PSC: The missing chapter in my story

When I was writing down and remembering my journey to diagnosis of UC and learning all about this nasty disease, I left out a part of the story.  UC affects me so up close and personal (like I become fast friends with the bathroom) that sometimes I forget about the other stuff, but people have asked and inquired about the "other" condition that I have wrong with me, so I'll do my best to explain.

Remember when I was first sick, and saw the idiot nurse practitioner in Bella Vista that did not give me very good care, I had some lab work run...he ran a huge lab work up (which was the right decision), but when I came back to his office to follow up a week later, I had some questions about some of the results that were outside of the normal range.  One of these tests was a test on my alkaline phosphatase levels--these are liver enzyme tests that, I guess, are run in a general lab work up.  Here is what my readings looked like:

722 is what MY level was.  50-136 is what the "normal" level should fall between. I asked this NP repeatedly if this level should be something we should be concerned about because (to me) these levels seemed RIDICULOUSLY high and out of control.  (dont they to you???). Plus, I knew how CRAPPY I felt, so I just thought this was data that confirmed just how bad I felt.  But nope, he disregarded it.  Awesome.

Fast forward to my diagnosis of UC and that first year as we tried to get things under control...my [wonderfully fantastic] GI doctor that I actually trust, continued to watch these levels and continued to re-check them with even more blood work (he is EXTREMELY thorough).  While they did drop some, which was good, they never lowered enough for him to feel comfortable with where I was at.  He thought that based on the medications I was taking at the time, that I should not have such elevated liver enzyme levels.  I really thought nothing of this because during that year, I was medicated, I was not having nearly as many flare-ups, and I finally had some "normal" colon operation. Woohoo.

Wrong.  He wanted to pursue this and check it out even further...

The first thing that I had to do was additional blood work (quickly becoming a staple in my monthly routine). Then, I had to do what's called an MRCP. That, my claustrophobic friends, is a fancy way to say a reeeeeeeeealllllllly long MRI. I was strapped down, and I could not be sedated because they had to talk to me through head phones and tell me when to breathe. My breaths had to be even and steady because they were trying to get a picture of the spot where my liver, kidneys, gall bladder, and bile ducts all come together, which is like the size of a dime (so they tell me). It was very important that I breathe correctly and evenly. Ok, I'll do my best. Yikes. I was worn out after that experience. An hour later after being in the closed (TINY) MRI tube, and I was done. Ick. Icky. But, they were checking for bone deficiencies (which can also be a possibility when alkaline phosphatase levels are high). Those tests came back fine. Sooooo...

In the Spring of 2010, on a Friday, he had me do a liver biopsy....and I was awake!!!!!  Let me repeat that...I was awake for the procedure.  Apparently, the liver is tricky to get at, plus (I can't remember the name for it, but it is an organ that can really really bleed out)....so, they use a local anesthetic to numb the pain, they keep you awake, use an ultrasound monitor as a guide as they go in and with the largest needle I have ever seen in my life, they took a biopsy of my liver. 

Afterwards, you are required to lay flat on your back for 3 hours to prevent any bleeding or for anything bad to happen.  Repeat of peeing in a bed pan.  Cool.  At this point, I'm 24 years old, and I've had numerous opportunities to pee in a bed pan.  How many of YOU can say THAT?!?

Honestly, I expected this to be absolutely nothing.  I thought he was just being thorough and checking out every avenue.  I don't know why I believed that idiot nurse practitioner that it was nothing.   I guess I just wanted to believe it was nothing.  Ha.  After all I'd been through, I thought I deserved it to be nothing.  What a horrible way to view things.  We don't deserve anything.  Each new day is a new day by God's grace, so that thinking has since been re-programmed. 

We waited and waited for what seemed like months, but I think it was only days or a week for the results (I guess I was more nervous than I thought to find out)...and it turns out I have what's called primary sclerosing cholangitis (PSC).   (That link will take you to the Mayo Clinic website where they explain it way better than I can, but here's my attempt)...

Basically, not only is there inflammation in my colon due to my UC, but also in the bile ducts leading to my liver.  Because of the chronic inflammation in these ducts, the process of "sclerosis" even in the liver begins to happen which causes the ducts to harden and form scar tissue on them.  According to their website, it is a "progressive disease that leads to liver damage."  Who knows how fast the disease will progress?  Everyone with it is different, but there is a correlation between people with UC that also have PSC because both are auto-immune disorders (and in this case, they both cause inflammation in the body that is irreversible). 

What do they do to treat it? Nothing. There is nothing they can do. All I do is keep tabs on it by doing routine blood work and that nasty MRCP annually! Ugh!

Is it progressing in my liver? Not very quickly...as far as I know! :-). I'm not jaundiced yet. Ha!

What does it mean for my daily life? I try to eat things (like broccoli) that are good for my liver. I also avoid alcohol as much as possible. Since my liver is progressively getting worse, I try not to make things worse, so no boozing for me. Thankfully, it's not a tough sacrifice for me.

OK: Anyone else have this crazy disease? I've heard it's extreeeeeeemely rare. Great! :) I'd love to hear from you if you have it!!!