Patient Encounter 1

An 18-month-old female is presenting for a routine check-up. She was diagnosed with sickle cell anemia at birth, identified through neonatal screening. Today, her hemoglobin is 8.6 g/dL (86 g/L or 5.3 mmol/L) and she is afebrile.

What acute complications is she at risk for developing?

What preventative treatment should she receive?

What additional information do you need to know before creating a plan for this patient?

Folic Acid

Folic acid supplementation with 1 mg daily is generally recommended in adult SCD patients, women considering pregnancy, and any SCD patient with chronic hemolysis.5 Because of accelerated erythropoiesis, these patients have an increased need for folic acid. There are conflicting studies in the SCD population, especially among infants and children, but if the child has chronic hemolysis, supplementation is recom-mended.18

Fetal Hemoglobin Inducers

Fetal hemoglobin (HbF) induction in patients with SCD, especially those with frequent crises, has been shown to decrease RBC sickling and RBC adhesion. A direct relationship between HbF concentrations and the severity of disease have been demonstrated in studies.2

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