Watch and wait ends, Vidaza begins.

[Chirley]

Hi,

I thought I'd give an update on my treatment.

Vidaza has been ceased due to neurological problems.

I'm currently in hospital with mobility issues. My neuro examination has worsened substantially and now it's unsafe to walk unassisted. I have developed reflex abnormalities up to the level of T3 and I'm grossly ataxic.

My neuro doc says the increased signal in the cervical spinal cord has increased since the last MRI and the fluid taken at lumbar puncture shows increased protein and albumin. Tests on the protein have come back positive for CJD. This has caused excitement at the laboratory and with my neuro and haem docs, the neuro doc is getting one of his colleagues to come and see me as well because it's rare to get someone testing positive for this protein.

On the other hand my haem doc still thinks the Vidaza may have caused the neuro problem and so it's definitely no longer a treatment option. He says even if it's not the causitive agent it may be potentiating a disease that would otherwise lay dormant.

I've just had an echo of my heart but the only comment the tech made was to say he could see iron deposits in my liver (funny place to look for the heart!!)'

The food in this hospital is excellent and abundant and dinner is on it's way so I'll sign off.


Regards.


Chirley

[Birgitta-A]

Hello Chirley,
How sad that you tested positive for CJD. Hope they will find a treatment that will decrease the symptoms!

When I had an MRI examination of my heart to control if I had iron deposits (not a trace but that is 3 years ago) they controlled the liver too because the liver tries to take care of the iron - it goes to the heart when the liver cant take care of more iron.

Good that the food at the hospital is OK!
Kind regards
Birgitta-A

[Chirley]

HI,

I thought I'd post an update for those I haven't kept in the loop. Turns out I have a metabolic disorder which prevents me from absorbing copper and also causes me to excrete copper in my urine. The Prof of Metabolic Medicine that I saw thinks I have had this problem from birth and have been very lucky not to have health issues a long time before this.

This copper deficiency initially caused anaemia which on the surface looked like iron deficiency because of a copper/iron chemical interaction somehow (I'm ignorant of the nitty gritty chemical stuff). It then caused problems within the bone marrow due to lack of copper interfering somehow with the maturation/production of stem cells. This caused the RCC problems and low WCC as well. It also caused iron to build up in the cells and caused ringed sideroblasts and increased iron stores. It should NOT have caused my increased blasts and that remains a concern.

I have been having copper injections and my copper level has been normal for about 8 weeks now. My FBC has been almost normal as well and I haven't required a transfusion for 9 weeks.

I had and still have demyelination of the spinal cord in the cervical and thoracic spine. This was caused by the copper deficiency and neuronal/nerve death. I was told after my first MRI (I've had 3 now) that I also had white matter lesions in my brain, this has not been mentioned to me again. Consequently I have a grossly ataxic gait and abnormal neuro reflexes and still need a wheely walker to be able to walk. The thinking is that I'd be very lucky to achieve any improvement but I'm optimistic. My GP says the neuro problem is very similar to Multiple Sclerosis in its’ effect.

Only other issue at the moment is increased liver enzymes and mildly enlarged liver. My haem doctor seems reluctant to blame the liver problem on my ferritin which is 4,700 at the moment. So, it's off to a hepatologist to see what the problem is. The only symptom I get from the liver is a dull, achy, heavy feeling under my ribs front and back on the right side.

I had a BMB today to see if I really have MDS or whether all the haem problems were/are copper related.
FBC today was.
Hb 122 (115-165)
RCC 3.8 (3.9-5.6)
WCC 2.7 (3.5-12)
Neuts 1.3 (1.5-8)
Plats 102 (150-400)
as you can see, they're pretty darn good but not quite normal which is the reason the haem doc is reluctant to say I don't have MDS.

Keep your fingers crossed for me. Getting results next Thursday.


Hope you're all remaining well.

[Birgitta-A]

Hi Chirley,
Congratulation to the good counts ! I remember when you got several units of PRBCs every time you got a transfusion and when you hardly had any neutrophils at all .

As I probably have written before we manage very well with one third of the liver. Even if the liver enzymes are increased you probably won't feel anything from the liver as soon as the ferritin level will decrease now when you not are transfusion dependent.

Hope the BMB won't show more blast cells than we should have and that the neurological symptoms will decrease!
Kind regards
Birgitta-A

[cathybee1]

Hello, Chirley, your count numbers are a very happy new year gift. I have read your posts with great interest since Bruce is now exploring the possibility that his hematological problems are related to his inability to absorb copper.

Though Bruce has been on oral copper supplementation since October, his copper levels have continued to drop.

The result of the first test was 71 which is barely normal (normal range here is 70-140 ug/dL), it went up to 78 after he started supplementation, but subsequently dropped and is now at 54.

He is now on a 2 week testing regime for a number of minerals and copper markers and the doctors are now talking about IV copper supplementation. He is going to have a comprehensive nutrititional evaluation (involving urine collection and bloodwork) next week.

One interesting thing that surfaced at the last doctor's visit was the relationship between fiber and copper absorption. Fiber apparently can bind to minerals and prevent absorption by the body. We have been told so many good things about fiber, and not much about the downside. As a result, Bruce has changed the schedule for taking his supplements to a time when he is not ingesting any other supplements, food or fiber.

The doctors were initially very dismissive about our request to test for copper because it is so unusual to have a copper deficiency in a developed country. After hearing your story, I hope that other patients with bone marrow disease diagnoses make sure that copper testing is included in their work ups.

[Chirley]

I saw my GP this AM for yet another UTI. While I was there he phoned my haem doc.

Haem doc told my GP that my BMB last Thursday showed improvement but that I still have MDS (damn,damn,damn). Also they are now thinking that I DO have some other CNS problem besides the copper deficiency because of the CJD protein (14-3-3) in my CSF and because my neuro findings are progressing despite copper replacement (am having bladder incontinence problem which is new and devastating).

At least I've been alerted to the findings and won't get upset in the haem docs office on Thursday.

It looks like the fight goes on.

Take care everyone.

[cathybee1]

Grrrr, Chirley. It never stops, does it? Sorry about the neurological issues continuing, but improvement in the BMB sounds like a good thing to me. Even if it's still MDS.

We have talked to our doctor about your copper protocol, that may be the next thing for Bruce to try.

Hope you're still dry.

[Chirley]

Hello,

I saw my haem doc this am and he showed me a small portion of my BMB result. It shows that I still have dysplastic red cells, still with ringed sideroblasts and increased platelet precursors. I didn't see any other portion of the result except blasts are back down to 3%. Even though I still have MDS, this is great news because the doc says today that he doesn't want to give me Vidaza again because he thinks it may have increased the copper metabolism problem. He thinks this because the lab has looked at my previous BMBs and not seen any evidence of copper deficiency prior to starting Vidaza. So we are back to square one in regards to the cause of my metabolic problem and how long I may have had it.

The oral copper doesn't seem to be holding my levels now and this weeks test shows my serum copper has fallen to 10 (13-25) from 14.4 last week. My haem doc isn't looking after the copper side of things and the neuro doc and the Prof are both on holidays so all my haem doc was willing to do was to increase the oral copper dose to 6 mgs/day. He is also reluctant to start venesections (don't know why) for the increased ferritin and also doesn't want to start Exjade because he doesn't know if it will interfere with the iron/copper balance and whether copper may also be chelated. I'm booked for a repeat MRI brain, cervical and thoracic spine next Friday.

So, it's watch and wait again.

Regards.

[cathybee1]

Chirley, what a roller coaster ride you've had.

Bruce had metabolic testing yesterday, we're hoping to find out more why he hasn't been responding to the oral copper.

I hope the doctors know more than we do about this. There is so little information available about it.

Your posts have been very helpful. I can't tell you how much I appreciate them. I hope you are feeling a bit better.

[Chirley]

Hi Catherine,

Yes, it's been a roller coaster alright but at the end of the day, I'm alive and the MDS has improved.

When I was in hospital in Oct/Nov my neuro doc asked another neuro doc from another hospital to come and see me for a second/third/fourth opinion. This new doc told me that my best chance of survival in the short term was to have an urgent BM transplant. Well, that night, I was convinced I had a short time to live because no donor had been found in 7 months of looking. It sounds as if I gave in but I meditated on it and came to peace with death. So, even though it was an emotional time, it did make me confront my mortality and realise, while not exactly wanting it, I would be able to accept it peacefully when the time comes.

In the meantime, I've been given a reprieve and even though I have physical problems I'd rather not have, I'm still enjoying the life that has been given back to me.

On the copper metabolism thing, it's all above my head. It has something to do with cations, enzymes, proteins and their interactions (or lack of). Even my haem doc says he doesn't understand it but that he isn't a Prof of Metabolic Medicine. He said that I was lucky enough to come to the attention of Australias' most qualified doc in metabolic diseases but even he is not sure of the mechanism of the chemical reactions (if that's the right way of expressing it). Apparently, there's a huge amount still to be discovered in this field and that's part of the reason the Prof is so interested.

I wish you and your husband all the best.


By the way, how is his Vit D level? I had very low levels.

[Chirley]

Hi,

Just got my last copper level. Has dropped a little further but not too bad, now down to 9 (13-25).

Catherine, has there been any news about Bruces' test results?

About staying dry... floods have receded they reckon about 20 to 30 billion dollars damage. Now a category 4 (hopefully won't be a 5) cyclone heading for Queensland as well. On radar it is massive, supposed to be as bad as Katrina was. I wish everybody in it's path the best of luck and hope for no loss of life we have had enough loss of life already.

Regards.

[Birgitta-A]

Hi Chirley,
It is really very difficult to have so uncommon symptoms as you have. Perhaps you only had copper deficit from the beginning (from childhood?) and all other symptoms have started when the copper deficit not was discovered and treated. If I remember correctly you had neutropenia for years before you were told that you have MDS and then they at last found out about the copper.

When you have had copper deficit during so many years nobody can expect that the symptoms will disappear soon - it will perhaps take a very long time with copper treatment before your bone marrow will be able to produce blood cells that not are dysplastic.
Kind regards
Birgitta-A

[cathybee1]

Hello, Chirley.

I am praying that Australia is spared this time. Drought, flood and now this.

Bruce has a Dr's appt next Monday and Bruce hopes to go over the results of his metabolic tests. He changed the timing of intake of his copper supplements, and it appears his copper is up a little to 62, but still well below normal (normal 70-140). Ceruloplasmin is very low. His Zinc, B12 and Folate levels are normal, his iron and ferritin levels are high -- ferritin at about 1200. Results of his bloodwork today show his HGB has dropped a little to 9 and his ANC is very very low. He is currently being treated every other week with Aranesp and Neupogen injections. His last transfusion was in November.

We'll keep you posted, again you are in our thoughts about the storm.

[Chirley]

Hi,

My last copper level has come in at 7.8 so it's dropped again. I have my MRI on Wednesday (my appt was changed) and I see my haem doc on Thusrday. I have an appointment to see my neuro doc at the Childrens' Hospital on the 23rd. (he prefers the Kids Hospital because it has a nicer atmosphere. He says children don't whinge as much as adults.)

One thing that I've noticed and I'll mention it to my haem doc next time, is that as my copper drops so are the liver enzymes. It seems to that my liver can't cope with a normal copper level AND high ferritin. I'll be happy when/if I'm started on the venesections that have been mentioned on a few occasions.

Catherine, Queensland didn't fare too badly from TC Yasi. A couple of smaller towns were destroyed but luckily the bigger population centres were only slightly affected. One of my old school friends from high school, lives in Tully which was badly damaged. Karen and her husband own a mango plantation just outside the town and I haven't been able to contact them due to power and phone outages. I know they're okay because all the injured have been identified but this will be the 2nd time in 5 years a cyclone will have destroyed their crop. Luckily Australians and specifically Queenslanders are very resilient and always bounce back better than ever. I hear that they have too many people wanting to volunteer to help with the clean up and rebuilding and have had to ask people to wait a while until the army/SES (State Emergency Service, a volunteer organisation) have determined what needs to be done and what order to do it in. A lot of the mess is already being cleaned up and the rebuilding phase can start shortly. I'm very proud of my fellow Australians and the concept of "mateship".

Oh, I almost forgot. I've been referred to the University of Queensland Neurological and Balance Holistic Clinic. I should have access to everything from OT to PT, I'm looking forward to it. It's not a private clinic and so not eligible to be covered under my health insurance but because they have Masters and Doctoral students working there, the costs are kept to a minimum.

Regards.

[cathybee1]

Chirley, that news about the Queensland Clinic is MAJOR. I'm imagining you will be their star patient, and get the star doctors as well.

Also good news about the cyclone. The news we've seen here has been dire in terms of damage, and good in terms of deaths from the storm. Heard someone from there refer to Australia as a lucky country -- though to many of us elsewhere, we would not say you have had very good luck this year. Even so, I have been very impressed at the resolve of you folks to move ahead. I am proud to share the planet with you Ozzies (is that a proper word?). I was thinking of you, Chirley, quite a bit as the news was unfolding, and was very relieved to see your post today.

This copper business is discouraging...it's been 6 months since diagnosis, and the doctors have just recently started to show some interest -- we're hoping to have a different course of treatment offered to us on Monday, as the oral supplements just don't seem to be cutting it. Bruce has been feeling a bit worse this week, so we'll also see next week if the Aranesp is still letting him "hold his own."

Bruce's bilirubin levels and liver #'s have been out of whack for years, but that has now taken a back seat as they work on his blood. His ferritin levels last time were at around 1300, not terribly high as some folks, but still much higher than normal.

All in all, it's been a good year so far. We took a drive yesterday to the wildlife refuge and looked at the eagles and hawks wintering there. Bruce is not able to be as active as he was even a year ago, but there are still many things we can do to enjoy life.

Hugs

[Chirley]

Hello Cahterine,

Sometimes I feel getting a doctor to "really listen" is like pulling teeth. I hope all goes well for Bruces's appointment. I look forward to seeing what kind of copper replacement the doctor will suggest.

I think with the cyclone thing, we had so many lives lost and trauma with the floods that the cyclone damage is being treated as minor in comparison.

Everyone I know whether directly affected by the floods or not, feel traumatised by the sheer speed, devastation and horrendous loss of life caused by the flash floods. One of my brothers teaching colleagues and his wife lost their home in the inland tsunami at Grantham. They witnessed their next door neighbours house float past with the family inside. That family died.

My uncle died (at home) of natural causes the night of the flood and the police couldn't retrieve his body for almost 3 days. It's summer here and having a body in the house for that long is not ideal. My elderly aunt is still recovering from being stranded in the house with no phone or electricity and with her dead husband still lying in their bed.

There are floods in the southern states now but we will all recover and in 6 months time Australia will benefit from the full rivers, lakes, dams and artesian basin. Crops will be bountiful and towns renewed and sparkling. (just don't mention the tax increases to pay for it all!!)

We are a lucky country and we thrive on our diversity and unity.

Thanks for the hugs and the same back at you.

[cathybee1]

Your post was horrific and inspiring, Chirley. You are lucky to be an Australian.

I'll let you know how it goes tomorrow.

Here's another hug for luck!

[cathybee1]

Hi Chirley

We went to the Family Doctor yesterday, who told us that Bruce is still experiencing quite a bit of liver inflammation. Though his copper level has come up a bit (still below normal) and his reticulocytes are okay, the ceruloplasmin level has not gone up. So the doctor feels that the ceruloplasmin, which works in the liver, has not been able to access the increasing levels of copper -- meanwhile the bone marrow is working as hard as it can but without all the necessary ingredients.

The doctor still does not have the results back from the metabolic testing from 2 weeks ago. His plan is now to consult with a copper specialist (I admit at this point I raised some doubts about there being such a critter ). He is still thinking that IV infusion of copper will be the next step, but hasn't scheduled it.

I expressed some impatience at the length of time it has taken by the Doctors to recognize that Bruce doesn't seem to be metabolizing copper. I still think this Doc is our best option -- he asked at some point whether we wanted to go back to the Specialist in Los Angeles, but the MDS Specialist sort of washed his hands of the copper issue by saying he didn't know anything about it.

So guess it's time to go back to pubmed and see if anything new has rolled in. I personally believe that we won't be able to rule copper deficiency in or out as a cause of the MDS like symptoms until we can get Bruce's levels back into the mid-normal range.

Other news...Bruce is still "holding his own" from the Aranesp injections -- His Hbg has stayed in the low 9's from the Aranesp injections and actually went up a bit from 9.0 last week to 9.3 this week. Last transfusion was in mid November.

That's all for now. We're packing up this week for a few days of skiing (I ski, Bruce watches) next week in eastern Oregon. It seems like we are firmly in this La Nina pattern -- it has been dry and warm the last few weeks, with that forecast continuing, but there should still be enough snow to ski on. Quite different from the weather you have been having.

[Chirley]

Hello,

I just got my last copper result and it has fallen again to 4.8 (13-25) despite increasing the dose to 3mgs twice a day.

I also got my MRI result which shows the same level of demyelination in my cervical cord but increased demyelination in my thoracic cord. The haematologist wasn't happy with these results and got me an urgent appointment with the neurologist who I ended up seeing later that day. The neurologist wasn't concerned and said that there was nothing he could do about the demyelination anyway and that my disability is almost certainly permanent. He did say I will have to go back on the copper injections and I told him that the haematologist offered to arrange IV copper. His response was that IV copper "is, like, a million dollars" (shades of Paris Hilton) and to stop whinging and just give myself the needles.

I discussed this with my haematologist and after a lot of thought I've decided to stop treatment. My haematologist said that he wouldn't think badly of me and that he would still provide any supportive care that I need. My family accepts and understands my decision. So now, there's no point to the weekly blood tests, weekly hospital visits or the neuro rehab that I was going to do.(the neuro said that it wouldn't do any good anyway)

So I'm going to enjoy my respite from all the medical stuff for the first time in many years. I hope I don't get withdrawals.

Bye for now.

Chirley

[cathybee1]

That sounds like quite a discouraging day, Chirley. I'm so so sorry.

A big hug, Catherine

[Greg H]

Chirley,

I am sorry to hear about all that negative news. And I certainly understand your frustration and your decision.

It could be that a good vacation from constantly fighting the disease and fighting to get care is just what you need right now, particularly given your hematologist's willingness to continue supportive care.

Later on, you may decide to pick up the gloves again. Or not. Either way, I know folks here will support your decision and continue to truly value your presence among us.

The best of luck to you.

Greg

[lindy]

Chirley, sorry to hear those news.
But like Greg said, we understand your frustration & decision.
Perhaps you need that break from all the 'treatment'.
Sometimes prayers do get answered. Best wishes.

[mausmish]

Chirley,

I agree with Greg and Lindy. Best of luck to you and please keep us posted here on how you're doing. All of that treatment wears on you after awhile.

Karen

[Chirley]

Copper is now down to 2.1 ceruloplasmin .06

The only effects so far are platelets down to 100 and total white cell count to 2.5 (both had been in normal or nearly normal range). I have also developed new parasthesia in my left arm. Oddly enough my ferritin has gone up 300 in the 2 months since I haven't had a transfusion. I have no idea what would cause that, I thought it should start falling.

Catherine, has Bruce got his results yet? How's his copper level going? Can't help myself, I may have refused treatment but I can't stop visiting this site, I like to see how everyone is doing.

Wish you all the best.

[Susan L]

Hi - Don't feel bad for taking a break from treatment - I have just decided to take a break myself. Tired of fighting insurance etc... Tried ATG, Decogen, Vidaza - and faught about the Gleevec or Revlimid - insurance says I dont need either- I have just decided I want to just wait a couple of months and see how I do. (Havent told the doc yet - we'll see what they say) I wish you the best and I know - I am still interested in how everyone is doing. Take care -