Study of Ivacaftor in Subjects With Cystic Fibrosis (CF) Who Have the R117H-CF Transmembrane Conductance Regulator (CFTR) Mutation (KONDUCT)

Inclusion Criteria:

   - Male or female with confirmed diagnosis of CF

   - Must have at least 1 allele of the R117H CFTR mutation

   - Percent predicted forced expiratory volume in 1 second (FEV1) 40 percent (%) to 90%
   (for subjects aged 12 years or older) or 40% to 105% (for subjects aged 6 to 11 years)
   predicted normal for age, sex, and height

   - 6 years of age or older

   - Minimum weight of 15 kilogram (kg) at screening

   - Females of childbearing potential must not be pregnant

   - Willing to comply with contraception requirements

Exclusion Criteria:

   - CFTR gene mutation leading to CFTR channel with gating defect (that is, any 1 of the
   following mutations: G551D, G178R, G551S, S549N, S549R, G970R, G1244E, S1251N, S1255P,
   or G1349D)

   - History of any illness or condition that might confound the results of the study or
   pose an additional risk in administering ivacaftor to the subject

   - An acute upper or lower respiratory infection, pulmonary exacerbation, or changes in
   therapy (including antibiotics) for pulmonary disease within 4 weeks before the first
   dose of study drug

   - Abnormal liver function, at screening, defined as greater than or equal to (>=) 3 time
   upper limit of normal (ULN), of any 3 or more of the following: serum aspartate
   transaminase (AST), serum alanine transaminase (ALT), gamma-glutamyl transpeptidase
   (GGT), serum alkaline phosphatase (ALP), total bilirubin

   - Colonization with organisms associated with a more rapid decline in pulmonary status
   (for example, Burkholderia cenocepacia, Burkholderia dolosa, and Mycobacterium
   abscessus) at screening

   - History of solid organ or hematological transplantation

   - History of alcohol, medication or illicit drug abuse within 1 year before the first
   dose of study drug

   - Ongoing participation in another therapeutic clinical study or prior participation in
   an investigational drug study within 30 days before screening

   - Any "non-CF-related" illness within 2 weeks before Day 1 (first dose of study drug).
   "Illness" was defined as an acute (serious or non-serious) condition (for example,
   gastroenteritis)

   - Use of any inhibitors or inducers of cytochrome (CYP) P450 3A