Ivacaftor will restore CFTR function in treated CF patients with the G551D mutation. Improvement in ventilation, salt balance and well-being will contribute to better exercise capacity at all levels of lung function. While potential improvements may be variable across the spectrum of lung function, even small gains at low levels of FEV1 may have significant benefit for some subjects.
Name: ivacaftor
Description: active armType: DrugIvacaftor
Name: placebo
Description: active armType: DrugPlacebo
Description: Respiratory exercise testing, including spirometry and V02 max.
Measure: exercise capacity Time: one month, 3 monthsDescription: Cytokine levels (IL-1β, IL-6, TNFα, IL-8, VEGF & Activin A) determined using cytometric bead analysis and / or ELISA
Measure: Inflammatory profile Time: One month, 3 monthsAllocation: Randomized
Crossover Assignment
There is one SNP
Airway Infection, Inflammatory Markers and Exercise Capacity in Patients With Cystic Fibrosis and at Least One G551D Mutation Taking VX770 (Ivacaftor). --- G551D ---
CPET in CF Patients With One G551D Mutation Taking VX770 Ivacaftor will restore CFTR function in treated CF patients with the G551D mutation. --- G551D ---
CPET in CF Patients With One G551D Mutation Taking VX770 Ivacaftor will restore CFTR function in treated CF patients with the G551D mutation. --- G551D --- --- G551D ---
- All participants will have at least one copy of the G551D mutation. --- G551D ---