title: [Spinal muscular atrophy and respiratory failure. How do primary care pediatricians act in a simulated scenario?].
abstract: BACKGROUND
  Spinal muscular atrophy type 1 (SMA-1) tends to be fatal in the first year of life if there is no ventilatory support. The decision whether to start such support is an ethical conflict for healthcare professionals.
  
  
  METHODS
  A scenario of acute respiratory failure in an infant with SMA-1 has been included in a training program using advanced simulation for Primary Care pediatricians (PCP). The performances of 34 groups of 4 pediatricians, who participated in 17 courses, were systematically analyzed. Clinical, ethical and communication aspects with parents were evaluated.
  
  
  RESULTS
  The initial technical assistance (Administration of oxygen and immediate ventilatory support) was correctly performed by 94% of the teams. However, the PCP had problems in dealing with the ethical aspects of the case. Of the 85% of the teams that raised the ethical conflict with parents, 29% did so on their own initiative, 23% actively excluded them, and only 6% involved them and took their opinion into account in making decisions. Only 11.7% asked about the quality of life of children and 12% for their knowledge of the prognosis of the disease. None explained treatment alternatives, nor tried to contact the pediatrician responsible for the child.
  
  
  CONCLUSIONS
  When faced with a simulated SMA-1 infant with respiratory failure, PCP have difficulties in interacting with the family, and to involve it in the decision making process. Practical training of all pediatricians should include case scenarios with an ethical clinical problem.
authors: Área de Pediatría, Servicio de Críticos, Intermedios y Urgencias Pediátricas, Hospital Clínico Universitario de Santiago de Compostela, Santiago de Compostela, A Coruña, España. Electronic address: carmeagra@hotmail.com.
authors: M C
authors: MC
authors: Agra Tuñas
authors: Fundación Pública Urxencias Sanitarias 061 de Galicia, Santiago de Compostela, A Coruña, España.
authors: L
authors: L
authors: Sánchez Santos
authors: Servicio de Pediatría, Complexo Hospitalario de Pontevedra, Pontevedra, España.
authors: M
authors: M
authors: Busto Cuiñas
authors: Área de Pediatría, Servicio de Críticos, Intermedios y Urgencias Pediátricas, Hospital Clínico Universitario de Santiago de Compostela, Santiago de Compostela, A Coruña, España; Instituto de Investigación de Santiago (IDIS), Santiago de Compostela, A Coruña, España; Red de Salud Materno Infantil y del Desarrollo (Red SAMID II), Instituto Carlos III, Madrid, España.
authors: A
authors: A
authors: Rodríguez Núñez
mesh_terms: D006801:Humans; D000072143:Pediatricians / Q000941:ethics; D010818:Practice Patterns, Physicians'; D011320:Primary Health Care; D011788:Quality of Life; D012131:Respiratory Insufficiency / Q000628:therapy*; D014897:Spinal Muscular Atrophies of Childhood / Q000628:therapy*
keywords: Atención primaria; Atrofia muscular espinal tipo 1; Clinical ethics; Communications; Comunicación; Conflicto ético; Decision taking; Ethical conflict; Familia; Family; Pediatrics; Pediatría; Primary care; Simulación; Simulation; Spinal muscular atrophy type 1; Toma de decisiones; Ética asistencial