Study to Evaluate Lumacaftor and Ivacaftor Combination Therapy in Subjects 12 Years and Older With Advanced Lung Disease

A Phase 3b, Open-Label Study to Evaluate Lumacaftor and Ivacaftor Combination Therapy in Subjects 12 Years and Older With Cystic Fibrosis and Advanced Lung Disease, Homozygous for the F508del-CFTR Mutation

The purpose of this study is to evaluate the safety and tolerability of LUM/IVA combination therapy in subjects 12 years and older with CF and advanced lung disease and who are homozygous for the F508del CFTR mutation

Inclusion Criteria:

• Homozygous for the F508del-CFTR mutation; historical genotype must be documented in the participant's source documents.
• Percent predicted FEV1 <40 of adjusted for age, sex, and height at Screening

Exclusion Criteria:

• Participant currently receiving invasive mechanical ventilation.
• History of any comorbidity 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 participant
• Any clinically significant laboratory abnormalities at screening that would interfere with the study assessments or pose an undue risk for the subject
• A 12-lead electrocardiograms (ECG) demonstrating QTcF >450 msec at Screening
• History of solid organ or hematological transplantation
• History of alcohol or drug abuse in the past year
• Ongoing or prior participation in an investigational drug study (including studies investigating lumacaftor and/or ivacaftor) within 30 days of screening.
• Use of strong inhibitors, moderate inducers, or strong inducers of CYP3A
• Pregnant and nursing females: Females of childbearing potential must have a negative pregnancy test at Screening and Day 1.
• Sexually active subjects of reproductive potential who are not willing to follow the contraception requirements
• Use of beta blockers or the equivalent at Screening.