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

doi:10.1177/0883073818764941

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 journal › Article › Research › peer-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 language	English
Pages (from-to)	397-404
Number of pages	8
Journal	Journal of Child Neurology
Volume	33
Issue number	6
DOIs	https://doi.org/10.1177/0883073818764941
Publication status	Published - 1 May 2018
Externally published	Yes

Keywords

ataxia
neurodegenerative disorders
neuropathy
outcomes
quality of life

Access to Document

10.1177/0883073818764941

Link to publication in Scopus

Cite this

Ejaz, R., Chen, S., Isaacs, C. J., Carnevale, A., Wilson, J., George, K., Delatycki, M. B., Perlman, S. L., Mathews, K. D., Wilmot, G. R., Hoyle, J. C., Subramony, S. H., Zesiewicz, T., Farmer, J. M., Lynch, D. R., & 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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title = "Impact of Mobility Device Use on Quality of Life in Children With Friedreich Ataxia",

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.",

keywords = "ataxia, neurodegenerative disorders, neuropathy, outcomes, quality of life",

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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doi = "10.1177/0883073818764941",

language = "English",

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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 journal › Article › Research › peer-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

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

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