cvid

HOW I FOUND OUT I WAS A ZEBRA

I was diagnosed with common variable immunodeficiency in early 2013 (which is not common at all). In a nutshell my immune system is severely compromised and I do not produce the immunoglobulins or antibodies that I need to stay healthy and fight infections. My lungs are scarred from every bronchial infection and chronic cough I’ve ever had, my t-cells have multiplied way beyond normal ranges and my lymph nodes are swollen in my abdomen, chest, armpits and neck.

In the medical world people with primary immune deficiencies are called zebras. We’re not your typical horse; the most obvious medical answer is not always the right one. Medical professionals need to think outside the box when dealing with us. The most obvious answer to my medical mysteries were that I had cancer or that I was a hypochondriac, neither of which are true.

ROAD TO DIAGNOSIS

It took a good 2.5 years to figure out my diagnosis, though medical mysteries dating way back can now be explained via my diagnosis. When I finally got health insurance with my first job in Seattle I visited the doctor and started learning what was wrong with me. Unfortunately I went from “you may have lymphoma” to “we don’t know what’s wrong with you”. It all started with a routine blood test, where I learned I had a very low platelet count, I was referred to a hematologist/oncologist where we discovered my spleen was enlarged and various CT scans and PET scans declared that my swollen lymph node situation was widespread.

I’ve had two lymph nodes biopsied, one via a mediastinoscopy (they snagged a lymph node in my chest via an incision in my throat) and one enlarged lymph node in my abdomen was accessed via a needle biopsy. These results were inconclusive and mentioned things like “sarcoid-like” and “follicular hyperplasia” which in reality meant a whole lot of who knows what. My spleen continued to increase in size causing pain and inability to eat a full meal since my stomach was crushed between my ever growing spleen and liver. Three days after my wedding my spleen was removed so I could be pain free and my husband and I could start a family. I actually had a fairly healthy pregnancy with the exception of contracting swine flu in my second trimester even though I had been vaccinated (turns out vaccines don’t always work when you have cvid – but I didn’t know then).

After my oldest was born I got really sick again. So sick that I really thought I was going to die. The holidays were ruined and I coughed my way into the new year. I coughed so much I bruised my ribs and even caused a hairline fracture. I went back to my oncologist and he referred me to an immunologist, a few blood tests later and finally an answer: cvid.

My immunoglobulin levels were:

IgG: 115 mg/dL (normal = 750 – 1600 mg/dL)

IgA: <5 mg/dL (normal = 85 – 385 mg dL)

IgM: 2 mg/dL (normal = 55 – 375 mg/dL)

SOLUTION?

Only IgG immunoglobulins can be replaced. There are a couple methods to doing this, IVIG (intravenous immunoglobulin replacement therapy) via IV in an infusion center or at home every 3-4 weeks or sub-q (subcutaneous) injections you give yourself every week via needles placed in multiple sites. After 40 IV sessions I gave sub-q a try once and went right back to the IV. Right now sub-q is not for me; it required 4 needles 2x/wk and too much time. I’m back at the infusion center receiving IVIG.

THE PLAN

To keep from keeling over I currently receive infusions of immunoglobulins every 3 weeks (4 bottles = 40 mg). Infusions take anywhere from 4-5 hours. I also take a broad spectrum antibiotic (z-pack) every 3 weeks, I wash my hands a lot and wear a mask when in hacking/sneezing crowds.

There are all kinds of complications with cvid. My IgG levels currently average around 1100 mg/dL so I’m officially “normal”. My immunologist would like to keep me over 1000 mg/dL. My lungs are permanently damaged and I’ve officially been diagnosed with interstitial lung disease. I use an inhaler when needed and am working towards strengthening what good lung I have left. I suffer from chronic fatigue syndrome and I will need to have an endoscopy every 5 years to check for stomach cancer. I also have my lymph nodes checked from time to time. When I get sick even with a simple cold it’s a big deal. I keep in frequent contact with my primary doctor, immunologist, pulmonologist, dermatologist and oncologist when needed. The goal is to stay on top of any illness and to live a healthy lifestyle.

At the end of the day sick people terrify me! But this diagnosis is of some relief as I now know why I get so sick and for so long and I have validation for feeling so crummy. IVIG took some getting used to. I still have my hard days and from time to time I also worry about my life expectancy. But all I can do is keep myself as healthy as possible, continue to be screened for cancers and hope for the best.