Upright Activity and Exercise Intolerance: Critical Concepts in the Evaluation of Chronic Fatigue

it’s a pleasure to be here thank you very much for having me I am a firm believer in all kinds of crosstalk and medicine I think it’s really important that we share and today I want to talk to you about some things I’ve learned and while you’re looking at my disclosures I’ll just give you a little background I’m a general internal medicine specialist and I practiced general internal medicine for about a year but during that time became very interested in all the patients that we couldn’t explain so it I framed a lot of it around the case definitions of fibromyalgia and chronic fatigue syndrome but of course we’ve learned more and more and more over the years in 2000 I decided to leave my primary care practice and just set up a fatigue consultation clinic to learn more about it so I did that clinic for a long time we it’s been almost 20 years now that I’ve been doing it become a specialist in this area and it’s been really amazing so I want to share with you some things that I think are really important for all clinicians to know about fatigue and also some insights about how we now diagnose myalgic encephalomyelitis or mecfs so I’m hoping when we get done you’ll be able to and you know that we’ll do better job at assessing post-exertional malaise particularly as defined in the new criteria for mecfs and to assess impairment of function and I’m going to if assessing function is not easy so I’ll share with you some things that we’ve developed in our clinic to make it a little bit more efficient and then most important I think is to learn how to assess for orthostatic intolerance syndromes which are a part of why people have abnormal function and post exertional malaise so I’m going to share with you a case and I’m sure our cases probably all look pretty close there are lots of ways people present but this is a woman who came to me when she was 30 in her history we always go back and sort of take a lifelong medical history she was a little underweight but she was active in childhood as a teenager she had some little constellation of problems we might be familiar with she had anxiety she had migraines some GI symptoms dysmenorrhea and she got mono but recovered normally she went to college it was challenging as it is for many who were freshmen in college and she was put on citalopram and she was diagnosed as hypothyroid I’m not sure of whether or not she was had florid hypothyroidism or if she was given that as an adjunct or to help her with her energy in her mood she started having migraines as the stress increased and she was told she had mono again and she didn’t make it through a freshman year but she left school picked herself up got more healthy she started running and from the age 20 to 26 she was able to work in retail and and run she actually ran a half marathon at age 26 she was in a motor vehicle accident modest bad enough that she sustained a concussion and whiplash and Loeb expressed a strain but not bad enough that she was sent for assessment to the ER again she was able to recover she was biking doing yoga weights Pilate and then she met a boyfriend and he didn’t want her on all of her meds he wanted her to be more natural they were she was active and she weaned off her style pram and she’d been taking pee pee eyes a little bit of symptoms increased and they moved to Utah which is where I am they went on a trip to Europe that summer and in December she developed a flu-like illness cough sinus symptoms she was treated with antibiotics flared again not that unusual to have to take couple courses of antibiotics for sinusitis but she really never fully recovered her strength and her lack of stamina and help wellness was really the last straw for their relationship and her boyfriend left and she restarted some citalopram and move with her mother but within the very next month she had another flu-like illness chills fever then developed a sinus infection she was treated with some nice satin and she crashed she crashed in February it was like she couldn’t get out of bed everything was in slow motion she had to take a medical leave from work and she had been seen a number of providers when I saw her GI endocrine or ob/gyn she was seen a mental health provider and a PRN really didn’t have a primary care doctor when she came to see me these are this was her description of her complaints barely able to walk from the bed to the couch low energy she crashes up to trying to do things she’s weak dizzy bumping into walls she’s got some pain burning achy stiffness some joint pain some weird chests things tension headaches migraines and some paresthesias in her hands and feet sweating heat and cold entire tolerance sound and light sensitivity and she had a you know pre-existing anxiety disorder by now she was really depressed and anxious and having suicidal thoughts because her life was really had sunk so I want to give you this little background this is mostly to help you understand my approach if you think about the way we define these illnesses this isn’t necessarily about the numbers kind of is but and this is just my own opinion about how do we group things there’s a big world of chronic fatigue and chronic pain many many many things that can be that walk into our offices fire myalgia is especially the first criteria was an attempt to describe global hyperalgesia and sensory sensitivity and central sensitivity and the chronic fatigue case definitions have been all over the map they’ve changed they started out as a clearly a post viral syndrome and then the term emmy myalgic encephalomyelitis i think of as a smaller group of patients in number but also with more complicated cific kind of presentation and then in 2015 we I was part and nancy clymas as well and a number of us were part of a an Institute of Medicine committee to try to review the literature and come up with maybe clinical diagnostic criteria based on evidence-based for mecfs so this project all of the literature review happened in 2014 there’s never a good time for a cut-off it turned out that a lot of really interesting things were published in the next year or two after this review and if you want to read this it was published on February 10th for their a couple of references there and I just mentioned that the Institute of Medicine then changed to become the National Academy of Medicine but the purpose of the report was to increase clinical diagnosis and improve care for mecfs particularly and to kind of find a way to distinguish it from all the other myriad causes of fatigue and the core diagnostic new core diagnostic criteria focused on the common core symptoms based on the evidence base these aren’t all the symptoms but these are the common core things that all the patients in the literature had in common and they we the idea was to create a more simplified case definition so that clinicians could really use it and separate patients out with this kind of a problem so these core criteria are it’s pretty straightforward number one is we stopped using the term fatigue as the primary defining symptom the first symptom is impaired function manifest as fatigue so mecfs is an illness that dramatically impairs people’s ability to function the second is post exertional malaise and this means illness relapse after physical or cognitive activity actually mecfs patients can have relapse after emotional or that you know up right cognitive work as well as physical activity sleep is very dysregulated and then you have to also have either marked cognitive impairment or orthostatic intolerance and these all have to be present and they must be moderate to severe and present at least 50% of the time and this is determined by some very good research if you use these symptom and severity sorry the severity frequency and severity criteria it really nicely separates mecfs at least from healthy controls and then recognize that there are a lot of other common presenting feel features of illness including all types of pain evidence of immune impairment and sometimes symptoms of infection but those core criteria in order to buy these clinical criteria must be present as a cluster and at moderate to severe and present at least 50% of the time so there really aren’t exclusionary criteria but a differential diagnosis of course is expected to try to understand what all the contributors might be to these symptoms and that they should be addressed and supported and of course you can use a working diagnosis of mecfs but there really aren’t very many other illnesses that just put people down and are completely unable to function so I want to just go through some of the things we do in our clinic to try to make it a little less time-consuming and to zoom in on these features of illness so we do a mental health screen of course we do the interview and everything we happen to use one called the hospital anxiety and depression scale which is down below and then we use the sf-36 it’s really called the rand 36 because essa 36 is a research tool that you need to pay to use in research but the version of it that is accessible to clinicians for use in the clinic for free is called the RAM 36 helps spend more time on this we use the fibromyalgia impact questionnaire to assess the impact of pain on function I’m going to talk to you about the concept of hours of upright activity we have a good day bad form that I designed and then we take a good history about not just symptoms but the consequences of physical cognitive emotional and upright activity what happens when they try to do things we do orthostatic testing I’m gonna say consider cardiopulmonary exercise testing which is what C putt is and talk to you about why and we’re not going to have time to talk about everything but cognitive impairment measuring of carbon cognitive impairment can also be helpful so why the sf-36 to the round 36 this is a a 36 item questionnaire that scores in eight domains and you can see on the left there that there’s four that are considered more physical and four that are considered more mental health and if you think about it like this the ones in the middle have crossover but even more important than that on the right is a published document that shows the top line so the bottom on the bottom line are these domains so physical function role physical bodily pain etc going across the bottom and then how high they score and high score is more normal so the the highest line is the scores for the general population and you can see the general population does pretty well except for kind of the one called vitality which is wealth a little tired right we don’t have pep because we all over do all the time and then it does kind of come down on the mental health a little for the same reason I think but if you look at the scores of these diseases congestive heart failure depression dialysis hepatitis you can see that they go lower and lower on the scale because so the reason the sf-36 is important is because it’s validated across many many many illnesses so you can interpret what the score means so kind of maintain that picture in your mind of those chronic illness scores hovering around the middle of that graph right around 50 and I’m going to show you and this has come this is true in almost all studies of mecfs this is a little study we did and can you see the scores in our mecfs patients and the scores of our healthy controls so the healthy controls do relatively well and our mecfs patients they’re just like down in the the lowest known illness in sf-36 scores because of the severe physical impairment and it usually comes up a little on the pain component because this is not primarily fibromyalgia patients although they can get pain and then relatively preserve mental health scores and you can see that these are my established patients who participated in a study who are pretty well adjusted but they have severe physical impairments so on the right are the scores from the case I read to you of this 30 year old and if you can imagine those numbers that go top to bottom going left to right on the graph and you can see and 0 is about as low as you can go I think we have floor effects with this disease remember the high is good she scored zero zeros everywhere little knots a little higher on bodily pain and a little higher on mental health but so you know we know she has secondary mental health problems as well so the only thing I want to say about the sf-36 the round 36 a little tricky because it has a complicated scoring scheme but it is possible to figure it out and we actually got it programmed into our electronic health record so we could just plug the numbers in when the patient does the questionnaire or you can have your medical assistant score it for you and give them an algorithm to do it so why the fibromyalgia impact questionnaire this is a this is a very simple 20 question questionnaire that assesses pain symptoms and the impact of pain on function you can actually get you can actually use the version that’s called the sickness impact questionnaire to take that word fibromyalgia out of there if you want but why I use this this is a this is a questionnaire that’s been validated and with large large numbers of patients and I use this because it’s easy when people speak patients fill it out I can see where pain fits in in terms of being a limiting part of their or functionality and so you can see those scores there the majority people who sign up for fibre miles of clinical trials drug trials of that kind of thing the average scores in the 50s so you can see that you can become much much more ill with widespread pain based on this score if you keep that in mind our our case this 30 year old scored 86 on the fibromyalgia impact questionnaire which communicates something right away about the impact of this illness on her ability to function especially as framed as a pain and it’s interesting because she really didn’t have fibromyalgia tender points on physical exam so the next tool I want to talk about is something ice bar ode that I developed after hearing dr. David Bell give a talk and he is one of the pioneers in the the research and and an history of chronic fatigue syndrome he was a pediatrician who followed his patients who developed post viral syndrome completely up into adulthood and I was at the state of the knowledge conference early on at the NIH and heard him give a talk and thought you know what Dean the light bulb went on in my head and I realized that asking about this might be very helpful so I incorporated it into our electronic health record and I asked patients not just when they’re new patients but every visit and hours of upright activity is defined for these this purposes as hours spent with feet on the floor okay so we were all engaged in upright activity right now and non upright activity is when you elevate your feet you’re in a recliner or you’re in a bed and I could tell you from now doing this with hundreds of patients this is just based on my experience if you think most of us are healthy we put our feet stay on the floor until we go back to bed we get out of bed our feet stay on the floor or give or take so you just subtract it from 24 and if you sleep eight hours or seven hours or nine hours and we’ll give you a little benefit of up to ten hours where you elevate your feet that’s what normal people do and I’ve noticed that many of my other fatigue patients particularly people with I would diagnosis fibromyalgia with pain amplification fatigue and sleep problems people who benefit from low-impact exercise and actually trying to stay as active as possible their hours of upright activity are usually around 10 to 12 and this is kind of the middle of the bell curve right and when people do better it goes it changes right when they’re more treated when they’re not doing well it gets worse day to day but people with mecfs typically I used to say one to seven and then I got this call from a patient or something online that said where’s the zero I mean some of us can’t get out of bed so some people are completely bedridden with mecfs and really can’t engage in upright activity for more than a few minutes at a time so let me I’m going to go on and develop this concept and try to convince you why it’s important to ask these things we have what’s called it we have another thing we do when this is when we see new patients is we asked them about good days and bad days and we asked them how many good days we they’re two different questionnaires we don’t ask it at the same time but we ask how many good days a month and on good days how much how many hours of upright activity and then we ask them to give specific examples of what they can do on a good day and what they can’t do on a good day and the same thing for bad days and let me show you our case so our case our 30 year old woman on a evaluation at our clinic she had she said she had five to ten good days a month and on those days she had one to two hours of activity tolerance we asked about non upright activity to be sure they’re answering the question correctly so that we could see that adds up to twenty four and then we get asked for specific things and this is spontaneous she write these these are not like picking from a list she can drive go on a short walk stretch run an errand on on these good days but she can never clean make her own meals or go to work on good days what about bad days well she says they’re 20 to 25 days the majority of the month and maybe 30 minutes of upright activity and on those day on the bad days the best she can do is set up read watch TV and eat and on she can never walk or have conversations on bad days so this has become a really valuable you’re welcome to take it adapt it do whatever these are all filled out before the patient comes and I have a really good idea about their activity tolerance by the time they come into clinic so let’s talk about this concept of post exertional malaise the terms may not be very good because lots of people have a consequence lots of illnesses have a consequence after activity but people with mecfs have illness relapse from activity not just soreness not just more tiredness but there’s a threshold that they hit and whether it’s cognitive work or emotional work or whether it’s physical or upright activity if they surpass that they’re going to have a payback and that insidious thing is they often don’t feel anything while they’re doing the activity so the more sick they are and the more orthostatic symptoms they have and other things they can feel it but many people do not feel the consequence of their activity for a 24 hour 36 48 hour lag so this is really challenging for patients of particularly the people who are more moderately ill and I’ve seen people who are almost fully recovered relapse when they started an exercise program so I just think I have a big respect for the problems with energy and we’re still trying to understand why but the reason it’s important to know is it completely colors the way you do physical rehab the way you ask people to do exercise they need to do it very briefly and they need workup solely and they may never be able to work beyond that threshold of their disease so just to share with you a little study we did a long time ago when I was trying to describe these patients to a friend of mine who is a physiologist and a researcher at the University of Utah I said they had a really great pain research clinic and really understood fibromyalgia before anybody else did and I used to go to them didactics and I said hey there’s these patients that can’t exercise you know if they exercise they get they get sick and he said well we should be able to measure that I just love phd’s so um he designed a study week together where they would draw the blood before exercise and then draw the blood at these four times after exercise thirty minutes eight hours 24 and 48 hours to look at gene expression changes the patient’s really it wasn’t hard exercise all the patients that we sent were able to do it they got on an Aerodyne bike they pedaled at 70% of age predicted maximum heart rate for 25 minutes and yeah it’s not just sort of break out a sweat by the end but everybody was able to do the exercise so we looked at sensory receptor sorry yeah the the gene expression of new sensory receptors adrenergic receptors and cytokines before and after exercise and that blue arrow right there is the time of the exercise and I should have made it go up and down so our we tried to get deconditioned controls and our controls everything is zero at baseline and you can see there’s a little bump of gene expression following this exercise stressor and then when we did it and I carefully picked these these are carefully picked people that I had been my patients that I knew had exercise intolerance because they tried and tried and tried and really couldn’t work on exercise and this is what we saw in gene expression of those 20 patients and what that meet and these are fold increases in gene expression of sensory receptors adrenergic receptors that’s this I don’t know a lot into the autonomic nervous system sensory being pain and fatigue and he’s a specialist in the way fatigue pain and fatigue signaling occurs in the spinal cord and the doors horn and then cytokines and what we were stunned to see is there was a marked multi system physiologic reaction of our patients to a sip a simple single bout of exercise and they hadn’t recovered by the following morning you can see at the 24 hour mark so if we and they hadn’t come they hadn’t recovered by the third party so if we’d sent those people to exercise every day you can see how something bad might have happened to them we also looked at a fatigue in multiple sclerosis patients I think that purple bar is a mistake and this is a very difficult lab I think it was a lab error but you can see in the other so these are all completely separate blood draws right so that there’s very consistent findings on this gene expression so this really changed my thinking about mecfs at an early time this is um this was ten years ago that this was bushed and it was published in a pain journal so it didn’t really get much attention they also looked at high intensity exercise with volunteers you can see you can bump the beta 1 receptors and everybody at some point with exercise and just quickly to show you that we looked as pretty small study but we looked to see if the severity of illness correlated with how high those bars got and the most severe patients were at number one and the least severe were 3 and 4 and then these are the these are the healthy controls and the little and the asterisks is there to show you the clinical significance of these values so I just want to say that it’s been difficult to replicate this because of a number of reasons one is there are issues with gene expression the second is there’s a lot of heterogeneity in people with fatigue syndrome and so just putting people in this study based on their diagnosis isn’t necessarily going to show you the same thing it also depends on how well managed they are a gnostic RDR when they come and a whole bunch of different variables die just this was a fairly recent paper by Lily Chu and her colleagues that should try to understand and describe post exertional malaise and 90% of these 150 subjects experienced post exertional malaise after physical cognitive or emotional distress cognitive work thinking hard and I’ve really come to think about how our brain works and how a lot of what we do is very automatic and very low amount of work I think this is why kids with mecfs can play video games but they can’t do their homework it’s like a lot of their automatic responses and things that are just don’t take a lot of thinking they can do but it’s very hard for them to do math to write a paper or to read things for content so the symptoms the fatigue was most commonly exacerbated symptoms but once they had extended their activity they had more cognitive impairment worse sleep more headaches more pain and more flu-like feelings just from doing one of those things including cognitive work and particularly and also emotional distress can also cause the same problems and at least one inflammatory immune related symptom was reported by 60% and they also had gastrointestinal or the static mood related neurologic and other symptoms so does it help to pace should we tell people to stay within their energy envelope and this is kind of an old study it’s been around a long time Lenny Jason has took a lot of research with with chronic fatigue syndrome and he did some studies in 114 well-defined patients and when they did several studies but when he had done these studies he compared patients who were these were studies of helping them learn to adapt and stay within their energy Volokh and they got some of them got counseling and some got at you know normal care some got really specific coaching on pacing meaning not to X not to extend beyond your energy envelope and those patients who were able to keep their energy close to available energy were put in one group and those were not successful at the task were put in another group and this is the way it looked after twelve months and that is and the the solid lines are the people who are able to stay within their energy envelope day to day and you can see that over time their physical function score is improved and their fatigue severity went down and this is not people who exercise more this is people who learn to function within their energy envelope and not exceed it I think this is really important for people with mecfs really needing to have a different approach to maintaining their physical conditioning and those and I’m probably speaking to the choir but when I speak to other physician audiences they really don’t understand how important it is to work with this impatience but exceeding cellular energy capacity results in illness symptoms its physiologic its multi system it’s not fear of exercise it’s not fear of pain I see lots and lots and lots of fibromyalgia I know I know a lot about it and how to treat it and how patients respond to different interaction interventions but the best the key to management of mecfs is to understand these limitations and learn to effectively pace all activity in order to avoid inducing severe or prolonged p.m. which is basically illness relapse and I think if people don’t stay in their energy envelope they go downhill they have cereal worsening and people who stay in their energy envelope and the symptoms die down of their excess activity have the best chance for stability and actually have an opportunity to improve if they’re not experiencing setbacks I wanted to say a word about functional capacity evaluations and that people who apply for disability often are sent for functional capacity evaluation and these are really misleading measure of disability and patients with mecfs because they don’t evaluate post exertional malaise so patients can usually do what they need to do during the evaluation but I had a patient who was bedridden for a week after completed a functional capacity evaluation I want to talk briefly about orthostatic intolerance I think that orthostatic intolerance is one of the most poorly understood poorly addressed aspect of presentations that patients that come to us we’re just not trained to ask the right questions we’re not trained to measure it it’s a tiny bit time-consuming or it seems excessive and inaccessible to send people for tilt table testing and those kind of things and let me define orthostatic intolerance orthostatic intolerance just means that symptoms come from standing and they’re relieved by a line down that’s it so it doesn’t say why and the differential of orthostatic intolerance is actually really big but if you don’t ask about it and sometimes the patient’s don’t know they have it they just know they feel horrible when they’re up and moving around so I think this is a really important aspect of evaluating patients and trying to understand what’s going on with them when they have fatigue this is taken from the Institute of Medicine report and it describes these symptoms as being caused by cerebral under perfusion and peripheral cardiovascular symptoms and signs so patients can feel all these symptoms they may not faint honestly I think sometimes early an illness or if they’re really pushing and trying to function but normally they just can’t think straight the disoriented they have headaches they have weird fleeting neurologic symptoms and can’t stay up very long and then they can get palpitations and GI symptoms and cold hands and feet a lot of a lot of it feels just like anxiety because when you if you’re if you stand and you get venous pooling and you get diminished returned to the heart you get this big adrenergic response either of norepinephrine from your sympathetic nervous system or the release of adrenaline and epinephrine from your directly from your adrenal glands to try to keep you upright and it can create a tremendous amount of symptoms and in kids who develop pots I think this is often entirely missed and they’re diagnosed with anxiety and depression and treated as though they’re having panic attacks so it’s worsened by heat dehydration prolonged standing deconditioning feels worse after exercise and there we could give I could give an entire talk to son orthostatic intolerance and not have enough time so this is something I want to bring to your attention and then you know you can self educate there’s a big a big literature about pots there’s a big you can look up treatment you can look at other kinds of orthostatic hypotension this is the chronic kind and you there’s a big differential that I can’t even begin so cardiac pulmonary disease drug side effects a lot of people don’t know that and yaz the birth control has Droste respira known in it which is a which blocks aldosterone receptors and can give people orthostatic hypotension anything in the central nervous system or the cord cranial cervical instability Parkinson’s other things and in anything wrong with the peripheral nervous system spinal cord injury small fiber neuropathy and we you know a lot of the research and mecfs is pointing toward central so neuro inflammation and in and some small fiber neuropathy and small fibers basically innervate the sensory system and your autonomic nervous system so you don’t have to have a peripheral neuropathy to matter I’m sorry you know it doesn’t have to be peripheral it can be patchy and involve your autonomic nervous system in a way that’s a bit more insidious and the other thing to think of as well recognize acute symptoms of orthostatic intolerance you know getting up out of the hot tub having you know bleeding or being dehydrated when your body’s not accustomed to it but chronic orthostatic intolerance is in a kind of chronically compensated state and becomes much more difficult to you have to think about it actually because patients just have no appetite and they just got nonspecific complaints and sleep problems probably from all that allergic overdraw and a takes have okay is heinous even vertiginous dizziness because probably possibly because of altered perfusion to the to the auditory nerve and maybe even to the brain and tremor so again I’m just trying to remind people that once you diagnose orthostatic intolerance it’s on you right to try to understand why people have orthostatic intolerance and there’s a lot we can understand about why and then there’s going to be some that we aren’t able to understand the cause so why do we care about it well we I care about it in my patients because it’s measurable and treatable if people have worth the static intolerance you might be able to understand what the underlying disease is but more than anything you can use very common measures of fluids and sodium and compression socks and well known medications to kind of tweak and help their autonomic nervous system function better and remember that it can exist in someone who has low normal or high blood pressure so this is a drop in blood pressure or it’s actually even more complicated than that it isn’t just blood pressure and I’ll talk about that in a minute but if it it is a change from body position rather than what your resting vital signs are so the the terms of orthostatic chronic or and tarts that we’re familiar with that have definitions orthostatic hypotension that means your systolic pressure code goes down at least 20 millimeters of mercury when you stand within the first three minutes or 10 at drop in 10 of the diastolic pressure pots is defined as increase of your heart rate of more than 30 in adults and more than 40 in adolescents and kids and then neroli mediated hypotension or nearly made it syncope depend on where you read it is more like a delayed faint after people have been up for a long time well what I can tell you from doing testing now and hundreds of people is it’s great to have these categories but people present with other findings that don’t necessarily fit in this category and this is there is another paper coming out looking at this with bedside orthostatic measures but this paper looked at tilt table testing in 150 mecfs patients they also measured stroke volume index and cardiac index with the supe super sternal a or tic Doppler imaging and they looked at it you know lying down before the tilt and twice the tilt and they weren’t able to show big differences in heart rate and blood pressure in this patient group but the stroke volumes in cardiac output were told the story and decreased as in stroke volume index and cardiac index during the tilt were significantly larger in mecfs patients so these well compensated patients don’t necessarily manifest changes in their heart rate and blood pressure during this test but they’re getting really significant venous pooling and diminished preload and diminished cardiac output just from standing which has its own set of complications so these are the things that we do I say easy because nothing’s ever easy but these are the things we do in our clinic this is really just for reference but there was a paper published this was in training to understand neurogenic orthostatic hypotension in Parkinson’s and some other neurologic diseases and they developed an orthostatic hypotension questionnaire that was divided into the impact on symptoms and activity well we borrowed this and we call it the orthostatic intolerance questionnaire because it isn’t necessarily orthostatic hypotension and we ask patients these six questions directly from this questionnaire they score zero to ten and ten is severe in this case in terms of symptoms and activity and we compared it to the patient’s reported hours of uh productivity this is the work of a graduate student in our office G Hanley and our we actually did a study using a ten minute lean test that was she’s studying the data but Apella wall who’s now in medical in medical school designed the protocol so we tested the orthostatic intolerance questionnaire and hours of uh productivity in twenty five female patients and matched controls and they completed these questionnaires this is just a subset of this study looking at hours of uh productivity and using these questionnaires and we divided our mecfs patients into four groups depending on their hours of uh productivity tolerance so one to two of course the zeros couldn’t come to clinic great so one two – three – four five – seven and greater than eight hours of upright activity and then we use these questionnaires and this first table is looking at symptoms of orthostatic intolerance so this is one set of the orthostatic intolerance questionnaire and there are so if you look down there are one two three there’s six questions about symptoms from orthostatic intolerance and then the purple column it’s a healthy group and they stop them score more than one on a score of one to ten you can either do one to ten or you can do to a hundred just depending on how you use it so that was interesting to me because I’d been seeing these scores and I didn’t really understand what healthy controls would do on this orthostatic questionnaire but they don’t they don’t have any positive or hardly any positive answers on this orthostatic questionnaire and then if you look across the mecfs group they’re getting more and more severe well I should say they have more and more hours of upright activity less and less hours of upright activity tolerance so starting with the the best patients and over to the ones who can barely have any upright activity tolerance I highlighted anything that was five or above but you can see that the scores for all of the mecfs patients were much higher in terms of orthostatic symptoms with the highest scores being in the severe patients who could hardly get out of bed and then if we look at the activity portion of the orthostatic intolerance questionnaire this is the we call them who an OE this so then we you can see that the healthy group asked about standing a short time standing a long time walking a short time walking a long time you know really have very very low scores very normal scores and then across our mecfs group the scores get much higher so how do we do this how do we test for orthostatic intolerance well the gold standard is head up tilt table testing which I had a hard time ever getting and it’s not very standardized so we do what’s called the ten minute NASA lean test it’s not really ten minutes that’s a little bit misleading because you have the patient lie down for 10 or 15 minutes and equilibrate then you have them stand for 10 minutes and then you have to give them some recovery time they just lean against the wall we put a pulse ox on their finger we do a manual blood pressure and this is one of the first patients I tested at my clinic someone I basically called fibromyalgia and her mood stuff and you can see that in the supine resting position her blood pressure was 116 over 60 heart rate 85 and look at the pulse pressure this is kind of an approximation of that cardiac index in those studies the pulse pressure is the difference between the systolic and diastolic pressure and immediately when she stands up her blood pressure starts to go down or her heart rate starts to go up and what I’ve learned from doing a lot of these if you look at the difference between 8 minutes and 10 minutes when they start going back down again the patient’s going to go down but this her pulse pressure when we stopped the test was a fraction of what it was when we started the test which is a reflection of venous return to the heart so she meets criteria for systolic orthostatic hypotension because her systolic pressure went down more than 20 and she meets criteria for pots because her heart rate went up 41 beats per minute and what was really enlightening for me is we reproduced her symptoms during the test she had all these weird symptoms I couldn’t explain and a lot of people thought were psychiatric symptoms so what about our case in the questionnaire it’s there’s a typo in here I’m sorry but she had 76 out of a hundred or seven point six out of ten depending on average and that would be 47 out of 60 on the symptom assessment and 29 out of 40 on the activity intolerance so this shouldn’t be hundred on the bottom of there and when we did her tilt table test her heart rate went from 75 to 118 and it was really difficult to read her blood pressures I’ll show you the values in a minute and she dropped her pulse pressure by about 50% here’s the actual numbers people with a low broad blood pressure to begin with usually have a little smaller pulse pressure pulse pressure was just barely 30 and then we just honestly couldn’t really measure her blood pressure and her heart rate went from 75 up to 119 so you can see she has marked orthostatic intolerance and this is giving her a lot of her functional impairment how we don’t know let me just say quickly because I think my time is almost up cardiopulmonary exercise testing can be done and there are some really good studies that show patients with any mecfs are unable to replicate their cardiopulmonary exercise testing parameters on day two and this is very unusual this is not typical for any other illness so this is the red would be the peak vo2 for mecfs patients test one two test two that was a long time ago and more recently 2,000 in 2014 Betsy Keller and all you need to look at is the grey bars are the results on the first test and the light grey bars are the results on the second test and you can see that almost every parameter that she has up here of cardiopulmonary exercise testing is lower on day two and that’s because we hurt them on day one and the exercise of day one makes it so they can’t replicate the test on day two so I think I’m going to skip my cognitive impairment slides except to say that we have good evidence that people with mecfs have cognitive impairment some of it is primary maybe neuro inflammatory and some of it is secondary form orthostatic intolerance and other variables but slowed information processing is the the manifestation which makes it really difficult for people to function in the workplace because they can’t multitask they can’t finish things on time and if they’re put in a quiet room and they’re able to do things in small increments they’re able to do a lot more but it’s the main reason and I’ll just maybe leave observations about cognitive impairment so in summary really wanted to help you learn how to ask the right questions to understand if people have impaired function if they have post exertional malaise use some questionnaires that will help you understand their function and I’m really trying to encourage more physicians to do orts or a bedside orthostatic testing to understand and then maybe get motivated to go down the path on understanding that better so I think I’ll stop there and take the time for questions thank you dr. Bateman there’s a there’s a microphone up here in the center aisle for anybody who would like to ask questions we’ve got about 5 to 7 minutes before the next speaker thanks I’m gonna humble myself completely first of all thank you so much that was that was really great I’m gonna have to sit down with the numbers cuz I my mind is going but my question to you is and I’m confused so I just like some clarification the pulse pressure what is a healthy pulse pressure in for say normal population it varies by age and and a number of factors but if you think you know what do we say 120 over 80 yeah it’s a normal blood pressure so a normal pulse pressure of a middle-aged person who’s got normal activity is usually 40 right so if the pulse pressure is very close together and I’m sorry the pulse pressure is very low and you think that means there’s less venous return so yeah I said I skipped a step so the pulse pressure is arterial right okay and the pulse pressure reflects the high and low pressures which is determined by the cart you know the cardiac index and how much force and volume of blood is being pumped yes and that’s determined by venous return to the heart so passing passive filling of the heart so in the you know I you can see that intact and like really tachycardia but some of our patients really don’t have that much tachycardia not enough that would influence and studies actually have shown there’s kind of the preload failure in patients when they’re upright we have quite a bit of evidence now and that’s reflected indirectly in the clinic by those changes it’s complicated because you have to take into account age and elasticity of the aorta and all the stuff it’s just a rough way of seeing that your patients probably getting diminished cerebral precision so basically the amount of volume being pumped through the blood vessels has gone down per unit time right right and so we see a lot of people with ehlers-danlos mm-hmm so we’re getting sort of lots of expansion and and I can’t remember exactly I’d have to look at the numbers but the pulse pressure there I think we would expect it to be quite low as well right I don’t know that you know so really this is just a ruff-ruff way to assess a change from supine to upright yeah and so there are lots of ways lots of different implications for any given pulse pressure yeah but this is just a way to convince yourself because there’s venous pooling it’s diminished cardiac return and then you can’t just look at the heart rate and blood pressure although sometimes they tell you the whole story but it’s really looking at signs and symptoms and the can the post pressure and all the findings to convince yourself that the patient’s having a phenomenon but then you can address and just one more comment is um so we work with cardiologists at Women’s College Hospital and she’s just flooded this cardiologist she’s become the Guru for orthostatic intolerance and for the chronic fatigue syndrome patients they’re finding that a lot of their their problems come from deconditioning and they have to try to tease out what’s what and then there’s the yellows dan los folks as well so there’s so we’re trying to figure out how we can recondition and also use the support hose and you know salt loading fluid learning all that kind of stuff to try to get you know the volume pump back up again so it’s a big it’s a big deal it’s like this is one of the biggest deals that we’ve had to deal with so thank you so much yes thank you for confirming that I do I agree it’s a big deal and we never looked at it much in the past and we should be looking at it and then we should educate ourselves about the differential and how to address it I thank you for your lecture it was very informative I was curious because I know there are also different levels but how you sort of start they’re reconditioning program or so that you don’t cause a relapse right so deconditioning is an important aspect of you know of orthostatic intolerance it takes about three weeks for astronauts in space to get completely their autonomic nervous system to get completely discombobulated and when they come back to gravity they have a hard time even walking did orthostatic intolerance so we try to keep it in mind maybe in our patients now not everybody meets mecfs criteria right so a lot of people can condition actively through syndromes of orthostatic intolerance and our patients may have more of a ceiling about what they could do and that makes it even more complicated but we try to get people to do things line down semi-recumbent and in water because then you know any I call it ortho I friendly exercise so things that don’t trigger the orthostatic intolerance then people can do more with their exercise before they’re limited and that’s been really helpful it’s hard to get people to a pool but whether you’re horizontal or in deep water water is very very effective at minimizing the orthostatic intolerance and is good in lots of other ways of course and if the water is cooler actually might help a lot of people don’t like that but they face a constrict a little bit and and you don’t want to be in hot water because then people basically dilate and it makes things worse thanks again I’m thinking of a patient as bizarre as it sounds we actually have a patient that if you give her she’s about 50 years old if you give her a crossword puzzle any time of day she will develop within two or three minutes Bell’s palsy it lasts about 24 hours and it just goes away so long as you don’t give her another crossword puzzle but you can set your you can set your watch by it every time any time that’s interesting would you say that that does she meet mecfs criteria okay well that’s cognitive work so yes I believe that and I’ll tell you something I had a good friend who is a super wasn’t a good friend he was a super successful cutthroat litigator attorney who had a mind like a steel trap and he could lower his blood sugar by thinking seriously he had to be much oh sorry that’s how you had diabetes so he had an insulin dependent diabetes and he had to be really careful when he was working on briefs and working on legal things because he would get hypoglycemic from thinking from cognitive work that was a really good little lesson for me


  1. Fibromyalgia is cerebral spinal a fluid leaks caused by a sacral spinal cyst Tarlov.cyst disease period. Start there for instance the. # 1 expert for leaks Cedar Sinai leaks program dr Sheivink says no phsycal therapy. That runs against current recommendations for the treatment of FM. You could have a nuerologist do an ent. He could locate and give you the size of the.lesion in the sacrum
    But he won't know anything about it. Nuerosugeons are carefully trained to ignore them, not Tarlov or the twenty lesser spinal cysts. The NIH has thrown down the gauntlet informing micro vascular nuerosugeon. The minimumly invasive technique tarlov cyst surgery using tubular retractors. Called Tarlov clipping. Safe effective that obliterates the cyst. Three day recovery instead of 5 months for full on surgery which at best is a temporary fix.


  2. So great to see Dr B presenting to physicians in a wider forum 😊

  3. I'd liove a transcription of this. I learn best that way.

  4. @16:12 Hours of Upright Activity (HUA)
    @23:00 Gene expression during exercise for CFS
    @28 Pacing importance
    @31:45 Orthostatic Intolerance Evaluation
    @49 Questions

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