Greg Bales

On Living with Sjögren’s Syndrome

The announcement last week by Venus Williams that she was withdrawing from the U.S. Open after having been diagnosed with Sjögren’s Syndrome came as a surprise and raised a lot of questions in the press about Sjögren’s. As Reeves Wiedeman quipped, “That clacking noise was the sound of the world’s tennis journalists heading to Wikipedia.” In my house, we were as surprised as anyone, but the symptoms that Williams described as experiencing were very familiar. We already knew about Sjögren’s Syndrome: my wife Kathy Lyons was diagnosed with it two years ago. It seemed only right to invite her to write a post about her experience with the autoimmune disease.—gb


Venus Williams by Swiv, on Flickr, available under a Creative Commons license The news that Venus Williams has withdrawn from the U.S. Open because of symptoms brought on by Sjögren’s Syndrome has, at least for a day or two, brought some unusual attention to an autoimmune condition that plagues many. Sjögren’s (pronounced “SHOW-grins”) occurs when the body’s white blood cells attack moisture-producing glands. As a result, almost all sufferers have dry eyes and dry mouth. Most Sjögren’s patients also suffer from fatigue; many experience joint pain and swelling, muscle pain. Sjögren’s can affect a sufferer’s internal organs and the central nervous system. One manifestation of the disease even masquerades as multiple sclerosis.

Early in 2009, I noticed a pea-sized lump behind the hinge of my jaw. My mother had had a benign tumor removed from one of her parotid glands years before, and I was worried that I could have a similar tumor. So I went to see an otolaryngologist, who sent me to have an ultrasound with needle-guided biopsy. The ultrasound tech couldn’t find anything that looked like a mass, so they skipped the biopsy and simply pronounced me the proud owner of messed up parotid glands. Taking my symptoms into consideration, the doctor suggested that Sjögren’s might be the culprit. When we were about to embark on our second IVF attempt that Fall, I decided to find out for sure. My father had died of complications of lupus in 2007. He was diagnosed just days before his death. Lupus was what was on my mind, honestly. Lupus kills people (and it doesn’t). It also causes miscarriages (and it doesn’t).

Getting an autoimmune diagnosis can be a trial in and of itself. There are several criteria to be met for Sjögren’s, some of which are inconclusive and overlap with rheumatoid arthritis and lupus. (A rheumatologist in one of the recent Venus Williams stories referred to Sjögren’s as “a less dangerous, close cousin of lupus.”) There are tests for dry eyes and dry mouth. There is a lip biopsy, which some think is the most definitive test, but it can give a false negative. And then there are blood tests, which can also be inconclusive. So rheumatologists make a diagnosis based on a combination of these tests and reports of a patient’s symptoms. In my case, I had the abnormal parotid gland, my optometrist had given me a diagnosis of dry eyes years before, and my dry mouth problems had become severe and undeniable, both to me and my dentist. All of that, combined with positive blood tests, confirmed the diagnosis.

They say the average time to diagnosis is six and a half years, but Sjögren’s is a progressive disease, sometimes starting out quietly. Some with the disease can trace their symptoms back to childhood. After finally getting a name to put on the mystery, it’s hard not to go back and try to find the beginning…. When didn’t I keep a glass of water by the bed in case I woke up, parched, in the night? When didn’t I feel exhausted every day? What year was it when I got that supposed “exercise-induced asthma” diagnosis? (Venus experienced this one, and so did I.) Is this why I coughed so much as a child?

Both Sjögren’s and lupus in pregnant women present a small chance of a birth defect called congenital heart block, which is caused when autoimmune antibodies cross the placenta and create inflammation in the fetus’s heart that then interferes with the heart’s electrical conductivity and keeps it from beating normally. If this happens and the baby survives, it will most likely need a pacemaker put in after birth. The likelihood of heart block happening is low—only between 1% and 5%—and if it’s going to happen, it will usually be apparent by Week 24 of pregnancy. Ultrasound image of a fetal heart scan, from a 2001 paper by Prontera, Jaeggi, Pfizenmaier, Tassaux, and Pfister in ADC Fetal & Neonatal Edition; click the image to link to the the paper So I had fetal heart scans (see image, left) every other week from 18–24 weeks during my pregnancy. Greg and I were nervous about it at first, but felt more relaxed with every good scan. At 24 weeks the pediatric cardiologist declared us pretty much home free.1

There is thought to be an inherited predisposition for autoimmune disease, although it doesn’t necessarily seem to be the case that the same disease is handed down from parent to child. The statistic that crops up everywhere is that there is a 10% likelihood that a patient’s child will also have autoimmune disease. In this respect my family seems to be overachieving. My father had three children. My brother has rheumatoid arthritis, I have Sjögren’s Syndrome, and my sister… ? Well, let’s just cross our fingers and knock on wood on her behalf! So as little G grows, I’ll be watching with a wary eye, hoping he won’t represent the next generation of autoimmune madness, that he won’t eventually find his own body attacking itself with wild abandon.

I often think of Sjögren’s and related illnesses as being “mysterious in a bad way.” Diseases like Sjögren’s, lupus, rheumatoid arthritis, and fibromyalgia present with strange symptoms at times. (I think back to January 2008, when I caucused for a not-to-be-mentioned presidential candidate in the sweaty, stinky Herbert Hoover Elementary gymnasium with a red hot thumb swollen to the size of a short hotdog.) As Venus Williams has mentioned in interviews, there’s great relief in getting a diagnosis, in finally having an answer to the mysteries that your body has presented you with. But, as it turns out, it’s not always that simple. Autoimmune diseases often pile on. Sometimes people are diagnosed with more than one right off the bat. Sometimes they start out with one and then start collecting others. (Sjögren’s is referred to as “primary” when it’s diagnosed alone, “secondary” when it’s found in conjunction with another rheumatic disease like lupus or rheumatoid arthritis.) As one high-risk obstetrician who treated me while I was pregnant (and there were many) said, “So you’ve been tested and you know you don’t have lupus… yet.” (Hooray for bedside manner!) This slipperiness can drive autoimmune patients crazy. The Sjögren’s patients I know online who are also suffering from other conditions simultaneously desperately want to match each physical problem with a disease. Do I have this rash because of the Sjögren’s, the lupus, or the fibromyalgia? On some level, I just want to scream, “Does it matter?! Your body is trying to destroy itself! Who cares which disease is responsible!” But it’s human nature to want to categorize and label, to make sense of things, to find answers to mysteries.

Illnesses like Sjögren’s are sometimes referred to as “invisible” diseases because sufferers often function adequately most of the time: they go to work, raise families, look healthy. Some “Sjögies” I know online feel extremely put upon—that they’re suffering alone, without any recognition or sympathy, even sometimes from their families.2 Even worse, they often have terrible interactions with physicians (including rheumatologists!) who don’t understand Sjögren’s and the toll it can take on the body and mind. Guilt-fueled by nature and also aware of my very good fortune in having excellent health care at my disposal, my position is usually to keep it to myself, to be grateful that I don’t have cancer or heart disease. (Have I mentioned that my cholesterol levels are kickass?) But I can’t deny that it hurts when I’m too exhausted to play with my son. It hurts to admit that by the time Greg gets home from work, I’m relieved to hand G over to him so I can lie down for a while and try to rally in time for dinner. It sucks not to have the energy to cook dinner. It sucks to dread the next random malfunction of my body. It sucks to wake up in the morning to swollen joints, to feel like I’m 37 going on 77.

I’m sure a lot of tennis fans will be eager to see Venus return to the court, to improve her ranking, to triumph over adversity and a self-destructive body. I hope for her sake that happens, but the reality is that it may not. Chronic illness may force her to reimagine and reinvent herself as something other than a professional athlete. Personally, my wish for her is the same as my wish for myself and everyone: to have a life of wholeness in body—but more importantly, in mind and spirit—and to accept and embrace a certain amount of mystery in life. Luckily, it’s not always a bad thing.

1 To the best of my knowledge, Sjögren’s wasn’t responsible for the complications I had with the pregnancy (see the PDF linked in this post). I had the bad luck of having placenta previa—what was termed by at least one obstetrician as having a “rotten placenta”—and that seems to have been the main culprit.

2 Isn’t the word Sjögies wonderful? Like students of an annoying southern university or the nickname for a breed of dog. I’d like to get Greg a bumper sticker that reads “I ♥ my Sjögie.”

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Comments

September 18, 2011

Wonderfully written! Hit home with me on so many levels, kept reading & saying ‘me too!’. I’m a Sjogie with Fibro. It’s hard dealing with the fatigue and having people thinking you’re just lazy! Thanks for writing this. If you ever find that bumper sticker, will you let me know where I can get mine? :)

September 19, 2011

Thanks, Kelly! And so glad to have you back me up on the fatigue where my husband will see it. What a thing it is for me in particular to be married to someone who views naps for anyone over the age of 4 as a moral failing! Darned Protestant work ethic! :)

May 10, 2015

You say above that you had positive blood tests for Sjogren’s. If those positive tests included either SSA or SSB antibodies, then you have broken the rule that anyone who blogs about Sjogren’s has to be seronegative with either no lip biopsy or a negative lip biopsy.

As a person who also has seropositive Sjogren’s, which is to say Real Sjogren’s instead of Fake Sjogren’s (seronegative for SSA or SSB with either no lip biopsy or a negative lip biopsy) , I can actually relate to anything that you say about Sjogren’s – what works, what doesn’t, what it feels like, how it progresses, and so forth. Comments by the Fake Sjogren’s bloggers are useless and misleading.

It is impossible to tell the difference between seronegative Sjogren’s, and seronegative Lupus, or Fibromyalgia, or Chronic Fatigue Syndrome with dry eye, or a misdiagnosed disease of the parotid, or a misdiagnosed cancer, or a psychological problem.
However, I do believe that people with Fake Sjogren’s are actually sick and not just depressed or otherwise totally psychological, I am just saying that they are misdiagnosed with Sjogren’s. They will find this out as soon as they change Rheumatologists.

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