Thalassemia

Thalassemia is an inherited blood disorder that causes the body to produce fewer healthy blood cells and less hemoglobin and iron-rich protein in red blood cells. There are two types of thalassemia.

• Alpha thalassemia

Alpha thalassemia major or hydrops fetalis is known as the most severe form of alpha thalassemia.

• Beta-thalassemia

The most severe form of beta-thalassemia is known as beta-thalassemia major or Cooley’s anemia.

Causes

Haemoglobin in red blood cells has two kinds of protein chains,

<!--[if !supportLists]-->·         <!--[endif]-->Alpha-globin

<!--[if !supportLists]-->·         <!--[endif]-->Beta-globin

If your body is unable to make enough of these protein chains, red blood cells will not be formed properly and will not be able to carry enough oxygen. Genes in our body control how our body makes haemoglobin protein chains. When these chains are missing or altered, thalassemia occurs.

Signs and symptoms

 Symptoms of thalassemia are caused by a lack of oxygen in the bloodstream because in thalassemia the body doesn’t make enough healthy red blood cells and haemoglobin. Some of the most common symptoms of thalassemia are:

•             Anemia
•             Slowed growth
•             Delayed puberty
•             Bone problems
•             An enlarged spleen

Treatment:

Treatment of thalassemia depends on the type and severity of the problem. People with alpha and beta-thalassemia probably don’t need treatment. But if their problem is major, then there are three treatments available for them.

1.            Frequent blood transfusion.
2.            Chelation therapy.
3.            Stem cell transplant