The recent development of triple CFTR modulation means that up to 90% of people with CF will be eligible for ‘highly effective’ CFTR modulator therapy. In this podcast, Rachael Moses, Multimedia Editor of Thorax, and Dr Peter Barry, Consultant Respiratory Physician, Manchester Adult Cystic Fibrosis Centre, discuss the long-term adherence to and effects of ivacaftor on lung function, weight and healthcare utilisation in people with cystic fibrosis and the Gly551Asp mutation.