About the Study

Ivacaftor is designed to keep CFTR proteins at the cell surface open longer to improve the flow of salt and water across the cell membrane, which helps hydrate and clear mucus from the airways.

Inclusion Criteria

  • Confirmed diagnosis of CF by sweat chloride value or CF mutation criteria. Must have 1 of the following 9 CFTR mutations on at least 1 allele: G551D, G178R, S549N, S549R, G551S, G1244E, S1251N, S1255P, or G1349D. Hematology, serum chemistry, and vital signs results at screening with no clinically significant abnormalities that would interfere with the study assessments, as judged by the investigator.

Exclusion Criteria

  • History of any illness or condition that, in the opinion of the investigator, might confound the results of the study or pose an additional risk in administering study drug to the subject Colonization with organisms associated with a more rapid decline in pulmonary status at screening History of abnormal liver function or abnormal liver function at screening History of solid organ or hematological transplantation Use of any moderate or strong inducers or inhibitors of cytochrome P450 (CYP) 3A within 2 weeks before Day 1 Participation in a clinical study involving administration of either an investigational or a marketed drug within 30 days or 5 terminal half-lives before screening Hemoglobin (Hgb) <9.5 g/dL at screening Chronic kidney disease of Stage 3 or above Presence of a non-congenital or progressive lens opacity or cataract at Screening Other protocol defined Inclusion/Exclusion Criteria may apply.

Study Team

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