Help! Am scared for my son!

Hello,

My 15 year old son was diagnosed with small duct PSC about a month ago, via biopsy. We just had our fist visit with the hepatologist today and he immediately wanted to put my son on Ursodiol. I did some research on it and I really did not find it very encouraging. I see a high risk of mortality and not really an improvement on the disease itself, only lower liver enzymes. I also read something about Vancomycin in children and I suggested it to the doctor but he was against it.
What do you guys think about these medications? What are your experiences?
I am thinking about taking my son to see at least 2 more doctors before I put him on any medication. What are the best doctors in the USA on this disease? Please, I will do anything to help my son, even if I need to travel to see another doctor. My son is worth more than any amount of money. He is a very sweet boy. Please help me!!!

Lenora,
Hello and welcome to the group. Sorry about your son but realize that in the youth the disease usually progresses slower. Hopefully he will have many years before major intervention is needed. Regarding Ursodiol (or URSO for short). It is a very good medication that I took for over 4 years from diagnosis to transplant. I had no side-effects from taking it. It is NOT a cure for PSC BUT it does improve quality of life for the patient by thinning the bile which in turn allows it to flow better through the strictured ducts. I would not be afraid to allow your son to take it for it will keep his numbers down hopefully for a long time. There may come a day when it doesn’t do that but he hopefully has many years if and when that occurs. I was on 1200 MG a day and I would spread it out and take it in what worked best for my stomach, etc. I was never on Vancomycin but others here on the forum have been and will hopefully chime in.
Please know that we are here for you to help in any way. Allow your son to just live life normally, plan for the future and not let this disease stop him from all his ambitions and goals in life. It will let him know when he needs to rest or when intervention is needed. Just take one day at a time and let him be the young man that he needs to be without mothering him to death with worry over this diagnosis. I wish you and your entire family the very best. Let us know if you need anything.

Mark
PSC 2011 / Liver Transplant 2015

I was a high-dose urso guinea pig back when that was in fashion and I’ve been on oral vanco for the last 4 years or so. I’ve had a lot of success with vanco. Dr. Cox at Stanford is the pioneer of this treatment and would be a good source of additional information or for a reference for a receptive doctor closer to home.

PSC patients are rare birds for most GIs and Heps who rightly dedicate most of their time and energy for the hepatitis and fatty liver patients they see every day (teaching/transplant centers being the exception). It is important that you and your son become subject matter experts of this disease. In many cases the only way your doctor will know about the latest PSC research is if you brief it to him/her.

Leonora,

I’m not going to tell you to relax and not to worry about your son, as a parent myself, i know this is not possible. But know this, this is a disease of glacier pace - especially at the beginning. There are a ton of great resources here who can both answer any questions and do their best to calm your nerves.

You will find the doctor that works best for you, my advice is to keep looking until you are satisfied. To repeat what others have said, find the largest transplant teaching hospital in your area and attach yourself to the hepatologist on the team.

As far as medication, all I can tell you is my own experience. My Hep and I decided long ago not to use Urso, mainly for two reasons. First for the unclear long term results and outcomes and secondly because I had previously had my colon removed and therefore wouldn’t benefit from the potential decrease in colon cancer for those on it. I know nothing about vanco as it has only really become a well known medication in the last year or so and I am towards the later stages of this disease and the benefit appears to be when it’s started early.

My most valuable advice, (in my opinion at least!), is to let your son live basically naive to the disease. He will google want he wants to, when he wants to. Don’t stain these years for him by titling him as “sick”. More than likely he has decades ahead of his where this disease will simply by a minor thought in the back of his mind. I say this from the perspective of being in my mid 30s, being diagnosed in my late teens and having never made a single life decision based on the fact that i was “sick”. This mentally yielded me a career, my high school sweetheart as my wife and two beautiful kids.

And when you are googling - remember many articles are old and outdated or statistics include the whole population of liver diseases.

I know that nothing helps at the moment. We get that.

E

Hi Leonora,

I too have a small duct Psc diagnosis. I’m 43 and have had this for a couple of years. My hep says that urso will do very little apart from maybe keep my lft’s down and therefore she didn’t seem too motivated to put me on it. Personally I would prefer not to take a drug anyway if there is no real benefit. I think urso is more beneficial for later stages of the large duct form of the disease but I will leave that for others on this board to comment on. i don’t know too much about Vanco but have read there has been positive results for children with Psc. I dont know anything about vanco and small duct Psc though.
My advice is to find a hepatologist who you feel comfortable with. It will just click and you will know. I have dealt with 4 different hepatologist all with an active interest in Psc. For me it came down to a combination of experience, enthusiasm, communication (I.e good ‘bedside’ manner in talking about the realities and implications) and knowledge. Doctors who are bad at communicating, even if they are very knowledgable, are the worst for me. That’s when the anxieties kick in after you leave their office!
Even though I do a lot of research,I take everything with a large pinch of salt and I always go by my heps medical opinion.
Also remember that small duct is the ‘better’ variant of this disease, and there is no cancer risk and only a small chance it will become large duct Psc. Plus it is usually a very slow moving disease. I’m not a parent but I totally get what you must be going through. As a parent you will be feeling it as strongly as if you had the Psc yourself, I’m sure. This is very recent for your son and please be reassured that he has many many years ahead of him to live life to the full. I do hope you are able to take some time and find some space to take good care of yourself too!!

Hi Leonora,

I want to add my voice to those who both expressed sympathy with your son and yourself, and at the same time share that this disease can progress very slowly and each case is different. I’m older, but have had the disease since early 2005 and am only now having some troubling symptoms. Urso will not cure the disease, but it does have an effect of helping the bile pass through easily. You’re in no rush to decide whether it is something for your son to take. Get another opinion, find a doctor who you are comfortable with, continue to learn about the disease.
One question for you is, why the biopsy? PSC can be diagnosed in less invasive ways. did your hepatologist recommend that?

Steven

I’m in the process of getting diagnosed what kind of biopsy diagnosed your son ? Liver biopsy or ERCP? Thanks in advance and I pray for slow progression of disease and fast recovery for your son and everyone else of this forum !

Small duct PSC can only be diagnosed by biopsy because the mri scan of the large ducts will usually show them as normal with no beading or narrowing. Sometimes multiple biopsies are required as they can only test the very small sample they collect. This diagnosis is by someone literally looking at the sample through a microscope.

Hello Leonora,
I do have Small Duct PSC, I’m on URSO since it was diagnosed. Sure, Urso doen’t heal, but it seems to keep my liver on a reasonable state. Without URSO everything runs bad quickly, for me this is a good reason to stay on URSO!
I would never ever take Vancomycin for PSC, too risky in my eyes - if you really need it as antibioticum it might not work any more.
so far my personal opinion - make the best of it.
Regards
Rippi