Episode 14: A Winding Road Through POTS, Chiari, Ehlers-Danlos, and Tethered Cord with Dr. Michael Goodkin Link: https://tinyurl.com/5b8vzhue

00:00:01 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.

00:00:29 Jill (Standing Up to POTS): Hello fellow POTS patients and nice people who care about POTS patients. I'm Jill Brook and today we are going to hear from Dr. Michael Goodkin, who is retired from private practice after 36 years as a non-invasive cardiologist. He is double board certified in internal medicine and cardiology and he became interested in POTS after his daughter was diagnosed with the disorder in 1996. He then also treated more than 400 POTS patients prior to retiring in 2016, and he is spending his retirement working tirelessly to improve physician education about POTS to ultimately improve patient care. He is particularly interested in novel treatment regimens for POTS, some of which he'll discuss with us today. Thank you for speaking with us, Dr. Goodkin.

00:01:22 Dr. Goodkin: It's an honor to be here.

00:01:23 Jill (Standing Up to POTS): So, for starters, I just wanted to remind our listeners that POTS can manifest very differently from patient to patient. At one end of the spectrum, a patient may be only mildly affected, and their POTS could be just a small nuisance, and perhaps they're only identifiable medical problem. And maybe just a few lifestyle changes or safe simple common treatments might get them back to life as usual. But on the other end of the spectrum, a patient may have severe POTS as just one part of a much bigger medical picture with multiple interconnected complex conditions. And for these patients, it can be a difficult journey to even just identify all of the issues, much less get effective treatment for them. Dr. Goodkin, does this sound accurate and what has your daughter’s POTS experience been like?

00:02:24 Dr. Goodkin: Her experience is on the bad end of the spectrum. It started out typical. She couldn't stand up, fainting, and couldn't get out of bed to get to school and we didn't know what was going on 'cause as a cardiologist-like guy. She got a tilt test till test, tilt test was actually consistent with POTS and neurally mediated hypo tension. I wound up treating her 'cause there was nobody else. We had a neurologist who had trained at Vanderbilt, but he wasn't much help. She said, “I know somebody who sees this doctor and we went to an expert pediatrician who was fantastic. Diagnosed her with chronic fatigue syndrome. Neurally mediated hypotension. Didn't call it POTS, but it was POTS.

00:03:11 Jill (Standing Up to POTS): So can I just stop you for a second? So you said neurally mediated hypotension.

00:03:16 Dr. Goodkin: Right, so that’s basically... there are some people on a tilt test. Their blood pressure will fall now. A lot of people they feel faint. All those patients, their blood pressure falls. There's some people, their blood pressure steadily drops. I thought what she really had is POTS. The key is orthostatic intolerance. That's what makes people feel terrible. I would say 80% of the people I stood up in the office had POTS if they had orthostatic intolerance symptoms. But a lot of them stood up and their heart rate went up 10, 20, 25. It doesn't mean there's nothing wrong with them, they just didn't meet criteria for POTS. They have a serious problem. Some people, their heart rate and blood pressure didn't move at all, and they couldn't stand up and they had to sit. So it’s not simple, a lot of people fall in the cracks. So she was doing terribly and went to college. She was a voice major. She couldn't stand up for her singing lessons. She went back for second semester, came home, and was bedridden. So just prior to that, the doctor was concerned that she had a thing called Chiari one malformation, which can cause POTS.

00:04:27 Jill (Standing Up to POTS): So what is Chiari malformation? My understanding is, it's a small percentage of POTS patients, but for those POTS patients, it's a really big deal, isn't it?

00:04:36 Dr. Goodkin: Right, and the ones that are most likely to have it are the Ehlers Danlos Syndrome patients. So Ehlers Danlos is when people have abnormal connective tissue. The connective tissue provides tensile strength. It's not normal, so they have joint hyper mobility. They have spine problems, and one of the things that they’re likely to get is chiari one malformation. Back when we had to deal with it, the criteria were that the cerebellar tonsils went down 6 meters millimeters. Now it's 2 millimeters.

00:05:04 Jill (Standing Up to POTS): So just to make sure our audience might have a clue, that means that the base of the brain - is it correct is actually kind of getting squished?

00:05:14 Dr. Goodkin: It's sagging down into the hole where the spinal cord comes into the skull, which is called the foramen magnum and spinal fluid flow gets blocked and there's brainstem issues. My daughter had an abnormal neurologic exam. She also had a special test called a somatosensory revoke potential with her neck extended that localized to the Craniocervical junction consistent that there was something wrong there. So she had the surgery. She was actually better after the surgery. Her POTS wasn't gone, but her orthostatic intolerance was actually better.

00:05:48 Jill (Standing Up to POTS): So I'm sorry to interrupt you, but just to make sure we understand. So is it correct - so the Chiari malformation was putting a lot of pressure on the base of the of the brainstem and the surgery was to release that pressure?

00:06:00 Dr. Goodkin: Right, the surgery involves mainly drilling bone away to make more room for the back of the brain. And in her case it was fairly successful, but unfortunately the surgeon that did it drilled too much bone away and we had complications. Six years later, she's not doing so well. She went someplace else. They diagnosed her with craniocervical instability. Basically, the ligaments that attach your head to your neck are loose. So she had a cranial cervical fusion. She had rods and screws put in. She, prior to that, she had cranial traction when they pulled up on her head. She felt much better. Unfortunately the surgery did not reproduce. Nine years later, one of the screws was up against the spinal cord. They had to take out the fusion, put in a new kind of fusion called the condylar screw fusion, where they stick a screw straight through C2 into the condyles of the skull. I happen to think it's a better surgery, but it's not so easy, and most surgeons are leery of doing it because when you stick the screws in, you can hit stuff you don't want to hit, like cranial nerves and the vertebral artery. Anyway, that surgery was successful. They also put a plate in to cover the big hole in her skull, but her wound kept splitting open and it took three years before we figured out that the problem was that she was adrenally insufficient. Her adrenal glands didn't make enough cortisol. It may or may not have been related to Ehlers Danlos, but it made her wounds not heal properly. It caused the wound to (?), so once it was treated, she felt much better. The last surgery healed fine and she's needed no further surgeries except she has a lot of pain. She was on narcotics. Turns out the narcotics caused her pituitary to be under active. Most people don't know that that a significant percentage of people on narcotics have pituitary dysfunction, so that's been addressed. The problem now, she has a lot of pain and has an implanted pain pump. That puts Dilaudid (Transcriber’s note: Dilaudid is the brand name for hydromorphone) and baclofen, a muscle relaxant, into her spinal fluid. It's much better tolerated than the oral narcotics. There are not a lot of people that are good at doing that. In any event, she still has problems with autonomic dysfunction. Her resting heart rate is high, although she doesn't have POTS, she doesn't really have much in the way of orthostatic intolerance. But her stomach doesn't empty, that's called gastroparesis. That's very common. I saw tons of gastroparesis when I was in practice. I saw a young woman with feeding tubes. Now there are better drugs for it now. And she gets Botox into the pylorus, which is the part of the stomach that empties into the first part of the intestine, the duodenum. It relaxes that, helps her gastroparesis, and her stomach empties better. She gets Botox in her neck because of the pain. So this has been an ordeal. Now, fortunately, most patients won't have anything like this. Most patients will have some trouble, dizziness, lightheadedness, some people will be horrible, be incapacitated - 25% of patients can't work or go to school.

00:09:23 Jill (Standing Up to POTS): Well, yes, so you saw 400 patients. What percentage of POTS patients have it this bad?

00:09:30 Dr. Goodkin: Probably, probably a quarter. And, you know, it depends. At one point I had a referral practice people - I was put on a website and I got people from all over the place. I had all kinds of crazy stories. It's so variable, everybody has a different story. Some people are sick for many, many years. Some people get better. Now, in 2000, my daughter’s doctor and I are talking and he's telling me he had these kids that got physical therapy and their chronic fatigue and POTS was getting better. I said, “physical therapy?” And then a few months later, I took my daughter to an osteopath 'cause her neck hurt and helped her. Couple of months later I'm in the office and I have this guy with POTS and fainting. He's gonna lose his job. It’s so horrible. And I get this brainstorm to send them down. I see him two months later, “Chris, how you doing?” “I'm doing great!” “What?“ “Yeah, I saw Dr. Ballou, he did something. There's something wrong with my neck. I only went back the second time to thank him.”

00:10:28 Jill (Standing Up to POTS): Wait so one trip to an osteopath?

00:10:31 Dr. Goodkin: Yes, that was the first guy. If he hadn't gotten better, I probably would have never sent another patient.

00:10:35 Jill (Standing Up to POTS): So can I just make you back up and make sure absolutely everybody understands what an osteopath is?

00:10:40 Dr. Goodkin: Most of the training is exactly the same as empties and most osteopaths go out and practice just like MD's. I had placed several and plenty of osteopaths in my cardiology group. They did regular cardiology training. They do specialized manipulation and within osteopathy there's a special field called cranial osteopathy which I'd never heard of, and believe it or not, the group that oversees cranial osteopathy is the Cranial Academy. And they have a website, and I would go on the website and find people all over the place to treat people. These are very skilled people who mostly do the same thing.

00:11:16 Jill (Standing Up to POTS): So I'm sorry, can I just back up one second so when you say that he does cranial manipulation, you literally mean that he what pulls or?

00:11:23 Dr. Goodkin: No no, no no. He's not working in your head necessarily. They have fantastic sensation in their hands. They know how stuff is supposed to move and feel. And they have ways of releasing tissue. Sometimes it takes weeks, several treatments, but overtime it tends to get better. So I would say in adults I had about 40% significant responses. That didn't work at all sometimes. Adults respond less well the older you are, the less likely it is to work. The younger you are, the better. So children have a much better response than adults.

00:11:57 Jill (Standing Up to POTS): Did this work even in POTS patients without EDS?

00:12:01 Dr. Goodkin: It works better in patients without EDS. EDS makes it harder to treat because they move stuff, but it doesn't stay. So EDS patients are harder to treat. Children are easier to treat. Some anecdotes, I'm sitting in the nurses station, I hear two nurses talking. “Oh my daughter is 14. She has POTS. It's doing terrible. She's on six drugs and she's being seen at a certain place.” So I talked to her. What happened?” “Oh, she had a whiplash.” “That’s fine. You need to go see my guy.” I saw her three months later. “What happened?” “Oh, she got treated. She's 85% better. She's taking no meds.” So for parents of children, I obviously have been through this had a kid that was sick, didn't know about any of this stuff back then. But for kids especially that are moderately ill, the sickest kids, I don't think respond, but some of the kids will dramatically change their course. So we think injury in kids or biomechanical stuff is a significant factor.

00:13:00 Jill (Standing Up to POTS): Is the hypothesis that it's pressure on the brain same as in Chiari or?

00:13:06 Dr. Goodkin: No, no it's not that simple. I don't know, I couldn't tell you how hurting your shoulder or your back turns into POTS. I don't, I don't understand it. Probably some of what the osteopaths do involves the vagus nerve. The most common site of injury that we found in adult patients with sacrococcygeal precedents - 75% of patients at sacrococcygeal issues. So we think POTS from pregnancy is from biomechanical changes in the pelvis. So nobody would think that, right? We had a couple of patients getting dramatic responses. So this is totally different stuff. Now, exactly how this affects the vagus and these nerve complexes that are lower in in the pelvis and the coccyx, I can't tell you.

00:13:53 Jill (Standing Up to POTS): You and could I trouble you to just remind any listeners what the vagus nerve is.

00:14:00 Dr. Goodkin: There are 12 cranial nerves that come out of the brainstem. It's the lower part of your brain. And there's two - one on each side - with the vagus nerve as number 10 and it comes down both sides and it's really important for health. People with POTS have under active vagus nerve, so the vagus nerve is this parasympathetic nervous system. The sympathetic nervous system is adrenaline. And those nerves are overactive in people with POTS.

00:14:27 Jill (Standing Up to POTS): So they have too much fight or flight, not enough rest and digest. Is that right?

00:14:32 Dr. Goodkin: Exactly, exactly. And so there's all kinds of things written about the vagus nerve. So now my daughter has an external vagus nerve stimulator. When she's sympathetically overactive, she puts on the vagus nerve stimulator for, you know, 20-30 minutes. Things get better. I have a guy living with me who has Ehlers Danlos, POTS, mast cell activation syndrome, and he uses it also. Now, they both got it from chiropractic neurologist. He's figured out that POTS is from abnormal sensory processing. Sounds, smell, hearing, touch, and gravity. And he uses an external vagus nerve stimulator. Special eye movements called saccades, a tuning fork. It's taken them years and years and years to figure this out. In all truth she didn't get much better, although she uses some of his exercises. He gives you autonomic exercises to do so. Unfortunately, he usually sees the worst of the worst. And the people that refer to him are people that see a lot of Ehlers Danlos - geneticist and a neurosurgeon who does lots of EDS work. And they send him a lot of his patients. He treats people 4 hours a day, five days a week from one to three weeks.

00:15:52 Jill (Standing Up to POTS): And is he trying to retrain the nervous system?

00:15:55 Dr. Goodkin: Basically. I mean it's still more complicated than that. He's has very profound knowledge of this stuff.

00:16:01 Jill (Standing Up to POTS): So to make sure that I understand and our listeners understand - so if POTS is a problem of sensory perception, you had mentioned the gravity and that one had caught my attention because I, I think of POTS as a problem handling gravity. And so you're saying that maybe the body actually misperceives gravity?

00:16:17 Dr. Goodkin: Miss perceives gravity and a lot of other senses. The same sort of phenomenon is present in autism. They don't talk, they get a lot of noise. They react inappropriately. So some of that is going on in POTS, and it probably has a major effect in at least half the patients he sees. These are people that nobody can fix.

00:16:36 Jill (Standing Up to POTS): Wow, if you're listening to this, I hope you're thinking, “Wow! We are lucky to have such a passionate physician on our side.”

00:16:44 Dr. Goodkin: Osteopathic manipulation is powerful. I'm not talking about backs and knees and hips. And again, for especially the EDS patients, stuff is constantly going out of place because everything is too loose - their neck and shoulders and ribs and stuff like that. So my daughter and the guy living with me both get treated every two weeks.

00:17:06 Jill (Standing Up to POTS): Is there any risk?

00:17:07 Dr. Goodkin: No.

00:17:0 Jill (Standing Up to POTS): So, worst case scenario is it doesn't work for you?

00:17:11 Dr. Goodkin: Right. So there's two indications: Number one is to fix your autonomic dysfunction. Number two if you have pain and stuff is out of place, especially if you have EDS. So not many people know about this. This is powerful. Now one dimension, and the other thing I'm working on: exercise training. So exercise training is obviously indicated. The problem is there's no place to do it. The treatment is supposed to start up for six or eight weeks to be recumbent bicycle, rowing machine, swimming - that's the standard stuff. Recommended by Ben Levine. Recommended by Children's Hospital of Philadelphia. Now there are more sophisticated programs. Now, my daughter and the guy that lives with me, they're both on a waiting list, putting an implantable implanted vagus nerve stimulators. So it's last December from the international meeting, Kevin Tracy is a neurosurgeon by training, but his life has been about inflammation, discovered that stimulating the vagus nerve knocks out inflammation. They've taken patients with refractory rheumatoid arthritis and ulcerative colitis and put them into remission by stimulating their vagus nerve four or five minutes a day. So they put a little tiny thing on the right side of the chest - the little battery - and attach it to the vagus nerve. Now the vagus nerve is tiny. I'm sure they're good at doing it. He's a neurosurgeon, so I'm sure he's good at dissecting down and finding the thing. They're going to start treating POTS, mast cell activation.

00:18:48 Jill (Standing Up to POTS): Right. And, you know, just quickly, since we've mentioned mast cell activation a couple times, do you want to give a quick description of what that is?

00:18:56 Dr. Goodkin: So mast cells are a type of white blood cell in the body. It's actually there to fight infection and people with mast cell activation syndrome in response to certain stressors, sometimes eating, anxiety, whatever - they dump their contents. Now one of them is histamine, so mast cells are involved with allergy. And sometimes there's a lot of chemicals inside mast cells, so they get dumped out a lot of things can happen.

00:19:21 Jill (Standing Up to POTS): So all kinds of different inflammatory or allergic type reactions, right?

00:19:27 Dr. Goodkin: The guy that lives with me has horrible mast cell activation syndrome. So normally mast cell activation patients are on famotidine, which is Pepcid. It blocks H2 receptors on mast cells. And they're on Zyrtec or cetirizine, which blocks H1 receptors. They're antihistamines, so they work pretty well. Cromolyn sodium stabilizes mast cells that line the GI tract. Allergists run into a lot of POTS. They just wind up with allergists. I think they're clued in, and a very high percentage have antibodies to G coupled receptors. So people are trying to treat that with IVIG. Now, my daughter is on IVIG, which is pain in the neck. She has a portacath in.

00:20:16 Jill (Standing Up to POTS): And just for listeners, IVIG is intravenous immune globulin.

00:20:19 Dr. Goodkin: Globulin, right. So, it's five days a week, 4 hours a day, every four weeks.

00:20:26 Jill (Standing Up to POTS): And you're getting infused with a whole bunch of donor antibodies.

00:20:30 Dr. Goodkin: Exactly. So the nurses come to the house and...

00:20:34 Jill (Standing Up to POTS): It's kind of a big deal with lots of side effects.

00:20:37 Dr. Goodkin: Well, she doesn't have really much in the way of side effects. She thinks it works to some degree. Some people there's very variable responses. I don't think this is going to be the answer to POTS.

00:20:48 Jill (Standing Up to POTS): So, you have been on quite an odyssey of researching and digging up every conceivable safe therapy that might help your daughter.

00:20:58 Dr. Goodkin: Right. Now, the biggest problem in POTS is that people can't find a doctor.

00:21:03 Jill (Standing Up to POTS): That's a lot coming from you because I think maybe some of our listeners have always assumed that if you're just a patient and you have no connections and you're not medically trained, that everything is harder. So the fact that you, as a physician who's, you know, double board certified, that you still had trouble finding good, good, knowledgeable physicians - that's something.

00:21:26 Dr. Goodkin: I had trouble, and after I thought I was one of them that I was seeing - had seen - a lot of patients. I started to think I knew what I was doing. Although I looked back and didn't know as much what I was doing as I thought I did. Half the problem is it's hard to find somebody that understands POTS, that can talk the language, that understands what orthostatic intolerance is - that you can't take a shower and you can't stand in line and you’re ready to faint. You run out of line. You can't go to a concert. They don't understand. Obviously I understood because I lived with somebody that had it. And what I found is that I worked in my last 12 years in a 96 man cardiology group. My guys didn't know much. My electrophysiologist weren't that good. First of all, your chance of being diagnosed by your primary care physician is about 9%. So what happens is you have – everybody who has palpitations, they’re fainting and lightheaded, they get sent to cardiologist. And it's always the general cardiologist. The general cardiologists aren't very good at POTS. They usually don't stand you up in the office to look for it. They're not confident at all with medications, so they're going to miss the diagnosis sometimes. Usually they'll wind up ordering a tilt test and then it shows up on the tilt test. And then what they usually do is send you to a cardiac electrophysiologist. So there are about 3000 cardiac electrophysiologists who actually see any POTS in the United States. There's probably about 600,000 adult patients. So that's not going to work out very well. You could do the math - 200 patients each. That’s not gonna happen. Electrophysiologists, I think if they had their choice, they would see no POTS. Well, because it's really hard. You can't fix anybody we don't have curative stuff at this point. You give them drugs, you give them Florinef (Transcriber’s note: Florinef is the brand name for fludrocortisone) and midodrine, and I used a lot of amphetamines. Most cardiologists won't use amphetamines. Amphetamines were my best drug. You tell them to exercise, and they're still not doing well. So it's very frustrating to treat POTS. I hope we have better stuff down the line - vagus nerve stimulators and some people will hopefully feel better with osteopathic manipulation, but a lot of people, you know, just can't fix them. No doctor likes treating stuff that they start out, “I can't fix this.” They’ll do all the stuff and it doesn't work. Problem is, general cardiology needs to take this over. So I've been writing to the president of the American College of Cardiology for years. I got one of them to authorize a review article which was published in March 2019 in our big journal, Journal of American Cardiology. It was a good article. The problem is general cardiologists didn't get any training at fellowship because electrophysiologists are supposed to take care of it. They don't get any training while they're working, and nobody told them you need to know something and be able to take care of these patients.

00:24:14 Jill (Standing Up to POTS): Yeah, you know, it's interesting from my perspective as somebody who took 17 years to get a diagnosis and during that time I felt crazy and my self-esteem you know really went down. I don't think that maybe doctors realize how much it would have helped me just to have a diagnosis, even if they couldn't do anything further.

00:24:32 Dr. Goodkin: So I know when I was on a website. I have all these people come in, you know, I would know right away all the right questions they ask. And “yeah, yeah yeah yeah,” of course then I couldn't fix them. They were expecting a miracle, which was hard. Now I think it's a little more likely that I can come up with something. I think in the next five years we'll have stuff. IVIG helps some. Now, I know cardiologists are never going to order IVIG. So one of the problems is that the American Autonomic Society is very small. It's like 200 people. How are they gonna take care of a million patients? So you have 3900 pediatric cardiologists. You have about 30,000 adult cardiologists. And within cardiologists, about 3000 cardiac electrophysiologists. Well, there's probably 400,000 kids and 600,000 adults. There's just enough people so it would be good if primary care could do this, but I don't think it's going to happen. Cardiologists are the only ones that are going to handle the drugs for orthostatic intolerance. Neurologists are not going to handle it, but everything else should be handled by neurology. There are 18,000 neurologists in the United States. Neurology needs to teach their guys. There's about the same number of POTS patients, as there are multiple sclerosis and Parkinson's. Could you imagine a neurologist that didn't know anything about multiple sclerosis or Parkinson's? And I'm sure that people listening to this, they've minted neurologists. They know they didn’t know anything about POTS. It's a neurologic disease. It's a disease of the autonomic nervous system. Some of it's from injuries, some of it's from autoantibodies, some of it’s from autoimmune diseases, and probably some other things we don't even know about. But it should fall to them. So if anybody is going to order IVIG, it should be a neurologist. Now my daughter happened to find a good pain neurologist to order it.

00:26:21 Jill (Standing Up to POTS): So is something like a simple stand test taught in medical school.

00:26:26 Dr. Goodkin: I don't know. I mean, cardiologists know that it exists, but I probably did a couple thousand standing tests. I had a very low threshold to stand people up. Anybody young that came up and no matter what they had, I stood them up. It's interesting – POTS patients like to lean against something when they stand up. I would make them stand in the middle of the room. They hated it. But that's what you're supposed to do. You can't let them cheat on the standing test.

00:26:50 Jill (Standing Up to POTS): But that seems like something so easy that any doctor could do.

00:26:54 Dr. Goodkin: They should do it. So my experience, when I didn't have a referral business, was that I would see a new patient every three or four months. They would just walk in the door, because POTS is really common and it's missed. And some of the patients that are not really sick, you can treat successfully. So I have patients on all kinds of stuff who I've heard from recently. One her treatment regimen is intermittent IV fluids, Procrit - Procrit, by the way, works great. (Transcriber’s note: Procrit is usually prescribed to treat anemia.)

00:27:23 Jill (Standing Up to POTS): That raises red blood cells?

00:27:27 Dr. Goodkin: Right. So what we what we would do is we would check people for iron deficiency. About 60% of the women are iron deficient. They get treated with intravenous iron, because oral iron doesn't work, it makes them sick. So intravenous iron. I know that they usually feel significantly better just from the iron. And that's the experience of hematologists. You treat iron deficiency with iron. The patients feel better independent of their hemoglobin changing. Once we knew there weren't iron deficient, then we'd measure a red cell mass in plasma volume, and if the red cell mass was down by more than 10%, we would treat people with Procrit. I’d say we probably treated 75 people. I worked with the hematologist and it was completely unpredictable. My guess is 60% of the patients had a significant response. Some of the ones with really low red cell mass down 45% did not respond, and some of the ones down 10% did great, completely unpredictable. I had one pathology resident - she couldn't stand up to do an autopsy - wound up on Procrit. She’s doing great. So I never knew what was going to happen. Now, the problem with that is my hematologist was concerned. There's safety issues with Procrit, although almost all the safety problems are with dialysis patients and cancer patients with blood clots. In young healthy women it’s not an issue, but most people are not going to risk it. There's too much risk if you get one patient with that, 'cause something goes wrong, have problems. So I don't see that being a big thing.

00:28:51 Jill (Standing Up to POTS): Yeah, so I mean, the overwhelming message that I'm getting is that there's a lot of very promising treatments out there that just need the research funding to collect the data and that things really could be closer than we think for a lot of POTS patients.

00:29:08 Dr. Goodkin: Well, now my daughter had a condition that hardly anybody knows about, called tethered cord. So in tethered cord the spinal cord is pulled out too tight. It causes terrible bladder symptoms, low back pain, and pain in your legs. The operation is simple, you go in, you cut the fibrous attachment that holds a spinal cord down. It springs up. I've had people with POTS have their POTS go away after spinal cord tethering. In fact, the guy that lives with me, Josh, hoping he has tethered cord and get a surgery to relieve a lot of his symptoms. He already had a craniocervical fusion. Some people with EDS are the ones that seem to have it the worse.

00:29:45 Jill (Standing Up to POTS): So, do you have any advice for patients or their parents who might be listening now who suspect that they are one of these more complex patients?

00:29:56 Dr. Goodkin: The EDS diagnosis is not so simple. Even genetic testing for the most common form of EDS, they haven't located the genes. Hypermobile is the most common type. Genetic testing will show up all the other types. I think you just try it, I mean osteopathic manipulation is easiest in to treat. It's not going to fix your autonomic dysfunction, most likely. Might get rid of your pain. Pain is actually the most common problem in EDS patients. Joint dislocations are big problems. A lot of physical therapy is needed.

00:30:29 Jill (Standing Up to POTS): OK, so if there are any physicians listening to this interview, what do you hope is the main message that they will take away from this?

00:30:38 Dr. Goodkin: Have a low threshold to stand somebody because it doesn't take long. The people with POTS - t standing test is supposed to be 10 minutes - people with POTS, their heart rate goes up in two or three minutes, sometimes shorter than that. Once it's up, you don't have to keep them standing up. Once the diagnosis is made, you let them sit down. You don't have to make them stand up till they pass out.

00:30:59 Jill (Standing Up to POTS): And I can't resist giving a plug for standing up to pots because the our Charter is to raise funds to support research that helps today's POTS patients. Not basic research that will help people 30 years later. Well, Dr. Goodkin, I can't thank you enough for being a warrior out there on all of our behalf and for working so hard for all of us and for sharing your experience and lessons with us today. I feel like I want to give a shout out to your daughter with best wishes and gentle hugs for all she has endured. And you have been on a quite a unique journey, and I feel a great deal of gratitude that you are working so hard to make it mean something for all of us.

00:31:44 Dr. Goodkin: Well, if I can do something to help...

00:31:47 Jill (Standing Up to POTS): Yes, well thank you Dr. Goodkin. And to any listeners who are feeling overwhelmed by the huge amount of information and medical terminology in this episode, we have other episodes that go into greater depth explaining many of the conditions, Ehlers Danlos, for example, or other comorbidities and treatments mentioned in this episode. And our website, standinguptopots.org is also a great place to catch up on all this. So thank you, Dr. Goodkin, and I hope you'll join us again in the future and update us on how quest for more answers is going.

00:32:20 Dr. Goodkin: All right. Thanks a lot.

00:32:18 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. For a copy of the transcript for this or any other POTScast episode, please email volunteer@standinguptopots.org