BAD NEWS

Mariam,

The more that I research and learn for your case, the more I am convinced that these ferritin readings do not reflect transfusion related iron overload in Aysam.  As Andy has said, the flareups - the elevated WBC and ferritin are probably correlating with each other. 

We are here for you,

Sharmin

they told me yestarday why his wbc are alway high ?
never have they been low in his life ?
um they are asking me i am suppse to have answers fo them

Mariam,

Your doctor's queries just provide more evidence that you need to be at a better center for your son's care.  Hopefully his issues will be resolved very soon. 

For now you can take comfort in a few things:

If the doctors felt that your son was in immediate danger they would have admitted him.
Other thalassemia patients have been in this situation(as per earlier posts)- similar symptoms - and they are now doing well.
Perhaps, your son's ferritin levels are not reflecting the actual iron concentration in his body - the rise in ferritin levels have been very drastic and unless if he has some other hereditary/non hereditary conditions that causes him to store iron - his iron levels are probably not as high as they ferritin levels indicate. 
Ferritin levels can be very fickle, they can change in response to other things in your body. 
I hope that the other clinic is able to resolve some of the other issues - and most likely the ferritin levels will look lower at that time too. 
It is very rare that a two year old child would actually have a LIC of 4300 after only 20 or so transfusions.  I don't think that a  ferriscan or SQUID will be available or suitable to your young child.  In your son's case, because of some of the other liver function levels it may be a good idea for a liver biopsy to be done.  If it is done at the center that Smurfette recommended to you, chances are that it will be very safe (as I have said, my son has had two liver biopsies and they were a breeze).  I think it would provide you with some very important information about what it actually going on if his liver tissue is studied.  At his age, he may not do too well with the ferriscan anyway. 

If you have questions about the liver biopsy you are welcome to ask me:)

Smurfette, have you ever had a liver biopsy?  Any others in Australia? 

Mariam,  your son's blood tests are producing very rapidly changing numbers, that seem to be all over the place within a short time.  Usually if ALT levels appear high they are tested for in 1 month time again.  In your son's case they increased in a matter of days.  It is difficult to say what exactly is causing these changes, the auto antibody he tested positive for may provide some clues - as well as the wbc and the fact that he is prone to frequent herpes episodes. 

We are all here for you Mariam - praying for you and looking for information - so don't feel alone.  It is not acceptable to any of us that our little Aysam - born in a time of such technical advances - should be suffering when his disease is a manageable one.  We will do everything we can to learn about what is going on with him to give him the best chance to get things 'on track'.  You are right, as his mother you NEED to be informed and you shouldn't settle for anything less - I wouldn't.  Hopefully the next clinic will give you all of the knowledge you need from them. 

Sharmin

Sharmin,

To answer your question on biopsies, Yes I have had many, but I wouldnt recommend them on a 2yr old...

They are much too painful for me let alone a small child..

I have spoken to Miriam, hopefully we can meet up on wednesday nite if I am able to, permitting i dont have prior engagements..

I have explained a few things to Miriam about why maybe her sons ferritin is up and his Lft's...

I am going to get my mum to come along with me too, so she can get a parents perspective as well...

I agree with you Sharmin, in todays world, the little man shouldnt be suffering the way he is..

I am so appaulled at that hospital..

I know Nov is a long way Mariam, but keep faith and it will all become clearer for you...

I promise that we will meet..

Take care


Oh and another thing, I have read most of the posts in here, I am alittle appaulled as to how many dont read posts properly or dont understand the..

We live in australia and there is no way in the world he could have contracted the Hep C virus or any other virus...I know that the blood banks do the right thing...but then again anything can happen YES...

I am sure that Miriam would have been notified long time ago if any of that happened..

So please read and understand the posts..

If I sound harsh, I dont mean too..but they way some posts are written they are misinterpreted and the respondant will panic and worry for no reason..

Sorry about this..

www.youtube.com

type in

baby stops breathing while asleep

you must all see this
its aysam
you will be shocked

they told me yestarday why his wbc are alway high ?
never have they been low in his life ?
um they are asking me i am suppse to have answers fo them

Hi Aysam,
most of times WBC are high in Thal  people. Normally a machine count the different cells, but the machine can't distinguish certain things.
You have to know that leukocytes (WBC) has an cell nucleus. Erythrocytes don't have an cell nucleus. It is known that thals have a lot of premature red cells (normoblasts) in their blood. And know it becomes tricky, this premature red cells contains a cell nucleus. Unfortunately the machine can't differentiate WBC from premature cells. Every cell with an nucleus will be count as leukocyte.

The only way to find out the real wbc count is to take a blood drop and make an blood smear. With an microscope the can count each type of cell in a blood smear.

Since I can think I remember only high WBC, except the times they have made a blood smear.

Best wishes for your little one

Smurfette,

Thanks again  You are a god sent for little Aysam, and having your mom there will be so helpful to Mariam.  
I wish that I could meet your mom, I have so much to learn from her.

This really is the best thing that could happen for little Aysam, it is comforting to know that he is in good hands now.  

Smurfette, when you had your liver biopsies were you awake?  My son, probably because he was very young, had his biopsies under general anesthesia and he did not experience any pain after the procedure.  I am thinking that it may be worthwhile doing just because Aysam's ferritin is very unlikely to 4300 - it is important to know what the iron levels in his body actually are.  Also, it will give us more information about his liver.  There really isn't any other way of measuring these things in a 2 year old.  What do you think?    

Once again, it is a great thing that you and your mom are doing!  Mariam and Aysam are lucky to have you there.  

Sharmin

A non invasive way to measure iron in liver is Ferriscan or Squid

This is a short info about SQUID
www.eiseninfo.de/kurzinfo.htm

Liver Iron Susceptometry with the Hamburg SQUID Biosusceptometer

 

The non-invasive quantification of liver (and spleen) iron concentration by means of an instrument like the Hamburg Biosusceptometer can replace the traditional method of taking a biopsy in order to evaluate the iron overload in patients suffering from Hemochromatosis and Thalassemia. This technique, also called magnetic biopsy measures the difference between the magnetic susceptibility of the liver and that of water. The iron concentration is calculated from the relation between the susceptibility of the liver and the specific susceptibility of the paramagnetic iron complex of ferritin and hemosiderin. The magnetic biopsy has three advantages compared with the traditionally used liver-biopsy:

- At first, it is a non-invasive method without any risk,

- secondly, the iron concentration is more representative and less influenced by the distribution of the iron in the liver as it is the case with the traditional liver biopsy,

- thirdly, the online result is at the doctor's and the patient's access immediately at the end of the measurement.

On the other hand, the biomagnetic susceptometry of the liver iron is strongly influenced by the presence of magnetic contaminants (the magnetic fields are one million times weaker than the magnetic field of the earth !). For example, this method cannot be applied in patients with a pacemaker. Other metallic objects ( metallic buttons in the underwear, jewellery) may disturb the magnetic field also and have to be removed. Ortodental objects or a port-a-cath certainly do disturb the magnetic field, however, the analysis can correct for these effects in most cases.

For some young patients (age < 5 y), eventually, a sedation has to be envisaged.

 

The procedure of biomagnetic liver iron susceptometry in Hamburg is the following:

Usually, we start with SQUID measurements from 8:30 a.m. during all working days. In case of a large group of patients, we devide them into groups of 8 persons each who are going to be measured in the morning and afternoon. Additionally, we can arrange a blood sampling (after overnight fasting and interruption of any chelation therapy for at least 12 hours) for the determination of NTBI, ferritin, transferrin saturation and other clinico-chemical parameter (extra arrangement necessary).

For the measurement with the SQUID biosusceptometer, the patients have to take off the shoes, coat, trousers (bras, body) and all their jewellery. They can leave T-shirt, slip and socks on.

        In a first phase we measure the liver volume (10 min) by sonography, adjust the appropriate position of the body for the susceptometry and determine the raw shape of the body (10 min). Then, we measure the distance between the liver and the skin and determine the ellipsoidal shape of the liver (5 min).

        Finally, we perform the liver iron quantification with the SQUID biosusceptometer (10 - 15 min). We use a water baloon (weight 7-9 kg) to simulate the magnetic properties of the body tissue overlying the liver (skin, muscles, fat, rips).

If the patient's spleen is big enough, (> 3-400 ml), we may also do a magnetic biopsy of the spleen with the same method as for the liver.

Here a link for ferriscan
http://www.ferriscan.com/

As I know they analyze also children age of 1,6 years with Squid i don't know about ferriscan.

Thanks for the info Gabri.  It is interesting to know that the SQUID may even give a better LIC than liver biopsy - because of the distribution of iron.  A biopsy is localized and may not give as accurate of an indication. 

My son has been doing SQUID tests for many years - it does involve some time lying down and holding your breath for some time.  But if they can do it for a 2 year old it would be best. 

My only argument for biopsy would be to test for possible pathology or issues with the liver - unless if there are better ways to study the health of liver. 

The ferriscan is not done on young children. 

Sharmin

Hi Miriam

As what Gabri said, a non-invasive way to measure liver iron concentration (LIC) might be a good option for young Aysam. LIC is able to give a good indication of the total body iron stores.

SQUID is not available in Australia but FerriScan is definitely available in Sydney. FerriScan do not have any side effect on children. The reason why it is not used for young children is that the procedure require the child to stay still during scanning process. (And we all know this is hard especially for young children) However, if this is a necessary procedure for the child, the MRI centre would usually offer ways to help to keep the child still. Eg: scanning the child when he is asleep. The MRI scan itself is not harmful to children. There is no contrast agent used and MRI uses magnetic and radio frequency (not radiation).

Please speak with the doctor about having a FerriScan during your next visit and see if he/she would recommend Aysam to have a FerriScan.

Kind regards
Sylvia