Ethical aspects of home long term ventilation in children with neuromuscular disease

Increasing numbers of children and young adults are now receiving home ventilation. Whereas in some neuromuscular conditions the outcome and quality of life gains are clear cut; in others eg Spinal Muscular Atrophy Type I there are a few outcome studies with conflicting results and it is more difficult to balance the ethical concepts of beneficence, non-maleficence, autonomy and distributive justice. As a result there are widespread variations in the clinical management of these children. This article examines decision-making in these areas seen from the perspective of the individual, the family and society.