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

NCT ID: NCT06953583

Last Updated: 2025-11-10

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

PHASE3

Total Enrollment

255 participants

Study Classification

INTERVENTIONAL

Study Start Date

2025-06-09

Study Completion Date

2029-11-22

Brief Summary

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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, for people with Friedreich's Ataxia (FA) who are at least 16 years old. But, it is not yet available for children and teens with FA who are younger than 16 years old. The main objective of this study is to learn how BIIB141 works in the body and about its safety in children and teens who are 2 to 15 years old.

The main questions researchers want to answer in this study are:

* How does BIIB141 affect the participants' FA symptoms balance and stability?
* How many participants have medical problems during the study?
* Are there any changes in the participants' overall health during the study?
* Are there any changes in the participants' heart health?
* Are there any changes in how the participants move through puberty? Puberty is the time in someone's life when their body changes from a child to an adult.

Researchers will also learn more about:

\- How the body processes BIIB141 in children and teens

This study will be done as follows:

* Participants will be screened to check if they can join the study. The screening period will be up to 28 days, after which participants will check into their study research center.
* There are 2 parts in this study. During Part 1, participants will take either BIIB141 or a placebo once a day.
* In Part 1, participants will take BIIB141 or the placebo in a study research center on Day 1, and then at in-person visits at Week 4, Week 12, Week 26, and Week 52. On all other days, they will take BIIB141 or the placebo at home. Part 1 lasts up to 52 weeks.
* During Part 2, participants from Part 1 will either continue taking BIIB141 or start it if they were taking the placebo. Part 2 will last up to 104 weeks.
* In Part 1, participants will have up to 10 visits to their study research center and a phone call at Week 2. In Part 2, participants will have visits at Weeks 4, 8,12, 26, and every 26 weeks after that until they leave the study, and a phone call at Week 2. There will be a final phone call to check on the participants' health 31 days after their last dose.
* Each participant will be in the study for up to about 3 years

Detailed Description

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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 Week 52 and the concentration of omaveloxolone after single and multiple dose administration. The primary objective of Part 2 open-label extension (OLE) trial is to evaluate the safety and tolerability of long-term omaveloxolone use and the secondary objective is to evaluate the efficacy of omaveloxolone following long-term use.

Conditions

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Friedreich Ataxia

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors
Part 1 of the study is placebo-controlled and Part 2 is open-label.

Study Groups

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Part 1 RCT: Omaveloxolone

Participants will receive a single oral dose of omaveloxolone once a day (QD) for up to 52 weeks in Part 1 of the study.

Group Type EXPERIMENTAL

Omaveloxolone

Intervention Type DRUG

Administered as specified in the treatment arm.

Part 1 RCT: Placebo

Participants will receive placebo, orally, QD for up to 52 weeks in Part 1 of the study.

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type DRUG

Administered as specified in the treatment arm.

Part 2 OLE: Omaveloxolone

Participants who complete Part 1 of the study and are eligible will receive a single oral dose of omaveloxolone, QD for up to 104 weeks in the Part 2 OLE study.

Group Type EXPERIMENTAL

Omaveloxolone

Intervention Type DRUG

Administered as specified in the treatment arm.

Interventions

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Omaveloxolone

Administered as specified in the treatment arm.

Intervention Type DRUG

Placebo

Administered as specified in the treatment arm.

Intervention Type DRUG

Other Intervention Names

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BIIB141, SKYCLARYS, RTA-408

Eligibility Criteria

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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 reported by the participant and/or the parent/caregiver a. Children 7 to \< 16 years must also have an upright stability score (USS) score of 10 to ≤ 34 at baseline

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

1. If alanine aminotransferase (ALT), aspartate aminotransferase (AST), and/or total bilirubin (TBL) are \> 2× upper limit of normal (ULN) at the previous visit assessment, Part 2 Day 1 should be delayed until ALT and AST are \< 1.5× ULN and TBL is \< 2× ULN
2. If BNP is \> 200 pg/mL at the previous visit assessment, Part 2 Day 1 should be delayed until BNP is \< 200 pg/mL
3. 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
4. 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
Minimum Eligible Age

2 Years

Maximum Eligible Age

15 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Biogen

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Medical Director

Role: STUDY_DIRECTOR

Biogen

Locations

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UCLA Neurology Outpatient Clinic at Westwood

Los Angeles, California, United States

Site Status NOT_YET_RECRUITING

Norman Fixel Institute for Neurological Diseases UF Health

Gainesville, Florida, United States

Site Status RECRUITING

USF Health Morsani College of Medicine Department of Neurology

Tampa, Florida, United States

Site Status RECRUITING

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

Philadelphia, Pennsylvania, United States

Site Status RECRUITING

St Jude Children's Research Hospital

Memphis, Tennessee, United States

Site Status RECRUITING

Children's Hospital of the King's Daughters

Norfolk, Virginia, United States

Site Status RECRUITING

Murdoch Childrens Research Institute (MCRI)

Parkville, Victoria, Australia

Site Status NOT_YET_RECRUITING

Universitätsklinikum Innsbruck

Innsbruck, , Austria

Site Status NOT_YET_RECRUITING

L2 Ip - Instituto de Pesquisas Clinicas Ltda - ME

Brasília, Federal District, Brazil

Site Status NOT_YET_RECRUITING

University of Campinas (UNICAMP) School of Medical Sciences

Campinas, São Paulo, Brazil

Site Status NOT_YET_RECRUITING

PSEG Centro de Pesquisa Clinica

São Paulo, , Brazil

Site Status NOT_YET_RECRUITING

McGill University

Montreal, Quebec, Canada

Site Status NOT_YET_RECRUITING

CHU de Quebec -Universite Laval

Québec, , Canada

Site Status NOT_YET_RECRUITING

Rigshospitalet - Juliane Marie Centret (JMC) Copenhagen

Copenhagen, , Denmark

Site Status NOT_YET_RECRUITING

CHU de Montpellier - Hôpital Arnaud de Villeneuve

Montpellier, Hérault, France

Site Status NOT_YET_RECRUITING

AP-HP - Hôpital Armand Trousseau

Paris, , France

Site Status NOT_YET_RECRUITING

Universitätsklinikum Aachen

Aachen, , Germany

Site Status NOT_YET_RECRUITING

UKGM - Universitätsklinikum Giessen und Marburg GmbH - Standort Gießen

Giessen, , Germany

Site Status NOT_YET_RECRUITING

UKE Hamburg

Hamburg, , Germany

Site Status NOT_YET_RECRUITING

CHI at Temple Street

Dublin, , Ireland

Site Status NOT_YET_RECRUITING

Ospedale Pediatrico Bambino Gesù IRCCS

Rome, Lazio, Italy

Site Status NOT_YET_RECRUITING

IRCCS Eugenio Medea - Polo. Scientifico Veneto

Conegliano, Veneto, Italy

Site Status NOT_YET_RECRUITING

Fondazione IRCCS Istituto Neurologico Carlo Besta

Milan, , Italy

Site Status NOT_YET_RECRUITING

Radboud Universitair Medisch Centrum

Nijmegen, , Netherlands

Site Status NOT_YET_RECRUITING

Hospital Sant Joan de Deu

Espluges de Llobregat, , Spain

Site Status NOT_YET_RECRUITING

Hospital Universitario La Paz - PPDS

Madrid, , Spain

Site Status NOT_YET_RECRUITING

Sheffield Children's Hospital

Sheffield, South Yorkshire, United Kingdom

Site Status NOT_YET_RECRUITING

University College London, Institue of Neurology

London, , United Kingdom

Site Status NOT_YET_RECRUITING

Countries

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United States Australia Austria Brazil Canada Denmark France Germany Ireland Italy Netherlands Spain United Kingdom

Central Contacts

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Patient Navigator

Role: CONTACT

Phone: 1-877-223-3576

Email: [email protected]

US Biogen Clinical Trial Center

Role: CONTACT

Phone: 866-633-4636

Email: [email protected]

Facility Contacts

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Related Links

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Other Identifiers

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2025-520896-13

Identifier Type: OTHER

Identifier Source: secondary_id

296FA301

Identifier Type: -

Identifier Source: org_study_id