Calidad de vida en ninos y adolescentes con Atrofia Muscular Espinal
Introduction: Quality of life (QoL) is a key aspect in the treatment of patients with Spinal Muscular Atrophy (SMA) International information regarding QoL in SMA is scarce, and is not available in our country. Objective: To characterize QoL in a sample of Chilean children and adolescents with SMA. Subjects and Method: Observational, cross sectional study. A general questionnaire and the PedsQLTM 3.0 Neuromuscular Module Inventory were applied to parents of children with SMA aged 2 to 18 years. It has three areas: Disease, Communication, and Family. A score 60 was considered as good QoL, 30 60 as regular, and 30 as low. MINITAB 17(r) software was used, considering significant a p 0.05 value. Results: We recruited 38 patients, with median age 8 years (2 18) 52.63% were male, and 17 (44.7% with SMA I. All had genetic confirmation. The total score of QoL was 51.92 17, representing 31% good, 55% regular, and 14% low. Regarding SMA I, it was 46.5 15.2 and SMA II III, 56.3 17.4 (p 0.071) Concerning the area of Disease, it was 53.83 18.1, Family 48.6 23.14, and Communication 33.3 (IQR: 0.0; 83.33) In this last area, children with SMA II III, older than 6 years. with non invasive ventilatory support, or living out of the metropolitan area had higher scores, however, in multivariate analysis, only SMA type was significant, which explained 40,9% of the variation in the communication area score. Conclusions: In this sample of SMA pediatric patients, the QoL was regular or good in most of them. The lowest area was communication, with a higher score in those children with higher motor function.