21. Demystifying Dysautonomia with Svetlana Blitshteyn, M.D.

Episodes have been transcribed to improve the accessibility of this information. Our best attempts have been made to ensure accuracy,  however, if you discover a possible error please notify us at info@bendybodies.org

00:00:00 

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 today. We have the great pleasure of speaking with Dr. Svetlana  Blitsheyn, 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.Blitsheyn completed her neurology training at Mayo Clinic Graduate School of Medicine. Bleach stain  completed her neurology training at mile clinic graduate school of medicine. As 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.  

00:01:07 

Jennifer Milner 

Dr.Blitsheyn has been an invited speaker at national and international conferences, including at the World  Health Organization. Blue stain has been an invited speaker at national and international conferences, including  at the world health organization. Dr. Blitsheyn has been the principal investigator on a number of important  research studies concerning POTS and auto-immunity, 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 us news and world report  Medscape neurology today, new scientists and others. 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, US human health award, the American Academy of neurology student prize,  

00:02:13 

Jennifer Milner 

Dr. Blitsheyn. Hello and welcome to Bendy Bodies. Thank you so much for having you today. So let's start with  you.  

00:02:20 

Dr. Svetlana Blitsheyn 

The basics. Dysautonomia simply means abnormal autonomic nervous system, and it's synonymous was the  autonomic dysfunction of any kind. What exactly is dysautonomia simply means abnormal autonomic nervous  system, and it's synonymous was the autonomic dysfunction of any kind. It's not the diagnosis. It's a descriptive  term 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 and parasympathetic nervous system and each of these  systems can malfunction resulting in specific syndromes and disorders within this umbrella term of  dysautonomia.  

00:03:00 

Dr. Svetlana Blitsheyn 

Our specific diagnosis was their objective criteria that have been defined. None of these autonomic disorder  diagnosis are subjective and all have objective clinical criteria. The diagnosis relies on their assessment of blood  pressure and heart rate in the supply and standing position using a simple 10 minute stand test that can be  performed at the doctor's office or tilt table tests.  

00:03:29 

Jennifer Milner 

Excellent. Okay. What might make someone suspect that they have some dysautonomia?  

00:03:37 

Dr. Svetlana Blitsheyn 

So, clinical symptoms of dysautonomia are numerous and the most common ones are orthostatic intolerance.  That's the defining feature of an autonomic disorder. What that means is in difficulty standing and multiple  similar symptoms arising on 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. When you sit down or lie down, of  course, this is the hallmark, but there are other symptoms such as chronic dizziness, tations, tachycardia,  lightheadedness, headaches, brain fog, sleep problems, and many others.  

00:04:22 

Jennifer Milner 

Okay. Are there specific populations that might be at a higher risk for dysautonomia and as we are the bendy  bodies podcasts, why should bendy people, why should hypermobile people know about it?  

00:04:37 

Dr. Svetlana Blitsheyn 

Dysautonomia again as an umbrella term encompasses a wide variety of autonomic disorders, the most common  ones being postural orthostatic tachycardia syndrome. 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, how it relates to Bendy Bodies. Well, we know that one of the common co morbidities of parts is Ehlers-Danlos syndrome and 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.  

00:05:48 

Dr. Svetlana Blitsheyn 

You often will, if you see someone who has a hypermobility in pots that will lead you to talk about looking at,  an EDS. 

00:05:58 

Dr. Svetlana Blitsheyn 

Well, so in my clinic, of course I have many patients with EDS and pots. How I figured this out is from history.  When patients present to us, they're not going to volunteer the textbook information. Some will say that they  have been hypermobile, but mainly will present with complaints of joint pain, a body pain, easily dislocate the  joints, easy bruising and many other manifestations. 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 another hand, one may say that anyone presenting  with parts, should be screened for EDS. That's certainly a good practice in those of us who see a large number of  patients.  

00:06:56 

Dr. Linda Bluestein 

We know that in 2017, that criteria for hypermobile, the criteria for all the Ehlers Danlos syndromes changed  with the international consortium, right? So, in particular, the criteria changed for hypermobile EDS and then  they introduced the new classification, HSD or hypermobility spectrum disorders. In, 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?  

00:07:32 

Dr. Svetlana Blitsheyn 

Oh, that's a great question. I don't think there is answer to that. All of the studies that have been done are looking  at association between pots and Ehlers-Danlos syndrome, specifically the hypermobile type A, but the scores,  the hypermobility spectrum disorders are much more common and the hypermobility as a sign is very common  and by no means they constitute a disorder. This is something I have to always remind my neurology colleagues  who think, well, everyone, all of the young women who present with migraine or, dizziness, all of them are  quite hyper mobile. They're all gymnast or dancers or swimmers. Of course we have to make a distinction that  having a hypermobility as a sign absolutely does not constitute a hypermobile Ehlers-Danlos syndrome. There  are criteria that needs to be met there.  

00:08:34 

Jennifer Milner 

Yes, yes. I agree with that. So, when you're talking about, looking at pots with hypermobility, you said earlier  that you do, there are a couple of really easy tests for pots, the stand test and the table tests. What, is there more  of a diagnostic workup that is recommended for and how prevalent is that kind of a workup? Okay.  

00:09:01 

Dr. Svetlana Blitsheyn 

I'll describe how I do these evaluations, because I think it's important for clinicians to hear, what the process  may be. All us, all neurologic evaluations, I start to lose the physical exam after I take a thorough history and  listen to the patients and all of the complaints and the physical exam includes for the study blood pressure and  heart rate measurement. You lay down the patient, allowing them to rest for a few minutes, and then you  measure their blood pressure and heart rate. After that you stand them up. She had their blood pressure and heart  rate again increments of two to three minutes for a 10 minute stand test. This easy in-office test is very  important because it can give you clues whether at disorder of the orthostatic intolerance is present. Now in the  time of social distancing, I asked the patient to perform the same test in the comfort of their own home.  

00:10:01 

Dr. Svetlana Blitsheyn 

This gives me a good approximation of what their vital signs are doing while they're supine and standing  following that. It's important to do a full neurologic exam with a special attention to the sensory exam, because  at least 50% of patients with pots have more bit 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. That usually includes EKG, basic blood work and thyroid function tests. Personally, I prefer to go  beyond that in order to be thorough and not to miss any other diagnosis. Typically my patients have seen at least  a dozen of other physicians and the basic workup is complete by the time they see me in terms of neurologic  evaluation, if the patient has headache, which, the most common comorbidity with POTS, and also Ehlers Danlos, I obtain MRI of the brain without contrast one time to rule out structural abnormalities, like your information, and then patients with POTS, MRI of the brain is usually entirely normal, which can be very  reassuring.  

00:11:25 

Dr. Svetlana Blitsheyn 

On that note, if there is a 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 with obstructive sleep apnea,  central sleep apnea, narcolepsy, and other sleep disorders may be present. In many cases, I refer the patient for  it's still table tests for diagnostic confirmation, because in some cases where access to a tilt table test is difficult.  In some cases where access to wait till table test is difficult. In some cases where access to wait till table test is  difficult. I rely on the 10 minutes stand test, which we call a poor people's tilt table tests. Most of the time when  there is access to tilt table tests, I certainly order that.  

00:12:20 

Jennifer Milner 

That's great. Thank you. You mentioned a small fiber neuropathy. I know there are other issues that Chiari  malformation, so I'm wondering, first of all, purely out of curiosity on my side, 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?  

00:12:40 

Dr. Svetlana Blitsheyn 

Yes. In patients with Ehlers-Danlos I certainly see more Chiari malformation. If you ask me that question, in  terms of my pots patients, I would have to say, no.  

00:12:54 

Dr. Linda Bluestein 

Just explain for the listeners that are not familiar. Could you explain what both Chiari malformation and small  fiber neuropathy what they are and why they are relevant in this conversation?  

00:13:04 

Dr. Svetlana Blitsheyn 

Chiari malformation is usually like 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. And,  Chiari malformation, you have cerebellum specifically the cerebellar tonsils protruding down through the  foramen Magnum, and if it's, protruding more than by five millimeters down, that can be symptomatic. The  symptom set may include those that are related to the obstruction of the CSF flow of this cerebral flow. Also  those that can potentially, compress brainstem and cerebellum itself naturally when you have a connective tissue  disorder with weakness and abnormalities in collagen, ligaments, and the even muscles may become loose and,  no longer normal in structure or function. That weakness in a ligament tissue may resolve in protrusion of  brainstem cerebellum. Similarly, elsewhere in the body, you may have similar situations where we refer that to  hernia where organs or tissues protrude through a weak, muscular wall.  

00:14:50 

Dr. Svetlana Blitsheyn 

Small fiber neuropathy refers to abnormal structure of small nerve fibers that are everywhere in the body and  autonomic neuropathy. Isn't the same as small fiber neuropathy. There needs to be a distinction made, a thin  unmyelinated, alpha and Delta fibers that, underlies small fibers, may be damaged and they may be damaged by  toxins, medications, antibodies, or alcohol, diabetes, and many other conditions, which results in, neuropathic  pain and objective findings that we see on neurologic exam, which traditionally includes a loss of, temperature,  sensation and pain sensation. This distinction made, a thin unmyelinated, alpha and Delta fibers that, underlies  small fibers, may be damaged and they may be damaged by toxins, medications, antibodies, or alcohol, diabetes,  and many other conditions, which results in, neuropathic pain and objective findings that we see on neurologic  exam, which traditionally includes a loss of, temperature, sensation and pain sensation. That's a very important  part of neurologic exam. 

00:15:51 

Dr. Svetlana Blitsheyn 

That's how we can identify our patients with pots and Ehlers Danlos who may have small fiber neuropathy. At  least 50% of patients with spots have small fiber myopathy objectively confirmed, and at least 70% of patients  was hypermobile Ehlers-Danlos syndrome. Also have small fiber neuropathy is very important for clinicians to  

identify that and the way to diagnose it is of course, we ask questions on history on the questions will be  surrounding neuropathic pain. Not always the patients will have burning pain or numbness tingling. Sometimes  they will have itching. Sometimes they will describe a full body paint, a bone kind of pain. Sometimes they'll  describe this as having rubber bands on their feet. Sometimes they will describe the pain as, a sensation of heat  or having heavy legs. When we have this history and we have findings on our exam of sensory dysfunction, we  must obtain very important.  

00:17:02 

Dr. Svetlana Blitsheyn 

Two tests. One is a test of 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. Very important point is that when we in medical world suspect that our patient has neuropathy, we  must order two tests. One is EMG test of large fibers, and another test is a skin biopsy or test the small Hybris.  There is also, a full autonomic function test that is only in certain areas of the country. That's, unfortunately isn't  available to all of us everywhere, but there is an important test there called acute quantitative. The motor X and  reflux tests that can measure sweat output and determine whether a patient has a small fiber neuropathy, with  that method.  

00:18:14 

Dr. Linda Bluestein 

Very good. And, and we wanted to have more of an explanation in terms of, syncope. You had mentioned  earlier, like neurocardiogenic syncope, and again, some of our listeners, they heard the word syncope they'll  know what that means, others won't. Could you just explain more about that?  

00:18:32 

Dr. Svetlana Blitsheyn 

Sure. Neurocardiogenic syncope is a condition that usually is 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 the lifetime, at least 25% of all patients may have a  syncopal event, especially when they're younger and that the cons for a great majority of ER presentations. This  is what we call a simple syncope, single, simple, faint, single, simple syncope, 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. This becomes only a disorder when syncope is repetitive. Of course, to a significant frequency, it  obviously becomes disabling when that the car's very frequently and then pairs your functional status. And  clearly it becomes extremely disabling. When you have syncope, every time you stand up, it does come, we  affect young people.  

00:19:52 

Dr. Svetlana Blitsheyn 

It was the onset of puberty, which is the most common age of onset, not only for new or cause of genic syncope,  also commonly it's a common age of onset for postural orthostatic tachycardia syndrome during a period of  growth spurt or, during, the onset of menstruation. One can have these, 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 function  and when it's repetitive and affect your functional status, then it becomes a medical condition that needs medical  attention, briefly put syncope just means an abrupt loss of a suitable blood flow. When that happens, obviously  you fall down and that's a body's natural way in restoring cerebral perfusion and blood flow back to your brain.  

00:21:04 

Dr. Linda Bluestein 

Syncope is basically when you get a syncope or presyncope also, right, so people can have syncope or complete  loss of consciousness, or they can feel like they're going to pass out. Both of those would happen in like, for  example, pots, is that true?  

00:21:19

Dr. Svetlana Blitsheyn 

Okay. So let's make some distinctions here. Apart is defined. Pots as a disorder has three clinical criteria. One is  you need to meet a criteria of an increase in heart rate by at least 30 beats per minute, within 10 minutes, then  tests or a tilt table test, and at least 40 beats per minute in teenagers, or those who are under the age of 19 that's  number one criteria. Number one criteria. Number two is blood pressure remained stable during that time.  Number three is the chronicity of symptoms where symptoms must be present. Symptoms of orthostatic  intolerance must be present for at least six months. That's the definition of parts of 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. And that's the criteria for neurocardiogenic syncope. We also have a  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 three minutes of a tilt table test.  

00:22:51 

Dr. Svetlana Blitsheyn 

If the heart rate does not rise in compensation to a decreasing blood pressure, we call that neurogenic orthostatic  hypotension. Now that we know these three distinct patterns, now you asked a very important question in parts,  do these patients pass out? Do they come close? What's going on there? So of course, if we go by defined  clinical criteria, once you meet criteria for parts by exclusion, you can not meet criteria for new Yorkers, genic,  syncope. However, if you go to the clinical world of reality, that some patients will faint as well. In addition to  having pots, some patients will faint 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 what we call presyncope. We  think it's because the physiology still compensating. When you have a kind of a slower to decrease in cerebral  perfusion, perhaps you have activation of autonomic reflexes that they're still preserving, based on this increase  in heart rate that are still preserving some perfusion to the brain and therefore you do not pass out.  

00:24:20 

Dr. Svetlana Blitsheyn 

It's a very important distinction to make. Now, some people will have abnormal tilt table test showing your  polygenic syncope. As I mentioned before, transition, traditionally your current agentic syncope is viewed as an  episodic disorder, meaning that in between the episodes of syncope, the patients should be asymptomatic again,  when you, practice in reality, there are also patients who have neurocardiogenic syncope confirmed, they pass  out, and then between the episodes of syncope, they will have disabling symptoms such as fatigue,  lightheadedness, dizziness, headaches, and all the rest of the symptoms of dysautonomia.  

00:25:06 

Jennifer Milner 

Yes. I wanted to follow up with you on some of the things that you mentioned with the fatigue and the  headaches. Can you go more into what are some of the common symptoms, and co-morbidities, that might go  along with people who have been diagnosed with pots.  

00:25:22 

Dr. Svetlana Blitsheyn 

We divide symptoms of packs into two categories. One is orthostatic. The orthostatic symptoms such as  dizziness, palpitations, lightheadedness weakness, and overall feeling faint. The second category is non  orthostatic symptoms. These includes general symptoms such as fatigue, chronic dizziness, headaches, brain  fog, which is difficulty concentrating and other symptoms of cognitive dysfunction, sleep disturbance, and the,  sometimes the alterations in mood. These are the non orthostatic symptoms of the set. Dysautonomia  comorbidities is a very common, as they always say, paths, rarely exists alone. The Nomia comorbidities is a  very common, as they always say, paths, rarely exists alone. At least 80% of patients with pots have significant  co-morbidity at least one the most common comorbidity. As I mentioned before, it's small fiber neuropathy  occurring in at least 50% of patients with pots. Another very common comorbidity is headache, across, in at  least 40% of patients with a third common comorbidity is Ehlers-Danlos occurring in at least 25% of patients  with pots. 

00:26:51 

Dr. Svetlana Blitsheyn 

And then we have other comorbidities. One of them is a mast cell activation syndrome, and now there is irritable  bowel syndrome. There are Comorbid or immune conditions affecting at least 25% of all patients with spots  with the most common, what immune condition being Hashimoto's thyroiditis.  

00:27:15 

Jennifer Milner 

Excellent. Thank you.  

00:27:17 

Dr. Linda Bluestein 

You wrote a paper on, B-1 deficiency in pots. Would you be able to elaborate on that?  

00:27:25 

Dr. Svetlana Blitsheyn 

Okay. Vitamins deficiency is quite common in our patients was the most common being iron deficiency without  anemia. Levels of vitamin B12 also appear to be lower in our patients compared to the age matched controls in  terms of vitamin D one deficiency. I am aware only of my study and in my cohort of 65 patients, the prevalence  of vitamin B1 deficiency was 4%, unfortunately only one patient out of that. KC serious, recovered with vitamin  B one supplementation. Regardless of that fact, I personally always check all of my patients with spots for  common vitamin deficiencies, including B12 B-1, D Iron, B-6 and others.  

00:28:32 

Jennifer Milner 

Excellent. Thank you. 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 and especially if not diagnosed and  treated and how could someone avoid getting to that point?  

00:28:55 

Dr. Svetlana Blitsheyn 

It's a very good question from multiple studies. We know that pots can be quite disabling as congestive heart  failure and at least 25% of our patients are unable to work or attend school. And, prognosis, has been a little  tricky. A question, about the decade ago, there was a notion among our colleagues that you can outgrow pots.  The statement came from one earlier study that suggested that 80% of patients no longer experienced past five  years after they were diagnosed; many of us who specialists in pots simply were not seeing these high numbers  in real life. 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, but only 19% fully recover. That was a mean  followup of five years. So, why, while we would love to tell patients that pots has a good chance of recovery.  

00:30:13 

Dr. Svetlana Blitsheyn 

I think reality is that parts 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. I think a realistic approach to long term prognosis is a lot better than painting a rosy picture and telling young patients that may outgrow pots in  their twenties only to get their hopes that they're at one that doesn't happen. Now, having said that seams appear  to have a better prognosis than adults with pots. They certainly have patients who were very sick during their  teen years and unable to attend school, but then improve enough to be able to attend college and do all their age  appropriate things. So, in summary, I tell my patients that prognosis is hopeful, but expectations for a full  recovery need to be tapered and lifestyle adjustments, as well as some form of medical management should be  expected. 

00:31:26 

Dr. Linda Bluestein 

And, and I wanted to follow up with actually this what you were just saying in terms of expectations and this  age group. I'm thinking that this ties in with the B-1 question that, this is a group of kids that would be doing,  like I said, age appropriate things, which of course also includes, drinking alcohol and many instances as they  

turn 21. And, but with the potential for vitamin B deficiencies, and of course the potential effects of alcohol on  other, hemodynamic variables, I guess, do you have any advice for, listeners about that?  

00:32:02 

Dr. Svetlana Blitsheyn 

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  make call lead to mass cell activation. Alcohol causes sleep disturbance, alcohol can cause balance difficulties.  Many of our patients, do not drink. Sometimes adults would be able tolerate a glass of wine and there, but a  majority of our patients do not consume alcohol. Now it seems we'll be teens and college students will be  college students. They do advise to stay away from alcohol. If you have to socialize, and if you have to go to a  party, limit your alcohol intake to no more than one drink per night, and that's very little and the grand scheme  of things about, it's not worth causing an exacerbation of your pots and other comorbid conditions.  

00:33:12 

Dr. Svetlana Blitsheyn 

It's not worth ending up missing the next day of school because the day before the night before you were  drinking alcohol. Being very cognizant of negative impact of alcohol on our patients, yet understanding that  saying don't ever drink alcohol, or you shouldn't drink alcohol at all isn't also realistic. Isn't also realistic. Limit  your alcohol intake, if you must no more than one drink. Of course, if you have to drink, don't drive, ask for a  ride.  

00:33:47 

Jennifer Milner 

Excellent. Thank you. I'm wondering in this whole big picture, how does auto-immunity fit into all of this?  

00:33:56 

Dr. Svetlana Blitsheyn 

There has been significant research interests in the past few years to determine whether pots is an auto-immune  disorder. While we haven't conclusively answered this question thoroughly, yet it does appear that pots may  have a strong autoimmune basis. Years ago, I received a grant to study auto-immune markers in all things in  disorders. In patients with pots, I noticed that a significant number of my pots patients had positive markers of  autoimmunity and comorbid autoimmune disorders. We, when we applied statistical analysis, we did find that  past patients had a higher prevalence of nuclear antibodies, antiphospholipid antibodies and other markers than  the general population. The prevalence of most defined autoimmune conditions was higher in my cohort of a  hundred patients with pots then in general population, with any type of autoimmune disorders affecting one in  five patients with spots and Hashimoto's thyroiditis being the most prevalent condition affecting about 11% of  our patients.  

00:35:13 

Dr. Svetlana Blitsheyn 

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. It makes sense that  these antibodies would be potential biomarkers in this disorder. Now, last year we have made significant the one  by Dr. David Kim from the university of Oklahoma, who immunized rabbits with adrenergic receptor peptide  and simulate the pots in rabbits. All of these findings undoubtedly have therapeutic implications was  immunotherapy, which is already being used in certain selected patients with spots quite effectively.  

00:36:03 

Dr. Linda Bluestein 

Are you referring to subcutaneous, gamma globulins and also IVIG. Are you referring to subcutaneous, gamma  globulins and also IVIG. Can you elaborate on that? 

Page 8 of 10 

Episodes have been transcribed to improve accessibility of this information. Our best attempts have been made to ensure accuracy,  however, if you discover a possible error please notify us as soon as possible. 

Transcript for Bendy Bodies Podcast, Episode 21: Demystifying Dysautonomia with Dr. Svetlana Blitsheyn 

00:36:25 

Dr. Svetlana Blitsheyn 

Yes. The, topic of immunotherapy in patients with spots is growing, there is a, there is emerging evidence  through large case small case studies that immunotherapy in the form of IVIG and sometimes plasma have been  very effective in our most disabled patients with pots refractory to standard medications. Interestingly, you  mentioned subcutaneous immunoglobulin, on presenting a case series of patients with spots who improve  significantly with subcutaneous immunoglobulin. I am presenting this research at the upcoming American  Autonomic Society meeting in November. What we know is that many of these patients have positive  antibodies, whether it's going to be adrenergic antibodies that as unfortunately are not available on clinical basis  to obtain in the United States, whether it's going to be into nuclear antibodies, but the antibodies there is always  some kind of abnormal immune marker. That's very important because this further suggests that in these patients  refractory to standard therapy and by standard therapy, I don't mean, fluids and salt intake.  

00:38:04 

Dr. Svetlana Blitsheyn 

I mean, all of the available medications that we have for pots, these patients have failed, have not been proved.  They're essentially bed bound, that we should consider immunotherapy in these patients. Now, large studies,  clinical trials, placebo controlled studies are underway to determine whether IVIG is effective in patients with  pots, but then see those studies become available. 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 serious that outlines  significant improvement in these patients. For example, in my case, two patients have gone from bed bound to  working full-time, when they were given subcutaneous immunoglobulin in the span of six to nine months. You  need to be very cognizant and we need to push the insurance companies to cover these, effective therapies,  because the cost of coverage of immunoglobulin, whether in the IVA in the intravenous or subcutaneous cost is  going to be a lot less than the cost of covering a medical care for a very disabled individual who is 20, 30 years  old, and have many years ahead of them.  

00:39:41 

Dr. Svetlana Blitsheyn 

That's a very important point that we need to emphasize. Similarly, a lot of my patients ask me, well, can I get  IVG because they have heard that IVIG 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. Not everyone who walks through your  door as a pots patient will need to have IVIG. You need to go through the standard therapy first. We have to  emphasize to the patients, clinicians, and insurance companies, that it's only those who are refractory, who failed  to improve with standard medications, who are very disabled, young people who are unable to participate in  school or work. Those are the patients that need to be tried on the immunotherapy.  

00:40:36 

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 Blitsheyn, certified neurologist and director of the dysautonomia clinic. Dr.  Svetlana Blitsheyn, thank you so much for taking the time to come on the bendy bodies podcast and sharing  your expertise with us today.  

00:40:58 

Dr. Linda Bluestein 

Thank you for joining us for this episode of bendy bodies with 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, for a limited time, you  could win autographed copy of the popular textbook disjointed navigating the diagnosis and management of  hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders just by sharing what you love  about the bendy bodies podcast on Instagram, tag us at bendy underscore bodies and on Facebook at Bendy  bodies podcast. The thoughts and opinions expressed on this podcast are solely of the co-hosts and their guests.  They do not necessarily represent the views and opinions of any organization. 

00:42:05 

Dr. Linda Bluestein 

The thoughts and opinions 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.