I recently filled out an application to attend/speak at the 10th Annual Patient Summit USA conference. They were looking for speakers on “Rare Diseases” – I blog about Autonomic Nervous System Disorders (sometimes called “dysautonomia,” even though that nomenclature is not widely used here in the US), and I do think it is a rare group of diseases, as does the NIH and The Rare Disease Clinical Research Network. (I also blog about a few other conditions, as well as doctor-patient communication, but that doesn’t matter right now.)

My current passion is to bring attention to the fact that there are MANY forms of ANS dysfunction, not just the one that gets all the attention – POTS – that literally all of the patient support sites seem to rally around. Perhaps it is because POTS tends to effect attractive young women after a growth spurt. Many of the current ANS disorder “activists” that rally around POTS publicly admit that they do NOT know much about ANS disorders as a whole, and therefore, only talk to the public about POTS. By doing so, they are completely ignoring the patients of the other 30 or so types of ANS disorders! Also, what they DON’T share, is that POTs symptoms often subside as their bodies adapt to the rapid growth, and go on to live fairly normal lives. In fact, many POTS patients have sports teams!!  (While those of us with other types of autonomic dysfunction can barely walk around our homes…) To present POTS as THE FACE of “dysautonomias” (or more appropriately, “Autonomic Nervous System Disorders”) is to present information that is deceptive to the audiences that need to be reached to raise awareness of the body of disorders. It is certainly not the most devastating or life-altering ANS disorder. People NEED to know about ALL of these conditions. What needs to be be made clear is that most of us with ANS disorders DO NOT get better (as do many of the young POTS crowd). We get WORSE. We can DIE from complications related to the destruction of our autonomic nervous system – the system responsible for making us breathe, making our heart beat, making our blood circulate, and making our digestive process work as it should.When these systems stop working properly – the body can’t sustain itself.

This body of disorders is so hard to diagnose (according to the specialists in the field), and is so unfamiliar to the medical community, that it is near impossible to find a doctor that knows how to diagnose or manage a patient with an autonomic nervous system disorder that is not the glamorized POTS or secondary to Parkinson’s disease or diabetes. Even the so-called Autonomic Disorders Treatment Centers in the US (there are 5 that are linked) do not test and treat everyone with some form of the disorder. I am unlucky enough to have a form of ANS dysfunction that the Boston center doesn’t treat/handle, and I am forced to expand my search outside of the Boston area to find someone to treat me. (Contrary to popular belief, Boston is NOT the cutting edge “medical mecca” it is rumored to be.)

AutonomicWHAT?

Don’t you love it when the medical personnel you rely upon to help you has no idea what you are talking about? Trust just FLIES out the window…..

I have a medical/science education and background, and I am an very experienced in researching medical issues using sources that are valid and peer-reviewed, not the consumer-focused platforms most of the more outspoken activists for “dysautonomia’ (actually, just POTS) seem to reply upon. My medical background allows me to understand the systems affected by this body of disorders and explain how these disorders affect our bodily functions. I have no underlying motive that would make me personalize the information as others have done (seemingly to glorify one’s daughter or young relative that has a POTS diagnosis and generate sympathy and funds for their sports endeavors…)

A significant amount of “confirmation bias” is evident in the information currently being presented to the public, which is extremely detrimental to the goal of educating not only laypersons, but medical professionals that have never even heard of these conditions. Every nurse, medical assistant, ER physician, gastroenterologist, cardiology technologist and nuclear medicine professional that I have met in the past six months had never even heard of Autonomic Nervous System Disorders!! This meant that I spent my last three hospital stays educating every single professional I had contact with – ludicrous! If I had not done so, I would have been given medications contraindicated for a person with my condition, and treated with measures inappropriate for my condition.

What scares me is this: what happens when someone that is NOT a skilled researcher with a medical background develops an ANS disorder? Such a patient would not have the ability to advocate for themselves, nor would they know that they HAVE TO do just that since the medical profession is not at all familiar with these diagnoses.

It is frightening what could have happened…and what COULD happen if awareness of ANS disorders remains ethereal, as it is now.

Well, I didn’t intend to launch into a diatribe….I apologize for rambling on. I guess it just show how passionate I am about getting the word out about these disorders to medical professionals. I don’t ever want anyone to suffer through what I have had to endure – and my ANS disorder isn’t anywhere as severe as many other types – I just don’t want to see people with these issues get misdiagnosed and receive improper treatment – no patient should have to go to the ER armed with printouts explaining their condition to staff that seems to think you are making the whole thing up! Medical professionals should be aware that these conditions exist – and they should learn how to do a bit of proactive medical research themselves – it takes less than 30 seconds to pull up a journal article abstract explaining ANS dysfunctions on any of the many computers and tablets present throughout our ERs. No one should be questioned in the humiliating and disbelieving manner in which I was when I was trying to explain to the ER staff WHY I was there and what condition I have. It shouldn’t be happening in the 21st century. Not here in the U.S.

Do the thing you fear and the death of fear is certain. — Ralph Waldo Emerson

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Comments
  1. kdsc says:

    There are patients with POTS that did not get the typical teenager version that they outgrow.. me being one of them. In fact, my POTS has gotten significantly worse as I have gotten older – though I suspect that some of it may be because other underlying things that aren’t diagnosed have gotten worse.
    But as I was reading this post, something occurred to me: how many patients like me that have been diagnosed with POTS, actually have more or perhaps even a different autonomic nervous system disorder and don’t know it? Most of my doctors wanted me to go through more testing, especially autonomic-wise and sent me to a hospital in Philadelphia specifically for this reason. When I got to that appointment, they actually refused to do those kinds of tests despite my symptoms. They told me there was no need. I had the tilt table test and that was all I needed to know. This despite other things going on with me outside the realm of POTS *and* things that could even be causing the POTS symptoms.

    Maybe more doctors need to be trained and educated in not only ALL autonomic dysfunction disorders (yes, including POTS… because believe it or not, I have yet to find a doctor around here who actually know all the facts about it) but also in the importance of knowing each individual patient and how each of their bodies work (if I’ve learned one thing: as patients, we are NOT all created equal). I have a really complex case. I focus on POTS right now because that’s the only definitive answer I have and even then, how definitive is it? They think I was born with POTS.. I’ve been struggling since I was a child (and I do mean as far back as I can remember) with health issues. I thought for years that it was in my head and I needed to just get over myself (long story).

    It just frustrates me to no end that there are doctors out there who aren’t willing to look outside the box. Not willing to see the zebra(s). They aren’t willing to figure out why a patient is going through what they are going through because “it is what it is” and they can mask some of the symptoms with some medication (never mind for people like us, the medications usually don’t work in the way they are supposed to).

    Also, how does one not believe in dysautonomias? How does he explain the very visual symptoms side of things? Being your own advocate is hard, especially when you have something like this because you are exhausted and have everything else going on. I wish we could all have an awesome advocate assigned to us to step in when we can’t.

    • Lori says:

      You are SOOOOO right! I have been try into put together a list of research articles that will educate people (including medical folks) about ANS disorders. It is alarmingly hard to find a distinct diagnostic criteria specifically for the various types of autonomic issue. Why?
      First – there are 2 separate and distinct “camps:” the neurologists, and the cardiologists. I experienced the clash of “beliefs” first-hand. Since one of my docs was one of THE big names in autonomic dysfunction, it was a bit hard to comb through the biases of each…I did “side” with my cardiologist and PCP, who both thought that treating the BP spikes I have was far more important than forcing me to take medicine that made me ill, just to keep my BP high (actually, too high).
      Second – a lot of docs throw the POTS label at anyone with orthostatic hypotension, when in fact, that is NOT a true POTS symptom!! The literature originating from the Mayo Clinic is abundantly clear about this. It specifically states that “orthostatic intolerance” is the hallmark of POTS – which is, as the POTS moniker suggests, is TACHYCARDIA that occurs during a change in posture, and NOT a drop in blood pressure. One of the Mayo papers goes on to list “varieties” of POTS, which is where diagnostics of this convoluted mess of disorders gets very murky. It does, however, make this statement:
      “The emphasis on postural tachycardia does, however, have a disadvantage in that it ignores nonorthostatic symptoms such as paroxysmal episodes of autonomic dysfunction, including sinus tachycardia, BP fluctuations, vasomotor (especially acral) symptoms, and fatigue.” (Low, p., et al, “POTS (Postural Orthostatic Tachycardia Syndrome),” Journal of Cardiovascular Electrophysiology, Volume 20, Issue 3, pages 352–358, March 2009.)

      I DO think that a majority of POTS patients are, in fact, misdiagnosed. I think that since POTS is the most publicized version of autonomic disorders (my point exactly), doctors that are not specialists will label their patients with “POTS” because it is much easier than to continue to try and diagnose whatever it is their patient has. Most doctors do not conduct diagnostics like Dr. House – I’m sure we all wish they would!!

      I believe most doctors are inclined to jump to POTS, because of my own experiences with many physicians at several facilities, including one of THE leading published experts on the body of autonomic disorders (Roy Freeman, MD, Beth Israel Deaconess Medical Center, Boston MA). Every single doctor I have seen (that were familiar with ANS disorders, as not all were) clearly stated that I do NOT have POTS, but that I have some other incarnation of an autonomic nervous system disorder that has not been clearly identified, since my symptoms are constantly changing, evolving, and compounding. However, POTS was their first stop on their diagnostic trip.

      From my first hospitalization for these mysterious symptoms that just suddenly struck me one day, it was made ABUNDANTLY clear to me because my BP plummets, because I have the reduced gastric motility, and because I have a variety of issues that point to a possible autoimmune component, I DO NOT HAVE POTS.

      By having people consistently harp on the supposed fact that they have POTS, when in fact they have another form of an autonomic disorder, is detrimental not only to themselves, but everyone else that is suffering from these disorders. The NIH would not be conducting research on these other disorders if in fact POTS was the catch-all diagnosis. I hope people open their eyes, read the professional literature about these disorders, and demand that their doctors pay attention to the research that exists so they they can get the proper treatment they deserve.

  2. Hi! I do wish you all the best in your application!!!! Thank you for your passion and willingness to speak out! I have adult diagnosed POTS along with several auto immune disorders, as well as, still many things that have not been diagnosed or explained but do fall under the ANS. Trying to find out POTS as a diagnosis was highly difficult so I can not imagine trying to get a diagnosis for any one of the less known Dysautonomias. I can only imagine the misdiagnosis out there. And can hardly bare to think about the number of patients who have been mistreated by medical personnel as a result of symptoms that can not be readily medically explained by routine procedures and test. I still do not have many answers despite having a medical team comprising of a cardiologist, neurologist, rheumatologist, urologist, ENT, GI, PCP, dermatologist, ophthalmologist and a dietician. I feel much like every physician I go to wants to treat the first symptom I discuss and then treat the next one and so on. Then they pass me off to the next doctor who specializes in not disorders of the ANS. Instead of listening to all my symptoms and then developing a plan of attack. Hearing “that’s not my area” are my least favorite words. I know we have specialist and panels of doctors but there is not one doctor that I have seen that treats the entire ANS. My PCP is very good at listening to everything but treating my ANS is beyond her medical knowledge. This is my second PCP because the first told me that he did not believe in dysautonomias and well I broke up with him quickly. I do not understand why every patient with an ANS disorder has to go through that. Most of the information I have found has been found based on my own research or the research of other patients. I am however personally grateful that there is some information out there about POTS because without it…I would basically have little explanations and resources. I hope that the research, resources become more prevalent for all ANS disorders.

    I can’t help but wonder if there isn’t something more going on with my body because I do have so many symptoms that have yet to be explained beyond “your body does not respond to anything normally that it should”. I was referred to Vanderbilt and am going in April. Hopefully I will get more answers then.

    Again thank you for speaking out about Dysautonomias!!!!!

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