Working Memory and School Readiness in Preschool-Aged Children With Sickle Cell Disease
Condition: Sickle Cell Disease
MeSH terms: Anemia, Sickle Cell
Study type: Observational

Summary: 
      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.
    

Detailed description: 
      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
    

Intervention name(s): Evaluation of cognitive and pre-academic skills; Parent questionnaires
Intervention type(s): Other; Other
Intervention description(s): Participants will complete testing to evaluate fluid reasoning, knowledge, quantitative reasoning, visual-spatial processing, verbal and non-verbal working memory, and knowledge of pre-academic skills (such as colors, letters, numbers, counting, sizes, comparisons, and shapes).; Parents will complete questionnaires to assess behavioral manifestation of executive functions, functional independence and adaptive functioning, child and parent characteristics, situational/demographic life stress, demographics, education level, and household income.
Other intervention name(s): Testing; Parent report
Observational model: Cohort
Time perspective: Prospective
Sampling method: Non-Probability Sample
Study population: 
        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.