POTS Is More Common Than Most Doctors Realize. Here Is Why It Keeps Getting Missed

For many people living with postural orthostatic tachycardia syndrome (POTS), the hardest part is not the dizziness, the fatigue or the inability to get out of bed. It is being told, implicitly or explicitly, that nothing is wrong. Despite affecting far more people than most clinicians realize, POTS remains one of the most misunderstood and dismissed conditions in medicine.

We spoke with Arthur L. Jenkins III, M.D., a board-certified neurosurgeon and complex spine specialist at Jenkins NeuroSpine, about why that is, and what patients and physicians need to understand about the real scope, complexity and treatability of this condition.

Why is POTS so commonly misunderstood or dismissed, and what makes it difficult for clinicians to recognize?

Arthur L. Jenkins III, M.D.: POTS, postural orthostatic tachycardia syndrome, is poorly understood (very little insight and wisdom) and misunderstood (misinformation and “wrogma”) for many different reasons.

First, it overlaps with multiple other conditions, ranging from coronary artery disease to dehydration. It also tends to affect young women who don’t otherwise look ill, and sadly, the fact that they don’t look ill is one of the reasons that empathy is not as triggered in people who see them but don’t notice their suffering. Other conditions also cause light-headedness, dizziness, worse when standing, that are considered to be very rare, and in the common vernacular, when you hear hoof beats, you’re supposed to think “horses, not zebras.” But POTS is a condition that is common, more common than recognized.

Another reason it is poorly understood and misunderstood is that there are different mechanisms by which POTS occurs. There may be hormonal components to this; there are definitely autonomic nervous system components to this condition, and frequently, there are other associated neurologic conditions besides POTS that are often run in conjunction with the POTS because of an underlying dysautonomic state.

Unfortunately, unlike cardiac, spinal and vascular conditions for which many diagnostic modalities can help point the clinician in the right direction, autonomic dysfunction cannot easily be quantified, other than through evaluation of symptoms. This lack of a blood test, an EKG or an MRI that shows the problem makes many people and clinicians doubt that it actually exists. The autonomic nervous system itself is very complicated in its interactions and involves a balance between the “rest-and-digest” parasympathetic nervous system and the “fight-or-flight” sympathetic nervous system. These complementary but nearly opposite neurological networks make up the “yin and yang” of autonomic balance.

At this point, we can’t even quantify which aspect of the POTS symptoms comes from which part of the nervous system. But we do know that both the sympathetic and the parasympathetic nervous systems can be impacted by problems at the junction of the skull and the upper cervical spine. Because this is a very complicated region, many clinicians choose not to study this area because of the potential risks of intervening in one of the most densely packed parts of the body in terms of small vital organs such as nerves, arteries, veins, mouth and esophagus. Everything that happens in the body passes through this area in one direction or another. That seems challenging for clinicians or caregivers to entertain hypotheses as to what is going on, let alone dare to surgically enter this region to attempt therapeutic interventions.

From what you see clinically, how much can POTS affect a person’s ability to work, exercise, attend school or maintain relationships?

ALJ: From what I have personally seen clinically, POTS can range from a minor annoyance to a profoundly disabling condition. I have seen patients who cannot get out of bed at all. I have seen patients so dizzy and dysfunctional from any movement at all, making them completely disabled. And many times, these were patients who were originally the primary caregiver for the family, and then they became so disabled that the family had to rearrange their lives around caring for them.

Fortunately, for many patients, there are treatments that we can do to mitigate the problem, although we have yet to cure it completely. And sadly, not every patient responds to these treatments. So clearly, we have a lot to learn. But, these disabled patients, other than the look of abject misery on their faces, rarely show any signs that would engender empathy, such as skin breakdown, bruising, tumors and the like.

One of the associated conditions that we have found in our patients, as well as in surveys of patients with hypermobility disorders that often precede the onset of POTS, are episodes of transient severe neurologic dysfunctions. These have been called “pseudo seizures,” “drop attacks” and our new term, “transient locked-in syndrome in hypermobility (TLISH).” And once again, because there is no obvious EEG finding with this consistent with a seizure, people assume they’re making it up. But this TLISH, or “pseudo-seizure” activity, can cause almost as much disability as the POTS, because now patients are afraid to go outside or be driving, or riding a bike, or doing any other type of activity for which it might be dangerous to suddenly lose complete control of their body, even if they are still awake for the event.

There appears to be a growing awareness of the connection between POTS and hypermobility disorders like Ehlers-Danlos syndromes. How are these conditions related, and why is it important for patients and physicians to recognize that overlap?

ALJ: The association between POTS and hypermobility is extremely strong. Most patients with POTS have a history of being very flexible when they were younger, often participating in sports or activities for which hypermobility, hyperflexibility, or otherwise having a contortionist ability to move their body in ways nobody else can in their peer group, may have led to excellence in these athletic endeavors such as ballet and other forms of dance, cheerleading, gymnastics, and being able to pitch and throw in ways that other peers cannot in baseball. But later in life, this hypermobility turns to joint instability and neurologic stretching and entrapment syndromes where the nerves pass over or around these joints. In particular, one of the most severe and disabling causes of the dysautonomia that causes POTS is instability at the craniocervical junction, (between the skull base and the second cervical vertebra) where the normal mobility of the cervical spine is already highly mobile, and hypermobile patients move so much that they stretch and damage major nerves and sometimes even arteries and veins that travel to and from the brain.

In addition to POTS, other neurologic and neurovascular entrapment syndromes that hypermobile patients are susceptible to include thoracic outlet syndrome, cubital and carpal tunnel syndrome, and May-Thurner syndrome, among others. We have seen that for many of these patients, there is a “mechanistic association” with worsening symptoms (when the patient moves their head or body in a particular way, the symptoms are triggered), and oftentimes, when that movement is stabilized, prevented, limited or returned to a baseline normal position, the symptoms improve. This helps to establish that there is a biomechanical component to these symptoms, and that gives us the optimism that there is also an associated biomechanical treatment that can ameliorate these symptoms.

“It doesn't help to diagnose a condition for which there is no treatment.” Frequently, clinicians who are not knowledgeable in these treatment strategies will take a nihilistic approach and say, “Why bother evaluating? There’s nothing you can do for this.” Or they approach the idea that “surgery is always bad,” so it should be avoided at all costs, instead of “let somebody who understands the risks, benefits and alternatives fully make those choices.”

We (my research colleagues and collaborating physicians) are working to educate general clinicians and the public that there are treatment options that can change lives profoundly, if implemented, and implemented early in the disease process. These treatments can change and, in some cases, save lives that through neglect or ignorance of the options might have otherwise been consigned to inevitable decline. It is clear that the longer a condition like this goes on, the harder it is to treat, the more extensive the treatment needs to be, and sometimes less impact for the patient.