Current management of sickle cell anemia

Abstract

Proper management of sickle cell anemia (SCA) begins with establishing the correct diagnosis early in life, ideally during the newborn period. The identification of affected infants by neonatal screening programs allows early initiation of prophylactic penicillin and pneumococcal immunizations, which help prevent overwhelming sepsis. Ongoing education of families promotes the early recognition of disease-released complications, which allows prompt and appropriate medical evaluation and therapeutic intervention. Periodic evaluation by trained specialists helps provide comprehensive care, including transcranial Doppler examinations to identify children at risk for primary stroke, plus assessments for other parenchymal organ damage as patients become teens and adults. Treatment approaches that previously highlighted acute vaso-occlusive events are now evolving to the concept of preventive therapy. Liberalized use of blood transfusions and early consideration of hydroxyurea treatment represent a new treatment paradigm for SCA management.

Figure 1.

Survival curves of infants with SCA in the United States and Jamaica, by era. This research was originally published in Blood. (From Quinn et al. 2010; reprinted, with permission, © American Society of Hematology.)

Figure 2.

Isoelectric focusing (IEF) electrophoresis technique for identification of SCA. Blood specimens from AA, AS, and SS patient controls are shown on the left, along with a manufactured Hb AFSC control in the center. Newborn samples, typically obtained from dried blood spots, are tested for the abnormal FS pattern indicating SCA, versus the normal FA pattern or FAS pattern indicating sickle cell trait.

Figure 3.

Algorithm for the management of painful vaso-occlusive events.

Figure 4.

Transcranial Doppler screening algorithm for children with SCA.

Figure 5.

Blood smear changes with hydroxyurea therapy. A illustrates the untreated patient with anemia and numerous sickled forms; B is after initiation of hydroxyurea treatment with macrocytosis and more target cells; and C is after reaching a stable hydroxyurea MTD with less anemia and fewer sickled forms. (From Ware 2010b; reprinted, with permission.)