A Study Evaluating the Effectiveness and Safety of Risdiplam Administered in Pediatric Patients With Spinal Muscular Atrophy Who Experienced a Plateau or Decline in Function After Gene Therapy

NCT ID: NCT05861999

Last Updated: 2025-12-22

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

PHASE4

Total Enrollment

28 participants

Study Classification

INTERVENTIONAL

Study Start Date

2024-08-14

Study Completion Date

2029-03-31

Brief Summary

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This is an open-label, single-arm, multicenter clinical study to evaluate the effectiveness and safety of risdiplam administered in pediatric participants with SMA and 2 SMN2 copies who previously received onasemnogene abeparvovec and experience a plateau or decline in function. Participants to be enrolled are children \<2 years of age genetically diagnosed with SMA.

Detailed Description

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Conditions

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Muscular Atrophy, Spinal

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Risdiplam

Participants will receive risdiplam orally once daily for 72 weeks (Treatment Period). The Treatment Period will be followed by a 1-year Treatment Extension Period for a total study duration of 120 weeks (approximately 2.5 years) for each participant enrolled.

Group Type EXPERIMENTAL

Risdiplam

Intervention Type DRUG

Participants will receive risdiplam orally at the currently approved dose. The dose should be adapted for weight and age.

Interventions

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Risdiplam

Participants will receive risdiplam orally at the currently approved dose. The dose should be adapted for weight and age.

Intervention Type DRUG

Other Intervention Names

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RO7034067

Eligibility Criteria

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Inclusion Criteria

* \<2 years of age at the time of informed consent
* Confirmed diagnosis of 5q-autosomal recessive SMA, including genetic confirmation of homozygous deletion or compound heterozygosity predictive of loss of function of the Survival of Motor Neuron 1 (SMN1) gene
* Confirmed presence of two SMN2 gene copies as documented through laboratory testing
* Administration of onasemnogene abeparvovec pre-symptomatically or post-symptomatically
* Has received onasemnogene abeparvovec for SMA no less than 13 weeks prior to enrollment
* If treated with risdiplam prior to onasemnogene abeparvovec, risdiplam treatment must not have exceeded 3 weeks and must be discontinued 1 day prior to onasemnogene abeparvovec administration.
* In the opinion of the investigator, has demonstrated a plateau or decline in function post-gene therapy (with a duration of 26 weeks or less) documented by 2 individual time points in the functions as follows: swallowing AND one additional function/ability (respiratory, motor function, other) per appropriate expectation.

Exclusion Criteria

* Previous or current enrolment in investigational study prior to initiation of study treatment
* Any unresolved standard-of-care laboratory abnormalities per the onasemnogene abeparvovec prescribing information
* Concomitant or previous administration of an SMN2-targeting antisense oligonucleotide
* Concomitant or previous use of an anti-myostatin agent
* Participants requiring invasive ventilation or tracheostomy
* Presence of feeding tube and an OrSAT score of 0
* Hospitalization for pulmonary event within the last 2 months, or any planned hospitalization at the time of screening
* Any major illness requiring hospitalization within 1 month before the screening examination or any febrile illness within 1 week prior to screening and up to first dose administration.
Minimum Eligible Age

3 Months

Maximum Eligible Age

24 Months

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Hoffmann-La Roche

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Clinical Trials

Role: STUDY_DIRECTOR

Hoffmann-La Roche

Locations

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University of Arkansas for Medical Sciences

Little Rock, Arkansas, United States

Site Status RECRUITING

Valley Children's Hospital

Madera, California, United States

Site Status RECRUITING

Stanford Univ Medical Center

Palo Alto, California, United States

Site Status RECRUITING

Children's Hospital of Colorado

Aurora, Colorado, United States

Site Status RECRUITING

University of Florida Pediatrics

Gainesville, Florida, United States

Site Status RECRUITING

Children's Healthcare of Atlanta Center for Advanced Pediatrics

Atlanta, Georgia, United States

Site Status RECRUITING

Helen DeVos Children's Hospital at Spectrum Health

Grand Rapids, Michigan, United States

Site Status RECRUITING

Children'S Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status RECRUITING

University of Texas Southwestern Medical Center

Dallas, Texas, United States

Site Status RECRUITING

Children's Hospital of the King's Daughter

Norfolk, Virginia, United States

Site Status RECRUITING

Charité - Universitätsmedizin Berlin SPZ Abteilung Neuropaediatrie

Berlin, , Germany

Site Status RECRUITING

UKGM Standort Gießen

Giessen, , Germany

Site Status RECRUITING

Soroka Medical Center

Beersheba, , Israel

Site Status RECRUITING

Schneider Children's Medical Center of Israel

Petah Tikva, , Israel

Site Status RECRUITING

Sourasky MC, Dana-Dwek Children's Hospital

Tel Aviv, , Israel

Site Status RECRUITING

Uniwersyteckie Centrum Kliniczne

Gdansk, , Poland

Site Status RECRUITING

Instytut Pomnik Centrum Zdrowia Dziecka

Warsaw, , Poland

Site Status RECRUITING

Sidra Medicine

Al Rayyan, , Qatar

Site Status RECRUITING

Great Ormond Street Hospital For Children

London, , United Kingdom

Site Status RECRUITING

Countries

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United States Germany Israel Poland Qatar United Kingdom

Central Contacts

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Reference Study ID Number: BN44621 https://forpatients.roche.com/

Role: CONTACT

Phone: 888-662-6728 (U.S. Only)

Email: [email protected]

Other Identifiers

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2023-505161-81-00

Identifier Type: CTIS

Identifier Source: secondary_id

BN44621

Identifier Type: -

Identifier Source: org_study_id