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).