New Diagnosis (Not Pots Just An Overreaction To Norepinephrine), Any Ideas?

[Beckles]

Hi all,

So I've had a long struggle with diagnosis... (it started over 20 years ago)

About 10 years ago a neuro decided that it must be POTS (though strange because I frequently get fast HR while lying down) but never bothered to do a tilt table test. He put me on propranonol and said that was about the best they could do. It helped a bit and I struggled through, got a little better for a few years and then got worse again. I then started to see a new neuro who said 'yep it's probably POTS but lets do that TTT to make sure, and now we know about some other things we can do for POTS'. But then I got pregnant and my symptoms vanished, not very surprisingly the TTT came back negative. The neuro thought it was the increased blood volume and that the problems would return soon after delivery. They didn't (despite large blood loss). They did return 1.5 years later when I stopped breastfeeding though. So I went to a new doctor (cause I'd moved). He did a 3 minute poor man's tilt test got a mild HR increase no change in BP, obvious blood pooling in the feet and me with just mild dizzyness. Anyway, after this and looking over my history he decided that it wasn't POTS but instead that my autonomic nervous system was overreacting to norepinephrine.

I'd never heard of this and haven't been able to find out much about it.

So I thought I'd ask the wisdom of the forum and see if anyone had heard anything about it. Is it similar to hyperadrenergic POTS?

From the way the doc described it, it seems that my body is not responding normally to norepinephrine, not just that I have too much of it but that my body is reacting wrongly to what's there.

He said there's a test for it but he doesn't want to do it because it's difficult, uncomfortable and not conclusive.

He said there wasn't a treatment either but they could do things that might help with the symptoms: the normal high salt approach and then possibly putting me back on propranonol.

Thanks!!!

[MomtoGiuliana]

I have not heard of this diagnosis. Although it sounds like POTS (to me).

My only thought is that POTS can be quite variable from day to day for many people. I know I do not meet the diagnostic criterion of HR spiking 30 bpm on standing every day. Perhaps you only had a mild HR change on that particular day you had the test. BTW, change in BP from lying to upright doesn't matter for diagnosis--some people w POTS have BP changes up others down, others no significant change.

[anna]

Did your Dr. say anything about NET disorder?! http://en.wikipedia.org/wiki/Norepinephrine_transporter

[issie]

For me, I have high NE and definite dysfunction with it. They said I have HyperPOTS because of high NE levels and I have higher bp levels too. The drug they have me trying is clonidine - I'm on my 6th day and it is helping the tacky issues. I was having them every hour and it was waking me up. It has slowed down considerably and my bp is in the more normal range with sitting now - but, not while standing. So, seeing some improvements. The other drug I know that I've also been on before the clonidine that lowers NE levels is Tramadol. There's things that can be done for it. I can't do the betas because of MCAS (MCAD) problems. Betas cause degranulation of the mast cells.

There's still hope and things you can do. It does however sound like HyperPOTS. We do have good days sometimes and rarely we might not get the 30 point hike. If you're really hydrated - you might not get it.

Issie

[Guest Alex]

Beckles,

sorry to hear about your problems, but hopefully you can find some answers here

In my case it's not my dr who is challenging the POTS diagnostic, but the patient. Like you mentioned, I do have occasional increases in heart rate of 30+ bpm, (now I'm loading on water and salt, plus taking a whole bunch of meds for POTS so the readings are definitely influenced by them), but I also experience most of my symptoms while laying down. Spikes in BP (horrible feelings associated with them) aches and pains, pins and needles, tachycardia sometimes associated with shakiness, and the dreaded chest pain. More than that, before having a POTS label, I used to have these "panic attacks" in the middle of the night (not precipitated by bad dreams or anything like that as the ER dr's tried to convince me) or early in the morning.

I have no blood pooling, no dizziness, and what is mostly frustrating is that my dr has no plausible explanation for me - at least not yet. I inquired about hyperadrenergic POTS but I was told I definitely do not have that. I've had my adrenal glands checked and re-checked (blood work, 24 h urine, CT scan, ultrasound) and they are fine, as sometimes they might be playing a role in this.

Have you tried getting a second opinion? What about having your adrenal glands checked out? Or if you want to stick to the dr you're currently seeing, ask him more about that test s/he mentioned to you, if you think that's going to put your mind at ease.

I am taking propranolol 10 mg in the AM and 10 in the PM and still get "adrenaline surges" quite frequently. I'm afraid of increasing the dose as my BP has always been on the low side. Before my POTS diagnostic I was on bisoprolol with horrible side effects, and no resolution of my symptoms.

Good luck finding some answers, and I'd be curious to learn more about the test your dr mentioned to you.

Alex

[Beckles]

Thanks guys! It's wonderful just to talk about this with people who understand.

MomtoGiuliana: Yeah I do wonder if POTS is so totally off the cards. The only proper TTT I've had was when I was completely symptom free and I know in general my standing threshold varies greatly, sometimes I'll get a 30+ bpm increase within one minute and other times not within the first 10. I might talk to the doc again about it next appointment and see if we can do the test again.

Anna: No he didn't. That does look interesting, though different from the impression I got from his description. He said there wasn't any good name for this syndrome and so he'd just explain what he thought was happening. I'm definitely planning on pressing him for more details next time. This time I was just so surprised by the diagnosis change I didn't think of that many questions.

Issie: Thanks! From reading about POTS the hyper version always sounded more like me, I do get some high spikes in my BP but it's generally pretty normal/lowish. How did they test your NE levels? Is it a particularly complicated procedure or just a blood test or something?

Alex: Sorry to hear about your problems too! Yeah I've always wondered about the POTS diagnosis since standing hasn't been my biggest problem (though I can't do it for that long, fall over after 10 minutes or so). I'll definitely keep you posted with what info I can gather on this 'new' syndrome and the tests for it. So far all I know is that it's like POTS except you don't get as high a heart rate increase upon standing and you don't loose conciousness (presyncopes are really common but apparently an actual faint would rule this one out), and it's something to do with an autonomic overreaction to NE.

[Chaos]

It sounds to me like a form of POTS and maybe he's just trying to be more specific? I know it's been hypothesized in the past that in some people there is an abnormal increase in norepi with standing and that others have normal levels of norepi but, it was theorized, that they were more sensitive to the norepi and reacted as though they had too much. I've not seen any studies to prove that theory but maybe others have.

As far as the catecholamine test...it's not hard but may be hard to find a doc/lab that knows how to do it correctly outside of one of the major locations like Mayo/Vandy/CC etc. They have you lie quietly in a darkened room for 30 minutes, draw blood thru an IV (started before the 30 minute rest time) and then have you stand by the bed for 10 minutes (or you're on a TTT). Then they re-draw the blood test and compare the values for dopamine, epi and norepi. I had one test that didn't show enough of a rise to qualify for "hyper" and one that did. The one that didn't show the result was one where the nurses were in the room and talking to me for the whole rest time so that may have played a role. LOL

[issie]

Beckles,

The NE test is as Chaos described it - (she and I see the same doc at Mayo) - it is absolutely necessary for it to be really quite when you do the lying 30 minutes because they want a resting almost asleep level and then want to see what happens when you stand. The nurses told me that they put signs on the doors for people in the hallways to be really quite around the rooms that they are doing these test in. Unfortunately, I forgot to turn my cell phone off and it rang right before they drew my resting level - but, despite the scare - I still tested high and as HyperPOTS. So, despite the interruption - got the anticipated results.

Issie

[Beckles]

Thanks!

[targs66]

Sorry if I'm "muddying the waters" here, but could it be neurally mediated hypotension?

I had a normal TTT and was told that the results were "normal" even though my starting BP was 79/48. When tilted upright, my heart and BP don't get drastically higher, which is the response that would indicate POTS. However, I had a TTT about a year later at Johns Hopkins, and they had me hooked up to an IV. During the normal part of hte test, my BP was low-ish, but again I didn't meet the criteria for POTS. However, at the end of the test, they administered a synthetic form of adrenaline (isoproterenol) through the IV and started doing the tilting again. My HR dropped from 107 to 65 and my BP dropped to 67/42.

Apparently that response is indicative of neurally mediated hypotension, where you react abnormally to adrenaline (epinephrine). I don't know enough about the differences between ephinephrine and norephinephrine to know if your responses meet the criteria for NMH, but just wanted to let you know about this particular kind of dysautonomia.

Good luck with finding some answers!

[Beckles]

Thanks targs! I'll definitely look into that.