Psychosocial and Neurocognitive Aspects of Sickle Cell Disease.

Abstract

Sickle cell disease (SCD) comprises a group of recessively inherited blood disorders and is the most common genetic disorder in the world (Embury et al., 1994). It is a chronic condition of variable severity that mainly affects people of African and African-Caribbean heritage. Over the last 20 years life expectancy has increased significantly, particularly for patients receiving western healthcare so that increasing attention has been directed to the psychosocial adaptation and neurocognitive profile of children and adolescents with SCD. Previously, research suggested that, like children with other chronic health conditions, children and adolescents with SCD were at increased risk for emotional and behavioural disorders. More recent research has not demonstrated increased rates of such difficulties. Increasing evidence suggests that children with severe disease are, however, at significantly increased risk for cerebro-vascular events and neurocognitive difficulties. This paper reviews the literature regarding the psychological and neurocognitive functioning of children with SCD and outlines a number of ways that psychological input may significantly contribute to more effective health care for these children and their families.