Department of Respiratory Diseases and Intensive Care, Reference Center for Adult Rare Pulmonary Diseases, University Hospital of Dijon-Bourgogne, Dijon, France; University of Bourgogne Franche-Comté, Dijon France; Centre des Sciences du Goût et de l'Alimentation, UMR 6265 CNRS 1234 INRA, University of Bourgogne Franche-Comté, Dijon, France.
Department of Respiratory Diseases, University Hospital of Lille, Lille, France; Centre for Infection and Immunity of Lille, INSERM U1019-UMR9017, University of Lille Nord de France, Lille, France.
Respir Med Res. 2022 May;81:100901. doi: 10.1016/j.resmer.2022.100901. Epub 2022 Mar 3.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive diaphragm weakness and deteriorating lung function. Bulbar involvement and cough weakness contribute to respiratory morbidity and mortality. ALS-related respiratory failure significantly affects quality of life and is the leading cause of death. Non-invasive ventilation (NIV), which is the main recognized treatment for alleviating the symptoms of respiratory failure, prolongs survival and improves quality of life. However, the optimal timing for the initiation of NIV is still a matter of debate. NIV is a complex intervention. Multiple factors influence the efficacy of NIV and patient adherence. The aim of this work was to develop practical evidence-based advices to standardize the respiratory care of ALS patients in French tertiary care centres.
For each proposal, a French expert panel systematically searched an indexed bibliography and prepared a written literature review that was then shared and discussed. A combined draft was prepared by the chairman for further discussion. All of the proposals were unanimously approved by the expert panel.
The French expert panel updated the criteria for initiating NIV in ALS patients. The most recent criteria were established in 2005. Practical advice for NIV initiation were included and the value of each tool available for NIV monitoring was reviewed. A strategy to optimize NIV parameters was suggested. Revisions were also suggested for the use of mechanically assisted cough devices in ALS patients.
Our French expert panel proposes an evidence-based review to update the respiratory care recommendations for ALS patients in daily practice.
肌萎缩侧索硬化症（ALS）是一种神经退行性疾病，其特征为膈肌逐渐无力，肺功能逐渐恶化。延髓受累和咳嗽无力导致呼吸发病率和死亡率升高。与 ALS 相关的呼吸衰竭显著影响生活质量，是导致死亡的主要原因。非侵入性通气（NIV）是缓解呼吸衰竭症状的主要公认治疗方法，可延长生存时间并改善生活质量。然而，启动 NIV 的最佳时机仍存在争议。NIV 是一种复杂的干预措施。多种因素影响 NIV 的疗效和患者的依从性。本研究旨在为法国三级护理中心的 ALS 患者制定实用的循证护理建议，以规范其呼吸护理。
对于每项建议，法国专家小组系统地搜索了索引文献，并准备了书面文献综述，然后进行了共享和讨论。主席编写了综合草案，以便进一步讨论。所有建议均得到专家小组的一致批准。
法国专家小组更新了 ALS 患者启动 NIV 的标准。最新标准制定于 2005 年。纳入了启动 NIV 的实用建议，并审查了可用于 NIV 监测的每个工具的价值。提出了优化 NIV 参数的策略。还对 ALS 患者使用机械辅助咳嗽设备的建议进行了修订。
我们的法国专家小组提出了一项基于证据的审查，以更新日常实践中 ALS 患者的呼吸护理建议。
