RecipeForDisaster

Good luck! I really want to try a vasodilator for my HYCH, but my BP is so low that no one wants me to try it. I don’t have a particular time of day that is worse, but I sleep very poorly.

My renin is always high. The rest are within normal limits. I’m told that is because of my low BP - the renin is trying to compensate?

Congratulations! What worked for you?

Yes, I feel awful after eating even a small amount. It makes me dread eating. Not much helps, other than IV fluids, which make me want to eat.

2 hours ago, Caterpilly said:

Oh didn’t even think to try grubb as a possibility but that would be amazing if that ever happens. Maybe I will give that a try. I recently tried Chemali at Cleveland clinic. His staff said I could email him all my info and give my desperate plea why I can’t travel to him and all that stuff but he didn’t care. He was just like he has to see me in person, which he totally does not. And at a minimum he could’ve at least said if I already had all the testing he does or not. It’s the main thing I wanna know, like if I missed anything. After almost 30 years, I’ve had and tried a lot.

oh OK yes autoimmune SFN diagnosis would get you IVIG. With regular SFN There’s nothing they’ll do for me besides from offer medication like gabapentin that I can’t tolerate. And yea Dr Novak sounds like he’s doing great things. It really does suck now even in the age of TeleMed that everyone still can’t access all these good specialists and it’s just luck of the draw based on where you live or if you have the ability to travel and people to care for you, which I do not. CHOP in Philadelphia did a TeleMed at least with me, because I’m still trying to figure out what the mitochondrial dysfunction is from but no diagnosis there yet either. But TeleMed was all I needed. They would not have been able to help me in person so I’m glad they at least did that.  I wish more places would, 90% of the time TeleMed is all I need. 

Glad Dr Novak was able to get you that diagnosis because I know how infuriating and devastating it feels when you know something is wrong and regular testing is not showing it and no one believes you. It’s like one of the worst parts of all this. You at least want to know what’s taking away your functioning. 

anyway, thank you for the kind words and information, I appreciate it. Oh, and what’s sequential compression? Are those like the blood pressure type cuffs that you put on your legs that squeeze? I do have those…

Oh, and what’s sequential compression? Are those like the blood pressure type cuffs that you put on your legs that squeeze? I do have those…

And what type of vagus stimulation do you use? I recently got a gamma core… but my nerves are so sensitive. It seems like it might be increasing some of this weird sensitivity I get in my face/head and neck and some Head/neck/shoulder pain, but I’m not 100% sure so will have to experiment more and see if I  consistently get these issues.  sometimes they said they will go away the more you use the device… Which, if that’s the case, I would want to keep trying. I just don’t want to give myself more, long-term chronic pain or sensitivity issues so not sure how much I should push it yet but we’ll see… Have you noticed improvements with it? And after how long of using it? Just out of curiosity how long are your sessions are each day?

Having diagnoses is very nice, although I am still missing a lot of explanations for what is wrong with me. And, HYCH isn't even known by most doctors, and the diagnoses I have would not be considered a big deal by most people. Yet I’m in really bad shape overall.

I completely agree that for the most part, there is not really a lot to see, and we could be seen via telemedicine for most visits. It stinks to spend half a day traveling to Boston (with help) for no good reason  

Yes, they are the inflation cuffs that go all the way up your legs. They improve my circulation and BP.

I have put together ear clips and a TENS device for my tVNS. I haven’t been using it, but I should. It was an hour per night. I stopped because I was not sure I was getting much benefit, but it definitely wasn’t hurting anything.

2 hours ago, Caterpilly said:

Hmm.. yeah that’s interesting and might be true. These places like to push it fast in like an hour or maybe an hour and a half and I was getting just 1 L. My veins suck though, so between that and finances, my experiment had to end anyway.
 

 I think they got ruined after chemo many years back and now just keep getting weaker as I get older, so I was running out of viable veins and a picc and port would not be a good idea for me, midline would be lesser of evils but still not good. During chemo they broke the picc line guide wire in my arm and the metal got lost and then they found it a crumpled up ball inside. It sliced through my vein, attached to my nerve, and I had to get surgery to remove it later on.  now I have permanent sutures in there,  it was a whole mess.  And it hurt the whole time. I had it . so unless I really needed this and it worked, ha I’m not jumping for any lines. Plus, it’s just too expensive since my insurance won’t cover it. So I was hoping to just trial it to see if it could get me back to the baseline that I lost the last year or two.  Or if it’s something I could use to get me out of crashes… But not sure. And Me being so sick and homebound now the midline would be a lot to deal with and I don’t have anyone to help take care of me. But… all that said, if I had to revisit it later, and I was giving myself the infusions at home through a midline… Then I guess I could adjust the rate and amount myself.

I can’t have a PICC or port, either. I get a new IV every time, although I can usually keep it for 3-4 days. It stinks, but I’m glad I can do it myself at home.

4 hours ago, Caterpilly said:

@RecipeForDisaster

OK, thanks, good to know how you can still be symptomatic lying down as well. Some doctors make you feel like you should feel miraculous and that’s not the case and I hope Dr. Novak figures you out as well! and OK he was not the one who did the IVIG, that is so hard to get. I don’t know anyone by me that would do it experimentally. But that’s good He was at least trying other things with you. I still have found nothing after 29 years it’s just beyond devastating.  if I could at least tolerate medicines better maybe I would’ve hit on some thing, but not tolerating a lot of meds makes it more challenging too . There’s not too many options for us as it is but my body like rejects everything. And then the things it does tolerate just don’t work. 🤷‍♀️

I heard that Dr Blair Grubb may be doing televisits again. I’d try for that for sure. 
My neuromuscular specialist at home orders my IVIG, because it’s technically for autoimmune SFN, which he diagnosed. I do like that I don’t have to reach him in Boston. It would be so great if everyone could see Dr Novak easily - he really is a pioneer. Even getting a weird diagnosis of HYCH was something other than repeatedly being told I don’t have POTS: I knew!

Don’t give up - there are so many options out there! I have a whole room of devices like sequential compression, transdermal vagal stimulation, lots of compression gear… plus a giant box of meds, some for every day, and some to bail me out at bad times.

4 hours ago, Caterpilly said:

Oh, interesting about the time of day patterns and possible auto immune relation. Also, yeah, I probably have CFS too… That’s always what they said as a kid but I figured they said that because I just didn’t test positive for anything else, but I hate accepting that diagnosis because it’s such a dead end sadly. It’s a shame how research hasn’t really moved or treatments in decades and there’s still so much dismissiveness. But I get horrible posted exertional malaise from the littlest things and never recover so it’s probably a big part of it. and exercise, even going low and slow, anything done consistently always makes me worse and I lose functioning, long-term or cause permanent chronic pain areas so it’s really messed up.

and yes, funny you mentioned about the IV Saline, because as part of my year of expensive last ditch efforts, because everything feels pretty close to over at this point after almost 3 decades… The only way I could access them was by paying for those expensive mobile IV places. I tried about seven IVs I think in total, saline plus various vitamins and supplements, I also have mitochondrial dysfunction, so I didn’t know if it would help from that perspective, either… And gastroparesis… but I really couldn’t tell if it was doing much. Sometimes a few days after the IV I might’ve been slightly less faint and a little more “pushable” but it didn’t really seem consistent so I don’t think it really did much sadly. Other people say it works wonders though, so you never know.

My bet is you had less effects from IV fluids because they ran too fast(ant a normal rate like over 3-4 hours )and/or you didn’t get enough. I need 2 liters run very slowly. I have it at 80mL per hour right now.

1 hour ago, Sarah Tee said:

@Caterpilly, my symptoms are not exactly linked to being upright. Mine run according to the time of day, and once they start, lying down doesn’t make a big difference. I do feel worse if I stand still while symptomatic, of course. (No headaches.)

There is research into autoimmune diseases showing a daily pattern, so I just figure that’s what is happening with me.

There has been some research on patients with chronic fatigue syndrome who have low cerebral blood flow showing that it did not resolve immediately after they were lowered down to flat again from tilt table testing. So although being upright is important, it obviously isn’t the only factor.

I think many folks here feel unwell on waking or wake up during the night feeling unwell. I go through phases of feeling shocking when I wake up, or feeling okay until I eat.

***

Change of topic:

Have you ever managed to get IV saline, and did it help?

I don’t feel perfect when I lay down, either. I feel awful when I’m trying to sleep, and when I have eaten, and sometimes I don’t get better when I lay down during a crash. I can still have BPs in the low 70s while flat.

6 hours ago, Caterpilly said:

@RecipeForDisaster

oh ok, thank you. Bummer! But good to know. Has he been able to identify your underlying cause? I’m curious what battery of tests he orders and if I already had everything done anyway. Well, except for the Doppler thing, I don’t know if they do that anywhere else around here, haven’t had that. 

it stinks because he sounds like the type of researcher I would need to be around to maybe still have any hope for my case after almost 30 years…especially If he sometimes can experiment with different things like IVIG , etc. Even being nearer to NYC, no one good with this stuff is out there I wish someone would just take interest and research me because I feel like there’s a lot to learn there lol I’m like my own longitudinal study 😂.  

Nope, he has theories and has diagnosed me with HYCH, but it hasn’t changed my management. He isn’t the one who ordered IVIG nor IV fluids. He has tried plenty of other meds - some of which have been helpful. I still have hope that he will figure me out.

He does a battery of autonomic testing, including SFN biopsies, QSART, TTT, and cranial Doppler, plus Washington University lab panels.

9 hours ago, Caterpilly said:

Yeah, it stinks that they’re not able to ship the stat device everywhere yet. But hopefully that will eventually happen once it’s perfected. 

Oh, that’s interesting your form of low cerebral blood flow is caused by vasoconstriction. I didn’t realize that it wasn’t just low blood volume or blood pooling. What are your most prominent symptoms? Do you still feel faint and lightheaded all the time? Do you get a lot of headaches? Also, do you ever feel faint or light head lying down and after waking from sleep?
 

This symptom puzzles me the most because one is supposed to feel better lying down, but I’ve been feeling so faint, weak, even lying, especially when I wake up. I always wake up feeling drugged like I’ve had anesthesia, very lightheaded and dizzy, but now the added faintness and shakiness is. bizarre. this is why I’m curious if the stat device will tell me if I have low blood flow even when I’m lying or sitting. For me, I still think I get orthostatic symptoms even sitting, I feel so drained.
 

i hope Dr Novaks research can start to make some headway for us all. I didn’t even know about those two new conditions he discovered until recently. My autonomic doctor in NYC never mentions anything. I’m in New Jersey but I’m homebound and disabled so I can’t travel far and having trouble just leaving the house even once every few months this year. It’s gotten so bad. I’ve been sick for 29 years now, only getting worse, intolerant of most meds or the ones I tolerate don’t work. So I literally have no treatments to take.  So it’s just so challenging and scary cause it feels like I’ve run out of time. And being next to NYC you think there would be studies available or good researchers like Dr. Novak but there’s not. I’ve had so many doctors say try IVIG at this point, but no one actually can do it. I have Medicare and you need very specific diagnoses to get it here. None of the doctors who actually can prescribe it will do it since my case is still just a mystery. POTS, small fiber neuropathy, gastroparesis, mitochondrial dysfunction, etc isn’t enough.  I wish I could see Dr Novak but Id never handle or recover from the trip at this point 😞

did you have to see him here in America to get diagnosed? Was he able to TeleMed with you or were you able to get your diagnosis locally? How do they see the vasoconstriction part? Or low blood flow? Just curious if it’s only a special test done at research institutions or if it’s something you can get locally. 

I see Dr. Novak. He doesn’t see patients remotely outside Massachusetts - and very rarely even then. I also think he said he was not taking new patients. He diagnoses this with cerebral Doppler.

On 1/3/2024 at 5:51 PM, MikeO said:

wish you the best.....mike.

No i have been turned away from the neuro folks.

That is the type of specialist I see for my IVIG. He’s pretty good. He doesn’t really manage my hypotension, but I have so many other doctors. If I just had him, he probably would.

19 hours ago, MTRJ75 said:

I think LDN has made the biggest difference in my case. Thankful neuro was willing. It at least got me from housebound to be able to occasionally leave the house now. Still having significant problems with exercise though. 

That is so great! I hope many others get to try it. It didn’t help me, but that was about 8 years ago. I tried all different dosages.

23 hours ago, MikeO said:

Glad to hear you neuropathy has improved. 

I’m glad I am not doing further damage to my nerves. But this one was not in my top 10 problems, and the IVIG isn’t fun. I don’t feel like I have a choice, though. I’m too young to have this kind of progressive nerve damage.

2 hours ago, MikeO said:

Good question. please post back if dysautonomia international gets back to you. i know the folks in the state i live in get the IVIg infusions and it's not just POTS folks (nOH and OI people as well) They don't get the infusions out of the blue and need to test positive for some immune tests (i see ANA come up frequently).

Some folks respond and others do not.  

Positive ANAs in 2 different ways wasn’t enough for me to qualify for IVIG. My autonomic issues and HYCH didn’t help, either. I had to have a positive SFN biopsy, and I think my abnormal FGFR3 result also helped. I was never offered any kind of DMARD or other biologic, but then I could not find a reasonably helpful rheumatologist, either. It took years to get started, and it’s expensive and not enjoyable, but at least it’s helping my neuropathy.

9 hours ago, little_blue_jay said:

Is this product still working for you?

If so, how often do you use it, and have you a link to the item please? 

I haven’t used it in a while, but I need to try again because there are good studies. The big city neurologist said I’m too sick for it to help me at this point. 
 

any TENS machine will work. Here are the earclips. https://www.tenspros.com/black-ear-clip-electrodes-EAE01.html?gad_source=1&gclid=Cj0KCQiA1rSsBhDHARIsANB4EJb-x0IbovkQ1B5VDCEI8Si8Xv3Z_P9Rtd60OD91a5ut6hisAtE_TxYaAsVCEALw_wcB

8 hours ago, Amyschi said:

So glad you can get them at home.  True, even if my HR isn't super bad, could still use it.  I can relate to being in that state at times, mostly bedbound with weakness.  It is so scary now, as I am 64.   May I ask your age?  If not, its OK!  I am hoping to start a thread on dysautonomia in older age.  I'm imaging there are not too many of us, but I am curious.  At my age, you start getting more into the population that has PAF or MSA, sadly, I would imagine.  I developed my dysautonomia problems in my mid-40s and really noticed it in early 50s at the time of menopause.   I imagine most of you started much younger, unfortunately.

I don’t have POTS, and am on 2 different beta blockers (day vs. night), so my HR is rarely that out of whack. I’m glad they aren’t using that for a metric - I once routinely got to a HR of 180 walking, but not now that I’m stabilized on meds.
 

I’m 43, and I don’t have much weight cushion, either. I was really thin until I had to start meds for trigeminal neuralgia, which put 20lbs back on me, and I’m almost a good weight again. I’ve been sick for about 15-20 years, but had some of this all my life - I just thought it was how everyone felt, and believed that everyone passed out sometimes.

11 hours ago, Amyschi said:

Glad the IV fluids help you!  Do you have to go to a center for this after the doctor orders?  I'm wondering if they would order :just: for loss of appetite if your vitals are maybe not severe enough to necessarily warrant it?  I'm more interested in the sensation here - I suffer from digestive upset and it is always hard to eat, but I have always been able to.  But this is more of an "aversion", as I said - just curious if anyone relates to that in specific.  Thank you!

I have homecare orders to get fluids whenever I need them. It took a lot of work to get it arranged, but I feel so much better knowing I can get them at home when needed. It wouldn’t just be because I can’t eat, but for a whole slew of problems to include that. Once I did not eat for 5 days, because I didn’t feel like I could, and was waiting until I got hungry. I never did. But I also could barely sit up, talk, etc. in those cases, I am definitely food averse. I can appreciate that something looks good and would taste good, and sometimes I even acknowledge that my body wants food, but I can’t imagine eating it. 

I never want to eat and don’t feel like I can when I’m not doing well. IV fluids always get me interested in food again - I love to eat, but it is too hard on me when I’m not at my best.

8 minutes ago, Sarah Tee said:

@RecipeForDisaster, I want to quit this job too! Where do I send my resignation letter?

🙂

Maybe if we ALL quit, they’ll go out of business??

2 hours ago, Sea otter said:

I am just so tired. Of educating myself about POTS, educating others, even doctors, searching for ways how to help myself, trying various treatments that don't work and only giving me side effects, trying to raise awareness usually without much success. When I think I have some "control" over my body or that is getting better, flare happens. Plus triggers are always somewhere around the corner. I am doing everything I can but I feel quilty, like it's my fault that I am not getting better. I don't want my whole life to revolve around illness so I try sometimes to ignore it. But soon reality hits, as it is affecting every aspect of my life. Why am I writing this? Because I know you will understand. 😌

Hang in there. We definitely understand. I always say "I quit this job" because the combination of feeling awful all the time, and having to fight so many things (getting appts, getting to appts, getting meds or equipment, billing issues, infusion arrangements…) at the same time is not a "job" I feel like having. There’s not much I can do other than keep working on all of it, but it’s frustrating and draining for sure.

3 hours ago, Sarah Tee said:

I hope you find out about the low BP someday. Do you think it might be related low blood volume? There is a new device for measuring blood volume. Maybe Dr Novak will buy one. I believe they are “only” about $US50,000, plus you need a phlebotomist, a blood gas analyser (in most hospitals already), a laptop, and the occasional cylinder of carbon monoxide. It’s been approved in Europe as a medical device so perhaps it will show up soon in the US.

It’s very cool that you are one of the people in the original study 🙂

I do think it’s at least partly due to low blood volume. Dr. Novak doesn’t seem interested in testing for it - I think he just knows I have it and doesn’t care to prove it, but I really do. IV fluids fixes it for a short time, which I guess partly proves his point. He said "it’s just that your vital organs aren’t getting blood" - that’s my problem. Oh, is that it? But who knows, he’s at a very big hospital, so maybe they’ll get the device anyway. They had a Daxor at Yale, though.

I really hope I get to help others by making my data available. 

8 hours ago, Sarah Tee said:

Oops, I meant to say “poor you” as well (not in a condescending way). I hope this new research has given him some ideas to better treat HYCH and that that will help you.

Am I right in thinking HYCH is fairly uncommon? In his first paper on it, I think Dr Novak only mentioned 16 patients, whereas for OCHOS he had about 100. And maybe you are one of those original 16?

Edit: In the paper I linked to, he has data from 72 HYCH patients.

Based on what he said in a lecture, I estimate he has about 130 OCHOS patients now.

Wouldn’t that be great? He wanted to try galantamine for it, but it kept me up all night even if I took it only in the morning (it made my palpitations worse, too). I may try again. My BP is really low, which doesn’t help. That is not part of HYCH, and is undiagnosed, and makes everything a lot harder to survive and treat. 
 

I think HYCH and OCHOS are uncommon because most clinicians haven’t heard of it and don’t have equipment to diagnose it. I bet there are many more. I had it for at least a decade before he found it. But I have more stuff that is not diagnosed. 
 

I'm sure I’m one of those 16. I remember him saying that the cerebral Doppler was somewhat experimental and that he was doing research. It’s neat to be able to be part of that - I’m always happy to be a Guinea pig.

16 hours ago, Sarah Tee said:

Oh no, poor person in Hawaii!

Hope your visit goes well and the traffic isn’t too bad.

Thanks! I was so inspired by that and couldn’t wait to hear how he healed that person, and would use that experience to heal me… um, nope. I hate going to the city, but it’s only a few times a year.

Funny thing is, when I first started seeing him many years ago, he said that he had a patient just like me from Hawaii (he has patients all over the world). I got excited and asked how he helped them, and he said he never figured them out!

I will mention that - he might be tickled! It’s a huge pain to go to Boston but it only takes about 2 hours. It’s parking, aggressive driving, and traffic that are the problem.