Spinal muscular atrophy type 1: Is long‐term mechanical ventilation ethical?

Abstract

We present a baby with spinal muscular atrophy type 1, an inherited disorder causing progressive weakness, leading to complete paralysis of respiratory, facial and limb muscles. Without intervention, death occurs in infancy due to respiratory failure. Mechanical ventilatory support can prolong life, but the child's quality of life is highly debatable. We discuss the appropriateness of initiating and continuing intensive care for this child and others in a similar position.

Medical impasse: A problem as big as Texas?

“…1,2 This case has been presented previously, and the parents and medical team have had diametrically opposed views that could rightly now be considered entrenched. 3 This is a medical impasse, but interestingly, no mention is made of consultation from a clinical ethics group. Isaacs and Kilham argue for a legal decision, accepting that the decision may not be 'right' but the decisionmaking impartial.…”

Will this be a life worth living

“…On the contrary, health-care workers have a duty of care to the child with severe life-limiting disabilities to assess the harms as well as the benefits of life-prolonging treatment. 2,3 It is not acceptable to shirk such an assessment because it is difficult. Continuing life support may commit the child to intolerable suffering with few compensations.…”

“…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. We disguised the case and wrote that we were reporting it without parental permission, an approach accepted by the previous editor-in-chief of the JPCH.…”

“…We disguised the case and wrote that we were reporting it without parental permission, an approach accepted by the previous editor-in-chief of the JPCH. 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.…”

Response to medical impasse: A problem as big as Texas?

“…1,2 This case has been presented previously, and the parents and medical team have had diametrically opposed views that could rightly now be considered entrenched. 3 This is a medical impasse, but interestingly, no mention is made of consultation from a clinical ethics group. Isaacs and Kilham argue for a legal decision, accepting that the decision may not be 'right' but the decisionmaking impartial.…”