My son's story

I was told a couple of weeks ago my son may have PSC or AIH with PSC overlap. I'm not sure I have accepted it yet and not sure what I am looking for here other than support and/or maybe help with understanding. Or maybe getting people's thoughts on if this can truly be PSC.

To give you a little history, my son just turned 14 years old. Up till now has been a healthy kid (no history of health problems), athletic and participates in numerous sports (football, track, hockey). His dream was to join the Naval Academy and we have spent a lot of time getting him set up for this. But on April 5th of this year, our lives took a turn that I never expected.

On April 5th, my son and his two brothers all came down with some sort of stomach bug. They all had nausea and vomiting. The two brothers recovered after 48 hours, but on the third day my son wasn't getting better and complained of pain in his right upper quadrant. After a doctor visit, it was noted he had 102.5 fever and his eyes had a hint of yellow in them... so we were sent to the ER. There it was determined his liver enzymes were slightly elevated (still under a 100) and his Bilirubin was 5. He had also developed a rash over his entire body and his liver was slightly inflamed. They sent us home and said this was more than likely some sort of viral infection that had infected his liver and sent us home pending a bunch of viral tests.

Everything came back negative, but his labs continued to improve over the next week. His liver values returned to normal, but the bilirubin dipped down and then came back to 5 again during this time. The doctors said they weren't concerned and told me it may take a bit for his body to rid itself of the bilirubin. The doctors agreed we were ok to take him on a planned family vacation.

When we left for our trip, I thought my son was looking a little more jaundiced so I called his pediatrician. She assured me his labs were looking good, but if I was concerned to take him to the local ER. Within a day of the phone call, my son went downhill quickly. He started looking very jaundiced, nausea and vomiting, and pain so intense he couldn't walk. I had to get him a wheel chair. I took him to the ER and his liver enzymes were around a 100 and his bilirubin was up to 7. They did an ultrasound and MRCP. Everything looked normal, but it was noted there was sludge in the gallbladder. The ER doctor suggested we may have to remove his gallbladder. I didn't want to have the surgery done there and asked if he was cleared to fly home, which he was.

Once we got home, I was put in touch with a GI Specialist near my house. She ordered a ton of blood work but seemed to be still thinking this was viral and took a very casual approach to our situation. At this point my son had lost 15 lbs in 2 weeks since he was unable to eat, so I went looking for another doctor.

I found the second doctor at Children's National who I have been very happy with and she was much more aggressive in her approach. She admitted him, had a biopsy performed and a team of doctors re-review the other's hospital MRCP scan. And this is where we stand: MRCP is negative for any type of abnormalities. The only thing that was noted was sludge in the gallbladder. His blood work came back negative for everything they tested for to include pANCA and the other Autoimmune markers. IgG was elevated. His Alk Phos has always been normal, but GGT elevated. Liver Enzymes are continuing to climb but still in the 100s. The Bilirubin had climbed to 12. The liver biopsy did show "mild bile duct injury, and periportal fibrosis, with mild inflammatory activity most consistent scelorising cholangitis." They discharged us with a antibiotics and ursidiol.

Since he started the medications, he has improved greatly. We returned to his doctor after four days of being on the medication and she decided to add steroids to see if helped clear up the jaundice quicker and stop the inflammation of his liver. This is where things got interesting. Pre steroids, he went from Bilirubin of 12 to 8 in 4 days, she put him on steroids and in another week he was down to 4 and in another week we are in the normal range. BUT the ALT has continued to rise, we are in the 190s now and AST dropped to 60. The GGT jumped from 200 to 500s and the ALK Phos, while normal, dropped to the lower end of normal. All other blood work is normal. As she said, this is a bit confusing.

I'm confused. It doesn't seem to fit AIH or PSC, but has symptoms of both. The MRCP is normal, but she did say we will do an ERCP in a month to see what things look like. She wants to continue with immuno suppressive treatment.

I took him to Children's Hospital of Philadelphia for a second opinion and that doctor based his opinion on the biopsy report only and said it has to be small duct PSC. Didn't seem to want to take anything else into consideration and basically told me and my son to plan on a liver transplant in the next 10 years.

My son was crushed and it was the first time through all this that I saw him breakdown. Not only are his dreams taken away, but now he is potentially being diagnosed with something that can prohibit from living a normal lifespan. I was upset with the doctor for saying these things in front of my son. I left there angry. Although, I'm not sure if I was angry with the doctor or the diagnosis.

I went back to Children's National and discussed with our doctor what CHOP had to say. She agreed PSC is something that is on the table now for diagnosis, but feels we are still to early into this to know. Everything I have read, it just seems like presentation and labs don't support PSC but I think it is the biopsy that is making PSC a possible diagnosis.

I don't know, just trying to understand.... trying to figure out how to support my son through all this. As his mother I want him to have the best life possible.

I think it is worth checking dr Cox and Oral Vancomycin that has helped many children with psc. This site has also many people who have been using vanco to keep PSC in check.
I understand that your son’s diagnosis is not clear but maybe good idea to call dr Cox and hear his opinion.

Has he seen a Hepatologist yet? You mentioned your GI doctor but GI's are not normally specialist with liver disease. I'm real concerned about the high bilirubin. He may need to have an ERCP procedure done to open up his bile ducts so they can drain. You haven't mentioned any itching problems. Has he been complaining of that yet? With that high a bilirubin I would think that would be a problem. Please go to a major hospital that does liver transplants and ask for an appointment with a hepatologist. They are experts in this field and are tied directly with the transplant team that can evaluate your sons condition more closely. This is not the end of the world for him and hopefully with the right care he can still fulfill those dreams. Hopefully if he does have PSC he will not need a transplant for many, many years down the road. There is much they can do to relieve the symptoms and help things settle down but you need to find a hepatologist that has dealt with PSC if indeed that's what he has. I wish you well, and please let us know if there's anything we can do.


PSC 2011 / Liver Transplant 7-2015

Hi Mark,

Yes his current doctor is a Hepatologist. Since his biopsy he was started on ursodiol, cipro and prednisone. Prednisone was started a week after the other two meds. His Billirubon has went from 12 to 1.5 over the last three weeks and hopefully still trending down. We will check labs next week. The itching didn’t start until he was close to 12 and he tore himself apart. We were using Benadryl and lotions to help. Thankfully the itching is almost gone but still there at night. I’m hoping once the bilirubon is completely out this will pass. He is currently being seen at Childrens NAtional in DC. It is a large hospital that does a lot of transplants. Our doctor assured us we are not ready to start talking about transplant. She said the numbers that tell how well his liver is functioning are good and over all his liver is still preserved.

My question is, if this PSC and his Bilirubin got so high, wouldn’t we have seen something on the MRCP or could this be happen with small duct PSC?

Thankfully he at least is getting his energy back. Not to his old self, but getting there. He does get frustrated but remind him less than a moth ago I was pushing him in a wheel chair due to his fatigue and pain. I just want his labs to normalize so I can breath a bit.

Hi! I don’t have any good advice, but my daughter was diagnosed with AIH/PSC overlap last summer. I believe small duct PSC would not necessarily show up on an mrcp and can cause symptoms as acute as large duct disease, but I am not an expert.

My daughter was never jaundiced, but initially had sky high ast/alt and elevated alk phos, normal bilirubin. They tested her for all the autoimmune markers and all that bloodwork was normal, but liver biopsy showed signs consistent with AIH and small duct PSC. She was only sent to a hepatologist after we tested her for Celiac because of persistent diarrhea and her liver enzymes were found to be high. She did eventually have urq pain, but only after we were well into the testing process.

She takes Ursodiol (for the PSC), azathioprine (for the AIH and inflammatory bowel disease) and Flagyl (for the IBD). Her liver numbers have totally normalized and she is symptom free right now. I know that this disease takes everyone differently, but it can stagnate and move very slowly–i know of someone in their thirties now who is still symptom free who was diagnosed at ten. I hope, if your son does get a firm diagnosis of PSC, that it will be of this sort.

Is AIH still a possibility, or did they rule it out? If it is, what are they doing to treat it?

I’m sorry your son is going through this.

I don't really have any advice, seems like everyone else has covered it. But I am 15 and have had PSC since I was 11, so I have progressed a lot more. So if you or your son wants general support or have any general questions of what is to come, feel free to message me. Best of luck.