Thalassemia is a hereditary disease that has a hereditary chromosome inheritance (the parents must be carriers of the disease in order to appear in their children) resulting from an imbalance in the production of the constituent chains of hemoglobin. This imbalance may be a partial inability or total inability to produce one of the constituents of hemoglobin .
• Minimally: Do not need treatment and may be mistakenly diagnosed as anemia due to iron deficiency, iron does not benefit Thalassemia, but it makes the disease worse must be avoided.
• Moderate: avoid iron and take folic acid when needed, and when severe anemia blood is transferred but these patients are not who rely on blood transfusion periodically
• Major: It consists of
1 – blood transfusion periodically (every 2-4 weeks) to maintain hemoglobin more than 9 g / dl
2 – iron steroids such as dysfroxamine and given under the skin by pump for 8-12 hours a day, and the most important side effects of this treatment affect the hearing and vision and must follow up these effects
3 – a large dose of ascorbic acid (vitamin C) to increase the elimination of iron in the urine, as drinking tea reduces the absorption of iron in the bowel
4 – daily dose of folic acid
5 – compensation of hormones in the case of influence on the glands, such as insulin and growth hormone
6. remove the spleen to reduce the need for blood transfusion, and removal are after the age of 6 years to reduce the likelihood of developing blood poisoning after the removal, as the patient is given vaccines against certain types of bacteria (Almaekerobac) and given some drugs Kalpnacelin after removal.
7 – remove the gallbladder at the same time the process of removal of the spleen, and to form the stones consisting of bilirubin.
8 – Bone marrow transplantation for specific children who have a brother or a donor, including tissue matching and preferably transplantation before the age of 3 years. This is considered a cure for the disease.
• In the case of deletion of genes and treatment as in thalassemia beta
• If three genes are removed, treatment is treated as in the central beta thalassemia
1. Oxford handbook of clinical medicine 8th edition
2. Dvidson’s principles and practice of medicine 21st edition
3. illustrated texte of pediatrics -Tom lissauer, Graham Clayden 3rdedition