Evaluating hydroxyurea after one year

I went to the Dutch Hematology Congres and was told the name of Hydrea changed in the soon future. Just due the simple fact that an other compagny get the rights of this med.
I have friends who used it. One stopped already and the other will stop it soon because it's just not working for her. I often wonder of it will help me (does someone know?) but I dont know.

was told the name of Hydrea changed in the soon future. Just due the simple fact that an other compagny get the rights of this med.

I heard that the company will be sold or may be already sold. There is the Italian and the American hydroxurea. I use the Italian one, but  I find it with difficulty.....so may be this is the one being sold

To answer your question, I think using hydrea is a personal expierence that differs from one person to another.

But since you are not a thal you have to check whether the defiency of pk enzyme affect the fetal hb or not, because if it does there is no use of using it.

Please Andy correct me if i am wrong

manal

In spite of the low HB, there are some good news.

Today i was at the endocrinologist, she was happy with Ahmad's growth and she commented that she does not bother with growth as much as the growth rate and being constant and his rate is very good.

It has been two years now since she has been examining my son. There is a certain equation she uses with ( 0 ) as a reference to the normal and as she told me it is accepted for a chronic diseased patient to reach (-2).

Two years ago Ahmad was (-0.8) and today he is (-0.6) and she said that she will see him in 6 months instead of 3 months (Thanks God)

I also must add that i feel that hydrea increases
1- Activity
2- Apetite
3- Growth


Manal

PK deficiency affects the red cell membrane, causing early death of the RBC's. Because it is not a hemoglobin problem, I don't know if hydroxyurea would have any benefit.

For new developments towards a cure for Pk deficiency see http://www.thalassemiapatientsandfriends.com/index.php?topic=3368.msg34308#msg34308

Finally, i got the result for the ferriscan as follows:

Average Liver Iron Concentration  1.2 mg/g dry tissue  (Normal 0.17 - 1.8 )
                                                               21 mmol/Kg dry tissue ( Normal 3 - 33 )


I am definetly happy that the LIC is between the normal range, but i feel it is rather in the high normal or close to, what do you think Andy??

Anybody has any idea if this MRI shows stones in the gall bladder or not???

How frequent should i do this scan???

The rest of the report said that every thing is okay and all organs are normal except for mild hepatosplenomegaly which is normal in case of untransfused thal


Manal

Manal,

The LIC is really good and shows that there hasn't been any significant iron absorption yet. However, iron load in non-transfusing intermedias can slowly build up over the years, so some regular monitoring is recommended. You do have a baseline measurement now, which will be helpful over the years.
The Standard of Care Guidelines for Thalassemia have this to say:

18.1.6 Assessment of iron overload.
Ferritin and iron saturation levels should be monitored
annually. If the ferritin levels are persistently greater than
1,000 ng/mL or the iron saturation is greater than 60
percent, obtain a quantitative assessment of liver iron.

Quantitative assessment of liver iron refers to the scan or biopsy. This will only be necessary to do once his ferritin stays above 1000 over several months. For now, continue with a low iron diet and encourage tea drinking with meals. I think hydroxyurea also has an impact, since overall bone marrow activity will be lower, which in turn leads to a lower rate of iron absorption from food.

Thank you Andy for your reply

My son's ferritin is 64 now, and i think that this proves what doctors said in conference about how tricky ferritin could be in intermedias.

But as you said, that now i have a reference to compare to.

hi manal
Firstly: I want to congratulate u for good news concerning Ahmed's growth.
Secondry: i would like to know exactly what is the difference between LIC and T2 MRI assessment of liver iron
Thirdly: i dunno if LIC can show gallbladder stones or not but you can do abdominal sonar for stones.

mohamed.
 

hi manal

here in europe the brandname of hydroxurea is

"LITALIR"....

they have it in caps and in injektions...

grats for the good work....panos

Manal,

I am so glad to hear that Ahmad's ferriscan came back in normal ranges  i hope the trend will continue,and i hope ferriscan would be available for Pakistani patients soon.

Zaini.

Mohamed

Thank you my friend for your kind wishes . I am not sure if  understand your question properly, but in the scan that i did the liver iron concentration (LIC) is measured by ferriscan (this is a software or technology that analyse the MRI taken to show the LIC.

Please check this post
http://www.thalassemiapatientsandfriends.com/index.php?topic=1677.msg22876

There are three ways to know LIC:
1- Ferriscan  (it is a normal MRI and not an invasive procedure)
2- SQUID (available in limited countries)
3- Liver biopsy (invasive procedure, and may not show the exact concentration because the iron is not evenly distributed in the tissues of the liver so the sample taken may not show the real concentration)

As for the heart iron concentration, it is measured by T2*


Please feel free to discuss any other points

Manal

Panos,

Thanks a lot for yur wishes. Thanks for the information, i really didn't know that hydroxurea is available in this name


Zaini
Thank you dear and i wish the same too. I also hope it will be in Pakistan soon. Actually it is not available here except in one center which is used by Novartis to moniter the clinical trails for exjade, so maybe Novartis in Pakistan could help in this. I think it is just a deal the center do with the ferriscan company to send the MRI film through the computer to be analysed in Australia (as we do here) as the center will be using their normal MRI machines. Hope this will be available soon my friend

Manal

PK deficiency affects the red cell membrane, causing early death of the RBC's. Because it is not a hemoglobin problem, I don't know if hydroxyurea would have any benefit.

For new developments towards a cure for Pk deficiency see http://www.thalassemiapatientsandfriends.com/index.php?topic=3368.msg34308#msg34308

I won't benefit from it. It was a nice try. My hopes for a kind of "cure" for PK deficiency are minimal. But for thal I am not that negative

Dore,

I wouldn't say you will never see a cure. Please see my recent post at http://www.thalassemiapatientsandfriends.com/index.php?topic=3368.msg34308#msg34308

Dore,

I hope that you see a cure for PKD very soon.  

Sharmin