Thalassemia Patients and Friends
Discussion Forums => Thalassemia Major => Topic started by: Mukta on August 24, 2015, 11:11:08 AM
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Hello Andy Sir and rest of the folks on this group ,
My son Rudra (3.5 mths old) has been diagnosed to have thalassemia major . In last 21 days he has already gone through 2 transfusions.
At 2 mths and 15 days (4.2 kgs at that time) his hemoglobin had dropped to 5.5 g/dl after which the doc did a transfusion of 100 ml blood. His HB shot up to 12.4 g/dl after this transfusion. At 21st day after his 1st transfusion we went for HB test again at which his HB was at 8.8 g/dl . Since he had HB dropped below 10 the doc advised us for the 2nd transfusion of 50 ml (As per his weight of 4.9 Kgs) . His HB after transfusion is 12.1 g/dl.
In these days we got his Hemoglobin electrophoresis and DNA study test done which more of less confirmed his case to be of thal major .
His DNA study report had the following observations :
Child : Mutation type : IVS 1-5 (G-->C) B-Thalassemia trait Homozygous.
Both Parents Mutation type : IVS 1-5 (G-->C) B-Thalassemia trait Heterozygous.
Pre - Transfusion HB electrophoresis report
HBF : 90.9 , Hb A0 : 6.9 , Hb A2 : 0.7
We are getting him treated in Jupiter Hospital , Thane (Pretty close to Mumbai/Bombay) , India under a pediatric and hematologist.
I have been following and reading a lot in this group from last 20 days and the group post have helped me immensely to understand this disorder much better and in a large extent given me strength to understand how we can manage this disorder and what are my options of treating him. Thanks to everyone in this group for putting up such a great forum.
My questions to the group to start with :
1) Want to know some good doc in Bombay , India for second opinion and more guidance on how we can manage this disorder and if anything we can do more for such a small child.
2) The 2 transfusion he had to undergo the doc advised us to go for irradiated blood which will help in future for BMT. Is there anything else I should take care w.r.t the blood transfusion
3) Should I worry so soon about the iron accumulation .
4) It is normal for such a small baby to drop his HB in like 20 days. From what I had read for small babies it takes usually 50 - 60 days cycle.
This is my first post to the group . Sorry for long post but wanted to provide all info.
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1) Want to know some good doc in Bombay , India for second opinion and more guidance on how we can manage this disorder and if anything we can do more for such a small child.
Sorry, can't help you with respect to this question. Perhaps our members from India can help.
2) The 2 transfusion he had to undergo the doc advised us to go for irradiated blood which will help in future for BMT. Is there anything else I should take care w.r.t the blood transfusion
I don't see how irradiated blood can help BMT in future (two different concepts). Irradiated blood is sometimes used for neonatal and paediatric patients to prevent Transfusion Associated Graft-Versus Host Disease (TA-GVHD). You should try to maintain 10g/dl. We never used irradiation for our daughter.
3) Should I worry so soon about the iron accumulation .
It is too early to worry about iron accumulation. Iron chelation usually starts after 12 to 15 transfusions. Children as young as 2 can start exjade.
4) It is normal for such a small baby to drop his HB in like 20 days. From what I had read for small babies it takes usually 50 - 60 days cycle.
It is possible that the HB drop in 20 days with the use of irradiated blood. Irradiation reduces the storage-life of red cells and whole blood.
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Irradiation of blood is given to prevent transfusion related graft vs host disease. There are few hospitals in India which provides irradiated blood for transfusion. You must follow Dr's advice and I don't see any reason for not using irradiated blood if your hospital is providing one. Since you are planning for transplant, you should also opt for blood filters for Leuko depletion of blood and also to prevent transfusion associated complication like CMV virus. CMV is one of the big complication that arises after bone marrow transplant and patients exposed to CMV virus before transplant are more susceptible to develop CMV post transplant.
You can find details of blood filters from the link below.
http://www.pall.in/main/medical/product.page?id=48145
You can order Pall filter from the below link at at subsidized rate. They charge a nominal membership fee. For bulk order the price per filter will be 50% lesser than the market price.
http://www.thalassemicsindia.org/what-is-thalassaemia.php
Since your child's HB had dropped to a very low level 5.5, it will take a few transfusions (2-3) before your child's HB will start stabilising. Once your start maintaining a HB of 9+, you will find the transfusion cycle to be more between 5 - 6 weeks for the first 2-3 years.
Regards,
Himanshu
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DearHimanshu,
After 2/3 yrs the duration of week will be reduce or not? I mean more transfusion will need?
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Dear Lokkhi maa, more transfusions or more blood bags on every transfusion will be needed as your son will grow up and gain more weight. Which on the other hand is important so a child can grow up healthy and most of all with healthy bones...
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Thanks Himanshu.
Blood transfusion from same donor is helpful? Our doctor says it's better to use blood of a known person. However, One of my friend who is a regular donor to a thalassemic girl says that doc suggests that they should keep a gap of 4-5 months before giving the blood from same donor. Please advise.
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The quantity or frequency of the transfusion depends on some factors like body weight, activity level of the child and overall general health of the child. The HB tends to fall faster for very active child or in case of general illness like cough, cold, fever etc. Usually the amount required for transfusion is decided on body weight - the rule is 12 - 15 ml/ kg of body weight. Initially when the baby is small and activity levels are low, the frequency and quantity of transfusion will be lesser. As the baby grows and gains body weight, the quantity of blood required during each transfusion will go up. For every KG of body weight gain, the quantity of blood transfused will go up by 15 ml.
For the first 2-3 years, your frequency of visit to the hospital for transfusion may remain low at one visit in 4-6 weeks as it is the quantity of blood required that will go up and not how frequently the child is transfused. The frequency of transfusion will stabilize between once in 4 - 5 weeks as the child grows further and the activity level goes up, say for the next 3-6 years.
Once the child attains a body weight of 25 - 30 kgs, the frequency of visit to hospital possibly would be between once in 3- 4 weeks. By that time the child possibly would be 10 - 12 years old.
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Using the same group of donors regularly leads to fewer antibody reactions from the blood and is a good practice when it can be managed.
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Dear Mukta..
Dr Vijay Ramanan from Pune is one of the very good hematologist from India. Ph No- 02024484213. I m taking his consultation since 5 yrs.. & it is very useful
I just want to ask u all that Doctor says , if all the activity of patients are normal then we can wait till the HB level 6 ( no BT ), is it ok...
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Dear Mukta,
Its right that "should keep a gap of 4-5 months before giving the blood from same donor".I have selected 4 person for donate to my baby who are donate after every 4 months gap..
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Hello Himanshu Kumar,
Thanks a nice reply.If blood volume arise then the overload iron will be manageable by chealaton therapy but if the transfusion gap reduce day by day then how my baby or all of thal baby will tolerate the horrible transfusion pain...
How and when all of our baby will be free from the pain ???
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Himanshu : The link of the blood filter does not seem to work. Can you re-post it if possible. I did visit a thalassemic day care center recently at Thane and they did mention I should insist the hospital to use a filter. They also suggested I should be insisting them for NAT tested blood to be very safe with blood transfusions.I will be insisting for these before we take him for his next transfusion and also will talk to the hematologist. Will also try to get more information on irradiated blood from the hematologist I will be meeting soon.
Masarika : Thanks for posting about Dr Vijay Raman. I am planning to meet this doc next week.
I am also planning to meet a couple of other hematologist from Bombay whom I have heard have been treating Thalassemia major children.
1) Dr. Sunil Parekh : Hematologist : Practices at Bombay Hospital
2) Dr.M R Lokeshwar : MD Pediatrics : Practices at Lilavati hospital
If anyone have any experience with them please share.
Thanks to Canadian_family and Andy for your comments.
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Dear Mukta..
Dr Vijay Ramanan from Pune is one of the very good hematologist from India. Ph No- 02024484213. I m taking his consultation since 5 yrs.. & it is very useful
I just want to ask u all that Doctor says , if all the activity of patients are normal then we can wait till the HB level 6 ( no BT ), is it ok...
DO NOT let the HB fall below 9 in any case. Low HB may result in enlarged liver and spleen. The spleen/Liver recycles red blood cells / break downs old and damaged cells. In patients with thalassemia the red blood cells have an abnormal shape, making it harder for the spleen to recycle them; the red blood cells accumulate in the spleen, making it grow. An enlarged spleen can become overactive - it starts destroying healthy blood cells the patient receives during transfusions.
Maintaining high HB is also important for normal growth of the child. Poor HB also leads to weak bones and deformation of bones and HB. Don't worry about iron build up and go for regular transfusions. There are good chelators available these days. KEEP HB ABOVE 9.
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Pasting the link again. trust this will work. Though the earlier link is also working - I just checked again.
You can order blood filter from thalassemicsindia.org. The second link gives details of the product. Thalassemicsindia provides Pall filters.
http://www.thalassemicsindia.org/
http://www.pall.in/main/medical/product.page?id=48145
If any one is actively looking for BMT solutions, may visit the following centers
1. CMC vellore
2. Apollo Chennai
3. Narayana Hrudalaya / Dr. Pietro Sodhani / Eugenio La Mesa.
The above centers have huge experience in BMT.
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Thanks Himanshu for further assistance.
I will get myself registered with thalassemicsindia.org . Looks to be very useful for thelessemics.
I am aware of CMC Vellore and Narayan Hrudulaya (under guidance from Dr Sondani's team) and plan to visit them in coming months.Did not knew about Apollo Chennai.Will try finding more details. Any one with any experience on Kokilaben Dhirubhai Ambani Hospital,Mumbai . They have also been listed by cure thalassemia group and its in my city itself.
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Friends ,
Just thought to update this thread with important updates from the hematologist we have been meeting. Hope these help someone.
The below updates are apart from the common things I have read on this form or internet about thalassemia
1) Dr Suniel Pariekh : (Spoke to him for around an 1 hour)
- Suggested us to start his Ferritin/SGPT/SGOT level test after 2 transfusions itself. We found it was actually good as his ferritin did come pretty high at about 1000 after 2 transfusions. Suggested us to do this test every three months. These test are to be conducted before blood transfusion process.
- On the amount of blood to be transfused he said he even at times goes as high as 20ml/kg to get some more time between transfusions. All his patients have responded well to the transfusions.
- Referred us to 200+ page handbook for thalassemia patients on http://www.thalassaemia.org.cy/
- Did not talk much about BMT process. Suggested we still have time and should only concentrate on our son as of today as he is too small.
2) Dr Sameer Melinkeri (Dinanath Mangeshkar hospital , Pune) : (Spoke to him for around 45 min)
Got his reference from someone know who got his thalassemia major son's BMT done from this doc in this hospital. Concentrated our talks more on BMT process.
- Shared his recommendation on BMT : If there is a sibling match 1st priority , BMT(Stem cell) in Chang Gung Memorial Hosp, Taiwan 2nd priority , BMT with Unmatched donor 3rd priority , BMT with Haploidentical parents 4th priority.
- Will look into starting hydroxy for my son in future.
- On our recommendation gave a go ahead to get my son's , wife's and my high resolution HLA done. The hospital has tie up with Histogentics. Results are still awaited. Cost : 16500 Rs per person.
- Cost of BMT in Dinanath Mangeshkar hospital is around 8 lacs for sibling match.
3) Dr Vijay Raman : (Spoke to him for around 15 min)
- Gave us some info on Novel therapy . Recommended we start my 4 mth old son on wheat grass powder. We have already done that in low amounts and would increase gradually to his recommended dosage.
- Shared a detailed list of test we should perform on monthly , quarterly and yearly basis. Will share the list with the group in a separate thread shortly.
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Andy ,
I have read in many of your post you suggest to use L-methylfolate . My son Rudra now 8 months old takes like 2.5 mg folic acid tablet(FOLVITE brand , pfizer company) daily. I have a confusion if L-methylfolate (in some form) is what you are suggesting instead of the folic acid tablet.
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If he does well with it, folic acid is fine to take. Many people cannot metabolize that form and need to take L-methylfolate.
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Andy Sir.
How I can understand that I can metabolize or can not metalabolize folic acid tablets ?
I also take 5 mg folic acid tablet daily .
Hallo Mukta
Can you tell me the availability of L - methyl folate in India in which brand of manufacturer ?
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So many brands are available of L-Methyfolate in India
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Abahaya I had the same question for Andy.
How do you know if the tablet is working ? Andy should I just attempt to give him L-Methyl folate tablet and stop the folic acid tablet as L-Methyl folate is more broken down version of folic acid if I understand correctly ?
What would be the equivalent dosage of 2.5mg of folic acid tablet when compared to tablet of L-Methyl folate .
Abahaya the brands which I have found are Vitalin (0.8 mg) , THF (1000 mcg) , Tetrafol (7.5 mg , Sun pharma)
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I don't know is L-Methyfolate available in Bangladesh or not?
Dear Andy,
Whats the benefit between Folic acid and L-Methyfolate? Both are help to create new RBC?
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Either one is needed to make RBC's. L-methylfolate has been shown to work better and some patients react to folic acid, so they can only take the folate from. You would know if a child reacted to folic acid., because folic acid might cause abdominal cramps, diarrhea, rash, sleep disorders, irritability, confusion, nausea, stomach upset, behavior changes, skin reactions, seizures, gas, excitability, and other side effects.
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What will be the dose of L - methyl folate equivalent to 5 mg folic acid tablet ?
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L-Methyfolate not available in Bangladesh.. :sadyup
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The dose of folate is the same as for folic acid. For a child, 2 mg is plenty.
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So many brands are available of L-Methyfolate in India
Dear Dharmesh Ji
Please tell me a good brand of 5 mg L - methyl folate tablets .
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FOR KIDS
TETRAFOL PLUS TABLET
Mfg: SUN
(Other Products from SUN) Comp: L-methylfolate 2.8 MG+Mecobalamin 2 MG+Pyridoxal 5-phosphate 25 MG Form: TABLETPack Size: 10 (Units)
MRP (per pack) :
Rs. 140.00
FOR ADULT
L-FOLINK 800mcg
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Dear HANUMANT,
Can I get help from you about the medicine? I will pay you by western Union.If possible pls inform.
Bcoz its not available in Bangladesh.
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Hi Lokhi maa,
sending parcel from india to bangladesh is not possible for. I can send it within India.
Regarding use of L-methylfolate, first u need to establish that plain folic acid is not working for ur child. It doesnt make sense to jump on l-methylfolate blindly.
Morover , to transfusing thals , doctors no more suggest even folic acid or l-methylfolate
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:mom :mom
Thanks HANUMANT..
Regarding use of L-methylfolate, first u need to establish that plain folic acid is not working for ur child.
How we got the result about folic acid?
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Hallo Lokkhi ma
From Andy Sir's previous reply I understand that if anyone tolerate folic acid , facing no side effects to taking it, can metabolize it then folic acid is fine instead of taking L-Methyfolate.
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Thanks Andy , Dharmesh for the answers. Abyaya yes you are correct if folic acid tablet is not showing any side effects than it should be fine and no need to jump on to L-Methylfolate. I will continue my son on Folic acid since he does not show any abnormal sings.
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now it is possible to send Sarvayush wheatgrass tablets parcel to Bangladesh.
If u want let me know.