PROJECT SUMMARY / ABSTRACT
The primary objective of this study is to evaluate the feasibility of a novel approach to ameliorating cognitive
deficits in children and adolescents with sickle cell disease (SCD). SCD is an understudied, life-limiting disease
affecting 100,000 individuals in the United States and over 300,000 newborns globally every year. Youth with
SCD are at substantial neurological risk given their increased likelihood of overt stroke and silent cerebral infarct.
However, even individuals without signs of cerebral infarct exhibit lower cognitive performance compared to
healthy siblings due in large part to non-stroke, disease effects on the central nervous system. Disease-related
cognitive issues are diffuse but often affect working memory function. Very few studies have examined
therapeutic approaches to treating cognitive deficits in SCD; however, recent research supports the use of a
home-based, eHealth working memory training program (Cogmed). In a randomized controlled trial, Cogmed
training (i.e., repeated and adaptive working memory skills practice) resulted in improved working memory but
adherence to the intervention was low. Early engagement with the training program emerged as predictive of
better adherence. Therefore, in an effort to promote engagement, an enhanced version of the program was
developed that incorporates social support components (e.g., multifamily Cogmed Kickoff events, social
incentives, peer navigator support) and memory strategy training. The study will evaluate the following specific
aims in a single-arm pilot clinical trial: 1) Assess the feasibility of Cogmed+ (Cogmed plus social support
components and memory strategy training) in youth with SCD; and 2) Determine the effect of Cogmed+ on rates
of treatment adherence. In this study, 50 youth ages 7-16 with SCD will complete a cognitive screening, after
which 30 participants with working memory deficits will proceed to the Cogmed+ program. Participants will
complete Cogmed on a tablet device from home 3-5 times per week for 5-8 weeks. Participants will attend weekly
videoconference calls with a Cogmed coach who will review training progress and teach memory strategies
during four coaching calls (weeks 2-5). Outcomes will be assessed by examining the number of Cogmed
sessions completed and rates of attendance to Cogmed Kickoff events and memory strategy training sessions.
Results will also be compared to a historical comparison group of youth with SCD who completed the basic
version of Cogmed. Findings will provide critical data regarding a novel strategy for improving access to an
efficacious treatment for youth with SCD. If such an augmented approach proves beneficial, resultant data will
inform a randomized controlled trial to examine the effectiveness of a stepped care model that selectively delivers
more intensive Cogmed treatment packages only as needed. This line of investigation, which balances practical
access and resource considerations, could serve as a scalable care model and ignite efforts to address SCD-
related cognitive impairment for the millions of people living with SCD globally.
