ALPHA THALASSEMIA HB H

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ALPHA THALASSEMIA HB H
« on: June 13, 2011, 11:27:51 AM »
The loss of three alpha genes produces a serious hematological problem (three-gene deletion alpha thalassemia). Patients with this condition have a severe anemia, and often require blood transfusions to survive. The severe imbalance between the alpha chain production (now powered by one gene, instead of four) and beta chain production (which is normal) causes an accumulation of beta chains inside the red blood cells. Normally, beta chains pair only with alpha chains. With three-gene deletion alpha thalassemia, however, beta chains begin to associate in groups of four, producing an abnormal hemoglobin, called "hemoglobin H". The condition is called "hemoglobin H disease". Hemoglobin H has two problems. First it does not carry oxygen properly, making it functionally useless to the cell. Second, hemoglobin H protein damages the membrane that surrounds the red cell, accelerating cell destruction. The combination of the very low production of alpha chains and destruction of red cells in hemoglobin H disease produces a severe, life-threatening anemia. Untreated, most patients die in childhood or early adolescence.

My 3 yr boy was sick last 20 oF May due to gastoenteritis ,admitted in the hospital and on the 21 of May they found out that he have thalassemia -alpha thalassemia but last Friday he was seen by the Pedia Hematology and they explain to me that my wee boy have the HB H type which mean me n my hubby are thal carrier as well so they check our bloods to finds out who have 2 alpha genes missing n who has the 1 alpha genes missing... im working on saturday night so i have a chance to look to the laboratotory results then i found out that my husband has mcv n mch low , while mine are all normal... im still waitin for all the test to come back..

My sons heamoglobin from 8.1 , n now its 8.5 i dunno if he is goin to be tranfuse or not... he is awaitin echo as well as he have a heart murmur ??? due to anemia or he have a valve problem... i hope he would never get a lupus cause its too much already .. i have a lupus diagnose 1998 and this is my third life.. i hope my son is as strong as me n a survivor as well..

when the doctor examine him last friday i was shock as he is so cooperative , they dnt have problems in getting bloods from him n he never fights back or tried to puledl his hands away..

 

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