PBC Advocate

Evolution in chronic illness

As a chronic illness warrior who has dealt with migraines, meningitis, plantar fasciitis, multiple surgeries to repair damaged hyper-mobile ligaments and joints, a rare autoimmune liver disease called PBC, chronic bronchitis and allergic asthma, among a myriad of other challenges, they all pale in comparison to the big one.

I always felt that my PBC was my biggest challenge. While this is
a very slowly progressing disease, eventually leading to liver failure and the need for a liver transplant, progression normally takes decades. In my case, it was seven years from the earliest stage (0/1) of disease to the point where my liver is now cirrhotic and functioning at less than 30% of normal (stage 4). This is why I’m a zebra. In medical school, new students are taught to think common, not rare with the saying “when you hear hoof beats think horses not zebras”. Simply put, if some patient presents with a stomach virus, vomiting and diarrhea, dehydration and weakness, think virus gastritis (stomach flu).

Last week, after four days of being unable to keep and food, drink or medication down due stomach nausea and vomiting, with localized discomfort in my stomach area, I was sent to the Emergency Department of my local hospital. Whenever I see a new doctor, I always make a point of explaining that I am a zebra so they do consider the rare from the beginning, as well as the common. The doctor ran blood work (which all looked good). He also ran a CT scan to rule out an obstruction in my bowel, and again the results were all good.

Then came the news which explained his somewhat awkward tone. The CT scan showed a mass in my left kidney. I mentioned that cysts are not uncommon in PBC patients and his response was simply “they said it’s a mass”. Breathe. Breathe again. A mass as in cancer? Yes.

As it turns out, I have both a tumor (renal cell carcinoma, RCC), stage 1, enlarged lymph nodes adjacent to the tumor that are as big as the tumor itself, a kidney stone (which has been there for several years and has not gotten bigger), and a cyst in the kidney. They also saw a very small cyst in my pancreas. And finally an enlarged spleen and enlarged common bile duct as well as portal hypertension which all are signs my liver is starting to fail

Here’s the rub for all of this. I must have the tumor removed so I can be cancer free. To obtain a transplant, a recipient must be cancer free 2-5 years depending on the transplant center’s guidelines and the type of cancer. The good news is that my cancer (RCC) is easily removed at this stage and generally does not require any further treatment like chemotherapy or radiation treatment.

Now for the liver… as I move towards End Stage Liver Disease (ESLD), also called liver failure and undergo evaluation for listing on the transplant list I can’t get listed until the cancer is gone for a minimum of 2-5 years. It doesn’t look like my liver will hold on that long. That means I’ll be put on the “too sick to transplant“ list until such time that my cancer is clear long enough. The likelihood of this working out in my favor is low, less than 20%. A Catch 22 scenario. What to do?

To start with, the tumor removal. Without this step, the cancer could metastasize and spread. I have to have the kidney stone removed as well but it will be done as a separate procedure to allow my kidney heal before the tumor removal which is planned as a robotic assisted partial nephrectomy endoscopic surgery where they’ll take the lower part of my kidney and close it up so it still functions well. With this plan my prognosis is excellent with only a 1-2% recurrence rate. Hopefully my liver can hang on long enough to allow the RCC to clear.

Have you ever sat on a teeter-totter backwards? Its disorienting and awkward. You sit there looking at where you’ve come from instead of where you’re going. Thus my evolution as a chronic illness warrior. I must evolve and adapt to this new challenge to succeed, to live, to thrive.

I’m not afraid of what’s coming, I’m afraid of being stuck where I was or where I am now. Evolution is about change.

Change is nothing more than a new opportunity to live my life according to my contract as an honest, trusting, and joyful woman.

Cheers!

Cathy
PBC Advocate