Physiotherapy and cystic fibrosis: what is the evidence base?

PURPOSE OF REVIEW: To provide a comprehensive overview and evidence to support the role of physiotherapy in the management of individuals with cystic fibrosis (CF) including airway clearance, exercise, and musculoskeletal concerns which can affect activities of daily living and respiratory health. RECENT FINDINGS: Several long-term studies have looked at the efficacy of airway clearance techniques, including active cycle of breathing techniques, autogenic drainage, high frequency chest wall oscillation, postural drainage, positive expiratory pressure (PEP), and oscillating PEP. Each of these studies reported some efficacy of airway clearance in maintaining health with no one technique being superior to another. However, one study suggested that high frequency chest wall oscillation was not as effective as PEP in maintaining health in CF patients. Individual preference needs to be considered when selecting a technique. Recent studies have found exercise to increase mucociliary clearance peripherally. Musculoskeletal issues, including posture, bone density, urinary incontinence, and pain should be assessed and managed in individuals to improve the mechanics of breathing and overall well-being. SUMMARY: The role of physiotherapy in CF is complex and includes airway clearance, exercise, and management of the long-term sequelae of musculoskeletal issues. More rigorous physiotherapy studies are required to assist with evidence based practice.

Long-term multicentre randomised controlled study of high frequency chest wall oscillation versus positive expiratory pressure mask in cystic fibrosis.

BACKGROUND: Positive expiratory pressure (PEP) is the most commonly used method of airway clearance (AC) in Canada for patients with cystic fibrosis (CF) whereas, in some countries, high frequency chest wall oscillation (HFCWO) is the preferred form of AC. There have been no long-term studies comparing the efficacy of HFCWO and PEP in the CF population. OBJECTIVES: To determine the long-term efficacy of HFCWO compared with PEP mask therapy in the treatment of CF as measured by the number of pulmonary exacerbations (PEs). METHODS: A randomised controlled study was performed in 12 CF centres in Canada. After a 2-month washout period, subjects were randomised to perform either HFCWO or PEP mask therapy for 1 year. RESULTS: 107 subjects were enrolled in the study; 51 were randomised to PEP and 56 to HFCWO. There were 19 dropouts within the study period, of which 16 occurred prior to or at the time of randomisation. There were significant differences between the groups in the mean number of PEs (1.14 for PEP vs 2.0 for HFCWO) and time to first PE (220 days for PEP vs 115 days for HFCWO, p=0.02). There was no significant difference in lung function, health-related quality of life scores or patient satisfaction scores between the two groups. PEP mask therapy required a shorter treatment time. CONCLUSIONS: The results of this study favour PEP and do not support the use of HFCWO as the primary form of AC in patients with CF. CLINICAL TRIAL REGISTRATION NUMBER: NCT00817180.