Sukalpa Dhar, Kolkata: Thalassemia is not a disease but a genetic disorder which happens only to the children. Unfortunately, it is transmitted to the offspring from the child’s parents itself, unknowingly. There is no dominant father or dominant mother in case of an occurrence of a thalassemic baby. It is equally shared between the parents. Both the mother and father equally share the gene which carries the malady of thalassemia. If both the parents carry equal amount of thalassemic gene i.e. only if 50% of thalassemic gene comes from the mother and 50% of the thalassemic gene comes from the father then only a baby might become a thalassemic one. If either of the parents is a thalassemic gene carrier then the offspring will not be a thalassemic one.
It is therefore necessary that before marriage both the man and woman needs to test whether he or she is a thalassemic carrier or not. If none of the bride or groom is a thalassemic gene carrier then they can easily marry each other. One must understand that a thalassemic gene carrier is otherwise completely healthy and normal. To know whether a person is a thalassemic carrier or not he or she has to undergo a test know as the HPLC test. It should be done only once in a life time. A thalassemic carrier can easily marry a non carrier. There is no problem. But if both the man and woman who are about to marry each other are thalassemic carriers then there are three choices (a) they might not marry each other or (b) they can marry but decide not to have any offsprings or (c) after marriage when the wife is pregnant within 12 weeks that is within 3 months they have to test whether the baby which is there in the womb is thalassemic or normal. If the baby is not thalassemic then the pregnancy will continue and if it is seen that the baby is thalassemic then abortion will have to take place. Then within 3 months of pregnancy abortion has to take place because, after 3 months of pregnancy abortion is illegal. It should be noted that there is 25% chance that a carrier can have thalassemic babies in each pregnancy.
This genetic disorder has been existent in our society since the last 75 years. Thalassemia is a genetic disorder which was originated in the Mediterranean area and got spread all over the world now. There are two types of thalassemia (i) Alpha Thalassemia and (ii) Beta Thalassemia. An alpha thalassemic baby dies in the mother’s womb or at the moment of birth itself. A beta thalassemic baby remains healthy upto 6 months after birth. Then subsequently various symptoms show up (1) inanimateness (2) pale (3) diarrhea (4) loose motion (5) sickness (6) non consumption of food. Then consult a doctor and try to do a blood test after the doctor’s consultation which is called primary screening. After undergoing the primary screening if the doubt prevails then without waiting too long one should undergo the HPLC test immediately. If the HPLC test shows positive then the treatment will start up ideally under a haematologist.
When the world is fighting against the constant rise in covid19 cases in the mean time thalassemia cases is also having their steady and continuous leap day by day. If people stay side by side without covering their face with an N95 mask then Covid 19 will definitely spread. The rate of thalassemia is also increasing day by day in all over the world especially in the SAARC countries because we are least bothered about our future generations. For the benefit of the future generation it is good to take such steps so that not a single thalassemic baby is born. To create awareness international thalassemia day is observed on 8th May every year. We can free the world from this deadly disorder very easily.
If a thalassemic baby takes birth in a particular family then that baby will cause untold sufferings to that family. The hemoglobin level gets reduced in a thalassemic patient because new hemoglobin is not being created naturally. If the baby doesn’t have hemoglobin then it cannot carry oxygen and if the baby cannot carry oxygen then it dies due to multi organ failure. The hemoglobin level in a human body should be in between 9 to 10 g/dl. To keep the hemoglobin level of the baby between 9 to 10 g/dl we have to infuse blood to the required level.
Thalassemia has no treatment till date. A thalassemic baby will not survive more than 6 years without infusing blood. A thalassemic child is kept alive by transfusing blood. Blood transfusion is the only process to keep the child alive.
There is no other treatment of thalassemia. Through the help of transfusion a thalassemic child can be kept alive for a maximum period of 17 to 20 years with the hope that someday may be some kind of treatment might come up for thalassemia. But unfortunately so far there is no treatment for thalassemia. That thalassemic child dies due to heart attack because of abundance of iron in his or her body. Every time blood is transfused to the child’s body in each bottle of blood an extra of 200 mg of iron get into the body. This creates another problem of excess of iron in the body. That iron is not helping in creating haemoglobin in the body.
This excess iron needs to be driven out. This process of driving out the excess iron from the body is called chelation therapy. If the excess iron is not taken out from the body that excess iron gets deposited in liver, pancreas, heart and the baby dies due to heart failure or liver failure or jaundice or diabetes or enlargement of spleen. That means one after another complications will arise. Upto 10 years the child can be kept healthy through the help of blood transfusion.
This is a total genetic disorder. It carries a huge costing. If one can spent Rs 1 lakh per month for a single thalassemic baby then the baby can be kept alive for many years and in the subsequent years he or she can also work and do service. But it is not a one time matter. The child should be kept under continuous surveillance under doctors. The child will have to undergo continuous blood transfusion and chelation till the end of his or her life. There is another treatment for this which is called the bone marrow transplant. In this process our original bone marrow is destroyed and in lieu of that new and fresh bone marrow is inserted in the bones. It costs between Rs 25 to 30 Lakh. Here too the success rate is only 80%. So the parents should take care that both of them shouldn’t be a thalassemic carrier. It is guaranteed that if both the parents are not a thalassemic carrier then their offspring will never become a thalassemic one. This is the easiest process to stop thalassemia.
Choosing the right partner for marriage is an age old culture where we always want that the husband and wife’s pair should be the best possible one. That will subsequently lead to the arrival of a healthy child. In ancient times people used to depend and believe on horoscope because science was yet to be invented. Horoscope was believed to be the most appropriate technique.
But nowadays even if the horoscope shows a pair to be a perfect match we still have to test their blood under a microscope to be sure that both the husband and wife is not a thalassemic carrier. One should not depend on horoscope only. Presently, we judge everything scientifically so the matching of blood is also very essential and scientific. So we have to put the blood of a prospective husband and wife under a microscope and test whether both of them are carriers or not. Both of the husband and wife should not be thalassemic carrier. So our slogan is horoscope to microscope. This will be the most scientific approach to deal with the malady of thalassemia. We have 23 pairs of chromosomes. From which 23 chromosomes come from father and the other 23 come from the mother. It is guaranteed that if either of the parents is normal and the other is the carrier then too thalassemia will not occur. If both the father and mother are carriers then only, there is a possibility of birth of a thalassemic baby. Every carrier will also not create thalassemic babies. Even than every year in India 20 lakh unit of blood is used to keep alive a thalassemic patient.
Multiple awareness programmes in educational institutes such as schools, colleges and universities need to be held. Both print and digital media should come forward to help in spreading of awareness regarding thalassemia. We can also make the HPLC test a compulsory one. We can include a chapter on thalassemia in the school curriculum and make it compulsory to study, because it is a major problem since 1925 and still counting. In Kolkata, eminent orthopaedic surgeon and Rotarian, Dr. Ramendu Homchaudhuri is immensely contributing towards creating awareness regarding thalassemia and eradication of this deadly genetic disorder through sustained campaign, talk show, media interview, visits to educational institutions and interact with participants towards achieving the goal.
The article has been written after taking a detailed interview with Dr. Ramendu Homchaudhuri & with his approval. He is the chairman of the Thalassemia Prevention Committee Rotary International District 3291, Kolkata. Dr. Ramendu Homchaudhuri is working passionately for years, with the goal to stop birth of new thalassemia child by sustained awareness program and MANDATORY CARRIER DETECTION TEST (HPLC).
Photo | Representative Image
