The purpose of the study is to examine the real-life safety and effectiveness of the novel combination ivacaftor+lumacaftor in eligible patients with cystic fibrosis (CF). All patients with CF were eligible if they were 12 years and older, started ivacaftor+lumacaftor outside of a clinical trial between December 15th 2017 and December 15th 2018 in an accredited CF center in France. Patient followed-up is based on standardized recommendation of the French Cystic Fibrosis Society. Each patient is followed 1 year.

Each patient is followed one year with visits at months 1, 3, 6 and 12.

At each visit, the following data are recorded:

• Treatment discontinuation or not. If the treatment was discontinued, reasons for discontinuation
• Adverse effects
• Lung function (spirometry)
• Body mass index
• Pulmonary exacerbations (intravenous antibiotics)
• Sputum microbiology
• Liver enzymes are measured at each visit

At the initial and 12 visits, a yearly CF examination is proposed to the patients:

• Blood tests
• Chest CT scans
• Body plethysmography

Inclusion Criteria:

• Patient aged 12 years or older.
• Patient with Cystic Fibrosis with presence of two mutations DF508 in the CFTR gene
• Patient treated with ivacaftor+lumacaftor (Orkambi)

Exclusion Criteria:

• Refusal to participate in the study
• Start of Orkambi as part of a clinical trial

Patient with Cystic Fibrosis with presence of two mutations DF508 in the CFTR gene and treated by Orkambi in usual care