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Health maintenance for patients with sickle cell disease starts with early diagnosis, preferably in the newborn period, and includes penicillin prophylaxis, vaccination against pneumococcus bacteria, and folic acid supplementation. Treatment of complications often includes antibiotics, pain management, intravenous fluids, blood transfusion, and surgery, all backed by psychosocial support. Like all patients with chronic disease, patients are best managed in a comprehensive, multidisciplinary program of care.

Blood transfusions help benefit sickle cell disease patients by reducing recurrent pain crises, risk of stroke, and other complications. Because red blood cells contain iron, and there is no natural way for the body to eliminate it, patients who receive repeated blood transfusions can accumulate iron in the body until it reaches toxic levels. It is important to remove excess iron from the body because it can gather in the heart, liver, and other organs and may lead to organ damage. Treatments are available to eliminate iron overload.

A single randomized trial (MSH) of 299 patients with a follow-up of 21 months demonstrated that compared to placebo. Hydroxyurea was associated with lower annual rates of pain crises, longer time to first and second pain episodes, the need for transfusions, and reduced frequency of acute chest syndrome. Droxia, the prescription form of hydroxyurea, was approved by the FDA in 1998 and is now available for adult patients with sickle cell anemia. Hydroxyurea is also used in children.

A complication of blood transfusions is transfusional iron overload. The body does not have a way to get rid of iron received from blood transfusions. An agent that chelates iron is therefore needed to get rid of excess iron in the body. 

There are two agents. One agent is given intravenously, and the other is by mouth. Iron buildup is

Patients with sickle-cell disease (SCD) receiving chronic transfusions of red blood cells are at risk of developing transfusional iron overload over time. Transfusional iron overload is characterized by an increase of labile plasma iron (i.e., non-transferrin-bound iron) in the body, which can lead to functional impairment in vital organs. The organs that are at risk of damage due to transfusional iron overload include the liver, heart, pancreas, thyroid, pituitary gland, and other endocrine organs. A buildup of labile plasma iron in these organs can lead to hepatic cirrhosis, cardiomyopathy, diabetes mellitus, hypoparathyroidism, impaired growth, infertility, and hypogonadism.