Spinal muscular atrophy type I: Do the benefits of ventilation compensate for its burdens?

Abstract

Abstract:We report the progress of an 8-year-old child with spinal muscular atrophy (SMA) type 1. The parents elected in infancy that the child should be on long-term ventilation, but all attempts to establish this care at home have failed, so the child remains ventilated in the hospital. The leader of the long-term ventilation team reports on the child's progress and describes a week in the child's life. Two paediatricians argue that the benefits of long-term ventilation have not and do not compensate the child for the burdens imposed on her by this treatment and explain why they would not support the withdrawal of long-term ventilation now. They argue that long-term ventilation might have been avoided by applying to a court of law when the child was an infant. An ethicist discusses ethical aspects of decision-making in SMA type 1.

Image of the Month: Answer

“…One important factor omitted from your otherwise excellent editorial 1 and associated report 2 and review 3 of the ethics of long-term ventilation of infants with spinal muscular atrophy is that death at 1-2 years of age is not accompanied by the patient's comprehension of dying. Dying in infancy is likely to be much less distressing for the patient than when dying is accompanied by awareness later in life.…”

Will this be a life worth living

“…4,5 In a few countries, children with SMA type 1 are sometimes kept alive indefinitely using artificial ventilation. Our paper questions the ethical justification.…”

“…Our paper questions the ethical justification. 4 Although ethicists baulk at such value judgements, paediatricians feel the need to advocate for children and to ask early, 'Will this be a life worth living?' Interestingly, when we first reported disputed management of an infant with SMA type 1, 3 our intensivists declined to seek the parents' permission for publication, assuming certain refusal and further risk to relationships.…”

“…3 British Medical Journal policy forbids publication of paediatric cases without parental permission, and we debated the relative importance of public education versus confidentiality. 6 We submitted an updated report on the child to the JPCH, 4 intending to publish without parental permission. However, new evidence shows Australian children are hardly ever ventilated for SMA type 1, 5 and the reviewers and editor appropriately declined publication without permission, because the child was too readily identifiable.…”

“…The senior author who is the main carer of the patient then asked the parents' permission, which they gave unhesitatingly. 4 They trust her because of her devoted care of their child, a nice illustration of the importance of trust.…”