Thalassemia

Definition of Thalassemia

Thalassemia, also known as Mediterranean Anemia, Cooley's Anemia or Homozygous Beta Thalassemia, is a group of inherited disorders in which there is a fault in the production of hemoglobin (oxygen-carrying pigment found in red blood cells).

Description of Thalassemia

Blood is red because the red blood cells contain an oxygen-carrying substance called hemoglobin. The principal function of hemoglobin is to combine with and transport oxygen from the lungs and deliver it to all body tissues, where it is required to provide energy for the chemical reaction of all living cells.

Hemoglobin contains a large amount of iron. When red blood cells are broken down, most of the iron from the hemoglobin is used again to make new hemoglobin.

In the case of thalassemia the hemoglobin is fragile and breaks down sooner than normal, thus leaving the person with not enough hemoglobin in their body. This lack of hemoglobin causes anemia.

There are different types of anemia. The most common is iron-deficiency anemia. This happens when people do not have enough hemoglobin because they're not eating enough of the foods that contain iron (See Health Profile on Anemia).

Thalassemia is a different type of anemia. This happens when people do not have enough hemoglobin and is caused by the inheritance of a defective gene.

There are two forms of thalassemia:

Thalassemia trait

People with thalassemia trait carry thalassemia, but they are not ill. They are healthy and normal, however, some may have slight anemia.

People with thalassemia trait also have slightly more hemoglobin called hemoglobin A2 in their blood.

Thalassemia trait is present at birth, it remains the same for life, and it can be handed down from parents to children.

Thalassemia major
This a very serious blood disease that begins in early childhood.
Children with thalassemia major are normal at birth but become anemic between the age of three months and eighteen months. They become pale, do not sleep well, do not want to eat, and may vomit frequently after feedings.
If thalassemia major goes untreated, children usually die between one and eight years of age.