Paediatric Respiratory Reviews 14 (2013) 263–269

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Paediatric Respiratory Reviews

Review

Physiotherapy in cystic fibrosis: optimising techniques to improve outcomes

S. Rand 1,2,*, L. Hill 1, S.A. Prasad 1
1
Cystic Fibrosis Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK
2
Portex Unit, UCL Institute of Child Health, London, UK

EDUCATIONAL AIMS

 To highlight that CF physiotherapy management has needed to adapt to a more dynamic and individualised approach over the last
2 decades.
 To outline some alternative treatment regimen options for patients with CF and their families, which can be an effective method of
optimising adherence.
 To outline how the introduction of newborn screening has provided new challenges for physiotherapists working in CF.
 To discuss how physical activity and exercise now plays an important role in the management of individuals with CF.

A R T I C L E I N F O S U M M A R Y

Keywords: Optimisation of physiotherapy techniques to improve outcomes is an area of cystic fibrosis (CF) care,
Cystic fibrosis
which has developed considerably over the last two decades. With the introduction of newborn screening
Physiotherapy
and an increase in median life expectancy, the management of individuals with CF has needed to adapt to
Inhalation therapy
Airway clearance therapy a more dynamic and individualised approach. It is essential that CF physiotherapy management reflects
Exercise the needs of a changing cohort of paediatric CF patients and it is no longer justifiable to adopt a ‘blanket’
prescriptive approach to care. The areas of physiotherapy management which are reviewed and
discussed in this paper include inhalation therapy, airway clearance techniques, the management of
newborn screened infants, physical activity and exercise.
ß 2012 Elsevier Ltd. All rights reserved.

INTRODUCTION to optimise physiotherapy techniques in order to potentially

As the range of treatments for CF increases, the clinician is faced
with the dilemma of developing a treatment schedule that
provides optimal benefit, takes into account the impact of an
increasingly complex therapeutic regimen on patient adherence
and which works within the financial restraints imposed on the
system.1 Median life expectancy in CF has increased significantly
over the last two decades.2 The challenge faced by clinicians is to
balance CF treatment needed to maintain optimal health status
(thus allowing people with CF to achieve personal and professional
milestones) with the burden that treatment imposes.3 It is
therefore essential that physiotherapy treatments are optimised
to ensure clinical efficacy and optimal outcomes. In essence, the
aim of paediatric CF management is to transition ‘healthy’ children
to adult care with normal lung function and nutritional status. The
purpose of this review is to discuss some of the methods available
* Corresponding author. Tel.: +44 2079052226; fax: +44 2078298634.
E-mail address: Sarah.Rand@gosh.nhs.uk (S. Rand).
improve outcomes.
INHALATION THERAPY
Inhalation therapy is an important part of CF physiotherapy
care and is aimed at altering the properties of the airway surface
liquid and/or reducing the viscosity of mucus to facilitate
mucociliary clearance.1 The most common mucoactive agents
pertinent to CF physiotherapy care are dornase alfa (rhDNase) and
hypertonic saline (HTS). When incorporating inhalation therapy
into CF therapeutic routines careful consideration should be paid
to the timing of treatment, the delivery method of these nebulised
medications and adherence.
Timing of inhalation therapy
Hypertonic saline
Hypertonic saline (HTS) is a sterile salt-water solution,
delivered via inhalation therapy, usually at a concentration of
between 3% and 10% in volumes of 3 ml to 10 ml via a nebuliser.4
HTS has been shown to accelerate mucociliary clearance in the CF

1526-0542/$ – see front matter ß 2012 Elsevier Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.prrv.2012.08.006
264 S. Rand et al. / Paediatric Respiratory Reviews 14 (2013) 263–269
airway5,6 and three mechanisms are believed to contribute to this
improvement. Firstly, restoration of the depleted airway surface
liquid volume occurs which peaks almost immediately after a dose,
but which may be sustained for several hours.7 Secondly, there is
an improvement in mucus rheology8 and thirdly stimulation of
cough.5,6 Significant improvements in lung function, quality of life
and ease of expectoration with short and long-term use of HTS
have been reported.4,9
The clinical trials which have demonstrated the clinical efficacy
of HTS, have all delivered the medication before airway clearance
therapy. As a result the currently accepted clinical practice for
nebulised HTS is that each dose is nebulised immediately prior to
airway clearance treatment. Alternative timing regimens have
been suggested and are empirically used in clinical practice. These
include the proposed use of hypertonic saline after airway
clearance therapy when the excess bronchial secretions have in
theory been mobilised. A recent systematic Cochrane review
reported no current evidence to indicate that alternative timing
regimens are any more efficacious than the current practice.10 A
recent study of CF adults evaluated the use of HTS before, during
and after airway clearance therapy. There was a non-significant
trend towards an improvement in lung function when HTS was
inhaled before airway clearance compared to during or after
treatment. Satisfaction and perceived effectiveness were highest
when HTS was inhaled before or during airway clearance.
Tolerability was not affected by the timing regimen and satisfac-
tion was lowest when HTS was inhaled after airway clearance
therapy.11 The differences in perceived effectiveness and satisfac-
tion shown in this study may have important implications for long-
term adherence.4
The advice therefore remains that patients’ nebulise HTS before
airway clearance treatments as this is the only regimen which has
demonstrated clinical efficacy to date.10 The best time of day for
inhalation of HTS has also not been established and clinical
practice should therefore be based on convenience and tolerability
rather than on a ‘‘one size fits all’’ approach.10
Dornase alfa
Dornase alfa (rhDNase) is a genetically engineered version of a
naturally occurring enzyme, which was developed to reduce the
visco-elasticity of sputum. Daily inhalation of dornase alfa has
been shown to reduce the number of pulmonary exacerbations,
improve pulmonary function and is well-tolerated and safe in
patients with mild, moderate and severe CF.12 Therapy with
dornase alfa over a one, six or 12 month period is associated with
an improvement in lung function.13 Alternate day treatment with
dornase alfa has also been shown to be equally effective as daily
treatment in some patients and in those patients using this
simplified alternate day treatment regimen may help to reduce
treatment burden.14
As with HTS there are some recognised practices with regards to
timing of dornase alfa. Traditionally, clinicians advise nebulisation
30 minutes prior to airway clearance therapies (ACTs) and that at
least one hour should be allowed between the nebulisation of
dornase alfa and a nebulised antibiotic to avoid denaturation.3 This
recommendation is based on evidence that dornase alfa makes
sputum more pourable within 30 minutes.15 However alternative
timings have been suggested. Inhalation after airway clearance
treatments may facilitate drug deposition in the more peripheral
airways.16 Inhalation in the morning may capitalise on faster
daytime mucociliary clearance and on the clearance effects of
daytime activities such as exercise. Inhalation in the evening, may
increase the dwell time in the airways since mucociliary clearance
and cough are suppressed overnight.16
A recent Cochrane review concluded that the current evidence
from a small number of subjects does not indicate that inhalation
of dornase alfa after airway clearance is more or less effective than
the traditional recommendation of inhalation 30 minutes prior to
airway clearance techniques (ACTs).16 It has also been hypothe-
sised that for children with well-preserved lung function inhala-
tion before airway clearance may be more beneficial for small
airway function as two small studies reported FEF25 to be
significantly worse when dornase alfa was inhaled following
airway clearance.17,18 Clinicians are often anxious that evening
inhalation may induce cough and impair sleep quality and in
practice this is therefore not routinely recommended.16 In general
it can be concluded that the timing of dornase alfa can be largely
based on pragmatic reasons and individual preference with respect
to the time of airway clearance and time of day.16 Providing
alternative regimens to patients and families can be a very effective
method of optimising adherence to treatments in CF.
Delivery methods
In order to balance treatment demands with lifestyle, therapies
have been developed which allow patients to administer treat-
ments independently and often within the home environment.3
Optimising inhalation techniques is essential to ensure optimal
deposition and drug delivery with each dose. The success of
inhalation therapy is highly dependent on indication, the
administration device and the inhalation technique. New tech-
nologies have been developed to produce more homogenous
particle sizes and to increase the speed of nebulisation.3 These
include vibrating mesh technology (VMT) systems and adaptive
aerosol devices (AAD). These devices are silent, battery powered,
portable and have faster delivery times. In addition AADs also
reduce wastage of medication by using breath actuated technology
and allow clinicians to download technique/usage data which can
facilitate optimisation of technique and adherence.19
In paediatrics the use of a facemask for inhalation therapy is the
first line approach for the infant and younger child, however some
toddlers may be able to manage a mouthpiece.20 As children
become older their tolerance of facemasks may reduce and their
ability to nebulise effectively through a mouthpiece improves. The
use of a nose-clip and mouthpiece in the interim periods can be
useful to ensure optimal deposition of the inhaled medication in
the lungs. Deposition of medication in the nasal space/sinuses may
have some efficacy in patients with nasal obstruction and/or sinus
disease and therefore a facemask may be appropriate for these
individuals regardless of age.
Patients and parents/carers must always be carefully instructed
and trained regarding the optimal inhalation technique and this
should be regularly re-assessed and optimised regardless of the
choice of device.20 In essence an optimal inhalation technique is
dependent on the individuals age, ability, willingness to learn and
the degree of lung disease20 and these factors need to be taken into
consideration at all times.
Appropriate cleaning and maintenance of nebuliser equipment
is essential to avoid bacterial contamination of equipment, to
decrease the potential risk of acquiring pathogens and to ensure
efficiency of the device.21 Written cleaning and sterilisation
guidelines should be provided to all patients and families and
cleaning practices should be reviewed on a regular basis.
Adherence and tolerance
The median number of daily treatments prescribed for patients
with CF is seven (inter-quartile range (IQR) 5–9).22 Poor adherence
has been identified as the greatest cause of treatment failure.
Although technological improvements have reduced the inhala-
tion time considerably, adherence to inhalation therapy remains
poor.
 S. Rand et al. / Paediatric Respiratory Reviews 14 (2013) 263–269 265

Monitoring and improving adherence to inhalation therapy is
challenging as it is predominantly based on patients and parents/
carers self-reporting. Adherence is known to be over-estimated
when self-reporting is used and clinician assessment of patient
adherence plays an important role in the physiotherapy manage-
ment of patients with CF.19 The incorporation of electronic
monitoring within some newer devices (e.g. AADs) allows
clinicians to download information on treatment time, duration
and dose completion which can be used to provide accurate and
detailed longitudinal adherence data.19 Using this technology a
wide discrepancy between median self-reported adherence of 80%
(IQR 60–95%) and actual downloaded adherence of 36% (IQR 5-
84.5%) has been reported.19 It is therefore essential that the
treatments that actually take place are effective and ensure
optimal deposition and drug delivery.
Initiating inhalation therapy can be challenging, particularly in
the paediatric population, and failure at this stage can potentially
affect future adherence to the therapy. A test dose of inhaled
antibiotics, dornase alfa and HTS should be undertaken to assess
suitability, tolerability and/or clinical efficacy of the medication for
the individual and should include spirometry and pulse oximetry
before and after the test dose in a hospital setting.23,24
The initial administration of HTS can be particularly challen-
ging. This is mainly due to the unpleasant taste and the fact that
HTS can cause transient airway narrowing, coughing and
pharyngeal discomfort.4,10,24 These symptoms do become less
severe with regular use and it has been documented that only
about 8% of those with CF find HTS intolerable.24 It is suggested
that an effective way to improve tolerability of HTS is to reduce the
volume of HTS given rather than reduce the concentration. It is also
reported that a second test dose is often tolerated much more
readily and following approximately 10 nebulised doses the
tolerability is much improved.24 If bronchoconstriction occurs
during the test dose routine premedication with a bronchodilator
is recommended.4 Combining HTS and salbutamol in one nebuliser
is also common clinical practice in the UK, which reduces the
overall inhalation time and ensures adequate bronchodilator
administration whilst still administering HTS.
Interspersing or combining inhalation therapy with ACTs (e.g.
positive expiratory pressure (PEP) therapy or high frequency chest
wall oscillation (HFCWO) therapy)25 are also useful practices for
those patients who may find HTS less tolerable as a single therapy
dose (Figure 1). Deposition has shown to be equal or better when
inhalation therapy is combined with oscillating PEP in patients with
chronic obstructive pulmonary disease (COPD).26 In patients with CF
deposition has been reported to be greater in the lung periphery
when inhalation therapy was combined with PEP therapy compared
to conventional nebulisation but patients received 43% less of the
dosage with a longer nebulisation time.27 The evidence is therefore
lacking for this practice but clinicians report an overall reduction in
treatment time (compared to separate inhalation and airway
clearance sessions) when using this practice. Specific devices, which
allow for concurrent nebuliser therapy and airway clearance
treatment are now available, however there is currently no robust
evidence to support their efficacy. Clinicians have reported concerns
regarding the deposition of the mucolytic of the aerosolised drug
when it is used in conjunction with an airway clearance device as it is
required to travel through the ACT part of the device. There has been
some empirical evidence of a reduction in the lifespan of the device
caused by deposition of medication (salt crystals in particular when
used with HTS). Care should be taken to weigh up the pros and cons
when considering one of these devices until more robust evidence is
available.
AIRWAY CLEARANCE THERAPY
Airway clearance techniques (ACTs) remain integral to the
physiotherapy management of individuals with CF.28 There are

Table 1
Airway clearance techniques.

Airway clearance device Types Advantages Disadvantages

Breathing techniques ACBT, AD, AAD, FET
Positive expiratory Facemask PEP, Mouthpiece
pressure (PEP) therapy PEP, Infant PEP,
Hi-PEP
Oscillatory PEP (OPEP) therapy Acapella,
Flutter,
RC-Cornet, Bubble PEP
Conventional physiotherapy (CPT) Modified Postural Drainage
& Percussion (PD&P)
High frequency chest The Vest,
wall oscillation (HFCWO) The Smart-vest
Non-invasive ventilation (NIV) BiPAP, CPAP
Intrapulmonary percussive IMP2 (Percussionaire)
ventilation (IPV)
Intermittent positive pressure ‘The Bird’
breathing (IPPB)
No equipment required AD difficult for young children
Can combine with other ACTs to comprehend.
Portable Relatively inexpensive
Can combine with other ACTs Infection control risk
Provides visual feedback when No oscillatory component
used with a manometer
Individualised settings
Different interfaces available
Portable Relatively inexpensive
Easy to use Infection control risk
Vibratory component Easy to break (acapella and flutter)
Majority can be used in different
PD positions
No equipment required May require assistance
Can combine with other ACTs Can be passive if used in isolation
Can combine with other ACTs and Expensive
inhalation therapy Less portable
Individualised settings Can be passive if used in isolation
Effort independent Can be uncomfortable
Can combine with other ACTs and Expensive
inhalation therapy Less portable
Different interfaces available Potential tolerability issues
Beneficial in the acute setting
Can combine with breathing techniques Expensive
and inhalation therapy Less portable
Different interfaces available Noisy
Beneficial in the acute setting
Can combine with OPEP and inhalation therapy Less portable
Different interfaces available Usually only used in the
Beneficial in the acute setting hospital setting

PD – postural drainage, ACBT – active cycle of breathing techniques, AD – autogenic drainage, AAD – assisted autogenic drainage, FET – forced expiratory technique, PEP –
positive expiratory pressure, OPEP – oscillatory PEP, BiPAP – bilevel positive airways pressure, CPAP – continuous positive airways pressure, VPAP – variable positive airways
pressure.
266 S. Rand et al. / Paediatric Respiratory Reviews 14 (2013) 263–269
many techniques available that require different levels of patient
ability and different types of equipment with varying space,
portability, noise, appearance and cost characteristics (Table 1).3
The rationale for why ACTs are used in CF is well documented.
The underlying theory is that facilitating secretion clearance will
reduce bacterial load, decrease infection and inflammation of the
lungs and thus reduce airway damage, ultimately delaying the
disease process. A systematic review has reported that ACTs have
short-term beneficial effects on mucus transport in CF, however
there is currently no robust evidence regarding their long-term
effects.29
In the last two decades a number of new airway clearance
devices have been developed with the aim of improving efficiency
and optimizing outcomes in CF. Despite several, albeit methodo-
logically flawed studies there is still no evidence to suggest that
one airway clearance technique has superiority over any other. The
only clear conclusion is that there may be some short-term benefit
to airway clearance compared to no airway clearance.29,30 More
recently emphasis has shifted to determine ways of optimising the
application of techniques. There is universal agreement that ‘a one
size fits all’ approach to airway clearance is no longer justifiable.
Similarly one technique will not be suitable throughout the
lifespan from the newborn-screened infant through to the
transitional teenager. Each person with CF requires an individual
approach to their airway clearance regimen, which will require
regular review in order to optimise its efficacy (Figure 1). Each
regimen can and should be adapted to suit the individual and each
patient (once old enough) should be taught how to adapt his/her
regimen within a single treatment session to achieve the most
effective result.31
During times of respiratory exacerbation and admissions ACTs
should be reviewed and optimised to ensure the focus of clearance
meets the need of the patient at that particular stage in their
disease. The potential for introducing a short-term alternative to
the normal routine should be considered. Non-invasive ventilation
(NIV), intrapulmonary percussive ventilation (IPV) or intermittent
positive pressure breathing (IPPB) are all potential additional
therapies during acute exacerbations.
Non-invasive ventilation
The use of NIV has become increasingly accepted as an option
for individuals who have severe CF lung disease with respiratory
failure, nocturnal hypoventilation or as a bridge to lung
transplantation.32,33 NIV is believed to unload the respiratory
muscles and improve gas exchange during sleep.34 NIV may also
have beneficial effects during exercise and airway clearance
therapy but there is limited evidence to support these claims. A
recent retrospective review suggested that NIV may slow or
reverse the decline in lung function in adults with advanced CF
lung disease with the greatest improvement seen in the first year
but was reported to extend to 3 years of follow-up.35
NIV may be useful as an adjunct to airway clearance therapy but
there is a lack of robust evidence to support this claim.36 A
reduction in fatigue, dyspnoea and increased muscle strength and
oxygenation have been reported.36 There remains however limited
research to suggest that NIV enhances sputum clearance alone.33
NIV can be used in combination with other ACTs such as active
cycle of breathing techniques (ACBT) or autogenic drainage (AD)
and in combination with inhalation therapy to aid deposition of
nebulised medications (Figure 1).
Clinicians should consider early introduction of NIV as part of
the airway clearance programme in order to familiarize the patient
with the machine with a view that long-term use may be required
in the future. Selection of an appropriate interface is imperative
and should be chosen according to individual assessment as the
interface represents a crucial determinant of the success of NIV
therapy.37
Intrapulmonary percussive ventilation
IPV is increasingly being used in CF to optimise airway
clearance in the acute setting. IPV has the benefit of working
several different physiological principles by combining internal
thoracic percussion and inspiratory pressure through rapid mini
bursts of air superimposed on a spontaneous breathing pattern. It
is proposed that the percussive oscillatory vibrations enhance
movement of retained secretions, high density aerosol delivery
hydrates viscous mucous and the PEP feature recruits alveolar lung
units, prevents airway closure and assists in expiratory flow
acceleration.38 A number of studies have compared IPV to other
chest physiotherapy techniques (conventional physiotherapy,
flutter and HFCWO) in CF and have reported equal efficacy.38–40
Mucociliary clearance may be optimised by combining IPV with
the inhalation of HTS and/or using it in conjunction with other
ACTs such as AD and ACBT. This combination may be particularly
useful in those patients with small airways disease, airways hyper-
reactivity and/or thick tenacious secretions.23 IPV may also be
considered for the breathless fatigued patient either during a
respiratory exacerbation or in end stage disease, when other
airway clearance options have become ineffective. IPV is currently
predominantly used as an inpatient modality due to cost and
portability however, with the recent introduction of the Nippy
Clearway (B&D Electromedical), which also has the benefit of an
oscillatory function IPV therapy may soon be an option to consider
in the home environment.
Intermittent positive pressure breathing
IPPB is another consideration to help optimise treatment
efficacy in the inpatient setting. Although there is little evidence
for its use in CF, experienced clinicians suggest that it may be
useful for patients who are fatigued or dyspnoeic due to an
inability to clear secretions effectively,23 often during an acute
exacerbation in severe disease. As with IPV and NIV, IPPB can be
combined with inhalation therapy or used in conjunction with
other airway clearance techniques such as AD and ACBT. An OPEP
device may also be added onto the expiratory port in the circuit, if
an oscillatory component is thought to be useful to the patient.
Adherence with airway clearance techniques
No single ACT is suitable for all patients and the therapist
should be well educated in all aspects of airway clearance and
associated therapies. Several factors including gender, age and
disease progression may influence the degree to which an
individual adheres to any treatment regimen. Adherence with
ACT is variable but often reported as low particularly in those with
chronic respiratory disease such as CF.41 Adherence may be
improved if alternative techniques are available, a gradually
developed treatment package is provided and patients are given
information, time and assistance to process the information and
organise their everyday life.20 Patient satisfaction and perceived
efficacy are reported to be intimately related to adherence to a
technique. In infants ACTs are performed by the parents/carers but
as children grow they should be taught techniques, which can be
performed independently, though some degree of supervision may
still be required. As CF children get older, they can also begin to
play a more active role in their treatment and a take part in
decisions regarding treatment techniques, which may help to
facilitate better adherence. In order to optimise the effectiveness of
airway clearance in CF it is essential to maximise adherence, and
 S. Rand et al. / Paediatric Respiratory Reviews 14 (2013) 263–269 267

Breathing Oscillatory
PEP techniques/CPT HFCWO IPPB NIV IPV Exercise
PEP
Combine with Combine with Combine with Combine with Combine with
Combine with Combine with Combine with
inhalaon inhalaon inhalaon inhalaon inhalaon
inhalaon inhalaon breathing
therapy +/- therapy +/- therapy +/- therapy +/- therapy+/-
therapy therapy techniques, NIV
intersperse intersperse intersperse intersperse intersperse

Combine with
Combine with Combine with Combine with Combine with
Combine with breathing
breathing PEP, OPEP, IPPB, breathing Add OPEP device breathing
PEP, OPEP techniques,
techniques NIV, IPV, HFCWO techniques techniques
exercise

Figure 1. Suggestions for optimising airway clearance techniques.

PEP – positive expiratory pressure, CPT – conventional physiotherapy, OPEP – oscillating PEP, HFCWO – High frequency chest wall oscillation, IPPB – intermittent positive
pressure breathing, NIV – Non-invasive ventilation, IPV – intrapulmonary percussive ventilation.

adherence seems to correlate best with patient satisfaction
regarding the technique.42 Regular review during stable periods
of the disease as well as during exacerbations is essential to ensure
the airway clearance regimen meets the specific needs of the
individual at that time.
MANAGEMENT OF NEWBORN SCREENED INFANTS
Newborn screening (NBS) for CF is widely available in many
countries and infants are now diagnosed with CF in the first 2
months of life, often before they have developed any overt
symptoms.20 As a result a new cohort of CF patients is emerging.
The physiotherapy approach to these so called ‘asymptomatic’
infants varies both nationally and internationally. The current UK
consensus guidelines outline the approach to this cohort of
patients.43 However it is well recognised that even in the UK
practice varies from centre to centre. The focus of ‘treatment’ for
these ‘asymptomatic’ infants is aimed at preservation of lung
health, nutritional status and promotion of normal development.
From a physiotherapy perspective this involves the establishment
of a comprehensive physiotherapy regimen of physical activity/
exercise and ACTs. NBS provides a window of opportunity soon
after diagnosis for early education of parents/carers and promotion
of a lifestyle involving physical activity and exercise.
A more ‘dynamic’ treatment approach to the physiotherapy
management of NBS infants has been adopted in many centres.43 In
some centres the emphasis on routine daily specific airway
clearance has shifted to a daily structured physical activity and
exercise regimen with the addition of an airway clearance
component when required. This remains a contentious issue as
some clinicians believe that a specific airway clearance regimen
should be commenced at diagnosis and continued on a daily basis
regardless of clinical status, in order to attempt to compensate for
the impaired mucociliary clearance and to prevent or at least delay
the ensuing process of inflammation.44,45 ACTs currently used in
infants are poorly evidence based and in general have been
adapted from techniques developed for older patients with chronic
sputum retention.20 The main types of ACTs which can be used for
young infants with CF are infant PEP, modified postural drainage46
with percussion and assisted autogenic drainage (AAD) with or
without bouncing on a gym ball.47
In the toddler years blowing games, bubbles and bubble PEP
with more structured forced expiratory techniques (FETs) can be
introduced. Patient preference is an important aspect of ACT
management and although young infants are unable to voice their
preference for one treatment over another it is important that
parents and carers are given options to adapt treatments to fit into
their lifestyles. As children become older and are able to make their
own decisions it is imperative that they are made to feel part of the
decision making process.
Physical activity, including exercise is now emerging as perhaps
the most important aspect of care of in CF. Physical activities in
infants aim to alter ventilation distribution, alter breathing patterns,
increase expiratory flow and elicit shear forces to enhance
mucociliary clearance. Many CF centres now advise a daily
structured exercise programme from the day of diagnosis. Swim-
ming, infant massage, baby gym and music classes are all extremely
beneficial. Physical activity can also be used in combination with
ACTs e.g. infant PEP can be used in combination with bouncing on a
gym-ball. The aim of physical activity education should be to
reinforce a lifestyle involving fitness and exercise from an early age.
The emphasis and importance of active parental role models should
be discussed with NBS parents from diagnosis. On-going parental
education and facilitation is also an important component of the
physiotherapy management of these babies.
PHYSICAL ACTIVITY AND EXERCISE
The importance of exercise in maintaining a healthy lifestyle is
well recognised in both health and disease.23 Exercise is now an
essential component of the management of CF and is seen as
‘medicine’ for CF (Table 2).48 The current guidelines for physical
activity for healthy children and adults are applicable in CF and can
be used as a basis for exercise advice until respiratory disease
progresses.49 Physical activity refers to any body movement
produced by skeletal muscle and occurs in a variety of forms such
as free play, exercise and organised sports.49 Exercise training,
however can be defined as regular participation in vigorous
physical activity to improve physical performance, cardiovascular
function, muscle strength or a combination of all three.49
It is well recognised that even in health the recommended
physical activity guidelines are often not met, with an estimated 3
out of 10 boys and 4 out of 10 girls not undertaking the
recommended 60 minutes of daily moderate to vigorous intensity
exercise.50,51 The total level of habitual activity in individuals with
CF has been reported to be comparable to that of their peers but the
Table 2
Benefits of exercise in cystic fibrosis.
Benefits of exercise in cystic fibrosis
Improve physical functiona
Improve cardiovascular performancea
Improve muscle strengtha
Airflow effects (generation of peak expiratory flow bias and shear forces) to
facilitate mucociliary clearanceb
Improve fluid balance and retention of serum electrolytesc
Potential to improve bone mineral densityd
Improve quality of lifee
Potential to slow the rate of lung function declinef
Potential positive effects on CF related diabetes
a
Bradley and Moran, 2002.
b
Dwyer et al., 2011.
c
Schmitt et al., 2011.
d
Frangiolas et al., 2003.
e
Orenstein 1989.
f
Nixon et al., 1992.
268 S. Rand et al. / Paediatric Respiratory Reviews 14 (2013) 263–269
type of activity was less vigorous in nature.52,53 It is also well known
that in disease states a negative feedback loop can be created where
reduced exercise capacity levels lead to reduced habitual activity
levels thereby further reducing exercise capacity.54 There is
therefore an important role for physiotherapists to optimise the
frequency, intensity and type of physical activity and exercise
undertaken by individuals with CF. It is essential that clinicians
promote and develop new models of care, which aim to facilitate
participation in exercise rather than just offer verbal advice.
Parent’s perception of their child’s physical activity competency
have been shown to be related to the child’s actual participation in
activity55 so it is essential that children engage in moderate to
vigorous activities from an early age in order to promote life-long
participation in effective physical activity and exercise. Parents of
children with CF frequently express concerns about their child’s
ability to participate in physical activity or sports. Teachers and
sports coaches are also often reluctant to allow participation in
vigorous sports in particular.53 Education of parents, school-
teachers and sports coaches is an important part of the role of
physiotherapists.
Exercise Testing
Exercise testing is underutilised in the CF population56 and as a
result individualised and tailored exercise prescription remains
inadequate despite the ever-increasing evidence base to support
its use. In order to optimise the benefits of exercise, the
management of individuals with CF should include assessment
of fitness, education regarding benefits of exercise, exercise
prescription/advice, monitoring of adherence to exercise, and
where appropriate introduction of strategies to maintain or
improve exercise levels regardless of disease severity.
There is some evidence to suggest that there is an association
between aerobic capacity and survival in CF.57,58 Regular exercise
also has the potential both in the short and long term to slow the
annual rate of decline in lung function.59–62 An on-going
programme of education, regular exercise testing (to guide
exercise prescription) and practical facilitation of participation
in regular activity are all important factors in ensuring that the
benefits of exercise are achieved.
CONCLUSION
There has been a dramatic change in the physiotherapy
management of individuals with CF over the past two decades.
This change is particularly evident in the paediatric CF population
due in part to the introduction of NBS. The introduction of NBS has
also provided new challenges as the management of ‘asympto-
matic’ infants requires a more ‘dynamic’ treatment approach
compared to traditional physiotherapy methods. CF children are
now generally fitter and healthier. Physiotherapists need to have a
broad understanding of the wide variety of treatment options now
available. Physiotherapists also require the knowledge and ability
to adapt and change treatments according to individual needs,
taking in to consideration factors such age, the stage of disease and
social circumstances. Attention to detail, regular review and
optimisation of treatment type and techniques to meet individual
requirements is imperative if one is to achieve the best outcome
from physiotherapy intervention in CF.
FUTURE RESEARCH DIRECTIONS
 Long term effects of physical activity and exercise on individuals
with CF.
 Airway clearance techniques and interventions for the newborn
with CF.
 Methods to improve adherence to physiotherapy treatments in
CF.
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