Copper treatment

[Chirley]

This isn't related to bone marrow failure but is related to the underlying copper deficiency.

I had a repeat gastroscopy on Thursday just to check that my stomach had untwisted completely and everything looked reasonable.

The gastro doc did find that my oesophagus wasn't working properly (full of saliva but stomach was empty) and this explains why my food still gets stuck and I vomit about every second meal. He thinks my Neuro condition is worsening and causing this problem.

Because I've had a pretty rough couple of months we aren't going to do anything about it unless I start having problems like losing too much weight or breathing in the vomit etc.

This is extremely disappointing because I've been having my copper replacement infusions regularly. It seems as if replacing the copper doesn't necessarily mean it's able to be transported effectively to the nervous system. I've also noticed lately that even with the copper infusions my blood counts never quite recover any more. My Hb used to climb to normal or almost normal levels after an infusion but now the best they go is into the low 100s.

I wish there were other people with this same problem that I could talk to. I have no comparisons, I don't know if this is the expected course.....whether I'm doing well or if I should be doing better. I don't know if this condition is terminal, if I will become bedridden, if I will become confused/demented. While the Neuro disease is unpleasant so is the uncertainty and isolation.

[Birgitta-A]

Hi Chirley,
Has the surgery (with complications) led to weight loss as you hoped it should do? Can you move better or are you still weak?

Too bad that your oesophagus isn't working properly but when you are in better condition that perhaps could be treated - the muscles near the stomach seem to hold food instead of letting it slide down to the stomach.

Even if it was positive when your counts were normal you are - lika many of us - used to live with low counts. I remember that you had hardly any neutrophils at all and received many blood txs.

As you write it must be the uncertainty and isolation that are the most difficult problems because your disease is so uncommon. As I have written before I admire your fighting spirit and the way you try to find the best possible treatments.
Kind regards
Birgitta-A

[Chirley]

Hi Birgitta, I've lost 15kgs (33lb). I haven't noticed any improvement in my mobility but my hip is hurting less.

I had hoped my mobility would improve but in reality I doubted it. I was really aiming to maintain what mobility I do have for longer so I can stay in my own home.

Things are tough here at the moment.

My mother hasn't been well and I had to contact her doctor and insist on some tests being done. I find it stressful dealing with my mothers health problems because she is very abusive and lies, and does everything to undermine her own health care. She told her doctor that she never vomits or has abdo pain or faints but she does these things every day. Anyway, long story short....they found an abdominal aneurism and a clogged artery to her bowel which is causing her to lose blood supply to her bowel. She needs treatment or her bowel could die but she is throwing her usual tantrums and I'm tired of it.

Just to top of a not so good month, a friend of the family went missing and the police eventually found him dead in his car where he gassed himself. He was 39yo.

When things are bad, they have no where to go but up.

[Birgitta-A]

Hi Chirley,
Good that your hip is hurting less and that you hopefully will maintain your mobility!

Can't your brother try to help your mother - I know that he doesn't live near her but you can't take more for the moment.

How sad with the friend who didn't want to live any longer.
Kind regards
Birgitta-A

[Cheryl C]

Hi Chirley - That is an uncharacteristically sad post from you and I'm sending you a hug. You are such a brave lady and an encouragement to others. I'm sure all of us on the forum will be thinking of you and wishing you peace and hope and a solution to your mother's problems as well as your own concerns.

[Chirley]

Yes, I feel sad at the moment but as ever, I'll get over it.

I asked my brother for help. I was direct and to the point so there could be no misunderstanding.....he refused. I'm trying hard not to harbour resentment against him for this. He is very unreliable. He told my father he would visit him yesterday for his 86th birthday, my father waited expectantly all day....he never turned up, he never phoned. I'm sure there will be some excuse where once again, my brother was the victim of circumstances beyond his control......he's always the victim and is NEVER responsible for anything. I love my brother, but I don't often like him.

I used to think suicide was an individuals right. Having a cousin in Sweden suicide last year, a cousins husband in Sydney suicide the year before and this family friend this year......it's made me realise that it's an extremely, cowardly, selfish act. The devastation left behind is horrendous. The trauma for the spouses, the children, the parents, the siblings, the friends is awful.

I think the secret to dealing with life's slings and arrows is to ignore them until the immediate pain diminishes and you can think clearly and logically. So, with my head firmly buried in the sand I'll deal with day to day life.....dog to vet for yearly needles.....order groceries for delivery....cook special meal for Dads birthday......do the laundry.....take up and hem the new curtains....clean the oven (that's one step too far)......pay someone to clean the oven.

[Birgitta-A]

Hi Chirley,
You know that in Sweden they are thinking about doctor assisted suicide if you have a very severe disease, painful symptoms and don't want to wait for death coming. Most persons in Sweden don't believe in God - very different from the US.

I have had problems with my neck - very painful - the last month. Then I have to do like you - deal with the day to day life. Too much pc work.

Tomorrow I will take my 18 yo blue persian cat to the vet. I hope she is allergic to some food - if she has some fungus infection over her eyes I can't keep her because a fungus infection is very dangerous for me. I have to take Neupogen every week to get some WBC.

Too bad with your brother. I don't Think you could do more than you already have done. Can't your mother's doctor explain to her that it is very important with treatment. The last time she was very ill she got much better when she accepted treatment.
Kind regards
Birgitta-A

[Snuuze]

Yes, pay someone to clean the oven! Or ignore it; a little more build-up won't slow down the world.

My heart breaks for you and all that is coming your way. Some times life just isn't fair, and recalcitrant family members are no help. Thank you for sharing your feelings with us on Marrowforum. It's a safe place and you let us know that it's ok to be down and to admit it.

Sue

[Chirley]

I'm finally feeling stronger mentally and physically. It's 10 weeks since my stomach surgery and I have started being able to eat without vomiting.

I've lost 18kgs (about 40lb) and I'm starting to feel the difference. My back is still painful but my hip is a lot better. My wish for increased mobility hasn't come true (yet).

I just received a phone call from my GP. My last blood test results show my Hb is 104....he is happy with this and so am I although I didn't have the heart to tell him it was almost certainly effected by haemoconcentration due to dehydration with my constant vomiting. My WCC and neuts are still low but stable and my lymphs are high. Platelets weren't mentioned so they must be okay.

I'm starting to wonder if this surgery has somehow altered my vitamin/ mineral absorption. Even though my vitamin levels have declined, it seems that my copper isn't being excreted as fast or maybe I'm not absorbing zinc and I have been able to absorb and metabolise copper better. I really don't know, but since surgery my bone marrow has been functioning better. While I was on tube feeds, they were bypassing my duodenum and going straight into my jejunem. These feeds had copper in them. Maybe I absorb copper better this way. If it turns out that having copper supplements into the jejunum is effective....I'd be happy to have a PEG placed and administer the copper at home.

It's really just conjecture, but I know my bone marrow is functioning better and I'll be grateful if it continues.

[Cheryl C]

Your positive spirit is emerging again, Chirley. So pleased you have found couple of good spin-offs from all the pain and frustration.

[Birgitta-A]

Hi Chirley,
Good that you at last feel better and that your counts are holding! Interesting that you perhaps absorb less zinc or more copper after the operation. I have friends who loose kalium that can be quite dangerous.
Kind regards
Birgitta-A

[Marlene]

Glad you are feeling better. It sure would be great if this helps with the copper issue also. Wishing you continued improvements.

[Chirley]

And now for something a bit different.

I have now got another health issue which is almost certainly auto immune. I have to go for more tests to determine the exact diagnosis, cause and treatment but it does make me wonder about the recent drop in my blood counts even with copper treatment. The counts are still pretty good but sit at a lower range than they did a few months ago.

This is all getting a bit too much.

[Birgitta-A]

Hi Chirley,
You are right - one more disease is really too much when you already are fighting so severe symptoms.
kind regards
Birgitta-A

[Chirley]

Sometimes I think.....no more..please. Then I feel terrible for the self pity. My nineteen year old second cousin has started having Neuro problems and it seems as if she might have cerebral Lupus. She has already had multiple PEs, has an IVC, has had chemo, has had blood transfusions, her hair has not grown back, she couldn't finish High School, she can't work, her mother has to help her bathe, and she may need to have a hysterectomy. Now that is sad and it puts my problems into perspective. Darned genetics....I'm so pleased I didn't have children.

On another note...I'm booked in for my portacathogram on Friday...fingers crossed for a good result.

[Birgitta-A]

Hi Chirley,
Yes, your poor second cousin has really a very severe disease but still we always hope that we will live to high age without serious symptoms and die suddenly without a long period in hospital.
Kind regards
Birgitta-A

[Chirley]

It seems my Physician and I have finally worked out a regime for copper treatment that while not keeping my bone marrow functioning normally, at least keeps me reasonably healthy.

It's been 8 weeks since my last infusions and my counts aren't good. So my Physican has determined that a week of infusions every sixth week keeps my counts at a reasonable level. They never fully recover but at least they don't drop to blood transfusion stage and my neuts only decline to between 0.5 and 1 and not into critical levels if we stick to the six weeks. I'm so happy that we have pushed the boundaries and worked out a regime that keeps my health reasonably stable while retaining some quality of life. It sure beats the two weeks off and one week on treatment that I was having.

I'm very pleased to be in "boring" category health wise.

[Birgitta-A]

Hi Chirley,
Good with the six weeks regime!
Kind regards
Birgitta-A

[Birgitta-A]

Hi Chirley,
Here is info about copper. You perhaps know that disulfiram (Antabus) is used to stop persons that have alcohol problems from drinking alcohol.
http://www.checkorphan.org/grid/news...menkes-disease
Kind regards
Birgitta-A

[Chirley]

I was discharged from hospital yesterday. I had 5 doses over 5 days of Copper Sulphate 4mgs in 200mls of Normal Saline, each run over 4 to 5 hours. I had 100mgs of Hydrocortisone before and after each dose but still developed welts. I also had Claratyne anti histamine tablets. I became very hypotensive at 78/40 to 90/50 and my pulse dropped into the 40s occasionally. My physician thought these hypotensive episodes were neurological. I was given a couple of litres of intravenous Saline to try to increase my blood pressure and it did rise to 95/55 by the time i was discharged.

While I was in hospital I noticed I wasn't passing urine very often (I didn't pass urine from midday one day until 7am the following morning and it wasn't a lot. I didn't mention this to my doctor. Since I've come home, I've hardly passed any urine, my feet are swollen and I have some slight pitting oedema on my shins. My eyes are swollen and I have developed fluid around my neck (like a double chin), my cheeks are swollen and heavy and it's making my mouth droop at the corners. I don't have a cough or breathlessness but my chest does feel a bit tight (no wheeze). I have gained 5.5kgs in 6 days.

I don't know whether to assume this is just fluid retention from the steroids (hasn't been this bad before) or whether my low blood pressure has caused a kidney problem. I feel quite tired and shaky but I don't have any nausea. I just don't feel right, but I don't want to go rushing off to see my GP if this is only going to be a temporary steroid side effect.

Any thoughts? I'm hoping this is something I can ignore.

BTW, Birgitta...that's an interesting article. It comes at a time when I might need a bit more help because I was the guinea pig for the Medical Registrar examinations when I was in hospital and my Neuro symptoms/signs have worsened since my last Neuro examination.

[Neil Cuadra]

Chirley,

Would an over-the-counter diuretic help you reduce your fluid retention while you wait to see if it's a temporary condition?

[Birgitta-A]

Hi Chirley,
If I was in your shoes I should contact my GP at once. You are much too tough.
Kind regards
Birgitta-A

[Chirley]

Thanks Neil and Birgitta. There really is no such thing as an effective over the counter diuretic here. Diuretics are on prescription.

I rang my Physician and he didn't want me to take a diuretic because my BP is too low. I was told to ring him back last night if I hadn't started passing the fluid. Unfortunately, nothing has happened. I'm still swollen but I felt like I was just being annoying so I didn't ring him back.

I don't really know how many days it takes for the effect of the steroids to wear off. My last dose was on Monday afternoon and it's now Thursday morning. I suppose what I'm wondering is at what stage does the excess fluid stop being a steroid effect and start becoming a medical problem.

At least I know to refuse that fifth dose of copper next time.

[Marlene]

Until you can get to your doctor you can try celery, watermelon , parsley, apple cider vinegar, cucumbers, hot lemon water or caffeinated drinks. These all have diuretic properties without depleting nutrients.

[Sally C]

Hi Chirley,
I totally agree with Birgitta's advice. My husband got many IV's of Solumedrol with transfusions and it never did that to him. With this going on since Mon. I worry that toxins are building up in your body regardless of the reason.
I wish you well.
Sally