Recent PSC diagnosis for brother

Hello,

I hope everyone is well and safe during these difficult times. My brother, who is 25 years old, was diagnosed with PSC about a month ago. He had never been sick and never had any symptoms until about a month ago when he randomly started turning yellowish. He went to the doctor and they indicated that he has PSC. He then went on to spend 18 days in the hospital undergoing 4-5 ERCPs and several interventional radiology procedures. After about 7-8 procedures (all requiring anesthesia), they were able to insert an external biliary drain. He was then sent home with instructions that he would be back in a month to see if the inflammation had subsided. After about two weeks, he spiked a fever and went in to the ER. Yesterday, they performed another interventional radiology procedure, in which they decided to install a SECOND external biliary drain. He is now being discharged with two drainage bags coming out of his abdomen. I have been reading the posts of various members and am yet to see drainage bags as a form of treatment for PSC. Does anyone know if this is proper protocol? He has been seen by a new doctor every day at the hospital so its hard to obtain quality information from the doctors. Also, because the hospital does not allow visitors, I am stressed about whether they are handling this condition properly. As you can imagine, It has been an extremely difficult month for my family, particularly because Covid-19 means he cannot have any family with him. Any insight, tips, or information is much appreciated. Thank you.

Patrick,
Thanks for your post. I’m sorry to hear of this for your brother. Yes, I have heard of the external biliary drains but that is usually not done except as a last resort, at least here in North Carolina where I was treated. That is usually when they cannot gain the access they need through the common bile duct. I had a major blockage in my left hepatic duct at one point where the specialist could not gain entry through standard ERCP methods and it was discussed at the time that the drainage bags might be a possibility in the future. However, he wanted to wait a few weeks and try again. On the second try he was finally able to gain entry to the blocked duct and provide the needed relief. Your brothers situation evidently was much worse than mine and they weren’t able to get it uncleared.
What do his labs look like? Do you know what his current MELD score is? His bilirubin level?
We are here to help in any way we can.

Regards,
Mark
PSC 2011 / Liver Transplant 2015

Mark,

Thanks so much for the reply.

His total bilirubin is 1.3. His AST is 64, ALT is 103, and his ALP is 280.

I do not know his MELD score but doctor told me that on a 0-4 scale (a 4 being cirrhosis), he is at a 2.

He went home today with both drains. I am not sure what the future entails since we have not been given a long term plan (other than another IR procedure in two weeks).

I appreciate your feedback.

Patrick, those numbers look fairly good. A lot of PSC-ers would love to have them!

I never had a drain, so I do not have any specific advice there.

Is his hospital a transplant center? It would be better if his doc were a hepatologist and not a Gastro. Specialist vs a generalist…

Jeff

Patrick,
I agree with Jeff. Those numbers really are not all that bad. It would be interesting to find out from his doctors why they had to put in the drains. Were the blockages in the ducts that bad that ERCP couldn’t clear them?
And yes, definitely make sure he has a specialist. It’s actually a hepatologist. I think Jeff’s spell check changed it to hematologist. A hepatologist is a specialist in the diagnosis and treatment of liver disease. And preferably that doctor needs to be tied in with a transplant center.
Tell you brother to not let this diagnosis change his plans for his future. This is not an automatic death sentence by any means. And if transplant is in his future, the prospects are very good. Both Jeff and I have had transplants in recent years and life couldn’t be better. Take care and let us know how we can be of further help.

Mark

Thank you Mark and Jeff for the replies. Yes, the doctor indicated that the strictures were of such a nature that he could not safely enter the area. The last IR doctor I spoke to yesterday indicated that he does not see this improving to the point that they can remove the drain (which I found to be a strange comment to make considering he is not a transplant hepatologist). The second biliary drain they inserted has not had any fluid come into the bag - it is just an empty bag, which is very weird/concerning. Also my brother’s throat is very inflamed and he cannot speak from having the tube put in his throat during all these procedures. We hope to see a specialist soon with knowledge and training in PSC. I have had some doctors mention that a transplant could be needed and some say it is too early to have that discussion, which is extremely frustrating. What is crazy is none of the doctors ever communicated the lab results to me or my brother, which had some positive information that would have been nice to hear. I had to manually login to this online portal to see whether his labs were accessible and then surf the web to see whether his numbers fall within normal ranges.

Patrick,
I’m sorry you all are going through this. I do hope you can get in with a specialist soon. You have the right to be receiving better care than this for your brother. You will need to be his advocate during this time and speak up for his needs. One other option if you can’t get an appointment soon with a hepatologist is to go to any large hospital that does liver transplants through the emergency room if need be. They always have a transplant hepatologist on-call 24/7. Hopefully, you will not have to go that route but sometimes we have to use desperate measures to get the care we need. Good luck.

Mark

Hi Patrick,
Your brothers LFT numbers being low can be deceptive for PSC (and is sometimes deceptive to even the Doctors). In 2012, one month prior to my transplant from PSC (and I had days left to live on day of surgery, liver heavily diseased and in complete failure and no hope of cadeveric doner in time) my LFT’s were ALP 198, ALT 51 (normal !!), and AST 72. These numbers in preceding months/years leading up were not enough to MELD score me anything close to a waiting list , hep’s told me to go home (this is the MELD scoring systems dirty little secret, it doesn’t always work for PSC, you may be the one to have to educate and re-educate your brothers health care peeps on that lesser known reality). My life was saved by a live doner - my sibling - who is and will forever be the biggest hero in my life, and my familys. Good luck to you, through his own self advocacy and advocacy from you, including the kind of research you are doing (knowledge is Power) he can get through this. RJM

Hello Patrick,
I have also been diagnosed with PSC, fibrosis stage 2/3 and lft’s only mildly elevated with alk phos of 75. I just want to reiterate what others have mentioned here that ERCP’s are as good as the operator. There is high skill variation among ERCP docs and some are able to do more heroic procedures. I am fortunate to live in Pittsburgh where we have docs like Dr. Andrew Slivka who is recognized around the world for his skills in ERCP. Please see a hepatologist who works well with a skilled interventionalist. I am a nephrologist myself so have some more background but this disease catches you by surprise. I am on oral vancomycin and that seems to keeping things stable for now. The drains may very well be temporary until you find somebody who can get through. Please dont give up. I’m sure others can help you find somebody based on where you live if you post that info.

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