Status:

ACTIVE_NOT_RECRUITING

Defining Endpoints in Becker Muscular Dystrophy

Lead Sponsor:

Virginia Commonwealth University

Collaborating Sponsors:

Edgewise Therapeutics, Inc.

Conditions:

Becker Muscular Dystrophy

Muscular Dystrophies

Eligibility:

MALE

6+ years

Brief Summary

This is a 24-month, observational study of 50 participants with Becker muscular dystrophy (BMD)

Detailed Description

Becker Muscular Dystrophy (BMD) is most frequently due to in-frame mutations in the dystrophin gene that are associated with reduced levels of frequently shortened dystrophin, though other mutations m...

Eligibility Criteria

Inclusion

  • For ages 6-12
  • Clinically affected (defined as weakness on bedside evaluation in a pattern consistent with BMD)
  • Genetic confirmation of an in-frame dystrophin mutation
  • Ambulatory
  • Willing and able to give informed consent and follow all procedures and requirements
  • For ages 13 and older
  • Clinically affected (defined as weakness on bedside evaluation in a pattern consistent with BMD)
  • Genetic confirmation of a dystrophin mutation
  • Willing and able to give informed consent and follow all procedures and requirements
  • For participants in the MRI substudy:
  • 1\. Ambulatory, defined as able to walk 10 meters without assistive devices (orthotics allowed)

Exclusion

  • For ages 6-12
  • Out of frame dystrophin mutation
  • Use of chronic corticosteroids at baseline, defined as greater than 6 months of chronic use, will be limited to 20% of the overall population
  • Non-ambulatory, defined as the inability to walk 10 meters without assistive device (excluding orthotics)
  • \>16 hours of ventilatory support
  • Any other illness that would interfere with the ability to undergo safe testing or would interfere with interpretation of the results in the opinion of the site investigator.
  • Under the age of 6 at time of enrollment
  • For MR Cohort: Have contraindications to MRI or MRS (e.g., non-MR compatible implanted medical devices or severe claustrophobia)
  • For ages 13 and older
  • Loss of ambulation prior to age 16
  • Use of chronic corticosteroids, defined as greater than 6 months of chronic use, will be limited to 20% of the overall population
  • Less than 30% of the overall population will be non-ambulatory, defined as the inability to walk 10 meters without assistive device (excluding orthotics)
  • \>16 hours of ventilatory support
  • Subjects aged 13-16 only: time to rise \>10 seconds
  • For MR Cohort: Have contraindications to MRI or MRS (e.g., non-MR compatible implanted medical devices or severe claustrophobia)

Key Trial Info

Start Date :

April 13 2022

Trial Type :

OBSERVATIONAL

Allocation :

ESTIMATED

End Date :

May 1 2026

Estimated Enrollment :

80 Patients enrolled

Trial Details

Trial ID

NCT05257473

Start Date

April 13 2022

End Date

May 1 2026

Last Update

June 8 2025

Active Locations (11)

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Page 1 of 3 (11 locations)

1

University of California, Irvine

Orange, California, United States, 92868

2

University of Colorado Anschutz Medical Campus

Aurora, Colorado, United States, 80045

3

University of Iowa

Iowa City, Iowa, United States, 52242

4

University of Kansas Medical Center

Kansas City, Kansas, United States, 66160