A Study to Evaluate the Safety and Efficacy of Long Term Treatment With VX-661 in Combination With Ivacaftor in Participants With Cystic Fibrosis Who Have an F508del-CFTR Mutation
Condition: Cystic Fibrosis
MeSH terms: Cystic Fibrosis; Fibrosis
Intervention: TEZ/IVA; IVA
Intervention description: Fixed dose tablet for oral administration.; Tablet for oral administration.
Intervention MeSH terms: Ivacaftor
Phase: Phase 3
Study type: Interventional
Primary purpose: Treatment
Intervention model: Single Group Assignment
Masking: None (Open Label)
Enrollment: 1131.0
Sex: All
Minimum age: 12 Years
Healthy volunteers: No

Summary:

      This is a Phase 3, multicenter, open-label, 3-part rollover study in subjects with CF who are
      homozygous or heterozygous for the F508del-CFTR mutation and who participated in studies
      VX13-661-103 (Study 103, NCT02070744), VX14-661-106 (Study 106, NCT02347657), VX14-661-107
      (Study 107, NCT02516410), VX14-661-108 (Study 108, NCT02392234), VX14-661-109 (Study 109,
      NCT02412111), VX14-661-111 (Study 111, NCT02508207), VX15-661-112 (NCT02730208), and
      VX16-661-114 (NCT03150719). The study is designed to evaluate the safety and efficacy of
      long-term treatment of VX-661 in combination with ivacaftor.
    

Eligibility criteria:

        Inclusion Criteria:

        Part A:

          -  Participants entering the Treatment Cohort must meet all of the following criteria:

          -  Elect to enroll in the Treatment Cohort

          -  Completed study drug Treatment Period in a parent study (NCT02070744, NCT02347657,
             NCT02516410, NCT02392234, NCT02412111) or study drug treatment and the Safety Follow
             up Visit for participants from NCT02508207.

          -  Willing to remain on a stable CF regimen through the Safety Follow-up Visit.

          -  Participants re-enrolling in the Part A Treatment Cohort must meet all of the
             following criteria:

               -  Previously received at least 4 weeks of study drug before discontinuing in Part A
                  of Study NCT02565914 to participate in another qualified Vertex study.

               -  Completed the last required visit of another qualified Vertex study before or
                  during the Returning Visit in Part A Study NCT02565914.

          -  Participants entering the Part A Observational Cohort must meet the following
             criteria:

               -  <18 years of age (age on the date of informed consent/assent in the parent study)

               -  Completed study drug Treatment Period in a parent study or study drug treatment
                  and the Safety Follow up Visit for subjects from NCT02508207, but do not elect to
                  enroll in the NCT02565914 Treatment Cohort; or

               -  Received at least 4 weeks of study drug treatment and completed visits up to the
                  last scheduled visit of the Treatment Period of a parent study (and the Safety
                  Follow up Visit for participants from NCT02508207), but do not meet eligibility
                  criteria for enrollment into the Treatment Cohort

        Part B:

        Participants who meet all of the following inclusion criteria will be eligible for Part B.

          -  Did not withdraw consent from the parent study or Part A of Study NCT02565914.

          -  Completed study drug treatment during the Treatment Period in Part A of - Willing to
             remain on a stable CF medication (and supplement) regimen through the 96 week visit of
             Study NCT02565914.

        Participants re enrolling in Part B must meet all of the following criteria:

          -  Previously received at least 4 weeks of study drug before discontinuing Study
             NCT02565914 to participate in another qualified Vertex study, which is defined as a
             Vertex study of investigational CFTR modulators that allows participation of
             participants in Study NCT02565914.

          -  Completed the last required visit of another qualified Vertex study before or during
             the Returning Visit in Part B.

          -  Willing to remain on a stable CF medication (and supplement) regimen through the 96
             week visit in Part B.

        Part C:

          -  Participants who meet all of the following inclusion criteria will be eligible for
             Part C.

          -  Did not withdraw consent from Part B of Study NCT02565914.

          -  Completed study drug treatment during Part B of NCT02565914.

          -  Willing to remain on a stable CF medication (and supplement) regimen through the 96
             week visit of Part C.

        Exclusion Criteria:

          -  History of any comorbidity that, in the opinion of the investigator, might confound
             the results of the study or pose an additional risk to the subject.

          -  Pregnant and nursing females.

          -  Sexually active subjects of reproductive potential who are not willing to follow the
             contraception requirements.

          -  History of drug intolerance in the parent study that would pose an additional risk to
             the subject.

          -  Participation in an investigational drug trial (including studies investigating
             VX-661/ivacaftor or lumacaftor/ivacaftor) other than the parent studies of NCT02565914
             or other eligible Vertex studies investigating VX-661 in combination with ivacaftor,
             or use of a commercially available CFTR modulator.

        Other protocol defined Inclusion/Exclusion criteria may apply.