Biological interventions to improve cognition in children with sickle cell disease.

Children with sickle cell disease (SCD) experience widespread cognitive deficits along with numerous other medical consequences including stroke, acute chest syndrome, and premature death. Molecular changes within sickled cells reduce the oxygen-carrying capacity of the blood, but the specific mechanisms by which cognitive deficits occur are not yet fully understood. Therefore, cognition remains a critical target, but currently, biological interventions for children with SCD aim to improve health and disease outcomes, but none are aimed at improving cognition. The goal of the present study was to determine whether blood transfusion, an essential and life-saving biological intervention which increases oxygen carrying capacity, would improve cognition in children with SCD. Our study focused on executive abilities given their importance in school readiness and academic performance and had three specific aims. The first aim assessed for differences in executive abilities between children with SCD receiving transfusion (both near and far from transfusion), children with SCD receiving Hydroxyurea (HU; a commonly used drug that increases overall blood flow), and a cohort of healthy children tested at two time points. The second aim assessed the relationship between behavioral functioning and cognition in children with SCD. Finally, the third aim assessed the relationship between blood biomarkers of SCD and executive abilities in children receiving transfusion. The results of the study found that children with SCD (transfusion and HU) had poorer executive abilities than healthy children. Importantly, children near transfusion (high oxygen delivery) had improved executive abilities compared to far from transfusion (poor oxygen delivery). They also had better executive abilities near transfusion than children with SCD receiving HU at their first test session, despite having greater disease severity. Health-related quality of life emerged as the aspect of behavioral functioning most strongly related to executive abilities. Lastly, when blood biomarkers remained high over a 4--6 week interval (e.g., hemoglobin) executive abilities remained high further from transfusion. Our study demonstrates that executive abilities are vulnerable, yet malleable in children with SCD receiving transfusion. The findings provide new information that could influence the development of future treatment options tailored to the specific needs of this population struggling with SCD. (PsycINFO Database Record (c) 2018 APA, all rights reserved)