PBC - Leashing The Beast

I haven’t yet published two posts in a row on the same subject, but I wrote myself into a corner last week.

If I were recently diagnosed with PBC, I'd want to know how the progression of the disease is monitored.  I might not want to know so much about post-transplant complications, but I should relate my experiences in that area.

I don’t know the actual percentages but my esteemed and cheerful surgeon assured me that the set-backs I had were a somewhat common occurrence.  My surgeon is the Professor and Chief of the Division of Transplant Surgery.  He did his transplant fellowship with Thomas E. Starzl, MD, the “Father of Transplantation”.  I was comforted by the fact that here was a man who knew whereof he spoke.

There are four enzyme levels used to gauge the severity of Primary Biliary Cirrhosis (PBC).

• Alkaline Phosphatase (AP) - Reference Range 39-117
• Alanine Aminotransferase (ALT) - Reference Range 0-47
• Aspartate Aminotransferase (AST) - Reference Range 0-47
• Bilirubin - 0.0-1.3  

When I was first diagnosed in October of 2000, the red flag was a high Alk Phos.  I believe it was around 800.  When a patient has elevated liver values, a whole series of tests are run to determine the cause.  In my case, further evaluation determined the presence of AMA (antimitochondrial antibodies) in my system.  This is the determining factor in a diagnosis of PBC.

After I began taking Ursodiol to slow the progress of the disease, my enzyme levels dropped significantly.  Over the years, these numbers remained very much the same, sometimes dropping to near normal.  It wasn’t until the end of 2010 that it all began to climb, but not by much.

By the beginning of April, after four hospitalizations for severe bleeds and being placed on the transplant list, some of my numbers were considerably higher, but my MELD score was only 10.  The absence of lab values that would indicate the demise of my liver originally led my care team to suspect a number of ailments.  At first, it was Irritable Bowel Syndrome, then gluten sensitivity, then later after the first bleed, ulcers were suspected.

Fortunately, on June 30 (my birthday) the tide began to turn with an AP of 1117.

After I was discharged, my numbers continued to improve.  Then, in November, it all went wonky.

I was deemed to be having a “rejection episode”, and was given Solu Medrol infusions (a type of steroid) for three days.  When there was no improvement, I was hospitalized for five days to receive Thymo Globulin infusions.  This medication is derived from rabbit proteins.  Yes, rabbit.  I referred to it as “Rabbit Juice”, which got a few laughts.  This series of treatments basically reboots the immune system.

It was Dad’s first Thanksgiving without Edie (Grace), so he and Wingman spent the holiday at the hospital with me.  Wingman cooked a big dinner and baked homemade bread.  I ordered up some pumpkin pie, and we had us a fine old time.  Considering.

My enzyme levels had become stabilized, and I met with my surgeon on December 1.  We had an optimistic conversation with him, and left his office in a mood approaching cheerfulness.  It was snowing that day, and as we were walking through the parking lot to the truck, I let go of Wingman’s hand for “just for a second”.  My feet flew out from under me, and as most people do, I put out my hands to break my fall.  Of course, I broke my right wrist instead.  Radius and Ulna snapped so that my wrist looked like a dinner fork.  Parking valets ran to my aid, a wheelchair was brought, and I was in the ER within minutes.  A Security person took photos of the bottoms of my shoes, quickly establishing a defense in case I decided to litigate.

I was entirely too discouraged by then to retain legal representation, so I instead settled in for a winter of sulking.  I can assure my readers that this part is not a common hazard for transplant patients.  I began mousing with my left hand and became so accustomed to it that I haven't yet switched back over.

 

The wrist healed remarkably well, and I began working with a personal trainer to get my muscle tone back.  When one lies on their ass for several months, the body generally rebels at movement.  Things were going swimmingly until May.  When I had my first rejection episode in November, I was surprised, because I felt perfectly fine.  This time, however, I knew something was wrong.  I felt like crap.  No energy, nausea, huge fatigue.

Again with the Solu Medrol infusions followed by hospitalization for ten days of Rabbit Juice.
 

By now you’re probably thinking, “WTF?”.   So were we.

Shortly after this humbling experience had wound down, my case was transferred back to my long-time hepatologist.  I was beginning to feel almost perky when I met with him.  The good doctor explained that, prior to the transplant surgery, I had become really sick.  My liver had shut down almost completely, causing considerable harm to the overall functioning of my system.  When this happens, most patients have a difficult recovery, and the rejection episodes are somewhat expected.  This cleared up a lot of the mystery for Wingman and me.

I had thought many times before this that I had reached a turning point, only to be slapped back down again.  But I knew deep in my bones that I was finally on my way this time.  And I am.  Recently, Wingman told me that he’s missed me and he’s glad I’m back.

Me too.  I might even get back on that bicycle before summer is over.

RAM