Airway clearance physiotherapy and health-related quality of life in cystic fibrosis.

OBJECTIVE: Airway clearance physiotherapy is recommended in cystic fibrosis, but limited evidence exists to suggest how much treatment is enough. As a secondary analysis of a prior study investigating the safety, efficacy, and participants' perceptions of a novel airway clearance technique, specific cough technique (SCT) compared to forced expiration technique (FET), we aimed to evaluate whether the intervention was associated with changes in health-related quality of life (HRQoL). METHODS: We conducted randomised, controlled individual trials with six adults (N-of-1 RCTs). Each trial included eight weeks of treatment, twice a week, using saline inhalation in horizontal positions, one with SCT and one with FET, in random order. Efficacy was measured by sputum wet weight (g) after each session. Perceived usefulness and preference were self-reported at the end of the study. Lung function was assessed at baseline and at the end of study. HRQoL was measured using the Cystic Fibrosis Questionnaire-Revised (CFQ-R) at baseline (week 1) and at completion of the study (week 8). Individual HRQoL scores (0-100) were coded and analysed using CFQ-R Software Program, version 2.0. RESULTS: Patient-reported outcomes were completed by all subjects. Individual CFQ-R-Respiratory Symptoms Scores (CFQ-R-RSS) showed a positive change, meeting the minimal important difference (MID) ≥ 4 points in five participants and a negative change in one individual. A strong correlation (r = 0.94 (p<0.01) was found between total sputum weight (g) and the positive changes in CFQ-R-RSS, and between changes in lung function and CFQ-R-RSS (r = 0.84 (p = 0.04). CONCLUSION: The airway clearance intervention was associated with clinically meaningful changes in patient-reported symptoms on the CFQ-R in the majority of the participants. This finding warrants further investigation regarding treatment, duration and frequency. A long-term study may reveal beneficial effects on other clinically meaningful endpoints, such as pulmonary exacerbations, high-resolution computed tomography scores and HRQoL. TRIAL REGISTRATION: The study was registered in ClinicalTrials.gov, under the number NCT0 1266473.

Evaluation of a novel technique in airway clearance therapy - Specific Cough Technique (SCT) in cystic fibrosis: A pilot study of a series of N-of-1 randomised controlled trials.

OBJECTIVES: The aim of this pilot study was to evaluate the efficacy, safety and participants' perception of a novel technique in airway clearance therapy - specific cough technique in cystic fibrosis. METHODS: We conducted randomised controlled individual trials (N-of-1 randomised controlled trials) in six adults. Each trial included 8 weeks of treatment with two interventions each week, one with specific cough technique and one with forced expiration technique. The efficacy was investigated by a blinded assessor measuring wet weight of sputum (g) after each session. Perceived usefulness and preference was self-reported at the end of study. Additional measurements included oxygen saturation and heart rate before and after each session and lung function (week 2). RESULTS: Three of six participants produced significantly higher mean sputum weight when using specific cough technique, differences being 21%, 38% and 23%, respectively. In three of the six participants, mean sputum weight was lower after forced expiration technique than after specific cough technique in each of the eight treatment pairs. Participant-reported outcomes were completed in all participants. Specific cough technique was reported to be easier to use in daily treatments and more normalising in everyday life. CONCLUSION: Specific cough technique was well tolerated and accepted by the participants with cystic fibrosis. Specific cough technique was non-inferior to forced expiration technique in terms of sputum production, thus specific cough technique appears to represent a promising alternative for clearing sputum in airway clearance therapy.

Pre-flight evaluation of adult patients with cystic fibrosis: a cross-sectional study.

BACKGROUND: Air travel may imply a health hazard for patients with cystic fibrosis (CF) due to hypobaric environment in the aircraft cabin. The objective was to identify pre-flight variables, which might predict severe hypoxaemia in adult CF patients during air travel. METHODS: Thirty adult CF-patients underwent pre-flight evaluation with spirometry, arterial oxygen tension (PaO2), pulse oximetry (SpO2) and cardiopulmonary exercise testing (CPET) at sea level (SL). The results were related to the PaO2 obtained during a hypoxia-altitude simulation test (HAST) in which a cabin altitude of 2438 m (8000 ft) was simulated by breathing 15.1% oxygen. RESULTS: Four patients fulfilled the criteria for supplemental oxygen during air travel (PaO2 HAST < 6.6 kPa). While walking slowly during HAST, another eleven patients dropped below PaO2 HAST 6.6 kPa. Variables obtained during CPET (PaO2 CPET, SpO2 CPET, minute ventilation/carbon dioxide output, maximal oxygen uptake) showed the strongest correlation to PaO2 HAST. CONCLUSIONS: Exercise testing might be of value for predicting in-flight hypoxaemia and thus the need for supplemental oxygen during air travel in CF patients. Trial registration The study is retrospectively listed in the ClinicalTrials.gov Protocol Registration System: NCT01569880 (date; 30/3/2012).