A Sense of Self Hope in Lewy Body Disease Sorel Coward University of South Australia A report prepared for the Honourable Kelly Vincent (MLC) South Australian Parliamentary Internship December, 2017. PREFACE T his report was written for The Honourable Kelly Vincent, who, is a member of the Legislative Council (MLC) in the South Australian Parliament and leader of Dignity Party. The author is a post-graduate student and was commissioned to prepare this report as part of a Master’s of Social Work through the University of South Australia. THE HON. KELLY VINCENT, MLC A SENSE OF SELF i ACKNOWLEDGEMENTS I am grateful for the support and encouragement given to me by my sponsoring MP, The Honourable Kelly Vincent MLC, and her staff in Dignity Party. I am grateful for the inspiration and leadership offered to me by the South Australian Parliamentary Internship scholars, namely Professor Haydon Manning (Flinders University), Dr. Wayne Errington (University of Adelaide), and Dr. Cenz Lancione (University of South Australia). I am grateful for the support of my fellow students, especially on the occasion when I made the oral presentation of this report in Old Parliament House. Finally, I am grateful for my wife, Jane, who has been by my side for twentyfive years of marriage, and has supported me through three academic degrees. All this achievement has been possible because of you. I thank you all. Sorel Coward A SENSE OF SELF ii TABLE OF CONTENTS PREFACE....................................................................................................... I ACKNOWLEDGEMENTS .............................................................................. II ABBREVIATIONS ......................................................................................... V EXECUTIVE SUMMARY .............................................................................. VI CASE STUDY: MALCOLM’S STORY ............................................................ 1 CHAPTER 1: AGEING, DISABILITY & DEMENTIA IN AUSTRALIA ................ 4 AGEING ...................................................................................................... 4 DISABILITY .................................................................................................... 5 DEMENTIA ................................................................................................... 6 DEMENTIA IN ABORIGINAL AUSTRALIANS ........................................................ 7 CHAPTER 2: LEWY BODY DISEASE ............................................................. 9 TERMINOLOGY ............................................................................................. 9 CORE FEATURES ......................................................................................... 10 KEY ISSUE #1 : DIAGNOSIS .......................................................................... 10 The problem of misdiagnosis ............................................................ 11 The problem caused by low awareness of DLB ............................. 13 New Clinical Diagnostic Criteria ...................................................... 14 REM Sleep Behaviour Disorder ......................................................... 15 Improving DLB Diagnosis: ALBA Screening Instrument (ASI) ........ 17 Improving DLB Diagnosis: The Lewy Body Composite Risk Score 19 KEY ISSUE #2 : CAREGIVING ....................................................................... 20 The burdens and consequences of DLB carer stress .................... 20 Measuring the burdens experienced by DLB carers ..................... 22 Nursing Home Admission .................................................................. 24 CHAPTER 3: A NEW MODEL OF CARE ..................................................... 25 Person-Centred Care ........................................................................ 26 Palliative Care.................................................................................... 26 Supportive Dementia Care .............................................................. 27 RECOMMENDATIONS ............................................................................... 29 ABORIGINAL AUSTRALIANS .......................................................................... 29 RESEARCH ................................................................................................. 29 EDUCATION AND AWARENESS ..................................................................... 29 DIAGNOSIS ................................................................................................ 29 CAREGIVING ............................................................................................. 30 REFERENCES .............................................................................................. 31 A SENSE OF SELF iii APPENDIX A: REM SLEEP BEHAVIOUR DISORDER SCREENING QUESTIONNAIRE ....................................................................................... 35 APPENDIX B: REM SLEEP BEHAVIOUR DISORDER QUESTIONNAIRE – HONG KONG (RBDQ-HQ) ....................................................................... 36 APPENDIX C: ALBA SCREENING INSTRUMENT ....................................... 37 APPENDIX D: THE LEWY BODY COMPOSITE RISK SCORE (LBCRS) ........ 41 APPENDIX E: RELATIVE STRESS SCALE (RSS) ............................................ 45 APPENDIX F: SUPPORTIVE CARE ............................................................. 46 A SENSE OF SELF iv ABBREVIATIONS AD ASI DLB LBD LBCRS MCI NHA PD PDD RBD RBD1Q RBD-HK RSS Alzheimer’s Disease ALBA Screening Instrument Dementia with Lewy Bodies Lewy Body Disease / Lewy Body Dementia Lewy Body Composite Risk Score Mild Cognitive Impairment Nursing Home Admission Parkinson’s Disease Parkinson’s Disease Dementia REM Sleep Behaviour Disorder RBD Single Question RBD Hong Kong scale Relative Stress Scale A SENSE OF SELF v EXECUTIVE SUMMARY T hroughout his life, Robin Williams made us feel so good to be alive. He brought to us a reason to smile, to laugh, and to cry. Robin Williams also, very privately, struggled with depression for most of his life. On August 11, 2014, Robin Williams committed suicide. After his death, an autopsy of his brain showed that he had Lewy Body Disease (LDB). Prior to his death he had been diagnosed with Parkinson’s Disease, was known to experience periods of deep depression, anxiety and increasing episodes of psychosis, which are all classic symptoms of this newly discovered disease. In Australia, Lewy Body Disease (LBD) is the second most commonly diagnosed neurodegenerative dementia, after Alzheimer’s Disease (AD). As the disease slowly progresses, it causes almost total disability and eventual nursing home admission (NHA). For the person with LBD, and those family members who care for them, the progression of this disease is often totally overwhelming. Surprisingly, very little is known about LBD. We know nothing of what causes it or who might be at risk of developing it. At present, there is no known cure for LBD. Significant challenges exist in diagnosing and managing people with LBD. A diagnosis of LBD brings with it frustration, misunderstanding, a lack of appropriate and support along with social isolation. This report will investigate LBD in Australia, discuss some of the key issues, and recommend some important initiatives to better the lives of everyone affected by Lewy Body Disease. Sorel Coward A SENSE OF SELF vi CASE STUDY: MALCOLM’S STORY The following is a fictitious case study that can help us gain an appreciation of how someone with LBD, and their carers, might experience the world. The study has been drawn together from a number of different LBD stories that have been published elsewhere. This is the story of Malcolm. Malcolm is male, Caucasian, and seventy-five years of age. He and June have been married for fifty years. June, who is seventy-three years of age, lives on the outskirts of Perth, and has been caring for Malcolm who was diagnosed with LBD six years ago. However, Malcolm had been living with dementia symptoms for almost a decade before he received his diagnosis. After seeing many specialists, he had originally had been diagnosed with Alzheimer’s Disease. Malcolm had been experiencing increasing amounts of anxiety, hallucinations, paranoia, and aggression – all of which were not responding to the treatments normally effective in managing Alzheimer’s Disease. During one crisis, June was not able to cope with Malcolm’s behaviour and an acute emergency admission to hospital was a consequence. Unfortunately, the first treating hospital doctor was not experienced in the management of LBD, and administered an anti-psychotic medication to control Malcolm’s hallucinations, paranoia and aggressive behaviour. Because of this lack of experience, the doctor was unaware of the severe sensitivity associated with administering an anti-psychotic drug to someone with LBD. Malcolm had an adverse reaction to this drug and it was some time before his world return to normal again. A SENSE OF SELF 1 During the hospital admission, it became clear to June that she did not feel she could cope anymore. June felt that she was on her own, and felt misunderstood, tired, and frustrated; there was no one else to help. She just couldn’t go on any more. It was with a feeling of great relief that she agreed with the hospital staff that it would be best if Malcolm was admitted to a residential aged care facility. June also felt overwhelmingly guilty and sad that she could not keep her promise to Malcolm - a promise to keep him at home until he died. Malcolm spent a further ten months in the psychiatric ward of the hospital. The reason it took so long was that most aged care facilities had refused outright to admit Malcolm once they were told of his LBD diagnosis. In every instance, they said they did not have the staff or the skills to cope with the aggressive behaviour and violence that so often characterises people with LBD, like Malcolm. Finally, a place was found but it was a long way from where June lived. For the first time in their fifty-year marriage, June and Malcolm began living apart. Now, more than ever before, June felt alone and vulnerable. Malcom is now living in an aged care facility specialising in psychiatric disorders and behaviours, and while June feels the facility is “pretty ordinary”, in terms of its decor and facilities, the staff can effectively manage Malcolm’s complex behaviours. June catches two buses to get from her home to the nursing home. The journey takes an hour. June spends four hours with Malcolm before returning home on public transport. She does this four days a week. Although June’s quality of life has improved since Malcolm’s admission, as has her health, she is not so sure that being in a nursing home is the best option for Malcolm. With the burden of 24/7 care of Malcolm now lifted, June has had the time to reflect more about her experience of dementia. She feels that there is inadequate access to respite for dementia carers, like herself, because through the whole time she was caring for Malcolm, she had to carry this burden almost entirely on her own. June now feels that had she had more opportunities for having a break, along with some community based care support, it probably would have been possible, and better, for her to carry on caring for Malcolm at home, at least for a while longer. To this end, June has made the suggestion that the government would do well to fund a system of local respite homes, especially for those in the early stages of dementia. Ideally, when Malcolm would have been in respite care, June feels that changes could have been made to better support her in her work of caring. Unfortunately, this is more of an ideal than a reality A SENSE OF SELF 2 because, presently, only one of two options are available to people with dementia: care by one’s spouse/children or care in a nursing home. June had been in touch with a local carers group and there she discovered that whilst palliative care support and community based health care was provided to support people caring for those with many different illnesses, June did attend an Alzheimer’s Disease support group but, because Malcolm’s situation was so different from Alzheimer’s Disease, she felt they did not really understand her or could offer her support. June also contacted a local palliative care group but was unable to get any assistance because dementia was regarded as a chronic health condition, not as an end-of-life event. A few weeks ago, June received news that Malcolm’s health was deteriorating and he was only expected to live a few more weeks. It had been seven years since his diagnosis. A social worker came to help June with transport and to help with organising Malcolm’s funeral. June is very grateful for this help, but, quietly in her heart, she feels that the help she so desperately needed came too late. Imagine living your whole life ….and not remembering it, Imagine forgetting those you love the most… Imagine becoming as vulnerable as a small child… Imagine how terrifying it must be to have dementia. A SENSE OF SELF 3 CHAPTER 1: AGEING, DISABILITY & DEMENTIA IN AUSTRALIA The people of Australia, as a society, are experiencing a quantum change in their sense of self-identity. For a long time, we have focussed upon growth, development and expansion and with that an emphasis on supporting and promoting the survival of the strongest. Now, however, our population has aged so well that Australians are amongst the oldest peoples in the world. The proportion of people in our midst who will be regarded as being ‘older’, i.e. over the age sixty-five years, is dramatically increasing and is projected to continue to keep growing in an exponential manner. With that ageincrease in our population, the incidence of dementia illnesses is also steadily increasing. Now we must change our self-identity from strengthening the fittest to one where we start promoting the wellbeing of those who are the weakest and most vulnerable in our society. AGEING A ustralians now enjoy a life expectancy that is among the highest in world. Men aged 65 years, in 2013-2015 can expect to live another 19.5 years with an expected age at death being 84.5 years, whilst women aged 65 years in 2013-2015 can expect to live another 22.3 years, with an expected age at death being 87.3 years1. Over the last two decades the median age of the Australian population has increased by three years over the last two decades from 34 years in 1996 to 37 years in 2016. In June 2016, South Australia had the second highest median age (after Tasmania) of 42 years2. Noting that the median age of the world’s 1 Australian Institute of Health and Welfare (AIHW), Deaths, AIHW Website, viewed 10 October 2017, <https://www.aihw.gov.au/reports/lifeexpectancy-death/deaths-in-australia/contents/life-expectancy>. 2 Australian Demographic Statistics (ABS), June 2016, ABS website, viewed 10 September 2017, <http://www.abs.gov.au/ausstats/abs@.nsf/featurearticlesbyCatalogue/38 4EDBC8C1EB7717CA2581470023DAD1?OpenDocument>. A SENSE OF SELF 4 human population is 30 years3, this means that South Australians represent a community that has one of the highest median ages in world. Between 1996 and 2016, the proportion of Australia’s population aged 65 years and over increased from 12% to 15%. The rapid growth of ageing is projected to continue to increase over the next quarter of a century with 25% of all Australians predicted to be over the aged of 65 years by 20504. Between 1996 and 2016, the proportion of Australians aged 85 years and over doubled by 100% (1% to 2%)5 and by 2050 is expected to increase by another 250% to 1.8 million people6. Issues pertaining to ageing in Australia, and especially in South Australia, are of critical importance to both current and future policy development, particularly in regards to age-related health and disability. DISABILITY Along with an ageing Australia, the occurrence of age-related disability in Australia is also expected to significantly increase. In fact, it has already begun. The 2015 Survey of Disability, Ageing, and Carers (SDAC)7 indicated that some one in five Australians, over the age of eighteen years (18.3% or 4.3 million people), reported they were living with some form of disability or 3 United Nation 2017, World population prospects the 2017 revision: key findings and advanced tables, UN Department of Economic and Social Affairs: Population Division, New York. 4 Australian Human Rights Commission 2012, Age positive: promoting positive and diverse portrayals of older Australians, viewed 29 October, 2017, <https://www.humanrights.gov.au/sites/default/files/FTFOlderAustralians.pdf >. 5 Australian Demographic Statistics (ABS), June 2016, ABS website, viewed 10 September 2017, <http://www.abs.gov.au/ausstats/abs@.nsf/featurearticlesbyCatalogue/38 4EDBC8C1EB7717CA2581470023DAD1?OpenDocument>. 6 Australian Human Right Commission 2008, A human rights approach for ageing and health – respect and choice: home based and residential care for older people (2012), footnote 3: The Treasury, Australian Government, Australia to 2050: future challenges (2010), p. 56. 7 Australian Bureau of Statistics, Disability, ageing, and carers, Australia (SDAC) 2015, Australian Bureau of Statistics Website, viewed 4 September 2017, <http://www.abs.gov.au/ausstats/abs@.nsf/mf/4430.0>. A SENSE OF SELF 5 chronic health condition. The majority of people with a disability, nearly 79%, reported their impairment was a result of a physical health condition, and 21% reported their disability as being caused by a behavioural or mental disorder. These statistics, however, do not describe the clearest of pictures because disability is disproportionately experienced by people sixty-five years and over, with health problems and illness among the greatest causes of disability in this age group. This is clearly demonstrated in table 1. TABLE 1: PSYCHOSOCIAL DISABILITY AND AGE DEMENTIA Dementia is a condition that mainly affects older people and describes a syndrome, of a chronic and progressive nature, that causes cognitive impairment including memory, thinking, behaviour and personality. A SENSE OF SELF 6 Dementia results in increasing degrees of disability8. Dementia is most often caused by, and is usually only one symptom, of a number neurodegenerative illnesses including Alzheimer’s Disease, Lewy Body Disease, Vascular Dementia, and Fronto-Temporal Lobal Degeneration (FTLD), Huntington’s Disease, Parkinson’s Disease and Creutzfeldt-Jacob Disease. Secondary dementias are those caused by other (usually nonneurodegenerative illnesses) such as HIV, Korsakoff’s Syndrome (alcohol abuse related dementia), and acquired brain injury)9. While it is true some cognitive decline is normal with advancing age, it is not true that dementia is a normal part of ageing, notwithstanding commonly held myths in the general community to the contrary. Noting the above discussion about Australia’s ageing population, dementia is going to be one of the most significant sources of disability in this country. DEMENTIA IN ABORIGINAL AUSTRALIANS Like in most other regards about Aboriginal people in Australia, dementia amongst our First Nation people is characterised by unique complexities and social injustice. Importantly, there are significant cultural distinctions between how dementia is viewed amongst First Nation people and the wider Australian community. Firstly, in most Aboriginal cultures, dementia is often not recognised as a medical condition10. Instead dementia is often viewed as becoming “childlike”, “sickness” or “madness”. These are considered normal changes in some ageing Aboriginal people. But it is not until these behaviours break 8 World Health Organisation and Alzheimer’s Disease International 2012, Dementia: a public health priority, WHO, Geneva. 9 Alzheimer’s Australia 2017b, Types of dementia, Alzheimer’s Australia National Website, viewed 2 October 2017, <https://www.fightdementia.org.au/about-dementia/types-of-dementia>. 10 Arkles, RS, Jackson Pulver, LR, Robertson, H, Draper, B, Chalkley, S & Broe, GA 2010, Ageing, cognition, and dementia in Australian Aboriginal and Torres Strait Peoples, Neuroscience Research Australia and Muru Marri Indigenous Health Unit, University of New South Wales, Sydney. A SENSE OF SELF 7 Aboriginal cultural norms that assistance from outside of the community is sought and a diagnosis is determined11. It is an established fact that Aboriginal people experience one the poorest health outcomes of any community of people in the world and their experience of dementia follows a similarly tragic course. Aboriginal people experience dementia at a younger age and at a higher prevalence than the rest of the people of Australia. In fact, the prevalence rate amongst rural Aboriginal Australians has been determined at a rate of 5.2 times greater than the overall Australian rate of 2.4%12. One explanation for this is because it an established fact that Aboriginal people are exposed to significantly higher rates of known dementia risk factors, which include: • • • • • • • Smoking Physical inactivity Poor nutrition Being overweight or obese Excess alcohol consumption A low standard of education Heart disease and diabetes13 11 Arkles, RS, Jackson Pulver, LR, Robertson, H, Draper, B, Chalkley, S & Broe, GA 2010, Ageing, cognition, and dementia in Australian Aboriginal and Torres Strait Peoples, Neuroscience Research Australia and Muru Marri Indigenous Health Unit, University of New South Wales, Sydney, p. 2 12 Flicker, L, and Holdworth, K 2014, Aboriginal and Torres Strait Islander people and dementia: a review of the research, viewed 1 December 2017, < https://www.dementia.org.au/files/Alzheimers_Australia_Numbered_Publica tion_41.pdf>, p. 15. 13 Flicker, L, and Holdworth, K 2014, Aboriginal and Torres Strait Islander people and dementia: a review of the research, viewed 1 December 2017, < https://www.dementia.org.au/files/Alzheimers_Australia_Numbered_Publica tion_41.pdf>, pp. 13-14. A SENSE OF SELF 8 CHAPTER 2: LEWY BODY DISEASE Early in the twentieth century, Frederick Lewy (1885-1950) first discovered abnormal deposits of the alpha-synuclein protein in the brains of some patients who experienced dementia. LBD was first described by Japanese psychiatrist and neuropathologist Kenji Kosaka in 1976. To this day, autopsy remains the only definitive method of diagnosis of the Lewy Body Disease. It has been only been clinically diagnosable, i.e. by observable symptoms, since 2005. As such, LBD is a relatively new disease. TERMINOLOGY LBD is an umbrella term that encompasses Parkinson’s Disease Dementia (PDD) and Dementia with Lewy Bodies (DLB). The essential difference between each is the onset of timing. With the emergence of motor symptoms first PDD is the diagnosis and the emergence of cognitive symptoms first results in diagnosis of DLB. For much of its short history LBD has been, and is still commonly described, as Lewy Body Dementia. Increasingly, however, the term Lewy Body Disease is being used by peak bodies like Dementia Australia (formerly Alzheimer’s Australia). This report will principally focus on DLB. When it is appropriate, the term LBD will be used to describe both DLB and PDD. Essentially, there are only subtle differences between the two disorders. A SENSE OF SELF 9 CORE FEATURES The central characteristic of DLB is progressive loss of cognitive function that significantly interferes with everyday life (dementia). The disease can include the following types of symptoms:14 Movement disorders Parkinsonism) including tremors, rigidity, slowness of movement, difficulty walking and postural instability Cognitive impairment Confusion, visual reasoning, executive decision making, short term memory loss Neuro-psychiatric Visual hallucinations, delusional thinking, paranoia, sleep disturbances e.g. REM Sleep Behaviour Disorder Behavioural Sudden fluctuations of attention and alertness, sometime referred to a delirium Mood Depression, apathy, anxiety, agitation Autonomic Blood pressure control, temperature regulation, swallowing, fatigue, bladder and bowel function There is no cure to arrest the progression of DLB. The drugs that are used to manage symptoms have all been developed for other disorders, including AD, PD and a number of psychiatric conditions. KEY ISSUE #1 : DIAGNOSIS Health professionals, especially primary health physicians, struggle to correctly diagnose DLB. For a disease that only recently was clinically defined enough to permit a diagnosis being made this is not surprising. A key issue in regards to the management of DLB is both elevated incidences of misdiagnosis as well as under-diagnosis. Delays in a timely and accurate diagnosis often result in lost opportunities for earlier medical and social interventions for those with DLB. These lost 14 Lewy Body Dementia Association (LBDA), Lewy body dementia: the state of the science, viewed online 22 August 2017, <https://www.lbda.org/sites/default/files/2016_lbd_state_of_the_science_w hite_paper.pdf>. A SENSE OF SELF 10 opportunities of care and support quickly erode into the quality of life of those living with DLB as well as carer experiencing burn-out. As is the case with so many others aspects of DLB, and other similar dementia related illnesses, the diagnostic issues are complex and multifaceted. People with DLB will often report that they will have seen multiple specialists before a correct diagnosis is determined on average some eighteen months after the first visit to a doctor. T HE PROBLEM OF MISDIAGNOSIS Currently the only definitive method of diagnosing DLB is via a post-mortem and the staining of brain tissue. Clearly, this is too late for clinicians, patients and carers. Prior to death, a differential diagnosis is obtained by asking the question “What else could it be”? In the case of DLB there are two other likely alternative answers to this question: Parkinson’s Disease and Alzheimer’s Disease. These two illnesses share a significant overlap in symptoms, particularly early in disease progression. Consequently, people with DLB are frequently misdiagnosed with one of these illnesses in up to 78% of instances15. As has previously been discussed, a contributing factor lies in the order that various DLB symptoms may be experienced, and reported. Some people with early DLB will come to their doctor and report that they 15 Galvin, J, Duda, J, Kaufer, D, Lippa, C, Taylor, A & Zarit, S 2009, 'Lewy Body Dementia: The Caregiver Experience of Clinical Care', Neurology, vol. 72, no. 11, pp. A476-A476. A SENSE OF SELF 11 have may be having a problem, predominantly, with their movements. This group of DLB people may not experience cognitive problems until later, if at all. In this instance, a clinician is likely to make a diagnosis of PD, and understandably so. Just like with DLB, there is no objective test for PD: it too is diagnosed by making an observation of symptoms. Studies have frequently shown 15%-25% of people with a PD diagnosis are misdiagnosed 1617. Other people with DLB, however, present with problems being experienced through cognitive impairment resulting in a diagnosis of AD, which is also another illness that lacks any type of test to conclusively diagnosis it. Consequently, people with AD are also frequently misdiagnosed in a similar fashion18. The situation is further complicated by post-mortem revelations that have shown that a significant proportion of people with DLB pathology also had AD pathology19, with some studies demonstrating rates as high as sixty percent of people with DLB having mixed pathology with AD20. The diagnostic situation is this: three similar illnesses, all with overlapping symptoms, that follow a unique course with each person who is offered a provisional diagnosis based on the observation of clinical symptoms prior to death and a confirmed diagnosed only post-mortem. The difficulty in making a diagnosis of DLB is further complicated by the fact that there is a very high degree of subjective variability in what symptoms are experience, in the order of their appearance and their degree of impairment that they cause. Consequently, people with DLB are going to experience the disease uniquely, making the task of objective diagnosis all the more difficult. 16 Jellinger, KA 2003, 'How valid is the clinical diagnosis of Parkinson's disease in the community?', Journal of Neurology, Neurosurgery and Psychiatry, vol. 74, no. 7, pp. 1005. 17 Joustra, J, Gardberg, M, Röyttä, M & Kassinen, V 2014, ‘Diagnostic accuracy of parkinsonism syndromes by general neurologists’. Parkinsonism & Related Disorders, vol. 20, no. 8, p. 840. 18 Qian, W, Schweizer, T, Munoz, D & Fischer, CE 2016, 'Misdiagnosis of Alzheimer’s Disease: inconsistence between clinical diagnosis and neuropathological confirmation’, Alzheimer's & Dementia: The Journal of the Alzheimer's Association, vol. 12, no. 7, pp. 293. 19 Alzheimer’s Association 2017, Mixed Dementia, viewed on 8 November 2017, <https://www.alz.org/dementia/mixed-dementia-symptoms.asp>. 20 Barker, WW, Luis, AC, Kashuba, GA, Luis, GM, Harwood, GD, Loewenstein, GD, et al 2002, 'Relative Frequencies of Alzheimer Disease, Lewy Body, Vascular and Frontotemporal Dementia, and Hippocampal Sclerosis in the State of Florida Brain Bank', Alzheimer Disease & Associated Disorders, vol. 16, no. 4, pp. 203-212. A SENSE OF SELF 12 T HE PROBLEM CAUSED BY LOW AWARENESS OF DLB A correct diagnosis is difficult to come by because doctors, patients, and carers, together, often do not have even a basic awareness of DLB. Many people associate dementia with the word ‘Alzheimer’s’. If the presenting symptoms do not correlate with typical AD then ‘dementia, not further described’, becomes the diagnosis and the common identifier. The problem, of course, is that dementia is not an illness, per se, but a symptom of some other pathology. It is easy to imagine that a busy health professional is not going to be able to easily diagnose a condition they are not familiar with, especially in a ten to fifteen-minute consult. Primary health physicians have reported they lack knowledge of support services in the community, and that they have low confidence in their diagnostic and management skills of conditions that include behavioural problems as symptoms, such as dementia21. In addition, patients and carers are not likely to mention, for example, sleep disturbances or behavioural problems (key DLB symptoms) when discussing problems with thinking or difficulties with movement. Furthermore, studies have shown that few in the general community have an appreciation of the distinction between normal age-related cognitive decline and dementia. The combination of little awareness by doctors and family/friends create a situation where misdiagnosis becomes highly likely. This lack of awareness, in fact, is sometimes described by some as being a ‘symptom’ of DLB, that is, being misunderstood and a significant delay in receiving the correct diagnosis. The best way to appreciate why increasing DLB aware is so critical can be best summarised can be found in the epithet of The Lewy Body Society (a registered charity in England and Wales): “ The more people who know, the fewer people who suffer ” 21 Galvin, J, Duda, J, Kaufer, D, Lippa, C, Taylor, A & Zarit, S 2009, 'Lewy Body Dementia: The Caregiver Experience of Clinical Care', Neurology, vol. 72, no. 11, p. A476. A SENSE OF SELF 13 N EW C LINICAL D IAGNOSTIC C RITERIA One of the inherent problems of DLB diagnosis, as has been discussed above, is inherent inaccuracies associated with diagnosing neurodegenerative diseases like DLB, AD and PD with clinical observations alone. Using the established DLB clinical guidelines has been demonstrated to have high specificity (i.e. they can differentiate between diseases) but low sensitivity (i.e. the illness has to be significantly advanced before it can be detected)22. The detection of DLB sooner remains an elusive goal, as does detection of the DLB in the prodromal (before the manifestation of symptoms) stage. Previously, the criteria were those used from DLB 2005 consortium23. In the middle of 2017 a new set of clinical criteria for the diagnosis of DLB were published by the same consortium of researchers24. These are as follows: Dementia is required. Cognitive impairment in attention, reasoning and problem solving, visuospatial skills. with At least two of the following: • • • • Delirium-like fluctuating cognition. Unpredictable changes in thinking, attention and alertness Repeated visual hallucinations. A common DLB symptom occurring about 80% of the time) REM Sleep Behaviour Disorder. Sometimes appearing up to a decade or more before the onset of DLB. Parkinsonism, specifically slowed movements, tremor when limbs at rest, and muscle rigidity. 22 Nelson, P, Jicha, G, Kryscio, R, Abner, E, Schmitt, F, Cooper, G, et al 2010, 'Low sensitivity in clinical diagnoses of dementia with Lewy bodies', Journal of Neurology, vol. 257, no. 3, pp. 359-366. 23 McKeith IG, Dickson DW, Lowe J, et al. 2005, ‘Dementia with Lewy bodies: diagnosis and management: third report of the DLB Consortium,’ Neurology, vol. 65, no 1, pp. 1863–1872. 24 Mckeith, I, Boeve, B, Dickson, D, Halliday, G, Taylor, -P, Weintraub, D, et al 2017, 'Diagnosis and management of dementia with Lewy bodies Fourth consensus report of the DLB Consortium', Neurology, vol. 89, no. 1, pp. 88100. A SENSE OF SELF 14 Of note, is the fact that DLB patients, in particular, are extremely sensitive to many antipsychotic medications that are used to treat the neuropsychiatric symptoms of the disease. The adverse reactions can actually worsen the symptoms and in a few circumstances, they have caused death. This makes managing the behavioural side of DLB particularly challenging. REM S LEEP B EHAVIOUR D ISORDER In 2005, REM Sleep Behaviour Disorder (RBD) was noted as being highly suggestive of the presence of DLB pathology. In the 2017, DLB clinical criteria, RBD has now been recognised as a core feature of DLB diagnoses, which is not surprising given how rare this disorder is and how commonly it occurs in people with DLB (about 80% of DLB diagnoses feature sleep disturbance caused by RBD) First described in 1986, RBD, as its names describes, manifests itself as a disturbance of sleep that occurs during REM sleep (stage 5 sleep). It is characterised by two primary symptoms: unusually graphic and disturbingly violent dreams and violent behaviours that are the acting out of those dreams. Diagnosis occurs in an overnight sleep study where it is indicated by the loss of muscle atonia that normally occurs during REM sleep thereby allowing them to act their dreams25. RBD is usually noticed either by a bed-partner or in a sleep study, often while undertaking a diagnosis for something else. The person with the disorder is usually unaware of the behaviour, save only when either their partner or they themselves are injured by the violent dream enactment behaviour itself. The prevalence of RBD is not well understood. A recent (2015) population based study estimated the prevalence of RBD to be 7%26. Initially, RBD was thought to be idiopathic however a growing body evidence suggests that a person with RBD has a very high risk of later developing neurodegenerative illnesses. In a recent UK study, the risk of neurodegenerative pathology was found to be 25 Mahowald, MW, Schenck, CH 2011, ‘REM sleep parasomnias’, in MH Kryger, T Roth, C Dement (eds.), Principles and Practice of Sleep Medicine, 5th edn, Elsevier-Saunders, Philadelphia, 1083e1097. 26 Mahlknecht, P, Seppi, K, Frauscher, B, Kiechl, S, Willeit, J, Stockner, H, et al 2015, 'Probable RBD and association with neurodegenerative disease markers: A population-based study', Movement Disorders, vol. 30, no. 10, pp. 1417-1421. A SENSE OF SELF 15 • • • 33% at five years post RBD diagnosis, 75% at ten years, and 90% at fourteen years 27. The most frequent neurodegenerative illness that was diagnosed post RBD diagnoses was DLB, then PD and MSA. Essentially what this means is that nearly everyone who develops RBD will going to develop a neurodegenerative disease and most likely the diagnosis will be DLB. Correspondingly, current estimates indicate that nearly 75-80% of DLB patients will develop RBD post DLB diagnosis28. It follows then, that in the presence of other diagnostic criteria, an RBD diagnosis has the capacity to strongly confirm the presence of current DLB pathology. It is has become apparent, then, that RBD can also be, and should be, used as a highly predictive DLB screening mechanism. One of the inherent difficulties in the diagnosis of RBD is that a sleep study is necessary to confirm it. This is expensive, time consuming and often not of practical utility, noting that this procedure is available only in a specialised sleep laboratories. As interest in RBD has grown, particularly with its association to DLB and PD, a number of screening instruments have been developed to make a provisional RBD diagnosis and thereby identify the patients for whom an overnight sleep study would be recommended. Two instruments are commonly used for RBD screening: the thirteen question RBD Screening Questionnaire (RBDSQ)29 and the RBD Hong Kong scale (RBD-HK)30. Their 27 Iranzo, A, Fernández-Arcos, A, Tolosa, E, Serradell, M, Molinuevo, JL, Valldeoriola, F, et al. 2004, 'Neurodegenerative disorder risk in idiopathic REM sleep behaviour disorder: study in 174 patients', PLoS ONE, vol. 9, no. 2, pp. e89741. 28 Murray, EM, Ferman, JT, Boeve, FB, Przybelski, AS, Lesnick, GT, Liesinger, MA, et al 2013, 'MRI and pathology of REM sleep behaviour disorder in dementia with Lewy bodies', Neurology, vol. 81, no. 19, pp. 1681-1689. 29 Marelli, S, Rancoita, P, Giarrusso, F, Galbiati, A, Zucconi, M, Oldani, A, et al 2016, 'National validation and proposed revision of REM sleep behavior disorder screening questionnaire (RBDSQ)', Journal of Neurology, vol. 263, no. 12, pp. 2470-2475. 30 Li, S, Wing, Y, Lam, S, Zhang, J, Yu, M, Ho, C, et al 2010, 'Validation of a new REM sleep behavior disorder questionnaire (RBDQ-HK)', Sleep Medicine, vol. 11, no. 1, pp. 43-48. A SENSE OF SELF 16 length, however, make them unsuitable for inclusion in large scale epidemiologic surveys. A one question adaption of these two instruments31 (RBD1Q) has recently demonstrated that it has equivalent, or better, accuracy and sensitivity than RBDSQ and RBD-HK. The question that is asked is “Have you ever been told, or suspected yourself, that you seem to ‘act out your dreams’ while asleep (for example, punching, flailing your arms in the air, making running movements, etc.)?” There are two limitations about these three RBD screening tools: Firstly, screening tools cannot replace a video-polysomnogram that can measure any loss of REM sleep muscle atonia, which is the only current method of RBD diagnosis. Secondly, the utility of these screening tools outside of the cohort of patients in sleep clinics (who are likely to be knowledgeable of their condition) remains unknown. Notwithstanding this, the RBD1Q certainly possesses the potential utility for inclusion in large scale epidemiological studies which would provide a mechanism to more accurately determine the prevalence of RBD and in so doing increase our knowledge of the risk of DLB in the general population. Further studies along these lines will more be able to clarify the link between RBD and DLB, with a view to assessing what environmental, lifestyle or medical interventions might mitigate the onset of future DLB. A copy of the REM Sleep Behaviour Screening Questionnaire has been placed in Appendix A, at the end of this report. IMPROVING DLB D IAGNOSIS: ALBA S CREENING INSTRUMENT (ASI) Up until now, the only methods availability to suggest the presence of DLB were general dementia screening tools. However, earlier this year a new screening instrument was published with high specificity and high sensitivity for DLB:32 the ALBA Screening Instrument (ASI). The ASI has been proven useful in detecting DLB quite early on in the 31 Postuma, RB, Arnulf, I, Hogl, B, Iranzo, A, Miyamoto, T, Dauvilliers, Y, et al 2012, 'A single-question screen for rapid eye movement sleep behavior disorder: A multicenter validation study', Movement Disorders, vol. 27, no. 7, pp. 913-916. 32 Garcia, BM, Fernandez, M, Ojea, QM, Rojas, J, Garcia, BM, Bogliotti, E, et al 2017, 'ALBA Screening Instrument (ASI): A brief screening tool for Lewy Body Dementia', Archives of Gerontology and Geriatrics, vol. 70, pp. 67-75. A SENSE OF SELF 17 progression of the disease. It also has the advantage of being able to be easily administered by both medical and non-medical staff. Published in mid-2017 by the Lewy Body Association of Argentina (ALBA), little can be reported about the ASI, at this stage, except to say that in the research domain it has demonstrated great promise in the earlier and more accurate diagnosis of DLB than has hitherto been possible. The ASI consists of seventeen questions divided into three sections: screening for dream enactment behaviours, visual hallucinations, and cognitive symptoms. The instrument can be administered by non-medically trained people in about ten minutes. The ASI was validated against the 2005 clinical criteria for DLB33. Consequently, in the light of the new diagnostic criteria outlined above, the ASI will need further validation against the 2017 clinical standards. However, noting that a factor analysis of the ASI showed that its focus on screening for REM sleep disorders was very strong it is highly likely that the ASI will retain its validity in the new standard. TABLE 2: ASI SCORES. (OTHER DEMENTIAS INCLUDE ALZHEIMER’S DISEASE AND VASCULAR DEMENTIA) These results34 demonstrate that the ASI has good specificity in discriminating DLB from other forms of dementias as well as mild cognitive impairment that is a normal age-related non-pathological condition. 33 McKeith, I, Dickson, D, Lowe, J, Emre, M, O’Brien, J, Feldman, H, et al. 2005, Diagnosis and management of dementia with Lewy Bodies: third report of the DLB Consortium, Neurology, vol. 65, no. 12, pp. 1863–1872. 34 Garcia, BM, Fernandez, M, Ojea, QM, Rojas, J, Garcia, BM, Bogliotti, E, et al 2017, 'ALBA Screening Instrument (ASI): A brief screening tool for Lewy Body Dementia', Archives of Gerontology and Geriatrics, vol. 70, p. 72. A SENSE OF SELF 18 A copy of the ASI has been placed in Appendix B at the end of this report. IMPROVING DLB D IAGNOSIS: T HE L EWY B ODY C OMPOSITE R ISK S CORE The Lewy Body Composite Risk Score (LBCRS), a screening instrument designed by researchers at Florida Atlantic University, has also been another recent development in the fight to improve DLB diagnosis35. Published in 2015, the LBCRS consists of ten yes/no questions. Four of the questions aim to detect motor symptoms characteristic of DLB. The remaining six questions focus on DLB non-motor symptoms. Impressively, the LBCRS was able to significantly discriminate between AD (the most common misdiagnosis) and DLB with the following results:36 Visual hallucinations Delusional misidentifications REM Sleep Behaviour Disorder Cognitive fluctuations AD 8.4 % 1.1 % 5.3 % 43.9 % DLB 47.8 % 34.8 % 41.5 % 92.2 % A copy of the LBCRS can be located in Appendix C, at the end of this report. *** The two DLB screening tools discussed in this paper not only provide mechanisms to more quickly and accurately diagnose DLB, but also provide hope to those experiencing the disease that early diagnoses will result in the more accurate interventions being able to be used and with them significant improvements in quality of life, especially early on in the disease progression. Furthermore, the high discrimination capacity of both these instruments can mean that candidates for clinical trials and inclusion on a DLB register will be more easily identified. 35 Galvin, JE 2015, 'Improving the clinical detection of Lewy body dementia with the Lewy body composite risk score', Alzheimer's & Dementia: Diagnosis, Assessment & Disease Monitoring, vol. 1, no. 3, pp. 316-324. 36 Galvin, JE 2015, 'Improving the clinical detection of Lewy body dementia with the Lewy body composite risk score', Alzheimer's & Dementia: Diagnosis, Assessment & Disease Monitoring, vol. 1, no. 3, p. 319. A SENSE OF SELF 19 KEY ISSUE #2 : CAREGIVING People who are aged, and those with dementia in particular, are one of the most vulnerable groups of people in our community. When a person starts to experience the symptoms of dementia it is nearly always a family member, usually a spouse or the children, who take up the responsibility of caregiving. As the disease progresses, the burden, correspondingly, increases as well, and often intolerably so. The rationale for placing someone in a nursing home is often made on the basis that it is “for their own good”. However, it is often the case that it is not so much the needs of the person with dementia that are of primary concern in this decision-making process but instead the welfare of carer and his or her’s wellbeing. The decision to place an individual in a nursing home is usually made when the family-carer is no longer able to provide adequate care. Unfortunately, this is a common scenario. An important social question here becomes apparent: is it just to place a person with dementia in nursing care, simply out of concern for the carer’s wellbeing? It is unfortunately true that often there are no other alternatives. In our society, the two primary forms of care for people with dementia is either family-based care or institutional care. Particularly in the instance of Dementia with Lewy Bodies, it is also true that, with the exception of where the disease progression is very advanced, often neither of these alternatives are the most optimal. Numerous studies have found that neuropsychiatric symptoms are the most predictive of caregiver burden. The most problematic neuropsychiatric symptoms were disruptive behaviours (e.g. agitation and aggression), delusions, hallucinations, and mood disturbances. These findings have been found regardless of particular dementia diagnoses37. Consequently, in regards to Dementia with Lewy Bodies, the prominence of neuropsychiatric symptoms would predict a higher carer burden than many other forms of dementia. This is, in fact, the case38. T HE BURDENS AND CONSEQUENCES OF DLB CARER STRESS 37 Cheng, S 2017, 'Dementia Caregiver Burden: a Research Update and Critical Analysis', Current Psychiatry Reports, vol. 19, no. 9, pp. 1-8. 38 Leggett, AN, Zarit, S, Taylor, A & Galvin, JE 2011, 'Stress and Burden among Caregivers of Patients with Lewy Body Dementia', Gerontologist, vol. 51, no. 1, pp. 76-85. A SENSE OF SELF 20 A 2010 Lewy Body Dementia Association survey of caregivers found that 90% of people with DLB were unable to attend to even the most basic of daily needs such as shopping and cooking, and over 60% needed assistance with activities of daily living such as personal hygiene. The most common burdens reported were fears about the future of their loved one, balancing caregiving with other personal responsibilities, social isolation, and high levels of uncertainty. It is also of interest to note. that in relation to AD, the commonest form of dementia, DLB caregivers are often younger with a mean age of 55.9 years and were predominantly female.39 This places DLB spousal caregivers in a unique situation within the dementia world in that being younger, and especially female, caregivers may also have the additional responsibilities of providing care and support to either their children and/or their parents40. DLB Family caregivers frequently report experiencing chronic stress with adverse outcomes on their physical, emotional, and mental health. Frequently, over-stressed carers will report decreased well-being, depression, anger, anxiety, and a variety of health and medical problems that are normally exacerbated by experiencing abnormally high levels of stress41. Pearlin, and his colleagues, proposed a highly influential model of care, namely, The Stress Process Model of Caregiving42. In this model, and others that are similar to it, caregiving is understood to be, in itself, a significant stressor. If this stressor is then appraised as a burden then there is a high likelihood of psychological distress developing in the carer. In a 2010 DLB caregiver study43, Leggett and his colleagues found ample support that the Stress Process Model of Caregiving explained the significant burdens of DLB 39 Lewy Body Dementia Association 2010, Caregiver burden in Lewy Body dementias: challenges in obtaining diagnosis and providing daily care, viewed 2 December 2017, <https://www.lbda.org/sites/default/files/caregiver-burden-in-lbd--released-7-12-2010.pdf> 40 Zweig, Y & Galvin, J 2014, 'Lewy body dementia: the impact on patients and caregivers', Alzheimer’s Research & Therapy, vol. 6, no. 2, pp. 21. 41 Leggett, AN, Zarit, S, Taylor, A & Galvin, JE 2011, 'Stress and Burden among Caregivers of Patients with Lewy Body Dementia', Gerontologist, vol. 51, no. 1, pp. 76-85. 42 Pearlin, L, Mullan, J, Semple, S & Skaff, M 1990, 'Caregiving and the Stress Process: An Overview of Concepts and Their Measures', The Gerontologist, vol. 30, no. 5, p. 583. 43 Leggett, AN, Zarit, S, Taylor, A & Galvin, JE 2011, 'Stress and Burden among Caregivers of Patients with Lewy Body Dementia', Gerontologist, vol. 51, no. 1, pp. 76-85. A SENSE OF SELF 21 carers. They further elaborated this approach to asserting that DLB caregiving burden should not be understood as linear form of caring (e.g. accommodating a gradual but systemic loss of memory as in AD). Instead DLB caring should be appreciated multidimensional and contextually driven. Because of the multidimensional nature of DLB symptomology, rather than these stressors being additive it would be the author’s hypothesis that they are instead exponential in their nature. Further research would be required to determine whether or not this was true. So, the emerging picture is that the greatest burden experienced by those caring for people with DLB stems from the behavioural and emotional problems associated with the neuropsychiatric symptoms of this particular disease. This, in turn, compounds the stresses experienced by having to provide support for activities of daily living that are common amongst all carers of people with dementia in general. Another important, but often over-looked, source of stress and burden amongst carers of people with DLB is that of isolation. People who have DLB, and those who care for them, find themselves socially isolated because there is little public awareness about DLB and consequently they often feel misunderstood, alone, and feeling poorly supported.44 The majority of community dementia support groups are often focussed to address support for those caring for AD or PD and consequently are of limited benefit to DLB carers and their unique challenges. Clearly, the caring for a person with DLB is a situation with unique complexities. It is vital that DLB carers receive not only adequate, but also appropriately targeted, support. M EASURING THE BURDENS EXPERIENCED BY DLB CARERS A 2016 study by Svendsboe and colleagues45 examined directly the question of burden of care experienced by people caring for those with DLB. They compared carers of people with AD with carers of those experiencing DLB and found that 55.4% of caregivers generally were 44 Leggett, AN, Zarit, S, Taylor, A & Galvin, JE 2011, 'Stress and Burden among Caregivers of Patients with Lewy Body Dementia', Gerontologist, vol. 51, no. 1, pp. 76-85. 45 Svendsboe, E, Terum, T, Testad, I, Aarsland, D, Ulstein, I, Corbett, A et al 2016, 'Caregiver burden in family carers of people with dementia with Lewy bodies and Alzheimer's disease', International Journal of Geriatric Psychiatry, vol. 31, no. 9, pp. 1075-1083. A SENSE OF SELF 22 spouses, and 44.6% were children. In other words, the only non-institutional caring that was supporting people with DLB was family based. What happens, then, to those people who are single, widowed or divorced, and who have no children, remains a serious social justice question for which there are, at present, no just answers. Even with mild impairment, this group of people are going to end up with a premature nursing home admission, and sometimes that is going to be involuntarily so. Using the Relative Stress Scale (RSS)46, the Svendsboe study they found that carers of people with DLB experienced significantly higher levels of stress when compared with AD and other dementia carers, and represented a particularly vulnerable group of people. Consequently, people who care for those experiencing DLB are at significantly higher risk of burn-out. In an earlier 2007 study, Ulstein and his colleagues demonstrated that the RSS was a very useful tool in measuring dementia carer burden.47 The outcomes of this study recommended the following responses when measuring dementia care burden: those scoring • ≤ 23 (low risk) Should be referred to community based education • ≥23 - ≤30 (intermediate risk) Should be closely monitored for the presence, or onset of, depression and other stressrelated psychiatric disorders. Individual counselling and support group participation is recommended. • >30 (high risk) Referral for psychiatric assessment and treatment The Svendsboe study48 found that 31% of DLB carers were in the intermediate or high-risk group. It is clearly demonstrated, then, that the need for more effective support for DLB carers is pivotal. 46 Greene, JG, Smith, R, Gardiner, M & Timbury, GC 1982, 'Measuring behavioural disturbance of elderly demented patients in the community and its effects on relatives: a factor analytic study', Age and ageing, vol. 11, no. 2, pp. 121. 47 Ulstein, I, Bruun, WT & Engedal, K 2007, 'The relative stress scale, a useful instrument to identify various aspects of carer burden in dementia?', International Journal of Geriatric Psychiatry, vol. 22, no. 1, pp. 61-67. 48 Svendsboe, E, Terum, T, Testad, I, Aarsland, D, Ulstein, I, Corbett, A et al 2016, 'Caregiver burden in family carers of people with dementia with Lewy bodies and Alzheimer's disease', International Journal of Geriatric Psychiatry, vol. 31, no. 9, pp. 1075-1083. A SENSE OF SELF 23 A copy of the RSS has been placed in Appendix E. N URSING H OME A DMISSION One of the greatest stressors that a carer has to deal with is the difficult decision of deciding when nursing home admission become impossible to avoid. People with DLB, and their carers, have already experienced the frustration, isolation, and the consequences that have resulted from the significant lack of professional knowledge about the disease and how to ‘treat’ it. There are constant concerns about overmedication or being inappropriately medicated. A second stressor, in regards to nursing home admission, arises from the testimonies of numerous carers who have reported that many facilities are reluctant to admit a person with an DLB diagnosis. This is because of the behavioural and emotional symptoms that people with DLB exhibit, the disruption it causes to others, and the lack of staff training to manage these complex behavioural and emotional symptoms.49 This is especially so given that many of these behavioural and emotional symptoms cannot be safely managed pharmacologically in people who have DLB. 49 Zweig, Y & Galvin, J 2014, 'Lewy body dementia: the impact on patients and caregivers', Alzheimers Research & Therapy, vol. 6, no. 2, pp. 21. A SENSE OF SELF 24 CHAPTER 3: A NEW MODEL OF CARE This report began by asserting that the majority of older people do not develop dementia. The risks of developing dementia, nevertheless, exponentially increase with age. Noting that Australia has one of the oldest populations in the world, Professor Julian Hughes, in a 2013 seminal discussion paper for Alzheimer’s Australia50, has proposed two important conceptual questions in regards to the future of dementia care in this country. The first of these issues is that if the prevalence of dementia rises with age should it be now be regarded as normal part of aging? Secondly, if the prevalence of dementia increases as we approach death, should we then begin to understand dementia as a terminal condition, as opposed to a chronic health condition as we currently do. TABLE 3: PROJECTED INCREASE OF DEMENTIA IN AUSTRALIA (HUGHES, 2003) Table 3, above, presents some extraordinary data: by 2050 the number of older people with dementia is project to increase from between 90% to 592%. It represents the single most acute crisis in health that our society has ever had to face. In his paper, Hughes explores three philosophies of dementia care: 1. Person-centred care 2. Palliative Care 3. Supportive Care 50 Hughes, J 2013, Models of dementia care: person-centred, palliative and supportive, viewed 29 November 2017, < https://www.dementia.org.au/files/NATIONAL/documents/AlzheimersAustralia-Numbered-Publication-35.pdf>. A SENSE OF SELF 25 P ERSON -C ENTRED C ARE Person-Centred Dementia Care51 has been, especially in recent years, the predominant model of dementia care in this country. In this philosophy four fundamental tenets are affirmed. People with dementia should be: • • • • Valued treated as Individuals Their Perspective must inform our understanding and our responses And their Social life and their relationships must be promoted as the very basis of personhood. (VIPS) A criticism of this approach, however, is that this philosophy does not consider dementia as an end-of-life issue, and, consequently, it provides little guidance in relation to the very difficult decisions regarding nursing home admissions, medical treatments and the withdrawal of medical interventions. P ALLIATIVE C ARE At present, the resources and the responses of our society to the dying are qualitatively more life promoting than for those with a diagnosis of dementia. Slowly, there is an increasing movement to promote to the palliative care model as a response to dementia. In palliative care: • • • The focus is not on dying but on living and living as well as possible Distressing symptoms are actively treated Care is holistic and is a synthesis of physiological, psychological, social and spiritual interventions. A number barriers, however, present themselves in regards to palliative dementia care. Principal among these is that, conceptually, this philosophy was developed largely in response to cancer care. Inherent in this model is the concept that people with cancer often die within months, however with dementia that process can span many years. 51 Brooker, D & Netlibrary, I 2007, Person-centred dementia care making services better, Jessica Kingsley Publishers, London ; Philadelphia. A SENSE OF SELF 26 S UPPORTIVE D EMENTIA C ARE A new and innovative philosophy, Supportive Dementia Care, has been proposed by Professor Hughes as a new and innovative philosophy of dementia care that synthesises both person-centred care and palliative care. Professor Hughes describes Supportive Care as… “… a full mixture of biomedical dementia care, with good quality, person-centred care, psychosocial and spiritual care under the umbrella of holistic palliative care throughout the course of a person’s whole experience of dementia, from diagnosis until death, and for families and close carers, beyond.”52 In its essence, it is a form of multidimensional, biopsychosocial and spiritual dementia care that extends across time, and across the complete course of diseases like DLB. At the end of this report, Appendix F display a table of the proposed components of Supportive Dementia Care. Given the very high degree of subjective differences in dementia care, particularly amongst people with DLB, this model cannot, of course, be a complete description of the facets and possibilities of this new model. At the very least Supportive Dementia Care provides a philosophy of care for people with dementia, particularly those with DLB. It represents a quantum change in our attitude to caring for with dementia; it represents 52 Hughes, J 2010, ‘End-of-life decisions and dementia’, in D Ames, D Burns & J O’Brien (eds), Dementia, 4th edn, Arnold Health Sciences, London, p.301. A SENSE OF SELF 27 an innovative change that is imperative for our health system and finally, Supportive Dementia Care engenders hope, dignity, value and respect. A Daughter’s Decree to Lewy Body A SENSE OF SELF 28 RECOMMENDATIONS ABORIGINAL AUSTRALIANS • Dementia should be incorporated into pre-existing preventative health strategies and programmes aimed at lowering the exposure of Aboriginal people to known dementia risk factors. • A focus on awareness, education and support services for Aboriginal people with dementia and their carers needs to be radically improved, particularly for those residing in remote areas. RESEARCH • It is recommended that future epidemiological studies include the RBD1Q to determine more accurate prevalence data for REM Sleep Behaviour Disorder. • With the use of the new DLB and RBD screening tools, it is recommended that a national LBD register be established. This register can then provide a pool of participants with probable DLB, PDD, and RBD for inclusion in clinical trials. EDUCATION AND AWARENESS • Public awareness campaigns and education programmes to raise the profile of dementia generally and Lewy Body Disease in particular is a critical need. • It is imperative that medical education about and training in DLB diagnosis amongst health professionals, particularly for primary health physicians, be prioritised. DIAGNOSIS • The DLB diagnostic guidelines should be circulated widely amongst South Australia’s Local Health Networks and the various Divisions of General Practice. • Training of primary health care physicians needs to emphasise the clinical relationship between REM Sleep Behaviour Disorder and the later development of neurodegenerative diseases in general and DLB in particular. A SENSE OF SELF 29 CAREGIVING • The majority of dementia related support is addressed toward AD or PD. The report illustrated that the majority of this support network does not address the unique burdens LBD caregivers experience, especially in regards to the neuro-psychiatric symptoms which are a particularly demanding source of stress. It is recommended that specialised support for LBD carers be developed. • In this report, the underlying assumption about caregiving is that this will be provided by either a spouse or by children, especially in the early to moderate levels of dementia progression. Single, widowed and or divorced people without children, and who also have a diagnosis of dementia, represent one of the most vulnerable groups of people in our society today. This group of people face the unjust situation of being admitted to an aged care facility, not because their mental health or quality of life warrant it, but because there are at present no options. Alternatives to nursing home admissions need to be investigated and developed that this social injustice may be addressed. • This reported has also alluded to the fact that when the time of nursing home admission becomes impossible to avoid that the opportunities of professional care are often limited for those with a LBD diagnosis. It is recommended that training in the aged care sector include specialist LBD education and training for its workforce. As our society grows the number of people with LBD is going to increase sufficiently that dedicated LBD facilities will need to be developed in response to this growing need. • In particular the adoption of the model of Supportive Dementia Care is recommended and governments are encouraged to explore this option. • In particular, noting the significant increase we are facing in this country now, it crucial that provisions be made for the construction of more nursing homes that are specifically set up to handle behaviourally challenging clients such as those with DLB • It is strongly recommended that “Supportive Care” become the preferred conceptual model for supporting dementia patients in general, people with DLB and dementia carers in particular. A SENSE OF SELF 30 REFERENCES Alzheimer’s Association 2017, Mixed Dementia, viewed on 8 November 2017, <https://www.alz.org/dementia/mixed-dementia-symptoms.asp>. 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Murray, EM, Ferman, JT, Boeve, FB, Przybelski, AS, Lesnick, GT, Liesinger, MA, et al 2013, 'MRI and pathology of REM sleep behaviour disorder in dementia with Lewy bodies', Neurology, vol. 81, no. 19, pp. 1681-1689. Nelson, P, Jicha, G, Kryscio, R, Abner, E, Schmitt, F, Cooper, G, et al 2010, 'Low sensitivity in clinical diagnoses of dementia with Lewy bodies', Journal of Neurology, vol. 257, no. 3, pp. 359-366. Pearlin, L, Mullan, J, Semple, S & Skaff, M 1990, 'Caregiving and the Stress Process: An Overview of Concepts and Their Measures', The Gerontologist, vol. 30, no. 5, pp. 583-594. Postuma, RB, Arnulf, I, Hogl, B, Iranzo, A, Miyamoto, T, Dauvilliers, Y, et al 2012, 'A single-question screen for rapid eye movement sleep behavior A SENSE OF SELF 33 disorder: A multicenter validation study', Movement Disorders, vol. 27, no. 7, pp. 913-916. Qian, W, Schweizer, T, Munoz, D & Fischer, CE 2016, 'Misdiagnosis of Alzheimer’s Disease: inconsistence between clinical diagnosis and neuropathological confirmation’, Alzheimer's & Dementia: The Journal of the Alzheimer's Association, vol. 12, no. 7, pp. 293. Svendsboe, E, Terum, T, Testad, I, Aarsland, D, Ulstein, I, Corbett, A et al 2016, 'Caregiver burden in family carers of people with dementia with Lewy bodies and Alzheimer's disease', International Journal of Geriatric Psychiatry, vol. 31, no. 9, pp. 1075-1083. United Nation 2017, World population prospects the 2017 revision: key findings and advanced tables, UN Department of Economic and Social Affairs: Population Division, New York. World Health Organisation and Alzheimer’s Disease International 2012, Dementia: a public health priority, WHO, Geneva. Ulstein, I, Bruun, WT & Engedal, K 2007, 'The relative stress scale, a useful instrument to identify various aspects of carer burden in dementia?', International Journal of Geriatric Psychiatry, vol. 22, no. 1, pp. 61-67. Zweig, Y & Galvin, J 2014, 'Lewy body dementia: the impact on patients and caregivers', Alzheimer’s Research & Therapy, vol. 6, no. 2, pp. 21. A SENSE OF SELF 34 APPENDIX A: REM SLEEP BEHAVIOUR DISORDER SCREENING QUESTIONNAIRE REM Sleep Behavior Disorder Screening Questionnaire Please answer each question by checking the approriate box NO † I sometimes have very vivid dreams † 2. My dreams often have aggressive or action-packed content † 3. The dream content mostly matches my nocturnal behavior 4. I know that I move my arms or my legs in my sleep 5. When this has happened, I have sometimes (almost) hurt my sleeping partner or myself 6. I experience or have experienced the following phenomena during my dreams: † iss io 1. n YES † † † † † 6.1 speaking, shouting, swearing, laughing loudly † † 6.2 sudden limb movements, “fights” † † 6.3 gestures, sequences of movements that are pointless during sleep, e.g. waving, saluting, shooing away a fly, falling out of bed † † 6.4 things that have fallen down around the bed, e.g. bedside lamp, book, glasses † † 7. At times I'm woken up by my own movements † † 8. On waking up, I can usually remember the content of my dreams well † † 9. My sleep is often disturbed † † 10. I have/have had a disease of the nervous system (e.g. stroke, head trauma, Parkinson's Disease, RLS, narcolepsy, depression, epilepsy, inflammatory disease of the brain). If so, which one? † † t U Re se vie W wC ith o ou py tP er m † Do No Copyright © 2007 International Parkinson and Movement Disorder Society (MDS). All Rights Reserved. RBDSQ - United States/English RBDSQ_AU1.0_eng-US.doc A SENSE OF SELF 35 APPENDIX B: REM SLEEP BEHAVIOUR DISORDER QUESTIONNAIRE – HONG KONG (RBDQ-HQ) A SENSE OF SELF 36 APPENDIX C: ALBA SCREENING INSTRUMENT (This document has been translated from the original Spanish) Neurology Department Cognitive and Behavioral Neurology Section Asociación Lewy Body Argentina ALBA SCREENING INSTRUMENT (ASI) Instructions for administration and evaluation Clinical Questionnaire Ask the questions of the questionnaire as they are written. Ideally, the questionnaire must be conducted in the presence of a family caregiver. Questions 4, 6, 7 and 8 are scored two points if the answer is yes, as long as the information comes from the caregiver. However, if affirmative answers to these questions are from the patients, the score must be “1”. Physical Examination For the assessment of both Items 12 and 13, the presence of tremor must be clear and evident at first sight. If the patient argues that the tremor occurs due to medication or another factor, nevertheless please assign a score of “1”. Cognitive Examination Every cognitive item must be scored “1” or “0” depending on an incorrect or correct performance, respectively, for each item. Item 14 (Category fluency): Score “1” if the patient names less than 12 animals (e.g.11). Item 15 (Inverse Digit Span). Score “0” points for the sequence correctly repeated. (The correct response for this trial is 7-2-8-5). Item 16 (Executive and visuoconstructional Skill: Clock-test). Score: One point is allocated for each of the following three criteria: A SENSE OF SELF 37 Circle (0 points): the clock face must be a circle with only minor distortion acceptable (e.g., slight imperfection on closing the circle); Numbers (0 points): all clock numbers must be present with no additional numbers; numbers must be in the correct order and placed in the approximate quadrants on the clock face; Roman numerals are acceptable but numbers cannot be placed outside the circle contour; Hands (0 points): The patient must draw two hands jointly, indicating the correct time; the hour hand must be clearly shorter than the minute hand; hands must be centred within the clock face with their junction close to the clock centre. A point is assigned for a given element if any of the above-criteria are not met. Item 17 (Visuoconstructional Skill: Pentagon copy) Score: To be acceptable, there must be two figures that appear to be intersecting. At least one of the figures must have five angles. Tremor, rotation, relative size, and symmetry are ignored. Consider only lines that the patient has drawn (ignore any lines from the original model that the patient may have included as part of the copy). If the patient has drawn more than one copy, grade the best copy. A SENSE OF SELF 38 ALBA SCREENING INSTRUMENT (ASI) Assessment Protocol Clinical Questionnaire 1. Do you have, or have you had, any nightmares in recent times that have called your attention? Yes - No /1 2. Do dream images seem vivid to you? (Do they seem very real)? Yes - No /1 3. Are the vivid dreams frequent? Yes - No /1 4. Have you ever woken up and tried searching for a dream character or some object that you were dreaming of? Yes - No /2 5. Have you felt that a person close to you, or a family member that has passed away, or somebody from your childhood is currently in your home? Yes - No /1 6. Have you seen an image of any of these people, even fleetingly, although they are not really there? Yes - No /2 7. Have you ever heard the patient talking or screaming during sleep? (Care giver) Yes - No /2 Yes - No /2 9. Have you experienced forgetfulness in recent times? Yes - No /1 10. Have you had any episodes of confusion, even during a hospital stay? Yes - No /2 7(a) Do you know if you have ever spoken or screamed while sleeping? (Patient alone) 8. Have you ever seen the patient acting out his/her dreams while sleeping? (Punching, flailing of arms, kicking)? (Care giver). 8(a) Do you know if you have ever acted out dreams (Punching, flailing of arms, kicking)? (Patient alone) /1 11. Could you say that your mental status changes from time to time or on different days, some better and some worse? Physical Examination A SENSE OF SELF 39 12. Check for postural tremor, asking patient to stretch hands before him/her and spread the fingers. Yes - No /1 13. Check for rest tremor, asking patient to put hands at rest on lap or table. Yes - No /1 Cognitive Examination Yes - No /1 15. Inverse Digit Span. Ask the patient to repeat the following series in reverse order: 5-8-2-7. Read at a speed of one digit per second. Yes - No /1 16. Executive and Visuoconstructional Skill (Clock-test). “Draw a circular clock. Put in all the numbers and set the time to 10 past 11.” Circle/ Numbers/ Hands /3 Yes - No /1 14. Category Fluency. Say: “I want you to name me as many animals as you can. You will have 60 seconds before I tell you to stop. Do you have any questions?” 17. Visuoconstructional Skill (Pentagon copy). Ask the patient to copy the interlocking pentagons: Total Score: /24 A SENSE OF SELF 40 APPENDIX D: THE LEWY BODY COMPOSITE RISK SCORE (LBCRS) 53 Purpose of Use The Lewy body dementias, composed of two related disorders: Dementia with Lewy bodies (DLB) and Parkinson’s disease dementia (PDD) are a challenge to diagnose, particularly outside of expert centers. One of the great challenges in differential diagnosis of neurodegenerative disorders is attributing clinical symptoms to specific pathologies to guide treatment choices and discuss prognosis and clinical course. While PDD provides a potentially easier route to diagnosis because the cognitive disorder begins in face of an established movement disorder and criteria have defined a mild cognitive impairment (MCI) state, DLB is a more difficult entity to diagnose with delays in diagnosis approaching 18 months leading to significant burden to patients and caregivers. Patients with DLB are often misdiagnosed. While consensus criteria for DLB have excellent specificity (79-100%), there is no standardized way to assess or operationalize many of the cognitive and behavioural symptoms which markedly decreases sensitivity in clinical practice (range 12-88%). We developed the Lewy Body Composite Risk Score (LBCRS) to improve the ability to detect DLB and PDD in clinic and research populations and increase the likelihood of determining whether Lewy bodies are contributing pathology to the cognitive diagnosis. The LBCRS was derived from clinical features in autopsy-verified cases of healthy controls, Alzheimer’s disease (AD), DLB, and PD with and without dementia. The LBCRS was tested in a consecutive series of 256 patients compared with the Clinical Dementia Rating and gold standard measures of cognition, motor symptoms, function, and behaviour. The LBCRS increases diagnostic probability that Lewy body pathology is contributing to the dementia syndrome and should improve clinical detection and enrolment for clinical trials. Administration and Scoring Guidelines The questions are completed by a clinician after interview with patient and caregiver and a complete physical and neurological exam. The operationalization of physical findings as being present for at least 6 months or symptoms occurring at least 3 times over the past 6 months permitted the scoring of the LBCRS by totalling the sum of signs and symptoms rated as present. In the context of a patient with cognitive impairment, the LBCRS can increase the probability that Lewy bodies are a significant contributor to the clinical diagnosis. The LBCRS has 10 Yes/No questions; 4 questions Galvin, JE 2017, The Lewy Body Composite Risk Score (LBCRS), viewed 17 November 2017, <http://med.fau.edu/research/Lewy%20Body%20Composite%20Risk%20Scor e%20Form%20and%20Instructions.pdf>. 53 A SENSE OF SELF 41 cover motor symptoms while 6 questions cover non-motor symptoms. The clinician rates the presence or absence of physical signs and can elicit information from either the patient or caregiver regarding the presence or absence of symptoms. Interpretation of the QDRS A screening test in itself is insufficient to diagnose a dementing disorder. The LBC RS is, however, quite sensitive to suggesting that there is a high probability that Lewy bodies are a contributing pathology to the underlying cognitive decline either as a single pathology or as a mixed dementia. The LBCRS discriminates DLB, PDD and MCI due to Lewy body disease from other forms of cognitive impairment. The LBCRS is scored on a continuous scale with a range of 0 - 10. Based on receiver operator characteristic curves from 265 individuals included in the development and validation samples, LBCRS scores differentiate with the following cut - points: Non-Lewy Body Case Probable Lewy Body Case 0- 2 3 - 10 Using the cut-off of 3 or greater, the LBCRS was able to discriminate: DLB vs. AD Area under the curve Sensitivity Specificity Positive Likelihood ratio Negative Likelihood ratio 0.94 (0.90 – 0.97) DLB vs. any dementia 0.94 (0.91 – 0.98) MCI DLB vs MCI AD 0.96 (0.91 – 1.0) 94.2 78.2 4.1 97.9 86.1 7.0 100 72.9 3.2 0.08 0.02 0.0 A SENSE OF SELF 42 LBCRS Permission Policy James E. Galvin, MD, MPH grants permission to use and reproduce The Lewy Body Composite Risk Score, also referred to as the “LBCRS”, without modification or editing of any kind solely for (1) clinical care purposes, defined as a physician’s use of the LBCRS for non-research patient care services, and (2) non-commercial research, defined as investigator-initiated clinical research that is not funded or supported, in whole or in part, by any for-profit entity (collectively, the “Purpose”). The Purpose specifically excludes any use, reproduction, publication, and/or distribution of the LBCRS for any other reason or purpose, including without limitation (a) the sale, distribution, publication, or transfer of the LBCRS for any consideration or commercial value; (b) the creation of any derivative works of the LBCRS, including translations thereof; (c) the use of the LBCRS as a marketing tool for the promotion or sale of any drug; (d) incorporation of the LBCRS in an electronic medical record application software; and/or (e) any use of the LBCRS in connection with research or clinical trials that are supported, in whole or in part, by any for-profit entity. All copies of the Lewy Body Composite Risk Score (LBCRS) should include the following notice: "Reprinted with permission. Copyright 2015. Lewy Body Composite Risk Score: A Rapid Method to Improve the Clinical Detection of Lewy Body Dementia is a copyrighted instrument of James E. Galvin. All Rights Reserved." Individuals or corporations intending to use the LBCRS for any use other than the Purpose stated above, including clinical trial or commercial purposes, must obtain Dr. Galvin’s prior written permission. Dr. Galvin is employed by the Charles E. Schmidt College of Medicine, Florida Atlantic University. Please contact James E. Galvin, MD, MPH (galvinj@health.fau.edu) for more information. Copyright 2015: The Lewy Body Composite Risk Score is a copyrighted instrument of James E. Galvin. All Rights Reserved. A SENSE OF SELF 43 LEWY BODY COMPOSITE RISK SCORE Please rate the following physical findings being present or absent for the past 6 months and symptoms as being present or absent for at least 3 times over the past 6 months. Does the patient… YES NO Have slowness in initiating and maintaining movement or have frequent hesitations or pauses during movement? Have rigidity (with or without cog-wheeling) on passive range of motion in any of the four extremities? Have a loss of postural stability (balance) with or without frequent falls? Have a tremor at rest in any of the four extremities or head? Have excessive daytime sleepiness and/or seem drowsy and lethargic when awake? Have episodes of illogical thinking or incoherent, random thoughts? Have frequent staring spells or periods of blank looks? Have visual hallucinations (seeing things not really there)? Appear to act out his/her dreams (kick, punch, thrash, shout or scream)? Have orthostatic hypotension or other signs of autonomic insufficiency? TOTAL SCORE Copyright 2015 The Lewy Body Composite Risk Score, James E. Galvin A SENSE OF SELF 44 APPENDIX E: RELATIVE STRESS SCALE (RSS) Never Seldom Sometimes 1. Do you ever feel you can no longer cope with the situation 2. Do you ever feel you need a break? 3. Do you ever get depressed by the situation? 4. Has your own health suffered at all? 5. Do you worry about accidents happening too ..….? 6. Do you ever feel that there will be no end to the problem? 7. Do you find it difficult to get away on holiday? 8. How much has your social life suffered? 9. How much has the household routine been upset? 10. Is your sleep interrupted by……? 11. Has your standard of living been reduced? 12. Do you ever feel embarrassed by……? 13. Are you at all prevented from having visitors? 14. Do you ever get cross or angry with……? 15. Do you ever feel frustrated at times with……? Greene, JG, 1982 A SENSE OF SELF 45 Often Always APPENDIX F: SUPPORTIVE CARE Hughes, J 2013 A SENSE OF SELF 46