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A Study to Evaluate the Safety and Efficacy of VX-121 Combination Therapy in Subjects With Cystic Fibrosis

Phase 2
Completed
Conditions
Cystic Fibrosis
Interventions
Drug: Placebo
Drug: TEZ/IVA
Drug: TEZ
Drug: IVA
Registration Number
NCT03912233
Lead Sponsor
Vertex Pharmaceuticals Incorporated
Brief Summary

The purpose of this study is to evaluate the safety, tolerability and efficacy of VX-121 combination therapy in subjects with cystic fibrosis (CF).

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
87
Inclusion Criteria
  • Part 1: Heterozygous for F508del and an MF mutation (F/MF)
  • Part 2: Homozygous for F508del (F/F)
  • FEV1 value ≥40% and ≤90% of the predicted mean for age, sex, and height

Key

Exclusion Criteria
  • History of clinically significant cirrhosis with or without portal hypertension
  • 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
SEQUENTIAL
Arm && Interventions
GroupInterventionDescription
Part 2: VX-121/TEZ/VX-561 TC - High DoseVX-121Following run-in period with TEZ 100 mg qd/IVA 150 mg q12h for 4 weeks, participants received VX-121 20 mg qd/TEZ 100 mg qd/VX-561 150 mg qd TC for 4 weeks in the treatment period and TEZ 100 mg qd/IVA 150 mg q12h for 4 weeks in the washout period.
Part 1: PlaceboPlaceboParticipants received placebo matched to VX-121/TEZ/VX-561 triple combination (TC) for 4 weeks in the treatment period and placebo matched to TEZ/VX-561 for 18 days in the washout period.
Part 1: VX-121/TEZ/VX-561 TC - Low DoseVX-121Participants received VX-121 5 milligram (mg) once daily (qd)/TEZ 100 mg qd/VX-561 150 mg qd TC for 4 weeks in the treatment period and TEZ 100 mg qd/VX-561 150 mg qd for 18 days in the washout period
Part 1: VX-121/TEZ/VX-561 TC - Low DoseTEZParticipants received VX-121 5 milligram (mg) once daily (qd)/TEZ 100 mg qd/VX-561 150 mg qd TC for 4 weeks in the treatment period and TEZ 100 mg qd/VX-561 150 mg qd for 18 days in the washout period
Part 1: VX-121/TEZ/VX-561 TC - Low DoseVX-561Participants received VX-121 5 milligram (mg) once daily (qd)/TEZ 100 mg qd/VX-561 150 mg qd TC for 4 weeks in the treatment period and TEZ 100 mg qd/VX-561 150 mg qd for 18 days in the washout period
Part 1: VX-121/TEZ/VX-561 TC - Medium DoseTEZParticipants received VX-121 10 mg qd/TEZ 100 mg qd/VX-561 150 mg qd TC for 4 weeks in the treatment period and TEZ 100 mg qd/VX-561 150 mg qd for 18 days in the washout period.
Part 1: VX-121/TEZ/VX-561 TC - Medium DoseVX-561Participants received VX-121 10 mg qd/TEZ 100 mg qd/VX-561 150 mg qd TC for 4 weeks in the treatment period and TEZ 100 mg qd/VX-561 150 mg qd for 18 days in the washout period.
Part 1: VX-121/TEZ/VX-561 TC - High DoseTEZParticipants received VX-121 20 mg qd/TEZ 100 mg qd/VX-561 150 mg qd TC for 4 weeks in the treatment period and TEZ 100 mg qd/VX-561 150 mg qd for 18 days in the washout period.
Part 1: VX-121/TEZ/VX-561 TC - High DoseVX-561Participants received VX-121 20 mg qd/TEZ 100 mg qd/VX-561 150 mg qd TC for 4 weeks in the treatment period and TEZ 100 mg qd/VX-561 150 mg qd for 18 days in the washout period.
Part 2: TEZ/IVATEZ/IVAFollowing run-in period with TEZ 100 mg qd/IVA 150 mg every 12 hours (q12h) for 4 weeks, participants received TEZ 100 mg qd/IVA 150 mg q12h for 4 weeks in the treatment period and TEZ 100 mg/IVA 150 mg q12h for 4 weeks in the washout period.
Part 2: TEZ/IVAIVAFollowing run-in period with TEZ 100 mg qd/IVA 150 mg every 12 hours (q12h) for 4 weeks, participants received TEZ 100 mg qd/IVA 150 mg q12h for 4 weeks in the treatment period and TEZ 100 mg/IVA 150 mg q12h for 4 weeks in the washout period.
Part 2: VX-121/TEZ/VX-561 TC - High DoseTEZFollowing run-in period with TEZ 100 mg qd/IVA 150 mg q12h for 4 weeks, participants received VX-121 20 mg qd/TEZ 100 mg qd/VX-561 150 mg qd TC for 4 weeks in the treatment period and TEZ 100 mg qd/IVA 150 mg q12h for 4 weeks in the washout period.
Part 2: VX-121/TEZ/VX-561 TC - High DoseVX-561Following run-in period with TEZ 100 mg qd/IVA 150 mg q12h for 4 weeks, participants received VX-121 20 mg qd/TEZ 100 mg qd/VX-561 150 mg qd TC for 4 weeks in the treatment period and TEZ 100 mg qd/IVA 150 mg q12h for 4 weeks in the washout period.
Part 1: VX-121/TEZ/VX-561 TC - High DoseVX-121Participants received VX-121 20 mg qd/TEZ 100 mg qd/VX-561 150 mg qd TC for 4 weeks in the treatment period and TEZ 100 mg qd/VX-561 150 mg qd for 18 days in the washout period.
Part 1: VX-121/TEZ/VX-561 TC - Medium DoseVX-121Participants received VX-121 10 mg qd/TEZ 100 mg qd/VX-561 150 mg qd TC for 4 weeks in the treatment period and TEZ 100 mg qd/VX-561 150 mg qd for 18 days in the washout period.
Primary Outcome Measures
NameTimeMethod
Safety and Tolerability as Assessed by Number of Participants With Treatment-Emergent Adverse Events (AEs) and Serious Adverse Events (SAEs)From Day 1 Through Safety Follow-up (up to Day 75 for Part 1 and up to Day 85 for Part 2)
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 (SwCl) ConcentrationsFrom Baseline Through Day 29

Sweat samples were collected using an approved collection device.

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 Plasma Concentration (Ctrough) of VX-121, TEZ and Its Metabolite (M1-TEZ) and, VX-561 and Its Metabolites (M1-VX-561 and M6-VX-561)Pre-dose at Day 15 and Day 29

Trial Locations

Locations (26)

HagaZiekenhuis van den Haag

🇳🇱

Den Haag, Netherlands

Erasmus Medical Center

🇳🇱

Rotterdam, Netherlands

Tulane Medical Center

🇺🇸

New Orleans, Louisiana, United States

Royal Brompton & Harefield NHS Foundation Trust, Royal Brompton Hospital

🇬🇧

London, United Kingdom

University of Minnesota

🇺🇸

Minneapolis, Minnesota, United States

Pneumologisches Studienzentrum Muenchen-West

🇩🇪

Muenchen, Germany

Columbia University Medical Center

🇺🇸

New York, New York, United States

University of Texas Health Science Center at San Antonio

🇺🇸

San Antonio, Texas, United States

Ruhrlandklinik Westdeutsches Lungenzentrum am Klinikum Essen

🇩🇪

Essen, Germany

University Medical Center, Utrecht, Department of Pulmonology and Tuberculosis

🇳🇱

Heidelberglaan, Netherlands

Charite Paediatric Pulmonology Department

🇩🇪

Berlin, Germany

UMC St. Radboud

🇳🇱

Nijmegen, Netherlands

All Wales Adult Cystic Fibrosis Centre, University Hospital Llandough

🇬🇧

Penarth, United Kingdom

University of Kentucky.

🇺🇸

Lexington, Kentucky, United States

Keck Medical Center of University of Southern California

🇺🇸

Los Angeles, California, United States

Boston Children's Hospital

🇺🇸

Boston, Massachusetts, United States

Vanderbilt University Medical Center

🇺🇸

Nashville, Tennessee, United States

Nationwide Children's Hospital

🇺🇸

Columbus, Ohio, United States

Santiago Reyes, M.D.

🇺🇸

Oklahoma City, Oklahoma, United States

Academic Medical Center

🇳🇱

Amsterdam, Netherlands

University Hospitals Birmingham NHS Foundation Trust

🇬🇧

Birmingham, United Kingdom

Hospital de Santa Maria

🇵🇹

Lisbon, Portugal

Wythenshawe Hospital

🇬🇧

Manchester, United Kingdom

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

🇬🇧

Newcastle Upon Tyne, United Kingdom

Kaiser Permanente

🇺🇸

Oakland, California, United States

Wake Forest University Baptist Medical Center

🇺🇸

Winston-Salem, North Carolina, United States

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