The purpose of this study is to determine whether the treatment with Ivacaftor remains effective and safe in the patients with cystic fibrosis (and at least one G551D CFTR mutation) in the real life setting, after the drug has been approved by the Health authorities.
Name: CF patients with a G551D mutation and treated with Ivacaftor
Description: pulmonary function
Measure: FEV1 (in liters and in % predicted) Time: until one years after initiation of treatmentDescription: number of oral antibiotic treatments and number of days of oral antibiotic treatments, number of IV courses and days of IV antibiotics per year
Measure: number of antibiotic treatments and number of days of antibiotic treatments Time: until one year after initiation of treatmentDescription: Evolution of bacteria and fungi in sputum
Measure: respiratory colonization Time: until least one year after initiation of treatmentDescription: Weight (and BMI-Zscore for children)
Measure: nutritional status Time: until one year after initiation of treatmentDescription: Dates and reasons for interruption and discontinuation of treatment with Ivacaftor Adverse events, indicating what in the physician's opinion might be due to Ivacaftor
Measure: Adverse events Time: until one year after initiation of treatmentCohort
There is one SNP
Ivacaftor in French Patients With Cystic Fibrosis and a G551D Mutation - Efficacy and Safety Results After the First Year of Treatment in the Real Life Setting.. Ivacaftor in French Patients With Cystic Fibrosis and a G551D Mutation The purpose of this study is to determine whether the treatment with Ivacaftor remains effective and safe in the patients with cystic fibrosis (and at least one G551D CFTR mutation) in the real life setting, after the drug has been approved by the Health authorities. --- G551D ---
Ivacaftor in French Patients With Cystic Fibrosis and a G551D Mutation - Efficacy and Safety Results After the First Year of Treatment in the Real Life Setting.. Ivacaftor in French Patients With Cystic Fibrosis and a G551D Mutation The purpose of this study is to determine whether the treatment with Ivacaftor remains effective and safe in the patients with cystic fibrosis (and at least one G551D CFTR mutation) in the real life setting, after the drug has been approved by the Health authorities. --- G551D --- --- G551D ---
Ivacaftor in French Patients With Cystic Fibrosis and a G551D Mutation - Efficacy and Safety Results After the First Year of Treatment in the Real Life Setting.. Ivacaftor in French Patients With Cystic Fibrosis and a G551D Mutation The purpose of this study is to determine whether the treatment with Ivacaftor remains effective and safe in the patients with cystic fibrosis (and at least one G551D CFTR mutation) in the real life setting, after the drug has been approved by the Health authorities. --- G551D --- --- G551D --- --- G551D ---
Inclusion Criteria: - French patients with CF aged 6 or older who are homozygous or heterozygous for the G551D mutation - Treated with Ivacaftor - First prescription of Ivacaftor before June 1st 2013 (including patients randomized in the VX770 clinical trials) Exclusion Criteria: - CF patients younger than 6 years old - CF patients who have received lung transplantation - CF patients without a G551D mutation. --- G551D ---
Inclusion Criteria: - French patients with CF aged 6 or older who are homozygous or heterozygous for the G551D mutation - Treated with Ivacaftor - First prescription of Ivacaftor before June 1st 2013 (including patients randomized in the VX770 clinical trials) Exclusion Criteria: - CF patients younger than 6 years old - CF patients who have received lung transplantation - CF patients without a G551D mutation. --- G551D --- --- G551D ---