The Face of Sjogren's Syndrome

I’m pretty angry right now. I'm not angry at the world (at least not about this) but I am still steamed nonetheless.

I’ve been complaining of a variety of symptoms since I was 17 years old. It started out with my parotid glands (the two main salivary glands located in front of the ears). Periodically, they would swell slightly and ache. When these episodes would occur, even smiling became painful and to eat or drink anything meant having some serious, stabbing pain in those glands. The episodes didn’t happen often – perhaps once every few months. When I complained to my family doctor he told me I likely had salivary gland stones and that it was no big deal. No testing, no referrals…nothing.  I can't be too angry with him because in all honesty, there'd be no reason to assume different in a 17 year old.

Over time, the symptoms got worse. My glands started to swell and hurt more frequently – a few times a year turned into a few times a month which turned into a few times a week and then almost daily. My mouth began to feel as though a wad of dry cotton was always wedged into it, my eyes became to become dry and irritated, my skin started to flake, my hair began to thin, my joints started to ache, and fatigue set in. I complained to the campus physician in college only to be told that I probably had a cold…and that the fatigue was because I was a busy college student. I was sent away with no answers other than to scale back on my semester hours. I complained multiple times during medical school to my medical provider who (in softer terms) told me that I was “imagining things…medical students are always fatigued and always think they have symptoms of some disease.” Again, no referrals, no tests, no answers – and worse, a doctor who thought I was full of beans.

I complained my doctor here in Vermont who essentially told me the same thing – that fatigue is a part of residency, that I probably had salivary gland stones, and that it was no big deal. When I developed 5 cavities in spite of meticulous dental hygiene, my dentist told me I just needed to work at brushing and flossing a little harder. Fed up, I finally gave them all the finger and self-referred to an ENT specialist. I would have done it sooner, but this is the first time I've had insurance where I could self-refer...anyway, I digress. After taking a careful history (the only history that anyone had ever bothered to take), he performed an ultrasound of my salivary glands and told me what I had known for years…that something was wrong and that the scan of my glands showed that I likely had an autoimmune disorder He referred me to one of the local rheumatologists and I felt like I would finally get somewhere.

And then I met the rheumatologist. Again, he took a history and did a thorough physical examination and essentially told me that I was full of beans. After all, when he stuck his gloved finger in my mouth, he could feel saliva – so clearly, nothing was wrong. In fact, he told me that he thought serologies would likely be negative, but if I wanted to really search I could consider a lip biopsy to which I promptly said, "No thanks." In the end he had his rheumatology fellow order laboratory testing.

Yesterday, I received a follow-up call from the rheumatology fellow. He informed me that my ANA (anti-nuclear antibody) testing was “very positive” with a titer of 1:640. Specific autoimmune markers for Sjogren’s syndrome (ss-A and ss-B) and RF (rheumatoid factor) were also quite positive. Adding insult to injury, the asthma that was diagnosed 10 years ago may not, in fact, be asthma – it may be respiratory tract involvement by this autoimmune disease. Voila! It only took 11 years and multiple doctors to prove that I was not making my symptoms up, that my complaints were more than the random complaints of a tired hypochondriac. Ergo, I am now starting on Plaquenil, a drug that I never hoped would be introduced into my life. I will now require yearly ultrasounds to check for lymphoma (patients with Sjogren’s are at increased risk for lymphoma), yearly eye examinations to check for retinal complications from Plaquenil and to make sure that the Sjogren’s isn’t damaging my corneas, and yearly laboratory and rheumatologic evaluations to monitor my organ function for both toxicity from Plaquenil as well as worsening involvement by this autoimmune disease (think renal problems, neuropathy, etc). Sounds like fun, right?

I admit that I’m a little miffed about this. I’m miffed because for 11 years, nobody believed me when I told them that there was a problem. I was brushed off with gentle platitudes and constant reminders that I was just a complaining high schooler/college student/medical student/resident. It makes me a little angry because had anybody ever bothered to take me seriously, perhaps this could have been figured out quite literally a decade ago before all the symptoms really set in. Perhaps I could have been started on medications that would have prevented progression of the disease – or at least delayed the progression.

But instead, I have been living in a state of relative misery for the last 11 years. My glands hurt nearly every single day. They don’t just ache, they feel as though someone is stabbing them repeatedly with a steak knife. They remain slightly swollen, making things like opening my jaw occasionally difficult. My mouth is so devoid of saliva that no matter how good my dental hygiene I will almost always have dental caries. I develop spontaneous small ulcerations of my gums without warning and my nose is often so dry that it spontaneously drips blood.   I can’t swallow bread or other dense foods without drinking copious amounts of water and even then food gets stuck. My eyes feel as though a fan is always blowing in them and no amount of eye drops will permanently combat the slight blurriness that has developed. In fact, just about everything (and yes, I do mean EVERYTHING) is dry. Not even my sweat glands function appropriately these days. My joints and muscles ache and I am fatigued – and when I say fatigue, what I mean is that some days I am almost too exhausted to get out of bed despite having had a full night’s sleep. No matter how much sleep I get or how much rest I indulge in, there are some days where it takes all of my energy reserves to sit through work without falling asleep at the microscope. Some days just walking upstairs to the bedroom takes the wind out of my sails. It is at times almost debilitating. But I deal with it because letting these problems defeat me is not an option.

If anybody had ever bothered to listen to me when I was complaining about mild symptoms at 17, the last ten years might have been partially mitigated with medication. Nothing can really stop or cure an autoimmune disease but symptoms can be managed and disease progression can be slowed. Maybe if something had been done sooner I’d have more gland function than I do – I might have been a “better oiled” machine, so to speak. Perhaps the aches and pains would have been less noticeable, and the fatigue that has plagued me since late college could have been avoided. The “what if’s” are tremendous but in a sense it doesn’t matter because nobody did anything except tell me that it was all in my head.

I am not thrilled to have an autoimmune disease because quite frankly, it sucks. It sucks to have a condition that cannot be stopped, a condition where what has been lost can never be recovered. I am not thrilled to be starting on long-term Plaquenil because it is not a completely benign drug – it can have some very serious side effects. But I am trying to be optimistic – now there is a diagnosis and something is being done about it – and I look forward to the promise of feeling better. I want to wake up in the morning feeling rested and I want to be able to walk upstairs without feeling as though I am walking through molasses. Maybe I'll be able to enjoy a run on the treadmill in the basement. I want to play my violin for long stretches of time without having to take breaks because the joints in my fingers ache. I want to enjoy food without feeling pain and I want my to keep all of my natural teeth. And maybe, just maybe, Plaquenil will allow me these small comforts. The thought is really nice.