Copper deficiency induced bone marrow failure

[Chirley]

Hi,

I've decided to cease posting in my other thread and to start this one instead because the thread title is more pertinent.

My story so far.

Low WCC noted in 2003 when being treated for severe pneumonia. Later in 2003 found to be anaemic. Received first blood transfusion. Diagnosed with Fe deficiency anaemia. Numerous investigations including BMBs.

Progressive anaemia which became transfusion dependent and BMBs started showing abnormalities along with Fe deficiency. I was told ? Evolving MDS.

WCC started showing severe neutropenia. Finally in early 2010 BMB showed
definite MDS RCMD. Started Vidaza. Repeat BMB after 3 cycles showed increasing blasts. Now, MDS RAEB. 1.

In the meantime I had started experiencing neuropathic pain in my feet and legs and electric shocks in my arms when I bent my neck. This was thought
to be Vidaza related. The neuro symptoms progressed and I started having trouble with balance and eventually had difficulty walking. MRI showed cervical demyelination of the spinal cord. CSF showed increased protein and
the presence of an abnormal protein called 14-3-3. After 2 further cycles of Vidaza it was ceased in case it was the cause and because BMB showed blasts had increased again.

Finally after numerous tests i was found to be copper deficient. By this stage I had cerebellar degeneration and cervical and thoracic demyelination and couldn't walk without assistance.

It has been established that I have a genetic abnormality of the copper transport proteins which prevents absorption of copper from the gut and also
inability of the kidneys to retain copper in the blood. Hence treatment by copper replacement is a chase your tail proposition. No matter how much copper is given intravenously, it will always be excreted.

So far I have had copper replacement in the form of oral copper sulfate (no effect), subcutaneous injections of copper histidine (which did work but were painful and caused aseptic abcesses) and now, ongoing intravenous copper
sulfate.

My bone marrow failure has resolved with just occassional CBC abnormalities and my neurological progression has slowed considerably but I'm not convinced that disease progression has stopped.

Regards

Chirley

[cathybee1]

Chirley, I'm so glad you posted this. This has been such a long journey for you. In reading this, I realized I have yet another question for you. Which copper transport protein is not working properly, and how did they determine it?

[Chirley]

Hi Catherine, that's a good question.

I asked the Prof the same thing. They don't know how many transport proteins there are let alone how they work or what other interactions they have.

That's part of the reason I became a bit down after my visit to him. He said that just because the blood copper levels are normal it doesn't mean the copper is being utilized in all the necessary places. There may be a transport protein missing that stops the copper from being utilized at cellular level.

Then I read an article about a man who had copper deficiency who didn't respond to copper replacement and ended up having brain stem involvement. He became blind and wheelchair bound despite treatment.

There is so little known about copper and it's metabolism that when i read an article like that, i have to accept that it's a possibility that it might happen to me. Fingers crossed it won't, but it is possible.

Regards

[Marlene]

Thanks for posting this in separate thread. It really highlights the complexity of bone marrow diseases. In your case, anemia was really a symptom of the low copper.

[cathybee1]

Ah Chirley, the Professor may be a smart man, but it seems like all he did was to give you bad news! I am glad that you have had such good response to the copper supplementation so far, in terms of resolving the MDS symptoms, and hope the neurological issues continue to improve as well.

The report from Dr. Paquette at UCLA arrived Friday. Neither of the two University hospital pathologists who looked at Bruce's bone marrow slides saw a clear cut case of MDS, however, Dr. Paquette thinks that's what is going on. A diagnosis by exclusion.

Dr. Paquette nixed the copper deficiency as being an important factor contributing to anemia. And the local hematologist talked to a doctor from the Mayo Clinic, who said Bruce didn't fit the profile for someone with copper deficiency anemia.

As the IV supplementation has not normalized Bruce's copper or ceruloplasmin levels, Bruce is leaning towards stopping the supplementation. He is going to continue transfusions, and do a trial of Exjade.

[Chirley]

Hi Catherine, I understand Bruces' decision to stop the copper supplementation.

I was also told that my BMB and blood results weren't consistent with copper deficiency but my blood tests did definitely get better with copper replacement. I suspected at the time that the Vidaza had started to work and that's why my blood results had improved and I put this to my hematologist. He just said that if it had been a Vidaza response I would have relapsed once the Vidaza was ceased. I still have doubts about whether it is the copper helping or the result of Vidaza but I have to go with the hematologist and defer to his knowledge.

The same article I saw about the man who went blind also mentioned that untreated copper deficiency can progress to leukemia and certainly my blast level was increasing.

I have to admit to considering refusing any more copper treatment and just having transfusions when needed. it's just a matter of quality of life versus length of life. I have started to hate my visits to daycare for copper. At least with transfusions it would only be one visit every two weeks or so and there is no evidence to show that copper replacement will stop neurological deterioration let alone improve the damage already done.

The daycare I go to for treatment are having Christmas week off and only having minimal patients the following week, so my treatment has been pushed back a couple of weeks anyway and to try and get my blood levels up again I'll have to have daily visits for a couple of weeks and , frankly, I'm soooo over them. I'm sure my doctor thinks I asked to have more time off and doesn't realize that they just want to have less staff on over the holiday period. Anyway, I'll think of it as my opportunity to have a nice long think about my treatment options.

Regards

[cathybee1]

Chirley, I do understand why you desperately need a break from the daycare. The hassle of your situation must be exhausting. Bruce is in a little bit better situation in terms of being able to get most of his copper treatments at home...the downside has been very high out of pocket expenses for supplies and IV supplements. The doctor wrote a letter for our insurance, but so far, none of the costs have been reimbursed.

Bruce also wants to see if his copper levels will drop much without the supplementation...since they haven't risen noticeably with the IV copper, will the reverse be true?

In his report, Dr. Paquette offered Revlimid and Vidaza as treatment possibilities. But since Bruce is already so neutropenic (and platelets are getting low as well), Bruce didn't want to deal with possible side effects from those drugs now.

Bruce's hematologist called today. He doesn't want to start Bruce on Exjade immediately because some of Bruce's liver numbers aren't so great. So, the liver biopsy is back on the front burner again, so the doctors can see how much damage from iron has actually occurred.

[Chirley]

Hi,

Well, I did it. I have cancelled my daycare appointments. It's been 4weeks since I've had IV Copper and the last time I was without parental copper I relapsed into bone marrow failure within 10 weeks. So the next 6weeks or so will be interesting to say the least.

I have noticed my neuropathic pain in my feet has worsened and I have increased spasticity and stiffness in my legs but I expected my neuro problems to get worse so maybe it's psychological.

Catherine, hope you and Bruce had a lovely Christmas and New Year. Mine was a little sad because my Dads only remaining brother passed away. My uncle died in Sweden and Dad felt so useless being on the other side of the world and not being able to be there for him when he was so sick. My Dad is acting like nothing happened but I can read him like a book and I know he's hurting. I brought out an old photo album and sat down with Dad and asked him questions about the photos and he was soon talking about his brothers and sisters and telling funny stories from his childhood. I hope it helped him to talk about it just a little.

I was wondering how Bruce is going without his copper treatments. Is he
requiring more transfusions now? is he going to start treatment with the Revlimid or Vidaza?

We are just about to get a storm so I had better go and bring my dogs in..they are both very well..I'm very blessed.

Regards

[cathybee1]

Sorry I haven't been on for a while...stuff has been happening here.

First, Chirley, I am so so sorry about your uncle. I'm glad you could be there for your dad. I hope that this time away from daycare will be restorative for you psychologically and that you can enjoy the summer with your pups. I so wish that there were some better choices for you.

Bruce decided to not try the suggested pharmaceuticals. The odds the specialist in L.A. gave for Revlimid or Vidaza working were pretty low, and the immunosuppression side effects of both drugs pretty daunting. So Bruce wants to continue with transfusions.

Bruce had his liver biopsy 5 days ago in a hospital (with conscious sedation thank goodness). The biopsy is being evaluated for both iron and copper. The hematologist called today to say that preliminary results of the iron testing indicate that Bruce has moderate fibrosis in the liver which the hematologist said were due to iron deposition. I wasn't here when Bruce got the call. Bruce told the hematologist he wants to go ahead with a trial of Exjade as soon as all the insurance paperwork allows.

The good news we got today is that Bruce had bloodwork in hospital before the liver biopsy at which time his hgb was at 9.1. Today, he had another blood draw, and his hgb was at 9.4. Different labs but it has been a month since the last transfusion, and it was a pleasant surprise that his hgb levels are still holding. The other pleasant surprise was that the MCV levels are still near normal.

Bruce has had no IV copper since before Christmas. The blood draw today was the first testing for copper done in a month, and we won't know where those levels are at for another week.

For now, Bruce continues to feel well, and yes, we are grateful for our blessings too.

Hugs to you

[Chirley]

Hi, I have just returned from a visit with the Prof of Metabolic Medicine.

I can't say I was looking forward to this visit because I found him quite rude and abrupt last time. Well, he was different today. Whew.

I was given the results of my chromosome studies from last visit and I've been found to have del 15q which oddly enough has no prior relationship to either MDS or copper deficiency. So now they are scratching their heads yet again. Apparently he thinks I also have some portions of the same chromosome that while still present, have been switched off.

Del 15q is associated with Prader Willi Syndrome but I have none of the physical or behavioral features (except insatiable hunger, but doesn't everyone). So now, I have ? MDS, copper deficiency bone marrow failure and myelopathy and Prader Willi.....seems like the more you go to doctors the
more they find.

As a result of these findings they are now adding copper testing to the investigations they do on children with suspected Prader Willi, so hopefully my situation might produce some good.

Ferritin remains at 1600 something. I told him that I had decided to discontinue copper replacement and he was predictably not very happy with my decision. He thought I would decline neurologically quite quickly and then he "will be left to pick up the pieces". I told him that as a female I reserved the right to change my mind if or when I couldn't tolerate the symptoms any more. He said it was part and parcel of Prader Willi to be non compliant and that's why I won't do everything they tell me to do. Sometimes instead of telling me to do something it would be nicer if they asked but hey, what would I know, I'm only the patient.


There is a doctor coming here from England in a few months time who specializes in Prader Willi in adults and he wants me to see him, so that could be interesting.

I have guessed the spelling of Prader Willi, that's how little I know about it so I suppose it's Google time again. Life is one long lesson isn't it?

Regards

[Birgitta-A]

Hi Chirley,
Yes, your disease is really getting more and more complicated :confused:. Hope it will be OK to discontinue copper replacement at least for some time!
Kind regards
Birgitta-A

[cathybee1]

Chirley:

Yes, all this does complicate things a little. And now if you don't want to do something a doctor thinks you should do and/or you're hungry, it's because you have a chromosome abnormality? Wow!

Bruce is waiting for the Exjade to arrive (supposedly it's coming in the mail). We still don't have the results of the copper test done a week ago, but that's not unusual. So, no news here, except it's VERY cold.

Hugs

[Chirley]

Hi Birgitta. Yes, it's quite baffling and I think it's creating some curiosity/excitement for the Prof. On my part I feel quite detached from it all now. It's as if it's happening to someone else and I'm a slightly bemused bystander.

Catherine, that's exactly what I said to the Prof....I can do and eat anything I like and blame it on my genes ;). I said it as a joke but I don't think he knows what that is so I didn't get even a smile, oh well, I tried to lighten the mood.

My parents are both in their eighties and the Prof has given blood test requests for them to both have chromosome analysis and copper levels. They have both agreed but I think they are puzzled by the whole thing.

Ahh, that medical journal article the neuro is writing about me is getting more interesting with each test I have. Maybe I will ask for royalties!

Is Bruces' Hb still holding? No transfusion lately?

It's very cool here considering it's summer. I still have a duvet on the bed at night, and it's raining day and night. Still it's nice to snuggle into bed on a cool rainy night isn't it?

Regards

[Lbrown]

I hope your poor baby professor doesn't strain himself picking up the pieces!

:p

Honestly. Some people!

Deb

[Chirley]

I'm confused. Not hard to achieve but still....

My neurologist rang me this afternoon and told me that they don't know what the deletions on long arm 15 mean. This is different to what the Prof told me but I agree with my neurologist that I don't fit the Prader Willi profile so the deletion is likely to be more distal. So I Googled.....big mistake....

Del 15q interstitial deletions have been associated with poor prognosis MDS and post MDS AML.

So now what do I think? The Prof is going away for 3 months, the neuro is clueless about MDS and I REALLY didn't want to go back to the hematologist just yet because I was declining further treatment.

Denial and procrastination sounds good.

Regards

[Birgitta-A]

Hi Chirley,
You know as long as nobody knows if you have MDS or if your symptoms depend on lifelong copper deficit you shouldn't worry about what del 15q means to MDS patients.
Kind regards
Birgitta-A

[Lbrown]

I agree with Birgitta. And I also think just because they find some association between this and that doesn't mean one causes the other. You should be in charge of your treatment, which means it is your right to refuse treatment.

Deb

[cathybee1]

I'm also with Birgitta on the del 15q.

Bruce will probably go in for another blood test next week. It is weird, but I am getting able to tell from the sound of his voice whether it is transfusion time. He has had a few experiences like this over the course of his anemia. Plateaus and then ka-boom, the numbers drop big time. But so far, so good. :)

How are YOU feeling??

Hugs

[cathybee1]

How are you doing, Chirley? Inquiring minds want to know...

[Chirley]

Hi,

Thanks for asking. I'm still feeling okay.

I think I'm becoming anaemic because I'm pale again (I actually like being a little pale....makes me look younger). I'm also a little breathless at times and I have a mild chronic headache which are all my indicators that I'm due for a blood transfusion.

If I'm right and I do need a transfusion, it will have been a disappointingly fast decline. Its been just over 7 weeks since I had my last copper treatment and almost 9 weeks since my last blood test. The last test showed Hb well and truly in normal range and copper just below normal range. My blood levels aren't affected until the copper is pretty much near zero.

Neurologically I am having a very slow but steady deterioration. Walking is such hard work and dangerous because I'm so unsteady that I could easily fall that I spend most of my day sitting and reading or being on the net. I keep putting off getting a wheelchair because there is some kind of psychological barrier that I'm avoiding even to try and understand. My GP says it's denial, I say it's optimism. I'll deal with it when I have to.

I have an appointment on Thursday to have my port flushed and also to see the hematologist. I want to discuss the chromosomal abnormality with him. I've been thinking about it and realized it must be new damage because I certainly wasn't born with the syndrome caused by this particular deletion and because it was not found on the 10 or so BMBs I've had done.

I rang Celgene and spoke to a patient liaison person who said there have been no reports of this deletion being caused by Vidaza but that they were going to document it as an adverse event. I gave them permission to contact my doctor about it but only after I see him on Thursday because he has not been told of this finding yet.

My little dogs went for their yearly vaccinations and check up this morning and both were given a clean bill of health. The vet said that my old (13) white dog is doing incredibly well considering she has had Addisons Disease for 9 years and had an obstructing liver tumor that almost made her bleed out about 4 years ago. The liver tumor disappeared without treatment and the vet said he had never seen that before and he considered it a miracle. The arthritis medicine is working very well for her and she's a playful old girl again.

I'm very lucky.

Going to cook my dogs sausage and mashed potato for dinner as a treat after the needles.

Regards

[cathybee1]

Thanks, Chirley, for the update. I am very glad to hear from you and to hear you have an appt with your hematologist. I am very sorry about the neurological issues.

I am so so happy about your pups check up!!

I heard you are having a very hot summer.

Bruce is feeling much better since the last transfusion. His copper results finally came back from early January. It was at 61 (-10 from normal) -- it has been lower, so for now he is taking an oral copper sulfate supplement and calling it good. And he is still tolerating the Exjade.

Hugs to you,

[Chirley]

Hi,

I've just returned from my haem appt. So much for being tired and headachy....my blood test was very nearly normal. Hb 132!! WCC 4 neuts 2.2 and platelets 157. The only abnormality was my usual low lymphocytes.

I also had a copper level taken but I won't get results until next Tuesday.

I am over the moon that I have not had copper replacement in 8 weeks and my CBC is so good. My doctor is extremely surprised, to say the least, but doctors being what they are is a little more reserved. He said that if the chromosome deletion is new it is an indicator of MDS after all. He even mentioned the dreaded transplant word. Previous BMBs wouldn't have shown this deletion because it was a micro assay and these aren't done on BMBs normally so there is nothing to compare it against. We just have to wait and
see if my parents tests show the same abnormality we won't get those results for at least three months when the Prof is back in the country.

I'm assuming if it is MDS after all, then the 5 cycles of Vidaza that I had were incredibly effective despite my blast count increasing when I was having it.

It also turns out that he didn't notice that I'd stopped having treatment and the daycare centre didn't tell him that I'd cancelled so he was a bit perturbed about that but I told him that my actions were vindicated by the blood results.
He had to agree ;). He has asked me to see him again the next time I have my port flushed in 2 months but if I have any problems in the meantime or even if I just change my mind about the copper treatment he wants me to ring him. He also told me that he supports patients in their treatment decisions and he will continue to be there for me no matter what I decide to
do.

Next visit is to the neurologist who won't be quite so accommodating. Oh well, can't have it all.

Regards

[cathybee1]

Lots of good news here. I would be so over the moon too -- yay! I hope your CBC levels continue to hold, perhaps that might indicate a longer lasting effect of copper supplementation?

I'll keep my fingers crossed your visit to the neurologist goes better than expected too.

Hugs.

[Lbrown]

Awesome CBC! Do you feel a little less tired now?

I find sometimes I feel like my counts have to be in the toilet judging from how tired I am, and then I find out they're not so low after all. It usually perks me up. I guess it shows some of it is the mental strain.

Hope the results stay that great and your doctor is right about it being your decision as to what treatment to do.

Deb

[Chirley]

Hi, I almost can't believe my results. I'm definitely pale and I am headachey, tired ++ and just the last day or two starting to get breathless so when I found out yesterday that my CBC was good, I thought how much of the physical symptoms can be influenced by the mind.

However, today i still feel the same although I know I shouldn't. I hope it's just temporary because we are finally having some typical summer weather. Hot, humid, daily rain showers etc basically ugh. I was warned by the neurologist not to get hot (just like people with MS) so maybe that's got something to do with the way I'm feeling. I have an air conditioned house but sometimes you have to go out. At least, that's what my dogs tell me :).

It's my Dads birthday today, so Mum and I are going out for lunch. Dad asked me to do this for him as a present because Mum has been in a bad mood for a couple of weeks and he wants a break, he thinks it might also cheer her up. I love my Mum very much but I have to admit she's a highly volatile, bad tempered, opinionated little person. She's 80 and Dad still finds that cute and amusing, life with her is not boring! They have been married nearly 63 years. I arranged for them to get a letter from The Queen for their 60th anniversary and surprisingly, they got one from the (then) Prime Minister and his wife as well.

Regards

[Lbrown]

Apparently people with sarcoidosis have to stay out of the sun.

Well, you still need to take care of yourself despite the counts. Have a good lunch with your mum!

Deb

[riccd2001]

just wondering... what units are you using for Hgb? AFAIK for the SI system of measurement, the normal range units for Hgb are grams per litre(M 81 to 112; F 74 to 99). Your CBC result of 132 seems to be really high; did doc say anything about that?:cool:

[Chirley]

Hi, my results say g/l with a normal range of 115 to 165. It doesn't give male and female normal values probably because I was already known to be female.

It's definitely in the middle range of normal but this has happened once before and three weeks later my Hb was 70. I just have to wait and see and hope that my counts stay good. I just wish I felt as good as my tests look.

Maybe the discrepancy in normal values explains why when people say they aren't transfused until they are 70 or 75 I always think that it can't be very good for them long term to let themselves become that anaemic on a regular basis.

Regards

[riccd2001]

I was looking at the the wrong "cut/paste reference..." :o

For sure, normal hemoglobin levels are different in women and in men. In women, normal hemoglobin ranges from 120 g/L to 160 g/L (12.0 g/dL to 16.0 g/dL). In men, normal hemoglobin levels range from 140 g/L to 180 g/L (14.0 g/dL to 18.0 g/dL). Usually the mark for transfusion in Ontario is 90 g/L for all :cool:

[Chirley]

G'morning.

I just received a phone call from the hematologists secretary with last Thursdays copper level. It was 3.8 which when I work it out using the US reference numbers, equates to 21ish. The ceruloplasmin was 0.09 (ouch) and Fe has come down to 1186. Originally the secretary said the Fe was 86 and I just about fainted with happiness but she rechecked and found she'd made a mistake. Oh well, 1186 beats >5000 any day.

This isn't a BM issue but oh dear, my neighborhood is going downhill and I really want to move. There has been 3 people shot in my neighborhood in the last week, one of them was less than 100meters away. Then yesterday a car lost control and smashed into a house only 3 houses away from mine. The house almost collapsed and the little girl in the bedroom had only just been taken to the lounge by her Grandmother. So lucky! The driver of the car was arrested with guns, drugs and proceeds of crime money. This used to be a family neighborhood but the Government has bought a lot of the houses and moved dysfunctional people into them. The average value of the houses the Government is supplying to these people would be $ 400,000 each. Now they look like rubbish tips with car bodies on blocks in their front yards, rubbish everywhere etc. I probably couldn't sell my house now because no one would want it.

Well, that feels better now that I've had a whinge about our ludicrous State Government and it's policies.

Regards

[cathybee1]

I love the little insight into your parents...and I hope your day with them was a fun one.

[cathybee1]

Gosh, looks like I missed all yesterday's posts when I posted last. Terrible news about your neighborhood, Chirley. My mom lived in a neighborhood that transitioned like that, into one where drugs and shootings were more common, and yes, government subsidised housing did play a part in that transition. Hers eventually transitioned back but it took a number of years.

ouch about the copper level and ceruloplasmin. But very very good news about the Ferritin.

Bruce goes back to the hematologist tomorrow. Hopefully we'll find out how his kidney and liver numbers are doing since he's been on the Exjade.

[Chirley]

Hi, nothing much to report.

I have just returned from a visit to my GP. I got the usual lecture about restarting the copper infusions. He ordered a blood test and I couldn't see the point of having it done but in the end curiosity got the better of me and I did have it.

Physically, I have become weaker. I had a fall not long ago and couldn't get up by myself....that wasn't fun. I can walk only very short distances and with a lot of effort. I have bought a mobility scooter which will be delivered tomorrow. My voice is weak and hoarse and I'm incredibly tired. I'm not sure but I think I might be having small siezures, just sometimes I realize that I haven't been aware of what's happening around me. This is almost always preceded by flashing lights and a partial loss of vision.

My old dog became ill during the week and I felt that I couldn't give her the care that she needed so I had to have her euthanized. I know I did the right thing even though it hurts emotionally and I miss her so very much.

I expected to be lectured by my family for refusing treatment and having to watch me become weaker but they have been wonderfully supportive. It must be hard for my parents and I do feel guilt for putting them through this.

I'm not sure if I have any bone marrow failure, I think the tiredness, breathlessness and pallor can probably all be attributed to the copper deficiency rather than anaemia but I suppose I'll find out when I get the blood test results.

If I get to the stage where I feel absolutely awful, I reserve the right to change my mind about having more copper infusions. My hematologist has found a treatment centre a bit closer to where I live that is willing to give me the copper if I change my mind. That would make life a lot easier. The neurologist, on the other hand, told me that while I refuse the copper he won't help me. Not much he can do anyway I suppose. No great loss.

Regards

[Birgitta-A]

Hi Chirley,
How sad that you have become weaker and that your old dog had to be euthanized. Very good with the mobility scooter!

It is always difficult to decide about treatment when treatment means lower quality of life and the results of the treatment isn't too impressive. Most of us will have to face this kind of problems sooner or later.
Kind regards
Birgitta-A

[cathybee1]

Hi Chirley. I'm very very sorry about your dog. The continued weakness and fatigue must be frightening.

You do have difficult choices to face, and I'm glad your family is behind you.

It's very very good that your hematologist is trying to give you a more attractive option if you elect to go back to treatment.

Bruce just got back from another blood test. He did have his copper tested last week for the first time in a month but no results back. He thought last week it was transfusion time, but his numbers were still a little higher than expected. He upped his dose of Exjade a little bit, and is still tolerating it well.

I'm sending big hugs your way. Losing a dog is very very hard.

[mausmish]

Chirley, very sorry about your dog. It's always so hard losing our beloved pets, all the more so when you're not feeling well yourself. Hugs and good luck to you whatever you decide about the treatments. Karen

[Chirley]

Hi,

It's been almost 12 weeks since I had a copper infusion and my Hb remains good at 128. My WCC has fallen to 2.1 and neuts are 0.8. My liver enzymes have worsened slightly (he didn't give me numbers).

I'm happy with my results but it shows that the copper deficiency is starting to impact my bone marrow. Last time my counts dropped dramatically once they started being effected. I just have to wait and see.

Copper level isn't back yet.

I'm not sure but I think I might have something wrong. I fell asleep in my lounge chair yesterday afternoon and I woke up shivering with cold. It's summer here and the temperature was around 32 degrees yesterday, so I know that shivering is distinctly abnormal.

My mobility scooter arrived yesterday but I was too tired to do much with it. My remaining little dog (Gidget) hopped on and we drove around the yard a few times. She seemed to enjoy it. Maybe today I can go for a ride to the local park.

Regards

[Birgitta-A]

Hi Chirley,
Good that your Hgb is quite high! You have managed with very low neutrophils before so 0.8 is not so low for you - the shivering hopefully didn't depend on fever.

Fine that you and Gidget liked the scooter. It must be perfect for excursions and shopping.
Kind regards
Birgitta-A

[cathybee1]

Hi Chirley,

That is a good Hgb number, as Bruce's hematologist would say, you are still "holding your own." (I never know exactly how to take that one, to be honest.)

I hope the shivering is temporary. Please keep us posted.

And I am glad your scooter arrived. Gidget will love a walk to the park.

You are baking in the heat and we got about a foot of snow today here! My dog Gus went nuts, buried his head in it, and moved forward, his head being a kind of snowplow! I guess he's missed playing in it after the dry winter.

Bruce is going for his transfusion tomorrow, he put it off for almost a week, but is now experiencing the pounding in his head that's a sure sign he needs a couple of liters.

He got his copper results yesterday. He is at 59 now (normal is at 70).

Hugs, Catherine.

[Chirley]

Hi,

After a lot of trouble, I finally got my copper level back today it's down to 0.9 (13-25) and that would have been a week ago.

I worked out that if I multiply my results by 6 it brings it into the same type of measurement that is used in the US. That means that my result would be something like 5.4 And no, I don't mean 54, it really would be 5.4.

Because my GP Is on sick leave again, I spoke to another doctor to get these results and he wanted me to go straight to hospital :confused:

I'm due to see my hematologist and have another port flush in just under 4 weeks but I can tell that my Hb has started dropping and once that happens it drops really fast. Last time it dropped over 50 in 18 days. A sudden drop like that is harder for the body to adjust to than a chronically low Hb.

Oh well, looks like I'm about to start the treatment centre round-about again. I have enjoyed the respite.

Regards

[Birgitta-A]

Hi Chirley,
Yes, as you wrote you have better start treatment with copper again - too bad but your quality of life will be very low if your HGB is falling.

Now you have tried to stop copper and unfortunately it didn't work. We can't do more than examine the options and follow the results of our own "trial".
Kind regards
Birgitta-A

[Chirley]

My oh my. Now I know what drama is.

I was on my way to have a blood test on Wednesday when I felt like I was going to faint so I sat on my walker and someone pushed me into the pathology clinic. I just got there when I collapsed. When I woke up I had vomited all over the place and been incontinent of bladder and bowels. Talk about being horrified! Well, I kept vomiting and passing in and out of consciousness and the pathology ladies called my GP and he came and took my blood pressure but he couldn't get one. So they called an ambulance and took me to hospital with lights and sirens.

At the hospital they thought I has septic shock because my blood pressure was still really low and my neuts were low(they didn't say numbers) so they had the resus team and the ICU team with me. After a lot of investigations they decided to admit me to ICU but didn't have a sterile room there so they transferred me this hospital with a medical escort. Here I was admitted to a neuro ward with 3 other people ??

After lots more tests it has been determined that they don't think I have an infection but my neurological problem has progressed and effecting the part of the brain which controls blood pressure called autonomic something or other. However after being here three days they are now thinking I need to go into an isolation room, sometimes doctors and hospitals are hard to comprehend!

Anyway I have been very well looked after and I see my hematologist every day and my neurologist twice a day. I'm on a continuous potassium infusion and have started copper infusions as well. My blood pressure is better and sits around 80/35 although sometimes it drops lower. Apparently I now have C3 myelopathy which is at a level where the diaphragm and breathing can be effected but so far, so good.

I had another MRI which incidentally mentions that I have no bone marrow. The hematologist says that he thought that should have improved with the copper that I had last year, so I think I might be having another BMB in the future. My last bone marrow said 10 to 30 percent cellularity and because my numbers came really normal we thought that must have improved.

The neurologist said that this autonomic what ever thingy might be permanent and I might be in big trouble so I'm keeping my fingers crossed that it improves.

I apologize if I'm not making a lot of sense because I have also been a little bit confused the last couple of days and forget things really fast.

Regards

[Marlene]

Wow Chirley!!!!! That's very extreme. I'm glad you were in right place when you fainted and that they could respond so quickly.

Hopefully the automonic nervous system will stabilize with the copper. Sounds like you are good hands.


Hang in there....

[Neil Cuadra]

What an ordeal, Chirley. I'm surprised you are able to recount it in such detail. You must have been very dehydrated when you first got to the hospital so I'm glad they tended to you promptly. We have our fingers crossed for you too.

[Birgitta-A]

Hi Chirley,
Too bad with the new symptoms!

I really don't think they can study the bone marrow in detail with MRI - BMB is of cause much better.

Hope your blood pressure will stay normal after treatment with potassium and copper! When potassium is low the heart can beat very slowly and this can cause fainting.
Kind regards
Birgitta-A

[cathybee1]

Oh, Chirley, what a frightening experience. I am glad they were able to treat you immediately and that you are feeling better. Everything IS connected to everything else, isn't it?

Let's hope that the neurologist is wrong and that this is a temporary situation.

Hugs!

[Chirley]

G'day, I'm feeling better already. The potassium has been ceased and I ve been transferred to the oncology ward.

My blood pressure is responding and is consistently at 100/45 ish now. My Hb has dropped to 80 so I'm about to start my first blood transfusion of 1 unit in a year.

I have quite bad bone pain but I'm looking on that as a good thing because it means the bone marrow is producing cells :D

I'm still getting some visual loss and still can't walk without a lot of assistance but the physio took me for a little walk around the bed and I felt very satisfied that I managed that.

I don't know what I can do to atone my GP who I managed to vomit on (amongst other things :o). I think a nice bottle of wine might be the thing.

Better go, my copper is infusing with my saline IV so the blood is going to go into an IV in my arm and it will be out of action for a couple of hours.

Regards

[Birgitta-A]

Hi Chirley,
Good that you are feeling better! Not so positive that your HGB has decreased. Hope you will continue to improve!
Kind regards
Birgitta-A

[cathybee1]

Thanks for the update, Chirley. I've been thinking about you. I'm so glad your blood pressure has gone back up.

I doubt this is the first time someone has vomited on a doctor....but yes, wine is definitely a good consolation prize.

Hugs

[Chirley]

Turns out I have tested positive for MRSA. Odd, I have only had a very low grade temperature once.

I'm finally going to get my own room :o.

My poor room mate has only just got over being unconscious and fitting from an infection (neutropenia due to chemo for advanced non hodgkins lymphoma) and now we've shared a room for 3days. I feel very bad for her, but nothing I can do about it.

I'm waiting for my doctor to come and see me. Infection control practioner has already been and done the whole education thing.

I can only have got this on my last visit to this hospital a few weeks ago. I'm not very happy.

Regards

[Birgitta-A]

Hi Chirley,
Too bad with MRSA after all other negative issues! Do you know where you have the infection?
Kind regards
Birgitta-A

[Chirley]

Hi,

At first I was told I was colonized with MRSA from a nasal swab but now that I have developed fever I think they are suspecting an actual infection somewhere too.

Is it possible to have an infection without fever due to low neutrophils? It seems as soon as my neutrophils rose from 0.28 to 0.9 I started getting a feVer. I am assuming, I now have something to fight an infection with so that's why I'm only getting the fever now. Does that sound feasable?

Regards

[Lifeguard]

Chirley, I'm so sorry about your frightening turn :(. I hope they find out what type of infection you have. I'm glad you’re being well cared for and that you have your own room. I don't know if you can, but there is a MDS telephone forum on tonight hosted by the new Leukaemia Foundations MDS Coordinator. I posted a reply to you on the Talk Blood Cancer forum, but with the new site it seems my 2012 MDS posts have not been reposted. If I don't speak with you tonight, I'm sure we will catch up soon.

Rachael

[Birgitta-A]

Hi Chirley,
As far as I understand low neutrophils only means that we can't fight infections as well as if we have a neutrophil count of 1.5 or higher. Then we know that your neutrophils have been very effective and you have managed with very low counts.

Your neutrophils perhaps increased at once when you got the MRSA infection and tried to defeat the infection. Your body eventually reacted a little later and you got fever from the ordinary cytokines that give fever when we are infected.

Hope they will find an effective drug for the MRSA infection!
Kind regards
Birgitta-A

[Chirley]

Thanks for that Birgitta. I thought there might have been a reason why I didn't have much of a fever at the beginning.

The fever has gone again but they forgot to do a blood test on me yesterday so I don't know how my counts are.

I had the worst pain I have ever experienced last night. My legs started tightening up around 9 am yesterday and just got worse over the course of the day. My feet were pointing at odd angles. The physiotherapist couldn't get my knees to bend. Everything from my hips down just froze solid. That was painful but then I got neuropathic pain too and the pain got so bad I was screaming despite having Endone and parecetamol. My hematologist came in from home and ordered 10mgs morphine, tropisitron and two temazapam and I had 2 hours of relief before it started again. So I had the morphine repeated and it's 8hours later and the pain hasn't come back.

Im not claiming to have a high pain tolerance, I feel pain as much as anyone else but I'm not particularly whimpy either and the pain I had last night was so bad I would describe it as intolerable.

The nurses were beautiful, caring and helpful. My doctor was wonderful for coming in and I am very grateful for all the support I got last night.

I'm on the verge of tears right now with gratitude to the people looking after me.

Regards

[Chirley]

My hematologist came in a couple of hours ago and told me that my counts have crashed despite the copper treatments I've been having for the last week. He said he thinks the MDS has reared it's ugly head again.

He is going away for a week and another doctor will be looking after me but I need another BMB and possibly another lumbar puncture sometime soon.

Personally, I think they have done the test on someone else and not me. I was supposed to have had that blood test taken out of my port line at 4.30 this morning but I have no memory of it a all. Admittedly, I was heavily drugged, so maybe they did it and I didn't realize.

Regards

[Birgitta-A]

Hi Chirley,
How terrible with painful musclecramp and neuropathic pain! If they should return you now know that the combination of morphine and temazapam, that relaxes the muscles, is effective.

Too bad with the low counts - we can't expect that the counts should be OK when you have the MRSA infection and have had fever. I still don't think you have MDS but copper defiency your whole life. Hopefully the counts will increase when the infection is treated and you get copper.

You are a true hero!
Kind regards
Birgitta-A

[Birgitta-A]

Hi Chirley,
Low magnesium or potassium can cause muscle cramps.
Kind regards
Birgitta-A

[cathybee1]

Chirley, I'm praying for them to figure this out and for you to feel better quickly.

Hugs.

[Chirley]

I've just been discharged after three weeks in hospital and I feel lucky to have survived two different severe episodes.

I still haven't seen my haematologist since the time he told me that it looks like I do have MDS after all. I was just given a copy of some of my blood work and I saw that I had increased metas and myelos on my peripheral blood tests. Would I be right in assuming that these are blast cells?

I feel well enough in myself but I am now quite disabled. I am now wheelchair bound and will be receiving in home help. I'm very grateful to be home at all because my neurologist told me that I needed to be placed in a nursing home. At that stage I was in a permanent state of rigidity and couldn't move either my arms or legs. It was extremely painful, not to mention devastating to be so dependent and useless. I very much wanted to die while I was in that condition. Apparently my myelopathy has now extended to C3 from where it used to be at T2.

It took some time but my electrolyte imbalance, acid/base problem, kidney failure, liver failure, protein deficiency etc etc etc. have all improved if not totally resolved. My CBC doesn't look too bad either.

I still have problems maintaining my blood pressure and I take Fludrocortisone to help keep the systolic stay in the low 90s.

I have been having almost daily copper infusions which take 4hours each on average and I have been having hydrocortisone with each infusion so I think that's helped with the blood pressure too.

One good thing (I think) is that I have lost 3 kilograms in the last week alone. When I'm able, I'll try to stand on my scales and see how much weight I've lost all together.

This is my first day home, I've already had a two hour afternoon nap and I need to go to bed again. I think all the muscle relaxers I have to take to stop the spasms and rigidity are making me extremely tired but better tired than totally paralyzed :eek:

Regards

[Neil Cuadra]

Chirley,

I'm glad you are home, have help, and have reached a mostly stable state. Being unable to move must have been awful.

Be careful with the scale. This is not a good time to fall, after all that recovery progress!

[Birgitta-A]

Hi Chirley,
Very good that you are at home again!

Myelocytes are more mature than blast cells and metamyelocytes are more mature than myelocytes - both these types of WBCs can be seen in blood but it is not common.

Too bad with the painful cramps! Good that the muscle relaxers have effect!

Hope you will manage well at home though you have these new symptoms!
Kind regards
Birgitta-A

[cathybee1]

Chirley, what a terrible few weeks. It must be so wonderful to be back home, and know that you're able to move again. Please take it easy for a while.

Hugs.

[mausmish]

Welcome home!

[Marlene]

Glad you're home. I'm sure you'll recover faster at home than in the hospital.

[cathybee1]

Thinking about you, Chirley.

Hugs

[Chirley]

Hi, it turns out that when I opened my pack of discharge meds, there was no Fludrocortisone. I didn't know if iwas meant to continue and they forgot to give them or they had been ceased. I rang the hospital and didn't get any useful information. My hematologist was away and my GP was on leave so I just decided that if I didn't have any, I couldn't take them so no use worrying.

Over the course of the last week I have become so debilitated that I can't stand up to transfer from the bed to the chair etc, without feeling lightheaded, nauseous, breathless and sweaty. My hematologist came back today so I rang him and he immediately faxed a prescription for Fludrocortisone to my local pharmacy. Turns out I was supposed to stay on them to maintain my blood pressure. Maybe I'll start feeling better.

My Dad has a digital blood pressure machine, he took his BP standing up and it showed 90/68 then we tried mine while I was lying down. We tried three times but it just kept reading error. We assumed it was too low to register.

I have managed to weigh myself, I've lost 3.8 kilograms in the last week alone and although I can do with losing weight, I'm not trying to at the moment and that seems like a lot of weight to lose in a short time.

On the upside, I've managed to have my first haircut in 5 months, absolute bliss! My wheelchair ramps have been installed, my bathroom has been adapted and I'm learning to steer my wheelchair, although my cat is paranoid about her tail :D don't know why.

I had to hire a wheelchair because the one I bought on eBay didn't arrive.
When I contacted them, they said that the courier didn't pick it up and theyhadnt noticed and now it won't arrive until well after Easter. Needless to say I wasn't (aren't) happy.

I'm booked to go to daycare Tues through Fri next week and Mon the following week for copper infusions. At least this treatment centre is only 35 kilometers away and my commute should be ok. For the benefit of people who don't know Brisbane, the traffic congestion in this city is horrendous. It used to take me one and a quarter to one and a half hours to drive 32 kilometers to work and the same for the return journey. Peak hour starts from 6 in the morning and lasts until 9 to 9.30. Public transport like train and bus is amongst the most expensive in the world.

Afternoon nap time, so I'll say goodbye.

Regards

Chirley

[cathybee1]

I'm so glad you're back on your prescription now, you're getting set up for your wheelchair, and that Daycare commuting will be more manageable now! Your cat will adapt to your new vehicle, they are wonderful that way. Of course, they are Drama all the way, getting there.

Hugs!

[Chirley]

Hi,

I was reading back on my posts and realized that I didn't make it clear that my muscle problems are all neuro related.

The neurologist said I have generalized dystonia, hypertonicity and spasticity. He also said it was all my fault for refusing treatment, at this stage he doesn't know if it's permanent but he suspects it is. He is very blunt and ruthless not to mention pessimistic.

I've heard there is some kind of port that can be implanted into the spinal cord that can deliver drugs to ease spasticity and I'm going to raise this with him next time I see him. Botox can be injected into individual muscles but my problem is too generalized for that to be of any help.

Regards

Chirley

[Birgitta-A]

Hi Chirley,
What a terrible neurologist you have! How can a doctor be blunt, ruthless and pessimistic when he has a patient with severe symptoms like you have? He should follow the old rule: to cure sometimes, to comfort always, to hurt the least, to harm never.

Good that you manage to continue to fight! Peaceful Easter!
Kind regards
Birgitta-A

[Gosia.P]

Hello Chirley,
I have been reading your blog and I'm so impressed how strong you are. You are incredible in your extremely 'adventurous' journey, looking forward to join you through this forum.
My name is Gosia (47) mother of 3 boys - Jake 26; and twins : Adam and Danny ( turning 11 on 4/17) *live in Indianapolis IN.*
It's been 6 years since my initial SAA (4/4/2006) diagnosis. Was treated with h ATG and cyclosporin, *6 months of post hATG transfusions and after all - great recover and in the remission until January of 2012. Received second hATG during 'leap week' 2/27 -3/2, now on cyclosporin 2x150 and tampering prednisone. My numbers are quite good, HhB around 8, and platelets 33, whites high due to high doze of prednisone - I was on 2x 30 for over a month.*
About 10 days past hATG I started developed quite intense pain in my extremities/ limps' joints kind of shooting up through my bones to higher joints - knees and elbows. We thought at this time that its the serum sickness - I has an episode of it about 2 weeks after my first hATG that resolved within 48-72 h without further problems. Vicodin was the med to take and it worked. *So this time when I started experiencing pain, we made sure I had plenty of it to help me out, but we did not expect what was creeping down on me. This time pains have not been letting go, is getting worst. I have constant tingling in me feet and palms, my wrists and fingers; feet and toes are kind of stiff and sensitive to temp. ( a lot of coldness in me feet); *this is *absolutely constant, and it even doesn't bother me. Worst part is when my " power thunderstorms and lightnings come" shooting pain initiates either in my wrists that spreads to my fingers and twists them in weird directions left lock in odd positions. Same happens with my ankles, with pain either soothing up to my knees or to my feet and doing the same to my toes.
So far I had 2 really bad episodes of the pain. First time I was trying to fight it with Vicodin for about 5 hour but gave up; it was way too much! *my husband had to take me to the ER - thank God we had a little rolling desk chair or he would have to carry my to the bathroom and the car. The pain was so bad that touching anything with my feet was making me scream!!!!
The doc at ER said is neuropathic pain syndrome! He immediately contacted (5am!!!) one call hematologist from my Doc's office and had a consult; same with my family doc. I'm still under great impression how fantastic he was!*
He treated me with morphine - and it HELPED.*
Prescribed Percocet which helps if I read the upcoming signs correctly *(trying to be toon into my body), and I guess I have been lucky enough since within the week of my ER visit I has one more bad episode, that I had to use morphine - I have it at home, my hematologist prescribed it..

Chirley, reading about you, and reading about this syndrome, I have to admit - it scares me!!! I know the outcome can be different it each case, but ...
I would love to hear from you, your story is giving me inspiration and strength and is helping me stay positive. Any tips, advise how you have been dealing with neuropathy would be appreciated, as I'm so new to it and I have not found many people who are AA and dealing with this on this forum.*
Tuesday is my hemo appointment and we are going to choose a Neuro doc that will be working closely with my hematologist.*

Thanks*
Gosia
**
**

[Chirley]

Hi Gosia,

Sorry I've taken so long to get back to you. It's 4.30 in the am here and unusually I can't sleep. Since I've been on the muscle relaxers I've tended to sleep 18 hrs a day.

I hope you get some answers from your neuro, mine is not very communicative. I've found out that I have myelopathy from my MRI reports, he didn't tell me. To this day I don't really know what it is. I found out that I have spasming from his registrar when I had an episode in front of her aand she said to my neuro that the spasming was obvious (first time I'd heard it called that). I had to ask three times why my body becomes rigid and stuck in odd positions before he replied "generalized dystonia" and then I had to Google it. I've complained about the pain to him on a number of occasions and the first time he just said that he wouldn't give me pain relief because it would effect my balance and I might have a fall. The next couple of times (over a period of 18months) he didn't bother answering.

When I was in pain in hospital the nurses tried to contact him for four days and he never answered his phone or pages. It was left to my hematologist to deal with my neuro issues. I'd love to change neuros or at least get a second opinion but when I raise the subject with my haem or GP they tell me the neuro is one of the best there is. I'd hate to think what the others must be
like!

My neuro has never ordered an MRI or any blood tests (except the original diagnostic blood test) or Lumbar Puncture or EMGs or any kind of investigation they have all been ordered by my haem. The neuro does do a pretty thorough physical exam, he seems to love poking me with needles to see if I can feel it. :)

I'm not usually a person who volunteers for invasive testing but it's been 15 months since I've had a BMB and 18 months since I've had a LP (it was quite abnormal) and I'm thinking it's time I had some follow up testing just for comparison.

If it's of any comfort I think there's a difference between neuropathy and myelopathy. I have a combination of the two. Neuropathy seems to be more concerned with peripheral nerves and myelopathy with the central nerves/spinal cord or brain ( don't quote me). I have hyper intense white matter lesions in my brain as well as lesions in my spinal cord so I don't really know what bits cause what effect (once again, no communication).

Some days are better than others and I can't seem to get a grip on cause and effect yet. I did think at one stage that the more I tried to do the worse I felt later (body seizing up) but I have had a couple of times now when I've simply woken up and nothing wanted to work, so I don't know why that would happen.

I'm on NO pain relief. Sometimes I think I can't take any more but I live through it and it eases off eventually. But, next time I see my GP I'm going to ask for pain killers because I can't see any benefit in "being brave". The neuro can't use the argument that I'll fall over, any more because I now use a wheel chair.

Do you have blood pressure problems? My BP today was 88/48 lying down and taking tablets to increase it. I also have lots of salt on my food and have started eating a bag of potato crisps every day (only for the salt, of course:p). I'm also starting to have a problem with keeping my potassium up in the normal range but I think that maybe the steroid is keeping the salt (above normal on blood tests) at the expense of the potassium but that's just my
theory.

I have heard them talking about neuropathy in the treatment centre I go to and apparently it's a common side effect of chemotherapy. I don't know if it goes away when the chemo is stopped or if it improves or even goes away completely. Mine is caused by something completely different and I'm told that it's permanent and likely progressive, but I just have to wait and see.

Best of luck to you and. ,yes, it's nice to not feel so alone. I don't really feel as if I belong to this forum but it's the closest thing I can find that relates to my condition.

Hugs

Chirley

[Marlene]

Gosia,


What other drugs are you on? Any anti-biotics or anti-fungals or anti-virals?

Many things can cause neuropathy. Toxicity from drugs, insufficient nutrients, diabetes, candida, viral/bacterial infections, auto-immune attack, structural/spinal issues and more.

Marlene

[Gosia.P]

Sorry I didn't reply right away. Wanted make sure I have all the info.
Tuesday was my hemo appointment. My numbers are looking good. HB was still high 10. I was transfused 2 weeks ago. Looks likemplatelets went up a bit, which would be a good sign.
As to my pain - no changes. Still feel crappy and immobile. Haven't been driving in a month!
Discussed with my hemo doc Neuro consult, as to the diagnosis given to me in the ER - neuropathic pain syndrome. He doesn't think that the problems are Neuro but extended serum sickness, but made sure I'll be seen by a neurologist. It happened today. Poor guy was quite clueless as it comes to aplastic anemia and the treatment, we ( my husband goes to all of my appointments) had to explained a lot to him. But he listen very carefully and examined me. Based on the exam he does not see any Neuro changes !!!!!! *I'm so happy to hear that!!!! He relates my pain and problem to serum sickness and prednisone. Will be going back on 4/30 for a Neuro test EMG and a follow up visit with him. He wants to make sure that he is not missing anything. I have to say, that I like his approach.

Marlene,
You asked what drugs I'm taking, here is the list: prednisone 2x 15mg was on 2x30 for 5 weeks and we are tapering it down now; cyclosporin 2x 150 mg, Lasix 1x20 mg for edema and synthroid for my thyroid, had Graves Disease and my thyroid was ablated in 2003, and Paxil 1x20mg to remain sane in this whole mess :-)

I do hope that this long lasting serum sickness will easy off soon, I can handle the pain quite well now as I'm listening to my body and know when to take meds to easy it off, yet my biggest challenge is lack of decent mobility.*Also swelling - my face, neck, shoulders... Bothers me a lot. I don't see how much the Lasix is helping...
Any ideas...

Charlie take care, hope you doing well

Gosia

[Marlene]

Hi Gosia,

Good that they don't think you have neuropathy. Your edema can contribute to the overall pain. The swelling can compress nerves causing some problems. Hopefully you'll get off the prednisone soon and the swelling will come down. John never got a really good response when he used lasix orally. The IV lasix worked much better for him.

Even though they have ruled out neuropathy, keep in mind that Paxil can cause it in some. So if things don't clear up, you may want to consider another med.

BTW.....John ended up with peripheral neuropathy....I guess you would call it collateral damage from treatment. Totally unexpected.

[Chirley]

Hi Gosia, I'm pleased you are getting good care and hopefully you are having a temporary episode of serum sickness. While I was over the moon to think I was chatting with someone who knew what I was going through, I wouldn't wish it on my worse enemy.

I'm getting the most diabolical conflicting advice from my doctors. My neuro ordered Diazapam for spasticity and has never followed up. I haven't seen him since then and I have no appointments to see him. My rehab doctor changed the dose and times of the Diazapam and told me not to stop them, she even said to take extra doses when needed. This week, my haem doctor told me to start reducing the dose with the aim to be off them in a week or so, he thinks they will increase my risk of having a fall and a little spasticity/hypertonicity might be beneficial in helping me walk! Who do I believe?

I had almost daily IV Copper when I was an inpatient for three weeks and I've just finished another week of daily IV copper as an outpatient but my haem tells me my copper level isn't improving much. He said my urinary excretion must be increasing and he'll order another 24 hour excretion test later (when I've had a bit of a break from medical things). My CBC is almost normal still just low platelets (slight) and lymphs. The biochem is still abnormal. Potassium is low and decreasing each time, bicarbonate is low, eGFR is low, protein is low, liver enzymes still high. LDH is high. I can't remember the rest but there is an awful lot of red results on the results form.

I'm getting used to my new wheelchair but will still need to touch up some paintwork on the odd bit of furniture and doorways :o. Not to mention grazed knuckles... The cat has learned to move out of the way and my dog knows to always keep an eye on me....I think she's learned to sleep with one eye open.

I've had a LOT of Hydrocortisone in the last month or so and I'm also on Fludrocortisone daily and finally my blood pressure is responding and I've had a systolic of up to 100 once this week. Mostly it's around 90 and I function very well at that level. I just get light headed when it goes down to the mid 80s or lower.

The new treatment centre is working well. I get my own room and a bed (not a chair) and wifi is available, there is my own ensuite, the staff are lovely and there are constant offers of tea/coffee/cakes/biscuits/ sandwiches etc. The lady who makes the daycare appointments (not the medical receptionists) is coming to my house today to buy my now unnecessary treadmill.

The other treatment centre was very posh/designer label "have a nice day" kind of place. This treatment centre the staff seem more real and sincere. The emphasis is on patient care and comfort rather than on what kind of skimpy uniform the nurses can wear and whether they have a hair out of place.

It's starting to get cool here, it's down to 24/26 degrees during the day and I feel like I need a light coat. Winter is coming.

My cat just decided to hop onto my wheelchair and thinks she's found a new bed, I've got news for her ;)

Regards

Chirley

[cathybee1]

I am so glad the transition to this new treatment center is going so well, and some of your numbers are improving. Banged up furniture can be fixed while you adjust to your new wheels.

Bruce's copper has been sitting around 59-61. He has stopped chasing finding out why, and mostly manages his situation via blood transfusions and Exjade. He is experiencing more fatigue that previously right before his transfusions. He is going approximately once a month between transfusions now.

Glad some of the medications are helping you

Hugs, Catherine

[Chirley]

Ive just finished my third daily treatment for this week and although my doctor is only supposed to attend this daycare centre on Thursdays, for some reason he popped in to see me today.

He seems to be pleased with the progress I've made :o.

Mondays blood tests were not as good as expected, reds, whites and platelets all low. Biochem was awful. Potassium, phosphate, calcium, protein, albumin, globulins all low and as usual raised liver enzymes.

All in all most of the blood test results came back highlighted in red. But after just two extra doses of copper and tweaking the steroids my tests are a lot better. Only low whites (slightly) and platelets (went down further for some reason), reds recovered nicely and Hb came up too. Biochem is better. Still a lot of abnormal results but generally improving.

My doctor told me today that I can hopefully start tapering the Fludrocortisone by the middle of next week. He said it has to be slow because I'm on both hydrocortisone and the Fludro at the moment and he doesn't want to cause adrenal insufficiency.

I think I'm improving neurologically. It's hard to tell. It seems as if it's two steps forward and one step back but I'm convinced I'm regaining a bit more function. Plasticity is a wonderful thing. I can actually walk two or three steps if I hold onto something (on my good days). Just when I get confident things are coming along nicely something brings me back to reality. For instance, I fell and put my right hand out to catch myself and I've hurt my wrist and now I'm in a brace, it makes it difficult to use the wheelchair. Then sitting in daycare for many hours at a time increases the spasticity in my legs and I have to take muscle relaxants to ease the spasms, but the relief after the spasms subside is wonderful.

I was told today that they have definitely attributed my copper deficiency metabolism problem to the chromosome deletion so at least after 9 years of investigations and treatment we finally know the root cause of the problem. It doesn't help the condition, treatment or outcome but hopefully it will help children born with this deletion because now they know to test copper levels. Previously they just attributed the neuro degeneration in these children to the whole syndrome but they are looking at it differently now. I almost feel proud that in some little way I just might help some child from dying from progressive neuro disease.

I'm incredibly tired I've been up since 5 am and it's after nine pm and do it all tomorrow again.

Goodnight.

Chirley

[Birgitta-A]

Hi Chirley,
Very positive that your Hgb and biochem are better after extra copper and that you perhaps are improving neurologically!

Wonderful that you and your uncommon disease can help children with the same chromosome aberration! I - like you and many other members - have thought many times that if your first doctor nine years ago had controlled copper your prognosis should have been much better. I admire your courage!
Kind regards
Birgitta-A

[cathybee1]

Birgitta said everything I could say, but better! It's so great to hear from you and hear you're improving.

Hugs :)

[Chirley]

Hi, looks like I spoke too soon. My blood results on Friday weren't good. Despite Mondays Copper level showing it was in normal range, even before this week of copper infusions, I've developed pancytopenia again.

My ceruloplasmin level was low so I'm not able to utilize the copper at a cellular level. My doctor says my liver is getting worse and I think, he was relating the lack of copper transport protein to the liver dysfunction. All my proteins are low and getting lower despite increasing my protein intake.

He said that I may have to have further venesections even though my red cell count is low because he needs to take some of the load off my liver and hope the function improves. (red cells low but Hb just in normal range).

He admits to feeling at a loss at how to treat this, apparently there is a lot that isn't known about metals and how they are metabolized. I think I might have to make an appointment to see the metabolic medicine doctor and ask him to contact my hematologist and discuss the possible reasons and treatment options for this.

I feel okay physically I'm just having problems coping with the highs and lows and the hopes and disappointments. The emotional toll is leaving me exhausted.

One positive thing is that I can start tapering the Fludrocorttisone next Thursday with the aim to cease it entirely within the following 10 days. The tapering is wholly dependent on my BP staying at 100 or above. If it drops
below that point I have to restart the full dose. Fingers crossed.

Regards

Chirley

[Birgitta-A]

Hi Chirley,
How terrible to have a disease that is so complicated as yours! You must be very tired due to the hopes and disappointments and the fact that nobody really knows what treatment you should have.

Personally I don't like the idea of venesections when your RBC count already is low. You loose a lot of valuable proteins in venesections too.

Hope your BP will continue to stay at 100 or above!
Kind regards
Birgitta-A

[cathybee1]

Chirley, this news does seem puzzling -- you truly have had a roller coaster ride the past few months. I am praying that your BP behaves and the doctors can better coordinate your care.

[Chirley]

Hi, just a quick update.

I saw the Prof of metabolic medicine today and he admitted that there is a lot of unknowns with my disorder. He verified that even though I'm having IV copper and my blood levels can look normal, that I'm not utilizing the copper at a cellular level. He said that this was the reason I had high urinary excretion. This means that despite treatment, I may still get all the other complications of copper deficiency like aneurisms, cardiac arrythmias (already have) etc.

He also said that ferritin levels are next to useless because of the copper protein/ferritin relationship so my ferritin might be higher or lower than the blood reading. This applies to the zinc level as well.

He recommended that the hematologist arrange a liver biopsy to assess metal
levels at a cell level. Yuk!

I'm feeling well and can now walk around my house with a walker (wobbly, but do able). My only real complaint is fatigue which has worsened in the last couple of weeks but that may be weather related. Short days, overcast, rainy, cool, windy, all I want to do is snuggle in and sleep. This weather is unusual for a Brisbane winter.

Copper infusions scheduled for the rest of this week and a couple of days next week. I will see the hematologist on Thursday and hope he doesn't want to do a liver biopsy :eek:

Regards

Chirley

[cathybee1]

Hi Chirley! Ugh :(

If a liver biopsy is one test you haven't had before, Bruce's went very smoothly. There are risks. We did a lot of talking to the surgical radiologist ahead of time.

Bruce did insist on conscious sedation.

[Birgitta-A]

Hi Chirley,
Good that you now can walk around the house with a walker! Can it be the result of IV copper?

I understand that you don't like the idea of a liver biopsy - can't they measure the iron in the liver and heart with MRI? I have had that done.
http://www.ironhealthalliance.com/disease-states/myelodysplastic-syndromes/measurement-of-iron/magnetic-resonance-imaging.jsp

Kind regards
Birgitta-A

[cathybee1]

Chirley, hope that everything is ok. I haven't seen a post from you in a while.

Hugs, Catherine

[Chirley]

Hi, I haven't been around for a while, I haven't been feeling very well but nothing to put my finger on.

No news really. For some reason my ferritin level is increasing without transfusions and I've been having some discomfort (sometimes pain) in the upper right quadrant. Liver enzymes still up but not getting any higher, for which I'm grateful. I had a venesection yesterday, maybe I'll start feeling more comfortable. I'm also booked for a liver USS tomorrow with view to avoiding the liver biopsy if everything looks okay.

I'm still having daily IV copper for 5 days every 3rd week. My copper level is mostly in normal levels now but my ceruloplasmin remains below normal. For some reason my CBC varies between all cell lines being slightly low (with normal Hb) to only the platelets being low. The platelets never reach normal levels now even though they were the only cells that remained normal until I
had Vidaza. The low ceruloplasmin is a bit of a problem because it means I can't metabolize the copper properly. My doctor hasn't said so, but I think it's the reason my CBC is still slightly abnormal with normal copper levels. I hope reducing the ferritin will help raise the ceruloplasmin.

Neurologically, I'm deteriorating slowly. I was assessed by an OT a few weeks ago and I'm losing upper body strength now. I have completely lost the use of my wrist extensors which explains why I've been having trouble using my manual wheelchair. The OT has recommended I buy a power chair and I'm just waiting for more input into which type of chair is the most suitable. I haven't been able to leave the house for anything other than medical appointments for about 6-7 weeks now so getting a power chair to be able to go shopping would be a big step forward.

I went for a driving assessment to see if I can use hand controls and while I passed the assessment it was recommended that I don't drive because it would be too tiring and would decrease my ability to complete my ADLs. The OT recommended I use the power chair in wheelchair taxis and buses instead. It would have cost around $70,000 to buy a van capable of carrying a power chair with lift and hand controls and I thought I could catch a lot of taxis and buses for that kind of money anyway and I wouldn't have the stress of battling the traffic and finding parking spots. So, I'm not particularly disappointed about not driving.

Even though I've been told that I don't have the capacity to increase my strength (too much nerve damage) I've bought myself a Wii and use that everyday to help retain the strength I do have and to help with hand eye co ordination and balance, it's also fun.

Well, that's my saga. Basically status quo. Hope I didn't bore anyone.

Regards

Chirley

[cathybee1]

Not boring at all, Chirley. I hope you get your power chair soon and that the pain resolves. I wish someone, somewhere could figure out how to stop the neurological progression.

Bruce currently is getting transfusions once a month, and taking 1000 mg of Exjade daily. He had a kidney stone last month and has some stenosis in one of the arteries leading to the kidney, but so far between that and the Exjade his creatinine levels haven't worsened. Knocking wood. The cardiologist took him off Plavix and put him on a statin. His platelets dropped below normal after he started the Plavix, and haven't returned to normal, so we don't know if they will.

I'm so glad to hear from you. Hugs,

Catherine

[Chirley]

Hi, I've been having copper every day this week and today I had another venesection as well.

My blood tests are still a lot better than before starting the copper treatments but the platelets are still very slowly declining and the liver enzymes are very slowly increasing. The ceruloplasmin level is still below normal too.

I asked the haematologist about all of the abnormalities today but he didn't have any answers.

Every time I come to daycare for treatment they leave my chart on my bedside table but I've never looked at it before. Today I had to move it to use my phone so I looked at the chart. The chart is dated April this year and has my diagnosis as MDS/CNS degeneration. I was told in late 2010 that my MDS diagnosis had been changed to Cu deficiency bone marrow failure. I am also
on the Federal Governments Cancer Registry.

So, I'm asking myself, is the diagnosis on my chart wrong or is Cu def BM failure now classified as a MDS subtype. Or do I still have MDS RAEB 1 and Cu deficiency?

I asked whether I need to have the liver biopsy that the Prof said I should have. The haematologist hasn't had a letter from the Prof so he said he doesn't want to do the biopsy at the moment. I rang the Profs secretary and he said that the Prof hasn't written ANY letters to my haematologist EVER. Don't you just love the way these people don't communicate! Anyhow, I asked
the secretary to ask the Prof very nicely to write a letter to my haematologist so he can be kept in the loop.

In the meantime my haematologist is considering repeating my Lumbar puncture to see if the abnormal 14-3-3 protein is still detectable and that might provide something to gauge the rate of neurological disease progression. Another LP might provide some reassurance that the degeneration has been stopped, so I'm pushing for it to be done.

The USS I had done of my liver 3 weeks ago is pretty good it just shows iron overload, hence the extra venesection today.

You know what, I'm bored with this whole thing, isn't it wonderful. So much better than lurching from crisis to crisis.

Ho hum, yawn. :D

[cathybee1]

Hi, Chirley.

Glad your blood levels are holding again. I hope the ceruloplasmin and liver numbers eventually start moving in the right direction.

It's surprising that the CU deficiency isn't listed on your chart. But your situation is so unique. Just wondering if there is a treatment advantage/priority given to your care based on the diagnosis listed on your chart.

Bruce and I are on our way to a remote lake cabin for a week. He is doing well, all things considered but needs a break. He is already scheduled for a transfusion as soon as he gets back. His hgb level is holding above 9 for a couple of weeks post-transfusion, and then seems to drop fairly quickly.

[Chirley]

Hi,

I realize that this isn't an issue for those who have had Vidaza for MDS but my diagnosis of MDS is in doubt.

I've had 5 cycles of Vidaza and it's just possible that I didn't need it. Now I found an Internet site that says the Vidaza given to laboratory animals caused a range of cancers. These cancers ranged from skin to lung, breast, lymphoma and Leukaemia. I can't find that site again and don't know how reliable it is.

I emailed Celgene and asked them if I could have any follow up research on the long term carcinogenic effect of Vidaza. I didn't receive a reply so I rang Celgene Australia and spoke to a patient advocate. She said that the email had been referred to her but that she wasn't permitted to let me have that information.

Does it seem right to anyone here that this kind of information can be withheld?

I'm thinking of asking the oncology pharmacist to try to get this information for me. Does anyone have access to this follow up research? If I remember correctly the rate of haematopoetic and solid tumors was quite high but I admit that I wasn't reading the article closely and was just skimming through.

I got a call from the daycare I go to with the results of my last copper level (they are taking a week to come back now!) and it was well below normal. The level has dropped considerably since I've been having venesections and it seems a bit more than co incidence.

Another thing to ask my haematologist about.

Regards

Chirley

[Neil Cuadra]

Depending on how you define "long term" there may not be human studies far back enough to show what the long term effects are. But whatever data was used to gain approval for use of the drug out to be made available to patients.

[milliken2]

Chirley;
Do you have a good rapport with your pharmacist? If so - ask him to get into his medical computer, and print out all of the information on Vidaza. Too - have you checked out the Physician's Desk Reference? Some of what you read may be 'gobletygook' to you - but if I can help, let me know.
Also - the pharmacist may also be able to give you the package insert from the manufaturer. If not - email the manufacturer and ask for a copy of the drug indications/contra-indications and specific uses. This is true of any drug that you take.
Eons ago, when I was in nursing school - you had to hand write your drug cards, but I went the extra mile, and my pharmacist always gave me a package insert (they generally throw them away) and I attached it to my drug card and care plan. Got kudos from the instructors for making the extra effort. Good Luck To You.

Beth

[Chirley]

Thanks for your replies. I was given the normal drug info from the package by both the doctor and pharmacist and the doctor made sure I knew all about Vidaza before I agreed to have it.

I thought that the long term effect of Vidaza may not be known because it hasnt been in use for very long but it has been around since the 80s which means there should be something available after 30 years of use. The fact that there is little or no data available suggests that no one has lived long enough to suffer secondary cancers. The other alternatives are that there is data available and I can't access it or the data is still the property of Celgene and they don't want to release it.

I'm still going to ask the pharmacist to help. It would be good to know if I need screening tests for particular cancers or even just be aware not to ignore certain symptoms.

Thanks again.

Chirley

[Birgitta-A]

Hi Chirley,
As far as I understand most drugs used to treat cancer can cause other types of cancer. You know Revlimid is not approved in Europe for MDS because a few patients in one study developed AML.

Here is an abstract about therapy-related myeloid neoplasms:
http://onlinelibrary.wiley.com/doi/10.1111/j.1476-5381.2010.01100.x/full
Kind regards
Birgitta-A

[Chirley]

Thanks Birgitta, I understood the broad concept of the article. It's unfortunate that there is no real data regarding hypomethylating agents but maybe that's a good thing ;)

I feel a little like someone who's escaped a jail cell to find I might have tunneled into the exercise yard.

I guess it's just like everyone here, there's nothing to do but wait and see.

Its 18 months since I've had a BMB and even though the last one was a vast improvement on the previous ones, it was still abnormal and now with my counts starting to bounce around a bit and my platelets steadily declining, I would feel a little more secure if I had another BMB but my doctor doesn't seem to think it's necessary.

Regards

Chirley

[Birgitta-A]

Hi Chirley,
You know I don't think you have a bone marrow disease but low copper eventually since you were born. All symptoms can depend on that - good that your bone marrow manage to produce cells after so many years with harly any copper at all.

Platelets are often a problem - they are so sensitive. I have been worried about my platelets since dx. I try to avoid everything that eventually can decrease platelets: drugs, food and other substances. I have asked my doctor when I will stop taking Thalidomide and try Revlimid instead. He has told me that we shall wait until they are 50 - last count 72.

I have not had a BMB since before Thalidomide May 2010 - I can't say I miss it though I have so severe fibrosis that they can't aspirate (that's the part that hurts).
Kind regards
Birgitta-A

[Chirley]

I'm in daycare this week. My copper level had dropped last time so my doctor increased the dose of copper sulfate from 4mgs/day to 5mgs. I asked if I could have this run in over an extra hour because I already get tummy pains and diarrhea from the 4 mgs. The nurse agreed to do this but then the pharmacist (new) came in and insisted the infusion was run faster. Needless to say I haven't had very good nights this week.

I saw my doctor this morning and I'm booked to have my liver biopsy next Tuesday. Apparently it's done with a skin local anesthetic under ultrasound in radiology. My liver enzymes are still slowly climbing despite the venesections and the Prof thinks that I have protein transport problem associated with the Hephaestrin and COMMD1 genes which causes an intestinal absorption problem and a renal tubule wasting syndrome. He thinks that the copper that I'm receiving is building up in my liver and causing damage.

The problem is that if the copper is accumulating in the liver it will cause liver failure and I'll die but if I don't get IV copper I'll get progressive neurological damage and bone marrow failure and I'll die. I've thought about what I want to do given the possible outcomes. This decision is really very easy, I've already decided what to do if the Prof is right about the copper accumulating in the liver. I've decided that I'll stop having the copper infusions and just have supportive care. I definitely don't want to die from liver failure.

Then again, there is so little known about my disorder that no one knows what's going to happen and I'll ignore this other stuff until the findings come back.

I was just interrupted by a phone call from radiology to tell me I have to be
admitted to hospital for the biopsy......has anyone else had to be admitted, does this sound routine? I HATE hospitals.

Regards

Chirley

[cathybee1]

Ugh, between a rock and a hard place. I hope the professor is wrong for a change. Bruce was admitted to hospital for his liver biopsy.