Actitudes éticas de los pediatras de Cuidados Intensivos ante pacientes con atrofia muscular espinal tipo 1 / Ethical attitudes of intensive care paediatricians as regards patients with spinal muscular atrophy type 1

INTRODUCCIÓN: La atrofia muscular espinal tipo 1 (AME-1) es una enfermedad progresiva e incurable que plantea problemas éticos entre los profesionales de Pediatría. Nuestro objetivo ha sido conocer las opciones éticas de los pediatras de UCIP ante los pacientes con AME-1 y fracaso respiratorio. MATERIAL Y MÉTODOS: Estudio descriptivo transversal, mediante una encuesta anónima enviada a las UCIP de España y accesible en la web de la Sociedad Española de Cuidados Intensivos Pediátricos. RESULTADOS: Analizamos 124 respuestas (70% mujeres, 51% menores de 40 años, 54% de UCIP con más de 10 camas, 69% con experiencia previa con estos niños y 53% con creencias religiosas). En el último caso atendido, la mayoría de los pediatras optó por la ventilación no invasiva (VNI), realizando después limitación del esfuerzo terapéutico (LET). Ante un hipotético caso futuro, la mitad de los pediatras apoyarían la misma opción (VNI+LET) y el 74% apoyaría la decisión de la familia, aunque no coincidiera con la suya. No se observaron diferencias según la edad, la experiencia previa o el sexo. Los pediatras con creencias religiosas son menos partidarios de la LET inicial. El 63% considera que la calidad de vida de un niño con AME-1 y ventilación invasiva es muy mala. CONCLUSIONES: Ante un niño con AME-1 y fracaso respiratorio, la mayoría de los pediatras de UCIP están a favor de iniciar la VNI y realizar LET cuando dicho soporte no sea suficiente, pero apoyarían la decisión de la familia aunque no estuvieran de acuerdo con ella

INTRODUCTION: 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. MATERIAL AND 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

[Ethical attitudes of intensive care paediatricians as regards patients with spinal muscular atrophy type 1]. / Actitudes éticas de los pediatras de Cuidados Intensivos ante pacientes con atrofia muscular espinal tipo 1.

INTRODUCTION: 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. MATERIAL AND 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.