Hi,
In my research I have found very little information that is specifically to do with small duct Psc. For any small duct psc’ers out there what symptoms first prompted you to go to seek medical help and how do you manage your current symptoms?
Rick,
You are right, there is not much information out there on small duct PSC. I did a little research myself and didn’t come up with anything of significance. It does appear that it moves much slower than regular PSC and has fewer side effects, etc. It seems it’s much more rare than large duct PSC. Hopefully some other on the forum will respond with further information. If you have a particular concern, etc. feel free to post and someone should give you a response.
Mark
PSC 2011 / Liver Transplant 2015
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Hi Mark,
Yes, there are certainly far fewer symptoms with small duct Psc and severity seems far less too. For me the symptoms that I had were mainly nausea, night sweats, pain under my sternum but also across my chest, fatigue, weight loss as well as colitis symptoms that all appeared at the same time, so quite a lot to deal with. Literally the few weeks before all this I felt very healthy. One question I have is if someone has classic PSC, does this mean that they already have small duct Psc or is it possible to have classic Psc without small duct Psc? I’m wondering whether small duct’s patients have similar symptoms as a group in themselves, that large duct patients don’t have. I’m pleased to say that my large ducts are all fine according to my latest Mrcp, so I don’t get any itching or anything like that (which I am very grateful about!) but I am constantly aware of discomfort in my chest and a constant feeling of something ‘not quite right’. My doc regards these as non specific symptoms but they seem quite specific to small duct Psc to me. There is talk of people having Psc as sometimes being asymptomatic. Does this mean that as far as they are concerned they don’t feel anything wrong at all, even slightly?
Rick,
With classic PSC you start out with a perfectly healthy liver but the bile ducts are getting diseased. As the PSC progresses and the larger left and right hepatic duct as well as the common bile duct become more diseased, the strictures of the ducts gets higher and higher up in the biliary tree until it is in the small ducts of the liver which then over time destroys the liver. So you start out with a perfectly healthy liver, but over time the lack of bile flow due to the ducts being blocked starts to kill liver cells until one day the liver is dead. Now this moves faster in some patients than others. For me it was rather quickly as well as Jeff, the other moderator in the forum. But we have other patients who have had PSC for 15-20 years and still no transplant, so we all move a different paces.
Now one thing I am not for sure about is whether or not small duct psc eventually moves on down to the larger ducts, kind of reverse from what it does to classic PSC. That’s a question for your hepatologist.
Hope this helps. Sometime I’ll show you what my liver looked like when they took it out during my transplant. It’s something what this disease does to that poor liver. Take care.
Mark
Hi Rick,
sorry to hear about your small-duct psc diagnose!
When I first got diagnosed I assumed I had small duct PSC because I didn’t have any or very few strictures/scarring in my common bile duct. Sadly I do have a lot of inflammation and scarring in my right hepatic duct so the hepatologist told me that I do have ‘normal’ PSC. Otherwise I wouldn’t be noticable on my MRCP.
I’ve looked at the internet for some time regarding small duct PSC. Just like Mark said, small-duct psc progresses much slower and with less complications, so that is at least a little bit of good news!
But I think it still depends on the individual. I have read that some people ‘start’ with small-duct psc and over time they develop large-duct psc as well. And apparently a lot of people with small-duct psc also have crohn’s disease, as if those two are connected somehow…
To answer your question about the classic psc (as far as I know from reading about it); if you have classic psc, it does mean you also have small-duct psc. There is however also a small group of people who only have large-duct psc!
I’ve heard somewhere that the symptoms and problems you have regarding psc are often the result of where the strictures and inflammation are most present. So for me it’s a good thing that my common bile duct is rather good, resulting in probably less complications and a slower progress of the disease.
I think it’s understandable that you feel something isn’t ‘quite right’ because it simply isn’t! Your small ducts inside your liver are probably inflamed and continue to scar (if this is possible in such small ducts, I have no idea). Your body is constantly ‘fighting’ this disease, so even if the doctors disregard it, I do believe you can feel it.
My symptoms are mostly fatigue, pain in the liver area and recently I’ve experienced a lot of headaches and shortness of breath (but I don’t know if they are related to my psc or other health problems).
Good luck with everything and I hope you have a good weekend 
Kind regards,
Sophie
Thanks Sophie. Your symptoms are very similar to mine. I also don’t sleep well at all. I sleep all night but it is what i would call un-refreshing sleep. Most mornings I wake up exhausted. I put this totally down to the PSC as before I didn’t have this at all. The other symptom I have that has me slightly worried is that my urine is few shades too dark for my liking. As a result I drink lots of water. I also have tinnitus that only developed when I developed the other symptoms.
I’m still interested to know that when people describe having asymptomatic PSC, do they have no symptoms whatsoever??? i.e. nothing that even suggests slightly that there maybe something wrong, like dark urine or fatigue?? I know the main symptoms are itch, jaundice and fatigue but I wonder for people that have recently developed large duct PSC and perhaps only found out through a blood test (which maybe they were having for a different reason) do they have any symptoms at all in those early stages?
I am still on a mission to discover how symptoms of small duct psc differ from large duct as I can’t find any info online at all. As you say Mark, large duct usually follows the course of the large duct eventually closing off so the small ducts are eventually affected but it seems you can have this large duct form of psc for a long time before the small ducts are affected. In my case the small ducts are the only ducts affected (at least for the time being!!) and I’m feeling range of symptoms because of this, but I can’t get any helpful information from anywhere on this it seems. Also, I wonder if any drugs developed in the future for large duct psc will have any benefit for small duct patients??
Sorry, lots of questions but great to have this forum to be able put all my questions out there.
Rick,
Yes, you are very correct that you can have large duct PSC for a long time before the small ducts are affected. When I was diagnosed in 2011 I believe I had the disease for many years before that, the only indication as I reflect back was I always had a tanned appearance and I didn’t live in the sun all the time. When I was diagnosed and they performed all the tests including many MRCP’s, Endo Ultra sounds and ERCP’s, my hepatologist told me that I had a perfectly normal/healthy liver. That was in 2011. But over the course of the next 4 years that perfectly healthy liver quickly became very sick and diseased. When I was listed in January, 2015 with a MELD of 12, that quickly changed by April to 19 and then the middle of July to a MELD 36. Transplanted two days later. Things can change rapidly, it all depends on the speed of the progression of the PSC to the large ducts. In fact when they transplanted me to reduce the possibility of PSC recurrence they did not hook the new livers bile ducts up to my common bile duct, but did a Rou-n-Y procedure and connected me up directly to the colon. I certainly hope yours advances very slowly and you get many many years before any serious problems develop. Just to give you a little perspective I’ve included a photo of my diseased liver at my transplant. So this got this way from 2■■■■, just 4 years. Take care.
Mark
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Mark,
Thank you for sharing this. I had my car in the garage the other day for a new clutch. The mechanic told me I had very few miles left in the old clutch. I would say the same for your old liver!
Interestingly I also had the darkened skin also for a few months but this cleared up. The changes I made at that time were improving my diet and stopping drinking alcohol. I’m not sure if that helped with improving the darkened skin, but I haven’t had it since.
I am starting to think that small duct patients may have a wider range of symptoms at a much earlier time but also at much less severity. I would be interested to hear from other small duct psc’ers out there on their experience of symptoms.
My son has small duct PSC and we’re new to this and don’t quite know what’s what. My son keeps getting a low grade fever and is quite pale and I was wondering if this is a common symptom generally or whether it could be a feature of small duct. Our GI just shrugs her shoulders about this and all his swollen glands.
Melanie1,
Hi…The last sentence in you post set off a red flag in my mind. My first advice to you would be to find a hepatologist connected with a major hospital that does liver transplants. You don’t need a local GI taking care of your sons liver disease, espcially PSC. Go find a hepatologist yourself and self-refer him if you have to. Shrugging ones shoulders does not get it in my book. Liver disease is serious business and the sooner you get him under the proper care the better.
Regarding the low grade fever, it sounds like he might be having a small infection in his bile ducts. If the fever gets above 102 you need to take him to the ER. Hopefully it’s nothing serious, but please find him a transplant hepatologist soon. Let us know of any way we can help.
Mark
PSC 2011 / Liver Transplant 2015
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Thank you, following this up now.
Hello Melanie,
My son has just recently been diagnosed with Small Duct PSC. I AM SCARED! My son is only 15! How fast does this disease progresses? What can I expect?
I’m so sorry. Small duct PSC is less aggressive and is less likely to cause liver cancer. The progression is slower on average which gives you more time. I’m in the process of getting a second opinion from a hepatologist and will be asking about vancomycin as my gastroenterologist didn’t mention it. I’ve only just stopped spending every day crying and feeling overwhelming panic and I know you’re scared.
The advice here has been great, everyone says let your son live his life normally, let him have fun, get him to exercise. Do your research, look at vancomycin and keep up with the research. Also best to stay away from some of the internet stuff as it is very frightening. I’m really sorry though and I hope you can find your way through this.
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Lenora,
Hello and welcome. I know the news of this is frightening to you but
there is help and hope. The best decision you will make right now is to
get him under the care of a hepatologist associated with a transplant
hospital, not your local hometown GI. This is key to his long term care
needs. As Melanie stated already, small duct psc generally progresses
slower than large duct. Educate yourself all you can through this site,
make sure your family has a full understanding of the facts of what you are
facing, you will need their support in the years ahead. But for now, let
him live his life as normally as possible. Don’t mother him to death,
worrying about his every move and what he eats, just let him be the young
man he is and his body will tell him when he needs to rest or further
intervention is needed. This is very important. He may live many many
years before anything worse happens with the progression, so just take one
day at a time. We are here for you to help in any way we can.
Mark
PSC 2011 / Liver Transplant 2015
Mark Wilson
Hi Mark,
Thanks for your supportive words. I am trying not to baby him too much, but it’s difficult for a mother to see your son is in this situation, and not know what will happen next. I would do anything to give him my liver and let him live a long healthy life.
Leonora.
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Hi, I didn’t know the difference between small and large duct PSC until my visit with an actual transplant doctor this week and then reading this post yesterday. I have small duct PSC.
I’ve probably had this disease between 5 and 10 years, but was just diagnosed. I hope it stays slow moving.
The itching comes and goes for me. Sometimes I’m beyond tired, beyond exhausted, and into severe fatigue like I’ve read so many people here struggle with. It comes with muscle weakness, to the point I don’t have the strength to lift my arms or move my legs.
The transplant doctor and I talked about some more severe symptoms I had last year, and she’s pretty sure that was an infection that went untreated, but now I know what to look for and what to do. The infections are horrible. But for the most part day to day is okay, I’m just tired most of the time. I don’t get nausea associated with the disease, like I’ve read.
Thanks for this post!
Hi michrichwood,
We’re you diagnosed by biopsy? That’s how they diagnosed me in January 2016. I am sorry to hear the extent of your fatigue. I have to say I have never had it to the extent that I can’t lift my arms and legs but it still causes me big problems. Especially at work when I am chairing meetings. I honestly don’t know how I get through them. It’s like living in a permanent brain fog and I find it hard to think straight. I think I have probably had this disease for 3 or 4 years at least and the fatigue back then was the first sign anything was wrong. I have since found that caffeine makes my tiredness worse (strangely!) so I have given up coffee even though it’s one of the only proven things that has a protective effect on the liver. I also use mindfulness, yoga, counselling and diet which go some way to making me feel less fatigued.
What were the severe symptoms you refer to? Was it actual pain in the liver area?
I was dizzy and so weak. No appetite. Brain fog like you wouldn’t, but probably would, believe. I slept and slept. Every movement left me winded. I can’t remember all of it when I try to think back, I just remember it being so debilitating.
I had a biopsy first, then the MRI. My original doctor was reluctant to diagnose. So I went to a liver transplant hospital. I thought the new doc would want to do more tests, but in she walked and just gave the diagnosis. She said the biopsy showed the classic “onion skin” which I didn’t understand or remember hearing before.
it’s tthe periductal concentric fibrosis reference.
I need to be more consistent with meditation, like you! I do find that the whole foods, plant based diet I adopted last year has helped a lot. I’ve cut out all oils and try to only eat fats from nuts and seeds.
Good to meet you!
Nice to meet you too! I had the ‘onion skin’ too in my small ducts and my understanding that as there was no other explanation, a diagnosis of small duct psc was given. It was described by the doctor as a ‘diagnosis by exception’ I.e. everything else had been ruled out so only small duct PSC remained as a possibility.
What took me to hospital in the first place were symptoms similar to what you experienced. I had huge fatigue, rapid weight loss, night sweats and periodic pain under my sternum particularly after drinking alcohol. I haven’t had these symptoms since. I was never given an explanation for them apart from being given the SD PSC diagnosis. I do wonder if there was more going on for me at the time like a minor infection.
Wow. So, no other symptoms since? Did you have an MRI? Are your liver enzymes elevated? Do you still experience fatigue?