Characterization and survival of patients with spinal muscle atrophy type 1 on home mechanical ventilation program of ministry of health, Chile

Introduction: Spinal muscular atrophy type 1(SMA1) is the most frequent genetic cause of mortality in infants. Without ventilatory assistance, the life expectancy does not reach two years. Providing noninvasive/invasive ventilatory support is a controversial issue. In any case, they have clinical stability, make it possible for them to be moved and managed at home, where patients and their families benefit. Our objective was to describe and characterize the patients with SMA1 admitted in the home mechanical ventilation program in Chile (HMVP), and its economic implications. Methods: A retrospective study was designed. Patients with SMA1 with invasive and non-invasive ventilation during the period 2006-2016 in the HMVP were included. We describe age, gender, the age of entry, evaluating the decrease in the care costs, length of stay and mortality of all patients with SMA1. Results: Since 2008, 36 children have been admitted, 34 with genetic confirmation of their diagnosis, all patients ventilated 24 hours/day, 31 in invasive ventilation. During the period 8 patients died, all due to respiratory failure, 3 in non-invasive ventilation modality. 13 was women, 19 lived in Santiago (capital of Chile). Mean age of admission was 2.0 years(range 0.5-10.9 years) and permanence time of 3.1 years(range 0.1-9.0 years). All invasive patients have at their home paramedic for 12 hours provided by HMVP. Mean age of survival was 11.9 years(95% CI9.5-14.2) EE: 1.2. The costs for patients admitted to HMVP decrease on 76,5% vs. intensive care units. Conclusion: There is important clinical stability, decrease in the resources used and an increase in survival.