Cystic Fibrosis
Conditions
Keywords
cystic fibrosis, CFTR modulators, 6-minutes walk test, physical activity
Brief summary
The aim of the study is to identify a link between the new CFTR modulators and physical activity in cystic fibrosis patients. The triple combination of CFTR modulators (ELEXACAFTOR / TEZACAFTOR/ IVACAFTOR) has recently changed the management of cystic fibrosis. This treatment has been shown to rapidly improve patients' respiratory function, with a gain in FEV1 at 1 month ranging from 10.4% to 13.6%. It also reduces the number of respiratory exacerbations and improves the nutritional status and quality of life of treated patients. To date, there is limited data on the impact of these new therapies on physical activity. Few studies have investigated changes in exercise or physical activity parameters under ELEXACAFTOR / TEZACAFTOR/ IVACAFTOR. The 6-minute walk test is a validated field test used routinely to assess the exercise capacity of patients with chronic respiratory diseases, including cystic fibrosis.
Interventions
OTHER6-minute walk test
6-minute walk test in a 40-metre corridor. Included patients are used to performing this test, which is part of the standard care performed during patient follow-up, at least once a year. Parameters collected during the test: distance walked, minimum SpO2, average SpO2, maximum heart rate, average heart rate, average heart rate over the last 2 minutes, dyspnea scale.
Sponsors
Hospices Civils de Lyon
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
12 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Patients aged ≥ 12 years with cystic fibrosis, heterozygous for the F508del mutation of the CFTR gene, treated with ELEXACAFTOR/ TEZACAFTOR/ IVACAFTOR. * Patients followed in the respiratory diseases department of the Renee Sabran Hospital * 6-minute walked test performed in the year preceding initiation of ELEXACAFTOR / TEZACAFTOR/ IVACAFTOR * 6-minute walked test performed after treatment initiation (between the 3rd month and the 14th month) * No opposition to participation
Exclusion criteria
* \- Age \< 12 years * Pregnancy * Discontinuation or reduction in dosage of ELEXACAFTOR/ TEZACAFTOR/ IVACAFTOR * Patients objecting to the use of their data for research purposes
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| distance walked in the 6-minute walk test (metres) | between the 3rd month and the 14th month post treatment initiation | Distance walked in the 6-minute walk test, compared before and after the introduction of ELEXACAFTOR/ TEZACAFTOR/ IVACAFTOR. |
Countries
France
Outcome results
None listed