Spinal muscular atrophy type 1: is long-term mechanical ventilation ethical?
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.
Ryan
Monique M
MM
Department of Neurology, Children's Hospital at Westmead, Westmead, and University of Sydney, Sydney, Australia.
Kilham
Henry
H
Jacobe
Stephen
S
Tobin
Bernadette
B
Isaacs
David
D
D006801:Humans; D015278:Intensive Care Units, Pediatric; D009517:New South Wales; D011788:Quality of Life; D012121:Respiration, Artificial / Q000941:ethics*; D014897:Spinal Muscular Atrophies of Childhood