Thanks everyone for your support, thoughts, and prayers during our trip to Utah.

Our first meeting on Tuesday was with the hepatologist who is also the Medical Director of Intermountain’s Liver Transplant Team. We had two surprises when meeting with him. First, he said he believes I do not have PSC, after studying my imaging he thinks it is "pseudo PSC" or "portal billiopathy." This was pretty shocking because we have been told this is PSC for four years and TONS of doctors, boards, and committees have reviewed my imaging at UCSD.

Portal billiopathy means that the collateral vessels created from my portal thrombus have grown so extensively into the liver that the vessels are clamping down onto my bile ducts causing the strictures (that look like PSC) and causing all of my symptoms. Second, he said due to the high pressure in the portal system (from the blockage in the portal vein) the ERCPs exchanging stents are too risky to continue. Pulling out the old stents could cause significant bleeding, which is something that has already been a problem in past procedures. This doctor was concerned that if the bleeding became too severe, the only way to stop it would be a procedure that changes my anatomy making transplant no longer a possibility for the future. He called me a ticking time bomb multiple times and wanted to move towards transplant as quickly as possible. After this appointment, Tuesday evening, we messaged my hepatologist at UCSD and called USC to move up our appointment with them from 4/1 to 3/26 to try and get another institution to review my case. 

Wednesday, we were in appointments all day from 8am-3pm. It included labs, an education class, and meetings with a social worker, dietician, finance, and transplant coordinator. We then met with a transplant surgeon on Thursday morning. The surgeon actually had a differing opinion than the hepatologist, and he still feels like the diagnosis is likely PSC. It was discussed that even if we can't agree on the underlying diagnosis, it appears that transplant is the only path forward. Unfortunately, there is not just one test that can give us a definitive diagnosis. We discussed with the surgeon the different surgical approaches that are needed due to my unique anatomy from the portal thrombus. The transplant surgical team seems very capable and trained for the complications associated with my anatomy that make it a difficult transplant, he said they would need the 3 head surgeons together for the operation but they could do it.

The transplant committee was scheduled to meet right after our appointment with the surgeon, at that time they were planning to review my case. We were told that we would receive a call after the meeting, likely that I would be listed, and we would just have to wait a few days for final insurance approval. We got to the airport and flew home. 

When driving home from the airport, we got a call from the transplant surgeon saying that after consulting the whole team they decided *not* to list me. (Shock, disorientation, AGAIN). My current MELD score is 8 (when the stents are working well) and anytime someone's score is under 15 without cancer, the benefit of transplant is questionable, so they really have to think carefully about the risks involved. When the whole team put their heads together, they decided IF the diagnosis is truly portal biliopathy (which no one can truly confirm), decompression of the portal system could potentially relieve the pressure of the vessels around the bile ducts and relieve my symptoms. If the bile ducts remain scared down, that would indicate it is truly PSC. The surgeon would like to try a unique shunt surgery (connecting the inferior mesenteric vein to the vena cava) before trying a transplant in hopes that this is portal biliopathy. Theoretically, if some blood flow is redirected out of the liver, it should reduce the pressure on all these varices that are restricting the bile ducts. We are waiting to hear back from the surgeon to schedule this in the next 3 weeks. At this point, we will fly back out there for this surgery, and it will require a 2-4 day stay in the hospital. 

We are hoping to talk to my current hepatologist at UCSD today. We also moved up our appointment with USCs hepatologist and transplant team to this Tuesday. We have already had several calls with the team at USC and have been updating them with all the current information. We are hoping it will be helpful to have my case reviewed by a third prestigious institution since there are so many unique variables. The challenges of my case truly seem one of a kind and extremely rare.