Sarah Bills

Date of Award

Summer 2023

Document Type

Open Access Dissertation



First Advisor

Jeffrey Schatz


Objective: Sickle cell disease (SCD) is a genetic blood condition that places youth at increased risk for deficits in attention and executive functioning suggestive of increased rates of Attention Deficit/Hyperactivity Disorder (ADHD). There is pronounced inconsistency in reported prevalence rates for attention deficits and ADHD diagnoses among youth with SCD, which is due in part to variability in methodology of previous studies. The primary aim of the present study is to use systematic screening to identify the prevalence of inattentive ADHD symptoms and ADHD diagnoses in a pediatric SCD population seen at a large hematology clinic. A secondary aim is to explore medical and social-environmental predictors of inattentive ADHD symptoms and ADHD diagnoses. Methods: One hundred and seven children with SCD (ages 7-11 years) completed a psychosocial screening at the Center for Cancer and Blood Disorders (CCBD) at Prisma Health Children’s Hospital in Columbia, South Carolina during a routine clinic appointment. Inattentive symptoms were assessed using the Strengths and Weaknesses of Attention-Deficit/Hyperactivity-symptoms and Normal-behaviors (SWAN) rating scale, a parent report scale of symptoms of inattention typically associated with ADHD. Follow up diagnostic procedures were completed for participants with positive screenings on the SWAN rating scale to determine if full inclusion and exclusion criteria for Predominantly Inattentive and Combined subtypes of ADHD (ADHD-I/C) were met. Information on indicators of disease severity (i.e., genotype, history of cerebrovascular disease) and social-environmental factors were collected through parent report and medical chart review.

Results: The prevalence rate of clinically elevated inattentive symptoms in the current sample (26%) was significantly greater than that observed in a large sample of school age youth (5.3%; Χ 2 = 92.86, p < .001, w = .93). Additionally, the prevalence rate of ADHDI/ C diagnoses in the current sample (13.1%) was significantly greater than that reported in the general population of Black youth in the United States (5.5%; Χ 2 = 11.82, p = .001, w = .33). Indicators of disease severity (i.e., genotype, history of cerebrovascular disease) did not predict inattentive symptoms or formal ADHD diagnoses. However, elevated family distress/dysfunction did significantly predict SWAN inattentive symptoms, B= .23, t(104) = 2.39, p = .019, R2 = .052.

Conclusions: Children with SCD evidenced elevated rates of inattentive symptoms and formal ADHD diagnoses, indicating increased risk for neurodevelopmental concerns. These findings highlight the importance of systematic screening and subsequent interventions for neurodevelopmental difficulties within the pediatric SCD population. Indicators of disease severity did not predict inattentive symptoms or formal ADHD-I/C diagnoses, suggesting that established medical predictors of neurocognitive deficits in pediatric SCD may not translate to symptoms of neurodevelopmental disorders such as ADHD. Family distress and dysfunction did significantly predict elevated inattentive symptoms, indicating that parent and family factors may have a greater impact on the presentation of ADHD in pediatric SCD. Future studies are needed to further examine biopsychosocial predictors of neurodevelopmental conditions such as ADHD in pediatric SCD.


© 2023, Sarah Bills