Objective: The rates of decline in respiratory measurements, including Peak Cough Flow (PCF) have not been established in Amyotrophic Lateral Sclerosis (ALS). Additionally, optimal prescription of cough adjuncts which aim to increase cough strength are unknown. The primary aim of this study was to quantify declines in respiratory function in ALS using PCF, Sniff Nasal Inspiratory Pressure (SNIP) and Slow Vital Capacity (SVC). Secondary aims were to measure respiratory morbidity, audit the characteristics of those prescribed cough adjuncts, and compare outcomes between treated and untreated cohorts. Methods: A prospective, longitudinal, observational, cohort study evaluated respiratory measures, morbidity, and physical function in ALS patients at three monthly intervals, over one year. Patient and disease characteristics of those prescribed cough adjuncts were profiled at the time of device prescription. Results: one hundred and eight participants with mean age 62.1 ± 11.5 years participated. PCF declined rapidly at a rate of 124.8L/min/year (p < 0.001). SNIP, SVC (%predicted), and ALSFRS-R also declined significantly at rates of 18.72cmH2O, 17.49%, and 9.62 units per year respectively (p < 0.001). Thirty-two (29.6%) patients reported 56 incidences of chest infection and 21 died. Patients prescribed a cough adjunct (44.4%) had significantly lower average PCF, SNIP, SVC percent predicted, and ALSFRS-R (p < 0.001). Conclusions: This study identified a rapid rate of decline in PCF, a similar decline in SNIP, and slower declines in SVC and ALSFRS-R. Cough adjunct prescription was triggered by declining respiratory measures and recommended PCF thresholds, but also by respiratory symptoms. Chest infections were common in patients regardless of cough adjunct prescription and should be closely monitored.

中文翻译：

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肌萎缩性侧索硬化症患者一年以上的呼吸测量和气道清除装置处方。

目的：在肌萎缩性侧索硬化症（ALS）中尚未确定呼吸测量的下降率，包括峰值咳嗽流量（PCF）。另外，旨在增加咳嗽强度的最佳咳嗽辅助剂是未知的。这项研究的主要目的是使用PCF，嗅鼻吸气压力（SNIP）和慢肺活量（SVC）来量化ALS的呼吸功能下降。次要目标是测量呼吸道疾病的发生率，审核那些处方咳嗽辅助剂的特征以及比较治疗组和未治疗组的结局。方法：一项前瞻性，纵向，观察性队列研究对一年以上的三个月期ALS患者的呼吸测量，发病率和身体功能进行了评估。在处方器械时，对这些处方咳嗽辅助剂的患者和疾病特征进行了概述。结果：一百零八名参与者的平均年龄为62.1±11.5岁。PCF以124.8L / min /年的速度迅速下降（p <0.001）。SNIP，SVC（预测为％）和ALSFRS-R也以每年分别为18.72cmH2O，17.49％和9.62单位的速度显着下降（p <0.001）。三十二（29.6％）例患者报告了56例胸部感染发生，其中21例死亡。开具咳嗽辅助剂的患者（44.4％）的平均PCF，SNIP，SVC预测值和ALSFRS-R均显着较低（p <0.001）。结论：本研究确定了PCF的快速下降，SNIP的类似下降以及SVC和ALSFRS-R的下降较慢。呼吸措施的下降和推荐的PCF阈值以及呼吸道症状触发了咳嗽辅助处方。无论是否使用咳嗽辅助处方，患者均很常见胸部感染，应密切监测。