Abstract

Increasing numbers of children and young adults are recipients of long-term ventilation at home. The decision to opt for long-term ventilation in children with Spinal Muscular Atrophy Type-1 (SMA-1) is one that polarises opinion amongst clinicians. Whilst in other neuromuscular conditions outcomes and positive impact on quality of life are evident, outcome studies and quality of life surveys that focus on SMA-1 are scarce, with variable results and consist of small numbers. As a result, effectively balancing the four ethical principles of beneficence, non-maleficence, autonomy and distributive justice becomes a bioethical conundrum. On these grounds, clinical management of these children varies internationally and is influenced by local societal norms, legal frameworks and whether healthcare is publically or privately funded. The article examines what ethical challenges paediatricians and families face in long-term ventilation decisions in children with the condition.