Thalassemia Patients and Friends
Discussion Forums => Thalassemia Intermedia => Topic started by: sirovietnam on September 20, 2011, 03:46:48 AM
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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. :wah
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
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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.
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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.
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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,
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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.
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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.
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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!
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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
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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
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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.
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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
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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.
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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
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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.
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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
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Kim,
Now that I have more complete information, I can better advise. Your son is an HbE beta thalassemic. This can manifest as either transfusing or non-transfusing thal intermedias depending on the severity of the beta gene mutation. Your son's HbA is non-existent. His hemoglobin consists of HbE, caused by a common Asian variation of the beta gene, HbF which is produced by the neighboring gamma gene, and HbA2 produced in small quantities by another neighboring gene. No normal beta globin is produced by the beta gene. This is beta zero and when combined with HbE this is likely to require transfusions.
The way you describe your son's health and activity level, he does appear to need regular transfusions, perhaps once a month, maybe a little longer. The problem with this is that it does add iron, which means after awhile he will need to begin a chelation program. The other problem is that he risks more antibody reactions with ongoing transfusions. This is also a common transfusion related issue and it causes the spleen to enlarge as it removes red blood cells. In the past, the routine was to surgically remove the spleen to slow down this process, but this has proven to not be a good policy in the long term, as it can lead to other problems as patients age. It should not be removed unless there is a clear danger of rupture. Talk to the doctor about your child's apparent antibody reaction to the transfusion and see if there is anything that can be done to more closely match his blood for transfusions. The Hb should not drop within a few days and as long as it does, antibody reactions are the most likely cause. If you can get him past this and on a transfusion regimen when needed, his overall health should improve dramatically. I have heard this often in cases of thal intermedias. I am sorry that the doctors won't cooperate and try hydroxyurea, but perhaps in a few years, once this new drug is marketed, the doctors will be much less reluctant because this drug is being developed specifically for raising the Hb, especially in thals with Hb levels in the range that your son has. This drug is being tested in Asia also, so I do believe there will be real attempts to get this drug into the hands of Asian patients. I expect that we may also see some copying from certain drug companies in Asia, as there is with chelators. So, keep hope. I think the best thing right now is to test his Hb regularly and transfuse when needed. I do think with his lowest recorded Hb level being 7 and a high HbF, that he is an excellent candidate for Hb inducing drugs.
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Hi Andy!
Thank you very much for your advice,
It's mean that now my son just keep regulary transfusion please? I really worry about him, while his Hb drop too fast, and he cannot take any drugs.
For hydroxyurea, it is so expensive? Where can we come to treat? have any association can help us? My son really need your help!!
Now his health is not good, he has cough everyday, although he takes medicine but still cough, i don't know how can i do for him.
HIgh appreciate for your advice,
Thanks again,
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I think his health will be better with regular transfusions, but the blood needs to last longer, so talk to your doctor to see if there is any better matching available to help prevent antibody reactions from the transfused blood. The Hb will have to be tested between transfusions to find out how often he will need to take blood. Once regular transfusions start, the goal is to stay above an Hb of 9 so that the quality of life is improved and bone marrow activity is reduced.
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Hi Andy,
I asked our doctors many times about this, and asked him that has any drugs for my son to help prevent antibody reaction from the transfused blood, increase his Hb, and slow down his hb dropping.
OUr doctors just told that not have drugs, just follow up and take acid folic everyday, nothing more.
I really worry about that, and these time, my son is not good, he always vomit, cough, fever.
Really high appreciate for your advice,
Thank you very much,
Kim
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Kim,
Get him transfused when he gets listless and sickly. After a few months, the length of time he can go between transfusions will become set. As I said, the antibody reaction seems to be the biggest obstacle so far. But, you cannot know if this is continuing unless he gets transfused. Hopefully, his blood will last longer. Until he is no longer sickly, I cannot advise he try anything like hydroxyurea, as his system probably won't easily tolerate the introduction of any drug. Once he is transfusuing and maintaining his Hb level for a few weeks, his health should improve.
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Hi Andy!
Really high appreciate for your advice,
I will try to ask the doctor for my son try hydroxyurea.
Hydroyurea is expensive or not, please?
Somebody told me that should be bring my son travel abroad to treat for him, because in Viet nam, will be take along along times to treat for Thal.
Please kindly help to advise me where can we come to treat?
Thank you very very much for everything from you.
Kim
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Hi Kim,
Hydroxyurea is a fairly inexpensive drug. A study on its use in Algeria suggested that it should be considered a potential therapy for thalassemia in poorer nations exactly because of its low cost. You are right about treatment being better elsewhere. In your region, Singapore might be the best place to go to get a proper evaluation and recommendations for the best treatment program for your son, but I would assume it would not be cheap to do so. I don't know the state of medical care in Vietnam but I must assume that it is not well advanced at this time. Can you tell me is Vietnam progressing in terms of general medical care?
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Dear Andy,
I don't know how can i talk to your about Vietnam progressing in terms of general medical care.
In Vietnam, we have to pay a lot of money to cure Thal, the insurance company just pay a little, that's why we cannot cure this.
I am really worry about my son, i read on internet, they told that Thal patients cannot be cure, and slowly to grow up.
I have sister in USA, she will be act as guarantee for my son come to USA to cure Thal, is it possible or not, please?
I asked many doctors about hydroyurea, but nothing from them. I really want bring my son come to Singapore to treat for him. But we dont have enough money to do it, i wish have any association can help us.
And now, my son has high fever after transfusion. How can i do now, Andy?
High appreciate for your advice,
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Hi Kim,
The only cure currently available for thalassemia is bone marrow transplant. For most patients, a cure is not possible due to lack of a matching donor and the cost. So, treatment is the usual course of action. Transfusions and chelation once necessary. Keeping the Hb above 9 is the best way to help his growth.
The fever is probably because white blood cells are present in the transfused blood. Is he getting whole blood or packed red cells? Is the blood being filtered? This is important to prevent reactions to the blood.
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Hi Kim, how is your son doing?
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Kim,
How old is your son?
And, I have to agree with Andy on one aspect - you should STOP transfusions for a while to see how low his HB drops and whether it stabilises or not. I would even say that you may want to see the HB drop to 6.0 before restarting a transfusion schedule.
With his HbF levels, he should do fine for a long time without transfusions. Transfusing such a child make actually create transfusion dependency, where none may exist at the moment.
RE: infections - water quality is a big issue across Asia - do you boil or filter the water for your son? Or is it raw water from the pump or the tap? Similarly, things like salads and uncooked vegetables can pose a threat to your son. All such "raw" food should be thoroughly rinsed and washed with boiled water.
I hope this helps.
Poirot
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Thank you every body for your all comment.
My son is 7 years old.
The doctos just give acid folic, supply vitamin B for him, and nothing else.
I was so worry, his HbF dropped down quickly, just 3 weeks, the doctors have just requested blood transfusion for him, no medicine to stop his HbF dropping down. His healthy was not good, always fever, flu, cough, ....
Then we used Vietnamese traditional medicine (base on medicine herbs). Now he is better, i don't know how is he in the future.
His HbF has 8.41 at this time. His skin still pale, and have a cough. From last Nov, 2011, he could extend the time for blood transfusion, it takes 2 months.
I always cooked everything before gave to him to eat.
if you have any ideas to help my son Please advise me. We still need all of your helps!
Thanks again,
Best regards,