All the light we cannot see

Good morning. Lovely. Well, I've titled this talk all the light we cannot see. Because in many ways conditions like pots exist in that space. Partly understood. Often overlooked. And sometimes not believed at all. And perhaps the most important thing we can bring as clinicians is not certainty. But humility. And I think before we talk about the condition, I'd like to begin somewhere else. with the person who lives with that condition. Because conditions don't have identities. They don't have dreams or fears or aspirations. But people do. And unless we understand what a condition does to a person's life. Their sense of self, their dignity. Their future. It is almost impossible. To truly understand the condition itself. So I want to take you back 10 years to the first patient I ever met with this condition. It had been a particularly busy day. We were short of beds and the bed man manager in my hospital asked me if I could help discharge patients who didn't really need to be in the hospital. To make space. For those who did. And she handed me a list and one of the names on that list was a lady called Jane, a 32-year-old woman. And I asked why Jane was in hospital. And I was told she was a frequent flyer. That she had multiple medical and psychological diagnoses. And that in all likelihood what was wrong with her was simple anxiety. Jane had been admitted following a collapse. But all her blood tests were normal. All the other tests were normal and therefore the conclusion was simple. Jane didn't need to be there. So I went to the ward and I picked up her notes and I looked through her list of diagnoses. And you can you see the list of diagnoses on the screen. Yeah. Yeah, great. And so this is exactly what I saw when I opened her file and these were all listed there. And then after looking at that, I went to meet her. And I have to admit I wasn't expecting someone who looked unwell. And what I found surprised me. You know, Jane was actually sitting upright in bed, neatly dressed, composed. Looking at least on the surface entirely well. But there was a clue. That something might be wrong and that was that she had a nasogastric tube hanging from her nose. So I introduced myself and I explained to her quite matter-of-factly that we were short of beds. And that all her tests were normal. And that she would be going home. It was late in the evening so I told her she could leave the following morning. And what struck me was that she didn't resist. There was no argument, no frustration, just quiet acceptance. And I remember thinking even then that this was perhaps someone who was used to being dismissed, used to being made to feel like a problem. Rather than a person. And as I stood up to leave, I saw something that changed everything for me. My whole life changed that day in a single moment. And that was this. Does anyone know what this is? I do. — Yeah, and so this is a piece of paper that was partly hidden in the notes. This is a do not resuscitate form. And on it was Jane's signature. A 32-year-old woman had signed a document that said that in the event of a cardiac arrest she did not want anyone to try and save her life. And I was stunned. I remember thinking is she in her right mind?

What 32-year-old chooses this? And I turned back to her and I said, Jane, why have you signed this? All your tests are normal. There is nothing here that suggests that you have a life-threatening condition. You have your whole life ahead of you. Why have you signed this? And she looked at me and smiled. And she said well, because I think life should be more than just the absence of death. What is the point of having a life if I can't live it? And I didn't see any light at the end of this tunnel. And just as I was trying to process what she had said my phone rang. And I left the ward. But that night I couldn't sleep. I found myself tossing and turning thinking about Jane. Trying to understand why a 32-year-old woman would sign a do not resuscitate form. And the more I thought about it the less my earlier assumptions made sense. Anxiety doesn't do this. Hypochondria doesn't do this. Jane had looked entirely well. And yet something was clearly very wrong. And for the first time I realized that perhaps the problem wasn't her story. But my understanding of it. And so I wanted to go back and hear her story. So the next morning I went straight back to the ward. And she was still there thankfully. And I sat down beside her and I said tell me your story. And she did. And the story is this. That she was 22 when all of this began. Up until then she had been completely well. She had plans. She wanted to become a teacher. She was planning her wedding, building a future. And then quite suddenly everything changed. One weekend while she was out looking at wedding venues, she developed what seemed like a simple viral illness. A sore throat and then some flu-like symptoms. Except this wasn't a normal illness. It went on for about a week and kept her in bed during the course of that week. And then when she finally got up something wasn't right. She noticed that she was profoundly dizzy. She couldn't stand up for more than a few minutes without feeling as though she might collapse. Her heart would race the moment she stood up. Her legs would tremble and her breathing would become labored. And at first she thought it was just the aftereffects of being unwell, deconditioning or something that would pass. But days turned into weeks and she felt no better. Instead of improving, she got worse. So she went to see her GP. And they did some basic tests and everything came back normal. She was reassured and told things would improve. But they didn't. And so began a journey. A journey through the health care system. She was initially referred to a neurologist. The neurologist did more tests, found nothing. She was told she had something called a functional neurological disorder and that she would just recover with time. She didn't. She saw a cardiologist for the palpitations that were getting worse. Again, more tests. Again, largely normal apart from a fast heart rate. She was given beta blockers and told they would help. They didn't. And then she saw another doctor for the overwhelming fatigue. She was told it was chronic fatigue syndrome. And then a gastroenterologist for the nausea, the bloating, the constipation. And she was told it's irritable bowel syndrome. And each time she came seeking answers each time she left with a label. A label that explained something. But understood nothing. And gradually what began as an illness became something else. Every door she knocked on gave her a new name for her symptoms. And quietly showed her the way out. And over time the list of diagnoses grew. One label became many. But none of them moved her closer to recovery.

recovery. They described her, but they didn't help her. And alongside those labels came medications. Each specialist added something, another tablet, another treatment, and then sent her on her way. And her symptoms didn't improve, they worsened. Gradually, she lost the ability to stand, and eventually she became wheelchair-bound. Her nausea and bloating became so severe that she could no longer maintain adequate nutrition. And so, a nasogastric tube was inserted. And then she had urinary symptoms. She found that she could no longer void her bladder adequately, and she was advised to begin self-catheterizing. And at 32 years of age, this was the life she was living. And the more she asked for help, the more she encountered one of two responses. Either, "You are too complex. " Or, "There's nothing wrong. This is just anxiety. It's all in your head. " And in between those two positions, there was no space for her to be understood. And then she began to lose pieces of her life. She had to give up her job. She had to stop driving. Her partner, who she had planned this wedding with, when she became ill, had now become her carer. He helped her wash. He helped her get to the bathroom. And she could no longer see her friends, and over time even her closest friends drifted away. And she began to realize that the future she'd imagined for herself was no longer certain. She even started to lose hope that she might one day become a mother. And then there was the feeding tube. As the number of medications increased, this feeding tube would often block. And when it blocked, she could no longer take her medications, she could not receive nutrition, so she would have to go to the emergency department. And with each visit, she felt something changed, not just in her body, but in how she was seen. And she began to sense a growing impatience, and at times something even closer to hostility. And gradually, she started realizing she was slowly becoming invisible. So, listening to Jane that day had a profound impact on me. I didn't yet know how to help her, but for the first time, I felt a deep sense of empathy, that something that I hadn't experienced before. This was a young woman who had seen her dreams slowly dismantled, her sense of identity stripped from her, and her spirit worn down. So, that day I thought I had to try and help in some way, and I arranged for her care to be transferred under me, so she would remain under one doctor rather than having to go to a variety of different doctors. And she remains under my care to this day. And over the years, I started to learn not just from textbooks, from but from her. And the more I listened, the more I started to recognize the same patterns in other patients, patients with long lists of diagnoses, no clear answers. And gradually, those patients became many. And I now have about 4,000 patients like Jane. And I've heard all their stories, and they're all remarkably similar. Different names, different lives, but the same experience of not being seen. It is only at this point that it feels right to talk about the science, because without understanding the person, the science can feel incomplete. What is interesting is that this condition we now recognize as POTS is not new. The first descriptions hark back to 1871, during the American Civil War, and there was a physician by the name of DaCosta, who observed a pattern. And this pattern, the pattern he described is remarkably familiar. It's so basically, what would happen is they would recruit young men. Uh they would draw them into the war from civilian life. And these young men would be subjected to prolonged physical stress. Uh marching for hours, sometimes up to 20 miles a day, with poor nutrition, little water, and harsh environmental conditions. And then many of them would fall ill. Um a viral illness, sometimes gastrointestinal symptoms, profound exhaustion, and they would then collapse. And they would fall out of line and be

admitted to hospital. And over time, they would recover from that infection, but when they returned to marching, something had changed. They could no longer keep pace. They would become breathless, fatigued, and DaCosta mentioned that their heart rates would race. be very high. And — [clears throat] — this was a particularly noticeable thing when they changed posture when they were upright. And when you hear that description, you realize that we've probably been seeing this condition for a very long time. What is POTS? POTS stands for postural orthostatic tachycardia syndrome. This is a At one level, this is simply a description. It's uh characterized by chronically abnormal hemodynamic high heart rate uh on standing up and disabling symptoms that have gone on for more than 3 months. I said, one level it's simply a description, a description of what happens when a person stands up. They stand up, their heart rate goes fast, they don't like it, and we've termed that POTS, postural orthostatic tachycardia syndrome. — [snorts] — But if we're honest, that description only tells us a very small part of the story. It tells us what we can measure, but not what the patient experiences. And sometimes I worry that by calling it a diagnosis, we're risking giving the impression that we fully understand it, when in reality, we're often just describing a single physiological response, with fast heart rate on standing. But as we've seen with Jane, and in Jane's story, this is not a condition that is confined to standing. So, this is one of the fundamental points to mention. This is not just about standing. It affects multiple systems. It is not just about a fast heart rate. being stood up and having a fast heart rate. It affects function. It affects almost every system in the body. And it affects identity, and it affects quality of life. So, perhaps POTS is not so much an explanation, but very much a starting point. Of course, when you are faced with any condition, you go back to the textbooks and say, "What has been written about this condition? " And when we read about POTS in the textbooks, there are some criteria that have been laid out uh to allow us to make the diagnosis. And these criteria include a demonstration of an excessively fast heart rate when a person is standing. And this rise in heart rate is meant to be sustained, so it stays there. The heart rate goes up and stays there. And it is usually diagnosed in the absence of a fall in blood pressure. But these are textbook definitions. A rise in heart rate of more than 30 beats per minute. And these definitions are helpful, they give us a framework, they give us consistency. They allow us to study the condition, but it is important to remember that these definitions are man-made. They're attempts to draw boundaries around something which is perhaps far more complex, and as with all such boundaries, and they're never entirely black and white. And some patients will meet these criteria perfectly, others will fall just short. And yet, their lived experience is no less real. So, if we focus only on whether a number has been reached, which is what most of mainstream medicine does, we risk missing the person in front of us. And I guess it's important, because what matters to the patient is not whether their heart rate rises by 28 or 32 beats per minute, but whether they can stand, live, and function. So, I just wanted to show you perhaps it'll be more helpful for you to see exactly what happens here. So, I'm going to show you what happens to my heart rate when I stand up. Sorry. This is mine. On the left, this is me lying on the bed. Uh you will see — up. When I stand up, you can see my heart rate goes up from 71, and it goes up, as you can see, to about 91. So, it goes up by 20 beats per minute when I stand up. On the other side, I have a patient who has POTS. And you can see their heart rate when they stand up is 73. But as they stand up

the heart rate goes higher and higher. And it keeps going on. And so you can see a very rapid rise, which is quite dramatic. This person's heart rate has gone up to 124 and actually if you watch the video long enough, it'll go up even above 130. And more importantly, it doesn't settle. And this is the part we measured, the number, the heart rate. But what we don't see is what that feels like. To have a heart rate that high when you're standing up doing nothing. Uh the patient is dizzy. The patient feels exceptionally fatigued. There's a sense of instability. So this graph is um only the surface of a much deeper experience. I'm going to stop that and then we'll I can see. Um okay. And so when we think about POTS, it's tempting to think of it as a cardiac condition. But you know, although there are cardiac symptoms such as palpitations, lightheadedness, chest discomfort, breathlessness that is only part of the picture because this is not just about the heart. Um patients will often describe a bunch of other symptoms affecting multiple systems simultaneously often. Dizziness, fatigue cognitive difficulties, what patients call brain fog gut symptoms, bloating, nausea, altered bowel habits, urinary symptoms. They can sleep for 10 hours and wake up like they haven't slept at all. So when you step back and look at all of this together, you begin to realize that this is perhaps not a collection of unrelated symptoms but a single process manifesting in different ways. And this is exactly what we'd seen with Jane. Different labels for different symptoms but perhaps one underlying story. So perhaps the problem was never that she had too many diagnoses but that we hadn't yet found the right framework. And so our understanding of this condition has evolved. But initially it was felt that because these people were worse when they were upright the most likely mechanism would be blood pooling in the lower limbs. And many of these patients will show you what their legs look like when they stand up and you can see this kind of bluish dark red bluish purplish coloration of the legs, which is due to pooling of blood in the legs. So when a healthy person stands up, the body will automatically redistribute blood and our blood vessels tighten and keep the blood up going to the brain. But in patients with dysautonomia or POTS, the system doesn't seem to work as effectively and the blood collects in the legs. And this is what we call acrocyanosis. And when the blood pools like this, less of it returns to the heart and therefore less reaches the brain. And that is what drives the symptoms, dizziness, lightheadedness, fatigue. Um and the body tries to compensate by increasing the heart rate to try and get more blood moving up to the brain because there's this shortage of blood because some is sitting in the legs. So that may well be the reason why we see the heart rate go as fast as it does. Perhaps the body's attempt to deal with the problem. Uh but Sorry, I'll go to the next one. But I think blood pooling is perhaps only one part of the story because blood pooling explains why they feel worse when they're upright. But we've already discussed the fact that these patients even have many symptoms when they're not upright like the fatigue, the lack of refreshing sleep, the brain fog, etc. And what we're perhaps beginning to understand that this may be part of something broader. An imbalance between the autonomic nervous system meaning that balance between our flight and fight responses and our rest and digest responses. So if you think about all these symptoms, they're all to do with too much flight and fight, too little rest and digest. These people are always simultaneously wired and tired. And therefore a much better term to describe this constellation of symptoms is a dysautonomia, not POTS because POTS only refers to the heart rate and the standing. Whereas a dysautonomia does go

some way in trying to explain what may be going on. The system that is meant to regulate our heart rate, blood pressure, circulation, digestion, all the things that we don't consciously think about becomes dysregulated. And in essence, it's like having too much adrenaline in you all the time. And because adrenaline affects everything, uh the symptoms are not confined to one organ. They're widespread. And that is why we see cardiac symptoms, the fast heart rate, etc. alongside gastric symptoms because when we are in high adrenaline states, it affects our gut and it affects um our thinking and hence the neurological symptoms. And it affects our urinary tract sleep and it affects our breathing. So these are not separate problems but different expressions of the same underlying imbalance. Uh and our understanding is therefore evolving from thinking about a condition like this in purely hemodynamic terms to recognizing it as a disorder of autonomic regulation. And that shift, I think, changes how we start seeing these patients. And when we look at these patients, we often see long lists of comorbidities. Chronic fatigue syndrome, irritable bowel syndrome, ADHD. And I know you have a talk on this from another speaker during the course of this conference. Anxiety, functional neurological disorder, chronic pain syndrome, chronic urinary retention, chronic migraine, dysfunctional breathing, microvascular angina, inappropriate sinus tachycardia. And you can see that these list these diagnoses I understand that they all have a rationale but what they're doing is they're trying to describe a particular symptom or a particular presentation. But it is worth asking how helpful they really are because in many cases these labels don't necessarily move the patient forward. They don't provide us with an understanding of what may be going on. And sometimes, unfortunately, they may even hold the patient back because once a label is applied it begins to shape how the patient is seen by the next clinician and the one after that. And over time and over a period of time, a narrative starts emerging. And the narrative can be very difficult to challenge. So why does this happen? And the honest answer is we don't fully know. But there are patterns that begin to emerge. Uh in many patients, this seems to represent an unmasking of an underlying genetic vulnerability. So by far and away the most patients with this will eventually be diagnosed with a condition called joint hypermobility syndrome or belong to the hypermobility spectrum disorders where our connective tissue is more lax. Our joints are more lax. Our blood vessels are more lax. And if the blood vessels themselves are more lax or more compliant, they're more likely to dilate and accommodate blood, which can then pool in the legs. And so the system [clears throat] becomes more vulnerable to dysregulation. And often there is a trigger. So patients are born with a genetic vulnerability. They function fine. Then they get an illness. And that in the old days used to be glandular fever. And now in modern times, it's COVID. And after this everything goes haywire and the patient starts manifesting these symptoms. And then they get given a name. So now long COVID, much of long COVID is autonomic dysfunction, which has been unmasked in vulnerable people, genetically vulnerable people. Um who then start manifesting symptoms of autonomic imbalance. So rather than thinking of this as something that appears out of nowhere, it may be helpful to think of it as a predisposition that has been unmasked, you know. Uh and that perspective again changes how we see the patient. The How do we make the diagnosis? The diagnosis is um you know, there are it's usually clinical. There are, of course, tests you can do. The tests are active stand test or tilt test where you can actually measure the heart rate when the patient is strapped to a bed, raise the bed upright, and then see what happens to their heart rates to try and detect this excessive rise in heart rate. And these tests are helpful, but in my

experience, the diagnosis often begins somewhere else. And it begins with listening. With choosing to actually believe the patient. Because patients will often describe the symptoms. They'll say, "I feel dizzy on standing. I have palpitations. I have fatigue, a sense that my body is not responding the way it should. " And that description is usually the most important clue we have. The tests can help us. They can support the diagnosis. And they can give us objective confirmation. But they are not the diagnosis. And importantly, if the tests are not entirely confirmatory, that should not in itself dissuade us. Because the absence of a clear result does not mean the absence of disease. And if we wait only for the numbers, then we may miss the story. And it is the story that often tells us the truth. How do we manage this condition? There are treatments, and I'll go through those. There are lifestyle changes that the patient can make. Increasing the amount of blood volume so that there is more blood to go around the body, and that can be done through increasing fluid intake, increasing salt, wearing compression garments, improving one's physical conditioning. But in my experience, some of the most important interventions don't come in a prescription. They come through how we engage with the patient. Uh compassion and listening, really listening, has a therapeutic value in itself. Because for many of these patients, this may be the first time that someone has taken their symptoms seriously. The first time they felt believed, validated. And that sense of validation can be profoundly important, not just emotionally, but in how they begin to understand their own illness. And then, of course, there is advocacy, you know, being the person who is in their corner, who helps them navigate a system that they may have struggled with for years. Sometimes, what the patient needs most is not another test, but someone who's just willing to stand with them. And that in itself can be transformative. So, in terms of lifestyle changes, with the best will in the world, lifestyle changes can make a 5 to 10% improvement. Um and we normally say you want to have at least 3 L water, 6 to 10 g of salt a day, supplement with electrolytes, try a low-carbohydrate diet, sleep hygiene, wearing compression garments, and conditioning exercises. And in terms of medications, they can medications I mean, they don't you can't cure this condition because it's a genetic vulnerability that has been unmasked, but you can alleviate symptoms, thereby improving quality of life. And sometimes, treatments can be life-changing for patients. Uh the problem we have with this condition is we find ourselves in a difficult position. As clinicians, we're kind of rightly guided by evidence. We look for guidelines, randomized trials, clear recommendations to guide us as how to as to how we should practice. Uh but with conditions like this, the evidence base is still evolving. So, we're left in a place of uncertainty. And in that space, we have a choice to do nothing, wait for some evidence to come along, or to act thoughtfully, carefully, and in partnership with the patient. And the problem is if we wait for perfect evidence, some patients may continue to lose parts of their lives. And that is not a neutral decision. It's also worth remembering that evidence does not appear in isolation. It's built on experience, on careful observation, on trying treatments, on finding out what helps, and then sharing that knowledge. So, sometimes progress requires a degree of clinical courage, not recklessness, but measured courage, which is guided by physiology, making careful observations, and listening to the patient's feedback. So, during the course of working with these people, I was able to establish good rapport with them. And I said, "Look, you know, we don't have anything that is out there. There's no document that is telling us exactly how to manage you. But if your heart rate is a problem for you, let's lower the heart rate and see what happens. And if the blood's not getting around, let's see if we can give you medications that can make your blood vessels squeeze or help you retain more water. And if you don't feel rested and

digested, then let's try these medications which can act as vagal enhancers like pyridostigmine. And if you're in pain, let's try gentle anti-inflammatories that can relieve your pain. " So, trying to tackle their symptoms, their most troublesome symptoms, um it can be very helpful. And many of these medications are certainly not harmful. So, if the patient benefits, they come back and they say, "Thank you. " And if they don't benefit, they still come back and say, "Thank you for trying. " And I think it's really important that these patients have someone fighting their corner. So, patient advocacy is so important here. And I have in the past, you know, been able to help patients get uh PIP payments and blue disability badges. And it's very important for doctors to liaise with employers and schools and patients' families to make them aware that this is a real thing. Because often, these patients not only have to struggle with disbelief from the medical fraternity, but also from their own families. So, it's it can be very enriching for a doctor to contribute in different phases of this person's life. I just wanted to share with you some feedback, reflection from one of my patients. This was another young woman, not very different from Jane. I'm just going to admit a few more people. Uh not very different from Jane, and she told me that just a year ago, she'd reach a point where she no longer wanted to go on. She was thinking of taking her own life. And a year later, she found herself in a very different place. And she says, "I'm now looking forward to watching my daughters grow up. " And that is quite a journey. And it reminds me that even small shifts in how we understand and support these patients can have very profound effects. So, finally, I'd like to come back to Jane and tell you what happened to Jane. Thankfully, Jane is doing much better now. She is now a mother, something she once never thought would be possible. She's able to walk unaided around her home and in her garden. She can prepare meals, and she can shower independently, and she can work 2 days a week from home. And she loves what she does. And the one most wonderful thing is that she has a sense of identity again. And perhaps more importantly, that DNR form, the form that started this journey, is no longer there. It's been torn up and left in the past. One day, Jane came to see me just to say thank you. She said, "Dr. Gupta, I can see the light now. It feels like a prisoner has been set free. " And for many years, I congratulated myself that I had set that prisoner free. But as I look back now, I realize something very different. That prisoner was me. Because it was only when I accepted that there is so much life that we cannot see, light I truly began to understand the patients in front of me. Thank you very much. Well, I Yeah, you need to unmute yourself this time. I'm sorry. I'm happy to take any questions. — [sighs] — How do most people feel when they have POTS? Alone. Invisible. That is how they feel mentally. Physically, they feel almost like as if you've just had a car accident, and you've come got out to speak to the person that has hit you. That's how they feel all the time. Their legs shake, their heart races, they're breathing hard, their mind is cloudy. Their stomach is in knots, and they want to sit down because they

can't bear being stood up for a prolonged period of time. And then they say, "I just wish I could get some sleep. I sit down. I wish I could have a glass of water. " But they can't because that is what adrenaline does. It is very rare for someone to just come out of a car accident and just go to sleep. This is what happens to these patients on a daily basis. How long does it take to diagnose this? An average of 7 years. 7 years? Oh. At least. Oh my god. Um I would like to personally thank Dr. Sanjay Gupta. I don't have pots, but I did trigger a dysautonomia after COVID um vaccine. So, I can relate with many things you said, and I'm more than thankful that you were here today. Oh. Thank you.