Saturday, April 21, 2012

What Is Causing Your POTS? And Why It IS A BIG DEAL: A Printable, Sourced Guide.

Many of us hear by our doctors that POTS is "no big deal." 

Or they just plain have no clue what it is.  

Or, "you just have POTS."

Why do they think this?  Why don't they know what it is?  If an estimated million people have it, and even more have dysautonomia in some form, why don't they know?

As  patients (and hopefully the caregivers that see how sick we can be on a day to day basis), we know how big of a deal it is.  We know what a struggle POTS is, and how unpredictable and disabling it can be, even if it is generally mild and flares occasionally.   So why is this the view by so many in the medical profession, and why is it so unheard of?   I have been contemplating and compiling this list for a while now, and in light of the people we have lost recently in the POTS community, I thought this may be useful for those of us trying to find the cause of our POTS.  Also to explore why it is a big deal, even if POTS per say will not kill you, and why, in my opinion, it is absolutely necessary to find out what is causing your POTS.  Not only to rule out the really dangerous stuff, but to avoid the medication merry-go-round, as I like to call it.     


Here are some of the issues:

WRONG!
1. POTS is very misunderstood, by both patients and medical professionals.  It is also relatively unknown, but it is not rare.  There is a major identity crisis going on with POTS, and it can be confusing and ever changing, with developing definitions that vary from doctor to doctor.  The advances made in the autonomic studies field over the last decade are pretty extensive and amazing, and frankly pretty hard to keep up with.  From a review of the medical journal literature, it seems there is a lot of misunderstanding and even disagreement on what exactly POTS is, what causes it, and the many theories and proposed subtypes of POTS.

How that information is passed on to the medical profession as a whole (or is not passed on, as many have not even heard of it) I am not yet aware of.  Is it being taught in medical school and courses that doctors take to renew their licenses?  We sure don't see any brochures in doctors offices, like you do with many other conditions.  I'm assuming only doctors in the field that these journal articles are being published in are reading them regularly, so that narrows the audience quite a bit. Autonomic journals, neurology journals, and cardiology journals.  And an occasional pediatric journal. That's not a lot.  So when a patient goes to the ER or to their primary care MD or Internist, they are most likely not reading these regularly, and may not have heard of POTS, or are basing their knowledge on a limited review of literature.


2. POTS is a spectrum.  I hear many people online express their disbelief that there are people actively living with POTS.   The reality is that there are many, many people with POTS who lead fairly normal lives, or have waxing and waning symptoms that come and go.  This does not mean it is easy to live with, or that it can not be disabling, or is "no big deal".  There are an estimated 25% of people with POTS that live with the same quality of life as those with COPD and congestive heart failure.  There have even been recent deaths of very young people with POTS in the online community.  All of them had conditions beyond POTS, and died of those complications, not from POTS per say, as POTS is a collection of symptoms - not a disease.  But POTS is a warning sign that something is wrong - there is always something causing POTS, and I firmly believe that YOU HAVE TO FIND OUT YOUR CAUSE.

Even if you have mild POTS, it may get worse in the future (like me!), and if you never found the cause, treatment will be more difficult (like me!), or you may have had an underlying condition that you did not know of (like me!).  If a doctor or family member, or anyone for that matter, tries to blow off your POTS as no big deal, I'm including information in this post on why it is, and feel free to print it, cut and paste, or do whatever with it to get started figuring out what else it going on with your health that is causing your POTS - it is copyright free.   I wrote this from scratch, so "Merry Find Your POTS Cause Day." 

That's me again, I swear!
3.  You are responsible (or your parent is if you are a dependent) for your own health and advocating for your treatment.  I really do know how hard this is, after a decade of chasing down doctors.  Sometimes we may have to steer our doctors in the right direction, and have do our own research when finding what is causing our POTS.  You must take responsibility for your own treatment and care, or find an advocate who will for you if you are too sick, or just plain worn out.  It is OK to be worn out, this is an exhausting disease, and many of us have to fight through basic daily activities that others take for granted.  It can be overwhelming to have to keep fighting and fighting, especially when you know something is wrong and no one is listening.  But you have to keep fighting.  If a doctor refuses to help you dig deeper to better tailor your treatment - FIRE THEM.  They are not God, and they do not all have your best interest at heart.  Some do.  But do not let yourself be brushed off when you know something is wrong. You are a paying customer, and have to be your own advocate (parents of POTSies included!).  Have I said be your own advocate enough?  

For example: I have had OI/POTS since 2002.  It was mild at first after the initial scariness, loosing career number 1, and a bad period that triggered getting an "Orthostatic Intolerance" diagnosis from a tilt table test.  There was about a 2 year period where I was on a Beta Blocker, and felt fairly normal and great.  Then things went south again, and I managed to loose career number 2.  Then later, during my first pregnancy and while nursing, my POTS was GONE.  It was awesome.  But it slowly came back after I stopped nursing.  I had given up looking for the cause, and on local doctors in general.  It had become exhausting, and with ever changing symptoms, very frustrating.  I just dealt with it and pushed through.  Then I got pregnant again, and my POTS (and whatever else I have) went completely bonkers, and I received terrible treatment and almost died and lost my baby.  And lost career number 3.  If I had known what my underlying issues were, I firmly believe my life would be different than it is now (I am one of the 25%, and am home and often bed bound). Luckily my daughter survived unscathed, but I am now disabled.

4. You can get better, and most do.  When I was feeling well, I was not online in support groups - I was out living.  I found the support groups depressing, or felt guilty that others were so sick an I was able to drive my boat around and enjoy taking my son to playgroups and the park.  There ARE many POTSies with mild cases that they grow out of, or - like me - the symptoms come and go.  With an estimated number of POTSies of 500,000 - 1 million, the support groups would be A LOT bigger if everyone was as sick as those of us online actively seeking support.   Most people with POTS do get better (whatever that means), and see their symptoms abate to some degree.   I'm hoping the researchers start publishing studies with more statistics regarding the POTS population dynamics, recovery (or not) rates, and what conditions co-exist and cause POTS.  The statistics are tough, because how many are misdiagnosed or undiagnosed?   Point being, POTS is heterogeneous (mixed), we are all affected to a different degree, and many other issues complicating their health as well.  But many people (an estimated 80%) see symptoms abate.

5. Lists always seem better in odd numbers, so this is purely a place filler as number 5.  Sidenote to take up place filler: It was suggested to me that the remainder of this post be called "WTF is causing your POTS" to be more attention grabbing, but I DO try and keep the "adult" content to a minimum on here and keep it suitable for all ages. It is catchy though.  So....WTF is causing your POTS?


So stick with me as I attempt to break down the situation, and remember that I am not a medical professional, I am a former scientist doing a review of the published medical journal literature, so please check with your doctor regarding anything and everything health related.

I am writing the remainder of this post in a way that you can cut and paste, or print out and take to a doctor if needed - hence the citations and lack of (or not too much of) my normal conjecture, and no silly pictures.


YOU DO NOT "JUST HAVE POTS," and why:
  •  POTS; or Postural Orthostatic Tachycardia Syndrome is a syndrome, meaning it is a set of symptoms caused by something else, defined technically from the medical dictionary as: "syndrome /syn·drome/ (sin´drōm) a set of symptoms occurring together; the sum of signs of any morbid state; a symptom complex."  So, it is not a disease in itself.  Or it would be named POTD.  It is a sign that something else is wrong, and needs to be investigated.  

  • POTS is generally defined as an excessive increase in heart rate (≥ 30 beats/min) with upright posture, associated with orthostatic, including palpitation, chest pain syndrome, dyspnea on standing, mental clouding, and difficulties with concentration in the absence of orthostatic hypotension. It can produce substantial disability among otherwise healthy people. (1,2.3,4).   

  •  Here is where we run into problems.  Most of the recent literature defines POTS as the increase in heart rate WITH OUT a drop in blood pressure (2,3,4,5,6).  I had a world renowned doctor tell me this the other day- you do not have POTS because you have Othrostatic Hypotension.  What?  The orthostatic hypotension is separate, and I believe the recent push to remove it from the definition of POTS is to recognize that some (maybe many) people with POTS have an INCREASE in blood pressure, or no change at all, and doctors were misdiagnosing people as not having POTS because their blood pressure did not drop. Here are a few of the definitions of POTS out of recent publications:   
    • In an article about POTS by Dr. Grubb from 2008 (8), he states, "POTS is currently defined as the presence of symptoms of orthostatic intolerance associated with a heart rate increase of 30 bpm (or rate that exceeds 120 bpm) that occurs within the first 10 minutes of standing or upright tilt, not associated with other chronic debilitating conditions such as prolonged bed rest or the use of medications known to diminish vascular or autonomic tone.  Most authors feel that a patient should have been symptomatic for >3 months.....It should be noted that many patients with orthostatic intolerance due to POTS will not demonstrate orthostatic hypotension (defined as fall of >20/10 mm Hg on standing). Instead, they may display no change, a small decline, or even a modest increase in blood pressure."
    • The drop in blood pressure upon standing, or othrostatic hypotension, seems to be considered a separate diagnosis, as is apparent here from 2006, Raj, et al., (2): "POTS is defined as the presence of symptoms of orthostatic intolerance for at least 6 months accompanied by a heart rate increase of at least 30 beats/min within 5-30 minutes of assuming an upright posture. This should occur in the absence of orthostatic hypotension (a fall in blood pressure >20/10 mmHg). The syndrome must occur in the absence of prolonged bed rest, medications that impair autonomic regulation (such as vasodilators, diuretics, antidepressants or anxiolytic agents), or any other chronic debilitating disorders that might cause tachycardia (such as dehydration, anemia or hyperthyroidism). It is important to recognize that this syndrome is typically disabling. Hence, the mere observation of orthostatic tachycardia is not, by itself, sufficient to make the diagnosis of POTS."
    •  Low et al. (9), in a review based on work of the Mayo Clinic published in 2009, defines POTS as, "the development of orthostatic symptoms associated with a heart rate (HR) increment ≥30, usually to ≥120 bpm without orthostatic hypotension. Symptoms of orthostatic intolerance are those due to brain hypoperfusion and those due to sympathetic overaction."

  • Does this mean that if you have a drop in blood pressure upon standing, you don't have POTS anymore?  Just Neurally Mediated Hypotension (NMH), or Neurocardiogenic Syncope if you actually pass out (NCS)?  I'm hoping it just means they are two separate diagnosis, as many of us are told we have both POTS and NMH/NCS.  To make it even more confusing, the Autonomic Disorders Consortium - who are a part of the National Institutes of Health - now call drop in blood pressure that causes fainting Neurally Mediated Syncope (NMS), and on their website they call POTS "Hypovolemic Postural Tachycardia Syndrome."  

  • POTS is also often classified as primary or secondary.  An example of an 2003 classification of postural tachycardia syndrome by Dr. Grubb (18):

    Primary forms: Partial dysautonomic, Immune mediated pathogenesis, Adolescence, Hyperadrenergic state
    Secondary forms: Diabetes mellitus, Amyloidosis, Heavy metal poisoning, Sjogren syndrome, Hypermobility syndrome, Paraneoplastic syndrome
     
  • Dr. Stewart, a highly regarded pediatric POTS specialist and researcher, has come up with a "flow based" classification system for POTS, which I tend to like. "The author's laboratory has described 3 groups of patients with POTS distinguished by differences in peripheral blood flow and peripheral arterial resistance. These groups are as follows:
    • A low blood flow, high-arterial resistance, high-Pv group, which is denoted as "low-flow" POTS and is characterized by pallor and generally decreased blood flow, most notably in the dependent parts of the body. This low-flow condition is related to defects in local blood flow regulation and mild absolute hypovolemia. The authors have recently shown that this defect in local blood flow regulation is due to a reduction in neuronal nitric oxide (nNOS).
    • A normal blood flow, normal arterial resistance group with normal Pv, which is denoted as "normal flow" POTS and is characterized by a normal supine phenotype, with normal peripheral resistance when supine but enhanced peripheral resistance when upright. Specific venous pooling within the splanchnic vascular bed is observed, making this a redistributive form of hypovolemia.
    • A high blood flow, low arterial resistance group with normal-to-decreased Pv, which is denoted as "high-flow" POTS. It is related to a long tract neuropathy and is characterized by high cardiac output caused by inadequate peripheral vasoconstriction when supine and upright. Patients are typically acyanotic and warm to the touch with extensive filtration, resulting in dependent edema."(38)
  • So the definition and classifications of POTS are a bit wishy washy, and there seems to be some disagreement within the filed, but at least everyone agrees that POTS is a SYNDROME, defined by an increase of heart rate of at least 30 beats per minute upon standing within a 10 minute period. 

  • This means something is causing your POTS, by definition of the name, being that it is a SYNDROME When someone (a doctor, for instance) says your POTS is idiopathic, that does not mean it has no cause, it means is has no KNOWN cause. There is a big difference. The medical dictionary definition of idiopathic: "idiopathic [id″e-o-path´ik] self-originated; occurring without known cause."

  • Stating someone, "just has POTS," infers that it is no big deal, and does not need to be investigated.  Other syndromes are often marginalized, such as Fibromyalgia Syndrome, or Chronic Fatigue Syndrome, or Irritable Bowel Syndrome.  Well, AIDS was a syndrome (that's what the "S" stands for ), but they eventually found the cause (HIV), and it is now classified as a disease.  And it's pretty darn serious.  Medscape defines POTS as "... a relatively benign disorder that is often self-limiting."   FYI-They will be hearing from me regarding this mis-characterization.  

Since POTS is by definition a syndrome caused by something else, lets look at the known causes of POTS and see if this syndrome is "no big deal."  Finding the cause of your POTS can lead to better medication and targeted treatments - as the different medications and non-pharmaceutical treatments are geared towards different causes. 



Some of the Major Known Causes of POTS:



Hypovolemia:  This is a condition of low blood volume (specifically plasma), different from traditional dehydration, that is very highly correlated with POTS thought to be a main cause/symptom.  It can co-exist with many of the other causes listed below.  Not enough blood is getting to the brain and vital organs, especially upon standing, and this can cause the increase in heart rate and lightheadedness associated with POTS. (5,6)  I wonder what a constant shortage of blood does to a person's body long term, but that is conjecture.  In terms of science, conclusions from the study conducted by Dr. Robertson and Dr. Raj (and others, see 6) stated:

"Patients with POTS have paradoxically unchanged plasma renin activity and low aldosterone given their marked reduction in plasma volume. These patients also have a significant red blood cell volume deficit, which is regulated by the renal hormone erythropoietin. These abnormalities suggest that the kidney may play a key role in the pathophysiology of POTS."
Hypovolemia is also known as Hypovolaemia, and can mean a hidden form of anemia as well.   Find great info on the Renin-Angiotensin-Aldosterone System here.


Hyperadrenergic state:  This is characterized by a measure of plasma norepinephrine (NE) levels in both a supine (laying flat) and standing position (at least 15 minutes in each position prior to blood sampling). The supine norepinephrine is often high normal in patients with POTS, while the upright norepinephrine is usually elevated (>600 pg/ml), a reflection of the exaggerated neural sympathetic tone that is present in these patients while upright (2,8).  Most researchers agree that this makes up about 10-29% of the POTS population (2,4,8,9,13).
In some subjects, this hyperadrenergic response may simply be a compensatory reaction to either hypovolaemia or peripheral dysautonomia with venous pooling.(2)
Summary: Increased NE is released by the body to tighten veins to prevent pooling in the body, or as a response to hypovolemia.


Acrocyanosis (Blood Pooling), or impaired vascular innervation: blood pooling either in the extremities or in the abdomen is well documented in POTS patients (1-10,16,17). "Impaired innervation of the veins or their response to sympathetic stimulation plays a key role in the aetiopathogenesis of POTS. This leads to dependent venous pooling in the legs and reduced venous return to the heart. Thus there is redistribution of blood in the peripheral circulation. This could be worsened by capillary leakage, which leads to further hypovolaemia and causes reflex tachycardia. There is sympathetic cardiac activity without vasoconstriction, inducing a fall in central venous pressure. When this process continues, circulatory collapse may occur." (17)
Summary: blood pooling causes your heart to work harder and beat faster, in an attempt to circulate blood to vital organs, including your brain.  Blood pooling is another "symptom" that is caused by something else, and the cause of the blood pooling needs to be found (many times it is a form of neuropathy, and/or can be found in conjunction with hypovolemia).

Muscle pump defects: "Physical forces comprise a primary defense against the pooling of blood in the dependent lower extremities in human beings. This occurs through the activity of the skeletal muscle pump, in which contractions of leg and gluteal muscles increase interstitial pressure and propel sequestered venous blood back to the heart. The efficacy of this pumping is augmented by the presence of one-way venous valves. Patients with incompetent venous valves or those in whom venous valves are congenitally absent suffer from severe orthostatic intolerance. Skeletal muscle may also be involved in neurogenic compensation through chemoreceptors and through local control mechanisms. Data indicate that although the muscle pump is normal in most patients with POTS, the muscle pump is defective in patients with low-flow POTS who also have decreased resting peripheral blood flow unrelated to exercise capability but exacerbated by bed rest. Therefore, ambulation is essential for the reduction of POTS symptoms. Lower body exercise may be very helpful, but its use has not yet been examined in a systematic way" (38)  Summary: Defects in valves or your muscles in the legs may cause pooling, thus causing POTS symptoms. 



Autonomic Neuropathy (AN): It is estimated that 50% of all POTS patients have neuropathy issues, specifically Autonomic Neuropathy (1,4), defined by the Mayo Clinic as:
"Autonomic neuropathy is a nerve disorder that affects involuntary body functions, including heart rate, blood pressure, perspiration and digestion.  It isn't a specific disease, instead autonomic neuropathy refers to damage to the autonomic nerves. This damage disrupts signals between the brain and portions of the autonomic nervous system, such as the heart, blood vessels and sweat glands, resulting in decreased or abnormal performance of one or more involuntary body functions."

Both Peripheral (PN), and specifically Small Fiber Neuropathy (known as SFN, a type of PN), have been associated with Autonomic Neuropathy, POTS, and other diseases that cause POTS, such as Diabetes and Sjogrens (30).  Several lines of evidence indicate that sympathetic denervation of the legs may be an underlying mechanism for POTS. (29) AN can be caused by many things, and are divided into Inherited, Aquired, Primary, and Secondary categories.  

POTS is listed as a Primary Aquired cause according to Medscape, where there is a detailed explanation of the various forms and causes of AN. "Postural orthostatic tachycardia syndrome (POTS) is a syndrome most common in young females with orthostatic intolerance characterized by palpitations with excessive orthostatic sinus tachycardia, sensation of lightheadedness, and near-syncope. POTS may be associated with an infectious prodrome and thus may represent the chronic sequelae of a forme fruste of post-viral pandysautonomia.  Antibodies against ganglionic receptors (AAG) are found in 9% of patients with POTS."(36) 

Small fibers specifically (SFN) involve skin and involuntary muscles, including cardiac and smooth muscles (autonomic fibers). Together, they control pain, thermal sensation, and autonomic function. (30) When autonomic fibers are affected, patients may experience dry eyes, dry mouth, orthostatic dizziness, constipation, bladder incontinence, sexual dysfunction, trouble sweating, or red or white skin discoloration.(33) Examination may show orthostatic hypotension and skin changes. The skin over the affected area may appear atrophic, dry, shiny, discolored, or mildly edematous as the result of sudomotor and vasomotor abnormalities. (30)

Summary: many of the diseases and conditions listed in this post cause AN, which can then cause POTS and Orthostatic Intolerance symptoms.  With a number as high as 50% of all POTS patients having some form of AN, it is my opinion that EVERYONE be checked thoroughly for it while hunting for the cause of your POTS.   POTSgrrl just did a series on her blog with some great resources and info about AN, check it out here.


Ehler's-Danlos Syndrome (EDS) and Joint Hypermobility Syndrome (JHS):  According to the The Ehlers-Danlos National Foundation, these are a rare, complex set of conditions defined as "a group of heritable disorders of connective tissue often characterized by hyperextensible skin, hypermobile joints, easy bruisability of the skin and a bleeding diathesis (tendency). EDS is named for two physicians (Ehlers and Danlos) who described forms of the condition in the early 1900's. Ehlers-Danlos syndrome may be classified into six major types. These types are not gradations in severity, but represent distinct disorders that "run true" in a family."(11).  Joint hypermobility syndrome (JHS) is one of the most common heritable collagen disorders. In addition to articular manifestations these patients can present with multiple extra-articular manifestations such as cutaneous scaring, ocular lid laxity, genitourinary dysfunction, peripheral nervous disorders and chronic fatigue syndrome. Autonomic dysfunction has been reported to occur in patients with JHS and include symptoms of syncope, presyncope, palpitations, chest discomfort, fatigue, and heat intolerance, orthostatic hypotension, postural orthostatic tachycardia syndrome, and uncategorized orthostatic intolerance. (10,12) 
Summary: What causes POTS in people with EDS? "Physicians propose that these syndromes occur together due to abnormal connective tissue in dependent blood vessels in those with EDS, which permits veins to distend excessively in response to ordinary hydrostatic pressure. Simply put, this connective tissue abnormally allows excessive amount of blood to pool in these patient’s lower limbs when they stand." (12)


Mast Cell Activation Disorder/Syndrome (MCA/MCAD/MCAS): This is another huge area where recent research is finding a fairly strong correlation between people with POTS and people with MCAD, especially the HyperPOTS group (31).  Mast cells are everywhere, and when they "act up" or degranulate inappropriately, symptoms such as flushing, hives, rashes, dermatographism (raised skin in response to light touch or scratching), and shortness of breath can occur, even anaphylaxis, on top of POTS symptoms. "MCA should be considered in patients with POTS presenting with flushing. These patients often present with a typical hyperadrenergic response, but β-blockers should be used with great caution, if at all, and treatment directed against mast cell mediators may be required." (31)  Find more info and links in the STOP POTS library.
 

Adrenal Disorders:  Your adrenal glands are located on the top of each kidney, and produce hormones (including sex hormones and cortisol), which help you respond to stress and have many other functions. Adrenal disorders can cause your adrenal glands to make too much or not enough hormones, and symptoms can mimic POTS. With Cushing's syndrome, there's too much cortisol, while with Addison's disease, there is too little. Some people are born unable to make enough cortisol. Tumors can also cause disorders in your adrenal glands., also mimicking/causing POTS symptoms.(26)


Autoimmune Diseases:  There are a number of autoimmune diseases found to coexist with and possibly cause POTS (maybe by causing neuropathy?).  I will list a few, but this is just a starting point, and they are in no particular order of occurrence.    

  • Autoimmune Autonomic Ganglionopathy (AAG) and Autoimmune Autonomic Neuropathy (AAN):  "The typical AAG case is a previously healthy young or middle-aged subject, more likely to be a female, presenting with a severe panautonomic failure that evolves within days to 1-2 weeks, similarly to the somatic counterpart Guillain-Barre' syndrome (GBS). The course is generally monophasic with slow, often incomplete recovery. The clinical picture is dominated by orthostatic hypotension, widespread anhidrosis, dry mouth, dry eyes, sexual dysfunction, urinary retention, impaired pupillary responses, reduced heart rate variability and gastrointestinal symptoms ranging from gastroparesis (manifesting as early satiety, postprandial abdominal pain, bloating and vomiting), diarrhea, constipation and in the most severe cases intestinal pseudoobstruction. the term autoimmune autonomic ganglionopathy (AAG) has replaced the more generic term autoimmune autonomic neuropathy (AAN). The autoimmune disorder was suspected in the original description when it was considered as an autonomic variant of Guillain-Barre' syndrome." (15) As in GBS, an antecedent event, such as a viral syndrome, recent immunizations or surgical procedures, is often reported. (15) Mayo Clinic findings report 15-25% of their patients with POTS have AAG. (16)  "Milder forms of dysautonomia, such as postural tachycardia syndrome, are associated with ganglionic AChR in 10-15% of cases." (49)   

  • Sjogren's :  I can't find a single paper on Sjogren's and POTS compared specifically (please send me a link if you have one!), but Sjogrens is cited in multiple papers as a potential cause (18).  Sjogren's is known to cause forms of neuropathy, which can cause POTS.(21-23)  POTSgrrl has many Sjogren's related posts on her blog, and it is worth checking out if you fit the profile.   Sometimes Autoimmune Autonomic Neuropathy can be caused by Sjogren's (21). "The possibility of Sjögren's syndrome should be considered in patients, especially women, who develop acute, subacute, or chronic sensory and autonomic neuropathies, with ataxia and kinesthetic loss." (22) "As described in epidemiology section, peripheral neuropathy is the most common neurological complication of pSS"(23)  Find lots of great info on symptoms and diagnosis via The Sjogren's Foundation here.

  • Fibromyalgia (FM): Many POTS patients also suffer from FM, which is another syndrome, but I'm including it because of the extreme prevalence.  It is not known if FM causes POTS, or vice versa, or if they simply co-exist, but research is being done regarding the relationship. (19,20) "fibromyalgia (FM) syndrome is defined by chronic widespread pain and tenderness, additional symptoms, including disabling fatigue and dizziness, are often reported by patients with this chronic illness. Although nonrestorative sleep may play an important role for chronic fatigue in FM, other mechanisms, including dysfunction of the autonomic nervous system (ANS), need to be considered....One of the most common events experienced by FM patients during tilt table testing is postural orthostatic tachycardia syndrome, which is defined as a heart rate increase of more than 30 beats per minute after more than 3 minutes of standing upright." (19) 

  • Chronic Fatigue (CFIDS/ME/CFS): Chronic fatigue syndrome (CFS), also known as chronic fatigue and immune dysfunction syndrome (CFIDS), myalgic encephalomyelitis (ME) and by other names, is a complex and debilitating chronic illness that affects the brain and multiple body systems.(32)  There is a ton of overlap with CFS and POTS, and recent findings suggest that people with POTS experience it in a more severe state that is more debilitating. "CFS (chronic fatigue syndrome) is commonly co-morbid with POTS (postural tachycardia syndrome). Individuals with CFS/POTS experience unrelenting fatigue, tachycardia during orthostatic stress and ill-defined neurocognitive impairment, often described as 'mental fog'."(37)

  • Guillain-Barré Syndrome: Dysautonomia is commonly observed in Guillain-Barré syndrome (GBS). (34)  Autonomic involvement is common and an important cause of morbidity and mortality in Guillain-Barré syndrome.(35) In severe cases of GBS, loss of autonomic function is common, manifesting as wide fluctuations in blood pressure, orthostatic hypotension (a fall in blood pressure on standing, leading to an increased risk of collapse), and cardiac arrhythmias.

  • Others mentioned in the literature: There are many other autoimmune diseases and conditions that found to coexist with or possibly cause POTS.  Some mentioned in the literature and by others are Lupus, Amyloidosis, Ankylosing spondylitis, Raynaud's, Celiac, and generalized connective tissue disorders.

Sudden Onset: Post-Viral Onset - it is though that sudden post viral onset of POTS is due to an autoimmune response. Approximately 50% of cases report an acute or subacute onset, often following a viral illness (such as the Epstein-Barr (EB) virus, aka "mono", or a similar herpes related virus), suggesting an immune-mediated process.(15) It is thought that an underlying autoimmune condition is "triggered" by a viral event, thus causing POTS. This is also thought to be a common cause for adolescent POTS.
Pregnancy and traumatic events (such as surgery, car accidents, head trauma, etc...) can also cause POTS, "Patients frequently report that their symptoms began following acute stressors such as pregnancy, major surgery, or a presumed viral illness, but in others cases, symptoms develop more insidiously."(2) " It is common after stress such as sepsis, pregnancy, fever, surgery or trauma." (17)
Many people with Lyme disease report this as well - that the infection is worsened after a sudden onset event, triggering POTS.


Cardiac Diseases: YOU MUST RULE THESE OUT.  These are the things that can cause POTS symptoms (or coexist with POTS) that can be life threatening if untreated.  They include: structural abnormalities (congenital defects or "holes" in the heart, valve defects, Cardiomyapathy), Cardiac Atrophy - or deconditioning causing the heart to get smaller (the Grinch Syndrome!),  and any other defect of the heart.  Demand a full cardiac work up if you are told you have POTS to rule out any structural or electrical issues with your heart.


Diabetes:  There are a few different types of diabetes, some of which can cause symptoms of POTS.  It is a HUGE topic (check out the Diabetes Basics here), and way too much info to summarize.  Diabetes causes autonomic neuropathy (39) - which sometimes causes POTS symptoms, and diabetes insipidus "is a condition in which the kidneys are unable to conserve water." This loss of water can cause dehydration, hypovolemia, and POTS symptoms. 

Lyme/Chronic Lyme: More and more people with POTS are finding they have Lyme, and vice versa.  It is a HUGE topic, very political, complex, and out of my scope of knowledge.  I would prefer to have a guest blogger post something very detailed about this, instead of me doing a general overview (any takers???).  But Lyme is known to cause POTS, and you should look into it of you live or have been to an area that is know to have Lyme disease.  (25) "In an appropriate clinical setting, evaluation for POTS in patients suffering from post LD syndrome may lead to earlier recognition and treatment, with subsequent improvement in symptoms of OI." (7) This cited paper by Dr. Grubb has great info on people treated with antibiotics for Lyme that develop POTS down the road, which he terms "post treatment LD syndrome," and many links to other papers regarding POTS and Lyme. (Resources for Lyme: 25. Truth About Lyme Disease, Lyme Net, Igenex Testing)


Mitochondrial Disease:  "Mitochondrial cytopathies are a diverse group of inherited
and acquired disorders that result in inadequate energy production. They can be caused by inheritable genetic mutations, acquired somatic mutations, exposure to toxins (including some prescription medications), and the aging process itself
." (41) According to Mito Action, "
It is not immediately clear why mitochondrial dysfunction would affect the autonomic nervous system, although the two are surely related. The mitochondria affect certain abilities of the autonomic nervous system, including the ability to sense temperature (the mitochondria are important in providing additional energy needed to generate heat). The functions of the muscles rely on the mitochondria, as does the brain. In this way, the relationship is complex and can be difficult to understand. It is probably more helpful to think about some of the symptoms of dysautonomia instead." Orthostatic intolerance/POTS might be a significant feature of autonomic nervous system dysfunction in patients suffering from mitochondrial cytopathy. (40) Need another guest poster please!  This is a BIG and serious group of diseases/conditions and deserves an "expert" write up.



Chiari Malformations/Hindbrain compression/Syringomyelia:
This is also hot topic.  Chiari has been thought to cause POTS. (42) "Orthostatic intolerance (POTS), seen predominantly in young women, is characterized by symptoms of lightheadedness, fatigue and palpitations in the upright posture. With standing, plasma norepinephrine levels rise dramatically and heart rate often increases by more than 30 beats per minute, although blood pressure does not usually fall. A theory recently popularized in the media suggests that some cases of orthostatic intolerance are related to hindbrain compression, with or without a Chiari I malformation"(45) See the Chiari Institution (the leading center in the country) for more detailed info on all conditions related to Chiari and Syringomyelia, which is"damage to the spinal cord due to the formation of a fluid-filled area within the cord." according to the NIH, which can also cause POTS like symptoms.



Multiple Sclerosis (MS): "Multiple sclerosis is an autoimmune inflammatory demyelinating disease of the central nervous system that is a leading cause of disability in young adults. Autonomic dysfunction is commonly seen in patients with MS. In patients of MS, autonomic dysfunction has been thought to be related to involvement of reflex pathways in the brainstem. Autonomic dysfunction in the form of POTS can occur in MS patients." (14)

   

Other cited possible causes and areas of research:

  • Increased Angiotensin II and decreased Nitric Oxide (NO): "We described a subset of “low flow POTS” in which marked upright tachycardia is associated with supine pallor, acrocyanosis, and hypovolemia, tachycardia, decreased cardiac output, and increased peripheral resistance . We observed increased plasma angiotensin-II (Ang-II) not accounted for by changes in angiotensinogen, renin, or angiotensin converting enzyme (ACE). Increased Ang-II and decreased nitric oxide (NO) increase central and peripheral sympathetic activity." (27) "Patients with POTS have blunted vasopressor response to Ang II and impaired baroreflex function. This impaired vasoconstrictive response might be exaggerated with upright posture and may contribute to the subsequent orthostatic tachycardia that is the hallmark of this disorder." (46)
  • Liver Cirrhosis/Disease: "Peripheral arterial vasodilation is one of the earliest observations in the cirrhotic patient and experimental animals with cirrhosis. Arterial vasodilators and arteriovenous fistula are other examples in which renal sodium and water retention occur secondary to a decreased filling of the arterial vascular tree. An increase in cardiac output and hormonal stimulation are common features of cirrhosis, arteriovenous fistula and drug-induced peripheral arterial vasodilation." (43) Updated: "In cirrhosis of the liver, according to the peripheral arterial vasodilation hypothesis, relative underfilling of the arterial tree triggers a neurohumoral response (activation of renin-angiotensin-aldosterone system, sympathetic nervous system, nonosmotic release of vasopressin) aimed at restoring circulatory integrity by promoting renal sodium and water retention" (44)
  • Other parasites, such as Chagas
  • Paraneoplastic neurological syndromes (PNS): Can be defined as remote effects of cancer that are not caused by the tumor and its metastasis, or by infection, ischemia or metabolic disruptions. (24)
  • The Nutcracker Syndrome:  A rare clinical complex which occurs in relatively young and previously healthy people, which is characterised by compression of the left renal vein (LRV) between the aorta and superior mesenteric artery (SMA) (48) "symptoms filled the criteria of chronic fatigue syndrome or idiopathic chronic fatigue (CFS/CF). An association between severe NC and autonomic dysfunction symptoms in children with CFS/CF has been presented." (47) 
  • Mycoplasma pneumoniae: an infection of the lungs from the bacteria Mycoplasma pneumonia. "Postural orthostatic tachycardia syndrome (POTS) is due to disruption of the body's autonomic system. Infections, especially viral, have been postulated as possible triggers for this syndrome. Mycoplasma pneumoniae has never been reported to be associated with POTS though most of the organism's extrapulmonary manifestations have an immune basis. We report a case of POTS associated with acute mycoplasma infection." (50) 
  • Norepinephrine transporter protein deficiency: Another recent autonomic condition producing a complex form of POTS is NET protein deficiency. This has been reported in only a single family.  Investigators have identified a single point mutation in the NET protein with both central and peripheral effects on vascular regulation. (38)

Hopefully this is enough to get you started.  After this giant science review/project, I have come to a few humble conclusions and suggestions for solving the problem with POTS:

A.  Fix the naming issue.  Since POTS is a SYNDROME, name each cause of "primary POTS" as a disease.  If you are chronically Hypovolemic, that is your disease - Chronic Hypolvolemia.  If you have HyperPOTS, you have Hyperandregenic Disease.  You get the point.

The definition of a disease is:

dis·ease  (d-zz)
n.
1. A pathological condition of a part, organ, or system of an organism resulting from various causes, such as infection, genetic defect, or environmental stress, and characterized by an identifiable group of signs or symptoms.
2. A condition or tendency, as of society, regarded as abnormal and harmful.
3. Obsolete Lack of ease; trouble.
The American Heritage® Dictionary of the English Language, Fourth Edition copyright ©2000 by Houghton Mifflin Company. Updated in 2009.

We need official, descriptive names for all the "brands" of POTS - name these suckers, and we will be treated more seriously in the ER and by medical professionals in general.  When I go, I NEVER say POTS unless I know the nurse/doctor - I say dysautonomia, or orthostatic hypotension, or autonomic neuropathy, or some other big scary sounding fancy name that will get their attention (but is true).  For some reason, the name POTS is just not taken seriously (maybe due to the obvious affiliations with flowers, cooking, and marijuana?).   


B. Uniform Diagnostic Criteria:  The Autonomic Consortium, or someone, needs to come up with a diagnostic flow chart for doctors for patients that have an idiopathic or "just POTS" diagnosis.  (Maybe this will be my next big project, in conjunction with Dysautonomia International)



C.  AWARENESS AWARENESS AWARENESS: Getting ALL medical professionals up to date on POTS, so there is no more "lazy medicine" when it comes to a POTS diagnosis.  No more "you just have POTS, increase your salt and fluid", or "florinef and beta blockers" as the gold standard.  They need to know that they HAVE TO FIND THE CAUSE, and any coexisting diseases, conditions, and/or health isues.

And not just doctors need to know that POTS is a symptom that something else is wrong; EMT's, Nurses, CNA's, etc...all need to know as well.  Being that 1 in 100 kids have POTS, all the schools and pediatricians need to understand POTS as well.  Then on to the general public. If you say "MS" - most folks kind of know what it is, that is serious, and that it exists. We need the same for POTS.

ALL DONE.  At last.  I hope this is helpful, and if I missed any causes or issues that co-exist with POTS, please please please let me know.  As I stated, I didn't use any other organization's website to do this, I wanted it to be independent - so I expect it may be incomplete.  Would love feedback!


 ------------------------------------------------------

 Sources (not all cited according to accepted standard, but links provided):

1.  Postural tachycardia syndrome (POTS) Neurology. Low PA, Opfer-Gehrking TL, Textor SC, et al. 1995;45:S19–25. [PubMed]

2. The postural tachycardia syndrome (POTS): pathophysiology, diagnosis – management. Raj SR. Indian Pacing Electrophysiol J. 2006;6:84–99. [PMC free article] [PubMed]

3.  Sleep Disturbances and Diminished Quality of Life in Postural Tachycardia Syndrome. Raj SR.. J Clin Sleep Med. 2011 April 15; 7(2): 204–210. 

4. Consensus statement on the definition of orthostatic hypotension, neurally mediated syncope and the postural tachycardia syndrome. Freeman R et al. , Auton Neurosci. 2011 Apr 26;161(1-2):46-8. Epub 2011 Mar 9.

5. The Autonomic Dysfunction Consortium, NIH Rare Diseases Network 

6. Renin-aldosterone paradox and perturbed blood volume regulation underlying postural tachycardia syndrome. Raj SR, Biaggioni I, Yamhure PC, et al. , Circulation. 2005;111:1574–1582. [PubMed] 

7. Postural orthostatic tachycardia syndrome following Lyme disease. Kanjwal K, Karabin B, Kanjwal Y, Grubb BP, Cardiol J. 2011;18(1):63-6. (PUBMED)


8.  Postural Tachycardia Syndrome, Blair P. Grubb, MD, Clinician Update, Circulation. 117: 2814-2817 doi: 10.1161/​CIRCULATIONAHA.107.761643

9. Postural Tachycardia Syndrome (POTS), Low PA, Journal of Cardiovascular Electrophysiology
Volume 20, Issue 3, pages 352–358, March 2009

10.  Comparative Clinical Profile of Postural Orthostatic Tachycardia Patients With and Without Joint Hypermobility Syndrome, Khalil Kanjwal, MD, Bilal Saeed, MD, Beverly Karabin, PhD, Yousuf Kanjwal, MD, and Blair P Grubb, MD, Indian Pacing Electrophysiol J. 2010; 10(4): 173–178. 

11. The Ehlers-Danlos National Foundation

12. Orthostatic intolerance and chronic fatigue syndrome associated with Ehlers-Danlos syndrome. Rowe PC, et al. J Pediatr. 1999;135:494. [PubMed] 

13.A review of postural orthostatic tachycardia syndrome.  Carew et al., Europace 11 (1): 18-25. doi: 10.1093/europace/eun324

14. Autonomic Dysfunction Presenting as Postural Orthostatic Tachycardia Syndrome in Patients with Multiple Sclerosis. Kanjwal K, Karabin B, Kanjwal Y, Grubb BP. Int J Med Sci 2010; 7(2):62-67. Available from http://www.medsci.org/v07p0062.htm 

15.Other Autonomic Neuropathies Associated with Ganglionic Antibody. Paola Sandroni and Phillip A. Low, Auton Neurosci. 2009 March 12; 146(1-2): 13–17. Published online 2008 December 4. 

16. The spectrum of autoimmune autonomic neuropathies. Klein C, et al. Ann Neurol. 2003;53:752–758. [PubMed]

17. Postural orthostatic tachycardia syndrome. Agarwal AK, Garg R, Ritch A, Sarkar P. Postgrad Med J. 2007 Jul;83(981):478-80.

18. Orthostatic hypotension: causes, classification, and treatment. Grubb BP, Kosinski DJ, Kanjwal Y. Pacing Clin Electrophysiol. 2003 Apr;26(4 Pt 1):892-901. 

19. Autonomic dysfunction in fibromyalgia syndrome: postural orthostatic tachycardia. Staud R. Curr Rheumatol Rep. 2008 Dec;10(6):463-6.

20. Neurally mediated hypotension in systemic lupus erythematosus patients with fibromyalgia.Tang S, Calkins H, Petri M. Rheumatology (Oxford). 2004 May;43(5):609-14. Epub 2004 Feb 24.

21. An Algorithm for the Evaluation of Peripheral Neuropathy.  ANN NOELLE PONCELET, M.D., Am Fam Physician. 1998 Feb 15;57(4):755-764.

23. Neurological Disorders in Primary Sjögren's Syndrome. Tobón GJ, et al. Autoimmune Dis. 2012;2012:645967. Epub 2012 Mar 5.

24. Paraneoplastic neurological syndromes, Jérôme Honnorat and Jean-Christophe Antoine,
Orphanet J Rare Dis. 2007; 2: 22. 

26. National Institutes of Health  

27.  Defects in cutaneous angiotensin-converting enzyme 2 and angiotensin-(1-7) production in postural tachycardia syndrome.Stewart JM, Ocon AJ, Clarke D, Taneja I, Medow MS. Hypertension. 2009 May;53(5):767-74. Epub 2009 Mar 16.

28.Postural Tachycardia syndrome. Low P, Opfer-Gehrking T, Textor S, et al. (POTS). Neurology 1995; 45:519-525.

29. The Neuropathic Postural Tachycardia Syndrome Robertson et al.,N Engl J Med 2000; 343:1008-1014 October 5, 2000

30.  Small fiber neuropathy: A burning problem, JINNY TAVEE, LAN ZHOU, MD, PhD
Neuromuscular Disease Center, Neurological Institute, Cleveland Clinic, Cleveland Clinic Journal of Medicine vol. 76 5 297-305. 
 
31. Hyperadrenergic Postural Tachycardia Syndrome in Mast Cell Activation Disorders. Shibao C et al., Hypertension. 45: 385-390.

32. CFIDS Association of America

33.Small-fiber neuropathy. Lacomis D Muscle Nerve. 2002 Aug;26(2):173-88. (Medline)

34. Orthostatic hypotension as a presenting symptom of the Guillain-Barré syndrome. Zhang Q et al. Clin Auton Res. 2010 Jun;20(3):209-10. Epub 2010 Jan 8.


35..Immune-mediated autonomic neuropathies. Etienne M, Weimer LH. Curr Neurol Neurosci Rep. 2006 Jan;6(1):57-64.

36. Clinical and laboratory indices that enhance the diagnosis of postural tachycardia syndrome. Mayo Clin Proc. Dec 1998;73(12):1141-50. Novak V, Novak P, Opfer-Gehrking TL, et al. [Medline].

37. Increasing orthostatic stress impairs neurocognitive functioning in chronic fatigue syndrome with postural tachycardia syndrome. Ocon AJ, Messer ZR, Medow MS, Stewart JM. Clin Sci (Lond). 2012 Mar;122(5):227-38.

38. Orthostatic Intolerance Review. Julian M Stewart, MD, PhD  Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College.

39.Diabetic autonomic neuropathy. Vinik AI, Maser RE, Mitchell BD, Freeman R. Diabetes Care. 2003 May;26(5):1553-79.

40.  Clin Cardiol. 2010 Oct;33(10):626-9.  Autonomic dysfunction presenting as orthostatic intolerance in patients suffering from mitochondrial cytopathy. Kanjwal K, Karabin B, Kanjwal Y, Saeed B, Grubb BP.

41. Mitochondrial cytopathy in adults: What we know so far. Cleveland Clinic Journal of Medicine vol. 68 7 625-626 

42.  Orthostatic intolerance and syncope associated with Chiari type I malformation. Prilipko O, Dehdashti AR, Zaim S, Seeck M. J Neurol Neurosurg Psychiatry. 2005 Jul;76(7):1034-6.

43. Peripheral arterial vasodilation hypothesis: a proposal for the initiation of renal sodium and water retention in cirrhosis. Schrier RW, Arroyo V, Bernardi M, Epstein M, Henriksen JH, Rodés J. Hepatology. 1988 Sep-Oct;8(5):1151-7.

44.  Update on peripheral arterial vasodilation, ascites and hepatorenal syndrome in cirrhosis. Knotek M, Rogachev B, Schrier RW. Can J Gastroenterol. 2000 Nov;14 Suppl D:112D-121D.

45.  No increased herniation of the cerebellar tonsils in a group of patients with orthostatic intolerance. Garland EM, Anderson JC, Black BK, Kessler RM, Konrad PE, Robertson D. Clin Auton Res. 2002 Dec;12(6):472-6.

46. Altered systemic hemodynamic and baroreflex response to angiotensin II in postural tachycardia syndrome. Mustafa HI, Raj SR, Diedrich A, Black BK, Paranjape SY, Dupont WD, Williams GH, Biaggioni I, Robertson D. Circ Arrhythm Electrophysiol. 2012 Feb 1;5(1):173-80. Epub 2012 Jan 13.

47. Does severe nutcracker phenomenon cause pediatric chronic fatigue?Takahashi Y, Ohta S, Sano A, Kuroda Y, Kaji Y, Matsuki M, Matsuo M. Clin Nephrol. 2000 Mar;53(3):174-81.

48.  Ann Vasc Surg. 2011 Nov;25(8):1154-64. Epub 2011 Mar 25. The nutcracker syndrome.
Venkatachalam S, Bumpus K, Kapadia SR, Gray B, Lyden S, Shishehbor MH. Ann Vasc Surg. 2011 Nov;25(8):1154-64. Epub 2011 Mar 25
50. Postural Orthostatic Tachycardia Syndrome Associated With Mycoplasma pneumoniae.Kasmani, Rahil MD, MRCP; Elkambergy, Hossam MD; Okoli, Kelechi MD, MRCP. Infectious Diseases in Clinical Practice: September 2009 - Volume 17 - Issue 5 - pp 342-343









 

42 comments:

  1. you didnt touch basis on the genetic aspect of POTS, as there is a hereditary component in some families. Also they are now saying that 50% of patients with Fibro actually have a form of EDS. There is mitochondrial disease and also dysfunction which is much milder form…with over 300 types its very hard to figure it out all..but a lot of the symptoms overlap.

    There is also MCAD, Mastocytosis

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    Replies
    1. There are detailed descriptions of all the conditions you mentioned, including the only published documented genetic defect that causes POTS, the NET deficiency. Perhaps you didn't read ll the way down? Either way, they're all there. And this was not an EDS paper, but I'm sure she will add in any facts from published journal articles - as this is geared towards being credible with MDs, who want to see peer reviewed stats and data.

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  2. This is FANTASTIC. I have bookmarked it in order to harangue my doctors in future visits. Thank you thank you THANK YOU!!!

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    Replies
    1. Thanks!! Hope it is helpful. I'm all about aiding in haranguing doctors to get results :)

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  3. Well done. Reads very well and seems pretty comprehensive. There is current research looking at peripheral or kidney dopamine in some POTS patients. There are some new autoimmune processes also being investigated. RamaK

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    Replies
    1. Thanks Rama! Do you have specifics? I'd LOVE to add them, am especially interested in the dopamine/kidney research. Thanks!

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  4. Also at times the blueing can be caused by venous status as a result of excessive peripheral vasoconstriction rather than pooling as a compensatory mechanism for either stomach blood pooling or abnormal cerebral autoregulation.
    I forgot to abnormal cerebral autoregulation appears in many POTS patients and its unclear whether its the primary problem or a consequence of ineffectual coping mechanims.

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  5. This comment has been removed by the author.

    ReplyDelete
    Replies
    1. That's infuriating! I suspect you are not in the minority, so many of us get blown off and important dx's are missed! Glad you are getting answers, best of luck!

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  6. Beautifully done. Thank you! My doctor has used the term "migraneous POTS" which led to a change from a medication that did not work to a medication that has made a world of difference. I havent found this term in the literature... have you?

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    Replies
    1. Thanks. And no- I'm going to look it up! Will let you know/post any info I can find. I'm assuming they are related to migraines?

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    2. I wonder if this could be it> http://www.ncbi.nlm.nih.gov/pubmed/18392420

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  7. My POTS is more than likely secondary and caused by small fiber neuropathy and is progressive. I have been told that Dr Grubb thinks perhaps as much as 20% of POTS may be caused by SFN but there have been no real studies. Jane

    http://www.ncbi.nlm.nih.gov/pubmed/17701872

    Approach to the evaluation of small fiber peripheral neuropathy and disorders of orthostatic intolerance.
    Goodman BP.
    Source

    Department of Neurology, Mayo Clinic, Scottsdale, Arizona 85259, USA.
    Abstract

    Small fiber peripheral neuropathy is a frequently encountered neurological disorder, which can be difficult to diagnose. In this article, the differential diagnosis of small fiber neuropathy is discussed, along with role of autonomic testing, skin biopsy, and quantitative sensory testing, in establishing a definitive diagnosis of small fiber peripheral neuropathy. Disorders of orthostatic intolerance, including postural orthostatic tachycardia syndrome (POTS), are also discussed, emphasizing diagnostic evaluation and a treatment approach to these disorders.

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    1. Good article Jane! I'd love to see more studies in this area as well. I'm being biopsied next week, My doc thinks I have SFN as well. Do you know what is causing yours?

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    2. Mine is more than likely caused by chemical exposure while I was in the Army. I see Dr Vinik in Norfolk for my SFN. No, I am not a diabetic but he is the best in our area to see. He is difficult to get an appointment with but worth it. Your doctor will have to submit paperwork. http://www.evms.edu/evms-strelitz-diabetes-center/sdc-research-vinik.html

      Jane

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    3. Jane I see him too! Or at least saw him. I live 2 blocks away from EVMS :). Are you in Hampton Roads? I'm unfortunately switching from him because I can't wait 4-6 months for follow ups. There is a new Neuro in town that specializes in SFN and Dysautonomia, so I am seeing him., and he is great so far.
      Thanks again!
      Claire

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    4. I live in Newport News. I see him every 2 or 3 months now. At first it was more time between appointments. Who do you see?

      Jane

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    5. Oh...He mainly treats the organ destruction caused by the SFN. Dysautonomia is just along for the ride.

      Plus...I am the Jane that gave your group the dysautonomia sites and poster.

      Jane

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    6. Jane, if you feel like it - please email me at stoppotsvirginia@gmail.com, would love to ask you a few questions about him. Not sure if I should look for someone else. And not sure about the sites and poster reference? Sorry - brain foggy this AM :) Thanks again!
      Claire

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  8. Amazing article, my daughter has RSD/CRPS and EDS, she now suffers from POTS as well. She probably had it mildly before the RSD/CRPS but it is getting much worse. It has actually gotten much worse since the tilt table test. In those prone I am hearing/seeing that the tilt table test will argrevate POTS.

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    Replies
    1. I'm sorry your daughter is dealing with all those disabling and painful conditions! I've seen RSD linked to dysautonomia, it would be interesting to see how it related to POTS specifically. TTT seem to make us all feel terrible, I'm sorry again to hear this!

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  9. It is becoming more and more evident that POTS can occur after a TBI. Most doctors believe it is more 10% and some believe it is perhaps 20%

    Jane

    Autonomic dysfunction presenting as
    postural tachycardia syndrome following
    traumatic brain injury.... http://www.dynakids.org/Documents/POTSfollowingTBI.pdf

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    Replies
    1. Thanks Jane!! I can't believe I missed including that- I will add it in (duh *facepalm* for me!)

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  10. This is a lot of information to digest, thank you! I have NCS as well as a few other "fun" things but currently no POTS diagnosis.

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  11. Claire,

    Well written and well researched. I really appreciate all the work you put into this and you can bet it will be marked and in my files. I'm really enjoying your blog and hope your new project will be very sucessful too. We need people like you to advocate and help to get POTS better known. We're all on a journey together and hopefully we will find some answers to help improve the quality of our lives.

    Thanks for all your hard work,
    Issie

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  12. Fantastic job, Claire! Another side note to add...many of us (like me!) have multiple underlying causes, which further complicates the whole picture and definitely complicates treatment. So glad you spent the time to put this together...this direction is ESSENTIAL! Shae

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  13. Wonderful job. Thanks
    Naneen

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  14. Please add to your list of causes that certain MEDICATIONS can cause damage to the autonomic nervous system, resulting in POTS. We (her doctor and I) suspect that my daughter's POTS/autonomic neuropathy resulted from the 5 years of Lupron injections she received as a young child to treat precocious puberty. (Lupron, originally a chemotherapy drug, apparently has well-established effects on the autonomic nervous system.) Also, many veterans of the Gulf War suffer from POTS-like symptoms, possibly caused by vaccinations they received in anticipation of possible germ warfare and the like. In any event, many drugs can damage the autonomic nervous system, and many doctors are not aware of it.

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  15. Very useful info. Thank you! I have read, alo, that Acute Intermittant Porphyria can cause POTS. This is a listed cause on DINET.

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  16. This was very helpful. You did an incredible job of research. I have had some (underlying) cause of POTS since 2001. Have had problems with it off and on for past 11 years. It has been bad off and on since end of june. Now it is constant. Had a TIA (mini-stroke) couple of days ago. I am hoping to go to Mayo Clinic in Rochester MN. There is a Dr Low there I would like to see. Have you or anybody had any luck at this Clinic? Again thanks for the information.

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  17. Very informative article. My 16 year old daughter has many of the different autoimmune diseases and syndromes that you explained are linked to POTS, it makes alot of sense. We are sad, but hopeful. Thanks again the information.

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  18. So helpful I go to Cardiologist tomorrow after being sent from Doctor to Doctor being misdiagnosed for months.

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  19. I think anyone who is diagnosed with pots should be evaluated by a LLMD. lyme literate medical doctors are the only ones that will be able to give you a true diagnosis of lyme bc mainstream doctors refuse to treat lyme for a big controversial ordeal. you can not go to your regular doctor and get a diagnosis or treatment mainly bc the government created the disease and therefore is covering it up. please see the websites that is under lyme on here and get tested through igenex, treatment could help the pots go away.

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  20. I had heart irregularities for 5 years on and off after contracting and testing positive (to CDC criteria) for Lyme Borreliosis/Neuroborreliosis via IGeneX IgG WB in late 2008.

    90% of my heart irregularities occurred on laying down - usually to sleep at bedtime and consisted of: 1. exaggerated/emphasised beat, 2. irregular beat, 3. missed beat/stop then exaggerated re-start of beat, 4. totally random internal abdominal pains, 5. Subconscious alert /sinking falling feeling which woke me up, 6. totally random internal abdominal twitches/rippling sensations/muscles being involuntarily activated, 7. effects changed whether laying on my back, front, right side or left side and were not consistent/sometimes laying on my back kept symptoms minimised, sometimes say my right side was best for this etc.

    And didn't consist of 1. vast racing or slowing down of BPM. My beat always kept between 50 and 80 in a resting state averaging around and usually 60 BPM while all the above occurred. 2 No ill effects other than sometimes a feeling of heart muscle strain on waking the next morning. 3. Never felt dizzy or anything like that.

    Symptoms would start after laying down for 30s but sometimes take up to 10 minutes before starting.

    In October 2012 my symptoms got so bad that I had to resort to sleeping upright for 6 weeks and symptom 5 started occurring while i was upright and out and about. This drove me to decide on try IV Ceftriaxone treatment, to the J Burrascano guidelines 16th Edition 2008. 4 days per week.

    After 3 weeks of treatment I was laying down to sleep again with reduced symptoms 50%. After 4 weeks 70% and after 5 weeks 80%, 6 weeks 90%.

    To date 21/07/13 I get a relapse of symptom 5 when I get run down after going to bed late/having long exhausting days. But after recharging my energy levels this resides. Otherwise I'm lots better.

    I hope this information may help some people on this site.

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  21. Thank you! Your research and information and comments are very helpful!

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  22. I was diagnosed with MVPS/Dysautonomia with POTS after many years with no insurance and going to a bunch of doctors who all told me I was crazy. I had RSD around 1996 but was told my nerve would grow back as it was only stretched in my ankle not broken. It did slowly repair my foot. I tried to tell them that my RSD symptoms looked gone to them but I felt like my nervous system had been taken over. Sometimes electrical impulses will bother me such as my old vacuum cleaner or being around my laptop. I would get so weak I couldn't walk, even more so after a big meal, went to ER thinking I was having a heart attack only to be told I was just "stressed". Thank God for the Mitral Valve/Dysautonomia Center in Birmingham which diagnosed me after so many years. I am now NOT CRAZY. They asked me if I was having "a long list of symptoms" most of which I did. Then tilt table, in depth echo, and part of a stress test. Am now learning to live with it, and have seem great strides with lots of liquid. Have always been anemic. They said my low blood volume and low blood pressure caused most of this. Is there some way to increase my blood volume without getting blood? Lots of water helps, but I can relapse quickly if I miss drinking water a lot or get stressed then Irritable Bowel starts! I REALLY BELIEVE THAT THE RSD started all this and now my autonomic nervous system is shot.

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  23. Recent published work has implicated competitive autoantibodies against alpha 1 adrenoreceptor and some voltage gate autoantibodies in POTS. QSART is abnormal in roughly 30% cases and epigenetic suppression of norepinephrine transporter may be common.

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  24. I have noticed that my hear rate fluctuates by 30 or 40 bpm when I stand. This is probably due to three factors. Firstly, I am treated for thyroid cancer and I have to take extra thyroid hormone which has made me chronically hyperthyroid for twenty years. Second, I have mild mitral valve regurgitation, which worsened from mitral valve prolapse that I found fifteen years ago. And third, I am tall at 6'4. Your mention of hyperthyroidism was spot on, and so was your discussion of heart structure abnormalities. The psychological strain of this disease is difficult, especially when I have the pulse monitor in my hand. It makes me feel bad. For the most part I have not noticed this condition until i become hyperthyroid from my medication, when my bpm shoots up from 75bpm sitting to 120 bpm standing. I become out of breath and I have chest pains. I chased a lot of doctors about this issue, and most were very dismissive. They looked that the wave forms of the beats of my heart and disregarded the variation in bpm. I know I feel cloudy when I think and I have fainted about five times, once wetting myself and collapsing in a McDonalds in Cairo. It was very embarrassing. I really hope you might expand on the hyperthyroidism and heart valve issue. I know that the valve condition is unlikely to reverse, but can I improve the situation with normal thyroid levels and exercise? What do you think?

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  25. Awesome love ur sense of humour too

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  26. Awsome information, love ur dense of him or too. Wish docs would stop diagnosing people with syndromes and that's it "their jobs done no more investigation required" been teuying to find answers for my crap for 15 years. Several docs an a few "specialists and I am still no closer. Till I hand my doc link to ur site that it. So trhanks .

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  27. Great article, and well put together. The issue of POTS versus Orthostatic Hypotension was particularly interesting. I have seen quite a number of posts on forums of people who have been diagnosed with POTS, who also get a significant drop in BP. But, as your info points out, the diagnostic criteria for POTS is the significant HR increase without a drop of 20/10 (Sys/Diastolic BP). So there seems to be a lot of confusion out there with both doctors and patients regarding POTS versus OH. When we first started taking BP and HR measurements for our daughter (who has EDS), the measurements showed mostly a significant HR increase, which fitted the criteria of POTS. However, that was done using lying down and then standing readings at home. When she was given a Tilt Table Test, it showed that her BP also fell significantly and immediately on being tilted. The cardiologist therefore diagnosed her with OH, and said the HR increase was an appropriate compensatory mechanism to try to increase the cardiac output. Because she did have this compensatory HR increase he said that there was no Autonomic Dysfunction. She did not receive any further testing to identify the cause of the OH, but from our research, and our observation of quite obvious blood pooling on standing, we gather it is due to hyper distension of the veins due to EDS. Even with the diagnosis of OH from the cardiologist, her regular GP's did not really understand that the OH is chronic, as opposed to the occasional "head rush" some young people get when they stand too quickly. We were told that "everyone gets that - it's not an issue". However, if she got OH when she was 72, instead of having had it already for 10 years at the age of 22, a diagnosis of OH would be taken seriously. Instead, she will have to contend with it all her life, with the added complication that OH increases with age, and yet she is expected to just ignore it and get on with things. While there is significant literature on POTS in the Ehlers Danlos Syndrome, there seems to be very little information on OH due to excessive venous pooling caused by EDS. This makes me wonder how many people with EDS have POTS, and how many with EDS have OH instead, and why some get POTS and some get OH when they all have a similar underlying disorder with their connective tissues?

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  28. My friend suggested me
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