Essential strategies and tactics for managing sickle cell disease
Key to patients’ well-being is the family physician’s watchfulness—through periodic lab testing and health checks and diligent application of preventive measures.
PRACTICE RECOMMENDATIONS
› Offer rapid access to narcotic analgesia for patients with sickle cell disease (SCD) who have recurrent vaso-occlusive crises, to prevent unnecessary hospitalization. A
› Provide oral penicillin prophylaxis against pneumococcal disease in patients < 5 years of age who have sickle cell anemia (SCA), but not in children whose SCD is less severe. A
› Screen all patients with SCA annually, beginning at age 2 years until age 16 years, for their risk of stroke, using a transcranial Doppler study. A
› Administer the COVID-19 mRNA vaccine series to all patients with SCD, unless contraindicated. A
Strength of recommendation (SOR)
A Good-quality patient-oriented evidence
B Inconsistent or limited-quality patient-oriented evidence
C Consensus, usual practice, opinion, disease-oriented evidence, case series
Chronic anemia can 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
- serologic testing for HIV.
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