Diary From A Research Patient At Vanderbilt

[roxie]

Thanks for keeping is posted . So you can see hyper pots on the valsalva?

Did they give you any lifestyle advice on how to get stronger and increase activity?

[kitt]

Great information Kelly, thanks for sharing this amazing experience.

Interesting about blood volume, many of have 'assumed' this is an issue for 'us', and according to your information, (great source), this may not be the case.

You did benefit from the IV saline because of the salt. So would you want to continue occasional IV treatments once you're home, after a crash for instance, or under other circumstances?

Glad you got to meet Dizzyde, what a privilege to meet a fellow patient and forum friend.

Hope to hear more after you're rested and have time to digest and process the experience even further.

Thanks,

K

[sue1234]

Thanks so much for that final, detailed information! That is nice to know that I wouldn't HAVE to participate in the binder part. Sounds like a great place to offer up our bodies for research! (while alive and kicking, that is!)

[Kellysavedbygrace]

Yes. The main message is "dont give up." So much of this battle is mental and since there is no known cause, no known cure with a very heterogenous presentation our job is to try and live as normal a life as possible. A few things are known to help most:

- regular exercise both aerobic and strength building (which I am doing and working on increasing)

- salt

- caffeine (many POTS pts, not all)

- fluids

- your own research and your testing (to try to find the best pharmacological and non- pharmacological treatments.) Dr. Biaggioni said, "As a patient you shouldn't have to research, but with POTS you have to research.". Each patient is different. So it helps have to try one treatment for a couple of weeks, then stop that treatment and see how it effects your symptoms. The key is to be patient, do it one at a time and be scientific about it.

[Kellysavedbygrace]

The Valsalva is primarily used to help identify if the autonomic reflexes are intact. When there is more severe autonomic failure these reflexes don't respond in normal limits. So the minimum normal change in Valsalva from baseline is 10. If your SBP doesn't increase by 10 it is thought to be evidence of failure of the autonomic reflexes (often seen in PAF, MSA, less often in POTS.). My 75 pt increase is interpreted as an exaggerated sympathetic response to stimuli. However, increased catecholemines (such as NE) are thought to be more helpful in interpreting a hyperadrenergic response. The problem with Catecholemines are that they can vary so much day to day- you have to essentially catch them misbehaving. While at Vanderbilt my catecholemines were fairly low but I have had this tested before both at Cleveland Clinic and at MUSC. In both cases my NE levels were significantly elevated. So I was surprised that they were normal this past week - don't know why but was told that they can vary much from day to day. Nevertheless, I knew before going to Vanderbilt that I have a hyperadrenergic case.

[roxie]

Thanks. I'm trying to figure out if I have hyper pots. I know lat year my dr told one of his patients that testing for it was a waste of time bc you either do or don't have it. I've been afraid to ask him. I'm not sure if his opinion will change in a year. I may just ask him or at least ask for my autonomic results.

I also had a 24 hr urine. Don't know if it was catacholamines. Ill have to pull out the results tomorrow but the sr said "tou don't hold water" like I pee too much or somethig. I think that's a sign of hyperpots?

[abbyw]

Thanks, Kelly for the excellent information. It sounds like this was a really important experience for you to help figure out what helps and what doesn't.

The blood volume issue is especially telling, I know that increasing my fluid intake doesn't seem to help me at all.

It was also interesting to see that they are drugs that help with the tachy, but don't actually make you feel any better. That fits in with how we feel symptomatic even when our BP and HR are fine.

Any insight on what caused the POTS in your case?

Thanks,

Abby

[boymommy3]

So glad you had such a good experience! We are huge supporters of the Vanderbilt medical community. We have a son with a CHD and have spent lots and lots of time at Vanderbilt Children's Hospital. I cannot say enough great things about our experiences at Vandy! I will for ever believe that God orchastrated our being there because it was not our first choice.

If my sysmptoms persist I would love to participate in this program someday.

[Kellysavedbygrace]

So hyperadrenergic POTS is just a way to describe the characteristic of having an exaggerated sympathetic response to stimuli. Some have heightened responses and some of us have lower responses. The way most doctors would clinically define that is a Catecholemine response of 600 or more upon standing. In order to get this data you need to have a serum Catecholemine test accompanied by a Postural change. At Cleveland Clinic they did that for me on the tilt. At Vanderbilt they did that during a posture study. Thus, they could compare the Catecholemine difference between supine and fully upright. All that to say unless there is something I don't know about I don't think you can get that info on a urine test. But check w our doc to be sure.

As for my suspected cause of POTS? I suspect the autonomic dysfunction is caused by a mutation of my mast cells. (MCAD). But this is just a theory. I really dont know for sure. It is interesting that Dr. Biaggioni does not think my POTS is secondary to MCA but that they coexist and feed each other in a downward spiral. So for now I'm working with the Vanderbilt docs and my POTS doc to trial a few new drugs that might help my Autonomic dysfunction and I'm working w my Mast Cell doc to trial a few new drugs that help block the degranulation of mast cells and their chemical mediators.

[peregrine]

As far as I know, the 24-hour urine samples are mostly done for urine sodium (to figure out how much you are losing and make sure you're not eating too much salt too) and, as an initial screening test, to rule out pheochromocytoma; I don't think you can test for hyperPOTS based on urine alone. Many docs don't seem to test for hyperPOTS even if it looks like you have it (and not all autonomic testing facilities do a standing catecholamine draw) - what seems to mostly happen is looking at symptoms and trying to treat for it and see if there's a good response.

[roxie]

Thanks, I think they did the 24 hr urine for sodium & pheo. Last I knew my dr didn't see the point in upright catacholamone testing, he said you have it or you don't.

[peregrine]

Yeah, mine said the same thing - it looked like hyperPOTS based on the heart rate response to tilt (with pooling, just to be confusing), it responds to beta-blockers and especially clonidine, so... not much point, especially since I have issues with needles causing syncope so an upright blood draw would be tricky (though with an IV I now know it might work out).

[roxie]

That's interesting peregrine. I pulled out labs yesterday and I'm not sure some high, some normal.

However, I start clonidine in decemeber because of how much my heart runs wild in my sleep. It has had more of an effect on my heart than any med I can think of. Sometimes my resting heart rate is in the 60s! And now usually around 75. That's never happened before.

I don't have my latest ttt results on hand or my autonomic stuff but I have old ttt's the one done by dr Stewart in NYC. He diagnosed clearly low flow pots with pooling.

[RichGotsPots]

First off great diary post!

Second off looks like Vandy is reading my posts lol http://forums.dinet.org/index.php?/topic/18585-testing-for-endothelial-dysfunction/?hl=endothelial

I posted back in 2011 for endothelial tsting in pots. @kelly can you tell us what the results of the testing was? Also did they tell you which meds you trialed?

Did you see my post about salt causing adrenaline? I do think there is a difference btwn iv saline and eating salt or drinking it..

@i hate banannas, 24 hour urine catch tests for pheo and the way kelly got tested is for hyper. @kelly can you tell us what your supine ne was compared to standing?

Abdominal binders are give me too much chest pressure but thigh sleeves are perfect for me..

[Guest wishing&hoping]

To Peregrine: where did u get the compact folding stool? Sounds awesome for travel, long lines, etc. Often just walking a block makes me dizzy and I' m tired of sitting on cold benches! I have been almost totally homebound for the last 2 weeks due to a flare and the cold weather and am going nuts!

Also wondering about use of compression hose, etc. I sweat uncontrollably and unpredictably in between chills. Does it make that worse? As it is I' m constantly removing and adding layers. Menopause doesn't help.

Thanks for any advice. This forum is great!

adina

[sue1234]

Rich, abdominal binders do the same to me within minutes of putting it on. What are thigh sleeves?

[heathmcev]

Hello from Vandy everyone!

As Kelly mentioned, I'm currently inpatient at the Clinical Research Center which she just returned home from. I'm here until Tuesday (8 1/2 days total). I'll post more about my experience once back home - I'm having a hard time writing a lot from here as I've been so tired between studies (massive brain fog too - my poor nurses have been unbelievably patient with me. They're used to us!!! Lol)... but I'm keeping a journal and good notes. Kelly it was so wonderful to meet you - i wish we had more time to talk in person!

My experience so far has been exactly like what Kelly has posted. I couldn't have imagined a warmer, friendlier, more knowledgeable group of drs, nurses & researchers than I've come across here. This is a very impressive place!! I had very realistic expectations coming in (aka "I may get sent home on the same tx I'm already on & may not learn anything new, but I want to contribute to studies")... but I've found that I've learned more than my tired brain can yet process! Being in a controlled, quiet environment has helped me learn things about my body that I hadn't been able to focus on before. With a controlled diet, and space to focus on my health & nothing else, with regular vitals being taken throughout the day, I'm seeing what my own patterns are - more clearly than I've ever been able to. And that's not even getting into what the team here has told me! Ill know much more tomorrow & Tuesday regarding specific results of tests I had last week. So far, from the Autonomic Reflex Testing, Dr Biaggioni has confirmed POTS with exaggerated sympathetic response (hyperadrenergic) - which is confirmation for me of what my home pots dr (Dr Khurana) has previously dx'd. I also had a normal Qsart.

I'll post more later, with specifics. I told Kelly I didn't want to hijack her thread by adding my experience, but she encouraged me to post here as well. Since our stays overlapped and we therefore participated in the same studies, it certainly streamlines things. Thanks Kelly for opening up the discussion.

[FarmerAmy]

You mentioned that your total blood volume is normal. And you also said that the saline IV likely helped because of all the salt--not the fluids.

Do you understand this? I thought you are supposed to salt load because the salt helps to retain fluids, thereby increasing your blood volume. But it seems this is not true in your case. The salt is having some different beneficial effect.

[peregrine]

wishing&hoping - I got mine from REI (mods, if this link is not allowed, please delete it, and my apologies!):

http://www.rei.com/product/765283/rei-trail-stool

It is small enough when folded to fit with the bottom in the water bottle pocket on the side of my pack, and then I use the strap that goes horizontally across the side of my pack to hold the top in. I think the weight limit is maxed out at 225 pounds, just FYI. When flying I take it out of the side pocket and put it in the overhead bin (my pack goes under the seat in front of me). The only downside is that it is less good for sitting in long lines that move steadily - like airport security - since you have to move too often; it is also pretty short, which can be good if squatting helps you but can be harder if you have trouble getting up from low chairs. I find that holding the corners helps me rise up (bad knees and hips). My model is the one pictured - no carry bag or cloth bottom. The other big thing I use it for is museums - they usually let you take it in even if they make you check your bag, as long as you tell them it's a medical need and not a camera tripod!

(sorry to hijack the thread, folks)

[Guest wishing&hoping]

Thanks, Peregrine! My fault for hijacking the thread....but d/n know how else to ask.

In process of applying to Vandi. Was running errands this PM and came close to passing out. Realizing I can't go out alone until more stable. Feeling very discouraged. Also had horrific stomach pain; due, I think to stenosis in abdominal arteries. This is scary!

Adina

[kitt]

Kelly,

Was going through my bookmarked papers, and found this:

technical theory published by the researchers at Vanderbilt:

"Mast cells are localized in close proximity to blood vessels and peripheral nerves and are therefore strategically positioned to modulate sympathetic activity, vascular tone, and angiogenesis.²⁰ Histamine is a powerful vasodilator that could explain the cutaneous vasodilatation responsible for flushing. With regard to the pathophysiology underlying the association between POTS and MCA, we propose a positive feedback loop by which MCA, with the subsequent release of vasoactive mediators, may contribute to vasodilation, reflex sympathetic activation, central volume contraction, norepinephrine release, and orthostatic intolerance (Figure 4). Conversely, our results indicate that exercise can lead to MCA, presumably through sympathetic activation."

[issie]

I have found that meds to stablize mast cells have been some of my best meds - so far. I have had a lot of improvement when I added GastroCrom. It is true that you have to watch your triggers. But, with MCAS sometimes, you don't know what those triggers will be. Something you eat or use one day - may be fine - the next day they won't. So, it's a very difficult thing to figure out. but if there are definite triggers - they are usually always triggers. Lots of trial and error on this. As we sometimes find that we are allergic to nothing but react to everything.

Some of us also have autoimmune disorders too. This seems more common then not.

So, we will see what the combination of things I'm doing brings. I think there is a lot to be said for stablizing mast cells and its connection to POTS.

Issie

[kitt]

The excerpt is from a Vandy paper. Applies to hyper pots patients exclusively. Makes a lot of sense!

http://hyper.ahajournals.org/content/45/3/385.full

[Kellysavedbygrace]

Back home now...

A little over a week since I left Vanderbilt. On the way home my hubby took me to Pensacola to see my POTS doc, Dr. Randy Thompson. He helped me get scripts for the new meds to try and adjusted the exact types, doses based on his experience.

And then I came home...this house is loud, with four kids and their friends, in comparison to my quiet private room where they fed me and cleaned too. Then Monday my 9 year old needed an emergency appendectomy. So for the past couple of days I've been the caregiver and now I'm back in bed trying to recover. I share this because I know you all know that post exertional crash we get.

Thanks all for your positive comments on this thread.

Dizzyde, you must be home now. I look forward to hearing about the rest of your experience.

Rich, I think you are right. Dr. Robertson, Dr. Raj and Dr. Biaggioni must all be secretly following yourposts which inspire them!

As far as the endothelial studies. I did not receive any results. At this stage in that trial they are simply

collecting and aggregating data- too soon for them to share results and interpret. I believe a few things they are looking for there are:

- inflammatory markers in the endothelial wall

- NO production following stressful stimuli

- venous functioning before, during and after cutting off circulation.

I did read the thread on BP and salt and think it is interesting. Apparently this thinking has been out there for many years. For me it causes me to question why salt helps instead of hurts since my body apparently produces too much adrenaline. Maybe it is the same way a stimulant works, if the drug produces it, my body gets to create less of it.???

My supine vs upright NE at Cleveland Clinic last year was 158 supine and 680 upright on tilt after 9 mins. In October it was 653 during a supine draw. So, I was surprised it both were in the normal range during my Vandy visit. It could be just the day to day variation in catecholemines but I suspect the more likely reason is the couple of months I've been on high dose antihistamines and low dose aspirin therapy

for mast cell disease. NE is a mast cell mediator.

Kitt, thanks for sharing the link to that Vanderbilt MCA article. How neat it was to be in a place where the doctors not only understand POtS, but they also have studied MCAD.

As a side note, I likely would not have been diagnosed as having MCAD at Vanderbilt because I did not have a flushing episode while there. And although they don't typically do the extensive lab testing that I had done by my hematologist for MCAD, Prostaglabdin D2, the very specific mast cell mediator that was

elevated in my blood work confirming my MCAD diagnosis was discovered by Dr. Oates right there at the Clinical Research Center at Vanderbilt. Cool huh?

Issie, I agree. For me my most helpful meds thus far have been mast cell blocking meds. I'm hoping to try GastroCrom later this spring.

[AllAboutPeace]

Yes. The main message is "dont give up." So much of this battle is mental and since there is no known cause, no known cure with a very heterogenous presentation our job is to try and live as normal a life as possible. A few things are known to help most:

- regular exercise both aerobic and strength building (which I am doing and working on increasing)

- salt

- caffeine (many POTS pts, not all)

- fluids

- your own research and your testing (to try to find the best pharmacological and non- pharmacological treatments.) Dr. Biaggioni said, "As a patient you shouldn't have to research, but with POTS you have to research.". Each patient is different. So it helps have to try one treatment for a couple of weeks, then stop that treatment and see how it effects your symptoms. The key is to be patient, do it one at a time and be scientific about it.

Kelly,

Thanks for sharing all of this. I'm just curious about the 'caffeine'. Did they say specifically why it is helpful? Is it for mental clarity or other reasons?

I hope your 9 year old is recovering well from the surgery (and that you have recovered from the experience). My friends 12 yr old went through this recently and they just got her into surgery in time. Scary stuff!