Treatment patterns for sickle cell disease among those with cerebrovascular disease in the US

Background: New treatments and guidelines in sickle cell disease (SCD) have improved the quality and lifespan of SCD patients. Over 90% with SCD will live into adulthood, and the majority will live past 50 years of age. However, data on comorbidities and treatments among SCD patients with and without cerebrovascular disease (CVD) are limited.Objectives: To describe the outcomes and preventive treatments used on SCD patients with and without CVD, based on a dataset of over 11,000 SCD patients.Methods: We identified SCD patients with and without CVD from the Marketscan administrative database using validated ICD-10-CM codes from January 1, 2016 to December 31, 2017. We summarized treatments received (iron chelation, blood transfusion, transcranial Doppler and hydroxyurea) and tested for differences by CVD status using the t-test for continuous variables and the chi-square for categorical variables. We also tested for differences among SCD, stratifying by age (=18 years). Results: Of the 11,441 SCD patients, 833 (7.3%) had CVD. SCD patients with CVD were more likely to have diabetes mellitus (32.4% among those with CVD vs. 13.8% without CVD), congestive heart failure (18.3% vs. 3.4%), hypertension (58.6% vs. 24.7%), chronic kidney disease (17.9% vs. 4.9%), and coronary artery disease (21.3% vs. 4.0%). SCD patients with CVD were more likely to receive a blood transfusion (15.3% vs. 7.2%) and hydroxyurea (10.5% vs. 5.6%). Fewer than 20 patients with SCD were given iron chelation therapy, and none received transcranial doppler ultrasound. Hydroxyurea was prescribed among a greater percentage of children (32.9%) than adults (15.9%). Conclusions: There appears to be an underutilization overall of treatment options among SCD patients with CVD. Further research would confirm these trends and explore ways to increase utilization of standard treatments among SCD patients.