Journal of Cystic Fibrosis 21 (2022) 272–281

Contents lists available at ScienceDirect

Journal of Cystic Fibrosis

journal homepage: www.elsevier.com/locate/jcf

The effectiveness of exercise interventions to increase physical activity

in Cystic Fibrosis: A systematic review
M. Curran a,b,c,∗, AC. Tierney a,c,f,g, B. Button d,e, L. Collins b, L. Kennedy b, C. McDonnell b,
B. Casserly b, R. Cahalan a,c,h
a
School of Allied Health, University of Limerick, Limerick, Ireland
b
University Hospital Limerick, Limerick, Ireland
c
Health Research Institute, University of Limerick, Limerick, Ireland
d
Departments of Respiratory Medicine and Physiotherapy, The Alfred, Melbourne, Australia
e
Department of Medicine, Nursing and Health Sciences, Monash University, Melbourne, Australia
f
Department of Dietetics, Nutrition and Sport, La Trobe University, Melbourne, Australia
g
Health Implementation Science and Technology Research Group, Health Research Institute, University of Limerick, Limerick, Ireland
h
Physical Activity for Health Research Cluster, Health Research Institute, University of Limerick, Limerick, Ireland

a r t i c l e i n f o a b s t r a c t

Article history: Physical activity (PA) and exercise have numerous benefits in Cystic Fibrosis (CF) including improved lung
Received 29 March 2021 function, exercise capacity and quality of life. Despite these benefits, the effectiveness of interventions to
Revised 10 October 2021
promote PA in this population are still largely unknown. The objective of this review was to synthesise
Accepted 25 October 2021
existing research and determine whether exercise interventions are effective in promoting PA in peo-
Available online 6 November 2021
ple with CF. Using the PRISMA guidelines, a comprehensive search was conducted. Fifteen studies (463
Keywords: participants) met the inclusion criteria. Eleven studies demonstrated improvements in PA in both short-
Physical activity promotion and long-term interventions. However, the interventions were variable across the included studies, with
Exercise a large inconsistency in PA assessment tools used. Aerobic training and activity counselling were the two
Cystic fibrosis elements identified in this review which most consistently improved PA. Future research should consider
Interventions larger sample sizes and the use of accurate instruments to assess and track PA levels longitudinally.
Training
© 2021 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

1. Introduction
Cystic Fibrosis (CF) is a chronic and life limiting condition. Im-
proved CF management has led to a median survival age in 2019
of 48.4 years [17]. CF is a complex, multisystem disease, charac-
terised by recurrent pulmonary infections and nutritional deficien-
cies [9]. The course of CF progression is denoted by exacerbations
which can cause increased coughing, increased energy expenditure
with a reduced body mass index (BMI) and declining pulmonary
function [35]. As a result, people with CF (PWCF) may avoid or
limit physical activity (PA) due to factors such as fatigue and short-
ness of breath resulting in progressive reductions in lung function,
deficits in skeletal muscle aerobic and anaerobic capacity and mus-
cle strength [50].
The benefits of PA amongst PWCF include improved lung func-
tion [47], quality of life (QOL) [45] and reduced hospital admissions
[14]. Higher levels of PA are positively related to exercise capacity
[24]. Most notably, it has been found that aerobically fit patients
∗
Corresponding author.
E-mail address: maire.curran@ul.ie (M. Curran).
live longer than their less fit peers [38]. Therefore, it is not sur-
prising that PA is a key component of CF management [9].
PA can be described as any bodily movement that causes
an increase in energy expenditure above that of resting en-
ergy expenditure [8]. Exercise is a type of PA which consists of
planned, structured and repetitive body movements that aim to
improve/maintain physical fitness [37]. PA can be measured either
subjectively or objectively. Subjective measures rely on user recall
and are an estimation of PA levels. These include self-report ques-
tionnaires, exercise diaries and logs [40]. Objective measures are
a more direct measurement of PA and can be measured through
the use of pedometers, accelerometers and activity monitors [6].
Despite the clear benefits of PA in CF, a recent systematic review
found that adults with CF fail to meet recommended PA and step
count guidelines [49]. Therefore, interventions which aim to in-
crease PA in this cohort are of considerable interest.
PA interventions in CF can take numerous forms including in-
dividualised exercise programmes, behaviour change interventions,
supervised or unsupervised training, telehealth interventions, edu-
cation or self-monitoring [20]. There are no definite optimal strate-
gies to integrate exercise into CF management [25]. The benefi-

https://doi.org/10.1016/j.jcf.2021.10.008
1569-1993/© 2021 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
M. Curran, AC. Tierney, B. Button et al. Journal of Cystic Fibrosis 21 (2022) 272–281

Table 1
Keywords.

Cystic Fibrosis OR CF OR mucoviscidosis

Physical activ∗ OR physically active OR exercise OR aerobic exercise OR anaerobic exercise OR physical fitness OR fitness OR active OR physical exertion OR
physical education OR training OR sport∗ OR walk∗ OR exercis∗ OR activity OR exercise therapy OR habitual activity OR exertion
promot∗ OR uptake OR increase OR start OR educat∗ OR program∗ OR Diary OR Interview∗ OR Physical activity tool OR physical activity measurement OR
Questionnaire∗ OR Recall OR Self report∗ OR Self-report∗ OR Subjective OR Survey OR Acceleromet∗ OR Activity monitor∗ OR activity monitors OR calorimet∗ OR
CSA monitor OR Direct observation OR Doubly labelled water OR DLW OR Global positioning system∗ OR Heart rate monitor∗ OR Indirect calorimet∗ OR Objective
OR Pedomet∗ OR step∗ OR step count∗ OR MET∗ OR metabolic equivalent OR energy expenditure

cial effects of CFTR modulators on health outcomes for PWCF has ing databases: Academic Search Complete, AMED, Biomed, CINAHL
been substantial and may impact PA and aerobic capacity in this complete, MEDLINE, Pubmed, PsycINFO, and SPORTDiscus. Key-
population [44]. A previous Cochrane review evaluated the effec- words included in the search strategy relate to (i) Cystic Fibrosis,
tiveness of randomised controlled trials (RCTs) that used exercise (ii) PA or exercise intervention (iii) subjective or objective measure
interventions to promote PA levels in PWCF [15]. Methodological of PA (such as pedometer, accelerometer, self-report exercise di-
differences and variability in research parameters of included tri- aries) or the promotion of exercise. Articles were limited to those
als reported insufficient evidence to support any specific strategy involving humans and published in English.
or intervention to maintain or increase PA levels in PWCF. There-
fore, the aim of this review was to systematically search, synthesise 2.4. Quality assessment of methodology
and evaluate the literature, including all study designs, investigat-
ing the effect of relevant interventions on PA levels (primary out- MC and RC independently assessed the quality of included ar-
come) and other secondary outcomes (lung function, aerobic ca- ticles with the use of a modified Downs and Black quality assess-
pacity, QOL and body composition) in PWCF. ment tool for methodological quality [19]. The Downs and Black
tool is a reliable and valid measure for assessing the quality of
2. Methods
randomised and non-randomised health care studies [19]. Previ-
ous research has frequently employed a modified version by sim-
2.1. Overview
plifying the power question and awarding a single point if a study
had sufficient power to detect a clinically important effect, where
An initial search of relevant databases identified one Cochrane
the probability value for a difference being due to chance is <5%
systematic review conducted in 2013 which specifically investi-
[34]. The modified version which we employed in this study there-
gated interventions to promote PA in CF, however this review fo-
fore has a maximum score of 28. Each paper was assigned a grade
cused solely on RCTs [15]. This current review aims to expand
of “excellent” (24–28 points), “good” (19–23 points), “fair” (14–18
on the study design investigated while also incorporating recent
points) or “poor” (<14 points) [34]. Quality appraisal of all in-
research in this area. The review is reported following the Pre-
cluded studies can be found in supplementary materials.
ferred Reporting Items for Systematic Reviews and Meta-analyses
(PRISMA) guidelines [31]. This review is registered on the PROS-
PERO database of systematic reviews to prevent duplication of re- 2.5. Data extraction and synthesis
search and the published protocol can be accessed via the PROS-
PERO database (CRD42020156783). A narrative synthesis of the Assessors (MC or RC) were not blinded to the journal, author,
findings was performed to provide a summary of the effects of the or title of the papers when extracting data. The data extracted in-
PA intervention, the PA assessment tool used and overall quality cludes (i) author, (ii) sample size, (iii) participant gender, (iv) par-
of each study. A meta-analysis was not possible due to the hetero- ticipant age, (v) inclusion/exclusion criteria, (vi) intervention and
geneity in the PA assessment tools used across the studies. control description, (vii) physical activity outcome measure, (viii)
main results and (ix) Downs and Black score (Table 2).
2.2. Study criteria
3. Results
The purpose of the review was to identify studies which aimed
to increase PA levels in PWCF. Therefore, articles had to consist of
The search terms yielded 3722 results, which was reduced to
an exercise intervention and had to specifically assess PA levels, ei-
2432 once duplicates were removed. The PRISMA flowchart can be
ther subjectively or objectively. The population included PWCF, in-
found in Fig. 1. The Rayyan Qatar Computing Research Institute
cluding both adults and children. All study designs were included.
(QCRI) system was used to keep track of and organise all yielded
No date limits were placed on the search. All data collection was
results from the search strategy (Ouzzani et al, 2016). MC manu-
completed as of November 2020, therefore no publications after
ally removed duplicates before importing titles for screening into
this date are included in the review. Primary outcomes reviewed
Rayyan QCRI. Two reviewers (MC and RC) screened the remain-
were assessment of PA levels, subjectively or objectively. Secondary
ing titles and abstracts of studies identified through the search
outcomes were health outcomes including lung function, aerobic
strategy against inclusion/exclusion criteria. Any disagreements be-
capacity, QOL and body composition. Exclusion criteria were any
tween reviewers were resolved through discussion or a third in-
studies that did not consist of a CF population, did not assess PA
dependent reviewer if required (AT). Thirty-two full text articles
levels (subjectively or objectively), or assessed the efficacy of other
were reviewed against the inclusion and exclusion criteria and 15
interventions (such as medication/nebulisation/airway clearance).
were included for review. References of all included papers were
Studies which solely assessed adherence to an intervention and did
screened, although this did not yield further articles.
not include a PA outcome measure were excluded.

2.3. Search strategy 3.1. Trial characteristics

Two assessors (MC and RC) independently searched for the There was a variety of study designs used across each of
presence of an agreed range of keywords (Table 1) in the follow- the included studies. One study used an ABAB single subject

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M. Curran, AC. Tierney, B. Button et al. Journal of Cystic Fibrosis 21 (2022) 272–281

Table 2
Description of studies included in the review.
Sample Mean age Inclusion/Exclusion Intervention and Control Downs and
Study size Gender (years) criteria Description PA Measure Results Black Score
Beaudoin et al 14 6M/8F 33.7 Participants with CF; 12-week combined aerobic and SenseWear Armband After 12 weeks 16/28
[3] age>18 years; sedentary resistance training study. Pro 3 and a PA questionnaire-assessed PA was
(less than 100 min/week Exercise group: aerobic and questionnaire (not significantly higher in the
of structured exercise resistance training exercises 3x per stated which one). training group compared to
assessed by a PA week. Once every 4 weeks, However, it is the control group, MD 19.85 %
questionnaire and phone participants received a supervised reported the (1.92 to 37.80).
interview; FEV1 >40% training session and a phone call questionnaire No differences between
predicted; clinically stable on a weekly basis includes 14 questions, groups were found in total
for the last 6 weeks; IGT; • Aerobic training consisted of and each question energy expenditure, or the
CF-related diabetes walking, jogging, cycling and was given 1 to 4 number of daily steps.
without pharmacological elliptic trainer. points (1 = never, Patients in the exercise group
treatment or elevated 1-h • Resistance training consisted of 2 = sometimes, improved significantly their
plasma glucose 5 - 7 exercises for large muscle 3 = often, and 2-h plasma glucose values and
concentration during an groups using their own body 4 = always) by the presented a reduction of 17.2%
oral glucose tolerance test weight, free weights and elastic subject. A maximum in total glucose excursion.
Exclusion criteria: current bands (goal 8 - 12 repetitions with of 56 points were
pulmonary exacerbation; a weight of 30% - 50% of one possible, and the
use of oral or intravenous repetition maximum). addition of the
corticosteroids; low SaO2 Control group: no information was results divided by 56
during exercise; history of reported in the original gives the score in the
haemoptysis in the last 6 publication. percentage of PA.
weeks.
Bernard et al [4] 3 3F 10.6 School-aged child ABAB study design: One and Exercise diary and a All three participants 19/28
diagnosed with CF, three-month follow up. Sportline pedometer demonstrated a higher
medically recommended The exercise target was 4 times average daily pedometer
exercise, nonadherence to per week for 20 minutes. reading during the treatment
exercise despite standard Treatment consisted of two parts: phases than the baseline
efforts, current training and implementation. phases.
non–school-related During training, the experimenter BMI and fat percentage did
exercise that was no went to the child’s home for three not change from baseline.
more than two times per 2-hr sessions. All three participants
week for a maximum of Session 1: Consisted of training demonstrated higher levels of
20min each time, and the parent(s) to set up a token exercise at the one and
FEV1 falling in the mildly economy to reinforce the child’s three-month follow up.
impaired range. exercise behaviour. The token
economy was designed so that the
child earned a small, immediate
reward. The experimenter met
with the child to teach her how to
properly exercise to ensure that
she was exercising at the
appropriate level.
Session 2: Subjects provided with
information on proper diet and
practice exercise techniques
Session 3: Discussion around
problems encountered with the
token economy and problem solve
with the family about how to
rectify them.
Cox et al [13] 10 4M/6F 30 Diagnosis of CF 8-week feasibility study. Omron Pedometer & No significant difference in PA 20/28
Excluded if comorbidity (No long-term follow up) SenseWear Pro3 participation.
affecting PA participation, Online intervention (ActivOnline) armband Subjects reported system
post lung transplant, were provided users with graphical usability and perceived
pregnant or if they had representation of PA entries, and benefit favourably via semi
no access to internet at direct communication between structured interviews and
home. subjects and research team. All likert scales. Positively
subjects received a phone call at received by participants but
baseline and each fortnight. The they would have preferred
purpose of the phone call was to more mobile interface such as
foster goal setting by discussing a mobile application.
barriers and enablers to PA. No significant different in
No control group. modified shuttle walk
distance or CFQR.
Gupta et al [22] 52 30M / 12.5 Diagnosis of CF, no 1-year randomised controlled trial. HAES No significant difference in 24/28
22F intravenous antibiotics (Patients followed up every 3 habitual activity found.
prior to 1 month of months for a period of 12 months) In experimental group there
enrolment, FEV1 ≥20% Intervention: home-based exercise was significant improvement
predicted. programme, 3x per week. in maximal oxygen uptake.
Excluded if: Resistance training and plyometric Experimental group had a
musculoskeletal disorder jumping exercises. Telephone significant improvement in
such as rheumatoid guidance every 2 weeks. their exercise capacity, quality
arthritis, muscular Control: continued routine PA. of life, and serum vitamin D
dystrophy or chronic levels.
renal failure. Differences between groups
for changes in pulmonary
function were non-significant.
Changes in FEV1 and FVC
showed no significant
difference between the 2
groups.
(continued on next page)

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M. Curran, AC. Tierney, B. Button et al. Journal of Cystic Fibrosis 21 (2022) 272–281

Table 2 (continued)

Study Sample Gender Mean age Inclusion/Exclusion Intervention and Control PA Measure Results Downs and
size (years) criteria Description Black Score

Hebestreit et al 38 19M / 19.5 (int.) Diagnosis of CF; age ≥12; 6 month randomised controlled MTI/CSA 7164 Change in time spent in 18/28
[23] 19F 19.4 FEV1 ≥35% of predicted; trial. Actigraph vigorous activities (assessed
(control) ability to perform PA. (Patients were seen at baseline accelerometer by accelerometry) was
Excluded if CF related or and after 3, 6, 12, 18 and 24 significantly different between
non-CF related conditions months) the groups during long-term
posed a risk when Intervention: Patients added 3 follow-up. Although there was
exercising. hours of sports per week to a trend for vigorous activities
previous activity level for at least in favour of the intervention
6 months. Activity counselling and group throughout the initial
individual activity plan developed. 12 months of the study, the
Control: Maintained current level effect was not significant.
of activity for 12 months. Positive effects on peak
oxygen uptake, maximal
workload, vigorous PA, forced
vital capacity and perceived
health.
Hommerding 34 20M / 13.4 (int.) Diagnosis of CF; 7-20 3-month randomised controlled Exercise Diary Significant increase in PA 20/28
et al [26] 14F 12.7 years; disease stable; no trial. (self-reported) for
(control) exacerbation in last 15 (No long-term follow-up) intervention group.
days. Intervention: Patients were given a No change in FEV1 , maximum
Excluded if cognitive manual with guidelines for aerobic exercise capacity or QoL.
impairments made testing physical exercise; telephone calls
impossible; bone and made every two weeks to
muscle abnormalities; reinforce recommendations.
heart disease. Control: Verbal exercise
recommendations only, at the
beginning of the study.
Klijn et al [28] 20 Not in- 13.6 (int.) 9-18 years; stable clinical 12-week randomised controlled HAES No significant change in PA 21/28
dicated 14.2 condition; absence of trial post intervention or at
(control) musculoskeletal (12-week follow-up) follow-up.
disorders; FEV1 >30% of Intervention: Two days per week Patients in intervention group
predicted. for 12 weeks of anaerobic significantly improved
activities. Each session last 30-45 anaerobic performance,
minutes. aerobic performance (VO2
Control: Continue normal daily peak) and QOL.
activities and physiotherapy After follow up period
regime. anaerobic performance and
QOL only were improved. No
improvements in control
group.
Kriemler et al 39 25M/14 23.8 CF diagnosis; age 12+ 6 month randomised controlled MTI/CSA 7164 No significant change in PA. 23/28
[29] F (endurance) years; FEV1 ≤35% of trial Actigraph FEV1 increased significantly in
19.0 predicted; ability to (Follow up at 3, 6, 12 and 24 & self-reported both intervention groups
(strength) perform PA without harm. months) training diary compared with the control
20.3 Excluded if non-CF Intervention: patients randomly group at 3, 6 and 12 months.
(control) related chronic diseases assigned to Strength training or This remained improved at 24
and conditions posed an Endurance training. months for the strength
increased risk when Strength training consisted of training group.
exercising. upper and lower body Body composition, average or
strengthening exercises and moderate and vigorous PA and
included sets and reps. This was muscle power were not
checked by a fitness instructor and significantly different.
adapted as required.
Aerobic training group: This was
based on preference with HR
monitors that provided a target
HR.
Training of 3×30 mins / week for
6 months. Measurements at
baseline, 3, 6, 12 and 24 months.
Control Group: To continue normal
routine and keep activity level
constant.
Moola et al [32] 13 Int: 2M, 11.6 CF diagnosis 8-week randomised controlled Accelerometry Positive trends were also 23/28
5F Transplant candidates trial. (device not stated) reported in terms of increases
Control: were not considered for (No long-term follow-up) in PA, reductions in time
3M, 3F this study. Medically Family-mediated PA counselling spent being sedentary, and
unstable children, such as program. Each session was improvements in most
those with acute approximately 90 min in duration. dimensions of quality of life
respiratory distress or Participants engaged in four pre- to post-intervention.
infection, were not counselling sessions to examine This was not significant.
contacted for the acceptability and feasibility of The intervention was found to
participation. Patients PA counselling in the care of be feasible and acceptable
with cognitive and children with CF. with good recruitment,
intellectual disabilities The PA counsellors delivered retention, adherence, and
were excluded from the manualised content to acceptability.
study or those outside of participants, discussed PA goals
local region due to travel and behaviours, taught PA
constraints to CF centre. self-management and provided
empathy and support for PA
challenges.
(continued on next page)

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M. Curran, AC. Tierney, B. Button et al. Journal of Cystic Fibrosis 21 (2022) 272–281

Table 2 (continued)

Study Sample Gender Mean age Inclusion/Exclusion Intervention and Control PA Measure Results Downs and
size (years) criteria Description Black Score

Paranjape et al 78 45M/ 10 Diagnosis of CF, age
[36] 33F 6-16y, no concurrent IV
antibiotic treatment and
the ability to perform
exercise.
Excluded if more than
10% decline in lung
function compared to
previous clinic visit or
required treatment with
oral or intravenous
antibiotics for a
pulmonary exacerbation.
Schneiderman- 65 38M/ 13.4 (int.) CF diagnosis; 7-19 years;
Walker et al 27F 13.3 FEV1 ≥40% of predicted.
[46] (control) Excluded if enrolled in
other studies, history of
non-compliance or
irregular clinic visits.
Selvadurai et al 66 28M/ 13.2 Children admitted to
[48] 38F (aerobic) hospital with an
13.1 intercurrent pulmonary
(resistance) infection.
13.2 Excluded if: pulmonary
(control) hypertension or required
daytime oxygen prior to
the pulmonary
exacerbation.
Swisher and 12 5M / 7F 11.9 CF diagnosis, medically
Moffet [51] stable, free of acute
pulmonary exacerbation.
Excluded if cognitive
impairment affected
understanding and
completing
questionnaires.
Tomlinson et al 9 6M/ 3F 30.9 People with CF > 14
[53] years of age and clinically
stable at the time of
recruitment.
Two-month pre-post study design. HAES
(No long-term follow-up)
Subject-designed exercise regimen
based on self-reported activity
assessment. 20–30 minutes of
moderate to vigorous activity at
least five times a week. The type
of exercise was jointly decided by
the participant and
physiotherapist.
3 year randomised controlled trial.
(Tested yearly for 3 years)
Intervention: 3-year home exercise
programme; a minimum of 20
minutes of aerobic exercise, at a
heart rate of approximately 150
beats/min or 70%-80% of their max
HR. 3 times weekly. Monitoring
techniques were reviewed during
phone and clinic conversations.
Control: usual PA participation.
Randomised controlled trial in the
inpatient setting.
(Outcomes assessed at hospital
discharge and 1-month post
discharge)
Subjects randomized to three
groups: aerobic training, resistance
training and control group.
Aerobic training group: 5×30 mins
aerobic activities per week.
Resistance training group
exercised upper / lower limbs
against graded resistance machine/
Control: standard chest
physiotherapy.
3 month pilot study.
Each participant completed PA and
QOL questionnaires at baseline
and 3 months.
10,0 0 0 step daily target.
Discussions held on ways to
increase PA. Weekly follow up
phone calls to obtain pedometer
counts and discuss PA.
Control: None.
8 week home based online
exercise intervention.
All participants undertook eight
weeks of video-calls
supervised by the same exercise
therapist, receiving up to three
supervised exercise sessions per
week.
Exercise sessions were intended to
be 30 min in duration. All sessions
were undertaken in participants
own home, on a one-to-one basis
with the exercise therapist. The
content of each session was
personalised to each participant
for the purposes of this study,
dependent on equipment available
in participants’ homes.
Self-reported activity 18/28
demonstrated that weekend
PA increased, although this
was not statistically
significant.
Study participants
demonstrated significant
improvement in exercise
capacity as measured by the
MSWT.
Body image perception
significantly increased.
In secondary analyses,
subjects improving exercise
capacity showed significant
increases in lung function and
self-reported habitual activity.
Daily exercise diary. Activity diary scores higher 20/28
Date, type of activity, for exercise group at Year 1, 2
duration in minutes and 3 (self-reported). Activity
and level of intensity diary scores were higher for
(from 1 to 5 where, the exercise group compared
1 = easy, 5 = too with the control group,
difficult to talk). indicating that the exercise
group was participating in
more PA than the control
group at year 1 (P= .06), year
2 (P= .006), and year 3 (P=
.01).
Control group showed greater
annual decline in percent of
predicted FVC than exercise
group, and greater decline in
FEV1 . Exercise group reported
an improved sense of
wellbeing.
7-day activity diary & Significantly improved PA 23/28
MTI/CSA 7164 levels for both aerobic and
Actigraph resistance training group but
not control group.
Aerobic training group had
significantly better peak
aerobic capacity, activity
levels and QOL than resistance
training group.
Resistance training group had
greater weight gain, FEV1 and
lower limb strength.
PA questionnaire for PA scores (PAQ) improved in 6 14/28
children and of 12 participants
adolescents (PAQ) & (self-reported). Only 4
Pedometers (brand participants returned log
not stated) sheets of pedometer data. Of
these, 3 met the goal of
10,0 0 0 steps at least 50% of
the time. QOL improved in
many dimensions of the CFQ,
particularly in the domain of
emotional (8 of 12 improved)
and respiratory (7 of 12
improved).
Accelerometer The study was not powered to 17/28
(GENEActiv) detect changes in these
outcomes and no changes
were seen across all variables
during the course of the
study.
Participants found using
Skype for exercise useful, with
ratings ranging from
7/10–10/10 (mean 9/10)
All participants stated they
would be happy to take part
in future research studies.
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M. Curran, AC. Tierney, B. Button et al. Journal of Cystic Fibrosis 21 (2022) 272–281

Table 2 (continued)

Study Sample Gender Mean age Inclusion/Exclusion Intervention and Control PA Measure Results Downs and
size (years) criteria Description Black Score

Tuzin et al [54] 10 8M / 2F 9.4 Age 7-15 years, disease of 4 or 6 week multiple baseline
moderate severity, disease design.
stability for 4 weeks+, (No long-term follow-up)
physician approval for Participants took part in one of
participation in exercise three studies.
programme. Studies I and II: four weeks. This
employed a token economy.
Study III: 6 weeks.
Home based, parent managed
behavioural programme to
increase PA.
[BMI: Body mass index; CF: Cystic fibrosis; CFQR: CF questionnaire-revised, F: female; FEV1 : Forced expiratory volume in 1 second; FVC: Forced vital capacity; HAES:
Habitual activity estimation scale; M: Male; PA: Physical activity; QOL: Quality of life].
Activity Point Self-reported activity levels - 10/28
Questionnaire & Eight children increased total
Caltrac electron activity 42.5% to 321 %, and 2
activity meters. children exercised more
consistently. Study II recorded
further activity increases at
6-week follow-up.
Activity meters also employed,
but were ineffective at
recording data (still enhanced
accuracy to some extent as
participants believed they
were being objectively
monitored). These were
ineffective as they monitored
activity in a vertical plane and
these children tended to
partake in activities involving
non vertical acceleration.
Study III validated reported
activity with increases of 7%
to 27% in VO2 max, 8% and
31.6% in VE max, and 14.2%
and 20% in Wmax.

Fig. 1. PRISMA flow chart with search strategy and results.

277
M. Curran, AC. Tierney, B. Button et al.
study design [4], one pre-post study [36], one multiple base-
line design [54], one open label study with two parallel arms
[3], four feasibility/pilot studies [13,32,51,53] and seven were RCTs
[22,23,26,28,29,46,48]. Most studies were conducted in an outpa-
tient setting, apart from one which was conducted in an inpatient
hospital setting [48]. The intervention period ranged from 18 days
[48] to three years [46].
3.2. Participants
A total of 463 PWCF participated in the 15 included stud-
ies. The number of subjects ranged from three [4] to 78 [36]. Of
the overall sample, 239 (n=51.6%) were males and 204 (n=44.1%)
were females, while gender breakdown was not provided in one
study [28]. Mean (SD) forced expiratory volume in one second
(FEV1 ) (% predicted) where provided across the included studies
was 75.4% (15.5%). Mean FEV1 predicted ranged from 50% [13] to
100% [26]. Three studies did not provide FEV1 data [4,51,54]. Mean
(SD) age of participants was 15.9 (5.8) years and ranged from 9.4
years [54] to 33.7 years [3]. Mean (SD) BMI data provided across
nine studies was 19.4 kg/m2 (2.63). BMI ranged from 14.6 [22] to
23.7kg/m2 [3]. Ten studies were conducted in children/adolescents
[4,22,26,28,32,36,46,48,51,54]. Three studies assessed a mixed chil-
dren/adult population [23,29,53], while two studies assessed adults
with CF only [3,13].
3.3. Intervention types
The impact of several interventions on PA were explored, in-
cluding two studies which used pedometers [13,51]. One of these
studies also incorporated an online intervention whereby partici-
pants logged their PA online on a daily basis and were provided
with a real-time graphical representation of their PA entries, as
well as an inbuilt messaging system [13]. Another study used an
online personalised video calling intervention where three exercise
sessions per week were supervised online over eight weeks [53].
Three studies used behavioural interventions. A token economy
was conducted in one paediatric study in which the target was
20 minutes of exercise, four times a week. The token economy
was designed so the child earned a small, immediate reward [4].
Another study conducted a family-mediated activity counselling
whereby four PA counselling sessions were provided to partici-
pants [32]. Finally, Tuzin et al [54] conducted a home-based token
economy and behavioural programme in which participants were
rewarded for increasing PA.
Gupta and colleagues [22] examined the effect of a resis-
tance training programme. This was a home-based intervention
whereby participants completed strength training three times per
week for one year. Three studies consisted of aerobic interven-
tions [26,36,46]. Hommerding and colleagues [26] provided a man-
ual to participants with guidelines for aerobic exercise, while
Schneiderman-Walker et al. [46] conducted a three-year home ex-
ercise programme where participants were expected to engage in
a minimum of 20 minutes of aerobic exercise three times a week.
Paranjape et al [36] assessed a home exercise regimen consisting
of moderate-vigorous PA exercises to be completed for 20-30 min-
utes at least five days a week. One study conducted an anaero-
bic intervention whereby participants completed anaerobic activi-
ties two days a week for 12 weeks [28].
Four studies assessed a combination of both resistance and aer-
obic exercises [3,23,29,48]. Beaudoin et al [3] used a combination
programme of both aerobic and resistance exercises, three times
per week for 12 weeks [3], while Kriemler and colleagues [29] ran-
domly assigned participants to either strength training or aerobic
training. Hebestreit et al [23] requested participants to add three
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Journal of Cystic Fibrosis 21 (2022) 272–281
hours of sports to their previous activity levels and they were en-
couraged to participate in strength training. Finally, Selvadurai et al
[48] completed a combination of aerobic and resistance interven-
tion in the inpatient setting.
3.4. Outcome measures
PA was measured either subjectively, objectively, or both
(Table 2). Subjective measures only were used in five studies
[22,26,28,36,46]. These included the Habitual Activity Estimation
Scare (HAES) which is valid and reliable in CF [22,28,36]. Two stud-
ies used an exercise diary [26,46] however the psychometric prop-
erties of these are yet to be established in CF. Objective measures
only were used in four studies [13,23,32,53]. Two of these used
the Actigraph which is valid and reliable in CF [23,29], while one
study did not state which device was used [32] and one study as-
sessed PA using an Omron pedometer [13] of which psychometric
properties were unclear. Six studies used both a subjective and an
objective assessment [3,4,29,48,51,54]. However it was unclear if
the tools used by two studies (Activity Point Questionnaire/Caltrac
electron activity meters [54]; unnamed pedometer/Physical Activ-
ity Questionnaire [51]) were psychometrically robust.
4. Primary outcome: PA
Six studies demonstrated a statistically significant increase in
PA [3,23,26,46,48,54]. Five further studies demonstrated increases
in PA but this was not statistically significant or was not tested for
significance [4,22,32,36,51]. Four studies showed no difference in
PA levels [13,28,29,53]. No study showed a deterioration in PA.
4.1. Studies demonstrating a significant improvement in PA
The six studies which noted a significant improvement in PA
ranged in duration from an average of 18 days (average inpa-
tient duration) [48] to three years [46]. Interventions were variable
across each of these studies. One partially supervised intervention
asked participants to add three hours of sports per week to their
previous activity level prior to the intervention and combine this
with activity counselling and an individualised activity plan [23].
At 18-24 months vigorous PA (hours (h)/week) was significantly in-
creased from baseline PA to 1.63±0.82 (p=0.047). Hommerding and
colleagues [26] provided a manual to participants for aerobic exer-
cise with telephone calls to reinforce activity. Self-reported PA sig-
nificantly improved (p=0.01). Schneiderman-Walker et al [46] con-
ducted an aerobic exercise program over three years. PA participa-
tion was significantly greater at the end of year one (p=0.06), year
two (p=0.006) and year three compared to baseline PA (p=0.01).
Selvadurai et al [48] completed a supervised inpatient program
comparing aerobic and resistance training in a powered study. PA
was assessed in a subgroup of participants pre-chest infection. Re-
sults demonstrated a significant improvement in PA for aerobic and
resistance training groups when compared to the control group
(P<0.05). Similarly, Beaudoin and colleagues also assessed the ef-
fectiveness of a combined aerobic and resistance training program.
Results demonstrated a significant improvement in PA in the ex-
ercise group compared to the control group (+11.57%, p<0.01). Fi-
nally, Tuzin et al [54] conducted a home-based, parent managed
behavioural program to increase PA. Participants completed indi-
vidualised aerobic activities. It was found that total activity in-
creased by between 42.5% and 321% from baseline in eight out
of ten participants with two children exercising more consistently.
The mean increase in PA was 92.6%.
M. Curran, AC. Tierney, B. Button et al.
4.2. Studies showing improved PA (not statistically significant)
Five studies found improved levels of PA, however some of
these studies did not reach significance while others were un-
able to complete data analysis to determine whether the differ-
ence was significant. Moola et al [32] conducted an eight-week
PA counselling intervention and participants demonstrated an av-
erage increase in moderate and vigorous intensity PA of six min-
utes per day (to 49±21 min/d; median: 49.5 min/d). The control
group participants remained were largely unchanged from base-
line (47±15 min/d; median: 42 min/d). Similarly, Paranjape and
colleagues [36] showed that PA increased but was not statistically
significant (HAES Weekday: p=0.40, HAES Weekend: p=0.17) fol-
lowing a subject-designed exercise regimen program. However, in
a secondary analysis, it was found that participants who had in-
creased their aerobic capacity recorded a significant increase in PA
(p=0.03). Bernard et al [4] found that PA levels were increased
during the intervention when compared to baseline but did not
complete further data analysis. Gupta and colleagues [22] com-
pleted a one-year RCT and found that self-reported PA was im-
proved but was not statistically significant between groups (HAES
weekend: p=0.15, HAES weekday: p=0.49). Finally, Swisher and
Moffett [51] found that self-reported PA improved in 50% of par-
ticipants (6/12). Furthermore, only four participants returned pe-
dometer data thereby limiting objective data analysis.
4.3. Studies demonstrating no/unclear improvement in PA
Four studies did not show any improvement in PA. Cox et al
[13] completed an eight-week, online intervention study and did
not find a change in sedentary time or in mild activity (p=0.8
and p=0.3, respectively). However, PA was maintained from hos-
pital discharge to the end of the intervention. Klijn et al [28] com-
pleted a 12-week RCT evaluating anaerobic activities. Compliance
with the program was high (98%), however this did not signifi-
cantly increase PA. Kriemler et al [29] conducted a six-month RCT
comparing strength training to aerobic training in 39 participants
and found that PA was not significantly changed throughout the
study duration. Finally, Tomlinson et al [53] completed an online
video calling intervention but as only seven participants completed
this study, they were unable to complete statistical analysis to de-
tect differences in PA and only provided descriptive statistics.
5. Secondary outcomes
FEV1 was positively impacted in three studies out of the ten
measuring this outcome [29,46,48]. However, only two of these
studies also had an associated significant increase in PA [46,48]. In
a three-year intervention, FEV1 declined more slowly in the aerobic
exercise group compared to the control group [46]. Selvadurai et al
[48] found that FEV1 was significantly improved following an inpa-
tient exercise intervention, however this was noted across both in-
terventions (aerobic: 6.54%; resistance 10.09%) and within the con-
trol group (4.51%). Improvements in aerobic capacity were found
in six of nine studies measuring this outcome [22,23,28,36,48,54].
Three of these studies had an associated increase in PA [23,48,54].
A significant improvement in QOL and wellbeing was observed in
four studies out of the ten assessing this outcome [22,23,28,48],
whereby two of these also reported an increase in PA [23,48].
Of the ten studies that measured body composition, only two re-
ported a significant change. One study found a significant de-
crease in skinfold thickness in the exercise group [23], while one
study reported an increase in body mass following aerobic train-
ing (2.09%), resistance training (7.25%) versus control group (2.69%)
[48].
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Journal of Cystic Fibrosis 21 (2022) 272–281
6. Quality assessment
The quality assessment using the Downs and Black Checklist is
presented in an online supplement. Scores ranged from 10/28 to
24/28 (Table 2). One study was excellent quality, eight studies were
good quality, five were fair, and one was poor quality.
7. Discussion
The aim of this systematic review was to systematically search,
synthesise and evaluate the effect of exercise and other relevant in-
terventions on PA participation amongst PWCF. A review of the lit-
erature identified 15 studies which met the inclusion criteria with
11/15 studies showing improvements in PA participation levels.
7.1. Optimal intervention for increased PA in PWCF
The components of effective interventions in this review
were difficult to evaluate as they varied in length, the level
of supervision provided, the type of exercise completed (aer-
obic/resistance/combined) or whether they assessed behavioural
strategies, token economies or an online intervention. Specific ac-
tivity guidelines do not exist in patients with CF [7], however in
this review, all studies which demonstrated an increase in PA con-
sisted of aerobic training or included aerobic training as part of
the intervention. Aerobic training has been found to promote PA
in other cohorts such as pulmonary artery hypertension [57] and
chronic obstructive pulmonary disease (COPD) [41]. Strategies to
increase PA in PWCF should include personalised programs [12].
For example, it has been suggested that aerobic training might
be especially indicated in PWCF with decreased aerobic capacity,
while resistance training may be more effective in those with re-
duced muscle strength [7]. A minimum of 20 minutes of exer-
cise was noted across each of these studies for the exercise du-
ration. The frequency varied from twice per week [3] to five times
per week [48] while the intensity of aerobic exercise was variable
across studies. This review found that the duration of the inter-
vention was effective at increasing PA across both short- and long-
term studies.
Each of the effective interventions incorporated activity coun-
selling through regular monitoring or supervision techniques. Most
studies monitored participants with either weekly or fortnightly
phone calls or during clinic conversations. Telephone assisted
counselling for PA has established a substantial body of evidence
to support its effectiveness in promoting PA in COPD [27], healthy
populations and in chronic disease [10]. Exercise is encouraged for
all PWCF but requires appropriate guidance [33]. As a result, there
is emerging evidence for personalised exercise training which may
be beneficial in chronic lung disease [1].
Technology is quickly becoming more popular and widespread
in health care provision, and it has been found that PWCF are con-
fident in the use of health-care technology [16]. Two studies in this
review used a telehealth intervention and found it was feasible and
well accepted by PWCF in both [13,53]. Wearable technology is a
fast developing, emerging area within PA research and this is likely
to continue as these devices become more widely available and
more readily accepted as a PA assessment and intervention tool [5].
Two studies in this review aimed to specifically increase PA using
a pedometer [13,51] with contrasting results found. Previous re-
search reported that pedometers can increase step count in COPD
[2] and asthma [11]. The pedometer studies in this review were
limited by small sample sizes which may reflect the differences ob-
served. Future research should consider the use of telehealth and
wearable devices as a means to assess and potentially increase PA
levels in PWCF.
M. Curran, AC. Tierney, B. Button et al.
7.2. The relationship between PA participation and secondary
outcome measures
Two studies which reported increased PA also showed improve-
ments in FEV1 [46,48]. FEV1 is an important prognostic indicator in
PWCF, that can be objectively and reliably measured, and continues
to be a key endpoint in CF interventions [52].
Aerobic capacity was increased in three studies which found
improved PA participation. These interventions were quite vari-
able as one was a hospital-based supervised study [48], one was
a token economy [54], and one was a six-month partially super-
vised home-based study [23]. Furthermore, in a secondary anal-
ysis, the authors of one study found that participants that im-
proved aerobic capacity also improved PA [36]. In contrast to
this, three studies which demonstrated an increase in PA, did not
find a simultaneous increase in aerobic capacity [3,26,46]. There-
fore, due to this inconsistent relationship between PA and aer-
obic capacity, it cannot be assumed that an increase in either
variable will impact on the other. The effects of CFTR modula-
tors such as lumacaftor/ivacaftor should also be considered go-
ing forward as this has previously been shown to increase both
PA and aerobic capacity [44,56]. It was not stated if the partici-
pants in any study in this review were receiving CFTR modulator
therapy.
Of the ten studies which assessed QOL, only two studies found
an associated increase in QOL with increased PA [23,48]. In con-
trast, a Cochrane review found that an exercise training program,
as part of pulmonary rehabilitation, in COPD and in interstitial
lung disease, found significant improvements in both QOL and PA
[18,30]. This research suggests that it may be more challenging
to improve QOL in a complex multi-system disease such as CF,
with a singular mode of intervention (exercise intervention only)
than in other respiratory populations [15]. Additionally, it should
be noted that most of the included interventions in both of these
reviews [18,30] consisted of group training, rather than individ-
ual training. The segregated nature of CF exercise interventions
due to strict infection control policies [12] may also be a factor to
consider.
Reductions in skinfold thickness and in lean body mass were
observed in just two studies [23,48], without affecting BMI. This
is supported by previous literature in CF in which similar results
were observed [21,39]. Increasing or maintaining weight in CF is
difficult due to pancreatic insufficiency which results in malabsorp-
tion and maldigestion of nutrients and fat-soluble vitamins [42].
While BMI was not affected in any of these studies, it is important
to consider the method of assessment of body composition. BIA
or DEXA may provide more accurate data for assessing changes in
body composition [39].
7.3. Strengths and limitations
This review had a wide-ranging search strategy with no date
limitations and included a broad range of study designs, with
the quality of trials evaluated using established tools. There were
a large number of participants, albeit a heterogeneous popula-
tion. There was a broad range of PA measurement tools used
in the included studies. Comparisons are therefore difficult, par-
ticularly considering there was a large variability in outcomes
(METs/Steps/Total PA time). The lack of a consistent variable meant
that pooled effects of analysis was not possible. Furthermore, the
validity and reliability of some outcome measures were not es-
tablished. It is unclear if some of the included studies accounted
for hospitalisations/exacerbations which may impact on patient re-
sponse. Therefore, regular monitoring of PA throughout the year
accounting for these events would be an effective strategy to over-
come this issue [49].
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Journal of Cystic Fibrosis 21 (2022) 272–281
7.4. Clinical application
Aerobic training and activity counselling were the two elements
identified in this review which most consistently improved PA.
While supervised exercise sessions may be provided in some cen-
tres, the infection control guidelines in CF mean that PWCF are ex-
cluded from the group based interventions such as the traditional
pulmonary rehabilitation programmes [43]. The evolution of tele-
health approaches in PWCF may assist in overcoming these barri-
ers associated with infection control, while also reducing the bur-
den of travelling for exercise sessions [55]. Further research how-
ever is required to evaluate the use of telehealth interventions for
PA prescription. The variability of the study designs, interventions
and participants limits the clinical applicability of the findings.
Nevertheless, positive trends were seen for improved PA partici-
pation despite heterogenous studies.
7.5. Future research directions
There is a need for high-quality RCTs with sufficient study par-
ticipants and objectively measurable, reproducible, and sensitive
primary PA outcome measures. The optimal training components
(e.g. type, frequency, intensity, duration) to increase PA require fur-
ther research and investigation. Furthermore, future studies might
consider the effect of interventions on subgroups of PWCF, such as
division by age, gender or disease severity. Consideration should
also be given to the potential effect that CFTR modulators may
have on several health outcomes in PWCF, including PA and aer-
obic capacity.
8. Conclusion
This review indicates that exercise interventions can increase PA
participation in PWCF. Some of the effective interventions included
aerobic training and activity counselling, however, the exact com-
ponents of effective interventions are yet to be determined. PA can
be increased in short- and long- term interventions. The interven-
tions were variable across the included studies, with a large incon-
sistency in PA assessment tools. Future research should consider
larger sample sizes and the use of valid, reliable instruments to as-
sess and track PA levels over a longer period of time. Furthermore,
consideration should be given to telehealth interventions or alter-
native strategies that are effective in optimising PA in other patient
populations or should consider subgroups of CF such as division by
gender, age or severity of disease.
Declaration of Competing Interest
The authors declares that there is no conflict of interest.
Supplementary materials
Supplementary material associated with this article can be
found, in the online version, at doi:10.1016/j.jcf.2021.10.008.
References
[1] Armstrong M, Vogiatzis I. Personalized exercise training in chronic lung dis-
eases. Respirology 2019;24(9):854–62.
[2] Armstrong M, Winnard A, Chynkiamis N, Boyle S, Burtin C, Vogiatzis I. Use
of pedometers as a tool to promote daily physical activity levels in pa-
tients with COPD: a systematic review and meta-analysis. Eur Respiratory Rev
2019;28(154).
[3] Beaudoin N, Bouvet GF, Coriati A, Rabasa-Lhoret R, Berthiaume Y. Combined
exercise training improves glycemic control in adult with cystic fibrosis. Med
Sci Sports Exerc 2017;49(2):231–7.
[4] Bernard RS, Cohen LL, Moffett K. A token economy for exercise adher-
ence in pediatric cystic fibrosis: a single-subject analysis. J Pediatr Psychol
2009;34(4):354–65.
M. Curran, AC. Tierney, B. Button et al.
[5] Bort-Roig J, Gilson ND, Puig-Ribera A, Contreras RS, Trost SG. Measuring and
influencing physical activity with smartphone technology: a systematic review.
Sports Med 2014;44(5):671–86.
[6] Bradley JM, Kent L, Elborn JS, O’Neill B. Motion sensors for monitoring phys-
ical activity in cystic fibrosis: what is the next step? Phys Therapy Rev
2010;15(3):197–203.
[7] Burtin C, Hebestreit H. Rehabilitation in patients with chronic respiratory dis-
ease other than chronic obstructive pulmonary disease: exercise and physical
activity interventions in cystic fibrosis and non-cystic fibrosis bronchiectasis.
Respiration 2015;89(3):181–9.
[8] Caspersen CJ, Powell KE, Christenson GM. Physical activity, exercise, and phys-
ical fitness: definitions and distinctions for health-related research. Public
Health Rep 1985;100(2):126–31.
[9] Castellani C, Duff AJ, Bell SC, Heijerman HG, Munck A, Ratjen F, Sermet–
Gaudelus I, Southern KW, Barben J, Flume PA. ECFS best practice guidelines:
the 2018 revision. J Cyst Fibros 2018.
[10] Castro CM, King AC. Telephone-assisted counseling for physical activity. Exerc
Sport Sci Rev 2002;30(2):64–8.
[11] Coelho CM, Reboredo MM, Valle FM, Malaguti C, Campos LA, Nascimento LM,
Carvalho EV, Oliveira JCA, Pinheiro BV. Effects of an unsupervised pe-
dometer-based physical activity program on daily steps of adults with
moderate to severe asthma: a randomized controlled trial. J Sports Sci
2018;36(10):1186–93.
[12] Conway S, Balfour-Lynn IM, De Rijcke K, Drevinek P, Foweraker J, Havermans T,
Heijerman H, Lannefors L, Lindblad A, Macek M. European cystic fibrosis soci-
ety standards of care: framework for the cystic fibrosis centre. J Cyst Fibros
2014;13:S3–S22.
[13] Cox NS, Alison JA, Button BM, Wilson JW, Holland AE. Feasibility and accept-
ability of an internet-based program to promote physical activity in adults
with cystic fibrosis. Respir Care 2015;60(3):422–9.
[14] Cox NS, Alison JA, Button BM, Wilson JW, Morton JM, Holland AE. Physical
activity participation by adults with cystic fibrosis: an observational study.
Respirology 2016;21(3):511–18.
[15] Cox NS, Alison JA, Holland AE. Interventions for promoting physical activity in
people with cystic fibrosis. Cochrane Database Systmat Rev 2013(12).
[16] Cox NS, Alison JA, Rasekaba T, Holland AE. Telehealth in cystic fibrosis: a sys-
tematic review. J Telemed Telecare 2012;18(2):72–8.
[17] Cystic Fibrosis Foundation (2019) ’Patient Registry: Annual Data Report’. Avail-
able at: https://www.cff.org/Research/Researcher-Resources/Patient-Registry/
2019- Patient- Registry- Annual- Data- Report.pdf (Accessed 2020).
[18] Dowman L, Hill CJ, Holland AE. Pulmonary rehabilitation for interstitial lung
disease. Cochrane Database Systmat Rev 2014(10).
[19] Downs SH, Black N. The feasibility of creating a checklist for the assessment
of the methodological quality both of randomised and non-randomised studies
of health care interventions. J Epidemiol Commun Health 1998;52(6):377–84.
[20] Foster C, Hillsdon M, Thorogood M, Kaur A, Wedatilake T. Interventions for
promoting physical activity. Cochrane Database Systemat Rev 2005(1).
[21] Gruber W, Orenstein DM, Braumann KM, Beneke R. Interval exercise training
in cystic fibrosis—effects on exercise capacity in severely affected adults’. J Cyst
Fibros 2014;13(1):86–91.
[22] Gupta S, Mukherjee A, Lodha R, Kabra M, Deepak KK, Khadgawat R, Talwar A,
Kabra SK. Effects of exercise intervention program on bone mineral accretion
in children and adolescents with cystic fibrosis: a randomized controlled trial.
IndJ Pediatrics 2019;86(11):987–94.
[23] Hebestreit H, Kieser S, Junge S, Ballmann M, Hebestreit A, Schindler C,
Schenk T, Posselt H, Kriemler S. Long-term effects of a partially supervised
conditioning programme in cystic fibrosis. Eur Respir J 2010;35(3):578–83.
[24] Hebestreit H, Kieser S, Rüdiger S, Schenk T, Junge S, Hebestreit A, Ballmann M,
Posselt H-G, Kriemler S. Physical activity is independently related to aerobic
capacity in cystic fibrosis. Eur Respir J 2006;28(4):734–9.
[25] Hebestreit H, Kriemler S, Radtke T. Exercise for all cystic fibrosis patients: is
the evidence strengthening? Curr Opin Pulm Med 2015;21(6):591–5.
[26] Hommerding PX, Baptista RR, Makarewicz GT, Schindel CS, Donadio MV,
Pinto LA, Marostica PJ. Effects of an educational intervention of physical activ-
ity for children and adolescents with cystic fibrosis: a randomized controlled
trial. Respir Care 2015;60(1):81–7.
[27] Hospes G, Bossenbroek L, ten Hacken NH, van Hengel P, de Greef MH. En-
hancement of daily physical activity increases physical fitness of outclinic
COPD patients: results of an exercise counseling program. Patient Educ Couns
2009;75(2):274–8.
[28] Klijn PH, Oudshoorn A, van der Ent CK, van der Net J, Kimpen JL, Helders PJ.
Effects of anaerobic training in children with cystic fibrosis: a randomized con-
trolled study. Chest 2004;125(4):1299–305.
[29] Kriemler S, Kieser S, Junge S, Ballmann M, Hebestreit A, Schindler C, Stüssi C,
Hebestreit H. Effect of supervised training on FEV1 in cystic fibrosis: a ran-
domised controlled trial. J Cyst Fibros 2013;12(6):714–20.
[30] McCarthy B, Casey D, Devane D, Murphy K, Murphy E, Lacasse Y. Pulmonary
rehabilitation for chronic obstructive pulmonary disease. Cochrane Database
Systemat Rev 2015(2).
[31] Moher D, Shamseer L, Clarke M, Ghersi D, Liberati A, Petticrew M, Shekelle P,
Stewart LA. Preferred reporting items for systematic review and meta-analysis
protocols (PRISMA-P) 2015 statement. Systemat Rev 2015;4(1):1.
281
Journal of Cystic Fibrosis 21 (2022) 272–281
[32] Moola FJ, Garcia E, Huynh E, Henry L, Penfound S, Consunji-Araneta R,
Faulkner GE. Physical Activity Counseling for Children With Cystic Fibrosis.
Respir Care 2017;62(11):1466–73.
[33] Moran A, Brunzell C, Cohen RC, Katz M, Marshall BC, Onady G, Robin-
son KA, Sabadosa KA, Stecenko A, Slovis B. Clinical care guidelines for
cystic fibrosis–related diabetes: a position statement of the American Di-
abetes Association and a clinical practice guideline of the Cystic Fibro-
sis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care
2010;33(12):2697–708.
[34] O’Connor SR, Tully MA, Ryan B, Bradley JM, Baxter GD, McDonough SM. Failure
of a numerical quality assessment scale to identify potential risk of bias in a
systematic review: a comparison study. BMC Research Notes 2015;8(1):1–7.
[35] Orenstein DM, Winnie GB, Altman H. Cystic fibrosis: a 2002 update. J Pediatr
2002;140(2):156–64.
[36] Paranjape SM, Barnes LA, Carson KA, von Berg K, Loosen H, Mogayzel PJ Jr.
Exercise improves lung function and habitual activity in children with cystic
fibrosis. J Cyst Fibros 2012;11(1):18–23.
[37] Pescatello LS, Riebe D, Thompson PD. ACSM’s guidelines for exercise testing
and prescription. Lippincott Williams & Wilkins; 2014.
[38] Pianosi P, Leblanc J, Almudevar A. Peak oxygen uptake and mortality in chil-
dren with cystic fibrosis. Thorax 2005;60(1):50–4.
[39] Prévotat A, Godin J, Bernard H, Perez T, Le Rouzic O, Wallaert B. Improvement
in body composition following a supervised exercise-training program of adult
patients with cystic fibrosis. Respiratory Med Res 2019;75:5–9.
[40] Prince SA, Adamo KB, Hamel ME, Hardt J, Gorber SC, Tremblay M. A com-
parison of direct versus self-report measures for assessing physical activity in
adults: a systematic review. Int J Behav Nutr PhysActivity 2008;5(1):56.
[41] Qiu S, Cai X, Wang X, He C, Zügel M, Steinacker JM, Schumann U. Using step
counters to promote physical activity and exercise capacity in patients with
chronic obstructive pulmonary disease: a meta-analysis. Therapeutic Adv Res-
piratory Dis 2018;12:1753466618787386.
[42] Sabharwal S. Gastrointestinal manifestations of cystic fibrosis. Gastroenterol
Hepatol 2016;12(1):43.
[43] Saiman L, Siegel JD, LiPuma JJ, Brown RF, Bryson EA, Chambers MJ, Downer VS,
Fliege J, Hazle LA, Jain M. Infection prevention and control guideline for cystic
fibrosis: 2013 update. Infect Control Hospital Epidemiol 2014;35(S1):s1–s67.
[44] Savi D, Schiavetto S, Simmonds NJ, Righelli D, Palange P. Effects of
Lumacaftor/Ivacaftor on physical activity and exercise tolerance in three adults
with cystic fibrosis. J Cyst Fibros 2019;18(3):420–4.
[45] Sawyer MG, Reynolds KE, Couper JJ, French DJ, Kennedy D, Martin J, Stau-
gas R, Ziaian T, Baghurst PA. Health-related quality of life of children and
adolescents with chronic illness–a two year prospective study’. Qual Life Res
2004;13(7):1309–19.
[46] Schneiderman-Walker J, Pollock SL, Corey M, Wilkes DD, Canny GJ, Pedder L,
Reisman JJ. A randomized controlled trial of a 3-year home exercise program
in cystic fibrosis. J Pediatr 20 0 0;136(3):304–10.
[47] Schneiderman JE, Wilkes DL, Atenafu EG, Nguyen T, Wells GD, Alarie N,
Tullis E, Lands LC, Coates AL, Corey M, Ratjen F. Longitudinal relationship be-
tween physical activity and lung health in patients with cystic fibrosis. Eur
Respir J 2014;43(3):817–23.
[48] Selvadurai HC, Blimkie C, Meyers N, Mellis C, Cooper P, Van Asperen P. Ran-
domized controlled study of in-hospital exercise training programs in children
with cystic fibrosis. Pediatr Pulmonol 2002;33(3):194–200.
[49] Shelley J, Boddy LM, Knowles ZR, Stewart CE, Dawson EA. Physical activity and
associations with clinical outcome measures in adults with cystic fibrosis; a
systematic review. J Cyst Fibros 2019;15(5):590–601.
[50] Swisher AK. Not just a lung disease: peripheral muscle abnormalities in cystic
fibrosis and the role of exercise to address them. Cardiopulmonary Physical
Therapy J 2006;17(1):9.
[51] Swisher AK, Moffett K. The effect of coaching on physical activity and quality
of life in children and adolescents with cystic fibrosis: a quality improvement
pilot study. Int J Allied Health Sci Practice 2010;8(2):8.
[52] Szczesniak R, Heltshe SL, Stanojevic S, Mayer-Hamblett N. Use of FEV1 in cystic
fibrosis epidemiologic studies and clinical trials: a statistical perspective for
the clinical researcher. J Cyst Fibros 2017;16(3):318–26.
[53] Tomlinson OW, Shelley J, Trott J, Bowhay B, Chauhan R, Sheldon CD. The feasi-
bility of online video calling to engage patients with cystic fibrosis in exercise
training. J Telemed Telecare 2020;26(6):356–64.
[54] Tuzin BJ, Mulvihill MM, Kilbourn KM, Bertran DA, Buono M, Hovell MF,
Harwood IR, Light MJ. Increasing physical activity of children with cys-
tic fibrosis: A home-based family intervention. Pediatric Exercise Science
1998;10(1):57–68.
[55] Ward N, Stiller K, Holland AE. Exercise as a therapeutic intervention for people
with cystic fibrosis. Expert Rev Respiratory Med 2019;13(5):449–58.
[56] Wark PA, Cookson K, Thiruchelvam T, Brannan J, Dorahy DJ.
Lumacaftor/ivacaftor improves exercise tolerance in patients with cystic
fibrosis and severe airflow obstruction. BMC Pulmonary Med 2019;19(1):1–8.
[57] Weinstein AA, Chin LM, Keyser RE, Kennedy M, Nathan SD, Woolstenhulme JG,
Connors G, Chan L. Effect of aerobic exercise training on fatigue and phys-
ical activity in patients with pulmonary arterial hypertension. Respir Med
2013;107(5):778–84.