“ Consider the Following Situation,” Parental Treatment Preferences for Children With Rare Diseases: A Content Analysis of Pediatric Advance Care Plans

Abstract

Introduction

Caring for a child with a rare disease frequently involves making difficult medical decisions with limited disease-specific information. Advance care planning can assist families in articulating their values and treatment preferences. We aimed to describe parental treatment preferences for life-sustaining therapies and reconsideration of treatment preferences among families of children with rare diseases.

Methods

We used inductive content analysis of Respecting Choices Next Steps FAmily CEntered (FACE) pediatric advance care planning interviews and documents to describe parental treatment preferences in a hypothetical clinical scenario.

Results

Eighteen parents across 14 interviews completed an advance care plan. In 13 of 14 advance care plans, parents indicated their preference was to continue all treatments. In 10 of 13 of those plans, parents indicated circumstances in which they would reconsider continuing all treatments which we described using 4 themes: having concerns about child’s quality of life; after allowing some time to see; having certainty in child’s decline; and being at peace.

Conclusions

While almost all families who completed an advance care plan indicated preference to continue all treatments, most families would reconsider this plan in particular circumstances. Using language expressed by families described here may help providers and families communicate effectively around treatment plans, particularly among the understudied community of children with rare diseases.

Keywords

advance care planning rare diseases pediatric palliative care qualitative research end-of-life care life support care

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