As much as a third of COVID-19 survivors who did not require hospitalization are now facing long term symptoms. A subset of “long-haul” COVID-19 patients have been diagnostic with some form of autonomic dysfunction. To combat this, two physicians at the University Medical Center – pediatric infectious disease subspecialist Dr. Ina Stephens and pediatric cardiologist Dr. Dan Schneider – recently established a stand-alone Pediatric Dysfunction Clinic in the medical center’s Battle Building. university.
Postural orthostatic tachycardia syndrome is a form of autonomic dysfunctiona disruption of the wiring which controls the involuntary functions. POTS is estimated to have an impact of up to three million Americans and can grow next events including bodily trauma, pregnancy, serious infections, hospitalizations and viral infections, such as COVID-19.
“There are lots of reasons for having autonomic dysfunction,” Stephens said. “POTS, orthostatic hypotension…orthostasis, sort of falls into the category [of autonomic dysfunction].”
JARS is characterized by orthostatic intolerance, or the inability to tolerate standing. The syndrome encompasses a wide range of symptomsincluding exercise intolerance, fatigue, fainting, palpitations, blurred vision, dizziness, nausea, headache, tremors, and trouble concentrating or thinking.
“It’s not deadly – it’s extremely debilitating and life-destroying for young people, 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 in adolescents, with up to one in 100 teenagers having been diagnosed, often reporting their first symptoms during puberty. Although the severity of dysautonomia varies, 25 percent of POTS patients are too sick to go to school or work.
“The missing school seems to be the one that grabbed me the most,” Stephens said. “These children cannot go to school, let alone participate in activities…and [some] cannot sit without being braced for the entire clinic visit.
Stephens noted that the majority of long-distance COVID patient 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 to 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 coronavirus’ cytokine stormsa extreme immune response. The SNS is responsible for the “fight or flight» answer, a poorly regulated mechanism in POTS patients.
“The myriad of symptoms that can occur with autonomic dysfunction include so many different bodily systems, from the neurological to the heart to the gastrointestinal tract,” Stephens said. “I was sending out several referrals and realized…we needed to set up a clinic to take care of these kids.”
Because 41 percent POTS patients receive their diagnosis from a cardiologist, Stephens and Schneider attend to patients in the Cardiology Clinic space of the Battle Building. The clinic is currently limited to two physicians due to time and location constraints, but refers patients to sub-specialists as needed.
The influx of patients with COVID-19-related POTS has increased the demand for specialists, as well as research. In December 2020, Congress awarded $1.15 billion to the National Institutes for Health for research into the long-term effects of COVID-19. In May 2021, the American Autonomic Society offered up to $600,000 in research grants to fund studies on POTS and its comorbidities. These efforts have led to advocacy victories, such as POTS receiving 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 regimenscompression garments, increase salt and water intake and medications.
Beta-blockers and indwelling IV lines are two common POTS treatments that Stephens avoids. Beta-blockers can exacerbate fatigue in patients with POTS. Indwelling lines provide only short-lived benefits and may be life-threatening bacteremia. Instead, Stephens and Schneider implement holistic care plans that address specific patient symptoms.
“I think the main reason I wanted to do this clinic is because I was really seeing this constellation of similar symptoms, and it’s basically an unrecognized issue,” Stephens said. “I think even just having this clinic educates other pediatricians.”
Approximately three out of four POTS patients are initially misdiagnosed, and although 94 percent of POTS patients are women, they face longer diagnostic delays than their male counterparts.
Receiving a POTS diagnosis took 10 years for Shannon Linford, who is a member of the patient advisory board for Dysautonomia International and moderates the Virginia and Dysautonomous adolescents Facebook support groups. Approximately 15 percent of POTS patients are diagnosed a decade after their first POTS appointment.
“There has never been a time like this in POTS history where we need more support,” Linford said. “We desperately need more doctors who understand POTS.”
In addition to misdiagnosis, dysautonomia specialists are geographically dispersed, some with waiting lists of years. A 2013 investigation conducted by Dysautonomia International found that half of POTS patients traveled more than 100 miles from home for medical care.
Training of doctors and increased access to autonomic dysfunction specialists can help ease the diagnostic process for patients. Dysautonomia International – an organization that promotes the research and defense of autonomic dysfunctions – offers training Resourcesincluding Continuing medical education training events, a video library and annual conferences.
The long-term goal of the University’s Autonomic Dysfunction Clinic is to form a round table, where multiple specialists can assess a patient in a single visit.
“Ultimately, we’d like to have a multidisciplinary clinic where we’re all there at the same time,” Schneider said. “I hope that will happen in the future, but we have to start somewhere.”