Daily Monitoring of Respiratory Symptoms and Spirometry During ETI Treatment in Persons With Cystic Fibrosis.

The goal of this observational study is to provide optimal monitoring and support when initiating ETI treatment in eligible persons with cystic fibrosis (aged 12 y +) and to document on a daily basis, from 72 hours before the start of treatment and then for 14 days i) i) FEV1 changes (home spirometry), ii) ii) respiratory symptoms changes, iii) any possible side effects. Through a dedicated electronic platform, these data will be monitored every day by the medical team, which will be fully available for any questions or concerns patients may have.

Inclusion Criteria:Cystic fibrosis diagnosisAt least 12 years oldCarrier of at least one copy of F508del mutationAbility to perform reliable and reproducible spirometryMedical and psychological stabilitywritten consentowning a smartphoneExclusion Criteria:Lung transplantFEV1 > 120 % pr (%GLI) at the inclusion testPulmonary exacerbation