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CompletedNCT05291091Updated Sep 21, 2026

Phase 2 Study of EDG-5506 in Becker Muscular Dystrophy (GRAND CANYON)

A Phase 2 interventional study of Sevasemten 10 mg and Sevasemten 5 mg in Becker Muscular Dystrophy, sponsored by Edgewise Therapeutics, Inc.. Completed at 51 sites in 12 countries. Open to male participants aged 12 Years to 50 Years. Per ClinicalTrials.gov, last updated 2026-09-21.

Sponsored by Edgewise Therapeutics, Inc. · Phase 2, Interventional, and Treatment

Phase
Phase 2
Study type
Interventional
Enrollment
244
Allocation
Randomized
Ages
12 Years to 50 Years
Sex
Male
01

Study summary

A study of sevasemten (EDG-5506) in Becker muscular dystrophy (known as CANYON) and pivotal cohort (known as GRAND CANYON). The EDG-5506-201 CANYON study was expanded to include an additional 175 adult participants in a cohort called GRAND CANYON, that is a multicenter, randomized, double-blind, placebo-controlled study to evaluate the safety and efficacy of sevasemten in adults with Becker.

Read the detailed description

The EDG-5506-201 protocol was amended to include an additional cohort thus consists of two parts.

Part 1: CANYON is a double-blind, randomized, placebo-controlled design to investigate the effect of sevasemten on the safety, pharmacokinetics, biomarkers, and functional measures. Approximately 32 adults and 18 adolescents with Becker muscular dystrophy are planned to enroll in this study. This study will have up to a 4-week Screening period, a 12-month Treatment period, followed by a 4-week follow-up period.

Approximately 32 adult participants will randomize to Cohort 1 or Cohort 2 in a 1:1 ratio then each cohort will further randomize to sevasemten or placebo in a 3:1 ratio.

Approximately 9 adolescent participants will enroll in Cohort 4 and randomize in a 2:1 ratio to sevasemten or placebo. Cohort 5 will randomize an additional 9 participants in a 2:1 ratio to either sevasemten or placebo after Cohort 4.

Part 2: GRAND CANYON or Cohort 6 is a double-blind, randomized, placebo-controlled design to investigate the safety and efficacy of sevasemten in adults with Becker muscular dystrophy after 18 months of treatment. Approximately 175 adults with Becker muscular dystrophy are planned to enroll in this study. This study will have up to a 4-week Screening period, an 18-month Treatment period, followed by a 4-week follow-up period.

Approximately 175 adult participants will be randomized in Cohort 6 in a 2:1 ratio either to sevasemten or placebo.

Enrollment for CANYON and GRAND CANYON has been completed.

02

Conditions studied

  • Becker Muscular Dystrophy

Keywords

  • Becker Muscular Dystrophy
03

Who can participate

Ages eligible
12 Years to 50 Years
Sexes eligible
Male
Accepts healthy volunteers
No

Eligibility criteria

GRAND CANYON eligibility is listed below.

Key Inclusion Criteria:

  1. Adults (aged 18 to 50 years, inclusive) with a documented dystrophin mutation and phenotype consistent with Becker muscular dystrophy, and history of being ambulatory beyond 16 years of age without steroids; history of being ambulatory beyond 18 years of age with steroids.
  2. Able to complete the 100-meter timed test in \< 200 seconds with or without use of mobility aid devices.
  3. Able to perform the North Star Ambulatory Assessment scale and achieve a score of 5 to 32, inclusive.

Key Exclusion Criteria:

  1. Medical history or clinically significant physical examination/laboratory result that, in the opinion of the investigator, would render the participant unsuitable for the study. This includes contraindications to magnetic resonance imaging such as non-compatible implanted medical devices or severe claustrophobia.
  2. Cardiac echocardiogram ejection fraction \< 40%
  3. Forced vital capacity predicted \<60% or using daytime ventilatory support
  4. Receipt of oral corticosteroids for the treatment of BMD in the previous 6 months.
  5. Receipt of an investigational drug within 30 days or 5 half-lives (whichever is longer) of the screening visit in the present study.
04

Study design

Phase
Phase 2
Primary purpose
Treatment
Allocation
Randomized
Intervention model
Sequential assignment
Masking
Quadruple (Participant, Care provider, Investigator, Outcomes assessor)
Enrollment
244 participants (actual)

Study arms

  • Experimental
    Adult Cohort 1

    Drug: Sevasemten Drug: Placebo

    Drug: Sevasemten 10 mg · Drug: Placebo

  • Experimental
    Adult Cohort 2

    Drug: Sevasemten Drug: Placebo

    Drug: Sevasemten 10 mg · Drug: Placebo

  • Experimental
    Adult Cohort 6

    Drug: Sevasemten Drug: Placebo

    Drug: Sevasemten 10 mg · Drug: Placebo

  • Experimental
    Adolescent Cohort 4

    Drug: Sevasemten Drug: Placebo

    Drug: Sevasemten 5 mg · Drug: Placebo

  • Experimental
    Adolescent Cohort 5

    Drug: Sevasemten Drug: Placebo

    Drug: Sevasemten 12.5 mg · Drug: Placebo

Interventions

  • DrugSevasemten 10 mg

    Sevasemten is administered orally once per day

  • DrugSevasemten 5 mg

    Sevasemten is administered orally once per day

  • DrugSevasemten 12.5 mg

    Sevasemten is administered orally once per day

  • DrugPlacebo

    Placebo is administered orally once per day

05

What researchers measure

Primary outcomes

  1. Number of treatment emergent adverse events in those treated with sevasemten or placebo

    All participants

    Time frame: 12 months (CANYON Cohorts 1, 2, 4, 5), 18 months (GRAND CANYON Cohort 6)

  2. Severity of treatment emergent adverse events in those treated with sevasemten or placebo

    All participants

    Time frame: 12 months (CANYON Cohorts 1, 2, 4, 5), 18 months (GRAND CANYON Cohort 6)

  3. Change from Baseline in serum Creatine Kinase

    Adult participants

    Time frame: Averaged across Months 6, 9, 12 Months (CANYON Cohorts 1, 2)

  4. Change from Baseline in total North Star Ambulatory Assessment score

    Adult participants

    Time frame: 18 months (GRAND CANYON Cohort 6)

Secondary outcomes

  1. Change from Baseline in total North Star Ambulatory Assessment score

    Adult participants

    Time frame: 12 Months (CANYON Cohorts 1, 2)

  2. Pharmacokinetics as measured by steady state plasma concentration

    All participants

    Time frame: 12 Months (CANYON Cohorts 1, 2, 4, 5), 18 months (GRAND CANYON Cohort 6)

  3. Change from Baseline in growth as assessed by height centile on World Health Organization growth charts

    Adolescent participants

    Time frame: 12 months (CANYON Cohorts 4, 5)

  4. Incidence of laboratory test-related treatment emergent adverse events

    Adult participants

    Time frame: 18 months (GRAND CANYON Cohort 6)

  5. Change from Baseline in serum Creatine Kinase

    Adult participants

    Time frame: 18 months (GRAND CANYON Cohort 6)

  6. Change from Baseline in plasma fast skeletal muscle Troponin I

    Adult participants

    Time frame: Averaged across Months 6 and 12 (CANYON Cohorts 1, 2); 18 months (GRAND CANYON Cohort 6)

  7. Change from Baseline in serum myoglobin

    Adult participants

    Time frame: Averaged across Months 6, 9, and 12 (CANYON Cohorts 1,2); 18 months (GRAND CANYON Cohort 6)

  8. Change from Baseline in total NSAD score (CANYON Cohorts 1,2)

    Adult participants

    Time frame: 12 Months (CANYON Cohorts 1, 2)

  9. Change from Baseline in 4-stair climb test

    Adult participants

    Time frame: 18 months (GRAND CANYON Cohort 6)

  10. Change from Baseline in the 10-meter walk/run test

    Adult participants

    Time frame: 12 Months (CANYON Cohorts 1, 2), 18 Months (GRAND CANYON Cohort 6)

  11. Change from Baseline in 100-meter timed test

    Adult participants

    Time frame: 12 Months (CANYON Cohorts 1, 2), 18 Months (GRAND CANYON Cohort 6)

  12. Change from Baseline in stride velocity (95th percentile)

    Adult participants

    Time frame: 18 Months (GRAND CANYON Cohort 6)

  13. Month 18 change from Baseline in fat fraction of upper leg muscles as assessed by Magnetic Resonance Imaging

    Adult participants

    Time frame: 18 months (GRAND CANYON Cohort 6)

06

Study locations

51 sites
  • Arkansas Children's Hospital
    Little Rock, Arkansas 72202, United States
  • UC San Diego
    La Jolla, California 92037, United States
  • UCLA Medical Center
    Los Angeles, California 90095, United States
  • UC Irvine Medical Center
    Orange, California 92868, United States
  • Stanford Neuroscience Health Center
    Palo Alto, California 94304, United States
  • UC Davis Medical Center
    Sacramento, California 95817, United States
  • UC Denver
    Aurora, Colorado 80045, United States
  • University of Florida
    Gainesville, Florida 32611, United States
  • Rare Disease Research
    Atlanta, Georgia 30329, United States
  • Northwestern University
    Chicago, Illinois 60611, United States
  • Indiana University School of Medicine
    Indianapolis, Indiana 46202, United States
  • University of Iowa
    Iowa City, Iowa 52242, United States
  • University of Kansas Medical Center
    Kansas City, Kansas 66160, United States
  • Kennedy Krieger Institute
    Baltimore, Maryland 21205, United States
  • University of Massachusetts Memorial Medical Center
    Worcester, Massachusetts 01605, United States
  • University of Minnesota
    Minneapolis, Minnesota 55455, United States
  • Washington University School of Medicine
    St Louis, Missouri 63110, United States
  • University of Rochester Medical Center
    Rochester, New York 14642, United States
  • Rare Disease Research, LLC NC
    Hillsborough, North Carolina 27278, United States
  • University of Cincinnati Gardner Neuroscience Institute
    Cincinnati, Ohio 45219, United States
  • Nationwide Children's Hospital
    Columbus, Ohio 43205, United States
  • University of Pennsylvania
    Philadelphia, Pennsylvania 19104, United States
  • University of Pittsburgh
    Pittsburgh, Pennsylvania 15224, United States
  • National Neuromuscular Research Institute
    Austin, Texas 78759, United States
  • Neurology Rare Disease Center
    Denton, Texas 76208, United States
  • Virginia Commonwealth University Health
    Richmond, Virginia 23298, United States
  • St Vincent's Hospital Melbourne
    Fitzroy, VIC, 3065, Australia
  • University Hospital Gent
    Ghent, Belgium 9000, Belgium
  • Universitaire Ziekenhuizen Leuven
    Leuven, Belgium
  • Centre Hospitalier Régional de la Citadelle
    Liège, 4000, Belgium
  • Rigshospitalet
    Copenhagen, Denmark
  • Centre de Reference des Maladies Neuromusculaires et de la SLA - AP-HM Hopital de La Timone
    Marseille, 13005, France
  • CHU de Nantes
    Nantes, 44093, France
  • CHU de Nice - Hopital Pasteur 2 - Centre de reference des Maladies Neuromusculaires
    Nice, 06001, France
  • AP-HP Hopital Pitie-Salpetriere
    Paris, 75013, France
  • Klinikum der Ludwig-Maximilians-Universitaet Muenchen
    Munich, 80336, Germany
  • Hadassah University Hospital
    Jerusalem, 91240, Israel
  • Schneider Children's Hospital of Israel
    Petah Tikva, 49202, Israel
  • Fondazione IRCCS Ca'Granda Ospedale Maggiore Policlinico di Milano
    Milan, 20122, Italy
  • Azienda Ospedale - Università Padova
    Padova, 35128, Italy
  • Fondazione Policlinico Universitario A. Gemelli IRCCS - Universita Cattolica del Sacro Cuore
    Rome, 00168, Italy
  • Leids Universitair Medisch Centrum
    Leiden, 2333 ZA, Netherlands
  • Optimal Clinical Trials
    Auckland, 1010, New Zealand
  • Hospital Universitario Vall d'Hebron
    Barcelona, 08035, Spain
  • Hospital Universitari de Bellvitge
    Barcelona, 08907, Spain
  • Hospital Universitario Donostia
    Donostia / San Sebastian, 20014, Spain
  • Hospital Universitari i Politecnic La Fe
    Valencia, 46026, Spain
  • University College London Hospital
    London, NW1 2PG, United Kingdom
  • St. George's University Hospitals NHS Foundation Trust
    London, SW17 0QT, United Kingdom
  • Newcastle Freeman Hospital
    Newcastle, NE7 7DN, United Kingdom
  • Salford Royal Hospital
    Salford, M68HD, United Kingdom
07

References and documents

Related links

Individual participant data

Plan to share: No

08

Registry details

Key details

Study ID
NCT05291091
Lead sponsor
Edgewise Therapeutics, Inc.
Collaborators
Medpace, Inc., ImagingNMD, SYSNAV
Responsible party
Sponsor
First posted
Mar 22, 2022
Start date
Nov 10, 2022
Primary completion
Sep 7, 2026
Completion
Sep 7, 2026
Last update
Sep 21, 2026

Study contacts

Joanne Donovan, MD, PhD
study chair · Edgewise Therapeutics, Inc.
Roxana D. Dreghici
study chair · Edgewise Therapeutics, Inc.

Oversight

Data monitoring committee
Yes
FDA-regulated drug
Yes
FDA-regulated device
No
View the source record on ClinicalTrials.gov ↗

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