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Objective Sickle cell disease (SCD) is a genetic blood disorder that may affect patients’ mood and behavior. However, measuring the prevalence of internalizing symptoms (anxiety and depression) in patients with SCD has been elusive. We assessed internalizing symptoms in adolescents with SCD to evaluate prevalence and to test whether neurocognitive performance and frequency of pain-related episodes were associated with internalizing concerns. Methods One hundred eighty-five patients (57% HbSS/HbSß0-thalassemia, 43% HbSC/HbSß+-thalassemia), ages 12–18 years, received a neuropsychological evaluation as a part of a larger cohort study. Internalizing symptoms were measured using the Behavior Assessment System for Children, Second or Third Edition. Scores on the depression and anxiety scales were compared to normative values using Wilcoxon signed rank test. Spearman correlations examined associations between neurocognitive performances and internalizing symptoms. Robust multivariable regression models measured associations between internalizing symptoms and age, sex, sickle genotype, total hemoglobin, fetal hemoglobin, socioeconomic status, and frequency of pain episodes. Results Parent- and self-reported ratings of internalizing symptoms were not elevated compared to normative expectations. Overall, 1.8% and 6.3% of the sample displayed clinically elevated symptoms of anxiety and depression based on self-report, respectively. There were no associations between internalizing symptoms and neurocognitive performance (all p > .05). In multivariable analyses, the frequency of pain episodes was positively associated with self-reported anxiety (p = .006) and parent-reported depressive symptoms (p = .017). Conclusions Adolescents with SCD do not report elevated internalizing symptoms compared to normative expectations. Further research is needed to examine the trajectory of internalizing symptoms and the bidirectional relationship between pain and psychosocial functioning in SCD.

BACKGROUND: Children with sickle cell disease (SCD) experience neurodevelopmental delays; however, there is limited research with preschool age children. This study examined neurocognitive risk and protective factors in preschoolers with SCD. PROCEDURE: Sixty-two patients with SCD (60% HbSS/HbSβ(0)-thalassemia; 40% HbSC/HbSβ(+)-thalassemia) between the ages of 3 and 6 years (Mean=4.77 years) received a neuropsychological evaluation as routine systematic surveillance. Patients were not selected for disease severity, prior central nervous system findings, or existing cognitive concerns. Thirty-four patients (82% HbSS/HbSβ(0)-thalassemia) were prescribed hydroxyurea (HU) at the time of their neuropsychological evaluation. On average, these patients had been prescribed HU at 2.15 (Standard Deviation=1.45) years of age. The average dose was 28.8 mg/kg/day. Besides genotype, there were no group differences in medical or demographic factors based on HU treatment status. RESULTS: Patients with HbSS/HbSβ(0)-thalassemia scored below normative expectations on measures of intelligence, verbal comprehension, and school readiness (false discovery rate adjusted p-value [pFDR]0.05). Greater social vulnerability at the community level was associated with poorer performance on measures of intellectual functioning, verbal comprehension, visuomotor control, and school readiness, as well as parent report of executive dysfunction (pFDR

Objective Risk for neurocognitive deficits in sickle cell disease (SCD) is well established, yet minimal research has evaluated the risk for deficits in adaptive functioning. We assessed adaptive functioning in pediatric patients with SCD to test the hypothesis that disease, treatment, and demographic factors were associated with adaptive outcomes. Methods Two hundred fifty-six patients (57% HbSS/HbSß0-thalassemia and 43% HbSC/HbSß+-thalassemia), ages 8–18, received routine neuropsychological assessments as part of a larger prospective lifetime cohort study. Adaptive functioning was measured using the Behavior Assessment System for Children, Second or Third Edition. Adaptive scores were compared with normative values using t-test or Wilcoxon signed rank test and linear regression models were used to measure associations between adaptive functioning and age, hydroxyurea (HU) use, sickle genotype, and socioeconomic status. Furthermore, we examined the influence of intellectual and executive functioning on adaptive behavior using hierarchical linear regression analyses. Results Parent ratings of adaptive functioning skills did not differ from normative expectations (all false discovery rate [FDR] adjusted p-value [pFDR] > 0.05). Social vulnerability was negatively associated with adaptive scores on most adaptive scales in both genotypes (pFDR Conclusions Poorer parent-rated adaptive skills were associated with increased social vulnerability, lower Full-Scale IQ, and parent-rated executive difficulties. Most adaptive scores were in the normal range; however, parent ratings may not fully capture the impact of disease complications and neurocognitive deficits on daily functioning.