Primary Sclerosing Cholangitis (A liver disease).

[XtrPickels]

When I was hired (Nov '08) at the hospital I currently work at there were routine blood tests done. Randomly, my liver enzymes were significantly elevated. Not much attention was paid because I didn't have a primary care in this area. When I chose a PCP, we re-investigated. 10 months later we might have a reason:

Primary Sclerosing Cholangitis

Seems to be an auto-immune disease that attacks the bile ducts. Eventually leads to liver failure, jaundice and or liver cancer. There is no cure or method to slow the progression. When it gets bad, you get in line for a new liver. "They" say that typically occurs 10-15 years after diagnosis. I'm 28.

I'm not sure how I feel about this. I guess I'm not sure how quickly things will progress. I can't predict where I'll be in 10-15 years. I'm most happy to know what is going on. Literally the only thing I can do is to keep living life. I don't know if this going to change my outlook on anything. At least I have a fun party topic. I'm in good company; Late Great Walter Payton, who died of bile duct cancer associated with PSC at 49 and Chris Ledoux, Cowboy and country artist extraordinaire.

I have one more blood test and an ERCP to confirm.

Anyone have any experience? Anyone know what I should expect?

[splat]

Pickles - find a really good homeopath. I would suggest you google a homeopath named David Russell in Arizona and talk to him. I've seen him bring people back from conditions every other doctor thought incurable and keep people with cancer alive decades beyond their projected expiration date.

[Tye 1on]

Well that's kinda a kick in the nuts, eh? Not sure it will help with this particular condition, but milk thistle root extract is gaining a pretty good rep for mitigating liver issues, has had some pretty amazing results with industrial accident type poisonings. Hopefully the last tests conflict with the current hypothesis and they tell you to just knock it back on the drinkin'....

[XtrPickels]

The biggest kick in the nuts is that didn't drink until I was 23. Even now I have maybe 1 a month (if that).

Thanks for the info guys.

[skatha]

Do not take milk thistle and do not waste your time with a homeopath. Get hooked up with a hepatologist, a liver specialist, ASAP. Chris Klug had PSC, maybe you should contact him thru his website http://chrisklug.com for a little support

[XtrPickels]

I'm currently seeing a gastroenterologist at least until he performs the ERCP and p-ANKA blood tests. I may switch to a hepatologist once those results are in.

Always learning and keeping an open mind though.

[skinnyskier]

Sorry to hear about it. Hang in there during the uncertain period between funny lab tests and a definitive diagnosis. I know how hard it can be to consider both the possibility that you have PSC, and the possibility that you don't-you never know until the workup is complete.

In terms of options, I agree with skatha-you'll want to get to a hepatobiliary center, usually a university hospita, if it really looks like PSC. Your best bet MIGHT be Dartmouth, which is closer than NYC or Boston. But, if you end up looking at Boston, I know somebody who is somebody in GI there

[PSCgirl]

xtr- I just wanted to let you of a few resources in case you haven't already found them.

Yahoo group for PSC patients- http://health.groups.yahoo.com/group/psc-support/

PSC Partners, amazing group and resource site! www.pscpartners.org

Facebook groups.- http://www.facebook.com/group.php?gi...7120173&ref=ts

http://www.facebook.com/group.php?gi...2998944&ref=ts

Re: Milk thistle. Evidence has shown benefit, but is not yet conclusive. My doctor says it's not proven, but not harmful and approves of me taking it.

It sounds like you have a great attitude! When I was diagnosed I was scared out of my mind for months. Our knowledge is expanding by leaps and bounds! Sorry you have been diagnosed with this, but I wanted to welcome you to these sites for community and information.

[PSCgirl]

Also, are you in Mass?? There is a conference in May in Conn. Outstanding weekend, lots of speakers and workshops. Loads of fun people too. Info is on the PSC Partners site.

[Roo]

Re: Milk thistle. Evidence has shown benefit, but is not yet conclusive.

Where is the evidence for this?

[PSCgirl]

http://www.umm.edu/altmed/articles/m...le-000266.html
"Several scientific studies suggest that substances in milk thistle (especially a flavonoid called silymarin) protect the liver from toxins, including certain drugs such as acetaminophen (Tylenol), which can cause liver damage in high doses. Silymarin has antioxidant and anti-inflammatory properties, and it may help the liver repair itself by growing new cells."
(additional resources linked at bottom of that page)

http://www.ahrq.gov/clinic/epcsums/milktsum.htm
"Evidence exists that milk thistle may be hepatoprotective through a number of mechanisms: antioxidant activity, toxin blockade at the membrane level, enhanced protein synthesis, antifibriotic activity, and possible anti-inflammatory or immunomodulating effects."

http://mayoclinic.com/health/silymar...CTION=evidence

Those are just three. Of course, there is more.

As I said, none of this is conclusive. There is no harm in taking milk thistle for liver disease and it may be beneficial.

[Tye 1on]

Sloan Kettering has similar findings here.

I agree with PSCgirl 110%. There are studies that show pretty strongly that it MAY help, and very solidly that it won't hurt. But i sure didn't mean to infer it as any kind of cure-all, i was just surprised it had any kind of documented benefit.

[haydukelives]

very sorry to hear this.

could you explain the rational for a test...ERCP....which does have risks....to confirm a diagnosis that has no treatment?

[PSCgirl]

This may not be directed at me, but I thought I would share what I know.

That depends on who you ask, lol. ERCPs are becoming increasingly controversial and as you say, do carry risk.

I believe ERCP is the gold standard for initial diagnosis. It is definitive, MRCP is not. Just in the year and a half since I have been diagnosed, the practice of using ERCP routinely has dropped among my PSC peers. PSC can mimic several other conditions, so certainty in diagnosis is important. Beyond that, I feel MRCP is sufficient for follow-up UNLESS cancer is suspected. At which time an ERCP with FISH (Fluorescent in situ hybridization) testing would be prudent. Brushings of the bile duct tissue are taken during FISH testing. Cholangiocarcinoma is the dreaded fear of PSC patients, it is deadly and not often cured, so to catch it early is essential. If caught early enough, Mayo has a protocol which has been very successful thus far. Along with lab markers, which can be broad and vague, the FISH test is vital.

Also worth mentioning, all heps and gastros are not alike in the ERCP department. Some are far more skilled than others and have much fewer instances of pancreatitis and other complications. You would want to ask your doctor how many he or she has performed, and how many of those patients had major side effects. I have had only one, it went very well. My labs are currently all normal, and I should not need another peek inside for several years unless things change for me. MRCP is increasingly gaining trust as a monitoring procedure.

I hope that helps, I am happy to answer what I can. Again, those other sites have lots of peeps who know more than I do.

[skinnyskier]

It looks like PSCgirl either happened upon our little corner of the internet, or someone pointed her in this direction, since these are her first posts on TGR. Regardless, thanks for posting the info and support!

[PSCgirl]

Indeed. Google alert. You're most welcome!

[Toadman]

I'm currently seeing a gastroenterologist at least until he performs the ERCP and p-ANKA blood tests. I may switch to a hepatologist once those results are in.

Always learning and keeping an open mind though.

PSC is what lead to liver cancer for Walter Payton. It progresses at different rates in people. As mentioned earlier, Chris Klug had it, got a liver transplant and won a bronze medal. He currently isn't taking any immuno suppressors either. He went to the Univeristy of Pittsburgh, where they have been doing research into weening transplant recepients off of the immuno drugs. (I met him a few years ago in Seattle where he participated in a run/walk with other transplants from the state of Washington) The good thing is that research into liver disease and transplant techniques are progressing.

I would suggest you give up alcohol, you will have to before you go on the transplant list. (Which I hope is a long way off for you.) They'll be able to track your bilirubin levels and determine how far along you are with the disease. Eat a healthy diet, try and stay away from processed foods. Drink lots of water. A good hep. and a talk with a nutritionist will go along way.

I sure hope they do come up with a cure for this disease as well as PBC, which is closely related to PSC. I have several friends who belong to a running group in Seattle (Team Transplant). PM me if you want additional info.

[XtrPickels]

Wow,
Surprised this thread kept going. I had to search for it to add in an update and there was new information. Most of the reason that I started it is for the same reason I love TGR, its a wealth of knowledge and hopefully down the road someone else can find the information.

My ERCP was yesterday and the procedure itself went well. It did confirm, however, that I do infact have PSC.

Going to try to address a few things:

Hayduke: My reason is pretty simple. I really like to know things. Its harder for me to know that something is wrong yet not know why or what to do about it. WHAT the diagnosis is, isn't as important as knowing the diagnosis. Additionally, if it wasn't PSC, we'd keep looking and perhaps find it to be something else that was treatable. Beyond that, new findings in medicine happen each day. There could be something for PSC just around the corner and knowing that you have it is the first step in attempting to fix it.

My master's is in Exercise Physiology, so I'm lucky to have a pretty good understanding of the body and health. I attempt to maintain a very healthy lifestyle through both diet and exercise.

I'm certainly not at the liver-transplant stage and this is still very early in the game. The next steps are investigating Colitis /Chrohns which are highly associated with PSC.

I'd like to thank everyone for their insight, thoughts and well wishes. Although someone may write suggestions off, I carefully think about each one and try to understand its merit.

PSCgirl- I hope you ski or at least can tolerate the rest of the shenanigans around this place. Its pretty special. Thanks for the information.

Do you have any experience with Actigall?

[PSCgirl]

I had a quite an impressive and long reply typed out, my computer apparently decided it was not just right. So...

Actigall is also known as Urso. I believe Actigall is a brand name. In any case, same medication. I take Urso, and have been given Actigall in the hospital as it is what they carry. It is an interesting medication, the story behind it anyway. Human bile is made up of three different types of bile. Two are thicker and more irritating than the third. Unfortunately, this thinner, gentler bile only makes up a small percentage of the combination. Urso, as you may know, means bear. Bears have bile that is 100% this thinner, gentler bile, and so we have found a way to replicate it. The goal of Urso is to increase the proportion of the less caustic bile. I hear different things from different people. We all react differently to meds, this is no different. For me, I have limited experience with it. I have been diagnosed for about a year and half. I did go without Urso for a few weeks at one time, and I did notice an increase in symptoms. A little background- my PSC was discovered when I was in the ER for what I thought was a Crohn's flare- fever, chills, malaise, aches, general "I'm gonna die". It was then my LFTs were discovered to be high,and the ERCP followed confirming PSC. Since that time, my labs have been all normal and I have not had another incidence of what I now know was cholangitis, not Crohn's flare. The caveat we have discovered with Urso is that while it does improve symptoms and lab values, it has been shown to not slow progression of the disease or prolong time to transplant. For some, it doesn't even improve symptoms, or it is not tolerated for other reasons so they discontinue taking it. There were also studies (Dr. Lindor at Mayo-Rochester) with high doses (12-20 mg/kg) which were halted when it was discovered high dose Urso can actually be harmful. I take a fairly low dose.

I am glad to hear you are in the early stages. I am as well, and with the rate at which things are progressing I am hopeful for a full future living with PSC and not suffering from it.

[XtrPickels]

Interesting.
I'd say that I am symptom free. I feel today as I've always felt. Perhaps my "normal", isn't. I suppose I'm fairly interested in seeing if the medication / other things / finding out if I have Crohn's changes how I feel... I'm not sure I can feel better, but I'm not going to complain if I do.

Thanks again for the info.

[PSCgirl]

I consider myself pretty much symptom free as well. I usually have a feeling in the RUQ sort of like a brick sitting there under the ribs, but it isn't painful. Occasionally, usually when I have eaten some junk or not had enough water, it aches. (Or the time I was off Urso) I usually drink tons of water and it goes away. I have no itching, jaundice, fatigue or loss of appetite, the hallmark symptoms.

[Toadman]

Interesting.
I'd say that I am symptom free. I feel today as I've always felt. Perhaps my "normal", isn't. I suppose I'm fairly interested in seeing if the medication / other things / finding out if I have Crohn's changes how I feel... I'm not sure I can feel better, but I'm not going to complain if I do.

Thanks again for the info.

As PSCgirls said, Urso only masks the symptoms and doesn't do much else. Dr. Kowdley here in Seattle at the UW Medical Center did a study on it.

For those that get towards end stage liver failure and put on Transplant list, there really isn't much that masks the symptoms. The itching, which can to a certain extent be addressed with steroidal anti-itch creams can help. Interestingly, Dr Kowdley would put liver patients on anti-cholestrol meds to help with some of the itching issues. Each person reacts differently to the meds. Different amounts affect people differently. There is no one pill that cures all. I'm fortunate to know many transplant recipients of all organ types. Most are doing quite well and living productive and healthy lives. But the meds are getting better, the understanding of transplants,and dealing with immuno suppressors has gotten better. And people who a decade a go would never have dreamed of getting off the meds are now living w/o the need for immuno suppresors.

[XtrPickels]

Just an update

Apparently PSC is highly associated with Ulcerative Colitis and Surprise Surprise I'm positive for that as well. The UC seems to be very mild, at least at this point in time.

I still feel fine so we'll see how everything goes.

[PSCgirl]

I'm sorry to hear that, but not surprised. Only 2% of people with IBD get PSC (aren't we so lucky?) but upwards of 70% of PSCers have Crohn's or Colitis, they are definitely related. Mostly men with Colitis. I am a woman with Crohn's, so I'm even luckier!

I am glad you are feeling well!

[XtrPickels]

I figure I should update this.

I've had an interesting health year.
Back in January I got hit with some pretty significant fatigue, itching and finger pain. It was one of those things where it crept up on me slowly. Eventually Dockaroo (who was living in VA at the time and saw me infrequently) pointed out that I was much more tired than I use to be. It took her noticing it for me to realize that I was.

I was at the point where I had to stop partway down ski runs. I had to pull over to sleep on the way home from skiing. I would itch my forearms insatiably during the day and my ankles til they bled at night. My fingers had turned into little sausages that had trouble bending.

Because of the finger pain, my primary care (who's office is next to mine) put me on a medrol dose pack (corticosteriod). Immediately, as in the very next day, all my symptoms were gone. Itching gone. Fingers normal, fatigue gone.

The PSC shouldnt have responded to steroids like that, so my Gastroenterologist and PC doc began suspecting the PSC was secondary to auto-immune hep.

I ended up on prednisone (20mg daily) and imuran ( an immuno-suppresent) in addition to the ursodiol. Through today, those symptoms have not returned.

It was also discovered in April that I was osteopenic. Unfortunately this was discovered due to me breaking my hip, requiring 3 screws and 3 months non-weight bearing (that thread is elsewhere).

At this point the hip is doing well. I've been riding my mountain bike and dirt jumper pretty hard, but have decided to not race cyclocross this year. (That shit is hard on your body).

After breaking my hip I was switched off prednisone and onto Entocort / budesonide, which is another streroid but with less systemic effects. I stopped that on September 1st. We'll be monitoring my blood results and LFT's.

My liver functions tests have actually returned to "normal". A large part of this is Ursodiol. My liver specialist said that it probably wasn't uber-usefull and that I could stop it if i wanted. I did and my LFT values jumped back up. However, the combination of the immuno-suppresant, steroid and urso have my numbers signficantly lower than when I was one the Urso alone.

In addition to the PSC, auto immune hepatitis (I have to point out its autoimmune and not viral), Colitis and Osteopenia it seems that I am rocking the early stages of rheumatoid arthritis, ankylosing spondylitis and sjogrens syndrome. I had felt "symptoms" but frankly thought they were just normal nuances. I suppose that at this point, I have little recolection of what's normal and assumed it was "normal" for your fingers and SI joints to ache a bit or for your eyes to be a little irritated... it's Colorado after all and the air is dry here...

I feel fortunate to have a laundry list of afflictions, yet they are currently doing little to slow me down. I also feel fortunate to have discovered these when I am relatively young and symptoms free. Perhaps I can get a jump on the situation, mitigating any future progression.

Additionally I am fortunate to be in health care myself. Because I deal with patients every day and have a greater than average understanding of the body it is A) significantly easier for me to cope with the situation and B) significantly easier for me to navigate the health care system. I can see how this would be a daunting task.

For now, all is well.
/blog