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

NCT ID: NCT03277196

Last Updated: 2024-10-23

Study Results

Results available

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Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE3

Total Enrollment

86 participants

Study Classification

INTERVENTIONAL

Study Start Date

2017-08-16

Study Completion Date

2023-10-02

Brief Summary

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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 fibrosis (CF) who are \<24 months of age at treatment initiation and have an approved Ivacaftor-Responsive mutation.

Detailed Description

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Conditions

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Cystic Fibrosis

Study Design

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Allocation Method

NON_RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Ivacaftor Arm

Participants less than (\<) 24 months of age and weighing 5 to less than (\<) 7 kilogram (kg) received 25 mg IVA every 12 hours (q12h), 7 to \<14 kg received 50 mg IVA q12h, and those weighing 14 to \<25 kg received 75 mg IVA q12h.

Participants more than or equal (\>=) 24 months of age and weighing \<14 kg received 50 mg IVA q12h, and those weighing more than or equal to (\>=)14 kg received 75 mg IVA q12h in the treatment period for up to 96 weeks.

Doses were determined based on safety and pharmacokinetic (PK) data from parent study, age and weight.

Group Type EXPERIMENTAL

IVA

Intervention Type DRUG

Granules for oral administration.

Observational Arm

Group Type NO_INTERVENTION

No interventions assigned to this group

Interventions

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IVA

Granules for oral administration.

Intervention Type DRUG

Other Intervention Names

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VX-770 ivacaftor

Eligibility Criteria

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Inclusion Criteria

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 Criteria

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
Minimum Eligible Age

0 Months

Maximum Eligible Age

24 Months

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Vertex Pharmaceuticals Incorporated

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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University of Alabama at Birmingham

Birmingham, Alabama, United States

Site Status

Stanford University

Palo Alto, California, United States

Site Status

Alfred I DuPont Hospital for Children

Wilmington, Delaware, United States

Site Status

Nemours Children's Hospital

Orlando, Florida, United States

Site Status

Center for Advanced Pediatrics

Atlanta, Georgia, United States

Site Status

Northwestern University

Chicago, Illinois, United States

Site Status

Riley Hospital for Children at Indiana University Health

Indianapolis, Indiana, United States

Site Status

John Hopkins Hospital

Baltimore, Maryland, United States

Site Status

Boston Children's Hospital

Boston, Massachusetts, United States

Site Status

Childrens's Hospitals and Clinics of Minnnesota

Minneapolis, Minnesota, United States

Site Status

Children's Mercy Hospital

Kansas City, Missouri, United States

Site Status

Billings Clinic Hospital

Billings, Montana, United States

Site Status

Nationwide Children's Hospital

Columbus, Ohio, United States

Site Status

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status

Texas Children's Hospital

Houston, Texas, United States

Site Status

Seattle Children's Hospital

Seattle, Washington, United States

Site Status

University of Wisconsin

Madison, Wisconsin, United States

Site Status

Queensland Children's Hospital

South Brisbane, , Australia

Site Status

The Hospital for Sick Children

Toronto, Ontario, Canada

Site Status

Universitaetsklinikum Heidelberg, Zenter fuer Kinder-und Jugendmedizin

Heidelberg, , Germany

Site Status

Children's Health Ireland at Crumlin

Dublin, , Ireland

Site Status

Children's University Hospital Temple Street

Dublin, , Ireland

Site Status

University Hospital Limerick

Limerick, , Ireland

Site Status

Paediatric Clinical Research Facility

Edinburgh, , United Kingdom

Site Status

Alder Hey Children's Alder Hey Children's NHS Foundation Trust

Liverpool, , United Kingdom

Site Status

Great Ormond Street Hospital for Sick Children

London, , United Kingdom

Site Status

Royal Brompton & Harefield NHS Founcation Trust, Royal BromptonHospital

London, , United Kingdom

Site Status

Royal Manchester Children's Hospital

Manchester, , United Kingdom

Site Status

Oxford University Hospitals NHS Trust, John Radcliffe Hospital

Oxford, , United Kingdom

Site Status

Countries

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United States Australia Canada Germany Ireland United Kingdom

Provided Documents

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Document Type: Study Protocol

View Document

Document Type: Statistical Analysis Plan

View Document

Other Identifiers

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2017-001379-21

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

VX15-770-126

Identifier Type: -

Identifier Source: org_study_id

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