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A Study of VX-659 Combination Therapy in CF Subjects Homozygous for F508del (F/F)

Phase 3
Completed
Conditions
Cystic Fibrosis
Interventions
Drug: TEZ/IVA
Drug: IVA
Drug: Placebo
Registration Number
NCT03460990
Lead Sponsor
Vertex Pharmaceuticals Incorporated
Brief Summary

This study will evaluate the efficacy of VX-659 in triple combination (TC) with tezacaftor (TEZ) and ivacaftor (IVA) in subjects with cystic fibrosis (CF) who are homozygous for the F508del mutation (F/F).

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
116
Inclusion Criteria
  • Homozygous for the F508del mutation (F/F)
  • Forced expiratory volume in 1 second (FEV1) value ≥40% and ≤90% of predicted mean for age, sex, and height

Key

Exclusion Criteria
  • Clinically significant cirrhosis with or without portal hypertension
  • Lung infection with organisms associated with a more rapid decline in pulmonary status
  • Solid organ or hematological transplantation

Other protocol defined Inclusion/Exclusion criteria may apply

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
VX-659/TEZ/IVA TCVX-659/TEZ/IVAFollowing a run-in period of 4 weeks with TEZ/IVA, participants received VX-659 240 mg/TEZ 100 mg/IVA 150 mg as FDC tablets in the morning and IVA 150 mg as mono tablet in the evening for 4 weeks in the TC treatment period.
TEZ/IVAPlaceboFollowing a run-in period of 4 weeks with Tezacaftor (TEZ)/Ivacaftor (IVA), participants received TEZ 100 milligram (mg)/IVA 150 mg as fixed-dose combination (FDC) tablet in the morning and IVA 150 mg as mono tablet in the evening for 4 weeks in the triple combination (TC) treatment period.
VX-659/TEZ/IVA TCIVAFollowing a run-in period of 4 weeks with TEZ/IVA, participants received VX-659 240 mg/TEZ 100 mg/IVA 150 mg as FDC tablets in the morning and IVA 150 mg as mono tablet in the evening for 4 weeks in the TC treatment period.
VX-659/TEZ/IVA TCPlaceboFollowing a run-in period of 4 weeks with TEZ/IVA, participants received VX-659 240 mg/TEZ 100 mg/IVA 150 mg as FDC tablets in the morning and IVA 150 mg as mono tablet in the evening for 4 weeks in the TC treatment period.
TEZ/IVATEZ/IVAFollowing a run-in period of 4 weeks with Tezacaftor (TEZ)/Ivacaftor (IVA), participants received TEZ 100 milligram (mg)/IVA 150 mg as fixed-dose combination (FDC) tablet in the morning and IVA 150 mg as mono tablet in the evening for 4 weeks in the triple combination (TC) treatment period.
TEZ/IVAIVAFollowing a run-in period of 4 weeks with Tezacaftor (TEZ)/Ivacaftor (IVA), participants received TEZ 100 milligram (mg)/IVA 150 mg as fixed-dose combination (FDC) tablet in the morning and IVA 150 mg as mono tablet in the evening for 4 weeks in the triple combination (TC) treatment period.
Primary Outcome Measures
NameTimeMethod
Absolute Change in Percent Predicted Forced Expiratory Volume in 1 Second (ppFEV1)From Baseline at Week 4

FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration.

Secondary Outcome Measures
NameTimeMethod
Absolute Change in Cystic Fibrosis Questionnaire-Revised (CFQ-R) Respiratory Domain ScoreFrom Baseline at Week 4

The CFQ-R is a validated participant-reported outcome measuring health-related quality of life for participants with cystic fibrosis. Respiratory domain assessed respiratory symptoms, score range: 0-100; higher scores indicating fewer symptoms and better health-related quality of life.

Safety and Tolerability as Assessed Number of Participants With Treatment-Emergent Adverse Events (AEs) and Serious Adverse Events (SAEs)From first dose of study drug in TC treatment period up to 28 days after last dose of study drug or to the completion of study participation date, whichever occurs first (up to Week 8)
Observed Pre-Dose Concentration (Ctrough) of VX-659, TEZ, TEZ Metabolite (M1-TEZ), and IVAFrom Day 1 and Week 4
Absolute Change in Sweat Chloride (SwCl)From Baseline at Week 4

Sweat samples were collected using an approved collection device.

Trial Locations

Locations (46)

Yale New Haven Medical Center

🇺🇸

New Haven, Connecticut, United States

St. Luke's CF Center of Idaho

🇺🇸

Boise, Idaho, United States

Helen DeVos Children's Hospital CF Center

🇺🇸

Grand Rapids, Michigan, United States

Rutgers-Robert Wood Johnson Medical School

🇺🇸

New Brunswick, New Jersey, United States

Sanford Research/ USD

🇺🇸

Sioux Falls, South Dakota, United States

Children's Foundation Research Center/ Le Bonheur Children's Hospital

🇺🇸

Memphis, Tennessee, United States

University of Tennessee Medical Center-Adult Cystic Fibrosis Clinic

🇺🇸

Knoxville, Tennessee, United States

Charite Paediatric Pulmonology Department

🇩🇪

Berlin, Germany

Cork University Hospital

🇮🇪

Dublin, Ireland

The Johns Hopkins Hospital

🇺🇸

Baltimore, Maryland, United States

Boston Children's Hospital

🇺🇸

Boston, Massachusetts, United States

University of Miami/Miller School of Medicine

🇺🇸

Miami, Florida, United States

Baylor College of Medicine

🇺🇸

Houston, Texas, United States

Advocate Children's Hospital- Park Ridge/ North Suburban Pulmonary and Critical Care Consultants

🇺🇸

Niles, Illinois, United States

Children's Mercy Hospital

🇺🇸

Kansas City, Missouri, United States

Washington University School of Medicine / St. Louis Children's Hospital

🇺🇸

Saint Louis, Missouri, United States

Albany Medical College

🇺🇸

Albany, New York, United States

Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center

🇺🇸

Pittsburgh, Pennsylvania, United States

Seattle Children's Hospital

🇺🇸

Seattle, Washington, United States

Indiana Clinical Research Center, IU Health University Hospital

🇺🇸

Indianapolis, Indiana, United States

The University of Iowa Hospitals and Clinics

🇺🇸

Iowa City, Iowa, United States

Royal Adelaide Hospital

🇦🇺

Adelaide, Australia

Prince Charles Hospital

🇦🇺

Chermside, Australia

Pneumologische Praxis Pasing

🇩🇪

Muenchen, Germany

Our Lady's Children's Hospital

🇮🇪

Dublin, Ireland

Institute for Respiratory Health Inc./ Sir Charles Gairdner Hospital

🇦🇺

Nedlands, Australia

John Hunter Hospital & Hunter Medical Research Institute

🇦🇺

New Lambton Heights, Australia

Hospital Universitari Vall d'Hebron Servicio de Broncoscopia

🇪🇸

Barcelona, Spain

Hospital Universitari Vall d Hebron

🇪🇸

Barcelona, Spain

Papworth Hospital NHS Foundation Trust, Papworth Everard

🇬🇧

Cambridge, United Kingdom

Hospital Universitario Virgen del Rocio

🇪🇸

Sevilla, Spain

Wythenshawe Hospital

🇬🇧

Manchester, United Kingdom

Royal Brompton & Harefield NHS Foundation Trust, Royal Brompton Hospital

🇬🇧

London, United Kingdom

Liverpool Heart and Chest Hospital

🇬🇧

Liverpool, United Kingdom

University Hospital Llandough

🇬🇧

Penarth, United Kingdom

University of Utah/ Primary Children's Medical Center

🇺🇸

Salt Lake City, Utah, United States

University of Alabama at Birmingham

🇺🇸

Birmingham, Alabama, United States

Respiratory Diseases of Children and Adolescents

🇺🇸

Oklahoma City, Oklahoma, United States

Oregon Health & Science University

🇺🇸

Portland, Oregon, United States

Cincinnati Children's Hospital Medical Center

🇺🇸

Cincinnati, Ohio, United States

Columbia University Medical Center

🇺🇸

New York, New York, United States

Northwell Health, Long Island Jewish Medical Center

🇺🇸

New Hyde Park, New York, United States

SUNY Upstate Medical University

🇺🇸

Syracuse, New York, United States

Vanderbilt University Medical Center

🇺🇸

Nashville, Tennessee, United States

The Alfred Hospital

🇦🇺

Melbourne, Victoria, Australia

St. Vincent's University Hospital

🇮🇪

Dublin, Ireland

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