Disease of Thalassemia

Survival and quality of life are now much better for people who have moderate or severe forms of thalassemia. This is because: * More people are able to get blood transfusions now. * Blood screening has reduced the number of infections from blood transfusions. Also, treatments for other kinds of infections have improved. * New iron chelation treatments are available that are easier for some people to take. * Some people have been cured through blood and marrow stem cell transplants. Living with thalassemia can be challenging, but several approaches can help you cope. Follow Your Treatment Plan It's important to follow the treatment plan your doctor gives you. Get blood transfusions as he or she recommends. Take your iron chelation medicine. This is important because the leading cause of death among people with thalassemias is heart disease caused by iron overload. Iron buildup can damage your heart, liver, and other organs. Although the iron chelation treatment can ...

Two genes (one from each parent) are needed to make enough beta globin protein chains. If one or both of these genes are altered, you will have beta thalassemia. This means that you don't make enough beta globin protein. If you have one altered gene, you're a carrier. This condition is called beta thalassemia trait or beta thalassemia minor. It causes mild anemia. If both genes are altered, you will have beta thalassemia intermedia or beta thalassemia major (also called Cooley's anemia). The intermedia form of the disorder causes moderate anemia. The major form causes severe anemia.

Four genes (two from each parent) are needed to make enough alpha globin protein chains. If one or more of the genes is missing, you will have alpha thalassemia trait or disease. This means that you don't make enough alpha globin protein. * If you have only one missing gene, you're a silent carrier and won't have any signs of illness. * If you have two missing genes, you have alpha thalassemia trait (also called alpha thalassemia minor). You may have mild anemia. * If you have three missing genes, you likely will have hemoglobin H disease (which a blood test can detect). This form of thalassemia causes moderate to severe anemia. Very rarely, a baby will have all four genes missing. This condition is called alpha thalassemia major or hydrops fetalis. Babies with hydrops fetalis usually die before or shortly after birth.

* Thalassemias are inherited blood disorders. "Inherited" means they're passed on from parents to children. * Thalassemias cause the body to make fewer healthy red blood cells and less hemoglobin than normal. * People who have thalassemias can have mild or severe anemia. This condition is caused by a lower than normal number of red blood cells or not enough hemoglobin in the red blood cells. * The two major types of thalassemia are alpha thalassemia and beta thalassemia. There are different forms of each type. * Thalassemias occur when the genes that control the production of hemoglobin are missing or altered. Your body won't work properly if your red blood cells don't make enough healthy hemoglobin. * Family history and ancestry are the two risk factors for thalassemias. If your parents have missing or altered hemoglobin-making genes, you may have thalassemia. Thalassemias occur most often among people of Italian, Greek, Middle Eastern, Asia...

Your body makes three types of blood cells: red blood cells, white blood cells, and platelets (PLATE-lets). Red blood cells contain hemoglobin, an iron-rich protein that carries oxygen from your lungs to all parts of your body. Hemoglobin also carries carbon dioxide (a waste gas) from your body to your lungs to be exhaled. Hemoglobin has two kinds of protein chains: alpha globin and beta globin. If your body doesn't make enough of these protein chains, red blood cells don't form properly and can't carry enough oxygen. Your body won't work well if your red blood cells don't make enough healthy hemoglobin. Genes control how the body makes hemoglobin protein chains. When these genes are missing or altered, thalassemias occur. Thalassemias are inherited disorders. That is, they're passed on from parents to their children through genes. People who get abnormal hemoglobin genes from one parent but normal genes from the other are called carriers. Carriers often have no...