Eight months from starting hydroxyurea

Hello everyone

Just an update for my son and some questions too.

I did a CBC yesterday and results wereas follows:

Hb: 7
Hematocrit: 23% (has increased)
Red cell count: 3.55
MCV: 65 (increased)
Leucocytic count: 6,600
Platelets: 237000
Biliruin: 1.8

All kidney and liver functions are normal

More than a month ago, i did the TSH and was a bit high than the normal, 4.5 and the range is (0.3- 4.3), so the doctor ordered a full test for thyroid and the results were:

Free T3: 3.66 (2.5-5.3)
Free T4: 0.8  (0.9-1.8)
TSH: 1.803 (0.3- 4.3)

So the TSH is back to normal, thanks God but the Free T4 is now a little low than the normal, should i be concerned or it is aminor difference. Andy could you please comment on this

On Sunday i will be visiting the doctor but last month, his weight and height was normal for his age and also the rate of growth was normal too.

Concerning the hydroxyurea, three months after we started it, his Hb reached almost 8 (actually it was 7.9) then the following two he had several infections and the Hb went down to 6.5 again due tothe infection.

Now he has been fine from mid of September, why the HB hasn't gone up again?? Why???
I am so frustrated because any time he can geta coldnow (due to weather changes) and the the HB will fall again.

It is as if i am up the stairs but i am can not reach the top, just standing in my place and infections have no mercy

Andy
Ahmad is taking 350 mg daily (17.5 per kilo) now of hydrea, do you think the dose should be increased though i prefer not to exceed the 20mg per kilo as recommended by the Iranina studies to be a safe dose with no complications

manal

Manal,

You could bump the hydroxyurea up to 20 mg per kilo but not higher.

Has he had any scans yet? Ferriscan is available in Egypt now and I highly recommend it. Before any other thyroid analysis is made, I think iron load should be assessed. Iron affects the glands quickly, so this is an important factor. Even though his ferritin has held low, we learned once again at the conference, that in non-transfusing intermedias, scans need to be done because ferritin is not an accurate measurement of iron load.

Hi Manal,

It is good to see his hg begin to increase again.  I hope that it gets back up to 80 and beyond soon, and that he stays safe from infections.  Remember, he has been at an age where he will tend to get every cold and infection out there - but then he will stop getting sick so often because he will already have been exposed to most of the common infections. 

I was going to mention what Andy said as well, be sure to check his glands for iron as the ferritin is not a very good measure of iron overload in thal intermedias.

Hugs to Ahmad 

Sharmin

Thank you Andy for your reply.

When we were in Singapore, i discussed doing ferriscan for him with some doctors (including his doctor) and they told me that starting the age of 10 i can go for the T2* and the ferriscan.

When i meet his doctor on Sunday, i will repeat asking for the scan.

But what does a low free T4 may indicate? And is it that low?

manal

Thanks Sharmin

Is there a test to measure iron overload in the thyroid gland?? Or we evaluate only the liver and the heart??

Manal,

I think that it is a good idea to measure the heart and the liver, but I think that mri can also measure other glands in the body.  Dr. Wood will be doing some tests for Lil A measuring his pituitary gland and his pancreas.  I wonder if looking at the pituitary would be helpful because the pituitary affects thyroid levels as well.

Perhaps some chelation at some point may be required - depending on the results. 

Sharmin

T4 tests free thyroxine, so a low test may indicate a need for further evaluation.

In a non-transfused patient, a liver iron scan will give the first indication that correction by chelation may be needed. If gland scans are available, they are a very useful tool, but the liver scan is where it should all start.

This involves another of the questions I posed to Dr Wood. At what age should scans begin? The new protocol is to begin one year after transfusions begin. The children are sedated, but it has been found that iron begins to build almost immediately after transfusions so monitoring begins one year later. Yes, this does mean two year old kids are now being scanned. I think with intermedias who do not transfuse, that scans should also be done to see if excess iron is being absorbed from the diet, as this problem is far more recognized these days.

may Allah bless ur son, Manal................

Thanks Keep Smiling

Manal

I have visited the doctor, she agreed to increase dose of hydroxyurea to 20mg/kilo. Also we increased the L-carnitine again to 5 days per week instead of 4. We will see how things will go after this change in dose

Concerning the scans, the doctor said that he won't need this before he is 10 years and didn't seem annoyed about the slight decrease of T4. She is more concerned about having a normal TSH, but any way i will repeat these tests next month

Generally he is good except that the spleen is 6.5cm enlarged (hope the increase the dose of hydroxy will help supress the spleen). On the contrary, the liver size has decreased so much,it is only 1cm enlarged, we never had this small number before

My son these days love to play football and do wrestling with his friends, i am so scared he would hurt his spleen and he does not seem to understand to avoid close contact

The good thing is that his height increased 1 cm since our last visit which was a month and half.

Generally he is active, thanks God

manal

Manal,


please do not be scared at all and let him enjoy his life and do what he likes.
Otherwise, he will feel different.

Lena.

Yes Lena, i agree.

I just tell him every now and then without stressing and that is why he doesn't seem to care. I do not stress at all because as you said i don't want him to feel any different but at the same time i can not help being worried. But better me than him

Thank you my friend hope you are enjoying your day

manal

I understand your being worried but you'll be able to find a way to handle it.

And yes Manal, I am enjoying my day-- today I started translating Naguib Mahfouz. His book is "hadith al sabah oua al masaa" and as always there is a lot of arabic atmosphere in it. (although only non-Arabs can talk of arabic atmosphere and its magic--for the Arabs this atmosphere is part of their lives.)

Take care,

Lena.

This is for Manal and Sharmin,

About the thyroid and pituitary glands..


I am a thal minor but I have not yet tested my DNA. I am very symptomatic too. Always unwell with something.

I am hypothyroid, and I also had a pituatary microadenoma for a few years, that was treated with pills. In some cases the two can be related. I am sorry I dont know the medical details of how it all works.

My concern always was that i felt iron made me very sick, though my ferritin was always 10. Hemoglobin is also usually 9.5.

But around the time i Stopped taking iron on a trial basis, the pituatary problem became normal and I was able to stop the medication. My ferritin and Hemoglobin are still same.

I also posted on another place about diabetes. I think i am concerned if any extra iron in my body may hurt or cause my organs to function abnormally.

Another thing i noticed, many people who have hormonal problems, often have more than one. Sometimes my pancreatic hormones are out of range, other times we check my cortisol, etc.  Maybe they are all inter related at times?


From what i understand, its always better to have a regular hormonal evaluation too, to see whatever health issues we can prevent.

(I am not a doctor so forgive me if i dont make sense sometimes.      )

 

Lena

This is a great book. I have read, but i really enjoyed i more when i saw the T.V series that was produced some years ago. It had more of this magic you are talking about. I will check it is available on CD, i will senditto you

Good luck with the  book, Naguib Mahfouz's novels seem simple but very deep in their meanings. In his novels you can feel the atmosphere of the alleys, the houses, people and places that he talks about

manal