I chronicled some of my own issues with immune deficiency here but I want to use this space to provide more detailed information about CVID for those seeking a diagnosis or contemplating treatment. I will reiterate that I am not an expert, but an advocate and a storyteller, so please don’t treat this page like it’s “web MD” but rather a resource as you seek to understand your own challenges with immunity.
Despite it’s name, Common Variable immune Deficiency is not actually common, and is only found in about 1 in 25,000 persons. It is most frequently diagnosed in the third or forth decade of life. (I was diagnosed at 43.) About 20% of CVID patients have had symptoms since birth. PatientInfo.com describes it as “an umbrella diagnosis in that it encompasses a group of genetic disorders which result primarily in hypogammaglobulinanemia, or failure of antibody production. Patients typically present with recurrent infections, particularly of the respiratory tract. Gastrointestinal disease, autoimmune and inflammatory features and lymphoma are also more common in CVID.”
CVID “imitates” many diseases, such as Lupus and Rheumatoid Arthritis, and it triggers numerous other diseases. The most obvious problem is that we are prone to frequent infections because we have fewer antibodies (immunoglobulins) in the blood. Patients may have low antibody levels (hypogammaglobulinemia) – that’s what I have – or could be completely lacking in antibodies (agammaglobulinemia).
CVID is different for each patient, based on whether they have low IgG, IgA, or IgM. (Insert link HERE for IgG, IgA, etc). And it gets even more specific because IgG is comprised of four subclasses. A CVID patient will be deficient in some combination of those four subclasses. Each part of your Immune system (IgA, IgG, IgM, and IgE) does different “things” – each area protects you against specific diseases or infections, and going further, each subclass of IgG projects you against different diseases – which means every patient presents with different combinations of deficiencies, and therefore has different challenges. I am deficient in IgA, and total IgG – with subclass deficiencies in IgG1,2, and 4 – which means that my specific problems correlate directly to my unique deficiencies.
People with CVID may develop enlarged lymph nodes, an enlarged spleen, and painful swelling of the joints (polyarthritis). We may also present with gastrointestinal complaints, such as abdominal pain, bloating, nausea, vomiting, IBS – diarrhea,or constipation. And we carry a greater risk of virtually all types of cancer, but especially cancers of the lymphoid system and GI tract.
These clinical descriptions mirror the severe, chronic infections I’ve experienced: lymphadenopathy, six-month long sinus infections, multiple bouts of periorbital cellulitis, neutropenia, lots of pneumonia and bronchitis, septicemia, chronic uti’s, full body fungal infections, mycoplasma, asthma, issues with wound healing, IBS, diverticulitis, colitis, eczema, and I know that’s not all but now I’m sad and I need a cupcake.
Which leads me to another point – CVID patients often experience very rare symptoms or complications …..which is why we referred to as “ZEBRAS” in the medical community. A University of Maryland doctor, Theodore Woodward, told his interns that “if you hear hoofbeats, think horses, not zebras.” (It’s along the same lines as “if it looks like a duck and quacks like a duck, it’s a duck.” ) But for a patient with CVID, when we hear hoofbeats, we automatically think “Zebra” because we have to consider that we may fall into that 1 % of patients who have a rare illness or reaction. (I’d much rather fall into the upper 1% for wealth or intelligence.) The real challenge is finding doctors who “think Zebra” right along with us.
If you think you may be a “Zebra,” you need to find a great immunologist. (I will provide some links at the bottom of this post). You will also need to become a well-informed advocate for yourself in order to inform your medical team of your unique challenges. Just today, I was being scheduled for an upper endoscopy, and I had to carefully explain that the oxygen nasal cannulas used during anesthesia have repeatedly triggered facial cellulitis, which is a dangerous infection that should just never ever happen. My doctor stared at me for a moment and said “You have got to be kidding me.” (Um, nope, it’s right there in my records.) I was able to give them the treatment protocol developed by my own medical team after years of experience, using terms she understood. You are your best advocate.
How is CVID diagnosed? Find out Here
Top Ten Warning Signs of CVID: Click Here
Find an Expert Immunologist Here.