title: [Ethical attitudes of intensive care paediatricians as regards patients with spinal muscular atrophy type 1].
abstract: BACKGROUND
  Spinal muscular atrophy type 1 (SMA-1) is a progressive and fatal disease that leads to ethical problems for Paediatric professionals. Our objective was to determine the ethical options of Paediatric Intensive Care Unit (PICU) paediatricians as regards a child with SMA-1 and respiratory failure.
  
  
  METHODS
  A cross-sectional descriptive study was conducted using an anonymous questionnaire sent to PICUs in Spain (which can be accessed through the Spanish Society of Paediatric Critical Care web page).
  
  
  RESULTS
  Of the 124 responses analysed, 70% were from women, 51% younger than 40 years, 54% from a PICU with more than 10 beds, 69% with prior experience in such cases, and 53% with religious beliefs. In the last patient cared for, most paediatricians opted for non-invasive mechanical ventilation (NIV) and limitation of therapeutic effort (LET) in case of NIV failure. Confronted with a future hypothetical case, half of paediatricians would opt for the same plan (NIV+LET), and 74% would support the family's decision, even in case of disagreement. Age, prior experience and sex were not related to the preferred options. Paediatricians with religious beliefs were less in favour of initial LET. Less than two-thirds (63%) scored the quality of life of a child with SMA-1 and invasive mechanical ventilation as very poor.
  
  
  CONCLUSIONS
  Faced with child with SMA-1 and respiratory failure, most paediatricians are in favour of initiating NIV and LET when such support is insufficient, but they would accept the family's decision, even in case of disagreement.
authors.affiliation: Área de Pediatría, Servicio de Críticos, Intermedios y Urgencias Pediátricas, Hospital Clínico Universitario de Santiago de Compostela, Grupo de Investigación CLINURSID, Departamento de Enfermería, Universidade de Santiago de Compostela, Santiago de Compostela, España. Electronic address: carmeagra@hotmail.com.
authors.forename: María Carmen
authors.initials: MC
authors.lastname: Agra Tuñas
authors.affiliation: Grupo de Trabajo de Ética, Sociedad Española de Cuidados Intensivos Pediátricos (SECIP), Madrid, España; Servicio de Pediatría, Unidad de Cuidados Intensivos Pediátricos, Complejo Hospitalario Universitario de Badajoz, Badajoz, España.
authors.forename: Ramón
authors.initials: R
authors.lastname: Hernández Rastrollo
authors.affiliation: Grupo de Trabajo de Ética, Sociedad Española de Cuidados Intensivos Pediátricos (SECIP), Madrid, España; Servicio de Pediatría, Hospital Puerta del Mar, Cádiz, España.
authors.forename: Arturo
authors.initials: A
authors.lastname: Hernández González
authors.affiliation: Grupo de Trabajo de Ética, Sociedad Española de Cuidados Intensivos Pediátricos (SECIP), Madrid, España; Unidad de Cuidados Intensivos Pediátricos, Complejo Hospitalario Universitario de La Coruña, A Coruña, España.
authors.forename: Carmen
authors.initials: C
authors.lastname: Ramil Fraga
authors.affiliation: Grupo de Trabajo de Ética, Sociedad Española de Cuidados Intensivos Pediátricos (SECIP), Madrid, España; Unidad de Cuidados Intensivos Pediátricos, Hospital Sant Joan de Déu, Barcelona, España.
authors.forename: Francisco José
authors.initials: FJ
authors.lastname: Cambra Lasaosa
authors.affiliation: Grupo de Trabajo de Ética, Sociedad Española de Cuidados Intensivos Pediátricos (SECIP), Madrid, España; Servicio de Pediatría, Hospital Puerta del Mar, Cádiz, España.
authors.forename: Sebastián
authors.initials: S
authors.lastname: Quintero Otero
authors.affiliation: Grupo de Trabajo de Ética, Sociedad Española de Cuidados Intensivos Pediátricos (SECIP), Madrid, España; Departamento de Pediatría, Complejo Hospitalario Universitario de Granada, Ibs Granada, Ciberehd, Granada, España.
authors.forename: Angela
authors.initials: A
authors.lastname: Ruiz Extremera
authors.affiliation: Área de Pediatría, Servicio de Críticos, Intermedios y Urgencias Pediátricas, Hospital Clínico Universitario de Santiago de Compostela, Grupo de Investigación CLINURSID, Departamento de Enfermería, Universidade de Santiago de Compostela, Santiago de Compostela, España; Grupo de Trabajo de Ética, Sociedad Española de Cuidados Intensivos Pediátricos (SECIP), Madrid, España; Instituto de Investigación de Santiago (IDIS), Red de Salud Materno infantil (SAMID II), Madrid, España.
authors.forename: Antonio
authors.initials: A
authors.lastname: Rodríguez Núñez
mesh_terms: D001291:Attitude of Health Personnel; D002648:Child; D003422:Critical Care; D003430:Cross-Sectional Studies; D004992:Ethics, Medical; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010372:Pediatrics; D057566:Self Report; D014897:Spinal Muscular Atrophies of Childhood / Q000628:therapy
keywords: Atrofia muscular espinal tipo 1; Clinical ethics; Communication; Comunicación; Critical care; Cuidados intensivos; Decision making process; Encuesta; Familia; Family; Mechanical ventilation; Non-invasive ventilation; Spinal muscular atrophy type 1; Survey; Toma de decisiones; Ventilación mecánica; Ventilación no invasiva; Ética asistencial