Watch and wait ends, Vidaza begins.

[cathybee1]

Thank you so much for this, Birgitta. And I agree completely that copper and zinc screening should be part of the basic group of tests that are run. As it turns out, our hematologist thought it was ridiculous Bruce even wanted to be tested for copper tho Bruce was tested for zinc -- it's a Family Practice doctor who is currently in charge of Bruce's IV copper supplementation because the hematologist said it wouldn't help. Bruce would switch hematologists, but he is the senior doctor in the only practice within 100 miles.

[Chirley]

Hello,

When I saw my doctor again on Tuesday he told me that after I finish this week of daily visits, I don't have to come back until the following Tuesday and depending on the blood test results taken then, he will try to work out a maintainance regime. I do have to restart the oral copper as well and I'll be taking 12mgs/day just to supplement the IV copper.

The RN in charge of the day care told me that I've also been booked for a BMB on Tuesday as well. This was news to me, my doctor didn't mention it. I think it must be a mistake because I think it makes more sense to repeat the BMB after my copper level has been maintained in the normal range for a period of time and it's hasn't even reached normal levels yet.

The ambulance is due to arrive so I'd better get ready.

Regards

[Chirley]

Hi,

this is the last day of daily IV copper (it seems so much more than a month). My copper level taken on Monday was 10. Still not in normal range but I've had a further 20mgs of copper since then. The previous 20mgs of copper had raised the level from 7.5 to 10 so I don't count that as a very good response and I don't have high expectations for my next test on Tuesday.

My doctor is going on conference leave for a couple of weeks starting the 13th June so I'm hoping we have established the maintainance regime by then so the copper doesn't start dropping and I have to go through the loading dose daily visit thing again.

Also my little dog Tina appears to be getting a little better each day. I have put her on a severe weight loss diet which doesn't seem to be working but I'll perservere with her pleading looks and little whimpers. She can weight bear quite well on her right hind leg and a little bit on her left hind leg, so she can walk around the garden even if it's only very slow with a lot of sit down breaks.

Regards

[Birgitta-A]

Hi Chirley,
Good that both you and Tina are a littler better! She perhaps had severe lumbago.
Kind regards
Birgitta-A

[cathybee1]

Hi Chirley

I do hope that you will be able to start a maintenance routine next week and this daily hellish commute is done with. And so glad that Tina is doing better as well.

Bruce's Hgb count only dropped another .1 last week. But he had a 3 week break in his copper pushes, there was a drop in his copper to 61 (74 is beginning of the normal range) so we're expecting some more drops in the hgb and a blood transfusion in the next few weeks -- but so far, so good.

Hugs, Catherine

[Chirley]

Catherine, I think that different people must need differing amounts of copper (or lack of) before their blood counts are effected. Bruces' Hb is low even with the copper level approaching the lower limit of normal whereas my Hb stays pretty much in normal level until the copper level is close to zero, then it drops 30-40/week. I did note that my RCC was below normal right up until my copper reached 10 (13-25). Also my WCC drops at around copper of 5 and is critically low at about 3 or less. Platelets are only effected when the copper is near zero. I have no idea why my lymphocytes remain low even when the copper is in normal range, I must remember to ask my doctor.

I have just finished another 24hour urinary copper and creatinine clearance test but the results probably won't be available until after my doctor goes on leave so I expect to have to wait a few weeks for the results.

Birgitta I know you said that you don't think dogs are capable of putting on an act for attention BUT I'm almost sure my dog is doing just that. I know she has an injury but I think she likes the attention she gets when I carry her outside and feed her by hand etc. When she doesn't know I'm watching her, she can walk (limping) on all four legs but if she knows that I'm watching her she only walks a couple of steps on 3 legs then sits down. If she wants attention I'm happy to give it to her, she is a wonderful gentle companion and deserves pampering in her old age.

Regards

[Birgitta-A]

Hi Chirley,
Yes, it really looks like your clever dog is trying to get more attention - good that she uses all four legs when she doesn't know that you are watching so the muscles get exercise.
Kind regards
Birgitta-A

[freedom99]

Good Luck in your Vidaza treatment.
My wife had three cycles of seven days each within 28 day periods.
Vidaza can be hard on the body and often the patient gives up too soon so persist even through the down times.
A medication called Kytril was taken a few hours before the treatment. The cost for this was over $300 for 15 pills but it worked with very few periods of nauseau.
After 3 cycles a BMB showed that the Vidaza was not doing anything and terminated.
Blood counts do go down but that's usually normal for a period of time before they start going up.
The goal is to become transfusion independant and stop the addition of iron from the RBC transfustions.
Every situation is different and so is yours so we hope and pray this will work for you.
It normally takes about 5 to 6 cycles before Vidaza has a positive impact.
My wife had her Vidaza as intramuscular injections which would only take about 15 minutes each time we went in. Again the treatment is different for everyone.
Hang in there and try to look beyond all this to your friends, family and the beauty of earth as it unfolds each day in all it's splendour.
God Bless

[Chirley]

Thanks for the good wishes Freedom99.

I actually had 5 cycles of Vidaza during which time my counts all dropped and the blast count increased. I also developed CNS myelopathy/demyelination. Further testing showed severe copper deficiency. Copper replacement resulted in normalisation of my blood counts (nearly).

Now, the dilemma is.... did I have MDS in the first place or was it all copper deficiency? My doctor now thinks that I didn't have MDS but that I do/did have some other underlying disorder due to abnormal blood tests and BMBs that evolved over a period of 8 years. The problem is that we don't know what the underlying problem is or whether it's progressive etc.

I don't understand why they stopped your wifes' Vidaza after only 3 cycles, it seems very quick to give up on it. My doctor was going to add Sodium Valproate to the Vidaza if it didn't start working after 6 cycles but we didn't get that far. Did that ever get mentioned to your wife as an option?

Regards

[freedom99]

Hi Chirley;
The body can be an amazing organism with all it's parts working together but also so very much a mystery when things go out of wack.
For your diagnosis to change so radically must be hard for you and the doctors to deal with.
My doctor looked at my wife's BMB and blood counts and seen no movements and did not want to push the envelope any further with the Vidaza (a nasty treatment) and considering she may only have less then a year to consider the transfusions as a better alternative than pushing for transfusion independance.
Makes some sense but with the input of these forums we can strive to reach further.
Remember that 30 years ago a child with leukemia had a 90%chance of dying and now today that same child will have a 90%chance of living. I find that so awesome.
Keep the good fight and stand strong.
Thanks for your input.

[Chirley]

Freedom99, I wouldn't take a lot of notice of the life expectancy given by your wifes doctor.

There is a gentleman at the clinic I used to go to who was elderly with other medical conditions. His MDS converted to AML and his doctor gave him a life expectancy of 2-3 months with no treatment offered. He got a second opinion from my doctor who started him on Vidaza. In the first month he got PCP pnuemonia and broke his hip but they perservered.

That was three years ago. He has now converted back to MDS and only requires RC transfusions rarely, but he does have regular platelet transfusions. He hasn't had any further infections and is feeling well enough to be an active participant in his building development company again.

He did have Sodium Valproate added to the Vidaza to increase it's effectiveness.

Regards

[Chirley]

Copper update.

My copper level from 7/6/11 was in NORMAL range at 13.2 (13-25). That test was taken on the day of my first weekly visit so it actually reflected the copper level post 4 weeks of daily IV copper.

Copper went from 4.4 to 13.2 over a 4 week period with a total of 48mgs of copper being given. Mayo clinic recommendations were for IV copper 12mgs over 1 week to fix copper deficiency. No way that regime would have worked.

24hr urinary copper still shows the excretion at 9 whatevers (<1) this seems to show that the excess excretion is constant despite what the blood level is. My doctor and I were wary that the more copper I was given, the more I would lose in the urine but luckily that doesn't seem to be the case.

Now the next hurdle is to see if the 12mgs/day oral copper and the 4mgs/week IV are enough to maintain the blood level.

Regards

[cathybee1]

Hi Chirley,

That's good news about your copper getting back into normal range.

Bruce is going in for a transfusion tomorrow a.m. Hgb is now at 8, which we expected after seeing the results of the last copper, which dropped from the mid 80's to mid 60's. (I wish Australia and US used the same gauge, so we could compare these numbers more directly.)

We saw his family doctor yesterday, and I asked him directly how much copper sulfate he is giving Bruce in the push right now. It started at 3 mg/week, is now at 4 mg/week. Doctor is going to start dosing him twice weekly starting next week, tho he will titrate the dose up gradually. When the copper went up, so did the reticulocytes, and less dramatically, the ceruloplasmin. So Dr. told us that means the liver is still capable of producing ceruloplasmin. Which is good.

And I don't know why it took this long, but Bruce is doing his first urinary excretion testing today. More data.

The nurse who administers the push talked to Bruce again about having a port installed. So far he has been resisting it. She told him at the rate his veins are being worked, for transfusions, and bloodwork, and the IV pushes, they won't hold up long, though she is trying to access different sites each time. He is going to consider it if it appears that the copper fixes the anemia problem. I know lots of people on the forums have ports -- but his resistance up to now is the rural nature of the place we live -- in a mountainous, windy and often dusty spot, where we haul firewood up a flight of stairs to heat with 7 months of the year.

It must be heaven to be at home. I'm sending all kinds of good energy your way that you don't have to repeat the commute from hell any time soon.

Hugs, Catherine

[Birgitta-A]

Hi Chirley,
Congratulations ! Hope your copper level now will stay in the normal range when you continue treatment!
Kind regards
Birgitta-A

[Chirley]

Thanks Birgitta, I'm very happy. It's a long time since things have gone in the right direction.

Catherine, I'm sorry to hear Bruce needs aother transfusion.
This was only the second 24 urine excretion test that I've had. I did have a spot morning urine when I was in hospital and the copper deficiency was first found. It showed lots of protein and too much copper and then the whole metabolic testing/diagnosis began.

I think the amount of copper required to maintain normal levels is underestimated. It took 4 times the amount for 4 times the duration that the Mayo recommended to get my copper JUST into normal range. I think that maintaining the level is probably going to take 6 to 8 mgs IV a week. That's a bit of a guess but I worked out the rate of excretion and added that to the replenishment and that's the figure I came up with (I know what I mean but I'm not very articulate). Although maybe it takes a lot more in the beginning because the body/tissue stores have to be replenished and isn't available in the blood and therefore the low blood copper levels initially.

My only CBC abnormality is continuing lymphopenia. I look at my counts now and it's hard to believe they're mine.

As for the port. I can't recommend it enough. I've had mine for over 3 years with no problems. After the initial tenderness, you forget you have it. I do anything I want and I can't feel it. The only problem I've ever had happened only a few weeks ago. The nurse inserted the neddle and commented that she didn't feel the needle hit the bottom of the port but it was okay and I had my treatment that day with no problems. I was sent home with the needle still in because I was having more treatment the next day. The next day when the next nurse flushed the port, the needle was in the wrong place and there was a stinging feeling and the skin around the port swelled up a little. It turned out the needle had come out of the port. The nurse took that one out and inserted a new one and no damage done.

As for the commute, aghh. I still have to do it twice a week. Once to have IV copper weekly and again to another medical centre/hospital to collect the oral copper (It has a one week expiry). The copper I take orally is made specifically for me at a production pharmacy which also requires a commute which is typically 2 hours each way. It only takes this long because the bus stops and collects and drops off lots of other people for medical appointments along the way. I'm usually first on and last off both ways. On the positive front, I'm getting to know other suburbs quite well and the coffee shop at the medical centre does serve good coffee. It helps fill in the time while I'm waiting for the community bus to return and pick me up.

I'm about to pick up more copper today so I'd better get ready.

Regards

[mausmish]

That's great news about your copper, Chirley! Glad something is finally going right.

[cathybee1]

In all that flurry of talk about US, I didn't ask about you pup. Hope she is still doing well.

That's very good news that you will only have to get your IV copper 1x/week now...but when I read that you still have a 4 hour commute weekly to pick up your oral copper, I wondered why they can't mail it to you. I get one of my prescriptions specially formulated and mailed to me cause it's such a distance from us (a 2 hour drive each way, just like your commute) -- it needs to be refrigerated too, so they package it inside one of those freezer paks. I suspect you've already had this conversation with them, but thought I'd mention it, just in case.

Last week, our doctor mentioned something similar to your comment about needing to give higher doses of the copper initially to get the serum level to come up. But he has been cautious so far, not wanting to overdose either.

Bruce starts his 2x/week injections tomorrow. Interestingly, my brother in law has been experiencing increasing neurological problems, neuropathy, and numbness. He's been seeking an answer for the past 4 years. I asked him today if he had ever been tested for copper. He had not. What's with these doctors?? Such a simple test.

It's finally turned to summer here, I can't believe the solstice is almost here. I suspect you're ready for the days to start getting longer...

Hugs, Catherine

[Chirley]

Hi Catherine,

Teeny dog is okay. She can walk on all four legs most of the time with a pronounced limp. I've had her on a strict diet for a few weeks now and was sure she was losing heaps of weight so we weighed her yesterday. She's lost a grand total of......0.1 of a kilo!!! How disappointing is that. I blame the steroids she has for her Addisons Disease, her diet has been really severe and my other dog has lost loads of weight.

Not only have I asked if the oral copper can be sent to me, I've also asked if it can be sent to the daycare that I go to for the IV copper but because it's specifically made for me, has to be kept at 4 to 8 degrees, expires in 7 days and has to be paid for (I suspect this is the main reason, cynic that I am), I'm told I have to pick it up from the pharmacy in person and sign for it. It's annoying because I think it's a waste of time. Last time I was on oral copper my blood level dropped consistently every week and my Hb dropped fro 116 to 73 in a matter of weeks. It also costs $26 for the copper and $15 for the commute. I could have lunch out with friends with that money.

I just bought a breadmaker on ebay and it was delivered this morning so I need to go out and get some yeast so I can have a play.

Regards

[Chirley]

Yet more good news. Last weeks copper level came back at 13.4 so it has risen a little even on the once a week regime.

I received a phone call from my neurologist when I got home from daycare today. He wants me to go into the childrens hospital where he works and sign a consent form and give blood for gene testing to be sent to Sweden. He said the cost for this is normally $2000 but that the hospital has agreed to absorb the cost. I asked if this was for research or for me and he said that it would be for research but in the end would benefit me as well.

As selfish as this sounds, and I know it's only another blood test, but I was just starting to think I could have a break from the ongoing medical interventions and settle down to a routine of maybe IV copper once a fortnight and forget about health matters in the interim. Now the neuro is talking about finding out why I can't absorb copper and "treating" the source of the problem which sounds good in theory but could mean more intrusive therapy in reality.

Am I being selfish in wanting to forget about further testing? Last time I gave blood for a research study (I was supposed to be a normal subject)they found out I was homozygous for MTHFR which increased my risk for arterial clots and vascular disease, I didn't really want to know this because there was nothing I could do to alter my genes.

Going to watch Wimbledon, Gnight.

Regards

[Neil Cuadra]

Chirley,

It's OK to take a break once in a while, when you get the opportunity to avoid the seemingly ceaseless tests. It's not all-or-nothing; you can skip an optional test this time and do one the next time. If you skip this one, don't feel selfish or guilty about it.

Over the long-term the testing that our doctors do benefits both us and medical research, but it could do you good to enjoy the luxury of a little mental vacation and let yourself be Chirley instead of "the patient" some of the time. It's part of taking care of yourself.

[cathybee1]

Chirley, I think you could very diplomatically tell your doctor that you are worn out from the Commute from Hell and it's time to play with your bread machine and your dog for a while. Scientists tend to get excited about looking at other peoples' genes , nothing selfish about wanting to keep that information to yourself for a while

Seriously..you do deserve a break.

Hugs, Catherine

[Chirley]

Hi,

New day, different thoughts. I think I will give the blood for research and then decide later what to do if they find anything they want to treat.

Is it possible to put on weight on 200mgs hydrocortisone IV a day for a month? I seem to have put on a lot of weight in a very short time and my face seems a bit swollen. I've been watching my diet because my cholesterol and triglycerides were high for the first time ever. Well, they have come down to low normal but now the weight thing. I hope it's not a side effect of copper, eeek.

Thought I'd post photos of the rest of my "family", Gidget the dog and Betty the cat. Teeny dog has already been posted.

Regards

[mausmish]

Great photos - they both look like sweethearts.

I think it's common for any steroid to produce the puffiness and weight gain from fluid.

[cathybee1]

That is a VERY handsome family you have. Now I see the image link to post photos. Thanks for pointing it out to me.

Part of Bruce's urine test came back today. Interesting. It turned out it was a heavy metals panel. Bruce showed a little bit arsenic excretion (what would that be from?), and a lot of bismuth excretion which is one of the ingredients in his supplement to sooth his acid reflux. The copper wasn't back yet, that cost another $25 and additional time to process.

Chirley, I believe we should send our copper related tests to Australia to be processed, they'd probably get back to us sooner than we're getting them now.

Hugs, Catherine

[Chirley]

Hi Catherine,

I asked the pharmacy to provide the formula for the copper solution but they declined. I was told "it's just copper sulfate in water". It does have the side effect of causing frank fat excretion with the bowel motion if you have too much. It's not very pleasant and I've had to reduce the dose the doctor wants me to take. My GP said that it would have a similar effect as Xenical weight loss capsules. I figure that if I'm having diarrhoea problems then I'm more than likely just passing the copper out and there is no point taking it in the higher dose anyway.

Regards