title: A cohort study of children and young people with progressive neuromuscular disorders: clinical and demographic profiles and changing patterns of referral for palliative care.
abstract: BACKGROUND
  Progressive neuromuscular disease in children is life limiting and these children and young people would benefit from palliative care services, but data are limited on the number and demography of these children.
  
  
  OBJECTIVE
  To describe the clinical and demographic profile of children referred to a Children's hospice in the UK with progressive neuromuscular disease.
  
  
  METHODS
  All children and young people with progressive neuromuscular disorders referred to Martin House Children's Hospice between 1987 and 2010.
  
  
  METHODS
  Retrospective cohort study.
  
  
  RESULTS
  300 children with progressive neuromuscular disease were referred to the hospice. Seventy percent (210) of these children had Duchenne Muscular Dystrophy, 22% (67) had Spinal Muscular Atrophy (34 with Type I) and 8% had other neuromuscular diseases. Numbers of referrals have not significantly increased over the last 15 years, although an increasing number come from a South Asian background (from 4% to 32%) and a higher number of children have conditions other than Duchenne Muscular Dystrophy. A total of 55.3% (166) of all referrals came from areas of the highest deprivation. Survival patterns varied by diagnostic group, but ethnicity and deprivation were not associated with survival in these children.
  
  
  CONCLUSIONS
  The profile of children with progressive neuromuscular conditions who were referred for palliative care has changed over the last 20 years, with a different spectrum of underlying diagnoses and a greater number from a South Asian background. The higher than expected proportion of children living in areas of high deprivation has been consistent over time.
authors: Paediatric Epidemiology Group, Division of Epidemiology, University of Leeds, Leeds, UK. l.k.fraser@leeds.ac.uk
authors: Lorna K
authors: LK
authors: Fraser
authors: Anne-Marie
authors: AM
authors: Childs
authors: Michael
authors: M
authors: Miller
authors: Jan
authors: J
authors: Aldridge
authors: Sue
authors: S
authors: Manning
authors: Patricia A
authors: PA
authors: McKinney
authors: Roger C
authors: RC
authors: Parslow
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D015999:Multivariate Analysis; D020388:Muscular Dystrophy, Duchenne / Q000453:epidemiology / Q000401:mortality; D009468:Neuromuscular Diseases / Q000453:epidemiology* / Q000401:mortality; D010166:Palliative Care / Q000706:statistics & numerical data*; D011204:Poverty Areas; D015995:Prevalence; D012017:Referral and Consultation / Q000639:trends; D012189:Retrospective Studies; D014897:Spinal Muscular Atrophies of Childhood / Q000453:epidemiology / Q000401:mortality; D006113:United Kingdom / Q000453:epidemiology