My son has beta Thal and need your advice from all of you...

Dear Andy,
We are from Vietnam, my son is 6 years old, he has beta Thalassemia, and was blood transfusion for 6 times.
Now his HBg is 8.4
He always sick and fever.
As our doctors told that, they cannot cure Thal, my son must be live with it all life. He cannot go to school, because he stayed at the hospital for 1 week per month. 
Pls kindly help to advise the proper treatment for this sick and how can we raise his Hbg???

High appreciate for all reshttp://ponds from all of you!

Thank you very much,

Best regards,

Kim

Hello,

Welcome to thalpal. It seems like your son has Thalassemia (Intermedia) and needs transfusion from time to time. With lower Hemoglobin (HB), the body cannot fight and he might be getting more sick.

There are treatments like Hydroxyurea which might help in increasing your son's Fetal Hemoglobin. You can refer to the following site - http://www.thalassemia.com/non_transfusion_2.html

Chemotherapy Treatment should include an offer of chemotherapy for increasing fetal hemoglobin with hydroxyurea or Butyrate therapy. This approach has been successful in selected individuals. The doses of hydroxyurea are much lower than those used to treat sickle cell disease, and toxicity must be carefully monitored

Also, view the Diet, Nutrition and Supplements section in our site : http://www.thalassemiapatientsandfriends.com/index.php/board,26.0.html


Also, your son should avoid high Iron food as that would and drink tea with his meals to reduce Iron absorption. View another section from the same site I mentioned above.

Non-transfused thalassemia intermedia patients are encouraged to avoid high-iron and iron-supplemented foods, and encouraged to drink tea with meals, which decreases iron absorption. Serum ferritin is evaluated in adolescents. Desferrioxamine is instituted early in the development of hemosiderosis. Iron overloaded individuals receive a liver biopsy. Early cardiac evaluation with Holter monitoring and stress ECHO cardiogram is done in individuals with significant hemosiderosis. In addition, folic acid deficiency appears to be more common in these individuals

Also, it is recommended that he starts some of the vitamins listed on our site : http://www.thalassemiapatientsandfriends.com/index.php/board,26.0.html
and specifically look at this post : http://www.thalassemiapatientsandfriends.com/index.php/topic,118.0.html

Do let us know if you have any questions and we will try to help.

Hi Kim,

I agree with Narendra that it appears to be thal intermedia. Was a hemoglobin electrophoresis test also done? Values would be expressed as HbA, HbA2 and HbF and possibly other hemoglobin variants. Does his health improve after he gets transfused? If it does, he may need a more regular transfusions schedule. I also suggest that you try some supplements and pay special attention to his diet and make sure he is getting nutritious foods and avoids junk foods.

HI Narenda,
Hi Andy,

Thank you very very much for yours advise,
And i am so so sorry for my late reply, because i have stayed in the hospital with my son from last week until now!

His health improves after he gets transfused,
how can increasing my son's Fetal Hemoglobin, please advise me!
And my son can be back the normal life without blood transfusion, please? We have any approach to help him, please?

Very high appreciate for your advice,
Thank you very much,
Sincerely,

Hello Kim,

It is good to hear that his health improves after blood transfusion. It is natural to be infected when the Hemoglobin is low. With higher fetal HB, the severity of thalassemia is decreased. Fetal HB can be increased by using hydroxyurea or Butyrate therapy as mentioned in my previous post. For some thal (Intermedia), their hemoglobin levels have increased.

Your son should take the vitamins and a diet that helps him with thalassemia. He should take a low-iron diet and drink tea with meals to decrease absorption of iron. Also, a daily supplementation of 1 mg folic acid should be taken.

Kim,

There is one thing you do have in your favor and that is the center where your child is being treated. It is an excellent center and they will be able to guide you well. It may be that a regular transfusion schedule will be needed, but this will depend on both his physical state and his growth. If growth is slow, transfusions may be the only answer for now. Be assured that there is a new drug in trials that will be able to free most thal intermedias from transfusions. I expect this drug will see approval within 5 years as long as trials continue to go as good as they have been.

Hi Narendra,

I have read some your previous post, and i asked the doctors about hydroxyurea on this afternoon, they told me that they will think about that, and let me know later??
and now my son has take the vitamin (Calcium, Vitamin B, E, ZInc) and daily supplementation acid folic and also drink tea for daily.
Can he drink artichoke or not? because i heard that artichoke will be good for his liver and spleen.

Hi Andy,
As you told that there is a new drug in trial that will be able to free most thal intermedia from transfusion, but it will be come to Vietnam or not? And how can my son can take it?
Hope my son will be treated by this new drug asap.

This morning, my son has just test fetal HB, i will keep update to you!!

Thank you for everything!

Dear Andy!

I have just get the test result as the enclosed file, our doctor told that my MPV is decrease, what will happen with my son, please?

Thanks in advance for your advise!

Kim

Dear Andy!

I have just get the test result for my son as below:

RBC 4.00
HGB 8.6
MCV 68.1
MCh 21.6
HDW 4.35
PLT 407
MPV 6.9
WBC 13.56
%Mono 2.6
% NEut 8.14

and our doctor told that my son has low platelet count, what will happen with my son, please?

Now my son is taking vitamin D, vitamin B, ZInc, aicd folic, VItamin E, are they all safe together please?

Thanks in advance for your advise!

Kim

Kim,

Your son shows signs of infection. The white cell count is high. The MPV (mean platelet volume) is low. The platelet level is checked on the form, but it is within normal range, with normal being as high as 450. None of this is very alarming, so please don't worry too much. The high WBC may be related to spleen enlargement, so I need a bit more information.

Have you been told his spleen is enlarged?

What is the lowest that his Hb has been recorded at? With an Hb over 8, I don't know if this is his normal range or if it is higher due to transfusions. Do you know his Hb before his very first transfusion?

Has he had any antibody reaction to transfusions that you know about? A sign would be that his Hb dropped too fast after a transfusion.

To answer some of your other questions, yes he can have artichoke. In fact, if there are natural herbal remedies that you know work for purification of the organs or for fighting infections, you should be able to try them. I will also say yes, you will eventually see Hb inducing drugs in Vietnam once they are approved, just as you see other medicines like chelators becoming available. TIF is well aware of the Vietnamese situation. As of yet, I don't think there is a TIF association in Vietnam (and anyone who knows better please correct me) but I am sure that one will be formed. Southeast Asia presents huge thalassemia problems and they cannot be ignored. Things will improve.

HI Andy,

Thank you so much for your advise,

At right now, his spleen is enlarged,

The lowest that his HB has been recored at 7.9. His Hb beofre his first transfusion is 11.1

I know that his Hb dropped too fast after a transfusion. And i asked his doctor about hydroxyurea, he told that cannot use it for my son.

I worry too much, while my son has taken vitamin E, D, B, acid folic and avoid all iron food, but he his HB still dropped too fast.

How can his HB increase, please?

As our doctor told that not  have TIF association in Vietnam, or any association about thelassemia in Vietnam. So dont have any medicine to increase the Hb. And he told me that just have one way, i must be have more one baby, and use this baby plancenta and cell transplant for my son. With this approach, can be treat for my son??

Really high appreciate for your advise,

Sincerely,

Kim

Hi Kim,

The rapid drop in Hb level is most likely due to an antibody reaction from the transfusion. As time passes, these reactions will normally quiet down, but if persistent, drugs like prednisone might be used to try to slow this reaction so that the blood lasts longer. This has nothing to do with nutrition, but is related to the matching process for the transfusions.

This question has to be asked. Should a child whose Hb has never been below 7.9 be transfused, especially considering he does not have access to the most modern thal treatment? 20 years ago an intermedia with that Hb would not have been transfused but advances in treatment, specifically new chelators, have changed the approach to treating intermedias in developed countries. In the past, the negatives associated with transfusion, iron build up and antibody reactions, would rule against transfusion unless the Hb dropped into the 6 range. I don't know enough about your medical system to know if proper intermedia management is a real possibility, but at this point I have a hard time saying he should be transfusing, especially since an antibody reaction appears to have occurred. If possible, it would be good if he can avoid transfusions for awhile and see if his Hb level can maintain in the 8 range.

I don't know why the doctor says no to hydroxyurea. It may be that it isn't available for treating thal in Vietnam. If I could see the results of a hemoglobin electrophoresis and could see the percentage of HbF, it might give some indication if trying hydroxyurea is feasible.

Having another child will of course risk another thal child. There is also no guarantee it will be a match unless PGD and IVF are used. It is only practical if this is available to you.

Hi Andy,

The lowest that my son Hb has been 7.0, this time was terrible for us, he was so tired and fever over 42 degree.

As our doctors told that if we use hydroxyurea, it will be blood posisoning, so they cannot use it.

I would like send the hemoglobin electrophoresis as the attached file for your ref.

Please advise me which drugs can increase his Hb and try to slow this reaction.

Now my son is pale and his character has change, he is so diffucult, grouchy, Thal has concern to his character or not?
Sorry for all my questions to bother you, but i really worry for him, because he changes everyday.

Thank you very much,
KIm

Kim,

The electrophoresis reveals that he is an HbE beta thal. Give me some time to review his case with this understanding. Your doctor apparently does not understand how hydroxyurea is used in thalassemia, as it cannot be referred to as "blood poisoning". Hydroxyurea suppresses production of ineffective blood cells, while at the same time stimulating the production of HbF, which raises the Hb. With his HbF as high as it is, he would actually be considered a good candidate for hydroxyurea, but if the doctors are unwilling to try it, there isn't much you can do.

Hi Andy,

Really high appreciate for your advice immediately,

I asked our doctors to give him some drugs to raise Hb but they just told me that my son just need blood transfusion and don't need to take any drugs except acid folic. While i cannot stand to see my son health goes from bad to worse.

Please help my son, we really need your help!

Thanks in advance,

Kim