maggs

Yes, I agree with @MomtoGiuliana . While EDS isn't the only condition associated with POTS that can cause aneurysms, I believe it's the most common. But the most common form of EDS is hypermobile EDS (hEDS), which doesn't carry as high as risk for aneurysms as the vascular type of EDS (vEDS). But really any connective tissue disorder (eg: EDS, Marfan Syndrome) can cause aneurysm and is a comorbid condition with POTS (meaning they commonly occur together). There is genetic testing for vEDS which makes it a little easier to diagnose (hEDS doesn't have genetic testing currently).

@Bailee Without knowing your age, I'm not sure how high of a heart rate is really safe for you. While it isn't entirely accurate/perfect, the typical way your max heart rate is calculated is 220 minus your age. For example, I'm 37 years old so my max heart rate is 220-37=183 BPM. The CDC explains it well here: https://www.cdc.gov/physicalactivity/basics/measuring/heartrate.htm I've had POTS for a decade, and while everyone's experience is different, my POTS specialist told me to start exercise slow and as tolerated, and had me stay around 50-70% of my max heart rate initially. Personally, I found that manageable but difficult while I adjusted to my medication, but in time it got easier. Metoprolol helped, but we swapped to ivabradine once it came out a few years ago and, for me, it's been much better at controlling symptoms; I've been able to increase my physical activity significantly with it. But, again, everyone is different. The couple of other people I know with POTS do quite well with metoprolol. My advice? Ask questions if you're worried- and use the CDC's link as a guide. But ultimately trust that your body will communicate when something is too much. As someone who has done years and years of PT, a good physical therapist will listen and adjust what they're asking you to do based off what you communicate about how an exercise makes you feel. If it's too hard or hurts they should tone it down. If it's too easy they should turn it up. But they always use their initial evaluation and doctor's recommendations to come up with a starting point- and I bet that's what your PT is doing. Just because their starting point/initial plan sounds difficult doesn't mean it will be. But if it is just let them know and it can be adjusted so you can tolerate it. Hope that helps!

@CallieAndToby22 Sinus issues suck! I've had 3 sinus surgeries so far, and assume I'll need more in the future. But I cannot recommend sinus rinses with a squeeze bottle (rather than a neti pot) highly enough! I'm willing to bet I would have needed more sinus surgeries already had I not used it! But I hope you can get some better help soon! If there's any other info I can offer let me know! I do this as a sort of hobby- I've been fighting the medical system long enough that I've learned a thing or two over the years on navigating it all.

@RecipeForDisaster Ah! I've only been to Boston/New England once but fell in love with it! I hope to one day move to Boston. Have you had genetic testing done yet? I did a quick search, and I know Alabama isn't super close to where you likely are, but the University of Alabama at Birmingham also offers an Undiagnosed program separate from the NIH (https://www.uab.edu/medicine/genetics/patient-care/clinical-services/undiagnosed-diseases). And Columbia I'm guessing is close-ish to you, they have the DISCOVER program for undiagnosed patients (https://precisionmedicine.columbia.edu/content/rare-diseases). Maybe one of those could help you? I don't know your personal story or symptoms, but sometimes getting into a place that is interested in the difficult cases is all it takes to start heading in the right direction in my experience. If neither of those locations are close enough to you, I can look for more. I'm much more familiar with what's available on the west coast (since that's where I am), but this has become something I'm passionate about and am happy to dig into so you have more options.

Hi @gertie! I think the GI symptoms could possibly be related to dysautonomia, but if you have that many food allergies have you been checked for mastocytosis or mast cell activation syndrome (MCAS)? Your experiences sound more like that to me... Also, have you had food allergy testing to see if these are true IgE mediated allergies or not?

@CallieAndToby22 I do have Modafinil for really bad days, but it causes tachycardia (as all uppers do), so I can't use it regularly. Mostly I've managed by getting an accommodation at work for a late start and only use the Modafinil when I absolutely have to be up early. It doesn't help with the waking up part (I usually sleep through alarms for hours), but it helps once I'm up with staying awake. I'm hopeful that as I lose more weight some of this will be easier to manage- before my diagnoses my doctors just kinda threw prednisone at me and I gained about 100lbs. Now that things are starting to add up/get figured out I've been able to stay off prednisone and lose about 30lbs. It's all a work in progress. But sleep is so freaking important- it's impossible to feel well when you aren't sleeping (or getting restorative sleep). I wish there was an easier answer out there for each of us. Maybe someday. @RecipeForDisaster I'm not sure where you live, but Cedar-Sinai in Los Angeles has the Center for the Undiagnosed (https://www.cedars-sinai.org/programs/undiagnosed-patient-center.html) that's separate from the NIH. I applied to the NIH's UDP years ago and was denied- from what I understand that's pretty common for adults to be denied (they generally accept more kids, but they are looking for genetic stuff specifically). But Cedar-Sinai operates quite different. They do charge a $500 fee to have their panel of doctors review your case, but after that it's all billed through insurance like normal doctor visits. If my current work-up falls through I plan to head to Cedar-Sinai next. But I will say that my new allergist/immunologist seems to be onto something with MCAS and EDS for me. My old doctors tried to rule it in or out years ago without success, so we'll see what comes of it this time around. There's also resources like Grand Rounds (https://grandrounds.com/) and Crowdmed (https://www.crowdmed.com/) that have been helpful for me, though I'll say CrowdMed can be hard to navigate because it's hit and miss on who participates in your case (random people, med students, retired doctors, etc). Obviously there's no guarantee for quality with Crowdmed based off how it operates, but I still found the input useful. As for Grand Rounds, they were able to get a second opinion for me through a fantastic doctor who made some incredibly helpful recommendations. Anyway, just a thought.

It's amazing how different people with dysautonomia can have such different symptoms! I have a different issue- I could sleep all day and all night every day if nothing forced me to get up. Has anyone else here had a sleep study? Just curious. I've had a few and I'm borderline narcoleptic and have mild positional sleep apnea. Sadly, even when I do sleep plenty I don't wake up refreshed and I could very easily lay back down and fall back asleep, so I can relate to the non-restorative sleep. I'm sorry to hear about what essentially sounds like insomnia. That sounds really rough.

I should add that I work with a dietician that is familiar with POTS/MCAS. She swears by Heather's Tummy Fiber for diarrhea and constipation in POTS (and generally). I've used it on and off over the years and it does help, I'm just terrible at remembering to use it regularly (which is what you have to do for it to be most effective). Maybe that would be worth a try? Her instructions for me were to start with a small amount mixed in water and increase the dosage every 3-7 days as tolerated until my GI symptoms resolved. It's funny how Heather's helps both diarrhea and constipation, just at different dosages/amounts. Also, maybe you could try using, say, Tylenol when you next need it but at a lower dosage than is usual? Like a child's dose? I wonder if that could help you in terms of trying it out without as big a risk to making pain worse. The only other thought I had was palmitoylethanolamide. I don't know if it's been used in POTS patients much, but it's a mild OTC supplement that can help manage pain. My dad has been using it for years and it's the only pain med he can tolerate. I've taken it a few times and have never had a reaction, even when my symptoms have been bad. I know it's tough figuring out how to manage it all, especially in the beginning. But you'll get there in time. Hang in there.

The person that brought up EDS read my mind! There's a common trifecta with POTS/MCAS/EDS, and that combo would explain a lot of your symptoms I think. You're near me, so I'd highly recommend considering seeing Dr. Peng Chen at Cedar-Sinai (he's their POTS specialist) and Dr. John Fagan at Casa Colina's EDS center in Pomona. You could also purchase a neck brace and see if wearing that helps reduce some of your symptoms. I also agree with checking your blood pressures and pulse laying down and standing to see where you're at with those. To clarify, are you able to lay down if it's on your side? Or are you struggling to lay down for any length of time at all? I hope you get some helpful advice soon!

Dysautonomia is kinda a catch-all term used for a category of related conditions. It is a neurological disorder, specifically of the autonomic nervous system (ANS), which is the part of the nervous system that controls automatic functions like heart rate, blood pressure, and breathing. So dysautonomia can include various orthostatic conditions (like POTS), meaning your ANS fails to compensate for things like blood pressure when you change positions from laying down to standing up, for example. Dysautonomia can also cause problems in your GI tract since the peristalsis of your GI tract is automatic; that can cause things like gastroparesis, for example. I'm no expert on dysautonomia, but I'll make an educated guess on your first question. Chronic means it has lasted longer than 3-6 months, progressive means symptoms are likely to worsen over time, and generalized dysautonomia relates to any number of symptoms due to issues within the ANS. Put another way, it is ANS dysfunction that may be lifelong with varied symptoms due to different body systems being affected by ANS dysfunction. I suspect if you do a little reading on what the ANS is and what its various functions are a lot of this will be clearer. The ANS controls all the things our body does that we don't have to think about, which is why dysautonomia can be so difficult. I have POTS (Postural Orthostatic Tachycardia Syndrome) for example. For me it means being sick adds a lot more stress to my body than most people, that I struggle with and might pass out in the heat, that I'm usually pretty dehydrated, and that my blood pressure sometimes gets too low and I could get dizzy and pass out. I have had to make alterations in my life to compensate for it, but it's something I've learned to manage and live with. I used to camp a lot in the summer, for example. But now that is something I doubt I'll ever do again- if I want to camp it will have to be in fall or spring, when it's not as hot out. I also have to make sure I drink a lot more fluids, and have worn a mask during cold and flu season for years to reduce the likelihood of getting sick since my body doesn't handle it as well as it used to. I'm guessing you are asking these questions because you or someone you love has been diagnosed with dysautonomia. I'm very sorry for this diagnosis, but know you aren't alone in it. Some forms of dysautonomia are more common than others, but help and support are out there.

I've had POTS for about a decade, and I don't have much of an issue with most OTC medications. I think it varies a lot person to person, but since I have MCAS as well and therefore a history of ulcers I just try to avoid oral NSAIDs personally (I'm fine with topical like Voltaren gel or injections of NSAIDs at my doctor). I used to have to avoid medications containing sudafed (that would make me even more tachycardic), but since being on Corlanor I haven't had any issues with it any more. Everyone is different, but Corlanor was life-changing for me at managing the tachycardia and other symptoms. This summer was the first summer I bothered trying to spend a little time outside in years (I don't do well in the heat with POTS) and I not only did well, I didn't notice any major issues later (like increased fatigue). But unfortunately with POTS it does mean a lot of trial and error with a lot of stuff (including OTC meds) before you'll know what you can tolerate and what you can't. It sucks playing trial and error with your body, but for me it was the only way to know what I can tolerate and what I can't. It was worth it in the end knowing that most medications don't aggravate my symptoms. Not sure if any of that helps, but I wanted to take a moment to reply.

A YouTuber I follow has had a positive experience with the POTS Care clinic after being misdiagnosed elsewhere, but I'm seen by a POTS specialist at Cedar-Sinai in Los Angeles. Dr. Peng Cheng is his name, and he accepts my insurance. I've been undiagnosed for too long to ever be able to afford a place like the POTS Care Clinic, and honestly my doctors at Cedar-Sinai have done a fantastic job of managing my symptoms of POTS for years now. Not sure if that helps anyone out, but I figured I should share. If anyone is interested, the YouTuber's video is here: