RecipeForDisaster

4 hours ago, Sarah Tee said:

@RecipeForDisaster, I’m sorry to hear your friend didn’t get any progress from having the test. I imagine it might have cost a lot of money too. Oh dear.

It did. The arterial line and jugular cath are more important to me than the money, personallyI guess Systrom can deduce mitochondrial function using this test. He also really wanted me to do it. I’m relieved that I didn’t - mine would likely come up with the same findings as my friend. Basically, he offered her Mestinon if she wanted, and that was it, permanently. I’m already on it.

4 hours ago, Sarah Tee said:

Okay, found it. It is called an invasive CPET or iCPET, and the person who has used it to investigate CFS is Dr David Systrom.

Info about the test:

https://www.uclahealth.org/medical-services/pulmonary/pvd/conditions-treatments/invasive-cardiopulmonary-exercise-testing-icpet

Some of Dr Systrom’s research:

https://journal.chestnet.org/article/S0012-3692(21)00256-7/fulltext

It seems like the standard CPET is fairly widely available but the iCPET is specialised. Do correct me anyone if I’m wrong.

Both do however seem to be aimed at investigating shortness of breath, although maybe Dr S uses them to look at it in a different way.

I do get a bit puffed when I’m symptomatic, but I don’t think I have actual shortness of breath. Maybe if I went for a jog? Usually I notice my exercise intolerance at home when climbing the stairs or doing squats. Mine is more like having no energy reserves to power my muscles than being short of breath.

Edited to add: There are also references to a two-day CPET. That is having the test twice, on consecutive days.

https://www.healthrising.org/blog/2019/01/17/decoding-2-day-cpet-chronic-fatigue-syndrome/

https://neuroimmune.cornell.edu/research/physiology/

My friend had this. It’s pretty invasive, and yes, harder to find than a regular CPET. You do exercise hard, with catheters, so I think I’d be short of breath. She "failed"(notable findings) with very low preload during exercise, and didn’t end up having any recommendations for it in the end. She wanted me to also do the test, and I’m glad I didn’t, because she is back where she started. She has actual POTS and I don’t.

I would just love to have this done. I wanted to do the Daxor test, which is supposed to be at Yale, but no one wants to order it. 

On 4/20/2023 at 2:31 AM, Sarah Tee said:

@RecipeForDisaster, I’m sorry it has come with difficulties. I’ve suffered several ongoing “injuries” from medications so far (even after I stopped taking them), and I’ve only been on lower-risk treatments.

Do I read right that it is helping in general? I do hope so.

I’m a lot less ill than most people posting here, although I have been getting worse for 18 years, and I wonder where it is heading. So IVIg would not be indicated for me at the moment, but maybe it will be in the future. I feel like there is an auto-immune or auto-inflammatory condition bubbling away under the surface. I wish it would just make itself known so I could get onto a targeted treatment.

I also have another problem, had my large bowel removed to treat ulcerative colitis, and the surgery has failed recently after 15 years of working well. If I just had one to deal with, I would probably be able to have a more normal life, maybe do some work from home.

IVIG is helping my SFN only, so far. I definitely have other autoimmune disease (same, mystery, a few clues like ANA but nothing useful), and we hoped IVIG would accidentally fix those. Hopefully! I would not hesitate to have started IVIG for my SFN years before I did, because the damage might be milder. 

9 minutes ago, Sarah Tee said:

My specialist said we could pursue getting me tested, and, if it came back positive, I might possibly qualify for IVIg.

I’ve decided not to pursue it at this point. I have some numbness on my big toes that appeared in my early 20s (possibly at the same time my OI symptoms began to sporadically appear, so maybe the time of the original autonomic “injury”), but no pain, just occasional hot or itchy feet at night.

The numbness has never progressed and the the hot, itchy feeling is hardly worth mentioning compared to everything else that’s wrong with me.

IVIg might help me overall, but nobody would order it for the level of SFN I have (if that’s what it is).

Maybe the IVIg would help you overall, and that might make it worthwhile exploring the SFN angle.

That was the thing for me, the SFN started so slowly that I didn’t really notice it or see that it was not normal, and no way was it in my "top ten problems", so I never brought it up. It’s fairly bad now, but I still have many more pressing issues. Hopefully halting my nerve damage is worth using IVIG, but I am truly using IVIG to try to help those more critical issues, one of which is suspected severe autoimmune dysautonomia, even though I qualified for it using my SFN biopsy. That said, I’ve had a lot of challenges with IVIG, from financial ones to infusion reactions, BP drops, and aseptic meningitis.

3 hours ago, MTRJ75 said:

He never even mentioned IVIG as a possibility. 

I'm not even sure what we're going to do after this. It's funny that he mentioned the best medication he knows of for this is LDN, when I introduced him to LDN about a year or so ago. 

It sounds very promising. I tried various LDN doses for a few years and didn’t have any improvement. I don’t think I’d do the biopsy without a plan for what happens if it’s positive, though.

I also drink olive brine - some drink pickle brine! Sometimes just a handful of flaky salt. I crave it.

49 minutes ago, MTRJ75 said:

After failing at exercise attempts spectacularly this year & then having  my worst flare up in over a year due to a combination of physical activity and brief sunlight, my neurologist is sending me for a small fiber neuropathy biopsy. 

Although, he says that there's not much that can be done about it even if confirmed. 

Is this true? 

It makes the most sense, since I wake up burning like I was baking in the sun over-night and exercise leads to that same burning and cramping, right before sweating and adrenaline dumps. This might be the largest obstacle for me right now. 

It’s hard to get qualified for IVIG even if the biopsy is positive. I think having something come up on lab work like Washington University (FGFR3 for me - autoimmune) will help. It took me months to get my prior authorization approved, but IVIG is helping the neuropathy. Otherwise, I think they just use meds to help control the symptoms, and I don’t think anything but IVIG truly helps the neuropathy itself.

I’m not normally hyperadrenergic, but I always have this, usually more like 3am. A dream often sets it off. It’s horrible - I’m up for the day from it, because I get up around 5 normally, and I’d never get comfortable enough to get back to sleep that fast.

I did end up getting infusions, because no one wanted me having another reason to be weak, cold, and anemic. I tolerated them well and they got my levels over 100 when nothing else could.

21 hours ago, Sarah Tee said:

@chimeraskeep, that is interesting. If I lived in the US, he would be the specialist I would try to see.

(I have plain OI, no HR or BP abnormalities, and he seems to be the only person who publishes on “my kind”, although I am sure all the specialists know we exist. I might have HYCH or OCHOS, the two new syndromes he named, but I’ve been told they can’t be tested for in Australia.)

People from many countries show up to see him! I know there are other people who are good with this stuff - and I guess I can’t be too excited, as it’s not like I’m doing well. It’s not him prescribing my IVIG, either. I do like him, but aside from giving me a label, it hasn’t been THAT helpful.

3 minutes ago, MikeO said:

Here at my local provider they now have a specialist and pharmacy that focuses on meds (side effects) or interactions it has been a game changer some peeps. I do still not understand the cerebral doppler stuff. Pass out with no change in vitals is teling. I get the lack of cerebral perfusion and when it goes low one's body most likely will respond. for me it is taking convulsions as my brain try's to adjust and can be a bit violent. also lack of pulmonary filling will spark this as well and a standard TTT should pick up on this.

Just my thoughts. 

The Doppler lets them see how well perfused your brain is, and how that changes with position. It’s also aligned with exhaled CO2 readings in this case. My brain had 37% less circulation when I was upright (that fits into severe). I did drop my BP and raise my HR (which was already up), but not enough to get a diagnosis from those alone. That said, I always say that during my TTTs, I am not myself at all - off my meds, fighting traffic and navigation for the 2 hour trip up, parking, near misses, etc. I hate big cities, so I always show up way "better" than my baseline. I promise that if I could really have a TTT done at home, it’d be telling.

1 hour ago, Sarah Tee said:

@RecipeForDisaster, that’s interesting, I hadn’t heard of galantamine. Is it for the acetylcholine angle?

I’m sorry it didn’t work for you. Insomnia is the last thing any of us need as a side effect.

The other day I was reviewing all the medications I have tried. I mused, “I think midodrine was my favourite”. My dad said, “But midodrine didn’t work, did it?” And I said, “No, it didn’t work, but it had the least side effects”.

🙂

I hope some very clever and dedicated PhD students are out there devoting themselves to understanding and reducing side effects in medications so we can benefit from their work.

Yeah, I tried many doses at different times for a long time. I did not want to give up. I am not sure if it was that or as a cerebral vasodilator(I think the latter). I definitely need that! My heart pounding is bad enough without any meds exacerbating it. Same for my insomnia, sigh.

I’d still try the Nimotop but I don’t think anyone wants to write for it. That’s funny, I have meds that I feel that way about, but midodrine isn’t one of them! I have side effects starting at 5mg. It does help, but I have to take tiny doses hourly or so in order to get enough to have any benefit. It’s pretty annoying. I wish it was extended release, so I could take a tiny dose of that one or two times a day. I do know what you mean, though, about the meds that don’t do anything bad OR good. 
 

Yes, wouldn’t that be great? I’m always happy to be a Guinea pig for that reason. I hope to help others.

2 hours ago, Sarah Tee said:

@rondo, I have orthostatic intolerance with normal heart rate and blood pressure.

Specialists have been diagnosing chronic orthostatic intolerance for quite a while without checking cerebral blood flow.

I have seen two autonomic specialists here in Australia who had no problem making the diagnosis, so I have my fingers crossed that your specialist will be helpful.

It is not weird at all to want to be diagnosed with something. After all, you have already been living with the symptoms. You are not wishing anything bad on yourself, just looking for a name and an explanation (of sorts).

In terms of treatment, as far as I can tell from peering at extracts from his textbook, Dr Novak hasn’t yet found a specific treatment for low cerebral blood flow. You and your specialist will be trying the same selection of interventions as someone diagnosed with HYCH, for instance.

You know, it is funny you posted because I was just thinking “Boo hoo, my diagnosis doesn’t even have a name”. I would have to call it chronic orthostatic intolerance, with normal BP and HR, caused by autonomic dysfunction.

He has been trying galantamine - I had trouble tolerating it and didn’t feel a big difference, but I didn’t get my dose up very high. I know it made my insomnia worse, even taking it in the morning, and I think it made the pounding worse. I had high hopes for it. Some people get a calcium channel blocker, like Nimotop. I think my BP is too low.

2 hours ago, Sarah Tee said:

If anyone here contributes to Wikipedia, autonomic testing for cerebral blood flow velocity could be added to the entry for transcranial Doppler (with a suitable footnote or two).

I used to contribute bits and bobs a long time ago, but I don’t any more.

That’s really neat! I’m the same, I used to edit, but haven’t in forever.

31 minutes ago, Sarah Tee said:

Just watching research grant information from last year and thought it might be of interest which researchers/institutions received grants for transcranial Doppler equipment to use during TTT.

(Several researchers have looked at cerebral blood flow velocity in dysautonomia patients. Dr Peter Novak published an article naming HYCH as a new OI syndrome diagnoseable with this technology.)

Here are the recipients:

• Tae Chung, Johns Hopkins
• Melissa Cortez, University of Utah
• Nate Robbins, Dartmouth Hitchcock
• Satish Raj, University of Calgary

So if you see one of these doctors, or attend one of these institutions, you may be able to have your cerebral blood flow checked soon.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6157889/

https://www.brighamandwomensfaulkner.org/about-bwfh/news/expanded-autonomic-testing-helps-to-pinpoint-cases-of-orthostatic-intolerance

https://pubmed.ncbi.nlm.nih.gov/26427910/

Is there any particular treatment outcome?

Dr. Novak has some medication suggestions in his textbook, “Autonomic Testing”, which your specialist may have. Nothing definite but might be something you haven’t tried yet. Otherwise, treatment is along the same lines as other OI syndromes.

I was glad to get the HYCH label so I’d have -something- other than generic dysautonomia . I’m kind of amazed that the technology is expensive enough to warrant a grant, but I’m glad more doctors will be able to do this. I probably would have had an inconclusive TTT without the Doppler.

Very interesting! I’d get tested if they had it in Boston. I know I do much worse after I eat literally anything…

1 hour ago, edriscoll said:

This us a Patient-reported outcome study on the effect of dyspnea on the quality of life for people living with dysautonomia  DINET members are encouraged to complete a short survey to participate in this IRB-approved study by the University of North Carolina Pembroke.  Read more about this study on their website or visit their Facebook page for more info. 

Done! Seems like a well designed survey. I was impressed at the number of choices offered.

I’ve had my whole genome sequenced, and there is a novel finding of unknown significance in there, related to familial dysautonomia. My mother and sister have some elements of my health problems, too. I was told the same thing - it would have been severe enough to be discovered by my teens if it was FD. But it’s interesting. They are going to sequence me again twice a year in case they learn more about these genes.

5 hours ago, MikeO said:

You must be blessed with the creative mind. Good job for sure! Me on the other hand not always so good. I duck taped my BP monitor to my chest once while trying to take frequent readings. I advise against it.

I might be creative, but if I wasn’t, I’d be doing even worse

I do things that are not so beautiful, like that one. You should see my couch desk. Velcro is my friend. 

12 hours ago, MikeO said:

Dang! you girls are so creative....

Gotta do what you gotta do! I’m looking into making a foot hammock for my desk - I work on my couch, and I noticed foot hammocks when looking for my ottoman. I think that’d be helpful. You’d be amazed at all of the adaptations I’ve made! My IV backpack is multifunctional, nothing like the basic bag I was given.

1 hour ago, Pistol said:

@RecipeForDisaster I have a little camp stool i bought for 15 dollars and I can easily put that over my shoulder. I can use it to put my legs up, sit down if I unexpectedly end up standing in line ... I also have a seated walker but that one is only for big events, like clothes shopping with my daughter ... 😬

Oh yes, my mom has one of those! I ordered an inflatable "patio" ottoman from Amazon for now. We’ll see how it goes. I know it’ll be a huge help to be able to put my feet up wherever I’m sitting.

I sit in meetings or waiting rooms a lot, and get very uncomfortable when my feet are not up. I was thinking that some kind of foldable or inflatable ottoman would be great, as long as it didn’t look too weird in a meeting. Does anyone elevate their feet in public and how do you do it?

7 hours ago, Jyoti said:

That sucks!   I mean...I hope the prednisone makes a difference and the meningitis is very quick to exit.  My goodness---take good care.

I was thinking about both the anti-inflammatory and vascular-tightening effects of steroids and thought I'd see if there was anything out there without the same side effects that might accomplish something similar.  I found this --think I will do some more exploring.  It is just too impossible to consider that there is something that improves things dramatically and yet can't be taken advantage of.  I will post if I decide to try this combo.

Looks like the CE (one component of the above described cocktail) is amazing.  It seems to do everything; https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3116297/   But guess what?  It could build up toxicity if taken for more than six weeks straight.  So.... same kind of problem.  I'm not defeated, though.  There's got to be something!

My neurologist cheerfully told me "you’ll get aseptic meningitis every time now". I thought we had a regimen figured out (first time with this product, with a rest day between treatment day, tons of hydration, and still with IV premeds), but this time, it just hit me a little later and maybe a bit less badly. I haven’t tried oral steroids for it before, but the longest it’s lasted without them has been a week. 
 

I think my dysautonomia/low BP feels better, but I’m so sleep deprived (up since 2:30am), it’s hard to tell. Hopefully tomorrow will be a clearer day. If it does work, good luck to me convincing people I need steroids for this! 

That is a very interesting herb. I think I researched it before. I hope it’s the magic bullet we are hoping for!

2 hours ago, Jason_X said:

I frequently have issues with pounding heart sensation even though my heart rate is low (<70 bpm). I have very low anxiety, but it makes it hard to sleep.  I take Corlanor and Bystolic to help control heart rate, and it doesn't seem to reduce the sensation of pounding, but not completely.

Totally me too, just different meds. They help, but not enough. I can have uncomfortable pounding when my HR is in the 60s and am not anxious at all.