Central Nervous System Complications in Different Types of Sickle Cell Disorders: A Nation-Wide Analysis

Introduction: Sickle cell disorders have long been associated with central nervous system (CNS) complications. Morbidity and outcomes of sickle cell anemia have been extensively studied. However, few studies are available examining CNS complications in adult patients with different types of sickle cell disorders.

Methods: We used the National Inpatient Sample (NIS) ICD-10 codes between 2016 and 2020 in STATA BE software to perform a retrospective study and evaluate CNS complications in adult hospitalized patients with different types of sickle cell disorders. We have applied descriptive statistics to detect baseline characteristics of these patients and logistic regression analysis to assess CNS complications while adjusting for comorbid conditions.

Results: 105,976 patients were hospitalized with sickle cell disorders between 2016 and 2020. The most common sickle cell disorder was sickle cell anemia (SS) (88%), followed by sickle-hemoglobin C disease (SC) (5.8%), sickle-cell thalassemia (ST) (5%), and other unspecified sickle cell disorders (1%). Most patients were women (57%), black (90%), and with a mean age of 34 years. We have analyzed different CNS complications in these patients, including stroke, migraine, and seizures.

All sickle cell disorders were associated with increased association with stroke except for ST, though these findings weren't statistically significant (p =0.92, p=0.71, and P=0.76, respectively). It was observed that African Americans were less likely to have stroke (Odds ratio (OR) 0.7, 95% CI, 0.5-0.97; p =0.03). Hypertension (OR 1.6, 95% CI,1.36-1.8), congestive heart failure (OR 1.4, 95% CI, 1.1-1.7), hyperlipidemia (OR 2.5, 95% CI, 2.1-2.9), and thrombophilia (OR 4.7, 95% CI, 3.5-6.2) had a strong association with stroke in patients with sickle cell disorders. There was no statistically significant difference in the incidence of migraine among sickle cell disorder patients.

Interestingly, the odds of developing seizures were less in SC (OR 0.6, 95% CI, 0.4-0.9; p<0.01) and in ST (OR 0.5, 95% CI, 0.35-0.8; p<0.003). Congestive heart failure (OR 2, 95% CI, 1.7-2.7), chronic kidney disease (OR 1.4, 95% CI, 1.1-1.8), and end-stage renal disease (OR 2.5, 95% CI, 2-3.1) patients with sickle cell disorders had increased likelihood of mortality.

Conclusion: Our analysis didn't show a statistically significant difference in the likelihood of CNS complications in different types of sickle cell disorders, except in less odds of developing seizures in SC and ST. However, multiple comorbidities were associated with worse outcomes. More studies are needed to assess morbidity among different sickle cell disorders in adult patients.

No relevant conflicts of interest to declare.