Kampelmacher M J, Westermann E J, van den Berg L H, Jennekens F G, van Kesteren R G
Centrum voor Thuisbeademing, Universitair Medisch Centrum Utrecht, Postbus 85.500, 3508 GA Utrecht.
Ned Tijdschr Geneeskd. 2004 Mar 13;148(11):509-13.
In two patients (men aged 64 and 62 years, respectively) with amyotrophic lateral sclerosis (ALS) tracheostomy ventilation was initiated following (imminent) acute respiratory failure; in one patient this was done because advance directives were lacking, while in the other non-invasive ventilation (NIPPV) was no longer an option. A third ALS patient, a woman aged 36 years, already had chronic respiratory failure when she presented at the local centre for home mechanical ventilation. As a result, the placement of a percutaneous endoscopic gastrostomy (PEG) was impossible. Instead, she had to use a nasopharyngeal feeding tube, which diminished the effectiveness of her NIPPV. The fourth patient, a man aged 60 years, was referred in good time and hence had sufficient time to consider domiciliary ventilation. Following the placement of a PEG he started NIPPV electively. In patients with ALS, domiciliary ventilation should be discussed early in the course of the disease. Advance directives with regard to domiciliary ventilation are important, not only to avoid undesired (invasive) ventilation, but also with respect to the placement of a PEG and the appropriate use of oxygen and morphine.
两名肌萎缩侧索硬化症（ALS）患者（分别为64岁和62岁男性）在急性呼吸衰竭（即将发生）后开始进行气管切开通气；其中一名患者是因为缺乏预先指示而进行了气管切开通气，而另一名患者则是因为无创通气（NIPPV）已不再是一种选择。第三名ALS患者是一名36岁女性，在当地家庭机械通气中心就诊时已经患有慢性呼吸衰竭。因此，无法进行经皮内镜下胃造口术（PEG）。相反，她不得不使用鼻饲管，这降低了她无创通气的效果。第四名患者是一名60岁男性，得到了及时转诊，因此有足够的时间考虑家庭通气。在置入PEG后，他选择性地开始了无创通气。对于ALS患者，应在疾病早期就讨论家庭通气问题。关于家庭通气的预先指示很重要，这不仅是为了避免不必要的（侵入性）通气，而且对于PEG的置入以及氧气和吗啡的合理使用也很重要。
