Newly Diagnosed and Pregnant

General
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Wanted to share my story and see if anyone has a similar diagnosis and or path. I’m happy to have found a place where I can talk through some of these issues and learn more to have better conversations with my doctor. This disease can be isolating as it seems to be so understood.

My story began in July 2015 when I was pregnant with first baby. I was about 35 weeks pregnant when I started experiencing very intense itching. I called my doctor feeling like something was seriously wrong. They dismissed it and said to take Benadryl, that it could be my skin stretching or my hormones causing a skin reaction. I kept thinkingit felt like it was under my skin. After 4 days, I was losing my mind and researching my symptoms because I couldn’t sleep, I thought I might have cholestatis. My doctor (and probably most of my family) thought I was crazy but he agreed to a blood panel. 2 days later my doc called me and said to come to the hospital right away I was going to have an emergency c-section. He explained it was cholestasis and my bile acids came back at 14. Fast forward 6 months after my son was born I got a physical to make sure everything was all settled. My liver enzymes came back very very elevated. I got an ultra sound that day but there were no findings. My primary doctor decided I should see a heptologist to monitor my enzymes. The heptologist made me feel like I didn’t need to be there that it could just be my body reacting to pregnancy.
Shortly after I saw her I began seeing blood in my stool. I had a colonoscopy and that came back clear. She monitored my enzymes for a couple months and they remained high. She decided to go ahead with an MRCP (w and w out contract) this also came back clear. Next was a Liver biopsy. She couldn’t find a good read so she decided to send it to a pathologist at Mount Sinai in NYC. Report came back as follows: showed few portal ducts containing dilated portal ducts and medium sized ducts containing predictable fibrosis, one showing mucinous metaplasia. Associated hepatic arteoles are thickened. There is minimal portal duct inflammation and ductular reaction. Many inlet venules are dilated. 6 of 30 portal tracts lack bile ducts. Mild portal track fibrosis. Lobules show rare inflammation with a pattern of alternating regeneration and atrophy. No a1at globules are seen on pas-d. Iron stain negative.
**Finding suggest primary or secondary Schlerosing cholangitis stage 2. **
If imaging studies are negative, small ducts will be considered.

So since I already had the MRCP and it came back with no findings we moved on to the ERCP. This was awful, I left the hospital and after a few hours had extreme pain. I ended up in the hospital with pancreatitis for 8 days! The good news is that the doctor who performed the procedure let me know that based on his findings, I do not have large duct PSC. I ended up switching liver specialists at this point because I felt like my original heptologist had other priorities and I knew I needed to have really good care going forward because something was wrong. So my new doctor is calling me a small duct PSC case. He said the possibility of this developing in to large duct is unknown and every case is different. He is treating me with 250mg of Ursodiol daily. The Urso brought my enzymes down after about a month, they are still elevated on it but no longer in the triple digits. He monitors me every 6 months. I have crazy itching episodes about every couple months now that last about a week and it’s hell. I have only tried the Questrian powder once, but I didn’t really feel like it worked well.
I am currently 9 weeks pregnant with my second child and already having a tough time dealing with itch not really being able to take much for it.While I am very happy that my doctor cleared me to have a baby and I am excited, I’m so nervous that this is going to make my PSC worsen or even cause my small duct to develop in to large duct because of the stress pregnancy puts on my body.

Sorry for the lengthy post here! Looking forward to having a place where I can find out more about this crappy disease, hear others stories and have some people going through the same situation to talk to during the tough times.
Thanks for listening!

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Sara,
Thank you for sharing your story. It’s unfortunate that there are physicians who just are not qualified to properly diagnose liver disease. I’m so glad you found a hepatologist that appears to be level headed and working with you through this. Stage 2 is not too bad and hopefully the PSC will move slowly. I know how the intensity of the itching can make you go almost crazy, been there done that. Each of us here on the forum have dealt with our itching in a variety of ways. What works for one might not work for another. For me, I tried everything over the counter I could find and some prescription based things and nothing worked. Finally my hepatologist prescribed Rifampin 300 mg twice a day and it eliminated my itching 98% most of the time. I’m not sure how it would interact with you being with child but you might ask your hepatologist about that as a possible relief. I would suggest the next time you need an ERCP that you make sure you are having it done in a hospital system that does liver transplants. Make sure they give you an physician that is trained in advanced endoscopy and does this type of procedure every day of the week. This procedure is not for the average GI doctor to be messing around with. As you have found out it’s a very invasive procedure and can trigger an infection or pancreatitis putting you in the hospital. I was fortunate with the 5 I had in a 4 year span to not get bad enough to need intervention through hospitalization. And, we are all different so again it can happen and does at times. We are here for you to help in any way we can. Make sure they are checking your labs on a regular basis and I would encourage you to track those labs in a spreadsheet or a method that works best for you to watch trends. Keep an eye on your MELD score. When your MELD gets up there around 12-15 I’d be asking them to refer you for transplant listing. PSC can change pretty quickly sometimes. Mine jumped form a 12 in 1/2015 to 19 in 4/2015. Then in late July, 2015 it jumped to 36. I was transplanted 2 days later. Hopefully yours will move very slow and you will not have to face transplant for a long time, but you need to keep track of things. Here’s a link for the MELD calculator. https://optn.transplant.hrsa.gov/resources/allocation-calculators/meld-calculator/

Take care,
Mark
PSC 2011 / Liver Transplant 7-2015

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Sara, congrats on the second baby.
Like Mark, I am glad you are under the care of a liver specialist, and seem satisfied with him.

There have been a few previous posts about pregnancy and psc. Try doing a search for that and similar terms and I hope you will find some useful info.

Jeff

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Thank you so much for your response and feedback! Wanted to add that I actually did get the ERCP from a heptologist in a hospital who specializes in them, so that was discouraging that it left me that way. I think I may go to a hospital in NYC next time. I am happy to hear the Rifampin worked for you, I will definitely ask my doc about that.

Thanks so much for your reply! hearing your story makes me feel a lot better.

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Thank you! I will look in to that.