Cognitive Rehabilitation in Sickle Cell Disease

The majority of school-age children with sickle cell disease (SCD) experience neurocognitive deficits, even in the absence of stroke. In particular, deficits in attention and working memory have emerged as two of the most common neurocognitive sequelae of SCD. Thus, the goal of the present proposal is to address feasibility and compliance of a novel computerized cognitive training program, Cogmed. Pilot data will also be collected to establish preliminary efficacy. Twenty-four children meeting initial age and diagnostic criteria will be identified and approached about participation by their attending physician during regularly-scheduled SCD clinic visits. Baseline assessments will include a brief measure of intellectual functioning, a brief cognitive testing battery evaluating processing speed and working memory, in addition to questionnaires regarding behavior and quality of life. Children will then be randomized to the computerized CT program Cogmed (n=12) or a waitlist control (n=12). Participants enrolled in the computerized CT program will be asked to complete 25-sessions of Cogmed over a five to eight week period (3 to 5 sessions per week). Following completion of the program, children and their parents will be asked to return to clinic for a follow-up visit. After a five to eight-week waiting period, children in the waitlist condition will also be asked to return to clinic for a second visit. Following this assessment, participants initially enrolled in the waitlist will be offered an opportunity to participant in the intervention. If interested, they will follow the same intervention protocol described above. These children will return to clinic for a third visit following completion of the intervention. Compliance rate and its confidence interval will be calculated for the overall study population. A t-test for binomial proportion with continuity correction will be used to examine whether the compliance rate is lower than the target. Participants' change in criterion outcomes will be evaluated (i.e., those neurocognitive measures such as attention, executive functioning and working memory, that are most closely related to the trained tasks).

sickle cell disease, pediatric, learning disability, cognitive impairment, memory, attention, Children with sickle cell disease

These children are enrolled in a waitlist condition, after which they will be offered the opportunity to complete the intervention.

Efficacy will be defined by participant performance on cognitive outcome measures including executive functioning outcomes from the Cogstate.

Inclusion Criteria: 1) Children with a diagnosis of SCD (all genotypes) 2) a T-score ≥75th percentile on either the Metacognition or Executive Composite of the BRIEF; and/or 3) a standard score ≥1 standard deviation below the mean (<90) on the tasks of executive function or WM from the Cogstate (mean=100; SD=10) Exclusion Criteria: 1) Estimated IQ ≤ 75), or motor, visual, or auditory handicap that prevents computer use; 2) a diagnosis of depression or a pervasive developmental disorder (by history); 3) clinical stroke (via record medical history); or 4) non-English fluency.