What a Real POTS Evaluation Looks Like and When to Stop Managing It Alone

Getting a postural orthostatic tachycardia syndrome (POTS) diagnosis is only the beginning. For many patients, the harder questions come next: What tests actually matter? Who should they see? What is real medical guidance versus noise on social media? And when conservative management stops working, what comes next?

Arthur L. Jenkins III, M.D., a board-certified neurosurgeon and complex spine specialist at Jenkins NeuroSpine, offers candid answers on what a proper evaluation looks like, how to navigate a landscape full of misinformation and what newly diagnosed patients most need to hear about protecting their long-term health.

What does a proper evaluation for POTS actually involve, and what symptoms should prompt someone to seek a specialist rather than continuing to self-manage?

ALJ: A proper evaluation of POTS starts with recognizing that it is both common and a treatable condition. Findings consist primarily of an elevation of the heart rate, often with an associated lightheadedness or dizziness, and in many cases a drop in blood pressure when going from sitting to standing or lying down to sitting up.

A basic history must also be taken, starting with: How often do these episodes happen? How long has this been going on? Is the condition getting worse? Is there some progression to the symptoms? Are there other associated symptoms that occur either at the same time, immediately before or after these events? Has the patient ever been injured because they fell down as a result of this? Is there any history or family history of heart disease, other neurovascular conditions, seizures or trauma? This is only the beginning of that evaluation.

One would also want to perform a basic Beighton scale [a scale that looks at the presence of hypermobile joints in the body, where a score of 5 is usually found in patients with hypermobile Ehlers-Danlos syndrome] evaluation to determine the degree of hypermobility. And there are a few other signs and symptoms that we can check for. My two favorites are the thumb, forearm proximity and the “reverse namaste” maneuver because those are easily demonstrated either on a video or in-person evaluation.

Additional testing of blood electrolyte levels to make sure that there is not a metabolic issue. More advanced testing, including EKG [electrocardiogram], Holter monitor, tilt test, MRIs and CT scans of the brain and cervical spine. And in some cases, CT angiography or MR angiography of the head and neck in different positions, and even digital subtraction angiography, can all point to different aspects of the condition, and conditions that cause POTS and POTS-like symptoms. In addition, an investigation into a history of COVID exposure may also be relevant.

Many researchers now believe that there is an autoimmune component to the ligamentous instability that leads to hypermobility, craniocervical instability and autonomic dysfunction. Even my nonhypermobile patients who had cervical trauma frequently also report COVID exposure before the onset of these symptoms. It is believed that the alar ligaments in the upper cervical spine/skull region, in particular, may be related to the craniocervical instability dysautonomia, although this has not yet been proven, and we don't have an absolute test to look for those damaged ligaments.

Social media has increased awareness of POTS, but it has also created confusion and misinformation about diagnosis and treatment. What are some of the biggest misconceptions you are seeing online right now?

ALJ: I would say the biggest disinformation that's out there is these pseudo-influencer people who exemplify the Dunning-Kruger phenomenon [where the less the person understands the problem, the more confident they are in the accuracy of their opinions]. Frequently, these people are espousing ideas or treatments that have not been validated, other than in their limited experience, sometimes only one or two case scenarios, and they feel they can generalize their experience to preach to people broadly. There's a big difference between "…this worked for me" and "…this has worked for hundreds of my patients."

But in this modern “TikTok society,” it’s easy to garner “likes” and “eyeballs” if you are saying something definitively and confidently (even without any background check or factual basis for their confidence in their recommendations). There are so many desperate people looking for answers. Sadly, there's no limit to what an unscrupulous influencer will peddle to desperate people, including over-the-counter treatments, peptides and other quackery. In addition, their insistence on avoiding organized medicine has created a counterculture movement that generates a general distrust of surgeons and/or actual organized researchers, creating more harm than good.

By the same token, social media has created a platform where people like this can associate. I know there are many Facebook support groups for POTS, dysautonomia and hypermobility syndromes, and I can imagine Reddit and other media have just as many places. As long as people are searching for answers, social media does give them a platform in which to sit around and commiserate. The issue becomes that one doesn't want to confuse hopeful optimism with clinically validated guidance.

Even the most experienced surgeons and clinicians who treat these patients don't always agree on the best way to work up and intervene. And artificial boundaries like academic “moats,” the challenges of organizing multisite research collaborations, and sometimes even the cost of doing research — as opposed to just continuing to treat patients the way you think is appropriate — are all barriers to actually advancing the science, rather than just one or two clinicians' particular arts. True research evaluates, in an iterative process, every intervention and every change in an intervention to validate its impact on the outcomes. There have definitely been times in my research where I have identified things that we thought would work and did not, and there have definitely been times that we've identified interventions that had a much bigger impact than we thought they would.

So some of this is misinformation, some of this is a lack of information and some of this is the challenges of getting everybody to “play the same tune at the same time.” Unfortunately, we don't have a single conductor whom every “musician” is able to follow in this field.

For someone newly diagnosed with POTS who may feel overwhelmed or frightened, what are the most important things they should know about symptom management, lifestyle adjustments and improving quality of life safely over time?

ALJ: The most important thing for any patient who believes they have POTS and is looking for ways to manage, mitigate and maneuver within this condition is that this condition tends to get worse over time. Early in the process, the symptoms can be managed by improving fluid intake and preventing dehydration, because [dehydration] will make the symptoms worse by decreasing blood volume. But as the symptoms themselves and the condition itself get worse, there is less and less that somebody can do without more invasive interventions.

There are drugs that people can take that will help keep the pressure higher. And there are drugs that people can take that will reduce the heart rate bump. Normally, everyone’s heart rate goes up when you stand up, and people with POTS have a much more dramatic rise in the heart rate and, sometimes, a drop in blood pressure. But as the condition advances, those treatments, those medical treatments and those homeopathic treatments lose their relative efficacy.

At the point where you are unable to participate in the activities that are important to you, whether that be being a productive member of the family, participating in sports that are important to you, working or, in some cases, even getting out of bed, that’s when you need to take the treatments up a level of intensity and start thinking about surgical solutions. It's always better to intervene earlier rather than later to avoid the chronic damage to the nerves that comes with repeated trauma from the regional or global hypermobility. Many patients get by wearing a neck collar of some kind or another. And while we have particular protocols for evaluating patients for neck collars, neck collars themselves are not a panacea or an end treatment. They are an interim step in the diagnosis and a temporary relief, but not a long-term option because the more you use a collar, the weaker your neck muscles get, and the less stability you have in your neck at baseline.

I would also caution in general against the use of Botox on any cervical muscles — this is not the same as doing Botox for thoracic outlet syndrome-related muscles, the scalenes. Weakening the very muscles that are providing neck stability has the potential to make the condition worse, even if a well-intended doctor is trying to convince you that the neck pain that is often associated with this may respond to Botox. There's a big difference between Botox for migraines (treating the perifacial muscles) and weakening the inherent stability of the cervical spine at a time when the spine is already under attack.

Find one of the EDS Centers of Excellence, and connect yourself with the Ehlers-Danlos Syndrome Society webpage. And recognize that sooner is better than later to intervene, but intervention needs to be weighed against the risks of the procedure, the known consequences of the procedure, and in full awareness of what alternatives are available. Surgery is neither risk-free nor a guarantee of a cure. But in the right hands and in the right patient, it can lead to a profound improvement in quality of life.

Have hope, never give up and never stop advocating for yourself. Just because the person you're talking to now doesn't understand you, it doesn't mean that nobody will understand you. And just because we may not have a specific treatment for you today does not mean that one, five or 10 years from now, there may not be a better way of doing it. Know that there are those of us out there who will listen and provide, at the very least, care until there is a cure.