Systematic Review of the Methodological Quality and Outcome Measures Utilized in Exercise Interventions for Individuals with Cystic Fibrosis Methodological Quality and Outcome Measures Utilized in CF Exercise Studies
Main Article Content
Abstract
Background: The aims of this study were to evaluate the methodological quality of interventional exercise studies in individuals with cystic fibrosis (CF) and link the reported outcome measures of these studies to the International Classification of Functioning, Disability and Health (ICF) model.
Methods: Electronic searches of PubMed, Scopus, Embase, CINAHL, SPORTDiscus, and Cochrane review were performed in the spring of, 2022. The methodological quality of studies was evaluated using the Effective Public Health Practice Project Quality Assessment Tool (EPHPP). Outcome measures were linked to the categories and sub-categories of the ICF model using ICF linking rules.
Results: We screened 4,769 studies and extracted data from 89 eligible manuscripts. Of these. 41% were one group, pre-post design; 36% were RCTs; 11% were two-group pre-post design; 6% were controlled clinical trials; and 6% were either case reports or case series. Using the (EPHPP), 49% were ranked as high quality, 34% moderate, and 17% low. In addition, body function was the most addressed ICF category followed by activities/participation and, subsequently, body structure.
Significance: This review supports the recommendation of exercise as an intervention to improve a variety of outcomes, ranging from Body structure (e.g., body composition) to Participation (e.g., engaging in community, social, and civic life). We recommend that investigators review the spectrum of potential ICF outcomes when designing rehabilitation trials, to both reflect the potential effects of exercise on a variety of outcomes, but also to facilitate mechanistic studies that are able to link which body structures and body functions are most important to improving patient outcomes at the activity and participation domains. Addressing environmental factors within the ICF is also important for people with CF, such as resource allocation towards exercise and rehabilitation resources. Finally, this review highlights the need for more vigorous methodological designs to minimize risk of bias and improve quality of exercise studies in CF.
Keywords:
Cystic Fibrosis, exercise, ICF, quality assessment, systematic review
Article Details
How to Cite
ALOTAIBI, Najlaa Z. et al.
Systematic Review of the Methodological Quality and Outcome Measures Utilized in Exercise Interventions for Individuals with Cystic Fibrosis.
Medical Research Archives, [S.l.], v. 12, n. 12, dec. 2024.
ISSN 2375-1924.
Available at: <https://esmed.org/MRA/mra/article/view/5917>. Date accessed: 06 jan. 2025.
doi: https://doi.org/10.18103/mra.v12i12.5917.
Section
Research Articles
The Medical Research Archives grants authors the right to publish and reproduce the unrevised contribution in whole or in part at any time and in any form for any scholarly non-commercial purpose with the condition that all publications of the contribution include a full citation to the journal as published by the Medical Research Archives.
References
1. Foundation CF. About Cystic Fibrosis. 2024. https://www.cff.org/intro-cf/about-cystic-fibrosis
2. Balfour-Lynn IM, Welch K, Smith S. Inhaled corticosteroids for cystic fibrosis. Cochrane Database of Systematic Reviews. 2019;(7) doi:10. 1002/14651858.CD001915.pub6
3. Coelho CC, Aquino EdS, Diniz ALR, et al. Tissue oxygenation in peripheral muscles and functional capacity in cystic fibrosis: a cross-sectional study. Experimental Physiology. 2020;105(9):1571-1578. doi:https://doi.org/10.1113/EP088764
4. Stein L, Pacht C, Junge S, et al. Skeletal Muscle Function in Young Patients With Cystic Fibrosis. Article. Pediatric Exercise Science. 2016;28(3):364-373. doi:10.1123/pes.2015-0184
5. Rodriguez-Miguelez P, Seigler N, Ishii H, et al. Exercise Intolerance in Cystic Fibrosis: Importance of Skeletal Muscle. Med Sci Sports Exerc. Apr 1 2021;53(4):684-693. doi:10.1249/MSS.0000000000002521
6. Hebestreit H, Kriemler S, Schindler C, et al. Effects of a Partially Supervised Conditioning Program in Cystic Fibrosis: An International Multicenter, Randomized Controlled Trial (ACTIVATE-CF). Am J Respir Crit Care Med. Feb 1 2022;205(3):330-339. doi:10.1164/rccm.202106-1419OC
7. Radtke T, Smith S, Nevitt SJ, Hebestreit H, Kriemler S. Physical activity and exercise training in cystic fibrosis. Cochrane Database of Systematic Reviews. 2022;(8)doi:10.1002/14651858.CD002768.pub5
8. Ozipek M, Arikan H, Calik-Kutukcu E, et al. Deviations of body functions and structure, activity limitations, and participation restrictions of the International Classification of Functioning, Disability, and Health model in children with cystic fibrosis and non–cystic fibrosis bronchiectasis. Pediatric Pulmonology. 2020;55(5):1207-1216. doi:https://doi.org/10.1002/ppul.24708
9. Silverman SR, Schertz LA, Yuen HK, Lowman JD, Bickel CS. Systematic review of the methodological quality and outcome measures utilized in exercise interventions for adults with spinal cord injury. Spinal Cord. 2012/10/01 2012;50(10):718-727. doi:10.1038/sc.2012.78
10. Radtke T, Nevitt SJ, Hebestreit H, Kriemler S. Physical exercise training for cystic fibrosis. Cochrane Database of Systematic Reviews. 2017; (11) doi:10.1002/14651858.CD002768.pub4
11. Ben B, Latour JM, Tomlinson OW. A systematic review to explore how exercise-based physiotherapy via telemedicine can promote health related benefits for people with cystic fibrosis. PLOS Digital Health. 2023;2(2):e0000201. doi:10.1371/journal.pdig.0000201
12. Page MJ, McKenzie JE, Bossuyt PM, et al. The PRISMA 2020 statement: an updated guideline for reporting systematic reviews. BMJ. 2021;372:n71. doi:10.1136/bmj.n71
13. Alotaibi N, Lowman J, Horton T, et al. The Effects of Exercise and Physical Activity in People with Cystic Fibrosis Based on the ICF Domains. 2024. https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42023332797
14. Covidence. Covidence. 2024. https://www.covidence.org/
15. World Health Organization. International Classification of Functioning, Disability and Health. 2024. https://icd.who.int/browse/2024-01/icf/en
16. Cieza A, Brockow T, Ewert T, et al. Linking health-status measurements to the international classification of functioning, disability and health. J Rehabil Med. Sep 2002;34(5):205-10. doi:10.1080/ 165019702760279189
17. Cieza A, Fayed N, Bickenbach J, Prodinger B. Refinements of the ICF Linking Rules to strengthen their potential for establishing comparability of health information. Disability and Rehabilitation. 2019/02/27 2019;41(5):574-583. doi:10.3109/0963 8288.2016.1145258
18. Cieza A, Geyh S, Chatterji S, Kostanjsek N, Ustun B, Stucki G. ICF linking rules: an update based on lessons learned. J Rehabil Med. Jul 2005; 37(4):212-8. doi:10.1080/16501970510040263
19. Effective Public Healthcare Panacea Project. Quality Assessment Tool for Quantitative Studies. 2024. https://www.ephpp.ca/quality-assessment-tool-for-quantitative-studies/
20. Thomas BH, Ciliska D, Dobbins M, Micucci S. A Process for Systematically Reviewing the Literature: Providing the Research Evidence for Public Health Nursing Interventions. Worldviews on Evidence-Based Nursing. 2004;1(3):176-184. doi:https://doi.org/10.1111/j.1524-475X.2004.04006.x
21. Armijo-Olivo S, Stiles CR, Hagen NA, Biondo PD, Cummings GG. Assessment of study quality for systematic reviews: a comparison of the Cochrane Collaboration Risk of Bias Tool and the Effective Public Health Practice Project Quality Assessment Tool: methodological research. Journal of Evaluation in Clinical Practice. 2012;18(1):12-18. doi:https://doi.org/10.1111/j.1365-2753.2010.01516.x
22. Deeks JJ, Dinnes J, D'Amico R, et al. Evaluating non-randomised intervention studies. Health Technol Assess. 2003;7(27):iii-x, 1-173. doi:10.3310/hta7270
23. Jackson N, Waters E, for the Guidelines for Systematic Reviews in Health P, Public Health T. Criteria for the systematic review of health promotion and public health interventions. Health Promotion International. 2005;20(4):367-374. doi: 10.1093/heapro/dai022
24. Physiotherapy Evidence Database. PEDro scale. 2024. https://pedro.org.au/
25. Maher CG, Sherrington C, Herbert RD, Moseley AM, Elkins M. Reliability of the PEDro Scale for Rating Quality of Randomized Controlled Trials. Physical Therapy. 2003;83(8):713-721. doi: 10.1093/ptj/83.8.713
26. de Morton NA. The PEDro scale is a valid measure of the methodological quality of clinical trials: a demographic study. Australian Journal of Physiotherapy. 2009/01/01/ 2009;55(2):129-133. doi:https://doi.org/10.1016/S0004-9514(09)70043-1
27. Adair D, Hider A, Filbrun AG, et al. Assessing the Utility of an Outpatient Exercise Program for Children With Cystic Fibrosis: A Quality Improvement Project. Brief Research Report. Frontiers in Pediatrics. 2022-January-13 2022;9doi: 10.3389/fped.2021.734292
28. Almăjan-Guţă B, Cluci O, Rusu A, Ciuca O. Individualized unsupervised exercise programs and chest physiotherapy in children with cystic fibrosis. Timisoara Physical Education and Rehabilitation Journal. 2016;6(11):51-54. doi:doi:10.2478/tperj-2013-0018
29. Andréasson B, Jonson B, Kornfält R, Nordmark E, Sandström S. Long-term effects of physical exercise on working capacity and pulmonary function in cystic fibrosis. Acta Paediatr Scand. Jan 1987;76 (1):70-5. doi:10.1111/j.1651-2227.1987.tb10417.x
30. Asher MI, Pardy RL, Coates AL, Thomas E, Macklem PT. The effects of inspiratory muscle training in patients with cystic fibrosis. Am Rev Respir Dis. Nov 1982;126(5):855-9. doi:10.1164/ arrd.1982.126.5.855
31. Aspinall SP, Hill DM, Mackintosh KA, McNarry MA. Reduced exertion high-intensity interval training (REHIT) in an adult with Cystic Fibrosis: A mixed-methods case study. Respir Med Case Rep. 2020;31:101173. doi:10.1016/j.rmcr.2020.101173
32. Beaudoin N, Bouvet GF, Coriati A, Rabasa-Lhoret R, Berthiaume Y. Combined Exercise Training Improves Glycemic Control in Adult with Cystic Fibrosis. Med Sci Sports Exerc. Feb 2017;49 (2):231-237. doi:10.1249/mss.0000000000001104
33. Bieli C, Summermatter S, Boutellier U, Moeller A. Respiratory muscle training improves respiratory muscle endurance but not exercise tolerance in children with cystic fibrosis. Pediatr Pulmonol. Mar 2017;52(3):331-336. doi:10.1002/ppul.23647
34. Blau H, Mussaffi-Georgy H, Fink G, et al. Effects of an intensive 4-week summer camp on cystic fibrosis: pulmonary function, exercise tolerance, and nutrition. Chest. Apr 2002;121(4) :1117-22. doi:10.1378/chest.121.4.1117
35. Blomquist M, Freyschuss U, Wiman LG, Strandvik B. Physical activity and self treatment in cystic fibrosis. Arch Dis Child. Apr 1986;61(4):362-7. doi:10.1136/adc.61.4.362
36. Braggion C, Cornacchia M, Miano A, Schena F, Verlato G, Mastella G. Exercise tolerance and effects of training in young patients with cystic fibrosis and mild airway obstruction. Pediatr Pulmonol. 1989;7(3):145-52. doi:10.1002/ppul.1950070306
37. Carr SB, Ronan P, Lorenc A, Mian A, Madge SL, Robinson N. Children and Adults Tai Chi Study (CF-CATS2): a randomised controlled feasibility study comparing internet-delivered with face-to-face Tai Chi lessons in cystic fibrosis. ERJ Open Res. Oct 2018;4(4)doi:10.1183/23120541.00042-2018
38. Cerny FJ. Relative effects of bronchial drainage and exercise for in-hospital care of patients with cystic fibrosis. Phys Ther. Aug 1989;69(8):633-9. doi:10.1093/ptj/69.8.633
39. Clément M, Jankowski LW, Beaudry PH. Prone immersion physical exercise therapy in three children with cystic fibrosis: a pilot study. Nurs Res. Nov-Dec 1979;28(6):325-9.
40. de Jong W, Grevink RG, Roorda RJ, Kaptein AA, van der Schans CP. Effect of a home exercise training program in patients with cystic fibrosis. Chest. Feb 1994;105(2):463-8. doi:10.1378/chest. 105.2.463
41. de Jong W, van Aalderen WM, Kraan J, Koëter GH, van der Schans CP. Inspiratory muscle training in patients with cystic fibrosis. Respir Med. Jan 2001;95(1):31-6. doi:10.1053/rmed.2000.0966
42. Dekerlegand RL, Hadjiliadis D, Myslinski MJ, Holsclaw D, Ferrin M. Home-based Inspiratory Muscle Training In Adults With Cystic Fibrosis: A Case Series Report. Cardiopulmonary Physical Therapy Journal. 2014;25(3):85-91.
43. Del Corral T, Cebrià IIM, López-de-Uralde-Villanueva I, Martínez-Alejos R, Blanco I, Vilaró J. Effectiveness of a Home-Based Active Video Game Programme in Young Cystic Fibrosis Patients. Respiration. 2018;95(2):87-97. doi:10.1159/000481264
44. de Oliveira ACN, de Oliveira JCM, Mesquita-Ferrari RA, Damasceno N, Oliveira LVF, Sampaio LMM. Inflammatory process modulation in children with cystic fibrosis submitted to aerobic training. Archives of Medical Science. 2009 2023-11-19 2009;5(3):422.
45. de Oliveira CQ, de Matos Fonseca V, Senna G, Scudese E, de Miranda Chaves CR. Effects of Aerobic and Resistance Training on Body Composition and Physical Capacity of Adolescents with Cystic Fibrosis. Article. Journal of Exercise Physiology Online. 2017;20(2):55-63.
46. Donadio MVF, Cobo-Vicente F, San Juan AF, et al. Is exercise and electrostimulation effective in improving muscle strength and cardiorespiratory fitness in children with cystic fibrosis and mild-to-moderate pulmonary impairment?: Randomized controlled trial. Respir Med. May 2022;196:106 798. doi:10.1016/j.rmed.2022.106798
47. Dunlevy CL, Douce FH, Hill E, Baez S, Clutter J. Physiological and Psychological Effects of Low-Impact Aerobic Exercise on Young Adults with Cystic Fibrosis. Journal of Cardiopulmonary Rehabilitation and Prevention. 1994;14(1)
48. Edlund LD, French RW, Herbst JJ, Ruttenburg HD, Ruhling RO, Adams TD. Effects of a swimming program on children with cystic fibrosis. Am J Dis Child. Jan 1986;140(1):80-3. doi:10.1001/archpedi .1986.02140150082044
49. 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. Jan 10 2012;38:2. doi:10.1186/1824-7288-38-2
50. Elce A, Nigro E, Gelzo M, et al. Supervised physical exercise improves clinical, anthropometric and biochemical parameters in adult cystic fibrosis patients: A 2-year evaluation. Clin Respir J. Jul 2018;12(7):2228-2234. doi:10.1111/crj.12796
51. Enright S, Chatham K, Ionescu AA, Unnithan VB, Shale DJ. Inspiratory muscle training improves lung function and exercise capacity in adults with cystic fibrosis. Chest. Aug 2004;126(2):405-11. doi:10.1378/chest.126.2.405
52. Estévez-González AJ, Donadio MVF, Cobo-Vicente F, et al. Effects of a Short-Term Resistance-Training Program on Heart Rate Variability in Children With Cystic Fibrosis-A Randomized Controlled Trial. Front Physiol. 2021;12:652029. doi:10.3389/fphys.2021.652029
53. Franco CB, Ribeiro AF, Morcillo AM, Zambon MP, Almeida MB, Rozov T. Air stacking: effects of Pilates mat exercises on muscle strength and on pulmonary function in patients with cystic fibrosis. J Bras Pneumol. Oct 2014;40(5):521-7. doi:10.1590 /s1806-37132014000500008
54. Goldbart AD, Cohen AD, Weitzman D, Tal A. Effects of rehabilitation winter camps at the Dead Sea on European cystic fibrosis patients. Isr Med Assoc J. Nov 2007;9(11):806-9.
55. Gruber W, Orenstein DM, Braumann KM. Do responses to exercise training in cystic fibrosis depend on initial fitness level? Eur Respir J. Dec 2011;38(6):1336-42. doi:10.1183/09031936.00192510
56. 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. Jan 2014;13(1):86-91. doi:10. 1016/j.jcf.2013.06.005
57. Gruber W, Orenstein DM, Braumann KM, Hüls G. Health-related fitness and trainability in children with cystic fibrosis. Pediatr Pulmonol. Oct 2008;43 (10):953-64. doi:10.1002/ppul.20881
58. Gruber W, Orenstein DM, Braumann KM, Paul K, Hüls G. Effects of an exercise program in children with cystic fibrosis: are there differences between females and males? J Pediatr. Jan 2011;1 58(1):71-6. doi:10.1016/j.jpeds.2010.07.033
59. Gruber W, Stehling F, Olivier M, et al. Effects of a long-term exercise program on motor performance in children and adolescents with CF. Pediatr Pulmonol. Dec 2020;55(12):3371-3380. doi:10.1002/ppul.25064
60. Gruber W, Welsner M, Dillenhöfer S, et al. Health-Related and Motor Performance-Related Fitness and Physical Activity Among Youth With Cystic Fibrosis. Percept Mot Skills. Oct 2021;128 (5):2097-2116. doi:10.1177/00315125211036415
61. Gulmans VA, de Meer K, Brackel HJ, Faber JA, Berger R, Helders PJ. Outpatient exercise training in children with cystic fibrosis: physiological effects, perceived competence, and acceptability. Pediatr Pulmonol. Jul 1999;28(1):39-46. doi:10.1002/(sici) 1099-0496(199907)28:1<39::aid-ppul7>3.0.co;2-8
62. Gulmans V, van der Laag J, Wattimena D, et al. Insulin-like growth factors and leucine kinetics during exercise training in children with cystic fibrosis. J Pediatr Gastroenterol Nutr. Jan 2001;32 (1):76-81. doi:10.1097/00005176-200101000-00020
63. Güngör S, Gencer-Atalay K, Bahar-Özdemir Y, Keniş-Coşkun Ö, Karadağ-Saygı E. The clinical effects of combining postural exercises with chest physiotherapy in cystic fibrosis: A single-blind, randomized-controlled trial. Turk J Phys Med Rehabil. Mar 2021;67(1):91-98. doi:10.5606/tftrd. 2021.5214
64. Gupta S, Mukherjee A, Lodha R, et al. Effects of Exercise Intervention Program on Bone Mineral Accretion in Children and Adolescents with Cystic Fibrosis: A Randomized Controlled Trial. Indian J Pediatr. Nov 2019;86(11):987-994. doi:10.1007/s1 2098-019-03019-x
65. Hebestreit H, Kieser S, Junge S, et al. Long-term effects of a partially supervised conditioning programme in cystic fibrosis. Eur Respir J. Mar 2010;35(3):578-83. doi:10.1183/09031936.00062409
66. Hebestreit H, Kriemler S, Schindler C, et al. Effects of a Partially Supervised Conditioning Program in Cystic Fibrosis: An International Multicenter, Randomized Controlled Trial (ACTIVATE-CF). Am J Respir Crit Care Med. Feb 1 2022;205(3):330-339. doi:10.1164/rccm.202106-1419OC
67. Heijerman HG, Bakker W, Sterk PJ, Dijkman JH. Long-term effects of exercise training and hyperalimentation in adult cystic fibrosis patients with severe pulmonary dysfunction. Int J Rehabil Res. 1992;15(3):252-7. doi:10.1097/00004356-199 209000-00011
68. Heijerman HG, Bakker W, Sterk PJ, Dijkman JH. Oxygen-assisted exercise training in adult cystic fibrosis patients with pulmonary limitation to exercise. Int J Rehabil Res. 1991;14(2):101-15. doi:10.1097/00004356-199106000-00002
69. Holmes CJ, Racette SB, Symonds L, Arbeláez AM, Cao C, Granados A. Comparison of Bioelectrical Impedance Analysis with DXA in Adolescents with Cystic Fibrosis before and after a Resistance Training Intervention. Int J Environ Res Public Health. Mar 29 2022;19(7)doi:10.3390/ijerph19074037
70. Hommerding PX, Baptista RR, Makarewicz GT, et al. Effects of an educational intervention of physical activity for children and adolescents with cystic fibrosis: a randomized controlled trial. Respir Care. Jan 2015;60(1):81-7. doi:10.4187/respcare.02578
71. Horvat M, Carlile JR. Effects of progressive resistance exercise on physical functioning and self-concept in cystic fibrosis. 01/01 1991;45:18-23.
72. Hulzebos HJ, Snieder H, van der Et J, Helders PJ, Takken T. High-intensity interval training in an adolescent with cystic fibrosis: a physiological perspective. Physiother Theory Pract. Apr 2011;27 (3):231-7. doi:10.3109/09593985.2010.483266
73. Johnson S, Burger S, Williams J. Implementing Exercise Journaling and Exercise Encouragement in Hospitalized Adults with Cystic Fibrosis. Article. MEDSURG Nursing. 2021;30(4):263-274.
74. Kaltsakas G, Chynkiamis N, Anastasopoulos N, et al. Interval versus constant-load exercise training in adults with Cystic Fibrosis. Respir Physiol Neurobiol. Jun 2021;288:103643. doi:10.1016/j. resp.2021.103643
75. Kaplan TA, McKey RM, Jr., Toraya N, Moccia G. Impact of CF summer camp. Clin Pediatr (Phila). Mar 1992;31(3):161-7. doi:10.1177/000992289203100307
76. Kenis-Coskun Ö, Aksoy AN, Kumaş EN, et al. The effect of telerehabilitation on quality of life, anxiety, and depression in children with cystic fibrosis and caregivers: A single-blind randomized trial. Pediatr Pulmonol. May 2022;57(5):1262-1271. doi:10.1002/ppul.25860
77. 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. Apr 2004;125 (4):1299-305. doi:10.1378/chest.125.4.1299
78. Kriemler S, Kieser S, Junge S, et al. Effect of supervised training on FEV1 in cystic fibrosis: a randomised controlled trial. J Cyst Fibros. Dec 2013;12(6):714-20. doi:10.1016/j.jcf.2013.03.003
79. Ledger SJ, Owen E, Prasad SA, Goldman A, Willams J, Aurora P. A pilot outreach physiotherapy and dietetic quality improvement initiative reduces IV antibiotic requirements in children with moderate-severe cystic fibrosis. J Cyst Fibros. Dec 2013;12(6):766-72. doi:10.1016/j.jcf.2013.01.003
80. Massery M. Musculoskeletal and neuromuscular interventions: a physical approach to cystic fibrosis. J R Soc Med. 2005;98 Suppl 45(Suppl 45):55-66.
81. McCreery J, Mackintosh K, McNarry M. Effects of Inspiratory Muscle Training on Heart Rate Variability in Children with Cystic Fibrosis: A Pilot Study. Journal of Science in Sport and Exercise. 2021;3(1):66-74. doi:10.1007/s42978-020-00079-w
82. McNamara C, Johnson M, Read L, et al. Yoga Therapy in Children with Cystic Fibrosis Decreases Immediate Anxiety and Joint Pain. Evid Based Complement Alternat Med. 2016;2016:9429504. doi:10.1155/2016/9429504
83. Moorcroft AJ, Dodd ME, Morris J, Webb AK. Individualised unsupervised exercise training in adults with cystic fibrosis: a 1 year randomised controlled trial. Thorax. Dec 2004;59(12):1074-80. doi:10.1136/thx.2003.015313
84. O'Neill PA, Dodds M, Phillips B, Poole J, Webb AK. Regular exercise and reduction of breathlessness in patients with cystic fibrosis. Br J Dis Chest. Jan 1987;81(1):62-9. doi:10.1016/0007-0971(87)90109-4
85. Orenstein DM, Franklin BA, Doershuk CF, et al. Exercise conditioning and cardiopulmonary fitness in cystic fibrosis. The effects of a three-month supervised running program. Chest. Oct 1981;80(4):392-8. doi:10.1378/chest.80.4.392
86. Orenstein DM, Hovell MF, Mulvihill M, et al. Strength vs aerobic training in children with cystic fibrosis: a randomized controlled trial. Chest. Oct 2004;126(4):1204-14. doi:10.1378/chest.126.4.1204
87. Paranjape SM, Barnes LA, Carson KA, von Berg K, Loosen H, Mogayzel PJ, Jr. Exercise improves lung function and habitual activity in children with cystic fibrosis. J Cyst Fibros. Jan 2012;11(1):18-23. doi:10.1016/j.jcf.2011.08.003
88. Prévotat A, Godin J, Bernard H, Perez T, Le Rouzic O, Wallaert B. Improvement in body composition following a supervised exercise-training program of adult patients with cystic fibrosis. Respir Med Res. May 2019;75:5-9. doi:10. 1016/j.resmer.2019.04.001
89. Reuveny R, DiMenna FJ, Gunaratnam C, et al. High-intensity interval training accelerates oxygen uptake kinetics and improves exercise tolerance for individuals with cystic fibrosis. BMC Sports Sci Med Rehabil. 2020;12:9. doi:10.1186/s13102-020-0159-z
90. Rovedder PM, Flores J, Ziegler B, et al. Exercise programme in patients with cystic fibrosis: a randomized controlled trial. Respir Med. Aug 2014;108(8):1134-40. doi:10.1016/j.rmed.2014.04.022
91. Ruddy J, Emerson J, McNamara S, et al. Yoga as a Therapy for Adolescents and Young Adults With Cystic Fibrosis: A Pilot Study. Glob Adv Health Med. Nov 2015;4(6):32-6. doi:10.7453/gah mj.2015.061
92. Sahlberg M, Eriksson BO, Sixt R, Strandvik B. Cardiopulmonary data in response to 6 months of training in physically active adult patients with classic cystic fibrosis. Respiration. 2008;76(4):413-20. doi:10.1159/000156862
93. Sahlberg M, Svantesson U, Magnusson Thomas E, Andersson BA, Saltin B, Strandvik B. Muscular strength after different types of training in physically active patients with cystic fibrosis. Scand J Med Sci Sports. Dec 2008;18(6):756-64. doi:10.1111/j.1600-0838.2007.00691.x
94. 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. Oct 2014;48(20):1513-7. doi:10.1136/bjsport s-2012-091892
95. Santana Sosa E, 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. Jan 2012;44 (1):2-11. doi:10.1249/MSS.0b013e318228c302
96. Sartori R, Barbi E, Poli F, et al. Respiratory training with a specific device in cystic fibrosis: a prospective study. J Cyst Fibros. Jul 2008;7(4):313-319. doi:10.1016/j.jcf.2007.12.003
97. Sawyer A, Cavalheri V, Jenkins S, et al. High-Intensity Interval Training Is Effective at Increasing Exercise Endurance Capacity and Is Well Tolerated by Adults with Cystic Fibrosis. J Clin Med. Sep 25 2020;9(10)doi:10.3390/jcm9103098
98. Sawyer EH, Clanton TL. Improved pulmonary function and exercise tolerance with inspiratory muscle conditioning in children with cystic fibrosis. Chest. Nov 1993;104(5):1490-7. doi:10.1378/chest .104.5.1490
99. Schindel CS, Hommerding PX, Melo DA, Baptista RR, Marostica PJ, Donadio MV. Physical exercise recommendations improve postural changes found in children and adolescents with cystic fibrosis: a randomized controlled trial. J Pediatr. Mar 2015;166(3):710-6.e2. doi:10.1016/j. jpeds.2014.12.001
100. 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. Nov 2011;27(8):548-56. doi:10.3109/ 09593985.2010.545102
101. 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. Mar 2000;136(3):304-10. doi:10.1067/mpd.2000.103408
102. 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. Mar 2002;33(3):194-200. doi:10.1002/ ppul.10015
103. Shaw I, Kinsey JE, Richards R, Shaw BS. Individualized supervised resistance training during nebulization in adults with cystic fibrosis. Pak J Med Sci. Sep-Oct 2016;32(5):1152-1157. doi:10.12669/pjms.325.9960
104. Strauss GD, Osher A, Wang CI, et al. Variable weight training in cystic fibrosis. Chest. Aug 1987;92(2):273-6. doi:10.1378/chest.92.2.273
105. Tomlinson OW, Shelley J, Trott J, Bowhay B, Chauhan R, Sheldon CD. The feasibility of online video calling to engage patients with cystic fibrosis in exercise training. J Telemed Telecare. Jul 2020 ;26(6):356-364. doi:10.1177/1357633x19828630
106. Turchetta A, Salerno T, Lucidi V, Libera F, Cutrera R, Bush A. Usefulness of a program of hospital-supervised physical training in patients with cystic fibrosis. Pediatr Pulmonol. Aug 2004; 38(2):115-8. doi:10.1002/ppul.20073
107. Van Biervliet S, Declercq D, Dereeper S, Vermeulen D, Würth B, De Guschtenaere A. The effect of an intensive residential rehabilitation program on body composition in patients with cystic fibrosis. Eur J Pediatr. Jun 2021;180(6):1981-1985. doi:10.1007/s00431-021-03943-1
108. van de Weert-van Leeuwen PB, Hulzebos HJ, Werkman MS, et al. Chronic inflammation and infection associate with a lower exercise training response in cystic fibrosis adolescents. Respir Med. Mar 2014;108(3):445-52. doi:10.1016/j.rmed.2013.08.012
109. Vivodtzev I, Decorte N, Wuyam B, et al. Benefits of neuromuscular electrical stimulation prior to endurance training in patients with cystic fibrosis and severe pulmonary dysfunction. Chest. Feb 1 2013;143(2):485-493. doi:10.1378/chest.12-0584
110. Welsner M, Gruber W, Mellies U, et al. Trainability of Health-Related and Motor Performance Fitness in Adults with Cystic Fibrosis within a 12-Month Partially Supervised Exercise Program. Pulm Med. 2021;2021:5581812. doi:10. 1155/2021/5581812
111. Yalçınkaya G, Alpaydın AÖ, Özalevli S. Effect of pulmonary rehabilitation on adult cystic fibrosis patient: a case report. Pulmoner rehabilitasyonun erişkin kistik fibrozisli hasta üzerindeki etkisi: bir olgu sunumu. Journal of Exercise Therapy and Rehabilitation. January 2020;6(3):220-225.
112. Zach MS, Purrer B, Oberwaldner B. Effect of swimming on forced expiration and sputum clearance in cystic fibrosis. Lancet. Nov 28 1981;2(8 257):1201-3. doi:10.1016/s0140-6736(81)91440-9
113. Zach M, Oberwaldner B, Häusler F. Cystic fibrosis: physical exercise versus chest physiotherapy. Arch Dis Child. Aug 1982;57(8):587-9. doi:10.1136 /adc.57.8.587
114. Zeren M, Cakir E, Gurses HN. Effects of inspiratory muscle training on postural stability, pulmonary function and functional capacity in children with cystic fibrosis: A randomised controlled trial. Respir Med. Mar 2019;148:24-30. doi:10.1016/j.rmed.2019.01.013
115. Calthorpe RJ, Smith SJ, Rowbotham NJ, et al. What effective ways of motivation, support and technologies help people with cystic fibrosis improve and sustain adherence to treatment? BMJ Open Respir Res. Aug 2020;7(1)doi:10.1136/bmjre sp-2020-000601
116. Braithwaite J, Testa L, Lamprell G, et al. Built to last? The sustainability of health system improvements, interventions and change strategies: a study protocol for a systematic review. BMJ Open. Nov 12 2017;7(11):e018568. doi:10.11 36/bmjopen-2017-018568
117. Lima EA, Rodrigues G, Mota BC, Castro SS, Mesquita RB, Leite CF. Which Components of The International Classification of Functioning, Disability and Health (ICF) are Covered by Cardiac Rehabilitation Assessment Tools among Individuals with Heart Failure? Heart & Lung. 2024/01/01/ 2024;63:65-71. doi:https://doi.org/10.1016/j.hrtlng.2023.09.010
118. Rauch A, Kirchberger I, Stucki G, Cieza A. Validation of the Comprehensive ICF Core Set for obstructive pulmonary diseases from the perspective of physiotherapists. Physiother Res Int. Dec 2009;14(4):242-59. doi:10.1002/pri.448
119. Bui KL, Nyberg A, Maltais F, Saey D. Functional Tests in Chronic Obstructive Pulmonary Disease, Part 1: Clinical Relevance and Links to the International Classification of Functioning, Disability, and Health. Ann Am Thorac Soc. May 2017;14(5): 778-784. doi:10.1513/AnnalsATS.201609-733AS
120. Mandrusiak A, MacDonald J, Watter P. The International Classification of Functioning, Disability and Health: an effective model for describing young people with cystic fibrosis. Child: Care, Health and Development. 2009/01/01 2009; 35(1):2-4.doi:https://doi.org/10.1111/j.1365-2214.2008.00903.x
121. Mariani A, Gambazza S, Carta F, et al. Prevalence and factors associated with urinary incontinence in females with cystic fibrosis: An Italian single-center cross-sectional analysis. Pediatr Pulmonol. Jan 2022;57(1):132-141. doi:10. 1002/ppul.25723
122. Vargas L, Kazmerski TM, von Berg K, Patterson D, West N, Bradley M. A Qualitative Exploration of Women With Cystic Fibrosis and Urinary Incontinence. Urogynecology (Phila). Sep 19 2024; doi:10.1097/spv.0000000000001572
123. Button BM, Holland AE, Sherburn MS, Chase J, Wilson JW, Burge AT. Prevalence, impact and specialised treatment of urinary incontinence in women with chronic lung disease. Physiotherapy. Mar 2019;105(1):114-119. doi:10.1016/j.physio.20 18.07.006
124. Wheatley CM, Wilkins BW, Snyder EM. Exercise is medicine in cystic fibrosis. Exerc Sport Sci Rev. Jul 2011;39(3):155-60. doi:10.1097/JES.0b 013e3182172a5a
125. American College of Sports Medicine. Exercise is Medicine®. Accessed 11/11/2024, https://www.exerciseismedicine.org/
126. Hoffmann TC, Glasziou PP, Boutron I, et al. Better reporting of interventions: template for intervention description and replication (TIDieR) checklist and guide. BMJ : British Medical Journal. 2014;348:g1687. doi:10.1136/bmj.g1687
127. Quittner AL, Sweeny S, Watrous M, et al. Translation and linguistic validation of a disease-specific quality of life measure for cystic fibrosis. J Pediatr Psychol. Sep 2000;25(6):403-14. doi:10.10 93/jpepsy/25.6.403
128. Solé A, Olveira C, Pérez I, et al. Development and electronic validation of the revised Cystic Fibrosis Questionnaire (CFQ-R Teen/Adult): New tool for monitoring psychosocial health in CF. Journal of Cystic Fibrosis. 2018;17(5):672-679. doi:10.1016/j.jcf.2017.10.015
129. Borg GA. Psychophysical bases of perceived exertion. Med Sci Sports Exerc. 1982;14(5):377-81.
130. Wilson RC, Jones PW. A comparison of the visual analogue scale and modified Borg scale for the measurement of dyspnoea during exercise. Clin Sci (Lond). Mar 1989;76(3):277-82. doi:10.104 2/cs0760277
131. Bijur PE, Silver W, Gallagher EJ. Reliability of the Visual Analog Scale for Measurement of Acute Pain. Academic Emergency Medicine. 2001/12/01 2001;8(12):1153-1157. doi:https://doi.org/10.1111/j.1553-2712.2001.tb01132.x
132. Zigmond AS, Snaith RP. The hospital anxiety and depression scale. Acta Psychiatr Scand. Jun 1983;67(6):361-70. doi:10.1111/j.1600-0447.1983. tb09716.x
133. Smets EM, Garssen B, Bonke B, De Haes JC. The Multidimensional Fatigue Inventory (MFI) psychometric qualities of an instrument to assess fatigue. J Psychosom Res. Apr 1995;39(3):315-25. doi:10.1016/0022-3999(94)00125-o
134. Ruf KC, Fehn S, Bachmann M, et al. Validation of activity questionnaires in patients with cystic fibrosis by accelerometry and cycle ergometry. BMC Med Res Methodol. Apr 3 2012;12:43. doi:10.1186/1471-2288-12-43
135. Orenstein DM, Kaplan RM. Measuring the quality of well-being in cystic fibrosis and lung transplantation. The importance of the area under the curve. Chest. Oct 1991;100(4):1016-8. doi:10.1 378/chest.100.4.1016
136. Orenstein DM, Nixon PA, Ross EA, Kaplan RM. The quality of well-being in cystic fibrosis. Chest. Feb 1989;95(2):344-7. doi:10.1378/chest.95.2.344
2. Balfour-Lynn IM, Welch K, Smith S. Inhaled corticosteroids for cystic fibrosis. Cochrane Database of Systematic Reviews. 2019;(7) doi:10. 1002/14651858.CD001915.pub6
3. Coelho CC, Aquino EdS, Diniz ALR, et al. Tissue oxygenation in peripheral muscles and functional capacity in cystic fibrosis: a cross-sectional study. Experimental Physiology. 2020;105(9):1571-1578. doi:https://doi.org/10.1113/EP088764
4. Stein L, Pacht C, Junge S, et al. Skeletal Muscle Function in Young Patients With Cystic Fibrosis. Article. Pediatric Exercise Science. 2016;28(3):364-373. doi:10.1123/pes.2015-0184
5. Rodriguez-Miguelez P, Seigler N, Ishii H, et al. Exercise Intolerance in Cystic Fibrosis: Importance of Skeletal Muscle. Med Sci Sports Exerc. Apr 1 2021;53(4):684-693. doi:10.1249/MSS.0000000000002521
6. Hebestreit H, Kriemler S, Schindler C, et al. Effects of a Partially Supervised Conditioning Program in Cystic Fibrosis: An International Multicenter, Randomized Controlled Trial (ACTIVATE-CF). Am J Respir Crit Care Med. Feb 1 2022;205(3):330-339. doi:10.1164/rccm.202106-1419OC
7. Radtke T, Smith S, Nevitt SJ, Hebestreit H, Kriemler S. Physical activity and exercise training in cystic fibrosis. Cochrane Database of Systematic Reviews. 2022;(8)doi:10.1002/14651858.CD002768.pub5
8. Ozipek M, Arikan H, Calik-Kutukcu E, et al. Deviations of body functions and structure, activity limitations, and participation restrictions of the International Classification of Functioning, Disability, and Health model in children with cystic fibrosis and non–cystic fibrosis bronchiectasis. Pediatric Pulmonology. 2020;55(5):1207-1216. doi:https://doi.org/10.1002/ppul.24708
9. Silverman SR, Schertz LA, Yuen HK, Lowman JD, Bickel CS. Systematic review of the methodological quality and outcome measures utilized in exercise interventions for adults with spinal cord injury. Spinal Cord. 2012/10/01 2012;50(10):718-727. doi:10.1038/sc.2012.78
10. Radtke T, Nevitt SJ, Hebestreit H, Kriemler S. Physical exercise training for cystic fibrosis. Cochrane Database of Systematic Reviews. 2017; (11) doi:10.1002/14651858.CD002768.pub4
11. Ben B, Latour JM, Tomlinson OW. A systematic review to explore how exercise-based physiotherapy via telemedicine can promote health related benefits for people with cystic fibrosis. PLOS Digital Health. 2023;2(2):e0000201. doi:10.1371/journal.pdig.0000201
12. Page MJ, McKenzie JE, Bossuyt PM, et al. The PRISMA 2020 statement: an updated guideline for reporting systematic reviews. BMJ. 2021;372:n71. doi:10.1136/bmj.n71
13. Alotaibi N, Lowman J, Horton T, et al. The Effects of Exercise and Physical Activity in People with Cystic Fibrosis Based on the ICF Domains. 2024. https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42023332797
14. Covidence. Covidence. 2024. https://www.covidence.org/
15. World Health Organization. International Classification of Functioning, Disability and Health. 2024. https://icd.who.int/browse/2024-01/icf/en
16. Cieza A, Brockow T, Ewert T, et al. Linking health-status measurements to the international classification of functioning, disability and health. J Rehabil Med. Sep 2002;34(5):205-10. doi:10.1080/ 165019702760279189
17. Cieza A, Fayed N, Bickenbach J, Prodinger B. Refinements of the ICF Linking Rules to strengthen their potential for establishing comparability of health information. Disability and Rehabilitation. 2019/02/27 2019;41(5):574-583. doi:10.3109/0963 8288.2016.1145258
18. Cieza A, Geyh S, Chatterji S, Kostanjsek N, Ustun B, Stucki G. ICF linking rules: an update based on lessons learned. J Rehabil Med. Jul 2005; 37(4):212-8. doi:10.1080/16501970510040263
19. Effective Public Healthcare Panacea Project. Quality Assessment Tool for Quantitative Studies. 2024. https://www.ephpp.ca/quality-assessment-tool-for-quantitative-studies/
20. Thomas BH, Ciliska D, Dobbins M, Micucci S. A Process for Systematically Reviewing the Literature: Providing the Research Evidence for Public Health Nursing Interventions. Worldviews on Evidence-Based Nursing. 2004;1(3):176-184. doi:https://doi.org/10.1111/j.1524-475X.2004.04006.x
21. Armijo-Olivo S, Stiles CR, Hagen NA, Biondo PD, Cummings GG. Assessment of study quality for systematic reviews: a comparison of the Cochrane Collaboration Risk of Bias Tool and the Effective Public Health Practice Project Quality Assessment Tool: methodological research. Journal of Evaluation in Clinical Practice. 2012;18(1):12-18. doi:https://doi.org/10.1111/j.1365-2753.2010.01516.x
22. Deeks JJ, Dinnes J, D'Amico R, et al. Evaluating non-randomised intervention studies. Health Technol Assess. 2003;7(27):iii-x, 1-173. doi:10.3310/hta7270
23. Jackson N, Waters E, for the Guidelines for Systematic Reviews in Health P, Public Health T. Criteria for the systematic review of health promotion and public health interventions. Health Promotion International. 2005;20(4):367-374. doi: 10.1093/heapro/dai022
24. Physiotherapy Evidence Database. PEDro scale. 2024. https://pedro.org.au/
25. Maher CG, Sherrington C, Herbert RD, Moseley AM, Elkins M. Reliability of the PEDro Scale for Rating Quality of Randomized Controlled Trials. Physical Therapy. 2003;83(8):713-721. doi: 10.1093/ptj/83.8.713
26. de Morton NA. The PEDro scale is a valid measure of the methodological quality of clinical trials: a demographic study. Australian Journal of Physiotherapy. 2009/01/01/ 2009;55(2):129-133. doi:https://doi.org/10.1016/S0004-9514(09)70043-1
27. Adair D, Hider A, Filbrun AG, et al. Assessing the Utility of an Outpatient Exercise Program for Children With Cystic Fibrosis: A Quality Improvement Project. Brief Research Report. Frontiers in Pediatrics. 2022-January-13 2022;9doi: 10.3389/fped.2021.734292
28. Almăjan-Guţă B, Cluci O, Rusu A, Ciuca O. Individualized unsupervised exercise programs and chest physiotherapy in children with cystic fibrosis. Timisoara Physical Education and Rehabilitation Journal. 2016;6(11):51-54. doi:doi:10.2478/tperj-2013-0018
29. Andréasson B, Jonson B, Kornfält R, Nordmark E, Sandström S. Long-term effects of physical exercise on working capacity and pulmonary function in cystic fibrosis. Acta Paediatr Scand. Jan 1987;76 (1):70-5. doi:10.1111/j.1651-2227.1987.tb10417.x
30. Asher MI, Pardy RL, Coates AL, Thomas E, Macklem PT. The effects of inspiratory muscle training in patients with cystic fibrosis. Am Rev Respir Dis. Nov 1982;126(5):855-9. doi:10.1164/ arrd.1982.126.5.855
31. Aspinall SP, Hill DM, Mackintosh KA, McNarry MA. Reduced exertion high-intensity interval training (REHIT) in an adult with Cystic Fibrosis: A mixed-methods case study. Respir Med Case Rep. 2020;31:101173. doi:10.1016/j.rmcr.2020.101173
32. Beaudoin N, Bouvet GF, Coriati A, Rabasa-Lhoret R, Berthiaume Y. Combined Exercise Training Improves Glycemic Control in Adult with Cystic Fibrosis. Med Sci Sports Exerc. Feb 2017;49 (2):231-237. doi:10.1249/mss.0000000000001104
33. Bieli C, Summermatter S, Boutellier U, Moeller A. Respiratory muscle training improves respiratory muscle endurance but not exercise tolerance in children with cystic fibrosis. Pediatr Pulmonol. Mar 2017;52(3):331-336. doi:10.1002/ppul.23647
34. Blau H, Mussaffi-Georgy H, Fink G, et al. Effects of an intensive 4-week summer camp on cystic fibrosis: pulmonary function, exercise tolerance, and nutrition. Chest. Apr 2002;121(4) :1117-22. doi:10.1378/chest.121.4.1117
35. Blomquist M, Freyschuss U, Wiman LG, Strandvik B. Physical activity and self treatment in cystic fibrosis. Arch Dis Child. Apr 1986;61(4):362-7. doi:10.1136/adc.61.4.362
36. Braggion C, Cornacchia M, Miano A, Schena F, Verlato G, Mastella G. Exercise tolerance and effects of training in young patients with cystic fibrosis and mild airway obstruction. Pediatr Pulmonol. 1989;7(3):145-52. doi:10.1002/ppul.1950070306
37. Carr SB, Ronan P, Lorenc A, Mian A, Madge SL, Robinson N. Children and Adults Tai Chi Study (CF-CATS2): a randomised controlled feasibility study comparing internet-delivered with face-to-face Tai Chi lessons in cystic fibrosis. ERJ Open Res. Oct 2018;4(4)doi:10.1183/23120541.00042-2018
38. Cerny FJ. Relative effects of bronchial drainage and exercise for in-hospital care of patients with cystic fibrosis. Phys Ther. Aug 1989;69(8):633-9. doi:10.1093/ptj/69.8.633
39. Clément M, Jankowski LW, Beaudry PH. Prone immersion physical exercise therapy in three children with cystic fibrosis: a pilot study. Nurs Res. Nov-Dec 1979;28(6):325-9.
40. de Jong W, Grevink RG, Roorda RJ, Kaptein AA, van der Schans CP. Effect of a home exercise training program in patients with cystic fibrosis. Chest. Feb 1994;105(2):463-8. doi:10.1378/chest. 105.2.463
41. de Jong W, van Aalderen WM, Kraan J, Koëter GH, van der Schans CP. Inspiratory muscle training in patients with cystic fibrosis. Respir Med. Jan 2001;95(1):31-6. doi:10.1053/rmed.2000.0966
42. Dekerlegand RL, Hadjiliadis D, Myslinski MJ, Holsclaw D, Ferrin M. Home-based Inspiratory Muscle Training In Adults With Cystic Fibrosis: A Case Series Report. Cardiopulmonary Physical Therapy Journal. 2014;25(3):85-91.
43. Del Corral T, Cebrià IIM, López-de-Uralde-Villanueva I, Martínez-Alejos R, Blanco I, Vilaró J. Effectiveness of a Home-Based Active Video Game Programme in Young Cystic Fibrosis Patients. Respiration. 2018;95(2):87-97. doi:10.1159/000481264
44. de Oliveira ACN, de Oliveira JCM, Mesquita-Ferrari RA, Damasceno N, Oliveira LVF, Sampaio LMM. Inflammatory process modulation in children with cystic fibrosis submitted to aerobic training. Archives of Medical Science. 2009 2023-11-19 2009;5(3):422.
45. de Oliveira CQ, de Matos Fonseca V, Senna G, Scudese E, de Miranda Chaves CR. Effects of Aerobic and Resistance Training on Body Composition and Physical Capacity of Adolescents with Cystic Fibrosis. Article. Journal of Exercise Physiology Online. 2017;20(2):55-63.
46. Donadio MVF, Cobo-Vicente F, San Juan AF, et al. Is exercise and electrostimulation effective in improving muscle strength and cardiorespiratory fitness in children with cystic fibrosis and mild-to-moderate pulmonary impairment?: Randomized controlled trial. Respir Med. May 2022;196:106 798. doi:10.1016/j.rmed.2022.106798
47. Dunlevy CL, Douce FH, Hill E, Baez S, Clutter J. Physiological and Psychological Effects of Low-Impact Aerobic Exercise on Young Adults with Cystic Fibrosis. Journal of Cardiopulmonary Rehabilitation and Prevention. 1994;14(1)
48. Edlund LD, French RW, Herbst JJ, Ruttenburg HD, Ruhling RO, Adams TD. Effects of a swimming program on children with cystic fibrosis. Am J Dis Child. Jan 1986;140(1):80-3. doi:10.1001/archpedi .1986.02140150082044
49. 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. Jan 10 2012;38:2. doi:10.1186/1824-7288-38-2
50. Elce A, Nigro E, Gelzo M, et al. Supervised physical exercise improves clinical, anthropometric and biochemical parameters in adult cystic fibrosis patients: A 2-year evaluation. Clin Respir J. Jul 2018;12(7):2228-2234. doi:10.1111/crj.12796
51. Enright S, Chatham K, Ionescu AA, Unnithan VB, Shale DJ. Inspiratory muscle training improves lung function and exercise capacity in adults with cystic fibrosis. Chest. Aug 2004;126(2):405-11. doi:10.1378/chest.126.2.405
52. Estévez-González AJ, Donadio MVF, Cobo-Vicente F, et al. Effects of a Short-Term Resistance-Training Program on Heart Rate Variability in Children With Cystic Fibrosis-A Randomized Controlled Trial. Front Physiol. 2021;12:652029. doi:10.3389/fphys.2021.652029
53. Franco CB, Ribeiro AF, Morcillo AM, Zambon MP, Almeida MB, Rozov T. Air stacking: effects of Pilates mat exercises on muscle strength and on pulmonary function in patients with cystic fibrosis. J Bras Pneumol. Oct 2014;40(5):521-7. doi:10.1590 /s1806-37132014000500008
54. Goldbart AD, Cohen AD, Weitzman D, Tal A. Effects of rehabilitation winter camps at the Dead Sea on European cystic fibrosis patients. Isr Med Assoc J. Nov 2007;9(11):806-9.
55. Gruber W, Orenstein DM, Braumann KM. Do responses to exercise training in cystic fibrosis depend on initial fitness level? Eur Respir J. Dec 2011;38(6):1336-42. doi:10.1183/09031936.00192510
56. 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. Jan 2014;13(1):86-91. doi:10. 1016/j.jcf.2013.06.005
57. Gruber W, Orenstein DM, Braumann KM, Hüls G. Health-related fitness and trainability in children with cystic fibrosis. Pediatr Pulmonol. Oct 2008;43 (10):953-64. doi:10.1002/ppul.20881
58. Gruber W, Orenstein DM, Braumann KM, Paul K, Hüls G. Effects of an exercise program in children with cystic fibrosis: are there differences between females and males? J Pediatr. Jan 2011;1 58(1):71-6. doi:10.1016/j.jpeds.2010.07.033
59. Gruber W, Stehling F, Olivier M, et al. Effects of a long-term exercise program on motor performance in children and adolescents with CF. Pediatr Pulmonol. Dec 2020;55(12):3371-3380. doi:10.1002/ppul.25064
60. Gruber W, Welsner M, Dillenhöfer S, et al. Health-Related and Motor Performance-Related Fitness and Physical Activity Among Youth With Cystic Fibrosis. Percept Mot Skills. Oct 2021;128 (5):2097-2116. doi:10.1177/00315125211036415
61. Gulmans VA, de Meer K, Brackel HJ, Faber JA, Berger R, Helders PJ. Outpatient exercise training in children with cystic fibrosis: physiological effects, perceived competence, and acceptability. Pediatr Pulmonol. Jul 1999;28(1):39-46. doi:10.1002/(sici) 1099-0496(199907)28:1<39::aid-ppul7>3.0.co;2-8
62. Gulmans V, van der Laag J, Wattimena D, et al. Insulin-like growth factors and leucine kinetics during exercise training in children with cystic fibrosis. J Pediatr Gastroenterol Nutr. Jan 2001;32 (1):76-81. doi:10.1097/00005176-200101000-00020
63. Güngör S, Gencer-Atalay K, Bahar-Özdemir Y, Keniş-Coşkun Ö, Karadağ-Saygı E. The clinical effects of combining postural exercises with chest physiotherapy in cystic fibrosis: A single-blind, randomized-controlled trial. Turk J Phys Med Rehabil. Mar 2021;67(1):91-98. doi:10.5606/tftrd. 2021.5214
64. Gupta S, Mukherjee A, Lodha R, et al. Effects of Exercise Intervention Program on Bone Mineral Accretion in Children and Adolescents with Cystic Fibrosis: A Randomized Controlled Trial. Indian J Pediatr. Nov 2019;86(11):987-994. doi:10.1007/s1 2098-019-03019-x
65. Hebestreit H, Kieser S, Junge S, et al. Long-term effects of a partially supervised conditioning programme in cystic fibrosis. Eur Respir J. Mar 2010;35(3):578-83. doi:10.1183/09031936.00062409
66. Hebestreit H, Kriemler S, Schindler C, et al. Effects of a Partially Supervised Conditioning Program in Cystic Fibrosis: An International Multicenter, Randomized Controlled Trial (ACTIVATE-CF). Am J Respir Crit Care Med. Feb 1 2022;205(3):330-339. doi:10.1164/rccm.202106-1419OC
67. Heijerman HG, Bakker W, Sterk PJ, Dijkman JH. Long-term effects of exercise training and hyperalimentation in adult cystic fibrosis patients with severe pulmonary dysfunction. Int J Rehabil Res. 1992;15(3):252-7. doi:10.1097/00004356-199 209000-00011
68. Heijerman HG, Bakker W, Sterk PJ, Dijkman JH. Oxygen-assisted exercise training in adult cystic fibrosis patients with pulmonary limitation to exercise. Int J Rehabil Res. 1991;14(2):101-15. doi:10.1097/00004356-199106000-00002
69. Holmes CJ, Racette SB, Symonds L, Arbeláez AM, Cao C, Granados A. Comparison of Bioelectrical Impedance Analysis with DXA in Adolescents with Cystic Fibrosis before and after a Resistance Training Intervention. Int J Environ Res Public Health. Mar 29 2022;19(7)doi:10.3390/ijerph19074037
70. Hommerding PX, Baptista RR, Makarewicz GT, et al. Effects of an educational intervention of physical activity for children and adolescents with cystic fibrosis: a randomized controlled trial. Respir Care. Jan 2015;60(1):81-7. doi:10.4187/respcare.02578
71. Horvat M, Carlile JR. Effects of progressive resistance exercise on physical functioning and self-concept in cystic fibrosis. 01/01 1991;45:18-23.
72. Hulzebos HJ, Snieder H, van der Et J, Helders PJ, Takken T. High-intensity interval training in an adolescent with cystic fibrosis: a physiological perspective. Physiother Theory Pract. Apr 2011;27 (3):231-7. doi:10.3109/09593985.2010.483266
73. Johnson S, Burger S, Williams J. Implementing Exercise Journaling and Exercise Encouragement in Hospitalized Adults with Cystic Fibrosis. Article. MEDSURG Nursing. 2021;30(4):263-274.
74. Kaltsakas G, Chynkiamis N, Anastasopoulos N, et al. Interval versus constant-load exercise training in adults with Cystic Fibrosis. Respir Physiol Neurobiol. Jun 2021;288:103643. doi:10.1016/j. resp.2021.103643
75. Kaplan TA, McKey RM, Jr., Toraya N, Moccia G. Impact of CF summer camp. Clin Pediatr (Phila). Mar 1992;31(3):161-7. doi:10.1177/000992289203100307
76. Kenis-Coskun Ö, Aksoy AN, Kumaş EN, et al. The effect of telerehabilitation on quality of life, anxiety, and depression in children with cystic fibrosis and caregivers: A single-blind randomized trial. Pediatr Pulmonol. May 2022;57(5):1262-1271. doi:10.1002/ppul.25860
77. 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. Apr 2004;125 (4):1299-305. doi:10.1378/chest.125.4.1299
78. Kriemler S, Kieser S, Junge S, et al. Effect of supervised training on FEV1 in cystic fibrosis: a randomised controlled trial. J Cyst Fibros. Dec 2013;12(6):714-20. doi:10.1016/j.jcf.2013.03.003
79. Ledger SJ, Owen E, Prasad SA, Goldman A, Willams J, Aurora P. A pilot outreach physiotherapy and dietetic quality improvement initiative reduces IV antibiotic requirements in children with moderate-severe cystic fibrosis. J Cyst Fibros. Dec 2013;12(6):766-72. doi:10.1016/j.jcf.2013.01.003
80. Massery M. Musculoskeletal and neuromuscular interventions: a physical approach to cystic fibrosis. J R Soc Med. 2005;98 Suppl 45(Suppl 45):55-66.
81. McCreery J, Mackintosh K, McNarry M. Effects of Inspiratory Muscle Training on Heart Rate Variability in Children with Cystic Fibrosis: A Pilot Study. Journal of Science in Sport and Exercise. 2021;3(1):66-74. doi:10.1007/s42978-020-00079-w
82. McNamara C, Johnson M, Read L, et al. Yoga Therapy in Children with Cystic Fibrosis Decreases Immediate Anxiety and Joint Pain. Evid Based Complement Alternat Med. 2016;2016:9429504. doi:10.1155/2016/9429504
83. Moorcroft AJ, Dodd ME, Morris J, Webb AK. Individualised unsupervised exercise training in adults with cystic fibrosis: a 1 year randomised controlled trial. Thorax. Dec 2004;59(12):1074-80. doi:10.1136/thx.2003.015313
84. O'Neill PA, Dodds M, Phillips B, Poole J, Webb AK. Regular exercise and reduction of breathlessness in patients with cystic fibrosis. Br J Dis Chest. Jan 1987;81(1):62-9. doi:10.1016/0007-0971(87)90109-4
85. Orenstein DM, Franklin BA, Doershuk CF, et al. Exercise conditioning and cardiopulmonary fitness in cystic fibrosis. The effects of a three-month supervised running program. Chest. Oct 1981;80(4):392-8. doi:10.1378/chest.80.4.392
86. Orenstein DM, Hovell MF, Mulvihill M, et al. Strength vs aerobic training in children with cystic fibrosis: a randomized controlled trial. Chest. Oct 2004;126(4):1204-14. doi:10.1378/chest.126.4.1204
87. Paranjape SM, Barnes LA, Carson KA, von Berg K, Loosen H, Mogayzel PJ, Jr. Exercise improves lung function and habitual activity in children with cystic fibrosis. J Cyst Fibros. Jan 2012;11(1):18-23. doi:10.1016/j.jcf.2011.08.003
88. Prévotat A, Godin J, Bernard H, Perez T, Le Rouzic O, Wallaert B. Improvement in body composition following a supervised exercise-training program of adult patients with cystic fibrosis. Respir Med Res. May 2019;75:5-9. doi:10. 1016/j.resmer.2019.04.001
89. Reuveny R, DiMenna FJ, Gunaratnam C, et al. High-intensity interval training accelerates oxygen uptake kinetics and improves exercise tolerance for individuals with cystic fibrosis. BMC Sports Sci Med Rehabil. 2020;12:9. doi:10.1186/s13102-020-0159-z
90. Rovedder PM, Flores J, Ziegler B, et al. Exercise programme in patients with cystic fibrosis: a randomized controlled trial. Respir Med. Aug 2014;108(8):1134-40. doi:10.1016/j.rmed.2014.04.022
91. Ruddy J, Emerson J, McNamara S, et al. Yoga as a Therapy for Adolescents and Young Adults With Cystic Fibrosis: A Pilot Study. Glob Adv Health Med. Nov 2015;4(6):32-6. doi:10.7453/gah mj.2015.061
92. Sahlberg M, Eriksson BO, Sixt R, Strandvik B. Cardiopulmonary data in response to 6 months of training in physically active adult patients with classic cystic fibrosis. Respiration. 2008;76(4):413-20. doi:10.1159/000156862
93. Sahlberg M, Svantesson U, Magnusson Thomas E, Andersson BA, Saltin B, Strandvik B. Muscular strength after different types of training in physically active patients with cystic fibrosis. Scand J Med Sci Sports. Dec 2008;18(6):756-64. doi:10.1111/j.1600-0838.2007.00691.x
94. 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. Oct 2014;48(20):1513-7. doi:10.1136/bjsport s-2012-091892
95. Santana Sosa E, 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. Jan 2012;44 (1):2-11. doi:10.1249/MSS.0b013e318228c302
96. Sartori R, Barbi E, Poli F, et al. Respiratory training with a specific device in cystic fibrosis: a prospective study. J Cyst Fibros. Jul 2008;7(4):313-319. doi:10.1016/j.jcf.2007.12.003
97. Sawyer A, Cavalheri V, Jenkins S, et al. High-Intensity Interval Training Is Effective at Increasing Exercise Endurance Capacity and Is Well Tolerated by Adults with Cystic Fibrosis. J Clin Med. Sep 25 2020;9(10)doi:10.3390/jcm9103098
98. Sawyer EH, Clanton TL. Improved pulmonary function and exercise tolerance with inspiratory muscle conditioning in children with cystic fibrosis. Chest. Nov 1993;104(5):1490-7. doi:10.1378/chest .104.5.1490
99. Schindel CS, Hommerding PX, Melo DA, Baptista RR, Marostica PJ, Donadio MV. Physical exercise recommendations improve postural changes found in children and adolescents with cystic fibrosis: a randomized controlled trial. J Pediatr. Mar 2015;166(3):710-6.e2. doi:10.1016/j. jpeds.2014.12.001
100. 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. Nov 2011;27(8):548-56. doi:10.3109/ 09593985.2010.545102
101. 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. Mar 2000;136(3):304-10. doi:10.1067/mpd.2000.103408
102. 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. Mar 2002;33(3):194-200. doi:10.1002/ ppul.10015
103. Shaw I, Kinsey JE, Richards R, Shaw BS. Individualized supervised resistance training during nebulization in adults with cystic fibrosis. Pak J Med Sci. Sep-Oct 2016;32(5):1152-1157. doi:10.12669/pjms.325.9960
104. Strauss GD, Osher A, Wang CI, et al. Variable weight training in cystic fibrosis. Chest. Aug 1987;92(2):273-6. doi:10.1378/chest.92.2.273
105. Tomlinson OW, Shelley J, Trott J, Bowhay B, Chauhan R, Sheldon CD. The feasibility of online video calling to engage patients with cystic fibrosis in exercise training. J Telemed Telecare. Jul 2020 ;26(6):356-364. doi:10.1177/1357633x19828630
106. Turchetta A, Salerno T, Lucidi V, Libera F, Cutrera R, Bush A. Usefulness of a program of hospital-supervised physical training in patients with cystic fibrosis. Pediatr Pulmonol. Aug 2004; 38(2):115-8. doi:10.1002/ppul.20073
107. Van Biervliet S, Declercq D, Dereeper S, Vermeulen D, Würth B, De Guschtenaere A. The effect of an intensive residential rehabilitation program on body composition in patients with cystic fibrosis. Eur J Pediatr. Jun 2021;180(6):1981-1985. doi:10.1007/s00431-021-03943-1
108. van de Weert-van Leeuwen PB, Hulzebos HJ, Werkman MS, et al. Chronic inflammation and infection associate with a lower exercise training response in cystic fibrosis adolescents. Respir Med. Mar 2014;108(3):445-52. doi:10.1016/j.rmed.2013.08.012
109. Vivodtzev I, Decorte N, Wuyam B, et al. Benefits of neuromuscular electrical stimulation prior to endurance training in patients with cystic fibrosis and severe pulmonary dysfunction. Chest. Feb 1 2013;143(2):485-493. doi:10.1378/chest.12-0584
110. Welsner M, Gruber W, Mellies U, et al. Trainability of Health-Related and Motor Performance Fitness in Adults with Cystic Fibrosis within a 12-Month Partially Supervised Exercise Program. Pulm Med. 2021;2021:5581812. doi:10. 1155/2021/5581812
111. Yalçınkaya G, Alpaydın AÖ, Özalevli S. Effect of pulmonary rehabilitation on adult cystic fibrosis patient: a case report. Pulmoner rehabilitasyonun erişkin kistik fibrozisli hasta üzerindeki etkisi: bir olgu sunumu. Journal of Exercise Therapy and Rehabilitation. January 2020;6(3):220-225.
112. Zach MS, Purrer B, Oberwaldner B. Effect of swimming on forced expiration and sputum clearance in cystic fibrosis. Lancet. Nov 28 1981;2(8 257):1201-3. doi:10.1016/s0140-6736(81)91440-9
113. Zach M, Oberwaldner B, Häusler F. Cystic fibrosis: physical exercise versus chest physiotherapy. Arch Dis Child. Aug 1982;57(8):587-9. doi:10.1136 /adc.57.8.587
114. Zeren M, Cakir E, Gurses HN. Effects of inspiratory muscle training on postural stability, pulmonary function and functional capacity in children with cystic fibrosis: A randomised controlled trial. Respir Med. Mar 2019;148:24-30. doi:10.1016/j.rmed.2019.01.013
115. Calthorpe RJ, Smith SJ, Rowbotham NJ, et al. What effective ways of motivation, support and technologies help people with cystic fibrosis improve and sustain adherence to treatment? BMJ Open Respir Res. Aug 2020;7(1)doi:10.1136/bmjre sp-2020-000601
116. Braithwaite J, Testa L, Lamprell G, et al. Built to last? The sustainability of health system improvements, interventions and change strategies: a study protocol for a systematic review. BMJ Open. Nov 12 2017;7(11):e018568. doi:10.11 36/bmjopen-2017-018568
117. Lima EA, Rodrigues G, Mota BC, Castro SS, Mesquita RB, Leite CF. Which Components of The International Classification of Functioning, Disability and Health (ICF) are Covered by Cardiac Rehabilitation Assessment Tools among Individuals with Heart Failure? Heart & Lung. 2024/01/01/ 2024;63:65-71. doi:https://doi.org/10.1016/j.hrtlng.2023.09.010
118. Rauch A, Kirchberger I, Stucki G, Cieza A. Validation of the Comprehensive ICF Core Set for obstructive pulmonary diseases from the perspective of physiotherapists. Physiother Res Int. Dec 2009;14(4):242-59. doi:10.1002/pri.448
119. Bui KL, Nyberg A, Maltais F, Saey D. Functional Tests in Chronic Obstructive Pulmonary Disease, Part 1: Clinical Relevance and Links to the International Classification of Functioning, Disability, and Health. Ann Am Thorac Soc. May 2017;14(5): 778-784. doi:10.1513/AnnalsATS.201609-733AS
120. Mandrusiak A, MacDonald J, Watter P. The International Classification of Functioning, Disability and Health: an effective model for describing young people with cystic fibrosis. Child: Care, Health and Development. 2009/01/01 2009; 35(1):2-4.doi:https://doi.org/10.1111/j.1365-2214.2008.00903.x
121. Mariani A, Gambazza S, Carta F, et al. Prevalence and factors associated with urinary incontinence in females with cystic fibrosis: An Italian single-center cross-sectional analysis. Pediatr Pulmonol. Jan 2022;57(1):132-141. doi:10. 1002/ppul.25723
122. Vargas L, Kazmerski TM, von Berg K, Patterson D, West N, Bradley M. A Qualitative Exploration of Women With Cystic Fibrosis and Urinary Incontinence. Urogynecology (Phila). Sep 19 2024; doi:10.1097/spv.0000000000001572
123. Button BM, Holland AE, Sherburn MS, Chase J, Wilson JW, Burge AT. Prevalence, impact and specialised treatment of urinary incontinence in women with chronic lung disease. Physiotherapy. Mar 2019;105(1):114-119. doi:10.1016/j.physio.20 18.07.006
124. Wheatley CM, Wilkins BW, Snyder EM. Exercise is medicine in cystic fibrosis. Exerc Sport Sci Rev. Jul 2011;39(3):155-60. doi:10.1097/JES.0b 013e3182172a5a
125. American College of Sports Medicine. Exercise is Medicine®. Accessed 11/11/2024, https://www.exerciseismedicine.org/
126. Hoffmann TC, Glasziou PP, Boutron I, et al. Better reporting of interventions: template for intervention description and replication (TIDieR) checklist and guide. BMJ : British Medical Journal. 2014;348:g1687. doi:10.1136/bmj.g1687
127. Quittner AL, Sweeny S, Watrous M, et al. Translation and linguistic validation of a disease-specific quality of life measure for cystic fibrosis. J Pediatr Psychol. Sep 2000;25(6):403-14. doi:10.10 93/jpepsy/25.6.403
128. Solé A, Olveira C, Pérez I, et al. Development and electronic validation of the revised Cystic Fibrosis Questionnaire (CFQ-R Teen/Adult): New tool for monitoring psychosocial health in CF. Journal of Cystic Fibrosis. 2018;17(5):672-679. doi:10.1016/j.jcf.2017.10.015
129. Borg GA. Psychophysical bases of perceived exertion. Med Sci Sports Exerc. 1982;14(5):377-81.
130. Wilson RC, Jones PW. A comparison of the visual analogue scale and modified Borg scale for the measurement of dyspnoea during exercise. Clin Sci (Lond). Mar 1989;76(3):277-82. doi:10.104 2/cs0760277
131. Bijur PE, Silver W, Gallagher EJ. Reliability of the Visual Analog Scale for Measurement of Acute Pain. Academic Emergency Medicine. 2001/12/01 2001;8(12):1153-1157. doi:https://doi.org/10.1111/j.1553-2712.2001.tb01132.x
132. Zigmond AS, Snaith RP. The hospital anxiety and depression scale. Acta Psychiatr Scand. Jun 1983;67(6):361-70. doi:10.1111/j.1600-0447.1983. tb09716.x
133. Smets EM, Garssen B, Bonke B, De Haes JC. The Multidimensional Fatigue Inventory (MFI) psychometric qualities of an instrument to assess fatigue. J Psychosom Res. Apr 1995;39(3):315-25. doi:10.1016/0022-3999(94)00125-o
134. Ruf KC, Fehn S, Bachmann M, et al. Validation of activity questionnaires in patients with cystic fibrosis by accelerometry and cycle ergometry. BMC Med Res Methodol. Apr 3 2012;12:43. doi:10.1186/1471-2288-12-43
135. Orenstein DM, Kaplan RM. Measuring the quality of well-being in cystic fibrosis and lung transplantation. The importance of the area under the curve. Chest. Oct 1991;100(4):1016-8. doi:10.1 378/chest.100.4.1016
136. Orenstein DM, Nixon PA, Ross EA, Kaplan RM. The quality of well-being in cystic fibrosis. Chest. Feb 1989;95(2):344-7. doi:10.1378/chest.95.2.344