Webinar 2 of 5
Pediatric Orthostatic Intolerance: A Focus on Management by Dr. Peter Rowe
Dr. Peter Rowe Presentation: Pediatric OI
[Whitney Fox]: Hello, and welcome to the second webinar in our medical education series on pediatric Myalgic Encephalomyelitis, also known as ME or ME/CFS, with Dr. Peter Rowe. This webinar will focus on Orthostatic Intolerance, or OI, in pediatric ME.
ME is a multi-system neuroimmune disease that profoundly limits activity required for work and daily living. The symptoms of OI can be challenging to explain and understand. Some people have had OI since they were quite young. For others, it developed after an infectious trigger or other physical stressor. This webinar will define OI, as well as explain how to diagnose and treat it for people with pediatric ME. It will also include a panel discussion with adults who experienced pediatric onset of Orthostatic Intolerance.
In our first webinar, Dr. Rowe provided a history of ME, reviewed the diagnostic algorithm, and provided evidence-based treatment options. A link to the first webinar will be provided in the description below. Dr. Rowe is a professor of pediatrics at the Johns Hopkins University School of Medicine and the director for the Chronic Fatigue Clinic at the Johns Hopkins Children's Center. Thank you, Dr. Rowe for your decades of research, care, and education on ME/CFS. Welcome Dr. Peter Rowe.
[Slide 1]
[1:31] [Dr. Peter Rowe]: Well, thank you, Whitney and Bridget and Denise, for the invitation to do another ME Action webinar, this time on pediatric Orthostatic Intolerance. And we're going to focus on practical things that people can do. So we're going to focus on management issues. But I want people to be aware that-- sorry -- that this is becoming a much bigger problem. It's always been the most treatable aspect, in our view, of how we address symptoms and function in those with ME/CFS. But it's turning out to have been increased in prevalence a tremendous amount by the COVID pandemic. So this is also very important in managing patients with Long Covid.
[Slide 2]
[2:20] I don't have any relevant financial relationships with any of the manufacturers of commercial products or medicines discussed in this talk. But I will talk about unapproved commercial products in my presentation, mainly because in pediatrics many of the drugs that we use to treat Orthostatic Intolerance have not been formally approved for those purposes.
[Slide 3]
[2:44] What I want to cover today is first to discuss the clinical features of common Orthostatic Intolerance syndromes, then go on to show you how we usually diagnose Orthostatic Intolerance, and then finally end with some common medications that generalists often use to treat Postural Tachycardia Syndrome or POTS, Neurally Mediated Hypotension or NMH, and other Orthostatic Intolerance syndromes. So that's the overview.
[Slide 4]
[3:16] Let's jump right into the clinical features of the common Orthostatic Intolerance syndromes.
[Slide 5]
[3:23] [Dr. Peter Rowe] So this is a definition we've modified from one of Phillip Low's articles. He's a major autonomic leader from the Mayo Clinic. The term orthostatic means upright. So the term Orthostatic Intolerance refers to a group of clinical conditions in which symptoms worsen with quiet, upright posture. And 1 many of those symptoms, but not all, are improved upon lying down.
Let me emphasize a couple of items there. Every now and then you'll read people, read articles by people who say that the only form of chronic Orthostatic Intolerance is Postural Tachycardia Syndrome. And I'll show you later that that's simply not the case, that there are many other ways of getting the same group of symptoms. The other thing is that when we are talking about upright posture, that involves both sitting and standing. Orthostatic symptoms can come on much more rapidly when you're standing, but they can occur if people are sitting quietly for long periods of time. When we talk about the symptoms improving upon lying down, we're all familiar, I think, with lightheadedness, dizziness, getting immediately better on lying down. But we've learned over the years that fatigue and brain fog, once they've been provoked by upright posture, can persist for a long time. And there was an article about 100 years ago showing that fatigue can be present for up to 48 hours after people have had a faint episode.
[Slide 6]
[4:57] The main challenge that we face as humans when we move from supine to standing positions is that you shift 500 to 750 milliliters of about a five to six liter blood volume into the lower half of the body. And this slide, also from Phillip Low's book on clinical autonomic disorders, shows very nicely that there's a distention of the vessels and higher pressure in the vessels of the legs. It doesn't show quite accurately or as accurately as we would like that actually a lot of blood pools in the abdominal circulation upon standing. And the main challenge we face is how to get some of that blood back up to the heart and brain.
[Slide 7]
[5:39] Initially, when we stand, with that amount of blood shifting to the legs, there's a drop in blood pressure. And because the vessels in the chest and neck are used to being stretched, and the message from that is that there is adequate blood going to the brain, when they're not being stretched, that sets off a nervous system alarm message, sending a signal to the cardiovascular centers in the brainstem to increase the outflow of what's termed sympathetic nerve activity. This is the activity that's part of the fight or flight response, and often leads to both norepinephrine or noradrenaline release from the nerves, and an increase in release of catecholamines, the epinephrine and norepinephrine from the adrenal gland. So with that increase in sympathetic nerve activity, you get about a 10 or 20 beat increase in heart rate. You get a little bit more forceful contraction of the heart. But the biggest change comes from vessel constriction in the legs, shifting that blood back up to the heart and brain. When that doesn't happen well, these are the symptoms of Orthostatic Intolerance.
[Slide 8]
[6:49] And this is a busy slide, so I'm going to break it down into two categories. The symptoms on the left are thought to be due to reductions in cerebral blood flow, in brain blood flow. So that makes sense for things like lightheadedness or fainting. But if you're not getting adequate blood flow to the cortex of the brain, you're also going to have problems with diminished concentration, trouble with word finding, processing problems. You can get headaches as a result of reductions in brain blood flow, blurry vision. And in all forms of Orthostatic Intolerance, you get a higher prevalence of both fatigue and exercise intolerance, and particularly low impact exercise. The symptoms in the right column are thought to be due to a so-called hyperadrenergic response to that reduction in brain blood flow. And this is, again, an excessive sympathetic nervous system response. That can then lead to dyspnea, which is the medical term for shortness of breath. And this looks a little bit like a panic attack when we see it on the tilt table, but it's an attempt to suction more blood back up to the chest when you take a deep breath. You can get chest pain, palpitations, and tremulousness.
These are familiar symptoms to anyone who's had an epinephrine shot for allergies or asthma. But in this case, the epinephrine is being released within the body. It's not being given by a needle. Anxiety is very common, as is excessive sweating or diaphoresis. And one can also see nausea as another symptom of Orthostatic Intolerance. So these symptoms are really throughout multiple systems in the body.
[Slide 9]
[8:35] This slide summarizes what we think is going on in terms of physiology to lead to one of the orthostatic syndromes on the bottom. So most patients with Orthostatic Intolerance, for reasons that are not always clear, have an increased amount of blood that pools in the lower half of the body, possibly due to a defect in the ability of the vessels to constrict properly. They often will have a reduction in the amount of blood in the vascular system, so a reduction in intravascular volume. This is measured using nuclear medicine techniques. It's not done very often at a clinical level, but in research studies, people with Orthostatic Intolerance have about a 10 to 15% reduction in the amount of blood that would be expected based on their body size. When you then put people through a standing test or a tilt test, because of the excessive pooling and the low volume, then you're going to get a marked reduction in brain blood flow. That elicits an exaggerated sympathoadrenal response. And then the type of problem that you get and we can measure can vary. You can have Classical Orthostatic Hypotension on the left, which occurs within the first three minutes upright, Delayed Orthostatic Hypotension after the first three minutes, the Neurally Mediated Hypotension, POTS. And we're recognizing that it's important to pay attention to orthostatic symptoms because you can get a lot of orthostatic problems, even in the face of a normal heart rate and blood pressure response to upright posture. All of the other problems I just mentioned are defined by heart rate and blood pressure measurements, as we'll see in a second.
[Slide 10]
[10:33] [Dr. Peter Rowe] There is a very common problem in pediatrics called Initial Orthostatic Hypotension, where patients will transiently get a big reduction in their systolic blood pressure or diastolic blood pressure within 15 seconds of standing. But this then resolves in most patients. It can occur together with POTS, but those patients would typically have chronic symptoms. If there's no real impairment in function and the patients are just transiently lightheaded when they stand up, that problem does not necessarily need to be treated with medications. We just need to teach those adolescents how to stand up, clenching the muscles of their legs differently.
Orthostatic Hypotension requires a sustained reduction of 20 millimeters of mercury in the systolic pressure or 10 in the diastolic blood pressure, the bottom number, within three minutes of being upright. This is much less common in children, much more common in older adults who have things like Parkinson's Disease or diabetes. But in children, we can see it in two common scenarios, one being when their volume depleted from a gastrointestinal illness or vomiting, and the other that needs to be paid attention to is that we can see Orthostatic Hypotension in adolescents who have an eating disorder and have been overly restrictive in their caloric intake. Delayed Orthostatic Hypotension refers to the same drops of pressure, but it has to occur after the first three minutes upright.
[Slide 11]
[12:14] The next slide shows a graphic display of the Initial Orthostatic Hypotension from a paper by Julian Stewart. This is the person's blood pressure lying flat. The time on the X-axis is in seconds, and this is a person who stands up and then has a plummeting of their blood pressure and a compensatory increase in heart rate. And this slide also shows that this problem mainly occurs with active standing rather than with passive upright tilt table testing, and I'll show you that in a minute.
[Slide 12]
[12:51] Postural Tachycardia Syndrome requires in adolescents a 40-beat or more increase in heart rate. The old definition was written for all ages, but it turns out that the 30-beat per minute value from people 20 and older would over-diagnose a lot of adolescents. So for adolescents, it requires a 40-beat change in heart rate in the first 10 minutes of standing or head up tilt. And it's not just a heart rate change. You have to have chronic orthostatic symptoms like lightheadedness and fatigue and brain fog, and you can't have that drop in blood pressure that is associated with Orthostatic Hypotension.
We've defined Neurally Mediated Hypotension as a more than 25-millimeter mercury drop in blood pressure during standing or head up tilt, often associated at the time of that drop in blood pressure with a reduction in heart rate. One would usually expect an increase in heart rate to compensate for the drop in blood pressure. In this case, you get a very characteristic slowing of the heart rate just at the time when blood pressure is low. We're recognizing that another phenomenon in adolescents and adults is inappropriate sinus tachycardia. Sinus rhythm refers to the typical healthy rhythm of the heart. And so this is the inappropriate tachycardia is coming from the sinus node - the pacemaker of the heart. But for some reason heart rate is above 100 beats per minute at rest. These patients can have similar symptoms to those with POTS, and it can occur either together with, or independent of POTS.
So you can get, in addition to the high heart rate at baseline, a 40-beat increase with standing. And I mentioned low orthostatic tolerance. These are people with a lot of orthostatic symptoms in the absence of heart rate and blood pressure changes. And preliminary evidence from a number of investigators suggests that many of these individuals have reductions in their cerebral blood flow.
[Slide 13]
[15:02] So Neurally Mediated Hypotension is a mouthful. The other term for this is Vasovagal Syncope. Another synonym is Neurocardiogenic Syncope, Vasodepressor Syncope, or Reflex Syncope, more simply. But not all of the patients who have NMH have had fainting in day-to-day life. So we prefer the term "Neurally Mediated Hypotension" to describe this phenomenon.
[Slide 14]
[15:29] This is, at all ages, the most common cause of recurrent fainting or syncope. It's much more common in women, in adolescents, and in those who are at the bottom end of the blood pressure distribution -- so having a lower blood pressure than average. This can be something that is seen following an infection. Often there are other family members who are affected and have recurrent fainting. And in the past, because the routine physical examination and the laboratory tests were normal, many people with this were thought to have a psychosomatic problem. Along came tilt table testing in the late 1980s, and it became very clear that these blood pressure drops were not under conscious control. And so this helped with people being taken much more seriously. It is important to recognize that the drop in blood pressure is not detected unless the upright stress is prolonged.
So in our early studies of ME/CFS patients, people would be symptomatic from an early point of being put upright on a tilt table, but they didn't develop the drop in blood pressure until a median of 29 minutes. So one or two minute tests are not going to pick this up. And as I mentioned, fatigue can be quite common for hours after people have a syncopal episode. And we think that when people are upright, they develop a debt of fatigue that they simply can't sleep off. And they activate this reflex pathway several times a day, leading to some chronicity in the fatigue.
[Slide 15]
[17:11] [Dr. Peter Rowe] This is an example of what happens on the tilt table, where the table has a footboard for weight bearing. The table is brought up to a 70 degree angle. Here's one of our patients who developed a drop in heart rate, or sorry, an increase of nine beats per minute in heart rate, but was immediately lightheaded and pale. And then you can see at the five minute point that the bottom falls out of the systolic blood pressure. It's down below 50. And at that moment, there's a drop in heart rate in black. And this patient had such a rapid onset of a reduction in brain blood flow that they had some tonic-clonic movements or seizure movements that resolved when the table was placed flat again.
[Slide 16]
[17:56] On to Postural Tachycardia Syndrome. This has been a phenomenon that has been described as early as the 1870s, when soldiers after the Civil War who were fatigued and had trouble functioning were said to have Irritable Heart Syndrome or Effort Syndrome. And the great Canadian physician, Sir William Osler, called it Neurocirculatory Asthenia or Neurocirculatory Exhaustion. POTS is much more common, as is NMH, in women. In women, the ratio is about four women for every male that has POTS. We find that it's quite uncommon to see this under the age of 10. And it more commonly begins in early adolescence. The onset can be insidious, meaning without any obvious trigger. But we see it very frequently after some kind of infection. And that's especially true in the COVID era. It can occur after immunization. We don't think immunization is responsible for the vast majority of patients. We think it's just--if it wasn't the immunization, it might be the next cold that person gets. In other words, we think that the people who develop this have some kind of genetic predisposition and the trigger can be a variety of things. Surgery and trauma can lead to the onset of POTS as well. The symptoms can be disabling, and they often are in patients who have ME/CFS -- meet criteria for that condition as well as POTS. I didn't see this, and we certainly didn't recognize it often when I was in training in the 1980s. So we think that there's been a big increase in not only the recognition of POTS, but also the incidence in the last probably 30 years now.
[Slide 17]
[19:59] I mentioned earlier that POTS and NMH are often the most common of these disorders that we see, but they can occur together. So here's somebody who early on in the TILT test had a 44 beat increase in their heart rate in association with lightheadedness, nausea, leg weakness. The person was pale and sweaty. And then they maintained their blood pressure for a while, and suddenly at the 20-minute point you get that plummeting of blood pressure with the same slowing of heart rate that leads to the loss of consciousness.
[Slide 18]
[20:38] The big issue, though, in the genesis of symptoms is the reduction in brain blood flow.
[Slide 19]
[20:50] And so one of the really interesting observations that's come out in the last little while came from Linda Van Campen and Frans Visser. This is a husband and wife cardiology team in the Netherlands. I had met them at a meeting in 2017 and helped with the writing of this paper, but this was all of their data.
[Slide 20]
[21:12] [Dr. Peter Rowe] And I want to show you in their patients; they used a technique where they put a Doppler probe on each internal carotid artery for about 30 seconds and each vertebral artery for 30 seconds. So over the course of about two minutes, they could tell you by adding up the flow through each of those vessels, all of the blood flow getting to the brain. And you can see at the bottom, here's the Doppler waveform of each heart rate, each heartbeat. And this is the person standing. And you don't have to be an ultrasound specialist to notice that there's been a big reduction in the flow through that vessel.
[Slide 21]
[21:50] They applied this to their adult patients with ME/CFS. They've since applied it to pediatric patients as well, showing similar kinds of changes. But look at what happens with healthy people who are kept up on a head up tilt table for 30 minutes. They do get a 7% reduction in their brain blood flow at the end of that half hour. If you take all of the ME/CFS patients, and this was a huge study compared to others that had been published before -- almost the same size as the combined world's literature on ME/CFS and Orthostatic Intolerance -- they showed that 90% of their patients had a significant reduction in brain blood flow. And on average, for the whole group, it was a 26% reduction. When you break that down, the people with POTS had a 29% reduction in brain blood flow. The ones with Delayed Orthostatic Hypotension had a 28%. But the ones who had a normal heart rate and blood pressure, and who you might have been tempted as a physician to say, "Gee, there's nothing wrong. We can't find anything going on here." They nonetheless had a 24% reduction in brain blood flow. So I think it's no wonder that they were tired, lightheaded, and couldn't think well. This was over three times the reduction that a healthy individual would sustain.
[Slide 22]
[23:09] Okay, we've talked a little bit about tilt testing, but let's look at all of the different diagnostic methods. First, for many of us in medicine, comes the history, right? What's going on? And it's important with adolescents to make sure you're not just asking them, "Are they lightheaded when they stand?" Because they don't often recognize what lightheadedness is. And I've had patients for whom we had to ask, "Well, what happens when you stand up?" "Well, I get a head rush." Well, I would view a head rush as the same thing as lightheadedness. We also want to know how long can you stand still before you feel unwell? And it's very important to ask about how people feel in specific settings. So for example, waiting in line or shopping. Many times our patients can only be in the shopping mall for about five minutes before they want to hightail it out of there and go home. And my joke to our trainees is that if you've got an adolescent who can't go to the mall and can't tolerate going to the mall, you've got an organic medical problem until proven otherwise. Other types of prolonged standing, like at a reception, if you're in the chorus or at a religious service, can bring this on. And especially that's the case if the environment is hot. So a hot shower often triggers these symptoms. And our patients are very intolerant of hot baths and saunas. And the summer weather is particularly tough for them to handle unless they're in a really good air-conditioned room. We want to know if they've ever fainted. We also want to know whether they've adopted some kind of postural counter maneuver that reduces the likelihood of orthostatic symptoms. So many of them will study in a reclining position. They'll bring their knees up to their chest, or they'll tuck their feet under them. And another attempt to accommodate these symptoms is to fidget and move around a lot when people are standing.
[Slide 23]
[25:08] I'll show you some of those pictures later. Another thing we see in about three quarters of the patients is that acrocyanosis is common. This is this purple, reddish purple discoloration of the dependent limbs. And this is a picture we took of a college student five minutes into her standing test showing that her blood was pooling excessively in the hands. And that's my hand behind hers as a color contrast. On the right, I've tested for what we call capillary refill. And that's when you press your fingers on the skin. Within three seconds, the blood ought to flow back into the area that you see blanched there. This took about eight seconds in this individual, and it still had not filled in.
[Slide 24]
[25:56] So to test for Orthostatic Intolerance, we often do a standing test. And typically, that's done after a variable amount of time lying flat, usually for about 10 minutes. So you can capture both the Orthostatic Hypotension and the POTS intervals. There are two types of standing tests. One is called passive, and the other is called active. In addition, more formally, in laboratory settings, you can do a head up tilt table test. Or often in research settings, you can do lower body negative pressure. And that's when you put a compartment in the lower half of the body, and you create a vacuum effect in that space.
[Slide 25]
[26:39] [Dr. Peter Rowe] So for the passive standing test, and this is a variant of a paper variation on a theme by Hyatt, who introduced this test back in 1975, this is the test we most commonly use in the clinic. Patients are supine for five minutes. We check their blood pressure and heart rate every minute. Then we have them stand with their feet about six inches apart and away from the wall. And so they're leaning their upper back against the wall. We measure heart rate and blood pressure each minute. And every one or two minutes, we ask them if their symptoms have changed on a zero to 10 scale. So we want to know about fatigue, lightheadedness, brain fog, headaches, nausea, that sort of thing. So this is easily done in a clinical setting and doesn't cost a lot extra.
[Slide 26]
[27:34] An active standing test is done when the person is just, instead of leaning against the wall, they're standing still in the middle of the room.
[Slide 27]
[27:43] Head up tilt testing is shown in the picture on the right. Usually the angle of the table is 70 degrees, and usually for 45 minutes. Some neurology clinics will only test for 10 minutes because they're primarily looking for POTS and Hypotension. Many centers will do a second stage where if you tolerate that first 45 minutes upright, the table comes back down and a medication called isoproterenol is started to help push heart rate up about 20%. And then the table is brought back upright for 15 minutes. The lower body negative pressure, as I mentioned, is mainly used in research settings.
[Slide 28]
[28:26] Well, onto the practical aspects of treatment. This is why we think this is important. This was the response of ME/CFS subjects to open treatment of their Orthostatic Intolerance, and this was a mixed population of adolescents and adults that we published back in 1995. They had an overall sense of well-being that ranged from 0 to 100 on this scale. Zero means that you're dying. 100 is as good as you could imagine feeling. So this was a pretty impaired group. They came in with a wellness score on average of 35. And with open treatment, that is, this was not a blinded trial, but we gave them either Fludrocortisone, Midodrine, beta blockers, or other medications that are used for Orthostatic Intolerance. And by the end of four months, their wellness score was up to 70. This has essentially been a pattern of response we've seen, perhaps a bit better now because we have a couple of new medication options, but this is what we see in our ME/CFS population to this day.
[Slide 29]
[29:33] So when we're talking about treating Orthostatic Intolerance, we want to explain what's going on and demystify the process, make sure people understand that because they feel lightheaded when they stand, that doesn't mean they should be completely in bed. We bring them back every one to two months for medical monitoring and seeing how our medication changes have worked. We want to provide guidance about new treatments that people hear about on the internet. And a big part of what we do is help get accommodations for fluids, extra time on tests, flexible deadlines, that sort of thing in school. A number of our patients who are really impaired need home and hospital schooling or full home tuition.
[Slide 30]
[30:20] [Dr. Peter Rowe] In treating Orthostatic Intolerance, we start with non-pharmacologic, non-drug measures. Then step two is something that we focus on as general physicians that you don't hear about as much from cardiologists, but it's very important to look at other things that could contribute to the Orthostatic Intolerance. I'll show you some of those in a second. When we begin medications, we hope to be able to use a single drug -- monotherapy -- but often we have to use drugs that work on different possible mechanisms of the Orthostatic Intolerance, and I call that rational polytherapy.
[Slide 31]
[30:59] So back to our diagram, remember that the problems occur, the symptoms occur because you've got increased pooling, a low blood volume, and an exaggerated sympathoadrenal response in all of those red boxes.
[Slide 32]
[31:15] So where possible, you want to avoid the factors that can precipitate these issues.
[Slide 33]
[31:20] So the things that cause increased pooling or decreased blood volume include really prolonged sitting or standing. We want people to take breaks, move around, get up from their chair, and find some way of not standing for long periods of time. We want them, where possible, to avoid overly warm environments. We definitely don't want them to restrict their sodium. Many people have heard from medical authorities that everyone should cut down on sodium intake, but that's not the case for this population of patients. They will simply get sicker if you deplete their sodium intake.
We want, where possible, to avoid too much bed rest because that can lead to deconditioning. Varicose veins are a problem, not so much for the adolescent patients, but they can occasionally be a site of blood pooling excessively in the abdomen or the limbs. Higher carbohydrate intake tends to shift more blood to the gut, so our patients often prefer a higher intake of protein and fat, more of a South Beach sort of diet.
Our medications can sometimes cause problems. Things like diuretics usually aren't used in these patients, but you have to be careful that they're not receiving them. Alpha-blockers are drugs like Phenergan and Compazine that are sometimes used for nausea, and antipsychotic drugs will also lower, will also cause increased pooling of blood. People are paying a lot more attention these days to excess histamine release from mast cells as another factor that will lead to increased pooling of blood in the limbs, and it's important to recognize that alcohol can cause not only flushing and a shift of blood to the skin, but also causes us to urinate more. So too much alcohol can be really problematic for these patients, and our young adult patients often say that they are cheap dates because within an intake of only a half a glass of wine will make them feel unwell.
[Slide 34]
[33:31] Things that can increase their catecholamines, their epinephrine and norepinephrine levels, can include any type of stress, any type of exercise, which may be partly why you see Post Exercise Symptom Exacerbation in patients. Pain involves a release of epinephrine, as does hypoglycemia, and we teach our trainees to watch which of their asthmatic patients are really shaky after they have an albuterol puffer. And we often think that if that's the case, they probably also have, in addition to their asthma, Orthostatic Intolerance, and so we switch them from albuterol to a different rescue medication.
[Slide 35]
[34:13] [Dr. Peter Rowe] Another older technique, first published in 1944, is to raise the entire frame, raise the head of the bed at night. This causes the kidney to think that it needs to conserve blood in the circulation and not waste any of that volume into the urine or waste less of it. So patients awaken in the morning with a higher blood volume if they have the head of the bed raised. Notice that this guy has a pillow under his hips as a stopper. Many people will not tolerate this angle, but we often will ask people to try putting two red bricks under the headboard area. Again, raising the entire angle of the bed, not using a wedge pillow just in the top part of the body. The success of this, the efficacy of this has been retested more recently by a Dutch group in 1992.
[Slide 36]
[35:11] Another way to get blood out of the legs and abdomen is to use external compression garments, and so this can involve stockings, and our patients prefer the 20 to 30 millimeter mercury stockings more than the tighter ones, because the tighter ones are tough to get on and off. The area covered matters -- so if you've got waist high stockings, they're better than the thigh high stockings, and those in turn are better than the knee highs. Many adolescents prefer using the body shape or garments like you see on the side here, or abdominal binders which can be put under the t-shirts and aren't very conspicuous.
[Slide 37]
[35:52] Many patients adopt postural counter maneuvers without knowing why, but it's very common for our patients to stand with their legs crossed like you see in this picture, and that individual also has some heels on, which then activates the calf muscles better than standing flat footed, so that can be helpful as well. Squatting, sitting with the knees to the chest, leaning forward when you're sitting, or getting the knees higher than the hips can all be helpful. We want the adolescents to use their leg muscles as a pump to get the blood out of their legs and up to the heart again.
[Slide 38]
[36:29] These are some of the postural counter maneuvers that our patients will adopt, and in the upper right is, you can see that this individual has her leg up, her left leg up on the seat while driving.
[Slide 39]
[36:44] In terms of fluid intake, we want people - at least - to get two liters of fluid intake over the course of the day, and we want them drinking regularly every couple of hours, so that means they'll need access to fluids at school. If they're very somnolent in the beginning of a mononucleosis episode or after COVID, we want the parents to awaken them and not let them sleep more than 12 hours at a time. They can then go back to sleep after they've had something to drink and moved around a bit, and that way, if they sleep for 16 to 18 hours, they're not awakening super dehydrated.
They might benefit from cooling garments in the hot weather. In terms of salt, we want them primarily to add more salt to their foods, increasing that according to taste, and if they don't like the taste of salt, they can supplement their dietary intake with salt tablets or Oral Rehydration Solutions.
[Slide 40]
[37:43] [Dr. Peter Rowe] These are some of the cooling garments that patients can use. This is a neck wrap. This is a cooling beanie, and you see on the bottom one of the cooling vests that are more expensive. Many of these products can be found on sites that provide information for people with Multiple Sclerosis because they often have really big problems with temperature, with heat intolerance.
[Slide 41]
[38:11] In terms of oral rehydration products, these are just three of many, many products that are on the market currently. Trioral has a very high sodium intake, well above what you'd find in Pedialyte or Gatorade, and this follows the World Health Organization Oral Rehydration Solution composition. Ceralyte is a rice-based product. Many people have seen Liquid IV, which has become quite popular in the last few years, all of them providing way higher intake of sodium than you'd get from things like Gatorade.
[Slide 42]
[38:51] Activity is a tough one because we don't want people, if it's at all possible, in bed or sleeping too much. That's ideally we would have them up more during the day, but for the really impaired patients, they have to begin their exercise quite gradually and slowly, and our goal in advancing the exercise and activity is to avoid any Post Exertional Symptom Exacerbation or Post Exertional Malaise. For that reason, we're really wary of rigid advancement schedules for graded exercise. We find that the exercise is usually better tolerated after the Orthostatic Intolerance is treated, and if we are successful in getting blood flow to the brain and better blood flow around the body, we usually don't have to cajole our patients to be more active. They just do that spontaneously. Our experience has been that manual forms of physical therapy can be a bridge to better tolerance of activity and exercise, and this is where a physical therapist works on muscle tightness, spasm, myofascial tightness, and helps with a freer movement of the limbs; and that seems to help when people are ready to begin activity. Where possible, we want people to avoid complete inactivity.
[Slide 43]
[40:19] I mentioned earlier that a big feature of how we address the problems is to treat contributory conditions, and this is a diagram that we published this year on our approach to ME/CFS and Long Covid, where you see that treating the Orthostatic Intolerance is really important, but that there are many things around this hub that can contribute to the Orthostatic Intolerance, one of them being depression, which can be a secondary phenomenon if you just can't function normally.
We mentioned that with the high levels of epinephrine and norepinephrine, you can get anxiety as a very similar kind of physiology, and so this can be a bit of a vicious cycle in people who get lightheaded, feel unwell, start to hyperventilate or are anxious, which then in turn makes them release more epinephrine. A lot of our patients have problems with moving things through the intestinal tract. That's probably a consequence of the high sympathetic tone that they have, because if you are frightened or in a fight or flight mode, the last thing you want to do is stop and go to the bathroom. You really shut down your GI motility, your gastrointestinal movements, when you are in a high sympathetic drive state. 30% of our patients have a delayed reaction to milk protein, and that's manifested by epigastric or upper abdominal pain, reflux, and feeling full easily. We also see a number of patients who have Mast Cell Activation Syndrome, where they have multiple medicines or foods that they don't tolerate, and often this is associated with facial flushing or redness, as well as hives and itching. Allergies are capable of leading to more Orthostatic Intolerance in a similar manner through the release of excessive histamine. Our patients typically will feel worse after any kind of other viral infection.
We are focusing a lot on areas of focal tightness in the limbs and spine, one of them being Thoracic Outlet Syndrome. This is where people have marked symptoms of arm fatigue and tingling when their arms are overhead, and a common way in which that comes out is if adolescents have more hair than I do, they'll feel that when they're shampooing their hair. They often have to break that up into several segments.
Migraines and chronic daily headaches are common. There's a subset of patients who have neuroanatomic causes of their Orthostatic Intolerance with pressure on the brain stem or in the neck, in the cervical region. We've seen Chiari Malformations, Cervical-spinal stenosis, and then in people with a lot of loose connective tissue, they can have Cranio-Cervical Instability or even Tethered Cord, which can lead to urinary symptoms and leg pain. About 60% of our patients have Joint Hypermobility. The most extreme form of that is Ehlers-Danlos Syndrome. We think this is because the vessels are stretchier so that when they stand up, they pool more blood in the lower half of the body. And many people with Ehlers-Danlos Syndrome can also have a variety of Vascular Compression Syndromes, which are shown here. And this includes May-Thurner Syndrome, Median Arcuate Ligament Syndrome, Nutcracker Syndrome, and Eagle Syndrome. And I mentioned before that sweating can be a big problem. Hyperhidrosis refers to excessive sweating, and many of those patients also have Orthostatic Intolerance.
[Slide 44]
[44:12] Among the medications that we start with…
[Slide 45]
[44:14] [Dr. Peter Rowe] Again, back to our diagram that describes the main mechanisms, if you're pooling too much, you want something to cause those vessels to constrict better than they're doing on their own. If you've got a low blood volume, you want to use something that expands the volume. And if you've got an excessive sympathoadrenal response, we want something to block the effect of those catecholamines on the heart.
[44:39] So among the Vasoconstrictor medications are Midodrine, Dexedrine, Methylphenidate, and a number of the other medicines that are used for Attention Deficit Disorder. The antidepressants in the SSRI, which is the Selective Serotonin Reuptake Inhibitor class, or SNRI class, can have a blood pressure and a vasoconstricting effect. Droxidopa is harder to get hold of, and it's often reserved for those older adults with Orthostatic Hypotension.
For volume expansion, the biggest component here is the dietary sodium intake. We occasionally need to give IV Sodium Chloride. Fluidocortisone helps the kidney hang onto the sodium in the diet. Clonidine can be another drug that helps with volume expansion. Oral contraceptive pills can do so for young women who are especially tired at the time of their periods or more lightheaded. And Desmopressin is a drug that reduces urine formation at night. A variety of drugs will help with heart rate modulation, the main category being the Beta-Blocker drugs. But we use a lot of Pyridostigmine Bromide to balance this, and Ivabradine for those who have inappropriate sinus tachycardia.
[Slide 46]
[46:00] One of the questions that comes up is: with this many options, how do you begin the initial treatment? And you can follow either an algorithm approach or an individualized approach. And we don't have any clinical trial data to support which one is better than the other. So most of the advice you'll hear is based on the experience of individual clinicians.
[Slide 47]
[46:25] Here is an algorithm for treating POTS. And you'll see at the top that these authors talk about hydration, salt intake, stockings, and so on. At the bottom, you see that they're recommending for POTS that everybody get as a first-line agent either a Beta-Blocker or Midodrine. And whichever one they didn't get, they'll get as the second line. Then the drugs like Fludrocortisone are second line. Their third line are the antidepressants. And they would say they rarely use these others.
[Slide 48]
[47:00] We prefer an individualized approach where, if you look at the person's systolic blood pressure and it's quite low, we want to give them something to raise blood pressure like Fludrocortisone or Midodrine. On the other hand, if they have an increase in their heart rate at baseline, a Beta-Blocker is good, and ivabradine if the heart rate is above 100. But we always have other clinical clues.
So we had a boy once who came in with a salt shaker on his bed stand. He would shake salt onto his palm and lick it. Well, we wanted to give him something that would help him reabsorb that sodium and not urinate it out, and that was Fludrocortisone. Beta-Blocker drugs can be good for prevention of headaches. So if you've got somebody with Orthostatic Intolerance and a headache, a Beta-Blocker is not a bad choice. For young women with a lot of bad menstrual period pain or increased fatigue at the time of their periods, birth control pills or Depo-Provera are often helpful. On the other hand, if they have low mood or anxiety, you could use one of the antidepressants as a first-line agent, one of which is Cymbalta or Duloxetine that's been shown to be helpful if people have a lot of myalgias or muscle pain, because it's been demonstrated to be effective in people with Fibromyalgia. In those with hypermobility of the joints where we think their vessels are just too stretchy, we want to give them something that is a vasoconstrictor like a stimulant medication or Midodrine. And then for patients who have excessive sweat, Clonidine and Guanfacine can be quite helpful.
[Slide 49]
[48:38] So hopefully I've given you the sense that this management of this kind of problem requires a lot of attention by the patients and by us as practitioners to the factors that provoke their symptoms, often requires a willingness to try several medications before we find a good fit and requires a realization that the medicines often can treat symptoms but don't necessarily cure the underlying condition. This isn't too different than asthma where we don't set out to cure the problem, we set out to make that person functional again. And so management of Orthostatic Intolerance is one part of a comprehensive program of care that often needs to rely on the help of our physical therapy colleagues.
[Slide 50]
[49:24] [Dr. Peter Rowe] I wanted to list a couple of references. We have a book that will be coming out from the Johns Hopkins University Press later this month called "Living Well with Orthostatic Intolerance" and that can be ordered online from the Johns Hopkins University Press. The rest are formal medical papers that talk about these different types of Orthostatic Intolerance and at the bottom is the paper that I showed you a slide on the cerebral blood flow from Dr. van Campen and Visser.
[Slide 51]
[49:54] There are a number of web resources. The Solve ME/CFS Initiative has a number of talks on their site. I did a longer talk that specifies the medications we use. On YouTube, you have to type in the term Dr. Peter Rowe because if you just type in the name you'll get the director of the old movie, "The Little Shop of Horrors". I definitely wasn't involved in that. Dysautonomia International has a number of talks and a very good conference each year -- as does the Ehlers-Danlos Society. So you can find a lot of good talks on these resources.
[Slide 52]
[50:32] Our work at Johns Hopkins has been largely made possible by philanthropic donations including the endowed professorship from the Sunshine Natural Wellbeing Foundation. A number of families have been instrumental in providing further endowment funds and we've had excellent help from our research coordinators, nurses, summer students and others who have done fundraisers. So I’m going to end there.
We will be doing the next talk focusing more on the manifestations of connective tissue laxity of the Joint Hypermobility and Ehlers-Danlos Syndrome. I want people to understand how to address those problems. And then we'll have two other talks in the future, one on Biomechanical problems like the Thoracic Outlet Syndrome that I mentioned and on the Neuroanatomic problems like you see on that wheel and spoke diagram. So thank you very much for your attention and we will see you again soon.
[51:34] [Whitney Fox]: Thank you so much, Dr. Rowe. We will provide a link to resources cited by Dr. Rowe in the description. ME Action Maryland's objective is to educate and equip medical professionals to recognize, diagnose, treat and support people with ME and comorbidities. My name is Whitney Fox and I will be facilitating today's discussion. I am co-chair and co-founder of ME Action Maryland, which was founded in 2020 with Melinda Lipscomb. Along with co-chair Bridget Collins, we lead monthly advocacy calls and monthly support calls that are open to all, not just Maryland residents. We welcome people living with ME, Long Covid and associated diseases, caregivers and healthy allies. This panel contains discussion that may be difficult for some viewers. Important warnings include stereotypes, discrimination, ableism, sexism, racism, and childhood illness. ME affects people of all ages, genders, races, and socioeconomic backgrounds, but is more frequently reported in women. Black, Indigenous and People of Color are less likely to be diagnosed even though all racial backgrounds show comparable prevalence. The prevalence among transgender and gender diverse people has not yet been thoroughly studied. People with ME and OI experience a wide variety of symptoms severity, and this panel is not representative of all experiences. Now we will transition to the lived experience of people with OI.
We have two speakers today. Joshua Weitzman from Potomac, Maryland, now living in Los Angeles, was diagnosed with OI at a top ranked children's hospital as a late stage adolescent. His symptoms worsened for a year before seeing a clinician with a private practice who offered treatment options. Joshua then saw a top ME specialist who diagnosed ME and explained PEM. Cynthia Adinig from Northern Virginia was recently diagnosed with POTS, ME, and Long COVID with the help of a supportive primary care provider and shares what it was like to be a child with undiagnosed OI. We will discuss their individual journeys and unique experiences with OI, ME, and comorbidities. Welcome Cynthia and Joshua. Thank you for joining us today.
[53:53] [Cynthia Adinig]: Hi, my name is Cynthia Adinig. I'm from Northern Virginia, 39 years old now. wow. I am four years into having Long Covid, had very disabling symptoms this entire time, and I have been diagnosed with ME/CFS, MCAS, so many things, esophageal spasms, esophageal tear, POTS. And now, I can very much see that I have undiagnosed EDS and probably something autoimmune in there. While it sucks to be a part of this multi-acronym club, it actually, it explained so much of my childhood that was confusing to me and what I thought was Cynthia-esque quirks were actually undiagnosed illnesses that was very prominent in my family and things that were signs that should have been checked out by a doctor that wasn't.
[55:12] [Joshua Weitzman]: I am diagnosed with both ME/CFS and POTS. And it was kind of a sudden onset. I have had Crohn's disease since I was nine. And so I was used to chronic illness because of the immunosuppression. I had a lot of colds and viruses, but I was always able to bounce back. And then in 2021, I had an upper respiratory infection, which we, looking back, think might have been COVID. And in the following weeks, I just developed Orthostatic Intolerance. And then in 2022 is when I got the official diagnosis of Dysautonomia and ME/CFS. And since then, I had to take, I study in music business at NYU, and I had to take medical leave and it's been pretty disabling.
[56:09] [Whitney Fox]: When was the first time you remember experiencing OI? How did it feel? And what were you doing when you realized that this is unusual, and maybe it's harder than it should be?
[56:22] [Cynthia Adinig]: Oh, man. So I was teased a lot for being the slow one in the family. My dad would say that I'm slower than molasses in January. That's what I was told my whole young life, which is true. But I mean, but not, I'm glad that I was like, I recognized that the simple walk to the park that my siblings were doing. By the time we got there, I had to go sit on the swing and rest and they would just walk to the park and go run around play basketball. I recognized there I was doing some pacing and resting. And I would be there by myself for like 30 minutes. And then I would meander on over to my siblings and do a little bit of shooting around with them and then like my arms would be tired. And the time that really hit me that, like, this is just my tribe and how this works, was when I was at the Dysautonomia International Conference a year ago, and I was like, I like tripped over air. It felt so good that like, no one turned around, no one was surprised. Talking to others that you know, to others those three days and they're like, you did it the kid you were flipping upside down against the wall like all the time or like, oh, yeah, I remember like, you were the one that was licking salt after you know, after dinner, you just I would just chase throw the salt in my hand and like make it lick it. I'm like, Oh my goodness, we were just figuring out ways to, like, stabilize us.
[57:58] [Joshua Weitzman]: So I didn't realize it at the time that it was Orthostatic Intolerance. But following you know, the infection. Every time I stood up, I would feel so dizzy. When I would walk around, I was just out of breath. And, like, felt these like palpitations. At one point, when I actually fainted. And so we went to do, I think, three emergency rooms in one week. And it was at one of those emergency rooms that just walking to the bathroom felt like a marathon. And that's something that I wasn't used to feeling that weak, because I've been admitted in the hospital a bunch of times with Crohn's. And I had no issues just walking to the bathroom. When I was standing up, my pulse was 190. When I lied back down, it went back all the way to 60.
[58:47] [Whitney Fox]: Can you talk some more about the difference between the symptom of chronic fatigue that you felt with Crohn's versus how that's different now that you have ME and LI?
[59:00] [Joshua Weitzman]: That's a huge difference. So dealing with Crohn's, fatigue is one of the big symptoms of Crohn's. And I've always just dealt with fatigue. It's always been one of my big complaints. But by that I mean, when my Crohn's was in remission, I felt healthy. And then throughout the day, I would begin to feel exhausted. And then I would sleep and wake up refreshed. And my battery level is back to, you know, if someone healthy is like 100%, mine was always kind of like at 70, 80%. So that's kind of chronic fatigue. You just deal with fatigue every day. It limits some of my activities. I take a nap, and I'm kind of able to do things again, and my baseline feels healthy. Since I've had ME/CFS and Orthostatic Intolerance, there has not been one day where I felt healthy. Not one second, not one moment. My baseline is that exhaustion feeling. It's that feeling of I didn't sleep for like five days straight, I was cramming my exams. That's like my baseline every day. My battery life is like 2%. Any activity beyond that goes into the negative range. And once you get into the negatives, like I'm in debt, so to speak. That's when I get those Post Exertional Malaise symptoms.
[1:00:28] [Whitney Fox]: I know many of us have faced challenges with accessing diagnosis. Who noticed your symptoms when you were a child?
[1:00:38] [Cynthia Adinig]: Yeah, absolutely no one. No one noticed it at all. And I think part of it is because I did figure out ways to maneuver around it and like changes to function. And also, I really heavily embraced that I was a nerd. So I didn't really try to push myself to exercise to for it to be noticed. Everyone knows that Cynthia was napping after high school every day. So no, people didn't notice it. And I was just considered the quirky nerd. And because I was a quirky nerd with all these nerdy achievements, no one really was like, oh, she's actually slow and sickly.
[1:01:23] [Joshua Weitzman]: My parents and I, with all the syncope, some of the cardiologists I saw made the connection that I have Orthostatic Intolerance. The pediatric emergency room, and they mentioned POTS to me. That's when it kind of all made a little bit of sense.
[1:01:42] [Whitney Fox]: Did you find any difficulty when you were going to doctors due to your age?
[1:01:50] [Joshua Weitzman]: With the POTS cardiologist, they see a lot of young people my age who like, you know, get COVID or Mono. And then they're dealing with like full blown, you know, Orthostatic Intolerance. The issue is at the beginning, I went to a lot of cardiologists who just see people with, you know, heart disease and things like that. Some of these physicians were kind of dismissive because they said, oh, well, you don't have heart disease. Yeah, you're syncope and you're having this orthostatic stuff. But we did an echo. That's fine. We did a CAT scan. That's fine. You're good to go. So I had one physician who really validated my POTS diagnosis. But he didn't really say I had Chronic Fatigue Syndrome when I was describing like, hey, like when I do too much, I get this payback. He kind of, you know, insinuated that I wasn't presenting like middle aged women. And I'm a man. So in his mind, because of that, the diagnosis doesn't make sense. It wasn't until 2022 that I saw a physician who was really knowledgeable on things like Mast Cell Activation Syndrome and POTS. And through a Lean Test, that's when I was officially diagnosed.
[1:03:09] [Whitney Fox]: What was that experience like having the lean test?
[1:03:13] [Joshua Weitzman]: Well, one, it made me feel pretty sick. But it was also pretty validating when I did the lean test, because when I stand up and my hands begin to kind of get bluish, and you see all these visual changes. In my head, it was like proving to the clinician that, hey, this is so visible. My feet are getting all red and blood is pooling. As he's asking me questions, the brain fog really kind of kicks in. And so it was really validating, because I still was like second guessing myself. But also it was pretty tough doing that lean test.
[1:03:52] [Whitney Fox]: What was your experience like for the diagnostic process?
[1:03:55] [Cynthia Adinig]: When I was hospitalized for starvation dehydration for my severe MCAS, I was telling the doctors, hey, I think I have Long Covid, pretty sure I have POTS. I need to do a tilt table test or you need to do a format test, standing, sitting test, like I'm going to the doctor, telling them all these things. And I'm sure they're looking at me like, why would you even know? This is so specific. But if I hadn't gotten in there and been like, hey, while I'm here, I'm already here for like five, six days for starvation dehydration. You've got all these IVs in my arms and all this other jazz. You have me hooked up to the heart monitor. Go ahead and do the standing, do whatever you guys have available here so I can get diagnosed. If I hadn't been on top of it, it would have been several more months longer before I was diagnosed. I definitely feel like race plays a huge part as to why the passion for my full recovery is not there. I have a phenomenal primary care provider. She has fought for me every step of the way. She's the reason why I'm diagnosed with ME/CFS. She helped me get diagnosed with POTS. She was seeing that I was fitting all these diagnostic criteria, but the specialist she was sending me to would say, oh, well, yeah, this has merit, but still not wanting to do the official diagnosis. I'm so thankful, it's the reason why I'm able to get accommodations--sometimes--when I go places. And I do credit that as to helping me be as well as I am today. You can see other people of color, especially those in the South, four years in still don't have a Long Covid diagnosis. Four years in, I still don't have a POTS diagnosis. We have a long ways to go in getting regular providers comfortable diagnosing, but also a long way to go in terms of people of color, having someone else fight for their health, even when they don't know something is wrong.
[1:06:00] [Whitney Fox]: What lifestyle changes and accommodations have been the most helpful for managing your OI symptoms?
[1:06:06] [Cynthia Adinig]: I feel like I missed out on so much joy of childhood, because not because of so much external internal pressure to achieve at that level. And it's especially if you're being told that you are below in another area, like physicality, like not being able to open a bag of chips and never, you know, never would have been a jar of pickles. And so what I've done differently is I have, I bought this little grip things. I bought that for my son. So my son can open his own bag of chips. He can open all his jars. He can open all his Gatorades. And so I'm like, I'm like quietly proud when I see him go and get it, like, without prompting and seeing like him be excited about his independence, knowing that I would’ve felt so, like, much of a drain on my family for having to ask. I'm thankful for the lessons learned that I'm not passing down to my son when it comes to accommodations and not making it such a big deal to have them or need them. And that it's not--it's not tied to your value and it's not tied to your identity. It's just, it's no different from meeting glasses. One of the fun things that we did, we laid on the couch and we put up, I put my feet up in the air and lay on the couch that way. It felt amazing. And I have been doing it since I was little. And now I know why. I took that as an opportunity, even though I said I wasn't feeling any symptoms, in to show him what he could do in the future if he were to feel something was not better. And so I had him, you know, sit next to me and prop his legs up. And he's like, actually, that's actually amazing. I was like, I know, right? Like, you don't really realize how much, how much it better it feels because you're so used to it, not feeling great, but you don't know what optimal feels like. Knowing that these illnesses can be, like, dynamic and shift just because he's not experiencing a symptom now doesn't mean he may not experience it later. And I wanted him to know and recognize it for when it was happened to be like, one, it's not going to make him less scared, right. He's going to be like, Oh, that's the thing mommy told me about. And then also he's going to know what to do and be like, okay, double heartbeats. That's probably palpitations, tachycardia, heavy legs. Okay.That's my blood pulling. It was compression socks on. It's just like, Oh, it's just something that can help you if you need it. But I still make sure that he has the language or knows how to translate that into other with other kids or adults or physicians. I try to make it light, fun.
[1:08:58] [Joshua Weitzman]: Lifestyle outside of pharmaceutical stuff. Definitely the fluids. It's at the beginning, like I was doing a lot of hydration, but with not a lot of salt. And then I felt awful. So definitely things like Liquid IV and LMNT is one of them too. Electrolytes in the fluids has made a huge difference. I used to love taking hot showers. Now I take cold, cold showers because the heat, I guess there's like a vasodilation effect and it just really flares up symptoms. Eating small meals. It's funny because even with Crohn's, I used to love eating like, you know, chicken tenders and fries and then have washing it down with, like, a milkshake or ice cream. And once I developed Orthostatic Intolerance and I tried doing that, it was not good. And all the symptoms flared up. And so I realized quickly, I'm going to have to change the way I eat and eat in small meals. Even like simple stuff, like when I'm standing in line to get something, I will sometimes kind of like pace around the line. I started just standing in one place. And I think it's kind of like subconscious that I do that because my body knows like you can't really stay in one place. The blood will kind of begin pooling. I like to sit with like, my legs are crossed often. I just sit in like strange positions.
[1:10:22] [Whitney Fox]: Can you elaborate some more on how your orthostatic symptoms impact your ME symptoms and the other way around as well? Do your ME symptoms impact your orthostatic symptoms? (Joshua Weitzman) Yeah. So they definitely play off each other. And it's really difficult sometimes to tell what is what. If I have a day where I wake up and I just feel so sick, is it because my Orthostatic Intolerance is flared up that day? Or is it because I'm in Post Exertional Malaise? Is that extra brain fog I feel because I'm in PEM? Or is it because my POTS is flaring up? It's frustrating too because the advice I was given by my ME specialist was, you know, one way to pace is to keep track of the pulse, your heart rate, throughout the day. But when you have POTS and you know, it just spikes up to like 150, 160, it's just difficult to pace with that.
[1:11:23] [Cynthia Adinig]: Those are, I feel like, are some of the hardest to differentiate between each other. Thankfully, I got a really good education by who I call the OGs of ME/CFS, who are several decades in, to explain that Post Exertional Malaise is really the hallmark of ME/CFS.
[1:11:49] [Whitney Fox]: What should medical providers know about Orthostatic Intolerance and ME?
1:11:55] [Joshua Weitzman]: I feel like the information with both the ME clinicians and the POTS and Dysautonomia clinicians is, I feel like the information is a little bit siloed. A lot of POTS clinicians I've seen will tell me, you do aerobic exercise, you know, run. They don't really seem to be knowledgeable on, you know, Post Exertional Malaise or some of the symptoms that, you know, people with ME deal with. And then on the ME side, you know, they tell me to pace as much as I can. And so I'm kind of getting just like, you know, conflicting stuff. So I think, you know, if both ME specialists, POTS and Dysautonomia specialists, and physical therapists, if they all could kind of collaborate, that would be great. Unlike Crohn's, which, you know, a lot of physicians seem to know, with POTS, a lot didn't. I just kind of felt like I was on my own. A lot of the things that I found that helped me, I just kind of stumbled upon through trial and stuff. A lot of physicians or clinicians are like, "Oh, I don't treat that. That's too complicated." I want them to know that they can do the simple things and it makes a huge difference. So an emergency room physician sees a patient come in and they have a diagnosis of POTS. You could just give them IV fluids and that can make a huge difference. You know, you don't need to be a specialist to just do simple things that could really help the patient.
[1:13:27] [Cynthia Adinig]: One of the things I like to tell providers is help patients feel comfortable resting and feel like it's an asset to their health. Offer spaces for financial and emotional support to everyone because patients are not going to be forthcoming on that. On a broad scale, instruments, medical instruments and tools are not built for brown skin. I also think providers need to just objectively understand -- and this includes of color -- that they're going to have biases against people of color in terms of trusting them in terms of their health, the seriousness of their health. And it's okay to have those biases. We all have them from the society, that doesn't make us a bad person. You just have to be aware that they exist so that you can counter them when they crop up and make those corrections before it translates into any sort of actions involving a patient. Physicians should know that these are all dynamic. These are not end all, be all, you must have three of the five to fit into this. That's definitely not how it works for people of color. And to ask us to fit into that criteria is exactly why we go so undiagnosed for so long. But also understand that asking a patient to be looking for these things -- before they know to look for these things -- is also how these things get overlooked. Allow space for the patient to, like, recognize that they didn't recognize these symptoms. Allow space for the symptoms that just not traditionally present at all. Allow space for the patient to not have had the level of severity that would cause any visible symptoms. And really go more off of what the individual's baseline is and what the individual is saying is bothering them the most as a guide in the direction you go in.
[1:15:49] [Whitney Fox]: What advice would you give to parents of young children who might be experiencing these symptoms and have trouble communicating it? How to help give their child the language?
[1:16:03] [Cynthia Adinig]: Wow. I would say one, making sure your child feels comfortable with their body and confident with what they're feeling and safe. Because my son knows that I'm going to take whatever he says seriously and really try to make sure that he has the best outcome, he's comfortable coming to me and saying that he has it. And then he knows I'm going to try to figure out what he's trying to say, even if he doesn't have one--These symptoms are so hard to describe sometimes. A lot of kids carry that burden of not being a complainer. I feel like I have an advantage because I experienced it in my own body. It would be so monumentally hard for me if I did not have these exact same illnesses. Because I became so severe that I had to figure it out and piece together what was missing my entire life. I can only imagine how someone who's never felt this in their body has to try to figure out what all is wrong, and then separating that from like normal child development, puberty. Honestly, I just, I asked him a lot of questions. I just go from top to bottom because it affects your whole body. And if you, and again, if you've had these symptoms for a long time, even before a parent recognized it, even they may miss some things that are they are highlighting. So asking specific spots that we know based on research that are the most prone is a great way to figure out if even they're missing highlighting some symptoms that they're having.
[1:17:55] [Whitney Fox]: And those are some really great examples that healthcare professionals could use when communicating with a child and their caregivers in an appointment.
[1:18:05] [Cynthia Adinig]: That's correct, which is exactly why I wrote the guide that says all these things. I wrote a guide of pediatric Long Covid that is through the lens of what parents should be looking at in terms of, I think I split it up with MCAS, Dysautonomia, and ME/CFS.
[1:18:27] [Whitney Fox]: Thank you, Dr. Rowe. Thank you to our panelists and thank you to the volunteer advocates with ME Action Maryland who contributed to this webinar. Also thank you healthcare providers for taking the time to learn about Orthostatic Intolerance in pediatric ME. If you have questions for Dr. Rowe, please email us at [Maryland@MEAction.net]. We appreciate your participation and hope you will join us for our next webinar on the manifestations of connective tissue laxity in pediatric ME/CFS.
