Cognitive functioning in young adult patients with sickle cell disease

Many patients with sickle cell disease (SCD) experience cognitive problems, related to intellectual and executive functioning as well as processing speed. Previous studies focused mostly on children. Consequently there is a general lack of information on the cognitive functioning of young adult patients, while these patients have to make important decisions regarding study, work and social life. More knowledge on the cognitive functioning of young adult patients may help to better tailor the health care of this group of patients to prevent them from stagnating in society. Methods. Ten young adult patients with SCD (HbSS; mean age 22 years) attending the AMC sickle cell clinic were included in the study. The control group consisted of 20 healthy volunteers of the same age and ethnic background (mean age 23 years). Neuropsychological tests were conducted to measure intelligence, executive functioning, memory, spatial ability, language, and psychomotor processing speed. Results. It was found that the SCD patients scored significantly lower than the controls in the areas of intellectual and executive functioning. Significant differences in cognitive functioning were also observed between the individual patients. Conclusion. In this study it was established that cognitive impairment in patients with SCD may extend into young adulthood. Our findings emphasize the need for psycho-education, early screening of cognitive functioning in patients with SCD and the implementation of short rehabilitation programs