Question for Andy...

hiya andy,

how are you? hope you are keeping well.
i would like to ask something.  my older son recently had his second t2 scan which revealed that the iron around the heart has gone from severe to moderate and the liver from moderate to totally clear of iron. he was on combi of desferal and ferriprox.  his ferritin is 147.  he has now been told to not have desferal but to increase ferriprox from 9 tablets to 12 to be taken every day.  he weighs 60kg.
what is your opinian?
looking forward to hearing from you.

emby   

Hi Emby,

It's a good improvement but there is still work to be done in the heart and Ferriprox is known for its ability to remove iron from the heart. Desferal is known for having a better ability for removing iron from the liver. Now that the liver iron is gone, the need for desferal is not currently there, as long as another chelator is being used. Even if it turns out that he does have to return to desferal later on, it is good to have a break, as all chelators have side effects and desferal's side effects are more of the long term use variety, so taking a break whenever possible is a good thing. The Ferriprox can work on the current problem and desferal isn't really needed, so I do agree to raising the Ferriprox dose and attacking the heart iron. I am sure he will also appreciate the break. Explain to him that it may or may not be a permanent break from the needle, so that if he does have to resume later on, he won't be so disappointed. As long as he doesn't have any problems using Ferriprox, it is preferred.

I have heard many doctors express a belief that no chelator should be depended on for an entire lifetime because of the long term effects, so switching to another chelator is something most patients should consider at some point during their lives. As more chelators are approved, this does become much easier. Since heart failure is the number one cause of death in thals, a chelator that works better in the heart is important and Ferrirpox does provide this. I am hoping we see similar results with long term Exjade use, but right now we can only be certain about Ferriprox.

I would also recommend that you make sure he is getting plenty of antioxidants in his diet and supplements.

hi andy,

thank you for your reply.
he is not bad with needles as long as i make up the mixture. he is still my baby. 
yes it does make sense to use just the ferriprox just now also because his ferritin readings are within normal range and continuing with desferal could have toxic effects.
thank you again..i just needed reassurance.

emby 

I only want to mention what I have experienced:
I stopped desferal for a year long and took only ferriprox. At the end of the year, heart (already clear) was even clearer and liver (clear too) was clear again but less clear than a year before when desferal was used(together with ferriprox, of course).

You can make your own conclusions...

Lena.

What Lena said is why I say to look at this as a break from desferal but not necessarily a permanent break. Depending on what happens with liver iron levels, desferal could become necessary again at some point in the future.

That's right Andy. I am on desferal again and do not think of stopping it ever again, just lower it maybe.
I think it is necessary for the liver to keep it clear--that or exjade.

A mistake many thals do, is to over-evaluate things when stopping desferal. They think they get a permanent divorce from it and they get dissappointed when returning to it.
No, combination therapy is what makes things run.

Lena.

I would like to stress on the importantce of doing T2* and ferriscan (  if available ) cause ferritin levels are not always a reliable mean of measuring iron overload in organs. We can have a normal level of ferritin as in Emby's son and at the same time iro is being loaded in the organs.

manal

Manal is right,
T2* is necessary and doing it once a year is a must to check out the iron in the organs and reevaluate the chelation therapy.
There are occasions when serum ferritin is high and there is no iron concentration in the organs and the opposite can also happen, though I would say these are mainly exceptions. The rule is high sf leads to high organ iron and the opposite.
That is, at least,what I have seen happening.

Lena.

Lena,

Do you know at what age children get their first T2* in Greece?

Andy,

I think one of our "baby" thals of about 8-9 years old has done her first, but let me check it out and I will let you know for sure.

Andy,

I  just got by my doctor the info on MRI for kids. Here is what she says:

As MRI-T2 has no side effects or radiation, it is considered to be safe for kids as much as for adults.
The only thing that matters is the young one's ability to stay calm inside the mri machine and his/her fear when inside. Even a kid of 4 years old =if he/she is capable of staying motionless= can proceed to the MRI, if he/she can tolerate the whole procedure.

I hope everything is clear now.

Lena.

Hi Lena,

Thanks for the info. Did the doctor say anything about at what age the T2* is first recommended?

Andy,

MRI, T2* same thing. Since the kid is capable of staying still inside the mri mashine, it is recommended.
Age does not matter.

Lena.

Hello Andy,

Just for information, I called Cooley's anemia foundation (few months ago) to arrange for T2* for Little Miss A, I think Elieen Scott (Patient Co-ordinator) is the person I talked to, and she said, in US there is no recommended age for T2*. However, the child needs to be atleast 6 years and able to follow the instructions (that is, holding the breath for certain time and exhaling at certain time). She said, every child is different and not many six year olds can follow proper instructions. So, like it depends when your child is ready.

Thanks

Thanks Lena and CF. I get many emails and one recent one was asking when the child should have the first heart iron evaluation. I think from what everyone has said that probably 6-10 years old is the range. It may vary according to the patient's history of compliance.