Notes from the UK PBC Foundation Conference

Subj: UK Conference Notes

Date: 10/22/99 3:47:37 PM Central Daylight Time

From: caroline_marks@ (caroline marks - England)

Notes from the UK PBC Foundation Conference - Queens Medical Centre, Nottingham - Sunday 17th November 1999.

Speakers: Professor  James Neuberger from Queen Elizabeth Hospital Birmingham, and Dr Steve Ryder from Queens Medical Centre, Nottingham.

These are notes that I took while attending the conference and are purely for interest and discussion purposes only. I was not appointed to take notes and the below is not my opinion but the opinions of the above mentioned. I do not necessarily agree with everything that was said and do not wish to offend or upset any other person with this text. However, I do hope that you will find it interesting and I am sure that it will spark a lot of discussion. These were the main topics covered:

NONE-PRESCRIPTION DRUGS:

• Brufen and aspirin are NOT recommended for pbc sufferers. Paracetamol tablets considered safe to take, 4-5 a day and won't damage the liver as most people with pbc have good liver function (unless you are very jaundiced). I

• f you are in pain then it is better to take aspirin and be relaxed about it than to be in pain.

• Antibiotics are considered safe to take.

DIET:

• If you are jaundiced, fats may make you unwell.

• Eat what you fancy, but eat sensibly and have a balanced diet.

• For some people, small, frequent meals are better.

• Neuberger does not consider any dietary measure are helpful for pbc.

LETHARGY:

• Cause unknown:

• Mark Swain (Canada) suggested that there was an abnormality in the pituitary gland in pbcers. He did some interesting work on rats. Neuberger considers this irrelevant, but admits that it is a start at discovering the cause of the lethargy.

• All sorts of studies have been carried out but so far nothing has been discovered.

• You should try to eliminate other causes of lethargy:

  • 1. depression, a common cause for lethargy and common in people diagnosed with a serious condition.

  • 2. Review any drugs you are taking as medication (ie anti-histamines) can cause lethargy in some cases, but is relatively uncommon.

  • 3. Thyroid problems are another cause for lethargy and 1 in 5 pbc sufferers have this condition. Have your thyroid checked as this can be treated.

  • The only advice is to pace yourself.

• It is known that the lethargy is definately due to the liver because as after transplant the lethargy disappears, but they don't know why. Anti-sickness drugs have been used with little or no success for treating lethargy.

ITCHING:

• The level of itching does not imply the aggression of your pbc.  You may have itching or you may not.  It is irrelevant to the stage of your pbc.

• Questran and Questran Lite are the usual treatment for itching.

• Rifampicin an antibiotic used in the treatment of TB, is sometimes used but this has side-effects: colours the urine, tears and saliva orange. Other possible side-effects (in addition to those common to the group) - loss of appetite, jaundice, and inefficiency of oral contraception.

Why do you itch?

• They do not know. Originally it was thought to be caused by the build up of bile acids but they no longer think that is the case. There is a suggestion that it is caused by opiates (naturally occurring). Drug addicts also get severe itching and Tony Jones, Holland, has been using the withdrawal drug Naltrexone. (http://www.well.com/user/woa/revia/reviafaq.htm.) (FAQ's) He admits that there are raised eyebrows for people using this drug as it is used for drug addicts.

• What are the side effects of naltrexone?   In the largest study, the most common side effect of naltrexone affected only a small minority of people and included the following: nausea (10%), headache (7%), dizziness (4%), fatigue (4%), insomnia (3%), anxiety (2%), and sleepiness (2%). These side effects were usually mild and of short duration. As treatment for alcoholism, naltrexone side effects, predominantly nausea, have been se vere enough to discontinue the medication in 5-10% of the patients starting it. For most other patients side effects are mild or of brief duration. One serious possibility is that naltrexone can have toxic effects on the liver. Blood tests of liver function are performed prior to the onset of treatment and periodically during treatment to determine whether naltrexone should be started and whether it should be discontinued if the relatively rare side effect of liver toxicity is taking place.

DRY EYES/MOUTH/VAGINA:

• Only suggestion was treating with artificial tears , etc.

ALCOHOL:

• In Hepatitis B or C, drinking will make liver damage worse as the liver cells themselves are damaged. But in PBC the cells themselves are not damaged, therefore:

• Neuberger feels that if you have pbc and no jaundice then small amounts of alcohol may do you no harm, indeed they may be beneficial. Once you become jaundice you would need to review this. This is purely his own, prejudiced opinion.

LIVER BIOPSY and PBC STAGES:

• This is a very controversial subject and there is great uncertainty amongst the medical profession regarding this subject.

• Neuberger feels it is not always necessary to perform a liver biopsy in pbcers. There are risks attached. It is used to confirm diagnosis, but if you have the symptoms of pbc and have had an ultrasound and you are a classic case for pbc with no other conditions, he feels that a biopsy will not tell you anything you don't already know. He suggests you ask WHY you are having a biopsy.

• As the average liver weighs approx. 1 and a half kilo grammes and only a 55,000 part is biopsied, Neuberger feels it is difficult to tell the condition of the liver from the biopsy.

• Livers removed after transplant from pbc sufferers have been studied and the livers have shown different stages.

• The biopsy will not give you a prognosis and no indication of the outcome of your disease.

• Doctors are only able to estimate about five years ahead and the biopsy will not tell you how long you will live.

• Neuberger feels there are better ways of assessing the condition of your liver: ie BILURUBIN levels. When these levels run high in your blood you know that the disease is getting worse.

• Yellowing eyes can be misleading as eyes can appear different colours at different times, different mirrors etc. He suggests that you try not to keep watching for signs of yellowing in your eyes. Biopsies can be used as a way of monitoring treatment, but should always be done with caution.

AMA's:

• When you have ANY bug, your body supplies a defence. Your body sees it as protein that is foreign so it makes antibodies to get rid of it. We have different antibodies for different things, flu etc. Eric Gershwin (USA) has discovered a presence of E2 antibody in pbcers. This is a useful marker of the condition, found in 95% of sufferers and rarely seen in any other condition.

• There was a blood screening programme carried out in the North East of England as part of a study on thyroid testing and pbc was picked up from the tests. This is when it was found to be more common than originally thought. Twenty years ago, only very ill, yellow people were diagnosed but now it is picked up earlier because of screening. It is noticed at regular health screening etc and men are picked up in this way.

• There IS an increase in pbc, but they are not sure if this is because of the screening. Neuberger feels that the increase of family members having pbc is not genetic, ie as in haemophilia, but because it is diagnosed in one person in the family so other members of the family are aware of the signs etc. PBC is not necessarily present in both identical twins where one twin is a sufferer.

TRANSPLANTS:

• Good for the right people at the right time.

• Not a cure for everyone.

• Life after transplantation can be good, but never normal.

• Many pbcers do not receive a transplant and will not need to.

RESEARCH:

• Looking for the cause:

• The ultimate aim is for no more transplants to be necessary. A lot of money is going into transplant research but Neuberger wishes more could be spent on avoiding this eventually.

Does PBC have an infection as the cause?

• It was mentioned that when people move, ie from England to Australia, they will take on the diseases of the place they move to. There ARE clusters of pbc. David Trigger (deceased) did a study into the water supply in areas of Sheffield where pbc was more prevalent. Neuberger is cautious about this.

After transplant pbc can return. Why? Is it bacteria, virus, fungus?

• Neuberger and his team took bile duct cells from pbcers livers and grew them in culture dishes. They found that E2 was present on the membrane of the cells in pbcers and not on none-sufferers.

• They are looking into a viral connection. There is a strong suggestion of this but they are not sure. They have a long way to go and may be going down the wrong road.

HRT (and BONE THINNING):

• Ryder strongly recommends HRT for the first five years of the menopause when it is most beneficial for prevention of bone thinning.

• HRT does have contra-indications but he thinks that it should be given as it will help against bone thinning and that therefore any negative effects are outweighed.

• He was quite clear that you should only stay on it for about five years to slow down the bone thinning process which is dramatically increased at menopause. Do not worry if you have been on it for longer, it is purely that the benefits have already been gained and he feels it is pointless to stay on it longer. He recommends that you have a low oestrogen HRT.

• He did not mention the other benefits women feel from HRT in this instance.

• Ryder also strongly recommends a bone density test when you are first diagnosed with pbc and said he tests all his patients for bone thinning and monitors regularly. If you are diagnosed before menopause you should be checked again at the onset of the menopause. If you are in doubt about having started the menopause a blood test will confirm this.

• Calcium is recommended for those only on a restrictive diet, ie vegetarians, he thinks that a balanced diet is sufficient. He recommends only taking calcium if there is evidence of bone thinning.

• Steroids can increase bone thinning but in certain cases are beneficial to pbc.

• Extra fluoride is not recommended for this same reason. After fluoride treatment the bones will look thicker on x-ray, but are actually thinner. Ok in toothpaste but not as extra in tablets etc.

• Calcitonin, a bone building element only makes a marginal difference and there are side-effects. (http://www.tirgan.com/calciton.htm)

• Bisphosponates (http://www.theberries.ns.ca/Archives/osteo-steroids.html) taken for a long time can have an improvement of 5-10% in bone density, but has side-effects and is not suitable for everyone. This treatment is given over 2-3 years and can be repeated. He recommends weight-bearing exercise, if possible, ie walking. Smoking is obviously not recommended. Keeping active is important.