My PSC Journey

Diagnosis

About three years ago, I received a diagnosis called Primary Sclerosing Cholangitis (PSC) which is a rare liver disease (affecting 3 out of every 100,000 people in the U.S.) without a known cause or cure.  The disease prevents the bile that’s produced in the liver from properly draining into the small intestine.  Without proper drainage, the bile buildup causes stones and cirrhosis of the liver, which will ultimately require a liver transplant to survive.   Given the serious nature of the condition, I decided to share this information only with my immediate family (obviously, that has now changed).

Cholangitis Attacks

As you might imagine, this news came as quite a shock to me and my family.  But, I was asymptomatic at the time, so it was easy to live in denial for a while.  About 6-9 months later I was on a business retreat in the mountains of Colorado when I suddenly felt very ill.  Instead of participating in the meeting I slept for about 18-20 hours straight.  I had no idea at the time that I was having a cholangitis attack (liver infection) and that my life was in danger without medical attention.  Luckily, my body was able to fight off the infection and I returned home none-the-wiser.  About a year later I was on vacation with my family at the beach and I had another cholangitis attack, this time I found my way to the emergency room and spent a week in the hospital recovering with the help of antibiotics.

Proactive Monitoring & Therapy

I get pictures (MRCP) taken of my liver every so often by my heptologist to track the progression of my disease.  A few months ago the pictures showed what appeared to be a lot of gall stones that had formed behind the strictures in my liver and my doctor was very surprised that I had not experienced more symptoms yet.  He proposed that we insert a drainage tube through my ribs and into my liver that would help to drain out the bile and hopefully a lot of the gall stones.  The tube would be taped to my side and i would come back to the hospital periodically over a period of 6 weeks where they would insert instruments into the tube and try to balloon strictures in my liver and remove gallstones.  

Recent Saga

On January 9th I checked into Emory Radiology for my procedure.  When I came out of anesthesia I knew something was wrong.  The amount of pain I was experiencing far exceeded what the doctors expected.  After a couple of days in the hospital on narcotics I went home.  Only to return 3 days later with uncontrollable pain.  They did an MRI but could not determine the reason for the excessive pain.  I was put on a narcotics cocktail of 3 drugs in an effort to control the pain.  We then installed a 2nd liver drain in my abdomen with the hope that it might be less painful and we might be able to remove the 1st one.  No such luck.  Finally, we switched to plan B and used the drains to help guide an endoscope (tube that goes down your throat) into my primary bile duct and install a stent to improve the flow of bile from the liver into the small intestine.  Once they installed the stent the doctors were able to remove the drains altogether (yippee).  Finally pain free, I was able to go home after a 5 day stay in the hospital.  

Unfortunately, a few days later I developed a fever that progressed over the next 3-4 days until I was told to return to the hospital for treatment of a probable liver infection (a clear risk of the procedure, but not one that you expect to happen).  So I’ve been hospitalized for the 3rd time in three weeks, this time with a cocktail of IV antibiotics to fight the liver infection.  After 5 more days in the hospital it looks like I might be able to finally go home (fever free) with a PICC line installed so that I can administer my own IV antibiotics for the next 4-6 weeks.  I’m writing this from my hospital room right now as I await the doctor’s decision.

The Road Ahead

This will not be the last time I am hospitalized for PSC.  I know this.  But my hope is that we were able to clear away enough gallstones and sludge from my liver and balloon enough strictures during the procedures and the painful time that the drains were in that we have extended the life of my liver.  Also, since I now have a stent inside my primary bile duct we should be able to conduct future therapies endoscopically (down my throat) rather than having to punch a new hole in me.  

PSC is a strange disease.  Some people need a liver transplant within 12 months of diagnosis and others spend 20 years or more with their original liver.  There’s just so much variance that statistics aren’t much help.  So, I will go on about my daily life until the next time that PSC decides that it’s time to remind me of it’s presence and we will deal with the challenges as they come.  

As word of my hospitalization spread I was asked continuously “what can I do to help?”.  And I never knew what to tell them until now.  You can be a hero and become an organ donor online right now, before you close your web browser.  It takes 5 minutes.  There are over 35,000 people waiting for a kidney and over 10,000 people waiting on a liver this very moment.  On average, 18 people die every day waiting for an organ transplant.  Take the 5 minutes now.  Click a mouse, save a life, be a hero.  Your organs aren’t much good to you once you’ve passed, but they are a source of hope for thousands who sit waiting for that miracle phone call “we have your new liver/heart/lung/kidney/etc" : http://organdonor.gov/becomingdonor/stateregistries.html 

The life you save could be mine.