New treatment centre

[Chirley]

Well, so far so good. I'm on day 2 of my copper treatment.

I missed out on one whole cycle of copper and I have been having increased spasticity and spasms in my legs and feet along with spasms in my hands which is a completely new phenomena. It's been disconcerting to have my right index finger bend back by itself to the point where I've been sure it's going to break but luckily that hasn't happened.

I had a blood test yesterday and surprisingly, my platelets are in the normal range for the first time in many, many months. All my other full blood count results are normal too. I don't have the copper level yet but I expect it will have dropped quite a lot. I can't figure out how my FBC has become normal when I stopped the infusions but I'll take what I can get

I've been told that this centre is closing for 4 weeks over Christmas and I will miss out on another whole cycle of copper. I can't say I'm pleased about it but at least I have been given a lot of notice. The communication here is a lot better.

This centre is a lot less busy and the nurses have more time to chat, the doctors seem to be less stressed, the physical environment is quite nice. There are electric recliners, LCD TVs for each patient, the toilet facilities are a lot easier to use for someone like me with mobility issues. There are IV pumps for each patient (they used to have to hunt for them in the previous centre) and every patient has a BP machine (no sharing).

The best part is that it takes 5 minutes to get here from my house. I come by Ambulance transport but I think I'll start catching wheelchair taxis so I can be more independent.

Regards

Chirley

[Neil Cuadra]

Chirley,

I'm glad to see that you started a new thread. It reinforces the feeling that you have a new start with a new facility that will do things right.

[SLB]

That sounds great Chirley. What is the treatment centre called? Is it part of Logan hospital? Hope this means things are getting sorted for you.

[Chirley]

Hi Sharnie, yes, it's the Logan City Hospital Day Therapy Unit.

I had a chat with one of the nurses today and she saidvthey are disappointingly, underutilized. They dont have an oncologist, just the one haematologist, so they don't do chemo. They do infusions for immunologists, neurologists, rheumatologists etc.

Their main work consists of elective blood transfusions, intragam, iron infusions etc.

They are hoping to expand their services in the near future but they have to get more medical staff to agree to visit the centre. All the RNs are already oncology and chemo trained so they are being used below their qualifications. Apparently they rubbed their hands in glee when I turned up needing such a lot of ongoing care.

My Mum has been back in Greenslopes this week. She went for dialysis on Monday and her permacath wouldn't work so they admitted her to insert a new permacath. I'm happy to say that the care she received this time was wonderful and they communicated with her better so she knew exactly what was happening and when. She is coming home this evening after her dialysis.

Regards

Chirley

[cathybee1]

Nice people and 5 minutes from home! Awesome!

[SLB]

That's great Chirley..must say I have never heard of it. That would be closer for me than greenslopes. I haven't had any real problems at greenslopes,well not with nurses anyway!sometimes the pharmacy can be so slow mixing the drug! And twice this cycle they have managed to send my chart back to patient records! Just a pain really. Glad your mum is getting better care however such a shame we have to 'shop' around for a hospital/ centre just to receive good medical care! Hope things continue to look up for you

[Chirley]

I went for my final dose of copper for this cycle after having the weekend off. I had my blood test taken because the NUM of the unit was accessing my port and noticed petecheii that werent there last week. The platelet count was good but my WCC was above normal (frustrating when they don't give you numbers) and is the first time in 10 years (at least) that it has even been more than low normal. My Hb was also described as "good".

I have had a stomach bug over the weekend and have a couple of mouth ulcers and a stye on my right eye. I have a sore or something in my right nostril as well. The NUM and all the RNs commented that I looked pale, tired and sick. Frankly that just about hit the nail on the head with how I feel. My BP has fallen to 84/40 again.

It's actually annoying to have these little things wrong, feel unwell and have reasonably normal counts.

Is it possible that the stress of my own and my parents situation over the last two months could cause these petty little infections and fatigue? I'm starting to believe that psychological stress can cause physical problems.

I'm kind of relieved that my WCC can respond to infections...at least that is a major improvement.

Regards

Chirley

[Birgitta-A]

Hi Chirley,
Very good that your bone marrow seems to be satisfied with getting enough copper after all these years with hardly any copper at all.

The neuro-system is far more difficult to repair.

Too bad with infections! It is true that stress will have a negative impact on the immune system.
Kind regards
Birgitta-A

[Chirley]

My copper level is well within normal levels (23...13-25) but my ceruloplasmin is still very low (no number supplied ).

I am getting worse neurologically. I have been getting worse slowly over the last few months but the rate of deterioration has increased. I have severe pain in my hands and feet which feel soooo cold they are burning numb. My legs have stiffened again and I'm having trouble bending my right leg at all, my feet have turned in and are pointed. I've only ever had loss of sensation, clumsiness and weakness in my hands but for the last couple of weeks I've been having spasms/cramping where my fingers want to bend backwards and feel like they are about to break. My face has started getting random twitches and I get sudden bouts of vertigo which make me sick.

The new haematologist has made it clear he won't monitor my neuro condition so I haven't spoken to him about it. I've gone to my GP and he is happy to
prescribe and monitor blood levels of any drug that a neurologist orders. There's the rub......my old neurologist says that there are no drugs that will help and that I'm going to get worse...he is no help at all. I've tried to make appointments to see other neurologists. In the public hospital system it will take 2 and a half years to get an appointment. Some private neurologists have closed their books and not taking new patients, some are taking appointments for next April or May 2013 and the one and only one I can get to see this year (28thDecember) is on Wickham Terrace in Brisbane and because I'm now in a motorized wheelchair that really isn't an option. Logistically, I just can't get there.

My usual neurologist has been extremely pessimistic about my prognosis from
the start and he has told me numerous times that I'm going to die a horrible lingering death (his words). I desperately want another opinion from someone with a less pessimistic personality (am I just being in denial?).

This neuro told me that while I have low ceruloplasmin levels I will never be able to utilize the copper even if the blood levels are high and that's why I'm still deteriorating neurologically. If this is true, then, why is my bone marrow able to get the copper and function normally ?

The GP told me that marijuana would be good for my symptoms but it's illegal here. I've never had an illicit drug in my life, I've never smoked and I drink moderately on occasion. I don't move in circles where I even know anybody who uses marijuana let alone where to get some. My 85 year old Dad thinks I should try it by baking it in biscuits but my upbringing, my fear of drugs and the fear of being caught makes me very reluctant but it might be a better option than taking heavy duty narcotics and relaxants.

I feel so lonely in this disease, I really don't fit in to any support group and even the doctors admit to knowing very little about how to help me.

Feeling very down today, but that's how I feel every time I've had anything to do with my neurologist.

Sorry to whinge.

Regards

Chirley

[Birgitta-A]

Hi Chirley,
Eventually there is nobody who knows how to really treat your neuro-symtoms but at least you should have treatment for the spasticity.

You have perhaps tried massage, vibration and functional electrical stimulation/biofeedback. Then there are some drugs (they don't mention marijuana) that could have some positive effect.
http://emedicine.medscape.com/articl...view#aw2aab6b6
Kind regards
Birgitta-A

[Chirley]

Thanks for your reply Birgitta. I have tried massage but unfortunately it just stimulates spasms as well as the spasticity. I have not heard of or tried the other things you mentioned.

That article that you sent me was very enlightening. There ARE things out there that can help, I just have to find someone willing to try them for me.

I've started re taking the Diazapam, it helps a little sometimes but at least it mellows me out so I don't stress about it as much. I realize it's not a long term solution and if I keep taking it I'll build up a tolerance.

One thing that did ring bells was the description of the different types of spasticity and mine seems to be the TBI type but I haven't had a traumatic brain injury....I have extensor spasticity where my legs (mainly my right) stick out straight and won't bend, then my feet bend in towards each other and
point, sometimes my toes bend under as well. Occasionally when I finally loosen my legs enough to bend at the knees, they lock in that position and then I can't straighten them.

When my leg sticks out straight at least my cat takes the opportunity to use it to rub her back along, so it's not a complete waste, there is some use for it.

Thanks again for the link, it's given me some hope.

Regards

Chirley

[Birgitta-A]

Hi Chirkey,
Yes, I think you have mentioned before that Diazepam did help a little - it is one of the best drugs for tension but as you are aware of there is a risk for tolerance.

In Sweden they rubbed muscles and joints with cat fur 100 years ago to keep them warm and decrease pain - your cat is perhaps treating you at the same time as her back is rubbed.
Kind regards
Birgitta-A

[Marlene]

Hi Chirley,

Just a comment on the copper...your high iron can interfere with copper and zinc. It's common knowledge that proper mineral balance is important but no one really knows or understands the complete picture. High iron is disruptive to maintaining this balance. Hopefully, as you eliminate more iron, things will settle down.

Brigitta's comment about the cat treating you is interesting. I have been reading quite a bit about restoring voltage to the body in order to heal. And animals can sense areas in the body needing help (those that are low in voltage). By physically connecting to you, they are donating electrons to your body to help boost your energy. This happens with all body work like massage, reflexology, reike, etc. Energy/electricity is often overlooked as an integral piece of health. I just finished Dr. Jerry Tennant's book "healing is voltage" which helped me make the connection between the various alternative modalities we used over the past ten year to restore John's health.

Take care,
Marlene

[Chirley]

I have just returned from my GP and I was given a copy of my last BMB results from early 2011.

The conclusion states....the dysplasia persists but has reduced (no vaciuolation, reduction in ring sideroblasts). The counts have also improved with the replacement of copper, however, it still shows that the patient has an underlying myelodysplasia.

Earlier in the report it makes note of dysplastic megakaryocytes which is not a feature of copper deficiency.

Blasts were noted to be high normal at 3%. This was down from 8%.

Now I'm really confused. Did the copper alone improve the MDS? Is it, has it ever been MDS? Did the Vidaza work?

Does any of it matter as long as I'm well?

The GP has referred me to a Haematologist at Greenslopes as well as to a General Physician. The new treatment centre is excellent for receiving the copper treatments but unfortunately the medical input is rather poor. I have seen the medical staff four times, each time it has been a new doctor and each time they didn't know what my treatment was for or how to interpret the blood results. Yesterday the doctor told me that my blood tests were excellent. I asked what my copper level was, she said 30!! I said that it wasn't good then. She asked why and I said that it was way above normal levels and it needed to be correlated with the ceruloplasmin levels. She said "oh, well, that's still well below normal"! I'm presented with ...was my blood test contaminated and useless (has happened a lot) or is the copper level really high, and with a low transport protein this makes a high level more toxic. Then I asked how my liver enzymes were. Turns out they didn't do that test! The hospital pathology is trying to save money so they now only do liver enzymes as a specific request rather than as part of a biochemistry profile and the previous doctor didn't think of asking for it.

I'm supposed to be on a 3 weekly venesection program to reduce the iron burden on my liver but the doctors at this treatment centre refuse to order them. My ferritin has come down below 1000 and they are happy with that but unfortunately, my liver isn't. The GP told me today that if I don't have the iron reduced I will definitely progress to cirrhosis which is irreversible.

Looks like I'm on the merry-go-round again.

At least now I have a very competent and enthusiastic GP looking out for me.

Regards

Chirley

[Birgitta-A]

HiChirley,
As far as I understand it is hardly possible to see any difference between bone marrow cells in MDS patients and copper defiency patients.

Very strange that they don't do liver tests in patients where they should follow the test results!

Too bad that you can't have venesections!

Hope you feel as well as possible!
Kind regards
Birgitta-A

[Chirley]

Hi, my treatment has been going well at the new centre, I haven't even experienced the abdo pain and diarrhoea I used to get every time at the previous centre.

Yesterday I was having my copper infusion when my port started hurting. I called the nurse and when she flushed the line it became obvious that the needle had moved and the copper had been leaking into the tissue. No big deal. The needle was replaced and the copper started again. About half an hour later I started getting itchy all over, then I got a rash. At first it was only on my arms but it progressed to my whole body except from my knees down. The infusion was stopped and I was given more steroids and anti histamines. After an hour the rash had subsided and the copper was restarted. Within a half hour the rash was back very badly. It was originally like hives but then they all merged into each other and became raised and hot. It felt like my skin was burning. They rang my doctor who said to stop the treatment and sent me home with antihistamines.

I returned for more treatment today but they wouldn't give it to me because the rash was still there and still quite bad. The haematologist came and ordered 50mgs prednisilone daily for 5 days, then stop, he said I didn't need to taper.

He is going to contact the drug company to see if the have changed the components. If the drug has not been changed, he is not going to let me have any more copper. He admits that this will be a big problem. I feel kind of confused but there is nothing I can do but wait and see.

I also spoke to the NUM about an article I read where they transplanted male donated bone marrow to females and then found Y chromosomes in the recipients cerebral tissue, thereby proving that transplanted stem cells can engraft in nerve tissue. We both decided that if I can't have copper infusions any more, that this option should be explored. My question is...if BMTs fix chromosomal abnormalities caused by MDS or AML, would it fix my chromosome deletion too? Am I being silly even considering it?

Any opinions?

Regards

Chirley

[Birgitta-A]

Hi Chirley,
It is difficult to understand that a small amount of the copper solution outside the vein could cause such a severe allergic reaction. We hope the prednisone will help and that you can tolerate that dose (I can´t sleep if I take more than 20 mg).

Very interesting that transplanted stemcells from blood or bone marrow can engraft in nerve tissue. Still we don't know if your chromosome deletion has caused your symptoms or if they depend on copper defiency.

Hope the rash will disappear and that you will be able to continue the treatment!
Kind regards
Birgitta-A

[Chirley]

Hi Birgitta, they don't think that the copper leaking into the tissue caused the allergic reaction but just a coincidence. I have developed a red, sore swollen area where the copper leaked just like when I was giving myself injections of copper in my stomach but I'm not concerned, I'm sure it'll get better.

I have also been given a copy of a letter written by the Prof of Metabolic Medicine I used to go to which he wrote to my haematologist. This letter states that he was of the opinion that the chromosome deletion that I have has caused the copper transport problem and not the other way around. He admits that this is just his opinion because it is so rare that it is impossible to be certain.

I know this is a really naive question and I am displaying my ignorance but, if BMTs can fix chromosomal abnormalities, why don't they transplant children with life threatening diseases because of chromosome abnormalities? Diseases like Angelmans etc.

I discussed my concerns about not being able to have more copper with a haematologist (he was covering the regular doctor who was on leave), I explained that the ensuing bone marrow failure didn't concern me as much as the neurological deterioration. I admit that I'm very frightened of increasing loss of function, becoming bed ridden and developing dementia, not to mention the neuropathic pain which is really hard to treat

I have to wait two weeks before I will find out if I can continue the copper treatments or not. It's going to be a long, stressful two weeks, perhaps I need to make an appointment with my GP so I can talk to someone objective. I can't discuss this with my family, I feel I have to protect them from worries, they have enough of their own.

If you didn't have bad times in your life, you wouldn't appreciate the good bits.

I have a new member of the family from the Council Animal Rescue.....Baby Boots (already named by the Council). She already has my dog and old cat under her spell....she's really funny and energetic.

Regards

Chirley

[Birgitta-A]

Hi Chirley,
Very cute cat!

As far as I understand patients with Angelman's disease have the chromosome aberration in all cells in their body from conception.

Patients with blood cancer diseases like MDS have developed their chromosome aberrations (if they can be detected) in the stemcells during their lives. Then it is possible to destroy all their stemcells and transplant new healthy stemcells that hopefully will engraft in the bone marrow.
Kind regards
Birgitta-A

[Chirley]

It's been a hard two weeks of waiting.

I had an urgent appointment with an Immunologist last week. The consensus of opinion is that I have become sensitised to the CuSo4.

I am currently undergoing a desensitisation program. This is Day 1.

I started last night with 4mgs of Dexametasone then a further dose of 2 Loratadine tablets this morning, then I was started on the IV Copper. They have reduced the dose from 5mgs in 500mls to 1 mg in 500 mls and are titrating the rate up over a number of hours until I'm eventually on 80mls an hour. I'm going to be here for a very long time. Later this afternoon the Daycare NUM will phone the Immunologist to get new orders for tomorrow.

The Immunologist explained that the desensitisation process can involve very low dose exposure over a long period of time, so I don't know if or when I can get up to a therapeutic dose again but I'm just happy to be offered a treatment at all.

There was some talk that I may have had a clot in my port (I had my port needle dislodge last time) and when my port was flushed it caused the clot to move through my heart and break into micro embolisms which can resemble a rash. We are hoping this was the cause but the doctors are not willing to take a chance and are treating it as a drug sensitivity, given my previous anaphylaxis history.

I had a frank talk with the Immunologist about the consequences of not have Copper treatment and we had a talk about appropriate palliation and end of life care in that circumstance. I told him that I want to die as quickly, painlessly and peacefully as possible if that situation arises. It was good to finally discuss my needs, wants and fears with someone who is in a position to make sure they are met.

So far today, all is good with no reaction at all, fingers crossed.

Regards
Chirley

[Birgitta-A]

Hi Chirley,
Hopefully you will be able to continue with the copper treatment!

A short time after my dx I wrote a paper to my children and told my doctor about end of life. When I am really ill I only want to be taken care of - no examinations or treatments.
Kind regards
Birgitta-A

[Chirley]

Hi, well my desensitisation regime was interrupted when the pharmacy "forgot" to order the copper. I started the regime all over again and had a slight reaction when I was given 3 mgs over six and a half hours (slight rash, skin prickling).

Next day I started 4mgs over six and a half hours but broke out in widespread hives etc.

For now the plan is to limit my dose to 3 mgs per day for 5 days every 3rd week. I doubt my blood copper will be stable at this dose but I'm happy to be having any treatment. I am still on Dexamethasone on treatment days followed by prednisilone. I also take Clarityne and IV Phenergan.

I slept for 11 hours last night (due to the Phenergan) and I feel great today. Better than I have in many weeks.

This new treatment centre has gone above anything I would expect. They have been wonderful and even ring me at home to make sure that I'm well.

Amazing considering this is a free public hospital and its the best treatment centre I've been to. They have even supplied me with a state of the art electric bed instead of the electric reclining chairs just so my legs don't spasm or stiffen as much.

Regards

Chirley

Oh, forgot to mention, for some reason my urea, creatinine and eGFR have become abnormal....I don't know whether it can be the steroids, the allergy or something else but it's just a matter of waiting and see if my kidney function changes.

[Neil Cuadra]

Originally Posted by Chirley

I slept for 11 hours last night (due to the Phenergan) and I feel great today. Better than I have in many weeks.

Chirley,

When you get the benefits of a really good night's sleep that results from medication it's one of the rare cases where side effects are very welcome!

This new treatment centre has gone above anything I would expect. They have been wonderful and even ring me at home to make sure that I'm well.

Amazing considering this is a free public hospital and its the best treatment centre I've been to. They have even supplied me with a state of the art electric bed instead of the electric reclining chairs just so my legs don't spasm or stiffen as much.

I'm very glad about this. It's such good news after your past experiences.

[Birgitta-A]

Hi Chirley,
Yes, perhaps will your bone marrow manage with a low dose of copper.

Good with the new bed!

Hopefully the kidney tests will be OK next time they are controlled.
Kind regards
Birgitta-A

[Chirley]

Hi, I had Dexamethasone, hydrocortisone, prednisilone over the last few weeks. I was told I didn't need to taper but I'm not feeling well.

My last steroid was Thursday night (it's Saturday am here). I had a mild upper back pain all day yesterday but overnight I developed BAD thoracic back pain, headache (mainly occipital) all my teeth are aching, my sinuses feel blocked and heavy, my legs are swollen, my feet are swollen.....in short I feel very much like I'm getting the flu but this isn't our flu season. Oh, I forgot to mention my chest is wheezy when I breathe out.

I've Googled steroid withdrawal and this doesn't sound like it.

Maybe I've managed to catch some virus due to the steroids.

I don't know whether to be concerned or not.

I really don't feel well.

Regards

Chirley