Pulmonary, Allergy, and Critical Care Division, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Muscle Nerve. 2023 May;67(5):341-353. doi: 10.1002/mus.27779. Epub 2023 Jan 19.
Adults with amyotrophic lateral sclerosis (ALS) have been using home mechanical ventilation for over 50 years. More recently, home respiratory care has evolved to include portable home ventilators, airway clearance devices, and physiological assessments with telemonitoring capability.
National organizations currently offer incentives for providing a pulmonary care specialist within a multidisciplinary ALS clinic; however, several critical gaps exist between the available technology and employing a clinician with the necessary expertise.
Lack of formal training and poor financial incentives have led to a paucity of both clinicians and active clinical research engaging in the home respiratory care of ALS. Criteria for noninvasive ventilation (NIV) initiation are controversial, and few guidelines exist on the ideal subsequent adjustments of NIV with evolving disease. Consequently, many patients with ALS tolerate NIV poorly and must face the harrowing decision of hospice vs tracheostomy. Advancement of respiratory care in ALS has been hindered by critical gaps in pulmonologist availability, training in chronic respiratory failure, financial support, clinical research, and clarity on ventilation management beyond initiation.
Only a multifaceted approach will suffice for addressing the voids in ALS respiratory care, including various education initiatives, financial incentives, clinical research programs, and elevating the standard of respiratory care.
患有肌萎缩侧索硬化症（ALS）的成年人使用家用机械通气已有 50 多年。最近，家庭呼吸护理已发展为包括便携式家用呼吸机、气道清除设备以及具有远程监测功能的生理评估。
目前，国家组织为在多学科 ALS 诊所中提供肺部护理专家提供激励措施；然而，在可用技术和雇用具有必要专业知识的临床医生之间存在几个关键差距。
缺乏正规培训和较差的经济激励措施导致从事 ALS 家庭呼吸护理的临床医生和积极开展临床研究的人员都很匮乏。无创通气（NIV）启动的标准存在争议，并且很少有关于随着疾病的发展如何理想地调整 NIV 的指南。因此，许多 ALS 患者对 NIV 耐受性差，必须面对临终关怀与气管切开术的艰难抉择。呼吸护理在 ALS 中的进展受到肺病专家可用性、慢性呼吸衰竭培训、经济支持、临床研究以及通气管理启动之外的清晰度等方面存在关键差距的阻碍。
只有采取多方面的方法才能解决 ALS 呼吸护理方面的空白，包括各种教育计划、经济激励措施、临床研究计划以及提高呼吸护理的标准。
