Being Tested for Fatal Interstitial Lung Disease with Sjögren’s Syndrome

I have been dealing with shortness of breath for about 8 months now. Honestly, I would crack jokes about how I was out of shape and out of breath for the past five years. I can even remember hiking the Swiss Alps in college and sitting alone on a large rock while my comrades were literally miles ahead, sucking in for clear air. Maybe that was my Sjögren’s all along, even then, destroying my lungs, my ability to breathe, and my potential for a long life.

According to recent studies, 9-24% of Sjögren’s patients experience “clinically significant lung involvement” (this statistic was found in an article comfortingly entitled, “Clinical pulmonary involvement in primary Sjögren’s syndrome: prevalence, quality of life and mortality”). Most commonly, Sjögren’s lung involvement goes bad – interstitial lung disease (ILD). Under that branch, the most common type of ILD that Sjögren’s patients experience is a type that causes scarring of the lungs, which means it is irreversible and therefore, fatal.

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When I told my rheumatologist I was having shortness of breath, she asked questions about chest pains (“yes, but I’ve had pains for years”) or coughing (“nothing abnormal, no”) and recommended that I partake in pulmonary function testing (PFTs). Might I add from personal experience, PFTs are not pleasant when your main concern is shortness of breath. It definitely tests your lungs and, for me, my lightheadedness and consciousness. At the end of the test, the tech asks me when I’m seeing my doctor for a follow up. I say in a month, why? He sits me down to tell me that my lung volume is less than what it should be. It’s “in the normal range”, but low for a 23 year old nonetheless. He can’t explain why, and I should see my referring doctor for a follow up. I thought it odd, but figured I would Google for more info later. While waiting for my next appointment of the day, I Googled my pulmonary function test results.

Bad idea.

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If you have Sjögren’s and have never Googled about lung involvement, I don’t suggest you do unless you have a psyche of steel and anxiety is nonexistent to you. I flashed through webpages, reading about fatal lung diseases, the most common form of death in Sjögren’s, 3-5 years of survival, no treatment, poor prognosis. While seeing “unexplained progression” and “poor outcomes”, I felt violated by my first rheumatologist’s assessment of “Sjögren’s won’t kill you, you will just die with it.”

I suddenly realized that this whole time, my rheumatologist was testing me out of concern for ILD. She did a chest X-ray weeks ago at one of my routine rheumatologic follow ups – normal. But sometimes those results do come back normal for Sjögren’s patients who still end up getting diagnosed with ILD. I suddenly became an expert at reading PFTs and determining what they mean – only knowing that I still knew nothing at the end of the day. The next step was to schedule a CT scan to examine if there was lung disease present, which the hospital called me to schedule immediately due to my doctor’s “testing required by” date of the end of the week. The next two weeks leading up to the CT scan were truly agonizing knowing I had no information, and yet way too much.too much info chandler.gif

My initial appointment happened on a Tuesday, and my husband went out of town for a month leaving the following day. Sometimes the universe likes to kick you while you’re down, eh? While alone, I spent my time searching the internet for answers and asking for relief or personal stories from my Sjögren’s groups on Facebook. People were so kind, so supportive, so understanding of the freakout. It made me realize that even if I wasn’t diagnosed with ILD, this fear, dread, and anxiety for Sjögren’s patients every single time we feel a chest pang or notice we’re panting a little extra for breath, is REAL. And it’s VALID. Something out there is big and it’s mean and it’s threatening our very existence with this disease that is not supposed to kill us, we’re supposed to die with it.

I spent my next two weeks analyzing the idea of mortality. What if, at 23 years old, I was told that I had 3-5 years left to live? Or even ten years left? And so, I thought. My parents and sister would be devastated, but they would live on and for me, proudly and emphatically. I took my dogs for walks and marveled at the various colors of tree bark, the vibrance of the leaves, the refreshing whip of the cool wind, the awe of cars rolling busily by, thinking that others were so lucky that they didn’t realize it all had an end date. I washed the dishes in the sink, musing that I would want people to wear blue to my funeral. It’s my favorite color. I would become hysterical with sobbing and desperate for time at the thought of leaving my husband behind in a life that we built. Then I decided I would leave him letters, and ask my family to promise to watch after him. One day I was so numb, I just laid my face on the kitchen floor to feel the cool laminate and stared at all the dust under the stove, unmoved and unbothered for God knows how long. My parents reminded me that we had no information, and to not go to a dark place. But for me, it was impossible. How do you not think about the possibility of mortality when it’s staring you in the face?

Needless to say, every day of those two weeks, I was either peeling myself off the floor to get through, or I was empowered by the appreciation for life that many don’t get the opportunity to experience. That personified my mental state until my rheumatology appointment, which eventually came despite my impatience.

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She said she thinks it’s safe to say that I do not have interstitial lung disease. I stared at the monitor, confused and feeling like my body was an empty vessel. To someone else, this would be blissful news that confirmed hopes, dreams, and prayers. But for me, it was empty. Because I knew it wasn’t over. I didn’t have a cancer scare from a benign bump. I was still gasping for breath after taking the stairs and clutching my chest in daily pain. Next is my pulmonology appointment to assess to possibility of airway disease, then an echocardiogram to look for congestive heart failure. But in terms of lung involvement alone, I was in such a place of preparation for bad news that I had no emotion for the good news. And here’s the cardinal sin that no one is ever allowed to utter aloud – I almost missed the fear of dying.what gif.gif

Not in a suicidal or dramatic way, but in an invigorating, appreciation-beyond-compare-for-life kind of way. Now, I try my absolute hardest to actively remember that life is short and to be intentional about appreciating all that I have, but before that was assured for me again? I was lit on fire by everything that I saw, tasted, felt, and heard (but not by everything that I smelled, because I have dogs whose farts are out of this world). It felt odd going from a place of emotion and pain and terror and love and loss to “no, you’re fine. Carry on.” But I learned something.

We don’t need the threat of death to keep ourselves aware of life’s joys. Frankly – not to sound pessimistic – but it’s always there. Why do we let something out of our control choose how much time we have left to enjoy? Wouldn’t we want to die knowing that we lived EVERY day, not just those last 3-5 years? Countless autoimmune diseases and chronic illnesses come with fatal complications. This is nothing new to us, and time only threatens us with more inflammation and damage. So we need to strengthen our minds, bodies, and hearts NOW so if that unexplained symptom does become an inevitable fatality, we nod certainly and say, “Alright! At least I know! I’ve been living.” Because I think if we can live life that way every day, we would experience less turmoil and spend WAY less time on the internet out of fear and anxiety over death.

As always, I will update this community with my appointments and results as we go along. For those diagnosed with ILD, I would love to hear your stories and honor your strength. For those going through the testing, I hope this post resonates with you and provides you with some comfort and understanding. For all those readers mentioned and everyone else, please go for a walk and imagine that it is one of the last walks you will take. Note what you see, who you walk with, how you feel, and carry that appreciation and privilege with you for as long as you have on this earth.

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***I would like to add that the Sjögren’s Syndrome Foundation (in accordance with the LAM Foundation) has noted a lack of care and information for Sjögren’s patients who develop ILD and other lung complications, so they have worked to transform 26 existing pulmonary clinics into specific Sjögren’s Syndrome pulmonary clinics!!! This is so crucial and can even lead to longer life spans and more research, so take some time to thank the Foundation and maybe even donate to an effective and powerful cause. You can read more about this endeavor here:

One thought on “Being Tested for Fatal Interstitial Lung Disease with Sjögren’s Syndrome

    CHICK-A-DEE 🥰🤗😍😊😘


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