Evaluating hydroxyurea after one year

Dear friends,

Find attached to the post a written summary for my son's results for the past year. He started the hydrea on February 10th, 2009.

I also did a CBC yesterday and was soooo disappointed of the results (HB 6.7) because physically he looks better than his hb shows, actually I  was expecting more than 7.5 cause his face looks good to me  

By the end of this week i will be doing the echo and the ferriscan

As for today, i did his annual abdominal and pelvic ultrasound and the significant points were:
1- Several gravels in gall bladder (this is leading to a little bit enlarged colon)
2-  Total spleen length is 13.5cm (normal for his age is 9.5 cm), I wonder how big it will be without hydroxyurea
3- Liver is fine, no enlargement
4- No extra medullary hematoposeis detected
5- Other organs are completly normal


Andy,
1-If you have a chance to see the attachement, could you tell if hydrea is working for my son or not??
Just for one month, the HB was 7.8 and actually the doctor told me to consider it 8 and for the rest of the year it was not that high. I know that he got sick several times, but also i can not see a satisfactory increase, what do you think???

2- Also this hematocrit thing is really getting me crazy because what i know is that the bigger it is, the higher the HB.
You will see that yesterday's CBC showed an increase in hematocrit (24.7%) this is the highest he had ever reached since he was diagnosed and still you can find his HB very low, do you have any explaination for this???

3- Does the change in the dosage of hydrea has any significance??

Waiting for your feedack

Manal,

Have the HbF levels also been recorded? These would give you a better idea if the hydroxyurea is increasing the Hb, as it would ne noticed in rising HbF levels. I do think that there may be some other benefits from the hydroxyurea. You mentioned spleen size and I noted that the other organs were not enlarged and that there was no indication of extramedullary hematoposeis. The hydroxyurea may be having some effect in this regard.

Hematocrit can be affected by factors like hydration level, RBC count and blood plasma volume, so it can be inaccurate. Hemoglobin measurements are considered to be more accurate. I will mention that every source points out that each patient is an individual and a hemoglobin insufficient for one person may be adequate for another. Your objective observations of your son's physical health and development are just as significant as the blood tests.

Thank you Andy for your quick reply

In the year 2007, my son was on hydrea for 6 months. Before starting hydrea, his fetal HB was 11% (by HPLC) and after those six months it became around 30% (By HPLC), so it did increase, but didn't reflect that much on the total HB (his HB was around 7.5)

Unfortunatly, this time i didn't do the HPLC but i think i can do one and compare it to his normal levels which was 11% on 2007. But again what is the benefit of having an increase in fetal HB if it didn't reflect on the total HB

I agree with you that hydrea has other benefits that we can not neglect (though i am getting worried about the spleen size in the last couple of months), but i too can not ignore that it is mainly taken to increase HB

Have you ever heard of anyone gaining higer levels after using it for more than one year as in my son's case??

I too agree of the importance of clinical observation and it is very good as the doctors and I observe, maybe this is the only thing that keeps me going on ( i will be posting some recent photos of him too in the gallery),  but i really can not ignore the fact of being so worried cause i have nothing to count on (hydrea) and can not predict what will happen

Thank you so much Andy

Manal

Manal,

The increase in HbF does show that the hydroxyurea is working. The goal is to increase production from the gamma gene, while reducing the ineffective erythropoiesis in the bone marrow. Every study I have seen has shown a continuing increase in HbF into and after the second year. I would definitely recommend patience. The benefit to the blood by reducing the excess alpha chains should not be overlooked. The reduction and prevention of extramedullary hematopoiesis, which is extremely common in non-transfused intermedias is another major benefit.
There is a nice powerpoint about the use of hydroxyurea in thalassemia at
http://www.thalassaemia.org.cy/pdf/syria/day%202/Day%202%204.pdf

Note the other positive effects of hydroxyurea, in addition to HbF induction, including the neutralization of the excess alpha chains by the gamma chains. The excess alpha chains are one of the biggest problems in thalassemia and their reduction is a big positive.

Thank you Andy so much for the non stop information you provide us, makes things very clear.

I went to the hematologist and she said the same thing too  and the dose of the hydrea now is 500mg daily and L-carnitine will be taken daily too

The very good news is that she is so happy with his growth. He is now very close to 50th percentile

Will be updating you on the echo and the ferriscan as soon as  i get the results.

Thank you Andy for everything.

Manal

Manal,

I am so glad to hear that!  I wish Ahmad all the best.  Please keep us posted on how he does with the new dose of hydrea and the daily L-carnitine.

Love,
Sharmin

Thanks Sharmin so much for your wishes Sure i will keep you updated

Take care

Manal

dear Manal,

-so sorry of the low Hb level   but this may be only due to the infections he got. so keep on administrating OHurea and do not give up. you r doing great to ur son.
-about other thalassemics taking OHxy urea: i see some them regularly in Dr Ali Maklouf clinic of Anu El Reesh. so i can give you feedback about their evaluation.
-about spleen: do not worry.it is not a big deal, also it can decrease in size again
-about gall bladder gravels:So Sorry to hear that there are gravels.
overall you are doing great job and put in your mind that Ahmed is quite normal with COMPLETELY NORMAL LIFE. always consider that Ahmed is A NORMAL PERSON WHO IS ONLY TAKING SOME MEDICATIONS
Mohamed

Thanks Mohamed for the re-assurance

Hope everything is going well with you and good luck in your paper

manal

Today we did the ferriscan to my son, what an expierence....

Thanks God it all went well and the result will be in 15 days because it takes tiime, since it is analysied in Australia.

I must admit i was too worried but it had to be down

Manal

Manal,

I am so glad he had the Ferriscan. This is very important because this will tell you if his iron absorption from food is normal or excessive. This is extremely important for planning his treatment program.

Hi Manal,

First of all, good job Mr. Ahmad  So proud of you! 
Secondly, yes, this test is very important for intermedias so that you can stay on top of his actual iron levels.  I hope that the results tell you that you have nothing to worry about. 

All the best to you and Ahmad,

love,

Sharmin

Andy, Sharmin,

Thank you for your support, i am glad he did it too. I hope the results will be satisfactory

Manal

Manal,
 I am so glad to find out that Ahmed's fetal Hb has increased. I hope his spleen will improve and ferriscan results will be good.

Andy,
I was just wondering how they measure the effect of hydroxy urea when it is tried without stopping transfusions? Would there still be a rise in fetal HB in an HPLC? My sons doctors mentioned that when they start it initially they will try to space out transfusions instead of stopping them. They plan on starting both hydoxy urea and exjade in May when he turns two but it will be one by one. The doc said that exjade is mostly tolerated well but hydroxy urea sometimes has side effects. Any advice on what to watch out for or monitor?
Zahra

Thank you Zahra for your wishes

When taking hydroxyurea, you should be monitering kidney and liver results each month until you are sure it doesn't affect any of these functions. When you are sure of this, these tests are done every 3 months and then twice a year. The good thing is that if any negative change occurs to these functions they are completly reversed once you stop the hydroxy, so no worry from this point.

To know if hydroxy is working or not (in case of transfusions), you will find that the gap between transfusions has increased and it was reported in some thal major cases (mentioned in the above study that Andy has provided) that sometimes transfusion was eliminated
http://www.thalassaemia.org.cy/pdf/syria/day%202/Day%202%204.pdf

Also you have to do an HPLC before treatment to know the level of Fetal HB before and after treatment. It is supposed that the transfused blood has no  fetal Hb so if there is any increase in the level of fetal hb, it is the patient's  own increase

Personally, my son felt some stomache ache when he took it for the first time, but what i did is that  i started giving it just before going to bed to avoid the feeling of dizziness. The last week and due to some circumstances, i had to give it to him in the mid of the day and found out that he did well and had no trouble which indicates that the body has got used to it

Please feel free to ask whatever you want

Manal