease • Wickham striae (white lines over lesions) are also present.
control. They should only be used for 1 to 2 weeks/month.
• Maintenance treatment for those with chronic disease Investigations
should be with a topical vitamin D preparation alone.
Dovonex is quite irritating to sensitive areas so a prepara- • Diagnosis is usually clinical but when uncertain biopsy
tion such as Silkis may be better. should be arranged to confirm.
• Potent or very potent topical steroids should not be used • Patients should be screened for hepatitis C as an associa-
on the face, flexures, or genitals. tion is recognized.
• Antifungals should not be used unless there is evidence • Exclude causative medications such as thiazide diuretics,
of fungal infection. spironolactone, beta blockers, and NSAIDs.
Management • filiform wart: long and slender, found on face and

neck;
• Give the patient an information leaflet and inform that • palmar and planter (verrucae): central dark dots in
85% will improve by 18 months but it can be a relapsing middle and may be painful;
condition. • mosaic: when warts coalesce.
• Offer antipruritic management such as antihistamines • Differentials to consider:
and/or menthol cream. • Actinic keratosis
• Treat itchy active area with potent or superpotent topical • Seborrheic keratosis
steroids until lesions improve and explain to patient that • Lichen planus
treatment may take weeks. Do not apply steroids to skin • Knuckle pads
which has postinflammatory changes (i.e., when lesions • SCC (rare but increased on sun-exposed sites or in
change from purple to grey-brown). immunocompromised patients)
• For facial lesions tacrolimus ointment or pimecrolimus • Palmoplantar keratoderma
cream may be an alternative to steroid.
• For scalp lesions offer a topical steroid scalp preparation. Management
• Mucosal areas are difficult to manage. Inform patient to • Advise the patient that warts are not harmful and most
see dentist 6 monthly to monitor for oral cancer. resolve spontaneously. To reduce risk of transmission wear
a waterproof plaster when swimming and flip-flops in
Referral the shower and avoid sharing shoes, socks, and towels.
To avoid autoinoculation do not scratch, bite, or suck
• Refer if: warts.
• there is clinical uncertainty; • No medical therapy is often most appropriate. Only con-
• it is refractory to treatment; or sider treatment if unsightly, painful, or if the patient
• there is scarring alopecia, ulceration, nail involvement, requests treatment for a persisting wart.
aggressive disease, or any suspicion of squamous cell • Salicylic acid topical preparations of 10% to 26% applied
carcinoma (SCC). daily after paring with occlusion if possible. Apply for 3
months. The main complication is irritation and they are
contraindicated in areas of poor healing and the face.
PATIENT SUPPORT ORGANIZATION • Cryotherapy. Keep the wart frozen for 5 to 30 seconds
UK Lichen Planus. www.uklp.org.uk/. repeating every 2 to 4 weeks for at least 3 months (six
treatments). Advise about risks of pain, blistering, and
hypopigmentation.
• Combination of salicylic acid and cryotherapy can be used.
Infection and Infestation • Children with verruca do not need to be banned from
swimming but can instead wear verruca socks.
Infection manifests itself in most medical specialities but is of
particular importance in dermatology. This chapter is by no Specific Wart Treatment Options
means a comprehensive listing; instead it is designed to refresh • Plantar. Paring of the skin then salicylic acid up to 50%
knowledge and is an attempt to look systematically at skin or 2-weekly cryotherapy for up to 4 months. Another
infection groupings and to remind busy general practitioners option for painful plantar warts is to treat with corn
of the most common skin infections and infestations. plasters.
• Plane. Await self-resolution, use 2% to 10% salicylic acid
cream or mild cryotherapy. Apply topical retinoic acid if
Viral Infections they are persistent.
Warts • Facial wart. Do not use salicylic acid. Mild cryotherapy
• Human papillomavirus (HPV) and warts are common. (5–10 seconds to the wart but avoid surrounding skin)
Most clear by 2 years but they can last up to 10 years in is an option and repeat every 2 to 3 weeks. Make sure
adults. patients are consented for risks associated with cryotherapy.
• Diagnosis is usually clinical and can be aided by paring • Warts in children. Await self-resolution as much faster
down the wart which will often reveal small black dots spontaneous clearing than in adults; salicylic acid is an
which represent coagulated capillaries. option, cryotherapy is often poorly tolerated.
• Malignant change is rare except in immunocompromised
individuals. Referral
• They are categorized as: • Consider referral if:
• common wart: firm and raised, resembles cauliflower, • symptomatic warts present for at least 2 years that are
on hands and feet; unresponsive to topical treatment and cryotherapy;
• plane wart: round, flat topped, found on back of hands; • uncertain diagnosis;
• immunocompromised patient; Eczema Herpeticum

• extensive mosaic warts; • Occurs in those with atopic eczema. The lesions occur
• anogenital warts, which should be referred to genito- on the eczematous skin and are punched out erosions or
urinary medicine (GUM). vesicles and are often widespread. There may be systemic
upset including fever and lethargy, and there may be
Herpes Simplex enlarged inguinal or axillary lymph nodes.
• Acquired by direct contact. • Treat with systemic acyclovir, which usually requires admis-
• Eruption may be preceded by itching, burning, tingling; sion for treatment with intravenous acyclovir, particularly
and lesions, when they appear, can be very painful. if there is systemic upset.
• Provide patients with written information. • Consider giving antibiotics for superadded bacterial infec-
• Consider the option of no treatment for herpes simplex tion which may occur.
infections that are mild and uncomplicated as episodes
will self-resolve. Neonatal Herpes Simplex Virus
• See specific treatments in the sections covering herpes • High risk if mother has genital herpes at the time of
simplex subtypes (to come). delivery, particularly if it is a primary episode. It can
• Advise patients to reduce the chances of transmission to range from localized to disseminated.
others by not kissing, participating in oral sex, or sharing • Have a low threshold for seeking advice from a
cups while symptomatic. paediatrician.
• Advise caution to contact lens wearers during an episode
to reduce the chance of herpes keratoconjunctivitis.
• Admission may be required for those who: Disseminated Herpes Simplex Virus Can Be Life
• are immunocompromised; Threatening—Early Recognition and Urgent
• are systemically unwell; Referral Are Required. Herpes Zoster
• are unable to swallow and are at risk of dehydration; • Patients over 70 (who are not immunocompromised) in
• have suspected herpes keratoconjunctivitis; or the United Kingdom are now entitled to a shingles vaccine
• have suspected eczema herpeticum (to come). which is expected to reduce the burden of shingles in
• Consider referral if: this vulnerable group.
• frequent, persistent, or severe herpes simplex virus • The first manifestation of an attack is usually pain which
(HSV); may be severe and can be accompanied by fever, headache,
• in third trimester of pregnancy with genital herpes; and malaise. The rash onset usually follows within 3 days
• lesions are refractory to oral treatment; or of pain and follows a typical dermatomal distribution.
• diagnosis is uncertain. If the rash crosses the midline then consider alternative
diagnoses.
Herpetic Gingivostomatitis • Patients are infectious until all lesions are crusted over
• Presents with white vesicles on the tongue, buccal mucosa, (day 5–7 from rash onset) and should avoid contact
palate, and lips. with those who have never had chickenpox or who are
• Usually resolves by 2 weeks. immunocompromised.
• Treatment is acyclovir 200 mg five times a day for 5 days. • School or work need only be avoided if the rash is weeping
or cannot be covered.
Herpes Labialis • Treatment is with acyclovir 800 mg five times a day for
• Presents as grouped vesicles on the lips and perioral skin. 7 days (alternatively valaciclovir or famciclovir can be
• Usually resolves by 10 days. used). Ideally this should be initiated within 72 hours of
• Advise on appropriate ultraviolet (UV) protection as sun- the rash developing but consider starting it up to 1 week
light is a trigger. after onset of rash particularly in those at high risk of
• Use topical acyclovir (available over the counter) as soon complications.
as symptoms occur. • Consider referral or admission:
• If recurrent consider long-term acyclovir 200 to 400 mg • under ophthalmology if there is ophthalmic involve-
twice daily. ment with a red eye or visual complaints. If there is
ophthalmic shingles with no eye involvement then
Genital Herpes start treatment and review in 1 week;
• May cause dysuria mimicking features of urinary tract • under otorhinolaryngology (ears, nose, throat [ENT])
infection (UTI). if the Ramsay Hunt syndrome is present. This may
• Treatment is with acyclovir 500 mg five times a day for present with deep ear pain, rash in the ear canal or on
5 days. the auricle, and there may be associated facial droop,
• Both the patient and partner(s) need a full sexually trans- tinnitus, ipsilateral hearing loss, and vertigo;
mitted infection (STI) screen which may best be under- • under medics if the patient is immunocompromised
taken by the GUM clinic. and the rash is widespread.
Postherpetic Neuralgia (≤3 cm in size) with an overlying greyish thick crust.

• The pain of postherpetic neuralgia can be severe and debili- Removal of crust reveals a punched-out ulcer. It tends to
tating although it is usually self-limiting, lasting around heal slowly with scarring. Regional lymphadenopathy can
3 to 5 weeks. It can last for months or years. It occurs be seen.
in one in five people after shingles and is more common • It is more common in diabetic patients or elderly neglected
in the elderly, in females, in the immunocompromised, skin and more common in tropical climates.
and in those who have had a severe attack of shingles. • Complications include cellulitis, gangrene, bacteraemia,
• Offer the patient an information leaflet. staphylococcal scalded skin syndrome, and toxic shock
• Step 1 management is paracetamol, codeine, or NSAID. syndrome.
• Step 2 management is neuropathic analgesics, usually • Differential diagnosis includes:
amitriptyline or gabapentin. • ecthyma gangrenosum. Appearance similar but caused
• Topical treatments, including capsaicin or lidocaine plas- by pseudomonas species. Associated with significant
ters, may be effective if oral medications have failed. mortality;
• Consider referral for pain management in those with • orf/ecthyma contagiosum. Consider diagnosis if there is
refractory symptoms. relevant history of exposure to sheep or goats;
• pyoderma gangrenosum. Tends to have similar appear-
PATIENT SUPPORT ORGANIZATION
ance but usually a typical purplish appearance on edges
of the ulcer;
Herpes Viruses Association. 41 North Road, London, N7 • arterial ulcers. Especially if history of arterial disease.
9DP; helpline: 0845 123 2305; email: info@herpes.org.uk;
website: www.herpes.org.uk.
• Investigations should include blood test to rule out neu-
tropenia and diabetes, and wound swabs.
• Treatment depends on extent of lesions. If localized, anti-
bacterial washes such as Hibiscrub and topical mupirocin/
Bacterial Infection fusidic acid can be used. More extensive infections require
oral flucloxacillin or phenoxymethylpenicillin if group A
• It is worth remembering that skin infection can be either strep isolated. Treatment may be required for several weeks.
a primary disease process or a consequence of underlying If there are clinical concerns or doubt about diagnosis or
skin disease. management then refer to secondary care as surgical
debridement may be necessary for extensive infection.
Staphylococci and Streptococci
• Both these organisms account for some of the most Folliculitis
common encounters, often causing significant morbidity • Present with multiple small papules or pustules on an
and, potentially, mortality. erythematous base with or without hair follicle visible in
the centre. Sometimes it can present with deeper lesions
Impetigo as painful nodules that may result in scarring and per-
• Superficial infection of the upper layers of the skin most manent hair loss. Usually caused by S. aureus (very occa-
commonly encountered in children. sionally caused by gram-negative organisms).
• Usually highly contagious, the child is systemically well, • Common subtypes are based on distribution:
with classic honey-coloured crusting lesions on the face • Beard: folliculitis barbae
or digits and readily spreadable locally. Rarely presents • Lower legs: pseudofolliculitis (secondary to hair removal
as bullous lesions. or shaving)
• Usually caused by Staphylococcus aureus but occasionally • Trunk or buttocks: Malassezia or pseudomonas
Streptococcus pyogenes. folliculitis
• Treatment for localized lesions can be with topical mupi- • Scalp: folliculitis decalvans
rocin. Give oral antibiotics (such as flucloxacillin or eryth- • Eosinophilic folliculitis
romycin) for more widespread or severe cases. • Treatment:
• Provide education to patients about how it is transmitted • Avoid or treat the causative factor. Antibacterial washes
and how to prevent transmission. include chlorhexidine or Hibiscrub.
• Topical or oral antistaph antibiotic again depending on
Ecthyma disease extent; may require 4 to 6 weeks of treatment.
• Presentation is similar to impetigo but deeper layers of • If Malassezia infestation then consider ketoconazole
skin are involved leading to ulceration on lower legs, cream/shampoo regularly until clear then once or twice
buttocks, thighs, ankles, and feet. Patients tend to give weekly for maintenance.
a history of previous trauma such as insect bites. It is • Refer if:
caused by S. pyogenes. • diagnostic doubt;
• Lesions are painful and tend to start with vesicles or • there is poor response to treatment as patients can be
pustules on inflamed skin which deepens to form ulcers considered for isotretinoin or phototherapy.
Furuncles, Carbuncles, and Abscesses • Always treat underlying causes such as tinea or ulcers;
• A furuncle or a boil is a deeper infection of the hair follicle advise weight management.
with subcutaneous tissue involvement leading to abscess for- • Advise rest, elevation, analgesia.
mation. A carbuncle is when multiple inflamed hair follicles • Mark the area and observe for tracking.
coalesce to form a single lump which discharges pus. • Refer if the patient is systemically unwell or not respond-
• Treatment is with an oral antistaph antibiotic (e.g., flu- ing to oral treatment.
cloxacillin) if infection is caught early but it may require
surgical incision and drainage. Recurrent Cellulitis
• Cellulitis is recurrent if the patient suffers with two or
Erysipelas more episodes of cellulitis per year.
• Erysipelas is the name given to a more superficial form • Treatment is with prophylactic antibiotics such as:
of cellulitis that is usually seen following a breach in skin. • penicillin V 250 mg twice daily for 1 year then 250 mg
Common sites are the face and legs, and can present once daily;
bilaterally. • for penicillin-allergic patients clarithromycin 250 mg
• It is caused by group A streptococcus (S. pyogenes), occa- once daily or erythromycin 250 mg twice daily.
sionally by Staphylococcus. Other pathogens may be • Stop treatment after 2 years. If symptoms recur after 2
causative in immunocompromised patients. years restart prophylaxis and continue long term.
• Patients present with well-demarcated erythema, oedema
which is hot and tender to touch. Necrotizing Fasciitis
• The patient is usually systemically well unless septic. • This is a rare but an important surgical emergency and
• Treatment is with oral flucloxacillin 500 mg to 1 g four must be referred for immediate management. It is often
times daily or clarithromycin 500 mg twice daily. If there a polymicrobial infection involving the deep planes of
is facial involvement, consider co-amoxiclav. Treatment the skin including fascia. Although often numerous bac-
may need to be prolonged for 10 to 14 days. teria may be involved including anaerobes, S. pyogenes is
• Refer if the patient is systemically unwell or not respond- the most rapid, often being termed flesh eating and the
ing to oral treatment. patient is moribund within a couple of hours.
• Presentation:
Cellulitis • Level of pain is out of proportion to clinical presentation
• Cellulitis is commonly encountered in primary care. It • Greyish violaceous area with erythema
is usually caused by S. aureus but can be S. pyogenes and, • Crepitus
uncommonly, Haemophilus influenza. • Patient develops blisters that become necrotic
• Cellulitis involves the deep dermis and the patient is • Systemically unwell
systemically unwell; the area will be red, hot, painful, • Treatment involves pattern recognition in a septic dete-
and generally swollen with an ill-defined border and may riorating patient with emergency onward referral for
have an obvious point of entry for infection. debridement, intensive support, and antibiotics. It requires
• Risk factors include: multidisciplinary input.
• gravitational eczema;
• leg ulcers; Erythrasma
• tinea infection; • Presents with macerated areas that are often patchy and
• trauma; involving either the interdigital or intertriginous areas or
• lymphoedema; may also appear as reddish-brown plaques in the axillary
• high body mass index (BMI); areas. Caused by Corynebacterium minuttissimi.
• diabetes; and • Treatment is either topical clindamycin or topical
• immunocompromised patients. erythromycin.
It is highly unusual to encounter bilateral lower leg cellu-
litis; often chronic venous stasis with red legs will be wrongly Gram-Negative Bacilii
labelled as bilateral cellulitis, leading to incorrect treatment. • Most commonly Pseudomonas, usually aeruginosa. Infection
• Differential diagnosis: generally develops on skin which is already damaged, for
• Chronic venous insufficiency. Usually bilateral. No example in severe tinea infection. Skin becomes macerated,
associated tenderness or heat. Patient is systemically develops a foul small, and may appear with a greenish tinge
well. Follows a more chronic history. because of pigment production from the bacteria.
• Allergic contact dermatitis. Usually there is a history • Treatment is aimed at treating the underlying skin condition.
of contact with an allergen. Presents acutely with asso-
ciated blisters and itching. Lyme Disease
• Treatment, if infection is limited, is with oral flucloxacillin, • Lyme disease is caused by the spirochaete organism Bor-
otherwise the patient will require intravenous flucloxacillin relia, transmitted by the Ixodes tick. The type of Borrelia
or penicillin with the addition of clindamycin if severe. is dependent on which continent the tick is encountered.
• Ticks removed before 24 hours of attachment are unlikely • Topical management with terbinafine/imidazoles for 2
to be problematic; however, cutaneous lesions developing to 4 weeks can be considered for limited infection.
within a month of tick removal should be considered for • Systemic treatment with terbinafine/imidazoles for 2 to
evaluation of early Lyme disease. 4 weeks should be given if infection is extensive or the
• A history of being in woodland areas and of tick bites is rash is very inflamed.
useful. It is usually seen in spring and summer seasons. • If a kerion present refer dermatology:
• The most common presentation is that of erythema chroni- • Due to risk of scarring oral therapy is offered and this is
cum migrans, a distinctive rash which starts as an erythe- usually terbinafine although itraconazole can also be used.
matous papule expanding to an annular erythema with • For children griseofulvin is first line.
central clearing. It can be seen between 1 and 33 days • Also use antifungal shampoo for the first 2 weeks of
after exposure and fades over 3 to 4 weeks but only 70% therapy.
to 80% cases develop the typical rash. • Treat family members with antifungal shampoo for
• There may be a mild flulike illness and about two-thirds 2 weeks.
of untreated patients will develop symptoms, including:
• arthritis; Hands
• neuroborreliosis; • White discolouration in skin folds is often present.
• Bell palsy or other cranial nerve involvement; and • Always examine feet, hands, and nails as spread is common.
• cardiac borreliosis; peri/myocarditis leading to conduc-
tion defects. Feet
• Long-term sequelae include chronic arthritis and neuro- • Interdigital skin gets broken down and is a common cause
logical problems. of secondary cellulitis.
• Advise patients travelling to at-risk areas to: • Give topical treatment unless there is coexistent nail
• wear white (for easy spotting of ticks), long-sleeved involvement in which case systemic therapy is indicated.
clothes, long trousers tucked into socks, long boots; • Advise the patient to wear breathable footwear to reduce
• use pesticides/repellents; chance of recurrence.
• check whole body for ticks closely when home and
repeat the following day; Nails
• if a tick is seen use tweezers and firmly remove it in • Presents with subungual hyperkeratosis and yellow or
whole and disinfect the skin. white discolouration of nails.
• The presence of typical rash merits immediate treatment. • Offer patient information leaflet. Self-care is often appro-
• Serology for antibodies to Borrelia burgdorferi, although priate if the patient is not bothered by the nail or is
these may be negative in the first few weeks postinfection. concerned about side effects of treatment.
• Treatment is with doxycycline (100 mg two or three times • For nail clippings first cut the nail back then remove the
a day), amoxicillin, or erythromycin (less effective) for 2 friable debris to be sent for mycology. This will reduce
to 3 weeks. false negative results.
• Explain that cure rates are often 60% to 80%.
• Topical treatment is only for isolated superficial infections.
Fungal Infections Preparations such as tioconazole or Loceryl can be used.
Tinea • Systemic treatment is with terbinafine 250 mg once daily
• Consider it if there is an asymmetrical rash with scale and should be for 6 weeks for fingernails and 3 months
and a leading edge. for toenails. Alternatively, pulsed therapy of itraconazole
• Take skin scrapings from the advancing edge of the rash. can be used giving 400 mg once daily for 1 week every
• False negatives are not uncommon and samples should month. Two cycles are needed for fingernails and four
be repeated with treatment given if strong clinical suspi- cycles are required for toenails. Get baseline liver function
cion of tinea. tests (LFTs) before starting oral treatment.
• For mild tinea use topical agents but if widespread or • Inform the patient that even with successful treatment it
severe then oral therapies are indicated. can take months for the toenail to grow out and look
• Terbinafine is first line oral agent but alternatives are healthy again.
imidazole preparations. If using terbinafine then get base- • In children weight-adjusted terbinafine or griseofulvin
line liver function tests before initiation and repeat after can be used.
6 weeks of treatment. • If recurrent infection then long-term twice weekly topical
• Advise patients not to share towels or hats and to disinfect antifungal (terbinafine or imidazoles) can be used.
combs, scissors, pillows, and bed covers.
Candidiasis
Scalp Infection Oral
• Features are hair loss and scaling. • In healthy adults candidiasis may be the symptoms of
• Take skin scrapings and hair follicles for mycology. underlying immunodeficiency so think about risk factors
• Offer patient information leaflet. for diabetes, malnutrition, cancer, and HIV.
• Review known triggers if present such as poor denture • ongoing active infection despite two courses of
hygiene, inhaler technique, and diabetic control. treatment.
• First line treatment is miconazole gel or nystatin but if • Contact Health Protection Agency if there is an outbreak
extensive then offer oral fluconazole. in an institution such as a school or nursing home.
Skin Head Lice

• Advise the patient to avoid tight-fitting clothes and keep • Affected children can still attend school.
the area as dry as possible to avoid reinfection. • Treat all affected family members on the same day; options
• If obese give lifestyle counselling on weight loss. include dimeticone, malathion, or wet combing.
• For localized infection use topical imidazole creams or • Insecticide preparations should be used twice (repeated
terbinafine. If the rash is very inflamed or itchy consider again at 7 days) and detection combing should be
adding topical steroid to the antifungal cream. undertaken 3 days posttreatment to make sure lice are
• Creams should be used for 7 days and if no improvement eradicated.
then review the diagnosis and treatment.
Pubic Lice
Pityriasis Versicolour • If transmitted via sexual contact then refer to GUM clinic
• Most commonly presents in children and teenagers as for further STI screening and contact tracing. Alternatively
round or oval macules and papules that have a fine scale screen for chlamydia and other STIs, inform patient to
and may coalesce. Usually it is asymptomatic but is avoid sexual contacts until both have been treated, and
occasionally mildly itchy. Typically occurs on the chest, undertake contact tracing for sexual contacts over the
back, and upper arms. It is caused by yeasts of the genus past 3 months.
Malassezia. • Treat with topical malathion 0.5% or permethrin 5% to
• Explain that it is a self-limiting, noncontagious condition be used twice 7 days apart.
that is usually easily treated though skin change back to • If present in a child then, although nonsexual contact is
normal can take months. most common, consider the possibility of sexual abuse.
• Ketoconazole shampoo or selenium sulfide (contraindicated
in pregnancy) shampoos can be used if extensive. Skin Lesions and Malignancies
• Topical imidazoles can be applied if localized. Use for 2
weeks. • There are many different types of skin lesions and malig-
• If treatment fails then take skin scrapings to confirm nancies. Discussing them all would be beyond the remit
diagnosis and consider using systemic therapy such as of this book. Some of the common skin lesions and
itraconazole or fluconazole. malignancies that are seen in General Practice are described
• Consider referral if: in this section.
• diagnosis is uncertain and scrapings are negative; • Common terminologies used when describing skin lesions:
• there is extensive disease; • Macule: pigmented or nonpigmented flat lesion which
• immunocompromised, pregnant, or under 12 years is not palpable
old requiring systemic therapy. • Papule: raised pigmented or nonpigmented lesion which
is palpable and less than 1 cm in diameter
• Nodule: raised pigmented or nonpigmented lesion which
Parasitic Infections is palpable and greater than 1 cm in diameter
Scabies • Plaque: a plateau-like elevation above the skin surface
• Typically presents with widespread itching and a rash where the diameter is greater than the thickness of the
that may exhibit papules, vesicles, or nodules and lesion
linear burrows that classically affect the interdigital web
spaces and flexor aspects of wrists, elbows, groin, and
Benign Skin Lesions (Du Vivierk, 2013; Wolff
axillae.
• Treat the patient and all household and sexual contacts and Johnson, 2009)
with permethrin 5% Dermol cream (or malathion as Seborrhoeic Keratosis
alternative) immediately and after 1 week. • It is the most common of the benign epithelial lesions.
• Advise patients to machine wash clothes and bed linen Commonly seen after the age of 30. Can range from a
at 50°C. few scattered lesions to hundreds in some elderly patients.
• Treat itch and concurrent bacterial infection if present. Usually asymptomatic but they can be itchy or become
• If crusted scabies is present then consider underlying inflamed.
immunodeficiency. • Lesions range from small, barely elevated papules to plaques
• Inform patients that itch may last up to 4 weeks posttreatment. with a warty surface and a typical stuck-on appearance.
• Consider urea or crotamiton emollients to treat ongoing itch. They can be nonpigmented or pigmented.
• Consider referral if: • Patients usually present worried as they think they have
• crusted scabies or diagnosis is in doubt; a melanoma. They give a history of a longstanding
pigmented mole that has grown in size or become itchy in sun-exposed areas such as the face, nose, ears, cheeks,
or inflamed. The presence of multiple similar lesions on dorsum of hands, and forearms. Usually solitary, very
further general skin examination is often reassuring. occasionally they can be multiple.
• Differential diagnoses include: • Differential diagnoses include SCC (difficult to clinically
• solar lentigo: generally macular and lacks the warty differentiate) and hypertrophic solar keratosis.
appearance; • Refer for surgical excision or curettage and cautery depend-
• actinic keratosis: scaly rough appearance. Seen on sun- ing on site as they cannot be clinically distinguished from
exposed areas; SCCs. Multiple lesions may require systemic retinoids or
• pigmented basal cell carcinoma (BCC): appears more methotrexate.
pearlescent and nodular;
• melanoma: uneven pigmentation, irregular border.
Precancerous Skin Lesions
• Management is with reassurance. If inflamed curettage or
cryotherapy can be offered. Actinic (Solar) Keratosis
• Refer if there is diagnostic uncertainty. • Occur predominantly on chronically sun-exposed skin.
Rough, occasionally sore or itchy spots. They may be
Dermatofibroma/Histiocytoma single or multiple, dry, rough, adherent scaly yellowish-
• This is a common, probably reactive benign skin tumour brown lesions. They are more easily felt than seen due
more frequently seen on extremities. Often seen in adults, to the roughness. Surrounding skin can be normal or
it can be asymptomatic but unsightly. Occasionally it can erythematous.
be tender. • They are commonly seen on face, bald scalp, dorsum of
• Presents as a papule or nodule with a smooth surface. hands, and forearms of patients over the age of 50.
Firm in consistency and the characteristic feature is the • Differential diagnoses include:
dimple or pinch sign (the tumour indents on lateral pres- • Bowen disease: flat scaly lesions commonly seen on
sure with the fingers as it is adherent to overlying skin the shin;
but free from underlying structures). • seborrhoeic keratosis: warty stuck-on appearance;
• Reassure the patient. • superficial BCC: pearlescent appearance and telangiectasia.
• Refer if there is diagnostic uncertainty or if it is causing • If left untreated some can progress into squamous cell
troublesome symptoms. carcinoma.
• Advise prevention or further worsening by recommending
Sebaceous Hyperplasia a high factor UVB/UVA sunscreen (usually >30 factor).
• These are common benign lesions that are often mistaken • Treatment options:
for BCC. They are usually secondary to enlarged sebaceous • Cryotherapy: Usually freeze for 5 to 10 seconds after
glands around a single hair follicle often on the face. Seen the white halo has appeared.
in older people and patients who have had solid organ • 5% fluorouracil/Efudex: Usually apply twice daily
transplant and are on ciclosporin. for 4 weeks on and then 4 weeks off. If a vast area
• Present as small papules, 2 to 5 mm in diameter, flesh or is involved then best to divide into quarters or sec-
yellow coloured with telangiectasia and central punctum. tions and apply treatment in cycles. Warn the patient
Usually there are multiple lesions in one area. regarding erythema and erosions. If the reaction is
• Differential diagnosis is a small BCC which is usually too intense it can be applied once daily or 5 days
solitary. per week.
• Management is with reassurance. Excision or removal by • 5% imiquimod/Aldara: Highly effective but can cause
light electrocautery under local anaesthetic can considered intense reaction which may discourage patients. Treat-
if there is diagnostic uncertainty. ment regime is 3 days per week for 4 weeks.
• Zyclara (3.75% imiquimod): Can be used for facial
Keratoacanthoma lesions. Treatment regime is to apply for 2 weeks then
• These are rapidly growing benign tumours which tend discontinue for 2 weeks.
to simulate SCC but usually self-resolve in 4 to 6 months. • 3% diclofenac/Solaraze Gel: Used twice daily for 3
They tend to rapidly grow for 3 months and reach up months. Better tolerated but not as effective as imiqui-
to 3 cm in diameter before regressing spontaneously mod or fluorouracil.
over the next 3 months. They are most commonly • Picato gel: Treatment is for 3 days which allows for
seen in older age groups, patients who have had chronic better compliance.
sun exposure, and also in transplant recipients on • Other treatments not commonly used are laser treat-
immunosuppressants. ment, photodynamic therapy, and facial peels.
• Presents as a small spot that rapidly grows in size causing • If patients are finding the reactions secondary to topical
alarm. They are erythematous or flesh-coloured, well- treatment too intense then advise to use for 5 days per
defined nodules with a central keratin-filled crater. The week and use topical steroids on the other 2 days to help
appearance mimics an erupting volcano. Commonly seen skin recover.
• Refer if not responding to treatment or patient not tol-

erating treatment. Basal Cell Carcinoma (Rodent Ulcer)
• This is the most common type of skin cancer. It is usually
Bowen Disease (Squamous Cell Carcinoma secondary to chronic sun exposure although genetic muta-
In Situ) (Ashton & Leppard, 2009) tions are a known cause. It is locally invasive, erodes
• This is described as an intraepidermal carcinoma of the gradually through the skin, but is rarely known to metas-
skin that can occasionally progress to squamous cell car- tasize, hence the name rodent ulcer
cinoma. Usually asymptomatic, patients usually present • Key features:
with a persistent solitary rough patch. • Pearly margins
• It is a well-defined solitary plaque which may be slightly • Telangiectasia
raised, scaly, and erythematous. Similar but non scaly lesions • Usually firm but can be cystic
on the glans or vulva are called erythroplasia. HPV-induced • Tends to bleed when excoriated
Bowenoid changes in the vulval or penile area are called • Commonly seen in middle-aged or elderly fair-skinned
Bowenoid papulosis. It carries premalignant potential and individuals with history of chronic sun exposure (e.g.,
hence should be treated. Common sites are sun-exposed gardeners, sailors, people who have worked abroad in
areas such as the face, dorsum of hands, forearms, and tropical areas)
shins. • Commonly seen on the face but can be present any-
• Management options include: where on the body
• cryotherapy; • There are various subtypes (Table 22.2):
• 5% fluorouracil/Efudex twice daily for 4 weeks; • Superficial BCC: most common subtype. Appear as
• Imiquimod/Aldara three times per week for 12 weeks; thin plaques with a fine border and telangiectasia. Best
• referral for surgical treatment such as curettage and seen with use of a hand lens.
cautery, excision. • Nodular BCC: can present as well-defined nodules or
• Treat lesions on shins with care as can lead to leg ulcer- papules, classically described as pearly (i.e., skin-
ation due to poor healing. coloured smooth surface with telangiectasia).
• Ulcerated BCC: some nodular BCCs can progress to
Cutaneous Horn form a central ulcer covered with crust and rolled out
• Horny outgrowths from the skin. These are of importance borders and telangiectasia.
as at the base of these lesions there could be an underly- • Pigmented BCC: some BCCs can be pigmented and
ing actinic keratosis, Bowen disease, or SCC. mimic a nodular or superficial spreading melanoma.
• Refer to Secondary Care. • Morphoeic or sclerosing BCC: difficult to diagnose but
fortunately not very common. Presents as a macular
Atypical/Dysplastic Naevus scarlike area. Often ill defined, hypopigmented, and
• Although not malignant it is considered as a precursor sclerosed appearance. Can progress to ulcerated or
to melanoma. It occurs due to dysfunctional proliferation nodular BCC. More aggressive in nature.
of atypical melanocytes. They can arise from preexisting • Refer for excision on a routine basis.
moles or de novo.
• Sometimes patients present with a mole that looks dif- Squamous Cell Carcinoma
ferent from their other moles or has been gradually evolv- • SCC is a malignant metastasizing tumour that arises from
ing. However, sometimes these are picked up coincidentally the keratinocytes within the epidermis. It can arise de novo
when the patient presents for other pathology (e.g., when or evolve from preexisting precancerous skin conditions
examining chest for a cough). such as Bowen disease or an actinic keratosis. It can also
• Usually differentiated from benign naevi by their large, be an underlying pathology in chronic nonhealing ulcers.
variegate appearance with irregular borders and tendency • Main risk factor is chronic sun exposure; therefore it is most
to stand out among other moles. commonly seen in outdoor workers and people who live
• Differential diagnosis is malignant melanoma. They are in or travel to tropical countries. It is also more common
difficult to distinguish. in the immunosuppressed. Other risks are phototherapy
• Refer for excision due to malignant potential. and sunbeds, exposure to polycyclic hydrocarbons (tar,
mineral oils), arsenic ingestion, and human papillomavirus.
Malignant Skin Lesions • Patients usually present with a solitary keratotic papule/
nodule that has either persisted for months or has been
gradually growing in size. Usually there are no associated
GUIDELINE
symptoms such as pain, bleeding, or itching. Appearance
National Institute for Health and Care Excellence. (2015). can be of a scaly plaque to indurated plaque or nodule.
Suspected cancer: Recognition and referral. NICE clinical It can have an ulcerated or crusted surface. Commonly
guideline 12. Retrieved from www.nice.org.uk.
seen in sun-exposed areas such as the face, forearms, bald
scalp, ears, and lower legs.
TABLE
22.2 BCC Subtypes
BCC Subtype Presentation Differential Diagnosis Management

Superficial Thin plaques with a fine Fungal infection: usually very itchy and Cryotherapy
border, telangiectasia does not bleed Topical imiquimod or Aldara
Often bleeds on minimal Bowen disease: scaly patch Refer to dermatology
excoriation Eczema: especially if multiple; usually
there is a history of eczema and
good response to topical steroids
Nodular BCC Pearly appearance, nodule Dermatofibroma (firm, pinch sign Usually surgical
or papule with positive) Refer to dermatology
telangiectasia If surgery is not an option then
radiotherapy or hedgehog
inhibitors are considered
Ulcerated Crust-covered ulcer with a Squamous cell carcinoma Usually surgical
rolled border, Primary chancre of syphilis (history Refer to dermatology
telangiectasia would help) If surgery is not an option then
radiotherapy or hedgehog
inhibitors are considered
Pigmented Pigmented well-defined Seborrhoeic keratosis: typical stuck-on Surgical
nodules, can be warty appearance Refer to dermatology
ulcerated Malignant melanoma: usually without
preexisting mole
Morphoeic Appears as sclerosed Scar tissue Surgical: most require Moh
yellowish ill-defined Scleroderma: difficult to differentiate surgery, which is highly
plaques. More aggressive and sometimes diagnostic biopsy is specialized
only option
BCC, Basal cell carcinoma.
• A history of a nodule developing on a background of a called melanocytes. Two-thirds arise de novo and only
scaly or plaquelike area should also raise suspicion of SCC. one-third arise from preexisting moles.
• Differential diagnoses: • High-risk factors:
• Bowen disease: Usually scaly plaque with no induration. • Fair-skinned people with red hair (i.e., skin type 1)
• Keratoacanthoma: Short history, rapid growth, and tends • History of severe sun burns in younger years
to self-resolve in 4 to 6 months. However, clinically • People with multiple moles (>50)
difficult to differentiate from SCC, hence best to treat • People with dysplastic naevi
as SCC until proven otherwise. • Use of sunbeds
• BCC: Especially the early stages and the well-differentiated • Family history or personal history of melanoma.
ones; however, BCCs are pearlier in appearance with • There are four main subtypes:
telangiectasia and tend to be less indurated. 1. Lentigo maligna melanoma (LMM): Malignant cells are
• Chondrodermatitis nodularis helicis (CNH): On the free limited to epidermis. The growth of this tumour is usually
border of helix of ear. Usually develop spontaneously, in the horizontal plane rather than the vertical. Presenta-
grow rapidly, tend to be less than 1 cm in size, and tion is usually as a 1- to 3-cm macular pigmented lesion
are tender, unlike SCCs. on sun-exposed areas, usually in an elderly patient. Can
• Refer for excision which has to be complete (both periph- evolve into superficial spreading or nodular melanoma.
eral and deep margins). This should be along a 2-week 2. Superficial spreading melanoma (SSM): Malignant cells
suspected cancer pathway. usually lie along the dermoepidermal junction. Again
• Patients with recurrent SCCs are offered systemic treat- the growth is more in the horizontal plane. Presenta-
ment such as Acitretin to reduce the incidence of further tion is usually of an enlarging macular irregularly pig-
lesions developing. mented lesion with irregular borders. Surrounding skin
may show inflammation.
Malignant Melanoma (National Institute for 3. Nodular melanoma (NM): The growth is mostly in the
Health and Care Excellence, 2015) vertical plane rather than the horizontal. Presentation
• Malignant melanoma (MM) is one of the most malignant is usually of a darkly pigmented dome-shaped nodule.
neoplasms of the skin. They are tumours that arise from The surface tends to break down and bleed or ooze
the pigmented cells within the dermoepidermal junction and crust over. Sometimes lesions are not darkly
• BOX 22.1 American ABCDE List for • Treat infections with antibiotics according to swab
Assessing Moles sensitivities.
• Treat itch with topical steroids moderately potent (e.g.,
This is a good and simple way to assess a mole for superficial Eumovate) to potent (e.g. Betnovate) for around 1 week
spreading melanoma:
A: Asymmetry in shape; one half is different from the other
at a time to avoid skin atrophy.
B: Borders: irregular borders • Consider compression stockings for resistant cases. If
C: Colour: more than one shade of pigment to the lesion compression stockings are being considered then check
D: Diameter >6 mm or also ugly duckling appearance, meaning ankle-brachial pressure index (ABPI) first and use below-
it stands out as an odd-looking mole among others knee compression stockings if there is no evidence of
E: Enlargement: history of increase in size; Expert—seek expert
advice
arterial insufficiency (class 2 are suitable for most people
or class 1 if these are not tolerated):
• ABPI less than 0.3 or greater than 1.3: avoid compres-
sion stockings
pigmented and can be flesh coloured or erythematous; • ABPI greater than 0.3 but less than 0.8: use only class
these are termed amelanotic melanoma. 1 (mild) stockings
4. Acral melanoma: These tend to occur on palms, soles, • ABPI greater than 0.8 but less than 1.3: use any up
and nail beds. Any nontraumatic de novo pigmentation to class 3 stockings
or change in mole in these areas should be referred for • Consider the differential of allergic contact dermatitis in
further assessment to rule out malignant potential. spreading eczema.
• Any patient that presents with a change (shape, size, colour) • Consider referral:
or itch and/or bleeding in a longstanding mole or history of • for patch testing if suspected allergic contact dermatitis;
developing new moles after the age of 30 should be referred • if inability to control condition despite full primary
to rule out a malignant melanoma (Box 22.1). care intervention;
• Referral should be urgent under the suspicion of cancer • if evidence of fibrosis or evidence of ulceration;
pathway for assessment and excision. The primary exci- • if ABPI is less than 0.8 then refer to vascular.
sion is usually carried out with a 2-mm margin and once
histologically confirmed wide local excision is carried out
ranging from 5 mm to 2 cm based on the type of melanoma. PATIENT SUPPORT ORGANIZATION
National Eczema Society. 11 Murray Street, London, NW1
9RE; helpline: 0800 089 1122; website: www.eczema.org.
Venous/Varicose Eczema
GUIDELINES
National Institute for Health and Care Excellence. (2012). Leg Ulcers
Venous eczema and lipodermatosclerosis. NICE clinical
knowledge summaries. Retrieved from https://cks.nice.org.uk.
Raju, S., Hollis, K., & Neglen, P. (2007). Use of GUIDELINE
compression stockings in chronic venous disease: Patient Scottish Intercollegiate Guidelines Network. (2010).
compliance and efficacy. Annals of Vascular Surgery, 21(6), Management of chronic venous leg ulcers. SIGN guideline
790–795. 120. Retrieved from www.sign.ac.uk.
• This ranges from haemosiderin deposits to active eczema

then to lipodermatosclerosis, atrophe blanche, and the • Of leg ulcers, 70% are venous, 10% are arterial, and the
increasing chance of active ulceration. rest include neuropathic, BCC, pressure ulcers, and
• Give patients general advice, including: autoimmune conditions.
• avoid prolonged sitting with legs down or standing; • Involve nursing colleagues with experience in wound
• stay physically active; management early in the presentation.
• give advice on avoidance of skin injury; • It is important to remember to treat associated conditions
• spend 1 hour a day in bed with legs raised; and such as pain, eczema, oedema and to inform patients
• use regular emollients. with regard to signs of infection.
• Topical steroids can be used for a flare of eczema; in lipo- • Pain management. Remember to take a history to deter-
dermatosclerosis very potent steroids may be required. mine if the cause is arterial, neuropathic, or cellulitic.
For persistent venous eczema consider a trial of potent • Give healthy lifestyle advice as this promotes healing and
topical steroid ointment under medicated bandages reduces risk of recurrence especially smoking cessation,
such as zip socks which are changed once or twice weight control, diet, and alcohol advice.
a week. • Ensure the ulcer is cleaned and dressed at least weekly.
General Advice (e.g., pyoderma gangrenosum, vasculitis, contact derma-

titis, BCC).
• Give advice on exercise, as mobility keeps the calf muscle • Consider referral if there is no improvement after 3 months.
pump active and helps reduce oedema.
• Tight-fitting footwear is to be avoided and patients need
to remain vigilant about avoiding trauma to the legs. PATIENT SUPPORT ORGANIZATION
• Advise patients to elevate legs when immobile; eleva- The Lindsay Leg Club Foundation. Ipswich, PO Box 689, IP1
tion should be to the level of the heart if possible so 9BN; tel. 01473 749565; website: www.legclub.org.
this means advising patients that they should put
pillows under their feet when in bed and not rely on
footstools.
Hyperhidrosis
Management
GUIDELINES
• Use an emollient frequently.
• Moderately potent topical steroid can be used to improve Hyperhidrosis. NICE clinical knowledge summaries. Retrieved
surrounding skin venous dermatitis. from https://cks.nice.org.uk.
• Antibiotics are only indicated if there is clinical suspicion Primary Care Dermatology Society. (2014). Hyperhidrosis.
of infection and should not be initiated on swab results Retrieved from www.pcds.org.uk/clinical-guidance/
without clinical suspicion. hyperhidrosis.
• Compression stockings are effective in reducing recurrent
ulcers and chronic lower leg swelling but check ABPI via
Doppler. Only use compression therapy if ABPI is above Primary Hyperhidrosis
0.8, it is not a diabetic ulcer or neuropathy, there is no
phlebitis, no deep vein thrombosis (DVT), and no cel- • Affects scalp, face, axillae, hands, or feet.
lulitis. Discontinue compression therapy if features of • No apparent cause.
arterial insufficiency or infection occur. • Has lasted at least 6 months.
• Compression bandaging should be applied as follows: • Has at least two of the following characteristics:
• For people who are immobile, four- or three-layer 1. Bilateral
bandaging is more suitable. 2. Impairs activity of daily living
• For people who are mobile, two-layer bandaging is 3. At least one episode per week
more practical. 4. Does not occur during sleep
• For most patients below-knee compression stockings 5. Onset before 25 years of age
are most suitable; remember to prescribe two at a time 6. Other family members affected
and for patients to get a new prescription every 4
months as they lose elasticity. Generalized Hyperhidrosis
• To aid ulcer healing pentoxifylline 400 mg three times
daily can be prescribed for up to 6 months with or without • Generalized hyperhidrosis affects the entire skin surface
compression bandages as this improves microcirculation, area and is usually secondary to other medical conditions
although check local guidelines as this is usually initiated or induced by drugs.
in secondary care. • Many cases are idiopathic.
• If an ulcer is failing to heal then screen for iron deficiency • Common drugs include antidepressants, alcohol, substance
and diabetes. Also consider the differential diagnosis which abuse.
can include basal cell cancer. • Clinical features include asymmetrical sweating, general-
• It is common for ulcers to recur, so support stockings are ized sweating, sweating during sleep, clinical features of
needed long term. systemic illness.
• Medical conditions include but not limited to anxiety,
Referral pregnancy, infections, malignancy, menopause, hyperthy-
roidism, and hyperpituitarism.
• Refer routinely to the vascular surgeons if the ABPI is
below 0.8 or if there are significant varicosities. Investigations
• Refer urgently to vascular if the ABPI is below 0.5 or if In obvious primary hyperhidrosis these are not indicated. If
ischaemic changes occur due to compression stockings. generalized hyperhidrosis is a differential then further investiga-
• Refer to dermatology if there is no improvement despite tion is required and often includes FBC, urea and electrolytes
primary care therapy or if there is deterioration, if malig- (U&E), LFT, ESR/C-reactive protein (CRP), fasting blood
nancy is suspected, or if there is an atypical pattern of glucose (FBG), thyroid function test (TFT)—if clinically indi-
ulceration which may signify an underlying disease process cated then chest x-ray (CXR) (neoplasm) and HIV serology.
first choice is lymecycline. If this is not controlling it

Management then the dose can be doubled to 1 tablet twice daily.
• Provide patient information leaflet. While off license there is no evidence of harm by using
• Advice: avoid caffeine and spicy foods, avoid tight-fitting this approach. Alternative antibiotics include doxycycline,
clothes. metronidazole, or macrolides.
• Use 20% aluminum chloride hexahydrate roll-on at • If no improvement after 3 months or in severe disease,
night. Use this at night on dry skin and wash it off in then a combination approach of clindamycin 300 mg
the morning until symptoms controlled. Do not apply twice daily and rifampicin 300 mg twice daily should be
within 12 hours of shaving. Once control achieved then used for 3 months. LFTs should be checked before start-
use weekly. ing and soon after initiation as rifampicin can cause
• If feet are most affected then aluminum dusting powder. hepatotoxicity.
• For skin irritation caused by aluminum 1% hydrocortisone • Combined oral contraceptive pill (COCP) may be helpful
ointment can be used for up to 2 weeks. in women without contraindications.
• Pro-Banthine can be used as second line medication. • Antiandrogens: Dianette can be effective; however, it is
• If anxiety is an issue then offer cognitive behavioural contraindicated in the obese and patients at risk from
therapy (CBT) as alternative to propranolol or antidepres- clots. If eligible, and symptoms are worse between periods,
sants which can worsen sweating. then consider giving with extra 50 to 100 mg cyproterone
• Review in 1 to 2 months to reassess. acetate on days 5 to 14 of the cycle.
• If not controlled then consider referral to dermatologist. • Spironolactone 100 to 200 mg may be effective.
• Refer:
Management of Generalized Hyperhidrosis • to dermatology if medical therapies are ineffective or
This is targeted at finding the cause and treating it. If aetiol- if widespread scarring disease is present;
ogy remains unclear and ongoing sweating occurs then con- • to surgery if large abscesses need incised and drained.
sider referral to dermatology.
PATIENT SUPPORT ORGANIZATION The Hidradenitis Suppurativa Trust. Cliffe House, Anthonys
Hyperhidrosis UK. www.hyperhidrosisuk.org. Way, Rochester, ME2 4DY; email: enquiries@hstrust.org;
website: www.hstrust.org.
Hidradenitis Suppurativa
Urticaria
GUIDELINES
• Urticaria can be divided into the following subtypes:
Primary Care Dermatology Society. (2014). Hidradenitis • Idiopathic: most common
suppurativa. Clinical guidance, updated 2017. Retrieved from
www.pcds.org.uk/hidradenitis_suppurativa. • Immunologic: IgE related (food allergy, infections,
reactions to external agents on body), C1 esterase
inhibitor deficiency, and vasculitic
• Non-immunological: physical stimuli (heat, cold, sweat,
• Hidradenitis effects about 1% of the population and ranges water, pressure, solar rays, dermographism) or medica-
from mild papules, pustules, and nodules to fluctuant tion related
abscesses, draining sinuses, and severe scarring. • The outlook is that most patients with chronic urticaria
• It is a clinical diagnosis but consider performing FBC get better by 1 year.
(for possible coexisting anaemia) and swabs of lesions • Investigations. For acute idiopathic urticaria (lasting <6
prior to antibiotic therapy. weeks) no investigations are usually indicated. In chronic
• Start treatment early to reduce the chance of future scarring. or recurrent cases where a secondary cause is suspected
• Advise weight loss in obese patients, which is essential, the following can be considered:
and smoking cessation. • FBC, ESR/CRP, Thyroid Peroxidase antibodies (TPO)
• Advise the patient on loose-fitting clothes, antiperspirants, • Helicobacter pylori testing
and good hygiene. • Exclusion of suspected medication (e.g., aspirin,
• Wash with Dermol or chlorhexidine to reduce future NSAIDs, ACE inhibitors, opioids, penicillin)
infections. • Dietary changes
• For acute flares then a 2-week course of antibiotics based • Hot/cold challenge
on local protocol and swab microbiology results. • Check for dermographism
• For chronic disease then at least 3 months of oral antibiot- • Investigate for autoimmune condition if associated
ics is needed as these have an antiinflammatory effect and joint pain or malaise
• IgE tests are rarely necessary and only to confirm spe- • trichotillosis (compulsive hair pulling): different length
cific triggers. hairs;
• Differentiate ordinary urticaria from physical and contact • traction alopecia: from hair styling.
urticaria as the management in the last two is about avoid- • Causes of scarring alopecia include:
ing the stimulus. • infection (bacterial or fungal);
• Give lifestyle advice including avoidance of overheating • lichen planus;
such as taking hot baths, alcohol, spicy foods, and • SLE.
caffeine. • Investigations:
• First line management is a nonsedating antihistamine • FBC, ferritin, and TFT (consider syphilis if diffuse
(initially cetirizine or loratadine but can be increased to alopecia)
fexofenadine if there is no improvement). • Enquire about childbirth, recent surgery, causative
• If urticarial is severe add a short course of prednisolone medications
(30 mg for 3 days). • Fungal culture
• If an antihistamine is not effective then the dose can be • Consider serology for SLE
increased to four times the licensed dose (so twice up to
four times daily) unless the patient has a history of long Treatment Alopecia Areata
QT syndrome or severe renal or liver problems.
• If this is not effective then try an alternative nonsedating • Provide patient information leaflet and consider counsel-
antihistamine and/or add a sedating antihistamine such ling and psychologic support.
as chlorphenamine or hydroxyzine to help sleep at night. • Advise sunblock on bald patches.
• Try soothing agents such as 1% menthol in aqueous cream • Nonextensive (<50% hair affected): no treatment is often
or calamine. indicated as spontaneous remission occurs in up to 80%
• Refer: of patients.
• urgently to dermatology if suspected urticarial vasculitis • Extensive alopecia (>50% hair affected) areata: wigs.
(this is tender urticaria, associated joint pains, bruising, • Trial of potent or very potent steroids for 3 months (in
or static weals that are present >24 hours or contact nonpregnant patients who do not want referral). If refer-
urticaria); ring consider a trial of this treatment while awaiting review.
• if persistent urticaria is unresponsive to three different • Intralesional steroid injection such as triamcinolone may
antihistamines each for 4 to 6 weeks; be tried but warn about the possibility of atrophy.
• in rare case to immunologist for radioallergosorbent • Refer:
test (RAST) or skin prick testing if thought due to • if there is more than 50% hair loss or no regrowth;
food, drug, or latex allergy; • patient preference.
• in rare case to dermatology for patch testing if suspected
contact urticarial.
Alopecia Alopecia Online. www.alopeciaonline.org.uk.
GUIDELINES
British Association of Dermatologists. (2012). Guidelines for References
the management of alopecia areata. www.bad.org.uk/
alopecia. Ashton, R., & Leppard, B. (2009). Differential diagnosis in dermatology
(3rd ed., p. 186). Boca Raton, FL: CRC Press.
Du Vivier, A. (2013). Atlas of clinical dermatology (4th ed., pp. 150–152).
Philadelphia, PA: Saunders.
• Alopecia may be either: National Institute for Health and Care Excellence (2015). Melanoma
1. nonscarring (no inflammation, follicular openings overview. London: NICE.
present, atrophy absent); or National Institute of Health and Care Excellence. (2012). Plaque Pso-
2. scarring (inflammation usually present, no follicular riasis: Assessment and Management. Clinical Guideline 153. Available:
openings, atrophy present). www.nice.org.uk.
• Causes of nonscarring alopecia include: Wolff, K., & Johnson, R. A. (2009). Fitzpatrick’s color atlas and syn-
• alopecia areata: scalp looks normal, exclamation hairs opsis of clinical dermatology (6th ed., pp. 215–216). New York:
at edges, pull test positive; McGraw-Hill.
• tinea capitis: subtle scale may be seen;
23
Allergic Problems
AZIZ SHEIKH
C H A P T E R CO N T E N T S • Adverse reactions to food may result from allergy (hyper-

Food Allergies sensitivity)—either IgE or non-IgE mediated—or intoler-
Diagnosis ance (reactions that are not clearly immunologically
Management mediated) (National Institute for Health and Care Excel-
Useful Contacts lence [NICE], 2011; Sampson, 2004).
For Patients • The severity of allergic reactions is highly variable, with
For Professionals symptoms ranging from mild cutaneous symptoms (e.g.,
Anaphylaxis exacerbation of atopic eczema/dermatitis and urticaria)
Diagnosis predominantly experienced in those with non-IgE-medi-
Management ated food allergy to systemic life-threatening anaphylaxis
Useful Contacts that may be seen in those with IgE-mediated food allergy.
For Patients • Patients will often use the term allergy to refer to any of
For Professionals a number of food-related adverse reactions; double-blind,
References placebo-controlled studies, however, show that only a
minority of these reactions have an allergic basis (Rona
et al., 2007; Sampson, 2005).
• In addition to the immediate effects of allergic reactions,
food allergies can have a significant impact on patients’
Food Allergies everyday lives (Primeau et al., 2000). Activities such as
food shopping, eating outside the home, and travelling
abroad can become challenging. Accurate diagnosis and
GUIDELINES/SYSTEMATIC REVIEWS
good long-term management are therefore crucial in main-
National Institute for Health and Care Excellence. (2011). taining quality of life and minimizing anxiety (Munoz-
Food allergy in children and young people. Diagnosis and
Furlong, 2003).
assessment of food allergy in children and young people
in primary care and community settings. NICE clinical • Evidence-based guidelines for the diagnosis and manage-
guideline 116. ment of food allergies are available (Boyce et al., 2010;
Boyce, J. A., Assa’ad, A., Burks, A. W., et al. (2010). NICE, 2011). Note that data from randomized controlled
Guidelines for the diagnosis and management of food allergy trials are limited in the area of food allergies (de Silva
in the United States: Summary of the NIAID-Sponsored
et al., 2014); these guidelines are therefore based on a
Expert Panel Report. Journal of Allergy and Clinical
Immunology, 126, 1105–1118. systematic review of the best available evidence.
de Silva, D., Geromi, M., Panesar, S. S., et al. with the
EAACI Food Allergy and Anaphylaxis Group. (2014). Acute Diagnosis
and long-term management of food allergy: Systematic • Differentiating food allergy from intolerance is important
review. Allergy, 69(2), 159–167.
because the former will typically require meticulous avoid-
de Silva, D., Geromi, M., Halken, S., et al. with the EAACI
Food Allergy and Anaphylaxis Group. (2014). Primary ance of the food(s) in question; continued exposure to the
prevention of food allergy in children and adults: Systematic triggering food(s) in those with IgE-mediated food allergy
review. Allergy, 69, 581–589. increases the risks of major systemic allergic reactions such
as anaphylaxis (Sheikh & Walker, 2002; Wood, 1999).
• Attempt to differentiate food allergy from intolerance by
the following features of the history and examination:
• The UK incidence of severe food allergic reactions leading • Family history: Food allergy usually occurs in those
to hospitalization has increased in recent years (Gupta, with a personal and/or family history of allergic dis-
Sheikh, Strachan, & Anderson, 2007). orders (e.g., atopic eczema, hay fever, asthma).
403
TABLE Foods Commonly Responsible for Triggering reactions from the patient/carer and, if necessary, refer
23.1 Allergic Reactions to the list of the most common trigger foods to deduce
likely culprits (see Table 23.1). Confirm clinical suspicion
Children Adults
with objective allergy test if IgE-mediated disease is sus-
Milk Fish and seafood pected (skin-prick test and/or serum-specific IgE) or a trial
Egg white Tree nuts of dietary exclusion if non-IgE-mediated food allergy is
suspected.
Peanuts Peanuts • Refer to an allergist if provision for suitable testing is
Wheat Additives unavailable, in the event of diagnostic uncertainty, and/
Soya beans Fruits or if the patient has experienced a life-threatening reaction
(Durham & Church, 2006).
• Recommend the avoidance of food(s) found to trigger
symptoms. Advise careful checking of food ingredients
• Type of food: Although almost any protein-based food (e.g., by reading food labels, asking waiting staff and
may provoke allergic symptoms in sensitized individu- caterers when eating outside the home).
als, most reactions occur in relation to exposure to a • Encourage patients to be proactive in seeking relevant
small group of foods (Table 23.1) (Teuber, Beyer, information. For example, organizations such as Allergy
Comstock, & Wallowitz, 2006). UK and the Anaphylaxis Campaign (see Useful Contacts),
• Speed of onset: Symptoms soon after food intake (usually as well as many larger food manufacturers and catering
<1 hour and often within minutes) are suggestive of chains, now publish allergen information online.
IgE-mediated allergy. • Recommend that patients with pollen-food allergy
• Effect of re-exposure: Re-exposure to the same food(s) syndrome avoid the offending fruits and/or vegeta-
will tend to produce similar reactions in those with bles in their raw form; in most cases, the allergen is
food allergy; the picture is often much more variable removed by peeling or destroyed by heating and the
in those with intolerance. peeled/cooked fruits and/or vegetables can then be
• Pollen-food allergy syndrome (known until recently as safely consumed.
oral allergy syndrome) is an IgE-mediated hypersen- • Enlist the help of a dietician to ensure that patients fully
sitivity to raw fruits, root vegetables, and some nuts. understand about the foods that they need to avoid,
It is most likely to occur in patients with tree pollen to help them manage avoidance when eating in and
allergy (i.e., those with spring hay fever). Contact outside the home, and to ensure their diet is nutritionally
urticaria and/or angioedema of the lips and oropharynx adequate.
occur through a cross-reactivity between specific epi- • Issue self-injectable adrenaline to those with a history of
topes in pollens and fresh fruit/vegetables, manifesting anaphylaxis and recommend a medical identity bracelet/
as an itchy oropharynx and swelling of the lips and necklace.
tongue. More severe reactions may occur, but are • Refer patients with severe food intolerance(s) to a gas-
unusual (Perry, Scurlock, & Jones, 2006). troenterologist. Any long-term exclusion diet should be
• The clinical picture: Food allergy will typically trigger supervised by a dietician (Grimshaw, 2006).
symptoms indicative of inflammation in one or more • Review patient periodically to ensure long-term manage-
organ systems: These include features of angioedema ment is effective.
(particularly of the lips and tongue), urticaria, con-
junctivitis, rhinitis, bronchospasm, gastrointestinal Reintroducing Foods After a Period of Avoidance
oedema (cramps, vomiting, and diarrhoea), and ana- • Children with allergy to milk and eggs will commonly
phylaxis (Perry et al., 2006). Food-related symptoms develop tolerance to these foods as they grow older. In
of tiredness, joint and muscle pains, sleep disturbance, those with a history of reactions that are not considered
and emotional upset are all more suggestive of a diag- to be life threatening, the careful reintroduction of these
nosis of food intolerance. foods at a later date may be appropriate.
• Consider reintroducing:
Management • milk at the age of 3 years (by which time 85% of
Symptomatic Treatment children with a history of milk allergy will be tolerant);
• Treat symptomatically with H1-antihistamines; if life- and
threatening features are present (respiratory difficulty or • eggs at the age of 6 to 10 years (by which time 55%–80%
symptoms suggestive of hypotension) then treat as for of children with a history of egg allergy will be toler-
anaphylaxis (see below). ant) (Teuber et al., 2006).
• Persistent symptoms. Refer to specialist services.
Further Management Note: Peanut and fish/seafood allergy is, in the majority
• Attempt to unequivocally identify the food(s) respon- of individuals, lifelong (Teuber et al., 2006), and attempts
sible for triggering reactions. Take a detailed history of at reintroduction are not normally recommended although
CHAPTER 23 Allergic Problems 405
research in reintroducing peanut proteins as part of a care- Anaphylaxis

fully supervised programme is ongoing.
GUIDELINES
Pregnancy, Lactation and Prevention of Allergy
• Pregnant and breastfeeding women do not need to change Joint Task Force on Practice Parameters; American Academy
their diet or take supplements in an attempt to prevent of Allergy, Asthma and Immunology; American College of
Allergy, Asthma and Immunology; and Joint Council of
allergies in infants. Allergy, Asthma, and Immunology. (2005). The diagnosis and
• Although those with a family history of allergic disorders management of anaphylaxis: An updated practice parameter.
have been advised to avoid peanuts during pregnancy, Journal of Allergy and Clinical Immunology, 115, S483–S523.
lactation, weaning, and until the age of at least 3 years Soar, J., Pumphrey, R., Cant, A., et al. (2008). Working
(Fiocchi, Assa’ad, & Bahna, 2006; Friedman & Zieger, Group of the Resuscitation Council (UK) 2008. Emergency
treatment of anaphylactic reactions—guidelines for healthcare
2005), there is concern that this may actually be increas- providers. Resuscitation, 77, 157–169.
ing the risk of peanut allergy through preventing the Simons, F. E. R., Ardusso, L. R. F., Dimov, V., et al., for
development of immunologic tolerance (Du Toit et al., the World Allergy Organization. (2013). World Allergy
2008; McLean & Sheikh, 2010). This is therefore no Organization Anaphylaxis Guidelines: 2013 update of the
longer recommended. evidence base. Internal Archives of Allergy and Immunology,
162, 193–204. www.worldallergy.org/anaphylaxix.
• Encourage lactating mothers of infants with a parent or Dhami, S., Panesar, S. S., Roberts, G., et al., for the
sibling with an atopic disease to exclusively breastfeed for EAACI Food Allergy and Anaphylaxis Guidelines Group.
4 to 6 months (Friedman & Zieger, 2005). (2014). Management of anaphylaxis: A systematic review.
• Encourage mothers of infants with a parent or sibling Allergy, 69(2), 168–175.
with an atopic allergic disease who are unable to exclusively
breastfeed for the first 4 months of life to use a hydrolysed
cow’s milk formula (Friedman & Zieger, 2005). • Anaphylaxis is a severe, life-threatening generalized or
systemic hypersensitivity reaction (Johannson et al., 2004).
It is rapid in onset and may cause death (Sampson et al.,
Useful Contacts 2006).
For Patients • Anaphylaxis is commonly triggered by foods, drugs, and
The Anaphylaxis Campaign, 1 Alexandra Road, Farnbor- the venom of stinging insects. It may also be induced by
ough, Hampshire GU14 6SX. Tel.: 01252 546100; exercise, latex, among a number of other triggers. Some
helpline: 01252 542029 (Monday–Friday 9 am–5 pm); cases are idiopathic.
fax: 01252 377140; email: info@anaphylaxis.org.uk; • The UK incidence of anaphylaxis is believed to be increas-
website: www.anaphylaxis.org.uk ing (Department of Health, 2006; Gupta et al., 2007).
Allergy UK, Planwell House, LEFA Business Park, Edgington • It is estimated that there are between 10 and 30 deaths
Way, Sidcup, Kent, DA14 5BH. Helpline: 01322 619898; from anaphylaxis in the United Kingdom each year, many
email: info@allergyuk.org; website: www.allergyuk.org of which are potentially preventable (Newton, 2006;
MedicAlert Foundation, 327 Upper Fourth Street, Milton Pumphrey & Gowland, 2007).
Keynes, MK9 1EH. Tel.: 01908 951045 (Monday–Friday • The classification of reactions into anaphylactic (IgE-
9 am–5 pm, Saturday 9 am–3 pm); email: info@medicalert mediated hypersensitivity reactions) and anaphylactoid
.org.uk; website: www.medicalert.org.uk (non-IgE-mediated mast cell degranulation) is of little
practical relevance to the management of anaphylaxis and
Supermarkets can provide information on products that is now largely avoided.
are “free from” certain ingredients. • Evidence-based guidelines for the management of ana-
phylaxis are available, though note that randomized con-
For Professionals trolled trials of commonly recommended treatments have
British Society for Allergy and Clinical Immunology, Studio not been conducted (Sheikh, Shehata, Brown, & Simons,
16, Cloisters House, 8 Battersea Park Road, London SW8 2008; Sheikh, Simons, & Choo, 2009; Sheikh, ten Broek,
4BG. Tel.: 0207 501 3910; fax: 0207 627 2599; website: Brown, & Simons, 2007). Guidelines are based on the
www.bsaci.org best available evidence.
Education for Health, The Athenaeum, 10 Church Street, Warwick • Anaphylaxis can have a significant long-term impact on
CV34 4AB. Tel.: 01926 493313; email: info@education a patient’s everyday life beyond the immediate ill effects
forhealth.org; website: www.educationforhealth.org of a reaction. Managing an unfamiliar set of risks may
Skills for Health has developed competences to describe what be challenging for patients, particularly immediately after
health professionals need to do, what they need to know, diagnosis. The possibility of further reactions can lead to
and which skills they need to carry out activities related to increased anxiety. Allergen avoidance requires careful
the diagnosis and management of allergy. These are avail- vigilance and may adversely affect the patient’s family
able on their website: http://tools.skillsforhealth.org.uk/ and social life. Good long-term management is therefore
suite/show/id/69 essential in maintaining quality of life (Akeson, Worth,
& Sheikh, 2007; Avery, King, Knight, & Hourihane, 1. Commence life support (basic and advanced) if indicated.
2003; Mandell, Curtis, Gold, & Hardie, 2002; Panesar, 2. Give oxygen.
Walker, & Sheikh, 2003). 3. Give adrenaline (epinephrine) 1:1000 solution (intramus-
cular [IM] injected into the anterolateral aspect of the
Diagnosis middle third of the thigh) if not already administered
• Anaphylaxis presents a range of signs and symptoms, (many patients with a history of anaphylaxis will have
which can sometimes result in diagnostic difficulties. A been issued with adrenaline for self-injection). The Resus-
diagnosis is likely when all three of the following criteria citation Council cautions against the use of the intravenous
are met: (IV) route except by experienced practitioners treating
• Sudden onset and rapid progression of symptoms profound shock (Working Group of the Resuscitation
• Life-threatening airway and/or breathing and/or cir- Council [UK], 2008).
culation problems 4. Give inhaled beta2-agonist if there is severe bronchospasm.
• Skin and/or mucosal changes (flushing, urticaria, 5. Repeat adrenaline 5 to 10 minutes after first dose if there
angioedema) is no clinical improvement.
• There may also be gastrointestinal symptoms such as 6. Give IV crystalloid fluid infusion if symptoms of hypo-
vomiting. tension persist (a repeat dose may be necessary) (Working
• Exposure to a known allergen for the patient supports Group of the Resuscitation Council [UK], 2008).
the diagnosis (Working Group of the Resuscitation Council 7. Consider the use of antihistamines and/or glucocorticosteroids.
[UK], 2008). 8. Following treatment, the patient should be observed for
• Isolated skin and/or mucosal changes do not indicate a minimum of 6 hours in a clinical area suitable for
anaphylaxis. resuscitation if necessary (Working Group of the Resus-
• Diagnosis of anaphylaxis should always be followed up citation Council [UK], 2008).
by specialist-led investigation into the underlying cause. • Trigger. Refer to an allergist to identify the trigger objec-
tively and for consideration of desensitization therapy
Management in those with venom-triggered reactions (Muraro et al.,
• Management is best considered in two stages: treatment 2007; Working Group of the Resuscitation Council
of the acute attack and follow-up care (Dhami, 2014; [UK], 2008). This will involve a detailed history and
Muraro et al., 2007; Simons, 2006; Simons, 2011; Simons, subsequent investigations, which may include skin-
2012; Simons, 2013; Soar, 2008). prick testing, serum-specific IgE tests, and allergen
challenge.
Acute Management (Table 23.2) • Allergen avoidance. Reinforce any allergen advice issued
The key steps for the treatment of an anaphylactic reaction to patients and families, tailored to individual age
are shown in the algorithm (see Appendix 27). and circumstances; dietetic referral may be indicated
in cases of food allergy. Encourage patient/parent to
be proactive in seeking relevant information (e.g.,
TABLE Route of Administration and Drug Dosage
23.2
checking product labels on foods, pharmaceuticals,
for Agents Used in the Emergency Treatment and cosmetic products). Advise avoidance, where
of Anaphylaxis possible, of products carrying “may contain” allergen
Drug (Route of labels.
Administration) Age-Related Dosage • Adrenaline. Issue adrenaline autoinjector for self-
Adrenaline 1:1000 (IM) <6 years: 150 µg (0.15 mL) administration (and educate the patient on when and
6–12 years: 300 µg (0.3 mL) how to use it). Delayed use of adrenaline can lead to
>12 years (small or prepubertal fatality (Pumphrey & Gowland, 2007), so advise
child): 300 µg (0.3 mL) patients not to hesitate in self-administering. In addi-
>12 years: 500 µg (0.5 mL) tion to verbal explanation, use a trainer autoinjector
Chlorphenamine (IM <6 months: 250 µg/kg and ask the patient to demonstrate its correct use.
or slow IV) 6 months–6 years: 2.5 mg Ensure autoinjector dosage is correct. Advise patients
6–12 years: 5 mg to take note of their autoinjector expiry date and make
>12 years 10 mg
arrangements for repeat prescription. Note that adrena-
Hydrocortisone (IM or <6 months: 25 mg line autoinjector users can subscribe to an expiry alert
slow IV) 6 months–6 years: 50 mg service by letter, email, or SMS text message (see Useful
6–12 years: 100 mg
>12 years: 200 mg Contacts). Multiple autoinjectors may need to be pre-
scribed to cover different sites, such as one for home
Crystalloid fluid (IV) Children: 20 mL/kg and one for school or the workplace.
Adults: 500–1000 mL
• Alert bracelet. Recommend purchase of a medical iden-
IM, intramuscular; IV, intravenous. tity bracelet/necklace or smart card documenting history
of anaphylaxis and that adrenaline is carried.
CHAPTER 23 Allergic Problems 407
• Asthma control. Optimize asthma management in those Boyce, J. A., Assa’ad, A., Burks, J. A., et al. (2010). NIAID-sponsored expert
with a history of asthma, as the risk of fatality is panel. Guidelines for the diagnosis and management of food allergy in
increased in this group (Pumphrey & Gowland, 2007). the United States: Summary of the NIAID-Sponsored Expert Panel
• Liaise with nursery/school/school nurse/work as appro- report. Journal of Allergy and Clinical Immunology, 126, 1105–1118.
Choo, K., & Sheikh, A. (2007). Action plans for the long-term manage-
priate (Vickers, Maynard, & Ewan, 1997).
ment of anaphylaxis: A systematic review of effectiveness. Clinical
• Review the patient 6 months after diagnosis, and there- and Experiment Allergy, 37, 1090–1094.
after every year, and following any subsequent reactions. Department of Health (2006). A review of services for allergy. London:
Reviews should be used to support patients’ self- Author.
management as appropriate: for example, reinforcing de Silva, D., Geromi, M., Halken, S., et al. with the EAACI Food
allergen avoidance advice, encouraging carrying adrena- Allergy and Anaphylaxis Group. (2014). Primary prevention of
line autoinjector, retraining in autoinjector use, or food allergy in children and adults: Systematic review. Allergy,
referring to specialist for retesting. A management plan 69(5), 581–589.
incorporating training in adrenaline use, support, and de Silva, D., Geromi, M., Panesar, S. S., et al. with the EAACI Food
follow-up may prove useful (Choo & Sheikh, 2007; Allergy and Anaphylaxis Group. (2014). Acute and long-term man-
Hourihane, 2001; Muraro et al., 2007; Nurmatov, agement of food allergy: Systematic review. Allergy, 69(2), 159–167.
Dhami, S., Panesar, S. S., Roberts, G., et al. with the EAACI Food
Worth, & Sheikh, 2008).
Allergy and Anaphylaxis Guidelines Group. (2014). Management
of anaphylaxis: A systematic review. Allergy, 69(2), 168–175.
Useful Contacts Durham, S. R., & Church, M. K. (2006). Principles of allergy diagno-
sis. In S. T. Holgate, M. K. Church, & L. M. Lichtenstein (Eds.),
For Patients Allergy (3rd ed., pp. 3–16). Philadelphia, PA: Mosby Elsevier.
The Anaphylaxis Campaign, 1 Alexandra Road, Farnbor- Du Toit, G., Katz, Y., Sasieni, P., et al. (2008). Early consumption of
ough, Hampshire GU14 6SX. Tel.: 01252 546100; peanuts in infancy is associated with a low prevalence of peanut
helpline: 01252 542029 (Monday–Friday 9 am–5 pm); allergy. Journal of Allergy and Clinical Immunology, 122, 984–991.
fax: 01252 377140; email: info@anaphylaxis.org.uk; Fiocchi, A., Assa’ad, A., & Bahna, S. (2006). Food allergy and the
website: www.anaphylaxis.org.uk introduction of solid foods to infants: A consensus document.
Allergy UK, Planwell House, LEFA Business Park, Edgington Annals of Allergy and Asthma Immunology, 97, 10–21.
Way, Sidcup, Kent, DA14 5BH. Helpline: 01322 619898; Friedman, N. J., & Zieger, R. S. (2005). The role of breast-feeding
in the development of allergies and asthma. Journal of Allergy and
fax: 01322 470330; email: info@allergyuk.org; website:
Clinical Immunology, 115, 1238–1248.
www.allergyuk.org Grimshaw, K. E. C. (2006). Dietary management of food allergy in
The MedicAlert Foundation, 327 Upper Fourth Street, children. Proceedings of the Nutrition Society, 65, 412–417.
Milton Keynes, MK9 1EH. Tel.: 01908 951045; email: Gupta, R., Sheikh, A., Strachan, D. P., & Anderson, H. R.
info@medicalert.org.uk; website: www.medicalert.org.uk (2007). Time trends in allergic disorders in the UK. Thorax, 62,
91–96.
Supermarkets can provide information on products that Hourihane, J. (2001). Community management of severe allergies
are “free from” certain ingredients. must be integrated and comprehensive, and must consist of more
than just epinephrine. Allergy, 56, 1023–1025.
For Professionals Johannson, S. G. O., Bieber, T., Dahl, R., et al. (2004). Revised nomen-
British Society for Allergy and Clinical Immunology, Studio clature for allergy for global use: Report of the Nomenclature
Review Committee of the World Allergy Organization, October
16, Cloisters House, 8 Battersea Park Road, London SW8
2003. Journal of Allergy and Clinical Immunology, 113, 832–836.
4BG. Tel.: 0207 501 3910; fax: 0207 627 2599; website: Mandell, D., Curtis, R., Gold, M., & Hardie, S. (2002). Families
www.bsaci.org coping with a diagnosis of anaphylaxis in a child. Allergy & Clinical
Education for Health, The Athenaeum, 10 Church Street, Immunology International, 14, 96–101.
Warwick CV34 4AB. Tel.: 01926 493313; email: McLean, S., & Sheikh, A. (2010). Does avoidance of peanuts in early
info@educationforhealth.org; website: www.educationfor life reduce the risk of peanut allergy? British Medical Journal (Clini-
health.org cal Research Ed.), 340, c424. doi:10.1136/bmj.c424.
Adrenaline prescribing information. Includes autoinjector Munoz-Furlong, A. (2003). Daily coping strategies for patients and
demonstration and details of expiry alert system; websites: their families. Pediatrics, 111, 1654–1661.
http://www.epipen.com, www.jext.co.uk Muraro, A., Roberts, G., Clark, A., et al. (2007). The management of
anaphylaxis in childhood: Position paper of the European Academy
of Allergology and Clinical Immunology. Allergy, 62, 857–871.
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Akeson, N., Worth, A., & Sheikh, A. (2007). The psychological impact in children and young people in primary care and community settings.
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Experimental Allergy, 37, 1213–1220. Newton, J. (2006). An epidemiological report for the department of
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Allergy and Immunology, 14, 378–382. ment plans for the acute and long-term management of anaphylaxis:
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122, 353–361. (2007). H1-Antihistamines for the treatment of anaphylaxis with
Panesar, S., Walker, S., & Sheikh, A. (2003). Primary care management and without shock. Cochrane Database of Systematic Reviews, (1),
of anaphylaxis. Primary Care Respiratory Journal, 12, 124–126. CD006160, doi:10.1002/14651858.CD006160.pub2.
Perry, T. T., Scurlock, A. M., & Jones, S. M. (2006). Clinical mani- Simons, F. E. R. (2006). Anaphylaxis, killer allergy: Long-term manage-
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CD007596. pages/reaction.pdf.
24
Diabetes and Endocrinology
RUSSELL DRUMMOND, FRANCES MCMANUS,
KATE HUGHES, SHARON MACKIN, DAVID CARTY
Type 2 Diabetes Mellitus Thyroid Disease
Diagnosis Hypothyroidism
Glycated Haemoglobin A1c Primary Hypothyroidism
What Type of Diabetes? Secondary Hypothyroidism
Primary Care Investigations Subclinical Hypothyroidism
Referral Hyperthyroidism
Screening for Undiagnosed Type 2 Diabetes and Primary Hyperthyroidism
Those at Risk Secondary Hyperthyroidism
Pharmacologic Therapies in Preventing Type 2 Diabetes Graves Disease
Management of Type 2 Diabetes Thyroid Nodules
Glycaemic Control Sick Euthyroid
Treatments Thyroid Disease in Pregnancy
Home Glucose Monitoring
Hypoadrenalism
Hypoglycaemia
Diagnosis
Sickday Rules for Patients on Insulin Therapy
Treatment
Blood Pressure Management
Hypercalcaemia
Other Cardiovascular Risk Factors
Symptoms
Lipids
Causes
Antiplatelet Therapy
Initial Tests
Smoking
Primary Hyperparathyroidism
Diabetic Nephropathy
Treatment
Diabetic Retinopathy and Maculopathy
Hirsutism
Diabetic Foot Disease and Neuropathy
Treatment
Erectile Dysfunction Referral
Driving Regulations
Gynaecomastia
General Advice for Driving with Diabetes
Clinical Evaluation
Gestational Diabetes Management
Screening and Diagnosis Hypogonadism in Adult Men
Treatment
Issues Postpregnancy
Type 1 Diabetes in Pregnancy
409
Type 2 Diabetes Mellitus • fasting plasma glucose (FPG) of >7 mmol/L; or

• 2-hour plasma glucose of >11.1 mmol/L after 75-g
GUIDELINES oral glucose tolerance test (OGTT);
• glycated haemoglobin A1c (HbA1c) >48 mmol/
Management of diabetes. SIGN guideline 116. www.sign. mol (6.5%).
ac.uk. b. In the absence of hyperglycaemic symptoms, a further
National Institute for Health and Care Excellence. (2015). glucose test on a separate day should be performed.
Type 1 diabetes in adults: diagnosis and management. NICE • Impaired fasting glucose: FPG of 6.1 to 6.9 mmol/L
clinical guideline 17. www.nice.org.uk. and a 2-hour OGTT <7.8 mmol/L
Type 2 diabetes in adults: Management. NICE clinical • Impaired glucose tolerance: FPG <7 mmol/L and a
guideline 28. www.nice.org.uk. 2-hour OGTT 7.8 to 11 mmol/L
Type 2 diabetes: Prevention in people at high risk. NICE Glycated Haemoglobin A1c
clinical guideline 38. www.nice.org.uk. • The role of HbA1c in diagnosis of diabetes has been
Diabetic foot problems: Prevention & management. NICE recognized. However, it is less sensitive than a FPG mea-
clinical guideline 19. www.nice.org.uk. surement and an HbA1c less than 48 mmol/mol (6.5%)
does not exclude diabetes.
• It should NOT be used in:
• suspected type 1 diabetes;
• Diabetes mellitus has become a global epidemic with a • children;
rapidly expanding prevalence exceeding predictions. Recent • short duration of osmotic symptoms (i.e., <3 months);
UK data suggest: • pregnancy;
• almost 3.5 million people are diagnosed with diabetes • suspected medication-induced secondary diabetes (e.g.,
in United Kingdom; steroids, antipsychotics);
• an estimated 500,000+ are undiagnosed; • suspected pancreatic diabetes;
• more than 6% of the total population are affected; • those with haemoglobinopathies/known factors that
• current National Health Service (NHS) expenditure affect red cell turnover.
for diabetes care is in the region of 10% of the total
budget; What Type of Diabetes?
• approximately 90% type 2, 10% type 1 with small • This question can sometimes be challenging, but some
subgroups secondary/other diabetes; clues can make a diagnosis more likely (Table 24.1).
• an alarming increase in numbers of children with type • Other subtypes of diabetes may have features of either
2 diabetes, a previously unknown entity. type 1 or type 2:
• The key roles in primary care management of diabetes
are:
TABLE
• prompt recognition and diagnosis; 24.1 Typical Features of Type 1 and 2 Diabetes
• identification of at-risk individuals and intervention
to prevent type 2 diabetes; Type 1 Diabetes Type 2 Diabetes
• identification of all patients with diabetes in practice Young age (<40 years) Older generally
within a registry and a means for recall that enables
annual review; Presentation in childhood More indolent presentation
• prevention of microvascular and macrovascular com- Presence of ketones Metabolic syndrome
plications through management of glycaemia and car- Acute osmotic symptoms Obesity
diovascular risk factors; with appropriate management/
referral for established complications; Normal BMI South Asian predisposition
at lower BMI
• encourage patient self-management;
• optimize pregnancy outcomes for woman with diabetes; Approx. 1 in 25 chance Stronger genetic
• appropriate liaison/referral to secondary care. of developing predisposition
condition if first-degree
relative affected
Diagnosis Autoantibodies present Absence of autoantibodies
• The World Health Organisation (WHO) criteria is the C-peptide low and C-peptide raised unless
internationally accepted diagnostic criteria for diabetes. eventually significantly advanced
It is defined as: undetectable disease with beta cell
destruction
a. the presence of symptoms of hyperglycaemia (polyuria,
polydipsia, and weight loss) with: BMI, Body mass index.
• random plasma glucose of >11.1 mmol/L; or
CHAPTER 24 Diabetes and Endocrinology 411
TABLE HbA1c in Relation to Mean Blood

24.2 Referral
Glucose Levels
• Those with type 2 diabetes are generally managed in
HbA1c Mean Blood
primary care, unless:
(mmol/mol) HbA1c (%) Glucose (mmol/L)
• significant micro-/macrovascular complications;
31 5 5.4 • failure to reach glycaemic and blood pressure targets
42 6 7.0 despite maximal therapy;
• unclear diagnosis;
53 7 8.6 • younger than 40 years of age.
64 8 10.1 • Women with gestational diabetes or preexisting diabetes
75 9 11.7 in pregnancy should be referred immediately to a com-
bined diabetes and obstetrics team.
86 10 13.3
97 11 14.9 Screening for Undiagnosed Type 2
108 12 16.5 Diabetes and Those at Risk
119 13 18.1 • Around 500,000 people in the United Kingdom are
130 14 19.6 thought to have undiagnosed type 2 diabetes. Many
patients have disease for 7 to 10 years preceding diagnosis
and present with established complications. Despite this,
1. Pancreatic diabetes: there is a lack of evidence to suggest population screening
• Known pancreatic pathology for type 2 diabetes is cost effective or that it would improve
• Features of pancreatic exocrine dysfunction population health. However, high-risk individuals should
2. Secondary diabetes: be screened, including:
• Associated causative agent (e.g., steroids, antipsychotics) • all adults aged 40 years or above, unless pregnant;
• Features of endocrinopathy (e.g., acromegalic or • people aged 25 to 39 of South Asian, Chinese, black
cushingoid appearances) African, African-Caribbean, or other high-risk ethnicity;
3. Latent autoimmune diabetes in adults (LADA): • those with a condition known to increase risk of type
• Slow and delayed presentation of type 1 diabetes 2 diabetes (e.g., polycystic ovarian syndrome).
• Autoantibodies present • National Institute of Health and Care Excellence (NICE)
• Low and declining levels of C-peptide has published guidance on preventing type 2 diabetes in
• Often misdiagnosed as type 2 particularly in patients those at high risk (NICE, 2012).
not phenotypically type 2 diabetes • The first priority is to identify high-risk patients using a
• Quicker progression to insulin therapy than validated risk assessment tool (e.g., the Diabetes Risk
type 2 Score Assessment tool, available at the Diabetes UK
4. Genetic diabetes (e.g., maturity onset diabetes of the website: www.diabetes.org.uk):
young [MODY]): 1. Low or intermediate risk:
• Strong family history a. Advice on lifestyle intervention
• Age less than 40 years and not requiring insulin b. Reassurance whilst stressing that the result doesn’t
mean no risk
Primary Care Investigations c. Reassess in 5 years
• Make the diagnosis using WHO criteria. 2. High risk score:
• Assess need for emergency treatment: a. Perform FPG or HbA1c.
a. Hyperglycaemia with ketones b. FBG below 5.5 mmol/L or HbA1c below 42 mmol/
b. Children with glycosuria: mol = moderate risk
• Do a point-of-care glucose test and discuss if • Inform patient of increased risk of developing
hyperglycaemia. type 2 diabetes.
• DO NOT wait on labs to come back and do NOT • Discuss risk factor modification.
arrange for child to come back for a second lab test • Direct to appropriate local weight-loss or fitness
to confirm diagnosis, regardless of symptoms. programmes.
c. Suspicion of type 1 diabetes • Reassess in 3 years.
d. Blood glucose above 30 mmol/L c. FBG 5.5 to 6.9 mmol/L or HbA1c 43 to 47 mmol/
e. Acutely unwell and hyperglycaemic mol = high risk
• Other tests are limited to secondary care: • Inform patient that he or she is high risk for
a. C-peptide developing type 2 diabetes but still preventable.
b. Auto-antibodies (Anti-GAD) • Offer referral to intensive lifestyle-change pro-
c. Genetics gramme with emphasis on increasing physical
activity to at least 150 minutes moderate activity • liver function tests;

per week, weight loss, and dietary advice to increase • lipids;
wholegrain food and vegetables with reduction • urine for albuminuria (urinary albumin-to-creatinine
in fatty foods and simple carbohydrates. ratio [ACR] convenient and widely available).
• Review progress and biochemistry on a minimum • Patients on medication for treatment of diabetes qualify
annual basis. for a medical exemption certificate allowing them free
d. FBG at or above 7 mmol/L or HbA1c at or above prescriptions, therefore form FP92A should be completed.
48 mmol/mol:
• If asymptomatic, retest on another day as per Glycaemic Control
WHO guidelines for overt diabetes. • The United Kingdom Prospective Diabetes Study (UKPDS)
• If not diagnostic of diabetes, then intervention was a randomized control study from 1977 to 1997 that
as per high risk. showed a 25% reduction in microvascular complications
with intensive glycaemic control versus conventional targets
Pharmacologic Therapies in Preventing (HbA1c 7% versus 7.9%). These risk reductions were
Type 2 Diabetes largely seen in retinopathy and nephropathy. There was
• NICE suggests metformin can be used to prevent progres- no impact on macrovascular disease outcomes from
sion to type 2 diabetes in patients whose FBG/HbA1c are improvements in glycaemic control.
deteriorating despite best efforts in lifestyle intervention, • Current guidelines advise glycaemic targets that are indi-
or in those where such intervention is inappropriate. vidualized to each patient.
• Orlistat can also be considered in those progressing bio- • Scottish Intercollegiate Guidelines Network (SIGN) sug-
chemically to type 2 diabetes with obesity where intensive gests aiming for an HbA1c below 53 mmol/mol
lifestyle intervention has failed. It needs to be used along- (<48 mmol/mol early in the diagnosis) whilst NICE sug-
side a lowfat (<30%) diet, and weight-loss goals should gests below 48 mmol/mol if no risk of hypoglycaemia.
be reviewed regularly to monitor effectiveness. • Sometimes relaxed targets are appropriate (e.g., severe
hypoglycaemia risk, end-of-life care).
Management of Type 2 Diabetes Treatments
• The focus of type 2 diabetes management is ultimately • Lifestyle intervention with education on diet and physical
to prevent development of microvascular (retinopathy, activity should be trialled for 3 to 6 months.
neuropathy, nephropathy) and macrovascular (coronary • If HbA1c remains above target, then consider pharmaco
artery, cerebrovascular and peripheral vascular disease) therapy.
complications associated with it. This is achieved by tar- • Treatment of type 2 diabetes is becoming more complex,
geting glycaemia and controlling cardiovascular risk factors. and less algorithm based with new evidence emerging on
• At diagnosis and annual review, patients should have the the newer hypoglycaemic agents and potential cardiovas-
following: cular benefits they confer.
• Explanation of type 2 diabetes, complications, and • Optimization of therapy takes time and the urge to
means of reducing risk intensify therapy needs to be balanced with time to
• Weight and height achieve desired effect. This can lead to treatment inertia,
• Blood pressure meaning patients remain suboptimally controlled for
• Cardiovascular examination, including peripheral pulses months, and in some cases years, despite further options
• Examination of feet for neuropathy and ulcers for treatment intensification. To minimize this, at least
• Visual acuity 3-monthly review of therapy is suggested until HbA1c
• Referral to national retinal screening programme is within target.
• Smoking status documented and advice given
• Ask men about erectile dysfunction Metformin and First Intensification of Treatment
• Mood assessed • Remains the first line oral agent for glycaemic control in
• Driving status and advice with regard to Driver and type 2 diabetes.
Vehicle Licensing Agency (DVLA) regulations • Cost effective and has data showing cardiovascular benefits
• Referral to dietician and diabetes specialist nurse for (UKPDS).
education on lifestyle intervention • Biguanide: Mechanism of action not entirely understood
• Referral to a locally available type 2 diabetes education but decreases hepatic gluconeogenesis and improves insulin
programme sensitivity.
• Discussion about contraception for women of child- • Start with 500 mg daily and titrate to a maximum toler-
bearing age ated dose of 2 g daily (standard release).
• Laboratory tests should be sent for: • Patients may experience gastrointestinal (GI) side effects
• urea and electrolytes; and should be reassured that these are often mild and
• glucose and HbA1c; Table 24.2 defines HbA1c units should settle. A modified-release preparation may be
and corresponding average blood glucose levels; helpful.
• Theoretic risk of lactic acidosis may require a break from • Side effects: fluid retention, weight gain, increased risk of
therapy during acute illness. heart failure, small increased risk of bladder cancer,
• Contraindications: severe liver failure, kidney injury with increased risk of bone fractures, and liver dysfunction.
estimated glomerular filtration rate (eGFR) less than • Contraindications: pre-existing heart failure or high risk
30 mL/min (with dose reduction at higher eGFR). for heart failure, previous or active bladder cancer or
• If after 3 to 6 months of lifestyle intervention and met- significant family history of bladder cancer, hepatic impair-
formin, HbA1c is above 58 mmol/mol, then the first ment, haematuria of unknown aetiology.
treatment intensification should take place. Treatment • Use with caution in those at risk of falls or at increased
should be individualized but options include: risk of bone fracture (e.g., osteoporosis).
• metformin + sulphonylurea • Monitor liver function before treatment, after 2 months,
• metformin + dipeptidyl peptidase-4 (DPP-IV) inhibitor and periodically thereafter.
• metformin + sodium-glucose cotransporter 2 (SGLT-2)
inhibitor Sodium-Glucose Cotransporter 2 Inhibitors
• metformin + pioglitazone • These include empagliflozin, dapagliflozin, and cana-
• The target HbA1c should be less than 53 mmol/mol. gliflozin. The choice of agent will depend on local policy.
• Act by blocking the SGLT2 receptor in the proximal
Sulphonylurea tubule of kidney, preventing reabsorption of glucose and
• Gliclazide most commonly used. Works by stimulating water resulting in glycosuria.
pancreas insulin secretion. Side effects include hypogly- • Benefits: weight loss, reduction in blood pressure, absence
caemia and weight gain. Contraindications include eGFR of hypoglycaemia unless used with other hypoglycaemic
below 30 mL/min due to risk of severe hypoglycaemia. agents.
• Typical starting dose of 80 mg once daily and titrated to • Side effects: volume depletion, hypotension, electrolyte
a maximum of 160 mg twice daily. Lower dose of 40 mg disturbance, genitourinary infections, ketoacidosis.
daily if concern of hypoglycaemia. • Ketoacidosis has been reported in patients with type 2
• Patients should be made aware of the symptoms of hypo- diabetes taking an SGLT2 inhibitor, even at near-normal
glycaemia and be trained to check their own blood sugars glucose levels. Patients need to be counselled on symptoms
at relevant times. of ketoacidosis (nausea, vomiting, abdominal pain,
• DVLA restrictions should be discussed (see p. 416). anorexia) and advised to seek medical attention if they
develop these.
Dipeptidyl Peptidase-4 Inhibitors • Contraindications: renal impairment with eGFR below
• Options include saxagliptin, sitagliptin, linagliptin, alo- 60 mL/min, concomitant use with loop diuretic, keto-
gliptin, vildagliptin; and the choice between these should acidosis, recurrent genitourinary sepsis.
be based on local policy. • Use with caution in patients at increased risk of volume
• Incretin-based therapy. Blocks DPP-IV, preventing inac- depletion.
tivation of GLP-1 and subsequent inhibition of glucagon • Cardiovascular outcome studies:
release, increased insulin secretion from pancreas, and • Empagliflozin, Cardiovascular Outcomes and Mortality
decreased gastric emptying. These events lead to reduced in Type 2 Diabetes (EMPA-REG) study showed patients
blood glucose levels. with type 2 diabetes and established cardiovascular disease
• Expected to reduce HbA1c by approximately 0.5%. taking empagliflozin had a significant reduction in major
• Benefits: weight neutral, low risk of hypoglycaemia unless adverse cardiovascular events (cardiovascular death, non-
used with insulin or sulphonylurea. fatal myocardial infarction [MI], and nonfatal stroke)
• Side effects: GI side effects, nasopharyngitis, pancreati- and all-cause mortality. These results were driven by a
tis. Dose adjustment needed for renal impairment with 38% relative risk reduction in heart failure admissions.
exception of linagliptin. While there are no data to • The Canagliflozin and Cardiovascular and Renal Events
suggest increase in cardiovascular (CV) death associated in Type 2 Diabetes (CANVAS) study also showed
with DPP-IV use, given current conflicting data, they cardiovascular benefit of Canagliflozin in high-risk
should be used with caution in those at high risk of heart cardiovascular patients. There was however an increase
failure. in toe and metatarsal amputations.
Further study results in this group are awaited.
Pioglitazone
• Thiazolidinedione, PPAR-γ agonist decreasing peripheral Second Intensification
insulin resistance. • If HbA1c remains above 58 mmol/mol after 3 to 6 months
• Decreases triglycerides but increases high-density lipo- on two agents, a second intensification of therapy should
protein (HDL) and low-density lipoprotein (LDL) take place. Options include:
cholesterol. • metformin, DPP-IV inhibitor + sulphonylurea;
• Dose 15 to 45 mg once daily. • metformin, pioglitazone + sulphonylurea;
• Risk of hypoglycaemia if used alongside a sulphonylurea • metformin, pioglitazone or sulphonylurea + SGLT-2
or insulin. inhibitor;
• metformin, sulphonylurea + glucagon-like peptide 1 Other Therapies

(GLP-1) agonist (only if BMI >35 kg/m2 in England • Meglitinides can be used for patients intolerant to
and Wales, or >30 kg/m2 in Scotland); sulphonylurea.
• insulin therapy. • Similar to sulphonylureas
• More expensive
Glucagon-Like Peptide 1 Agonists • Acarbose:
• These include liraglutide, exenatide, lixisenatide, • Blocks intestinal alpha glucosidase preventing starchy
semaglutide—all of which are injectable. They work by compounds being broken down to simple sugars.
enhancing pancreatic insulin secretion in a glucose- • Not as effective, and intolerable GI side effects.
dependent manner, inhibit glucagon release, and delay
gastric emptying thus promoting satiety. Home Glucose Monitoring
• There are various regimes from twice-daily injection to
once weekly. • Required for ALL patients on insulin, and in those on
• Benefits: promotes weight loss whilst reducing hypergly- sulphonylurea who drive or are at risk of hypoglycaemia.
caemia, low risk of hypoglycaemia if not used alongside • Not generally required for patients with type 2 diabetes
insulin or sulphonylureas. on other regimes.
• Side effects: GI side effects often intolerable, skin reac-
tions at injection sites, pancreatitis, theoretical risk of Hypoglycaemia
increased pancreatic and medullary thyroid cancer but • All patients on insulin or sulphonylurea should be edu-
not proven. cated on the symptoms of hypoglycaemia and how to
• Contraindications: gastroparesis, pancreatic pathology, treat it.
previous or pre-existing thyroid cancer, chronic kidney • They should be advised to check blood glucose to confirm
disease (CKD) with eGFR below 30 mL/min, hepatic hypoglycaemia (<4 mmol/L) if they have symptoms.
impairment. • If conscious and swallow safe, then they should take 15
• They are expensive: to 20 g of oral glucose:
• Most health boards will have specific criteria to monitor • 200 mL fresh fruit juice
effectiveness of treatment before funding for more than • 120 mL Lucozade
6 months. • 4 jelly babies
• NICE suggests a minimum HbA1c reduction of 1% • 4 to 5 dextrose tablets
AND weight loss of 3% or more be achieved within • Blood glucose should be rechecked 15 minutes later and
6 months of starting treatment or therapy should be treatment repeated if needed.
discontinued. • Once resolved, the patient should eat some complex car-
• Cardiovascular outcome trials have also shown signifcant bohydrate (e.g., sandwich).
cardiovascular benefit of GLP-1 agonist therapy in the • If unconscious or swallow not safe, caregiver should give
LEADER (Liraglutide) and SUSTAIN-6 (Semaglutide) intramuscular (IM) glucagon 1 mg and call ambulance.
trials. • Medically trained staff will be able to administer intra-
venous (IV) glucose (150 mL 10% glucose or 75 mL
Insulin 20% glucose).
• Once-daily NPH insulin is usually sufficient for initiation • Identify and correct cause for hypoglycaemia.
of insulin in patients with type 2 diabetes.
• A total daily dose of 0.3 to 0.5 units/kg is a reasonable Sickday Rules for Patients on Insulin Therapy
starting dose (maximum first dose of 10 units). • If possible, patients should keep up dietary intake.
• Metformin should continue. • If unable, oral fluids are important to prevent dehydration.
• Other hypoglycaemic agents should be reviewed for con- • A trial of easily digested foods (soup, jelly) may be effective.
tinuation, stopping, or dose adjustment. • Check blood glucose at least 4-hourly.
• Insulin analogues such as detemir and glargine may • DO NOT omit insulin therapy.
be preferred for those at risk of overnight or severe • If hypoglycaemia occurs, treat with sugary drinks.
hypoglycaemia. • If refractory hypoglycaemia, seek medical advice.
• If NPH insulin is insufficient, twice-daily mixed prepara- • If blood glucose above 15 mmol/L, check for ketones.
tions can be tried. • If ketones positive, administer 10% of total daily insulin
• Before starting insulin, the patient must: dose in short-acting insulin and repeat blood glucose and
• have education on insulin administration and blood ketones after 2 hours. If not resolving, repeat step but if
glucose monitoring from a diabetes educator; worsening seek medical help.
• have open access to a clinician capable of advising on • If hyperglycaemia but no ketones, patient can give a cor-
dose adjustments during initiation; rection dose of short-acting insulin. A correction dose
• know how to recognize and treat hypoglycaemia; can be calculated as 100/total daily insulin dose. For
• discuss driving regulations and occupational hazards. example, a patient on 50 units of insulin per day would
have a correction dose assuming that 1 unit of insulin • Nicotine replacement therapies are recommended but
will correct blood glucose by 2 mmol/L. vapours are not, in view of a lack of safety data.
• This can be repeated after 2 hours also, but if worsening
seek medical advice. Diabetic Nephropathy
Blood Pressure Management • Microalbuminuria is the first sign of diabetic kidney

disease, and is associated with increased cardiovascular
• Targets: morbidity and mortality.
• SIGN suggests targeted blood pressure below 130/ • It is defined as:
80 mmHg for all patients with diabetes. • 24-hour urinary albumin of 30 to 300 mg;
• NICE suggests targets of below 140/80 mmHg (or • urinary ACR above 2.5 mg/mmol in men (3.5 mg/
<130/80 mmHg if nephropathy, retinopathy, or cere- mmol in women).
brovascular disease). • Overt diabetic nephropathy is defined by an ACR above
• The UKPDS study showed every 10-mmHg reduction 30 mg/mmol and is a strong indicator of cardiovascular
in systolic blood pressure (SBP) reduced 10-year risk risk and progression to end-stage renal failure.
of CV death by 15%. • Urinary ACR and eGFR should be monitored at least
• Intensive blood pressure control to these targets reduces annually in patients aged 12 years and older with diabetes.
the development of microvascular complications. • If microalbuminuria present, ACE inhibitor should be
• Further intensification to below these values is not started irrespective of blood pressure as it will slow progres-
recommended (ACCORD). sion to overt nephropathy and can reverse microalbuminuria.
• Treatments: • Intensive glycaemic and blood pressure control is vital to
• An angiotensin-converting enzyme (ACE) inhibitor preventing progression.
is first line therapy, in addition to intensive lifestyle
intervention: Diabetic Retinopathy and Maculopathy
a. If intolerant, an angiotensin receptor blocker should
be used instead. • Up to 40% of patients with type 2 diabetes have retino-
b. If of black origin, a calcium channel blocker is more pathy at diagnosis.
appropriate. • All patients with type 2 diabetes should undergo retinal
• Beta blockers and alpha blockers are not first line in screening at diagnosis, and at least annually thereafter.
patients with diabetes and hypertension, but can be used • Retinal screening is best performed as part of the national
for intensification of therapy or other therapeutic reasons. screening programme using digital photography.
• BP should be monitored once or twice monthly until • Fundoscopy is not reliable.
consistently within target. • Retinopathy is defined as:
• background retinopathy;
• preproliferative retinopathy;
Other Cardiovascular Risk Factors • proliferative retinopathy.
Lipids • Proliferative or referable preproliferative retinopathy should
• Statin therapy should be offered to all patients with diabe referred to ophthalmology for consideration of laser
betes whose 10-year CV risk is 10% or more, regardless photocoagulation therapy.
of cholesterol. • Urgent referral if neovascularization is near the macula
• This generally includes all patients with diabetes aged or associated vitreous haemorrhage.
over 40 years, and younger patients with microvascular • Macular oedema should also be referred urgently.
complications. • Intensive blood pressure and glycaemic control should
• Primary prevention doses are: be targeted to prevent progression, and patients must
• atorvastatin 20 mg (NICE); stop smoking.
• simvastatin 40 mg or atorvastatin 10 mg (SIGN).
• Secondary prevention doses are higher with atorvastatin Diabetic Foot Disease and Neuropathy
80 mg recommended.
• Fibrates may be considered for those intolerant of statin • The principal risk factors for the development of foot
but there is a lack of evidence of efficacy. ulceration in patients with diabetes are neuropathy and
peripheral vascular disease.
Antiplatelet Therapy • All patients with diabetes should have their feet screened
• Not recommended for primary prevention. at diagnosis, then at least annually and if any foot prob-
lems occur. Assessment must include the peripheral cir-
Smoking culation (pulses), sensation (with a 10-g monofilament),
• All patients should be advised to stop and referred to the presence of structural abnormalities, and any history
smoking cessation services. of previous ulceration. The feet should be risk stratified
(low, moderate, high, or active foot disease) and manage- • Proof of blood glucose monitoring to demonstrate accept-
ment plan discussed. able control and at times appropriate to driving must be
• Foot care education should be given to all patients with available. Three months of such monitoring (on a glu-
diabetes. Importantly, the person’s current risk of develop- cometer with date and time function) is reviewed by the
ing foot problems and who to contact if a foot emergency patient’s doctor prior to renewal of licence.
should arise. Moderate- and high-risk feet should have
input from the foot protection service. General Advice for Driving With Diabetes
• All patients with active ulceration and signs of sepsis, • Check blood glucose immediately before driving.
ulceration with critical limb ischaemia, gangrene, or deep- • Aim to be above 5 mmol/L before commencing journey.
seated soft tissue/bone infection should be referred urgently • Check blood glucose every 2 hours as a minimum on
to the acute hospital and the multidisciplinary foot care long journeys, but sooner if symptoms of hypoglycaemia.
service informed. • Keep quick-acting carbohydrate in the car at all times to
• For all other active foot problems and suspected Charcot enable prompt treatment.
(swelling, redness, and warmth of a foot with or without • Follow-up treatment of hypoglycaemia with complex
pain, especially if the skin is intact), refer urgently to the carbohydrate (e.g., sandwich).
multidisciplinary foot care service. • If blood glucose is below 4 mmol/L at any point, treat
• For the management of neuropathic pain NICE (2013) rec- the hypoglycaemia and do not resume driving until at
ommends offering a choice of amitriptyline, duloxetine, gaba- least 45 minutes of a blood glucose above 5 mmol/L has
pentin, or pregabalin initially. If one is not effective or tolerated been achieved, and patient feels well enough to continue
then one of the remaining three agents should be offered. safely.
Erectile Dysfunction Gestational Diabetes

• Erectile dysfunction is common and a marker of underly- GUIDELINES
ing cardiovascular disease.
• Ask about symptoms at annual review and offer a phos- Diabetes in pregnancy: Management from pre-conception
phodiesterase type-5 inhibitor if no contraindications. to the postnatal period. NICE clinical guideline 3. www.nice
• If pharmacologic therapy is unhelpful, refer to a local .org.uk.
erectile dysfunction service. Scottish Intercollegiate Guidelines Network. (2013).
• Testosterone therapy is controversial and should not be Management of diabetes. SIGN guideline 116. www.sign
.ac.uk.
commenced without consulting endocrinology.
Driving Regulations
• Gestational diabetes mellitus (GDM) is defined as glucose
• Patients treated with insulin may hold a Group 1 (car or intolerance with onset or first recognition during
motorcycle) or a Group 2 (lorry or bus) licence; however, pregnancy. Maternal risk factors for GDM are shown in
the DVLA must be informed. Their licence will be reviewed Table 24.3; women should be assessed for these factors
every 3 years, or sooner in the context of any of these in early pregnancy, and if present should be offered screen-
events: ing with a 75-g oral glucose tolerance test at 24 to 28
• More than one severe hypoglycaemic attack (requiring weeks of gestation, or earlier if they have a history of
third-party assistance) in the preceding 12 months GDM in a previous pregnancy.
whilst awake for group 1 licence holders. or
• Any severe hypoglycaemic attack whilst driving Screening and Diagnosis
• Impaired hypoglycaemic awareness
• Retinopathy requiring laser treatment • Current treatment of GDM is influenced by a number
• Loss of vision in one eye of large clinical trials, showing that treatment of GDM
• Unable to read a car number plate (with glasses if
needed) at 20 m in daylight or have reduced visual TABLE Risk Factors for Gestational Diabetes
24.3 (NICE, 2015)
acuity of less than 6/12 (with glasses)
• Neuropathy or peripheral vascular disease that impacts Body mass index (BMI) >30 kg/m2
on their ability to drive a standard manual car Previous macrosomic baby weighing ≥4.5 kg
• Requirements for patients holding a Group 2 licence are
tighter and in addition to the above, the DVLA needs Previous gestational diabetes mellitus
to be informed if: Family history of diabetes (first-degree relative with
• the patient is taking any medication for diabetes, regard- diabetes)
less of perceived hypoglycaemic risk; Minority ethnic family origin with a high prevalence of
• even only one severe hypoglycaemic has occurred in a diabetes
12-month period, which must be reported immediately.
TABLE Diagnostic Criteria for Gestational Issues Postpregnancy

24.4 Diabetes Mellitus
• Following pregnancy the majority of women with GDM
IADPSG NICE (2015)
will no longer require any treatment.
Fasting plasma glucose ≥5.1 ≥5.6 • Women should be advised of the risk of GDM in any
(mmol/L) subsequent pregnancy, and to seek medical advice if further
1-hour glucose (mmol/L) ≥10 pregnancy is desired.
• Women should be offered testing at 6 weeks postpartum,
2-hour glucose (mmol/L) ≥8.5 ≥7.8
and annual testing thereafter to assess for underlying type
IADPSG, International Association of the Diabetes and Pregnancy Study 2 diabetes.
Groups; NICE, National Institute of Health and Care Excellence.

• Type 1 diabetes is associated with a number of adverse
foetal and maternal outcomes, including congenital mal-
with insulin improves pregnancy outcomes, including formations, preeclampsia, miscarriage, and macrosomia.
birthweight and macrosomia, and demonstrating a con- • Women with type 1 diabetes should be referred for
tinuum of risk for maternal glucose levels and adverse prepregnancy counselling and have regular checks of
pregnancy outcomes. HbA1c, aiming for HbA1c below 48 mmol/mol (6.5%)
• The optimal way to diagnose GDM, however, remains prior to conception. Women with HbA1c of greater
controversial. The most widely adopted diagnostic criteria than 86 mmol/mol (10%) should be advised against
were developed by the International Association of the pregnancy. Women with type 1 diabetes should be treated
Diabetes and Pregnancy Study Groups (IADPSG) and with high-dose folic acid, medications such as statins,
these criteria have been endorsed by various other bodies and ACE inhibitors should be stopped prior to consider-
including the American Diabetes Association (ADA). In ing pregnancy.
2015 NICE updated their guidelines with diagnostic • To help reduce the risk of preeclampsia, women should
criteria as outlined in Table 24.4. be treated with aspirin from 12 weeks of gestation.
• The majority of centres in the United Kingdom will use
these criteria; other centres, particularly in the United States, Type 2 Diabetes in Pregnancy
will use alternative criteria, including two-step approaches
using a 50-g then 100-g oral glucose tolerance test. • Increasing numbers of women are entering pregnancy in
recent times with type 2 diabetes. The safety of modern
Treatment medications such as DPP-4 inhibitors, SGLT-2 inhibitors,
and GLP-1 analogues has not been studied extensively
• Once diagnosed with GDM, women should be taught in pregnancy and these medications should be stopped
to undertake blood sugar monitoring to assess fasting prior to pregnancy.
and postprandial glycaemia and to guide treatment. Dietary • Women with type 2 diabetes should be referred for pre-
modification is the mainstay of treatment, and all women pregnancy planning; the majority will require to be
should be seen by a dietician as soon as possible after switched to insulin therapy prior to conception.
diagnosis. Other lifestyle advice, including advice on
smoking and exercise, should be offered.
• Metformin is used in the majority of women who fail to Thyroid Disease
reach their glycaemic targets with diet alone. Although
GUIDELINES
it crosses the placenta, it is thought to be safe for use in
pregnancy, with no significant effect on perinatal devel- Okosieme, O., Gilbert, J., Abraham, P., et al. (2015).
Management of primary hypothyroidism: Statement by the
opment. Approximately 50% of women treated with
British Thyroid Association Executive Committee. Clinical
metformin will require additional therapy with insulin. Endocrinology, 84, 799–808.
• Glibenclamide, a sulphonylurea, does not cross the pla- Pearce, S. H. S., Brabant, G., Duntas, L. H., Monzani, F.,
centa in significant amounts and has been used in a number Peeters, R. P., Razvi, S., & Wemeau, J. L.. (2013). ETA
of studies in pregnancy, although there has been limited guideline: Management of subclinical hypothyroidism.
European Thyroid Journal, 2, 215–228.
data on long-term pregnancy outcomes.
Ross, D. S., Burch, H. B., Cooper, D. S., et al. (2016).
• Insulin therapy is used in women who fail to reach gly- American Thyroid Association guidelines for diagnosis and
caemic targets with dietary measures or with metformin, management of hyperthyroidism and other causes of
or in those for whom metformin is not tolerated. Undi- thyrotoxicosis. Thyroid (online).
agnosed type 2 diabetes should be considered in women Perros, P., Colley, S., Boelaert, K., et al. (2014). British
Thyroid Association guidelines for the management of thyroid
with a fasting glucose of 7 mmol/L and above; these
cancer. Clinical Endocrinology, 81, 1–122.
women should be treated initially with insulin, with or
without metformin.
• Thyroid disease is common. Thyroid function tests (TFTs) • Urgent 9 am cortisol if secondary hypothyroid, or refer
are among the most frequently requested tests in primary for URGENT short synacthen test if high suspicion of
care, and an understanding of how to interpret them and glucocorticoid deficiency
what to do next is essential for daily working as a general • Full blood count (FBC) for associated anaemia and
practitioner (GP). macrocytosis
• Most laboratories will report thyroid-stimulating hormone • Urea and electrolytes (U&Es) for hyponatraemia
(TSH) and free T4 as standard, but some will include total
T4 and T3. T4 and to a lesser extent T3 are bound in Treatment
plasma by thyroid binding globulin, and it is the unbound • Give levothyroxine. In younger patients without cardiac
component that is physiologically active. T3 is more active disease, 50 µg daily is a typical starting dose. For older,
than T4. comorbid patients and those with heart disease, 25 µg
should be started.
Hypothyroidism • Repeat TFTs 6 weeks following initiation, and titrate
dose in 25- to 50-µg increments. Repeat testing and dose
• Symptoms include lethargy, weight gain, cold intolerance, escalation should not occur before this. Aim for normal
muscle cramps, slowed cognition, constipation, dry hair TSH and T4.
and skin, hoarse voice, and fluid retention. • T3 therapy is controversial and there is little evidence to
• Severely hypothyroid patients may present with: suggest greater benefit than T4, but a small number of
• myxoedematous appearance—periorbital puffiness, patients report improved symptoms.
macroglossia, thin hair; • Despite normalization of TFTs, patients may complain
• cardiac failure; of symptoms. Levothyroxine should not be increased.
• hypothermia; • Review medications as some drugs can affect absorption
• peripheral neuropathy; of thyroxine (antacids, proton pump inhibitors [PPIs],
• encephalopathy; iron).
• coma.
Referral
Primary Hypothyroidism • Most primary hypothyroidism is managed in primary
• Primary hypothyroidism is most common and results from care.
the failure of the thyroid to produce adequate thyroid • Refer:
hormone. Typically causes high TSH, low T4. a. all secondary hypothyroid;
• Causes include: b. primary hypothyroid:
• autoimmune (Hashimoto): thyroid peroxidase (TPO) • Young patients
antibody positive, usually with goitre; • High doses (i.e., >200 µg daily) where compliance
• postradioiodine therapy or thyroid surgery; is good
• iodine deficiency (endemic goitre); • Large goitre causing obstructive symptoms
• postradiotherapy to head/neck; • Severe hypothyroidism
• congenital; • Cardiac disease
• drugs: lithium, amiodarone, chemotherapy agents. • Pregnancy or within 6 months postpartum
Secondary Hypothyroidism Subclinical Hypothyroidism

• Secondary hypothyroidism is due to failure of the pituitary • High TSH but normal T4.
to produce TSH. Results in low TSH, low T4. Usually • Treatment still debated but generally accepted if:
associated with other pituitary hormone deficiencies. • TSH is above 10 mU/L;
• Causes: • repeated testing trending toward overt hypothyroidism;
• Pituitary tumours • consider if significant cardiac disease.
• Postpituitary surgery or radiotherapy • More likely to adopt a wait and see approach in extreme
• Postcranial irradiation elderly patients as TSH rises with age.
• Empty sella
• Rarer pituitary disorders (e.g., infiltrative, infarction/ Hyperthyroidism
haemorrhage)
• In secondary hypothyroidism exclude cortisol deficiency • Symptoms: flushes, anxiety, tremor, palpitations, heat
as a matter of urgency. Do not initiate thyroid replace- intolerance, loose stools, oligomenorrhoea, hair loss, eye
ment until glucocorticoid deficiency is excluded. symptoms in Graves disease.
Investigations in Primary Care Primary Hyperthyroidism

• TFTs • Suppressed TSH, high T4, and/or high T3
• TPO antibodies for primary hypothyroid • Causes:
• Graves disease: positive TSH-receptor antibodies • Propylthiouracil (PTU):

(TRAB) usually. TRAB-negative variant also with • Used in first trimester of pregnancy or if intolerant to
typical features on thyroid imaging carbimazole.
• Autoimmune (TPO antibodies) • Dose 200 to 400 mg daily in divided doses initially.
• Toxic multinodular goitre • Lower risk of agranulocytosis than carbimazole.
• Toxic nodule • Hepatitis more common.
• Thyroiditis: subacute de Quervain, postpartum • Check LFTs 4 to 6 weeks after initiating treatment
• Drug induced: amiodarone and periodically thereafter. Discontinue and seek advice
• Factitious: thyroxine abuse if abnormal.
• Very rarely thyroid cancer • Monitor TFTs every 6 to 8 weeks until euthyroid and
3-monthly thereafter on maintenance therapy.
Secondary Hyperthyroidism Radioactive Iodine
• Secondary is very rare • Given to those who relapse or show resistance to anti-
• Causes: thyroid medication.
• TSH-oma • Likely required in toxic nodular disease.
• Thyroid hormone resistance • Risk of hypothyroidism post-treatment.
• Results in high TSH, high T4/T3 • Not suitable for carers of young children or in pregnancy
due to radiation risks.
Graves Disease Thyroid Surgery
• Autoimmune thyrotoxicosis with diffuse goitre • Rarely required.
• Associated with TRAB antibodies • Likely to need levothyroxine post-surgery.
• Associated eye symptoms: • Other risks include recurrent laryngeal nerve damage,
• Gritty, dry eyes hypoparathyroidism, haemorrhage, and infection.
• Pain
• Proptosis and lid lag Referral
• Periorbital oedema • All hyperthyroidism should be referred.
• Diplopia/ophthalmoplegia • Treatment should be discussed with endocrinologist before
• Altered colour vision initiating.
• Visual loss • Patients with Graves disease who become pregnant should be
referred to endocrinology and obstetrics as early as possible.
Investigations in Primary Care • Sight-threatening Graves eye disease should be referred
• TFTs as an emergency to ophthalmology:
• TRAB and TPO antibodies • Globe subluxation
• Symptoms of corneal ulceration
Treatment • Visual loss
• Nonselective beta blockers can be used for symptom • Impaired colour vision
management.
• Do not start antithyroid treatment unless discussed with Thyroid Nodules
endocrinologist.
• Thyroiditis does not usually require antithyroid treat- • Thyroid cancer is rare (<1% of all cancers).
ment and prescribing it may precipitate profound • TFTs are usually (but not always) normal in thyroid cancer.
hypothyroidism. • Increasing concern if:
Antithyroid Drugs • rapid growth;
• Usually first line in autoimmune disease. Patients typically • larger than 1 cm;
receive 12 to 18 months of antithyroid therapy before a • lymphadenopathy.
trial off it. Less effective in other disease but may be used • All thyroid nodules should be referred.
as a holding bridge to other therapy. • Followed up at least annually by endocrinology:
• Carbimazole: • for papillary and follicular cancers, treatment aims to
• Varying doses from 10 to 40 mg daily depending on suppress TSH lifelong.
severity of thyrotoxicosis and response to treatment
• Counsel patients about the risk of agranulocytosis: Sick Euthyroid
a. Should stop therapy and seek urgent medical
attention if severe sore throat, mouth ulcers, and • Ideally, TFTs should not be checked in acute/subacute illness.
fever. • Typically inappropriately normal or suppressed TSH with
b. Full blood count should be taken to ensure white low T3/T4 but various patterns seen.
cell count normal, and if so can recommence • Any decision to commence treatment in such cases needs
treatment. monitoring closely.
Thyroid Disease in Pregnancy • Tertiary hypoadrenalism:

• Patients receiving oral glucocorticoid treatment more
• Woman with hypo- and hyperthyroidism should be referred than 2 to 3 weeks are at risk; therapy should not be
to endocrinology as soon as pregnancy confirmed. stopped abruptly.
• Woman with hypothyroidism should increase levothyroxine • Inhaled, topical, intramuscular, and intraarticular
dose by 25% to 30% as soon as pregnancy confirmed: steroids may also cause tertiary hypoadrenalism.
• Target TSH <2.5 mU/L in first trimester.
• TSH levels are normally lower in the first trimester Diagnosis
due to biochemical similarities to human chorionic • Measure serum cortisol levels before and after stimulation
gonadotropin (hCG). with synthetic ACTH (synacthen). Patients should not be
• Diagnosis and treatment of subclinical hypothyroidism taking exogenous steroids. False negative results can occur in
in pregnancy is controversial. acute secondary or tertiary hypoadrenalism (the adrenal glands
• Woman with hyperthyroidism will usually require smaller take time to atrophy and response to ACTH is preserved in
doses of antithyroid medication and may be able to stop the short term). The cutoff for diagnosis varies according to
it in pregnancy. the assay used and should be confirmed locally.
• PTU is preferred in the first trimester due to risk of • Differentiating between primary, secondary, and tertiary
teratogenicity associated with carbimazole. hypoadrenalism is based on history (drug history, pres-
• TRAB antibodies should be checked in pregnancy in ence of other autoimmune conditions, family history) and
women with a history of Graves; if positive there is a further investigations, including plasma ACTH levels,
risk of neonatal Graves disease. aldosterone and renin measurements, adrenal androgens,
• Women with Graves disease are at risk of recurrence adrenal autoantibodies, and, depending on clinical suspi-
in the postnatal period; TFTs should be checked 4 to cion, pituitary function tests or adrenal/pituitary imaging.
6 weeks after delivery. These would be undertaken by the specialist endocrine
team.
Hypoadrenalism Treatment
REVIEWS AND GUIDANCE • Glucocorticoid replacement, most commonly hydrocor-
tisone (complete form FP92A to exempt prescription
Charmandari, E., Nicolaides, N. C., & Chrousos, G. P. (2014).
Adrenal insufficiency. GP Lancet, 383, 2152–2167.
charges) in divided doses mimicking diurnal rhythm (e.g.,
Husebye, E. S., Allolio, B., Arlt, W., et al. (2014). 10 mg, 5 mg, 5 mg).
Consensus statement on the diagnosis, treatment and • Mineralocorticoid replacement only required in primary
follow-up of patients with primary adrenal insufficiency. adrenal insufficiency and is not needed if daily dose of
Journal of Internal Medicine, 275, 104–115. hydrocortisone is over 50 mg.
• Adrenal androgen replacement is not currently recom-
mended in the United Kingdom due to a lack of evidence
regarding efficacy.
• Hypoadrenalism can be primary (most commonly auto- • All patients should be educated in sickday rules:
immune or Addison disease), secondary (most commonly • Double or triple hydrocortisone dose during intercur-
hypopituitarism due to pituitary adenoma), or tertiary (most rent illnesses. Need to reduce slowly if illness persists
commonly exogenous glucocorticoid administration). for more than a few days.
• Fatigue is a presenting feature common to all aetiologies. • Seek medical assistance if vomiting or unable to toler-
Patients with primary hypoadrenalism may demonstrate ate oral hydrocortisone treatment and wear medic alert
skin pigmentation (increased ACTH levels) and symptoms bracelet/carry steroid card.
of mineralocorticoid deficiency. In secondary and tertiary • IM hydrocortisone (100 mg) for self-administration
hypoadrenalism, mineralocorticoid secretion is normal; in case of emergencies and patients should be familiar
therefore plasma potassium levels are normal. in how and when to use this.
• All aetiologies can present with a hypoadrenal crisis; • Inform medical staff promptly of steroid dependence
symptoms consist of abdominal pain, vomiting, dehydra- if they are unwell or undergoing invasive proce-
tion, and hypotension. This is a medical emergency. dures so that timely replacement treatment can be
• Primary hypoadrenalism: initiated.
• Prevalence increased in recent years, likely due to rise • Abrupt cessation of steroids can precipitate adrenal crisis;
in autoimmune hypoadrenalism. patients should always be prescribed and dispensed ade-
• More common in women than men. quate amounts of hydrocortisone to cover holiday periods
• Incidence peaks between 30 and 50 years. and sickdays.
• Secondary hypoadrenalism: • Monitoring of treatment is usually undertaken by spe-
• More common than primary hypoadrenalism. cialist endocrine team and involves assessing adequacy of
• Tends to present at a later age, around 60 years. replacement while minimizing risks of overreplacement
(hypertension, diabetes, reduced bone mineral Parathyroid Hormone Independent (Low Parathyroid
density). Hormone)
• Adrenal crisis is a life-threatening emergency that requires • Drugs: calcium supplements, thiazide diuretics, high dose
prompt treatment. vitamin A and D
• Give 100 mg hydrocortisone IM or IV and urgent • Humoral hypercalcaemia of malignancy:
referral to hospital. • PTH-related peptide secretion by tumours, particularly
breast and squamous cell lung cancers
Hypercalcaemia • Bony metastases
• Multiple myeloma
GUIDELINE • Renal failure
Bilezikian, J. P., Brandi, M. L., Eastell, R., et al. (2014). Initial Tests
Primary hyperparathyroidism: Management guidelines.
Journal of Clinical Endocrinology and Metabolism, 99, • Serum corrected calcium above 2.6 mmol/L
3561–3569. • Urea and electrolytes
• PTH
• Vitamin D
• Calcium is the most abundant mineral and has vital roles • Urinary calcium to creatinine ratio (<0.01 suggestive
in cellular function, cardiovascular stability, and bone of FHH)
health. It is under homeostatic control of parathyroid • Serum protein electrophoresis and urinary Bence Jones
hormone (PTH), with lesser roles for vitamin D and protein
magnesium. Approximately half is bound to albumin in • Further tests guided by results
blood, and it is the unbound component that is physiologi-
cally active. Levels need to be corrected to albumin con- Primary Hyperparathyroidism
centration as a result.
• Corrected calcium = measured calcium + (0.02 × [40 • Of PHPT, 85% caused by single adenoma. Remainder
– albumin]) due to parathyroid hyperplasia; less than 1% parathyroid
• Normal levels of corrected calcium are 2.2 to 2.6 mmol/L carcinoma.
• Severe hypercalcaemia is generally considered at 3 mmol/L • Common in postmenopausal woman.
and above, but severe symptoms and/or end-organ damage • Many patients have mild to moderate hypercalcaemia
at lower levels can occur. and are asymptomatic.
• Consider multiple endocrine neoplasia (MEN)
Symptoms 1 if:
• Thirst • younger than 40 years;
• Frequent urination • parathyroid hyperplasia;
• Bony pain • strong family history of PHPT;
• Nausea and vomiting • other endocrine tumours.
• Constipation
• Renal colic Treatment
• Lethargy • Rehydration. Advise patient to drink 3 L of water per day
• Confusion unless contraindicated.
• Altered mood • Loop diuretics are not indicated and may increase risk
of nephrocalcinosis.
Causes • Stop offending drugs.
Parathyroid Hormone Dependent (High Parathyroid • Parathyroidectomy if:
Hormone or Inappropriately Normal Parathyroid • younger than 50 years;
Hormone) • serum calcium above 2.9 mmol/L;
• Primary hyperparathyroidism (PHPT; common) • confirmed nephrolithiasis/nephrocalcinosis;
• Tertiary hyperparathyroidism: • kidney injury as a result of hypercalcaemia;
• Occurs following prolonged period of hypocalcaemia • osteoporosis.
• Seen in end-stage renal disease and malabsorptive • Many patients are too frail or do not meet criteria for
conditions surgery.
• Chronic vitamin D deficiency • Observe symptoms and calcium levels.
• Familial hypocalciuric hypercalcaemia (FHH): • Bone density scan every 2 to 3 years and advise weight-
• Rare defect of the calcium sensing receptor gene bearing exercise and bisphosphonate therapy where
• Autosomal dominant appropriate.
• Mild hypercalcaemia of no important sequelae • Cinacalcet 30 mg twice a day may be suggested off-
• Lithium licence.
TABLE
24.5 Causes of Hirsutism and Clinical Features
Cause of Hirsutism Associated Clinical Features

Polycystic ovary syndrome Menstrual disturbance
Long duration of symptoms
Obesity/features of insulin resistance
Congenital adrenal hyperplasia Family history of congenital adrenal hyperplasia
Ethnicity: Ashkenazi Jewish, Hispanic
Virilizing tumour (ovarian or adrenal) Rapid onset
Other signs of virilization (clitoromegaly, voice change)
Abdominal mass
Significantly elevated serum androgens
Ovarian hyperthecosis Postmenopausal onset. Often gradual but can have virilization and significantly elevated
androgens
Iatrogenic Drug history (e.g., androgens, including DHEA, cyclosporine, minoxidil, dexamethasone,
some anticonvulsants, antipsychotics, and antidepressants). Transfer of testosterone
from gels
Endocrinopathy Other relevant features of Cushing syndrome, acromegaly, thyroid dysfunction,
prolactinoma
Uncommon for hirsutism to be presenting feature
Idiopathic hyperandrogenism Hirsutism with no other clinical features and elevated serum androgens
Idiopathic hirsutism Hirsutism with no other clinical features and normal serum androgens
Hirsutism disease and a significant risk of false positives, and is not

recommended.
GUIDELINES • Serum testosterone is usually significantly elevated (twice
the normal) in malignant disease.
Martin, K. A., Chang, J., Ehrmann, D. A., et al. (2008).
Evaluation and treatment of hirsutism in premenopausal
• The laboratory measurement of testosterone is challeng-
women: An Endocrine Society clinical practice guideline. ing, partly due to variation in the sex hormone binding
Journal of Clinical Endocrinology and Metabolism, 93, globulin (SHBG) seen in a number of conditions, includ-
1105–1120. ing obesity and diabetes. Measurement of the free androgen
Arslanian, S. A., Ehrmann, D. A., et al. (2013). Diagnosis index (which takes account of SHBG) is useful, as is
and treatment of polycystic ovary syndrome: An Endocrine
Society clinical practice guideline. Legro Journal of Clinical
measurement of testosterone in specialist laboratories using
Endocrinology and Metabolism, 98, 4565–4592. tandem mass spectroscopy rather than immunoassays.
Escobar-Morreale, H. F., Carmina, E., Dewailly, D., et al. Liaising with local services for the interpretation of dif-
(2012). Epidemiology, diagnosis and management of ficult cases is advised.
hirsutism: A consensus statement by the Androgen Excess Table 24.5 lists the common causes of hirsutism and
and Polycystic Ovary Syndrome Society. Human
Reproduction Update, 18, 146–170.
associated clinical features.
• PCOS is the most common diagnosis. Diagnosis depends
of finding two of the following three features:
• Androgen excess
• Hirsutism (an excess of terminal hair growth) is common. • Ovulatory dysfunction
Most women have either no physiological abnormality • Polycystic ovaries
or benign pathology (most commonly polycystic ovary • TFTs, prolactin, and 17-hydroxyprogesterone (17OHP)
syndrome); however, there are a number of rare but impor- should be checked to screen for mimics. Synacthen-
tant conditions to consider and exclude. stimulated 17OHP is the diagnostic test for congenital
• Caused by elevated androgens, originating from adrenal or adrenal hyperplasia.
ovarian source and peripheral conversion of testosterone to
the more active compound dihydrotestosterone. This periph- Treatment
eral conversion may explain why many women are symp- • Lifestyle:
tomatic despite a serum testosterone within the normal range. • Weight loss and improved insulin sensitivity will
• Investigation of mild hirsutism with no associated clinical improve many features of PCOS, including unwanted
features has a low probability of detecting underlying hair growth.
• Metformin can be used as an insulin sensitizing agent; TABLE

however, effect on hirsutism is minimal. 24.6 Aetiology of Gynaecomastia
• Hair removal:
Nondrug Causes Drugs
• Plucking, waxing, and shaving are effective and cheap
although they must be performed frequently. Physiological (neonatal, Digoxin
• The illusion of thicker hair is caused by the shaved tip adolescence, old Spironolactone
of hair having a blunt edge. age) Cannabis
Hypogonadism Acid suppression: ranitidine,
• Laser and electrolysis treatment is more expensive but hCG-producing cimetidine, omeprazole
can be effective for up to 6 months, is most effective tumours (testis, Opiates
in light-skinned women with dark hair, and is not lung) Oestrogens
suitable for use on extensive areas of skin. Liver disease Androgen deprivation therapy
• Warn those planning to seek either of the last two Renal disease (e.g., bicalutamide; prostate
Hyperthyroidism cancer)
options to check that the practitioner is a member of Anabolic steroids
the appropriate professional body (e.g., the Institute Antiretrovirals
of Electrolysis, British Medical Laser Association). Metoclopramide, domperidone
• Pharmacological treatment:
• Pharmacological treatment should not be evaluated
for efficacy before 6 months.
• The OCP is the first line treatment. An OCP which Clinical Evaluation
contains a progestin with antiandrogenic properties • Assess for thyroid status, signs of chronic liver disease,
may be more effective (e.g., drospirenone/Marvelon or associated features of hypogonadism (loss of body hair,
or cyproterone acetate/Dianette). female pattern of fat distribution, loss of muscle mass).
• If no improvement is seen, an antiandrogen (spirono- • Testicular and abdominal examination for the presence
lactone or cyproterone acetate) can be added but OCP of a palpable mass.
must be prescribed concurrently due to risk of female • Take a full drug history including any herbal remedies
foetal virilization. and recreational drugs.
• Spironolactone (50–200 mg) can be used; may cause
hyperkalaemia, diuresis, and hypotension. Biochemical Tests
• Cyproterone acetate is generally well tolerated but there • Testosterone (early morning sample, repeat at least once if
is a risk of hepatotoxicity; LFTs should be monitored. low), luteinizing hormone (LH), hCG, alpha fetoprotein
Combination treatment in the form of Dianette carries (AFP), oestrogen, and LFTs particularly if rapid onset
a higher risk of venous thromboembolism (VTE) than with pain.
other forms of low-dose OCP. • Elevated hCG or oestrogen suggests possible neoplasm
• Finasteride inhibits 5α-reductase activity. It is generally and testicular ultrasound is indicated, proceeding to
well tolerated. imaging of adrenals if negative.
• Topical treatment (i.e., eflornithine cream) is only • Most will have normal investigations and cause is
effective in reducing hair regrowth and should only idiopathic.
be used in combination with hair removal methods.
Management
Referral • Majority of adolescent gynaecomastia will resolve without
Rapid virilization and/or significantly elevated testosterone intervention. In most adult cases removal of any contribut-
levels should be referred to endocrinology. In addition, if ing medication, reassurance, and observation is sufficient.
the diagnosis is unclear or there is no response to treatment, • Tamoxifen can be used in some cases but is rarely required.
endocrine referral is warranted.
Hypogonadism in Adult Men
Gynaecomastia
GUIDELINE
• Gynaecomastia is benign proliferation (>0.5 cm) of glan-
Bhasin, S., Cunningham, G. R., Hayes, F. J., et al. (2010).
dular breast tissue as distinct from a discrete breast mass Testosterone therapy in men with androgen deficiency
(when carcinoma must be excluded) or diffuse enlargement syndrome: An Endocrine Society clinical practice guideline.
due to adiposity and obesity. Journal of Clinical Endocrinology and Metabolism, 95,
• Usually bilateral but can be unilateral. 2536–2559.
• Normal physiological phenomenon in many boys during the
neonatal period and adolescence. It is also a common finding
in older, middle-aged men without underlying pathology.
• Can be associated with a number of endocrine and non- • The term hypogonadism refers to a reduction in sperm or
endocrine conditions as well as many drugs (Table 24.6). testosterone production. Table 24.7 lists its classifications.
TABLE
24.7 Classification of Hypogonadism
LH/FSH Testosterone Prolactin Aetiology

Primary hypogonadism Elevated Low Normal Testicular failure
Secondary hypogonadism Inappropriately normal or low Low Normal or Hypothalamic or pituitary disease
elevated
FSH, Follicle-stimulating hormone; LH, luteinizing hormone.
• Symptoms suggestive of androgen deficiency in men are Bilezikian, J. P., Brandi, M. L., Eastell, R., et al. (2014). Primary
loss of libido, erectile dysfunction, hot flashes/sweats (if hyperparathyroidism: Management guidelines. Journal of Clinical
severe and of rapid onset), and breast discomfort. Less Endocrinology and Metabolism, 99, 3561–3569.
specific symptoms include reduced energy levels, low Charmandari, E., Nicolaides, N. C., & Chrousos, G. P. (2014). Adrenal
mood, poor concentration, sleep disturbance, reduced insufficiency. GP Lancet, 383, 2152–2167.
muscle bulk, and increased body fat. Diabetes Improvement Plan. (2014). Scottish government publications.
Retrieved from www.gov.scot/Publications/2014/11/6742/downloads.
• Enquire about illnesses (e.g., mumps) or trauma that could
Diabetes UK. (2015). Facts and stats. Retrieved from www.diabetes.org.uk.
have affected the testicles and drugs that interfere with Diabetes UK. (n.d.). Diabetes risk score assessment tool. Retrieved from
testicular function/testosterone metabolism (e.g., gluco- www.diabetes.org.uk.
corticoids, opioids, and alcohol). Escobar-Morreale, H. F., Carmina, E., Dewailly, D., et al. (2012).
• Examination should include testicular size and the develop- Epidemiology, diagnosis and management of hirsutism: A consensus
ment of secondary sexual characteristics. Other physical statement by the Androgen Excess and Polycystic Ovary Syndrome
findings include a reduction in facial/body hair, small/ Society. Human Reproduction Update, 18, 146–170.
shrinking testes, incomplete/delayed sexual development, Green, J. B., Bethel, M. A., Armstrong, P. W., with the TECOS Study
inability to conceive, and height loss/low trauma fractures. Group. (2015). Effect of sitagliptin on cardiovascular outcomes in
• Biochemical assessment consists of a fasting venous serum type 2 diabetes. The New England Journal of Medicine, 373, 232–242.
sample for total testosterone between 8 and 10 am along Husebye, E. S., Allolio, B., Arlt, W., et al. (2014). Consensus statement
on the diagnosis, treatment and follow-up of patients with primary
with gonadotrophins (LH, FSH) in patients with symp-
adrenal insufficiency. Journal of Internal Medicine, 275, 104–115.
toms. A repeat sample should be taken to confirm the Legro, R. S., Arslanian, S. A., Ehrmann, D. A., et al. (2013). Diagnosis
diagnosis along with prolactin and thyroid function tests and treatment of polycystic ovary syndrome: An Endocrine Society
in secondary hypogonadism. Assessment should not be clinical practice guideline. Journal of Clinical Endocrinology and
made during acute or subacute illness. Metabolism, 98, 4565–4592.
• Refer to an endocrinologist patients with symptoms, with Marso, S. P., Bain, S. C., Consoli, A., et al. (SUSTAIN-6 investigators).
osteopenia or osteoporosis, with evidence of pituitary (2016). Semaglutide and cardiovascular outcomes in patients with
disease, or with markedly low testosterone levels from type 2 diabetes. The New England Journal of Medicine. doi:10.1056/
primary testicular failure for further investigation and a NEJMoa1607141
decision about testosterone replacement therapy. Recent Marso, S. P., Daniels, G. H., Brown-Frandsen, K., et al (LEADER trial
research suggests that raising testosterone in hypogonadal investigators). (2016). Liraglutide and cardiovascular outcomes in
type 2 diabetes. The New England Journal of Medicine, 375, 311–
symptomatic men older than 65 years of age may provide
322.
some benefit in sexual function and mood but not in Martin, K. A., Chang, J., Ehrmann, D. A., et al. (2008). Evaluation
respect of vitality or walking distance (Snyder et al., 2016). and treatment of hirsutism in premenopausal women: An Endocrine
• Conditions in which testosterone administration is associ- Society clinical practice guideline. Journal of Clinical Endocrinology
ated with a high risk of adverse outcome include prostate and Metabolism, 93, 1105–1120.
cancer, an unevaluated prostate nodule or raised PSA, Metzger, B. E., Lowe, L. P., Dyer, A. R., et al. (HAPO Study Cooperative
breast cancer, a haematocrit over 50%, severe lower urinary Research Group). (2008). Hyperglycaemia and adverse pregnancy
tract symptoms, and poorly controlled congestive heart outcome. The New England Journal of Medicine, 358, 1991–2002.
failure. National Institute for Health and Care Excellence. (2012). Type 2
diabetes: Prevention in people at high risk. NICE clinical guideline
38. Retrieved from www.nice.org.uk.
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Bhasin, S., Cunningham, G. R., Hayes, F. J., et al. (2010). Testosterone NICE clinical guideline 3. Retrieved from www.nice.org.uk.
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and Metabolism, 95, 2536–2559. 19. Retrieved from www.nice.org.uk.
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25
Persistent Physical Symptoms and
Symptoms Without Apparent Disease
CHRISTOPHER BURTON
Prevalence changes in the way the brain processes sensory informa-
Principles of Management Medically Unexplained tion and changes in the way the mind interprets symptoms.
Symptoms While in recent years, attention has focused on the mind’s
Clinical Assessment interpretation of symptoms (the cognitive approach),
History recent research suggests increasing evidence for altered
Examination (and pre-conscious) brain processing of body signals.
Investigation • This peripheral – central combination is most easily under-
Beware of Overassessing stood through the example of pain. An initial injury can
Referral trigger symptoms through peripheral mechanisms (noci-
Management ceptive pain). However in some patients the pain persists
Explanation or can spread to affect areas unrelated to the original
Specific Syndromes injury. This occurs through central sensitisation. Many
persistent physical symptoms have both peripheral and
central processes.
• In some of the functional syndromes such as irritable
bowel syndrome it is clear that for a substantial number of
• This chapter considers the common problem of physical patients there are clear peripheral factors - such as dietary
symptoms which cannot be fully explained by current intake of FODMAPs (Staudacher & Whelan 2017) and it
conceptualisations of organ disease. This includes persistent is likely that additional peripheral features will be identified
physical symptoms which appear disproportionate to over time in other syndromes. It may be helpful to think
evident organic disease processes and clusters of symptoms of them as disorders of brain-body communication. It is
in syndromes such as fibromyalgia and irritable bowel not appropriate to think of symptoms and syndromes as
syndrome. mental problems just because currently known physical
• These physical symptoms are sometime termed “functional disease has been ruled out.
somatic symptoms” (because they are characterised by • Persistent physical symptoms and emotional distress (low
changes in function rather than structure) or problems mood and anxiety) often co-occur (Henningsen, Zim-
of “central sensitisation”. The term medically unexplained merman & Sattel 2003). However it is clear that emotional
symptoms is still in common usage but is discouraged: distress is neither necessary nor sufficient to cause persistent
it implies that such symptoms cannot be understood or physical symptoms.
are in some way different from other symptoms. Neither • There are many reasons for the interaction between symp-
of these is true. If used at all, the term medically unex- toms and emotional distress. Symptoms are inherently
plained symptoms (MUS) should be reserved for symptoms distressing and physical symptoms predispose patients to
of relatively recent onset in which there is no immediately anxiety and depression. In a reciprocal way, susceptibility
apparent organic cause. Even then it may be better to to anxiety or depression also predisposes patients to develop
think of them as “symptoms without apparent disease”. persistent physical symptoms. Despite the fact that these
• Symptoms can be understood as having both peripheral interactions are common, many patients with symptoms
and central components. Peripheral components include will not have anxiety or depressive disorders.
tissue damage or inflammation and altered function (e.g. • Persistent physical symptoms may occur as a single symp-
smooth muscle spasm). Central components include toms (e.g. dizziness), as one or more defined syndromes
426
CHAPTER 25 Persistent Physical Symptoms and Symptoms Without Apparent Disease 427
(e.g. tension type headache, fibromyalgia or irritable • Around half of patients referred to specialists because of
bowel syndrome) or in a less specific pattern of multiple symptoms turn out to have no organic disease (Nimnuan,
symptoms, in multiple body systems on multiple Hotopf, & Wessely, 2001). Many meet the criteria for
occasions. The syndromes are sometimes referred to as one of the functional somatic syndromes.
Functional Somatic Syndromes. There are a number of • At least 2% of adults have persistent physical symptoms
classifications of multiple symptoms and systems although (McGorm, Burton, Weller, Murray & Sharpe 2010).
none is widely used in general practice. These include Patients generally seek care in an episodic rather than
Somatic Symptom Disorder in DSM5 (which emphasises continuous fashion but often end up with a number of
the presence of psychological or social features in addi- different investigations or specialist referrals as each
tion to physical symptoms) and Bodily Distress Disorder symptom is taken seriously and appropriately worked up.
which emphasises the diversity of symptoms and body Most patients with persistent physical symptom are not
systems involved. immediately identified by general practice teams. As a
• Table 25.1 lists a range of disorders currently considered prompt, it may be useful to think of patients presenting
broadly within the spectrum of functional somatic multiple symptoms in multiple body systems on multiple
syndromes occasions.
• Two situations arise in which patients may deliberately • A small number of patients (around 2 per thousand) have
present nonorganic symptoms: severely disabling persistent physical symptoms. They are
• Malingering, in which symptoms are fabricated for often high users of healthcare and some will be known
personal gain to every General Practice. They may have had extensive
• Factitious disorder, in which the symptoms appear to specialist referrals with negative results and may be seen
have no direct gain other than access to health care as facing, and presenting, multiple challenges.
(formerly known as Munchausen syndrome) • It is important to remember that some symptoms without
These two conditions will not be considered here. apparent disease are actually early or subtle symptoms of
a disease which becomes apparent over time. Occasionally
Prevalence this includes serious conditions such as cancer. Where a
specialist has made an appropriate assessment of symptoms
• In patients seeing a GP between 15 and 30% will have which have been present for several months, this is uncom-
at least one symptom without apparent disease. Patients mon (occurring in 1-5% of patients). However with recent
with only an occasional consultation for such symptoms symptoms in primary care it may be more common and
have a low risk of the symptoms becoming persistent so GPs should always consider the possibility of organic
(Verhaak, Meijer, Visser & Wolters, 2006). However when disease alongside the probability of a functional symptom.
symptoms have been present for many months, persistence
- or the resolution of one symptom but the occurrence
of another - is more common. Principles of Management Medically
Unexplained Symptoms
TABLE • Symptoms without apparent disease are common and
25.1 Common Symptom Disorders by Specialty
when first presented it may be difficult to rule out physical
Cardiology: chest pain with normal coronary arteries, disease. They may occur in isolation, as part of a picture
palpitations of persistent physical symptoms, or as a feature of a func-
Ear, nose, throat: dizziness, globus pharyngis, functional tional somatic syndrome.
dysphonia • Principles of assessment are broadly similar across the
Gastroenterology: functional dyspepsia, irritable bowel spectrum of severity, although strategies for each severity
syndrome, proctalgia fugax group will differ. The approach outlined here can be used
consistently across different symptoms, although the spe-
Gynaecology: chronic pelvic pain
cific questions, examinations, and investigations will vary
Maxillofacial: facial pain, temporomandibular joint by symptom.
dysfunction
Musculoskeletal: chronic widespread pain, fibromyalgia
Clinical Assessment
Neurology: nonepileptic attacks, functional weakness,
tension-type headache
History
• Take a careful history, actively listen:
Respiratory: unexplained breathlessness/hyperventilation
• Many functional symptoms have characteristic patterns:
Urology: chronic genital/prostatic pain the unchanging nature of tension-type headache, the
Generalized: chronic fatigue syndrome, multiple chemical bloating and relation to meals of irritable bowel syn-
sensitivity drome (IBS). The absence of typical features of func-
tional syndromes should raise concern.
• Sometimes patients will volunteer a symptom which • In making the decision whether to investigate further,
is much more suggestive of pathology, so check for the following principles can be useful:
red flags but try to listen first. • Functional symptoms do not cause abnormal results
• Many patients with new or persistent symptoms will on screening tests—if these occur and are nontrivial,
have specific concerns about cause: Active listening then follow them up with more tests or referral.
encourages patients that disclosing concerns or distress • Normal results of diagnostic tests produce an immedi-
is part of a normal consultation. In contrast, “Do you ate sense of relief in patients but this is only temporary.
think stress could be causing this?” is often seen as Repeated testing and relief may lead to an escalating
threatening (Peters et al., 2009) and is best avoided. cycle of reassurance seeking.
• By actively listening and responding to the story, for • Patient satisfaction and symptom resolution isn’t altered
instance “That must have felt terrible,” you convey by whether investigations are conducted immediately
empathy and give the patient the legitimate opportunity or deferred.
to describe the emotional aspect of his or her symptoms. • If a plausible (non-pathological) explanation for symp-
• You earn yourself the opportunity to make a judgment toms is given before the investigation, a negative result
or recommendations based on having collected sufficient is likely to lead to greater reassurance than if it is
information—you can demonstrate this by summarising withheld until after (or not given at all).
the history back to the patient and ensuring that what • GPs request more investigations than patients actually
he or she has described and you have heard matches up. want. Sometimes tests are ordered as the only thing one
can offer; often patients don’t want more tests so much
Examination as either a reasonable explanation of what is causing their
• A new symptom or a change in an old one usually war- symptoms (rather than what they don’t have) or some
rants examination. understanding of and support for their attempts to cope.
• Introduce the examination positively: “I would like to
properly examine your…” rather than “… take a quick Beware of Overassessing
look at… .” The patient has to believe you when you find • It usually doesn’t make sense to repeat investigations where
nothing abnormal. nothing much has changed.
• Consider anticipating normal findings: “I think this is • When tests have all been normal and a reasonable special-
X, in which case the examination will be normal, but I ist opinion (sometimes more than one) has concluded
need to check that.” Then when you find nothing, that there is no serious cause, it is unlikely that a new one
is no surprise. will find the missing problem.
• Use the break in the consultation between history and • It is essential to remember that incidental and clinically
examination to ask the patient if he or she has any ideas unimportant findings are common (e.g., unexpected and
or concerns about what might be causing the symptoms. clinically irrelevant lesions will be found on 5%–10% of
You won’t have eye contact so it is a less threatening point cranial MRI scans).
in the consultation, and you can specifically direct the
examination to areas of concern. Referral
• Talk the patient through key points of your examination, • Specialists are good at recognizing disease but most have
either in advance (“I’m going to check your abdomen for no more skills in managing functional symptoms than
any lumps or swellings”) or afterwards (“The backs of their GP colleagues. Multidisciplinary pain services are
your eyes look perfect, I was checking to see if the pres- of value for some patients.
sure in your brain is normal, and it is”). • Specialist teams for managing functional symptoms are
• Use tests that indicate functional signs as explanation currently rare; liaison psychiatry and psychology services
rather than to prove there’s nothing wrong. (If you can may be able to assess and offer cognitive behavioural
identify pain on light touch [allodynia] or increased sen- therapy (CBT) or other psychologic therapies.
sitivity to pain [hyperalgesia] by pinching the skin of the • Outcomes for patients with complex persistent physical
abdominal wall, then demonstrate them as part of the symptoms who engage in treatment can be good, though
“proof ” of your explanation.) Similarly, if you can dem- not all patients benefit.
onstrate normal movement in one situation and not in
another (and you don’t think the patient is malingering) Management
then use that to point out that the body can do this, but
the brain is sometimes inhibiting it. • There is no specific medical treatments for persistent
physical symptoms so most management is generic and
Investigation may include explanation, (e.g. low dose tricyclic for chronic
• Investigations have an important role in patients with pain) selective prescribing, and the use of simple cognitive
new or changing symptoms. Doctors find them reassuring or behavioural techniques.
and if anxious patients lead to anxious doctors, it is under- • None of these have been formally tested in primary care,
standable that we turn to them. but they are in keeping with more general principles of
CHAPTER 25 Persistent Physical Symptoms and Symptoms Without Apparent Disease 429
management and also on recognition that the previously • BOX 25.1 Explanations
recommended approach of reattribution is unlikely to be
beneficial. Example 1: Functional Dyspepsia
You keep describing these stomach cramps but the endoscopy
was normal. That tells me there is nothing wrong with the
Explanation structure of your stomach, no disease, or ulcer, or infection….
We often find this, that from time to time the stomach doesn’t
• Brief interventions in primary care to reattribute physical work properly, it churns around or cramps up, giving the sort of
symptoms to emotional states are unlikely to be effective symptoms you describe. This is a problem with the way your
and are actively resisted by patients (Rosendal et al., 2013). stomach functions (called functional dyspepsia) rather than a
• Many doctors tell patients what they don’t have, but then sign of any disease. Treatment often helps to smooth that
function out, but it’s important for you to know that even if this
fail to provide an explanation for what is (or could be) functional dyspepsia happens it can’t cause you further harm.
causing their symptoms.
• Studies indicate that effective explanations should make Example 2: Dizziness
sense to both the patient and doctor, should not convey We have tested your balance and it works fine (this might have
blame, and should lead to therapeutic action (Dowrick, included Dix Hallpyke test and a stepping test) but when we did
Ring, Humphris, & Salmon, 2004). those tests, even though your balance performed normally, your
brain told you things weren’t okay. This condition is called
• Explanations may be brief, but also need to be flexible; and functional dizziness (or disequilibrium).
explanation for a relatively new symptom can be straightfor- What it means is that although your brain is getting the right
ward, for a simplistic explanation for a symptom that has signals from your inner ears, these signals are then setting off
been going on for a long time may be counterproductive. false alarms. False alarms are quite common and normal;
• Box 25.1 contains two examples of explanations. Both sometimes they occur after an episode of illness and sometimes
for no good reason at all. Unfortunately, what happens is that
include a plausible explanation, are blame free, and outline the more they bother you, the more your brain looks out for
therapeutic action and partnership. them, and then the more they happen… . What is important
• Neither of these explanations in Box 25.1 include ques- here is that you find a way to trust your own balance more (we
tions about stress, anxiety, or depression. However, if have seen that it works) and rely on these unsteadiness alarms
the patient raised them, they could certainly be fit in. in your brain less (because they’re false alarms).
There are some techniques called vestibular rehabilitation
If issues or concerns have been handled gently during which help and they are available from (… a range of websites or
the consultation, sometimes a plausible positive explain the United Kingdom from the Meniere’s Disease Foundation). I
nation gives the patient the opportunity to volunteer want you to look at these and start practicing them, and I will see
his or her own concern (for instance, with the dizziness you again in 4 weeks to see how you are starting to recover.
explanation: “That’s a relief, and there was me worry-
ing I had a brain tumour”) in a lightly self-deprecating
fashion.
• Care for patients with persistent physical symptoms GPs can become confident in making these diagnoses,
includes management of depressive and anxiety disorders following appropriate diagnostic criteria (see chapter 11 for
where these are identified, but should be as part of a Fibromyalgia and chapter 9 for IBS). In the case of Chronic
wider formulation. Fatigue there are a number of different sets of criteria, however
all include persistent and disabling fatigue for more than 6
Specific Syndromes months. The presence of severe fatigue following effort (for
instance on the next day) is seen as particularly important
Several clinical syndromes are currently thought to involve by some groups. Further details about diagnosis and recom-
problems with the generation and persistence of symptoms mendations for treatment are to be found in the NICE
more than primary peripheral pathology. These include fibro- Clinical Guideline 53, although GPs should be aware that
myalgia, Irritable Bowel Syndrome and ME/CFS (Myalgic the guidelines are contested by some patient groups. The
Encephalomyelitis /Chronic Fatigue Syndrome). A fuller list current edition of the guideline was published in 2007 and
is provided in Table 25.1. at the time of writing (2019) it is in revision.
These are increasingly recognised as complex disorders
with multiple body and brain processes. In each syndrome
the balance of importance of different processes (e.g. inflam- References
matory, endocrine, perceptual) is currently still being resolved
Dowrick, C. F., Ring, A., Humphris, G. M., & Salmon, P. (2004).
– and it is likely that it varies between individuals and sub-
Normalisation of unexplained symptoms by general practitioners: A
types. Classification of these syndromes continues to evolve. functional typology.
In some cases this can become an emotive topic, and it is Henningsen, P., Zimmermann, T., & Sattel, H. (2003). Medically
important to discuss the complex nature of these syndromes unexplained physical symptoms, anxiety, and depression: A meta-
with patients (who may otherwise surmise that you think analytic review.
that they are imagining their symptoms or bringing them McGorm, K., Burton, C., Weller, D., Murray, G., & Sharpe, M.
on by faulty thinking). (2010). Patients repeatedly referred to secondary care with symptoms
unexplained by organic disease: Prevalence, characteristics and refer- Rosendal, M., Blankenstein, A. H., Fink, P., Sharpe, M., Morriss, R.,
ral pattern. & Burton, C. (2013). Enhanced care by generalists for functional
Nimnuan, C., Hotopf, M., & Wessely, S. (2001). Medically unexplained somatic symptoms and disorders in primary care. Cochrane Database
symptoms: An epidemiological study in seven specialities. of Systematic Reviews. Accepted for Publication.
Peters, S., Rogers, A., Salmon, P., Gask, L., Dowrick, C., Towey, M., Staudacher, & Whelan 2017, The low FODMAP diet: recent advances
et al. (2009). What do patients choose to tell their doctors? Qualitative in understanding its mechanisms and efficacy in IBS.
analysis of potential barriers to reattributing medically unexplained Verhaak, P. F., Meijer, S. A., Visser, A. P., & Wolters, G. (2006). Persistent
symptoms. presentation of medically unexplained symptoms in general practice.
26
Palliative Care and Care of
the Dying Patient
BEN DIETSCH
Basic Principles Pain—Neuropathic
Emergencies Presenting Features
Management
Metastatic Spinal Cord Compression (MSCC) and Cauda
Equina Syndrome Pain—Other Types
Presenting Features Malignant Bone Pain
Management Smooth Muscle Spasm
Skeletal Muscle Spasm
Superior Vena Cava Obstruction
Tenesmus
Presenting Features
Management Pain—Total Pain
Definition
Hypercalcaemia of Malignancy
Presenting Features
Presenting Features
Management
Management
Pain—Nonpharmacologic Interventions
Seizures/Status Epilepticus
Options
Presenting Features
Management Pain—Interventional Techniques
Presenting Features
Opioid Toxicity
Management
Presenting Features
Management Respiratory
Breathlessness
Haemorrhage (Massive)
Cough
Risk Factors
Other Symptoms
Management
Gastrointestinal Symptoms
Irreversible Airway Obstruction/Acute Respiratory Distress
Nausea, Vomiting, and Regurgitation
Risk Factors/Causes
Constipation
Management
Diarrhoea
Agitation (Terminal Agitation) Acid Reflux, Gastritis, Oesophagitis
Presenting Features Hiccup
Management Ascites
Symptom Management Guidelines Anorexia, Cachexia, Dysphagia
Pain—General Considerations Malignant Bowel Obstruction
Assessment of Pain Oral Symptoms
Management—General Principles Oral Candidiasis
Use of Medications Off Label Mouth Ulcers and Oral Stomatitis
Opioid Side Effects Xerostomia
Tolerance, Dependence, Addiction in Patients Taking Opioids Sialorrhoea
Patient Information Neurological Symptoms (See Also “Advanced Neurological
Legal Implications—Drugs and Driving, Travel Abroad Diseases”)
Pain—Nociceptive Seizures
Presenting Features Intracerebral Malignancy
Management—Nonopioids Fatigue
Management—Opioids Delirium
431
Chronic Confusion End-Stage Renal Failure

Management Symptoms
Skin Symptoms Indicators of Poor Prognosis (Being in the Final Months to
Lymphoedema and Oedema Year of Life) in Advanced and End-Stage Renal Failure
Sweating (Paraneoplastic) (Chronic Kidney Disease 4 or 5)
Pruritus Pharmacological Considerations in Chronic Kidney Disease
Malignant Wound Management (Bleeding From Wounds, 4 and 5
Odour and Infection, Fistulae) General Considerations
Depression and Anxiety Symptom Management Specific to Advanced
Depression Chronic Kidney Disease/End-Stage Renal Failure
Anxiety Medications Used to Manage Advanced
Bleeding Chronic Kidney Disease/End-Stage Renal Failure (and Its
Special Notes on Steroids Complications)
Indications for and Use of Steroids (Dexamethasone) Managing Medications Used in Advanced
Monitoring Patients Taking Corticosteroids Chronic Kidney Disease/End-Stage Renal Failure
Stopping/Withdrawing Steroids Management of Symptoms Specific to Advanced
Management of New Onset Steroid-Induced Diabetes Chronic Kidney Disease/End-Stage Renal Failure
Care in the Last Days of Life End-Stage Respiratory Disease
Prognostication Indicators of Poor Prognosis (Being in the Final Months to
Stopping Treatments Year of Life) in Advanced Respiratory Disease
Medications Symptom Management Specific to End-Stage Respiratory
Nutrition and Hydration Disease
Noninvasive Ventilation Oxygen in End-Stage Respiratory Disease
Anticipatory Medications and Care Anticipating Care Needs and Management
Syringe Driver End-Stage Heart Failure

Symptoms
Indications and Practicalities
Indicators of Poor Prognosis (Being in the Final Months to
Starting Doses Year of Life) in Advanced Heart Failure
Advanced Neurological Disease Symptom Management Specific to End-Stage Heart Failure
General Problems Medications Used to Manage Heart Failure (and Its
Communication Symptoms)
Nutrition, Hydration, and Poor Swallow Managing Cardiac Medications to Improve Symptoms in
Recurrent Infection Advanced End-Stage Heart Failure (Table 26.17)
Muscle Spasms/Spasticity (See Also Section on Pain) Secondary Prevention
Activities of Daily Living Pharmacological Considerations in End-Stage Heart Failure
Dementia Medications to Avoid
Management of Behaviour That Challenges Managing Cardiac Devices
Management of Reduced Oral Intake and Infection (See Types of Implantable Cardiovascular Implanted Electronic
Earlier Discussion - 'Nutrition, Hydration & Poor swallow') Devices
Parkinson’s Disease and Parkinson Plus Syndromes Deactivation of Defibrillator Component of Devices at End
Motor Neurone Disease (MND)/Amyotrophic Lateral of Life
Sclerosis ALS Practicalities of Defibrillator Deactivation
Ventilatory Support Deactivation of Other Pacemaker Function at End of Life
Cough After Death
• Palliative care seeks to optimize the quality of living

Basic Principles for those with advanced, incurable life-limiting illness
(LLI). As such, the approach addresses physical, psy-
GUIDELINES chological, social, and spiritual needs as identified by
Scottish Palliative Care Guidelines. www. the patient.
palliativecareguidelines.scot.nhs.uk/. • National Institute for Health and Care Excellence (NICE)
R Twycross, A Wilcock, P Howard. (2017). Palliative care defined end-of-life care as that provided in the last year
formulary (6th ed.). City: Publisher. www.palliativedrugs.com of life; but palliative care can encompass a much longer
Ltd. period spent living with life-limiting illness.
M Watson, C Lucas, A Hoy, I Back. (2009). Oxford
handbook of palliative care. Cambridge: Oxford University • When referring to care of the dying patient, the author has
Press. in mind those with a likely prognosis of days to a week.
• Good management and decision making in palliative and
end-of-life care require careful assessment of the
CHAPTER 26 Palliative Care and Care of the Dying Patient 433
presenting problem or symptom, an appreciation of the • Symptoms suggestive of spinal metastases (spinal
context of the person’s underlying life-limiting illness, an pain which is severe and unremitting, aggravated by
understanding of the person’s wishes, and considered straining, local spinal tenderness, nocturnal spinal
communication to draw these strands together. pain preventing sleep) AND neurological symptoms
• Planning for symptom control and end-of-life care needs including radicular pain, weakness in any limb, dif-
is crucial to the patient with life-limiting illness. ficulty in walking, sensory loss, or bladder or bowel
• The outcome of any discussions and decisions should be dysfunction.
recorded clearly and in ways which make it readily acces- • Consider cauda equina compression in any patient with
sible to other healthcare professionals that may be involved advanced malignancy and the following neurological signs
in the person’s care (e.g., on the patient’s summary care and symptoms:
record). • Symptoms suggestive of spinal metastases in the lower
• Patients with capacity should be fully involved in deci- spine AND low back pain, saddle anaesthesia/paraesthesia,
sions about treatment and encouraged to consider and bowel or bladder dysfunction, and weakness in one or
document Advance Care Plans (ACP), including: both legs.
• Advance Decisions to Refuse Treatment (ADRT), Do Not
Attempt Cardiopulmonary Resuscitation (DNACPR) Management
orders and Treatment Escalation Plans (TEPs); • Patients unlikely to benefit from further investigation/treatment.
• Advance Statements describing what they would ideally Circumstances in which further imaging/treatment may
like to happen at the end of life (e.g., preferred place not be appropriate include:
of care or death, wishes around hospital admission or • patients whose spinal metastases have previously been
care at home); deemed untreatable; or
• what should happen if they develop an intercurrent illness; • those who are in the last days to week(s) of life
• who they would want included in any communication (i.e., whose overall clinical condition means they are
or decisions involving their treatment and care, includ- unsuitable for surgical intervention and not likely to
ing Lasting Power of Attorney (LPA). live long enough to benefit from radiotherapy); and/
• If or when capacity to make specific decisions is lost, any or
information recorded in advance will assist best interest • those who have capacity to refuse investigation.
decision making. a. Patients in these circumstances should not be admit-
• Recognizing the context of the presenting problems helps ted to hospital.
to determine appropriate management options (i.e., what b. Management of these patients may include a trial of
is likely to be successful or not). For instance, some prob- dexamethasone 16 mg orally (daily dose; usually admin-
lems may be the natural mode of death: respiratory failure istered as 8 mg twice daily), provision of adequate anal-
(in the context of advanced motor neurone disease), neu- gesia, and provision for care needs and equipment.
tropenic sepsis (in advanced myelosuppression/neutropenia • Patients with potential to benefit from investigation and
due to extensive bone metastases or refractory haemato- treatment. This is anyone not fulfilling the above criteria,
logical malignancy), lower respiratory tract infection (in including those with established MSCC for whom inter-
the context of advanced respiratory disease), anorexia/ vention may be appropriate for pain relief, if not to
cachexia (in the context of advanced dementia). improve function.
• Contact the local MSCC coordinator to arrange assess-
ment and further management (surgery or radiotherapy):
Emergencies a. immediately (often via acute oncology hospital
Metastatic Spinal Cord Compression (MSCC) service) if the patient has neurological signs and
symptoms suggestive of MSCC;
and Cauda Equina Syndrome b. urgently (within 24 hours) to discuss the care of
GUIDELINE patients with cancer and any symptoms suggestive
of spinal metastases.
National Institute for Health and Care Excellence. (2008). • In practice, when MSCC is suspected and further
Metastatic spinal cord compression in adults: Risk
assessment, diagnosis and management. NICE clinical imaging desired/appropriate:
guideline 75. www.nice.org.uk/guidance/cg75. 1. then urgent hospital admission for whole spine MRI
will be required; and
2. dexamethasone 16 mg daily PO should be commenced.
Superior Vena Cava Obstruction

Presenting Features
• Consider metastatic spinal cord and cauda equina Com- Presenting Features
pression (MSCC) in any patient with advanced metastatic • Patients with known diagnosis of malignancy and
malignancy and the following signs and symptoms: lymphadenopathy.
GUIDELINE some patients can be symptomatic with a rise of any-

thing above normal.
Scottish Palliative Care Guidelines. (n.d.). SVCO. www.
palliativecareguidelines.scot.nhs.uk/guidelines/palliative- Management
emergencies/Superior-Vena-Cava-Obstruction.aspx.
• Confirm corrected calcium and renal function.
• In the palliative setting where treatment of underlying malig-
nancy is no longer appropriate, intravenous (IV) rehydration
• SVCO is most frequently due to extrinsic compression and bisphosphonates (most usually pamidronate 90 mg or
by carcinoma of the lung, lymphoma, and other cancers zoledronic acid 4 mg) are the mainstay of treatment:
or thrombosis formation. • Pamidronate can be given subcutaneously via hyper-
• Signs and symptoms: dermacolysis (e.g., 90 mg pamidronate in 1 L normal
• Breathlessness, headache, dizziness, feeling of fullness saline over 12 to 24 hours).
in head are common symptoms. • Hypercalcaemia can become refractory to treatment and
• Signs include oedema of conjunctivae, face, hands, and/ may be a mode of death. In a moribund patient with
or arm(s); dilated veins in the neck; dilated collateral other biochemical abnormalities/irreversible pathologies,
veins in the arms and chest wall; stridor and cyanosis. not treating may be appropriate.
• Onset may be acute (often when due to thrombosis) or chronic.
Management Seizures/Status Epilepticus

• Dexamethasone 16 mg daily, orally to reduce peritumour GUIDELINE
oedema
R Twycross, A Wilcock, P Howard. (2017). Palliative care
• Oxygen if available formulary (6th ed.). City: Publisher. www.palliativedrugs.com Ltd.
• Urgent hospital admission: for radiological investigation;
definitive management is often anticancer therapy (includ-
ing radiotherapy/chemotherapy), thrombolysis, or endo-
vascular stent. Presenting Features
• In patients with advanced malignancy, supportive symp- • Seizures can result in patients with primary or secondary
tomatic management with opioids and benzodiazepines brain tumour(s): they may be the initial presentation of
should also be given (see “Symptom Management Guide- these pathologies.
lines > Respiratory > Breathlessness”). • They may also be a consequence of preexisting epilepsy
• When hospital admission for further investigation/ (especially when a person becomes unable to take oral
management is not appropriate or desired, dexamethasone antiepileptic medication), other structural brain lesions
and oxygen can be tried for symptom benefit (as above) (e.g., multiple sclerosis), metabolic abnormalities arising
and focus should be on pharmacologic management of in advanced incurable disease, or alcohol withdrawal.
associated symptoms/distress. • Seizures may be of any type but in patients with intra-
cerebral malignancy they are often focal with subsequent
Hypercalcaemia of Malignancy secondary generalization.
GUIDELINE
Management
• Acute emergency where further hospital management is con-
Scottish Palliative Care Guidelines. Hypercalcaemia. www.
palliativecareguidelines.scot.nhs.uk/guidelines/palliative-
sidered appropriate/desirable.
emergencies/Hypercalcaemia.aspx. a. Exclude hypoglycaemia.
b. Hospitalization for further investigation may be
indicated especially if this is the first seizure from an
unknown cause or when seizure becomes prolonged.
c. Diazepam 10 mg per rectum or midazolam 10 mg
Presenting Features buccal/subcutaneous (SC)/intramuscular (IM) (repeated
• Patients may have known (lytic) bone metastases, but not after 30 minutes if necessary).
always. • When hospital admission is not appropriate (e.g., at end-
• Malignancies most often associated with malignant hyper- of-life or terminal event):
calcaemia are: myeloma, lung, breast, renal, and thyroid. • Control acute seizure
• Signs and symptoms (often worsen over days): a. Diazepam 10 mg per rectum or midazolam 10 mg
• Common: malaise, weakness, nausea, constipation, buccal/SC/IM.
polyuria This can be repeated every 20 to 30 minutes
• Severe: delirium, vomiting, seizure, coma until seizure activity is controlled.
• Corrected calcium (adjusted for albumin) often needs b. If ineffective, consider phenobarbital IM, up to
to be greater than 3 mmol/L to be symptomatic but 10 mg/kg (seek specialist advice).
• Prevent further seizures—ensure regular antiseizure 4. If SaO2 less than 90% give supplemental oxygen and
medication. attempt bag-valve-mask ventilation.
a. Continuous subcutaneous infusion (CSCI) mid- 5. If no response to 2 to 4 mg naloxone consider alternative
azolam 20 to 30 mg/24 h. Plus as needed (PRN) diagnosis (e.g., other sedative, neurologic event, sepsis).
midazolam 5 to 10 mg SC/IM 1-hourly. CSCI dose 6. NB buprenorphine will require higher doses.
can be titrated every 24 hours according to PRN • Is there respiratory depression?
midazolam use; OR Respiratory rate less than 8 breaths/min.
b. CSCI phenobarbital 200 mg CSCI/24 h. CSCI 1. Titrate partial reversal. Naloxone 100 µg (IV/SC: dilute
dose can be titrated every 24 hours according to to 4 mL with 0.9% NaCl). Give 1 mL (100 µg).
PRN use (see below). 2. Repeat every 2 minutes until respiration restored to
• If seizures remain poorly controlled despite 50 mg or avoid complete reversal of analgesia.
more of midazolam CSCI, add phenobarbital 200 mg 3. Try other conservative methods to stimulate respiratory
CSCI/24 h. This can be titrated by 100 mg daily to rate (i.e., oxygen, bag-valve-mask).
400 mg CSCI/24 h. 4. Administer 100 µg (1 mL) every 2 minutes until the patient’s
• Longer term prevention: see “Symptom Management respiratory status is satisfactory and sustained above 8.
Guidelines > Neurological Symptoms > Seizures.” 5. It is important to titrate against respiratory rate, not
level of consciousness, as total antagonism will cause
Opioid Toxicity return of severe pain with hyperalgesia and physical
withdrawal/agitation.
GUIDELINE 6. Further boluses may be necessary if respiratory rate
drops below 8 because naloxone is shorter acting than
Electronic Medicines Compendium. www.medicines.org.uk/
emc/medicine/21095. many opioids; consider continuous SC infusion.
7. If SaO2 is less than 90% give supplemental oxygen,
consider bag-valve-mask.
8. Consider if transfer to hospital may be appropriate.
Presenting Features • NO respiratory depression:
• Signs and symptoms: Consider opioid toxicity if the patient • Monitor respiratory rate, level of consciousness, oxygen
has experienced: saturation, blood pressure (BP), pulse initially every
• a drug error (e.g., inadvertent dose increase) or rapid 15 to 30 minutes.
escalation of opioid;
• unexplained drowsiness or deterioration in any patient
Haemorrhage (Massive)
receiving an opioid;
• confusion/hallucinations, myoclonic jerks, drowsiness, Risk Factors
pinpoint pupils; • Tumour adjacent to/invading large blood vessels (e.g.,
• reduced respiratory rate. head and neck cancers, bronchogenic carcinoma)
• Important considerations include: • Disorders of coagulation (e.g., thrombocytopenia, dis-
• management is dictated by level of respiratory depression; seminated intravascular coagulation [DIC])
• full reversal is not always necessary and can precipitate • Sometimes (but not always), the patient will experience
acute withdrawal reaction and pain; warning bleeding (e.g., minor haemoptysis)
• normal oxygen saturation does not exclude respiratory
depression—reduced respiratory rate occurs first, result- Management
ing in raised carbon dioxide levels. Low O2 saturations • Preparing the patient/family for this possibility is important.
(SaO2) are a late sign; • Midazolam 10 mg PRN should be available to administer
• if the drug has been given recently, the patient’s condi- in the event of distress caused by massive haemorrhage.
tion may continue to deteriorate as the drug is absorbed; This can be given as IM injection as cutaneous circulation
• naloxone is removed from the body more quickly than many will often be reduced.
opioids. Repeat doses or hospitalization may be required.
Management Irreversible Airway Obstruction/Acute

• Is there imminently life-threatening respiratory depression?
Respiratory Distress
Respiratory rate less than 4 breaths/min AND semi- Risk Factors/Causes
conscious or unconscious. • Patients with tumour encasing or involving a large airway
1. Give full reversal. Naxalone 400 µg (IV: dilute to 4 mL (e.g., trachea or bronchus):
with 0.9% NaCl OR SC: use undiluted). • Tumour may be overgrowing a tracheostomy or causing
2. Repeat every 2 minutes until respiration restored. intrinsic/extrinsic pressure on a large airway such as a
3. Consider transfer to acute hospital, call 999 as assisting thyroid malignancy or bronchial carcinoma.
ventilation via bag-valve-mask may be required. • Massive pulmonary embolism (PE)
• Advanced respiratory disease (e.g., COPD, pulmonary e. If agitation/distress persist despite the above:
fibrosis) • PRN: levomepromazine 12.5 to 25 mg 4-hourly
• Advanced heart failure PRN SC
• Motor neurone disease • Regular: switch CSCI to a combination of
levomepromazine (25–150 mg CSCI) and mid-
Management azolam (20–100 mg CSCI)
• High-dose benzodiazepines are often required to manage f. If above ineffective, seek specialist advice.
respiratory and psychological distress (e.g., midazolam
5–10 mg SC/IM).
• Opioids may also be helpful (morphine 2.5–10 mg Symptom Management Guidelines
SC if opioid naïve or an appropriate PRN dose if Pain—General Considerations
already taking opioids—see “Symptom Management
Guidelines > Respiratory > Breathlessness”). GUIDELINES
• Both benzodiazepines and opioids may be required CSCI
if obstruction happens over the course of hours/days. National Institute for Health and Care Excellence. (n.d.).
Opioids for pain relief in palliative care. NICE pathways.
https://pathways.nice.org.uk/pathways/
Agitation (Terminal Agitation) opioids-for-pain-relief-in-palliative-care.
British Pain Society. For various professional and patient
Presenting Features information/guidelines and pain scale, see www.
• See also “Symptom Management Guidelines > Neurologi- britishpainsociety.org/british-pain-society-publications/
patient-publications/.
cal Symptoms > Delirium.” World Health Organisation. (n.d.). WHO’s cancer pain ladder
• Agitation at the end of life is often a combination of delirium, for adults. www.who.int/cancer/palliative/painladder/en/
extreme physiological symptoms, and psychological distress in Medications and Healthcare Products Regulatory Agency.
response to an irreversible problem that is leading to death. (2009). Off label or unlicensed use of medication: Prescribers’
• Terminal agitation is often a diagnosis made in retrospect, responsibilities. www.gov.uk/drug-safety-update/
off-label-or-unlicensed-use-of-medicines-prescribers-
but can be heralded by: responsibilities.
• rapid escalation in physical symptoms which is not responding
to management (e.g., intractable pain or breathlessness);
• rapid escalation in psychological distress;
• worsening delirium. Assessment of Pain
• Pain is a common problem in advanced Life Limiting
Management Illness (LLI): Its presence should be identified and its
• It is crucial to recognize and manage agitation as quickly management reviewed regularly.
as possible: sedation may be most appropriate manage- • Pain may be due to:
ment in this situation. • underlying LLI;
• The patient and those important to him/her will require • treatment for disease (e.g., radiotherapy);
support and explanation. • comorbidities and debility (e.g., arthritis, ulcers).
• Sedatives/anxiolytics should be administered PRN as fre- • Various means of scoring pain are available. Patients able
quently as is necessary until the patient is settled, and to communicate can often provide a vivid description
continued CSCI. and rating using a visual analogue scale.
• Sedative/anxiolytics choice should be titrated in propor- • For those unable to articulate or verbalize (e.g., in severe
tion to distress/agitation: learning disability or advanced dementia), other pain scales
a. Patient anxious/frightened but lucid: are helpful such as the Abbey Pain Scale.
• explore fears • Breakthrough and incident pain:
• PRN: lorazepam 0.5 mg PO 4-hourly OR mid- • Breakthrough pain is a spontaneous and unpredictable
azolam 2.5 to 5 mg half-hourly SC increase in pain in a patient whose background pain
b. Patient has continuous/worsening anxiety: is otherwise well controlled.
• Regular: diazepam 2 mg twice daily (titrated to 5 mg • Incident pain is an increase in pain associated with an
twice daily) OR midazolam 10 mg CSCI/24 h identifiable trigger such as movement.
• Increase midazolam CSCI in steps of 30% to 50%
(up to 100 mg); continue midazolam PRN Management—General Principles
c. Patient confused/agitated/hallucinating: • Consideration of the underlying aetiology (e.g., nocicep-
• PRN and regular: haloperidol 2.5 mg SC stat + tive, neuropathic, mixed, acute/chronic) will guide initial
5 mg CSCI/24 h + haloperidol PRN management. Start at the lowest effective analgesic dose
d. If worsening confusion/agitation: and titrate gradually.
• Increase haloperidol to 10 mg CSCI/24 h + halo- • In addition to pains caused directly by the LLI, pain may be
peridol PRN functional or due to concurrent problems. As such, approaches
• Consider adding midazolam, as above other than those used palliatively may be required.
• The most appropriate analgesia should be given:

• regularly, at the right dose, by the right route, at the Tolerance, Dependence, Addiction in
right time/frequency; and Patients Taking Opioids
• a suitable analgesic should be available PRN to treat • Increasing pain in patients with LLI is often due to disease
breakthrough or incident pain. progression; however, there is increasing recognition of
• The World Health Organisation’s pain ladder has been tolerance, dependence, and even addiction.
used for many years to guide management of cancer pain. • This may be due to the ongoing use of opioids in patients
• If the nature of the pain is unclear, try simple analgesia with increasing life expectancy made possible by newer
and non-pharmacological approaches before moving on palliative therapies in oncology, for example.
to opioids and adjuvant analgesia. • Consider potential for these problems in patients with
• Consider patient ability to take and self-administer medi- LLI but prognosis of months to years.
cation. Various preparations of analgesia are available to • Tolerance (requiring increasing doses of analgesia to achieve
enable administration by mouth, TD patch, enteral tube, the same reduction in pain) may respond to switching
or CSCI. between opioids; for example, switch from morphine to
• Review the efficacy of analgesia frequently (daily if neces- oxycodone.
sary) and consider giving patients scope to escalate their • Dependence is seen in patients taking long-term opioids,
own analgesia. If analgesia proves ineffective after esca- but is rarely a problem so long as they have sufficient
lation or has burdensome side effects, be prepared to prescribed medication.
stop it. • Addiction is rare, but may be preexisting: if so, it deserves
• Consider underlying causes which might be treatable: consideration when making prescribing arrangements for
• Management of constipation or urinary retention strong opioids (and other addictive medication). It may
• Spinal metastases/impending metastatic spinal cord lead to tolerance of opioids.
compression
• Painful bone metastases that may respond to radiotherapy Patient Information
• Surgical fixation of pathologic fractures • Provide information to patients when starting strong
• Infection around tumour (often increases pain in head opioids, including information on potential side effects
and neck tumours) and who to speak with for further advice.
• If the source of pain has been managed/treated, be • Ensure they are confident with how/when/why to take
prepared to reduce analgesia (e.g. metastasis treated with their analgesia, dosing, what they should take regularly,
palliative radiotherapy). and when required (PRN). Local palliative care services
• See sections on end-stage neurological disease, renal and will often have patient information leaflets, or these can
heart failure for specific guidance on analgesia in these be found online.
contexts.
Legal Implications—Drugs and Driving,
Travel Abroad
Use of Medications Off Label Drugs and Driving
• In palliative care, many medications are used off label • Patients driving while taking strong medication should
(i.e., outside the terms of the license/marketing authoriza- be made aware that:
tion, such as amitriptyline for neuropathic pain). • side effects from prescribed medications may affect
• This should be explained to patients when starting these their ability to drive;
medications. • ultimately, patients themselves are responsible for
making the assessment on their ability to drive safely:
they should not drive if they feel there may be any
Opioid Side Effects impairment in their ability to do so;
• When starting a regular opioid, anticipate common side • the law on drugs and driving (see www.gov.uk/drug-
effects such as constipation, dry mouth, drowsiness/ driving-law) states that it is illegal to drive if either:
sedation, and nausea. a. you’re unfit to do so because you’re on legal or
• Consider starting: illegal drugs; OR
• a regular laxative (docusate sodium 100–200 mg twice b. you have certain levels of illegal drugs in your blood
daily +/− senna 7.5 mg at night); (even if they haven’t affected your driving).
• an antiemetic (haloperidol 1 mg at night/cyclizine For this second point, there is a statutory “medical
50 mg three times daily). This may only be required defence” to protect those patients who may test posi-
for a week or so, and can then be stopped. tive for certain specified drugs taken in accordance
• Explain to patient that tolerance to any drowsiness/sedation with the advice of a healthcare professional. They
normally develops. must also be fit to drive when taking these medica-
• To combat dry mouth, ensure good oral hygiene, encour- tions. It may therefore be helpful for patients to keep
age sips of fluid to keep mouth moist, and/or use saliva some suitable documentation with them when they
replacements. are driving that provides evidence that they are taking
the controlled drug as a medicine prescribed or sup- (PPI) or ranitidine (see notes on NSAIDs in renal and
plied by a healthcare professional. cardiac disease).
• Naproxen 250 to 500 mg PO twice daily is often the
Travelling Abroad NSAID of first choice.
• When travelling abroad, patients taking prescribed medi- • Topical NSAIDs are proven to be effective in gel
cations (in particular controlled drugs) will need to con- formulation.
sider any laws governing their use, import, and export in
the country from which they are leaving and any Management—Opioids
country(ies) to/through which they are travelling. • Opioids remain the mainstay of managing acute nocicep-
• The UK Home Office recommends patients carry a cover- tive pain in patients with life-limiting illness.
ing letter from their doctor/prescriber as supporting evi- • Strong opioids in small doses are often preferred to
dence that the medication they are carrying was prescribed weak opioids, as they come in various preparations;
for their use. This is sufficient for up to a 3-month supply dosing is flexible; they provide scope for titration
of Schedule 2, 3, or 4 (parts I & II) controlled drugs without having to switch opioid; they may have fewer
for personal use. It will also permit them to take more side effects.
than 100 mL of liquid medication and essential medical • When using opioids, as with any analgesia, consider:
equipment in their hand luggage. The letter should state: • right analgesia: Most people tolerate morphine so it is
• patient name, date of birth, and address; appropriate to begin with this;
• the intended destination(s) and dates of outward/return • right dose: Start with lowest possible dose in an opioid-
travel; naïve individual;
• names, forms, doses, strengths, and total amounts of • route: If possible, begin with oral opioid to allow more
medications being carried. accurate dose titration. If oral route unavailable, con-
• Medication should be kept in original packaging. sider low-dose TD or CSCI;
• If travelling for 3 months or more and carrying 3 months • right frequency: Oral opioids are prescribed on a regular
or more of Schedule 2, 3, or 4 controlled drugs for per- basis, with additional PRN dose available for break-
sonal use into/out of the United Kingdom, a personal through pain;
import/export license is required (available at gov.uk). • review: Be prepared to increase dose if analgesia is
• The requirements for prescriptions and controlled drug suboptimal or stop if no benefit.
import/export for all countries in which the patient will • Anticipate common side effects (see previous discussion).
pass through customs must also be fulfilled. Patients should • When possible, use only one opioid at a time; there is
be advised to check requirements with the relevant embas- no benefit in combining weak and strong opioids or two
sies or consulates and customs procedures before travelling. strong opioids.
• When given by injection, subcutaneous (SC) is preferred
Pain—Nociceptive to intramuscular (IM) or intravenous (IV).
• Prescribers should be aware of the potential for opioid-
GUIDELINES induced hyperalgesia. This manifests where (often rapid)
National Institute for Health and Care Excellence. (n.d.). NICE
opioid escalation leads to increasing pain which is often
pathways: Opioids for pain relief in palliative care. https:// more diffuse and less well defined than the presenting
pathways.nice.org.uk/pathways/opioids-for-pain-relief-in pain. Patients will exhibit hypersensitivity in the form of
-palliative-care. hyperalgesia and/or allodynia. Management requires reduc-
World Health Organization. (n.d.). WHO’s cancer pain tion in opioid dose or switching to an alternative (and
ladder. www.who.int/cancer/palliative/painladder/en/.
reduction in equivalent dose).
• See Appendix 33 for comparative doses of different opioids.
• The following opioids are used most often:
Presenting Features • Codeine
• Nociceptive pain is associated with tissue damage. It is a. Most often used for moderate pain, often in com-
often well localized and described as sharp or dull in nature. bination formulation with paracetamol.
Synonyms include physiological or inflammatory pain. b. Starting dose: 30 mg four times daily to 60 mg
• It will normally correlate with an area of known physical four times daily.
injury or disease. c. Maximum dose 240 mg daily is equivalent to
approximately 20 mg oral morphine in 24 hours.
Management—Nonopioids • Tramadol
• Simple analgesia such as paracetamol and nonsteroidal a. Use for moderate to severe pain.
antiinflammatory drugs (NSAIDs) can be effective. b. Has additional nonopioid analgesic effects.
• When using NSAIDs: c. Starting dose: 50 to 100 mg four times daily.
• chose those with fewest side effects and consider gastric d. Maximum dose 400 mg daily is equivalent to approxi-
protection in the form of a proton pump inhibitor mately 40 to 80 mg oral morphine in 24 hours.
• Morphine 3. Most often used second line for patients taking

a. First choice strong opioid for moderate or severe morphine who experience opioid neurotoxicity
pain. and suboptimal analgesia.
b. Preparations include immediate release (tablets, oral b. PRN oral dose for breakthrough pain should be 1/6
solution, injection) and modified release (tablets, of the 24-hour oral dose; PRN dose for SC injection
capsules, granules - normally 12-hourly modified should be 1/6 of the total 24-hour CSCI dose.
release) formulations. c. Starting dose:
c. Can be given CSCI when patients are unable to 1. Estimates of potencies vary, but safe to consider
swallow. Morphine PO:SC potency ratio is 1 : 2, so it twice as potent as morphine (i.e., oral morphine
starting dose CSCI should be half the total 24-hour to oral oxycodone potency ratio is 1 : 2.)
oral morphine dose. 2. Based on this, it is usual practice to halve the
d. PRN oral dose for breakthrough pain should be 1/6 previous morphine dose when switching to oxy-
of the 24-hour oral dose; PRN dose for SC injection codone. e.g. morphine PO 30 mg in 24 hrs =
should be 1/6 of the total 24-hour CSCI dose. PRN oxycodone PO 15 mg in 24 hrs.
dose is normally prescribed 4-hourly. 3. If oxycodone is the first opioid used, start with
e. Starting dose: 1 to 2 mg 4-hourly and PRN PO.
1. Opioid naïve patient: morphine immediate release d. Titration:
2.5 to 5 mg PO 4-hourly regularly and PRN, 1. Use same principle as for morphine.
OR morphine 12-hourly modified release 10 mg e. As with morphine, it comes in a variety of formula-
twice daily plus morphine immediate release 2.5 tions and oxycodone PO:SC potency ratio is approxi-
to 5 mg PO 4-hourly PRN mately 1 : 2.
2. Patient on weak opioid (e.g., codeine 240 mg • Diamorphine
daily): morphine immediate release 5 mg 4-hourly a. Indications:
and PRN, OR morphine 12-hourly modified 1. Useful for moderate to severe pain if SC/CSCI
release 10 mg twice daily plus 5 mg immediate administration is required.
release PO 4-hourly PRN b. Available as powder for solution for injection in
f. Titration: Aim to establish lowest effective regular ampoules—it can be reconstituted to the desired
(modified release dose) as soon as possible to provide volume. This is helpful in minimizing volume of
even analgesia over 24 hours. SC injections (or CSCI infusion) for patients requir-
1. Ask patient to complete daily record of all mor- ing large doses of opioid.
phine used. c. Oral morphine: SC diamorphine potency ratio is
2. After 2 to 3 days calculate average daily (24-hour) 1 : 3. E.g., morphine 30 mg PO in 24 hrs = dia-
total morphine dose required. morphine 10 mg CSCI/24 h
3. Divided total 24-hour dose by 2 and give this as a d. Diamorphine PRN dose (SC) should be 1/6 of the
twice daily 12-hourly modified-release preparation. total daily diamorphine CSCI dose.
4. Provide PO PRN dose for breakthrough pain, • Fentanyl (TD patch)
equivalent to 1/6 of the oral 24-hour dose. a. Indicated for chronic pain management in:
For example, patient uses 6 × 5 mg immediate 1. patients who are unable to swallow or prefer not
release morphine doses in 24 hours. Total 24 to take oral medications;
hours oral morphine dose = 30 mg. 2. patients experiencing intolerable side effects
12-hourly modified-release morphine dose = from morphine (especially constipation, nausea/
15 mg twice daily. vomiting, hallucinations);
Oral PRN dose = 5 mg immediate release mor- 3. situations where there is risk of tablet diversion/
phine PO 4-hourly. misuse;
For example, patient taking 30-mg modified- 4. situations where it is safer, in severe renal
release morphine twice daily. In addition, uses impairment/failure (glomerular filtration rate
four 10-mg PRN doses every day for a week. [GFR] <30%).
Total 24-hour oral morphine dose = 30 mg b. For patients with prognosis of months (due to time
+ 30 mg + 40 mg = 100 mg. required for titration).
Increase 12-hourly modified-release morphine c. Considered less constipating than morphine or
to 50 mg twice daily. oxycodone—reduce laxative dose if switching from
Increase oral PRN dose to 15 mg immediate these opioids.
release morphine PO 4-hourly. d. TD dose is expressed in micrograms per hour. Small-
• Oxycodone est dose is 12 µg/h. This is equivalent to at least
a. Indications: 30 mg oral morphine in 24 hours.
1. Useful for moderate to severe pain. e. Equivalences to morphine/potencies vary, but Appendix
2. Used if morphine is contraindicated or not tolerated. 33 draws on several sources when estimating
equivalence. TD absorption may be influenced by f. Morphine and other mu-receptor agonists at an

skin temperature, circulation, and patch adherence. appropriate dose (buprenorphine TD 5 µg/h is
f. Starting dose: equivalent to approximately 12 mg morphine
1. Should not be used on opioid naïve patients PO in 24 hours) may be used for breakthrough
given strength of dose. pain.
2. Often more practical to titrate patient on alter- g. Titration:
native PO/CSCI strong opioid first, and when 1. If over a period of several days, three or more
satisfactory analgesia achieved, to convert this PRN doses of breakthrough opioid analgesia are
to equivalent fentanyl TD patch. being used daily, increase buprenorphine TD
3. NB: When switching patient from morphine or patch strength.
oxycodone to fentanyl, a temporary partial opioid 2. Increase TD patch dose weekly—this makes it
withdrawal reaction can occur as fentanyl is pre- unsuitable for acute pain where rapid titration
dominantly centrally acting: Gastric flulike signs is necessary.
of opioid withdrawal can occur (e.g., shivering, • Other opioids are encountered less frequently.
sweating, diarrhoea). These can be managed by • Fentanyl (immediate release transmucosal)
giving a PRN dose of the previous opioid. a. Indications:
g. Titration: 1. Various preparations of transmucosal fentanyl
1. Patches are reapplied to a fresh site every 3 days and are now available, including sublingual and buccal
may take 3 days or so to reach steady-state plasma tablets, lozenge, and nasal spray.
concentrations and maximum therapeutic benefit. 2. Transmucosal fentanyl products are designed to
2. Morphine or oxycodone PRN should be pre- provide rapid onset pain relief for a short dura-
scribed for breakthrough pain if the patient does tion which better fits the profile of breakthrough
not have severe renal failure. pain or incident pain.
3. If three or more PRN doses of breakthrough b. It is thought best practice to use PO strong opioids
opioid analgesia are required every day, the first line for breakthrough pain and then switch to
strength of the next patch applied can be increased transmucosal fentanyl only if the patient experiences
by between 12 and 25 µg/h. prolonged undesirable effects or onset of action is
4. Increase TD patch no more often than every too slow.
3 days (preferably weekly), giving patch time c. Immediate release fentanyl should be prescribed on
to reach maximum therapeutic benefit between the recommendation of a specialist in palliative care
increases. It is therefore unsuitable for acute pain or MDT. Starting dose/titration:
requiring rapid titration of analgesia. 1. The manufacturers of the various products publish
• Buprenorphine (transdermal patch) guidelines on their use and titration.
a. Indications: 2. They should not be used in opioid naïve patients
Patch helpful in same patient groups as fentanyl. or acute noncancer pain.
b. Given partial agonist and antagonist action of • Fentanyl (SC/CSCI)
buprenorphine on opioid receptors, there may be a. Indications:
less tendency toward tolerance. 1. Pain in severe/end-stage renal failure eGFR less
c. Reports of potency compared to other strong opioids than 30 mL/min
vary: The Appendix 33 has a cautious conversion, 2. When rapid titration of analgesia is required
considering fentanyl TD 1.4 times more potent (often at end of life)
than buprenorphine TD. b. Starting dose:
d. Available in lower strength doses than fentanyl 1. Opioid naïve: 12.5 to 25 µg 1-hourly PRN SC;
transdermal patches, so more scope for titration. 100 µg CSCI/24 h.
Patch strengths of 5, 10, 15, or 20 µg/h are avail- 2. Switching from other opioids: see Appendix 33.
able as 7-day patches; 33, 52.5, and 70 µg/h patches (Fentanyl is considered approximately 100 times
can be changed every 3 to 4 days. more potent than morphine.)
e. Starting dose: c. Titration:
1. Opioid naïve patient: start at lowest dose (i.e., 1. On established fentanyl CSCI dose: use PRN
5 µg/h), changed every 7 days. SC dose (1-hourly) equivalent to 1/8 to 1/10 of
2. Switching from another strong opioid: fentanyl 24-hour CSCI dose.
Use cautious conversion ratio based on current 2. As with other strong opioids CSCI, if three or
opioid dose (see Appendix 32). more PRN doses are required in 24 h, increase
Switching from another strong opioid to trans- CSCI by the equivalent amount:
dermal buprenorphine may cause opioid with- For example, fentanyl 100 µg CSCI/24 h back-
drawal (gastric flulike symptoms), which can ground analgesia plus 4 × 12.5 µg SC PRN in
be managed by PRN doses of the previous 24 hours: increase CSCI fentanyl to 150 µg
opioid. CSCI/24 h.
• Alfentanil (SC/CSCI) GUIDELINE

a. Indications:
1. Pain in severe/end-stage renal failure eGFR less
Neuropathic pain. NICE clinical guideline 173. www.nice
than 30 mL/min .org.uk.
2. Where morphine neurotoxicity develops
3. Where volume required prevents use of fentanyl
4. Note: Alfentanil’s duration of action is too short
to be useful as a SC PRN analgesic. Use fentanyl (although it may be associated with a particular sensory
for PRN dosing. dermatomal distribution).
b. Starting dose: • Abnormal sensation in the region of a pain is often a
1. Switching from other opioids: see Appendix 33 good indicator of neuropathic aetiology.
(Alfentanil is considered approximately 30 times • If it becomes chronic, indicators of chronic regional pain
more potent than oral morphine.) syndrome may develop (e.g., autonomic features with
2. SC PRN dose of alternative opioid should be changes in skin/hair/nails, oedema, and motor function).
available (author recommends fentanyl).
c. Titration: Management
1. As with other strong opioids CSCI, if three or • Opioids and NSAIDs may sometimes be effective at man-
more PRN doses are required in 24 hours, aging neuropathic pain but specific neuropathic analgesics
increase CSCI by the equivalent amount. are often required. These are a diverse group, including:
• Methadone • antidepressants;
a. Methadone is an opioid with mixed properties • antiepileptics;
including mu-opioid receptor agonist and NMDA • anaesthetics (local: lidocaine; systemic: ketamine).
receptor channel blocker. • Indications for neuropathic analgesia:
b. It has a highly variable plasma half-life and should • Monotherapy for neuropathic pain alone, OR
only be used under specialist supervision for analgesia. • Neuropathic pain unresponsive to opioid plus NSAID
c. Indications: • Choice:
1. In palliative care, it is most often used when pain • The most commonly used for long-term manage-
fails to respond to more conventional analgesia ment are amitriptyline, duloxetine, gabapentin, and
(i.e., regular opioids and nonopioids) and adjuvant pregabalin.
(e.g., severe, mixed nociceptive/neuropathic pain). • Both antidepressants and antiepileptics have been shown
2. It can be used in end-stage renal failure. to have similar tolerability and efficacy in neuropathic
d. Starting dose: pain.
1. Switching from other opioids: This process is • Carbamazepine is normally used only for trigeminal
complicated, usually requiring supervision in a neuralgia.
hospice inpatient unit. • Others such as nortriptyline, sodium valproate, and
2. Ultimately, a regular dose is often administered ketamine are usually started by a specialist.
twice daily. • Steroids (dexamethasone 8 mg) can be used short
e. Titration: term (maximum 2 weeks) for pain due to nerve com-
1. PRN dose is most often 1/6 to 1/10 of the total pression, while waiting for neuropathic analgesia or
24-hour oral methadone dose. This should be definitive treatment (e.g., radiotherapy) to have an
given no more frequently than every 3 hours effect.
(unlike other opioids). • It is usual practice to start with either an antidepressant
2. Patients requiring additional analgesia within 3 or antiepileptic:
hours of a methadone dose may be prescribed a. If initial drug poorly tolerated/ineffective, switch
the following PRN: nonopioid or previous opioid to a drug from the other category.
PRN (at half the previous dose). b. If no benefit, try combination of antidepressant
3. Increases in regular dose should be undertaken and antiepileptic.
no more frequently than once a week, due to • Commonly used neuropathic analgesics:
the accumulation of methadone. • Amitriptyline (tricyclic antidepressant—serotonin and
norepinephrine reuptake inhibitor [SNRI])
a. Its sedative side effect can be useful in patients whose
Pain—Neuropathic pain causes difficulty sleeping.
b. Benefit in neuropathic pain often seen in suban-
Presenting Features tidepressant doses.
• Neuropathic pain results from a lesion/disease of the c. Starting dose: 10 mg PO at night.
sensory nervous system. d. Titration: Can be increased weekly, often done
• Its character is often described as burning, shooting, pins stepwise from 10 to 25 mg then by 25 mg weekly
and needles, or numbness and is usually poorly localized if required. Maximum dose 150 mg.
• Duloxetine (SNRI) Pain—Other Types

a. Similar efficacy and side effect profile to amitriptyline
b. Starting dose: 30 mg daily Malignant Bone Pain
c. Titration: Increase after 1 week to 60 mg. Can be • Presenting Features
increased by further 30 mg weekly. Maximum dose • Pain is well localized to bony skeleton in region of
120 mg. known metastatic disease.
• Gabapentin • Management
a. Generally preferred over pregabalin. • Radiotherapy: appropriate in patient with prognosis of
b. Starting dose: typically 300 mg daily (100 mg three months as benefit can take 2 to 3 months. Pain flare
times daily or 300 mg once daily). Requires dose may occur shortly after treatment and will require
reduction in renal failure. additional analgesia. Analgesics may need to be reduced
c. Titration: Increase by 300 mg daily every 3 to 7 if successful pain relief is achieved by radiotherapy.
days. Benefit usually experienced by 600 mg three • Intravenous bisphosphonates have a role in treating
times daily, but maximum recommended dose is painful metastatic disease.
1200 mg three times daily. • Surgery: for painful osteolytic metastases at risk of frac-
• Pregabalin ture (or following pathological fracture) in patient fit
a. Advantage over gabapentin is twice daily dosing enough to undergo surgical intervention.
(rather than three times), but no significant analgesic • Analgesia:
benefit. a. NSAIDs and strong opioids are most commonly
b. Starting dose: 75 mg twice daily; requires dose used (see earlier).
reduction in renal failure. b. Steroids may provide short-term relief while titrating
c. Titration: Increase weekly by 150 mg daily in divided other analgesia but should not be used for more
doses (i.e., to 150 mg twice daily then 225 mg than 2 weeks.
twice daily, if required). Maximum dose 300 mg
twice daily. Smooth Muscle Spasm
• Other neuropathic analgesics (used under specialist • Presenting features
guidance) • Spasmodic pain in oesophagus, bowel (including
• Steroids rectum), or bladder
a. May be helpful as a short course in managing pain • Management
due to metastatic spinal cord compression or nerve • Oesophageal spasm: nitrates (glycerol trinitrate [GTN])
root compression while definitive treatment awaited or nifedipine 5 mg three times daily PO (titrated to
or to manage pain flare following radiotherapy or 20 mg TDS);
where no radiotherapy/surgery is possible. • Bowel spasm: hyoscine butylbromide 20 mg PO/SC
• Nortriptyline (NRI) 4-hourly PRN or QDS
a. Alternative to amitriptyline • Bladder spasm: oxybutynin 5 mg PO twice daily
• Ketamine (titrated to 5 mg PO QDS)
a. NMDA-receptor channel blocker
b. Used in neuropathic pain unresponsive to other Skeletal Muscle Spasm
measures • Presenting Features
c. Potential for urinary tract, hepatobiliary, and neu- • Painful chronic spasm associated with nerve injury in
ropsychiatric side effects, so most often given as a advanced neurologic disease or malignancy
short course • Management (see also “Advanced Neurological Diseases”)
d. May be given as CSCI (100–500 mg CSCI/24 h) • Baclofen, tizanidine, dantrolene, and diazepam all have
or regular oral dose (10–100 mg four times daily). similar efficacy. Start with baclofen and use diazepam
• Methadone only if short course (<1 month).
a. May be used in complex mixed neuropathic/ a. Baclofen: starting dose of 5 mg can be given once, twice
nociceptive pain (see Pain - Nociceptive > Manage- or three times daily (maximum starting dose 5 mg three
ment - Opioids earlier). times daily). Titration: increase daily dose by 5 mg to
• Lidocaine plasters 15 g (in divided doses) every week to maximum 100 mg
a. Increasingly used to treat localized neuropathic daily. Withdraw slowly over 2 weeks if discontinuing.
pain, but data on efficacy are poor. Mechanism of b. Tizanidine: staring dose 2 mg once daily. Titration:
effect is unclear and may owe a significant part to increase by 2 mg every 4 days (divided doses).
placebo. Maximum dose 9 mg four times a day.
b. Consider only if other neuropathic analgesics have c. Dantrolene: starting dose 25 mg once daily. Titra-
failed or are contradicted. tion: increase daily dose by 25 mg once a week and
c. If used, benefit over a 2-week period should be give as divided doses. Maximum 100 mg four times
assessed and plasters stopped if no benefit seen. a day. Monitor liver function (LFTs).
d. Diazepam: dose 2 to 5 mg at night and PRN for

maximum 4 weeks. Options
• Radiotherapy for painful metastases
Tenesmus • Splints for fractured bones or braces for spinal stabilization
• Presenting Features • TENS: useful for localized neuropathic pain
• Painful sensation of rectal fullness, often due to local • Physiotherapy: especially useful for pain associated with
tumour. immobility or muscle spasm/contractures
• Management • Complimentary therapies: evidence for these is inconsis-
• Manage aggravating factors such as constipation. tent, but they may well address components of total pain
• Use NSAIDs, amitriptyline, nifedipine, or antiepileptics (see earlier); for example: acupuncture, reflexology, aro-
(doses as detailed earlier: see Pain - neuropathic and matherapy, art and music therapies.
Pain - other types > smooth muscle spasm).
Pain—Interventional Techniques
Pain—Total Pain Presenting Features
GUIDELINE • Pain resistant to systemic analgesia or where effectiveness
of analgesia limited by side effects.
Twycross, R. (2003). Introducing palliative care (4th ed.).
London: Radcliffe Medical Press.
• Pain limited to one region or clear source (e.g., compres-
sion of specific nerve[s] by malignant tumour).
Management
Definition • Consider referring patients for specialist advice if any of
• Total pain is the concept that pain includes many con- the following techniques may be helpful:
tributing factors, notably: • Cordotomy: for pain in one side (hemithorax), such as
• biological: disease, treatment, comorbidities, fatigue; that caused by mesothelioma.
• psychological: anxiety/depression, fear, experiences, • Neurolysis: indicated in patients with limited prog-
change in body image; nosis when pain fails to respond to other measures
• social: loss of role, job, change in relationships, isolation; and a clear cause/pathway is evident. Effectiveness
• spiritual: faith, loss, search for meaning, fear of unknown. and tolerability are assessed with local anaesthetic
before proceeding to neurolytic block. Regions that
Presenting Features can be targeted include coeliac plexus (often impli-
• Patients will often identify the factors that contribute to cated in pancreatic/upper GI pain); superior hypo-
the pain (but not always and may need help exploring gastric (pelvic pain); ganglion of Impar (perineal
these). pain).
• Pain resistant to management with analgesics often has • Neuraxial (intrathecal and epidural): Indications include
a total component. intolerable neuropathic pain especially with sympa-
• It may manifest as overwhelming pain and/or be dispro- thetically mediated component. When systemic anal-
portionate to the degree of pain expected from known gesia is ineffective or limited by side effects. The
disease. challenge with this technique is provision of ongoing
specialist monitoring and management in the com-
Management munity as this needs to be undertaken by experienced
• Consider holistic, individualized approaches to the man- specialist teams.
agement of pain (i.e., choose management options address-
ing the contributing factors as identified by the patient): Respiratory
• Analgesics as for nociceptive/neuropathic pain. But be pre-
pared to reduce/stop analgesics if no benefit experienced. PATIENT RESOURCES
• Helping the patient achieve sleep at night can be a
Breathlessness Intervention Service, Cambridge University
particularly helpful initial step. Hospitals. www.cuh.nhs.uk/breathlessness-intervention-
• Other approaches: physiotherapy, transcutaneous elec- service-bis/resources/patient-information-leaflets.
trical nerve stimulation (TENS), complimentary thera-
pies, counselling, management of anxiety/depression,
addressing social issues, distraction, relaxation.
Breathlessness
Pain—Nonpharmacologic Interventions
• Breathlessness is a very common symptom in palliative
• Consider nonpharmacologic interventions where possible care—in patients with malignant, respiratory, and neu-
or when patients are reluctant to try analgesia. rological disease.
• Consider if there is a potentially reversible problem with non-harmacological therapies initially, while severe breath-
an appropriate treatment acceptable to the patient lessness in patients with days-weeks will likely rely more
(Table 26.1). As with all decisions in palliative care, con- on opioids and benzodiazepines (Fig. 26.1).
sider options for management in the context of the life- • Local palliative care centres may have specific breathless-
limiting illness and disease trajectory. ness clinics.
• Where the underlying cause of dyspnoea cannot be treated • Management options include:
or when patient wishes, prognosis, or performance status a. Education
make it inappropriate to do so, management should be • Improving patient understanding of the physiology
aimed at palliating symptoms. of breathing/breathlessness and providing tech-
• Mainstays of management are nonpharmacological thera- niques to optimize breathing can be very helpful.
pies, opioids, and benzodiazepines. Anxiolytic antidepres- A useful resource is the Breathing, Thinking, Func-
sants may also have a role in patients who have sufficient tioning (BTF) approach designed at Cambridge
prognosis to benefit. University Hospitals Breathlessness Intervention
• Choice of palliative management option will depend Service.
largely on estimated prognosis: Breathlessness in patients • Focus on improving breathing technique, using
expected to live months-years should use predominantly positions to ease breathlessness, changing thought
TABLE
26.1 Potentially Reversible Causes of Dyspnoea and Management Options
Cause Potential Management

Large airway obstruction Stent, laser/cryotherapy
Small airway obstruction Optimize bronchodilator dose and delivery: salbutamol and
ipratropium nebulizer, oral corticosteroids
Pulmonary embolus Anticoagulation: low-molecular-weight heparins
Pulmonary oedema Diuretics
Pulmonary fibrosis Optimize treatment, oral corticosteroids
Pleural effusion Pleural drain (temporary or indwelling PleurX drain), pleurodesis
Ascites Paracentesis
Pain (in chest wall/pleura or elsewhere) Analgesia
Infection Antibiotics
Radiation pneumonitis (consequent to radiotherapy) Dexamethasone (8 mg daily)
Anaemia Blood transfusion
Anxiety Beta blocker/lorazepam
Acute Type 2 respiratory failure (in acute exacerbation of Non-Invasive Ventilation (NIV) if decompensated respiratory
COPD) acidosis
Superior vena cava obstruction Radiotherapy, stent
Months prognosis
Weeks prognosis
Correct reversible cause
Education (BTF) Days prognosis
Fan As ‘Months’, plus:
Physiotherapy Morphine PO (Immediate or
Antidepressant (SSRI) Morphine SC/CSCI
Modified Release) Lorazepam PO,
Oxygen if hypoxaemic Consider Lorazepam/ Midazolam SC/CSCI
diazepam if anxiety
• Fig. 26.1 Summary: management options for breathlessness according to likely prognosis. BTF, Breathing,
thinking, functioning; CSCI, continuous subcutaneous infusion; SC, subcutaneous; SSRI, selective serotonin
reuptake inhibitor.
processes around breathlessness, and maintaining d. Antidepressants

function and efficient use of energy. • Selective serotonin reuptake inhibitors (SSRIs) may
• CBT and relaxation are also helpful. benefit breathless patients, especially where anxiety
• Teaching patients simple techniques for recovering is a contributing factor (e.g., sertraline, dosed as
control over their breathing when they feel particu- for anxiety/depression).
larly breathlessness is valuable. For example, the e. Opioids
use of a fan, sitting forward, and focusing on breath- • Reduce sensation of breathlessness and respiratory
ing out (rather than in). effort through reducing response to hypercapnia,
b. Physiotherapy hypoxia, and exercise.
• Maintaining activity helps maintain function, • Morphine is first choice strong opioid; but if side
prevent muscle wasting and deconditioning. effects limit dose, oxycodone is an alternative and
• Physiotherapists are also crucial in optimizing effi- is titrated in the same way (although twice as potent
cient breathing technique. as morphine).
c. Fan • Starting dose will depend on whether the patient
• Air flow over the nose and mouth has been is already using morphine (for pain or breathless-
shown to be effective in reducing the sensation of ness) or not (Tables 26.2 and 26.3).
breathlessness. f. Benzodiazepines
• A handheld fan is a simple, portable, and effective • Patients with prognosis of months: avoid.
device for easing breathlessness. • Prognosis of weeks: use if significant anxiety com-
• Similarly, air flow through an open window or larger ponent to breathlessness. Use oral benzodiazepine
fan is effective. with shorter half-life (lorazepam) initially.
TABLE
26.2 Starting Doses & Titration of Morphine for Breathlessness in Opioid Naïve Patients
Months Prognosis Weeks-Months Prognosis Days Prognosis

Morphine PO (immediate 1 mg twice daily → 1 mg four times daily → 1 mg every 4 h → 2 mg 2.5–5 mg every 4 h
release) every 4 h → 3 mg every 4 h → 5 mg every 4 h → further
increases by 30%–50% (increase weekly)
Morphine PO (modified 5 mg twice daily 5 mg twice daily. Titrate total daily dose once N/A
release) a week by 10 mg. I.e. to 10 mg twice daily
→ 15 mg twice daily. Maximum effective
dose usually 30 mg daily
Morphine SC N/A N/A 2.5–5 mg every 4 h
Morphine CSCI/24 h N/A N/A Start with 10 mg CSCI/24 h
Oxycodone is an alternative in patients unable to tolerate morphine. As morphine to oxycodone potency is 1:2, start with half the equivalent morphine dose.
CSCI, Continuous subcutaneous infusion; SC, subcutaneous.
TABLE
26.3 Starting Doses & Titration of Morphine for Breathlessness in Patients Already Taking Morphine
Months Prognosis Weeks Prognosis Days Prognosis

Morphine PO PRN (immediate 25%–100% of the 4-hourly analgesic dose (e.g., morphine total 60 mg PO in 24 h →
release) 4-hourly analgesia dose = 1/6 of 24-h dose = 10 mg PO → breathlessness dose =
2.5–10 mg PO morphine)
Morphine PO (modified release) Titrate pre-existing morphine modified release N/A
(MR) dose according to regular PRN morphine
immediate release (IR) use. (e.g., Morphine MR
30 mg BD but taking 4 doses of morphine IR
5 mg every day for breathlessness → increase
Morphine MR to 40 mg twice daily).
Morphine SC PRN N/A N/A Half of oral PRN dose (PO:SC 1 : 2),
calculated as above.
Morphine CSCI/24 h N/A (unless vomiting and needs temporary Half of current oral 24 h dose
replacement, then as for Days Prognosis)
If established on oxycodone, the same principles apply.

CSCI, Continuous subcutaneous infusion; PRN, as needed; SC, subcutaneous.
TABLE
26.4 Breathlessness: Benzodiazepine Dose and Choice
Months Prognosis Weeks Prognosis Days Prognosis

Lorazepam Avoid If significant anxiety If significant anxiety
500 µg every 4 h 500 µg–1 mg every 4 h
Diazepam Avoid Avoid unless significant anxiety. Minimal effective N/A
dose (e.g. 2 mg twice daily, titrated slowly)
Midazolam SC N/A N/A 2.5–5 mg every one hour
Midazolam CSCI N/A N/A Minimum 10 mg CSCI/24 h
CSCI, Continuous subcutaneous infusion; SC, subcutaneous.
• Prognosis of days: benzodiazepines often used par- Nonproductive

enterally in combination with opioid. Intent of • If irreversible cause, a dry cough should be suppressed.
benzodiazepine use is to relieve distress. Sedation a. Peripheral suppressants: simple linctus may help pha-
may be a consequence, but is likely anyway due ryngeal irritation
to exhausting effect of dyspnoea and deteriorating b. Central suppressants:
condition. • Opioid naïve patients: pholcodine 10 mL three
• Doses as in Table 26.4 times daily; codeine 30 to 60 mg four times daily
g. Oxygen or morphine 2.5 to 5 mg PO every 4 hours. Titrate
• Long-term therapy for patients who are hypoxaemic morphine as for pain.
due to life-limiting illness (e.g., COPD, interstitial • Patients established on strong opioid: dose equivalent
lung disease, cardiac failure, cystic fibrosis, neuro- to PRN analgesic dose can be tried.
muscular disorders, obstructive sleep apnoea [OSA], • Diazepam 5 mg PO daily if other measures
pulmonary hypertension). ineffective.
• Ambulatory oxygen should be supplied to patients
qualifying for long-term oxygen therapy who are Other Symptoms
mobile and wish to leave the home OR patients Bronchorrhoea
who desaturate on exercise. • Bronchorrhoea is large volume mucous production which
• Nocturnal oxygen can be used for patients with is seen most often in alveolar cell (and other) lung cancers.
nocturnal hypoxaemia. • Radiotherapy should be considered, but if this is not
• Oxygen’s potential for psychological dependence possible, the following can be tried:
and associated complications (drying/bleeding of • Antimuscarinics: glycopyrronium (SC and CSCI),
nasal mucosa, hazards of equipment/tubing, risks hyoscine (transdermal, SC, and CSCI)
in hypercapnic respiratory failure) means it should • Corticosteroids PO: dexamethasone 4 to 8 mg PO
only be started if absolutely necessary. daily
• Octreotide CSCI 300 µg CSCI/24 h
Cough
• Management is guided by whether the cough is productive Haemoptysis
or not, and whether the patient is able to expectorate. • Consider using antibiotics (if due to infection); low-
molecular-weight heparin (if due to pulmonary embolism);
Productive, Able to Expectorate radiotherapy (for bleeding tumour).
• Treat cause/infection: antibiotics • Tranexamic acid 1 g PO three times daily can be used to
• Loosen secretions: 0.9% saline nebulizers 2.5 to 5 mL improve coagulation; reduce dose to 500 mg three times
four times daily; carbocisteine 375 to 750 mg three times daily 1 week after bleeding stops. Alternatively, can be
daily stopped 1 week after bleeding ceases, but resume if bleed-
• Physiotherapy ing recurs.
• Bronchospasm: salbutamol • Minor/moderate haemoptysis may herald a massive haem-
orrhage. For management of massive haemoptysis see
Productive, Unable to Expectorate “Emergencies—Haemorrhage (Massive)”
• Antimuscarinics (e.g., glycopyrronium; see section on
end-of-life symptom control); hyoscine transdermal patches Lymphangitis Carcinomatosis
may be helpful if prognosis of weeks • Corticosteroids (dexamethasone 8 mg PO daily) may
• Cough suppressants (see upcoming discussion) improve symptoms.
• Diuretics may have some benefit (furosemide 40 mg + • Bowel obstruction

daily) • Vestibular dysfunction
• Psychological factors: anxiety, fear
Gastrointestinal Symptoms • Paraneoplastic
• Infection
GUIDELINES
Potentially Reversible Causes
Watson, M., Lucas, C., Hoy, A., et al. (2009). Oxford
handbook of palliative care. Oxford: Oxford University Press. • Constipation, pain, infection, hypercalcaemia, ascites,
NHS Scotland. Scottish palliative care guidelines. www raised intracranial pressure, medications
.palliativecareguidelines.scot.nhs.uk/.
Management
Based on the following principles:
1. Identify underlying cause
2. Treat/manage cause if reversible
Nausea, Vomiting, and Regurgitation 3. Have a clear rationale for pharmalogical management:
• Nausea and vomiting should be assessed separately: They Choose antiemetic or antinausea medication based on
can occur independently or together. likely cause
• When considering vomiting, it is important to distinguish 4. Administer antiemetic:
this from expectoration and true regurgitation. a. regularly: give a stat dose before starting regular
• Expectoration: should be managed as for cough antiemetic;
• Regurgitation: often due to intrinsic or extrinsic com- b. by appropriate route (often parenteral, especially if
pression of oesophagus by tumour. If due to oesophageal vomiting);
mass, it may be amenable to stenting/balloon dilatation c. be prepared to titrate regular dose after 24 hours; and/
(if patient can tolerate procedure) or
d. rotate antiemetics if initial choice is ineffective after
Potential Causes 24 to 48 hours.
• Drugs: opioids, NSAIDs, antibiotics, iron supplements Table 26.5 summarizes antiemetic choice by underlying
• Metabolic: hypercalcaemia, uraemia aetiology.
• Treatments: radiotherapy, chemotherapy • Specific considerations:
• Intracerebral malignancy (primary or secondary) • Some nausea/vomiting may require combinations
• Gastrointestinal stasis, constipation of antiemetics. When choosing combinations, use
TABLE
26.5 Antiemetic Choice According to Underlying Aetiology/Cause
Stimulus/Cause of Nausea or Antagonist—Appropriate

Vomiting Central Receptor Antiemetic Choice Peripheral Action
Drugs (morphine), metabolites, D2 (CTZ) Haloperidol None
toxins, uraemia, hypercalcaemia
Gastric stasis D2 (CTZ) Metoclopramide Cholinergic/blocks dopamine
brake in gut; prokinetic effect
Raised intracranial pressure H1 (VC) Cyclizine Antimuscarinic (slows bowel)
Motion Achm (VC) Hyoscine Hydrobromide Anticholinergic; reduces
secretions and spasm
Vestibular D2, 5HT2, H1 Prochlorperazine
Various D2, 5HT2, Achm, Levomepromazine
H1 (VC and CTZ)
Cytotoxic chemo, Radiotherapy 5HT3 Ondansetron 5HT3 receptors in bowel;
(RT) (metoclopramide) ondansetron is very
constipating
Anxiety, fear GABA mimetic Lorazepam
(cerebral cortex)
Receptors: 5HT2/3, serotonin; Achm, acetylcholine; D2, dopamine; H1, histamine.

Location of receptors (VC), vomiting centre; (CTZ), chemoreceptor trigger zone.
antiemetics with different (complimentary) central • Patients may experience infrequent/irregular bowel
actions. Be aware that some combinations will be evacuations and difficulty in defecating. Stool may be
antagonistic (e.g., cyclizine + metoclopramide antag- hard.
onize one another’s actions peripherally). • Causes include debility, diet (reduced intake/low residue),
• Ondansetron is very specific in its mode of action and very poor fluid intake, medications (opioids, antimuscarinics,
constipating. Aside from its use in cytotoxic chemotherapy diuretics, 5HT3 receptor antagonists), and biochemical
and radiotherapy, the only other main indication is dis- abnormalities (hypercalcaemia, hypokalaemia).
seminated abdominal/gastrointestinal (GI) malignancy • Consequences include pain, urinary retention, confusion,
(which can release large amounts of serotonin). overflow diarrhoea.
• Route of administration and dosing: • Many laxatives are available, and often classified by action
• Antiemetics should be given parenterally if vomiting although there is much overlap.
is persistent or severe as this will impair absorption of • Laxative choice should be based upon patient preference/
oral medications. ability—almost all will work if given in sufficient dose.
• If antiemetic is given CSCI, regular opioid dose can
also be replaced in this way until nausea/vomiting is Management
controlled. • Anticipate constipation (e.g., when starting an opioid
• Once nausea/vomiting is settled for over 48 hours on prescribe prophylactic laxatives).
CSCI medication, switch to the equivalent oral anti- • Encourage general measures to aid regular bowel habit
emetic dose. (e.g., fluid intake, fibre in diet, mobility, good access to
• Unless underlying cause resolves, antiemetics will likely toilet).
be required long term. The exception is when starting • Use oral laxative first (rather than rectal):
an opioid, as tolerance to nausea may develop in a • Combination of stimulant and softener/osmotic is
week or so. usual.
• Table 26.6 summarizes dosing and route of administration. • Titrate dose until desired consistency and frequency
are achieved.
Constipation • Patients with spinal cord compression/cauda equina/
• Constipation is a common problem in patients with life- neurological disease may need a bowel regime to aid
limiting illness. defecation. This often consists of senna tablets 3 nights
TABLE
26.6 Antiemetics: Dosing by Route of Administration
Dose and Frequency
Antiemetic PO SC CSCI Notes

Haloperidol* 500 µg–1.5 mg at night and every 2–5 mg/24 h Good at End of Life (EoL), when anxiolytic /
4 hr PRN anti-psychotic properties may also be
beneficial
Metoclopramide* 10 mg three times daily to 20 mg 30–100 mg/24 h Good prokinetic, comparatively large
four times daily (10 mg every 4 hr volume for SC injection
PRN with maximum 24-hr dose of
100 mg)
Cyclizine 50 mg twice/thrice daily or every 8 hr 100–150 mg/24 h Can cause cutaneous irritation
PRN
Levomepromazine* 6.25 mg at night and every 4 hr PRN 12.5–25 mg/24 h Broad-spectrum, second line antiemetic.
(up to 25 mg in 24 h) Useful PRN in addition to another
regular antiemetic
Ondansetron 4–8 mg twice/thrice daily 8–16 mg/24 h Very constipating
Domperidone 10 mg three times Unavailable Unavailable Good prokinetic which does not cross
daily blood-brain barrier (no antidopaminergic
effects), but only available PO and PR
Lorazepam 500 µg–1 mg once N/A Useful for anticipatory nausea/vomiting
daily and every
4 hr PRN
*Avoid in Parkinson disease and Lewy body dementia.

TABLE
26.7 Laxatives Classified by Mode of Action and Dose
Stimulants • Senna 15 mg at night (prophylaxis); 15–30 mg Avoid in bowel obstruction

twice daily (established constipation)
• Sodium picosulphate 5–10 mL twice daily
• Bisacodyl 5–10 mg at night (prophylactic); 20 mg at
night to 20 mg twice daily (established constipation)
Softeners • Docusate 100–200 mg twice daily May be useful in partial bowel obstruction
Osmotic • Macrogols Avoid lactulose unless indicated in hepatic
impairment (requires large volume of fluid,
causes bloating/flatus and abdominal cramps)
Bulk forming • Ispaghula husk, methylcellulose Generally avoided as may worsen constipation if
patient unable to take sufficient fluids
Rectal agents • Glycerol suppository (lubricates/softens stool) Avoid in patients at risk of bleeding (e.g.,
• Bisacodyl suppository (stimulates rectal mucosa) thrombocytopenia) or infection (neutropenia)
• Microenema
• Phosphate enema
Peripheral opioid • Methylnaltrexone bromide (injection) For opioid-induced constipation where other
antagonists • Naloxegol (oral) measures have failed
per week, with glycerol/bisacodyl suppository given the

following morning to empty rectum and sigmoid colon. Acid Reflux, Gastritis, Oesophagitis
• Table 26.7 classifies laxatives by mode of action and dose. • Can be a problem in tumours affecting the stomach and
oesophagus.
Diarrhoea
• Ensure adequate fluid intake while managing the cause. Management
• Causes include: • Proton pump inhibitors can be used at up to double dose
• laxatives; (e.g., lansoprazole 30 mg twice a day, omeprazole 40 mg
• other medications (e.g., antibiotics); daily).
• infection; • The H2-antagonist ranitidine can be used in doses up
• faecal impaction: sudden onset diarrhoea after period to 600 mg daily (in divided doses twice daily or four
of constipation; times daily). It can also be given CSCI (150–300 mg
• radiotherapy (colitis); CSCI/24 h).
• malabsorption:
a. Pancreatic insufficiency (cystic fibrosis [CF], pan- Hiccup
creatic cancer, pancreatectomy): steatorrhoea (i.e., • Diaphragmatic spasms, often caused by irritation from
pale, fatty stools with offensive odour) gastric distension or hepatomegaly. Renal failure and
b. Gastrectomy: steatorrhoea steroids are other potential causes in patients with
c. Colectomy: profuse, watery stools LLI.
• carcinoid tumour: profuse diarrhoea.
Management
Management • Antiflatulent/prokinetic/PPI, for example:
• Laxatives: stop and review doses • peppermint water 10 mL PRN; or
• Antibiotics: check for/treat Clostridium difficile • metoclopramide 10 mg three times daily PO; or
• Faecal impaction: appropriate laxatives, often macrogols • lansoprazole 30 mg daily.
• Radiotherapy (colitis): steroids (dexamethasone 8 mg daily • Smooth muscle relaxant (e.g., nifedipine 5 mg three times
for 1–2 weeks) daily)
• Malabsorption—steatorrhoea: replace pancreatic enzymes • Suppression of central hiccup reflex, for example:
with every meal/snack/drink that is not clear fluid; PPI • baclofen 5 to 10 mg three times daily PO;
• Carcinoid tumour: 5HT3 receptor antagonist (ondanse- • haloperidol 1 to 3 mg twice daily;
tron), octreotide • midazolam 10 mg + CSCI/24 h for severe hiccup in
• Nonspecific: loperamide or opioids (e.g., codeine, morphine) patients in last days of life.
• Encourage patients to eat what they like rather than spe-

Ascites cific foods or supplements.
• May be due to malignant or nonmalignant aetiology. • Steroids (e.g., dexamethasone 2–4 mg daily PO) may
• If patient is too frail for either diuretics or invasive pro- help improve appetite but effects are often short lasting.
cedures, symptom management with analgesia and anti- • Megestrol acetate 80 to 160 mg daily may be better for
emetics should be maximized. longer term appetite stimulation but there is a risk of
thromboembolism.
Causes • Both steroids and progestogens increase catabolism of
• Nonmalignant: advanced hepatic disease with portal hyper- skeletal muscle.
tension, cardiac failure • Prokinetics may improve early satiety.
• Malignant: tumours of ovary/endometrium, bowel, liver
(primary or secondary), peritoneal metastases from other Cachexia
tumours (e.g., breast) • This is a common but complex problem seen in advanced
LLI including heart failure, respiratory disease, and cancer.
Management • Likely related to factors such as development of chronic
• Nonmalignant: diuretics (spironolactone and furosemide) inflammatory state (due to cytokine production), abnormal
are especially valuable in portal hypertension. Expect 0.5 metabolism, and anorexia.
to 1 kg/24 h weight loss; may take 2 to 4 weeks to achieve • The consequence is loss of fat and skeletal muscle which
significant reduction in ascites: is not improved by increasing nutritional intake alone.
a. Monitor for electrolyte disturbance, hypotension, and • Management: manage anorexia, optimize nutrition.
renal function. Monitor U&Es weekly or before chang-
ing dose. Dysphagia
b. Dose/titration: • Look for reversible cause (e.g., oesophageal tumour) ame-
• Start spironolactone 100 to 200 mg every morning. nable to stent/balloon dilatation.
Increase by 100 mg every 3 to 7 days (maximum • Prokinetics may be helpful.
response after 2–3 days). Usual maintenance 300 mg
every morning. Maximum 400 mg every morning Malignant Bowel Obstruction
or 200 mg every morning in patients who are frail/ • Most often associated with cancer of bowel or ovary.
elderly/renally impaired. Intrinsic or extrinsic compression of the bowel by tumour
• If no change after 2 weeks, consider adding furosemide (e.g., peritoneal metastases) can cause obstruction in mul-
40 mg for a few days. This can be increased by 40 mg tiple sites.
every 3 to 7 days to a maximum of 160 mg/day. • Often a clinical diagnosis: Investigations may show site of
c. Stop diuretics if not tolerated, ascites unchanged, or obstruction, but not always (i.e., functional obstruction).
renal function impaired. • Symptoms may wax and wane for many months before
• Malignant: complete obstruction develops.
a. Diuretics can be tried, but likely to be less suc- • Symptoms depend on site of obstruction:
cessful than in nonmalignant ascites, as ascites is • High obstruction of gastric outlet and small bowel—
often due to factors other than portal hypertension large-volume vomiting is predominant feature, often
alone. faeculent once established.
b. Paracentesis provides effective relief of symptoms, but • Low obstruction of colon—constipation is predominant
fluid may reaccumulate within weeks and require repeat feature with vomiting coming later.
drainage. • Both can cause colicky abdominal pain and vomiting
c. Indwelling, tunneled ascitic drains (PleurX peritoneal with or without nausea.
catheter drainage system) are recommended for patients
with recurrent ascites resistant to treatment. Patients Management
can empty these themselves every few days as needed. • Consider surgical/interventional options.
• In advanced malignancy with peritoneal disease,
Anorexia, Cachexia, Dysphagia obstruction is likely to be at several sites and patients
• All three can cause significant distress to the patient and are likely to be unfit for surgery. But suspected obstruc-
those important to them. Managing these symptoms is tion at a single site may be amenable to surgery (e.g.,
important, but consideration needs to be given to the colostomy for obstructing rectal mass); stent to gastric
fact that they are often an inevitable consequence of outlet or duodenum.
advanced disease in the final days of life. • Parenteral fluid and nutritional support may be an
appropriate temporary measure for patients in whom
Anorexia there is potential for intervention by surgery or stent.
• There is a loss of appetite for food. • Symptomatic treatment for patients unsuitable for surgery:
• Manage underlying/contributing factors if possible (e.g., • On initial presentation, management is aimed at encour-
nausea, constipation, oral problems, ascites). aging peristalsis (prokinetic [e.g., metoclopramide
30–120 mg daily CSCI]—stop if causes colic), reducing Management

peritumour oedema and compression (dexamethasone • Stimulate natural saliva (e.g., using sugar-free chewing
8 mg daily PO/SC), and softening stool (docusate gum).
100–200 mg twice daily). • Use artificial saliva (e.g., Biotene Oral Balance gel®, AS
• If symptoms do not resolve in 2 to 3 days or obstruc- Saliva Orthana®)
tion is thought to be complete, treat nausea (cyclizine • Pilocarpine:
150 mg CSCI/24 h), colic (hyoscine butylbromide • Particularly useful after radiotherapy for head/neck
60–120 mg CSCI/24 h), and pain (CSCI opioids). cancer
In complete bowel obstruction, the aim is to reduce • Dose 5 to 10 mg PO four times daily (pilocarpine 4%
the volume and frequency of vomits and associated eyedrop solution is an alternative; use 3 drops PO four
symptoms. times daily)
• If high-volume vomiting, consider Ryle tube for drain-
age or pharmacological management (hyoscine butyl- Sialorrhoea
bromide as above, octreotide 300 µg CSCI/24 h, or • Seen most often in patients with advanced neurological
ranitidine 150 mg CSCI/24 h). disease
Management
Oral Symptoms • Tricyclic antidepressants (e.g., amitriptyline 10 mg at night)
Oral Candidiasis • Hyoscine hydrobromide transdermal patch 1 mg/72 h;
• Typical presentation is with altered taste and adherent white up to two patches can be applied at one time
candida plaques but may present with redness/soreness. • Propantheline 15 mg three times daily
• When diagnosing oral candidiasis, consider if oesophageal • Atropine (1% eye drops) 2 to 3 drops three times daily
extension is likely.
Management Neurological Symptoms (See Also “Advanced

• Good denture hygiene and mouth care
Neurological Diseases”)
• Oropharyngeal: nystatin 100,000 units/mL (1 mL four
times daily for 7 days) or miconazole gel (5–10 mL Seizures
four times daily for 7 days) for mild infections; flucon- • Seizures may be due to pre-existing epilepsy or a present-
azole (50 mg once daily for 7 days) for moderate-severe ing or later feature of intracerebral malignancy or neu-
infections rodegenerative disease.
• Oesophageal candidiasis: fluconazole 50 mg daily for 7
to 14 days Management
• In immunocompromised: longer courses of fluconazole • For acute management see “Emergencies - seizures.”
may be necessary • If known epileptic on established therapy:
• consider increase in current antiepileptic dose (AED);
Mouth Ulcers and Oral Stomatitis • consider addition of levetiracetam.
Mouth Ulcers—Treatment • If initiating AED for new seizure in patients with irrevers-
• Corticosteroids (e.g., hydrocortisone oromucosal tablets ible intracerebral lesion/malignancy:
2.5 mg four times daily) a. in patient with intracerebral mass/metastases with
peri-tumour oedema, consider dexamethasone 16 mg
Oral Inflammation/Stomatitis—Treatment daily PO (temporary measure); will require titration
• Correct reversible causes such as badly fitting dentures. downwards to lowest tolerated dose;
• Maintain oral hygiene with mouthwashes if brushing too b. Antiepileptics:
painful (e.g., sodium chloride, sodium bicarbonate, or • Sodium valproate 200 mg PO twice daily (modified
chlorhexidine [alcohol free]). release); titrate by 200 mg twice daily every 3 days,
• Coating agents can help in oral mucositis due to chemo/ maximum 2.5 g daily; OR
radiotherapy (e.g., GelClair® and Orabase®). • Levetiracetam 250 mg PO twice daily; titrate by
• Local anaesthetics may help (e.g., lidocaine ointment 5%, 250 mg twice daily every 2 weeks, maximum 3 g daily.
cocaine hydrochloride 2%). • Second line (e.g., uncontrolled seizures currently taking an
• NSAIDs may help (e.g., benzydamine 0.15% mouthwash). oral antiepileptic or when other antiepileptic contraindicated/
• Systemic analgesics (opioids and nonopioids) may be not tolerated); levetiracetam, dose as above.
helpful but may need to be given parenterally (CSCI).
• Treat any secondary infection. Intracerebral Malignancy
• Intracerebral malignancy may cause a variety of symptoms,
Xerostomia but most usually:
• Often the consequence of medications (e.g., morphine, • weakness, incoordination, dysphasia;
antimuscarinics) or treatment for cancer (e.g., radiotherapy). • headache;
• seizures; • BOX 26.1 Confusion Assessment Method,

• nausea/vomiting; Four Item
• reduced consciousness.
• Radiotherapy may exacerbate symptoms. 1. Acute onset and fluctuating course
a. Is there evidence of an acute change in mental status
• Dexamethasone may reduce any symptoms related to from the patient’s baseline?
intracerebral oedema. b. Did the abnormal behaviours fluctuate during the day or
change in severity?
Management With Steroids 2. Inattention
• Consider high-dose dexamethasone trial (e.g., 8 to 16 mg a. Did the patient have difficulty focusing attention (e.g.,
being easily distractible) or have difficulty keeping track of
PO daily). what was being said?
• Symptomatic improvement should be seen in 2 to 3 days. 3. Disorganized thinking
• Monitor for hyperglycaemia, measuring Capillary Blood a. Rambling or irrelevant conversation, unclear/illogical flow
Glucose (CBG) minimum twice weekly in patients with of ideas, unpredictable switching from one subject to
no known diabetes or daily in patient with diabetes. another
4. Altered level of consciousness
• If unable to swallow, dexamethasone can be given sub- a. Vigilant (hyperalert), lethargic (drowsy, easily roused),
cutaneously (best given once/twice daily in doses before stupor (difficult to rouse), unrousable
lunchtime to reduce risk of insomnia). If YES to 1+2+3 or 4, a diagnosis of delirium is
• Discontinue dexamethasone if: suggested.
• no improvement after 1 week;
• side effects result from steroids (e.g., uncontrolled
hyperglycaemia, restlessness).
• If improvement in symptoms after 1 week, titrate dose
down to lowest tolerated. Typical reduction of daily dose
by 2 to 4 mg, reduced weekly. See Table 26.10. Diagnosis/Presenting Features
• Following cerebral radiotherapy, steroid dose should not • Onset typically acute (within hours or days) and fluctu-
be reduced until 1 week after radiotherapy is completed. ates hourly/daily.
Reduce as above. • Cognitive function impaired (e.g., worsened concentra-
tion, slow responses, and disorientation).
Fatigue • Perception altered (e.g., visual or auditory hallucinations).
• Fatigue is a common symptom in patients with advanced • Physical function impaired (e.g., reduced mobility, reduced
LLI, especially cancer and its treatment. movement, restlessness, agitation, changes in appetite,
• It is often related to cachexia (see GI symptoms sleep disturbance).
management). • Social behaviour (e.g., lack of cooperation with reasonable
requests, withdrawal, or alterations in communication,
Management mood, and/or attitude).
• Check for symptomatic anaemia. • Be particularly vigilant for signs of hypoactive delirium.
• Perform thyroid function tests. • Several tools for identifying/diagnosing delirium are
• Manage other symptoms (e.g., uncontrolled pain, insom- available. The key is to suspect delirium, especially in
nia, mood disturbance). patients who may present with hypoactive type. Con-
• Review concurrent medications. sider use of the Confusion Assessment Method (Box
• Recommend: 26.1).
• regular exercise;
• sleep hygiene; Management
• pacing/prioritization of activities. 1. Identify/treat reversible causes (if appropriate), for example:
• For fatigue refractory to these measures consider a psy- • infection (e.g., urinary tract infection [UTI], lower
chostimulant (e.g., methylphenidate or modafinil). respiratory tract infection [LRTI]);
• medications: morphine, sedatives, steroids, anticho-
Delirium linergics, tricyclics, neuroleptics, dopaminergics;
• metabolic: hyperosmolar hyperglycaemic state (HHS)/
GUIDELINES hypoglycaemia, hepatic failure, hypercalcaemia, uraemia,
National Institute for Health and Care Excellence. (2013). hyponatraemia;
Delirium: Diagnosis, prevention and management. NICE • hypoxia;
clinical guideline 103. www.nice.org.uk/guidance/cg103. • dehydration;
Inouye, S.K., et al. (1990). Clarifying confusion: The • urinary retention, constipation;
confusion assessment method. A new method for detection • drug/alcohol withdrawal, including withdrawal of
of delirium. Annals of Internal Medicine, 113, 941–948.
nicotine, antidepressants;
• psychological distress.
2. Provide constant environment: Skin Symptoms

• Quiet room, subdued lighting
• Ensure access to sensory aids (i.e., glasses, hearing aids) GUIDELINES
• Clock, calendar, routine should be on clear display to
International Lymphoedema Framework and Canadian
patient in room Lymphedema Framework. (2010). International
• Familiar objects and people (involve family, friends, Lymphoedema Framework position document: The
carers) management of lymphoedema in advanced cancer and
• Few interruptions oedema at the end of life. www.lympho.org.
• Repeated reassurance and explanation (use lucid British Lymphology Society and Lymphoedema Support
Network. (2016). Consensus document on the management
intervals) of cellulitis in lymphoedema. www.lymphoedema.org/images/
• Simple, respectful communication (use short sentences, pdf/CellulitisConsensus.pdf.
calm manner; allow thinking time for patient)
• Avoid moving patient between rooms
3. Ensure safety of patient and others.
4. Medications:
• Chronic/mild delirium: Lymphoedema and Oedema
i. Haloperidol only (benzodiazepines do not improve Classification of Oedema in Patients With
cognition; may worsen it). Dose 1 to 5 mg SC or Life-Limiting Illness
500 µg to 5 mg PO stat and PRN, with typical • Oedema in advanced disease is common and often mul-
maintenance dose 2.5 to 10 mg CSCI/24 h or 0.5 tifactorial in nature, occurring when net capillary fil-
to 3 mg twice daily PO tration exceeds lymphatic drainage. It may or may not
• Acute/severe delirium (+/− agitation): have impaired lymphatic drainage (lymphoedema) as a
i. First line: haloperidol only. Dose 2.5 to 5 mg PO/ component.
SC/IM hourly; max 20 mg/24 h, with maintenance • Causes of impaired lymphatic drainage (lymphoedema):
dose typically half of the first 24-hour dose OR metastatic lymphadenopathy, surgery (especially when
based on stat doses used lymph nodes removed), radiotherapy, immobility,
ii. Second line: add benzodiazepines—only if sedation longstanding increased flow
is needed and/or alcohol or benzodiazepine with- • Causes of increased capillary filtration: hypoalbuminae-
drawal is a factor mia, venous hypertension (cardiac failure, extrinsic
Note: Chronic confusion, dementia (behaviour that chal- compression from tumour, inferior vena cava obstruc-
lenges), and other symptoms specific to neurodegenerative tion/superior vena cava obstruction (SVCO), venous
disease are included in additional sections. thrombosis), medications (e.g., corticosteroids, NSAIDs,
hormones, chemotherapy)
Chronic Confusion • Oedema and lymphoedema may present therefore in
patients with advanced cancer, heart failure, respiratory
• Presenting features: disease, renal failure, neurological disease, or liver disease.
▪ Onset/course typically chronic (days to weeks) As oedema becomes chronic, inflammatory and fibrotic
▪ Pattern is constant: little/no fluctuation, may be changes develop.
progressive • Lymphoedema services are often part of local palliative
▪ Cognitive function may be normal or impaired (dis- care provision and will normally manage patients with
orientated) BUT consciousness not altered primary (congenital) lymphoedema and secondary lymph-
▪ Hallucinations unusual oedema due to the effects of disease or its treatment. They
▪ Physical impairment rare; health generally good are also a useful source of reference for patients with more
▪ Memory loss prominent in confusion caused by demen- general oedema at the end of life.
tia, mood may be depressed
▪ Confusion due to dementia will gradually worsen over Signs and Symptoms
months • Oedema may be a well-recognized complication of the
underlying diagnosis (e.g., in cardiac or hepatic failure).
Management • Consider underlying venous thrombosis if asymmetric
1. Exclude depression. oedema develops acutely in a limb.
2. General measures as for delirium (see Delirium). • Lymphoedema classically presents as follows:
3. Medication: • In a discrete anatomic location, due to underlying cause
• Chronic confusion: risperidone 0.25 to 1 mg at night (e.g., arm lymphoedema following axillary node clearance).
(gradual increase to 1 mg twice daily) • Skin will indurate or pit when pressure is applied.
• Acute on chronic confusion with delirium: haloperidol • Skin becomes thickened and changes become chronic:
as for delirium (see Delirium) Hyperkeratosis, papillomata, and lymphangiectasia
• Insomnia: trazodone 50 to 100 mg at night develop.
• Cellulitis and lymphorrhoea (leakage of lymph fluid due • Second line (if poor response to amoxicillin/flucloxacillin
to breaks in skin) are recognized complications. after 48 hours):
a. Clindamycin 300 mg four times daily
Management • Duration of antibiotics. Continue antibiotics until all signs
• Of lymphoedema: Management is aimed at reducing of acute infection have resolved. This often means treat-
swelling and thereby restoring function and reducing ment for a minimum of 2 weeks but may be as long as
discomfort. It is tailored to the individual and their stage 1 to 2 months. Note that skin changes/discoloration may
of underlying illness; early intervention is important to persist after infection resolves.
prevent chronic changes. Contraindications are unusual • Other measures. Do not use compression garments in the
but include uncontrolled cardiac failure. Patients diagnosed acute attack but replace as soon as the affected area is
with acute deep vein thrombosis (DVT) must wait 8 weeks able to tolerate them. Elevation of affected limb and bed
before receiving intensive lymphoedema intervention and rest is important. Exercise can be resumed once inflam-
those with arterial insufficiency (ABPI <0.5) should not mation subsides.
have compression.
• Consider investigation/treatment for underlying or exac- Sweating (Paraneoplastic)
erbating causes (e.g., for malignancy, venous thrombosis, Presenting Features
cardiac failure, anaemia, or management of medications • Uncontrolled sweating is often associated with malignancy.
exacerbating oedema). • It may be associated with paraneoplastic pyrexia (i.e.,
• Complete/complex decongestive therapy (CDT) is the pyrexia in absence of infective cause).
mainstay of management. Management is initiated by • Sweating may affect one specific part of the body or be
specialist lymphoedema practitioners and where pos- more generalized.
sible, self-care is taught to the patient/family. CDT
involves: Management
▪ manual lymphatic drainage (MLD). This stimulates • Antipyretics:
lymphatic tissues, decongesting deep lymphatics and • Paracetamol 500 mg to 1 g four times daily
increasing drainage of lymph from the affected site. • Naproxen 250 to 500 mg twice daily (or alternative
MLD is undertaken by specialized professionals who NSAID)
can teach a simplified version (simple lymphatic drain- • Antimuscarinics:
age) to patients or carers; • Amitriptyline 10 to 50 mg at night
▪ compression bandaging, hosiery, or Velcro compression • Propantheline 15 to 30 mg twice daily
devices. These increase tissue pressure to reduce oedema • Hyoscine hydrobromide or glycopyrronium
and promote drainage of lymph (when used with MLD) • Other:
while preventing backflow evacuated lymph. Multi- • Propranolol 10 to 20 mg twice daily, gabapentin
layered compression can contain dressings to absorb
lymphorrhoea; Pruritus
▪ skin care and infection prevention. Skin care with the Causes
intent of avoiding infection/cellulitis includes main- • A relatively common symptom in advanced LLI, pruritus
taining skin hydration (using emollients daily), careful may have a wide variety of causes.
hygiene, and avoidance of skin trauma;
▪ exercise. Undertaken under compression to improve Management
venous drainage, uptake of lymph, and to soften fibrosis. 1. General:
It may be active or passive; • Establish, treat, and remove any causes (e.g., skin infec-
▪ elevation of the affected limb (to a level above the tion, medication).
heart) can be helpful in patients with advanced disease • Keep skin moisturized: Use emollient daily, use aqueous
who are unable to exercise. cream instead of soap, avoid hot baths, dry skin by
• Of cellulitis associated with lymphoedema: patting.
• First line: • Avoid sweating (see section on managing sweating)
a. Oral amoxicillin 500 mg 8-hourly is treatment of and keep skin cool.
choice • Macerated skin should be dried and hydrocortisone
b. If evidence of Staphylococcus aureus infection: flu- 1% used if localized inflammation (dermatitis) present.
cloxacillin 500 mg four times daily in addition to • Topical antipruritics include menthol 0.5% to 2%,
or as alternative to amoxicillin phenol 0.5% to 3%, and camphor 0.5% to 3%.
c. In patients with confirmed penicillin allergy: eryth- • Use sedative antihistamines at bedtime or regularly
romycin 500 mg four times daily or clarithromycin (e.g., chlorphenamine 4 mg three times daily).
500 mg twice daily 2. Cause specific:
d. If complicating factors (e.g., animal scratch/bite) • Rashes: antihistamines (topical cream or systemic);
then discuss with microbiologist menthol in aqueous cream; hydrocortisone 1% cream
• Opioids: chlorphenamine 4 mg three times daily or • Infection may be acute or chronic. If evidence of infection
cetirizine 10 mg daily; switch to alternative opioid; develops, treat as for cellulitis (e.g., flucloxacillin or
ondansetron (4–8 mg twice daily) erythromycin).
• Cholestasis: measures to resolve cholestasis if appropri- • Odour is often due to the presence of anaerobic bacteria.
ate (e.g., biliary stent); cholestyramine (often poorly Options for managing odour include metronidazole topical
tolerated), sertraline 50 to 100 mg once daily, rifam- gel 0.75% applied to the wound or dressing daily OR
picin 150 to 600 mg once daily, naltrexone 12.5 to metronidazole PO 400 mg three times daily for 1 week.
25 mg once daily (if not taking opioid analgesia) If odour improves, metronidazole may be reduced to
• Uraemia: localized itch can respond to topical capsaicin 200 mg once daily (indefinite).
cream; ultraviolet B (UVB) phototherapy, gabapentin
100 mg after haemodialysis or doxepin 10 mg twice daily Exudate and Enterocutaneous Fistulae
• Hodgkin lymphoma: prednisolone 10 to 20 mg three • Exudate requires containment with highly absorbent dress-
times daily ings. Undamaged skin around the wound should be pro-
• Paraneoplastic/other causes: paroxetine 5 to 20 mg tected with a barrier cream such as Cavilon™.
once daily or sertraline 50 to 100 mg once daily, mir- • When fistulae develop, management should include:
tazapine 15 mg daily • protecting surrounding skin (see earlier);
• containing any effluent—using ostomy collection bags
Malignant Wound Management (Bleeding From if necessary;
Wounds, Odour and Infection, Fistulae) • reducing fistula output. Octreotide may be given sub-
• Wounds due to cancer can cause a variety of problems. cutaneously (e.g., 100 µg SC twice or thrice daily).
• Ideally, palliative radiotherapy, surgery, or even chemo-
therapy may help their management, but in advanced
Depression and Anxiety
disease these are not always possible.
• In all cases, aim to make dressings as unobtrusive and Depression
comfortable as possible, change dressings as infrequently • Depression is a significant problem in patients with LLI
as possible, and protect surrounding healthy tissues. of all types.
• Pain should be managed using approaches as outlined in • Its presence should be actively sought, and it should not
“Symptom Management—Pain.” If wounds are signifi- be assumed that low mood or depression in patients with
cantly painful, systemic analgesia will often be required: LLI is inevitable.
• It is advisable to make PRN analgesia available which
can be given prior to changing dressings on malignant Diagnosis
wounds. • Many physical symptoms of depression are common in
• If changing of dressings causes significant distress, LLI, such as loss of appetite, disturbed sleep, fatigue,
lorazepam 500 µg to 1 mg PO can also be given prior psychomotor slowing.
to dressing change. • In palliative care patients, withdrawal, anhedonia, feelings
• An increase in pain in a wound may be a sign of of guilt, hopelessness, or wishes for death/suicide may
infection (see upcoming discussion). suggest depression.
• The simple question “Are you feeling depressed?” can be
Bleeding (for Other Sources of Bleeding, helpful in identifying depression.
See “Bleeding”) • A variety of screening tools are available—those that have
• Haemostatic can be used topically or systemically: fewer questions are often more appropriate for the pal-
• Topical: Tranexamic acid 10% (500 mg/5 mL ampoule) liative population (e.g., Edinburgh Depression Scale).
applied to gauze and held against bleeding surface of wound. • Use of the Distress Thermometer can assist in identifica-
• Systemic: Tranexamic acid 1 g PO three times daily. tion of factors contributing to distress.
Due to potential side effects, halve dose or stop 1 week
after bleeding is stopped. Resume if bleeding recurs. Management
• Other options: • Assess for medications that may affect mood, delirium,
• Gauze soaked in adrenaline 1 : 1000 solution (1 mg hypothyroidism, and dementia.
in 1 mL) and applied to areas of bleeding (note • Ensure good palliative care is provided, including adequate
prolonged/repeated use may cause further ischaemia management of any physical symptoms, spiritual needs,
and further bleeding) and social support needs. Review depressive symptoms
• Alginate dressings once these have been addressed.
• As for any depressive episode, mild-moderate depression
Odour and Infection may be managed with psychological support and cognitive
• Tumours breaching the skin surface (whether primary or behavioural therapy.
metastatic) are liable to ischaemia, necrosis, and anaerobic • If an antidepressant is indicated (moderate-severe depres-
infection. sion) and anticipated prognosis long enough to benefit
(i.e., >1 month) then choice can be based upon side • Sertraline (start with 25 mg daily PO) and citalopram
effects/safety profile and patient comorbidities: are alternatives.
• First choice is usually an SSRI. If partially effective, • Pregabalin is useful in patients with concurrent neu-
titrate dose. If ineffective switch to alternative SSRI ropathic pain.
or mirtazapine. • Benzodiazepine may also be required.
• Sertraline (SSRI, 50-mg starting dose in depression;
25 mg starting dose if anxiety). Safer in chronic renal Bleeding
failure and after myocardial infarction (MI).
• Citalopram (SSRI, 10-mg starting dose). Good in • For massive haemorrhage, see “Emergencies.”
anxiety, safer in patients with seizures. Oral suspension • For haemoptysis, see “Symptom Management—Respiratory.”
available. • For bleeding wounds, see “Symptom Management—Skin.”
• Mirtazapine (15-mg starting dose). Possibly faster mode • Bleeding can occur from a number of anatomic sites;
of action than other antidepressants. Sedative effect management may be directed at the bleeding site or via
and appetite stimulation may be helpful. Safe in cardiac systemic therapy.
failure and diabetes. • Ensure that any medications that may encourage bleeding
• Amitriptyline. Useful if concurrent pain or insomnia. Avoid have been reviewed and stopped if necessary (e.g., LMWH,
if significant cardiac disease/after MI, in hepatic failure, in warfarin, aspirin, clopidogrel, NSAIDs).
patients with glaucoma. Greater risk in overdose. • Treat for any infection which may exacerbate bleeding
• Duloxetine (serotonin norepinephrine reuptake inhibi- (e.g., UTI, chest infection).
tor [SNRI]). May be useful when patients have con- • Consider referring for radiotherapy (e.g., to bladder, lung),
current neuropathic pain. local coagulation (e.g., cryotherapy or diathermy) if
• Methylphenidate. May be used in patients whose prog- appropriate.
nosis is thought to be weeks at most, when time is • Systemic treatment (for any bleeding site):
insufficient for conventional antidepressant. May have • Tranexamic acid PO. Give 1 g three times daily initially.
onset of action within days. Dose 5 mg PO twice daily One week after bleeding stops, this may be stopped
(maximum 40 mg daily). completely or reduced to 500 mg three times daily. If
bleeding recurs, resume 1 g three times daily and con-
Anxiety tinue indefinitely.
• May present with depression, or alone. • Note: There is risk of clot retention if used for
• In patients with days to live, manage with benzodiazepines. haematuria.
• For patients with longer prognosis: • Local treatment:
• Venlafaxine (SNRI) can be helpful. Start with 37.5 mg • Topical tranexamic acid: 10% solution (500 mg/5 mL
PO twice daily. applied on gauze) for cutaneous bleeding or epistaxis;
TABLE
26.8 Dexamethasone Starting Doses (PO)
Daily Dose (Daily Dose >4 mg Usually Given in Two Divided

Indication Doses, Before Lunchtime)
Appetite (anorexia) 4 mg
Liver capsule pain 4–8 mg
Pain due to nerve compression 4–8 mg
Bowel obstruction 8 mg
Note: Often given as a trial SC. Ensure steroid reviewed daily as may
stimulate appetite which can cause significant distress in patient with
irreversible obstruction.
Intracerebral oedema/raised ICP, nausea and 8–16 mg
vomiting due to raised ICP Note: If for nausea/vomiting in ICP give in combination with antiemetic
SC/CSCI.
Superior vena cava obstruction 16 mg
Metastatic spinal cord compression 16 mg
Subcutaneous Dexamethasone:
Dexamethasone can be given by SC injection. The injectable formulation varies, most often either 3.3 mg/mL or
3.8 mg/mL. For practical purposes, consider dexamethasone 4 mg PO equivalent to either 3.3 mg or 3.8 mg SC.
CSCI, Continuous subcutaneous infusion; ICP, intracranial pressure; SC, subcutaneous.

TABLE
26.9 Steroids: Approximate Equivalent Anti-Inflammatory, Glucocorticoid and Mineralocorticoid Properties
Steroid Dexamethasone Prednisolone Hydrocortisone

Equivalent anti-inflammatory dose 1 mg 7.5 mg 25 mg
Relative glucocorticoid activity 30 4 1
Relative mineralocorticoid activity Minimal mineralocorticoid action 0.8 1
5% solution (500 mg/10 mL) as mouthwash or for TABLE

rectal bleeding 26.10 Tapering Dexamethasone Dose
• Silver nitrate stick applied to bleeding points
Starting Weekly Reduction If Recurrent
• Sucralfate paste or suspension
Dose in Daily Dose by Symptoms
>8 mg 4 mg then once Increase back to lowest
Special Notes on Steroids daily <8 mg daily as effective dose, then
below try more cautious
GUIDELINE reduction (e.g.,
<8 mg 2 mg 1–2 mg weekly)
Diabetes UK. (2018). End of life diabetes care. https://www. daily
diabetes.org.uk/resources-s3/2018-03/EoL_Guidance_2018_
Final.pdf.
National Institute of Clinical Excellence. Clinical Knowledge
Summary ‘Corticosteroids - oral’. https://cks.nice.org.uk/
corticosteroids-oral#!scenario. vomiting (e.g., due to intracerebral mass/oedema), but
steroids should not be routinely continued when a patient
is in the last days of life (discussion to come).
Monitoring Patients Taking Corticosteroids

Indications for and Use of Steroids • CBG should be monitored in all patients: daily in patients
(Dexamethasone) with known diabetes (to enable titration of oral hypo-
• Steroids are indicated in the palliative management of glycaemic medications/insulin) and twice weekly in those
various conditions. The most commonly used in palliative not known to have diabetes.
care is dexamethasone—dose varies depending on • Monitor symptom benefit and reassess after 1 week.
condition/symptom being managed (Table 26.8). • See management of steroid-induced diabetes (below).
• When used, consideration should be given to their side
effects, notably hyperglycaemia, muscle wasting (proximal Stopping/Withdrawing Steroids
myopathy), mood disturbance, osteoporosis, and adrenal • At the time of starting steroids, a plan should be made
suppression. for review and reducing the dose.
• Corticosteroids vary in their ratio of mineralocorticoid • Monitor patients for signs of hypoadrenal crisis when
to glucocorticoid properties. See Table 26.9. This deter- steroids withdrawn.
mines their therapeutic action - glucocorticoid properties • Stop steroids abruptly if:
being used in palliative care for anti-inflammatory action. • steroids bring no symptomatic benefit after 1 week;
High mineraolcorticoid action is generally undesirable • unacceptable side effects occur less than 1 week after
(due to resulting water retention), but useful in adrenal starting;
replacement. • a patient enters the last days of life (stop steroids when
• Generally, just one corticosteroid should be prescribed. patient is dying and no longer responsive). In this
See Table 26.9 for equivalent anti-inflammatory doses. situation, steroids can be stopped abruptly, however
• Dexamethasone should be started at an appropriate dose long the duration. But if steroids are potentially man-
(see Table 26.8), and reduction in dose attempted once aging significant symptoms (e.g., raised ICP or SVCO),
symptoms improved (or after 2 weeks at most). consider alternative symptom management options
• Gastric protection in the form of lansoprazole (or similar) (e.g., prophylactic midazolam CSCI) to prevent
should be given, especially to patients also taking NSAIDs. seizures.
• Check capillary blood glucose (CBG) in all patients prior • Taper steroids gradually when:
to commencing corticosteroids. • patient has been taking more than 4 mg dexamethasone
• To avoid insomnia, steroids should be given in divided for over 1 week or a lower dose for over 2 weeks in total
doses with the latest dose at lunchtime. (as this may have suppressed endogenous production);
• Steroids may be given by SC injection. This may be helpful • risk of rebound symptoms exists (e.g., significant cere-
as a temporary measure in a patient with dysphagia or bral oedema);
• The aim is to stop all courses of steroids completely, unless • Regardless of how much experience a professional has,
the patient suffers significant recurrence of symptoms. In there always remains some uncertainty. It is important
this case, maintain on minimal effective dose. to openly acknowledge this, especially to the patient and
• Table 26.10 provides guidance on how to taper the those caring for him/her.
steroid dose. • The following clinical signs may suggest a person is dying:
• Global clinical deterioration despite optimal manage-
Management of New Onset ment of underlying life-limiting illness or concurrent
Steroid-Induced Diabetes problems
• Steroid-induced diabetes can occur in patients previously • Daily deterioration in performance status
not known to have diabetes. It can occur with any cor- • Profound fatigue, minimal oral intake (sips of fluid
ticosteroid taken for more than a few days. only), and difficulty swallowing oral medications
• Steroids taken in the morning tend to cause a rise in • Signs such as agitation, Cheyne–Stokes breathing,
blood glucose around late afternoon/early evening. This significant deterioration in level of consciousness,
can be managed by giving gliclazide or isophane insulin mottled skin, noisy respiratory secretions
in the morning. • In this situation, ensure that a person’s symptomatic and
• CBG should be checked before starting steroids in all physical care needs are anticipated, and that measures are
patients. If CBG is above 8 mmol/L, check with a venous put in place to address their holistic care.
sample and manage accordingly before starting steroids. • Involve the patient as much as they are able or desire.
• In patient with LLI, aim for CBG in the range of 6 to When no longer able to contribute, consider any advance
15 mmol/L to minimize symptoms of hyperglycaemia. decisions they had made and recorded.
• When discontinuing steroids, reduce hypoglycaemic • Attending to the understanding, wishes, and needs of
medications. those important to them—especially any carers—will be
• Patients already known to have diabetes should have their crucial in providing successful care in the last days of life.
medication titrated according to any changes in CBG
exacerbated by steroids. Stopping Treatments
• Management of new steroid-induced diabetes when steroids
given once daily (mornings): GUIDELINES
• Check CBG before evening meal.
• If CBG consistently above 15 mmol/L, start gliclazide Nutrition support for adults: Oral nutrition support, enteral
40 mg with breakfast. tube feeding and parenteral nutrition. NICE clinical guideline
• Titrate gliclazide by increments of 40 mg daily until 32. www.nice.org.uk/.
CBG 6 to 15 mmol/L. Maximum morning dose General Medical Council. (2010). Treatment and care
240 mg daily. If still high, add gliclazide 40 to 80 mg towards the end of life: Good practice in decision making.
www.gmc-uk.org.
in evening.
• If gliclazide ineffective, switch to morning insulin and
seek specialist advice.
• As death approaches, it is appropriate to rationalize and
consider stopping treatment and medications.
Care in the Last Days of Life • Making an informed decision to stop any treatment can
Prognostication be made by any patient with capacity.
• If a medication or treatment is stopped either intentionally
GUIDELINES or because it is no longer possible to administer it, thought
should be given to ensure ongoing symptom control is
Care of dying adults in the last days of life. NICE clinical
unaffected. Alternative means of providing medications
guideline 31. www.nice.org.uk. for symptom control should be used whenever possible.
Thomas, K., Wilson, J.A. and GSF Team. (Dec 2016).
GSF PIG 6th Edition. National Gold Standards Framework Medications
Centre in End of Life Care. http://www. • Medications that can be stopped:
goldstandardsframework.org.uk.
Leadership Alliance for the Care of Dying People. (2014).
• Oral medications for which there is no parenteral
One chance to get it right: Improving people’s experience of replacement once a patient loses the ability to swallow
care in the last few days and hours of life. www.gov.uk/ • Oral medications not contributing to symptom control
government/uploads/system/uploads/attachment_data/ (e.g., prophylactic medications such as statins)
file/323188/One_chance_to_get_it_right.pdf. • Medications that should be continued/alternative found:
• Medications required for symptom control (e.g., long-
term opioids for pain)
• Judging whether a person is entering the last few days of • Medications whose withdrawal may cause severe symp-
life can be challenging. toms (e.g., long-term antiepileptic medications)
• Means of continuing medications: • For example, SC/CSCI opioids for breathlessness, with
• When the oral route is no longer viable, consider SC/CSCI benzodiazepines for breathlessness and
liquid formulation that can be given via percutaneous anxiety. See “Symptom Control—Breathlessness,” “Care
endoscopic gastrostomy (PEG)/radiologically inserted in the last days of life - anticipatory medications,” and
gastrostomy (RIG) (if available). Only continue medica- “Syringe Driver” for doses/guidance.
tions considered necessary for ongoing symptom relief.
• Most medications for symptom control at end of life Anticipatory Medications and Care
can be given CSCI via a syringe driver (see “Syringe
Drivers”). For guidance on doses of CSCI medications at end of life,
see “Syringe Driver.”
Nutrition and Hydration • Everything possible should be done to anticipate symptom,
• A patient with capacity to do so may choose to stop care, and support needs for people in the last days of life
clinically assisted nutrition and hydration at any point. and those important to them.
• Consider the benefits, burdens, and risks of nutrition and • Equipment needed to manage a patient at home (e.g.,
hydration separately. hospital bed, commode, hoist) should be anticipated
• For a person in the last hours to days of life, the burdens wherever possible and adequate support by professional
and risk of clinically assisted nutrition and hydration carers arranged.
usually outweigh the benefits. Guidance from the General • Anticipatory medications should be readily available for
Medical Council (GMC) and Department of Health is subcutaneous administration:
available to assist decision making. • Medications should have appropriate community
• Both should be reviewed regularly, particularly if the administration instructions/record.
patient exceeds his or her anticipated prognosis. • A minimal number of medications should be available
• Nutrition: to avoid confusion (for more options, see relevant
• Most people in the last days to week of life will gradu- symptom control sections).
ally stop eating. • Commonly used anticipatory medications are listed in Tables
• Clinically assisted (parenteral or enteral) nutrition may 26.11 and 26.12, along with appropriate indications for
be stopped by any patient with capacity to make this their use and doses. All injections should be administered
decision or in the patient’s best interests if he or she subcutaneously. There may be variation to this in local guid-
is not competent to give consent (if those responsible ance: local guidance should be followed wherever possible.
for a best interests decision conclude that the burdens • If patients regularly require PRN doses (e.g., three or
and risks outweigh benefits). more PRN doses of analgesia daily), their regular (CSCI)
• Hydration: dose should be increased accordingly.
• In most circumstances, the dying process entails a • Note: Guidance around opioid use in renal failure will
person gradually ceasing to take oral fluids and when vary according to local policy. See “End-Stage Renal
in the last hours to days of life, parenteral replacement Failure” for guidance.
is unlikely to be appropriate.
• A person’s hydration status and needs should be assessed Syringe Driver
as he or she enters the last few days of life.
• Clinically assisted fluids can be stopped by any patient GUIDELINES
with capacity to do so or in the patient’s best interests
if not competent to consent (if those responsible for MIMS Online. (2016). Syringe driver compatibility. www.mims.
co.uk.
a best interests decision conclude that the burdens and Palliativedrugs.com. Syringe driver compatibilities. https://
risks outweigh benefits). palliativedrugs.com.
• Regular mouth care should be provided to patients in
the final days to week of life.
Noninvasive Ventilation Indications and Practicalities

• As with other treatments, noninvasive ventilation (NIV) • Syringe drivers are used to enable delivery of symptom-
can be stopped at any time by a patient competent to control medications when an alternative to the oral route
make the decision to do so. is required, for example:
• Specialist support from palliative care teams/professionals • at end of life when the patient is no longer able to
with working knowledge of the legal and ethical guidelines swallow;
and practicalities around stopping NIV should be available • as a temporary measure in a patient with vomiting or
to healthcare professionals and patients in this situation. dysphagia.
• When stopping, medication may be given to alleviate • They are battery-powered devices that enable the admin-
any symptoms experienced, particularly for patients requir- istration of medications by CSCI. The most common in
ing NIV 24 hours a day: current use is the CME McKinley T34.
TABLE
26.11 Anticipatory PRN Medications For Symptom Control (Opioid Naïve Patients)
Symptom/Indication Medication PRN Dose (SC Unless Stated Otherwise)

Pain Morphine (opioid naïve patient) 2.5–5 mg 1-hourly
Diamorphine (opioid naïve patient) 2.5–5 mg 1-hourly
Nausea/vomiting Haloperidol 1–2 mg 4-hourly
Levomepromazine 6.25–12.5 mg 4-hourly
Secretions Glycopyrronium 200–400 µg 4-hourly
Hyoscine butylbromide 20 mg 4-hourly
Delirium Haloperidol 1–5 mg SC 4-hourly (see also section on delirium)
Agitation Midazolam 2.5–5 mg 1-hourly
Seizures Midazolam OR 10 mg for prolonged/distressing seizures (may also be
given via buccal route; see also section on seizures)
Diazepam 10 mg PR
Risk of distressing life-threatening Midazolam 10 mg IM (if haemorrhage)
event (e.g., massive
haemorrhage, airway obstruction)
IM, Intramuscular; PRN, as needed; SC, subcutaneous.
TABLE
26.12 Anticipatory PRN Opioid Analgesia: Patients on Regular/Established Opioids
Regular Opioid PRN SC Opioid Additional Information

Morphine or oxycodone—ORAL 1/12 of patient’s current daily (24 h) oral Replace regular oral opioid with CSCI
dose dose when patient is unable to swallow
Morphine or oxycodone—CSCI 1/6 of total daily CSCI dose
Diamorphine—CSCI 1/6 of patient’s current CSCI dose
TD opioid patch (e.g., Provide PRN medication at appropriate dose Continue TD opioid patch until death;
buprenorphine or fentanyl) (see approximate equivalences in “Pain” or titrate analgesia by adding in regular
Appendix 33 and/or seek specialist advice) CSCI opioid if indicated
Methadone Seek specialist guidance
Ensure patient has anticipatory medications for other symptoms also - see Table 26.11
CSCI, Continuous subcutaneous infusion; PRN, as needed; TD, transdermal.
• Infusion through the skin is achieved by plastic/Teflon • Combinations of medications (e.g., analgesia plus anti-
cannula: emetic) can be used. When using combinations of
• The best sites for these are upper arms and anterior medications:
chest wall and suprascapular region, but abdominal • check compatibility data as some will not mix and
wall and thighs can also be used. may precipitate, affecting symptom control (see Appen-
• Sites should be inspected regularly for signs of dix 31);
reaction/irritation. They should be rotated every 3 to • water for injection is the normal diluent, with a few
5 days. exceptions;
• CSCI administration is off label use for most medications. • dilute contents to the maximum volume possible to
• The conventional infusion period is 24 hours, which helps reduce risk of skin reactions at the infusion site;
ensure contents remain stable and sterile and achieves • usually up to three drugs are combined;
more even plasma concentration of medication than • combination and dosing is limited by volume: The
repeated SC injections: maximum fill volume of a McKinley T34 is 34 mL
• Contents are changed (and adjusted as necessary) once in a 50-mL syringe, although 22 mL in a 30-mL syringe
daily. is more commonly used.
• It is good practice to ensure patients have additional PRN tables (Appendix 33)/antiemetic dosing table in
medications available for symptom control. It can take symptom control section (Table 26.6)).
hours to appreciate the benefit of starting CSCI medica- c. If symptoms controlled and a switchback from CSCI
tions and SC alternatives should be available for symptoms to PO is indicated, remember to increase doses
such as breakthrough pain or nausea. where necessary. CSCI is usually stopped when the
first PO dose is given.
Starting Doses
TABLE Starting Doses for CSCI Medications in the
See also “Symptom Management Specific to Advanced 26.13 Last Days to Week of Life
Chronic Kidney Disease/End-Stage Renal Failure” for patients Symptom/
with known renal impairment. Medication CSCI/24 h
• Syringe driver starting doses:
• Table 26.13 gives common staring doses for CSCI Pain (opioid Morphine 10 mg CSCI/24 h OR
medications. naïve) Oxycodone 5–10 mg CSCI/24 h OR
Diamorphine 10 mg CSCI/24 h
• See section on symptom control of nausea and vomit-
ing for CSCI doses of antiemetics (Table 26.6). Agitation Midazolam 10–20 mg CSCI/24 h
• Patients on established medications. Remember to stop the Nausea/ Haloperidol 3–5 mg/24 h OR
equivalent oral medications (e.g., modified release opioid), vomiting CSCI equivalent of alternative antiemetic
if switching to CSCI delivery (Table 26.14): Secretions Glycopyrronium 600 µg–1.2 mg/24 h
• Converting from PO to CSCI and vice versa:
a. Medications are normally more potent SC (CSCI) Risk of Midazolam 20 mg+/24 h
seizures
than PO, and as such a dose reduction is usual.
b. Refer to local guidelines on dose conversion, seek CSCI, Continuous subcutaneous infusion.
specialist advice (and refer to opioid equivalence
TABLE Guidance on Switching From Established PO and Transdermal to Continuous Subcutaneous Infusion
26.14 Medications
Symptom/Medication CSCI/24 h
Pain: PO:SC potency ratio for both morphine and oxycodone is
Morphine or oxycodone PO regular dose (immediate approximately 1 : 2, so HALVE the total oral 24-h dose and
or modified release) continuing as CSCI equivalent administer CSCI
For example, morphine 100 mg total PO/24 h = morphine 50 mg
CSCI/24 h
For example, oxycodone 40 mg total PO/24 h = oxycodone
20 mg CSCI/24 h
Pain: PO morphine: SC diamorphine potency ratio is approximately 1 : 3
Morphine PO regular dose switching to equivalent For example, morphine 30 mg total PO/24 h = diamorphine 10 mg
CSCI diamorphine CSCI/24 h
(see “Symptom Control—Pain” and Appendix 33 for
guidance)
Pain: Continue transdermal patch and supplement with SC PRN strong
Transdermal buprenorphine or fentanyl patches opioid (morphine, diamorphine, or oxycodone if no renal
impairment)
If requiring three or more PRN SC opioid doses daily, supplement
with CSCI opioid
For example, additional three doses of morphine 5 mg SC in 24 h
Start morphine 15 mg CSCI/24 h in addition to transdermal patch
Seek specialist advice on dosing.
If nausea/vomiting: CSCI equivalent of previous antiemetic See Table 26.6 in
“Symptom Management Guidelines; Gastrointestinal Symptoms”
Established antiepileptic medication Midazolam 20 mg+/24 h
Levetiracetam and sodium valproate are used CSCI in some
regions: seek specialist advice.
For renal failure, seek specialist advice and refer to “End-Stage Renal Failure.”

• Converting from transdermal to CSCI: • Similarly, hydration needs must be assessed in context
a. When a patient is in the last days to week of life, and managed according to individual risk/benefit.
transdermal opioids should be continued. Any addi- • A decision may be reached to continue feeding/fluids
tional analgesic requirements are supplemented by orally at risk, and manage complications symptomatically
PRN SC and CSCI strong opioid. should they occur.
• Mouth care should always be provided to patients when
Advanced Neurological Disease they are no longer able to swallow.
• See also sections: Recurrent Infection

• “Symptom Management Guidelines; Pain—Other • Infections such as UTI or chest infection/aspiration are seen
Types; Skeletal Muscle Spasm” in advanced respiratory disease due to the consequences
• “Symptom Management Guidelines; Neurological of the disease itself (recurrent aspiration from impaired
Symptoms—Seizures, Fatigue, Delirium” swallow or interventions to manage symptoms—e.g., UTI
associated with catheterization).
General Problems • When treating infection, consider general principles (see
“Basic Principles”), routes available to administer medica-
GUIDELINE tion (PO, PEG, RIG), and likelihood of treatment success
(may be reduced if multiple-drug-resistant infection or
NG 97. Dementia: assessment, management and support for
people living with dementia and their carers. https://www.
irreversible underlying aetiology such as aspiration from
nice.org.uk/guidance/ng97. at-risk feeding).
• If treatment of underlying infection is not desired or
indicated, symptoms should be managed.
Muscle Spasms/Spasticity (See Also

• Most symptoms can be managed successfully, using the Section on Pain)
general guidelines above (see section on symptom control). • Further to pain-relieving measures as described previously,
• Needs can change rapidly and so require ongoing assess- the following may be considered in people with neuro-
ment and specialist input. degenerative disease causing painful muscle spasm:
• In addition to local palliative care services, local specialist • Gabapentin and clonazepam may also be used if other
services for people with advanced neurological diseases medications are poorly tolerated.
often exist. (e.g., specialist nurses/teams to support people • Botulinum toxin injections can be used for isolated
with Motor Neurone Disease, Multiple Sclerosis, Parkin- muscle spasm.
son’s Disease). • Physiotherapy input and specialist spasticity services
• There are a few specific considerations/medications out- are usually available locally.
lined in this section. • Midazolam CSCI may be required as the end of life
approaches.
Communication
• Patients should be reviewed by a speech and language Activities of Daily Living
therapist. • Equipment is available (usually via physiotherapy/occu-
• They should have access to both low-level (e.g., alphabet pational therapy services and/or specialist services for
board) and high-level (e.g., eye-gaze computer systems) people with advanced neurological disease) and should
augmentive and alternative communication (AAC) be provided to aid activities of daily living and
technologies. mobility.
• Deteriorating communication ability should be a trigger
for advance care planning (see “Basics Principles” i.e., Dementia
initial section of chapter).
• Dementia may be the primary diagnosis or comorbidity
Nutrition, Hydration, and Poor Swallow in a patient with life-limiting illness.
• Management of reduced oral intake should be considered • As with all illnesses, medications to address the underlying
early in the course of disease. If appropriate and desired, pathology are the mainstays of management.
procedures to provide long-term enteral access (e.g., PEG) • Advanced dementia may have associated complications,
should be planned and undertaken while a patient remains including:
well enough to undergo the procedure. • reduced nutritional intake and weight loss (forgetting
• Poor nutrition, anorexia/cachexia and aspiration may be to eat or poor swallowing);
inevitable consequences of the end stage of advanced neu- • recurrent infections (aspiration, debility, reduced fluid
rological disease; and where reversal of underlying cause is intake);
not possible, symptomatic treatment should be provided • behaviour that challenges and makes the patient at
(see “Care in the Last Days of Life”). risk to self or others.
• Worsening of symptoms may be due to loss of

Management of Behaviour That Challenges dopaminergic response. Optimize administration and
• Drug treatments for the control of violence, aggression, regular dosing of dopaminergic medications but
and extreme agitation should be used to calm the person consider stopping these at end of life/when of no
with dementia and reduce the risk of violence and harm, benefit.
rather than treat any underlying psychiatric condition. • Difficulty with swallowing may cause problems with
Aim to reduce agitation or aggression without causing administration of oral dopaminergic medications:
unnecessary sedation. • Madopar is available in dispersible formulations and/
• If required give medications for behaviour that challenges or other dopaminergic medications can be given via
orally first line, SC second line. However, IM injections PEG if present.
may be used if required. • Rotigotine transdermal patches are available and can
• If SC/IM preparations are needed, lorazepam, haloperidol, be used under specialist guidance.
or olanzapine should be used. Wherever possible, a single • Pain: optimize analgesia (see “Symptom Control—
agent should be used in preference to a combination. Pain”). Skeletal muscle relaxants (e.g., midazolam
• If a patient exhibiting agitation/delirium is at the end of CSCI) may be necessary, especially where dopaminergic
life, manage as for agitation (see “Emergencies”). medications can no longer be taken or are of little
• People with Alzheimer’s disease, vascular dementia, mixed benefit.
dementias, or dementia with Lewy bodies with severe • Nausea and vomiting. Domperidone and cyclizine are
non-cognitive symptoms (psychosis and/or agitated behav- preferred antiemetics (see section on symptom control
iour causing significant distress) may be offered treatment related to nausea and vomiting).
with an antipsychotic drug. (See also “Parkinson’s Disease • Hallucinations/delirium:
and Parkinson Plus Syndromes.”): • Exclude any potentially reversible causes.
• Target symptoms for treatment should be identified • Dopaminergic drugs are a potential cause.
and individual risk/benefit considered. Treatment should • Quetiapine (minimum 12.5 mg at night) is the only
be discussed with patients and/or those important to recommended antipsychotic.
them. • In advanced disease, where a parenteral route is required,
• The antipsychotic should be commenced at low dose levomepromazine +/− midazolam may be more appro-
and reviewed regularly (monthly). Monitor for neu- priate (see “Emergencies—Agitation”).
roleptic sensitivity (i.e., changes in cognition and
physical function). Motor Neurone Disease (MND)/Amyotrophic
Management of Reduced Oral Intake and Lateral Sclerosis (ALS)
Infection (See Earlier Discussion - 'Nutrition, GUIDELINE
Hydration & Poor swallow')
• Managing these complications of advanced dementia Motor neurone disease—assessment & management. NICE
should always be undertaken in context (i.e., with involve- clinical guideline 42. www.nice.org.uk.
ment of the patient, those important to the patient, and
with due consideration to the stage of underlying disease)
(see “Basic Principles”).
Parkinson’s Disease and Parkinson • Many principles of symptom management in MND are
Plus Syndromes common to other advanced life-limiting illness, as above,
but those in this section pertain specifically to motor
GUIDELINES neurone disease.
National Institute for Health and Care Excellence. (2006). Ventilatory Support
Parkinson’s disease: Diagnosis and management in primary
and secondary care. NICE clinical guideline 35. www.nice. • NIV. Patients can be offered NIV if they develop signs
org.uk. and symptoms of respiratory impairment (e.g., breathless-
NICE Pathway. (2014). Managing Parkinson’s disease. ness, weak cough/sniff, disturbed/nonrefreshing sleep,
http://pathways.nice.org.uk/pathways. nightmares, daytime drowsiness, morning headache,
fatigue, shallow breathing, recurrent chest infection, acces-
sory muscle use, abdominal paradox, orthopnoea, increased
respiratory rate).
• Advanced end-stage Parkinson disease may cause specific • This is set up and monitored according to patient wishes
symptomatic problems: Bradykinesia, rigidity, tremor, and with the local specialist multidisciplinary team (MDT)/
pain are possible. respiratory ventilation service. It can be stopped by a
• Goal of management is to optimize dopamine treatment/ patient with capacity at any time (see “Care in the Last
administration, while avoiding dopamine antagonists. Days of Life”).
• Many similarities exist between symptoms experienced

Cough in end-stage renal failure (ESRF) and other LLI toward
• Breath stacking (manual or assisted) and mechanical cough the end of life (e.g., pain, fatigue/lethargy, nausea/vomiting,
assist devices can be used to improve effectiveness of cough. anorexia).
This may be particularly helpful in clearing secretions • However, there are some symptoms specific to ESRF,
associated with infection. which are often a consequence of uraemia or drug toxic-
ity. For example:
End-Stage Renal Failure • pain due to renal disease, renal osteodystrophy, carpal
tunnel syndrome, osteoporosis, osteomyelitis, renal
neuropathy;
GUIDELINE
• calciphylaxis (painful tissue ischaemia due to calcifica-
Scottish Palliative Care Guidelines. (2015). Renal disease tion of small blood vessels);
in the last days of life. www.palliativecareguidelines.scot • itch;
.nhs.uk/.
• fluid overload;
• restless legs, muscle cramps.
• In addition to symptoms due to disease, the demands
of treatment can be a significant source of distress
for patients with advanced renal disease; these can be
• Renal failure is considered end stage when estimated a significant factor in patients choosing to withdraw
glomerular filtration rate (eGFR) falls to below from dialysis.
15 mL/min/1.73m2 (chronic kidney disease [CKD]
Stage 5). Indicators of Poor Prognosis (Being in the Final
• However, symptoms are common in patients with CKD4 Months to Year of Life) in Advanced and End-Stage
(eGFR 15–29 mL/min/1.73m2) and symptom control/ Renal Failure (Chronic Kidney Disease 4 or 5)
pharmacological considerations mentioned later are appro- • Symptomatic renal failure (e.g., nausea/vomiting, fluid
priate for all patients with eGFR below 30 mL/min. overload, anorexia, pruritus, declining performance status)
• Note on eGFR and creatinine clearance. Most pharma- in patients having conservative management
cokinetic studies used an estimate of creatinine clearance • Patients choosing conservative management
based on Coackroft-Gault equation. Dose adjustments • Poor tolerance of dialysis (e.g., hypotension preventing
of drugs were therefore based upon this. Estimated GFR haemodialysis)
(eGFR)—most often based on MDRD calculation—is • Progressive cachexia and decline in performance status
not the same as creatinine clearance, but an acceptable and increasing symptom burden despite dialysis
substitute when considering whether adjustments need • Patients declining or withdrawing from dialysis (Note:
to be made in the dosing of medications. eGFR is adjusted Average survival for patients discontinuing haemodialysis
to body surface area and as such should be used with varies but is likely in the range of several days to 2 weeks.)
caution if body size is unusually low or high. eGFR should
be taken as an indication of the need to modify drug Pharmacological Considerations in Chronic
doses. Kidney Disease 4 and 5
• Chronic kidney disease may be the underlying LLI or a
comorbidity. It is most often due to: NB: Modifications to medications are appropriate in any
• diabetes; patient with established acute kidney injury (eGFR <30) as
• hypertension, cardiovascular disease; well as those with CKD.
• renal disease (e.g., glomerulonephritis, polycystic kidney
disease [PKD]), infection, or obstructive uropathy; General Considerations
• malignancy (e.g., myeloma, tumour causing obstructive
uropathy, effects of chemotherapy); • Renal failure can reduce the oral absorption, alter the
• medications (e.g., lithium, long-term NSAIDs). distribution, and reduce the excretion of many different
• Patients with end-stage renal failure may be managed drugs (and/or their metabolites):
in a variety of ways: either conservatively or with a • Where possible, use medications that do not rely on
form of renal replacement therapy (peritoneal dialysis renal excretion.
or haemodialysis). • Nephrotoxic medications should be avoided if there
is some residual renal function.
Symptoms • Medications that rely on renal excretion should be
reduced in dose.
• Patients with end-stage renal failure have at least as many • Some medications will be removed by dialysis. In patients
symptoms as patients with advanced disease, cancer, pos- having dialysis, seek advice on medications from specialist
sibly more. teams.
• Detailed guidance on dose adjustment of medications in

renal failure can be found in the British National For- Medications Used to Manage Advanced
mulary (BNF), Palliative Care Formulary, or Summary Chronic Kidney Disease/End-Stage Renal Failure
of Product Characteristics (available online). (and Its Complications)
• In patients with end-stage renal disease estimated to be in • Calcium, vitamin D
their final hours to days of life: • Phosphate binders
• Side effects from potential accumulation of medica- • Diuretics
tions and/or their metabolites need to be weighed • Iron, erythropoietin: for anaemia
carefully against the need to achieve timely symptom • Antihypertensives
control, comfort, and dignity. • Secondary prevention of cardiovascular disease (e.g., statins,
• If there is a delay/difficulty in sourcing preferred 'renally antiplatelet therapy)
safe' medications, and patients are experiencing uncon- • Fluid restrictions
trolled symptoms/distress, then the priority should
be to provide symptom control with the medications Managing Medications Used in Advanced
available. Chronic Kidney Disease/ End-Stage Renal Failure
• In this situation smaller doses of less ideal media- • Medications for advanced CKD and its complications
tion (e.g., morphine/diamorphine) can be given less should be reviewed when it becomes clear that a patient
frequently. is deteriorating.
• Note: Patients with a previously normal renal function • Table 26.15 provides a guide as to when it may be appro-
dying from any cause may well develop renal failure/acute priate to discontinue these medications.
kidney injury (AKI) due to the physiologic processes that
occur as death approaches (e.g., reduced fluid intake). Management of Symptoms Specific to Advanced
In this patient group, modifications to well-tolerated, Chronic Kidney Disease/ End-Stage Renal Failure
established medications for symptom control (e.g., mor- • The following guidance is appropriate for all patients with
phine) are generally not needed. It is not appropriate eGFR below 30 mL/min/1.73m2 (CKD 4 or 5). It sug-
to monitor renal function in the final days of life. gests alternative medications for symptom management
in advanced renal disease. The general principles guiding
Symptom Management Specific to Advanced use of these medications are mostly described under the
Chronic Kidney Disease/End-Stage Renal general symptom control section. Please also refer to this
section.
Failure • Local practice may vary: Seek advice of local specialist
• As with most advanced LLI, maximizing therapy aimed palliative care team.
at managing the disease and its complications is an impor- • Patients undergoing dialysis: Seek specialist advice for
tant part of symptom management. these patients as some medications (e.g., gabapentin and
• As disease progresses and prognosis deteriorates it is impor- pregabalin) are cleared by dialysis.
tant to review these medications and discontinue them • Table 26.16 summarises the medications most appropriate
where possible. to use, and those best avoided.
TABLE
26.15 Management of Medications in Advanced Chronic Kidney Disease/End-Stage Renal Failure
Medication When to Consider Stopping

Medications for anaemia: iron, erythropoietin Stop in final weeks of life
Medications to maintain dialysis access (e.g., warfarin) Stop when dialysis stops
Medications to reduce cardiovascular risk (e.g., statin, aspirin) Stop when dialysis stops, or tablet burden felt to be too great
Calcium, vitamin D Stop when dialysis stops or when patient is no longer
swallowing
Phosphate binders Stop when patient is no longer eating
Diuretics Continue for as long as possible
Medications for symptom control (e.g., long-term analgesia, Continue while patient is able to swallow and replace where
antiemetics) possible with equivalent continuous subcutaneous infusion
medication until death
TABLE Summary of Medications for Symptom Control When Estimated Glomerular Filtration Rate Is Below
26.16 30 mL/min/1.73m2
Symptom Avoid Manage With

1. Pain Codeine, tramadol, NSAIDs (if any remaining Paracetamol, buprenorphine TD, fentanyl TD,
renal function), morphine, diamorphine, fentanyl SC/CSCI, alfentanil CSCI,
oxycodone (used in some centres; use methadone, amitriptyline (titrate slowly),
with caution) gabapentin (reduce dose), pregabalin
(reduce dose), ketamine (specialist use)
2. Anxiety, depression Venlafaxine, mirtazapine, duloxetine Sertraline, lorazepam/diazepam (reduced dose)
3. Nausea/vomiting Metoclopramide (long term) Domperidone, haloperidol (reduce dose),
cyclizine (reduce dose), ondansetron,
levomepromazine (reduce dose)
4. Restless legs — Clonazepam, gabapentin (reduced dose)
5. Muscle jerks (myoclonic) Diazepam (if possible) Lorazepam, clonazepam
6. Breathlessness Diazepam (if possible) Fentanyl SC PRN/CSCI, alfentanil CSCI,
lorazepam, midazolam PRN (reduced dose)
7. Delirium — Olanzapine, haloperidol (reduced dose)
8. Agitation — Midazolam (reduce doses), levomepromazine
(reduce doses)
9. Respiratory secretions Hyoscine hydrobromide Glycopyrronium, hyoscine butylbromide
10. Pruritus — Capsaicin, gabapentin
11. Seizures Phenobarbital Sodium valproate, midazolam CSCI
For doses of medications in the table above, please see the main text.
CSCI, Continuous subcutaneous infusion; NSAID, nonsteroidal antiinflammatory drugs; PRN, as needed; SC, subcutaneous.
Pain starting CSCI as considered twice as potent SC, com-

• Paracetamol: 500 mg to 1 g four times daily pared to PO); may require dose reduction in severe
• NSAIDs: ONLY for use in patients undergoing dialysis renal failure.
if they have no residual renal function • Others:
• Opioids: • Amitriptyline: start with low dose (10 mg at night)
• Buprenorphine transdermal (TD) patches: no dose and titrate slowly.
reduction required. Useful for chronic, stable pain. • Gabapentin: requires dose reduction. Creatinine clearance
Consider the time taken to titrate dose—use in (mL/min) 15 to 29 ml/min: 100 to 600 mg daily, titrated
patients with weeks to months to live to give time slowly. Creatinine clearance below 15 ml/min: 100 mg
for titration. alternate days to 300 mg daily. (See manufacturer’s
• Fentanyl TD patches: may require dose reduction: summary of product characteristics for guidance; these
titrate slowly. Useful for chronic, stable pain. Consider doses are both lower than manufacturer's guidance.)
the time taken to titrate dose—use in patients with • Pregabalin: requires dose reduction. Creatinine clear-
weeks to months to live to give time for titration. ance 15 to 29 ml/min: 25 to 150 mg once daily. Cre-
• Fentanyl injection SC PRN or CSCI: useful for opioid atinine clearance below 15 ml/min: 25 to 75 mg once
naïve patients with severe renal failure in the last days daily.
to week of life requiring rapid titration. Starting doses • Clonazepam: specialist use. May be helpful for nerve
typically 12.5 to 25 µg SC 1-hourly PRN and 100 µg pain. Dose 500 µg to 2 mg at night.
CSCI/24 h. • Ketamine: specialist use. Typical starting dose 5 to
• Alfentanil injection CSCI: useful for replacing other 10 mg PO four times daily.
opioids via CSCI at end of life, and those requiring
rapid opioid titration. Alfentanil SC is 10 times as Anxiety, Depression
potent as diamorphine SC (i.e., 10 mg diamorphine • Sertraline: no dose reduction required
SC is approximately equivalent to 1 mg SC alfentanil). • Lorazepam (severe anxiety): 500 µg 6-hourly
It is too short acting to use as SC PRN. • Diazepam (severe anxiety): avoid if possible. If necessary,
• Methadone: specialist use. May be used orally or start with reduced dose (e.g., 2.5 to 5 mg at night, titrate
converted to CSCI at end of life (halve oral dose if slowly.
Nausea/Vomiting End-Stage Respiratory Disease

• Manage according to likely cause (see section Symptom
Management Guidelines > GI—nausea etc…)
GUIDELINES
• Ondansetron: no dose reduction required (i.e., 4–8 mg
PO/SC twice daily National Institute for Health and Care Excellence. (2010).
Chronic obstructive pulmonary disease in over 16s:
• Cyclizine: no dose reduction required but may exacerbate
Diagnosis and management. NICE clinical guideline 101.
dry mouth, therefore start with 25 to 50 mg PO/SC www.nice.org.uk.
thrice daily National Institute for Health and Care Excellence.
• Haloperidol: reduce dose by 50%—start with 500 µg at (2011, updated March 2017). End of life care for adults. NICE
night quality standard 13. www.nice.org.uk.
Royal College of General Practitioners. (2016). Gold
• Metoclopramide: reduce dose by 50%—start with 5 mg
standards framework proactive identification guidance.
PO/SC thrice daily https://www.goldstandardsframework.org.uk/cd-content/
• Domperidone: start with low dose (e.g., 10 mg PO once uploads/files/PIG/NEW%20PIG%20-%20%20%20
or twice daily) 20.1.17%20KT%20vs17.pdf
• Levomepromazine: start with low dose (i.e., 6.25 mg PO/
SC at night and/or 6-hourly PRN)
Restless Legs • The end stage of respiratory disease is not clearly defined.
• Clonazepam: 500 µg PO at night, titrate slowly • However, when the underlying disease becomes unre-
• Gabapentin: dose reduced according to renal function as sponsive to usual medical treatment (resulting in persistent
mentioned above under 'pain'. or worsening symptoms) and estimated prognosis is less
than 1 year, these factors suggest that the patient is in
Muscle Jerks (Myoclonic) the 'end stages and approaching the end of life'.
• Lorazepam or clonazepam (doses as mentioned above)
Indicators of Poor Prognosis (Being in
Breathlessness the Final Months to Year of Life) in Advanced
• Opioids: fentanyl or alfentanil (doses as mentioned above Respiratory Disease
under 'Pain') • Frequent exacerbations and hospital admissions
• Benzodiazepines: lorazepam (doses as mentioned above) • Patients too unwell for pulmonary rehabilitation
or midazolam starting at 2.5 mg SC 1-hourly PRN (at • Patients who qualify for long-term oxygen therapy (LTOT)
end of life) • COPD with forced expiratory volume 1 (FEV1) less than
30% predicted
Delirium • Medical Research Council Dyspnoea Scale grade 4/5 (i.e.,
• See section on symptom management of neurological breathless on walking 100 yards or on minimal exertion)
symptoms, delirium. • Cachexia, weight loss, low body mass index (BMI)
• Olanzapine is an alternative, starting at 5 mg daily. • Comorbidities such as heart failure
Agitation Symptom Management Specific to End-Stage

• See section on "Emergencies—agitation". Respiratory Disease
• Lower doses of midazolam (starting at 2.5 mg SC 1-hourly)
and levomepromazine (6.25 mg SC 4-hourly) may be • Many symptoms experienced in end-stage respiratory
effective. disease can be managed according to general symptom
management guidelines for pain, breathlessness, cough,
Respiratory Secretions secretions, anxiety, and depression (as detailed above).
• Glycopyrronium 200 µg SC 4-hourly or hyoscine butyl- • It is important always to consider whether symptoms in
bromide 20 mg SC 4-hourly end-stage respiratory disease are reversible, for example:
• treating infection with antibiotics;
Pruritus • treating inflammation with steroids;
• See section on symptom management—skin, pruritus. • managing bronchospasm with salbutamol and
ipratropium.
Seizures at End of Life • Management options specific to the underlying cause of the
• See section on emergencies—seizures. respiratory disease should always be optimized (e.g., beta-2
• If CSCI required, midazolam is preferred. Phenobarbital agonists and muscarinic antagonists, corticosteroids).
should be avoided unless intractable seizures in last hours • When symptoms are no longer controlled by optimal
to days of life. medical treatment, palliative approaches should be
• Sodium valproate preferred if oral antiepileptic is appro- employed. It is appropriate to manage uncontrolled symp-
priate; dose unchanged. toms with opioids or benzodiazepines. Anxiety about
casing respiratory depression should not prevent use of • Heart failure may be the sole underlying life-limiting
these medications where needed. illness, but is often encountered as a comorbidity or
• Local specialist teams (Respiratory, Palliative Care) should consequence of another condition (e.g., cardiomy-
remain involved in supporting patients and their needs. opathy secondary to effects of chemotherapy or cor
pulmonale).
Oxygen in End-Stage Respiratory Disease • All management options for heart failure are designed to
• Oxygen may be helpful in the palliation of breathlessness improve symptoms; some will improve survival, none
due to hypoxia (see section on Symptom Management cure the disease.
Guidelines > Respiratory—breathlessness). • Severity of heart failure is classified according to the
• Appropriate use of oxygen: New York Heart Association (NYHA) functional
• Many patients will be on LTOT (PaO2 <7.3 kPa). system:
• If not, short-burst oxygen therapy (oxygen given inter- • Class I (no limitation of physical activity or symptoms,
mittently for 10–20 minutes at a time) can be con- but heart failure symptoms in the past) to class IV
sidered for breathlessness not responding to other (symptomatic at rest and discomfort from any physical
measures. activity).
• Use a 24% or 28% Venturi mask at a flow rate of 2 • NYHA grading allows assessment of symptomatic
to 4 L/min. response to treatment.
• Use oxygen with caution if symptoms of CO2 retention • Further classification can be based upon which side of
such as headache, lethargy, hand flap, and confusion. the heart is predominantly affected. Treatment is based
• As disease progresses, routine measurement of oxygen on whether left ventricular systolic dysfunction (reduced
saturations becomes less appropriate. In fact, it may cause LVEF) is present or not.
undue concern to patients for whom no remedial measures
are possible. Symptoms
• Focus should be switched to managing symptoms of • Many patients with heart failure experience similar symp-
breathlessness regardless of measured oxygen saturations. toms to those with advanced malignant disease, such as
breathlessness, pain, fatigue, and depression.
Anticipating Care Needs and Management • However, there are some symptoms of heart failure (such as
• Patients will often have extensive experience of hospital- fluid retention) that require disease-specific management.
ization for management of exacerbations with their condi- Disease-specific treatments are the mainstay of symptom
tion and may well have strong opinions on the treatment control.
they would or would not want. • Consequently, some modifications to general palliative
• If so, they should be encouraged to record an advance symptom management are required to address the differ-
care plan or advance decision to refuse treatment. ing aetiology of symptoms in heart failure (described
• For patients with respiratory disease, opportunity should below).
be created to discuss considerations such as whether they • The risk of sudden death in patients with heart failure
want further hospital admission, antibiotics (at home/in has declined, but this potential may still cause anxiety
hospital or not at all), and ventilation (were this to be and underlines the need for advance care planning.
considered appropriate). • Patients should be screened for depression.
• Clinical condition and symptom burden can deteriorate • Initial management is pharmacological. However, cardiac
rapidly in patients with advanced respiratory disease. For resynchronization therapy pacing (CRT-P) or defibrillator
those patients who choose to have symptoms managed (CRT-D) devices may improve cardiac function and
in the community, arrangements should be made to ensure symptoms.
anticipatory medication for symptom control/end-of-life
care is readily available to them at home. Indicators of Poor Prognosis (Being in
the Final Months to Year of Life) in Advanced
Heart Failure
End-Stage Heart Failure
• NYHA grade III/IV with symptoms becoming resistant
GUIDELINES
to maximum tolerated medications
• Increasing frequency of episodes of decompensation/
National Institute for Health and Care Excellence. (2010). hospitalization
Chronic heart failure in adults. NICE clinical guideline 108.
www.nice.org.uk/.
• No further possible interventions
CIED Working Group. (2016). Cardiovascular implanted • Complications of heart failure or medications used to
electronic devices in people towards the end of life, during manage it (e.g., renal failure, hypotension at rest,
CPR and after death: Guidance from the Resuscitation hyponatraemia)
Council (UK), British Cardiovascular Society and National • Anaemia
Council for Palliative Care. Heart. doi:10.1136/
heartjnl-2016-309721.
• Life-limiting comorbidity
• Factors common to other LLI (i.e., cachexia, declining
performance status)
Symptom Management Specific to End-Stage • Antiarrhythmic: Continue if symptomatic tachycardia

Heart Failure present. Otherwise, these can be discontinued relatively
early.
• Medications designed to manage disease should be con- • Antianginals. Can be stopped if no angina.
tinued for as long as possible as they all have symptomatic • Antihypertensives become less important in advanced
benefit. cardiac failure. Stop if symptomatic hypotension.
• Digoxin should be stopped in renal failure, and in
Medications Used to Manage Heart Failure (and patients with sinus rhythm.
Its Symptoms)
• In patients with left ventricular systolic dysfunction: Secondary Prevention
• First line: ACE inhibitors and beta blockers • Cholesterol-lowering drugs can be discontinued early.
• Second line (if symptoms despite first line medications):
an aldosterone antagonist or an angiotensin II receptor Pharmacological Considerations in
antagonist
• Digoxin if worsening heart failure despite first and
End-Stage Heart Failure
second line treatment Medications to Avoid
• Sacubitril valsartan (Entresto) • Some medications may worsen symptoms and are best
• In all types of heart failure: avoided; but a pragmatic approach must also be applied
• Diuretics for relief of fluid retention/congestive symptoms to symptom control—particularly for patients believed
• Amlodipine for hypertension and/or angina to be in the last days to week(s) of life.
• Aspirin in atherosclerotic disease • Concurrent renal failure may necessitate careful consid-
• Amiodarone, anticoagulants may be used eration of medications used to control symptoms, such
as opioids (see section on renal failure).
Managing Cardiac Medications to Improve • Medications to avoid where possible:
Symptoms in Advanced End-Stage Heart Failure • NSAIDs and cyclooxygenase 2 (COX-2) inhibitors:
(Table 26.17) can exacerbate heart failure and fluid retention and
• Medications that improve survival and symptoms: may cause renal toxicity
• Aldosterone antagonists, ACE inhibitors, angiotensin • Antimuscarinic medications, including cyclizine
receptor blockers, beta blockers • Tricyclics: may exacerbate arrhythmias
• Continue these for as long as possible • Corticosteroids, progestogens
• Consider dose reduction if symptomatic hypotension, • Medications that prolong the QT interval
worsening renal impairment, or tablet burden
• Medications that improve symptoms:
Managing Cardiac Devices
• Loop diuretics: Continue unless patients become clini-
cally hypovolaemic or anuric. To symptomatically Types of Implantable Cardiovascular Implanted
manage fluid overload at the end of life. Furosemide Electronic Devices
may be given by CSCI, starting at a CSCI dose over • Patients with heart disease/failure may have one of several
24 hours identical to their previous oral dose. implantable devices. It is important to consider which
type of device a patient has, as they are managed differ-
ently at end of life.
• Commonly encountered devices can be categorized as
TABLE Management of Medications in Advanced follows:
26.17 Cardiac Failure • Pacemakers for bradycardia
Medications That Can Medications to Continue • Cardiac resynchronization therapy devices: These are
Be Stopped Early as Long as Possible biventricular pacemakers, used primarily for managing
cardiac failure. If purely for pacing this is termed CRT-P.
Cholesterol-lowering Loop diuretics If it includes additional defibrillator component for
drugs Note: Furosemide can be
continued continuous patients at risk of ventricular arrhythmia causing sudden
subcutaneous infusion if death this is termed CRT-D.
required (see text) • Implantable cardioverter defibrillator (ICD): for treat-
Antihypertensives ACE inhibitors ment of ventricular arrhythmia
• Patients with cardiac failure may have either CRT-P,
Digoxin (if in sinus Beta blockers CRT-D, or ICD.
rhythm or renal failure)
• Note: More unusually, other devices with external
Antiarrhythmic (if no Angiotensin receptor components such as a left ventricular assist device
symptomatic blockers (LVAD) may be present. Specialist advice should be
arrythmia)
Aldosterone antagonists sought on the management of these in patients at the
end of life.
• Deactivation itself is undertaken by a cardiac physiolo-

Deactivation of Defibrillator Component of gist who should be able to see the patient in his or
Devices at End of Life her own home if necessary.
• The aim of deactivating the defibrillator function is to • Emergency deactivation:
prevent inappropriate shocks at the end of life. • Where there is no time to arrange a cardiac physiolo-
• Defibrillation shocks may be delivered by ICD or CRT-D gist, temporary defibrillator deactivation can be achieved
devices. by placing a doughnut-shaped magnet over the device
• When the defibrillator function is deactivated, any pacing and taping it securely in place.
function is normally left unchanged. • This should only be used only as a temporary measure
• Deactivation of a defibrillator is not the same as a Do while full deactivation is being arranged.
Not Attempt Cardiopulmonary Resuscitation (DNACPR) • The magnet will not deactivate the pacemaker
decision. Both require full discussion when possible. function.
• Discussion about the circumstances in which the defibril-
lator function of a device may be turned off should be Deactivation of Other Pacemaker Function at End
started prior to implantation: of Life
• This should be revisited as a patient’s condition • Pacemakers for bradycardia: These are not normally deac-
deteriorates. tivated (as they aid symptom control), unless specifically
• A competent patient can make a decision for deactiva- requested by a patient.
tion at any stage. • CRT-P: Not normally deactivated as beneficial in symptom
• It may be recorded in an advance care plan. control.
• It should be explained to patients that deactivation is • In rare circumstances, patient may request deactivation
not painful, death is not likely to be immediate, and of his or her pacemaker. This should be a shared, informed
the pacemaker function will not be deactivated. decision and deactivation undertaken by a cardiac physi-
ologist as for a defibrillator.
Practicalities of Defibrillator Deactivation • Note: CRT-P and pacemakers are not deactivated by
• Planned deactivation (in the community): magnets.
• This should come as a shared, informed decision, often
after several discussions with the patient, those impor- After Death
tant to the patient, and multidisciplinary teams involved • An active pacemaker (including CRT-P) needs no imme-
in care. diate management.
• The patient’s informed consent should be recorded • If still active, an ICD/CRT-D should be fully deactivated
(or—if made in the person’s best interests—appropriate by a cardiac physiologist as soon as possible after death
people involved, and documentation completed). and before attempts are made to remove the device.
• The general practitioner, specialist nurse, or palliative • All cardiovascular implanted electronic devices should be
care team should liaise with local cardiac services to explanted prior to cremation. They are usually then
arrange deactivation. returned to local device services for disposal.
Appendix 1
Routine Schedule of Immunizations
Age Immunization
2 months DTaP/IPV/Hib and PCV and MenB and Hep B
Rotavirus (oral drops)
3 months DTaP/IPV/Hib/Hep B
Rotavirus (oral drops)
4 months DTaP/IPV/Hib/Hep B and MenB and PCV
12–13 months Hib/MenC and MMR and PCV and MenB
2–8 (up to 18 for children in clinical risk groups) years annually. Nasal flu spray
Given at school to children from reception to year 4.
3 years 4 months–5 years (preschool) DTaP/IPV, or dTaP/IPV, and MMR
12–13 years (girls only) HPV (2 doses given 6–24 months apart)
14 years (school year 9) Td/IPV and MenACWY. Check MMR status and vaccinate
if necessary.
65 onwards Flu annually and PCV once
70 years Shingles
aP, Acellular pertussis; D, diphtheria; d, low-dose diphtheria; flu, influenza; Hep B, hepatitis B; Hib, Haemophilus influenzae b; HPV, human papillomavirus;
IPV, inactivated polio vaccine; MenACWY, meningitis A,C,W,Y; MenB, meningitis B; MenC, meningococcal C; MMR, mumps, measles, rubella; PCV, pneumo-
coccal vaccine; T, tetanus.
471
Appendix 2
Incubation Period and Infectivity of
Common Diseases
Disease Incubation Period Period of Infectivity Exclusion From School or Nursery
Bacillary dysentery 1–7 days Mean 7 days For 48 hours after last diarrhoea
(shigellosis)
Campylobacter 1–10 days 1–3 weeks For 48 hours after last diarrhoea
Cryptosporidiosis 1–14 days 2–4 weeks For 48 hours after last diarrhoea
Enteroviral infection 2–3 days 1–2 weeks None
Escherichia. coli enteritis 2–48 hours or longer 12 days or longer For 48 hours after last diarrhoea
Gastroenteritis (rotaviral) 2–4 days 6–10 days 48 hours from last diarrhoea or vomiting
Gastroenteritis (adenoviral) 8–10 days 7–14 days 48 hours from last diarrhoea or vomiting
Gastroenteritis (Norwalk 4–77 hours 0–3 days 3 days after onset
virus)
Gastroenteritis N/A N/A 48 hours from last diarrhoea or vomiting
(unidentified)
Giardiasis 5–20 days 2 weeks 24 hours from last diarrhoea
Salmonellosis 4 hours to 5 days Adults 4 weeks (median) For 24 hours after last diarrhoea
Typhoid and paratyphoid 3–56 days 2 weeks to indefinite Until 24 hours after last diarrhoea
Other Diseases
Chickenpox 11–20 days –4 to +5 days 5 days from start of rash
Conjunctivitis 3–29 days 2 weeks None
Haemophilus influenzae 4–5 days Indefinite (untreated) 24 hours from start of antibiotics
Head lice N/A Indefinite (untreated) None
Hand, foot, and mouth 3–5 days 7 days None
disease
Hepatitis A 2–6 weeks –17 days to +2 weeks Exclude until 7 days after onset of jaundice
or symptom onset if there is no jaundice
Hepatitis B 6 weeks to 6 months None (although may be too ill to attend
or longer during acute infection)
Hepatitis C 2 weeks to 6 months None
Herpes simplex 1–6 days 1–8 weeks (primary infection) None
1–3 days (recurrence)
Impetigo N/A N/A Until lesions crusted or 48 hours after
starting antibiotics
Infectious mononucleosis 33–49 days At least 2 months None
Influenza 1–3 days 3–7 days None
472
APPENDIX 2 Incubation Period and Infectivity of Common Diseases 473
Disease Incubation Period Period of Infectivity Exclusion From School or Nursery

Measles 9–18 days –2 to +3 days From prodromal symptoms to 4 days after
the onset of the rash
Meningococcal disease N/A Indefinite (untreated) <2 days For the duration of the illness
(treated)
Meningococcal infection 2–10 days Until 48 hours after starting treatment
Mumps 15–24 days Days –6 to +4 3 days before to 5 days after the start of
the swelling
Pertussis 5–21 days ≥6 weeks, or 1 week if given 48 hours after starting antibiotic treatment
a macrolide if they feel well enough to attend, or
21 days after onset of illness if no
antibiotics given
Rabies 9 days to 9 weeks Until death N/A
(possibly up to 2
years)
Rubella 15–20 days Day 1–6 1 week before the rash appears to 6 days
after
Scabies 7–27 days Indefinite until treated Until after first treatment
Scarlet fever 1 –5
2 days 3 days if treated 24 hours after starting antibiotics
Slapped cheek disease 13–18 days –6 to –3 (i.e., prodrome only) None
Streptococcal pharyngitis 1 –5
2 days Indefinite (untreated) None
Tetanus 4–21 days Not contagious None
Threadworms 2–4 weeks Indefinite None
Tinea 2–4 weeks Indefinite None
Tuberculosis 4 weeks to years Smear positive: until 2 weeks Until 2 weeks after starting treatment. No
after starting treatment exlusion necessary for nonpulmonary Tb
Warts 1–24 months While present None
Appendix 3
A Suggested Table of Immunizations
for Travel
This schedule is for an adult who has been fully immunized as a child according to the UK recommendations. Few travellers will
need all the immunizations below; they should only be given if appropriate to the travel planned. Individual health problems and
exposure risks should always be taken into account. Other vaccines such as tuberculosis (TB) and chickenpox may be needed.
Day 0 Rabies, Japanese encephalitis, tickborne encephalitis, hepatitis B

Day 7 Rabies, Japanese encephalitis
Day 14 Tickborne encephalitis
Day 28 Rabies, Japanese encephalitis, hepatitis B
At some point in the above schedule (either together or BCG, cholera, hepatitis A, meningococcal ACWY, polio, tetanus,
spread out over the month). Immunization early in the and diphtheria (as Td), typhoid, yellow fever
month (at least 1 week before departure) will allow time
for immunity to develop.
If exposure to risk continues, further immunizations against hepatitis B will be needed at 2 months and at 1 year from the first
dose, and against tickborne encephalitis at 9 months to 1 year after the last dose.
474
Appendix 4
Notification of Infectious Diseases
The notification of the following diseases is required by law in the United Kingdom, and the doctor is not excused from notification
by considerations of confidentiality. The following list applies to England and Wales, with variations for Scotland and Northern
Ireland indicated.
Acute Encephalitis Malaria*

Acute Infectious Hepatitis* Measles
Acute Meningitis Meningococcal Septicaemia
Acute Poliomyelitis Mumps
Anthrax Plague
Botulism† Rabies
Brucellosis† Rubella
Cholera Scarlet Fever*
Diphtheria Severe Acute Respiratory Syndrome (SARS)†
Enteric Fever (typhoid or paratyphoid fever) Smallpox
Food Poisoning* Tetanus
Haemolytic Uraemic Syndrome† Tuberculosis
Infectious Bloody Diarrhoea*,† Typhus*
Invasive Group A Streptococcal Disease*,† Viral Haemorrhagic Fever (VHF)
Legionnaires Disease* Whooping Cough
Leprosy*,† Yellow Fever
*Not in Scotland
†
Not in Northern Ireland
Also notifiable in Scotland: Clinical syndrome due to E. coli 0157 infection. Haemophilus influenza b. Necrotizing fasciitis. Tularaemia. West Nile Fever.
Also notifiable in Northern Ireland: Chickenpox. Dysentery. Gastroenteritis <2 years. Leptospirosis. Relapsing Fever.
475
Appendix 5
Child Health Promotion
History and Examination Health Education

Neonatal Examination
a. Elicit and consider concerns expressed by the parents a. Feeding and nutrition
b. Sleeping position
b. Review family history, pregnancy, and birth c. Baby care
c. Assess risk of hearing defect and refer accordingly d. Sibling management
e. Crying and sleep problems
d. Full physical examination, including weight and head circumference f. Transport in a car
g. Advice on reducing risk of SIDS
e. Check for CDH and testicular descent
f. Inspect eyes, check red reflex
g. Check PKU, thyroid tests, cystic fibrosis, medium chain acyl-CoA deficiency
have been done/are organized
h. Screen for haemoglobinopathy, if relevant
i. Vitamin K according to protocol
j. Consider need for BCG and Hep B vac
First 2 Weeks
In addition to the neonatal examination:
a. Assess the level of support and assistance that each new parent is likely to a. Nutrition
require b. The effects of passive smoking
b. Newborn hearing test c. Accident prevention—bathing, scalding by
feeds, fires
d. Immunization
6–8 Weeks
a. Check history, review growth and development, and ask about parental a. Immunization
concerns b. Nutrition
c. Dangers of fires/falls/overheating/scalds
b. Physical examination, weight, head circumference (and length, if indicated); d. Recognition of illness and what to do
testes in boys
c. Check for CDH
d. Enquire about concerns regarding vision, squint, and hearing
e. Check whether the baby is in the high-risk category for hearing loss and
refer if necessary
f. Discuss and perform immunizations
2, 3, and 4 Months and 13 Months
a. Primary immunizations
476
APPENDIX 5 Child Health Promotion 477
History and Examination Health Education

6–9 Months
a. Enquire about parental concerns regarding health and development, vision, a. Accident prevention—choking, scalds,
and hearing and burns (including sunburn), falls
b. Look for evidence of CDH b. Anticipate increased mobility (eg, safety
c. Check for testicular descent gates, guards)
d. Observe visual behaviour and look for squint c. Nutrition
d. Dental prophylaxis
e. Distraction test for hearing (HV) e. Reinforce advice about safety in cars and
f. Infant feeding and obesity risk passive smoking
f. Developmental needs
18–24 Months
a. Enquire about parental concerns particularly regarding behaviour, vision, and a. Accident prevention—falls from heights,
hearing drowning, poisoning, road safety
b. Confirm that the child is walking with a normal gait, and that speech and b. Nutrition
comprehension are appropriate for age c. Developmental needs—language and play
c. Arrange detailed vision, hearing, or language assessment if indicated d. Need to mix with other children—
d. Remember the prevalence of iron deficiency anemia playgroup, etc.
e. Avoidance and management of behaviour
problems
e. Measure height
Note: Inform the community paediatric services if there is any anxiety about a child’s educational potential.
36–54 Months
a. Enquiry and discussion about vision, squint, hearing, behaviour, language a. Accidents—fires, roads, drowning
acquisition, and development b. Road safety
b. Discuss, if appropriate, whether the child is likely to have special educational c. Preparation for school
problems and refer as appropriate d. Nutrition and dental care
c. Measure the height and chart it
d. Refer for hearing test if concerned
Department of Health. (2004). National service framework for children, young people, and maternity services. London: DoH.
Appendix 6
Stages of Child Development
Summary of Development: Birth to 16 Weeks

0–4 Weeks 6–8 Weeks 12–16 Weeks
Social Watches mother and may smile Responsive smile by 6 weeks Recognizes family, shows
and vocalizes pleasure
Motor
Ventral suspension Head hangs down until 3–4 Head held in horizontal plane, Head maintained well above plane
weeks then up momentarily and briefly up by 8 weeks of body by 12 weeks
Prone Head to side, pelvis high, knees Chin up intermittently at 6 weeks, Head and shoulders up, and
drawn up under abdomen well up at 8 weeks chest by 16 weeks
Pelvis flat Weight on forearms
Supine Head to side, limbs flexed or ATNR posture common but head ATNR declining
ATNR posture to midline by 8 weeks Head and hands now to midline
Pull-to-sit Complete head lag Less head lag Slight head lag
Held sitting Very round back Back rounded Back straighter and head up
Head drops forward Head briefly up
Held standing Walking and placing reflexes Sags at hips and knees, getting Increasingly bears weight on legs
present until 6 weeks head up by 8 weeks
Hands Strong grasp reflex Grasp reflex present but slight by 8 Grasp reflex fades between 12
Hands often closed weeks and 16 weeks
Fingers extend more often Can hold rattle briefly
Vision Blink and pupil reflexes Smoother conjugate eye Follows through 130 degrees
Eye righting reflex movements Hand regard common (12–20
Random movements but can Fixates on face/objects and weeks)
fixate follows through 45–90 degrees
Vocalization/hearing Cries, stills, or startles to Eyes turn to sounds Varied coos, squeals, and laughs
sounds Starts vocalizing Turns to sounds
ATNR, Asymmetric tonic neck reflex.
478
APPENDIX 6 Stages of Child Development 479
Summary of Development: 4–10 Months

4–6 Months 6–8 Months 8–10 Months
Personal and Responsive to all comers Discriminates between family and strangers Wary of strangers
social Smiles at self in mirror Attracts attention Waves bye-bye
behaviour Excited at approach of food Hand feeds biscuit Attempts to use spoon
Gross motor No head lag in traction Lifts head up in supine Sits steadily, pivots, and leans
Rolls prone to supine Rolls supine to prone/creeps Can get from prone to sitting
Back straight in supported Sits without lateral support Pulls self to stand and crawls
sitting Bears weight on feet (5–8 months)
Fine motor Reaches and grasps toys Transfers cube hand to hand (5–7 months) Pincer grasp of pellet (8–12
and vision (4–6 months) Can hold two cubes months)
Plays with toes Any squint reported after 6 months is Releases object and looks for
Very alert visually abnormal it (7–11 months)
Points at 1 mm sweet
Language and Varied sounds and squeals Starts to babble da-da Varied babble ma-ma, ba-ba,
hearing Consonants such as ba or da Turns to sounds (4–8 months) da-da
Indicates and understands
“no” Locates sounds well
Summary of Development: 12–24 Months

12–15 Months 18–24 Months
Personal and Shows affection and may be shy Becoming egocentric, clinging, and resistant
social behaviour Indicates wants, points, claps hands (10–18 months) Loves domestic mimicry
Mouthing stops (12–15 months) Definitely stopped mouthing and casting (by 18
Enjoys casting (12–15 months) months)
May manage cup and spoon with spills (10–17 Helps undress
months) Independent with cup and spoon (15–24 months)
Gross motor Walks holding on (8–12 months) Walks well (12–18 months)
Walks alone (11–15 months) Climbs stairs, kneels (14–22 months)
Fine motor and Fine pincer grasp May show hand preference (after 15 months)
vision Bangs bricks together (8–14 months) Builds 2 to 3 cubes
Holds two cubes Turns pages (15–24 months)
Scribbles (12–18 months)
Language and Mama, Dada, with meaning (9–15 months) Can point to three parts of body, has 6–20 words
hearing Three to four clear words (12–18 months) and jargon (15–24 months)
480 APPENDIX 6 Stages of Child Development
Summary of Development: 2–5 Years

2–3 Years 3–4 Years 5 Years Old
Personal and Enjoys solitary play, alongside peers, Plays with peers, sharing toys Plays complicated
social skills not sharing Enjoys make-believe play cooperative games Makes
Possessive: tantrums if thwarted Shows concern and sympathy for friends
Feeds quite neatly, using spoon and others, and able to take turns by Comforts playmates and
fork or fingers 4 years siblings in distress
May be clean and dry by day, with Easily manages spoon and fork and Almost completely
supervision, or may refuse to then knife independent in self-help
cooperate Mostly dry day and night skills now
Can wash hands, dress and Can carry out simple
undress by 4 years, except domestic tasks and run
fastenings errands
Gross motor Now very mobile Up stairs one foot per step at 3, Enjoys running, jumping,
Runs, kicks ball, tries to throw and down by 4 years climbing, swings and
Walks up and down stairs two feet to Can walk, then run on tip-toe, and slides, and starting to play
a step hop, by 4 years ball games
Propels tricycle by pushing with feet on Enjoys climbing, pedals a tricycle Can stand on one leg, hop
floor skillfully 10 times, and heel-toe
walk a narrow line
Fine motor Neat prehension, and controlled Tower of 9–10 and imitates 3 cube Can write name, copy a
and vision release bridge at 3 years, steps or gate square and a triangle
Tower of 6–8 bricks by 4 years Draws man with detailed
Holds pencil in fist Awkward tripod grasp of pencil at 3 features and limbs
Circular scribble (24 months) copies years—copies circle and imitates Can fold paper and use
vertical line, imitates circle (30 cross scissors to cut out shapes
months) Dynamic tripod after 4 years; draws Performs Snellen chart type
Simple puzzles and can thread large man with head, trunk, and legs of vision test
beads Can do letter-matching vision tests,
Difficult age to test vision using linear charts, each eye
Recognizes two-dimensional symbols separately, by 3 12 to 4 years
and may match letters at 30 months
Language Listens to simple stories and Intelligible but immature speech, Enjoys riddles and jokes
and hearing understands two-part instructions 3–5 word sentences, knows Understands negatives and
Can say 50–100 single words and join name and sex, at 3 years complex questions and
two to three (“Daddy gone car”) Long stories, constant more instructions
Many questions: what? and who? abstract questions, grammar Gives long descriptions and
Long monologues, still some jargon, mostly correct, and speech clear, explanations
enjoys nursery rhymes and jingles by 4 years Speech easily intelligible with
Toy tests of hearing or may point to Knows age and address, 6+ few errors
named pictures colours and can count to 4+ Manages full audiometry and
Can do cooperative (conditioned) speech discrimination now
hearing tests
Appendix 7
Stages of Puberty
A Female breast changes
BI BII BIII BIV BV

Prepubertal Breast bud Juvenile smooth Areola and papilla Adult
contour project above breast
B Pubic hair changes—female and male
PHI PHII PHIII PHIV PHV

Preadolescent Sparse, pigmented, Dark, coarser, Filling out Adult in quantity
No sexual hair long, straight, mainly along curlier toward adult and type with spread
labia and at base of penis distribution to medial thighs in male
C Male genital stages
GI GII GIII GIV GV

Preadolescent Lengthening of Further growth in Development of Adult genitalia
penis length and glans penis, darkening
circumference of scrotal hair
481
482 APPENDIX 7 Stages of Puberty
(A) The stages of breast development in a female. Stage with distribution of the horizontal (or classically feminine)
1 (BI): Preadolescent: elevation in papilla only. Stage 2 (BII): pattern. Spread to medial surface of thighs but not up linea
Breast bud stage: elevation of breast and papilla as a small alba or elsewhere above the base of the inverse triangle (spread
mound. Enlargement of areolar diameter. Stage 3 (BIII): up linea alba occurs later and is rated Stage 6).
Further enlargement and elevation of breast and areola, with (C) Male genital development. Stage 1 (GI): Preado-
no separation of their contours. Stage 4 (BIV): Projection lescent: testes, scrotum, and penis are of about the same size
of areola and papilla to form a secondary mound above the and proportion as in early childhood. Stage 2 (GII): Enlarge-
level of the breast. Stage 5 (BV): Mature stage; projection ment of scrotum, and testes. Skin of scrotum reddens and
of papilla only, due to recession of the areola to the general changes in texture. Little or no enlargement of penis at this
contour of the breast. (This last stage may not be reached stage. Stage 3 (GIII): Enlargement of penis, which occurs
in women until after their first pregnancy.) at first mainly in length. Further growth of testes and scrotum.
(B) Pubic hair development: male and female. Stage 1 Stage 4 (GIV): Increased size of penis with growth in breadth
(PHI): Preadolescent. The vellus over the pubes is not further and development of glans. Testes and scrotum larger; scrotal
developed than that over the abdominal wall (i.e., no pubic skin darkened. Stage 5 (GV): Genitalia adult in size and
hair). Stage 2 (PHII): Sparse growth of long, slightly pig- shape. (The volume of the adult testis varies in size from 12
mented downy hair, straight or slightly curled, chiefly at the to 25 mL.)
base of the penis or along the labia. Stage 3 (PHIII): Con- Both sexes: axillary hair. Stage 1: Preadolescent. No
siderably darker, coarser and more curled. The hair spreads axillary hair. Stage 2: Scanty growth of slightly pigmented
sparsely over the junction of the pubes. Stage 4 (PHIV): hair. Stage 3: Hair adult in quality and quantity.
Hair now adult in type, but area covered is still considerably
smaller than in the adult. No spread to the medial surface With permission from Lissauer, T., & Clayden, G. (2005). Illustrated
of the thighs. Stage 5 (PHV): Adult in quantity and type textbook of paediatrics (3rd ed.). London: Mosby.
Appendix 8
Predicted Normal Peak Flow Values in
Children (Under 15 Years of Age)
Height
(cm) (ft–in) Peak Flows (L/min)
91 3–0 100
99 3–3 120
107 3–6 140
114 3–9 170
122 4–0 210
130 4–3 250
137 4–6 285
145 4–9 325
152 5–0 360
160 5–3 400
168 5–6 440
175 5–9 480
483
Appendix 9
Peak Expiratory Flow in
Normal Subjects
660 660
75 190 Men
650 650
72 183
640 640
69 175
630 630
620 66 167 620
610 610
63 160
Ht. Ht.
600 600
(in.) (cm)
590 590
580 580
570 570
560 560
550 550
540 540
530 530
520 520
Standard deviation men = 48 L/min
510 510
Standard deviation women = 42 L/min
500 500
69 175 Women
490 490
480 66 167 480
470 63 160 470
460 60 152 460
450 145 450

57
Ht. Ht.
440 440
(in.) (cm)
430 430
In men values of PEF up to 100 L/min less than
420 predicted and in women less than 95 L/min less than 420
predicted are within normal limits
410 410
400 400
390 390
380 380
PEF
(L/min)
15 20 25 30 35 40 45 50 55 60 65 70
Age (years)
484
Appendix 10
FEV1/FVC Charts
Females
Height (m)
Age (Years) 1.45 1.50 1.55 1.60 1.65 1.70 1.75 1.80
25 FEV1 2.5 2.7 2.9 3.1 3.4 3.6 3.8 4.0
FVC 2.9 3.1 3.3 3.6 3.8 4.0 4.2 4.4
30 FEV1 2.4 2.6 2.8 3.0 3.2 3.4 3.7 3.9
FVC 2.8 3.0 3.2 3.4 3.6 3.9 4.1 4.3
35 FEV1 2.3 2.5 2.7 2.9 3.1 3.3 3.5 3.7
FVC 2.6 2.9 3.1 3.3 3.5 3.7 4.0 4.2
40 FEV1 2.1 2.3 2.6 2.8 3.0 3.2 3.4 3.6
FVC 2.5 2.7 2.9 3.2 3.4 3.6 3.8 4.0
45 FEV1 2.0 2.2 2.4 2.6 2.9 3.1 3.3 3.5
FVC 2.4 2.6 2.8 3.0 3.3 3.5 3.7 3.9
50 FEV1 1.9 2.1 2.3 2.5 2.7 2.9 3.2 3.4
FVC 2.2 2.5 2.7 2.9 3.1 3.3 3.6 3.8
55 FEV1 1.8 2.0 2.2 2.4 2.6 2.8 3.0 3.2
FVC 2.1 2.3 2.6 2.8 3.0 3.2 3.4 3.7
60 FEV1 1.6 1.8 2.1 2.3 2.5 2.7 2.9 3.1
FVC 2.0 2.2 2.4 2.6 2.9 3.1 3.3 3.5
65 FEV1 1.5 1.7 1.9 2.1 2.4 2.6 2.8 3.0
FVC 1.8 2.1 2.3 2.5 2.7 3.0 3.2 3.4
70 FEV1 1.4 1.6 1.8 2.0 2.2 2.4 2.7 2.9
FVC 1.7 1.9 2.2 2.4 2.6 2.8 3.0 3.3
485
486 APPENDIX 10 FEV1/FVC Charts
Males
Height (m)
Age (Years) 1.55 1.60 1.65 1.70 1.75 1.80 1.85 1.90
25 FEV1 3.4 3.6 3.8 4.1 4.3 4.5 4.7 5.0
FVC 3.9 4.2 4.5 4.8 5.1 5.4 5.7 6.0
30 FEV1 3.3 3.5 3.7 3.9 4.2 4.4 4.6 4.8
FVC 3.8 4.1 4.4 4.7 5.0 5.3 5.5 5.8
35 FEV1 3.1 3.3 3.6 3.8 4.0 4.2 4.5 4.7
FVC 3.7 4.0 4.3 4.5 4.8 5.1 5.4 5.7
40 FEV1 3.0 3.2 3.4 3.6 3.9 4.1 4.3 4.5
FVC 3.6 3.8 4.1 4.4 4.7 5.0 5.3 5.6
45 FEV1 2.8 3.0 3.3 3.5 3.7 3.9 4.2 4.4
FVC 3.4 3.7 4.0 4.3 4.6 4.9 5.2 5.4
50 FEV1 2.7 2.9 3.1 3.3 3.6 3.8 4.0 4.2
FVC 3.3 3.6 3.9 4.2 4.4 4.7 5.0 5.3
55 FEV1 2.5 2.8 3.0 3.2 3.4 3.7 3.9 4.1
FVC 3.2 3.5 3.7 4.0 4.3 4.6 4.9 5.2
60 FEV1 2.4 2.6 2.8 3.1 3.3 3.5 3.7 4.0
FVC 3.0 3.3 3.6 3.9 4.2 4.5 4.8 5.0
65 FEV1 2.2 2.5 2.7 2.9 3.1 3.4 3.6 3.8
FVC 2.9 3.2 3.5 3.8 4.1 4.3 4.6 4.9
70 FEV1 2.1 2.3 2.5 2.8 3.0 3.2 3.4 3.7
FVC 2.8 3.1 3.3 3.6 3.9 4.2 4.4 4.8
Girls Boys
Height Height
Metres Inches FEV1 FVC Metres Inches FEV1 FVC
0.80 32 0.41 0.46 0.80 32 0.40 0.46
0.90 35 0.56 0.64 0.90 35 0.56 0.65
1.00 39 0.75 0.86 1.00 39 0.76 0.89
1.10 43 0.98 1.11 1.10 43 1.00 1.17
1.20 47 1.24 1.43 1.20 47 1.28 1.52
1.30 51 1.55 1.79 1.30 51 1.61 1.92
1.40 55 1.90 2.20 1.40 55 2.00 2.38
1.50 59 2.29 2.68 1.50 59 2.43 2.92
1.60 63 2.73 3.22 1.60 63 2.93 3.53
1.70 67 3.23 3.82 1.70 67 3.49 4.22
1.80 71 3.77 4.48 1.80 71 4.11 4.99
Appendix 11A
Summary of Management of Asthma
in Adults
Reproduced from Scottish Intercollegiate Guidelines Network and British Thoracic Society. (2016). British guideline on the
management of asthma.
Asthma-suspected Asthma-diagnosed
Diagnosis and Evaluation: • assess symptoms, measure lung function, check inhaler technique and adherence
assessment • adjust dose • update self-management plan • move up and down as appropriate
ded
nee
l as
contro
prov
e Continuous or
to im frequent use of
o ve up erapy
M g th oral steroids
ollin
tc ontr
wes High-dose
tain lo therapies
main
d and
to fin
wn Additional
e do
Mov add-on
therapies
Consider trials of: Use daily steroid tablet
Initial add-on in the lowest dose
No response to LABA
therapy -stop LABA and consider Increasing ICS up to providing adequate
increased dose of ICS high dose control
Regular Addition of a fourth Maintain high-dose ICS

preventer If benefit from LABA but
drug, e.g., LTRA,
control still inadequate
SR theophylline, beta Consider other
-continue LABA and
agonist tablet, LAMA treatments to minimize
increase ICS to medium
dose use of steroid tablets
Consider monitored Add inhaled LABA to

low-dose ICS If benefit from LABA but
initiation of treatment
Low-dose ICS (normally as a control still inadequate
with low-dose ICS -continue LABA and
combination inhaler)
ICS and consider trial of
other therapy-LTRA,
SR theophylline, LAMA
Infrequent, Refer patient for Refer patient for

short-lived specialist care specialist care
wheeze
Short-acting 2 agonists as required-consider moving up if using three doses a week or more
487
Appendix 11B
Summary of Management of Asthma
in Children
Asthma-suspected Asthma-diagnosed
Diagnosis and Evaluation: • assess symptoms, measure lung function, check inhaler technique and adherence
assessment • adjust dose • update self-management plan • move up and down as appropriate
d
ede
s ne
ntrol a
e co Continuous or
prov
to im
eu p py frequent use of
Mov g thera
llin oral steroids
o ntro
st c High-dose
lowe
ain therapies
aint
and m
find
own to Additional
Mo ve d
add-on
therapies
Consider trials of: Use daily steroid tablet
Initial add-on in the lowest dose
No response to LABA
preventer -stop LABA and increase Increasing ICS up to providing adequate
dose of ICS to low dose medium dose control
Regular Addition of a fourth Maintain medium-dose

preventer If benefit from LABA but
drug–SR theophylline ICS
control still inadequate
-continue LABA and
increase ICS to Consider other
Very low (paediatric) low dose treatments to minimize
dose ICS use of steroid tablets
Consider monitored Very low (paediatric) If benefit from LABA but
initiation of treatment dose ICS
Plus control still inadequate
with very low-to -continue LABA and
low-dose ICS (or LTRA <5 years) ICS and consider trial of
other therapy—LTRA
Children ≥5 years
-add inhaled LABA
Infrequent, Refer patient for Refer patient for
short-lived Children <5 years specialist care specialist care
wheeze -add LTRA
Short-acting 2 agonists as required-consider moving up if using three doses a week or more
488
Appendix 11C
Management of Acute Severe Asthma
in Adults in General Practice
489
490 APPENDIX 11C Management of Acute Severe Asthma in Adults in General Practice
Management of acute severe asthma in adults in general practice
Many deaths from asthma are preventable. Delay can be Assess and record:
fatal. Factors leading to poor outcome include:
• Peak expiratory flow (PEF)
• Clinical staff failing to assess severity by objective • Symptoms and response to self treatment
measurement
• Heart and respiratory rates
• Patients or relatives failing to appreciate severity
• Oxygen saturation (by pulse oximetry)
• Underuse of corticosteroids
Caution: Patients with severe or life-threatening attacks may
Regard each emergency asthma consultation as for acute not be distressed and may not have all the abnormalities listed
severe asthma until shown otherwise. below. The presence of any should alert the doctor.
Moderate asthma Acute severe asthma Life-threatening asthma
INITIAL ASSESSMENT
PEF >50%–75% best or predicted PEF 33%–50% best or predicted PEF <33% best or predicted
FURTHER ASSESSMENT
• SpO2 ≥92% • SpO2 ≥92% • SpO2 <92%

• Speech normal • Can’t complete sentences • Silent chest, cyanosis or poor
• Respiration <25 breaths/min • Respiration ≥25 breaths/min respiratory effort
• Pulse <110 beats/min • Pulse ≥110 beats/min • Arrhythmia or hypotension

• Exhaustion, altered consciousness
MANAGEMENT
Treat at home or in surgery and
Consider admission Arrange immediate ADMISSION
ASSESS RESPONSE TO TREATMENT
TREATMENT
• 2 Bronchodilator: • Oxygen to maintain SpO2 94%–98% • Oxygen to maintain SpO2 94%–98%

– via spacer (give 4 puffs initially if available • 2 Bronchodilator and ipratropium:
and give a further 2 puffs every • 2 Bronchodilator: – nebulizer (preferably oxygen
2 minutes according to response – nebulizer (preferably oxygen driven) (salbutamol 5 mg and
up to maximum of 10 puffs) driven) (salbutamol 5 mg) ipratropium 0.5 mg)
If PEF >50%–75% predicted/best: – or via spacer (give 4 puffs initially – or via spacer (give 4 puffs initially
• Nebulizer (preferably oxygen driven) and give a further 2 puffs every and give a further 2 puffs every
(salbutamol 5 mg) 2 minutes according to response 2 minutes according to response
• Give prednisolone 40–50 mg up to maximum of 10 puffs) up to maximum of 10 puffs)
• Continue or increase usual treatment • Prednisolone 40–50 mg or IV • Prednisolone 40–50 mg or IV

hydrocortisone 100 mg hydrocortisone 100 mg immediately
If good response to first treatment
(symptoms improved, respiration and • If no response in acute severe
pulse settling and PEF >50%) continue asthma: ADMIT
or increase usual treatment and
continue prednisolone
Admit to hospital if any: If admitting the patient to hospital: Follow up after treatment or
discharge from hospital:
• Life-threatening features • Stay with patient until ambulance
• Features of acute severe asthma arrives • GP review within 2 working days
present after initial treatment • Send written assessment and • Monitor symptoms and PEF
• Previous near-fatal asthma referral details to hospital • Check inhaler technique
Lower threshold for admission if • 2 bronchodilator via oxygen-driven • Written asthma action plan
nebulizer in ambulance
afternoon or evening attack, recent • Modify treatment according to
nocturnal symptoms or hospital guidelines for chronic persistent
admission, previous severe attacks, asthma
patient unable to assess own condition,
or concern over social circumstances • Address potentially preventable
contributors to admission
Appendix 11D
Management of Acute Severe Asthma
in Children in General Practice
491
492
Management of acute asthma in children in general practice

Age 2–5 years Age >5 years
ASSESS AND RECORD ASTHMA SEVERITY ASSESS AND RECORD ASTHMA SEVERITY
Moderate asthma Acute severe asthma Life-threatening asthma Moderate asthma Acute severe asthma Life-threatening asthma
• SpO2 ≥92% • SpO2 <92% SpO2 <92% plus any of: • SpO2 ≥92% • SpO2 <92% SpO2 <92% plus any of:
• Able to talk • Too breathless to talk • Silent chest • Able to talk • Too breathless to talk • Silent chest
• Heart rate ≤140/min • Heart rate >140/min • Poor respiratory effort • Heart rate ≤125/min • Heart rate >125/min • Poor respiratory effort
• Respiratory rate ≤40/min • Respiratory rate >40/min • Agitation • Respiratory rate ≤30/min • Respiratory rate >30/min • Agitation
• Use of accessory neck • Confusion • PEF ≥50% best or predicted • Use of accessory neck • Confusion
muscles • Cyanosis muscles • Cyanosis
• PEF 33%–50% best or
predicted • PEF <33% best or predicted
• 2 agonist 2–10 puffs via • Oxygen via face mask • Oxygen via face mask • 2 agonist 2–10 puffs via • Oxygen via face mask • Oxygen via face mask
spacer and facemask (given • 10 puffs of 2 agonist • Nebulize every 20 minutes spacer and mouthpiece • 10 puffs of 2 agonist or • Nebulize every 20 minutes
one puff at a time inhaled or nebulized salbutamol with: (given one puff at a time nebulized salbutamol 5 mg with:
separately using tidal 2.5 mg - salbutamol 2.5 mg inhaled separately using tidal • Oral prednisolone - salbutamol 5 mg
breathing) • Oral prednisolone 20 mg + breathing) 30–40 mg +
• Give one puff of 2 agonist - ipratropium 0.25 mg • Give one puff of 2 agonist - ipratropium 0.25 mg
every 30–60 seconds up • Oral prednisolone 20 mg every 30–60 seconds up to 10 • Oral prednisolone
to 10 puffs according to or puffs according to response 30–40 mg
response IV hydrocortisone 50 mg if • Consider oral or
• Consider oral vomiting prednisolone 30–40 mg IV hydrocortisone 100 mg if
prednisolone 20 mg Assess response to treatment vomiting
Assess response to treatment
15 mins after 2 agonist 15 mins after 2 agonist
REPEAT 2 AGONIST VIA REPEAT 2 AGONIST VIA

IF POOR RESPONSE REPEAT OXYGEN-DRIVEN NEBULIZER IF POOR RESPONSE REPEAT OXYGEN-DRIVEN NEBULIZER
IF POOR RESPONSE IF POOR RESPONSE
ARRANGE ADMISSION 2 AGONIST AND ARRANGE WHILST ARRANGING 2 AGONIST AND ARRANGE WHILST ARRANGING
ARRANGE ADMISSION
ADMISSION IMMEDIATE HOSPITAL ADMISSION IMMEDIATE HOSPITAL
APPENDIX 11D Management of Acute Severe Asthma in Children in General Practice
ADMISSION ADMISSION
GOOD RESPONSE POOR RESPONSE GOOD RESPONSE POOR RESPONSE

• Continue 2 agonist via spacer or nebulizer, • Stay with patient until ambulance arrives • Continue 2 agonist via spacer or nebulizer, • Stay with patient until ambulance arrives
as needed but not exceeding 4 hourly • Send written assessment and referral details as needed but not exceeding 4 hourly • Send written assessment and referral details
• If symptoms are not controlled • Repeat 2 agonist via oxygen-driven nebulizer • If symptoms are not controlled • Repeat 2 agonist via oxygen-driven nebulizer
repeat 2 agonist and refer to hospital in ambulance repeat 2 agonist and refer to hospital in ambulance
• Continue prednisolone for up to 3 days • Continue prednisolone for up to 3 days
• Arrange follow-up clinic visit within 48 hours • Arrange follow-up clinic visit within 48 hours
• Consider referral to secondary care asthma • Consider referral to secondary care asthma
clinic if 2nd attack within 12 months clinic if 2nd attack within 12 months
LOWER THRESHOLD FOR ADMISSION IF: LOWER THRESHOLD FOR ADMISSION IF:
NB: If a patient has signs and NB: If a patient has signs and
• Attack in late afternoon or at night symptoms across categories, • Attack in late afternoon or at night symptoms across categories,
• Recent hospital admission or previous severe attack always treat according to • Recent hospital admission or previous severe attack always treat according to
their most severe features their most severe features
• Concern over social circumstances or ability to cope at home • Concern over social circumstances or ability to cope at home
Appendix 12
Care Pathway for Respiratory
Tract Infections
493
494 APPENDIX 12 Care Pathway for Respiratory Tract Infections
Care pathway for

At the first face-to-face contact in primary care, including walk-in centres and emergency
respiratory tract
departments, offer a clinical assessment, including:
infections (RTIs)
• history (presenting symptoms, use of over-the-counter or self-medication, previous
medical history, relevant risk factors, relevant comorbidities;
• examination as needed to establish diagnosis.
Address patients’ or parents’/carers’ concerns and expectations when agreeing to the

use of the three antibiotic strategies (no prescribing, delayed prescribing, and
immediate prescribing).
Agree to a no antibiotic or delayed antibiotic prescribing However, also consider an immediate The patient is at risk of
strategy for patients with acute otitis media, acute sore prescribing strategy for the following developing complications
throat/acute pharyngitis/acute tonsillitis, common cold, subgroups, depending on the severity
acute rhinosinusitis, or acute cough/acute bronchitis of the RTI
No antibiotic Delayed antibiotic No antibiotic, delayed Immediate antibiotic prescribing or

prescribing prescribing antibiotic, or immediate further investigation and/or
antibiotic prescribing management
Offer patients: Offer patients:
• reassurance that • reassurance that antibiotics Depending on clinical Offer immediate antibiotics or further
antibiotics are not are not needed immediately assessment of severity, investigation/management for patients
needed immediately because they will make little also consider an who:
because they will difference to symptoms and immediate prescribing • are systemically very unwell;
make little difference may have side effects (eg, strategy for: • have symptoms and signs suggestive
to symptoms and may diarrhoea, vomiting, rash); • children younger than 2 of serious illness and/or complications
have side effects (eg, • advice about using the years with bilateral particularly pneumonia, mastoiditis,
diarrhoea, vomiting, delayed prescription if acute otitis media; peritonsillar abscess, peritonsillar
rash); symptoms do not settle or • children with otorrhoea cellulitis, intraorbital or intracranial
• a clinical review if the get significantly worse; who have acute otitis complications;
RTI worsens or • advice about reconsulting if media; • are at high risk of serious
becomes prolonged. symptoms get significantly • patients with acute sore complications because of preexisting
worse despite using the throat/acute pharyngitis/ comorbidity. This includes patients
delayed prescription. acute tonsillitis when with significant heart, lung, renal, liver,
• The delayed prescription with three or more Centor or neuromuscular disease;
instructions can either be criteria* are present. immunosuppression; cystic fibrosis;
given to the patient or and young children who were born
collected at a later date. prematurely;
• are older than 65 years with acute
cough and two or more of the
following, or older than 80 years with
acute cough and one or more of the
following:
• Hospitalization in previous year
• Type 1 or type 2 diabetes
• History of congestive heart failure
• Current use of oral glucocorticoids
Offer all patients: • advice about the usual natural history of the illness and • advice about managing symptoms
average total illness length: including fever (particularly analgesics
• Acute otitis media: 4 days and antipyretics).
• Acute sore throat/acute pharyngitis/acute tonsilitis: 1 week For information about fever in children
• Common cold: 1½ weeks younger than 5 years, refer to “Feverish
• Acute rhinosinusitis: 2½ weeks Illness in Children” (NICE clinical
• Acute cough/acute bronchitis: 3 weeks guideline 47).
*Centor criteria are presence of tonsillar exudate, tender anterior lymphadenopathy or lymphadenitis, history of fever, and an absence of
cough.
Reproduced with permission from National Insititute for Health and Care Excellence. (2008). Respiratory tract infections—antibiotic
prescribing. NICE clinical guideline 69. www.nice.org.uk.
Appendix 13
Guidance for DMARD Prescribing
GUIDELINE Prescribing DMARDS in Patients

British Society of Rheumatology. (2017). BSR and BHPR With Comorbidities
guideline for the prescription and monitoring of non-biologic
disease-modifying anti-rheumatic drugs. Rheumatology, • Pre-existing lung disease is not a specific
56(6), 865–868.
contraindication to DMARD therapy; however,
caution is advised when using drugs associated
with pneumonitis in patients with poor respiratory
reserve.
Generic Recommendations Before • In patients with deranged liver biochemistry,
Commencing Any DMARD hepatotoxic DMARDs should be used with caution,
with careful attention to trends in test results.
a. The decision to initiate DMARDs should be made in • In patients with impaired liver synthetic function (e.g.,
conjunction with the patient/carer and be supervised cirrhosis), DMARD therapy should be used with
by an expert in the management of rheumatic diseases. extreme caution.
b. Patients should be provided with education about their • Patients with chronic viral hepatitis infection should be
treatment to promote self-management. considered for antiviral treatment prior to
c. When appropriate, patients should be advised about immunosuppressive DMARD initiation.
the impact of DMARD therapy upon fertility, • DMARDs must be used with caution in chronic
pregnancy, and breastfeeding. kidney disease, with appropriate dose reduction and
d. Baseline assessment should include height, weight, increased frequency of monitoring.
blood pressure, and laboratory evaluation: FBC, eGFR, • Cardiovascular disease and prior malignancy are not
ALT, and/or AST, albumin. considered contraindications to DMARD therapy.
e. Patients should be assessed for comorbidities because
these may influence DMARD choice, including
evaluation for respiratory disease and screening for Drug Monitoring
occult viral infection.
f. Vaccinations against pneumococcus and influenza are Recommended DMARD Blood Monitoring
recommended. Schedule When Starting or Adding
a New DMARD
Drug-Specific Recommendations
• Check FBC, creatinine/calculated GFR, ALT and/or
• Methotrexate: All patients should be co-prescribed folic AST, and albumin every 2 weeks until on stable dose
acid supplementation at a minimal dose of 5 mg once for 6 weeks; then once on stable dose, monthly FBC,
weekly to be taken on a different day than the creatinine/calculated GFR, ALT and/or AST, and
methotrexate. albumin for 3 months; thereafter, FBC, creatinine/
• Azathioprine: Patients should have baseline thiopurine calculated GFR, ALT and/or AST, and albumin at least
methyltransferase (TPMT) status assessed. every 12 weeks. More frequent monitoring is
• Hydroxychloroquine: Patients should have baseline appropriate in patients at higher risk of toxicity.
formal ophthalmic examination, ideally including • Dose increases should be monitored by FBC,
objective retinal assessment (e.g., using optical creatinine/calculated GFR, ALT and/or AST, and
coherence tomography) within 1 year of commencing albumin every 2 weeks until on stable dose for 6 weeks
an antimalarial drug. then revert to previous schedule.
495
496 APPENDIX 13 Guidance for DMARD Prescribing
Drug Specific Monitoring Recommendations
Summary of Monitoring Requirements
Drug Laboratory Monitoring Other Monitoring

Apremilast No routine laboratory monitoring None
a
Azathioprine Standard monitoring schedule None
Ciclosporin Extend monthly monitoring longer term BP and glucose at each monitoring visit
a
Gold Standard monitoring schedule Urinalysis for blood and protein prior to
each dose
Hydroxychloroquine No routine laboratory monitoring Annual eye assessment (ideally
including optical coherence
tomography) if continued for >5 years
Leflunomide Standard monitoring schedulea BP and weight at each monitoring visit
Mepacrine No routine laboratory monitoring None
Methotrexate Standard monitoring schedule None
Methotrexate and leflunomide combined Extend monthly monitoring longer term None
Minocycline No routine laboratory monitoring None
Mycophenolate Standard monitoring schedule None
Sulfasalazine Standard monitoring schedule for None
12 months then no routine monitoring
needed
Tacrolimus Extend monthly monitoring longer term BP and glucose at each monitoring visit
BP, Blood pressure.

a
Standard monitoring schedule as per the guidance in ‘Drug Monitoring’
Perioperative DMARD Management • When prescribing takes place in primary care, it should
be supported by local written shared care agreements,
• Steroid exposure should be minimized prior to surgical highlighting responsibilities of each party (patient,
procedures; and increases in steroid dose to prevent secondary care, primary care).
adrenal insufficiency are not routinely required. • Contact rheumatology team urgently and consider
• DMARD therapy should not routinely be stopped in interruption in treatment if any of the following
the perioperative period, although individualized develop: white cell count <3.5 × 109/L; mean cell
decisions should be made for high-risk procedures. volume >105 fL; neutrophils <1.6 × 109/L; creatinine
increase >30% over 12 months and/or calculated GFR
Intercurrent Infections <60 mL/min; unexplained eosinophilia >0.5 × 109/L;
ALT and/or AST >100 units/L; platelet count <140 ×
• During a serious infection, methotrexate, leflunomide, 109/L; unexplained reduction in albumin <30 g/L.
sulfasalazine, azathioprine, apremilast, mycophenolate • As well as responding to absolute values in laboratory
mofetil, ciclosporin, and tacrolimus should be tests, it is also relevant to observe trends in results (e.g.,
temporarily discontinued until the patient has gradual decreases in white blood cells or albumin, or
recovered from the infection. increasing liver enzymes).
• For clinically urgent abnormalities, emergency access to
Recommendations for Shared specialist rheumatology advice, with response within
Care Agreements one working day, should be available as per National
Institute for Health and Care Excellence guidelines.
• The prescriber has responsibility for ensuring patients
are adhering to monitoring guidance.
Appendix 14
Dermatomes and Myotomes
C2
C4
C5
T4
T2
T1 T2
T7
C6
T10 T1
T12
L1 S5
C7 S4
C8 S3
L2 C7
L3 S2
L4 L2
S1
L5
Muscle group Nerve supply Reflexes

Diaphragm C(3), 4, (5)
Shoulder abductors C5
Elbow flexors C5, 6 Biceps jerk C5, 6
Supinators/pronators C6 Supinator jerk C6
Wrist extensors C6
Wrist flexors C7
Elbow extensors C7 Triceps jerk C7
Finger extensors C7
Finger flexors C8
Intrinsic hand muscles T1 Abdominal reflex T8–12
Hip flexors L1, 2

Hip adductors L2, 3
Knee extensors L3, 4 Knee jerk L3, 4
Ankle dorsiflexors L4, 5
Toe extensors L5
Knee flexors L4, 5 S1
Ankle plantar flexors S1, 2 Ankle jerk S1, 2
Toe flexors S1, 2
Anal sphincter S2, 3, 4 Bulbocavernosus reflex S3, 4
Anal reflex S5
Plantar reflex
497
Appendix 15
Testing Peripheral Nerves
Nerve Root Muscle Test—By Asking the Patient to

C3, 4 Trapezius Shrug shoulder, adduct scapula
C4, 5 Rhomboids Brace shoulder back
C5, 6, 7 Serratus anterior Push forward against resistance
C5, 6, 7, 8 Pectoralis major (clavicular head) Adduct arm from above horizontal and forward
C6, 7, 8, T1 Pectoralis major (sternocostal head) Adduct arm below horizontal
C5 Supraspinatus Abduct arm the first 15 degrees
C5, 6 Infraspinatus Externally rotate arm, elbow at side
C6, 7, 8 Latissimus dorsi Adduct horizontal and lateral arm
C5, 6 Biceps Flex supinated forearm
C5, 6 Deltoid Abduct arm between 15 and 90 degrees
Radial Nerve
C7, 8 Triceps Extend elbow against resistance
C5, 6 Brachioradialis Flex elbow with forearm halfway between pronation and supination
C6, 7 Extensor carpi radialis longus Extend wrist to radial side with fingers extended
C5, 6 Supinator Arm by side, resist hand pronation
C7, 8 Extensor digitorum Keep fingers extended at MCP joint
C7, 8 Extensor carpi ulnaris Extend wrist to ulnar side
C7, 8 Abductor pollicis longus Abduct thumb at 90 degrees to palm
C7, 8 Extensor pollicis brevis Extend thumb at MCP joint
C7, 8 Extensor pollicis longus Resist thumb flexion at IP joint
Median Nerve
C6, 7 Pronator teres Keep arm pronated against resistance
C6, 7, 8 Flexor carpi radialis Flex wrist towards radial side
C7, 8, T1 Flexor digitorum sublimis Resist extension at PIP joint (while you fix the proximal phalanx)
C8, T1 Flexor digitorum profundus I and II Resist extension at the DIP joint
C8, T1 Flexor pollicis longus Resist thumb extension at interphalangeal joint (fix proximal phalanx)
C8, T1 Abductor pollicis brevis Abduct thumb (nail at 90 degrees to palm)
C8, T1 Opponens pollicis Thumb touches fifth fingertip (nail parallel to palm)
C8, T1 First and second lumbricals Extend PIP joint against resistance with MCP joint held hyperextended
498
APPENDIX 15 Testing Peripheral Nerves 499
Nerve Root Muscle Test—By Asking the Patient to

Ulnar Nerve
C7, 8 Flexor carpi ulnaris Abducting little finger, see tendon when all fingers extended
C8, T1 Flexor digitorum profundus III and IV Fix middle phalanx of little finger, resisting extension of distal phalanx
C8, T1 Dorsal interossei Abduct fingers (use index finger)
C8, T1 Palmar interossei Adduct fingers (use index finger)
C8, T1 Adductor pollicis Adduct thumb (nail at 90 degrees to palm)
C8, T1 Abductor digiti minimi Abduct little finger
C8, T1 Opponens digiti minimi With fingers extended, carry little finger in front of other fingers
Nerve Root
L4, 5, S1 Gluteus medius and minimus (superior Internal rotation at hip, hip abduction
gluteal nerve)
L5, S1, 2 Gluteus maximus (inferior gluteal nerve) Extension at hip (lie prone)
L2, 3, 4 Adductors (obturator nerve) Adduct leg against resistance
Femoral Nerve
L1, 2, 3 Iliopsoas Flex hip with knee flexed and lower leg supported (patient lies on back)
L2, 3 Sartorius Flex knee with hip externally rotated
L2, 3, 4 Quadriceps femoris Extend knee against resistance
Sciatic Nerve
L4, 5, S1, 2 Hamstrings Flex knee against resistance
L4, 5 Tibialis posterior Invert plantarflexed foot
L4, 5 Tibialis anterior Dorsiflex ankle
L5, S1 Extensor digitorum longus Dorsiflex toes against resistance
L5, S1 Extensor hallucis longus Dorsiflex hallux against resistance
L5, S1 Peroneus longus and brevis Exert foot against resistance
S1 Extensor digitorum brevis Dorsiflex hallux (muscle of foot)
S1, 2 Gastrocnemius Plantarflex ankle joint
S1, 2 Flexor digitorum longus Flex terminal joints of toes
S1, 2 Small muscles of foot Make sole of foot into a cup
Source: Medical Research Council. (1976). Aids to the examination of the peripheral nervous system. London: HMSO. Crown copyright material is reproduced
with the permission of the Controller of HMSO and the Queen’s Printer for Scotland.
Appendix 16
Drug Levels
Drug Therapeutic Range

For the following drugs, blood should be taken predose:
Carbamazepine 4–12 mg/L
Ethosuximide 40–100 mg/L
Phenobarbital 20–40 mg/L
Phenytoin 10–20 mg/L (child: 6–14 mg/L)
Primidone As for phenobarbital
Theophylline 10–20 mg/L
For the following drugs, blood should be taken at the times indicated:
Digoxin (8–12 hours after last dose) 0.6–2.0 µg/L toxicity 1.8–3.0 µg/L
Lithium (12 hours after last dose) 0.4–1.0 mmol/L
Valproate (2 hours after last dose) 50–100 mg/L
500
Appendix 17
Checklist to Guide the Review of
a Patient With Multiple Sclerosis
This is not a list of questions to be asked of every person with MS on every occasion. It is a list to remind clinicians of the
wide range of potential problems that people with MS may face, and which should be actively considered as appropriate. A
positive answer should lead to more detailed assessment and management.
INITIAL QUESTION
It is best to start by asking an open-ended question such as: • Balance and falling
“Since you were last seen or assessed has any activity you • Chewing and swallowing food and drink
used to undertake been limited, stopped, or affected?” • Unintended change in weight
• Pain or painful abnormal sensations
Activity Domains • Control over your bladder or bowels
Then, especially if nothing has been identified, it is worth • Control over your movement
asking questions directly, choosing from the list below those • Vision and your eyes
appropriate to the situation based on your knowledge of the • Thinking, remembering
person with MS: • Your mood
“Are you still able to undertake, as far as you wish, the • Your sexual function or partnership relations
following?” • How you get on in social situations
• Vocational activities (work, education, other occupation)
• Leisure activities Final Question
• Family roles Finally, it is always worth finishing with a further open-ended
• Shopping and other community activities question:
• Household and domestic activities “Are there any other new problems that you think might be
• Washing, dressing, using toilet due to MS that concern you?”
• Getting about (either by walking or in other ways) and
getting in and out of your house Reference
• Controlling your environment (opening doors, switching National Institute of Health and Care Excellence. (2014).
things on and off, using the phone) Multiple sclerosis in adults: Management. NICE clinical
If restrictions are identified, then the reasons for these guideline 186. www.nice.org.uk.
should be identified as far as possible considering impairments
(see below), and social and physical factors (contexts).
Common Impairments
It is worth asking about specific impairments from the list
below, again adapting to the situation and what you already
know:
“Since you were last seen have you developed any new
problems with the following?”
• Fatigue, endurance, being overtired
• Speech and communication
501
Appendix 18
Medical Management of Obesity
Pharmacological Management
Consider pharmacological management with orlistat if:
1. adequate weight loss has not been achieved with appropriate lifestyle measures; and
2. the patient has been appropriately counselled about adverse effects; and
3. the patient has a body mass index (BMI) >28 with associated risk factors or >30.
Continue pharmacological management:
1. beyond 3 months only if 5% of their initial body weight has been lost (although allow for less ambitious targets in people with
type 2 diabetes in whom rate of weight loss might be slower);
2. beyond 12 months after discussing the benefits and limitations of treatment with the patient.
Surgical Management
Consider referral for bariatric surgery if:
1. BMI ≥40 or BMI 35–40 with a comorbidity that would be improved with weight loss (e.g., type 2 diabetes, hypertension); and
2. all nonsurgical options have been tried and clinically beneficial weight loss has not been achieved/maintained; and
3. the patient is receiving or will receive intensive treatment in a specialist service; and
4. the patient is fit for anaesthesia; and
5. the patient commits to long-term follow-up.
National Institute of Health and Care Excellence. (2014). Obesity: Identification, assessment and management. NICE clinical guideline 189. www.nice.org.uk.
502
Appendix 19
Immunizations in Pregnancy
Immunizations Contraindicated in Pregnancy Vaccine Comments

Vaccine Comments Diphtheria/tetanus
BCG Live mycobacterium Hepatitis A The patient may choose
immunoglobulin as a safer
Cholera (oral) No evidence of safety; benefit unlikely to alternative.
outweigh theoretical risk
Hepatitis B
Measles Live virus. Avoid pregnancy for
3 months after vaccination Immunoglobulin
Mumps Live virus. Avoid pregnancy for Influenza The vaccine is positively indicated
3 months after vaccination in pregnant women. They are
more prone to pulmonary
Rubella Live virus. Avoid pregnancy for complications than
3 months after vaccination nonpregnant women.
Typhoid Live bacterium Japanese encephalitis Inactivated virus but there is no
Ty21a consensus on its safety.
Varicella Live virus. Consider giving VZIG if Meningococcus

exposed to chickenpox in pregnancy Pneumococcus
Yellow fever Live virus. Give patient a written waiver if Pertussis The vaccine is positively indicated
travelling to a country requiring a in pregnancy (ideally between
certificate. If risk of contracting the 16 and 32 weeks) to protect
disease is high the patient may newborn babies via passive
choose to have the immunization. immunity.
Immunizations which may be given in pregnancy if Polio (oral) Paralysis seems more likely in
patient and physician judge that the potential pregnancy than in the
benefit outweighs the risk non-pregnant woman.
That risk is theoretical. In almost all of these Neonatal infection carries a
immunizations there is no evidence either way. If high mortality rate.
deciding to give the immunization, it may be thought Rabies
prudent to wait until the second or third trimester in
case a reaction to the vaccine were to trigger a Typhoid (Vi capsular
first-trimester miscarriage. polysaccharide)
Adapted from Centers for Disease Control and Prevention. (2008). CDC
Health Information for International Travel. Atlanta, GA: Mosby; National
Institute for Health and Care Excellence. (2008). Antenatal care: Routine
care for the healthy pregnant woman. National Collaborating Centre for
Women’s and Children’s Health. Commissioned by the National Institute
for Health and Clinical Excellence. NICE clinical guideline 62. www.nice.
org.uk; Martinez, L. (Ed.). (2002). International travel and health. Geneva:
World Health Organization.
503
Appendix 20
Edinburgh Postnatal Depression Scale
Instructions for Users

1. The mother is asked to underline the response which comes closest to how she has been feeling in the previous 7 days.
2. All 10 items must be completed.
3. Care should be taken to avoid the possibility of the mother discussing her answers with others.
4. The mother should complete the scale herself, unless she has limited English or has difficulty with reading.
5. The EPDS may be used at 6–8 weeks to screen postnatal women. The child health clinic, postnatal checkup, or a home visit
may provide suitable opportunities for its completion.
Scoring the EPDS
• Response categories are scored 0, 1, 2, and 3 according to increased severity of the symptom.
• Items marked with an asterisk are reverse scored (i.e., 3, 2, 1, and 0). The total score is calculated by adding together the
scores for each of the 10 items.
• Mothers who score above a threshold 12/13 are likely to be suffering from a depressive illness of varying severity.
Nevertheless, the EPDS score should not override clinical judgment. A careful clinical assessment should be carried out to
confirm the diagnosis. The scale indicates how the mother has felt during the previous week, and in doubtful cases it may be
usefully repeated after 2 weeks. The scale will not detect mothers with anxiety neuroses, phobias, or personality disorders.
As you have recently had a baby, we would like to know how you are feeling. Please UNDERLINE the answer which comes
closest to how you have felt IN THE PAST 7 DAYS, not just how you feel today. Here is an example, already completed.
1. I have felt happy:
Yes, all the time
Yes, most of the time
No, not very often
No, not at all
This would mean: “I have felt happy most of the time” during the past week. Please complete the other questions in the same
way.
In the Past 7 Days:
1. I have been able to laugh and see the funny side of things:
As much as I always could
Not quite so much now
Definitely not so much now
Not at all
2. I have looked forward with enjoyment to things:
As much as I ever did
Rather less than I used to
Definitely less than I used to
Hardly at all
3. *I have blamed myself unnecessarily when things went wrong:
Yes, some of the time
Not very often
No, never
4. *I have been anxious or worried for no good reason:
No, not at all
Hardly ever
Yes, sometimes
Yes, very often
504
APPENDIX 20 Edinburgh Postnatal Depression Scale 505
5. *I have felt scared or panicky for no very good reason:

Yes, quite a lot
Yes, sometimes
No, not much
No, not at all
6. *Things have been getting on top of me:
Yes, most of the time I haven’t been able to cope at all
Yes, sometimes I haven’t been coping as well as usual
No, most of the time I have coped quite well
No, I have been coping as well as ever
7. *I have been so unhappy that I have had difficulty sleeping:
Yes, sometimes
Not very often
No, not at all
8. *I have felt sad or miserable:
Yes, quite often
Not very often
No, not at all
9. *I have been so unhappy that I have been crying:
Yes, quite often
Only occasionally
No, never
10. *The thought of harming myself has occurred to me:
Yes, quite often
Sometimes
Hardly ever
Never
© The Royal College of Psychiatrists. (1987). The Edinburgh Postnatal Depression Scale may be photocopied by individual researchers or clinicians for their
own use without seeking permission from the publishers. The scale must be copied in full and all copies must acknowledge the following source: Cox, J. L.,
Holden, J. M., & Sagorsky, R. (1987). Detection of postnatal depression: Development of the 10-item Edinburgh Postnatal Depression Scale. British Journal of
Psychiatry, 150, 782–786. Written permission must be obtained from the Royal College of Psychiatrists for copying and distributing to others or for reproduction
(in print, online, or by any other medium). Translations of the scale, and guidance as to its use, may be found in Cox, J. L., & Holden, J. (2003). Perinatal mental
health: A guide to the Edinburgh Postnatal Depression Scale. London: Gaskell.
Appendix 21
Admission Procedures for Patients
With Mental Health Problems
Compulsory Admission
The team needed to complete a Section 2 or Section 3 consists of:
a) the general practitioner (GP) (or an independent section 12–approved doctor if the GP is not available);
b) an approved mental health professional (AMHP);
c) an approved psychiatrist (duty consultant or specialist registrar).
The procedure to follow between 9 am and 5 pm for assessment of a patient who may need to be sectioned from home might
be as follows:
• Obtain relevant information from a partner who may know the patient better.
• Review the records to assess:
• risk of violence;
• past history of outcomes of previous sections;
• previous responses to treatment.
• Phone the family or carer to obtain their assessment of the:
• current situation and urgency;
• need for police support;
• risk of violence, access to weapons.
• Ensure the patient is at home and someone is in to allow access.
• Explain the procedure and arrange a time to visit.
• Contact the duty AMHP and duty psychiatrist:
• Provide basic information: name, date of birth, address, past history, current problem, reason for assessment, phone
number of patient, name of carer or relative at home, how to contact you in the next few hours, name of key worker, any
known risk of violence or self-harm.
• Decide on the need for police support.
• Arrange a time to meet together at the home.
• If a joint visit is not possible decide:
• when each will visit;
• how to discuss assessments and decide on need for sectioning;
• where to leave the section form for GP to sign (e.g., will it be left with relative or brought to surgery?).
• If the GP visits first, take the section forms and complete, if appropriate.
• Avoid sedating the patient as this makes subsequent assessment difficult.
• Avoid asking the relative to sign Section 2 or 4, as this increases guilt and may disrupt future relationships.
• If the two doctors cannot do a joint assessment, they must examine the patient within 5 days of each other.
• Organize a hospital bed (the AMHP is responsible for arranging transport).
• If the GP and AMHP disagree on the need for sectioning:
• record the basis of each decision in writing;
• identify a home care plan;
• decide who will reassess and when;
• instruct the carer in what to do if the situation changes.
• Ask family or carer to make a GP follow-up appointment to find out how they are coping and what has happened to the
patient, and future plans.
• Complete item of service claim form.
506
APPENDIX 21 Admission Procedures for Patients With Mental Health Problems 507
Voluntary Admission
• If patient is known to hospital staff and a bed is available and the patient accepts admission: arrange admission, write a letter,
organize transport.
• If patient is known to hospital staff but there are no beds available: contact the bed manager or psychiatric nurse manager.
1. If a bed is found, arrange admission as above.
2. If a bed is not immediately available and a 2-hour delay is acceptable, if supported by CPN or crisis team:
a) arrange for the hospital to phone you and family when bed available;
b) write letter and give to patient or relative;
c) ask the family to phone the GP if they have not heard from hospital in 2 hours.
3. If a bed is not immediately available and a delay is not acceptable:
a) phone the A&E psychiatric registrar or A&E mental health nurse;
b) consider referral to A&E;
c) other options according to local guidance.
• If the patient is not known to hospital staff: if Monday to Friday, 9 am to 5 pm then:
a) check catchment area and relevant consultant;
b) phone the consultant on pager or mobile or contact his or her secretary to ask the psychiatrist to phone you ASAP:
• if the consultant is available arrange for domiciliary visit or admission;
• if consultant is not available, phone the SHO or registrar and proceed as above;
• if out of hours, follow the local protocol for voluntary admission if available.
Note: The out of hours service should ensure that the protocol is in the doctor’s bag.
Appendix 22
The Early Warning Form for Use in
Psychotic Illness
EARLY WARNING SIGNS

Name: ………………………………………………………………………………..
I am at risk of developing episodes of: …………………………………………………………………………….……………………
………………………………................................................................................................................................................….
My early warning signs are (e.g., changes in sleep, eating/drinking or mood, becoming quiet or loud or more withdrawn):
1. ………………………………………………………......………………………………………….....................….……………………
2. ………………………………………………………………………………………………………......................………..……………
3. …………………………………………………………………………………………………………....................….…………………
Whenever I have any of these signs I will respond by: ……………………………….......................................................………….
……………………………………………………………………………………………….................……….…………………………..
……………………………………………………………………………………………….................…………………………………...
My health worker is: ………………………….... Phone ………………………………………………..
My home contact is: …………………………… Phone ………………………………………………..
My advocacy contact is: ……………………..... Phone ………………………………………………..
If I have any concerns about my illness I will contact: ................................
..................................…………………………………………. immediately.
Reproduced with permission from Falloon, I.R.H., et al. (1993). Managing stress in families: Cognitive and behavioural strategies for enhancing coping skills.
London: Routledge.
508
Appendix 23
AUDIT
The Alcohol Use Disorders Identification Test: Interview Version

Initially give an explanation of the content and purpose of the questions and the need for accurate answers. Read questions
as written. Record answers carefully. Begin the AUDIT by saying, “Now I am going to ask you some questions about your
use of alcoholic beverages during this past year.” Explain what is meant by “alcoholic beverages” by using local examples of
beer, wine, vodka, etc. Code answers in terms of “standard drinks.” Place the correct answer number in the box at the right.
1. How often do you have a drink containing alcohol? □

(0) Never [Skip to Questions 9–10]
(1) Monthly or less
(2) 2 to 4 times a month
(3) 2 to 3 times a week
(4) 4 or more times a week
2. How many drinks containing alcohol do you have on a typical day when you are drinking? □
(0) 1 or 2
(1) 3 or 4
(2) 5 or 6
(3) 7, 8, or 9
(4) 10 or more
3. How often do you have six or more drinks on one occasion? □

(0) Never
(1) Less than monthly
(2) Monthly
(3) Weekly
(4) Daily or almost daily
Skip to Questions 9 and 10 if Total Score for Questions 2 and 3 = 0
4. How often during the last year have you found that you were not able to stop drinking once you had started? □
(0) Never
(2) Monthly
(3) Weekly
Continued
509
510 APPENDIX 23 Audit
5. How often during the last year have you failed to do what was normally expected from you because of drinking? □
(0) Never
(2) Monthly
(3) Weekly
6. How often during the last year have you needed a first drink in the morning to get yourself going after a heavy □
drinking session?
(0) Never
(2) Monthly
(3) Weekly
7. How often during the last year have you had a feeling of guilt or remorse after drinking? □
(0) Never
(2) Monthly
(3) Weekly
8. How often during the last year have you been unable to remember what happened the night before because you □
had been drinking?
(0) Never
(2) Monthly
(3) Weekly
9. Have you or someone else been injured as a result of your drinking? □

(0) No
(2) Yes, but not in the last year
(4) Yes, during the last year
10. Has a relative or friend or a doctor or another health worker been concerned about your drinking or suggested you □
cut down?
(0) No
(2) Yes, but not in the last year
(4) Yes, during the last year
Record total of specific items here: □

An AUDIT score in the range of 8–15 represents a medium level of alcohol problems where brief interventions would be
appropriate. Scores of ≥16 represent a high level of alcohol problems with higher levels of intervention and monitoring
recommended. Scores of ≥20 suggest dependent drinking and merits further assessment.1
AUDIT-C uses the first three questions only. If the score is ≥3 then complete the full questionnaire.
1
Babor, T. F., Higgins-Biddle, J. C., Saunders, J. B., et al. (2001). Audit, the alcohol use disorders identification test: World Health Organization (WHO).
www.who.int/Alcohol_AUDIT.
Appendix 24
International Prostate Symptom
Score (IPSS)
Less Than Less About More Than
Not at One Time Than Half Half the Half the Almost
All in Five the Time Time Time Always
1. Incomplete emptying 0 1 2 3 4 5
Over the past month, how often have
you had a sensation of not
emptying your bladder completely
after you have finished urinating?
2. Frequency 0 1 2 3 4 5
you had to urinate again less than
2 hours after you finished urinating?
3. Intermittency 0 1 2 3 4 5
you found you stopped and started
again several times when you
urinated?
4. Urgency 0 1 2 3 4 5
you found it difficult to postpone
urination or felt sudden urges to
urinate?
5. Weak stream 0 1 2 3 4 5
you had a weak urinary stream?
6. Straining 0 1 2 3 4 5
you had to push or strain to begin
urination?
Five
Three Times
None Once Twice Times Four Times or More
7. Nocturia 0 1 2 3 4 5
Over the past month, how many
times did you typically get up to
urinate from the time you went to
bed at night to the time you got up
in the morning?
(Scoring items 1–7: 0–7 = mild; 8–19 = moderate; 20–35 = severe)
Continued
511
512 APPENDIX 24 International Prostate Symptom Score (IPSS)
Less Than Less About More Than
Not at One Time Than Half Half the Half the Almost
All in Five the Time Time Time Always
No
Strong
Feelings
Mostly Either Mostly
Quality of Life Delighted Pleased Satisfied Way Dissatisfied Unhappy Terrible
If you were to spend the rest of your 0 1 2 3 4 5 6
life with urinary conditions just the
way they are now, how would you
feel about it?
Appendix 25
Body Mass Index
2
Body mass index = W/H
Women
Height (ft)
4'9" 5'0" 5'3" 5'6" 5'9"
100
15 Obese
90 14
13 Marginal
80
12
W
Weight (stones)
= 28.6
Weight (kg)
2 11 Desirable
70 H
weight range
10
W
60 = 23.8
2 9
H
50 W 8 Underweight
2
= 18.7
H 7
W
40 2
H
Obese
28.6
30
Marginal/overweight
150 160 170 180 23.8
Height (cm)
Desirable weight
18.7
Underweight
Men Height (ft)

5'3" 5'6" 5'9" 6'0" 6'3"
120
18 Obese
110 17 Marginal
16
100
15
90 14 Desirable
Weight (stones)
W
Weight (kg)
= 30 weight
2 13 range
H
80
12
W
= 25
70 H
2
11
10 Underweight
60 W
= 20
H
2 9
W
50 2
H
Obese
30
40 Marginal/overweight
25
160 170 180 190
Height (cm) Desirable weight
20
Underweight
513
Appendix 26
Reference Ranges for Young Adults
Reference ranges vary according to laboratory and test method. The following are given as typical ranges but if the laboratory
performing the test gives a range that differs from these it should be used instead. The range will also vary according to age
and gender.
Blood
Biochemistry and Immunology
Serum or plasma Immunoglobulins:
Acid phosphatase: IgG 6–13 g/L
total 1–5 IU/L IgM 0.5–2.0 g/L
prostatic 0–1 IU/L IgA 1.0–4.0 g/L
ACTH 10–80 ng/L Lactate dehydrogenase 70–250 IU/L
Alkaline phosphatase 30–300 IU/L Magnesium 0.7–1.0 mmol/L
Alanine aminotransferase 5–35 IU/L Osmolality 280–295 mosmol/kg
Amylase <120 IU/L Phosphate (inorganic) 0.8–1.4 mmol/L
Asparate aminotransferase 5–35 IU/L Potassium 3.4–5.2 mmol/L
Bicarbonate 21–26 mmol/L Prolactin Male: 80–400 mu/L
Bilirubin <17 mmol/L Female: 90–520 mu/L
Calcium 2.26–2.60 mmol/L Postmenopausal female:
80–280 mu/L
Chloride 95–105 mmol/L Protein:
Cholesterol <5.5 mmol/L total 60–80 g/L
Complement: albumin 35–50 g/L
C3 0.69–1.5 mg/L PSA 0–4 ng/mL
C4 0.12–0.27 mg/L Sodium 133–145 mmol/L
Total thyroxine 70–140 nmol/L
Cortisol: Free T4 10–26 pmol/L
9:00 am 130–690 nmol/L Free T3 3–9 pmol/L
midnight Half the am value Triiodothyronine 1.2–3.0 nmol/L
Creatinine 70–130 mol/L TSH 0.3–3.8 mu/L
Creatine kinase <200 IU/L Triglycerides** <0.55–1.90 mmol/L
α-Fetoprotein <10 ku/L Urea 2.5–6.7 mmol/L
514
APPENDIX 26 Reference Ranges for Young Adults 515
γ-Glutamyl transferase: Uric acid:

men 11–51 IU/L men 0.15–0.42 mmol/L
women 7–33 IU/L women 0.10–0.36 mmol/L
Glucose (fasting) 3.4–5.5 mmol/L
Growth hormone <5.5 mu/L Arterial blood gases
pH 7.35–7.45
PaO2 12–14 kPa
PaCO2 4.6–6.0 kPa
*To convert cholesterol from mmol/L into mg/dL multiply by 39.

**To convert triglycerides from mmol/L into mg/dL multiply by 89.
Haematology Urine
Haemoglobin 13.5–18.0 g/dL (men) Sodium 100–250 mmol/24 h
11.5–16.0 g/dL (women)
Potassium 14–120 mmol/24 h
MCV 82–98 fL
Albumin:
MCH 26.7–33.0 pg – microalbuminuria 20–200 mg/L
MCHC 31.4–35.0 g/dL – proteinuria >200 mg/L
WBC 3.2–11.0×109/L Albumin/creatinine

9
ratio:
Neutrophils 1.9–7.7×10 /L – microalbuminuria 2.5 mg/mmol (men) or 3.5 mg/
Monocytes 0.1–0.9×109/L mmol (women) to 30 mg/mmol
– proteinuria >30 mg/mmol
Eosinophils 0.0–0.4×109/L
Creatinine clearance 85–125 mL/min (men);
Basophils 0.2–0.8×109/L 75–115 mL/min (women)
Platelets 120–400×109/L Osmolality 350–1000 mosmol/kg
Reticulocytes 25–100×109/L (or <2%)
Ferritin 30–230 µg/L (male)
6–80 µg/L (female)
14–180 µg/L
(postmenopausal female)
Appendix 27
Anaphylaxis Algorithm
Reproduced from Working Group of the Resuscitation Anaphylactic reaction?
Council (UK). (2008). Emergency treatment of anaphylactic
reactions. Guidelines for healthcare providers. London: Resus-
Airway, Breathing, Circulation, Disability, Exposure
citation Council (UK), by permission of the Resuscitation
Council.
Diagnosis—look for:
• Acute onset of illness
• Life-threatening airway and/or breathing and/or
circulation problems1
• And usually skin changes
• Call for help

• Lie patient flat
• Raise patient’s legs
Adrenaline2
When skills and equipment available:

• Establish airway
• High flow oxygen Monitor:
• IV fluid challenge3 • Pulse oximetry
• Chlorphenamine4 • ECG
• Hydrocortisone5 • Blood pressure
1Life-threatening problems:
Airway: swelling, hoarseness, stridor
Breathing: rapid breathing, wheeze, fatigue, cyanosis, SpO2
<92%, confusion
Circulation: pale, clammy, low blood pressure, faintness,
drowsy/coma
2Adrenaline (give IM unless experienced with IV adrenaline)
IM doses of 1:1000 adrenaline (repeat after 5 mins if no better)

• Adult 500 μg IM (0.5 mL)
• Child >12 years 500 μg IM (0.5 mL)
• Child 6–12 years 300 μg IM (0.3 mL)
• Child <6 years 150 μg IM (0.15 mL)
Adrenaline IV to be given only by experienced specialists
Titrate: Adults 50 μg; children 1 μg/kg
3IV fluid challenge:
Adult: 500–1000 mL
Child: crystalloid 20 mL/kg
Stop IV colloid if this might be the cause of anaphylaxis
4Chlorphenamine 5Hydrocortisone
(IM or slow IV) (IM or slow IV)

Adult or child >12 years 10 mg 200 mg
Child 6–12 years 5 mg 100 mg
Child 6 months–6 years 2.5 mg 50 mg
Child <6 months 250 μg/kg 25 mg
516
Appendix 28
Problems Associated With Specific
Causes of Disability
517
518
Muscular/
Psychiatric/ Skeletal and
Audiovisual Endocrine Psycologic CNS Cardiovascular Skin Other Inheritance
Cerebral palsy Visual impairment Depression Epilepsy Orthopaedic Genitourinary
1 : 500 Hearing Variable problems problems
impairment intellectual Neuromuscular Incontinence
capacity problems Constipation
Dental problems
Recurrent aspiration
Oesophagitis,
gastrooesophageal
reflux ± bleeding/
anaemia
Swallowing/eating
difficulties
APPENDIX 28 Problems Associated With Specific Causes of Disability
Down syndrome Visual impairment Hypothyroidism Depression Epilepsy usually Congenital heart Atlantoaxial Blood dyscrasias Most cases are
1 : 700 (multifactorial), Annual TFT Alzheimer clonic/tonic defects (present instability Childhood sporadic; 4%
cataracts recommended type in 40%–50%) Skin disorders, leukaemia due to
Hearing dementia alopecia, Sleep apnoea translocation
impairment (clinical onset eczema Increased involving
(multifactorial) uncommon susceptibility to chromosome
Annual before 40 infections 21 or rarely
assessments years) Coeliac disease parental
recommended mosaicism
Prader-Willi Strabismus NIDDM Hyperphagia Scoliosis, kyphosis Infantile failure to Atypical. Most
1 : 10,000– myopia (secondary to impulse Hypotonia thrive, then cases are
25,000 obesity) control Skin picking hyperphagia and sporadic
Hypogonadism difficulties severe obesity
Delayed puberty Self-injury High tolerance to
pain
Decreased ability to
vomit
Sleep apnoea
Osteoporosis
Undescended
testes
Dental abnormalities
Fragile X 1 : 6000 Visual impairment Attention deficit/ Epilepsy Aortic dilatation, Connective tissue Hernias (CT related) X-linked
(multifactorial) hyperactivity Usually clonic/ mitral valve dysplasia Abnormalities of
Hearing Variable tonic, prolapse (related Scoliosis speech and
impairment intellectual complex to connective Congenital hip language
Recurrent ear capacity partial tissue dysplasia) dislocation
infections Disabled in
social
functioning
Phenylketonuria Variable Epilepsy Eczema Autosomal
1 : 10,000– intellectual Hyperactivity recessive
20,000 capacity Tremor and
Phobic anxiety pyramidal
Disabled in tract signs
social Extrapyramidal
functioning syndromes
Angelmann Glaucoma Easily excitable Severe Joint contractures Speech impairment Variety of
syndrome Hyperactive developmental and scoliosis (in Movement and genetic
1 : 10,000 delay adults) balance disorder mechanisms
Epilepsy Characteristic EEG on
changes chromosome
15
Williams Hyperacusis Variable Perceptual and Cardiac Joint contractures Renal abnormalities Microdeletion on
<1 : 20,000 Strabismus intellectual motor abnormalities Scoliosis chromosome
capacity function Hypertension Hypotonia 7
Attention deficit reduced CVAs
problems in Chronic
childhood hemiparesis
Rett 1 : 14,000 Refractory errors Severe Epilepsy Prolonged QT Osteopenia Hyperventilation Usually sporadic
Females intellectual Vasomotor interval Fractures Apnoea X-linked
disability instability Scoliosis Reflux
Feeding difficulties
Growth failure
Continued
519
520
Muscular/
Psychiatric/ Skeletal and
Audiovisual Endocrine Psycologic CNS Cardiovascular Skin Other Inheritance
Noonan Strabismus Mild intellectual Epilepsy Pulmonary valvular Scoliosis Abnormal clotting Autosomal
<1 : 10,000 Refractive errors disability stenosis Talipes factors, platelet dominant,
Vision/hearing ASD, VSD, PDA equinovarus dysfunction may be
impairments Pectus carinatum/ Undescended tests, sporadic
excavatum deficient
spermatogenesis
Lymphangiectasia
Hepatosplenomegaly
Cubitus valgus,
hand
abnormalities
Tuberous Retinal tumours Variable Cerebral Rhabdomyomatas Bone Kidney and lung Autosomal
sclerosis Eye intellectual astrocytomas Hypertension Rhabdomyomata hamartomata dominant
1 : 6000– rhabdomyomatas capacity Epilepsy Polycystic kidneys
17,000 Behavioural Liver
difficulties rhabdomyomata
Sleep problems Dental abnormalities

Skin lesions
Neurofibromatosis Hearing Various Variable Variable clinical Skeletal Variable clinical Autosomal
1 : 300 impairment endocrine intellectual phenomena abnormalities phenomena dominant
(glioma affecting abnormalities capacity depending on especially depending on the
auditory nerve) site of the kyphoscoliosis location of the
tumours neurofibroma
Epilepsy Tumours are
susceptible to
malignant change
Other varieties of
tumours may be
associated
Adapted from an original unpublished version by Michael Kew and Glyn Jones and reproduced with the kind permission of the University of Queensland.
Appendix 29
The Community Dependency Index
© Professor Pamela Eakin, reproduced with permission. fasten and unfasten clothes, prevent spoiling of clothes, use
Score the patient under the following nine headings. A toilet paper without help. He may use equipment or stable
score <75 suggests moderate disability; <50 suggests severe fittings for support if needed (e.g., rail, raised toilet seat or side
disability. of bath). If he uses a commode he must be able to position it
for use, empty it and clean it out.
Personal Toilet 5 = Client has difficulty with part of this activity or client
5 = Client can wash hands and face, comb hair, clean teeth needs help because of imbalance or in handling clothes or in
and shave. Must be able to get to water, brushes, without help using toilet paper or in flushing the toilet.
and operate them independently. 0 = Client needs help to empty the commode or is not able
0 = Any help or supervision needed or difficulty with to transfer.
personal toilet. Note: If the client can use the toilet independently during
the day but has the commode at night, which someone else
Feeding empties, then score = 5.
10 = Independent. The client can feed himself a meal from a
Walking 50 Yards Outside the House or Using
tray or table when someone puts the food within reach. He
a Wheelchair
must put on his own assistive device if this is needed, cut up
food, spread butter and so on. He must accomplish this in a Walking
reasonable time (that is acceptable to the client). 15 = Client gets in/out of the house unassisted. He can walk
5 = Food must be cut for the client or some help is at least 50 yards without help or supervision outside his home.
necessary with the items above. Unreasonable time or effort He may wear braces and prostheses and use walking aids. He
required if feeds independently. must be able to reach and operate aids without help.
0 = Client unable to feed himself. 10 = Client has difficulty or needs minimal help or
supervision in any of the above but can walk at least 50 yards.
Moving From (Wheel) Chair to Bed and Return
Wheelchair
15 = Independent in all phases of this activity. 5 = Client cannot walk but can propel a wheelchair
Chair: client can safely stand up from sitting in his chair independently. He must be able to go round corners and turn
(high chair allowed) without help from another person, and sit around. He must be able to get in and out of the house
down again. Client must be able to get in/out bed without independently (access). He must be able to push the
help, and, once in bed be able to turn and move up and down wheelchair at least 50 yards. If the wheelchair is used indoors
in the bed as necessary. he must be able to manoeuvre himself to a table, bed or toilet.
Or wheelchair: client can safely approach the bed in his Do not score for wheelchair use if the client gets a score for
wheelchair, lock brakes, lift footrests, transfer safely to the bed walking.
and lie down. Once in bed he must be able to turn and move 0 = Client unable to walk or propel a wheelchair for 50
up and down the bed as necessary. Client must be able to yards.
transfer back into the wheelchair safely including changing the
position of the wheelchair for the return transfer. Dressing and Undressing
10 = Client can independently sit down and stand up from 10 = Client is able to put on and remove and fasten all clothing
chair, or transfer in and out of a wheelchair, but still has and tie shoelaces (adaptations/aids allowed). This activity
difficulty or needs help in bed. includes putting on and removing prostheses, braces and
5 = Help or supervision needed to ensure client’s safety in corsets where these are prescribed. Special clothing, such as
all parts of this activity; or client performs all or parts of this slip-on shoes or dresses that open down the front, may be
activity with difficulty. used where necessary.
0 = Client unable to perform this activity. 5 = Client has difficulty or needs help in putting on and
removing or fastening any clothing. Where helped he must do
Getting On and Off the Toilet or Commode
at least half the work himself. He must accomplish this in a
(During Day and Night)
reasonable time.
10 = Client is able to reach the toilet/commode area 0 = Client needs help with all or most of dressing.
unassisted. He is able to transfer on and off the commode,
Continued
521
522 APPENDIX 29 The Community Dependency Index
Bathing Self Note: If the client does not have stairs in the house, count
5 = Client can use a bath or shower. He must be able to do all as 10 because they are not an obstacle to independence in
the steps involved in whichever method is employed without the home, even if they are obstacles in the community.
another person helping him. Verbal supervision is allowed.
Continence of Bowels
0 = Client has difficulty or needs help.
Note: Where the client’s home does not have bathing/ 10 = Client is able to control bowels and has no accidents.
shower facilities, score 5 for using a bath/shower in another He can use a suppository or take an enema when necessary
facility or having an all-over wash if independent. Where client (e.g. spinal cord injury). He can manage external devices
has an all-over wash because he is unable to use the bath/ (e.g. colostomy).
shower then score = 0. 5 = Client needs help with any of the above.
0 = Client does not have bowel control.
Ascending and Descending Stairs
Continence of Bladder
10 = Client is able to get up and down stairs safely, without
help or supervision. He may and should use the handrails and 10 = Client able to control his bladder day and night. Clients
walking aids when needed. He must be able to carry walking who wear external device and leg bag must put them on
aids up and down the stairs if needed. If a stair lift or vertical independently, clean and empty the bag and stay dry day
lift is used, the client must be able to use it without help or and night.
supervision (including transfers). 5 = Client has control of bladder, but cannot get to the
5 = Client has difficulty, needs help or supervision in any toilet or commode in time (e.g. due to poor mobility) or needs
one of the above items. help with an external device.
0 = Client unable to climb the stairs. 0 = Client does not have bladder control.
Appendix 30
Nottingham Extended Activities of
Daily Living Questionnaire (EADL)
Score the patient on each item on a scale of 0 to 3 where “3” represents independent function, “2” represents alone with
difficulty, “1” represents alone with help, and “0” represents unable.
Subscale Item
Mobility 1. Do you walk around outside?
2. Do you climb stairs?
3. Do you get in and out of the car?
4. Do you walk over uneven ground?
5. Do you cross roads?
6. Do you travel on public transport?
Kitchen 7. Do you manage to feed yourself?
8. Do you manage to make yourself a hot snack?
9. Do you take hot drinks from one room to another?
10. Do you do the washing up?
11. Do you make yourself a hot drink?
Domestic 12. Do you manage your own money when you are out?
13. Do you wash small items of clothing?
14. Do you do your own housework?
15. Do you do your own shopping?
16. Do you do a full clothes wash?
Leisure 17. Do you read newspapers or books?
18. Do you use the telephone?
19. Do you write letters?
20. Do you go out socially?
21. Do you manage your own garden?
22. Do you drive a car?
Total (0–66)
523
Appendix 31
Drug Stabilities in Syringe Drivers
Notes on using tables of drug mixture stabilities • It is considered best practice to give dexamethasone as a
• The following tables are separated into mixtures contain- bolus dose as it has a long duration of action and fre-
ing two or three drugs, ordered by diamorphine first, quently causes compatability problems in mixture.
then the other drugs in alphabetical order. • The following combinations are not stable:
• The maximum dose for each drug in each syringe size is • diamorphine, dexamethasone, and levomepromazine
given. Provided the doses for every drug in the combina- • diamorphine, dexamethasone, and midazolam
tion is less than or equal to these maximum values, then • diamorphine, cyclizine, and metoclopramide
the mixture is stable for 24 hours. Above the maximum • octreotide and levomepromazine
doses stated the solution is either unstable or has not • octreotide and cyclizine
been tested and it is not possible to say whether it is • octreotide and dexamethasone
stable or not. • diamorphine, metoclopramide, and ondansetron
• All drug mixtures should be protected from light where
possible.
Maximum Dose (mg) Known to be Stable in:

14 mL in a 20-mL 17 mL in a 30-mL
Drug Combination 8 mL in a 10-mL Syringe Syringe Syringe Comments
Two drug combinations for subcutaneous infusion which are stable for 24 h
Diluent: Water for Injections BP
Diamorphine and 160 If diamorphine 280 If diamorphine 340 If diamorphine If exceed these doses
cyclizine 160* dose >160, 280* dose >280, 340* dose >340, then likely to get
cyclizine cyclizine cyclizine precipitate
dose must dose must dose must *Maximum
be no more be no more be no more recommended daily
than 80 than 140 than 170 dose 150
Diamorphine and 800 400 – – If exceed these doses
haloperidol 24 32 then likely to get
precipitate
Diamorphine and 1200 – – –
hyoscine HBr 3.2
Diamorphine and 1200 – – –
hyoscine butylbromide 160
(Buscopan)
Diamorphine and 47 82 90 –
ketorolac 40 74 90
Diamorphine and 400 700 850 Mixture can be irritant,
levomepromazine 80 140 170 dilute to largest
(Nozinan) possible volume
524
APPENDIX 31 Drug Stabilities in Syringe Drivers 525
Maximum Dose (mg) Known to be Stable in:

14 mL in a 20-mL 17 mL in a 30-mL
Drug Combination 8 mL in a 10-mL Syringe Syringe Syringe Comments
Diamorphine and 1200 2100 2550 Mixture can be irritant,
metoclopramide 40 70 85 dilute to largest
possible volume
Diamorphine and midazolam 400 700 850 —
16 28 34
Diamorphine and octreotide 200 350 425 —
0.9 1.6 1.9
Diamorphine and ondansetron 40 70 85
5 9 11
Three drug combinations for subcutaneous infusion which are stable for 24 h
Diluent: Water for Injections BP
Diamorphine and cyclizine and 160 280 340 Above these doses
haloperidol 160 280 340 the mixture is likely
16 28 34 to precipitate
Only stable if
diamorphine and
haloperidol are well
diluted before
dexamethasone is
added
Use only if no other
options
Diamorphine and haloperidol 560 980 1190 —
and midazolam 4 7 8.5
32 56 68
Diamorphine and 400 700 850 —
levomepromazine and 80 140 170
metoclopramide 24 42 51
Reproduced with permission from Scottish Intercollegiate Guidelines Network. (2008). Control of pain in patients with cancer. SIGN guideline 106. Edinburgh:
SIGN. Available from the Scottish Intercollegiate Guidelines Network, 9 Queen Street, Edinburgh EH2 1JQ. Online: www.sign.ac.uk.
Appendix 32
Guidelines for the Urgent Referral of
Patients With Suspected Cancer
Cancer Type Referral Guidelines

Lung cancer Urgent referral for chest x-ray
• Haemoptysis
• Unexplained or persistent (>3 weeks):
– Cough
– Chest/shoulder pain
– Dyspnoea
– Weight loss
– Chest signs
– Hoarseness
– Finger clubbing
– Features suggestive of metastasis from a lung cancer (e.g., brain, bone, liver, or skin)
– Cervical/supraclavicular lymphadenopathy
– Unexplained change in symptoms in someone with underlying chronic respiratory disease
Urgent referral to a chest physician
Any of the following:
• Chest x-ray suggestive/suspicious of lung cancer (including pleural effusion and slowly resolving
consolidation)
• Persistent haemoptysis in smokers/ex-smokers >40 years of age
• High suspicion of lung cancer despite normal chest x-ray
• History of asbestos exposure and recent onset of chest pain, shortness of breath, or unexplained systemic
symptoms where a chest x-ray indicates pleural effusion, pleural mass, or any suspicious lung pathology
• Signs of superior vena cava obstruction (swelling of face/neck with fixed elevation of jugular venous
pressure) (consider immediate referral)
• Stridor (consider immediate referral)
Upper Gl Urgent referral
cancer • Dysphagia: food sticking on swallowing (any age)
• Dyspepsia at any age combined with one or more of the following:
– Chronic Gl bleeding
– Progressive unintentional weight loss
– Iron deficiency anaemia
– Persistent vomiting
– Suspicious barium meal result
• Unexplained upper abdominal pain and weight loss, with or without back pain
• Upper abdominal mass
• Obstructive jaundice (depending on clinical state)
• Consider urgent referral for those with unexplained worsening of dyspepsia combined with at least one of
the following:
– Barrett oesophagus
– Peptic ulcer surgery >20 years ago
– Known dysplasia, atrophic gastritis, intestinal metaplasia
• Consider urgent referral for those without dyspepsia who have:
– persistent vomiting and weight loss
– unexplained weight loss and iron deficiency anaemia
• Refer urgently for endoscopy patients age ≥55 years with unexplained and persistent recent-onset
dyspepsia alone
526
APPENDIX 32 Guidelines for the Urgent Referral of Patients With Suspected Cancer 527

Lower Gl Urgent referral
cancer All ages
• A definite palpable right-sided abdominal mass consistent with involvement of the large bowel
• A definite palpable rectal (not pelvic) mass
• Men with unexplained iron deficiency anaemia and an Hb of ≤11 g/100 mL
• Women who are not menstruating with unexplained iron deficiency anaemia and an Hb of ≤10 g/100 mL
≥40 years old
• Rectal bleeding with a change in bowel habit to looser stools and/or increased frequency of defecation
persistent for 6 weeks
≥60 years old
• Rectal bleeding for at least 6 weeks without a change in bowel habit and without anal symptoms
• Change of bowel habit to looser stools and/or increased frequency of defecation, without rectal bleeding
and persistent for 6 weeks
Breast cancer Urgent referral
• Patients with a discrete hard lump with fixation
• Women aged ≥30 with a discrete lump that persists after their next period, or presents after the
menopause
• Women aged <30 with a lump that enlarges, or is fixed and hard, or in whom there are other reasons for
concern (eg, family history)
• Those with a lump or with suspicious symptoms and a past history of breast cancer
• Those with unilateral eczematous skin or nipple change that does not respond to topical treatment
• Those with nipple distortion of recent onset
• Those with spontaneous unilateral bloody nipple discharge
• Men aged ≥50 with a unilateral, firm subareolar mass
Conditions that require referral—but not necessarily urgently
• Discrete lump in younger women (age <30 years)
• Breast pain not responding to initial treatment and/or with unexplained persistent symptoms
Gynaecologic Urgent referral
cancer • Clinical features suggestive of cervical cancer on examination
• Unexplained vulval lump
• Vulval bleeding due to ulceration
• Postmenopausal bleeding (PMB) in women not on HRT
• On tamoxifen with PMB
• On HRT: persistent or unexplained bleeding for >6 weeks after stopping HRT
Consider urgent referral
• Persistent intermenstrual bleeding and negative pelvic examination
Urgent ultrasound scan (USS)
• Palpable abdominal or pelvic mass that is not obviously uterine fibroids or gastrointestinal or urologic. If the
scan suggests cancer, or if urgent USS is not available, urgent referral should be made
Urologic Urgent referral
cancers Prostate:
– Hard irregular prostate typical of carcinoma
– Normal prostate but rising or raised age-specific PSA, with or without lower urinary tract symptoms
Bladder and kidney:
– Painless macroscopic haematuria
– Age ≥40 with recurrent or persistent urinary tract infection associated with haematuria
– Unexplained microscopic haematuria in adults aged ≥50 years
– Abdominal mass identified clinically or on imaging thought to arise from the urinary tract
Testis:
– Swelling or mass in the body of the testis
Penis:
– Any suspected penile cancer
Non-urgent referral of microscopic haematuria in a patient aged <50 years
– To a renal physician if there is proteinuria or a raised serum creatinine
– To a urologist if there is no proteinuria and serum creatinine is normal
Haematologic Urgent referral
cancers • Blood count/film reported as suggestive of acute leukaemia (immediate referral)
• Spinal cord compression or renal failure suspected of being due to myeloma (immediate referral)
• Persistent unexplained splenomegaly
Continued
528 APPENDIX 32 Guidelines for the Urgent Referral of Patients With Suspected Cancer

Skin cancers Urgent referral
• A lesion suspected to be melanoma
• Suspicion of squamous cell carcinoma:
– Non-healing keratinizing or crusted tumours >1 cm with significant induration on palpation
– Patients in whom squamous cell carcinoma has been diagnosed from a biopsy undertaken in general
practice
– After an organ transplant with new or growing cutaneous lesions
Head and neck Urgent referral
cancer • Unexplained lump in the neck that is of recent onset or a previously undiagnosed lump that has changed
over 3–6 weeks
• Unexplained persistent swelling in the parotid or submandibular gland
• Unexplained persistent sore or painful throat
• Unexplained persistent pain in the head or neck for >4 weeks associated with otalgia (earache) but a
normal otoscopy
• Unexplained ulceration of oral mucosa or mass persisting for >3 weeks
• Unexplained red and white patches of the oral mucosa (including suspected lichen planus) that are painful
or swollen or bleeding
• Any other patients with persistent symptoms or signs (>6 weeks) related to the oral cavity in whom a
definitive diagnosis of a benign lesion cannot be made
• Unexplained tooth mobility lasting >3 weeks (to a dentist)
• Hoarseness for >3 weeks. Order urgent chest x-ray. If positive, refer to a team specializing in the
management of lung cancer. If negative, refer urgently to a team specializing in head and neck cancer
Thyroid cancer
• Tracheal compression due to thyroid swelling (immediate referral)
• Solitary nodule increasing in size
• Thyroid swelling associated with:
– history of neck irradiation
– family history of an endocrine tumour
– unexplained hoarseness or voice changes
– cervical lymphadenopathy
– prepubertal patient or patient aged 65 or older
Brain tumours Urgent referral: when a brain tumour is suspected in a patient with:
– progressive neurologic deficit
– new onset seizures
– headaches
– mental changes
– cranial nerve palsy
– unilateral sensorineural deafness
• Headaches of recent onset with features suggestive of raised intracranial pressure (eg, vomiting,
drowsiness, posture-related headache, pulse-synchronous tinnitus, or other focal or non-focal neurologic
symptoms)
• A new, qualitatively different, unexplained headache that becomes progressively severe
• Suspected recent-onset seizures (refer to neurologist)
Consider urgent referral when there is rapid progression of:
• subacute focal neurologic deficit
• unexplained cognitive impairment, and/or behavioural disturbance
• personality change
Sarcoma Urgent referral
Suspected spontaneous fracture needs immediate x-ray. Refer if it suggests possible cancer. A palpable lump
with one or more of the following characteristics:
– Size >5 cm
– Painful
– Increasing in size
– Deep to fascia, fixed or immobile
– Recurrence after previous excision
• Suspected Kaposi sarcoma in a patient who has HIV
• Increasing, unexplained, or persistent bone pain or tenderness, particularly pain at rest (especially if not in
the joint) or an unexplained limp
Investigate urgently first
APPENDIX 32 Guidelines for the Urgent Referral of Patients With Suspected Cancer 529
Children’s
Cancers Referral Guidelines
General Urgent referral
• When a child presents three or more times with the same problem and investigation reveals no clear
diagnosis
• Persistent back pain after investigation and taking parental anxiety into account
Leukaemia • Unexplained petechiae (refer immediately)
• Hepatosplenomegaly (refer immediately)
• Take blood for FBC and film (and refer urgently if positive) if any of the following is present:
– Fatigue
– Pallor
– Unexplained irritability
– Unexplained fever
– Persistent or recurrent upper respiratory tract infections
– Generalized lymphadenopathy
– Persistent or unexplained bone pain
– Unexplained bruising
Lymphoma • Hepatosplenomegaly (immediate referral)
• Mediastinal or hilar mass on chest x-ray (immediate referral)
• Lymphadenopathy (particularly if there is no evidence of previous local infection) with at least one of the
following:
– Nodes >2 cm in size
– Nontender, firm/hard nodes
– Progressively enlarging
– Associated with other signs of general ill health, fever, and/or weight loss
– Involves axillary nodes (in the absence of any local infection or dermatitis) or supraclavicular nodes
• Shortness of breath and unexplained petechiae or hepatosplenomegaly
Brain and CNS Urgent referral
tumours Altered level of consciousness (immediate referral)
• Headache and vomiting that cause early morning waking or occur on waking (immediate referral)
• Children aged <2 years with any of the following (immediate referral):
– Bulging fontanelle
– Extensor attacks
– Persistent vomiting
– New onset seizures
• Children of any age with any of the following (immediate or urgent referral):
– New onset seizures
– Cranial nerve abnormalities
– Visual disturbance
– Gait abnormality
– Motor or sensory signs
– Unexplained deteriorating school performance or developmental milestones
– Unexplained behavioural and/or mood changes
• Children age ≥2 years, and young people, with persistent headache where you cannot carry out an
adequate neurologic examination
• Children <2 years old with any of the following:
– Abnormal increase in head size
– Arrest or regression of motor development
– Altered behaviour
– Abnormal eye movements
– Lack of visual following
– Poor feeding or failure to thrive
– Squint (where the urgency will depend on other factors)
Neuroblastoma Refer urgently children with:
– proptosis
– unexplained back pain, leg weakness
– unexplained urinary retention
Continued
530 APPENDIX 32 Guidelines for the Urgent Referral of Patients With Suspected Cancer
Children’s
Cancers Referral Guidelines
Wilms tumour Refer urgently children with haematuria
Soft tissue Refer urgently children with an unexplained mass if associated with one or more of the following
sarcoma characteristics:
– Nontender
– Progressively enlarging
– Size >2 cm in maximum diameter or deep to fascia
– Associated with enlarging regional lymph node
Bone sarcoma Refer children or young people with rest pain, back pain, or unexplained limp (discuss with paeditrician). Refer
urgently if there is persistent localized bone pain and/or swelling and x-ray suggests cancer
Retinoblastoma Refer urgently children with a white pupillary reflex, a new squint, or change in visual acuity suggestive of
cancer, or any visual problems and a family history of retinoblastoma
Crown copyright. Reproduced with permission. More details of the guidelines, and proformas for the referral of patients, are available on www.dh.gov.uk (search
on “suspected cancer”). This version is taken from the draft for the second consultation, 2004. The definitive version, 2005, is now available on www.nice.org.
uk. It does not differ from this version in any material way but it enlarges on the points made above.
Appendix 33
Opioid Dose Conversion Chart
(all doses in milligrams (mg) unless otherwise stated)
Oral
Opioid Opioid Transdermal
(24 h) CSCI Opioid (24 h) Subcutaneous PRN Opioid q4h Patch
Buprenorphine
Diamorphine
Diamorphine
Oxycodone
Oxycodone
Oxycodone
Morphine
Morphine
Morphine
Alfentanil
Fentanyl
Fentanyl
Fentanyl
20 10 10 5 5 500 µg 100 µg 2 1 1 12.5 µg – 10 µg/h
30 15 15 7.5 10 1 mg 200 µg 2.5 1.5 2 25 µg – 15 µg/h
45 20 20 10 15 1.5 300 µg 4 2 2.5 37.5 µg 12 µg/h 20 µg/h
90 45 45 20 30 3 600 µg 8 4 5 75 µg 25 µg/h 35 µg/h
140 70 70 35 45 4.5 900 µg 12 6 8 100 µg 37 µg/h 52.5 µg/h
180 90 90 45 60 6 Max. CSCI 15 8 10 Max. PRN 50 µg/h 70 µg/h
900 µg as 100 µg as
230 115 115 60 75 7.5 limited by 20 10 12.5 limited by 62 µg/h 105 µg/h
270 135 135 70 90 9 volume 25 12 15 volume 75 µg/h 122.5 µg/h
(50 µg/mL) (50 µg/mL)
360 180 180 90 120 12 30 15 20 100 µg/h 140 µg/h
450 225 225 110 150 15 35 18 25 125 µg/h (Manufacturer’s
recommended
540 270 270 135 180 18 45 20 30 150 µg/h maximum is
630 315 315 160 210 21 50 25 35 175 µg/h 140 µg/h)
720 360 360 180 240 24 60 30 40 200 µg/h
Notes:
• The conversions stated here are only approximate, and are based upon manufacturers’ and other sources of data. There may be local guidelines stating
different conversion ratios: these should be followed where they exist.
• When switching between opioids, it is recommended that the calculated equivalent dose of the new opioid is reduced by 25–50%.
• Alfentanil: Fentanyl based on 5:1 (this is the modal average taken from several different data sources; range 3.3–6:1)
• Fentanyl PRN dose is calculated as 1/8th of 24 h dose (mean average taken from different sources; range 1/10th to 1/6th )
531
532 Index
Index
Page numbers followed by “f ” indicate figures, “t” indicate tables, and “b” indicate boxes.
A Acute severe asthma, management of

Abbreviated Mental Test Score, 234, 234b in adults, 489, 490f
Abdominal migraine, 43 in children, 491, 492f
Abdominal pain Acute stroke, 182–183
child with, 43–44 Acyclovir, for disseminated HSV, 391
dysuria and, 50–51 Adenomyosis, 192
inflammatory bowel disease, 115 Adenoviral gastroenteritis, incubation period and infectivity of,
irritable bowel syndrome, 113 472t–473t
in pregnancy, 217 Adherence, 14
acute fatty liver, 124 Adjustment reaction, 320
Abortion Act (1967), 263 Admission
Abruptio placentae, 217 procedures, for patients with mental health problems, 506–507,
Abscesses, 393 506t–507t
amoebic liver, 301 ulcerative colitis, 117
breast, 225 Adolescents
dental, 367 with Down syndrome, 32
peritonsillar, 366 irregular periods, 194–195
Absence seizures, 177 self-harm and suicide of, 316
Abuse termination of pregnancy in, 264
child (nonaccidental injury), 63–64 Adrenal crisis, 421
in older people, 241–242 Adrenaline, for anaphylaxis, 406, 406t
Acarbose, for type 2 diabetes mellitus, 414 Adrenarche, 60
Achilles tendon rupture, 153 Adults
Achilles tendonopathy, 153 asthma, management of, 489, 490f
Acid reflux, palliative care and, 449 acute severe, 487, 487f
Acne, polycystic ovary syndrome, 196 atopic eczema in, 382
Acne conglobata, 386 with Down syndrome, 32–33
Acne fulminans, 386 Advance directives
Acne rosacea, 387–388 chronic schizophrenia, 331
Acne vulgaris, 386–387 Huntington disease, 188
Acoustic neuroma, 357 Advocacy, 30
Acral melanoma, 399 Age-related macular degeneration (AMD), 377–378
Acromioclavicular joint arthritis, 149 Aggressive behaviour, rapid tranquilisation, 327
pain, 150 Agitation, 436
Actinic keratosis, 396–397 acute psychosis, 327
Activities of daily living, advanced neurologic disease and, 462 end-stage renal failure and, 467
Acupuncture management of, 436
low back pain, 147 in older people, 232–234
migraine, 172 presenting features of, 436
Acute closed angle glaucoma, 376 Agoraphobia, patients with, 325–326
Acute confusion, in older people, 232–234 Air travel, in chronic heart failure, 76
Acute coronary syndrome (ACS), 71–73 Alanine transaminase, 124
cardiac rehabilitation for, 72–73 Alcohol, 360
factual advice for, 73 cardiovascular disease, 86
immediate management of, 72b chronic heart failure, 76
myocardial infarction and, 72 compulsory admission, 329
Acute dacryocystitis, 375 dyspepsia, 110
Acute infective conjunctivitis, 372 essential tremor, 189
Acute pulmonary oedema, 79 gout, 157
Acute respiratory distress, 435–436 hypertension, 68
Acute right heart failure, 79 insomnia, 322
532
Index 533
myocardial infarction, 73 management of, 406–407

older people, 230 acute, 406–407, 406t
osteoporosis, 164 Angelmann syndrome, problems associated with,
posttraumatic stress disorder, 321 517t–520t
prepregnancy advice, 212–213 Angina
snoring management, 364 see also Chest pain
stroke prevention, 183 with hypertension, treatment of, 69
Alcohol Use Disorders Identification Test (AUDIT), 509–510, stable, 73–75
509t–510t unstable, 71–73
Alcoholic hepatitis, 127 Angiotensin converting enzyme (ACE) inhibitors
Aldosterone antagonists, for heart failure, 78 cardiovascular disease, 88
Alert bracelet, for anaphylaxis, 406 chronic heart failure with left ventricular systolic
Alfentanil, 441 dysfunction, 77
Alkaline phosphatase, 124 hypertension, 70
Allergic conjunctivitis, 372 Angiotensin II receptor antagonist
Allergies, 403–408 chronic heart failure with left ventricular systolic
see also Anaphylaxis dysfunction, 77
chronic simple glaucoma, 378 chronic renal disease, 352
contact dermatitis, 384–385 Ankle injury, 152–153
cellulitis and, 393 Ankle oedema, postural, 241
Allopurinol, 157 Ankle sprain, 152
Alopecia, 402 Ankylosing spondylitis, 161
Alopecia areata, 402 Anogenital warts, 270, 306
Alpha-blockers Anorexia, 334
for benign prostatic hypertrophy, 347 in end-stage heart failure, 79
for hypertension, 70 palliative care and, 450
Alzheimer disease, 237, 237t Anserine bursitis, 152
see also Dementia Antenatal care, routine, 214–215
Ambulatory blood pressure monitoring (ABPM), 67b Antiandrogens, for hidradenitis suppurativa, 401
Amelanotic melanoma, 398–399 Antibiotic-associated diarrhoea, 117
Amenorrhoea, 195–196 Antibiotics
injectable progestogens and, 256 acne vulgaris, 387
Aminosalicylates, 115 acute infective conjunctivitis, 372
Amitriptyline, 441, 456 acute pyelonephritis, 344
for migraine prophylaxis, 173 chronic obstructive pulmonary disease, acute
Amlodipine exacerbation, 104
for hypertension, 70 community-acquired pneumonia, 100
for stable angina, 74 halitosis, 368
Amniocentesis, 215 leg ulcers, 400
Amoebic liver abscess, in travellers returning from abroad, 301 lower urinary tract infection, 341
Amphetamines, pregnancy and, 224 otitis media
Amyotrophic lateral sclerosis, 463–464 acute, 355
ventilatory support for, 463 diffuse, 356
Anaemia prophylaxis
iron deficiency (see Iron deficiency anaemia) during IUD insertion or removal, 259
in pregnancy, 222 joint replacement patient, 145
prepregnancy care for, 213 rhinosinusitis, 361
Anal fissure, 138–139 sore throat, 364
Analgesia streptococcal tonsillitis, 364
Achilles tendonopathy, 153 urinary tract infection, 341
dysmenorrhoea, 192 Anticholinergics
low back pain, 146 for Ménière’s disease, 359
migraine, 171 for motor neurone disease, 185
osteoarthritis, 145 for multiple sclerosis, 187
rheumatoid arthritis, 159 for Parkinson disease, 181
shoulder pain, 149–150 for urge incontinence, 345
spondyloarthropathies, 161 Anticoagulation, in atrial fibrillation, 80–83
urinary stone, 349 paroxysmal, 84
Analgesic-overuse headache, 175 risk factors for, 81–82, 82t
Anaphylaxis, 405–407 Anticonvulsants
algorithm, 516, 516f adverse effects of, 178b
diagnosis of, 406 in pregnancy, 212
534 Index
Antidepressants management of
for breathlessness, 445 in adults, 487, 487f
factors influencing choice of, 316–317 in children, 488, 488f
for posttraumatic stress disorder, 321 in pregnancy, 223
for rheumatoid arthritis, 159 prepregnancy care for, 212
Antiemetics, 447t–448t Asylum seekers see Immigrants
for migraine, 171 Atenolol, for stable angina, 74
for Parkinson disease, 182 Atopic eczema, 382–384
Antifibrinolytics, menorrhagia, 194 complications of, 382
Antihistamines, rhinitis, 363 emollients in, 383
Antihypertensives general advice for, 382–383
for hypertension, 68–69 bottle and breastfed infants, 383
in pregnancy, 220 with irritant contact dermatitis, 384
Antiinflammatory therapies, for acne vulgaris, 387 referral for, 384
Antiplatelet therapy steroids for, 383–384, 383t
for cardiovascular disease, 88 mild flares, 383
for myocardial infarction, 73 moderate flares, 383–384
Antipsychotics severe flares, 384
for chronic schizophrenia, 332 Atrial fibrillation (AF), 80–85
for Parkinson disease, 182 anticoagulation in, 82–83
Antithrombin III deficiencies, 248 risk stratification for, 81–82, 82t
Antithyroid drugs, 419 atrial flutter and, 84
Anxiety bleeding risk in, assessment of, 82, 82t
disorders, 322–326, 323t bradycardia and, 85
end-stage renal failure, 466 cardioversion versus rate control in, 81
generalized, 323–325 identification of, 81
irritable bowel syndrome, 113 paroxysmal, 83–84
multiple sclerosis, 186 prevalence of, 80
in older people, 238 sick sinus syndrome and, 85
palliative care and, 456 supraventricular tachycardia and, 84–85
social, 325 ventricular tachycardia and, 85
SSRI for, 324 workup of, 81b
Aphthous ulcers, 367 Atrial flutter, 84
Apixaban, for atrial fibrillation, 83 Attendance Allowance, 145
Approved mental health professional (AMHP), Attorney, older people and, 243
328 Atypical antipsychotics, for chronic schizophrenia, 332
Arc eye, 374 Atypical naevus, 397
Arrhythmias, in heart failure, 79–80 Autoimmune hepatitis, 126
Arterial thrombosis, 248 Autonomic dysreflexia, 188
Arterial ulcers, 392 Autonomy, respect for, in four principles, 23, 23t
Artificial saliva, 367 Axillary hair, development of, 482
Artificial tears, 373 Azathioprine, 495
Ascites, 128
palliative care and, 450 B
Aspergillus infection, refractory otitis externa, 356 Bacillary dysentery, incubation period and infectivity of, 472t–473t
Aspirin Bacille Calmette-Guérin (BCG) immunization, immigrants, 101
contraindications, 95, 110, 154 Back pain, low, 145–148
for hypertension, 68 Baclofen, 442
in pregnancy, 220 for Parkinson disease, 182
for migraine, 171 Bacterial infection
renal function effects, 350 in atopic eczema, 382
tinnitus and, 360 in skin, 392–394
Assistive technologies, rehabilitation and, 28 Bacterial vaginosis, 198
Asthma, 93–98 Bacteriuria
acute, 98 asymptomatic, 342
acute severe, management of in pregnancy, 221
in adults, 489, 490f Balanitis, 55
in children, 491, 492f Barbiturates, pregnancy and, 224
children, 94, 98 Barrett oesophagus, 112
control, for anaphylaxis, 407 Barrier methods, 260
with hypertension, treatment of, 69 Basal cell carcinoma, 397–398
infants, 94–95 subtypes of, 398t
Index 535
Beard, seborrheic dermatitis in, 386 Blood pressure (BP)

Bedding protection, 345 in cardiovascular disease, control of, 87
Bedwetting, children with, 51–52 in hypertension, 67
Behaviour, change in, agitation in older people and, 233 management of, in type 2 diabetes, 415
Bell’s palsy, 361 in pregnancy, 220
Bence Jones protein, 350 Blunt injuries, in eye, 374
Beneficence, in four principles, 23–24, 23t Body, seborrheic dermatitis in, 386
Benign paroxysmal positional vertigo (BPPV), 359–360 Body hair, excess, child with, 60
Benign prostatic hypertrophy (BPH), 344 Body mass index, 513, 513f
bladder outflow problems, 346–348 Bone densitometry scan, 120
Benign skin lesions, 395–396 Bone mineral density (BMD), reduced, injectable progestogens and,
Benzodiazepines 256
for bipolar affective disorder, 333 Bone sarcoma, 526t–530t
for breathlessness, 445–446, 446t Bottle-fed infants, atopic eczema and, 383
for generalized anxiety, 323 Botulinum toxin, 189
for insomnia, 322 type A, in chronic migraine, 173
for Ménière’s disease, 359 Bowen disease, 397–398
for posttraumatic stress disorders, 321 Bowenoid papulosis, 397
in pregnancy, 224 Boys, FEV1/FVC chart, 486t
Bereavement, following suicide, 320 Bradycardia, 85
Beta2-agonists, chronic obstructive pulmonary disease, 102–103 Brain tumours, 526t–530t
Beta-blockers Branch retinal vein occlusion, 376
for anxiety disorders, 324 Breakthrough pain, 436
for chronic heart failure with left ventricular systolic dysfunction, Breast abscess, 225
77 Breast cancer, 202, 526t–530t
for chronic simple glaucoma, 378 etonogestrel-releasing implant (Implanon), 257
contraindications, 95 injectable progestogens and, 255
for hypertension, 70 oral contraception, contraindications, 248
for migraine prophylaxis, 173 Breast development, in female, 481f, 482
for myocardial infarction, 73 Breast engorgement, 225
for panic disorder, 324 Breastfed infants, atopic eczema and, 383
for phobias, 325 Breastfeeding, 224–225
for posttraumatic stress disorders, 321 food allergies and, 405
for social anxiety/social phobia, 325 inflammatory bowel disease, 118
for stable angina, 74 oral contraception, contraindications, 248
Betamethasone valerate, for severe flares, 384 Breathlessness
Biguanide, 412 chronic obstructive pulmonary disease, 101
Bile salt malabsorption, 119 end-stage heart failure, 79
Biliary colic, 135 end-stage renal failure, 467
Biliary/pancreatic disease, 113 MRC dyspnoea scale, 102
Bilirubin elevation, 124 palliative care, 443–446, 444f, 444t
Binge eating disorder (BED), 334 Breech presentation, 222
Bioethics, 20t Bronchodilators
Biologic therapy monitoring, 156 asthma, 95, 98
Biomedical ethics, 20t chronic obstructive pulmonary disease, 102–103
Bipolar affective disorder, 314–315, 333–334 Bronchorrhoea, 446
Birth, development in, 478t–479t B-type natriuretic peptide (BNP), for heart failure, 76
Bisoprolol, for stable angina, 74 Bulimia, 334
Bisphosphonates, 164 Bullous myringitis, 355–356
Bladder outflow problems, benign prostatic hypertrophy and, Bunions, 153
346–348 Buprenorphine, 440
Bladder retraining, 345 Burnout syndrome, 5
Bleeding
assessment of, 82 C
palliative care and, 455–457 Cachexia, palliative care and, 450
Blepharitis, 372–373 Calcium channel blockers
Blepharospasm, 375 for hypertension, 70
Blindness see Visual handicap for migraine prophylaxis, 173
Blood for stable angina, 74
reference ranges for, in young adults, 514t–515t Calcium intake, osteoporosis and, 164
in urine, child with, 53 Calgary Cambridge method, 15
Blood monitoring schedule, DMARD and, 495 Campylobacter, incubation period and infectivity of, 472t–473t
536 Index
Cancer Central retinal vein occlusion, 376

injectable progestogens and, 256 Cerebral palsy, problems associated with, 517t–520t
oral contraception, risk of, 250–251 Cerebrospinal fluid leak, vaccination for patients with, 283
screening, in older people, 230 Certification, in visual handicap, 379
suspected, guidelines for urgent referral of patients with, Cervical cancer, 206
526–530, 526t–530t Cervical caps, 260
Candida infection, 197–198 Cervical lymphadenopathy, child with, 57
breastfeeding problems, 225 Cervical myelopathy, 149
refractory otitis externa, 356 Cervical screening, 203, 205–206, 206t
vaginal, 197 CHA2DS2-VASc, 82, 82t
Candidiasis, 394–395 Chalazion, 375
oral, palliative care and, 451 Chemical burns, in eye, 373–374
Capacity, legal issues of, 30–31 Chemical induced acne, 386
Capsulitis, chronic, 150 Chest pain, 71
Car travel, in pregnancy, 213 see also Angina
Carbamazepine, 500t child with, 46
Carbimazole, 419 noncardiac, 75
Carbonic anhydrase inhibitors, in chronic simple glaucoma, 378 Chickenpox see Varicella
Carbuncles, 393 Child abuse (nonaccidental injury), 63–64
Cardiac devices, end of life Child development, stages of, 478–480, 478t–480t
after death, 470 Child health promotion, 476–477, 476t–477t
deactivation in Children
defibrillator component, 470 see also Adolescents; Infants
pacemaker function, 470 with abdominal pain, 43–44
practicalities of, 470 acute infective conjunctivitis in, 372
management of, 469–470 asthma, management of, 488, 488f
types of, 469 acute severe, 491, 492f
Cardiac disease, 316 atopic eczema in, 382
prophylactic antibiotics for, 259 with bedwetting (nocturnal enuresis), 51–52
Cardiac rehabilitation, for myocardial infarction, 72–73 with blood, in urine, 53
Cardiology, for stable angina, 75 cancers, general, 526t–530t
Cardiovascular disease cataracts of, 377
erectile dysfunction and, 266 with cervical lymphadenopathy, 57
hormone replacement therapy, 202–203 with chest pain, 46
oral contraception, contraindications, 248 with constipation, 42–43
Cardiovascular problems, 65–92 with cough, 40–41
prevention of, 85–88 with depression, 316
CHD prevention clinic, annual workup at, 88 with diarrhoea, 44–45
intensive management in, 87 with Down syndrome, 32
lifestyle changes in, 86–87 with dysuria and abdominal pain, 50–51
primary, 86 with excess body hair, 60
secondary, 87–88 with febrile seizure, 48–49
statin in, 87 with fever, 38–40
Cardioversion, in atrial fibrillation, 81 with glycosuria, 411
CARE approach, 15–16 with groin swelling, 55–56
Care pathway, for respiratory tract infections, 493–494, 493t–494t with headaches, 49–50
Carers, 33 health, 37–64
caring for, 33 with itchy perineum, 54
chronic heart failure, 77 with large head, 47–48
needs, 332 with murmur, 45
Parkinson disease, 179 with nonaccidental injury, 63–64
resources for, 33 with nonblanching rash, 56–57
stress, older people and, 232 normal peak flow values in, 483, 483t
Carpal tunnel syndrome, 151 with painful limp, 60–61
Cataracts, 377 with painful penis, 55
in atopic eczema, 382 with painful scrotum, 56
Catheter ablation, in paroxysmal atrial fibrillation, 84 with pica, 57–58
Cauda equina syndrome, 147, 344 with precocious puberty, 59–60
Cellulitis, 393 with protein, in urine, 52–53
lymphoedema and, 453–454 secretory otitis media (glue ear), 357–358
Cement, in eye, 374 with seizure, 49
Central retinal artery occlusion, 375–376 short, 58–59
Index 537
with small head, 47 for generalized anxiety, 323

tall, 59 for obsessive-compulsive disorder, 326
with tic, 50 for phobias, 325
warts in, 390 Cognitive impairment
with wheeze, 41–42 see also Dementia
who collapse, 45–46 Alzheimer disease, 238–239
who does not walk aged 15 months, 47 Huntington disease, 188
with worms in the stool, 54–55 multiple sclerosis, 187
Chiropractic treatment urinary incontinence, 345
low back pain, 147 Cognitive symptoms, dementia and, 237
migraine, 172 Coital headache, 175
Chlamydia infection, 269–270, 305 Colchicine, as alternative to NSAID, 156
pelvic inflammatory disease, 198 Cold, common, 362
secondary dysmenorrhoea, 192 Collapses, child who, 45–46
Chlorphenamine, for anaphylaxis, 406t Colonoscopy, ulcerative colitis surveillance, 117
Chlorthalidone, for hypertension, 69–70 Colorectal cancer, 115, 200
Cholecystitis, 135 Colposcopy, 206
Choledocholithiasis, 135 Combination therapies, for acne vulgaris, 387
Cholelithiasis (gallstones), 134–135 Combined oral contraceptive (COC) pill,
oral contraception, risk of, 250 247–253
Cholera, 297 advice for, 250–251
Cholesteatoma, 356–357 assessment of patient and, 247–248
Cholinesterase inhibitors, in Alzheimer disease, 237 bleeding and, 251
Chondrodermatitis nodularis helicis (CNH), 398 choosing, 249
Choriocarcinoma, 200–201 contraindications, 248
Chorionic villus biopsy (CVB), 215 diarrhoea or vomiting and, 251
Chromosomal abnormalities, types of screening test for, 215 effectiveness of, 247
Chronic allergic eye disease, 372 hypertension, 68
Chronic cor pulmonale, 79 menorrhagia treatment, 194
Chronic daily headache, 175 missed pills and, 251, 252f
Chronic diarrhoea, 118–119 noncontraceptive benefits of, 251
Chronic heart failure in older women, 262
with left ventricular systolic dysfunction, 77–78 procedure and advice for, 250, 250t
management of, 76–77 side effects of, 251
general measures for, 76–77 stopping, 250
Chronic kidney disease (CKD), 351–353, 352t in young people, 262
Chronic liver disease, 127–129, 128t Combined transdermal patch, 253
Chronic medical conditions, vaccination for patients with, 283 Combined vaginal ring, 253–254
Chronic obstructive pulmonary disease, 101–102 Comedolytic therapies, for acne vulgaris, 387
Chronic paroxysmal hemicrania, 175 Commodes, 346
Chronic plaque psoriasis, 388–389 Common disease, incubation period and infectivity of, 472–473,
Chronic schizophrenia, 330–332 472t–473t
drug treatment of, 331–332 Common wart, 390
Chronic simple glaucoma, 378–379 Communication, advanced neurologic disease and, 462
Chronic venous insufficiency, 393 Communication skills, 11–18
Chymopapain discolysis, 147 adherence and compliance and, 14
Ciliary body ablation, for chronic simple glaucoma, 379 complaints and, 16
Cirrhosis, etonogestrel-releasing implant (Implanon), 257 consultation and, 14–15
Citalopram, 456 Calgary Cambridge method of, 15
Clinical pharmacist, primary care and, 3 CARE Approach to, 15–16
Clinical staff, vaccination for, 283 email, 17
Clock drawing test, 234 inner, 15
Cluster headache (migrainous neuralgia), 174 structure of, 14–16
CNS tumours, 526t–530t telephone, 16–17
Cocaine, in pregnancy, 224 ideas, concerns, effect and expectations in, 12–13
Cochlear implants, vaccination for patients with, 283 interpreters and, 14
Codeine, 438 nonverbal, 12
Coeliac disease, 119–121 paralinguistics and, 12
Coffee, in hypertension, 68 physical arrangement of the room and, 14
Cognitive behavioural therapy (CBT) professional requirement of, 11–12
for anxiety disorders, 322–323 rapport, 12
for depression, 317–318 shared decision making and, 13–14
538 Index
Communication skills (Continued) barrier methods, 260

sharing information and, 13, 13t connective tissue diseases, 162
verbal, 12 emergency, 261–262
Communications technology, primary care and, 4 Family Planning Association (FPA), leaflets from, 247
Community dependency index, 521 hormonal, 247–258
Community-dwelling older people, falls and, 240 hormone replacement therapy and, 205
Community Treatment Order (CTO), 329 intrauterine devices, 258–260
Comorbidity, 7–8 intrauterine system, 259–260
Complaints, how to react to, 16 and learning disability, 263
Compliance, 14 migraine, 172
Comprehensive geriatric assessment, 231 nonhormonal methods of, 258–261
Comprehensive health care, 31 and older women, 262–263
Compression, joint limb injury, 152 postnatal, 224
Compression stockings, for leg ulcers, 400 in special cases, 262–263
Compulsory admission, 328–330 and young people, 262
Compulsory treatment, 330 Contractures, in multiple sclerosis, 186
Condoms Conventional antipsychotics, for chronic schizophrenia, 332
female, 260 Convulsions see Epilepsy
male, 260 Cor pulmonale, 101, 104
Conductive hearing loss, 357 Corneal abrasion, 374
Confidentiality, 14 Corneal foreign body, 373
email consultation and, 17 Coronary angiography, for stable angina, 75
telephone consultation and, 16–17 Coronary artery bypass grafting (CABG), for stable angina, 74–75
Confusion, 452b Corticosteroids, monitoring patients taking, 457
chronic, 453 Cortisol, reference ranges for, in young adults, 514t–515t
Confusion Assessment Method (CAM), 232–233 Cough, 100–101
Conjunctivitis child with, 40–41
acute infective, 372 motor neurone disease and, 464
allergic, 372 mucolytic therapy, 103
in contact lenses, 374 nonproductive, 446
incubation period and infectivity of, 472t–473t palliative care and, 446
Connective tissue diseases, 162–163 productive
Consanguineous couples, prepregnancy genetic counselling, 210 able to expectorate, 446
Consent unable to expectorate, 446
expressions of, 13–14 Counselling, for depression, 317–318
informed, 13–14 Coxibs, 155t
legal issues of, 30–31 Cradle cap, 385
patient under 16 years old, termination of pregnancy in, 264 CRB-65 score, 100
Constipation Creutzfeldt-Jakob disease, 235b
child with, 42–43 Crohn disease, 115–118
in multiple sclerosis, 187 Cryotherapy
palliative care and, 448–449 for actinic keratosis, 396
urinary incontinence and, 344 for warts, 390
Consultation, 14–15 Cryptosporidiosis, incubation period and infectivity of, 472t–473t
Calgary Cambridge method of, 15 Crystalloid fluid, for anaphylaxis, 406t
CARE Approach to, 15–16 Culture-negative cystitis, postmenopausal, lower urinary tract
email, 17 infection for, 343
inner, 15 Cushing disease, in resistant hypertension, 71
structure of, 14–16 Cutaneous horn, 397
telephone, 16–17 Cystic fibrosis, 362
Contact lenses prepregnancy genetic counselling, 210
prescribing for patients with, 379 Cystitis, 341–342
problems with, 374 interstitial, 343
Contact tracing Cytomegalovirus (CMV), 124, 218
pelvic inflammatory disease, 199
sexually transmitted infections, 268 D
tuberculosis, 101 Dabigatran, for atrial fibrillation, 83
Contextual factors, in four-topic approach, 24 Dacryocystitis, acute, 375
Continence appliances, 345–346 Dantrolene, 442
Continence pads, 345–346 Data subjects, 21
Contraception, 247–275 Day-to-day activities, impairment and, 27
see also Sterilization De Quervain tenosynovitis, 151
Index 539
Deafness, otitis media acute, 355 Dermatofibroma, 396

Death see Dying patient, care of Dermatologic presentations, in travellers returning from abroad,
Debriefing, in posttraumatic stress disorder, 321 302, 302t
Decongestants Dermatomes, 497, 497t
for common cold, 362 Detention, of older people, 243
for rhinosinusitis, 361 Detergent, in eye, 374
Deep vein thrombosis see Venous thromboembolism Deterioration, in older people, 234
Defibrillation, 72 Developmental disability, 28–29
Deliberate self-harm, 326 Dexamethasone, 456t–457t, 457
Delirium Diabetes mellitus
end-stage renal failure and, 467 antenatal care, 215
in older people, 232–234, 237–238 cardiovascular risk factors in, 415
palliative care and, 452–453 lipids and, 415
Delusional halitosis, 368 smoking and, 415
Delusions, 314–315, 327 chronic renal disease and, 347, 351
Dementia, 462–463 driving and
hormone replacement therapy related risk, 203 general advice for, 416
with Lewy bodies, 235b regulations for, 416
management of behaviour, 463 endocrinology and, 409–425
in older people, 234–239, 234b–235b erectile dysfunction and, 416
in Parkinson disease, 181 foot disease and neuropathy, 415–416
reduced oral intake and infection in, management of, 463 genetic, 411
Dental abscess, 367 gynaecomastia and, 423, 423t
Dental problems, 367–368 hirsutism and, 422–423
Dental procedures, phobias and, 325 hormone replacement therapy, 203
Dental treatment, antibiotic prophylaxis, 145 hypercalcaemia and, 421
Dentures, sterilization, 367 in hypertension, treatment of, 69
Depression, 314–319 hypoadrenalism and, 420–421
bipolar affective disorder, 333–334 hypogonadism in adult men, 423–424, 424t
chronic heart failure, 76 injectable progestogens and, 255
chronic obstructive pulmonary disease, 103 latent autoimmune diabetes in adults, 411
definitions and scoring, 315t in myocardial infarction, 73
elderly people, 316–317 nephropathy, 415
end-stage renal failure, 466 oral contraception, contraindications, 248
during episodes of, 333 pancreatic, 411
Huntington disease, 187–188 polycystic ovary syndrome, 196
management of, 315 in pregnancy, 223
menopause-related, 202 prepregnancy care for, 211
multiple sclerosis, 186 retinopathy and maculopathy, 415
myocardial infarction, 73 secondary, 411
obsessive-compulsive disorder, 326 thyroid disease, 417–420
osteoarthritis, 144 type 1
palliative care and, 455–456 in pregnancy, 417
Parkinson disease, 181 typical features of, 410t
postnatal, 318–319 type 2, 410–416
care for, 226 blood pressure management in, 415
presenting complaints, 314 diagnosis of, 410–411
prevention of recurrence, 318 glycaemic control for, 412
referral, 315 glycated haemoglobin A1c, 410, 411t
specific treatment home glucose monitoring, 414–415
of major depression (or mild depression on ICD-10 criteria), management of, 412–414
316 pharmacologic therapies in preventing, 412
of minor depression (or mild depression on ICD-10 criteria), in pregnancy, 417
315–316 primary care investigations in, 411
stopping maintenance therapy and managing discontinuation referral for, 411
reactions, 317 screening for, 411–412
stroke, 182–183 treatments for, 412–414
suicidal risk, 316, 319–320 typical features of, 410t
tinnitus, 360 vaccination for patients with, 283
Dermatitis Diabetic neuropathy, 415–416
irritant and allergic, 384–385 Diamorphine, 439
seborrheic, 385–386 Dianette, for acne vulgaris, 387
540 Index
Diaphragms (barrier method), 260 Diuretics, 345

Diarrhoea for chronic heart failure with left ventricular systolic dysfunction,
child with, 44–45 77–78
chronic, 118–119 chronic obstructive pulmonary disease, acute exacerbation, 104
coeliac disease, 120 tinnitus and, 360
combined oral contraceptive (COC) pill and, 251 Diverticular disease, 135–136
Crohn disease, 115 Diverticulitis, acute mild uncomplicated, 136
inflammatory bowel disease, 115 Diverticulosis, 136
irritable bowel syndrome, 113 Dizziness, 358–359, 429b
palliative care and, 449 Dopamine agonists, for Parkinson disease, 180
in travellers returning from abroad, 301–302 Down syndrome (DS), 32–33
Diazepam, 443 genetic counselling, 211
Diclofenac/Solaraze Gel, for actinic keratosis, 396 health care for
Diet adolescent with, 32
see also Nutrition adults with, 32–33
in cardiovascular disease, 87 children with, 32
in chronic heart failure, 76 older people with, 32–33
in myocardial infarction, 73 maternal age-related risk, 210
Dietary advice, premenstrual syndrome, 199 prenatal diagnosis, 215
Dietary Approaches to Stop Hypertension (DASH) diet, in problems associated with, 517t–520t
hypertension, 68 Doxazosin, for hypertension, 70
Dietary deficiencies, in older people, 231 Driving
Digital ganglia, 151 acute psychosis, 328
Digital rectal examination, 347 chronic heart failure, 76
Digoxin, 500t dementia, 236
for heart failure, 78 diabetes
Dihydropyridine calcium channel blocker, for hypertension, 70 general advice for, 416
Dilemma, 20 regulations for, 416
Diltiazem epilepsy, 176
for hypertension, 70 obstructive sleep apnoea, 364
for stable angina, 74 opioids, 437–438
Dipeptidyl peptidase-4 inhibitors, for type 2 diabetes mellitus, Parkinson disease, 180
413 Drug induced acne, 386
Diphtheria, vaccination, 283 Drug levels, 500, 500t
Disability, 26–36 Drug misuse, in pregnancy, 224
human rights and, 27 Drug stabilities, in syringe drivers, 524–525, 524t–525t
prevalence of, 27 Drug therapy, for depression, 317
problems associated with specific causes of, 517t–520t Dry eye, 373
Disability Employment, 146 Dry mouth, 367
Disabled people Duchenne muscular dystrophy, genetic counselling, 210
chronic obstructive pulmonary disease, 103 Duloxetine, 442, 456
eligibility for benefits, 145 Duodenal ulcer, 111
financial benefits, 179 Duodenitis, 111
osteoarthritis, 145 Dying patient, care of, 431–470
Parkinson disease, 179 advanced neurologic disease and, 462–464
rheumatoid arthritis, 161 anticipatory medications in, 459, 460t
Disablement Resettlement Officer, 331 basic principles of, 432–433
Discriminatory abuse, in older people, 242 end-stage cardiac failure and, 468–470
Disease-modifying antirheumatic drugs (DMARDs), 156, 158–159, end-stage renal failure and, 464–467
160t end-stage respiratory disease and, 467–468
prescribing, guidance for, 495–496, 495b last days of life, 458–459
blood monitoring schedule in, 495 noninvasive ventilation in, 459
generic recommendations before, 495 nutrition and hydration in, 459
during intercurrent infections, 496 prognostication and, 458
management, perioperative, 496 starting doses in, 461–462, 461t
monitoring of, 495–496, 496t stopping treatments in, 458–459
in patients with comorbidities, 495 symptom management guidelines, 436–458
recommendations for, 495 syringe driver for, 459–462
for shared care agreements, 496 Dysarthria, in multiple sclerosis, 186
Disseminated HSV, 391–392 Dysmenorrhoea, 192
Distress, agitation in older people and, 233 intrauterine devices and, 258
Dithranol, for chronic plaque psoriasis, 389 Dyspareunia, 267
Index 541
Dyspepsia, 110–113 End-stage renal failure, 464–467

nonsteroidal antiinflammatory drug-related, 155–156 indicators of poor prognosis for, 464
Dysphagia, 110, 113, 366 medications for, 465
in multiple sclerosis, 186 management of, 465, 465t
palliative care and, 450 pharmacologic considerations in, 464
Dysphonia, functional, 366 symptom management for, 465–467, 466t
Dysplastic naevus, 397 symptoms of, 464
Dysthymia, 314 End-stage respiratory disease, 467–468
Dysuria and abdominal pain, child with, 50–51 anticipating care needs and management of, 468
indicators of poor prognosis of, 467
E oxygen in, 468
Ear, 355–361 symptom management in, 467–468
Ear discharge, 356–357 Enemas, 115
Ear pain, 355–356 Enteric fever, in travellers returning from abroad, 300
Early warning form, for use in psychotic illness, 508, 508t Enteroviral infection, incubation period and infectivity of,
Eating disorders, 334–336 472t–473t
Eclampsia, 221 Eosinophilia, in travellers returning from abroad, 302–303
Ecthyma, 392 Epilepsy, 175–179
Ecthyma contagiosum, 392 contraception and, 263
Ecthyma gangrenosum, 392 prepregnancy care for, 212
Ectopic beats, in normal heart, 80 Episcleritis, 374
Ectopic pregnancy, 216–217, 264 Eplerenone, in cardiovascular disease, 88
intrauterine devices and, 258 Epley manoeuvre, 360
Eczema herpeticum, 391 Epstein-Barr virus, in travellers returning from abroad, 301
Edinburgh postnatal depression scale, 504–505, Erectile dysfunction, 265–267
504t–505t diabetes and, 416
Education Erratic bleeding, injectable progestogens and, 256
osteoarthritis, 144 Erysipelas, 393
rheumatoid arthritis, 160 Erythema chronicum migrans, 394
Edward syndrome, prenatal diagnosis, 215 Erythrasma, 393
Eggs, reintroducing of, 404 Erythroderma, in atopic eczema, 382
Electronic medical records, primary care and, 4 Erythrodermic psoriasis, 389
Email consultation, 17 Erythroplakia, 366
confidentiality and, 17 Erythroplasia, 397
Emergencies, in palliative care, 433–436 Escherichia coli enteritis, incubation period and infectivity of,
Emergency contraception, 261–262 472t–473t
Emollients Essential tremor, 189
for atopic eczema, 383 Estimated glomerular filtration rate (eGFR), 351
for chronic plaque psoriasis, 388 Ethical analysis, 20t
Emotionalism, in multiple sclerosis, 186 Ethical norms, 20–21, 20t
Empathy, 10, 12 Ethical reasoning, 21–24
Employment, epilepsy and, 176 benefits, burdens, and risks for, 22b, 22t
End-of-life care, 432 four principles of, 22–24, 22b, 23t
Endometrial ablation, 194 four topics in, 24, 24b
Endometrial cancer, hormone replacement therapy, 202–203 Ethical theory, 20t
risks, 203 Ethics, 19–25
Endometrial polyp, 194 different meanings of, 19–20, 20t
Endometriosis, 192–193 general practice, 19
hormone replacement therapy use, 203 law and, 20–21, 20f
Endometritis, 226 professional, in general practice, 24, 24t–25t
Endomysial antibody test, 118 professionalism and, 20–21, 20f
Endoscopy Ethnic groups, high-risk, prepregnancy genetic counselling, 210–211
dyspepsia, 110 Ethosuximide, 500t
gastrooesophageal reflux disease, 112–113 Etonogestrel-releasing implant, Implanon, 256–258
End-stage cardiac failure, 78–79, 468–470 Evidence-based general practice, 9
cardiac devices in, management of, 469–470 Evidence-based rehabilitation, 28
indicators of poor prognosis of, 468 Excessive daytime somnolence, in Parkinson disease, 181
medications for, 469 Exercise
management of, 469, 469t ankle rehabilitation, 152–153
pharmacologic considerations in, 469 cardiovascular disease, 86
symptom management in, 469 chronic heart failure, 76
symptoms of, 468 chronic obstructive pulmonary disease, 102
542 Index
Exercise (Continued) FEV1/FVC charts, 485–486, 485t–486t

fibromyalgia, 167 Fever
hypertension, 68 child with, 38–40
knee, 152 with jaundice, 124
low back pain, 146, 148 postnatal care for, 226
myocardial infarction, 73 in travellers returning from abroad, 299–300
neck pain, 148 Fever-arthralgia-rash (FAR) arboviruses, in travellers returning from
older people, 230 abroad, 301
osteoarthritis, 144 Fibre intake, irritable bowel syndrome, 114
osteoporosis, 164 Fibroids, 194
plantar stretching for plantar fasciitis, 153 hormone replacement therapy use, 203
polycystic ovary syndrome, 196–197 Fibromyalgia, 167
pregnancy, 213 Fifth disease, 219
premenstrual syndrome, 200 Filiform wart, 390
rheumatoid arthritis, 160 Financial abuse, in older people, 242
spondyloarthropathies, 161 Financial aspects, in older people, 239
Exertional headache, 175 Finger clubbing, child with abdominal pain and, 43
Exhaustion, 226 Fitness to work see Employment
Exposure therapy Flashes, 376–377
fear of flying, 325–326 Flexural psoriasis, 389
social anxiety/social phobia, 325 Floaters, 376–377
Eye abnormalities, in atopic eczema, 382 Fluid intake, in chronic heart failure, 76
Eye drops, instilling, 372 Fluorouracil/Efudex, for actinic keratosis, 396
Eye lash problems, 375 Focal seizures, 177
Eye lid Folic acid supplements, of prepregnancy care, 210, 213
hygiene, stages of, 373 epilepsy, 212
problems, 375 Folliculitis, 392
Eye problems, 371–380 Food allergies, 403–405
diagnosis of, 403–404, 404t
F management of, 404–405
Face, seborrheic dermatitis in, 386 foods after a period of avoidance, reintroducing, 404–405
Face Arm Speech Time (FAST), 182 further, 404
Facial palsy, 361 prevention for, 405
Facial psoriasis, 389 symptomatic treatment for, 404
Facial twitching, 375 useful contacts in, 405
Factitious disorder, 427 for patients, 405
Factor V Leiden, 248 for professionals, 405
Faecal incontinence, postnatal, 226 Foot
Falls, in older people, 239–240, 239b diabetic, 415–416
Familial hypercholesterolaemia (FH), 88–89 problems, 153
Family Law Reform Act (1969), 264 tinea, 394
Family planning clinic, 247 Footwear
Fan, for breathlessness, 445 osteoarthritis, 144
Fasting sugar, 88 Foreign body, in eye, 373
Fatigue Fragile X syndrome, prepregnancy genetic counselling, 210
in end-stage heart failure, 79 Fragüe syndrome, problems associated with, 517t–520t
in multiple sclerosis, 186 Frailty, 231
palliative care and, 452 in older people, 231
postnatal care for, 223 Frontotemporal dementia, 235b
Febrile seizure, child with, 48–49 Frovatriptan, for migraine, 173
Febuxostat, 157 Functional deterioration, of older people, 232
Female condoms, 260 Functional dyspepsia, 112, 429b
Females Fundal height, 220
breast development in, 481f, 482 Fungal infections
FEV1/FVC chart, 485t in skin, 394–395
pubic hair development, 481f, 482 superficial, in atopic eczema, 382
Femidom, 260 Furuncles, 393
Femoral nerve, testing, 498t–499t Furunculosis, acute, of external ear, 355
Fentanyl, 439–440
Fertility, inflammatory bowel disease, 117–118 G
Fertility awareness, 260–261 Gabapentin, 442
Fetoscopy, 215 Gait assessment, simple, 239b
Index 543
Gallbladder disease Glycosuria

hormone replacement therapy, 203 children with, 411
oral contraception, contraindications, 248 in pregnancy, 221–222
Gallbladder polyps, 135 Gonorrhoea, 269
Gallstones (cholelithiasis), 134–135 pelvic inflammatory disease, 198
oral contraception, risk of, 250 Good health care, cycle of, 34, 34f
Ganglion, 151 Gout, 156–158
Gardnerella vaginalis, 198 with hypertension, treatment of, 69
Gastric cancer, 110 Gram-negative bacilli, 393
Gastric ulcer, 111 Graves disease, 419
Gastritis, 111 Grief, suicide, 320
palliative care and, 449 Groin swelling, child with, 55–56
Gastroenteritis, incubation period and infectivity of Grommets, 358
adenoviral, 472t–473t Group A streptococcal infection, 284
Norwalk virus, 472t–473t Guttate psoriasis, 366, 389
rotaviral, 472t–473t Gynaecologic cancer, 526t–530t
unidentified, 472t–473t Gynaecomastia, 423, 423t
Gastroenterologic problems, 109–133
Gastrointestinal bleeding, 110–111 H
Gastrooesophageal reflux (GOR), 112–113 H1-antihistamines, for food allergies, 404
Helicobacter pylori and, 111–112 H2-receptor antagonists
infant with, 40 dyspepsia, 111
Gels, instilling, in to eye, 372 vomiting in pregnancy, 216
General practice ethics, 19 Haematologic cancers, 526t–530t
General practitioner (GP) consultant, primary care and, 5 Haematology, reference ranges for, in young adults, 515t
Generalised hyperhidrosis, 400–401 Haematuria, asymptomatic, 350–351
Generalized anxiety, 323–325 Haemochromatosis, 127
Genetic counselling, prepregnancy care, 210 Haemodialysis, vaccination for patients on, 283
Genetic diabetes, 411 Haemophilia, vaccination for patients with, 283
Genetic disorders, 210 Haemophilus influenzae, incubation period and infectivity of,
Genetic testing, in Huntington disease, 188 472t–473t
Genital gonorrhoea, 269, 305 Haemophilus influenzae type B, 284
Genital herpes, 271, 391 Haemoptysis, 446
in pregnancy, 212, 219 Haemorrhage (massive), 435
Genital psoriasis, 389 Haemorrhoids, 137–138
Genital warts, in pregnancy, 212, 219 postnatal, 226
Gestational diabetes, 221–222, 416–417 Hair removal, for hirsutism, 423
diagnostic criteria for, 417t Halitosis, 368
issues postpregnancy, 417 delusional, 368
risk factors for, 416t Hallpike test, 359–360
screening and diagnosis for, 416–417 Hallucinations, 314–315
treatment of, 417 Hallux rigidus, 153
Gestational trophoblastic hydatidiform mole, 200–201 Hand, foot, and mouth disease, incubation period and infectivity
Giant cell arteritis, 166, 174 of, 472t–473t
Giardiasis, incubation period and infectivity of, Hands
472t–473t dermatitis, 384–385
Gilbert syndrome, 124 tinea, 394
Ginkgo biloba, in Alzheimer disease, 237 Has-Bled bleeding risk score, 82t
Girls, FEV1/FVC chart, 486t Head
Glandular fever, 365 large, child with, 47–48
Glaucoma, 316 small, child with, 47
chronic simple, 378–379 Head and neck cancer, 526t–530t
Glenohumeral joint Head lice, 395
arthritis, arthrosis, 149–150 incubation period and infectivity of, 472t–473t
instability, 150 Headache, 171
Glibenclamide, 417 analgesic-overuse, 175
Gliclazide, 413 child with, 49–50
Globus hystericus, 366 cluster (migrainous neuralgia), 174
Glomerulonephritis, 352, 364 diagnosis of, 171
Glucagon-like peptide 1 agonists, for type 2 diabetes mellitus, 414 migraine (see Migraine)
Glycaemic control, for type 2 diabetes mellitus, 412 nonmigrainous, 174–175
Glycated haemoglobin A1c, 410, 411t oestrogen withdrawal, 253
544 Index
Headache (Continued) serology, 285

tension-type, 171, 173–174 vaccination, 285–286
Health care Hepatitis C, 125, 286
comprehensive, 31 incubation period and infectivity of, 472t–473t
ethics, 20t Hepatitis E, 126, 284–285
providing, 29–30 Hepatocellular carcinoma, 129
utilisation, 8 Herpes genitalis, 271, 306–307
Health disability, 29 Herpes labialis, 391
Health promotion, child, 476–477, 476t–477t Herpes simplex
Hearing aids, 360 atopic eczema, 382
Hearing loss, 357–358 incubation period and infectivity of, 472t–473t
Hearing tests, 358 labial, 219
Heart disease, vaccination for patients with, 283 skin, 391
Heart failure, 75–80 Herpes zoster (shingles), 292–294
acute pulmonary oedema and, 79 ear involvement (Ramsay Hunt syndrome), 356
admission in, grounds for, 78 immunization, in older people, 230
arrhythmias and, 79–80 ophthalmic, 375
chronic treatment of, 293–294
with left ventricular systolic dysfunction, Herpetic gingivostomatitis, 391
77–78 Hiccup, palliative care and, 449
management of, 76–77 Hidradenitis suppurativa, 401
definition of, 75 Hip replacement, 145
diagnosis of, 75–76 Hirsutism, 60, 196, 422–423, 422t
BNP in, 76 Histiocytoma, 396
serum natriuretic peptides in, 76, 76t HIV/AIDS, 271–275
end-stage, 78–79, 468–470 antibody test, 272
cardiac devices in, management of, 469–470 infection, 307–311, 308t
indicators of poor prognosis of, 468 HIV testing, 307–308
medications for, 469, 469t management of patients living with, 309
pharmacologic considerations in, 469 patients with low CD4+ counts, 310
symptom management in, 469 possible exposure to bloodborne viruses, 310–311
symptoms of, 468 practicalities of testing, 308–309
with hypertension, treatment of, 69 primary, 309
incidence of, 75 routine care, 309–310
palpitations and, 79–80 notification and surveillance, 275
with preserved ejection fraction, 78 positive, managing, 273
prevalence of, 75 postexposure prophylaxis for, 274
prognosis of, 75 prevention and early diagnosis for, 271
right, 79 routine care for, 273
second line pharmacologic management of, 78 safer sex for, 271–272
specialist review of, referral for, 78 symptoms needing special consideration, 273
Heartburn, in pregnancy, 216 testing, 272–273
Heat therapy antenatal screening, 212
low back pain, 147 in pregnancy, 219
rheumatoid arthritis, 160 vaccination for people with, 282
Heavier periods, intrauterine devices and, 258 venepuncture for, 274
Heel pain, 153 HLA-B27, 161
Helicobacter pylori infection, 111–112 Hoarseness, 366
Hepatic encephalopathy, 128 Holism, 10
Hepatitis Home, older people at, 231–232
alcoholic, 127 Home blood pressure monitoring (HBPM), 67b
autoimmune, 126 Home glucose monitoring, for type 2 diabetes mellitus,
viral, 125, 284–286 414–415
oral contraception, contraindications, 248 Hormonal contraception, 247–258
Hepatitis A, 125–126, 284–285 acne vulgaris, 387
incubation period and infectivity of, 472t–473t Hormone replacement therapy, 202–205, 262–263
in pregnancy, 219 connective tissue diseases, 162
vaccination, 285 contraception and, 205
Hepatitis B, 125, 285–286 migraine, 173
incubation period and infectivity of, 472t–473t osteoporosis, 164
practicalities, 286 Hot flash, 201
in pregnancy, 212, 219 House dust mite, 95
Index 545
Human Fertilisation and Embryology (1990), 263 I

Human immunodeficiency virus (HIV) see HIV/AIDS Ice therapy
Human papillomavirus (HPV), 206, 287 joint, limb injury, 152
Human rights, disability and, 27 low back pain, 147
Huntington chorea, genetic counselling, 210 rheumatoid arthritis, 160
Huntington disease, 187–188 Idiopathic thrombocytopaenic purpura, child with nonblanching
Hutchinson sign, in ophthalmic herpes zoster, 375 rash and, 56
Hydatidiform mole, 200–201 Idiopathic urticaria, 401
Hydramnios, 220 IgA nephropathy, 351
Hydration Imiquimod/Aldara, for actinic keratosis, 396
advanced neurologic disease and, 462 Immigrants
for dying patient, 459 BCG immunization, 101
Hydrocele, groin swelling and, 55 Immunizations
Hydrocephalus, prenatal diagnosis of, 215 inflammatory bowel disease, 118
Hydrocortisone, for anaphylaxis, 406t influenza, 102
Hydroxychloroquine, 495 pneumococcal infection, 101–102
Hypercalcaemia, 349, 421 in pregnancy, 503, 503t
of malignancy, 434 routine schedule of, 404t
Hypercalciuria, 349 for travel, 474, 474t
Hyperemesis gravidarum, 216 tuberculosis (BCG), 101
Hyperhidrosis, 400–401 Immunoglobulins, reference ranges for, in young adults, 514t–515t
generalised, 400–401 Immunologic urticaria, 401
primary, 400 Immunosuppressed patients
Hyperkalaemia, in aldosterone antagonists, 78 contacts of, 282
Hyperprolactinaemia, 196 vaccination for, 282
Hypertension, 66–71 Imperforate hymen, 195
antenatal care, 214 Impetigo, 392
autonomic dysreflexia and, 188 incubation period and infectivity of, 472t–473t
blood pressure targets in, 67 Incident pain, 436
BP measurement, practicalities of, 67 Incomplete vaccination history, 282
chronic renal disease and, 351 Incubation and infectivity periods, of common diseases, 278–312,
definition of, 66, 66b 472–473, 472t–473t
detection of, 66–67 Indapamide, for hypertension, 69–70
drug treatment for, 68–69 Infantile acne, 386
antihypertensives in, 68–69, 69t Infants
patients with other medical problems, 69 acute otitis media, 355
poor control in, 70 asthma diagnosis, 94–95
primary prevention in, 68 atopic eczema in, 382
nondrug treatment for, 68 seborrheic dermatitis in, 385–386
oral contraception viral-associated wheeze, 94–95, 95t
contraindications, 248 weight faltering
risk of, 250 after first few weeks, 62–63
in pregnancy, 220–222 in first few weeks, 62
prepregnancy care for, 211 who vomits or screams when feeding, 61–62
referral for, 71 Infectious diseases
resistant, 70–71 intercurrent, DMARD and, 496
workup in, 67–68 notification of, 279, 280t, 475, 475t
Hypertensive retinopathy, oral contraception, contraindications, Infectious mononucleosis, 365
248 incubation period and infectivity of, 472t–473t
Hyperthyroidism, 418–419 Infective endocarditis (IE), prophylaxis of, 85
in resistant hypertension, 71 Infectivity periods, incubation and, of common disease, 278–312,
Hypoadrenalism, 420–421 472–473, 472t–473t
Hypoglycaemia, 414 Infertility, 264–265
Hypogonadism, 164 pelvic inflammatory disease, 198
in adult men, 423–424, 424t polycystic ovary syndrome, 197
Hypomania, 333 Inflammatory bowel disease (IBD), 114–118
Hypothyroidism, 418 see also Crohn disease; Ulcerative colitis
in resistant hypertension, 71 arthritis and, 161
Hypoxia, chronic obstructive pulmonary disease, 101 oral contraception, risk of, 251
Hysterectomy Influenza, 287–288
cervical screening following, 206 incubation period and infectivity of, 472t–473t
menorrhagia, 194 in travellers returning from abroad, 301
546 Index
Influenza (Continued) Isolation, of older people, 232

vaccination, 102 Issue, 20
in chronic heart failure, 76 Ivabradine, for heart failure, 78
in older people, 230
Informant Questionnaire on Cognitive Decline in the Elderly J
(IQCODE), 235–236 Japanese encephalitis, 297
Information, sharing of, 13, 13t Jaundice, 123–127
Informed consent, 13–14 neonatal, 226–227
Inguinal hernia, 139 Joint replacement, 144–145
groin swelling and, 55 Justice, in four principles, 22–23, 23t
Inhalers
asthma, 94, 96 K
chronic obstructive pulmonary disease, 103 Keratinised warts, 270, 306
spacers, 96, 96t Keratoacanthoma, 396, 398
Injectable progestogens, 255–256, 257t Ketamine, 442
Insomnia, 321–322 Kidneys see entries under Renal
rules to reduce, 322 Knee
Insulin, for type 2 diabetes mellitus, 414 acute injury, 151
sickday rules for patients on, 414–415 anterior pain, 151–152
Intellectual disability, 28–31 exercise, 152
advocacy, 30 steroid injections, 152
comprehensive health care, 31
health and learning disability, 29, 31b L
importance of language and, 29 Labial herpes, in pregnancy, 219
legal issues of consent and capacity and, 30–31 Labyrinthine sedatives, 359
mental health issues, 31 Lacrimal problems, 375
principles of practice and, 30 Lactation
providing health care and, 29–30 see also Breastfeeding
reasonable adjustments and, 30 migraine in, 173–174
Intermenstrual bleeding (IMB), 195 suppression of, 225
intrauterine devices and, 258 Language, importance of, 29
International Prostate Symptom Score (IPSS), 511–512, 511t–512t Large head, child with, 47–48
Interpreters, working with, 14 Laryngeal carcinoma, 366
Intra-articular injections, 145 Laryngoscopy, 366
Intracerebral malignancy, palliative care and, 451–452 Laser, for chronic simple glaucoma, 378–379
Intracranial pressure, raised, 175 Late-life depression, 232
Intrauterine devices (IUD), 192, 194, 199, 258–260 Latent autoimmune diabetes in adults (LADA), 411
adverse effects of, 258–259 Lateral epicondylitis (tennis elbow), 150–151
emergency contraception, 261–262 Law, 20–21, 20f
follow-up for, 259 Laxatives, 449t
insertion of, 259 child with constipation, 43
intrauterine system for, 259–260 constipation management, 114
removal or refit, 259 irritable bowel syndrome, 114
risks of, 258 palliative care, 448
Intrauterine growth retardation, maternal asthma, 211 Learning disability, 28–29
Intrauterine system, 204 contraception and, 263
Intravaginal weighted cones, 345 Left atrial appendage closure, for atrial fibrillation, 83
Inverse care law, 9–10 Left ventricular systolic dysfunction, chronic heart failure with,
Iridoplasty, for chronic simple glaucoma, 379 77–78
Iridotomy, for chronic simple glaucoma, 379 Leg ulcers, 399–400
Iron deficiency anaemia, 121–122, 122t Legal aspects, in older people, 239, 243
pica and, 57 Legal norms, 20t, 21
prepregnancy care for, 213 Lentigo maligna melanoma (LMM), 398
Irregular periods, 194–195 Leukaemia, 526t–530t
Irreversible airway obstruction, 435–436 Leukoplakia, 366
Irritability, in older people, 238 Levodopa, for Parkinson disease, 180–181
Irritable bowel syndrome, 113–114 Levonorgestrel-releasing intrauterine system (LNG-IUS)
Irritant contact dermatitis, 384–385 endometriosis treatment, 193
Ischaemic heart disease, etonogestrel-releasing implant (Implanon), menorrhagia treatment, 194
257 Lhermitte sign, 149
Ischaemic stroke, oral contraception, risk of, 250 Lichen planus, 366, 389–390
Ischaemic vascular dementia, 237 Lidocaine plasters, 442
Index 547
Lifestyle Maculopathy, diabetic, 415

cardiovascular disease and, 86–87 Major depressive illness, 314
epilepsy and, 177 Malabsorption, 119
Light therapy, 315 Malaria, 295–297
Limp, painful, child with, 60–61 in travellers returning from abroad, 300, 300t
Linaclotide, constipation management, 114 Male
Lipid-lowering measures, in cardiovascular disease, 87 FEV1/FVC chart, 486t
Lipids genital development in, 481f, 482
diabetes mellitus and, 415 pubic hair development in, 481f, 482
in myocardial infarction, 73 Male condoms, 260
Listeria Malignant bone pain, 442–443
in pregnancy, 218 Malignant bowel obstruction, palliative care and, 450–451
prepregnancy advice, 212 Malignant melanoma (MM), 398
Lithium, 500t Malignant skin lesions, 397–399
bipolar affective disorder, 333–334 Malignant wound management
Liver disease exudate and enterocutaneous fistulae in, 455
chronic, 127–129, 128t odour and infection in, 455
hormone replacement therapy, 203 palliative care and, 455
vaccination for patients with, 283 Malingering, 427
Liver function test, abnormalities, 123–127 Mammography, 203
Liver transplant, 129 Management strategy, osteoarthritis, 143–144
Liver tumours Mania, 333
etonogestrel-releasing implant (Implanon), Manual lymphatic drainage (MLD), 454
257 Massage, low back pain, 147
injectable progestogens and, 255 Mastitis, 225–226
oral contraception, contraindications, 248 Mastoiditis, 355
Living wills see Advance directives Material abuse, in older people, 242
Long-term conditions, 7–10 Measles, incubation period and infectivity of, 472t–473t
clinical guidelines for, 8–9 Measles, mumps, and rubella (MMR), 288–289
comorbidity and multimorbidity, 7–8 Median nerve, testing, 498t–499t
deprivation effects and, 8 Medical assistants, primary care and, 4
empathic, person-centred care for, 10 Medical ethics, 20t
evidence-based general practice and, 9 Medical indications, in four-topic approach, 24
generalism and, 10 Medical management, of obesity, 502, 502t
global burden of, 8 Medical procedures phobia, 325
health care utilisation and, 8 Medical records, patient access to, 21b
inverse care law and, 9–10 Medically unexplained symptoms, 426–430, 427t
mental and physical, 8 clinical assessment of, 427–428
organisational, 9 examination of, 428
patients with, general practice and, 10 explanations for, 429, 429b
polypharmacy and, 8 history of, 427–428
practice organisation and, 10 investigation of, 428
prevalence of, 7 management of, 428–429
Low back pain, 145–148 overassessing and, 428
Lower gastrointestinal cancer, 526t–530t prevalence of, 427
Lower limb problems, 151–153 principles of management of, 427–429
in older people, 240–241 referral of, 428
Lower respiratory tract infection, 99–101 specific syndromes and, 429
Lung cancer, 526t–530t Medication-overuse headache, 174–175
Lung disease, vaccination for patients with, 283 Medicines, normative domains in, 20t
Lyme disease, 288, 393–394 Megalencephaly, 47
Lymphadenopathy, cervical, child with, 57 Meglitinides, for type 2 diabetes mellitus, 414
Lymphangitis carcinomatosis, 446–447 Melanoma, 396
Lymphoedema, 453–454 Memory difficulties, in older people, 238
cellulitis associated with, 453–454 Ménière’s disease, 359
Lymphogranuloma venereum (LGV), 305 Meningococcal disease, incubation period and infectivity of,
Lymphoma, 121, 526t–530t 472t–473t
Meningococcal infection, 289–290
M incubation period and infectivity of, 472t–473t
Macrocephaly, 47–48 Menopause, 201–202
Macroscopic haematuria, 350 Menorrhagia, 193–194
Macule, 395 Menstrual diary, 199
548 Index
Menstrual migraine, 173 Multimorbidity, 7–8

Mental Health (Care and Treatment) (Scotland) Act (2003), 330t effects of, 8
Mental Health Act (England and Wales) (1983), compulsory prevalence of, 7–8
admission, 328 Multiple pregnancy, 220
Mental health issues, 31 Multiple sclerosis, 185–187
Mental Health (Northern Ireland) Order 1986, 330t patient with, 501, 501b
Mental health problems, admission procedures for patients with, Mumps, incubation period and infectivity of, 472t–473t
506–507, 506t–507t Murmur, child with, 45
Mental health professionals, primary care and, 3–4 Muscle jerks, 467
Metastatic spinal cord and cauda compression (MSCC), 433 Musculoskeletal problems, 142–169
Metatarsalgia, 153 Mycoplasma genitalium, 198
Metformin, for diabetes Mycoplasma hominis, 198, 343
gestational, 417 Mycoplasma pneumonia, 100
type 2, 412–413 Myeloma, 350
Methadone, 441–442 Myocardial infarction (MI), 72
Methotrexate, 116, 159, 495 oral contraception, risk of, 250
Methylphenidate, 456 Myoclonic seizures, 177
Methysergide, for migraine prophylaxis, 173 Myokymia, 375
Metoprolol, for stable angina, 74 Myopia, surgery for, 379–380
Microalbuminuria, diabetic nephropathy and, 415 Myotomes, 497, 497t
Microbial keratitis, 374 Myringitis, 355
Microcephaly, 47 bullous, 355–356
Microscopic colitis (lymphocytic or collagenous colitis), 118–119
Microscopic haematuria, 350–351 N
Migraine, 171–174 Nails
hormone replacement therapy, 203 psoriasis, 389
with hypertension, treatment of, 69 tinea, 394
oral contraception, contraindications, 248 Nasal polyps, 362
Migrainous neuralgia, 174 National Institute for Health and Care Excellence (NICE),
Mild dyskaryosis, cervical, 206, 206t guidelines from, feverish illness in children, 39, 39t
Mild flares, steroids for, 383 Nausea
Mild inflammatory acne, 386 end-stage heart failure, 79
Milk, reintroducing of, 404 end-stage renal failure, 467
Mini-Mental State Examination (MMSE), 234 palliative care, 447–448
Minor depression, 314 Neck pain, 148–149
Mirtazapine, 456 Necrotizing fasciitis, 393
Miscarriage, 216 Neglect
previous, 211 in child abuse, 64
recurrent, 211 in older people, 242
suspected, in 14 weeks of pregnancy, 216–217 Neonatal examination, 476t–477t
Mixed dementia, 237 Neonatal HSV, 391
Mobility, loss of, in older people, 232–234 Neonates
Mobiluncus species, 198 acute infective conjunctivitis in, 372
Moderate dyskaryosis, cervical, 206, 206t jaundice, 226–227
Moderate flares, steroids for, 383–384 Nerve root compression
Moderate inflammatory acne, 386 low back pain, 147
Monoamine oxidase B (MAO-B) inhibitors, for Parkinson disease, neck pain, 148, 150
180–181 shoulder pain, 150
Moodiness, in older people, 238 Neural tube defects
Morphine, 439 with family history, 211
for breathlessness, 445t folic acid supplements and, 210, 213
Morphoeic basal cell carcinoma, 397, 398t prenatal diagnosis of, 215
Morton neuroma, 153 Neuroblastoma, 526t–530t
Mosaic wart, 390 Neurofibromatosis, problems associated with, 517t–520t
Motor neurone disease, 184–185, 463–464 Neurogenic bladder, 346
ventilatory support for, 463 Neurologic disease, advanced, 462–464
Mouth, 364–368 activities of daily living in, 462
ulcers, palliative care and, 451 communication and, 462
MRC dyspnoea scale, 102, 102t muscle spasms/spasticity, 462
Mucolytic therapy, chronic obstructive pulmonary disease, nutrition, hydration, and poor swallow and, 462
103 recurrent infection in, 462
Mucosal chronic otitis media, 356 Neurologic problems, 170–190
Index 549
Neuropathic pain, 441–442 N-terminal-pro-BNP (NT-pro-BNP), for heart failure, 76

New York Heart Association Classification, of heart failure Nuchal translucency scan, 215
symptoms, 76t Nurse practitioners, primary care and, 2–3
Nifedipine, for hypertension, 70 Nutrition
Night cramps, in older people, 240–241 advanced neurologic disease, 462
Nipple, sore, 225 chronic obstructive pulmonary disease, 102
Nociceptive pain, 438–441 dying patient, 459
Nocturia, 347 gout, 157
Nocturnal enuresis, 51–52 irritable bowel syndrome, 114
Nodular basal cell carcinoma, 397, 398t motor neurone disease, 184
Nodular melanoma (NM), 398–399 older people, 230–231
Nodule, 395 osteoporosis, 164
Nonaccidental injury see Child abuse prepregnancy advice, 213
Nonalcoholic fatty liver disease, 126–127 rheumatoid arthritis, 161
Nonblanching rash, child with, 56–57
Noncardiac chest pain, stable angina and, 75 O
Nonclinical staff, in contact with patients, vaccination for, 283 Obesity, medical management, 502, 502t
Noncoeliac gluten sensitivity, 121 Obsessive-compulsive disorder, 326
Nonimmunologic urticaria, 401 Obstetric problems, 209–228
Nonkeratinized warts, 270 Obstructive sleep apnoea (OSA), 363–364
Nonmaleficence, in four principles, 23t, 24 in resistant hypertension, 71
Nonspecific urethritis (NSU), 270, 305–306 symptoms and signs in, 363t
Nonsteroidal antiinflammatory drugs (NSAIDs), 118–119, 155t Obstructive uropathy, 347
acute pyelonephritis, 342 Occupational therapy
adverse reactions, 154 for motor neurone disease, 184
chronic heart failure, 76 for Parkinson disease, 179
classification, 156 Oedema, 453–454
contraindications and cautions, 95, 154–155 in end-stage heart failure, 79
COX-2 selective inhibitors, 112, 156 Oesophageal adenocarcinoma, Helicobacter pylori and, 112
dysmenorrhoea, 192 Oesophageal stricture, 113
dyspepsia, 155–156 Oesophagitis, 113, 366
endometriosis, 193 palliative care and, 449
gastrointestinal toxicity, 154 Oesophagogastroscopy, 123
gout, 156–158 Oestrogen therapy
headache, 175 see also Hormone replacement therapy
knee pain, 152 bone conservation, 202t
low back pain, 146 higher doses, 249
mastitis, 225 migraine, 173
menorrhagia, 194 postmenopausal
migraine, 171 lower urinary tract infection, 343
menstrual, 173 urinary incontinence, 345
nociceptive pain, 438 premenstrual syndrome, 200
osteoarthritis, 145 Oestrogen withdrawal headache, 253
peptic ulcer, 112 Ointments
premenstrual syndrome, 199 in atopic eczema, 383
prescribing practice, 155 for dry eye, 373
rheumatoid arthritis, 159 instilling, in to eye, 372
spondyloarthropathies, 161–162 Older people
tennis elbow (lateral epicondylitis), 151 abuse in, 241–242
topical, 144 acute confusion in, 232–234
urinary stone, 349 acute infective conjunctivitis in, 372
Nonverbal communication, 12 agitation in, 232–234
Noonan syndrome, problems associated with, 517t–520t alcohol in, 230
Normative domains, in medicine, 20t cataracts in, 377
Nortriptyline, 442 delirium in, 232–234
Norwalk virus, gastroenteritis, incubation period and infectivity of, dementia in, 234–239, 234b–235b
472t–473t depression in, 316–317
Nose, 361–364 with Down syndrome, 32–33
Nottingham Extended Activities of Daily Living Questionnaire, exercise in, 230
523, 523t falls in, 239–240, 239b
Nottingham Extended Activities of Daily Living scale, 231 frailty in, 231
Novel oral anticoagulant agents (NOACs), 82 health of, 229–245
550 Index
Older people (Continued) Otitis externa, 356

at home, 231–232 malignant, 356
legal aspects in, 243 refractory, 356
loss of mobility in, 232–234 Otitis media
low back pain in, 147–148 acute, 355, 364
lower leg problems in, 240–241 chronic, 356–357
nutrition of, 230–231 with effusion, 357–358
screening and prevention in, 230–231 grommets, 358
sex and, 242–243 Ottawa Ankle Rules, 152, 152t
smoking in, 230 Ottawa Knee Rules, 151
suicide in, 232 Ovarian cancer
urinary incontinence in, 344–345 hormone replacement therapy, 203
Oligohydramnios, 220 risk of, 200t
Omission, in older people, 242 screening, 200, 200t
Ondansetron, 448 Overflow incontinence, 345
Ophthalmic herpes zoster, 375 Overlap syndromes, 126
Opiates, pregnancy and, 224 Oxycodone, 439
Opioid-induced hyperalgesia, 438 Oxygen therapy
Opioids breathlessness, 446
addiction, 437 chronic obstructive pulmonary disease, 103
for breathlessness, 445, 445t end-stage respiratory disease, 468
dependence, 437
dose conversion chart, 531, 531t P
driving and, 437–438 Pain
in end-stage renal failure, 466 assessment of, 436
legal implications of, 437–438 breakthrough, 436
nociceptive pain, 438–441 in end-stage renal failure, 466
patient information, 437 general considerations for, 436–438
side effects of, 437 incident, 436
tolerance, 437 interventional techniques for, 443
toxicity, 435 malignant bone, 442–443
travelling abroad and, 438 management of, 436–437
for urinary stone, 349 legal implications-drugs and driving, travel abroad,
Optic neuritis, 376 437–438
Optometrist, for cataracts, 377 opioid side effects in, 437
Oral acetazolamide, in chronic simple glaucoma, 378 patient information and, 437
Oral allergy syndrome, 404 tolerance, dependence, addiction in patients taking opioids,
Oral antibiotics 437
for acne rosacea, 387–388 use of medications off label for, 437
for acne vulgaris, 387 in multiple sclerosis, 187
Oral candidiasis, 394–395 neck pain, 148
Oral contraception, 247–253 neuropathic, 441–442
see also Progestogen-only pill nociceptive, 438–441
contraindications, 248 nonpharmacologic interventions, 443
dysmenorrhoea, 192 osteoarthritis, 145
endometriosis, 193 skeletal muscle spasm and, 442–443
hormone replacement therapy users, 203 smooth muscle spasm and, 442
intermenstrual bleeding, 195 tenesmus and, 443
irregular periods, 194 total, 443
premenstrual syndrome, 200 Palliative care, 431–470
Oral glucose tolerance test (OGTT), 214 see also Dying patient, care of
Oral retinoids, for acne vulgaris, 387 basic principles of, 432–433
Orgasm, disorders of, 267–268 bleeding in, 456–457
Orlistat, for prevention of type 2 diabetes, breathlessness and, 443–446, 444f, 444t
412 cough and, 446
Osgood-Schlatter disease, 152 depression and anxiety in, 455–456
Osteoarthritis, 143–145, 144t end-stage chronic obstructive pulmonary disease in, 103–104
Osteopenia, 163 gastrointestinal symptoms and, 447–448
Osteoporosis, 163–165 neurologic symptoms of, 451–453
corticosteroid-induced, 164–165 oral symptoms in, 451
menopause-related risk, 201–202, 205 skin symptoms in, 453–455
in older people, 240 symptom management guidelines, 436–458
Index 551
Palmar wart, 390 Physical medicine, 27

Palpitations, in heart failure, 79–80 Physician assistants/associates, primary care and, 3
Pancreatic diabetes, 411 Physiotherapists, primary care and, 3
Pancreatic disease, 113 Physiotherapy
Panic attack, first aid for, 325 breathlessness, 445
Panic buttons, 334 chronic capsulitis, 150
Panic disorder, 324 glenohumeral joint instability, 150
Papule, 395 knee pain, 152
Paraffin-containing ointments, for dry eye, 373 low back pain, 146
Paralinguistics, 12 motor neurone disease, 184
Paraplegia, 188–189 osteoarthritis, 144
Parasitic infections, in skin, 395 Parkinson disease, 179
Parasympathomimetic drugs, in chronic simple glaucoma, 378 rheumatoid arthritis, 160
Parathyroidectomy, 421 spondyloarthropathies, 161
Paratyphoid fever, incubation period and infectivity of, 472t–473t tennis elbow (lateral epicondylitis), 151
Parkinson disease, 179–182, 463 Pica, child with, 57–58
Parkinson plus syndromes, 463 Picato gel, for actinic keratosis, 396
Parkinsonism, 179 Pigmented basal cell carcinoma, 396–397, 398t
Parotid tumour, 361 Pill-in-the-pocket, in paroxysmal atrial fibrillation, 83, 84b
Paroxysmal atrial fibrillation (PAF), 83–84, 84b Pilocarpine, in chronic simple glaucoma, 378
Paroxysmal supraventricular tachycardia, 84–85 Pimecrolimus, for moderate flares, 384
Parvovirus B19 infection (fifth disease/slapped cheek syndrome), Pioglitazone, for type 2 diabetes mellitus, 413
219 Pituitary adenoma, 196
Patellofemoral pain, 152 Pityriasis versicolour, 395
Patient preferences, in four-topic approach, 24 Pizotifen, for migraine prophylaxis, 173
Peak expiratory flow, in normal subjects, 484, 484f Placenta praevia, 217
Peak flow Plane wart, 390
asthma, acute, 98 Plantar fasciitis, 153
chronic obstructive pulmonary disease, 102 Plantar wart, 390
values, normal, in children, 483, 483t Plaque, 395
Pelvic exercises, 224 Pneumococcal immunization, 101–102
Pelvic inflammatory disease (PID), 192, 198–199 in chronic heart failure, 76
Pelvic pain, in pregnancy, 216 in older people, 230
Penile sheaths, 346 Pneumococcal infection, 289–290
Penis, painful, child with, 55 Pneumonia
Pentoxifylline, for leg ulcers, 400 admission criteria (CRB-65 score), 100
Peptic ulcer, 112, 156 community-acquired, 100
Percutaneous coronary intervention (PCI), for stable angina, 74–75 Mycoplasma, 100
Perennial rhinitis, 362 risk factors for, 100
Perineal pain, postnatal, 226 Polio, vaccination, 283
Perineum, itchy, child with, 54 Pollen-food allergy syndrome, 404
Perioral dermatitis Polycystic kidneys, 351
acne rosacea and, 387 adult, 351
acne vulgaris and, 387 genetic counselling, 210
Peripheral arterial disease, 140 Polycystic ovary syndrome, 194, 196–197
Peripheral nerves, testing, 498–499, 498t–499t Polymyalgia rheumatica, 165–166, 165b
Peripheral vascular disease Polypharmacy, 8
oral contraception, contraindications, 248 Pompholyx eczema, 384
Peritonsillar abscess (quinsy), 366 Population, challenges, primary care and, 2
Persona, 261 Portal hypertension, 127–128
Pertussis, 290–291 Posterior fossa tumour, 358
incubation period and infectivity of, 472t–473t Postexposure prophylaxis, for HIV, 274
Petit mal, 177 Postherpetic neuralgia, 392
Phaeochromocytoma, in resistant hypertension, 71 Postlaryngectomy, 366
Phenobarbital, 500t Post-Lyme syndrome, 288
Phenylketonuria, problems associated with, 517t–520t Postnatal care, 224–227
Phenytoin, 500t Postnatal depression, 318–319
Philosophical ethics, 20t management of, 226
Phobias, 325 Posttraumatic stress disorder, 221, 321
Phone etiquette, 16–17 Postural ankle oedema, 241
Phosphodiesterase type-5 (PDE5) inhibitor, 266 Povidone-iodine, for acute infective conjunctivitis, 372
Physical abuse, in older people, 242 Practice organisation, long-term conditions and, 10
552 Index
Prader-Willi syndrome, problems associated with, 517t–520t principles and practice of, 1–6
Precancerous skin lesions, 396–397 clinical pharmacist in, 3
Precocious puberty, child with, 59–60 medical assistants in, 4
Prednisolone, as alternative to NSAID, 156 mental health professionals in, 3–4
Preeclampsia, 220 nurse practitioners in, 2–3
Pregabalin, 442 physician assistants/associates in, 3
Pregnancy physiotherapists in, 3
see also Ectopic pregnancy; Prepregnancy care technology in, 4
abdominal pain in, 217 Primary hyperaldosteronism, in resistant hypertension, 71
abnormal lie in, 222 Primary hyperhidrosis, 400
anaemia in, 222 Primary hyperparathyroidism (PHPT), 421
antenatal care for, 214–215 Primary hyperthyroidism, 418–419
asthma in, 223 Primary hypothyroidism, 418
bacteriuria in, 221 Primary sclerosing cholangitis, 126
connective tissue diseases in, 162 Primidone, 500t
depression, 316 Prion disease, 235b
diabetes in, 223 (see also Gestational diabetes) Professional ethics, in general practice, 24, 24t–25t
disease-modifying antirheumatic drug (DMARD), Professional norms, 20t
contraindications, 159 Professionalism, 20–21, 20f
drug misuse in, 224 Progestogen-only pill, 254–255
eclampsia in, 221 advice for, 254–255
epilepsy in, 223 health risk of, 254
excessive weight gain, 220 indication for, 254
food allergies and, 405 for older women, 254, 262
fundal height in, 220 procedure for, 254
glycosuria in, 221–222 risks of, 254
heartburn in, 216 side effects of, 255
high head in, 222 Progestogens
hypertension in, 220–222 endometriosis, 193
immunizations in, 503, 503t hormone replacement therapy, 203
inflammatory bowel disease in, 117–118, injectable, 255–256, 257t
223–224 intermenstrual bleeding, 195
jaundice, 124 irregular periods, 194
lower urinary tract infection in, 343 menopause, 202
migraine in, 173–174 menorrhagia treatment, 194
nausea and vomiting of, 216 Prognostication, 458
pelvic pain in, 216 Prokinetics, dyspepsia and, 112
postmaturity, 222–223 Prolapsed disc, 147
preeclampsia in, 220–221 Propranolol, for migraine prophylaxis, 172
premature rupture of membranes, 222 Propylthiouracil, 419
problems in, 216–224 Prostaglandin analogues, in chronic simple glaucoma, 378
proteinuria in, 221 Prostate, carcinoma of, 348–349
pruritus in, 219 Prostatism, 316
in rhesus-negative women, 222 Prostatitis
termination of, 263–264 acute, 342
referral request for, 21b chronic, 343
thyroid disease in, 223, 420 Protein
vaginal bleeding in, 216 reference ranges for, in young adults, 514t–515t
Pregnancy-induced hypertension (PIH), 220 in urine, child with, 52–53
Premature ejaculation, 267–268 Protein C deficiency, 248
Premature rupture of membranes, 222 Protein S deficiency, 248
Premenstrual spotting, 195 Proteinuria, 88
Premenstrual syndrome, 199–200 asymptomatic, 350
Prenatal diagnosis, screening and, 215 chronic kidney disease and, 351
Prepregnancy care, 210–212 in pregnancy, 221
Pressure sores, in multiple sclerosis, 186 Proteus infection, 343
Pressure ulcers, 188–189 Proton pump inhibitors
Pretravel advice, 294–299 gastrooesophageal reflux disease, 112
Prevotella species, 198 nonsteroidal antiinflammatory drug-induced ulcer, 155
Primary biliary cholangitis, 126 Prucalopride, constipation management, 114
Primary care Pruritus
challenges of, 2 palliative care and, 454–455
models of, 4–5 in pregnancy, 219
Index 553
Psoriasis, 388–389 Recurrent cellulitis, 393

chronic plaque, 388–389 Recurrent uveitis, 374
erythrodermic, 389 Red eye
facial, 389 following surgery, 377
flexural, 389 gritty irritable, 372–374
genital, 389 painful, not gritty, 374–375
guttate, 389 Red flags
nail, 389 low back pain, 147
pustular, 389 neck pain, 149
scalp, 389 5-α reductase inhibitor, for benign prostatic hypertrophy, 347
Psychiatric crises, 328 Referral, for erectile dysfunction, 266–267
Psychiatric history, prepregnancy care for, 212 Reflex anoxic syncope (RAS), 45
Psychiatric problems, 313–339 Refractory coeliac disease, 120
compulsory admission, 328–330 Refugees see Immigrants
Psychologic therapy, in posttraumatic stress disorder, 321 Registration, in visual handicap, 379
Psychological abuse, in older people, 242 Regurgitation, palliative care and, 447–448
Psychopathic disorder, 329 Rehabilitation, 27–28
Psychosis aims of, 27
acute disorders, 327–328 evidence-based, 28
admission, 328 medicine, 27
drug treatment, 327–328 Rehydration, for primary hyperparathyroidism, 421
in older people, 237–238 Reiter syndrome, 161
in Parkinson disease, 181 Remote prescribing, 17
precautions when visiting disturbed patient at home, 328 Renal disease, vaccination for patients with, 283
referral, 328 Renal failure
Psychotherapy, for depression, 317–318, 332 end-stage, 464–467, 465t
Psychotic illness, early warning form for use in, indicators of poor prognosis for, 464
508, 508t medications for, 465, 465t
Puberty, stages of, 481–482, 481f pharmacologic considerations in, 464
Pubic hair development, 481f, 482 symptom management for, 465–467, 466t
Pubic lice, 395 symptoms of, 464
Public health ethics, 20t with hypertension, treatment of, 69
Puerperal psychosis, 226, 318 in resistant hypertension, 71
Pulmonary rehabilitation unit, 103 Renal function, decline, frailty and, 231
PUO see Pyrexia of unknown origin Renal problems, 340–353
Pustular psoriasis, 389 Research ethics, 20t
Pyelonephritis, 351 Respiratory disease, end-stage, 467–468
acute, 344 anticipating care needs and management of, 468
Pyoderma gangrenosum, 392 indicators of poor prognosis of, 467
Pyrexia of unknown origin (PUO), 40 oxygen in, 468
symptom management in, 467–468
Q Respiratory problems, 93–108
Quality of life, in four-topic approach, 24 Respiratory tract infections
Quinsy (peritonsillar abscess), 366 care pathway for, 493–494, 493t–494t
lower respiratory tract, 99–101
R Restless legs syndrome, 467
Rabies, 297–298 in older people, 241
incubation period and infectivity of, 472t–473t Retarded ejaculation, 268
Radial nerve, testing, 498t–499t Retinoblastoma, 526t–530t
Radioactive iodine, 419 Retinopathy, diabetic, 415
Raised intracranial pressure, 175 Rett syndrome, problems associated with, 517t–520t
Ramsay Hunt syndrome, 356, 361 Revascularization, stable angina, 74–75
Rapid tranquilisation, for self-harm, 327–328 Rhesus-negative women, pregnancy in, 222
Rapport, 12 Rheumatic fever, 364–365
Rash, nonblanching, child with, 56–57 Rheumatoid arthritis, 158–161
Rate control, in atrial fibrillation, 81 Rheumatoid factor, 159
Raynaud syndrome, with hypertension, treatment of, 69 Rhinitis, 362–363
Reactive arthritis, 161 Rhinosinusitis, 361–362
Reading, in older people, 238 RICE treatment, 152
Reasonable adjustments, 30 Rickettsial infection, in travellers returning from abroad,
Recovery, of older people, 232 300–301
Recurrent abrasions, in eye, 374 Right heart failure, 79
Recurrent allergic eye disease, 372 Rivaroxaban, for atrial fibrillation, 83
554 Index
Rodent ulcer, 397 obsessive-compulsive disorder, 326

Rosacea, acne vulgaris and, 387 panic disorder, 324
Rotator cuff lesions, 149–150 phobias, 325
Rotaviral gastroenteritis, incubation period and infectivity of, premenstrual syndrome, 199–200
472t–473t social anxiety, social phobia, 325
Routine schedule, of immunizations, 404t Self-harm, 326–327
Royal College of Physicians Three Questions, 97t Self-help
Royal National Institute for the Blind (RNIB), 379 of depression management, 315
Rubella of generalized anxiety, 323
incubation period and infectivity of, 472t–473t of panic disorder, 323
in pregnancy, 217–218 of phobias, 325
prepregnancy care for, 212 Self-management asthma, 97
osteoarthritis, 144
S Semen, normal values of, 265
Safe sex, 199 Sensorineural hearing loss, 357
Salicylic acid, for warts, 390 Sertraline, 456
Salmonellosis, incubation period and infectivity of, 472t–473t Serum natriuretic peptides, for heart failure, 76, 76t
Salt, in hypertension, 68 Severe dyskaryosis, cervical, 206, 206t
Sarcoma, 526t–530t Severe flares, steroids for, 384
Scabies, 395 Severe headache of sudden onset, 175
incubation period and infectivity of, 472t–473t Severe inflammatory acne, 386
Scalp Sex hormone binding globulin, 422
infection, 394 Sexual abuse, in older people, 242
psoriasis, 389 Sexual activity, in chronic heart failure, 76
seborrheic dermatitis in, 386 Sexual desire, lack of, 267
Scarlet fever, incubation period and infectivity of, 472t–473t Sexual intercourse, in pregnancy, 213
Schistosomiasis, in long-term travellers, 303 Sexual problems, 265–268
Schizophrenia, 314–315 multiple sclerosis, 187
chronic, 330–332 older people, 242–243
Sciatic nerve, testing, 498t–499t postnatal, 226
Sciatica, 147 Sexuality issue, in paraplegia, 189
Sclerosing basal cell carcinoma, 397 Sexually transmitted diseases, 268–269
Scotland and Northern Ireland, regulations for, 329–330 in long-term travellers, 304
Screening Shared decision making, 13–14
breast cancer, 203 Shared medical appointments, primary care and, 5
cervical, 203, 205–206, 206t Shigellosis, incubation period and infectivity of, 472t–473t
chronic simple glaucoma, 378 Shingles vaccine, 293
older people, 230–231 Short stature, 58
ovarian cancer, 200, 200t Short-wave diathermy, low back pain, 147
postnatal depression, 226 Shoulder exercises, 148, 150
prenatal, 215 Shoulder pain, 149–150
prostate cancer, 348 Sialorrhoea, palliative care and, 451
Scrotum, painful, child with, 56 Sick euthyroid, 419
Seasonal rhinitis, 362 Sick sinus syndrome, 85
Sebaceous hyperplasia, 396 Sickle cell disease, 210–211
Seborrheic dermatitis, 385–386 Silent chest, in child with wheeze, 41
Seborrheic eczema, 387 Sinusitis, 40
Seborrheic keratosis, 395–396 Sjögren syndrome, 162, 367
Secondary hyperthyroidism, 419 Skeletal muscle spasm, 442–443
Secondary hypothyroidism, 418 Skin cancers, 526t–530t
Seizures, 434–435 Skin infection, 390–395
child with, 49 bacterial, 392–394
at end of life, 467 breastfeeding and, 225
management of, 434–435 fungal, 394–395
palliative care and, 451 parasitic, 395
presenting features of, 434 viral, 390–392
Selective serotonin reuptake inhibitors (SSRIs), 316 Skin infestation, 390–395
anxiety disorders, 322–323 Skin lesions, and malignancies, 395–399
discontinuation reactions, 317 benign, 395–396
generalized anxiety, 323 malignant, 397–399
irritable bowel syndrome, 114 precancerous, 396–397
menopause-related vasomotor symptoms, 201 Skin problems, 381–402
Index 555
Slapped cheek syndrome, 219 noncardiac chest pain and, 75

incubation period and infectivity of, 472t–473t referral for, 74
Sleep problems revascularization in, 74–75
fibromyalgia, 167 workup in, 74b
insomnia (see Insomnia) Staphylococci, in skin, 392–393
Small head, child with, 47 Statins, 127
Smoking cessation, 97 for cardiovascular disease, 87
age-related macular degeneration, 378 for hypertension, 68
cardiovascular disease, 86–87 Status epilepticus, 176, 434–435
chronic heart failure, 76 Sterilization, 263
chronic obstructive pulmonary disease, 101–102 Steroid-induced diabetes, management of, 458
contraception, young people and, 262 Steroid-induced osteoporosis, 164–165
Crohn disease, 115 Steroid injections
diabetes mellitus, 415 carpal tunnel syndrome, 151
dyspepsia, 110 contraindications, 154
gastrooesophageal reflux disease, 112 knee, 152
hypertension, 68–69 osteoarthritis, 145
infertility, 265 postinjection advice, 154
myocardial infarction, 73 shoulder pain, 149
in older people, 230 soft tissue lesions, 153–154
oral contraception, contraindications, 248 spondyloarthropathies, 162
prepregnancy, antenatal care, 213 tennis elbow (lateral epicondylitis), 151
snoring management, 364 Steroid therapy
Smooth muscle spasm, 442 asthma, acute attack, 97
Snoring, 363–364 chronic obstructive pulmonary disease, acute exacerbation,
Social anxiety/social phobia, 325 102–103
Social isolation, in cardiovascular disease, 86 polymyalgia rheumatica, 165–166
Social situations, older people in, 238–239 rheumatoid arthritis, 159
Sodium-glucose cotransporter 2 (SGLT-2) inhibitors, for type 2 temporal arteritis, 165–166
diabetes mellitus, 413 Steroids, 442
Sodium valproate, for migraine prophylaxis, 173 for atopic eczema, 383–384, 383t
Soft, nonkeratinised warts, 306 indications for, 457
Soft tissue lesions, steroid injection, 153–154 inhaled
Soft tissue sarcoma, 526t–530t asthma, 94–95
Soiling, child, 43 chronic obstructive pulmonary disease, 103
Solar keratosis, 396–397 infants, 94–95
Solar lentigo, 396 pregnancy, 223
Somatic complaints, 314 for intracerebral malignancy, 452
Sore mouth, 366–367 for localized acute dermatitis, 385
Sore throat, 364–365 stopping/withdrawing, 457–458
Spacers, 96 Steroids, topical
Spasm intranasal
skeletal muscle, 442–443 rhinitis, 362
smooth muscle, 442 secretory otitis media (glue ear), 357
Spasticity venous eczema, 399
in multiple sclerosis, 186 Stomatitis, oral, palliative care and, 451
paraplegia, 188 Stool, worms in, child with, 54–55
Spirometry Straight leg raising, 147
asthma, 93–94 Streptococcal infection
chronic obstructive pulmonary disease, 101 see also Pneumococcal immunization
Spleen, active, vaccination for people without, 282–283 group B, in pregnancy, 218–219
Spondyloarthropathies, 161–162 psoriasis, 388
Sports, active in, with hypertension, treatment of, 69 in skin, 392–393
Sprains, 152 tonsillitis, 364
Squamous cell carcinoma, 397–398, 399b Streptococcal pharyngitis, incubation period and infectivity of,
Squamous cell carcinoma in situ, 397 472t–473t
Squamous chronic otitis media, 356–357 Stress
Stable angina, 73–75 agitation in older people and, 233
cardiovascular risk reduction of, 74 in hypertension, 68
drug treatment of, 74 Stress incontinence, 344–345
selecting, 74 menopause-related, 202
management of, 74 Stress reactions, 320–322
556 Index
Stroke, 182–184, 358 Testing peripheral nerves, 498–499, 498t–499t

etonogestrel-releasing implant (Implanon), 257 Tetanus
oral contraception, contraindications, 248, 255 incubation period and infectivity of, 472t–473t
Strongyloides, in long-term travellers, 303 vaccination, 283
Styes, 375 Thalassaemia, 210–211
Subarachnoid haemorrhage, 175 Theophylline, 500t
Subclinical hypothyroidism, 418 Therapy, 27–28
Sudden infant death syndrome, prevention of, 224 Thiazides, for hypertension, 69–70
Suicide Thiopurines, 116
bereaved by, 320 Thompson test, 153
deliberate self-harm relationship, 326–327 Threadworms, incubation period and infectivity of, 472t–473t
depression, 316, 319–320 Throat, 364–368
management of, 319–320 feeling of lump in, 366
in older people, 232 sore, 364–365
Sulphonylurea, for type 2 diabetes mellitus, 413 Thromboembolic disease, hormone replacement therapy related
Superficial basal cell carcinoma, 397, 398t risk, 203
Superficial spreading melanoma (SSM), 398 Thrombogenic mutations, 248
Superior vena cava obstruction, 433–434 Thrombolysis, 72
Surgery Thrombosed external haemorrhoid, 138
for chronic simple glaucoma, 379 Thrush
for myopia, 379–380 denture wearers, 368
for rheumatoid arthritis, 161 oral, 365
Surgical problems, 134–141 Thyroid disease
Suspected lithium toxicity, bipolar affective disorder, 334 in diabetes, 417–420
Swallow, poor, advanced neurologic disease and, 462 in pregnancy, 223, 420
Sweating (paraneoplastic), palliative care and, 454 in resistant hypertension, 71
Swimming, epilepsy and, 177 Thyroid nodules, 419
Syncope, in children, 45 Thyroid surgery, 419
Syphilis, 271, 307 Thyroiditis, postnatal, 223
Syringe drivers, 459–462 Tic, child with, 50
drug stabilities in, 524–525, 524t–525t Tick bites, 288
indications and practicalities of, 459–461 Tickborne encephalitis, 298
Systemic corticosteroid, for irritant and allergic contact dermatitis, Tinea, 394
385 incubation period and infectivity of, 472t–473t
Systemic lupus erythematosus (SLE), 162, 351 Tinea capitis, 402
acne rosacea and, 387 Tinnitus, 360–361
oral contraception, contraindications, 248 Tizanidine, 442
Tonic-clonic or generalised seizures, 177
T Tonsillectomy, 365–366
Tacrolimus, for moderate flares, 384 Tonsillitis
Tall stature, 59 acute recurrent, 366
Tay-Sachs disease, 211 chronic, 366
Team, primary care, 2–4 streptococcal infection, 364
clinical pharmacist in, 3 Toothache, 367
medical assistants in, 4 Topical calcineurin inhibitors, in moderate flares, 384
mental health professionals in, 3–4 Topical corticosteroids, for chronic plaque psoriasis, 388
nurse practitioners in, 2–3 Topical haemorrhoidal preparations, 138
physician assistants/associates in, 3 Topical medications, anal fissure, 139
physiotherapists in, 3 Topiramate, for migraine prophylaxis, 172
Telangiectasia, treatment of, 388 Total pain, 443
Telemedicine, primary care and, 4 Toxic shock syndrome, 260
Telephone consultation, 16–17 Toxoplasmosis, prepregnancy care for, 218
communication skills for, 16 Trabeculectomy, for chronic simple glaucoma, 379
confidentiality and, 16–17 Trabeculoplasty, for chronic simple glaucoma, 378–379
Telephone triage, primary care and, 5 Traction alopecia, 402
Temporal arteritis, 166 Tramadol, 438
steroid therapy, 165–166 Transcutaneous nerve stimulation (TENS)
Tenesmus, 443 low back pain, 147
Tennis elbow (lateral epicondylitis), 150–151 rheumatoid arthritis, 160
Tension-type headache, 171, 173–174 Transdermal patch, combined, 253
Terazosin, for hypertension, 70 Transient ischaemic attacks (TIAs), 183–184
Terbinafine, for tinea, 394 oral contraception, contraindications, 248
Index 557
Transurethral resection of prostate (TURP), 348 Ulcerative colitis, 114–117

Transverse lie, 222 Ulcerative gingivostomatitis (Vincent’s angina), 365
Trauma, eye, 373–374 Ulnar nerve, testing, 498t–499t
Travellers Ultrasound
immunizations for, 474, 474t congenital anomalies, prenatal diagnosis, 215
long-term, screening of, 303–304 ectopic pregnancy, 216
general approach, 304–305 low back pain, 147
management of specific STIs, 305–311 Ultraviolet light burns, in eye, 374
schistosomiasis, 303 Uncertain vaccination history, 282
sexually transmitted infection, 304 United Kingdom, taking medicines out of, 295
strongyloides, 303 Unstable angina, 71–73
tuberculosis, 303–304 cardiac rehabilitation for, 72–73
returning from abroad, symptoms in, 299–303 factual advice for, 73
amoebic liver abscess, 301 myocardial infarction and, 72
dermatologic presentations, 302, 302t Unsteadiness, 360
diarrhoea, 301–302 Upper gastrointestinal cancer, 526t–530t
enteric fever, 300 Upper limb problems, 149–151
eosinophilia, 302–303 Urea breath test (UBT), 111
Epstein-Barr virus, 301 Urethral syndrome, 342
fever, 299–300 Urethritis, nonspecific, 270
fever-arthralgia-rash (FAR) arboviruses, 301 Urge incontinence, 345
influenza, 301 Urinals, 346
malaria, 300, 300t Urinary catheters, and problem solving, 346
rickettsial infection, 300–301 Urinary drainage bags, 346
viral haemorrhagic fever, 301 Urinary incontinence, 344–346
viral hepatitis, 301 in older people, 344–345
Travellers’ diarrhoea, 295 postnatal, 226
Travelling, opioids and, 438 stress, 344–345
Travel-related vaccination, 297–299 menopause-related, 202
cholera, 297 urge, 345
Japanese encephalitis, 297 Urinary problems, 340–353
rabies, 297–298 Urinary stones, 349–350
special considerations for vaccines on the UK vaccination Urinary tract infection, 341–344
schedule, 297 lower
tickborne encephalitis, 298 in catheterized patients, 343–344
typhoid, 298–299 in men, 342–344
yellow fever, 299 in postmenopausal women, 343
Trichomonas vaginalis (TV) infection, 270, 306 during pregnancy, 343
Trichotillosis, 402 paraplegia, 188
Tricyclic antidepressants (TCAs) urinary incontinence and, 344
anxiety disorders, 324 Urine
insomnia, 322 blood in, child with, 53
irritable bowel syndrome, 114 protein in, child with, 52–53
low back pain, 146 reference ranges for, in young adults, 515t
migraine prophylaxis, 173 Urologic cancers, 526t–530t
osteoarthritis, 144 Urticaria, 401–402
tension-type headache, 174 Uveitis, spondyloarthropathies and, 161
Triptans, for migraine, 172
Tubal occlusion, 263 V
Tuberculosis, 101, 292 Vaccination, general considerations, 279–283, 280t
incubation period and infectivity of, 472t–473t adverse reactions, 281
in long-term travellers, 303–304 contraindications, 281
Tuberous sclerosis, problems associated with, live vaccines, 281–283
517t–520t special cases, 282–283
Twin-to-twin transfusion syndrome, 215 special considerations for use of, 281–282
Twitching, 375 organization of an immunization programme in primary care,
Typhoid fever, 118, 298–299 279
incubation period and infectivity of, 472t–473t prevaccination quality control checks, 281
procedures when giving an immunization, 280–281
U specific infectious diseases and immunizations, 283
Ulcerated basal cell carcinoma, 397, 398t vaccine damage payments, 281
Ulcerated sore mouth, 366–367 Vaccines see Immunizations
558 Index
Vaginal bleeding Vitamin C, 100

intermenstrual, 195 Vitamin D, 347
postmenopausal, 195 in older people, 231
postnatal care and, 225 osteoporosis management, 164
in pregnancy, 216 prepregnancy care, 213
Vaginal discharge, 197 Vitamin E supplementation, in Alzheimer disease,
Vaginal dryness, menopause-related, 202 237
Vaginal ring, combined, 253–254 Vitamin K, 178
Vaginismus, 267 postnatal administration, 224
Vaginosis, bacterial, 198 of prepregnancy care, epilepsy, 212
Valproate, 500t Vomiting
Varicella (chickenpox), 292–294 combined oral contraceptive (COC) pill and,
incubation period and infectivity of, 472t–473t 251
prepregnancy care for, 218 dyspepsia alarm feature, 110
treatment of, 293 end-stage renal failure and, 467
Varices, 128–129 infant, 61–62
Varicose eczema, 399 palliative care and, 447–448
Varicose veins, 139–140 in pregnancy, 216
Vascular dementia, 235b prolonged vertigo, 358
Vascular disease
injectable progestogens and, 255 W
in resistant hypertension, 71 Walking sticks, 144
Vasculitis, 351 Warfarin, contraindications to, in atrial fibrillation, 83b
Vasectomy, 263 Warts, 390–391
Venepuncture, HIV and, 274 anogenital, 270
Venous eczema, 399 incubation period and infectivity of, 472t–473t
Venous thromboembolism, oral contraception, contraindications, Watery eye, 375
248, 249t, 250–251 Weakness
Venous thrombosis, 248 in end-stage heart failure, 79
Ventricular ectopics, frequent, 80 in multiple sclerosis, 186
Ventricular tachycardia, 85 Weight
Verapamil, for hypertension, 70 faltering, infant with
Verbal communication, 12 after first few weeks, 62–63
Vertigo, 358–359 in first few weeks, 62
benign paroxysmal positional, 359–360 loss, 122–123
Ménière’s disease and, 359 dyspepsia alarm feature, 110
treatment, 358–359 polycystic ovary syndrome, 196–197
Vestibular neuronitis, 358 in pregnancy, excessive gain, 220
Vestibular rehabilitation exercises, 359 Weight reduction
in older people, 240 cardiovascular disease, 86–87
Vincent’s angina (ulcerative gingivostomatitis), 365 chronic heart failure, 76
Violent/potentially violent patients, dealing with, 334 gout, 157
Viral haemorrhagic fever, in travellers returning from abroad, 301 hypertension, 68
Viral hepatitis, 125, 284–286, 301 myocardial infarction, 73
oral contraception, contraindications, 248 snoring management, 364
Viral infections, in skin, 390–392 Wheeze, child with, 41–42
Virtual ward, primary care and, 5 Whispered voice test, 357–358
Visual acuity testing, 371 White coat hypertension, 66
Visual handicap, 379 Whooping cough see Pertussis
Visual impairment Williams syndrome, problems associated with,
acute distortion, 376 517t–520t
acute disturbance, 375–377 Wilms tumour, 526t–530t
acute painless loss of vision, 375–376 Wilson disease, 127
decreased vision with pain, 376 Withdrawal
gradual loss, 377–379 oestrogen, headache, 253
long-term deterioration of, after surgery, 377 steroids, 457–458
multiple sclerosis, 187 Women
temporal arteritis, 166 health, 191–208
Vitamin A, 213 orgasmic problems in, 268
Vitamin B6 (pyridoxine), 200 Worms, in stool, child with, 54–55
Vitamin B12 status, in older people, 231 Wrist ganglia, 151
Index 559
X reference ranges for, 514–515

Xerostomia, palliative care and, 451 blood, 514t–515t
X-rays haematology, 515t
low back pain, 147 urine, 515t
osteoarthritis, 145
Z
Y Zinc, 100
Yellow fever, 299 Zolmitriptan, for migraine, 173
Yellow flags, 146 Zyclara, for actinic keratosis, 396
Young adults
cataracts of, 377
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Practical General Practice

Adam Staten, MA (Cantab), MBBS, MRCP (UK),

Paul Staten, MBBS, MA (Cantab), DRCOG, MRCGP

For additional online content visit

Edinburgh London New York Oxford Philadelphia St Louis Sydney 2019

First edition 1988

Senior Content Strategist: Pauline Graham

Last digit is the print number: 9 8 7 6 5 4 3 2 1

Preface, vii 16 Contraception, Sexual Problems, and Sexually

2 Long-Term Conditions, 7 20 Ear, Nose, and Throat Problems, 354

3 Communication Skills, 11 21 Eye Problems, 371

4 Ethics, 19 22 Skin Problems, 381

Section 2: Manual of Clinical Practice 24 Diabetes and Endocrinology, 409

8 Respiratory Problems, 93 26 Palliative Care and Care of the

10 Surgical Problems, 134 Section 3: Appendices

Appendix 7 Stages of Puberty, 481 Appendix 19 Immunizations in Pregnancy, 503

Bullets Where the order is important we number the list:

Annemieke Bikker, MSc Lynsay Crawford, MBChB

David Carty, MBChB, PhD, FRCP Russell Drummond, MBChB

Lynn A. Legg, PhD, MPH Hilary Lockhart Pearce, MBChB, MRCPCH

Stewart W. Mercer, MD, PhD, FRCGP, FFPHM, FRCPE

Aziz Sheikh, BSc, MBBS, MSc, MD Harry Hao-Xiang Wang, PhD

add to the pressures of working within primary care and

Telephone Triage proactive, and multidisciplinary input. It is an elabora-

Prevalence of Long-Term Conditions index condition (a condition of primary concern) is

prevalence of 69% in 18- to 44-year-olds, 93% in 45- to Healthcare Utilisation

Working With Interpreters Email Consultations

Confidentiality • The physical arrangement of the room can facilitate or

compassionate way, responding positively with clear Communication Skills for

General Practice Ethics f. Public health obligations

Shorthand for Interpretation

in general practice is very much concerned with every- Ethics Law

Benefits Burdens Risks

Interpretation Examples Not to Be Confused With

P Public trust Upholding the confidence of society in the profession

S Standards Performance benchmarks in clinical practice

Human Rights and Disability

• Environmental modification (internal and external EVIDENCE-BASED PRACTICE RESOURCES

HEALTH CARE FOR PEOPLE WITH INTELLECTUAL DISABILITIES

Challenge One Possible Solution

RESOURCES FOR PARENTS AND Resources for Carers

1. Person with disability

World Health Organization. (1946). Preamble to the Constitution of the

www.improvinghealthandlives.org.uk/uploads/doc/vid_11084_ World Health Organisation. (2013). Fact sheet 352. Disability

The Child With Pica

What to Do diarrhoea worse. However, overflow diarrhoea will not

What to Do • Assess for local tenderness found in costochondritis and

• Microcephaly can be caused by a variety of genetic and

What to Do • The first priority is to manage the seizure: check airway,

• Ask about a history of previous confirmed or uncon- • Management of a confirmed UTI:

• It is important to establish whether the enuresis is • Manage a UTI appropriately.

What to Do What to Look for on Examination

The Child With an Itchy Perineum • Do not overwash the area.

What to Look for on Examination What to Ask About the Child

What to Do • Plot parental heights on a chart and calculate the expected

What to Do • If hair is present at 6 to 8 years and is sparse, refer for

• The complaint of distress or vomiting with feeds is very What to Do

care for advice. These children need a hydrolysed formula What to Do

Hypertension • BOX 7.1 Definition of Hypertension

4. Blood: 6. Dietary Approaches to Stop Hypertension (DASH) diet: