The PBC Genetics Study Research Team 01223THE 769088 BEAR FACTS

SPRING 2015 Issue No. 70 Support for Life

‘How would your life be if the PBC Foundation did not exist?’ is the question that was asked CONTENTS of me some weeks ago. My immediate Nationwide Thanks...... 2 response was to think of the time I would PBC Clinic...... 4 have to spend on other pursuits, offset of course by the time spent seeking out reliable View from the Bar...... 7 sources of PBC information and finding Money Matters...... 8 some means of support for my illness. And then, once I’d made a start in these Regional Volunteers...... 10 endeavours, how could I resist sharing it Dates for Your Diary...... 13 with people around the country in similar circumstances to my own. Maybe then, if I Project Uk...... 14 received a positive response from my fellow Your Letters...... 16 patients I could start up a Support Group which would lead to a charity. Ultimately, as this train of thought reveals, Fundraising & Awareness...... 18

SPRING 2015 NEWSLETTER I think the existence of the PBC Foundation is inevitable until the day Food for Thought...... 20 we find a cure. And may that be not far over the horizon!

any things have changed over the nineteen the Bear Facts to over sixty six counties worldwide. Myears that the Foundation has given to This publication alone removes, for thousands of PBC supporting positive aspects in the life of those with sufferers, the crushing isolation that was prevalent PBC, their families and friends. This month, May before the Foundation existed. 2015, sees another significant change when the name ‘Primary Biliary Cirrhosis’ is replaced with e are looking forward to celebrating the Foundation’s ‘Primary Biliary Cholangitis’ throughout the entire twenty year anniversary next year and we will be world. An overwhelming number of our members Wtalking and writing about the many advances in the as well as medical professionals, motivated by the care and support of PBC that you, the team and I have need to remove the negative connotations of the achieved over the years. I heard from two of our very first word ‘cirrhosis’, have successfully campaigned for volunteers the other day, John and Teresa Pawson from its removal while retaining the acronym PBC. We Lincoln. We talked about the old days and how things at the Foundation accept that the name change is have so much changed for the better. More in the next not universally welcome but feel that the wishes of issue but in the meantime, we would love to hear your a large majority of our members must be respected stories – please do keep sending them in to us, it is a and, as in this case, successfully implemented. joy sharing your experiences with all of our members. Such is the power of patients’ support groups! Amongst many others who played a part, the main I am sure we will be sharing stories of our next drivers were: Prof Ulrich Beuers, Prof Robert Gish, International PBC Day which is 13th September 2015. Prof Raoul Poupon, Ingo van Thiel and EASL. The No doubt there will be some whacky scheme here in PBCers also responded kindly and positively to the office that somebody will have dreamt up for us to our request for support. carry out!

ne of our earliest achievements was having The question I would now like to ask you – how would the best quality information on PBC available your world be if the PBC Foundation did not exist? Oin leaflet formfree for all in need. We quickly went on to supply a leaflet on PBC specially written I look forward to hearing your thoughts and experiences. for GPs, again without charge. Our membership is entirely free to anyone who would like our Keep well, help and support and, as you know, we supply

Spring 2015 1 The Bear Facts NATIONWIDE THANKS The PBC Foundation (UK) Ltd NATIONWIDE THANKS Helpline Number NATIONWIDE THANKS 0131 556 6811

Our grateful thanks go to each and every one of you who has taken the time and made the effort to raise money for the PBC Foundation (UK) Ltd. Without your help, dedication and selfless acts of generosity, in whatever guise, we would be, quite simply, unable to continue our work. It never ceases to amaze us here on the team how diverse and ingenious some of the fundraising ideas are: quite often truly inspirational. On behalf of our members, present and future, our sincere thanks to you all.

Anglesey Ladies Lunch Lauds Phyllis Congregation Donation from Dalmeny Kirk We were delighted to receive a donation of £65 raised Dalmeny Kirk just outside Edinburgh, may well be through a raffle, held during a monthly ladies’ lunch known for its beautiful architecture and is indeed in Anglesey, Wales. The group held the raffle for considered the most complete example of a Norman the Foundation because their local member, Phyllis church in Scotland. It is also evident they have Dunne, has PBC – a condition which hardly any of warm hearts and a generous spirit among their the group had heard of. We’ve had some wonderful congregation too. A retiring collection following comments over the years but surely there are few the Communion Service in March raised a much better testimonials than the comments from the appreciated donation of £83.04. The Kirk also ladies that Phyllis was looking so good for eighty five provided the location for two of our dancers to train years old that we, as an organisation, must be doing in preparation for the Strictly fundraising event in something right. Our grateful thanks to the ladies for February. Our sincere thanks to all at Dalmeny Kirk their support and our best wishes to Phyllis. for their kind consideration of our cause.

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Coleraine Colleagues Collection Benefits PBC Demon Dancers Jive the Night Away in Fife John and Irene Porterfield of Demon Dance organised a Christmas Charity Ball in Dunfermline to raise funds for the PBC Foundation. “Jive Aid”, as the event was known, brought dance enthusiasts together from all corners to enjoy a thoroughly entertaining night. The event was held in the stunning Glen Pavilion building, situated within Pittencrieff Park, the park itself being a gift from the world renowned philanthropist Andrew Carnegie, to the people of Dunfermline. We know John and Irene’s event was a resounding success and the participants will not have been out of place in a venue that has seen rock superstars Big Country perform. Proceeds from the event and the raffle held Joan Millican of Co. Londonderry, has had PBC on the night, raised a staggering £2,271 for the PBC for twelve years and works part-time for the Social Foundation. Our sincere thanks to everyone who Security Agency in Coleraine. Having attended a participated, all who attended and in particular, John workshop run by us in Northern Ireland, she was and Irene for their generous consideration of our enthusiastic about the opportunity it provided to speak cause. to other sufferers. She was equally enthusiastic in -:- -:- -:- -:- -:- -:- -:- -:- -:- -:- -:- -:- galvanising her colleagues to raise money for the Foundation and we were delighted to receive a Sisters Make a “Moray”mint for PBC cheque for £72 from Joan, together with a picture of You’ve heard of money for old rope, or where there’s her being presented the cheque by Sharon Anderson. muck there’s brass? Well this was never more true Many thanks to Joan and her colleagues. than in Morayshire where our Regional Volunteer,

The Bear Facts 2 Spring 2015 The PBC Genetics Study NATIONWIDE THANKS Research Team NATIONWIDE THANKS 01223 769088 NATIONWIDE THANKS

Irene Ferguson, and her sisters Trisha and Diane coming to the Foundation. Last year, Julia raised worked throughout the year attending car boot £125 in sales and she has already started work on sales. Along with raising vital funds, amounting to 2015’s stock, so get your orders in early. We can a grand total of £1,000 over eleven months, they only admire Julia’s creative skills and thank her most raised awareness of both PBC as a condition and sincerely for her continued support. the existence of the PBC Foundation. Bravo ladies, -:- -:- -:- -:- -:- -:- -:- -:- -:- -:- -:- -:- our grateful thanks for your continued efforts and invaluable support. Linda Turns Back Time on Tyneside

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A “Luxury” Donation from Northamptonshire it’s “Fair” to Say In November 2014, Luxury Fairs (www.luxuryfairs. co.uk) held a Luxury Christmas shopping event at the Great Barn, Aynho, Northamptonshire. This prestigious event saw local craftsmen and woman sell beautiful goods to bustling crowds of Christmas shoppers. At this event however, 10% of all stallholders takings went to the PBC Foundation, so shoppers could stock up on festive goodies and raise money for us at the same time. We were delighted Linda Muir of County Durham sent us £25 in therefore, to receive a cheque in the post for £200 for sponsorship of our London marathon runners. Accompanying the donation was a lovely little which we send our sincere thanks. notecard depicting a picture of Saint George’s Church -:- -:- -:- -:- -:- -:- -:- -:- -:- -:- -:- -:- in Jesmond, Newcastle upon Tyne. The Foundation receives many little notecards with donations but Pupils Primary Example in Co Fermanagh this one has special meaning for Linda. The church Anne McMenamin, Secretary at St Josephs’ Primary is where Linda and her late husband Rob were School Ederney, Co. Fermanagh wrote a lovely married fifty years ago on St Patrick’s Day. On the letter to us recently. A long term member of the anniversary, Linda and her family enjoyed a get- PBC Foundation, she encouraged the children to together and lovely meal. Linda also explained it raise money for the Foundation during their charity had further personal significance as she attended fund raising day. Well, if we could hire the pupils of the church and was a Sunday school teacher there St Josephs as our fundraisers we’d be a wealthy in the late 1950s to 1960s. Linda was keen to share organisation indeed, as the industrious pupils raised a little bit of the history of the church explaining it a phenomenal £200 for the Foundation. Well done to was gifted to the parish by a wealthy shipbuilder, the pupils and teachers, our sincere thanks to you all. Charles Mitchell and the foundation stone was laid in 1887. It cost over £30,000 for everything down to the -:- -:- -:- -:- -:- -:- -:- -:- -:- -:- -:- -:- purchase of the hymn books. Our grateful thanks to Handmade Crafts a Hit in Bristol Linda for her support of our runners and moreover, for We always admire the entrepreneurial spirit and sharing her fond memories with us. never more so when it raises funds which allow us to carry out our programmes for member benefits. Julia If you have raised funds for us, and haven’t yet been mentioned: do bear (!!) with us. It is important to us Merrifield of Kingswood, Bristol, spends the year that every effort is mentioned and acknowledged with making handmade cards and desk calendars, which our gratitude. she then sells to willing colleagues with the profits

Spring 2015 3 The Bear Facts PBC CLINIC The PBC Foundation (UK) Ltd PBC CLINIC Helpline Number PBC CLINIC 0131 556 6811

by Professor James Neuberger, Queen Elizabeth Hospital, Birmingham URSODEOXYCHOLIC ACID Ursodeoxycholic acid (UDCA) is a naturally occurring bile acid which, as most readers will know, is used in the treatment of Primary Biliary Cholangitis. The beneficial effect of bile from bears in those with jaundice has been known for many centuries but the impact of the major bile acid in bear bile, UDCA, was first identified in the mid-1980s.

Since that time, there have been a number of well-conducted The commonest side‑effect noted to date is diarrhoea. However, studies comparing UDCA with placebo. In the early days, most of like all medications, risks and benefits must be balanced and no these studies involved patients with moderate to advanced PBC. drug should be considered free of side effects. In particular, UDCA These early studies were relatively short-term (short in the context should be used with caution in women who might become or are of PBC) lasting only a few years in many cases and, therefore, pregnant so if you are or may become pregnant whilst on UDCA, conclusions were guarded. Since the early days, there have been you should discuss this with your doctors. many more studies involving people with PBC, including more at an earlier stage of the condition and lasting longer periods of time. It is important to make sure that the dose of UDCA is adequate. Studies suggest the appropriate dose for people with PBC is Overall, the balance of studies clearly confirm that liver function between 13 and15mg/kg body weight/day. Studies, including tests improve considerably in most patients and indeed, often those where members of the PBC Foundation have contributed become normal. information, shows that still some people are getting lower doses. There is often symptomatic improvement both in the lethargy and Although UDCA is often given two or three times daily, some sometimes in the itching. Most, but not quite all, studies suggest studies have suggested that it can be taken as a single dose. that there is a significant delay in the time to transplantation, or end-stage disease. More recently, studies show that those people At present, UDCA (Ursofalk) is the only drug that is licensed for whose liver tests become normal, or very near normal, within one the treatment of PBC. There are other preparations of UDCA year of starting UDCA, have a better outcome. It is not completely available. clear whether the better outcome is a direct result of the UDCA or Most doctors currently recommend that UDCA should be offered to just whether those who respond to UDCA would do better anyway. all those with PBC and abnormal liver tests. In those who respond, While UDCA is not cheap (whether it is the taxpayer, the insurance it is reasonable to remain on UDCA long-term. For those whose company or the person themselves who pays for the medication, liver tests do not respond or for people who cannot tolerate the it is cost-effective. A study from Canada suggested that UDCA, treatment, other medications should be considered. although somewhat expensive, is cost‑effective, in that, it reduces It is not yet clear whether UDCA should be given alone or in a number of complications such as bleeding varices, or ascites. combination with other drugs to all people with PBC. Although The effect of UDCA on the degree of scarring in the liver remains there are undoubted benefits from its use, it is unlikely that this less certain but appears beneficial. alone will be the answer for all those with PBC. The continued studies looking at UDCA in combination with other medicines The way in which UDCA works is not clear. There is a number (including other bile acids such as obeticholic acid, steroids such of possible mechanisms: these include having a flushing effect as prednisolone and budesonide, other immunosuppressive of bile in the biliary tree and an immunological effect normalising agents and other drugs such as fibrates) will in time identify the many of the abnormalities seen in the blood of people with PBC optimum method of treatment. Until we have a cure for PBC, we and protecting cells from the toxic effects of other bile acids. We can only improve on treatment by having well conducted clinical do not really know how UDCA exerts its effects. trials and this means support and participation from those with PBC! So if your doctor asks whether you would like to take part However, we do know that UDCA is safe and well tolerated. A in a trial, please do consider this invitation carefully. recent analysis of studies using UDCA for people with PBC, published by Zhu and colleagues in March 2015, found that there It remains uncertain when treatment with UDCA should be started. were 31 studies of sufficient quality to merit analysis. The authors While most clinicians would agree that those with abnormal liver concluded that UDCA, alone or in combination with other drugs, tests should be offered treatment, it is unclear whether those with was associated with a significant reduction in ether death or liver no symptoms and normal liver tests should be offered treatment. transplantation and few side-effects.

Itching, also known as pruritus, is a feature of Primary Biliary Cholangitis, as it is of several other liver diseases.

The severity of the itch can vary enormously between people with It is very important to appreciate that the severity of the itching bears PBC with some people having no itching and others having very no relationship to the severity of liver damage. Indeed, there are many severe itching. Furthermore, the severity of the itching for each people with very early disease and little, if any, damage in the liver individual person can fluctuate greatly during the course of the PBC. yet have very severe itching; the opposite situation also exists where There is also variation in the severity of the itching both within the day people with quite advanced disease have no itching. Thus, although and between days. For many people, the itching can be severe for the itching can have a major impact on symptoms and quality of life, several weeks or months and then improve. Studies have suggested it does not seem to reflect the degree of liver damage. that during the day itching is cyclical in intensity, becoming worse and then improving without obvious cause.

The Bear Facts 4 Spring 2015 The PBC Genetics Study PBC CLINIC Research Team PBC CLINIC 01223 769088 PBC CLINIC

Cause of Itching some vitamins and some medications and therefore, it is important The cause of the itching in PBC is not really fully understood. It used to take such medicines at different times from the Colestyramine. to be thought that the bile acids that are retained in PBC were the If in doubt, talk to your pharmacist, nurse or doctor. Colestyramine cause of the itching but subsequent studies have suggested that this does have some side effects including bloating and diarrhoea. This is might not be the cause. More recently, a number of studies, based lessened by the use of Questran light rather than Questran. The dose on those that were originally done in Leeds, have suggested that the of Colestyramine needs to be adjusted to the severity of the itching. itch may be related to retention of naturally occurring chemicals called opioids which are not that dissimilar to chemicals that are similar A number of other drugs have also been shown to be effective in to the opium group of drugs. For instance, some drug addicts who some people with itching associated with PBC: take opiates do complain of severe itching and itching can also be associated with drugs such as Morphine. Thomas De Quincy, in his UDCA: In most people with PBC Ursodeoxycholic acid (Urso) has book ‘Confessions of an Opium Addict’ describes this well. It seems no effect on the itching. that many people with PBC do have increased levels of naturally occurring opioids and cells are thought to be more sensitive to the Rifampicin: An antibiotic type of drug which is used particularly effect of these chemicals. This is not only of some academic interest in the treatment of tuberculosis. Several studies have suggested but it has also allowed for new treatments to be introduced. that it has an effect in 30-40% of people with itching in PBC. The drug is thought to act as an antagonist of the PXR, not only does More recently, other chemicals that have been implicated in the it colour the urine a bright orange, but it has side effects in that cause of itching include lysiphosphatidic acid (abbreviated to LPA) it may damage the liver, so monitoring of liver tests is important and autotoxin (or also as ATX or ectonucleotide pyrophosphatase/ after the introduction of the Rifampicin. It also interacts with other phosphodiesterase 2). LPA is a powerful neuronal activator and ATX drug metabolism so you would need to be very careful as to which is an enzyme that helps make LPA. medications you are taking although usually it is well tolerated. Knowing the causes of itching will help in the treatment. Naltrexone: A drug that was used for drug and alcohol dependency. Because it interferes with the opium-type drugs (on the μ-receptor) Treatment of Itching it has been shown to be effective in people with PBC and itching. The medication has to be given carefully as psychological side Symptomatic Treatment: Inevitably, people have found out what effects may occur especially in the first few days. In effect, a cold makes the itching worse and what makes it better and adopt turkey-like reaction may occur. Usually, a small dose is needed various strategies in their lives to reduce the severity of the itching. to start with and the dose is gradually increased but caution must Temperature can affect the itching and many people find a hot bath be taken in the first few days to make sure there are no adverse or locally applied heat will exacerbate the itching. Other people find effects. Again, it works in about one quarter of people. clothes of certain materials make the itching worse. Physical activity and diet usually have little effect on itching. Creams and simple Sertraline: More recently, the antidepressant drug, Sertraline has moisturisers are sometimes very effective. been shown as being effective in relieving the itching in some people. This drug is known to be an inhibitor of serotonin reuptake. Medication The effect is unrelated to the antidepressant impact and it must not The only drug that is licensed for treatment of itching in people with be assumed that, because the drug is used as an antidepressant, PBC is Questran, also called by its ‘proper’ name Colestyramine. the treatment is effective via treating the depression and that the This is a resin which is taken by mouth and interferes with the re- doctor believes the person is depressed. Again, one in four people absorption of those chemicals that cause itching. have a good response. It is very important to understand the mechanism of action of the Other Treatments: Sometimes cleaning the blood can be effective. drug as this affects how the drug can be taken most effectively. There are several devices including plasmapheresis that have Colestyramine is not absorbed and remains in the gastrointestinal been shown to be of help in some people. This is quite an invasive tract. In both the normal person and those with PBC, chemicals that technique and a little bit like haemodialysis. Sometimes this is cause the itch are released in the bile (which is made in the liver and combined with albumin and known as MARS. There are significant stored in the gall bladder) in the duodenum and travel down the bowel risks to it but it can be used to good effect on occasions. to where they are re-absorbed and so the chemicals that cause the itch are re-cycled. Colestyramine binds to these chemicals (and also Other approaches which have been successful in some people other molecules) and prevents their re-absorption. include phototherapy with UVB light and also nasobiliary draining where bile is removed through a tube that goes from the bile duct, The implications therefore are twofold: firstly, Colestyramine is more through the duodenum, stomach, gullet and out through the nose. effective, when given, when there is most bile in the bowel. Bile is made continuously by the liver and stored in the gall bladder; when Liver Transplantation: A very effective treatment for itching. the stomach expresses its contents into the small bowel, signals Usually, it is used as a last resort in those with early disease but, are sent to the gall bladder to pump the bile into the bowel. Thus, in those with advancing disease, transplantation is highly effective. the most effective time to take the Colestyramine is after a fast and when there is food in the bowel. This tends to be around the time of Prednisolone: In small doses may help a minority of people with the first meal of the day and therefore Colestyramine is usually most PBC but side effects are significant. effective when given just before and just after breakfast. Other doses should be given during the day just before lunch or the evening meal. Overall then, itching or pruritus can be a major problem in people with Secondly, because the Colestyramine acts by preventing re- PBC. However, adjustment of treatment usually allows good control absorption of the chemicals that cause the itch, it is therefore clear of this problem. In most cases, people’s symptoms are controlled that it will take several days or weeks before the excess chemicals are with Colestyramine. For those who do not respond or cannot tolerate depleted and Colestyramine becomes effective. Colestyramine does the medicine, there are alternative treatments but these may need have other side effects: firstly, it may interfere with the absorption of supervision from a specialist unit

Spring 2015 5 The Bear Facts PBC CLINIC The PBC Foundation (UK) Ltd PBC CLINIC Helpline Number PBC CLINIC 0131 556 6811

Palak Trivedi Academic Clinical Lecturer and SPR in Hepatology and Gastroenterology : NIHR Centre for Liver Research : University of Birmingham (UK)

Ursodeoxycholic acid (UDCA) represents the PBC, although certain factors may influence the mainstay of treatment in PBC and there is now good likelihood one way or another; including age at evidence that therapy slows progression of liver which you are diagnosed and the amount of scar injury. By drawing comparisons between liver blood tissue present within the liver. Up till recently, there tests at the start of treatment and twelve months has been little in the way of 2nd line therapy for thereafter, doctors will be able to tell how well individuals with PBC; however, several clinical trials UDCA is working via determining your ‘biochemical are currently underway, which specifically cater for response’. Although many criteria exist which classify non-responders. The only way to evaluate benefits response, they are all broadly similar. The important and side effects of new treatments is through clinical thing to remember is if these criteria are successfully trials which compare and contrast one agent with attained then outcomes with regard to your liver another and also ensure drug safety. Perhaps the condition are practically the same as an individual medication furthest in development is a compound without PBC. called obeticholic acid (OCA). This medication targets a molecule expressed in the gut and liver involved Perhaps the most widely used and most accurate in making certain parts of bile. A recent study led by response criteria are the Paris criteria. This model Dr. Gideon Hirschfield illustrates promising results looks at measured levels of bilirubin (the substance wherein OCA was successful in improving liver blood that leads to jaundice) as well as certain liver tests in patients who could not attain response with enzymes known as AST, ALT and ALP in your blood. UDCA alone. These findings have led to investigation Biochemical response is considered positive (good) beyond just a few individuals, and OCA is now if all of the following conditions are met within 1 – 2 undergoing larger trials in several centres across the years after being on UDCA: world. Other emerging treatments include bile-acid therapies related to UDCA, certain types of steroid, - AST + ALT levels below twice the upper limit of as well as agents which target specific immune-cells normal range, thought key to driving liver injury seen in PBC.

- ALP levels below three times the upper limit of In any event there are now several potential options normal range, and to consider for biochemical non-responders whereas - Bilirubin levels fall to normal. previously there were none. Be sure to ask your treating physician if you are a non-responder, for one Biochemical response is successfully achieved of these novel therapeutic options may just be the by approximately two-thirds of individuals with right one for you.

The Bear Facts 6 Spring 2015 The PBC Genetics Study VIEW FROM THE BAR Research Team VIEW FROM THE BAR 01223 769088 VIEW FROM THE BAR

Hello, peeps. Before we get started on this here page, a little history lesson, if you will . . . Once upon a time, there was a condition called Primary Biliary Cirrhosis. Unfortunately, this name created stigma where there needn’t be, fear where there needn’t be and confusion where there needn’t be. As it is now, in those days the word “Cirrhosis” had a subconscious link to the word alcohol, incorrectly. In order to start to tackle that connection, we looked at naming this article, “View from the Bar”. The other reason we used this name was that the grandfather of this page, our dearly beloved Snoddy, was a lawyer by training and, as we know, many lawyers even to this day still do most of their work at one bar or another. The last share we have for you brings us the joy that we can experience only when someone isn’t thinking for themselves. You And so we have it: the view from the bar. A (usually) light-hearted know the story: you call a cake maker and ask them to add the writing, look at the world in all its glory: heroes, villains, courage and idiocy “Happy birthday my darling”, and underneath that, “lots of love from all in one page. us all”, etc., etc., etc. Well, what happens if someone decides to Rather unusually, we are going to begin with a quotation as opposed write, “Underneath that”? to ending with one. These words resonated with me and come back to my attention every now and again, so we thought we would share Well, then we get these… them with you.

Our deepest fear is not that we are inadequate. Our deepest fear is that we are powerful beyond measure. It is our light, not our darkness that most frightens us. We ask ourselves, “Who am I to be brilliant, gorgeous, talented and fabulous?” Actually, who are you not to be? You are a child of God. Your playing small does not serve the world. There is nothing enlightened about shrinking so that other people will not feel insecure around you. We are all meant to shine, as children do. We were born to make manifest the glory of God that is within us. It is not just in some of us; it is in everyone and as we let our own light shine, we unconsciously give others permission to do the same. As we are liberated from our own fear, our presence automatically liberates others. Marianne Williamson

What do they say to you? Are they inviting you to be the best you that you can be, each and every single day? I don’t know to which God, if any, these words refer but I wonder if they bring back the innocence, the happiness, the fearlessness of childhood where we would often just be without the fear that adulthood brings.

Occasionally, these words are attributed to Nelson Mandela but they are, indeed, Marianne Williamson’s.

Now, I have to admit that I am not entirely familiar with the words of the bible but when I saw this quotation recently, I wasn’t sure about it:

“And the Lord said unto John, ‘Come forth, John, and you will receive eternal life’. But unfortunately for John, he came fifth and won a toaster instead.”

Anyway, enough wordplay. Let’s go back to history again and let me ask you where you were on March the 14th this year. Specifically, do you remember where you were just before half nine that very morning? I know many geeks that morning were having a breakfast of pie, or is that pi?

You see, on that particular morning of the fourteenth of March, 2015 at 9.26 and 53 seconds we were at the moment of Pi: 3.141592653

I wonder if the speaking clock shared that with us. “At the third stroke, the time sponsored by Accurist will be… Pi time!” We bid you a wonderful, fun-filled couple of months until the OK, maybe not. next time and, please… for us… shine your light!

Spring 2015 7 The Bear Facts MONEY MATTERS The PBC Foundation (UK) Ltd MONEY MATTERS Helpline Number MONEY MATTERS 0131 556 6811

otal income was higher than last year at £367,592 (up from T£279,188), Total expenditure increased to £326,358 (2013: £308,436) and the end result was a net surplus of £41,234 and year-end reserves of £290,801

Income For The Year to 31 December 2013 Income For The Year to 31 December 2014 Income For TheYear to 31 December 2013-£279,188 Income For TheYear to 31 December 2014-£367,592 £279,188 Income For TheYear to£367,592 31 December 2014-£367,592

Charitable Trading Income Interest Receivable Gifts and Donations Charitable Trading Income Interest Receivable Gifts and Donations 1% 0% 0% Charitable Trading Income Interest Receivable Legacies 1% Charitable Trading Income Interest Receivable Legacies Gifts and Donations 1% 0% 0% Sponsorship and Fund Raising 1% Sponsorship and Fund RaisingLegacies Grants Receivable Grants Receivable Sponsorship and Fund Raising Charitable Trading Income Charitable Trading Income Interest Receivable Grants Receivable Interest Receivable Charitable Trading Income Gifts and Donations Interest Receivable 36% Gifts and Donations 26% Gifts and Donations Gifts and Donations 36% 26% Grants Receivable 51% Legacies 2%

Legacies 2% Grants Receivable Legacies 63% 2% Sponsorship and Fund Raising Sponsorship and Fund Raising 8% 10% Legacies Grants Receivable 2% 63% Grants Receivable Sponsorship and Sponsorship and Fund Raising 51% Fund Raising Sponsorship and8% Expenditure on Generating Funds + Charitable Activities 10% Grants Receivable Fund Raising For The Year Ended63% 31 December 2014 - £326,358 8%

Expenditure on Generating Funds + Charitable Activities Governance For The Year Ended 312% December 2014 - £326,358 Expenditure on Generating Funds + Charitable ExpenditureResearch on Generating Funds + Charitable Health & Professional Awareness,Fundraising & 4% Activities for the Year to 31 December 2013 InformationActivities for the YearPromotions ended 31 December 2014 Expenditure on Generating Funds + Charitable Activities 5% 37% For The Year£308,436 Ended 31 December 2013 - £308,436 £326,358 Awareness,Fundraising & Promotions Governance Patient Support & Helpline 2% Awareness,Fundraising & Promotions Health & Professional Information Health & Research Patient Support & Helpline Governance Research Health & Professional Awareness,FundraisingHealth & Professional Information & Professional 4% 1% Governance ResearchPromotions Health & Professional Governance InformationInformation 1% 5% Governance37% HealthInformation & Professional Awareness,Fundraising & 5% Information 3% Awareness,Fundraising & Promotions 3% ResearchResearch Promotions Awareness, Patient Support & Helpline 9% 35% 35% 9% Fundraising & 52% Promotions 35% Awareness,Fundraising & Promotions Patient Support & Helpline Health & Professional Information Research Governance Patient Support & Helpline 52% Awareness, Patient Support Patient Support & Helpline Fundraising & 52% & Helpline Patient Support Promotions 52% & Helpline 37% 52%

The Bear Facts 8 Spring 2015 The PBC Genetics Study MONEY MATTERS Research Team MONEY MATTERS 01223 769088 MONEY MATTERS

otal income increased to £367,592 with increases in general donations of £5,316. Total fundraising showed an increase of £2,181. There was a decrease in Gift Aid donations T (-£8,100). There were a number of significant individual donations received including £12,500 for specific purposes, which we gratefully acknowledge. This year we received grants from the Scottish Executive of £10,000 (last year £25,000). Much effort was again expended on various grant applications. New grants this year included £21,824 from Awards for All and £4,117 from the Health and Social Care Alliance. The total for other grants of £194,635 showed an increase of £82,525 compared with last year. Of the total grants received £90,909 were for restricted purposes.

here were increases in a number of categories of expenditure, mainly conferences, meetings and workshops, reflecting the Expenditure on Generating Tincreased activity during the year. The majority of other overhead costs were broadly in line with 2013. Funds + Charitable Activities

he costs of the charitable activity ‘Patient Support and Helpline’ for the Year to increased from £161,295 to £170,342, an increase of £9,047. 31 December 2014 TThis was mainly due to more self-management workshops taking place, joint development workshops and increases in meeting - £326,358 with volunteers. A number of conferences were also attended in both the USA and Europe advocating the interests of our members. This resulted in higher conference, meeting and travel expenses being incurred. However, we were fortunate to receive Awareness, Fundraising & Promotions...... 121,036 Grants or be reimbursed for this expenditure. The costs of the charitable activities ‘Awareness’ and ‘Health and Professional Patient Support & Helpline...... 170,342 Information’ also increased, showing increases of £6,918 and £5,576 respectively when compared with 2013. Health & Professional Information...... 14,707 esearch expenditure decreased to £12,889 from £28,533, a Rdecrease of £15,644. Research ...... 12,889 he end result for the year was a net surplus of £41,234 compared with a deficit of £29,248 last year and reserves Governance...... 7,384 Tcorrespondingly increased at £290,801. Of the £41,234 surplus for the year £12,714 related to unrestricted reserves and £28,520 326,358 related to restricted reserves.

e will again concentrate efforts on fundraising and grant This has support costs allocated to activities applications in all areas, hopefully maintaining and building Wupon the success and levels achieved during 2014 and therefore be able to continue to provide the high level of service and support to our members. Hugh Pollock CA

Spring 2015 9 The Bear Facts REGIONAL VOLUNTEERS The PBC Foundation (UK) Ltd REGIONAL VOLUNTEERS Helpline Number REGIONAL VOLUNTEERS 0131 556 6811

A huge big thank you to all our Volunteers who give their time to support, on a national basis, you, our membership. Please help and support them whenever you can, they are volunteers and all either currently have PBC, or have been transplanted and know only too well what it is like to live with this condition. We in the office very much appreciate all they do.

Cleveland West Midlands Hello Everyone Greetings to you all, My name is Lynn Robson and I am a new volunteer My name is Esther Daniel and I am the new PBC for the Cleveland area. Volunteer for West Midlands. I only became aware of PBC when I was diagnosed four years ago, which I suspect is a similar situation for many of you. I also have auto-immune hepatitis so my initial reaction was shock and bewilderment. However, my consultant advised me to seek support from the PBC Foundation website and, within days, The Bear Facts magazine and information pack arrived and alongside brilliant support from my local hospital, I’ve been able to adjust to this new aspect of my life. My diagnosis also resulted in me taking early retirement from teaching, so you could say it’s been life changing. Whilst visiting Edinburgh in November last year, my husband and I popped into the PBC office, to introduce ourselves, and were warmly greeted by Linda and I live in the Solihull area with Roger my lovely Alan. I found myself enquiring about becoming a husband and Maiya and Mylo our little dogs. volunteer and here I am! I’d love to get together with anyone from the area, for a chat and a coffee initially, I was diagnosed some time ago. At first my mind went and then hopefully we can start thinking of some into shutdown as I thought this is not happening to fundraising (nothing too ambitious to start with!) me. Fitness, Professional teaching, Tai Chi, Student I’m looking forward to hearing from you and beginning of Practical Philosophy, conscious of what I ate, so I my role as a volunteer. Lynn Robson thought I can handle this.

Now the symptoms were getting in the way of work; I Fife mentioned to my GP I was concerned I was becoming Hi everyone, a hypochondriac, the answer was no, it’s just Our January meeting in Glenrothes was attended symptoms of the PBC. I went home, searched Google by most of our regular members and a new member and found my life saviour, the PBC foundation. Wow! which was great and it was lovely to see you all again. I got in touch and realised I was not alone. I also hope to be there for others, who like me are scared of what Our next meetings are scheduled as follows:- the future has in store. Sunday 26 July 2015 - Glenrothes Sunday 25 October 2015 – Dunfermline I’m hoping to start fundraising and looking forward to Sunday 24 January 2016 – Glenrothes meeting as many from my area as possible. Full details are listed in the Dates for your Diary I hope you will get in touch either by email, section. [email protected] or phone me on 01564 774 At our last meeting we chatted about organising a 643 leave a message. coffee morning to raise funds and awareness and we’re picking up on this again at our April meeting in During the better weather I like bowling and I hope Dunfermline. to start Tai Chi classes for PBC as soon as I find a venue. If you would like to attend any of the Fife meetings or would like to be involved in the coffee morning I’ve just returned from my first PBC Annual then please just let me know. In the meantime if you Conference and believe me, I’ve witnessed such need anything I can be contacted on 07834690053 warm, friendly and caring people doing their work with or [email protected] Mo Christie such honesty and dedication. Esther Daniel

The Bear Facts 10 Spring 2015 The PBC Genetics Study REGIONAL VOLUNTEERS Research Team REGIONAL VOLUNTEERS 01223 769088 REGIONAL VOLUNTEERS Kent & East Sussex Hi Everyone, Hampshire We had our first meeting of the New Year on the 8th Hi everyone, March on a lovely spring afternoon. We had a good The annual LiDBA bike ride is taking place on th attendance and it was great to see once again Monica Sunday, 7 June 2015 starting at 10am from and Alan Davies and Meryl Church. We were delighted Bohunt School, Longmoor Road, Liphook, to hear that Paula Allinson has agreed to become a Hampshire. I usually run a tombola on the day volunteer for the Group. We had our afternoon tea where with my co-volunteer Jane Thomas and willing there was time for a chat to catch up on members’ news. helpers. I also hope to have a team of riders We held our usual raffle which together with the on the day. We usually raise a small amount of proceeds of the boxes brought by Dee and Paula raised money from this but more importantly are able £86. to raise awareness of the condition and the work Our next meeting this year will be on Sunday 7th of the Foundation. If you get a chance to come June at the school between 2pm - 5pm. All are welcome along, we’d love to see you. including family and friends, and, if possible, bring a Looking ahead, on Saturday 12th September small raffle prize and a plate of food to share for the 2015 I will be holding a Quiz Night as part of the afternoon tea (all coffee and tea is provided). International PBC Day. This will start at 7.30pm The dates, times and venue for our future meetings at Liphook Bowling Club. The cost per ticket is are listed in the “Dates for your Diary” page in the Bear £2.50 and there will be a raffle with many lovely Facts. We look forward to seeing you all whenever you prizes. As I am limited to numbers, this will be can make it. a ticketed event only (with a map on the reverse John Creed and Mary Woods of the tickets). Please contact me as soon as John Tel: 01474813098 possible on 01428 725399 or email me at bob_jo_ or e-mail: [email protected] [email protected] to reserve a place. Mary Tel: 01732848693 Jo Elliott or e-mail: [email protected] West Sussex Somerset Hello everyone, Hello to all members in Somerset, As you may be aware, I am also a Volunteer for Dean and I will be holding our first meeting for the the Hampshire area. Members in West Sussex who area in a lovely room at the Princes Theatre, Princes live close to Hampshire may be interested in the Street, Burnham on Sea. It is to be held in the Pizey following event. The annual LiDBA bike ride is taking Room which is upstairs but there is access via a place on Sunday, 7th June 2015 starting at 10am lift. Directions to the room are as follows: enter and from Bohunt School, Longmoor Road, Liphook, pass the ticket office, you will see the stairs directly Hampshire. I usually run a tombola on the day with ahead of you, the room is on the first floor. Should my co-volunteer Jane Thomas and willing helpers. a lift be necessary, past the ticket office there is a I also hope to have a team of riders on the day. We turning to the left with a sign to the lift. The room usually raise a small amount of money from this but is to the right. I will put some directions up through more importantly are able to raise awareness of the the theatre. Coffee and tea will be provided by the condition and the work of the Foundation. If you get theatre coffee shop and we will, if people are happy a chance to come along, we'd love to see you. to, hold a small raffle, the proceeds of which will go directly to the PBC Foundation. If any member would I am holding two meetings at The Gatehouse, like to bring any handmade cards or items of interest, Chichester Gate from 11am to 12.30pm on Saturday, you are welcome to do so. 5th September and Saturday, 7th November 2015. If We are really looking forward to holding the you live in or around the West Sussex area, or are in meeting at this exciting venue and hope that many the area on that day, it would be lovely to see you – if of you in the area can attend. There are buses that you could let me know beforehand this would helpful run nearby, the numbers of which I am not familiar – please see details in the diary section. with. Trains run into the Highbridge & Burnham As usual, if you would like a meeting in your area, junction with a bus stop outside. could you please contact me with suggestions If you need more information regarding times, I for suitable venues. My contact details are 01428 will endeavour to find out the details nearer the time. 725399 or email me at [email protected]. Here’s looking forward to seeing some of you soon. Jo Elliott Di and Dean Rundle

Spring 2015 11 The Bear Facts REGIONAL VOLUNTEERS The PBC Foundation (UK) Ltd REGIONAL VOLUNTEERS Helpline Number REGIONAL VOLUNTEERS 0131 556 6811

Chief Executive Officer: Collette Thain, MBE West Lothian & Helpline: 0131 556 6811 Fax: 0131 556 8488 Angus North East England Geoff Bray Elisa Duffew Lanarkshire Tel: 01307 819100 Tel: 01287 622946 Borders & Berwickshire Northern Ireland (West) Hello everyone, Ena Mitchell Philomena McPeake Tel: 01750 42244 Tel: 07547 037942 Later this year, in the summer months, E: [email protected] Northamptonshire I am organising a fundraising abseil for Cambridgeshire Mary Bates Alison Cowzer Tel: 01327 350349 willing PBC members, and their healthy, Tel: 01733 769057 able bodied family and friends. The Margaret Lynch Cheshire/Derbyshire/ Tel: 01327 350899 event will take place at the Edinburgh Manchester Team Lynn Warren Nottinghamshire International Climbing Arena, at Ratho, and Tel: 0161 973 0638 Wendy Wheat will involve a period of instruction followed Tel: 07584 026815 Cleveland by the abseil, under the expert guidance Lynn Robson Sylvia Hodgkiss Tel: 07872 643242 Tel: 07549 429254 of an instructor. Obviously all necessary E: [email protected] E: [email protected] safety precautions will be taken including Edinburgh Oxfordshire harness, helmet and someone qualified Esther Davidson Trixie Rowe Tel: 0131 467 7260 Tel: 01869 349886 to belay. If you are physically limited in or 07791 952451 your ability to climb as a result of joint pain Perthshire Fife Ann Cameron then worry not! I will be taking the lift to Mo Christie Tel: 01828 628829 Tel: 07834 690053 the top of the arena where the Sky Ride Republic of Ireland begins, and the team at Ratho are very Gloucestershire & Bristol Marian Power Sarah Mustoe Tel: (353) 1 2950528 accustomed to supporting people with Tel: 07544 658614 Somerset disabilities and limitations. Hazel Nelson Diane & Dean Rundle Tel: 01179 575839 Tel: 01278 760426 My own offspring are eager to participate Hampshire Team Stirlingshire Jo Elliott Gillian Creelman (still not sure how they managed to Telephone: Contact Jane Tel: 01324 880256 Thomas in First Instance persuade me to do it though!) and some of Wales (South East) their friends are also being roped in (sorry, Jane Thomas Rachel Powell Tel: 01256 811187 Tel: 01495 301481 had to use it). E: [email protected] Wales (South & West) Hertfordshire Val Harris So far, our Mo Christie from the Fife region Majella Hayman Tel: 01792 447006 Tel: 01992 415 193 E: [email protected] has expressed not only an interest... but Inverness-shire and Wales (North) positive enthusiasm for the event, and Highlands Pat Rannard Mo’s star fundraising daughter, Eve, might Paul Humphreys Tel: 01407 740472 Tel: 01847 851275 also join in. In addition to participants West Midlands/ Kent & East Sussex Worcestershire Team willing to undertake the abseil, we will have Mary Woods Brenda & Eric Whitmore PBC members on site to man a stand, Tel: 01732 848693 Tel: 01922 402705 offering information leaflets, publicity and John Creed Anne & Mervyn Harper merchandising to raise awareness. Tel: 01474 813098 Tel: 01562 885391 Paula Allinson Esther Daniel Tel: 01959 577830 Tel: 01564 774643 I’m also hoping to get some media E: [email protected] E: [email protected] coverage of the event, and the PBC Lanarkshire & West Lothian West of Scotland Foundation staff are going to help with this. Fiona Selalmas Catherine Gildea Tel: 07802 543549 Tel: 0141 334 3724 Pauline Bain Tel: 07780 708045 West Sussex The date will be booked once I have some Jo Elliott Lincolnshire Tel: 01428 725399 indication of how many people might be May Dunwell E: bob_jo_elliott @yahoo.co.uk interested in taking part - so if you fancy Tel: 01724 337314 West Yorkshire the challenge and a fun day out, for our Morayshire Lisa Workman Irene Ferguson Tel: 07754 787214 excellent cause then please get in touch by Tel: 01542 834620 E: [email protected] email [email protected] Fiona Selalmas & Pauline Bain Email: [email protected] Website: www.pbcfoundation.org.uk

The Bear Facts 12 Spring 2015 The PBC Genetics Study DATES FOR YOUR DIARY Research Team DATES FOR YOUR DIARY 01223 769088 DATES FOR YOUR DIARY

Date Venue Time Organiser

JUNE 2015 04/06/2015 Holiday Inn (Coffee Bar), Corstorphine Road (next to zoo), Esther Davidson 0131 467 7260 EDINBURGH, EH12 6UA 7.00pm - 9.00pm or 07791 952451

07/06/2015 The Granville School, 2 Bradbourne Park Road, Mary Woods 01732 848693 or Sevenoaks, KENT, TN13 3LJ 2.00pm - 5.00pm John Creed 01474 813098

07/06/2015 LiDBA Bike Ride, Bohunt School, Longmoor Road, Liphook, Jo Elliott HAMPSHIRE, GU30 7NY 10am onwards 01428 725399

12/06/2015 Princes Theatre (Pizey Room), Princes Street, Di & Dean Rundle Burnham on Sea, SOMERSET, TA8 1EH 10.00am - 1.00pm 01278 760426 or 07886 087868

15/06/2015 Self Management Development Session, Friends’ Manchester Meeting House, PBC Foundation Office 6 Mount Street, MANCHESTER, M2 5NS 10.00am - 3.30pm 0131 556 6811

17/06/2015 Self Management Development Session, The Cider Museum, PBC Foundation Office Pomona Place, Hereford, HEREFORDSHIRE, HR4 0EF 10.00am - 3.30pm 0131 556 6811 JULY 2015 11/07/2015 Hazel Nelson's home, Hazel Nelson BRISTOL (Please conatct Hazel Nelson for details) 12.00 noon - 2.00pm 01179 575839

13/07/2015 Hillcroft Hotel, East Main Street, Whitburn, Fiona Selalmas WEST LOTHIAN, EH47 0JU 10.00am - 12.00 noon 07802 543549

26/07/2015 Holiday Inn Express, Leslie Roundabout, Mo Christie 07834 690053, Glenrothes, FIFE, KY6 3EP 2.00pm - 4.00pm [email protected] SEPTEMBER 2015 03/09/2015 Holiday Inn (Coffee Bar), Corstorphine Road (next to zoo), Esther Davidson 0131 467 7260 EDINBURGH, EH12 6UA 7.00pm - 9.00pm or 07791 952451

05/09/2015 Trixie Rowe's house, Trixie Rowe OXFORDSHIRE (Please contact Trixie for details) 12 noon onwards 01869 349886

05/09/2015 The Gatehouse (Weatherspoons), Chichester Gate, Jo Elliott Terminus Rd, Chichester, WEST SUSSEX, PO19 8EL 11.00am - 12.30pm 01428 725399

12/09/2015 Quiz Night, Liphook Bowling Club, London Road, Jo Elliott Liphook, HAMPSHIRE, GU30 7AN 7.30pm onwards 01428 725399

13/09/2015 International PBC Day 2015

13/09/2015 The Granville School, 2 Bradbourne Park Road, Mary Woods 01732 848693 Sevenoaks, KENT, TN13 3LJ 2.00pm - 5.00pm or John Creed 01474 813098 OCTOBER 2015 25/10/2015 King Malcolm Hotel, Queensferry Road, Mo Christie 07834 690053, Dunfermline, FIFE, KY11 8DS 2.00pm - 4.00pm [email protected]

31/10/2015 Venue to be confirmed, BRISTOL 11.00am - 1.00pm Hazel Nelson 01179 575839

31/10/2015 Hillcroft Hotel, East Main Street, Whitburn, Fiona Selalmas WEST LOTHIAN, EH47 0JU (Halloween theme) 10.00am - 12.00 noon 07802 543549 NOVEMBER 2015 07/11/2015 The Gatehouse (Weatherspoons), Chichester Gate, Jo Elliott Terminus Rd, Chichester, WEST SUSSEX, PO19 8EL 11.00am - 12.30pm 01428 725399

12/11/2015 Holiday Inn (Coffee Bar), Corstorphine Road (next to zoo), Esther Davidson 0131 467 7260 EDINBURGH, EH12 6UA 7.00pm - 9.00pm or 07791 952451

22/11/2015 The Granville School, 2 Bradbourne Park Road, Mary Woods 01732 848693 or Sevenoaks, KENT, TN13 3LJ 2.00pm - 5.00pm John Creed 01474 813098

Spring 2015 13 The Bear Facts PROJECT UK The PBC Foundation (UK) Ltd PROJECT UK Helpline Number PROJECT UK 0131 556 6811

pictures and a little of what you were doing, and what activity led you there. If this is of any interest at all to you, then please do get in touch with us and we can give you the information to get you up and running.

As we have said here often, this is an incredibly exciting time within the world of PBC with developments occurring in each and every field. The International Liver Congress in Vienna hosted by EASL was incredibly important for PBC, and not just for the announcement of the name change!

There was a symposium on PBC where many new things were presented. Firstly, there was a PBC Risk Management Score which comes from one of the work strands within UK-PBC. For a long time, we have known about Urso response/non-response and that there needs to be different levels of medical care for people dependent on where they are on their PBC journey. There have been concerns from members with regards to them “only seeing their consultant once a year to monitor bloods”, etc. The key is that sometimes this is appropriate: in many cases, this is the best and most efficient use of resources as well as providing an appropriate level of care to someone with non-progressing or slow-progressing PBC. However, sometimes this level of care is absolutely inappropriate as a patient may need more care and more monitoring. What we need to do is assess each individual case and make sure every single patient gets the best level of care within their PBC. We hope that the PBC Risk Assessment Score will be of huge help with this in the future. s you will have already read on the front page, we are absolutely The formula does tend to be cautious and therefore overstate, albeit slightly, the potential risk in each patient but that is better than underestimating any potential delighted with the news that The A risk. Keep an eye on our next edition where you can read Dr Mells’ own words on PBC Foundation now supports those the score and what it means for people affected by PBC. affected by Primary Biliary Cholangitis. I know there has been much said around As alluded to in the Letters page, we have also been involved in the writing of the the name change and not everyone was new UK guidelines on the care of PBC. Again, in conjunction with many great minds within the health professionals working in PBC (including many on our Medical in favour but it has now happened. It was Advisory Board) and with BSG (the British Society of Gastroenterology) we are putting the will of the vast majority of stakeholders, into place the UK guidelines which will cover everything from diagnosis through including patients, patient advocates, to level of care, be that with trained specialists or with liver units. The whole idea health professionals and those working behind these guidelines is to help people with a prompt diagnosis and then referral within the pharma/biotech industries and to the most appropriate service as quickly as possible. We are optimistic that these guidelines will be finished this summer but we shall keep you posted either here or we are absolutely delighted to have a name in our newly redesigned website. for this condition which doesn’t induce stigma, judgement or isolation through Did someone mention website? Yes, we are delighted to say that we are well on our inherent inaccuracy. way to releasing our new website. In fact, we fully anticipate that the website will be live well before you get your next edition of the Bear Facts in August. Not only will We shall now embark on a sponsored awareness the new website be in English, but we are setting up the key information pages in 16 campaign to highlight the name change and to ensure other languages. Add to this our new compendium in June which will also be added that even more people know the facts about PBC, not steadily as that is translated into more and more languages through the summer. the dusty myths, legends or inaccuracies of old. As we have been anticipating this change happening, Our intention is for the website to be much more interactive with more input, more we have been running down our old stock of pens, updates, more live news as well as incorporating even more twitter and facebook leaflets, etc. If anyone would like a reminder of the feeds into the home page. If you follow us on twitter or on facebook, you will be kept old days, then now is the time to order some of our informed of updates and short-notice events also. merchandise with the old name on it. If you would like to follow us on twitter, you can follow #PBCFoundation and/or We very much hope that you will be part of the #PBCRobert. We regularly update our information there, letting you know what is awareness campaign. We would love to hear from going on live as it is happening. you if you have had any successes or any funny stories when you have been spreading the word. We have much to look forward to next year as we celebrate 20 years of the To help you, we shall be printing new leaflets, etc., Foundation. One of the ideas for next year is to hold an art exhibition which highlights with the name change. As a member, you have a the PBC Journey. We would be looking to form a team of artists, each of whom would wonderful opportunity to reach more people and spend time talking with someone affected by PBC. Through this process, the artist shatter the ignorance that surrounds PBC. One of the would be inspired to create a piece that best describes that journey or a moment things that we have which could help is an information within it. This will be a major project and we are looking to use it to highlight the pack linked to our “Brew with a View” initiative. difficulties faced by those affected by PBC, to celebrate resilience, innovation and the changing landscape of those affected by PBC as well as to raise awareness In simple terms, we are hoping to hear from you and and funds. to see your pictures of any activities you are doing and also some photos of where you show us where Would you like to be involved? Are you an artist? Are you affected by PBC and feel you are enjoying your well-earned brew and with you have something to say, to share or to inspire? Do you know of an artist that you whom. It would be a great pleasure to share your feel could be involved? If this is the case, then please contact us.

The Bear Facts 14 Spring 2015 The PBC Genetics Study PROJECT UK Research Team PROJECT UK 01223 769088 PROJECT UK

We are absolutely delighted to share with you first another upcoming One of the other ways in which we are trying to positively affect project. There will be a formal announcement very soon and there diagnosis numbers within the UK is by our involvement in a joint shall be a full article in the August Bear Facts giving you the details. working group which was designed to look at abnormal liver tests and However, what I can say is that Perspectum Diagnostics have the protocols surrounding them. This is another focus working group generously donated a software package which will allow for colour set up with the BSG and is a multi-disciplinary team incorporating MRI technology to be used for measuring cell change within the consultants, specialists from laboratories, GPs and patient groups liver in PBC. We shall put out to tender the opportunity to be granted amongst others. We shall be looking at current protocols, which this technology for a hospital. The offer of the technology comes we know to be unsatisfactory, and making recommendations for with training and technical assistance also, which is an incredible future protocols for how health professionals deal with patients with package. We very much thank Perspectum for their generosity and abnormal liver tests. look forward to this project coming to fruition by the end of this year. Historically, liver biopsy was used to “stage” the histology, or cell We have realised just how big this task is and this will take change, within the liver. For all that it was the best we used to have, considerable time to ensure we provide evidence-based data and it was never the “Gold Standard” in terms of detail throughout the recommendations to ensure that anyone with abnormal tests, not entire liver. Even now, liver biopsy is still not without risk so we are just those affected by PBC, are diagnosed and helped within a much always looking for better scanning technology. Nowadays, more shorter time frame and with much greater effectiveness. liver clinics are using ultrasound, MRI and fibroscan technology to look at damage within PBC. The intention behind this project is to It is with great pleasure that I can tell you that The PBC Foundation discover whether or not colour MRI gives a better idea of the level has representation on the steering committee of ELPA, The European of damage throughout the whole liver than biopsy does. Again, look Liver Patients’ Association. The steering committee is responsible for the announcement and the details within the next edition. for the direction and strategic outline of ELPA’s work and to inform the process of decision and policy making within ELPA itself. This is This year, more than ever, raising awareness is going to be one of another wonderful opportunity for us to be able to positively influence our key issues to address within the Foundation. One of our ongoing outcomes for patients throughout all of Europe and we very much campaigns is to give the medical professions the information and look forward to contributing on this platform. support they need to diagnose PBC quicker and earlier within the disease. When we look back at members who were diagnosed We shall be working to further our reach in terms of information, even just five years ago, their average time from first symptoms to patient support and advocacy ensuring our members throughout diagnosis was far too long, often being cited as years. Our newer Europe and the world have the same services as in the UK. members are now talking in terms of just months between first symptoms and diagnosis. Whilst we acknowledge there is still much This edition has been so full of announcements, of new projects to be done, we have been very successful in educating the health and of innovations and developments that I’ve not even had the professions about PBC. chance to discuss with you the Volunteers’ Conference in April or even the formation of our brand new “Events Team”. The conference One of the tools we have found most useful is the Nurses in Practice continues to grow from strength to strength. It is wonderful to see events. Hosted by Cogora, these events have grown in reach and in the Volunteer network continue to grow in number, in quality, in number since our first involvement. These events are now hosted in outreach and in confidence. The Foundation is blessed with many 12 cities around the UK and they give us a real opportunity to speak hard working Volunteers who are your local contacts, who work hard directly to nurses, health visitors, etc., giving them all the information to make sure you have local meetings within a reasonable distance, they need to deal with PBC in much better terms. who ensure they are informed about our work and how we can help you, our membership. Here, you can see Linda at one of our NiP stalls. This is where we stand for the day speaking to between 300 to 1,000 nurses giving We are delighted with another new development for 2015: our “Events out lots of leaflets that find their way into GPs’ hands also. Team”! They are a group of Volunteers who will help however and whenever they can in any fundraising events. These may be events you are hosting, we are hosting or others are hosting on our behalf but they shall be working hard to ensure the event goes with a bang and that we raise as much awareness and profile as well as much needed funding as possible.

If you have read our Letters page, you will have read some of the reactions from our members who have joined us on one of our self- management sessions. We continually strive to have in place the funding to make these happen. We are proud of the partnerships we have formed. In fact, we learned in May that two of our partners have now been funded to start their own self-management modules, which is just fabulous.

Please check in our Diary pages to see if we are hosting a workshop near you soon. In June, we will be in Manchester, Hereford and in London. The London event is a last minute opportunity afforded us by Royal Free Hospital. We do not yet know the venue of the London event, for all we know it will be in the hospital. We shall also benefit from hearing from one of the resident hepotologists within the unit, Dr Douglas Thorburn. If you would like to attend any of these events, then please do contact Alan or Linda at the office to book your free place at this event. Until the next time… Robert Spring 2015 15 The Bear Facts YOUR LETTERS The PBC Foundation (UK) Ltd YOUR LETTERS Helpline Number YOUR LETTERS 0131 556 6811

We want to hear your news and views on anything – not only PBC – as this is your magazine and we need input from you. For this page only, please address correspondence to: The Editor, PBC Foundation (UK) Ltd, 2 York Place, Edinburgh, EH1 3EP, Scotland, UK.

If you would like to reply to any of the letters featured on these pages, please do so by sending your letter to PBC Foundation (UK) Ltd (unless otherwise stated), marked for the appropriate person’s attention and we will forward it.

Dear Editor Dear Editor How I understand how Debra Tyson felt from her letter in the latest magazine. First of all our thanks to Collette for returning my husband’s call recently When we lived in Kent the doctors at the hospital looked at the liver readings and regarding my problem obtaining concluded “drinker”, and wanted to do nothing! travel insurance and secondly to say I am sorry for not answering Eventually, after very stern words at the hospital, saying I am not a drinker, and sooner. This is solely due to the time demanding to see a proper liver specialist, I got a diagnosis. Up to then they consuming and massive problem had found type two diabetes and under-active thyroid, and no gall stones. Also we have had filling in online and a hiatus hernia and acid reflux! telephone applications trying to find a travel insurance policy. The liver man said “Do you itch?” At the time I could scratch skin raw with dreadful itch! So I was finally put onto UDCA. We have no problem finding cover for my PBC as a pre-existing condition - When we moved to Somerset I found Dr Gotto at Yeovil more up to speed with as many insurance companies now PBC. Itch now controlled well with Rifampicin, and he has several other patients recognise this. I found the problem now on this, most free of itch, and one with greatly reduced itch. When first put arises as soon as you mention the on it we were the first along with one lady he was putting on this medication. words “clinical drug trial” and once learning this information they refuse So it makes a great difference when you get medical staff who understand PBC, to insure me for anything (even and you are not treated as someone who brought problems on yourself by excess omitting the PBC). It seems they are drinking. not interested because they do not want to take the risk of a “clinical Thanks for your informative magazine, it’s great not to feel alone with serious drug trial” client. problems. Kenneth Holmes, Somerset On further investigation we have at long last found an insurance company namely Global Travel Insurance who Dear Editor Dear Editor will give us a one year joint European I just wanted to say thank you for Please find enclosed a donation and multi-trip policy for £158 with NO the workshop held in Plymouth in I would like to say how much I look medical screening required, on the April. I had never been to one before forward to the Bear Facts magazine. It downside the excesses are much and didn’t know what to expect but is good to read the letters from others higher than normal! I hope this may it was very relaxed and informative. who have PBC and be made aware of be of some help to others who may It was also lovely just to be able to the latest developments in treating the find themselves in a similar situation. talk with other people who have the condition. Christina Gordon, London same condition, maybe we all have I would also like to take this Editor’s note: different symptoms but we all sort opportunity of thanking you so much The following enquiry was received of get it. I did want to put a post on for sending me recently by the PBC Foundation. Facebook but I’m not sure how (don’t The “Bear Facts” Magazine regularly Unfortunately, Mr Dixon is not a use it that much) just recommending which I very much enjoy reading, member of the PBC Foundation and that if anyone has the opportunity to and for all the the Editor would invite Mr Dixon to attend a workshop it is well worth hard work you and your team do on contact us directly with his address making the effort! So again thank our behalf. or contact details so we can ensure you for an enjoyable day. Jean Dodd, Oxfordshire Helen Black, Devon our response is received successfully.

The Bear Facts 16 Spring 2015 The PBC Genetics Study YOUR LETTERS Research Team YOUR LETTERS 01223 769088 YOUR LETTERS

Dear Editor I have called the office several times Dear Editor Dear Editor and spoke with Alan who is always I have just read through one of I would like to thank you so much for happy to deal with enquiries. the magazines and have found it your valuable information. extremely interesting and helpful. I also wanted to inform all PBC sufferers The website has been such a huge about the self-management workshops Reading the article on ‘Whether help to me as there is nothing here Robert delivers. There was one held in to move or not’ has inspired me to in New Zealand that I can find my hometown of Bournemouth. Well, seriously consider going back to and I have been able to use the what a lovely low key meeting. It was swimming again which I used to information supplied to take much so nice to meet and talk with fellow do nearly every day before I was more control of my illness and sufferers and find out all we needed to diagnosed over 3 years ago. ability to recognise symptoms and know and more about PBC. It was truly progress. an informative and happy day. Robert I’ve come a long way in that time and is an asset to the Foundation in the I feel some sort of exercise would be To read the experience of others has way he took the group. of benefit. been fabulous. Pat Wallace, North Yorkshire Susan Higgs, New Zealand I would say to anyone, if you have never been to a workshop, I would strongly advise you to go. It is informative, Editor’s note: everyone is so friendly and we had a The following enquiry was received recently by the PBC Foundation. lot of fun. Simply a really good day to Unfortunately, Mr Dixon is not a member of the PBC Foundation and the Editor learn more about your PBC. would invite Mr Dixon to contact us directly with his address or contact details Janet Edwards, Dorset so we can ensure our response is received successfully.

Dear Editor Dear Editor I recently contacted the Foundation Back in November 2013, I presented myself to the doctor as I was feeling office for help in finding travel really fatigued. The doctor ordered a blood test and when the result came back referred me to a liver unit at the local hospital while waiting for the appointment. insurance. I also had an ultrasound on my liver. It was not until June 2014 that I eventually got to see a Professor at the hospital who confirmed after further blood tests Alan provided me with a list of that I had early stage PBC. I was prescribed Ursodeoxycholic Acid. companies but I couldn’t find any This year, April 2015, I moved to a new area and joined a new medical company who would insure me, practice. I had with me a letter from the liver clinic saying I should be referred including the PBC. They all seem to the liver clinic in the local hospital of my new area. The doctor I saw did not to think it’s akin to the Black Plague, know of PBC and looked it up in his medical book. He sent me to have further which is ridiculous. bloods done and when he got the results back, he said my LFT had come back to normal and told me I was now in remission. He also said he was going to stop prescribing Ursodeoxycholic Acid. I have, however, found cover with He also refused to make the referral to the local hospital. I fear he is wrong JustTravelCompany.com, excluding as I have not read of anyone in remission. I would be pleased if you can advise my previous illnesses, for eleven me in any way. days in the USA. They wanted nearly Paul Dixon, Cleveland £500 to cover me for the PBC. Other insurance companies I tried don’t The enquiry was forwarded to our medical advisers for their perusal. The provide cover excluding previous response to the enquiry, provided by Professor David Jones is detailed below: illnesses any more. Dear Mr Dixon The PBC Foundation passed this on to me to look at. I am really pleased that I found Just Travel Company in a your liver blood tests have returned to normal with Ursodeoxycholic Acid. This newspaper advert. It’s very reasonable is what happens in about half of PBC patients taking it. It does not mean, at £50.75 for luggage, cancellation, however, that the disease has gone away (you are right that there is no such and any new illness. thing as remission in PBC) just that it is well controlled by the treatment.

I hope this information might be useful Advice in all the treatment guidelines (including the UK guidelines which we for anyone else who doesn’t mind are currently writing) is that you continue with UDCA life-long, even if the travelling without insurance for their blood tests have returned to normal. Stopping UDCA always leads to the tests PBC. deteriorating. Professor David Jones, Consultant Hepatologist, Freeman Hospital, Pamela Barratt, Cheshire Newcastle

Spring 2015 17 The Bear Facts FUNDRAISING & AWARENESS The PBC Foundation (UK) Ltd FUNDRAISING & AWARENESS Helpline Number FUNDRAISING & AWARENESS 0131 556 6811

Greetings . . . as ever, I trust this article finds you well and enjoying the full panoply of spring. My walk to and from the PBC Office is a riot of cherry blossom, bluebells and daffodils at the moment and the buds are bursting with life.

As the seasons bring renewal, so we look at new developments on the horizon for the Foundation: International PBC Day 2015: 13th September 2015

As strange as it seems to be talking about the autumn in the same breath as spring, we want to sow the seeds of ideas in your brains now. International PBC Day is a key opportunity for the Foundation and our members Rob Waugh to involve the community at large and raise awareness of the condition as widely as possible.

The ongoing mission of PBC Day for the Foundation is to get members to be more physically active and to involve others in this, educating them about PBC in the process.

In keeping with this, the theme for 2015’s PBC Day is “Brew with a View”.

This follows on from a previous year’s theme but whereas that was focussed on mountaineering, this time we want you to be as creative as possible, e.g., the “brew” could be a Mojito and the “view” could While fun and entertainment were the attraction, the purpose of the be palm trees and azure seas, or a night was to raise funds and awareness for the PBC Foundation. thermos flask up the London Eye. Among the many messages we promote among members is that The possibilities are endless. staying active can be highly beneficial in improving quality of life We want to involve as many of you around the country, and the and dancing is a prime example of that. world if possible, in a collective campaign on the 13th Sept to One of the dancers on the night, raise awareness en masse. We want you to organise a local Mhari (from Haddington), herself event or personal challenge in your local area, let us know what has PBC and training was hard but that is, where it is and share photos of the event. We’ll provide rewarding for her. She, along with you with information packs to plan your activities and plot each the rest of the competitors, ensured individual event on a map to show what a wide network of PBC that competition for the top prize friends there are out there. was tight. However there has to be The event could be a coffee morning in a beautiful location where a winner and it was Darren "The you tell your PBC story, or you could invite your friends and Dazzler" McLean of Pencaitland. His their friends to hike up a mountain for a cuppa on the summit at high tempo routine and snake hips sunrise? Get your thinking caps on. won the crowd's affection and he was duly presented with the trophy by the We’ll be putting more information on our website and social Commonwealth Judo Gold Medalist media, but if you would like to be kept posted and receive an info Renicks sisters. The biggest cheer of the night was saved for the pack, drop a line to [email protected] moment when the total raised by the competitors, a whopping £8,468.64, was announced to the audience. Looking back on the past quarter, we have had some fantastic opportunities to raise awareness and funds. I want to thank Since then, even more funds have come in and our best estimate you for being so proactive in visiting Coffee#1 to buy charity of the cash profit is £19,500, which goes a huge way to supporting gingerbread men. While we’ll talk about this in the next issue, it our work in an increasingly competitive funding environment. was a resounding success. I also want to thank our competitors in the Virgin London Marathon, who raised in excess of £10,000 As ever, we appeal for your help in supporting our fundraising between them and even got a primetime interview on BBC Radio efforts. We have a small team and the demands on our services 5Live before the event. More of that in the next issue. must be met on a constant basis by fundraising, which boils down to me. Many corporate funders only donate to charity through ★ ★ employee schemes, e.g., John Lewis, W H Smith and New Look. Strictly Come Dancing Fundraiser Wherever you work, please check with your employer if they have On Saturday the 14th February, nine Edinburgh-based novice a charity donations scheme for Employees. Alternatively, if they dancers took to the floor with their professional partners ina offer paid leave to do charity work as companies like Aegon do, Strictly Come Dancing style competition. Most of the competitors then could you use that time to organise a Brew with a View on had never done any sort of dancing before and had only 1-2 International PBC Day? months of lessons prior to the event. The results surpassed everyone's expectations as routine after routine was cheered on I look forward to hearing from you. by over 200 members of their friends and family from Edinburgh, But until then, keep well the Lothians and Fife.

The Bear Facts 18 Spring 2015 The PBC Genetics Study FUNDRAISING AND AWARENESS Research Team FUNDRAISING AND AWARENESS 01223 769088 FUNDRAISING AND AWARENESS

New Information Leaflets Available

B C M Y B C M Y 40% 80% B C M Y B C M Y Prinect/FOGRA 4 Dipco 2.1 Format 74 © 2004 FOGRA/Heidelberger Druckmaschinen AG 80% B C M Y B C M Y 40% 80% B C M Y B C M Y 40% 80% B C M Y B C M Y 40% 80% B4 C4 M4 Y4 MY CY CM B C M Y 40% 80% B C M Y CMY CMY B4 C4 M4 Y4 40% 80% B C M Y B C M Y 40% 80% B C M 5 2 5 2 Please5 2 5 2 contact the office if you require us to send you any of these leaflets 4 3 4 3 4 3 4 3 3 3 3 3 2 4 2 4 2 4 2 4 1 5 1 5 1 5 1 5 0 11 12 13 14 15 16 17 18 19 2 together with dark pigmentation of the skin, particularly the year or two. Furthermore, the severe symptoms of tiredness and together with dark pigmentation of the skin, particularly the year or two. Furthermore, the severe symptoms of tiredness and trunk. The reason for the pigmentation is unclear – it may be lethargy, sometimes pruritus, can be out of all proportion with trunk. The reason for the pigmentation is unclear – it may be lethargy, sometimes pruritus, can be out of all proportion with related to persistent scratching. A few patients have marked the underlying histological, or biomedical abnormalities. These related to persistent scratching. A few patients have marked the underlying histological, or biomedical abnormalities. These PBC Foundation (UK) Ltd hypercholesterolaemia with xanthomas and xanthelasmata symptoms do not necessarily respond to ursodeoxycholic acid, hypercholesterolaemia with xanthomas and xanthelasmata symptoms do not necessarily respond to ursodeoxycholic acid, 2 York Place around the eyes and, occasionally, elsewhere. A small or other treatments currently under investigation. In patients around the eyes and, occasionally, elsewhere. A small or other treatments currently under investigation. In patients Edinburgh proportion of patients present with the signs and symptoms of complaining of fatigue remember that thyroid disease is common proportion of patients present with the signs and symptoms of complaining of fatigue remember that thyroid disease is common portal hypertension – particularly ascites, or marked bleeding in patients with PBC (an overlap of autoimmune disorders) so check portal hypertension – particularly ascites, or marked bleeding in patients with PBC (an overlap of autoimmune disorders) so check EH1 3EP from oesophageal varices. thyroid function tests. from oesophageal varices. thyroid function tests. UK As for other end stage liver diseases, transplantation is now a As for other end stage liver diseases, transplantation is now a Tel: 0131 556 6811 Is there a familial risk of PBC? widely accepted option. Current results for liver transplant for Is there a familial risk of PBC? widely accepted option. Current results for liver transplant for PBC are better than for any other major disease group. Up to PBC are better than for any other major disease group. Up to Fax: 0131 556 8488 Some recent studies suggest that there is an increased familial ninety percent of patients transplanted for PBC should be alive Some recent studies suggest that there is an increased familial ninety percent of patients transplanted for PBC should be alive incidence of PBC – particularly between mothers and after one year, eighty percent after five years, with good quality incidence of PBC – particularly between mothers and after one year, eighty percent after five years, with good quality daughters. The exact size of this risk is unknown, but it is of life. The timing of transplantation is difficult. As with other daughters. The exact size of this risk is unknown, but it is of life. The timing of transplantation is difficult. As with other unlikely to be more than two percent. No clear genetic factor liver diseases, it is a combination of assessment of prognosis unlikely to be more than two percent. No clear genetic factor liver diseases, it is a combination of assessment of prognosis has yet been implicated. At present, screening of relatives of without transplantation (length of life) and severity of has yet been implicated. At present, screening of relatives of without transplantation (length of life) and severity of patients with PBC, even daughters, is not necessary, or symptoms (quality of life). This assessment should be made in patients with PBC, even daughters, is not necessary, or symptoms (quality of life). This assessment should be made in advised, unless relatives have symptoms or laboratory one of the UK Liver Transplant Centres. All centres are very advised, unless relatives have symptoms or laboratory one of the UK Liver Transplant Centres. All centres are very findings, which could be attributed to PBC. happy to receive referrals for initial consideration and advice, findings, which could be attributed to PBC. happy to receive referrals for initial consideration and advice, usually from consultant colleagues from any DGH. Patients who usually from consultant colleagues from any DGH. Patients who have no other major co-morbidity may be considered for have no other major co-morbidity may be considered for How common is PBC? transplantation into their mid-late sixties. How common is PBC? transplantation into their mid-late sixties.

This varies in different parts of the world. Recent studies in This varies in different parts of the world. Recent studies in the United Kingdom suggest that, at least in some places, the What treatments are available for other features of PBC? the United Kingdom suggest that, at least in some places, the What treatments are available for other features of PBC? prevalence may be as high as two hundred and fifty per million prevalencePBC may be as high as fortwo hundred and fifty per million PBC Patient PBC Foundation of the population, or one in a thousand women over the age of Pruritus: This is commonly treated with cholestyramine. Over of the population, or one in a thousand women over the age of Pruritus: This is commonly treated with cholestyramine. Over forty. A recent estimate is of twelve to fifteen thousand half of patients with pruritus respond to this treatment which forty. A recent estimate is of twelve to fifteen thousand half of patients with pruritus respond to this treatment which patients in the UK. As many as thirty to forty percent of these interrupts the enterohepatic circulation, possibly affecting bile patients in the UK. As many as thirty to forty percent of these interrupts the enterohepatic circulation, possibly affecting bile may be asymptomatic. The average age is sixty to sixty-five, acid metabolism, which may be associated with pruritus. may be asymptomatic. The average age is sixty to sixty-five, acid metabolism, which may be associated with pruritus. and about nine out of ten are women. Cholestyramine may cause dose related diarrhoea. and aboutGeneral nine out of ten are women. PractitionersCholestyramine may cause dose related diarrhoea. Information Booklet (UK) Ltd Cholestyramine binds bile acids and other medications, hence Cholestyramine binds bile acids and other medications, hence should not be given at the same time as ursodeoxycholic acid, should not be given at the same time as ursodeoxycholic acid, What specific treatment is currently available? or other regular medication. Second line treatments include What specific treatment is currently available? or other regular medication. Second line treatments include rifampicin. Dry skin is a prominent feature of the pruritus, so rifampicin. Dry skin is a prominent feature of the pruritus, so There is no ‘cure’ for PBC at present. The widely accepted liberal use of skin moisturisers is strongly recommended. There is no ‘cure’ for PBC at present. The widely accepted liberal use of skin moisturisers is strongly recommended. treatment is with the bile acid, ursodeoxycholic acid. Hypercholesterolaemia: Some patients have markedly raised treatment is with the bile acid, ursodeoxycholic acid. Hypercholesterolaemia: Some patients have markedly raised General Information Systematic analysis of the results of controlled trials of serum cholesterol. As with other patients this can and should be Systematic analysis of the results of controlled trials of serum cholesterol. As with other patients this can and should be ursodeoxycholic acid in PBC suggest that there may be some treated - preferably with a fibrate. ursodeoxycholic acid in PBC suggest that there may be some treated - preferably with a fibrate. slowing of the clinical course of the disease, although little Osteoporosis: Since PBC occurs most frequently in post slowing of the clinical course of the disease, although little Osteoporosis: Since PBC occurs most frequently in post benefit to symptoms. Ursodeoxycholic acid treatment is menopausal women and cholestatic liver disease may contribute PBC (Primary Biliary Cirrhosis) benefit to symptoms. Ursodeoxycholic acid treatment is menopausal women and cholestatic liver disease may contributePrimaryPBC (Primary Biliary Cirrhosis) surprisingly free of adverse effects. No consensus exists as to to osteoporosis, consideration should be given to assessing bone surprisingly free of adverse effects. No consensus exists as to to osteoporosis, consideration should be given to assessing bone Leaflet whether patients known to have PBC, but who are mineral density (DEXA Scan). If osteoporosis is present, treatment for General Practitioners whether patients known to have PBC, but who are mineral density (DEXA Scan). If osteoporosis is present, treatment for General Practitioners asymptomatic, should be treated with ursodeoxycholic acid is as for any other patient with osteoporosis. Primary asymptomatic, should be treated with ursodeoxycholic acid is as for any other patient with osteoporosis. and, if so, for how long. Prescribing for patients with PBC: In general, liver function is and, if so, for how long. Prescribing for patients with PBC: In general, liver function is well preserved until late (cirrhotic stage with evidence of portal well preserved until late (cirrhotic stage with evidence of portal Because of the ‘immune’ nature of the disease, a number of Professor O.F.W. James for The PBC Foundation Because of the ‘immune’ nature of the disease, a number of BiliaryProfessor O.F.W. James for The PBC Foundation hypertension) in its clinical course. This means that most hypertension) in its clinical course. This means that most immunosuppressant regimes have received clinical trials in the immunosuppressant regimes have received clinical trials in the medications may be given in a normal fashion. Because PBC is a medications may be given in a normal fashion. Because PBC is a past twenty years. The majority of these have either shown no past twenty years. The majority of these have either shown no cholestatic disorder, sex hormones should not be given without cholestatic disorder, sex hormones should not be given without benefit, or unacceptable side effects, or both. A number of Biliary benefit, or unacceptable side effects, or both. A number of a strong indication. Paracetamol containing medications are not a strong indication. Paracetamol containing medications are not controlled trials of, for example, antioxidant treatment, or low controlled trials of, for example, antioxidant treatment, or low recommended in late disease (certainly not more than 4 grams recommended in late disease (certainly not more than 4 grams dose steroids, are currently underway – usually in conjunction dose steroids, are currently underway – usually in conjunction Cirrhosis in any twenty-four hours). in any twenty-four hours). with ursodeoxycholic acid. Because of the unpredictable clinical with ursodeoxycholic acid. Because of the unpredictable clinical course of the disease, treatment and trials are difficult to interpret course of the disease, treatment and trials are difficult to interpret and advising individual patients about whether to take treatment Supplied as a service to medicine by: Cirrhosis and advising individual patients about whether to take treatment Supplied as a service to medicine by: The PBC Foundation (UK) Ltd The PBC Foundation (UK) Ltd is difficult. Some patients may remain asymptomatic for twenty Dr Falk Pharma UK Ltd, Unit K, Bourne End Business Park, is difficult. Some patients may remain asymptomatic for twenty Dr Falk Pharma UK Ltd, Unit K, Bourne End Business Park, 54 Queen Street, Edinburgh, EH2 3NS, Scotland, UK 54 Queen Street, Edinburgh, EH2 3NS, Scotland, UK years, while others can go from asymptomatic to complicated Cores End Road, Bourne End, Buckinghamshire SL8 5AS years, while others can go from asymptomatic to complicated Cores End Road, Bourne End, Buckinghamshire SL8 5AS (portal hypertension), even death, from liver disease within a Telephone: 0131 225 8586 (United Kingdom) (portal hypertension), even death, from liver disease within a Telephone: 0131 225 8586 (United Kingdom) Revised May 2009 Telephone: +44 131 225 8586 (International)PBC Foundation (UK) Ltd Revised May 2009 Telephone: +44 131 225 8586 (International) Website: www.pbcfoundation.org.uk2 York Place Website: www.pbcfoundation.org.uk Edinburgh EH1 3EP UK Tel: 0131 556 6811 Fax: 0131 556 8488 [email protected] www.pbcfoundation.org.uk The trigger could be an infection, or a toxin. It cannot be How is PBC diagnosed? spread by sexual intercourse. Generally the average age of [email protected] Where can I receive more information and support? The PBC Foundation (UK) Ltd is a Scottish Registered Charity, Number SC025619 presentation is around sixty, although it may be diagnosed www.pbcfoundation.org.uk The presence of liver disease is detected by liver function tests at any age in adults. Registered Company Number: 207493 on a single sample of blood. Also, people with PBC nearly The PBC Foundation is a nationwide charity which always have the specific antibody, AMA (antimitochondrial offers information and support for those who have How common is PBC? antibody) and finding this in the blood is useful in reaching a PBC and those close to them. diagnosis. As physical obstruction of the bile ducts are a Services Include: It is thought that PBC is most common in Northern Europe relatively common cause of jaundice, quite often the bile ducts � A nationwide helpline and North America, although it is found in people all over need to be x-rayed, or visualised by ultrasound, to confirm the world. In the regions where it is most common, it has there is no such blockage lower down the biliary tree. � Information packs for newly diagnosed patients been shown that as many as one woman in a thousand Diagnosis of PBC can be confirmed with a biopsy, which involves � A quarterly magazine for members and between the ages of thirty and eighty may have PBC taking a very small sample of cells from the liver with a needle. the medical profession, offering advice on quality although in many the illness may be silent (with no This also helps to determine the extent of any liver damage. of life issues symptoms) and will never become serious. � Information leaflets for GPs and consultants Is there a treatment for PBC? � Facilitating research projects Charity of the Year What are the symptoms of PBC? � Raising awareness of PBC amongst the general Although there is presently no cure for PBC, there have public and throughout the medical profession These vary between people. The severity of the symptoms been some breakthroughs in the treatment of the illness in often bears no correlation with the severity of liver damage. the last few years. � Advice on applying for DLA � Employment advice in relation to PBC as a Common symptoms in PBC include: Ursodeoxycholic acid is currently licensed for treatment of Nominating The PBC Foundation as your ‘Charity of the Year’ boosts PBC in the UK, and may help to slow down the disease. potential disability More information is available from either your family employee morale and encourages � Itching (”Pruritus“) doctor or hospital specialist. The PBC Foundation (UK) Ltd, 54 Queen Street, Lethargy and chronic fatigue A range of treatments is now available which have been Edinburgh, EH2 3NS, Scotland, UK � found to ease the symptoms. Drugs that dampen down the Telephone: 0131 225 8586 (United Kingdom) teamwork, all while raising money for a � Dry eyes and/or dry mouth body’s immune system and slow the progression of the Telephone: +44 131 225 8586 (International) Indigestion disease are under investigation. Facsimile: 0131 225 7579 worthwhile cause. � Like most chronic liver diseases, PBC can impair the www.pbcfoundation.org.uk Joint pain � Thecapacity of thmerchandisee liver to break down toxic drugs and pictured on this page is available for order direct from the PBC Foundation � Abdominal pain chemicals. These include prescribed and over the counter medicines, alcohol and even foods. Proper advice on a As your charity partner, we offer support Diarrhoea � healthy and balanced diet is, therefore important, as a Primary Biliary Cirrhosis � Pale stools and/or dark urine officegood diet is advis able foror ma inthroughtaining health for people our online shop which can be foundfor employee on our led websfundraisingite: www.pbcfoundation.org.ukactivities with liver diseases. Eating little and often is a good guideline for those who have PBC as this ensures that the Common signs of PBC include: Information on any other liver disease is available Patient Information Booklet bile acids always have something to work on and this helps that can be tailored to the size, location, (When it has become more advanced) from The British Liver Trust. to alleviate digestive problems. Please visit our onlineTelephone: 014 2shop5 463080 to view a selection of fashionable clothing and accessories. Jaundice As the liver may not be creating the correct balance of aims and needs of your company. � minerals and vitamins, the diet should be rich in calcium, Supplied as a service to medicine by: � Easy bruising vitamins and proteins. As in virtually everybody, foods Reddened palms containing unsaturated fats (such as oily fish) are probably Dr Falk Pharma UK Ltd, � preferable to fatty foods, which are high in saturated fats, Unit K, Bourne End Business Park, Cores End Road, Your employees can raise funds for The PBC Foundation through: Swelling of ankles The PBC Foundation (UK) Ltd � but a normal fat intake must be continued. If there is Bourne End, Buckinghamshire SL8 5AS 54 Queen Street, Edinburgh, EH2 3NS, Scotland, UK steatorrhoea (fatty stools), specific advice must be sought Telephone: 0131 225 8586 (United Kingdom) � Swelling of abdomen Revised May 2009 from the doctor. Telephone: +44 131 225 8586 (Internationa l) Marathons, runs or cycling events www.pbcfoundation.org.uk - packs of 10 -.indd 1 Notecard  PacksOutdoor challenges or18/03/2010 11:35something more adventurous Airmail Pack Office based events and competitions contains 5 cardsWe can provide you with:  T-shirts, collection cans, and other branded of each design fundraising materials only £2.75  PR and communications support to spread the Airmail - Pigeon Airmail - Balloon news about your charitable efforts  A wide range of exciting volunteering opportunities LegalLegal & & General General holding holding a a staff staff fundraising fundraising and and awareness awareness day day For more information, contact our Grants Officer on 0131 556 6811 or Tulip Pack contains 5 cards of each design [email protected] CorporateCorporate Partnership Partnership Opportunities Opportuni�es only £2.75 Tulip - Red Tulip - Yellow The PBC Foundation relies on donations, PBC FoundationFounda�on (UK) Ltd, 2 York Place, Edinburgh, EH1 3EP grants and sponsorship to fund its free Poppy Pack contains 5 cards of each design Tel. 0131 556 6811 services. With your help, we can ensure that info@pbcfounda�[email protected] only all PBC sufferers have access to the www.pbcfounda�on.org.ukwww.pbcfoundation.org.uk £2.75 information and support Sco�shScottish Registered Charity Number: SC025619 they need. Re-usable Poppy - Trio Poppy - Single Canvas Bag Do you know an £4.50 organisation that Trolley gives to charity? Badges Token £1.00 £1.00 If so, contact our Pens Fundraising and £1.00 Available in Purple and Red Grants Manager Rob Waugh on Collecting Boxes 0131 556 6811 Our collection boxes are proving to be very to obtain a copy of our popular and are a fantastic way of raising money for the PBC Foundation! If you would ‘Corporate Partnerships like one, please contact the office. Brochure’

Spring 2015 19 The Bear Facts FOOD FOR THOUGHT The PBC Foundation (UK) Ltd FOOD FOR THOUGHT Helpline Number FOOD FOR THOUGHT 0131 556 6811

Another selection of tasty dishes to tantalise your taste buds Quick Kedgeree Caribbean Beef Stew in slow cooker (Serves 4) (Serves 4) Ingredients Cooking oil spray Ingredients 1 chopped red onion 450g/1lb braising steak 1 tsp ground coriander 450g/1lb diced pumpkin or other squash 1 tsp curry powder 1 onion chopped 1 crushed garlic clove 1 red pepper deseeded and chopped 2 cardamom pods 2 garlic cloves finely chopped 2/3cm fresh root ginger peeled and grated 2.5cm piece fresh ginger finely chopped Pinch of chilli flakes 1 tbsp sweet or hot paprika Salt and pepper 225ml/8fl oz beef stock 200g brown rice rinsed and drained 400g/14oz can pigeon peas drained and rinsed 500ml water 400g/14oz canned chopped tomatoes 100g frozen peas 400g/14oz canned black eyed beans drained and rinsed 200g smoked mackerel fillets Salt and pepper 2 hard-boiled eggs quartered Chives and lemon wedges to serve Method • Trim off any visible fat from the steak, then dice the meat. Method • Heat a large, heavy-based saucepan without adding any • Heat a large pan, spray with cooking oil and gently fry the extra fat. onion for 3 minutes. • Add the meat and cook stirring constantly for a few minutes • Add spices, cardamom, ginger, chilli flakes, salt and pepper until golden all over. and fry for a further two minutes. Stir in the rice. • Stir in the pumpkin, onion and red pepper and cook for 1 • Cover with water and bring to the boil. minute, then add garlic, ginger, paprika, stock and tomatoes • Cook on a low heat until rice is almost cooked and the water and bring to the boil. has been absorbed. Add the peas and cook through for a • Transfer the mixture to a slow cooker and cook low for seven few minutes. hours. • Remove from the heat and carefully add the flaked mackerel • Add the pigeon peas then the black eyed beans to the stew and quartered eggs. Check seasoning and garnish with and season to taste with salt and pepper. chives and lemon wedges. • Re-cover and cook on high for 30 minutes then serve.

The stew works well with lamb instead of beef - use neck of lamb and replace beef stock with lamb stock. Quick Fish with Chilli Dressing (Serves 2) Ingredients 2 fresh white fish fillets of choice (haddock, cod, plaice, etc.) approx. 200g per fillet. Salt and pepper.

For the dressing Method 1 red chilli finely chopped and deseeded (or a few chilli flakes) • Mix dressing in a bowl. Pinch of castor sugar • Steam, microwave or bake seasoned fish fillets until 2 tbsp Thai fish sauce cooked through. 2 tbsp lemon juice • Spoon dressing over fish. 2 tbsp chopped parsley • Serve with a green salad or steamed broccoli and Snipped chives boiled potatoes.

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The Bear Facts 20 Spring 2015