As many as a third of COVID-19 survivors that did not require hospitalization now face long-term symptoms. A subset of “long-haul” COVID-19 patients have been diagnosed with a form of autonomic dysfunction. To combat this, two University Medical Center physicians — pediatric infectious diseases subspecialist Dr. Ina Stephens and pediatric cardiologist Dr. Dan Schneider — recently established a pediatric autonomic dysfunction clinic in the University Medical Center’s Battle Building.
Postural Orthostatic Tachycardia Syndrome is one form of autonomic dysfunction, a dysregulation of the wiring that controls involuntary functions. POTS is estimated to impact up to three million Americans and can develop following events including trauma to the body, pregnancy, severe infections, hospitalizations and viral infections, such as COVID-19.
“There's lots of reasons for having autonomic dysfunction,” Stephens said. “POTS, orthostatic hypotension … orthostasis, kind of fall under the category [of autonomic dysfunction].”
POTS is characterized by orthostatic intolerance, or the inability to tolerate standing up. The syndrome encompasses a wide range of symptoms, including exercise intolerance, fatigue, fainting, palpitations, blurry vision, lightheadedness, nausea, headache, tremors and impaired concentration or thinking.
“This is not life-threatening — it's enormously debilitating and ruining young people's lives, and I think we can make a difference,” Schneider said.
Although POTS can strike at any age, the clinic serves pediatric patients 21 and under. Autonomic dysfunction is prevalent among teenagers, with as many as one in 100 teens having been diagnosed, often reporting their first symptoms during puberty. While the severity of dysautonomia varies, 25 percent of POTS patients are too ill to attend school or work.
“Missing school seems to be the one that grabbed me the most,” Stephens said. “These kids can't go to school, let alone participate in any activities … and [some] can't sit up without having to be propped during the entire clinic visit.”
Stephens noted that the majority of long-haul COVID patients’ symptoms — such as dizziness, heart palpitations and fatigue — point to autonomic dysfunction. The link between POTS and COVID-19 is not yet fully understood, but researchers propose that autonomic dysfunction could be directly caused by the COVID-19 virus. Alternatively, POTS could be a byproduct of sympathetic nervous system activation during the coronavirus’ cytokine storms, an extreme immune response. The SNS is responsible for the “fight or flight” response, a poorly regulated mechanism in POTS patients.
“The myriad of symptoms that can happen with autonomic dysfunction include so many different bodily systems, from neurologic, to the heart, to the GI tract,” Stephens said. “I was sending out multiple referrals and realized … we need to put a clinic together to take care of these kids.”
Because 41 percent of POTS patients receive their diagnosis from a cardiologist, Stephens and Schneider care for patients in the cardiology clinic space of the Battle Building. The clinic is currently restricted to the two physicians due to time and location constraints, but refers patients to sub-specialists when necessary.
The influx of COVID-19 related POTS patients has increased the demand for specialists, as well as research. In December 2020, Congress allocated $1.15 billion to the National Institutes for Health for research on the long-term effects of COVID-19. In May 2021, Dysautonomia International offered up to $600,000 in research grants to fund studies on POTS and its comorbidities. The organization increased its research grant offering to $800,000 in 2022. Dysautonomia International also successfully advocated for POTS to receive a diagnostic code in the International Classification of Diseases-10.
There is no known cure for POTS, although researchers continue to investigate its causes. Common treatment options for POTS patients include exercise regimens, compression garments, increasing salt and water intake and medications.
Beta blockers and indwelling IV lines are two common POTS treatments that Stephens avoids. Beta blockers may exacerbate fatigue in POTS patients. Indwelling lines only yield short-lived benefits, and risk life-threatening bacteremia. Instead, Stephens and Schneider implement holistic care plans that address patients’ specific symptoms.
“I think the main reason I wanted to do this clinic is because I was really seeing this constellation of the similar symptoms, and it's basically an unrecognized problem,” Stephens said. “I think even just having this clinic is educating other pediatricians.”
Receiving a POTS diagnosis took 10 years for Shannon Linford, who serves as a patient advisory board member for Dysautonomia International and moderates the Virginia and Teens with Dysautonomia support groups on Facebook. Approximately 15 percent of POTS patients receive their diagnosis a decade after their first POTS-related appointment.
“There has never been a time like this in the history of POTS where we need more support,” Linford said. “We desperately need more doctors who understand POTS.”
In addition to diagnostic error, autonomic dysfunction specialists are geographically scattered, some with years-long waitlists. A 2013 survey conducted by Dysautonomia International discovered that half of POTS patients have traveled over 100 miles from home for medical care.
Physician training and increased access to autonomic dysfunction specialists can help to ease the diagnosis process for patients. Dysautonomia International — an organization that promotes research and advocacy for autonomic dysfunction — offers educational resources, including Continuing Medical Education training events, a video library and annual conferences.
The long-term goal for the University’s autonomic dysfunction clinic is to form a roundtable, where multiple specialists can evaluate a patient in a single visit.
“Ultimately, we would love to have a multidisciplinary clinic where we're all there at the same time,” Schneider said. “Hopefully that'll happen in the future, but we have to start somewhere.”
Correction: In a previous version of this article, it was stated that the American Autonomic Society offered up to $600,000 in research grants, and implied that this effort led to POTS receiving a diagnostic code in the International Classification of Diseases-10. This has been changed to reflect that Dysautonomia International offers this grant as well as advocated for the POTS diagnostic code.