Thalassemia Patients and Friends

Discussion Forums => Thalassemia Intermedia => Topic started by: Manal on January 27, 2010, 01:45:29 AM

Title: Evaluating hydroxyurea after one year
Post by: Manal on January 27, 2010, 01:45:29 AM
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) :wah :wah
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 :(
Title: Re: Evaluating hydroxyurea after one year
Post by: Andy Battaglia on January 27, 2010, 03:15:03 AM
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.
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on January 27, 2010, 04:46:49 AM
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 :wah

Thank you so much Andy

Manal
Title: Re: Evaluating hydroxyurea after one year
Post by: Andy Battaglia on January 27, 2010, 05:51:27 AM
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.
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on January 29, 2010, 01:53:24 PM
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  :biggrin 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
Title: Re: Evaluating hydroxyurea after one year
Post by: Sharmin on January 29, 2010, 04:27:50 PM
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
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on January 30, 2010, 11:53:26 AM
Thanks Sharmin so much for your wishes :hugfriend Sure i will keep you updated

Take care

Manal
Title: Re: Evaluating hydroxyurea after one year
Post by: mohamed on January 31, 2010, 07:09:26 AM
dear Manal,

-so sorry of the low Hb level :rolleyes   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

Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on February 01, 2010, 02:49:00 AM
Thanks Mohamed for the re-assurance :biggrin

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

manal
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on February 03, 2010, 02:06:35 AM
Today we did the ferriscan to my son, what an expierence.... :whew

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
Title: Re: Evaluating hydroxyurea after one year
Post by: Andy Battaglia on February 03, 2010, 02:49:37 AM
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.
Title: Re: Evaluating hydroxyurea after one year
Post by: Sharmin on February 03, 2010, 06:21:42 AM
Hi Manal,

First of all, good job Mr. Ahmad  :hugfriend :hugfriend 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
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on February 03, 2010, 09:53:04 AM
Andy, Sharmin,

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

Manal
Title: Re: Evaluating hydroxyurea after one year
Post by: zahra on February 03, 2010, 10:06:47 AM
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
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on February 03, 2010, 10:30:52 AM
Thank you Zahra for your wishes :hugfriend :hugfriend

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 :wink

Manal
Title: Re: Evaluating hydroxyurea after one year
Post by: Dori on February 03, 2010, 01:04:01 PM
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.
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on February 04, 2010, 01:53:08 AM
Quote
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 :dunno

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 :huh

manal

Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on February 04, 2010, 02:01:13 AM
In spite of the low HB, there are some good news. :biggrin

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
Title: Re: Evaluating hydroxyurea after one year
Post by: Andy Battaglia on February 04, 2010, 04:18:16 AM
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
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on February 09, 2010, 12:55:33 AM
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

Title: Re: Evaluating hydroxyurea after one year
Post by: Andy Battaglia on February 09, 2010, 02:54:33 AM
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:

Quote
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.
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on February 09, 2010, 03:09:25 AM
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.
Title: Re: Evaluating hydroxyurea after one year
Post by: mohamed on February 09, 2010, 04:56:19 AM
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.
 
Title: Re: Evaluating hydroxyurea after one year
Post by: baal on February 09, 2010, 06:17:42 AM
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
Title: Re: Evaluating hydroxyurea after one year
Post by: Zaini on February 09, 2010, 07:51:25 AM
Manal,

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

Zaini.
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on February 09, 2010, 10:59:37 AM
Mohamed

Thank you my friend for your kind wishes :wink. 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
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on February 09, 2010, 11:07:57 AM
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
Title: Re: Evaluating hydroxyurea after one year
Post by: Dori on February 10, 2010, 07:46:50 PM
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 :)
Title: Re: Evaluating hydroxyurea after one year
Post by: Andy Battaglia on February 10, 2010, 07:49:37 PM
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
Title: Re: Evaluating hydroxyurea after one year
Post by: Sharmin on February 10, 2010, 08:48:20 PM
Dore,

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

Sharmin
Title: Re: Evaluating hydroxyurea after one year
Post by: Bostonian_04 on February 23, 2010, 05:35:10 PM
Manal,
Just read this posting. I am very happy to see that AHmad is doing wonderful and his iron is also in normal range. All the credit goes to your continued hard work and vigilence. Very happy for you....
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on February 25, 2010, 12:56:36 AM
Bostonian, thank you so much for your kind words....but actually all the credits goes to Andy and everyone here in this wonderful place. I just follow your advices backed with all your continous support :hugfriend :hugfriend

manal
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on March 11, 2010, 01:06:13 AM
Hello my friends,

A quick updade about my son...to remeind you with his treatment, last January we increased his dose of hydroxurea to 500mg daily (23mg/kilo) and L-carnitine was given daily instead of 5 days per week
His results before and after the change in dose:

HB                   Before 6.7          After 6.7
RBCs count       Before 3.82         After 2.80
Hematocrit        Before 24.7%     After 19%
MCV                 Before 64           After 67
Ret count         Before 8.5%       After 3.2%
Bilirubin             Before 2             After 1.8
WBCs count      Before 5,100       After 5,300

The doctor lowered the dose to 15mg/kilo for the hydroxyurea and L-carnitine is back to 5 days again and i am really comfortable for this change because we never had the RBCs count that low, it is always in the range of 3.5 to 3.8 so i am afraid that the marrow is very suppressed with no increase in the HB.... Am i right Andy????????

The doctor still didn't lose hope of hydroxy with my son because of the single increased he had last year, but she said we are trying to fine tune the dose in this stage, wish us luck


The doctor was happy with the ferriscan result and the echo and the ECG were normal too.
The spleen is 5 cm large and liver is 3cm large

Before my next visit, i am thinking of doing an HPLC to check the level of fetal Hb too

Manal

 
Title: Re: Evaluating hydroxyurea after one year
Post by: Andy Battaglia on March 11, 2010, 03:45:07 AM
Manal,

Yes, some fine tuning is needed. The RBC and retic count drops are a little more than what's desired. Does he have a good appetite?
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on March 11, 2010, 04:48:35 AM
Yes Andy, his appetite is okay and sometimes vey good but he hardly gain weight

manal
Title: Re: Evaluating hydroxyurea after one year
Post by: Andy Battaglia on March 17, 2010, 03:00:30 AM
Hi Manal,

I just posted in the about hydroxyurea and nitric oxide in the Documents section. Reading the results of the study made me wonder if you should experiment with different brands of L-carnitine or try it in combination with another supplement. It might be worth thinking about trying to see if you can any change in Hb. One other little piece of information I ran across mentioned that L-carnitine worked better when the subjects ate a lot of carbohydrates.
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on March 17, 2010, 05:24:18 AM
Thank you Andy do much

I read the post in the document section and will see if there are other brands here in the market that induces NO in blood like what you mentioned
Quote
L-carntine, L-arginine and combinations of Alpha Lipoic Acid and Acetyl L-Carnitine, or L-arginine and lysine


I was too interested in the powder Bobby is taking and wheather it is suitale for children or not???

http://www.bodybuilding.com/store/bsn/xplode.html

manal
Title: Re: Evaluating hydroxyurea after one year
Post by: Andy Battaglia on March 17, 2010, 05:27:33 AM
Way down at the bottom of the label, it says

Quote
This product is only intended to be consumed by healthy adults 18-50 years of age.
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on March 17, 2010, 05:33:18 AM
Yes Andy i saw it but some of the supplements we use like resveratrol for example, have the same note too
Quote
used by 18 years and above

I guess it is really difficult to decide :-\ :huh

manal
Title: Re: Evaluating hydroxyurea after one year
Post by: Andy Battaglia on March 17, 2010, 05:42:47 AM
Because this supplement has a proprietary blend and we do not know what is in that, we have no way of knowing if it's safe for a child. Being that it's a bodybuilding product, I would say it should be adults only.
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on March 17, 2010, 05:52:54 AM
Thanks Andy, it better be safe
Title: Re: Evaluating hydroxyurea after one year
Post by: Dori on March 30, 2010, 09:52:41 PM
What was said on the conference in Berlin is that all thals should try hydrea. (I think trying it for a year or so). Does someone post already about the important things that were told in Berlin?
Also I have heard people like us should eat something every two hours (to keep the energy up to date) and one glas of water every 2 hours (although that was more said about sc).

I hope it helps a bit.
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on June 20, 2010, 03:03:02 PM
Quote
A quick updade about my son...to remeind you with his treatment, last January we increased his dose of hydroxurea to 500mg daily (23mg/kilo) and L-carnitine was given daily instead of 5 days per week
His results before and after the change in dose:

HB                   Before 6.7          After 6.7
RBCs count       Before 3.82         After 2.80
Hematocrit        Before 24.7%     After 19%
MCV                 Before 64           After 67
Ret count         Before 8.5%       After 3.2%
Bilirubin             Before 2             After 1.8
WBCs count      Before 5,100       After 5,300

The doctor lowered the dose to 15mg/kilo for the hydroxyurea and L-carnitine is back to 5 days again and i am really comfortable for this change because we never had the RBCs count that low, it is always in the range of 3.5 to 3.8 so i am afraid that the marrow is very suppressed with no increase in the HB.... Am i right Andy??

The doctor still didn't lose hope of hydroxy with my son because of the single increased he had last year, but she said we are trying to fine tune the dose in this stage, wish us luck


The doctor was happy with the ferriscan result and the echo and the ECG were normal too.
The spleen is 5 cm large and liver is 3cm large

Before my next visit, i am thinking of doing an HPLC to check the level of fetal Hb too


Dear friends
The above quote was to remind you of my last situation.

Yesterday, i had a visit to the doctor after three months. During those 3 months we lowered the hydroxy to 15mg/kilo and L-carnitine was back to 5 days/week.

I am so happy to tell you that Ahmad Hb is 7.7 (increased by 1 gram), i am soooooooo happy (I know this reading is so low to all of you :rotfl, but it is the highest i have ever seen and wish i can see higher and higher)

Anyway, below I am comparing his results according to the dosing
                  HU 17.5- 20mg/kg               HU 23mg/kg                    HU 15mg/kg
                 +L-carnitine 5/week                +L-carnitine daily             +L-carnitine 5/week
 
                    (feb2009- feb2010)       (one month only)             (last three months)
HB                    6.7                                6.7                                 7.7
RBCs count        3.82                              2.80                                3.69
Hematocrit         24.7%                           19%                               24.1%
MCV                  64                                  67                                 65.3
Ret count          8.5%                             3.2%                               8.3%
Bilirubin              2                                   1.8                                  2.1
WBCs count       5,100                            5,300                               7,500


So his readings today is the one in the third column.

Andy, I was wondering........
1-Why the reticulocyte count is not decreasing
2- We have been for one year on a dose between 17.5mg to 20mg/kilo and i saw no change in Hb, do you think that taking the 15mg/kg, is reason for the increase in HB although all other readings are the same

When my son was diagnoised in 2006, the HPLC showed 11.4% of fetal Hb.

Yesterday, i did another HPLC and found that the fetal HB is 27.5% (after using HU)

Hopefully, i will do another one after three months, to check it again, but during this time i will be giving my son all possible natural  fetal HB inducers (resveratrol, carao, aloe vera, wheat grass) in addition to HU in order to see if they will help all togather or not

As for kidney and liver functions, they are normal, thanks God
His weight is in the 25th-50th percentile
His height is in the 50th percentile

Love you all

manal



Title: Re: Evaluating hydroxyurea after one year
Post by: Sharmin on June 20, 2010, 06:21:11 PM
Manal,

That is wonderful! I am so happy for Ahmad (thank god).  I hope his hg remains high and that he continues to thrive:) 

Sharmin
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on June 20, 2010, 09:13:09 PM
Thank you Sharmin for your wishes. I hope so too :hugfriend

Manal
Title: Re: Evaluating hydroxyurea after one year
Post by: Andy Battaglia on June 20, 2010, 09:34:26 PM
Manal,

Th retic count should be higher than normal at this point because there has been a spike in Hb and HbF, and this would be indicated by an increase in new red blood cells being formed. I think dropping the hydroxyurea dose has had a positive effect and perhaps a happy medium has been reached where there is less bone marrow suppression, allowing an increase in HbF production. However, I think it is more than that, as the L-carntine and magnesium (you are giving magnesium, correct?) in combination with hydroxyurea are working as expected. If possible, you may want to try to duplicate the homeopathic treatment that little A has been receiving to see if that can also boost the HbF. It has been shown that all the various Hb inducers have a cumulative effect, so please do add the other inducers.

One lesson here is that patience is required with hydroxyurea. Even though some effect may be measured in months, the real value of hydroxyurea becomes more apparent in the second and third years of use.
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on June 20, 2010, 11:22:41 PM
Andy,

Do you think dropping the dose more will have a better effect??

As for the magnesium, i used to give it regularly except for a while till my new shippment of the supplement arrives

Definetly i would love to try the homeopathic treatment, but i think that Sharmin's doctor won't give his ''recipy'' through corresponence and unfortunatly, we don't have this kind of treatment here (though there are some doctors but working without a lisence :( :()

 
Title: Re: Evaluating hydroxyurea after one year
Post by: Sharmin on June 21, 2010, 05:28:05 AM
Hi Manal and Andy,

i will be speaking with the homeopathic doc soon.  He will probably give me the ingredients in the meds when I meet with him.  Unfortunately I have not been able to see him as my son's radial fracture seems to have slipped after recovering.  He will now require further treatment to his arm.  We will see the orthopedic surgeon tomorrow to determine what method of treatment he will require.  I am praying that the recovery will not take too long so that he can get back to playing the sports he loves. 

Sorry for the delay, i will post the ingredients as soon as we get this issue sorted out.

Best,

Sharmin
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on June 21, 2010, 08:21:18 AM
Sharmin,

Wish little A a speedy recovery.

Is he still on steriods??? I think they don't help in healing fractions, correct me if i am wrong

Give him a big hug for me :hugfriend

manal
Title: Re: Evaluating hydroxyurea after one year
Post by: Sharmin on June 24, 2010, 09:15:09 PM
Thanks Manal,

Little A's surgery went well.  He had two pins put in his wrist and is in a cast again.  I hope that his recovery goes well this time.  I also think that the prednisone may have had an effect on how the fracture healed.  I feel that the prednisone is not helping his hemoglobin at all, because he had no improvement with the prednisone - instead he responds to the homeopathic meds.  I am tempted to ask the doctor to wean him off of the steroids until his arm heals this time.  Poor kid has been through so much.  The had to rebreak and straighten his wrist :(  He is being a trouper though. 

I hope all is going well! 

Sharmin
Title: Re: Evaluating hydroxyurea after one year
Post by: Zaini on June 25, 2010, 06:19:43 AM
Sharmin,

I am really sorry to hear about Little A'a arm,give him a big hug for me plz  :hugfriend .

Zaini.
Title: Re: Evaluating hydroxyurea after one year
Post by: Dori on June 25, 2010, 06:42:13 AM
I expect his arm is swollen? My father had an operation to get those pins out plates out. Poor arms. I hope his arm heals soon and that he may wean off prediisone. He handles all well?
 :hugfriend
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on June 25, 2010, 10:39:50 AM
Dear little A :hugfriend

Wish you a speedy recovery my sweet heart, love you so much :hugfriend

manal
Title: Re: Evaluating hydroxyurea after one year
Post by: Sharmin on June 28, 2010, 05:24:41 AM
Thank you so much Zaini, Dore and Manal,

He is doing well so far.  His attitude has been better than I expected.  He is on summer holidays - I so wish he could be out playing hockey and soccer but the docs have asked him to be extra careful this time.  Your wishes mean so much to us  :hugfriend

much love,

Sharmin
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on October 05, 2010, 05:45:46 PM
Hi Andy

Could there be any defiency in the body that is causing hemolysis????

1-Last March Ahmad's HB was 6.7,(high dose of hydrea)
2- June it was 7.7 (Lowered the dose and at that time we agreed that this is the suitale dose for his body)
3- August it was 7.2 (same low dose)
4- Today it is 6.7 again and he was NOT ill since August, so WHY????????????????????

I was assuming that if he was not ill the hb -if not increased-, it will be at least stable. What could be dropping the HB, is there anything i can test???????????????????????

PS
I noticed that in the two times i increased the dose, the result after one month is usually low but the following month, it is in the peak ( up to 1 gram increase and then starts declining). So the question is could be this big increase due to an accumalative effect of the drug that appeared after a couple of month and started declining because i lowered the dose again????

I don't think that lowering the dose could be the reason ( for having the highest reading in HB)because he was on low dose for a long time and with no significant increase

I really don't know does what i say make sense or thereis no relation????

 Anyway i will be at the clinic in a couple of hours :(

manal
Title: Re: Evaluating hydroxyurea after one year
Post by: Andy Battaglia on October 05, 2010, 06:18:53 PM
Hi Manal,

Has there also been any change in his retic count or bilirubin levels? These would give some indication if hemolysis is taking place.
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on October 05, 2010, 07:16:22 PM
Hi Andy

1- March the retic count was 3.2%, bilirubin 1.8 , hb 6.7 (result after one month of high dose)

2- June the retic count was 8.3, bilirubin 2.1 , hb 7.7

3- August the retic count was 4.3 %, bilirubin 1.9 , hb 7.2

4- Today the retic count was 8.5 %, bilirubin 2.2 , hb 6.7


When the hb was high in june the retic was high and when it was low as of todaythe reticwas high too. howcome :huh :huh

Also for the first time in his life the platlets are lower than the normal, 110,000 (normal 150,000 - 450,000)
Title: Re: Evaluating hydroxyurea after one year
Post by: Andy Battaglia on October 05, 2010, 08:02:20 PM
I looked back at the tests you posted at the beginning of this thread and noticed that he also had a drop in Hb after summer of 2009. Is this possibly a seasonal change? Is there any change in him physically with the lower Hb?
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on October 05, 2010, 08:12:07 PM
This drop was due to continous infections he had during July and August 2009, so the drop was justified.

But from this last August everything was fine that is why i am frustrated for this decrease.

As for his activity, it is the same. but definetly he is paler

manal
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on October 06, 2010, 12:06:51 AM
Just came back from the clinic.

The doctor said that in thal there is no justification for the decrease and there is no pattern.

She asked us to do another CBC after one week after increasing the hydrea dose to 450mg instead of 400 and lowered the dose of L-carnitine, but taken daily instead of 5/week. She also doulbed the folic acid dose. All this for this week until we do the CBC

Last August, the spleen was 3 cm enlarged, but today it is 7 cm enlarged. So she said that this is the reason for the hemolysis and also this is the reason for the decrease in the platlets as the spleen is ''eating'' them.

Please your prayers are extremely needed
Manal
Title: Re: Evaluating hydroxyurea after one year
Post by: Andy Battaglia on October 06, 2010, 01:09:16 AM
Manal,

I know the list is based on reactions in alpha thalassemia, but take a look at the list in this post to see if your son may have had exposure to anything that might cause hemolysis. http://www.thalassemiapatientsandfriends.com/index.php?topic=3410.msg34830#msg34830


Meanwhile, I have posted in a new topic asking for any ideas for reducing spleen size. One of our adult members recently told me she may have to have her spleen removed because it is 26 cm. Incredibly, she is used to living with it at around 20 cm. I am really hoping we get some suggestions, so that she can find a way to keep her spleen.
Title: Re: Evaluating hydroxyurea after one year
Post by: Sharmin on October 06, 2010, 01:59:04 AM
Manal,

My prayers are always with you and little Ahmad.  I hope and pray that his spleen will shrink and that his hemoglobin will increase.  I am hopeful that some new method of treatment for thal intermedia will be helpful to you very soon.  Have you ever tried nigella?  

The homeopathic medication I was giving lil A worked initially but eventually had no effect and we so we had to repeat the ritux - we abandoned the use as I think there may have been a link between the homeopathic medication and his loss of appetite.  Otherwise I would have recommended that to you.  

I am sending you lots of love and prayers,


:pray :pray

Sharmin
Title: Re: Evaluating hydroxyurea after one year
Post by: Zaini on October 06, 2010, 05:56:53 PM
Manal,

I am sure Ahmed's hb will get back to his normal levels,we are all praying for you and Ahmed,couldn't this be due to hot weather? coz i notice Z goes pale fast in summer then in winter.

Praying for you,keep us updated. :hugfriend

Zaini.
Title: Re: Evaluating hydroxyurea after one year
Post by: Babynorth on October 07, 2010, 07:37:08 PM
Dear Manal, I do not have enough knowledge to give you advice. But I will like to give you this  :hugfriend
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on October 08, 2010, 01:19:56 AM
Andy
Thank you so much for your concern :hugfriend. I will check the post and concerningthe list, to my knowledge, he was not exposed to any of those things

Sharmin
Thank you So much my friend. I have started the nigella today but the oil is only available right now, hope it works :hugfriend

Zaini
I really do hope so my friend :hugfriend. I know what you mean about the weather, but actually his Hb usually increases in the summer rather than the winter. May be in the summer he sleeps more and eat better than when he is in school

Babynorth
Thank you my dear, this means a lot :hugfriend

Manal

Title: Re: Evaluating hydroxyurea after one year
Post by: Sharmin on October 08, 2010, 02:08:40 AM
I wish you the best with the nigella Manal.  I used to give him nigella with honey and aloe vera juice - it seemed to work at the time. 

Please keep us posted,

Sharmin
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on October 08, 2010, 08:54:12 AM
Thanks Sharmin, definetly i will :hugfriend
Title: Re: Evaluating hydroxyurea after one year
Post by: maha on October 08, 2010, 11:10:59 AM
The nigella oil is also very good Manal. But adding honey is necessary to bind the stools otherwise the stools would be very loose and watery. Wishing you and Ahmad all the very best.

maha
Title: Re: Evaluating hydroxyurea after one year
Post by: Zaini on October 08, 2010, 06:50:26 PM
Manal,

I am sorry if i am getting this wrong but nigella seeds aren't available there?

Zaini.
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on October 09, 2010, 01:40:45 AM
Thanks Maha for your wishes :hugfriend

Zaini
Actually we have nigella here, but Ahmad hates the taste, so i used to get him a supplement that has the full seed grinded inside a capsule, he used to swallow it not knowing what is inside, but this brand for unknown reason is not found, so i am getting him some other capsules but they have the oil only, which is better than nothing 

Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on October 16, 2010, 11:39:58 PM
Quote
She asked us to do another CBC after one week after increasing the hydrea dose to 450mg instead of 400 and lowered the dose of L-carnitine, but taken daily instead of 5/week. She also doulbed the folic acid dose. All this for this week until we do the CBC

I did the CBC and the hb was 6.8, platlets count back to normal and retic decreased by 1% (it is 7.5% now). The spleen decreased by 2 cm (it is 5cm now). The doctor said we will continue with the same dose

I will be ordering the red root and hope that it will help decrease the spleen

Manal
Title: Re: Evaluating hydroxyurea after one year
Post by: Andy Battaglia on October 17, 2010, 01:26:34 AM
Manal,

That is encouraging to see some small improvements after only one week. It may be that there will be a need for some regular fine tuning of the dosages.
Title: Re: Evaluating hydroxyurea after one year
Post by: Sharmin on October 17, 2010, 02:49:05 AM
Manal,

Thank you for the update.  It seems like things are better.  I am so glad to hear that his spleen size has decreased.  I hope that things continue to improve.  I will continue my prayers. 

Sending you my best,

Sharmin
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on October 17, 2010, 04:59:32 AM
Thank you Andy and Sharmin so much for your replies. I really hope things will be better :hugfriend
manal
Title: Re: Evaluating hydroxyurea after one year
Post by: olivia mary on November 02, 2010, 03:26:59 AM
Manal I hope you son is doing better!  :hugfriend You are an amazing mother! and your family is in my prayers. Olivia recieved her transfusion today and during her exam the MD told me her spleen was 3cm. What happens now? Do we just watch it? at what point do they talk about removing it?

Thanks Kathleen  :biggrin
Title: Re: Evaluating hydroxyurea after one year
Post by: Manal on May 06, 2011, 05:40:19 PM
Hi Kathleen
If Olivia is on regular transfusion I guess she should have a normal spleen as she keeping a high level of hb, never the less 3 cm is not something to worry about.

I remember from the conference that I attended that the spleen is removed when
1- it is over sized and it threatening the patient's life by rapture
2-when it is destroying the red blood cells causing continous drop in hb and therefore decreasing the gaps between transfusions and this will be accompanied with slow growth of the child because spleen enlargement   Consumes the body's energy and therefore decreases the rate of growth.
Manal