It started with a toothache. Well okay, it probably started much sooner than that – maybe with the German Measles I caught at age 17, three years after I had the vaccine, or maybe the weird double pneumonia after a five day camping trip when I was 22. I remember collapsing onto the floor when trying to walk from my bed to my sofa and it took over a month and several antibiotics before that thing finally went away. Maybe the ear infections that made me give up swimming. Maybe it was the severe GERD, that I had absolutely no risk factors for. Certainly the GERD surgery I allowed myself to be talked into around age 37 was a factor. What doctors like to call a triggering event. Shortly after that surgery, I developed terrible fatigue and then a toothache. Then I sold my house and moved into a flat with a mold problem. I developed an asthmatic cough.
But the thing that really caught my attention was the toothache.
Toothache isn’t really the way to describe what I had. It was nerve pain in my mouth that radiated into my face and burned its way down my throat. I had a private therapy practice at the time, so taking sick leave or even taking a day off hit me directly in my ability to pay my mortgage. I lived alone.
At first, I assumed the problem was TMJ and I sought alternative treatments – massage, acupuncture, got my dentist to make a mouthguard. I ignored the other symptoms – the increasing night sweats and chills (I’d been having those since I was 19, so they were easy to ignore by now), the increasing difficulty breathing (I’d had a complete asthma evaluation just before my GERD surgery and they’d decided my asthmatic cough was actually GERD related – no sign of breathing problems), the brain fog (a feeling like I had taken a bunch of Benadryl and everything was far away and my brain was filled with cotton), the fatigue (ok, I didn’t totally ignore the fatigue – I went to the NP at my doctor’s office and had some blood tests. We decided it was Seasonal Affective Disorder and I bought a light box), the joint pain, the bone pain. It wasn’t until I developed a nasty sinus infection and took a course of Amoxicillin that I understood that my toothache was not TMJ. All of my symptoms resolved on the antibiotic. My TM joint, which had been locked out of place, relaxed and went back where it belonged. I felt renewed. Until I stopped the antibiotics.
That was the beginning of five years of searching for answers. I went to multiple primary care doctors, dentists, neurologists, oral surgeons, ENT docs, asthma and allergy docs. I even spent a week at Mayo. Everyone diagnosed me based on what they were trained to see. I had TMJ. I had a neuralgia. I had depression. I had developed asthma, though it was all small airway loss and quite dramatic compared to the evaluation I'd had three years earlier. I had sinus infection after sinus infection. I got pneumonia. I remember calling a friend and asking him to check on me in the morning, because I was afraid I might die in my sleep.
I'll never forget the Mayo dentist: "It's awful to have pain when there isn't any reason, isn't it?"
I became an antibiotic seeker the way some people are drug seekers. The antibiotics were the only thing that worked at all to help me feel better. I learned that if you go to an urgent care after hours and tell them you had tooth surgery and are having increased pain, it isn’t hard to get a prescription for antibiotics out of them. One ENT doctor decided I had an infected submandibular gland in my mouth and gave me six months worth of antibiotics. I filled that script before he could change his mind (which he did) and I’m convinced those antibiotics saved my lungs from serious damage.
Finally, after five very long years during which I kept myself alive by promising myself I could kill myself if things weren't better in six months and extending the six months every six months, an ENT doctor at Massachusetts General Hospital diagnosed me. He drew a bunch of blood, gave me a pneumonia shot, had me back in a month and drew more blood. Turns out he was checking my Immunoglogulins. That’s IgG, IgA, IgM, and IgE. I’m a bit unusual in that I was only low in IgG, but I was low in all four subgroups of IgG. And the second part of the test involved checking whether I could mount an antibody response to the pneumonia vaccine. I couldn’t. Now I had a name for what was wrong with me:
It's one of many Primary Immunodeficiencies: Common Variable Immune Deficiency
You can read about all of the primary immunodeficiencies here: Immune Deficiency Foundation
From the pamphlet on CVID:
Common Variable Immune Deficiency is a disorder characterized by low levels of serum immunoglobulins (antibodies) and an increased susceptibility to infections. The genetic causes of the low levels of serum immunoglobulins are not known in most cases. It is a relatively common form of immunodeficiency, hence, the word “common.” The degree and type of deficiency of serum immunoglobulins, and the clinical course, varies from patient to patient, hence, the word “variable.”
Most people with CVID don’t present with a toothache as a primary symptom, which may have confused the picture. Although my asthma symptoms that resolved with antibiotics, my recurrent sinus infections, my pneumonia in spite of having had a pneumonia vaccine, my joint pain that resolved with antibiotics – these are all classic presenting symptoms of CVID. The real problem and the real reason I went undiagnosed for five hellish years is simple. Doctors are taught that when they hear hoof beats, they should look for horses, not zebras. I am a zebra. What I have is rare enough that doctors are told they probably won’t ever see a case of it in their practice. On the other hand, what I have isn’t all that rare. It’s just horribly underdiagnosed. The other problem I had is one that seems to be common among my CVID friends – I was not making much inflammation. I could have a clear CT scan of my sinuses and end up very very sick later that day. I always had to answer no when asked if I had a fever. My temperature went down when I was sick. Elevated white blood cells? Never. Signs of inflammation in my bloodwork? Never. No wonder I couldn’t convince anyone I had an infection. Even the doc who diagnosed me ended up lecturing me about taking too many antibiotics. I had to find a doctor who really understands CVID. Now I’m in the best hands possible, in my opinion. Now I’m getting the appropriate treatment.
Treatment is infused gamma globulin. Some of you have probably had a gamma globulin shot somewhere along the line. Some of you may have had IVIG. It’s the pooled antibodies of thousands of plasma donors. It basically replaces a part of the immune system that isn’t working. It gives me the army to fight off bacteria. Some people do it once a month in infusion centers via IV. I choose to do a small amount every day subcutaneously. I have a small pump. I draw up the meds and stick a needle in myself every day. I never resent that I have to do that. I’ve been doing it for five years and I’m still grateful every single day when I go to draw that precious gamma globulin into my syringe. I’m crying as I write this. That’s how much this treatment means to me. Tonight, I came home from an eight hour work day, did some pilates, made dinner, ate, and cleaned up after myself. Five years ago, that would have been unthinkable. I would have been on my sofa trying to stay awake long enough to drag myself to bed. That’s the difference gamma globulin has made in my life. I still get infections more often than most people. I still need a longer course of antibiotics. I’m by no means the picture of health. But I got my life back.
By the way – that tooth? A vigilant dentist finally found a tooth with internal root resorption. When I had it pulled, he discovered infection that had burrowed up and perforated the lining of my nasal cavity. Never showed on any x-ray, CT scan, or even a three phase bone scan. I still have pain in that area. Made the mistake of letting the dentist put an implant in. But the pain is far more tolerable than it used to be. It improved almost immediately when I started the gamma globulins.