NCT04058353

Brief Summary

This study will evaluate the efficacy, safety and pharmacodynamics of elexacaftor (ELX, 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 gating or residual function mutation (F/G and F/RF genotypes).

Trial Health

98
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
271

participants targeted

Target at P50-P75 for phase_3

Timeline
Completed

Started Aug 2019

Shorter than P25 for phase_3

Geographic Reach
13 countries

93 active sites

Status
completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

First Submitted

Initial submission to the registry

August 14, 2019

Completed
1 day until next milestone

First Posted

Study publicly available on registry

August 15, 2019

Completed
13 days until next milestone

Study Start

First participant enrolled

August 28, 2019

Completed
10 months until next milestone

Primary Completion

Last participant's last visit for primary outcome

June 12, 2020

Completed
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

June 12, 2020

Completed
1.1 years until next milestone

Results Posted

Study results publicly available

July 2, 2021

Completed
Last Updated

July 2, 2021

Status Verified

June 1, 2021

Enrollment Period

10 months

First QC Date

August 14, 2019

Results QC Date

June 11, 2021

Last Update Submit

June 11, 2021

Conditions

Outcome Measures

Primary Outcomes (1)

  • Absolute Change in Percent Predicted Forced Expiratory Volume in 1 Second (ppFEV1) for ELX/TEZ/IVA Group

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

    From Baseline Through Week 8

Secondary Outcomes (6)

  • Absolute Change in Sweat Chloride (SwCl) for ELX/TEZ/IVA Group

    From Baseline Through Week 8

  • Absolute Change in ppFEV1 for the ELX/TEZ/IVA Group Compared to the Control Group

    From Baseline Through Week 8

  • Absolute Change in SwCl for ELX/TEZ/IVA Group Compared to the Control Group

    From Baseline Through Week 8

  • Absolute Change in Cystic Fibrosis Questionnaire-Revised (CFQ-R) Respiratory Domain Score for ELX/TEZ/IVA Group

    From Baseline Through Week 8

  • Absolute Change in CFQ-R Respiratory Domain Score for ELX/TEZ/IVA Group Compared to the Control Group

    From Baseline Through Week 8

  • +1 more secondary outcomes

Study Arms (2)

Control: IVA or TEZ/IVA

ACTIVE COMPARATOR

Following an IVA or TEZ/IVA run-in period of 4 weeks, participants either received IVA 150 milligrams (mg) every 12 hours (q12h) or TEZ 100 mg once daily (qd)/IVA 150 mg q12h in the treatment period for 8 weeks.

Drug: IVADrug: TEZ/IVA

TC: ELX/TEZ/IVA

EXPERIMENTAL

Following an IVA or TEZ/IVA run-in period of 4 weeks, participants received ELX 200 mg qd/TEZ 100 mg qd/IVA 150 mg q12h in the treatment period for 8 weeks.

Drug: ELX/TEZ/IVADrug: IVA

Interventions

FDC tablet for oral administration.

Also known as: VX-445/VX-661/VX-770, elexacaftor/tezacaftor/ivacaftor
TC: ELX/TEZ/IVA
IVADRUG

Mono-tablet for oral administration.

Also known as: VX-770, ivacaftor
Control: IVA or TEZ/IVATC: ELX/TEZ/IVA

Fixed-dose combination (FDC) tablet for oral administration.

Also known as: VX-661/VX-770, tezacaftor/ivacaftor
Control: IVA or TEZ/IVA

Eligibility Criteria

Age12 Years+
Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

  • Subject has a confirmed diagnosis of CF and is heterozygous for F508del and either a gating or residual function mutation (F/G and F/RF genotypes)
  • Forced expiratory volume in 1 second (FEV1) value ≥40% and ≤90% of predicted mean for age, sex, and height

You may not qualify if:

  • 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

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (93)

University of Alabama at Birmingham

Birmingham, Alabama, 35233, United States

Location

Banner University of Arizona Medical Center

Tucson, Arizona, 85724, United States

Location

Miller Children's Hospital / Long Beach Memorial

Long Beach, California, 90806, United States

Location

Stanford University

Palo Alto, California, 94304, United States

Location

University of California Davis Medical Center

Sacramento, California, 95817, United States

Location

University of California San Francisco, Lung Transplant Program

San Francisco, California, 94143, United States

Location

National Jewish Health

Denver, Colorado, 80206, United States

Location

University of Miami Miller School of Medicine

Miami, Florida, 33136, United States

Location

Central Florida Pulmonary Group, PA

Orlando, Florida, 32803, United States

Location

Indiana University

Indianapolis, Indiana, 46202, United States

Location

The University of Iowa Hospitals and Clinics

Iowa City, Iowa, 52242, United States

Location

University of Kansas Medical Center

Kansas City, Kansas, 66160, United States

Location

University of Kentucky

Lexington, Kentucky, 40536, United States

Location

Massachusetts General Hospital Cystic Fibrosis Center Clinical Rsearch Center

Boston, Massachusetts, 02114, United States

Location

Boston Children's Hospital

Boston, Massachusetts, 02115, United States

Location

Michigan Medicine

Ann Arbor, Michigan, 48109-5212, United States

Location

University of Minnesota

Minneapolis, Minnesota, 55455, United States

Location

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

St Louis, Missouri, 63110, United States

Location

Dartmouth Hitchcock Medical Center

Lebanon, New Hampshire, 03756, United States

Location

Northwell Health- Long Island Jewish Medical Center

New Hyde Park, New York, 11040, United States

Location

Mount Sinai Beth Israel

New York, New York, 10003, United States

Location

Columbia University Medical Center

New York, New York, 10032, United States

Location

University of North Carolina Hospitals

Chapel Hill, North Carolina, 27514, United States

Location

UC Health Holmes

Cincinnati, Ohio, 45220, United States

Location

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

Cleveland, Ohio, 44106, United States

Location

Nationwide Children's Hospital

Columbus, Ohio, 43205, United States

Location

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

Toledo, Ohio, 43606, United States

Location

University of Oklahoma Health Sciences Center

Oklahoma City, Oklahoma, 73104, United States

Location

Oregon Health & Science University

Portland, Oregon, 97239, United States

Location

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, 19104, United States

Location

Children's Hospital of Pittsburgh of UPMC

Pittsburgh, Pennsylvania, 15224, United States

Location

Dell Children's Medical Group

Austin, Texas, 78723, United States

Location

The University of Texas Southwestern Medical Center

Dallas, Texas, 75390, United States

Location

Texas Children's Hospital

Houston, Texas, 77030, United States

Location

University of Utah / Primary Children's Medical Center

Salt Lake City, Utah, 84132, United States

Location

University of Washington Medical Center

Seattle, Washington, 98195, United States

Location

University of Wisconsin Hospital and Clinics

Madison, Wisconsin, 53792, United States

Location

The Prince Charles Hospital

Chermside, Australia

Location

Alfred Hospital

Melbourne, VIC, Australia

Location

Perth Children's Hospital

Nedlands, Australia

Location

The Royal Children's Hospital

Parkville, VIC, Australia

Location

Mater Adult Hospital

South Brisbane, Australia

Location

Queensland Children's Hospital

South Brisbane, Australia

Location

Westmead Hospital

Westmead, Australia

Location

Cliniques Universitaires de Bruxelles Hopital Erasme

Brussels, Belgium

Location

Universitair Ziekenhuis Brussel - Campus Jette

Brussels, Belgium

Location

Universitair Ziekenhuis Gent

Ghent, Belgium

Location

Universitaire Ziekenhuizen Leuven - Campus Gasthuisberg

Leuven, Belgium

Location

Stollery Children's Hospital

Edmonton, Canada

Location

McGill University Health Center

Québec, Canada

Location

St. Michael's Hospital

Toronto, Canada

Location

St. Paul's Hospital

Vancouver, Canada

Location

Juliane Marie Center, Rigshospitalet

Copenhagen, Denmark

Location

Centre Hospitalier Lyon Sud

Benite Cedex, France

Location

Groupe Hospitaler Pellegrin, CHU De Bordeaux

Bordeaux, France

Location

CHRU de Lille - Hopital Albert Calmette

Lille, France

Location

CHU Marseille - Hopital Nord

Marseille, France

Location

CHU de Montpellier - Hopital Arnaud de Villeneuve

Montpellier, France

Location

Hopital Cochin

Paris, France

Location

Hopital Necker, Enfants Malades

Paris, France

Location

Hopital Pontchaillou CHU de Rennes

Rennes, France

Location

Charite Paediatric Pulmonology Department

Berlin, Germany

Location

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

Erlangen, Germany

Location

Ruhrlandklinik Westdeutsches Lungenzentrum am Klinikum Essen

Essen, Germany

Location

Justus-Liebig-Universitaet Gießen Zentrum fur Kinderheilkunde und Jugendmedizin

Giessen, Germany

Location

Universitätsklinikum Halle (Saale) / Universitätsklinik und Poliklinik für Innere Medizin, Schwerpunkt Pneumologie

Halle, Germany

Location

Pneumologisches Studienzentrum Muenchen-West

München, Germany

Location

University Hospital Wuerzburg

Würzburg, Germany

Location

Cork University Hospital

Cork, Ireland

Location

Beaumont Hospital

Dublin, Ireland

Location

St. Vincent's University Hospital

Dublin, Ireland

Location

University Hospital Limerick (Adults)

Limerick, Israel

Location

Azienda Ospedaliero Universitaria Ospedale Riuniti

Ancona, Italy

Location

IRCCS Istituto Giannina Gaslini-Ospedale Pediatrico

Genova, Italy

Location

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico

Milan, Italy

Location

Malattie Apparato Respiratorio 2 - Fibrosi Cistica

Orbassano, Italy

Location

Ospedale Pediatrico Bambino Gesu

Rome, Italy

Location

Azienda Ospedaliera di Verona-Ospedale Civile Maggiore

Verona, Italy

Location

University Medical Center, Utrecht, Department of Pulmonology and Tuberculosis

Heidelberglaan, Netherlands

Location

UMC St. Radboud

Nijmegen, Netherlands

Location

HagaZiekenhuis van den Haag

The Hague, Netherlands

Location

Hospital Universitari Vall d´Hebron Servicio de Broncoscopia

Barcelona, Spain

Location

Hospital Virgen de la Arrixaca

Murcia, Spain

Location

Hospital Universitario Virgen del Rocio

Seville, Spain

Location

Hospital Universitario y Politecnico La Fe

Valencia, Spain

Location

Papworth Hospital NHS Foundation Trust, Papworth Everard

Cambridge, United Kingdom

Location

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

Exeter, United Kingdom

Location

Clinical Research Facility, Queen Elizabeth University Hospital

Glasgow, United Kingdom

Location

St. James University Hospital

Leeds, United Kingdom

Location

Liverpool Heart and Chest Hospital

Liverpool, United Kingdom

Location

Royal Brompton & Harefield NHS Foundation Trust, Royal Brompton Hospital

London, United Kingdom

Location

Wythenshawe Hospital

Manchester, United Kingdom

Location

Southampton General Hospital

Southampton, United Kingdom

Location

Related Publications (1)

  • Barry PJ, Mall MA, Alvarez A, Colombo C, de Winter-de Groot KM, Fajac I, McBennett KA, McKone EF, Ramsey BW, Sutharsan S, Taylor-Cousar JL, Tullis E, Ahluwalia N, Jun LS, Moskowitz SM, Prieto-Centurion V, Tian S, Waltz D, Xuan F, Zhang Y, Rowe SM, Polineni D; VX18-445-104 Study Group. Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes. N Engl J Med. 2021 Aug 26;385(9):815-825. doi: 10.1056/NEJMoa2100665.

MeSH Terms

Conditions

Cystic Fibrosis

Interventions

elexacaftor, ivacaftor, tezacaftor drug combinationivacaftortezacaftor, ivacaftor drug combination

Condition Hierarchy (Ancestors)

Pancreatic DiseasesDigestive System DiseasesLung DiseasesRespiratory Tract DiseasesGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesInfant, Newborn, Diseases

Results Point of Contact

Title
Medical Monitor
Organization
Vertex Pharmaceuticals Incorporated

Publication Agreements

PI is Sponsor Employee
No
Restriction Type
OTHER
Restrictive Agreement
Yes

Study Design

Study Type
interventional
Phase
phase 3
Allocation
RANDOMIZED
Masking
QUADRUPLE
Who Masked
PARTICIPANT, CARE PROVIDER, INVESTIGATOR, OUTCOMES ASSESSOR
Purpose
TREATMENT
Intervention Model
PARALLEL
Sponsor Type
INDUSTRY
Responsible Party
SPONSOR
Expanded Access
Yes

Study Record Dates

First Submitted

August 14, 2019

First Posted

August 15, 2019

Study Start

August 28, 2019

Primary Completion

June 12, 2020

Study Completion

June 12, 2020

Last Updated

July 2, 2021

Results First Posted

July 2, 2021

Record last verified: 2021-06

Data Sharing

IPD Sharing
Will not share

Details on Vertex data sharing criteria and process for requesting access can be found at: https://www.vrtx.com/independent research/clinical-trial-data-sharing

Locations