Drop it like it's POTS

October is Dysautonomia Awareness Month, so it's fitting that I finally got to the bottom of my dysautonomic symptoms just in time. 

Dysautonomia refers to a dysfunction of the autonomic nervous system (ANS), which is the portion of the nervous system that regulates automatic responses - breathing, heart rate, blood pressure, temperature regulation, blinking, digestion. The ANS is broken down into two different components. The sympathetic nervous system (SNS) is commonly referred to as "fight or flight," while the parasympathetic nervous system (PNS) is described as "rest and digest." Both are required to work in coordination with one another for optimal functioning. When there is a dysfunction in either one, a plethora of symptoms can be present. There is currently no treatment for dysautonomia, but with proper diagnosis, the symptoms can be managed. 

For as long as I have been old enough to report my symptoms to my doctors, I remember telling them I was always tired. I was tested for mono pretty much every time I went to a doctor not feeling well, and it was never mono.

Fast forward to my EDS diagnosis, when I began researching my symptoms more under the umbrella of EDS. I quickly realized that my headaches, lightheadness, dizziness, and fatigue were very common in the hypermobility community, and often went along with dysautonomia. I began monitoring my heart rate, first manually and later using a Fitbit, and realized that I was experiencing abnormal fluctuations of my heart rate. My symptoms were often worse when standing up and relieved when lying down. My husband observed that I sometimes would get very flushed and then go pale when I would become fatigued after standing or walking for a while. I began to become very intolerant of heat. Sometimes, exercise would feel great, and other times, it would knock me out for days afterward. My symptoms became unpredictable and quite limiting. 

After passing out during a blood draw at a rheumatology appointment, my wonderful rheumatologist mentioned that it was likely I had dysautonomia, but was unable to diagnose it because of her medical specialty. She added a new medicine to my regimen that helped a lot with my fatigue, but I continued to experience all of my symptoms. So I brought it up to doctor after doctor. I visited with a cardiologist, a neurologist, and endocrinologist, an allergist, my PT. Nobody was able to get to the bottom of it. 

After a horrible appointment with my neurologist, in which he clearly had no idea who I was or what to do with me, he finally agreed to order a Tilt Table Test, which is often used in the diagnosis of dysautonomia. In this test, I was strapped to a table and kept in a flat position for 20 minutes while my heart rate and blood pressure were continually monitored. Then, the table was tilted upright to about 60 degrees, and I was required to remain in that position while my vitals continued to be monitored. Just 3 minutes into the upright position, my blood pressure fell dramatically low and I was on the verge of passing out, only maintaining consciousness because of the IV fluids cranked into my bloodstream. However, I did not return to the neurologist to interpret the results, as by the time he ordered the test I was about to move. 

Finally, after 2 years of no treatment for my many non-orthopedic symptoms, I moved across the country and found myself in the office of a dysautonomia specialist. As I sat in his office filling out forms, I knew I was in the right place. Never before had I looked at an intake form and felt like the questionnaires were so tailored to my symptoms. He spent time talking to me, hearing about my symptoms, and looking at my forms as well as my report from the Tilt Table Test. He concluded that I was definitely experiencing dysautonomia and orthostatic intolerance (difficulty with being in an upright position, most often from lack of blood perfusion to the brain), but was a bit confused with my presentation. My symptoms were consistent with a form of dysautonomia called Postural Orthostatic Tachycardia Syndrome (POTS), while the Tilt Table Test only showed syncope but not POTS. So he brought in a second specialist from the office, and they decided to test me using a system called ANSAR. This test has the ability to monitor both the PNS and SNS responses to regular sitting, various forms of breathing, standing up quickly, and remaining in standing. 

On ANSAR testing, it was apparent that my body responded to the challenge of standing with excess SNS activation, in a way consistent with POTS. Meanwhile, my PNS was not regulating well, and I was experiencing and displaying signs of cardiogenic presyncope, likely as a result of laxity in my blood vessels due to the faulty connective tissue with EDS. My heart rate and blood pressure were right on the edge of the diagnostic criteria, and my new specialists realized my vital signs probably had a blunted response on testing because of the medication that was so helpful with my fatigue, which happens to also be used to treat dysautonomia. They agreed that I was, in fact, experiencing both POTS and cardiogenic presyncope, and developed a treatment plan according to those results.

So finally, after years of dysautonomia, I found doctors who were able to diagnose and treat my issues. I began a new medication and new supplements, received a pair of prescription-strength compression stockings, and began a new exercise regimen to retrain my nervous system to respond more appropriately to physical provocations. My new rest position for immediate relief, recommended by these doctors, involves moving my couch cushions in a way that allows me to lay slightly upside-down to increase blood perfusion to my brain. The relief of this maneuver is immediate and magical! For my exercise regimen, I am required to walk 40 minutes nonstop every day, at a pace slow enough to maintain a level, low heart rate. Meanwhile, for the next 6 months I need to avoid activities that historically have spiked my heart rate, including aerial and intense hikes. 

It is hard. I already miss aerial, and I'm always itching to fly on silks. I wish I could spend my weekends hiking up mountains. But ultimately, I am so relieved and excited that I finally found doctors who are willing to help me. I am already starting to feel some of the benefits since I immediately implemented their suggestions and recommendations. It is so heartening to know that I am finally on the mend, and under the care of some of the top dysautonomia experts in the country. I know that as more research and awareness is brought to EDS, dysautonomia, and the connections between the two, more helpful solutions will emerge. And I trust that these doctors will inform me of those new solutions.