Neuromuscular diseases as a group are relatively common with a prevalence of about 1 in 3000. The majority of these conditions are genetic and become clinically apparent during childhood. The likelihood of respiratory impairment varies greatly among the different conditions; with some notable exceptions, it is more likely in children with more severe global weakness. Acute respiratory failure associated with respiratory infection is the most frequent reason for unplanned hospital admission, and chronic respiratory failure is a frequent cause of death. With appropriate intervention, the incidence of unplanned hospital admission can be reduced and life expectancy can be improved. This guideline attempts to summarise the available evidence in this field and to provide recommendations that will aid the healthcare professional in delivering good quality patient care.
There are a number of excellent disease-specific guidelines and consensus statements on neuromuscular diseases, but none which focus specifically on respiratory management. Many of the principles of respiratory management are not disease specific and the objective of this guideline is to provide recommendations that can be applied to all children with neuromuscular weakness (NMW). The evidence for much of current practice is weak and is based largely on observational studies. The Guideline Committee attempted to identify and summarise the existing evidence, and when that is lacking, provide expert consensus opinion.
Hull J, Aniapravan R, Chan E, Chatwin M, Forton J, Gallagher J, Gibson N, Gordon J, Hughes I, McCulloch R, Russell RR, Simonds A. 2012, British Thoracic Society guideline for respiratory management of children with neuromuscular weakness, Thorax, Vol:67, ISSN:0040-6376, Pages:1-40.