Pulmonary rehabilitation for cystic fibrosis: A narrative review of current literature

https://doi.org/10.4081/monaldi.2021.1501

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Abstract

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.

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References

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

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Published
2021-03-11
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Physiotherapy - Reviews
Keywords:
Pulmonary rehabilitation, cystic fibrosis, exercise capacity, lung function, quality of life
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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.

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