Clinical trial • Phase III • Neurology|Rare Disease

Omaveloxolone for Friedreich's ataxia

Phase III trial of Omaveloxolone for Friedreich's ataxia.

Overview

Trial Therapeutic Area
Neurology|Rare Disease
Trial Disease
Friedreich's ataxia
Trial Stage
Phase III
Drug Modality
Small molecule
Paediatric Trial
Yes
Orphan Drug
Yes

Key dates

Initial CTIS Submission Date
07-07-2025
First CTIS Authorization Date
28-10-2025

Trial design

Randomised, open-label, omaveloxolone (biib141) qd vs placebo (hard capsules) qd (doses not specified in provided data)-controlled Phase III trial in Spain, Austria, Denmark and others.

Randomised
Yes
Open Label
Yes
Comparator
Omaveloxolone (BIIB141) QD vs Placebo (Hard capsules) QD (doses not specified in provided data)
Target Sample Size
199

Eligibility

Recruits 199 paediatric patients.

Vulnerable Population
Children aged 2 to < 16 years (vulnerable population). Parental permission/consent and age-appropriate assent are required; multiple country- and age-specific parental ICF and assent documents are provided (examples in documents: Parental-Permission-ICF, Assent forms for ages 2-5, 6-11, 12-14, 15-16, and Optional-Future-Research/Genetic research assent/ICF documents).

Inclusion criteria

  • {"criterion_text":"- Part 1 • Aged 2 to < 16 years at the time of informed consent.\n- •Diagnosed with genetically confirmed FA, i.e., homozygous for guanine-adenine-adenine (GAA) repeat expansion inintron-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.\n- •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\n- Part 2 1. They have completed Part 1 RCT of the study and no discontinuation criteria (Section 8) have been met.\n- 2. Safety and tolerability data from Part 1 RCT are supportive of continuation in the judgement of the Investigator."}

Exclusion criteria

  • {"criterion_text":"- Key Exclusion Criteria: •Glycosylated hemoglobin A1C (HbA1c) > 11% •B-type natriuretic peptide (BNP) > 200 pg/mL •Ejection fraction (EF) < 40% (based on ECHO performed at Screening visit) •Clinically significant abnormal laboratory test results •Clinically significant cardiac disease except mild to moderate cardiomyopathy"}

Endpoints

Primary endpoints

  • {"endpoint_text":"- Part 1 Primary Efficacy Endpoint •Change from baseline in USS (mFARS, Section E)","definition_or_measurement_approach":"Change from baseline in Upright Stability Score (USS) as part of mFARS (Section E); primary efficacy evaluated at Week 52 (Part 1 objective)."}
  • {"endpoint_text":"- Secondary Efficacy Endpoints •Change from baseline in FA-HI •Change from baseline in mFARS •Change from baseline in PGI-S and CGI-S •Change from baseline in FA-ADL","definition_or_measurement_approach":"Change from baseline in listed scales/assessments (FA-HI, mFARS, PGI-S, CGI-S, FA-ADL) measured as change versus baseline (timepoints not further specified here)."}
  • {"endpoint_text":"- Part 2 •Incidence of AEs and SAEs •Change from baseline in ECHO •Change from baseline in height, weight, and BMI •C-SSRS •Tanner scale","definition_or_measurement_approach":"Safety and tolerability measures including incidence of adverse events and serious adverse events; changes from baseline in ECHO, growth measures (height, weight, BMI); use of Columbia-Suicide Severity Rating Scale (C-SSRS) and Tanner staging as safety/endpoints."}

Secondary endpoints

  • {"endpoint_text":"- Part 1 •Incidence of AEs and SAEs during the treatment period •Change from baseline in ECHO •Change from baseline in height, weight, and BMI •C-SSRS •Tanner scale •PK: Plasma concentrations of omaveloxolone by visit and timepoints","definition_or_measurement_approach":"Safety endpoints: incidence of AEs/SAEs during treatment; changes from baseline in ECHO and growth metrics; C-SSRS and Tanner scale assessments; PK measured as plasma concentrations of omaveloxolone by visit and timepoints."}
  • {"endpoint_text":"- Part 2 Secondary Efficacy Endpoints: •Change from baseline in mFARS, including USS •Change from baseline in FA-ADL •Change from baseline in FA-HI •Change from baseline in PGI-S and CGI-S","definition_or_measurement_approach":"Efficacy endpoints in the open-label extension measured as change from baseline in mFARS (including USS), FA-ADL, FA-HI, PGI-S and CGI-S."}

Recruitment

Digital Remote Recruitment
Yes
Planned Sample Size
199
Recruitment Window Months
49
Consent Approach
Parental permission/consent is required for minors; age-appropriate assent forms are provided (multiple age bands: e.g., ages 2-5, 6-11, 12-14, 15-16, and other assent templates). Country- and language-specific informed consent and parental permission forms are available (examples in English, Spanish, French, German, Italian, Dutch, Danish). eConsent/remote consent platforms are used (documents reference IQVIA eConsent materials, 'IQVIA Complete Consent' screenshots for onsite and remote).

Methods

  • Country-specific recruitment brochures and study planners (examples present for ES, AT, DK, IE, NL, DE, IT, FR) targeted to potential participants/patients and caregivers.
  • Recruitment informed-consent procedure documents (K1) for multiple countries describing site-level recruitment arrangements.
  • Email to potential participants (document: Email-to-potential-participant_DNK_Danish_Public) — Denmark.
  • GP-letter (document: K2_296FA301_GP-letter_IE_English_Public) — Ireland.
  • Patient advocacy / site patient advocacy contact lists (document: Site_Patient advocacy_Contact List for ICF_AT_Public).
  • Thank-you cards, study-planner materials and other participant-facing printed materials (country-specific K2/K3 documents).

Geography

Total Number Of Sites
14
Total Number Of Participants
127

Spain

Earliest CTIS Part Ii Submission Date
06-10-2025
Latest Decision Or Authorization Date
29-10-2025
Processing Time Days
23
Number Of Sites
2
Number Of Participants
16

Sites

Site Name
Hospital Sant Joan De Deu Barcelona
Department Name
Neurpediatric Service
Principal Investigator Name
Alejandra Darling
Principal Investigator Email
alejandra.darling@sjd.es
Contact Person Name
Alejandra Darling
Contact Person Email
alejandra.darling@sjd.es
Site Name
Hospital Universitario La Paz
Department Name
Neurpediatric Service
Principal Investigator Name
Maria del Mar Garcia Romero
Principal Investigator Email
yambee@hotmail.com
Contact Person Name
Maria del Mar Garcia Romero
Contact Person Email
yambee@hotmail.com

Austria

Earliest CTIS Part Ii Submission Date
02-10-2025
Latest Decision Or Authorization Date
03-11-2025
Processing Time Days
32
Number Of Sites
1
Number Of Participants
8

Sites

Site Name
Medizinische Universitaet Innsbruck
Department Name
Department of Neurology Frauen-Kopf-Klinik
Principal Investigator Name
Sylvia Boesch
Principal Investigator Email
sylvia.boesch@i-med.ac.at
Contact Person Name
Sylvia Boesch
Contact Person Email
sylvia.boesch@i-med.ac.at

Denmark

Earliest CTIS Part Ii Submission Date
17-10-2025
Latest Decision Or Authorization Date
28-10-2025
Processing Time Days
11
Number Of Sites
1
Number Of Participants
14

Sites

Site Name
Rigshospitalet
Department Name
Department of Pediatric Neurology, Juliane Marie Centret
Principal Investigator Name
Peter Born
Principal Investigator Email
alfred.peter.born@regionh.dk
Contact Person Name
Peter Born
Contact Person Email
alfred.peter.born@regionh.dk

Ireland

Earliest CTIS Part Ii Submission Date
14-10-2025
Latest Decision Or Authorization Date
31-10-2025
Processing Time Days
17
Number Of Sites
1
Number Of Participants
5

Sites

Site Name
Children's Health Ireland
Department Name
Neurology
Principal Investigator Name
Declan O’Rourke
Principal Investigator Email
declan.orourke@cuh.ie
Contact Person Name
Declan O’Rourke
Contact Person Email
declan.orourke@cuh.ie

Netherlands

Earliest CTIS Part Ii Submission Date
03-10-2025
Latest Decision Or Authorization Date
06-11-2025
Processing Time Days
34
Number Of Sites
1
Number Of Participants
5

Sites

Site Name
Radboud universitair medisch centrum Stichting
Department Name
Department of Pediatrics
Principal Investigator Name
Nienke Van Os
Principal Investigator Email
nienke.vanos@radboudumc.nl
Contact Person Name
Nienke Van Os
Contact Person Email
nienke.vanos@radboudumc.nl

Germany

Earliest CTIS Part Ii Submission Date
15-10-2025
Latest Decision Or Authorization Date
29-10-2025
Processing Time Days
14
Number Of Sites
3
Number Of Participants
23

Sites

Site Name
University Medical Center Hamburg-Eppendorf
Department Name
Klinik und Poliklinik für Kinder- und Jugendmedizin
Principal Investigator Name
Deike Weiss
Principal Investigator Email
d.weiss@uke.de
Contact Person Name
Deike Weiss
Contact Person Email
d.weiss@uke.de
Site Name
Universitaetsklinikum Aachen AöR
Department Name
Klinik für Neurologie Sektion Translationale Neurodegeneration
Principal Investigator Name
Kathrin Reetz
Principal Investigator Email
kreetz@ukaachen.de
Contact Person Name
Kathrin Reetz
Contact Person Email
kreetz@ukaachen.de
Site Name
Justus-Liebig-Universitaet Giessen
Department Name
Zentrum für Kinderheilkunde und Jugendmedizin Abteilung für Pädiatrische Neurologie
Principal Investigator Name
Andreas Hahn
Principal Investigator Email
Andreas.Hahn@paediat.med.uni-giessen.de
Contact Person Name
Andreas Hahn

Italy

Earliest CTIS Part Ii Submission Date
03-10-2025
Latest Decision Or Authorization Date
30-10-2025
Processing Time Days
27
Number Of Sites
3
Number Of Participants
26

Sites

Site Name
IRCCS Foundation Istituto Neurologico Carlo Besta
Department Name
Dipartimento Neuroscienze Pediatriche SC Neuropsichiatria Infantile – Disordini del Movimento (NPI)
Principal Investigator Name
Isabella Moroni
Principal Investigator Email
isabella.moroni@istituto-besta.it
Contact Person Name
Isabella Moroni
Site Name
Ospedale Pediatrico Bambino Gesu
Department Name
Neurorehabilitation Unit
Principal Investigator Name
Gessica Vasco
Principal Investigator Email
gessica.vasco@opbg.net
Contact Person Name
Gessica Vasco
Contact Person Email
gessica.vasco@opbg.net
Site Name
Associazione La Nostra Famiglia
Department Name
UOC Riabilitazione Neuromotoria
Principal Investigator Name
Gabriella Paparella
Principal Investigator Email
gabriella.paparella@lanostrafamiglia.it
Contact Person Name
Gabriella Paparella

France

Earliest CTIS Part Ii Submission Date
17-10-2025
Latest Decision Or Authorization Date
03-11-2025
Processing Time Days
17
Number Of Sites
2
Number Of Participants
30

Sites

Site Name
Assistance Publique Hopitaux De Paris
Department Name
Service de Neuropédiatrie
Principal Investigator Name
Florence RENALDO
Principal Investigator Email
florence.renaldo@aphp.fr
Contact Person Name
Florence RENALDO
Contact Person Email
florence.renaldo@aphp.fr
Site Name
Centre Hospitalier Universitaire De Montpellier
Department Name
Service de Neurologie Pédiatrique
Principal Investigator Name
Agathe ROUBERTIE
Principal Investigator Email
a-roubertie@chu-montpellier.fr
Contact Person Name
Agathe ROUBERTIE
Contact Person Email
a-roubertie@chu-montpellier.fr

Sponsor

Primary sponsor

Full Name
Biogen Idec Research Limited
Organisation Type
Pharmaceutical company
Country Of Registered Address
United Kingdom

Contract research organisations

Name
IQVIA Limited
Responsibilities
eConsent, site payments and Drug Dev.
Name
PPD Global Central Labs
Responsibilities
Central Labs
Name
PPD Development LP
Responsibilities
Patient Cards, Patient services
Name
Suvoda LLC
Name
Medidata Solutions Inc.

Third parties

  • {"country":"United States","full_name":"Small Planet","duties_or_roles":"Investigator Meeting Planner","organisation_type":"Health care"}
  • {"country":"United States","full_name":"Suvoda LLC","duties_or_roles":"","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"United Kingdom","full_name":"IQVIA Limited","duties_or_roles":"eConsent, site payments and Drug Dev.","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Praxis Communications LLC","duties_or_roles":"Development of Patient Recruitment Materials","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"Belgium","full_name":"PPD Global Central Labs","duties_or_roles":"Central Labs","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Q2 Solutions LLC","duties_or_roles":"","organisation_type":"Laboratory/Research/Testing facility"}
  • {"country":"United States","full_name":"PPD Development LP","duties_or_roles":"Patient Cards, Patient services","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Cyberchrome Inc.","duties_or_roles":"Patient Retention- Printing and Shipping of Patient Recruitment Materials","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"Ireland","full_name":"Accellacare Limited","duties_or_roles":"Home Health","organisation_type":"Pharmaceutical company"}
  • {"country":"Ireland","full_name":"Teckro Limited","duties_or_roles":"Site Technical Portal - Site Communication and Training","organisation_type":"Pharmaceutical company"}
  • {"country":"Germany","full_name":"Azenta Germany GmbH","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Medidata Solutions Inc.","duties_or_roles":"","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"United States","full_name":"WCG Clinical Inc.","duties_or_roles":"eCOA/ePRO","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Greenphire LLC","duties_or_roles":"Patient Travel","organisation_type":"Non-Pharmaceutical company"}

Investigational products

Investigational Product Name
BIIB141
Active Substance
Omaveloxolone
Modality
Small molecule
Routes Of Administration
ORAL
Route
ORAL
Orphan Designation
Yes
Frequency
QD
Investigational Product Name
Hard capsules (Placebo)
Modality
Other
Frequency
QD

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