1h
Hi everyone
Hi Joki . My son 19 years old has been sick off and on since November of last year. He started with nausea, vomiting, diarrhea and became jaundice after I picked him up from college. His main common bile duct was dilated , pancreatitis with elevated amylase and lipase. Fever . He was in the hospital for 7 days and was diagnosed with acute gallbladder infection. Started him on Flagyl and Cipro and he started feeling much better.
We got discharged from the hospital but as soon as he stopped talking ATB his symptoms would come back fever elevated liver and pancreas enzymes. He had a gallbladder removed in January of 2020 so they did a liver biopsy then too which was normal. Because of over use of ATB he developed a c diff which is only treated with oral Vancomycin as soon as we started it he started feeling better. After 6 weeks we checked the blood work and the enzymes were normal, but 4 days later from stopping it his symptoms would come back and the enzymes would go up .
He had 3 ERCP, 3 MRI , two abdominal cat scans , US all normal.
He was tested for all autoimmune liver decease all negative even PCP caused my autoimmune pancreatitis was negative IGG4.
About 2 months ago we stopped Vancomycin po and started URSO 300 mg three times a day and it was working for 2 months liver enzymes went back to normal, however he started with stomach pain and symptoms came back again. So he had ERCP done last week and the doctor said it is consistent with small ducts PSC.
We started him back on Vancomycin again and his dose is rather small 125 mg twice a day but we know this does works for him from before.
He had colonoscopy and endoscopy both normal so no UC or bowel problems .
Any advice for us ?
I thank you so much.
Desperate mother
Olivera,
With psc, it generally progresses very slowly. His colonoscopy/endoscopy were good.
But take a deep breath. People can live for many years with psc, but yes, we have to be ready at any time for a symptom to rear its ugly head.
When I was first diagnosed with it, I found a quote that said that from diagnosis to a transplant, the average time was 12 years. I lasted 4 years, two months.
Living with psc takes a mindset of focusing on the future, and living your (son’s) life the way he wants to.
Would recommend that he be treated by a hematologist, not just a GI. You want a liver specialist, not a generalist.
Jeff
Thank you for responding back appreciate. I am so happy you are doing well after the liver transplant.
I just know from now on our lives has changed so much, I cannot sleep with out thinking about him and being afraid of the symptoms coming back.
He is so young and was never sick before at least we have not noticed anything .
We are seeing Hepatologist at Cleveland Clinic in OHIO.
The only confusing part is his liver biopsy was fine, I thought that was the only way to diagnose small ducts PSC?
Maybe his hepatologist will schedule the second liver biopsy.
His symptoms are severe when he is off ATB.
URSO was working for about 2 months then liver enzymes went up a bit not much.
We resumed Vancomycin po last week he seems to be doing ok at the moment.
I am not sure if there is anything else I should be doing to help him out ?
Any supplements, food?
His diet is pretty good, we cut out fat and he doesn’t eat meat at all .
I truly thank you for any advice.
Olivera
Hello, Olivera. I was also diagnosed at 19, though I was mostly asymptomatic for the first dozen years.
If PSC was diagnosed via ERCP, it is by definition normal, large-duct PSC. In this case I’m speculating that the strictures were only seen on the smaller ducts within the liver. In contrast, small-duct PSC affects only the tiniest ducts that can’t be seen on imaging (ERCP, MRCP) and is only conclusively diagnosed by hitting the right spot on biopsy.
Fat soluble vitamin deficiency is a big problem with this disease. If his hepatologist/PCP have not already done so, request a blood test to check on ADEK and also for a DEXA scan every 2-3 years. I developed osteoporosis about the time I started developing more serious PSC symptoms and there is a good chance this could have been avoided if my doctors and I had been more diligent. To this point, low fat isn’t necessarily the answer when it comes to diet assuming it doesn’t exacerbate symptoms.
I’m also on vancomycin and it has been a life saver. What is interesting is that many, but not all, who experiment with treatment cessation usually see PSC returning in the 1-2 month range. Your son’s enzymes and symptoms returned 4 days after stopping vanco?
Hi JTB .
You are right she did see strictures on the small ducts with ERCP and with his symptoms or recurring symptoms of cholingitis , plus ERCP and MRCP normal.
How long have you been battling this disease?
Are you still on Vancomycin? Do you have UC or any other inflammatory bowl disease?
I thank you for fat soluble vitamins information will definitely ask his Hepatologist.
I am just so worried and scared not knowing what future is holding up for him .
You guys give me inspiration and hope .
Thank you so much for the bottom of my heart
Yes his symptoms would come back as soon as we stop ATB about the 4 th day. The only difference was when we started URSO symptoms did not come back on the 4 th day, came back after 2 months. FYI
Thanks
I was diagnosed about 20 years ago when I was 19 years old. I started to get very sick in 2013 and started oral vancomycin treatment about the time I was going through the transplant listing process due to recurring cholangitis. I’ve been on vancomycin for 7 years with normal liver function tests, no symptoms, and no further disease progression (based on Fibroscan and MRCP). I have no IBD symptoms and have had a negative colonoscopy.
Did your son’s liver function tests (ALT, AST, ALKP, GGT, bilirubin) normalize on vancomycin by itself? Were his numbers elevated when his symptoms resumed on just urso? Is he currently taking both vanco and urso and are the numbers back down to normal?
Hi job.
You don’t know how much I appreciate you sharing your experience with us and helping us out . It is true means a world to us . So inspirational and hopeful.
Yes his liver enzymes did go down completely after use of Vancomycin in June, but as soon as we stopped it his enzymes went up within 3 to 4 days.
I was diagnosed about 20 years ago when I was 19 years old. I started to get very sick in 2013 and started oral vancomycin treatment about the time I was going through the transplant listing process due to recurring cholangitis. I’ve been on vancomycin for 7 years with normal liver function tests, no symptoms, and no further disease progression (based on Fibroscan and MRCP). I have no IBD symptoms and have had a negative colonoscopy.
Did your son’s liver function tests (ALT, AST, ALKP, GGT, bilirubin) normalize on vancomycin by itself? Were his numbers elevated when his symptoms resumed on just urso? Is he currently taking both vanco and urso and are the numbers back down to normal?
We stopped Vancomycin again in July and started him on URSO 300 mg three times a day and he was great on 8/18/20 liver enzymes never better but week later went back up again. So right now we really don’t know what to do . Start Vancomycin again and keep him on URSO too ? What do you recommend?
Again I thank you so so much any input is appropriate it .
I would definitely have the heptologist keep your son on Vanco, perhaps starting again at a higher dosage. My son is on 500 mg 3x/day. He has always been asymptomatic but a regular physical with a blood test came back with very high liver enzymes. Since starting Vano, his blood test results have been normal.
Since your son has responded to Vanco previously, he should get back on it, and stay on it until we find a cure (and we will).
Jace .
I thank you so so much for responding back . Will definitely go back on Vancomycin again.
I am so so happy Vancomycin is working for your son too .
I am truly hopeful that we will find the cure for this horrible disease.
Jace
Will do . We really don’t have a choice , they are just a babies too young to give up .
I will definitely stay in touch. I probably will need a lots of encouragement and a lots of hope.
Thank you again so much this really helps me and him to try to find about as much as possible about this disease so we can be prepared In some way.
Olivera
Hi Jace and everyone.
I need help knowing how do you diagnose small ducts PSC?
My son had ERCP last week and that is how he was diagnosed, even though his liver biopsy was fine in January when all of this started.
I thought to diagnose small ducts PSC you need liver biopsy and MRCP and ERCP are normal with small ducts , so I just would love to know how was she able to see small ducts narrowing as she put it with ERCP?
His MRCP was normal.
Thank toy so much
Hi, my PSC started 15-20 yes ago from nowhere.Attacks again from nowhere 3-4 times p yr. From 48 hrs agony to jaundice,dark brown pee,grey stools. All liver function tests off the scale,but with large amount of tea and other fluids after couple weeks all was back to normal. With no drugs.
After about 12 yrs got sent to hosp after liver function test result scared my GP.
Consultant did soopa doopa MRI pics close ups of liver ,lots of ducts totally missing running on about 50%. End of my story really.
No treatment or drugs . Diet ? No nasty fats,no alcohol (for a long time), only pure butter no make believe butter, little cheese,eat loads fruit and veg, . I’m still here lol had one attack in last 3.5 yrs. Went to bed for 24/36 hrs drank loads of tea ignored colour of stuff going down toilet and after 7 days carried on normal service resumed.
No drugs,no docs, not to be taken as advice just a personal thing here in UK.
Olivera - your son’s late August results are interesting as his elevated ALT tracks fairly evenly with elevated ALKP. My results prior to vanco were similar and, while they strongly suspected AIH overlap, my antibodies were negative and they could never find any evidence of this on biopsy.
Many studies suggest that getting liver function values down near normal, specifically ALKP and GGT, significantly lowers PSC patient odds of reaching an end point (generally bad things). I think the goal is to work with your hepatologist in trying to get your son’s values and symptoms normal and stable. If your son’s numbers are still elevated on 250mg/day of vanco, an option is to talk with your doctor about whether an increased dose is warranted. Typically (Mayo, Stanford), patients start around 1500mg/day and work down slowly over time.
PSC is typically diagnosed by MRCP or ERCP. MRCP has improved substantially over the years and has the benefit of being noninvasive, but I believe that ERCP still has better fidelity to spot PSC. If PSC can be seen on this imaging, it is considered the normal, large-duct version. This could involve ducts that are extrahepatic (big ducts outside the liver), intrahepatic (smaller ducts inside the liver), or both. If PSC is not seen on imaging, but all the other clues are there (symptoms, liver function tests), a liver biopsy may find it in the ducts in the liver that are too small to be seen on MRCP or ERCP imaging. This is considered small-duct PSC and diagnosis can be difficult as the biopsy can quite easily miss areas of disease involvement.
My speculation is that your son is diagnosed with large-duct PSC affecting the smaller, yet still visible on ERCP ducts in the liver. This is a really good question for your hepatologist.
Hi JTB .
I thank you for taking the time and responding back to me .
From what I understand is the small ducts PSC that’s is exactly how the doctor wrote it .
Hi JTB.
Thank you for responding back to me so much. This has been so frustrating to find out what has been happening with my son since November of last year.
We finally got the diagnosis of the small ducts PSC after 3 ERCP,S and 3 MRCP and liver biopsy. Which all of it was normal.
