Clinical Outcomes of a Neurocognitive Screening Program for Pediatric
Sickle Cell Disease
Abstract
Objective: Sickle cell disease (SCD) is associated with neurocognitive
and academic impairment. Routine screening is recommended to evaluate
for possible concerns. This paper describes results from a
neurocognitive screening battery and evaluates relationships with
psychosocial and medical variables. Methods: Participants included 61
patients stratified among three age groups, including ages 6-7, ages
11-12, and ages 15-16. Patients completed a screening assessing
cognitive performance, academic functioning, and attention (older
subjects completed a task of executive function) and a measure assessing
HRQL. Caregivers completed a clinical interview and forms evaluating
HRQL, executive functioning, and attention. The impact of relevant
medical variables and socio-economic status (SES) was also considered.
Results: Between 20% and 33% of participants scored ≤ 1 SD below
age-based means on intellectual subtests, and between 31 and 48% scored
≤ 1 SD below grade-based means on academic subtests. Attention concerns
were noted in 25% of patients, while 20-31% of parents reported
executive function concerns. Neither disease subtype nor hemoglobin were
associated with cognitive or academic functioning. There was an
association between mean corpuscular volume (MCV) and both cognitive
abilities and psychosocial health. Cognitive scores were correlated with
SES, suggesting an impact of socioeconomic disparities on performance.
Conclusions: Sickle Cell Disease negatively impacts cognitive and
academic functioning both directly and indirectly due to pain, missed
school, and through socioeconomic and racial disparities. These concerns
may increase over time as disease processes and medication compliance
often worsen. Longitudinal research is needed to further explore these
trends.