My POTS diagnosis: worth the struggle
Updated: Dec 27, 2022
I have Postural Orthostatic Tachycardia Syndrome (POTS), a common neurological manifestation of Sjogren’s. Getting a diagnosis and treatment was difficult, but it changed my life for the better. POTS and other autonomic disorders (dysautonomia) are common in Sjogren’s patients (33, 50). Because few clinicians are familiar with dysautonomia, symptoms tend to be dismissed or blamed on anxiety, functional neurological disorders, or fibromyalgia.
My story demonstrates how proper diagnosis can significantly improve quality of life.
While sicca (dryness) is the most well-known symptom of Sjogren’s disease, patients rarely name it as their top concern. Extreme fatigue, pain, and cognitive impairment (brain fog) usually have the most impact on quality of life (169). Increasingly, research points to dysautonomia and small fiber neuropathy as important contributors to these sometimes-debilitating symptoms (181, 184). Sjogren's patients who experience excessive fatigue, lightheadedness, dizziness, fainting or near-fainting, standing intolerance, brain fog, or other symptoms typical of dysautonomia should be evaluated for autonomic disorders. To learn more about symptoms, see the POTS page and Table 2 of this article (236).
My POTS Story
Starting in my late teens, I experienced both POTS and Sjogren's symptoms. I had years of unexplained fatigue, lightheadedness, extremely dry eyes, muscle aches, and stiffness. Managing my sleep, exercise, diet, and stress helped me function. This worked until it did not. At age 44 my lightheadedness and fatigue suddenly became so extreme that I could no longer drive my children to school or even walk around the block without feeling like I might faint. I went from being an engaged parent, physician, and athlete to mostly bedridden, for no obvious reason. I was devastated.
Many tests and specialty consultations over the next year did not reveal a diagnosis. When standard tests all came back normal the specialists would tell me that nothing was wrong. One doctor suggested I needed to distract myself and find a hobby. None of my doctors suspected Sjogren’s or POTS, even though I had classic symptoms of both. Dry eyes and joint pain eventually led to a Sjogren’s diagnosis, although I had to advocate for this with my rheumatologist because I was negative for the typical autoantibodies. Sjogren’s treatments improved my joint pain and stiffness, but I was still left with debilitating fatigue, difficulty concentrating, and was unable to stand for more than 10 minutes without getting lightheaded.
How did I convince my rheumatologist to treat me for presumptive Sjogren’s? I used strategies like the ones explained on the Clinician Handouts page.
How I ended up in the Emergency Department
After seven years of debilitating fatigue and brain fog, I started to feel so lightheaded that I could not stand for more than a few minutes. When it got to the point that I had to crawl to the bathroom, I finally went to the emergency room, a place I try to avoid at all costs. My heart rate while sitting upright was 150. I was unsteady and sick, but I was composed. The emergency doctors assumed I was having a panic attack, which I told them was unlikely, as I did not feel anxious. My symptoms improved somewhat after IV saline. This was a clue to my POTS diagnosis that none of us understood at the time. My heart and lungs were fine, and I was told to follow up with primary care.
There was no sense of urgency from my doctors, no curiosity expressed about why I was so incapacitated. They ran the obvious tests but came up empty. Once they figured this was unlikely to kill me, I was on my own. My doctors admitted that they simply did not know how to help me.
It was 2009. I had to diagnose myself with POTS.
Desperate for answers, I started searching the medical literature from bed, despite my brain-fogged state. My research led me to POTS and its relationship to Sjogren’s. I devised a home standing test, and … bingo. The results indicated I had POTS. I was relieved to learn what was wrong after suffering mysteriously for so many years. I was also beyond disappointed that I had to diagnose myself. That should not have happened. I am resolved to raise awareness among patients and doctors so that others are spared my ordeal. My doctors agreed I had POTS after learning about it from me, yet for three months I was referred from doctor to doctor since no one wanted to manage a problem they were unfamiliar with. I implemented lifestyle measures that I learned about online, but I still needed medication. Finally, I found a neurologist who was willing to work with me, and together we chose treatments after I copied and highlighted journal articles for him.
I still have POTS, but learning to manage it let me reclaim precious parts of my life. I started attending many of my children’s school and sports events again, although by that time one of them was in college. My exercise tolerance increased. I can now ride my bike for 30 minutes, something that brings me joy. Treatment helped clear my head, allowing me to concentrate enough to work on Sjogren’s Advocate for a few hours most days. Standing still for too long still makes me feel shaky and lightheaded; I have learned to do “blitz errands” at off hours to reduce this problem. POTS requires constant management, but treatment has profoundly improved my quality of life.
While awareness of dysautonomia and its association with Sjogren’s is on the rise, there is still work to be done. Many patients still struggle to get an accurate diagnosis and appropriate treatment. I will soon post a link to a webpage that demonstrates how to use a new guide for assessment, testing, and treatment to advocate for dysautonomia care with your clinicians.
A special thank you to Gillian Freney for the helpful comments and edits.