Diagnosis and management of sickle cell disorders

Sickle cell disease (SCD) is a wide-spread inherited hemolytic anemia that is due to a point mutation leading to a valine/glutamic acid substitution in the beta-globin chain, causing a spectrum of clinical manifestations in addition to hemolysis and anemia. Acute painful crisis is a common sequela that can cause significant morbidity and negatively impact the patient's quality of life. Remarkable improvements in our understanding of the pathogenesis of this clinical syndrome and the role of cell adhesion, inflammation, and coagulation in acute painful crisis have led to changes in the management of pain. Due to the endemic nature of SCD in various parts of the Middle East, a group of physicians and scientists from the United States and Middle East recently met to draw up a set of suggested guidelines for the management of acute painful crisis that are reflective of local and international experience. This chapter brings together a detailed etiology, pathophysiology, and clinical presentation of SCD, including the differential diagnoses of pain associated with the disease, with evidence-based recommendations for pain management and the potential impact of low-molecular weight heparin (LMWH), from the perspective of physicians and scientists with long-term experience in the management of a large number of SCD patients.