Advance Care Planning in Sickle Cell Disease: A Scoping Review

Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by abnormal red blood cell sickling, leading to pain, organ dysfunction, and early mortality. Its severe, unpredictable course and the emergence of complex decisions surrounding transformative therapies have prompted recommendations to integrate palliative care (PC) to support patients and families. Advance care planning (ACP) is a component of PC that seeks to align medical care with patient values and preferences. Individuals with SCD may benefit from ACP throughout the lifespan, yet best practices remain poorly defined. This scoping review, conducted using Joanna Briggs Institute methodology and reported per PRISMA-ScR (PRISMA extension for scoping reviews) guidelines, explores what is known about ACP in SCD and identifies future research priorities. Nine articles published between 2010 and 2025 met inclusion criteria. The limited available evidence suggests that patients are open to ACP discussions with trusted clinicians, but few patients had participated in formal or informal ACP. Personal and environmental factors may influence ACP engagement, including patient-clinician trust, patient and clinician understanding of ACP in SCD, timing of ACP conversations, and previous experiences with critical illness or end-of-life care. Proposed steps for advancing ACP in SCD include education, early PC integration, and strengthened patient-clinician communication and relationships. This scoping review is the first to summarize proposed barriers, facilitators, and strategies to improve ACP for individuals living with SCD.