OBJECTIVES: Sickle cell disease (SCD), a rare genetic blood disorder, involves multicellular interactions between blood and endothelial cells that affect multiple organ systems leading to both acute and chronic complications. This study estimated complication rates among pediatric and adult SCD patients in a large US administrative claims database with commercial (COM) and Medicare Advantage (MA) health plans.

METHODS: Retrospective analysis of pediatric (0-17 years) and adult (18+ years) patients with 2+ non-diagnostic claims for SCD from January 1, 2014–September 30, 2019 in the Optum Research Database. Index date was the first date with a SCD claim. Patients were continuously enrolled for 6 months before (baseline) and 6+ months after the index date (follow-up) and excluded if they had evidence of pregnancy or clinical trial participation. Follow-up SCD-related complication rates per 1,000 person-years (PY), excluding those with baseline evidence, were stratified by insurance type. SCD-related complications included: nephropathy (acute kidney injury [AKI], chronic kidney disease [CKD]), neurologic injury (stroke, transient ischemic attack [TIA], neurocognitive deficit), and priapism.

RESULTS: 2,524 pediatric (16.7%) and adult (82.3%) patients with SCD were identified (COM: 62.1%; MA: 37.9%; COM mean (SD) age: 32 (18) years, 46.0% male; MA mean (SD) age 63 (15) years, 37.4% male). Mean (SD) follow-up was 2.7 (1.8) and 2.6 (1.6) years for COM and MA patients, respectively. Top three 6-month baseline Agency for Healthcare Research and Quality (AHRQ) comorbidities for COM and MA patients were anemia; 46.7%, 58.7%; hypertension: 16.5%, 70.0%; and heart disease: 20.8%, 57.8%. COM and MA SCD-related complication rates per 1,000 PY for AKI were: 25.6, 108.8; CKD: 16.5, 91.6; stroke: 23.1, 68.7; TIA: 9.7, 29.0; neurocognitive deficit: 2.6, 4.8; and priapism: 9.7, 4.3.

CONCLUSIONS: SCD-related complications may occur early and increase with age as shown in this sample of commercial and Medicare Advantage SCD patients.