Watch and wait ends, Vidaza begins.

[Chirley]

Thanks for that Birgitta, I thought of haemodilution too but my platelets were higher than they have ever been and the doctor determined that I was actually dehydrated and needed the extra litre of IV Saline. in reality my Hb would have been lower after the fluid load.

Oddly, my neutrophils were higher than they have EVER been and so were my lymphocytes. My monocytes were just on the the high side of normal. Liver enzymes had decreased for the first time but protein and globulins were well below normal.

I was supposed to have two weeks off treatment but when I looked at my appointment book I noticed that I've been booked in for another week of daily copper in a weeks time. I hope the secretary has made a mistake. I'll phone them next week just to check.

I made the mistake of going shopping yesterday. Christmas crowds already, absolutely hideous. I was almost knocked over by a run away child once and twice by pushy young women pushing prams. I think I'm going to have to give in and start using a wheelchair for shopping trips, it's becoming a every man for himself world out there.

Regards

[Birgitta-A]

Hi Chirley,
You know I have heard several dietists on video tell us that patients with leukemia and similar diseases should try to drink 8 glasses (2 l) of extra fluid per day in order to help the bone marrow. I have read the same info at some of the well known clinics home sites too so since dx I drink about 4 l of fluid each day - I don't know if it has any positive effect. In any case my bone marrow that was very fibrotic at dx har maneged surprisingly well during almost 6 years now. There is no risk for dehydration and the liver/kidneys perhaps can get rid of more toxic products when they have more fluid to work with.

You have some positive results with high platelets and neutrophils as well as lower liver tests . Hope you don't need copper next week! Take care and do avoid Christmas crowds - many of these people can be infected and give their disease to you even if they don't knock you over.
Kind regards
Birgitta-A

[Chirley]

I've just come back from seeing the Prof.

Basically he said I DO have a copper transport problem which causes an inability to absorb copper from the gut while at the same time prevents the kidney from stopping the excretion of copper.

Side effects are aneurisms, joint problems, collagen disorders, heart attack, bone marrow failure, bone marrow cancers, liver failure, stroke, spinal cord degeneration and brain degeneration.

Supplementing the copper either with S/C injection or IV infusion while increasing blood levels may not necessarily increase tissue stores and may therefore not be of benefit in preventing all the side effects.

There are 4 different genes that are known (and more unknown) to be
involved in copper transport (besides the Wilsons, and Menkes genes) and they are hoping to develop a specific test for these gene mutations. There is a drug trial being undertaken for one of the gene mutations that cause copper/iron imbalances but as he's not sure if that is my problem I'm not eligible at this stage.

I asked about the dramatic drop in my Hb a couple of weeks after venesection and whether that had anything to do with marginal bone marrow reserves. He told me that I have iron deficiency??? I told him my ferritin was very high and he said it was a non iron ferritin called apo ferritin and the only way to measure real ferritin and tissue copper was to have a liver biopsy. Yuk! I have a nasty feeling he is going to ask my haem doctor to organize that.

The Prof seems to hate portocaths and insists I should have mine removed and have subcutaneous copper instead, I simply said no, I wasn't doing that.

I asked why I would be stable neurologically for months only to start deteriorating in the last few weeks. He said that all kinds of external things can affect function for example tiredness, heat, concurrent infection etc. and
that hopefully the deterioration is going to be temporary. I need to avoid physical stressors.

Before I left the clinic I had more DNA studies with karyotyping along with iron studies and copper levels.

All in all the visit was very informative if not wholly optimistic.

Regards

[Birgitta-A]

Hi Chirley,
You really have a very complicated disease. Good that you at last met a doctor who has studied this kind of symptoms. Now you have to avoid a lot of "external things" and hopefully the symptoms will decrease.
Kind regards
Birgitta-A

[cathybee1]

Well, Chirley...I am glad you have access to specialists who can help explain what might be going on. I'm so glad you took such good notes. And yes, it was very informative.

The rationale given to you for doing a liver biopsy aligns with what family doctor/hematologist told us. Of course, if there was excess copper in the liver, that would suggest Wilson's.

On our side, we are still awaiting results from our visit to the MDS Specialist 3 weeks ago. Bruce called and talked to a supervisor there. The doctor's report is waiting signature.

Meanwhile as of bloodwork last Friday, Bruce's hgb was 9.7 and copper is now down in the low 50s.

Bruce's family doctor believes that ceruloplasmin numbers would improve if Bruce's liver was working better, suspects a wheat or gluten sensitivity, and wants Bruce to get off wheat/gluten for 6 months. I did read one article about the relationship between celiac disease and anemia/MDS mimicry.

If you have any more details on the trial for copper/iron, I would love to have it.

Hugs to you.

[Chirley]

Hi Catherine, I have to see the Prof again in 8 weeks and I'll try and ask him more about that drug trial then but I wasn't really interested because I have to agree to haveblood taken for genetic testing and research and I would have to pay $1200 per test to cover the cost of the research labs. Only after I had these tests and had the appropriate results would I be eligible for the trial. I'm not interested in spending more money on iffy theories that may and more likely, may not benefit me or any one else.

I'm more than happy for the research people to have my body after death if they think it will help research but I've had enough poking and prodding and spending my savings with no discernible benefit.

The Prof is not any easy man to talk to. He expects that when he says jump, that you say how high?. This is not my style and I can envisage a huge argument occurring f I have to visit him very often. The volunteer lady who escorted me to the lab for the blood test, told me that she gets on well with the Prof but when he gets all arrogant and bullying she tells him off, she also refers to him by his first name just to remind him that he is no different to any one else. She told me that almost everyone is scared of him. It's one thing to respect a persons knowledge and experience and another to respect them as a person and I don't respect him as a person.

I was supposed to have 2 weeks off treatment but the next week of treatment coincided with my doctor being away for 2 weeks, so my treatment was moved forward a week. So I have daily treatment again next week and the next lot of treatment will be due Christmas week.....damn.

You know Catherine it's not so much the copper deficiency that is the biggest problem, it's the other medical problems that can't be treated. I told you that the surgeon doesn't want to fix my hip, well, I also have a stomach problem and I had surgery many years ago, unfortunately that surgery has failed and I need to have more surgery. I saw the surgeon yesterday but he thinks I have an increased risk of a gastric leak due to tissue compromise because of the copper deficiency and he doesn't want to operate either. He didn't say no, but he said he needed talk to my hematologist and neurologist and get their opinion on the risks versus benefits. The condition in my stomach is precancerous so it's not as if the surgery isn't necessary. It makes you realize that everything is connected to the copper problem.

Oh well, I'll make myself feel better by going handbag shopping

Regards

[cathybee1]

Chirley, I hope the surgeon you talked to yesterday will come through. Not doing the surgery doesn't sound like a very good option.

I would not look forward to another meeting with that Professor....and yes, everything affects everything else. Everything is connected. We have no idea whether copper is even implicated in Bruce's anemia. The family doctor thinks that Bruce's liver is the prime culprit. No real easy way to fix that.

At this point, after a year and a half of uncertainty about the diagnosis (!), I do think that living life as best you can, including shopping for a new handbag is the best revenge.

It's interesting about how trials work there -- if I'm understanding you correctly. I wouldn't be interested in a $1200 blood test either. Here, if they use you as a guinea pig, they foot the bill. But our system here isn't grand and glorious either -- there's lots we have to pay for, even with insurance.

Hugs,

[Chirley]

Catherine, I've never heard of anyone paying for their research blood tests either. I think the Prof is trying to get me to cover some of the costs just to keep the lab interested in doing the research because the amount of people this research would help is probably very small.

I didn't end up getting a new handbag but I did get a couple of new blouses even though I feel like I'm incredibly overweight and nothing fits well or looks good. InterEstingly the Prof wouldn't give an opinion on the weight gain but the surgeon blamed it jointly on my immobility and the steroids, which he said was "a large dose".

We are having an unusually dry start to our wet season and I need to water the garden but I have to be a bit sparing because water costs me about $320 every 3months and it's rumored to be due for a hefty increase. (isn't everything?).

Regards

[cathybee1]

New blouses are nice too!

[Chirley]

Catherine, I wasn't told which gene is involved in the drug trial but I did see (upside down and in bad doctor handwriting) what looked like HFE c282y. I looked it up and it seems to be a haemachromatosis gene mutation.

Hope this helps.

Regards

[cathybee1]

Yes...that is one of the genes Bruce was tested both last year and in October to rule out Hemachromatosis. He is negative. Interesting...

[truthlover]

Hi Chirley

I had high-risk MDS and did 3 cycles of Vidaza. After a couple of days, at the beginning of each cycle, I had severe muscle pain but it passed. I did not have much nausea. The injection sites swelled and became very itchy. That never really went away before the beginning of each cycle. All of my counts dropped like a stone but that included the blasts. Essentially my marrow was being cleaned out but nothing was being produced.

The treatment was very successful! My initial chances of success for my BMT were 30%. However 198 days post-transplant there is no sign of MDS or cancer. So far, so good.

Take it one day at a time. Good luck!
Michelle

[Cheryl C]

I wish you success with the Vidaza treatment Chirley. Very brave of you!

[Chirley]

I think I'd better point out the date of my last post. Oops.

I had Vidaza in 2010 and it was stopped because after 6 months I had no response except an increasing blast count. At that stage I was being worked up for a bone marrow transplant. It was after the Vidaza was stopped that I was found to have copper deficiency. My last bone marrow biopsy still showed MDS but my peripheral counts respond (never completely normal) well to copper replacement.

I really have no idea what my current diagnosis really is. Officially I still have MDS and I'm on the Federal Governments Cancer Registry but who knows? My current physician seems to think I will definitely progress to AML at some stage...I'll prove him wrong.

[Cheryl C]

Oh dear! It would be nice if the latest post would come up when we click on the link in New Posts wouldn't it (not that I want to criticise this website - it's fantastic). Sorry about that Chirley.

I didn't know there was a national cancer register. I suppose I'm on it too, then. I never think of myself as having cancer. Do you?

[Chirley]

Yep, you're on the cancer registry. I never thought of MDS as cancer until my blasts started increasing...that's when I accepted that it was indeed a cancer. I only found out I was on the cancer registry when I was given a copy of my bone marrow biopsy results from Sullivan and Nicolaides. Right there on the bottom in black and white it said that they had submitted my details to the cancer registry as per legislation. Who knew there was a law making them divulge that information to the government? Nothing is private any more. I'm a bit reluctant to agree to my medical information being embedded in a chip in my Medicare Card too. Seems a bit Big Brotherish to me.

[aussie danielle]

Hi there,Good luck with the treatment Chirley, i hope you do well with it
warm regards aussie Danielle

[alyasa]

Hi

Thanks for the info. And that you limit your use of supplements. Seems like a better way to go.

From on earlier post from beloved and generous Birgitta regarding blasts and neupogen FYI:

Originally Posted by Birgitta-A

Hi Chirley,
You know in Sweden the very good researcher, professor Eva Hellstöm-Lindberg, after many years of research on the leucemic transformation in MDS patients treated with Neupogen has said that there is no increased risk for transformation to AML for MDS patients. "We conclude that Epo and G-CSF treatment does not affect overall survival or risk of AML evolution in comparison with supportive care only."
http://bloodjournal.hematologylibrar...ract/106/3/803

I have been treated with Neupogen 2 injections/week since Sept 2007 when I had neutropenic fever and now when I get Thalidomide I take Neupogen 3 injections/week. My latest BMB June 2010 still showed less that 5% blast cells.

I think that a lot of antibiotics can decrease all counts especially the platelets - look at hematological adverse reactions for the antibiotics you are receiving.

As you already are aware of it is very common that the Vidaza response often takes up to 6 months - we hope for the response to come soon!
Kind regards
Birgitta-A

[Cheryl C]

Alyasa - Are you mixing me (Cheryl) up with Chirley? I posted to you last night (Aussie time) regarding Echinacea and Medihoney.

Unfortunately we haven't heard from Chirley for quite some time and we are concerned about her.