The initiation of invasive long-term ventilation (I-LTV) for an adolescent with Rett Syndrome (RTT) involves many serious bioethical considerations. In moving towards a more inclusive model of patient participation, transparency surrounding the main influencing factors around this decision is important. We aimed to identify the main drivers influencing a clinician's decision to support initiation of I-LTV for an adolescent with RTT. We used an anonymous online vignette-based factorial survey. The survey was distributed internationally through eight professional multi-disciplinary organisations to reach clinicians working in paediatrics. We analysed 504 RTT vignettes completed by 246 clinicians using mixed effect regression modelling. The main three significant influencing factors identified were: parental agreement with the decision to support initiation, the family's support network, and proximity to a tertiary care centre Additional comments from participants focused on family support, and the importance of on-going communication with the family. As the rights of those with disabilities improve and participation of adolescents in decision-making becomes more established, effective communications with the family around goals of care and particular sensitivity and reflective practice around methods of consensus building will likely contribute to a positive decision-making process at this difficult time.

中文翻译：

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处于重症监护途径关键点的雷特综合征青少年：检查临床医生启动有创长期通气的决定

对患有 Rett 综合征 (RTT) 的青少年开始有创长期通气 (I-LTV) 涉及许多严肃的生物伦理考虑。在走向更具包容性的患者参与模式的过程中，围绕这一决定的主要影响因素的透明度非常重要。我们的目的是确定影响临床医生决定支持 RTT 青少年启动 I-LTV 的主要驱动因素。我们使用了一项基于匿名在线小插图的因子调查。该调查通过八个专业的多学科组织在全球范围内分发，以覆盖儿科临床医生。我们使用混合效应回归模型分析了 246 名临床医生完成的 504 个 RTT 小插图。确定的主要三个重要影响因素是：父母对支持启动决定的同意、家庭的支持网络以及是否靠近三级护理中心参与者针对家庭支持以及与家庭持续沟通的重要性的其他评论。随着残疾人权利的改善和青少年参与决策的程度越来越高，围绕护理目标与家人的有效沟通以及围绕建立共识的方法的特殊敏感性和反思实践可能会有助于积极的决策过程在这个困难的时刻。