Oskoui Maryam, Ng Pamela, Liben Stephen, Zielinski David
Department of Pediatrics, McGill University, Montréal, QC, Canada.
Department of Neurology and Neurosurgery, McGill University, Montréal, QC, Canada.
Pediatr Pulmonol. 2017 May;52(5):662-668. doi: 10.1002/ppul.23616. Epub 2016 Sep 29.
Increasing numbers of families are requesting active supportive management for their child with spinal muscular atrophy type 1 (SMA1), leading to longer survival and greater prevalence of affected children. Strong opinions exist among physicians for and against the provision of care measures prolonging life.
To describe current practice in the care of SMA1 in Canada, and explore the factors underlying inter-physician variability.
A cross-sectional survey of Canadian hospital-based pediatric neurologists and pediatric respirologists was performed in 2015. Odds ratios and 95% confidence intervals were calculated to compare proportions between groups.
There was a 54% completion rate (99 physicians). Over half of participants believed that a disease modifying therapy was likely within 10 years. Quebec respirologists were 50 times less likely to offer long-term non-invasive ventilation (NIV) than respirologists in other provinces (OR 50.6, 95% CI 2.4-1075.3), and 20 times less likely to discuss tracheostomy with families (OR 20.4, 95% CI 2.0-211.8). High raters of perceived happiness of affected children were more likely to find NIV an acceptable measure for acute (OR 6.7, 95% CI 1.7-26.0) and chronic (OR 13.7, 95% CI 4.0-46.4) respiratory failure and prophylactic use (OR 5.8, 95% CI 2.2-15.6).
Physician knowledge, opinions, subjective perception of child happiness, and regional factors, all influence physicians' practices and the shared decision-making process. Parents may not be informed or offered all the services available to their child. Knowledge translation initiatives are needed to enhance SMA1 care. Pediatr Pulmonol. 2017;52:662-668. © 2016 Wiley Periodicals, Inc.
越来越多的家庭要求对患有1型脊髓性肌萎缩症（SMA1）的孩子进行积极的支持性管理，这使得患病儿童的存活时间延长，患病率增加。医生对于是否提供延长生命的护理措施存在强烈的不同意见。
描述加拿大目前对SMA1的护理实践，并探讨医生之间存在差异的潜在因素。
2015年对加拿大以医院为基础的儿科神经科医生和儿科呼吸科医生进行了一项横断面调查。计算比值比和95%置信区间以比较组间比例。
完成率为54%（99名医生）。超过半数的参与者认为在10年内可能会有疾病改善疗法。魁北克省的呼吸科医生提供长期无创通气（NIV）的可能性比其他省份的呼吸科医生低50倍（比值比50.6，95%置信区间2.4 - 1075.3），与家庭讨论气管切开术的可能性低20倍（比值比20.4，95%置信区间2.0 - 211.8）。认为患病儿童幸福感高的评分者更有可能认为NIV是急性（比值比6.7，95%置信区间1.7 - 26.0）和慢性（比值比13.7，95%置信区间4.0 - 46.4）呼吸衰竭以及预防性使用（比值比5.8，95%置信区间2.2 - 15.6）的可接受措施。
医生的知识、意见、对儿童幸福感的主观认知以及地区因素，都会影响医生的实践和共同决策过程。家长可能未被告知或未获得其孩子可享有的所有服务。需要开展知识转化倡议以改善SMA1的护理。《儿科肺病学》。2017年；52:662 - 668。© 2016威利期刊公司
