My Hyperadrenergic POTS Story

My POTS kicked in August of 2016.  At the time, I was trying to be a bit healthier that I had been by cutting out soda and eating better.  I think that the first symptom was that I had to pee a lot more often.  I remember that my wife kept making fun of my tiny bladder during that phase.  Then the light headedness and tachycardia started slowly increasing over the next couple of weeks.  In September it really hit me hard.  I was in my office at work and nearly passed out, which was followed shortly after by a super intense adrenaline storm.  I went to the ER complaining of chest pressure and dizziness, but I was feeling a bit better by the time I got there.  My blood pressure was high and I did in fact have tachycardia.  They also took an ekg and some basic labs that came back normal and he told me that he thought that it could possibly be either SVT, AFib, or just acid reflux.  He told also told me that I should set up an appointment with a cardiologist.  

The next day I called and set up appointments with a general practitioner and a cardiologist, and literally the way I chose them was that I called the offices that were nearest to my house and asked for the doctors with the next available appointments.  I got in to see the general practitioner the following day, and it turns out that he was one of the most experienced POTS doctors in Utah and actually dedicates 1 day per week to treating POTS patients.  I still can't believe how lucky I got by finding him the way that I did.  He said that he wanted to see the outcome of the cardiologist appointment before he did anything.  After visiting him, he said that he wanted to see the results of the cardiologist before he did anything else.

During the next week before my cardiologist appointment, I started taking my blood pressure regularly and I noticed that I would get some crazy swings.  One day when I was worried about a problem at work that I couldn't get there to address, I took my blood pressure and it came in at 180/125.  This was before I knew much about blood pressure and I didn't realize that my blood pressure was high enough that I should have called 911.  I also tried going back to work in this week, but I only lasted one day back.  My co-workers were all extremely concerned about how pale I was, and the tachyardia and light headedness was more than I could handle.

Based on my description of what happened in my office the previous week, the cardiologist assumed that I had SVT, so he put me on a calcium channel blocker, a 2 week holter monitor at the same time, and scheduled me for a echocardiogram.  My blood pressure was still crazy during those 2 weeks, but the calcium channel blocker helped keep my blood pressure from crisis levels.  The results of the holter and echo didn't show anything useful other than bouts of tachycardia, fairly frequent skipped heart beats, and that my heart was showing signs high blood pressure.  I asked the cardiologist about the rest of the symptoms and he said that it just sounded to him like anxiety and that I should take some anti-anxiety meds for it. 

I then went back to the GP the next week during one of my adrenaline storms and he took my pulse while lying down and again standing up.  He didn't say anything to me at this point other than he wanted to run a ton of labs.  The most interesting test was the lying down vs standing catecholamines blood draw which measured my norepinephrine at about 250 while lying down and about 850 while standing up.  I also had low testosterone.  After getting the results a week later, had me come back into his office where he diagnosed me with hyperadrenergic pots.  He told me to start the mediterranean diet, to stop taking the calcium channel blocker, to start taking .1mg of clonidine, and to start regularly exercising.  Unfortunately, he didn't mention anything about salt, and he printed out some documentation on the mediterranean diet that included going salt free.  About a week later, all of my symptoms were much worse.  I ended up in the emergency room again after constantly being on the verge of passing out and blood pressure that kept repeatedly spiking to crisis levels.  I also was having some strange tingling in my feet, legs, hands, arms, and face.  The ER did an ekg, an MRI, and a bunch of lab work but again didn't find anything. 

The next day I went back to my GP where he then told me that he messed up the print out and that to go up to 8Gs of salt supplements per day, about half of which coming from sports drinks and the rest coming from sodium chloride tablets.  He also put me on 100mg of Losartan, .2 mg Clonidine patch, .2mg of Clonidine pills, and zoloft.  This was obviously a huge jump up from where I was at and a lot of new meds to take on at a single time.  Things got really confusing at this point.  My brain fog went away and light headedness went away, but my blood pressure got extremely high constantly - like 150-160 / 100-110, and any activity or anxiety would put me into crisis mode.  That dosage of Clonidine gave me bradycardia and chest pains too.  My sleeping heart rate at this point came all the way down to about 40.

I went to a second cardiologist at this point who had me do a stress test.  The first round that I did with him showed what he said were some anomalies so he told me to start taking a daily aspirin and that he wanted to do a second stress test combined with an echocardiogram.  I cut back significantly on the salt after the stress test since the cardiologist indicated that I might have angina, so by the second stress test everything came back normal.  My blood pressure started coming back down to reasonable levels as well.

I went back to my GP, explained everything that had happened, and he basically told me at that point that I needed to play around with the Clonidine and salt dosages until I found what worked for me.  He also sent me to do a renal ultrasound to check for any abnormalities with my adrenal glands.  That ultrasound didn't turn up anything and they tried to get me to get a renal cat scan, but my insurance denied it so I never had it done.

I was about 2 months into this situation by now and I started playing around with the dosages, but things really clicked for me when I came across Julian Stewart's research.  Things started to make sense particularly when I realized that his classification of low flow POTS included pallor.  After realizing that my hyperadrenergic pots was caused by an ACE2 deficiency, I started reading everything that I could find on the ACE2, the RAAS, the effects of Angiotensin II, and norepinephrine.  Understanding how all of these work helped me realize exactly what was happening inside my body.

About a month later, I was taking .2mg of Clonidine, 100mg of Losartan, and 3-4 grams of salt, which is when I was finally healthy enough to go back to work, even though I still wasn't in a very good state.  I was fired a few weeks later and when I got home that day, I took my blood pressure and it was 180/120.  I popped an additional .2mg of Clondine, and my blood pressure came back to reasonable levels within 30 minutes.

I continued to play around with the dosages for the next few months until I settled on 1-2 grams of salt all coming from sports drinks, 100mg of Losartan, 25mg of zoloft, testosterone supplements, and .1mg of Clonidine per night, and I also would take an additional Clonidine if I felt myself surging.  

What I really discovered though through trial and error is that I have to keep my blood volume high by consuming more salt and fluids than my kidneys process.  Losartan is a must, and Clonidine is amazing for suppressing the adrenaline storms.

It has now been nearly 2.5 years since this started for me and this condition barely affects me now as long as I stay consistent with my treatment.  I feel extremely fortunate that I was able to find a good doctor as quickly as I did that helped me understand it and learn how to treat as I did as I know that most people that become affected by this condition aren't as lucky as I have been.

Treatment Advice for Hyperadrenergic POTS from an ACE2 Deficiency / RAAS Dysfunction

Let me first start with my disclaimer that I am not a doctor and I have no medical training.  All advice I give is in the context of what I have read, what I learned from my doctor, and my own personal experiences.

It has now been about 2.5 years since I first became debilitated by this condition and I am pleased to say that after 3 months of debilitating hell, followed by another 6 months of treatment refining, that I have been very stable ever since.   Because this is such a tricky condition to get diagnosed and to treat, I am posting my advice in the hopes that it may help someone some day that is looking for answers.

First, it is important to understand the terminology:

POTS - a symptom of many different possible underlying conditions that results in a sustained elevated heart rate while standing.

Hyperadrenergic POTS - a sybtype of POTS that results in high adrenaline / norepinephrine surges that can potentially send blood pressure to very high levels.

Low Flow POTS - the name that Julian Stewart (the leading expert on this type of POTS) uses instead of Hyperadrenergic POTS, but is also more specifically focused on low blood volume / hypovolemia than the common descriptions of hyperadrenergic POTS.

RAAS disfunction - the RAAS is the complicated system of the body that is responsible for the regulation blood volume.

ACE2 deficiency - a rare, specific type of RAAS dysfunction and causes a high amount of Angiotensin II.

Before visiting a doctor, make sure that know your own symptoms and that they compare with the combined symptoms of POTS, Pheochromocytomas (since the hyperadrenergic pots adrenaline storms present in a similar manner), and frequent urination.  It would be worth doing the poor man's tilt table test before visiting your doctor.  Buy a pulse oximeter and heart rate monitor, lie down for 10 minutes, take your heart rate and blood pressure, then stand up and measure your heart rate after 1 minute, 5 minutes, and 10 minutes.  If your heart rate stays elevated by more than 30 beats per minute or if your heart rate is higher than 120, then you probably have POTS.  If your blood pressure is significantly elevated as well, then you may have hyperadrenergic POTS.  Keep in mind though that you may get somewhat different results throughout the day depending upon your recent food and fluid intake as well as your anxiety levels.  

There are very few doctors that can diagnose and have experience treating this condition.  Finding a doctor that can help will be frustrating and difficult.  There is a list of doctors on dinet.org's website that are experienced treating POTS, but also understand that there are some really good doctors that are more accessible than the experts that will appreciate the challenge of helping you figure it out.  Look for doctors that will listen to you when you show them your research, are receptive to your treatment option suggestions, that may do research on their own between appointments, and won't be in a rush to push you out the door so that they can move on to their next patient.  You may need to try out 10-20 different doctors until you find one that can genuinely help.

Second, ask your doctor to perform a tilt table test and a catecholamines blood test that compares your norepinephrine after 10 minutes of laying down vs after 10 minutes of standing up.  The tilt table test can be used to diagnose POTS and hyperadrenergic pots in some cases, and the catecholamines blood test can be used to diagnose hyperadrenergic POTS.   Specifically, if your norepinephrine is relatively normal while supine after 10 minutes but is more than 3 or 4 times higher after standing for 10 minutes. 

Unfortunately, there is no commonly available test that can be done to confirm that you have the dysfunctional RAAS / ACE2 deficiency subtype of hyperadrenergic POTS.  There is however a commonly available RAAS test that measures the ratio of Renin to Aldosterone that your doctor will probably perform if you suggest that your RAAS might be dysfunctional.  This test however will likely give misleading results.  The ACE2 deficiency results in a paradoxically high amount of Angoitensin II (which the test doesn't specifically measure), but a relatively normal Renin to Aldosterone ratio.  If you are male, you should also ask to have your testosterone checked since low male testosterone is common with POTS, and in my opinion is a very good indicator of the dysfunctional RAAS / ACE2 deficiency subtype when combined with the other hyperadrenergic pots symptoms and frequent urination.

If you do have a deficient ACE2, the main treatment is to prevent your RAAS from activating, which means that you can NEVER let yourself become dehydrated and you might need to supplement with additional fluids and salt.  If you are in a debilitated state due to your symptoms, then you are probably in a constant hypovolemic state with a frequently activated RAAS.  The only way to get your broken RAAS to deactivate is to get your blood volume up through fluids and salt - which I know seems somewhat counter intuitive considering that you are likely dealing with scary blood pressure surges.  The amount varies from person to person, but I would recommend that you increase fluids and salt through Gatorade, Propel, Nuun Tablets, and/or Pedialyte.  Salt sticks and/or sodium chloride tablets combined with increased water works as well.  Don't assume that you can achieve the needed salt by putting more salt on your food.  Everyone needs to play around with it until they figure out their sweet spot.  If you go too high, your blood pressure will rise from having too much blood volume, so what you're looking for is the sweet spot where your blood pressure and tachycardia stable out, but before your blood pressure starts to rise.  Because you can do real damage to your body if your blood pressure gets too high, you should go slowly, and I once again drop my disclaimer that you should consult your doctor before doing this.  My recommendation is to keep a very detailed diary of fluid and salt intake keeping in mind the sodium that you are consuming through food, and then increase by 1 gram of salt per week.  If the trend of your blood pressure immediately rises during the first week or two, then you probably aren't hypovolemic and probably don't have an ACE2 deficiency.  If the trend of your blood pressure over the week stays flat or lowers, then you could very well be hypovolemic.  Keep increasing slowly each week until the overall trend of your your blood pressure starts to rise.  When it does start to rise, then you know you've gone too far, and you should go back to the previous week's supplementation levels.  It takes some time to find that sweet spot.  I only need an additional 1-2 grams of salt per day, but I think that some people need as much as 8-10.

Appropriate medications are also extremely helpful.  Losartan is great as it suppresses the effects of the Antiotensin II, which is what ultimately causes a lot of the symptoms.  I take 100mg per day.  Lisonopril should theoretically provide the same benefits as Losartan.  Clonidine is a life saver during the adrenaline storms.  I take .1 mg every night before bed, and I'll take another .1 or .2 during the storms, but fortunately I haven't had a storm in a long time.  The clonidine pills are extremely powerful, but relatively short lasting so it can be a bit ticky to keep a leveled dosage in your body. The Clonidine patches can be used to keep you a bit more level. I haven't tried beta blockers myself, but most doctors will probably start with them because they are known to reduce tachycardia and blood pressure.  I however recommend against them because Dr. Grubb's research has shown a paradoxical effect where it can actually increase blood pressure for some of those with this condition due to unopposed alpha receptors, and I have also spoken to several of potsies that confirm this problem.  Dr Grubb has suggested that alpha-beta blockers can be an effective alternative to  beta blockers, although I have not tried them myself.  I also recommend an ssri to help prevent some of the anxiety driven adrenaline storms because even a little bit of anxiety can trigger some crazy adrenaline storms.  I recommend taking some anti-oxidant supplements because Julian Stewart has done some studies where it has shown some positive benefits since high Angiotensin II levels promote oxidative stress.  I also highly recommend getting your testosterone levels checked if you are male and supplement if you are low.  Testosterone supplements probably won't help much with this condition specifically, but will make you feel better overall.  And of course, a strong healthy heart makes a huge difference, so daily cardio exercise is crucial even though it is the last things in the world that you want to do.  Rowing machines, recumbant exercise bikes, and possibly even swimming are good ways to do cardio while minimizing POTS symptoms.