How were you diagnosed?

And what did your liver panel blood tests show? I've been diagnosed based on symptoms and enzyme count....have itching, fatigue, some pain, this and that.

I thought maybe I had PBC. What about you?

And I didn't think there was anything worse than PBC....I hear now that this is basically even less treatable and that by 43 (i'm 33 now) i'll be dead.

Unless some medical breakthrough happens. Not holding my breath. What is going on?

How many people get this? I just can't find anything on this regarding liver disease.

John

John,
My son was diagnosed 8 months ago @ the age of 16. He had weight loss, fatigue, itching, vomiting. I took him in because he was vomiting every night & he had such a drastic weight loss & was always tired. Blood test indicated this what was possibly wrong. He had CT scans, EKG’s, XRays etc. The colonoscopy confirmed the UC & the liver biopsy confirmed the PSC. There is a lot of information if you google it. The Mayo Clinic has some good information. Everyone seems to progress differently. One thing you should not do is is give up hope. They are making strides with the bile ducts. Look @ how far we have come with treatments for cancer. One thing I learned on here is you can have a living donor for your liver. Talk to your GI Dr. about it. Journaling helps. This site helps. My son just had his entire colon removed last Tuesday & he will be back in school tomorrow. He refuses to let this change his life & embraces each day as a gift & does not take time for granted.
My thoughts are with you. Keep the faith.
AJ

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Hi John

I have found various stats on PSC from 1/100,000 to 5/M. It is an auto-immune disease and there are many theories to cause from genetics to infections. I think genetics is a fall back for the Docs these days and it means, ‘we don’t have a clue’.

I was diagnosed in 2009… At that point I was anaemic and they could not figure out why. After an ectoscope and liver biopsy, and tests to rule out hepC ( there was contaminated blood in Canada in the 80’s and I had a transfusion back then)… I was diagnosed with PSC. Yikes!!
The good news is that it can be stabilized for several years with different meds. And, if you need a transplant, PSC patients generally do better than others with liver disease.

My son was my donor and I had the transplant in 2012. It has been very difficult, emotionally, physically and financially… We are both fine and I am starting to weave back the threads of my life.
There are many twists and turns and everyone’s course is different.

Stay connected and get the info and support you need to make the best decisions for you and your circumstances.

John,
Do NOT think like that. PSC is only conclusively diagnosed via a liver biopsy. PSC is a crappy disease, but it is not a death sentence!
I was diagnosed in 2000 at age 39. Although I had symptoms for years, I just really got sick over the past year. This thing is different for everyone. But by the generosity of a family who loved their daughter, I received a liver in May. Things are going great and I love every day of life. You should do the same. Medicines, research, and transplantation are improving all the time. Learn all you can about this, speak to others and live your life as best you can. Live more, love more and don't let little things bother you. Lose the negative attitude, you can control that. Don't drink alcohol or do things harmful to your liver. Stay healthy as possible apart from your liver.

Be "A LIVER!" :-)

Mike from Kentucky.

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Hi John,

I was diagnosed 10 years ago asymptomatic through elevated liver panels.

I was also told that in ten years I was most likely going to reach an "endpoint". An endpoint being transplant or death. It is now ten years later and I am still happy with my own liver. I do itch sometimes, I have cholangitis attacks on average once or twice a year, and I have needed ERCPs five times or so. But I am still alive and kicking and in no way close to an endpoint as far as I know. I haven't started to think about transplant yet.

PSC is awfully different for everybody. I also have met people that are alive after 25 years with PSC, and I have met people that had three transplants within 10 years. There are only two things you can do. One is giving up before you try, and the other one is trying to live your life as well as possible, and dealing with the cards you were dealt one at a time. I chose to do the latter.

Regards,

Chaim Boermeester,

Israel

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Here is an article that gives an overview of the different liver conditions.

60-LindorManagementofPSCJul13.pdf (530 KB)

Donna, wow your daughter's condition is heartwrenching. Who is treating her?

Hi guys,

Thanks for telling your stories. I did check Mayo Clinic but I didn't think it had enough information. I've learned more from reading the posts on this forum.

I mentioned PBC because that's what I thought I had. I was researching my symptoms online. In fact, if you type in liver + itching you will find plenty of PBC information but little about PSC. My scan/tests indicate PSC but yes I am awaiting the biopsy.

Not too long ago, I was freaking out about Hep C until I found out that it's basically curable for most people.


Now, I find that I am in both denial and despair. I've been told to wait for the biopsy results, but I don't see how I can just go about my life as if this can't be my reality. The fact is, I have almost every reason to think it is and couldn't feel normal about it even if I wanted to.

Plans of living to old age, plans of finding a soul mate, plans of seeing others grow old--out the window. I tried a shrink but to no avail.

The gastro doctor/hepatologist isn't knowledable so I am looking for another one. He has not dealt with anyone who has had this and it shows.

Anyone else have massive elevations? If so, which enzymes were the first signs? My gastro doc said my bilirubin levels aren't too bad so I shouldn't jump the gun.


Ruby, I am a little confused about the prevalence of this. I don't know where you got your information, But I read that 1-5,000 people have PBC and about 1 in 15,000 have this. Wikipedia says "prevalence 0.22–8.5 per 100,000." Maybe I'm confused about incidence and prevalence,, but this doesn't seem rare to me.

AJ: I'm sorry to hear about your son but I am happy to hear that he is positive about this. I don't mean to be a downer here, but it's hard to be positive knowing that I am at the mercy of a donor. I do not want to die young, I fear death regardless, and though I envy people who believe in God, I do not share this faith. I don't want to get into a religious debate. I'm not an atheist or a theist. I've just always *felt* like we're alone, mainly because of how much suffering happens because of disease, natural disasters, and pointless accidents.

I'm also mostly alone. See, I am adopted. My biological mother died of cirrhosis supposedly brought on by alcohol and hep C (I do know that she drank, but I now wonder whether she actually had Hep C and not something similar to this).

My mother is elderly. My siblings are on average 20 years older than I am. None has had a cancer or liver disease or anything life threatening at a relatively young age. They also seem to share a matter-of-fact attitude that I do not possess--not so much about my situation since it's still a secret between my mother and me, but about how people deal with life. It's not just that I don't think I can connect to them about this.

I can't turn to this large blood-related family for a liver donation anyway. I only learned that one can actually get a transplant from a living donor. I didn't think this was even possible.

I would be curious to hear from others who have gotten onto the organ transplant waiting list and what that is like.

To Ruby and others: I am also anemic. Do you know if that was the cause of your fatigue? I also had a Vit D deficiency and thought maybe that was the culprit. The doctor said he doesn't know why this would cause fatigue and that usually fatigue is caused by anemia or something else. But it sounded like he was guessing.

Also, what meds, because I read somewhere here that Urso (which is what the doctor mentioned) actually makes things worse and it not designed for PSC sufferers?

Chaim and OTHERS: What elevated panels did you have? And did you get a biopsy too? You Chaim look like a young guy. How have you dealt with this emotionally? For me, it's hard with the symptoms. Based on what I've read, asymptomatic people seem to fare better. I like your attitude but I presently do not share it. How does one write up a 15-year plan with this hanging over one's shoulders?

"If you get to the point where you require a transplant it's critical to get it done before you get too sick."

I am hoping that when the time comes, there will be someone there smiling, eager to give me some of his or her liver because they 1) care 2) are a compatible blood type. But I am a freelance reporter. I am really nothing special. And, I hate to say it, I think my ethnicity may be a problem. I'm Mexican-American. I've not dealt with much discrimination in my lifetime, at least outward discrimination, but I do think that this may pose a problem when the time comes. I hope I'm being too cynical.

I was diagnosed by my GI doctor doing a ERCP on me. My blood work out the roof which was done before I even had this ERCP done. I have found through Facebook and this website about everything. I learn from other people who has this awful disease on what they have experience. I was diagnosed on June 25, 2013 and then in September I did my liver biopsy found out I am in the middle of stage two and three. I weighted before we did this ERCP 160 and now I weight 130 and I can't eat or drink without throwing it up. I don't insurance so UNC Chapel won't see without insurance, so I am having the liver transplant doctor come to Wake Forest Baptist Hospital to come to see me at the clinic their. John hang in their I have learned everyone don't have the same symptoms, some may not have issues some do more than others. I have learned to take a deep breath and take it one day at a time. I have found to stay focus on friends and family who make you happy and loving, supportive when you do have your ups and downs. When you are up high on the mountain thank friends or family and GOD for giving you another blessing day to be happy because, when I am down on the mountain I do have a hard time understand why me but, my family has to tell me their is a reason for everything. Hang in their John. GOGGLE liver disease and use PSC SEEKING Cure it is awesome for use for reference on this awful disease. You take care of your self.

Look into oral vancomycin. The clinical studies have been promising. You just have to find a doc willing to administer. Dr. Bowlus in Northern Cal is one who will treat adults. Dr. Cox at Stanford treats pediatric patients.

HI, I had Hepatitis C for 20 years, contracted from a 1992 blood transfusion. It was diagnosed in 2008; and cured in 2012 by using vitamins, herbs and amino acids - no prescriptions . Now I have developed symptoms that seem like PBC or PCS. It seems worse than the Hep C.


I am so sorry for your son. My 16 year old daughter was advised to have her colon removed 2 years ago. She went on oral vancomycin a year ago and now has a normal colon and liver. That may have not been an option for him but at least now for his liver you might want to consider that treatment.

Re a living donor, keep in mind that there is a higher incidence of recurrence when the donor is a family member.
■■■■■■■■■■■■■■■■■■■■■■■■■■ said:

John,
My son was diagnosed 8 months ago @ the age of 16. He had weight loss, fatigue, itching, vomiting. I took him in because he was vomiting every night & he had such a drastic weight loss & was always tired. Blood test indicated this what was possibly wrong. He had CT scans, EKG's, XRays etc. The colonoscopy confirmed the UC & the liver biopsy confirmed the PSC. There is a lot of information if you google it. The Mayo Clinic has some good information. Everyone seems to progress differently. One thing you should not do is is give up hope. They are making strides with the bile ducts. Look @ how far we have come with treatments for cancer. One thing I learned on here is you can have a living donor for your liver. Talk to your GI Dr. about it. Journaling helps. This site helps. My son just had his entire colon removed last Tuesday & he will be back in school tomorrow. He refuses to let this change his life & embraces each day as a gift & does not take time for granted.
My thoughts are with you. Keep the faith.
AJ

AJ, for your son to come through major abdominal surgery that quick is wonderful. My colectomy kicked my rear, and the recovery was rough. He also has a great attitude, for a lot of what we go through is mental and trying to remember that the obective is to drain the swamp, but the daily alligators are a real %^$%^@# at the time.

Jeff

■■■■■■■■■■■■■■■■■■■■■■■■■■ said:

John,
My son was diagnosed 8 months ago @ the age of 16. He had weight loss, fatigue, itching, vomiting. I took him in because he was vomiting every night & he had such a drastic weight loss & was always tired. Blood test indicated this what was possibly wrong. He had CT scans, EKG's, XRays etc. The colonoscopy confirmed the UC & the liver biopsy confirmed the PSC. There is a lot of information if you google it. The Mayo Clinic has some good information. Everyone seems to progress differently. One thing you should not do is is give up hope. They are making strides with the bile ducts. Look @ how far we have come with treatments for cancer. One thing I learned on here is you can have a living donor for your liver. Talk to your GI Dr. about it. Journaling helps. This site helps. My son just had his entire colon removed last Tuesday & he will be back in school tomorrow. He refuses to let this change his life & embraces each day as a gift & does not take time for granted.
My thoughts are with you. Keep the faith.
AJ

Thanks for the idea. I will ask for it.

Cactusgirl said:

Look into oral vancomycin. The clinical studies have been promising. You just have to find a doc willing to administer. Dr. Bowlus in Northern Cal is one who will treat adults. Dr. Cox at Stanford treats pediatric patients.

I'm going to find a good psychologist and then return when I'm not so wound up. I am probably bringing too much negativity on the board.


John, We have all been there. Take a deep breath. First you need a definitive diagnosis. Then you can look at treatments. There is a lot of research underway on both of these conditions. There is a phase 3 trial in Europe on a new medication that looks very promising for PSC with nor-urso (I do not know its applicability to PBC). And there are some top hepatologists here in the US involved in clinical trials (for some of which you may be a candidate). So please do not despair. Educate yourself and become your own advocate. I have a whole library of research that I can provide you if you are interested.

I was thinking about that. Hopefully there will be some research on this within the coming years, and hopefully some more treatment options.

I'll let you know how the biopsy goes, though I read it was only for staging purposes.

With regard to how people get this -- there are several theories. There is probably some genetic predisposition that is triggered by some event such as long term antibiotic use or exposure. The theory I am focused on is the "leaky gut" theory. An imbalance in the gut microflora can lead to inflammation of the epithelial cells of the colon that will then allow bacteria to escape into the hepatic system. So my query is if this could be a cause, can rebalancing the gut microflora be part of a cure. Of course researchers must first determine if the microflora is imbalanced and then exactly which bacteria. There will hopefully be a clinical study on this soon.

Good evening John!!!! I was diagnosed when my bloodwork started coming back abnormal. My liver counts are frequently monitored because of the medicine I am on for MS. They did a specialized test, (forget what it is called) and the count was over 900. I was planning an overseas trip to Thailand so I demanded my PCP find out what was going on before I went to Thailand. I got in right away to see a specialst at Dartmouth. The Dr at first thought I had PBC also. I had a liver biopsy and more bloodwork. Results came back positive for PSC. we were all shocked was going to cancel my trip, (this diagnosis came lterrally the day before I was supposed to leave) my Dr encouraged me to go as going or not going was going to make a difference either way. I was set up for an MRI and the results of that also showed PSC. I am at an early stage now, only detected at this point because of random bloodwork. I did go to Thailand and had the most incredible time of my life. It has taught me to truly take life one day at a time.

John,
Are you going to the Mayo Clinic in Rochester, MN? I’m from Kentucky but after about 5 years my common bile duct became blocked and several attempts to get through by local doctors failed. They recommended Mayo Clinic. I went there and became a patient of Doctor Gores. He’s an excellent Hepatologist. He connected me up with Doctor Reynolds an Interventional Radiologist. Doctor Reynolds got through the blockage and ballooned the strictures. If you can connect with those guys, you’ll be treated well. But, Mayo is a 10 hour drive from Kentucky. The time came when my Bilirubin went from the normal (~1.4) to 7, I got yellow and jaundiced. Then it rocketed to 22. I was like a pumpkin in color and my MELD score (This determines whether you will be on the transplant list) went to 23. Mayo will typically list you when you hit a MELD score of 16. There’s a program on the Mayo website which you can access to plug in your blood chemistry numbers to get the MELD score. When my MELD hit 23 I decided to get listed locally (Kentucky). I can tell you that it’s harder to get a liver at Mayo Clinic than is was here in this region. The transplant regions differ greatly, and everyone should check to see roughly at what MELD scores folks are getting transplants. We actually had some people move from California to this region in order to get listed and transplanted quicker.
All of that being said … You are probably early on the stage of your disease. The thing you need to do is get informed, allow yourself to get bummed out but not depressed. You need to get to a point where you can live your life and not let this rule you. Statistically we have more to worry about Heart disease than this disease. Many more Americans pass away from Heart than any liver disease. Try to live life as positive as possible, and others have shared already that you could go decades before you need a liver. Time is on your side. Medicine is advancing in this area. Exercise, try to stay healthy in all parts of your body, DON’T DRINK ALCOHOL!

Be determined that this crappy disease will not beat you!

Some of the blood chemistry to check:
Bilirubin
Liver Enzymes: AST / ALT / Alkaline Phosphatase
Calcium
Sodium
Glucose
Magnesium (A big one - effects the heart)
Creatinine (tells you the health of your kidneys)
Vitamin D / D2 / D3
Hemoglobin
White cell count

Be “A LIVER” :slight_smile:

Mike from Kentucky