Watch and wait ends, Vidaza begins.

[Chirley]

Oh, I forgot to mention, the Ambulance Service sent a private car with driver for the journey to and from Daycare. Private limo service!!! What luxury, I had the best commute ever. Plus the driver was very flirty but in a funny, tongue in cheek way. They think they will send this service on Friday as well. I'm being spoilt.

Regards

[mausmish]

Great! You deserve to be spoilt!

[Chirley]

Well, I finally did it. I told my haematologist that I have been referred elsewhere for a second opinion. He was alright with it, said that he preferred people to get another opinion if they had any worries, he even knows the doctor I'm going to go to and has a high opinion of him.

My copper level didn't fall this week but neither did it go up so at twice a week Rx it still won't rise into normal range. I really want some time off so I negotiated a deal. I'll go to daycare for IV copper every day next week and have the whole of the following week off. I have been going to daycare for 12 weeks now, over 30 visits altogether and although I've had some reasonable (and even good) commutes, on the whole they are pretty horrible. Yesterday I was picked up at 7.30am for a 10.30 appointment. I arrived early and was taken in for treatment early. Treatment finished at 3pm and I waited until 7pm to be picked up by the ambulance. We visited 2 other hospitals on the way home (picked up a confused man who became violent and had to stop on the side of the freeway to restrain him) and I got home at 8.55 pm. This is do able once a week, tolerable twice a week and miserable any more than this.

The oral copper has been ceased for the short term to see whether I'm absorbing it. That's a bonus, although it's not too bad to drink it still has an aftertaste.

Cool and windy this weekend, nice to curl up with a book.

Regards

[Chirley]

I finished my extra week of daily IV copper and am about to start my one week respite. Bliss. I still have to have a blood test on Tuesday but that's a small price to pay for my first week off in 13 weeks.

I have been having the "sensation" of bladder irritation for at least 4-5 months and have mentioned this to my GP, neuro and haematologist. G.P made no comment, neuro thought it was a sensory problem caused by my spinal cord problem and the haematologist didn't seem interested. I finally asked the nurse in daycare to do a dipstick test. She came back and told me I had protein and haemolyzed blood in my urine, she also said she had mentioned it to the haem doc who ordered the sample to be sent to the lab for testing. Then, as I was leaving the hospital, I was given copies of my blood results. Full blood count is still mostly normal, just low platelets and lymphocytes. The biochemistry result was a bit more alarming. Every time I get chem results they have more red writing on them. This time I had high urea (higher), low protein (lower), low albumin (lower), high alp (same), high ast (same), high alt (higher) and low anion gap (lower). I am almost certain my doctor never looks at the biochem results.

Anyone here have any idea what could be causing the biochem abnormalities? I realise it's mostly liver and kidney function measurements but was wondering if the urinary findings might account for some of the blood results.

I'm starting to wonder whether getting into the red on the biochem to get black on the CBC is worth it.

Regards

[celebrations]

Hi Chirley,

The biochemistry result was a bit more alarming. Every time I get chem results they have more red writing on them. This time I had high urea (higher), low protein (lower), low albumin (lower), high alp (same), high ast (same), high alt (higher) and low anion gap (lower). I am almost certain my doctor never looks at the biochem results.

This is it!
Next time I going to take the old result with me and I will force him to talk that over with me! Because it has been the third time in a row that my biochemistry results were'nt in order.
Also my glucosis count is too high.
Solidary greetings, Bergit

Excuse me, may I ask, what gender and what age (just about) you are?

[Chirley]

Good morning Bergit.

I'm 55 year old female.

Perhaps, haematologists are so focused on getting the CBC in the black that they just don't see the whole picture.

I asked my haematologist a while ago what treatment, if any, he was considering for my increased ferritin/liver dysfunction. He said he had "taken his eye off the ball on that problem and must deal with it some time". He seems to have forgotten about it again. I don't think I should have to nag to get some treatment.

I think middle aged/older women become invisible. Nobody truly sees us any more.

Regards

[celebrations]

Thank you for your personal informations.

The sentence your hemo said to you about your biochemistry results is definitely not professional.

I will make myself visible the next time, I promise. :Wink:

As for your ferritin count, do you take Exjade to chelate? Are you transfusion-dependant?
Would you mind telling me your liver counts, just to compare.
Mine were: P-AST 36, P-ALT 57, P-GGT 31.

I hope you don't feel bothered by my inquiries.
Regards, Bergit

[Chirley]

G'morning.

My last ferritin was 2,084. I was transfusion dependant but I'm not now. I have had 2 transfusions since this last ferritin level was done. The highest level was 5,000 but that came down with time and a few weeks of Exjade, which I can't take any more because it caused increased pancreas enzymes, stomach pain and dehydration.

My ferritin level isn't an accurate indicator of iron overload in my case because an accurate ferritin test depends on a stable copper level(something to do with the protein pathways) and that is where my main problem lies.

ALP- 240, AST- 60, ALT- 95, GGT (normal)- 15

I'm not so concerned about the level of the results as the worsening trend. Every time I get results they are just a little worse than the time before. I have been told that increased liver enzymes do not necessarily mean you have liver disease, but my liver USS went from completely normal to echogenic with enlargement in a space of 7 months. I saw a gastroenterologist who confirmed that I do have liver disease. This is why I would like my high ferritin level fixed. I feel like I'm hitting my head against a brick wall. Perhaps there is a reason the haematologist doesn't want to treat yet but at least he should discuss it with me.

I know the treatment options are limited for chelation. Exjade is not an option, Desferal chelates copper as well (hard time retaining copper as it is) and venesection also reduces copper along with iron. I bought some wheatgrass powder but I can't take it because wheatgrass contains zinc and my zinc level is already very high. (zinc prevents copper absorption). I have seen people with end stage liver disease and it's not pretty. I would rather have bone marrow failure any day.

I'm more than happy to provide any information you'd like. Hope your doctor takes you seriously when you see him and you get some answers. I'd be asking about the high glucose level too.

Regards

[cathybee1]

Aargh, I posted a message to you last week I got in, but it apparently didn't "take."

Anyway, we had a nice trip away from the doctors (Bruce's and mine) though we missed our pup a lot. I'm sorry to hear about your continuing ups and downs. Nevertheless, I'm very glad you are getting a break from the CFH.

Bruce had his port installed a week ago. No complications, he was in and out of the hospital in 4 hours. I had training 2 days later in how to access and deaccess the port at the hospital infusion center. The training was excellent, they had a kind of CPR dummy that I got to practice on twice, then I accessed Bruce's port (for the first time!) under the nurse's supervision.

Yesterday, we went to the doctor's where I accessed the port and giving him the IV copper. Tomorrow is my first solo run.

We also met with the doctor yesterday. The doctor has now concluded that Bruce is not absorbing copper thru his intestines. The day before our trip, and after a couple of weeks of IV copper, his copper level then was 85 (normal starts at 71). Last week, after 2 weeks away, his copper level had dropped to 65. That was despite adding in the liquid copper sulfate.

We still don't know if all this will result in improved hgb numbers, but getting the copper levels stabilized seems like a good first step. Plus will save 350 miles and 6 hours of travel a week. Bruce's reticulocyte level is normal, ceruloplasmin bounces in and out of normal range, so the doctor is still expressing optimism the hgb will improve.

Btw, Ferritin was at 1400 last test.

Bruce had a stent placed in one of his coronary arteries a few months ago, and was put on Plavix. His platelet count, which was the only bloodline still in the normal range up to that point, has now dropped.

Meanwhile, he's still feeling okay. He's complaining more about arthritis these days than breathlessness, which is itself a little miracle.

Hugs, Catherine

[Chirley]

Hi Catherine, good to hear from you. I was wondering how Bruce was after having his port installed, 4 hours wow, they made me stay in overnight!

I also worked out that without copper replacement my copper level drops 2 points a week but the ref range here is 13-25, so 2 a week makes it drop below normal very quickly.

Bruce may very well have a good reason for c/o about arthritis. Copper deficiency can cause loss of collagen which can lead to joint/ tendon/ cartilage problems along with artery weakness (risk of aneurism). I don't know if I mentioned that I've been having joint pain and specifically R hip pain for at least a year. I finally had an MRI last week and I have tendonitis of 2 tendons, bursitis of 2 bursa and a labral tear. I'm booked in to have a steroid injection under ultrasound guidance tomorrow AM but may need an arthroscopy if there is no improvement. A CT of the lumbar spine also shows a new fracture of L3 so something is definitely wrong with bone integrity too.

My haem doc has decided that my copper level isn't being maintained with visits twice a week so for the time being we have both settled for daily visits one week with the next week off. The oral copper sulfate has been ceased a) because it didn't seem to work anyway b) it caused stomach pain and diarrhoea.

When I had last week off from the IV copper my renal function returned to normal i.e urea/creatinine dropped and eGFR came back into normal range. That was a relief, I didn't want liver AND kidney problems.

BTW, I think you are very brave accessing Bruces' port. I have been having my port used for over 3 years now and still turn my head away when the nurse inserts the needle. I can't imagine inserting the needle myself into my own port but I'm a bit whimpy that way. I don't mind needles too much but giving them to myself isn't my favourite thing. I did ask if I could have the port accessed at the daycare and then give the copper to myself at home but the answer was a resounding NO. In fact, I have recently found out the reason I have to travel so far to daycare is that no where else would agree to give me the copper because it has never been given in huge doses like mine before, unless it was in hospital as an inpatient.

I love my 2 dogs very much but I have just finished wrestling with them to cut their toenails and I'm pooped. It's like trying to hang onto greased pigs. If you were an outside observer you'd think I was killing them. They're going to sulk for the rest of the day now, BUT, I won.

Regards

[cathybee1]

I'm so glad your renal problems resolved...AND especially that you won't have to be making that trip every week.

And I had to laugh about toenail clipping. Gus used to be good about it, but one time I hit his quick, and ever since, he's been a bit paranoid. Guess I would be too. Now instead of getting a part of cookie after every foot, he gets one after every 2 toes. So, not such a bad deal for him.

I'm with you in that I'm also not sure how I would feel about accessing my own port. There is a video on Youtube about a woman doing it, using a mirror. The same doctor we go to has 2 other patients who do home infusions and access their own ports. So it can be done. But I don't think it would be easy, as you can't actually see very well down there.

Part of the training was we got to actually look at, touch, and play with a port. It helped me get over my discomfort about pushing that needle into Bruce's chest.

After our lesson last week, Bruce left the needle accessed, to see how that would be. It really interfered with the way he sleeps tho, so we are now going to access/medicate/deaccess each time. We went thru the whole cycle at the doctor's office and it took less than an hour (with her help of course). I will be taking bp and pulse beforehand as well, and I have a log to record everything in. Bruce will continue to have every other week blood draws at the hospital.

They gave us a lidocaine cream, called EMLA, that numbs the skin up. The first time port was accessed, it was without the cream, and Bruce really felt the sting. Monday, he had not much sensation there at all. As it turned out, I had a little item on my lower lip that had to be cut out, and they used the cream on me as well. Kind of like a mild novacaine sensation.

[cathybee1]

Just a quick note...as of blood draw yesterday, Bruce's hgb at 7.9, hct at 22, he is going in for a transfusion today. The last transfusion was exactly a month ago and he had 2 weeks of 2x/week copper infusions via his port. He was asymptomatic, this was really a surprise.

it will be another 2 weeks before we know what his current copper levels are.

He has been putting off having another bmb, but I suspect that is the next step. He has an appt with the hematologist on the 6th of Sept. There's always something to look forward to!!

Otherwise, we have been enjoying the tail end of summer, it has not been too hot here, and not a drought year, yay!

Hope you are doing ok, Chirley, that your every other week schedule to the hospital is working for you, and that all your crittters are frisky.

Hugs, Catherine

[Chirley]

Good to hear from you Catherine. It would have been better news if Bruce hadn't needed a transfusion.

I haven't started the week on, week off yet. I had copper on Tuesday and was shocked......my CBC was completely normal for the first time in 8 years. Platelets, lymphocytes...everything. My haematologist was confused because the copper level (previous week) had dropped substantially from the previous level. Anyway we discussed what to do and decided that he would phone me tomorrow with this weeks copper level and decide what my treatment schedule will be next week based on those results. He also said that while my CBC remains good he can't see any point in chasing our tails trying to keep the copper in normal levels. So in the last 3 weeks, I've only had 2 visits. I feel very rested and content.

That being said I have a suspicion that my CBC was a one off because I had just had a depot steroid injection in my hip 5 days before, but I'm not complaining.

I have also had another 24 urine copper, protein and creatinine clearance ordered along with a spot urine for cells, casts and polyoma virus PCR. I'm not so sure about having it done at the moment because I'm concerned the steroid needle will influence the results and the results could be very important. My doc told me he has been thinking I could have a type of renal disease which causes copper to leak into the urine.

I've also been told I need an orthopaedic surgeon review for my hip as I might require surgery to fix the the torn labrum. I think they would have to do some very persuasive talking for me to agree to that.

I asked again about giving myself copper at home and the answer was NO,NO, NO.

I have just finished reading a book called Edgar Sawtelle. It was well written, compelling, powerful etc etc and I wouldn't recommend it to my worst enemy. I cried all the way through and kept reading only because I thought it would have a happy ending, well guess what....it didn't. Ughh.

Both dogs (and cat) are happy and well at the moment so all in all things are going very well here.

Regards

[Birgitta-A]

Hi Chirley,
Wonderful with normal counts ! We hope that your bone marrow likes the copper and slowly tries to work normally!
Kind regards
Birgitta-A
72 yo, dx MDS Interm-1 2006, good result with Thalidomide + Prednisone since June 2010, latest counts HGB 13.2, WBCs 5.0 and platelets 95

[Chirley]

Hi,

I am on the last day of daily visits this week. I just got my copper level that was taken on Monday. The copper is at 15.9, it has never been this high and now I've had this extra week of treatment as well. So...I have 2 whole blissful weeks off from treatment. CBC was only slightly abnormal with platelets at 140 and lymphs down again so all in all this is very exciting news.

My doc said that maybe the steroid injection I had into my hip may have decreased inflammation in the kidney and allowed me to retain more copper than usual or it interfered with the copper/protein pathway somehow but he admits he knows very little about metabolic processes and we are both waiting on my appointment with the Prof that I have at the end of November.

I had a bit of a heart scare last weekend and ended up in ED for tests. Everything tested okay except for sinus bradycardia (pulse of 40) and low blood oxygen (sats of 90) so I have to have a dobutamine stress echo next week just as a follow up then the week after I have an appointment for the second opinion by another haematologist, so even though I have 2 weeks off, I'm not going to be completely medical appt free but it's better than it has been and every bit helps.

Even though my legs don't obey my brain and my torn cartilage in my hip gives me hell, I'm going to try to get some exercise regime started even if it's going to be static, something like gentle yoga, stretches and a very short treadmill walk and see if I can't get the rest of my body back into some semblance of health and fitness.

Yes, Spring has sprung and so has my optimism.

Regards

[cathybee1]

Hi Chirley,

Other than the news about your heart, there was some encouraging reports. Your copper level being so high is awesome. And 2 weeks away from the long commute and hospital visit is lovely. And feeling more optimistic and having some energy to go along with it is the best.

I imagine your spring weather there is quite beautiful.

We are hoping for a copper result back soon. It will be interesting as it will be the first since we started the more regular copper infusions. Bruce continues to feel well, despite his ever-sinking blood counts. The port is helping; he had a transfusion through it last week.

Hugs, Catherine

[Chirley]

Hi,

I had my appt for the 2nd opinion yesterday. Basically he agreed with the diagnosis of copper deficiency induced bone marrow failure although he did wonder about my last BMB still showing abnormalities even though the copper had been replenished and was in normal range at the time.

He went on to say that he reviewed all my BMB results and blood results going back years and he would never have thought of testing me for copper levels. He said that my blood test results were not consistent with known abnormalities caused by copper deficiency. He said that if I was his patient I would probably still be diagnosed with MDS with increasing blasts and still be on Vidaza. He considers my Haematologist and Neurologist to be right on the ball.

He reassured me that although my liver is stressed by the high ferritin, it should not get any worse as long as the ferritin level doesn't increase. He also thinks that venesection would be the preferred method of ferritin reduction but there is no hurry to start. I mentioned wheatgrass and he wasn't in favour because it's naturally high in zinc.

I'm having my routine blood test today, will ring the Haematologists secretary on Friday for results, hopefully the 24 hr urinary copper-protein-creatinine results will be available too.

I'm booked for more IV copper next Monday and fingers crossed that I only need one visit next week.

Regards

[Birgitta-A]

Hi Chirley,
Very interesting with a 2nd opinion in your very complicated case. I still think it is too early to know if your bone marrow will manage better when the copper level is higher.

Then you got severe neurological symptoms during the Vidaza treatment even if we don't know the reason for these symptoms. I have never seen that kind of adverse effects in connection with Vidaza.

Remember that we manage well with one third of the liver so we can have high liver tests during a long time without any other symptoms.
Kind regards
Birgitta-A

[Chirley]

Hi Birgitta,

Good to hear from you. I think the BMB I had might have been too early (January) in the copper treatment to have shown a sustained bone marrow response. I've been thinking lately that I'd like another BMB just to see what's happening now that I've been having copper replacement either oral/subcut or IV since last November. Do you think it would seem forward of me to ask my doctor for another BMB?

Regards

[Marlene]

Hi Chirley,

When someone has been deficient in a nutrient, it can take the body quite a long time to regain health. Once levels are restored, the body will prioritize how and when it will heal. In some cases, severe, long standing damage may not be reversible. Just like when treating drugs, it can restore you all the way, or just part of the way, or just stabilize you stopping any more progression, or do nothing at all.

This is very apparent with B12 deficiency especially when it involves the central nervous system. Some nerve damage is beyond the bodies ability repair. And it takes a very long time for the repairs to happen the longer you've been deficient. Nerves heal very slowly.

So finally, to your point, no, I don't think it would be forward of you ask for another BMB. But maybe, wait a bit longer til you get some iron off body. Too much iron will have an impact on your bone marrow and you may see more improvement as the iron comes off.

[cathybee1]

I am very glad your second opinion is behind you, and there's agreement about continuing the treatment. I truly hope you will be able to find the "sweet spot" for your oral and IV copper dosing, so that you only need to go in once a week.

I had a little meltdown last week. Things had been looking promising with Bruce's copper treatments, we had an appt with the hematologist who made an appt 2 months out, then on the way back home, we got a cellphone call, the family doctor referred Bruce back to a big city hospital for an updated MDS workup. Bruce's last copper was 56 (70 is normal), so it has continued to drop. All his blood lines are also low. The family doc believes that despite the IV infusions, Bruce is "clearing copper too efficiently." Bruce's serum zinc levels are also low. When this happened, Bruce had only had his port in for a month, so he was a bit crestfallen.

Bruce asked for another two weeks at a reduced copper dose, then if there's no improvement, he'll go back for a BMB and we'll make the long trip back to Los Angeles.

Otherwise, he is still feeling physically ok, and has started moving wood for winter. He's quite a guy!

[Chirley]

Thanks for your advice Marlene. It's a good idea to wait until the iron is normal but I'm still waiting for my doctor to decide when the time is right to start venesections..He certainly doesn't rush into things, my Hb has been normal for some time now.

Catherine, I'm sorry things aren't going well with Bruce. It's so disheartening when you have treatment and then more treatment and copper levels just drop consistently. It seems that keeping copper within normal levels is a time consuming and relentless task.

I'm waiting for my last 24 hr urinary copper result which I did last week. My haematologist thinks I may have nephrotic syndrome which is allowing the copper to be excreted in abnormally high quantities so I have also had a 24 hr test for protein and creatinine. I had a spot morning urine test for casts etc as well and I hope to get these results on Friday. The reason he is going down this route is that my blood protein is consistently low, the albumin is sometimes low and dipstick urinalysis consistently shows protein and haemolysed blood with no infection present.

The reason I'm mentioning this is because I wonder if Bruce has had his kidney function checked out. I don't mean his urea or creatinine because they can be normal with nephrotic syndrome, I mean actual dipstick urinalysis. This very simple test which can be done by the nurse in daycare or the doctors surgery seems to be totally out of fashion but it is also an extremely valuable and easy test. I don't understand why it isn't done routinely. I had to ask the nurse to do the test on my urine because it didn't cross my doctors mind and the nursing staff don't mention it's available either. The nurse was very willing to do the test but only after I asked for it. She was concerned when she told me that there was a LOT of protein and haemolysed blood in it. Just goes to show if I hadn't asked for it we would never have known there was a problem.

Regards

PS I just bought an iPad 2....I hate learning new technology!!

[Chirley]

I just received what I think is good news.

I was booked in for more copper on Monday and then depending on my blood results more visits later in the week but it's school holidays here again and daycare has been booked out for people from the country coming to the city for their initial treatments. So, I have been given next week off too. This makes 3 weeks off treatment in a row. I feel incredibly lucky. I am booked in for every day of the following week but that's okay, I will have had a wonderful break.

Regards

[cathybee1]

It's wonderful and necessary to get some time away from doctor's offices and hospitals. I am glad you are getting a break. Hope you've been getting to spend lots of good time with your furry ones. Things have settled back down emotionally for both Bruce and I after the flurry of activity and phone calls last week.

That is a good suggestion about the dipstick test. Bruce's bloodwork for normal kidney function screening has been ok. But we know that like you Bruce is excreting copper too quickly. Bruce also submitted a stool sample for heavy metal analysis. We are going to see the family doc next week, so the timing is right to ask about it.

Hugs, Catherine

[Chirley]

Hi, I'm writing my very first post from my iPad, I didn't realize it was so incredibly simple to use.

I'm going to the GP tomorrow for a medical exam to apply for a Disability Pension. I've been on paid leave from work for 21 months and I only have 4 more weeks left. I'm not able to return to work so I have no other options available. The pension combined with an income stream from my superannuation will be quite sufficient but it's still a big step to leave the workforce permanently.

I'm feeling a little anxious about it but decided that I will do some volunteer work. There is a new zoo opening next year and it's only about 5 minutes taxi ride away so I have already put my name down to do volunteer work in the administration area. My real passion would have been to shovel elephant $&@$ or muck out giraffe stables but unfortunately I have to be satisfied with office duties.

Regards