Part 4: Now We Have a Name: Primary Biliary Cirrhosis

Yesterday I began explaining  how I finally reached a diagnosis of  an autoimmune liver disease called Primary Biliary Cirrhosis. If you missed it, you can find the intro here.

It was mid-December, and I had just received a call from my rheumatologist who reported that my liver enzymes were not elevated because of lupus, or infections or medications, as we had originally suspected, but indicated a problem with the liver itself. The last thing she mentioned before we hung up (at 4pm on a Friday afternoon) was the fact that she couldn’t believe that I wasn’t more symptomatic of liver issues based on my labwork and imaging.

As if kindled by the power of suggestion (and, shoot, maybe it was…) the next day I began to have severe, almost unmanageable pain that radiated from my right lower back to my abdomen. At first I thought it was kidney stones but within 24 hours nausea, vomiting, and the inability to eat made it obvious that I had a new problem. And remember the swim meet, and my doctor’s concern that I would hit a crisis over the weekend?  Her crystal ball seemed incredibly reliable.

I threw my things into the car, and headed back to Virginia to deal with whatever “this” was. More than anything, I wanted Dylan to be able to compete, because I was so tired of my illnesses causing us to cancel – like, EVERYTHING. And I think my audible moans were kind’ve freaking him out, but the lamaze breathing was failing me big time, and we were

dylan

He  loves posing after long swims…

determined to keep things as normal as possible. Once home,  (and on  Sunday) I was able to reach one of our family docs who spent a couple of hours pouring over my symptoms and labwork, and concluded that I was probably dealing with a gallbladder issue.

A few days later my gastroenterologist ordered more scans to confirm the gallbladder problems, as well as another round of labwork. (By then I was wishing they had just installed a faucet.) He also suggested that we consider a liver biopsy when they removed my gallbladder.  He ended the appointment by saying that he had a “hunch” as to what was going on but didn’t want to elaborate until he had more information. That was annoying.

 

mytree

Suck it, liver disease. Christmas waits for no one!

And…it was mid December, so getting on anyone’s schedule was virtually impossible. It became obvious that we wouldn’t have real answers until after Christmas. The next few weeks became even crazier, juggling holiday preparations and begging for appointments, while feeling absolutely miserable. I remember being offered a glass of wine and just staring at it, realizing that my “go-to” drink was probably not the best idea. My friend Chardonnay had overstayed her welcome.

 

We actually managed to have a lovely Christmas, setting aside the limbo between a “hunch” and a diagnosis. My pain was well-controlled, (thank you, pharmacology)  and my family helped me to be “present” during our celebrations. Two days after Christmas I received an email  notifying me that my bloodwork was in. I stupidly followed the link which logged me onto

norman

Not the place to read about a terrifying diagnosis.

the patient portal while sitting at the kitchen table, in the middle of a holiday breakfast. The “positive” next to the words “Primary Biliary Cirrhosis” took my breath away. I sat with my open laptop while my extended family buzzed around me – Norman Rockwell-esque, drinking coffee and laughing and chatting beside the fire. And I knew that I was having one of those before/after moments.

 

A google search quickly revealed that I needed to close the computer.

I turned back to my family and we packed up and headed home. The hidascan confirmed that my gallbladder had died, and we only had a few days to prepare for my surgery and clear the house of Christmas clutter.  And learn as much as we could about Primary Biliary Cirrhosis. My health took a nosedive as my compromised immune system fought with my decaying gallbladder, and I battled severe infections that required IV antibiotics and multiple trips to the Emergency Room.

The New Year couldn’t come fast enough, and I was finally able to have my gallbladder removed and the liver biopsied to stage the disease. The weeks following surgery were typical of an immune compromised patient in that it was fraught with atypical complications – collapsed lungs, cellulitis, a bladder that went to sleep, a wound dehiscence, and a confounded surgeon who muttered out loud that I really needed “Dr. House.”

Recovery was made even more challenging because I was trying to settle in to the realization that life was forever going to be different. A week following my surgery, we met with my GI doctor who explained that I was at a stage 2 of the disease, with “stage 3 inflammation.” Jim and I were rooting for nothing higher than a stage 2, because once your disease progresses to stages 3 and 4, the “outcome is much poorer” according to people who write scary things on medical websites.

We viewed the early stage as a gift, and continue to be grateful that we caught it early. My doctor immediately started me on Ursodiol – the ONLY medication for Primary Biliary Cirrhosis, and the early staging makes it much more likely that I will respond to treatment.

To be perfectly frank, I was devastated with the PBC diagnosis, and will share later how it temporarily made me lose my freaking mind shattered me. But finally so many things made sense. It turns out that many women who have PBC have a prior history of Urinary Tract Infections. In fact, it is even a predictor of the disease. The fatigue that had become so life-altering was another classic symptom. And the lymph nodes that swelled every time I had wine  was my body’s way of protecting me from ingesting something that was very, very bad for my liver.

It had become abundantly clear that I was not just “taking a few months off” to reevaluate my career and become more fit. I needed a new plan. A Plan B. 

Read about Jenny here