Part Two: Moving Toward a Diagnosis of Liver Disease

courageThank you for being patient as we re-post the early stories from this blog. In the past 24 hours, we’ve been able to help multiple people who have my same immune deficiency make the connections between CVID and PBC (one of the liver diseases that I have). Until recently, I was the only patient in both my immunologist’s AND hepatologist’s office with both diseases. My big mouth efforts at education and advocacy helped my immunologist identify another patient with PBC. So just pass on by if this doesn’t relate to you. Rumor has it that April the Giraffe may be pregnant again if you are looking for riveting content.

I’m doing the re-posting – God is doing the multiplying. Below begins the link between CVID and liver disease:

If this is your first time visiting (and ESPECIALLY if you are a CVID  or PBC patient,) I encourage you to start here   in order to understand the events that preceded my diagnosis of Primary Biliary Cirrhosis.

I will warn you that I explain this in excruciating detail because when I was first diagnosed with Primary Biliary Cirrhosis, I began scouring the Internet for resources. I found sites with great clinical descriptions, but I needed to hear from people who were living- and better yet, thriving, – with this disease. I needed firsthand accounts of what symptoms they had, which treatments worked (and which didn’t) what they ate, and how they coped.

What I found was….very little. I wondered whether my new comrades were either too sick to write, or  just busy living.

It dawned on me that as a fantastic googler voracious researcher and a decent storyteller,  I could parlay these abilities into advocacy, and create a space to help others who, like me, are frantic for information, and desperate to hear that it’s all going to be okay. The other reality is that in hindsight, things were missed….by the doctors, and by me. We now know that the symptoms I experienced in the year leading up to my diagnosis are common for PBC patients. What’s NOT common is PBC. The pieces of the puzzle were all there, but it took too long to put it together, and for this reason I go into excruciating detail about my experience…. not because my medical history is compelling reading material, but in the hope that it could help others reach a diagnosis sooner.


CVID couldn’t hold me back from the glamorous duties in children’s ministry

After I began treatment for CVID (Common Variable Immune Defiency), I enjoyed about a couple of healthy-ish years. I even avoided the colds that plagued many of my colleagues who worked with the germ factories children in our ministry, thanks to my weekly infusions of hizentra, an immune replacement therapy. As time went on, the side effects of my medication lessened, and the Thursday night infusions became second nature.

But in November of 2014 I came down with what would be the first of many chronic, and almost constant, urinary tract and kidney infections.  The next twelve months brought infection after infection, and antibiotic after antibiotic. It was so disheartening, and I was frantic for a cure. If you have had a UTI, you know how much they absolutely SUCK. If you haven’t, go drink a gallon of hot acid, and then try to pee. (I know my sons are thrilled that I am writing about tinkle.)

Determined to get to the bottom of these chronic infections, I consulted with a naturopath/nutritionist. She put me on a delightful diet that included no wheat, no sugar, no gluten, and no dairy. But I did get to have green smoothies in the morning, so, you know, that was awesome. In spite of my phenomenal attitude, within days I felt better. All UTI symptoms disappeared within 48 hours. The digestive issues that plague most CVID patients went away. And while it was REALLY (!!) hard to eat this way, feeling better was such a relief that I stuck with it.

pigeonMy new “clean” diet allowed me to enjoy a five month period free of symptoms, and allowed me to dive back into work and family. The only remaining issue was fatigue. I was still so tired by the end of each workday that I would call my mother on the way home so that I could stay awake.

I was having a routine visit with my immunologist in June of 2015 who was again commenting on how well I was doing until he felt a lymph node in my neck. I had recently noticed that if I had more than 1 glass of wine (please don’t judge me), the nodes in my neck would swell. He quickly jumped into action because  lymph node swelling with alcohol consumption is a common symptom of lymphoma. Knowing that  CVID increases my lymphoma risk by 200Swag%, he called for an immediate biopsy.

Except….I knew it wasn’t lymphoma. I had this nagging suspicion that it was more about the wine, and my body reacting to it. I managed to put off his pleas for the biopsy long enough to enjoy our summer. But in September, with the nodes still swollen, he put his foot down and scheduled me for surgery. This will remain one of those “things I wish I’d done differently.” And while Jim and I were positive that it wasn’t cancer,  in mid- September I checked into Loudoun Hospital for the biopsy. (What you need to know is that for an immune compromised person, a simple procedure is never simple, and almost always carries risk and complications. We have to be sure there is reason to  cut.)

True to my history, the “routine” cervical node biopsy set off a chain of craptastic health issues that filled the next month. While the actual biopsy was“routine,”the days following the procedure were not. Ulcers filled the inside of my mouth, throat, and esophagus, and made eating unbearable. I began having constant sweats…every fifteen minutes I became completely drenched. It was very sexy. And the UTI/kidney issues returned with a wickedness I had yet to experience. But by far the strangest symptom was that I would wake in the middle of the night with an ache in my liver, and a flu-like feeling all over. As expected (and thank you, God), the biopsy was clean. But I knew that something else was very wrong.

Over the next month, I recovered from most of the effects of the surgery. What never left was the profound fatigue and the daily UTI symptoms. And the “rubber band legs” had become a constant symptom. I assumed that  my growing list of symptoms was just delayed recuperation from the surgery. But then one day I hit the floor.