man in bed with eyes opened suffering insomnia and sleep disorder

POTS: The Most Common Medical Condition You Never Heard Of

Last month the world recognized Rare Disease Day on February 28th.  There are medical conditions that are not rare at all, but still fall into the abysmal cracks in our healthcare system.  Some might call these orphan conditions.  I live with one of these conditions – postural orthostatic tachycardia syndrome (POTS).

POTS is fairly common, but is rarely discussed in medical school, rarely highlighted by the media, and rarely the focus of pharmaceutical industry interest.  POTS patients are rarefied in every way possible… except for the fact that there are over 1 million of us in the US alone.  POTS might be the most common medical condition that no one has ever heard of.

Mayo Clinic researchers estimate that 1 in 100 teens develops POTS.  Including adult onset patients like myself, there are an estimated 1 to 3 million Americans living with POTS.  85% of patients are female, most often between the ages of 12-50.

POTS is one of the most common forms of dysautonomia.  Dysautonomia (pronounced dis-oughta-no’-me-uh) is an umbrella term that includes many different disorders of the autonomic nervous system, which impact over 70 million people worldwide.  The autonomic nervous system originates deep in the brain and extends from the top of your scalp to the bottom of your feet.  It is responsible for regulating involuntary bodily functions including your heart rate, blood pressure, digestion, kidney and bladder function, temperature control, sweating, tear and saliva production, and even your immune system.  There are very few processes that go on in your body that don’t involve your autonomic nervous system in some capacity.

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POTS symptoms can include lightheadedness, a fast heart rate, fainting, fatigue, chest pains, shortness of breath, GI motility problems, nausea, migraines and more.  Most of these symptoms are due to blood pooling in the lower limbs of POTS patients when they stand up, which doesn’t leave enough blood flowing to the heart, lungs and brain.  The symptoms tend to worsen when the patient stands up, and are improved when the patient lies down.

Experts compare the disability seen in POTS to the disability seen in congestive heart failure or chronic obstructive pulmonary disease. Some patients are able to continue with normal daily activities, but 25% of patients are so disabled by POTS that they cannot work or attend school.  Some patients require the use of a wheelchair or become bedridden.

Many doctors are not familiar with POTS, or perhaps they have heard of it, but are not quite sure how to diagnose it.  As a result, the average POTS patient takes six years to get diagnosed.  While several studies from major academic research centers show that POTS patients have normal psychological profiles, about 85% of POTS patients are given psychiatric labels prior to being diagnosed with POTS.  The multitude of symptoms caused by their autonomic dysfunction are often dismissed as anxiety or they are told that their symptoms are “all in their head.”

How is POTS diagnosed?

POTS is most often diagnosed using a tilt table test, but when this is not available, some doctors may use do an active stand test, also called a “poor man’s tilt.”  If POTS is suspected, the patient should be referred to a doctor with experience in diagnosing and treating POTS.

Without a doubt, POTS is not “all in your head.”  While the exact cause of POTS is unknown, researchers have made some progress in understanding the condition.  20% of POTS patients have evidence of cardiac autonomic neuropathy and 50% of POTS patients have sudomotor neuropathy – both parts of the autonomic nervous system.  Researchers at Harvard are currently studying abnormalities in the vasomotor nerves in POTS patients – also part of the autonomic nervous system.  The vast majority of POTS patients have low blood volume, with deficits in both plasma and red blood cells.  Male and female POTS patients are more likely to have iron storage deficiencies than healthy individuals.  A significant percentage of POTS patients also have Ehlers-Danlos Syndrome, an inherited connective tissue disorder.  Perhaps most interestingly, there have been several studies in the past few years that have identified evidence of autoimmunity in POTS patients, including antibodies that target the autonomic nervous system.  Research in this field is ongoing.

While there is no cure at this time, there are treatments that may help manage symptoms.  Non-pharmacological treatments include increased salt and fluid intake, medical compression stockings, recumbent exercises, eating smaller meals throughout the day, and avoiding prolonged standing and hot environments.  Pharmacological treatments commonly used in POTS are aimed at expanding blood volume (fludrocortisone, desmopressin), improving vasoconstriction (midodrine, phenylephrine, octreotide) and reducing tachycardia (beta blockers, ivabradine). Other medications may be used, depending on each patients’ unique presentation.

Another similarity between POTS and rare diseases is a lack of research funding.  Everything we know about POTS has been discovered with less than $1 million a year in National Institutes of Health (NIH) funding.  While $1 million may seem like a lot of money, for comparison, multiple sclerosis impacts 400,000 Americans and receives over $100 million in NIH funding per year.  Parkinson’s disease impacts an estimated 1 million Americans and receives over $135 million in NIH funding per year.  Imagine what scientists could discover about POTS and what new treatments might be developed if the NIH invested 100 times more money in POTS than they currently do?  A girl can dream…

 

Guest author Lauren Stiles is a New York attorney and co-founder of Dysautonomia International, the leading non-profit advocacy organization for individuals living with POTS and other disorders of the autonomic nervous system.  Dysautonomia International funds research, educates medical professionals and empowers patients to be their own best advocates.

If you’re worried about any undiagnosed symptoms you have been having, CrowdMed, with its teams of Medical Detectives can help you find the answers you’re looking for.  Click here to find out more about us.  

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  • Kim Pearch

    Great article with information that can be used to educate others. But what do we have to do to get Dysautonomia off of the “Rare Disorder” list. I’m so frustrated when I continue to hear people say, “Rare.” How can it be rare when there is an estimated 70 MILLION people worldwide living with some form of Dysautonomia? I hope soon! Thank you again for a top notch article!

    • Lauren Stiles

      Dysautonomia International is working on it. “Hypovolemic POTS” was once given a grant from the Office of Rare Diseases back when the POTS researchers thought that might be a rare subset of POTS. Then the research showed that this was not a rare subset, that most POTS patients have some extent of hypovolemia. POTS landed on the rare lists because of this grant and it seems like every rare disease organization copies lists form other organizations. It will take some time to fix this, but we have some very motivated medical students and nurses working on it. In the meanwhile, we should rely on peer-reviewed medical journal articles that provide the best estimates, which range from 500,000 (older estimates) to 1-3M (more recent estimates).

      • Kim Pearch

        Yes, I absolutely agree and value that we should rely on peer-reviewed medical journal articles for estimates and anything other medically researched information. I would need to check my sources, as far as, the 70 million “worldwide” affected by dysautonomia that I referred to in my previous comment. It was not my intent to use an estimate that was not referenced. Working together to raise awareness and funding for Dysautonomia is key to educating the medical community and the community at large. Alter all, that is our common goal so that physicians can identity the symptoms, properly diagnose and subsequently treat patients living with the various forms of dysautonomia. Again, thank you for writing this article and reinforcing the need to rely on researched based articles.

  • hester

    Anybody with POTS who cannot swallow and produces excessive saliva that also tastes bitter?

    • Lauren Stiles

      Hester, difficulty swallowing is not talked about much in the medical literature on POTS, but esophageal motility is something that can be a problem for some POTS and other dysautonomia patients, and it seems that this can cause swallowing problems for some of us (me included). The movement of food and liquids is pushed down through your esophagus by peristaltic contractions controlled by your autonomic nervous system. If this is not working properly (too fast, too slow or just poorly timed contractions) this could cause swallowing problems and/or reflux. A bitter taste in your mouth is often reflux or post-nasal drip. Try to find a good autonomic neurologist, a GI motility specialist, or an ENT that focuses on swallowing disorders.

      • hester

        Thank you Lauren. It makes a lot of sense.

        • Susan Kennedy

          What an incredible article and also extremely well written, I have Çhroņiç Fantique Disease. All the Doctors have ķnown since 2004 that my heart rate was 140bpm, even after 2 heart attachs, nothing was done. It was through my network that I heard about POTS. Once I read the medical definition, it was bang on. So it is true:PATIENT HEAL THY SELF!

  • smileybean

    I have Chiari Malformation and ended up w/ POTS due to CM. It’s so hard to live with. It’s amazing how it falls under the “rare” elements of diseases when there are so many who have it. This is a great article which I have shared hoping to educate others. Thanks for this.

    • Tricia

      Do you have ehlers danlos syndrome?
      They are both common togather with it.

      • smileybean

        i’ve never been tested for EDS.

  • CatoYounger

    Does it always have to be government research? A private charity dedicated to this condition should be able to raise more than a million dollars. I have this, but I’m not going to wait around for the government to learn more or get better..

    • Lauren Stiles

      100% agree! This is exactly why we started Dysautonomia International in 2012, a 501(c)(3) non-profit that advocates on behalf of people living with all forms of dysautonomia. We fund medical research, educate physicians, raise awareness with the public and empower patients to be their own best advocates. We have issued $266,000 in POTS research grants since our launch, including the largest private POTS grant in history. Check out http://www.dysautonomiainternational.org/POTS-Fund to learn about some of the research we are funding.

      We also hold Dysautonomia Lobby Day each year to let Congress and NIH know that we need more federal research money for all forms of dysautonomia.

      You can reach us at info@dysautonomiainternational.org if you want to get involved with fundraising, advocacy or awareness projects. We would love to hear from you and anyone else who wants to learn more or get involved.

  • Brian White

    Dysautonomia is also a very big part of Multiple Sclerosis. Check out “Jade’s autonomic system reboot” A playlist I have on youtube. It shows that angioplasty of renal, and neck veins helps many people recover from dysautonomia. Also check out lottie vigue. There is a video somewhere. She had pots probably from Lyme disease, and she had a great response to angioplasty too.

  • http://www.tanyamarlow.com/ Tanya Marlow

    I have POTS and Myalgic Encephalomyelitis – theres a lot of overlap between the two conditions, and a big subgroup of those who have ME also have POTS. I’m glad that there’s more awareness and treatment for POTS; hoping for the same for ME.

  • http://www.itsnotjustlyme.com Yessi Young

    I never knew how many people are affected by POTS. You can make your own tilt table test by lying down and sitting up. This method is not as severe as the tilt, but if your blood pressure is abnormal doing this, then you definitely know there is an autonomic problem. My POTS at its worst was scary. I could jog for 3 minutes and my heart rate would not change, I would not sweat, but Id feel like fainting and my vision would blank out. Treating other factors like my gut, immunity, hematological issues and antigens has reduced my POTS to the point I no longer have symptoms.

    • Atara

      No, you don’t have to have a rise in blood pressure to have an autonomic problem. NCS would show a drop in heart rate and blood pressure. That too is dysautonomia. Sorry you were only informed about POTS.

      • MamaBeans

        She didn’t say a rise in BP, she only said if your BP is abnormal there’s likely an Autonomic issue.

  • Atara

    I am finding conflicting scientific views. Just this week my cardiologist just today affirmed that NCS and POTS share overlapping clinical features and that any article attributing clinical features of dysautonomia just to POTS is incorrect in that regard. If you have NCS, :you also have cognitive fog,temp issues, fatigue, weakness, … but are brady as opposed to tachy. My own experience and that of other NCS patients in forums is that both POTS and NCS patients share similar clinical features, yet this is contrary to some of the articles posted. My cardiologist places little value on adding a POTS diagnosis to NCS as the treatments have so much overlap and treated the same with few exceptions (Ritalin cannot be given for POTS). He also explained that NCS is not a broad term and excludes single episode fainting occurrences. Again, this is quite different than the ideas shared to me in posts to explain why most articles, headlines, and p.r. displays highlight POTS rather NCS and other dysautonomia forms, why some even purport to know that one is more common than the other. We really should discuss dysautonomia, and lose the subset focus. I Whatever the truth is, advocacy groups should be very careful in the accuracy of the education they provide.

    • MamaBeans

      Ritalin and Adderall are both used in POTS so I’m not sure where you got that info. Also, they’re are lots of subsets of dysautonomia that are very different from one another and we don’t all deserve to be painted with the same brush. AIDS, Lupus, Sjogren’s and Rheumatoid arthritis are all subsets of autoimmune diseases, should we just lose the focus on subsets there, too?

      • Atara

        Set aside the Ritalin part of the above paragraph because that may have been misinformation given to me. I do object to a subset focus when it comes to raising awareness about dysautonomia when it comes to NCS and POTS, where patients so often overlap and it seems both groups experience the fatigue, fogginess, pre-syncope, temperature deregulation, spatial disorientation, visual disturbances. I question those that assert that the presence of those clinical features means that the patient also has POTS. My experience personally and listening to others leads me to believe that both NCS and POTS patients share alike clinical features but differ by whether the patient become brady or tachy. The subsets are not wildly different as are your autoimmune examples. My goal is to get better clarification from researchers whether NCS patients can have clinical features similar to POTS and do not just suffer from vasovagal fainting. That is extremely important for their treatment and the understanding that NCS patients receive when they tell their other doctors and apply for disability, etc. Currently, we don’t have a consensus and the assertions supporting that statement that the clinical features indicate POTS could be based on assumption. It also could be that the two are so close that they should be studied together and patients should expect some fluidity over time. If we don’t take a macro view, we won’t know how many of us overlap and, of those of who do, whether brady or tachy dominates, what triggers which, etc.

        • MamaBeans

          I don’t know if you’re making this needlessly complicated on purpose or ?
          The main problematic symptom in POTS is the Tachycardia. The main problematic symptom is NCS is the syncope. Everyone who has POTS ha tachycardia, but doesn’t faint, everyone who has NCS faints but doesn’t get tachy, it’s a different illness. They can overlap but they are pretty clearly distinct. I have POTS because I have Ehlers-Danlos. I don’t want POTS research folded into NCS research and I don’t see what good that would do.
          It’s also looking more and more likely that ME/CFS and Fibromyalgia will be found to be dysautonomias, as well. Not every ANS dysfunction is equal and I see no good coming from treating them as if they are.

          • Atara

            Let me try to present the question needing to be
            answered in a clearer way so that it isn’t so complicated. We are all clear that POTS refers to tachycardic episodes and NCS refers to bradycardic. The research on POTS has identified clinical features that can be associated with POTS that are not attributed to NCS. This creates issue for NCS patients as we need clarity as to what our diagnosis entails. What does NCS mean? Does it just refer to bradycardic episodes or does it too have the same clinical features? Many who are diagnosed with NCS but not POTS
            (i.e. who do not report tachycardia episodes) report the same not-to-be-dismissed symptoms (such as headaches, spatial disorientation, visual disturbances,
            temperature deregulation, fatigue, etc.) as do POTS patients. Some doctors who treat NCS patients are telling them that these symptoms are general dysautonomia
            symptoms whereas others believe that these symptoms mean that the patient has POTS. So, we currently
            have two ideas put out there about NCS patients. Some assert that NCS refers just to
            fainting and that the presence of these other symptoms must indicative POTS
            (even in the absence of tachycardic episodes), whereas other doctors believe that a NCS diagnosis means fainting plus the other symptoms (which vary by person of course). NCS patients just need to have
            some clarity to it so that their doctors and the general public knows what they have, how to understand them. Either the NCS patients who have these symptoms have POTS or both NCS & POTS share significant commonalities. When I don’t know what NCS entails on its own, I can’t understand what it means to say it overlaps with POTS. My own conclusion would be that the patients is sometimes brady and other times becomes tachy. But someone else might conclude that the patient never becomes tachy but warrants the POTS overlap diagnosis solely on the basis of the shared symptoms. So NCS had to mean something that we can all understand. What does NCS entail is the question that I have. It doesn’t matter which is the answer but it doesn’t matter that the term NCS develops some uniform understanding.

            I hope the question is now clear. I suppose we don’t need to ask or agree on how to answer the question. I thought that we needed to look and compare the two groups, which would require studies of us both. Perhaps not. Let’s just start with getting some understanding that there is much confusion and frustration being experienced by NCS patients as to what NCS means.

  • Dian Larkin

    PLEASE help us generate donations – my son Ari’s ‘iron will’ bike tour to raise awareness & research $ for #POTS – http://www.thepedalforpots.com – in association with Dsyautonomia International. POTS has destroyed my son’s life. He needs to do something to help, and maybe to heal a little bit. This bike tour will be more than grueling for him, but he has an iron will and he will accomplish it with help from the POTS community. The bike tour gives purpose and meaning to a life that otherwise has none. He had to quit practicing law because of #POTS. No job. No life. The bike tour is his only goal now – and every $$ raised for POTS research is a $ of hope for POTS sufferers. Thank you, Dian Larkin/ dianyudelove@aol.com ari.diaconis@gmail.com http://www.thepedalforpots.com dysautonomiainternational.org