Episode 21

Demystifying Dysautonomia with Svetlana Blitshteyn, M.D.

Nov 5, 2020 · 44m
Svetlana Blitshteyn, M.D

Description

Dysautonomia is an umbrella term used to describe disorders of the autonomic nervous system (which controls all the automatic functions of the body like blood pressure, heart rate, digestion, temperature regulation, etc.). Dr. Svetlana Blitshteyn, a board-certified neurologist and director of the Dysautonomia Clinic, joins us as we dig into these disorders and explore their defining features.  POTS (Postural Orthostatic Tachycardia Syndrome), small-fiber neuropathy, and neurocardiogenic syncope are frequent comorbidities with EDS (Ehlers-Danlos Syndromes), Marfan Syndrome, and other disorders of connective tissue. Dr. Blitshteyn discusses who is considered high-risk for POTS, explains neurocardiogenic syncope and small-fiber neuropathy, and talks about the difference between the three disorders. She explores common symptoms of POTS and outlines frequent comorbidities such as MCAS (Mast Cell Activation Syndrome), IBS (Irritable Bowel Syndrome), and EDS.  Finally, Dr. Blitshteyn answers the question - Is there a link between dysautonomia and autoimmune issues?  An in-depth exploration of dysautonomia and what that might entail for the hypermobile population, this episode is important for patients and healthcare professionals alike who are eager to learn about these complex disorders.  Learn about Dr. Blitshteyn, https://www.dysautonomiaclinic.com/ Facebook: https://www.facebook.com/DysautonomiaClinic/ Twitter: https://twitter.com/dysclinic  Learn more about Dr. Linda Bluestein, the Hypermobility MD at our website and be sure to follow us on social media: Website: https://www.hypermobilitymd.com Instagram: @hypermobilitymd Twitter: @hypermobilityMD Facebook: https://www.facebook.com/hypermobilityMD/ Pinterest: https://www.pinterest.com/hypermobilityMD/ LinkedIn: https://www.linkedin.com/in/hypermobilitymd/  And follow guest co-host Jennifer at the links below: Website: www.jennifer-milner.com Instagram: @jennifer.milner Facebook: https://www.facebook.com/jennifermilnerbodiesinmotion/

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Guests

Dysautonomia Clinic
Dr. Svetlana Blitshteyn is a neurologist and founder of the Dysautonomia Clinic, specializing in POTS, small fiber neuropathy, and related conditions. She is a Clinical Associate Professor of Neurology at the University at Buffalo.

Transcript

[00:11] Jennifer Milner: Welcome to Bendy Bodies with the Hypermobility MD, where we explore the intersection of health and hypermobility for dancers and other artistic athletes. This is co-host Jennifer Milner, here today with Dr. Linda Bluestein. Before we introduce today's special guest, please remember to subscribe to the Bendy Bodies Podcast and leave us a review. This really helps grow the audience and increase awareness about hypermobility and associated disorders. This podcast is for you.
[00:41] Today we have the great pleasure of speaking with Dr. Svetlana Blitshteyn, board-certified neurologist and director of the Dysautonomia Clinic. She's also the clinical assistant professor of neurology at the University at Buffalo Jacobs School of Medicine and Biomedical Sciences. Dr. Blitshteyn completed her neurology training at Mayo Clinic Graduate School of Medicine and is a member of the American Academy of Neurology and American Autonomic Society. She serves on the medical advisory board for multiple nonprofits including Dysautonomia International, Dysautonomia Information Network, and the Ehlers-Danlos Society.
Dr. Blitshteyn has been an invited speaker at national and international conferences including at the World Health Organization. She has been the principal investigator on a number of important research studies concerning POTS and autoimmunity, POTS and pregnancy, POTS and vitamin deficiencies, and others. She co-authored a popular patient handbook called POTS: Together We Stand: Riding the Waves of Dysautonomia and has been interviewed by numerous media outlets including U.S. News and World Report, Medscape, Neurology Today, New Scientist, and others.
[01:53] She's the recipient of the Patient's Choice Award 2019 from Dysautonomia Support Network, Business First 40 Under 40 Award, Mayo Clinic Neurology Research Award, the American Headache Society U.S. Human Health Award, the American Academy of Neurology Student Prize, and others. Dr. Blitshteyn, hello and welcome to Bendy Bodies.

[02:28] Svetlana Blitshteyn, M.D.: Thank you so much for having me today.

[02:29] Jennifer Milner: So let's start with the basics. What exactly is dysautonomia?

[02:33] Svetlana Blitshteyn, M.D.: Dysautonomia simply means abnormal autonomic nervous system, and it's synonymous with autonomic dysfunction of any kind. It's not a diagnosis, it's a descriptive term, kind of like headache, which doesn't specify what type of headache or why the headache is taking place. It's important to remind ourselves that there is sympathetic, parasympathetic, and enteric nervous system, and each of these systems can malfunction, resulting in specific syndromes and disorders.
[03:07] Within this umbrella term of dysautonomia, there are specific diagnoses with their objective criteria that have been defined. None of these autonomic disorder diagnoses are subjective — all have objective clinical criteria. The diagnosis relies on the assessment of blood pressure and heart rate in the supine and standing position using a simple 10-minute stand test that can be performed at the doctor's office, or a tilt table test.

[03:40] Jennifer Milner: Excellent. So what might make someone suspect that they have some sort of dysautonomia?

[03:50] Svetlana Blitshteyn, M.D.: Clinical symptoms of dysautonomia are numerous, and the most common ones involve orthostatic intolerance. That's the defining feature of an autonomic disorder, and what that means is difficulty standing, with multiple symptoms arising in the standing position and symptoms improving when you sit down or lie down. Of course, this is the hallmark, but there are other symptoms such as chronic dizziness, palpitations, tachycardia, lightheadedness, headaches, brain fog, sleep problems, and many others.

[04:34] Jennifer Milner: So are there specific populations that might be at a higher risk for dysautonomia? And as we are the Bendy Bodies Podcast, why should hypermobile people know about it?

[04:50] Svetlana Blitshteyn, M.D.: Dysautonomia, again as an umbrella term, encompasses a wide variety of autonomic disorders, the most common ones being postural orthostatic tachycardia syndrome and neurocardiogenic syncope. Both of these disorders commonly occur in young women between ages 15 and 50, but of course younger patients and older patients can also have POTS or neurocardiogenic syncope. Many of these women are of childbearing age, and most of the young women are Caucasian.
[05:28] How it relates to Bendy Bodies? Well, we know that one of the common comorbidities of POTS is Ehlers-Danlos syndrome, with multiple studies showing a prevalence of about 25 to 30%. And commonly when we have patients with POTS, we must think about evaluation and assessment for Ehlers-Danlos syndrome.

[06:00] Jennifer Milner: So you often will — if you see someone who has hypermobility and POTS, that will lead you to talk about looking at an EDS diagnosis?

[06:12] Svetlana Blitshteyn, M.D.: In my clinic, of course, I have many patients with EDS and POTS. How I figure this out is from history. When patients present to us, they're not going to volunteer this textbook information. Some will say that they have been hypermobile, but many will present with complaints of joint pain, body pain, easily dislocatable joints, easy bruising, and many other manifestations. So it's up to us clinicians to listen to the history and figure out: is this a patient that may have Ehlers-Danlos syndrome?
On the other hand, one may say that anyone presenting with POTS should be screened for EDS. And that's certainly a good practice in those of us who see a large number of patients with POTS.

[07:08] Dr. Linda Bluestein: So we know that in 2017, the criteria for all the Ehlers-Danlos syndromes changed with the International Consortium. In particular, the criteria changed for hypermobile EDS, and then they introduced the new classification, HSD, or hypermobility spectrum disorders. In your experience, does the overlap between dysautonomia and specifically POTS — does that hold true also for the hypermobility spectrum disorders, or is that more specific to EDS?

[07:44] Svetlana Blitshteyn, M.D.: It's a great question, and I don't think there is a definitive answer to that. All of the studies that have been done are looking at the association between POTS and Ehlers-Danlos syndrome, specifically the hypermobile type. But of course, the hypermobility spectrum disorders are much more common, and hypermobility as a sign is very common — and by no means does it constitute a disorder. This is something I have to always remind my neurology colleagues who think that all of the young women who present with migraine or dizziness are quite hypermobile — that they're all gymnasts or dancers or swimmers. But of course, we have to make the distinction that having hypermobility as a sign absolutely does not mean hypermobile Ehlers-Danlos syndrome. There are criteria that need to be met.

[08:46] Jennifer Milner: Yes, I agree with that. So when you're talking about looking at POTS with hypermobility, you said earlier that there are a couple of really easy tests for POTS — the stand test and the tilt table test. What is the full diagnostic workup that is recommended for POTS, and how prevalent is that kind of workup?

[09:13] Svetlana Blitshteyn, M.D.: I'll describe how I do these evaluations because I think it's important for clinicians to hear what the process may be. As with all neurologic evaluations, I start with the physical exam after I take a thorough history and listen to the patients and all of their complaints. The physical exam includes orthostatic blood pressure and heart rate measurement. You lay down the patient, allow them to rest for a few minutes, and then you measure their blood pressure and heart rate. After that, you stand them up and check their blood pressure and heart rate again in increments of 2 to 3 minutes for a 10-minute stand test. This easy in-office test is very important because it can give you clues whether a disorder of orthostatic intolerance is present. And now, in the time of social distancing, I ask the patient to perform the same test in the comfort of their own home, which gives me a good approximation of what their vital signs are doing while they're supine and standing.
[10:19] Following that, it's important to do a full neurologic exam with special attention to the sensory exam, because at least 50% of patients with POTS have comorbid small fiber neuropathy. Regarding diagnostic tests, I usually order a lot of tests to look for possible underlying causes, to exclude POTS mimics, and to look for common comorbid conditions. Some specialists, however, and even some consensus statements recommend minimal workup, which usually includes EKG, basic blood work, and thyroid function tests. But personally, I prefer to go beyond that in order to be thorough and not to miss any other diagnosis.
[11:03] Typically my patients have seen at least a dozen other physicians, and the basic workup is complete by the time they see me. In terms of neurologic evaluation, if the patient has headache — the most common comorbidity with POTS and also Ehlers-Danlos — I obtain an MRI of the brain without contrast, one time, to rule out structural abnormalities like AV malformation. In patients with POTS, MRI of the brain is usually entirely normal, which can be very reassuring. If there is significant sleep disturbance, which can happen in our patients with POTS and certainly in patients with Ehlers-Danlos syndrome, I refer the patient for a sleep study to rule out obstructive sleep apnea, central sleep apnea, narcolepsy, and other sleep disorders that may be present.
[11:58] In many cases, I refer the patient for a tilt table test for diagnostic confirmation. In some cases where access to a tilt table test is difficult, I rely on the 10-minute stand test, which we call Pullman's tilt table test. But most of the time when there is access to a tilt table test, I certainly order that.

[12:31] Jennifer Milner: That's great, thank you. You mentioned small fiber neuropathy. I know there are other issues, like Chiari malformation. So purely out of curiosity, just from what I've seen with some of my dancers — do you see a fair amount of Chiari malformations in people with connective tissue disorders?

[12:52] Svetlana Blitshteyn, M.D.: Yes, in patients with Ehlers-Danlos, I certainly see more Chiari malformation. But if you ask me that question in terms of my POTS patients, I would say no.

[13:05] Dr. Linda Bluestein: Could you explain for the listeners who are not familiar — could you explain what both Chiari malformation and small fiber neuropathy are, and why they are relevant in this conversation?

[13:17] Svetlana Blitshteyn, M.D.: Chiari malformation refers usually to a congenital malformation of the cerebellum — the back part of the brain — where normally it has to be above the foramen magnum, which is an opening between the skull and the spine. In Chiari malformation, you have the cerebellum, specifically the cerebellar tonsils, protruding down through the foramen magnum. And if it's protruding more than 5 millimeters down, that can be symptomatic. The symptoms may include those related to obstruction of CSF flow — of spinal cerebral fluid flow — and also those that can potentially compress the brainstem and the cerebellum itself.
[14:18] Naturally, when you have a connective tissue disorder with weakness and abnormalities in collagen, ligaments and even muscles may become loose and no longer normal in structure or function. That weakness in ligamental tissue may result in protrusion of the brainstem and cerebellum. Similarly, elsewhere in the body you may have similar situations that we refer to as hernias, where organs or tissues protrude through a weak muscular wall.
[15:02] Small fiber neuropathy refers to an abnormal structure of small nerve fibers that are everywhere in the body. Autonomic neuropathy is not the same as small fiber neuropathy — that distinction needs to be made. Thin unmyelinated alpha and delta fibers that underlie small fibers may be damaged, and they may be damaged by toxins, medications, antibodies, alcohol, diabetes, and many other conditions. This results in neuropathic pain and objective findings that we see on neurologic exam, which traditionally includes loss of temperature sensation and pain sensation. That's a very important part of the neurologic exam — that's how we can identify our patients with POTS and Ehlers-Danlos who may have small fiber neuropathy.
[16:10] At least 50% of patients with POTS have objectively confirmed small fiber neuropathy. And at least 70% of patients with hypermobile Ehlers-Danlos syndrome also have small fiber neuropathy. It's very important for clinicians to identify that. The way to diagnose it is, of course, through history, and the questions will surround neuropathic pain. Not always will patients have burning pain or numbness or tingling. Sometimes they will have itching. Sometimes they will describe a full body pain, a bone kind of pain. Sometimes they'll describe it as having rubber bands on their feet, and sometimes they will describe the pain as a sensation of heat or having heavy legs.
[17:04] When we have this history and findings on exam of sensory dysfunction, we must obtain two very important tests. One is an EMG, which is a test of muscles and nerves that will identify whether the patient has large fiber neuropathy. The second test is a skin biopsy to investigate whether the patient has small fiber neuropathy. So a very important point: when we in the medical world suspect that our patient has neuropathy, we must order two tests — one is the EMG test of large fibers, and the other is a skin biopsy, or test of small fibers.
[17:55] There is also a full autonomic function test that is only available in certain areas of the country and unfortunately isn't available everywhere. But there is an important test called QSART — quantitative sudomotor axon reflex test — that can measure sweat output and determine whether a patient has small fiber neuropathy.

[18:25] Dr. Linda Bluestein: Very good. And we wanted to have a bit more explanation about syncope. You mentioned earlier neurocardiogenic syncope, and some of our listeners will know what that means, while others won't. Could you explain a little more about that?

[18:44] Svetlana Blitshteyn, M.D.: Sure. Neurocardiogenic syncope is a condition that is generally viewed as an episodic autonomic disorder, meaning that in between the episodes of syncope, the patient remains asymptomatic. Now, we've got to make this distinction: syncope is very common in everyone. In a lifetime, at least 25% of all patients may have a syncopal event, especially when they're younger, and that accounts for a great majority of ER presentations. This is what we call a simple syncope, simple faint, or simple vasovagal syncope, as it is commonly called in the medical world. These are events that are common and not necessarily representative of a disorder.
[19:37] This becomes a disorder only when syncope is repetitive and occurs at a significant frequency. It obviously becomes disabling when it occurs very frequently and impairs your functional status. And clearly it becomes extremely disabling when you have syncope every time you stand up. It does commonly affect young people. It can occur with the onset of puberty, which is the most common age of onset not only for neurocardiogenic syncope, but also for postural orthostatic tachycardia syndrome. During a period of growth spurt or during the onset of menstruation, one can have this dysregulation of the autonomic nervous system. Again, in and of itself it may not represent a disorder, but when it's repetitive and affects your functional status, then it becomes a medical condition that needs medical attention.
[20:53] Briefly put, syncope just means an abrupt loss of cerebral blood flow. When that happens, you fall down, and that's sort of the body's natural way of restoring cerebral perfusion and blood flow back to your brain.

[22:16] Dr. Linda Bluestein: So syncope is basically a complete loss of consciousness, or people can also feel like they're going to pass out — what's called presyncope. Both of those would happen in, for example, POTS. Is that true?

[22:33] Svetlana Blitshteyn, M.D.: Let's make some distinctions here. POTS as a disorder has three clinical criteria. Criteria number one is that you need to meet a threshold of an increase in heart rate by at least 30 beats per minute within a 10-minute stand test or a tilt table test — and at least 40 beats per minute in teenagers or those under the age of 19. Criteria number two is that blood pressure remains stable during that time. And criteria number three is the chronicity of symptoms, where symptoms of orthostatic intolerance must be present for at least 6 months. That's the definition of POTS.
[23:24] The definition of neurocardiogenic syncope is an abrupt drop in both heart rate and blood pressure within 10 minutes of standing, associated with loss of consciousness. We also have criteria for orthostatic hypotension, which is another common disorder that involves a drop in blood pressure by more than 20 over 10 millimeters of mercury within 3 minutes of a tilt table test. And if the heart rate does not rise in compensation to a decreasing blood pressure, we call that neurogenic orthostatic hypotension.
[24:17] So now we know these three distinct patterns. You asked a very important question: in POTS, do these patients pass out? Do they come close? What's going on there? If we go by defined clinical criteria, once you meet criteria for POTS, by exclusion you cannot meet criteria for neurocardiogenic syncope. However, in the clinical world of reality, you know that some patients will faint as well. At least 25 to 30% of patients with POTS can faint. Most patients do not — most patients come close, they feel like they're going to faint, but they never do, which we call presyncope. We think it's because the physiology is still compensating. When you have a slower decrease in cerebral perfusion, perhaps you have activation of autonomic reflexes that are still preserving some perfusion to the brain based on this increase in heart rate, and therefore you do not pass out.
[25:35] Now, some people will have abnormal tilt table tests showing neurocardiogenic syncope. And as I mentioned before, traditionally, neurocardiogenic syncope is viewed as an episodic disorder, meaning that in between episodes of syncope the patient should be asymptomatic. Again, in reality, there are also patients who have neurocardiogenic syncope confirmed by tilt table test — they pass out — and in between episodes of syncope they will have disabling symptoms such as fatigue, lightheadedness, dizziness, headaches, and all the rest of the symptoms of dysautonomia.

[26:20] Jennifer Milner: So I wanted to follow up with you on some of the things that you mentioned — the fatigue and the headaches. Can you go a little bit more into what are some of the common symptoms and comorbidities that might go along with people who have been diagnosed with POTS?

[26:35] Svetlana Blitshteyn, M.D.: We divide symptoms of POTS into two categories. One is orthostatic — so there are orthostatic symptoms such as dizziness, palpitations, lightheadedness, generalized weakness on assuming an upright posture, and an overall feeling of faintness. The second category is non-orthostatic symptoms, and these include general symptoms such as fatigue, chronic dizziness, headaches, brain fog — which is difficulty concentrating — and other symptoms of cognitive dysfunction, sleep disturbance, and sometimes alterations in mood.
[27:26] Comorbidities are very common. As they always say, POTS rarely exists alone. At least 80% of patients with POTS have at least one significant comorbidity. The most common comorbidity, as I mentioned before, is small fiber neuropathy, occurring in at least 50% of patients with POTS. Another very common comorbidity is headache, occurring in at least 40% of patients with POTS. A third common comorbidity is Ehlers-Danlos, occurring in at least 25% of patients with POTS. And then we have other comorbidities — one of them is mast cell activation syndrome, another is irritable bowel syndrome. Comorbid autoimmune conditions affect at least 25% of all patients with POTS, with the most common autoimmune condition being Hashimoto's thyroiditis.

[28:27] Jennifer Milner: Excellent, thank you.

[28:28] Dr. Linda Bluestein: You wrote a paper on B1 deficiency in POTS. Would you be able to elaborate on that a little bit?

[28:40] Svetlana Blitshteyn, M.D.: Vitamin deficiency is quite common in our patients, with the most common being iron deficiency without anemia. Levels of vitamin B12 also appear to be lower in our patients compared to age-matched controls. In terms of vitamin B1 deficiency, I am aware only of my own study, and in my cohort of 65 patients, the prevalence of vitamin B1 deficiency was 4%. Unfortunately, only one patient out of that case series recovered with vitamin B1 supplementation. Regardless of that fact, I personally always check all of my patients with POTS for common vitamin deficiencies including B12, B1, D, iron, B6, and others.

[29:44] Jennifer Milner: Excellent, thank you. And looking at these disorders — at POTS specifically, but also some of the others that you've talked about — just so people know, how disabling can these disorders be, especially if not diagnosed and treated? And how could someone avoid getting to that point?

[30:10] Svetlana Blitshteyn, M.D.: From multiple studies, we know that POTS can be quite disabling — as disabling as congestive heart failure or COPD. At least 25% of our patients are unable to work or attend school. Prognosis has been a bit of a tricky question. About a decade ago, there was a notion among our colleagues that teens outgrow POTS. This statement came from one earlier study that suggested that 80% of patients no longer experience POTS 5 years after diagnosis. Many of us who specialize in POTS simply weren't seeing these high numbers in real life.
[30:57] More recently, there was a study out of Mayo Clinic that painted a more realistic picture by demonstrating that only about 50% of teens with POTS improve, and only 19% fully recover — with a mean follow-up of 5 years. While we would love to tell patients that POTS has a good chance of recovery, I think the reality is that POTS is going to be a chronic disorder that can fluctuate in severity for a majority of patients. The range of severity varies greatly, with some patients being able to work, travel, and participate in sports, while others will have significant difficulty doing all of those things. A realistic approach to long-term prognosis is a lot better than painting a rosy picture and telling young patients that they may outgrow POTS in their 20s, only to have their hopes shattered when that doesn't happen.
[32:05] Having said that, teens appear to have a better prognosis than adults, and I certainly have patients who were very sick during their teen years and unable to attend school but then improved enough to attend college and do other age-appropriate things. In summary, I tell my patients that prognosis is hopeful, but expectations for a full recovery need to be tempered, and lifestyle adjustments as well as some form of medical management should be expected.

[32:39] Dr. Linda Bluestein: I wanted to follow up on what you were just saying in terms of expectations and this age group. I'm thinking this ties in with the B1 question — this is a group of kids that would be doing age-appropriate things, which of course also includes drinking alcohol in many instances as they turn 21 — but with the potential for vitamin B deficiencies and the potential effects of alcohol on other hemodynamic variables. Do you have any advice for listeners about that?

[33:15] Svetlana Blitshteyn, M.D.: Interestingly, a lot of our patients have alcohol intolerance. They're not drinkers. They figure out early on that alcohol makes them feel worse. Alcohol may result in a drop in blood pressure. Alcohol causes flushing and may lead to mast cell activation. Alcohol causes sleep disturbance. Alcohol can cause balance difficulties. So many of our patients do not drink — they're hardly drinkers. Sometimes adults are able to tolerate a glass of wine here and there, but a majority of our patients do not consume alcohol.
[33:56] Now, teens will be teens and college students will be college students, but I do advise them to stay away from alcohol. And if you have to socialize and go to a party, limit your alcohol intake to no more than one drink per night. That's very little in the grand scheme of things, but it's not worth causing an exacerbation of your POTS and other comorbid conditions. It's not worth missing the next day of school because the night before you were drinking alcohol. So being very cognizant of the negative impact of alcohol on our patients, yet understanding that saying "don't ever drink alcohol" isn't realistic either. Limit your alcohol intake if you must to no more than one drink, and of course if you do drink, don't drive — ask for a ride.

[34:59] Jennifer Milner: Excellent, thank you. And then I'm wondering, in this whole big picture, how does autoimmunity fit into all of this?

[35:09] Svetlana Blitshteyn, M.D.: There has been significant research interest in the past few years to determine whether POTS is an autoimmune disorder, and while we haven't conclusively answered this question yet, it does appear that POTS may have a strong autoimmune basis. Years ago, I received a grant to study autoimmune markers and autoimmune disorders in patients with POTS. I noticed that a significant number of my POTS patients had positive markers of autoimmunity and comorbid autoimmune disorders. When we applied statistical analysis, we did find that POTS patients had a higher prevalence of antinuclear antibodies, antiphospholipid antibodies, and other autoimmune markers than the general population. The prevalence of most defined autoimmune conditions was higher in my cohort of 100 patients with POTS than in the general population, with any type of autoimmune disorder affecting 1 in 5 patients with POTS, and Hashimoto's thyroiditis being the most prevalent condition, affecting about 11% of our patients.
[36:26] Over the past few years, various antibodies have been identified in patients with POTS. These antibodies are critical to the structure and function of the sympathetic and parasympathetic nervous system, so it makes sense that these antibodies would be potential biomarkers in this disorder. Last year we made significant progress when an animal model was introduced by Dr. David Kim from the University of Oklahoma, who immunized rabbits with adrenergic receptor peptide and simulated POTS in rabbits. All of these findings undoubtedly have therapeutic implications with immunotherapy, which is already being used in certain selected patients with POTS quite effectively.

[37:16] Dr. Linda Bluestein: So are you referring to subcutaneous gamma globulins and also IVIG? And if so, can you tell us a little bit more about when it's appropriate to do that lab testing? I know as of at least fairly recently, insurance often did not cover the testing. Can you elaborate on that?

[37:38] Svetlana Blitshteyn, M.D.: Yes. The topic of immunotherapy in patients with POTS is growing. There is emerging evidence through large and small case series that immunotherapy in the form of IVIG and sometimes plasmapheresis has been very effective in our most disabled patients with POTS who are refractory to standard medications. You mentioned subcutaneous immunoglobulin — I am presenting a case series of patients with POTS who improved significantly with subcutaneous immunoglobulin at the upcoming American Autonomic Society meeting in November.
[38:35] What we know is that many of these patients have positive antibodies — whether it's adrenergic antibodies, which unfortunately are not available on a clinical basis in the United States, whether it's antinuclear antibodies, or whether it's antiphospholipid antibodies — there is always some kind of abnormal autoimmune marker. That's very important because it further suggests that in these patients who are refractory to standard therapy — and by standard therapy I don't mean just fluids and salt intake, I mean all of the available medications that we have for POTS — these patients have failed to improve, are essentially bedbound, and we should consider immunotherapy for them.
Large clinical trials and placebo-controlled studies are underway to determine whether IVIG is effective in patients with severe POTS. But until those studies become available — and as we know, these studies take a long time and a lot of resources to complete — we already have preliminary evidence from real-life cases and case series that outline significant improvement in these patients. In my case series, for example, two patients have gone from bedbound to working full-time after being given subcutaneous immunoglobulin over a span of 6 to 9 months. We need to push insurance companies to cover these effective therapies, because the cost of covering immunoglobulin — whether intravenous or subcutaneous — is going to be a lot less than the cost of covering medical care for a very disabled individual who is 20 or 30 years old with many years ahead of them.
[40:57] Similarly, a lot of my patients ask whether they can get IVIG because they have heard it can be very effective. We need to understand that IVIG is a blood product, that IVIG is very expensive, that insurance needs to cover it, and not everyone who walks through your door as a POTS patient will need IVIG. You need to go through standard therapy first. We have to emphasize to patients, clinicians, and insurance companies that it's only those who are refractory — who fail to improve with standard medications, who are very disabled, young people unable to participate in school or work — those are the patients who need to be tried on immunotherapy.

[41:49] Jennifer Milner: Excellent, thank you. Well, you have been listening to Bendy Bodies with the Hypermobility MD. Today we've been speaking with Dr. Svetlana Blitshteyn, board-certified neurologist and director of the Dysautonomia Clinic. Dr. Blitshteyn, thank you so much for taking the time to come on the Bendy Bodies Podcast and sharing your expertise with us today.

[42:11] Dr. Linda Bluestein: Thank you for joining us for this episode of Bendy Bodies with the Hypermobility MD, where we explore the intersection of health and hypermobility for dancers and other artistic athletes. Please leave us a review on your favorite podcast player. Remember to subscribe so you won't miss future episodes. Be sure to subscribe to the Bendy Bodies YouTube channel as well. Thank you for helping us spread the word about hypermobility and associated conditions. Visit our website www.bendybodies.org for more information.
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[42:57] The thoughts and opinions expressed on this podcast are solely those of the co-hosts and their guests. They do not necessarily represent the views and opinions of any organization, and do not constitute medical advice and should not be used in any legal capacity whatsoever. This podcast is intended for general education only and does not constitute medical advice. Your own individual situation may vary. Do not make any changes without first seeking your own individual care from your physician. We'll catch you next time on the Bendy Bodies Podcast.