A recent burden that has come to my heart as I have moderated on this forum for a number of years now, is the lack of good solid information of what the new PSC patient should expect as the disease progresses, the procedures and tests one will need along the way, etc. I’d like to start a discussion about this. I would appreciate if we would put our heads together and come up with a list of the most common questions you as the PSC patient wished you would have been told at diagnosis and other things along the way that if it had been in a printed booklet, would have made your journey with PSC much easier. I’d like to develop a good presentation with all your help and then present this to a group of hepatologists to see if they would agree to have this edited and published. I believe if we could come up with a good solid booklet for our new patients it would bring comfort to many a new patient and their families. We all know the fear and uncertainty that we experienced when first diagnosed. Not knowing if we would live to see our children marry and have grandchildren, etc. Please give this some thought and then start replying to this post with your thoughts.
On a side note, I’d appreciate your thoughts in regards to an upcoming privilege I’ll have in April. I have been asked by one of my hepatologists at Duke to attend a Liver Day on Capital Hill to lobby congress for funding for liver disease research, including PSC. I’ll have the opportunity to be in several different meetings to discuss my PSC story with members of Congress. I believe this is a rare opportunity and I want to be well prepared when I go. It’s set for April 18th.
Take care everyone and I look forward to hearing your thoughts on this hopeful guide for PSC patients.
For your congressional lobby efforts: One aspect that would be helpful in my opinion is to relax some FDA guidelines a bit, in order to provide new treatment efforts available sooner.
FMT (fecal transplant…FDA tried to ban this and people were using youtube to share tips how to prepare this at home). Eventually, patient-pressure worked and FDA somewhat relaxed its opinion and now this is widely utilized option for CD infection (and UC seems to work as well, while PSC is trialed, but FDA does not allow these outside of clinical trials, I recall).
Oral Vancomycin. This seems to work quite well in PSC, but because of FDA, this is not widely available for PSC patients (unless you know the “right” doctors etc). Allowing treatment options like this to be available, even without $100M trial, would be helpful.
i know there are no cures for PSC but i wish my doctor would have given me suggestions as things i could do for a better quality of life : like drinking coffee, start working out, take vitamin D etc.
I think you have a great idea with a presentation and/or booklet to help inform and guide new and old PSC patients. I have been diagnosed with PSC for 30 years and have had UC for 45 years. I am currently on the liver transplant list with a MELD of 16. I take 13 different meds and have no real side effects. I am in incredible health considering that I am on the transplant list. Here is a list of questions that might be helpful if answered by research, data or personal experiences.
- I believe people would like to know if there is a way to distinguish between a slow and fast progression of PSC.
- Should I be screened for PSC if I have Crones or UC?
- What are the treatment options?
- What treatment options do I have for the symptoms I may have. EX: Itching (hydroxazine and rifampin), Leg cramping (lots of fluids and rolling out the lactic acid from muscles), etc.
- What are the symptoms that should take me to the emergency room? For me it is a sudden onset of aching muscles, fatigue, fever, and throwing up. These symptoms have presented themselves 18 time in the last 8 years and every time I have thrown another blood infection.
I believe there is a very direct correlation between the auto immune aspects of the intestinal flora/biome and PSC. My doctors have just put me on Xifaxan. With this said, Are there any research studies that target the gut for preventing or slowing PSC?
I think this is a start but I have many more suggestions and experiences with the hepatologist/GI/ and transplant doctors that might be helpful. Please reply if you would like more of my suggestions. I can go into more discussions with meds, experiences or lifestyle.
Thanks for replying to this post. YES, I would like any and all input you can bring to the table for this hope. This has got to be a collaborative effort from all of us to make this possible. We all have reacted to PSC in different ways yet similar as well in many aspects. Once we have enough information and responses from this forum, I’d like to organize it in a way that I can present it to some hepatologists at Duke for consideration to have it edited and done in a booklet form that can be given to new and existing PSC patients. I have seen so many of our patients that come to this forum that it’s obvious they are oblivious to all that is happening to them. They get a diagnosis but then they are not given a clear path of what to expect, so the incredible fear for our families, our futures, etc. just looms up and overwhelms us. We’ve got to do a better job educating PSC patients from the very beginning. So something like this that they could walk out of the office with and go home and read I believe will go along ways in helping them deal with this rare disease. We’ve got to give folks hope amidst such a scary diagnosis, for I have seen that wonderful things can come out of all this sickness with liver transplantation. So, please be as detailed yet concise in anything you can add to this hope. Thank you again for your contribution.
Good idea on trying to draw up a list of suggestions. Two areas that I think are important are the role of biopsy and the use of ERCP.
Regarding the biopsy, according to the studies that I have read, the advantages of having a biopsy do not out weigh the risks. Its my limited understanding that biopsies are not currently being recommended by the broader community of heptalogists.
In my discussion with specialists at Vanderbilt, they expressed their reticence to perform an ERCP, due to the potential complications. (At the time I was suffering from cholangitis induced by a stent that became occluded. It was put in at a different hospital.) An ERCP can be necessary and helpful, but how frequently they should be utilized and under what circumstances is a discussion point.
Thanks Steve. All good points. I’ll be sure to include them.
Congrats on the lobbying opportunity, and good luck.
I was diagnosed following an ERCP. The doc came in, said I had a liver disease called psc, that there was no cure and that it would lead to a transplant or death, and that they could only treat the symptoms.
He apologized and left. I immediately called my wife to let her know what had just happened and to research the daylights out of a liver disease called psc.
That time in between that call and her call back was excruciating. Questions? Yes
Why me and what happened?
What can be done to prevent or minimize any symptoms?
What are the general time frames involved?
What can I expect at each stage of the progression of psc?
What do I need to look out for/avoid?
All for now.
yes I agree, when my doctor diagnosed me he said I had PSC that it was a liver disease that would give me cirrhosis and there was nothing doctors could do about it. When I naturally proceeded to ask him questions he told me to Google it!! I feel like all those questions we all have initially should be able to be answered.
Thanks for all the comments folks. Keep them coming. I really hope this will make a difference over time. We’ve got to advocate for those new PSC patients that come after us so they will have more information and not over react as if this is an automatic death sentence.
My doctor did the opposite. He understated the seriousness of PSC to the point that a common cold seemed more harmful than PSC. Well, it took about 1 second of googling to find out that isn’t the case.
I don’t know which approach is the worst, but I think a doctor should inform the patient of being diagnosed with a serious diseases, such as PSC, in the same way as they do with cancer.
I’m sorry that your doctor spoke of PSC so carelessly. It certainly doesn’t resemble at all anything as harmless as the common cold as you have well found out. I do hope now you are under the care of a good hepatologist associated with a transplant hospital. This will be very key in the years ahead as the disease progresses.
Thanks for contributing to this thread. I believe this will be helpful in what we are desiring to accomplish.
A really good starting point for introducing someone to PSC are the PSC clinical guidelines. These are updated roughly every 5 years and cover many of the common questions, including those involving the method of diagnosis, variants of PSC, risk monitoring, symptom management, and potential therapies. I think that an easily digestible, cliff-notes version of these guidelines would be very useful for new patients. Here are the guidelines from 2015: https://www.nature.com/articles/ajg2015112.pdf?origin=ppub
Thank you very much for that jtb. Very useful info.