Thalassemia

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Treatment

Most individuals with thalassemia traits require no treatment. They may want to consider genetic counseling, however, because they may pass the trait on to their children.

People with hemoglobin H disease or beta thalassemia intermedia will experience variable amounts of anemia throughout their life. They can lead relatively normal lives but will require regular monitoring and may occasionally need a blood transfusion. Folic acid supplementation is often given to help combat anemia, but iron supplementation is not recommended.

Those with beta thalassemia major will usually require blood transfusions about every 3 or 4 weeks throughout their life. These transfusions help maintain hemoglobin at a high enough concentration to provide oxygen to the body and prevent growth abnormalities and organ damage. Frequent transfusions, however, can raise iron to toxic levels, resulting in deposits of iron in the liver, heart, and other organs. Regular iron chelation therapy is used to help decrease iron in the body. This involves the administration of a drug that binds with the iron and helps flush it out of the body through the urine. Surgery to remove the spleen (a splenectomy) may also be required.

Bone marrow transplant is also used for treatment of beta thalassemia major.

Fetuses with alpha thalassemia major are usually miscarried, stillborn, or die shortly after birth. Experimental treatments, such as fetal blood transfusions and even fetal marrow transplant, have been successful in a very few cases in bringing a baby to term.

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