Chronic anemiacan be managed with transfusion when elevating the Hb level is required (eg, preoperatively, to prevent stroke, to manage priapism). For some patients, ongoing transfusion is required; care should be taken to avoid iron overload and hemolysis due to antibody formation. Ongoing surveillance for these complications is required.6
Other chronic problems.Patients with SCD who develop avascular necrosis, vaso-occlusive ulcers, pulmonary hypertension, renal disease, recurrent priapism, or ophthalmologic complications should be co-managed with a care team.6
Pharmacotherapy and SCA
A principal goal in the management of patients with SCA is prevention of vaso-occlusive events, including ACS and acute pain crises.
Hydroxyurea, a key component of SCA treatment, is a ribonucleotide reductase inhibitor that increases the level of Hb F, thus reducing the absolute number of symptomatic vaso-occlusive events and increasing arterial blood flow. It is most useful for patients who have multiple crises. The drug prolongs survival and reduces the need for transfusion and hospitalization.4,5
Hydroxyurea can be started in patients at age 9 months; blood testing should be performed at the start of treatment and the dosage titrated based on blood counts. Initial blood work includes:
Hb level;
Hb electrophoresis with the quantitative percentage of Hb F;
complete blood count with differential and reticulocyte counts;
chemistry profile (electrolytes, lactate dehydrogenase, total protein, albumin, total bilirubin);
liver function tests (aspartate aminotransferase, alanine aminotransferase);
measurement of renal function (blood urea nitrogen, creatinine);
serum vitamin B12 and folate;
serum iron, total iron-binding capacity, and ferritin;
hepatitis B, hepatitis C, and parvovirus B19 antigen; and
