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Question about allergic reaction to Desferal

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LoriM:
A little background:  My daughter is 6 and has Beta Thal Major. 
In 2009 her liver biopsy showed her iron level at about 20.  She began ExJade and we eventually had to discontinue because she was having a lot of headaches.
In July 2009 her Ferriscan showed her iron level at 31.  She started Sub Q Desferal treatments about this time.
In July 2010 her Ferriscan showed her iron level at 24. We had to discontinue the Desferal because of the rash it was leaving on her stomach and because even after all this time she was hating getting the poke.  We resumed the ExJade and she did not get headaches this time around.
In July 2011 her Ferriscan showed her iron level at 35.5. 

Since just before Thanksgiving we have been going to the hospital every 3-4 weeks.  She is admitted and gets her transfusion on the first day and then 4 bags of IV Desferal (takes about 48 hours depending on the speed of the drip)

We were just in the hospital this week and she developed a red, itchy rash with very small red bumps on her torso during her second bag of Desferal.  (This time she was getting a higher dose than she has before).  Her chelation was stopped and after 48 hours of meds for the allergic reation, she still has some redness, tiny bumps and itching.  Her doctor was out of town on Friday so the hospital sent us home and next week he is supposed to come up with a new game plan.

I'm not sure what to think.  The ExJade isn't able to keep up with her iron and if she's allergic to the Desferal she is in big trouble. 

Any advice, suggestions, thoughts, comments?  Anything is helpful.

She is being seen at DeVos Children's Hospital in Grand Rapids, Michigan.  We are wondering if we should be making the trip to the Children's Hospital in Chicago every three weeks for treatments instead. 

I really am open to any and all input.

Thanks, Lori M, mom to beautiful Wei Wei

Andy Battaglia:
Hi Lori,

What is her weight and what was the daily dosage of Exjade? Did she tolerate Exjade well? What was the recent dosage of desferal over 24 hours that was taken IV? I can give better advice once I know the answers.

LoriM:
She is at 18.9 (I think that's 41 lbs)  She is taking two 125 mg tablest of ExJade twice daily.

She started on ExJade but had daily headaches that didn't go away.  She switched to Desferal but it left a nasty rash on her stomach at the injection site (which the docs ignored until it was unbearable) then back to ExJade.  She has been tolerating it well since returning to it.  But it doesn't seem to be keeping up. 

She's been doing four bags of Desferal every three weeks (about 48 hours) since just before Thanksgiving.  She was in last week and her torso broke out with small. itchy bumps and turned red.  The Desferal was discontinued.

I am very concerned because the ExJade isn't keeping up and now she's allergice to the Desferal. 

I am asking that she have a HFE mutation test done to see if she might also have herediatry hemochromatosis. 

I have asked the hospital to begin whatever procedure is necesssary to get her on the new chelation medication even though she is not an adult  I am guessing this could take a while and I don't want to wait until after we see if the Desferal desensitization works (if the doctor is even going to try that)

Thanks so very, very much!

Andy Battaglia:
Lori,

It is a good idea to have her checked for hemochromatosis as the liver iron levels are cause for concern.  Is her transfusion schedule normal? Has she had any antibody reactions that resulted in more frequent transfusions? Has she had long intervals with no or under-chelation?

As far as the IV desferal, is benadryl or another antihistamine administered during the IV to try to prevent the skin rash? Perhaps administering less desferal per session at a slower rate may also help. If she is not already getting it, she should be taking 100 mg vitamin C at the beginning of the desferal IV.

I suggest you contact Dr Thompson at Chicago and discuss whether a higher dose of Exjade could be used for awhile. The high end dose given for high iron load patients is 40 mg/kg but I don't know if this dose would be allowed for a child. Splitting the dose as you are doing is an excellent way to minimize the side effects. Keep in mind that it is also now permissible to mix Exjade with a variety of soft foods, which can also help if their is any nausea from the drug.

LoriM:
I spoke with her doctor's nurse yesterday and asked them to run the mutation check for hemochromatois the next time we come in.  (Jan 24)  Her transfusion schedule is pretty set.  It has varied between 4 and 3 weeks.  We went to four because of her high iron levels, but her Hgb as getting to low (down to 7.4 and 7.5 the last two times).  As far as antibody reactions requiring more frequent transfusions... I do not know about those.  She has been pretty consistant at 3 to 4 week intervals since we brought her home and started transfusions in 2007.

Her doctor was resistant to starting her on chelation when we first brought her home at 20 months of age.  But I knew she'd been having transfusions in China since six months of age so I kept pushing.  He finally started ExJade to shut me up, I think.  She has been on ExJade or Desferal consistantly since, with no long intervals without.  Underchelated, maybe... all I had to go on was his say-so on dose.


Benadryl or antihistamines are administered during the transfusion, but not during the IV Desferal.  The initial skin rash was ignored by the hospital staff for months and months.  I could not get them to take it seriously.  The most recent rash during the IV chelation was the first rash she'd had with IV chelation and was too severe.  The discontinued it per the pharmacist's advice.  She received steroids and antihistamines for 18 hours and we were sent home.

Thank you for the advice on the vitamin C.  If and when she can restart the IV Desferal, I will make sure that happens.  Would that be orally?  And something I would bring with me or something the hospital should have?

I have a call out to Dr Thompson and we are waiting for them to schedule us an appointment and an MRI. 

Do you know there is more than one type of Ferriscan?  She has been getting annual Ferriscans at the local hospital, but Chicago wants to do their own.  Also, her doctor wants to do a liver biopsy if her next Ferriscan is still high to verify the Ferriscan.  Does that make sense to you?

I spoke with the doctor's nurse yesterday and we may be trying to desensitize her to the Desferal (five day admission required)  I also asked about the posibility of starting to get an exception to allow her to take the new chelator.  My thought was I don't want to wait until we find out if the desensitization works before we start the process as I would imagine it could take some time.

Is hereditary hemochromatosis in Thal patients common?  It doesn't sound like a good combination to me.

Thank you so much for your advice and input.  I greatly appreciate you sharing your knowledge with me.
Lori

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