Bill Varner's Transplant Journal

On Monday, May 18th, I completed several days of pre-transplant testing at the Medical College of Virginia (MCV) in Richmond, Virginia. There are essentially two purposes for this testing: (1) to determine if I am physically and mentally fit enough to endure the stresses I’ll undergo during and after the transplant, and (2) to establish baseline measures on a number of procedures so the transplant team will have a basis for identifying changes in my condition after the transplant. As diagnostic tests go, these were pretty routine: colonoscopy, liver ultrasound, nuclear stress test, pulmonary function test, chest x-ray, etc. There were also plenty of “interviews” with, for example, the social worker, financial counselor, psychologist, and transplant coordinator.

Other than some scheduling goof-ups, these tests were pretty uneventful, but I can’t miss the opportunity to share the delightful experience that is the Stress Test. This had been described in advance as a treadmill test, so I arrived with walking shoes, ready to hit the treadmill.  The transplant coordinator kept telling me how tired I was going to be after this test, but I dismissed that under the assumption that I was a little more fit than the typical stress test customer. This assumption was….what’s the word….uh, wrong.

During the typical stress test, the patient walks briskly and/or runs on a treadmill until their pulse reaches a predetermined rate. A nuclear dye is injected in order to illuminate the vessels of the heart which are scanned before and after exercise. Simple enough.

Complicating matters just slightly is the fact that the hepatology team prefers to have the stress “chemically induced” rather than created via some sort of strenuous effort like a brisk walk. I didn’t appreciate the impact that would have on the remainder of the day, but it was significant. It also explains why I give the Truth in Advertising award to the the accurately named Stress Test. It’s not a “Coronary Artery Test” or a “Treadmill Test”…it’s a Stress Test.

That should have told me something.

So, here’s the scene: I’m on a stretcher, a nurse is intently reviewing an electrocardiogram to my left, another nurse is on my right flicking a syringe of persantine (the chemical stressor), another nurse is by the door of the exam room (in case I make a run for it?), and a nurse practitioner is at my feet, armed with an antidote to the persantine in case things go poorly. They explain to me that after the persantine is administered, all of my blood vessels (let’s review…they said “ALL”) would dilate, causing my heart to beat faster, essentially creating an effect similar to vigorous exercise, especially if during exercise you are accustomed to your heart leaping from your chest and your eyeballs spinning in their sockets, in which case this is EXACTLY like exercise.  The nurse practitioner further adds that I might experience a headache, nausea, dizziness, and tightening across my chest.  She asks me to let them know if any of the symptoms become unbearable because, as she explains, “We don’t want your heart stopping.” I assured her I was onboard with the plan, especially the “heart-not-stopping part.” I was then instructed to “breathe normally.” Right.

So in goes the persantine injection, and everyone in the room immediately looks at their watches. Now, to me it seemed awfully premature to be recording the time of death but, given the description of the various risks and side effects that I might experience, not beyond the realm of possibility. As it turns out, the persantine’s effectiveness peaks at 2 minutes and 45 seconds after the injection, so they were all bracing for the precise moment when my chest might detonate. I have to admit that the initial impact of the injection was not altogether unpleasant, and I just felt a little flush. After that, though, the whoosh of blood through your body becomes increasingly louder, to the point that I felt as though it must have been audible to those around me. I thought a little chit-chat might help lighten the mood, but immediately discovered that “casual” was not a mood I could evoke with clinched fists and tightened jaw. I had overheard the nurse practitioner talking about her dog earlier, so I asked, “What kind of dog do you have?” At least that was the question that I expected to ask, but what came out of my mouth was a series of high-pitched squeaks and hisses that must have resembled panic more than curiousity, causing the nurse practitioner to lean forward intently in her chair. I quickly replayed the odd sound in my head, hoping to confirm that I hadn’t said anything that might have been misinterpreted as, “Please jab the antidote into my sternum.” Scrambling for a non-verbal solution, I gave everyone a quick, strained smile, and they each resumed their at-ease postures.

Well, I survived the test, apparently with flying colors, and was escorted back to the waiting area to enjoy my spent heart and nuclear headache. The nurse who brought me out suggested that I get a coke since that would help reverse the symptoms and the discomfort. One might expect that since it was common knowledge that a caffeinated drink would help expedite the recovery, the staff might have an ample supply of these on hand. One would only expect that if one hadn’t been to MCV before; these guys know as much about customer service as the Governor of South Carolina knows about hiking the Appalachian Trail.

As a quick aside, my favorite among MCV’s recently implemented customer service efforts is the WalMart-inspired “greeters” posted at the main hospital entrances.  I’m sure this idea looked great on paper, and I’ve actually seen it implemented effectively at other organizations, but MCV’s effort is enough to make you laugh. The greeters look as though they’ve been assigned to their post as a form of disciplinary action against them, or because they lost a bet. Most of them have a remarkable ability to avoid engaging patients and family members in any way possible, even if you stand 10 feet in front of them and look lost, which I have done in an effort to see if they could be baited into action. For the record, they can’t.

Anyway, back to my coke. At this point, it helps to understand that although the procedure is over, the blood vessel dilation is not, and that seems have a serious impact on a variety of functions: hearing, vision, stamina, balance, patience, speech, reasoning, etc. Essentially, any function requiring oxygen is impaired. So, while finding a coke sounds like an easily manageable task, to me it was an Epic Journey. I stumbled from the waiting room, with each step creating mini seismic events through my head, only to discover that three of the four elevators to the first floor were out-of-order, and the fourth was behind a long line of grim-faced patients and their families. It seemed noteworthy to me that no one else was as agitated as I was by the delayed trip to the first floor, so I opted for the stairs. Anyone who knows me well knows that I typically take the stairs if: (a) the building is on fire, or (b) I have been shoved. But in this case it was my only realistic alternative to the elevator. I finally made it to the ground level, found the machine (“Exact Change Only” of course…started feeling like I was being tested) and sat wild-eyed on the floor next to a trash can (no joke) restoring my blood vessels to their appropriately constricted state, one sip-at-a-time. Within a matter of mere minutes (if by “mere” you mean “just over 300″) I felt fine, fully restored.

This is a test I hope to not soon repeat. In fact, fear of a repeat performance of the Stress Test is enough to make me promise to choose from the Heart Healthy items on the menu next time I go out to eat.

I’ve always found it puzzling that, throughout the course of my diagnosis and treatment for PSC, not one healthcare provider has ever mentioned the potential impact that nutrition can have on the disease. Not a single primary care physician, gastroenterologist, hepatologist, nurse, etc. has even uttered a word about it. On those occasions when I have brought up the topic and raised questions, typically with my hepatologist, he has been quick to dismiss the potential for nutrition to have any meaningful impact on the disease, its progression, my prognosis, or my symptoms. Don’t get me wrong, I’m not operating under the illusion that I can eliminate my PSC by eating better. My eyes are wide open to the fact that I’m stuck with PSC and that a transplant is the only real “treatment” option available, and even that, technically, does not eliminate the disease. However, I’m also aware that everything that I eat during the course of a day has some impact on my liver. It also seems obvious to me that some of the things I eat may have a detrimental effect on my liver, other things may have no effect whatsoever, and some things may actually have a positive effect.

That’s why I have begun making more of a nuisance of myself in pushing for some expert guidance on a nutrition plan tailor-made for my individual circumstances, and until recently my questions have fallen on deaf ears. So if the best clinical expertise that I can access seems underwhelmed by the value of nutrition, shouldn’t I just accept their feedback and move along? My answer to that is a firm “No” because I believe this is a profound blind spot among a large portion of physicians and other clinicians practicing in hospitals and academic medical centers today. To be sure, there are exceptions to this blind spot, so I’m talking about tendencies and trends rather than universal, unanimous truths. I should also acknowledge that I am part of the health care industry, not as a clinician, but working as a VP for strategic planning and communications in a Virginia-based health system. So, having worked in hospitals for the past 20 years, I might have an insight into the inner workings of the industry, but I also have to admit that I’m directing some blame at an industry I’m part of. Fair enough.

Here’s what I’ve seen from the inside. For most physicians, nutrition is a tool they pull from the toolbox for one reason only: obesity. This shouldn’t be a real surprise since obesity has become one of the most serious public health issues of the 21st century. The latest statistics suggest that about one-third of Americans are obese, having a Body Mass Index (BMI) of 30 or higher. Consequently, physicians have grown accustomed to obesity being the most common nutritional issue they face. As a result, nutrition has become synonymous with weight loss and counting calories, and that severely underappreciates its far broader potential.

By the way, according to the Centers for Disease Control, as we become more obese, the risk for the following conditions increases:

• Coronary heart disease
• Type 2 diabetes
• Cancers (endometrial, breast, and colon)
• Hypertension (high blood pressure)
• Dyslipidemia (for example, high total cholesterol or high levels of triglycerides)
• Stroke
• Liver and Gallbladder disease
• Sleep apnea and respiratory problems
• Osteoarthritis (a degeneration of cartilage and its underlying bone within a joint)
• Gynecological problems (abnormal menses, infertility)

If the risk of “liver and gallbladders diseases” increases with weight gain, it seems reasonable to expect that it’s possible to reduce (but not eliminate) the risk of further damage to our livers by dropping a few pounds — especially anyone whose BMI is in the “obese” range. So, even if obesity were the only health issue we could tackle by focusing on a more rigorous nutrition plan, this would be quite an accomplishment that would take a significant burden off our hearts and improve the odds of a favorable surgical outcome.

But I think we should expect far more from our physicians, and even from our health insurers when it comes to providing advice and guidance around nutrition. I think we should expect a plan that helps us go beyond losing weight, and that broadens our focus on eating healthful foods that strengthen us, foods that don’t overburden our livers, and foods that may even help prolong our lives. I find it ironic and disappointing that my health insurer will gladly cover $600-700/month to pay for my dosage of Ursodiol, which even my hepatologist admits its probably having no impact at all on the course of the disease other than making my lab results look better, but they won’t pay $100-150 for a single visit to a nutritionist who can probably have a meaningful impact on my health (by the way, my health plan will pay for a referral to a nutritionist only for newly diagnosed diabetes patients).

Here’s the last thing I’ve got to say about this. After seven years of visits to a hepatologist, I was recently able to get a referral to a nutritionist. I’m not sure if my hepatologist finally had an epiphany, or if I just wore him down. Either way, my insurer still does not view the visit as “medically necessary” so I’ll be happily paying for the visit out of my own pocket. I am already very disciplined and informed about what I eat, and I don’t need to lose any weight, but I can tell you that I learned quite a lot in my hour-long visit with the nutritionist. I also have a renewed since of control, which can be priceless for those of us with PSC since there’s so little we can do to affect the trajectory of our illness. Nope, I’m not cured. And yes, I still need a transplant. But on the day I receive my new liver, I’ll be as healthy and strong as I possibly can be, and I will have stacked the deck in my favor to have as positive an outcome as possible.

Dr. Luketic finally called me back with the results of my lab work from March 11th. Rather than just leaving a message on my cell phone telling me exactly what the labs looked like, which he has frequently done in the past, he asked me to give him a call back. This alone put my anxiety level on “Standby” status. So I called him back immediately and the news wasn’t what I wanted to hear. My liver enzymes were elevated considerably. Now, when I hear something like that, a part of me actually experiences some relief, since it at least offers some explanation for why I’ve felt so miserable lately. But from a longer term perspective this was alarming news, since for the last several years my lab results have been just marginally out of the normal range, and haven’t always seemed to be correlated with the ups and downs of how I’ve been feeling. This lab work definitely marks a turn in my condition. Dr. Luketic went on to say that my liver currently has no dominant stricture (which it has had in the past) and that the elevated labs appear to be the result of the more diffuse strictures that are present throughout. This is discouraging since the dominant strictures have proven themselves to be manageable with endoscopically placed stents, while the diffuse strictures will continue to cause deterioration of my liver because they are basically “untreatable” — a word no one wants in their medical vocabulary. If I had been in the mood to play “Let’s Be Careful With Our Adjectives” with Dr. Luketic, I would have suggested “unrepairable” over “untreatable” since my liver, just like a broken window pane, can’t be “repaired” but it can definitely be “replaced.”

Dr. Luketic seemed to know where my mind was already, and switched gears to the topic of the transplant. This part requires a brief education on the concept of MELD scores. MELD stands for “Model for End-Stage Liver Disease” and is a quick measure for determining the degree of a liver patient’s illness. This becomes a critical measure because it can help determine the degree of urgency for being formally placed on a transplant list and the degree of priority for being allocated a donor liver. Here’s what Dr. Luketic explained about each level of the MELD score:

During my appointment in January 2009, I was at a 10. During my visit a week ago, my score had elevated to a 12. Ordinarily, although this is not positive news, it would not be cause for significant alarm because I’m still at a relatively low score and not nearly as ill as some of the people with whom I share waiting room space during our quarterly visits to the Hume-Lee Transplant Center in Richmond. But Dr. Luketic had two things on his mind. First, he was concerned that the score had changed two points in so short a period. This could potentially be a new trajectory in the progression of my condition. Second, he acknowledged that his judgment was probably being unduly influenced by a patient he had just seen whose score was at 12 three months ago, and who just today is at 30. In other words, he wasn’t feeling comfortable taking any chances with a “Wait and See” approach. Neither was I, so we both quickly agreed that although I was not yet at the threshhold level (a 15) for the transplant evaluation process, we should go ahead and initiate that process. If things continue to decline as rapidly as they have over the past month, I’ll already have the evaluation behind me and can be listed right away. If things slow down, it would be necessary to update some of the tests from time to time. Without hesitation, I agreed that I wanted to go through the full pre-transplant workup ASAP.

Dr. Luketic then said, “I know this is a lot to take in, and it’s a very serious decision to be making late on a Friday afternoon when you just learned of this five minutes ago, so if you want to take some time to think about it, this can wait a few days.” It wasn’t until then that I realized a couple things. First, for the past two years since my mom’s transplant, I have always had the perspective that I wanted to be transplanted as soon as possible. Other than the liver condition, I’m in good shape, and it seems obvious to me that a relatively healthy body will have a better transplant outcome than one nearer to death. That philosophy has resulted in my being eager to move things along as quickly as possible, so when I was asked about the possibility of doing the pre-transplant workup and getting listed, I heard my mouth say “yes” since it had rehearsed its lines well and delivered them on cue when we moved from dress rehearsal to live performance. The rest of me wasn’t so sure, but decided to stand behind my mouth’s boldness. After all, there appeared to be no realistic alternative.

The other thing that I was just realizing, and this is a lesson that should be firmly committed to memory in order to avoid repeating, is that a phone call of this nature should not take place on a cell phone while sitting alone in the car parked in the driveway. I had returned Dr. Luketic’s call on the drive home from work, and suddenly found myself idling in the driveway, staring at the back yard. Life-altering news deserves an audience, or at least a safety net, and all of mine were in the house, barely visible through the glare on the kitchen window, so I hung up the phone and took the twenty steps to the door — my first steps as someone acknowledged to be dangerously ill. My first steps as someone who could no longer delude himself about the possibility of avoiding a transplant. Ah, a little of the molasses legs with some tunnel vision thrown in for added effect. So, through the kitchen door I go, and I’m immediately met with, “Dad, we’re going to the movie. Can I PLEEEAASSE get a treat?” Nothing like an entry from a six-year-old’s “List of Life’s Biggest Concerns” to help you downshift into the world of more mundane matters. It also helped me realize that it was a fine time to have a treat.

So we did.

December 2008 through January 2009 proved to be very difficult and brought some new additions to my existing symptom portfolio which already includes itching and fatigue. I’ve now turned a nice shade of yellow — not exactly the “canary” variety, but more of a tasteful light pastel that’s most noticeable in what used to be the whites of my eyes. Only my vanity suffers on this one, but the yellowness is typically joined by nausea which has all but eliminated my appetite. As a result, I lost even a few more pounds over the holidays.

The other thing I’m now dealing with is insomnia. I had already experienced occasional bouts of something like this, but in retrospect that wasn’t really insomnia, it was my sleep being temporarily disrupted by fierce itching. My insomnia seems altogether different in that it is not always caused by itching and is far more persistent. I’ll go to sleep by 11pm or midnight, and within an hour-and-a-half, I’m wide awake.

It may be important at this point to explain that I love sleep. I have historically been able to sustain myself on about 5-6 hours of sleep each night, so I’ve never been the type that needs 8-10 hours to feel rested, but I still LOVE sleep. I’m also a firm believer in naps, although I rarely get the opportunity to indulge in them. So sincere is my belief in naps that I’d like to see a sanctioned 20-minute Power Nap each afternoon during the work day; I’m convinced it would result in another 1-2 hours of productive time (by the way, I’ve never proposed this at work for fear that it would be interpreted as laziness, but I support the concept 100%).

Back to the insomnia, and now this self-professed sleep lover is facing nights of about 1.5 – 2.5 hours of sleep. I can do this for a few nights. I can even go a week. But shortly thereafter I have found that my sanity begins to erode. During my first prolonged struggles with insomnia, I made a concerted effort to stay as positive as I could and saw the additional awake hours as productive time. I’d come downstairs each morning at about 2am and get caught up on work, read the news online, do some miscellaneous surfing, watch a movie, or read a book. Prior to this, I had always thought insomnia meant that you were awake because you weren’t sleepy. The truth is far more insidious — my body is completely exhausted and wants desperately to go to sleep, but I just can’t make it happen. So I shuffle through the following day, zombified, nauseated and itching like I’m on fire.

During this time, I felt like I had entered a new chapter in the progression of my PSC, and one that I didn’t care for. My doctors prescribed something for the nausea, moved me from cholestyramine to rifampin for the itching, and put me on Ambien for the sleep. Unfortunately, nothing really touched the nausea, and the Ambien would knock me out within 15 minutes, but by 2am I was up again. The latter was corrected by switching me to Ambien-CR which has proven very effective, although I intensely dislike needing to take something every night just to sleep.

By January 2009, I was still feeling pretty miserable and my hepatologist was sure I needed an ERCP to open the common bile duct back open. I’ve never looked forward to an ERCP as much as this one, so you can imagine my disappointment upon waking from the procedure to learn that they were unable to stent me since the common bile duct, and the ducts that branch immediately from it were wide open. A few weeks later I had an MRI to determine if there was a mass causing a blockage somewhere within my liver. I already have a golfball-sized mass that was discovered there two years ago, so I wondered if it had grown. The MRI determined that the mass had not grown, and that my deteriorating condition was not the result of a dominant stricture, but rather diffuse strictures all throughout the microscopic ducts that cannot be treated endoscopically.

This was pretty unwelcome news since it now puts me in a stage of my disease that has no real treatment option other than managing the symptoms. Fortunately, within a week or two of the ERCP, my symptoms started to subside somewhat. The nausea comes and goes, as does the itching, so it’s obvious to me that I was having some sort of acute attack in December 2008 and January 2009. Ultimately my hepatologist confirmed that this had all likely been a bout of cholangitis, and that I could expect to experience similar events more frequently in my future.

The majority of 2006 and 2007 were focused on managing the few symptoms I was having, particularly the itching. During 2007, I began having stents endoscopically placed in the common bile duct of my liver. Each time I had a stent placed, my doc would also take the time to sweep out any stray bile stones. Almost without exception these provided considerable, though short-term, relief.

In the beginning I had more than a little anxiety about the procedure. To be honest, anything that starts with “We’ll be placing a tube down your throat” tends to make me uneasy. But I’ve learned to view the stents as a good thing since their impact on my symptoms has been dramatic. The downside of the stents is that they have to be replaced every three months. Of the numerous stents that I had placed, I had complications only twice. One stent somehow slipped out of place a few weeks after being inserted, requiring an additional procedure to return it to its proper location. On another occasion, I ran a fever (103.7) and had to be hospitalized overnight. This was definitely not pancreatitis, but was very likely caused by the manipulation of the liver during the procedure.

In late 2007, I had a stent removed and did not replace it with a new one. This was something that my doc had attempted earlier in the year, since the stents can often cause the common bile duct to remain open on its own. The first time this didn’t work, but the second time it did, and I managed to enjoy most of 2008 stent-free. My symptoms had subsided for the most part, and I learned that I could easily tolerate two or three nights of itching a week without needing to return for a stent. I was also beginning to experience some discomfort (the classic “upper right quadrant” discomfort associated with PSC) but this too was only uncomfortable and not debilitating.

It’s possible to be asymptomatic with PSC for a very long time, and that was the case with me. I was initially diagnosed on September 8th, 2000, but it is very likely that I had the disease at least two years prior to that. For years I was symptom free and able to feel rather smug and complacent about my condition. I considered a transplant to be only a distant possibility. I took very good care of myself and didn’t do anything that might make my condition worse, but in general PSC was a background issue and little more than a footnote to my day-to-day life. 

That all started to change during the Christmas holidays in 2005. For two or three nights between Christmas and New Years, I was bothered by bouts of itching. At this stage, the itching was relatively minor but nonetheless troubling. I tentatively dismissed it as little more than dermatitis from wintertime dry skin. In the back of my mind, though, I knew that this itching felt somehow different. I also knew that itching is a classic symptom of PSC in many people (most eventually experience some combination of jaundice, fatigue, itching, abdominal swelling, and pain). What I didn’t know was that these bouts of itching were child’s play compared to what I would soon experience.In March of 2006, the hammer came down…hard.

During a meeting at work, the itching struck. I could barely sit still, and focusing was nearly impossible since my attention was consumed with my discomfort. I pulled a close friend aside immediately after the meeting and told him what was going on. He helped me get my head back on straight long enough to call my physician and describe my condition. I could tell by my doctor’s reaction that this was not good news, but he proposed some quick bloodwork to rule out any other possible causes for the itching. By the next day, he had enough evidence from the bloodwork (i.e., elevated liver enzymes) to suggest that I was having an acute issue, almost certainly related to the liver, but still of unknown origin. He proposed an MRI which I would have to wait 10 days for.

Now, it’s important to understand here that this is not your run-of-the-mill itching. This isn’t a bad bout of poison ivy. This isn’t dermatitis. This is mind-numbing, debilitating, painful itching. The medical term for it is pruritus. Pruritus can actually disable its victim, and make working difficult if not impossible. In many instances, those with pruritus injure themselves by scratching, to no avail, and will frequently develop wounds as a result of their creative efforts to find relief. At its worst, intractable itching has even been known to cause the occasional suicide. While I won’t be going there, I can certainly understand why this might drive someone to such extreme measures. This itching is alive, vibrant, electrified, and completely inescapable. When I’m experiencing it, I usually have a significant sense of claustrophobia because the itching seems to close in on you and doesn’t give you room to breathe, think or relax.

So, to say that my 10-day wait for the MRI was long is an understatement. I suffered mightily. I could usually get myself to sleep by loading up on Benadryl, which can take some of the edge off the itching and, in my case, knocks me out. But I was generally up again by within less than an hour and frequently could not go back to sleep. During this time I got anywhere from 45-90 minutes of sleep each night. QR29HZ8TK5Y6

I was diagnosed with Primary Sclerosing Cholangitis (PSC) on September 8, 2000, at the age of 35. Primary sclerosing cholangitis is a disease in which the bile ducts inside and outside the liver become narrowed causing restriction of the flow of bile through the liver. This results in inflammation and scarring of the liver, and ultimately damages the liver cells. It’s not good, and while there is no cure, the disease can be succesfully “treated” via a liver transplant in many cases.

Like many people diagnosed with PSC, I was not originally experiencing symptoms of any kind. In fact, I was feeling fine. I had piqued the curiosity of my family physician because I had recently been declined for life insurance coverage following peculiar results on the standard screening bloodwork. Specifically, the insurance company believed that I was a chronic drinker since several of my liver enzymes were well outside of normal ranges. I’ll never forget the very condescending and patronizing call from the insurance company representative asking about my “drinking habits.”

• THEM: “So, how many drinks DO you have every day?”
• ME: “Well, none really.”
• THEM: “Uh huh…is it possible you are forgetting how many? Have you ever had long periods of time you can’t account for, or have you ever blacked out?”
• ME: “What? Blackouts?!? No, I don’t drink.”
• THEM: “Uh huh…maybe you should talk to someone. I can refer you to a counselor.”
• ME: “I don’t think I need a counselor. I just want insurance.”
• THEM: “Uh huh.”

Let’s just say I didn’t get insurance then and I only have group insurance now through my employer…and I still don’t drink. At this point, my physician still didn’t know what was wrong, but he knew something wasn’t right. His initial diagnosis was that I must have gallstones, but an ultrasound quickly ruled that out. He then repeated the bloodwork to validate the results, but that didn’t provide any new information either.

Well over a year later my mom was diagnosed with PSC, and when I informed my physician of this, the puzzle pieces started to come together. While PSC is generally not believed to be hereditary, my doctor recognized that my lab results were consistent with PSC and ordered an ERCP procedure. The ERCP is a delightful experience that involves running a fiber-optic tube into your mouth, through your stomach, into the intestines and up into the liver to illuminate the ducts. My results were available immediately since the ERCP displayed the classic signs of PSC: where a healthy common bile duct looks like a straw, mine looked like a “string of pearls” with constrictions every few millimeters.

I would later learn that several of the features of my experience up to this point were not uncommon to those diagnosed with PSC or other chronic liver conditions:

1. I was relatively young.
2. I felt fine.
3. The first sign of “trouble” was strange lab work.
4. I was inaccurately diagnosed or “un-diagnosed” for a lengthy period.
5. An ERCP offered the only definitive diagnosis.