PBC - The Nature of the Beast

Around the time my liver disease became life-threatening, I searched the Internet for blogs by people who had gone through similar experiences.  And lived to tell the tale.  I also was looking for plain talk about technical stuff.  I was unable to find what I wanted, and vowed there and then that I would someday write a blog containing answers to the questions swirling around in my head.

Sorry - can’t do that.  There are few answers.  All I can provide is what I learned through my personal experience, which will probably differ wildly from what others encounter.

In the year 2000, I had gone to my doctor to get myself checked out.  I’d just turned 50 and thought maybe I should start taking better care of myself.

A short while after running some blood tests, my doc called me at home.  She said my liver enzyme numbers were unusually high, and she wanted to send me to a hepatologist.  Besides a rush of fear, I was immediately sure that my long-held love of partying had finally caught up with me.  Like many people, I assumed that liver disease was all about alcohol, and I was now branded as a lush.  I was mortified that I’d done this to myself, and was sure that all my friends, co-workers, bosses would shun me.

After more blood tests and a liver biopsy, I was diagnosed with Primary Biliary Cirrhosis (PBC).  To my relief (from an ego standpoint), I leaned that alcohol does not cause this illness.  PBC is an autoimmune disease that appears to be genetic.  It is considerably more common in women (by a ratio of 9 to 1), and their offspring often have the condition.  Female siblings generally share the disorder.  We are born with it, but it often doesn’t show up in blood tests until much later in life.  All of my results were normal up until the time they just weren’t.  After much research, a credible cause has not been determined.  Nor has a cure.

This condition slowly destroys the bile ducts, which are a whole network of various sized ducts branching through the liver, ultimately delivering bile to the intestine.  When the bile ducts aren’t working as they should, the bile is prevented from being transported to the intestine and enters the bloodstream.  The primary and most common symptom is fatigue.  Over time, the main ingredient in the bile (bilirubin) accumulates in the blood, resulting in jaundice which causes the skin and eyes to become yellow.  Because the bilirubin is deposited in the tissues, severe itchiness occurs.  Urine gets darker, and the stools are paler.

Some patients will develop ascites, which is a build-up of fluid in the abdominal cavity.  Ascites can cause severe swelling of the abdomen, making it necessary to undergo a procedure to drain the fluid.

As the liver continues to decompensate, portal hypertension arises causing gastrointestinal bleeding.  Varices, which are extremely dilated veins, begin to appear in the esophagus.  These can rupture and cause an esophageal bleed, which could be fatal if not treated immediately.  To reduce the risk of bleeds, doctors use endoscopic techniques to “band” the varices, using an actual rubber band to tie off the veins.

As the liver's function becomes more impaired, it's prevented from cleansing toxins that are produced by the digestive process.  These toxins enter the blood stream causing, among other things, a build up of ammonia in the brain.  This is Hepatic Encephalopathy (aka HE), the main symptoms being forgetfulness, confusion, irritability, disorientation.  One of the more interesting indications of HE is a reversal of the sleep cycle, causing the patient to become nocturnal.  This symptom began early on for me, and Wingman became concerned over my strange behavior.  He was actually more irritated than concerned.  The condition made me lose my focus to the extent that I was unable to recognize the fact that I was getting weird.

In February of 2010, Wingman and I went to Culebra, an island just East of Puerto Rico in the Caribbean.  We’d always wanted to go to the islands, and it was a timely blessing for us to have that chance before my health went down the tubes.

We spent time with our friends Grady (Artist in Residence) and Walt.  We went sailing in Walt’s little boat.

It was an amazing journey, and with my fragile immune system, it’s not one I’ll be experiencing again anytime soon.  I couldn’t keep up with the guys as we walked around the island, and they were teasing me for being such a sissy.  Looking back, I believe the PBC fatigue was kicking in big time.  As was the encephalopathy.  I misunderstood three-quarters of everything that was going on, to the considerable annoyance of my companions. Nobody could understand why I'd become so slow-witted.  Least of all myself.

On my birthday of the same year, Wingman arranged several get-togethers for me with various long-time friends.  I was touched by his thoughtfulness, not realizing he could sense which way the wind was blowing.

I’d always wanted a bicycle my size, and that was my gift from Wingman.  Not long after this, my stamina faded to the extent that I haven’t been able to ride since.

When I was first diagnosed in 2000, my hepatologist prescribed a drug called Ursodiol.  It’s been shown to slow the progress of the disease, and it did so for me.  From that time forward until 2011, I experienced very little evidence of the disease, beyond fatigue.  When I began having extremely serious symptoms, my liver enzymes were still showing only mild elevation.  As a result, it took longer to determine the fact that I was actually approaching end-stage liver disease.

The truth is that every PBC patient has a different experience with the disease.  I cannot tell anyone what to expect.  I can only relate how it went down for me.  A number of ER visits with life threatening episodes, undergoing multiple endoscopies, transfusions, and eventually receiving a liver transplant is how it happened for me.

There are those PBC patients who never develop serious symptoms, and who eventually die from an entirely unrelated cause.  I had heard this, and sometime around the mid 90‘s, I asked my doctor about it.  He agreed that this was so, but then told me he felt I’d go into liver failure around 2010 or 2011.  That fucker was right.  I generally don’t use the names of key characters in my blogs, and I won’t in this case either.  But he’s totally my amigo, my advisor, my warmth fix, my consistently intuitive champion.  He’s so supremely self confident that I listen to and take every word he says for the truth.  I didn't feel that way in the beginning - I took his confidence as arrogance.

The rate at which the disease progresses is determined through blood tests.  The results of three specific tests are plugged into a formula, which produces a MELD score (Model for End-Stage Liver Disease).  I need to note at this point, that I never learned the scientific functions of these components of testing.  I was too busy being concerned with the bottom-line number after the “equal” sign.

A number of PBC victims are unable to work or participate in usual activities.  They have frequent hospital admissions due to dangerous bleeding episodes requiring multiple transfusions, and are mentally impaired due to encephalopathy.  Despite these life-threatening incidents, they have a low MELD score, which is associated with a statistically ok survival.
 
This creates a very frustrating situation for both the patient and their care team.  I was one of those patients.  After my second hospitalization for a bleed that put me in the ICU, my MELD score was only six.  Two hospitalizations later, it was up to ten.  When I was placed on the transplant list, I was officially in end-stage liver disease, but my MELD score didn’t agree.  There was doubt that my score would climb quickly enough to qualify me for a donor liver before my old one became inoperative.

Therefore, I was approved at that time for a living donor transplant should I be able to connect with a suitable and altruistic volunteer.  However, the hospital had a no “good samaritan” policy.  In other words, a stranger or even someone I knew only casually would not be accepted.  The donor had to have the same blood type, be under 55, and a family member or long-term, close and trusted friend.  Setting up a website to appeal for a donor, or advertising my plight on Facebook was no longer an option.  Of course, I told my story to absolutely everybody I met.  That included the checker in the grocery store or the shopkeepers in our favorite haunts in Capitol Hill.  I never knew who might overhear and be able to help me.

Out of left field, a prospective donor stepped up in the form of a young student that we met through a neighbor.  Greg captured our hearts when we first met him, and we had grown very close.  He was and still is my hero.  Greg went to get his blood typed, and called me on my birthday to tell me that he was a match.  There were long discussions regarding the risks to his health, the fact that his gall bladder would have to be removed, and that he would miss a semester of school.  We even spoke with his mother, who was passing through town around that time.  She had been a good samaritan donor for a kidney patient, and was supportive of Greg's decision.  She is no doubt pleased with her kindhearted son.

Then, heartache.  Greg was turned down as a donor because of the difference in our weight.  Anybody who weighed 80 pounds more than me couldn’t be accepted.  At first, we just didn’t understand.  We thought that he would only be donating a lobe of his liver, after which it would regenerate to full size.  So, what’s the problem?  It was explained that the lobe he gave me would grow as well, and would become as large as his current liver.  And that would be too large for my tiny chassis.  This was entirely new information to me, and I was stunned that I hadn’t already been told.  Finding a donor under these circumstances was so unlikely, that the minute details just had not yet  been related to me.  Makes perfect sense now.

Greg was devastated and even asked us if we thought it would help for him to lose weight.  Poor bunny.  I told him to keep his battle gear ready, because he would undoubtedly be called upon for other things in the coming months.  Relying on humor, I told him that my biggest disappointment had to do with my hope that his liver might have given me better rhythm or enhanced my dancing skills.  His friendship continues to be our rock and our sanity.  He's been an enormous help in getting us through the really hard days of my recovery.

When I was talking with my doctor later about the MELD dilemma, he mentioned something I’d heard not long after I was first diagnosed.  There has been speculation that Ursodiol can actually “mask” blood test results after long term usage of the drug.  Even though he didn't advise me to stop taking the Urso (I don’t think it was legal for him to suggest it), I stopped anyway.  A short while later, I ended up calling the transplant team to ask whether or not it would matter, and they were like, "Sure, go ahead.  Whatever."  That was at the beginning of July, and here’s what happened next.

These factors are used to calculate the MELD score:
INR - Reference range 0.9 - 1.1

The higher this number, the more likely it is that the patient will experience uncontrolled bleeding.  While I was on the list, my teeth took a dive on me.  This can happen with liver disease.  I needed oral surgery to remove two crumbled molars that had become infected.  My INR was already 1.2, but was still considered to be within the safety zone.
Bilirubin - Reference range  0.0 - 1.3

This is the stuff that enters the bloodstream, turning patients orange, producing unbearable itching, and allowing toxins to enter the brain so that hepatic encephalopathy sets in.  Bilirubin and INR are the two components most responsible for increasing the MELD.
Creatinine - Reference range 0.40 - 1.20

Due to increased toxins in the bloodstream, the kidneys take a beating.  When this level goes up, the kidneys are not happy.  And a higher level wouldn’t make enough difference in the final score to matter.

I had weekly blood tests, and could pull up my results from the University of Colorado Hospital website within a couple of hours.  Besides my MELD score, I also anxiously awaited my hematocrit results.  Anytime that dropped, I was in danger of having another bleed.  Please, no more of those.

Once results appeared on the hospital site, I’d enter the numbers in the MELD Calculator, breathlessly hoping for a higher score.

A bar-graph reflecting patient MELD scores by blood type was maintained on the site.  It was updated several days a week.

Each time my score changed, I’d pull up the latest chart to see where I stood in the liver lottery.  For example, a patient with a score of 16 is in a group of ten people waiting for a liver.  That person is in line behind a group of 12, another group of 7, another group of 3, and so on.

However, the next updated chart will show an entirely different number of folks in each group.  Some will have advanced, due to a higher score, and some will have fallen back, some will be removed from the list for whatever reason, and some will die.

I was continually emailing friends with a screenshot of the latest MELD chart.  Since I had some time on my hands, I attempted to make my reports entertaining.  I had gotten pretty loopy.

If you will recall, my possible living donor was turned down at the beginning of July.  At that time, I had a MELD of 17.   By the 26th of that same month, my score was 22.

Only 15 days later, I chalked up a 27.

September 2, 2011 

Check out that Bilirubin at 14 (1.3 max) - I was so orange
The INR was crazy high at 3.6 (1.1 max)
Creatinine - Irrelevant

These charts generally only go up to around 29, and there I was at 31 with somebody else at 42.  Quite unusual.  For all I know, quitting the Urso had nothing to do with the meteoric rise in my score, so it's not something I'd advise anybody else to do (disclaimer).

So I’m looking at this and thinking, why is my phone not ringing?  Here’s what I learned.

There are many things about having a high score that I didn’t know at the time. Anybody with a MELD that falls within the top ten numbers on the chart could get a call at any time.  Even with a score that's the second highest on the bar graph, the next liver that becomes available must match the recipient in a number of ways.  Besides being the same blood type, the size of the donor is a major factor.

Imagine that your liver is sitting down there just underneath the bottom right of your ribcage.  It’s nestled in its own cozy little crib with no room to spare.  They can hardly take the very next liver that comes along and just drop it right in, especially if the donor is a Sumo wrestler.  That liver could not be placed into the cubby-hole of a tiny waif like me no matter how high I am on the list.  In this case, the transplant team reviews the vitals of the next person and the next and on down until they find a winner, or they have to make some kind of compromise.

Bottom line, the decision lies in the judgment of the surgeons - because those are the dudes who have to make it all work.  Being in the top 10, I could get a call at any time, but having the second highest score didn’t necessarily increase the likelihood.

Eight days later, a Transplant Coordinator called me, and the next day I had transplant surgery.  The date was 9/10/11.

Much of the rest of this story is contained in two other blog posts - “Caregiving Defined” and “Waiting”.

A couple of concerns I had about the surgery never came to pass.  This information might be useful to anybody awaiting a transplant.  Due to several prior hospitalizations with additional outpatient IV treatments in between, my veins had become reluctant to accept the line.  It’s true to this day that I can handle one IV insertion, but when it takes three attempts, I get pretty stressed.

During prior hospitalizations, it was especially difficult when I would have several IV’s going at once.  The lines would get clogged and have to be reinserted, often taking more than one try.  I would get to where I’m like, “hey, that fuckin’ hurts”.  When they took me down to surgery, I was told I’d be getting a tiny painless stick in the back of my hand, and the rest would be done after I was out.  They did not lie.  Nor did they make me count backwards from any number.

The other IV concern I had was taken care of by the insertion of a PICC line (peripherally inserted central catheter) during the surgery.  This is a main line inserted (in my case) next to the armpit and terminating in a large vein in the chest near the heart.  The PICC may have single or multiple lumens (secondary lines joined into the main line).  At the end of the line outside the body, each lumen has a special cap that can be attached to a drip or syringe.  With the PICC, blood draws can be done and medications administered without having to poke more holes.  I think I had about four or five of these lines.

Now here’s the kicker.  I still have PBC, and my immune system will eventually begin attacking the new liver.  It took 50 years for the disease to do enough harm to show up in my liver enzyme tests.  Within that amount of time, my spirit having been graced with a reprieve, will most certainly leave this vessel.  In the interim, I’m allowed to dwell on this earthly plain with humble gratitude for my blessings.

RAM