We are going on year #5 of diagnosis. Two summers ago he got really sick and we spent the next 6-8months having stents put in, taken out, ect... He did really good until last month; about 9 months since his last stent was removed. We were tickled pink we almost made it a year. So....backstory-bili went from 1.3 to 3 in 5 days. Stent was placed December 22nd, 2015 and we thought that was the issue. Of course we have been told the scarring/narrowing of the small bile ducts have gotten worse, but the stents always seemed to help. My husband had bloodwork done today because he still hasn't "perked up". He also continues to have the yellowing of the skin. His bilirubin actually increased to 3.5 despite the stent still being in place. This is not scheduled to come out until Feb 23rd. So of course we are really scared. Is this the beginning of the progression where nothing can be done and we just "wait"? Any input would be greatly appreciated; and I'm not looking for the "nice" version. We have the whole weekend to wait to talk to his doctor and so we thought we could pick all your brains. Thank you!
Lisa, having PSC by definition is a waiting game with a roller coaster ride thrown in for good measure.
It sounds like something is happening, but bili at 3.5, that does not seem too high. Do you know the meld score? How is his other bloodwork figures?
I understand the scared part, but know that you both will be able to handle whatever happens next.
Jeff
Have you considered vancomycin? That has helped some people.
JeffDC said:
Lisa, having PSC by definition is a waiting game with a roller coaster ride thrown in for good measure.
It sounds like something is happening, but bili at 3.5, that does not seem too high. Do you know the meld score? How is his other bloodwork figures?
I understand the scared part, but know that you both will be able to handle whatever happens next.
Jeff
You are absolutely right Jeff, it is a waiting game. As we all know we were just hoping to "buy" more time since he really had been doing well.
We've really been lucky (if you can say anybody with PSC is "lucky") in the respect that he hasn't been that sick. This is his 4th stent placement in the last 1.5yrs and with each placement his bili and bloodwork improved; indicating to us the worsening bloodwork was caused more from the large bile duct narrowing vs the narrowing up in the liver. This time it hasn't improved even though the stent was placed over a month ago.
His liver enzymes are of course worse than they were before the stent placement but in the grand scheme of things I wouldn't say they are terrible. His bili has also been higher (highest was 4.2) but again it has always gotten better with stent placement.
We have never been told a MELD score and I guess it is because his bloodwork has always improved. When he was first diagnosed (which was 4yrs ago, not 5. My apologies) the biopsy showed very mild liver damage, but at that time the only "symptoms" was elevated liver enzymes that were not explained by other reasons.
So, this is where we sit. Thank you for your reply. Any and all info is appreciated!
Lisa
Ted said:
Have you considered vancomycin? That has helped some people.No we haven't really discussed that. I did mention it last year when he was having so many problems, needing to have stent after stent placed, but we were told "antibiotics" really are only for infections. I also think because the stent placements "did the trick" nothing else was examined.He was on urso for awhile, but since it never really showed that it helped in the long run the doctor went ahead & took him off of it. I suppose the same is for the vanco. Maybe it will be an option now that the stent does not seem to be helping, more for helping to relieve symptoms.Thank you for your reply. That option had kind of slipped from my mind. We will definitely discuss it with his doctor.Lisa
Sounds a lot like me. Diagnosed 2001 and no real problems until 2015. Last year was… interesting. 4 ERCPs plus pancreatitis and now a cirrhosis diagnosis via MRCP. Itch is unstoppable, even with all the meds. Zoloft seems to help though, but it’s really a luck of the draw if I have a full night of sleep or not due to itch. My skin is pretty much destroyed, and I’m being listed for transplant with a MELD score of 8. The cirrhosis + transplant listing news is not even a week old, so I’m letting that sink in while my wife begs me to smile more. It’s not easy, but I won’t give up, and neither should you.
That being said, a 3.5 bili, while high, it isn’t TOO high. After my second ERCP last year mine jumped up to a 7 for a few days. I was never yellow before that. I don’t think there will ever be a “nothing can be done” situation, though. So many things are on the horizon that even with all of the bad news the doctors are telling me, I’m still fighting for a cure.
My plan is that in case my case get worse, I will immediately start vanco. I went to see the docs who are running the vanco trials and that was their advice too. Not much to lose, but lot to gain with vanco, so it sounds like a no-brainer.
Not all doctors are familiar with it, so need to talk to good liver doc about it.
Hey Ted, my thoughts exactly but I’m getting mixed opinions. My GI (who specializes in hepatology and sees more that one PSC patient) has been more open to the idea as of late, but my transplant hepatologist says that since vanco is one of the “heavy drugs given for stronger infections”, she says that long term use might lead to bacterial resistance, which in turn might lead to sepsis, which in turn would disqualify me for transplant. I tried it last year and didn’t see any real effect but it’s not out of the table for options in my opinion.
Nomad - oral Vanco is pretty unique in that it never enters your blood stream so taking it long term won't lead to any resistance issues with regard to systemic infections or the bacterial cholangitis that we experience. That's not to say there aren't resistance issues associated with the drug (VRE; the possibility of Vanco resistant MRSA), but it should be noted that these issues do not exist outside of the intensive care unit in a hospital setting. Basically, if you remain healthy and take Vanco in your living room for 60 years, you are unlikely to contribute to Vancomycin related resistance issues (there is little VRE or MRSA outside of a hospital). On the flip side, if you are a warm body in the ICU, there is a good chance you are contributing to Vancomycin related resistance issues even if you have never taken the drug (by getting VRE from other patients). Location is the single biggest contributing factor here and anything you or your doctor does to keep you from getting sick enough to need a transplant and out of the hospital (including prescribing Vancomycin) is helping to minimize Vancomycin related resistance issues.
Hey jtb, good to hear from you again. Thanks for the input. While my transplant hepatologist is against it, my GI is on board so I am seriously thinking about starting it again. Is there any new research on it since last year? What doses are given to adults?
Case studies from Georgetown and Cal Poly were released in the last year. The Georgetown one got a bunch of attention at the annual American College of Gastroenterology conference last fall (highest voted abstract in the liver category). I don't have them on hand at the moment but I can provide links later if you are interested. Dosing seems to be all over the map. The Stanford protocol starts at 1500mg/day and goes up to 3000mg/day if numbers don't normalize. Other doctors emulate the C-diff studies that found that low dose (500mg/day) is just as effective as higher doses. My personal thinking is that it makes sense to start at 1000-1500 then to taper to a lower dose once numbers normalize.
Always interested. I might even present this to my hepatologist to see if she changes her mind. I’ll probably try 500mg tid and see what happens. Will keep you updated as usual.
Thank you all for your input. Here is a bit of update:
My husband had another ERCP on the 4th which showed his stent was plugged with sludge. It also showed the right branch was plugged-which has not happened before I guess. His doc says he's never been able to do anything with the left branch, but the right has always been fine-he's always just had to clean out the large bile duct & place a stent. So I guess good in the respect that he was able to removed the stent, clean out the duct & he was able to open the right branch. Bad in the respect that there was even more progression in the disease. He also put my husband back on Ursodial for the next month or so to help the bile drain. Did say he would probably have him take the ursodial whenever there is a stent to help prevent it plugging again.
I asked him about the vanco and I really don't think he is up on it; or maybe I'm not understanding the use of it. He said it would really only be used for continuous problems with infection due to the narrowing and it wasn't the best option because it is used more for gram + & the infection is usually caused from gram -. Definitely going to do more research on this.
So that is where we are at. My husband will have his bloodwork checked again in 2-3 weeks to make sure labs are improving and go from there. :)
Lisa
jtb said:
Georgetown case study (published but journal doesn't have public link): https://www.eventscribe.com/2015/ACG/ajaxcalls/postersinfo.asp?titl...Cal Poly case study (interesting case where OV treated the patient's PSC but not UC - it often treats both): http://digitalcommons.calpoly.edu/cgi/viewcontent.cgi?article=1016&...Keep us in the loop and if you give it a shot remember that brand is super important. You can't go wrong with oral Ani or Prasco (if still available), or likely with any compounded IV generic.Very interesting info jtb. Thank you for sharing. I wonder if it makes a difference if the patient has Crohn's vs UC. So as I commented in my update-is vanco only used when there is persistant infection? My husband has more "structural" issues, he's never really had the pain/fever/chills.
For the purpose of treating PSC, oral Vancomycin (OV) is not being used as an antibiotic in the traditional sense. We don't know exactly how it works but the best guess involves a part of our immune system called regulatory T-Cells (Tregs). These cells help to prevent autoimmune response and people with PSC typically have a lower than normal level of Tregs in their gut. One way to boost the level of Tregs in the gut is through antibiotics. Very generally, gram+ bacteria tend to inhibit the production of Tregs and gram- bacteria tend to induce Tregs. OV wipes out the gram+ bacteria causing an overgrowth of gram- bacteria. The end result is that formerly deficient PSC patients tend to have normal levels of gut Tregs when they are taking OV. This boost in Tregs is typically accompanied by normalized LFTs, the elimination of symptoms, and in some cases improved imaging. The use of vancomycin has another benefit - it is one of the few drugs that never enters your bloodstream when taken orally so there aren't a lot of side effect to worry about.
My issue was also recurrent cholangitis infections - my common hepatic duct was clogged to the point they could no longer punch through via ERCP. These are due to a combination of scar tissue and PSC inflammation. By treating the PSC and stopping the inflammation, you may find that bile starts to flow again preventing future infections.
Anecdotally and from trials, OV has shown to help PSC-UC, PSC-Crohn's, and non-PSC UC. It doesn't seem to work with non-PSC Crohn's. The former conditions involve a deficiency of gut Tregs. Tregs for non-PSC Crohn's patients are typically normal - the speculation is that they are broken/not working in some as yet unknown way.
Most doctors don't do a lot of digging into the latest PSC research. If you are interested in OV as a treatment for PSC, get to know the treatment and provide the case studies for your doctor. The Stanford doctors have been treating PSC patients the longest (23 years) with OV and their clinical trials are a good place to start your research. If you need links or have any questions, let me know. I hope your husband feels better.
Oh my gosh jtb thank you so much for the easy to understand explanation. I have to be honest-I am a nurse and I was getting a bit confused. Yes please send me links so I can print info off. I plan on doing that with the couple of links you posted earlier.
Georgetown case report is not really published. All conference presentations will be 'published' as a supplemental copy by the official journal of that professional organization. Usually nobody will cite a presentation in any manuscript. No agency will count the impact factor of this type of citation. In addition, in this case, at the end of that page, you see 'No. P460. ACG 2015 Annual Scientific Meeting Abstracts. Honolulu, HI: American College of Gastroenterology',which means it was presented at annul meeting of American College of Gastroenterology of 2015 at Honolulu, HI. The physical location of the presentation board is at number P460.
In reality, this kind of data is un-publishable.
.
jtb said:
Georgetown case study (published but journal doesn't have public link): https://www.eventscribe.com/2015/ACG/ajaxcalls/postersinfo.asp?titl...Cal Poly case study (interesting case where OV treated the patient's PSC but not UC - it often treats both): http://digitalcommons.calpoly.edu/cgi/viewcontent.cgi?article=1016&...Keep us in the loop and if you give it a shot remember that brand is super important. You can't go wrong with oral Ani or Prasco (if still available), or likely with any compounded IV generic.
For many PSC pts, including myself, Vanco is a myth, even some type of superstition to some. But science is science. It needs concrete evidence. Short-term Vanco does show beneficial effects in some PSC patients. But there is no enough evidence to support its long-term use. You may say that Stanford study published at 2008 is a long-term study. Unfortunately that's just a case report, not a well-designed study, thus it's not strong enough. As the progress of PSC is highly unpredictable. It's hard to attribute all beneficial effects to one single treatment, if no control group is included.
Be cautious to interpret results from conference presentation. Most of the time, data presented at professional conference is not complete, or well-thought. The purpose of such presentation is to solicit scientific discussion on the subject, so the authors can go home and further work on the project, or write a grant proposal to seek financial support for future project. In certain professional organization, certain type of presentation has to be publishable. If so, they should be in print within a few months after the conclusion of the meeting.
If you want to know what is the real scientific research,
Check Dr. Lindor at Mayo and his work on ursodiol
http://www.ncbi.nlm.nih.gov/pubmed?term=lindor+KD+and+ursodiol+AND+%28Clinical+Trial[ptyp]%29&cmd=DetailsSearch
There are 42 original publications with him as an author. (I didn't go over them so the real number might be much less)
Check Dr. Cox at Stanford and his work on vancomycin
http://www.ncbi.nlm.nih.gov/pubmed/?term=vancomycin+Cox+KL
Total 4 publications on this subject.
It doesn't matter how long someone has been working on something. In biomedical field, you have to have some subjective materials to support your claim.
Again, it's not to deny the potential of vancomycin on PSC. We just don't know. Then we need to keep open mind and be honest.