Highlights
- •Some groups of youth with neuromuscular disorders on degenerative disease trajectories and in different countries report lower psychosocial quality of life, but not significantly, compared with typically developing peers.
- •Challenges to identify formation and body image may compromise well-being in younger adolescents with Duchenne muscular dystrophy, but changes over time in the same population are not known.
- •Information about mental health and social participation in youth with neuromuscular disorders is currently restricted to those cared for in specialist clinics and cannot be compared with other populations.
Abstract
Objective
To investigate quality of life (QOL) and psychosocial well-being in youth with neuromuscular
disorders (NMDs) who are wheelchair users.
Data Sources
MEDLINE, Embase, CINAHL, and PsycINFO (January 2004–April 2016) and reference lists
of retrieved full-text articles.
Study Selection
Peer-reviewed studies were included when data describing self-reported QOL and psychosocial
well-being could be separately understood for those using wheelchairs and 12 to 22
years of age. There were 2058 records independently screened, and potentially eligible
articles were obtained and examined by all reviewers. Twelve observational and 3 qualitative
studies met the inclusion criteria.
Data Extraction
Population representativeness, measurement tools, and outcomes, where possible, with
comparison groups. Two reviewers independently appraised studies for risk of bias
to internal validity and generalizability.
Data Synthesis
Heterogeneity of measurement and reporting precluded meta-analysis. Data were cross-sectional
only. Compared with same-age typically developing peers, physical QOL was scored consistently
and significantly lower in youth with NMDs, whereas psychosocial QOL was not. Psychosocial
QOL was highest in youth nonambulant since early childhood and in those recruited
via single tertiary specialist clinics. Mental health and social participation could
not be compared with same-age populations.
Conclusions
Despite low physical QOL, psychosocial QOL in youth with NMDs appeared comparable
with same-age peers. The psychosocial well-being of younger adolescents on degenerative
disease trajectories appeared most compromised; however, the longitudinal effects
of growing up with a NMD on mental health and social participation are unknown. Interpretation
was hampered by poor description of participant age, sex and physical ability; lack
of population-based recruitment strategies; and inconsistent use of age-appropriate
measures. Understanding of self-reported QOL and psychosocial well-being in youth
with NMDs transitioning to adulthood is limited.
Keywords
List of abbreviations:
DMD (Duchenne muscular dystrophy), NIV (Noninvasive ventilation), NMD (Neuromuscular disorder), PedsQL (Pediatric Quality of Life Inventory), QOL (Quality of life), SF-36 (Medical Outcomes Study 36-Item Short-Form Health Survey)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: November 10, 2016
Footnotes
Supported by the Harold and Sylvia Rowell PhD Scholarship of the Muscular Dystrophy Association of Western Australia.
Disclosures: J.D. declares funding from Biogen to attend an interstate workshop on assessment for spinal muscular atrophy. The other authors have nothing to disclose.
Identification
Copyright
© 2016 by the American Congress of Rehabilitation Medicine
