Ethical attitudes of intensive care paediatricians as regards patients with spinal muscular atrophy type 1

Abstract 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 (LTE) in case of NIV failure. Confronted with a future hypothetical case, half of paediatricians would opt for the same plan (NIV + LTE), 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 LTE. 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 LTE when such support is insufficient, but they would accept the family's decision, even in case of disagreement.