Why the study?
This study aimed to analyze the health-related quality of life of patients with spinal muscular atrophy based on SMA type, demographic and clinical features, and compare it with matched healthy controls.
Is health-related quality of life impaired in children with spinal muscular atrophy compared to healthy matched controls?
Is health-related quality of life impaired in children with spinal muscular atrophy compared to healthy matched controls?
Children with spinal muscular atrophy have significantly lower health-related quality of life across all subscales compared to healthy matched controls, particularly in physical well-being and psychosocial aspects.
SMA children have lower HRQoL than matched peers; leaves open whether interventions improve outcomes in this population.
OBJECTIVES: This study aimed to analyze the health-related quality of life (HRQoL) of patients with spinal muscular atrophy (SMA) based on the type of SMA, demographic and clinical features and compare HRQoL of these patients with a matched healthy control group. METHODS: This was a case-control study of Patients with SMA in Iran. Sixty-six patients with SMA type II and III aged 8-18 years and also 264 healthy age, sex, and socio-economic matched individuals were enrolled. To assess the quality of life, we used the Persian version of the KIDSCREEN-27. RESULTS: The health-related quality of life between children with type II and type III SMA was not significant in all 5 subscales. However, HRQoL in healthy children was significantly higher than in SMA children in all 5 subscales. CONCLUSION: The quality of life in children with SMA was lower than the healthy control group in all subscales, and physical well-being and psychosocial aspects are the main domains of life impaired by SMA disease. However, no significant difference between the quality of life in children with SMA type II and type III was observed.
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Zamani et al. (2022) studied this question.
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