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. 2020 Jul;32(3):235-241.
doi: 10.1097/PEP.0000000000000713.

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

Elise L Townsend  1 , Sarah D Simeone, Kristin J Krosschell, Ren Zhe Zhang, Kathryn J Swoboda; Project Cure SMA Investigator's Network
Affiliations

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

Elise L Townsend et al. Pediatr Phys Ther. 2020 Jul.

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.

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References

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