Neurocognitive Screening and Neighborhood Impact for Children and Adolescents with Sickle Cell Disease

Background: Sickle Cell Disease (SCD) is a genetically inherited blood disorder affecting over 100,000 people in the United States and is predominately seen in racial minorities. Given cerebrovascular involvement, observed differences in neurocognitive functioning exist across child development. Exploring the role of broader socioeconomic disadvantages specific to patient’s communities is imperative to understanding the potential impact on neurocognitive and academic performance. This project aimed to understand resources/referrals provided through embedded neurocognitive testing, as well as explore the relationship between indices of socioeconomic and educational disadvantage and neurocognitive performance as part of serial screening during the comprehensive SCD visit.

Methods: Data were collected retrospectively from the electronic medical record between January 2023 and July 2024 for patients who completed neurocognitive screening across 4 age cohorts; 3-4, 6-7, 11-12, and 15-16 years. Screening batteries were age-based and assessed for cognitive performance, academic functioning, and attention. Caregiver-proxy and self-report were completed for domains of executive functioning, emotional concerns, and sleep. The area deprivation index (ADI) and childhood opportunity index (COI) were determined using publicly available census block data for the patient at the time the screening was completed. ADI is measured on a 1-10 ordinal scale with 1 being the least disadvantaged and 10 being the most. COI is measured on a 5-point Likert Scale of Very Low to Very High that correlates with numeric values of 1 to 5 (i.e. 1 = Very Low). Descriptive statistics were completed using SAS 9.4. Spearman correlations were run to adjust for nonparametric data. ADI and COI were analyzed separately due to concerns with collinearity.

Results: Forty-five patients with an average age of 9.3 years (range 3-17) completed neurocognitive screening. The majority had SS genotype (64%), identified as male (53%), and attended the appointment with their mother (68.4%). One patient had a history of overt stroke and moyamoya disease. Six patients were not yet attending school (range Preschool to 11^th grade). Of patients scheduled, the majority completed testing (71.42%).

Of patients enrolled in school, updating a 504-plan (29%) or IEP (8.9%), or initiating a 504-plan (6.7%) or IEP (4.4%), were the most common recommendations. Of patients not enrolled in school, 83.3% were referred to the education liaison to navigate preschool options. To address emotion concerns (anxiety, inattention, executive functioning, health-related quality of life), recommended follow-up included: brief intervention/monitoring with embedded psychologist (26.7%), specialized psychologist at institution (17.8%), or school-based counselor (4.4%). Sleep study referrals were placed for 29% of patients.

Average State ADI for our tested cohort was a 5.6 (+/-3.06), which is similar to average state ADI for our overall SCD population (mean = 6). Patients also resided in areas considered to have “Very Low” overall COI (44%) as well as subindices; Education (51%), Health and Environment (37%), and Social and Economic (44%).

Higher State ADI scores (greater disadvantage) were associated with lower working memory (p<0.001), math (p=0.006) and spelling (p=0.002), as well as worse sleep initiation/maintenance (p <0.001). Lower State COI scores (lower childhood opportunity) were associated with lower reading (p=0.004), spelling (p<0.001), and math (p=0.005) performance and worse sleep initiation/maintenance (p <0.001).

Conclusion: Serial monitoring for changes in neurocognitive functioning is necessary for understanding the impact of SCD on brain health. Embedded neurocognitive screening within comprehensive SCD care can improve access to resources needed by these patients. In addition to identifying the needs of patients with SCD, it is imperative to explore what resources are available to these patients through measures such as the ADI and COI. Future goals of this research include comparing the efficacy of neurocognitive screening to full neuropsychological testing, identifying barriers patients face in accessing testing, and providing valuable resources based on the results of these tests.