A Study to Evaluate the Safety and Efficacy of Long-term Treatment With TEZ/IVA in CF Participants With an F508del CFTR Mutation

NCT ID: NCT03537651

Last Updated: 2024-04-19

Study Results

Results available

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Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE3

Total Enrollment

130 participants

Study Classification

INTERVENTIONAL

Study Start Date

2018-04-25

Study Completion Date

2023-09-29

Brief Summary

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This study evaluates the long-term safety and tolerability of tezacaftor in combination with ivacaftor (TEZ/IVA) in participants with cystic fibrosis (CF) aged 6 years and older, homozygous or heterozygous for the F508del mutation.

Detailed Description

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Conditions

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Cystic Fibrosis

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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TEZ/IVA

Part A: Participants weighing less than (\<)40 kilograms (kg) at Day 1 received tezacaftor (TEZ) 50 milligrams (mg) once daily (qd)/ivacaftor (IVA) 75 mg every 12 hours (q12h) and the participants weighing greater than or equals to (\>=) 40 kg at Day 1 received TEZ 100 mg qd/IVA 150 mg q12h in the treatment period for 96 weeks. Doses were adjusted upward for changes in body weight and/or age.

Part B: Participants weighing \<30 kg at Day 1 received TEZ 50 mg qd/IVA 75 mg q12h and the participants weighing \>=30 kg at Day 1 received TEZ 100 mg qd/IVA 150 mg q12h in the treatment period up to 192 weeks. Doses were adjusted upward for changes in body weight and/or age.

Group Type EXPERIMENTAL

TEZ/IVA

Intervention Type DRUG

Fixed-dose combination tablet for oral administration.

IVA

Intervention Type DRUG

Tablet for oral administration.

Interventions

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TEZ/IVA

Fixed-dose combination tablet for oral administration.

Intervention Type DRUG

IVA

Tablet for oral administration.

Intervention Type DRUG

Other Intervention Names

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VX-661/VX-770 tezacaftor/ivacaftor VX-770 ivacaftor

Eligibility Criteria

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Inclusion Criteria

* Completed the Week 24 Visit in Study 113 Part B or the Week 8 Visit in Study 115
* Eligible CFTR Mutation

Exclusion Criteria

* Pregnant and nursing females
* History of poor compliance with study drug and/or procedures in a previous study as deemed by the investigator
* Ongoing participation in another study with investigational drug
Minimum Eligible Age

6 Years

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

Providence Alaska Medical Center

Anchorage, Alaska, United States

Site Status

Arkansas Children's Hospital

Little Rock, Arkansas, United States

Site Status

Children's Hospital Los Angeles

Los Angeles, California, United States

Site Status

Children's Hospital Colorado

Aurora, Colorado, United States

Site Status

Nemours/ Alfred I. duPont Hospital for Children

Wilmington, Delaware, United States

Site Status

Johns Hopkins All Children's Hospital Outpatient Care Center

St. Petersburg, Florida, United States

Site Status

Center for Advanced Pediatrics

Atlanta, Georgia, United States

Site Status

St. Luke's CF Center of Idaho

Boise, Idaho, United States

Site Status

Riley Hospital for Children Indiana University Health

Indianapolis, Indiana, United States

Site Status

Boston Children's Hospital

Boston, Massachusetts, United States

Site Status

Children's Hospital & Clinics of Minnesota

Minneapolis, Minnesota, United States

Site Status

The Children's Mercy Hospital

Kansas City, Missouri, United States

Site Status

Dartmouth Hitchcock Medical Center

Manchester, New Hampshire, United States

Site Status

UBMD Pediatrics/ CF Center of Western New York

Buffalo, New York, United States

Site Status

Columbia University Medical Center

New York, New York, United States

Site Status

SUNY Upstate Medical University

Syracuse, New York, United States

Site Status

Wake Forest Baptist Health

Winston-Salem, North Carolina, United States

Site Status

Rainbow Babies and Children's Hospital/University Hospitals Cleveland Medical Center

Cleveland, Ohio, United States

Site Status

Children's Hospital of Pittsburgh of UPMC

Pittsburgh, Pennsylvania, United States

Site Status

Medical University of South Carolina (MUSC)

Charleston, South Carolina, United States

Site Status

Sanford Children's Speciality Clinic

Sioux Falls, South Dakota, United States

Site Status

Austin Children's Chest Associates

Austin, Texas, United States

Site Status

Cook Children's Medical Center

Fort Worth, Texas, United States

Site Status

Baylor College of Medicine

Houston, Texas, United States

Site Status

Children's Hospital of The King's Daughters

Norfolk, Virginia, United States

Site Status

Seattle Children's Hospital

Seattle, Washington, United States

Site Status

Children's Hospital of Wisconsin

Milwaukee, Wisconsin, United States

Site Status

Perth Children's Hospital

Nedlands, , Australia

Site Status

John Hunter Hospital & Hunter Medical Research Institute and John Hunter Children's Hospital

New Lambton, , Australia

Site Status

Lady Cilento Children's Hospital

South Brisbane, , Australia

Site Status

The Children's Hospital at Westmead

Westmead, , Australia

Site Status

Universitair Ziekenhuis Brussel - Campus Jette

Brussels, , Belgium

Site Status

Universitaire Ziekenhuizen Leuven - Campus Gasthuisberg

Leuven, , Belgium

Site Status

British Columbia's Children's Hospital

Vancouver, British Columbia, Canada

Site Status

The Hospital for Sick Children

Toronto, Ontario, Canada

Site Status

McGill University Health Centre, Glen Site, Montreal Children's Hospital

Montreal, Quebec, Canada

Site Status

Juliane Marie Center, Rigshopitalet

Copenhagen, , Denmark

Site Status

Groupe Hospitaler Pellegrin, CHU De Bordeaux

Bordeaux, , France

Site Status

Hopital Necker, Enfants Malades

Paris, , France

Site Status

Universitaetsklinkum Koeln, CF-Studienzentrum

Cologne, , Germany

Site Status

Universitätsklinikum Essen

Essen, , Germany

Site Status

Clinic of J.W. Goethe University

Frankfurt, , Germany

Site Status

Justus-Leibig-Universitat Zentrum fur Kinderheilkunde und Jugendmedizin

Giessen, , Germany

Site Status

Medizinische Hochschule Hannover

Hanover, , Germany

Site Status

Universitaetsklinikum Jena, Mukoviszidose-Zentrum

Jena, , Germany

Site Status

Universitaetsklinikum Tuebingen Klinik fuer Kinder- und Jugendmedizin

Tübingen, , Germany

Site Status

Our Lady's Children's Hospital

Dublin, , Ireland

Site Status

University Hospital Limerick

Limerick, , Ireland

Site Status

Klinika Mukowiscydozy IMD Oddozial Chorob Pluc Szpzoz IM. Dzieci WarszaWY

Łomianki, , Poland

Site Status

Inselspital - Universitaetsspital Bern

Bern, , Switzerland

Site Status

Kinderspital Zuerich

Zurich, , Switzerland

Site Status

Leeds General Infirmary

Leeds, , United Kingdom

Site Status

Royal Brompton & Harefield NHS Foundation Trust, Royal Brompton Hospital

London, , United Kingdom

Site Status

Southampton General Hospital

Southampton, , United Kingdom

Site Status

Countries

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United States Australia Belgium Canada Denmark France Germany Ireland Poland Switzerland United Kingdom

References

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Heneghan M, Southern KW, Murphy J, Sinha IP, Nevitt SJ. Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del). Cochrane Database Syst Rev. 2023 Nov 20;11(11):CD010966. doi: 10.1002/14651858.CD010966.pub4.

Reference Type DERIVED
PMID: 37983082 (View on PubMed)

Sawicki GS, Chilvers M, McNamara J, Naehrlich L, Saunders C, Sermet-Gaudelus I, Wainwright CE, Ahluwalia N, Campbell D, Harris RS, Paz-Diaz H, Shih JL, Davies JC. A Phase 3, open-label, 96-week trial to study the safety, tolerability, and efficacy of tezacaftor/ivacaftor in children >/= 6 years of age homozygous for F508del or heterozygous for F508del and a residual function CFTR variant. J Cyst Fibros. 2022 Jul;21(4):675-683. doi: 10.1016/j.jcf.2022.02.003. Epub 2022 Feb 18.

Reference Type DERIVED
PMID: 35190292 (View on PubMed)

Southern KW, Murphy J, Sinha IP, Nevitt SJ. Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del). Cochrane Database Syst Rev. 2020 Dec 17;12(12):CD010966. doi: 10.1002/14651858.CD010966.pub3.

Reference Type DERIVED
PMID: 33331662 (View on PubMed)

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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2017-002968-40

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

VX17-661-116

Identifier Type: -

Identifier Source: org_study_id

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