Abstract
Sickle cell disease (SCD) is a life-threatening vascular disease that burdens affected persons physically. SCD-related vaso-occlusive crises (VOCs) are one of the primary causes of morbidity and mortality. Our objective was to examine the epidemiology of pain crises and the relationship between pain crises and major acute complications among SCD patients. Using the Medicaid Analytic Extracts from 2009–2013, patients with SCD were selected and the first clinical claim indicating SCD during the identification period was defined as the index date. Patients were required to have continuous Medicaid enrollment for ≥6 months pre- and 12 months post-index period. Clinical outcomes included mortality, inpatient pain crises, and complications. Cox regressions were applied to examine the relationship between pain crises and deaths or acute complications, respectively. A total of 20 909 patients were included with a mean age of 17.9 years. The rate of VOC events in 100 person-years was 142.20 for adults and 53.91 for pediatric patients. Patients with VOCs were associated with a higher risk for death (hazard ratio=1.56; 95% confidence interval: [1.19–2.05]) or acute complications including acute chest syndrome, stroke, pulmonary embolism, splenic sequestration, and pulmonary hypertension. SCD patients have a substantial burden of disease-related complications. This study suggests that inpatient vaso-occlusive crisis is a key risk factor for acute complications.
Highlights
Sickle cell disease (SCD) is a life-threatening vascular disorder, which early on progresses into a systemic disease.[1,2] SCD can affect various races and occurs more often among African-American and Hispanic-American births.[3]
To understand the burden and impact of Vaso-occlusive crises (VOCs) among SCD patients in a real-world setting, this study evaluated the rate of complications and associations between VOC and lifethreatening complications
Patient demographics and baseline clinical characteristics including age, sex, race, geographic region, Charlson comorbidity index (CCI) scores, baseline SCD management including SCD medication use and SCD management procedures, and baseline health care utilization (HRU); the number of baseline VOC events were controlled in the model as covariates
Summary
Sickle cell disease (SCD) is a life-threatening vascular disorder, which early on progresses into a systemic disease.[1,2] SCD can affect various races and occurs more often among African-American and Hispanic-American births.[3]. Vaso-occlusive crises (VOCs), known as acute sickle cell pain crises, are the most common, painful complication of the disease and the main reason why patients seek medical care in hospitals. SCD patients’ erythrocytes have the propensity to change into crescent shapes with abnormal adhesive properties, which increase interactions with white blood cells, platelets, endothelial cells, and extracellular matrix proteins. All these abnormal interactions accelerate the development of clinical vasculopathy and promote cerebrovascular and pulmonary vascular diseases. Several diary studies of SCD patients have reported high rates of pain as well as an intense severity of pain during study days.[6,7,8] VOC is the leading cause of hospitalization among SCD patients,[9] and health care utilization (HRU) among SCD patients remains high due to patients’ difficulty with acute pain management and the lack of new effective therapies.[10]
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