1st Pan-Middle Eastern Conference on Haemoglobinopathies ..May 2009, Syria

Mariam, what is Aysam's weight and dose of exjade

manal

his weight is  14kilo his on 375 a day

just need to know
doctor says that his iron is high but it wont affect until 10years so we have time to take his levels down
i asked for a biopsy and she said that no need for on we will wait first she don think the liver or any organs is damegd ?
well a the confrence in singapore i heard a doc say he did liver biopsys fortwo pautents one with high feritin one with low and the shocking thing was the one with low ferritin levels had damage to the heart and liver so not everton is the same i suppose dont you agree ?

Mariam, the chelating dose for his weight should be at least 420 mg. Usually it takes up to a year to see a decrease in ferritin when you start the chelating dose.

In aysam's case i believe so much that taking desferal via IV for a while will help so much in decreasing these levels.

As for organ damage, i think that only the MRI or the biopsy are the only tools that tells you about the damage

manal

420 wow big diffrence from what he is on now
the doc just started him on 375 last week
i dont knwo manal the dc said in a few months she wll also put up the dose
yes you are right that the organ damage nowne can now until mri or biopsy well this doc thinks she nows everythink
and no they will not do iv for him they have refused to do this
manal his hb is each time going down faster why you think ?
his live gose up then down like a roler coaster
if he has alreday damage in live dose it sow in his bloods and also

3400 ferritin to high ?
i am going to lebanon all i waitting for his aysam papper work itr is takeing so long he needs to be lebenisse to get cheaper hostpital there so iam leaveing this country and i am praying by may my sons papper work are finished as i wont to take aysam to lebaonon and syria

manal you there candian famaliy anyone

Hi Mariam
I can understand how frustrating it can be when you are doing everything right and not seeing the results you expect. Have patience and inshallah you will be able to see a drop in his ferritin. Last month my son`s SF was over 3000 and this month it is 1858. You will be able to see a drop in his SF soon.
Lots of love to little Aysam
maha

i am so glad and hapy to hear ur
angles iron has drped so fast
bu sweety in my sons case its been from september last year untill now his ferritin lebvels are in the high ow it has gone down um maybe 300  only

Mariam
What time do you give exjade to Aysam. Is it the same time everyday? Are you using a plastic spoon and cup to mix exjade? You have to wait for atleast half an hour before he gets to eat anything? If you are following all of these and still his ferritin isn`t dropping then like Andy said for some it takes six months to one yearfor the SF to drop.You just have to play the waiting game.
take care
maha

Mariam, the doctor is right about starting the exjade with a low dose until the body gets used to it to avoid the side effects, then this dose is increased to the chelating one which is the effective one. Even the effective one takes time to see results. The effective or the chelating dose is 30mg for every kilo, so for aysam's weight , it is 14 kilos x 30mg = 420mg
This is the end dose he should reach according to his weight but the doctor has to start with a low dose until he/she reaches the the chelating one.

I am sure that when you go to Lebonan, they will agree about the desferal via IV . Good luck in all your procedures.

As for the increased hemolysis, was aysam ill or had any infections recently. Infection usually cause the Hb level to decrease more than the normal frequency. If not, an antibody test should be done to detect if there are any antibodies in his system that contribute to this hemolysis. The name of the test ids Coomb's test

manal

every day at 5oo t 600 am i isolve it in a bit of water and apple juice and just shake it in the bottle he only takes it in the bottle the only way he will
and you think manal that in lebaonon they will do iv desfral

Hi Mariam,

Exjade should be stirred and not shaken. Stir with a wooden or plastic spoon, but never expose Exjade to metal. Do not skip any days. As He gets up to the recommended dose you will begin to see more progress.

http://www.thalassemiapatientsandfriends.com/index.php?topic=1928.msg16165#msg16165

2.1 Dosing Information
The recommended initial daily dose of Exjade is 20 mg/kg body weight.
Take Exjade once daily on an empty stomach at least 30 minutes before food, preferably at the same time
each day. Do not chew tablets or swallow them whole. Do not take Exjade with aluminum-containing
antacid products. Calculate doses (mg/kg per day) to the nearest whole tablet. Completely disperse tablets
by stirring in water, orange juice, or apple juice until a fine suspension is obtained. Disperse doses of <1 g
in 3.5 ounces of liquid and doses of ≥1 g in 7.0 ounces of liquid. After swallowing the suspension,
resuspend any residue in a small volume of liquid and swallow.
Individualize the decision to remove accumulated iron based on anticipated clinical benefit and risks of
Exjade therapy. In patients who are in need of iron chelation therapy, it is recommended that therapy with
Exjade (deferasirox) be started when a patient has evidence of chronic iron overload, such as the
transfusion of approximately 100 mL/kg of packed red blood cells (approximately 20 units for a 40-kg
patient) and a serum ferritin consistently >1000 mcg/L.
2.2 Dose Modifications
After commencing initial therapy, monitor serum ferritin every month and adjust the dose of Exjade if
necessary every 3-6 months based on serum ferritin trends. Make dose adjustments in steps of 5 or
10 mg/kg and tailor adjustments to the individual patient’s response and therapeutic goals (maintenance or
reduction of body iron burden). If the serum ferritin falls consistently below 500 mcg/L, consider
temporarily interrupting therapy with Exjade. Do not exceed Exjade doses of 30 mg/kg per day since
there is limited experience with doses above this level.
The risk of toxicity of Exjade may be increased when inappropriately high doses are given in patients
with low iron burden or with serum ferritin levels that are only slightly elevated. The safety and efficacy
of Exjade when administered with other iron chelation therapy have not been established.

Yes Mariam, i think so because the way of management of thal is the same in both Egypt and Lebonan and this is what they do here. It will also be a good idea if you emailed Dr. Ali Taher and ask him about his opinion on this

manal

manal
i did email dr ali taher last night no reply he hasnt ever replied to me not once

HI professer how are you ?

i met you before at singapore

 

question doctor please



i am comeing to lebanon hopefully very soon im just finishing my kids pappers to become lebanese and this is what has taken so long

but inshala this is happeing in the comeing months my concern for the time being is



























 

aysam who is born 1/5/06

weight   14 kilos

height   91.6

 

is takeing exjade

the dotors here in autralia took your advise and put the dose up to 375 wich he has started two weeks ago the transfussions stayed as they are one every four weeks

220mls of blood

his liver funnction test seem to be playing up every

 two weeks anyways my concern is his ferritin

always over 3000 it has maybe gone done couple of grams it was 4000

i give him exjade at 5am to  6am

 every morning mix it with apple juice

so i am doing everything right

i have asked for liver biopsy

but she said that at this point

no need for the biopsy she is suggestion to wait if the iron dosnt come down in a few months than we do one and if she finds the liver is damaged than it will be life threating wich then she will tell me he might die and also she is saying at this momment the iron is high but wont damage the liver until ten years i dont know this is very confussing to me you are the profsional please

i have one boy and never am i going to have kids again he meens so much to me i want him to live a normal life and when they talk to me about death it really scares me tell me doctor untill i am able to come to lebaonon what shall i do his iron is arond 3600 and he is needing blood every three weeks his stomach is always bloated and he isnt realy getting taller he always blinks alout i dont know if this has anythig to do with thalassemia but his couler is of white and when i did a xtray for his chest for a infection they notice a lession in his arm this is by accident they saw it they say its due to the bone marrow please doctor get back to me with your advise as soon as possible

i am going through depression at this becuse i know i cant go to lebaonon until he is a lebanese citizen for the chronic center i have saved 4 months of exjade and the hospital are going to give me 7 months supply so i will be ok for one year as i was told that they are very expensive in  lebanon

please professer ali taher help me hep him

i will be waitting for yor email

 
 

kind reagrds

mariam saba