Status:

RECRUITING

A Study to Learn More About the Effects and Long-Term Safety of BIIB141 (Omaveloxolone) in Participants With Friedreich's Ataxia Aged 2 to 15 Years Old (BRAVE)

Lead Sponsor:

Biogen

Conditions:

Friedreich Ataxia

Eligibility:

All Genders

2-15 years

Phase:

PHASE3

Brief Summary

In this study, researchers will learn more about the effects and safety of BIIB141, also known as omaveloxolone or SKYCLARYS®. This drug has been approved, or made available for doctors to prescribe, ...

Detailed Description

The primary objective of Part 1 randomized controlled trial (RCT) is to evaluate the efficacy of omaveloxolone at Week 52 and the secondary objectives are to evaluate safety of omaveloxolone through W...

Eligibility Criteria

Inclusion

  • Part 1 RCT: Key inclusion criteria:
  • Diagnosed with genetically confirmed Friedreich's Ataxia (FA), i.e., homozygous for guanine-adenine-adenine (GAA) repeat expansion in intron-1 of the frataxin gene, or GAA repeat expansion in 1 allele and with point mutations or deletions, or other non-GAA expansion mutations in the other allele.
  • Symptomatic for FA as confirmed by clinician assessment. a. Children 7 to \< 16 years must also have an upright stability score (USS) score of 10 to ≤ 34 at baseline
  • Part 1 RCT: Key exclusion criteria:
  • Glycosylated hemoglobin A1C (HbA1c) \> 11%
  • B-type natriuretic peptide (BNP) \> 200 picograms per milliliter (pg/mL) at screening
  • Ejection fraction (EF) \< 40% \[based on echocardiogram (ECHO) performed at screening visit\]
  • Clinically significant cardiac disease except mild to moderate cardiomyopathy
  • Part 2 OLE: Eligibility criteria:
  • Participants have completed Part 1 RCT of the study and no discontinuation criteria have been met
  • Safety and tolerability data from Part 1 RCT are supportive of continuation in the judgement of the investigator
  • If BNP is \> 200 pg/mL at the previous visit assessment, Part 2 Day 1 should be delayed until BNP is \< 200 pg/mL.
  • If any other clinically significant laboratory abnormalities are present based on the previous visit assessments, Part 2 Day 1 should be delayed until the abnormalities are resolved.
  • In the event of intercurrent illness or other change in health status of the participant, additional Part 1 screening assessments may be repeated prior to initiation of Part 2, based on the judgement of the investigator in consultation with the medical monitor.
  • If dosing has been interrupted at the end of Part 1, Part 2 Day 1 should be delayed until resumption of study drug treatment is appropriate per Section 8.2.
  • Note: Other protocol-defined Inclusion/Exclusion criteria may apply.

Exclusion

    Key Trial Info

    Start Date :

    June 9 2025

    Trial Type :

    INTERVENTIONAL

    Allocation :

    ESTIMATED

    End Date :

    November 22 2029

    Estimated Enrollment :

    255 Patients enrolled

    Trial Details

    Trial ID

    NCT06953583

    Start Date

    June 9 2025

    End Date

    November 22 2029

    Last Update

    December 18 2025

    Active Locations (34)

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    Page 1 of 9 (34 locations)

    1

    UCLA Neurology Outpatient Clinic at Westwood

    Los Angeles, California, United States, 90095

    2

    Norman Fixel Institute for Neurological Diseases UF Health

    Gainesville, Florida, United States, 32608-3928

    3

    USF Health Morsani College of Medicine Department of Neurology

    Tampa, Florida, United States, 33612

    4

    Children's Hospital of Philadelphia - Buerger Center for Advanced Pediatric Care - PIN

    Philadelphia, Pennsylvania, United States, 19104