PSC patient or not. A 7 years question

Life with Primary Sclerosing Cholangitis
1

Hi everyone,

This is my first post on this excellent & helpful forum, even though I keep on reading it for years

I am from Greece ( so please accept my apologies for my poor English) and back in 2010 (28 years old at that time) I started a journey trying to identify my Liver problem since I had elevated AST, GGT and ALT (3-5 times the limits) .

After a trip to India, I felt fatigue and I had routine blood tests which showed the above values. I was admitted to hospital since my GI was afraid of some Hepatitis virus where I stayed for a full week so that all major diseases could be ruled out.

After a week I was sent home with “a unidentified Liver problem”, even though my Liver values lowered ( close to normal) without any treatment . After close monitoring (blood tests, MRCPs, Ultra Scans, Elastography etc) all tests were negative but those liver enzymes stabilised at 1.5 x times of the normal levels. ALP etc were all normal

The doctors indicated that I should have biannual tests ,a practice which I followed and my liver enzymes where 1.5x to normal with no major alterations.

In 2012 an hepatologist (one of the best in the country) told me that I suffer from PSC since all other diseases have been phased out and started prescribing Ursofalk (250mg pills , 4 per day)
My liver enzymes came to normal. All tests were still negative and the annual MRCP showed no signs of PSC or anything. I arranged appointments with the doctor every six months where he had specialised blood tests, MRCP, elastography and utralsound with a pattern (some every six months while some others every year)

However, last year I met another doctor, a Professor in Hepatology, at a social event who offered to check my condition since he suggested that PSC is rare, but you cannot identify it by simply eliminating other diseases.

Anyway, after having another series of tests the new doctor thinks that I am not a PSC patient because every test is negative and the MRCP doesn’t show abnormalities or even signs of them. I am seriously considering stoping my visits to both of them (the normal one and the new one) since I am puzzled and probably lost.

It has been 7 years of agony, testing, reading, contacting doctors, affecting my family (wife, daughter , parents etc) and I don’t really know what to do. I am considering visiting Mayo Clinic in the USA to get another- better educated opinion - a choice that can be really costly due to the fact that my Insurance doesn’t cover international treatments and by any means such a trip is very expensive. Therefore I need to plan ahead so I would deeply appreciate if anyone knows an approximate price for Mayo Clinic (on their site I don’t seem to find any price indication for PSC tests) .

of course any other advice would be more than welcome.

Thank you

Thanasis

2

Hi! Sorry to hear about all of your troubles. This sounds very close to my story. I have a question, did you have a liver biopsy
as well? I have been diagnosed with small
Duct PSC only from a liver biopsy since my MRCP, ERCP And fibroscan had all come back with normal results. The biopsy showed scarring in my small
Ducts only.
I am also on urso 250mg 3x a day. My enzymes are only normal on urso. Prior to taking the meds I was always about 3x the normal levels.

3

TNA,

Do you have IBD (ulcerative colitis, crohn’s)? SaraC is spot on about the liver biopsy. If numbers and symptoms point towards PSC but nothing shows up on imaging, it could be the small-duct variant. Small-duct PSC is diagnosed through biopsy. Also sometimes there can be PBC or AIH despite testing negative to antibodies and a biopsy is the best way to figure this out.

4

Thank you both for your replies

No I didn’t have a liver biopsy because I was advised not to by both the doctors. The both claimed that "the risk is not justified at this stage " and that there is a 2% probability of contamination of an external disease while the confidence of the biopsy delivering tangible results is limited to 70% based on the absence of all other symptoms.

@jtb . No I don’t have IBD or any other symptoms of related diseases. I was tested multiple times for both PBC and AIH through various tests.

Again, thank you both

5

Γεια σου Θανάση. Βλέπω πως είσαι από Ελλάδα. Το ίδιο κι εγώ απ’ ό,τι κατάλαβες. Μπορούμε λοιπόν να ανταλλάζουμε εμπειρίες. Από το 2014 παιδευόμαστε με τη διάγνωση της χολαγγείτιδας για την κόρη μου. Εχω διαβάσει τ’ άντερά μου. Θα σου πω τη γνώμη μου για την περίπτωσή σου, με κάθε επιφύλαξη. από τους βιοχημικούς δείκτες νομίζω πως η ανεβασμένη φωσφατάση είναι αυτή που παραπέμπει περισσότερο στην psc και η ggt. H Mrcp βάζει τη σφραγίδα με τα χαρακτηριστικά της ευρήματα. Είσαι πολύ τυχερός που δεν βρίσκουν εκεί την εικόνα της psc. Υπάρχει λοιπόν το ενδεχόμενο της small duct psc που είναι πιο αισιόδοξη ιστορία και επιβεβαιώνεται με βιοψία. Η κόρη μου έκανε δύο μέχρι τώρα. Απορώ γιατί δεν σου τη συστήνουν οι γιατροί. Υπάρχει βέβαια σε μεγάλο ποσοστό το ενδεχόμενο να μην τύχεις σε δείγμα ιστού με παθογνωμονικά χαρακτηριστικά γιατί η νόσος δεν είναι διάχυτη. Σε ποιους γιατρούς έχεις πάει? Ελπίζω τελικά να μην είναι psc. ;Έχετε αποκλείσει αυτοάνοση ηπατίτιδα? Φανταζομαι πως έκανες ανοσολογικό έλεγχο. Σου εύχομαι καλή τύχη

Hi Thanasis. I see you are from Greece. The same thing I did than you did. So we can exchange experiences. Since 2014 we have been studying the diagnosis of cholangitis for my daughter. I have read my arms. I will tell you my opinion about your case, with every reservation. By biochemical markers I think elevated phosphatase is the one that points more to psc and ggt. Mrcp puts the stamp with its characteristic findings. You are very lucky not to find the picture of psc there. So there is the possibility of a small duct psc that is more optimistic and confirmed by biopsy. My daughter has made two so far. I wonder why doctors do not recommend it. There is, of course, a large percentage of the possibility that you do not get on a tissue sample with pathognomonic characteristics because the disease is not diffuse. Which doctors did you go to? I hope it will not be psc. Have you blocked autoimmune hepatitis? I imagine you have immunological control. I wish you good luck (translation by moderator support)

6

Hello. Can you please translate into English. I don’t read Greek. Thanks.

Mark

7

Hi again,

@vango mentioned his daughter’s quest with PSC and that she had a liver biopsy twice.
He also suggests that psc is mostly identified by elevated ALP values and GGT while MRCP verifies the presence of the disease when there are tangible findings. Vango also mentions than the biopsy may not give definite results and he also suggested I was tested for AIH and that small duct PSC is also possible…

To answer the above,

I have been tested various times for AIH which was negative . Small duct PSC is also a possibility. I have never had any Bilirubin or ALP elevation (they are always normal).

(removed the names of the doctors)

8

Elevated GGT with normal ALP is interesting. Are you on any medications or do you drink alcohol?

Biopsies are a shot in the dark as to whether they will catch a relevant section of the liver, so you can get a lot of false negatives. That said, they are the only way to conclusively diagnose small-duct PSC, PBC, and AIH. Antibodies aren’t 100% reliable for ruling out PBC and AIH.

9

@jtb thanks for the answer.
I remember eating fish yes, but it wasn’t raw .

10

I freely admit I don’t know much about this, but if it were I, I would try to work on the fatigue with other things. For instance, not eating sugar, exercising, going to bed early, eating adequate protein, etc… I would stop pursuing a diagnosis for a while and see what happens. Even if you were perfectly diagnosed, I doubt it would change the course of your illness. So I would just wait and see if things get worse or better.

11

Thanks for the answer. Just to clarify it, I don’t feel tired or something now.
But I guess you are right about the fact that most probably it will not change the course ot it either way. It is however very stressful not to know whether it is PSC or something else which I can treat (somehow)

12

Tna,
There is one sure way that your doctors can identify if you have PSC or not. That is by doing an advanced endoscopic procedure called an ERCP. They will go down through your throat, into your stomach, and up into the common bile duct. They will then proceed up both your left and right hepatic ducts. With that examination they can conclusively determine if you have PSC. That is the most accurate method to make a definite diagnosis. Hope this helps.

Mark
PSC 2011 / Liver Transplant 2015

13

Thank you fcmmark. To Tna, I just want to offer a bit of my experience. I was diagnosed in 1998 with PSC. I’ve spent the time since worrying. I’m 50 now. I have actually done very well, though, and so I shouldn’t have stressed out all that time.

Even knowing all these years that I have PSC, I still wonder if I’ve been misdiagnosed. They have done ERCP’s and MRCP’s though, so it’s probably correct. The thing is, it seems there’s little-- other than ERCP’s and application of stents-- that they can do to the liver. Beyond that, it seems they can monitor me and catch cancer early if it appears. They take blood tests twice per year. They try to get me to submit to bone density scans, but I refuse now. They try to get yearly colonoscopies. I refuse them too. As for the medical people involved, I see them as helpful in an emergency, less helpful in everyday ways.

As I age, my friends get health problems, so I don’t feel so unusual. We’re all here for a good time, not a long time, even if we make it to a respectable 80 years of age. If you feel fine, I’d continue to monitor for any changes, but I wouldn’t go hog wild trying to get to the Mayo. If something eventful happens, that’s when you’ll want to put your pennies together to go there. Living in an indeterminate way is the human condition. We have to enjoy things to the maximum now.

People on this website will likely disagree with me, but we must also consider that stress burdens our bodies. Even reading this website too much can be a burden. If I lived in Greece, I’d feel so happy for the azure water. I’d go and eat on a beautiful outdoor veranda. I’d play soccer with my guy friends if I were a man, which I’m not. I’d just live for the moment and look forward to lunch and supper and not think about PSC which is an unpleasant thought. I wouldn’t drink alcohol, but have a good time. Yes, the Greek economy is horrible, but the Haitians have it worse, so I’d send money to them.

14

Lara,
From what you are stating, your doctors are doing what they are supposed to do. Keep in mind that stents should not be left in very long with PSC, as the PSC will stop up those stents and you will really start feeling pretty bad. My docs never put stents in me but each patient is different. There is no pain in a bone density scan. You just lie on a table, the scanner goes across your body and you are done. You don’t even have to undress. As far as the colonoscopies, you really need to have that done. They are trying to rule out UC as well as check for cancer. There’s just only so much that can be done with PSC. There’s no known cure for the disease other than transplant. When you do get to that point, I would suggest you make sure they do not hook your bile ducts back up to the common bile duct but to do the R&Y procedure routing them into your colon directly. This greatly reduces the chance of recurrent PSC. I do wish you well.

Mark

15

Colonoscopies can be helpful, but all in moderation. Thank you for the tip about the R&Y procedure. Your points about the stents do not apply to me at this time.

16

Hello Thanasis,
Since a Small Duct PSC is very hard to find, I would like to save you and others my odyssey, because the doctors are always faced with a riddle and often start all sorts of unnecessary things. This has hurt me more than helped.
My story:
• At the age of 13, it started with bellyache, I had no endurance, and got tired much faster than the others. I went to the hospital, they took the appendix out “for precaution” and found nothing.
• At 18, I was sent to the isolation station because of sudden high liver values. At that time, I had my first biopsy - across the abdomen and quite dangerous - without a result. The liver values returned to normal without any intervention.
• Liver values up and down again about every 5 years, and every time riddles for the doctors!
• 15 years ago again an attack, this time very bad with itching all over the body (bile was in the blood). The doctor sent blood to the lab and called me next morning, I had to go to the hospital immediately because of suspected acute liver failure, liver values partially 10 times above normal. Three weeks of comprehensive diagnostics with biopsies followed - no results, but admission to the University of Düsseldorf. There further diagnoses, much blood to many laboratories but again no results (at that time Small Duct PSC was still unknown).
• 8 years later, I had very high liver values again. On recommendation I went to a young doctor at a local hospital having a reputation of a “Diagnostic-Bloodhound”(never gives up). Again, a lot of blood sent to many laboratories and all imaging techniques used, in addition many biopsies in many places of the liver. The samples went to several laboratories and university hospitals in the Rhineland. One sample (only one!) showed the typical pattern for Small Duct PSC, namely, the remains of a cholesterol-clogged small bile duct. Thus (and only thereby!) the diagnosis of Small Duct PSC was secured!
A new flare up of my liver “announces” itself in such a way: Meat does not taste well, tastes almost disgustingly, and all food has a metal taste, like biting in a tin can. I have never liked alcohol and therefore I do not drink, a sip of wine makes me instantly slack. I do not take any painkillers and, if possible, no medications that processed by the liver. It is now known that liver, pancreas, and intestines are very closely intertwined. In addition to URSO, I use 3 gentle herbal remedies to help, if necessary sometimes an ASPIRIN (goes over the kidney), and thus my system works quite satisfactorily.
URSO is not able to cure, but it keeps the liver on “status quo” - no further decay. The new of Prof. Trauner is said to be able to heal, so are hopes at present. NOR-URSO was also tested by Prof. Strassburg at the University of Bonn, where we also tried to get help. The Phase II test is now successfully completed, and phase III is being planned.
I think if my Small Duct PSC had been recognized earlier, I would have spared much of my 50-year history of suffering, my liver were in better shape and I would have more quality of life. Of course, with Small Duct PSC you have to pay attention to a balanced diet and immediately omit what does not do well. For me, all substances (food additives), which make food longer lasting or “facilitate” industrial production, immediately cause extreme problems.
Wishing you all the best
Rippi