Saying hello and some questions

Keep in mind that some of us with PSC can have: splenomegaly, and even proto-hypertension/ascites without having actual cirrhosis. The histopathology of a liver biopsy will show portal inflammation/fibrosis in the pre-sinusoidal regions … unusual but very demonstrative … massive ascites and pitting edema. Severe hepatic osteodystrophy, osteoporosis, etc. Subtle stuff.

lol … portal-hypertension … spell checker is killing me.

Hi everyone,

Wanted to give an update how it’s going and also wondering how all of you have been!
@detmsh I don’t have an enlarged spleen but do have Hepatomegaly and fibrosis of the liver, thankfully still in the early stages of PSC and no cirrhosis.

I went to an specialist and he has confirmed the diagnoses PSC. I do however still only have clear beading, strictures in my intrahepatic ducts so I think that is ‘good’ news.
Since a few months I’m on ursol (I believe it’s called) and my liver enzymes dropped! Is this a good sign or does it not make a big difference? My own doctor didn’t want to give me this because he said that it didn’t help but the specialist did recommend it. And because in not too high doses it’s a pretty tame medicine I wanted to try it.

However, I don’t feel much better than before taking those. I’m still exhausted (partly because of my Crohn’s disease and some small inflammation in my small intestine) but I notice a big difference from before I had liver problems "and only Crohn’s"
My doctor said that my fatigue, headache, etc. Is something I have to deal with, basically saying that there is not much they can do and that the fatigue is part of my two chronic illnesses. And yes it’s true but I always want to know what I can do to make things better. I already eat healthy, never drink alcohol, drinks lots of water and when my body is able to I try to exercise.
My vitamin levels are low, especially vitamin D and B12 and things like creatine. So I will take some extra vitamins in the hope of getting a little bit of energy!

I’m curious for those who are also pretty exhausted, how do you deal with it? Anything that helps?
And how do you explain to people how you feel when they don’t see anything on the outside?

And I do have a very itchy skin but always thought it was because of eczema. But can you have itchy skin from psc without having an elevated bilirubine level?

One last thing, as I’ve mentioned before I’m on Vedoluzimab/Entyvio for my Crohn’s. Are they still in trial with this medicine for psc? Any results maybe?
I’ve heard psc for people will Crohn’s is most likely less severe, but there was only one research so I don’t know how reliable those results are. Anything personal experience about this?

Good luck everyone with your health and especially those who are in (near) end stage psc, awaiting a transplant!

Wish all of you the best and hope some of you can answer (yet again lol) my questions.

Thanks in advance and take care,
Sophie

Hi Sophie,
Glad to hear from you again. I’d be glad to answer some of your questions.

  • Ursodiol (or Urso for short) is a very important drug to help with your quality of life. It helps thin the bile so it can flow around the strictures in your bile ducts. Yes, some doctors don’t think it helps all that much, but believe me it does help. They are not the ones with PSC but I can tell you it does make a difference. I was on 1200 mg a day for 4 years prior to transplant and it was a big help. I did have to play around with the times of day I took it and finally found a combination of times that worked best for me. I found if I took it all at night that I ended up getting up and down constantly to the restroom through the night. You just have to work it out.
  • Regarding the fatigue, that comes with PSC unfortunately. Most days, I would come home from work and just collapse in my chair and sleep for a while before supper. You will find yourself needing little naps at times, just listen to your body, rest when you need to and on those days you feel great get out and enjoy life!
  • I would encourage you to try your best to get around 30 minutes of walking in a day or some other “core-strengthening” exercises in. The further along your PSC gets you will experience muscle wasting, weight loss, etc., so you need to keep up your strength. You will need all you can muster for your transplant when that comes. And remember, there is no cure for PSC so transplant does lie ahead sometime in your future. It’s ok, and when that day comes it will be a wonderful day!
  • For energy, I would encourage you to start drinking some of these shakes that are high in protein. You will find your appetite waning as you get sicker and you need to keep up with protein intake. Be mindful to consult with your transplant hepatologist about any vitamins you decide to take. You’ve got to be sure you are not taking anything that will harm your liver.
  • In dealing with explaining to folks how you feel when nothing appears obvious on the outside, my suggestion is to be very open and frank with your family and close friends, co-workers, etc. Explain to them what’s going on, set up an email chain to provide people close to you with regular updates on your health. Make sure they know that you are fighting a life-threatening illness and that you need their support, not their pity but their support. The more open you are about your disease you will find a well of folks that will be there for you. I do a lot of interaction with the business community where I live and work and folks I saw all the time all knew about my disease, were very supportive throughout the entire process.
  • Dealing with itching, you will find that those of us here in the group have found different remedies that worked best for us on individual basis many times. I tried every cream, lotion and potion out there over the counter, and none of them worked. The only thing that brought back sanity to me and took away the itching 98% of the time was a prescription based drug called “Rifampin”. I was on 300 mg twice a day and took it for 4 years. It was a miracle drug for me. Ask your hepatologist about possibly prescribing that.
  • One thing that was always a key to tell me that my bilirubin levels had elevated to a pretty high point and I needed another ERCP procedure was the itch level. When you are really itching your bilirubin is up and when it creeps up there in the 3-5.0 range you most likely have a blockage that needs clearing. You asked about itching and not having an elevated bilirubin level, well when this happens how long has it been since your last lab work? Because it could very well easily had started going back up and you just didn’t know it. That’s my best guess on that.

Hope this helps, take care!

Mark

Hi Mark,

once again thank you so much for your help! What you do for this site and the people on it is truly amazing! Very grateful!

Thank you for the tips. I will look into those things! I didn’t know about the vitamins. Some things, you don’t suspect, can be bad for your liver. And I think we all have to be extra careful to make it as easy as possible for the liver. I will consult with my doctor.
And yes it has been over 6 months since my last bilirubin test so I have no idea how quickly it can go up…

Enjoy your weekend!

Kind regards,
Sophie

Glad to be of help Sophie. Let us know how your lab work turns out. If your levels are quite high compared with last time, I’d be asking your doctor for labs at least every 3 months. You may have to have them more often that that should your condition worsen. There at the last before my transplant I was having labs every 2 months, then monthly then weekly then every day there at the very last. Just keep on living life to its fullest and when the bumps come deal with them, but never ever lose hope!

Mark

Hi Sofie’ … Hope you are doing well! Been a very long time since I’ve been on this site.

You have the advantage of having intrahepatic PSC only, if there is an advantage with PSC, so your liver is likely to be doing well, relative to others with very obstructed bile flow. Your IBD is the wild card of course, as you already know. The enlarged spleen is the easiest trigger point wrt knowing that there is advanced disease.

I’ve been thru the advanced stages of portal hypertension as well as the bone disease, etc. We PSC’er can still remain healthy, just takes a lot more effort of course. Fatigue is the one thing that can haunt some of us, roughly 10% or so, not much fun.

Take care, hope you remain well!

Detmsh…

Could you elaborate on ‘the advance stages of portal hypertension’ and your experiences.

Eric1 -

I’ve experienced massive ascites with multiple paracentesis procedures performed - diuretics controlling things. Lower GI bleeding and a brief bout of Hepatorenal Syndrome but these have all been stable for a while now.

Sophie - My LFT’s will quite often stabilize themselves without ever having used URSO. That is one of the main issues with it - would patients who show URSO benefits have simply recovered on their own, without the drug? Many PSC patient will show multiple flare-ups. I have had these up and down flares myself several times.

Thank you detmsh! That is a good question indeed. And yes I think you are right that the blood levels can vary from time to time, depending on how active the disease is!
I will have another blood check in a few weeks, so I hope it’s still relatively well! My ferritine levels have been very low, so I am getting iron supplements. I am not sure if it is because of Crohn’s or psc or maybe both.

Hope you are well!

Kind regards and best of luck for everyone!

Hi Sofie, I have small duct psc/chrons and have done for over 2 years, after getting the symptoms/diagnoses for both in the space of a few weeks. The fatigue was a real problem for me and was always accompanied by a spacey feeling and some dizziness. I found that the biggest thing that helped me was giving up caffeine. I feel quite ‘alone’ in this finding as having checked the internet I can’t find anyone else that has improvements in fatigue by doing this. For me, within 24 hours of having no coffee/tea, my fatigue lifted. I have done a few tests since and had the odd cup of coffee, and if I have 2 or 3 over the course of a day, the fatigue returns - so it’s no more caffeine for me!

Hi Rick,
That is wonderful news about the fatigue!! I am very delighted for you that such a simple thing as not drinking coffee/caffeine helped with your fatigue. I haven’t heard about this before as well but I assume people would like to try this if they also experience a lot of fatigue.
Sadly I never drink coffee! I only drink water and herbal tea. And only once in a blue moon I drink a coke. So I can’t try it myself…
I am going to the hospital to get help with my energy level and building my condition. So hopefully that will give some results!

I hope your caffeine-free ‘diet’ will keep you as energized as possible can for a very long time! And hope both Crohn and PSC remain in remission for an even longer period!

Good luck and take care,
Sophie

Hi Rick,

Good to hear you have managed to improve your fatigue issues. I’ve been battling with that myself for over 5 years now. I was crazy active before the fatigue hammer dropped. Funny that most hepatoligist recommend that we liver patients drink coffee. Seems nobody can help us directly, kinda figure things out for ourselves as we move along. We all seem to be different.

Hi Detmsh,

Yes, this strange disease effects everyone differently. I have a suspicion that the effect coffee has on me may be something to do with the fact I have small duct psc and not classic psc. The fatigue I experienced after drinking it was debilitating. I will ask my hepatologist about this on my next visit although I am not sure she will have any answers. There is so little information about small duct psc on the internet, I don’t think anyone really knows too much about how it effects people. I still drink the recommended 3 cups of coffee a day but it is decaffeinated. I read something somewhere about the fact that it doesn’t matter if it is caffeinated or not for it to have some positive effect on the liver. I hope you are able to find some ways to manage your fatigue. 5 years is certainly a long time to have lived with it.

I have hated coffee all my life and made the mistake of telling my wife about the study that showed coffee may help with liver disease. Just to stop the “encouragement” I had several sips in front of her. Still hated it.

Hi Sophie
I was diagnosed with PSC 26 years ago. I was deathly ill at the time. It had also destroyed my gallbladder which was removed at the time. Definitely confirmed PSC with ERCP at the time.
I was also later diagnosed with Chrohns. I am now 71 years old now and have no symptoms of either condition. Liver lab results are normal. I am still working as a Physical Therapist. Ran 3 miles twice this weekend (because it was too rainy for biking) but usually run one day and bike 15 miles on the other day. I have not had any medical treatment or transplant for this condition other than my own trial and errors. One of the key things that was very helpful was visceral mobilization which I had to do on myself because there was no one in my area of Montana that knew how to do it correctly. This is of course is not a proven therapy for PSC but it definitely helps me.
Because of my experience with PSC, the motto for my office is, “The difficult we do immediately, impossible takes a little longer.” Yes Sophie, anything is possible.
Lance
PSC diagnosed in 1999, No liver transplant, have not taken any meds for PSC

By the way, I do not drink alcohol, smoke or even drink coffee. Never have done any of those.

Hi Jake
OK I have tried lots of stuff, vitamins etc. Don’t know if they help but I take them anyway. Have not taken any medication because there was really nothing available in 1999 when I was diagnosed. One thing that I think helped was visceral mobilization. I had to do it on myself as there was no one in this area who knew how to do it correctly. I learned this as a physical therapist but this is not standard training for physical therapists, massage therapists or chiropractors. I live in Montana and there is only one other person in this region who knows how to do this even though it is quite simple. You find someone in your area who knows how to do this and give that a try even though it is not scientifically proven…yet.
Keys to recovery in my mind are simple: eat well, sleep well, distress your life, adequate exercise hopefully outdoors, love much, have faith, visceral mobilization. : )
Lance
PSC since 1999, no meds, no liver transplant