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A Study Evaluating the Safety and Efficacy of VX-659 Combination Therapy in Subjects With Cystic Fibrosis

Phase 2
Completed
Conditions
Cystic Fibrosis
Interventions
Drug: Placebo (matched to VX-659/TEZ/IVA)
Drug: Placebo (matched to VX-659/TEZ/VX-561)
Drug: TEZ/IVA
Drug: TEZ
Drug: IVA
Registration Number
NCT03224351
Lead Sponsor
Vertex Pharmaceuticals Incorporated
Brief Summary

This is a Phase 2, randomized, double-blind, placebo- and tezacaftor/ivacaftor (TEZ/IVA)-controlled, parallel-group, 3-part, multicenter study designed to evaluate the safety and efficacy of VX-659 in triple combination (TC) with TEZ and IVA in subjects with cystic fibrosis (CF) who are homozygous for the F508del mutation of the CF transmembrane conductance regulator (CFTR) gene (F/F genotype), or who are heterozygous for the F508del mutation and a minimal function (MF) CFTR mutation not likely to respond to TEZ, IVA, or TEZ/IVA (F/MF genotypes).

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
124
Inclusion Criteria
  • Body weight ≥35 kg.

  • Subjects must have an eligibleCFTR genotype.

    • Part 1 and Part 3: Heterozygous for F508del and an MF mutation (F/MF)
    • Part 2: Homozygous for F508del (F/F)
  • FEV1 value ≥40% and ≤90% of predicted mean for age, sex, and height

Key

Exclusion Criteria
  • History of clinically significant cirrhosis with or without portal hypertension.
  • Glucose-6-phosphate dehydrogenase (G6PD) deficiency
  • Lung infection with organisms associated with a more rapid decline in pulmonary status.
  • History of solid organ or hematological transplantation.

Other protocol defined Inclusion/Exclusion criteria may apply.

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Part 3: VX-659/TEZ/VX-561 TCVX-659Participants received VX-659 400 mg qd/TEZ 100 mg qd/VX-561 200 mg qd in TC treatment period for 4 weeks.
Part 1: VX-659/TEZ/IVA TC - High DoseVX-659Participants received VX-659 400 mg qd/TEZ 100 mg qd/IVA 150 mg q12h in TC treatment period for 4 weeks and TEZ 100 mg qd/IVA 150 mg q12h in washout period for 4 days.
Part 3: VX-659/TEZ/VX-561 TCVX-561Participants received VX-659 400 mg qd/TEZ 100 mg qd/VX-561 200 mg qd in TC treatment period for 4 weeks.
Part 1: VX-659/TEZ/IVA TC - Low DoseIVAParticipants received VX-659 80 milligram (mg) once daily (qd)/TEZ 100 mg qd/IVA 150 mg every 12 hours (q12h) in TC treatment period for 4 weeks and TEZ 100 mg qd/IVA 150 mg q12h in washout period for 4 days.
Part 1: VX-659/TEZ/IVA TC - Low DoseVX-659Participants received VX-659 80 milligram (mg) once daily (qd)/TEZ 100 mg qd/IVA 150 mg every 12 hours (q12h) in TC treatment period for 4 weeks and TEZ 100 mg qd/IVA 150 mg q12h in washout period for 4 days.
Part 1: VX-659/TEZ/IVA TC - High DoseTEZ/IVAParticipants received VX-659 400 mg qd/TEZ 100 mg qd/IVA 150 mg q12h in TC treatment period for 4 weeks and TEZ 100 mg qd/IVA 150 mg q12h in washout period for 4 days.
Part 1: VX-659/TEZ/IVA TC - High DoseIVAParticipants received VX-659 400 mg qd/TEZ 100 mg qd/IVA 150 mg q12h in TC treatment period for 4 weeks and TEZ 100 mg qd/IVA 150 mg q12h in washout period for 4 days.
Part 2: TEZ/IVAIVAFollowing run-in period with TEZ 100 mg qd/IVA 150 mg q12h for 4 weeks, participants received TEZ 100 mg qd/IVA 150 mg q12h in TC treatment period for 4 weeks and TEZ 100 mg qd/IVA 150 mg q12h in washout period for 4 weeks.
Part 2: VX-659/TEZ/IVA TCTEZ/IVAFollowing run-in period with TEZ 100 mg qd/IVA 150 mg q12h for 4 weeks, participants received VX-659 400 mg qd/TEZ 100 mg qd/IVA 150 mg q12h in TC treatment period for 4 weeks and TEZ 100 mg qd/IVA 150 mg q12h in washout period for 4 weeks.
Part 1: PlaceboPlacebo (matched to VX-659/TEZ/IVA)Participants received placebo matched to VX-659/TEZ/IVA in TC treatment period for 4 weeks and placebo matched TEZ/IVA in washout period for 4 days.
Part 1: VX-659/TEZ/IVA TC - Low DoseTEZ/IVAParticipants received VX-659 80 milligram (mg) once daily (qd)/TEZ 100 mg qd/IVA 150 mg every 12 hours (q12h) in TC treatment period for 4 weeks and TEZ 100 mg qd/IVA 150 mg q12h in washout period for 4 days.
Part 1: VX-659/TEZ/IVA TC - Medium DoseTEZ/IVAParticipants received VX-659 240 mg qd/TEZ 100 mg qd/IVA 150 mg q12h in TC treatment period for 4 weeks and TEZ 100 mg qd/IVA 150 mg q12h in washout period for 4 days.
Part 1: VX-659/TEZ/IVA TC - Medium DoseIVAParticipants received VX-659 240 mg qd/TEZ 100 mg qd/IVA 150 mg q12h in TC treatment period for 4 weeks and TEZ 100 mg qd/IVA 150 mg q12h in washout period for 4 days.
Part 2: TEZ/IVATEZ/IVAFollowing run-in period with TEZ 100 mg qd/IVA 150 mg q12h for 4 weeks, participants received TEZ 100 mg qd/IVA 150 mg q12h in TC treatment period for 4 weeks and TEZ 100 mg qd/IVA 150 mg q12h in washout period for 4 weeks.
Part 3: PlaceboPlacebo (matched to VX-659/TEZ/VX-561)Participants received placebo matched to VX-659/TEZ/VX-561 in TC treatment period for 4 weeks.
Part 1: VX-659/TEZ/IVA TC - Medium DoseVX-659Participants received VX-659 240 mg qd/TEZ 100 mg qd/IVA 150 mg q12h in TC treatment period for 4 weeks and TEZ 100 mg qd/IVA 150 mg q12h in washout period for 4 days.
Part 2: VX-659/TEZ/IVA TCIVAFollowing run-in period with TEZ 100 mg qd/IVA 150 mg q12h for 4 weeks, participants received VX-659 400 mg qd/TEZ 100 mg qd/IVA 150 mg q12h in TC treatment period for 4 weeks and TEZ 100 mg qd/IVA 150 mg q12h in washout period for 4 weeks.
Part 3: VX-659/TEZ/VX-561 TCTEZParticipants received VX-659 400 mg qd/TEZ 100 mg qd/VX-561 200 mg qd in TC treatment period for 4 weeks.
Part 2: VX-659/TEZ/IVA TCVX-659Following run-in period with TEZ 100 mg qd/IVA 150 mg q12h for 4 weeks, participants received VX-659 400 mg qd/TEZ 100 mg qd/IVA 150 mg q12h in TC treatment period for 4 weeks and TEZ 100 mg qd/IVA 150 mg q12h in washout period for 4 weeks.
Primary Outcome Measures
NameTimeMethod
Safety and Tolerability as Assessed by Number of Participants With Treatment Emergent Adverse Events (TEAEs) and Serious Adverse Events (SAEs)Day 1 Through Safety Follow-up (up to Day 61 for Part 1, Day 85 for Part 2 and Day 57 for Part 3)
Absolute Change in Percent Predicted Forced Expiratory Volume in 1 Second (ppFEV1)From Baseline Through Day 29

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 Sweat Chloride ConcentrationsFrom Baseline Through Day 29

Sweat samples were collected using an approved collection device.

Relative Change in ppFEV1From Baseline Through Day 29

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

Absolute Change in Cystic Fibrosis Questionnaire-Revised (CFQ-R) Respiratory Domain ScoreFrom Baseline at Day 29

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.

Observed Pre-dose Concentration (Ctrough) of VX-659, TEZ, M1-TEZ, IVA, M1-IVA, and VX-561Pre-dose at Day 15 and Day 29

Trial Locations

Locations (46)

Rutgers-Robert Wood Johnson Medical School/ Rutgers-Robert Wood Johnson Medical School, Clinical Research Center

🇺🇸

New Brunswick, New Jersey, United States

Yale New Haven Hospital

🇺🇸

New Haven, Connecticut, United States

University of Michigan Health System

🇺🇸

Ann Arbor, Michigan, United States

University of Massachusetts Memorial Medical Center

🇺🇸

Worcester, Massachusetts, United States

The Johns Hopkins Hospital/ Johns Hopkins Hospital, David Rubenstein Child Health Building

🇺🇸

Baltimore, Maryland, United States

Sanford Research / USD

🇺🇸

Sioux Falls, South Dakota, United States

University of Tennessee Medical Center-Adult Cystic Fibrosis Clinic

🇺🇸

Knoxville, Tennessee, United States

St. Vincent's University Hosptial

🇮🇪

Dublin, Ireland

Schneider Children's Medical Center

🇮🇱

Tikvah, Israel

Hadassah Medical Organization

🇮🇱

Jerusalem, Israel

Boston Children's Hospital

🇺🇸

Boston, Massachusetts, United States

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

🇺🇸

Morton Grove, Illinois, United States

The University of Iowa Hospitals and Clinics

🇺🇸

Iowa City, Iowa, United States

Helen DeVos Children's Hospital CF Center

🇺🇸

Grand Rapids, Michigan, United States

Northwell Health, Long Island Jewish Medical Center

🇺🇸

New Hyde Park, New York, United States

Albany Medical College

🇺🇸

Albany, New York, United States

Columbia University Medical Center

🇺🇸

New York, New York, United States

SUNY Upstate Medical University

🇺🇸

Syracuse, New York, United States

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

🇺🇸

Pittsburgh, Pennsylvania, United States

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

🇺🇸

Memphis, Tennessee, United States

Galway University Hospitals

🇮🇪

Galway, Ireland

Providence Pediatric Pulmonary & Allergy/Immunology Clinic

🇺🇸

Spokane, Washington, United States

Cork University Hospital

🇮🇪

Cork, Ireland

University Hospital Limerick

🇮🇪

Limerick, Ireland

Carmel Medical Center

🇮🇱

Haifa, Israel

Ruth Children's Hospital Rambam Health Care Campus

🇬🇧

Nottingham, United Kingdom

The Chaim Sheba medical center

🇮🇱

Ramat Gan, Israel

Birmingham Heartlands Hospital

🇬🇧

Birmingham, United Kingdom

Papworth Hospital NHS Foundation Trust, Papworth Everard

🇬🇧

Cambridge, United Kingdom

Royal Devon and Exeter NHS Foundation Trust, Royal Devon and Exeter Hospital

🇬🇧

Devon, United Kingdom

The Newcastle upon Tyne Hospitals NHS Foundation Trust, The Royal Victoria Infirmary

🇬🇧

Fulham, United Kingdom

Greater Glasgow and Clyde NHS Board, Glasgow Clinical Research Facility

🇬🇧

Glasgow, United Kingdom

Regional Respiratory Centre Belfast City Hospital

🇬🇧

London, United Kingdom

Southampton University Hospitals NHS Foundation Trust

🇬🇧

Hampshire, United Kingdom

Liverpool Heart and Chest Hospital

🇬🇧

Merseyside, United Kingdom

Nottingham University Hospitals NHS Trust

🇬🇧

Nottingham, United Kingdom

Royal Brompton & Harefied NHS Foundation Trust

🇬🇧

London, United Kingdom

University Hospital of South Manchester NHS Trust, North West Lung Centre

🇬🇧

Manchester, United Kingdom

University of Miami/Miller School of Medicine

🇺🇸

Miami, Florida, United States

Indiana University Health

🇺🇸

Indianapolis, Indiana, United States

Respiratory Diseases of Children & Adolescents

🇺🇸

Oklahoma City, Oklahoma, United States

Vanderbilt University Medical Center

🇺🇸

Nashville, Tennessee, United States

University of Utah / Primary Children's Medical Center

🇺🇸

Salt Lake City, Utah, United States

Seattle Children's Hospital

🇺🇸

Seattle, Washington, United States

University Hospital Llandough in Cardiff

🇬🇧

Cardiff, United Kingdom

Children's Mercy Hospital

🇺🇸

Kansas City, Missouri, United States

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