Hurvitz Manju, Sunkonkit Kanokkarn, Defante Andrew, Lesser Daniel, Skalsky Andrew, Orr Jeremy, Chakraborty Abhishek, Amin Reshma, Bhattacharjee Rakesh
Division of Respiratory Medicine, Department of Pediatrics, University of California San Diego, Rady Children's Hospital, San Diego, California, USA.
Division of Respiratory Medicine, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Muscle Nerve. 2023 Jul;68(1):48-56. doi: 10.1002/mus.27848. Epub 2023 May 25.
INTRODUCTION/AIMS: Non-invasive ventilation (NIV) is routinely prescribed to support the respiratory system in Duchenne muscular dystrophy (DMD) patients; however, factors improving NIV usage are unclear. We aimed to identify predictors of NIV adherence in DMD patients.
This was a multicenter retrospective analysis of DMD patients prescribed NIV and followed at (1) The Hospital for Sick Children, Canada; (2) Rady Children's Hospital San Diego, USA; and (3) University of California San Diego Health, USA, between February 2016 and October 2020. The primary and secondary outcomes were 90-day period NIV adherence and clinical and socioeconomic predictors of NIV adherence.
We identified 59 DMD patients prescribed NIV (mean ± SD age = 20.1 ± 6.7 y). Overall, percentage of nights used, and average nightly usage, were 79.9 ± 31.1% and 7.23 ± 4.12 h, respectively. Compared with children, adults had higher percentage of nights used (92.9 ± 16.9% vs. 70.4 ± 36.9%; P < .05), and average nightly usage (9.5 ± 4.7 h vs. 5.3 ± 3.7 h; P < .05). Non-English language (P = .01), and absence of deflazacort prescription (P = .02) were significantly associated with higher percentage of nights used while Hispanic ethnicity (P = .01), low household income (P = .02), and absence of deflazacort prescription (P = .02) were significantly associated with higher nightly usage. Based on univariable analysis, older age and declining forced vital capacity were associated with increased percentage of nights used and increased average nightly usage.
Certain clinical and socioeconomic determinants had a significant impact on NIV adherence in DMD patients, providing insight into those at risk for high versus low compliance with respiratory therapy.
引言/目的：无创通气（NIV）通常用于支持杜氏肌营养不良症（DMD）患者的呼吸系统；然而，改善NIV使用情况的因素尚不清楚。我们旨在确定DMD患者NIV依从性的预测因素。
这是一项对2016年2月至2020年10月期间在（1）加拿大多伦多病童医院；（2）美国圣地亚哥拉迪儿童医院；（3）美国加利福尼亚大学圣地亚哥分校医疗中心接受NIV治疗并随访的DMD患者进行的多中心回顾性分析。主要和次要结局分别为90天期间的NIV依从性以及NIV依从性的临床和社会经济预测因素。
我们确定了59例接受NIV治疗的DMD患者（平均年龄±标准差=20.1±6.7岁）。总体而言，夜间使用百分比和平均每晚使用时间分别为79.9±31.1%和7.23±4.12小时。与儿童相比，成人的夜间使用百分比更高（92.9±16.9%对70.4±36.9%；P<.05），平均每晚使用时间更长（9.5±4.7小时对5.3±3.7小时；P<.05）。非英语（P=.01）以及未开具地夫可特处方（P=.02）与更高的夜间使用百分比显著相关，而西班牙裔（P=.01）、家庭收入低（P=.02）以及未开具地夫可特处方（P=.02）与更高的每晚使用时间显著相关。基于单变量分析，年龄较大和用力肺活量下降与更高的夜间使用百分比和更长的平均每晚使用时间相关。
某些临床和社会经济决定因素对DMD患者的NIV依从性有显著影响，为了解呼吸治疗高依从性和低依从性风险人群提供了依据。
