| "It is wise that you and your partner
have testing done to determine the risk of passing on the disorder
to a child" |
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Hemoglobin is made up of two kinds of protein
chains called alpha globin chains and beta globin chains. Individuals
with thalassemia do not produce enough of either of these two protein
chains and as a result, the red blood cells do not form properly
and cannot carry sufficient oxygen throughout the body.
Thalassemia is inherited, that is, they are passed on from parents
to their children through genes. The two main types of thalassemia
are called alpha thalassemia and beta thalassemia.
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Alpha Thalassemia
Four genes are needed to make enough alpha globin protein chains.
Alpha thalassemia occurs when one or more of the four genes
are missing or damaged.
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A person who has one gene missing or damaged
is called a silent carrier. This form of alpha thalassemia does
not cause any major changes in the hemoglobin or any health problems.
Affected individuals generally have no symptoms, but they are carrier
of the disease and they can pass on the genetic abnormality to
their children.
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If two genes are missing or damaged, the condition
is called alpha thalassemia minor, also called alpha thalassemia
trait. People who have the alpha thalassemia trait usually have
no significant health problems except mild anemia, which can cause
slight fatigue.
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If three genes are missing or damaged, it
will result in hemoglobin H disease. The condition causes abnormalities
in red blood cells and rapid destruction of these cells. Most affected
individuals have mild to moderate anemia and should receive regular
medical care.
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The most severe form of alpha thalassemia
is known as alpha thalassemia major or hydrops fetalis. In this
condition, all four genes are missing or damaged. Affected fetuses
are usually miscarried, stillborn or die shortly after birth.
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BetaThalassemia
Two genes are involved in making the beta globin protein chains.
Beta thalassemia occurs when one or both of the two genes are
variant.
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Beta thalassemia minor, also called beta thalassemia
trait occurs when one of the two genes are affected. A person with
this condition will usually experience no health problems other
than a possible mild anemia.
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If both genes are affected, a person may have
beta thalassemia intermedia or beta thalassemia major, also called
Cooley's anemia. People with beta thalassemia intermedia have mild
to moderate anemia. Beta thalassemia major is the most severe form
of beta thalassemia in which the complete lack of beta protein
in the hemoglobin causes a life-threatening anemia that requires
regular blood transfusions and extensive medical care.
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Prevention
Thalassemia is an inherited disease that cannot be prevented. However,
blood tests and family genetic studies can show whether an
individual has thalassemia or is a carrier. It is wise that
you and your partner have testing done to determine your hemoglobin
status if you're thinking of having children. This will help
determine your risk of passing on the disorder to a child.
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Disclaimer:
While every effort has been made to ensure accuracy of facts, the
articles and information above must never be construed as giving
professional health advice and as such Kurnia does not give any
warranty on accuracy, completeness, functionality, usefulness
or other assurances as to the content appearing in the above
article. Kurnia cannot be held responsible for any losses, injury
or death resulting from the use of the above information. |
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