Thalassemia

Thalassemia is an inherited blood disorder that causes body to make less hemoglobin or abnormal hemoglobin. Hemoglobin helps red blood cells spread oxygen through your body. Low levels of hemoglobin may cause anemia, an illness that makes you feel weak and tired. Severe anemia can damage organs and lead to death.

There are two main types of Thalassemia. 

1.Alpha Thalassemia
2.Beta Thalassemia



Beta Thalassemia

To make hemoglobin, both alpha and beta globin needed. Beta Thalassemia occurs when one or both of the two genes that make beta-globin don't work or only partly work as they should.

This is called beta Thalassemia minor or beta Thalassemia trait. It happens when you get a normal gene from one parent and a Thalassemia gene from the other and probably won't need treatment. 

When one gene is missing or damaged, your red blood cells might be smaller than normal and have no symptoms and you will not need treatment. But you are a silent carrier. This means you don't have the disease but can pass the defective gene to your child.

When you got a Thalassemia gene from each parent. You may have moderate or severe anemia. If you have moderate anemia (beta thalassemia intermedia), you may need blood transfusions.

People with severe anemia (called beta thalassemia major or Cooley's anemia) need blood transfusions throughout life. Symptoms of anemia usually begin within a few months after birth.


Alpha Thalassemia

This type occurs when one or more of the four alpha-globin genes that make hemoglobin are missing or damaged.

If two genes are missing or damaged: You will have very mild anemia that will typically not need treatment. This is called alpha thalassemia minor or alpha thalassemia trait.

If three genes are missing: You will have mild to moderately severe anemia. This is sometimes called hemoglobin H disease. If it is severe, you may need blood transfusions.

If all four genes are missing: This is called alpha thalassemia major or hydrops fetalis. The fetus will be stillborn, or the child will die soon after birth.



Symptoms & diagnose

Mild Thalassemia usually doesn't cause any symptoms.

Moderate or severe disease may cause symptoms of anemia and children with severe thalassemia may grow slowly (failure to thrive), have skull bones that are not shaped normally, and have problems with feeding, frequent fevers, and diarrhea.

With one or few test the physicians can determine the diagnose the condition. They may include a complete blood count (CBC), a gene test to see if you have the genes that cause thalassemia,a n iron level test, a blood test that measures the amounts of different types of hemoglobin, to help find out which type of thalassemia you have.



Treatments & Side Effects

Treatment depends on how severe your condition is.

  • Mild thalassemia, the most common form, does not need treatment.
  • Moderate thalassemia may be treated with blood transfusions and folic acid supplements. Folic acid is a vitamin that your body needs to produce red blood cells.
  • Severe thalassemia may be treated with
    • Blood transfusions and Folic acid. 
    • Blood or bone marrow stem cell transplant.
    • Surgery to remove the spleen.






When repeating blood transfusions, body get too much iron witch cause to damage heart and other organs. So avoid vitamins that contain iron, and don't take extra vitamin C, which can increase how much iron you absorb from food. If iron level is too much, you will need chelation therapy witch helps remove iron from body.




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