Use of the Assessment of Caregiver Experience with Neuromuscular Disease (ACEND) in Spinal Muscular Atrophy

Laurey Brown; Katie Hoffman; Chiara Corbo-Galli; Siyuan Dong; Katelyn Zumpf; Christa Weigel; Colleen Blomgren; Hannah Munson; Jessa Bidwell; Vamshi Rao; Nancy L. Kuntz; Abigail Schwaede; Kristin J. Krosschell

doi:10.3390/jcm13040921

Use of the Assessment of Caregiver Experience with Neuromuscular Disease (ACEND) in Spinal Muscular Atrophy

Laurey Brown^*, Katie Hoffman, Chiara Corbo-Galli, Siyuan Dong, Katelyn Zumpf, Christa Weigel, Colleen Blomgren, Hannah Munson, Jessa Bidwell, Vamshi Rao, Nancy L. Kuntz, Abigail Schwaede, Kristin J. Krosschell

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

Abstract

Background: Spinal muscular atrophy (SMA) has a remarkable impact on function and participation. Subsequently, the caregivers of individuals with SMA are impacted as well. Providers and the SMA community should be aware of the presence of and likely expectations for the existence of caregiver burden. Methods: The Assessment of Caregiver Experience with Neuromuscular Disease (ACEND) quantifies caregivers’ perceptions of function and quality of life pertaining to time, finance and emotion. Analyses were conducted among SMA types and ambulatory and ventilatory status. Participants with SMA had varying ranges of function and were on pharmaceutical treatment. Total ACEND score, longitudinal change in total ACEND score, total quality of life (QOL) score, change in total QOL score and subdomains for QOL, including time, emotion and finance, were all explored. Results: Overall, the ACEND demonstrated discriminant validity and some observed trends. Total ACEND scores improved for caregivers of those with SMA 2, remained stable longitudinally for caregivers of those with SMA 1 and 3 and were not influenced by ventilation status. The caregivers of individuals with SMA 1 had the lowest total quality of life (QOL) score, as did the caregivers of non-ambulatory individuals and those requiring assisted ventilation. Longitudinally, there were no changes in total QOL between caregivers of individuals with different SMA types or ambulatory or ventilation status. There were some differences in emotional needs, but no differences in financial impact between the caregivers of individuals with different types of SMA or ambulatory and ventilatory status. Conclusions: With this information enlightening the presence of caregiver burden and expected changes in burden with pharmaceutical treatment, providers, third party payors and the SMA community at large can better assist, equip and empower those providing the necessary assistance to enable the lives of those with SMA.

Original language	English (US)
Article number	921
Journal	Journal of Clinical Medicine
Volume	13
Issue number	4
DOIs	https://doi.org/10.3390/jcm13040921
State	Published - Feb 2024

Funding

Research reported in this publication was supported, in part, by the National Institutes of Health’s National Center for Advancing Translational Sciences (Grant Number UL1TR001422). The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. This work was assisted by a grant from the Undergraduate Research Grant Program, which is administered by Northwestern University’s Office of Undergraduate Research. This work was also supported, in part, by The Mazza Foundation Neuromuscular Disorders Program, Ann and Robert H Lurie Children’s Hospital and the Department of Physical Therapy and Human Movement Sciences at Northwestern University Feinberg School of Medicine.

Keywords

ACEND
caregiver burden
neuromuscular
pediatric
quality of life
spinal muscular atrophy

ASJC Scopus subject areas

General Medicine

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10.3390/jcm13040921

Cite this

Brown, L., Hoffman, K., Corbo-Galli, C., Dong, S., Zumpf, K., Weigel, C., Blomgren, C., Munson, H., Bidwell, J., Rao, V., Kuntz, N. L., Schwaede, A., & Krosschell, K. J. (2024). Use of the Assessment of Caregiver Experience with Neuromuscular Disease (ACEND) in Spinal Muscular Atrophy. Journal of Clinical Medicine, 13(4), Article 921. https://doi.org/10.3390/jcm13040921

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title = "Use of the Assessment of Caregiver Experience with Neuromuscular Disease (ACEND) in Spinal Muscular Atrophy",

abstract = "Background: Spinal muscular atrophy (SMA) has a remarkable impact on function and participation. Subsequently, the caregivers of individuals with SMA are impacted as well. Providers and the SMA community should be aware of the presence of and likely expectations for the existence of caregiver burden. Methods: The Assessment of Caregiver Experience with Neuromuscular Disease (ACEND) quantifies caregivers{\textquoteright} perceptions of function and quality of life pertaining to time, finance and emotion. Analyses were conducted among SMA types and ambulatory and ventilatory status. Participants with SMA had varying ranges of function and were on pharmaceutical treatment. Total ACEND score, longitudinal change in total ACEND score, total quality of life (QOL) score, change in total QOL score and subdomains for QOL, including time, emotion and finance, were all explored. Results: Overall, the ACEND demonstrated discriminant validity and some observed trends. Total ACEND scores improved for caregivers of those with SMA 2, remained stable longitudinally for caregivers of those with SMA 1 and 3 and were not influenced by ventilation status. The caregivers of individuals with SMA 1 had the lowest total quality of life (QOL) score, as did the caregivers of non-ambulatory individuals and those requiring assisted ventilation. Longitudinally, there were no changes in total QOL between caregivers of individuals with different SMA types or ambulatory or ventilation status. There were some differences in emotional needs, but no differences in financial impact between the caregivers of individuals with different types of SMA or ambulatory and ventilatory status. Conclusions: With this information enlightening the presence of caregiver burden and expected changes in burden with pharmaceutical treatment, providers, third party payors and the SMA community at large can better assist, equip and empower those providing the necessary assistance to enable the lives of those with SMA.",

keywords = "ACEND, caregiver burden, neuromuscular, pediatric, quality of life, spinal muscular atrophy",

author = "Laurey Brown and Katie Hoffman and Chiara Corbo-Galli and Siyuan Dong and Katelyn Zumpf and Christa Weigel and Colleen Blomgren and Hannah Munson and Jessa Bidwell and Vamshi Rao and Kuntz, \{Nancy L.\} and Abigail Schwaede and Krosschell, \{Kristin J.\}",

note = "Publisher Copyright: {\textcopyright} 2024 by the authors.",

year = "2024",

month = feb,

doi = "10.3390/jcm13040921",

language = "English (US)",

volume = "13",

journal = "Journal of Clinical Medicine",

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T1 - Use of the Assessment of Caregiver Experience with Neuromuscular Disease (ACEND) in Spinal Muscular Atrophy

AU - Brown, Laurey

AU - Hoffman, Katie

AU - Corbo-Galli, Chiara

AU - Dong, Siyuan

AU - Zumpf, Katelyn

AU - Weigel, Christa

AU - Blomgren, Colleen

AU - Munson, Hannah

AU - Bidwell, Jessa

AU - Rao, Vamshi

AU - Kuntz, Nancy L.

AU - Schwaede, Abigail

AU - Krosschell, Kristin J.

PY - 2024/2

Y1 - 2024/2

N2 - Background: Spinal muscular atrophy (SMA) has a remarkable impact on function and participation. Subsequently, the caregivers of individuals with SMA are impacted as well. Providers and the SMA community should be aware of the presence of and likely expectations for the existence of caregiver burden. Methods: The Assessment of Caregiver Experience with Neuromuscular Disease (ACEND) quantifies caregivers’ perceptions of function and quality of life pertaining to time, finance and emotion. Analyses were conducted among SMA types and ambulatory and ventilatory status. Participants with SMA had varying ranges of function and were on pharmaceutical treatment. Total ACEND score, longitudinal change in total ACEND score, total quality of life (QOL) score, change in total QOL score and subdomains for QOL, including time, emotion and finance, were all explored. Results: Overall, the ACEND demonstrated discriminant validity and some observed trends. Total ACEND scores improved for caregivers of those with SMA 2, remained stable longitudinally for caregivers of those with SMA 1 and 3 and were not influenced by ventilation status. The caregivers of individuals with SMA 1 had the lowest total quality of life (QOL) score, as did the caregivers of non-ambulatory individuals and those requiring assisted ventilation. Longitudinally, there were no changes in total QOL between caregivers of individuals with different SMA types or ambulatory or ventilation status. There were some differences in emotional needs, but no differences in financial impact between the caregivers of individuals with different types of SMA or ambulatory and ventilatory status. Conclusions: With this information enlightening the presence of caregiver burden and expected changes in burden with pharmaceutical treatment, providers, third party payors and the SMA community at large can better assist, equip and empower those providing the necessary assistance to enable the lives of those with SMA.

AB - Background: Spinal muscular atrophy (SMA) has a remarkable impact on function and participation. Subsequently, the caregivers of individuals with SMA are impacted as well. Providers and the SMA community should be aware of the presence of and likely expectations for the existence of caregiver burden. Methods: The Assessment of Caregiver Experience with Neuromuscular Disease (ACEND) quantifies caregivers’ perceptions of function and quality of life pertaining to time, finance and emotion. Analyses were conducted among SMA types and ambulatory and ventilatory status. Participants with SMA had varying ranges of function and were on pharmaceutical treatment. Total ACEND score, longitudinal change in total ACEND score, total quality of life (QOL) score, change in total QOL score and subdomains for QOL, including time, emotion and finance, were all explored. Results: Overall, the ACEND demonstrated discriminant validity and some observed trends. Total ACEND scores improved for caregivers of those with SMA 2, remained stable longitudinally for caregivers of those with SMA 1 and 3 and were not influenced by ventilation status. The caregivers of individuals with SMA 1 had the lowest total quality of life (QOL) score, as did the caregivers of non-ambulatory individuals and those requiring assisted ventilation. Longitudinally, there were no changes in total QOL between caregivers of individuals with different SMA types or ambulatory or ventilation status. There were some differences in emotional needs, but no differences in financial impact between the caregivers of individuals with different types of SMA or ambulatory and ventilatory status. Conclusions: With this information enlightening the presence of caregiver burden and expected changes in burden with pharmaceutical treatment, providers, third party payors and the SMA community at large can better assist, equip and empower those providing the necessary assistance to enable the lives of those with SMA.

KW - ACEND

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KW - neuromuscular

KW - pediatric

KW - quality of life

KW - spinal muscular atrophy

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Use of the Assessment of Caregiver Experience with Neuromuscular Disease (ACEND) in Spinal Muscular Atrophy

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