kitt

Flatout,

Am sorry you're going through this.

I'm confused but trying to understand.

You say: Pasting....

"But the clincher for mayo it seems was the dubious Holter monitor."

So did Mayo have your records in hand when they denied you for a dysautonomia eval?

I've never known Mayo to ask for records prior to a visit. Only a referral.

Can you share how all that came about? The holter monitor data. How did they happen to have it?

I'm very sorry you're having this experience.

I've been to Mayo in many times and have never heard of anything like this. I'm confused, but trying to understand this dilemma.

Thanks for sharing,

K

I was dxd hyperpots at Mayo and also hypovolemic via sodium and eventually blood volume testing. I don't know a lot about it, but my doctor explained that the hypovolemia causes a drop in bp so my body dumps a bunch of NE to raise it, which causes a kind of rebound hypertension and the high bp and high NE levels. Thus, I have to first salt and fluid load to build blood volume so I'm less likely to get the hyper episodes; the beta blocker then lowers the heartrate (and sometimes the bp too much). I will go from 90/60 flat to 140/90 within a couple of minutes, so I experience both ends of the bp spectrum. I also had abnormal QSART results and neuropathic issues.

I have hyper pots but clonidine causes rebound hypertension in me because my bp gets too low. Perhaps this distinction is not useful, as I think Rama has suggested (sorry...I don't always understand everything you say ); I'm thinking one day we may find they have subgroups based on cause, rather than manifestations of the syndrome (as "hyper" pots sometimes seems to be).

I don't really find this classification helpful; in fact, sometimes I resent it because they want to apply a one-size fits all treatment to hyper patients (ie you shouldn't salt load because you've got high NE...yet many of us do).

NM Potsie, NOBODY explains things better than you. I always 'get' what you say. You can speak up and I never feel like you speak down.

Thanks for that, and this explanation is great. Makes me smile, cause I always understand what you're saying, and more importantly you've usually helped me better understand what I'm thinking.

Smiles!

K

Kitt just as an FYI I was on 1800 mg/day of gaba (neurontin) for my back pain and the doc said the dosage could go much higher, so 2400 probably isn't excessive.

It did help me sleep but caused horrible emotional side effects--random bouts of crying, depression, apathy, etc. (I am not, nor have I ever been, an emotional person...this was totally out of character for me). These are commonly known side effects of the drug by doctors, but they aren't ever up front about it. It also lowers your seizure threshold, so you can't dabble in it...you have to get on and off slowly and over time or you risk seizures.

Lyrica is very similar to gaba chemically, but works better for some people as far as the side effects go. I was prescribed this but never took it (along with Zoloft, to counter the emotional side effects of the drugs...I just decided no more drugs and opted for surgery).

Not saying it doesn't work perfectly fine for some people...just giving you my experience so that you can watch for those side effects if you choose to try it.

NM Potsie,

Good to know!

I see the doc who prescribed it to me Thursday, so this is timely for me.

Thank you and Janet both for the timing.

My recollection though is that neurotin even at low dose, (400 mg?) seemed to have a 'dumbing' effect on me. It's been a while since I took it, but that was my initial memory. I admit I did not give it a good chance. It just seemed to have a very strong impact though, and I immediately thought..."No, I don't want to take this."

But it was prescribed for a type of pain that I don't associate with hyper pots. CFS pain or neuro pain.

The side effects you had are disturbing.

Janet, did you experience anything like that?

I'm really for as few drugs as possible, but when it comes to reducing sympathetic activity and sleep...I'll try!

Best,

K

"

Blair Grubb suggests otherwise, but nit the other research groups.

How does Dr. Grubb suggest otherwise?

Janet, Thanks much for sharing.

From what I've been told Mayo has told many people that they have a 'hyperadrenergic response' and are in a

'hyperadrenergic state' These are pots patients who've been to Mayo pots patients presenting with extreme hyperadrenergic pots... (NE over 1000) so am still confused about that.

Mayo has recently published a paper that is very clear about pots DX and classifies pots patients in two subgroups. Those with an NE level of 600-999 and those with an NE of 1000 to 2000. They've not acknowledged hyper pots patients with NE levels over 2000. (Am not suggesting people who have been treated at Mayo don't present with an NE higher than 2000, but Mayo has not acknowledged that subset in their peer reviewed publications...(Please correct me with a link if I'm mistaken.)

Janet, back to you.

Good to know neurontin helped you with sleep. I have more neurontin scripts than I can even count. It was prescribed to me many times for pain to due another issue, and I filled the scripts but I only took it a few times. Am sensitive to meds, so try to keep meds at a mimimum... BUT if it helps with sleep, I'll definitely talk with the doctor who prescribes it! Thanks for mentioning it. 2400mg a day sounds like a lot to me. Do you have side effects?

Clonodine caused extreme episodes of tachycardia for me when I was sitting and supine. (Odd, I know.) So I switched to Methyldopa, which is VERY helpful with sleep, but it does allow me to sleep but not until the wee hours of the night. A huge improvement though. Prior to Methyldopa, I was only sleeping for an hour or two at at a time during the night.

I'm interested in hearing about your neurotin dosage and your sleep issues.

Am surprised 10mg of Elavil was discouraged? It's such a low dose. I also was prescribed that, but like most scripts, I also discontinued it (with my doctors knowledge), but if it helps with sleep...I'm IN.

Thanks again for sharing Janet.

Best,

K

All patients with pheochromocytoma have hypovolemia as the excess cathecholamines reset the volume status at a lower level thru a complex mechanism. I have hyperpots with very elevated NE levels (3400) and have severe hypovolemia (by blood volume testing at Mayo) which my Mayo autonomic neuro MD felt was due to the very high catecholamines (same as the pheo mechanism). There is certainly also a vicious cycle created whereby the severe hypovolemia (which in my case was associated with ischemic ecg changes) then triggers the release of more catecholamines. In contast to many pots patients, my renin and aldosterone levels were appropriately very elevated. My tachycardia went away completely once I got on florinef and salt tablets. One of the mechanisms of this presentation is felt to be due to antibodies to alpha and/or beta adrenergic receptors.

Hyperpots, thanks for chiming in. You have hyper pots, and also HIGH BP, yet you're taking salt tabs for the tachycardia?

You didn't mention high blood pressure with regard to salt loading. You're hyper and salt loaded. Do you continue to?

Did any doctors express a concern with high salt intake due to high BP, renin and aldosterone levels?

Kitt

not sure if this is the 'right' answer but I believe there was a post here a while ago mentioning the research of some drs from Boston suggesting that the salt increases BP by adrenalin, not by expanding the volume. Maybe this is the reason why salt is not recommended for hyper POTS patients?! Just speculating.

Alex

Alex, you make a good point about salt increasing BP, but I don't find any information that it's caused by adrenalin?

Your post though made me realize though why salt loading (perhaps THE primary reason?) is NOT recommended for hyperadrenergic pots patients. MOST hyper pots patients have high BP, although not all. Their are few of us on Dinet with moderate and extreme hyper pots who have low BP. I'm one with hyper pots and low BP, so I know why my doctor was quick to prescribe the picc line and saline infusions.

Here's a link that explains that doctors really don't know 'why' salt increases blood pressure.

http://www.ncbi.nlm.nih.gov/pubmed/16467498

So not sure what you mean by adrenaline causing high BP? Would be interested in reading anything you might find though.

Diabeticgonewild,

No need to apologize to, (certainly not to me!) Just wanted to point out that the person who writes that blog does a great job and does a service to pots patients. Wish more people were able or willing to write a comprehensive blog and provide good information.

Diabetic, you provided a great piece of information on hypovolemia!

You wrote: Pasting...

that a "Daxor blood volume test" could be useful in determining low retention of fluids in hypovolemic POTS. Only certain hospitals have this machine.

Good information Dietbeticgonewild! Thank you for posted that.

Prompted me to look for what hospitals have this machine, and found the link to exactly which hospitals have the machine.

Here's the list by state and city...

http://www.daxor.com/clientlist.asp

Somebody suggested, on a blog nonetheless, that a "Daxor blood volume test" could be useful in determining low retention of fluids in hypovolemic POTS. Only certain hospitals have this machine.

Diabeticgone wild,

You mentioned a suggestion and used the phrase 'on a blog nonetheless'

This girl does an excellent job on her blog. I don't read it regularly, but she does an excellent job of blogging about pots.

I have a script to have a picc line put in to start saline IV's but haven't done it yet.

Hope to get some improvement. Have read of MANY pots patients getting saline, and they report a very significant improvement.

What confuses me though is that salt loading is not recommended for hyperpots patients.

Am confused by this. Does anyone know why?

My dopamine level was also elevated at 56 but the focus seemed mostly to be on my NE level which was 2922 so nothing was really said about the dopamine level being elevated. Something I want to ask about next time I go to Mayo because I have since wondered about the significance of an elevated dopamine level. I did notice on the Dinet Mechanisms of Pots page a paragraph about a hyperdopaminergic state and some references.

Janet

Janet,

Thanks for sharing your catecholamine levels. What meds do you take for hyper pots?

Are you able to sleep with an NE level that high?

Think the dopamine level being so high is something that is considered an 'incidental finding'....Unlike a very low dopamine level.

Mine is very high too, and no doctor has given it much significance.

Have the script and 'stuff' to do a 24 hour urine catecholamine test with an endocrinologist, but haven't gotten around to doing it yet. Will take the dopamine level up with her.

Am more concerned about serotonin levels. When dopamine is high serotonin is low and vice versa, but don't think it's something a doctor will necessarily 'treat'.

From my paste above it states "Remarkably, baseline plasma DBH activity in healthy indi- viduals varies more than 100-fold. Individual variation in DBH activity is primarily genetically determined."

A 100-fold variation in dopamine is huge, so I'm guessing that may be why it's not looked at too seriously.

Although from the posts here it seems to be common with both pots patients and hyper pots patients.

Janet, can you provide a link to (pasting from your post):

Dinet Mechanisms of Pots page a paragraph about a hyperdopaminergic state and some references.

Best,

K

Hi Kitt,

Sorry for not being clear in the earlier message! No, I didn't talk specifically to Dr. Grubb to see if he'd changed his ideas on hyperPOTS. It is the reference range listed on the lab paperwork that says 520. I don't have a scan of it right now though.

So probably the cutoff for hyperPOTS is still 600, but for some reason the hospital lab lists the "normal" range from 80-520 on the lab results page (even though other labs list the "normal" range as 700+.)

Also, the lab paperwork used the same reference ranges for both supine and upright. Weird.

Carrie,

When did you last see Dr. Grubb? It's odd about the NE reference ranges being the same standing and supine. Doesn't make sense in terms of dysautonomia.

As to your initial post, I still think you're safe to conclude you're not in a hyperadrenergic state. You may want to have catecholamine testing redone at some point, but all experts including Mayo, Vandy and Grubb have determined that 600 is the bottom line for hyperadrenergic pots.

Many with an NE in the 600's have been dx. as 'borderline' hyper pots by Mayo. Interesting.

Any research you find regarding a high dopamine level would be interesting. The only articles I found regarding HIGH dopamine and pots suggest a low serotonin level as I mentioned. I've researched extensively and other than articles referencing low dopamine levels, I didn't find anything significant. Please share what you find regarding HIGH dopamine.

Carrie, how high is your dopamine? Can you share the number and the reference range? Mine was more than double the norm on quest labs.

Am wondering if only a psychiatrist who specializes in pharmacology would know how to approach balancing these two neurotransmitters, (dopamine and serotonin). Can't imagine what other kind of doctor could sort this out?

Best,

K

High DBH...

vity (51).

Remarkably, baseline plasma DBH activity in healthy indi- viduals varies more than 100-fold. Individual variation in DBH activity is primarily genetically determined. Among the poly- morphisms found in the DBH gene that affect either enzyme levels or function (3, 4), the 1021C3 T polymorphism in the promoter region accounts for up to 52% of variation in plasma DBH activity (5).

Because of individual variability in DBH, associations be- tween DBH activity and various diseases have been elusive (25).

Elusive...Indeed.

Most of the literature about dopamine and illness concerns LOW dopamine, not high. The best articles I've found correlate high dopamine with low serotonin.

Dr Grubb is now saying that an NE of 520 is indicative of a hyperadrenergic state? This is contrary to everything I've read thus far. Vandy, Mayo and Dr. Grubb all seem to agree that 600 is the 'bottom line'.

I've not seen anything in any literature referring to Dr. Grubb and hyper pots and an NE of 520. Carrie, do you have a link to this?

Mayo has recently divided hyperadrenergic pots patient into two subsets. Those with an NE of 600 to 999, and those with an NE of 1000-2000.

I'd be interesting though in reading anything you can share that suggests Dr. Grubb has a different opinion.

Thanks for sharing Carrie.

Carrie,

I also have very high dopamine. More than double the norm.

You'll find a lot of information regarding serotonin and dopamine. When one is high the other is low in equal proportion. I have a good article 'somewhere' that explains it. Both should be like a 'half a glass of milk' when either dopamine or serotonin is high the other is in equal proportion low.

My norepinephrine is very high though, over 1400. Think you can feel comfortable that you're not in a hyperadrenergic state.

Best,

K

Another way to look at it would be - do symptoms of sympathetic activity predominate or does dizziness/weakness?

Rama, can you explain why you differentiate between sympathetic activity and dizziness/weakness, meaning why are the two symptoms indicative of a different causation? Are you saying people with dizziness and weakness do not have excessive symptoms of sympathetic activity?

It's interesting as I obviously have excessive sympathetic activity and generally don't have dizziness or weakness. Why is dizziness and weakness NOT a sign of excessive sympathetic activity?

Thank you for all of the comments!

Kitt, "fortunately" my main problems have been with temperature regulation, fibromyalgia, fatigue, and anxiety. Things feel manageable right now, and I've been lucky not to have headache, GI problems, or brain fog.

Ramakentesh, what a treasure chest of information! Thanks! QSART is not something I've read much about, so you're right that it is missing from the presentation. You've given excellent info. to get me started learning about it. I also didn't know much about the angiotensin II / aldosterone differences. All of this points to the idea that no-one's POTS is really the same thing. We all present with a similar end result, but figuring out the cause is going to be unique for each person. For example, I've always had low blood counts (RBC, WBC, electrolytes, minerals, hormones), pointing to HYPERvolemia causing a dilution of everything else. At the same time, most POTSies have HYPOvolemia. It's hard to explain both sides! You're also right that lots of work has to be done for hyperadrenergic POTS. Many people present with the hyper symptoms but have normal catecholamines. I also have the "hyper" form but low blood pressure. :-/

I really appreciate all of your suggestions. You know many things I do not yet!

With regards to the TH and COMT-- no, I've not seen studies relating SNP differences to POTS in particular. I was mostly referring to Amy Yasko's work and extensions of that in the CFS community-- more with the mood and mental symptoms rather than with tachycardia.

Thanks again. :-)

Carrie, can you share what kind of problems you've had with temperature regulation? Have you managed to resolve this? Any tips on how you did that?

Inability to regulate my body temperature is my second worst symptom with hyper pots, (First being the inability to sleep). I go from hot to cold to hot, and am sometimes hot and cold at the same time, (fire and ice sensations). I also have extreme 'sweats' particularly during the night.

Like you I'm hyper but have low blood pressure. Are you basing your hyperadrenergic status on catecholamine testing or something else?

Thanks again for sharing,

K

http://www.ncbi.nlm.nih.gov/pubmed/21947988

Here is one study showing the criteria for HyperPOTS. There is an increase in NE with standing above 600pg/ml. or an increase of systolic of 10 mm Hg.

There is also an association with HyperPOTS and MCAS. Symptoms like surges of HyperPOTS can happen without postural changes in MCAS. Here is a study on that:

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

Interesting thing with this study is that those with MCAS and OI (lower bp's) had higher NE levels with standing then those with MCAS and POTS and also those without MCAS and POTS. This might help to determine why some with high NE levels could have low bp's. (a connection to mast cell problems)

This study list the subset types of POTS that was known at the time of the writing of this paper.

http://www.medscape.com/viewarticle/522421_3

Issie

Good information Issie! I had not seen information regarding NE levels and BP data and mast cells.

Very, very fortunate that you had a nurse practitioner who was determined and went the extra mile to help find your dx.

I find most doctors don't take the time to Google on the internet looking for answers.

My experience with endocrinologists, (I have Hashimoto's Disease) is that they order tons of lab work, and use that data as their basis for all diagnostic and treatment plans.

Many patients with CFS have an HPA axis disorder. HPA = Hypothalalamic-Pituitary-Adrenal

It's almost impossible to find a endo who's willing and able to think outside the box, and figure out how to test for this.

You're lucky indeed to have encountered the nurse practitioner that you did.

Thank you again for sharing your story.

K

On the topic of food, am passionate about this topic.

About 8 years ago I gave up all sugar and sugar substitutes, and it's been the most significant thing (in my control) I've ever done for my health.

Here's a video that 60 minutes aired that claims sugar is toxic. Well worth watching.

http://www.cbsnews.com/video/watch/?id=7417238n

Outstanding! Thank you for sharing this.

While watching I kept thinking 'Cognitive dysfunction didn't affect her!' Bravissimo.

K

Diabeticgonewild,

You've done a great job on this topic.

Not only do you have AAG, but you've cleared up several misconceptions. Notably, that the primary symptoms listed are not always present. You listed your symptoms which in some cases are opposite of those listed, and made a technical topic 'real'

The links you provided are excellent, but it's your personal experience that I appreciate most.

Interesting that it 'started in your bladder'...How in the world did you go from experiencing 'urge incontinence' to getting AAG dx? You're fortunate to have a diagnose with this rare disease. Can you talk about that experience?

Thank you for sharing your personal journey with AAG.

Wishing you the best,

K

i Kitt

Dr Vernino was THE doctor at Mayo that identified (among others) the acetylcholine a3 nicotinic autoantibodies firstly in obvious cases of orthostatic hypotension and then it was suggested in some POTS patients. The work was published but critics of the paper suggested that the number of POTS patients that exhibited these autoantibodies were low (10-13%) and more importantly the titer levels in the POTs patients tested were TINY.

All sorts of people have all sorts of low titered autoantibodies in their bodies without any demonstratable pathology. As an example Ankylosing Spondylitis patients have over 150 weird autoantibodies floating around, most inactive.

Subsequent to publishing the early study on the a3 autoantibodies in POTS, Dr Theiben published anotehr paper suggesting that because of the presentation of POTS it is likely autoimmune.

But then another major research group were unable to find the a3 nicotinic autoantibodies in any POTS patients. And when Dr Vernino left Mayo and was hired by Ben Levine's group in Texas they were unable to replicate the previous results - that is the same doctor from Mayo who identified the a3 nicotinic receptor autoantibodies and implicated them in POTS was unable to replicate those results.

So AAG and POTS remain seperate entities.

And of even more interest was the fact that a Mayo related student published to more recent papers on new autoantibodies in POTS but all the patients in the cohort had profound orthostatic hypotension, excluding them from a diagnosis of POTS according to the recent consensus statement.

Hi Rama,

As I said, I'm very familiar with the fact that pots and AAG are separate identities.

Am going to stand behind the links I've already posted, and let them speak for themselves.

Mayo has a panel of over ten different antibodies that they check for in pots patients looking for an autoimmune cause to pots.

To your point that, (Quoting you) "All sorts of people have all sorts of low titered autoantibodies in their bodies without any demonstratable pathology"

Absolutely true! Thank you for making that point. Many, many times people have low titers of antibodies, and Mayo will be to the first to tell you this is 'an incidental finding', and not meaningful. Mayo is very quick to find all sorts of anomalies in their exhaustive, and I mean exhaustive! testing, and I know firsthand they consider many of those anomalies, (including low levels of various antibodies to all sorts of things) to be 'incidental findings', and not meaningful.

Rama, I've been to Mayo more times than I can count, and have had several major surgeries there. To suggest that a pots-related Facebook page has a doctor claiming (quoting you) that Mayo now send may of their POTS patients to him because they 'cant treat them' seems absurd to me.

Am curious to see the Facebook page please?

Mayo is far from perfect, but it's one of the finest diagnostic facilities in the world, and many of us have not only been treated there, but been helped tremendously by Mayo. I know you're not in the US, but Mayo is in a class unlike any other.

Also regarding hypotension...You're saying hypotension rules out pots? Not in my case. I have hypotension and I have hyper pots. Not unique. Unusual but not unique.

Am guessing you agree that many pots patients have an autoimmune etiology?

Some of us with other autoimmune illnesses have good reason to suspect we may well have an autoimmune basis for our pots.

When I was dx with Hashimoto's years ago by Mayo they told me that it put me at greater risk for developing Addison's 'later on'. When a person is dx with one autoimmune disease, it increases their risk of developing others.

Thanks for sharing Rama. Let's try and move forward and share data that may help others looking for information on autoimmune pots data.

Best,

K

Kitt,

AAG stands for autoimmune autonomic ganglionopathy - not the same thing as POTS.

the titres of the AChR ab are much more elevated in AAG than those found in some POTS patients.

If you read the symptoms associated with AAG - one of them is the blunted HR (lack of variability in HR - that's quite the opposite in POTS)

I think rama is strictly talking about POTS.

Alex

Alex, I understand what AAG stands for.

It's an autoimmune issue that can be found in pots patients. I posted the symptoms of AAG.

Here's the description under Autonomic Disorders Consortium. http://rarediseasesnetwork.epi.usf.edu/ARDCRC/patients/learnmore/AAG/

And from another study, (Dr. Low at Mayo)

Pasting:

Approximately 50% of cases report an acute or subacute onset, often following a viral illness, suggesting an immune-mediated process. In a recent review (Thieben, 2007) of 152 cases of POTS seen consecutively by Sandroni and Low, 6 of 42 patients (who had ganglionic antibody measured) had increased levels of antibody.

Link:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2671239/

AAG is a rare disease, but it is one of several forms of autoimmune disorders that can be associated with pots.

Alex, I'm not suggesting that pots and AAG are the same thing. I'm talking about people with pots who also have ganglionic antibodies, thus indicating an auto immune form of pots.

Best,

K

Vernino now works with Ben Levine and the two of them were unable to find the a3 acetylcholine nicotinic receptors in any pots patients.

Rama, Perhaps Vernino was unable to find a3 acetylcholine nicorinic receptors in pots patients, but Mayo has.

Pasting:

The pathophysiology has been well characterized owing to the discovery of nicotinic ganglionic acetylcholine receptor antibodies in patients with AAG, though at least half of cases presents without detectable autoantibodies.

Here's the link.

http://www.neurology.org/cgi/content/meeting_abstract/80/1_MeetingAbstracts/P03.024

Thank you Diabeticgonewild for sharing your treatment protocol.

I had plasmapheresis done many years ago. My husband was the donor. Are you using a donor or are they using your own blood?

How lucky you are to have a neurologist who understands how to treat you!

Is it someone local or someone from a big clinic, like CC, Mayo or elsewhere?

How are you responding to treatment?

Sue1234,

Sorry! I didn't mean 'you' personally at all! Just have had people insist that all hyper pots patients have high BP, and while that's usually true, it's occasionally not true.

As to the autoimmune testing Mayo does. It is not one, two or three tests. There are at least ten different tests including:

Anti-neuronal Nuclear Ab

N type Calcium channel

Achr Ganglionic neuronal Ab

Etc....

Don't have a link yet.

Here is a link though that describes symptoms associated with AAG.

http://rarediseasesnetwork.epi.usf.edu/ARDCRC/patients/learnmore/AAG/