Linked Life Data

18280979

Source:http://linkedlifedata.com/resource/pubmed/id/18280979

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
2008-2-18
pubmed:abstractText
The purpose of this paper is to report prolongation of survival for Werdnig-Hoffman's disease (spinal muscular atrophy type 1, SMA 1) 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 1 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 not develop the ability to speak and lose all ability to breathe from the point of the tracheotomy. In contrast, the majority of non-invasively managed SMA 1 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 1 patients, and it should be left up to the family to decide which, if either, they would like to use.
pubmed:commentsCorrections
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Mar
pubmed:issn
1526-0542
pubmed:author
pubmed:issnType
Print
pubmed:volume
9
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
45-50; quiz 50; discussion 55-6
pubmed:dateRevised
2009-10-14
pubmed:meshHeading
pubmed:year
2008
pubmed:articleTitle
The use of mechanical ventilation is appropriate in children with genetically proven spinal muscular atrophy type 1: the motion for.
pubmed:affiliation
Department of Physical Medicine and Rehabilitation, UMDNJ-New Jersey Medical School, University Hospital, Newark, NJ 07103, USA. Bachjr@umdnj.edu
pubmed:publicationType
Journal Article, Review