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Interventions for promoting physical activity in people with cystic fibrosis

  1. Narelle S Cox1,*,
  2. Jennifer A Alison2,
  3. Anne E Holland1

Editorial Group: Cochrane Cystic Fibrosis and Genetic Disorders Group

Published Online: 13 DEC 2013

Assessed as up-to-date: 5 DEC 2013

DOI: 10.1002/14651858.CD009448.pub2


How to Cite

Cox NS, Alison JA, Holland AE. Interventions for promoting physical activity in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2013, Issue 12. Art. No.: CD009448. DOI: 10.1002/14651858.CD009448.pub2.

Author Information

  1. 1

    La Trobe University, School of Physiotherapy, Melbourne, Victoria, Australia

  2. 2

    The University of Sydney, Clinical and Rehabilitation Sciences, Faculty of Health Sciences, Lidcombe, Australia

*Narelle S Cox, School of Physiotherapy, La Trobe University, Level 4 The Alfred Centre 99 Commercial Road, Melbourne, Victoria, 3004, Australia. nscox@students.latrobe.edu.au.

Publication History

  1. Publication Status: New
  2. Published Online: 13 DEC 2013

SEARCH

 

Background

  1. Top of page
  2. Background
  3. Objectives
  4. Methods
  5. Results
  6. Discussion
  7. Authors' conclusions
  8. Acknowledgements
  9. Data and analyses
  10. Appendices
  11. Contributions of authors
  12. Declarations of interest
  13. Sources of support
  14. Differences between protocol and review
  15. Index terms

For a glossary of terms please see the appendices (Appendix 1).

 

Description of the condition

Cystic fibrosis (CF) is a progressive, life-limiting, multi-system disease that predominantly affects children and young adults. The lungs of people with CF are affected by bronchiectasis progressing to respiratory failure (Koch 1993). Participation in regular physical activity and exercise is encouraged in CF (Yankaskas 2004) since exercise participation affords health benefits including enhanced clearance of pulmonary secretions (Bradley 2008; Dwyer 2011), improved blood glucose control and bone mineral accretion (Bradley 2008).

 

Description of the intervention

Physical activity refers to any bodily movement that results in an increase in energy expenditure above baseline resting energy expenditure (Casparen 1985). Physical activity and exercise are terms commonly used interchangeably; however, a subtle distinction exists between the two. Physical activity includes structured exercise and sport activities, as well as activities involved in play, work, transportation, chores and recreational pursuits (WHO 2010). Habitual physical activity implies the performance of physical activity within the context of regular daily life (Clanchy 2011). Physical activity participation can be quantified in a number of ways which can include recording the number of steps taken in a day, the amount of time spent in moderate intensity activity, daily energy expenditure or self-reported activity participation using a diary or questionnaire. Participation in regular physical activity for the general population has a number of health benefits, including reducing the risk of cardiovascular disease, improving glycaemic control, and helping overcome obesity (Haskell 2007; Thompson 2003). In addition, physical activity participation can improve mental health and quality of life (WHO 2010).

Despite the benefits of regular physical activity participation in the general population, only one quarter of adults in the USA report participating in 30 minutes of activity of at least moderate intensity on five or more days of the week (Kahn 2002). Furthermore, the 2012 Australian Health Survey reported participation in moderate-vigorous physical activity during the preceding week by only 30% of people older than 15 years of age (ABS 2012). Consequently, strategies to promote physical activity participation are of great importance. Physical activity promotion strategies can take a number of forms and may include mass-media campaigns for health behaviour change, point-of decision prompts to encourage increased activity or individually tailored interventions (CDC 2011).

Physical activity may also have specific health benefits for people with particular health disorders. In people with chronic illness, the ability to participate in regular physical activity can be hampered by factors such as the environment (natural and built), financial cost and emotional and psychological barriers (Rimmer 2004). These barriers contribute to people with chronic illness being less likely to report participation in moderate and vigorous physical activity compared to healthy populations (Marcus 2000). In some respiratory populations supervised exercise training have been used to change behaviour and result in increased participation in physical activity. Some examples of these are pulmonary rehabilitation, educational or motivational sessions (Conn 2008); walking programmes using pedometer feedback and Internet-based monitoring (Moy 2012); and text-messaging support (Nguyen 2009).

The effect of strategies to improve physical activity participation in people with CF has not previously been reviewed. For people with CF, healthcare professionals generally regard participation in physical activity as beneficial (Bradley 2008). The nature of treatments that can promote physical activity participation for people with CF may be quite variable, and could include such interventions as exercise training (supervised or unsupervised), reminders, counselling or education. Identifying the most effective strategies to promote increased participation in physical activity could therefore have long-term health effects. As well as increases in physical activity, such health improvements may be reflected as increases in exercise capacity, respiratory function and quality of life, all of which are associated with improved prognostic outcomes in CF (Hebestreit 2006; Schneiderman-Walker 2005; Troosters 2009).

 

How the intervention might work

Strategies likely to be successful in promoting increased physical activity participation are those which provide education regarding the health benefits of physical activity (Pate 1995), provide physical skills practice (Andersen 1998) and overcome perceived barriers by fostering enjoyment of activity and providing motivation (Pate 1995).

 

Why it is important to do this review

In an earlier Cochrane Review targeting previously sedentary healthy adults and excluding those who had pre-existing medical conditions, it was reported that professional advice with continued support could encourage short-term improvement in participation in physical activity, with more research required to identify the best long-term strategies (Foster 2005). GIven that the population included in that review were healthy, it is unknown whether the interventions identified which target behaviour adaptation or involve strategies for delivering physical activity programmes, can be applied to people with CF.

Previous reviews in CF have focused on exercise participation and measures of exercise or aerobic capacity (Bradley 2008). This review will enable a comparison of the relative efficacy of strategies that promote participation in physical activity, and thus allow clinicians to identify treatment options suited to encouraging increased participation in physical activity for people with CF. In addition, this review may identify avenues for future research into novel strategies, that encourage increased participation in physical activity whilst maintaining the strict infection control procedures, which are mandatory to prevent cross infection in people with CF (Saiman 2004).

 

Objectives

  1. Top of page
  2. Background
  3. Objectives
  4. Methods
  5. Results
  6. Discussion
  7. Authors' conclusions
  8. Acknowledgements
  9. Data and analyses
  10. Appendices
  11. Contributions of authors
  12. Declarations of interest
  13. Sources of support
  14. Differences between protocol and review
  15. Index terms

To evaluate the effect of treatment to increase physical activity participation in people with CF.

 

Methods

  1. Top of page
  2. Background
  3. Objectives
  4. Methods
  5. Results
  6. Discussion
  7. Authors' conclusions
  8. Acknowledgements
  9. Data and analyses
  10. Appendices
  11. Contributions of authors
  12. Declarations of interest
  13. Sources of support
  14. Differences between protocol and review
  15. Index terms
 

Criteria for considering studies for this review

 

Types of studies

Randomised and quasi-randomised controlled studies.

 

Types of participants

People with CF aged over five years, with any degree of disease severity. Diagnosis of CF confirmed by clinical criteria and sweat testing or genotype analysis.

 

Types of interventions

We included all strategies which could be considered to promote increased participation in daily physical activity for individuals with CF (refer to examples below), including interventions in both inpatient and outpatient settings. Where the study intervention commenced in the inpatient setting, the study needed to include follow up in the discharge period in order to evaluate the effects of the intervention on physical activity in daily life.

Potential interventions include:

  • one-off one-to-one counselling or advice;
  • self-directed or unsupervised participation in a prescribed physical activity programme;
  • supervised physical activity session in the home;
  • supervised physical activity session in a facility;
  • on-going face-to-face counselling or advice;
  • telephone support;
  • written material;
  • Internet-based or tele-health advice and motivation;
  • monitoring device for motivation, e.g. pedometer

Specific comparisons examined were:

  1. one or more interventions to promote physical activity versus no intervention;
  2. one or more interventions to promote physical activity versus a placebo intervention (e.g. attention control);
  3. one intervention to promote physical activity compared to another intervention to promote physical activity.

We also included studies whose goal was to assess physiological outcomes as a response to a physical exercise intervention, but which included physical activity participation as part of that assessment.

 

Types of outcome measures

 

Primary outcomes

  1. Participation in physical activity (change from baseline where possible, measured either subjectively, e.g. by an activity diary, or objectively using a monitoring device e.g. a pedometer)
    1. intensity of physical activity (measured or estimated using objective measures e.g. activity monitoring, or self-report of physical activity e.g. International Physical Activity Questionnaire (IPAQ)
    2. time spent in physical activity (measured in minutes per week, sessions per week, etc)
    3. energy expenditure (in calories or joules)
    4. step count (using a monitoring device such as a pedometer)
  2. Health-related quality of life measured by generic or disease specific assessments, or both

 

Secondary outcomes

  1. Exercise capacity (either maximal or submaximal where measured directly or by a standard field test)
  2. Pulmonary function tests (change in per cent (%) predicted or absolute measures from baseline, or rate of decline)
    1. forced expiratory volume in one second (FEV1)
    2. forced vital capacity (FVC)
    3. forced expiratory flows between 25% and 75% of expired volume (FEF25-75)
  3. Adverse events (e.g. musculoskeletal injuries)
  4. Body composition in terms of body mass index (BMI) and lean body mass
  5. Bone mineral density (defined on dual energy X-ray absorptiometry (DXA) scans)
  6. Adherence to the intervention programme
  7. Compliance with other CF treatments, e.g. airway clearance techniques and nebulised medication; any measure of compliance such as pill counts, self-report diaries, electronic monitoring
  8. Cost evaluation

 

Search methods for identification of studies

 

Electronic searches

We searched for relevant trials from the Group's Cystic Fibrosis Trials Register using the terms: physiotherapy AND exercise.

The Cystic Fibrosis Trials Register is compiled from electronic searches of the Cochrane Central Register of Controlled Trials (CENTRAL) (updated each new issue of The Cochrane Library), quarterly searches of MEDLINE, a search of Embase to 1995 and the prospective handsearching of two journals - Pediatric Pulmonology and the Journal of Cystic Fibrosis. Unpublished work is identified by searching the abstract books of three major cystic fibrosis conferences: the International Cystic Fibrosis Conference; the European Cystic Fibrosis Conference and the North American Cystic Fibrosis Conference. For full details of all searching activities for the register, please see the relevant sections of the Cochrane Cystic Fibrosis and Genetic Disorders Group Module

Date of the search of the Group's Cystic Fibrosis Trials Register: 05 December 2013.

In addition, we searched MEDLINE, CINAHL (Ebscohost), PsycINFO (OvidSP) and the Physiotherapy Evidence Database (PEDro) for all years available (Appendix 2). We searched for potentially relevant, completed but unpublished studies, by searching the clinical trials registers listed below using the search terms 'physical activity' or 'physical fitness' or 'habitual activity' and 'cystic fibrosis' and 'promotion' or 'education' or 'uptake' or 'encourage' or 'increase' or 'start'.

Date of the search of CINAHL, PsychINFO, PEDro and clinical trials registers: 10 September 2012.

 

Searching other resources

We reviewed the reference list of all included studies for any additional trials suitable for inclusion. 

 

Data collection and analysis

 

Selection of studies

Two authors (NC and AH) independently assessed the titles and abstracts of trials identified by the searches. The same two authors assessed full-text copies of potentially relevant trials for inclusion based upon the defined criteria. Each author compiled a list of studies that they believed met the inclusion criteria. The authors compared these lists and resolved any disagreements that occurred by discussion and consensus, with arbitration by the third author (JA) as necessary.

 

Data extraction and management

Two authors (NC and AH) independently extracted data using specially developed, standardised data extraction forms. One author (NC) entered the data into the Cochrane software Review Manager (RevMan 2011) and a second author (AH) reviewed it. They resolved any disagreements by discussion between review authors and, if necessary arbitration by the third author (JA).

We have reported data by type of intervention (supervised exercise training or unsupervised exercise training), and based on the time-point at which follow-up data were reported. We categorised these time-points as:

  1. less than or equal to one month (short-term);
  2. from one to six months; and
  3. greater than six months.

 

Assessment of risk of bias in included studies

Two authors (NC and AH) independently rated the risk of bias of the reviewed studies using the standardised grading system described in the Cochrane Handbook of Systematic Reviews of Interventions (Higgins 2011).  We assessed the following domains as having either a 'high', 'low' or 'unclear' risk of bias:

  1. random sequence generation;
  2. allocation concealment;
  3. blinding (of participants, personnel and outcome assessors);
  4. incomplete outcome data sets;
  5. selective outcome reporting;
  6. other sources of bias.

The authors resolved any disagreements by discussion to reach a consensus.

 

Measures of treatment effect

Where possible, the authors expressed the treatment effect for continuous outcomes in their original metrics using mean differences (MD) and 95% confidence intervals (CI). If trials had measured continuous outcomes using different scales, the authors planned to express the treatment effect as a standardised mean difference (SMD) with 95% CI. We report results for dichotomous data as risk ratios (RR) with 95% CIs.

If there had been more than one study with a specific intervention reporting outcomes at the same time-point, we had intended to perform a meta-analysis using the Cochrane statistical package RevMan, however, such data were not available (RevMan 2011).

 

Unit of analysis issues

Cross-over studies were not included in the review. If future versions of this review include cross-over studies, we will use the generic inverse variance method for the analysis of data.

 

Dealing with missing data

The review authors contacted the investigators of the studies included in this review for additional information where necessary.

In order to present MD and 95% CI data from the study by Hebestreit, the authors took the MD between groups from the results published in table 2 of the manuscript (Hebestreit 2010), and we calculated the 95% CI by multiplying the standard error (SE) by 1.96. We applied this method for all MD and 95% CI data presented pertaining to this paper.

 

Assessment of heterogeneity

The authors assessed clinical heterogeneity and intended to conduct meta-analyses where they agreed that the trials were sufficiently clinically homogenous in terms of study population, interventions and outcomes. However, we were not able to combine any studies in a meta-analysis; if we are able to do this in future updates, we will describe any heterogeneity between the included studies using the standard chi2 test and assess the impact of any heterogeneity on the meta-analysis using the I2 statistic (Higgins 2011): I2 = [Q-df/Q] x 100%, where Q is the chi2 statistic and df is its degrees of freedom (Higgins 2011). This describes the percentage of the variability in effect estimates that is due to heterogeneity rather than sampling error (chance). We will use the following guide to interpret the I2 values:

  • 0% to 40% might not be important;
  • 30% to 60% may represent moderate heterogeneity;
  • 50% to 90% may represent substantial heterogeneity;
  • 75% to 100% considerable heterogeneity.

 

Assessment of reporting biases

We sought to reduce reporting bias through the following measures:

  • we performed comprehensive searches to identify randomised controlled studies;
  • we attempted to seek out and include relevant unpublished studies;
  • we had planned to identify reporting biases using funnel plots to assess for small study effects (Higgins 2011), however this was precluded by the small number of included studies. 

 

Data synthesis

If meta-analyses are possible in future updates, we will use a fixed-effect model, unless there is substantial heterogeneity (i.e. over 50%) (Higgins 2011), in which case we will use a random-effects model. For outcomes where there were insufficient data, or a high degree of heterogeneity, we did not perform a meta-analysis and instead presented a narrative synthesis.

 

Subgroup analysis and investigation of heterogeneity

We intended to undertake the following subgroup analyses:

  1. children versus adults;
  2. supervised versus unsupervised interventions. 

However, the small number of included trials precluded these subgroup analyses. We will undertake these analyses in future updates of this review if sufficient numbers of studies are available.

 

Sensitivity analysis

If a sufficient number of studies are available in future updates, we will perform a sensitivity analysis by including only studies with an overall low risk of bias. If all studies are found to have a high risk of bias, we will perform a sensitivity analysis after the exclusion of studies that did not conceal allocation. 

 

Results

  1. Top of page
  2. Background
  3. Objectives
  4. Methods
  5. Results
  6. Discussion
  7. Authors' conclusions
  8. Acknowledgements
  9. Data and analyses
  10. Appendices
  11. Contributions of authors
  12. Declarations of interest
  13. Sources of support
  14. Differences between protocol and review
  15. Index terms
 

Description of studies

 

Results of the search

Electronic searches retrieved a total of 567 citations. After the removal of duplicates and screening of records, 10 full-text reports of studies and three abstracts were reviewed. An additional four potential references were identified from the reference lists of the full-text studies. Of the total 14 full-text studies reviewed, four studies met the criteria for inclusion and 10 were excluded. Of the three published abstracts, one study was excluded and two are awaiting classification and will be assessed for eligibility in a future update if further information becomes available (Alarie 2013; Marostica 2012). Please refer to the figures for the PRISMA diagram of study selection (Figure 1).

 FigureFigure 1. Study flow diagram.

 

Included studies

 

Trial Characteristics

All four included studies were randomised controlled studies. One study was conducted in the inpatient setting with follow up in the outpatient setting (Selvadurai 2002). The remaining three studies were conducted in people with CF with stable respiratory disease in the outpatient setting (Hebestreit 2010; Klijn 2004; Schneiderman-Walker 2000).

The duration of the intervention period of the included studies ranged from 18 days (Selvadurai 2002) to three years (Schneiderman-Walker 2000); with follow-up periods of one month (Selvadurai 2002), three months (Klijn 2004), two years (Hebestreit 2010) and three years (Schneiderman-Walker 2000).

 

Participants

A total of 199 individuals with CF participated in the four included studies. The number of participants recruited in each study ranged from 23 (Klijn 2004) to 72 (Schneiderman-Walker 2000). Two studies reported the number of male versus female participants, 28 male versus 38 female (Selvadurai 2002), and 19 male versus 19 female (Hebestreit 2010). Two studies did not report the breakdown of participant gender (Klijn 2004; Schneiderman-Walker 2000). The mean age of participants ranged from 13.2 years (Selvadurai 2002) to 19.5 years (Hebestreit 2010). Mean FEV1 % predicted ranged from 57% predicted (Selvadurai 2002) to 89% predicted (Schneiderman-Walker 2000). Three studies were conducted solely in paediatric patients (Klijn 2004; Schneiderman-Walker 2000; Selvadurai 2002), with one study being undertaken in a combined group of paediatric and adult patients (Hebestreit 2010).

All studies reported no significant differences in baseline characteristics between the intervention and control group in terms of measured parameters including pulmonary function, weight, height and body mass.

 

Interventions

All included studies used exercise interventions to promote physical activity. The specific interventions assessed in the studies were anaerobic training (Klijn 2004), predominantly aerobic training (Schneiderman-Walker 2000) or a combination of aerobic training and resistance training (Hebestreit 2010; Selvadurai 2002). Two studies used supervised, prescribed exercise strategies (Klijn 2004; Selvadurai 2002), the remaining two studies provided activity counselling and exercise or activity prescription planning at baseline with telephone and clinic consultation throughout the intervention period (Hebestreit 2010; Schneiderman-Walker 2000).

 

Outcomes

Participation in physical activity and exercise capacity were reported in all studies. Quality of life (QOL) was reported by three studies, all using different methods. Selvadurai used the 'Quality of Well-being scale' in hospitalised children undertaking supervised training (Selvadurai 2002); Klijn used the 'CF Questionnaire (CFQ)' (Klijn 2004); and Hebestreit used the 'Revised German CF questionnaire' (Hebestreit 2010).The 'Revised German CF questionnaire' is a translation of the originally developed CFQ (Schmidt 2009). The CFQ assesses nine domains relating to health-related QOL and three domains relating to symptoms, as well as subjective health perception (Henry 2003; Wenninger 2003). In stable patients a change in score of four points on the respiratory scale of the questionnaire corresponds to the minimum clinically important difference (MCID) (Quittner 2009).

All included studies assessed pulmonary function (FEV1 and FVC), while two studies also assessed FEF25-75 (Klijn 2004; Schneiderman-Walker 2000). Adherence to the intervention and compliance with other CF-related treatments were reported in two studies (Klijn 2004; Schneiderman-Walker 2000). None of the included studies presented a cost analysis of the intervention. Only one study reported the occurrence of adverse events, however it was unclear if these events prevented participants from completing outcome assessments (Selvadurai 2002).

 

Excluded studies

On reviewing the full-text papers, 10 potentially relevant studies were excluded. One additional study, available only in abstract form, was also excluded (Petrovic 2013).

Seven of the excluded studies were not randomised controlled studies (Bernard 2004; Gulmans 1999; Hind 2008; Orenstein 1981; Swisher 2010; Tunzin 1998; van Doorn 2010). One study was only conducted in the inpatient setting, with no outpatient follow up (Kuys 2011). Three studies only assessed physiological outcomes of exercise intervention and not physical activity participation (Moorcroft 2004; Petrovic 2013; Sosa 2012).

 

Risk of bias in included studies

Included studies were rated as low risk of bias for some aspects, and unclear for others. The authors' judgements of risk of bias for each study are outlined in their respective 'Risk of bias' table, and presented in the 'Risk of bias summary table' (Figure 2).

 FigureFigure 2. Risk of bias summary: review authors' judgements about each risk of bias item for each included study.

 

Allocation

Two studies were allocated low risk of bias for random sequence generation having identified the use of "random computer number generation" (Schneiderman-Walker 2000) or "selection of pre-folded papers" indicating group allocation (Hebestreit 2010). Two studies were rated as having an unclear risk of bias as the method of sequence generation was not described (Klijn 2004; Selvadurai 2002).

Three trials had a low risk of selection bias as allocation was concealed by using "concealed envelopes" (Klijn 2004; Selvadurai 2002) or "folded paper in an opaque bag" (Hebestreit 2010). For one study the method of concealment was not stated and it was therefore judged to have an unclear risk of bias (Schneiderman-Walker 2000).

 

Blinding

It was not possible to blind the participants in any included study, nor were caregivers blind to group allocation in any of the included studies. In one study, assessors of pulmonary function were "unaware of each patient's group assignment" (Schneiderman-Walker 2000). However, it is unclear whether assessors of other outcomes were also blind to group allocation. One study reported that "the primary researcher was blinded for the experimental condition", however, it was unclear if this researcher performed outcome assessments (Klijn 2004). In the two remaining studies there was insufficient information presented to determine if outcome assessors were blind to group allocation (Hebestreit 2010; Selvadurai 2002).

Overall, the risk of bias for blinding was unclear, as the extent to which unblinded assessment may have affected outcomes would vary depending upon the outcome; for example, assessor knowledge of participant group allocation could affect the reporting of QOL outcomes, or the amount of encouragement provided may influence the outcome of exercise tests. The influence of unblinded outcome assessors may be less likely to affect objective measures of physical activity such as activity monitoring.

 

Incomplete outcome data

Two studies were rated as having a low risk of bias for incomplete outcome data (Klijn 2004; Selvadurai 2002). One study was rated as having a high risk (Hebestreit 2010) and one as having an unclear risk of bias (Schneiderman-Walker 2000). All studies did report dropouts, with the proportion of dropouts at the final data-collection time-point ranging from 0% (Selvadurai 2002) to 26% (Hebestreit 2010). Selvadurai reported that two participants were unable to complete all aspects of the intervention programme; however, it is presumed these participants were still able to complete outcome assessments as the paper identifies "no children withdrew from the study" (Selvadurai 2002). One study stated that an intention-to-treat analysis was performed; however, only the results of those participants who had at least two years of follow up were presented (Schneiderman-Walker 2000). In this study seven participants dropped out prior to the two-year follow-up assessment; the authors stated that an intention-to-treat analysis revealed similar results to those reported. At two-year follow up, there was a 26% dropout in the study by Hebestreit, with no statement of analysis by intention-to-treat (Hebestreit 2010). There were three dropouts reported in the Klijn study (n = 23) (Klijn 2004).

 

Selective reporting

Overall, included studies were judged as having unclear risk of reporting bias as there was insufficient information to determine the effect of the slight variations in reporting evidence.

 

Other potential sources of bias

Other potential sources of bias were identified in three studies (Hebestreit 2010; Klijn 2004; Selvadurai 2002). In the supervised anaerobic training study, the discussion suggests participants were instructed not to alter their physical activity participation outside of supervised training, however, the nature of this instruction was not outlined in the methods (Klijn 2004). In a study of activity counselling and exercise advice compared to control, logistic and financial support (for example gym fees) was offered if needed, to enable participation in the intervention programme (Hebestreit 2010). In one study, participation in physical activity as assessed using accelerometer was conducted in a subgroup of participants who had previously had their baseline levels of physical activity established as a part of another study (Selvadurai 2002). The inclusion criteria for this alternate study were not stated.

 

Effects of interventions

Four studies have been included in a narrative synthesis. There were no studies included in a quantitative synthesis, as a consequence of the variation in the type and duration of studies, the methods of measuring outcomes and measurement units, the time-points at which outcomes were assessed, and the lack of reported data.

 

Supervised exercise training

Two studies, with a total of 86 participants reported the outcomes of supervised exercise training on participation in physical activity (Klijn 2004; Selvadurai 2002). In one study participants in the intervention group undertook anaerobic training, while control group participants received no intervention and continued with their normal daily activities (Klijn 2004). The second study of supervised exercise training was undertaken in the inpatient environment (Selvadurai 2002). Aerobic or resistance training (or a combination of both) was performed by children in the intervention groups, whilst control group participants received standard chest physiotherapy without any physical training (Selvadurai 2002).

 

Primary outcomes

 
1. Participation in physical activity
 
a. At less than or equal to one month

One study reported the effects of a supervised, inpatient programme of aerobic or resistance exercise on physical activity at one month following hospital discharge (Selvadurai 2002). There was no significant difference in physical activity participation between those who undertook aerobic training in the hospital and a control group who undertook no physical training sessions, MD 1.20 MJ/day (95% CI -0.47 to 2.87 MJ/day). Similarly, there was no significant difference in physical activity participation between those who undertook a resistance training program and the control group with no physical training at one month following discharge, MD 0.65 MJ/day (95% CI -0.86 to 2.16 MJ/day) ( Analysis 1.1).

In one study comparing supervised anaerobic training and a control group who undertook no exercise training, the authors stated that there were no significant changes in physical activity participation for either group at the conclusion of the 12-week training programme (Klijn 2004). No data for activity participation were reported.

 
b. At one month up to six months

For the study comparing supervised anaerobic training and a control group who undertook no exercise training, the authors stated that there were no significant changes in physical activity participation for either group at at three-months follow up (Klijn 2004). No data for activity participation were reported.

 
c. At over six months

No studies of supervised exercise training reported outcomes for physical activity participation at over six months.

 
2. Change in QOL
 
a. At less than or equal to one month

In one study, the supervised, inpatient aerobic training group significantly improved quality of well-being score at one month following discharge compared to a no physical training control group, MD 0.10 points (95% CI 0.03 to 0.17 points) (Selvadurai 2002) ( Analysis 1.2). Inpatient resistance training produced no change in QOL score compared to a control group with no physical training at one month post discharge, MD 0.03 points (95% CI -0.04 to 0.10 points) ( Analysis 1.2).

In the second study, at the completion of a 12-week supervised anaerobic training programme, children in the training group had a mean increase in QOL score for the domain of physical functioning only (Klijn 2004). Physical functioning score changed by a mean 18.1 points in the anaerobic training group, compared to a 3.9 point change in the no intervention control group. A between-group analysis was not presented.

 
b. Over one month and up to six months

No studies of supervised exercise training assessed QOL at one to six months.

 
c. At over six months

No studies of supervised exercise training assessed QOL at over six months.

 

Secondary outcomes

 
1. Change in exercise capacity

Two studies of supervised exercise training assessed exercise capacity. One used a cycle ergometry test (Klijn 2004), with the second study using a treadmill test (Selvadurai 2002).

 
a. At less than or equal to one month

In the Selvadurai study, supervised, inpatient aerobic training resulted in a significant improvement in change in VO2 peak measured on a treadmill compared to no physical training control at both discharge from hospital, MD 8.53 ml/kg/min (95% CI 4.85 to 12.21 ml/kg/min) and one month following discharge, MD 4.91 ml/kg/min (95% CI 1.13 to 8.69 ml/kg/min) (Selvadurai 2002) ( Analysis 1.3). Supervised, inpatient resistance training resulted in no difference in change in VO2 peak relative to the no physical training control group at discharge from hospital, MD 1.95 ml/kg/min (95% CI -1.61 to 5.51 ml/kg/min), or at one month following discharge, MD -0.40 ml/kg/min (95% CI -4.03 to 3.23 ml/kg/min) (Selvadurai 2002) ( Analysis 1.3).

In the Klijn study, at completion of a 12-week supervised anaerobic training programme, the intervention group had a significantly greater improvement in VO2 peak measured by cycle ergometry, when compared to a no physical training control group, MD 2.10 ml/kg/min (95% CI 0.12 to 4.08 ml/kg/min) (Klijn 2004) ( Analysis 1.3).

 
b. Over one month up to six months

In one study, at three-month follow up, a 12-week supervised anaerobic training programme resulted in no significant difference in exercise capacity from baseline for the treatment group (Klijn 2004). No data were reported.

 
c. At over six months

No studies of supervised exercise training reported results for change in exercise capacity at over six months.

 
2. Pulmonary function tests
 
a. Change in FEV1 (% predicted)

Selvadurai reported data for change in FEV1 following supervised training at discharge from hospital (end intervention) and one month following discharge (Selvadurai 2002).

i. At less than or equal to one month

In the Selvadurai study there was no clear benefit seen when comparing supervised aerobic training during hospitalisation to a no training control group at discharge, MD 2.03% (95% CI -2.31 to 6.37%), and at one-month follow up, MD 1.53% (95% CI -2.93 to 5.99%) (Selvadurai 2002) ( Analysis 1.4). However, resistance training during hospitalisation resulted in a significantly greater improvement in FEV1 compared to the no training control group at both discharge from hospital, MD 5.58% (95% CI 1.34 to 9.82%), and one month post discharge, MD 5.08% (95% CI 0.66 to 9.50%) (Selvadurai 2002) ( Analysis 1.4).

In one study, there was no significant change in pulmonary function (including FEV1) at end of intervention for participants undertaking 12-weeks supervised anaerobic training compared to no exercise training control group (Klijn 2004). No data were presented.

ii. Over one month up to six months

In one study, there was no significant change in pulmonary function (including FEV1) at three-months follow up in participants who had completed either a 12-week supervised anaerobic training programme or no exercise training control group (Klijn 2004). No data were presented.

iii. At over six months

No studies of supervised exercise training reported outcomes for change in FEV1 at over six months.

 
b. Change in FVC (% predicted)

i. At less than or equal to one month

There were no changes in FVC when supervised, inpatient aerobic training was compared to a no training control group at either discharge from hospital, MD 0.06% (95% CI -2.55 to 2.67%) or at one month post discharge, MD -0.11%, 95% CI (-2.64 to 2.42%) (Selvadurai 2002) ( Analysis 1.5). Similarly, supervised, inpatient resistance training compared to no training control resulted in no change in FVC at hospital discharge, MD 0.17%, 95% CI (-2.31 to 2.65%) or at one month post discharge, MD 0.06% (95% CI -2.42 to 2.54%) (Selvadurai 2002) ( Analysis 1.5).

In one study there was no significant change in pulmonary function (including FVC) at end of intervention for participants who had undertaken 12-weeks supervised anaerobic training compared to no exercise training control group (Klijn 2004). No data were presented.

ii. Over one month up to six months

In one study, there was no significant change in pulmonary function (including FVC) at three-months follow up in participants who had completed either a 12-week supervised anaerobic training programme or no exercise training control group (Klijn 2004). No data were presented.

iii. At over six months

No studies of supervised exercise training reported outcomes for change in FVC at over six months.

 
c. Change in FEF25-75 (%predicted)

No studies of supervised exercise training reported outcomes for change in FEF25-75.

 
3. Adverse events

No adverse events related to the study interventions were reported. One participant in the Selvadurai study (aerobic training group) suffered a sprained ankle which was reported as unrelated to the training programme (Selvadurai 2002).

 
4. Body composition

Both studies of supervised exercise training programmes reported outcomes for body composition (Klijn 2004; Selvadurai 2002).

 
a. At less than or equal to one month

Fat-free mass in kilograms was reported in two studies (Klijn 2004; Selvadurai 2002). In the Selvadurai study, inpatient aerobic training had no effect on the change in fat-free mass at discharge from hospital compared to a control group with no physical training, MD 0.01 kg (95% CI -0.19 to 0.21 kg) or at one month post discharge, MD 0.04 kg (95% CI -0.19 to 0.27 kg) (Selvadurai 2002) ( Analysis 1.6).In the supervised, inpatient resistance training group the children had a significantly greater improvement in fat-free mass at both discharge, MD 1.80 kg (95% CI 1.57 to 2.03 kg), and at one month after discharge from hospital, MD 1.71 kg (95% CI 1.46 to 1.96 kg), compared to a no physical training control group (Selvadurai 2002) ( Analysis 1.6).

In children undertaking a 12-week supervised anaerobic training programme there was no difference in fat-free mass compared to a no exercise control group at the end of the intervention, MD -0.40 kg (95% CI -1.14 to 0.34 kg) (Klijn 2004) ( Analysis 1.6).

 
b. Over one month up to six months

In children undertaking a 12-week supervised anaerobic training programme there was no difference in fat-free mass compared to a control group with no exercise at three-months follow up, MD 0.30 kg (95% CI -1.27 kg to 1.87 kg) (Klijn 2004) ( Analysis 1.6).

 
c.At over six months

No studies of supervised exercise training reported outcomes for body composition at over six months.

 
5. Change in bone mineral density (defined on dual energy X-ray absorptiometry (DXA) scans)

Bone mineral density was not measured or reported in any of the studies of supervised exercise training.

 
6. Adherence to intervention

One study of supervised exercise training reported on adherence to the intervention (Klijn 2004).

 
a. At less than or equal to one month

Attendance at a supervised, outpatient anaerobic training programme was rated as excellent with a mean attendance rate of 98% of sessions (SD 4%) (Klijn 2004).

 
b. Over one month up to six months

No studies of supervised exercise training reported outcomes for adherence to the intervention at one to six months.

 
c. At over six months

No studies of supervised exercise training reported outcomes for adherence to the intervention at over six months.

 
7. Compliance with other CF treatments

Compliance with other CF treatments was not measured or reported in any of the studies of supervised exercise training.

 
8.Cost evaluation

Cost evaluation was not assessed or reported in any of the studies of supervised exercise training.

 

Unsupervised exercise training

Two studies, including 110 participants reported the outcomes of unsupervised exercise training on physical activity participation (Hebestreit 2010; Schneiderman-Walker 2000). In both studies, control group participants received no intervention and participated in their usual physical activity. Participants in the intervention groups received activity counselling and exercise or activity prescription planning at baseline (with telephone and clinic consultation throughout the intervention period) to undertake either predominantly aerobic training (Schneiderman-Walker 2000) or a combination of aerobic and resistance training (Hebestreit 2010).

 

Primary outcomes

 
1. Participation in physical activity
 
a. At less than or equal to one month

No studies of unsupervised exercise training reported physical activity outcomes at less than or equal to one month.

 
b. Over one month up to six months

Activity counselling and exercise advice resulted in no significant difference in participation in vigorous physical activity as measured by accelerometry, when compared to a control group with no intervention at three- to six-months follow up, MD 1.05 hours/week (95% CI -0.65 to 2.75 hours/week) (Hebestreit 2010). In order to present the MD and 95% CI data from the study by Hebestreit, the MD between groups was taken from the results published in table 2 of the manuscript (Hebestreit 2010), and the 95% CI was calculated by multiplying the SE by 1.96.

 
c. At over six months

At 12-months follow up, activity counselling and exercise advice resulted in no significant difference in participation in vigorous physical activity as measured by accelerometry, when compared to a control group with no intervention, MD 2.08 hours/week (95% CI -1.84 to 6 hours/week) (Hebestreit 2010). However, at 18- to 24-months follow up, participants who received activity counselling and exercise advice spent significantly more time in vigorous activity than participants in the control group, MD 1.63 hours/week (95% CI 0.02 to 3.24 hours/week); P = 0.047. Time spent in vigorous activity was determined via accelerometry over seven days.

In contrast, in the second study, physical activity participation was assessed using daily activity diaries in children with CF (Schneiderman-Walker 2000). Activity participation was reported as significantly greater in the home exercise programme group compared to the no intervention control group at the end of one year (P = 0.06), two years (P = 0.006) and three years (P = 0.01) (Schneiderman-Walker 2000). No data for activity participation were reported.

 
2. Change in quality of life
 
a. At less than or equal to one month

No studies of unsupervised exercise training assessed or reported on QOL at less than or equal to one month.

 
b. Over one month up to six months

Hebestreit reported that the only change in QOL was recorded in the subjective health perception category (Hebestreit 2010). Subjective health perception favoured the activity counselling and exercise advice group compared to the no intervention control group at three to six months, MD 9.91 points (95% CI 0.89 to 18.93 points) (P = 0.031).

 
c. At over six months

At 12-month follow up the activity counselling and exercise advice group demonstrated no difference in score for subjective health perception from baseline when compared to a no intervention control group, MD -2.31 points (95% CI -15.46 to 10.84 points) (Hebestreit 2010). However, at 18- to 24-months follow up, subjective health perception was better for the activity counselling and exercise advice group compared to the control group, MD 9.89 points (95% CI 0.64 to 19.14 points) (P = 0.036) (Hebestreit 2010).

 

Secondary outcomes

 
1. Change in exercise capacity

Both studies of unsupervised exercise training assessed exercise capacity using a cycle ergometry test (Hebestreit 2010; Schneiderman-Walker 2000).

 
a. At less than or equal to one month

No studies of unsupervised exercise training assessed or reported on change in exercise capacity at less than or equal to one month.

 
b.Over one month up to six months

Six months activity counselling and exercise advice produced significant differences in change in VO2 peak at three to six months when compared to a control group with no intervention, MD 2.04 ml/kg/min (95% CI 0.08 to 4.0 ml/kg/min) (P = 0.041) (Hebestreit 2010).

 
c. At over six months

In one study, a home exercise programme resulted in no significant difference in the annual rate of decline of VO2 peak, over a three-year period, compared to a control group with no intervention, MD 0.05 ml/kg/min (95% CI -1.10 to 1.20 ml/kg/min) (Schneiderman-Walker 2000) ( Analysis 2.1). Similarly, after 12 months there was no difference in VO2 peak between those who received six months activity counselling and exercise advice and a control group with no training, MD 0.70 ml/kg/min (95% CI -1.61 to 3.01 ml/kg/min) (Hebestreit 2010). However, at 18- to 24-months follow up, six months activity counselling and exercise advice resulted in a significant difference in change in VO2 peak when compared to a no intervention control group, MD 3.73 ml/kg/min (95% CI 1.32 to 6.14 ml/kg/min) (P = 0.002) (Hebestreit 2010).

 
2. Pulmonary function tests
 
a. Change in FEV1 (% predicted)

Change in FEV1 was reported in both studies of unsupervised exercise training.

In one study, a trend toward a greater annual rate of decline in FEV1 was reported in the control group with no intervention when compared to a three-year home exercise programme group, MD 2.01% (95% CI -0.06 to 4.08%) (Schneiderman-Walker 2000) ( Analysis 2.2). In the second study, activity counselling and exercise advice was found to have had no effect on FEV1 at any time-point (Hebestreit 2010). The raw data supplied by the authors have generated the following results: at three-months follow up MD -0.75% (95% CI -6.10 to 4.60%); at six-months follow up, MD 2.42 (95% CI -5.42 to 10.26%); at 12-months follow up, MD -0.36% (95% CI -8.02 to 7.30%); at 18-months follow up, MD 5.66% (95% CI -2.88 to 14.20%); and at 24-months follow up, MD -1.72% (95% CI -10.62 to 7.18%) ( Analysis 2.3).

 
b. Change in FVC (%predicted)

i. At less than or equal to one month

No studies of unsupervised exercise training assessed or reported on change in FVC at less than or equal to one month.

ii. Over one month up to six months

In one study, at three- to six-months follow up there was no difference in change in the FVC for activity counselling and exercise advice compared to no intervention control, MD 0.5% (95% CI -4.3 to 5.3%) (P = 0.837) (Hebestreit 2010).

iii. At over six months

A significantly greater annual rate of decline in FVC was seen in the control group of the Schneiderman-Walker study when compared to a three-year home exercise programme group, MD 2.17% (95% CI 0.47 to 3.87%) (Schneiderman-Walker 2000) ( Analysis 2.4).

In the Hebestreit study, for activity counselling and exercise advice there was no difference in change in FVC when compared to a no intervention control group at 12 months, MD 2.71 % (95% CI -4.36 to 9.78 %) (Hebestreit 2010), however at 18 to 24 months the activity counselling and exercise advice group had significantly greater improvement in FVC compared to the no intervention control group, MD 6.06 % (95% CI 0.43 to 11.69 %) (P = 0.036).

 
c. Change in FEF25-75 (%predicted)

Outcomes for FEF25-75 were only reported at over six months in a single study of unsupervised exercise training (Schneiderman-Walker 2000).

i. At less than or equal to one month

No studies of unsupervised exercise training assessed or reported on change in FEF25-75 at less than or equal to one month.

ii. Over one month up to six months

No studies of unsupervised exercise training assessed or reported on change in FEF25-75 at one to six months.

iii. At over six months

In one study, a greater annual rate of decline in FEF25-75 was seen in the control group when compared to a home exercise programme group, but the difference was not significant, MD 0.80% (95% CI -2.20 to 3.80%) (Schneiderman-Walker 2000) ( Analysis 2.5).

 
3. Adverse outcomes

No adverse outcomes were reported in any studies of unsupervised exercise training.

 
4. Body composition

One study of unsupervised exercise training assessed and reported outcomes for body composition (Hebestreit 2010). Raw data supplied by the study authors was used to assess this outcome.

 
a. At less than or equal to one month

No studies of unsupervised exercise training assessed or reported on body composition at less than or equal to one month.

 
b. Over one month up to six months

Hebestreit reported significant improvements in lean body mass (kg) favouring participants who received activity counselling and exercise advice compared to the control group with no intervention at three-month and six-month follow up (Hebestreit 2010): three months, MD 1.76 kg (95% CI 0.97 to 2.55 kg); six months, MD 2.16 kg (95% CI 0.55 to 3.77 kg) ( Analysis 2.6).

However, for skin-fold measurement (mm, across four sites) the results for three and six months were not statistically significant: at three-months follow up, MD -0.47 mm (95% CI -4.42 to 3.48 mm); at six-months follow up, MD -2.54 mm (95% CI -6.70 to 1.62 mm) ( Analysis 2.7).

 
c. At over six months

Hebestreit found no significant difference in lean body mass (kg): at 12 months, MD 1.88 kg (95% CI -0.13 to 3.89 kg); at 18 months, MD 1.77 kg (95% CI -0.80 to 4.34 kg); and at 24-months follow up, MD 1.28 kg (95% CI -1.43 to 5.07 kg) ( Analysis 2.6).

Hebestreit found a significant difference in skin fold measurements (mm, across four sites) between participants who received activity counselling and exercise advice compared to no intervention control at 12-months follow up, MD -5.54 mm (95% CI -10.86 to -0.22 mm) ( Analysis 2.7). However, the results at the remaining time-points were not significant: at 18-months follow up, MD -5.03 mm (95% CI -10.74 to 0.68 mm); and at 24-months follow up, MD -6.94 mm (95% CI -14.02 to 0.14 mm) ( Analysis 2.7).

 
5. Change in bone mineral density (defined on DXA scans)

Bone mineral density was not measured or reported in any of the studies of unsupervised exercise training.

 
6. Adherence to intervention

Adherence to the intervention programme was described in two studies of unsupervised exercise training (Hebestreit 2010; Schneiderman-Walker 2000).

 
a. At less than or equal to one month

No studies of unsupervised exercise training assessed or reported on adherence to the intervention at less than or equal to one month.

 
b. At over one month up to six months

No studies of unsupervised exercise training assessed or reported on adherence to the intervention at one to six months.

 
c. At over six months

A grading system of 0 to 2 (0 indicating poor compliance; 1 indicating partial compliance; 2 indicating full compliance) was used by exercise physiologists overseeing the three-year home exercise programme (Schneiderman-Walker 2000). Mean (SD) scores for exercise compliance in the intervention group did not change across the three years of the study (year 1: 1.51 (0.55); year 2: 1.51 (0.60); year 3: 1.49 (0.62)).

Hebestreit identified under-reporting, or insufficient compliance with physical activity participation following activity counselling and exercise advice (Hebestreit 2010). This presents a possible explanation for increases in physical activity participation not reaching the prescribed target of three hours per week. Participants in the activity counselling and exercise advice group had a self-reported increase in vigorous physical activity of 2.16 hours per week.

 
7. Compliance with other CF treatments
 
a. At less than or equal to one month

No studies of unsupervised exercise training assessed or reported on compliance with other CF treatments at less than or equal to one month.

 
b. Over one month up to six months

No studies of unsupervised exercise training assessed or reported on compliance with other CF treatments at one to six months.

 
c. At over six months

Compliance with other CF treatments was reported in one study (Schneiderman-Walker 2000). Compliance with prescribed physiotherapy regimen was graded 0 to 2 (0 indicating poor compliance; 1 indicating partial compliance; 2 indicating full compliance) and scored by the exercise physiologists administering the intervention. Information regarding physiotherapy compliance was provided subjectively by the patient, and their parent when necessary, in order for a score to be given. Mean scores for compliance with physiotherapy routines were lower than compliance scores for exercise participation, but were not significantly different between the exercise group (year 1 physiotherapy compliance mean score: 0.69) and the control group (year 1 physiotherapy compliance mean score: 0.95). Measures of variability were not reported for physiotherapy compliance scores.

 
8. Cost evaluation

Cost evaluation was not assessed or reported in any of the studies of unsupervised exercise training.

 

Discussion

  1. Top of page
  2. Background
  3. Objectives
  4. Methods
  5. Results
  6. Discussion
  7. Authors' conclusions
  8. Acknowledgements
  9. Data and analyses
  10. Appendices
  11. Contributions of authors
  12. Declarations of interest
  13. Sources of support
  14. Differences between protocol and review
  15. Index terms
 

Summary of main results

This systematic review aimed to examine the effect of interventions designed to promote physical activity participation in people with CF. A search of the literature identified 15 potentially relevant studies, of which only four met the inclusion criteria. All four included studies, providing data on 199 participants, assessed the effect of an exercise programme on participation in physical activity. None of the included studies were primarily designed to increase participation in physical activity, but rather assessed the effect of the exercise training intervention on outcomes including participation in physical activity.

The effects of the interventions were varied, and most evident with longer-term follow up. Inpatient, supervised aerobic training and resistance training did not result in significant changes in participation in physical activity at one month post-discharge (Selvadurai 2002), nor did supervised anaerobic training at three-month follow up (Klijn 2004). Six months of unsupervised exercise training following activity counselling and exercise advice had no effect on vigorous physical activity participation compared to a control group with no intervention when assessed at three to six months and 12-months follow up (Hebestreit 2010). There was, however, a significant improvement in activity participation at 18- to 24-months follow up, MD 1.63 hours per week (95% CI 0.02 to 3.24 hours per week) (Hebestreit 2010). Physical activity participation was also significantly greater in a home exercise programme group compared to a control group with no intervention at the end of each year over three years of follow up (year one (P = 0.06), year two (P = 0.006) and year three (P = 0.01)) (Schneiderman-Walker 2000). These results indicate that exercise training strategies of longer duration (greater than six months), and those that require self-directed behaviours, may have more impact on physical activity participation than short-term, supervised interventions. Furthermore, that duration of follow up may be an important factor in assessing interventions to promote physical activity with improvements in physical activity participation only being evident when outcomes were measured at or after 12 months.

Type and duration of exercise training interventions had inconsistent effects on exercise capacity and physical activity participation. Inpatient, supervised, aerobic training had a positive short-term influence on exercise capacity at one-month follow up post-discharge when compared to a control group with no physical training, but no influence on physical activity participation (Selvadurai 2002). Likewise, 12 weeks supervised anaerobic training improved exercise capacity compared to no training at the completion of the intervention period without concomitant changes in physical activity participation (Klijn 2004). Differences in exercise capacity following 12 weeks supervised anaerobic training were no longer evident at three month follow up. Whether changes in exercise capacity resulting from supervised inpatient aerobic training, or improvements in physical activity participation would become evident at longer-term follow up is unknown.

The studies in this review suggest that changes in physical activity participation do not always occur simultaneously with changes in exercise capacity. Six months of activity counselling and exercise advice produced significant differences in change in VO2 peak between the intervention group and a no intervention control group at three- to six-months and 18- to 24-months follow up (Hebestreit 2010), however, improvement in physical activity participation was only seen concurrently at 18 to 24 months post intervention. Meanwhile a three-year home exercise programme found no difference in annual rate of decline in VO2peak when compared to a no intervention control group, but did result in significant improvements in diary reports of activity participation when compared to the no intervention control group (Schneiderman-Walker 2000). The inconsistent nature of the relationship between changes in exercise capacity and changes in physical activity participation, suggests that inferences regarding physical activity participation can rarely be determined based on exercise capacity alone, and vice versa. The only intervention with a significant effect on both exercise capacity and physical activity participation, and only at a single time-point, was six months of activity counselling and exercise advice compared to a no intervention control when assessed at 18 to 24 months. It is unclear why this effect was not evident at other time-points, or for other interventions. There is insufficient evidence to draw conclusions on the relationship between exercise capacity and physical activity participation, and how any such relationship may influence the type, duration and follow-up period of interventions to promote physical activity participation.

Included studies reported limited effects of the interventions on QOL outcomes. Included studies reported effects on QOL that were unclear (Klijn 2004), inconsistent across time points (Hebestreit 2010) or too small to be clinically meaningful (Selvadurai 2002). Only one study reported an improvement in a single domain of QOL with a concurrent improvement in physical activity participation (Hebestreit 2010). This effect was only seen at a single assessment time-point, and was not found in any other studies where the effect of an exercise training programme may have resulted in an improvement in QOL or physical activity participation, but not both at the same time. In contrast, exercise training undertaken as pulmonary rehabilitation in people with chronic obstructive pulmonary disease (COPD) achieves both large and clinically important changes in QOL (Lacasse 2006). This may suggest it is more difficult to achieve changes in QOL in a complex, multi-system disease like CF with a singular mode of intervention (i.e. exercise training), than in other respiratory diseases. Additionally, it will be important to elucidate which elements of an activity counselling and exercise advice programme lends itself to simultaneously improve QOL and physical activity participation to see if this effect can be replicated at other time-points, or when combined with other interventions.

 

Overall completeness and applicability of evidence

The included studies had intervention periods of variable length, ranging from 18 days (Selvadurai 2002) to three years (Schneiderman-Walker 2000). The follow-up periods ranged from short term (one month) to long term (three years). Predominantly, the included studies were conducted solely with paediatric participants. Only one study had a combined cohort of adult and paediatric participants (Hebestreit 2010). The included studies in this review comprised a relatively young (maximum mean age 19.5 years) CF population, with only mild to moderate CF lung disease. Consequently, whether the results of this review are applicable to older people with CF, and those with more severe lung disease, can not be stated with certainty. The included studies were conducted in both hospitalised patients receiving intravenous antibiotics, as well as in outpatients. The effect of respiratory exacerbations, or fluctuating health status over longer follow-up periods, e.g. one to three years, on the reported results of physical activity participation is not clear.

The intervention strategies to promote participation in physical activity were all exercise training programmes. Only one study specified the exact nature of the exercises which constituted the training programme (available as an online supplement (Klijn 2004). Basic details of activity counselling and exercise advice were described (Hebestreit 2010) as was the development of a home exercise programme (Schneiderman-Walker 2000). However, strategies known to effect change in physical activity behaviour, such as motivational interviewing, were not described (Brodie 2005). It is unclear whether the inclusion of such strategies would have produced greater improvements in physical activity participation when coupled with the chosen intervention.

No studies were identified which investigated strategies other than exercise training programmes for their impact on participation in physical activity. It should be considered that other strategies, such as psychological interventions, print material or telemedicine applications, may also have an effect on physical activity participation in this population. More evidence is needed in this important area.

 

Quality of the evidence

There are limited RCTs addressing physical activity participation in people with CF. The quality of the four included studies in this review was graded according to the standardised risk of bias tool as described in the Cochrane Handbook of Systematic Reviews of Interventions (Higgins 2011). As a result of limitations to reporting, it was difficult to accurately determine study quality. Given that all of the included studies involved exercise training interventions, double blinding was impracticable. Blinded assessors were identified for only one outcome in one study (Schneiderman-Walker 2000). For the remaining outcome measures in this study, and for the remaining three studies, it is unclear what impact this may have had on outcome assessment. The inability to pool data for quantitative analysis of the primary outcomes underscores the limited evidence for exercise training programmes to promote participation in physical activity in people with CF.

 

Potential biases in the review process

Any type of physical activity measure was included in this review. This may pose a source of bias when comparing the results across studies, as some methods of physical activity assessment are more objective than others. For example, subjective physical activity assessment using questionnaires or activity diaries can have reduced accuracy given the need to rely upon participant (or caregiver) recall of events to provide information (Bradley 2010). Whilst objective measures of participation in physical activity, such as accelerometers or video observation, provide detailed information on participant movement; they are expensive and require technical expertise to operate, and may pose questions regarding inter-device variability (Bradley 2010). Futhermore, data obtained from objective measurement devices will be influenced by the type of monitor chosen, where on the body the monitor is worn, and the number of days over which activity data are collected (Trost 2005).

 

Agreements and disagreements with other studies or reviews

A current Cochrane Review indicates there are positive effects on health-related outcomes such as blood glucose control, bone mineral accretion and potentially clearance of pulmonary secretions associated with physical training for individuals with CF (Bradley 2008), however, the impact of this training on physical activity participation was not considered. A Cochrane Review of strategies to promote physical activity participation in adults without pre-existing medical conditions identifies that counselling and support by a professional can encourage increased participation in the short to medium term (Foster 2005). Meanwhile, a meta-analysis of interventions to promote physical activity in adults with chronic illness identified supervised exercise to be no more effective than education or motivational sessions, and that the greatest intervention effect was seen in education interventions which solely targeted physical activity (Conn 2008). This current review is the first to evaluate the impact of clinical interventions on participation in physical activity for people with CF.

 

Authors' conclusions

  1. Top of page
  2. Background
  3. Objectives
  4. Methods
  5. Results
  6. Discussion
  7. Authors' conclusions
  8. Acknowledgements
  9. Data and analyses
  10. Appendices
  11. Contributions of authors
  12. Declarations of interest
  13. Sources of support
  14. Differences between protocol and review
  15. Index terms

 

Implications for practice

From this review, it is difficult to draw strong conclusions regarding the most effective strategies for promoting participation in physical activity in people with CF. Only a small number of randomised controlled studies have investigated interventions to promote physical activity in this group. All included studies investigated exercise training with no evaluation of the strategies that have been found to be effective in other populations (Eakin 2007; van Sluijs 2007; Vandelanotte 2007). The outcomes reported were variable and we were not able to calculate any pooled effects. In addition, the included studies were undertaken in relatively young people with CF, without severe disease. This review provides very limited evidence that activity counselling and exercise advice to undertake a home exercise programme, conducted over at least six months, may result in improved participation in physical activity by people with CF, when assessed in the longer term. The length of follow up required to see benefit in terms of participation in physical activity may have implications with regards to participant motivation and adherence.

 
Implications for research

This review has only identified studies using exercise training to promote participation in physical activity in people with CF. Further research is needed to assess the effect of other strategies, which may include the use of print material, health coaching or telemedicine applications, on promoting the uptake and adherence to regular physical activity participation in this population. The use of the Internet to promote physical activity in a variety of adult populations, including those who are healthy, diabetic or overweight and those with physical disability, was found to have a small effect size (mean 0.44) with better outcomes achieved when there were at least five communications (e.g. email) with participants and when the intervention was of shorter duration (up to three months) (Vandelanotte 2007). In healthy adults, and those with chronic conditions, telephone interventions were effective in increasing physical activity participation, particularly when the intervention period ranged from six to 12 months, and included at least 12 telephone calls (Eakin 2007). In children and adolescents there is strong evidence that school-based interventions, which include family or community involvement, effectively promote physical activity participation (van Sluijs 2007). Future research might focus on identifying whether similar or alternative strategies are effective in promoting participation in physical activity for different groups within the CF population, such as males compared to females, children compared to adults, and severe CF lung disease compared to mild or moderate disease.

 

Acknowledgements

  1. Top of page
  2. Background
  3. Objectives
  4. Methods
  5. Results
  6. Discussion
  7. Authors' conclusions
  8. Acknowledgements
  9. Data and analyses
  10. Appendices
  11. Contributions of authors
  12. Declarations of interest
  13. Sources of support
  14. Differences between protocol and review
  15. Index terms

The authors would like to acknowledge the authors of included studies who responded to requests for additional information, and the valued assistance of Tracey Remmington.

 

Data and analyses

  1. Top of page
  2. Background
  3. Objectives
  4. Methods
  5. Results
  6. Discussion
  7. Authors' conclusions
  8. Acknowledgements
  9. Data and analyses
  10. Appendices
  11. Contributions of authors
  12. Declarations of interest
  13. Sources of support
  14. Differences between protocol and review
  15. Index terms
Download statistical data

 
Comparison 1. Supervised exercise training compared to no intervention

Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size

 1 Participation in physical activity (MJ/day)1Mean Difference (IV, Fixed, 95% CI)Subtotals only

    1.1 Predominantly aerobic training (short-term follow up ≦ 1 month)
131Mean Difference (IV, Fixed, 95% CI)1.20 [-0.47, 2.87]

    1.2 Predominantly resistance training (short-term follow up ≦ 1 month)
134Mean Difference (IV, Fixed, 95% CI)0.65 [-0.86, 2.16]

 2 Change in quality of life1Mean Difference (IV, Fixed, 95% CI)Subtotals only

    2.1 Predominantly aerobic training (short-term follow up ≦ 1 month)
144Mean Difference (IV, Fixed, 95% CI)0.10 [0.03, 0.17]

    2.2 Predominantly resistance training (short-term follow up ≦ 1 month)
144Mean Difference (IV, Fixed, 95% CI)0.03 [-0.04, 0.10]

 3 Change in exercise capacity (ml/kg/min)2Mean Difference (IV, Fixed, 95% CI)Subtotals only

    3.1 Predominantly aerobic training (short term; end of intervention)
144Mean Difference (IV, Fixed, 95% CI)8.53 [4.85, 12.21]

    3.2 Predominantly aerobic training (short-term follow up ≦ 1 month)
144Mean Difference (IV, Fixed, 95% CI)4.91 [1.13, 8.69]

    3.3 Predominantly anaerobic training (short-term follow up; end of intervention)
120Mean Difference (IV, Fixed, 95% CI)2.1 [0.12, 4.08]

    3.4 Predominantly resistance training (short-term follow up; end of intervention)
144Mean Difference (IV, Fixed, 95% CI)1.95 [-1.61, 5.51]

    3.5 Predominantly resistance training (short-term follow up ≦ 1 month)
144Mean Difference (IV, Fixed, 95% CI)-0.40 [-4.03, 3.23]

 4 Change in FEV1 (% predicted)1Mean Difference (IV, Fixed, 95% CI)Subtotals only

    4.1 Predominantly aerobic training (short-term follow up; end of intervention)
144Mean Difference (IV, Fixed, 95% CI)2.03 [-2.31, 6.37]

    4.2 Predominatly aerobic training (short-term follow up ≦ 1 month)
144Mean Difference (IV, Fixed, 95% CI)1.53 [-2.93, 5.99]

    4.3 Predominantly resistance training (short-term follow up; end of intervention)
144Mean Difference (IV, Fixed, 95% CI)5.58 [1.34, 9.82]

    4.4 Predominantly resistance training (short-term follow up ≦ 1 month)
144Mean Difference (IV, Fixed, 95% CI)5.08 [0.66, 9.50]

 5 Change in FVC (% predicted)1Mean Difference (IV, Fixed, 95% CI)Subtotals only

    5.1 Predominantly aerobic training (short-term follow up; end of intervention)
144Mean Difference (IV, Fixed, 95% CI)0.06 [-2.55, 2.67]

    5.2 Predominantly aerobic training (short-term follow up ≦ 1 month)
144Mean Difference (IV, Fixed, 95% CI)-0.11 [-2.64, 2.42]

    5.3 Predominantly resistance training (short-term follow up; end of intervention)
144Mean Difference (IV, Fixed, 95% CI)0.17 [-2.31, 2.65]

    5.4 Predominantly resistance training (short-term follow up ≦ 1 month)
144Mean Difference (IV, Fixed, 95% CI)0.06 [-2.42, 2.54]

 6 Change in fat-free mass (kg)2Mean Difference (IV, Fixed, 95% CI)Subtotals only

    6.1 Predominantly aerobic training (short-term follow up; end of intervention)
144Mean Difference (IV, Fixed, 95% CI)0.01 [-0.19, 0.21]

    6.2 Predominantly aerobic training (short-term follow up ≦ 1 month)
144Mean Difference (IV, Fixed, 95% CI)0.04 [-0.19, 0.27]

    6.3 Anaerobic training (short-term follow up; end of intervention)
120Mean Difference (IV, Fixed, 95% CI)-0.4 [-1.14, 0.34]

    6.4 Anaerobic training (follow up between 1 and 6 months)
120Mean Difference (IV, Fixed, 95% CI)0.30 [-1.27, 1.87]

    6.5 Predominantly resistance training (short-term follow up; end of intervention)
144Mean Difference (IV, Fixed, 95% CI)1.80 [1.57, 2.03]

    6.6 Predominantly resistance training (short-term follow up ≦ 1 month)
144Mean Difference (IV, Fixed, 95% CI)1.71 [1.46, 1.96]

 
Comparison 2. Unsupervised exercise training compared to no intervention

Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size

 1 Annual rate of change in exercise capacity (ml/kg/min) (follow up > 6 months)1Mean Difference (IV, Fixed, 95% CI)Totals not selected

 2 Annual rate of decline FEV1 (% predicted) (follow up > 6 months)165Mean Difference (IV, Fixed, 95% CI)2.01 [-0.06, 4.08]

 3 Change in FEV1 (% predicted)1Mean Difference (IV, Fixed, 95% CI)Subtotals only

    3.1 At 3-month follow up
135Mean Difference (IV, Fixed, 95% CI)-0.75 [-6.10, 4.60]

    3.2 At 6-month follow up
135Mean Difference (IV, Fixed, 95% CI)2.42 [-5.42, 10.26]

    3.3 At 12-months follow up
131Mean Difference (IV, Fixed, 95% CI)-0.36 [-8.02, 7.30]

    3.4 At 18-months follow up
133Mean Difference (IV, Fixed, 95% CI)5.66 [-2.88, 14.20]

    3.5 At 24-months follow up
128Mean Difference (IV, Fixed, 95% CI)-1.72 [-10.62, 7.18]

 4 Annual rate of decline FVC (% predicted) (follow up > 6 months)165Mean Difference (IV, Fixed, 95% CI)2.17 [0.47, 3.87]

 5 Annual rate of decline FEF25-75 (% predicted) (follow up > 6 months)165Mean Difference (IV, Fixed, 95% CI)0.80 [-2.20, 3.80]

 6 Change in lean body mass (kg)1Mean Difference (IV, Fixed, 95% CI)Subtotals only

    6.1 At 3-months follow up
135Mean Difference (IV, Fixed, 95% CI)1.76 [0.97, 2.55]

    6.2 At 6-months follow up
135Mean Difference (IV, Fixed, 95% CI)2.16 [0.55, 3.77]

    6.3 At 12-months follow up
130Mean Difference (IV, Fixed, 95% CI)1.88 [-0.13, 3.89]

    6.4 At 18-months follow up
131Mean Difference (IV, Fixed, 95% CI)1.77 [-0.80, 4.34]

    6.5 At 24-months follow up
128Mean Difference (IV, Fixed, 95% CI)1.82 [-1.43, 5.07]

 7 Change in skin fold measure (mm)1Mean Difference (IV, Fixed, 95% CI)Subtotals only

    7.1 At 3-months follow up
135Mean Difference (IV, Fixed, 95% CI)-0.47 [-4.42, 3.48]

    7.2 At 6-months follow up
135Mean Difference (IV, Fixed, 95% CI)-2.54 [-6.70, 1.62]

    7.3 At 12-months follow up
130Mean Difference (IV, Fixed, 95% CI)-5.54 [-10.86, -0.22]

    7.4 At 18-months follow up
131Mean Difference (IV, Fixed, 95% CI)-5.03 [-10.74, 0.68]

    7.5 At 24-months follow up
128Mean Difference (IV, Fixed, 95% CI)-6.94 [-14.02, 0.14]

 

Appendices

  1. Top of page
  2. Background
  3. Objectives
  4. Methods
  5. Results
  6. Discussion
  7. Authors' conclusions
  8. Acknowledgements
  9. Data and analyses
  10. Appendices
  11. Contributions of authors
  12. Declarations of interest
  13. Sources of support
  14. Differences between protocol and review
  15. Index terms
 

Appendix 1. Glossary


Physical activityAny bodily movement produced by the skeletal muscles that results in energy expenditure.

ExerciseA component of physical activity. Exercise is planned and structured.

InterventionA treatment or therapy, in this instance aiming to increase participation in physical activity.

PulmonaryIn relation to, or affecting, the lungs.

SecretionsA substance produced by and discharged from a part of the body e.g. pulmonary secretions - a substance produced by the lungs.

PrognosticRelating to prognosis; predicting the likely outcome of a disease.

SuppurativeThe formation and/or discharge of pus e.g. suppurative lung disease - a disease process producing pus in the lungs.



 

Appendix 2. Search Strategy: CINAHL (EbscoHost)


1. randomized controlled trials. mh

2.  clinical trials. mh

3.  placebos. mh

4.  humans NOT animals

5. 1 or 2 or 3

6. 4 and 5

 

7. Cystic Fibrosis. mh

8. CF. tx

9. mucoviscidosis. ti, ab

10. 7 or 8 or 9

11. 6 and 10

 

12. physical activity. mh

13. physical fitness. mh

14. habitual N3 activity. ti, ab

15. exercise. mh

16. exertion. mh

17. sport. ti, ab

18. 12 or 13 or 14 or 15 or 16 or 17

19. 11 and 18

 

20. promot*. tx

21. uptake. tx

22. encourage. tx

23. increase. tx

24. start. tx

25. educat*. tx

26. program*. tx

27. 20 or 21 or 22 or 23 or 24 or 25 or 26

28. 19 and 27

 

 

.ab. denotes a word in the abstract;

.mh. denotes a CINAHL exact subject heading

.ti. denotes a word in the title.

.tx. Performs a keyword search of all the database's searchable fields



 

Appendix 3. Search Strategy: MEDLINE (Ovid) & PsycINFO (Ovid)


1. randomized controlled trial. pt

2.  controlled clinical trial. pt

3.  randomized. ab

4.  placebo. ab

5.  randomly. ab

6.  trial. ab

7.  groups. ab

8.  1 or 2 or 3 or 4 or 5 or 6 or 7

 

9. cystic fibrosis or CF

10.  fibrocystic adj5 disease adj5 pancreas

11.  mucoviscidos$

12.  (cystic$ adj10 fibros$)

13.  9 or 10 or 11 or 12

14. 8 and 13

 

15. physical activity

16. physical fitness

17.  habitual adj3 activity

18. exercise

19. exertion

20. sport

21. 15 or 16 or 17 or 18 or 19 or 20

22. 14 and 21

 

23. promot$

24. uptake

25. increase

26. start

27. educat$

28. program$

29. 23 or 24 or 25 or 26 or 27 or 28

30. 22 and 29



 

Appendix 4. Search Strategy: PEDro


Abstract and Title: Cystic Fibrosis

Problem: Reduced exercise tolerance

Sub discipline: Cardiothoracics

Abstract and Title:   Cystic Fibrosis

Therapy:                  Fitness training

Sub discipline:         Cardiothoracics

Abstract and Title:    Cystic Fibrosis

Therapy:                  Behaviour modification 

Sub discipline:         Cardiothoracics



 

Contributions of authors

  1. Top of page
  2. Background
  3. Objectives
  4. Methods
  5. Results
  6. Discussion
  7. Authors' conclusions
  8. Acknowledgements
  9. Data and analyses
  10. Appendices
  11. Contributions of authors
  12. Declarations of interest
  13. Sources of support
  14. Differences between protocol and review
  15. Index terms

NSC conducted the search for trials and selected included trials; assessed trial quality; extracted, entered and analysed data; interpreted results and wrote the review. AEH selected included trials; assessed trial quality; checked extracted and entered data; edited the draft review. JAA was consulted on trials for inclusion and edited the draft review. All authors conceived the review.

 

Declarations of interest

  1. Top of page
  2. Background
  3. Objectives
  4. Methods
  5. Results
  6. Discussion
  7. Authors' conclusions
  8. Acknowledgements
  9. Data and analyses
  10. Appendices
  11. Contributions of authors
  12. Declarations of interest
  13. Sources of support
  14. Differences between protocol and review
  15. Index terms

Narelle Cox has received a PhD stipend from the National Health and Medical Research Council (NH&MRC), Australia. This review forms a part of those PhD studies. The NH&MRC will not interfere with the independence of the authors in regard to the conduct of the review and will not delay or prevent publication of the review.

 

Sources of support

  1. Top of page
  2. Background
  3. Objectives
  4. Methods
  5. Results
  6. Discussion
  7. Authors' conclusions
  8. Acknowledgements
  9. Data and analyses
  10. Appendices
  11. Contributions of authors
  12. Declarations of interest
  13. Sources of support
  14. Differences between protocol and review
  15. Index terms
 

Internal sources

  • No sources of support supplied

 

External sources

  • National Health and Medical Research Council (NHMRC), Australia.
    PhD Scholarship
  • Cystic Fibrosis Australia Research Trust, Australia.
    PhD Stipend

 

Differences between protocol and review

  1. Top of page
  2. Background
  3. Objectives
  4. Methods
  5. Results
  6. Discussion
  7. Authors' conclusions
  8. Acknowledgements
  9. Data and analyses
  10. Appendices
  11. Contributions of authors
  12. Declarations of interest
  13. Sources of support
  14. Differences between protocol and review
  15. Index terms

Studies where the primary focus was not activity promotion, but rather the assessment of physiological outcomes as a response to a physical exercise intervention were not originally intended to be included in the review. Such studies were included where they incorporated assessment of physical activity participation.

* Indicates the major publication for the study

References

References to studies included in this review

  1. Top of page
  2. AbstractRésumé scientifique
  3. Background
  4. Objectives
  5. Methods
  6. Results
  7. Discussion
  8. Authors' conclusions
  9. Acknowledgements
  10. Data and analyses
  11. Appendices
  12. Contributions of authors
  13. Declarations of interest
  14. Sources of support
  15. Differences between protocol and review
  16. Characteristics of studies
  17. References to studies included in this review
  18. References to studies excluded from this review
  19. References to studies awaiting assessment
  20. Additional references
Hebestreit 2010 {published and unpublished data}
  • Hebestreit H, Kieser S, Junge S, Ballmann M, Hebestreit A, Schindler C, et al. Long-term effects of a partially supervised conditioning programme in cystic fibrosis. European Respiratory Journal 2010;35(3):578-83.
Klijn 2004 {published data only (unpublished sought but not used)}
  • 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 controlled study. Chest 2004;125(4):1299-305.
Schneiderman-Walker 2000 {published data only (unpublished sought but not used)}
  • Reisman JJ, Schneiderman-Walker J, Corey M, Wilkes D, Pedder L, Levison H, et al. The role of an organized exercise program in cystic fibrosis - a three year study [abstract]. Pediatric Pulmonology 1995;Suppl 12:261.
  • Schneiderman-Walker J, Pollock SL, Corey M, Wilkes DD, Canny GJ, Pedder L, et al. A randomized controlled trial of a 3-year home exercise program in cystic fibrosis. Journal of Pediatrics 2000;136(3):304-10.
Selvadurai 2002 {published data only (unpublished sought but not used)}
  • Selvadurai HC, Blimkie CJ, Meyers N, Mellis CM, Cooper PJ, Van Asperen PP. Randomized controlled study of in-hospital exercise training programs in children with cystic fibrosis. Pediatric Pulmonology 2002;33(3):194-200.
  • Selvadurai HC, Van Asperen PP, Cooper PJ, Mellis CM, Blimkie CJ. A randomised controlled study of in-hospital exercise training programs in children with cystic fibrosis (CF) [abstract]. Pediatric Pulmonology 1999;Suppl 19:287-8.

References to studies excluded from this review

  1. Top of page
  2. AbstractRésumé scientifique
  3. Background
  4. Objectives
  5. Methods
  6. Results
  7. Discussion
  8. Authors' conclusions
  9. Acknowledgements
  10. Data and analyses
  11. Appendices
  12. Contributions of authors
  13. Declarations of interest
  14. Sources of support
  15. Differences between protocol and review
  16. Characteristics of studies
  17. References to studies included in this review
  18. References to studies excluded from this review
  19. References to studies awaiting assessment
  20. Additional references
Bernard 2004 {published data only}
Gulmans 1999 {published data only}
Hind 2008 {published data only}
  • Hind K, Truscott JG, Conway SP. Exercise during childhood and adolescence: a prophylaxis against cystic fibrosis-related low bone mineral density? Exercise for bone health in children with cystic fibrosis. Journal of Cystic Fibrosis 2008;7(4):270-6.
Kuys 2011 {published data only}
  • Hall K, Peasey M, Wood M, Cobb R, Bell SC, Kuys S. The effects of Nintendo Wii® exercise training in adults with cystic fibrosis [abstract]. Journal of Cystic Fibrosis 2010;9(Suppl 1):S71, Abstract no: 275.
  • Kuys SS, Hall K, Peasey M, Wood M, Cobb R, Bell SC. Gaming console exercise and cycle or treadmill exercise provide similar cardiovascular demand in adults with cystic fibrosis: a randomised crossover trial. Journal of Physiotherapy 2011;57(1):35-40.
Moorcroft 2004 {published data only}
  • Dodd ME, Moorcroft AJ, Webb AK. Improved fitness and decreased symptoms of muscle fatigue for upper and lower body following an individualised unsupervised home exercise training programme in adults with cystic fibrosis [abstract]. Pediatric Pulmonology 1998;Suppl 17:347.
  • Moorcroft AJ, Dodd ME, Morris J, Webb AK. Individualised unsupervised exercise training in adults with cystic fibrosis: a 1 year randomised controlled trial. Thorax 2004;59(12):1074-80.
  • Moorcroft AJ, Dodd ME, Webb AK. [Individualised home exercise training in cystic fibrosis - a one-year trial [abstract]]. Proceedings of the 13th International Cystic Fibrosis Congress. 2000:153.
Orenstein 1981 {published data only}
  • Orenstein DM, Franklin BA, Doershuk CF, Hellerstein HK, Germann KJ, Horowitz JG, et al. Exercise conditioning and cardiopulmonary fitness in cystic fibrosis. The effects of a three-month supervised running program. Chest 1981;80(4):392-8.
Petrovic 2013 {published data only}
  • Petrovic M, Kaluza I, Pohl W. Effects of individualised aerobic exercise training in adults with cystic fibrosis: A 4 year controlled trial [abstract]. Journal of Cystic Fibrosis 2013;12 Suppl 1:S28, Abstract no: WS14.4.
Sosa 2012 {published data only}
  • Sosa ES, Groeneveld IR, Gonzalez-Saiz L, Lopez-Mojares LM, Villa-Asensi JR, Barrio Gonzalez MI, et al. Intrahospital weight and aerobic training in children with cystic fibrosis: A randomized controlled trial. Medicine and science in sports and exercise 2012;44(1):2-11.
Swisher 2010 {published data only}
  • 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. http://ijahsp.nova.edu/articles/Vol8Num2/pdf/swisher.pdf (The Internet Journal of Allied Helath Sciences and Practice) (accessed 16 November 2012).
Tunzin 1998 {published data only}
  • Tuzin BJ, Mulvihill MM, Kilbourn KM, Bertran DA, Buono M, Hovell MF, et al. Increasing physical activity of children with cystic fibrosis: a home-based family intervention. Pediatric Exercise Science 1998;10(1):57-68.
van Doorn 2010 {published data only}
  • van Doorn N. Exercise programs for children with cystic fibrosis: a systematic review of randomized controlled trials. Disability and Rehabilitation 2010;32(1):41-9.

References to studies awaiting assessment

  1. Top of page
  2. AbstractRésumé scientifique
  3. Background
  4. Objectives
  5. Methods
  6. Results
  7. Discussion
  8. Authors' conclusions
  9. Acknowledgements
  10. Data and analyses
  11. Appendices
  12. Contributions of authors
  13. Declarations of interest
  14. Sources of support
  15. Differences between protocol and review
  16. Characteristics of studies
  17. References to studies included in this review
  18. References to studies excluded from this review
  19. References to studies awaiting assessment
  20. Additional references
Alarie 2013 {published data only}
  • Alarie N, Agnew LL, McIlwaine MP, Ratjen F, Davidson GF, Milner R, et al. Evaluation of physical activity using the habitual activity estimation scale (HAES) questionnaire in a multicenter study [abstract]. Journal of Cystic Fibrosis 2013;12 Suppl 1:S28, Abstract no: WS14.1.
Marostica 2012 {published data only}
  • Marostica PJ, Hommerding P, Makarewicz G, Baptista R, Donadio MV. Efficacy of an educational intervention of physical activity for children and adolescents with cystic fibrosis [abstract]. Journal of Cystic Fibrosis 2012;11 Suppl 1:S36.

Additional references

  1. Top of page
  2. AbstractRésumé scientifique
  3. Background
  4. Objectives
  5. Methods
  6. Results
  7. Discussion
  8. Authors' conclusions
  9. Acknowledgements
  10. Data and analyses
  11. Appendices
  12. Contributions of authors
  13. Declarations of interest
  14. Sources of support
  15. Differences between protocol and review
  16. Characteristics of studies
  17. References to studies included in this review
  18. References to studies excluded from this review
  19. References to studies awaiting assessment
  20. Additional references
ABS 2012
  • Australian Bureau of Statistics. Australian Health Survey: First Results 2011-2012. http://www.abs.gov.au/ausstats/abs@.nsf/Lookup/4364.0.55.001main+features12011-12 (accessed 28 November 2013).
Andersen 1998
  • Andersen RE, Crespo CJ, Bartlett SJ, Cheskin LJ, Pratt M. Relationship of physical activity and television watching with body weight and level of fatness among children. JAMA 1998;279(12):938-42.
Bradley 2008
Bradley 2010
  • Bradley JM, Kent L, Elborn JS, O'Neill B. Motion sensors for monitoring physical activity in cystic fibrosis: what is the next step?. Physical Therapy Reviews 2010;15(3):197-203.
Brodie 2005
Casparen 1985
  • Casparen CJ, Powell KE, Christenson GM. Physical activity, exercise, and physical fitness: definitions and distinctions for health-related research. Public Health Reports 1985;100(2):126-31.
CDC 2011
  • U.S. Department of Health and Human Services. Centers for Disease Control and Prevention. Strategies to Prevent Obesity and Other Chronic Diseases: The CDC Guide to Strategies to Increase Physical Activity in the Community. http://www.cdc.gov/obesity/downloads/PA_2011_WEB.pdf (accessed 28 November 2013).
Clanchy 2011
Conn 2008
  • Conn VS, Hafdahl AR, Brown SA, Brown LM. Meta-analysis of patient education intervention to increase physical activity among chronically ill adults. Patient Education and Counselling 2008;70:157-72.
Dwyer 2011
  • Dwyer TJ, Alison JA, McKeough ZJ, Daviskas E, Bye PTP. Effects of exercise on respiratory flow and sputum properties in patients with cystic fibrosis. Chest 2011;139(4):870-7.
Eakin 2007
  • Eakin EG, Lawler SP, Vandelanotte C, Owen N. Telephone Interventions for Physical Activity and Dietary Behavior Change: A systematic review. American Journal of Preventative Medicine 2007;32(5):419-34.
Foster 2005
Haskell 2007
  • Haskell WL, Lee I-M, Pate RR, Powel KE, Blair SN, Franklin BA, et al. Physical activity and public health: Updated recommendation for adults from the American College of Sports Medicine and the American Heart Association. Medicine & Science in Sports & Exercise 2007;39(8):1423-34.
Hebestreit 2006
  • Hebestreit H, Kieser S, Rüdiger S, Schenk T, Junge S, Hebestreit A, et al. Physical activity is independently related to aerobic capacity in cystic fibrosis. European Respiratory Journal 2006;28(4):734-9.
Henry 2003
  • Henry B, Aussage P, Grosskopf C, Goehers JM. Development of the Cystic Fibrosis Questionnaire (CFQ) for Assessing Quality of Life in Pediatric and Adult Patients. Quality of Life Research 2003;12(1):63-76.
Higgins 2011
  • Higgins JPT, Green S (editors). Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0 [updated March 2011]. The Cochrane Collaboration, 2011. Available from www.cochrane-handbook.org.
Kahn 2002
  • Kahn EB, Ramsey LT, Brownson RC, Heath GW, Howze EH, Powell KE, et al. The effectiveness of interventions to increase physical activity: A systematic review. American Journal of Preventive Medicine 2002;22(4 Suppl):73-107.
Koch 1993
Lacasse 2006
Marcus 2000
Moy 2012
  • Moy ML, Weston NA, Wilson EJ, Hess ML, Richardson CR. A pilot study of an Internet walking program and pedometer in COPD. Respiratory Medicine 2012;106(9):1342-50.
Nguyen 2009
  • Nguyen HQ, Gill DP, Wolpin S, Steele BG, Benditt JO. Pilot study of a cell phone-based exercise persistence intervention post-rehabilitation for COPD. International Journal of Chronic Obstructive Pulmonary Disease 2009;4:301-13.
Pate 1995
Quittner 2009
  • Quittner AL, Modi AC, Wainwright C, Otto K, Kirihara J, Montgomery AB. Determination of the Minimal Clinically Important Difference Scores for the Cystic Fibrosis Questionnaire-Revised Respiratory Symptom Scale in Two Populations of Patients With Cystic Fibrosis and Chronic Pseudomonas aeruginosa Airway Infection. Chest 2009;135(6):1610-8.
RevMan 2011
  • The Nordic Cochrane Centre, The Cochrane Collaboration. Review Manager (RevMan). 5.1. Copenhagen: The Nordic Cochrane Centre, The Cochrane Collaboration, 2011.
Rimmer 2004
  • Rimmer JH, Riley B, Wang E, Rauworth A, Jurkowski J. Physical activity participation among persons with disabilities: Barriers and facilitators. American Journal of Preventive Medicine 2004;26(5):419-25.
Saiman 2004
Schmidt 2009
  • Schmidt A, Wenninger K, Niemann N, Wahn U, Staab D. Health-related quality of life in children with cystic fibrosis: validation of the German CFQ-R. Health and Quality of Life Outcomes 2009;7:97-106.
Schneiderman-Walker 2005
  • Schneiderman-Walker J, Wilkes DL, Strug L, Lands LC, Pollock SL, Selvadurai HC, et al. Sex differences in habitual physical activity and lung function decline in children with cystic fibrosis. Journal of Pediatrics 2005;147(3):321-6.
Thompson 2003
  • Thompson PD, Buchner D, Pina IL, Balady GJ, Williams MA, Marcus BH, Berra K, Blair SN, Costa F, Franklin B, Fletcher GF, Gordon NF, Pate RR, Rodriguez BL, Yancey AK, Wenger NK. Exercise and Physical Activity in the Prevention and Treatment of Atherosclerotic Cardiovascular Disease: A Statement From the Council on Clinical Cardiology (Subcommittee on Exercise, Rehabilitation, and Prevention) and the Council on Nutrition, Physical Activity, and Metabolism (Subcommittee on Physical Activity). Arteriosclerosis, Thrombosis, and Vascular Biology 2003;23(8):e42-e49.
Troosters 2009
  • Troosters T, Langer D, Vrijsen B, Segers J, Wouters K, Janssens W, et al. Skeletal muscle weakness, exercise tolerance and physical activity in adults with cystic fibrosis. European Respiratory Journal 2009;33(1):99-106.
Trost 2005
van Sluijs 2007
  • van Sluijs EMF, McMinn AM, Griffin SJ. Effectiveness of interventions to promote physical activity in children and adolescents: systematic review of controlled trials. BMJ 2007;335(7622):703-7.
Vandelanotte 2007
Wenninger 2003
  • Wenninger K, Aussage P, Wahn U, Staab D, German CFQ study group. The revised German Cystic Fibrosis Questionnaire: validation of a disease-specific health-related quality of life instrument. Quality of Life Research 2003;12(1):77-85.
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  • WHO. Global recommendations for physical activity and health. Geneva: WHO Library Cataloguing-in-Publication Data, 2010. [ISBN: 978 924159 9979]
Yankaskas 2004