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Physiotherapy for Cystic Fibrosis in Australia and New
Zealand:
A Clinical Practice Guideline
Online Supplement*
Foreword
This Clinical Practice Guideline has been written by physiotherapists who are
experienced in the management of cystic fibrosis in Australia and New Zealand.
Chairperson: Associate Professor Brenda Button
Senior Clinician Physiotherapist
Alfred Hospital and Monash University, Melbourne
Editor: Professor Anne Holland
Clinical Chair, Physiotherapy
La Trobe University / Alfred Health, Melbourne
Writing group:
Jenny Bishop (group leader) Westmead Hospital, NSW
Ryan Black (group leader) Lady Cilento Children’s Hospital, Brisbane
Summar Bowen Princess Margaret Hospital, Western Australia
Kate Burton (group leader) The Prince Charles Hospital, Queensland
Brenda Button (group leader) Alfred Hospital, Victoria
Robyn Cobb (group leader) The Prince Charles Hospital, Queensland
Narelle Cox (group leader) La Trobe University, Victoria
i
*Any reference to this online supplement should cite the accompanying
Respirology paper as the primary publication:
Button BM, Wilson C, Dentice R, Cox N, Middleton A, Tannenbaum E, Bishop J, Cobb R, Burton K,
Wood M, Moran F, Black R, Bowen S, Day R, Depiazzi J, Doiron K, Doumit M, Dwyer T, Elliot A,
Fuller L, Hall K, Hutchins M, Kerr M, Lee A, Mans C, O’Connor L, Steward R, Potter A, Rasekaba T,
Scoones R, Tarrant B, Ward N, West S, White D, Wilson L, Wood J, Holland AE. Physiotherapy for
Cystic Fibrosis in Australia and New Zealand: A Clinical Practice Guideline. Respirology 2016; 21:
xxx-xxx
Rosie Day John Hunter Children’s Hospital, NSW
Julie Depiazzi Princess Margaret Hospital, Western Australia
Ruth Dentice (group leader) Royal Prince Alfred Hospital, NSW
Katherine Doiron Bond University/Pindara Private Hospital, Qld
Michael Doumit Sydney Children’s Hospital, NSW
Tiffany Dwyer Central Clinical School, Sydney Medical
School, University of Sydney; Royal Prince Alfred Hospital, NSW.
Alison Elliot The Children’s Hospital at Westmead, NSW
Louise Fuller Alfred Hospital, Victoria
Kathleen Hall The Prince Charles Hospital, Queensland
Anne Holland La Trobe University / Alfred Health, Victoria
Matthew Hutchins Mater Health Services
Melinda Kerr Royal Children’s Hospital, Victoria
Annemarie Lee Alfred Hospital, Victoria
Christina Mans Waikato DHB, Hamilton NZ
Anna Middleton (group leader) Children’s Hospital at Westmead, NSW
Fiona Moran (group leader) The Royal Children’s Hospital,Victoria.
Lauren O’Connor Gold Cost University Hospital, Queensland
Ranjana Steward Alfred Hospital, Victoria
Angela Potter Women’s & Children’s Hospital, SA
Tshepo Rasekaba Alfred Hospital, Victoria
Rebecca Scoones Starship Children’s Health, New Zealand
Esta-Lee Tannenbaum (group leader)Royal Children’s Hospital, Victoria
Ben Tarrant Alfred Hospital, Victoria
Nathan Ward Royal Adelaide Hospital, SA
Samantha West Westmead Hospital, NSW
Dianne White Royal Adelaide Hospital, South Australia
Christine Wilson (group leader) Royal Children’s Hospital, Queensland
Lisa Wilson Alfred Hospital, Victoria
Jamie Wood Sir Charles Gairdner Hospital and Institute for
Respiratory Health, Western Australia
Michelle Wood (group leader) The Prince Charles Hospital, Queensland
ii
Our thanks are extended to the following individuals for their valuable comments and
review of this document: Ms Nardia Robertson, Dr Audrey Tierney, Dr Dominic
Keating, Dr Kathy Stiller.
iii
Table of Contents
Foreword.........................................................................................................................i
Table of Contents...........................................................................................................iv
List of Abbreviations.......................................................................................................x
Overview .....................................................................................................................xii
1 Introduction.......................................................................................1
Background to the Clinical Guideline........................................................................1
Purpose and Scope.....................................................................................................1
Process........................................................................................................................1
Methods......................................................................................................................2
Dissemination.............................................................................................................3
Review process...........................................................................................................3
Disclosure statement..................................................................................................5
2 Airway Clearance Techniques..........................................................7
Rationale for Airway Clearance Techniques in Cystic Fibrosis................................7
Active Cycle of Breathing Technique........................................................................8
Autogenic Drainage...................................................................................................9
Positive Expiratory Pressure (PEP) Therapy...........................................................10
High Pressure PEP...................................................................................................11
Oscillating PEP........................................................................................................12
The Flutter®.........................................................................................................13
Acapella®.............................................................................................................14
RC-Cornet®.........................................................................................................14
iv
Postural Drainage.....................................................................................................15
Postural Drainage in gravity-assisted positions...................................................15
Modified postural drainage..................................................................................17
Percussion and vibration..........................................................................................17
Other airway clearance techniques...........................................................................19
High Frequency Chest Wall Oscillation (HFCWO)............................................19
Intrapulmonary Percussive Vibration (IPV)........................................................20
Physical Exercise as Airway Clearance...................................................................20
Clinical Decision Making regarding Airway Clearance Techniques.......................22
Recommendations....................................................................................................23
3 Inhalation Therapy as an Adjunct to Physiotherapy...................24
Inhalation Therapy Technique (including Adjuncts to Inhalation Therapy)...........24
Mode of Delivery.....................................................................................................26
Mixing Inhaled Medications....................................................................................27
Timing and Order of Inhaled Medications...............................................................28
Nebuliser Maintenance.............................................................................................29
Clinical Monitoring of Inhalation Therapy..............................................................30
Recommendations....................................................................................................31
4 Exercise.............................................................................................33
Assessment of exercise capacity..............................................................................33
Six minute walk test.............................................................................................34
The 3-minute step test..........................................................................................35
Modified Shuttle test (walk/run)..........................................................................36
v
Choosing a test of exercise capacity....................................................................37
Exercise prescription in cystic fibrosis....................................................................38
Facilitating physical activity in people with CF......................................................40
Recommendations....................................................................................................41
5 Musculoskeletal Complications of Cystic Fibrosis.......................41
Pain...........................................................................................................................42
Low Bone Mineral Density......................................................................................42
Vertebral compression and rib fractures..................................................................43
Increased thoracic kyphosis.....................................................................................44
Muscle strength........................................................................................................44
Muscle length...........................................................................................................45
CF-related arthropathy.............................................................................................46
Physiotherapy intervention for musculoskeletal problems......................................46
Optimise physical activity to maintain bone mineral density and muscle mass. .47
Optimise muscle strength.....................................................................................48
Optimise muscle length........................................................................................48
Manual therapy and pain management................................................................49
Recommendations....................................................................................................52
6 Physiotherapy management of the complex patient.....................53
Pneumothorax..........................................................................................................55
Allergic Bronchopulmonary Aspergillosis..............................................................57
Cystic fibrosis related diabetes.................................................................................58
7 Physiotherapy for pregnancy, labour and the post-natal period 61
vi
Physiological changes during pregnancy.................................................................61
Pre-pregnancy planning...........................................................................................62
Airway Clearance Therapy during pregnancy in CF...............................................62
Pelvic floor function.................................................................................................63
Exercise during Pregnancy.......................................................................................63
Other physiotherapy interventions during pregnancy..............................................64
Physiotherapy during labour in CF..........................................................................64
Physiotherapy post-Caesarian section in CF............................................................64
Physiotherapy in the post-natal period.....................................................................65
8 Physiotherapy management of continence....................................66
Recommendations....................................................................................................68
9 Physiotherapy management of the newly diagnosed patient.......69
The newly diagnosed infant.....................................................................................69
The newly diagnosed adult.......................................................................................70
Recommendations....................................................................................................71
10 Transition from paediatric to adult care – physiotherapy role...72
11 Physiotherapy management for end-stage disease.......................74
Non-Invasive Ventilation in Cystic Fibrosis............................................................74
Acute respiratory failure and bridge to transplant................................................74
Sleep disordered breathing and chronic respiratory failure.................................75
Non-invasive ventilation as an adjunct to airway clearance................................76
Exercise and non-invasive ventilation.................................................................77
General considerations for NIV...........................................................................77
vii
Recommendations................................................................................................78
Physiotherapy and Lung Transplantation.................................................................79
Transplant assessment..........................................................................................79
Preparation for transplantation.............................................................................79
Post-operative period...........................................................................................80
Rehabilitation post transplant...............................................................................81
Transplant-related problems.................................................................................81
Recommendations................................................................................................82
Cystic Fibrosis and the Intensive Care Unit.............................................................83
Intensive Care for Reversible CF Complications.................................................83
Intensive Care for Respiratory Failure.................................................................83
Extra-corporeal Membrane Oxygenation (ECMO).............................................83
Physiotherapy for CF in Intensive Care...............................................................84
Palliative or end of life care in cystic fibrosis..........................................................85
When to switch from active treatment to palliative care......................................86
The impact of lung transplantation on end of life care........................................86
Physiotherapy treatment at end of life in CF.......................................................87
12 Adherence to physiotherapy in cystic fibrosis..............................89
13 Infection Control during physiotherapy in cystic fibrosis...........92
Airway clearance and inhalation therapy.................................................................92
Gym Sessions and Exercise.....................................................................................93
Outpatient Practice...................................................................................................94
14 Delivery of physiotherapy treatment to inpatients and
outpatients with cystic fibrosis..............................................................95
viii
Physiotherapy treatment for inpatients.....................................................................95
Physiotherapy treatment for outpatients...................................................................96
15 Directions for Future Research......................................................98
16 References.........................................................................................99
APPENDICES......................................................................................129
Appendix 1 Protocols for airway clearance techniques.........................................129
Appendix 2: Three-minute step test protocol.........................................................130
Appendix 3: Modified shuttle test protocol...........................................................132
Appendix 4: Musculoskeletal Screening Tool.......................................................134
Appendix 5: Pelvic floor exercises for people with CF.........................................135
Appendix 6: Conflict of Interest Statements..........................................................136
ix
List of Abbreviations
3MST three minute step test
6MWT six minute walk test
ABPA allergic bronchopulmonary aspergillosis
ACBT active cycle of breathing technique
ACT airway clearance technique
AD autogenic drainage
BC breathing control
BGL blood glucose level
BMD bone mineral density
CF cystic fibrosis
CFRD cystic fibrosis related diabetes
CPAP continuous positive airway pressure
FEV1forced expiratory volume in one second
FET forced expiration technique
FVC forced vital capacity
GOR gastro-oesophageal reflux
HFCWO high frequency chest wall oscillation
HPOA hypertrophic pulmonary osteoarthropathy
IPG/CF International Physiotherapy Group / Cystic Fibrosis
IPV intrapulmonary percussive ventilation
MST modified shuttle test
NHLBI National Heart, Lung, and Blood Institute
x
NIV non-invasive ventilation
PaCO2partial pressure of carbon dioxide in arterial blood
PEFR peak expiratory flow rate
PD postural drainage
QOL quality of life
TEE thoracic expansion exercises
WHO World Health Organisation
xi
Overview
Physiotherapy management is a key element of care for people with cystic fibrosis
(CF). Airway clearance techniques, exercise and inhalation therapy are cornerstones
of treatment and are associated with improved long-term outcomes. As survival
improves, complications associated with CF such as musculoskeletal pain, urinary
incontinence and cystic fibrosis related diabetes are becoming more apparent and
require physiotherapy management strategies. Physiotherapists are members of the
multidisciplinary CF team throughout the lifespan, from initial diagnosis to the care of
end-stage disease.
Although considerable evidence exists to support physiotherapy management in CF,
there is considerable variation in clinical practice. The need for physiotherapy
guidelines which were applicable to the Australian and New Zealand healthcare
context was identified by members of the writing group, in order to support clinicians
in delivering best practice care. The objective of this document is therefore to
optimise physiotherapy management of people with CF in Australia and New
Zealand.
The Guideline provides recommendations for the key areas of physiotherapy
management for patients with CF, including airway clearance therapy, inhalation
therapy, exercise, musculoskeletal management, care of the complex patient,
management of the newly diagnosed patient, end of life care and infection control.
This Supplement provides more detail of the evidence underpinning the
recommendations and application of the physiotherapy techniques.
The Guideline will be due for review in 2019. Responsibility for organising the
review process will be taken by the incumbent chairperson of the Australian chapter
of the International Physiotherapy Group/Cystic Fibrosis (IPG/CF) and country
contact person for Australia in the IPG/CF.
xii
1 Introduction
Background to the Clinical Practice Guideline
Physiotherapy management is a key element of care for people with cystic fibrosis
(CF). Airway clearance techniques, exercise and inhalation therapy are cornerstones
of treatment and are associated with improved long-term outcomes (1-3). The Clinical
Practice Guideline provides recommendations for the key areas of physiotherapy
management for people with CF, including airway clearance techniques (ACTs),
inhalation therapy, exercise and musculoskeletal management. The recommendations
are graded according to National Health and Medical Research Council (NHMRC)
guidelines (4) and based on evidence up to and including June 2014. Elements of
clinical practice that were considered important but lacking research evidence, and not
likely to have research evidence in the future, were highlighted as ‘Practice Points’.
Purpose and Scope
The overall objective of this document is to optimise physiotherapy management of
people with CF in Australia and New Zealand.
The aims of the guideline are:
1. To provide recommendations regarding best-practice physiotherapy management
for physiotherapists caring for infants, children and adults with CF
2. To promote physiotherapy management of people with CF that is evidence-based
and reflects the best available knowledge
3. To standardise the physiotherapy care of people with CF across centres
4. To provide a reference tool to support training of physiotherapists in best-practice
CF management and to support isolated practitioners who care for people with CF.
Process
This Clinical Practice Guideline is an update of ‘Physiotherapy for Cystic Fibrosis: A
Consensus Statement’, which was endorsed by the Thoracic Society of Australia and
New Zealand in 2008.
All known physiotherapists who regularly care for people with CF across paediatric
and adult settings in both Australia and New Zealand were invited to participate in
1
this update. A call for volunteers was made via email and at the 9th Australian and
New Zealand Cystic Fibrosis Conference in Melbourne in 2011. Participants were
invited to nominate their areas of interest for the update. Group leaders were
appointed to coordinate the update of each chapter.
Updates of each chapter were prepared by the writing group and circulated to the
whole group for comment and revision. The document was compiled into one
editorially consistent text by the editor and circulated to the group for review.
All writing group members were invited to attend a workshop held in conjunction
with the 10th Australian and New Zealand Cystic Fibrosis Conference in Auckland in
2013. At this time, feedback was sought from group members on all sections of the
document, especially the recommendations. The draft document was then offered to
stakeholders for comment, including CF physicians, CF consumers, allied health
professionals and expert physiotherapists who were not part of the writing group.
Methods
The literature search for this document was conducted systematically using electronic
databases including MEDLINE, CINAHL, EMBASE and PEDro. Separate searches
were undertaken for each chapter and each search strategy was documented. Manual
search of relevant conference proceedings was undertaken. Searches were limited to
articles in English. Literature up to and including June 2014 was included. Both
randomised controlled trials and research conducted with less robust designs were
included. Groups were encouraged to make use of existing systematic reviews when
they were available.
One or more individuals from each group decided which studies should be included in
the update. Data from every included study were extracted into an evidence table by
one reviewer and checked by a second reviewer. Data extracted were study identifier,
number and characteristics of participants, design, intervention details where
appropriate, results and study limitations. The quality of each study was graded
according to the NHMRC evidence hierarchy and recommendations formulated (4).
Recommendations were formulated based on the quality, quantity and level of the
evidence; the consistency of the body of evidence; the likely clinical impact; and
generalisability and applicability to physiotherapy practice in Australia and New
Zealand (4). Elements of clinical practice that were considered important but lacking
research evidence, and not likely to have research evidence in the future, were
2
highlighted as ‘Practice Points’.
Dissemination
The evaluation of the 2008 Consensus Statement (5) showed that physiotherapists
used and preferred a wide range of methods for accessing the document, including
hard copy, soft copy and via the internet. This Clinical Practice Guideline will be
disseminated amongst physiotherapists working in CF centres throughout Australia
and New Zealand in hard copy and soft copy. It will also be made available on the
Thoracic Society of Australia and New Zealand (TSANZ) website. Evaluation
findings also showed that knowledge of the previous Consensus Statement treatment
recommendations were lower outside specialist CF centres and in specialist areas of
CF practice. This will be addressed by active dissemination of the guidelines to non-
CF centres in metropolitan regions, as well as regional centres and all known
physiotherapists involved in CF care outside of specialist centres. We will also
prioritise the provision of education in specialist areas of CF practice at CF,
respiratory and general physiotherapy conferences. A copy will also be provided to all
university physiotherapy schools in Australia and New Zealand. The guideline will be
published in Respirology.
Review process
The guideline will be due for review and updating in 2019. The process will be
coordinated by the incumbent chairperson of the Australian chapter of the
International Physiotherapy Group – CF (IPG/CF) and the contact physiotherapist for
the IPG/CF in Australia and New Zealand. Prior to this update, an evaluation of the
document will be undertaken via a survey of physiotherapy practice across all centres
in Australia, consistent with our evaluation of the 2008 Consensus Statement (5).
Update of the document will incorporate the findings of the evaluation, as well as new
evidence from the scientific literature.
3
Table 1: Definition of National Health and Medical Research Council grades of
recommendations (4)
Grade of
recommendation
Description
ABody of evidence can be trusted to guide practice
BBody of evidence can be trusted to guide practice in most
situations.
CBody of evidence provides some support for
recommendation(s) but care should be taken in its
application
DBody of evidence is weak and recommendation must be
applied with caution
4
Disclosure statement
Development of the 2015 Clinical Practice Guideline was partially funded by a grant
from Cystic Fibrosis Australia to support the editorial role of Dr Holland.
Development of the 2008 Consensus statement was funded in part by unrestricted
educational grants from Roche Pharmaceuticals and Solvay Pharmaceuticals. This
funding was used to support travel to the consensus conference in Melbourne in
September 2006 and to assist dissemination by hard copy and CD ROM. A grant from
Cystic Fibrosis Australia supported the editorial role of Dr Holland. These funding
bodies were not involved in formulation of the consensus statement or the
recommendations.
Declarations of interest were made by each author according to the policies of the
TSANZ. Any perceived conflicts of interest were managed by the Editor.
Declarations of interest are outlined in Appendix 6.
5
6
2 Airway Clearance Techniques
Rationale for Airway Clearance Techniques in Cystic Fibrosis
Cystic fibrosis is a genetic, life-limiting disorder. Obstruction of exocrine glands by
viscous secretions causes pathological change in a range of body systems. In the lungs
this is manifested as abnormal mucus secretion in the airways, which is responsible
for persistent infection and inflammation. This process is the major contributing factor
to severe airway damage and deteriorating lung function. The thickness and amount
of airway secretions overwhelm the body’s normal mucus transport mechanisms and
therefore treatment methods that improve mucus clearance are considered essential in
optimising respiratory status and slowing the progression of lung disease.
Chest physiotherapy has been defined as ‘the external application of a combination of
forces to increase mucus transport’ (6). Because the aim of these techniques is to clear
sputum from the airway, and to encompass the range of modern techniques available,
modern chest physiotherapy is usually referred to by the generic term of ‘airway
clearance techniques’ (7).
Airway clearance techniques (ACTs) are usually commenced as soon as the diagnosis
of CF is made, often soon after birth. In infants ACTs are performed by the parents,
however as children grow older they are taught techniques that can be performed
independently of an assistant (8). People with CF undertake this treatment both during
acute exacerbations and prophylactically between infections (9).
It has been stated that ‘physiotherapy has a major influence in limiting the adult
consequences of CF’ (10) p240. This is based on the assumption that ACTs have both
short-term beneficial effects, and are able to slow the rate of pulmonary deterioration
over time, via physical compensation for decreased mucociliary clearance. A
Cochrane review has concluded that ACTs have short-term beneficial effects on
mucus transport in CF however there was no evidence regarding long-term effects
(11). One uncontrolled study has evaluated the effects of withdrawing ACTs for three
weeks and found a detrimental effect on lung function (1). Beyond this study there is
little evidence regarding the long-term efficacy of ACT versus no treatment. Due to
the ethical concerns regarding the withholding of such a well-established treatment it
is now considered unlikely that such a controlled trial could be conducted, especially
in adults with established lung disease (7).
A Consensus Conference Report on Cystic Fibrosis Adult Care describes airway
7
clearance as a ‘cornerstone’ of treatment (12) p5S. As such it is the role of
physiotherapists to ensure that the ACTs prescribed have a sound basis in physiology
and can be effectively performed across the lifespan. This chapter discusses the
various ACTs that a physiotherapist may perform or prescribe for a person with CF.
Several systematic reviews note that no single ACT is superior, nor is one approach
suitable for all patients (13-15), such that treatments should be individualised.
Active Cycle of Breathing Technique
The active cycle of breathing technique (ACBT) consists of breathing control (BC),
thoracic expansion exercises (TEEs) and the forced expiration technique (FET) (16).
Studies using the ACBT have shown it to be an effective technique for the
mobilisation and clearance of airway secretions (17), and is as effective as other
widely used ACTs (13). Previous studies demonstrated that ACBT is not further
improved by the adjuncts of positive expiratory pressure (18), oscillating positive
expiratory pressure (19, 20) or mechanical percussion (21). An improvement in lung
function following the instigation of the ACBT has been shown in an uncontrolled
study (22).
Physiotherapy practice
During the ACBT, BC is followed by TEE. Breathing control is then repeated
followed by the FET. The entire ACBT is repeated until the huff sounds dry and is
non-productive, or it is time for a rest. A minimum of ten minutes in a productive
position is recommended. If more than one position is needed, two positions are
usually sufficient for one treatment session. The total treatment time is between ten
and thirty minutes. A weblink to the protocol for ACBT can be found in Appendix 1.
The ACBT regimen is flexible and can be adapted to suit the individual. The ACBT
should never be uncomfortable or exhausting and the huff should never be violent.
The sitting position alone is often effective and adherence to treatment is frequently
better than with other positions. In some people other gravity assisted positions may
be indicated. In CF and non-CF bronchiectasis it has been shown that the horizontal,
side lying position is as effective as the head down tipped position and preferred by
patients, who report fewer side effects such as head ache and sinus pain (23). The
ACBT can be used independent of an assistant and in any position. If an assistant is
present, chest percussion or vibration can be combined with TEE. The ACBT is
widely applicable in CF. It can be performed by all patients who can follow
instructions and is useful in all stages of disease. In young children (from 2-3 years),
8
blowing games and huffing can be commenced to educate the elements of ACBT, as
foundations for incorporation when developmentally and clinically appropriate. The
ACBT is a useful treatment option in patients where other techniques are
contraindicated (eg haemoptysis). In patients with a small pneumothorax treated
conservatively (no intercostal drain), breath holds (inspiratory pauses) are not
recommended.
During forced expirations, 69% of 40 adult patients studied had tracheomalacia
visible on dynamic CT, causing marked reductions in tracheal cross-sectional luminal
area (24). In assessment of cough sound, a barking, brassy, or vibratory cough (25) is
a strong indicator of airway malacia, and is associated with slower resolution of
infective exacerbations. Implications for practice include careful teaching and
modification of forcefulness of huffing and coughing to avoid early closure of
unstable airways.
Autogenic Drainage
Autogenic drainage (AD) is a technique based on the principle of reaching the highest
possible airflow in different generations of bronchi by controlled breathing (26). It
was introduced by Chevaillier (27), as a result of the observation that children with
difficult asthma frequently cleared more sputum during breathing exercises, playing,
laughing or spirometry than they did during conventional chest physiotherapy. One of
the aims of AD is to avoid airway closure that may be caused by coughing and forced
expiratory manoeuvres (28).
Autogenic drainage has three phases. During the ‘unstick’ phase, breathing takes
place at low lung volumes in order to unstick peripheral mucus. This is followed by
the ‘collect’ phase, where the mucus is collected from the middle airways by
breathing at tidal volume level. In the final ‘evacuate’ phase, breathing takes place at
higher lung volumes in order to evacuate secretions from the central airways. A
weblink to a description of the protocol for AD can be found in Appendix 1.
Greater expiratory flow is generated in smaller airways with AD compared to forced
expirations (29). Short-term studies have shown that AD is as effective as postural
drainage and percussion (30), oscillating PEP (31) and the ACBT (32). In a long term
comparative study in adolescents with CF, AD was as effective as postural drainage
and percussion, and participants showed strong preference for AD (33).
9
Physiotherapy practice
Autogenic drainage is not an easy technique to learn for either patient or therapist. A
physiotherapist wanting to use this technique should attend an AD course or spend
time with a physiotherapist experienced in teaching AD. Autogenic drainage requires
a patient that is ‘in tune’ with their body and can sense the location of the mucus.
Considerable patience is required when learning and undertaking AD as it can be time
consuming.
Autogenic drainage aims to prevent airway collapse and reduce limitation to
expiratory flow (34). As such it is likely to be useful in patients who have unstable
airways or evidence of airway hyperreactivity (35). Some patients become air-hungry
when breathing at low lung volumes. These patients may need to take a normal
resting breath and then return to breathing close to residual volume. More details abou
the execution of AD can be found via the weblink in Appendix 1.
The technique of AD may be adapted for infants and young children, where it is
known as assisted AD. The therapist places his or her hands on the child’s chest to
manually increase expiratory flow and prolong expiration towards residual volume
(36). These thoracic compressions are carried out gently, following the child’s
breathing pattern and stabilising the abdominal wall. Excessive force and discomfort
must be avoided as the child will resist the manoeuvres if uncomfortable. This
approach also requires specific training for the physiotherapist, as the small closing
volumes in infants increase the risk of early airway closure and worsening gas
exchange (37).
Positive Expiratory Pressure (PEP) Therapy
Positive expiratory pressure (PEP) therapy is defined as breathing against a positive
expiratory pressure of 10 – 20 cmH2O (38). The theoretical rationale for the use of
PEP therapy is that in the presence of small airway obstruction caused by secretion
retention, the relative resistance to airflow in collateral channels will be reduced. The
application of positive pressure to the airway will allow an increased volume of air to
accumulate behind the obstruction, temporarily increasing functional residual capacity
(FRC). The pressure gradient across the obstructed region will force secretions
centrally toward the larger airways, from where they can be expectorated (39, 40). It
is proposed that PEP is also effective through increasing the volume in neighbouring
lung units, which in turn provide an outward pull on the obstructed unit, allowing re-
expansion and improved airflow (41). Improvement in gas mixing and
oxyhaemoglobin saturation following PEP support this rationale (42).
10
In an early study by Falk et al (40), PEP was shown to be superior to postural
drainage in terms of sputum clearance. In comparison to conventional chest
physiotherapy over a 12 month period, McIlwaine et al (43) found that children who
performed PEP had better pulmonary function. Over a similar time period, McIlwaine
(44) found a significantly greater rate of decline in lung function in children using
oscillating PEP compared to PEP. However Newbold (45) found no significant
difference in decline of lung function between adults using the two therapies, and
West et al (46) found no significant difference in lung function, exercise tolerance,
sputum expectoration or patient satisfaction in children during a two week hospital
admission. In a crossover trial comparing a single session of PEP to one of PEP to
high frequency chest wall intervention, Fainardi et al found no significant difference
in lung function, sputum expectoration or exertion, however significantly more
patients preferred PEP (47). A Cochrane review concluded that PEP was no more or
less effective than other forms of ACT and that there was some evidence of patient
preference for PEP over other techniques (15). Patients with non-CF tracheomalacia
may benefit from PEP therapy by increasing cough expiratory flow (25).
Physiotherapy Practice
Positive expiratory pressure therapy can be delivered through a face mask or a
mouthpiece. Most studies are based on mask PEP. If a mouthpiece is used, a noseclip
may be necessary during the training phase. In both systems, PEP is created by
exhaling through a narrow opening, providing resistance to expiration. The resistor
should provide a steady PEP of 10-20cmH20 during the middle of expiration. When
teaching PEP to a child, it is may be helpful to delay the use of a manometer or
pressure indicator until the process of PEP breathing is practiced and a regular rhythm
is established; thereafter check for correct pressure plateau. A weblink to a description
of the protocol for PEP can be found in Appendix 1.
Contraindications: The use of PEP therapy is contraindicated where there is an
undrained pneumothorax.
Precautions: Inability to tolerate an increased work of breathing, raised intracranial
pressure, haemodynamic instability, recent facial, oral or oesophageal surgery, acute
sinusitis, active haemoptysis, middle ear pathology, and drained pneumothorax (48).
High Pressure PEP
High pressure PEP therapy is a modification of PEP therapy which includes a full
forced expiration against a fixed mechanical resistance (49). This method uses the
11
same PEP system as PEP therapy (Astra Tech, Denmark). The theoretical rationale for
high pressure PEP is that the forced expiration against a marked resistive load will
squeeze air from hyperinflated lung units into unobstructed and atelectatic lung units.
The back pressure effects a homogenised slow expiratory evacuation of all lung units.
The reduced airflow velocity is counterbalanced by the effects of dynamic airway
bronchial compression.
The expiratory resistance is individually determined by connecting the PEP set-up to a
pneumotachograph and performing a series of forced vital capacity (FVC)
manoeuvres with different resistors. The resistor that provides the greatest increase in
FVC over baseline values, along with a sustained plateau in expiratory flow and
avoiding early airway closure, is chosen for use during treatment (49).
Studies on the use of high pressure PEP have been limited and mainly conducted at
one centre where it is regularly used. Existing evidence shows that high pressure PEP
is of benefit in both the short and long term with improved sputum clearance, lung
function and a reduction in hyperinflation (35, 50, 51), but quality of the evidence is
low to moderate.
Physiotherapy practice
High pressure PEP is effective in patients with collapsible airways, tracheomalacia,
bronchomalacia or respiratory muscle weakness. A weblink to a description of the
protocol for high pressure PEP can be found in Appendix 1.
Contra-indications to high pressure PEP include pneumothorax, cardiac disease, frank
haemoptysis, lung surgery and asthma. High pressure PEP is not recommended for
patients who are exhausted and are unable to meet the demands of this energy-
consuming technique.
Oscillating PEP
Oscillating PEP refers to a variety of devices that combine PEP with oscillation of
airflow. The addition of oscillation is thought to loosen secretions and thus facilitate
airway clearance (52). When the resonance frequency of the pulmonary system is
achieved, the pressure variations are amplified, maximising the vibrations of the
airway wall. Three commonly used devices are the Flutter®, the Acapella® and the
RC-Cornet®.
Contraindications to oscillating PEP are frank haemoptysis and undrained
pneumothorax.
12
Precautions: Caution should be exercised in patients who are unable to tolerate an
increased work of breathing, those with raised intracranial pressure, haemodynamic
instability, recent facial, oral or oesophageal surgery, acute sinusitis or middle ear
pathology (48).
The Flutter®
The Flutter® is a pipe-shaped device consisting of a mouthpiece and a small ball
which occludes the opening of a plastic cone. When the patient exhales into the
device, PEP is generated. When this pressures reaches 10 – 25 cmH 2O, the ball rises
and expiratory pressure drops. The rise and fall of the ball and its movement along the
surface of the cone creates an oscillatory vibration of the air within the airways many
times a second throughout exhalation.
Of all the oscillating PEP devices available, the Flutter® has been the most
thoroughly studied. A laboratory study (53) demonstrated that a positive incline
optimises PEP, oscillation and flow amplitude effects. Short term clinical trials
indicate that the Flutter® is at least as effective as other ACTs (31, 54-57) and that it
significantly reduces sputum viscoelasticity (31). Long-term data are less conclusive.
In a one-year randomised controlled trial comparing the Flutter® with the PEP mask
in children with CF, greater deterioration in lung function and increased rate of
hospitalisation was seen in the Flutter® group (44). However, a more recent study
over 13 months showed no difference in lung function between groups randomly
assigned to PEP or Flutter® (45). Turboforte® is marketed as having similar
indications to Flutter®.
Physiotherapy practice:
The Flutter® produces a range of oscillation frequencies between 2-32 Hz. Only
frequencies between 8 – 16 Hz have been found to be useful for airway clearance
(52). The frequency can be modulated by changing the inclination of the device either
slightly up (higher frequency) or slightly down (lower frequency). The performance
of the Flutter® is gravity-dependent such that the device must be positioned upright in
order to produce oscillation. It therefore requires practise and skill to use the Flutter®
in positions other than sitting. Because of the complexity of the technique, children
may need supervision to use the Flutter® effectively. A weblink to a description of
the protocol for Flutter® can be found in Appendix 1.
13
Acapella®
The Acapella® combines the principles of high frequency airflow oscillation and PEP
by employing a counterweighted lever and magnet. During expiration, air passes
through a cone, which is intermittently occluded by a plug attached to a lever. A dial
at the distal end of the device adjusts the proximity of the magnet and
counterweighted plug, thereby adjusting the frequency, amplitude and mean pressure
of airflow through the device.
It is known that the in vitro pressure and frequency characteristics of the Acapella®
are similar to those of the Flutter® (58). However, there are limited data regarding its
clinical efficacy. In a randomised crossover trial of ACBT versus Acapella® in non-
CF bronchiectasis, no differences were found between weight of sputum expectorated,
although a greater proportion of patients preferred the Acapella® (59). In children
with CF, the Acapella® appears equally effective as PEP mask therapy during
treatment of an acute respiratory exacerbation (46).
Physiotherapy practice
The Acapella® produces a PEP range of 7-35 cmH20, and a frequency of airflow
oscillation of 0-30Hz. Adjusting the dial clockwise increases the resistance of the
vibrating orifice, which will allow the patient to exhale at a lower flow rate and with
increased PEP. The Acapella® is not gravity-dependent and can be used in any body
position. The Acapella® was compared to no treatment (3 month crossover, 1 month
washout) in 20 adults with non-CF bronchiectasis, with significant improvements in
quality of life, exercise capacity and sputum volume (60). Whilst caution must be
used in extrapolation to CF, this study provides some support for its use in chronic
suppurative lung disease. A weblink to a description of the protocol for the Acapella®
can be found in Appendix 1.
RC-Cornet®
The RC Cornet® consists of a mouthpiece, a hose contained within a semicircular
tube and a sound damper. During exhalation, a kink is produced in the hose which
moves along the length of the tube, producing PEP and airflow oscillation. Twisting
the mouthpiece alters the pressure and flow. By changing from positions one through
four, the twist on the hose is increased, thereby creating a larger pressure oscillation.
Pryor et al (61) compared two oscillating PEP devices (Flutter® & RC Cornet® with
PEP, AD, and ACBT in adolescents and adults with CF. This 12 months study was
completed by 53 of 75 recruits, with no significant differences between ACTs for any
14
outcome. At present expert clinical opinion suggests that the RC Cornet® may be
used in a similar manner to other oscillating PEP devices.
Physiotherapy practice
The RC Cornet® can be held at any angle during treatment, so it may be used with the
patient in sitting or recumbent positions. A weblink to a description of the protocol for
RC Cornet® can be found in Appendix 1.
Postural Drainage
Postural Drainage in gravity-assisted positions
Postural drainage was first introduced for the treatment of CF in the 1950s and
remained the cornerstone of therapy until the 1980s. Postural drainage consists of
placing the patient in a position that allows gravity to assist in draining mucus from
the periphery of the lungs. The recognised postural drainage positions were published
in 1950 (62).
Many studies use postural drainage as the comparison for the ‘newer’ techniques.
Reisman et al (63) reported postural drainage to be superior to the forced expiration
technique in terms of maintaining FEV1. Beneficial effects of postural drainage on
sputum clearance have been reported (64, 65). However, Mortensen et al (66)
demonstrated no advantage of postural drainage over PEP. Lannefors et al (67)
reported maximal clearance from the dependent lung region during postural drainage
using imaging and inhaled radiolabelled particles, indicating that gravity is not the
only factor influencing mucus clearance during postural drainage. Rather, different
positions result in ventilation changes which can make ACT more effective.
A growing body of research has challenged the efficacy and safety of the traditional
head-down postural drainage positions in infants. Gastro-oesophageal reflux (GOR) is
common in infants, children and adults with CF (68-70). A number of studies have
demonstrated provocation of GOR during head-down tilted postural drainage in
infants, children and adolescents with CF (68, 71, 72). Two additional studies did not
reproduce these results in infants, with no significant differences in GOR between
modified and traditional postural drainage (73, 74); however the head-down position
utilised was not as steep, older infants were studied and they avoided the prone head
down tilted position (73). Multiple episodes of GOR extending into the upper airway
were shown using pH multichannel intraluminal impedance in both head-down and
horizontally positioned children, with the potential for aspiration during treatment
15
(74). Studies to determine the individual contributions and safety profiles of various
techniques (positions, chest percussion and vibration) are urgently needed.
The longitudinal relationships between PD, GOR and clinical outcomes have been
examined in a number of studies. A long-term study has shown that infants with CF
who performed postural drainage had significantly worse lung function and more
radiological changes at five years compared to those who did not use head-down tilt
(75). Similarly, children who performed PEP had superior respiratory function at 12
months compared to those who undertook postural drainage and percussion (43). The
association between GOR, postural drainage and reduced respiratory function has not
yet been studied in adults. Using pH MII recording, Palm et al (76) have shown an
association between higher reflux burden and Pseudomonas infection, however the
studies were unable to demonstrate a causal relationship, with further prospective
studies needed to clarify this issue.
Alternatives to PD are available. A long-term study comparing postural drainage and
autogenic drainage (AD) (33) showed significant differences in the secondary
outcome of Huang score (p=0.04) in favour of AD and strong patient preference for
AD. Other measures including respiratory function tests, exacerbations, and
hospitalisations showed no significant between-group differences.
Other negative effects attributable to postural drainage have also been documented.
Increased dyspnoea related to positions using head-down tilt has been reported
compared to when the same treatment is performed in horizontal positions (23).
Oxygen desaturation during postural drainage with FET has been demonstrated (77),
although other authors stated that they were able to prevent desaturation in a group of
CF subjects with similar disease severity by incorporating periods of relaxed
breathing (78).
Physiotherapy practice
Postural drainage in gravity-assisted positions should not be used in infants with CF
or in patients of any age with symptoms of GOR (68, 71, 72). There is no consensus
as to whether postural drainage has a role in management of other patients. Clinically
silent GOR has been reported in 40% of 11 adults with CF awaiting lung
transplantation (79). The majority of Australian and New Zealand centres no longer
use postural drainage incorporating head-down tilt in any patients with CF. It is
generally accepted that other ACTs are at least as effective and have fewer risks. If
postural drainage is employed, careful individual assessment should be used to
establish whether gravity assisted drainage positions are necessary. Some patients
may not be able to tolerate the recognised positions and therefore a comfortable
16
position in which effective breathing techniques can be carried out is likely to be most
beneficial. It is inappropriate to use gravity assisted positions immediately following
meals. Caution should be exercised in the presence of cardiac failure, severe
hypertension, cerebral oedema, aortic and cerebral aneurysms, severe haemoptysis,
abdominal distension or after recent surgery or trauma to the head or neck.
Modified postural drainage
Modified postural drainage involves positioning for airway clearance without use of
head-down tilt. Positioning to facilitate changes in airflow and breath volumes is
conceptually aligned with contemporary approaches that rely less on gravity and
manual techniques, with greater emphasis on changes in airflow, ventilation, and
active participation of the patient. The recommended positions for infants include:
supine 30o head up, prone horizontal, left and right horizontal side lying, and upright
chest position for apical segment of upper lobes, leaning against therapist/carer
shoulder and avoiding slumped sitting which increases intra-abdominal pressure (68).
A schematic of these positions can be found via the weblink in Appendix 1.
The available evidence suggests that modified postural drainage is at least as
efficacious as positions that use head-down tilt and is superior to traditional postural
drainage in infants. In a five year follow up of infants randomized to either standard
postural drainage or modified postural drainage, the modified group had fewer
radiological changes and significantly better lung function at 6 years of age (75). In an
adult study comparing treatment in head down versus horizontal positions, there was
no difference in amount of sputum expectorated, but patients reported fewer side
effects in horizontal positions (23).
Physiotherapy practice
In infants and small children when active participation is not possible, modified PD is
the optimal treatment choice (75). The infant’s head should be well supported,
avoiding shaking movements during treatment which has been associated with
adverse outcomes in premature infants (80). Treatment should be commenced at least
1- 2 hours after a feed. A maximum of 20 minutes per treatment session is
recommended.
Percussion and vibration
Percussion (or chest clapping) involves clapping of the chest wall at a frequency of
approximately 3-6 Hz in order to produce an energy wave, which is transmitted
17
through the chest wall to the airways (6). Percussion is a useful technique to help
mobilise mucus and may stimulate increased tidal volumes and coughing in infants
and children. It is performed using a cupped hand with a rhythmical flexion and
extension action of the wrist. In adults percussion can be done with one or two hands.
In infants percussion is performed using two or three fingers of one hand.
Vibrations involve shaking of the chest wall. The hands are placed on the chest wall,
and during expiration a vibratory action in the direction of the normal movement of
the ribs is transmitted through the chest wall. Vibration produces a similar frequency
of oscillation to percussion but produces higher expiratory flow rates than PEP or
oscillating PEP (81). These effects may increase mucus transport.
The clinical effects of percussion and vibration on airway clearance are unclear.
Sutton and colleagues (82) did not find any increase in tracheobronchial clearance
when manual percussion and vibration were added to postural drainage (82).
However, addition of percussion and vibration to a regimen that included postural
drainage and the ACBT resulted in significant improvement in the FEV1/FVC ratio on
the day following treatment compared to treatment which did not include these
manual techniques (83). Percussion may increase hypoxemia (40) but this may be
prevented if combined with thoracic expansion exercises (78). Tannenbaum et al (84)
demonstrated a short-term decline in respiratory function immediately after manual
techniques were applied to children with CF undergoing anaesthesia. A literature
review by Gallon (85) suggests that percussion is only indicated in patients with
excessive sputum production.
Physiotherapy practice
Percussion and vibrations are used as an adjunct to postural drainage. Percussion
should never be uncomfortable and should be done over a layer of clothing or other
cushioning fabric to avoid sensory stimulation of the skin. Single-handed chest
clapping is advocated if self treatment is being undertaken. If the physiotherapist is
concerned that percussion may cause hypoxemia, the patient should be monitored
with a pulse oximeter. Vibrations should never be uncomfortable and should be
adapted to suit the individual patient. Percussion and vibrations should be used when
patients are unable to participate actively in ACT and require passive treatment (D).
Contraindications: Patients with severe osteoporosis, frank haemoptysis, fractured
ribs and chest injuries. Rib fractures were reported in a neonate with hyaline
membrane disease following percussion (86).
Precautions: Caution should be used in patients with hyper-reactive airways, severe
18
bronchospasm and osteopenia.
Other airway clearance techniques
High frequency chest wall oscillation and intrapulmonary percussive ventilation are
alternative airway clearance techniques which are commonly used in the United
States and Europe respectively. These techniques are not often used in clinical
practice with CF in Australia or New Zealand. The evidence and indications for these
techniques are presented in this section.
High Frequency Chest Wall Oscillation (HFCWO)
High frequency chest wall oscillation (HFCWO) is a patient-delivered form of airway
clearance therapy consisting of an inflatable vest and an air-pulse generator. It is also
known as high frequency chest compression (HFCC). The vest inflates to a nearly
constant background pressure with a superimposed frequency of air pressure
oscillations throughout inspiration and expiration (87). It has been proposed that
HFCWO assists sputum removal by increasing airflow at low lung volumes;
increasing expiratory flow bias, resulting in an increased annular flow of mucus
toward the mouth; and decreasing viscoelasticity of mucus by reducing cross-linking
(88).
A recent, large multi-centre randomised controlled trial conducted in Canada
compared the effects of PEP and HFCWO over 12 months in adults and children with
CF (89). In the HFCWO group the number of pulmonary exacerbations was
significantly greater and the time to first exacerbation was significantly shorter. The
groups were similar for lung function and quality of life (90-94).
Physiotherapy practice
High quality data does not support the use of HFCWO as a routine airway clearance
technique for the majority of individuals with CF.
If used for individuals who are not able to perform other ACTs, HFCWO is usually
commenced at low pressures and frequencies and then increased to therapeutic
optimum as the patient tolerates. Different devices allow a different range of
oscillation and frequency settings. The HFCWO should be paused approximately
every five minutes for huffing and coughing. Newer models have built in settings to
ensure pauses for forced expiration and coughing. The cost of HFCWO is prohibitive
for many patients and thus it is in use by a small proportion of patients with CF.
19
Contraindications: Unstable neck injury, intravenous port being accessed under vest,
pulmonary embolism, lung contusion, current haemoptysis, haemodynamic
instability, rib fractures, large pleural effusion or empyema.
Precautions: End stage disease (end expiratory volume may fall below closing
capacity), intravenous port under the vest (not currently accessed), recent oesophageal
surgery, distended abdomen, bronchospasm, osteoporosis, coagulopathy (95).
Intrapulmonary Percussive Vibration (IPV)
Intrapulmonary Percussive Ventilation (IPV) consists of an open breathing circuit
with a pressure-flow converter and a high output nebuliser. During IPV, high
frequency minibursts of gas (at 100-300 cycles/min) are superimposed on the
patient’s own respiration at pressures of 5-35 cmH20. The driving pressure and
frequency are individually titrated to patient comfort and thoracic movement. Three
forms of therapy are provided during IPV; percussive oscillatory vibrations to loosen
retained secretions, high-density aerosol delivery to hydrate viscous mucus plugs, and
PEP to recruit alveolar lung units.
Current evidence suggests that IPV is at least as effective as postural drainage and
percussion in patients with CF (91, 96, 97). It has similar short-term efficacy to the
Flutter® (98). More research is needed to evaluate the long-term efficacy of IPV in
comparison to modern airway clearance techniques.
Physiotherapy practice
Intrapulmonary percussive ventilation is not currently in use in Australia, despite
much experience with its use in Europe. The cost of the equipment required is likely
to remain a barrier to its use in the short-term.
Contraindications: Non-drained pneumothorax
Physical Exercise as Airway Clearance
Physical exercise that increases minute ventilation leads to the mobilisation of
pulmonary secretions and enhances airway clearance (67, 99-101). It is acknowledged
that some people with mild lung disease and good lung function use exercise together
with forced expiration (huffing), coughing and expectoration as stand-alone ACT.
Others with more extensive lung disease and larger volumes of sputum use exercise as
an adjunct to a formal airway clearance therapy regimen.
20
Recent publications lend physiological support to the contribution of exercise to
airway clearance. The contribution of exercise to airway clearance was evaluated in
14 adults with CF (102). Reduced mechanical impedance of sputum was reported
with treadmill exercise but not cycle exercise, however both forms of exercise
improved ease of expectoration. The authors postulated that this positive effect of
treadmill exercise may have been influenced by increased trunk oscillations with
walking. A transient increase in FEV1 and peak expiratory flow rate (PEFR) following
exercise has been demonstrated in CF (103-105), suggesting that exercise may result
in increased flow transients and bronchodilation. This may facilitate the clearance of
secretions and improve ventilation. Coughing induced by exercise also contributes to
its effectiveness as an ACT (106). A meta-analysis including 53 subjects and three
trials found that the addition of exercise to ACTs resulted in a significant increase in
FEV1 compared to ACT alone (107).
Whether exercise can be used as the sole alternative to other ACTs is less clear. In a
crossover trial, 34 children with CF participated in repeated bouts of whole body
exercise combined with expiratory manoeuvres (experimental intervention) compared
with breathing exercises and manual expiratory compressions (control). Sputum
clearance was the same for both conditions. The mean improvement in lung function
was significantly greater for the exercise group. Treatment satisfaction also favoured
the exercise group, suggesting that bouts of vigorous and age-appropriate exercise
combined with forced expiration strategies may be a suitable alternative for airway
clearance in some children with CF (108). In hospitalised subjects with CF, treatment
using conventional chest physiotherapy resulted in greater weight of expectorated
sputum than exercise alone (100). In contrast, Cerny and colleagues (106) found that
there were no differences in expectorated sputum weight or lung function in
hospitalised subjects who performed ACT alone or exercise alone. One crossover trial
has compared the effects of exercise, gravity-assisted drainage and PEP on mucus
clearance using inhaled radioactive tracer (67). All treatments incorporated the FET.
Although there were no statistically significant differences between the treatments,
there was a trend to lower mucus clearance following the exercise treatment.
Physiotherapy Practice
Clinically, physical exercise is used as an ACT to achieve the following:
reduce mechanical impedance of mucus
mobilise mucus
open up collapsed or plugged airways by increasing ventilation
increase expiratory flow which loosens mucus from the airway wall via shear
21
forces
increase resting lung volumes
increase regional ventilation via gravitational effects by exercising in different
positions such as upright, sitting, supine, side lying or prone lying
Patients with milder lung disease often prefer to carry out physical exercise before
ACT, as it mobilises secretions and makes ACT more effective, where as those with
advanced bronchiectasis and large volumes of daily sputum need to do ACTs before
being able to enjoy exercise. Use of short interval training (eg circuits) can be
beneficial for effective changes in airflow combined with pauses for breathing
control, forced expirations, and coughing if needed.
Suitable forms of exercise to promote mucociliary clearance include: walking,
running, jogging, horse riding, swimming, bicycling, rowing, dancing, martial arts,
step training, stair climbing, skipping, trampoline jumping, sailing, water and snow
skiing, snowboarding, surfing and other whole-body, sustained forms of exercise.
Team sports such as hockey, soccer, football, basketball, netball, polo etc combine all
the physiological benefits of exercise while incorporating group and social activity
which in turn promote regular participation in physical exercise.
Forced expirations and expectoration should be interspersed with physical exercise in
order to optimise ACT. More detail regarding exercise assessment and prescription
can be found in Chapter 4.
Clinical Decision Making regarding Airway Clearance
Techniques
There are a number of evidence based ACTs available for use in CF. In order to
maximise adherence and physiotherapist / patient co-operation, it is recommended
that an appropriate individualised physiotherapy program is developed using sound
clinical reasoning and input from the patient and their family. This has the benefits of
allowing the individual with CF to take ownership of their health and fosters
engagement with the team. The individualised program should include ACTs and
emphasise the importance of physical activity. Clinical and social factors that will
influence the decision about the most appropriate airway clearance regimen for the
individual include; age, independence, patient preference, cooperation, adherence,
financial status, family or social support, culture, and clinical status.
22
Recommendations
1. Airway clearance techniques should be performed across the lifespan in
CF (C).
2. The ACBT is an effective form of airway clearance and can be used by
people with acute and chronic lung disease independently or in
conjunction with other airway clearance techniques (B).
3. PEP therapy, oscillating PEP and autogenic drainage are effective forms
of airway clearance which may be performed independently (B).
4. Postural drainage in head-down positions should not be used routinely in
infants with CF (B) or in patients of any age with known or suspected
GOR (C). Modified postural drainage is recommended in infants and
young children where active participation in airway clearance therapy is
not possible (B).
5. Physical exercise may be used to reduce mechanical impedance of sputum
(B), achieve short term improvements in respiratory function (A) and
improve ease of expectoration (B).
Practice Points
Active cycle of breathing technique may be useful in patients where other
techniques are contraindicated, eg haemoptysis.
PEP therapy and oscillating PEP should be avoided if there is suspected
untreated pneumothorax.
When physical exercise is used as an airway clearance technique it should
be accompanied by modulated forced expirations and coughing to
optimize cephalad movement of secretions and evacuation from the
bronchial tree.
23
3 Inhalation Therapy as an Adjunct to Physiotherapy
Inhalation therapy is a significant component of the management of the respiratory
sequelae associated with cystic fibrosis (CF). It is a multidisciplinary area of practice,
with the input from each discipline varying between CF centres. Airway clearance
techniques may be enhanced with effective inhalation therapy and inhalation therapy
may be enhanced by effective airway clearance techniques. As a result,
physiotherapists should be adequately skilled in the area of inhalation therapy in order
to maximise the effectiveness of both treatments.
Inhalation Therapy Technique
(including Adjuncts to Inhalation Therapy)
The main determinants of deposition pattern for nebulized medications are breathing
pattern during inhalation, droplet size and age/condition of the lung (109). It is widely
accepted that a quicker breath results in greater central deposition (109). A slower
breath results in a more peripheral deposition pattern, improved homogeneity of the
deposition pattern and increased overall drug deposition (110). Slow steady breaths
with occasional deep breaths have therefore been traditionally recommended to
promote improved deposition (111). The specific device being utilised however may
affect the optimal breathing pattern (112). Adaptive aerosol delivery (AAD) devices
have the ability to alter their output in response to the patient’s breathing pattern. By
tailoring the inhalation time to the individual patient’s ability, there is better lung
deposition, reduced expiratory loss, reduced treatment times and better adherence
(113, 114). Physiotherapists need to be aware of the differences between nebuliser
types (eg jet vs mesh) and their impact on inhalation technique, dose received and
deposition pattern.
A study reported in abstract form suggests that the uniformity of drug deposition is
not substantially altered by side lying in healthy participants or people with CF (115).
This study confirmed that the upper lobes are relatively under dosed, receiving
approximately 40% of the density of deposition in the non-apical regions. Alternate
side lying during inhalation therapy can improve apical deposition in healthy lungs
(13%) and to a lesser extent in mild CF lung disease (4%).
Combining ACTs and inhalation therapy is one way the time-related burden of care
may be reduced in CF. However the literature provides limited evidence on the impact
of this practice. A study presented in abstract form has reported that inhaling
hypertonic saline during AD compared to prior, shortened the treatment session
24
without compromising the quantity of sputum clearance (116).
Combining PEP with inhalation therapy in patients with CF results in significantly
lower lung deposition, a reduction in the inner-outer ratio and no difference in the
apical-basal ratio of deposition (117). This indicates that although there was less
aerosol deposited in the lungs, there was a redistribution of the aerosol towards the
periphery. Whether this would impact on the effectiveness of the inhaled medication
remains unclear. A small study by O’Connell and colleagues (118) with four
participants previously intolerant of hypertonic saline demonstrated improved
tolerance in terms of chest tightness, cough and sore throat with the addition of PEP in
combination with hypertonic saline inhalation.
Frischknecht-Christensen et al (119) explored the introduction of a PEP device
(facemask PEP) with the use of inhaled β2 agonists via a metered dose inhaler (MDI).
The study showed improved bronchodilation, dyspnoea, cough and mucus production
when compared to administration of the β2 agonist alone. Stites et al (120) reported
that the use of HFCWO in combination with inhalation therapy did not result in
increased deposition of an inhaled solution compared to inhalation following standard
chest physiotherapy. However in patients with chronic obstructive pulmonary disease
(COPD), addition of the RC-Cornet® oscillating PEP device during nebulisation of
ipratropium bromide resulted in improved bronchodilation (121). These conflicting
results highlight the need for further research into the combination of ACTs and
inhalation therapy.
Physiotherapy Practice
There is no negative impact on lung deposition if people with CF nebulise in side
lying, using two minute intervals on each side. In clinical practice it is likely that
patients will choose to nebulise a full dose on one side and lie on the other side for the
next dose, or alternate the starting side and change when half the nebulisation time
has been completed. In the second scenario care must be taken to alternate the starting
side, in light of the known bias towards greater initial nebuliser output. Given that
many patients prefer to nebulise in side lying due to comfort and convenience,
permitting nebulisation in side lying may improve treatment adherence.
The combination of PEP with inhalation therapy (hypertonic or isotonic saline and
occasionally salbutamol) is commonly prescribed by physiotherapists around
Australia and New Zealand. Some physiotherapists also combine inhalation therapy
with positioning, and breathing techniques such as the ACBT or AD. There is
insufficient research investigating the combination of inhalation therapy and airway
clearance to make recommendations regarding this practice.
25
The combination of inhalation therapy with ACTs could be considered in those
patients who do not regularly perform any other form of airway clearance, or where a
large number of nebulized medications are prescribed. The incorporation of
physiotherapy adjuncts to inhalation therapy also has the potential to improve therapy
compliance. This may include positioning other than upright and the inclusion of
ACTs during inhalation therapy. It is recommended that the incorporation of adjuncts
within inhalation therapy be reviewed regularly and written instructions be provided.
Mode of Delivery
Given the varying physico-chemical behaviours of the drugs nebulized in CF, it is
important to use a specific nebuliser/compressor combination that has been proven to
be effective for that preparation. By changing from an inefficient nebuliser system to
an efficient one, there can be up to a ten-fold increase in the dose delivered (109).
Aspects that need to be considered are: flow/pressure characteristics of the driving
source, the tubing connecting the nebuliser and the driving source, the nebuliser itself
and the user interface (mask versus mouthpiece) (109).
Lannefors (122) recommended that when a medication may be administered via either
a dry powder inhaler, MDI or a nebuliser, the dry powder inhaler should be the
preferred option due to ease of use. The exception to this is for the administration of
inhaled steroids, where a MDI and a spacer should be utilised to reduce systemic
bioavailability and reduce growth of candida albicans in the mouth. In those patients
unable to use a dry powder inhaler (eg poor respiratory function leading to poor
inspiratory flow rates, cognitive impairments or muscular dysfunction) a MDI and
spacer should be chosen. For antibiotics, Tobramycin Inhalation Powder (TIP)
achieves a faster delivery time, greater portability and convenience, with low systemic
absorption and equivalent efficacy to Tobramycin Inhaled Solution (TIS); however it
may be associated with a greater incidence of cough (123).
For bronchodilators, the European Respiratory Society recommends any certified
nebuliser system as being appropriate and that a facemask or mouthpiece may be
used. A mouthpiece is preferred for administration of anticholinergics to avoid
irritation of the eyes (109). A mouthpiece may not be appropriate if the sinuses are a
target of therapy (124).
Ultrasonic nebulisers should not be used for dornase alfa as they can alter its physico-
chemical properties, rendering it ineffective (125). A list of recommended nebulisers
is provided in the dornase alfa consumer medicine information sheet. Disposable jet
26
nebulizers may not be as effective, with respect to inhalable mass and output rate, as
reusable jet nebulizers for the administration of tobramycin (126). Vibrating mesh
nebulizers may reduce treatment time compared to jet nebulizers (112, 127).
It is often recommended that patients use an expiratory filter when nebulising
antibiotics (111). This is primarily due to the potential side effects for other people. In
the hospital setting, it is recommended that a high efficiency expiratory filter be used
to prevent contamination of the environment and allergic reactions from staff. In the
community, people with CF should take their antibiotics in a well ventilated room by
themselves. If they have a sibling with CF, an expiratory filter should be used (128).
Physiotherapy Practice
Where possible, nebulized medication should be taken via a mouthpiece to maximise
delivery of the drug to the airways and avoid nasal filtration (129). The exceptions to
this are: young children who may be unable to coordinate the use of a mouthpiece
effectively, or where sinuses are a target of therapy, or those acutely unwell with
shortness of breath. Bronchodilators should be delivered by MDI and spacer (109)
except in situations where this may be clinically ineffective, eg paediatrics or acutely
unwell patients with shortness of breath (nebulisers should be used in these
circumstances). Metered dose inhalers with spacer should be used for the
administration of inhaled corticosteroids. Patients should be encouraged to rinse their
mouth with water and gargle afterwards to reduce the risk of thrush (129).
Mixing Inhaled Medications
The concept of mixing nebulized medications simultaneously is often raised in an
attempt to decrease the time burden associated with use of multiple inhaled agents.
The European Respiratory Society (109) advise against mixing medications due to
concerns about both safety and effectiveness, unless the specific mixture (including
preservatives if applicable) has been studied. Whilst some medications may be
chemically stable once mixed, there is often a lack of research regarding the
aerodynamic properties of these mixed solutions (130). A European Consensus
Statement has published very limited data regarding physico-chemical stability of
combinations of inhalation solution (112).
Physiotherapy Practice
Inhalation therapy is time-consuming and patients may wish to mix inhaled
medications in an effort to reduce the time-burden of CF health care. Given the lack
of research regarding the effect of mixing nebulized medications on aerodynamic
27
properties and chemical composition, it is recommended that medications are not
routinely mixed. Physiotherapists should direct patients to their CF doctor or
pharmacist for advice on the mixing of inhaled medications.
Timing and Order of Inhaled Medications
A Cochrane systematic review investigated whether changing when dornase alfa is
inhaled in relation to ACT or time of day impacts the overall effect of the airway
clearance session in people with CF (131). The searches identified 4 eligible trials that
examined inhalation of dornase alfa before versus after ACT . Meta-analysis of all the
available data showed that inhalation after instead of before airway clearance did not
change FEV1 (mean difference -0.03 litres, 95% CI -0.08 to 0.03 litres). Similarly,
FVC and quality of life were unaffected. However, FEF25 was significantly better with
dornase alfa inhalation before ACT, based on the pooled data from two small studies
in children with well preserved lung function (132, 133).
It has been demonstrated that a longer time interval between administration of dornase
alfa and airway clearance (eg inhalation before bedtime) is more effective than
inhalation immediately preceding treatment (134). There appear to be no detrimental
effects on sleep quality or nocturnal cough associated with administering dornase alfa
before bedtime (135). Given the long-term beneficial effects of dornase alfa on lung
inflammation in CF (136), ensuring that patients are adherent to daily inhalation is of
greater importance than the time of administration. It is therefore currently suggested
that dornase alfa be administered before bedtime if that is acceptable to the patient,
whilst acknowledging that this regimen may need to be altered for individuals in order
to optimise adherence.
The British Thoracic Society Nebuliser Project Group (111) recommended that
bronchodilators be administered prior to ACTs, however there is to date no objective
evidence that this enhances the benefits. If patients have known bronchodilator
responsiveness then bronchodilator therapy and ACTs should precede the delivery of
other inhaled medications (137). Nebulized antibiotics should be administered after
airway clearance and bronchodilators, in order to maximise the drug deposition within
the lungs and to protect against bronchoconstriction (128). It does however need to be
recognised that some patients with CF demonstrate increased airway obstruction post
bronchodilator therapy due to a reduction in smooth muscle tone (138).
The timing of hypertonic saline inhalation in relation to ACT (before, during or after)
does not appear to have a substantial effect on lung function after a single treatment
28
session (139). However, participants were more satisfied with the entire treatment
session when hypertonic saline was inhaled before or during ACT and perceived these
timing regimens as more effective. Participants who repeated the study tended to
retain their preferred timing regimen. .
Physiotherapy Practice
Bronchodilators should be administered prior to ACTs if patients have previously
demonstrated a benefit from bronchodilator therapy. It also generally advised that
saline (isotonic or hypertonic) be taken either before or during ACTs.
Children with well preserved lung function who use dornase alfa could be advised to
inhale it 30 minutes before ACT because this may be more beneficial for small airway
function, although other outcomes may not be affected. Apart from this, the timing of
dornase alfa inhalation can be largely based on pragmatic reasons or individual
preference with respect to the time of ACTs and time of day. Efforts should be made
to maximise the time interval between administration of dornase alfa and ACT; this
may involve administration of dornase alfa at bedtime.
For people with CF who use hypertonic saline, clinicians should encourage inhalation
before or during ACT. It is likely that a patient’s preferred timing regimen remains
constant over time and does not require repeated review once the daily routine is
established.
When providing advice to patients on order of inhaled medications, especially in
relation to timing of ACT, any specific recommendations or instructions that are
given by the prescribing physician or pharmacist should be followed.
Nebuliser Maintenance
Nebuliser devices are sources of bacterial contamination and can lead to an increased
risk of patient infection. It has been suggested that the inhalation of aerosols
contaminated with gram-negative bacteria generated from home-use nebulisers may
be a primary route for bacterial colonisation of the lung in CF (140).
There is no one method which has been recommended for cleaning home nebulisers.
Rosenfeld et al (141) suggested that soaking and rinsing with tap water for at least one
minute followed by air drying is an effective cleaning method. This finding was
published in an editorial and the weight of the evidence is unclear. A small study
reported that cleaning frequency was linked to home nebuliser contamination (i.e.
cleaning after each use was associated with a lower rate of nebuliser contamination)
29
but that there was no significant difference in contamination with respect to cleaning
or drying technique (142). Reychler et al (143) found that cleaning via a dishwasher
(temperature of 70oC) or immersion for 20 minutes in a litre of Hexanios 0.5%
hypochlorite solution or hot water (40oC) combined with detergent were all effective
against the common pathogens found in patients with CF. Acetic acid (vinegar)
however was found to be ineffective against Staphylococcus aureus and
Stenotrophomonas maltophilia.
Physiotherapy Practice
All physiotherapists should be aware of the possible contamination of inhalation
therapy equipment and the implications this may have for the patient’s health.
Cleaning methods should be reinforced as part of routine assessment and treatment. A
review of the patients’ cleaning technique may be incorporated into annual inhalation
therapy reviews, outpatient clinic appointments or during an inpatient admission and
this may be performed by any member of the CF health care team with appropriate
and relevant knowledge.
It is recommended that nebuliser equipment and inhalation devices be cleaned after
every use according to the techniques recommended by the local CF centre’s infection
control department. Nebuliser bowls should be replaced frequently according to the
manufacturer’s guidelines and pumps should be serviced at regular intervals
according to the manufacturer’s instructions.
Clinical Monitoring of Inhalation Therapy
Most inhaled medications have known side-effects. For instance, hypertonic saline,
mannitol, colistin and tobramycin may all cause bronchospasm (144). The British
Thoracic Society Nebuliser Project Group (111) recommended that patients should be
assessed in hospital for their first trial of isotonic colistin and should be pre-treated
with a bronchodilator. This group also recommended that spirometry be performed
before and after the test dose because bronchospasm can occur within 15 minutes in
over 85% of people. There is a detailed trial regimen that needs to be adhered to for
patients commencing inhaled mannitol due to the potential for bronchospasm (145).
The European Respiratory Society recommends that patients should be re-assessed
one month after commencing their treatment and then be re-assessed annually
thereafter (109). It has been demonstrated that the uptake of education in inhalation
therapy techniques is improved if repeated educational sessions are performed and if
the patient is asked to demonstrate their technique at these sessions (146).
30
Physiotherapy Practice
All new medications should be trialled in the presence of a suitably qualified health
professional. This review should include: preparation of nebuliser equipment and
medication, positioning and breathing technique and monitoring of potential side
effects. An inhalation therapy review should be performed annually by a designated
member of the CF health care team with appropriate and relevant knowledge in
inhalation therapy.
During an inpatient admission or during a clinic visit it may be appropriate for a
physiotherapist to review the patients’ inhalation therapy technique including
reviewing their positioning during inhalation therapy and making recommendations to
improve the effectiveness of the therapy. All physiotherapists should be aware of the
possible side effects of medication prescribed for inhalation therapy and the possible
implications of ineffective use of inhaled medications.
Recommendations
6. Where possible, nebulized medication should be taken via a mouthpiece
(C). The exceptions to this are: young children who may be unable to
coordinate the use of a mouthpiece effectively, or when therapy is
targeted at the sinuses, or those acutely unwell with shortness of breath.
7. To optimise dose delivery and treatment time, inhalation technique
should be adapted to the specific device being used, including
consideration of body position and concurrent ACTs (C)
8. Bronchodilators should be delivered by MDI unless there is clinical need
for nebulisation (C).
9. Metered dose inhalers with spacer should be used for the administration
of inhaled corticosteroids. Patients should be encouraged to rinse their
mouth and gargle with water afterwards to reduce the risk of thrush. (B).
10. Hypertonic saline may be administered before or during ACTs (B).
Practice Points
Positive expiratory pressure devices can be used whilst nebulising
hypertonic saline, isotonic saline or bronchodilators. Positive expiratory
31
pressure devices are not suitable for nebulising antibiotics or dornase alfa.
An expiratory filter should be used when nebulising antibiotics. Where
this is not possible, antibiotics should be administered in a well-ventilated
room with the person alone.
Nebuliser and compressor combinations with demonstrated efficacy for
specific medications should be used where possible.
Inhaled medications are not routinely mixed.
Efforts should be made to maximise the time interval between
administration of dornase alfa and ACT; this may involve administration
before bedtime if acceptable to the patient, although timing should be
individualised to optimise adherence.
32
4 Exercise
Exercise is a cornerstone of therapy for people with CF. Measures of exercise
capacity predict survival in children and adults with CF (147, 148) and those with
better physical fitness have better quality of life (149). There is some evidence that
structured exercise programs for people with CF improve fitness, thoracic mobility,
maintain bone mineral density and preserve or slow the rate of pulmonary decline
(150-156). For this reason it is recommended that all patients should be encouraged to
exercise on most days of the week (12). More recently it has become apparent that
structured exercise programs may not be the only approach to achieving these
benefits. A study in over 200 people with CF conducted over nine years showed that
patients with higher levels of physical activity in daily life (encompassing all
activities, not just exercise programs) had a slower rate of decline in FEV1 than those
who were less active (157).
The physiotherapist has an important role in the assessment of exercise capacity,
exercise prescription and facilitation of physical activity. Research has shown
exercise testing and training, both in inpatient and outpatient settings, to be safe (158).
Assessment of exercise capacity
Assessment of exercise capacity is an important practice for the evaluation of
functional capacity, response to treatment and disease progression (147, 159-161). For
physiotherapists, exercise tests also provide the basis for exercise prescription.
Assessment of exercise capacity should be undertaken prior to the prescription of a
new exercise regimen, and as a reassessment tool to assess the efficacy of the exercise
prescription. It has been suggested that an exercise test should be performed at least
annually to document changes in exercise capacity (162). However studies indicate
that exercise testing is currently underutilised by physiotherapists (163, 164).
Tests of exercise capacity include formal laboratory assessments of maximum
exercise capacity (cardiopulmonary exercise tests) and field tests. Maximal exercise
testing is a specialist role which is usually performed by respiratory scientists and is
currently outside the scope of routine physiotherapy practice. Field tests are useful
alternatives which provide relevant clinical information regarding exercise capacity
(163, 165). This document focuses on field tests of exercise capacity which are in
common use in the CF population. These are the six minute walk test, the three
minute step test and the modified shuttle test.
33
Six minute walk test
The six-minute walk test (6MWT) is an easy to administer, well-tolerated, self-paced
exercise tolerance test, reflective of functional exercise capacity (166). Patients are
required to walk as far as they can in six minutes. In patients with severe disease the
6MWT is suitable for assessment of response to a new intervention and as a one-off
assessment of exercise capacity or predictor of prognosis (166).
The 6MWT has traditionally been an important tool for preparation for lung
transplantation in CF. In a study reviewing 145 patients with lung disease (41 of these
with CF) it was concluded that a distance of less than 400 metres achieved in the
6MWT is a reasonable indicator to consider referral for lung transplantation (167).
The 6MWT is also a useful tool for assessing exercise-induced oxygen desaturation
(168) and has a strong correlation with peak oxygen uptake in end-stage lung disease
(169) and in severely ill children (170).
The 6MWT has limited utility in assessing exercise tolerance in people with mild CF
lung disease. Distance walked is poorly correlated with maximal exercise capacity for
those with mild lung disease (171) and adults with mild to moderate CF lung disease
cover similar distance to healthy volunteers (168, 172). This suggests in those with
mild lung disease there may be a ceiling effect for the 6MWT. Another limitation of
the 6MWT is that it is self-paced and thus dependent on the patient’s motivation.
Physiotherapy Practice
The protocol for the 6MWT has been published (166).
The 6MWT can be performed in both paediatric and adult CF populations. The main
outcome of the 6MWT is the distance walked, however it is not yet known what
minimum change in distance is required to represent a real clinical change in patients
with CF. It is recommended that the same track is used when re-assessing a 6MWT in
the same individual, as distance can be affected by the track layout. Two 6MWTs are
needed on each measurement occasion in order to reliably measure exercise capacity
in adolescents and adults with CF and overcome the known learning effect (173).
Normative values for 6MWT distance are available for children, adolescents and
adults (168, 174, 175); however as normative values may differ according to
population and region, local reference values are preferable.
Contraindications: The 6MWT is contraindicated in patients presenting with cardiac
conditions that are unstable in the month prior to testing.
34
Precautions include a resting heart rate of ≥120 beats per minute, systolic blood
pressure of more than 180 mmHg, or a diastolic blood pressure of more than 100
mmHg.
The 3-minute step test
The 3-minute step test (3MST) is a submaximal test of exercise capacity used to
determine exercise tolerance in individuals with CF (176-178). It is an externally-
paced test which involves stepping on and off a 15cm step for three minutes at a rate
of 30 steps per minute. It is a quick, portable and simple exercise test. The 3MST is
highly reproducible, provokes a greater increase in heart rate and breathlessness than
the 6MWT and equivalent levels of oxygen desaturation (176).
The 3MST may be useful to assess the need for supplemental oxygen in individuals
with moderate to severe lung disease as it is of sufficient intensity to provoke oxygen
desaturation in this group (177, 179). It is also responsive to change over the course of
antibiotic treatment in children with CF (178) and therefore may be a useful
alternative or adjunct to lung function tests to provide positive feedback during the
course of the admission. The 3MST is not a maximal test and it may be more sensitive
to change in heart rate and oxygen saturation in patients with CF and moderate-severe
lung disease than in those with mild disease (177, 179).
The 3MST may also be helpful to use as a screening test for patients at risk of poorer
outcomes. In a study reviewing 101 adult CF patients, those who experienced oxygen
desaturation on testing showed a greater decline in FEV1 and had more hospitalised
days over 12 months than their peers who did not experience desaturation on 3MST
(179). In addition, the 3MST can be effectively assessed using remote telemonitoring
(180), which may create opportunities for exercise assessment of geographically
isolated patients or during periods of home-based intravenous antibiotic therapy
To date, most studies pertaining to the 3MST have been conducted in children, with
only one study examining the predictive value of this test in adults with CF. Further
study is required to determine the sensitivity of the 3MST to clinical change in both
the paediatric and adult setting.
Physiotherapy Practice
The protocol for the 3MST can be found in Appendix 2.
The 3MST can be performed in both paediatric and adult CF populations. The main
outcomes of the 3MST are maximum heart rate, minimum oxygen saturation and
35
breathlessness scores. If the entire test is unable to be completed, the time or number
of steps completed should also be recorded. Due to its simplicity, short time and
minimal space requirements, the 3MST is suitable to perform across inpatient,
outpatient and home-based settings. There are no normative values for the 3MST
(177, 178).
Contraindications: As for the 6-minute walk test
Precautions: The 3MST should be avoided in individuals with significant lower limb
joint arthropathy (176).
Modified Shuttle test (walk/run)
The modified shuttle test (MST) is an externally-paced, incremental test of maximum
exercise capacity. The participant is instructed to walk or run around two cones placed
nine metres apart in time to pre-recorded auditory cues. The required speed becomes
progressively faster as each level is completed. The test is terminated when the
participant is no longer able to maintain the required pace or if they fail to be within
0.5 metres of the marker cone, at the time of the ‘beep’ on two consecutive shuttles.
The original 15-level MST (181) has been extended to a 25-level MST (182), as 15%
of CF adults and 46% of healthy volunteers completed the 15-level MST (183),
however to date information on the MST-25 is only available in abstract form.
The MST is sensitive to change when assessing the effects of intravenous antibiotic
treatment (184), and was shown to be more sensitive to change than FEV1 following a
hospital admission in adults with CF (159). It is a valid measure of exercise tolerance
in children (165, 185) and adults (181). The MST has been shown to have high
reproducibility and responsiveness in measuring exercise capacity in the adult CF
population (159).
The MST has the advantage of being an incremental test and therefore provides
sufficient challenge for those with milder disease. In clinical practice this test could
be used in an annual review setting, during an inpatient admission and when
determining oxygen requirements/desaturation patterns prior to prescribing an
exercise programme (181).
Physiotherapy Practice
The protocol for the MST can be found in Appendix 3.
The MST is appropriate to use in both paediatric and adult CF populations. The main
outcomes of the MST are distance covered, peak heart rate, minimum oxygen
36
saturation and breathlessness. If there is a change of greater than 40 metres in the
MST then it is likely that real clinical change has occurred (159). As the MST is an
incremental exercise test that elicits peak or near-peak exercise responses, it may not
be suitable for unwell patients. There are no normative values for the MST, however,
estimates of peak oxygen consumption can be made for adults with CF from distance
covered in the 15-level MST (159, 181, 184).
Choosing a test of exercise capacity
Exercise tolerance testing should be considered as a standard element of CF care
across the range of ages and disease severity. The choice of test should take into
consideration the following factors:
Age of patient – the 6MWT and MST have been validated in both adults and children
with CF. The 3MST has been validated in children but there are limited data
regarding its role in the assessment of exercise capacity in adults with CF.
Lung function severity – the MST can be used in patients across the range of disease
severity but may be overly challenging for those with severe disease. The 6MWT is
an excellent choice for those with severe disease but may have a ceiling effect in
those with mild to moderate lung disease. Likewise, the 3MST may only be sensitive
to changes in heart rate and oxygen saturation in those with moderate to severe
disease.
Clinic space – the 3MST has minimal space requirements. The MST requires a 10
metre track, whilst the 6MWT ideally requires a 30 metre track and therefore may not
be practical in some settings.
Access to equipment – all tests require a pulse oximeter to measure oxygen saturation
and heart rate. Equipment for the 3MST and the 6MWT are usually readily available.
The MST requires a recording of standardised instructions and timed audio cues.
Patient’s motivation level – both the 3MST and the MST are externally paced and
may be most suitable for patients with low motivation.
Time availability – the 3MST is very quick to perform. The 6MWT may require two
tests to be performed due to a learning effect.
Clinical status – the 3MST is easy to perform during a hospital admission and is
sensitive to change in clinical status in paediatric patients. The MST provides more
feedback to the patient regarding increase in distance covered from admission to
37
discharge and is sensitive to clinical change during hospitalisation. The 6MWT may
not be practical at hospital admission in patients who are very unwell.
At present, the literature does not suggest that there is one ‘best’ exercise test for
people with CF. It is therefore recommended that the choice of test be based on the
patient’s current health status, their motivation level, time, staffing and space
availability in the clinic area (172).
Exercise prescription in CF
The ideal exercise prescription for people with CF has not been established. Both
aerobic training (endurance exercise) and anaerobic training (resistance training or
high-intensity, short duration training) are beneficial (150). Aerobic training results in
improved maximum exercise capacity, strength and quality of life (186, 187).
Anaerobic training has positive effects on lactate levels, peak power (188) and fat-free
mass (187). Both types of exercise may have positive effects on respiratory function
(2, 187). Exercise programs have beneficial effects both during admission for acute
exacerbations (106, 187, 189) and in the stable outpatient (2, 188, 190). It is unclear
whether home-based, unsupervised training programs are as effective as supervised
programs.
Physiotherapy practice
Exercise should be considered and encouraged as part of overall physiotherapy
management in CF. From time of diagnosis, irrespective of age, exercise and physical
activity should be incorporated into the daily routine. For young children, their family
and siblings should also be encouraged to be involved in activity and exercise, to
assist in normalising the activity and making it ‘fun’. At puberty there is evidence of a
drop in physical activity in girls with CF (190-192). This reduction is associated with
a more rapid decline in respiratory function in girls compared to boys (191). Particular
care should therefore be taken during adolescence to tailor exercise programs to the
individual’s interests, environment, time availability and capabilities.
Aerobic exercise prescription should follow the same principles as those used in
healthy individuals (193) and patients with other chronic respiratory diseases (194): In
order to improve aerobic capacity, exercise training must:
occur on at least 3 days (preferably 5 or more days) per week
have a duration of 30 minutes per session, consisting of shorter intervals if
required
increase heart rate to approximately 75% of maximum heart rate.
38
Peripheral muscle force is reduced in people with CF (195). A low-weight, high
repetition training strategy can effectively increase FEV1, strength and body mass in
hospitalised children with CF (187). Resistance training should be performed on
alternate days to allow for recovery. A combination of both aerobic and strength
training is required to achieve maximum benefits from training (150).
People with CF may exhibit exercise-induced oxygen desaturation during training,
even when pulmonary function is relatively well preserved (181). Supplemental
oxygen during training increases exercise duration (196) however whether this results
in improved clinical outcomes is not clear. It is recommended that supplemental
oxygen be used during training in patients whose oxygen saturation falls to below
90% during exercise (197).
Exercise prescription in CF may consist of working with the patient to devise a formal
exercise program or may simply involve provision of guidelines for appropriate
intensity, frequency and duration of training. Formal exercise prescription should be
considered in the following settings:
Reported reduction in exercise tolerance/involvement in normal activities
Known reduction in muscle mass/strength
Osteopenia/osteoporosis
Onset of CF related diabetes
Patients awaiting lung transplantation
Exercise prescription should be tailored to the individual. When prescribing exercise
the following should be considered: age of patient, indications and contraindications
to exercise, interests of the patient, patient motivation, resources at the patient’s
disposal upon discharge, realistic goals, appropriate dosage (frequency, duration,
intensity), and guidelines for monitoring safe exercise levels. In children with CF
significant differences in oxygen uptake (VO2) and heart rate (HR) at rate of
perceived exertion (RPE) levels 4 and 7 using the OMNI scale have been
demonstrated (198). This indicates children can discriminate between exercise
training intensities and that regulation of exercise training can be successfully
achieved using the OMNI scale.
With the emergence of new technologies, alternative training stimuli may be
considered in addition to ‘traditional’ training approaches. Undertaking exercise using
a gaming console has been found to be comparable to both treadmill and cycle
39
exercise in regards to peak heart rate, achieved workload, oxygen saturation and
levels of perceived exhaustion and fatigue (189) and can provide high intensity work
in adults with CF (199). Gaming console exercise was rated favourably in terms of
patient enjoyment, which may in turn aid patient compliance and motivation with
long term exercise (189).
Special consideration should be given to meeting the metabolic demands of exercise
in CF and consultation with the dietitian may be required. Care should be taken to
ensure adequate fluid and salt intake, particularly in warm climates. Specific infection
control recommendations for exercise in gym settings are provided in Chapter 13.
Contraindications: Exercise training should not be performed in patients who are
febrile, or whose cardiovascular status is unstable.
Precautions to exercise training include pulmonary hypertension, cor pulmonale and
haemoptysis. Some patients have exercise-induced bronchospasm and should always
take their prescribed bronchodilators prior to exercising. Care should be taken in
patients with low bone mineral density, particularly with regard to resistance training.
Arthropathy and other musculoskeletal issues should be considered prior to
commencing an exercise program.
Facilitating physical activity in people with CF
To date there are only a few studies that have examined strategies to increase physical
activity in daily life in people with CF (200). Most studies have used a structured
exercise program and measured its impact on physical activity, with variable results.
The limited data available suggest that exercise training programs of longer duration
(more than six months) and those that require self-directed behaviours may have a
greater impact on physical activity in daily life (200). Whether other types of
interventions, such as behavioural and motivational strategies, may have a greater
impact on physical activity has not yet been explored.
The Australian guidelines for physical activity provide the following
recommendations (201):
Children and young people (ages 5 – 17 years) should engage in 60 minutes of
moderate to vigorous physical activity each day. On 1 – 3 days per week they
should also engage in activities that strengthen muscle and bone.
Adults (aged 18 to 64 years) should accumulate 150 – 300 minutes of
40
moderate intensity physical activity, or 75 – 100 minutes of vigorous intensity
physical activity, or an equivalent combination of the two, each week. Adults
should do muscle strengthening activities on at least two days per week.
Specific physical activity recommendations for people with CF have not yet been
published.
Recommendations
11. Exercise is recommended for patients with CF throughout the lifespan
(B).
12. An exercise test should be considered to assess response to therapy in the
inpatient and outpatient settings and as an assessment tool in the
prescription of exercise training programs (C).
13. A 6MWT should be performed as part of the initial assessment for lung
transplantation (C).
14. Exercise prescription should be tailored to the individual and comply
with recommended exercise guidelines (B).
15. Supplemental oxygen should be considered during training in patients
with severe exercise-induced desaturation (C).
Practice Point
An annual assessment of exercise capacity is useful to document changes in
exercise capacity and exercise responses (eg desaturation) over time.
5 Musculoskeletal Complications of Cystic Fibrosis
Musculoskeletal manifestations of CF are frequently characterised by acute or chronic
41
pain and arise as a result of multi-factorial abnormalities in bone mineralization,
altered respiratory mechanics and muscular imbalance secondary to pulmonary
disease. As survival in CF improves, the prevalence of these complications is
increasing and the resulting compromise to physical activity and airway clearance
may deleteriously impact on quality of life and suitability for transplant.
Pain
Reported rates of spinal pain (thoracic, lumbar, cervical) in CF populations are high.
The prevalence ranges from 43% to 94% (202-213), irrespective of clinical status, and
is both substantially higher than age-matched healthy control subjects and presents at
a younger age (203-205, 210, 214). Pain is generally reported to be of moderate
intensity (207-209, 211-213). The prevalence of pain does not appear to be influenced
by disease severity or gender (209). The incidence of chest pain varies from 16% to
64% (203, 208-210, 212, 215) with 30% to 77% reporting pain in more than one
location (209, 212). Although documentation of back pain is increased in those with
increasingly severe lung disease, it is not proportional to the degree of pulmonary
hyperinflation (216). The high frequency of musculoskeletal pain is associated with
decreased quality of life, respiratory symptoms, sleep disturbance, anxiety and
depression together with a reduced ability to perform chest physiotherapy and
exercise effectively (207, 209-213) (203, 217-219). Whilst some patients report
seeking pharmacological and non-pharmacological management to alleviate their
symptoms, approximately 25% of patients with CF fail to seek treatment of pain (203,
208, 210, 213).
Low Bone Mineral Density
Patients with CF have multiple risk factors for inadequate bone mineralisation - poor
nutrition, pancreatic insufficiency and malabsorption, deficiencies in calcium and
vitamins D and K, abnormal fatty acid status, liver dysfunction, reduced weight
bearing activity, delayed puberty, hypogonadism, CF-related diabetes, reduced lung
function, chronic infection, increased intravenous antibiotic use, glucocorticoid
therapy and immunosuppression, CFTR dysfunction in bone cells (220-222) and
abnormal bone remodelling favouring bone resorption (223-227). A significant
proportion of musculoskeletal problems in CF arise secondary to low bone mineral
density (BMD). Studies in children reveal conflicting results about BMD
abnormalities. While several studies suggest normal BMD in healthy well-nourished
children with CF, other studies have shown low BMD in young children with mild
42
lung disease and normal nutritional status with osteoporosis reported in 20-34% of
children and osteopenia in 28-47% (227). These differing results may support the role
for a dysfunction in bone metabolism from an early age (228, 229) or may reflect
difficulties in the interpretation of DXA scans in growing children (222). Longitudinal
studies show bone gains during puberty are decreased in CF adolescents compared to
healthy controls resulting in decreased attainment of peak bone mass (222, 224, 225,
230-234). Loss of bone mineralisation begins in late adolescence and is accelerated
throughout adulthood (222, 224, 235-237) . A meta-analysis found the prevalence of
osteoporosis in adults in CF to be 24% and osteopenia 38% (238) but ranges from 9-
70% across studies (224).
Adolescents and adults who had higher physical activity levels and better aerobic
capacity had higher BMD at the hip and lumbar spine than their less active and less fit
counterparts (226, 233, 234, 236, 239, 240). Duration and intensity of physical
activity is positively correlated with BMD in adolescents and adults with CF (240).
Individuals with more severe lung disease, decreased physical activity and low
aerobic capacity had the lowest BMD, higher prevalence of vertebral fractures and
more severe kyphosis (240).
Vertebral compression and rib fractures
Low BMD, osteoporosis and chronic steroid use increase the risk of fracture in people
with CF. Increased rates of long bone, rib and vertebral fractures have been reported
(234). Vertebral compression fractures and rib fractures appear to be 100 times and 10
times respectively more common in CF compared to the age-matched population
(241). While the majority of the literature suggests fracture risk is normal in children
with CF (242), fracture risk is increased by late adolescence (224, 226). A meta-
analysis found the prevalence of vertebral fractures in the adult CF population was
14% and non-vertebral fractures 20% (238), however rates have been reported to be
higher (38%) in more recent studies (240). Patient reported fracture rates occur in up
to 50% of adults with CF (241, 243, 244). The prevalence of vertebral fractures was
higher in adolescents and adults with severe lung disease compared to mild lung
disease (226, 240). Studies have not found any association between fracture rates and
BMD (233, 240, 245, 246) In addition, a significant proportion of spinal compression
and rib fractures may be underestimated, with radiological investigations revealing 76
asymptomatic fractures in 70 patients with severe lung disease (241).
Chest wall fractures and kyphosis may impair airway clearance, reduce pulmonary
function, reduce mobility, accelerate decline in heath status, contribute to pulmonary
43
exacerbation, decrease quality of life and may affect eligibility for transplantation
(225, 233, 240, 247).
Increased thoracic kyphosis
Studies have reported a high prevalence of abnormal kyphosis angles (>40 degrees),
in CF subjects, varying from 15-74% compared to a normal, age-matched population
(241, 248-250) and in 7% of children under 10 years (251). Although the aetiology of
CF-related kyphosis remains unclear, a number of concomitant factors appear to
influence its development. A higher incidence of vertebral wedging, particularly
>15% thoracic wedging, severity of pulmonary disease (226, 240), age and loss of
BMD have been related to the presence of thoracic kyphosis in patients with CF (241,
250, 252). The development of thoracic kyphosis may also contribute to rib and
vertebral fractures and reduced lung function (226)
The significance of thoracic kyphosis may depend on the extent of reversibility and
postural correction. While some studies found the presence of kyphosis to be stable
and unrelated to pulmonary function in terms of lung volumes or maximal expiratory
flowrates (253), other studies have concluded that the diagnosis of a thoracic kyphosis
is an indicator of deteriorating lung function and a marker of poor prognosis (248,
251). In some patients, the kyphotic deformity may be improved with postural
correction, suggesting that in the absence of a structural kyphosis in CF, changes in
soft tissue structures and muscular abnormality may contribute to the ‘habitual
hunched posture’ secondary to increased work of breathing and excessive coughing
(216).
Muscle strength
Changes in muscle strength, length and neuromuscular recruitment have been
demonstrated in patients with CF. Reduced lean muscle mass in CF subjects is
associated with malabsorption and deconditioning (254, 255). The associated
reduction in peripheral muscle strength and endurance in both children and adults
with CF is primarily reflective of lower muscle mass rather than reduced force-
generating capacity of muscle (256, 257). Intrinsic abnormality of CF skeletal
44
muscles including decreased efficiency of oxidative ATP with abnormal
mitochondrial density and metabolism, have been reported (256). Peripheral muscle
impairment is also noted in response to systemic inflammation and lack of moderate
to vigorous physical activity (258, 259). Impaired respiratory muscle strength may
occur in association with reduced respiratory muscle mass from nutritional
impairment (254). Chronic use of corticosteroids is associated with skeletal muscle
weakness (260).
Conversely, preserved respiratory muscle strength despite chronic hyperinflation may
represent relative training of respiratory muscles in response to chronic loading (261).
This hypothesis was supported by the work of Pinet and colleagues (262) who
concluded that CF patients with FEV1<60% predicted had thicker and stronger
abdominal muscles than did control subjects. It was postulated that this was a
consequence of the heavier respiratory work performed by these patients. de Jong and
colleagues (263) reported reduced peripheral muscle strength in CF patients with
airflow obstruction in the presence of preserved inspiratory muscle strength. In
relatively healthy CF subjects (FEV1 60-124% predicted) few differences were
apparent in muscular performance compared to healthy controls of similar moderate-
high activity levels (264).
Muscle length
Reductions in thoracic cage muscle lengths, in the presence of chronic accessory
respiratory muscle recruitment and associated postural habits that splint the shoulder
girdle, have frequently been hypothesised in CF (265). However, investigative
research is rare. Rose and co-workers (216) report that the habitual flexed posture
caused by altered breathing mechanics are reinforced by chronic coughing and result
in further tightening of muscles and restriction of movement of the shoulder, trunk
and chest. They performed postural examinations on young adults with CF and aged
matched controls, demonstrating three motions in which at least 45% of patients with
CF fell below control measures: scapular retraction, trunk extension and chest
mobility (expansion). Shoulder retraction and back extension were reduced in 71%
and 48% of CF subjects respectively. A more recent study in children (7-14 years)
found that those with CF had significantly shorter pectoralis major, pectoralis minor
and gastrocnemius-soleus muscles than age-matched controls (266).
45
CF-related arthropathy
There are several CF-related arthritic conditions which frequently present with joint
pain and discomfort. The predominant types are CF arthritis (monoarthritis,
polyarthritis), pulmonary hypertrophic osteoarthropathy and arthritis due to co-
existent conditions and drug reactions (202, 205, 248).
CF arthritis (also known as CF arthropathy) occurs in 2-9% of patients (204). It is a
rare syndrome of unknown pathogenesis. (267). Clinical presentation includes joint
pain, long bone pain, arthralgia and joint effusions, particularly during infective
exacerbations. Joint pains typically develop over 12-24 hours and last 4-7 days. The
patient is asymptomatic between episodes.
Hypertrophic pulmonary osteoarthropathy (HPOA) is associated with respiratory
failure, and is present in 2-7% of patients. HPOA presents with insidious onset of
bone and joint pain; and evidence of periostitis on XRay of distal long bones.
Clubbing is considered a form of HPOA and the degree of clubbing is linked to the
degree of pulmonary disease (268).
Ciprofloxacin-associated arthropathy occurs infrequently in children with CF (269). It
is likely that ciprofloxacin can induce arthropathy in adult as well as paediatric
patients (270).
Rheumatoid arthritis, vasculitis, spondyloarthropathies, sarcoidosis, amyloidosis all
have been reported in association with CF. Rheumatoid factor titre is higher in CF
than healthy controls. Rheumatic symptoms occur in 33% of adults with CF and
arthritis in 2.5-12% of patients (271).
Gout appears to occur with greater frequency in the CF population, 2.5% compared to
0.6% in a healthy population under 54 years of age. Gout should be considered in
individuals with recurrent or single joint arthritis. In cases of confirmed gout or
extreme hyperuricaemia pancreatic enzyme intake should be reviewed (272).
Physiotherapy intervention for musculoskeletal problems
Recently, two prospective studies have evaluated the efficacy of a combined approach
including spinal joint mobilisation, massage, specific muscle strengthening and
postural advice, with results suggesting short-term reduction in musculoskeletal pain
and improvement in respiratory symptoms (273, 274). The findings of these pilot
46
studies require confirmation in larger trials, however they lend preliminary support to
use of a package of therapeutic approaches to optimize BMD, muscle strength, length
and postural education as well as manual therapy to improve spinal mobilisation.
Optimise physical activity to maintain bone mineral density and
muscle mass
Nixon and colleagues (275) reported children and adolescents with CF performed
significantly fewer hours of vigorous activity than a normal age matched control
group (2 vs. 3.7 hours per week). Weight bearing exercise is the most effective non-
pharmacological method to improve BMD in the healthy population by stimulating
bone accretion during growth and after bone loss, preventing bone loss and improving
bone structural qualities (234). Physical activity has also been shown to increase bone
formation markers in premenopausal women (276).
Weight bearing exercise during childhood and adolescence appears to be particularly
osteogenic and a variety of weight bearing exercise programs have increased bone
mineral accrual in healthy children at different pubertal stages (225, 233, 234, 237,
277-279). As the foundation of bone health begins in childhood, and there is some
evidence of a sustained benefit from early vigorous physical activity (279, 280),
children and adolescents should engage in regular high impact and intensity weight
bearing exercise for 30 minutes three times a week. The pre-pubertal and early
pubertal years are particularly important to help maximise peak bone mass (222, 281,
282) as approximately one quarter of peak bone mass is gained in the two years
around the pubertal growth spurt (281, 283). Children who are more physically active
have higher bone mass than more sedentary children and the emphasis is on high
impact vigorous activities as light to moderate physical activity does not have the
same osteogenic effect (284).
High impact weight-bearing exercise continues to have benefits into adulthood in the
healthy population. A meta-analysis has shown that in women 18-50 years, brief high
impact jumping exercises improved BMD at the hip (285).Weigh- bearing exercise is
also beneficial in maintaining or increasing BMD in postmenopausal populations
(234, 281, 282, 286, 287) and therefore is likely to be beneficial in maintain BMD in
adults with CF. Adults should perform regular individualised weight-bearing, high
impact and resistance exercises, the combination of which appears to have additional
benefits(222, 278, 282, 288, 289). When admitted in hospital individuals with CF
should be encouraged to continue their usual exercise program where possible (222).
47
Weight-bearing exercises should include force generating activities that provide
loading of the skeleton above that of normal ADLs and should be of sufficient
magnitude to generate ground reaction forces greater than two times body weight,
impose an unusual strain distribution and have a high strain rate (e.g hopping,
jumping, skipping, trampolining, circuit training, resistance training, aerobics,
gymnastics) (281-283, 287, 289). Shorter, more frequent exercise sessions are more
beneficial as rest periods enhance bone adaptation and restore mechanosensitivity
(234, 285). The most successful interventions included 2-3 session of 30 minutes a
week (234) with exercises aimed at regions with low BMD (289) and progression of
intensity and impact (282, 289).
Optimise muscle strength
Habitual ‘slouched’ postures are common in CF patients and may predate structural
change. Physiotherapy programs should consider inclusion of strengthening of
thoracic extensor muscles and scapular stabilisers to improve endurance and limit
persistent thoracic flexion. It is well established that strengthening programs are
effective in the CF population (290). The majority of reported home programs that
apply a normal therapeutic training stimulus have yielded physiologic and
psychological dividends (2, 190, 291, 292). Improved adherence in CF patients is
associated with supervision, individualised and flexible programs (290, 293), and
knowledge of the condition and treatment rationale (294).
In CF, strength training programs of moderate to high intensity have produced
significant leg strength gains in programs of short and long duration (19 days – 12
months) (295-297). A combined circuit weight and aerobic training program
generated significant muscle strength gains in upper and lower limbs, with gains that
were maintained following a four week detraining period (298). Strength training for
children is still a novel area with protocols not clearly defined. This is an area for
future research (297).
Optimise muscle length
Thoracic stretches may have a role in the management of thoracic impairment to
target identified shortened muscles in CF patients. A systematic review of the efficacy
of muscle stretching demonstrated lasting increments in range of motion (ROM) in
the presence of reduced muscle flexibility, in response to programs of greater than
48
three weeks duration (299). While evidence is restricted to long muscles, a rationale
exists in the thoracic region where restriction is present and appropriate muscle
lengthening stimulus applied. Muscle length is particularly sensitive to the most
extreme lengths experienced (300) and thus the effectiveness of stretching is affected
by the muscle length when the stretch is applied. Additionally, education regarding
postural correction could be included eg reduction of habitual postures that promote
increased kyphosis, use of lumbar rolls, and active work pauses that include thoracic
rotation and extension. This rationale reflects the close relationship between joint
mobility and muscle flexibility in the presence of a relatively rigid thoracic cage
Manual therapy and pain management
Spinal pain is troubling and warrants assessment in each individual to determine the
likely source – be it intrapulmonary, pleural, bone (vertebral or rib fracture), joint
(costovertebral, costotransverse, sternocostal) or muscular. Recent studies in other
populations have concluded that strong evidence exists to support the use of manual
therapy and exercise in the treatment of chronic musculoskeletal spinal pain (301)
(302). Home exercise programs were similar if not more effective in the management
of chronic lower back pain particularly when goal-directed and combined with
education (302). Early reports regarding the use of manual therapy in the management
of pain and restriction in this population is encouraging, with preliminary reports
supporting the role of a combined approach of manual therapy and exercise
prescription in patients with CF (273, 274). The primary clinical approach to CF-
related arthropathy involves rheumatological management, which may include
physiotherapy interventions targeting pain reduction and muscle strengthening.
Physiotherapy practice
Physiotherapy management of CF musculoskeletal impairment is warranted to relieve
pain, promote physical activity, to optimise BMD and muscle mass, limit postural
deformity and minimise the burden of CF disease.
The CF physiotherapist should provide prompt assessment and treatment of acute
musculoskeletal pain. A concise screening tool for use in clinic may facilitate this
process; an example is given in Appendix 4. Key components are:
Subjective history, pain scales (eg Short-form McGill questionnaire)
Functional scales with standardised items (303, 304) or patient-specific
functional scale (305)
Objective assessment, including posture
49
Precautions and need for referral/medical investigation
Likely structural source
Evidence based interventions
Reassessment
Home program
Analgesia and commencement of additional mucolytic and IV treatments should
be considered if fractures (vertebral/rib) interfere with ACT (222)
When a patient with CF presents with a swollen, hot, painful weight-bearing joint,
principles of management include:
Rest affected joint/s
Decrease impact or load in activity and exercise
Reduce intensity and duration of exercise
Encourage exercise of other joints; avoid exercise that increases pain
Consult with other health care providers regarding optimal management of pain
and inflammation
When acute episode resolves, strengthening of muscles around the joint may be
required.
When a patient with CF presents with a painful but normal looking weight-bearing
joint, principles of management include:
Reduce intensity and duration of exercise
Encourage exercise of other joints; avoid exercise that increases pain
When acute episode resolves, strengthening of muscles around the joint may be
required.
When a patient with CF presents with problems in non-weightbearing joint/s,
principles of management include:
Modify exercise programs to reduce load on affected joint/s (eg walking rather
than basketball)
Consult with other health care providers regarding optimal management of pain
and inflammation.
Additional physiotherapeutic interventions for patients with CF may include but
50
are not limited to:
Assessment for joint laxity, stability, or stiffness
Maintain range of motion of affected joint/s and active exercise of other parts
Local measures such as ice may be used for painful and inflamed joints if
warranted
Manual therapy and therapeutic massage (306)
Referral to medical colleagues and other physiotherapists with expertise in
musculoskeletal issues
The optimal time to minimise or prevent musculoskeletal deformity may be in the
pre-pubescent years (approximately 8-12 years of age) prior to significant structural
change particularly of bone (265, 307, 308). The European consensus on standards of
care for patients with CF (162) includes the physiotherapists’ assessment of “posture,
chest mobility, muscle strength and endurance” every “1-3 months or at every
outpatient clinic visit”. This intensive approach is recommended in the presence of
0.5-1.0 (pediatric CF centre) / 1.0 (adult CF centre) physiotherapists per 50 CF
patients.
The CF nutrition consensus statement (309) supports the periodic assessment of BMD
from the age of eight years and then every three to five years if BMD is normal, every
two years if BMD is moderately reduced and annually if BMD is severely reduced.
More frequent scanning is recommended if significant new risk factors emerge.
Physiotherapists should promote physical activity to improve or maintain BMD and
muscle strength.
Key components of home exercise programs for people with CF are:
Individualised assessment. Exercise prescription should be patient specific in
terms of muscle strengthening, stretches, self/auto mobilisation and postural
correction.
A muscle strengthening program should be included if muscle
imbalance/weakness is contributing to postural impairment or pain with a
normal training stimulus applied.
A muscle stretching program should be included if muscle or joint stiffness is
contributing to postural impairment or pain.
Education regarding postural correction/health should be included eg
reduction of habitual postures that promote increased kyphosis, use of lumbar
51
rolls, and active work pauses that include thoracic rotation and extension.
Standardised measures and reassessment should be included to determine and
maintain program efficacy.
Musculoskeletal management in the presence of CF utilises diverse physiotherapy
skills given the wide scope of presenting problems. These may include: acute sports
injury, acute joint flares in the presence of CF related arthropathy, or acute pain
associated with coughing. Physiotherapists will draw on skills in biomechanical
assessment of posture and movement, exercise prescription (for the extremely
deconditioned to elite athlete), management of pelvic floor dysfunction, and
management of the musculoskeletal challenges of rapid body change during puberty,
pregnancy, and post lung transplantation. Ultimately physiotherapists have an
important role in the quantification of CF musculoskeletal impairment and the
provision of effective musculoskeletal interventions, to enhance quality of life for
people with CF.
Recommendations
16. A musculoskeletal assessment should be included at annual review from
approximately age eight (pre puberty). Earlier assessment is warranted if
pain or functional impairment is reported or BMD risk highlighted (C).
17. Regular physical activity, including weight-bearing exercise, should be
encouraged throughout the lifespan in order to optimise BMD (C).
18. Strength training programs should be prescribed in order to optimise
muscle mass (B)
Practice Points
Prompt assessment and treatment of acute musculoskeletal pain should be
provided to people with CF.
Consider provision of a musculoskeletal home program for individuals
with CF, based on individualised assessment.
52
6 Physiotherapy management of the complex patient
Cystic fibrosis is a complex multi-system disease and patients often experience
significant complications which may require alteration to their usual physiotherapy
management. These complications include haemoptysis, pneumothorax, allergic
bronchopulmonary aspergillosis (ABPA) and CF-related diabetes.
Haemoptysis
Haemoptysis is defined as the expectoration of blood from the lungs or bronchial
tubes as a result of pulmonary or bronchial haemorrhage (310). Whilst the presence of
occasional mild haemoptysis is common in CF and not life-threatening, massive
haemoptysis can lead to asphyxiation, airway destruction, shock and exsanguination
(311).
Mild haemoptysis affects approximately 62% of all patients with CF (312). The
overall incidence of massive haemoptysis in CF has been reported as around 1% in
children (313), and between 4% and 10% in adults (312, 314). Increasing age is the
greatest risk factor for the development of massive haemoptysis. Other risk factors
include moderate to severe lung function impairment, concurrent infection with
Staphylococcus aureus, vitamin K deficiency and diabetes (315).
Medical management of mild haemoptysis consists of observation combined with
antibiotic therapy to treat underlying infection (316) and the use of tranexamic acid
(317). For massive haemoptysis, the vessel may need to be occluded using bronchial
artery embolisation (316, 318, 319). Surgical ligation or excision of the affected
segment/ lobe is recommended if embolisation is not successful (318).
Physiotherapy practice
The CF Foundation Pulmonary Therapies Committee (USA) have developed
guidelines for the management of haemoptysis based on expert opinion using a Delphi
process (320).
Scant (<5mL) haemoptysis, first episode:
•Seek medical review (320)
53
•Continue with ACTs and inhaled therapies (320)
•Modify ACTs:
• Reduce the force of coughing
• Cease highPEP
• Minimise head down tilt positions
• If active bleeding, position into high sidelying with bleeding side down (321)
• Provide reassurance and education
• Do not withhold non-invasive ventilation
Scant haemoptysis, recurrent episode:
• Normal ACTs and inhaled therapies
• Normal exercise routine
Mild to Moderate Haemoptysis (<250mls/ 24 hrs)
• Seek medical review (320)
• Cease percussion, vibrations, oscillatory PEP techniques and head down tilt
positions
• Consider ACBT, AD (320) and gentle huffing and coughing
• Continue inhaled therapies in most situations (320)
• Ensure adequate humidification to ease sputum expectoration
• Cease vigorous exercise, encourage low intensity exercise
Massive Haemoptysis (>250mls/ 24 hrs)
• Seek medical review (320)
• If active bleeding, position into high sidelying with bleeding side down (321)
• Cease ACTs, hypertonic saline (320) and exercise until active bleeding resolved,
• Cease other inhaled therapies if considered to provoke bleeding
• Cease non-invasive ventilation
• Once active bleeding contained, continue as per moderate haemoptysis
54
Following Embolisation
• Ensure adequate analgesia and humidification
• Gentle mobilisation following surgeon/ radiologists advice, then gradually
increase intensity of exercise
• Airway clearance using ACBT or AD initially
• Gradually reintroduce normal ACT regimen
Practice point
When haemoptysis is present, the physiotherapist aims to maintain adequate
airway clearance and exercise regimens whilst promoting vessel healing
and minimising the risk of re-bleeding.
Pneumothorax
A pneumothorax is defined as the presence of air within the pleural space. A
pneumothorax may occur in an individual with CF as a result of rupture of sub-pleural
blebs on the visceral pleura (322, 323) or, less commonly, as a result of misplacement
of a central line (322). A pneumothorax can present a major problem in a patient with
CF as the collapsed lung can be stiff and take a long time to re-expand (324). This
may affect the ability to perform ACTs and lead to secondary complications related to
sputum retention.
The overall incidence of pneumothorax in patients with cystic fibrosis is 3.4% to
6.4% (323, 325, 326). There is an equal risk for men and women (325) and 72% are
adults (327). Pneumothoraces occur more frequently in patients with more advanced
disease (327), with 75% of affected patients having an FEV1 less than 40% predicted
(327). Recurrence rates of 41% ipsilaterally (328) and 46% contralaterally (323) are
reported.
For small pneumothoraces in an asymptomatic patient, medical management usually
involves observation and/ or aspiration. A large pneumothorax requires intercostal
drainage. Intravenous antibiotics should be commenced at the same time to prevent
55
infection and resultant sputum retention, which may delay re-expansion of the
collapsed lung (324). A recurrent pneumothorax requires more aggressive
management, including either a partial pleurectomy or a talc pleurodesis (324).
There is concern that pleurodesis can make transplantation more difficult as it
impedes lung removal, however the current consensus is that pleurodesis is not an
absolute contraindication to transplantation (329).
Physiotherapy Practice
There are no published data regarding physiotherapy management of patients with
pneumothorax. The following recommendations are based on expert opinion (320)
and clinician consensus.
Small Pneumothorax
Monitor shortness of breath
Cease PEP and other forms of PEP therapy and liaise with medical team
Review use of hypertonic saline for nebulisation, especially if it causes
coughing
Gentle coughing
Ensure adequate humidification for ease of sputum expectoration
Reduce exercise intensity
Avoid upper limb resistance exercises
Large Pneumothorax
If pneumothorax is undrained, cease treatment and liaise with medical team.
If pneumothorax is drained:
If patient uses PEP or non-invasive ventilation, consider cessation or reduction
in pressures while draining and for 48 hours afterwards, to avoid pleural
fistula and risk of recurrence
Review use of hypertonic saline for nebulisation, especially if it causes
coughing
Ensure adequate analgesia and humidification
56
Maintain shoulder range of motion
Chest support during airway clearance
Gentle huffing and coughing
Avoid upper limb resistance exercises
Submaximal exercise (walking, gentle cycling)
Pleurodesis:
Ensure adequate analgesia
Regular nebulisers as tolerated
Early mobilisation
Commence ACBT/ AD with gentle coughing
Practice point
When a pneumothorax is present, physiotherapists aim to ensure that
adequate airway clearance continues, whilst minimising the amount of
positive pressure generated inside the patient’s lungs. Gentle exercise can
continue.
Allergic Bronchopulmonary Aspergillosis
Allergic bronchopulmonary aspergillosis (ABPA) is caused by a hypersensitivity
response to Aspergillus fumigatus and other species). Aspergillus spores are trapped
in the mucus of large segmental bronchi, germinate to form hyphae which elicit an
immune response, and can lead to accumulation of focal pulmonary infiltrates, tissue
damage and eventual destruction of lung tissue (330, 331). It occurs in approximately
7-9% of individuals with CF (332), although it is more common in adults, those with
lower FEV1 and low body weight (330).
Detailed lung function testing in patients with ABPA shows abnormalities suggestive
of airway narrowing, gas trapping and small airways disease (333). The clinical
features of ABPA are variable but can include increased cough, wheezing, focal
pulmonary infiltrates, coughing up “plugs”, frequent exacerbations and deteriorating
lung function. A recent epidemiologic study also showed that some episodes of
57
massive haemoptysis and pneumothorax were associated with ABPA (330).
Diagnosis is made via clinical features. There is usually also a rise in total
immunoglobulin E (IgE), increased aspergillus specific IgG and IgE and/or skin
reactivity to aspergillus.
Medical management of ABPA involves oral steroids and/or antifungal agents as well
as treatment of underlying infection.
Physiotherapy practice
There is no literature specifically pertaining to physiotherapy in ABPA. Some centres
in Australia report successful use of hypertonic saline, with or without PEP, whilst
closely monitoring lung function to ensure there are no adverse effects.
Practice point
Physiotherapists should reassess the efficacy of usual ACTs in people with
ABPA. Treatment should be modified based on response and symptoms.
Cystic fibrosis related diabetes
Cystic fibrosis related diabetes (CFRD) shares features of both Type 1 and Type 2
diabetes. Scarring of the exocrine pancreas leads to partial Islet cell destruction,
causing disruption to the insulin secreting endocrine cells. CFRD manifests primarily
as an insulin deficiency, and is best diagnosed via glucose tolerance test (two hour
blood glucose>11, further classified as “with or without fasting hyperglycemia”).
Cystic fibrosis related diabetes has been associated with loss of weight and lung
function for several years prior to diagnosis, and has been reported to increase whole
body protein breakdown, as well as reduce lean body mass. It is the most frequent
major co-morbidity in CF, occurring in 5-30% of patients, and is more common in
patients with pancreatic insufficiency. Prevalence increases with age and is reported
to be as high as 50% of patients aged over 30 years. The mainstay of medical
treatment is insulin, usually in conjunction with specialist endocrinologists and much
ongoing education. Specialist CF dietary advice is also necessary for optimal blood
glucose level (BGL) control.
58
There is a wealth of literature on the role of exercise for type 1 and type 2 diabetes in
non-CF patients. In type 1 diabetes, exercise can acutely lower blood glucose,
improve glycaemic control and reduce HbA1c (334). Exercise also improves vascular
endothelial function (335). In type 2 diabetes, exercise also improves glycaemic
control and reduces insulin resistance (336). Strength training has been demonstrated
to be more effective than endurance training in this group (337). The American
Diabetes Association Clinical Care Guidelines for CFRD (338) recommend that
people with CFRD should be advised to perform moderate aerobic exercise for at
least 150 minutes per week (level of evidence – expert consensus or clinical
experience). They also suggest that people with CFRD be counselled to monitor blood
glucose levels (BGLs) before vigorous physical activity and ‘potentially consume
extra carbohydrate or alter their insulin dose, depending on the glucose level and the
intensity and duration of the planned exercise” (338), page 2705).
Physiotherapy practice
There are a number of important safety considerations in relation to diabetic patients
and exercise (338).
Fast-acting carbohydrate snacks should be immediately accessible to all patients with
CFRD during and after exercise. Patients with recurrent hypoglycemia may have poor
hepatic glycogen stores, particularly where there is CF-related liver dysfunction.
Carbohydrate intake pre-exercise is recommended in this situation to avoid
hypoglycemia with exercise.
Consideration should be given to monitoring of BGLs before and after strenuous
activity to determine BGL response to exercise. It is important to note that delayed
hypoglycaemia can occur up to 24-36 hours after exercise as the muscles refuel.
Monitoring will allow the CF team to take any necessary steps to prevent
hypoglycemia.
If BGLs are high prior to exercise then exercise can have a paradoxical effect on
blood glucose causing it to rise. The high blood sugar is a reflection of inadequate
insulin. Insulin is required during normal exercise to rapidly transport glucose into
muscle. If there is insufficient insulin then hepatic glycogen stores will be released
and blood sugar further elevated following exercise. It will however fall rapidly with
use of insulin following exercise (causing delayed hypoglycemia).
59
Patients are frequently advised to select insulin injection sites that are away from
areas used during the chosen form of exercise. Increased regional blood flow may
result in faster absorption of insulin from such sites.
Consideration should be given to salt supplementation and adequate hydration in
people with CFRD. Attention should be paid to appropriate footwear and foot care to
minimise the risk of diabetic ulcers.
During periods of acute illness/exacerbation or courses of corticosteroids, BGLs and
insulin requirements may be altered such that patients may require more careful
monitoring (338).
Practice points
People with CFRD can be encouraged to perform moderate aerobic exercise
for at least 150 minutes per week
Insulin injection sites should be away from areas used during the chosen
form of exercise.
Fast acting carbohydrate snacks should be immediately accessible during
and after exercise
Consider monitoring BGLs before and after strenuous activity.
60
7 Physiotherapy for pregnancy, labour and the post-
natal period
The improvement in health, quality of life and longevity in CF has increased the
desire and possibility of adults with CF becoming parents. In the 1980s, pregnancy
was thought to be too risky for women with CF. However, increased survival means
that pregnancy in women with CF is becoming more common and thus CF care teams
can give better advice regarding likely outcomes. There are few published data that
relate to the management of pregnancy in CF. Most literature reports the negative and
positive outcomes of relatively small case series (339-343). Women with CF can have
successful pregnancies following lung transplantation but the risks of organ rejection
and death are high (344).
Physiological changes during pregnancy
The pregnancy hormones of relaxin, progesterone, oestrogen and cortisols result in
laxity of ligaments in preparation for the birth process. These effects are greater in
mutigravidae than primagravidae women. It takes approximately 3-6 months for the
body to return to the pre-pregnant state after the birth (345). The maternal centre of
gravity shifts posteriorly during pregnancy to accommodate the increased abdominal
size resulting in changes in postural alignment and gait in later pregnancy. As thoracic
and lumbar curves increase, so does the strain on the vertebral joints. Back pain is
common affecting more than 50% of women during pregnancy. Back pain is
sometimes accentuated in women with CF especially during acute lung exacerbations
and increased coughing.
Cardiac output increases by approximately 40% by 20 weeks gestation. Blood volume
increases by approximately 40%, with a peak at 30 weeks gestation, secondary to new
placental circulation. General vasodilatation occurs. This results in increased cardiac
work during pregnancy (346). Progesterone stimulates the respiratory centres of the
brain to produce hyperventilation early in pregnancy. In late pregnancy secondary to
hormonal changes relaxation of smooth muscle occurs in the tracheo-bronchial tree
leading to a decrease in total pulmonary resistance. This may be beneficial to women
with more obstructive lung disease. However, at the end of pregnancy, residual
volume decreases secondary to elevation of the diaphragm. In the third trimester,
especially with multiple foetuses, the enlarged uterus pushes upward and outward.
The high abdominal wall tension raises the intra-abdominal pressure, even when the
woman is upright. The diaphragm does not descend appreciably, and therefore the
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FRC remains reduced. Expiratory reserve volume and FRC decrease by 15%. This
may contribute to difficulty in removing respiratory secretions.
Oedema in the lower limbs is common in pregnancy and is caused by the effects of
progesterone. When standing for long periods, gravity causes venous engorgement
further exacerbating the problem. Carpal tunnel syndrome is caused by oedema in
arms and hands compressing the distal segments of the median and ulnar nerves -
generally later in pregnancy but sometimes occurs as early as 16 weeks.
Pre-pregnancy planning
It is widely recognised that a planned pregnancy is likely to result in fewer problems
than an unplanned pregnancy. The multidisciplinary team involved in the care of
pregnant women should at least consist of a respiratory physician, obstetrician,
physiotherapist, nutritionist and psychosocial practitioner, all experienced with CF.
Inhaled, oral and intravenous medication and their potential for iatrogenic effects
together with optimal nutrition and dietary supplementation should be reviewed (347).
All women with CF are advised to approach pregnancy with a regular ACT routine.
An optimal ACT routine suitable for pregnancy should be discussed. Modifications to
physical exercise should also be planned. Domestic support during pregnancy and
afterwards together with child care support once the baby has been born are
necessary, so that the mother has enough time and energy to carry out regular ACTs,
inhalation therapy and exercise (348). Frequent contact with the multidisciplinary CF
team and the obstetric team should be encouraged during pregnancy and the postnatal
period (349).
Airway Clearance Therapy during pregnancy in CF
Head-down tilted postural drainage is not recommended during pregnancy because of
the high prevalence of symptomatic and clinically silent gastro-oesophageal reflux
(GOR) in adults with CF (79). This is further compounded by the hormonal effects of
progesterone during pregnancy resulting in a hypotonic lower oesophageal sphincter
together with the growing weight of the developing foetus pressing against the
stomach. Techniques that exacerbate nausea should be avoided.
Airway clearance techniques suitable during pregnancy include:
ACBT
AD
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PEP therapy
Oscillating PEP
Effective huffing from different lung volumes avoiding dynamic airway
collapse
Physical exercise (appropriate to pregnancy) as airway clearance therapy
Mucolytic agents that may be used during pregnancy as adjuncts to airway clearance
therapy include:
Dornase alfa – may be continued if being used prior to pregnancy
Hypertonic saline: 3 – 7%
Inhaled mannitol
Saline 0.9%
Positioning during Airway Clearance Therapy
Because of the physiological changes of pregnancy, upright sitting is usually the most
comfortable position for airway clearance. Consideration should be given to
positioning during ACTs to maintain a neutral lumbar spine for prevention and/or
minimisation of urinary incontinence during treatment (see Appendix 5). Some
women find left and right side lying horizontal or slightly head up to be more
effective during ACT. The supine horizontal position should be avoided during the
2nd and 3rd trimesters because of pressure of the foetus on the inferior vena cava
which may decrease venous return and cardiac output.
Pelvic floor function
Women with CF should be taught pelvic floor strengthening exercises to prevent
and/or treat urinary incontinence (see Chapter 8). These exercises should be
highlighted during pregnancy and in the post-natal period when urinary incontinence
is a common problem. Increase in fluid intake and activity levels such as walking
together with regular toilet habit help ease constipation.
Exercise during Pregnancy
Pregnant women are advised to modify their physical exercise program during
pregnancy. Contact sports should be avoided. Walking and swimming are appropriate
forms of exercise. Women should avoid overheating and dehydration during exercise
ensuring adequate water and electrolyte intake. Postural awareness, ergonomic advice,
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strengthening, mobilising and stability exercises and sometimes a lumbar sacral
support belt assist in managing these normal pregnancy changes. Symphysis pubis,
sacro-iliac joint and round ligament pain occur commonly around 29-32 weeks
gestation. An elastic binder, worn low below the belly to give support to the
symphysis pubis and sacro-iliac joints while weight bearing, can provide significant
relief. Diastasis of the recti muscles requires care in exercise.
Other physiotherapy interventions during pregnancy
Working and /or resting splints may be used to manage the symptoms associated with
carpal tunnel syndrome. Contrast bathing to increase circulation and decrease oedema
may also be helpful.
Lower limb oedema should be prevented by avoiding prolonged standing. Rest with
feet elevated and muscle pump exercises and elastic support stockings are beneficial.
Physiotherapy advice about comfortable supported sleeping positions using extra
pillows, relaxation and stress management techniques are useful strategies. Muscle
cramps are common especially in later pregnancy. They may be caused by ischaemia
and pressure of the uterus on the nerves or dietary issues. Calf stretches during the
day, support stockings, medical advice on nutritional requirements such as calcium or
salt and massage may relieve the problem. Plantar flexion should be avoided when
waking up.
Physiotherapy during labour in CF
Pain, shortness of breath on exertion and low oxygen saturation are common in
healthy women during labour. Oxygen saturation has been measured at 98% the day
after delivery, whereas in labour it went as low as 87% in women without CF (346).
Thus desaturation may be marked in women with CF during labour. Oxygen therapy
should be provided to maintain normal saturation. Bronchodilator therapy and
assistance with sputum clearance may be required for some women during labour.
Conservation of energy strategies should be employed. Adequate pain relief during
labour is a high priority for women with CF, with normal vaginal delivery highly
desirable in order to minimise post-delivery complications (348).
Physiotherapy post-Caesarean section in CF
Adequate post-operative pain relief, oxygen therapy (if required), appropriate
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inhalation therapy in the form of bronchodilators and mucolytic agents together with
optimal ACTs and early mobilisation are a priority after a Caesarean section.
Physiotherapy in the post-natal period
Physical support for the mother is a priority after birth. She needs to have time and
energy to carry out appropriate airway clearance therapy, inhalation therapy and post-
natal exercises.
These physiotherapy considerations and the following recommendations concur with
those detailed in the European guidelines for the management of pregnancy in women
with cystic fibrosis (350).
Practice Points
Postural drainage in head down tilted positions should not be used during
pregnancy.
Exercise should be modified during pregnancy according to usual
pregnancy guidelines.
During labour, appropriate pain relief, oxygen therapy, inhalational
therapy and ACT should be provided as required.
Following delivery, adequate pain relief, oxygen therapy, appropriate
inhalation therapy, ACT and early mobilisation should be considered.
Encourage frequent contact with the multidisciplinary CF team and the
obstetric team during pregnancy and the postnatal period.
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8 Physiotherapy management of continence
Urinary incontinence is the involuntary leakage of urine. The two types of urinary
incontinence are stress incontinence and urge incontinence. The reported prevalence
of urinary incontinence in girls and women with CF ranges from 22% to 74% (351-
357) in comparison with 13% in healthy women aged 18-24 years (358) . There is
limited literature in adult males with CF, with the prevalence reported to be from 8-
15%, compared to 7.5% in healthy men (357, 359, 360).
It is not known whether the cause of urinary incontinence in CF is chronic cough,
paroxysms of prolonged coughing or demands placed on the pelvic floor during ACT,
huffing, coughing and physical exercise (all essential elements of recommended daily
physiotherapy treatment), or underlying structural differences. It has recently been
demonstrated that women with chronic lung disease did not differ from control
subjects in pelvic floor muscle strength or timing of contractions; however women
with chronic lung disease did have reduced endurance of pelvic floor musculature
with prolonged coughing (361). Age has been reported to have a strong positive
correlation with the severity of stress urinary incontinence symptoms (356). Studies
reporting on the incidence of stress urinary incontinence in people with CF also report
increased anxiety, depression and a negative impact on quality of life (360, 362).
It has been demonstrated that treatment of urinary incontinence in women with CF by
a qualified continence physiotherapist with exercise, electrical stimulation,
biofeedback and bladder training resulted in significant improvements in pelvic floor
strength, reduction in leakage and improvement in quality of life which were
sustained for at least three months after the completion of treatment (363). Care
should be taken when teaching pelvic floor exercises as evidence suggests 40% of
women with incontinence incorrectly perform a pelvic floor contraction with verbal
education alone (364). It is not appropriate in the paediatric setting to use invasive
assessment procedures; however data presented in abstract form indicate that
adolescent females have correctly and successfully learnt pelvic floor exercises using
Real Time Ultrasound (365) without reported embarrassment. Positive outcomes have
also been demonstrated with surgical correction of severe stress urinary incontinence
in women with CF (366).
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Physiotherapy practice
Patients are often embarrassed about incontinence and will seldom raise the topic with
the health care team. However, if asked as part of routine assessment patients value
the opportunity to discuss the issue and learn strategies to prevent and / or resolve the
problem. All physiotherapists working in CF should include screening for
incontinence as part of routine care for both male and female patients.
Female patients should be taught to perform a contraction of the pelvic floor prior to
any activity that increases the load to the pelvic floor (such as coughing, huffing,
sneezing, laughing) to prevent leakage. This should become a lifelong habit and will
help in the prevention of leakage during all activities that apply force to the pelvic
floor. Where available, real time abdominal ultrasound can be used to ensure correct
technique without invasive procedures. Patients should also be taught optimal
positioning in upright sitting for ACT in positions that maintain a neutral lumbar
spine and optimise pelvic floor function (367) with the adding of perineal support in
those with urinary incontinence. Trampoline jumping, a commonly prescribed form of
physical exercise and airway clearance therapy is appropriate until the age of puberty.
Thereafter, jogging on the trampoline is more appropriate to avoid excessive force on
the pelvic floor.
Pelvic floor exercises should be prescribed routinely for women with incontinence.
Protocols for pelvic floor training, including handouts can be found through the APA
or Continence Association of Australia at
http://www.continence.org.au/resources.php. Treatment protocols are less well
described for men, who may require direct referral to a physiotherapist with expertise
in continence.
Female patients who continue to have a problem with bladder and bowel control
should be referred for a course of specialised assessment and treatment using exercise,
electrical stimulation, biofeedback and bladder training by a qualified continence
physiotherapist and may require referral to a gynaecologist or urologist.
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Recommendations
19. Women with CF and symptoms of stress urinary incontinence should be
taught rehabilitative strength and endurance exercises to provide better
control of the pelvic floor (C).
20. Men and women with CF should be screened for symptoms of stress
urinary incontinence (C)
21. Airway clearance should take place in postures that maintain a neutral
lumbar spine, to optimize pelvic floor function (C).
Practice Points
From puberty onwards, consider teaching females with CF pelvic floor exercises
as a way of preparing the pelvic floor to overcome the downward pressure
during activities such as exercise, forced expirations and coughing.
In patients who continue to have difficulties with bladder or bowel control
despite prescription of rehabilitative exercises, consider referral to a qualified
continence physiotherapist.
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9 Physiotherapy management of the newly
diagnosed patient
The newly diagnosed infant
Most children born in Australia are screened at birth for CF through a heel prick
blood test performed usually between 48 and 72 hours of age. Regardless of
symptoms, all infants and their families should meet with the CF multi-disciplinary
team soon after diagnosis, either as an inpatient or outpatient (368, 369).
Physiotherapy intervention should begin immediately unless specifically delayed for a
short period at the discretion of the CF team (eg post bowel resection for meconium
ileus, high levels of family anxiety).
The role of physiotherapy in airway clearance, exercise and active play should be
explained, demonstrated and practiced at initial education sessions, regardless of
symptoms (162, 370). Educational materials should be provided for the family. The
rationale for ACTs should be explained with relation to:
Pathophysiology – Even in asymptomatic infants there is evidence of inflammation,
infection and structural change in the airways (371, 372). Treatment of early changes
may preserve lung function and optimise long-term outcomes (373, 374).
Theory of airway clearance techniques - By improving mucociliary clearance, the
development of chronic lung disease may be delayed (36, 373).
Physiotherapy practice
To aid adherence, airway clearance for the newborn should fit in with the family’s
routine (1, 36, 375). General consensus amongst Australian physiotherapists working
with CF children suggests treatment of an asymptomatic infant should consist of five
modified PD positions performed 1-2 times daily as appropriate. These positions
should not include traditional gravity-assisted postural drainage in the infant due to
the risk of GOR (68, 72, 75). In each position percussion or thoracic compressions
should be performed for 3-5 minutes. Vibrations are not generally used with an infant.
Total treatment time should be a maximum of 25 minutes per session. Other
techniques such as infant PEP, assisted autogenic drainage (36), play and age-
appropriate daily physical activity (376) can be introduced, dependent on the
individual needs of the infant and family.
Generally treatment should be encouraged before or at least one hour after feeds (36,
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375) and be performed at a time that best suits the family routine. Some awake time
treatment should be encouraged to aid future compliance. Coughing should not be
induced in the asymptomatic infant, but imitation coughing may be encouraged from
early on. If the child has a respiratory exacerbation then treatment should be increased
to 2-4 times daily as appropriate and able. Airway clearance therapy may need to be
modified as the results of investigations become available (eg CT scans, CXRs and
sputum cultures).
On initial assessment the physiotherapist should encourage the parents to observe the
infant’s baseline signs and symptoms so they can identify possible signs of a chest
infection. These signs and symptoms should be based on World Health Organisation
(WHO) guidelines (377) and include changes in fever, cough frequency and/or sound,
mood, stools, reflux patterns, work of breathing or breathing patterns, appetite or
weight loss.
The use of music/singing, regular routines, toys and pacifiers can be demonstrated and
encouraged to assist with adherence to treatment (378). Positions and methods with
which to handle, distract and settle the child during ACTs can be introduced as
appropriate, particularly with first-time parents. The importance of exercise and
normal activity participation should be regularly discussed. Normal developmental
play and prone lying should be encouraged as the first steps towards an active
physical life style and routine (36).
Ongoing education and engagement of the patient/carers relating to appropriate
dosage and order of physiotherapy including ACT, adjunctive inhalation therapy and
regular exercise should be provided.
The newly diagnosed adult
Adults with a new diagnosis of CF usually have milder disease than those diagnosed
in infancy (162). Symptoms are more subtle and initial sweat chloride tests are lower
or equivocal. Only one system may be affected and there is a higher incidence of
pancreatic sufficiency (162). Despite presentation with a milder form of disease,
adults with a CF diagnosis still have to accept living with a genetic and potentially
severe chronic illness (373). There should be no presumption of knowledge of the
disease. Cystic fibrosis teams should assess what their adult-diagnosed patients know
and what they need to learn. Specific problems should be addressed initially and
further education introduced over a period of time in the outpatient or inpatient
setting. The focus of treatment should be on self-management but this may be
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dependent on disease severity and already established treatment regimens (373).
Education about CF and its management should consider the implications of the
disease on established lifestyles (373). Newly diagnosed adults may have unique
needs regarding hospitalisation with significant impact on home, social and working
lives. Establishing good communication and development of rapport are essential for
a professional and supportive relationship (36). Newly diagnosed adults may be active
seekers of alternate sources for information, such as the internet. The CF team should
ensure that patients are accessing reliable resources.
Care should be taken with early incorporation into a CF clinic until colonising
organisms are established. Exposure to the multidisciplinary resources of a CF clinic
can optimise management of the diverse symptoms of adult CF disease.
Physiotherapy practice
Some newly diagnosed adults are very well; therefore physical exercise, huffing and
coughing may be appropriate stand-alone ACTs (102). However, it is still important
that these patients are given information about other ACTs available to manage their
respiratory symptoms. Comprehensive ongoing education about techniques and the
rationale for their use may improve adherence in the future.
Those patients with established lung disease should be taught ACTs and exercise
regimens and educated about the role of nebulized drugs in their treatment.
Recommendations
22. Treatment for newly diagnosed infants may include:
a. Percussion for 3-5 minutes in each of 5 modified postural
drainage positions (B)
b. Daily age-appropriate physical play (C)
23. Physiotherapy treatment for the newly diagnosed child and adult should
include regular physical exercise (B); other forms of airway clearance
therapy should be added as required (C).
Practice point
Education by the physiotherapist begins at diagnosis
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10 Transition from paediatric to adult care – the
physiotherapy role
Transition can be defined as ‘the purposeful, planned movement of adolescents and
young adults with chronic physical and medical conditions from child-centred to
adult- orientated health care systems’ (379). It is widely accepted that young adults
with CF should receive their health care in adult settings (380, 381). However,
transition can be a stressful period for patients and families (382). Areas of most
concern include potential exposure to infection; leaving a well-known physician; and
meeting a new care team (383-385).
There is a lack of evidence in the literature evaluating existing CF transition
programs, particularly in Australia (386). The Cystic Fibrosis Standards of Care
Australia (2008) (349) lists six standards for transition, based on expert opinion:
Transition from Paediatric to Adult Care
Standard 1 Transition from paediatric to adult care involves a process of close
cooperation between paediatric and adult specialist care teams.
Standard 2 All CF Centres should have a transition programme incorporating active
education on adult issues, e.g. fertility, and the process should engage the young
person with CF and their family in a positive way.
Standard 3 The concept of transition should be raised soon after diagnosis with more
active discussions commencing around secondary school entry (12 years) and the
process finishing with transfer to adult care around school leaving age (18 years).
Standard 4 Paediatric and adult specialist care teams should meet regularly to discuss
individuals in transition.
Standard 5 The adult co-ordinator should meet individuals during the year before
transfer, and the adolescent should have the opportunity to visit the adult CF Centre at
this time.
Standard 6 A comprehensive summary of medical and social issues should be
available to the adult team well in advance of transfer. The local CF Association can
be involved to help facilitate the process.
There is no consensus regarding the best model of transition for patients with CF in
Australia. The goal of each centre should be to facilitate a structured transition
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process for the patient, by following the standards of care. A key feature of effective
transition includes early preparation and facilitating self-management skills of the
young person with CF (386-388).
Whilst there are no direct references to physiotherapy in the current literature
evaluating transition programs, there are some specific aspects of care that are
important to address in the physiotherapy management of transfer.
Physiotherapy practice
During the transition process, physiotherapists from the paediatric and adult centres
should communicate directly regarding the detailed aspects of each patient’s treatment
regimen (389). It may be beneficial for the young person with CF to meet the
physiotherapist from the adult centre prior to transfer. Infection control policies for
physiotherapy in the adult setting should be discussed as part of the transition process
(383). Physiotherapists in the paediatric centre can assist the transition process by
actively promoting self-management in the time leading up to transfer of care (387,
388, 390) and providing positive information about the adult service.
The close liaison between paediatric and adult physiotherapy teams should not cease
at the time of transfer but continue throughout the first year until the patient is well
established in the adult clinic. This is particularly important if a patient becomes
unwell soon after transfer, in which case the physiotherapists should communicate
directly to ensure optimisation of physiotherapy care.
Transition should be a planned, coordinated and gradual process engaging both the
young patient with CF and their parents in a non-confronting way (388). Cooperation
between physiotherapy staff at adult and paediatric centres is essential for successful
transition in CF.
Practice point
During the process of transition, paediatric and adult physiotherapists
will need to communicate directly regarding the detailed aspects of each
patient’s physiotherapy care.
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11 Physiotherapy management for end-stage disease
Non-Invasive Ventilation in Cystic Fibrosis
The management of severe lung disease is an important component of care for
patients with CF. With life expectancy now extending well into adulthood and many
patients choosing to be listed for lung transplantation, maintaining optimum
functioning in patients with end-stage lung disease is crucial. Non-invasive ventilation
(NIV) has an expanding role in the management of CF-related acute respiratory
failure and bridge to transplant; chronic respiratory failure; airway clearance; and
exercise.
The proven physiological benefits of NIV in CF include unloading of the respiratory
muscles resulting in improved respiratory muscle performance, increased alveolar
ventilation and improvement in gas exchange (391). Short-term physiological studies
have shown reduced respiratory muscle work during NIV in both adults and children
with CF, with reported reductions in work of breathing of 20– 60% (392-394). This is
associated with a 30% increase in minute ventilation and tidal volume, and reduction
in transcutaneous carbon dioxide of 7% (394). The results of these studies support the
theoretical rationale for respiratory muscle unloading with NIV in CF, resulting in
improved alveolar ventilation and better gas exchange. These effects are crucial to the
clinical application of NIV in CF.
Acute respiratory failure and bridge to transplant
A number of descriptive studies report the successful use of NIV to stabilise patients
with CF and acute respiratory failure (395-401). In this group of patients with severe
lung disease, NIV results in reduced PaCO2, respiratory rate and dyspnoea. Although
NIV does not reverse the respiratory deterioration inherent in end-stage disease, it
may allow the patient to be stabilised for long enough for donor lungs to become
available for transplantation. The use of NIV outside the group of CF patients
awaiting lung transplantation has also been reported (400) where it may be useful for
palliation of dyspnoea in end-stage disease.
There remains no disease-specific guideline for initiation of NIV during acute
respiratory failure in CF. Surveys aiming to establish current practice in centres
within Australia and overseas have suggested variability in indications for NIV and
method of initiation (402, 403). Many useful principles can be taken from experience
in other lung diseases (404). However, there are some special features of CF which
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should be taken into consideration when setting up NIV for this patient group.
1. Acid-base balance
Special care needs to be taken with analysis of arterial blood gases in CF patients with
acute hypercapnic respiratory failure. As well as a respiratory acidosis, these patients
frequently exhibit a metabolic alkalosis which may contribute to hypercapnia (405),
and may have implications for response to NIV in hypercapnic patients. In some
patients, electrolyte and volume correction may be required to restore acid-base
balance.
2. Humidification
Non-invasive ventilation delivers air at high flow rates and with low relative
humidity, which may overwhelm the capacity of the upper airway mucosa to warm
and humidify inspired air (406). The levels of humidity delivered during bilevel are
lower than the levels reported to cause airway drying in users of CPAP (407). This is
of greatest concern in patients with excessive secretions, who are at high risk of
sputum retention. Consideration should be given to heated humidification when NIV
is used in CF.
3. Inhaled therapies
Patients with CF often require frequent and multiple inhaled therapies, such as
bronchodilators and mucolytics. Consideration must therefore be given to the route of
administration of these therapies in the patient using NIV. For some patients, breaks
from NIV may be appropriate, at which time inhaled therapies can be given via their
usual route. For NIV-dependent patients however, administration of inhaled therapies
during NIV will be required. Connectors for MDIs are available; alternatively, a T-
piece connector for nebulisation can be used. The circuit may need to be altered to
ensure that the nebuliser is connected between the patient and the exhalation port.
Sleep disordered breathing and chronic respiratory failure
Hypoxia and hypercapnia occur commonly during sleep in moderate to severe CF
(408, 409). These alterations in gas exchange are frequently seen during sleep prior to
being evident in the daytime (410, 411). Repeated deterioration in gas exchange
during sleep may impair ventilatory drive and result in daytime respiratory failure
(412). Positive short-term effects of NIV during sleep in CF have been reported (410,
413). NIV is more effective than either oxygen therapy or CPAP in treating sleep-
related hypercapnia and prevents rapid eye movement (REM) related decreases in
minute ventilation. The major benefit of NIV during sleep, therefore, is prevention of
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alveolar hypoventilation and hypercapnia whilst providing the same degree of
correction of hypoxia as supplemental oxygen and CPAP.
Longer-term outcomes of NIV for chronic respiratory failure may include
improvements in daytime PaCO2, reduction in the number of days spent in hospital
and improvement in symptoms (414, 415). Young and colleagues examined outcomes
of domiciliary NIV over a 6 week period in a randomised, placebo controlled study
(416). Significant improvements in quality of life, chest symptom scores, dyspnoea,
nocturnal ventilation and increased exercise performance were reported. The
sustained reduction in nocturnal carbon dioxide levels was not reflected in awake
hypercapnia however. Recent retrospective studies have reported a stabilization or
even reversal of the decline in lung function in patients using NIV with advanced
disease (401, 417). These results need to be confirmed in prospective trials.
When initiating NIV for chronic respiratory failure in CF, care should be taken that
effective ventilation is delivered. The rapid, shallow breathing pattern seen during
REM sleep may be associated with impaired patient-ventilator synchrony, especially
if a mouthleak is present. Attention should be given to selection of an appropriate
interface, rectification of mouthleak and use of a back-up rate if required. In some
patients full polysomnography may be indicated to ensure that effective ventilation
has been achieved.
Non-invasive ventilation as an adjunct to airway clearance
Airway clearance techniques are an onerous aspect of CF treatment, especially when
patients are unwell, due to increased ventilatory demand (83), adverse effects on
respiratory muscle performance (418), alterations in gas exchange (77, 419) and
dyspnoea (23). A single session of NIV has been reported to unload the respiratory
muscles during airway clearance in both adults and children with CF, resulting in
decreased dyspnea and preventing oxygen desaturation during treatment (418, 419).
The aim of NIV during airway clearance is to provide respiratory muscle unloading.
Therefore when setting up NIV, the goal should be to provide as much pressure
support as possible by increasing inspiratory pressure (IPAP) as tolerated. Coaching
will be required so that the patient is able to perform forced expirations, cough and
where possible expectorate, without removing the mask. The use of various interfaces
such as nasal pillows or nasal masks to assist with ease of expectoration should be
explored.
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Exercise and non-invasive ventilation
Exercise capacity is correlated with survival in CF, and consequently exercise training
forms an important part of CF management across the lifespan (147). Listing for lung
transplantation further increases the requirement to maintain good physical fitness to
assist with post-transplantation rehabilitation. However, maintaining and improving
exercise capacity poses significant challenges in patients with advanced lung disease
where high work of breathing may limit exercise duration and patients may be NIV-
dependent.
One study has examined the use of CPAP to reduce work of breathing during exercise
in CF (420). Patients who were more hyperinflated and had more severe lung disease
showed improved exercise endurance, reduced oxygen consumption, reduced
dyspnoea, improved oxygenation and reduced work of breathing with CPAP
compared to exercise on room air. This is in contrast to subjects with mild disease
who showed increased oxygen consumption and increased dyspnoea with CPAP.
Bilevel NIV also improved ventilation, reduced desaturation and increased functional
walking performance in children and adolescents with CF (421). These data indicate
that NIV may have a role to reduce work of breathing and improve exercise
performance in patients with advanced lung disease. More recently, a randomised
controlled crossover trial showed that six weeks of nocturnal NIV significantly
improved the MSWT compared to nocturnal oxygen or placebo (416). The
mechanism for this improvement is not clear, but warrants further examination.
In clinical practice, NIV is used to assist exercise training in patients with CF where
severe dyspnoea limits training duration, or in patients who are severely unwell and
bridging to transplantation. In NIV-dependent patients training should not be
commenced until arterial blood gases have stabilised. It is important to allow time for
the patient to acclimatise to NIV at rest before beginning exercise training,
particularly if NIV has not previously been used. The aim of NIV during exercise is to
reduce work of breathing and therefore the patient should be given as much pressure
support as possible by increasing IPAP as tolerated. This will often require titration
during exercise. A full face mask may be required during exercise to prevent
mouthleak.
General considerations for NIV
Whether using NIV to treat acute exacerbations, chronic hypercapia, during
physiotherapy or exercise, the ventilator settings should be adjusted to reduce work of
breathing. Clinically these settings are determined using a combination of subjective
77
assessments of respiratory comfort and non-invasive clinical monitoring of SpO2,
respiratory rate and use of accessory muscles to support ventilation. This clinical
assessment has been shown to be as effective in reducing work of breathing as using
physiological outcomes (monitoring of transoesophageal and transdiaphragmatic
pressures) in young CF patients (422).
High flow nasal prongs are a useful adjunct to the treatment of people with CF in
acute respiratory failure on NIV. In some patients these can be used during periods off
NIV for eating, nebulisation, ACT and socialising. They may also be useful during the
process of weaning from continuous NIV.
Recommendations
24. Non-invasive ventilation should be considered in all patients with acute
respiratory failure who are listed for transplantation (C).
25. In patients with symptomatic nocturnal ventilatory failure, a trial of
nocturnal NIV may be undertaken (B).
26. NIV is a useful adjunct to airway clearance in patients with severe disease
in whom dyspnoea and fatigue limits effective airway clearance (B).
27. NIV may be a useful adjunct to exercise in patients with severe disease in
whom dyspnoea and fatigue contribute to deconditioning and limit
effective training (B).
28. Heated passover humidification should be incorporated into the circuit
for all applications of NIV in CF (C).
Practice point
When selecting an interface, consideration should be given to ease of
expectoration and prevention of mouthleak during sleep.
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Physiotherapy and Lung Transplantation
Lung transplantation is a well-established treatment which aims to improve the
quality of life and survival of those patients with end-stage lung disease.
Physiotherapists are key members of the transplant team, providing expertise in the
physical and functional assessment, respiratory management and rehabilitation of
patients both before and after surgery.
There are many systemic features of CF which have the potential to impact on lung
transplant suitability and outcomes, including skeletal muscle weakness (195) and
poor bone health (155). Physiotherapy management has a key role in optimising
wellbeing prior to transplantation and restoring the best possible level of function
after transplantation.
Transplant assessment
The assessment of potential recipients is performed by an experienced
multidisciplinary team at a transplant centre with input from the CF team. Extensive
physiological, functional and psychological assessment is undertaken.
Physiotherapy assessment of the potential transplant candidate focuses on the impact
of respiratory and musculoskeletal limitations on exercise, functional capacity and
social performance. Functional exercise capacity is measured with the six-minute
walk test, which is a good predictor of waiting list survival (167) and post-
transplantation outcome (423). Musculoskeletal abnormalities such as reduced muscle
mass, structural/postural thoracic kyphosis and shortened calf, hamstrings and psoas
muscles are commonly seen in candidates with CF. Patients with co-morbidities that
may seriously compromise the outcome of transplantation may be excluded.
The ability to adhere to treatments and to work together with the transplant team is
considered essential for a successful long-term outcome following transplant.
Preparation for transplantation
Time on the waiting list can vary from weeks to years, as organs are matched to
recipients on numerous factors including size, clinical need, blood group and tissue
typing. Once on the waiting list, patients are monitored closely by the transplant team
in order to ensure that candidates continue to meet selection criteria and are in optimal
physical condition for surgery.
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Most adult transplant centres offer dedicated pre-operative exercise training classes
for transplant candidates, in order to optimise physical fitness and strength. A recent
large, retrospective study which included 70 people with CF (424) showed that 6-
minute walk distance was well maintained from the time of listing to the time of lung
transplantation in those who undertook thrice weekly supervised endurance and
resistance exercise training. Those with a greater 6-minute walk distance prior to
transplantation had a shorter hospital stay in the post-transplant period.
Exercise prescription principles in the pre-transplant period should be consistent with
those for other individuals with CF (Chapter 4). Supervised exercise training 2-3
times a week and a home exercise routine are encouraged. Patients who regularly
attend other centres for ongoing outpatient treatment should be supervised by their
local physiotherapist and progress reported back to the transplant team.
Post-operative period
Physiotherapy treatment in the post-operative period aims to:
Optimise ventilation
Clear retained lung secretions
Promote independent function (ie bed mobility, transfers, ambulation)
Improve fitness/ activity tolerance
Facilitate self-management
The physiotherapy program is initiated in the intensive care unit (ICU) as early as the
first postoperative day. Sufficient analgesia is needed to allow effective airway
clearance and early mobilisation. Lung transplant patients often have a poor ability to
perceive the presence of secretions and this may persist in the long term, due to poor
cough reflexes from denervation post transplant (425). If sputum retention becomes a
problem for a recipient, inhalation therapy and an appropriate airway clearance
technique should be instituted.
On the ward, physiotherapy treatment focuses on achieving independence with
activities of daily living, increasing endurance (walking, stationary cycling, stair
climbing), and exercises addressing any specific musculoskeletal deficits. On
discharge, supervised rehabilitation usually continues as an outpatient at the transplant
centre for up to three months.
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Rehabilitation post transplant
Exercise rehabilitation is an established therapy for lung transplant recipients (426).
Although studies in CF are uncontrolled, three months of post transplant rehabilitation
has been associated with improvements in functional exercise capacity, strength and
quality of life in both adults (427) and children (428). A recent randomised controlled
trial of three months of rehabilitation in lung transplantation recipients with other
respiratory disorders, performed immediately following hospital discharge, showed
significant improvements in daily physical activity, quadriceps force and 6-minute
walk distance at one year following transplantation (429).
Patients attend a formal outpatient rehabilitation program comprising of exercise
training and education at the transplant centre. Primary goals include:
Improve physical condition (strength, endurance, posture)
Promote independence in maintaining and monitoring physical condition.
Improve the patient’s confidence in becoming involved in a full range of
activities of daily living and appropriate exercise activities
Nurture realistic expectations for employment, sport and leisure activities
The content of post transplant rehabilitation programs generally includes aerobic and
resistance exercise, performed at least three times per week in an outpatient setting
(426). Although most rehabilitation takes place in a group setting, patients with
resistant organisms may need isolation from other immune suppressed patients with
individual sessions during rehabilitation.
By approximately 12 weeks, most patients have achieved a good level of fitness and
function (426) and are able to be discharged from physiotherapy with a maintenance
home exercise program to be undertaken independently. Patients are encouraged to
maintain an active lifestyle. Those patients who require further rehabilitation are
referred closer to their local area where access to services is more convenient.
Transplant-related problems
Lung transplant recipients commonly experience problems that may require
physiotherapy intervention during hospital admission or outpatient follow up. These
include:
Acute or chronic respiratory tract infection
81
Acute or chronic graft dysfunction
Musculoskeletal morbidities (osteoporosis, pathological fracture, myopathy)
Sternal instability
Diaphragm dysfunction
Reduced functional performance
Declining exercise capacity
Changes in social and vocational roles
Because of these ongoing issues, consideration should be given to yearly assessment
of patients with CF who have undergone lung transplantation.
Recommendations
29. Patients with CF should undertake an exercise program designed to
optimise their physical function while on the transplant waiting list (C).
30. Patients with CF who have undergone lung transplantation should
participate in a formal, supervised rehabilitation program post-
operatively (B).
Practice Points
To ensure comprehensive assessment of patients with CF prior to lung
transplantation, consider the need for direct communication between the CF
physiotherapist and the transplant physiotherapist, in addition to
communication with other members of the multidisciplinary team.
Physiotherapy management early following lung transplantation follows
principles similar to the management of other thoracic surgery patients.
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Cystic Fibrosis and the Intensive Care Unit
Intensive Care for Reversible CF Complications
In some centres, people with CF who have severe but reversible conditions such as
haemoptysis, pneumothorax, antibiotic desensitisation and distal intestinal obstruction
syndrome are managed in the intensive care unit (ICU). Although there are limited
data describing outcomes following ICU admission in CF, several studies have
reported ICU survival of more than 80% for patients admitted to the ICU with
reversible complications (430, 431). In those patients requiring mechanical ventilation
for reversible conditions, longer intubation time has been associated with worse
outcome (430).
Intensive Care for Respiratory Failure
The admission of CF patients to the intensive care unit for the management of acute
on chronic respiratory failure remains controversial. There have been mixed results
for survival following intubation for respiratory failure in patients with end stage lung
disease. Efrati et al (431) reported 17 out of 18 adult patients dying before discharge,
however Hayes and Mansour (432) report 10 out of 10 adults surviving to 6 months
without lung transplantation. A retrospective analysis of 34 intubations reported 59%
survival to discharge of 22 patients requiring intubation as a result of
haemoptysis/pneumothorax and 33% of 12 patients requiring intubation for
respiratory exacerbation (433). Interestingly, studies have reported 100% survival in
children requiring intubation under the age of 18 months (434, 435) and 78% survival
in children under five years of age (436).
Due to the known benefits of NIV for acute on chronic respiratory failure in CF (395-
401), it is widely accepted that patients should not be considered for invasive
mechanical ventilation unless they have failed a trial of NIV.
Extra-corporeal Membrane Oxygenation (ECMO)
Extracorporeal membraneous oxygenation (ECMO) is increasingly being used as a
bridge to lung transplantation. The circuit for veno-venous ECMO, used to bypass the
lungs and provide respiratory support, is displayed in Figure 1. Although there is very
limited evidence for its use in CF (437), it may have advantages over mechanical
ventilation as it reduces the need for sedation and paralysis, it allows the patient to
participate in physiotherapy and allows the patient to eat and drink. Three case series
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have reported a total of nine individuals with CF who were safely and effectively
bridged to transplantation with veno-venous ECMO (437-439). Active rehabilitation
during the ECMO period included airway clearance, range of motion, early assisted
mobilisation including standing and walking, and strength and endurance exercises.
Some authors have suggested that the key to success is the early application of ECMO
after the need for mechanical ventilation is established (437).
Figure 1. Circuit for veno-venous ECMO.
From the Alfred Health ECMO Guideline. Used with permission.
Physiotherapy for CF in Intensive Care
Physiotherapy management of patients admitted to the ICU should follow the usual
principles of individualised assessment and treatment detailed elsewhere in this
document. Good communication between the CF and intensive care teams is
imperative for establishing physiotherapy treatment goals and optimal patient
management.
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Palliative or end of life care in cystic fibrosis
The model of care for palliative/end of life care in CF differs from the model
described for other diseases (eg cancer, AIDS) as there may not be a clear transition
from active treatment to palliative care. Palliative care is introduced in parallel to
active treatment as both therapeutic models help to relieve symptoms (440). The
likelihood of lung transplantation will influence decisions regarding when to move
from aggressive treatment to support and/or comfort care (441). Ninety percent of
patients die of respiratory failure (442) and most patients die during a pulmonary
exacerbation and/or viral infection (443).
The aim of end of life care is to help provide the best quality of life and comfortable
death for the patient in accordance with his/her wishes. The care should focus on
comfort and dignity and be tailored to each patient’s goals and values (444).
Increasingly, guidelines recommend that palliative care should be discussed earlier,
rather than when patients are in the end stage of their disease (445). This could occur
at transition from the paediatric to the adult unit; during an annual review; at the
consultant’s discretion; or at a time when a patient’s health declines, particularly
when due to lack of adherence. Long term, planned palliative care can empower
patients and enable them to be active in the decision-making process regarding their
end of life care (i.e. Advanced Care Directives, wills, Not For Resuscitation orders).
The palliative/end of life care management should be interdisciplinary and include:
skilled management of the symptoms causing discomfort
maximising quality of life
family/carer education and training
respite care for family and carers
assessment and treatment of psychological, social and spiritual needs
assistance to the patient, family and carers with planning for the end of life and
after
loss, grief and bereavement support (12).
Interdisciplinary care consists of continuing care from the CF team integrated with the
specialist palliative care team and, if the patient chooses to die at home, the pastoral
care personnel. In a study of all known CF deaths in Canada in 1995, 90% of patients
received on-going care from the CF physician and 7% received most of their care
from a family physician (443). Terminal care should be organised in the place chosen
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by the patient and their family where possible (440).
Clinical indicators of short-term survival include:
inability to maintain metabolic compensation for chronic respiratory acidosis
increased rate of decline in pulmonary function tests
lack of response to prolonged intravenous therapy
weight loss that can’t be halted with supplemental feeding / parental nutrition
increases in headache and chest pain (446).
Symptoms that cause discomfort in patients with CF include:
pain, including chest pain and headaches
dyspnoea
fatigue
weight loss
anxiety
depression
When to change from active treatment to palliative care
It is difficult for clinicians and family to decide when to change the focus of care.
During their last year of life many patients have increased admissions for pulmonary
exacerbations. During most of these admissions patients will respond to antibiotics +/-
short-term NIV and it is difficult to determine which exacerbation is the final one. As
a result preventative/therapeutic care (such as intravenous antibiotics, assisted
ventilation, vitamins and airway clearance) often continues even within the last 24
hours of life (447). The majority of patients will receive palliative care/comfort
measures alongside these treatments (443, 447).
The impact of lung transplantation on end of life care
For patients who are awaiting lung transplantation, the objective is to keep the patient
as well as possible until potential donor organs can be found and, as such, this goal
may warrant more invasive interventions such as mechanical ventilation, NIV and
enteral feeding (441). Prolonged waiting time and organ scarcity means some patients
may not survive to receive a transplant (448) and, as such, end of life care remains an
important subject for all CF patients with severe lung disease, including those who are
awaiting lung transplantation (449).
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Physiotherapy treatment at end of life in CF
There is no published literature specifically pertaining to physiotherapy treatment in
the terminal stages of CF.
The aims of treatment depend upon whether the patient is:
actively waiting for transplant
deteriorating whilst waiting for transplant
unsuitable for transplant
declines transplant listing
In the patient who is deteriorating but still waiting for transplantation, it may therefore
be appropriate to continue with therapeutic care in the terminal stages if the patient
wishes to do so. However in the dying patient in whom transplant is not a prospect,
treatment should be used only to relieve symptoms. The patient’s wishes regarding
the amount of treatment should be respected and if the patient is tired or does not want
treatment it should be omitted. Due to the long-term relationship of patients with their
clinicians, it may be helpful for the CF physiotherapist to continue to see the patient in
a support role even after active treatment is discontinued (8).
Airway clearance techniques
Minimising the work of breathing during airway clearance is an important
consideration in those with end-stage disease. Airway clearance techniques result in
increased ventilatory demand (83) and may compromise respiratory muscle
performance in those with severe disease (418, 419). Although many patients will be
able to continue the use of independent techniques, some patients require therapist-
assisted airway clearance such as percussion or thoracic compressions, and education
regarding methods of enhancing breathing control to minimise the respiratory work
required. A mucolytic should also be considered to keep secretions thin and easy to
expectorate. Intubation and ventilation is uncommon in the final stages of the disease,
however NIV may also be useful to unload the respiratory muscles, relieve dyspnoea
or as an adjunct to airway clearance (440). High flow nasal prongs may also be useful.
Other techniques
Towards the end of life, patients often report significant benefits from alternative
techniques such as soft tissue massage (306), positioning for relaxation, trigger point
release, music therapy and use of a fan to blow air on the face. The physiotherapist
can teach the family and carers to assist with many of these techniques, as well as to
assist with cough support and airway clearance.
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Practice points
Physiotherapy treatment at the end of life should be flexible and tailored
to each patient’s wishes.
Airway clearance for patients with end-stage disease should aim to
minimise work of breathing and maximise patient comfort.
Airway clearance may be continued even in the palliative stage if the
patient finds it beneficial to relieve symptoms.
Comfort measures such as soft tissue massage and positioning should be
considered in the palliative patient.
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12 Adherence to physiotherapy in cystic fibrosis
Physiotherapists frequently encounter adherence challenges in both inpatient and
outpatient settings for clients with CF and their families. Adherence to physiotherapy
has been reported as low as 53% in some centres (450). This may vary according to
the component of treatment - adherence to frequency of ACTs has been reported at
51% whilst adherence to duration of ACT sessions was 64% (451). Adherence for
inhaled dornase alfa in children has been reported at 67-84% (452, 453) whilst in
adults this varies from 24-82% (454). Exercise therapy is perceived differently from
other forms of treatment (450), with greater adherence to exercise than airway
clearance techniques. In an Australian study, 17.5% of adults with CF performed
‘exercise only’ in place of traditional airway clearance techniques (455).
Measuring adherence is inherently difficult. Assessment of adherence by patients,
parents and clinicians give consistently higher adherence rates than other
measurement methods (456, 457). For example, self-report data of 50% overall
adherence to treatment regimens was objectively measured electronically as 27% and
by diary as 46%. (451). New technologies may provide opportunities for better
assessment of adherence. The development of nebuliser systems that record usage,
such as the I-Neb® and Prodose® (not currently available in Australia or New
Zealand) have allowed for more accurate assessment of adherence to inhalation
therapy. In a 12-month observational study, children using an I-Neb® maintained
adherence to inhaled colistin at 60-70% (458), while a separate study showed adults
using a Prodose® maintained adherence to inhaled colistin at only 50% (459).
Many barriers to adherence exist, varying from patient-related factors, social and
economic, therapeutic, and healthcare related factors (460). Reasons for non-
adherence to treatments across all age groups include: boredom of techniques/therapy,
desire to be normal, forgetfulness, causes embarrassment, too time consuming,
interference with other activities, feeling the treatment is not needed or that it won’t
work, and also feeling too unwell (455, 461-464). A structured daily routine may
improve adherence. In a group of adolescents who provided nebuliser data from the I-
neb® over the course of a scholastic year, adherence was significantly better during
term-time and on weekdays as compared to school holidays and weekends (465). In
order to address non-adherence, an individual’s barriers to adherence must first be
identified.
Clinical and demographic variables are poor predictors of treatment adherence.
Knowledge and adherence are not correlated but more frequent contact with the health
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care team increases adherence (466). The degree of adherence to treatment is
influenced by a person’s style of coping and self-perceptions. It has been reported that
adherent clients scored higher on optimistic acceptance scale and hopefulness scale,
whilst partially adherent clients used distraction as a way of coping and non-adherent
clients used avoidance as a coping strategy (467). Therapeutic adherence improves
with strong personal beliefs in the effectiveness and necessity of the treatment being
undertaken (468) and when treatments have a perceived greater influence on quality
of life (462) or immediate symptomatic benefit. Consequences of poor adherence
include increased morbidity and mortality, reduced quality of life and increased health
care costs (469-473). Strategies to maximise adherence are therefore important to
long-term outcome in CF.
Physiotherapy practice
Adherence to prescribed ACTs, inhalation therapy and exercise should be explored
openly and non-judgementally at each visit. Engaging in a partnership approach, to
identify and discuss barriers to adherence openly and normalise difficulties opens the
way to behavioural change (474).
Many approaches to address individual adherence problems have been suggested,
including use of effective resource materials for education; tailored treatment
regimens to suit lifestyle; reduced complexity of home programs; and additional
contacts in person, phone or email to support and encourage adherence. It is important
that the patient understands all aspects of the treatment prescribed. One study
demonstrated that 33% of non-adherence with airway clearance techniques was due to
the patient following what they perceived to be the recommendations from team,
concluding that a portion of non-adherence is related to miscommunication (475).
Formal coaching and interviewing strategies that encourage collaboration and
affirmative interactions between health professional and client may be useful. For
example, motivational interviewing is an effective treatment for modifying behaviour
in chronic conditions (476), however it has not yet been adequately tested in CF
(477).
Management of adherence problems needs to be individualised with regard to
lifestyle, education needs, and multiple other influences. Multidisciplinary team
involvement to develop client-focussed management plans is suggested as such
problems may be outside the scope of core physiotherapy practice.
Age-appropriate approaches to encourage adherence include but are not limited to:
Infants: Role modelling and parent support in developing skills.
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Toddlers: Incorporate play, songs, and routines in a dynamic and positive session.
Children 4-8 years: Develop a dynamic activity based routine that incorporates ACT
and positive behavioural feedback for cooperation.
Children 9-12 years: Incorporate an airway clearance routine with some control
given to the child, coached by parent, on the background of an active lifestyle which
includes involvement in team sports, dance, or other preferred physical pursuits.
Adolescents: Identify individual goals and routines and how ACTs and exercise can
fit in to their timetable. Encourage sports participation especially for those with less
severe respiratory impairment; group exercise environments may still be appropriate
for those with greater functional impairment.
Adults 18-25 years: Explore individual goals and barriers to goal achievement;
continue to educate and support positive aspects of actions. People with CF tend to
prefer self-administered ACTs (478), which may promote adherence.
Adults 25+: Continue to support and advise regarding enjoyed activities, participation
options, and modifications if needed to ACT and exercise.
Practice points
Adherence to prescribed ACTs, inhalation therapy and exercise should be
explored openly and non-judgementally on a regular basis.
Management of adherence problems should be client-focussed and
individualised accordingly.
Effective communication strategies between the patient and CF team may
help resolve some aspects of non-adherence.
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13 Infection Control during physiotherapy in cystic
fibrosis
Respiratory tract infections have a significant impact on morbidity and mortality in
CF (479). Good infection control practices are critical to preventing transmission of
pathogens. Segregation and cohorting of inpatients and outpatients according to
respiratory organisms are now routinely practiced, both nationally and internationally
(480, 481).
The following respiratory organisms are commonly found in CF and may require
specific infection control practices: Pseudomonas aeruginosa , Staphylococcus aureus
(including MRSA), Burkholderia cepacia (B. cepacia), B. mallei and B. pseudomallei,
Aspergillus and Acinetobacter fungi, Stenotrophomonas maltophilia, Heteroresistant
vancomycin intermediate Staphylococcus aureus (hVISA) and Vancomycin Resistant
Enterococci (VRE) (482, 483). Because there may be differences in virulence and
outcome associated with different clones, even patients with the same organism may
need to be managed in separate cohorts (484). Physiotherapists should therefore be
familiar with the cohorting requirements of their own centre.
Education of patients and families regarding the importance of infection control and
discussion of infection control guidelines increases the knowledge and confidence of
patients and families in following these guidelines. Other factors suggested to
facilitate compliance with infection control include ready availability of materials to
perform hand hygiene (485).
Physiotherapy practice
A survey of infection control guidelines and practices amongst 19 CF centres across
Australia indicated widespread awareness and recognition of the importance of
practices to minimise the risk of transmission of organisms from patient to patient.
However, there is significant variation in infection control policies between centres,
which reflects the lack of robust scientific evidence in this area. It is therefore
important that physiotherapists familiarise themselves with local infection control
guidelines. The following practice points are based on consensus. Areas where there
is no consensus are identified.
Airway clearance and inhalation therapy
Both Pseudomonas aeruginosa and B. cepacia may be spread in droplet form by
coughing and these organisms can survive on dry surfaces for a number of days (483,
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486, 487). There is also potential for airborne transmission (488). B. cepacia has been
isolated from the hospital rooms and hands of patients following airway clearance
(489-491) and has been isolated from the outside surfaces of sputum cups (489).
These findings reinforce the need to segregate patients whilst performing airway
clearance and inhalation therapy, as well as the importance of hand washing to
prevent person to person transmission. Stethoscopes should be cleaned with alcohol
wipes between patients (492).
Bacterial contamination of home nebulisers of CF patients has been documented and
sharing equipment has been associated with transmission of B. cepacia (140, 493,
494). Under no circumstances should any respiratory equipment be shared between
patients with CF. Recommendations for cleaning respiratory equipment can be found
in Chapter 3. In most Australian centres the responsibility for cleaning respiratory
equipment lies with patients or families, with the advice and encouragement of
physiotherapists and nursing staff. Adherence to nebuliser disinfection guidelines can
range from 36-79% (495).
There is no consensus regarding the use of gloves, gowns and masks during
physiotherapy treatment in CF. Physiotherapists should consult their local infection
control policy with regard to when these measures are required.
Gym Sessions and Exercise
Coughing is common during exercise in patients with CF and therefore droplet spread
of organisms is likely. These droplets may be transmitted within one metre of an
infected patient (483) although it has been reported that contamination can occur at a
distance between 1 and 2 metres, although the probability is low (1.7%) (496). It is
therefore recommended that patients with different organisms, or in different cohorts,
do not exercise together. When people who are considered suitable for cohorting are
sharing the gym, universal precautions should be practised. Patients should be
educated and encouraged to maintain a two metre distance from other patients at all
times; patients should be taught handwashing on entering and leaving the gym; and
should be taught and encouraged to wipe down all exercise equipment with an
alcohol-based solution before and after use. All patients should be encouraged to
adopt behaviours which limit the spread and acquisition of organisms.
The consensus of Australian and New Zealand physiotherapists is that patients with B.
cepacia and MRSA should exercise on their own in the hospital setting, whilst
inpatients with VRE or hVISA should exercise in their own room or outdoors. In
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many centres exercise equipment is brought into the patient’s room to facilitate
exercise training.
Outpatient Practice
Exam rooms should be cleaned in between each patient to reduce surface
contamination and sinks should be frequently disinfected. Patients and staff should
perform frequent and repeated hand hygiene with an alcohol based hand rub
throughout outpatient clinic visits (487, 497). Some studies also recommend cohorting
of clinic rooms depending on microbiology (487). The introduction of cohorting has
been shown to reduce the prevalence of epidemic strains of Pseudomonas aeruginosa
(498).
Practice points
Physiotherapists should be aware of the colonising organisms and
cohorting requirements for each of their patients.
Patients should not share airway clearance therapy or inhalational
therapy equipment under any circumstances.
Patients should clean their own airway clearance equipment at home and
in hospital as advised by the CF team or local infection control
department.
Patients who are unsuitable for cohorting should not exercise in the same
gym area at the same time.
Patients with B.cepacia and MRSA should exercise on their own in the
hospital setting.
Inpatients with multi-resistant organisms (such as VRE or hVISA) should
exercise in their own room and outdoors.
Staff should practice strict handwashing between patients and
stethoscopes should be wiped with alcohol before applying to patients.
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14 Delivery of physiotherapy treatment to inpatients
and outpatients with cystic fibrosis
There is little published research regarding the optimum structure of physiotherapy
services for people with CF. There are no controlled trials to guide practice in this
area. The following recommendations represent expert clinical opinion and are
consistent with the Standards of Care for Cystic Fibrosis in Australia (349) and New
Zealand (499).
Physiotherapy treatment for inpatients
Patients should be assessed and physiotherapy treatment started on the day of
admission (349). The physiotherapy service should therefore be available seven days
a week. The physiotherapy service should be led by a physiotherapy clinician with a
special interest in CF in order to facilitate continuity of care.
Treatment should be tailored to the patient’s clinical status and based on the clinical
assessment findings. The physiotherapy treatment plan should specifically address
inhalation therapy, ACTs and physical exercise.
Airway clearance therapy should be tailored to the patient’s clinical status,
considering the most efficacious regimen and each patient’s personal preferences.
Patients who are admitted with an acute exacerbation with increased and/or retained
secretions will need to carry out more frequent ACT sessions than their baseline daily
regimen. The number of treatments will range from two to three or more treatments in
24 hours. Care should be taken to match the dosage (number and duration) of
treatment sessions to each patient’s condition. Physical exhaustion from too high a
dose of physiotherapy should be avoided. Timely initiation of non-invasive
ventilation should be considered where indicated. Ideally, an on call physiotherapy
service should be available after hours to provide treatment to patients with severe
respiratory insufficiency related to excessive and/or retained airway secretions who
are at risk of deterioration overnight.
A graduated physical exercise program incorporating cardiorespiratory exercise as
well as stretching, strengthening and mobilising exercises to normalize physical
function should be commenced as soon as the patient is in a fit state to do so. Patients
should not exercise while febrile or while they are requiring much of their physical
strength and energy for work of breathing, airway clearance therapy and coughing. An
objective measure of exercise tolerance using oximetry should be undertaken to assess
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baseline exercise capacity, to identify oxygen desaturation during exercise and to
assess whether supplemental oxygen is required.
During admission, age-appropriate assessment should be undertaken of comorbidities
such as urinary incontinence and musculoskeletal limitations. Prior to discharge, an
agreement should be made with the patient and / or family regarding airway clearance
therapy and exercise to be undertaken at home.
Physiotherapy treatment for outpatients
Patients attending the outpatient department of the Cystic Fibrosis Service should
have access to a physiotherapist with expertise in CF management at each clinic visit.
It is suggested that each patient with CF be assessed by the physiotherapist three to
six monthly so that their physiotherapy program can be reviewed and optimized.
Complex patients (eg during pregnancy, those listed for transplantation) may require
more frequent and detailed review. One uncontrolled study involving 12 adolescents
with CF has shown a reduction in the need for intravenous antibiotics with fortnightly
review by a physiotherapist; however controlled data are needed before this intensive
practice can be widely recommended (500).
A formal annual review by the CF team, including physiotherapy review, has been
advocated for people with CF (349, 499). However this does not occur in all settings
due to resource constraints (164). Regardless of whether such a formal review occurs,
each patient should complete the following reviews with their physiotherapist at least
once per year:
demonstrate their daily airway clearance therapy routine in a practical session
with their physiotherapist
go through their inhalational therapy program together with cleaning and
replacement requirements of all respiratory equipment. Arrangements should
be made for annual check and servicing of nebuliser pumps
carry out an age-appropriate exercise tolerance test, especially in adulthood
have a review of postural alignment, musculoskeletal and physical function. A
preventative and/or rehabilitative exercise program should be instituted as
appropriate to the age, condition and function of the patient
have an assessment of adherence to the physiotherapy program with
modifications as necessary, always considering the patient’s individual needs,
lifestyle, economic status and personal preferences in order to achieve an
optimum long term outcome.
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Practice points
Inpatients with CF should be assessed by a physiotherapist within 24 hours
of hospital admission and treatment commenced at the earliest opportunity.
The physiotherapy treatment plan for inpatients with CF should specifically
address airway clearance therapy, inhalation therapy and physical exercise.
Outpatients with CF should be reviewed by a physiotherapist every three to
six months.
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15 Directions for Future Research
There are a number of areas where insufficient evidence was available to the writing
team to make recommendations for physiotherapy practice. Priority areas for research,
where additional data are likely to have a clinically important impact on practice were
identified by the writing team. Key research questions that should be addressed in
robust trials include:
What is the most effective airway clearance technique for infants with CF?
What is the impact of physiotherapy techniques (positioning, percussion,
vibration) on GOR across the lifespan in CF?
Is PEP therapy equally effective when delivered using a mouthpiece compared
to a mask?
Is there an advantage to combining inhalation therapy and airway clearance
techniques?
Is physical exercise an effective stand-alone airway clearance technique for
people with CF, particularly in those with mild disease?
What is the role of strength training in children with CF?
What are the optimal prescriptions for exercise training and physical activity
participation for people with CF across the lifespan?
What is the role of exercise training in management of CFRD?
Does treatment of musculoskeletal complications impact on long term
outcomes in CF?
Are there long term benefits of nocturnal NIV in people with CF and chronic
respiratory failure?
98
16 References
99
Appendix 2: Three-minute step test protocol
Outcome Measures
• Heart rate
• Oxygen saturation
• Perceived level of breathlessness (eg Borg score)
Equipment
• Standard 6 inch (15 cm) step
• Stopwatch
• Metronome
• Pulse oximeter
• Borg scale
• Recording sheet on a clipboard
• A non-stick mat is optional and may be advised if the patient has any lower limb
joint pain
Safety
Oxygen saturation should be recorded continually and the test ceased if saturation
drops below 80% (or as indicated by respiratory physician).
Procedure
Set the metronome to 120 beats per minute, giving a stepping rate of 30 steps per
minute.
The test should be explained to the patient prior to commencement. Patients should be
given opportunity to practise the technique, rhythm and timing of stepping to the
metronome and the technique for changing the leading leg should be demonstrated.
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Allow adequate time for return to resting pulse rate and oxygen saturation levels
following practice. The patient should be told that they can stop the test at any time if
they are unable to continue.
Baseline pulse rate, oxygen saturation and Borg breathlessness rating should be
recorded prior to commencing the test.
Standard encouragement should be given at 1, 1.5 and 2 minutes. ‘You are one minute
/ half way / two minutes through the test and you are doing well’.
At the conclusion of the test, record the lowest value of SpO2 and the highest value of
pulse rate and note the times these occur. Ask the patient to rate their perceived
breathlessness on the Borg scale. If the patient stops stepping or the test is stopped
before the three minutes is completed, record the duration of stepping, number of
steps, reason for stopping as well as pulse rate and saturation at the time of stopping.
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Appendix 3: Modified shuttle test protocol
Outcome Measures
• Distance
• Heart rate
• Oxygen saturation
• Perceived level of breathlessness (eg BORG score)
Equipment
• Shuttle CD* and CD player
• Pulse oximeter
• Borg scale
• Recording sheet on a clipboard
• Cones to mark the turn-around points, placed 9 metres apart
Procedure
The modified shuttle test is a 15-level or 25-level walk/run shuttle test performed at
increasing speeds back and forth on a 10 metre course with an audio signal to indicate
the times the marker cones should be reached.
Patients should be familiarised with the procedure by conducting a practice test.
Measures of resting pulse rate, oxygen saturation and perceived level of
breathlessness using the Borg breathlessness scale should be taken prior to
commencing the test. If you need to determine levels of desaturation the probe should
remain on the patient throughout the test. If the patient voluntarily stops the test,
document the reason why, for example pain, breathlessness or other.
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There should be standardised verbal encouragement at the end of each level:
“good,keep going, you are doing well” and remind them they can run at any time if
they need to keep up the beeps.
The patient should continue the test until they are unable to do so, experience
desaturation to less than 80% or fail to maintain the set pace (0.5m away from the
cone when the beep sounds on two consecutive shuttles).
The distance covered, pulse rate, oxygen saturation, perceived level of breathlessness
using the Borg scale and the reason for stopping the test should also be recorded at the
completion of the test.
MST-15 is available from Dr Sally Singh, Dept of Respiratory Medicine,
Glenfield Hospital NHS Trust, Groby Road, Leicester LE3 9QP, UK
MST-25 is available from Mark Elkins, mark.elkins@sydney.edu.au
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Appendix 4: Musculoskeletal Screening Tool
A. Does pain limit your functional activities on more than two occasions per
week?
No
Yes - VAS/10_________
Source
Thoracic
Lumbar
Other________________
B Bone Mineral Density
Not applicable
Normal
Z score < -1
Z score < -2
# in last 12 months, site and mechanism__________________
C Thoracic kyphosis? (√ for Yes)
Head to wall
Radial styloid to wall
Clasp hands behind back with wrists together
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Appendix 5: Pelvic floor exercises for people with CF
1. Teach patients ‘the knack’ to protect them from leaking during increased load
to the pelvic floor during coughing, huffing, sneezing, laughing etc.
‘The knack’ is a contraction of the pelvic floor (a form of bracing of the pelvic floor)
prior to any activity that increases pelvic floor loading. This should become a lifelong
habit similar to the lifting strategy of bending the knees and keeping the back straight
during heavy lifting to prevent back injury.
2. Teach patients to carry out strength and endurance training of the pelvic floor
and lower abdominal muscles. These should be taught to those who experience
leaking during sneezing, nose blowing, coughing and huffing.
Instructions for the patient:
Pull the pelvic floor up towards the diaphragm
Hold for 3-5 seconds
While holding, superimpose three quick contractions – ‘pull up, up,up’
Dosage is 3 x 10 sets per day.
3. Teach optimal positioning for airway clearance therapy
Airway clearance should be carried out in positions that enhance pelvic floor function
(367).
In sitting, the lumbar spine should be held in a neutral or extended position to help
lower abdominal and pelvic floor muscle activity to provide maximum protection
against urinary leaking during all forms of airway clearance therapy.
If airway clearance is done sitting on a chair, the feet should be flat on the floor with
a 90 angle at the hips and knees to further improve pelvic floor control and guarding
against leaking during physiotherapy treatment.
Use “the knack” (pre-contraction) and “straighten the back” before increasing stress
to the pelvic floor including during huffing and coughing regardless of the
circumstances. If leaking feels imminent apply manual pressure over the pelvic floor
region or cross the legs if in a standing position.
Patients who continue to have a problem with bladder and bowel control need to be
referred to a specialist continence physiotherapist for a course of treatment.
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Appendix 6: Conflict of Interest Statements
Jenny Bishop Nothing to declare
Ryan Black Conference support, Novartis, 2013
Summar Bowen Nothing to declare
Brenda Button Pharmaxis Allied Health Advisory Committee; Novartis
Allied Health Advisory Committee; Vertex Allied Health Advisory Committee;
Conference support, Roche; Conference support, Pharmaxis.
Robyn Cobb Conference support, Novartis
Narelle Cox Conference support, Roche.
Rebecca Davis Nothing to declare
Rosie Day Conference support, Roche, 2007 and 2009
Julie Depiazzi Nothing to declare
Ruth Dentice Nothing to declare
Katherine Doiron Nothing to declare
Michael Doumit Nothing to declare
Tiffany Dwyer Nothing to declare .
Alison Elliot Nothing to declare
Louise Fuller Nothing to declare
Kathleen Hall Nothing to declare
Anne Holland (Editor) Nothing to declare
Matthew Hutchins Nothing to declare
Nikki Katz Nothing to declare
Melinda Kerr Nothing to declare
Annemarie Lee Nothing to declare
Christina Mans Nothing to declare
Anna Middleton Conference support, Solvay, Technipro, Pharmaxis
Fiona Moran Technipro Scholarship 2012
Kate Myslinski Conference support, Pharmaxis 2012. Novartis 2013
Lauren O’Connor Nothing to declare
Angela Potter Nothing to declare
Tshepo Rasekaba Nothing to declare
Rebecca Scoones Nothing to declare
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Ranjana Steward Nothing to declare
Esta-Lee Tannenbaum Nothing to declare
Ben Tarrant Nothing to declare
Nathan Ward Conference support, Roche 2007, Pharmaxis 2012
Samantha West Nothing to declare
Dianne White Nothing to declare
Christine Wilson Meeting support, Pharmaxis
Lisa Wilson Nothing to declare
Jamie Wood Conference support, Roche 2009, Pharmaxis 2012, Novartis
2013; Abbott Products scholarship 2011; Bronchitol workshop 2012.
Michelle Wood Travel support for attendance at clinical trials investigator
meetings, Vertex (2010 – 2014), PTC (2014), Aradigm (2014). Advisory board
consultancy fees, Vertex (2014).
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