How Important Is Knowing Your Type Of Pots?

[tigerbomb]

I was recently diagnosed with POTS via TTT. I requested to have my catecholamines checked at the same time, but no one at the testing facility had ever heard of that, so I just had the plain ol' TTT. It showed that when I'm upright, my heart rate oscillates between 100 and 140. My resting heart rate is around 80 (it's always been on the high side - even when I was still running, it never went below 70). My blood pressure stays more-or-less the same (though it's also higher than it used to be). My legs don't turn red or mottled or purple. I feel tremulous and anxious when I'm upright too. So, I suspect that I should be tested for hyperandrogenic POTS.

I have a lot going on medically right now, and I know that the dysautonomia is only part of the puzzle. I have EDS, and something is also wrong with my immune system - might be mast-cell related, might be autoimmune. I'm a medical mystery, haha.

I had an appointment with a cardiac electrophysiologist and POTS specialist last week. It was a bit weird...I'm always really turned off when a doctor will make incorrect statements about EDS, or try to second-guess my diagnosis. This guy did both, which made it hard to trust him from the start.

Anyway, he was talking about the different classifications for POTS, and said that because I have EDS, mine is the secondary form, due to blood pooling in stretchy veins, rather than any type of autonomic neuropathy or hyperandrogenic POTS. I told him that I'm active in the EDS patient community, and I know quite a few people who've had the hyperandrogenic form. He told me that, if I wanted, he would repeat my TTT so we could test catecholamines. He recommended the usual - lots of water and salt, compression stockings, and exercise. He also prescribed Mestinon. He didn't seem to think that it was very important to evaluate me for other causes of POTS.

However, I've read that people with hyperandrogenic POTS shouldn't have a ton of salt, and that Mestinon might be a bad idea for them. So, I'm thinking of having my TTT repeated and some additional autonomic testing done (though probably not with this guy - I really wasn't impressed). Alternately, I could just try it and see how it turns out.

So, I'm wondering...have you found that knowing your type of POTS helps you find more effective treatment? Did your doctors seem to think it was important? I'd appreciate any input!

[jpjd59]

My daughter had the autonomic testing done when she had her tilt table test but that has not helped us find a solution yet to make her feel better. It has been 16 months of trying beta blockers, salt, liquid, and many other medications (all of which she can't tolerate). It now looks like it is due to an immune dysfunction problem. (We will get test results next week).

[E Soskis]

I think it is most important to differentiate autoimmune dysautonomia vs any other type dysautonomia. The treatment for autoimmune dysautonomia (and presumably a few other similar disorders) can range from IV Igg all the way to plasmaphoresis (plasma exchanges). I have been receiving plasmaphoresis now for almost 2 years which has improved my cardiac and vascular dysfunction from the dysautonomia. If I had not persued the autoimmune part and simply stayed with a simple diagnosis of generalized dysautonomia, I would have never received the appropriate treatment - quite possibly would not have lived this long!

[westernmass]

How do you get tested for autoimmune dysautonomia? I have a hunch something autoimmune is at the root of my issues.

[yogini]

If your BP is normal and you HR is high, sometimes treatments that increase BP help anyway. An increased HR can be a compensatory mechanism to maintain BP. if you increase BP another way, your HR could slow down.

I would ask your dr. whether you have hyperPOTS could be a factor in determining your treatment, but i think it is much more complicated and individual to each patient. Have you looked into beta blockers?

[Kellysavedbygrace]

Getting to the root cause of your Dysautonomia is critical for identifying treatments that are helpful. Simply bc you have EDS makes me suspect Mast Cell Involvement but I'd ask your doc. Although it is a more rare presentation there are several participants active on this forum that have the triad: Dysautonomia, EDS and Mast Cell Activation Syndrome. I also agree w E that ruling out autoimmune Dysautonomia is important.

I was Dx w POTS and NCS last year w no cause and spent mostof that time at home or in bed. Recently I was Dx w MCAS and now on high dose antihistamines which have really helped. I'm able to be upright more but very tachy still w many symptoms. Also I have the hyperadrenergic type (w mostly low BPS) and salt is helpful for me daily but especcially when I'm symptomatic.

[Chaos]

I have EDS as well,and have been diagnosed with the neuropathic form of POTS but also have a hyperadrenergic component. I also am suspected to have an MCAS component and am being treated for that as well. I think it's helpful to know the type because in my case for example, the doc warned me, "it's going to be hard to treat you because what you do to treat one type will usually aggravate the other type, so it's likely going to be a long slow process to figure out the right combo that works for you." Just knowing that much helped me settle in with a longer term perspective and know that there wasn't going to necessarily be a quick fix with meds that was going to let me get back to life like I wanted it to be, as fast as I wanted it to be.

[ramakentesh]

Every doctor has different opinions and treatment options are currently the same so probably not.

[Jacquie802]

I was never told what type I have. I got sick after having a viral infection..

[peregrine]

Like Rama, how much it helps can be hard to tell since every specialist has a different opinion and approach.

For me, it was useful to look at my symptoms and look for patterns - for example, I don't pool visibly unless I've been in a hot shower, but I do sweat a lot and startle easily. During my TTT, I did pool visibly. My blood pressure is more often high than low. I get lightheaded and spacey, and overstimulated, but have never actually fainted due to POTS. If you look at my labs (e.g. the autoimmune ones), they are negative, and there was no sudden start or linked illness or accident. My standing/lying norepi has never been tested. Based on 24-hour urine sodium, my blood volume (this is a proxy and not an absolute like you would get with a radiological test) is pretty normal, and I retain salt just fine. I have joint hypermobility syndrome, which is (depending on who you ask) a form of EDS. I don't get any of the MCAS symptoms. My QSART was positive, so I probably have some mild neuropathy, and my toes are numb.

The end result of thinking about things that way was that it helped guide treatment. For example, because of the limited pooling and okay blood volume and salt retention and normal to high blood pressure, we have never tried midodrine or Florinef, but I do wear compression stockings and take salt because those are generally helpful as volume expanders (my neurologist described it that way to the medical student he had with him one day, who was trying to figure out why I was on betas and clonidine but also eating salt!). Because there doesn't seem to be any autoimmune component to my POTS, we haven't thought much about IVIG or similar treatments. Betas have been helpful. After a while of badgering my neurologist, he agreed to try clonidine, which has made many of the sympathetic-type symptoms (sweating, startling, being overwhelmed, resulting fatigue) much better, so my SNS was probably a bit overactive. The combination of joint hypermobility syndrome and numb toes also suggests some more minor role of pooling, so I probably have some combination of secondary (JHS/neuropathy) and primary (hyper-ish) POTS. Pyridostigmine hasn't helped, so my parasympathetic nervous system is probably okay.

So I guess I would say - types of POTS can be a useful way to think about it, but I find it's more helpful to look at your symptoms and see where they fit in terms of what parts of the body are affected, what treatments they respond to, etc. I find that figuring out what treatments I respond to is more helpful than looking at test results, mostly because in the end it's all about what works! Being a bit too rigid about classification can also keep you from trying something that might work, but isn't typical for a given type. *shrug*

[yogini]

I would like to add that I never found a cause for my POTS and I have been able to find treatments. Someday when doctors know a lot more about POTS, I think the cause information will be a lot more helpful to our recovery. Many people here search really hard and can't find a cause for their POTS. I personally found it a lot more productive to spend my time trying treatments rather than focusing on the cause, since there are lots of drugs that do work for POTS and it takes a while to find the right combo.

[ramakentesh]

I dont think hyperadrenergic POTS as a clinical entity even exists any more. Most docs now talk about neuropathic POTS (Small fiber neuropathy or AAG) verses non neuropathic POTS (Low flow, parasympathylosis, cerebral autoregulation or NET deficiency).

And even then treatment is usually the same unless you go to Mayo or Cleveland clinic and get IVIG.

[MomtoGiuliana]

In my case I never found out what type of POTS I have. My specialist discouraged it. He said it would be a waste of $ and energy and I would be no closer to feeling better. But I have improved greatly. If I had not improved I might have considered more testing. But as others have said, even with more information, it can still be trial and error to find the best treatment for you, regardless of how your POTS is classified.

[ramakentesh]

the etiologies are still being investigated and argued over.