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Meta-Analysis
. 2017 Nov 1;11(11):CD002768.
doi: 10.1002/14651858.CD002768.pub4.

Physical exercise training for cystic fibrosis

Thomas Radtke  1 , Sarah J Nevitt, Helge Hebestreit, Susi Kriemler
Affiliations
Meta-Analysis

Physical exercise training for cystic fibrosis

Thomas Radtke et al. Cochrane Database Syst Rev. .

Update in

Abstract

Background: Physical exercise training may form an important part of regular care for people with cystic fibrosis. This is an update of a previously published review.

Objectives: To assess the effects of physical exercise training on exercise capacity by peak oxygen consumption, pulmonary function by forced expiratory volume in one second, health-related quality of life and further important patient-relevant outcomes in people with cystic fibrosis.

Search methods: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search: 04 May 2017.We searched ongoing trials registers (clinicaltrials.gov and the WHO ICTRP). Date of most recent search: 10 August 2017.

Selection criteria: All randomised and quasi-randomised controlled clinical trials comparing exercise training of any type and a minimum duration of two weeks with conventional care (no training) in people with cystic fibrosis.

Data collection and analysis: Two authors independently selected studies for inclusion, assessed methodological quality and extracted data. The quality of the evidence was assessed using the GRADE system.

Main results: Of the 83 studies identified, 15 studies which included 487 participants, met the inclusion criteria. The numbers in each study ranged from nine up to 72 participants; two studies were in adults, seven were in children and adolescents and six studies included all age ranges. Four studies of hospitalised participants lasted less than one month and 11 studies were outpatient-based, lasting between two months and three years. The studies included participants with a wide range of disease severity and employed differing levels of supervision with a mixture of types of training. There was also wide variation in the quality of the included studies.This systematic review shows very low- to low-quality evidence from both short- and long-term studies that in people with cystic fibrosis aerobic or anaerobic physical exercise training (or a combination of both) has a positive effect on aerobic exercise capacity, pulmonary function and health-related quality of life. No study reported on mortality; two studies reported on adverse events (moderate-quality evidence); one of each study reported on pulmonary exacerbations (low-quality evidence) and diabetic control (very low-quality evidence). Although improvements were not consistent between studies and ranged from no effects to clearly positive effects, the most consistent effects of the heterogeneous exercise training modalities and durations were found for maximal aerobic exercise capacity (in four out of seven studies) with unclear effects on forced expiratory volume in one second (in two out of 11 studies) and health-related quality of life (in two out of seven studies).

Authors' conclusions: Evidence about the efficacy of physical exercise training in cystic fibrosis from 15 small studies with low to moderate methodological quality is limited. Exercise training is already part of regular outpatient care offered to most people with cystic fibrosis, and since there is some evidence for beneficial effects on aerobic fitness and no negative side effects exist, there is no reason to actively discourage this. The benefits from including physical exercise training in an individual's regular care may be influenced by the type and duration of the training programme. High-quality randomised controlled trials are needed to comprehensively assess the benefits of exercise programmes in people with cystic fibrosis and the relative benefits of the addition of aerobic versus anaerobic versus a combination of both types of physical exercise training to the care of people with cystic fibrosis.

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Conflict of interest statement

HH has received financial compensation for travel and accomodation or free meeting participation (or both) at the European Cystic Fibrosis Society conference and the North American Cystic Fibrosis Conference for chairing or presenting at sessions focusing on exercise in cystic fibrosis. For writing an educational booklet on exercise in cystic fibrosis, HH has received money from Novartis. HH is also the lead investigator on one of the studies included in the review (Hebestreit 2010). As he is the lead investigator of the international multicentre trial ACTIVATE‐CF (Hebestreit 2016), his institution has received grants from the Mukoviszidose e.V. and a Vertex Innovation Award.

TR belongs to the core study team of the ACTIVATE‐CF trial (Hebestreit 2016). TR has also received financial compensation for chairing and presenting at exercise sessions at the European Cystic Fibrosis Society conference. He has also received financial support (travel, accomodation) from Vifor Pharma Switzerland to participate at the European Cystic Fibrosis Society and European Respiratory Society conference.

SK is the lead investigator on one of the studies included in the review (Kriemler 2013) and also belongs to the core study team of the ACTIVATE‐CF trial (Hebestreit 2016)

SJN declares no known potential conflicts of interest.

Figures

1
1
Study flow diagram.
2
2
Methodological quality summary: review authors' judgments about each methodological quality item for each included study.
3
3
Methodological quality graph: review authors' judgments about each methodological quality item presented as percentages across all included studies.
See this image and copyright information in PMC

Update of

References

References to studies included in this review

Beaudoin 2017 {published data only}
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Santana‐Sosa 2014 {published data only}
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References to studies excluded from this review

Alarie 2012 {published data only}
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Albinni 2004 {published data only}
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Amelina 2006 {published data only}
    1. Amelina E, Cherniak A, Chikina S, Krasovsky S, Appaeva A. Inspiratory muscle training (IMT) in cystic fibrosis adults [abstract]. Proceedings of European Respiratory Society Annual Congress; 2006 Sep 2‐6; Munich, Germany. 2006. [Abstract no: P4112; CFGD Register: PE177a]
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Andreasson 1987 {published data only}
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Aquino 2006 {published data only}
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Asher 1982 {published data only}
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Barry 2001 {published data only}
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Bieli 2017 {published data only}
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Dwyer 2008 {published data only}
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Giacomodonato 2015 {published data only}
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Heijerman 1992 {published data only}
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Kriemler 2016 {published data only}
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Kuys 2011 {published data only}
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Lannefors 1992 {published data only}
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Lima 2014 {published data only}
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    1. Lima CA, Andrade ADFD, Campos SL, Brandao DC, Fregonezi, Mourato IP, et al. Effects of noninvasive ventilation on treadmill 6‐min walking distance and regional chest wall volumes in cystic fibrosis: randomized controlled trial. Respiratory Medicine 2014;108(10):1460‐8. [CFGD Register: PE216a] - PubMed
Lowman 2012 {published data only}
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NCT02277860 {published data only}
    1. NCT02277860. Gaming console home‐based exercise for adults with cystic fibrosis. clinicaltrials.gov/ct2/show/NCT02277860 (first received 27 October 2014).
NCT02715921 {published data only}
    1. NCT02715921. Impact of telerehabilitation training on pediatric cystic fibrosis patients: an exploratory study. clinicaltrials.gov/ct2/show/NCT02715921 (first received 12 January 2016).
NCT02821130 {published data only}
    1. NCT02821130. Orkambi exercise study (Orkambi) [Effects of Orkambi on exertional dyspnea, exercise performance, and ventilatory responses in adults with cystic fibrosis]. clinicaltrials.gov/ct2/show/NCT02821130 (first received 06 June 2016).
NCT02875366 {published data only}
    1. NCT02875366. A study of the effects of lumacaftor/ivacaftor on exercise tolerance in subjects with cystic fibrosis, homozygous for the F508del‐CFTR mutation [A phase 4, randomized, double‐blind, placebo‐controlled, parallel‐design study of the effect of lumacaftor/ivacaftor combination therapy on exercise tolerance in subjects aged 12 years and older with cystic fibrosis, homozygous for the F508del‐CFTR mutation]. clinicaltrials.gov/ct2/show/NCT02875366 (first received 15 August 2016).
NCT03117764 {published data only}
    1. NCT03117764. Evaluation of the impact of intravenous antibiotics on muscular strength in patients with cystic fibrosis. clinicaltrials.gov/ct2/show/NCT03117764 (first received 31 March 2017).
Orenstein 1981 {published data only}
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Orenstein 2004 {published data only}
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Ozaydin 2010 {published data only}
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Patterson 2004 {published data only}
    1. Patterson JE, Bradley JM. Inspiratory muscle training in adult patients with cystic fibrosis: a randomised controlled trial to evaluate the efficacy of the test of incremental respiratory endurance (TIRE). Thorax 2004;59(Suppl II):ii13. [Abstract no: S36; CENTRAL: 518434; CFGD Register: PE236; CRS: 5500050000000533]
Petrovic 2013 {published data only}
    1. 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. [CENTRAL: 875002; CFGD Register: PE204; CRS: 5500100000011658]
Rand 2012 {published data only}
    1. Rand S, Hill L, Prasad SA, Main E. Development of an incremental field exercise test for children with cystic fibrosis [abstract]. Journal of Cystic Fibrosis 2012;11 Suppl 1:S36, Abstract no: WS16.7. [CFGD Register: PE193]
Reix 2012 {published data only}
    1. Reix P, Aubert F, Kassai B, Bige V, Bellon G. Better satisfaction of cystic fibrosis paediatric patients with autogenic drainage associated to exercise compared to conventional chest physiotherapy [abstract]. Journal of Cystic Fibrosis 2009;8 suppl 2:S73, Abstract no: 293. [CFGD Register: PE183a]
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Salonini 2015 {published data only}
    1. Salonini E, Gambazza S, Meneghelli I, Tridello G, Sanguanini M, Cazzarolli C, et al. Active video game playing in children and adolescents with cystic fibrosis: Exercise or just fun?. Respiratory Care 2015;60(8):1172‐9. [CENTRAL: 1161387; CFGD Register: PE207b; CRS: 5500050000000473; EMBASE: 2015465751] - PubMed
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Shaw 2016 {published data only}
    1. Shaw I, Kinsey JE, Richards R, Shaw BS. Individualized supervised resistance training during nebulization in adults with cystic fibrosis. Pakistan Journal of Medical Sciences 2016;32(5):1152‐7. [CENTRAL: 1343881; CFGD Register: PE242; CRS: 5500135000001737; PUBMED: 27882012] - PMC - PubMed
Stanghelle 1998 {published data only}
    1. Stanghelle JK, Hjeltnes N, Bangstad HJ, Michalsen H. Effect of daily short bouts of trampoline exercise during 8 weeks on the pulmonary function and the maximal oxygen uptake of children with cystic fibrosis. International Journal of Sports Medicine 1988;9 Suppl 1:32‐6. [CENTRAL: 568629; CFGD Register: PE155; CRS: 5500100000002860; PUBMED: 3360541] - PubMed
Tuzin 1998 {published data only}
    1. 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.
Vallier 2016 {published data only}
    1. Vallier JM, Rouissi M, Mely L, Gruet M. Physiological Responses of the Modified Shuttle Test in Adults With Cystic Fibrosis. Journal of Cardiopulmonary Rehabilitation and Prevention 2016;36(4):288‐92. [CENTRAL: 1343882; CFGD Register: PE241; CRS: 5500135000001903; PUBMED: 27182761] - PubMed
Vivodtzev 2013 {published data only}
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References to studies awaiting assessment

ACTRN12617001009303 {published data only}
    1. ACTRN12617001009303. Action: PACT. Be Active. Online. A trial to promote physical activity in young people with cystic fibrosis.. www.anzctr.org.au/Trial/Registration/TrialReview.aspx?ACTRN=12617001009303 (first received 06 July 2017).
Almajan 2011 {published data only}
    1. Almajan‐Guta B, Avram C, Almajan‐Guta V, Rusu A, Ciuca I, Cluci O, et al. High motivation for playing sports in cystic fibrosis ‐ what we play is life [abstract]. Journal of Cystic Fibrosis 2011;10 Suppl 1:S64, Abstract no: 254. [CFGD Register: PE189]
Housinger 2015 {published data only}
    1. Housinger E, Sullivan M, Ruiz FE. Effects of an interdisciplinary motivational incentive‐based walking program in pediatric pulmonary patients during hospitalization. Pediatric Pulmonology 2015;50 Suppl 41:369. [Abstract no: 471; CENTRAL: 1092214; CFGD Register: PE222; CRS: 5500135000001403; EMBASE: 72081750]
Johnston 2004 {published data only}
    1. Johnston K, Jenkins, Roberts C, Stick S. Improved attitude to exercise in overweight children with lung conditions after an exercise intervention. Respirology (Carlton, Vic.) 2004;9(2 Suppl):A60. [CENTRAL: 475664; CFGD Register: PE231; CRS: 5500050000000476]
Lorenc 2015 {published data only}
    1. Carr SB, Ronan P, Robinson N, Agent P, Mian A, Madge S. A randomised controlled trial of Tai Chi in cystic fibrosis. Pediatric Pulmonology 2016;51:376. [Abstract no: 480; CENTRAL: 1212611; CFGD Register: PE243c; CRS: 5500135000001919; EMBASE: 612358588]
    1. Lorenc A, Ronan P, Mian A, Madge S, Carr SB, Agent P, et al. Cystic fibrosis‐Children and adults Tai Chi study (CF CATS2): Can Tai Chi improve symptoms and quality of life for people with cystic fibrosis? Second phase study protocol. Chinese Journal of Integrative Medicine. China, 2015 May 26 [Epub ahead of print]. [CENTRAL: 1343883; CFGD Register: PE243a; CRS: 5500135000001904; PUBMED: 26015075] - PubMed
    1. Madge S, Ronan P, Robinson N, Agent P, Mian A, Carr SB. Assessing the benefits of tai chi in people with CF and the feasibility of delivering Tai Chi over the internet (Skype®). Pediatric Pulmonology 2016;51 Suppl 45:370. [Abstract no: 465; CENTRAL: 1343884; CFGD Register: PE243b; CRS: 5500135000001922]
Mandrusiak 2011 {published data only}
    1. Mandrusiak A, MacDonald J, Paratz J, Wilson C, Watter P. A novel exercise program for young people with cystic fibrosis: Moving physiotherapy forward through targeted design. Physiotherapy (United Kingdom) 2011;97:eS1550. [CENTRAL: 1076180; CFGD Register: PE229; CRS: 5500050000000415; EMBASE: 71884245; Poster no.: SI‐PO‐210‐22‐Thu]
NCT00609050 {published data only}
    1. NCT00609050. Exercise training study for patients with cystic fibrosis [Self‐regulated exercise in CF: a randomized trial]. clinicaltrials.gov/ct2/show/NCT00609050 (first received 31 January 2008).
NCT00792194 {published data only}
    1. NCT00792194. Improvement of aerobic capacity in cystic fibrosis patients with a one‐year home training period. clinicaltrials.gov/ct2/show/NCT00792194 (first received 14 November 2008).
NCT02552043 {published data only}
    1. NCT02552043. Video game exercise effectiveness of a domiciliary pulmonary rehabilitation program in cystic fibrosis patients. clinicaltrials.gov/ct2/show/NCT02552043 (first received 15 September 2015).
NCT03100214 {published data only}
    1. NCT03100214. Effects of an early rehabilitation program during hospitalization in patients with cystic fibrosis. clinicaltrials.gov/ct2/show/NCT03100214 (first received 31 January 2017).
NCT03109912 {published data only}
    1. NCT03109912. Do More, B'More, Live Fit [Do More, B'More, Live Fit: an outpatient fitness‐training pilot program designed to optimize the habit of exercise in adolescents and young adults with cystic fibrosis]. clinicaltrials.gov/ct2/show/NCT03109912 (first received 22 February 2017).
Oliveira 2010 {published data only}
    1. Oliveira ACN, Jesus TA, Mesquita‐ Ferrari RA, Oliveira JC, Oliveira LVF, Sampaio LM. Aerobic training and quality of life in cystic fibrosis. European Respiratory Journal 2010;36 Suppl 54:P2651. [CFGD Register: PE245; CRS: 5500135000001906]
Phillips 2008 {published data only}
    1. Phillips AL, Lee L, Britton LJ, Hoover W, Lowman JD. Efficacy of a standardised exercise protocol in inpatient care of patients with cystic fibrosis [abstract]. Pediatric Pulmonology 2008;43(Suppl 31):385, Abstract 510. [CFGD Register: PE182]

References to ongoing studies

Donadio 2017 {published data only}
    1. Donadio M. Effect of exercise orientations in the posture and plantar pressure distribution in children and adolescents with cystic fibrosis. apps.who.int/trialsearch/Trial2.aspx?TrialID=RBR‐3r4h5s (first received 09 July 2014).
Gupta 2017 {published data only}
    1. Gupta S. Effects of exercise intervention program on bone mineral accretion in children and adolescents with cystic fibrosis. apps.who.int/trialsearch/Trial2.aspx?TrialID=CTRI/2013/04/003531 (first received 04 April 2013). - PubMed
Hebestreit 2016 {published data only}
    1. Hebestreit H. How to counsel patients regarding exercise and habitual physical activity?. Journal of Cystic Fibrosis 2016;51 Suppl 45:188. [Abstract no: S20.4; CFGD Register: PE244a; CRS: 5500135000001920]
    1. NCT01744561. Effects of a partially supervised conditioning program in CF (ACTIVATE‐CF) [Effects of a partially supervised conditioning program in CF: an international multi‐centre, randomized controlled trial]. clinicaltrials.gov/show/NCT01744561 (first received 05 December 2012). [CENTRAL: 1343886; CFGD Register: PE244b; CRS: 5500135000001724]
NCT02700243 {published data only}
    1. NCT02700243. Increase tolerance for exercise and raise activity through connectedness trial (INTERACT) [Pilot RCT study using a Fitbit device to improve exercise tolerance in 80 patients with cystic fibrosis]. clinicaltrials.gov/ct2/show/NCT02700243 (first received 21 February 2016).

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