Impact of Mobility Device Use on Quality of Life in Children With Friedreich Ataxia

Resham Ejaz, Shiyi Chen, Charles J. Isaacs, Amanda Carnevale, Judith Wilson, Kristen George, Martin B. Delatycki, Susan L. Perlman, Katherine D. Mathews, George R. Wilmot, J. Chad Hoyle, Sub H. Subramony, Theresa Zesiewicz, Jennifer M. Farmer, David R. Lynch, Grace Yoon

Research output: Contribution to journalArticleResearchpeer-review

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Objective: To determine how mobility device use impacts quality of life in children with Friedreich ataxia. Study Design: Data from 111 pediatric patients with genetically confirmed Friedreich ataxia were collected from a prospective natural history study utilizing standardized clinical evaluations, including health-related quality of life using the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Module. Results: Mobility device use was associated with worse mean PedsQL total, physical, emotional, social, and academic subscores, after adjusting for gender, age of disease onset, and Friedreich Ataxia Rating Scale score. The magnitude of the difference was greatest for the physical subscore (–19.5 points, 95% CI = –30.00, –8.99, P <.001) and least for the emotional subscore (–10.61 points, 95% CI = –20.21, –1.02, P =.03). Transition to or between mobility devices trended toward worse physical subscore (–16.20 points, 95% CI = –32.07, –0.33, P =.05). Conclusions: Mobility device use is associated with significant worsening of all domains of quality of life in children with Friedreich ataxia.

Original languageEnglish
Pages (from-to)397-404
Number of pages8
JournalJournal of Child Neurology
Issue number6
Publication statusPublished - 1 May 2018
Externally publishedYes


  • ataxia
  • neurodegenerative disorders
  • neuropathy
  • outcomes
  • quality of life

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