Stander Use in Spinal Muscular Atrophy: Results from a Large Natural History Database

Elise L. Townsend*, Sarah D. Simeone, Kristin J. Krosschell, Ren Zhe Zhang, Kathryn J. Swoboda

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Purpose: The purpose of this study was to describe stander use in a natural history cohort of drug therapy-naïve children with spinal muscular atrophy (SMA) who are not walking and identify factors associated with consistent stander use. Methods: Data from 397 children with SMA types 1 and 2 characterized the prevalence and frequency of stander use. Predictors of consistent stander use explored were SMA type, survival motor neuron 2 gene (SMN2) copy number, respiratory support, and motor performance. Results: Prevalence of consistent stander use was 13% in type 1 and 68% in type 2. SMA type, SMN2 copy number, respiratory support, and head rotation control each predicted consistent stander use. Conclusions: Findings characterize stander use in children with SMA who are not walking, address important safety considerations, identify factors that may inform physical therapists' clinical decision-making related to standing program prescription, and provide guidance for future prospective studies.

Original languageEnglish (US)
Pages (from-to)235-241
Number of pages7
JournalPediatric Physical Therapy
Volume32
Issue number3
DOIs
StatePublished - Jul 1 2020

Keywords

  • Functional Motor Scale Extend
  • Modified Hammersmith Functional Motor Scale-Extend
  • Test of Infant Motor Performance Screening Items
  • adverse events
  • intervention
  • motor performance
  • respiratory support
  • spinal muscular atrophy
  • stander use
  • supported standing
  • weight bearing

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Physical Therapy, Sports Therapy and Rehabilitation

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