Michelle

[futurehope]

I just wanted to ask this apart from the long post on the (Grubb??) article.

I get hypertension on standing. My norepinephrine levels are higher than normal on standing, but my epinephrine levels are fine.

I had gradual onset.

Does this sound like I could have the hyperadrenergic form of POTS to you??

I know you're not a doctor and I'd have to ask Dr, Grubb himself. I guess I'm looking for an opinion.

If you feel like you shouldn't answer, I understand.

[lthomas521]

Primary means that they can't find any other disease in you that could explain the POTS. If they find that you also have some other disease that could account for the POTS, then your case of POTS is considered secondary, not primary. Hyperadrenergic means that you get elevated norepinephrine levels when you stand up. If that theory about the faulty norepinephrine transporter in POTS patients is correct, it would account for high norepinephrine and normal epinephrine levels.

It's interesting that you get hypertension when you stand up. Have you had your blood volume tested?

[sfr100]

Primary means that they can't find any other disease in you that could explain the POTS. If they find that you also have some other disease that could account for the POTS, then your case of POTS is considered secondary, not primary. Hyperadrenergic means that you get elevated norepinephrine levels when you stand up. If that theory about the faulty norepinephrine transporter in POTS patients is correct, it would account for high norepinephrine and normal epinephrine levels.

It's interesting that you get hypertension when you stand up. Have you had your blood volume tested?

Is rhis why in hyperadrenergenic pots they try norepinephrine reuptake drugs?

[futurehope]

Ithomas521,

No, I have not had my blood volume tested, but I will pursue that at my next neuro visit.

Why do you ask? Is there some connection to high blood pressure? Is low blood volume "fixable"?

Also, in case you know the answer (you seem knowledgeable), it seems to me we have too much NE. If that's the case, why would we use a SNRI - selective norepinephrine reuptake inhibiter?! I thought we would WANT reuptake;we wouldn't want to INHIBIT it?

[Michelle Sawicki]

Hi,

I'm going to ask one of our medical advisors about hyperadrenergic POTS and also the beta-receptor supersensitivity that Jan was talking about. I'll either post it here or in the newsletter. However, keep in mind that the different doctors have different ways of viewing these disorders, and they may use different criteria when labeling people with the "hyperadrenergic" form. Since Dr. Grubb has his own health problems and is w-a-y busy, I have not asked him to be a medical advisor. So I will probably be asking this question to someone at Vandy, who may have a different take then Grubb on the whole thing.

Michelle

[lthomas521]

I asked about blood volume because the theory behind salt and water loading is that many people with orthostatic intolerance (including yours truly) have been shown to have abnormally low blood volume.

Normally, you'd expect someone with low blood volume to have low blood pressure, especially when they stand up. However, Dr. Bell described a patient who had abnormally low blood volume but a serious increase in blood pressure when he stood up. After they gave this patient some intravenous saline, his vital signs normalized. Dr. Bell explained that they would never have dreamed of giving a hypertensive person intravenous saline unless they had just measured his blood volume. Unfortunately, the intravenous saline is a very short-run solution to a chronic problem.

So you can't completely rule out low blood volume just because you get hypertensive when you stand up.

I don't know why Dr. Grubb has said lately that people with hyperadrenergic POTS shouldn't do salt loading. My vital signs are much more stable and much more normal and I can tolerate much more activity when I do the salt loading.

I also don't know why some doctors are recommending norepinephrine reuptake inhibitors for people with POTS. We know that some people with POTS don't have a functional norepinephrine transporter to begin with, and we know that some norepinephrine reuptake inhibitors can produce orthostatic intolerance (mainly, orthostatic hypotension) in people who didn't already have it. Very confusing.

[Guest tearose]

I'm trying so hard to understand more... it seems there are variations of what is happening in the hyperadrenergic state.

I get hypotension on standing. My norepinephrine levels are extremely higher than normal on standing, but my epinephrine levels are fine.

I have denervation in my legs and splanchnic area so I was told I need the large quantities of norepinepherine to keep blood circulating to my brain and organs. It was explained that to use any blocker or inhibitor would be dangerous. The way my body is compensating for poor baroreceptors is to dump the norepinepherine. Does anyone have this too?

I feel like such an alien

[Lukkychrm42]

I don't understand the whys of it happening, unless it's compensatory, but supposedly it's common among hyperadrenergic patients to experience postural hypERtension. I'm one of them. I have low BV and when I stand my BP goes from low to high.

I, too, was wondering why Dr. Grubb recommended salt loading EXCEPT in hyperadrenergic patients. My doctors who recognized the hyperadrenergic state said I should have a high salt diet. Now i don't know if that's for the BV, and doesn't matter if my BP doesn't fall on standing...

[Guest tearose]

I've been asked to explain how I know I have the hyperadrenergic form and how I know I have denervation of the baroreceptor reflexes...sure!

During my last visit to Mayo Clinic, they did a battery of autonomic nervous system tests which are familiar to most of us....the two most conclusive in the hyperadrenergic dx were:

1)The timed, blood draws taken from three positions....laying down, sitting up and standing up.

This test captured the levels of circulating chemicals like norepinepherine, serationin....etc. This test documented the off the charts levels of norepinepherine I experience.

2) The Thermoregulatory Sweat Test: this is where you lay on a table and they attach some temperature monitoring wires and dust you with a brown powder that will turn purple where you sweat. They roll you into a plastic greenhouse and turn up the heat lamps and measure time, temp, locations of sweat production........ ...this was extremely difficult for me!!!

This test documented the lack of ability of my legs to produce sweat. This is "small fiber" deficit/dysfunction occurs in people with denervation

Again, it was explained to me that I have an abnormal condition and a normal reaction.

meaning: my legs and abdomen have denervation and this is the abnormal part. The norepinepherine dump is my body having a normal reaction to protect me, by brain and my organs.

Hope this helps explain it better.

Anyone else know they have denervation???

[Guest Belinda]

I have reduced sweating also..and it is recommended I get the full thermo.reg sweat testing..I already had the one where they use electric shock to stimulate sweating and it showed reduction. Wonder where I can have the whole shabammy done..nice word shabbammy...

Belinda

[Radha]

do any of you know if there are any meds to help counteract this over dumping of norepinephrine? i get this too, and cant stand the hyper feeling running thru me, is there anything other than xanax? thanks

radha

[Guest tearose]

Michelle, I am looking forward to hearing what your medical advisor gives as an explanation! You'll have that information today right?

....okay, I'll be good and wait...

Radha, do you sit or lay down when you get the rush? I can't do meds so I do the behavior modification route to deal with the rush. I may be just "use" to it, but with the compression and seat cane and electrolytes, I seem to manage again.

Oh, also, do you know if you NEED the norepinepherine like I do or could you survive with less?

I think I am asking if you have an abnormal condition OR an abnormal reaction.

[lthomas521]

The Thermoregulatory Sweat Test: this is where you lay on a table and they attach some temperature monitoring wires and dust you with a brown powder that will turn purple where you sweat. They roll you into a plastic greenhouse and turn up the heat lamps and measure time, temp, locations of sweat production........ ...this was extremely difficult for me!!!

This test documented the lack of ability of my legs to produce sweat. This is "small fiber" deficit/dysfunction occurs in people with denervation

Dumb question: can the soles of your feet sweat, or is the lack of sweating just on the legs? If someone's feet sweat like crazy, especially on bad days, can she safely rule out denervation?

[futurehope]

Ithomas521,

During my test, the powder was put on the TOPS of the feet (among other places), not the soles. When the doctor mentioned that my toes don't sweat, I was shocked that they even could.

[Guest tearose]

Ithomas521....my tops of my feet pour sweat. As a professional patient I would venture a guess and say that in my case so ipso facto....no, you can still have sufficient denervation in your legs and sweat on your feet.

[futurehope]

Tearose,

I really like that phrase "professional patient". I think I'll give myself that title too.

[Michelle Sawicki]

I, too, was wondering why Dr. Grubb recommended salt loading EXCEPT in hyperadrenergic patients.

My guess would be that you wouldn't want to take measures to raise the blood pressure of a patient who already has high blood pressure.

Michelle

[morgan617]

That is absolutely correct. Salt is always contraindicated in hypertension. You don't want fluid overload, which puts even more strain on your heart. I have been on a no sodium diet for 10 years. Due to meneires and hypertension.

I sometimes wonder if some of you are dizzy because of the amount of salt you eat, as it can affect the inner ear. You want to retain your volume, but it also makes extra fluid in the inner ear which throws off the vestibular system. But what do I know. I only know about me and if I eat salt, I don't walk well at all. I don't walk well on a good day anymore....morgan

[Michelle Sawicki]

I asked Dr. Raj (from Vanderbilt) about hyperadrenergic POTS and also beta-adrenergic hyperresponsiveness, and I have included his reply here. As I pointed out earlier, and as Dr. Raj himself states, different doctors have different ways of viewing these disorders, and they may use different criteria when labeling people with the "hyperadrenergic" form of POTS.

Do I think that Dr. Grubb's definition of hyperadrenergic POTS in his latest paper is the same as the one they are using at Vanderbilt? No, I do not.

I wish I could give you guys clear-cut answers, but I can only go by what is publsihed and what the general consensus is on such matters, and it looks like the doctors are still working on collectively dividing the POTS subgroups.

Here is Dr. Raj's response:

Michelle,

One of the big problems with POTS is that almost everyone agrees that there are multiple diseases and groups under the umbrella of POTS, but no one really agrees on how to divide the subgroups. This is one of those cases.

I think that most (if not all) patients with POTS that I see are hyperadrenergic. We often use a high level of plasma norepinephrine (a biochemical marker of sympathetic nervous system activity) as an entry criteria for our POTS studies.

We still make a poorly defined distinction within this group. There is a group (the majority) in whom the standing norepinephrine level is high, but not ridiculously high (600-1000 pg/ml). There are others in whom the norepinephrine level is very very high (>1500 pg/ml). This latter group we often think of as "primary hyperadrenergic", while the former are thought of as "secondary hyperadrenergic" (with high sympathetic tone secondary to another problem such as hypovolemia). The clinical problem is that it is not clear if the 2 groups can really be separated cleanly, nor is it clear if they respond differently to different medications.

We do not routinely assess for beta-adrenergic sensitivty. I assume that by this you mean sensitivity to isoproterenol bolus doses. I know that there are some older reports about this in POTS and IST (inappropriate sinus tachycardia), but I don't think that I have seen that much about this of late.

I am not sure if I have answered your questions or just muddied the waters further.

As an aside, I have written a review article on POTS (in which some of the subtypes are discussed) for the Indian Pacing & Electrophysiology Journal that is to be published later this spring (I think that it is supposed to come out in April 2006).

Satish

[Sophia3]

Michelle

Thanks for sharing dr. R's reply. I think it makes perfect sense..the parts I understood!! I couple things about a bolus confused me but like he said, there are so many variations on a theme, so to speak.

W/o detailed testing of each and every one of is it's a gamble to figure out what works and what doesn't.

Still, I tend to go by Vandys version of hyper Adrenergic stuff as I have caught my BP very high over the years but just a handful of times. Usually I feel so ill anymore, I rarely take the BP. Too much effort when feeling totally yucky. lol

Thanks again for sharing this email

Sophia

[Guest tearose]

Thanks Michelle!

Although we can see they are still working on the types, variations and treatments....at least they are working on it!

That was kind of the Dr. to attempt to clear some things up for us!

tearose

[futurehope]

Michelle,

Thank you for asking the doctor.

I guess I'm the "secondary hyperadrenergic" according to him, and they still don't know what works for us.

[momdi]

Morgan617,

Im not sure that its always true that hypertensives cannot have salt. If the hypertension is a compensatory response to low blood volume and low blood sodium, then taking in more salt will actually reduce the BP (as explained to my son by Dr. Low at mayo) because it increases the blood volume and the body is no longer vasoconstricting in order to compensate, so the blood pressure actually goes down. Does that make sense?

momdi

[morgan617]

momdi, yea I guess that makes sense, but I have never seen a doctor not restrict salt in hypertensive patients. But then, I have never worked with doctors or in a field of doctors where pots was an issue. I still can't do salt because of my meneires.

Having worked on a cardio floor for 13 years, it was just ingrained in my system that you don't give anyone with htn anything that can raise it more. With pots that may old school, but I'm not really certain I think major excesses of anything is good for anyone. It would be interesting to be a nurse 30 years from now, to see how things have changed.

It was a wild ride to see how things changed in the 30 years I was in nursing. morgan

P.S. Thanks for getting the info Michelle. It sounds like a lot of over lapping and mystery stuff, even with the big guns. Time will tell...

[Poohbear]

Thanks for asking Dr. Raj this question! I'm glad he was able to respond (and so quickly too!)

I actually have a high bp and it was recommended I increase salt and fluids and I can tell you it helped a lot. I'm assuming for the same reasons momdi mentions and it was Vanderbilt that gave me my instructions so it looks like on this particular component Dr. Grubb is the "odd man out" so to speak.

I'm so glad we have a handful of Dr's who continue to do their best trying to sort these issues out. I think it will be years before they have clear, confident answers for us. I was diagnosed about 10 years ago (When NCS was developed as a diagnosis) and it is AMAZING how far things have come in that amount of time and yet there are still more questions than answers.