Hello! I’ve been reading posts for over a year, and am very grateful for all the information shared here. My PSC experience was intense at first, and after that much easier to manage.
I had been timid about posting, because my case seems much less severe than many of the people here. My hepatologist said recently he suspects it might be a secondary rather than primary condition. At first I rejoiced because as he explained it, SSC is not progressive the way PSC is, and a possible future transplant seemed out of the picture. I started taking ursodiol, after being wary of it initially, and my lab numbers went down. Last week I had my first cholangitis attack in 6 months - disappointing! - and have decided to post. My doc now says that a diagnosis of SSC instead of PSC might not make much difference in how the disease impacts me, and that it may not be easy to truly know.
I’d like to know how others have encountered an SSC diagnosis, and if their experiences are different from those of PSC.
My history: I have never been jaundiced, and itching has not been much of a problem. An ERCP for stone removal in May of 2017 lead to PSC diagnosis. Less than 2 days after subsequent gallbladder removal I was back in the hospital with severe cholangitis that lead to sepsis. High fevers landed me back in the hospital twice after that - I was admitted 3 times between June and August 2017.
A few attacks of various severity followed, but I now have antibiotics on hand if the fever climbs over 102.5 (it happens so fast!) and I know how to stagger doses of tylenol and ibuprofen to lower the fever as quickly as possible.
Thanks for listening to my story, and all the best to those of you who are battling this strange diagnosis daily!
Sarah,
Thanks for introducing yourself to the group. We are glad to have you as a part of this PSC family. It’s nice to be able to go through this disease with those who understand what you are going through. We are here for you to support in any way we can. It sounds like you have really been going through some intense things in recent years.
I did want to make one observation for you to consider. I’d like you to consider discontinuing the use of Ibuprofen as well as any other NSAID’s like Alieve, Motrin, etc. Since you already have a liver disease, taking NSAID’s will only increase problems with your liver and you don’t want that. It’s recommended by hepatologists to only use Tylenol for pain relief and no more than 3,000 mg a day. Now that will vary some depending on the doctor but that is a good general rule of thumb. I am glad to hear you have a course of antibiotics when a cholangitis attack hits. My hepatologist made sure I was equipped with an Rx for just this case. I kept a filled bottle in a drawer right by my bed. Be prepared is a good motto for PSC attacks as well.
I trust you have a good week ahead.
Thanks so much for your reply, Mark, and for welcoming me to the community.
In answer to the NSAID’s (what does that stand for? …
Can you offer more info on how to reduce fever in a cholangitis crisis? My hepatologist has indeed told me to be very conservative with them, but in the case of lowering fever he seemed OK with using them initially. I guess it’s a question of getting the fever down so I don’t have to go to the emergency room. In the hospital they were giving me 500 mgs every four hours, and said it was OK to take ibu. after 2 hours, and take that every 4 hours, staggered with the tylenol. I doubt that the nurses had any idea about what a PSC patient should do, though (nor do most doctors.)
BTW my doc has remarked that my liver seems surprisingly undamaged at this point. My total bilirubin has never been over 1.2, and is usually around 0.8.
Since taking ursodiol all are now down by around 30%.
Pre-ursodiol my Alk Phos averaged around 340; AST avg. 63; ALT avg. 100.
Hi Sarah,
Regarding NSAID’s - Non-steroidal anti-inflammatory drug. These drugs would include over the counter medications like Asprin, Alieve, Motrin, Naproxin and such. There are also prescription based NSAID’s. One of the biggest concern in using them when you have advanced liver disease is that they are processed through the liver, not the kidneys. So…with an already diseased liver fighting to do it’s job, the introduction of medications like Ibuprofen only add to the struggle its already in. If your doctor is seeing your liver relatively undamaged presently then the PSC has not advanced that much yet so you could probably safely take Ibuprofen from time to time.
Keep in mind though that with PSC you start out with a perfectly healthy liver, but then the PSC starts to attack the bile ducts, creating strictures (blockages). As those increase bile has more difficulty draining from the liver and then the bile starts to kill the healthy liver cells. Over time when the diseased is far advanced you are in Stage 4 cirrhosis of the liver and the need for a transplant is greatly needed.
I’m glad you are on Ursodiol, it is a big help in thinning the bile so it can flow better through the damaged ducts. It provides a quality of life to the PSC patient that we greatly need. It is not a cure, but it will help you especially in the early years with PSC. Don’t over worry about all these things, but live one day at a time and live it to its fullest! I was diagnosed at 46 years of age and all those thoughts that one gets when receiving such a diagnosis can cause panic and fear to set it. But you just have to get a hold of yourself, educate yourself AND your family and just enter in to fight this disease while living your life, planning your dreams, etc. I was so blessed to receive my new liver when I was 51 years of age when my MELD suddenly went to 36 over a 6 month period from 12 to 36. I pray you will have many years of symptom free life, but at the same time, make sure you are with a hepatologist who is checking your bloodwork regularly, getting annual MRCP’s and ERCP’s when needed. Make sure your hepatologist is associated with a transplant hospital and can easily get you on the way to being listed when you need it. I requested listing when I was MELD 12. That was in November, 2014. Well who would have thought six months later I would jump to 36. We are all different so there’s no set pattern. Just stay up on top of everything along the way and you will be fine.
Take care and do keep in touch.
Sarah,
I am not sure I would want to reduce the temp by myself. To a point, raising a body’s temperature is the best way the body can help kill whatever has triggered the response.
My doc was adamant that if my temp hit an unexplained fever of 101.5 or higher, get to the ER. The reason was that the hospital records would reflect this, to document what was a likely cholangitis attack so that my meld score could be updated.
I’m glad your liver and bilirubin are behaving. I have heard the term secondary sclerosis cholangitis but have never seen it in a post/from a member…
Thanks for sharing, Sarah. So sorry you have been stuck with these cholangitis attacks. Does your doctor have any suspicions as to what may have caused the SSC? What is making him lean in this direction? Have you had any subsequent ERCPs or MRCPs?
NSAIDs vs. acetaminophen is a much bigger concern when you are dealing with cirrhosis and liver failure. NSAIDs are gentler on the liver than acetaminophen but should be avoided because some of the side effects of end stage liver disease such as portal hypertension and kidney failure can be further stressed by NSAIDs use. Either category of drug is probably okay in the earlier stages of disease with the usual caveat that you should ask and go by what your doctor recommends.
Thanks again, Mark - all these quick responses make me very glad I finally posted after reading other posts for over a year! I have an appointment with my liver doc in a couple of weeks and will revisit all these topics. Everyone’s input is much appreciated, and especially the encouragement to live life to the fullest! I’m lucky - I have a wonderful husband eager to support me, and have been able to work without a lot of disruption (other than hospital stays.) BTW I’m now 66 - quite old for this diagnosis - and am a working musician. People still think I’m younger than I am, so there’s that…
Thanks, for the response, Jeff!
I have been using acetaminophen to bring down the initial high fever and keep out of the hospital, then forgoing it the following day after the antibiotics kick in. I’m going to go over all of this with a fine-tooth comb with my doc in a few weeks, and will report what he says.
Thank you for responding, jtb. I had not seen much on this forum about SSB, so I don’t know if that diagnosis has come up as an issue with the members here.
My doc thinks that the stone removed from my bile ducts (PSC was diagnosed as a result of that procedure) might indicate secondary sclerosing colangitis - a response to a specific condition (the stone) rather than an auto-immune response that’s typical of PSC. So far no jaundice, RUQ pain is rare, a pretty OK liver - but lots of spidery scarring in the small ducts.
I was just checking in and read your post. I’m sorry to hear about anyone dealing with any of this stuff!
I also have had much less trouble with my PSC than so many others. I’m grateful for that. But something that I have learned is that when something hurts ME, it hurts. Please don’t ever feel bad about asking for help or for needing to share with others who might understand what you are facing!
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️ Doug