Aplastic Anemia and Iron Overload

[kjraimondi]

Hello all,

I have received about 12 units of blood since I was diagnosed with AA in September. I am still in transfusion dependent and am waiting to see if the first round of ATG (which I received in September) will help my RBC rise. So far it has helped my WBC but I am still below normal. Last results were WBC 2.1, Neutrophils 1100, RBC 10.4 (transfused Friday) and Platelets 34k (transfused Thursday).

Had my iron levels checked and I am at 1200. My doctor says medicine is really an option is unsure of what we can do to bring the levels down. I see a lot of postings about wheat grass and appreciate any links anyone might be able to provide about ways to limit my Iron and bring my levels down.

Thanks so much in advance!

Ken

[Greg H]

Hey Ken!

There have been a number of good conversations on marrowforums about wheatgrass as a chelator.

Here are a couple:
http://forums.marrowforums.org/showthread.php?t=2280
http://forums.marrowforums.org/showthread.php?t=1809
And this discussion of Exjade gets into wheatgrass and the potential for black tea, calcium, and dairy to slow absorption of iron from food. (Though watch out for the blood thinning effects of tea, given your low platelets.)

Unless you eat beef liver topped with spinach every day, I gather that it would be hard to get enough iron from your diet to even approach what you are getting from your transfusions. Still, lots of folks around here avoid red meat, fortified cereals, and other sources of unneeded iron. And that's probably a good practice.

I've been doing a good bit of reading about iron overload and chelation, because my docs at NIH have recommended I begin using one of the chelating drugs in a couple of months. My ferritin is at just under 2800. We haven't addressed this previously because of the hope that I would respond to Campath, start making my own red blood cells, and gradually use up all that excess iron.

Such has not been the case, so chelation is on the table. The docs want me to wait two months before starting Exjade or Desferal because I've just started Danazol, which has potential liver impacts, and they don't want to add a chelator, with potential liver impacts, on top of that.

I took Wheatgrass early this year for some months, and believe it held down my increase in ferritin. But I stopped when my transfusion need suddenly increased and I decided to eliminate the wheatgrass, just in case it had some effect. It's pretty clear now that wasn't the case, so I started taking freeze-dried wheatgrass juice capsules a couple of days ago. I also have wheatgrass powder that I will mix daily into smoothies.

I have also tried compressed wheatgrass tablets. These are really big pills, but I learned to like the taste and just chewed them up.

What I have not tried is growing my own wheatgrass and juicing it (or buying the fairly expensive frozen juice), even though the relevant studies on wheatgrass as an iron chelator all seem to rely on fresh juice from very young plants. I'm not in a position to grown my own, and there's plenty of anecdotal evidence on marrowforums from folks who've used one or another of the powders or pills with success.

I am no expert on this, but I think there was a pretty big hysteria about iron overload in the bone marrow failure community about the time that Exjade -- the only oral chelating drug approved in the US -- was approved by the FDA. In fact, if you go read some medical journal articles from 2007 and 2008, you'll find docs writing that their patients are more concerned about iron overload than about their bone marrow failure.

Though Novartis (owner of the chelating drug patents) is spending lots of cash working on establishing the value of chelation, the hard data out there -- aside from thalassemia patients -- on iron overload and chelation is a mile wide and an inch deep. In the absence of data, however, there are lots of very firmly held opinions.

There's a helpful 2008 presentation by Dr. Gail Roboz on the AA&MDS website. I highly recommend viewing that presentation, which is long on common sense.

Her bottom line is that, if you're talking about a younger patient, who is looking at potentially years of transfusions, and there's no guarantee that the current therapy is going to result in transfusion independence, using a chelating drug probably make sense.

That's kind of where I'm at, and why my NIH docs want to start chelating. Since they've given me two months before that cranks up, I plan to pour on the wheatgrass to see if I can move the bubble with something that has fewer side effects than Exjade or Desferal.

I hope that's useful.

Take care!

Greg

[johine]

I take exjade daily for iron overload, There are strong warnings about organ damage when on exjade. you should discuss this with your doctor

[Birgitta-A]

Hi Greg,
You are still young and will perhaps later have a SCT. As you know iron overload can increases the risk of infection.
http://ash.confex.com/ash/2011/webpr...aper41888.html

Ferriprox – an oral iron chelator – is now approved by FDA:
http://www.fda.gov/NewsEvents/Newsro.../ucm275814.htm

I was transfusion dependent from dx 2006 until 2010 and received 142 units of PRBCs. I started with Desferal from a home pump in connection with txs after 40 units of PRBCs. Three years after dx I tried Ferriprox too but my WBCs decreased so I changed to Exjade. Exjade decreased my WBCs and I could only take 500 mg/day instead of 1250 – the normal dose for me.

I had no adverse effects from Desferal and no adverse effects from the other drugs except the low WBCs.

Now I don’t take any iron chelator and my ferritin level is decreasing very slowly – latest value 979. I have hemolysis - I think that’s why the ferritin level still is so high.

Hope the wheatgrass will have a positive effect!
Kind regards
Birgitta-A

[Greg H]

Hi Birgitta!

I think it's just that infection risk in SCT that has tipped my NIH docs (and me) toward chelation. It's good to hear that you had no adverse effects. It seems like so many folks have nausea and other GI upsets with either Exjade or Desferal.

Dr. Dumitriu at NIH seems to feel Desferal is actually preferable to Exjade, because of the longer, more gradual infusion of the drug. But I'm not sure that I'd be good at actually complying with the process on a daily basis.

Ferriprox seems to have been approved in the US for thalassemia patients but not MDSers -- though I suppose a friendly doctor might prescribe it anyway, since it's available in the States.

I'll be talking with my local hematologist on Thursday about the options.

Take care!

Greg

[Chirley]

Just a question about ferritin.

Is Apo ferritin as damaging to organs as ferritin? I wanted to ask my doctor about it but he has already left to attend the ASH conference.

Regards

[Birgitta-A]

Hi Greg and Chirley,
Greg, you know high ferritin increases the risk for infections in everybody not only in connection with SCT. I only received Desferal for four days with txs but after three years of txs that was not enough and then I started with oral drugs too.

Ferriprox is more dangerous for MDS patients due to the higher risk of agranulocytosis (no WBCs). I think Exjade is safer.

Chirley,
As far as I understand apoferritin is protein that binds iron to form ferritin. I don't think apoferritin is toxic. It is too much iron that is toxic.
Kind regards
Birgitta-A

[Marlene]

The way I understand it the ferritin is a protein which binds to iron so that it remains stable/safe. But once that system is overloaded, iron then gets stored in organ tissue. The free iron and stored iron is problematic.

Bacteria thrives in an iron rich environment. When John was on desferral he would end up some pretty huge bruises that would cover his entire belly. When this happened, we would stop the desferral until it cleared up to reduce the risk of infection. I think the chelators make the iron more available so it can bind to it and excrete it. Therefore, we did not want extra free iron floating about. We basically stop chelators when he got a cold/flu or any accident/trauma and then resumed when things cleared up. We may have been overly cautious though.

[Greg H]

Hey Marlene!

Thanks for that info. Those belly bruises sound rough. Was this a side effect of the desferal or a low platelet issue?

Thanks!

Greg

[Marlene]

It was a result of the sub-Q needles. We never knew if we hit capillary or blood vessel until we pulled the needle. He would have to stick himself every night to infuse the desferral over 8 hours. This is why compliance with desferal is not that great. John liked it much better when he could use his central line but once that got pullled, he had to go subQ.

I'm sure low platelets played a role also.

[Greg H]

Marlene,

Ouch. Thanks for that detail, which was not something I had considered. That's another point on the "con" side for desferal.

My thought, if I went that route, was that I'd actually do the chelation during the day instead of at night. I'm not sure I could sleep hooked up to a pump and Sub-Q rig. But I work from home mostly, so carrying it around during the day might not be so bad.

But the idea of puncturing blood vessels from time to time sounds like no fun.

Take Care!

Greg

[Marlene]

Two lessons learned from subQ des.

1) When the home health nurse come out to instruct. Make sure you shave the area before hand. It was He** getting the bandage off.

2) Not all needles are equal. I had to go outside of our provider to get sharp needles. We found in inconsistent quality with the needles. Some were not sharp and John had to push pretty hard to insert the needle. So I went out and bought our own.

And maybe there's a third.....Each time they delivered the meds and supplies, they gave us supplies we wouldn't use. And once delivered, they take nothing back. I would try to instruct them before each delivery what I needed and they would alway mess it up. And we wonder why healthcare is so expensive. When I spoke to customer service about it she said "Well at least you're not paying for it". I shook my head and knew then it was pointless to pursue. So I ended up donating the red needle disposal bucket ( got one every two weeks) and other stuff to charity.

[Birgitta-A]

Hi Greg,
I have had a port-a-cath without any problems (knock on wood) since 2007 and my home pump with Desferal was connected to the port. The hope pump looks like a lemon initially but gets smaller during the 4 days transfusion. I carried it in a small bag round my waist - that could probably be a problem for a young person but not for me.
Kind regards
Birgitta-A

[Greg H]

Marlene,

Thanks for those tips. I know what you mean about waste and our health care system. One of the factors in the "pro" column for desferal is that I understand it's much cheaper than Exjade. Of course, I won't be paying for it, either. But we're never going to get health care expense growth under control if everyone doesn't pitch in a bit. I found a neat Thalassemia site on the web that discusses various pumps and needles.

Birgitta,

It does seem like Desferal would be much easier with a port. I have seen the lemon pump, which I understand works with no batteries.

Thanks to you both,

Greg

[Birgitta-A]

Hi Greg,
Yes, inside the pump there is high pressure and the infusion is given for example 2 ml/hour - I don't remember but the figures are written on a paper glued to the pump. Actually a pump is quite expensive and in Sweden it is only used once. Here Desferal - that has been used about 40 years - is the first drug for iron chelation.
Kind regards
Birgitta-A

[kjraimondi]

Originally Posted by Greg H

Hey Ken!

There have been a number of good conversations on marrowforums about wheatgrass as a chelator.

Here are a couple:
http://forums.marrowforums.org/showthread.php?t=2280
http://forums.marrowforums.org/showthread.php?t=1809
And this discussion of Exjade gets into wheatgrass and the potential for black tea, calcium, and dairy to slow absorption of iron from food. (Though watch out for the blood thinning effects of tea, given your low platelets.)

Unless you eat beef liver topped with spinach every day, I gather that it would be hard to get enough iron from your diet to even approach what you are getting from your transfusions. Still, lots of folks around here avoid red meat, fortified cereals, and other sources of unneeded iron. And that's probably a good practice.

I've been doing a good bit of reading about iron overload and chelation, because my docs at NIH have recommended I begin using one of the chelating drugs in a couple of months. My ferritin is at just under 2800. We haven't addressed this previously because of the hope that I would respond to Campath, start making my own red blood cells, and gradually use up all that excess iron.

Such has not been the case, so chelation is on the table. The docs want me to wait two months before starting Exjade or Desferal because I've just started Danazol, which has potential liver impacts, and they don't want to add a chelator, with potential liver impacts, on top of that.

I took Wheatgrass early this year for some months, and believe it held down my increase in ferritin. But I stopped when my transfusion need suddenly increased and I decided to eliminate the wheatgrass, just in case it had some effect. It's pretty clear now that wasn't the case, so I started taking freeze-dried wheatgrass juice capsules a couple of days ago. I also have wheatgrass powder that I will mix daily into smoothies.

I have also tried compressed wheatgrass tablets. These are really big pills, but I learned to like the taste and just chewed them up.

What I have not tried is growing my own wheatgrass and juicing it (or buying the fairly expensive frozen juice), even though the relevant studies on wheatgrass as an iron chelator all seem to rely on fresh juice from very young plants. I'm not in a position to grown my own, and there's plenty of anecdotal evidence on marrowforums from folks who've used one or another of the powders or pills with success.

I am no expert on this, but I think there was a pretty big hysteria about iron overload in the bone marrow failure community about the time that Exjade -- the only oral chelating drug approved in the US -- was approved by the FDA. In fact, if you go read some medical journal articles from 2007 and 2008, you'll find docs writing that their patients are more concerned about iron overload than about their bone marrow failure.

Though Novartis (owner of the chelating drug patents) is spending lots of cash working on establishing the value of chelation, the hard data out there -- aside from thalassemia patients -- on iron overload and chelation is a mile wide and an inch deep. In the absence of data, however, there are lots of very firmly held opinions.

There's a helpful 2008 presentation by Dr. Gail Roboz on the AA&MDS website. I highly recommend viewing that presentation, which is long on common sense.

Her bottom line is that, if you're talking about a younger patient, who is looking at potentially years of transfusions, and there's no guarantee that the current therapy is going to result in transfusion independence, using a chelating drug probably make sense.

That's kind of where I'm at, and why my NIH docs want to start chelating. Since they've given me two months before that cranks up, I plan to pour on the wheatgrass to see if I can move the bubble with something that has fewer side effects than Exjade or Desferal.

I hope that's useful.

Take care!

Greg

Wow! Thanks for the insight!

[cathybee1]

This is all really great information...

[usaf1125]

I'm 79 have had 277 units of blood since 2005, I take 500ML of ExJade daily. My ferritn level took approx. 4 years to drop below 3000, it's gone as low as 2385 and back up to 2850 it floats up and down, but is staying below 3000, I'm happy, I take ExJade daily for the iron overload.

[BerryP]

Hi all, I am from England and diagnosed SAA Sept 2011, rabbit ATG failed and taking 250 mg ciclosporin and having prb transfusions every 4-6 weeks. Iron up to 2500 so I have started on a sub q balloon pump over 96 hours, (4 days on, 3 days off). This is my third lot and I am fine while I am on the pump, but when I take it out I am ill. I feel and have been v. sick, dizzy, feel like somebody is kicking me all over, my husband says my face is grey and I go to bed for 36 hours. Given this is half my time off the pump I am wondering if this will stop or whether I should look for an alternative. Speaking to the docs they say only given the pump to two other people who lasted one day and gave up!

Any advice would be most welcome. Docs dont want to give exjade as it kicks counts and pancytopenia won't take it. I am 58 and am starting to think bone marrow transplants look good. Husband worries cos he says you only talk to the survivors. Many thanks in advance.

[usaf1125]

I have been taking EXJADE once a day for over 8 years. My iron level has been up to 3000+. At my last infusion it was down to 2385 ng/ml, that was on 1/24/2012. I have had so far 282 units of blood. I don't know what "Pancylopenia" is, but I would push for the EXJADE. At your age a bone marrow operation is a must. I was to old when I was diagnosed for it. Being tired is a big part of it, I nap a lot. The only advice I can give is don't let it drive your life. I know my limits and live with them. I hope it all works out good for you. Best of luck, from the colonies.

[Polly S]

BerryP,

Hi I am also from the UK, I think we have spoken before on here. Have you been on the desferal for long? I have also started recently taking it, I have 4, 12 hr infusions a week via a balloon pump. I am interested to hear that you are so fatigued. I am completely knackered! I keep thinking that my hb has dropped but it is stable at 10.5. I didn't think this was a side effect of the desferal but I don't know what else I can put it down to. Have you spoken to your doc about this? I am under Proff Marsh at kings but am not seeing her for another couple of months.

Polly x

[Karenish]

Polly hi its Karen, my iron levels are now high - hb holding about 8.5 plates about 25 and I am on exjade, i take it in the evening with fruit juice as soon as i come in from work and then an hour later eat with lots of fluids.....no problems. Not sure what that panco thing is you mentioned, but exjade appears fine so far!
xxxx

[Polly S]

Thanks Karen, I think it was BerryP who mentioned the pancytopenia . I was on Exjade for a few weeks but it made me very sick and sent my renal function way off. Guess I am stuck with sticking myself with pins but if it does the job then I shouldn't complain. Glad Exjade is working out for you.

xx

[BerryP]

Hi all, sorry to muddy waters with pancytopenia, just means v. low on all blood counts. Still sticking myself with needles and still having pump 5 days on and two days off for excess iron. Spoke to docs about feeling and being so sick so they have halved dose to 2g. There are bruises/sore patches involved in keeping the needle in so long but sleeping with the pump is not a problem. Hoodies are good because you can stick the pump in the pocket.For me ciclosporin 250mg per day does not keep my blood up and therefore transfusions are necessary, it also means I feel more normal because hgb drops quickly below 8 and then I lose the plot big time Had not heard anyone else complain about gi effects and headaches associated with desferrioxamine, so thought I must be having allergic reaction. It does make my tongue swell, so when I am chewing on my tongue at night I know its time to take the pump out Hospital v good and let me run my own regime, i.e. I have sharps and kit at home and put sub q in on my own now which helps with the old empowerment. I am settled into doing the pump thing now and apart from minor infections I am glad I persevered. Best wishes to all, still vertical and still got the smile. Berry.

[Marlene]

John had Gi troubles from the desferral also. He would take one week off and then start up again. He never did a full dose and would infuse sub-Q 5 nights a week with two days off until his nausea got to be too much to handle. Then he would take a week off.

He found that warm, moist heat would help the bruises resolve quickly. He would get some really big ones. If they were too big, he would stay off the desferral until it resolved. Didn't want to risk infection.