[P3-107] Noninvasive Ventilation in 3 Children with Spinal Muscular Atrophy Type 1
Patients with untreated spinal muscular atrophy (SMA) type 1 inevitably die from respiratory failure before the age of 2 years. The choice of treatment includes letting nature take its course, tracheostomy and invasive mechanical ventilation, and noninvasive positive pressure ventilation (NPPV). Recent studies have shown an upward trend of the choice of NPPV. In Japan, however, few reports have been made on contriving management of NPPV for patients with SMA1. We herein report three patients with SMA1 (4-month-old male and 6-month-old female twins) for whom we applied NPPV combined with airway clearance techniques such as oral continuous suction and mechanical insufflation- exsufflation. We customized the interface masks to achieve maximum fitting to their faces. Two patients were initially difficult to be applied NPPV as they were unable to understand and cooperate with the treatment. However, short daytime induction of the NPPV interface in continuous positive airway pressure mode, and gradual increase of the condition eventually resulted in successful full time application of NPPV. We initially used low inspiratory positive airway pressure (IPAP), and increased IPAP according to their respiratory condition. Although our noninvasive ventilation management shares many common ways with published reports, individualized contrivance coupled with conventional protocols may be useful for better respiratory support for patients with SMA1.