No good choices, only hard decisions : the delicate nature of team family communication in pompe disease

Abstract

Objective: To describe the experience of a family going through the palliative care decision-making for a child with Pompe disease. Method: Qualitative research, case study design, approved by Ethics Committee (CAAE: 58643816400005327), conducted between 2017/2018. The setting was the Pediatric Intensive Care Unit of a university hospital in Brazil. Participants were family members of children admitted to the Intensive Care Unit and referred for palliative care. Results: The case study relates the decision-making on palliative care for a child with Pompe disease. The parents, surrounded by the palliative care available for the situation, experience the existential reality of the decision-making process to adopt palliative care for their child, together with the multi-professional team. Conclusions: Understanding the concept of quality of life that each family develops when faced with the seriousness of their child’s illness, when the possibilities of cure have been exhausted, is fundamental to the decision making process for adopting palliative care. It is essential to include the participation of the child’s or adolescent’s parents and other family members in the palliative care decision-making process, in order to give the family a voice, minimising their doubts and suffering, as well as offering shelter in a situation of extreme existential vulnerability.