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与 ALS/MND 患者无创通气依从性相关的因素。

Factors associated with Noninvasive ventilation compliance in patients with ALS/MND.

机构信息

University of Texas Health Science Center, San Antonio, TX, USA.

Drexel University College of Medicine, Philadelphia, PA, USA.

出版信息

Amyotroph Lateral Scler Frontotemporal Degener. 2021;22(sup1):40-47. doi: 10.1080/21678421.2021.1917617.

Abstract

: Although noninvasive ventilation (NIV) improves survival and quality of life (QOL) in ALS, use of NIV is suboptimal. To determine compliance with "early" NIV initiation, requisite for the feasibility of a large study of early NIV initiation, and examine factors impacting compliance. : Seventy-three ALS participants with forced vital capacities (FVC) >50% were enrolled. Participants with FVC over 80% (Group 1) were initiated on NIV early (FVC between 80 and 85%). Participants with FVC between 50 and 80% (Group 2) started NIV at FVC between 50 and 55%. Symptom surveys, QOL scores, and NIV compliance (machine download documenting use ≥4 hours/night >60% of time) were collected following NIV initiation. : 53.6% of Group 1 and 50% of Group 2 were compliant 28 days following NIV initiation, with increased compliance over time. Participants who were unmarried, had lower income, lower educational attainment, or limited caregiver availability were less likely to be compliant. Bothersome symptoms in non-compliant participants included facial air pressure, frequent arousals with difficulty returning to sleep, and claustrophobia. Both compliant and noncompliant participants felt improved QOL with NIV; improvement was significantly greater in compliant participants. : These data suggest ALS patients can comply with NIV early in their disease, and potentially benefit as evidenced by improved QOL scores, supporting both feasibility and need for a study comparing early versus late NIV initiation. Moreover, modifiable symptoms were identified that could be optimized to improve compliance. Further studies are needed to determine the impact of "early" intervention on survival and QOL.

摘要

尽管无创通气 (NIV) 可提高肌萎缩侧索硬化症 (ALS) 患者的生存率和生活质量 (QOL),但 NIV 的使用率并不理想。本研究旨在确定“早期”NIV 启动的依从性,这是进行早期 NIV 启动大型研究的可行性的必要条件,并探讨影响依从性的因素。

我们纳入了 73 名 FVC>50%的 ALS 参与者。FVC 超过 80%的参与者 (第 1 组) 早期开始接受 NIV (FVC 在 80%至 85%之间)。FVC 在 50%至 80%之间的参与者 (第 2 组) 在 FVC 在 50%至 55%之间开始使用 NIV。NIV 启动后收集症状调查、QOL 评分和 NIV 依从性 (记录使用时间≥4 小时/晚、≥60%时间的机器下载)。

NIV 启动后 28 天,第 1 组的依从率为 53.6%,第 2 组为 50%,且依从性随时间增加而增加。未结婚、收入较低、教育程度较低或照顾者可用性有限的参与者更不可能依从。不依从的参与者报告了一些不适症状,包括面部气压、频繁觉醒和难以重新入睡、幽闭恐惧症。依从和不依从的参与者都认为 NIV 改善了 QOL;依从性较高的参与者的改善更为显著。

这些数据表明,ALS 患者在疾病早期就可以依从 NIV,并且可能受益,因为 QOL 评分得到改善,这支持了进行早期与晚期 NIV 启动比较的研究的可行性和必要性。此外,确定了可以优化以提高依从性的可调节症状。还需要进一步的研究来确定“早期”干预对生存率和 QOL 的影响。

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