Watch and wait ends, Vidaza begins.

[Marlene]

Ha....that's funny. I'd take the admin over the elephant &*%$ any day. Maybe there's an APP for that!

[cathybee1]

Hey Chirley,

Laughed out loud. Elephants and giraffes win over offices for me too, but hey, it's the zoo!!

(shhhh) Bruce's copper result came in today. Normal!! Also his zinc. The bloodwork done at the same time (last week) was not. But the idea is to get it to stay in normal range for a few cycles and see. Ahhh, it was time for a little better news.

Saw the family doc Wednesday, said the stool test revealed Bruce is a "copper pooper." (exact quote). Family doc is anxious for Bruce to have another bmb. Bruce is adamant he will only do it with some kind of sedation this time. Right now it's at stalemate, Bruce's (part time but Senior in the practice) hematologist says he'll only do it in the office, where he is not equipped for any kind of sedation. Plan B is to drive 300 miles to the hospital where Bruce had his last bmb, under sedation. Family doc agreed to call another doctor in the practice, and see if common sense will trump office politics.

I get your mixed emotions about going on Disability. That is a tough one.

We're gonna go birdwatching this weekend, get out of town and away from the phone for a few days.

Hugs,

[Chirley]

Hi Catherine, it's good to hear Bruces copper is normal, pity the blood is still abnormal.

My copper is also normal but my platelets have dropped, I don't know the actual count. I was just told...low. So I'm booked for copper and platelets on Monday. I've never had platelets before, just blood.

The weather here is beautiful but that has encouraged hundreds of parrots to nest in the gum trees next to our house, the noise is incredible but at least it's mainly dawn and dusk. However a colony of bats have also taken to feeding on the date palms on the other side of our house, so they chatter and fight all night. We are also coming into storm season so we will start getting the usual summer storm bird calls which can be very loud and intrusive. It's a good thing I love nature and wild life.


Regards

[cathybee1]

Chirley, sorry about your platelets dropping. yes, it seems we get one thing fixed with Bruce and another goes wrong...just like an old car, I guess.

I forgot to mention that both of Bruce's index fingers are going crooked. The dr. thinks it's from the copper deficiency. I didn't know it manifested itself that way, but...

Had to laugh about your wildlife. And nice they're so close to home. Hope the parrots are colorful, but yes, they are very very noisy, aren't they. We seem to get our bats here in small quantities, ones or twos -- occasionally they find their way into our house. I have become an expert bat wrangler. Better outside than inside, I say.

Hugs, Catherine

[Chirley]

Hi Catherine, I talked to my doctor about joint pains etc and he said that iron overload causes finger/knuckle swelling and deformities. My whole body seems to be effected though. My left shoulder has been painful for a year or so and I cant lift my arm above about 70 degrees, my right shoulder is starting to go the same way, my right knee aches and grates and cracks and gives way and my right hip has arthritis tendinitis and a torn cartilage. I have a new broken bone in my spine...this makes number 4.

Add these problems to the myelopathy and my GP can't understand why I don't follow his advice and join a gym....I sometimes think they are out of touch with reality.

About the bats, we don't have Rabies in Australia but here in Queesnland we have a virus which is carried by bats and has always proved fatal when caught by humans. It has also recently been found in a domestic dog which had no symptoms but was tested because it lived on a farm where a number of horses had died from the virus. In horses it's called Hendra Virus although the virus itself is the Lyssa Virus. Our bat colonies number in the thousands and people are becoming very alarmed when they colonize trees in suburban areas.

Expecting the first storm of the season this afternoon, lovely.

Regards

[Chirley]

G'day, I've just come home from daycare. I don't know why I was told I needed platelets because they were a very respectable 137 last week and the test I had today the CBC was completely ...normal.

I'm still going to have IV Copper every day this week and depending on the combination of CBC results and blood copper levels I may have 2 weeks off and 1 week on or 3 weeks off and 1 week on. My 24 hr urinary copper still shows high excretion levels and high protein but the Doctor is not going to do anything about the protein loss unless it gets worse.

He has finally addressed the ferritin overload and I'll probably start venesections in about 2 copper cycles. He also said he wants me to do hydrotherapy and see an orthopod to arrange surgery on my hip. He thinks it's vital for me to remain as mobile as I can because he said that in contrast to the neurologist he wants to believe that my spinal cord damage is reversible.

Regards

Beached whale doing hydrotherapy...they'll send the Seaworld wildlife rescue team out to me and that could be embarrassing, while hip surgery is just plain scary but I suppose I'll have to seriously consider both options.

[Birgitta-A]

Hi Chirley,
Very strange with info about a platelet transfusion when your CBC was normal ! You have better ask again why they plan that.

Good that your Doctor hopes that your spinal cord damage is reversible !
Kind regards
Birgitta-A

[Chirley]

Good morning. I had my first venesection yesterday. The nurse inserted a canula into my ante cubital fossa and attached a blood bag, drained 500mls and that was it. No fuss, easy. Then I had a litre of saline and started my copper infusion.

Finally I'm moving in the right direction. Yesterdays blood test was good except platelets at 135 and MCV at 103, but still really good compared to what they used to be.

I've noticed lately that my MCV is quite often elevated and my doctor hasnt made any comment, would I be right in thinking it would be caused by the high ferritin level and not of any consequence?

Turns out the nurse thought I needed platelets the other day but my doctor had told her that someone with the same name as me needed platelets and she picked the wrong person. Good thing they triple check these things at the chair side.

Regards

[cathybee1]

So much good news, Chirley!!! Even the screw up was excellent.

Our news is not so happy, but, it's news.

Bruce had an "emergency" transfusion today. His hgb had dropped to 7.9 by Monday -- he was feeling so good over the weekend too, canoed for 6 hours. Scary when he can't feel how low he's getting. This is the 2nd lowest it's been before he has been transfused.

MRI results came back last week, iron stores in the liver. So a liver biopsy is in the works. Ha, and I thought the MRI was supposed to replace the liver biopsy.

Lots of talk by the family doc about scheduling a bmb right away, but the hematologist is away for 2 weeks. Even though Bruce doesn't want the hematologist to do the bmb, he needs to still bless it. Hurry up and wait.

I am so so glad we had a weekend away. I am also glad Bruce had his transfusion today, he was getting dopey (not surprising considering). We are going to the coast next weekend, and Bruce thought he could wait another week till the transfusion. I am filled with gratitude that he didn't delay getting the bloodwork, and that the transfusion was scheduled without a hitch. Did I tell you before that we are dealing with doctors in 2 states, so the bureaucracy up until this time has been pretty hellish. Everybody cooperated very well yesterday to get the paperwork through. The blood he got today wasn't a day away from expiration, either . The hospital left the port accessed, he came home, and got his copper infusion, ate, watched TV, and went to bed.

He's planning to move more wood tomorrow.

Hugs, Catherine

[Chirley]

Hi Catherine, I hate to say it and I hope I don't worry you but it seems as if Bruce definitely has something more than copper deficiency going on. If copper is in normal limits and he's still dropping his Hb than it obviously has to be something else too.

I am responding well to the copper but even so it's always in the back of my mind that I have some other problem too. My platelets still drop and my lymphocytes are more often than not still less than half normal. Also copper deficiency isn't supposed to cause increased blasts so there is always a seed of doubt in my mind and it prevents me from fully enjoying the response I have had to the copper. I don't want to get excited and then be dealt another blow, I've been there and done that and don't want to go there again.

My doctor was going to order a ferri scan but they aren't covered by Medicare or health insurance so he decided to do the venesections without it in the light of my increased liver enzymes. I feel blessed that I'm in a position where my Hb allows me to do this now.

Enjoy your weekend away. My Tassie holiday is 3 weeks away and I'm getting impatient for it to start.

Regards

[Marlene]

Hi Catherine,

Why do you need to do a liver biopsy? What's the point if you know you have iron overload... why risk it?

Thanks....Marlene

[cathybee1]

Hi Chirley. Yes, copper is now barely normal and no movement in the hgb. Last time it was in normal range (briefly), there was a lag before hgb moved.

Marlene - a very good question, and my question too. Bruce has had elevated bilirubin for years. He consulted a liver specialist at UCLA after our hematologist found he was homozygous for H63D gene, one of the gene markers for hemachromatosis (in other words, a carrier). The hematologist has maintained this resulted in a predisposition to hemachromatosis, the liver specialist said no, being a carrier in this case conferred very little risk.

His ferritin levels have increased since transfusions started, a normal occurrence. The liver biopsy issue came up early on, then the hematologist said the risk was too high. Bruce requested the MRI be done as an less invasive alternative. It finally got done a couple weeks ago.

When the MRI came back positive for iron stores, our wonderful GP hit the wall, he is opining Wilson's disease, and hemachromatosis. Both the GP and hematologist now want a liver biopsy and Bruce to see a liver specialist.

I suppose their point is to see if damage to the liver has progressed to the point of cirrhosis.

B and I had quite a talk about it today. This is all confusing to us. We don't know where the Wilson's disease idea came from. Some of Bruce's symptoms are consistent with Wilson's disease (low ceruloplasmin, low serum copper levels, liver issues). Others less so. And, from what I am reading, Wilson's is difficult to diagnose unless there are neurological issues. Treatment is chelation. Which of course is not currently indicated.

Nonetheless, doctors are all about data. The local ones now are downplaying the risks of a liver biopsy. At the moment, Bruce is willing to have one done in hospital (as well as a bmb because MDS is still out there as well) and then return to Los Angeles for consultation with the bone marrow specialist and liver specialist we saw last year.

[Marlene]

Well that is confusing. Isn't Wilson's too much copper in that your body hangs on to it? And it can show up as a brown spot or ring in the eyes? You certainly have some stuff to sort through on this one don't you.

[Chirley]

They considered Wilsons Disease for me too but decided to "opt" for deficiency instead because of the high urinary excretion but I've looked it up and you can have high excretion in Wilsons. In light of the raised liver enzymes I have questioned if I have Wilsons but they are sticking with the deficiency syndrome and won't even consider re evaluating despite all doctors agreeing that the presenting blood/BMB results weren't consistent with copper deficiency.

I just have to trust they are right.

Regards

[cathybee1]

Hi Marlene and Chirley,

Yes, it is confusing, Marlene...Wilson's is too much copper in the body. And apparently Wilson's is a tough one to diagnose. You're right on about the tests. Primary diagnostic tools, from what I have read, are the "slit-eye" test, urinary excretion test, and low cerulosplasmin.

As Bruce's urinary copper excretion test was done after Bruce was already taking 10 mg oral copper daily, and 32 mg/week IV, it is difficult for me to believe that information can be used to rule Wilson's in or out. No one's discussed the eye examination yet, but I suspect that is coming.

[Chirley]

BTW, no one has examined my eyes either.

Regards

[Marlene]

So I checked with Life Extension Org to see what they had to say about Wilsons and came across this. It's on liver degeneration overall but this page talks about Wilsons. Towards the end of the article they do address nutritional support for liver disease. Zinc stands out for Wilsons. Hope you both find it helpful. And I think, you should be able to look at your eyes for the coloring. I know I would be staring at the mirror .

http://www.lef.org/protocols/prtcl-125.shtml#wils

Good luck ladies....I will keep an eye out for info on Wilsons and if I come across anything, will post it.

[cathybee1]

Thank you, Marlene for the link -- very informative.

[celebrations]

Hi ladies,
can you please help me with this point (quote)

The local ones now are downplaying the risks of a liver biopsy.

I will have a liver MRI the 24th of october.
Since being transfusion-dependent (120 packs in the meanwhile) iron-overload is to be expected.
I am chelating sucessfully with Exjade (1000mg/d), last ferritin level was 1011.

Thank you very much
bergit

[Marlene]

So why are you having the liver MRI in the first place? An FE of 1000 is not that bad. Are you having problems with your liver?

[Chirley]

Marlene, I think Bergit means 1,000 mgs of Exjade a day.

You know it's getting really annoying here that more and more medical procedures/investigations are not being refunded by Medicare and or private health insurance and either whole cost or gap expenses are very high. To have a MRI liver for iron is not covered by either Medicare or health insurance and I don't know how much it is but my doctor tells me that it's really expensive.

In Australia we pay 1.5% of our income plus health insurance premiums or 2.5% (i think) of our income with no health insurance and we are SUPPOSED to be entitled to free public hospital care and subsidized GP and investigative care but the amount of money refunded for pathology and radiology is starting to whittle away to a small percentage of the actual costs.

I know we are faring better economically than a lot of countries but it's still hitting the average person in the hip pocket.

Now they are saying major recession early next year. Time to put my money under the mattress.

Regards.

[celebrations]

dear Chirley and Marlene,

my docs think - and they might be right (long story !) - that my ferritin level does not indicate my iron status properly.
My liver counts are elevated.
A liver MRI once in a while is standard in Germany, when you are transfusion dependant, so is a cardiac echo (is that the correct medical jargon?). I had it done some weeks ago, NO signs of cardiac iron overload. Yupee!
The MRI is covered by health insurance.

yes, and I take 1000mg Exjade per day.

Reading your dialogue I got the impression, that a liver biopsy would be dangerous (not that I will have to get one necessarily). Can you tell me why?
thanx,
Bergit

[Marlene]

Regarding the liver counts, Exjade can be the cause. Have you stopped taking Exjade for about 4 -6 weeks to see if that resolves the problem?

The liver tends to bleed more than other organs when cut/biopsied. So there's a higher risk of bleeding after the biopsy. This is even more important if your platelets are low or you do not clot quickly. With MDS, some find that their platelets are less efficient even when they are not dangerously low. And of course, if your WBC is low, infection is also risk.

In general, liver biopsies for all people carry a risk of bleeding. But the risk is even greater when dealing with less than optimal counts. You can have surgery with 50K platelets but I'm not sure if that would hold true for the liver biopsy.

[Chirley]

It's interesting that you mention that'd ferritin does not reflect your true iron stores. My doctor says the same thing to me. He is adamant that my ferritin is falsely low. Its about 1600-1700 at the moment but he thinks the one true ferritin reading ihad was one when my copper levels were zero. That ferritin reading was in excess of 5000.

I'm hoping to receive a disabilities pension shortly and then a lot of my tests etc will be covered entirely by Medicare. The MRI liver for iron will still cost money but hopefully I will receive a discount. My liver enzymes continue to increase slowly.

Regards

[cathybee1]

So...the last couple of days we have been focused on bmb's, liver biopsies and MRI's.

It turns out that we are in fact going to have to drive over 300 miles so Bruce can have a bone marrow biopsy with IV sedatives. I was pretty astounded that propofol is a ho hum for a colonoscopy, but according to our hematology office (the only one around), a bmb is always (underline always) done in office, and they are unable to set it up any other way.

So, after this sunk in, I started making phone calls; the University hospital in Portland, Oregon was very responsive, and scheduled Bruce to have it done next week. While we were setting this up, I asked if the liver biopsy could be done there as well. The hospital was obliging about performing it, but the hematologist balked, and wouldn't send an order. That made Bruce very crazy, 2 weeks ago, the hematologist not only wanted it done but wanted it done locally in a doctor's office. Bruce arrived home too late to do anything but fume, but the upshot was that after dinner Bruce and I watched a video on You Tube about liver biopsies, Bruce decided it was too risky, and today Bruce and the hematologist talked again. Besides the normal risks of a bleed (not statistically high but very very serious if they occur), Bruce is currently 5 months post coronary stent and on Plavix. Bruce would need to discontinue the Plavix for at least a week prior to the BMB, and the cardiologist has not yet weighed in. Bruce's main question to the hematologist was whether any different treatment outcome was likely following a liver biopsy. The hematologist said no, there were risks, reduced platelets since taking Plavix are a concern, but it would be good information to have, and it could still be done locally. They both agreed delay was good at least for the near term. I was glad I was taking the dog for a walk while this discussion went on. But I was very relieved Bruce was going to put it off, at least until we can get a copy of the MRI report to read ourselves.

Bergit, you are right, it is a given that transfusions lead to iron being stored in the liver. The MRI was supposed to be a non-invasive way to determine whether the liver had been damaged. The MRI did in fact show iron in the liver. But according to the doctors, the MRI is not sophisticated enough to show how much damage has occurred. And MRI's are very expensive here, even with insurance. So they are not routinely used for screening, because if serious damage is suspected, liver biopsies are the "gold standard."

The hematologist would have liked Bruce to start Exjade as soon as his ferritin hit 1000 early this year, but as you have already discussed, ferritin numbers are not always accurate. Exjade carries its own chance of gastrointestinal and renal side effects, and apparently chelates copper and zinc to some degree as well as iron.

So...next step is another bone marrow biopsy to see if there are more definitive signs of MDS this year than last. Do another consultation with a MDS Specialist and Liver Specialist. And see if anything changes now that copper and zinc levels are finally normalizing. Then go from there.