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Birmingham  Meeting

12.00 Saturday 10th March 2012

The Institute of Biomedical Research Building at the Medical School, University of Birmingham.

Professor David Adams MD, FRCP, FMedSci

Professor of Hepatology and Director NIHR BRU Centre for Liver Research

Q&A Session

   Is Modafinil now being used as a standard treatment for fatigue?

   How relevant is it if there are other autoimmune diseases in the family?

   Is there any information on the research being done in the use of Vancomycin (an antibiotic) in ‘pausing’ PSC?

   My son is on Rifampicin for itching. I’m concerned about him taking this long-term. Apart from this and Questran, what else is there?

These are just some of the excellent questions posed to Professor David Adams, James Ferguson and Gideon Hirschfield on 10 March 2012 at our Birmingham meeting. Attendees at the meeting had the amazing opportunity of tapping into this expert panel’s vast experience of all things PSC. Feedback following the meeting showed that this Q&A session was invaluable to those present, as each question was answered thoroughly and clearly. A huge thanks to David, James and Gideon for giving up their Saturday afternoon to come and talk to us.

PREDICTING THE COURSE OF PSC

Q: My wife suffers from a dominant stricture, which for the last 12 months appears to have been reasonably stable. Is it possible to extrapolate out to get an impression of how confident she can be that it will remain stable, or is it impossible to predict?

A: (JF) Dominant strictures are difficult because firstly - what is causing it – is it PSC or something else? And then it’s about how you manage it. Do you have an ERCP, or a stent, or a dilation of the stricture? And then, once it is stable and treated, what happens long term? With PSC it’s very unpredictable, and I think it could remain stable, or it could get worse. It’s just about keeping a close eye on things.

(GH) There’s a difference in how the world manages this. If you’re in North America, you probably won’t get an ERCP as much as if you’re in Canada or Finland. There’s a spectrum of how doctors manage strictures. They’re very hard to diagnose because they’re actually very small.  In Germany they manage a true dominant stricture quite aggressively, because then you prevent or delay some of the liver scarring. Essentially we can’t predict the progression.

(DA) Just to come back with an anecdote: one of the first patients I saw back in 1994 was a guy in his 60’s who presented with a dominant stricture, which everybody said must be a cholangiocarcinoma. We investigated him, biopsied it and it turned out to be PSC, and when we looked more closely he turned out to have IBD as well. He was 62 then, and he still comes to see me now, once a year, still with the dominant stricture, and his liver tests are completely normal, and his disease really hasn’t progressed over that 15 year period. It’s impossible to predict. I think you should look on the positive side, and not expect that there’s going to be a rapid progression.

Q: Is there any correlation between the stability of it, or it getting worse and the chance of it leading to cancer?

A: (DA) As you know, there is a risk of cancer, regardless of the inflammatory activity, which is what we are talking about. When the stricture causes symptoms, it’s because you have more inflammation and more infection there. That probably does make you more susceptible to cancer, but the stricture needs to be screened and if it’s stable then the risks are lower.

(GH) A majority of patients, say 30-50%, who present with cholangiocarcinoma and PSC, present at the beginning; that’s because of your genes – if you’re going to get it, there’s not much you can change. And then after that it plateaus out. It tends to be pretty obvious at the beginning, after which the risk is not zero, but about 15% will get cholangiocarcinoma. So the majority of patients do not get it. If you don’t have IBD, the risk of a common bile duct stricture becoming cancerous is actually quite low.

Q: One of the things that people with PSC always want to know is ‘How cirrhotic is the liver?’ I heard about a Fibroscanner which gives a good indication of how cirrhotic the liver has become. Can you speak about this and how useful it is?

A: (DA): As far as cirrhosis and fibrosis goes in PSC, it’s really quite complicated. In some of the diseases where you get cirrhosis, the fibroscan is quite helpful, because we can predict the stage of the disease by the amount of scarring there is in the liver. In PSC it’s not as easy as that because all the scarring is in the bile ducts, and the cirrhosis can be quite patchy within the liver, and doesn’t always correlate with the amount of symptoms and problems the patient gets. The relationship is more complicated in PSC. As far as working out how much scarring there is in the liver, with the fibroscan, we know that people with cirrhosis from fatty liver disease, or from Hepatitis C or from alcohol, we’ve got a good idea how useful it is in monitoring the condition. We don’t know in PSC, and one of he studies that James wants to set up is looking to see in PSC whether the fibroscan is any use at all.

(JF): We’ve got 3 fibroscan machines here, and as David says, they have been useful in other diseases, but we don’t know how useful they are in PSC. One of the difficulties is that some PSC patients have a lot of fibrosis in their bile ducts, but there are different types of PSC. Some people have a more cirrhotic picture, and that’s what makes it difficult to interpret. But yes, we are going to start doing it to see if it is useful.

(GH): Patients always like to know what stage their disease is at, assuming that’s the basis on which the clinician will decide what to do. However clinicians like to how the liver functions. When I see a patient, I’m not so worried about the stages, because he’s going to be continually followed up. It turns out that when liver doctors have looked at patients they are now dividing cirrhosis up into many stages. Because we are diagnosing earlier, we see lots of patients who’ve got cirrhosis who’ve got completely normal liver function and are decades away from needing a new liver, or may never need one. I think fibroscan will probably be useful in PSC eventually but it will take us a while to give you some information that will really help. Concentrate on how your liver functions, not how it looks.

(DA): One of the things we are developing in a research program here is using some new blood tests to see if we can use those to monitor both the activity of the scarring process and the inflammation. We’ll be looking at those in a range of different diseases including PSC. So it may be that in the future, we’ll use a combination of blood tests, fibroscans, and also another area that we plan to develop here, using magnetic resonance, MRI scans, where you can look at the whole of the liver and divide it into areas of scarring. I think in the future we’ll have much better ways of assessing the overall scarring in the liver. As Gideon says, the principle thing is how the liver is functioning.

FATIGUE

Q: Is Modafinil now being used as a standard treatment for fatigue?

A: (GH): The short answer is no. Modafinil is a stimulant. It has been used by psychiatrists for various symptoms. It does in some cases help with fatigue. There are a couple of studies from Newcastle of Modafinil in PBC. They now have stopped using Modafinil, which I think tells you that they don’t really think it works. They thought that really what they were treating were sleep disorders in patients who happen to have PBC, and that is why the result was not accurate. There was a study at the Mayo, predominantly of PBC, and again that was negative. The only time I have used Modafinil is in someone with PSC. Broadly speaking I don’t think it’s the solution for fatigue. I think the best treatment for fatigue is exercise. Fatigue is a complex subject and we’ve got people looking at it. It’s seen in PSC without a doubt, but it’s seen in all common inflammatory conditions: arthritis, PBC… and it seems to be a complex mixture of your brain, your nervous system and your muscles. That’s why exercise, whether you like it or not, is probably the most useful thing to intervene at present. Fatigue is going to become a big research theme in the UK for patients with chronic diseases.

Q: If you have a transplant, do you see people feel still fatigued post-transplant?

A: (GH) Yes, but its patient specific, and depends on how sick you were. Broadly speaking you definitely feel better after a liver transplant. But don’t expect to feel perfect. Questionnaires have been done in PBCers in a genetics project in Cambridge and Newcastle, and those who had transplants still had fatigue.  But you will feel better. That’s because the symptoms are complex and may have science behind them that we don’t really understand. Patients with PSC have more symptoms than average, but the problem in studies is finding something to compare them to. Your symptoms are real, but we just don’t understand them.

(DA) There’s some research from a colleague in Canada where he causes a type of PSC in rats, and these rats behave as if they’re very tired. When he’s looked at what causes that fatigue, it appears that the PSC activates the immune system and the white blood cells which go into the brain and activate factors there that cause these animals to behave in a fatigued way. If you stop the white blood cells going into the brain, they start behaving normally again. This is a complex area and there appear to be real mechanisms to blame.

Q: What sort of exercise?

A: (GH) Exercise that makes you sweat. Brisk walking (not running after your kids!) 20 – 40 minutes 3 times a week. It’s good for the heart and conditioning the muscles. We know that patients who do need liver transplants do better if they’re doing exercise and stay healthy, both pre and post transplant.

(DA) One of the problems in all liver diseases is sarcopenia - meaning the muscles waste away. We used to think we could treat this by nutrition, but found that this makes no difference at all. The one thing that improves muscle strength is exercise, and in particular isometric exercise (push-ups, sit-ups, weights.)  Sarcopenia affects you more if you are over 45.

LIVER FUNCTIONING V SCARRING

Q: I’ve seen scans of my liver – lots of scarring, but my liver function is fine. How can there be so much damage yet it function normally? What’s going on?

A: (DA) The liver has an amazing ability to regenerate and to cope with large amounts of damage. What is important is that you look well, have good muscles, and probably good liver function. Your liver is compensating and making up for the scarring. Your biliary system may look scarred, but is actually functioning alright. We shouldn’t be obsessed with the scans and tests, but should rather look at the patient.

(GH) The outcome of any disease is a combination of why you got it and how you cope with it. Patients are all different in how they or their liver reacts and compensates for the disease. We need to try to understand why some patients are well, even with extensive scarring, and others are not. This is why specialist clinics like James’s are so important. When you see a lot of patients, you see the breadth of the disease, which is not the case if you only see one or two patients.

(JF) What we are trying to do is to ‘stratify’ the patients, putting them into different groups. That will help us to treat them in the future.

ANTIBIOTICS AND INFECTION

Q: Is there any information on the research being done in the use of Vancomycin (an antibiotic) in ‘pausing’ PSC?

A: (GH) The pilot data is from paediatric patients. They haven’t published many papers and I’d say the jury is still out. There is a concern, with regular use of Vancomycin, with bacterial resistance. Especially if you have a transplant, we may run out of antibiotics that you can be given.

(DA) Some of you may have been given rifampicin for itching, another antibiotic, and this also has some interesting effects on some of the bacteria in the biliary system. But again, I don’t think there’s any evidence that long-term antibiotic use is beneficial. If you’re getting recurrent infections, rotating different antibiotics is beneficial to prevent resistance.

Q: What are the symptoms of an infection?

A: (DA) Classic symptoms of cholangitis (infection of the biliary system) are abdominal pain, sudden jaundice and fever. In patients with PSC, symptoms can be more subtle. You may feel a little ‘off’, a bit sick, and have sweats particularly night sweats. The disease can fluctuate even without a lot of symptoms, which can be wide-ranging. Sometime patients improve if they take rotating antibiotics, or sometimes we just give patients a supply so that they can start taking them if symptoms start. If you do get symptoms (e.g. night sweats), but they resolve quickly that’s fine, but tell the doctor next time you come to clinic.

(GH) Most doctors who don’t look after PSC patients believe that to have cholangitis, you will have very high fever, severe pain and high white cell count. If you go to your local A&E departments, they won’t believe you’ve got cholangitis. It’s a good case for advocacy on behalf of patients to educate other doctors that cholangitis with PSC can be a spectrum of symptoms, not necessarily a textbook case.

Q: I’ve had PSC for 10 years, and have only just learned today that night sweats are a symptom of cholangitis. There must be a lot of patients who don’t know what the symptoms are.

A: (JF) Night sweats are a common symptom – it might not be an infection, it may be other things.

(GH) What we’re trying to do as doctors is to determine when we intervene. You have PSC – that is an inflammatory condition of the bile duct. Inflammatory processes may make you feel tired, give you night sweats, even without an infection. If you start to get new symptoms that keep repeating themselves, then some intervention may help.

Q: I had a transplant 10 years ago. Had frequent infections, but am managing them better now. Barium follow-through showed that barium went up the bile duct into the liver, so I had the bile duct extended from 40 to 80 cm. Is it the accepted protocol in PSC transplant patients to have an extended bile duct?

A: (DA) In a normal liver transplant, the bile duct is rejoined to the new liver. In PSC, however, because the bile duct can be scarred and damaged, it’s usual to bring up a loop of the bowel. The problem then is that you’ve lost the control of the bile flow, so there is a risk of infection from the gut backing up into the liver. It’s a common problem. Some work has been done at the Royal Free hospital, going against the traditional method used for PSC and just reconnecting the existing bile duct, with some good results.  Whether this can be done on depends on the condition of the patient’s bile ducts.

(JF) If you’re getting recurrent cholangitis post transplant, this can be due to lots of reasons, and not necessarily due to infection from the gut.

ULCERATIVE COLITIS AND PSC

Q: In PSC with no UC, is it just a matter of time before UC develops, or will PSC just stay on its own?

A: (DA) In my many years of research, I believe that PSC comes from the gut in many patients. The more you look for UC in PSC patients, the more you find it, but it can be missed. But there are some patients who never do get IBD. This may be a subset of PSC, and work has been going on with Gideon, in Oslo and in Oxford, looking at the genetic mutations associated with PSC and IBD, which may help to unravel this picture.

(GH)  This is why when you are told you have PSC, you should have a colonoscopy with biopsies. If that’s normal, you need another one 5-10 years later. Only after 2 clear colonoscopies, will I believe there’s no IBD. Not everyone does biopsies, but it is a microscopic disease of the bowel, so you need them.

Q: Is there any evidence that PSC can improve or stabilize after removal of the colon?

A: (JF) When we looked at all our PSC transplant patients, the predictor was that about 1 in 5 will get a recurrence of PSC. You are more likely to get a recurrence if your colon is still in. There seemed to be a protective effect of having a colectomy after transplantation. But prior to transplantation, the story is different. What is also interesting is that if you have the ‘calmer’ form of IBD, your PSC is likely to progress more aggressively than if your IBD is problematic. So these are two conflicting pictures from the clinical data.

Q: I have had two clear colonoscopies and my last MRCP was also clear. Is this unusual? (Reported over the telephone – seeing consultant next week.)

A: (DA) I would guess from your telephone conversation they meant that there hadn’t been any changes. If it’s gone completely back to normal, that would be extremely unusual. There’s one form of PSC where this can happen and that’s called IGG4 PSC and Autoimmune Pancreatitis, which is a subset of PSC which responds very well to steroids. Have you taken steroids? (Yes, for arthritis.) If your MRCP is completely normal, I suggest you ask your clinic to check your IGG4. If you’ve got PSC that’s resolved, that would be extremely interesting for us in research.

GENETICS?

Q: If you’ve got PSC, what’s the risk for your children and should you be doing anything about it?

A: (DA) In most patients there is no family history of the PSC or IBD, although there are some cases where it runs in families which gives us an interesting insight into what causes the disease. The research that Gideon has been doing suggests that there are genetic mutations that make you more susceptible to factors in the environment that trigger PSC. Because genetic factors are inherited,  your children have a very slightly increased risk of developing PSC but the disease only occurs if you have several genetic mutations AND you are exposed to the triggering factor, the risk is small. Tell your children you have it, and if they get symptoms which may be IBD or PSC, they can tell their doctor. Chances are they’ll never develop it.

Q: How relevant is it if there are other autoimmune diseases in the family?

A: (DA) Strongest link is with PSC and IBD, but there are links with other forms of autoimmune disease. I think it’s very important that you tell your doctor of other autoimmune disease in the family, because that may give an indication that other autoimmune disease may be present, and also for us to know the links because it helps us understand the mechanisms behind the disease.

ITCHING

Q: Itching in PSC – is it more likely on certain parts of the body? Also spots mainly on the arms turning to ulcers and scars – is this related to PSC? If so, what treatment?

A: (DA) Itching can affect anywhere, but all over. Usually generalized itching – many people complain of soles of feet. It can be bad early on and then go away. And come again later on. We don’t really understand the mechanism for itching. Related to the second question, there may be a skin problem unrelated to PSC, so you should get the doctors to be sure about what is causing it. There are a range of skin conditions linked to UC and PSC, one typical one called pyoderma gangrenosum. There are a range of skin complications due to vasculitis, which is inflammation of blood vessels in the skin. So it’s important that he gets his skin looked at by a specialist, and probably biopsied too. If its vasculitis, that might change the treatment he’s receiving.

Q: My son is on Rifampicin for itching. I’m concerned about him taking this long-term. Apart from this and Questran, what else is there?

A: Don’t worry too much about long term Rifampicin. It’s a good antibiotic to be on. It reduces the itch by getting rid of some of the products that produce itch. There are other things that we can try: prozac-type antidepressants, anti-sickness drugs like Granisetron, drugs that are used in people with morphine – so there are a series of strategies, most of which don’t work well. In my experience the best treatments are Questran, sometimes Urso and Rifampicin.

TRANSPLANT

Q: Transplant Assessment. What would a PSC end-stage panel of bloods look like compared to other liver diseases?

A: (JF) Trying to pick the moment when a person is sick enough for transplant, (so you don’t put them to unnecessary risk by sending them too early) but not too sick for a transplant is very difficult. It’s harder in PSC because of the unpredictable nature of the illness. How do I decide? If someone is persistently jaundiced, that’s a concern. Also if the liver is very small and scarred, if the spleen is enlarged, or if ascites (abdominal fluid) is present. We try and consider all those factors and assess early. You have to have a UKELD of 49. The score comes from a number of blood tests. That predicts that risk of dying from liver disease is greater than dying from transplant. MELD & UKELD do not discriminate against PSC patients because there is on average a higher bilirubin, which gives a higher score. It’s a complex decision, and there are different types of patients. There’s not one answer.

Comment from the audience: We’d like our consultants to have a significant say in when we go on the list. We don’t want a system where there is a score.

(DA) We agree, but we do have to have a scoring system because there aren’t enough livers. They do give a guide as to when a patient needs a transplant. Whatever system comes out of the allocation process, the relationship between your and your physician will be maintained.

Q: Will a previous history of breast cancer affect going onto transplant list and outcome of transplant?

A: (DA) In the past, any history of cancer excluded you from receiving a transplant, as it was thought that immunosuppressant drugs would cause cancer to return. Experience has shown this is not the case, and in some types of breast cancer, there is evidence that drugs might reduce the chance of the cancer recurring. Previous history and type of cancer needs to be carefully assessed.

Q: Why do periods stop in young women with liver disease? Will they come back and what are the chances of having a family with liver disease or post transplant?

A: (JF) It could be the liver disease that’s caused them to stop but it’s important to be seen by a gynaecologist to see if there are other reasons. Secondly, we see many young people here in the clinic who have had families post transplant. You need to plan with your doctor if you are going to have a baby because some of the medication is not good for the baby and will need to be changed.

(DA) Even people with quite advanced liver disease can become pregnant.

Q: On scores & transplants: would the increase of donors lower the score, or would an increase of donors actually produce more livers?

A: (DA) The score is there to tell you that the patient’s liver disease is bad enough to need a transplant. Problem of donors is that there are some types of liver disease that we’d like to transplant, but the outcome isn’t good enough. For instance, liver cancer. For small cancers, transplant is normally 100% successful. For intermediate sized cancers, there’s a 30-40% chance of recurrence. That’s a good rate, but we can’t give those people the livers because that would take away the chances of those with a better success rate being transplanted. If we had more donors, we could transplant more risky patients.

Q: Would you change to an opt-out system for liver donation?

A: (DA) In Spain, for instance, where they have an opt-out system, they now have the best donor rates in the world. But there they created a group of donor physicians who go around intensive care units and identify potential donors. That helps to maximize the donor pool. It’s a difficult one. Some say that people will be suspicious of an opt-out system, and will opt out, whereas with the current system they might have become donors.

(JF)  Spain is also more complex because they have far more intensive care beds per capita, and people can go into IC and go on to become donors more easily than in the UK. In the UK, Wales will probably go to an opt-out system, and we’ll see if it makes any difference. It has to be weighed up – if you have a system imposed on the public, they might not want to donate. In the end it’s the family that has to agree, and if the public are not in favour, you won’t get donations.

Q: When we’ve been in the liver allocation talks with other support groups, we recognize that we need a united front to get people donating. Do you[does one] just go to the liver charities to raise awareness of donation, or do you[does one] go for everything?

A: (DA) I think you do everything.

Comment from Lynda: As well as raising awareness and getting people to become donors, they must also share their wishes with their families, so the family does not go against them.

(DA) That’s the most important factor. I have personal experience of this when my mother died. She’d wanted to be a corneal donor. A donor nurse at QMC in Nottingham dealt with all the formalities after the bereavement, and made it all so simple. If all the IC units in the country could operate in this way and make it easy for families during this stressful time, I’m sure this would increase the donor rate.

Q: Is it dangerous for a transplantee to be in contact with someone being treated with BCG viral treatment for bladder cancer?

A: (DA) (BCG is an effective immunotherapy treatment for bladder cancer.) I can’t see that that would be a problem.

Q: What is the situation about recipients being transplant donors?

A: Corneal donation is ok, but there is a worry as to whether the immunosuppressant drugs will affect other organs. But we’re not sure.

Q: Is living liver donation something that is favoured by the clinical community? Does the patient need to be proactive about it? When is it suitable?

A: (JF) We wouldn’t favour it if we had enough organs. You’re putting a well person at risk. Sadly we have to use it, as we don’t have enough livers. In some parts of the world, where culturally receiving an organ from someone who has died is unacceptable, their experience is quite successful. We do have a living donation program here, but it’s quite rare. In the future, with more livers needed, it’s something we’ll do more. We suggest it to every patient who needs a transplant. In the last year there was only one living adult donation, though it’s done for children more commonly. It’s a big operation to go through and it’s a very complex picture for ethical and all sorts of other reasons, but I think it will increase.

RESEARCH

Q: Are there any new treatments on the horizon for PSC?

A: (DA)There are some new treatments at an early stage. Hoping to start trial in 12-18 months using a drug which blocks white cells from entering the liver where they cause scarring. We are also talking to some drug companies about some drugs which block the immune/inflammatory response which we think would be really good in PSC. Thirdly, there is a new drug, at the end stages of development which will improve the treatment of IBD, but which the drug company wants licenced for IBD first before being used for PSC. So I think in the next few years there are new treatments coming along which will address the cause of the disease, possibly stopping or reversing it, rather than just treating the symptoms and complications.

Q: Have you had any encouraging results from the stem cell trials? Are those trials available for people with PSC?

A:(JF) They are currently in the early stages and are not available for people with autoimmune diseases like PSC and PBC. Trials are ongoing and in the next year or two we’ll know the results.

(DA) In the next round of trials, we’ll be looking at a type of stem cell which switches off the immune system, and PSC might be a good indication for those trials.  We’re also hoping to do some trials with regulatory T-cells which switch off inflammation, and PSC would be a good target. I hope in the next couple of years we’ll be coming to ask if you are interested in participating.

Q: Norurso?

A: (DA) Norurso is ‘super’ Urso – which has proved to be not quite as straightforward as we thought a few years ago, so I don’t think it will be a drug that will affect the disease process fundamentally. It might be useful to moderate the disease. Some of these other treatments could potentially affect he underlying disease process and that’s why they’re very exiting.

Q: What is the ‘Birmingham’ position on Urso?

A: (DA) Recent evidence suggests that high-dose Urso should not be used. Standard dose or perhaps slightly lower, has a beneficial effect on the colon in protecting against colon cancer, and may exert a slightly moderating effect on the PSC. I’m telling patients who’ve been on it to continue, but not on a high dose. (High dose is above 1.5 g per day. Normal dose 750mg per day, depending on body weight.) Patients on high-dose Urso were at greater risk of progressing to transplantation.  Also, there is some recent evidence that it slightly increased the cancer risk, but we don’t understand why. If you’ve been on high dose, you shouldn’t worry as the risk is very small.

Q: If Urso is stabilizing your bloods, but not doing anything for the progression of the disease, you may be masking something, and it will completely alter your UKELD score for transplant. You’d be better to come off it from that point of view.

A: No comments on that! It’s true also of PBC.

DIET

Q: Are there any foods to avoid that might make us feel more ‘liverish’?

A: (DA) I tell people to eat a healthy diet. Some people find digesting fatty foods more difficult, but a low-fat diet is part of healthy eating. We have some fascinating research which shows that some components in food might contribute to triggering the inflammatory response. It doesn’t translate into specific dietary recommendations as yet. The other issue is alcohol. You should stay within the national guidelines, and should check with your liver doctor what is recommended for you depending on how advanced your disease and your liver function.

Q: What about gluten intolerance?

A: A gluten free diet is only beneficial if you have coeliac disease. If you have coeliac disease it should be treated and can be linked to PSC. People have got the idea that gluten is bad for you. It isn’t. In coeliac disease there is a specific immune response to a component in gluten. You should only avoid gluten if you have symptoms when you eat it.

Q: My daughter has IBD/PSC and put herself on a gluten/dairy free diet. It has changed her life from no energy to being back at school and playing sport.

A: (DA) It’s probably due to the dairy more than the gluten. There are a group of patients with IBD who are lactose intolerant. I’d advise her to stick with the dairy free, go back on the gluten. If she gets symptoms, get tested, if not, she can enjoy her cakes and pizzas!

SPECIALIST CLINICS?

Q: How easy is it for people to be seen by a specialist, especially if you are not near a specialist centre?

A: (JF) It should be very easy. It’s your choice. It’s your right to go to your GP and ask for a referral to a specialist clinic. A specialist will not refuse to see you.

Comment from floor: It’s important to be seen in a clinic where they treat large numbers of PSC patients, not just one or two.

(DA) The government is emphasizing the need for training more specialist liver doctors in local hospitals, so they can pick up the disease and know when it should be referred to a specialist centre.

Q:  My son had Ulcerative Colitis, diagnosed 2005, then PSC 2009. He was told on 21st Nov last year that he had liver cancer. We were never told of the importance of PSC. He passed away in January, aged 26. I feel that his consultant didn’t understand about PSC, and that it’s important to get specialist treatment with this condition. I think you’re doing wonderful work, and may it continue. You have my support.

A: (DA) This is clearly tragic. One of the problems for doctors is that, whilst UC is fairly common, PSC is rare, and rapid progression to cholangiocarcinoma (liver cancer) is even more rare. It stresses the important of trying to educate our colleagues about PSC and of having specialist clinics.

Q: I live in the north-west and at the moment I’m fairly asymptomatic, and not being treated at a specialist unit. From what was said by James earlier about being seen early by a PSC specialist, should I move or stay?

A: (JF) Apart from the advantages of being seen at a big clinic as we’ve already discussed, the other potential advantage is that for treatments to work, it should be with early PSC, to prevent the disease progressing, so those are the patients we want to see. We see people from all over the country, and we don’t take over someone’s care, but enter into their joint care with their local doctors.

Q: I’m being treated in Sheffield. Is there specialist knowledge there?

A: (DA) There a very good liver doctors in Sheffield. The closest transplant unit is Leeds, but you’ll be in good hands in Sheffield. Early in the disease, symptoms can be bad and then settle down.

Q: My mother is being treated at Queens Medical Centre in Nottingham, and is receiving good treatment. How do patients in other liver units plug into the detailed knowledge about PSC at specialist centres?

A: (DA) The QMC is very good and we work very closely with them and sometimes share patients. We are working together with their researchers on several of the experimental approaches that we’ve talked about. So they are well plugged into our knowledge.

MISCELLANEOUS

Q: I’d like your thoughts on the use of circumin, the active ingredient of turmeric. It’s been used for a number of years for its anti-inflammatory properties.

A: (DA) As you say, it has been studied for its anti-inflammatory properties, but how it works isn’t clear. It has effects on the nervous system and the immune system. I don’t think there’s enough evidence to recommend its routine use, but if you tolerate it and think it helps, that’s fine. Nobody should take herbal or alternative medicines without talking to their doctor because many of the herbal medicines are actually quite toxic. People think that because it’s a natural product, it must be safe, but opium is a natural product, and taking that is not too good for you!

Q: I’m a personal trainer and can’t get qualification for treating people with liver disease. Do you have any pointers as to how I can take this forward?

A: (DA) There’s a very good school of Sport and Exercise Science in Birmingham University. They may be able to help.

Q: My daughter suffered from fungal infections before transplant and was treated with Intraconazole. Post transplant, this not working. Is this due to anti-rejection drugs or to Infliximab which is for her UC? Can someone offer some help or ideas?

A: (DA) Patients with IBD can have defects in the immune system which make them susceptible to fungal infections. Immune system is further switched off by anti-rejection drugs and we do see an increase in fungal infections post transplant. Infliximab has a major influence on how your immune system responds to infection. I would recommend a thorough work-up of her immune system. If there isn’t a problem she should probably see someone from infectious diseases. It may help to change infliximab for something else.

Q: Prior to being diagnosed with PSC, I had a choledochal cyst, which now appears to have stabilized. What is your stance on choledochal cysts – remove them?

A: (DA) There are different types of choledochal cysts, and are graded on where they are and what size they are. Surgeons have well defined rules as to when to operate.

(JF)  It would be worthwhile having a liver/biliary specialist monitoring the cyst. They can be associated with other disorders of the bile ducts.

Q: Is being on Bu Trans (an opioid analgesic) a good or bad thing?

A: (DA) On the whole it’s good to get off any form of strong painkillers. The opioid type can make itching a lot worse. Long term these drugs can cause fatigue, and you can get a degree of dependency. If it did come to a transplant, the anaesthetist would want to know