Perceived burden of respiratory physiotherapy in people with cystic fibrosis taking elexacaftor-tezacaftor-ivacaftor combination: a 1-year observational study.

Background: To limit the progression of disease, people with cystic fibrosis (pwCF) perform daily respiratory physiotherapy, which is perceived as the most burdensome routine in managing their condition. The elexacaftor-tezacaftor-ivacaftor (ETI) combination has changed respiratory management.

Objective: To investigate how the perceived treatment burden changed in 1 year of treatment with ETI.

Time Free From Hospitalization in Children and Adolescents With Cystic Fibrosis: Findings From FEV, Lung Clearance Index and Peak Work Rate.

Background: An exercise test combined with a multiple breath washout nitrogen test (MBWN) may offer a comprehensive clinical evaluation of cystic fibrosis (CF) disease in children with normal spirometry. The purpose of the present study is to explore whether information derived from spirometry, MBWN, and exercise tests can help the CF multidisciplinary team to characterize time free from hospitalization due to pulmonary exacerbation (PE) in a cohort of pediatric patients with CF.

Methods: This prospective observational study was carried out at the Lombardia Region Reference Center for Cystic Fibrosis in Milano, Italy.