P 900. Decision Making Regarding Ventilator Support in Children with SMA type 1—A Cross-sectional Survey among German Physicians

Background: Spinal muscular atrophy (SMA) is a neuromuscular disorder characterized by muscle atrophy and severe proximal muscle weakness. The clinical care of patients with SMA is very heterogeneous. Particularly, it is still controversial whether ventilator support should be recommended for the critically ill infants with SMA type 1. The aim of this study was to investigate the process of decision making regarding mechanical ventilation of children with SMA type 1.

Methods: A web-based survey with 17 questions and 2 case vignettes was conducted in 671 physicians in Germany and Switzerland from December 2016 to March 2017. In this survey, we focused on factors influencing the decision whether ventilator support should be initiated and the procedure in informed consent discussions with parents. Furthermore, we asked about the general attitude toward mechanical ventilation in infants with SMA type 1. The hypothetical clinical management in emergency settings was evaluated with the case vignettes.

Results: In this study, 165 physicians participated in the survey (response rate: 24%): 49.1% neuropediatricians, 12.1% pediatric intensive care physicians, 5.5% pediatric palliative care physicians, 1.2% pediatric pulmonologists, 14.5% with more than one of these specializations, and 17.6% not specified. Most of the physicians indicated to perform detailed informed consent discussions with affected families regarding diagnosis and life-prolonging measures such as ventilator support. Many physicians reported that, after these informed consent discussions, most of the families decided for ventilator support for their affected child. In contrast, a similar fraction of physicians reported that most of the families of their patient cohort decided against ventilator support. Only 11.5% of physicians estimated that about half of the families decided either for or against ventilator support. Our results show that decisions about ventilator support depend on physicians’ experience; 59.5% of physicians caring for families who decided against ventilator support indicated that they do not have experience with children requiring ventilator support, whereas 73.8% of physicians caring for families that decided for ventilator support reported that they also have appropriate experience. Regarding physicians’ general attitude toward mechanical ventilation of children with SMA type 1, 40.5% of physicians voting against ventilator support agreed that mechanical ventilation in children with SMA type 1 is not reasonable. In contrast, only 6.6% of physicians that decided for ventilator support agreed on this statement. With respect to hypothetical clinical management in emergency settings, physicians that decided for ventilator support were more likely to start noninvasive or even invasive ventilation in children with acute respiratory failure due to respiratory infection.

Conclusion: Our results show that physicians’ attitudes and experiences about mechanical ventilation in infants with SMA type 1 vary considerably and are likely to influence the outcome in informed consent discussions. Guidelines for the assistance of affected families regarding the decision about ventilator support and the proceedings in emergency settings are necessary to improve quality of life of these patients.

Acknowledgment This work was funded by Nachwuchsakademie für Versorgungsforschung Baden-Württemberg.