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Thalassaemia is a genetic blood disorder that impairs the oxygen carrying capacity of haemoglobin in the blood. A Thalassaemia minor is a healthy carrier of the Thalassaemia gene and may not exhibit any outward symptoms.

Children who inherit the Thalassaemia gene from both parents may sufer from Thalassaemia major and have acute anaemia, enlarged livers and spleens, deformed bones and other growth disorders. The only means of survival for such children is through regular blood tranfusions for the rest of their lives, the cost of which, along with other medical expenses can run into lakhs. There are almost 10,000 Thalassaemia majors born in India every year!!

3.5% of our population carries the gene for Thalassaemia. It is by far the most common affiction in India. There is a one in thirty chance that you are a carrier and not even know it. Ignorance about Thalassaemia is helping it to multiply faster than it is possible to contain its spread.
 
Yes we can!!! If you are a healthy carrier of
 
If your spouse is not a carrier; there is absolutely no chance that your children will have Thalassaemia major, though they may have the Thalassaemia trait like you.
 
If your spouse is a carrier; there is a 25% chance that the child may have Thalassaemia major. This  is true for every pregnancy that you may have.
 
 

When both parents are carriers, a test called Chorion Villus Sampling or CVS for short can be done on the foetus to ensure it is healthy. This can be conducted as early as the tenth week to guarantee that there is no danger to the child or mother. If the test is positive, the foetus can be aborted safely.

Remember there is a 75% chance of a healthy child in each pregnancy. However, you will need to take the prenatal test during each pregnancy. This is not an option!!

 
To find out if you are a carrier, all that is required is a simple blood test that will take less than 10 minutes of your time. At WE CARE TRUST. we offer this service at a subsidised rate on the fully computerised Variant machine.
Thalassaemia is an inherited genetic blood disorder there are two forms of Thalassaemia.
 
Thalassaemia Minor/Trait/Carrier
 
Thalassaemia Major
 
In Thalassaemia minor one out of two genes, responsible for production of Haemoglobin in red blood cells, is defective. Other gene is normal. Thalassaemia minors are therefore perfectly healthy;through they are slightly anaemic, they require no treatment.
 
But if thalassaemia minor marries to a person who is also thalassaemia minor, the couple may give birth to a thalassaemia Major child. Or if he marries to a normal person, couple may give birth to thalassaemia minor child.
 
In thalassaemia major both the genes responsible for production of Haemoglobin are defective, consequently red blood cells are not produced and thus necessitating for blood transfusions, two of three times a month as long as child lives. The only treatment is blood transfusion every 15-20 days throughout life. Repeated blood transfusion cause number of complications in the child. Overall the treatment is very traumatic and expensive. Though Thalassaemia major is incurable but it can be 100% prevented.
 
The Thalassaemia Major Child is born only if both the parent is Thalassaemia Minor.
 
It is therefore imperative to avoid marriage between two thalassaemia minors. However if they are already married or the marriage between two minor is unavoidable due to certain reasons, the only ray of hope is Prenatal Diagnosis (P.D)
 

This test was first developed in 1980,s for prenatal diagnosis. The best timings for CVS are between 9-12 weeks of pregnancy. An early test is better than a late test, because if foetus is affected the late termination will be more upsetting and painful.

P.D. is done on a foetus (unborn baby) during early pregnancy, to see if has inherited a particular baby will be either affected or UN affected, than only one can decide to terminate pregnancy.

In each pregnancy, couples at risk for thalassaemia have 3 out of 4 chances of a Normal child. Hence Prenatal diagnosis is advised in EVERY pregnancy.

If the results of prenatal diagnosis come as an unaffected foetus, patient can continue with the pregnancy. If the results are affected foetus, they are advised for the termination of the pregnancy,

 
 
 
In Thalassaemia Major, the child is not able to produce its own blood, and has to be given blood transfusions, two three times per month. While the blood transfusion, Prolongs life but are not devoid of it’s On problems like AIDS and Iron overload mainly.
 
The Iron content of transfused blood get accumulated in the vital organs of body, like heart, liver, kidney and endocrine organ, resulting in their failure and ultimately death
 
For the last 4-5 decades, only the drugs used initially for transfusional chronic iron over load is Desferrioxamine (DFO) and has been considered as gold standard in the management of iron overload. Through it is very efficient in chelating the iron, the compliance is poor as it has to be given by subcutaneous infusion for 8-10 hrs a day for 5-6 days in week by injection. Other problem encountered is cost involved which is too prohibitive for the common man to use and hence it is being used by not more than 10-20% of our patients
 
The Deferriprone or Kelfer (L1) was a breakthrough in iron chelation therapy as it was first orally effective chelating agent. India was the 1st country to introduce this drug and it has been widely used in our country as it is much cheaper, within the reach of many patients and can be given orally and hence compliance was very good. Several studies have confirmed the efficacy of this drug in reducing both Sr. ferritin & Tissue iron overload; however this drug has side effects of arthropathy, pain in the joint in 20-30% of cases and in occasional cases children developed life threatening complication like neutropenia and hence there was always an effort to find out a drug which is safer.
 
Deferasirox (Desirox or Asunra) is a new chelating agent which has been introduction recently, can be given once daily as oral chelator for treatment of iron overload associated with repeated blood transfusion. It is also called as Exjade (ICL 670).
 
Primary data has indicated that Deferasirox for one year is associated with improvement with myocardial iron overload (deposition of Iron in Heart) & labile plasma iron Dosage depends on the patient’s response, Sr. Ferritin level and goal of the therapy is to maintain and to reduce body iron level.
   
The drug is available in the form of dispersible tablets and should be dispersed in water, orange juice, and apple juice) and are available in the form of 250-500 mg. This is an orally absorbed Iron Chelator and consumed as drink once daily preferably half an hour before meal, preferably at the same time each day.
 
The patients With Thalassaemia major should begin chelation therapy once they had 10-20 transfusions or (100ml. /Kg) has been transfused or when ferritin levels have raise than 1000ug/I)
 
In conclusion various studies done so far Exjade fulfills the criteria of near ideal chelator has least side effects and can be given orally. Thus a dream drug has become reality
 
Extracts from Dr. Lokeshwar’s Articles on Iron Chelation Therapy
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
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