Is POTS the Grinch Syndrome?

Dr. Levine responds to the controversy

WOMAN EXPERIENCING DIZZINESS
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In 2010, investigators from the University of Texas Southwestern Medical Center published an article on the cause of postural orthostatic tachycardia syndrome (POTS). The article engendered a significant amount of controversy and anger among many sufferers of POTS, because of the widespread interpretation of its results, and because of the new name the authors proposed for this condition — Grinch Syndrome.

The outcry, in fact was so great that the chief author of this study, noted POTS researcher Dr. Benjamin Levine, agreed to this author’s request to comment on the issue publicly. His response appears below. 

About POTS

POTS is a condition in which a person’s heart rate increases to high levels when they stand up. It is often accompanied by severe lightheadednesspalpitations, and sometimes by syncope. POTS, which can become extremely disabling, has many aspects similar to various dysautonomias. It is most often seen in young, otherwise entirely healthy people, and in women much more often than in men.

The Study

Dr. Levine’s team studied 27 people referred to Southwest with POTS. The chief findings were: 1) None of the subjects had measurable abnormalities in their autonomic nervous system. 2) Many (about 70%) had small hearts and low blood volume, findings characteristic of deconditioning. And 3) On average, subjects responded very favorably to a prolonged, graded program of exercise therapy.

The authors concluded that POTS is "per se" a condition caused by deconditioning. Because of the small hearts they noted in most of their subjects, the authors proposed (likely with tongues in cheek) renaming POTS to Grinch Syndrome (because grinches, according to definitive sources, have small hearts).

The Reaction of POTS Sufferers

Newspapers around the country jumped on this article when it was published, keying on two points: the cool new name Grinch Syndrome, and the idea that POTS is self-induced by leading a sedentary life. This widespread mirepresentation of the study triggered an impressive reaction from POTS sufferers around the world.

People dealing with POTS took to social media in great numbers to complain about this study. In addition to the obvious desire of not wishing to be referred to as grinches, they asserted that attributing all cases of POTS to deconditioning is not only wrong, but likely harmful to POTS sufferers.

It is undoubtedly true that the vast majority if not all people with significant POTS are deconditioned. You would become deconditioned too if you could not stand up without becoming extremely lightheaded, or passing out. And the study indeed confirmed that this is the case.

But the fact that patients with POTS might be shown to have deconditioning does not prove (and in fact, it does not even strongly suggest) that deconditioning is the cause of POTS, rather than the result.

People who are diagnosed with POTS already have an extraordinarily difficult time getting their doctors and families to take their symptoms seriously.

They often go through a prolonged ordeal of being told by several doctors that they have anxiety or depression or some sort of conversion reaction rather than a true, physiologic condition. It is often weeks or months, and occasionally years, before some physician finally figures out the true diagnosis.

And now, even after the correct diagnosis is made, they face the prospect of being regarded as having brought the condition on themselves, due to "deconditioning," which is often medical talk for being too sedentary, passive, and lazy. And when their family is told that the problem is self-induced, via the mechanism of sloth, and is told this by a doctor no less, the amount of support POTS sufferers are likely to receive from loved ones, at the very time they are fighting to recover and are most in need of it, will predictably diminish.

So the hue and cry from POTS sufferers when this article was published is quite easy to explain. 

Dr. Levine Responds

Dr. Levine was moved to respond to the distress expressed by so many POTS sufferers after his study was published. In doing so he made three major points:

 

1) Promotion of Research Findings. ​First of all, let me reassure you that I have never "promoted" our work in any media outlets, and neither has anyone else in my institution. Frankly, I am not a big fan of media reporting of scientific data, and although I will speak to reporters when they call if I can provide any unique expertise, I do not search it out. As I am sure you know, we have no control over what reporters say about our work. Our audience for our papers is other physicians and scientists, who must interpret the data in light of their own experience and read of the literature. I caution all patients who must get their information from the media not to over-interpret what they read in the press or see on TV.

 

2) POTS is not caused by "laziness." Most of the patients I see were very high functioning (like our astronauts, upon whom we modeled our POTS training program) prior to something happening — for some it is a viral infection; others an injury or other illness; some it is pregnancy or the complications of delivering a baby. This "sentinel event" causes one common thing to happen — it puts people to bed.

 

It is important to emphasize that even 20 hours of bed rest can induce loss of plasma volume, impaired baroreflex function, and orthostatic light-headedness in many people. After only 2 weeks of bedrest or spaceflight, nearly 2/3 of people can't stand for 10 minutes!

 

It is also worth noting that earlier studies from our site showed that it was the most athletic subjects who were most impaired by even short duration (3 weeks) bed rest, with very long recovery periods. In fact, the volunteers who were least fit before going to bed, recovered relatively quickly in response to subsequent controlled training, generally within 2 weeks. On the other hand, the most athletic volunteers, after 3 weeks of bed rest and 2 months of intensive training, still had not recovered to their baseline fitness. Intriguingly, we studied the same subjects 30 years after the original study and found that, amazingly, not a single person was in worse shape 30 years later, than they were after 3 weeks of bedrest in their 20s.

 

In other words, 3 weeks of bedrest was worse for the body's ability to physical work than 30 years of aging! (see McGuire et al Circulation 2001).

 

Regardless of what caused the initiating event however, by the time the illness (or whatever happened) has run its course, the patients are left with profound disability (one patient in our study had not been able to even sit upright for more than 2 years) that seems to get better with our dedicated, focused, gradual exercise program beginning in the semi-recumbent position. The latter nuance is perhaps the key new wrinkle we have brought to the table in taking care of POTS patients.

 

Most POTS cannot tolerate the upright posture so starting sitting down or even reclining backwards is critical to their success. And it is hard! Many patients struggle to complete the first few weeks of the training program, which by the way begins with only 30 minutes per day, 3 days per week. But if they push through the initial discomfort, they usually find themselves gradually feeling better and stronger.

 

This highlights a critical point — We have never felt that a single POTS patient was lazy or irresponsible — if it were easy to treat POTS with exercise, everyone would do it!! However when we apply a highly specific training program focused on making the heart bigger, the vast majority of patients feel dramatically better and many are "cured", meaning only that they no longer meet the criteria for POTS. I should emphasize though that maintaining fitness is a life-long goal for our POTS patients and we encourage them to consider exercise as part of their personal hygiene. For those patients who have heard me speak, either privately to them as patients, or publicly in lectures, they know that I STRONGLY emphasize that "cardiovascular deconditioning" is a real and well-described process that has nothing whatsoever to do with laziness.

 

Let me re-emphasize this point: CARDIOVASCULAR DECONDITIONING IS NOT THE SAME AS "JUST BEING OUT OF SHAPE"!!!!! Anyone who interprets our data that way is misinterpreting our findings and our therapy. I have great sympathy for our patients many of whom are extraordinarily debilitated, and I have been frustrated for much of my career trying to find therapies that work consistently.

 

3) Regarding "Grinch Syndrome." Finally, let me finish with a few words about the Grinch. The vast majority of our patients who hear the term "The Grinch Syndrome" laugh and take comfort in the spirit in which it is presented. On the other hand, I understand that some patients who don't know me, and are concerned with being labeled by a name that is associated with being mean spirited, object to the use of the term Grinch Syndrome, and I respect those concerns. Frankly, if I had to do it over again, I probably would have been more reluctant to use the term, and I want all POTS patients to know that we mean them no ill will. There is no "fame and fortune" associated with this name and I get no personal benefit from using this term. However, I have seen how it is very effective at calling attention to what we have observed as the primary pathophysiology associated with the extreme incapacitation of these patients. As I often tell my patients, the term "POTS" simply puts a label on the fact that the patient's heart beats fast when they stand up. The "Grinch Syndrome" focuses attention upstream, on WHY the standing heart rate is so high — namely that the heart is "two sizes too small."

 

Based on simple population statistics, 2.5% of all the women in the world will have a heart that is more than two standard deviations below the mean -- the definition of "normal" in medicine. We believe that it is these women who are most at risk of getting POTS symptoms which can be induced incredibly quickly by even a brief period of bedrest.

 

Ultimately, though, I would like to emphasize that humor is a powerful tool for healing, not only for patients with POTS, but almost any disease. We treat all our patients with the deepest respect and are acutely aware of how debilitated they are, which we take very seriously. Mark Twain once said that "Humor is mankind's greatest blessing"; we believe it is an essential part of the healing process and hope that all of our patients smile when they think of The Grinch, and then go off to exercise!

 

  • Dr. Benjamin D. Levine”

A Word From Verywell

Dr. Levine is a dedicated and careful researcher, and his body of work on POTS has advanced our knowledge of this condition significantly, and as a result, thousands of people with this condition are being helped. 

It is unfortunate that many individuals — within not only the media but within the medical profession — were led to misinterpret his study as indicating that POTS is somehow a self-induced disease. Dr. Levine's elaboration on that point clearly indicates that it was never his intention to suggest that POTS is self-induced. Rather, his findings indicate that a relatively brief period of enforced bed rest, perhaps especially in some highly active people (usually women), may be enough to bring on this condition.

In any case, people with POTS who need to convince their doctors and family that their condition is not something they brought on themselves, now now have a statement from the horse’s mouth showing that POTS is something that just happens to people.

Sources:

Freeman R, Wieling W, Axelrod FB, et al. Consensus Statement on the Definition of Orthostatic Hypotension, Neurally Mediated Syncope and the Postural Tachycardia Syndrome. Auton Neurosci 2011; 161:46.

Fu Q, VanGundy TB, Galbreath M, et al. Cardiac Origins Of The Postural Orthostatic Tachycardia Syndrome. JACC 2010;55:2858-68.

Kimpinski K, Figueroa JJ, Singer W, et al. A Prospective, 1-year Follow-up Study of Postural Tachycardia Syndrome. Mayo Clin Proc 2012; 87:746.