Cystic Fibrosis

  • Thomas Radtke
  • Susi Kriemler
  • Helge Hebestreit


Pulmonary rehabilitation in cystic fibrosis (CF) utilizes the same components as in other respiratory conditions. However, there are some specifics that should be beard in mind. For one, CF is a multi-organ disease with quite variable clinical characteristics and disease burden among individuals. Thus, pulmonary rehabilitation cannot focus solely on the lungs but has to take upper airways, nutritional aspects, the cardiovascular system, liver disease, and the musculoskeletal system into account. Furthermore, people with CF may host various pathogens, which may be (directly or indirectly) transmitted from one person to the other. Infection control measures are, thus, of utmost importance and often interfere with traditional rehabilitation settings such as group sessions. The chapter will focus on aspects of pulmonary rehabilitation which are particularly important in CF. For general information on components of pulmonary rehabilitation, we refer to the respective chapters at the beginning of this book.


  1. 1.
    Saiman L. Update on infection prevention and control guidelines. Pediatr Pulmonol. 2013;48(Suppl 36):185–7.Google Scholar
  2. 2.
    Saiman L, Siegel JD, LiPuma JJ, Brown RF, Bryson EA, Chambers MJ, Downer VS, Fliege J, Hazle LA, Jain M, Marshall BC, O’Malley C, Pattee SR, Potter-Bynoe G, Reid S, Robinson KA, Sabadosa KA,Schmidt HJ, Tullis E, Webber J, Weber DJ. Infection prevention and control guideline for cystic fibrosis: 2013 update. Infect Control Hosp Epidemiol. 2014;35(S1):S1–S67Google Scholar
  3. 3.
    Pegues DA, Carson LA, Tablan OC, Fitzsimmons SC, Roman SB, Miller JM, Jarvis WR, Spohn W, Diakew D, Mccoy K, Johnson T, Wilmott RW, Kociela VL, Bivens K, Kanga JF, Christenson J, Woods C, Reisman J, Ciccaletaylor L, Wilson WM, Hennessey R, Eccelstone ER, Hunter E, Keely K. Acquisition of pseudomonas-cepacia at summer camps for patients with cystic-fibrosis. J Pediatr. 1994;124:694–702.CrossRefPubMedGoogle Scholar
  4. 4.
    Tummler B, Koopmann U, Grothues D, Weissbrodt H, Steinkamp G, Vonderhardt H. Nosocomial acquisition of pseudomonas-aeruginosa by cystic-fibrosis patients. J Clin Microbiol. 1991;29:1265–7.Google Scholar
  5. 5.
    Ridderberg W, Andersen C, Vaeth M, Bregnballe V, Norskov-Lauritsen N, Schiotz PO. Lack of evidence of increased risk of bacterial transmission during cystic fibrosis educational programmes. J Cyst Fibros. 2016;15:109–15.CrossRefPubMedGoogle Scholar
  6. 6.
    Christian BJ, D'auria JP. Building life skills for children with cystic fibrosis - Effectiveness of an intervention. Nurs Res. 2006;55:300–7.CrossRefPubMedGoogle Scholar
  7. 7.
    Greenberg D, Yagupsky P, Peled N, Goldbart A, Porat N, Tal A. Lack of evidence of transmission of Pseudomonas aeruginosa among cystic fibrosis patients attending health camps at the Dead Sea, Israel. Isr Med Assoc J. 2004;6:531–4.PubMedGoogle Scholar
  8. 8.
    Griese M, Busch P, Caroli D, Mertens B, Eismann C, Harari M, Staudter H, Kappler M. Rehabilitation programs for cystic fibrosis—view from a CF center. Open Respir Med J. 2010;4:1–8.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Steinkamp G, Wiedemann B. Relationship between nutritional status and lung function in cystic fibrosis: cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project. Thorax. 2002;57:596–601.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Klijn PH, Van Der Net J, Kimpen JL, Helders PJ, Van Der Ent CK. Longitudinal determinants of peak aerobic performance in children with cystic fibrosis. Chest. 2003;124:2215–9.CrossRefPubMedGoogle Scholar
  11. 11.
    Hanna RM, Weiner DJ. Overweight and obesity in patients with cystic fibrosis: a center-based analysis. Pediatr Pulmonol. 2015;50:35–41.CrossRefPubMedGoogle Scholar
  12. 12.
    Stephenson AL, Mannik LA, Walsh S, Brotherwood M, Robert R, Darling PB, Nisenbaum R, Moerman J, Stanojevic S. Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: a population-based cohort study. Am J Clin Nutr. 2013;97:872–7.CrossRefPubMedGoogle Scholar
  13. 13.
    Mckoy NA, Wilson LM, Saldanha IJ, Odelola OA, Robinson KA. Active cycle of breathing technique for cystic fibrosis. Cochrane Database Syst Rev. 2016;7:CD007862.Google Scholar
  14. 14.
    McIlwaine M, Button B, Dwan K. Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis. Cochrane Database Syst Rev. 2015;6:CD003147.Google Scholar
  15. 15.
    Radtke T, Nolan SJ, Hebestreit H, Kriemler S. Physical exercise training for cystic fibrosis. Cochrane Database Syst Rev. 2015; doi:10.1002/14651858.CD002768.pub3CD002768.
  16. 16.
    Quittner AL, Barker DH, Snell C, Grimley ME, Marciel K, Cruz I. Prevalence and impact of depression in cystic fibrosis. Curr Opin Pulm Med. 2008;14:582–8.CrossRefPubMedGoogle Scholar
  17. 17.
    West CA, Besier T, Borth-Bruhns T, Goldbeck L. Effectiveness of a family-oriented rehabilitation program on the quality of life of parents of chronically ill children. Klin Padiatr. 2009;221:241–6.CrossRefPubMedGoogle Scholar
  18. 18.
    Hebestreit H, Schmid K, Kieser S, Junge S, Ballmann M, Roth K, Hebestreit A, Schenk T, Schindler C, Posselt H-G, Kriemler S. Quality of life is associated with physical activity and fitness in cystic fibrosis. BMC Pulm Med. 2014;14:26.Google Scholar
  19. 19.
    Tejero Garcia S, Giraldez Sanchez MA, Cejudo P, Quintana Gallego E, Dapena J, Garcia Jimenez R, Cano Luis P, Gomez De Terreros I. Bone health, daily physical activity, and exercise tolerance in patients with cystic fibrosis. Chest. 2011;140:475–81.CrossRefPubMedGoogle Scholar
  20. 20.
    Cox NS, Alison JA, Button BM, Wilson JW, Morton JM, Holland AE. Physical activity participation by adults with cystic fibrosis: an observational study. Respirology. 2016;21:511–8.Google Scholar
  21. 21.
    Schneiderman JE, Wilkes DL, Atenafu EG, Nguyen T, Wells GD, Alarie N, Tullis E, Lands LC, Coates AL, Corey M, Ratjen F. Longitudinal relationship between physical activity and lung health in patients with cystic fibrosis. Eur Respir J. 2014;43:817–23.CrossRefPubMedGoogle Scholar
  22. 22.
    Hebestreit H, Kieser S, Rudiger S, Schenk T, Junge S, Hebestreit A, Ballmann M, Posselt HG, Kriemler S. Physical activity is independently related to aerobic capacity in cystic fibrosis. Eur Respir J. 2006;28:734–9.CrossRefPubMedGoogle Scholar
  23. 23.
    Moorcroft AJ, Dodd ME, Webb AK. Exercise testing and prognosis in adult cystic fibrosis. Thorax. 1994;49:1075–1076P.Google Scholar
  24. 24.
    Nixon PA, Orenstein DM, Kelsey SF, Doershuk CF. The prognostic value of exercise testing in patients with cystic fibrosis. New Engl J Med. 1992;327:1785–8.CrossRefPubMedGoogle Scholar
  25. 25.
    Pianosi P, Leblanc J, Almudevar A. Peak oxygen uptake and mortality in children with cystic fibrosis. Thorax. 2005;60:50–4.CrossRefPubMedPubMedCentralGoogle Scholar
  26. 26.
    Hebestreit H, Arets HG, Aurora P, Boas S, Cerny F, Hulzebos EH, Karila C, Lands LC, Lowman JD, Swisher A, Urquhart DS, European Cystic Fibrosis Exercise Working Group. Statement on exercise testing in cystic fibrosis. Respiration. 2015;90:332–51.CrossRefPubMedGoogle Scholar
  27. 27.
    Godfrey S, Davies CT, Wozniak E, Barnes CA. Cardio-respiratory response to exercise in normal children. Clin Sci. 1971;40:419–31.CrossRefPubMedGoogle Scholar
  28. 28.
    Radtke T, Nolan SJ, Hebestreit H, Kriemler S. Physical exercise training for cystic fibrosis. Paediatr Respir Rev. 2016b;19:42–5.PubMedGoogle Scholar
  29. 29.
    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.CrossRefPubMedGoogle Scholar
  30. 30.
    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. Pediatr Pulmonol. 2002;33:194–200.CrossRefPubMedGoogle Scholar
  31. 31.
    Lands LC, Smountas AA, Mesiano G, Brosseau L, Shennib H, Charbonneau M, Gauthier R. Maximal exercise capacity and peripheral skeletal muscle function following lung transplantation. J Heart Lung Transplant. 1999;18:113–20.CrossRefPubMedGoogle Scholar
  32. 32.
    Langer D, Gosselink R, Pitta F, Burtin C, Verleden G, Dupont L, Decramer M, Troosters T. Physical activity in daily life 1 year after lung transplantation. J Heart Lung Transplant. 2009;28:572–8.CrossRefPubMedGoogle Scholar
  33. 33.
    Williams TJ, Mckenna MJ. Exercise limitation following transplantation. Compr Physiol. 2012;2:1937–79.PubMedGoogle Scholar
  34. 34.
    Radtke T, Benden C, Kriemler S. Physical activity and exercise training in lung transplant recipients with cystic fibrosis: ‘what we know, what we don’t know and where to go’. Lung. 2016a;194:177–8.CrossRefPubMedGoogle Scholar
  35. 35.
    Wickerson L, Mathur S, Brooks D. Exercise training after lung transplantation: a systematic review. J Heart Lung Transplant. 2010;29:497–503.CrossRefPubMedGoogle Scholar
  36. 36.
    Langer D, Burtin C, Schepers L, Ivanova A, Verleden G, Decramer M, Troosters T, Gosselink R. Exercise training after lung transplantation improves participation in daily activity: a randomized controlled trial. Am J Transplant. 2012;12:1584–92.CrossRefPubMedGoogle Scholar
  37. 37.
    Coppoolse R, Schols AMWJ, Baarends EM, Mostert R, Akkermans MA, Janssen PP, Wouters EFM. Interval versus continuous training in patients with severe COPD: a randomized clinical trial. Eur Respir J. 1999;14:258–63.CrossRefPubMedGoogle Scholar
  38. 38.
    Vogiatzis I, Nanas S, Roussos C. Interval training as an alternative modality to continuous exercise in patients with COPD. Eur Respir J. 2002;20:12–9.CrossRefPubMedGoogle Scholar
  39. 39.
    Vogiatzis I, Terzis G, Nanas S, Stratakos G, Simoes DCM, Georgiadou O, Zakynthinos S, Roussos C. Skeletal muscle adaptations to interval training in patients with advanced COPD. Chest. 2005;128:3838–45.CrossRefPubMedGoogle Scholar
  40. 40.
    Mathur S, Hornblower E, Levy RD. Exercise training before and after lung transplantation. Physician Sports Med. 2009;37:78–87.CrossRefGoogle Scholar
  41. 41.
    Henke KG, Orenstein DM. Oxygen saturation during exercise in cystic fibrosis. Am Rev Respir Dis. 1984;129:708–11.CrossRefPubMedGoogle Scholar
  42. 42.
    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.CrossRefPubMedGoogle Scholar
  43. 43.
    Ruf K, Hebestreit H. Exercise-induced hypoxemia and cardiac arrhythmia in cystic fibrosis. J Cyst Fibros. 2009;8:83–90.CrossRefPubMedGoogle Scholar
  44. 44.
    Marcotte JE, Grisdale RK, Levison H, Coates AL, Canny GJ. Multiple factors limit exercise capacity in cystic fibrosis. Pediatr Pulmonol. 1986;2:274–81.CrossRefPubMedGoogle Scholar
  45. 45.
    Peckham D, Watson A, Pollard K, Etherington C, Conway SP. Predictors of desaturation during formal hypoxic challenge in adult patients with cystic fibrosis. J Cyst Fibros. 2002;1:281–6.CrossRefPubMedGoogle Scholar
  46. 46.
    Wheatley CM, Foxx-Lupo WT, Cassuto NA, Wong EC, Daines CL, Morgan WJ, Snyder EM. Impaired lung diffusing capacity for nitric oxide and alveolar-capillary membrane conductance results in oxygen desaturation during exercise in patients with cystic fibrosis. J Cyst Fibros. 2011;10:45–53.CrossRefPubMedGoogle Scholar
  47. 47.
    Boas SR. Exercise recommendations for individuals with cystic fibrosis. Sports Med. 1997;24:17–37.CrossRefPubMedGoogle Scholar
  48. 48.
    Swisher AK, Hebestreit H, Meija-Downs A, Lowman JD, Gruber W, Nippins M, Alison J. Exercise and habitual physical activity for people with cystic fibrosis: expert consensus, evidence-based guide for advising patients. Cardiopulm Phys Ther J. 2015;26:85–98.CrossRefGoogle Scholar
  49. 49.
    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.CrossRefPubMedGoogle Scholar
  50. 50.
    McKone EF, Barry SC, FitzGerald MX, Gallagher CG. The role of supplemental oxygen during submaximal exercise in patients with cystic fibrosis. Eur Respir J. 2002;20:134–42.Google Scholar
  51. 51.
    Marcus CL, Bader D, Stabile MW, Wang CI, Osher AB, Keens TG. Supplemental oxygen and exercise performance in patients with cystic fibrosis with severe pulmonary disease. Chest. 1992;101:52–7.CrossRefPubMedGoogle Scholar
  52. 52.
    Nixon PA, Orenstein DM, Curtis SE, Ross EA. Oxygen supplementation during exercise in cystic fibrosis. Am Rev Respir Dis. 1990;142:807–11.Google Scholar
  53. 53.
    Nonoyama ML, Brooks D, Lacasse Y, Guyatt GH, Goldstein RS. Oxygen therapy during exercise training in chronic obstructive pulmonary disease. Cochrane Database Syst Rev. 2007; Issue 2:CD005372.Google Scholar
  54. 54.
    Sabapathy S, Kingsley RA, Schneider DA, Adams L, Morris NR. Continuous and intermittent exercise responses in individuals with chronic obstructive pulmonary disease. Thorax. 2004;59:1026–31.CrossRefPubMedPubMedCentralGoogle Scholar
  55. 55.
    Stevens D, Stephenson A, Faughnan ME, Leek E, Tullis E. Prognostic relevance of dynamic hyperinflation during cardiopulmonary exercise testing in adult patients with cystic fibrosis. J Cyst Fibros. 2013;12:655–61.CrossRefPubMedGoogle Scholar
  56. 56.
    Ambrosino N, Cigni P. Non invasive ventilation as an additional tool for exercise training. Multidiscip Respir Med. 2015;10:14.CrossRefPubMedPubMedCentralGoogle Scholar
  57. 57.
    Henke KG, Regnis JA, Bye PT. Benefits of continuous positive airway pressure during exercise in cystic fibrosis and relationship to disease severity. Am Rev Respir Dis. 1993;148:1272–6.CrossRefPubMedGoogle Scholar
  58. 58.
    Lima CA, Andrade Ade F, Campos SL, Brandao DC, Fregonezi G, Mourato IP, Aliverti A, Britto MC. Effects of noninvasive ventilation on treadmill 6-min walk distance and regional chest wall volumes in cystic fibrosis: randomized controlled trial. Respir Med. 2014;108:1460–8.CrossRefPubMedGoogle Scholar
  59. 59.
    Moran A, Brunzell C, Cohen RC, Katz M, Marshall BC, Onady G, Robinson KA, Sabadosa KA, Stecenko A, Slovis B. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  60. 60.
    Moran A, Dunitz J, Nathan B, Saeed A, Holme B, Thomas W. Cystic fibrosis-related diabetes: current trends in prevalence, incidence, and mortality. Diabetes Care. 2009;32:1626–31.CrossRefPubMedPubMedCentralGoogle Scholar
  61. 61.
    Ziegler B, Oliveira CL, Rovedder PM, Schuh SJ, Abreu ESFA, Dalcin Pde T. Glucose intolerance in patients with cystic fibrosis: sex-based differences in clinical score, pulmonary function, radiograph score, and 6-minute walk test. Respir Care. 2011;56:290–7.CrossRefPubMedGoogle Scholar
  62. 62.
    Colberg SR, Sigal RJ, Fernhall B, Regensteiner JG, Blissmer BJ, Rubin RR, Chasan-Taber L, Albright AL, Braun B, American College of Sports Medicine, American Diabetes Association. Exercise and type 2 diabetes: the American college of sports medicine and the American diabetes association: joint position statement. Diabetes Care. 2010;33:e147–67.CrossRefPubMedPubMedCentralGoogle Scholar
  63. 63.
    Beaudoin N, Bouvet GF, Coriati A, Rabasa-Lhoret R, Berthiaume Y. Combined exercise training improves glycemic control in adults with cystic fibrosis. Med Sci Sports Exerc. 2017;49:231–7.Google Scholar
  64. 64.
    Moran A, Diem P, Klein DJ, Levitt MD, Robertson RP. Pancreatic endocrine function in cystic fibrosis. J Pediatr. 1991;118:715–23.CrossRefPubMedGoogle Scholar
  65. 65.
    Roggla G, Linkesch M, Roggla M, Wagner A, Haber P, Linkesch W. A rare complication of a central venous catheter system (Port-a-Cath). A case report of a catheter embolization after catheter fracture during power training. Int J Sports Med. 1993;14:345–6.CrossRefPubMedGoogle Scholar
  66. 66.
  67. 67.
    Ruf K, Winkler B, Hebestreit A, Gruber W, Hebestreit H. Risks associated with exercise testing and sports participation in cystic fibrosis. J Cyst Fibros. 2010;9:339–45.CrossRefPubMedGoogle Scholar
  68. 68.
    Flume PA, Strange C, Ye X, Ebeling M, Hulsey T, Clark LL. Pneumothorax in cystic fibrosis. Chest. 2005a;128:720–8.CrossRefPubMedGoogle Scholar
  69. 69.
    Flume PA. Pneumothorax in cystic fibrosis. Chest. 2003;123:217–21.CrossRefPubMedGoogle Scholar
  70. 70.
    Flume PA. Pneumothorax in cystic fibrosis. Curr Opin Pulm Med. 2011;17:220–5.CrossRefPubMedGoogle Scholar
  71. 71.
    Flume PA, Yankaskas JR, Ebeling M, Hulsey T, Clark LL. Massive hemoptysis in cystic fibrosis. Chest. 2005;128:729–38.CrossRefPubMedGoogle Scholar
  72. 72.
    Flume PA, Mogayzel PJ Jr, Robinson KA, Rosenblatt RL, Quittell L, Marshall BC, Clinical Practice Guidelines for Pulmonary Therapies Committee, Cystic Fibrosis Foundation Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: pulmonary complications: hemoptysis and pneumothorax. Am J Respir Crit Care Med. 2010;182:298–306.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  • Thomas Radtke
    • 1
  • Susi Kriemler
    • 1
  • Helge Hebestreit
    • 2
  1. 1.Epidemiology, Biostatistics and Prevention InstituteUniversity of ZürichZurichSwitzerland
  2. 2.Paediatric DepartmentJulius-Maximilians University WürzburgWuerzburgGermany

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