Norfolk and Norwich University Hospitals NHS Foundation Trust.
University of East Anglia, Norwich, UK.
Curr Opin Support Palliat Care. 2021 Dec 1;15(4):214-218. doi: 10.1097/SPC.0000000000000570.
To summarise recent developments in the use of noninvasive ventilation (NIV) for people with amyotrophic lateral sclerosis (pwALS), with a specific focus on how multidisciplinary teams (MDT) can support best practice.
We included 13 papers, 12 with observational designs and one qualitative interview design. The review is structured using four content domains: prognosis, the effectiveness of NIV, NIV tolerance, and MDT structure and delivery. Findings suggest a modest survival benefit of NIV for pwALS. Further evidence is needed to consider the appropriate time of initiation in the context of respiratory status and the influence of NIV on cognitive function over the course of the disease. A key emerging determinant of tolerability of NIV is secretion load. Palliative care professionals within the MDT can play a key role in supporting pwALS to consider the potential benefits of NIV in the context of their preferences, prognosis, symptom burden, and ALS variant.
ALS MDTs allow timely information sharing about NIV between experts that is likely to improve outcomes. MDT practice must continually adapt to reflect pwALS's preferences for care and ensure those caring for them at home have access to support using digital innovations.
总结最近在肌萎缩侧索硬化症（pwALS）患者中使用无创通气（NIV）的新进展，特别关注多学科团队（MDT）如何支持最佳实践。
我们纳入了 13 篇论文，其中 12 篇为观察性设计，1 篇为定性访谈设计。综述采用四个内容领域进行构建：预后、NIV 的效果、NIV 耐受性以及 MDT 的结构和实施。研究结果表明，NIV 对 pwALS 患者有一定的生存获益。需要进一步的证据来考虑在呼吸状况的背景下适当的起始时间以及 NIV 对疾病过程中认知功能的影响。NIV 耐受性的一个关键新兴决定因素是分泌物负荷。MDT 中的姑息治疗专业人员可以在考虑 NIV 的潜在益处时，在患者的偏好、预后、症状负担和 ALS 变异的背景下，为 pwALS 提供关键支持。
ALS MDT 允许专家之间及时共享有关 NIV 的信息，这可能会改善预后。MDT 实践必须不断适应，以反映 pwALS 对护理的偏好，并确保在家中照顾他们的人可以通过数字创新获得支持。
