Recurring Episodes / Attacks for 1.5 Years - Recently Diagnosed as PSC

Hello everybody, new member here. My name is Textbook, I'm a 26-year old male from Michigan. In December 2012, I had stomach pains in my upper-right quadrant , near the lower ribcage. I immediately found it strange because when I thought about it, I couldn't ever remember having pain in that area of my body in my entire life. It first started out just like a normal stomach ache, just in a different spot. I did a search online ( I Google everything ) and everything I found said that it was my gallbladder that was in pain, and it was most likely caused by gall stones. I waited an hour to see if the pain would go away. It did not, it got worse. I waited another hour, it got worse again. Being stubborn and not wanting to go to the hospital, I waited one more hour, and by that time the pain was unbearable. I drove home and had my wife and 4-month old son take me to the hospital emergency room. Of course, as soon as we get our vehicle parked in the parking structure, the pain started to subside. Since we were already there, we thought it best to see what was wrong. Although the pain was gone, a nurse palpated different areas of my stomach and when she pressed on my gallbladder I could feel the tender pain. They performed x-rays, an ultrasound, and blood work. The ultrasound was negative for gall stones, although they said my liver enzyme levels were elevated. They said it could be acalculous cholecystitis, which is just gallbladder pain without the presence of stones. Since I was no longer in pain, they discharged me without a diagnosis and recommended I get a primary physician and request a HIDA scan.

Over the next year, I continued to have episodes / attacks every 2-3 months , usually following a large fatty meal. The pain was always in my upper right quadrant, in my gallbladder, and always severe. Some new symptoms showed up, like nausea and vomiting, dark amber urine, dark circles under my eyes, and after symptoms had subsided, itching all over my body that would last up to 5 days. I recently went through some of my email / IM history and I believe I have had 7 to 9 episodes / attacks since the first one in December 2012. In my stubbornness and stupidity, I neglected to pursue medical treatment for any of them and suffered through the episodes until they passed. In January 2014 my wife, son, and I moved across the state and the first week of the move I was vomiting into a McDonald's cup as I drove down the highway. I decided that with the move, I should get a primary doctor and look into my issues again. I found a doctor and he was not very good at all. I explained that I had been having these episodes over a year with each one having the same symptoms and everything I had researched pointed to some type of biliary obstruction or acalculous cholecystitis. The doctor dismissed my research and blamed my episodes on GERD (acid reflux), caused by drinking soda. I went home frustrated, saying "this is why I don't go to doctors." I ignored his diagnosis and never filled the Prevacid prescription. I cut down on the sodas but didn't eliminate them entirely, as I felt the episodes I had were too severe to be caused by acid reflux. I also started eating smaller meals, and generally eating less often. From January to April 2014, I had no episodes, until one night in April where I had a few too many slices of pizza. What followed was two episodes in three days, both severe enough to cause vomiting. Two hours into the second episode, I decided that it was time to go to the hospital again. It had been a year and a half, maybe there were stones now and they were just missed or too small to be seen the first time. Since we had moved, we went to a different hospital. I was admitted into the emergency room with severe gallbladder pain. The ER doctor immediately dismissed my claims that it was a gallbladder issue, asking me how I knew it was the gallbladder if the ultrasound from 12/12 showed no stones. I told him I had done so much research and all of the symptoms fit. He said "you do too much research" and said he thought it was irritable bowel. He walked out of my room and I was frustrated and still in severe pain. What happened next was very fortunate. I had brought my bucket from home and in that ER room I vomited my guts out into it. I was given Fentanyl for the pain. When the doc returned, he was more willing to believe my symptoms were serious. He ordered an x-ray, ultrasound, and blood work. While the x-ray was normal, the ultrasound showed something this time - a very large amount of biliary sludge in my gallbladder. Still no stones, but sludge. My liver enzyme levels were elevated. I was admitted overnight and placed into an observation room. The following day, they did a HIDA scan. I sat on the HIDA table for two hours and the radioactive tracer dye never moved out of my liver in that two hours. They said that was not normal. They said they would wheel me back to my room and then put me back on the table 2 hours later (4 hours after dye injection). When they put me back on the HIDA table, it looked identical. After 4 hours, none of the dye had moved out of my liver and into the gallbladder. They ordered an MRI that night and would put me back on the HIDA table the following morning. They told me the MRI was normal and they could find no stones or blockages. The following morning, back on the HIDA table, the dye had finally moved from my liver to my gallbladder. With no evidence of stones, they believed my issues were caused by the sphincter of Oddi dysfunction. To diagnose sphincter of Oddi dysfunction, I would need to get an ERCP. I was discharged and told to follow up with the G.I. doctor that had seen me in the hospital and to get a primary physician. I called the G.I. doc the day after discharge and he could not fit me in for a follow-up appointment until a month later. The next day, the itching started. This wasn't the first time - it happened with former episodes. I was itching all over my entire body. I couldn't sleep. I wasn't eating for fear of another attack. I dry-heaved one evening after not having anything to eat or drink all day. I wasn't having the severe gallbladder pain, but all the other symptoms were still present. My eyes were jaundice. After 4 days of severe itching, I knew that I would either have to go back to the hospital or see a doctor ASAP. I had been calling all week to find doctors that were accepting new patients and could also see me right away. My mother-in-law finally called a doctor one morning and he said I could come in immediately. I didn't even shower, I rushed in, bedhead and all. When the doctor saw me, reviewed my hospital results, and I explained all of my symptoms, he did something amazing. He took it seriously. He got out his cell phone and called my G.I. doctor directly and told him I needed to have the ERCP right away. This was on a Thursday morning. The G.I. doctor said he could do it Saturday or Monday. While at this primary doctor, I also had more blood drawn. He said my liver enzyme levels during my hospital stay the previous week were "impressive", not in a good way. He wanted to see what they were now. He prescribed me periactin for the itching. When I mentioned the research I had done suggesting cholestyramine he prescribed that too. He then went above and beyond. He called me at 7:30 PM , after work, to tell me the results of my blood work. My liver enzyme levels were "pretty high" so he forwarded them to my G.I. and said the ERCP needed to be Saturday, not Monday. I went into the ERCP expecting our theory to be proven true - that I was suffering from a dysfunction of the sphincter of Oddi. When I finally woke up from anesthesia, the G.I. came over to explain his findings. Sphincter of Oddi dysfunction was thrown out. He said I had Primary Sclerosing Cholangitis. I had not heard of this. He first drew a crude photo on a piece of paper of a normal liver , gallbladder, pancreas, and the bile duct structures. He then flipped the paper over and drew a photo of what mine looked like. He explained that my biliary ducts were "pinched" and "kinked" in different spots and that was causing all of my symptoms. He then showed us the x-rays / photos and the bile ducts looked like a chain of hot dogs. He then wrote down 4 possible causes:

  1. IBD
  2. CMV
  3. HIV
  4. ??

He then told me my next steps - to get a colonoscopy and a liver biopsy, and to call my primary doctor on Monday.


As soon as I got home, I hid in my room and hopped on my computer to research. Rare disease. Progressive. No treatment. Cirrhosis. Liver Failure. Liver Cancer. Death.

I was devastated. Just a few hours earlier I was worried about recovery time from a surgery to cut a sphincter muscle. Now I'm reading about life expectancy and liver transplants. I read that Walter Payton died from it at age 45. I am still alone in my bedroom so I grant myself one minute. One minute to cry. I told myself I would cry for one minute, and then stop, and go on with my day. So that's what I did. It felt good. I informed my friends, family, and coworkers. The following day is Easter Sunday and I go and see my family in person and explain it all again to them. They are supportive. They ask me what's next and each person tells me if I need a liver I can have part of theirs. I don't even know if LDLT is possible with PSC is what I tell them. They're also not blood family - they adopted me when I was 4 years old. I don't know my biological father and my biological mother is dead. The only blood relative I have is my brother - he's a year older and we were adopted together. I tell them all that I have to wait until the liver biopsy to know anything more. I am scratching the entire time. The medicine doesn't seem to have helped. I still itch as I type this - a full 2 weeks since I was admitted in the ER. My G.I. doctor is supposed to call me tomorrow to schedule the biopsy and colonoscopy. I have an appointment with my primary doctor Friday morning to talk about everything and to get more blood drawn.

Right now the itching is the worst. I have open sores on my knees, legs, and feet. I scratch every waking second of the day and night. I can't sleep. I'm miserable. Somebody make the itch go away. The constant itching is getting to me, I can't watch our 20 month old son. I can't help my wife. Nothing relieves the constant torture. I'm going to call my doctor tomorrow and tell him the medicine isn't helping with the itching.

Hello Textbook,

Welcome.

This is a wonderful site. You can find lots of answers here to things that you didn't even know that you didn't even know. You can vent here. You can cry here.

I am end stage PSC. I have been on the transplant list for 2 years. Not giving up without a fight.

My best advise to you is research.

Oh yeah, the sodas and the fatty foods. Those days are gone.

Looking forward to hearing about your progress.

Kim

Hi Textbook,

I was diagnosed at 31 and read about Walter Payton too at that time. Now I'm 47 and doing fine, so take heart.

Payton had choliangiocarcinoma and you won't necessarily get that. I heard it's a 10% chance for people with PSC. Best to not think about that.

I too wonder about the liver biopsy being done because of its risks, but maybe they need more information.

Did they do anything during the ERCP to help you? For example, did they cut open the common bile duct? They did that for me and all my "gallbladder attacks" ceased immediately. I still have my gallbladder and they figure some stones passed because my symptoms ceased.

Did they give you a stent?

Your biggest problem if your pain has stopped is the itching. Did you read some of the posts on that subject? What has helped me immensely is castor oil on my legs and arms. It's easily attainable and helps preserve your skin's moisture for days. I wish I'd started that years ago. The cholestyramine didn't do anything for me. The next thing my doctor will try if I deem it necessary is the Revia, an opiode antagonist. He doesn't support the antibiotics, although I'm not so sure about that. Rifampin is an example.

Good luck.

Regarding the liver biopsy, I assumed it was to evaluate the current condition of the liver. If it was just to confirm diagnosis, I agree there wouldn't be a need since it can the condition can be clearly seen on scans. Following diagnosis, what steps lead to knowing whether you need a liver transplant, and if you do, when to get on a list? Does everybody just try to get on a waiting list as soon as they are diagnosed?

As far as I know, you don't go on a waiting list until somebody tells you so. They look at your MELD score. You can google MELD to learn more.

Its up to your doctor if they think you need to be placed on the transplant list.

Thank you everybody for all of the information. I will ask my biological brother, aunt, and uncle if they know their blood type. My brother and I were adopted together when we were 4 and 5 years old, so biological relatives (and medical history) make this a little more difficult. I really only have 3 biological relatives that I know, and two of them (my aunt and uncle) are not contacted often (a few times a year). So I would have a hard time asking them for part of their liver when I don't talk to them but just a few times a year.

My colonoscopy and liver biopsy have been scheduled for May 1. I have an appointment with my primary doctor tomorrow after getting blood drawn again yesterday. Hopefully I will know more about what's going on soon.

If I do need a liver transplant, I will ask members of my adoptive family who told me they wanted to help. Fortunately Michigan has a very good LDLT team at the Henry Ford hospital not far from me.

Hello Textbook. My daughter had a stent put in to her bile duct three weeks ago after becoming very ill. So far so good. She is doing well. The itching has stopped and she is gaining weight again. She has to have the stent removed in another four weeks and hopefully the bile duct will stay open. Ask your GI doctor about the stent procedure and if it could help you. As for her diet...No fatty foods or fizzy drinks. I understand your feeling scared and horrified, My daughter and I were beside ourselves with fear when she was diagnosed and 'googled'. We do still worry but are listening to others advice about living with PSC and it helps to get on here and talk. My daughter had a liver biopsy last year and she was fine except for the bad shoulder pain afterwards for which she was given painkillers. Try not to worry and I wish you all the best. Please let us know how you get on, I'm looking forward to reading future posts from you.

Thanks Blanch. I met with my primary doctor and he is upping my Questran (Cholestyramine) 4mg packets from twice a day to three times a day, to try and help with the itching. I got blood drawn again this morning and this time it will be tested for more stuff like INR. When I get those results I should be able to calculate my MELD. Previous blood tests did not test for INR because we thought it was the sphincter of Oddi dysfunction. These are my recent blood results:

Bilirubin:

  • 4.9 mg/dL

    Date
    April 23, 2014 12:50 p.m. CDT
    High
  • 8.2 mg/dL

    Date
    April 17, 2014 11:45 a.m. CDT
    High
  • 2.7 mg/dL

    Date
    April 10, 2014 5:14 a.m. CDT
    High
  • 2.7 mg/dL

    Date
    April 10, 2014 5:14 a.m. CDT
    High
  • 2.9 mg/dL

    Date
    April 9, 2014 6:25 a.m. CDT
    High
  • 2.3 mg/dL

    Date
    April 8, 2014 10:20 p.m. CDT
    High
  • 1.9 mg/dL

    Date
    Jan. 15, 2014 2:28 p.m. CST
    High

AST:

  • 151 IUnits/L

    Date
    April 23, 2014 12:50 p.m. CDT
    High
  • 298 IUnits/L

    Date
    April 17, 2014 11:45 a.m. CDT
    High
  • 49 IUnits/L

    Date
    April 10, 2014 5:14 a.m. CDT
    High
  • 49 IUnits/L

    Date
    April 10, 2014 5:14 a.m. CDT
    High
  • 90 IUnits/L

    Date
    April 9, 2014 6:25 a.m. CDT
    High
  • 87 IUnits/L

    Date
    April 8, 2014 10:20 p.m. CDT
    High
  • 97 IUnits/L

    Date
    Jan. 15, 2014 2:28 p.m. CST
    High

ALT:

  • 498 unit/L

    Date
    April 23, 2014 12:50 p.m. CDT
    High
  • 639 unit/L

    Date
    April 17, 2014 11:45 a.m. CDT
    High
  • 180 unit/L

    Date
    April 10, 2014 5:14 a.m. CDT
    High
  • 180 unit/L

    Date
    April 10, 2014 5:14 a.m. CDT
    High
  • 233 unit/L

    Date
    April 9, 2014 6:25 a.m. CDT
    High
  • 269 unit/L

    Date
    April 8, 2014 10:20 p.m. CDT
    High
  • 320 unit/L

    Date
    Jan. 15, 2014 2:28 p.m. CST
    High

Alkaline Phosphatase:

  • 206 unit/L

    Date
    April 23, 2014 12:50 p.m. CDT
    High
  • 226 unit/L

    Date
    April 17, 2014 11:45 a.m. CDT
    High
  • 144 unit/L

    Date
    April 10, 2014 5:14 a.m. CDT
    High
  • 144 unit/L

    Date
    April 10, 2014 5:14 a.m. CDT
    High
  • 139 unit/L

    Date
    April 9, 2014 6:25 a.m. CDT
    High
  • 161 unit/L

    Date
    April 8, 2014 10:20 p.m. CDT
    High
  • 202 unit/L

    Date
    Jan. 15, 2014 2:28 p.m. CST
    High

Creatinine:

  • 0.75 mg/dL

    Date
    April 23, 2014 12:50 p.m. CDT
  • 0.77 mg/dL

    Date
    April 17, 2014 11:45 a.m. CDT
  • 0.68 mg/dL

    Date
    April 10, 2014 5:14 a.m. CDT
    Low
  • 0.86 mg/dL

    Date
    April 8, 2014 10:20 p.m. CDT
  • 0.90 mg/dL

    Date
    Jan. 15, 2014 2:28 p.m. CST

Hello Textbook,

I understand not wanting to ask your Aunt and Uncle to donate to you. Can you ask someone else to ask for you? Sure they might say no, but maybe they will say yes. Believe me when I say that if you don't ask they won't say anything.

Kim

Hi Textbook,

My 24 year old boyfriend was diagnosed with PSC this time last year. He is from the Ann Arbor area of Michigan but is currently living out of state. There are many similarities between the two of you! I hope you are feeling better. A new diagnosis like this can completely throw your life upside down and it feels as though nothing will ever be normal or easy again. One of the best things I ever heard was to avoid looking at statistics online. If it says the 15 year survival rate is 70% (completely made up numbers) then the data is at least 15 years old, if not older and medical science moves quickly! We were at Mayo Clinic this month and the nurse practitioner there told us of a study they are starting in a few months... growing livers from stem cells! Way cool stuff. This site is a good one to help you feel connected and informed and also keep your spirits up.

Make sure you find a specialist who sees more than just a couple cases of PSC each year. This was one of Mayo's big messages to us. They said often times the providers that don't see a lot of patients wind up doing extra unnecessary procedures such as biopsies and stents and balloon dilations that might not actually be needed. Also the skill of the person doing the ERCP does matter. It sounds like you found yourself a very good Primary Care Provider at the end of your ordeal. I hope you find a hepatologist you like as much.

The itching sounds awful. My boyfriend takes Benadryl (recommended by his doctor) at night to help him sleep when he is itchy. Hopefully they get that sorted out quickly and you start to feel better.

Feel free to message me! Best of luck.

Thanks everybody for your thoughtful replies. Other than the persistent itching, I feel fine. I am now taking the cholestyramine three times a day and periactin (antihistamine) as needed. The itching doesn't seem to be as bad as it was, but it's not gone either. I saw my primary physician Friday and had more blood drawn. My INR is normal, at 0.97. Using my last measured bilirubin count of 4.9 mg/DL, that gives me a MELD of 12. Wednesday afternoon I'll start my preparations for my first colonoscopy. Miralax and Gatorade and sitting on porcelain throne. Thursday I get the colonoscopy and a liver biopsy to confirm diagnosis of PSC. If confirmed, they said they would refer me to the liver transplant team at Henry Ford Hospital. I'm not sure what happens next, as my MELD is currently only a 12.

The blood drawn Friday confirmed elevated antibodies for CMV (cytomegalovirus), which my G.I. doctor said could be one of the causes of my PSC. That did not come as a surprise to me, as I have had occasional cold sores my entire life.

  • CMV Ab IgG

    Date
    April 25, 2014 9:31 a.m. CDT
    Result
    4.3 AI (High)
    Normal: 0.0 AI - 0.8 AI

Dear textbook. My husband has been through the itching exactly like you. Many a nights I have wrapped cold towels around his legs and feet for help. He’s up to 16 grams of cholesteramine. Hang in there that helps. Take benedryl also at night time. We will keep you in prayer.

Fortunately my bilirubin levels have dropped in the weeks following my last episode. My itching is virtually gone now. I still get some hot feet at night, but that's happened all of my life so I can live with it. I'm back to eating normally. I feel completely fine right now, it's just when I have the episodes that take their toll.

Since I'm not itching now, I'm going to stop the cholestyramine and antihistamines and save them for my next episode.

I had a liver biopsy sample taken and a colonoscopy last week. The colonoscopy was completely normal, which was some good news and I have never experienced any symptoms of ulcerative colitis or IBS. I will find out the results of the liver biopsy mid-to-late this week. I believe if the PSC diagnosis is confirmed then I will be referred to a hepatologist at Henry Ford Hospital not too far from home.

Thanks everybody for your continued advice and support.

Hi text,

did you have digestive disorders earlier, based on your above post the whole thing started all of a sudden, normally i have seen cases where people have digestive disorder such as IBD/Crohns which then changeover to psc and pbc. You seem to be a rare case, i am researching this disiease for quite few years.

do give me your feedback

I have never had any digestive disorders prior to the PSC. I was rarely sick (maybe once or twice a year at most) until I started having the PSC episodes. I've never had IBD, Chrons, or UC or anything like that. Fortunately, for me, it's just the PSC.

I went to see my G.I. doctor on Monday. My liver biopsy results are "confusing" when my previous test results are also factored in. He basically said I'm still a mystery.

The liver biopsy confirmed signs of acute canalicular cholestasis but did not support chronic cholestasis. He said the liver biopsy does not appear to support the diagnosis of primary sclerosing cholangitis, however... he cautioned that the biopsy could have taken a tissue sample from an unaffected region of my liver or it could be negative for chronic cholestasis because the disease is still early in progression.

He also said that my blood tests returned an elevated ASCA antibodies count, which is associated with inflammatory bowel disease, such as ulcerative colitis, or more specifically, Chron's disease. I told him I have never experienced symptoms of either of those diseases and I did not think it was an inflammatory bowel disease. In addition, my colonoscopy was completely normal.

I have what appears to be two contradicting results.

X-Ray images of my liver during ERCP show strictures and beading - a sign of primary sclerosing cholangitis. The liver biopsy that was ordered to confirm that diagnosis did not.

Elevated ASCA antibodies indicate some type of inflammatory bowel disease, yet I have no symptoms and a clean colon.

He wants me to see an inflammatory bowel specialist at the University of Michigan.

Personally, based on what I saw on my ERCP images and based on the research I have done, I still believe my doctor's original diagnosis of PSC is accurate.

This excerpt from a Pathology book may support my theory that a liver biopsy isn't the correct diagnostic tool for PSC.

"Because the likelihood of sampling such smaller duct lesions on a random needle biopsy is minuscule, diagnosis depends not on biopsy but on radiologic imaging of the extrahepatic and largest intrahepatic ducts."

I'm going to try to obtain the X-rays from my ERCP from the hospital. I would like to look at them again and he recommended I show them to the U of M guy.

The good news is that I feel perfectly normal right now. I would still like to figure out what is wrong with me so I know what to do the next time I have an attack.

Good morning, Textbook. Going through your post was like an emotional roller coaster for me; starting out with the concern and worry for you, your wife and young child then ending on a somewhat positive note with the lack of itching and what seems to be a better healthcare team behind you. Please do keep us, Ben's Friend's, updated as you go through this journey. My fiance has PSC and UC which he was diagnosed with as a teen. You seem to be at a point he once was in, please feel free to ask questions. Looks like you have the itch controlled. We chose not to use meds (one less to worry about) and chose alternative methods that worked for him. A cream was prescribed though. I read (on this site and in researching) that the Questran (?) powder made the itch come back with vengeance once you stopped taking it. I hope that is not the case with you. If so, reach out and check in on previous posts regarding Pruritus (the itch name). I could go on writing... I'll stop for now, feel free to check in, ask questions and I guarantee you will find value in the time spent on this website. Enjoy the day, celebrate your family (I'm so happy to see you have a support team behind you!) and smile! God bless. -A

I went to the hospital I was admitted at in April and got all of my images put on a computer disc that I can take with me when I go to U of M. I called them to schedule an appointment but they said they wanted me to fax everything over to them before they would schedule it. I didn't do that last week and now I'm having another cholangitis attack. Stomach pain started at 9:30 PM, subsided a little bit, then I woke up at 6 AM and the pain was much worse. I just threw up my dinner. I am going to wait until my G.I.'s office opens and give him a call and ask him what I should do.

Stephen, thank you for the advice. I have had 10 attacks in the last 18 months. I only went to the E.R. the first time and the last time. If I went to the hospital every time I would never get out of debt. If this was the first time, or any different, or worse, believe me I would be on my way there. This is unfortunately getting to be a routine occurrence, and this one is unremarkable. I called my G.I. doc and he told me to come see him this afternoon, and we'll go from there.

If cholangitis is caused by a bacterial infection, why have I never been given antibiotics for my attacks? Is there any treatment for recurring but acute cholangitis attacks?