https://doi.org/10.4081/monaldi.2021.1501
Pulmonary rehabilitation for cystic fibrosis: A narrative review of current literature
Submitted: July 11, 2020
Accepted: December 22, 2020
Published: March 11, 2021
Accepted: December 22, 2020
Abstract Views: 4338
PDF: 2340
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Authors
Pulmonary rehabilitation is a key component in cystic fibrosis care. This review summarizes the recent evidence in the area of pulmonary rehabilitation for cystic fibrosis in the form of questions and answers regarding interventions, indications, benefits and risks of pulmonary rehabilitation. Pulmonary rehabilitation includes airway clearance techniques, exercise training, education and behaviour change and can improve patients’ exercise capacity, muscle strength, quality of life and nutritional status. Airway clearance techniques have beneficial effects for clearing mucous. Over the past years, evidence for the beneficial effects of exercise training on exercise capacity and overall lung health is growing. In cystic fibrosis, multiple factors result in reduced exercise capacity. All modalities of pulmonary rehabilitation should be offered to patients with cystic fibrosis, as the benefits in most cases outweigh the risks, though the optimal regimens need to be yet defined.
Hollin IL, Robinson KA. A Scoping review of healthcare costs for patients with cystic fibrosis. Appl Health Econ Health Policy 2016;14:151-9.
DOI: https://doi.org/10.1007/s40258-015-0211-4
Davies JC, Alton EW, Bush A. Cystic fibrosis. BMJ 2007;335:1255-9.
DOI: https://doi.org/10.1136/bmj.39391.713229.AD
Spruit MA. Pulmonary rehabilitation. Eur Respir Rev 2014;23:55-63.
DOI: https://doi.org/10.1183/09059180.00008013
Spinou A. Physiotherapy in cystic fibrosis Α comprehensive clinical overview. Pneumon 2018;31:35-43.5.
PRISMA. Transparent reporting of systematic reviews and meta-analyses. Available from: http://www.prisma-statement.org/
McIlwaine M, Bradley J, Elborn JS, Moran F. Personalising airway clearance in chronic lung disease. Eur Respir Rev 2017;26:160086.
DOI: https://doi.org/10.1183/16000617.0086-2016
Button BM, Wilson C, Dentice R, et al. Physiotherapy for cystic fibrosis in Australia and New Zealand: A clinical practice guideline. Respirology 2016;21:656-67.
DOI: https://doi.org/10.1111/resp.12764
Bradley JM, Moran FM, Elborn JS. Evidence for physical therapies (airway clearance and physical training) in cystic fibrosis: an overview of five Cochrane systematic reviews. Respir Med 2006;100:191-201.
DOI: https://doi.org/10.1016/j.rmed.2005.11.028
Dwyer TJ, Alison JA, McKeough ZJ, et al. Effects of exercise on respiratory flow and sputum properties in patients with cystic fibrosis. Chest 2011;139:870-7.
DOI: https://doi.org/10.1378/chest.10-1158
Sharma BB, Singh V. Pulmonary rehabilitation: An overview. Lung India 2011;28:276-84.
DOI: https://doi.org/10.4103/0970-2113.85690
Bolton CE, Bevan-Smith EF, Blakey JD, et al. British Thoracic Society guideline on pulmonary rehabilitation in adults. Thorax 2013;68:ii1-30.
DOI: https://doi.org/10.1136/thoraxjnl-2013-203808
Haskell WL, Lee IM, Pate RR, et al. Physical activity and public health: updated recommendation for adults from the American College of Sports Medicine and the American Heart Association. Med Sci Sports Exerc 2007;39:1423-34.
DOI: https://doi.org/10.1249/mss.0b013e3180616b27
Schneiderman-Walker J, Pollock SL, Corey M, et al. A randomized controlled trial of a 3-year home exercise program in cystic fibrosis. J Pediatr 2000;136:304-10.
DOI: https://doi.org/10.1067/mpd.2000.103408
Rovedder PM, Flores J, Ziegler B, et al. Exercise programme in patients with cystic fibrosis: a randomized controlled trial. Respir Med 2014;108:1134-40.
DOI: https://doi.org/10.1016/j.rmed.2014.04.022
Happ MB, Hoffman LA, Higgins LW, et al. Parent and child perceptions of a self-regulated, home-based exercise program for children with cystic fibrosis. Nurs Res 2013;62:305-14. Erratum in: Nurs Res 2014;63:25. DiVirgilio, Dana [corrected to Divirgilio, Dana].
DOI: https://doi.org/10.1097/NNR.0b013e3182a03503
Kriemler S, Kieser S, Junge S, et al. Effect of supervised training on FEV1 in cystic fibrosis: a randomised controlled trial. J Cyst Fibros 2013;12:714-20.
Selvadurai HC, Blimkie CJ, Meyers N, et al. Randomized controlled study of in-hospital exercise training programs in children with cystic fibrosis. Pediatr Pulmonol 2002;33:194-200.
DOI: https://doi.org/10.1002/ppul.10015
Gruber W, Orenstein DM, Braumann KM, Beneke R. Interval exercise training in cystic fibrosis -- effects on exercise capacity in severely affected adults. J Cyst Fibros 2014;13:86-91.
DOI: https://doi.org/10.1016/j.jcf.2013.06.005
Hebestreit H, Kieser S, Junge S, et al. Long-term effects of a partially supervised conditioning programme in cystic fibrosis. Eur Respir J 2010;35:578-83.
Santana-Sosa E, Gonzalez-Saiz L, Groeneveld IF, et al. Benefits of combining inspiratory muscle with 'whole muscle' training in children with cystic fibrosis: a randomised controlled trial. Br J Sports Med 2014;48:1513-7.
DOI: https://doi.org/10.1136/bjsports-2012-091892
Sosa ES, Groeneveld IF, Gonzalez-Saiz L, et al. Intrahospital weight and aerobic training in children with cystic fibrosis: A randomized controlled trial. Med Sci Sports Exerc 2012;44:2–11.
DOI: https://doi.org/10.1249/MSS.0b013e318228c302
Wheatley CM, Baker SE, Morgan MA, et al. Effects of exercise intensity compared to albuterol in individuals with cystic fibrosis. Respir Med 2015;109:463-74.
DOI: https://doi.org/10.1016/j.rmed.2014.12.002
Del Corral T, Cebrià I Iranzo MÀ, et al. Effectiveness of a home-based active video game programme in young cystic fibrosis patients. Respiration 2018;95:87-97.
DOI: https://doi.org/10.1159/000481264
Bishay LC, Nelson E, Williams K, et al. Effect of a wearable fitness tracker on exercise tolerance for adults with cystic fibrosis: a pilot randomized clinical trial. Pediatr Pulmonol 2018;53:345. Added to CENTRAL: 30 November 2018 | 2018 Issue 11.
Holmes H, Wood J, Jenkins S, et al. Xbox Kinect™ represents high intensity exercise for adults with cystic fibrosis. J Cyst Fibros 2013;12:604-8.
DOI: https://doi.org/10.1016/j.jcf.2013.05.005
Salonini E, Gambazza S, Meneghelli I, et al. Active video game playing in children and adolescents with cystic fibrosis: Exercise or just fun? Respir Care 2015;60:1172-9.
DOI: https://doi.org/10.4187/respcare.03576
O'Donovan C, Greally P, Canny G, et al. Active video games as an exercise tool for children with cystic fibrosis. J Cyst Fibros 2014;13:341-6.
DOI: https://doi.org/10.1016/j.jcf.2013.10.008
Smith A, Gouick L. WS05.2 30 day challenge – using social media to support adult CF patients to exercise in the adult CF service Dundee. J Cyst Fibros 2015;14;S9.
DOI: https://doi.org/10.1016/S1569-1993(15)30028-X
Cox NS, Alison JA, Button BM, et al. Feasibility and acceptability of an internet-based program to promote physical activity in adults with cystic fibrosis. Respir Care 2015;60:422-9.
DOI: https://doi.org/10.4187/respcare.03165
Cox NS, Eldridge B, Rawlings S, et al. A web-based intervention to promote physical activity in adolescents and young adults with cystic fibrosis: protocol for a randomized controlled trial. BMC Pulm Med 2019;19:253.
DOI: https://doi.org/10.1186/s12890-019-0942-3
Lang RL, Wilson C, Stockton K, et al. CyFiT telehealth: protocol for a randomised controlled trial of an online outpatient physiotherapy service for children with cystic fibrosis. BMC Pulm Med 2019;19:21.
DOI: https://doi.org/10.1186/s12890-019-0784-z
Hebestreit H, Kriemler S, Radtke T. Exercise for all cystic fibrosis patients: is the evidence strengthening? Curr Opin Pulm Med 2015;21:591-5.
DOI: https://doi.org/10.1097/MCP.0000000000000214
National Guideline Alliance (UK). Cystic Fibrosis: Diagnosis and management. London: National Institute for Health and Care Excellence (UK); 2017 Oct 25.
Flume PA, Mogayzel PJ Jr, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: pulmonary complications: hemoptysis and pneumothorax. Am J Respir Crit Care Med 2010;182:298-306.
DOI: https://doi.org/10.1164/rccm.201002-0157OC
Moran A, Brunzell C, Cohen RC, et al. Clinical care guidelines for cystic fibrosis-related diabetes: a position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care 2010;33:2697-708.
DOI: https://doi.org/10.2337/dc10-1768
Johannesson M. Effects of pregnancy on health: certain aspects of importance for women with cystic fibrosis. J Cyst Fibros 2002;1:9-12.
DOI: https://doi.org/10.1016/S1569-1993(01)00005-4
Burtin C, Hebestreit H. Rehabilitation in patients with chronic respiratory disease other than chronic obstructive pulmonary disease: exercise and physical activity interventions in cystic fibrosis and non-cystic fibrosis bronchiectasis. Respiration 2015;89:181-9.
Bott J, Blumenthal S, Buxton M, et al. Guidelines for the physiotherapy management of the adult, medical, spontaneously breathing patient. Thorax 2009;64:i1-51.
DOI: https://doi.org/10.1136/thx.2008.110726
Warnock L, Gates A. Chest physiotherapy compared to no chest physiotherapy for cystic fibrosis. Cochrane Database Syst Rev 2015;2015:CD001401.
DOI: https://doi.org/10.1002/14651858.CD001401.pub3
Main E, Prasad A, Schans C. Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis. Cochrane Database Syst Rev 2005:CD002011.
DOI: https://doi.org/10.1002/14651858.CD002011.pub2
Morrison L, Agnew J. Oscillating devices for airway clearance in people with cystic fibrosis. Cochrane Database Syst Rev 2014:CD006842.
DOI: https://doi.org/10.1002/14651858.CD006842.pub3
Elkins MR, Jones A, van der Schans C. Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis. Cochrane Database Syst Rev 2006:CD003147.
DOI: https://doi.org/10.1002/14651858.CD003147.pub3
Mckoy NA, Wilson LM, Saldanha IJ, et al. Active cycle of breathing technique for cystic fibrosis. Cochrane Database Syst Rev 2016;7:CD007862.
DOI: https://doi.org/10.1002/14651858.CD007862.pub4
Bradley J, Moran F. Physical training for cystic fibrosis. Cochrane Database Syst Rev 2008;1:CD002768.
Elbasan B, Tunali N, Duzgun I, Ozcelik U. Effects of chest physiotherapy and aerobic exercise training on physical fitness in young children with cystic fibrosis. Ital J Pediatr 2012;38:2.
DOI: https://doi.org/10.1186/1824-7288-38-2
Hulzebos HJ, Snieder H, van der Et J, et al. High-intensity interval training in an adolescent with cystic fibrosis: a physiological perspective. Physiother Theory Pract 2011;27:231-7.
DOI: https://doi.org/10.3109/09593985.2010.483266
Shoemaker MJ, Hurt H, Arndt L. The evidence regarding exercise training in the management of cystic fibrosis: a systematic review. Cardiopulm Phys Ther J 2008;19:75-83.
DOI: https://doi.org/10.1097/01823246-200819030-00002
Tejero García S, Giráldez Sánchez MA, Cejudo P, et al. Bone health, daily physical activity, and exercise tolerance in patients with cystic fibrosis. Chest 2011;140:475-81.
DOI: https://doi.org/10.1378/chest.10-1508
Paranjape SM, Barnes LA, Carson KA, et al. Exercise improves lung function and habitual activity in children with cystic fibrosis. J Cyst Fibros 2012;11:18-23.
DOI: https://doi.org/10.1016/j.jcf.2011.08.003
Klijn PH, Oudshoorn A, van der Ent CK, et al. Effects of anaerobic training in children with cystic fibrosis: a randomized controlled study. Chest 2004;125:1299-305.
DOI: https://doi.org/10.1378/chest.125.4.1299
Schmidt AM, Jacobsen U, Bregnballe V, et al. Exercise and quality of life in patients with cystic fibrosis: A 12-week intervention study. Physiother Theory Pract 2011;27:548-56.
DOI: https://doi.org/10.3109/09593985.2010.545102
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;59:1074-80.
DOI: https://doi.org/10.1136/thx.2003.015313
Bradley J, Moran F. Physical training for cystic fibrosis. Cochrane Database Syst Rev 2008;1:CD002768.
DOI: https://doi.org/10.1002/14651858.CD002768.pub2
Schneiderman JE, Wilkes DL, Atenafu EG, et al. Longitudinal relationship between physical activity and lung health in patients with cystic fibrosis. Eur Respir J 2014;43:817-23.
DOI: https://doi.org/10.1183/09031936.00055513
Hebestreit H, Kieser S, Junge S, et al. Long-term effects of a partially supervised conditioning programme in cystic fibrosis. Eur Respir J 2010;35:578-83.
DOI: https://doi.org/10.1183/09031936.00062409
Hebestreit H, Hulzebos EHJ, Schneiderman JE, et al. Cardiopulmonary exercise testing provides additional prognostic information in cystic fibrosis. Am J Respir Crit Care Med 2019;199:987-95.. Erratum in: Am J Respir Crit Care Med 2020;202:780.
Hebestreit H, Schmid K, Kieser S, et al. Quality of life is associated with physical activity and fitness in cystic fibrosis. BMC Pulm Med 2014;14:26.
DOI: https://doi.org/10.1186/1471-2466-14-26
Urquhart D, Sell Z, Dhouieb E, et al. Effects of a supervised, outpatient exercise and physiotherapy programme in children with cystic fibrosis. Pediatr Pulmonol 2012;47:1235-41.
DOI: https://doi.org/10.1002/ppul.22587
Pérez M, Groeneveld IF, Santana-Sosa E, et al. Aerobic fitness is associated with lower risk of hospitalization in children with cystic fibrosis. Pediatr Pulmonol 2014;49:641-9.
DOI: https://doi.org/10.1002/ppul.22878
Schindel CS, Hommerding PX, Melo DA, et al. Physical exercise recommendations improve postural changes found in children and adolescents with cystic fibrosis: a randomized controlled trial. J Pediatr 2015;166:710-6.e2.
DOI: https://doi.org/10.1016/j.jpeds.2014.12.001
Burtin C, Hebestreit H. Rehabilitation in patients with chronic respiratory disease other than chronic obstructive pulmonary disease: exercise and physical activity interventions in cystic fibrosis and non-cystic fibrosis bronchiectasis. Respiration 2015;89:181-9.
DOI: https://doi.org/10.1159/000375170
Lebecque P, Lapierre JG, Lamarre A, Coates AL. Diffusion capacity and oxygen desaturation effects on exercise in patients with cystic fibrosis. Chest 1987;91:693-7.
DOI: https://doi.org/10.1378/chest.91.5.693
Albert RK, Schuller JL, COPD Clinical Research Network. Macrolide antibiotics and the risk of cardiac arrhythmias. Am J Respir Crit Care Med 2014;189:1173-80.
DOI: https://doi.org/10.1164/rccm.201402-0385CI
Radtke T, Nevitt SJ, Hebestreit H, Kriemler S. Physical exercise training for cystic fibrosis. Cochrane Database Syst Rev 2017;11:CD002768.
DOI: https://doi.org/10.1002/14651858.CD002768.pub4
Kriemler S, Kieser S, Junge S, et al. Effect of supervised training on FEV1 in cystic fibrosis: a randomised controlled trial. J Cyst Fibros 2013;12:714-20.
DOI: https://doi.org/10.1016/j.jcf.2013.03.003
How to Cite
Kalamara, Evgenia I., Evangelos T. Ballas, Georgia Pitsiou, and Guergana Petrova. 2021. “Pulmonary Rehabilitation for Cystic Fibrosis: A Narrative Review of Current Literature”. Monaldi Archives for Chest Disease 91 (2). https://doi.org/10.4081/monaldi.2021.1501.
Copyright (c) 2021 The Author(s)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
PAGEPress has chosen to apply the Creative Commons Attribution NonCommercial 4.0 International License (CC BY-NC 4.0) to all manuscripts to be published.
Similar Articles
- E. Clini, S. Costi, M. Romagnoli, F. Florini, Rehabilitation of COPD patients: which training modality , Monaldi Archives for Chest Disease: Vol. 61 No. 3 (2004): Pulmonary series
- Francesco Giallauria, Rosa Lucci, Francesco Pilerci, Anna De Lorenzo, Athanasio Manakos, Marianna Psaroudaki, Mariantonietta D’Agostino, Alessandra Vitelli, Luigi Maresca, Domenico Del Forno, Carlo Vigorito, Efficacy of Telecardiology in improving the results of Cardiac Rehabilitation after acute myocardial infarction , Monaldi Archives for Chest Disease: Vol. 66 No. 1 (2006): Cardiac series
- E.M. Clini, P. Siddu, L. Trianni, R. Graziosi, E. Crisafulli, M.T. Nobile, Activity and analysis of costs in a dedicated weaning centre , Monaldi Archives for Chest Disease: Vol. 69 No. 2 (2008): Pulmonary series
- G.L. Casoni, C. Gurioli, P.N. Chhajed, M. Chilosi, M. Zompatori, D. Olivieri, V. Poletti, The value of transbronchial lung biopsy using jumbo forceps via rigid bronchoscope in diffuse lung disease , Monaldi Archives for Chest Disease: Vol. 69 No. 2 (2008): Pulmonary series
- Claudio F. Donner, Sandro Amaducci, Elena Bacci, Sandra Baldacci, Maria L. Bartoli, Gianfranco M. Beghi, Alida Benfante, Sara Brighindi, Lucio Casali, Daniela Castiglia, Mario Cazzola, Alessandro Celi, Silvana Cianchetti, Giorgio Colombo, Claudia Crimi, Federico L. Dente, Giuseppe Di Maria, Annalisa Di Maria, Manuela Latorre, Federico Lavorini, Sara Maio, Claudia Mannini, Riccardo Messina, Pier Luigi Paggiaro, Patrizia Pignatti, David Price, Nicola Scichilone, Marzia Simoni, Antonio Spanevello, Martina Stagno d’Alcontres, Shawna Tan, Roberto Torchio, Giovanni Viegi, Dina Visca, Emiel F.M. Wouters, Shaylynn Yu Hui Xin, Inhalation therapy in the next decade: Determinants of adherence to treatment in asthma and COPD , Monaldi Archives for Chest Disease: Vol. 88 No. 1 (2018)
- E. Clini, F. Prato, M. Nobile, M. Bondi, B. Serri, C. Cilione, D. Lugli, Interdisciplinary rehabilitation in morbidly obese subjects: an observational pilot study , Monaldi Archives for Chest Disease: Vol. 65 No. 2 (2006): Pulmonary series
- Francesco Giallauria, Marco Gentile, Paolo Chiodini, Franco Berrino, Amalia Mattiello, Luigi Maresca, Alessandra Vitelli, Maria Mancini, Alessandra Grieco, Angelo Russo, Rosa Lucci, Giorgio Torella, Salvatore Panico, Carlo Vigorito, Exercise training reduces high mobility group box-1 protein levels in women with breast cancer: findings from the DIANA-5 study , Monaldi Archives for Chest Disease: Vol. 82 No. 2 (2014): Cardiac series
- Marco Ambrosetti, Simona Sarzi Braga, Franco Giada, Roberto F.E. Pedretti, Exercise-based cardiac rehabilitation in cardiac resynchronization therapy recipients: A primer for practicing clinicians , Monaldi Archives for Chest Disease: Vol. 87 No. 3 (2017)
- Abhijeet Singh, Rajendra Prasad, Rajiv Garg, Surya Kant, Giridhar B. Hosmane, Abhisek Dubey, Abhisek Agarwal, Ram Kishun Verma, A study to estimate prevalence and risk factors of Obstructive Sleep Apnoea Syndrome in a semi-urban Indian population , Monaldi Archives for Chest Disease: Vol. 87 No. 1 (2017)
- Michele Vitacca, Marta Lazzeri, Enrico Guffanti, Pamela Frigerio, Francesco D’Abrosca, Silvia Gianola, Mauro Carone, Mara Paneroni, Piero Ceriana, Franco Pasqua, Paolo Banfi, Francesco Gigliotti, Carla Simonelli, Serena Cirio, Veronica Rossi, Chiara G. Beccaluva, Mariangela Retucci, Martina Santambrogio, Andrea Lanza, Francesca Gallo, Alessia Fumagalli, Marco Mantero, Greta Castellini, Mariaconsiglia Calabrese, Giorgio Castellana, Eleonora Volpato, Marina Ciriello, Marina Garofano, Enrico Clini, Nicolino Ambrosino, on behalf of AIPO, ARIR, SIP, AIFI and SIFIR, An Italian consensus on pulmonary rehabilitation in COVID-19 patients recovering from acute respiratory failure: results of a Delphi process , Monaldi Archives for Chest Disease: Vol. 90 No. 2 (2020)
<< < 1 2 3 4 5 6 7 8 9 10 > >>
You may also start an advanced similarity search for this article.
