Monaldi Archives for Chest Disease 2021; volume 91:1501

Pulmonary rehabilitation for cystic fibrosis:

A narrative review of current literature
Evgenia I. Kalamara1, Evangelos T. Ballas2, Georgia Pitsiou1,3, Guergana Petrova2,4
1Respiratory Failure Unit, General Hospital of Thessaloniki “G. Papanikolaou”, Thessaloniki, Greece; 2Medical
University Sofia, Bulgaria; 3Medical school Aristotle university of Thessaloniki; 4Pediatric Clinic, University Hospital
Alexandrovska, Sofia, Bulgaria

overall lung health is growing. In cystic fibrosis, multiple factors

Abstract result in reduced exercise capacity. All modalities of pulmonary
rehabilitation should be offered to patients with cystic fibrosis, as
Pulmonary rehabilitation is a key component in cystic fibrosis the benefits in most cases outweigh the risks, though the optimal
care. This review summarizes the recent evidence in the area of regimens need to be yet defined.
pulmonary rehabilitation for cystic fibrosis in the form of

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questions and answers regarding interventions, indications,
benefits and risks of pulmonary rehabilitation. Pulmonary
Introduction

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rehabilitation includes airway clearance techniques, exercise
training, education and behaviour change and can improve
patients’ exercise capacity, muscle strength, quality of life and Cystic fibrosis (CF) is an autosomal recessive disease and one

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nutritional status. Airway clearance techniques have beneficial of the most common life-shortening condition affecting
approximately 70,000 individuals worldwide [1], caused by
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effects for clearing mucous. Over the past years, evidence for the
mutations in the cystic fibrosis transmembrane conductance
beneficial effects of exercise training on exercise capacity and
regulator (CFTR) gene. The CFTR protein is expressed in epithelial
cells and has several functions, primarily serving as an ion channel.
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It regulates liquid volume on epithelial surfaces through chloride

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secretion and inhibition of sodium absorption.

Correspondence: Evgenia I. Kalamara, Pavlou Mela 3, Chortiatis, Airway disease is hypothesized to be mainly a result of
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57010 Thessaloniki, Greece. reduced airway surface liquid volume which subsequently leads
Tel. +30.6948107468. E-mail: [email protected] to mucociliary dysfunction, increase of bacterial load, and chronic
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inflammation. The abnormal composition and secretion of mucus

Key words: Pulmonary rehabilitation; cystic fibrosis; exercise capaci-
affects multiple organs and causes various complications, thus
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ty; lung function; quality of life; physiotherapy; airway clearance;

exercise training. rendering CF a systemic disease. Therefore, CF management
requires a multidisciplinary approach, preferably in the setting of
a specialized center. The treatment should be individualized to
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Conflict of interest: The authors declare that they have no competing

interests, and all authors confirm accuracy. the needs of each patient, and should aim at the management of
airway infection and the development of an active lifestyle, in
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Contributions: All the authors made a substantive intellectual contri- order to maintain good nutrition and exercise capacity [2].
bution, have read and approved the final version of the manuscript and
Pulmonary rehabilitation is an intervention customized to
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agreed to be accountable for all aspects of the work.

each patient and includes airway clearance techniques, exercise
Ethics approval: Not applicable. training, education and behaviour change. Its target is the
amelioration of the physical and psychological status of people
Availability of data and materials: The data used to support the find- with chronic respiratory disease and the promotion of long-term
ings of this study are available from the corresponding author upon adoption of health- improving behaviour. It is acknowledged as
request. an important adjunction to the treatment of many chronic
respiratory diseases [3]. In patients with CF, pulmonary
Received for publication: 11 July 2020.
Accepted for publication: 22 December 2020.
rehabilitation traditionally focused on airway clearance
techniques. Nowadays the management also extends to other
©Copyright: the Author(s), 2021 areas. Systematic exercise training and personalized rehabilitation
Licensee PAGEPress, Italy programs are offered to CF patients [4], while new technologies
Monaldi Archives for Chest Disease 2021; 91:1501 are being gradually incorporated to the rehabilitation process
doi: 10.4081/monaldi.2021.1501 during the past few years.
This review was conducted in order to map the recent research
This article is distributed under the terms of the Creative Commons
done in the area of pulmonary rehabilitation for cystic fibrosis and
Attribution Noncommercial License (by-nc 4.0) which permits any
noncommercial use, distribution, and reproduction in any medium, answer questions about its main aspects, as an aid for respiratory
provided the original author(s) and source are credited. medicine physicians, general practitioners, physiotherapists or other
clinicians involved in the care of CF patients.

[Monaldi Archives for Chest Disease 2021; 91:1501] [page 139]

Review

and/or strength. Patients should be able to continue effective training

Methods once supervised sessions have ended and regular physical activity
five times a week for 30 min each time is encouraged regardless of
This literature review took place between March 2019 and rehabilitation, as healthy living advice [10,11].
September 2020. Our protocol was drafted using the Preferred Notably, the American College of Sports Medicine recommend
Reporting Items for Systematic Reviews and Meta-analysis moderate-intensity aerobic (endurance) physical activity for a
Protocols, with appropriate modifications for the purposes of a minimum of 30 min on five days each week or vigorous-intensity
narrative review [5]. For this review all relevant information was aerobic physical activity for a minimum of 20 min on three days
identified using Embase, Cochrane Library, Science Direct, Scopus, each week in order to promote and maintain health in adults [12].
PubMed databases and Google Scholar. As far as home-based programs are concerned, several protocols
A literature search was performed. Search words were cystic have been used, based on aerobic and muscle strength training.
fibrosis, pulmonary rehabilitation, exercise capacity, lung function, Happ et al. utilized an at-home bicycle exercise regimen performed
quality of life, physiotherapy, airway clearance, exercise training. three times a week, consisting of two exercise routines of different
Relevant peer-reviewed journal papers were included if they were intensity performed in an alternating sequence during each session.
published between the period of 2000 to 2020 and written in Schneiderman-Walker et al. also used a three times weekly program
English. The papers could include subjects of various ages (children, that required a minimum of 20 min of aerobic exercise at a heart
adolescents, adults) and severity of disease. Papers were excluded rate of approximately 150 beats/min. While there is still no
if they did not fit into the conceptual framework of the study, for standardization regarding self-regulated, home-based exercise,
example papers focused on chronic respiratory conditions other than further evaluation is needed as such interventions are fairly easily
cystic fibrosis. conducted and low-cost [13-15].
The selected literature included 23 Randomized control trials, Kriemler et al. compared the effects of 6 months training

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8 Cochrane Systematic Reviews, 7 guidelines, 7 clinical studies, 12 (aerobic or anaerobic) versus no training and found similar
reviews, 1 systematic review, 2 other original articles. improvements in FEV1, FVC and exercise capacity. However,

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anaerobic training lowered static hyperinflation, while aerobic
training did not. The authors assumed that improvements in FVC
with strength training might be because of a decrease in

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What interventions does pulmonary rehabilitation hyperinflation in this group [16] .Based on these facts, aerobic and
include?
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anaerobic training can be regarded as similarly effective.
A randomized control trial by Selvadurai et al. compared
Airway clearance techniques aerobic and resistance training in children with cystic fibrosis (CF)
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An integral part of pulmonary rehabilitation is the application admitted to hospital with an intercurrent pulmonary infection with
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of airway clearance techniques, that are used to enhance the a control group. The study demonstrated that children who received
mucociliary clearance system, in order to transport secretions aerobic training had significantly better peak aerobic capacity,
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proximally up the airways [6]. activity levels, and quality of life than children who received the
These include conventional chest physiotherapy (mainly resistance training program. Children who received resistance
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involves postural drainage, percussion and vibration), active cycle training had better weight gain (total mass, as well as fat-free mass),
of breathing techniques (ACBT), which consist of breathing control lung function, and leg strength than children who received aerobic
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exercises, thoracic expansion exercises and forced expirations, training. Therefore it is implied that a combination of aerobic and
positive expiratory pressure (PEP) therapy, which is defined as anaerobic training may be the optimal training modality for CF
breathing against a positive expiratory pressure using a mask or patients [17].
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mouthpiece, autogenic drainage (three-level breathing sequence It is noteworthy that not all training modalities are suitable for
beginning at low lung volumes, followed by breathing at mid-lung all patients, as in the case of severe pulmonary impairment. Gruber
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volumes, followed by deep breathing, huff and coughing- it uses et al. used an individualized training program with supplemental
controlled breathing to achieve the highest possible airflow in oxygen over 6 weeks in patients with severe disease and found
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different bronchi generations), mechanical percussion, high- comparable improvements in exercise capacity with conventional
frequency chest compression and non-invasive ventilation (NIV). training in patients with less severe disease [18]. This study is in
Notably, PEP therapy can be oscillating or non oscillating. favor of individualized training interventions.
Oscillating PEP combines oscillation of airflow with PEP in order Concerning the intensity of training, most studies that managed
to loosen secretions [7,8]. Aerobic exercise can also be considered to improve exercise capacity used intensities at about 50% VO2 max
as an airway clearance technique, as it reduces mechanical or 60-85% of maximum heart rate [16,19-21]. A study of Whitley
impedance of sputum and enhances expiratory flow rates [9]. et al assessed the immediate effects of different exercise intensities
on pulmonary function and diffusion capacity. Moderate exercise
Exercise training improved diffusion, while vigorous exercise caused airflow
In pulmonary rehabilitation, out-patient, in-patient and home- restrictions [22]. Taking all these into account, the optimal exercise
based programs can be utilized. Exercise is a basic component of training protocol for CF patients is yet to be defined.
rehabilitation programs, and it includes lower and upper extremity
training, inspiratory muscle training, as well as chest physical
Education, behavioural change promotion
therapy techniques. Usually, the proposed schedule consists of a and adjunctive therapies
minimum of two sessions per week, preferably three or more, for In addition to these, in pulmonary rehabilitation programs
4, 6, 9 or 12 weeks (minimum of 12 supervised sessions psychosocial assessment and intervention are offered, as depression
recommended). The duration varies from 30 to 60 min and exercise and anxiety are common in patients with chronic lung disease.
can be continuous or intermittent and training can target endurance Evaluation of disability and education of both patient and family

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are part of this type of intervention [11]. Moreover, nutritional myocardial infarction, severe pulmonary hypertension, unstable
interventions are appropriate, considering the high probability of diabetes, severe exercise-induced hypoxemia, abdominal aortic
malnourishment and decreased muscle mass among patients with aneurysm >5.5 cm deemed inoperable, severe locomotor
chronic respiratory conditions [11]. Adjunctive therapies, such as impairment, severe peripheral vascular disease. Stable
bronchodilators, oxygen therapy, non-invasive ventilation (NIV) cardiovascular disease is not a contraindication. Active cigarette
and neuromuscular stimulation are also of considerable help during smoking is, in certain cases, considered as a relative
a rehabilitation program. Last but not least, patients referred to contraindication, although patients with COPD should be referred
pulmonary rehabilitation should be assessed about their smoking regardless of their smoking status [10,11].
status and smoking cessation services should be available [11]. As far as CF patients are concerned, pulmonary rehabilitation
is a key element of care. Airway clearance techniques are frequently
Integration of new technologies in pulmonary described as a cornerstone of CF treatment and should be performed
rehabilitation programs across the lifespan. Exercise is also recommended, and patients
It is noteworthy that for the last couple of years, there has been should be offered an individualized program, according to their
special interest in new technologies, including video games, social capability and preferences. Regular physical activity should be
media and web-based platforms and the potential for their encouraged and should include weightbearing exercise in order to
application in the rehabilitation process. optimize bone density, while strength training programs should be
In the CF population, a randomized controlled trial used the prescribed to optimize muscle mass. Nutritional and psychological
Nintendo Wii platform to deliver a 6-week home training program, interventions are also acknowledged as an integral part of the
with participants followed up for 12 months after the intervention. standard of care in CF [7,33].
Exercise capacity, muscular strength and quality of life were Even in complex CF cases, physiotherapy management is
improved in the short-term. The effects of training on muscle advised to be continued, with proper alterations and modifications,

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performance and quality of life were sustained over 12 months. rather than discontinued, when possible. For instance, in case of
hemoptysis the regimens are altered to minimize the risk of re-

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However, adherence was 95% at 6 weeks but substantially reduced
in the long term, with 65% of the subjects not using the active video bleeding and in case of pneumothorax physiotherapy is continued
game at all at 12 months [23]. Bishay et al. compared a fitness tracker when feasible but, minimizing the amount of positive pressure
generated inside the patient lungs [34]. The American Diabetes

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with a personalized exercise prescription and social media platform
to exercise prescription alone, although no significant difference in Association Clinical Care Guidelines for CF-related diabetes
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patient outcomes was noted between the two groups [24]. recommend that patients should perform moderate aerobic exercise
An RCT by Salonini et al. involving children and adolescents for at least 150 min per week, but monitoring of blood glucose
with CF compared a traditional stationary cycle training intervention levels before activity, consumption of extra carbohydrates or
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to a training intervention using the interactive Xbox Kinect alterations of insulin dosage may be required [35].
Pregnant women with CF are encouraged to maintain a regular
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platform. Heart rate, SpO2, dyspnea, fatigue and subject satisfaction

were evaluated. The study concluded that training with Xbox Kinect airway clearance routine, though postural drainage should be
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provided a cardiovascular demand similar to a stationary cycle, avoided. Exercise programs should be appropriate for the
while it caused less dyspnea and fatigue and was more enjoyable cardiorespiratory and musculoskeletal changes during pregnancy,
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than the stationary cycle. Therefore, such a modality has the and adequate hydration during exercise should be maintained [36].
potential to be used as an exercise intervention in young patients
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with CF. Similarly, Holmes et al. reported that training using the
Xbox Kinect may be a suitable alternative to conventional exercise
modalities for adults with CF [25,26]. O’Donovan et al. concluded What are the benefits of pulmonary rehabilitation
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that active video games are a useful source of light to moderate in general?
intensity physical activity in children with cystic fibrosis [27]. There
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are also other examples of studies including social media or web- Pulmonary rehabilitation is known to improve exercise capacity,
based platforms as an aid to exercise [28,29] and several relative muscle strength, dyspnea and health status compared with usual
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studies that are on-going [30,31]. care. Self-reported measures of activities of daily living (ADL) and
These new modalities have potentially beneficial effects on psychological status also improve. In addition to these, pulmonary
fitness and training adherence, however further research is needed rehabilitation modestly ameliorates physical activity levels and body
to study the long-term effects of such interventions [32]. weight/nutritional status [11].

What are the indications and contraindications What are the reasons for reduced exercise capacity
of pulmonary rehabilitation? in patients with cystic fibrosis?

Presence of long-term debilitating symptoms, dyspnea Medical Thick bronchial secretions, altered respiratory mechanics and
Research Council (MRC) score of more than 2, motivation of gas exchange and decreased lung function are hallmark features of
patient to follow the rehabilitation program, rehabilitation prior to the disease. Severe airflow limitation and dynamic hyperinflation
volume reduction surgery or lung transplantation are some of the results in increased dead space ventilation and exertional dyspnea.
criteria for patient selection for pulmonary rehabilitation in general. In certain patients, concomitant cardiovascular abnormalities may
However, rehabilitation is not usually appropriate when the patient play a role. Another important factor in the impairment to exercise
is considered unable to follow the proposed program or when is the involvement of peripheral muscles. Defective muscle
serious comorbidities are present, such as angina pectoris, recent metabolism, malnutrition, electrolyte disturbances, physical

[Monaldi Archives for Chest Disease 2021; 91:1501] [page 141]

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inactivity, systemic inflammation and oxidative stress, oral and strength training ameliorates exercise capacity, lung function
corticosteroid use and CF-specific gene defects can be implicated and HRQL [19]. Another RCT by Klijn et al. has shown that
in the peripheral muscle dysfunction. In certain cases, diaphragm anaerobic training has a beneficial effect on aerobic and anaerobic
strength is decreased despite a normal muscle mass, which can be performance, as well as on quality of life [50].
possibly attributed to functional changes due to hyperinflation [37]. Urquhart et al. reported that supervised, outpatient exercise and
physiotherapy are associated with improvements in QOL and
exercise tolerance, a reduction in intravenous antibiotic days, and a
trend towards reducing lung function decline in children with CF
What are the benefits of airway clearance techniques [58]. Perez et al. detected that VO2 peak was the only variable
in CF patients? significantly associated with time to hospitalization, therefore
greater aerobic fitness is associated with a lower risk of
Concerning airway clearance, bibliography clearly recommends hospitalization [59].
its application when compared to no airway clearance or cough An RCT by Schindel et al. studied the effect of exercise training
alone [11,38-40]. A 2015 Cochrane review found a significant on posture, and resulted that after 3 months the intervention group
increase in the amount of sputum expectorated in the patient groups showed a decrease in cervical and lumbar lordosis, thoracic
that applied airway clearance compared to spontaneous cough or kyphosis, lateral chest distance and abdominal protrusion [60].
not using any airway clearance technique, concluding that methods Rovedder et al. reported increased upper limb strength over a 3-
of clearing the airways have short-term benefits for moving mucous month combined aerobic and strength training program [14].
[39]. Most studies do not show significant differences in the lung
function of CF patients after chest physiotherapy [8,39,41-43].
Evidence from the Cochrane systematic reviews support that no one
Are there risks related to exercise?

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airway clearance regimen is better than another [8,31].
And if so, do the benefits outweigh the risks

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in the CF population?
What are the benefits of exercise training in CF

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A patient may experience dyspnea, productive cough and
patients? us
fatigue during exercise. Moreover, some patients with
bronchiectasis or asthma may suffer exercise-induced
Evidence suggests that CF patients with better physical fitness bronchoconstriction. Exercise-induced hypoxemia (drop in oxygen
have better quality of life [7]. Exercise programs can improve
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saturation by more than 4 or below 90%) may also be observed,
fitness, exercise capacity, thoracic mobility, quality of life, maintain especially in patients with advanced lung disease. An estimated 20-
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bone mineral density and lower the rate of decline in pulmonary 25% of all patients with CF may present exercise-induced
function [13,19,44-51]. Moreover, a training effect, as measured by hypoxemia [36]. Patients with exercise-induced hypoxemia, as well
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a decrease in lactate levels and heart rate can be achieved [52]. Both as patients treated with macrolides are also at higher risk of
aerobic or anaerobic physical training has a positive effect on developing cardiac arrhythmias during exercise. In general,
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primary outcomes in CF patients (exercise capacity, strength and approximately 5-10% of all patients present cardiac arrhythmias
lung function) [53]. with exercise. Furthermore, exercise can potentially trigger a
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pneumothorax or an episode of hemoptysis, although the evidence

Pulmonary function and exercise capacity is scarce. Musculoskeletal injuries are always possible with
A prospective, longitudinal study by Schneiderman et al. that exercise, just as in the healthy population [61].
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included 212 patients with CF recruited over a 9-year period, As far as CF patients are concerned, electrolyte losses, injury
concluded that patients with CF with increasing activity levels had
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to spleen or oesophageal hemorrhage in the presence of portal

a reduced rate of FEV1 decline compared to those who did not hypertension, hypoglycemia in the setting of CF-related diabetes
become more active over the study period [54]. Kriemler et al. and mellitus and worsening of preexisting arthritis are potential risks
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Hebestreit et al. both studied the effects of physical training over a during exercise. In addition to these, when pulmonary rehabilitation
6-month period and found improved exercise capacity and lung programs are organized, special attention should be given to hygiene
function in the intervention group compared to the control group aspects to avoid cross-infection.
[16,55]. Santana-Sosa et al. reported amelioration of muscle The number of different complications described may be
strength and VO2 peak after an 8-week exercise program [20,21]. A unsettling, however the frequency of true adverse events is small
retrospective cohort study of 2014 concluded that regular exercise and these mainly appear in patients with advanced disease.
is associated with a reduced decline in FEV1 and body mass index Individual assessment of each patient is always advised, but in most
(BMI) in adults with CF. It is noteworthy that exercise capacity cases the benefits of exercise clearly outweigh the potential harms
seems to be linked to the prognosis of CF patients. VO2 peak, peak [61-63].
work rate, ventilatory equivalent for oxygen and carbon dioxide
have been found as predictors of death or lung transplantation at 10-
year follow-up [56].
Discussion
Health-related quality of life, need for hospitalization,
body posture and strength Pulmonary rehabilitation is a key component in CF care.
VO2 peak correlates with HRQL in CF patients and when Traditionally, airway clearance techniques are considered a
improved it is associated with better nutritional status and health cornerstone of CF treatment and should be performed across the
perception [57]. Hebestreit et al. concluded that a combined aerobic lifespan in CF [7]. Evidence supports that airway clearance

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 Review

regimens have beneficial effects for moving mucous and are term effects of such interventions, however new means offer new
similarly effective [8,39]. possibilities for CF care. For instance, tele-rehab could be evolved
Regular exercise training is increasingly being recommended and become a safe standard of care, especially during the COVID-
for CF patients. Further research is necessary to accurately assess 19 era.
the benefits of exercise training in the CF population. Moreover,
there is a lack of studies investigating the effects of physical exercise
training on other significant outcomes, such as bone health, diabetic
control and pulmonary exacerbations. However, evidence for the References
beneficial effects of exercise training on lung health and exercise
capacity is growing. Exercise improves aerobic capacity and 1. Hollin IL, Robinson KA. A Scoping review of healthcare costs
probably lowers the rate of decline in pulmonary function. Exercise for patients with cystic fibrosis. Appl Health Econ Health
capacity is also correlated with the prognosis of CF patients [56]. Policy 2016;14:151-9.
Thus, there is no reason to discourage the integration of exercise in 2. Davies JC, Alton EW, Bush A. Cystic fibrosis. BMJ
all CF patients care [32]. This is also supported by evidence that 2007;335:1255-9.
show a relatively small frequency of true adverse events of exercise. 3. Spruit MA. Pulmonary rehabilitation. Eur Respir Rev
Personalized assessment of patients is needed, but in most cases the 2014;23:55-63.
benefits of exercise outweigh the potential harms [61,64]. 4. Spinou A. Physiotherapy in cystic fibrosis Α comprehensive
The optimal training components (type, frequency, duration, clinical overview. Pneumon 2018;31:35-43.5.
intensity) need to be determined. Taking into account the existing 5. PRISMA. Transparent reporting of systematic reviews and
literature, exercise training programs need to be tailored to the meta-analyses.
patient. For non-complex cases, perhaps a program of moderate- 6. McIlwaine M, Bradley J, Elborn JS, Moran F. Personalising

ly
intensity exercise, 5 times per week, for 30 min per session is a airway clearance in chronic lung disease. Eur Respir Rev
reasonable regimen [12,19-22,65]. There is an increasing interest in 2017;26:160086. Available from: http://www.prisma-state-

on
the integration of new technologies in the pulmonary rehabilitation ment.org/
process (video game activities, social media and web-based 7. Button BM, Wilson C, Dentice R, et al. Physiotherapy for cys-
platforms). These new modalities have potentially beneficial effects tic fibrosis in Australia and New Zealand: A clinical practice

e
on fitness and training adherence, however further research is guideline. Respirology 2016;21:656-67.
needed to study the long-term effects of such interventions [32]. us
8. Bradley JM, Moran FM, Elborn JS. Evidence for physical ther-
apies (airway clearance and physical training) in cystic fibro-
sis: an overview of five Cochrane systematic reviews. Respir
al
Med 2006;100:191-201
Limitations 9. Dwyer TJ, Alison JA, McKeough ZJ, et al. Effects of exercise
ci

on respiratory flow and sputum properties in patients with cys-

er

This article is a narrative review, therefore it represents a broad tic fibrosis. Chest 2011;139:870-7.
overview of a topic-related research area. Data extraction relies on 10. Sharma BB, Singh V. Pulmonary rehabilitation: An overview.
m

description of study findings rather than on continuous or Lung India 2011;28:276-84.

categorical statistical values. 11. Bolton CE, Bevan-Smith EF, Blakey JD, et al. British Thoracic
om

Society guideline on pulmonary rehabilitation in adults.

Thorax 2013;68:ii1-30.
12. Haskell WL, Lee IM, Pate RR, et al. Physical activity and pub-
Conclusions
-c

lic health: updated recommendation for adults from the

American College of Sports Medicine and the American Heart
on

Pulmonary rehabilitation is a multidisciplinary, individualized Association. Med Sci Sports Exerc 2007;39:1423-34.
intervention that incorporates exercise training, airway clearance, 13. Schneiderman-Walker J, Pollock SL, Corey M, et al. A ran-
education and behaviour change and a key component in CF domized controlled trial of a 3-year home exercise program in
N

patients care, as it can improve exercise capacity, muscle strength, cystic fibrosis. J Pediatr 2000;136:304-10.
quality of life and nutritional status of patients. 14. Rovedder PM, Flores J, Ziegler B, et al. Exercise programme
Both airway clearance techniques and exercise training are in patients with cystic fibrosis: a randomized controlled trial.
invaluable parts of pulmonary rehabilitation. Airway clearance Respir Med 2014;108:1134-40.
regimens have beneficial effects for moving mucous, and they seem 15. Happ MB, Hoffman LA, Higgins LW, et al. Parent and child
to be similarly effective. On the other hand, exercise improves aerobic perceptions of a self-regulated, home-based exercise program
capacity (commonly affected in CF due to multifactorial pathology) for children with cystic fibrosis. Nurs Res 2013;62:305-14.
and probably lowers the rate of decline in pulmonary function in CF Erratum in: Nurs Res 2014;63:25. DiVirgilio, Dana [corrected
patients. The optimal training components (type, frequency, duration, to Divirgilio, Dana].
intensity) need to be determined, but it seems that a combination of 16. Kriemler S, Kieser S, Junge S, et al. Effect of supervised train-
aerobic and anaerobic training may be the optimal training modality ing on FEV1 in cystic fibrosis: a randomised controlled trial. J
for CF patients. When planning a training program, personalized Cyst Fibros 2013;12:714-20.
assessment is needed, but in most cases the benefits of exercise 17. Selvadurai HC, Blimkie CJ, Meyers N, et al. Randomized con-
outweigh the potential harms, so there is no reason to discourage the trolled study of in-hospital exercise training programs in chil-
integration of exercise in all CF patients care. dren with cystic fibrosis. Pediatr Pulmonol 2002;33:194-200.
In the past few years, new technologies (video game activities, 18. Gruber W, Orenstein DM, Braumann KM, Beneke R. Interval
social media and web-based platforms) can also be integrated in the exercise training in cystic fibrosis -- effects on exercise capac-
rehabilitation process. Further research is needed to study the long- ity in severely affected adults. J Cyst Fibros 2014;13:86-91.

[Monaldi Archives for Chest Disease 2021; 91:1501] [page 143]

Review

19. Hebestreit H, Kieser S, Junge S, et al. Long-term effects of a 37. Burtin C, Hebestreit H. Rehabilitation in patients with chronic
partially supervised conditioning programme in cystic fibrosis. respiratory disease other than chronic obstructive pulmonary
Eur Respir J 2010;35:578-83. disease: exercise and physical activity interventions in cystic
20. Santana-Sosa E, Gonzalez-Saiz L, Groeneveld IF, et al. fibrosis and non-cystic fibrosis bronchiectasis. Respiration
Benefits of combining inspiratory muscle with 'whole muscle' 2015;89:181-9.
training in children with cystic fibrosis: a randomised con- 38. Bott J, Blumenthal S, Buxton M, et al. Guidelines for the phys-
trolled trial. Br J Sports Med 2014;48:1513-7. iotherapy management of the adult, medical, spontaneously
21. Sosa ES, Groeneveld IF, Gonzalez-Saiz L, et al. Intrahospital breathing patient. Thorax 2009;64:i1-51.
weight and aerobic training in children with cystic fibrosis: A 39. Warnock L, Gates A. Chest physiotherapy compared to no
randomized controlled trial. Med Sci Sports Exerc 2012; chest physiotherapy for cystic fibrosis. Cochrane Database
44:2–11. Syst Rev 2015;2015:CD001401.
22. Wheatley CM, Baker SE, Morgan MA, et al. Effects of exer- 40. Main E, Prasad A, Schans C. Conventional chest physiothera-
cise intensity compared to albuterol in individuals with cystic py compared to other airway clearance techniques for cystic
fibrosis. Respir Med 2015;109:463-74. fibrosis. Cochrane Database Syst Rev 2005:CD002011.
23. Del Corral T, Cebrià I Iranzo MÀ, et al. Effectiveness of a 41. Morrison L, Agnew J. Oscillating devices for airway clearance
home-based active video game programme in young cystic in people with cystic fibrosis. Cochrane Database Syst Rev
fibrosis patients. Respiration 2018;95:87-97. 2014:CD006842.
24. Bishay LC, Nelson E, Williams K, et al. Effect of a wearable 42. Elkins MR, Jones A, van der Schans C. Positive expiratory
fitness tracker on exercise tolerance for adults with cystic pressure physiotherapy for airway clearance in people with
fibrosis: a pilot randomized clinical trial. Pediatr Pulmonol cystic fibrosis. Cochrane Database Syst Rev 2006:CD003147.
2018;53(S2):345. Added to CENTRAL: 30 November 2018 | 43. Mckoy NA, Wilson LM, Saldanha IJ, et al. Active cycle of

ly
2018 Issue 11. breathing technique for cystic fibrosis. Cochrane Database
25. Holmes H, Wood J, Jenkins S, et al. Xbox Kinect™ represents Syst Rev 2016;7:CD007862.

on
high intensity exercise for adults with cystic fibrosis. J Cyst 44. Bradley J, Moran F. Physical training for cystic fibrosis.
Fibros 2013;12:604-8. Cochrane Database Syst Rev 2008;1:CD002768.
26. Salonini E, Gambazza S, Meneghelli I, et al. Active video 45. Elbasan B, Tunali N, Duzgun I, Ozcelik U. Effects of chest

e
game playing in children and adolescents with cystic fibrosis: physiotherapy and aerobic exercise training on physical fitness
Exercise or just fun? Respir Care 2015;60:1172-9.
27. O'Donovan C, Greally P, Canny G, et al. Active video games
us
in young children with cystic fibrosis. Ital J Pediatr 2012;38:2.
46. Hulzebos HJ, Snieder H, van der Et J, et al. High-intensity
as an exercise tool for children with cystic fibrosis. J Cyst interval training in an adolescent with cystic fibrosis: a physi-
al
Fibros 2014;13:341-6. ological perspective. Physiother Theory Pract 2011;27:231-7.
28. Smith A, Gouick L. WS05.2 30 day challenge – using social 47. Shoemaker MJ, Hurt H, Arndt L. The evidence regarding exer-
ci

media to support adult CF patients to exercise in the adult CF cise training in the management of cystic fibrosis: a systematic
er

service Dundee. J Cyst Fibros 2015;14;S9. review. Cardiopulm Phys Ther J 2008;19:75-83.
29. Cox NS, Alison JA, Button BM, et al. Feasibility and accept- 48. Tejero García S, Giráldez Sánchez MA, Cejudo P, et al. Bone
m

ability of an internet-based program to promote physical activ- health, daily physical activity, and exercise tolerance in
ity in adults with cystic fibrosis. Respir Care 2015;60:422-9. patients with cystic fibrosis. Chest 2011;140:475-81.
om

30. Cox NS, Eldridge B, Rawlings S, et al. A web-based interven- 49. Paranjape SM, Barnes LA, Carson KA, et al. Exercise
tion to promote physical activity in adolescents and young improves lung function and habitual activity in children with
adults with cystic fibrosis: protocol for a randomized con- cystic fibrosis. J Cyst Fibros 2012;11:18-23.
-c

trolled trial. BMC Pulm Med 2019;19:253. 50. Klijn PH, Oudshoorn A, van der Ent CK, et al. Effects of
31. Lang RL, Wilson C, Stockton K, et al. CyFiT telehealth: pro- anaerobic training in children with cystic fibrosis: a random-
on

tocol for a randomised controlled trial of an online outpatient ized controlled study. Chest 2004;125:1299-305.
physiotherapy service for children with cystic fibrosis. BMC 51. Schmidt AM, Jacobsen U, Bregnballe V, et al. Exercise and
Pulm Med 2019;19:21. quality of life in patients with cystic fibrosis: A 12-week inter-
N

32. Hebestreit H, Kriemler S, Radtke T. Exercise for all cystic vention study. Physiother Theory Pract 2011;27:548-56.
fibrosis patients: is the evidence strengthening? Curr Opin 52. Moorcroft AJ, Dodd ME, Morris J, Webb AK. Individualised
Pulm Med 2015;21:591-5. unsupervised exercise training in adults with cystic fibrosis: a
33. National Guideline Alliance (UK). Cystic Fibrosis: Diagnosis 1 year randomised controlled trial. Thorax 2004;59:1074-80.
and management. London: National Institute for Health and 53. Bradley J, Moran F. Physical training for cystic fibrosis.
Care Excellence (UK); 2017 Oct 25. Cochrane Database Syst Rev. 2008;1:CD002768.
34. Flume PA, Mogayzel PJ Jr, Robinson KA, et al. Cystic fibro- 54. Schneiderman JE, Wilkes DL, Atenafu EG, et al. Longitudinal
sis pulmonary guidelines: pulmonary complications: hemop- relationship between physical activity and lung health in
tysis and pneumothorax. Am J Respir Crit Care Med patients with cystic fibrosis. Eur Respir J 2014;43:817-23.
2010;182:298-306. 55. Hebestreit H, Kieser S, Junge S, et al. Long-term effects of a
35. Moran A, Brunzell C, Cohen RC, et al. Clinical care guidelines partially supervised conditioning programme in cystic fibrosis.
for cystic fibrosis-related diabetes: a position statement of the Eur Respir J 2010;35:578-83.
American Diabetes Association and a clinical practice guide- 56. Hebestreit H, Hulzebos EHJ, Schneiderman JE, et al.
line of the Cystic Fibrosis Foundation, endorsed by the Cardiopulmonary exercise testing provides additional prog-
Pediatric Endocrine Society. Diabetes Care 2010;33:2697-708. nostic information in cystic fibrosis. Am J Respir Crit Care
36. Johannesson M. Effects of pregnancy on health: certain aspects Med 2019;199:987-95.. Erratum in: Am J Respir Crit Care
of importance for women with cystic fibrosis. J Cyst Fibros Med 2020;202:780.
2002;1:9-12. 57. Hebestreit H, Schmid K, Kieser S, et al. Quality of life is asso-

[page 144] [Monaldi Archives for Chest Disease 2021; 91:1501]

 Review

ciated with physical activity and fitness in cystic fibrosis. fibrosis and non-cystic fibrosis bronchiectasis. Respiration
BMC Pulm Med 2014;14:26. 2015;89:181-9.
58. Urquhart D, Sell Z, Dhouieb E, et al. Effects of a supervised, 62. Lebecque P, Lapierre JG, Lamarre A, Coates AL. Diffusion
outpatient exercise and physiotherapy programme in children capacity and oxygen desaturation effects on exercise in
with cystic fibrosis. Pediatr Pulmonol 2012;47:1235-41. patients with cystic fibrosis. Chest 1987;91:693-7.
59. Pérez M, Groeneveld IF, Santana-Sosa E, et al. Aerobic fitness 63. Albert RK, Schuller JL, COPD Clinical Research Network.
is associated with lower risk of hospitalization in children with
Macrolide antibiotics and the risk of cardiac arrhythmias. Am
cystic fibrosis. Pediatr Pulmonol 2014;49:641-9.
J Respir Crit Care Med 2014;189:1173-80.
60. Schindel CS, Hommerding PX, Melo DA, et al. Physical exer-
cise recommendations improve postural changes found in chil- 64. Radtke T, Nevitt SJ, Hebestreit H, Kriemler S. Physical exer-
dren and adolescents with cystic fibrosis: a randomized con- cise training for cystic fibrosis. Cochrane Database Syst Rev
trolled trial. J Pediatr 2015;166:710-6.e2. 2017;11:CD002768.
61. Burtin C, Hebestreit H. Rehabilitation in patients with chronic 65. Kriemler S, Kieser S, Junge S, et al. Effect of supervised train-
respiratory disease other than chronic obstructive pulmonary ing on FEV1 in cystic fibrosis: a randomised controlled trial. J
disease: exercise and physical activity interventions in cystic Cyst Fibros 2013;12:714-20.

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