Academic literature on the topic 'WHO-HBSC'

Sickle cell disease (SCD) is associated with a range of serious complications, including painful episodes, acute chest syndrome, stroke, splenic sequestration, overwhelming bacterial sepsis, and a host of other less frequent complications that impair quality of life and threaten long-term survival (National Institutes of Health [NIH] 2002). More than 98% of all children born in the United States are tested for SCD as part of state newborn screening programs, making it possible to identify infants who are at risk for these many complications of SCD (Centers for Disease Control and Prevention 2009). It is estimated that about 72,000–80,000 individuals of all ages are living with SCD in the United States, a large number of whom will experience cognitive complication at some point in their lives. Nearly 2 million carry sickle trait but do not have the clinical manifestations of the disease. Approximately 1 in 500 infants of African descent and one in 36,000 of Hispanic descent are born with SCD in the United States each year (National Heart, Lung, and Blood Institute (NHLBI), NIH 2009). While the cognitive complications of cerebrovascular infarction (stroke) have long been recognized, research over the last two decades has shown that cognitive functioning in both children and adults can be impaired for many reasons other than stroke. This chapter describes the underlying structural and biological mechanisms that lead to cognitive impairment, the cognitive outcomes associated with SCD, and emerging pharmacologic, behavioral, and educational intervention strategies to address the cognitive needs of this population. Sickle cell disease describes a group of hereditary hemoglobinopathies that have in common the presence of an abnormal hemoglobin variant known as sickle hemoglobin (HbS). The homozygous condition (HbSS) is also the most severe, and is known as sickle cell anemia. Although there are numerous other variations of sickle hemoglobinopathy, the most common include HbSβthal° and HbSC. Under oxidative stress, the sickle hemoglobin causes erythrocytes to collapse into rigid sickle-shaped molecules, with a significant reduction in oxygen-carrying capacity (NIH 2002).