I was diagnosed a year ago and at the time the thought was cholangiocarcinoma. After two ERCP’s and removal of dog food sized stones I was diagnosed with PSC. I had a precancerous tumor in my ducts so the last year has been spent fighting inflammation, ERCP’s every 12 weeks or so and on august 4th the biopsies came back clean and the tumor had reversed and was no longer evident.
In November I was told that a living transplant and meld exception was the course to take, We have been working towards that goal but I was called to Dallas last week and told that because I had successfully reversed the tumor and my cancer risk was lessened I may no longer qualify as an exception.
Does that mean I will be inactive on the waiting list? Will all my potential living donors have to re-qualify later? I realize you “can” get removed from the list because of improvement, I realize I “should” be happy. (and I am happy I am healthier) but “great…wait to get sicker now” wasn’t something I had expected. Has anyone else had this happen to them? can I try to list at an alternate or do all transplant centers operate pretty close to each other on exceptions?
Hi. Glad your biopsies came back negative for cancer. Regarding transplant listing that depends on MELD score. What is your current MELD without the exception points?
Very good only like an 8 or a 9. We have been concentrating on controlling the inflammation and keeping the ducts clear. As part of that I have been using a supplement called bcq which is an anti inflammatory. Also because my gall bladder is fibrosed to my liver a major trigger that seems to cause inflammation and attacks is any kind of fats, so I try to stay 15-20% of a normal diet for fats. As long as I use the urso and the bcq and eat zero fat my blood work is pretty perfect.
Maybe I can do that long term, but according to all I have read and been told, the damage is still happening and someday anything I do will not help anymore. Just trying to determine what my new focus is.
You are pretty much mark-on on all the points you mentioned. A MELD of 8 or 9 at least where I live will not get you listed. I listed at a MELD of 12 in January of 2015, it went up to 19 around April, then suddenly jumped to 35 on July 20th. I was transplanted two days later. URSO does help. I was on 1200 mg for the 4 years leading up to needing a transplant and it did bring relief. Try to stay in the best shape possible and just keep living life to its fullest until you are forced to do otherwise. Unfortuantely PSC will catch up with you one day, but don’t let that be your focus everyday, just live for yourself, your family and plan for the future. As your disease progresses, make sure you have a power of attorney, healthcare power of attorney, etc. Make sure if you are married that your wife knows about everything, how to pay the bills, etc. The list goes on and on but it’s important to plan for the worst but hope and pray for the very best. Last January when I was listed and was feeling so poorly (however didn’t miss a day of work except for procedures and appointments) I finally went down and made my funeral arrangements. Picked everything out casket, vault, etc. Thankfully we didn’t need to execute that plan but it’s all ready so my wife doesn’t have to bear that burden. Hopefully you are a very long time from thinking about all that but keep it in mind. Keep up the good work!
Thank you for the info. I had hoped that all the MRI’s would give some indication of how long or the speed of progression but it seems that it is very individual and one day you just don’t compensate anymore. I don’t want to trade one problem for others, and am very hesitant about the post transplant effects since outwardly I am very healthy and have zero symptoms right now. I am still technically on the list and have a living donor so we will see what the decision is from the transplant center and hope it is the right one.
My husband also focuses a great deal on diet and has felt great so far since seeing a good specialist team and managing fats/other dietary changes.
For comparison - In Maryland they want him to get a transplant earlier but with a score of 7 he’s barely active on the list but they allowed him on.
It’s strange he’d be so low to me as he has to have a constant stent in place to allow bile to flow but they are petitioning for a score increase for him- they’re hoping for exception points too.
The transplant team at JH did bring up something interesting - a case where a Hep C positive liver was used early on for a young man with PSC then they cured the Hep C once transplanted since those are ‘lower quality’ livers. There’s no firm answer on if the cure for Hep C reverses long term increased cancer risk associated with Hep C but something we have sort of been wrapping our minds around.
Mark, you mention live the best life possible. They’ve asked us to notify of any overnight travel and not make big trips as we “might receive a liver”. This has been discouraging for my husband who enjoys traveling (especially if down the line he might not be up to it). With a MELD score of 7, we’re hoping to get someone over at JH to speak frankly on if that’s really necessary at this point but they weren’t able to comment.
How have you approached being low on the list early on while feeling good - what was the right answer for you there? Did you still take some trips with family if feeling up to it or strictly follow that overnight travel instruction.
Thanks for writing in. Keep in mind that some people are really sick with PSC and still have a very low MELD score. MELD is a major factor for transplant and that sometimes hurts the folks with very low MELD’s that need one sooner than later. You need to know the doctors reasoning for pushing for the HepC donor. If he doesn’t have a very good reason I personally would not have accepted the high risk donor liver. I have heard however of folks having good outcomes despite the HepC once they cured that after transplant with Harvoni treatments. I can’t say what’s best for your husband but I’d certainly ask for a more thorough explanation and possibly second read by another hepatologist at the transplant center before accepting high-risk. I’d rather go with a living donor before I did that if that would be a possibility for you.
As far as travel is concerned, when I was placed on the list I was a MELD 12 in January '15. We took a trip in late May and my MELD was 19. We did make sure we were within 6 hours of both transplant hospitals I was listed at just in case. They knew we would be travelling and they had all the contact numbers where to reach us. If he feels fine or I should say fine for a PSC patient I’d say go for it. We went to the mountains that summer with my MELD 19. I felt pretty rotten but so enjoyed being with my family. It would only be a month and a half later that my MELD jumped to 36 and I was transplanted 2 days later.
You say they are constantly replacing the stent??? Has he had a number of episodes of infection as a result of all those stents? Have they not tried a balloon dilation and see if it would hold for any length of time? If not, I’d certainly ask. Make sure it’s a advanced endoscopist doctor that is doing the procedure and I’d stick with the same doctor each and every time.
I hope this helps. Feel free to contact me back if needed.
On the Hep C / Risky Liver option: Johns Hopkins has began offering this option for low MELD score PSC patients as a means to get it done sooner. One patient of the Transplant Hepatology team there has elected to do this, there have been a handful of patients to do it nationally at this point as the Hep C meds are somewhat new from my understanding. The hepatology team presented that option as an emerging option. It’s still a big decision and one my husband feels a little uneasy about. It’s being mentioned in the conversation about living donor or low risk liver, where the Hep. C liver is something the surgeons would prefer to his waiting if the MELD isn’t promising a timely resolution.
That’s a good idea to just make sure within a 6 hour travel time to arrive at the listed hospitals. May I ask what made you decide to be listed at two? How did their transplant programs compare?
He has had a constantly replaced stint, they’ve done balloon dilation and found that it couldn’t stay open. The dye they use has caused infection in the past but he’s had a good couple round streak of no infections. There’s unfortunately no way around doing it for him because of how tight the structures have become there. He’s seen the same great guy on the JH Endoscopy team - you’re right it’s very critical to stick with a very skilled ERCP specialist.
I agree with your husband. I would be hesitant as well. I believe you need more concrete information as to why you should go with transplant this early with low MELD. Are the ERCP’s indicating a breakdown now of the ducts with having to go in so many times to re-stent? Is he functional, working still, etc. Has good and bad days or is he really really jaundiced, just feeling like death warmed over, etc.? I think not based on what you have told me. I just don’t see on the surface with the limited information I have that you should grab this chance so quickly with such a low meld score. If his MELD suddenly kicks way up like mine did they’ll push him to the top of the list. Now of course I’m not sure the transplant rate in your state, etc. That would be something to discuss with Mariel Carr. I’d encourage you to contact her especially if you decide to dual list, if you are in a high demand low-donor transplant region, etc. Her site is http://comparetransplantcenters.com.
The reason I decided to list at two centers is that in North Carolina we have two transplant regions. One region is Duke Medical & UNC Hospitals and the other region is CMC Hospital in Charlotte. Duke & UNC are allocated livers between themselves, whereas CMC in Charlotte covers that entire region. Of course if you should end up in a Status 1 situation, they will find a liver anywhere in the nation to keep you alive and transplant you. Being listed in two regions gave me the advantage of more opportunities for a transplant. Thankfully I got the call from my main hospital at Duke which is closer to home. My insurance though Blue Cross Blue Shield of NC paid all my transportation/hotel/parking expenses for my trips to CMC Charlotte for the evaluation process and would continue to have paid should I have been transplanted there.
I just wanted to be as prepared as possible especially when things turned for the worse. When I got the call I believe I only had weeks to live. Thank God I got that call! Glad to know he has a consistent ERCP specialist. It’s like a good auto mechanic. They know that engine inside and out and what it takes to make it purr again! I wish you well in this process. It’s certainly a team effort as a family and you need as many members of that team as possible to give you the love and support make it through this journey. Take care and keep in touch!
Here are a few photos. I trust they will give you hope of how your husband will hopefully be after transplant when that day comes. What a difference a year can make.
