POTS makes the sports section: It must be real!

If you ever visit my office it will be apparent that I’m a sports fan, and particularly a hockey fan. The main artwork in my waiting area (or as we call it, the loving room) is a signed lithograph by former Rangers captain Jacob Trouba. My exam rooms are numbered with iconic New York uniform numbers: #1 for the Rangers Ed Giacomen; #2 for Derek Jeter, of course; and #3 is a Knicks jersey number that can honor my favorite Knick ever, John Starks, or Josh Hart, who embodies so much of what I loved about Starks and also hails from my alma mater (as so many Knicks do).

My family has had Rangers season tickets for a dozen years. We’re on our second rebuild (sorry, “re-tool”), so many of my favorite players are scattered across the league. As time marches on, they’re also in broadcast booths, or out of the sport altogether.

But there’s one player I’m rooting for, even though he’s never been a Ranger and I’m not quite sure I’ve ever even paid close attention if I’ve seen him play at the World’s Most Famous Arena. But there’s a good reason that he matters to folks who do what I do for a living.

Early last summer while skimming the sports news in The Athletic - the website I gladly subscribe to and can least live without - I saw an article that caught my eye, not as a hockey fan but as a Cardiologist.

“(Nashville) Predators say captain Roman Josi will play next season despite POTS diagnosis.”

I admired the optimism of the article but was taken aback about it at the same time. Having cared for a number of POTS patients over the past decade, and incrementally more in the past year since starting this practice because the format affords me the time and bandwidth to properly manage these cases, I have come to accept the fact that you can’t put a time stamp on recovery from POTS as you can from, say, an ACL repair.

POTS stands for Postural Orthostatic Tachycardia Syndrome. Folks suffering from POTS have a chronic inability to tolerate being upright due to lightheadedness, weakness and brain fog. The Mayo Clinic definition requires three months of essentially total debilitation in order to be invited into the POTS club. It’s not a club you’d like to join.

POTS can occur without provocation but it is often triggered by a virus (COVID, mononucleosis, among others), emotional stress or head injury (which was probably the case for Josi). It can be related to inflammatory and immune conditions too. It’s a nasty, sneaky problem.

For most individuals suffering from POTS there is no clear light at the end of the tunnel. Symptoms can last for months, or years, or longer. Most people can find their way out of the mire, but some can be affected for much of their lives. It’s one of the truly frustrating aspects of the condition.

Another source of exasperation is the cloudy diagnosis and the unclear ownership of POTS by any medical specialty. Here’s a general rule about medical conditions that end with the word “Syndrome”. If the word follows a person’s name – Marfan Syndrome, Turner Syndrome, etc. – then that person identified and defined a specific condition and you can expect that there are clear diagnostic criteria for it. Whatever specialty the namesake practiced will probably feel responsible for its management.

If “Syndrome” trails behind not a proper name but a description, then all bets are off. It’s a designation that says “there are a bunch of symptoms and signs that can be associated with this thing but everybody is a little different and there’s no specific test so it’s always going to be sketchy.”

Postural….Orthostatic…Tachycardia…………………..SYNDROME.

Literally “When People Stand Up Their Heart Goes Faster…….Syndrome.” (Hint: everybody’s heart rate rises when they stand up, but some more than others and an unlucky few of those people are just wrecked by it).

Many patients with POTS symptoms (and 10 years ago that would be “most”; 20 years ago “just about all”) find their complaints doubted by medical professionals, school personnel and the rest of their community for weeks or months or longer. They keep missing great gobs of work or school because they don’t have the energy to attend. If they manage to drag themselves out, they’re often pretty much wasted for the day by the time they arrive. But their symptoms are all subjective. Are you having a fever? No. Diarrhea? Not really? Cancer? Anemia? Turning into a werewolf every night and then back, but really tired from being a werewolf because werewolves don’t sleep? No to all of those.

They’re assumed to be malingerers. It’s whispered behind their backs that they’re obviously faking. They must be just avoiding school, despite the fact than in general POTS patients tend to be high achieving, type A compulsive individuals before the illness. They shuttle from doctor to doctor, from specialist to specialist. It’s not the heart. It’s not the brain. It’s not an allergy. It’s not an infection. It’s none of those things, and all of those things at the same time. It’s just……you.

And the parent who’s dragging this teen from doctor to doctor, hospital to hospital, missing work, giving up life, consumed by seeing their previously ultra-lively offspring become a miserable dishrag before their very eyes might just be rewarded with a title. Not “Parent of the Year.” It’s “Munchausen by Proxy.” Yes, I have known parents who have been reported to social agencies – some more than once! – by well-meaning medical people who assume that they are doing something to make their child sick, or psychologically abusing them by convincing them to play half-dead to avoid taking the SAT.

My first POTS patient – though I didn’t recognize her as that at the time - was just like this. Mom showed up leading with the proclamation that her daughter would be in the National Honor Society if she could just get to school, which she hadn’t been able to do for 8 months. Hoo Boy! This lady must be nuts! She’s convincing her daughter to bail on her junior year rather than take the risk of falling short of Mom’s high bar for her. But they stuck with their story, and on at least two occasions that I remember, Mom was indeed reported to social work for her persistence. Something made me believe her and eventually I sent her to the Mayo Clinic in Minnesota. I actually think it was Mom’s idea but I like to take the credit. She came back two weeks later with a binder full of information on this POTS thing, which had been described since 1993 but still wasn’t widely known or believed. We followed Mayo’s treatment outline (jagged and rudimentary as it was) religiously. Gradually the girl dug herself out of the hole. Eventually she went to Syracuse (a year or maybe 2 after her original graduating class), nailed college life, attended law school and taught everybody a lesson. She was EXACTLY the person her mother said she was way back when I saw her for the first time.

I believe them all now. Someday, maybe one of these families will be pulling the wool over my eyes. That’ll be somebody else’s job to figure out. We can’t start their journey toward recovery until we believe they’ve got something to recover from.

Here’s another thing I have to say about POTS, and it’s very important. A few years back, Phil Fischer at Mayo, who’s one of the real champions of the condition, made the observation that all POTS patients will increase their heart rate (without dropping their blood pressure, incidentally, like a dehydrated person would) by 35 beats (40 if they’re an adolescent) within 10 minutes of standing up from lying down. It’s true. But so will a lot of other people. It’s a diagnostic necessity, but that heart rate change is NOT diagnostic for POTS. I’ve met completely healthy teens who have been told by their well-meaning medical provider that they have POTS just because they measured the heart rate in those positions and it met those criteria. Imagine going home and looking up POTS – or reading this blog - after your doctor just told you that you have it, right in the middle of track season, while you’re feeling just fine, and then thinking that you are destined for disaster. How confusing (and devastating) is that?

So a healthy person who does that with their heart rate does not have POTS. They need to drink more fluid, eat more salt and lay off the caffeine (first three treatments for POTS

BTW). They don’t have POTS any more than a healthy person with a fever has malaria (all patients with malaria will have fever; most people with fever do not have malaria).

But for folks who really do have POTS? It’s a long, hard road to recovery, further confused by mixed messaging, conflicting advice and lack of a quarterback to direct the care.

It’s important to rule out the physical, organic illnesses that can present with similar symptoms, which can be cardiac, neurologic, endocrine and – depending on the specifics of any one person’s complaints – gastrointestinal, allergic, rheumatologic or otherwise. If a patient is found to have one of those, then treatment should be aimed there.

But if not, then what? The mainstays of treatment are all non-medical, and may include some combination of ample intake of fluid and salt, regular exercise (easier said than done when you can’t get out of bed), stretching, regimented sleep habits, avoidance of caffeine and alcohol, donning compression garments and taking non-prescription supplements that might include iron, magnesium, vitamin D3 and others. These are easy to recommend even for patients who are along the spectrum but don’t qualify as POTS, since in general they’re actually just good health habits for most anybody.

Beyond that there are prescription medications that can be helpful, including fludrocortisone (which causes sodium retention), beta blockers (dampen the twitchiness of the heart rate response to posture), midodrine (boosts blood pressure) and ivabradine (which is a lot like beta blockers but with fewer side effects, but less Pediatric use, and it’s more expensive so hard to get insurance approval). Most of these are trial-and-error options which might take a month or so before showing effectiveness. I honestly believe that in general many POTS patients would benefit from being prescribed medications sooner, even before they meet diagnostic criteria. The reluctance goes back to the fact that most specialists bow out of the management after ruling out a primary problem in their specific organ system, and generalists are reluctant to prescribe them because they mostly belong to the specialists (specifically, us Cardiologists). It’s another case of the care for POTS patients falling through the cracks, or more accurately not definitively landing in anyone’s particular lane.

From my own perspective, I’ve found it satisfying since starting the practice a little over a year ago to be able to be able to carve out the time to care for POTS patients and their families in a way that I couldn’t within the confines of the hospital setting, where I might have patients scheduled at 20 minute intervals. These are routinely 2 ½ hour visits to start. It takes that long to hear the story, figure out which other organ systems need exploration (and who in those specialties can help), rule out the direct heart disease, and then map out a strategy. But the most important part is to elucidate what’s going on here, convince them

and their families that, yes, this is a thing, and to explain the mechanism of the disorder which then can demonstrate WHY the non-medical and maybe the medical measures may help. Yes, exercise is important for everybody but HERE’S why it’s particularly important for you, right now, for this problem.

Again, maybe the most important part of that visit is the recognition of the condition and the validation that it’s real.

Dr. Fischer, who I mentioned above, has officially retired, BTW, but he still takes my calls to chat about POTS. I think he’s one of those guys for whom “retirement” has a different meaning. Like, he’s retired, but I bet you can find him in the hallway at Mayo at least three days a week. I can relate to that! Anyway, he wrote a book a few years back called “Tired Teens,” aimed less at medical professionals than at POTS patients, their families, and the community around them (especially schools), because it truly takes a village to shepherd these folks to recovery. I keep a handful of copies in a drawer in my office, and I hand them out to POTS patients and families when I think it’ll help. And usually the greatest value isn’t really in any detail that’s included in the book, but rather in the fact that a guy would sit down and write a book about it and that somebody else would see a value in printing that book. Hear this: POTS is real! Somebody believes you! We wouldn’t waste paper on a fake illness!

I didn’t watch much of the Winter Olympics last month, but there was a particular highlight for me. The captain of the Swiss hockey team was none other than Roman Josi, who did indeed return to the Predators for training camp, just like the Athletic promised. He’s played in 53 out of 64 games for Nashville as of this writing, which would be pretty impressive at age 35 even without POTS.

I’m happy for him, but also happy that we now have a name and a face to attach to POTS. His is a story that we can share with our POTS patients has a happy ending. I’m grateful to Josi, the Predators and the NHL for disclosing his condition instead of just categorizing it as an “upper body injury,” (that’s what they do), which wouldn’t be helpful to the cause of POTS sufferers at all.

Kristaps Porzingis, former Knick and Celtic now a member of the Golden State Warriors, has also shared that he is suffering from POTS. He’s playing sporadically, getting in about 22 minutes some nights but missing more games than he’s playing. Before the revelation of his diagnosis, though, I recall that there was a lot of speculation among fans and sports media regarding his scattered availability, and on some level I’d bet that it was a relief to him when his condition received a label, and then quite valuable when the diagnosis became public to set the record straight. And in retrospect I recall a sentiment in the sports media a year or so ago that reminds me of the plight of every POTS patient. People wondered if he was faking it, if he really “wanted it” any more. I check the box scores every night (25 minutes, 30 points last night!), and root for him even more than I did when he was wearing the Orange and the Blue.

We’ve come a long way for POTS patients, but there’s still a long way to go. However, being able to point to a POTS patient who was able to represent their country athletically at the highest level within a year of their diagnosis is a great touch point for inspiring hope.

It may be a long tunnel, but there’s always a light at the end of it.  Thanks, Roman and Kristaps, for lighting the way.