What is the best intervention to prevent painful crises in patients with sickle cell disease other than hydration and analgesics?

The best intervention to prevent painful crises in patients with sickle cell disease is hydroxyurea. Hydroxyurea works by increasing the production of fetal hemoglobin (HbF), which in turn helps to reduce the sickling of red blood cells. Higher levels of HbF can lead to fewer hemolytic episodes and a reduced frequency of painful crises. This effect is important in managing sickle cell disease because painful crises are caused by the sickling of red blood cells, which leads to vaso-occlusive episodes that result in severe pain.

Additionally, hydroxyurea has shown to decrease other complications of sickle cell disease, such as acute chest syndrome and the need for blood transfusions, providing a more comprehensive approach to managing the disease. Its ability to provide long-term benefits and improve the overall health of patients makes it a key intervention apart from hydration and analgesics.

Other interventions, such as blood transfusion and corticosteroids, play different roles in managing complications of sickle cell disease rather than preventing painful crises. Blood transfusions can be used in crises or to prevent complications but do not serve as a long-term preventive measure. Corticosteroids can help with inflammation but are not typically employed for preventing painful crises in sickle cell