Raymond Poincaré Hospital, France.
Nurs Ethics. 2012 May;19(3):408-18. doi: 10.1177/0969733011429014. Epub 2012 Feb 8.
Spinal muscular atrophy (SMA) type 1 is a genetic neuromuscular disease in children that leads to degeneration of spinal cord motor neurons. This sometimes results in severe muscular paralysis requiring mechanical ventilation to sustain the child's life. The onset of SMA type 1, the most severe form of the disease, is during the first year of life. These children become severely paralysed, but retain their intellectual capacity. Ethical concerns arise when mechanical ventilation becomes necessary for survival. When professionals assess the resulting life for the child and family, they sometimes fear it will result in unreasonably excessive care. The aim of this article is to present an analysis of ethical arguments that could support or oppose the provision of invasive ventilation in this population. This examination is particularly relevant as France is one of the few countries performing tracheotomies and mechanical ventilation for this condition.
脊髓性肌萎缩症（SMA）是一种儿童遗传性神经肌肉疾病，会导致脊髓运动神经元退化。这有时会导致严重的肌肉瘫痪，需要机械通气来维持儿童的生命。SMA 类型 1 的发病，是该疾病最严重的形式，发生在生命的第一年。这些孩子会严重瘫痪，但保留智力能力。当机械通气成为生存的必要条件时，就会出现伦理问题。当专业人员评估孩子和家庭的结果生命时，他们有时担心这会导致不合理的过度护理。本文的目的是分析支持或反对为这一人群提供侵入性通气的伦理论点。这种检查特别重要，因为法国是少数几个为这种情况进行气管切开术和机械通气的国家之一。
