Spinal muscular atrophy: health related quality of life and burden to parents

Objective: To analyze the relationship between the quality of life (QoL) of children and adolescents with Spinal Muscular Atrophy (SMA) and the burden on their parents. Methods: A cross-sectional study with 64 parents (53 women) of children and adolescents with SMA was conducted through an online questionnaire. Health-related QoL of children and adolescents with SMA according to the parents was evaluated using PedsQL 4.0, and Zarit Burden Interview (ZBI) was used to assess the burden on the parents. Correlation and association tests evaluated the relationship between the QoL of individuals with SMA, the burden on parents, and the factors related to these variables. Results: Parents aged between 21 and 52 years (mean 36.9 ± 7.3 years) were evaluated, of which 62.5% had a moderate burden. PedsQL 4.0 total score ranged from 19.6 to 93.5 points, and ZBI from 8 to 57 points. A negative correlation was observed between these variables (r = -0.4; p = 0.001). Furthermore, ZBI scores were related to the education level of parents (rho = 0.3; p = 0.02), changes in emotional or psychological health caused by SMA diagnosis in parents (rho = -0.4; p = 0.004), and family income (rho = 0.3; p = 0.03). Conclusion: Health-related QoL in children and adolescents with SMA was related to the burden on their parents. This highlights the importance of therapeutic programs focused on individuals with SMA, as well as the needs of their parents.