Exjade doesn't seem to be working

My daughter is 6 and has Beta Thal Major. She transfused every three weeks and is usually in the low 9's when transfused. She has been on Exjade for a long time now and her iron levels continue to rise. In October she had a ferriscan and her iron level was 35,500. Every three weeks starting just before Thanksgiving she was admitted for transfusion followed by 48 hours of IV desferal chelation. In January, she developed a rash over 3/4 of her torso and the desferal was stopped. We had another Ferriscan and her levels are still at 35,500.  Her doctor was "happy" with the results, according to his nurse. Why, I have no idea. I am trying to get her case transferred to another doctor but you need permission to do that. I am furious that her doctor's main course of action is "wait and see". But that is a whole other story. After the reaction I did some research and asked that she be given the DNA test for hereditary hydrochromatosis. It took almost three months of pestering to get that done. We do not have the results back yet. At 2.5 years of age her liver biopsy showed her iron level at 20,000. Now it is at 35,500. I have had no luck finding info in on line that tells me what these levels mean and help me think this through. All I know this is a "dangerous" level according to her doctor. (yet he is happy here .... Argh).   She has been on either Exjade or sub q desferal since 2.5 years of age.

I would also appreciate any suggestions on how to read her CBC as some of her levels are outside the norm (glucose is the one I can remember off the top of my head). Are there any tests I need to ask for?  Any info I should be armed with because her doc is clueless.

Her headaches and stomache aches are increasing as well.

We will see the doctor at Children's Memorial in Chicago on April 19.

Thanks so much. I feel like I am fighting for her life and instead of helping me the doctor is standing in my way!

Lori

Lori,

Can you tell us the amount of desferal and exjade she is taking?  You may be able (under very very close monitoring by doctors) be best off giving her maximum does of both chelators because they work synergestically.  She should be on desferal 7 days a week 12 hours a day.  She should be getting exjade twice daily.  

i will write more later,

She is two 250 mg tablets daily. We last did desferal in January. She was admitted and would get 48 hours of desferal thru an IV. She developed an allergic reaction and treatment was discontinued.  The doctor has yet to come with a new course of action.

Anyone know what her iron levels should be in her liver. I know we want her ferritin to get to 500 before we decrease chelation (it is currently 3400). But what should her liver be at. (currently at 35,500)

Hi LoriM,

My daughter is 5 and 1/2...so almost 6 yrs...She weigh's ~ 50kg. When her ferritin was high we were giving her  exjade using the dose of 30mg/kg of ...so that will mean, 50X30 = 1500mg/day of exjade. Your daughter probably needs dose similar to this daily if she is also around 50kg. For that dose, we split the dose in half and gave 3 250mg tabs in the morning and 3 250mg tabs in the evening, everyday. Do note that with exjade, you want to monitor all kidley and liver function tests monthly.  we recently came back from oakland and the doctor mentioned that we should try to keep the ferritin between 400 & 700.
As per Dr. Lal, the liver iron should be between 3-6 mg for a regularly transfused child on regular chelation. I am not sure what the 35,500 number is. sorry, couldn't help there.

She only weighs 18.9 kg.  She's pretty small.  

Her ferritin is 3400.  

The Ferriscan said 34,000.  I know that is in the "danger" zone.  I'm wondering if the levels you were told would translate to 3,000-6,000??  

Another problem I have is that the tests are run and nothing is ever done.  Her liver has been kicking out the enzymes that tell us it is in trouble for a long time.  

Her glucose is high and this was kept from me.  We go back for another transfusion on April 10 and I will ask about a fasting glucose test, I think.

Her ferritan and Ferriscan numbers keep going up.  Nothing brings them down.  I pushed and pushed to get her tested for the hereditary iron-overload.  I called today and that result is not in yet.  Her Ferriscan is exactly the same now as it was in the Fall of 2011.  Every 3 weeks we went in for IV desferal treatments, but in January she developed an allergic reaction and treatment was discontinued.  I've been waiting since January for her doctor to come  up with a plan of action.  He was waiting on the Ferriscan.  I have no idea what he plans to do now.  

We see the specialist at Children's Memorial in Chicago on April 19.  I am hoping to be educated enough to have a good conversation with her.  

Thanks, Lori M

Sorry Ferritin is 3400.  Ferriscan was 35,500

Hi Lori,

I am not familiar with the Ferriscan score being expressed like this. Do you have access to the report? There should be something that translates the score into liver iron concentration or LIC. This number would be expressed as mg/g dry tissue.. That would be helpful if you can provide that score. I think the Exjade dose should be a bit higher because of the high ferritin level. I would suggest 500 mg 4 times a week and 750 mg 3 times each week. At what age did she start chelation? Has her ferritin been high for several years?
Knowing the results for the Hereditary Hemochromatosis test is essential.

Have you been to Chicago Memorial before? You should be able to get proper guidance there.

Sorry Ferritin is 3400.  Ferriscan was 35,500

Lori,
I'm confused about the ferriscan results.
It should be measured in mg/g dry liver tissue. My son's ferriscan results (when just home from China) was 17.4 and this was labelled by Boston Hospital Children's (Neufeld) as "severe iron overload." One year later, 10 of those months on 500 mg Ejxade (he was 36 pounds), and his ferriscan was 4.1 which was in the NORMAL range.
I'd ask for a copy of the ferriscan results if you don't already have them. And ask HOW they are measuring. What does 35,500 mean???
Nicole

Thanks for responding.  I do not know why it is measured like this.  I only know what I was told. We go again on Tuesday. I will ask for a copy of the results.  She started chelation between 2.5 and 3 years old. Yes, her ferritin has always been high.  I was told she was in the "danger" zone as far as the ferriscan reading goes.  That is all I know. Her levels have continual gone up on ExJade. The only time they declined was when she was on Desferal. But she had an allergic reaction the last time we were in the hospital. (January)

I called about the heredity hemochromatosis test and somehow it never got ordered. They ordered tests for two variants, but somehow didn't order the one specific for the Asian population. Just another blunder.

We went to Children's Memorial in Chicago when she first came home. She was about 20 months. They were supposed to coordinate her care with the local doctor, but I do not think our local hematologist has been in contact with them.   We go again April 19th and hope and pray for some good solid advice. On Tuesday we are talking to the hospital about switching doctors. Seems when a medical group starts buying up all the medical facilities in the area they think they can decide which doctor you have to see, regardless of the patients' confidence in their care.  I feel like the hospital is working against us. All I want is to keep her as healthy as possible for as long as possible and they don't seem to care at all.

Sorry... I am just so frustrated.

Lori

I got a print out of my daughter's ferriscan:

Based on Ferriscan analysis of T2 data, average liver iron concentration is:

35.5 mg/g dry tissue
636 mold/kg dry tissue

Overall, distribution of liver iron is homogeneous and skewed dense iron concentration throughout the iron.

The spleen is not enlarged.

We have to discontinue her Exjade as her kidneys are now in distress. Not much, yet. But given her iron levels are steadily rising with the Exjade we have been told to discontinue use.

Our doctor has said we need to start thinking about BMT. But given she is a Chiinese adoptee, I don't hold out much hope for that.

He is going to look into a waiver so she can go on the new chelation med. I think he referred to it as L1.

We do go to Chicago next week and will have more answers then.

I don't think he likes passing on bad news. Who does?  But I need honesty and not run around.

Thanks all

Any input is appreciated.

Lori

Lori...I feel your pain, as do most of us. Stay positive and please keep us updated. You know your child more than the Doctors do...remember that!

James

Can anyone point me to a chart of any sort that will show me exactly how high her iron level is?  I am having no luck on my own. The doctor did tell me that she could live for several years with her levels thos high, but I want to know as much as I can find out. I just need someone to be honest with me about what we are facing and i don't feel like he is.

Thanks, Lori

We have to discontinue her Exjade as her kidneys are now in distress. Not much, yet. But given her iron levels are steadily rising with the Exjade we have been told to discontinue use.

This is another good reason, why a holiday from the exjade is needed for children.

Lori,

If exjade is not suited to your child then L1 or desferal may be an option for you. Please note that both L1 and Desferal have been used for years (Desferal for atleast 40 years), both meds have enough experience to control the dosage.

Lori,

Her iron level is extremely high. The Ferriscan fact sheet is attached to the post at http://www.thalassemiapatientsandfriends.com/index.php/topic,1677.msg22876.html#msg22876

Has cardiac iron also been measured? Is the test being done for Hereditary Hemochromatosis?

L1 is Ferriprox and has only recently been approved in the US, although it has been used for over 20 years in other countries. Typically, this would be used in combination with desferal in high iron load patients.

Hopefully, you will be seeing Dr Thompson in Chicago. Talk to her about this high LIC and ask if there is a way that desferal can be re-challenged in your daughter and if there is anything that can be done to desensitize her to desferal. Action needs to be taken and also an explanation for the LIC level needs to be found.

Our doctor did mention a five day admission to do sub Q desferal to readjust her body to it. I am guessing we will now more after seeing Dr Thompson next week. Two variants of hereditary hemochromatosis were done. The third was missed and blood was drawn for that yesterday. I asked him to get a waiver for the feeiprox in January and as of yesterday he wasn't even sure it was available in the US. I assured him it was and he said he would start the process.

I do not know if cardiac iron has been measured. Can that be done with a ferriscan?

I got a print out of all four years worth of her tests and will send them tonDr Thompson ahead of our visit next week.

Thanks so much!!!!!!!!!
Lori