New Member, please help me.

Dear All,
I am a new member in this forum.
Actually My son is detected with HbE & beta Thalassemia six month back when he was sick .In general his Hb  lies between 8 to 8.5  but last time when he was sick his Hb reached to 7.3 and then 6.5. we are advised to go for electrophoresis test and it was identified as HbE-Beta thalassemia.
His HPLC report is
HbA=8.3%
HbA2=62.7%
HbF=23.2%
My wife is beta minor(HbA2=5.1%), Hb=10.3
My self is HbE trait.(HbA2=23%), Hb=14.99

After that we met haematology doctor here in Delhi(INDIA) and since then my son is advise to take Hydrea (250mg) and folvite(2.5mg).
At present his Hb is 9.1, but bilirubin(SB)=3.3 and platelets=605
Doctor told me it is quite normal. Is it?

Hi dip1973 and welcome,

The bilirubin level rises as red blood cells are filtered out and destroyed by the spleen. As you have seen, this process accelerates when the child is ill and the Hb drops. The bilirubin is a by product of the break down of the red blood cells and is not much of a concern. I would advise to try the hydroxyurea and folate program, as this can raise the hemoglobin level and may also help slow the breakdown of the red cells. It does require some patience, as the effects of hydroxyurea can take two years or more before the full effect is seen. This program is a good alternative to transfusion, and since the Hb level has dropped as low as 6.5, some action is required. I will also mention that hydroxyurea has a stronger effect when magnesium and L-carntine are also added as supplements. Our moderator, Manal has a son on hydroxyurea and may have some advice on the dosing.

How old is your son?

Hello Andy,
Thanks for you reply.
My son is 4yrs 6month old.
he is taking Hydrea from last 6 month.
his Hb was 6.5 befor 7 month when he was sick and it reaches to 7.3after 1 week  but doctor's advise was to transfuse him as his serrum iron was low and he was suffering from fever due to infection.
so my son is transfused befor 7 month (1st time). and after then he is on hydrea.
at present his hb is 9.1
Since last 7 month we have recorded his hb
and it was.
9.5(5week after transfusion) on 31st Jan
8.3 on 28th Feb
7.8 on 26th March
8.9 on 10th april
8.5 on 20th may
9.1 on 1st july.

his platelets are increasing currently it is 605, is it common?

Platelet readings are often wrong. It takes an experienced tech to distinguish between platelets and the irregular red blood cells found in thalassemia, so often the level is inflated by mis-reading. I always say test again and preferably at a different lab if possible. This is one of the most commonly found errors in testing regarding thalassemia, so don't be too concerned about this. The level is only slightly above normal. so that is also nothing to worry about, even if accurate.

The Hb levels since starting hydroxurea are encouraging.

Hello Andy, Hello manal.
I am Giving 250mg Hydrea to my son whose weight is 14 Kg only. Is it correct.
actually one doctor recommend  125mg and another doctor  250mg. I am little bit confused.
I am also giving 2.5mg folic acid.
please advise.
thank you.

Hello dip1973 and welcome to the site

Your son is taking around 17mg per kilo and usually Hydrea is given starting from 10mg per kilo up to 35mg per kilo (in some of the sickle cell patients).

So the dose he is taking is okay in terms of miligrams, but the important thing about hydrea is not the dose as much as at what dose the body will  react positivly. Personally my son reacted a little better when his dose was lowered to 15mg/kg rather than 23mg/kilo.

As for safty, studies showed that it is safe taking hydrea starting 10mg/kg until 20mg/kg

So it is important to fine tune the dose until you get the maxiumum benefit and don't forget to check the kidney and liver function in addition to white blood cells count at least every 3 months while your son is on hydrea.

Please feel free to ask any other thing

Take care
manal

Hello Manal Thank you for your reply.
I  started with 125mg hydrea dose for my son before 6 month ,then last three month the dose was 175mg (although doctor advise was 250mg) and his Hb was recorded as above. Now in the month July I started to give him 250mg does and the hb test is not done so far.If you see the above result i feel that 175mg hydrea shows at least reasonable response then is it required to increase his does up to 250mg.
as you have good experience on this medicine can you please advise me.


LFT was done before 3 month, other than high SB(Bilirubine =3.3) every things was fine.
KFT is not done so far.
and after every 30-45 days we check the CBC.

a

Hi dip1973,

Increasing or decreasing the dose will actually depend on his respone to the dose, it is lke trial and error. So moinitor his CBC after starting the 250mg and we will see how thiings will go. Please do check te kidney functions with the liver functions too

Do you know the percentage of his fetal HB before starting hydrea??

manal

After reading some of the posts about using hydroxyurea I'm curious if people with a-thalassemia tend to have low uric acid levels.  Mine are always low, and when I would question doctors about the significance they would just say I should be glad I don't have high levels. 

I have what appears to be HgH disease - I'll know for sure once the gene sequencing is done. 

Something else that is interesting to me is that I have one mutation of the MTHFR gene.  With one mutation folic acid is not as readily available because not as much is converted to folate.  People with two mutations can't convert any folic acid to folate and have to use the converted form of methylfolate as a supplement instead. 

Jan

Hello Manal
thank you for your advise.
I wiil go for CBC test in next 2weeks and will update the results.
before starting hydrea his Hb was in between 8.0 to 8.5

As I earlier mention also that my sons platelets are high about 605 and doctor advise him to take small amount of oral iron medicine.
so far I know that thal should not take oral iron ,is it true?
his RBC count in last CBC was 4.6 and SF was 65.

Hi dip1973

before starting hydrea his Hb was in between 8.0 to 8.5

I was asking about the fetal HB, not the total HB. You can read about fetal HB in this link
http://www.thalassemiapatientsandfriends.com/index.php?topic=3595.msg36626#msg36626

As for the platlets, i advise you to recheck them because in thal patients, some of the fragmented red blood cells are counted by unskilled lab technicions as platlets because they are similar in shape under microscope.

The decision of taking iron is done after doing a full iron studies and intermedias are not supposed to take iron by any means because they are already absoarbing an above normal percentage of iron from food in the gut due to their low HB.

manal

Hello Manal thanks for the reply.

My son's HbF was 23% as per HPLC test done 7 month before.

That high HbF makes him a perfect candidate for hydroxyurea. I would also question why iron has been suggested. What is the iron dose and what form is it?

Hello Andy,
Thanks for your response.
I have been advise to give my son oral medicine TONOFERON DROPS.
Each 20drops contains equivalent of elemental iron 25mg.
Doctor told me that his serum ferretine is low (SF=67) and platelets are high, so may be iron deficiency.
I am supposed to give him 10 drops for two month, initially I gave him 5 drops for 10-15 days but then I have stopped.

Dip

Dip

Having a serum ferritin of 67 is not low. My son is in the fifties and his doctor is fine with this. Are the platlets high in all his CBCs??

manal