Hospitalisasi

Respiratory Medicine 136 (2018) 98–110
Contents lists available at ScienceDirect
Respiratory Medicine
journal homepage: www.elsevier.com/locate/rmed
Review article
Airway clearance techniques in neuromuscular disorders: A state of the art T

review
Michelle Chatwina,∗, Michel Toussaintb, Miguel R. Gonçalvesc, Nicole Sheersd, Uwe Melliese,
Jesus Gonzales-Bermejof, Jesus Sanchog, Brigitte Faurouxh, Tiina Anderseni, Brit Hovj,
Malin Nygren-Bonnierk, Matthieu Lacombel, Kurt Pernetb, Mike Kampelmacherm,
Christian Devauxn, Kathy Kinnetto, Daniel Sheehanp, Fabrizio Raoq, Marcello Villanovar,
David Berlowitzd, Brenda M. Morrows
a
Academic and Clinical Department of Sleep and Breathing and NIHR Respiratory Biomedical Research Unit, Royal Brompton & Harefield NHS Foundation Trust, Sydney
Street, London, UK
b
Centre for Home Mechanical Ventilation and Specialized Centre for Neuromuscular Diseases, Inkendaal Rehabilitation Hospital, Vlezenbeek, Belgium
c
Noninvasive Ventilatory Support Unit, Pulmonology Department, Emergency and Intensive Care Medicine Department, São João University Hospital, Faculty of Medicine,
University of Porto, Portugal
d
Institute for Breathing and Sleep and Victorian Respiratory Support Service, Austin Health, Melbourne, Australia
e
Departement of Pediatric Pulmonology and Sleep Medicine, Cystic Fibrosis Center Essen, University of Essen, Germany
f
Sorbonne Université, UPMC Univ Paris 06, INSERM, UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique, AP-HP, Groupe Hospitalier Pitié-Salpêtrière
Charles Foix, Service de Pneumologie et Réanimation Médicale (Département "R3S"), Paris, France
g
Respiratory Care Unit, Respiratory Medicine Department, Hospital Clinico Universitario, Valencia, Institute of Health Research INCLIVA, Valencia, Spain
h
Pediatric Noninvasive Ventilation and Sleep Unit, Necker University Hospital, Paris, Paris Descartes University, Paris Research Unit INSERM U 955, Team 13, Creteil,
France
i
Norwegian Centre of Excellence for Home Mechanical Ventilation, Thoracic Department and Department of Physiotherapy, Haukeland University Hospital, Bergen
Norway, Department of Clinical Science, Medical Faculty, University of Bergen, Bergen, Norway
j
Dept of Peadiatric Medicine, Oslo University Hospital, Oslo, Norway and Norwegian Centre of Excellence for Home Mechanical Ventilation, Haukeland University
Hospital, Bergen, Norway
k
Department of Neurobiology, Care Sciences and Society, Division of Physiotherapy, Karolinska Institutet, Functional Area Occupational Therapy and Physiotherapy,
Allied Health Professionals Function, Karolinska University Hospital, Stockholm, Sweden
l
Adult Intensive Care Unit, Raymond Poincaré Hospital (AP-HP) Garches, France
m
Home Ventilation Service, University Medical Centre Utrecht, Utrecht, The Netherlands
n
Direction des Actions Médicales, Paramédicales et Psychologiques, Association Française Contre Les Myopathies-Téléthon, 91000 EVRY, France
o
Parent Project Muscular Dystrophy, 401 Hackensack Ave 9th Floor, Hackensack, NJ 07601, United States
p
Assisted Breathing Center, Women and Children's Hospital of Buffalo Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, The State University of
New York, United States
q
Respiratory Unit, Neuromuscular OmniCentre (NeMO), Neurorehabilitation, University of Milan, Niguarda Hospital, Milan, Italy
r
Neuromuscular Rehabilitation Unit, Nigrisoli Hospital, Viale Ercolani 7/b - 40125, Bologna, Italy
s
Department of Paediatrics and Child Health, University of Cape Town, Klipfontein Rd, Rondebosch, Cape Town, South Africa
A R T I C L E I N F O A B S T R A C T
Keywords: This is a unique state of the art review written by a group of 21 international recognized experts in the field that
Non-invasive ventilation gathered during a meeting organized by the European Neuromuscular Centre (ENMC) in Naarden, March 2017.
Tracheostomy It systematically reports the entire evidence base for airway clearance techniques (ACTs) in both adults and
Respiratory failure children with neuromuscular disorders (NMD). We not only report randomised controlled trials, which in other
Neuromuscular disease
systematic reviews conclude that there is a lack of evidence base to give an opinion, but also include case series
Homecare
and retrospective reviews of practice. For this review, we have classified ACTs as either proximal (cough aug-
mentation) or peripheral (secretion mobilization). The review presents descriptions; standard definitions; the
supporting evidence for and limitations of proximal and peripheral ACTs that are used in patients with NMD; as
well as providing recommendations for objective measurements of efficacy, specifically for proximal ACTs. This
state of the art review also highlights how ACTs may be adapted or modified for specific contexts (e.g. in people
∗
Corresponding author. Academic and Clinical Department of Sleep and Breathing, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, United Kingdom.
E-mail address: m.chatwin@rbht.nhs.uk (M. Chatwin).
https://doi.org/10.1016/j.rmed.2018.01.012
Received 15 November 2017; Received in revised form 20 January 2018; Accepted 22 January 2018
Available online 06 February 2018
0954-6111/ © 2018 The Authors. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
M. Chatwin et al. Respiratory Medicine 136 (2018) 98–110
with bulbar insufficiency; children and infants) and recommends when and how each technique should be
applied.
1. Introduction ineffective cough; together with severe bulbar dysfunction, are the
main causes of morbility and mortality in patients with NMD [7–9].
In healthy individuals, mucociliary clearance and cough mechan- Recurrent RTI's lead to further respiratory muscle weakness, with a
isms are effective and efficient in defending against secretion en- resulting vicious cycle of respiratory disease [10,11]. Hypoventilation
cumbrance, but these mechanisms may become ineffective if the sys- and managing secretions are amongst the most important problems
tems malfunction and/or in the presence of excessive bronchial from patients' perspective [12] and present the respiratory phy-
secretions. Mucus is transported under normal circumstances from the siotherapist with unique management challenges in the care of people
lower respiratory tract into the pharynx by cephalad-bias airflow and with NMD. Despite the clear implications, the problem of managing
the mucociliary escalator mechanism [1]. secretions has received little attention in the care of patients with NMD.
An effective cough is essential to clear airway secretions from the Patients with NMD's are living longer [13–15]; and consequently we
more proximal airways [2]. For an effective cough one needs firstly to are seeing more complex ventilator dependent and independent pa-
take a sufficiently deep breath in; the glottis needs to close briefly to tients. Respiratory physiotherapy is an essential part of the multi-
allow an increase in intrathoracic pressure; followed by expulsive disciplinary management of these individuals, but owing to the in-
glottic opening together with abdominal contraction, which results in herent heterogeneity of the condition; the growing number of available
air being forcibly expelled [3]. This cough expiratory airflow can be airway clearance techniques (ACTs) and associated technological de-
measured and is known as peak cough flow (PCF). Individuals with velopments, it is challenging for physiotherapists to understand what
weak or impaired inspiratory and/or expiratory muscles, with or assessments are required and what treatment options are available and
without glottis closure issues (bulbar insufficiency, tracheostomy), will appropriate for people with NMD.
have decreased PCF. As in other chronic disorders, the home organization of patients
Weakness of the inspiratory muscles leads to a progressive decrease with chronic respiratory disorders is challenging and time consuming.
in vital capacity (VC), but the lung volume changes that appear in some The cost and availability of respiratory experts in primary care, the
patients with neuromuscular disorders (NMD) are attributable to a geographical location of patients, lack of engagmenet of general prac-
combination of muscle weakness and alterations of the mechanical tioners (GP) and care coordination may lead to poor care quality and
properties of the lungs and chest wall [4]. Reduced ability to cough organization. Project “Leonardo” investigated the impact of a new care
leads to secretion retention, predisposing to progessive respiratory organization that included a partnership between patients, considered
morbity. Severe bulbar dysfunction and glottic dysfunction most com- here as key members of their own health team, their GP and their
monly occurs in patients with amyotrophic lateral sclerosis (ALS), dedicated care coordinator. This study suggested positive effects in
spinal muscle atrophy (SMA) type 1, other rarer neuromuscular dis- terms of increasing patient health knowledge and autonomy, improved
orders such as x-linked myotubular myopathy and pseudobulbar palsy care collaboration, appropriate resource utilization and readiness to
of central nervous system etiology [5]. Inability to close the glottis and make changes in health behaviours. A similar project worth's in-
vocal cords results in complete loss of the ability to cough and swallow. vestigation in the respiratory care of patients with NMDs [16].
Difficulty swallowing liquids may result in pooling of saliva and mucus In this state of the art review written by an expert group during the
in the pharynx, especially in the valleculae and the pyriform sinuses. 228th European Neuromuscular Centre (ENMC) international workshop
This results in the perception of excessive pharyngeal secretions, similar on ACT's in NMD, we aim to define ACTs using simple, common lan-
to post-nasal drip [6]. guage to help patients and all members of their care team. ACTs will be
Alterations in alveolar ventilation, atelectasis, mucus plugging, and classified into proximal (cough augmentation) and peripheral (secre-
recurrent respiratory tract infections (RTI's), as a consequence of an tion mobilizing) ACTs (Fig. 1). We further aim to provide standard
Fig. 1. Shows the classification of airway clearance techni-

ques that are used in individuals with neuromuscular dis-
ease. Single breaths are defined as a breath given to the
individual by the device followed by them exhaling or
coughing. Stacked breaths are defined as multiple breaths in
without the individual breathing out after the inspiration.
Once the individual is close to their total inspiratory capa-
city they can actively or passively exhale, or cough.
Mechanical insufflation is defined as an insufflation only
provided by a positive pressure device. Mechanical exsuf-
flation is defined as an exsufflation only provided by a
mechanical insufflation-exsufflation (MI-E) device.
99
being able to hold their breath. The “lower end” of a MIC is residual
volume (RV) as per VC. The “upper end” of a MIC is assisted inspiration,
which may be provided using glossopharyngeal breathing (GPB) [19], a
bag valve mask with or without a one way valve (lung volume re-
cruitment bag (LVR) (see Fig. 2), or a NIV device in a volume mode.
With respect to MIC generation, this technique is also delivered by
volume device and is typically not pressure limited or limited to a
pressure of 40cmH2O.
2.2. Peak cough flow (PCF)
Quantitative assessment of cough efficacy in NMD is typically made

by measuring PCF, although in contrast with other spirometric mea-
sures [20], no internationally agreed testing guidelines are currently
available. However, it has been suggested as a reliable measure in SMA
[21]. PCF is typically measured using a hand-held flow meter as per
peak expiratory flow rate monitoring for asthma [17] but may be
measured with various devices including portable spirometers or cali-
brated pneumotachographs. Pneumotachographs have been suggested
Fig. 2. Shows a lung volume recruitment bag (components from Intersurgical, distributed
to be preferable due to their high sampling frequency and greater ac-
via Breas Medical, Stratford up on Avon, UK). Note the one-way valve at the top with
arrows, which allows inspiration only.
curacy [22], although portable spirometers may provide satisfactory
measurements at flows > 270 L/min [23]. PCF measurements from MI-
E devices should only be taken as a trend as measurements are not
definitions (online supplement 1) and recommendations for appropriate calibrated. The Authors agreed with the following definition: PCF is the
and objective outcome measurements of ACTs. Within the above clas- peak expiratory flow measured during cough. PCF may be assisted
sifications, a full description of each ACT, along with its physiological (PCFassisted) or unassisted (PCFunassisted). During PCFunassisted, the patient
effects, evidence-base and limitations will be presented. This state of is instructed to cough from their maximum, unassisted inspiratory lung
the art review also aims to highlight how ACTs may be adapted or volume (i.e. to take a deep breath in prior to coughing). During
modified for specific contexts (e.g. in people with bulbar insufficiency; PCFassisted the patient is instructed to cough from their maximum, as-
children and infants) and recommends when and how each technique sisted inspiratory volume. This flow rate may be obtained from LIC
should be applied. A summary from each studies on the evidence for (PCFLIC) or MIC (PCFMIC). The choice of measurement interface can
ACT's is in online supplements 2, 3 and 4. affect PCF test performance and results. The Authors consensus view is
that PCF testing in NMD should be performed using a facemask.
2. Respiratory measurements for patients with NMD While the literature describing critical cut-off values for cough ef-
ficacy and the relationships of these critical values to clinical outcomes
The literature is overflowing with different terminologies for mea- is incomplete, there are a number of broad, clinically accepted and
sures of efficacy of proximal ACT. The lack of common terminology and often quoted PCF cut-off values. These include an able-bodied, normal
outcome measures limits the ability to compare outcomes and advance adult range of 360–840 L/min, [24]; and evidence that a PCF of >
the understanding and development of these interventions. For pre- 160 L/min is sufficient to eliminate airway debris and secretions fol-
viously defined volumes such as VC, we defer to the American thoracic lowing extubation and tracheostomy removal in adults [25]. Data from
society (ATS)/European respiratory society (ERS) definitions [17]. patients with Duchenne Muscular Dystrophy (DMD) suggests that a PCF
exceeding 270–300 L/min is required when a patient is well in order to
2.1. Insufflation capacity maintain PCF > 160 L/min and effectively clear secretions when un-
well [26]. In the ALS population PCF < 255 L/min could predict the
2.1.1. Lung insufflation capacity (LIC) risk of ineffective spontaneous cough during a respiratory tract infec-
The LIC, measured on exhalation, is the maximum, tolerable, tion (RTI) [27]. Another study reported aPCFunassisted cut-off point of
externally assisted insufflation capacity that does not involve the 2.77 L/s (166 L/min) predicted cough effectiveness [28]. Age adjusted
patient holding their breath [18]. The “lower end” of the LIC is re- PCF values have been published for children from the age of 4–18 years
sidual volume (RV) as per VC. The “upper end” of LIC is assisted old [29] along with normal values for adults [30]. After the age of 12
inspiration, which may be provided using a bag valve mask; non- years, PCF appears to be within the adult range, albeit at the lower limit
invasive ventilation (NIV) (typically volume pre-set ventilator [29]. In clinical practice, cough efficacy with mucus expectoration
mode), the inflation component of mechanical insufflation-exsuffla- classically requires values above 160–200 L/min [31]. The Authors
tion (MI-E), using an MI-E device or intermittent positive pressure consider that these values represent the best-available cut-offs for
breathing (IPPB). clinical use until more robust data is produced. PCF remains the most
common effect outcome measure of ACT in patients with MNDs
2.1.2. Maximum insufflation capacity (MIC) [26,32,33].
The MIC, measured on exhalation, is the maximum, tolerable, ex- Box 1 provides some recommendations for recording PCF, LIC and
ternally assisted insufflation capacity that is dependent on the patient MIC measurements based on this group's expert consensus.
100
Box 1
Recommendations For Measurements.
• PCF should be measured at each clinic review

• LIC and MIC measurements can be made using any device appropriate for the recording of VC
• Either an oro-nasal mask or a mouthpiece with a nose clip may be used for measuring LIC or MIC. The same interface and circuit should be
used for repeated measurements
• Bag valve mask circuits (resuscitation bags), lung volume recruitment circuits and preset volume cycle ventilators should have a pressure
limit to prevent the potential risk of barotrauma
• A pneumotachograph provides the most accurate measure of PCF, especially for patients with PCF < 270 L/min. If unavailable a paediatric
PEF meter is recommended
• PCF measurement should ideally be performed using an oronasal mask interface rather than a mouthpiece
• The same device should be used for repeated and longitudinal measures of PCF
• Values of PCF and volumes provided by MI-E devices should be used as an indicator of improvements or decline associated with the
intervention
3. Proximal airway clearance techniques to increase expiratory airflow. Expiratory assistance may also be
achieved by a self-induced thrust to the abdomen and/or chest from a
Cough is the primary defense mechanism against foreign bodies in stationary object such as a table [35].
the central airways. Proximal ACTs are techniques that aim to augment
the cough by assisting inspiration, expiration or both. They are often 3.1.1.1. Physiological effects. Compression of the abdomen causes a
described as “cough augmentation” techniques, supporting or imitating sudden increase in abdominal pressure; this causes the abdominal
a cough. The primary goal is to clear mucus from the larger airways by contents to push the diaphragm upwards, increasing expiratory airflow.
increasing PCF. Figure 3 presents the flow, volume and pressure profile Similarly, sudden thoracic compression causes air to be rapidly expelled,
of these techniques. with acceleration of airflow towards the mouth. The technique involves
the patient taking a spontaneous, or receiving an assisted, inspiration and
3.1. Assisted expiration at the start of the cough expiratory compression is applied. Care is taken to
ensure that the direction of the compression is in line with the expiratory
The aim of these techniques is to assist the expiratory muscles that chest wall movement, i.e. down and in; with the exception of the
are otherwise incapable of generating sufficient increases in intra-ab- techniques incorporating the Heimlich-type assist, when the abdominal
dominal and intra-thoracic pressure and/or to increase the expiratory pressure is up and in [36].
flow generated during the cough manoeuvre.
3.1.1.2. Limitations of the technique. MAC requires a cooperative
3.1.1. Manually-assisted cough (MAC) patient, good coordination between the patient and care giver, and
A MAC uses either, or a combination of, a manual Heimlich/ab- adequate physical effort and often frequent application by the therapist
dominal thrust manoeuvre and manual costo-phrenic compression [34] or family care giver. It may be ineffective in the presence of severe
Fig. 3. Shows schematic flow, volume and pressure curves

for respiratory patterns in proximal airway clearance tech-
niques (ACT's). Pressures are illustrated as measured at the
mouth and proximal ACT's are illustrated always followed
by a single cough maneuver. The grey shading relates to the
inspiratory component and the white to the expiratory
component. Normal tidal volume (Vt), forced vital capacity
(FVC), peak cough flow(PCF), air stacking (AS), glosso-
pharyngeal breathing (GPB), mechanical insufflation-ex-
sufflation (MI-E) and liters (L).
101
scoliosis [37]. Abdominal compressions should not be used for 1–1.5 h using a bag valve mask to deliver the technique the correct size should
following a meal, however, chest compressions can be used to augment be used for the age and size of the patient (i.e. infant (around
PCF at this time. Chest compression techniques must be performed with 220–360 mls), child (around 650 mls) and adult > 1500 mls) along
caution in the presence of osteoporotic ribs. Unfortunately, since it is with the correct volume to be delivered.
not widely taught to health care professionals, MAC is underutilized
[38]. Limits of effectiveness for the use of MAC in NMD have been 3.2.1.3. Evidence base. Dohna-Schwake and co-workers [46]
reported when PCFunassisted ≥ 140 L/min, VC ≥ 1030 L or maximum investigated the effects of a single breath assisted inspiration with an
expiratory pressure (MEP) ≥ 14cmH2O, using MAC can augment PCF IPPB device. IPPB increased PCF. This effect was similar for both young
to > 180 L/min [34]. In patients with ALS (ALS), In ALS, the predictor (ages 6–10 years) and older patients (aged > 10 years). Trebbia and co-
of ineffective MAC was a MAC PCF below 169 L/min [28]. workers [43] measured VC and PCF during a single breath assisted
inspiration with IPPB, MAC or both. Each technique improved VC and
3.1.1.3. Evidence-base. Increases in PCF with a MAC have been PCF as compared to baseline. Mellies and Goebel [49] performed lung
reported [34,37,39–43]. However, greater effect on PCF has been insufflation using IPPB set to 30 and 40 mbar. The study included NIV
shown by combining inspiratory and expiratory techniques dependent patients. IPPB increased VC and PCF. The highest individual
[18,34,40]. Sancho and co-workers [27] reported in stable ALS PCF was achieved with insufflation at 27 mbar and volume at 924 ml,
patients that mean PCFunassisted 245 ± 87 L/min was required for a which was significantly below the MIC. Contrary to others the authors
MAC to be effective during a RTI. concluded that a submaximal insufflation is ideal for generating the
best individual PCF, even in patients with severely reduced respiratory
3.1.2. Exsufflation alone system compliance [49]. The BTS guideline recommends that assisted
This is when a MI-E device delivers negative pressure (exsufflation) inspiration prior to coughing should achieve MIC and suggests these as
alone via a full-face mask or catheter mount attached to an artificial effective methods of improving cough efficiency, which should be used
airway. when appropriate [50]. However, these guidelines were published
before Mellies and Goebel's study [49].
3.1.2.1. Physiological effects. This aims to increase expiratory airflow
during the cough maneuver. 3.2.2. Stacked breaths assisted inspiration
Stacked breaths assisted inspiration is where the patient has re-
3.1.2.2. Limitations of the technique. This technique has been minimally peated inspirations without breathing out until they reach their MIC.
evaluated and the device is expensive. Various techniques can be used to achieve this (Figs. 1 and 2). Tech-
niques include: GPB, air stacking (AS) via a bag valve mask or a lung
3.1.2.3. Evidence base. Using exsufflation alone has been shown to volume recruitment circuit (this has a one-way valve to restrict ex-
increase PCF in adults with NMD [44] and in patients with ALS [45]. halation) (see Fig. 2) or a preset volume cycled ventilator.
GPB also known as auto or self-air-stacking or frog breathing. It
3.2. Assisted inspiration increases inspiratory capacity (IC) by pumping air into the lung using
the mouth, tongue, pharynx and larynx to compensate for the weakness
Inspiration prior to coughing is limited in weak patients with NMD. of the inspiratory muscles [19]. AS increases inspiratory capacity by
Augmentation of inspiratory lung volumes, through assisted inspira- providing a series of breaths in, without the patient breathing out in-
tion, is associated with increased PCF [46,47]. These techniques are between. The patient is instructed to either take a deep breath in first,
relatively inexpensive methods of cough augmentation. Assisted in- and then the inspiratory capacity is augmented via a series of breaths in
spiration may be either a single breath inspiration (e.g. inspiration, ex- without the patient breathing out, until they feel full of air. Or the
piration, inspiration, expiration) or stacked breath inspirations (e.g. inpatient is passively provided with a series of breaths without breathing
spiration, inspiration, inspiration, expiration). out. Once the patient is close to their total lung capacity (TLC), the
patient is instructed to cough with or without a MAC.
3.2.1. Single breath assisted inspiration
A single breath assisted inspiration is where the patient's inspiratory 3.2.2.1. Physiological effects. As with the single breath techniques, the
VC is augmented via a bag valve mask, NIV (in a preset pressure or stacked breath techniques aim to inflate the respiratory system to the
preset volume mode) or IPPB device with a single breath via an or- maximal desired volume, thereby increasing the inspiratory capacity.
onasal mask or mouthpiece. The objective of this technique is to reach During AS, significant gas compression occurs and absolute lung
LIC. The patient is given a long deep breath in by the chosen device, volumes can be estimated by simultaneous measurements of chest
after which they are instructed to cough (unassisted or with a MAC). wall volume changes, changes in lung volume and pressure variation at
the airways opening [51].
3.2.1.1. Physiological effects. Single breath assisted inspiration provides
a single, sustained inspiratory flow that inflates the respiratory system 3.2.2.2. Limitations of the technique. Glottic function may limit some
to the maximal desired volume. Once this volume has been attained, a techniques, although a one-way valve may mitigate this to some extent.
combination of static recoil and expiratory muscle recruitment provide If the patient is unable to perform stacked breath techniques then a
for an increased expiratory volume or a PCF beyond that which can be single assisted inspiration should be performed.
obtained by an unassisted cough. LIC is an objective, quantifiable,
reproducible measure that (inversely) correlates with glottic integrity 3.2.2.3. Evidence base. In a large group of patients, MIC/VC difference
the technique is indicative of ineffective glottic function [48]. correlated with the difference between PCFunassisted and PCFassisted [47].
The greater the MIC/VC difference, the greater the PCF. The lower the
3.2.1.2. Limitations of the technique. Although the technique can be VC, the greater the percent increase in MIC and PCFassisted [47]. Jenkins
delivered easily via a bag valve mask some centres will deliver the et al. [52], investigated 23 children's ability to learn AS using a LVR
technique via a volume preset ventilator, IPPB device or insufflation circuit (Fig. 2); eight of whom had some degree of learning difficulty.
from MI-E device, which has cost and resources implications. When Only four participants were unable to effectively AS. PCF after AS was
102
also greater in those patients with greatest muscle weakness [52]. In when instructed to cough [60,61]. Setting the MI-E device involves
patients with SMA and congenital muscular dystrophy (CMD), AS has adjustment of the positive and negative pressures, the inspiratory, ex-
also been shown effective, however AS was more effective in the piratory and pause times (seconds) and the inspiratory flow rate (L/
absence of scoliosis [53]. AS was evaluated in patients with DMD, min). One MI-E treatment usually consists of several phases of coughing
where 94.9% of patients could AS and the ability to increase expiratory and rest periods. These cycling periods are repeated several times or
volume was better via AS than with GPB [54]. Maximal insufflations are until secretions are substantially expelled [59]. An additional MAC can
extremely important to increase PCF in adult patients who have VCs be provided during exsufflation (usually in weaker patients).
of < 1500 ml [47]. The acute effects of AS on PCF and chest wall
compartmental volumes were evaluated in patients with ALS and 3.3.2.1. Physiological effects of MI-E. Studies have reported increases in
healthy controls at 45° head up. PCFMIC and chest wall PCF with low pressures [45,62–64] whilst others have reported efficacy
compartmental inspiratory capacity significantly increased in both with high pressures [7,65–69]. Studies evaluating proximal ACT's
groups (P < 0.001) [55]. showed MI-E to increase PCF [39,44,70] with MI-E superior to other
Specific evidence base is also available for GPB. Nygren-Bonnier and techniques [44,45,71] and the greatest change in PCF occurring in the
co-workers [56] evaluated the ability of children with SMA type II to weakest patients [45], with the exception of Lacombe and co-workers
learn GPB. 45% of children learned the technique and increased their [72] who found that in stronger patients MI-E did not produce the
VC and PCF. Bach et al. [54], found GPB could be learned in 27% of greatest change in PCF. One possible explanation for the difference in
patients with DMD. Although GPB was considered inferior to AS in pressures reported could be related to the baseline strength of the
terms of the ability to be taught, both techniques increased lung vo- patient groups. As spontaneous cough strength declines, there may be a
lumes and PCF. The haemodynamic effects of GPB in people with cer- need to increase pressures to improve expiratory flow, however optimal
vical spinal cord injury (SCI) showed that if GPB is performed correctly, MI-E dosage and frequency has not been determined. Fauroux and co-
the risks of clinically significant haemodynamic changes are low, al- workers [70], showed in children, who received insufflation and
though syncope may still occur [57]. The experiences of GPB were exsufflation at different pressure settings without them coughing, an
perceived as a possibility to make a difference in one's life by improving insufflation pressure of +40cmH2O and exsufflation pressures of −40
respiratory function, both immediately and for the future [58]. cmH2O achieved mean PCFs of about 120 L/min. This suggests that
higher pressures may clinically be required in weaker patients. Hov and
3.3. Assisted inspiration and expiration co-workers [73], found by survey that the provision of MI-E to children
in Europe that younger children were set up with lower pressures than
3.3.1. Assisted inspiration combined with MAC older. However, there was wide variation in settings prescribed. Indeed,
A very useful way to assist both inspiration and expiration consists current practice is to start with low pressures and to build up pressure
in combining assisted inspiration (one or multiple breaths) with a MAC. until efficacy is achieved. Titration of settings is often to improve
The combination of those cheap techniques is very common in the audibility of the cough or to an objective increase in PCF measurement
practice [25,34,40,41,50]. on the device.
Sancho and co-workers [74] reported airway closure in adults with
3.3.1.1. Physiological effects. This technique combines the physiological bulbar ALS with exsufflation pressures of −40 cmH2O evaluated with a
effects of inflating the respiratory system to its desired volume with the CT scan. They did not examine what happens with insufflation. An-
compression of the abdomen or chest to increase expiratory airflow in dersen and co-workers [60] recently showed the same phenomenon
conjunction with a greater inspiratory capacity. This therefore has the with video-recorded flexible transnasal fibre-optic laryngoscopy during
capacity to increase PCF further. MI-E in all ALS subjects regardless of bulbar symptoms, but in addition
they observed laryngeal collapse during insufflation in all subjects with
3.3.1.2. Limitations of the technique. As one would expect, combining bulbar symptoms [60]. The implication for clinicians is that bulbar ALS
techniques can increase reliance on carers. The lower limit of patients are unlikely to benefit from high pressures as they have pre-
effectiveness for AS plus MAC was best predicted by VC > 340 mL disposition to upper airway collapse. This has not been reported in
[34]. patients with other NMD.
Bench studies have added to this evidence base, although results
3.3.1.3. Evidence base. Bianchi et al. [35], showed that GPB combined may not be generalizable to clinical practice. High insufflation and
with self-induced thoracic or abdominal thrust was as effective as AS exsufflation (+50 cmH2O to −50 cmH2O) pressures were required
and MAC in wheelchair-dependent patients with NMD. The authors through endotracheal tubes and tracheostomies to produce high ex-
suggest that independently assisted cough via GPB plus table thrust piratory flows. The smaller the tube, the higher the resistance and the
should be utilized where possible [35]. Studies have found the greatest higher the pressures required to generate effective expiratory flows
improvement in PCF occurred when combining assisted inspiration (via [75]. In a paediatric lung model an insufflation time of > 1 s was re-
AS, mechanical insufflation or IPPB) with a MAC [18,40,43] and the quired for equilibration between insufflation pressure and alveolar
greatest change in PCF was in the weakest patients [18]. Therefore, it is pressure. Longer exsufflation time did not significantly change mean
recommended that AS be used with a MAC to achieve the greatest expiratory flows. Higher insufflation and exsufflation pressures both
improvement in PCF. increased mean expiratory flow, but greater exsufflation pressures had
a more substantial increase on mean expiratory flow [76]. One study in
3.3.2. Mechanical insufflation-exsufflation (MI-E) an adult lung model showed set pressures of 40 to −40 cmH2O with an
MI-E devices deliver a deep inspiration to the lungs (insufflation) insufflation time of 3 s, and exsufflation time of 2 s was required to
followed immediately (10 ms) by a deep expiration (exsufflation), by generate an exsufflation flow of 294 L/min [77]. The authors concluded
applying sequentially positive and negative pressure swings via a full- that increasing insufflation times may be more effective than exsuffla-
face mask or catheter mount attached to an artificial airway. The in- tion times in improving expiratory flows.
sufflation aims to “fill” the lungs and the exsufflation aims to “empty” Recommendations for children who use MI-E for airway clearance
the lungs of air. The rapid switch from positive to negative pressures are that they should be given long enough periods of rest during
aims to simulate the airflow changes that occur during a normal cough, treatment sessions to prevent respiratory muscle fatigue due to
potentially assisting secretion clearance [59]. Theoretically, MI-E coughing. Also at the end of a treatment session with MI-E it is im-
compensates both for weak inspiratory and expiratory capacities. It is portant to complete the session with an insufflation to leave an ap-
possible to coordinated the glottic closure and opening to MI-E cycles propriate functional residual capacity [50]. MI-E is reported safe to use
103
in small children in a post op setting, e.g. by gastrostomy surgery [78]. the need for reintubation are necessary. Proximal ACTs and NIV are
Long-term VC was shown to increase with regular use of MI-E, approaches that may be useful to address extubation failure [66].
supporting the suggestion that regular use of MI-E in people with NMD Conventional management of respiratory secretions in patients with
may contribute to the release of thoracic contractures [79]. Kim and co- artificial airways is suctioning via a catheter. With routine suctioning,
workers [41], showed improvements in PCF with MI-E but greater in- secretions in peripheral airways are not directly removed [83]. More-
creases in PCF were attained with the addition of a MAC to MI-E. over, suctioning can lead to serious complications [84]. In contrast,
Newer MI-E devices have the option to add high frequency oscilla- when MI-E is attached to an artificial airway it may clear secretions
tions (HFO) during insufflation, exsufflation or both. Sancho and co- from both lungs along with the central airways. Garstang et al. [85],
workers [69] found that HFO in addition to insufflations, exsufflation found that MI-E through tracheostomy tube was significantly less irri-
and in combination did not have an effect on PCF in medically stable tating, less painful, less tiring and less uncomfortable than endotracheal
subjects with ALS. suctioning in patients with traumatic SCI.
The effects of MI-E via tracheostomy with inflated cuffs were
3.3.2.2. Limitations of the technique. Patient reported complications of compared to suctioning in patient with ALS. MI-E was deemed more
MI-E use in adults are rare and MI-E treatments are usually well effective in eliminating airway secretions than tracheal suctioning.
tolerated [18,32,50,68,70]. However, reported side effects include Pulse oximetry (SpO2), peak inspiratory pressure, mean airway pressure
abdominal bloating, pneumothorax [80], nausea, bradycardia, and work of breathing improved significantly with MI-E sessions.
tachycardia, and abdominal distention [42]. Children have also Patients found MI-E more effective and more comfortable than suc-
reported thoracic wall discomfort and crying and agitation in tioning [86]. Different studies have reported on the effectiveness and
response to treatment with MI-E [64]. security of MI-E in patients, mainly with ALS, on long-term invasive
A major limitation is the cost and/or reimbursement of the devices. mechanical ventilation through a tracheostomy tube, particularly when
There are also discrepancies in the availability in the acute and long- MI-E is applied through un-cuffed tracheostomy tubes [87,88].
term settings. In some middle and low-income countries MI-E devices MI-E is increasingly used in the home management of both adults
are not available. and children with NMD. A survey of patients with NMD using MI-E at
MI-E can be difficult to perform in very young infants who are not home showed 46% used MI-E daily and 27% weekly. One third of pa-
able to have a minimal cooperation and who are not able to relax and tients had used MI-E to resolve a choking episode and 88% agreed that
accept being “insufflated” during the MIC maneuver. Should the tech- home MI-E had improved their/their child's overall respiratory health.
nique be ineffective at this time there is no reason not to try as the child One third reported negative features using MI-E, which were related to
gets older. the size and weight of the device and the requirements to administer
Lacombe et al. [72], suggested that the combination of MI-E to MAC the device [89]. Poor adherence was identified as the major barrier to
is useless in patients whose PCFassisted with an insufflation technique effective use [90]. The positive impacts included greater ability to
and MAC exceeds 5 L/s (300 L/min). In patients with ALS, the predictor manage the child's health, including avoidance of hospital admissions.
of effective MI-E was MI-E assisted PCF of 177 L/min [28]. Negative impacts were greatest for parents who were sole operators of
Andersen and co-workers [60] evaluated upper-airway malfunction the device, including a frequently disrupted lifestyle [91]. Siewers et al.
in patients with ALS during MI-E. Hypopharyngeal constriction during [92], investigated home use of MI-E in ALS. They concluded that health
exsufflation was observed in all subjects, most prominently in patients professionals need to take into account individual and social aspects of
with ALS and bulbar symptoms. This severely obstructed the airflow implementing MI-E in the home environment. Proper instruction and
and limited the efficacy of the treatment. They concluded that in- practical training and confidence in how to use the device, and trust
dividually customized settings can prevent airway obstruction and and continuity among careers are important factors for successful im-
thereby improve and extend the use of non-invasive MI-E. Settings that plementation [92].
can help these patients are triggered insufflation, decreasing the in-
spiratory flows and pressures and allowing a longer insufflation time in 3.4. Recommendations regarding proximal airway clearance
order to allow equilibrium of pressure from the device to the lungs.
The Adult BTS guidelines [93] recommend that MI-E should be
3.3.2.3. Evidence base. Experts suggest that using MI-E in very weak considered in the following: in SCI, if simpler techniques fail to produce
patients is a priority. However, few trials have studied the limits of an adequate result; in bulbar patients who are unable to AS; in any
effectiveness of MI-E, or its efficacy and safety in the long-term [32,50]. patient who remains unable to increase PCF to an effective level with
MI-E in a protocol with MAC, oximetry feedback, and home use of NIV other strategies and where cough effectiveness remains inadequate with
was shown to effectively decrease hospitalizations and respiratory MI-E alone, it is recommended that MI-E be combined with a MAC.
complications and mortality in a program for patients with ALS [67]. The Pediatric BTS guidelines [50] recommend that MI-E should be
Short-term studies [32,44,71,74], or bench studies [75] or feasi- considered in the following: in very weak children; those with bulbar
bility in case reports [62], have suggested that MI-E improves PCF's insufficiency, and those who cannot cooperate with MAC or AS, or in
enough to aid mucus clearance. The addition of MI-E may reduce the whom these methods are not effective [50]. MI-E should ideally be
frequency of pneumonia [64] and the treatment time when added to available in the acute setting in all hospitals that treat children with
MAC [81]. MI-E appeared to be as well tolerated as other cough aug- NMD as an alternative method of ACT, with the purpose of preventing
mentation techniques [32,68,70]. However, these studies did not report deterioration and the need for intubation and mechanical ventilation
on mortality, morbidity, quality of life, or serious adverse events [32]. [50].
When MI-E was compared to AS over a 12-month period there was no Contrary to the above suggestions, a more recent Cochrane review
difference in episodes of RTIs; days of antibiotic; mean duration of reported that more randomised controlled trials were warranted and
symptoms per RTI or hospitalization. However, a major confounder of reported the lack of robust evidence supporting the use of MI-E in
the study was that it was significantly underpowered [82]. people with NMD [32]. Considering the large evidence base outside
Because extubation failure can be a significant problem for patients randomised controlled trials for the use of MI-E, some clinicians believe
with NMD, the use of MI-E in ICU has been evaluated. Strategies that it would be unethical to randomise NMD patients not to receive a MI-E
can prevent the development of respiratory failure after extubation and device in a clinical trial, where MI-E is available as standard practice. In
104
Box 2
Recommendations For The Use Of Mechanical Insufflation-Exsufflation (MI-E).
• MI-E is the treatment of choice for the weaker group of patients with NMD
• Face masks should be used when using MI-E in patients without an artificial airway
• Inspiratory and expiratory timing/pressures should be individualized with progressive build-up of pressure until efficacy is achieved
• Higher expiratory than inspiratory pressures are advisable
• Patients with ALS are likely to benefit from lower pressures, triggered insufflation and longer insufflation time
• MI-E is possible through tracheostomy tubes, with higher pressures for smaller tube diameters
• Complete the session with an insufflation to leave an appropriate functional residual capacity in weaker patients or children
• In ICU, MI-E maybe as a useful technique to prevent re-intubation
• MI-E may be considered in the weaker children with bulbar insufficiency, and those who cannot cooperate with MAC or AS or in whom these
methods are not effective
the context of lower income countries, where access to MI-E devices is failure or intellectual impairment (See Table 1). This review will not include
limited or non-existent, randomised clinical trials might be ethically positive expiratory pressure (PEP) and oscillatory PEP devices as patients
permissible to inform local practice guidelines, and for the purposes of with NMDs generally cannot generate sufficient expiratory flow for the
advocacy. technique to be effective and we therefore do not recommend these devices
Box 2 highlights the recommendations from the authors of this re- for patients with NMD.
view for MI-E.
4.1. Manual techniques
4. Peripheral airway clearance techniques
Manual techniques consist of chest percussion and vibrations or
Peripheral ACTs aim to improve ventilation, loosen secretions and en- shaking. This is performed using a hand, fingers or facemask and is
hance mucus transport from peripheral airways to the central airways (12th generally well tolerated and widely used in babies, small children and
generation of the bronchial tree and above) with higher expiratory than in patients unable to cooperate with therapy. Chest vibrations consist of
inspiratory airflows (called biased expiratory flow) [94]. These include: a rapid extra-thoracic force at the beginning of expiration, followed by
manual techniques (MT), high frequency chest wall oscillations (HFCWO), oscillatory compressions until expiration is complete [96].
or compression (HFCWC), intrapulmonary percussive ventilation (IPV) and
chest wall strapping (CWS). See Figure 4 for the flow, volume and pressure 4.1.1. Physiological effects
profile of these techniques. Peripheral ACT do not necessarily require the The compression and oscillation applied to the chest are believed to aid
patient's co-operation [95]. The use of these techniques is possible in infants, secretion clearance via increasing peak expiratory flow to move secretions
children and adults, even in the presence of a tracheostomy and/or bulbar towards the large airways for clearance via suction or a cough [97].
Fig. 4. Shows schematic flow, volume and pressure curves

for respiratory patterns in peripheral airway clearance
techniques (ACT's). Pressures are illustrated as measured at
the mouth. The grey shading relates to the inspiratory
component and the white to the expiratory component.
Normal tidal volume (Vt), chest wall strapping (CWS), in-
trapulmonary percussive ventilation (IPV), high frequency
chest wall oscillation (HFCWO).
105
Table 1
Practical Aspects of Peripheral ACT's.
Does the patient Is the technique Is it possible to do the Can the Can the Can the technique be Can the technique Length of
need to co- possible when technique in technique be technique be applied to patients be applied to treatment
operate with the patient is conjunction with applied to applied to with tracheostomy patients with (minutes)
technique? fatigued? ventilator support? infants? children? (Y/N) bulbar failure
(Y/N) (Y/N) (Y/N) (Y/N) (Y/N) (Y/N)
Manual Techniques N N Y Y Y Y Y 5 each lung area

to be treated
HFCWO N N Y Y Y Y Y 10–30
HFCWC N N Y N* Y Y Y 10–30
IPV N Y Y Y Y Y Y 30 with mask
5 with
mouthpiece
CWS N N Y Y Y Y Y Unclear
High frequency chest wall oscillation (HFCWO); high frequency chest wall compression (HFCWC); intrapulmonary percussive ventilation (IPV); chest wall strapping (CWS); yes (Y); no
(N). As with all peripheral airway clearance techniques (ACT's) in individuals with neuromuscular disorders it is essential to ensure effective proximal ACT's, to prevent secretion
retention in the central airways. IPV may provide ventilator support if set correctly. *Manufactures recommendation is in children > 3 years old. In patient who require ventilator support
these techniques should be used in conjunction with ventilator support.
4.1.2. Limitations of the technique combination with ventilator support, in patients who are ventilator-
Care should be taken in infants not compress below airway closing dependent. The major limitation of this technique in patients with NMD
volume or effective flow will be compromised. The lack of clear phy- is that proximal ACT's are still needed to clear secretions from the
siological effects and evidence base represent the limitations of manual central airways. There is also the potential to mobilise a vast amount of
techniques in NMD. secretions into the central airways, with the potential to precipitate a
respiratory arrest. Therefore, it is essential to have equipment readily
4.1.3. Evidence base available to clear secretions from the airway [50,100]. The devices are
There is no evidence base for the use of manual techniques in NMD. also expensive compared to other methods of ACT's.
However, manual techniques are widely used by professionals to help
mobilise secretions. 4.2.1.3. Evidence base. Yuan and co-workers [101] investigated HFCWC
in patients with NMD. Data suggest safety, tolerability, and better
4.2. Instrumental techniques compliance with HFCWC compared with “standard chest physiotherapy”.
Crescimanno and Marrone [102] suggested that HFCWC is easy to use and
4.2.1. High frequency chest wall oscillations (HFCWO)/high frequency accepted by patients with NMDs. They showed improvements in their
chest wall compression (HFCWC) clinical and radiological condition and suggested it was helpful for patients
HFCWC provides compression of the chest wall at frequencies that with scoliosis in whom conventional respiratory physiotherapy is not
are similar to the resonant frequency of the lung, between 5 and 20 Hz possible. HFCWC has been shown to decrease the work of breathing and
[98], via an air pulse generator that delivers intermittent positive air- decrease the sensation of breathlessness in with ALS and a sub-group
flow into the jacket. As the jacket expands compressing the chest wall, it showed a decreased rate of FVC decline [103]. More recently, Lechtzin and
produces a transient/oscillatory increase in airflow in the airways vi- co-workers [104] evaluated the impact of HFCWC on healthcare use in
brating the secretions from the peripheral airways toward the mouth patients with NMD. Total medical costs decreased after initiation of HFCWC
and can be used in conjunction with ventilator support. along with inpatient admission costs and pneumonia costs. However, whilst
HFCWO also provides compression of the chest wall at frequencies it looks like HFCWC decreases RTI's in NMD, no data was provided on
that are similar to the resonant frequency of the lungs via a negative adherence or use. A case report of HFCWO of a child with SMA type 1
pressure ventilator attached to a cuirass. As the ventilator delivers ne- suggested the device was safe and the authors concluded that the increase in
gative pressure the air is sucked into the lungs. When the negative ventilator free time was attributed to improved secretion clearance [105].
pressure ceases the patient breathes out. The device has the ability to
deliver high frequency intermittent negative pressure on top of the 4.2.2. Intrapulmonary percussive ventilation (IPV)
patients spontaneous or NIV supported breathing. This also produces a IPV is delivered via an IPPB pneumatic device. IPV delivers air to the
transient/oscillatory increase in airflow in the airways vibrating the lungs at frequencies of 100–300 cycles per minute at peak pressures from 10
secretions from the peripheral airways toward the mouth. to 40 cmH2O. IPV superimposes high-frequency bursts of gas on top of the
Starting settings for this device are a frequency of 5 Hz building up patient's own respiration. This creates a global effect of internal percussion
to 10–15 Hz. There have been no studies evaluating treatment times or of the lungs, which promotes clearance from the peripheral bronchial tree.
frequencies in NMD. Therefore, treatments are individualized or based The high frequency airflow pulsates to expand the lungs, vibrate and en-
on manufactures pre-set programs. Often treatments in NMD are around large the airways. This potentially delivers air to the distal lung units, be-
5 min stages or until the patient feels the need to cough. yond accumulated secretions. IPV is reported to improve airway clearance
and lung function in patients with NMDs [106].
4.2.1.1. Physiological effects. There is no evidence regarding the
physiological effects of chest wall oscillations. An intuitive 4.2.2.1. Physiological effects. The physiological effects of IPV have been
explanation of the physiologic effects on mucus clearance relates to studied in vitro [107]. Increasing frequency increases positive end-expiratory
generation of air-liquid shear forces [59]. The eccentric flow pattern pressure (PEEP) and percussion (i.e. the peak of pressure), but decreases
(higher expiratory flow than inspiratory flow) may promote transport ventilation. Increasing inspiratory/expiratory (I/E) time increases PEEP and
of secretions centrally. There is also some evidence that high frequency decreases percussion. Increasing pressure increases PEEP and ventilation.
oscillations reduce mucus viscosity [99]. Higher expiratory than inspiratory flows are always produced by IPV,
favouring proximal secretion mobilisation [107].
4.2.1.2. Limitations of the technique. These devices should be used in Parameters of IPV devices can be set as follows: in order to obtain
106
the highest peaks of pressure, high frequency and short inspiration change consists of the significant increase in maximal expiratory flow
times are recommended [95]. However, lower frequencies and higher (+47–88%) for the same lung volume during CWS, thus augmenting
pressures are required when patients need assisted ventilation. Lower the gas-liquid interactions. The enhancement in airflow is in particular
pressures and higher frequencies should be set for infants and children. attributed to by a higher conductance (+24–43%) and improved lung
Again, pressures may be increased to obtain normal oxygen saturation elastic recoil (+39–144%). The latter is expected to dilate small
and carbon dioxide levels in ventilator dependent patients, such as airways, which in turn may facilitate mucus transport [113,115–120].
those with SMA type 1. The length of IPV session is related to patient However, the benefits of these physiological changes may be
comfort. With face masks, patients cannot tolerate IPV ventilation overwhelmed in NMD as CWS has been associated with a significant
longer than 1 or 2 consecutive minutes. However, the use of nasal in- decrease in tidal volume ( ± 25%).
terface increases comfort and allows sessions of 15 min or longer [108].
Co-operation from the patient is not required with IPV. IPV is especially 4.2.3.2. Limitations of the technique. The strapped thorax diminishes
useful in patients with NMDs and acute respiratory failure. The use of pulmonary system compliance while work of breathing and dyspnoea
IPV is possible with artificial airways such as an endotracheal tube or increase. In order to prevent this risk, the technique is widely carried
tracheostomy. IPV is also possible in association with mechanical out on ventilatory support [115].
ventilation [109].
4.2.3.3. Evidence base. There is no evidence regarding deflation and
4.2.2.2. Limitations of the technique. Devices may not be readily strapping to mimic breathing at a low lung volume as in the airway
available in some countries and are also expensive. Other important clearance technique, autogenic drainage (deflation). However,
limitations are the lack of evidence on adequate settings of parameters physiological arguments and clinical experience advocate for using
and the need for intensive training for professionals to build experience CWS which induces breathing at low lung volumes, increases lung
enough to be able to set parameters in different clinical conditions. elastic recoil and increases maximal expiratory flows [121–123].
Finally, IPV devices may hyperventilate patients when there is no
control of arterial carbon dioxide during titration, in children in 4.3. Recommendations regarding peripheral airway clearance
particular.
An expert report suggests that IPV and HFCWO/HFCWC may be
4.2.2.3. Evidence base. Preventive use of IPV has been suggested useful recommended but lacks evidence, as for other ACT [124]. Various ACT
to prevent pulmonary infections in adolescents with NMDs who have modalities, including IPV, may be used effectively, either alone or in
impaired ability to clear secretions. Antibiotic use was lower, and the combination. IPV appears safe, even in infants who require airway
hospital stay was shorter [110]. IPV has also been shown to improve clearance assistance. To date, however, criteria for children are lacking
persistent pulmonary consolidation and appeared to be a safe and to determine when such modalities should be used and which are the
effective therapy for these patients who have difficulty mobilizing most effective [124]. The Paediatric BTS guidelines suggest that oscil-
sputum and who do not respond to conventional therapeutic techniques latory techniques should be considered in children who have difficulty
[100]. In tracheostomised hypersecretive DMD patients, the addition of mobilizing secretions or who have persistent atelectasis, despite use of
IPV enhanced secretion clearance and was a safe technique [111]. other airway clearance techniques [50]. AARC clinical practice guide-
Bidiwala and co-workers [112] compared IPV to HFCWC in complex lines [125] contradicts the BTS guidelines and suggests that oscillatory
patients with a tracheostomy. They found that IPV was a superior techniques cannot be recommended due to insufficient evidence. The
treatment compared to HFCWC as it was associated with a significant BTS guidelines on airway clearance techniques in the spontaneously
decline in hospitalizations, decreased RTIs, decreased antibiotic, breathing adult [93] highlight that “The ATS consensus statement on
bronchodilator and steroid use. The authors concluded that IPV could the management of patients with DMD [126] concludes there is also
be more effective and beneficial in providing airway clearance in insufficient evidence to make any firm recommendations on the use of
specific subsets of the medically complex pediatric population. IPV with self-ventilating patients, but that the use of airway clearance
devices dependent on a normal cough is likely to be ineffective without
4.2.3. Chest wall strapping (CWS) the concurrent use of other proximal ACT's. Therefore, other techni-
CWS is the restriction of the chest wall through the application of ques, alone or in combination, may be required to clear secretions once
elastic material around the thorax. Strapping via CWS passively lowers mobilized centrally following intrapulmonary percussive ventilation.
the functional residual capacity (FRC) without using expiratory mus- Further research is required to evaluate the safety and efficacy of IPV in
cles. This has been demonstrated to be beneficial for lung secretion the care of patients with NMD.” Box 3 highlights the recommendations
clearance [113]. The principles and physiological effects of CWS are from the authors of this state of the art review for peripheral ACT's,
similar to that of Autogenic Drainage [114]. taking into account all the evidence published since these guidelines
and non-randomized controlled trials. It is acknowledged that oscilla-
4.2.3.1. Physiological effects. The single most important physiologic tory devices are expensive and may not be readily available.
Box 3
Reccomendations for Peripheral ACT's.
• Peripheral ACT should be commenced before and after clearing any secretions from the upper airway with proximal ACT's
• Peripheral ACT's do not require physical or intellectual patient co-operation
• Peripheral ACT's is possible in infants, children and adults, even in the presence of a tracheostomy and/or bulbar failure
• Deflation by CWS strapping, is promising and worth evaluating in a clinical trial
• MT should be considered as a treatment option
• In the ventilatory dependent patient, peripheral ACT should be used in combination with ventilator support
ACT: airway clearance technique; IPV: intrapulmonary percussive ventilation; HFCWO: high frequency chest wall oscillations; CWS: chest
wall strapping; MT: manual techniques.
107
5. Limitations of this state of the art review session about IN/Exssuflator from Philips-Respironics in 2016 (500€).
Tiina Andersen has received an honoraria for lecturing on secretion
Our main limitation is that this review was not performed as a clearance and cough augmentation from Phillips Respironics.
systematic review for each technique because it was performed during a David Berlowitz received support from Phillips Respironics to at-
meeting gathering 21 international experts. For that reason, the current tendance at ENMC meeting in Naarden, The Netherlands.
recommendations could be biased or incomplete. However, a summary
of the studies can be found in the online supplement, 2, 3 and 4. Statement of contribution
6. Conclusion All authors contributed to the preparation of the manuscript and

have reviewed the final version. MC contributed to the original version,
In this state of the art review we recommend that PCF is routinely reviewed and re wrote the manuscript to the final version which was
measured in patients with NMD. PCF and MIC measurments can be used significantly contributed to BM and MT. MT and NS drew Figs. 3 and 4.
to evaluate the efficay of proximal ACTs, however PCF measurements
from MI-E devices may not be accurate and should be used to establish Acknowledgments
a trend only. By splitting ACTs into peripheral and proximal ACTs we
have described treatment options for patients with NMD. For proximal The Authors would like to thank the Myotubular Trust for funding
ACTs, we recommend MAC and assisted inspirations, as single breaths, this state of the art review to be open access.
AS and GPB, in patients with higher PCF. These techniques can be
combined to futher increase efficacy. MI-E appears to be very effective Appendix A. Supplementary data
in patients with lower PCF's (< 160 L/min). MI-E should be considered
in weak children and those who cannot cooperate with MAC or AS or in Supplementary data related to this article can be found at http://dx.
whom these methods are not effective. Titration should be in- doi.org/10.1016/j.rmed.2018.01.012.
dividualized and is possible with patients with ALS, with appropriate
modifications. MI-E is widely considered an essential tool in the re- References
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Respiratory Medicine 136 (2018) 98–110

Contents lists available at ScienceDirect

Airway clearance techniques in neuromuscular disorders: A state of the art T

Fig. 1. Shows the classiﬁcation of airway clearance techni-

2.2. Peak cough ﬂow (PCF)

Quantitative assessment of cough eﬃcacy in NMD is typically made

• PCF should be measured at each clinic review

Fig. 3. Shows schematic ﬂow, volume and pressure curves

Fig. 4. Shows schematic ﬂow, volume and pressure curves

Manual Techniques N N Y Y Y Y Y 5 each lung area

6. Conclusion All authors contributed to the preparation of the manuscript and

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