Bach J, Alba A, Pilkington L A, Lee M
Arch Phys Med Rehabil. 1981 Jul;62(7):328-31.
The most common cause of death in patients with rapidly progressive neuromuscular disease has been respiratory failure. The medical community and society as a whole have not yet recognized the possibilities for markedly extended life expectancy and prolonged productivity in severely disabled neuromuscular patients with proper medical management and rehabilitation. In a population of 120 patients with early childhood onset, rapidly progressive muscular dystrophy, 29 were in the most advanced stage requiring mechanical ventilation 24 hr/day and had vital capacities of less than 10% predicted for age and height. Of these patients with severely weakened bulbar innervated muscles, 10 have been receiving positive pressure ventilation via indwelling tracheostomy tubes for an average of 3 years. The average age of the 29 patients is 27 years with a range of 15 to 54 years. Of the 29 patients, 24 live in the community. Three are married and have a total of 5 children. Some patients are in professional careers, or active in political and community organizations. The management of and potential for improved quality of life and increased longevity in these patients are discussed.
快速进展性神经肌肉疾病患者最常见的死因是呼吸衰竭。医学界乃至整个社会尚未认识到，通过适当的医疗管理和康复，严重残疾的神经肌肉疾病患者的预期寿命能够显著延长，生产能力也能得到延长。在120例儿童期起病、快速进展性肌营养不良的患者群体中，29例处于最晚期，需要每天24小时进行机械通气，其肺活量低于根据年龄和身高预测值的10%。在这些延髓支配肌肉严重无力的患者中，有10例通过留置气管造口管接受正压通气，平均时长为3年。这29例患者的平均年龄为27岁，年龄范围在15至54岁之间。在这29例患者中，24例生活在社区。3例已婚，育有5个孩子。一些患者从事专业工作，或活跃于政治和社区组织。本文讨论了这些患者的管理方法以及改善生活质量和延长寿命的潜力。
