31 Effects of ivacaftor in cystic fibrosis patients carrying a non-G551D gating mutation

Abstract

patients with the G551Dmutation highlight opportunities to understand EOR in ‘real world’ people with CF. We aimed to evaluate symptoms of EOR in people with CF prior and during treatment with ivacaftor. Methods: We collected 52 week data from 12 adults with CF and the G551D mutation from a regional centre at baseline, and following 6, 26 and 52 weeks of treatment with ivacaftor (150mg bd). EOR was measured by questionnaire [reflux symptom index (RSI), Hull airway reflux questionnaire (HARQ) score >13 EOR symptomatic]. Sweat chloride (mmol/L), lung function (FEV1 and FVC % of predicted) and weight gain (kg and kg/m) were also measured. Results: See the table.