Feasibility and utility of in-home body weight support harness system use in young children treated for spinal muscular atrophy: A single-arm prospective cohort study

PLoS One. 2024 Mar 19;19(3):e0300244. doi: 10.1371/journal.pone.0300244. eCollection 2024.

Abstract

Purpose: This single-arm prospective cohort study aimed to evaluate the feasibility and utility of in-home body weight support harness system (BWSS) use in children treated for spinal muscular atrophy (SMA).

Methods: Individuals with 2 or 3 copies of SMN2 who received pharmacotherapeutic treatment, had head control, and weight <50lbs were enrolled. Families were provided a BWSS and documented use. Motor outcome assessments were completed at baseline, month 3 and month 6. Families provided feedback in an end of study survey.

Results: All 32 participants (2.9 (SD 1.9) yrs), improved or remained stable on all outcomes. Average reported frequency of use was 4.1(2.3) hrs/week. Controlling for other covariates, frequency of use explained over 70% of the variability in change scores. Family feedback was overwhelmingly positive.

Conclusion: Use of in-home BWSS is a safe, feasible and useful option to increase exercise dosage after treatment in SMA and may help optimize motor abilities.

Trial registration: Study registered with: Clinicaltrials.gov Clinicaltrials.gov identifier: NCT05715749.

MeSH terms

  • Body Weight
  • Child
  • Child, Preschool
  • Exercise
  • Feasibility Studies
  • Humans
  • Muscular Atrophy, Spinal* / therapy
  • Prospective Studies
  • Spinal Muscular Atrophies of Childhood* / drug therapy

Associated data

  • ClinicalTrials.gov/NCT05715749

Grants and funding

MI received grant functing for this project from Novartis (previously Avexis) https://www.hcp.novartis.com/home/. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.