HBE-Beta Thal

Hello everybody,

I am from Kolkata, India and new to this forum.

Its only last month my 2.6 yrs son diagnosed with HBE Beta Thalassemia. He is fully active and going to pre school regularly. No symptoms are visible. I am providing below his latest blood report also few queries.

hb         7.8 g/dl
rbc        4.18 mill/cumm
mcv       62.2fl
mchh     18.7pg
rdw        30.3 %
ferritin    ng/ml
iron
tibc
rbc morphology

hbf          41.8%
hba2+e   48.4%

Although, Dotor said that its in minimal stage and provided folic acid tablet. Still I have below queries.

1. Is he Thalassemia Major or Intermedia?
2. Can it be predicted if he required transfusion in future? If yes, generally from which age it may required?
3. Is he required BMT? If yes, I have his cord blood and stem cell reserved. Will that be useful?
4. Can he lead normal life?
5. I need some food name which are available in India can increase HB level but without having iron.
6. Can Kapalbhati/pranayama/yoga can increase HB level? I need to know if HB level is in between 9-12 lifelong, then also transfusion may required?
7. Gene therapy can be used?

Thanks in advance to all for reply.
Since He is our only kid, we are now feeling helpless and without any clue what can happen in future.

Regards,
Ratri.

Hi Ratri,

1. Right now, he is too young to predict the future, because HbE beta thal can result in different outcomes, but currently, he would be called intermedia.
2. It can't be predicted yet if he will need transfusion.
3. BMT is not appropriate for intermedia classification.
4. He can lead a fairly normal life, even if transfusion is eventually required.
5. I will leave this for Indian members to advise (iron will be absorbed at a higher rate due to the low Hb, so high iron foods should be avoided).
6. Yoga will be very beneficial for his health and will help him optimize oxygen use in his body. Highly recommended.
7. Gene therapy is in trials. It is premature to draw any conclusions.

I would like you to contact Dr Vijay Ramanan in Pune. Your son is an ideal candidate for Dr Ramanan's therapy.
+91 93253 15471
Email
mvijayramanan@facebook.com

Thank you very much Andy.

Ratri,
welcome to Thalpal. Do not feel helpless or worry about future. With proper care and thal management your child will thrive and can do whatever he wishes to do. A good diet is very important and so is a multivitamin including Vit D to keep him healthy. I will suggest to continue to read the various forum threads specially Living with thalassemia and Thal intermedia to be knowledgeable about how to help your child. Also, note that even if he remains transfusion free, transfusions may be needed when he falls sick and Hgb falls too low.
Good luck and always ask questions when you have doubt. This forum is god sent for us as I feel Andy and other members have way more knowledge than many of the doctors treating Thal. (do not mean to offence a doctor but this is our personal experience).
 

Thank you so much Bostonian_04. Yes.. I became more confident when I found out this site. Also Reply from Andy and other members gave me more clear picture about Thals. I gone through already the threads you mentioned. Those are the person who made me like yes, there is an answer to Thals. But you know, it is just a month before my son got diagnosed, so sometimes, we again became helpless thinking all negative part of this disorder. Me and my husband blamed each other for not testing Thals before our marriage. And situation became worse sometimes. Also whenever my son falls sick, we got tensed, thinking now Hb will fall and we have to start transfusion. I know we should think positive but truly speaking we are trying hard to spend normal life as much as possible.

Dear Andy,
I also received Sept'2015 Blood report for him. Below is that. HB was 7.8 in month of Aug but it reduced to 7.6 this month. And doctor is saying I can go for one transfusion. Could you please tell me if he really requires transfusion now. Since he is active and no visible problem at all. But he falls sick ( fever and cold, running nose, cough) before 2 weeks. Now he is fine. Is it the reason his Hb falls by 0.2?


HB:              7.6 g/dl
PCV:            25.6%
Platelet:       399000/cmm
Erythrocyte: 4.28 million/cmm
MCV:           59.8 fl
MCH:           17.8pg
MCHC:         29.7%
RDW-CV:     29.2%
Total Leucocyte: 11730 /cmm

Neutrophils:      42%
Lymphocytes:    41%
Monocytes:       6%
Eosinophils:      11%
Basophils          0%

Reticulocytes :   3.5%
Serum Ferritin   99.08 ng/ml

1. My son is now 2.7 years old. Is it  like HB will reduce day by day?
2. If he gets transfusion is that mean he will become transfusion dependent and required blood each 3-4 weeks?
3. He had folvite tablet 1 mg during whole month of August. Also we provided good diet. But Hb level did not increase. We tried him to do Yoga. But it is very hard to make him sit for it. So is there any other things which can increase the level?
4. Like every other we also afraid of transfusion, so only trying hard to increase level without it, although we know some on the other day it may required. Could you please tell me under which level we can give transfusion. Since my son is fully active, is it really required now at this moment?

Please suggest.
Thanks you so much. You people are really hope of us to live a normal life.

Ratri

Dear Ratri,

Though your son is active but always try to keep the HB level 9 to 9.5.Low HB delay the growth development.

Ratri,

There is no significant difference between and Hb of 7.8 and 7.6. You could test a person twice in one day and get that much variation. The level of hydration alone will cause small changes like that.

Before transfusing, please contact Dr Vijay. He is doing remarkable things with patients and your child is a perfect candidate for his therapy. I would suggest starting wheatgrass powder or tablets immediately.

Thank you very much Andy. I'll start with wheatgrass tablets. Could you kindly let me know the regular dose.

I am staying in Kolkata and it's near about 2000 Km distance from Pune. Also we have to go and stay there. We'll surely go there, but before that we need some time and arrangement. Is there any kind of online appointment / discussion with Dr. Vijay can be made initially?

Thank you again.

Regards,

Ratri

Hello Andy,

I tried to contact Dr. Vijay Ramanan. But the clinic replied Doctor is not available for any sort of online appointment. Meanwhile My son's pediatrician is suggesting for transfusion asap ( His HB is 7.6).

Could you kindly let me know the name of wheatgrass tablet. How long it may take to increase HB level.

Waiting for your kind reply

Thanks
Ratri

Hey Ritra ,

I just met Dr Vijay Ramanan last week on 18th at his Pune's private clinic .
My Son (4 mths) has been diagnosed to be a thal major. On some points which Dr Vijay Raman mentioned when we spoke on our son's case.
 - As per his assessment we can let the HB go till 8 or 9 before you go for transfusion. So 7.6 is low as per his standards as well.
 - He started my son on wheatgrass powder for now. He suggested us to buy Globsure wheat grass powder . I think any good quality wheat grass powder should be fine . It is with orange flavor with added Vit E and it’s bitter to taste. Also important point here is the start of wheat grass powder is under what he called was Novel Therapy (heard that term for first time) & in future he might put him on Hydroxyurea   or other  medicines which if I understood correctly increases his HBF and hence would maintain his HB. He told us to meet him next after 4 months.
 -  Not surprised he does not give any online consultations as he seemed quite busy when we visited him and he just gave us about 12-15 mins for hearing our case and suggesting the plan of treatment.
 
 Hope this information helps you.

Ratri,

Dr Ramanan will only meet patients in person, due to his very busy schedule.

No one can predict if a patient's Hb will rise using wheatgrass. Dr Ramanan uses an app that will tell you if a response is more likely, but it is not exact. The best thing to do is take it and see what happens. Wheatgrass is a food and carries no risk, so it is perfectly safe to use.

I have apt with Dr Vijay Ramanan coming Tuesday ( 29/9/15)

One thing want to share with u all..

http://www.ashg.org/genetics/ashg07s/f11147.htm

THALIDOMIDE THERAPY IN A PATIENT WITH THALASSEMIA MAJOR. L. Aguilar Lopez1, J.L. Delgado-Lamas1, B. Rubio1, F.J. Perea2,3, B. Ibarra2,3 1) Servicio de Hematologia, Hospital de Especialidades UMAE, CMNO, IMSS, Guadalajara, Mexico; 2) Division de Genetica, Centro de Investigacion Biomedica de Occidente, CMNO, IMSS Guadalajara Mexico; 3) Doctorado en Genética Humana, Universidad de Guadalajara, Guadalajara, Mexico.

   The β thalassemia is characterized by a deficiency or absence of β globin chains. The homozygote state (Thalassemia Major), has high blood transfusion requirements since early age. We describe a 21 years old women with β Thalassemia Major diagnosed at 5 months of age. In 1997, the biochemical studies showed high HbF levels (62.3%) and HbA2 of 3.61%, the genotype was identified as -28 A-C/Cd 39 T-C. She had chronic blood transfusions, every 2 or 3 months, with an iron overload. She was splenectomized at the age of five years. She had received chelation therapy (Desferoxamina) with different time intervals. Her hemoglobin levels without transfusion were as low as 2.9 g/dL. The patient was received at the hematology service of the Hospital de Especialidades in Dcember 2001 with 4.0 g/dL, when she initiated the thalidomide therapy (100 mg per day), the first hemoglobin increase was observed after three moths to 7 g/dL, since then she has the thalidomide therapy uninterruptedly and never was transfused again with hemoglobin levels between 7.6 to 10.6 g/dL and almost 100% of HbF. She is at present in good health conditions with hemoglobin values of 10.2 g/dL. To our knowledge this is the first report of a Thalassemia Major patient treated with thalidomide with such a great results at the hemoglobin levels and general good health conditions. The molecular and physiological effects of the thalidomide must be deeply investigated, since it is know the angiogenic effect in cancer, the benefic effect in thalassemic patients could be mediated by its gene modulator effect however the true mechanism require to be investigated.

From what I understand, Dr Ramanan's therapy includes hydroxyurea, thalidomide and wheatgrass.

Thank you very much Mukta, Andy and msarika.

We went for a trip to jungle, so could not able to reply. My son enjoyed a lot and also participated in a early morning elephant ride. . The tour went fine and he was fully active, only 1 day he suffered with cough.

@ Mukta, I'll start trying the Wheat grass powder mentioned by you.

I also thought of visit a hematologist from Kolkata. Do anyone have any information about a good hematologist in Kolkata. I already met with a renowned hematologist but sadly he told all all negative views and told us not to come to him unless and until our pediatrics said so. And my pediatrician suggesting for transfusion. We also decided for transfusion, but before that want to visit a hematologist for at least some positive points.

Since My son's blood group is B- which is a rare group , Can anyone have any information where shall I contact for blood in Kolkata.

Thanks for all of your support and suggestions.

Ratri.

Dear All,

Kindly let me know if you have any information about good hematologist in Kolkata,India and blood donation center where I can get "B-" group.

Regards,

Ratri