THALASSEMIA

आपकी अज्ञानता ही आपके बच्चे को जीवनभर रक्त चढ़वाने पर मजबूर कर सकती है।

1. Thalassaemia is a hereditary disease in which defective hemoglobin is produced which is quickly destroyed in the body.

Thalassemia is primarily of two types:



  • Thalassemia Major
  • Thalassemia Minor

2. Thalassemia major patients cannot synthesise viable RBCs in their body. These patients depend upon regular (every 3-4 weeks) blood transfusions periodically throughout their lives for survival.

3. Thalassemia minor persons possess disease causing thalassemia gene in their body but do not suffer from the disease. These persons are capable of transmitting this defective gene to their offsprings. They lead apparently normal lives and just suffer from mild anaemia.



EMOTIONAL AND FINANCIAL BURDEN:

4. Due to repeated transfusions, there is iron overload in the patient’s body. This excess iron needs to be removed from the body by drugs which are very costly. Even after taking drugs regularly there is long term organ damage due to iron deposition in heart, liver, lung, kidney etc. This is the reason such patients have limited life span despite best care. Another risk is exposure to transfusion related infections like Hepatitis “B”, Hepatitis “C” and HIV. When there is a thalassemia-major child in the family there is intense emotional burden on the parents.


In a marriage between a carrier and
A normal individual:
  • 50 % chance: children - NORMAL
  • 50 % chance: children - CARRIER


In a marriage between 2 carriers:
  • 25 % chance: children – NORMAL
  • 50 % chance: children - CARRIER
  • 25 % chance: children – SUFFERERS




TREATMENT AND CURE

5. This disease burden can be reduced from the society by adequate prevention and awareness drives. The only cure is bone marrow transplantation which is costly and requires a HLA compatible donor.


DIAGNOSIS AND SCREENING:-
Thallasaemia minor people are apparently normal and so we need to carry out an awareness drive in the society so that all persons who have mild anaemia get themselves tested (HPLC) to rule out thalassemia. This way we can prevent the spread of this dreaded disease.


Once a thalassemia major/minor is diagnosed, all the members of his/her extended family (i.e paternal & maternal uncles and aunties of major or brother and sister of thalassemia minor) must get themselves investigated to rule out the disease/carrier status.


IMA Bareilly has installed a machine for HPLC in its premises where this test is done at subsidized rates. This test is to be done only once in a person’s life time to know one’s thalassemia status.


IMA Blood Bank , Bareilly has adopted around 135 thalassemia major patients to whom we supply blood free of cost and without replacement. We supply around 180 units per month to the thalassemics. Hence we require a team of dedicated voluntary donors.

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