Just wondering which is better??!!

Hi

In thal intermedia, which is better for the body, to keep the same low Hb provided that the body is adapting and tolerating to this low Hb and growth is okay or give a transfusion once or twice a year and making the body expierence the ''luxury'' of having high Hb and then taking it away bit by bit through the time. I think sometimes when you make the body ''try'' something new( living with high hb) it would be difficult to adapt again with the low one.

I am not talking here about dependency of transfusion, but i am mean the realizing the symptoms of hymolysis ( as we were just talking about it) or the feeling of going from a Hb of 10 to 7 again, isn't this is an over stress on a body that was doing fine and well adapting??

Hope you can get my point

manal

Hi Manal,

I understand what you are saying.  Thal intermedias who are growing well at a lower than normal hg learn to adapt to that hemoglobin.  For me the decision would be made according to whether or not the child is able to thrive, play, concentrate and enjoy life at the hg they are at. 

My son, Zaini's daughter and Maako all describe some change in their body when their hg drops from a higher level to a lower one, and the feeling unwell that is associated with it.   I think that it also depends on how rapidly the hg drops from the high level to the lower one, if it drops slowly than the body has a chance to adapt to the slow change.  If the hg drops quickly perhaps the body would have to adapt to the low hg all over again. 

I am really looking toward some of the new hg inducers to see if they can help the intermedia patients.  During my last Oakland visit I was told that some of them are very effective, but because of our complicated situation at the time we did not have enough time to discuss them at length.  I will definitely try to get more info in the future. 

I think deciding whether to transfuse an intermedia is such an individual decision.  Because my son is a major, the decision has always been so black and white for us. 

Sharmin

In thal intermedia, which is better for the body, to keep the same low Hb provided that the body is adapting and tolerating to this low Hb and growth is okay or give a transfusion once or twice a year

From what I have seen from the experience of a couple of "intermedias" on this site, and my personal experience, a "top-up" transfusion once or twice a year is not useful from any criteria, unless say, the HB has dropped below 7gms. This is because the "life" of a RBC is 3 weeks or so - so, the top-up will be washed out of the system within one month, unless you decide to top-up again ..... you see where this logic leads us to.

Also, I would like to say that keeping the HB level at 7.0-7.5 gms is not ideal for growth, when the kid is between 3-18 years of age.

cheers

Hi Manal

I guess first thing first start Ahmad on Hydro oxy urea if from 6.5 he goes to 7.5 it is really good. Also as Dr Ali said there is no harm in transfusing but he said not to keep him at 6. So first hydro oxy urea has to do its job. Once he is 7.5 and then if his growth is normal as Dr Marwaha said about his pictures that he looks perfect and you are doing ev everything right, u hv to keep monitoring him clinically. In one year a child has to grow  6-8cms(according to average for Indians, you have to check what is the normal percantile followed  in Egypt cos genes definetley play a roll in height). If he is gaining 6-8 cms in a year you are safe, if not then I think one or two transfusions will not do any harm.

Also some amount of haemolysis takes place in every individual. Varr(strong mutation beta carrier) being a carrier also has haemolysis. for intermedia's going from 10 to 7 is slow it is not in 3 weeks that is why they sustain longer gaps of transfusions.

Have you started hydro oxy urea?>

Luv

puja

Hi Manal,

I can tall you about my experiences as thal major. There was a time in my life where i was used to low pretransfusional Hb between 5-7. Because of the high tranfusions regime during the 80's my post HB where between 14 an 18. The problems i faced during this time where fatigue, tiredness end back pain because my body tried to produced the red cells by his own.

Compared to my sibling I'm short.  At least they are more then 10 cm taler than me.

Even if your son is used to low HB level, it will not be god for his growth. I think th e mean problem will be the medullary expansion, and extramedullary hematopoiesis. I heard from several intermedias having problems to their bones.

God bless you

Many thanks for all your replies and they all emphasis that intermedia is so much case by case, but from our discussion i reall y don't understand the logic about this fact. It is true that (as Poirot said)
 that the transfused blood is washed from the system with a maxiumum of 3 to 4 weeks and also it is true that some intermedias maintain quiet a high than normal Hb when transfused biannually and the Hb drops slowly  (as Puja said).

I know that intermedia produces little good blood but still the transfused blood has a short life span and the little good blood will not be able to maintain the high Hb lest it would have done this before tansfusion??  So what is the explaination of this two contradicting facts

Sharmin,
Thanks dear for your concern and hope that Dr. Vinchinsky will offer us other options

Puja,
I will probably start the hydroxyurea after finishing the concentrated supplements  i have told you about and also after examining the effect of carao as i prefer to test everything alone. Also i will have another assesment at the endocrinologist by the end of this month and will see what she will say.

Poirot and Gabri, happy to see you posting from quite a long time, missed you a lot

manal

Manal

I think you need to start hydro oxy urea. Supplements will work on with hydro as well. These are his growing years he needs to be more than 6.5 as Dr Ali said. I think all supplements including Carao and wheatgrass donot obstruct hydro's performance. Please he has to be at 8 atleast.

Puja, i totally agree with you and i will finalize everything with the doctor in my next meeting.

Andy, can you have any explaination for those contradicting facts??

manal

Dear Manal,

First of all you are doing an excellent job with your son - he is on a great program and he looks great.  Take your time to decide what is best for him.  Whether you decide that transfusing him during his growing years, giving him hydroxy etc for the time being is a good idea - just make sure you factor in everything  For example, transfusions may mean overall well being, more energy and growth, development - but it also means a big lifestyle change for you and your son - including full days at the hospital and also chelation.  My son does not complain about tx and chelation because he doesn't know any other way of living. 

After gaining complete knowledge and consulting with others, you may think to try one transfusion and see how it goes.  You will see if the difference it makes in your child is worthwhile to continue.  Again, because the decision was so black and white for me in which I had to transfuse my son - I don't know myself what I would chose to do if I were in your shoes.  I know that Dr. Vichinsky spoke at great length about increasing hg levels in intermedia patients - he spoke of this in the context of children who do not tolerate blood transfusions saying that he has found ways to "have them make their own blood" - but he said that he would discuss this with us further when my son's iron levels were better controlled and his antibodies were taken care of. 

These are some of the reasons I would like to see you in NY next year.  Don't worry Manal, your son is doing great and whatever decisions you make will only make his health better,

Take care,

Sharmin

Thank you Sharmin for your comforting message and i am eagerly waiting for what Dr. Vinchinsky will say.

manal

Manal,

Have you seen this post?  It may aid you in making decisions for the long term.

http://www.thalassemiapatientsandfriends.com/index.php?topic=56.msg1185;highlight=bilirubin+levels+in+thal#msg1185

It states some of the long term effects of thal intermedia and low hg - extramedullary hemopoesis and bone frailty is a little frightening. 

Sharmin

Hi Manal,

Intermedias who transfuse once or twice a year usually do so because they have had a temporary drop in hemoglobin level, often due to being ill. The purpose of the transfusion is to get back up to a level where it isn't such a strain for the bone marrow to do its normal job, which is to keep the intermedia Hb level. It isn't that the blood lasts long but it does enough to get things back to normal for the patient. With the transfusion, the bone marrow only has to do what is normal. Without, the bone marrow has to work to get the Hb back to the pre-illness level. This does make it easier for the patient in recovering from the low HB period.

Manal, I was very encouraged after seeing pics of your son, especially when Dr Marwaha said he looked very good. I know there is much discussion about whether or not your son should transfuse but I really do think a fair trial of hydroxyurea along with other supplements should be tried to see what level Hb can be achieved. If he didn't look so good, I would say it's time to transfuse, but as long as he is healthy and his Hb doesn't drop more, I think the alternative should be given a fair chance. Is alpha thal silent carrier totally ruled out?

Thanks Andy, things are now very clear.

Alpha thal silent carrier was never discussed before with his doctors. To my understanding if available in my case it would eliminate the severity of animea as their are less alpha in the system, right?

His CBC and other tests are due November 1st and i will test again the G6PD ( we had a talk about in Singapore ). I will ask if we have any tests showing the alpha mutations or not.

manay thanks Andy
manal

Yes Manal

Alpha silent helps. What are your husbands and your mutaitons?

If Ahmad has it - it is beneficial.

Chk the mutations of all three of you.

Thanks Puja


Ahmad's mutations are:
IVS1-6
HB Knossos codon 27


As for my husband and me  we didn't do any tests but we assume that he is the silent carrier since all his blood indices are in the high normal

manal