In this video, Dr. Sunil Bhat provides an in-depth discussion on the various treatment options for managing thalassemia major.

part-2-how-to-effectively-manage-thalassemia-major-dr-sunil-bhat-narayana-health-city

Part 2 | How to Effectively Manage Thalassemia Major? | Dr Sunil Bhat, Narayana Health City

[Music] Hello friends my name is Dr Sunil but and I'm the director and clinical lead of um pediatric Hematology Oncology and Bone M transplant at the majima medical center narana H stre Bangalore now I I told you um sometime ago that uh we'll be starting a series of videos uh for the general public as well as for the caregivers and the parents of the patients with talisia measure in first video I discussed with you what is talisia how does it um you know manifest and what are the causes of talisia uh measure now that was covered in the first video now in this uh today's discussion the today's video what we want to cover is um is how to effectively manage tmia how do we look after the patients who are diagnosed with talisia measure so as we know talisia measure is a problem where body or the bone marrow doesn't make enough blood or enough red cells and hence they're dependent on blood trans to be given from outside you need blood from outside because you're not making enough blood so there are two principles of management or in fact three principles of management of thalis measure one is to how to give them effective blood transfusion second is that what are the complications which come up with blood trans that's called iron overload I'll discuss about that in a little while but second is to how to look after iron overload because of the transfusions and the third is effective followup that means how do we follow up how do we how do we monitor these patients especially when they become older you know more than 5 8 10 15 years of age how do we monitor them in today's video I'll be discussing only the two first two parameters that means how to give effective blood transfusion how to do iron cation or iron overload the third aspect we'll do in a separate video that is how to monitor how to follow up now coming to the first point that is blood transfusion the first question which comes in our mind is that is blood transfusion is it required or can we just skip the blood transfusion answer to that is no we cannot skip blood trans thalis measures and because they don't making they're not making their own blood we need to give the blood from outside so there's no shortcut in that second question which comes in the mind always is that what is the level of blood or the hemoglobin we should maintain now we have to remember that in thalis measure because it's a slow process children even with hemoglobin five 6 7 will be otherwise active and normal they look like normal from outside but inside the low levels of hemoglobin will create its problems what problems it will create it it will let their organs to grow the liver becomes big the spleen becomes big they will have facial change changes they will they you know they will have bony changes and some of them are irreversible so hence friends the most important message I want to convey here is that please maintain the hemoglobin of these tmia major kids before giving blood 9.5 to 10.5 G per deciliter please maintain levels 9.5 to 10.5 before giving blood so this level has to be maintained before giving blood not after giving blood after giving blood this will become 13 14 we're not interested in that we're just interested in what level to maintain before giving blood and that is 9.5 to 10.5 so that's first important question which is asked second question which you ask always that what type of blood to be given and how much to be given so blood as I said earlier in the first video has got many components plasma white cells platelets and red cells this child has got only problem in the red cells so we do not want to give him anything else except for red cells so hence we should give this tacmic kid only pack cell transfusion we do not need to give them whole blood we need to give a back cell transfusion has only only white red cells in it and not does not have plasma does not have white cells and does not have platelets the third important thing is that how much blood to give we need to give them 15 to 20 ml per kg of paxel that means at one point of time you can give them 15 to 20 ml per kg of paxes that means if the if the kid is 10 kgs we can give 150 to 200 mL of paxel if a kid is 20 KES we can give 300 to 400 mL of paxel so the amount of blood which is given doesn't depend on the units or doesn't depend on anything else it just depends on the weight of the patient and based on that you can give blood 15 to 20 ml per kg the third question you ask about the blood transfusion is that or how long we should do this blood transfusion whatever blood is required to be given to the patient to the thalmic kid 15 to 20 ml per kg has to be finished in 4 hours we should not get the blood packet from the blood bank and hang it on the B side for more than 4 hours because it gets spoiled when it's kept on the room temperature so all the blood which is being to be required to be given for the particular kid for the Thum measure has to finish in 4 hours for example in a 10kg child you are giving 150 ml of PX this has to finish in 4 hours in a 20 kg child you are giving 300 M pxel this has to finish in 4 hours so whatever blood is required has to finish in 4 hours that that's the third thing now the fourth thing is that is there any other things in the blood we need to take care of yes we need to take care of two important things one is that as I said it has be packed cell and we need to remove the white cells in the blood which are there to be given to the patient we don't require to give white cells to the kid because the white cells in the blood which is collected from someone else will go in the kid's body and do something called as sensitization and it also lead to more reactions in the in the in the in the kids body thalis kids body so we need to remove these white cells before giving blood and that removal is called as lucco depletion and this lucco depletion is done by filters which can be done either at the blood bank level that me the blood bank does a liod depletion or you can do the LIC depletion at the bedside when you connect filter with the blood and that filtered blood goes to the patient and the last thing about blood is that this blood can transmit with them infections what infections HIV Hepatitis B hep C you know other infections can come from blood which is donated from the donor and can go to the patient so we need to test the blood always for these infections we test this Blood by various techniques Eliza is something which is very very commonly prevalent and is done but the newer method of testing the blood is n testing where you can check for these infections very early on and reduce the chances of infections coming from the donor to the to the patient so friends in the blood transfusion what I learned is that we need to maintain levels of 9.5 to 10.5 before giving blood we need to give pels we need to give luod depleted blood or lucoded pels we need to you know finish this blood in 4 hours whatever is to be supposed to give to the to the kid and last is that we need to give 15 to 20 ml per kg of pack cells and and the amount of blood which is to be given will depend on the weight of the uh weight of the kid who requires a blood transfusion so that was the first component and that is about the blood transfusion now the second component of our discussion as I said today is iron cation that means how to reduce the iron levels in the body now I told you that blood transfusion for the Thalia major kids is a Lifeline we need to give them the blood transf outside because they're not making their own blood but the blood you know regular blood transfusion every few weeks when the blood is given it brings with them some problems and what are those problems one problem is as I said infections and we address that how to minimize that the second problem which is a severe problem in talisia and that is iron overload iron overload means the the blood which you're giving to them has got iron in it and it does it it keeps on accumulating in the patient body and the levels of iron in the patient's body becomes very very high when the blood iron levels in the body becomes high this iron gets deposit in the various organs what organs heart liver hormone glands like thyroid like pancreas like adrenal gland like pitutary in the brain all these organs develop a lot of iron overload and that leads to problems it leads to Heart fail it leads to you know liver problems it lead to hormone problems and hence we need to remove this extra iron which is coming from the blood from the patient's body now how do we do that it has to be done by medications called cation so the question is that when should we start cation when we should start giving medicines for reducing the iron from the blood we check a parameter called fertin fertin is a test blood test which measures um the level of iron overload in our body and we check this fertin when the patients has received almost 9 to 10 blood transfusions we check the fertin level and see what the fertin level is if the fertin level is more than 1,000 then we will go for um start of cation in the baby so that's first when to start when the fertin has crossed 1,000 that usually happens after 9 to 10 transfusions number two is that what is the dose of iron cation there are three medicines which are used for iron cation Cur you know defo and deseral Des is injectable the other two are oral defox is the first line or the are the most commonly used iron cation your doctor can explain you which one to use but what is important is to give the correct dozing and as the children grow the dose of iron cation will also along with based on the fertin and based on the patient's weight we'll have to increase the dose of the medication as we go along so there is no defined dose for ulation it depend on the patient's weight so that is a second important thing the third important thing is that can we stop the iron culation no we cannot stop the iron culation iron cation is lifelong because your blood transfus lifelong as we keep on giving blood transfusions iron overload will keep on happening and we need to keep on remove the iron from the patients body and hence we cannot stop iron ination yes as I said earlier on we can increase or decrease the dose based on patient weight and P based on fertin levels or iron overload levels if the if the iron is levels are coming down if the fertin is coming down we can reduce or increase the dose now what is the magic number or what do we need to do with the with the iron overload we need to maintain our fertin as close to 1,000 as possible we should not let it go high and that is why it's always try to kept at at the level of 1,000 now there are modern ways where iron overload can be measured more appropriately and more effectively than fertin what are this modality is called as T2 star MRI so the kids who are more than 8 years of age should have a T2 star MRI you know at 1 to 2 years interval to understand what actually their iron overload in the body is and that may also help us to understand whether they need any modification of medications to reduce their iron overload that's so that is second the third very important point is about fertin when we check the fertin for iron overload what are the precautions to be taken one very important precaution is that fertin in in medical language we call it acute phase reactant that means any small infection fever cough cold vomiting loose motions can increase the fertin levels so if a tacmic kid requires a fertin estimation fertin blood test we should not do it if they're having any of these infections because it will be felicious High it will be just high but it's not actually high it's high because of infection so we should avoid doing fertin estimations when the patient is unwell or the baby is unwell who wait for at least for 2 weeks for the infection to settle down that's number one number two is that fertin is there's lot of interlab variation one lab will do some value second lab some other value will come so it's very important to keep monitoring whatever reasonable good lab you have you keep on folling with the same lab or a period of time and the third important thing is there is no importance of one fertin value what is important is the trend so you you when you keep on checking the fertin every 3 to 4 months from the same lab you'll see the trend of fertin and that Trend would be very important and that would help your doctor to take decisions on how much and what to do with the iron culation medicine whether increase whe they to decrease and they will take necessary precautions on that so friends I'll close this discussion today in this current video what we have discussed today in this current video was that how to give effective and safe blood transfusion to thalmic kids and also so how to give effective cation or IR reducing medicines to these kids and how what are the precautions to be taken especially when we are uh you know looking after the iron overload in these babies so with that I'll close this discussion today and I'll be coming with another interesting um aspect of talisma measure in the in in the in the next video and um and we'll discuss a different aspect of thalac measure in that video so thank you very much for attention [Music]

Dr.

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