What is the primary treatment for sickle cell anemia to increase fetal hemoglobin?

The primary treatment for sickle cell anemia aimed at increasing fetal hemoglobin (HbF) levels is hydroxyurea. Hydroxyurea functions by stimulating the production of fetal hemoglobin, which can effectively reduce the sickling of red blood cells and potentially decrease the frequency and severity of pain crises and other complications associated with the disease.

Fetal hemoglobin has a different structure compared to adult hemoglobin, and it does not sickle, thereby providing a protective effect against the sickling phenomenon seen in this condition. By increasing the levels of HbF, hydroxyurea can lead to a lower concentration of sickle hemoglobin in the blood, thereby alleviating many of the symptoms and complications of sickle cell anemia, such as vaso-occlusive crises and acute chest syndrome.

Other treatments, while they may serve different purposes in managing sickle cell anemia or other conditions, do not play a role in increasing fetal hemoglobin. For example, lithium is primarily used in the treatment of bipolar disorder, naftifine is an antifungal medication, and vancomycin is an antibiotic. None of these medications have a mechanism to raise fetal hemoglobin levels as hydroxyurea does. Thus, hydroxyurea is the cornerstone