Jumat, 15 Januari 2010



Some people may be unfamiliar with Thalassemia. Thalassemia is genetic disorder. People with Thalassemia, the age of red blood cells is very short, usually under or half the normal number of 120. This is due to defect in one gene of hemoglobin-forming substance ( globin gene alpha or globin gene beta ), so the the production of hemoglobin decrease or none. This disorder causes red blood cells getting smaller and not able to take the oxygen and break easily. To keep the normal condition, patients requiring blood subsidies once a month at least, because if not, they will be weak and lead to death.

Giving blood transfusions can affect the accumulation of iron that can lead to the possibility of heart damaged, liver and hormone glands. This iron accumulation can be anticipated by giving deferoxamine infusion for 8 – 12 hours per day in 5 to 7 a week. This treatment is very expensive. Luckily it’s been found the oral medicine deferasirox that consumed once a day.

Thalassemia Minor and Major

Thalassemia minor also called Trait Thalassemia / congenital. Patients with Thalassemia look like a normal person with a little anemic, but their children will have Thalassemia Major.

Major Thalassemia is inherited anemia. Marriage among Thalassemia gene carrier will produce Thalassemia Major pedigree which until now have not found the cure. Treatment can be done is getting blood transfusion for at least 4 weeks.

Symptoms of Thalassemia

The main symptoms are anemia ( the shortage of red blood cells accompanied by other complications in the system of production and distribution ( system hemopoetic ). Another symptoms, like : pale skin color most likely to turn blue, even blackened, Mongoloid facial ( facies cooley ), ther is jaundice, splenomegali and hepatomegaly caused enlarged abdomen ( the influence of lien and liver enlargement ), growth disorders and appetite disorders. On the type of Beta-Thalassemia Major, patients can have Jaundice, ulcers, gallstones and enlarged spleen. Children with Thalassemia will have growth disorders and enlargement bone thickening especially the head and facial bones. Its become weak and break easily, can occur if bone marrow is too active.

Handling of Thalassemia

The most effective treatment is getting blood transfusion of a lifetime and maintaining the Hemoglobin levels is always equal to or above 12 g/dl and anticipates the side effects. Bone marrow transplant also can be done. Bone marrow tissue of patients is replaced by the same bone marrow tissue of donor. In this case the cost is very expensive. Ironically, almost all Thalassemia patient is come from the poor families. And the other problem is the thalassemia patients have to share blood with accident victims also desperately need more blood. So to help people with thalassemia is not only a material but can also just give sympathy and sincere smile.

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