Sickle cell disease complications: Prevalence and resource utilization

Nirmish Shah,Menaka Bhor,Huseyin Yuce,Lin Xie,Jincy Paulose,Lars-Peter Kamolz

doi:10.1371/journal.pone.0214355

Abstract

ObjectivesThis study evaluated the prevalence rate of vaso-occlusive crisis (VOC) episodes, rates of uncomplicated and complicated VOC episodes, and the primary reasons for emergency room (ER) visits and inpatient admissions for sickle cell disease (SCD) patients.MethodsThe Medicaid Analytic extracts database was used to identify adult SCD patients using claims from 01JUL2009-31DEC2012. The date of the first observed SCD claim was designated as the index date. Patients were required to have continuous medical and pharmacy benefits for ≥6 months baseline and ≥12 months follow-up period. Patient demographics, baseline clinical characteristics, the rate of uncomplicated and complicated VOC (VOC with concomitant SCD complications) episodes, and reasons for ER visits and inpatient stays were analyzed descriptively.ResultsA total of 8,521 patients were included in the analysis, with a median age of 30 years. The average follow-up period was 2.7 years. The rate of VOC episodes anytime in the follow-up was 3.31 in person-years. During the first-year follow-up period, an average of 2.79 VOC episodes were identified per SCD patients, with 1.06 VOC episodes treated in inpatient setting and 0.90 VOC episodes in ER without admission. A total of 76,154 VOC episodes were identified during the entire follow-up period for the overall SCD patients. Most of the VOC episodes (70.3% [n = 53,523]) were uncomplicated episodes, and 29.7% were complicated episodes. Using primary diagnosis claims only, the most frequent complications during the VOC episode were infectious diseases (25.9%), fever (21.8%), and pulmonary disorders (16.2%). Among ER and hospitalizations related to VOC or SCD complication, ~85.0% had VOCs as the primary reason for admission; 15.0% had SCD complications as the primary reason.ConclusionIn summary, SCD and its related comorbidities and complications result in high acute health care utilization. In addition, VOC remains the primary reason for SCD patients’ ER visits and inpatient admissions.

Highlights

Sickle cell disease (SCD) is a life-threatening genetic disorder affects ~100,000 individuals in the United States, where it is one of the most common genetic blood disorders [1,2]
During the first-year follow-up period, an average of 2.79 vaso-occlusive crisis (VOC) episodes were identified per SCD patients, with 1.06 VOC episodes treated in inpatient setting and 0.90 VOC episodes in emergency room (ER) without admission
A total of 76,154 VOC episodes were identified during the entire follow-up period for the overall SCD patients

Summary

Introduction

Sickle cell disease (SCD) is a life-threatening genetic disorder affects ~100,000 individuals in the United States, where it is one of the most common genetic blood disorders [1,2]. Damage to the red blood cells (RBCs) occurs due to polymerization of deoxygenated hemoglobin S (HbS) and these damaged cells have abnormal structures as well as expression of adhesion molecules. This results in hemolytic anemia and the blockade of small blood vessels, which lead to vaso-occlusion and end organ failure [3]. Multicell adhesion between RBCs, white blood cells, platelets, and endothelial cells result in painful vaso-occlusive crisis (VOC) [4,5,6]. SCD causes recurrent VOC episodes as well as increases the risk of infections and other complications that often require emergency intervention [8]. Multicell adhesion is the driver of VOC, and treatment targeting this adhesion results in more VOC-free days [11]

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