INTRODUCTION
Sickle Cell Disease (SCD) affects an estimated 80,000 to 100,000 people in the US1,2 and is associated with large healthcare expenditures 3-8. In the United States, the disease predominantly affects African Americans, but SCD is the most common single-gene disease in the world and is a major global health concern9,10. SCD causes painful, debilitating, and life-threatening symptoms 11 and is known for considerable variation in symptom type and severity12. Health care for this complicated disease often requires multiple providers and treatment modalities11,13. Multiple studies have shown that the majority of SCD treatment expenditures come from hospitalizations3-8,14,15, which can occur multiple times a year for each patient. This research examines SCD expenditures over time by disease severity.
SCD has a range of disease complications and severity, and multiple factors have been examined as predictors of SCD severity. Genetic variations in fetal hemoglobin gene expression and the co-inheritance of alpha thalassemia are linked to symptom severity12,16,17. Other genetic impacts on symptom severity are associated with blood chemistry biomarkers18. However, the literature indicates that not all symptom heterogeneity can be explained by genetic factors. Patients with SCD often have other chronic health conditions and comorbidities that can benefit from coordination of their health care services 13,19-23. Asthma can especially exacerbate sickle cell disease complications21, and asthma care is well-known to benefit from community health workers interventions (CHW) 24. Other investigators have categorized acute ED and hospitalizations in SCD as unavoidable and avoidable. For example, an unavoidable hospitalization would be for SCD with fever and acute chest syndrome; unavoidable hospitalizations are necessary. In contrast, an avoidable hospitalization would be for sickle cell pain triggered by over-exertion or forgetting to obtain refills of pain medications. Care coordination (from CHWs) might have guided different choices and prevented the avoidable hospitalization13,19,20.
CHW may also be able to provide home assessments and education to ameliorate social and environmental factors that could reduce SCD severity. Exposure to tobacco smoke is associated with 73% more ED visits for acute chest syndrome21. Exposure to cold or wind increases the number of acute pain episodes that require hospitalization25,26. CHW can also assist with access to community resources, especially mental health services that could modulate SCD health care utilization 24,27,28.
In 2014, the Centers for Medicare and Medicaid awarded a grant to the University of Illinois at Chicago Pediatrics Department to fund the “Coordinated Healthcare for Complex Kids” (CHECK) program. The program was designed to provide comprehensive care coordination of services to Medicaid-enrolled children and young adults living in Chicago. The CHECK program enrolled patients with one or more chronic conditions, with the aim of determining whether health service coordination would reduce health care expenditures in patients with complex health needs23,29-33.
Patients with SCD were included in the CHECK program because their large treatment expenditures and disease complexities. A prior randomized trial of the CHECK program including patients with a range of diseases found no difference in health care expenditures between a CHECK treatment group (N=3126) and a Usual Care control group (N=3128) but this study could not account for heterogeneity within the small subgroup with SCD (n=12 received CHECK and n=21 received Usual Care)33. Therefore, additional analyses were needed to understand the specific experience of children with SCD using a larger sample. The aim was to describe expenditure patterns of patients with SCD enrolled in the CHECK program by level of hospitalization risk over a three-year period (baseline year, and one-and-two years after enrollment).