Copper deficiency induced bone marrow failure

[Birgitta-A]

Hi Chirley,
Hope the liver biopsy will show that most of your liver is OK! As you know we manage well with one third of the liver.
Kind regards
Birgitta-A

[Lbrown]

Hi Chirley,

Good luck with the biopsy. I had a liver biopsy about 20+ years ago. They marked the spot by pressing a dime really hard onto my skin. I kid you not. Hopefully they will have a better system by now. You have to be in hospital and they don't let you move for hours after in case it bleeds.

Can I ask what your professor is a professor of?

Thanks,
Deb

[Chirley]

Hi Deb, he's a Prof of Metabolic Medicine from the Neurosciences Department.

I first saw him in Oct 2010 when I was in hospital and first diagnosed with the copper def. Then I saw him again Dec 2011 as an outpatient and I can honestly say he was very rude and abrupt. Then I had to go back and see him again Jan this year and I REALLY didn't want to but he was much more gentle and communicative.

I saw him again in June and he was just delightful, goes to show that you can't judge someone from one visit.

I got a bit of a rebuke from my haematologist because the Prof told me I needed a liver biopsy in Dec last year. I assumed that he would write a letter to my haematologist and tell him, but nothing happened and i discreetly didn't bring the subject up either. But when I saw the Prof in June he was adamant I needed the biopsy, so I mentioned it to the haem who said that he didn't know about it and had never received a letter from the Prof. This week my haem brought a copy of the Dec letter that the Prof sent but had never reached him (he got it from the hospital file). The letter stated that the Prof had told me I needed a biopsy. My haem was not impressed that I didn't pass this onto him last year. I felt like I was in Grade 7 and had been caught out for not doing homework. (not that that ever happened ;-) ).

I found out yesterday that the RN in charge of Interventional Radiology where they do the biopsy, is an acquaintance I used to work with about 5 years ago. He was really good to work with and a quiet, calm, really good looking gentleman as well. He phoned me and assured me that they give sedation and explained the procedure. He also said he would stay with me the entire time. I just hope I don't embarrass myself.

It's a pity I'm not capable of nursing any more because having undergone lots and lots of procedures, treatments and investigations as well as being confronted with potentially dying in the short term and also now living with a chronic illness, I feel I would make a much more knowledgeable and compassionate nurse.

A friend is taking me out to a Sports Club tonight to see an Eagles tribute band and we have to arrive 3 hours early to reserve a table for twenty of us. The venue won't pre reserve the tables and we want good seats so it's my job to sit at the table in my wheelchair and scare other people away until the rest of the group arrives. See, I do serve some purpose.

Regards

Chirley

[Lbrown]

Thanks for the explanation Chirley. I definitely think that nobody can understand chronic illness unless they have gone through it or had someone close to them go through it.

Hope the concert is great and you manage to reserve that big table! I love the Eagles.

Deb

[cathybee1]

Love the Eagles music. I bet it was fun.

Your discussion of the lack of communication between the hematologist and the Professor resonated with me. Bruce's hematologist and family doctor don't talk. The hematologist thinks supplements are suspect. And the family doc thinks the hematologist has been too aggressive in drug dosages, given Bruce has liver issues. The hematologist is head of the only hematology practice within 100 miles. And we really like our family doctor. So, Bruce and/or I are the ones that are put in the middle. It has not been a relaxing experience.

Update on Bruce's copper level. He discontinued the IV copper treatments, and is now just taking the liquid copper sulfate. Last bloodwork showed his copper at normal levels (!). That was the first time in a long time he's moved up to normal range. His ceruloplasmin level hasn't budged. The frequency of transfusions has remained the same -- approximately every month. Bruce is feeling more tired in between transfusions but otherwise managing ok.

Bruce's ferritin is in the 1400-1500 range, down from 2500. The hematologist wanted to increase the Exjade dose to bring the ferritin level down more, but Bruce declined it. Bruce's reasoning was he's doing well on the current dose and didn't want to rock the boat.

[Chirley]

Hi,

I didn't get to go to see the Eagles tribute band. My friend rang half an hour before she was going to pick me up. She had just received a phone call telling her that her brother who has metastatic bowel cancer had been admitted to hospital and wasn't expected to last the night.

You know how some people just have ongoing dramas through no fault of their own? Margaret has 4 brothers. She is the legal guardian for her intellectually and physically disabled brother. He lives in a group home in Sydney and she lives in Brisbane. He has had major problems with his legs and has had to have multiple surgeries on his legs and he also got MRSA in his wounds. Margaret has to fly to Sydney to attend her brothers hospital appointments and stay with him while he's an inpatient. Also, all of her other brothers have had bowel cancer and had to have follow up chemo. She is the only girl in the family so when her Mum got sick and subsequently passed away, the whole responsibility for coordinating care etc fell on her. Her daughter has developed mental health issues, recently separated from her husband and isn't coping, so Margaret has stepped in and been looking after her Grandson 3 weekends out of 4. She also works full-time shift work in the operating theatre, her husbands business went under and they lost their home and all their savings and still had debt. Her husband disappeared and she was left to pay off the debt. They have since divorced because she can't afford to keep supporting him. Within 2 months after their 37 year marriage ended, he had a Phillipino fiancé.

Makes me appreciate that my life is boring in comparison and I'm really grateful that she still has time to be loyal to her friends.

[Chirley]

Well, I'm back home after my liver biopsy and I'm pleased to say it was nothing to worry about

I've seen a liver biopsy done with the patient lying on a bed and a Gastro reg just plunging a rather nasty looking needle into the patients right side. I remember thinking that I hoped I'd never need one of those.

I was admitted through the pre surgical area, taken to radiology, had an ultrasound, the interventional radiologist came in and introduced himself and placed a cannula in my arm. He told me that my haematologist mustn't like me because he had to do 3 biopsies to get all the test samples .

The nurse injected the sedation and next thing I know, it's all over and I'm waiting to go to the oncology ward where I had to stay overnight. I did have to have oxygen for quite a while because my lungs are scarred from pneumonia and my oxygen levels drop if I'm drowsy. I had a little bit of pain in my right shoulder and the wound bled a bit but really it was all pretty painless.

My haematologist told me he'll ring me in a few days to let me know the results. He said he doesn't know what to do if the copper level in the liver is high and he's hoping that we don't have to cross that bridge.

One thing I didn't know was that they insert the needles between the ribs, I'm glad I didn't know that bit.

Regards

Chirley

[Birgitta-A]

Hi Chirley,
Very good that the biopsy now is done - we hope that you have enough healthy livercells to manage!
Kind regards
Birgitta-A

[cathybee1]

So glad that's behind you now.

[Chirley]

I asked to have this week off treatment because Mum is sick but I was only allowed to postpone it for three days, so here I am back in daycare.

I've just been given my liver biopsy results.

I have moderate hepatitis caused by grade 3 marked increase in hepatocellular iron stores with greatly increased haemosiderin in the Kupffer cells, lobules, macrophage clusters and portal tracts. Copper staining was negative. The results are in keeping with both transfusional overload and acaeruloplasminaemia. This is supposed to confirm that I definitely have some copper transport protein abnormality.

In treatment terms it means I have to continue to have the increased copper sulfate dose, now 5mgs/day instead of 4 and I have to have a more structured venesection regime. These findings also explain the right upper quadrant pain ive been having, my doctor told me that the liver is stretching the capsule and causing this discomfort, so I don't feel like I'm imagining it now

Until some new form of treatment comes along it looks as if I'm locked into spending a third of my life having treatment. I can't say I'm happy about it but I'm luckier than most because at least I do have an effective treatment available. I should be counting my blessings.

So much has been happening in my life, not just with me or my Mum, but one of my friends' sons was wounded in Afghanistan and she has had to go to Germany to be with him and another friend had her brother pass away last week.

Stop the world, I want to get off and have a rest.

Regards

Chirley

[Birgitta-A]

Hi Chirley,
Good that you didn't have copper overload! Too bad with hepatitis due to iron overload. Hope venesections will help!
Kind regards
Birgitta-A

[Chirley]

I don't know what's going on. I have just received the blood test results taken before the start of this cycle of copper. My platelets had dropped again and my WCC was low too Hb and RCC were fine.

The confusing thing is my copper level was double upper normal! Way too much copper and I've had another week of high copper doses since then. I not get to see my doctor unti almost the end of the next lot of copper so I'm a bit worried that the copper level will be at dangerous levels. Also, if my copper isn't low, why are my platelets and WCC dropping?

I've started getting right upper back pain with referred pain to my groin. My doctor ordered a renal USS which I've just had, and a microurine which hasn't come back yet but the nurse took a dipstick sample and it was full of blood and protein with no white cells. As I'm supposed to have renal wasting of copper I was thinking it could be causing a kidney problem with the really high copper levels.

I'll just have to wait and see but it's a bit frustrating to have to wait three more weeks, the potential is there for kidney damage to happen while I'm waiting. I just can't afford to get sick with Mum and Dad needing me at the moment.

Regards

Chirley

[Birgitta-A]

Hi Chirley,
Really too bad with low WBC and platelets when the bone marrow should be working better with higher copper levels! Good that the HGB is holding though you seem to be bleeding from the kidneys.

All metals are toxic in high doses - hope the copper isn't too high.
Kind regards
Birgitta-A

[Chirley]

I received a phone call from my doctors secretary today. She said that the doc is happy with my above normal copper levels (I'm glad he is, because I'm not). She also said that I have a small kidney stone and I need to take a urinary alkalyser and drink lots of fluids. This last bit is quite a relief because I really did think the excess copper was causing nephritis.

Lesson to myself......don't over think things.

Now, let's hope it doesn't hurt too much when I pass the stone.

I still need to ask why my blood counts are low with a high copper.

Regards

Chirley

[Chirley]

I have been having nasty abdo pain, nausea, vomiting and diarrhea with my 5 mgs of copper.

I asked if I could change my schedule from 5 days every third week to 2 days a week (an extra days Tx over the same period) and reduce the dose to 4mgs.

Well, it's backfired on me. I just received a phone call from the cancer centre to tell me that my doctor agreed to the 2 days a week but at the higher dose of 5 mgs.

I don't know what my latest copper level is but the last one was well above normal levels, so I'm a bit puzzled why I have to have this larger dose.

My neutrophils have been trending down consistently over the last 10 weeks (about 0.2 every three weeks) and is only just in normal range and my platelets have been trending down over the same period and have been falling 5-10 every three weeks but this time they dropped by 20 and is now 85. My total WCC is below normal. Hb and RCC are good and I had another venesection yesterday. AST and ALT are raised but stable but the ALP is raised and increasing with every test.

My kidney pain has gone but for some reason I still have blood and protein in my urine....another thing to follow up on if it continues. I'm probably more aware of my kidney function now that my mother is having haemodialysis three days a week. She got VRE and ESBL within the first two dialysis sessions, makes me concerned about the hospitals infection control record.

Anyhow, that's my update. Nothing exciting. I feel reasonably well, just tired all the time and various weird neuro pains and sensations and tics. It's been quite warm here until today but somehow I managed to catch a cold about 7-8 days ago and it shows no sign of going away.

Regards

Chirley

[Birgitta-A]

Hi Chirley,
Too bad that you have so much GI problems with copper 5 mg but perhaps it is better to feel ill every third week than 2 days every week.

Good that Hb is OK even when you have venesections. Perhaps the platelets have decreased more than usual due to the infection.

Hope your mother is improving when she is having haemodialysis!
Kind regards
Birgitta-A

[Chirley]

Oh dear, this is feeling more and more like Jerry Springer.

My Mum has almost stopped eating and when she does have something she vomits. I've tried to contact her renal physician but I'm not getting anywhere. The dialysis unit have tried as well and at least he answers their phone calls but he hasn't been to see Mum and he hasn't ordered any tests or treatments. I'm frustrated beyond belief.

Next to my drama. The cancer centre I go to have decided that they are too busy to run my treatment slowly so they doubled the rate. They didn't tell me what they were doing and last Friday I started having abdo pain, sweats and vomiting within an hour of starting my Tx. I have never had this before until later in the evening so I couldn't understand what was happening. I had to have more steroids and IV anti emetics. It wasn't until the infusion finished in half the time that I realized the rate had been changed. No one said anything
and I had already had the reaction so I assumed it was nurse error and decided not to say anything. I went on to have a terrible evening and night at home.

Today I had my Tx started and I took notice what rate they were running it at and when I told the nurse it was too fast and that I'd get sick, she just said that this was their decision and that was what they were going to keep doing. I refused the rest of my treatment. They rang my Doctor who is a shareholder in the centre and he agreed they could run the infusion fast. The pharmacist
agreed with me that it would make me sicker. I have declined any further treatment. This is my right, my choice and my responsibility. I am not happy with the treatment centre but I'm not placing my responsibilities onto them. Oddly enough, I feel at peace with myself at the moment but I suppose when the bone marrow failure and progressive neuro degeneration kicks in I'll feel a little less sure that I've made the right decision. I've given it a good go.

Regards

Chirley

[Birgitta-A]

Dear Chirley,
It is almost impossible to believe that they can treat you like this! You have had so much problems with the copper infusions and then they give the infusions so fast that you feel terrible even when you take more steroids and IV anti emetics.

Can your doctor really make decision like that? Good that the pharmacist agreed with you. Don't the patients have any rights? Is there no institution where patients who get wrong treatment can complain?
kind regards
Birgitta-A

[Chirley]

Hi Birgitta,

This newspaper article will explain that our HRC is a toothless tiger and investigations NEVER favor the patient.



Lisa Beckwith, with husband Simon and children Jamie, Kiara, Olivia and Keely, was wrongly diagnosed with cancer. Picture: Peter Wallis Source: The Courier-Mail
A MOTHER-of-four's dream of having more children was dramatically cut short when she was caught up in a southeast Queensland hospital's clerical mix-up.

Lisa Beckwith said she remained "shattered" and "distraught" after she was mistakenly told by staff at Redcliffe Hospital she had stage 3 cervical cancer and was advised to have a tubal ligation.

She has spoken out about her ordeal that occurred in 2006 after The Courier-Mail's exclusive reports last week revealed the death of 87-year-old grandmother Lillian Ivy Lane of Margate.

She died after receiving "excessive" doses of drugs at Redcliffe Hospital after routine knee surgery.

Mrs Beckwith, 42, yesterday told of how the birth of her daughter Olivia, now aged six, turned to heartbreak.

"Two weeks before I was to have my daughter Olivia in 2006 I was told to have a tubal ligation," she said.


EDITORIAL: Death cover-up defies belief

She said she went to see hospital staff before her daughter Olivia was born to be told she would need a biopsy six weeks after delivering her baby. I said, 'excuse me, what for?' and (they) said 'for the cervical cancer you have'."

Mrs Beckwith said she was so concerned with the news she had been told, she signed a form to have a tubal ligation after Olivia's birth.

Several months after Mrs Beckwith had the procedure she was called in by hospital staff and told there had been a mix-up.

She said she was informed her details had been jumbled up with another patient's information which showed they had been diagnosed with cervical cancer. Mrs Beckwith contacted the Health Quality and Complaints Commission, an independent and impartial statutory body, in September 2006 regarding the issues relating to her care at Redcliffe Hospital.

She also lodged a complaint which stated during her caesarean delivery of Olivia she was given an epidural that a contained a drug she was allergic to morphine.

An internal review was conducted by the hospital's senior officers and she was provided with a written response which the HQCC said was resolved to their satisfaction.

Mrs Beckwith contacted the Health Minister Lawrence Springborg this year outlining her complaints regarding poor treatment at the hospital and received a letter from him in August.

In the letter Mr Springborg stated "the results of the hospital's internal investigations concluded that the treatment provided was reasonable".

A spokesman for Mr Springborg yesterday said Queensland Health "handles hundreds of thousands of cases each year and, unfortunately, in a very small percentage of cases mistakes do occur".

[SLB]

Hi Chirley, how horrible for you! Your rollercoaster ride seems to have way too many twists and turns with no end in sight!! Is there a possibility of changing doctors/hospitals? I truly hope things turn around for you soon. Sharnie.

[Marlene]

What a nightmare Chirley. Regarding your copper infusion rate...it's possible that the nurses did not communicate the issue correctly so it would be worth speaking directly to the doctor to attempt to resolve the problem. He'll have a harder time ignoring your request when it comes directly from you. Too many times hospital staff puts their own spin on the issues when discussing a patient and gives the doctor an incorrect picture of the situation. It sounds like the nursing staff did not want to slow down the infusion in the first place and just needed the doctor to side with him/her. It's a difficult situation when the doctor has a financial interest in the center. As much as we would like to think they do what's best for the patient, money will cloud their decision making.

You shouldn't have to fight so hard to be treated correctly. Wish I could offer more.

[Birgitta-A]

Hi Chirley,
Too bad that your "HRC is a toothless tiger and investigations NEVER favor the patients". Still there must be some way for you to get the copper infusions slowly at another clinic when the "copper specialist" has decided that you need copper infusions.
Kind regards
Birgitta-A

[cathybee1]

Chirley, this is awful news after everything you've been through the past months. What a ridiculous issue for them to take this kind of stand on. What could possibly be the clinical benefit of this? Marlene's idea is a good one.

[Chirley]

LOVING FAMILY: Trudy Olive holds her new baby Sarah with husband Andrew and daughter Laura. Their son Tom (inset) died in 2010. Picture: Megan Slade Source: The Courier-Mail
THE parents of a young boy who died before he could see a doctor at a regional hospital have been heartened by a promise from Premier Campbell Newman to speed up an investigation into the tragedy.

Andrew and Trudy Olive, of Mooloolah on the Sunshine Coast, lost four-year-old Tom at Nambour Hospital on August 25, 2010.

In a 30-minute emergency department ordeal, a student nurse tended their son with faulty equipment and Mr Olive had to begin CPR when medical staff failed to notice Tom's heart had stopped.

They have since discovered that a rare, muscle-destroying condition caused their son's death, but say they will always be left wondering whether he could have been saved with different care.

With an investigation by the Health Quality and Complaints Commission yet to be finalised after two years, the family recently wrote to the Premier and Health Minister Lawrence Springborg asking why it was taking so long.


Cancer tests mix-up ends dream for mum
Secret death: Error kills hospital patient
EDITORIAL: Death cover-up defies belief

The Premier has responded, saying he was "deeply saddened" by the loss of Tom.

"My heart goes out to you and your wife. As a parent, I can appreciate how painful it must be to lose a child and I am sorry you are yet to receive the answers you need," Mr Newman wrote.

Mr Newman has written to the Health Minister and asked him to personally find out why the investigation was taking so long.

He said he had asked the minister to let him know if there were any reasons for the delay.

Mr Olive said it was a step forward.

He said he and Trudy wanted to make sure other families did not have to go through what they did.

"We are dealing with our third health minister since that day and we are still waiting for the HQCC to complete interviews of staff," Mr Olive said.

"The community expects far more."

Mr Olive said Tom's condition did not have to be fatal and they had learned that with proper observation, death can be avoided.

The Olives said a coronial inquest could not be conducted until the HQCC investigation had been completed.

They have set up the Shine for Thomas Foundation to raise awareness of the illness.

*

[Chirley]

My GP is on extended leave and it isn't known if or when he is returning so, I found a new GP today. I had all my previous records faxed to him and I gave him a copy of a letter from the Prof that I see.

I explained what happened at the cancer centre and my doctors response to it. I had already made a phone call to the drug company that supplies the copper sulphate and had confirmed with their pharmacist that the recommended rate of administration is between 0.5 to 1.0mgs/hour. This means that the minimum time my infusion should be run over is 5 hours and if I experienced side effects (which I do), it should be run over a longer time up to 10 hours. I feel totally vindicated for refusing treatment.

This new GP immediately wrote a letter to the cancer centre expressing his dissatisfaction with their decision. He has agreed to try and find me a new treatment centre and treating haematologist and if this isn't possible, he has agreed with doing everything in his power to keep me comfortable for as long as I can survive.

I had an immediate rapport with this doctor and he spent at least an hour with me when the appointment was only supposed to be for 10minutes. (my apologies to subsequent patients who were forced to wait).

He said it wasn't rocket science to figure out that IV copper sulphate causes Gastro upsets (he said it was a well documented side effect) and that
administering it faster than the recommended rate was irresponsible and perhaps incompetent.

The last time I didn't have copper treatment, it took 12 weeks and two days before I collapsed and lost consciousness. At least this time I have an idea of the time frame I have to work with.

Regards

Chirley