A SURVEY OF ATTITUDES TOWARDS RESPIRATORY SUPPORT IN SPINAL MUSCULAR ATROPHY TYPE 1 IN AUSTRALIA AND NEW ZEALAND

Objectives: Without ventilatory support, early death from chronic ventilatory insufficiency is universal in infants with spinal muscular atrophy type 1 (SMA1). With mechanical ventilation via tracheostomy, however, long-term survival in SMA1 has been reported from a number of centres in USA and Europe. We aimed to clarify physician opinions regarding the use of invasive ventilatory support in SMA1 in Australia and New Zealand.

Methods: We surveyed neurologists, respiratory physicians, clinical geneticists and intensivists from all major paediatric hospitals in Australia and New Zealand regarding their clinical practice and views on supportive management of children with SMA1.

Results: A total of 165 participants were identified. Preliminary results show that a wide array of physicians has been sampled. The majority of respondents favor progression to invasive mechanical ventilation, where required, in children with respiratory failure in the setting of acute respiratory illness. Almost all physicians, however, oppose ongoing ventilatory support for chronic respiratory failure in SMA1. Most physicians feel that decisions regarding ventilatory support should be made by parents and doctors, but that hospital Clinical Ethics Committees should be involved where there is a discordance of opinion regarding further management. Conclusion: Respiratory support in SMA1 is an important issue with significant ethical, financial and resource management implications. Most physicians in Australian and New Zealand are opposed to ongoing ventilatory support for chronic respiratory failure in this disorder. Hospital Clinical Ethics Committees have a place in counseling physicians and parents as to the best means of management of children with this incurable condition.