Alpha-Thalassemiain Detail

Alpha-Thalassemia is a type of blood disorder affecting younger children. This disorder affects the way the body makes a particular type of hemoglobin called the alpha globin. With alpha-thalassemia, red blood cells are destroyed at a faster rate than usual. Normally, red blood cells last for a duration of four months before they are destroyed and new red blood cells are produced. With this blood disorder, the red blood cells lifespan is shortened, thereby resulting in an anemic condition that can cause fatigue and other related disorders. This disorder is not contagious in nature and is usually hereditary. If both the parents carry this disorder, there are very good chances of their children being passed on Alpha-Thalassemia.

Genetics

Alpha-Thalassemia occurs when the gene that is responsible for the secretion of alpha globins are either absent or becomes defective in nature. It can be either severe or mild, and usually occurs in children belonging to African, Middle East Asian, South East Asian countries, and China. Children with this type of thalassemia can show symptoms or may not show any symptoms at all. Some children might not require any treatment, but others need blood transfusions at frequent intervals to treat anemia and related disorders.

When a child has Alpha-Thalassemia, there is a mutation in chromosome 16. Alpha globin is made on Chromosome 16. A particular type of gene is required to tell chromosome 16 to generate alpha globin. When this gene is deficient or completely absent, chromosome 16 will not be able to generate Alpha Globin. This in turn affects the overall hemoglobin description in the body. Due to reduced hemoglobin description, red blood cells starts losing the ability to carry blood through the different parts of the body.

There are actually four types of genes that really help in the generation of alpha globin, which leads to four different forms of alpha-thalassemia.

1. When only one of the four genes is missing or is in abnormal state in the child, then the type of disorder is referred to as Silent Alpha-Thalassemia. Children with this type of disorder do not exhibit any symptoms of the disease, but can pass on the disorder to the next generation.

2. Two missing or mutated genes results in a type of condition called Minor Alpha-Thalassemia. Children with this type of disorder have red blood cells that are slightly smaller than the normal ones. These children are also slightly anemic, and thereby suffer from related minor disorders.

3. Three missing or mutate genes results in a condition called Hemoglobin H. Symptoms range from moderate to severe.

4. When all the genes are found missing or become mutated, the condition is called Major Alpha-Thalassemia. This condition might result in the baby dying shortly after delivery. This disease can also be diagnosed prenatally by analyzing the medical condition of the parents. Proper treatment can even save the baby, so it is important to find out whether this condition has been diagnosed in either of the parent’s families in the past.

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Alpha-Thalassemia

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