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

Abstract

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
Volume33
Issue number6
DOIs
Publication statusPublished - 1 May 2018
Externally publishedYes

Keywords

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

Cite this

Ejaz, R., Chen, S., Isaacs, C. J., Carnevale, A., Wilson, J., George, K., ... Yoon, G. (2018). Impact of Mobility Device Use on Quality of Life in Children With Friedreich Ataxia. Journal of Child Neurology, 33(6), 397-404. https://doi.org/10.1177/0883073818764941
Ejaz, Resham ; Chen, Shiyi ; Isaacs, Charles J. ; Carnevale, Amanda ; Wilson, Judith ; George, Kristen ; Delatycki, Martin B. ; Perlman, Susan L. ; Mathews, Katherine D. ; Wilmot, George R. ; Hoyle, J. Chad ; Subramony, Sub H. ; Zesiewicz, Theresa ; Farmer, Jennifer M. ; Lynch, David R. ; Yoon, Grace. / Impact of Mobility Device Use on Quality of Life in Children With Friedreich Ataxia. In: Journal of Child Neurology. 2018 ; Vol. 33, No. 6. pp. 397-404.
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abstract = "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.",
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author = "Resham Ejaz and Shiyi Chen and Isaacs, {Charles J.} and Amanda Carnevale and Judith Wilson and Kristen George and Delatycki, {Martin B.} and Perlman, {Susan L.} and Mathews, {Katherine D.} and Wilmot, {George R.} and Hoyle, {J. Chad} and Subramony, {Sub H.} and Theresa Zesiewicz and Farmer, {Jennifer M.} and Lynch, {David R.} and Grace Yoon",
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Ejaz, R, Chen, S, Isaacs, CJ, Carnevale, A, Wilson, J, George, K, Delatycki, MB, Perlman, SL, Mathews, KD, Wilmot, GR, Hoyle, JC, Subramony, SH, Zesiewicz, T, Farmer, JM, Lynch, DR & Yoon, G 2018, 'Impact of Mobility Device Use on Quality of Life in Children With Friedreich Ataxia' Journal of Child Neurology, vol. 33, no. 6, pp. 397-404. https://doi.org/10.1177/0883073818764941

Impact of Mobility Device Use on Quality of Life in Children With Friedreich Ataxia. / Ejaz, Resham; Chen, Shiyi; Isaacs, Charles J.; Carnevale, Amanda; Wilson, Judith; George, Kristen; Delatycki, Martin B.; Perlman, Susan L.; Mathews, Katherine D.; Wilmot, George R.; Hoyle, J. Chad; Subramony, Sub H.; Zesiewicz, Theresa; Farmer, Jennifer M.; Lynch, David R.; Yoon, Grace.

In: Journal of Child Neurology, Vol. 33, No. 6, 01.05.2018, p. 397-404.

Research output: Contribution to journalArticleResearchpeer-review

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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

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KW - neurodegenerative disorders

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