Watch and wait ends, Vidaza begins.

[Chirley]

Hi Catherine, I find it incredible that you have to insist on sedation. The clinic/doctor I go to won't do a BMB without sedation even if you ask for just local anesthetic. He/they say that hematology/oncology patients already suffer more than their fair share of needles and sharp things so why should they have pain with BMBs if it's totally unnecessary.

The clinic has a protocol of 5mgs IV Midazolam. This works wonderfully. While you're not exactly under anesthetic, just sleeping, there is no memory of the procedure. My doctor also insisted I have Midazolam when I had my lumbar puncture, apparently he had to have a number of attempts so I am grateful I was sedated.

BTW I'm glad Bruce is advocating for himself even if it means a long drive.

Regards

[donna j.]

Thank you! Since being diagnosed with MDS in July 11, I have had 2 bone marow biopsies and found them to be barbaric and unbearable. The pain was excrutiating. I dread the day I hear that I need another one. After just 2 rounds of Vidaza, I am black and blue, have lumps under the skin, and pain and marks where every injection and transfusion has gone in the last 30 days. Last week I counted 20 times i was stabbed, so the thought of sedation for the next BMT is so civilized. I will hold out for sedation.
Love this forum.

[cathybee1]

Donna, I'm very sorry that you had to endure such pain too...and I am with you, these forums are wonderful. by the way, yesterday, the local hematologist asked my husband why we were going to make such a long drive to have the bone marrow biopsy done under sedation, when he could do it in his office in 5 minutes. ...the rest of that story is that the consulting hematologist at the university hospital last year told Bruce they needed him to have his second bmb in a month, because the slides from the 5 minute one were unusable.

and Chirley, thank God you never had to fight for sedation and your doctors understand how much people with bone marrow diseases go through. No one should have to have 3 nurses hold them down during such an important procedure as a bone marrow biopsy, as happened with my husband.

on a lighter note, Marlene, wanted to tell you, I did look at Bruce's eyes after reading about the eye test for Wilsons. His eyes looked blue, just like always. Unfortunately after I shined a flashlight into his eyes, he couldn't see anything but a big spot for some time. So much for my examination skills.

[Marlene]

That's too funny Catherine....You'll just have to practice more .

Sounds like Bruce is on top of the liver biopsy. Unless the test is going to change the course of treatment or outcome, you have to question the need for it. Sometimes it's just to confirm what they already know. When John ruptured his quad tendon we were in Maine on vacation. They did an MRI on his leg and wanted to do the the surgery up there. We opted to get home asap and then have the surgery. When we met with the ortho, he examined John's leg and immediately knew what was wrong. We then told him we had the MRI result from the other doctor. He smiled and said "you don't need an MRI to see what happened here". On top of that, all they did in Maine was to wrap an ace bandage around his knee when he should have been in a brace. But we had no experience dealing with injuries like this. I could educate them on SAA though since they were confused by his counts at the time .

[celebrations]

thanx for answering my question concerning the risks of a liver biopsy.
Fortunately I do not (yet) suffer from low platelets and my wbcs are still in the range of 3.

In German hospitals they do the BMBs without sedation when you come to an appointment, whereas they give you the sedation (Dormicum), when you are hospitalized.
In both cases they of course give you a local anesthesia, but this does not prevent the sharp, unbearable pain in action. I am always shivering and sweating terribly shortly before
Reason: when you are treated externally, they don't have enough beds nor health care personal to watch you, in case the sedation causes any problems.

I had my last BMP in August with a bad result, so I know, there will be another before Christmas, one without sedation of course....
I can feel all your pains and I am so sorry for anyone, who has to undergo this.

Greetings,
Bergit

[Chirley]

Berg it, that was a different story for me. The one BMB.I had done while in hospital (different doctor too) was performed by a doctor from a pathology company and refused to give me sedation even though I asked for it. He just said that he would give me sedation if I were a man but that women had a higher pain threshold so I wouldn't need it. I told him I had tough bones and he replied that he was strong and went to the gym.

End result, he had to take the biopsy from the front of the iliac crest after failing from the back. So I got to watch it all. He also kept slipping off the bone and puncturing me in the abdomen, fortunately it didn't hurt at all when he did this and I have to agree with him that the procedure while not pleasant wasn't agony either. The fun part was watching the faces of the medical students while he was doing it......some of them looked a bit sick, others grimaced....it was hard not to laugh.

Regards

[cathybee1]

Bergit, I am so very sorry that you have had such unpleasant experiences too. Some kinds of sedation are not usually administered here either, outside of a hospital setting. We suspect that part of the local hematologist's refusal to give sedation might have been because of that.

Having said that, I am undergoing some injections now around my spine and hip for a chronic condition. I am getting these treatments in a doctor's office. Before each treatment, the doctor gives me an injection of versed and demerol, into the vein of my hand. It takes the edge off the pain of the injection considerably, and keeps me still so the doctor can do his job properly. I wonder why something like that couldn't also be offered to patients undergoing bone marrow biopsies.

Chirley, so glad you can laugh now about what sounds like a horrendous experience!! If a doctor of mine said he went to the gym as an expression of his qualifications, I think I would run away as fast as I could!!

Our family doctor said when he was a medical student, the university gave students $20 to volunteer as "patients" so their classmates could practice bone marrow biopsies. Ours only volunteered twice, the second time, the needle slipped off the bone, but the "doctor in training" continued. Contrast that to the hematologist in Portland we saw last year...when Bruce asked him directly if he had ever had a bmb, he said no. I suspect if all hematologists were required to undergo a bmb before being certified, more would share the attitude of your wonderful doctor there in Australia.

[Chirley]

Just a quick update. I went to daycare for my first visit in3 weeks for IV copper. My CBC was completely normal. Copper level won't be back until Friday, but I don't have to go back again for 4 weeks when I'm booked for daily copper for a week with another venesection as well.

Things are going really well for the first time in years. Now I need to concentrate on strengthening my bones and joints. I've just broken my second toe in 4 months and the orthopedic surgeon says I'm not a suitable candidate for keyhole hip surgery so I suppose open more major surgery is in store for me at some stage in the future so I have to try to improve my overall condition.

Regards

[Birgitta-A]

Hi Chirley,
Wonderful that your counts are normal ! Too bad with bone and joint problems - perhaps swimming can improve your condition.
Kind regards
Birgitta-A

[cathybee1]

Oh, Chirley, that's WONDERFUL news about your CBC. Strengthening bones and joints will no doubt be challenging, I hope you can find a program close to home that you will enjoy.

Bruce got through the bone marrow biopsy just fine. The Physician's Assistant who did it was the same one as last year. She was very slow and careful, and at once point when it was starting to be painful, told Bruce she must have hit a nerve, pulled the "awl" back out, administered more lidocaine, and started over.

Bruce has had 3 weeks in the low normal range for serum copper. Dr. then increased Bruce's IV copper a wee bit and the serum copper level dropped below normal. The ceruloplasmin level hasn't budged. His rbc seems to be holding a little better for the moment. So, we are waiting for the results of the bmb, and have made an appt with the MDS Specialist in Los Angeles for mid-November.

Otherwise, things are going well, Bruce continues to feel good.

[Chirley]

Hi, I just came from my 6 month neurological appt. Basically everything is stable with the exception of increased weakness in my hands and particularly my left hand.

The blood that was sent to Sweden for testing came back negative for the ATP 7A mutation so further testing is being done for CRT1.

The neurologist also supported the orthopaedic surgeons decision not to operate on my hip. He said that it would be a brave surgeon who would perform elective surgery on me with a co morbidity of cerebrospinal degeneration. I don't understand this but he also said that IV copper would increase my risk of infection, I told him my white cell count is now normal but he said it had to do with the coppers affinity with bacteria or something similar to this. I admit to zoning out and staring off into space when I start getting lectures about my disorder.

It turns out I have not only broken my 2nd toe but I have broken the bone (metatarsal) in my forefoot as well. The neurologist said how lucky I was that I had diminished pain sensation because otherwise I'd be having difficulty walking. I mentioned that it was itchy for a few hours after I injured it and he was very interested. He said that pain and itch use the same nerve receptors and it was just my perception that pain was an itch instead. The human body is very weird.

Regards

[Birgitta-A]

Hi Chirley,
Well, I suppose it is a good thing that your neurological status is stable except for weakness in the hands but we hope that you will improve now when the copper level at last is OK.

When our ferritn value is high we can get more infections and it is probably the same with high copper levels.
Kind regards
Birgitta-A

[cathybee1]

Hmmm, interesting that taking copper increases risk of infection. That is a little known side effect of IV copper, but there is so little information about it, any little bit is good to store away.

I'm so sorry you have broken your metatarsal. I hope this doesn't slow down your travel plans. Gosh, however would they splint that part of your foot??

And I so get what you said about zoning out and staring into space!!!! Bruce does it a lot, but he says "uh huh," along the way, and later says he was compartamentalizing. I'm not sure doctors even notice, they get on a roll sometimes, don't they?

[cathybee1]

Bruce's copper is subnormal again, and has been for the last 2 blood tests.

We got the marrow results back. The hematologist said there is very little change change from last year. The pathologist is still calling what he has macrocytic anemia. There is a very slight increase in ringed sideroblasts this time round. There are no genetic abnormalities.

At first, both of us were a bit overwhelmed because after all this time, there is still so much uncertainty. But it really is good news that there has been so little deterioration in the appearance of the bone marrow.

Our family doctor has written a page of questions for our consultation in Los Angeles in 2 weeks. The local hematologist is finally getting curious about why Bruce's copper levels can't be normalized. We raised the issue of Wilson's Disease with the hematologist. I provided him with the name of a doctor at the Mayo Clinic who wrote one of the articles about copper deficiency and bone marrow abnormalities. So...we are recruiting reinforcements.

Meantime, Bruce has stretched out his interval between transfusions a little bit. His last one was September 28, and as of yesterday, his hgb was still at 8.7 (not bad for him).

[Chirley]

Hi Catherine, it's great Bruce has maintained his Hb even with low copper levels. Even better that he has no further bone marrow problems.

I asked again about having a slit lamp test but I was told it's not necessary because they can see I have no rings by just looking. Odd, I thought it had to be by slit lamp but what would I know....

I haven't had a blood test for a couple of weeks so I don't know if I'm well. I'm considering refusing any more copper for a while just to see if my neurological condition does indeed progress. I know I will need transfusions again and my WCC and platelets will drop but a transfusion every two weeks is much more easy to tolerate than constant copper infusions. I have some thinking to do on this before I make a decision.

I have just returned from my Tassie holiday. It was wonderful but I'm glad to be home. The Tasmanian Devil certainly sounds and looks ferocious but at least the zoo keeper held onto the sharp bits while I stroked it's back. I come from a fairly warm tropical climate and Tasmania was cool/cold with drizzle, mist, wind and low cloud, I enjoyed it but I wouldn't want to live there.

Regards

[cathybee1]

Ah, Shirley...I am glad you got to see the Devil...yes, Tasmania looks like a cold, but very beautiful place. Rats that it was drizzly, but I guess that's what keeps it green.

I understand your hesitation to get back into the grind of the copper infusions. You're right, blood transfusions are relatively easy. The downside we are facing is the ever increasing iron load. I started compiling Bruce's lab reports today and putting the numbers into Excel, something I had been putting off. Seeing those ferritin levels climb is sobering.

It has been nice to be able to wait a little longer between transfusions. Bruce was gonna wait till after our trip to Los Angeles to deal with the transfusion, but decided he better have more bloodwork and get transfused if necessary before we travel. So he will go next Tues for bloodwork, type and banding.

We also got a call yesterday from the hematologist, wanting my records from the copper infusions we've been doing at home. So, it sounds like he is truly following up.

We did ask our family doctor a few weeks ago about the slit lamp test -- the slit lamp is the light used in optical exams. From what I read, the rings are supposed to be detectible from slit lamp exam before they are visible just by looking at the eye. Our doctor said that exam would have to be done by an opthalmologist. Hmmm, now wondering if that is one of the things they look at in a routine optical exam, or if it has to be requested specially.

While going thru a few inches of paper preparatory to our trip south to the doctor, I saw a little note that the Mayo clinic can do genetic testing of a blood sample which will identify if one has the Wilson's gene. Bruce inquired about that today.

Hugs. I am glad you got to take your trip and are enjoying being home again. I bet you missed the pups! There's no place like home.

[cathybee1]

The latest on this Medical Adventure:

Bruce had a consult with Dr. Paquette at UCLA on Monday. Dr. Paquette talks through his reasoning, but also talks very fast, so I'm not sure my notes were adequate to capture his impressions. But key points I got were:

1) Bruce's bone marrow abnormalities are very mild. Dr. P is not calling the problem MDS.

2) Dr. P believes that the problem might be autoimmune. He ordered some t-cell testing that wasn't done previously, which will take 2 weeks for results.

3) If autoimmune problem is confirmed, next step might be a trial of prednisone and/or cyclosporin.

4) A fresh take on the copper issue. Like the local hematologist, Dr. Paquette did not think the copper levels were low enough to warrant fingering it as the culprit, and suggested that if the ceruloplasmin (the protein the copper binds to) is low, you can add all the copper into the system you want, but any excess beyond the binding capacity of the ceruloplasmin will be excreted. So, as Bruce's ceruloplasmin is below normal, the copper will remain sub-normal as well. This was the complete opposite explanation of the family doctor to how the process works. In any event, neither Bruce's copper nor ceruloplasmin was tested prior to last October.

We're glad we made this trip. Dr. Paquette did tell Bruce he would like to see him more often than once a year, which made us feel very welcome.

[Marlene]

They're probably both right on some level. I did a quick search on ceruloplasmin and as you know, there's not a simple explanation. What I found interesting is the role it plays with iron. The wiki link says ceruloplasmin is not the only transporter of copper. So the question is why is his ceruloplasmin low? Is his liver not producing enough?

http://en.wikipedia.org/wiki/Ceruloplasmin

The zinc, copper and iron balance are so important. I think iron overload is very disruptive to many aspects of our body.

Such a puzzle.

[Chirley]

Hi, I find this topic very timely.

I'm in Daycare and I've just finished my 2nd venesection. I had a dose of copper and a blood test on Tuesday. The copper result is back today. It has dropped 2.2 in 4 weeks and is sitting at 10.1. I am totally thrilled with this result.

Previously my copper level would drop 2 a week but for the last couple of months the rate of loss has slowed considerably. I'm almost in disbelief. In my own mind I think this started happening with my first venesection.

Thinking back, I started having problems after I has pneumonia in 2003. I became anaemic, had a blood test was told I was iron deficient given a blood transfusion followed by an iron infusion and then my problems escalated. I was given more blood and more iron until I became allergic to all forms of iron and started coming up in blisters and having my skin come off in sheets (told it was Steven Johnson Syndome) . I was still testing iron deficient. I'm now wondering if the iron I was given upset the metal balance in my body. Now that I'm having iron taken off it's giving the copper a chance to be retained.

At least thats my theory.

BTW .... CBC on Tuesday had Hb at 143!!! Neuts were 2.6 and platelets 159. Only problem was AST/ALT even higher and have been increasing with every blood test for the last 3 months minimum. I was hoping they would reduce with the ferritin reduction.

I'm coming back for daily copper every day next week and then hopefully routinely we are aiming for 1 week on and 2 weeks off.

I'm booked to see Prof Bowling (metabolic medicine) the week after next and plan on asking about the other side effects of copper deficiency. I have practical knowledge of the hematological and neurological problems but there seems to be evidence of other issues like joint/bone issues and aneurisms. I also want his thoughts on my iron overload theory.

Regards

[Chirley]

I just got today's result pre venesection. Hb 124 neuts 2.0, platelets 130, lymphs 0.4, MCV 109..... Still really good but goes to show that even as little as 3 days can make a difference with copper deficiency.

[Birgitta-A]

Hi Chirley,
Very good that the copper level not is decreasing so fast and that your counts are so high!

You know the liver tests won't decrease so fast when the ferritin level is lower (and they don't increase directly when the ferritin level is too high).

Your disease is too complicated for me so I don't have any thoughts about your iron overload theory.
Kind regards
Birgitta-A

[cathybee1]

Chirley, first off, WOOHOO on Hgb of 143!!! 124 isn't so bad either. And I too will be interested to hear what your metabolism specialist thinks about the iron overload.

Marlene, thank you for the link. Yes, those balances are so critical...before this trip to L.A., I put all Bruce's bloodwork that we had records for (back to 1987) into Excel. So far, despite the steady increase in ferritin since the transfusions began last year, the copper and zinc really haven't moved that much. When they did briefly move into normal range, the ceruloplasmin did not.

We also know that Bruce's liver has not been functioning properly for some time...because of his high bilirubin level.

Ah, sometimes I wish there really were little nanobots we could just turn loose in there and have a look around.

[Chirley]

Hi, I've been in daycare for copper every day this week. I have just got my blood results, I was supposed to have another venesection but that's been put on hold. My Hb has dropped to 102. It was 119 on Monday and I haven't had any blood taken off for venesection or testing in the meantime.

My platelets and everything else were in normal range. This is a little worrying that my Hb has dropped so fast. I have to keep hoping that it's just a glitch and that the MDS hasn't raised it's head again.

I've been extremely tired lately and when I described the problem to my doctor this morning he said it wasn't tiredness but fatigueability. He thinks I need to return to the neurologist because there is a possibility that my neurological condition is progressing. I have trouble doing things for a period of time. For instance if I need to hold my arm out in front of me I can only keep it there for a few seconds before it becomes so tired that it drops. I have to rest my arm between forkfuls of food etc.

Just to top off a wonderful day my blood pressure was 80/45 so I'm having an extra litre of IV saline today as well.

I will just look on the positive that I have 2 weeks off from Copper treatments before the next round of daily visits. Hooray.

Regards

[cathybee1]

Chirley, ...I hope this is a little speed bump for you, and that the numbers and your fatigue will turn around soon.

[Birgitta-A]

Hi Chirley,
We all hope that your low Hb doesn't indicate that your bone marrow doesn't function so well - perhaps you had been drinking more than usual.

Everybody will be very tired with so low blood pressure. Unfortunately there are no good drugs for low blood pressure - they all only make the heart beat faster so you feel nervous. Exercise will increase the blood pressure but it is not easy to do anything when every movement is difficult.

Good with the copper, WBCs and platelets!
Kind regards
Birgitta-A