Effects of Lumacaftor/Ivacaftor on physical activity and exercise tolerance in three adults with cystic fibrosis

Daniela Savi, Stefano Schiavetto, Nicholas J Simmonds, Dario Righelli, Paolo Palange

Research output: Contribution to journalArticlepeer-review

Abstract

The combination of the corrector lumacaftor with the potentiator ivacaftor has been approved for treatment of cystic fibrosis (CF) patients homozygous for the Phe508del CFTR mutation. There are no reports detailing the effect of lumacaftor-ivacaftor on physical activity (PA) and exercise tolerance. We performed incremental cardiopulmonary exercise testing (CPET) and we assessed PA pre- and post 2 years initiation of lumacaftor-ivacaftor in three CF adults. PA of mild intensity improved by +13% in patient 1, + 84% in patients 2 and + 89% in patient 3. Oxygen uptake increased both at anaerobic threshold and at peak exercise (patient 1 + 33%, patient 2 + 42% and patient 3 + 20%). Daily physical activities and exercise tolerance improved after two years of lumacaftor-ivacaftor therapy.

Original languageEnglish
JournalJournal of Cystic Fibrosis
DOIs
Publication statusE-pub ahead of print - Mar 14 2019
Externally publishedYes

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