when to stop exjade

Hi Andy,
My son started exjade about 5 months ago a few days before he turned two and his ferritin is now 548. I have had two different doctors telling different times to stop it. The one who initially started it had said that they are now stopping once ferritin is below 300 but the one last week said they will stop it once ferritin is below 500 and maybe reduce dose even before that. Is it safe to go to 300 with exjade or is it better to take a gap at 500?
Zahra

Doctors will give various answers to this and we probably don't have enough information yet to know how low is safe for long term ferritin levels when using various chelators. With Exjade, much depends on how often monitoring takes place. If liver and kidney tests are run weekly, a ferritin of 300 should be safe, If tests are less frequent, then 500 may be a better goal. My feeling is lowering the dose to a maintenance level dose may be the best plan, so there are never any chelation gaps. 20 mg/kg should be sufficient when ferritin is below 500.

I agree with Andy. When you reach lower ferritin levels, you just lower the chelation dose.
 In no case, you stop completely because afterwards  you'll  have to increase dose to balance things. Some doctors are wrong about it, and they just prefer to stop chelation completely so that they can feel safe.

Lena

Thank you Andy and Lena,
I hope the doctor will lower the dose tomorrow then . I understand it is a maintenance dose. I dont know when they will transfuse again as one doc said if Hb is below 8.5 they will( the one who said not to stop chelating till 300) and the other said they wont transfuse unless he becomes inactive even if Hb goes below 7( the one who wanted to lower the exjade dose) . Is 20mg/kg still a maintenance dose when not transfusing?
Zahra

Zahra


20mg/kg is a maintance dose, but why should you be on maintance dose if you are not transfusing?
 You take the maintance dose when you the ferritn below 500 and still transfusing
manal

Manal has some right here. I was thinking of that too after I had posted. We are talking about thal intermedia here, not thal major where transfusion keeps going and thal major was what I had in mind when I posted. In thal intermedia the situation may differ. I do not know.
What do you think, Andy?

Lena

I would still say stay on a maintenance dose until the ferritin drops below 300. After that, ferritin should still be monitored as residual tissue iron stores and absorption from food may still pose some problem. Chelation can be resumed again if necessary.

Hi
Today my sons ferritin was 453 which is one week after the last 548 so his exjade dose was reduced to 20mg/kg. The doctor said if it was still below 500 next time maybe they would consider a break. There were other problems though. My son hadnt been eating well so his blood chemistry wasnt good. They gave him electrolytes like potassium and IV fluids before and after transfusions. B/c of these most of the parameters came in normal range except for CO2 which was still low. They sent us home with a warning that if he continues to not eat well he needs to be admitted. The doctor thinks all this happened b/c his Hb was low at 8.2 which he isnt used to . Earlier he had done ok when Hb was between 7 and 8 but he said it will take time for the body to get used to a low Hb again. This time the anemia was causing the loss of appetite and pain and hence the flawed blood chemistry so they decided to transfuse. Next time they hope the body will adjust better. By the way it was the doctor who had earlier said not to transfuse even below Hb 7 if patient on hydroxy urea is doing well who decided to transfuse today. 
Things are complicated . Next appointment in 4 weeks.
Zahra

Hi Zahra,

I just read this. Hope your son is doing well now. At such low ferritin, exjade can affect kidney and liver...Please make sure your doctor is watching both kidney and liver tests very carefully. for liver, the ALt # should remain below 50.

Hi,
Here in the US the doctor has different ideas about when to stop exjade. My sons ferritin is b/w 600 and 700 since we got here but each time the doctor sees him he asks to reduce exjade further. We have gone from 375 to 250 to 125 and now he says to stop citing the greater chances of exjade side effects like hearing loss at low ferritins. Seems here the aim is to keep ferritin b/w1000 and 2000 instead of b/w 500 and 1000 like it was in Abu Dhabi.
Who is right? I am reluctantly going along with this all the while wondering why his ferritin didnt increase much while decreasing exjade even though he is being transfused every 3 weeks?
Could it be that the iron from the blood transfusion is going into his organs and not showing up in ferritin levels???
Lets see where we stand after 3 weeks of no exjade.

Zahra

Hi Zahra,

Where are you in the US? Have you considered consulting with a Center of Excellence while you're here?

Virginia. I am looking into options for a cord blood or marrow transplant. Am pretty confused.

Zahra

You should definitely email Eileen at the Cooley's Anemia Foundation. Explain that you are looking into BMT options but also confused about the doctor's instructions regarding ferritin levels, and ask if she can give you some referrals. I do not think that ferritin level is acceptable at the Centers of Excellence. Eileen will give you some direction.

Patient Services Manager
Eileen Scott
eileen.s@cooleysanemia.org

Hi Andy,

I got a reply from Eileen. It was a little late b/c she was out of office. She doesnt seem very happy about the ferritin advice my doc gave and suggested having their experts talk to him. I am not comfortable with that. I have restarted maintenance chelation b/c I still have exjade left over even though she recommended not going against the doctors advice. I am really worried about him collecting the iron in his organs.

She doesn't know of any transplant centre in the Virginia area. Where else can I find out about this?

Zahra

Hi Zahra,

I agree with Eileen. Your doctor is not up on current treatment methods and needs to update his knowledge. A consultation at one of the US Centers of Excellence would be helpful. If the directions were to come from a thal specialist, your doctor would be much more likely to listen and later the chelation routine. As Canadian family recently mentioned, a ferritin of about 300 while on Exjade should be the goal. I don't particularly like to aim for lower than this with Exjade because we are not certain about long term side effects of Exjade. Even with desferal, problems can appear when the ferritin drops quite low, so under 300 may be a nice goal, but not necessarily with those two chelators. I think 100 is a realistic goal when on deferiprone.

As far as BMT in the eastern US, I prefer Dr Krishnamurti in Pittsburgh and Sloan Kettering in NYC. I know there are many centers offering BMT for thals but these are the ones I am most comfortable with, although the BMT center in Cincinnati is also highly recommended. For a list of BMT centers in the US, see http://www.marrow.org/PATIENT/Plan_for_Tx/Choosing_a_TC/US_NMDP_Transplant_Centers/tc_list_by_state.pl