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A Phase 3 Study of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Heterozygous for the F508del Mutation and a Minimal Function Mutation (F/MF)

Phase 3
Completed
Conditions
Cystic Fibrosis
Interventions
Drug: Placebo
Drug: VX-445/TEZ/IVA
Drug: IVA
Registration Number
NCT03525444
Lead Sponsor
Vertex Pharmaceuticals Incorporated
Brief Summary

This study will evaluate the efficacy of VX-445 in triple combination (TC) with tezacaftor (TEZ) and ivacaftor (IVA) in subjects with cystic fibrosis (CF) who are heterozygous for F508del and a minimal function mutation (F/MF subjects).

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
405
Inclusion Criteria
  • Heterozygous for the F508del mutation (F/MF)
  • 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
PlaceboPlaceboParticipants who received placebo matched to VX-445/TEZ/IVA for 24 weeks in the TC treatment period.
VX-445/TEZ/IVA TCIVAParticipants who received VX-445 200 mg/TEZ 100 mg/IVA150 mg as fixed-dose combination (FDC) tablets in the morning and IVA 150 mg as mono tablet in the evening for 24 weeks in the TC treatment period.
VX-445/TEZ/IVA TCVX-445/TEZ/IVAParticipants who received VX-445 200 mg/TEZ 100 mg/IVA150 mg as fixed-dose combination (FDC) tablets in the morning and IVA 150 mg as mono tablet in the evening for 24 weeks in the 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 Sweat Chloride (SwCl)From Baseline through Week 24

Sweat samples were collected using an approved collection device.

Number of Pulmonary Exacerbations (PEx)From Baseline through Week 24

Pulmonary exacerbation was defined as the treatment with new or changed antibiotic therapy (intravenous, inhaled, or oral) for greater than or equal to 4 sinopulmonary signs/symptoms.

Time-to-first Pulmonary Exacerbation (PEx)From Baseline through Week 24

Pulmonary exacerbation was defined as the treatment with new or changed antibiotic therapy (intravenous, inhaled, or oral) for greater than or equal to 4 sinopulmonary signs/symptoms.

Absolute Change in Body WeightFrom Baseline at Week 24
Absolute Change in Sweat ChlorideFrom Baseline at Week 4

Sweat samples were collected using an approved collection device.

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.

Absolute Change in BMI Z-score for Participants <=20 Years of Age at BaselineFrom Baseline at Week 24

BMI was defined as weight in kg divided by height in m\^2. Z-score is a statistical measure to describe whether a mean was above or below the standard. BMI, adjusted for age and sex, was analyzed as BMI-for-age z-score. A z-score of 0 is equal to the mean and is considered normal. Lower numbers indicate values lower than the mean and higher numbers indicate values higher than the mean. Higher values are indicative of higher BMI.

Absolute Change in Percent Predicted Forced Expiratory Volume in 1 Second (ppFEV1)From Baseline through Week 24

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

Absolute Change in Body Mass Index (BMI)From Baseline at Week 24

BMI was defined as weight in kilogram (kg) divided by height in square meter (m\^2).

Safety and Tolerability as Assessed by 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 28 weeks)
Observed Pre-dose Concentration (Ctrough) of VX-445, TEZ, M1-TEZ, and IVAPre-dose on Week 4, 8, 12, and 16

Trial Locations

Locations (115)

Maine Medical Partners

🇺🇸

Portland, Maine, United States

University of Arkansas for Medical Sciences

🇺🇸

Little Rock, Arkansas, United States

Miller Children's Hospital/ Long Beach Memorial

🇺🇸

Long Beach, California, United States

Kaiser Permanente

🇺🇸

Oakland, California, United States

Fakultni nemocnice v Motole

🇨🇿

Praha 5, Czechia

Women & Children's Hospital

🇦🇺

North Adelaide, Australia

ProMedica Toledo Hospital/ Toledo Children's Hospital/ Pediatric Pulmonary & Cystic Fibrosis Center

🇺🇸

Toledo, Ohio, United States

University of Wisconsin Hospitals and Clinics

🇺🇸

Madison, Wisconsin, United States

Dayton Children's Hospital

🇺🇸

Dayton, Ohio, United States

Children's Hospital of Philadelphia

🇺🇸

Philadelphia, Pennsylvania, United States

Mater Adult Hospital

🇦🇺

South Brisbane, Australia

Vermont Lung Center

🇺🇸

Colchester, Vermont, United States

Saint Francis Medical Center/ Children's Hospital of Illinois/OSF

🇺🇸

Peoria, Illinois, United States

Billings Clinic

🇺🇸

Billings, Montana, United States

Morristown Medical Center

🇺🇸

Morristown, New Jersey, United States

CHU de Nice - Hopital Pasteur

🇫🇷

Nice, France

Universitair Ziekenhuis Gent

🇧🇪

Gent, Belgium

University of Calgary Medical Clinic of the Foothills Medical Centre

🇨🇦

Calgary, Canada

Academic Medical Center

🇳🇱

Amsterdam, Netherlands

Azienda Ospedaliero Universitaria Ospedale Riuniti

🇮🇹

Ancona, Italy

Dr. von Haunersches Kinderspital

🇩🇪

München, Germany

The Royal Belfast Hospital for Sick Children

🇬🇧

Belfast, United Kingdom

Heidelberg Cystic Fibrosis Center

🇩🇪

Heidelberg, Germany

UMC St. Radboud

🇳🇱

Nijmegen, Netherlands

Karolinska Univeritetssjukhuset, Huddinge

🇸🇪

Stockholm, Sweden

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

🇬🇧

Exeter, United Kingdom

Southampton General Hospital

🇬🇧

Southampton, United Kingdom

Massachusetts General Hospital Cystic Fibrosis Center

🇺🇸

Boston, Massachusetts, United States

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

🇺🇸

Cleveland, Ohio, United States

Valley Children's Hospital/ Children's Hospital of Central California

🇺🇸

Madera, California, United States

Children's Speciality Services at North Druid Hills

🇺🇸

Atlanta, Georgia, United States

Nationwide Children's Hospital

🇺🇸

Columbus, Ohio, United States

Ann & Robert Lurie Children's Hospital of Chicago

🇺🇸

Chicago, Illinois, United States

Joe DiMaggio Cystic Fibrosis & Pulmonary Center/ Joe DiMaggio Children's Hospital/ Memorial Regional Hospital

🇺🇸

Hollywood, Florida, United States

Nemours Children's Specialty Care

🇺🇸

Jacksonville, Florida, United States

Augusta University

🇺🇸

Augusta, Georgia, United States

Northwestern Memorial Hospital

🇺🇸

Chicago, Illinois, United States

University of New Mexico Clinical & Translational Science Center

🇺🇸

Albuquerque, New Mexico, United States

Akron Children's Hospital

🇺🇸

Akron, Ohio, United States

The University of Texas Health Science Center at Tyler

🇺🇸

Tyler, Texas, United States

Children's Hospital of the King's Daughters

🇺🇸

Norfolk, Virginia, United States

The Royal Children's Hospital

🇦🇺

Parkville, Australia

Westmead Hospital

🇦🇺

Westmead, Australia

LKH - Universitätsklinikum der PMU Salzburg

🇦🇹

Salzburg, Austria

University of Graz

🇦🇹

Graz, Austria

Medizinische Universitat Innsbruck

🇦🇹

Innsbruck, Austria

Medizinische Universitat Wien

🇦🇹

Vienna, Austria

Cliniques Universitaires de Bruxelles Hopital Erasme

🇧🇪

Brussels, Belgium

Universitair Ziekenhuis Brussel - Campus Jette

🇧🇪

Brussels, Belgium

UZ Antwerpen

🇧🇪

Edegem, Belgium

British Columbia's Children's Hospital

🇨🇦

Vancouver, British Columbia, Canada

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

🇨🇦

Montréal, Quebec, Canada

Centre Hospitalier De L'Universite Laval

🇨🇦

Qubec, Canada

Saint John Regional Hospital

🇨🇦

Saint John, Canada

The Hospital for Sick Children

🇨🇦

Toronto, Canada

Vancouver Island Health Authority

🇨🇦

Victoria, Canada

Fakultni Nemocnice Brno

🇨🇿

Brno, Czechia

Centre Hospitalier Lyon Sud

🇫🇷

Benite Cedex, France

Groupe Hospitaler Pellegrin, CHU De Bordeaux

🇫🇷

Bordeaux Cedex, France

CHU Marseille - Hopital Nord

🇫🇷

Marseille, France

Hopital Cochin

🇫🇷

Paris, France

Hopital Foch (Suresnes), Hopital Foch, Adultes

🇫🇷

Suresnes, France

Friedrich-Alexander University of Erlangen-Nuremberg, University Children's Hospital

🇩🇪

Erlangen, Germany

CHU de Rouen - Hopital Charles Nicolle

🇫🇷

Rouen Cedex, Seine Maritime, France

Justus-Leibig-Universitat Zentrum fur Kinderheilkunde und Jugendmedizin

🇩🇪

Giessen, Germany

Hannover Medical School

🇩🇪

Hannover, Germany

Johannes Gutenberg-Universitaet

🇩🇪

Mainz, Germany

University Hospital Wuerzburg

🇩🇪

Wuerzburg, Germany

Universitaetsklinikum Tuebingen Klinik fuer Kinder- und Jugendmedizin

🇩🇪

Tuebingen, Germany

General Hospital of Attika "Sismanoglio"(Adult CF center, NHS)

🇬🇷

Maroúsi, Greece

Azienda Ospedaliero Universitaria Ospedale Pediatrico Meyer

🇮🇹

Firenze, Italy

HagaZiekenhuis van den Haag

🇳🇱

Den Haag, Netherlands

Erasmus Medical Center

🇳🇱

Rotterdam, Netherlands

Heart of England NHS Foundation Trust, Birmingham Heartlands Hospital

🇬🇧

Brimingham, United Kingdom

Royal Hospital for Sick Children

🇬🇧

Edinburgh, United Kingdom

Western General Hospital

🇬🇧

Edinburgh, United Kingdom

Royal Manchester Children's Hospital

🇬🇧

Manchester, United Kingdom

Leeds General Infirmary

🇬🇧

Leeds, United Kingdom

King's College Hospital

🇬🇧

London, United Kingdom

Children's Respiratory and Critical Care Specialists, P.A., Children's Hospitals and Clinics of Minnesota

🇺🇸

Minneapolis, Minnesota, United States

University of Washington Medical Center

🇺🇸

Seattle, Washington, United States

UCSF Gateway Medical Center

🇺🇸

San Francisco, California, United States

New York Medical College

🇺🇸

Valhalla, New York, United States

Harper University Hospital

🇺🇸

Detroit, Michigan, United States

IRCCS Istituto Giannina Gaslini-Ospedale Pediatrico

🇮🇹

Genova, Italy

Azienda Ospedaliera Universitaria Policlinico G. Martino

🇮🇹

Messina, Italy

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico

🇮🇹

Milano, Italy

Centro Regionale Fibrosi Cistica, A.O. Ospedale San Carlo

🇮🇹

Potenza, Italy

Azienda Ospedaliera di Verona-Ospedale Civile Maggiore

🇮🇹

Verona, Italy

University of Oklahoma Health Sciences Center

🇺🇸

Oklahoma City, Oklahoma, United States

The Children's Hospital at Westmead

🇦🇺

Westmead, Australia

Mount Sinai Beth Israel

🇺🇸

New York, New York, United States

The University of Texas Southwestern Medical Center

🇺🇸

Dallas, Texas, United States

Children's Hospital Los Angeles

🇺🇸

Los Angeles, California, United States

UC Health Holmes

🇺🇸

Cincinnati, Ohio, United States

Banner University of Arizona Medical Center

🇺🇸

Tucson, Arizona, United States

University of California Davis Medical Center

🇺🇸

Sacramento, California, United States

National Jewish Health

🇺🇸

Denver, Colorado, United States

Central Florida Pulmonary Group

🇺🇸

Orlando, Florida, United States

Nemours Children's Hospital

🇺🇸

Orlando, Florida, United States

Tampa General Hospital Cardiac and Lung Transplant Clinic

🇺🇸

Tampa, Florida, United States

Nebraska Medical Center

🇺🇸

Omaha, Nebraska, United States

UNC Marsico Clinical Research Center

🇺🇸

Chapel Hill, North Carolina, United States

Medical University of South Carolina

🇺🇸

Charleston, South Carolina, United States

CTSI Adult Translational Research Unit/Medical College of Wisconsin/Froedtert Hospital

🇺🇸

Milwaukee, Wisconsin, United States

Universitaire Ziekenhuizen Leuven - Campus Gathuisberg

🇧🇪

Leuven, Belgium

St. Paul's Hospital

🇨🇦

Vancouver, British Columbia, Canada

West Virginia University

🇺🇸

Morgantown, West Virginia, United States

University Medical Center, Utrecht, Department of Pulmonology and Tuberculosis

🇳🇱

Heidelberglaan, Netherlands

University of Florida, Shands Hospital

🇺🇸

Gainesville, Florida, United States

Tulane Medical Center

🇺🇸

New Orleans, Louisiana, United States

Austin Children's Chest Associates

🇺🇸

Austin, Texas, United States

Children's Hospital of Richmond at VCU, Children's Pavilion

🇺🇸

Richmond, Virginia, United States

University of Virginia Primary Care Center

🇺🇸

Charlottesville, Virginia, United States

University of Kansas Medical Center

🇺🇸

Kansas City, Kansas, United States

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