Pain Crises in Sickle Cell Disease: A Clinical Guide to Prevention and Treatment
Defining Sickle Cell Disease Sickle cell disease (SCD) is the consequence of homozygosity for a single amino acid change in the beta-globin chain that results in structurally abnormal hemoglobin S, or by compound heterozygosity for hemoglobin S and another β-globin chain abnormality, typically hemoglobin C or beta-0 thalassemia. Hemoglobin SS (HbSS) and HbS beta-0 thalassemia present in a similar clinical manner and are commonly referred to together as sickle cell anemia (SCA), which is the most severe subtype of SCD. 1,2 The sickling process causes secondary changes in cell shape, size, ca...