Who influences parents’ decision on life support treatments for children with spinal muscular atrophy type 1?

Background: We aimed at assessing the influence of those who are part of the parents’ social network (family, friends and health professionals) when they are making decisions about nutritional and respiratory support treatments for their SMA 1 children. Methods: We prepared a 46-question questionnaire to explore the factors that influence parents’ decisions. The influence of the different persons, the degree of satisfaction with the treatment elected and the consistency between the treatment information and the child’s status were assessed by the parents using a Likert scaleof ten (a higher score for a higher influence). Results: The secondary-care physicians have the main influence on parent’s decisions about nutritional support (mean=6.4) and about respiratory support (mean=5.8). This influence is higher among decisions about Percutaneous Endoscopic Gastrostomy (mean=8.0) and among decisions about Tracheostomy Ventilation (mean=8.0). The degree of satisfaction with the chosen treatment is high (mean=7.9), and consistency between information about treatment and development of the illness also has a high score (mean=8.3). The hospital ethics committee was involved in 13.3% of the cases. Conclusions: The secondary-care physician influences parents’ decisions about nutritional and respiratory support, particularly while choosing PEG and TV. The degree of satisfaction is very high regardless of the treatment chosen. The hospital ethics committee is not usually involved in the treatment decisions for SMA1 children.