Diagnosis + follow up

Hi all , historically my first attack occurred approx 2002/4 from zero to flat out in agony following projectile vomit for 24 hrs then nothing followed by jaundice .Gp reconised gall bladder prob ultrasound revealed 'sediment' in gall bladder .For the next 5 yrs every 6 months average a similar attack followed during this time attended a consultant,had mri but nothing because I didnt present symptoms.Then following 12 months without an attack i was declared fit.

2013 the attacks re appeared major disappointment didnt do anything about the first couple i now knew how to cope,pain ,jaundice, wait then recovered . After another in quick succession went to new Gp who sent me for blood test, went back for result week later and shock horror she sat me down rang hosp arranged for me to be met and seen immediately very little info came my way.

In fact i was annoyed because no one told me why I was in need of a hosp bed.I spent the next 7 days being investigated by Mr Melia a very nice man .I was interrogated v v intensely , no one believed i didnt swap bodily fluids,use needles or indulge in bloodsports like boxing .Bloods every day apparently Alps were off the scale ?? They were convinced I had Hepatitis ... I still wasnt told why it would be bad for me to go home at one stage a staff nurse convinced my family I could die without treatment. For what no one said.

Finially I had mri with state of the art machine (i was told) then together with wife and mr melia he took me to room and on a large screen showed me my liver and the lack of biliary tracts on half of it,also showing me the duct from gall bladder and narrowing due to abnormal cellular growth.

Following this as i felt fine i was discharged alps had dropped to 130 ish ...I went back for blood test which was ok i guess 'never heard anything. Then a bad attack so took my emergency blood test form specially for this and gave bloods bang in the middle of a bad attack .... No contact nothing so foned mr melia secretary ''hello just had bloods I am having an attack'' she promised to draw attention to this ...Weeks went by and nothing .....so I asked for appointment ok 5 months time ....unable due to circumstance to make tht appointment got a new one for 6 months time .....hmmmm do i feel let down ??? just let me think for a second.Btw i have asked to bring it fwd and was told out of the question... Any comments on the NHS ???

I've just seen this discussion Pendragon, I'm sorry no one has replied sooner.

You seem to have had a really tough time dealing with your condition. I'm trying to establish at what point the docs diagnosed you with PSC? Is the abnormal cell growth as a result of PSC?

My personal experience of the NHS has been quite the opposite if I am honest. Yes, there has been the odd doc that hasn't taken me seriously along the way but have generally had to eat humble pie upon reviewing results but by and large I would say I have been pretty lucky.

I fail to understand why your case isn't being given the due consideration it deserves, especially when presented with the history you describe. I would strongly recommend getting a referral to another hospital or doctor, ideally one that has experience of dealing with liver conditions.

If you have concerns with respect to your treatment and the NHS management of it then I would recommend that you contact PALS, (patient advisory and liaison service). You can generally find officers based at your local hospital who will be able to provide you with confidential advise you on how best to proceed. See the link below for more information.

http://www.nhs.uk/chq/Pages/1082.aspx?CategoryID=68

I have found that the average physician knows nothing about this condition, and consequently DOES NOT OR CAN NOT give an opinion on conditions involving PSC. When you consider how rare this disease is (1 in 10,000 people), you must also consider that it is most likely that none of your physicians have ever encountered someone with this condition. It was not a priority of their training as little is know about it and every PSC patient encounters different symptoms from the disease. This makes it even more difficult to diagnose by the average physician. If they have ever even heard of the disease their education on it was extremely limited. Each of us that suffer from this disease have found our own way forward with it. The one out of many physicians that I found that could diagnose me I found through a new-to-me doctor that knew of a doctor at Johns Hopkins...I traveled 2000 miles to meet with him for that diagnosis. He alerted me to the need to get to a university hospital where they do a lot of training and surgery in the hopes that I could find someone reasonably close who understood PSC. I went to the internet and searched under "liver" and found where they did liver transplants, thinking that they would most likely be in the know. I made an appointment in a Hepatology Clinic at the university hospital and I immediately found a person that understood and treated PSC. This clinic also commonly does MRCPs and ERCPs every day....am now scheduled for another ERCP. I went into this clinic in tears and found a physician that understood and is willing to take a call from me anytime and assures me I will get a call back as soon as she can get free enough to make a call. After reading some of the horror stories written into on this website, I feel grateful that I survived my prior ERCP procedures with GI doctors that knew nothing about PCS....at least I survived long enough to find a group that has experience in treating PSC. I will also participate in a research project on PSC. I encourage you to not let time lapse in researching and finding a knowledgeable physician who can work with you. Don't wait, suffer, and worry for a long period of time before you begin this search.

This, dear Pendragon, is an absolutely horrible story. The bad thing is: you're not alone; the good thing is: here in this group there exists a lot of experience to help you.

It has been said a number of times in other discussions, that we have to be our own experts. My doctors admit, that I'm their only patient with small duct PSC, that they know little about the disease and that I know by now much more about it than they will ever learn.

My story lasts much longer than yours (about 50 Years), but - thank god - it's not as bad as yours. I had attacks / flare-ups / severe problems in the upper and lower abdomen about every 10 years, first time at age 14. And doctors never found anything, symptoms disappeared within two or three weeks. In 2011 (attack no.4 or 5) the gastroenterologist at the hospital was a diagnostic-bloodhound and didn’t give up. Blood values were off as usual, but atypical for any “normal” rare liver problem. ERCP and MRT showed nothing. But one out of a number of biopsies had remainders of a clogged small duct and signs of a “duct detour” as well as cholesterol deposits. These findings led to my diagnosis of small duct PSC.

Now the explanation of specialists is as follows (my words, no medical slang): due to a genetic problem cholesterol slowly clogs small bile ducts. Too much bile remaining in a liver area makes the liver “throw up” – a new flare-up. The liver builds new small ducts as detour and all goes back to normal. Then the show repeats … 40 years later (at attack 4 or 5) the liver found now more room for detours, thus symptoms became permanent, and the biopsy had the chance to hit a clogged area, so they found it. (part of this text is a copy I posted some time ago in another discussion)

All the best for you

Rippi