I’m a 77-year-old woman, an unlikely demographic for a PSC diagnosis. I understand this disease, which occurs in 1 in 10,000 people, primarily strikes middle-aged men, IBD being a risk factor. I am not the right sex, age, nor do I have IBD,
After repeated blood work in 2023 indicating elevated liver enzymes, I had an abdominal ultrasound in December of that year. The scan revealed that my liver is mildly enlarged at 17.0 cm with coarse echotexture. There was evidence of mild hepatic steatosis and hepatomegaly. In January, 2024,I had a CT scan and in May, 2024, I had an MRI/MRCP. Among the findings are the following: The intrahepatic ducts in the right lobe of the liver are abnormal. The bile ducts appear disorganized near the porta hepatis with areas of stricturing. There is a dominant stricture involving the right main hepatic duct. The appearance is suggestive of primary sclerosing cholangitis. On September 27, I had an ERCP. Impression: short distal CBD stricture; dilated. Brushings obtained. No malignancy discovered.
I have no symptoms. I am concerned about what lies ahead, thus my interest in the PSC forum. Thank you.
Hi Phyllis. I am in a similar situation. 72 year-old female, no IBD, some liver fibrosis, beading and strictures of the bile ducts, normal liver enzymes. I do have Graves’ disease (a type of hyperthyroidism), which has been stable off-meds for several years. PSC is strongly associated with autoimmune disorders such as IBD and Graves’. I was diagnosed with PSC several months after a complicated cholecystectomy in 2017, which required an ERCP to remove retained gallstones. After I was diagnosed, occasionally I would get an uncomfortable pain in the back of my rib cage (it is kind of difficult to describe) but haven’t had the pain since about 2020. When I first joined the PSC community, another new member described feeling like a “ticking time bomb”, which is exactly how I felt. I settled down, and began feeling grateful that I was doing better than ok. I’ve had no progression of disease, and require only routine monitoring with my GI doc, after an initial meeting with the PSC specialist. You will probably hear other members speaking about vancomycin to limit or reverse progression. The unofficial consensus is positive but there is no hard data yet. Good luck to you on this journey.
I’m so glad to get your response. I do feel like a ticking time bomb. I’ve been googling PSC way too much. I feel fine now and I shouldn’t worry about what’s around the corner, but of course I do. Of all the complications, many of them serious, I’m most worried about the extreme itching, I know that doesn’t make sense, but I can imagine it and it sounds terrible!
I know I need to take one day at a time. I will try.
Phyllis,
About the best thing about PSC is that it is usually very slow to progress. There are people who have had it 20+ years and are doing ok. I lasted 4 years after my diagnosis before getting a transplant.
A quote I have heard is that people are more likely to die with PSC than due to it.
I understand the ticking time bomb analogy. We have to be in tune with our bodies, and listen to what it tells us. This helps when the inevitable symptoms rear their ugly head.
Tips I have learned:
Think long term, and have faith that you will get there.
Having something like PSC, you will realize that the fight is both physical and mental. Both are important to master.
PSC will test your strength, patience, flexibility and your sense of humor.
Thanks. My diagnosis is so recent and I’m
still trying to adjust to all this. If I don’t have any symptoms for the next 20 years, that would be amazing. I will be 97 by then!
Hi Phyllis. Agree, there is no need to get ahead of yourself. There are some helpful meds for itching on the market. Also, there are two new bile-acid transport inhibitors. One (Livmarli/maralixibat) is marketed for itching associated with other conditions, including Alagille syndrome, and the other drug is in clinical trials for PSC. The studies were done through Albireo Pharma (now part of Ipsen).
I just turned 73, and like you I am one of the rare older woman with a rare PSC diagnosis. I see many similarities between my experience and your stories.
I’m convinced the Living Donor transplant procedure is by far the best route for PSC, especially in older patients.
I received the ultimate gift of a portion of a healthy organ from a friend, in January '24. Both of us are recovering well, her liver has easily regenerated. I’m thrilled with this new chance. t has made all the difference to me, and given me back my life.
MELD score, used to determine the level of damage to the liver in cases of alcoholic liver disease and hepatitis DOES NOT WORK in case of PSC. The illness progresses without conforming to the MELD model, while waiting times for convenional organs (of patients taken off life support) are very, very long.
I hope your cases remain stable or slow growing - the very best outcome. However, I would start looking for a living donor and have one in place should you need a transplant. Family is the easiest place to look, of course, but if no family qualifies you do have other options.
My procedure was done at University Health Transplant Center in San Antonio, TX. They are rated very high as Living Donor specialists, and I highly recommend them. Especially important to me is their Champions For Life program, which helps people find possible donors by mounting campaigns on social media and other methods. I think it’s a crucial part for any transplant clinic, and I understand that a good number of hospitals now offer this.
All the best to you both! I’ll keep a look out for further posts from you - I haven’t been checking in enough lately!
Have a great Holiday Sason!
Hi Sarah. Thanks for the tips and additional info. I’m trying not to be complacent and at the same time, not thinking about PSC every moment of every day, like I did when I was diagnosed. My husband volunteered to be a living donor, but it hasn’t moved beyond that point. I hold my breath whenever I have routine labs. Liver transaminases are still in the single digits.
You’re comment about the MELD score and PSC is completely accurate. With PSC, it does not accurately reflect how sick we are. After several hospitalizations within three months, including one cholangitis attack and one sepsis, my doc was able to get several exception points approved that put me on the the top of the list.
Phyliss and Anne - very glad to hear you are both symptom free! By all means, don’t let it rule your life. I hope you have many more years without symptoms.
Jeff - they no longer use “exception points” in evaluating waiting list status, sadly. I was told that frequent cholangitis attacks is the only thing that will move PSC patients up the list. I had moved out of that stage, so I stayed at the same level, out of reach of conventional MELD considerations - PSC Purgatory is the term I learned here.
To jace0221:
I think the general age cutoff point for donation is 55.
You may be right about higher occurrence of return in living donor transplant. But If I had waited for conventional organ donation my survival chance would be much lower.
So I decided to pursue a living donor procedure.
All the best to you all!