Background. Children with sickle cell disease have an increased risk of neurodevelopmental disorders such as attention deficit hyperactivity disorder, intellectual disability, and specific learning disabilities. Little research has been done to characterize the sickle cell disease–related characteristics associated with neurodevelopmental disorders in the sickle cell disease population. Methods. This study was a retrospective chart review involving the outpatient records of 2 medical centers, Kennedy Krieger Institute and Johns Hopkins Hospital. Participants in the study included 59 children with sickle cell disease with a documented neurodevelopmental diagnosis, specifically attention deficit hyperactivity disorder, attention issues, behavioral issues, executive dysfunction, specific learning disabilities in math, reading, and reading comprehension, intellectual disabilities, developmental delay, fine motor disorders, language disorders, or autism spectrum disorders. Results. Children with sickle cell disease type hemoglobin S-β thalassemia plus had significantly higher odds of attention issues than children with sickle cell disease type hemoglobin SS (OR = 17.0, 95% CI = 1.99-145.00, P < .02). Children with sickle cell disease and a reported history of asthma had significantly higher odds of behavioral issues than children with sickle cell disease without a history of asthma, after adjustment for gender and sickle cell disease type (exact OR = 19.53, 95% CI = 1.16-1369.72, P < .04). Conclusion. Children with sickle cell disease may have increased risk for certain neurodevelopmental diagnoses based on their disease characteristics and associated comorbidities. These preliminary study results should be explored in a larger database.

Summary: Adults with sickle cell disease (SCD) are at risk for cognitive impairment, which causes significant morbidity. Guidelines support routine cognitive screening, but no screening test is validated in this population. We explored the Montreal Cognitive Assessment (MoCA) as a possible screening test in SCD. We administered the MoCA; a literacy test, the Wide Range Achievement Test, fourth edition (WRAT‐4); and a health literacy test, the Shortened Test of Functional Health Literacy in Adults (S‐TOFHLA) to adults with SCD and gathered clinical variables through chart review. Spearman's rho, Mann–Whitney, and Kruskal–Wallis tests and quantile regression models were used. Among our sample of 49 adults with SCD, the median MoCA score was 25.0 [interquartile range (IQR) 22.0–28.0]. Higher educational attainment was associated with MoCA scores (p = 0.001). In multivariable models, MoCA scores were associated with S‐TOFHLA (p = 0.001) and WRAT‐4 Reading (p = 0.002) scores, and overt stroke (p = 0.03) at the median. This pilot study adds to the limited literature of cognitive screening tests in adults with SCD and demonstrates a relationship between MoCA scores and measures of literacy and health literacy. The MoCA is a promising option for briefly screening for cognitive impairment in adults with SCD, though further study is needed to confirm its validity.

Objective: The objective of this study is to retrospectively determine the co-occurrence, associated characteristics, and risk factors for neurodevelopmental disorders (NDD) in a pediatric sickle cell disease (SCD) clinic population. Method: We investigated the co-occurrence and features of NDD in pediatric SCD through a retrospective cohort study conducted between July 2017 and January 2019. The participants were patients with SCD younger than 18 years of age identified from our institutions' clinic rosters and medical records databases. Results: A total of 276 participants were eligible for study inclusion, and 65 participants were found to have various NDD. Children with SCD and NDD were more likely to have a history of multiple SCD-related complications in comparison to children with SCD without NDD. Children with SCD and NDD were more likely to use disease-modifying therapies in comparison to children with SCD without NDD (χ² 27.2, p < 0.001). Conclusion: Children with SCD and NDD have higher odds of having certain disease-related complications and higher use of disease-modifying treatments than children with SCD who do not have NDD. Screening and diagnoses of NDD may be relevant to clinical management of pediatric SCD.