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THALASSEMIA
Filed under DiseaseFeb 7
Thalassemia comes from the Greek, Thalassa (sea) and emia (blood). This disease was first discovered in the Mediterranean region, Mediterranean, by Doctor Cooley, thus also known as Cooley’s anemia or Mediterannean anemia. Thalassemia is a genetic disorder inherited from both parents. This disorder is not a reduction or formation of proteins needed to form grains of red blood cells (erythrocytes), so the age of erythrocytes becomes shorter and easily broken.Normal erythrocytes aged 120 days or approximately four months, after which erythrocytes are broken and replaced with new ones. In children, thalassemia due to fragile red blood cells causes the child looks pale, had less blood (anemia) and require a red blood transfusion.
Types of thalassemia
Clinically, thalassemia divided into four, namely:
- Thalassemia Major
Patients who require regular blood transfusions every month. Patients with thalassemia major thalassemia gene have both parents. - Thalassemia Intermedia
Patients need a blood transfusion but not routinely, every 3-4 months. - Thalassemia Minor
Patients do not need a transfusion and a normal life, but the blood looks red blood cell size smaller than normal. - Based on the genetic
That depends of the type of protein chain is missing. If the missing alpha chain is called alpha thalassemia, and if it is missing is the beta, is called beta thalassemia.
Symptoms that appear depend on the severity of proteins forming hemoglobin abnormalities, most came from the ages of two months until the age of the child, rarely came as a teenager. Generally the child looked pale, enlarged abdomen due to enlargement of the spleen is working actively to help the bone marrow to blood to meet the needs of the entire body. The eyes appear yellow, seemed shorter than their peers, and his skin was black.
Until now thalassemia can be cured yet. Current treatments can be done only prolong life and improve quality of life of patients such as blood transfusions. Red blood transfusion on average 1 time per month on a regular basis for a lifetime.
But these actions risk of contracting infectious diseases due to transfusion, such as hepatitis B, C, and even HIV. Moreover, it can happen buildup of iron in all organs, especially the heart muscle, liver, lymph-forming hormone. Excessive iron that would make organ dysfunction, and should be removed with medication. There is injected under the skin of the abdomen for 8-12 hours / day, for 5-7 times / week, all his life.
There is also a drug in tablet form that also must be taken every day. We can imagine how painful and boring. But if not, the excess iron that would interfere with organ function, and can cause heart problems, absence of signs of puberty, so munculah infertility problems. Also change the face bones, teeth crooked, resulting in osteoporosis, the bone can be broken spontaneously.
In addition, patients should avoid iron-rich foods, especially red meat and liver. Expand calcium-rich food such as milk, cheese, food from the type of wheat, and tea that serves to inhibit the absorption of iron.
- Thalassemia Major
4 Responses to “THALASSEMIA”
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