Perception of Healthcare Utilization Among Individuals with Sickle Cell Disease: A Scoping Review

Background: Sickle cell disease (SCD) is characterized by a mutation in the hemoglobin beta chain which leads to misshapen red blood cells. Consequently, people living with SCD (PLWSCD) face lifelong affiliations of blood transfusions and a reduced quality of life due to excruciating pain and end-organ damage. Despite SCD being one of the most common genetic disorders with approximately 100,000 individuals bearing its burden in the United States (U.S), current literature suggests that there is a decrease in the number of individuals receiving continuous care from a physician. As chronic pain is one of the clinical determinators, the very manifestations of SCD lead to frequent emergency department (ED) visits and hospitalizations. Nonetheless, many PLWSCD choose to manage their chronic episodes at home despite their excruciating pain, suggesting that there is a level of avoidance towards the healthcare system.

Aims: As gaps remain in understanding the care seeking behavior for patients with SCD, the ultimate goal of this scoping review is to synthesize the evidence regarding barriers and facilitators influencing healthcare utilization among PLWSCD within the U.S.

Methods: This scoping review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analysis Extension for Scoping Reviews (PRISMA-ScR) Checklist. Utilizing a predefined search strategy, an initial search of the peer-reviewed literature was conducted on March 24, 2023, and updated on March 4, 2024. Of 708 articles screened, 70 were included. A minimum of two reviewers screened and extracted data independently through a web-based review management program. Risk of bias (RoB) was assessed for all studies using the relevant tool appropriate for the study design. Final results were synthesized by a narrative mix of qualitative and quantitative analysis.

Results: Findings indicate that the top four barriers that impact healthcare utilization amongst SCD patients included social (n = 25 studies), interpersonal (n = 23 studies), economic (n = 15 studies), and institutional-level factors (n = 11 studies). The top four most reported facilitators were technology (n = 9 studies), education (n = 7 studies), autonomy (n = 6 studies), and a positive patient-provider relationship (n = 6 studies). The top two forms of healthcare utilization were hospital admissions (n = 19 studies) and ED visits (n = 18 studies). Evidence-based interventions (EBI) such as individualized pain plans (IPPs) (n = 4 studies) and strategies designed for quality-improvement (QI) initiatives were found to decrease healthcare avoidance and minimize the harmful effects experienced within the SCD population (n = 3 studies).

Summary/Conclusion: There are limited studies that examine the effect of large healthcare disparities in SCD from an individual perspective. This is the first scoping review to assess healthcare utilization amongst SCD individuals in the U.S. comprehensively. Our findings can inform policymakers and stakeholders on the most effective ways to address the unmet needs that are glaringly evident in PLWSCD accessing care in the U.S. Recommendations include (1) enhancing patient experience and self-autonomy, (2) establishing support systems and multidisciplinary approaches to streamline SCD care (3) improving health literacy through tailored SCD education, and (4) applying a dual perspective approach with contextual lens to champion health equity. Future EBIs that target such disparities and consider the facilitators identified in this review can have beneficial effects on both the experiences and health outcomes of PLWSCD.