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Medication for Beta-thalassemia

Hi, I am Brajesh kumar, age-- 17 years , Gender -- Male , I am a patient of Bita -Thalassimia, I want to know about the medicine of my disease.
Asked On : Mon, 19 Jul 2010
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General & Family Physician 's  Response
Are you having any symptoms from the disease?
Thalassemias are inherited disorders caused by various gene mutations.Several clinical forms of β thalassemia are recognized; some of the more common forms are as follows:
Silent carrier β thalassemia: these patients have no symptoms, except for possible low RBC indices.
β thalassemia trait: Patients have mild anemia, abnormal RBC indices, and abnormal Hb electrophoresis results with elevated levels of Hb A2, Hb F, or both.
Thalassemia intermedia: This condition results in anemia of intermediate severity, which typically does not require regular blood transfusions.

Thalassemia major (Cooley anemia): This condition is characterized by transfusion-dependent anemia, massive splenomegaly, bone deformities, growth retardation, and peculiar facies in untreated individuals, 80% of whom die within the first 5 years of life from complications of anemia.

Coming to the treatment part -
Patients with thalassemia traits do not require medical or follow-up care after the initial diagnosis is made.
Patients with severe thalassemia require medical treatment, and a blood transfusion regimen was the first measure effective in prolonging life.

Hematopoietic stem cell transplantation (HSCT)-HSCT is recommended only for selected patients; it is the only known curative treatment for thalassemia

Splenectomy (removal of spleen)is the principal surgical procedure used for many patients with thalassemia.
A normal diet is recommended, with emphasis on the following supplements: folic acid, small doses of ascorbic acid (vitamin C), and alpha-tocopherol (vitamin E).
Medications needed for the treatment of various types of thalassemias are nonspecific and only supportive like Hydroxyurea ,Haemophilus b conjugate vaccine ,Folic acid, Vitamin C,Hydrocortisone ,Deferasirox,Diphenhydramine hydrochloride,Acetaminophen ,Somatropin etc
Answered: Wed, 21 Jul 2010
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