In this video, Dr. Sunil Bhat explains the basics of thalassemia major, a genetic blood disorder. He discusses its causes, how it ...

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Part 1 | What is Thalassemia Major and What Are the Causes? | Dr Sunil Bhat, Narayana Health City

[Music] Hello friends my name is Dr Sunil B I am the director and clinical lead of pediatric Hematology Oncology and bone transplant at the majar Sha Medical Center narana H Bangalore our country is the talisia capital of the world majority of talism miics born in the world are from India and we add approxim ately 20,000 children with halisi measure every year and also almost equal number of children with halisi intermedia every year uh in our country being a periodic hematologist and oncologist a significant amount of our practice is also revolves our thalac major whether it is looking after these patients for transfusions for complications and also for boneal transplants to cure them hence I thought it's a very good idea to at least you know address some of the concerns of talisma measure in a series of videos which would be a good educational tool uh for the the society at large and but but also to the families and the caregivers of the of the children who are having Talis measure so under the under the series of videos which I've decided I'm going to take different comp components of thalis care and address them in short videos which are and I would want to try to address common questions which come to the parents and the caregivers mind so the video now today which I'm trying to do is on the basic question of what is talisia and how does it come and know how does it happen in the children so as we know our blood uh which is the lifeline of our body has got two components one is called a cell cellular component one is called the plasma or the or the fluid component now the cellular components we have got the red cells or what we measure as hemoglobin we have got white cells and we have got platelets these are the three components of our blood platelets help in clotting white cells help in fighting infections and the red cells are the ones which give energy to our body and you know sustain life so to say now what happens in thalis major what happens is that the bone marrow which is the factory of all these cells does not produce enough red cells and the ones which are produced by the bone marrow also have a short life as a consequence of that these children do not have enough red cells or enough hemoglobin in their body and hence they require this blood or the red cells to be given from outside and that's what's called as blood transfusion now when when does this problem start you know manifesting in the children it usually starts manifesting usually at the age of 4 to 6 months where you see that these children these babies they progressively become pale they become lethargic and you know the activity comes down some of the patients may start having vomiting loose motions at that point of time and when you go and see a doctor they tell you that the baby is very very pale baby has got big liver and big spleen and when you do a blood test it shows that the hemoglobin levels are very very low white cells and platelets are normal in number because white cells and platelets do not have any problems in thalis measure and if we do not do anything for these children those who are now diagnosed with THM measure as I said usually at the age of 4 to 6 months of age if we do not do anything these the levels of the hemoglobin usually comes down and most of the children may not live more than 2 years of age hence to save them or to to sustain the life for these children they require blood transfusion from outside they require regular blood transfusion on a frequent intervals we discuss about this blood transfusion in a separate video in a separate session but what I focus today is that what the Thalia is and how it does you know how does it manifest and why does it come first of all in the children now we have also seen some children being diagnosed as I said the usual age of presentation is about 4 to 6 months and that's what usually this time this this disease is diagnosed but some children can present little later also it can some someone some children can present 1 years one and a half years 2 years and sometimes you later on also and there's a group of children who have this talasemia but not as severe that means they do not require blood transfer from outside the blood levels are low but they're able to maintain some amount of blood which is okay and enough probably for them to sustain life and that's called as talisia intermediate that is a less severe form of talisia measure and that's called as thalis intermediate so this is how this disease basically presents now the question is why does the disease come first of all what is the why what is the cause or what the reason for having thalis measure now thalis measure is a genetic Disease by genetic disease I mean that it comes it's hereditary it comes from our parents it comes it runs in the families so the parents of talisia measure kid basically are talisia miners or talisia carriers now I I'll be explaining you what that means the blood formation or the red cell formation in our body which is done by the bone marrow um basically is controlled by some genes which is in our in our genetic system where we call them betag globin genes and there are two genes which which are relevant or important in making this red cells or the or the hemoglobin in a in a in in a person and those are called as betaglobin genes now the parents of this talisma major baby are carriers that means they have got one normal Gene and one abnormal beta globin gene or they have got one normal Gene and the other Gene has got talisia mutation and because the out of the two genes one is functional one is working normally because of that they do not have any problems the hemoglobin levels are little low but they even do not know that they have something like this in their body hemoglobin are little low but they do not record blood transfusions they're like any other normal person in in the society in the community and when two of these carriers marry that means mother and father both have one normal Gene and one abnormal Gene both of them are unaware of this problem because the disease doesn't they don't have any manifestations of this disease and when they marry there's a chance that these two bad genes one from the father and one from the mother can go into the baby and that's what called thalis majure now this Talis majure kid will have two bad genes two bad betaglobin genes one from the mother one from the father because there are two bad genes in the baby they do not produce or they not make blood which they are supposed to make I you know I use a very very simple example to explain explain this we have two kidneys in our body which makes urine right you know we have heard so many stories if one kidney doesn't work the other kidne is working it's enough for us to you know make enough urine and we'll you know people are okay with even with one kidney but if the both the kidneys are there which are not working or they have failed then that's a problem the urine will not be formed and they will have problem because of kidney failure and that's exactly what happens in talisma measure both the parents are having one abnormal Gene but they they are unaware of it they don't know that they abnormal Gene and when they marry and they have children they have you know the baby can have talisma measure because both the abnormal genes can come together and it's like having two bad kidneys or two failed kidneys which do not do their job and so so the mechanism or the or the cause of this disease Thal measure is genetic it comes from parents and the both the parents of these children are basically having thalis minor or what's also called as thalis trait so thalis Miner thalmic carrier thalmic trait are all same they they three terminologies for the same condition which is that they they're the carriers of the Gene and and affected babies are called as thalis measures and some of them can be talisi intermediates also so so that was a summary of today's this video uh friends and we discussed how does Thalia manifest and what are the symptoms of talisia at what age it comes and we also discussed what are the causes why does a talisia measure come in in the baby and what are the reasons for that and I discuss that a genetic cause so we'll we'll close this discussion here today on this topic and I will be coming again with to you with a new topic on talisia in a different video thank you very much [Music]

Dr.

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