Children with sickle cell disease (SCD) are at risk for neurobehavioral problems because of the impact the disease can have on the central nervous system. Specific impairments in working memory are particularly prevalent in school-aged children with SCD. Working memory is more strongly associated with school readiness and academic success than intellectual ability in the general population. The adverse effects of low socioeconomic status (SES) and poverty on cognition and neurodevelopment emerge early, before children have entered formal education. In addition, they affect language and executive function skills (e.g., working memory) more than other skills. SES is a proxy variable for other risk factors. Higher SES is associated with less parental stress, more supportive parenting practices, and better cognitive stimulation based on the availability of books, computers, and outings.

PRIMARY OBJECTIVE:

• To examine working memory and school readiness in young children with sickle cell disease in comparison to demographically matched control children without sickle cell disease.

SECONDARY OBJECTIVE:

• To examine the relationships of family/environmental factors (caregiver stress, parental responsiveness, and cognitive stimulation in the home) and disease severity to working memory and school readiness skills in preschool-aged children with SCD.

Participants will be recruited through the Pediatric Sickle Cell Clinic at St. Jude. All participants with SCD will be evaluated once with laboratory measures of cognitive and pre-academic skills and parent questionnaires. Medical history will be evaluated for purposes of characterizing the health status and disease severity of patients and determining how medical factors are related to school readiness and working memory in SCD population. The study expects to accrue up to 99 participants with SCD.

Demographically-matched control children will be selected from a database of participants enrolled in an ongoing longitudinal study of neurocognitive development in children born in Shelby County, Tennessee. The control group will be randomly selected from an existing study group and will not be enrolled on the MEMREAD protocol. Control group participants will be excluded if they have any sensory or motor impairment that would preclude valid testing (e.g., blindness, paresis), or if they have received treatment with non-stimulant psychotropic medication. A de-identified data file that includes the following results for up to 168 demographically-matched healthy control participants (approximately 1:2 ratio of SCD to healthy control) will be provided for control comparison purposes:

• Health Evaluation (CV4)
• Stanford binet - Fifth Edition (CV4)
• Bracken Basic Concepts Scale - Third Edition (CV4)
• The Behavior Rating Inventory of Executive Function - Preschool Version (BRIEF-P) (CV4)
• The Scales of Independent Behavior - Revised, Early Development Form (SIB-R) (CV4)
• Demographics (CV4)
• Additional Covariates (if any) including gestational age at birth, developmental delays, major medical illnesses, surgery history, neurological event history, pre-kindergarten attendance, and daycare attendance

Inclusion Criteria:

• Patient at St. Jude Children's Research Hospital (SJCRH) with a primary diagnosis of sickle cell disease
• 48-68 months of age at time of enrollment
• English as primary language

Exclusion Criteria:

• Sensory or motor impairment that would preclude valid testing (e.g., blindness, paresis)
• Treatment with non-stimulant psychotropic medication
• Enrollment in kindergarten at time of assessment

Participants will be between 4 and 5 years of age, have a diagnosis of sickle cell disease (SCD) and be patients at St. Jude Children's Research Hospital.