Who else has small duct PSC?

I sign in again to look for fellow sufferers. What is most annoying to me is the widespread ignorance of doctors. They can’t read more than I can, none has any experience with the disease – I’m their only patient with this deseases - and I have got MUCH more time for reading...

I once thought the liver clinic of a large university could give good advice. That was absolutely NOTHING! Young doctors with no special knowledge, though very eager to learn, acted as a recruiting office for studies. Never again !!!!!!!!!!!!!

From all my reading one thing showed up: there are probably two very different types of small duct PSC. The first type has inflamed small bile ducts - possibly autoimmune reaction – which grow slowly downward until it ends up at a perfectly ordinary PSC. The others type has the small bile ducts clogged (cholesterol mud), so that the bile cannot flow, thereby causing inflammation. Type 2 seems to be my case,. Its progression is much slower, it is even less detectable, and there are probably even less options on what to do about it.

Anyway, the lack of bile drainage has not only messed up my liver but also drove my whole digestive system crazy. I must be extremely careful what I eat. Sure, fat, flatulence provoking or raw food is completely out. But pastries, factory-made cake from the supermarket, frozen pizza and some more things seem to disrupt my digestion as well. It looks to me that anything added to food for preserving it or turning it more "industry compatible", produces riots in my belly. I feel best when I eat nothing. But that is not a long-term solution ...

Has anyone else such problems? Who has found a way to deal with it or come around it?

Greetings to all

Rippi

I think detmsh nailed it - with a rare disease you need to be the expert. The trick is to find a doctor who will actually listen and not be afraid to act on your suggestions.

I struggled with fats as well and couldn't tolerate more than 20-30 grams a day. The problem is that this is very unhealthy. If you are stuck on a low-fat diet, try to prioritize the fats you consume (ex. you'll pull a lot more nutrition out of meat compared to cake). Remember that small and medium chain fats are free--they don't require bile to digest and don't cause the liver to make it in the first place. The easiest way to take advantage of this is through MCT supplements. Make sure to keep tabs on your fat soluble vitamins through your blood work. If you need to supplement any of these vitamins, do so at the same time as your medium chain fats to better ensure they get absorbed.

PSC is so complicated. Now I hear of two different small duct PSC. I sometimes don’t know if I’m coming or going. Certainly they tell you what you have and then you are left alone to find out about the disease. My PCP admitted I was her first patient and my G I tells me my fatigue and nausea aren’t symptoms of the disease. I don’t agree with him and told him so. I have an ileostomy total proctolectomy and i notice another symptom is the stool is like oil and water.I notice my urine changes color. I am swollen in the right side but told that is my ribs or muscle…I don’t think so. I had a vitamin D deficiency. No one told me this was connected. I found out the vitamins myself. I am self educating myself through this wonderful group. I’m thankful I found them. It has been 38 years since I had my ileostomy surgery from ulcerative colitis!!! My PSC was just diagnosed two years ago through elevated enzymes. Does anyone know or would you make a guestament. That my PSC is slow progressing and has probably been progressing for a long time. Sign me. In the dark…

Hi Rippi, Can you share how you determined that yours is the second type of small duct PSC? My son has small duct and I have never heard about 2 types. Do you have research articles that you can refer me to? My sons small duct is more severe than they expect it to be. He also has UC.



Jean said:

Hi Rippi, Can you share how you determined that yours is the second type of small duct PSC? My son has small duct and I have never heard about 2 types. Do you have research articles that you can refer me to? My sons small duct is more severe than they expect it to be. He also has UC.

Hello Jean,

just a very quick answer - more to come later:

http://www.ncbi.nlm.nih.gov/pubmed/24517468 is one article, there's another one I couldn't find in the moment.

I assume - so does my doctor - that I was born with the problem, it progresses very slowly, it took 50 years to produce constant symptoms.

regards

rippi



rippi said:



Jean said:

Hi Rippi, Can you share how you determined that yours is the second type of small duct PSC? My son has small duct and I have never heard about 2 types. Do you have research articles that you can refer me to? My sons small duct is more severe than they expect it to be. He also has UC.

Hello Jean,

just a very quick answer - more to come later:

http://www.ncbi.nlm.nih.gov/pubmed/24517468 is one article, there's another one I couldn't find in the moment.

I assume - so does my doctor - that I was born with the problem, it progresses very slowly, it took 50 years to produce constant symptoms.

regards

rippi

Good morning Jean,

Sad to hear that your 10 year old son already suffers from this disease. But it started early in my life too.

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.

Here comes the second article I mentioned : http://www.gastrojournal.org/article/S0016-5085(08)00116-9/pdf

This one is not as explicit as the first one, but if you look at the presented number-work and read between the lines, they also suggest too different types of small duct PSC, one being an early form of PSC and the other being something else with supposed genetic background.

Lots of reading for you! Good luck to you and my best wishes for your son

Rippi

hi everybody,

I must go back to the hospital, removal of my gall bladder. I apologize beforehand for not responding in time, next week I will be offline.

Regards

rippi

Hi, I 'm back.

A few days in the hospital, one gallbladder less, and against all expectation my digestion seems to have fewer problems since then. I wonder if my gallbladder (with stones and full of polyps) had signaled to my liver " I 'm full and do not need any more bile ", and now without the gallbladder, the liver has started bile production again ???

Everyone in the hospital did an excellent job, everything worked out perfectly, but with my small duct PSC I was something like an “exotic beast”. The Prof said my liver isn’t looking too bad - a bit of a wrong color but otherwise nice and smooth and no scars or anything.

For several weeks now I’m taking PANGROL, a digestive enzyme, and abdominal pain has decreased markedly. My other "helper" is zeolite . Good friends (both medical doctors) say that zeolite is a proven agent. It does not heal, but it helps to support the entire gastrointestinal tract. Anyway, my liver values ​​have been much better since then. You must not expect miracles, but for me it has reduced several problem areas. Red veins on the cheeks (typical for liver problems) have almost disappeared, a thick varicose vein in the lower leg is much smaller now, and above all, I can walk longer again without being totally fatigued.

There is a lot of information about zeolite on the Internet. A manufacturer (trade name PANACEO) has published a book with much information and almost too much self-praise, which is worth reading nonetheless.

Greetings to everybody, and thanks to you Stephen for your good wishes

Rippi

Rippi, glad you are on the recovery mode.

Jeff

Hey Rippi... Good to hear that you are on the road to recovery and that your liver values have improved :-) Do keep us posted on how you get on. Best wishes, Priya