Long-term ventilatory support in spinal muscular atrophy

摘要: Before ethical issues regarding prolonging life in patients with degenerative disease can be considered, the quality of medical intervention must delineated. We have followed 15 spinal muscular atrophy who been treated mechanical ventilation. They received assisted ventilation for an average 8 years 10 months (range 5 to 23 months). Three required full-time ventilator assistance at time initiation ventilation; remaining 12 used nighttime 7 months. Nine continue receive only. Two died after and 14 ventilation, respectively. Of more than 18 age, three graduated from college, two are high school, completed eleventh grade. One patient is a mother healthy child. employed, others found fulfilling volunteer work. Ventilator support has not significantly interfered these patients' plans expectations.