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:
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.