Navigating decisional conflict as a family when facing the decision of stem cell transplant for a child or adolescent with sickle cell disease

Highlights

• We describe decisional conflict in families facing real-time transplant decisions.

• The family’s perception of risk contributes to their decisional conflict.

• Pediatric patients with sickle cell disease feel uninformed about transplant.

• Families navigate decisional conflict together when facing a transplant decision.

• Decisional conflict can result in families delaying a curative treatment choice.

Abstract

Objective

Patients with sickle cell disease (SCD) face unpredictable disease, with stem cell transplant being a curative treatment option with risks. The aim of this study was to describe the level and source of decisional conflict in families of children/adolescents with SCD facing a transplant decision.

Methods

A multiple-case study approach described decisional conflict in various SCD severity/donor risk decisional contexts. Cases included parents, child/adolescent with SCD, and sibling donor. The level and source of conflict was measured via O’Connor’s Decisional Conflict Scale and analyzed using pattern-matching and cross-case synthesis. In-depth descriptions of conflict sources were obtained through thematic analysis of observation and interview data.

Results

Among 11 participants in four cases (i.e. family units) decisional conflict was not unique not to the decisional context. Conflict levels represented a level that can be linked with feeling unsure and decisional delay. The theme, ‘navigating decisional conflict together’, described how family units discussed uncertainties.

Conclusion

Varying levels and sources of decisional conflict exist in pediatric patients with SCD and their families considering transplant.

Practice Implications

In our cases, decisional conflict and the transplant decision occurred at the family-level. Clinicians should encourage all family members to participate in discussions concerning transplant.

Introduction

Decisional conflict can manifest when making a choice between treatment options involving risk and uncertain outcomes [1,2]. Importantly, decisional conflict can lead to patient and family distress, unmet informational needs, and delays in making a decision [1,2]. A recent integrative review investigating the pediatric decision-making process of those pursuing stem cell transplant (referred to as “transplant” hereafter) revealed that patients with sickle cell disease (SCD) and their families recognize that there are viable treatment options outside of a curative transplant (e.g. transfusions, hydroxyurea) [3]. This is in contrast to those with cancer and malignant disease perceiving transplant as the only option [3]. Furthermore, those with SCD undergoing transplant with a lower-risk, matched sibling donor have greater than 90 % overall survival [4], yet only 35 % of patients with SCD who have a matched sibling donor available choose to pursue transplant [5]. Others may only have a high-risk donor available. These findings suggest decisional conflict exists when faced with the decision of transplant as a curative treatment for SCD.

Shared decision-making is a mode in which families can collaborate and exchange information with their medical providers to reduce decisional conflict [6]. In pediatrics, this includes involving the child/adolescent in decision conversations to their capacity [6]. The American Academy of Pediatrics and various authors point out the importance of including the child/adolescent and family perspective in moving forward the knowledge of pediatric treatment decision-making [[6], [7], [8]]. Yet, previous investigations of transplant decision-making reveal minimal voice from the pediatric patient or sibling donor. To further the science of decisional conflict and shared decision-making in pediatrics, a greater understanding of parents’ and child/adolescent’s perspectives in the context of treatment decisions are necessary.

Additionally, the transplant decision-making process in pediatric SCD has primarily been investigated using retrospective methods or hypothetical scenarios to study decision preferences [[9], [10], [11], [12], [13], [14]]. This data has identified the severity of SCD and transplant risks as factors that are influential to this population’s treatment decisions [3,5], but the majority of knowledge available on this phenomenon is in the lower-risk setting of a matched sibling donor. The purpose of this study was to gain a better understanding of decisional conflict in real-time pediatric transplant decisions in the context of various SCD severity and donor risk contexts from the perspective of those who would engage in shared decision-making.

Therefore, our aim was to describe the level and source of decisional conflict in children/adolescents with SCD, their sibling donor(s), and their parent(s) when transplant is offered as a curative treatment option. Applying O’Connor’s framework [1,2], we sought to answer how and why the following contributes to decisional conflict in a child or adolescent with SCD, their sibling donor, and their parent(s): 1) perception of their disease and the proposed transplant, 2) awareness of personal values, and 3) assessment of personal support.