Iron Overload strategy
 

My doctor is starting me on Desferal soon, as my numbers are high. I would greatly appreciate anyone's thoughts on the situation:

I received my AA treatment in Dec 2006. I did not keep records of all the PRBC transfusions, but I put it down on paper and I think that I got around 31 units, most of them in the hospital, from Aug 2006 thru Mar 2007, when I became transfusion independent for a few months. Since I restarted transfusions in Sep 2007, I have had 11 units, basically 1 a month.

At my NIH eval in Aug2008:
ferritin: 1892 mcg/L
transferrin: 194 mcg/dl
iron: 251 mcg/dl (my doc was unsure about this one in the NIH letter)
alanine aminotransferase: 56 U/L

I am about to undergo a second AA treatment, possibly the Campath one at NIH, and I wonder if I should bypass the Desferal for now in hopes of a recovery and then using phlebotomy instead. What's the potential harm of a few more months at this stage?

Hi Bill,

I would say waiting 3 months is not a big deal but I would not wait much longer than that because as long as you are transfusion dependent, your iron will increase. Even after treatment, you may not get to an acceptable HGB level to do phlebotomies for quite a while. So there's a high probability you'll still have to do desferral anyway and the sooner you start, the better off you'll be in the long run. They say that iron overload does not really impact your health until you've had it for a long time but I think, in John's case, it did. It creates a lot of oxidative damage in your body.

It has taken John a really long time to get rid of the iron. He started with desferral, then phlebotomies, then Exjade. He stopped the desferral and went to phlebotomies when his HGB was around 11.5 (with the help of Procrit.) He did this because of side effects from the Desferral. Then the phlebotomies got to be too much in that he could not make up the blood volume. So then when Exjade was approved for use, he started that. But then the Exjade was too hard on his kidneys and he stopped it. We have come full circle in that he's back to phlebotomies to get the remaining iron off. His HGB this week was 12.4 and we had 250 ML taken off (they usually do 450-500ml/month). He'll do this every other month until it gets into range.

John could never tolerate full doses of either drug so the iron came off at a slower pace. The phlebotomies do work best for him. John's FE was 4000 in less than one year from diagnosis. We held off doing desferral because everyone thought he would be recovered enough in the next few month and he could address it then. Well that didn't happen. In hindsight, we should have started earlier but no one expected his FE to be so high so quickly and that it would take him so long to recover.

Hope this helps.

Marlene

Iron Overload
 

Marlene,

Thanks for replying with so much information.:)

It's true that I am being pretty optimistic about the 2nd tx. It's worth trying the Desferal; after all, if I tolerate it, it will help. The doc says I can sleep with it and not wear it during the day.

My doc was saying something about also getting Desferal during transfusions to help right at the time of transfusion? Have you ever heard of that? I think he meant injections.

Bill

Desferal
 

Hi Bill,
At dx May 2006 my HGB was 70 so I was transfusion dependent "from start". Jan 2007 I got a port-a-cath for Desferal (and transfusions). I get Desferal through a homepump continuously for 4 days starting at the same time as the transfusion without any adverse effects :). In Sweden all patients get Desferal in this mode because it is considered safe and effective.

Desferal – dosage and administration:
http://www.thals.org/en/download-res...kage/view.html
Kind regards
Birgitta-A

Many patients get desferral with the transfusions. John did not because he had a central line in and I would hook him up every night. We would do it 5 nights a week. After about three weeks, he would have to take a break due to side effects and then start up again. Home health care came out and trained me on how to do it and then they would send the meds and supplies weekly.

Once his line was pulled, we had to switch to Sub-Q infusions. This is where a small needle is placed in the fatty area of your belly or thigh and left in for the duration of the infusion. This was a pain. Again home health care came out and did a 15 minute training and we were on our own. We found the needles to be dull and had to search for a better brand ourselves. Sometimes I think these services go for the cheapest they can find. With Sub-Q, you also have a pump to infuse over 8 hour at night. On our own, we discovered you can get a numbing cream so the needle stick is not so bad. We also have used ice to numb the area. Why they don't tell you these things is a mystery to me. From time to time, John would get a huge bruise on his belly from the stick and not having platelets made it worse. When that happened, he would take a break from the desferral until it cleared.

Desferral works best when infused at a slow rate over a long period of time. Your urine will most likely turn an orange color since the chelated iron is excreted via the kidneys. John took vitamin e (400 IUs) and after one month of being on Desferral, vitamin C (250 mg then up to 500 mg).

When he was on Exjade, we found his Vitamin C and zinc levels to be really low so we upped the C and added in zinc. We think the Exjade may have chelated zinc and copper also. Just something to keep in mind as you do chelation is to make sure you're getting enough trace minerals and antioxidants. Living in Hawaii should help alot...lots of minerals in your soil and easy access to fresh fruits all year round.

The Exjade is the easiest to do because it's oral, but also comes with more risks.

Wishing you the best....

Iron overload strategy
 

Marlene,
Thanks again for all the information you shared about iron overload. What method was used to check for vitamin c and zinc levels? Was it done at the doctor's office?

Hi Margie,

The easy answer is yes....at a doc's office. However, he was not your "typical" doc. Many of the test are standard test but there are others out there used by docs who specialize in nutrition, metabolic diseases, autism, anti-aging, orthomolecular or funtional medicine.

Your GP can check many of your nutritional levels. I think its a good thing to find out and no one should object to it. They are either blood serum or urine test. Keep in mind most are screening tools for possible deficiencies and you need to assess the results with symptoms/life style/ eating habits in mind. Example....Someone who's a vegitarian, over 50 and is on acid reflux med is having some neurological symptoms get her B12 levels checked. The test comes back in the low normal range. However, this does not rule out a defeciency and needs to be explored further because this person is not getting adequate B12 in her diet and the absorbtion of nutients from gut is diminished due to age and her medication.

We started out looking for doc where we could do some one-stop-shopping. We wanted to get information on John's nutritional and endocrine status. We already knew he had some issues going so we were looking for confirmation and fresh eyes to look at his overall health. We got some good information from a urine test call an Organic Acid Profile from The Great Plains Laboratory http://www.greatplainslaboratory.com...g/full_oat.asp. This test showed a dietary and/or an increased need for antioxidants, specifically vit C. Also the need for B5 and a need to increase glutathione levels. Increasing glutathione can be done by adding in Vit E, C, NAC, Lipoic acid (all antioxidants).

He also ran an analysis on is Red blood cell which looked at mineral status and toxic elements. Interesting thing is that this test and serum copper/zinc profile were contradicting each other.

Basically, this gave us some solid data from which we could start a more targeted program. We did not follow through with everything this doc wanted to do for many reasons but mostly because our agenda and his were not aligned.

Hope this helps.

Marlene

Now I get it
 

Aloha,

Ok, thanks, my hemo DID say what I thought he said! And thanks for the link. I've learned that the more I know, the better.

And talk about being proactive! Just start giving Desferal from the very beginning... good move.

Bill

Thank You!
 

Wow, thank you for all the information!

Based on Birgitta-A's post, it seems like it would be a good idea to start the desferal right from the beginning in some or many cases of AA. (Is it the cost of the medicine and insurance companies that causes this???)

I feel that I am getting a late start on it, and it's only because Dr Paquette at UCLA made a point of it in his evaluation that I was even made aware of it.

btw, my hemo checked out Exjade and said that one of its side effects is decreased blood counts, so that was out for me.

I gave myself Neupogen shots in the belly for a couple of months after my ATG treatment, and had no problems with it, luckily. Poking myself the very first time was the hardest part!

Thanks again for the info! This board is great!

With both Exjade and Desferral, you need to have an excess of iron in your system. Chelating with normal or just slightly elevated levels is risky in it increases the probability you'll experience the hearing/visual side effects mentioned in the drug leaflet. Both drugs do not recommend starting therapy until FE is at 1000. And with Exjade, they recommend stopping it when your FE is at 500 for three consecutive readings. You can also adjust the dose down when your FE is lower to avoid side effects. Before starting, you should get a baseline hearing and vision check and then follow up yearly to make sure there's no damage.

Marlene

Desferal
 

Hi Bill,
You know I didn´t get Desferal from start - it was packed red blood cells May 2006. Then I got a port-a-cath Jan 2007 and Desferal Aug 2007. My liver functions well - my liver tests have never been elevated :) - and that made my dr wait with Desferal. I am afraid of adverse reactions due to too much Desferal - as Marlene has written chelating therapy when the ferritin level is too low can result in cataract and hearing loss :(.
Kind regards
Birgitta-A

My dad is 73 and has RAEB. He gets transfusions every other week and has high iron. He is getting supportive care. The dr. says he will die before iron overload. It seems we should be doing more. They tried Vidaza/procrit for 3 months and dr. days it wasn't working. He has no pain just short of breath with stairs and exertion. Please give me some advice. His blood counts go from 9 down to 6.5 and his platelets stay at 16 white 1.5.

Iron overload
 

Hi Dee.
Supportive therapy in MDS patients means:
Red blood cell transfusions
Neupogen or similar drugs for low white blood cells if the patient has infections
Platelet transfusions if the patient has bleedings
Iron chelating therapy if the patient has a high ferritin value, increased liver tests, hopefully will survive 6 months (different in different countries)

It is very difficult to know how long a patient will survive - we know that MDS patients live much longer today than some years ago due to better treatment. You have to know if your dad has blast cells in the blood and if so what %, his serum ferritin value and his liver test results.

Hope your dad will get effective supportive treatment :)!
Kind regards
Birgitta-A
69 yo, dx MDS Interm-1 May 2006, transfusion dependent (now 71 units of packed red blood cells), Desferal 4 days with transfusions for iron overload, Neupogen 2 injections/week for low white blood cells

I thought I'd chime in here about our chelation experience.

Our son Grant was on Exjade for a couple of years. Since he was continuously transfusing, we honestly didn't see a decrease in his ferritin levels. He wasn't super compliant either because it tasted so nasty so that has to be considered.

Now he is on Day +22 of a MUD BMT. As soon as he got the marrow, his doctor stopped the Exjade because it is known to affect wbcs. Unfortunately he appears to now be rejecting his transplant but in the meantime, has started Desferal infusions over 18 hours each day. The only side effect being "vin rose urine".

Wendy mom to Grant
dx 12/4/98 AA
http://www.caringbridge.org/visit/grantbeltrami

Thanks for all the replies
 

Thanks to everyone for sharing their information. I get it now that the Desferal starts later on, but that some can be given at transfusion time.

The doc had good news for me this past week: my ferritin was about 1400 (I don't remember the exact number), down from 1892 at NIH in August, and with no Desferal yet. I'd gotten 2 units of (amazingly fresh) PRBC 9 days earlier, and I think the ferritin was elevated due to that. My hemo also showed me my ferritin 3 months after ATG (Mar 2007): 2400!

My whole outlook on transfusions has changed. It used to be I couldn't wait for the next one, for the energy boost it provided. This was during a "wait and see" time in my care. Now that I'm more familiar with iron overload, the longer I hope I can last till the next one, to keep my ferritin low.

Now I'm wondering if I should wait and see where the ferritin is in a month or two, to see if it continues to decline on its own. All assuming that I don't get more red blood for a while. I'm thinking 1400 isn't all that bad.

An update
 

Just to update:

Cheers to Dr Paquette at UCLA. His regimen of CsA alone seems to have worked! My counts stopped declining about 3 months after I started the new regimen, and now they are SLOWLY increasing!

The best news of all: my ferritin has continued to decline (last PRBC tx was July 2008). The doc says my body is using it to make red cells, and that's a lot better than using Desferal! I am so glad I waited! Last month it was a little over 1000. Dr Paquette says no need for chelation!

One downer: CsA is linked to higher incidence of cancer, and I just had to have a squamous cell cancer lesion removed from an ear. They said they got it all.

So a reminder to everyone on CsA to be on the lookout for this kind of thing! I was lucky that my ENT doctor, who was following up on some hearing loss I suffered, saw the lesion and said it needed to be removed.

BTW, my hemotologist thinks that it could have been the CsA at the root of the hearing loss.

desferal sub-Q vs port
 

I'm new to the forum and have already learned so much. I have some questions about receiving desferal though a port. I started taking E-jade. I stopped because it didn't agree with my stomach. My fe counts were 10,000 when I started and they didn't move much. I then switched to desferal by sub-q pump for 5 days a week. My counts dropped to 5600 after several months. I had a bout of illness and stopped using the desferal and now I'm finding it very hard to start again. I got out of the habit of sticking needles in my stomach and would prefer not to if there is another way.
I was very surprised to hear that some of you have used your port. This option was never mentioned to me as a home treatment. Did you find it difficult to leave the port accessed for use. Were you worried about infection? I would love to hear more about the pros and cons of using the port.

John had a hickman in his chest for a while and we used it for the desferral. It was very easy to use and he preferred it that way. They taught us how to do care the of hickman and administer the desferral.

He did have to have the line pulled and he chose not to get another one. That's when we switched to the sub-Q pump. That was a challenge on many levels.

I guess it may depend on the type of line you have. John's was easy to get to since the double lumens were outside of his chest. A port-o-cath (sp) may be more difficult since that's installed under the skin.

Desferal
 

Hi Pat,
I got my port-a-cath Jan 2007 and the first months it was only used for transfusions. Aug 2007 after 40 units of packed RBCs I started with Desferal. You can find a picture of the small home pump at page 4 in this article:
http://www.cooleysanemia.org/updates/pdf/July2003.pdf

Now I have received 41 Desferal infusions in my port-a-cath during 4 days in connection with transfusions.

I bled a lot when i got the port-a-cath because I have low and dysfunctional platelets (I did get cyclocaprone during 5 days and a platelet transfusion without much effect). Otherwise there hasn't been any problems like infections though I have had very low neutrophils twice. I don't feel the Desferal infusion at all.

Hope you will be able to get Desferal infusions via a port :).
Kind regards
Birgitta-A
71 yo, dx MDS Interm-1 May 2006, transfusion dependent from dx, Desferal and Exjade for iron overload and 2 Neupogen injections/week for low WBCs.

Iron overload strategy
 

Infusing desferal subcutaneously can be a nuisance but the alternative is not worth thinking about. If you can avoid liver and heart damage why not try. David has been infusing since Dec 2005. He has had some breaks due to abscesses at the site but we seem to have a good system now. We keep antibiotics on hand because he can become delerious in a few hours. This has avoided hospital stays and surgical draining.

I help by swabbing the site and using iodine as well. Everything is covered with Op-site surgical tape which means he can shower. He now only leaves each infusuin in for three days and copes with the pump in bed . He has a break for a day and starts again. If he infuses too often his kidneys are affected. With this regime he has been able to maintain the ferritin around 2500 after 135 transfusions mostly of three units.

Some nurses want him to get a port-o-cath but his haemotologist says that is a last resort because of his infection history. His veins are becoming a problem.

I know it is easy for me to give this advice but it has kept my wonderful husband relatively healthy.

Good luck to everyone

Helen