title: Palliative care in children with spinal muscular atrophy type I: What do they need?
abstract: OBJECTIVE
  Our aim was to describe the clinical evolution and needs of children with spinal muscular atrophy type I treated in a domiciliary palliative care program.
  
  
  METHODS
  We undertook a retrospective chart review of nine consecutive patients. Descriptions of the clinical and demographic profile of children with spinal muscular atrophy (SMA) type I were referred to a pediatric palliative care team (PPCT).
  
  
  RESULTS
  Six males and three females were admitted to the PPCT, all before six months of age, except for one afflicted with SMA type I with respiratory distress. The median time of attention was 57 days (range 1-150). The domiciliary attention mainly consisted of respiratory care. The patient with SMA type I with respiratory distress required domiciliary mechanical ventilation by tracheotomy. In all cases, a nasogastric tube (NT) was indicated. As end-of-life care, eight required morphine to manage the dyspnea, four received it only by enteral (oral or NT) administration, and four received it first by enteral administration with continuous subcutaneous infusion (CSI) later. Three of the four patients with CSI also received benzodiazepines. While they were attended by the PPCT, none required hospital admission. All the patients died at home except for the one attended to for just one day.
  
  
  CONCLUSIONS
  Domiciliary care for these patients is possible. The respiratory morbidity and its management are the main issues. Application of an NT is useful to maintain nutritional balance. Morphine administration is necessary to manage the dyspnea. Palliative sedation is not always necessary.
authors.affiliation: Pediatric Critical Care Unit and Pediatric Palliative Care Unit,Hospital Infantil Universitario Niño Jesús,Madrid,Spain.
authors.forename: Alberto
authors.initials: A
authors.lastname: García-Salido
authors.affiliation: Pediatric Palliative Care Unit,Hospital Infantil Universitario Niño Jesús.Madrid,Spain.
authors.forename: María García
authors.initials: MG
authors.lastname: de Paso-Mora
authors.affiliation: Pediatric Palliative Care Unit,Hospital Infantil Universitario Niño Jesús.Madrid,Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Monleón-Luque
authors.affiliation: Pediatric Palliative Care Unit,Hospital Infantil Universitario Niño Jesús.Madrid,Spain.
authors.forename: Ricardo
authors.initials: R
authors.lastname: Martino-Alba
mesh_terms: D005260:Female; D006301:Health Services Needs and Demand; D006801:Humans; D007223:Infant; D008297:Male; D010166:Palliative Care / Q000379:methods*; D012189:Retrospective Studies; D014897:Spinal Muscular Atrophies of Childhood / Q000451:nursing*; D013727:Terminal Care / Q000379:methods*
keywords: Spinal muscular atrophy