When lockdowns lifted and most of the US began to reopen, many people were cautiously optimistic that we had turned the corner in the pandemic. Now? The optimism is still there, albeit tempered by doubt and uncertainty. Cases in New York are spiking again. The Delta variant, which is more easily transmissible than previous strains of the virus, is driving an increase in hospitalizations. Many people are still unvaccinated, and with school right around the corner, there is currently no approved vaccine for children under 12. We aren’t out of the woods yet, leaving many to ask where we go from here.
That question weighs heavily on the minds of some COVID-19 survivors, many of whom have recovered from the virus only to experience long-term complications. A recent study of nearly 2 million COVID-19 patients found that 23.2 percent had at least one post-COVID condition more than four weeks after their initial diagnosis; that number rose to 50 percent among patients with severe cases. Another study from the journal The Lancet found that as many as 76 percent of people hospitalized by COVID were still experiencing symptoms six months after initial onset. The most common are anxiety, fatigue, and difficulty sleeping. But some patients are experiencing lingering neurological symptoms, and are now receiving a second diagnosis: dysautonomia, the term for a group of neurological disorders estimated to affect more than 70 million people worldwide.
Dysautonomia isn’t actually a condition by itself, but an umbrella term for a number of specific syndromes, including postural orthostatic tachycardia syndrome (POTS), one of the more common types. All forms of dysautonomia present with dysfunction of the autonomic nervous system—the part that controls automatic functions. This means many systems in the body are involved, and there is the potential for a wide array of symptoms.
I was diagnosed with dysautonomia at age 16, although my symptoms began years earlier. For me these include episodes where I pass out—sometimes several times a day—along with nausea that is still uncontrolled even with three medications, and cognitive issues that affect my memory and comprehension. Things like showering, preparing food, and running errands all require extensive planning and periods of rest. My treatments are aimed at reducing my symptoms, getting me to a point where I can function day to day The last decade of my life has been spent navigating a healthcare system that often doesn’t know how to take care of me, forcing me to learn how to advocate for myself and other patients.
As many as half of dysautonomia patients are diagnosed following a viral or bacterial infection. In my case, it was the Epstein-Barr virus. Researchers who study postviral dysautonomia are already finding worryingly high rates of autonomic dysfunction in COVID survivors. With more than 35 million cases of COVID so far in the US alone, and many more to come, even a small fraction of cases leading to dysautonomia will add up to a lot of chronic illness that the healthcare system isn’t currently set up well to treat. Dysautonomia patients often go years without receiving a proper diagnosis, and many find it difficult to get treatment at all.
Living With the Unknown
I interviewed several other people who were diagnosed with dysautonomia prior to the pandemic about their experiences with diagnosis and treatment in order to find out how well equipped—or not—our healthcare system might be to manage the influx of newly diagnosed patients.
JJ, age 26, has been living for almost half his life with a form of dysautonomia called multiple system atrophy, a degenerative condition that affects the body’s involuntary functions, including blood pressure, breathing, bladder function, and motor control. He was initially misdiagnosed with anxiety; more than five years elapsed from the onset of his symptoms until he received the correct diagnosis. “It’s hard to manage something you don’t know the name of,” JJ says. On a day-to-day basis, he deals with a multitude of challenging symptoms, including nausea, dizziness, tachycardia (rapid heart rate), and shortness of breath. He says the doctors he sees are often uninformed about dysautonomia and unsure of how to treat him.
Although dysautonomia is not rare, many patients report that loved ones and even healthcare providers have never heard of it. This is especially true when it comes to the rarer forms, like multiple system atrophy. JJ moved to upstate New York from a neighboring state last year, and he has been unable to locate a provider willing to take over his care. As a result, he has been forced to discontinue several of his treatments, and many of his symptoms have worsened.
There are numerous conditions that commonly occur with dysautonomia, such as small fiber neuropathy and complex regional pain syndrome, both of which JJ is also diagnosed with. Complex conditions require comprehensive and collaborative care, something that isn’t always possible due to lack of continuing education for healthcare providers. But it’s more than that. Many times, patients encounter a provider who is either uninterested or even unwilling to learn about dysautonomia. Doctors are trained to categorize, to triage, and above all, to do no harm. As JJ puts it, “it’s easier when patients fit into boxes.”
Mia, who lives in the Hudson Valley, developed dysautonomia after contracting Lyme disease. (Her name has been changed to protect her privacy.) She began passing out several times a week, but like JJ was initially told her symptoms were mental health-related. “I kept going to doctors, saying ‘something is wrong with me,’ and nobody believed me,’” she says. Mia saw 38 doctors before she was eventually diagnosed with POTS. At that point, more than four years had passed, and she had exhausted most of her life savings.
Now, Mia’s health is more stable, but she still faces many challenges. Most of her care team is located more than an hour from where she lives. She still has to advocate for herself and educate local doctors on her condition. Work has been especially difficult, as employers aren’t always understanding of her limitations and are often reluctant to provide her with necessary accommodations. She worries about the parts of life she’s missed out on because of her illness.
Beverly is another patient living in the Hudson Valley. Her symptoms started around 2007, but she wasn’t diagnosed with dysautonomia until 2015. Her chest pain, dizziness, and shortness of breath were originally written off as stress and anxiety. A former professor, she explains she had to leave her job after teaching became impossible. “I knew something was wrong,” she remembers. “I couldn’t even walk up the stairs.”
Like Mia and JJ, Beverly saw numerous providers before she received answers about her condition. One doctor admitted: “I can’t find anything wrong with you, but we have to keep trying.” After years of trial and error, she traveled to see a provider who was recommended to her by a fellow patient, and together they were finally able to come up with a treatment plan.
While not a cure, treatments have enabled Beverly to better manage her symptoms and reclaim her quality of life. She says continuing education for providers is key, as is readily accessible information for patients. “If I can’t understand it,” she says, “how can anyone else?”
Why Is It So Hard to Get Care?
Beverly, Mia, and JJ are not alone in their struggles. In fact, many dysautonomia patients have difficulty accessing both testing and treatment. In the US, autonomic testing centers—the places best equipped to diagnose dysautonomia—are few and far between. This means patients are often forced to travel in order to get diagnosed and treated.
When providers are unfamiliar with dysautonomia, they often refer patients to places like Johns Hopkins or the Mayo Clinic. These medical centers can be extremely helpful resources, but they aren’t always accessible for patients. Many can’t afford to travel, and in some cases, insurance won’t cover out-of-state care. The only option left is to seek treatment locally and hope to find an informed provider.
New York State is home to some of the top hospitals in the world, yet many patients with chronic conditions still struggle to access informed care. Why is this? What does this mean for patients living in states with less robust healthcare networks? Perhaps more importantly, how can we fix it?
A recent article from the New England Journal of Medicine titled “Confronting Our Next National Health Disaster—Long-Haul Covid” attempts to shed light on possible answers to these questions. It highlights the difficulties faced by patients with chronic postinfection syndromes, and to examine what this tells us about the outlook for long-COVID survivors. “If the past is any guide, they [long-COVID sufferers] will be disbelieved, marginalized, and shunned by many members of the medical community,” the authors write. “Such a response will leave patients feeling misunderstood, aggrieved, and dissatisfied.”
At the core of many challenges faced by dysautonomia patients, and patients with chronic conditions in general, is often a lack of research funding and awareness. Without adequate funding, there are fewer research studies conducted, fewer fellowships created for providers, and ultimately, fewer answers and treatments available for patients. That is one reason why the average length of time from symptom onset to diagnosis for someone with POTS is two to five years, sometimes longer. Patients sometimes go into debt during the years they spend seeking diagnosis, and even then, treatments are still often inaccessible or not readily available.
That often leaves patients to be their own advocates when it comes to finding treatment. Alice is a nurse working in the Hudson Valley with more than 20 years of experience across multiple settings. (Her name has been changed to protect her privacy.) Over the last few years, she has cared for several patients with dysautonomia. She emphasizes the need for individualized care, especially for patients with chronic or complex conditions. More than anything, “you have to be able to listen to the patient,” she says. “As a patient, you are your own best advocate.” Sometimes, a provider seeing one patient with a condition will give them a push to do research, and open the door for future patients.
Lauren Stiles is intimately familiar with the challenges faced by dysautonomia patients. She is the president of Dysautonomia International, a nonprofit organization that seeks to identify the causes and cures for all forms of dysautonomia and to enhance the quality of life of people currently living with these illnesses. She told me about some of the ways the organization does this: maintaining patient and medical advisory boards, organizing and overseeing fundraising events to help fund research, hosting a yearly conference where healthcare providers and other experts can share findings directly with patients and families, curating their website with up-to-date information about the various forms of dysautonomia, and promoting continuing education for medical practitioners. Both last and this year’s Dysautonomia International conferences included presentations on the research that is being done to investigate the relationship between dysautonomia and long COVID.
On Hope and Moving Forward
There are many steps that can ensure competent and informed care for current and future dysautonomia patients. Large-scale investment in research and a focus on spreading awareness at all levels of the healthcare system—including in medical schools—are key. Tools such as the COMPASS-31 questionnaire can help providers screen for dysautonomia quickly and help prevent diagnostic delays.
A recently announced National Institutes of Health initiative is a promising step in the right direction. Congress has allocated $1.15 billion to investigate complications of COVID, including autonomic dysfunction. The fact that dysautonomia is more commonly on the radar of renowned medical experts is also promising. Dr. Anthony Fauci recently mentioned dysautonomia in a Facebook live event where he discussed the long-term effects of COVID.
I asked Beverly, Mia, and JJ about the best way to help dysautonomia patients going forward.
Beverly emphasized the need for patient support systems, as well as patient-provider collaboration. Mia addressed the medical community directly: “Listen to your patients, believe them.” JJ’s answer was similar: “It starts with believing us.”
Long COVID is now considered a disability under the Americans with Disabilities Act. Find out more here.