Status:

COMPLETED

A Study to Evaluate the Safety of Long-term Ivacaftor Treatment in Participants With Cystic Fibrosis Who Are Less Than 24 Months of Age at Treatment Initiation and Have an Approved Ivacaftor-Responsive Mutation

Lead Sponsor:

Vertex Pharmaceuticals Incorporated

Conditions:

Cystic Fibrosis

Eligibility:

All Genders

Up to 24 years

Phase:

PHASE3

Brief Summary

This is a Phase 3, 2-arm, multicenter study with an open-label ivacaftor arm and an observational arm to evaluate the safety and efficacy of long-term ivacaftor treatment in participants with cystic f...

Eligibility Criteria

Inclusion

  • Ivacaftor Arm: Participants From Study 124 (NCT02725567 ) Part B:
  • Participants transitioning from Study 124 Part B must have completed the last study visit of Study 124 Part B.
  • As judged by the investigator, parent or legal guardian must be able to understand protocol requirements, restrictions, and instructions; and must sign the informed consent form (ICF).
  • Ivacaftor Arm: Participants Not From Study 124 Part B:
  • Confirmed diagnosis of CF, or 2 CF-causing mutations.
  • An ivacaftor- responsive CFTR mutation on at least 1 allele. Participants will be eligible in countries/regions where ivacaftor is approved for use in participants 2 years of age and older.
  • As judged by the investigator, parent or legal guardian must be able to understand protocol requirements, restrictions, and instructions; and must sign the ICF.
  • Observational Arm:
  • Received ivacaftor treatment in Study 124 Part B and elected not to enroll or ineligible to enroll in the ivacaftor arm of Study 126.

Exclusion

  • Ivacaftor Arm: Participants From Study 124 Part B:
  • 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 ivacaftor to the participant.
  • Participants receiving commercially available ivacaftor treatment
  • Ivacaftor Arm: Participants Not From Study 124 Part B:
  • 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 ivacaftor to the participant
  • An acute upper or lower respiratory infection, or pulmonary exacerbation, or changes in therapy for pulmonary disease within 4 weeks of Day 1
  • Abnormal liver function at screening
  • Hemoglobin \<9.5 g/dL at screening
  • History of solid organ or hematological transplantation
  • Use of any moderate or strong inducers or inhibitors of CYP3A within 2 weeks of Day 1
  • Observational Arm:
  • Receiving ivacaftor treatment
  • Other protocol defined Inclusion/Exclusion criteria may apply.

Key Trial Info

Start Date :

August 16 2017

Trial Type :

INTERVENTIONAL

Allocation :

ACTUAL

End Date :

October 2 2023

Estimated Enrollment :

86 Patients enrolled

Trial Details

Trial ID

NCT03277196

Start Date

August 16 2017

End Date

October 2 2023

Last Update

October 23 2024

Active Locations (29)

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Page 1 of 8 (29 locations)

1

University of Alabama at Birmingham

Birmingham, Alabama, United States, 35233

2

Stanford University

Palo Alto, California, United States, 94034

3

Alfred I DuPont Hospital for Children

Wilmington, Delaware, United States, 19803

4

Nemours Children's Hospital

Orlando, Florida, United States, 32827