EPS1.04 Change in lung clearance index in adolescents with cystic fibrosis treated with elexacaftor/tezacaftor/ivacaftor: results from 12 months follow-up in the Danish cystic fibrosis cohort

Abstract

Objectives: The efficacy of elexacaftor/tezacaftor/ivacaftor (ETI) on cystic fibrosis (CF) lung disease as measured by percent predicted forced expiratory volume in 1 second (ppFEV1) has been well-established in adults. However, ppFEV1 is not sensitive enough to detect small airway disease progression at early stages of lung disease in children. In the eligible Danish pediatric cohort, we aimed to assess the real-world effectiveness of ETI on small airway disease using multiple breath washout (MBW).