For people diagnosed with Postural Orthostatic Tachycardia Syndrome (POTS) and Orthostatic Intolerance (OI), follow me as I document my progress towards recovery while on the research treatment program by Dr. B. Levine of Texas Click here for more...

Thursday, May 20, 2010

What is HyperAdrenergic POTS?

For those of you who are new to POTS and OI, did you know there are several flavours of POTS?  Here is one:  HyperAdrenergic Postural Orthostatc Tachycardia

Onset:
Symptoms appear gradually and progressively over time. Thought to be a genetic disorder.

Symptoms:
  • increase in urine output after standing (what a nuisance!)
  • presyncope
  • fatigue
  • migraine headaches
  • palpatations
  • tremor
  • anxiety
  • cold and hands and feet

Clinical Findings:
  • orthostatic tachycardia
  • orthostatic hypertension
  • exaggerated response to isoproterenol infusions
  • significantly elevated serum catecholamine levels with serum norepinephrine levels >600 ng/mL (this was found in 29% of patients in a study)
Treatment:

In the hyperadrenergic form of POTS, patients often respond best to agents that block norepinephrine or its effects. One agent that is particularly helpful is clonidine HCI in either pill or patch form. We start the oral form at 0.1 mg PO 1 to 2 times a day and filtrate upward. The patch form of clonidine is quite useful because it provides a constant and continuous amount of the drug for up to 1 week at a time. The combined and β blocking drugs labetalol and carvedilol are quite useful in some patients as pure β-blockers may exacerbate symptoms (because of unopposed receptor stimulation). Methyldopa has been reported to be useful in some patients, as has phenobarbital. In addition, both the SSRIs and norepinephrine reuptake inhibitors are useful in select patients.
People who have been diagnosed with this disorder typically have other fmaily members who suffer from it too. I picture long line-ups for the toilets at family reunions.

Here is an interesting article on recent research on what genes may impact POTS...

37 comments:

  1. I start my trial of clonidine tomorrow. I was diagnosed with hypovolemic/hyperadrenergic POTS and started conservatively on salt and an exercise regimen. We hoped the hyperadrenergic state was from the hypovolemia. I then progressed to Florinef and DDAVP and now am moving on to clonidine. Of note, I was in great physical shape when I became ill, and I have worked back into great shape again over the past 5 months. I may have a sub type that can not be cured with exercise, which is hard to face. No one in my family has POTS. Yes, the peeing is awful. The DDAVP has curbed this. Prior to Florinef and DDAVP, I was peeing 6 liters/ day. I have constant headaches and lots of POTS related anxiety, so this subtype is not fun. I hope the clonidine helps. If you have this sub type, beware of midodrine.

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    1. PLEASE READ & REPLY!!!
      I have hypovolemic and hyperadrenergic POTS and I take Midodrine 3 x daily, but I still feel horrible all the time. You said "If you have this sub type, beware of midodrine."
      WHY???

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  2. Leah - I think you bring up an important point. POTS/OI may not be cured through an exercise regime but I believe exercise is a very important element to help manage symptoms of any variety of POTS or OI.

    I would like to hear what you think but it is my impression that could be more research done on what medicines are most effective for the Hyperadrenergic POTS.

    Have you tried B2 and/or magnesium for the headaches?

    Who made the diagnosis if I can ask?

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  3. I absolutely agree that no matter what subtype of POTS you have an exercise regimen is key. The worst state a POTS patient can get themselves into is a deconditioned state. A deconditioned state guarantees a spiral downhill. I was put on bed rest for a spell due to misdiagnosis, and I got worse.

    My diagnosis of POTS was FINALLY made at Duke Medical Center. I came back home to Texas and saw Dr. Amer Suleman (a POTS cardiologist/ electrophysiologist). He is also in Dallas. I tried Dr. Levine, but I was very ill at the time, and Dr. Levine had a 4 month waiting period. I had a battery of autonomic testing and received infusions of albumin and saline in the hospital due to being severely dehydrated. I was then referred to a top notch endocrinologist in Houston who ran renin, aldosterone testing, and a water deprivation test. My renin and aldosterone were severely low, and I have diabetes insipidus (DI). This is not the typical DI, but a partial diabetes insipidus like syndrome that occurs in some POTS patients. My POTS was moderate to severe, and the Florinef and DDAVP have by no means cured me, but I no longer feel like I am on death's door on most days. I was able to resume an exercise regimen which has helped, but again, I am still extremely debilitated with being unable to sit up without head support or stand for more than 10 minutes. The odd thing is my POTS started after a swimming work out in May 2008. For a few months, my POTS symptoms only occurred after my swimming routine. I find this odd because swimming is a recommended POTS friendly activity. This is way out in left field, but I wonder if the lack of gravity forces in the pool and my supine position triggered a bizarre chain of events.

    I do take B2 and magnesium.

    I think more research is desperately needed on POTS in general. As a pharmacist, I can look at the drug regimens various patients are on, and that right there tells me the causes are heterogenous. Some of the drugs actually have quite the opposite effects in the body. If the root problem of a patient's POTS is not discovered, it is all just guess work. It's like throwing noodles at a wall and seeing what will stick. I don't think the researchers have a clear understanding of what causes POTS in the majority of patients.

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    1. i know this is an old post but i have POTS and need to find a doctor so bad, who is the endo dr you found in houston?? Please HELP THANKS!!

      chadnataly@yahoo.com

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    2. Leah, I live in Houston and have been told I have POTS. I have not been given any meds yet. I am looking for a doctor in Houston that knows something about this. Also, what Endocrinologist did you see?

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  4. Well, the clonidine was a bit of a bust. The past few days I have been warming to Dr. Levine's theory. It dawned on me that my echocardiograms back in December showed a relatively small heart, so this got me really thinking. Maybe I need to do a lot more exercise than the average person and a different kind of exercise to strengthen the heart. The bed was put on blocks and the rowing begins tomorrow. I'll report back in a few months.

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  5. Well good luck!! Don't forget the heart rate monitor. Super important if you want to make real progress. Please keep me (us) posted on your progress.

    I felt worse before i got better BTW but don't be discouraged!

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  6. Leah - I also live in Houston and now need to see an endocrinologist. Anyway I could get the name of you doctor? Would love someone that is familiar with dysautonomia. I have been seeing EP's in Houston with no luck. Now in the process of making an appointment with Dr. Suleman. Can't believe in this big city with such a huge medical center it is so hard to find doctors to help us POTS people.

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  7. There isnt current medical agreement on the primary etiology or sub-group breakup of POTS, so therefore it is hard to say whether there is even a sub-catagory where the primary problem is sympathetic excess rather than compensatory tachycardia.
    Mayo recently released a publication that suggested that excessive norepinephrine levels in some patients are merely an exaccerbating feature. In their experience (you can read it 'POTS; the Mayo Clinic experience) all forms of POTS are the result of an autoimmune mediated attack on a3 acetylcholine receptors.
    J.Stewart and Marvin medow break up POTS based on peripheral blood flow and autonomic versus compensatory tachycardia. They indicate that there may be a number of different subgroups: those with increased angiotensin II levels, those with parasympathetic withdrawal and faulty cerebral vascular autoregulation, those with NET deficiency (postural hypertension) and other catagories.
    I do believe that exercise helps most forms of POTS - but not all. There are patients who are so disabled from POTS that they experience syncope within 10 seconds of standing; who experience blood pressure above 200/120 or heart rates of 190 within 30 seconds of exercise of any kind.
    In my own experience exercise has helped quite significantly, but its by no means a panacea and it doesnt 'cure' the condition.
    And in some forms of POTS - such as those presumed to be caused by mitochondrial disfunction - it may actually worsen the condition.

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  8. The sub-groups which are emerging seem to relate to how physicians approach treatment rather than the cause of the POTS since no-one knows the cause as yet.

    That's a very interesting study- POTS:The Mayo Experience where they conclude:POTS is a relatively common condition. At least half of patients with POTS have neuropathic features, 1 in 3 has hyperadrenergic features, and 1 in 7 has serological evidence of an autoimmune pathogenesis. Hyperadrenergic and hypovolemic correlates are likely compensatory or exacerbating.

    Exercise is NOT a cure. However, it can significantly improve symptoms if managed correctly. As someone who has been severely ill with the condition and who also has been physically fit, I can say that a managed exercise program as Dr. Levine's protocol is, has helped me tremendously.

    I have not read anywhere that exercise can worsen the condition is POTS "caused by mitochondrial disfunction" and would love to get a copy of the research paper or study you refer to.

    I believe that the decision made as to whatever treatment one adopts should be EVIDENCE BASED. There is quite a bit of evidence now supporting managed exercise as not only improving the condition in patients with POTS, OI but also in patients with CFS and Fibromyalgia.

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    1. HI, I am trying to figure out how one gets in contact with you. I have just been diagnosed with POTS after having my 4th baby 16 weeks ago. Its been horrible. I was misdiagnosed as still having a CSF leak about 6-7 weeks in and went through lots of blood batches which created compounded issues and more bedrest! Not good for a POTSie. I feel a bit like I am drinking from a firehouse. I would really like to find a doctor in the Houston area, and don't know where to start. I asked my GP to get me into a PT regimine to help get me moving. Can you recommend someone in this area? ghillegeist@att.net

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    2. ......but also in patients with CFS and Fibromyalgia.

      I really can't speak for POTS but try going on an ME/CFS forum and saying that you will literally gets thousands of negative responses. There are NO credible research papers to support this only a lot of wasted research money. Speak for POTS by all means but not for CFS/ME

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  9. Lisa,

    Dr. Cristina Boccalandro (713) 800-6212. She is awesome! Dr. Suleman is great too, but he is a busy man, so you may have to keep calling if you have a follow up question. Both of these doctors are kind, compassionate, and very positive. They were refreshing to find.

    POTSRecovery,

    I am LOVING the rowing and weight training! I absolutely believe Dr. Levine is on the right track based on the results of my ECHO. I can't believe I am back on my feet when a year ago I hardly sit up. Exciting. Dr. Suleman adopts many of Dr. Levine's methods, and I believe Vanderbilt will as well based on the upcoming study.

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  10. Have any of you considered the diagnosis of Lyme disease. I have it and POTS is just one of my symptoms now that I have begun treatment. Many on the Lyme boards have POTS. If you go to http://lymenet.org and enter the Medical questions on the flash discussion, you can do a search of POTS there. Many Many of us with Lyme have POTS. I had the POTS diagnosis first, then Dysautonomia, then Lyme.

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    1. Yes I am also interested in the Lyme/POTS association. I only started getting the POTS symptoms AFTER being treated for Lyme. So now I don't know if I still have Lyme and to continue treatment, or if the POTS is the residual damage caused by Lyme and/or treatment. Have you had luck with POTS after your Lyme treatment?

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  11. Leah, who did you see at Duke? I am interested in finding a specialist in the Raleigh/Durham area if possible.

    I LOVE this blog! Thank you so much for this GREAT information! :)

    -Lauren

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  12. Hi, I was recently diganosed at mayo with hyper pots. I have symptoms for 13 years with 4 "relapses"" that left me bedridden at different times. Pregancies were not fun either! sure confused my dr! Right now I have been almost 100% bedridden (with the exception of going to the dr's office) for 6 months. I was happy to finally get a firm diganosis but I'm feeling bad about the prognosis. I know I am at a severe point, and 6 months in bed has been so hard, but the dr said best case it would be at least 1 year before I could realistically do "normal activites" like driving to the grocery store or to the kids school and didn't want to venture to guess when I would be able to travel , go to disneyland etc. I have 2 kids and I feel like I am missing out on so much already. It is also hard to explain to friends/neighbors why I look okay lying on the couch but I can't go out to eat with them or even talk to them standing up.

    One of my issues is I was born with a kidney problem which limits treatment options. I have been on a low sodium diet for over 10 years. I also have Factor V and wonder if anyone else had hyper pots and factor V? I also have developed flat feet(and my toes can bend really far suddenly?) during this 6 month of being bedridden and the mayo docs had no really explaination and the podatrist said that it is not due to an injury and is adult onset and believes it is connected neurlogically?

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  13. Hi, this is a response for the woman with the flat feet and bendy toes. I have also just recently been diagnosed with POTS and I guess it's the hyper kind because my norepinephine level was high while standing. I've also had POTS symptoms off and on for about 15 years or so. However, back in my teens (when I first got it) it was always manageable and it's kind of flowed and ebbed over the years. But, it's never had me bedridden or anything like that.
    One thing that I have learned is that there is a connection between POTS and hypermobility. I have always been somewhat "double jointed" and I tend to stand with my knees bent backwards and stuff like that. I also have flat feet (with bunions). Well, around the time I was diagnosed with POTS I was also diagnosed with Ehlers-Danlos Syndrome Type III which is the least severe of the hypermobility syndromes I believe. The point is that there is a connection between being extra bendy and POTS. They don't quite understand it but it may have something to do with your vessels being too loose I believe. When a person is hypermobile it basically means that their collagen is faulty. It is a genetic disorder so there's not much you can do about it except to try to eat the best diet you can and to try to form as much muscle as you can around your most troublesome joints. So, just a thought for you to consider. You may want to consider seeing a rheumatologist to get checked out for the hypermobility.
    I totally understand about having to explain this to people who think you look fine. It is VERY frustrating! I've started telling people that it's basically like I'm allergic to standing. :) Good luck!

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  14. HI.....really interesting to read all this information.

    I am wondering about the POTS along with the mitochondrial dysfunction, as this is what happens with ME/CFS. Has anyone here been diagnosed with ME/CFS? POTS and other forms of OI are common symptoms of the illness, which is poorly understood, but mitochondrial dysfunction is clearly a part of the illness. We experience post-exertional malaise, so exercise is very tricky - too much can send us to bed for days, or weeks.

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  15. I've started experiencing drastic spikes in blood pressure after working out in a hot gym last summer. Spikes in BP occurred immediately after standing and again when I sat back down, in response to changes in temperature, when chewing food, talking, in response to dreams at night, and emotional stress. The spikes were very high but they were temporary and my bp would come back down to a more normal level quicky after the initial spike. Has anyone with Hyper POTS experienced these type of symptoms? I was put on Clonidine but it lowered my nighttime bp too much. I then started taking Methyldopa 3 times a day. I am still having bp spikes at night in response to me dreaming (which I don't understand ). It seems that my body is over responding to stress stimuli by releasing to many hormones. Is this Hyper POTS? I live in Houston, TX and I can't find anyone of any help. I waited almost a year to see Dr Levine in Dallas but he didnt do any
    autonomic tests (just drew some blood and took my bp while exercising, etc.). He didn't give me a diagnosis. Who diagnosis Hyper POTS? Please contact me. Sewhite72@yahoo.com

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    1. Greetings--yes, b/p spikes and tachy during/after bad dreams! Have you had any more luck with treatments and discovering what you have?? I'm new to this but have recently been diagnosed with HyperAd.POTS by Emilia Arden in Portland who studied under Dr.Grubb. Pls let me know your treatment plan/symptoms as hypERtensive POTS is not always understood (like ever) unless you see a specialist. Hope you're doing great; look forward to hearing back: Tammi at MmeMozart@aol.com

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  16. Anyone with POTS has it because they have Lyme disease. You can argue me to your blue in the face, but this is fact. Lyme testing sucks, so don't rely on it. Try some Herbs to treat Lyme like Sida Acuta, Cats Claw, Japanese Knotwood, and Artemisinin. When you feel like your dying from the Toxins you've killed, you will then know it is Lyme. Best to all of you. Get rid of the Lyme and Co-Infections and you get rid of your Pots. The reason many of you get POTS after a tragic event is because the immune system is lowered and it lets the Lyme out. It's that simple. Do your on DD and you will see I am correct. Check out Lymenet.org and search "POTS" in their Forum. You will see a vast majority of the Lyme patients also have POTS.

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  17. Does anyone know if there is a correlation between a positive MIBG scan and Hyper Andrenergic POTS? Is adrenal hyperplasia found in patients with this form of POTS?
    PLEASE HELP!
    sewhite72@yahoo.com

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  18. Hello. Im not saying every single person that has pots has lyme, like someone else, but i can say that i had pots for 5 years and was even able to manage it with exercise and no meds until a year ago when i suddenly had a whole host of other symptoms. As it turned out I had lyme the whole time. My POTs specialist said "Geez, Im going to start testing all of my patients, seems like my POTs patients that have lyme are coming out of the woodwork." Now that I have had undiagnosed lyme for 5 years its much harder to treat. Moral of the story: if you have POTs please get tested for lyme. Or do a trial of antibiotics and see if you notice a difference in you symptoms. Before you may progress to late nuero lyme like me. Good luck fellow POTsies!

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    1. Who is your doc in houston?

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  19. PS. Thats my comment above. Just wanted to add that its hyper POTs that I got from lyme as diagnosed by a tilt table. Even though its genetic, Lyme can turn it on. Yuck!

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    1. Thats my reply to your reply? What kind of tilt table did you have, and where?

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  20. There are a lot of medications that may help POTS but don't use all of them for the sake of using, because people with POTS also have sensitivity to chemicals and medications. This is why I keep my medication use to the bare essentials. I only take betablocker everyday to control the rapid heart rates from standing up and movement, although I have tried other medicatins that can potentially help I also experienced side effects from them so I believe its better to just stick to the medications that help the most or ones that you feel you cant survive or function without

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  21. Ah I wondered what hyperadrenergic pots was. I got pots within a day after getting the flu shot so mine was a result of a vaccine injury. It was quick and definitely noticeable so that rules out hyperadrenergic. What I wonder about is beta blockers, I was put on one after I developed heart failure from the tachycardia (since doctors refused to help me since I was vaccine damaged). But I read that beta blockers can actually make pots worse because after all they are blood vessel dilators. I'm wondering if I should slowly get off the beta blocker and try fluorinef or licorice root instead. And no, not everyone with POTS has lyme, many have nerve damage, mercury poisoning, or metabolic damage, or adrenal damage as a result of any of these things or other factors.

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  22. I was diagnosed with pots this past april after donating blood and going in and out of the hospital. I had a tilt table test that showed my heart rate went up to 140's while standing and my BP went slightly up. I believe I have hyperadrenergic pots. does anyone have gastrointestinal issues and lost weight? Sent my stuff to the Mayo clinic and they told me I don't have Pots since I didn't pass out and my BP went up.

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  23. To the last commenter.

    One dont need to pass out with BP going up to get a hyperadrenergic POTS diagnoses.

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  24. Leah, Why beware of Midodrine? I was just kiagnosed in February I was put on Fludrocortisone first which worked for about 3 weeks, then stopped working. Since then I have been put on Midodrine. I am noticing temporary pain in rendom parts of my body (sometimes a spot on my leg, or arm, sometimes in my neck etc.) however, my two most prevelant symptoms are heart palpitations and frequent/urgent urination (wondering about DDAvP for this) but why to you say beware of Midodrint?

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  25. I have hyper POTS and have been nearly bedridden for several years. It has just been discovered that my POTS is caused by a floating kidney and will be treated by securing the kidney in its proper place.

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  26. can you explain the exercise routein you are talking about - I am working with someone who has H-POTS and is very overweight. Some form of movement would be very helpful - I am a health coach. thanks Lorraine

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  27. People, read Edward Shorter's From Fatigue to Paralysis. Then reconsider your diagnosis.

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  28. I was diagnosed with POTs nearly 10 years and more recently after seeing a Stanford doctor the hyperadrenergic type. Question to all you sufferers out there...anyone ever have your toes get stuck straight in the air? This has happened more than once and no doctor I have seen has ever seen it or knows why. I would appreciate any insight.

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