POTS (a remarkable condition) - Talk by Dr Sanjay Gupta

we're very pleased to have dr gupta join us today this was a suggestion made by one of our members postural orthostatic tachycardia syndrome is something that is quite prevalent amongst people with me cfs also fibromyalgia i'm sure dr gupta will talk to us a little bit about incidents um but it's it's one of those things that's actually it is quite difficult to get some clear advice and guidance on and to get some good understanding it's not something that gps are very well versed in and many people have had to do a huge amount of their own research and to try and make sense of what's going on so i think today's audience is likely to be a lot of people who have done a good amount of google research and will be very pleased to hear things directly from a specialist in the field so dr gupta is a consultant cardiologist and heart specialist works at york district hospital and i think i'm going to hand over to dr gupta then too you've got some slides to share with us and please keep those questions coming in as we go through the talk so can i hand over to you dr gupta a very very warm welcome from all of us we're delighted to have you thank you so much thank you for having me i really appreciate the fact that people have taken their time after lunch to join me um the good news here is that you're not going to lose either you'll find what i have to say interesting or i'll bore you to sleep and i in my own experience everyone can do with a bit more sleep so so you can't lose the first thing i'd like to say is i um i i feel privileged to come and talk to you um and the reason i do so is because i think as a medical profession as a whole we owe people with chronic fatigue syndrome and parts and fibromyalgia et cetera and apology uh for too long have we chosen to neglect patients and instead concentrate on what we know and what we've read in books and my background is that i was trained as a general cardiologist i wasn't someone who has any kind of level of expertise in pots or chronic fatigue syndrome and this is a very honest story um my first contact with any patient with pots was about seven or eight years ago and i recall going on to the ward and there's that you know you the junior doctor says yeah we've got this lady who complains of um her heart rate shooting up she's got this funny thing um called pots i don't know what that is and i was like she's got cfs she's a bit mad you know just that that was what and that is what i chose to believe at that time uh the problem was that this patient kept coming back uh into hospital and whilst the initial kind of my mindset at that time my indoctrinated mindset was well you know this is just an inconvenience this patient i don't have anything to offer this patient increasingly i started appreciating this lady for who she was she was a very sensible person she had ambitions she had worked hard and suddenly her life had been turned upside down and she had this multitude of symptoms and whilst they did nicely fit in any kind of thing i had read the reality was that this was a really genuine kind person who had ambition who had a supporting family but she was going through this horrendous thing and somewhere no one was interested because everyone was sort of focusing in on their thing you know when i go okay what's what's the cardiac thing i'm not really my my mindset wasn't who is this person who how how do i help this person my mindset was which of her do i understand that i can do something with and if it didn't fit nicely in a box then [Music] somehow rather than choose to think of her as a he think of me as a student needing to learn a bit more i would think that there was a problem with her brain or something like that um as i got to know her i started becoming really interested and we started working together and i started saying well you know i started really appreciating her for who she was and slowly and gradually i started meeting other people who had similar symptoms and they were all saying the same things and on the basis of that i decided that i was going to try and educate myself over this condition i was going to spend more time listening to patients rather than just listening to my colleagues or listening to books and it became very apparent that there was something very real here after that i did a series of videos online which seemed to resonate with people and now i have about 400 patients that i look after with pots many of whom also have chronic fatigue syndrome many of them have fibromyalgia and i think they i think dealing with these patients has made me a better doctor it's made me a kinder person it made me someone who has started understanding how important it is that we listen to our patients and no matter whether things fit nicely into boxes our aim as doctors should be to deal with the patient in front of us and it doesn't really matter what we're there to do is to try and help that patient and i never thought like that before and for a lot of patients it became very apparent that all they needed is just to feel supported all they needed was someone there who said look you know i believe you i you i don't think this is um all in your head i i believe you and i'm here to support you and i'm here to try for you and i'm here to come out of my comfort zone for you and as long as you did that they're the most satisfying patients to work with far more satisfying than giving someone a statin or giving someone some aspirin here we were actually being able to communicate build a rapport and you see this patient develop confidence and start feeling empowered and when they do and they send you a message saying look i've been able to stand up for the first time for my son's birthday in eight years i've been able to host a party today that is far more satisfying than any other part of cardiology that i have done so you know it's a privilege to talk to you because i have great admiration for the resilience uh that those patients who have these kind invisible and poorly acknowledged conditions because i really think it it must be very hard and to keep going with all the stresses that life throws us and then just feel unwell and to live with us is incredibly uh tough and um i admire this resilience i'm going to talk to you about pots and what i know about pots again as i say i'm not i'm no expert who's done a phd in parts my experience comes from practical experience dealing with lots of patients and many of whom are actually getting better so i'll share my slides now and then we can go through things um let's have a look here we go okay perfect so i've turned this talk uh pots a remarkable condition and the reason i call it a remarkable condition is because it is remarkable in the sense that it generally tends to affect otherwise young previously healthy and often high achieving people people who were remarkable in some way before they became unwell it is remarkable in the sense that it seems to affect so many different systems in the body it is remarkable in how debilitating it is for the patient and yet how invisible it is for the outsider to the outsider it is remarkable in how prevalent it is in our society and yet how little recognition it gets from the medical profession it is remarkable in that despite all the advances in technology we still don't quite know why it happens and how we go about curing it um so let me just sorry play this the first description of what we now recognize as pots was made by the guy on the left who is uh decosta he was a surgeon in philadelphia and in 1871 he observed that the in patients who were uh being in in soldiers who were being enlisted in the american civil war what would happen is that um these soldiers there were civilians really who had been enlisted in the war and they were sent on long hours of marching so they would be asked to walk up to 20 miles in a day at double quick pace with poor food poor water supplies and in bad weather and what he started noticing is that a group of these soldiers would then develop a viral infection and they would become exhausted and they would start falling out of line and after that they would be given some time off they would recover from the infection and when they returned to marching they would never be able to quite achieve the levels that they were able to before they became sick these patients would complain of fatigue breathlessness tiredness after mild exertion and he also noted that when you measured their heart rates their heart rates would tend to go up greatly in response to posture so if they were lying down their heart rates would be better if they stood up their heart rates would be much worse and the guy the the guy on the right is a typical such soldier who's been described so that is probably the first description of what we now recognize to be pots it was called the irritable heart of the soldier or de costas syndrome now the feature of pots pot stands for postural orthostatic tachycardia syndrome and by far the typical symptom is this idea of orthostatic intolerance when the patient is upright they don't like it they don't like being upright for a prolonged period of time that can be sitting or standing and it is usually far worse and far more troublesome if the patient is still so a lot of patients will say i'm not too bad if i'm walking around but if i stand still for any period of time i really struggle and they get a bunch of symptoms but by far and away the the symptom that brought it to light and made cardiologists interested is the symptom of palpitation these people complain of their heart rate racing and often they complain of lightheadedness they may also complain of chest discomfort and breathlessness but by far and away the thing that brought this these this cohort of patience to cardiology's attention was the fact that they complained of palpitations and lightheadedness when they were upstanding when you then go in and ask these patients they have a ton of other symptoms as well and these symptoms are not necessarily always related to standing they occur at all times and in particular all patients i have met with pots will complain of extreme fatigue they will complain of mental clouding brain fog difficulty carrying out cognitive tasks um it's like a cloud cloud over their heads they complain of headaches they complain of tremulousness they complain of blurring of the vision they may complain of auditory disturbance uh there by virtually every single patient with pots will complain of a disturbance in their sleep pattern so they never wake up feeling refreshed and they often have gastric symptoms and often carry a diagnosis of irritable bowel syndrome i think there are a bunch of patients who have probably had all these symptoms for a long time but because these symptoms tend to be a little bit non-specific you know i don't sleep well i'm tired doctors didn't really take much interest in those symptoms but the minute the patient said look i get heart palpitations the doctor said okay well why don't we send you to a cardiologist and the cardiologist typically would disregard all the other symptoms and say okay you've got heart palpitations when do you get the heart palpitations when i stand up let's let's see what happens so let's stand them up and see what happens and lo and behold they noticed that when these people stood up their heart rates on most occasions would shoot up and so the cardiologist would then say yes your heart rate does go up but it's going up regularly let's give it a name postural orthostatic tachycardia syndrome you stand up your heart rate goes up and you don't like it and that's all what that's all pots means uh so potts is not a once we think of pots as a diagnosis it is actually just a fancy term for the fact that you don't like being stood up for a prolonged period of time and your heart rate goes up the important thing to understand is there is no single patient that i have with pots who will not complain of all these other non-cardiac symptoms and the definition of pots the current definition of pots does not take into account all these other things and that is really important so at the moment people say pots is an increase in the heart rate of more than 30 beats per minute and more than 40 beats per minute if you're a child within 10 minutes of upstanding from a supine position and they say you have to have this heart rate to extend for more than 30 seconds and ideally for the duration of the time and they also say that at that point your heart your blood pressure shouldn't fall so you maintain your blood pressure but the heart rate shoots up and it stays there and it's a frequent and chronic observation that's fine i appreciate that but a much better term because the term that will take into account everything else that that patient is enduring is a dysautonomia and this is the difference between pots and the dysautonomia a dysautonomia simply means a disturbance in autonomic nervous system activity so when we think about this thing called the autonomic nervous system we're thinking of this balance between our flight and fight systems and our rest and digest systems and the disturbance in our flight and fight responses versus our rest in digest responses would be termed dysautonomia if i go back to the symptoms if you look about look at these these are all flight or fight to rest and digest symptoms you know if you think that these people are tired so they're not rested they do not sleep well they're not rested they have gut symptoms they're not digesting they're getting heart rates high flight they're getting temperature disturbance flight so they're getting breathless again flight of right so so that is a much better term uh of thinking of something as dysautonomia rather than pots because dysautonomia covers everything whereas pots just refers to this rise in heart rate and the reason it is so important is because if you just rely on the definition of parts and for some reason the patient doesn't get that heart rate then they tell they don't have parts and you're you can go away right but if you think of this and if you ask the patient about these other symptoms all these other symptoms then you've made your diagnosis without needing any additional tests if there is no other explanation and the patient says i don't like being stood up for a prolonged period of time and actually i never wake up feeling refreshed and i never feel um and i have gut issues and i get brain fog etc and there's no alternative explanation and they've been struggling for a bunch of months or years then this is the diagnosis and this is all i need to start saying that's what we have here the problem is the purists now and mistakenly so say well if you don't have this you don't have parts this is wrong the patient comes first definitions come after that and when you start asking patients about this and when you start looking and looking for it you start finding that this is an epidemic i am at this point in time getting four patients referred to me every day with these symptoms when i and and when i send them for tilt table testing or looking for tests some of them come back positive some of them come back negative but i treat them all based on my clinical assessment and when i do the majority start responding so at this point in time based around this current definition of paths people would say that up to three million people in the states have pots and generally the ages are between 15 and 50 and you see more women but of course you do see it in men as well and why does it happen and we're not quite clear but i have certainly observed in my patients that by far and away most of them have some kind of genetic vulnerability when i speak to them you will often find during conversation that their their mum or their aunt will have had similar symptoms maybe it was never formally diagnosed as that but they've had similar symptoms so there does appear to be a genetic vulnerability and by far and away virtually i would say the majority of my patients will admit to having um joint hypermobility syndrome or ayla's danlos syndrome joint hypermobility variant so that is the genetic vulnerability and the idea behind this particular condition joint type of mobility is that in a simplistic sense and my understanding of this is only simplistic is that they have much laxer blood vessels so their blood vessels tend to be more lacks and in some way that contributes to how they respond to autostasis they have there's a genetic vulnerability then something happens in their lives and that could be pregnancy uh glandular fever uh is very common trauma some kind of viral illness some kind of major surgery some kind of prolonged period of being bed bound even extreme stress something like that interestingly some people have mentioned the hpv vaccine i know that no one likes to talk about vaccines but this is what people have said to me um and then after that it is almost like they become ill and they never quite recover a bit like what decosta described with the soldiers they never quite recover and then they have they have flare-ups there are triggers which will cause flare up so genetic vulnerability something happens an index event happens the genie is let out and then some things make the genie more prevalent in the light in their lives and others sort of calm things down but the common triggers are dehydration heat alcohol exercise any form of inter-current illness and for the for all women things are worse around their periods hormonal changes have a big role in how much more symptomatic they get with their symptoms now i just wanted to i had a bottle here um this is the physiology of standing up because one of the things that we have to ask ourselves is what is it about this orthostasis why are they orthostatically intolerant and for that we have to understand what the physiology of standing up is this is what i describe to my patients again simplistic it may not necessarily meet every kind of physiologist's viewpoint but this is the simple way so in general if we think of a bottle half a half empty bottle if we think of this as our brain here and then this is the body and the water in the bottle is the blood when we're lying down uh our brains are well perfused with uh with our blood when we stand up gravity comes into the equation and therefore uh the blood gets sucked down and ordinarily because our brains would go without blood we would all collapse again however that doesn't happen because a couple of reflexes come into motion the first is this surge of blood into our legs is detected by our leg vessels and our leg vessels will constrict to try and keep the blood up and the second thing that happens is we produce adrenaline to try and increase our heart rate to keep the blood going and then we reach a steady state and we stay like that somewhere in those two actions is there is a fault in patients with pots who are not able to maintain an orthostatic position either they don't squeeze as well or for some reason their adrenaline levels the adrenaline levels that are produced are so high that their heart rate goes up excessively high and because their heart rate is going up excessively high the heart is working harder but because it's not getting much time to fill with blood you actually get less blood going around and that may be the mechanism as to why people don't like being stood up either they're not compressing either they're not their leg vessels are not stretching that is why ailes down loss and joint type of mobility may have a role to play because they have lax vessels and so maybe their leg vessels don't compress as well and also the heart rate may be going up excessively high and therefore you're not getting as effective uh a surge of blood to the brain and that is why they probably get the symptoms the reason i say this is because once we understand this then we understand why some of the treatments that are prescribed work or help but i'll talk to you about that in a second increasingly as people have started becoming aware of pots they're trying to work out what the mechanisms are and there is no one mechanism the neurologists if you go to the neurologist because you see the problem is pots patients some go to neurologists some go to a cardiologist some go to someone else so the patients who ended up with the neurologists uh sorry not the the patients who ended up with the cardiologists initially said well maybe these people just have less water in the bottle maybe that's the problem and indeed when you look patients with pots do have reduced blood volume and even in that volume the cells that they have are smaller so why is that why do they have less volume for that we have to understand what allows what regulates volume in our bodies and this is something called the renin-angiotensin system these are a bunch of hormones secreted by the kidneys which help us retain water and in patients with hypovolemic pots these patients who are supposedly having less blood the idea is that when you look at these hormones for some reason they don't produce the same magnitude of hormone in response to being dehydrated compared to other patients so their renin and angiotensin levels seem to be abnormally low and in addition because the blood cells themselves are smaller they're carrying less oxygen around so there is some credence to this hypothesis that maybe in some people they just have less water in the bottle to start off with then the neurologists came up with a theory about neuropathic bots and again so they were interested in looking and saying well maybe the there is enough water in the bottle but maybe you cannot squeeze adequately that's why you're getting the problem so they then started saying well let's study the nerves that control the squeezing effect in the legs and they found that when they started doing biopsy these people did have less sympathetic nerves in their legs and maybe that was the thing that they they were unable to respond to this this increase in blood volume in the legs by squeezing adequately and therefore less blood was being pumped to the heart and that became the hypothesis behind neuropathic parts so some people are told they have neuropathic pots some people are told they have hypovolemic pots and then some people have this thing called central hybrid adrenergic pots where they say okay what is happening here is perhaps in your case you're just producing too much adrenaline so remember um you know when we when we look at the way we are we're always in a nice kind of uh equilibrium uh between our flight and fight and our rest and digest systems if you come you know let's say you're lying there and i come and say boo i would activate your sympathetic system i'd activate your flight or fight response you would produce adrenaline and after a little while you'll get all the effects of that adrenaline and then the adrenaline will die down and everything will go down what we do know is that in uh dysautonomia the pivot has moved this way so a small trigger causes a huge surge now the question is why are we getting this huge surge in adrenaline is it that in some way we're producing too much adrenaline or is it that we're responding in a hyper exaggerated fashion to the adrenaline we are producing so we're producing normal levels of adrenaline and we're just responding in an exaggerated fashion or are we truly producing more adrenaline when we have looked when doctor when researchers have looked they have found that noradrenaline levels do tend to be higher both lying down and standing in these patients and actually one of the manifestations in these patients is because they have the higher adrenaline their blood pressure shoots up as well when they stand up for some forms of pots the blood pressure doesn't go up but there is a subgroup of patients with protein whom the blood pressure shoots up the heart rate shoots up and they actually will then say i feel very tremulous i'm very anxious i get very cold and those people are built as having hyper adrenergic pots some people have been found to have a problem with the receptor that clears the adrenaline so you produce the adrenaline maybe not as much adrenaline as you you know maybe the same amount of adrenaline but you cannot clear it because there's some kind of deficiency in the receptors that clear the adrenaline so the adrenaline stays around for a lot longer then there is another group of patients called who have something called muscle activation and i don't know whether many people know about this i i certainly didn't and i'm sure that those people those of the viewers today who are educated in it may have a much more sophisticated understanding of this but to my mind the way i understand this master activation mast cells are cells which for example if i take my pen here and i draw it against my skin what you will find is that my skin will become red and it will become indurated it will become swollen because of the insult and that reaction is carried out by mast cells and the idea is that what happens is that the mast cells through histamine make our blood vessels around them more permeable so everything leaks out to try and fight whatever the insulting agent so that is the idea so that's what mast cells do there is a group of patients who have something called mast cell activation syndrome in which okay in where what will happen is some kind of insult causes a ton of muscle release and what that huge surge in mast cells does is that it makes all our blood vessels leaky so for that period of time what happens is blood water leaks out of the bottle and so these patients get these symptoms where their heart rate then shoots up etc the blood pressure goes the heart rate shoots up they get all the same symptoms uh in these patients a diagnosis is made by measuring these histamine metabolites in in the urine and these patients often get a flushing episode so they they the hallmark of muscle activation is that they often flush quite a lot and these patients seem to respond with antihistamines so these are the different subgroups of pots another group of pots uh patients are patients who have physical deconditioning so that can also physical deconditioning itself can also cause a pots like syndrome the question is is it just the physical deconditioning or is it physical deconditioning in a patient with the genetic vulnerability the pre-existing genetic vulnerability but there's a guy called benjamin levine in america who did some really interesting research and basically what he was interested in finding out was what are the physiological effects of deep conditioning if you take someone and lie them in bed for uh you know and decondition them what happens so he studied the heart size of patients with pots and he found that on average patients with pots had a 16 smaller heart smaller muscle mass compared to normal people so they had smaller hearts the amount of blood volume that was being circulated was between 13 and 20 percent less in patients with pots so smaller heart less blood and that could be another reason behind the symptoms that happen as a result of this lack of blood going to the brain interestingly what he found was that if you then exercise these patients through a three-month graded exercise protocol you could actually increase their muscle mass back to about twelve because you could get a twelve percent increase in the mass of the heart and increase the blood volume by about seven percent just through a graded exercise program i'll talk to you about that later and then there is this thing called autoimmune parts where people have said well maybe it's something about the virus we react to the virus we produce our antibodies and perhaps these antibodies attack us in some way and therefore inhibit how we would normally respond in terms of our autonomic functions to standing up the reality is that whilst there are all these sub types of pots in truth they all overlap and i practically never spent too much time looking for what type of pots it is i try and do the things that seem to work for the majority of patients so people with pots worry and they say look i i got the neuropathic type have i got the hyper adrenergic type we don't quite know because there's considerable overlap and i don't think it really makes a big difference to how we manage them certainly how i manage them but this is these are the kind of theories behind parts and why it may have occurred in terms of these you know people say that the neuropathic process tends to be more common it tends to occur very suddenly the hyperadrenergic pots comes down more gradually and tends to be associated with high blood pressure on standing and the developmental type of pots which comes from puberty because the bottle has become bigger during puberty right so the bottle is becoming bigger so you've got the same amount and the idea with the patients who have a developmental type of pots is that there is hope that as the patient gets older and they reach a steady state the symptoms resolve and we do see that as well it's also important to say that uh a lot of patients with pots have a bunch of other associated disorders a lot of patients with pots uh are told they have anxiety whether anxiety co-exists uh or whether it is the fact that no one believes them that causes the poor patient the anxiety which is completely understandable i don't know but yes there is no doubt that a lot of patients who would come to me with symptoms of pots will also carry a diagnosis of anxiety and i would say about 40 percent 30 to 40 percent of patients with chronic fatigue syndrome probably have pots as well and certainly whenever i see chronic fatigue syndrome i'm always thinking could this patient have pots so i i would urge anyone with chronic fatigue syndrome to just think about that because there are things that we can do with pots which can make a difference to a person's quality of life deconditioning depression fibromyalgia mitral valve prolapse joint hypermobility syndrome reynolds disease irritable bowel syndrome migraines these are all things that i see very commonly in patients who have parts we also see things like secondary triggers alcoholism bed rest diabetes etc which seem to contribute to patients developing more symptoms but it's just interesting so whenever i see fibromyalgia in a patient you know they've been coming to the hospital for several months with chest pains etc and no one finds anything i'm thinking oh could this be pots could this be dysautonomia what should what should we do how should we investigate them um i think all patients should probably have a blood count at least you just make sure that there's nothing else like anemia because if you're if you just have less blood then that can typically cause similar symptoms of orthostatic intolerance so the simple blood test to tell you that you're not anemic can be helpful electrolyte deficiencies so that's important low potassium low magnesium levels can contribute i think every patient should have their b12 folate iron levels checked and virtually i would say 90 percent of my patients with bots are vitamin d deficient as well i think if there are any symptoms to suggest that there may be an autoimmune condition like dry eyes dry mouth or rash any other thing like that then we would check auto antibodies the if you speak to the immunologist they say check a serum tryptase to look for mast cell activation syndrome having said that when you talk to mast cell experts they say that's a notoriously poor test so again if if you have members who are who've been told oh well you don't have it because your trip takes it's normal i wouldn't go by that and i think there are some really good experts who have more sophisticated tests uh that they can run to look for muscle activation syndrome i think it's worth just checking catecholamines so a lot of people who are diagnosed with pots will go through 24-hour urinary catecholamine collections and that is largely to look for tumors that are secreting adrenaline that's what you're looking for that maybe it's not this condition called pots but it's a tumor sitting there releasing adrenaline and that's why the patient's getting their symptoms so i think it's a worthwhile thing to do but generally the pickup is incredibly low i don't think i've picked up um a tumor secreting adrenaline in any patient uh that i've come across so far alter monitoring is something which allows us to see what is happening to their heart when they're getting the palpitation generally in pots what you would expect is to see the heart rate rise completely normally so the rhythm is normal but the heart starts going faster when they stand up but once in a while you will find patients who get this thing called the supraventricular tachycardia where the heart goes fast because they've gone into an electrical disturbance and it's useful to know this not because this is a dangerous condition but because this is a curable condition if your heart rate shoots up because you've got a supraventricular tachycardia then we can cure that once we've identified it by doing something called an ablation and the heart rate when go up they can coexist so you can have pots and you can have superventricular tachycardia and if you treat the supraventricular tachycardia it doesn't mean the pots goes away but it does mean that the patient may be able to stand for longer because they're not getting the supraventricular tachycardia so that's the real utility of halter monitoring in this setting but then the diagnosis the test that everyone does to try and make the diagnosis is this thing called till table testing and tilt table testing involves lying the patient down strapping them to a table and then measuring their heart rate and their blood pressure in response to change in the level of tilting and change your response to change in posture and when you look at what should happen in healthy people and this is telling you when you're tilting them and what happens to their blood pressure here and what happens to their heart rate the heart rate goes up ever so slightly but not hugely and stays like that the blood pressure doesn't really change very much but if you look at a patient with pots the blood pressure doesn't change very much but you see this rise in heart rate and the heart rate stays much higher and then comes down when you tilt the patient backwards um um the the the um [Music] the question is how long do you need to tilt someone for to be able to see these changes and the answer is that virtually within three minutes the majority of patients will get their heart rate up so at three minutes the majority of patients will um get if if you have pots your heart rate should shoot up excessively by about three minutes by seven minutes virtually all patients tend to have demonstrated this rise this excessive rise in heart rate so that's how long you need to tilt them for you don't need to tilt them for more than that the problem is that when patients with pots do have tilt table tests it just destroys them it's it's a very very difficult uh test for patients because it's very uncomfortable and secondly often it will knock patients off that stable course for months or weeks you know after the tilt table test is it a necessary test and i would argue it isn't i don't think that the diagnosis is based on the test i'm not here as a doctor to treat a test i'm here as a doctor to treat my patient and therefore i would want much rather listen to the patient and regardless of the tilt table test make the diagnosis i um happened to go to a conference a few a couple of years ago when i when i met professor gaul in london and he was doing a talk and i asked him the question i said you know the problem with this is when you are making a diagnosis on the basis of a test you have to first and foremost know how good the test is and no one's done that work with till table testing what you really want is you want to take a bunch of people who have pot or who you think have parts you want to put them through till table testing you want to see how many come back positive how many come back negative and you then want to treat both groups as if they have parts and see how many respond and that will tell you how good your test is but no one has done that work so yes a tilt table test may help you make the diagnosis but does a negative tilt table test refute the diagnosis and if you ask most experts now who deal with this i would say 50 of patients who ultimately respond to the treatment for pots will have a negative tiltable test and this is the problem that if you end up having a negative tilt table test you're told you don't have it and your search continues you're left high and dry when you don't have bots go away well that would how does that work you know so personally i would say well okay the you know i do the test not because i don't believe the patient i do the test to validate the patient and i do the test to validate me but if the test comes back negative and the patient sounds like that that's what they've got and no one else has been able to offer an alternative explanation then you know why wouldn't i want to get on and just try and treat and hope that something works for the patient there are lots of disadvantages of a tilt table test it's not easily available it needs to be done in an experienced unit it's very uncomfortable for the patient if the patient takes medications that may then that may lower the diagnostic yield but taking the patient off the medications risks uh upsetting the balance that you've managed to achieve ideally it should be done in the morning because that's when the patient's symptoms are the worst there's inadequate data on accuracy patients do have good days as well and that you know and that's just very much the nature of the condition it's not something you can do in pregnant women and one of the groups of patients that really suffer are pediatric patients because you know many centers don't do till table testing on children because they think oh we need pediatric support etc but these are children you know who pediatricians don't get very interested they can't come to see a cardiologist an adult cardiologist and they lose out on the most formative years of their lives feeling miserable and just you know they can't go to school they can't hang out with their friends for lack of anyone wanting to just get on and try and make a diagnosis and this is a big problem with pots and i i certainly you know i'm an adult cardiologist but to my mind it just doesn't feel right that you've got kids out there who are suffering whose moms have got parts but no one would acknowledge that that the child could have the child may have exactly the same symptoms but the mom can't get anyone to get interested in the child so in my case i just say well come along and we'll just do whatever we can because where else what what else do you do how do you leave these young people who could be out there and enjoying themselves with their friends how would you leave them feeling like oh well you're just uh lightweight and and uh you just need to get on with it and you're just anxious and i think this is crazy so this is a real unmet need and i think as cardiologists as pots experts we have to work with pediatricians and we have to start saying to them that let you know let's work together let's try and get these kids some support now how do you go about managing this condition and the problem is that there is no one size that fits all but in general i think there are four prongs to trying to manage patients the first and foremost thing is to understand and to accept what the patient tells you i think i think to develop a rapport with the patient to to tell them that you believe them to tell them that you understand the struggles and to tell them that whilst you may not have all the answers you're there to support them it's really really important i think it's important to educate patients because a lot of patients have gained everything they have from doing google searches and you know inherently there are problems with that so to be able to educate them and give them the knowledge that i know and at the same time say to them that look i don't know everything but if there's anyone else you find who knows more than me then i'm very happy to work with them too because this is about you this is about getting you better i think it's important to identify and withdraw exacerbating medications so in terms of medications things like medications that lower the blood pressure unfortunately what happens in patients with pots who have hyper adrenergic pots is that when they stand up their blood pressure goes up now what will happen is they go to a gp the first thing the gp does is let's lower the blood pressure well that makes their symptoms worse because their blood pressure was not going up because they had high blood pressure their blood pressure was going up because they had too much adrenaline in their system so that kind of thing makes things a lot worse often these people are given snris uh because they're told they have anxiety so they're given things like venlafaxine deloxity snris block the uptake of adrenaline so you leave more adrenaline in the system so those are medications that will worsen the tachycardia will make things worse diuretics same things so there are tons of medications that can actually make symptoms worse and sometimes taking them off those medications can help i think diet plays a big role because a lot of patients with pots develop esophageal and gastric dysmotility remember it's a dis it's a it's a disturbance in your flight and fight and your rest and digest so a lot of patients with pots will find that they just don't get stuff through they get a lot of bloating they get nausea they get cramping in there and they're constipated for days in that sense the things that seem to help is it's changes in diet so a lot of people benefit from going gluten free some people benefit from a low histamine diet one of the things a lot of patients with bots will complain of is they'll say when i've had a big meal my heart rate shoots up i become dizzy the thing that's happening there is they're getting a condition they're getting something called splenic pooling splanknig pooling means that all the blood accumulates around their stomach beds which means that for the duration of that time there's less blood in the bottle there's less blood circulating around because the blood is collected around the stomach so small meals protein rich meals small regular meals so six meals a more ketogenic kind of diet not not crazy ketogenic but just more protein less simple carbs less sugars regular meals small meals makes a big difference gluten free makes a big difference um i think compression helps again with compression you know and i'll talk about compression in a second you're squeezing the bottle so you're allowing more blood to go to the brain sleep management yeah a lot of patients um virtually every patient with pots finds that they don't sleep as well as they should and when they don't sleep their symptoms get a lot worse so they really struggle so if you can improve their sleep in some way then that makes a huge difference to how they feel um stress and pain management and exercise now let me just uh talk you through these i think it's important to tell patients this is not a dangerous condition despite the fact that it feels hideous it doesn't actually shorten a patient's lifespan but it is also important to accept that it is a debilitating condition and it has a huge impact on the quality of life of the patient and it's important to have that conversation not only with the patient but with also with their kick with their husbands partners family members because often patients have faced this struggle where they just feel rubbish and people around them don't necessarily believe them i think it's important to say this is a chronic illness i think it should be managed by a specialist in a multi-disciplinary and holistic setting i think you have to involve a dietitian you have to involve physical therapists etc and all of these people should be talking to each other and all the decisions have to be made in partnership with the patient it is not a case of oh go and see a dietitian who you've never met who will just give the standard therapy it's a standard advice or go and see a physiotherapist who said it's about having a team of people who are interested in the condition and more importantly interested in that patient with that condition i think it's important for patients to understand they do that they do improve over time but it's never a straight line they zig and they zag and often you know they come with one condition with one set of symptoms and those set of symptoms get a bit better but something else creeps up but on the whole patients tend to improve and they do achieve acceptable quality of lives i think it's important patients should be empowered to look for aggravating triggers and aggravating activities and wherever it's possible uh minimizing those triggers is helpful as a pots doctor the big one of the most useful things i can do is be the patient's advocate and i certainly try and do that where um you know where you have a young person who's worried about their exams but they're just they just cannot do anything they cannot function first thing in the morning then as their doctor i would want to write to the school and say look you know this person is very capable they're very willing but they have this condition if you make small adjustments they'll do the best that they can for you without compromising their health and often you need a medical professional to take that initiative and give it like that to schools employers etc and that's why i think that's another thing that i found you know when i as a doctor i wish there was this wonderful pill i could give every patient and just get them better i can't so but i do think that doing things like that can help their lives and so why not as i said these are all the medications that can exacerbate parts um you can have this slideshow by all means in terms of diet as i say in terms of diet we talked about diet but i think it's important to fill the bottle up you want to fill it up as much as possible so you want to drink at least three liters of water a day and perhaps more more in hot weather i think most people drink more but they don't have enough salt and the problem with that is that if you drink more and you don't have enough salt then the water just goes out in the urine and therefore it becomes really important to keep that water in the body by increasing your salt intake a lot of people are worried that salt is bad salt is in this setting salt is one of the best things that you can do because you're trying to improve the blood pressure you're trying to increase fluid retention uh for a lot of people salt itself is unpalatable so you can take slow salt tablets rather than just taking table salt but also combining that with electrolytes oral rehydration cells is good i think magnesium supplementation is really helpful three quarters of the population are magnesium deficient and i think it's important to correct any nutritional and vitamin deficiencies in fact a lot of people who've taken vitamin b supplementation have benefited and i think it's important to avoid things like soda caffeine diuretics alcohol smoking wherever possible and with regards to sorry with regards to compression compression has the effect of reducing cooling it increases the amount of blood returning to the heart compression is a big problem because lots of people don't know how to get it what you want to do is you want to do you want to acquire uh riles class 2 stockings class 2 or class 3 stockings some can be bought commercially but the gps can prescribe these as well and ideally you want to compress up to as high as you can so the higher you compress you know if you go all the way there you have a much more effective return compared to just there so if you can if you can get stockings which go to above the waist or can be combined with a waist attachment or even these things called magic pants then that can help push you know improve symptoms a lot of people struggle to know where to get measured the best place to get measured is the lymphedema clinic because they do it all the time so if you have a local lymphedema clinic they can measure you out and then with those measurements you can either buy them commercially or get them from your gp now virtually all patients with pots have disturbed sleep and that contributes in a big way to fatigue now why is that why do they have disturbed sleep there's a very interesting guy called professor allen pokinki if you ever get a chance he's done a ton online and he's a sleep doctor and what he has noticed is that those people who don't sleep well if you measure their adrenaline levels their waking periods seem to be coinciding with adrenaline surges and what he is calculating what he's saying is or what he has said is that when we go into rem sleep and we have dreams or anything like that or if there's any disturbance around us because we're producing more adrenaline because the patients with pots are producing more adrenaline and adrenaline is a hormone of arousal the patient ends up waking up so if a dog barks you know two people are sleeping in in the in one person who doesn't have pots you get as tiny adrenaline rise they don't notice in a person with pots the same dog barks you get a huge adrenaline rise the patient wakes up often the patient will wake up with their heart pounding sometimes the patient may wake up not realize that woken up and then go to sleep again and wake up when the next kind of uh stimul you know stimulus comes along so in that sense it is important to try and work with sleep hygiene it's important to keep all phones and things away from the from the bedroom where you're sleeping so that there's no lights shining in the middle of the night there's no noises a lot of patients with pots have pain you know probably because of their joint type of mobility and whilst we're very good at taking painkillers during the daytime we don't necessarily concentrate on taking pain relief at night when we're sleeping because our automatic assumption is i will be sleeping so i won't experience pain but pain can can manifest with rise in adrenaline levels and therefore taking painkillers at night before going to bed is a good thing a lot of people ask me about sleeping tablets whether that can improve sleep patterns sleeping tablets help you go to sleep they don't help you maintain sleep and for a lot of patients with pots it is the maintaining sleep that is the problem can you reduce the adrenaline surges in some way by taking something like beta blockers and that's very interesting and this guy alan pokinki that i was talking to you about has tried that where he's given a small dose of beta blocker at night in the hope that the beta blocker will blunt any adrenaline surges and certainly those people seem to respond some of them seem to respond another thing that can be helpful is to sleep tilted and the idea behind that is that when you're sleeping like this when you sleep when you're sleeping flat then there's no blood there's no surge of blood in the legs so more blood goes to the kidneys and because more blood goes to the kidneys the kidneys think you're over hydrated so they produce more urine and you wake up more dehydrated in the morning if you sleep at an angle then there's less blood going to the kidneys so the kidneys don't produce that much urine overnight and therefore you wake up more hydrated and if you give this like a three month period your body adjusts and you retain more fluid so that's the idea behind sleeping tilted and there is some evidence that that can be quite helpful in terms of increasing blood pressure but it does take time it isn't something that happens overnight i think it's important also to say that there is a vicious cycle when people don't sleep well they feel more tired when they feel more tired they get more depressed when they get more depressed they experience more pain and acting on every component of that cycle the sleep the fatigue the depression the pain is important stress will make everything work worse and a lot of patients currently with pots have said that they've felt better since the lockdown because they're not so regimented they don't have to get up as quickly so working with employers to try and modify working schedules is important and patients should be encouraged to embrace other holistic and alternative therapies that improve their quality of life so i would always encourage my patients to see if an acupuncturist can help them physiotherapy yoga all that kind of thing with regards to exercise now there is obviously a difference of opinion i think between chronic fatigue syndrome and in parts and i can't really talk for chronic fatigue syndrome because i have very little expertise but in the pots patients the general feeling is that exercise helps conditioning it should be gradual and initially it should be non-upright and then as you develop a regime you can slowly build up to upright exercise with focus on resistance training and especially the lower limb muscles and benefits tend to be seen after four to six weeks and ideally the exercise regime should be at least every other day for 30 minutes the benefits can persist even after three months but the patient continue has to continue doing it otherwise the benefits tend to uh go away this was an interesting study done by benjamin levine again and basically what he was looking at is the physical component scores before training and after training and you can see that there was this sudden there was this quite marked increase in how people uh uh felt uh from their parts after a three-month training period similarly their social functioning score also improved after three months of training and he called this protocol the levine protocol and that's the protocol that is generally recommended these days he also showed that actually when you did this you could increase the blood volume and the mass of the heart so you can actually change the structure of the heart to allow it to accommodate more blood and to be able to pump more blood out with each heartbeat after a period of training so exercise certainly for pots is good and is encouraged but it has to be done gradually in terms of management one of the things to say is there are so there are medications that can help all these medications are off-licensed so they're not specifically licensed for this and one of the problems is that because they're off license there's a reluctance doctors are reluctant to prescribe them doctors want evidence they don't they want someone to go and say oh it's okay to prescribe it and no one's gonna say that unless there's big evidence well to my mind how are you gonna get big evidence if you're not going to acknowledge the patients if you're not going to even recognize that these patients exist how are you going to develop the big evidence how are you going to develop evidence with the medications if you're not going to use the medications you we cannot wait for 20 years for someone to come along and start funding the study these people are suffering now so in my own mind i say to the patient these are off license medications but here are some studies which have been shown to help and actually not harm do you want to try them out and more often than not patients will say yes i want to do anything that makes me feel better and so i'm very proactive with prescribing medications uh if the patient wants them based on small scale studies because this is the way i'm going to develop a database of experience with these medications so in terms of medications there are two types there are medications that help you retain more fluid in some way by either increasing the amount of water in the bottle or making the bottle smaller and then there are medications that reduce the heart rate so in terms of medications that make more volume or make the body smaller we use something called future cortisone which is an aldosterone analog helps you retain more water it's generally well tolerated it can cause high blood pressure in patients but generally this is not such a big problem in patients with pots because often their blood pressure tends to teach around the lower range it can sometimes cause fatigue nausea headaches and even edema swelling of their legs because of their retention that edema is one of the main reasons people don't like taking it but otherwise for some patients it makes a huge difference another medication that we use is desmopressin desmopressin is a spray i use it as a spray desmopressin historically used to be given to bed wetters right so the idea was you squirt this thing in their nose and they wouldn't wet the bed because they would retain the fluid in their bloodstream it's it's a short acting thing it works very quickly it works for about four hours and it's often used as a special occasion drug so once a once a week if a person is facing a day of having to stand up at work or something they can squirt this in their nose and hopefully if it works it should give them get up and go for the whole day or for at least four hours of that day as i can as i can show you here the studies are very small you know this guy raj satish raj she did it on eight patients so this is where the problems are but in my own experience i've found that whilst a lot of patients don't tolerate it the patients who jogger who do tolerate it find it to be something that really makes their quality of life better there is another medication called erythropoietin erythropoietin increases the amount of blood cells in our body it increases the blood cell volume it's a very costly medication it's given subcutaneously and the big problem with it is it can cause blood clots i've never used it myself but this is something that is purported to be another treatment option but i've never really used it because it requires subcutaneous administration and the risk obviously of causing blood clots but i do use let me just tell you about this i do use something called midodrin medidrin is another medication which is a medication which reduces the stretchability of our blood vessels so because the blood vessels don't stretch as much when people stand up there's less less um room for the blood to accumulate in the legs and that supposedly is a that that seems to help a lot of patients as well i can i do use it in children as well as in adults one of the big problems with midadren however is that because it makes all the blood vessels a bit tighter it can cause high blood pressure when the patient is lying down and therefore we don't give it for four hours before the patient lies down or goes to sleep but we do use it during the day time one of the problems we face is that a lot of patients with pots can't be up on their feet for a prolonged period of time anyway so they're lying down most of the day in which case it doesn't necessarily serve their purpose to take something like that but in those people who who are able to take it it does help so those are the kind of medications that will actually either increase volume or make the body smaller so whatever volume you have is more effective but then there are medications that reduce the heart rate in terms of medications the commonest medication that is prescribed are beta blockers propranolol and beta blockers do reduce the heart rate but they also have a bunch of other side effects like causing fatigue causing the blood pressure to drop so most patients i've come across don't seem to tolerate beta blockers as well interestingly there was a study again by benjamin levine comparing exercise training with beta blockers and what he found was that actually when you look at beta blockers compared to exercise training the heart rate comes down with beta blockers and it comes down with exercise training but when you look at the physical functioning score how the patient responds or how they say they're functioning exercise training with exercise training their physical function improves their social function improves with beta blockers those things don't improve so you reduce the heart rate but you don't necessarily make the patient feel better whereas exercise does do that a much better agent in my experience is something called ivabradin hyperbradin is a medication which purely acts on the heart rate it doesn't affect the blood pressure and that is my kind of go-to drug whenever someone comes to me with symptoms of pots because it lowers the heart rate and does so very effectively without giving the patient any side effects but again the data around divergence very small but anecdotally it does help 70 percent of my patients um and let me just talk you through anything else another thing that can sometimes help with intravenous saline and this idea that actually you just go on and put some some more water in the bottle very effective when it is given the problem is that the beneficial effects don't last very long so within 24 hours the patient goes back to feeling like they do and so people have said well can we not just give intravenous saline chronically continuously and the problem with that is there are no easy ways to do that because either you'd have to give the saline through peripheral cannula which block off or you would have to put in a port and the problem with that is that there is a substantially higher risk of causing blood clots and there are ver there are no centers that i'm aware of that do this and therefore it's not recommended but as a idea as an idea that if you can just put fluid into the person it works really well and for those people who do get into venous fluid they just think oh my god my cloud has been lifted over my head you know there are other medications there are medications one of them is clonidine clonidine is something which acts against adrenaline it's used as also as a blood pressure lowering agent for some reason but it does it does work but very poorly tolerated there's another medication called methyl dopa i have no experience at all with these medications but there these are other medications that are on the horizon some people use them but i think in no ways have i found that there's any literature to say that these are more effective than what we do anyway this is a particularly interesting medication called mestinon pyridostigmine mestinon is an acetylcholinesterase inhibitor so as i was telling you you have a disturbance between your flight and fight and your rest and digest and all the medications i've talked to you about so far are medications that are basically trying to blunt the effects of adrenaline nesting on works on the other side and it starts improving your vagal hormones so your rest and digestion means it's a little bit like having a screaming child in your house and uh beta blockers and everything else is like saying okay don't you're not allowed to scream any louder but mestanon's a little bit like that nourishment that a mother gives and she gives the mother the child a cuddle and makes him feel loved and all that and he stops screaming so mestinone is a really interesting medication it reduces the heart rate it is exceptionally good for gut motility in patients with gastroparesis gut dismatility and and lots of people tolerate it very well and for some people it has been a complete game changer they come back and they say well i can function i'm amazed it doesn't work in everyone 60 of patients get better with it but it's another medication to think of and then there's something called droxy dopa again these are supposedly reduced dizziness and fatigue one of the things which is a real problem and no one knows how to deal within uh potter's brain fog this idea of just cognitive dysfunction and there is this medication called modafinil uh which is a psychostimulant which has been shown to actually improve brain frog the the problem with stimulants is that yes they can increase improve brain fog but they may make all the other things worse because you've already got too much adrenaline in the system and you add in a stimulant you would make things worse for the patient but all the research points out that modafinil actually does work as a stimulant but it doesn't make the pots bit worse but it makes the brain fog better so in conclusion what i'd like to say is that you know i think now that we are becoming aware of this condition and there is some expertise patients should not hesitate to go out there and ask to go and see someone if they think that they have their symptoms they should not be put off by perhaps well-meaning but uh poorly informed doctors that they that they're they don't have it i think it's worth going and seeing someone who has an interest in that condition i think with treatment 70 to 80 percent of patients improve uh and return to an acceptable level of functioning as i say pots patients have been and continue to be neglected by the medical profession but things are changing and i think raising awareness and working in partnership with healthcare providers will improve understanding and will allow us to have hopefully gain expertise in more treatments and this is the way we're going to get there ultimately i think as i say it's a remarkable condition that affects remarkable people and a lot of these people find strength and resilience in the most terrible debilitating state and it is the remarkability of these people that will allow us to try and beat this condition so thank you so much i can't hear it i can see i can see people clapping but i can't hear anything so you can unmute yourself and if you haven't delighted chance i'm thank you so so so much dr gupta that's um an incredibly thorough um uh tour of the whole the whole field which has been absolutely fascinating um i'm wondering if you want to stop that's it lovely and and then we'll come back to the whole group i'm going to manage the questions through the chair just because that's the most efficient way of managing time and um but people have given me um i've got pages of them here and i've been trying to check whether they've been answered as you've gone through and and um fortunately you have covered um many many things there's a few a few ideas i just wanted to pick up which is about the differential diagnosis of of pots versus mecfs you know the symptomology is so similar and it's so different and that perhaps also leads into this whole thing about you know gps seem to be now willing to diagnose people as having cfs but they really seem to be still so informed and unable to access the sorts of science that you've just talked to us about in terms of parts and some of the things that you know some of the other symptomology that might be going on for people so there's one particular question which is like how do you talk to your gp when they're just not well informed and you may have cfs you may also have pots you may have other symptoms things going on as well fibromyalgia on code or whatever but um you know how should you approach your your gp because i looked yesterday on the potts uk website and there was about a dozen um specialists around the country who who say that they have a you know an interest and something to offer in terms of pots that is very very few so it's it's it's it's considered to be a rare disease but certainly um the medical response to it is rare i think i think you're right and in my experience even though there are doctors who have an interest in pots what you have to be interested in is in the patient you have to be interested in patients right again my aim is not oh if you don't have pots i'm not interested in you um you know my aim is whatever it is my problem now is that because to a man with a hammer everything is a nail so i often worry that everyone who comes to me gets diagnosed with pots and maybe i'm diagnosing it too much but i know that the treatments are not hideously dangerous and i'm very happy to be guided by the patients so there are times when someone comes along and and they say you know maybe i've got the diagnosis wrong so i would treat them and if they come back and they say look i'm better and i think well you great you know that's what we my ultimate aim was not to try and work out whether i was right or wrong my ultimate aim was to try and make the patient have some kind of quality of life so um wherever there is any kind of dilemma i to truly believe that it's worth just going for it and trying to treat the patient or making those things available to the patient that they can choose to try out in terms of where you go with the geps this is a really difficult scenario but there there are now major societies which recognize this condition so the canadian cardiovascular society has a position statement on parts the heart rhythm society has this condition so there are august bodies around the world that are now beginning to believe that this is a real thing and they had defined it in some ways and so being informed and being able to carry that literature and showing that to them and saying look you know i was looking at this and this is what this says and i think i've got all this and here is this someone who can potentially help me what do you think i think that would be much that would be a very compelling argument and i can't see how someone will turn around and say oh well it doesn't exist then because here is a learned body stating that this is something that does exist okay i would certainly be helpful um either for our group or with your support to produce a kind of little pack or an argument to to give to a gp i think the other the other obstacles um which um i don't know whether other people here would echo but is is that gps are so unwilling to prescribe things that are offline that um what they want is this kind of somebody behind them who says it's okay so they want the hand-holding relationship and i was wondering whether either you or anybody else in the country does this kind of partnering with gps whereby um you know the gp is actually doing the prescribing but you're able to provide a letter of support saying this appears to be a wise thing to do in this scenario these are the risks the patient needs to know the risks but but it's worth trying is that something you you're ever able to do i do i do i mean i i often um gps would say could you prescribe the initial doses and then we'd be happy to take on the thing to my mind it's a very simple straightforward argument this is not a dangerous condition all you're doing is to try and make the patient feel better if the patient comes back after trying what you've given them and said look i don't feel worse and actually i feel better and they haven't said that for the last 10 years then i think there's all the reason to go for it and so a lot of gps do see that they see that actually this person seems to have responded so people are doctors are developing that confidence but where the doctors don't have the confidence they'll write to me and say we're not comfortable doing this in which case what i would do is i do the prescriptions and send them to the patient every month or which is hard work but you know if something is suiting the patient the last thing it is unacceptable to me that a patient should continue to suffer because someone is not willing to put themselves out of their comfort zone that is i wasn't i do i absolutely it is against everything i believe in that if i cannot be bothered to put myself out of my comfort zone for my patient then there's no point in me being doing what i'm doing so you know there is you have to do things you have to do things and when if hopefully nothing ever will go wrong but if there's something that then that at least i have my conscience and i have the fact that i'm doing it and i have the patience on my side you see that is really important to have the patient on your side for the patient to truly believe that you're trying to do something to help them with no ulterior motive no you know and so communication is so essential this idea of making people understand that you're on their side and you don't know what the answer is but instead of them having to fight it on their own they now have someone besides them it's really interesting i'll give you an example of the kind of thing that happened i have a young girl with potts whose mother today so today um she rang me her mother has terminal cancer and she has to go to guernsey now the problem is they can't let her go to guernsey unless she gets a vaccine so so the the she goes to the gp and says look can you not on compassionate grounds give me this vaccine so that i can go and see my mother and the gp says well i appreciate that but we can't make a precedent to my mind that i cannot believe that isn't that exactly what is wrong in making a precedent of compassion what is wrong in stepping out of your comfort zone for the patient isn't that what we should be doing how can we say so this is where a pots doctor i think or a good or a caring doctor comes in because he would say no i will say to them that i think you've got a condition that needs you to get the vaccine so that you can get on this so that you can go water that simple thing will do more for her mental state to be able to go and spend that time with her mother while she's dying than any other medication i can give her so you're getting a very positive response from the chat on that i think we would um yeah we would love to take that quote about compassion and stepping outside your comfort zone and put it on every gp's office in the country and uh certainly the emmy and fibromyalgia community need that sort of advocacy on their behalf um i'm going to ask a few more general questions but just following that on with one very specific one quite a few people with mecfs are prescribed with amitriptyline as particularly as something that potentially aids sleep usually very low dose um so there's a question um i haven't noted who's asked it but is is there any sort of contraindications about amitriptyline and pots i mean i i don't think there's an absolute contraindication with any sort of thing you know so there are patients for example monoamine oxidase inhibitors can make the pots worse and so can try cyclic antidepressants but at high doses so the the first thing i would say is even though i said that about the ventilation and the deloxetine and the fact that you know if the patient says to me look when i come off it my mental health suffers i say well go back on it you are what matters not my thing you're what matters no numbers matter no diagnosis matters what matters is you and you're a physical being but you're a human you're you're you your mental health is important and if this makes you better then that's okay too i'm here i'm i'm here to support you you know we'll begin nothing is right or wrong this is all about you and what works for you so i wouldn't automatically stop people if they came in on an antidepressant or amitriptyline or anything like that but if they came to me and said you know since i started this i think i'm worse then i would say well maybe what you could do is talk to your doctor about trying to come off it and see whether you feel better and if you don't you can go back on it if provided you feel it's doing something good for you in some other way okay so it's the patient-centered medicine approach which is indeed here yeah uh one of the the probably more um sort of challenging bits of of your talk has always been um has been this whole issue about exercise so um the me cfs community has been as you will know from the 9s guidelines around me cfs if you've had a chance to read them the most recent draft that's been issued is very clear that graded exercise um is not something that should be recommended to people with with any cfs and yet it definitely is something that that that you've demonstrated some evidence because it really helps people with pots is what is the way through it and and i i guess what we're kind of thinking of is is horizontal exercise so exercise that doesn't tip you um and doing you know is is that is that a way forward because um we you know certainly we've always encouraged people to do as much exercise as you can get away with in your energy envelope so it's not no exercise it's just do as much as you can without triggering post-exertional malaise but yes is horizontal exercise useful i think so i mean i think i think the big problem is where deconditioning is bad there is absolutely no doubt about that deconditioning is bad so i i think anything that a person can do to try and avoid deconditioning i don't think it necessarily means that you have to go outside what you know outside your comfort zone to do things but it is important still to try and do a little bit of something within that envelope as you put it every day i think it is good for our mental health i think it's good for our muscles and our physical health i think it's good for our immunity so it's a very tough one i asked this question at the potts master class a couple of years ago no one had an answer and i said how do i know you know which what is right but what i do definitely know is deep conditioning is bad regardless of what the diagnosis is so wherever possible i would say horizontal exercise even things like just squeezing a pillow between your legs several times a day that kind of thing is good you know um whereas so yeah i think i think that's what i would say beaten bed bound i think is worsens the problem wherever possible but yeah you don't have to do you you shouldn't do something which just makes you feel completely awful in the hope that in some you know i mean maybe it's something we could pick up on our website forum that we want to get going would be would be to share ideas of um the kits just put one in the chat about the pilates classes but share ideas about the kind of exercise that people um who are really um incapacitated through emitted pots or whatever but do manage to find achievable um without putting you know particular pressure pressure on them we had a couple of questions right at the beginning which was all about the um this thing about the variation through the day um is that and you kind of certainly seem to imply that that you know mornings are generally worse for people and things improve as the day goes on is that is that if you're a bit more upright does that apply to people who are pretty horizontal anyway or is is that a kind of um i think i think it's being a cortisol thing or yeah i think it's the dehydration from the night before that contributes okay so the fact that you're dehydrated ironically i mean i think i think it's the standing up after the night that is the issue so if you're bed bound continuously i don't know whether it's so much of an issue of course people do get surges off to meals as well so if they have a big meal then kind of thing um but yeah i think i think um [Music] dehydration being a something to really think about dehydration is absolutely so rehydrating before you stand up that's so i definitely recommend that i would say to most people before you get out of bed take a jug of water some electrolytes etc with all this with this kind of um thing i talked about um the more because the pivot is moved this way the more the swings the more the pivot moves continues to move this way so if you can minimize if you can minimize the swings wherever possible the pivot starts moving back so again the idea that actually yes it is part of the condition that you will your heart rate would go up but wherever you can avoid it wherever you can reduce that by doing something beforehand it's good because that adrenaline stays in the body it doesn't tire you out okay um we're inevitably going to cover mention kovid um two things really which is have you begun to notice any correlation between pots and long covered in particular and secondly could you just say anything about the vaccine um whether there's any problems i would say um number one we are beginning to see a lot of i mean 10 of patients with uh you know cobit then develop this kind of constellation of unusual symptoms that goes on for a few months afterwards brain fog tiredness etc question is why those 10 what is this what is do they have a genetic vulnerability that has been brought out by by the virus or is it just is it just the virus i don't know in in those patients i've seen again the same things apply you know i tell them to keep really well hydrated wherever patients complain of dizziness to my mind dizziness is a lack of blood to the brain so if you can increase the volume in the body that seems to help the patients who have gotten better with their brain fog etc other patients who said yes when i increased my salt when i increased my water when i increased my electrolytes that made a difference concentrating on sleep regular again slow exercise graded exercise helps in a big way i i i wonder whether this is something that has brought about that has just pushed you know that maybe some people were carrying a vulnerability and again being hit by the viruses just brought that vulnerability out they certainly don't seem to be as badly affected as people who get ebv or glandular fever because you know it seems that those patients who have vulnerability get glandular fever get really unwell um with regards to the vaccine the the big the big risk from the virus tends to be in people who are older and by you know the majority of patients that i come across with parts etc tend to be relatively younger so there are generally people who although they're debilitated they're unlikely to come to a huge amount of harm from the virus itself however having the virus could undo all the good work that they've done with medications conditioning etc so i now write to my patients and my gp and the gps and i say well i think that's really important you know i think the fact that here is a person who is finally beginning to see the light at the end of the tunnel and and if they get the virus then that may knock them out and that may set them back you know so many uh so many uh years so i think you should think about prioritizing them so i i say that um even though it doesn't necessarily meet the criteria but i suppose perhaps that's one advantage of having a condition no one knows about if you've got a doctor who's on your side then he can just add his weight and everyone will just listen because they don't know enough about the condition so that's what i try and do so i i normally write to their gps and say look you know work with the patient work with the patient they've got it's a hard enough life anyway come on let's make it a bit easier after so many years of struggling and any comments on the different sorts of vaccine or is that too detailed no i don't i don't have uh enough insight into but i mean no not really again we don't know the the only thing i would say is that the vaccines have only been around for a very short time and we don't know what the long-term consequences are if there are any uh so at this point in time i think it's it's what gives you peace of mind as as someone who is a patient if you feel that you're already you know you're more socially isolated because of the virus then for mental health it is good to be able to go out and do the things you want to etc so so i work with the patient and go by what the patient wants if a patient comes to me and says i really think i need this then i support them but i have no real insight into which vaccine is better okay there um there was a question right at the beginning um from the list which is um um do people fully recover from pots i have a few patients who have come back and said to me that i think i can go back to work and they go back to an acceptable level of functioning and i also think that no one i have is just terrible all the time i think people sort of have flare-ups when they're particularly low and then they get better my problem is i only see them when they're particularly low because that's when they contact me so but but all the times when i'm not seeing them many of them are actually doing okay and then something else happens so so it's not all doom and gloom it's not that you're stuck and you will only ever just get worse you will still go through good phases and bad phases but i think to stay at a good phase requires a bit of work so the people who who are really invested you know were exercising every day who are really paying attention who are managing themselves who recognize when they need to lie down etc they go and they maintain the quality of life that they want or they expected i have very few people who've come back and said i'm cured forever i've not got anyone like that but i do have lots of people i have uh young girls there was a girl who was wheelchair bound for 12 years and she's now playing tennis and she's running around and she's had a baby she's gone back to university she works really hard she makes sure that she takes regular precautions but she's achieving those things that she never thought she could have achieved and i think that for me as a doctor is hugely seeing seeing someone i've worked with flourish and to be able to celebrate with them when they achieve these kind of little gains you know these little victories in their lives is hugely satisfying okay um there's there's quite a lot of kind of detail questions really about comorbidities and particularly um people with other forms of orthostatic intolerance not necessarily pots i think you've probably covered most of that in the talk i'm not sure if there's anything um else that that would be particularly useful to add at this point and for people i think i think there's i think it's important again to say that potts is one face of this beast called dysautonomia orthostatic intolerance vasovagal thing could be all those are different faces but they all when you ask them you know they all will say the same things they'll all have sleep disturbance they'll have brain fog etc which is really interesting because if it's not lost it doesn't mean it's any less troublesome for the patient it's just with pots the heart rate seems to go up and the blood pressure doesn't fall with other conditions of blood pressure falls and the heart rate goes up in response to that okay and um there's a couple of old questions come up about sorry they're not odd particular questions that come up about coffee um gluten sensitivity um any thoughts on any of those particular sort of dietetic things yeah i mean i think i think when i i didn't know about gluten sensitivity and i did a survey i have with my patients and i think two to one people said when they went gluten-free they felt better so 60 felt better now that's not everyone and therefore again i would encourage people to try and work out whether that is them or not i you go gluten-free and if you know don't notice a different and if you miss your food then go back you know similarly with coffee i don't think there's a kind of cookie-cutter approach to this i would say again coffee can make things worse if you're drinking tons of coffee try and reduce your coffee and take and see whether that makes a difference and if it makes a difference great keep going if it doesn't and you really enjoy your coffee then go for it okay so in terms of of getting our gps much much more up to speed is that are you part of any training program do you do you do you know what is there what is it what would a gp know about pots would they know anything unfortunately not very much yeah unfortunately not very much but this is where i would love you know i would very much welcome people who are interested in coming and spending time developing a database i have about 400 patients now but because we're always working we never have the time to use that to start inviting people to say look you know we have a cohort of this many patients how about you bringing your research and offering it to my patients how about us using our patients and maybe interviewing them and developing literature surrounding their experiences how about using patients who have gotten better to educate other patients how do we develop all that how we do we work in partnership now to try and get the message far away that's what we need unfortunately we you know in my case for example it's it's very tough to find that time to be able to do that but that's what i think is the natural progression where now we've got 500 patients 400 patients the biggest studies would possibly 30 patients why can't we get people to bring in the funding and let's get on and do bigger studies so that people can then have the data to give them that confidence to be able to do it right that was my plan my aim was to get a huge cohort of patience my aim was to try all these medications that no one wants to try out on them my aim was to to sort of document their experiences and then use that as evidence as pilot data to get bigger research studies draw bigger funding etc the problem is i just it's very difficult so that's where we need support that's where i would invite anyone who wants to do their their pieces or their research you know i would love for someone to come and do that work the patients look to do that so it sounds like that's um the model of advocacy and demands for research that's been happening within the mecfs community also needs to happen um in the field of pots as well um i'm wondering um because time is moving on and we've we're um heading towards nearly two hours which is quite a long talk for us what um there was a nice general question which is what is the most important thing you tell a person with pots [Music] the the most important thing is um the the most important thing to say to them is that you're not alone there are lots like you and there are people out there who believe you and who are there to support you and i think it's really really important to understand that and whatever it is you're not alone you know that that i think and and and validating the patient is by far the most important thing i think for the patient to believe that they've been listened to and for the patient to believe that there is someone on their side someone who will work with them i think that's more important than anything else i think that's a absolutely brilliant way to end because that's one of the things that people in the me cfs community have felt has been so denied to them for so long and the same with fibromyalgia the same with many of these of these particular conditions so it's an absolute honor and privilege for us to have you and your time on a saturday afternoon and i'm very grateful to you for that and it's it's a delight really that that um that your whole ethos and value system about practicing is based on on listening to patients and um goodness i think everybody here would thoroughly approve that so can i say a huge thank you to dr gupta for um an absolutely fascinating talk we've had massive information in there and um uh we we're going to be able to share the slides is that right yeah we can put the slides up on our website um the recording of the talk will be up on our website it's saturday afternoon so don't expect it to at least the beginning of next week and uh um and a big thank you to the the team in the background to um to carol for setting the talk up to simon aviano and uh and thank you so much to everybody for joining us from all over the place so the chat has got all these interesting places in there so thank you so much dr gupta really really enjoyed today thank you thank you

Channel: Sheffield ME & Fibromyalgia Group

Views: 49,161

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Id: O932tRHsIOo

Length: 104min 43sec (6283 seconds)

Published: Mon Feb 15 2021