Tracheotomy and children with spinal muscular atrophy type 1: ethical considerations in the French context.

Abstract

Spinal muscular atrophy (SMA) type 1 is a genetic neuromuscular disease in children that leads to degeneration of spinal cord motor neurons. This sometimes results in severe muscular paralysis requiring mechanical ventilation to sustain the child's life. The onset of SMA type 1, the most severe form of the disease, is during the first year of life. These children become severely paralysed, but retain their intellectual capacity. Ethical concerns arise when mechanical ventilation becomes necessary for survival. When professionals assess the resulting life for the child and family, they sometimes fear it will result in unreasonably excessive care. The aim of this article is to present an analysis of ethical arguments that could support or oppose the provision of invasive ventilation in this population. This examination is particularly relevant as France is one of the few countries performing tracheotomies and mechanical ventilation for this condition.

• Authors: Brigitte Rul, Franco Carnevale, Brigitte Estournet, Michèle Rudler, Christian Hervé
• Journal: Nursing ethics (Nurs Ethics) Vol. 19 Issue 3 Pg. 408-18 (May 2012) ISSN: 1477-0989 [Electronic] England
• PMID: 22323397 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Topics

• Age of Onset
• Child, Preschool
• France
• Humans
• Infant
• Infant, Newborn
• Informed Consent (ethics, legislation & jurisprudence)
• Moral Obligations
• Nursing Care (ethics)
• Patient Care Team (ethics)
• Personal Autonomy
• Practice Guidelines as Topic (standards)
• Professional-Family Relations
• Severity of Illness Index
• Spinal Muscular Atrophies of Childhood (classification, mortality, pathology, therapy)
• Tracheostomy (ethics)