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

CHC Montlegia

Status: Active, not recruiting

Conditions: Cystic Fibrosis

Treatments: Drug: Elexacaftor 100 MG / Ivacaftor 75 MG / Tezacaftor 50 MG, 2 tablets each morning + Ivacaftor 150 mg one tablet each evening

Study type: Observational

Funder types: Other

Identifiers

NCT05599230

ML001

Details and patient eligibility

About

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.

Enrollment: 20 patients

Sex: All

Ages: 12+ years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• Cystic fibrosis diagnosis
• At least 12 years old
• Carrier of at least one copy of F508del mutation
• Ability to perform reliable and reproducible spirometry
• Medical and psychological stability
• written consent
• owning a smartphone

Exclusion criteria

• Lung transplant
• FEV1 > 120 % pr (%GLI) at the inclusion test
• Pulmonary exacerbation

Trial design

20 participants in 1 patient group

CF patients before (3 days) and during (14 days) ETI treatment

Description:

Patients over 12 years old and eligible will start ETI treatment ( current standart treatment). * Morning: Elexacaftor 100 mg, Tezacaftor 50 mg, Ivacaftor 75 mg, 2 tablets * Evening: Ivacaftor 150 mg

Treatment:

Drug: Elexacaftor 100 MG / Ivacaftor 75 MG / Tezacaftor 50 MG, 2 tablets each morning + Ivacaftor 150 mg one tablet each evening