Evaluation of Long-Term Safety and Efficacy of Vanzacaftor/Tezacaftor/Deutivacaftor in Cystic Fibrosis Subjects 1 Year of Age and Older

Prior treatment

• Current participation in an investigational drug study other than the parent study. Participation in a noninterventional study, and/or screening for another Vertex study is permitted.
• Cohort 1 only: Pregnant or breast-feeding females. Female subjects must have a negative pregnancy test at the Part A and Part B Day 1 Visit before receiving the first dose of study drug.

Other illness

• History of any illness or any clinical condition that, in the opinion of the investigator, might confound the results of the study or pose an additional risk in administering study drug(s) to the subject.
• New development of a comorbidity during the parent study or Part A that, in the opinion of the investigator, might pose an additional risk in administering study drug to the subject. This includes, but is not limited to, the following: • Hepatic cirrhosis with portal hypertension, moderate hepatic impairment, or severe hepatic impairment that, in the opinion of the investigator, might pose an additional risk in administering study drug to the subject. •Solid organ or hematological transplantation.
• History of drug intolerance in the parent study that would pose an additional risk to the subject in the opinion of the investigator.
• History of poor compliance with study drug and/or procedures in the parent study as deemed by the investigator.
• Cohort 2, Pancreatic Enzyme Replacement Therapy [PERT] sub study only: Meets none of the exclusion criteria for Part A, History of any illness or any clinical condition that, in the opinion of the investigator, might confound the results of the sub study or pose additional risk to the subject by withdrawing PERT

Other

• The subject or a close relative of the subject is the investigator or a subinvestigator, research assistant, pharmacist, study coordinator, or other staff directly involved with the conduct of the study at that site.

• Subject, or subject’s legally appointed and authorized representative, will sign and date an ICF. Subject will sign an assent form.
• As judged by the investigator, the legal representative or guardian must be able to understand protocol requirements, restrictions, and instructions and the legal representative or guardian should be able to ensure that the subject will comply with and is likely to complete the study as planned.
• Part A: Did not withdraw consent from the parent study.
• Part A: Meets at least 1 of the following criteria: • Completed study drug treatment in the parent study. • Had study drug interruption(s) in the parent study, but did not prematurely discontinue study drug, and completed study visits up to the last scheduled visit of the Treatment Period of the parent study.
• Willing to remain on a stable CF treatment regimen through completion of study participation.
• Part B (Cohort 1): Did not withdraw consent from Part A.
• Part B (Cohort 1): Meets at least 1 of the following criteria: • Completed study drug treatment in Part A. • Had study drug interruption(s) in Part A, but did not permanently discontinue study drug, and completed study visits up to the last scheduled visit of the Treatment Period of Part A.
• Part A (Cohort 2, Pancreatic Enzyme Replacement Therapy [PERT] sub study only): Meets all of the inclusion criteria for Part A, Did not withdraw consent from Part A, Currently receiving PERT