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Validation of the Parent-Proxy Pediatric Charcot-Marie-Tooth Disease Quality of Life Outcome Measure
Journal article   Open access   Peer reviewed

Validation of the Parent-Proxy Pediatric Charcot-Marie-Tooth Disease Quality of Life Outcome Measure

Tong Tong Wu, Richard S Finkel, Carly E Siskind, Shawna M E Feely, Joshua Burns, Mary M Reilly, Francesco Muntoni, Timothy Estilow, Michael E Shy, Sindhu Ramchandren, …
Journal of the peripheral nervous system : JPNS, Vol.28(2), pp.237-251
06/2023
DOI: 10.1111/jns.12538
PMCID: PMC10521146
PMID: 36748295
url
https://pmc.ncbi.nlm.nih.gov/articles/PMC10521146/pdf/nihms-1895061.pdfView
Open Access

Abstract

Charcot-Marie-Tooth disease (CMT) reduces health-related quality of life (QOL) in children. We have previously developed and validated the English and Italian versions of the pediatric CMT-specific QOL outcome measure (pCMT-QOL) for children aged 8-18. There is currently no parent-proxy CMT QOL outcome measure for use in clinical trials, which could provide complementary information in these children and adolescents. This study describes the validation studies conducted to develop the parent-proxy version of the pCMT-QOL outcome measure for children aged 8-18 years old. Development and validation of the parent-proxy version of the pCMT-QOL outcome measure for children aged 8-18 years old was iterative, involving identifying relevant domains, item pool generation, prospective pilot testing and clinical assessments, structured focus-group interviews, and psychometric testing, conducted on parents of children with CMT seen at participating sites from the USA, United Kingdom, and Australia. We utilized previously described methods to develop a working parent-proxy version of the pCMT-QOL measure. From 2010-2016, the parent-proxy pCMT-QOL working version was administered to 358 parents of children with CMT aged 8-18, seen at the participating study sites of the Inherited Neuropathies Consortium. The resulting data underwent rigorous psychometric analysis, including factor analysis, test-retest reliability, internal consistency, convergent validity, IRT analysis, and longitudinal analysis, to develop the final parent-proxy version of the pCMT-QOL outcome measure for children aged 8-18 years old. The parent-proxy version of the pCMT-QOL outcome measure is a reliable, valid, and sensitive proxy measure of health-related QOL for children aged 8-18 with CMT.
Quality of life (QOL) Charcot-Marie-Tooth disease (CMT) Clinical Trial Endpoint Outcome Measure Validation Pediatric

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