The timing of hypertonic saline (HTS) and airway clearance techniques (ACT) in adults with Cystic Fibrosis (CF) during pulmonary exacerbation: Pilot data form a randomised crossover study.

BACKGROUND: Streamlining the timing of treatments in cystic fibrosis (CF) is important to optimise adherence while ensuring efficacy. The optimal timing of treatment with hypertonic saline (HTS) and airway clearance techniques (ACT) is unknown. OBJECTIVES: This study hypothesised that HTS before ACT would be more effective than HTS during ACT as measured by Lung Clearance Index (LCI). METHODS: Adults with CF providing written informed consent were randomised to a crossover trial of HTS before ACT or HTS during ACT on consecutive days. ACT treatment consisted of Acapella Duet. Patients completed LCI and spirometry at baseline and 90 min post treatment. Mean difference (MD) and 95% CIs were reported. RESULTS: 13 subjects completed the study (mean (SD) age 33 (12) years, forced expiratory volume in 1second % (FEV1%) predicted 51% (22), LCI (no. turnovers) 14 (4)). Comparing the two treatments (HTS before ACT vs HTS during ACT), the change from baseline to 90 min post treatment in LCI (MD (95% CI) -0.02 (-0.63 to 0.59)) and FEV1% predicted (MD (95% CI) -0.25 (-2.50 to 1.99)) was not significant. There was no difference in sputum weight (MD (95% CI) -3.0 (-14.9 to 8.9)), patient perceived ease of clearance (MD (95% CI) 0.4 (-0.6 to 1.3) or satisfaction (MD (95% CI) 0.4 (-0.6 to 1.5)). The time taken for HTS during ACT was significantly shorter (MD (95% CI) 14.7 (9.8 to 19.6)). CONCLUSIONS: In this pilot study, HTS before ACT was no more effective than HTS during ACT as measured by LCI. TRIAL REGISTRATION NUMBER: NCT01753869; Pre-results

Changing epidemiology and clinical issues arising in an ageing cystic fibrosis population

Improvements in the quality and implementation of medical care for individuals with cystic fibrosis (CF) have resulted in a dramatic improvement in survival. Many of these strategies have focused on the effective management of pulmonary disease which has delayed its manifestations into later years. With an increasing number of patients surviving to later years the impact of chronic inflammation and nutritional compromise on other organ systems over a lifetime are increasingly manifest. This review highlights the changing epidemiology of the ageing CF population and the complications that may ensue