Erdmann Anke, Spoden Celia, Hirschberg Irene, Neitzke Gerald
Institute for Experimental Medicine, Medical Ethics Working Group, Kiel University, 24105 Kiel, Germany.
German Institute for Japanese Studies, Tokyo, Japan.
Palliat Care Soc Pract. 2022 Mar 15;16:26323524221083676. doi: 10.1177/26323524221083676. eCollection 2022.
Amyotrophic lateral sclerosis (ALS) leads to death on average 2-4 years after the onset of symptoms. Although many people with the disease decide in favour of life-sustaining measures, some consider hastening death. The objectives of this review are to provide an insight into the following questions: (1) How do people with amyotrophic lateral sclerosis (PALS), their families and health care professionals (HCPs) communicate about life-sustaining and life-shortening options? (2) What are the challenges for all involved in decision making and communication about this topic? To answer these questions, we searched eight databases for publications in English and German on end-of-life issues of PALS. We included texts published between 2008 and 2018, and updated our search to May 2020. Sources were analysed in MAXQDA using deductively and inductively generated codes. After the final analysis, 123 full texts were included in this review. We identified a wide range of communicative challenges and six different and, in part, opposite communication patterns: avoiding or delaying communication on end-of-life issues, openly considering dying and actively seeking assistance, ignoring or disregarding patients' wishes, discussing and respecting the patients' wishes, engaging in advance care planning and avoiding or delaying advance care planning. The literature reveals a very heterogeneous response to end-of-life issues in ALS, despite several good-practice suggestions, examples and guidelines. We derive a strong need for harmonization and quality assurance concerning communication with PALS. Avoiding or delaying communication, decision making and planning, as well as ignoring or disregarding the patient's will by HCP can be judged as a violation of the ethical principles of autonomy and non-maleficence.
肌萎缩侧索硬化症（ALS）患者通常在出现症状后的2至4年内死亡。尽管许多患者决定采取维持生命的措施，但也有一些患者考虑加速死亡。本综述的目的是深入探讨以下问题：（1）肌萎缩侧索硬化症患者（PALS）、其家人和医护人员（HCPs）如何就维持生命和缩短生命的选择进行沟通？（2）对于所有参与该主题决策和沟通的人来说，面临的挑战是什么？为了回答这些问题，我们在八个数据库中搜索了关于PALS临终问题的英文和德文出版物。我们纳入了2008年至2018年期间发表的文章，并将搜索更新至2020年5月。使用演绎和归纳生成的代码在MAXQDA中对资料来源进行了分析。经过最终分析，本综述纳入了123篇全文。我们确定了广泛的沟通挑战以及六种不同的、部分相反的沟通模式：避免或推迟关于临终问题的沟通、公开考虑死亡并积极寻求帮助、忽视或无视患者的意愿、讨论并尊重患者的意愿、参与预先护理计划以及避免或推迟预先护理计划。尽管有一些良好实践建议、示例和指南，但文献显示对于ALS临终问题的反应非常多样化。我们强烈认为需要在与PALS的沟通方面进行协调和质量保证。HCP避免或推迟沟通、决策和计划，以及忽视或无视患者的意愿可被视为违反自主和不伤害的伦理原则。
