© 2003 BMJ Publishing Group & Royal College of Paediatrics and Child Health
LEADING ARTICLE
Ethics
Spinal muscular atrophy–type I
Department of Anesthesia, Children’s Hospital, Harvard Medical School, Boston, MA, USA
Correspondence to:
Correspondence to:
Dr M K M Hardart, Department of Anesthesia, Farley 517/MICU office, 300 Longwood Avenue, Boston, MA 02115, USA;
katiemoyn@aol.com
The challenge of defining a child’s best interests
Keywords: spinal muscular atrophy; ethics; family decision making; mechanical ventilation; physician attitude
| The first 150 words of the full text of this article appear below. |
Spinal muscular atrophy–type I or Werdnig-Hoffman disease is an autosomal recessive disorder of childhood that causes profound weakness and death from respiratory failure, typically by the age of 2 years in the absence of mechanical ventilation.1,2 Management of this condition is highly variable—while some patients and their families are not offered any form of respiratory support, patients in other settings are routinely treated with the full spectrum of respiratory assistance and supportive care.3 Unless this striking variability in practice can be justified on the basis of factors related to individual differences between patients, it must represent either a failure to appreciate key factors about the disease or the influence of professional values and norms on the decision making process.
The spinal muscular atrophies (SMA) are a spectrum of inherited neuromuscular diseases caused by the degeneration of anterior horn cells. The SMAs are categorised into stages based on age of
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This article has been cited by other articles:
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Inwald, D
(2008). The best interests test at the end of life on PICU: a plea for a family centred approach. Arch. Dis. Child.
93: 248-250
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Bush, A, Fraser, J, Jardine, E, Paton, J, Simonds, A, Wallis, C
(2005). Respiratory management of the infant with type 1 spinal muscular atrophy. Arch. Dis. Child.
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[Abstract] [Full Text]
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