title: Spinal muscular atrophy type I: do the benefits of ventilation compensate for its burdens?
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.
authors.affiliation: Children's Hospital at Westmead, Westmead, Australia.
authors.forename: Kelly
authors.initials: K
authors.lastname: Gray
authors.forename: David
authors.initials: D
authors.lastname: Isaacs
authors.forename: Henry A
authors.initials: HA
authors.lastname: Kilham
authors.forename: Bernadette
authors.initials: B
authors.lastname: Tobin
mesh_terms: D002648:Child; D003657:Decision Making / Q000941:ethics*; D005260:Female; D006801:Humans; D010290:Parents; D012121:Respiration, Artificial / Q000009:adverse effects; D014897:Spinal Muscular Atrophies of Childhood / Q000523:psychology / Q000628:therapy*; D020458:Third-Party Consent; D028761:Withholding Treatment / Q000941:ethics*
keywords: autonomy; benefit and burden; quality of life; withdrawal of treatment