TY - JOUR
T1 - Impact of Mobility Device Use on Quality of Life in Children With Friedreich Ataxia
AU - Ejaz, Resham
AU - Chen, Shiyi
AU - Isaacs, Charles J.
AU - Carnevale, Amanda
AU - Wilson, Judith
AU - George, Kristen
AU - Delatycki, Martin B.
AU - Perlman, Susan L.
AU - Mathews, Katherine D.
AU - Wilmot, George R.
AU - Hoyle, J. Chad
AU - Subramony, Sub H.
AU - Zesiewicz, Theresa
AU - Farmer, Jennifer M.
AU - Lynch, David R.
AU - Yoon, Grace
PY - 2018/5/1
Y1 - 2018/5/1
N2 - 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.
AB - 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.
KW - ataxia
KW - neurodegenerative disorders
KW - neuropathy
KW - outcomes
KW - quality of life
UR - http://www.scopus.com/inward/record.url?scp=85045045171&partnerID=8YFLogxK
U2 - 10.1177/0883073818764941
DO - 10.1177/0883073818764941
M3 - Article
AN - SCOPUS:85045045171
VL - 33
SP - 397
EP - 404
JO - Journal of Child Neurology
JF - Journal of Child Neurology
SN - 0883-0738
IS - 6
ER -