%0 Journal Article
%T Impact of Mobility Device Use on Quality of Life in Children With Friedreich Ataxia
%A Amanda Carnevale
%A Charles J. Isaacs
%A David R. Lynch
%A George R. Wilmot
%A Grace Yoon
%A J. Chad Hoyle
%A Jennifer M. Farmer
%A Judith Wilson
%A Katherine D. Mathews
%A Kristen George
%A Martin B. Delatycki
%A Resham Ejaz
%A Shiyi Chen
%A Sub H. Subramony
%A Susan L. Perlman
%A Theresa Zesiewicz
%J Journal of Child Neurology
%@ 1708-8283
%D 2018
%R 10.1177/0883073818764941
%X To determine how mobility device use impacts quality of life in children with Friedreich ataxia. 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. 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 (每19.5 points, 95% CI = 每30.00, 每8.99, P < .001) and least for the emotional subscore (每10.61 points, 95% CI = 每20.21, 每1.02, P = .03). Transition to or between mobility devices trended toward worse physical subscore (每16.20 points, 95% CI = 每32.07, 每0.33, P = .05). Mobility device use is associated with significant worsening of all domains of quality of life in children with Friedreich ataxia
%K ataxia
%K neurodegenerative disorders
%K outcomes
%K neuropathy
%K quality of life
%U https://journals.sagepub.com/doi/full/10.1177/0883073818764941