For those who know their POTS "type"...

[bombsh3ll]

Next week I will hopefully receive the results of my autonomic testing from UCLH in London. 

I had a stand test, and a tilt table test to 60 degrees, both of which confirmed POTS. Pulse pressure narrowed to 20 with increase in diastolic BP to 102 on the stand test, BP went up to 150/98 initially on the tilt test then gradually came back to around 125/90s, still jumping up and down a bit. Max HR was 120, with increment of about 40bpm. 

I also did some breathing tests lying down but NOT a valsalva as that is what precipitated my POTS onset. I do not know the significance of those but believe it relates to sympathetic/vagal balance and heart rate variability. 

My blood catecholamines were tested supine and after 10 mins of tilt. I was surprised I lasted that long but I was allowed to keep on my compression tights, was well hydrated, unfed and had taken 5mg diazepam prior. 

I am a little unclear despite a lot of reading about how subtypes are determined. I strongly believe I am hypovolaemic and narrow PP with raised diastolic would fit in with that, but am not sure if this is considered a subtype on its own. 

I have read in some places that hyperadrenergic pots is determined by a BP rise on standing/tilt, which mine does, and in other places that it is identified by catecholamine levels. Some authors refer to high levels supine, and others to either a defined increment or certain cut off level on tilt. 

The team were very nice but less knowledgeable than the average patient, so I would really like to hear from anyone diagnosed with a particular subtype by any of the top clinics/specialists how that was arrived at. 

TIA,

B xxx

[Pistol]

Hi @bombsh3ll - here is an article about POTS subtypes and their significance ( you probably already are aware of it ) 

https://ww2.mc.vanderbilt.edu/adc/42008

I was diagnosed with hyperadrenergic POTS after 2 TTT's ( one showed NCS and one was supposedly normal but later determined POTS by an autonomic specialist ). I also had basic autonomic testing in a major US autonomic clinic but they failed to diagnose me. Eventually I saw an autonomic specialist who suspected hyperadrenergic POTS based on symptoms. history and HR/ BP readings and then confirmed the diagnosis by testing neurotransmitters ( elevated both resting as well as upright and active, although not even symptomatic at the time ). In my case it appears to be genetic since 3 generations of females in my immediate family have either been diagnosed with it or have symptoms of it ( the young ones have not been diagnosed quite yet ). So - I have a designated subtype and an underlying cause ( genetic ) but it really does not matter in my case since it is chronic and progressive anyway. THAT BEING SAID - not every type of POTS has that prognosis, many types can be managed and even disappear after some time, so yes - knowing what type of dysautonomia you have is very important.     

[bombsh3ll]

That's interesting - I would really like to have the testing offered by Vanderbilt, particularly the blood volume test. We just can't get that in the UK. I believe my "hyperadrenergic" presentation is secondary to hypovolaemia as when I have been volume replete those symptoms, the chest pain, pounding heart, freezing cold extremities, sense of being full of adrenaline/impending doom, tremulousness are all reversible. It will be interesting to see what my blood catecholamines show, although I was only tilted to 60 degrees which isn't really close to standing. I would love to have tangible evidence of the blood volume deficit though. 

I am also no longer hypertensive now off licorice, although my BP and HR still go up when I stand, just not to the same levels. I have no reason to suspect neuropathy, although due to the sudden onset they did test for AChR antibodies. 

Have you identified the specific gene in your family? Clearly there is something heritable with that pattern. Nobody else in my family has it, although my dad and son have minor features of EDS like me. I don't believe EDS is causing mine although I am also being assessed for CSF leak and craniocervical instability. Those would be the only ways in my opinion that EDS could cause such a sudden onset. 

It must be hard knowing you could have passed it on. My one consolation since becoming ill, & something I reassure my children is that it is neither contagious nor heritable so they will not end up like me. I hope that's true. I'm thankful my youth wasn't blighted by knowing what was in store!

B xxx

[Lily]

I have very high norepinepherine during upright tilt.  The doctor suspected hyperandrenergic based on symptoms, and tested catecholamines during a TTT to check.  But he also says I have symptoms consistent with low blood volume, and he suspects I might have EDS.  I think that my situation is similar to what Bombshell said: hPOTS caused by my body trying to deal with low blood volume and veins that are too stretchy. 

[p8d]

I am hyper too, diagnosed via catecholamines after laying 10 minutes-blood draw, standing 10-minutes- blood draw.  Norepinephrine was very high standing.  My neurologist suspected hyper because on tilt my BP dropped at first then went up for a few minutes then went down slowly, it it still does this.  I don’t know what the numbers were during the tilt. I am pretty sure I am hypovolemic because I have a crazy gastropressor response.  That’s one test my autonomic center doesn’t have the equipment for yet.  500mls can raise my BP 20-30 points systolic so I fluid load in the morning then back off later on.  If I am well hydrated, usually later in the day, I don’t get the hyper symptoms unless I am upright too long.  That varies from 5 minutes AM to sometimes 30-40 minutes later on.  My underlying cause is likely autoimmune.  I have UCTD, diagnosed post pots and a positive on the Celltrend tests.  I started SCIG in November and am starting to see some benefits but not on BP/HR.

[bombsh3ll]

22 hours ago, Lily said:

But he also says I have symptoms consistent with low blood volume, and he suspects I might have EDS.  I think that my situation is similar to what Bombshell said: hPOTS caused by my body trying to deal with low blood volume and veins that are too stretchy. 

Lily, what symptoms/tests did your doctor base the low blood volume theory on? Did you have it measured? I truly believe this is a major issue for me. 

Regarding the EDS, I don't really feel stretchy veins could be the culprit in my case as my onset was so sudden, triggered by a forceful valsalva. I think more CSF leak or previously asymptomatic craniocervical instability tipped into becoming symptomatic due to the sudden increase in intracranial pressure. Compression of the lower brain structures could then lead to volume homeostasis disturbance. 

I mean, I've had the same veins all my life, three term pregnancies, no varicose veins, so why would they suddenly all become too stretchy after I tried to blow up a water balloon by accident aged 34?

That's not to say others with EDS may not have an issue with stretchy veins, but surely that would develop insidiously over time?

B xxx 

[bombsh3ll]

21 hours ago, p8d said:

on tilt my BP dropped at first then went up for a few minutes then went down slowly, it it still does this. 

Mine too, although the drop is too brief to capture on a standard BP monitor, it was only seen on the continuous finapres monitor in the first 7 seconds then it overshoots. I believe mine to be the sympathetic nervous system on overdrive trying to compensate for low blood volume. I am looking forward to getting my catecholamine levels, assuming they are accurate. 

21 hours ago, p8d said:

I have a crazy gastropressor response.

I have none! I've tried the famous water bolus thing even though my BP isn't low & concluded it was bunkum, so it is interesting to see someone who actually demonstrates this response!

I also want to do the Celltrend test. I just don't think anyone in the UK would treat a positive result. Also, from what I can gather, pretty much everyone who does it tests positive for something on it which makes me wonder about the clinical validity, although granted these are all dysautonomia patients not healthy people randomly sending off their blood.

If anyone has sent their blood to Celltrend and had a completely NORMAL result, I would really like to hear too. 

B xxx

[Lily]

44 minutes ago, bombsh3ll said:

Lily, what symptoms/tests did your doctor base the low blood volume theory on? Did you have it measured? I truly believe this is a major issue for me. 

My doctor did not do any tests for blood volume.  I complained about how I feel worse after lying down with my feet up compared to right before, and it takes a half hour or so before I feel better.  He said that it sounded like low blood volume and prescribed Florinef.

[p8d]

@bombsh3llevery time my Dr or I do orthostatic vitals we catch the drop and overshoot on “regular” monitors.  It’s interesting that yours is so quick.  We are all so different!  I only had one positive on the Celltrend which seems different from lots of other people.  I am amazed that you have no gastropressor reaction!  I am lucky in that I can generally, if not overstimulated, control my BP with meds coreg and guanfacine (shoutout to @Pistolfor this combination) and fluids.  For now it’s working ok but come summer...  I hope that you have a good appointment next week at UCLH.  Please let us know.

[bombsh3ll]

4 hours ago, Lily said:

My doctor did not do any tests for blood volume.  I complained about how I feel worse after lying down with my feet up compared to right before, and it takes a half hour or so before I feel better.  He said that it sounded like low blood volume and prescribed Florinef.

Well I always feel my best late at night and then like death again in the morning, I believe it is due to overnight volume loss so definitely a similar thing here. I do have the head of my bed raised but haven't found it helpful. Has the florinef helped?

[bombsh3ll]

3 hours ago, p8d said:

@bombsh3llevery time my Dr or I do orthostatic vitals we catch the drop and overshoot on “regular” monitors.  It’s interesting that yours is so quick.  We are all so different!  I only had one positive on the Celltrend which seems different from lots of other people.  I am amazed that you have no gastropressor reaction!  I am lucky in that I can generally, if not overstimulated, control my BP with meds coreg and guanfacine (shoutout to @Pistolfor this combination) and fluids.  For now it’s working ok but come summer...  I hope that you have a good appointment next week at UCLH.  Please let us know.

I will do! My blood pressure is normal now I am no longer on licorice root, and BP lowering meds have never helped me even when it was high (apart from making the numbers look better) so taking those isn't an option for me. 

B xxx

[Pistol]

@p8d - I am so happy that things are looking up for you!!!!!

4 hours ago, bombsh3ll said:

and BP lowering meds have never helped me even when it was high (apart from making the numbers look better) so taking those isn't an option for me. 

I am not sure if I follow - doesn't "making the numbers look better" mean your vital signs are improving? When my numbers went from hypertensive to normotensive it was what we wanted to happen - the numbers went from too high to normal-ish. Is there something I m missing?  

[bombsh3ll]

11 hours ago, Pistol said:

I am not sure if I follow - doesn't "making the numbers look better" mean your vital signs are improving? When my numbers went from hypertensive to normotensive it was what we wanted to happen - the numbers went from too high to normal-ish. Is there something I m missing?  

Hi, what I mean is I am primarily looking for symptomatic improvement ie to not be presyncopal all the time, & to be able to be upright and function, & whilst I'm pleased that my blood pressure is no longer in the range that puts me at risk of stroke etc, having better looking numbers doesn't necessarily translate to feeling any better for me. I believe the underlying problem for me is low blood volume, causing low stroke volume, cardiac output & cerebral blood flow. These cannot be measured by BP or heart rate. 

Blood pressure is the product of cardiac output and vascular resistance, so you could have two people with the same BP who have vastly different cardiac outputs - one is high output with low vascular resistance, the other has low cardiac output and is very peripherally constricted (like me). Lowering the peripheral resistance in the second case doesn't improve the cardiac output if they are volume deplete.

Did you feel better when your BP was lowered? That indicates a different underlying pathology if so - just shows how different we all are!

B xxx

[aelizabeth3300]

I actually had what my doctor called an Autonomic Nervous System test, and it somehow tested all of my autonomic nervous system responses in one test. I was on a finger heart monitor, a blood pressure cuff, and had my bare feet resting on some other device that I couldn't recognize (and didn't ask). They monitored me resting, standing, valsalva, holding my breath, etc. Found that my general nervous system condition was borderline abnormal and that I have hyperandrenergic dysautonomia. I want to get my catecholamines tested out of curiosity because my adrenaline storms seem to get really severe off meds. 

But I've never heard anyone else on here speak of the ANS test and I was wondering if anyone else had been diagnosed that way.

[bombsh3ll]

3 hours ago, aelizabeth3300 said:

I actually had what my doctor called an Autonomic Nervous System test, and it somehow tested all of my autonomic nervous system responses in one test. I was on a finger heart monitor, a blood pressure cuff, and had my bare feet resting on some other device that I couldn't recognize (and didn't ask). They monitored me resting, standing, valsalva, holding my breath, etc.

Most of that was done to me at UCLH, apart from the bare feet thing and the valsalva, which they would have done but my POTS was triggered abruptly by a forceful valsalva so I didn't do that part. I would love to know what info came from your feet! Strange such a high tech lab didn't do your catecholamines though.

What medication do you find helpful?

B xxx

[Pistol]

@aelizabeth3300 - I had all the same testing done at a major autonomic clinic here in the US, within the first year of onset of my worst symptoms. They tols me that my testing came back normal. However - the clinic had me seen by an intern and the neurologist that was an call for the autonomic clinic that day had nothing to say. I was diagnosed based on symptoms. TTT x 2 and neurotransmitter testing by another autonomic specialist in another facility a year later. I was told that autonomic testing is not always correct - it depends on your autonomic tome that day ( I was asymptomatic during testing  ) 

[bombsh3ll]

It is also not uncommon for people to be told tilt table tests are "normal"when they are not. If the person interpreting it is not aware of POTS, they may just see that there is no drop in BP and no deviation from sinus rhythm, and report the test as normal, despite an abnormally high (but regular) heart rate and/or rise in BP/narrowing of pulse pressure/grey, sweating, trembling patient reporting severe presyncope.

This happened to me on my first tilt test - I could see my HR and BP were sky high whilst they were being recorded, but then the cardiologist wrote back saying it was normal. I have heard of the same thing happening to others many times over. 

B xxx

[aelizabeth3300]

@bombsh3ll I want to do my catecholemines. After my dysautonomia diagnosis, they quickly observed another heart condition, and that has been my focus. So far I have only been put on beta blockers as treatment. My main symptoms come from a high HR, so this has been working quite well. Since I'm very small, tiny doses work well with me and I'm able to avoid most side effects. 

@Pistol That's strange that mine came back severely abnormal then because I felt relatively asymptomatic during testing as well.

[bombsh3ll]

I am updating this with the results of my catecholamine tests - surprisingly they were completely normal supine and also rose normally during 10 mins of 60 degree tilt (nearly double). 

The tilted level was about 465pmol/l so nowhere near the 600 that is commonly used to define hyperadrenergic POTS. 

I certainly feel hyperadrenergic - chest pain, non psychogenic anxiety, tremulousness, clammy hands, BP rise etc, but by blood results am not. 

My test results could have been skewed by the fact that I was still taking licorice root at the time, also I had fluid loaded as usual with a litre of ORS on waking, taken 5mg diazepam and was allowed to keep my waist high compression on for the tilt. These were agreed in order for me to have the best chance of lasting 10 minutes on the tilt, as before then is not worth repeating the blood sampling to detect a rise. 

The AChR antibody was also negative which I expected. I have no evidence of neuropathy. 

So I remain without a definitive type, although strongly suspect hypovolaemia. 

B xxx

[Pistol]

@bombsh3ll - I am sorry that you are left empty-handed again!!!! When I had my neurotransmitters checked it was supine x 30 min then walking x 30 min.  Supine was in the upper 600's and walking ( nonsymptomatic ) almost 900. ( I have no idea what it would be when symptomatic!!!! ). I did not stop my meds for the test ( or I would not have been able to walk at all ) and I was very well hydrated before and during the test. I personally am not a big fan of doing testing during a TTT b/c I do not feel it is the same as actually being active and upright. If anything - I believe it is the tilt that may have influenced the result of the catecholamine testing. 

[p8d]

Oh I am sorry @bombsh3ll how frustrating.  When I did my second catecholamine test I was using florinef and wore stockings.  This was not on the day of my TTT.  We have discussed checking again but this time because I am better controlled and physically stronger I was told I would have to stop meds which I refused to do.  Subsequently I had to stop the beta and alpha blockers to switch meds and it was truly awful.  Would there be any benefit from checking without stockings to induce the hypovolemia and norepinephrine dump?  

[bombsh3ll]

44 minutes ago, Pistol said:

I personally am not a big fan of doing testing during a TTT b/c I do not feel it is the same as actually being active and upright.

Thanks, I don't either, it doesn't represent real life physiology.

3 minutes ago, p8d said:

Would there be any benefit from checking without stockings to induce the hypovolemia and norepinephrine dump?  

I wouldn't be able to get the tests again on the NHS, & tbh it wouldn't help me access IV fluids as these are only available privately anyway. 

I was still highly symptomatic despite my licorice, fluids, dpam and compression so the results may not have been that far off what is "normal" for me. 

I am now off the licorice as it was no longer helping my symptoms and was making me hypertensive. Now, my bp is on the low side and rises to normal levels standing, yet I am still presyncopal. 

What I would really like is to have my blood volume properly measured, to prove once and for all I am hypovolaemic, but that isn't available in the UK.

B xxx