Abstract

Vaso-occlusive episodes (VOEs) are a hallmark of sickle cell disease (SCD), and account for >90% of health care encounters for this patient population. The Cooperative Study of Sickle Cell Disease, a large study enrolling >3000 patients, showed that the majority of SCD patients (80%) experienced 0-3 major pain crises/year. Only a small minority (~5%) experienced ≥6 VOEs/year. Our study sought to further understand this difference in VOE frequency between SCD patients. We analyzed 25 patients (13M/12F, mean age of 28.8) with ≥6 ED visits or hospitalizations/year (high utilizers), and compared these with 9 patients (6M/3F, mean age of 37.6) who had ≤2 ED visits or hospitalizations/year (low utilizers). All subjects were given a demographic survey along with questionnaires for depression, anxiety, and Health Locus of Control. Each subject then underwent quantitative sensory testing (QST) with three different modalities: pressure pain sensitivity, heat and cold sensitivity, and Von Frey monofilament testing. Laboratory and clinical data were collected through subjects' medical records. CBC and chemistry analysis showed high utilizers had higher WBC (p<0.01), ANC (p<0.01), total bilirubin (p = 0.02), and lower MCV (p = 0.03). Opioid use (morphine equivalents) over the past 6 months was significantly higher in the high utilizer group (12125.7 mg vs 2423.1 mg, p = 0.005). QST results showed lower pressure pain threshold at the ulna (224.4 KPa vs 338.9 KPa, p = 0.04) in the high utilizer group. High utilizers also had higher anxiety (9.0 vs 4.6, p = 0.04) and depression scores (10.0 vs 6.0, p = 0.051). While the low utilizer group had higher education levels with more associate and bachelor degrees (p = 0.009), there was no difference in income or employment. These data show that many biological and psychosocial factors contribute to high health care utilization in SCD. A multi-disciplinary and multi-faceted approach will be required to address this complex problem.

Highlights

  • Vaso-occlusive episodes (VOEs) are a hallmark of sickle cell disease (SCD), and account for over 90% of health care encounters for this patient population

  • We noted lower education levels with higher depression, anxiety, and health locus of control (HLOC) chance sub-scores in the high utilizer group

  • We found that patients with frequent VOE’s had higher white blood cell (WBC) counts, absolute neutrophil counts, and total bilirubin levels, suggesting more severe disease

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Summary

Introduction

Vaso-occlusive episodes (VOEs) are a hallmark of sickle cell disease (SCD), and account for over 90% of health care encounters for this patient population. An orphan disease (total number of patients in the US ~110,000), SCD has a huge medical-economic impact, with annual hospitalization costs of nearly $500 million by 2004 figures [1]. The Cooperative Study of Sickle Cell Disease (CSSCD) was a large natural history study, carried out in >23 Centers in the US, enrolling >3000 patients between 1977–93. An analysis of the frequency of VOE’s in CSSCD showed that most patients (80%) experienced 0–3 major pain crises/year; this was similar across all genotypes of the disease (SS, SC, Sβthalassemia, Sβ+ thalassemia). Similar findings have been reported in subsequent smaller studies, with 5–10% of SCD patients having frequent ED visits and hospitalizations [2]

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