In managing a child with sickle cell disease, which therapy would you include?

• A. Increased hydration
• B. Hydroxyurea
• C. Morphine to help with pain crises
• D. Acyclovir to prevent viral infection

Answer: (B)

Hydroxyurea is a disease-modifying therapy for sickle cell disease in children. It works by increasing fetal hemoglobin (HbF) production in red blood cells. HbF does not sickle, so higher HbF levels reduce the tendency of hemoglobin S to polymerize when deoxygenated. That means the red cells are less prone to sickling, which lowers the frequency of painful vaso-occlusive crises, reduces the risk of acute chest syndrome, and can lessen overall disease morbidity.

In a pediatric patient, hydroxyurea is the best option among the choices for ongoing management because it addresses the underlying disease process rather than just providing symptomatic relief. Hydration supports acute care and pain control is essential for crises, but these are not disease-modifying strategies. Acyclovir is not a standard preventive measure for viral infections in sickle cell disease, and while morphine is important for acute pain management, it does not prevent crises.