Any Hyperpots That Have Gotten Better?

[Joann]

I am on some other sites with Potsies and I asked one person who is very experienced in this area (she is an advocate and involved in many dysautonomia sites), if she was aware of any of hyper pots finding treatment that made them better, or at least able to live their life relatively normal. She said no, she did not know of one hyper pots person like that. That hyper pots patients are not like other pots patients. She did not say it but inferred that we just progressively get worse.

Is this true? Is there any hyper pots out there that still manage to work, drive, live a somewhat normal life? I am in my forties, will my life never get better? I do not want to believe this, but on that one site not one person knew of a person that was doing well.

If you are doing better or know of someone please let me know. Thanks

[HyperPOTS8]

I have very hyperPOTS with supine NE 1400 and upright 3400. I was dx at age 43, found several months ago that it is due to antiphospholipid syndrome (an autoimmune clotting disorder). Most of my symptoms from this disorder improved markedly with heparin injections and plavix (an aspirin like agent), but not the hyperPOTS. In November, I started IVIG and have already had significant improvement in the pots. I just turned 45.

[arizona girl]

Hi,

Before I give you my opinion on this. Would you mind sharing how you were found to be hyper pots? I went through some pretty specific testing and have some pretty specific symptoms that lead to my diagnosis.

Having said that the answer to the question is why or what may be causing those symptoms and labs that define hype pots. In my case, mine is most likely caused by autoimmune small fiber autonomic neuropathy, my neuropathy is most likely a result of a combination of an immune deficiency/cvid and autoimmune disease.

In my case at this time there probably is no cure yet, however there are treatments to manage my underlying causes and with that there have been improvements in my symptoms and labs, but it will require ongoing managment. I am not able yet to feel functional enough to go back to paid job, but I am able to contribute to the world in other ways. Thank goodness for the interenet and dinet, so that there is a way to purpose.

I don't know who told you such a bleak prognosis, it is far to soon in the research and understanding of this subset of pots to come to that conclusion. I also believe that this subset is much more common then currently documented, as my neuro suggested "it's not rare, just rarely diagnosed".

It is important however to rule out potential underlying causes as improvements can happen once those are treated properly. It will take work, but I believe there is hope to have improvement in symptoms with the right treatment plan for you.

[arizona girl]

Hyperpots, I'm on ivig too, in my case for the cvid. What brand are you on and how are you handling your infusion. I'm getting 70 grms once a month. I'm so happy they found your cause. Wow that was some pretty high ne, mine went from 400 to 1000ish. I'm still swinging but not as wide. I also had an mthfr gene defect that we just started treating with methylfolate multi support. I had hashimoto too and have had improvements with that after adding in T3 to synthroid.

I'm so glad for you.

[Joann]

Arizona Girl,

I am still going thru testing, but my symptoms seem to indicate hyper pots. I had several poor man tilt tests and I had a tilt test also.At my worst I had labs done that showed increased noeprhine (sp?). They thought I had a pheo, but that has been about 99% been ruled out. My tilt test was done while on medication and I was told it showed POTS. I just went to the Cleveland Clinic and they want to re-do the tilt test and do several other tests. Dr. Shields said the tilt test showed some irregularities and he would like me to do them off my meds. BUT... I cannot go off them. If I even am late for a dose I begin to feel very bad and my bp goes crazy. He said " you may need to be uncomfortable for the test" but this is not uncomfortable, I can't even get up to get to the doctors, I get near hypertensive crisis without them, so not sure what we will do for the tests.

I want them to let me know if this is low flow, or chemical or ? He has done a bunch of lab tests and nothing seems to be coming back wrong, one kidney doctor there just showed a trace amount of blood in urine, so that probably is nothing. I seem to be disgustingly healthy on paper, but totally not that in real life! 17 months of getting someone to help me figure this out. My bp meds have helped me get out of bed and some days I am able to do SOME normal things. Got hit with the flu last week and the day I started to feel better, my POTS flared worse than it has in months very scary. So I am trying to not do much and recover fully. Prior to the flu, I had been doing ok, avoiding my many things I know I can't do, so this flare reminded me of how incredibly scary and awful the bad episodes are. I keep hoping they won't happen again, but they do.

Hyper Pots8,- I am 45 also and this happened at 44. The woman who told me this had dealt with such health problems for so long, I don't think she realizes how devastating that thought was to me. She started out with what she thought was pots but she now has Not sure pure autonomic failure? I know its a lot worse than just POTS.

I don't know if I can say mine was immediate or over time. The first day it happened four times, then it happened over the weeks and then within 3-4 months it was debilitating. In seven months I was in hypertensive crisis and that was the first time I had anyone start to help me. I have found no doctors in my area that can help and I have searched this site for lists. Dr. Grubb is only an hour or so away but the wait list is a year right now.

[arizona girl]

J, what meds are you currently on for this. I'm right in assuming your bp/hr are normal whilst supine. Interesting that the cleveland doc wants you off the meds when you already had a positive tilt on them. Usually the concern with being on meds is that you will get a normal tilt and miss the problem.

Dr. Grubb is lovely, you could try to get in with his partner Bev earlier. Also, since you are so close I would call them on a regular basis for a cancellation. Mine appointment got moved up 6 months, by doing this. There is one really nice receptionist, that did this and another one who acts like fort knox to get in. So depends on who you get.

Glad they were able to rule out pheo, these hyper symptoms do mirror them, and I also have an adrenal nodule, and was told it wasn't pheo.

I do know this when I was on clonidine, I developed an addictive response to it requiring more and more of it to keep me low. I had to get off of it and that was awful. It needs to been done slowly and another bp med needs to be added in. I'm lucky I got through it, without a serious problem. Dr. grubb prescribed me labetalol, he also did not require I go through another ttt.

[Joann]

Arizonia,

I am on Doxazosin (an alpha) 2mg in a.m. and Metoprolol SuccER, 1/2 tab of 25 mg. a.m. 1/2 in p.m. In the beginning I tried whole tab in p.m., had side effects, then this past fall new doc had me add 1/2 in a.m. One doctor suggested switching doxazosin with clonidine, I haven't yet. I am scared of switching. Dr. Shields wanted me to just try and get off doxazosin and not replace with anything. I can't see getting off the meds helping me. I am extremely sensitive to the metoprolol, if I am late I know it. I wonder if its like the clonidine was for you.

My bp and heart rate are good when suspine, except when I have a bad flare, then the bp and heart rate go down, but are higher than normal. My bp is more concerning than the heart rate. Dr. Shields said he would like to see the tilt test redone without the bps because he said it was a really odd reading and wondered if they were contributing to it. He said it showed heart rhythm irrirregularity but her wasn't sure what they were. When I had the test done, I thought it wasn't going to show anything, because I did not have any bad flares. It showed my bp and heart rate going up but during the 45 minutes it went up and down back and forth.

I am going to call Dr. Grubb again, they wouldn't put me on the wait list until I fax my records and I didn't have them at the time. Now I have too many records!LOL.

So do you think, you are getting better? I don't mean that you are cured, but that with medication you will /are better?

[arizona girl]

Oh, yes, I've had improvements. Hr/bp are better not perfect, pain levels are better, but still have spasms, but the all over burning/sour like pain that I awoke with are gone. My infection rate has improved. I was running around with infections and didn't know it because my immune system is defective and I can't mount a normal fever. I just felt like ****. My bowels, hr and bp with the addition of T3 have improved. I still am working through other symptoms though.

I haven't taken dex or metro. Maybe do a post on those for side effects. Grubb put me on short acting labetalol, an alpha/beta, due to my reaction to clonidine. It works pretty good, I don't even have to take it all the time now. I also wasn't controlled by multiple bp's, before the labetalol. Some like atenenol caused supine brady/hypo. Inderal caused weight gain.

I can see why he wants you off the meds, he is concerned there may be an under lying heart problem and he can't rule it in or out while your on the meds. Heart issues do need to be ruled out as cause first. You should though be in a supervised weaning process, did he advise you on this?

What I think was happening with me and clonidine was that the excess norepi was helping my blood vessels to constrict because they weren't, due to the small fiber neuropathy. So, ineffect the norepi was squeezing my vessels to get the blood to my brain due to pooling. The clondine stopped that effort, and my body to overcome it produced more and more norepi to overcome the clonidine. Hope that makes sense. The body has amazing back up systems.

Yes that is how grubbs office operates, they have to have your records first. Mine were faxed by my cardiologist. Just so you know though they didn't even look through my large stack until I got there. I actually pulled out all the stupid crap the office sent that wasn't even medical, while I was waiting for grubb to come in. If you doc fax's it you will have a confirmation it was recieved and you can call and say that. My doc confirms that you received my med records on "date". I'd like to schedule my appointment now. Also another tool I use for medical records, is I have typed up a complete medical history with all my symptoms and tests, family history, and all the meds I'm on and have taken and side effects. I did it chronologically.

I wish you to a speedy diagnostic processs.

[cmreber]

Hi Joann! After going to the AZ Mayo Clinic and undergoing 5 days worth of tests, the results showed hyper POTS for me. I seem to be one of the lucky ones, however, because I have had good success with Metoprolol (I take 50mg twice a day); being on this and doing some lifestyle changes has seriously helped me get back to my normal life. Of course, I know that I am not the same as I was before I got sick (I was super active: dirt bike riding, street bike racing, snowboarding, kickboxing, surfing, hiking, mountain climbing, you name it, I did it LOL...) but I can get out of the house, drive, go shopping, and I hardly ever have to flake on my friends and family anymore. I even still travel, which is something that is incredibly important to me. I do work from home, which is nice since mornings are still the worst for me, so it's hard to say if I would be able to maintain a "typical" job, but this works for me. I have been fighting pretty hard to get back to my life because I can't imagine not doing the things that I love, but it has been a struggle. I responded, to your post though, because I wanted to give you some hope. At least in my case (and of course, everyone is different), I have seen drastic improvement from when I first got sick. Before, I had to have a chair in the shower and couldn't get out of bed until noon. Heck, I couldn't walk through WalMart to save my life back then, lol. I still get frustrated from time to time when I have a bad day or a few bad days in a row, but when I look at how far I have come in the past 2 years, I can count myself pretty lucky that I have seen as much improvement as I have. I can only hope it gets better from here too!

I really wish the best for you and everyone else on here, and I hope someday we all can look back at our "POTsy selves" and not recognize that person because we are so much closer to being our normal selves again!

[Altruism]

CMReber, I think you just made my day!!! Thank you!! What was the cause of your POTS btw? Viral, pregnancy? What other meds did you use? Thanks for sharing your positive story!

[Joann]

CMReber,

That is some wonderful news. What lifestyle changes did you make? and did you start on that amount of metroprol or did you build up to that amount? Did you follow an exercise program with it?

Are you able to walk up and down stairs, that is one thing I cannot do and really would like to be able to - to access my entire house (I live in a bungalow and haven't been able to get to my laundry room (some may think that is a bonus) my finished basement or my upstairs and storage area. Plus, I never realized how many places involve stairs or serious inclines. Obviously, I am happy that most days I can get up and do things in my house,

Thanks again for some good news. To know there is hope to at least be normal is great. I too, was very active before this and this will sound terrible, but when I see people that so obviously abuse there bodies with food, alcohol, drugs, laziness and they are doing the things I want to do, I get so mad. I mean we know life isn't fair, but I want to shake them and tell them to appreciate what they have!

Thanks again for the hope.

[HyperPOTS8]

Joann, you don't mention what your NE levels were, but mine were vary elevated and as with a pheochromocytoma that causes my volume level to be reset at a much lower level, eg my weight would drop qckly from my usual 104# to 97# and I had severe hypovolemia confirmed at Mayo. Florinef and high Na have been a key part of my management because the low blood volume would just cause even more release of catecholamines, ECG changes, etc. i dont know whether that may be the case for you or not. this disorder is extremely heterogeneous. mine also came on in a stepwise fashion as you describe. I went off the meds that were helping me (florinef, clonidine and salt tabs) to get the formalized testing at Mayo, it made me very sick as I suspected it would, but I did not get back to where I was when I went back on my meds--as I assumed I would. Anyways, now that the underlying cause is being treated with the IVIG, I am doing much better. I don't feel sick everyday. I have been able to return to work, from home. I am on the list to see Dr Grubb because as with when I went to London to see the world's biggest expert on antiphospholipid syndrome, there is no substitute for that kind of clinical experience. Please let me know if I can answer any questions

Arizona, I started IVIG in Nov at the 1mg/kg/month dose. I get Flebogamma which is one of the lowere osmotic agents so less likely to cause thrombotic complications. I get mine in three divided doses every ten days since I have a hypercoagulable disorder and IVIG is hypercoagulable I have to get it slowly. I am going to be gradually increasing the dose (eg 2gm per dose every 1-2 months) to see if I can get an even Better response because my tolerance for stimulating (eg driving on the highway) or stressful things is not normal and I still have major issues with labile BP, etc.

[Joann]

Hyperpots,

I haven't had my NE levels tested sitting/standing, but they were tested in the hospital when they were testing for the pheo and they were elevated at 870. What did you mean when you said you did not go back to where you were when you went back on the meds? That you took a step backwards when you went off the meds for testing and didn't get back to the same point when back on the meds.

So you salt load even with the high blood pressure? That is interesting that volume is low because of NE.

Today I was feeling pretty good and I over did it, so I am trying to not get up too much. I should have known better.

[HyperPOTS8]

Joann, By "set me back" I mean exactly what you said. Before I went off my meds someone on here warned me that could happen and it did. I was already on exactly what they told me to take before I went to Mayo because I knew I was hypovolemic and I knew I was hyperadrenergic (based on more simple testing by my local doctors). I would say it was still worth it to get the complete testing done and done properly and interpreted by autonomic experts--then the diagnosis is very clear. Also, I do not think most people get "set back" for a prolonged period by going off their meds for testing at least I have not read many people mention it.

Yes, I salt load even with high BP and that is what they recommended in my case at Mayo. My BP is very clearly much higher when my volume is down.

[cmreber]

Hi to Altruism and Joann,

The doctors still aren't sure exactly what caused this for me. We think it may have been a virus, though my Mayo Clinic results indicate that I have hyper joint mobility, lots of blood pooling and a neuropathic component. My husband had fractured his back in a snowboarding accident around the same time that I got sick, and I asked Dr. Goodman if there was any way the stress of that might have factored in, but he didn't think so. I still have no idea. When I passed out twice my family made me go to the ER and they found some abnormal heart rhythms, along with the tachycardia and bizarre BP fluctuations, so I had to stay for 3 days. Over the course of that time I had very strange BMs (sorry if TMI!) and a ton of abdominal cramping, so they did think that there was a possibility that I contracted a virus while visiting my husband in the hospital with his back. Who knows? (Kind of wish I did though!)

As far as what I take: I have always been borderline anemic, and was very anemic during that time. Aside from the BB, I take a multi vitamin and also an iron supplement. I also do the salt loading and high volume of liquids, which seems to help me a lot. I know that I am nowhere near the amount of salt they want me to be at (they said 10-20 grams per day!), but what I take in now is a lot for me. Growing up, my grandma (who raised me) had high blood pressure and was on a very strict, LOW sodium diet; therefore, we never used much salt and actually everything we ate was low sodium (nuts, crackers, vegetables, etc). Once I got older, I just never added salt to anything because I was used to not having it. When I got sick and found out I had POTS, my blood sodium levels (and also my potassium) were extremely low for whatever reason. Now, I probably make it anywhere from 5-8 grams daily, which is a lot of salt by my standards! I think maybe my body was starving for it and that's why it helps so much! lol I also never used to drink enough water throughout the day, and I still don't think I drink as much as the doctor would like, but a lot more than I used to, which seems to help a lot. I also have a lot of issues with low BP in the morning, so the doctor suggested I try a cup of coffee (I didn't want to be on any more medication), which as crazy as it sounds, seems to help! (My amazing husband brings it up to me every morning about half an hour before I'm ready to get up...so sweet!)

As far as the Metoprolol, I got put on that after they had sent me home with a Holter monitor and the results came back so abnormal. Then I went to see a cardiac specialist at Stanford and he diagnosed me with POTs and said that BBs are sometimes used to help people with POTS so I stayed on it to see if it would help, which it did. I started out on 25mg twice a day, but I was still having a lot of symptoms and tachycardia, so a couple months in, the dosage was raised to 50mg twice a day and I have been on that ever since. I have never had many issues with medications so I think I may tolerate it extremely well... from what I have read about others on here it seems like I take a higher dose than a lot of people. I still have some issues/ symptoms, especially in the mornings or at the end of my dose when it's time to take the next one, but compared to how I used to be, I think I'm doing pretty great now!

Exercise has been a little up and down for me, though sticking to it, even when it makes me feel crappy, seems to have helped almost as much as the BB. I actually started taking very short walks in the beginning. Then I worked my way up to Yoga, which has by far been the biggest help to me. I have made it to a few kickboxing classes, though much of the time that is a little too much for my poor POTsy body lol. I have also had a handful of successful snowboarding outtings (some better than others), so that was a HUGE turning point for me! I broke my foot in July, which put a little bit of a damper on the exercise, but I have been slowly coming back from that.

Joann, stairs have been my nemesis from the beginning of this, lol, but I am actually to the point now where I can go up them at a normal pace, rather than crawling up them, and I do get a bit of racing heart and out of breath, but nothing like I used to feel. I think part of that is the fact that we moved our bedroom upstairs and I just had to deal with them. I think perhaps my body has (very slowly) gotten a little more used to it over time. It was a bit of a nightmare when I had to negotiate the stairs while on crutches with my broken foot, but I think that after THAT fiasco, just walking up them seems like a piece of cake now LOL!

So sorry for the long post, but hopefully that helps you both and answers some of your questions

[ramakentesh]

The diagnosis of HYPER Pots based on elevated NE levels really only tells us that the patient has POTS with elevated NE levels - it doesnt tell us what the primary etiological mechanism is.

The concept of HYPER pots as is currently suggested as NE levels exceeding 600 as being etiologically significant has been recently challenged via research:

http://www.ncbi.nlm.nih.gov/pubmed/20035362

There is concensus that the likely causes of POTS are numerous and as many as 24 different etiological mechanisms have been proposed. ALl of these are still considered theoretical because nothing definitive has been proven and anyone who tells you otherwise is most likely mistaken.

In the past it has been claimed that Hyper POTS constitutes NET deficiency but only one proband with genetic NET deficiency was found. Then researchers in Australia demonstrated that MIBG dropout and NET expression in veins was decreased in a larger subset of POTS patients via a suggested acquired epigenetic mechanism. And then another study demonstrated that experimental NET deficiency did NOT increase overall NE levels! In other words those with NET deficiency who were thought to be the true hyper patients did not have elevated NE levels and were clearly not 'hyperadrenergic'!

More recent work by Julian Stewart and Vanderbilt has identified a larger subset of patients with kinda hyper POTS. Low blood volume being the main finding and in some elevated angiotensin II levels. Angiotensin II increases sympathetic activity and may act to increase the sensitivity of adrenergic receptors to normal levels of NE (although most of these I think had high NE as well).

What am i trying to say? Well really just that its very unclear what is going on. Some papers talk in unequivocal ways but if you speak personally to any of the major researchers Im sure they would agree that there is LOTS to learn and that our understanding of etiology is in its infancy.

Blair Grubb claims in his experience true hyper patients require medical intervention indefinately but time will tell.

My gut theory is and always has been that many POTS - even those with elevated NE levels may have an autoimmune basis.

[Joann]

Okay I hope this posts only once, My computer is doing funky things and I lost my reply so I am restarting it.

Ramakentesh,

Can I take you to my next doctors appointment? LOL. Seriously, this stuff gets me confused. I get the basics but the rest just zooms over my head. It is as if my mind refuses to learn this, I am not normally like this. I mean, I am a librarian for gosh sakes, I research for a living (not so much now )

CMReber,

Wow! Snowboarding, that gives me some hope for sure. Especially right now, I am going thru a bad time. Its like I am on the edge of a bad flare, feeling bad and having stomach issues, which only happen when this flares. Having tons of pressure below the sternum and on the side of my head. I think its a combo of things, getting over the flu, a huge snowstorm blew in, it is right before my cycle and maybe doing too much with all of this happening. Ugh.. I actually went to work yesterday for the first time in 3 weeks. My boss is able to keep my job for me if I come in 6 hours a week. So I went. I wasn't sure if even the drive would be too much, but I did it and I felt ok the entire time there. But when I got home, my daughter who is 12 needed my help. She watches a neighbor dog and had no friends available to go with her to walk the dog. So I went, not too smart, it was freezing and on the way back home I started to feel bad, and I havent felt good since then.

Little dog tried to bite me too! Walking is my exercise that I have been doing with this, but the last few weeks, i was in Cleveland, then my family was sick, then me, so I guess I am out of practice. Oh and I haven't had my monthly B12 shot, that probably is not helping. I thought about taking a liquid B12, but then I wonder, which is best which may not be good. I question every thing. Same with vitamin d. I need stuff at the grocery store and don't feel good enough to go, and my husband has to leave for a work trip (not great in a snow storm), so we may have to scrounge with what we have.

I am hopeful that in April, I will have some more answers, because right now it is difficult to know what will help, when I don't have any idea what is causing it. I just try to maintain until i find someone to help.

thanks for all of your help and comments. all of you

[kitt]

.

[HyperPOTS8]

Hi Kitt, I take lovenox and plavix longterm for antiphospholipid syndrome (APS) which is an autoimmune hypercoagulable disorder. Prior to this therapy I had severe, constant migraines, blank spells in my thinking (which were different from the brain fog of dysautonomia), bony ischemia which are all well described non thrombotic manifestations of APS. I happen to also have an autonomic disorder due to APS. What has helped me for the hyperadrenergic/autonomic trouble is clonidine and volume repletion with florinef and high salt/fluids. I don't know if you have tried clonidine in the patch form? Oh, and I also did the Levine protocol (and have since done regular aerobic and strength training) which I strongly advocate. Yes, one of the very first symptoms I had was a complete inability to sleep. I had to take ambien 10 mg nightly to sleep. Now I only need to take 5 mg qhs. That works well for me. I am hoping to gradually taper off it. I think getting sleep is extremely important--no one feels well if they have not gotten a good night's sleep.