Any info about Liver Issues in Thal Major children???

Hi all,

A friend of mine has a 5 year old little girl who is in the hospital. I don't know all the details but apparently she went in complaining that she had stomach pains.  At first, they thought it may be a blood infection or her kidneys.  Now they are saying she is having some kind of liver issue.  I can post more as I get more information but if there are any older topics relating to this issue for thal majors please let me know.

Also, if you may have any insight on this - let me know.  I believe she started to be transfused around 1 year old.  She may also post - but I wanted to get it started in case she doesn't have a chance. 

THanks in advance

Is she on Exjade?

Yes

OK. I ask because often thals are being treated in emergency situations by doctors unfamiliar with thal or its treatments. If they are not already aware, the doctors should be told about the liver and kidney function issues that can arise with Exjade use. Her creatinine and ALTs should be tested. If they are high, Exjade is the likely cause and should be temporarily stopped.

The liver and gall bladder are quite close in proximity - gall bladder issues are more common in this age group than liver - I'm sure the docs have considered this - but I wonder if there is a relation. 

As Andy said, Exjade may be having an effect on the liver. 

Sending my best to your friend's little girl, please keep us posted,

Sharmin

A duct blockage is also a possibility and is also more common in thals, but perhaps it is worth mentioning because it may not be suspected in her age group, but when thal is taken into consideration, the possibility is increased. I think when dealing with doctors who don't often see thal patients, that we should err on the safe side and make sure the doctors are aware of the implications of being thal.

Thanks Andy and Sharmin.  I have relayed the info to her and I think she's is good hands as she was at CHOP.  The last I heard they ruled it down to a kidney infection as her liver results were fine.

The other thing she posted on her FB page was that the little girl had to get a breathing tube due to respitory issues.  I haven't heard from her on details or anything else so I don't have much info as to what happened.  Once things settle for her I'll ask her what happened in the end.

 

I hope you hear soon from her and that the little one is doing well.
Keep us up to date.
I must add that when the fe levels are low Exjade likes "to play with" the liver (painful).
Warmest wishes, -D

I was finally able to get in touch with my friend via email.  What she told me is not pleasant. Its actually very discouraging....her daughter ended up in icu for a week. Please read her email below. Any thoughts or experiences that have been similar?

"sorry I haven't been able to answer, my daughter had multiple organ failure last Saturday:( what a nightmare..... The scarest week of our lives:( she had a kidney inf and from what the docs said mixed with the EXJADE triggered the organ failure...... Yes I know we were all shocked. So as of now she will not be taking EXJADE again EVER....we are going the old fashioned way of using the pump through the stomach every night it's the safest and has been around 30+ yrs:) so they really can pin point everything but hemetology have def ruled that the EXJADE did have a major part of what happened:(((((( she's been on it for 3yrs...... And the power of prayer has brought my little girl back to us!!!!!!!!! She has to follow up with alot of doctors but she's here with us and that's all that matters:) please when your son has to start chelation think really hard of you want EXJADE to be the drug of choice ..... Talk to docs and try and go the old fashioned way of the pump:) Ty for praying and thinking of my family"

What a frightening experience!  I am so glad that her little girls is safe!  Exjade has been known to cause problems for some people.  Little A has severe stomach aches and loss of appetite with exjade, symptoms he did not suffer before.  Desferal on the other hand does not help reach iron levels below 1000 and has effects on bones and growth.  It is very difficult to know what the right thing is.  Perhaps some combination of the drugs in small doses will be the answer.  I wish L1 were an option for us in North America and as Andy often indicates - starch desferal could be a life saver.

Sending prayers to this family for a full recovery for their daughter.

Sharmin

Its scary,as Sharmin said,its hard to decide,even with L1 there are joint pains and the risk of neutropenia so we can only pray that our kids remain safe.