Exjade in Children - Recent Observations

Respected Members,

I feel obligated to post some of the observations / effects felt in children using exjade. Although it is not a formal study but we were informed by our doctors at sick kids in Toronto, and regularly practiced. The goal of ferritin around 500 is the baseline in HSC (Hospital for Sick Children). Doctors would stop Exjade at levels 500 and less in children because of the following:

-Exjade would be stopped because children do not/cannot take enough liquids to handle the med and continuation of exjade can cause reduction in kidney function. This is serious side effect.
-Exjade would hamper with the sensory abilities of the children if continued for a longer period of time (specially when there is not enough iron in the body to chelate)
-Reduce Appetite

I understand that some members are proponents of 'Zero' ferritin for everybody, however, children are special with developmental issues. Usually a holiday from exjade is recommended if your ferritin is less than 500. I found that that normally 3 month holiday is prescribed at this point.

Best of luck to everybody

Canadian Family,

Thank you very kindly for this information - My son's ferritin is currently 400 - he is 14 years old so perhaps I should consider holding or at least reducing his exjade dose. 

Sharmin

Hi Sharmin,

Your son is 14 and is big enough to take decent amount of liquids (extremely important). If the appetite is fine and there are no visible signs of sensory problems (that is vision and hearing), I would talk to doctor and decide the dosage.

The post is more intended for children 2 to 12 year old. Also, one thing that I forgot to mention is that the ferriscan score of less than 3 is the key when deciding on the holiday.

My wife and I just returned back from teh Childrens Hospital of Philedelphia and the doctors there recommended a break if it was below 750...so yes breaks are becomming for common.

we have also seen increased ALT/AST for our child when ferritin is below 500. But interestingly Hemo in Children's hospital in Boston believes in reducing the exjade dosage to a lower level than completely stopping the exjade dose when ferritin hovers around 500. as per him, even with low ferritin, the free redicals are still harmful so chelation should never be completely stopped, it should be reduced. That's what we do for our child - when ferritin hovers at 500, we just do 1 tab of 125 mg Exjade/day...when feritin climbs to 700, we up the dose to 3 tabs of 125 mg exjade/day...This way, we have been able to maintain ferritin between 400 and 700 and kidney functions in normal range....

Actually what Dr. Vichinsky recommended is to reduce exjade once ferritin levels are low - but not to completely stop it.  The idea is to have at least a low dose chelator on board to bind free iron.  Ideally all thals would have very low iron load - and would be on a veyr small, maintenance level of chelator.  Dr. Vichinsky said that a thal patient should not go more than 72 hours without chelation.

When iron levels are low, a very small dose of exjade and antioxidant rich foods and supplements helps to bind free iron.  Free iron is what causes havoc and damages organs in the body. 

Now that many patients are becoming successful at reducing their iron levels - it is very important to have these discussions and to maintain a good balance. 

Today more than ever, patients have the opportunity to live very normal lives.  Low iron levels mean, normal appearance, growth and development. 

Sharmin

Also, one thing that I forgot to mention is that the ferriscan score of less than 3 is the key when deciding on the holiday.

My son was on Exjade 500 mg for 10 months and his ferriscan went from 17.4 (severe/critical range) to 4.1 (normal for a transfused patient). 10 months later ferriscan was 2.0. After consulting with Boston Hospital Children's we were told to reduce the Exjade to 375 daily, but that they do not recommend a "holiday." We now are doing the ferriscan next week (six months since last one). If still that low or lower, we will go to 250 mg.
He is transfused every 21 days.
Interestingly, I know of four families whose children took a "holiday" and then were unable to resume Exjade as for some reason they developed side effects when trying to resume it that they had not had previously.
Nicole

Hi nwalsh...my daughter goes to CHOP and they recommend lower the does too and not taking a "holiday".

My son was on Exjade 500 mg for 10 months and his ferriscan went from 17.4 (severe/critical range) to 4.1 (normal for a transfused patient). 10 months later ferriscan was 2.0. After consulting with Boston Hospital Children's we were told to reduce the Exjade to 375 daily, but that they do not recommend a "holiday." We now are doing the ferriscan next week (six months since last one). If still that low or lower, we will go to 250 mg.
He is transfused every 21 days.
Interestingly, I know of four families whose children took a "holiday" and then were unable to resume Exjade as for some reason they developed side effects when trying to resume it that they had not had previously.
Nicole

Wait, are you talking about T2*?

If so, T2's goal should be higher (more than 20) and not lower; more the higher, the better.

-P.

That's the liver iron score from a Ferriscan MRI. It reflects true liver iron concentration. Below 7 is considered safe. Below 3 is the ultimate goal. Some patients with very low ferritin levels, will test as low as 1.

Wait, are you talking about T2*?

If so, T2's goal should be higher (more than 20) and not lower; more the higher, the better.

-P.

Both scores are interpreted in different manner
For Heart higher is better i.e more than 20 and
for  Liver lower is better

Yes, I know that. But I was just confirming with nwalsh if he was talking about liver or heart.

-P.

Yes, I know that. But I was just confirming with nwalsh if he was talking about liver or heart.

-P.

Liver

Nicole

Hello!

I am Thal Major patient originally from Toronto and now in Edmonton. I was currently prescribed 2500mg of Exjade daily. I just can't stomach it. How do you parents get your children to drink it? Any suggestions..I am 31 btw.

Hello!

I am Thal Major patient originally from Toronto and now in Edmonton. I was currently prescribed 2500mg of Exjade daily. I just can't stomach it. How do you parents get your children to drink it? Any suggestions..I am 31 btw.

Hi Sophia,

Welcome to Thalpal!

We split the doses. Almost all thal majors on this board including me have achieved success in seeing constant lowering of Fe with split dose. You take half in the morning and other half in the evening, normally around 16:30 - 17:30 (that's my time but you can adjust as per your schedule but make sure you give it at least 7-8 hours before you proceed to sleep).

Splitting the dose also has good benefit of comparatively lower ALT/AST levels as well as creatinine as the load on kidneys and liver is split in to two times a day.

What's your Fe if you don't mind me asking?

Best,

-P.