Long-term Follow-up Study of Patients With Spinal Muscular Atrophy Receiving Risdiplam Treatment

Who is this study for? Patients with Spinal Muscular Atrophy
What treatments are being studied? Risdiplam
Status: Active_not_recruiting
Location: See all (39) locations...
Intervention Type: Drug, Device
Study Type: Interventional
Study Phase: Phase 4
SUMMARY

A multi-center, longitudinal, prospective, non-comparative study to investigate the long-term safety and effectiveness of risdiplam, prescribed based on clinician judgment as per the Evrysdi® U.S. Package Insert (USPI) in adult and pediatric participants with SMA. In this study, participants will be followed for the duration of the study or until withdrawal of consent, loss to follow-up, or death. Participants who discontinue risdiplam may still remain in the study, if they agree to continue participating in the follow-up assessments. An optional sub study will assess the feasibility, acceptability, and adherence of remote assessment of motor and bulbar functions in participants with SMA using wearable and smartphone-based biosensors. Approximately 39 participants from the main study are planned to be enrolled in the sub study.

Eligibility
Participation Requirements
Sex: All
Healthy Volunteers: f
View:

• Clinical diagnosis of SMA

• Prescribed or continued risdiplam based on clinical judgment of prescriber, as per the Evrysdi® USPI, after U.S. FDA approval (07 August 2020)

⁃ Sub study:

⁃ Participants in the main study (ML43702) are eligible to be included in the sub study only if all of the following criteria apply:

• Age ≥ 10 years at the time of signing Informed Consent Form

• Willingness and ability to use smartphone technology

• Fluency in English (written and spoken as per the judgment of the investigator)

• Willingness and ability to complete all aspects of the sub study, including respiration and swallowing measurements using respiratory inductance plethysmography (RIP) belts and surface electromyography (sEMGs)

• Hammersmith Functional Motor Scale-Expanded (HFMSE) \> 10

• Functional Oral Intake Scale (FOIS) \>1

• Willingness to be video recorded during in-clinic SMA-DAT tasks and ADAM sensor assessments and training

• Availability of a caregiver who is willing to participate throughout this sub study

Locations
United States
Arkansas
University of Arkansas for Medical Sciences
Little Rock
Arizona
Barrow Neurological Institute
Phoenix
Phoenix Children's Hospital
Phoenix
California
Loma Linda University Health Care
Loma Linda
Children's Hospital Los Angeles
Los Angeles
Valley Children's Hospital
Madera
University California - Irvine
Orange
University of California Davis Medical Center
Sacramento
Colorado
University of Colorado
Aurora
Washington, D.c.
Children's National Hospital
Washington D.c.
Medstar Georgetown University Hospital
Washington D.c.
Florida
Nemour's Children's Hospital, Florida
Orlando
All Children's Research Institute, Inc.
St. Petersburg
Advent Health Orlando
Winter Park
Georgia
Rare Disease Research, LLC
Atlanta
Kansas
University of Kansas Medical Center
Kansas City
Kentucky
University of Kentucky Medical Center
Lexington
Norton Children's Hospital
Louisville
Massachusetts
Massachusetts General Hospital
Boston
Michigan
University of Michigan Pediatric Rehabilitation Center
Ann Arbor
Helen DeVos Children's Hospital at Spectrum Health
Grand Rapids
Minnesota
Gillette Children's Specialty Healthcare
Minnetonka
Mayo Clinic
Rochester
Missouri
Washington University;Wash Uni. Sch. Of Med
St Louis
New York
Columbia University Med Center
New York
NYU Hospital for Joint Diseases
New York
Ohio
Cincinnati Childrens Hospital
Cincinnati
Pennsylvania
Penn State Milton S. Hershey Medical Center
Hershey
Childrens Hospital of Philadelphia
Philadelphia
Texas
University of Texas at Austin Health sciences, Dell Medical School
Austin
Neurology & Neuromuscular Care Center
Denton
Central Texas Neurology Consultants
Round Rock
Methodist Children's Hospital of South Texas
San Antonio
Utah
University Of Utah
Salt Lake City
Virginia
University of Virginia Children?s Hospital
Charlottesville
Washington
MultiCare Health System Institute for Research and Innovation
Tacoma
Wisconsin
Childrens Hospital of Wisconsin
Milwaukee
West Virginia
UBC (Remote Coordinating Center, no physical facility)
Morgantown
Other Locations
Puerto Rico
Instituto de Rehabilitación del Caribe
Santurce
Time Frame
Start Date: 2022-03-29
Completion Date: 2026-12-31
Participants
Target number of participants: 402
Treatments
Experimental: Risdiplam
Participants who are taking risdiplam prescribed based on clinician judgment, as per the Evrysdi® USPI are enrolled in this arm.
Experimental: Substudy Cohort
Participants from the main study will be enrolled in three sub-groups based on their Functional Oral Intake Scale (FOIS) score. Participants will be provided with a preconfigured study smartphone with the spinal muscular atrophy digital assessment tool (SMA-DAT) application and an ADvanced Acousto-Mechanic (ADAM) sensor. Participants will be asked to perform a programmed selection of SMA-DAT tasks for 24 days of the 27-day remote monitoring period.
Sponsors
Leads: Genentech, Inc.

This content was sourced from clinicaltrials.gov