Journal
PAEDIATRIC RESPIRATORY REVIEWS
Volume 9, Issue 1, Pages 45-50Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.prrv.2007.11.003
Keywords
spinal muscular atrophy; Werdnig-Hoffmann's disease; neuromuscular disease; respiratory management; mechanically assisted coughing; noninvasive mechanical ventilation; CoughAssist (TM)
Categories
Ask authors/readers for more resources
The purpose of this paper is to report prolongation of survival for Werdnig-Hoffman's disease (spinal muscular atrophy type I, SMA I) by use of non-invasive respiratory muscle aids compared with tracheostomy, and to present reasons for offering this as an option to the parents of these children. Ninety per cent of typical untreated SMA I patients die before 12 months of age and 100% by 24 months of age. Tracheostomy can prolong survival to over 20 years of age in some cases, but patients with tubes do notclevelopthe ability to speak and lose all abilityto breathe from the point of the tracheotomy. In contrast, the majority of non-invasively managed SMA I patients develop the ability to communicate verbally and maintain some autonomous breathing ability. Clinicians' treatment paradigms associate ventilatory support with invasive tubes and do not recognise aiding respiratory muscles. Clinicians also significantly underestimate the care providers' view of the patient's quality of life. As a result, they rarely offer non-invasive means to prolong life. In conclusion, both non-invasive aids and tracheostomy can prolong survival for SMA I patients, and it should be left up to the family to decide which, if either, they would like to use. (c) 2007 Elsevier Ltd. All rights reserved.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available