Part One: The Story of My Incomplete Immune System & CVID

Jenny and JimWe decided that it’s time to re-post the beginning of our saga...we have so many new readers (thank  you, and UNBELIEVABLE!) that we probably need to start over and explain that one of the reasons I was such a great hostess for those invasive devils known as ticks was because of my incomplete immune system. If you’ve already read this, PLEASE mosey on over to take a peek at Fiona, the baby hippo. It’s her birthday, and she’s much cuter than…ticks. Anyway, here is the first chapter in a very long, and as-yet completed, story.

From October, 2016: 

This begins the long story of a long journey to unravel a mystery from an enigma~from immune deficiency, to liver disease, and most recently, a diagnosis of lyme disease. I have no medical background (unless webMD counts as a medical background, and if so, I’m all good) and I will not tout a particular course of treatment…but I am a huge proponent of self-advocacy, empowerment through information,  and radical self-care. And as most stories go, this one starts a long, long time ago.

Because when it comes to my health,  I have always been a bit…atypical.

When I was little, I would occasionally wake up looking like I had swallowed a softball, the lymph nodes in my neck swollen to enormous proportions, terrifying my poor parents.

And once I peeled an orange and a teeny tiny piece of the peel got stuck under my fingernail causing AN INFECTION IN MY BLOODSTREAM. I got septicemia from eating an orange.

And I’ll never forget the incredulous look on a supervisor’s face when I explained to her that I could not fulfill my duties because a gnat had flown up my nose and caused cellulitis in the bone of my eye. I know she thought I was the worst excuse maker EVER.

Once I was overcome with arthritic pain in every jont in my body over the course of an hour. I was holding our baby (son #3) while my husband was mowing the yard, and by the time he came back in I was in agony, literally every joint inflamed and sore.  I couldn’t get off the floor without assistance, and I certainly couldn’t hold the baby. We had to call my mother in for support because Jim was traveling for work, and she remembers how I moaned in my sleep. While those symptoms eventually subsided, they left my doctors, and Jim and I, pretty shaken – and  with no explanation for what was happening.

All of these are clues on the trail to diagnosing an immune deficiency. Doctors poured over my bloodwork for years, checking my immune system with absolutely nothing showing up.  I remember one disturbing conversation with a specialist from UVA who predicted that I would eventually be diagnosed with “something” autoimmune, even though they couldn’t pinpoint it at that time. He suggested I just get on with life and be as healthy and happy as I could be. Um….okay.

But that is what we did. Jim and I made the deliberate choice to let go of the unknown, and just live.  We busied ourselves with our boys and one another, threw ourselves into work, and eventually forgot about the dire warnings that for years didn’t seem to be coming true.

Until 2011.

mask

Travel Tip for Healthy People who want to be left alone: Wear a Mask. For some reason, when you wear a mask, people think you don’t have a mouth. So they don’t talk to you. “Sir, what does your wife want to drink?”

Without going into details that you don’t need or want to hear, a dangerous bout of e-coli in August of 2011 left my immune system so depleted that over the course of a year my numbers dropped to dangerous levels.   I was totally thrown when what I thought was a routine appointment with my immunologist changed life forever. I remember feeling like I was having an out of body experience, floating above the room as he talked. He explained the diagnosis of CVID, Common Variable Immune Deficiency  (which isn’t ACTUALLY common). He described how my immune system was so bad that a single cold could kill me within days because septicemia or pneumonia would set in and take me down very quickly. I was alone during this appointment, and I don’t think I asked a single question as he rattled on about the need for life-long immune replacement therapy, drew pictures of my damaged cells, threw out statistics of my now-increased risk of cancer, and how I needed to wear a mask in public.

Have I mentioned that I worked in Children’s Ministry, around precious little germ factories?

baby

Job Perk 

Jim and I absorbed the news for a couple of days and scheduled an appointment with our family doctor. We really couldn’t believe things were that dire. ( To look at me, you’d never know that anything was wrong.) The need for life-long weekly therapy seemed extreme to us, and was shockingly expensive. That appointment sealed the deal for us, as the doctor explained that in over twenty years of practice, he had never seen an immune system as deficient as mine. He had not spoken with my immunologist, and pronounced the same warning that a simple cold would be life-threatening.

In hindsight, I can’t believe I questioned the need for Ig therapy. During the year prior to diagnosis, I would catch a cold and within hours have pneumonia. A sinus infection caused another case of periorbital cellulitis  in the bone of my eye, and my weight was plummeting. (I actually thought I was just THE BEST PERSON EVER at counting Weight Watchers Points.) I was plagued with digestive issues, and fatigue had become so constant that I was ordering my life around the ability to sleep. The strangest symptom came one day when I woke up and my legs had no strength. They felt like rubber bands, or like I was walking in jello. Even now, when I am not well, the rubber-band legs reappear to warn me that something’s up.

infusions

Thursday night infusion prep: easy peasy

In February of 2013 I began weekly infusions of  immune globulins  to replace my immune system. A nurse came to the house  to train me to do the treatments on my own, and it really wasn’t as bad as I feared. I do weekly subcutaneous infusions, which means that I don’t need to infuse the plasma through a vein, but rather right under the skin, making it much easier for a lay person to administer.

It took about two-three months for me to build up a healthy level of Igg. During the first few months of treatment, I continued to have frequent infections, and another hospitalization after yet another bout of cellulitis. But eventually, the treatments became second nature  and the side effects lessoned. My “tribe” sent gifts, cupcakes, DVD’s and love. Thursday nights became “my time”to retreat and infuse, and Friday mornings were set aside for recovery.

And then…. THINGS GOT SO MUCH BETTER.

christmas-eve

500 kids for Christmas Eve~no longer a threat thanks to Immunoglobulins.

After an initial adjustment period, the treatments became routine, and my health drastically improved. I managed to work in Children’s Ministry and never get a cold. The frequent, severe infections abated, and the “rubber band legs” disappeared. I enjoyed not thinking about my health, and my immunologist was thrilled with my progress. I would sail into his office, give blood, and we would chat about vacations and wine, and what colleges our kids were looking at.

We enjoyed this honeymoon for a couple of years – and while I still took precautions, I wasn’t ruled by fear. I once again busied myself with life: parenting, swim team, and work.  I sailed along  until the fall of 2014 when things began to change  – but we had no idea that we were at the beginning of a marathon.