Update after one month of Hydrea

Hi

I would like to give you an update on Ahmad after one month of hydrea. He is takinf 3ml everday right before he sleeps to avoid the dizziness stomach ache.


*HB before Hydrea was 6.1 ( Ahmad had several infections at that time and his average is 6.6 without anything)
Hb after hydrea is 6.7

*Reticulocyte count before was 6.4% and after was 5.3%

*Bilirubin before was 1.7mg/dl and after was 2.1

*Total Leucocytic count before was 10,300/cmm and after was 6,800

* Platelets before was 450,000 and after was 389,000

* MCV before was 52.6 and after was 59.9

*All other functions of liver and kidneys are normal

My Comment:

1- I know that hydrea decreases the WBCs count but it has fallen too much and i don't know if this will be the maxiumum decrease or it will still continue to go down

2- I don't know why bilirubin increased though Hb increased

3- As i mentioned the averge hb for Ahmad without Hyrdrea id 6.6, so i don't know is this result (6.7) means that his body didn't respond to the medicine or it is just starting to increase and therefore i need more time to judge

4- It is the first time to have Ahmad's MCV almost 60, the max. he ever reached was 54

Does any one have any comment on these results or theirindications??? please feel free to comment.
Also i will be visiting my hema. tomorrow night

Take care
Manal

Manal,

The Hb produced by the body normally is mostly HbA. These drugs induce HbF which adds to the total Hb. I don't think there would be any effect on what your body normally produces because the HbF is adding to the total Hb but in no way is this related to how much HbA is being produced, with or without the drug.

In beta thal there are not enough beta chains to match with the alpha chains to make hemoglobin, resulting in an excess of alpha chains. When you induce HbF, it works by creating more gamma chains (normally found in the fetus and newborns) and these gamma chains combine with unused alpha chains to create hemoglobin. This would not effect the production of normal hemoglobin as it is only using some of the unused alpha chains. Whatever beta chains the body produces will already be matching with the alpha chains, which are being produced in normal amounts even though there isn't enough beta chains to match with them, leaving plenty of unused alpha chains to combine with whatever gamma chains are produced using the drug.

Thanks Andy for your reply, i totally understand it. One thing i can't get is the percentage

 I don't think there would be any effect on what your body normally produces because the HbF is adding to the total Hb but in no way is this related to how much HbA is being produced, with or without the drug.

In doing HPLC after using inducers, we find that the percentage of F HB increased and Hb A decreased , isn't this an indication that hb F is taking over???

One last thing came to my mind, in beta thal, why don't they research for something that decrease the production of alpha chains so that it balances with the decrease in the beta chains production??

Manal

Let me give an example to explain what is meant by percentage of Hb. I will use some arbitrary numbers to demonstrate.

If you take 10 grams of hemoglobin and you say 50% is HbA and 50% HbF, then there are 5 grams of each type. If you can stimulate more production of HbF so that now you have 7 grams of HbF and a total of 12 grams of Hb, the percentages change even though the amount of HbA has remained the same. Now, 7 grams of HbF is 58% of the total Hb and only 42% is HbA, even though the amount of HbA is still 5 grams. What has changed is the amount of HbF and the total Hb. Even though the percentage Of HbA has dropped, nothing has happened to the total amount of HbA in the blood.

In a person with no hemoglobin disorder the amount of beta chains and alpha chains produced are exactly equal. In beta thal, the amount of usable beta chains is greatly reduced, resulting in a surplus of alpha chains. Reducing the amount of alpha chains would not result in any additional hemoglobin being produced. The goal is to increase the amount of chains that can match with the alpha chains to make good Hb. If gene therapy works, a new beta gene will be able to make beta chains that will match with these alpha chains. The alpha chains by themselves are not much of a problem, although there is some evidence that they may clutter up the blood, increasing the possibility of clotting. Because there isn't any danger posed to most thals by these alpha chains, there is no reason to reduce them.

Manal,

I think one month use is too early to make any judgments. Monitor the wbc's closely. Lowered wbc's can mean that he will get infections more easily, so as with many other drugs, the wbc's have to be regularly monitored. Keep in mind that even a small increase in Hb can mean the difference between transfusing and not.

Hi Manal,
I visited my haemotologist today, and she recommended me either splenectomy or hydroxyurea treatment.
For Hydoxyurea treatment, I was told that it will take around 3 months for results to be noticed and it's expected that WBC count during the treatment will drop. But after sometime, it should stablize.
Ahmad's number for WBCs is not bad at this time. I think doctors worry if it drops below 3-3.5.

Hope it helps.

--Hallu

Hi Manal,

I wish little Ahmad all the best.  I hope his hg improves in leaps and bounds - with whatever treatment you choose.  Please keep us posted, he is so lucky to have such a caring and considerate mom.   
Sharmin

Thanks Andy. Finally i got it. I wanted to make sure that if for any reason i stopped the hydrea, nothing will get worse. I feel more comfortable now

Hallu, thanks a lot for your feedback and advice.
 what about you, which treatment did you decide??

Sharmin, thanks a lot for your wishes and your compliment, you are always very supportive

Manal

Hi Manal,
 
this are real good news. Hope your son will get better and better.
 
I remember when i was a little child my Hb was 6 g/dl. Many reticulocytes where found and i had high leucocytic count (It ranged between 30000 and 40000). I was explaned that reticulocytes has an nucleoulus later they loose it and becomes an erytrocyt. Leucotcytes has also a nucleoulus and now  it becomes tricky. The mashines can't distinguish between a reticuloucyt and a leucocyt. Because of low count of reticuloucyte in nomal blood the mashine count every cell with an nucleoulus as it would be a leucocyt. To finde out the real total leucocytic count they have to exame a drop of blood under microcope and count the cells.
 
An other reason for leucocytic decrease  could be explaned by lower erytropoiesis. A sign of lower erytropoiesis is that both leucocytes and plates are falling. This means that his body is not working to hard to produce the blood cells in bone marrow.
 
The mean corpuscular volume (MCV) increasen a little bit. This means that the red pigment in a single erytrocyte increase :-) . Normal range is 82-92 fl
 
Bilirubin is the waste product of heam (part of heamoglobin). Don't worry, most thals have increased bilirubin. The only risk can be gallstones in future. This is a commun problem especially in thal intermedia.
 
Does the infection perstist after he started Hydroxy?
 
Did you stop Hydroxy now or will you continue for an other while. I thinkthis drug will work better on long term.

God bless you

Hallu, thanks a lot for your feedback and advice.
 what about you, which treatment did you decide??

Manal,
You are always welcomed!
As far as my case goes, I'm really struggling hard to arrive at some decision. With splenectomy, my main worry is: decreased immunity towards bacterial infections. Though, there are other side effects also such as increased platelets, but i believe that can be taken of by taking baby aspirin everyday. So the main downside is, decreased immunity.
With hydroxyurea, my concern is : what effect will the drug have if taken life long? I have to research more on it and I'll look forward to any advice from you since you must have dealt with the same question when opting for this treatment for Ahmad. Also, my doctor suggested that whenever I decide on having another kid, I'll have to discontinue the medicine for atleast 3 months. I'm not sure whether this affects fertility or birth related problems? I also checked the thread started by you on the same subject...Did you ever happen to get any more info on same?
Also, as far as dosage goes, the doctor said to start with 10mg/kg weight of body and then increase it slowly to 20mg/kg and then 25mg/kg. Are you administering it in the same way with Ahmad?

So have to do some homework before arriving at some decision.

Thanks,
Hallu

Thanks Gabri a lot for your reply and for the information .

Actually we started the hydrea after he became well and when i visited the hema, she told me that we will continue the medicine for 3month to judge. If he reacted positivly and his Hb increased, she will give it to him one day after the other instead of daily

take care
Manal

Hi Hallu

I know how confusing this situation, but as you said there is homework that should be done. Generally speaking, i think that if i were you i should try every possible solution before choosing a surgory though splenectomy is common to most thals.

My advice to you (if your condition can endure to wait few months) is to try some natural F Hb inducers like resveratrol or aloe vera.

Speaking of hydrea, to tell you the truth, i wanted from my deep heart to avoid giving it to Ahmad, that is why you will find many posts from me asking about natural inducers in an attempt to try to avoid it.  I can not forget that at the end , it is a chemotherapy, though i know that the dose i am giving is not that highly toxic, but as a mother this is like a rock on my heart ( this is an arabic proverb, hope that the translation can make you feel how i really feel).
Anyway, as i told you i tried to avoid it as much as i can until one day the hema, told me that she thinks that it would be much better that Ahmad starts it. At that moment i was about to tell her no and i should wait some more time ( actually i don't know what i am waiting for ). At that moment i had another feeling telling me stop interfering, may be i will be harming my son so i surrendered.
What i know about hydrea is that some patients can react to it after one, two or three months and others don't react at all. Liver and kidneys functions and CBC should be done monthly to assure that nothing is harmed because if the kidney or liver enzymes increased it will be stopped at once.
It really has a lot and wide range of side effects that you could have them all or have none of them or part of them. As my doctor told me, this medicine is a personal expierence for each patient.

It is writen in the pamphlet of medicine that if you want to have a child you should stop as you said for 3 month. It is true that it can cause infertility and also some patients are not affected. Here in Egypt there is some one who has been taking hydrea for 18 years and has two children. He is now 28 years and the very strange thing is that he didn't stop the medicine before having his children as conception happened by accident and both his children are normal.

On the net i found a research talking about children who administerd hydrea for 6 years and that was the longest time i have read a research about,but as i told you they are children so it is not yet clear who is infertile and who isn't.So as i told you everything is possible and every person has his own expierence. So this means that i will always be facing the unknown.

Concerning the dose, we first started with 25mg/kg and Ahmad felt dizzy, had stomaches, so we decreased it to 20/kg and i give it to him before he sleeps to avoid any discomfort and he is doing fine this way. Actually, when he got tired from the first dose, i wanted to know how he really feels, so i had a dose for my self ( I know this sounds crazy and i know that its wrong but i needed to know what my son is going through). So i guess you feel like a big stone tied to your stomach and you feel very down and dizzy. Therefore if you choose hydrea as an option, it is best to take it gradually to let you body get used to it.

Hallu, i guess my post doesn't seem encourging, but i am telling you how i feel towards it and may be my opinion is a bit more emotional. But still my advice to you is to try every possile thing including hydrea before going through splenectomy.

hope i could be of any help

Manal 

Hi,

Does any one know if childen vaccination should be given when hydrea is been taken or not??
Ahmad is supposed to take Polio and meningits and i forgot to ask his hema. Does the decrease of WBCs prevents giving vaccination?

Manal

Hi Manal,

Hope it would help,

While you are being treated with hydroxyurea, and after you stop treatment with it, do not have any immunizations (vaccinations) without your doctor's approval . Hydroxyurea may lower your body's resistance and there is a chance you might get the infection the immunization is meant to prevent. In addition, other persons living in your household should not take oral polio vaccine since there is a chance they could pass the polio virus on to you. Also, avoid persons who have recently taken oral polio vaccine. Do not get close to them and do not stay in the same room with them for very long. If you cannot take these precautions, you should consider wearing a protective face mask that covers the nose and mouth

http://www.drugs.com/cons/hydrea.html

LOVE,

ZAINI.

Hi Manal,

Hallu, i guess my post doesn't seem encourging, but i am telling you how i feel towards it and may be my opinion is a bit more emotional. But still my advice to you is to try every possile thing including hydrea before going through splenectomy.

I know you are speaking from your heart and I cannot express in words how I felt when I read your reply. Thank you so much for the information you have provided and for the concern that you have shown. I'm so touched with your gesture.

My situation is that my spleen has enlarged from 1600 cc in volume to 2100 cc in one year. Although thankfully, my Hgb did not drop that much and is still around 8. (Precisely from 8.3 to 8.1 ... so can't really say if it has dropped). However, my bilrubin is getting higher...as of now it is  5.7.
So the doctor suggested that though there is no rush as the condition is so far stable, but it seems that I have to either go for splenectomy or start on hydroxyurea. It's not an emergency call but I should start some treatment soon before things go worse.
I'm thinking in the same way as you suggested, to keep splenectomy as the last option because an organ once gone is gone forever and I'll prefer to keep it for the maximum time. However, at the same time I'm confused that with a huge spleen whether hydroxy urea treatment can be succeessful or not?

I can not forget that at the end , it is a chemotherapy, though i know that the dose i am giving is not that highly toxic, but as a mother this is like a rock on my heart ( this is an arabic proverb, hope that the translation can make you feel how i really feel).

I can imagine what you must have gone through when you opted this treatment for Ahmad. If I'm so confused after being an adult, then it can be felt what you must have gone thru when deciding on this. And BTW, we have a similar proverb(for rock on heart) in hindi

I like your idea of trying natural HbF inducers; how did it work for Ahmad? I remember you had once started him on resveratrol.

Hi,

Does any one know if childen vaccination should be given when hydrea is been taken or not??
Ahmad is supposed to take Polio and meningits and i forgot to ask his hema. Does the decrease of WBCs prevents giving vaccination?

You should definately check with your haemotologist before vaccinating Ahmad for Polio and meningitis. I have read similar precautions which Zaini provides in her post w.r.t vaccinations during hydroxyurea treatment.

--Hallu