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Primary Sclerosing Cholangitis (PSC) - Online Support Group

From wilsons to psc/aih/ibd + wilson's

Hi,

father of a 5 year old boy here. Last summer, my son was diagnosed with wilson’s disease after liver biopsy and high urine copper. LFT didn’t improve even after 6+ months of treatment and recently we have started to notice abdomen distention at the end of the day. MRCP revealed biliary dilation and colonoscopy scheduled next week, calprotectin came back high (290) few weeks ago. Diagnosis from doctors is AutoImmune Sclerosing Cholangitis (ASC) because of the aih component. Started treatment with Aza & UCDA and continuing treatment for wilson’s disease. We have been referred to immunology as well to look into immune response in general and some tests are ongoing, potential referral to genetic testing. We have started vsl#3, yakult, fish oil supplements for now along with fruit smoothies. Despite of the diagnosis above, he has no symptoms like fever, reduced energy, itching, diarrhea, abdomen pain. We are somewhat devastated as our 5 year old is diagnosed with 4 rare diseases within a year from being a healthy and energetic kid.

We tried to reach out to Dr.Cox using various ways but unsuccessful. Had a brief chat with Dr.Yinka Davis and looking to schedule an appointment with her in couple of weeks because of logistics. We are going to see if vanco works for my son and if it substitutes only ucda or aza as well.

We have learnt so much in last couple of weeks about PSC from this website and thanks for everyone who shared their experience and knowledge here. Really appreciate it.

I am going to show MRCP results below if someone can spot anything or compare with their reports.

Diffusely irregular and enlarged with contour nodularity. There is mild diffuse smooth intrahepatic biliary dilation up to 3 mm and moderate extrahepatic biliary dilation up to 8 mm. No suspicious hepatic masses. No filling defect in distal CBD.
 
1. Increased diffuse biliary dilation. Etiology unknown. No stone identified. No beaded morphology to suggest PSC.
2. Unchanged nonspecific diffuse liver disease. No mass.
3. New mild splenomegaly.

Any feedback appreciated.

SR,
Thanks for your post. Sorry your son is going through so much at such a young age. From all you have posted it sounds like you are on the right track. I agree with the MRCP report that it doesn’t sound like PSC. There are more tests they can run if necessary to rule out PSC but hopefully an ERCP will not be necessary. With no elevated bilirubin levels and other symptoms, it’s probably not necessary.
I do want to ask since you didn’t mention, has he seen a hepatologist? I would be sure that occurs before coming to any conclusions about ASC. Make sure it’s not just a GI running all these tests without seeing a specialist. Before starting him on any supplements I would really encourage you to run every one of those by the hepatologist. He seems mighty young to be taking fish oil. I went through full blown PSC and transplant and to this day have never taken fish oil. Just saying, please ask the specialist about each and every medication and supplement you give him. If he does have ASC or PSC that will be very important.
Please feel free to reach out to the group with any questions. We wish your son well and hope these things can be resolved for him as soon as possible.

Mark
PSC 2011 / Liver Transplant 2015

Thanks Mark for the feedback. Our doc is both GI & Hepatologist and we are currently under the care of ucsf. Stanford, Mayo clinic, ucsf seems to combine pediatric gi + hepatology programs together. My son had slightly elevated bilirubin levels in late October, prednisone was added to the regimen and from that time bilirubin is under control. Our doc okayed both probiotics (vsl#3) and fish oil.

After colonoscopy next week, we are awaiting to hear more from immunology. Based on that and Dr.Yinka Davis feedback, we are thinking about visiting Mayo Clinic in MN to get a fresh and different perspective. Hopefully we will get some clarity and answers as we are doubtful in the back of the mind for his diagnosis.

Immunology results came but nothing concerning at this time. colonoscopy results came back with active colitis in biopsy but doc’s couldn’t find any inflammation during the procedure. They think he has very early stages of asc/psc with ibd and asked us to stop wilson’s medication now, which we have been giving for the past 6+ months.

LFT didn’t came down with wilson’s treatment earlier but ALP was cut in half (340) with 2 weeks of urso. We are hoping it will go down further more as we continue urso. We are hoping for a quick appointment with Dr.Yinka Davis to confirm these findings and see if vanco is a good fit.