New. Just Diagnosed (Sort Of) At Mayo. Lots Of Questions.

[Kris4444]

Hi,

My name is Kris and I am confused and exhausted and hoping that you can give me some information and support. I just spent almost a week at Mayo in Rochester, MN where they did ANS testing on me. I was told that I don't have POTS but rather a "hyperadrenergic state".

My norepinephrine levels are 656 supine and 1199 upright. My blood pressure goes up when I stand. I asked the doctor if I had POTS and he laughed and said no. Yet when looking at my medical summary from Mayo my diagnosis is POTS. After doing a ton of searching I realized that I might have what is known as "Hyper Pots" but I understand that Mayo doesn't use that as a diagnosis?

All of my symptoms started back in 2008 with a terrible EBV infection. At that time I was dealing with Fibromyalgia and some herniated discs but other than that was fairly healthy. I was a professional horse trainer and very fit. I was 5'9" and 160 pounds. We could not seem to get the EBV under control. My titers were 2,850. Soon I began having issues with the tendons in my arms and feet. I had extreme pain in my joints and developed Raynaud's phenomenon, severe colonic inertia (I could not have a bowel movement), flushing, malar rash, excessive sweating and vertigo when I would stop exercising. After seeing many rheumatologists I was diagnosed with scleroderma. This diagnosis didn't seem to fit so I made an appointment with the leading sclero doctor and was told that I don't have sclero, rather I have undiferentiated connective tissue disease or UCTD because I had no positive ANA and no dialated capillary loops in my nailfolds to fit the diagnostic criteria for sclero. I did exhibit some signs of lupus (the malar rash) and sjogren's (dry eyes and mouth) and the gastroentestinal issues which could be related to sclero. I also ballooned in weight and I now weigh 225. I have NEVER been this big in my entire life!

The flushing, sweating and vertigo started taking over my life. I would have vertigo when I would ride my horse and when I exercised. I can sweat for up to 3 hours after a ride or run. I flush when I eat or drink alcohol, this can bring on sweating as well. The vertigo was at it's worst when I STOPPED exercising. I would get tunnel vision and the world would spin and I found myself very scared on more than one occasion that I would pass out. This has kept me from riding as well as exercising which I know is not helping my condition. I also developed cold urticaria and severe intolerence to heat. I tested positive for cryofibrinogen but was tested again for it at Mayo and it was negative.

Mayo was very thorough, they left no stone unturned. I saw a neurologist, gastroenterologist, vascular doctor, ENT, cardiologist.

They found that I do have severe vasospasm in my fingers when immersed in ice. I have fluid in my mastoid sinus area (another long story there) high norepinephrine and high normetinephrine. The gastro doc was a waste of time. Once she saw that the neuro didn't think I had an ANS issue she basically wrote me off since she was there to determine if the ANS was causing my constipation.

In August something strange happened. I started seeing a new GI doc for the constipation. He started me on Pristiq which is an SNRI. Two weeks later I was having severe diarrhea, up to 12 times a day. I was able to get off all of the laxatives I was on but somehow ended up hospitalized and after doing a biopsy they discovered I had microscopic colitis. How can this be when I've been struggling with constipation all this time?

I am still taking the Pristiq, which may be causing the high NE levels but it is the only thing that makes me have a bowel movement. The colon issues were so severe without it that I was having to do a bowel prep every Friday night after work and spending all weekend on the toilet. This is no way to live.

I had the flushing, vertigo and sweating BEFORE starting the Pristiq but the sweating definately got worse on it as well as my headaches (I have had migraines since I was 13).

Anyway, I guess I am just looking for opinions on what you think this is? Could it be hyper pots? Could my connective tissue disease be related to Ehrler's-Danlos? I have extremely hypermobile joints, especially my elbows, wrists and thumbs (thumbs dislocate very easily as well as my shoulders)

I meet with my rheumatologist on Friday. He is a God send and I know he is hoping that the testing from Mayo will give us new insight and possibly a new way to treat my many conditions. Any information that you can enlighten me with would be great. Thanks for reading my novel!

-Kris

[Kris4444]

I forgot to mention that they prescribed clonidine. .1 once a day to start and gradually building up to .2 three times a day. I am finding that even the small amount adds to my fatigue.

They did say it would be a delicate balance between the clonidine and the Pristiq. All I took away from Mayo was yet another diagnosis and another medication. I am beyond frustrated and confused.

[Chaos]

Hi! Welcome! SO sorry for all you've been going thru and continue to go thru.

So I'm a little confused...they said you didn't have POTS, but then your medical record summary says you do have POTS? Did you get any kind of report from the neuro about your ANS testing results?

When I see the uticaria, flushing, high norepi levels, GI issues it sounds like at least it might be worth looking into Mast Cell issues. Have you checked out Mast Cell Activation Syndrome as a possibility? There seems to be a fairly significant number of people who have MCAS as an underlying cause of their POTS type symptoms and having those high norepi numbers seems to fit that profile perhaps more than some other types. I see your BP goes up when you stand. What does your HR do?

The other possible thing that leaps out at me is the whole EBV trigger. Have you seen a good immunologist at any point?

[anaphylaxing]

Hi!

We share a lot of the same issues....I also didn't get diagnosed at Mayo.

I have POTS, joint hypermobiltiy vs ehlers danlos, and ?mast cell activation syndrome (flushing and other symptoms induced by triggers such as heat exercise , chemical exposures)

when I get a lot of exposures I also get night sweats

Did someone check you tryptase level?

All my labs are normal but i've improved with conservative pots treatment, strict trigger avoidance and an organic rotation low histamine diet, exercise etc

Hope you figure out what works for you

Ana

[Kris4444]

Chaos,

Welcome to my confusion! I am guessing that the diagnosis on the summary is for the coding for insurance? I have no idea. It lists all my diagnosis' with POTS being the first one listed. Maybe there is no code for a hyperadrenergic state? He used that term interchangably with hypersympathetic state. I asked him directly on day two when I read his notes and he said I had the hyper state if it was POTS and his answer was no, yet like I said, on the summary that is the diagnosis.

Yes there is an extensive report from the neuro using the above terms. All of the ANS testing was normal. My exceptions were the high epi levels and high bp, flushing, sweating etc.

I believe my endo did a work up for mast cells. I know at Mayo they were unable to test for histamine for some reason. My tryptase levels were normal.

Ana we do have a lot in common! How did you finally end up getting your diagnosis and how are you treating it? Any help would be greatly appreciated.

I meet with my rheumy on Friday. I think he will run more tests. Maybe I should schedule with my endo too since the epi levels are high? I really wish they gave me more direction upon leaving Mayo as to who I should see. They said my GP but this is way over his head.

-K

[Christy_D]

You sound a lot like my son. He was diagnosed with POTS in Cleveland, but at the Mayo they said he only had POTS symptoms..not POTS, because a 10 minute TTT didn't catch the POTS. The TTT he had in Cleveland was 40 or 45 minutes tracking minute by minute. The TTT at the Mayo, they only checked his hr/bp every 5 minutes, not very thorough. They also didn't catch his mast cell activation syndrome at the Mayo. His tryptase levels were fine. Dr Afrin in South Carolina did more extensive testing to catch the MCAS diagnosis.

We have a local neurologist to handle the POTS and a local hematologist who communicates with Dr Afrin in SC for the MCAS. This was all way over the pediatricians head as well..

[Kris4444]

Yes they only did a 10 min ttt for me as well. They didn't test for histamine although they wanted to but I was told something was going on at the lab where they couldn't run those tests...weird.

I have not seen an immuonologist. Maybe I need to do that or maybe I need to just get off all this stupid medication and see if my body corrects itself. I just want my life back. I miss being active and physically fit. I'm tired of being tired.

[corina]

Hi Kris, welcome at the forum! I cant really answer your questions just wanted to welcome you!

[Chaos]

Tryptase levels will usually be normal in MCAS. If you were to have systemic mastocytosis then they would usually be elevated. It's really hard to get a MCAS diagnosis from your regular docs, even allergists because it's not well known and because most of the factors that indicate it disappear from the blood within 1-2 hours. So you have to get to the lab, ( a lab where they know how to do the tests correctly, which is no easy feat) and get the tests run and have them processed correctly etc etc. That's on top of having a doc who knows which tests to run....also not an easy feat because these aren't your normal run of the mill tests. OBVIOUSLY the best and safest approach is to find a well qualified doctor who can order these tests and work with you to find out if this is your issue. Dr. Afrin in SC is one of those that several people on here have had good success with. Another is Dr. Castells in the NE part of the country.

Since that's not an option for everyone, a lot of people on here are doing this by trial and error to see if it helps their symptoms and taking H1 and H2 blockers on their own because that's the treatment for MCAS anyway and these are available over the counter. H1 blockers are things like claritin, zyrtec, allegra and benedryl. H2 blockers are things like pepcid, prilosec or zantac, I am not telling you to do this. I'm telling you what I, and others on here have done. I feel for you having gone thru all you have gone thru and having gone to a big place and come away with no answers. Also note that many people with MCAS need much higher doses or prescription meds to treat their symptoms so this is a first step only if you decide to try this on your own.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3069946/

http://hopealways.wordpress.com/2010/06/12/must-read-for-all-p-o-t-s-patients-and-their-doctors/

These are a couple of links to papers you might want to read and take to your doc about MCAS if you feel like it fits your situation. Below is a brief/concise explanation of the theory as to how mast cells could be involved with all this:

Here is the 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."

[Kris4444]

Hi Corina. Thanks for the welcome.

Chaos - This is a lot of great info. I will take a look at it at home and respond back then. Thank you!

Oh and we have sent my labs to an infectious disease doc and he said he didn't see any reason to do additional testing...

-K

[Katybug]

I agree with all of the above. I am another person with POTS, mcas, and eds (and horses). Of sounds like the same story that most of us with these symptoms have gone through. I would encourage you to have Lyme and Lyme co-infection testing done also if you haven't already as that can mimic these other syndromes and you are at high risk for exposure to ticks working around horses. I miss being able to ride my old girl. One of my former students exercises her for me now and I watch from the side lines if I can take the weather. :-(

[Guest Alex]

Kris,

I have no answers for you - I'm low flow POTS supposedly, but I wanted to stop by and welcome you here.

Lots on your plate, but I hope you'll find some answers here.

Good luck finding the right dr for you and the best course of treatment.

Best,

Alex

[Kris4444]

Katybug,

I haven't ridden in over a month. I have cold urticaria and will wake up covered in hives the day after I ride if I got cold. This summer was the first time that I was intolerant to the heat. I couldn't ride for long and when I would get down I would be bright, bright red, sweaty and have vertigo. I have had several people tell me that I needed to get down just because of how red I get. I used to ride multiple horses a day and teach lessons in the heat and cold. I have Raynaud's too which complicates things. I no longer recognize this life or myself. i really thought Mayo would figure this out. At first I was happy that at least we had a name for this and that I wasn't crazy. Now I've realized that all I got was another name for something that they can only treat the symptoms of. Very depressing. I am fairly certain that I've had the work up for lyme early on. I'm sorry you don't get to ride anymore. I don't know what I will do if it gets to that point. What kind of horse do you have and what discipline did you ride?

Alex, thank you for the welcome! it's nice that so many people have reached out. I really feel welcome!

Kris

[kitt]

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[Kris4444]

Hi Kit,

I'm sorry, maybe I wasn't clear. I didn't fail the TTT. They said it was normal. They did have me wear a holter and that was normal. The only abnormal findings were the high NE and normetanephrine and my flushing which was very obvious.

I called Mayo today and asked about the diagnosis on my summary. They transferred me to neuro and at first the girl said that if it's on the summary then that is the diagnosis. She put me on hold and came back and said that sometimes they use what is closest for coding for insurance like I thought.

I do have trouble sleeping sometimes and always wake up tired.

I see my favorite doctor on Friday. Fingers crossed that he will have a plan, maybe even test for mast cell stuff.

I rode my horse tonight for the first time in about 6 weeks. Only had some dizziness when I got down and a headache so maybe the clonidine is helping. They took me off of spironolactone. Maybe that is why I'm feeling better?

Thanks for reaching out. I appreciate everyone's comments and helpful insight.

Kris