NCT04509050

Brief Summary

This is a two-part, multi-center, prospective longitudinal, exploratory study of highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulators and their impact on children with cystic fibrosis (CF).

Trial Health

77
On Track

Trial Health Score

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

Enrollment
210

participants targeted

Target at P75+ for all trials

Timeline
43mo left

Started Nov 2020

Longer than P75 for all trials

Geographic Reach
1 country

35 active sites

Status
recruiting

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

Study Progress61%
Nov 2020Dec 2029

First Submitted

Initial submission to the registry

August 6, 2020

Completed
5 days until next milestone

First Posted

Study publicly available on registry

August 11, 2020

Completed
3 months until next milestone

Study Start

First participant enrolled

November 18, 2020

Completed
9 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

December 1, 2029

Expected
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

December 1, 2029

Last Updated

August 12, 2024

Status Verified

August 1, 2024

Enrollment Period

9 years

First QC Date

August 6, 2020

Last Update Submit

August 8, 2024

Conditions

Keywords

Cystic FibrosisCFCFTR Modulatortriple combination therapyelexacaftortezacaftorivacaftor

Outcome Measures

Primary Outcomes (4)

  • Part A Primary Outcome Measure: Change in weight-for-age z-scores

    Weight-for-age z-scores over time

    Baseline to 12 months

  • Part A Primary Outcome Measure: Change in height-for-age z-scores

    Height-for-age z-scores over time

    Baseline to 12 months

  • Part B Primary Outcome Measure: Change in weight-for-age z-scores

    Change in weight-for-age z-scores from baseline

    Baseline to 12 months

  • Part B Primary Outcome Measure: Change in height-for-age z-scores

    Change in height-for-age z-scores from baseline

    Baseline to 12 months

Study Arms (2)

Part A

Children with CF not on ivacaftor or elexacaftor/tezacaftor/ivacaftor CFTR modulator therapy.

Part B

Children with CF planning to start ivacaftor or elexacaftor/tezacaftor/ivacaftor CFTR modulator therapy. Participants from the Part A cohort of this study may enroll into the Part B cohort if they become eligible for these CFTR modulator therapies and plan to start them.

Drug: Ivacaftor or elexacaftor/tezacaftor/ivacaftor

Interventions

In Part B, approved CFTR modulator as prescribed at the discretion of the treating physician -not dictated by the BEGIN investigators

Also known as: Kalydeco or Trikafta, Vertex (VX)-770 or VX-445/VX-661/VX-770
Part B

Eligibility Criteria

AgeUp to 10 Years
Sexall
Healthy VolunteersNo
Age GroupsChild (0-17)
Sampling MethodNon-Probability Sample
Study Population

Part A - children with confirmed diagnosis of cystic fibrosis who are less than 10 years of age and not on ivacaftor or elexacaftor/tezacaftor/ivacaftor CFTR modulator therapy Part B - children with confirmed diagnosis of cystic fibrosis who are less than 7 years of age (or participated in Part A of the study) with an intention to start ivacaftor or elexacaftor/tezacaftor/ivacaftor CFTR modulator therapy

You may qualify if:

  • Part A:
  • Less than 10 years of age at the first study visit.
  • Documentation of a CF diagnosis.
  • Part B:
  • Participated in Part A OR less than 7 years of age at the first study visit.
  • Documentation of a CF diagnosis.
  • CFTR mutations consistent with FDA labeled indication of highly effective modulator therapy (ivacaftor or elexacaftor/tezacaftor/ivacaftor).
  • Physician intent to prescribe ivacaftor or elexacaftor/tezacaftor/ivacaftor.

You may not qualify if:

  • Part A and Part B:
  • Use of an investigational drug within 28 days prior to and including the first study visit.
  • Use of ivacaftor or elexacaftor/tezacaftor/ivacaftor within the 28 days prior to and including the first study visit.
  • Use of chronic oral corticosteroids within the 28 days prior to and including the first study visit.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (35)

The Children's Hospital Alabama, University of Alabama at Birmingham

Birmingham, Alabama, 35233, United States

RECRUITING

Stanford University Medical Center

Palo Alto, California, 94304, United States

RECRUITING

Children's Hospital Colorado

Aurora, Colorado, 80045, United States

RECRUITING

Nemours Children's Clinic

Jacksonville, Florida, 32207, United States

RECRUITING

University of Miami

Miami, Florida, 33136, United States

RECRUITING

The Nemours Children's Clinic - Orlando

Orlando, Florida, 32827, United States

RECRUITING

Riley Hospital for Children

Indianapolis, Indiana, 46202, United States

RECRUITING

University of Iowa

Iowa City, Iowa, 52242, United States

RECRUITING

University of Kansas Medical Center

Kansas City, Kansas, 66160, United States

RECRUITING

Boston Children's Hospital

Boston, Massachusetts, 02115, United States

RECRUITING

University of Michigan, Michigan Medicine

Ann Arbor, Michigan, 48109, United States

RECRUITING

Helen DeVos Children's Hospital

Grand Rapids, Michigan, 49503, United States

RECRUITING

Children's Hospitals and Clinics of Minnesota

Minneapolis, Minnesota, 55404, United States

RECRUITING

The Minnesota Cystic Fibrosis Center

Minneapolis, Minnesota, 55455, United States

RECRUITING

Children's Mercy Kansas City

Kansas City, Missouri, 64108, United States

RECRUITING

St. Louis Children's Hospital

St Louis, Missouri, 63110, United States

RECRUITING

The Cystic Fibrosis Center of Western New York

Buffalo, New York, 14203, United States

RECRUITING

Children's Hospital of New York

New York, New York, 10032, United States

RECRUITING

SUNY Upstate Medical University

Syracuse, New York, 13210, United States

RECRUITING

New York Medical College at Westchester Medical Center

Valhalla, New York, 10595, United States

RECRUITING

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, 45229, United States

RECRUITING

Nationwide Children's Hospital

Columbus, Ohio, 43205, United States

RECRUITING

Oklahoma Cystic Fibrosis Center

Oklahoma City, Oklahoma, 73104, United States

RECRUITING

Oregon Health Sciences University

Portland, Oregon, 97239, United States

RECRUITING

Hershey Medical Center Pennsylvania State University

Hershey, Pennsylvania, 17033, United States

RECRUITING

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, 19104, United States

RECRUITING

Children's Hospital of Pittsburgh of UPMC

Pittsburgh, Pennsylvania, 15224, United States

RECRUITING

University of Texas Southwestern / Children's Health

Dallas, Texas, 75207, United States

RECRUITING

Cook Children's Medical Center

Fort Worth, Texas, 76104, United States

RECRUITING

Baylor College of Medicine

Houston, Texas, 77030, United States

RECRUITING

Primary Children's Cystic Fibrosis Center

Salt Lake City, Utah, 84113, United States

RECRUITING

Vermont Children's Hospital

Burlington, Vermont, 05401, United States

RECRUITING

University of Virginia

Charlottesville, Virginia, 22903, United States

RECRUITING

Seattle Children's Hospital

Seattle, Washington, 98105, United States

RECRUITING

University of Wisconsin

Madison, Wisconsin, 53792, United States

RECRUITING

Biospecimen

Retention: SAMPLES WITH DNA

Serum, Plasma, Buffy Coat, Urine, Stool, oropharyngeal (OP) swab, Bronchoalveolar lavage (BAL)

MeSH Terms

Conditions

Cystic Fibrosis

Interventions

elexacaftor, ivacaftor, tezacaftor drug combinationivacaftor

Condition Hierarchy (Ancestors)

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

Study Officials

  • Bonnie Ramsey, MD

    Seattle Children's Hospital

    PRINCIPAL INVESTIGATOR
  • Lucas Hoffman, MD PhD

    University of Washington/Seattle Children's

    PRINCIPAL INVESTIGATOR
  • Katie Larson Ode, MD

    University of Iowa

    PRINCIPAL INVESTIGATOR

Central Study Contacts

Study Design

Study Type
observational
Observational Model
COHORT
Time Perspective
PROSPECTIVE
Sponsor Type
OTHER
Responsible Party
SPONSOR INVESTIGATOR
PI Title
Associate Professor University of Washington

Study Record Dates

First Submitted

August 6, 2020

First Posted

August 11, 2020

Study Start

November 18, 2020

Primary Completion (Estimated)

December 1, 2029

Study Completion (Estimated)

December 1, 2029

Last Updated

August 12, 2024

Record last verified: 2024-08

Data Sharing

IPD Sharing
Will not share

Locations