Episode 29 – POTS Basics: POTS and ME/CFS

00:01 Announcer: Welcome to the Standing up to POTS podcast, otherwise known as the POTScast. This podcast is dedicated to educating and empowering the community about postural orthostatic tachycardia syndrome, commonly referred to as POTS. This invisible illness impacts millions, and we are committed to explaining the basics, raising awareness, exploring the research, and empowering patients to not only survive, but thrive. This is the Standing Up to POTS podcast.

00:29 Cathy (Host): Hello everybody, and welcome back to this episode of the POTScast. I am Cathy Pederson, the founder and president of Standing Up to POTS, a POTS researcher, and a mother of a POTS patient. I'm filling in this week for Jill Brook and she will be back next week. I am here with my friend and fellow advocate Colleen Steckel, and I'm so glad that she agreed to join us as a guest on the podcast today. Colleen developed sudden onset myalgic encephalomyelitis at age 29 and was later also diagnosed with POTS. Colleen is on the board of M.E. International, that ‘M.E.’ is for myalgic encephalomyelitis, and has been a strong advocate for the myalgic encephalomyelitis community for many years. She began advocating at the national level when she joined MEadvocacy.org. She has also run the public Facebook support group, North Carolina/Ohio, ME and FM [fibromyalgia] support group since 2014. She has shared her story publicly before on the local newscast and representing M.E. Advocacy on ME/CFE Alert helping to raise awareness about these chronic invisible illnesses. Welcome, Colleen, to the POTScast.

01:55 Colleen (Guest): Thank you.

01:56 Cathy (Host): Would you be willing to share a little bit of your story with our listeners about your personal experience with POTS and then ME - myalgic encephalomyelitis?

02:07 Colleen (Guest): Thank you for having me. I'm happy to share my experience. I had sudden onset during a camping trip in August of 1989. It was the weirdest experience I'd ever had. It felt like something I had never experienced before. It was like the plug had been pulled out of my energy system. My whole body felt completely weak. And then I was nauseous and dizziness, vomiting and I didn't go to the doctor at that time, as I assumed it was normal flu and it would pass. Then I thought it might be caused by pregnancy and when it didn't resolve after our daughter was born, I sought answers, and in 1991 I was given the diagnosis of chronic fatigue immune dysfunction syndrome, called CFIDS, which was another name for myalgic encephalomyelitis at that time. I wasn't diagnosed with POTS until about 2016. Up until then, my dizziness and difficulty standing for long periods were mostly ignored by doctors. I managed it as best I could by drinking lots of fluids, including electrolyte drinks, and avoiding standing as much as possible.

03:21 Cathy (Host): Wow, what a story. So you were just out on a camping trip with your husband and all of a sudden these symptoms hit and don't go away. I think a lot of our listeners can relate to something like that, right? I know a number of people in the POTS community that can tell you the date that they got sick and how strange that really is.

03:47 Colleen (Guest): Exactly.

03:50 Cathy (Host): Yeah. Would you mind sharing some of your most debilitating symptoms and then maybe a little bit about how those have impacted your life since that camping trip?

03:59 Colleen (Guest): Sure. My most debilitating symptoms really are typical myalgic encephalomyelitis and I realize most people don't really understand what that constellation of symptoms looks like, but the main issue is post-exertional neuroimmune exhaustion – we call it PENE for short – or a lot of us just call it crashing or having a crash. If I'm active outside of my limited window of activity, then my brain function, my immune system, my muscle function, and my stamina get much worse. The crash can last anywhere from a few days to much longer, depending on what led to the crash. PENE is really an experience of taking all the symptoms I have all the time and making them all much worse. So it's not like I'm well and get sick, well and get sick, I’m sick and then I get sicker. For me, that getting sicker happens at about 30 hours after the activity. In the early years, I was mostly bedridden and severely ill. The pattern was not as obvious. As I regained some level of function and started doing more things, the pattern became much more obvious. I'd go to church on Sunday, then be OK on Monday. Totally crashed on Tuesday morning, then start feeling better about Friday. Then go to church on Sunday and the cycle would repeat. At times the symptoms would last closer to 10 days, and now I recognize that happens when I am exposed to a cold or other virus. When that happens, I don't get normal cold and flu symptoms, I just feel a whole lot worse with all of the M.E. symptoms. On top of dealing with crashes, I have ongoing cognition issues, lack of stamina in my muscles, immune system dysfunction, which includes opportunistic infections, multiple chemical sensitivity, suspected mast cell activation, and POTS. So it's a pretty recognizable pattern. For people who keep having repeated infections, this is something to consider. I am mostly homebound and I'm fortunate that my husband has graciously taken on managing our household and shopping, leaving me time to manage my health and to advocate for people with M.E.

06:24 Cathy (Host): I'm really fascinated by the delay in the crash. Colleen and I know each other and have known each other for a long time, and our listeners have heard about my daughter as well, and I think she probably has chronic fatigue syndrome. I'm not sure at this point she really is at the bigger symptoms of M.E., which we're going to get into. But it seems like there's not a delay in her crash. When she's done whatever, she definitely has that post exertional malaise that you're describing. I mean, she looks like she's got no muscle tone maybe the next morning or even later that day, but doesn't have that 30 hour window. I'm fascinated by that. Do you have any anything that you've ever read that might explain why there's that delay?

07:14 Colleen (Guest): Well, I've read some about it, but even just in my observation, there's discussion about oxidative stress. When we use muscles, the oxygen isn't getting into the tissues or the cells well, and the junk that's in the cells isn't coming out. And it ends up being this cascade of toxicity, I think I'd call it, 'cause it does also feel like a hangover. And so I'm thinking, just from observation and maybe stuff I've read, that when we do this activity and we have adrenaline going and all that stuff happening when we're being active, that the downstream effect of the activity takes a while to kick in. And I am fascinated that my reaction is very consistent in that 30 hours. We started to time it. It was like, Oh my gosh, this is literally if I do an activity at the same time every time - that's why church was the obvious - that was the same time every week, and the crash hit the same time every week. And it was like that is just an amazing pattern to see. And I wasn't going to church until I had started to feel better, I had higher function. I was able to get out, I was able to be around people, but it was difficult. So, understanding that, it just seems to be it’s a downstream effect that takes some time to happen. Now I've had periods where, especially if I'm really pushing myself physically, the muscle dysfunction – the not being able to move, the whole thing - that can happen immediately, but that's more of the muscle fatigue, that's more immediate. This exacerbation of symptoms 30 hours later is, I think, a fairly unique thing to M.E., and we're talking nothing that's more than malaise. Post exertional neuroimmune exhaustion - very hard to say, but it definitely offers a better insight into this difficult -- symptom is kind of difficult word because it's way more than a symptom, it's this downstream effect of total crash. So crash is really good way to term it. [Laughs]

09:28 Cathy (Host): Right, and that's what we say in my family, too, is talk about her crashing after she's done too much. I think that's really fascinating and you said a couple things in there that I thought was interesting, and one was oxidative stress. And as a biologist that starts some loops going in my head. And the other thing you said was toxicity in the muscles. And when the energy system in those cells is not working properly, I could see where you could have a delay like that and where you don't clear some of those toxins that maybe continue to build up in a way that wouldn't happen in someone that doesn't have that syndrome. Absolutely fascinating, and my guess is we don't understand exactly the route of what's happening, but I don't know that the experts do either. There's a lot to learn still.

10:18 Colleen (Guest): There is a lot to learn still.

10:20 Cathy (Host): Right. So, some of our listeners may be thinking, you know, “I know I've got POTS. I wonder if I also have M.E. - this myalgic encephalomyelitis - or chronic fatigue syndrome.” How would they know, in your experience, whether M.E. as the bigger example might be part of their picture?

10:42 Colleen (Guest): To understand myalgic encephalomyelitis, I recommend using the international consensus criteria (ICC) and the international consensus primer. Part of my advocacy and the purpose of M.E. International and MEAdvocacy.org is to help educate patients and doctors about M.E. as explained in the ICC. A criteria is something that helps doctors and patients have a grasp of what the disease is they're trying to diagnose or what they're experiencing. So the international consensus criteria - what we call the ICC - was created by clinical experts and others who collectively had diagnosed and or treated more than 50,000 patients. When I first read the IC primer in 2012 that goes with the international consensus criteria, I was shocked at how it explains so much of what I had been experiencing. And much of their advice for managing M.E. matched what I had already figured out how it helped me. Unfortunately, there are very few doctors who are trained to make this diagnosis. The first thing to watch for is that post exertional neuroimmune exhaustion. And the international consensus criteria describes that as, and I'm going to read this because I want to get this wording right: “The body is unable to produce sufficient energy on demand, like a furnace that has its pilot light on, but it cannot be turned up to address the need for additional heat.” Another part of that is muscle fatigue ability or low mental or physical stamina. My understanding is that someone with POTS who does not have in M.E. are able to gradually increase in exercising, although they may need to do this reclined because of the difficulty when they're standing up. But people with M.E. cannot exercise safely and trying to push to gradually increase how much exercise they do will lead to that post exertional neuroimmune exhaustion. So, watching for that specific symptom, the thing that we're talking about that happens when we do too much activity outside of our limited window of function, that crash - what does that crash look like? How is that happening? What is the downstream effect of activity? That's the first thing to look for, but there's a lot more to M.E. an just the post exertional neuroimmune exhaustion. Like I said, people with M.E. cannot exercise safely and trying to push gradually decrease how much they do will lead to the PENE. People with M.E. should never be pushed to exercise beyond their energy production limits. So thorough screening is really hard to get, but it is worth being a self-advocate to find answers. So, like I said, there are a lot of symptoms. And they're in a constellation - a recognized pattern of symptoms. And so those experts who had seen thousands and thousands of patients put this together in a way that doctors and patients could do that first level of screening. And it's called the international consensus criteria. It's a little hard to read, so MEAdvocacy.org at their website, on the resources page, the very first item is called the ICC Questionnaire. And we literally just took the international consensus criteria and we put it into a questionnaire format. So first thing you read is, do you have PENE? That's a yes or no. You do have to kind of decide whether you think you have PENE. If that's a yes, then you go into talking about the other constellation of symptoms that come with the immune dysfunction or the orthostatic intolerance and all of the other things that are this pattern of symptoms that leads to a thorough diagnosis.

14:55 Cathy (Host): I think that's really interesting, and I want to go back to talking about the exercise intolerance. A lot of people with POTS have that too, and this is part of the difficulty, right, is trying to figure out what POTS and what's M.E.-related and I I just want to share a story with our listeners. And this is why I think my daughter is on that spectrum. She's not as severe as you are for sure, but I feel like she's on that spectrum. And we tried everything to get her better when she was younger, and one of the things that we tried was the Levine protocol, which is a graded exercise protocol where you start recumbent, and you work your way up and there were heart rate targets and it was pretty rigorous. And I know several other teenagers who really benefited from that, and it brought them back to life. And my kid, it drilled her into the ground. I mean, she just got worse and worse. Her symptoms were exacerbated. We did it for about 9 weeks and her cardiologist said stop. You know, if you were going to start to see some benefit, it should have started to happen by now, and this is not the thing for you. So it's really difficult, I think. I think it's a matter of degree for some of these. Where with POTS that can have the exercise intolerance, do you have the M.E. sort of coming in? And I'll say another thing, and I think you taught me this term, is this idea of an energy envelope - that as long as you stay within your energy envelope, and we're going to hope that we do that for you today, that you're OK. You don't crash, you don't have that reaction 30 hours later. But if you push past that, right, you really pay for it. And so I wonder if you can talk just a little bit about what that energy envelope has meant for you, as far as living your everyday life and what that might mean for someone that's got severe POTS and/or M.E.

17:00 Colleen (Guest): Yeah, and that's an important topic and I really think we can't express enough how much that impacts daily life. So it's so hard for somebody who hasn't had this experience or hasn't lived with someone with that experience to understand what that means. So I would go to church on Sunday. And then people would want to go do something on Tuesday and say, “Well, I just saw you on Sunday. You must be feeling better.” Or I would see people at church and say, “Oh, you must be feeling better. I'm so glad to see you here.” And it's such a difficult thing to explain, and often I don't explain it to people who aren't in the community or who are just, you know, passing by or whatever. “It's nice to see you feeling better,” and like, “Thank you.” But in reality, that really just means that that's the day that I'm choosing to try to do something that maybe is inside my energy envelope. So over the years my envelope has changed drastically. The early years the envelope was literally zero. It was crawling to the bathroom. It was barely able to do anything around the house. So on a day-to-day basis now - where I'm at now - so to clarify, I've been sick 31 years. I've been higher functioning, lower functioning. Over the years I have found things that have helped me function better. They have improved my energy envelope. So inside that envelope, I can do much more. But I still have that envelope and I still have to make sure I don't go outside of it. And if I go outside of it, I have the crash. That means I'm homebound. My husband is doing all the shopping, my husband is taking care of all the bills. I don't have to worry about a lot of other things. He supports what I do in advocacy with the understanding that I'm using my energy in this area of my life because it's very rewarding. It's an important part of my life and if I was to have to do the around-the-house things, that would make it much harder to cope with an illness that takes so much away anyway.

19:17 Cathy (Host): Absolutely, yeah. And I think I love that idea that your energy envelope has sort of changed over the years, that sometimes it was really narrow, that it took very little effort for you to actually be outside of that and in trouble. And now that maybe it's a little bit bigger and you can do a little bit more, but you still have to stay within those bounds. I'm going to shift gears a little bit. My daughter's first diagnosis, and I don't think very many people know this, was chronic fatigue syndrome. She got sick. I thought she had strep. It was some sort of virus. Eventually we decided it was mononucleosis. And she was ten, and this kid had been a little Energizer Bunny. Good kid, but energy out the wazoo. And after she got sick with what we believe now was mono, she went into this unrelenting, crushing fatigue. She would describe the way her legs felt like she had cinder blocks for feet, that her legs were so heavy. She would say when she was getting up out of the recliner that she felt like she had to push an elephant off of her chest, just - and what was she was trying to say was it took so much energy for her to even move, to get up and go to the bathroom like you were talking about there. And she's 19 now and in college and doing better, she's added a few diagnoses over time. We've added some different treatments over time that, like you, have helped a little bit. But I'm really glad that we didn't stop with that first diagnosis of chronic fatigue syndrome that we continued to look to see what else was going on. Have you heard of other folks that are in that situation?

21:01 Colleen (Guest): This is such an important topic. In my 32 years I've seen so many given the chronic fatigue syndrome or M.E./CFS diagnosis who had not been thoroughly screened and they ended up having something completely treatable. Some as simple as vitamin deficiency, especially B12 and vitamin D deficiencies which can cause a lot of debilitating symptoms. It's hard to see so much unnecessary suffering, which is why I started advocating in the 1st place. A good example is a story I just saw of someone who had been given an M.E. diagnosis and has been sick more than seven years. She just discovered that she has spinal stenosis, and she is now set to have surgery, which is expected to lead to a full recovery. I really hope that's the case. If her doctors had been following the guidance in the M.E. IC primer, spinal stenosis would have been part of the screening process. So to clarify, the M.E. IC primer is written by most of the same doctors who wrote the International Consensus Criteria, and they started to figure out: 1) things that could help their patients, and 2) oh, look at the things that were caught, that they shouldn't have been having this diagnosis in the first place. They put into the primer a list of testing to do to make sure that the patients who are getting treated for M.E actually have M.E. And so, in that list of testing are some really basic things, including the B12 vitamin D, checking for spinal stenosis, making sure they don't have MS [multiple sclerosis]. I've certainly seen a lot of people over the years who were diagnosed with me who had MS, and vice versa. I have seen people in the MS Community who were given MS drugs, treated as if they had MS, and they did much worse until they realized MS was the wrong diagnosis and they had in M.E. These two diseases look an awful lot alike. So it's really important that there's an MRI done.

23:28 Cathy (Host): So let me just cut in here. So, MS is multiple sclerosis. Just to be sure that our listeners know that you're talking about. And the reason you'd have an MRI is because they'll see plaques on the brain. So, multiple sclerosis is a demyelination disorder, and you could literally see the demyelination on the axons in the brain. And so that's what you're talking about with MS.

23:53 Colleen (Guest): Exactly, and so an MRI is absolutely important. There can be signs in the brain for M.E. They don't look like MS. So sometimes those are seen, but most often neurologists are really looking to rule out MS and any signs they see in the brain of an M.E. patient are not well recognized. And so they don't do an M.E. diagnosis based on an MRI. One thing I do want to add is I think too often getting a diagnosis is seen as the last step, instead of the beginning of the journey to get answers. So like you said, getting a CFS diagnosis for your daughter wasn't the last step. It was very likely one of the first steps to really trying to understand what's causing the symptoms. Knowing what's causing the symptoms is the key to knowing how to treat the symptoms.

24:50 Cathy (Host): Absolutely, and I have to say when my daughter was finally diagnosed, I thought, OK, we're done. You know that we're going to get her on treatment and she's going to go back to her life and that's going to be that. I was really naive at that time, thinking that diagnosis was the hard part of our journey.

25:11 Colleen (Guest): I remember when you were going through that, and I remember speaking with you, and I remember praying that I was wrong, because I recognized so much of what I saw in your daughter. I really wanted this to be something you know, simple, something easily treated. And so over the years as I've watched her go through what she's gone through and knew what you were going through, having been down that road, I just always hope that, you know, we will find answers and we will get some real treatments.

25:44 Cathy (Host): Yeah, I think that's the great thing about you is that you're not doing any of this really for yourself. You're trying to help the next patient, the next physician, the next person, whoever that is to get faster diagnosis, better treatment, understand what it is that's going on. So that's the perfect segue to my next question, and I think a lot of folks really don't know the difference between M.E., which again is myalgic encephalomyelitis, and what a mouthful that is, and CFS which more people maybe know is chronic fatigue syndrome. So what's the difference between these two sort of in the scope, and also, in how they would be diagnosed?

26:28 Colleen (Guest): OK, so these labels have very different criteria attached to them, but few doctors are trained to diagnose M.E. So most doctors do a basic exam and basic lab tests and then diagnose with CFS or more recently M.E./CFS. So, part of my advocacy and that of M.E. International and other orgs that support use of the International Consensus Criteria is to share the International Consensus Primer that has a guide for diagnosing M.E. Basically they are stating that M.E. is a distinct disease which has been lumped in with a broad diagnosis criteria of chronic fatigue syndrome or chronic fatigue, and should be diagnosed separately. Now I do want to clarify that when I say M.E and I say CFS, I am referring specifically to the diagnostic criteria attached to those labels. We have a lot of people with the CFS label who have M.E. because their doctors didn't know to do that differentiation. What I'm saying is you've been given that label. There's a guide to make sure what you really have is chronic fatigue syndrome or myalgic encephalomyelitis. And once that differentiation is made, what they call ‘stratification’, then you have a much better idea of what treatments may be beneficial. Someone with chronic fatigue syndrome who doesn't have M.E. may be given very different treatments - effective treatments - but those effective treatments for CFS may be harmful for someone with M.E. So it is important that, again, we don't just stop at a diagnosis whether it's CFS, M.E., or M.E./CFS. We need to make sure that there's been a thorough screening take place, and that's where patient empowerment comes. Patients making sure that they really know what diagnosis is accurate.

28:31 Cathy (Host) You brought up this M.E./CFS and I have to say, and I think you yell at me every once in a while that when I write in the journal article, sometimes I do that because it's the way that a lot of scientists write about it. So instead of just saying M.E. or myalgic encephalomyelitis or just saying CFS or chronic fatigue syndrome, we sort of lump them together. And so this M.E./CFS has become a common label, I think for scientists like I am, but also for physicians, even though it doesn't have a diagnosis code. So how is that different from the M.E. and CFS that you were just talking about? Or is it an umbrella for everything?

29:07 Colleen (Guest): So in about 2015, the US government hired the Institute of Medicine to write a report about what is M.E, what is CFS? And they looked into the research up until that time and they looked at chronic fatigue syndrome research, they looked into the M.E./CFS as per the Canadian Consensus Criteria research, and they wrote a report. And in the report, they basically said we've looked at the M.E., we've looked at the CFS, we are going to look at how those two distinct different groups of patients are overlapping. What symptoms are the same in both of those patient populations? And the ones that have been studied under M.E, the ones that have been studied under M.E./CFS, the ones that have been studied under CFS. And they took all of that information and they overlapped it together. And they went, “Oh look, there are some overlapping symptoms within all of this umbrella” - and they called it an umbrella - “patient group.” And then they wrote a criteria based on all of the symptoms or the symptoms that overlap. So now we do have an M.E./CFS criteria that is on the CDC website, but it is not the same M.E./CFS criteria as the one written by the Canadian Consensus Criteria. It's really taking all of the patient groups, putting them together in an umbrella, and then looking to see what are common. So, while that is a way to screen for this big group of patients, what it also did was leave out many of the things that make M.E. distinct. And so it is still not synonymous with M.E. It is a different criteria from the M.E. criteria. So we're in the exact same situation we've been, no matter which label you've been given. You need thorough screening to clarify what is causing your symptoms, so it's important that patients advocate for themselves and make sure that they've gotten the thorough screening.

31:31 Cathy (Host): What should they be advocating for? What kind of testing is used to get the chronic fatigue syndrome diagnosis and/or the M.E. diagnosis? What should patients be asking their doctors to do?

31:46 Colleen (Guest): The first thing to ask them to do is to look at the International Consensus primer, because that gives a very thorough description of the biological abnormality seen in M.E. It also gives the testing. So M.E./CFS diagnostic procedure is not clear. Different organizations use that label suggest various routes to diagnose. There is some guidance on the CDC website to take a medical history, do a thorough mental and physical status exam, including looking for post exertional malaise, and doing basic lab tests to rule out other causes of symptoms. They suggest sending patients to some specialists to look for other conditions, so it is important, I mean they are pointing out we need to rule out other conditions. The guidance to diagnose M.E. in the IC primer includes taking medical history, same, looking for post exertional neuroimmune exhaustion, and doing basic lab tests to rule out other causes of symptoms. Guidance to diagnose myalgic encephalomyelitis in the primer includes taking medical history, looking for post exertion neuroimmune exhaustion, and starts with the basic lab tests that include vitamin deficiencies like vitamin D, vitamin B12, and CoQ 10. That's very seldom done, and CoQ10 is one of the basic building blocks for energy production. If you don't have enough, CoQ10, and that's something that we can lose as we age or we can lose from a variety of illnesses, just supplementing with CoQ10 can have a huge impact on the energy production system. It also includes additional testing about, like cortisol testing, DHEA sulfate, a chest X-ray, hormone screening, and a panoramic X ray of dental roots. These are thinking outside the box, really looking to see what could be causing all of these symptoms. I have come across patients who have found that they had abscesses in their teeth, and it was causing such debilitating symptoms that they had been diagnosed with chronic fatigue syndrome. Getting their teeth healed removed all the toxins out of their system. You can imagine how sick you are if you're just full of bacterial infection in your mouth, you're swallowing that, you know, it's infecting your ears. So it's so important to have this wide variety of testing to make sure things have been ruled out. The IC primer goes into even more depth. They order lab tests specific to M.E., which include a SPECT brain scan to look at the blood flow in the brain. So we talked about an MRI - looks at the shape and the health of the brain, but it doesn't tell you whether the blood is flowing to all parts of the brain. So a SPECT is recommended in the IC primer. I admit that's very difficult to get done. I have had it done and that information about my SPECT brain scan is on the M.E. International website. But then the other part of the testing that's looking specifically for M.E. is to start looking at the viral load in patients. They recommend viral testing for Antero virus, for Epstein Barr virus, cytomegalovirus, HHV 6, chlamydia pneumonia, mycoplasma, Borrelia burgdorferi, and Parvo B19. It also includes an extensive immune system panel including natural killer cell function. An important aspect of M.E. is that we have low natural killer cell function and it's often seen in people with M.E. It requires a doctor who knows how to order the test, but it's an important aspect of recognizing that when I crash, I am at risk for a lot more bacterial infections, and that has to do with my immune system dysfunction.

35:55 Cathy (Host): The natural killer cells are my absolute favorite cells. OK, so everyone probably knows by this point in the POTScast that I'm a huge nerd. And I teach human anatomy and physiology in my day job. And so when I'm teaching the immune system, I love natural killer cells because they take aim at anything abnormal in your body. So they'll kill cancer cells or they'll kill virally infected cells or they'll kill bacteria, or they'll kill fungus - they don't care. If it's doesn't get marked as your self recognition normal, healthy cell, they'll take it out. So if you're losing that cell or if the function is decreased in M.E., that's a huge problem, because these natural killer cells are amazing and they're really active. It's one of those behind-the-scenes sort of immune functions that nobody really knows about, but is super important. So that would leave you open to more infections for sure.

37:01 Colleen (Guest): Right, exactly. And one of the challenges is very few doctors are testing for that in the under the CFS or M.E./CFS label, they're not even looking in that part of the room. For years I've said my doctors looking in the yard and the fire is in the basement. It was like they're not looking where we need them to look, and so the importance of the M.E IC primer and the testing guidance in there is it's giving a doctor a window into, oh, this is where I'm supposed to be looking to have an understanding of what's happening to my patient. We often have plenty of natural killer cells. So the normal test to order is how many natural killer cells do you have? We have plenty, they just aren't working. So unless you're doing that other test - the function test, which needs to be done in a specific way - the doctor isn't getting the answers that are pointing them to the right area.

38:08 Cathy (Host): So the natural killer cell function is one major physiological change that you see in people with myalgic encephalomyelitis. Are there other physiological changes that are associated with that disorder?

38:23 Colleen (Guest): Yes, and the M.E. IC primer off is quite a bit on this, and so let me just share some of what the primer has stated: The collective pathophysiology of M.E. is multi system and quite distinct. The underlying pathophysiology of PENE involves a profound dysfunction of the regulatory control network within and between the nervous system. So M.E. is a disease of the nervous system as well as the immune system and the energy production. So some of the other changes that are understood to cause symptoms are abnormal physical responses to exercise - we've discussed that. And my experience with that is that my muscles, when used at what would be considered normal, can burn and stop working like someone at the end of a marathon. There's a lot of research happening into what might be causing dysfunction in our mitochondria. So pathophysiology, the mitochondria is a huge component. There is also altered central nervous system signals - decreased blood flow to the brain, which worsens after exercise, altered sensory information and pain processing in the brain, as well as brain inflammation. So for me that is meant over the years I've had a lot of difficulty doing normal tasks that inquire mental stamina, especially if I've been too active. So I do often make a choice between doing physical activities or mental activities. I can very seldom do both at the same time. The altered sensory information means at times I can't tolerate light and I'm always sensitive to being in a noisy environment, and my sense of smell is heightened. When we talk about, you know, what does this do to my lifestyle? We have had to adapt in a major way everything in my environment. My husband can't wear cologne. He showers in a completely different part of the house because I can't tolerate the smell of the shampoo. This is just a peek, a window, into the challenges of living with someone with M.E. who is coping with this pathophysiological altered sensory information processing in the brain. I have also had periods of uncontrollable pain. Medications didn't have much effect, but I did find acupuncture was very effective, very difficult to get – insurance doesn't cover it very well, but I was also able to get a TENS unit. [Transcriber’s note: A TENS unit is a transcutaneous electrical nerve stimulation unit, a device that delivers low voltage electrical current to provide pain relief]. It's a device that you put pads on your body and electricity goes through that and causes a tingling sensation. However, at this time I’ve found low dose naltrexone is brought my pain to a manageable level. [Transcriber’s note: For more information on low dose naltrexone (LDN), see episode 13 of the POTScast with Dr. Weinstock.] So I don't need the TENS unit very often. I haven't needed acupuncture for several years since having low dose naltrexone as part of my medications.

41:27 Cathy (Host): Oh, that's wonderful.

41:29 Colleen (Guest): Another aspect of this constellation of symptoms that's recognized in M.E. is a reduced stages of three and four sleep. So that impacts every aspect of our lives - not being able to get appropriate sleep and not because we're not doing sleep hygiene, it's literally a change in the biochemistry of what makes sleep happen. Something is not working right. Another one is lower than normal blood volume, a decreased capacity to use oxygen, oxidative stress, and increased acidosis in exercising muscles. So sleep is a big issue for most of us, but it's not something that just doing sleep hygiene can manage. Over the years I have found some things that have helped me get better sleep, but it is an ongoing challenge. I did have a sleep study done, that's another thing recommended to have done in the M.E. IC primer, and the doctors report for my sleep study - and I remember distinctly her telling me this over the phone - “your results were quirky.” So I'm not sure how one interprets a neurologist telling me that my sleep is quirky. The results were something she did not recognize. It was different than she expected and she didn't know what to do with that. She did offer some suggestions of increasing my melatonin to a very high dose that I had never heard anybody recommend, and I did increase my melatonin to what she recommended and that did actually have an impact. I would think that would be something that a doctor should be discussing with a patient and not a dosage that one would just go try. I also had severe vitamin D deficiency at that time. It had never been checked. I had been sick for over 20 years at that time. Now I know to watch my vitamin D levels. It felt like I was standing in a rocking boat all of the time. I had to hold on to walls or I had to hold on to my husband to be able to walk. Again, in a building with high ceilings like the church, I found myself very dizzy because everything was moving. Once my vitamin D levels were up, those symptoms went away. So again, this is the importance of knowing what testing needs to be done leads to knowing what treatments may have an impact. The next thing within the physiological changes is the immune system. So, we have talked about some of that, but there's also, along with the NK cell function, we're talking inflammatory cytokines, B cell activation, TH-1 shift towards TH-2 dominant immune response, and all of this leaves patients open to opportunistic infections and reactivated viruses. So getting that testing may lead to getting antivirals, which I did, and that again helped me improve my quality of life. It didn't stop the M.E., but it definitely improved my quality of life. Another aspect of the changes seen in many patients is we don't tolerate medications. We must make sure that we start with a very low dose, that our doctor is aware of the inappropriate or the way our body does not respond normally to medications. And so having a doctor well versed in the recognition of how any changes us is important at all levels of working with the doctor. So unless they understand that danger of giving an M.E. patient a normal dosage of a medication, we look at M.E. patients who are constantly being harmed because of lack of understanding of the basis of this disease.

45:16 Cathy (Host): Once you have gotten a diagnosis, what can be done about it? What are some common treatments that can be prescribed for people with myalgic encephalomyelitis?

45:30 Colleen (Guest): There's a lot and I worry that people go around and get told there are no treatments for this disease. There are treatments. There's no treatment to cure the disease, but there are absolutely treatments to help manage and M.E. The most common treatment is to guide patients to pace activity. At the basis, what we started off this talk about, is that we absolutely have to recognize our energy production on [inaudible], have to recognize that there is no getting around that system that is apparently broken. We need to learn to work within that system. But beyond that, beyond pacing ourselves or beyond changing our behavior, there is quite a bit we can do. The IC primer does offer treatment. After they've gone through explaining what's going wrong, they have a whole treatment section, and it's actually probably more than we can go into at this time. Those treatments are based on the physiological changes we talked about and I would recommend anybody interested in supplements and medications to take a look.

46:39 Cathy (Host): So, Colleen, you have both me which you were diagnosed with years ago and POTS, which is a more recent diagnosis for you. Can you tell which of your symptoms or POTS?

46:51 Colleen (Guest): I think my POTS symptoms really are a downstream effect of M.E. So one of the known me physiological changes is ongoing low blood volume. Having low blood volume leads to the natural reaction of a faster heart rate, especially when sitting up or standing. And because my main symptom is increased heart rate on standing with no other issues, again, it's really important to make sure you see a doctor who understands dysautonomia and makes sure that there isn't something else going on. But for me, my EP cardiologist was very clear. The only issue I was having was elevated heart rate and he said that is a natural reaction to what you're living with, with low blood volume. So I was prescribed Corlanor and that has been a great help to me.

47:43 Cathy (Host): And so for our listeners Corlanor or the generic name is ivabradine is sometimes used for POTS patients, but it's an off-label use. So, I think most people in this community probably know that there are lots of medications that are given to POTS patients, but none of them are approved by the FDA for POTS in particular. And this is another example of that. It's also a relatively new medication, and it's hard to get insurance to pay for it because it's expensive. But it does have FDA approval, it's not for POTS, but it's for heart failure. And so when people have heart failure, their heart is still pumping, but it's not very efficient. So, it's really beating faster is what happens in heart failure, which makes it even more tired and has more energy issues that it's got to deal with. And so this medication selectively blocks something called the HCN channel. And it's a little different. It's actually in a class by itself right now, because it's working in a part of the heart that's called the sinoatrial node. And so, some of our listeners may know that the sinoatrial node is often called the pacemaker of your heart. That's what sets your heart rate. And so in people with POTS, when you're laying down or sitting down, your heart rate is normal, typically, but when you go to stand up, it just takes off, right? That's that tachycardia. Well, this drug, the Corlanor, can selectively block this activity in the sinoatrial node, bringing it back down into normal levels. And when I was doing my research for this interview, I found a study where they actually looked at the impact of this medication in POTS patients. So I was really excited to see that, and they did show that it brought down the average heart rate from 94 down to 77 beats per minute. So that can be a fairly big number. So this is one that I hear in the POTS community, a lot of people really like this drug if they can get on it, if they can get the doctor to prescribe it, #1 'cause it's for heart failure, and if they can get the insurance to pay for it, #2. How did the POTS diagnosis change your treatment plan, because you had been diagnosed and working with the M.E. for a long time? How did the POTS diagnosis sort of change that for you?

50:18 Colleen (Guest): It really was something of a game changer. Just getting the medication ivabradine, and again remembering M.E. people don't tolerate medications well, I tolerate this medication very well. So that was the first surprise is that sure, we're going to try a new medication and it was a lovely surprise. The other thing is that so many of the symptoms that were preventing me from enjoying being within my envelope of energy were all related to the POTS symptoms. So, it was the not feeling good when I stood up, it was having my heart rate skyrocket when I stood up, feeling dizzy when I stood up, some nausea from, you know, that change of feeling when you go from sitting down to standing up or laying down to standing up, or having to lay down all the time and not being able to stand. This is huge impact on the daily quality of life. So the Corlanor helped a great deal, but I was still having a lot of problems and I still was limited, and it was clear that I couldn't hydrate enough. I literally could not put enough liquid in my body and I can imagine a whole bunch of POTS people raising their hand because that is absolutely part of the POTS diagnosis and part, you know, experience. And so the other thing is, now I get IV fluids. So for me that has been a huge improvement in the quality of life and I try not to be angry about all those years I wasn't getting that and imagining how much healthier and better off I would have been all these years later, if I really didn't have enough blood in my system flowing to my brain to be able to function more comfortably in any kind of environment where I had to sit or stand. The key to proper treatments is proper diagnosis, and getting the POTS diagnosis - whether it's downstream from M.E. or not, it's still POTS - getting the treatments for POTS has been a game changer for me. And I do usually get Lactated Ringer's so I know that with some in the POTS community it's just saline. [Transcriber’s note: Lactated Ringers is a sodium lactate solution given intravenously. It includes mixture of sodium chloride, sodium lactate, potassium chloride, and calcium chloride in water and is sometimes given as an alternative to traditional saline IV.] But my doctor has looked at all that I'm dealing with, and when he thinks Lactated Ringers is probably a better fit for me, 'cause he thinks may help offset that oxidative stress better. As my doctor likes to put it, “the solution to pollution is dilution,” and so while it might be helpful for me to get IV fluids more often via port, I have to take into consideration that I may not be able to fight off bacterial infections and I have limited options for antibiotics at this time. So, I'm just getting IV fluids every two weeks and it is making a difference without exacerbating my other symptoms. So, while I have POTS and many of the POTS patients will go in and get some kind of PICC or port line, I really don't see that as an option because of my myalgic encephalomyelitis. [Transcriber’s note: A peripherally inserted central catheter (PICC) is a is a tube that's inserted through a vein in your arm and passed through to the larger veins near your heart to deliver fluids or medication.]

53:22 Cathy (Host): And I want to share with our listeners, if you don't mind, that we set up this interview and we timed it after your IV saline and we set it for the point where you thought you would feel the best in that two weeks, so that you could think, so you could be articulate. I just want to point that out because as I listen, you sound like everything is wonderful. Your voice - you've got maybe that muscle memory, and you're very animated, but you have real physical struggles, neurological struggles, and so I just want to point out that that IV saline does make a big difference, but we're catching you in that window of the upswing. [Laughs] Is there anything else that you would like to share with our listeners today?

54:06 Colleen (Guest): I can't thank you enough for this opportunity to help more patients find their way to better options. This is really about leaving no one behind. In the past, by lumping all these different patient groups together, no one was getting an accurate diagnosis, which means without an accurate diagnosis, patients were mostly given behavior modification recommendations and sent home to wait it out. The expectation for many doctors that whatever we have would self-resolve. Post viral fatigue syndrome that does usually self-resolve and we're seeing that, as the pandemic goes through the country, that people get very sick and it takes them a while to recover. But for the most part, patients are recovering. It just takes time. And so doctors are very good at saying, “Well, you're not feeling well. You have chronic fatigue syndrome or post viral fatigue syndrome, and you just need to go home and you need to rest. And then, once you're feeling better, you need to get back out in the world. You need to start doing more.” And so, making sure that we get the message out that there are distinct diseases that don't self-resolve and that does include often POTS or myalgic encephalomyelitis. And that doctors - we want to make sure that patients aren't being blamed for not recovering. We need to make sure that patients are getting the thorough screening. We just need more education, so I'm so grateful to have an opportunity to, you know, to talk about this from my point of view, from what I've experienced, and I really just want to try to help patients to know you don't have to just go home and wait this out, and it's best not to try to just go home and wait this out. There are things to look into and we don't want to leave anybody behind.

55:58 Cathy (Host): Absolutely, absolutely. Well, Colleen, thank you so much for taking the time and the energy and the effort to participate in this POTScast. I really hope that we help listeners to understand that there is something that may be comorbid with their POTS, that maybe they have myalgic encephalomyelitis or they have chronic fatigue syndrome, and that they can ask doctors about that. So we'll be sure to have in the show notes some of the references that you mentioned today so that people can go and look at those. And hey listeners, we just want to remind you that this is not to be taken as medical advice. If you think that myalgic encephalomyelitis or chronic fatigue syndrome might be in your picture, please discuss that with your own health care practitioner. We hope that you'll click the subscribe button to help other people like you find this podcast. And as Colleen said so eloquently a minute ago, you are not alone. There are many people out there who are rooting for you, thinking about you, and advocating on your behalf. We hope you'll join us again soon.

57:15 Announcer: You can find us wherever you get your podcasts or on our website, www.standinguptopots.org/podcast and I would add, if you have any ideas or topics you'd like to suggest, send them in. You can also engage with us on social media at the handle @standinguptopots. Thanks for listening and we hope you join us. This show is a production of Standing Up to POTS. © 2021 Standing Up to POTS. All rights reserved. [Transcriber’s note: For a copy of this transcript or the transcript for any other episode of the POTScast, or if there is something we can do to make these transcripts easier to follow, please email volunteer@standinguptopots.org]