REVIEW
Physiotherapy in cystic fibrosis Α comprehensive clinical overview
More details
Hide details
1
Health, Sports and Bioscience, University of East London, UK
Corresponding author
Arietta Spinou
Health, Sports and Bioscience,
University of East London, Water Lane,
Stratford, E15 4LZ, London, UK
Pneumon 2018;31(1):35-43
KEYWORDS
ABSTRACT
Physiotherapy remains the cornerstone of cystic fibrosis (CF) management alongside medical treatment. Traditionally, physiotherapy intervention focussed on airway clearance during the clinically stable stage and chest infections. Research evidence consistently supports greater mucus clearance with chest physiotherapy compared to cough alone or no treatment. Various methods and techniques of airway clearance have been developed and investigated, and data suggest that most of them are of similar effectiveness. Nowadays physiotherapy management also extends to other areas, supported by studies and clinical practice. The physiotherapists plan, supervise and follow-up systematic exercise or personalised rehabilitation programs, which, similarly to airway clearance, are recommended in all patients with CF. Furthermore, based on a comprehensive assessment, physiotherapists incorporate the management of accompanying musculoskeletal problems such as back pain and postural disorders, as well as urine incontinence issues. In the era that aims to improve quality of life, it is essential that physiotherapists are aware of specific conditions that might affect the management of CF. Their role is to work alongside and within the CF multi-disciplinary team throughout patient’s treatment and consistently support the patient and carers, in particular whilst on clinical pathways of the lung transplantation and palliative care.
CONFLICTS OF INTEREST
No conflict of interest.
FUNDING
None
REFERENCES (85)
1.
Elborn JS. Cystic fibrosis. Lancet 2016; 388:2519-31.
2.
Matsui H, Grubb BR, Tarran R, et al. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell 1998; 95:1005-15.
3.
Pryor JA, Tannenbaum E, Scott SF, et al. Beyond postural drainage and percussion: Airway clearance in people with cystic fibrosis. Journal of Cystic Fibrosis 2010; 9:187-92.
4.
Bott J, Blumenthal S, Buxton M, et al. Guidelines for the physiotherapy management of the adult, medical, spontaneously breathing patient. Thorax 2009; 64:(Suppl 1):i1-51.
5.
National Guideline Alliance (UK). Cystic fibrosis: diagnosis and management. London: National Institute for Health and Care Excellence (UK); 2017 Oct.
6.
Flume PA, Mogayzel JP Jr, Robinson AK, et al. Cystic fibrosis pulmonary Guidelines. Treatment of pulmonary exacerbations. American Journal of Respiratory and Critical Care Medicine 2009; 180:802-8.
7.
Warnock L, Gates A. Chest physiotherapy compared to no chest physiotherapy for cystic fibrosis. Cochrane Database Syst Rev 2015:Cd001401.
8.
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.
9.
Main E, Prasad A, van der Schans CP. Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis. Cochrane Database of Systematic Reviews, 2005.
10.
Morrison L, Agnew J. Oscillating devices for airway clearance in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2014.
11.
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.
12.
McKoy NA, Wilson LM, Saldanha IJ, Odelola OA, Robinson KA. Active cycle of breathing technique for cystic fibrosis. Cochrane Database of Systematic Reviews 2016.
13.
McIlwaine M, Button B, Dwan K. Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2015.
14.
McIlwaine M, Wong LT, Chilvers M, Davidson GF. Long-term comparative trial of two different physiotherapy techniques; postural drainage with percussion and autogenic drainage, in the treatment of cystic fibrosis. Pediatr Pulmonol 2010;45:1064- 9.
15.
Lester MK, Flume PA. Airway-clearance therapy guidelines and implementation. Respir Care 2009; 54:733-50; discussion 751-3.
16.
Homnick DN. Making airway clearance successful. Paediatric Respiratory Reviews 2007; 8:40-5.
17.
Abbott J, Dodd M, Bilton D, Webb AK. Treatment compliance in adults with cystic fibrosis. Thorax 1994; 49:115-20.
18.
Goodfellow NA, Goodfellow AN, Hawwa FA, Reid JMA, Horne R, Shields DM, McElnay CJ. Adherence to treatment in children and adolescents with cystic fibrosis: a cross-sectional, multimethod study investigating the influence of beliefs about treatment and parental depressive symptoms. BMC Pulm Med 2015; 15:43.
19.
Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, Boucher RC. Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N Engl J Med 2006; 354:241-50.
20.
Wark P, McDonald VM. Nebulised hypertonic saline for cystic fibrosis. Cochrane Database of Systematic Reviews 2009.
21.
Elkins MR, Robinson M, Rose RB, et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 2006; 354:229-40.
22.
Dentice RL, Elkins RM, Middleton GP, et al. A randomised trial of hypertonic saline during hospitalisation for exacerbation of cystic fibrosis. Thorax 2016; 71:141-7.
23.
Elkins M, Dentice R. Timing of hypertonic saline inhalation for cystic fibrosis. Cochrane Database Syst Rev 2016; 12:Cd008816.
24.
Yang C, Chilvers M, Montgomery M, Nolan SJ. Dornase alfa for cystic fibrosis. Cochrane Database of Systematic Reviews 2016.
25.
Dentice R, Elkins M. Timing of dornase alfa inhalation for cystic fibrosis. Cochrane Database of Systematic Reviews, 2016.
26.
Bishop JR, Erskine OJ, Middleton PG. Timing of dornase alpha inhalation does not affect the efficacy of an airway clearance regimen in adults with cystic fibrosis: a randomised crossover trial. J Physiother 2011; 57:223-9.
27.
Shak S, Capon DJ, Hellmiss R, Marsters SA, Baker CL. Recombinant human DNase I reduces the viscosity of cystic fibrosis sputum. Proc Natl Acad Sci U S A, 1990; 87:9188-92.
28.
Daviskas E, Anderson SD, Jaques A, Charlton B. Inhaled mannitol improves the hydration and surface properties of sputum in patients with cystic fibrosis. CHEST Journal 2010; 137:861-8.
29.
Nolan SJ, Thornton J, Murray CS, Dwyer T. Inhaled mannitol for cystic fibrosis. Cochrane Database Syst Rev 2015:Cd008649. doi: 10.1002/14651858.CD008649.pub2.
30.
Bilton D, Bellon G, Charlton B, et al. Pooled analysis of two large randomised phase III inhaled mannitol studies in cystic fibrosis. Journal of Cystic Fibrosis, 2013; 12:367-76.
31.
Ibrahim WH. Massive haemoptysis: the definition should be revised. European Respiratory Journal 2008; 32:1131-2.
32.
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.
33.
Flume PA. Pneumothorax in cystic fibrosis. Curr Opin Pulm Med 2011; 17:220-5.
34.
MacDuff A, Arnold A, Harvey J. Management of spontaneous pneumothorax: British Thoracic Society Pleural Disease Guideline 2010. Thorax 2010; 65:Suppl 2:ii18-31.
35.
Flume PA. Pneumothorax in cystic fibrosis. Chest 2003; 123:217- 21.
36.
Smidt N, de Vet HC, Bouter LM, et al. Effectiveness of exercise therapy: a best-evidence summary of systematic reviews. Aust J Physiother 2005; 51:71-85.
37.
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.
38.
Radtke T, Nolan SJ, Hebestreit H, Kriemler S. Physical exercise training for cystic fibrosis. Cochrane Database Syst Rev 2015:Cd002768. doi: 10.1002/14651858.CD002768.pub3.
39.
Holland AE, Spruit MA, Troosters T, et al. An official European Respiratory Society/American Thoracic Society technical standard: field walking tests in chronic respiratory disease. Eur Respir J 2014; 44:1428-46.
40.
McCool FD, Rosen MJ. Nonpharmacologic airway clearance therapies: ACCP evidence-based clinical practice guidelines. Chest 2006; 129(1 Suppl):250s-259s.
41.
Bilton D, Dodd ME, Abbot JV, Webb AK. The benefits of exercise combined with physiotherapy in the treatment of adults with cystic fibrosis. Respiratory Medicine 1992; 86:507-11.
42.
Dwyer TJ, Alison JA, McKeough ZJ, Daviskas E, Bye PTP. Effects of exercise on respiratory flow and sputum properties in patients with cystic fibrosis. Chest 2011; 139:870-7.
43.
Lee AL, Rawlings S, Bennett KA, Armstrong D. Pain and its clinical associations in individuals with cystic fibrosis: A systematic review. Chron Respir Dis 2016; 13:102-17.
44.
Hayes M, Yaster M, Haythornthwaite JA, et al. Pain is a common problem affecting clinical outcomes in adults with cystic fibrosis. Chest 2011; 140:1598-603.
45.
Barker N, Raghavan A, Buttling P, Douros K, Lloyd Everard M. Thoracic kyphosis is now uncommon amongst children and adolescents with cystic fibrosis. Front Pediatr 2014; 2:11.
46.
Tejero Garcia 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.
47.
Sermet-Gaudelus I, Castanet M, Retsch-Bogart G, Aris MR. Update on cystic fibrosis-related bone disease: A special focus on children. Paediatric Respiratory Reviews 2009; 10:134-42.
48.
White D, Stiller K, Roney F. The prevalence and severity of symptoms of incontinence in adult cystic fibrosis patients. Physiotherapy Theory and Practice 2000; 16:35-42.
49.
Cornacchia M, Zenorini A, Perobelli S, Zanolla L, Mastella G, Braggion C. Prevalence of urinary incontinence in women with cystic fibrosis. BJU International 2001; 88:44-8.
50.
Moran F, Bradley JM, Boyle L, Elborn JS. Incontinence in adult females with cystic fibrosis: A northern Ireland survey. International Journal of Clinical Practice 2003; 57:182-3.
51.
Burge AT, Holland AE, Sherburn M, et al. Prevalence and impact of urinary incontinence in men with cystic fibrosis. Physiotherapy 2015; 101:166-70.
52.
Dodd ME, Prasad SA. Physiotherapy management of cystic fibrosis. Chronic Respiratory Disease 2005; 2:139-49.
53.
Orr A, McVean RJ, Webb AK, Dodd ME, et al. Questionnaire survey of urinary incontinence in women with cystic fibrosis. BMJ 2001; 322:1521.
54.
Abbott J. Health-related quality of life measurement in cystic fibrosis: advances and limitations. Chron Respir Dis 2009; 6:31-41.
55.
Dodd ME, Langman H. Urinary incontinence in cystic fibrosis. Journal of the Royal Society of Medicine 2005; 98(Suppl 45):28- 36.
56.
Reichman G, De Boe V, Braeckman J, Michielsen D. Urinary incontinence in patients with cystic fibrosis. Scandinavian Journal of Urology 2016; 50:128-31.
57.
McVean RJ, Orr A, Webb AK, et al. Treatment of urinary incontinence in cystic fibrosis. Journal of Cystic Fibrosis 2003; 2:171-6.
58.
Nankivell G, Caldwell P, Follett J. Urinary incontinence in adolescent females with cystic fibrosis. Paediatric Respiratory Reviews 2010; 11:95-9.
59.
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.
60.
Kelly A, Moran A. Update on cystic fibrosis-related diabetes. J Cyst Fibros 2013; 12:318-31.
61.
Lanng S. Glucose intolerance in cystic fibrosis patients. Paediatr Respir Rev 2001; 2:253-9.
62.
Antoniou S, Elston C. Cystic fibrosis. Medicine 2016; 44:321-5.
63.
Abbott J, Hurley AM, Morton MA, Conway PS. Longitudinal association between lung function and health-related quality of life in cystic fibrosis. Thorax 2013; 68:149-54.
64.
Gee L, Abbott J, Conway S, Etherington C, Webb A. Development of a disease specific health related quality of life measure for adults and adolescents with cystic fibrosis. Thorax 2000; 55:946-54.
65.
Ward N, Stiller K, Rowe H, Holland AE. The psychometric properties of the Leicester Cough Questionnaire and Respiratory Symptoms in CF tool in cystic fibrosis: A preliminary study. J Cyst Fibros 2017; 16:425-32.
66.
Abbott J, Hart A, Havermans T, et al. Measuring health-related quality of life in clinical trials in cystic fibrosis. J Cyst Fibros 2011; 10(Suppl 2):S82-5.
67.
Modi AC, Quittner AL. Validation of a disease-specific measure of health-related quality of life for children with cystic fibrosis. J Pediatr Psychol 2003; 28:535-45.
68.
Henry B, Aussage P, Grosskopf C, Goehrs JM. Development of the Cystic Fibrosis Questionnaire (CFQ) for assessing quality of life in pediatric and adult patients. Qual Life Res 2003; 12:63-76.
69.
Quittner AL, Buu A, Messer MA, Modi AC, Watrous M. Development and validation of The Cystic Fibrosis Questionnaire in the United States: a health-related quality-of-life measure for cystic fibrosis. Chest 2005; 128:2347-54.
70.
Alpern AN, Brumback LC, Ratjen F, Rosenfeld M, Davis SD, Quittner AL. Initial evaluation of the Parent Cystic Fibrosis Questionnaire--Revised (CFQ-R) in infants and young children. J Cyst Fibros 2015; 14:403-11.
71.
Elphick HE, Mallory G. Oxygen therapy for cystic fibrosis. Cochrane Database of Systematic Reviews 2013; (7):CD003884. doi: 10.1002/14651858.CD003884.pub4.
72.
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.
73.
Hardinge M, Annandale J, Bourne S, et al. British Thoracic Society guidelines for home oxygen use in adults. Thorax 2015; 70(Suppl 1):i1-43.
74.
Flume PA, Robinson KA, O’Sullivan BP, et al; Clinical Practice Guidelines for Pulmonary Therapies Committee. Cystic Fibrosis Pulmonary Guidelines: Airway clearance therapies. Respiratory Care 2009; 54:522-37.
75.
Stanford G, Parrott H, Bilton D, Agent P. Positive pressure- analysing the effect of the addition of non-invasive ventilation (NIV) to home airway clearance techniques (ACT) in adult cystic fibrosis (CF) patients. Physiother Theory Pract 2015; 31:270-4.
76.
Moran F, Bradley JM, Piper AJ. Non-invasive ventilation for cystic fibrosis. Cochrane Database of Systematic Reviews 2013;(4):CD002769. doi: 10.1002/14651858.CD002769.pub4.
77.
Oates GR, Stepanikova I, Gamble S, Gutierrez HH, Harris WT. Adherence to airway clearance therapy in pediatric cystic fibrosis: Socioeconomic factors and respiratory outcomes. Pediatr Pulmonol 2015; 50:1244-52.
78.
Proesmans M. Best practices in the treatment of early cystic fibrosis lung disease. Therapeutic Advances in Respiratory Disease 2017; 11:97-104.
79.
Davidson KL. Airway clearance strategies for the pediatric patient. Respir Care 2002; 47:823-8.
80.
Button BM, Heine RG, Catto-Smith AG, Phelan PD, Olinsky A. Chest physiotherapy, gastro-oesophageal reflux, and arousal in infants with cystic fibrosis. Arch Dis Child 2004; 89:435-9.
81.
Santos MD, Milross MA, Eisenhuth JP, Alison JA. Pressures and oscillation frequencies generated by bubble-positive expiratory pressure devices. Respir Care 2017; 62:444-50.
82.
Flume PA, O’Sullivan BP, Robinson KA, et al; Cystic Fibrosis Foundation, Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med 2007; 176:957-69.
83.
Lannefors L, Button BM, McIlwaine M. Physiotherapy in infants and young children with cystic fibrosis: current practice and future developments. J R Soc Med 2004; 97:(Suppl 44):8-25.
84.
Rowe SM, Miller S, Sorscher EJ. Cystic fibrosis. N Engl J Med 2005; 352:1992-2001.
85.
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.