Adolescents with Rett syndrome at critical care pathway junctures: Examining clinicians’ decision to initiate invasive long-term ventilation

Background 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. Objective We aimed to identify the main drivers influencing a clinician's decision to support initiation of I-LTV for an adolescent with RTT. Method 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. Results 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. Conclusion 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 practise around methods of consensus building will likely contribute to a positive decision-making process at this difficult time.