At A Loss About What To Do Next.

[~elizabeth~]

Your symptoms sound a lot like mine. All my family have dysphagia, some severely (also reflux issues). All my life I've been troubled by mucous issues, sometimes so bad they seemed to affect the stomach. All my life I have had hypotension, for most of the time it has been measured it's been 70/50mmHG. 3 years ago lots of odd things started happening, I developed lots of numbness and stiffness in my joints, severe dryness of eyes and mouth, shallow, rapid breathing and eventually worked out I had POTS. I also have positive ANAs, had a lip biopsy for Sjogren's (negative, as were blood tests), and was misdiagnosed with UCTD.

We have now finally worked out what is going on, thanks to the autonomic specialists in London. The underlying cause of my problems is joint hypermobility (Ehlers Danlos Syndrome), I'd say the vast majority of people in the UK who have POTS/autonomic dysfunction turn out to have EDS, there is a very strong correlation between the two conditions. You don't need to be hugely hypermobile to have EDS, in my family it manifests mostly with autonomic or connective tissue problems like hernias, varicose veins and reflux, rather than in joints. The positive ANA problem isn't inconsistent with this, quite a few people with EDS turn out to have mildly elevated rheumatological markers for reasons that aren't clear. It's not necessarily the case that dry eye/mouth (sicca) and sweating problems are rheumatological in origin, they can be due to purely neurological issues.

Although my BP overall is still low, it does rise on standing. Autonomic tests revealed this is because my noradrenalin levels rise a lot upon orthostasis, and this may well be why your BP rises after standing too. The function of noradrenalin is to constrict blood vessels to maintain blood pressure and circulation to the brain. Unfortunately if they constrict too much then the lack of oxygen causes numbness. In the hands/feet this is known as Raynaud's phenomenon, but it can happen in other parts of the body as well. We now know that a lot of my odd symptoms, including numbness and pain in my face/ear canals/tongue/throat and also right flank area are due to lack of blood supply, because treating my low BP with midodrine made them all hugely worse.

My autonomic tests also revealed that I have vagus nerve dysfunction, and I think this may be the cause of the dysphagia and mucous issues as starting pyridostigmine has worsened the symptoms. Both my sympathetic and parasympathetic nerves seem to be hypersensitive, which is the case when nerves are damaged and you develop something called receptor supersensitivity. It makes it very difficult to treat. I cannot tolerate meds to lower the norepinephrine, because they increase my muscle weakness. Drugs to raise BP trigger vasospam all over my body, and drugs that increase acetylcholine irritate my vagus nerve. I also have a painful, burning face condition, that seems to be co-existant Raynaud's/Erythromelalgia. Like you, I find that just small movements can trigger it, as even small rises in adrenalin levels trigger it (even just from changing position in bed).

I've wasted several years with doctors who had no idea what was wrong before finding the right specialists based around UCL in London who have actually pieced together exactly what is going on with my unusual set of symptoms. Local cardiology/rheumatology etc were a complete waste of time. I don't know where you are (in Europe?), but I'd recommend trying to find your nearest specialist autonomic unit as it doesn't sound as if you've seen any doctors so far who have the least bit of expertise in these disorders. It may well be the case that like me, you are a difficult case to help but at least reserve your judgment losing faith until you have seen a good autonomic neurologist. There is a list on DINET somewhere of specialists, including in Europe.

Neuropathy was confirmed by skin biopsy. Two out of four Qsweat test locations results were abnormal. One was a low response, and another no response. Two were normal.

A number of specific antibody tests were negative. There has been no specific tests for what I would guess to be most likely.

There may have been a bacterial or virus infection in the stomach/intestines, but I would not bet on it.

My original complaint was swallowing. I got caught up on the reflux train (ironicly I have it now, but not then). Then I ended up on PPIs, which did a number on my stomach. I did not tolerate them well. As I complained of no improvement, they prescribed larger and larger doses and stronger stuff. Eventually I figured out that the PPIs were no good for me and weaned myself off of them.

It took a long time before my stomach/intestines to settled down afterwards. I do wonder if I did not create an environment for the bad bacteria to get the upper hand. Who knows. I did have chronic diarrhea for months.

The swallowing was my first reason to start going to the doctor, and is still unresolved. I think that I finally figured it out. On an endoscope a doctor mentioned an interruption in the mucosa. I am not certain what that means, but I am guessing that it means an interruption in the mucus lining of my esophagus.

My main complaint with the swallowing was mucus. What I thought was mucus. I know now that it is abnormally thick saliva from my parotid gland. I did not know that then. This trouble swallowing led to weight loss, but I kept working. At work and at home. Building an addition at home, remodeling, keeping up with my bird project, gardens, orchard, etc. Working six days a week for a living. I lost 30lbs off of a 190lb 6' 2" frame.

I have had a chronic stuffy nose forever. I do not remember when the last time I had a runny nose. Just a dry stuffy nose. Along the way I have developed "cotton mouth". Never paid it a lot of attention, but I noticed it. It has came along with this and has not went away. Now I have on again and off again dry eyes, but can't say that is nor from medication. I have been off and on clonidine which can cause that.

[gjensen]

Dave, that sounds very familiar. Even the exact weights. My lowest weight was exactly 158. Concerning the heart rate. I get to 150 here and there. The typical is 120-130. Highest was near 250, twice. Highest documented was 220. Oddly the highest is when I have my "spells" and they are have been when I am at rest. They are quite painful when they come out of nowhere like that.

Elizabeth, you are right. Where I am lacking right now is a thorough work up. I like my doctor, and he actually pretty darn sharp. His focus has been digging into why. He felt that it was most important to see if a underlying cause could be found. I felt the same way. There is just a lot there to sort through.

I never considered EDS because there is no hyper joint mobility. I do have a hernia, reflux, etc. In fact I think the hernia has gotten worse. It gets to throbbing in certain positions. Varicose veins is prominent on both sides of my family.

I did not do well on pyrodostigmine either. Initially, I had hope. Then I ended up on a roller coaster and it increased my blood pressure and fluctuating blood pressure.

Both of these posts have been interesting and helpful. Can I ask what has given you two help and or relief? What kind of improvement have you had? Trying to get out of bed. LOL.

[~elizabeth~]

I think joint hypermobility is probably missed in men quite often, the muscles are larger/stronger and better able to stabilise the joints. The only men I know who are diagnosed have only got there after being diagnosed with autonomic dysfunction. I only noticed my hypermobility in my mid 30s, when I was young I couldn't even touch my toes I was so stiff. All my male relative on the EDS side have things like hernias, varicose veins, osteoporosis, orthostatic hypotension, and some have something called Adie's pupil (loss of parasympathetic innervation to the pupil), but not a single one has dislocating joints.

The pyridostigmine is helping my POTS a lot, it has reduced the tachycardia and agitation a lot and helped the dryness issues and gut motility and seems to be helping my muscle strength, but then it's causing these unpleasant electric shock/numbness feelings along the path of the vagus nerves.

[davecom]

Since I am still struggling every day I feel like it would be foolish for me to tell you what has helped - since I am no guru and haven't recovered myself. Am I significantly better than when things were at their worst - yes. I have less pain in general (knock on wood), more normal gastrointestinal function (hope I'm not jinxing everything by saying that... this has made me superstitious), and slightly lower heart rates. But when I get up I still sometimes see 130s heart rates. I still can't stand still. My hands and feet still turn purple (do yours by the way?). What has helped? Time, exercise, tons of liquids, a limited diet, following a strict routine (I walk every 2 hours for a set amount of minutes - exercise on a schedule too), and plenty of rest (I had to quit my job/career and I'm trying to start a new one from home but symptoms make it hard still).

What really concerns me about your case is that you're saying you have had these ultra high heart rates while sitting down - that's alarming. I've had elevated heart rates around 100 sitting or 120 sitting in a hot shower but never anything too crazy like what I get standing. How do your doctors explain that?

Dave, that sounds very familiar. Even the exact weights. My lowest weight was exactly 158. Concerning the heart rate. I get to 150 here and there. The typical is 120-130. Highest was near 250, twice. Highest documented was 220. Oddly the highest is when I have my "spells" and they are have been when I am at rest. They are quite painful when they come out of nowhere like that.

Elizabeth, you are right. Where I am lacking right now is a thorough work up. I like my doctor, and he actually pretty darn sharp. His focus has been digging into why. He felt that it was most important to see if a underlying cause could be found. I felt the same way. There is just a lot there to sort through.

I never considered EDS because there is no hyper joint mobility. I do have a hernia, reflux, etc. In fact I think the hernia has gotten worse. It gets to throbbing in certain positions. Varicose veins is prominent on both sides of my family.

I did not do well on pyrodostigmine either. Initially, I had hope. Then I ended up on a roller coaster and it increased my blood pressure and fluctuating blood pressure.

Both of these posts have been interesting and helpful. Can I ask what has given you two help and or relief? What kind of improvement have you had? Trying to get out of bed. LOL.

[gjensen]

No one has gave me an explanation for anything yet. When I first had the high heart rates at rest, they alarmed me to. Suddenly my one safe place was not safe.

The first one I had a weird sensation spread over me, and then BOOM. I checked my heart rate and it was 160 and my blood pressure was climbing. Went to the ER and all of that. It did not look like it was going to stop. It did eventually settle down.

It happened the other day, and part of what motivated me to write this post. I was laying down feeling awful like I described. Then that sensation came over me, everything went dark, I quit breathing, had the swallowing sensation. My heart rate went up real high then.

I do not get concerned about the heart rate and standing. I did at first, but it is tolerable. I will push it until it gets too bad. My head symptoms is what limits me. When I feel my cheeks going numb, I get off of my feet.

The spells at rest bother me. A weird numbing doom feeling takes over. Sometimes I can tell that one might hit. Other times it is with no warning. My breathing stops and everything. Everything goes dark. Just momentarily, but the heart races for some time afterwards. Afterwards I always go through this chattering shaking spell as if I was cold. I have had doctors tell me it was anxiety, LOL.

No. I do not think that I could get used to these spells. I do not like knowing another one will come sooner or later. I always wonder what it is and if it will settled own. It is a roller coaster afterwards with peaks and valleys. It always hits suddenly though. Very weird sensations accompany this.

I think that I would have rather heard this was normal for this LOL.

Under my finger nails stays purple pink now. My blood vessels enlarge in my legs more than they used to if I stand for a bit. That is all I have seen concerning these things. My lips become discolored and my face pale if I stand to long, or when I am having a flare like related to this post. My wife gets concerned about that.

Dave, you will get better. You have the right mindset. If I had to bet on it, I would say that you will continue to improve. I look forward to hearing progress.

[gjensen]

Elizabeth, I think that is a fascinating topic. I will be interested in learning more. I hope the new drug helps you. I might not have given it much of a chance. I get these weird spells, and one might have hit me as I was starting it. It is hard to say.

You are obviously very bright. I have enjoyed reading what you have shared.

[davecom]

Thanks, I hope so. Like you, I can't imagine living the rest of my life this way. In fact, with my congenital heart defects, I'm pretty sure this would eventually kill me if it didn't eventually heal - just too much constant stress on the heart.

Are they considering putting you on beta blockers since you have elevated heart rates sitting?

No one has gave me an explanation for anything yet. When I first had the high heart rates at rest, they alarmed me to. Suddenly my one safe place was not safe.

The first one I had a weird sensation spread over me, and then BOOM. I checked my heart rate and it was 160 and my blood pressure was climbing. Went to the ER and all of that. It did not look like it was going to stop. It did eventually settle down.

It happened the other day, and part of what motivated me to write this post. I was laying down feeling awful like I described. Then that sensation came over me, everything went dark, I quit breathing, had the swallowing sensation. My heart rate went up real high then.

I do not get concerned about the heart rate and standing. I did at first, but it is tolerable. I will push it until it gets too bad. My head symptoms is what limits me. When I feel my cheeks going numb, I get off of my feet.

The spells at rest bother me. A weird numbing doom feeling takes over. Sometimes I can tell that one might hit. Other times it is with no warning. My breathing stops and everything. Everything goes dark. Just momentarily, but the heart races for some time afterwards. Afterwards I always go through this chattering shaking spell as if I was cold. I have had doctors tell me it was anxiety, LOL.

No. I do not think that I could get used to these spells. I do not like knowing another one will come sooner or later. I always wonder what it is and if it will settled own. It is a roller coaster afterwards with peaks and valleys. It always hits suddenly though. Very weird sensations accompany this.

I think that I would have rather heard this was normal for this LOL.

Under my finger nails stays purple pink now. My blood vessels enlarge in my legs more than they used to if I stand for a bit. That is all I have seen concerning these things. My lips become discolored and my face pale if I stand to long, or when I am having a flare like related to this post. My wife gets concerned about that.

Dave, you will get better. You have the right mindset. If I had to bet on it, I would say that you will continue to improve. I look forward to hearing progress.

[AllAboutPeace]

Has anyone mentioned Paroxysmal Supraventricular Tachycardia (PSVT) to you regarding those very high heart rates while sitting. It can hit out of nowhere and causes a sudden very high heart rate. I've had it go instantly from 70's to 180's (and higher) while sitting quietly. It doesn't go incrementally up like sinus tachy - it just makes that big jump at once. The longest I've ever experienced it was for 15 minutes, but it didn't stick at the higher rates for the entire time - it cycled up and down. My EP says it's an 'electrical' issue.

I'm not sure if it fits in your case, but I thought it was worth mentioning. The heart rates can go from 60-250+ The following is a brief excerpt about symptoms:

PSVT often presents with the complaints of palpitations described as a rapid heart rate. There can also be a fullness in the throat that is associated with the rapid heartbeat. Other symptoms include the following:

lightheadedness,

weakness and fatigue,

shortness of breath,

chest pressure, and

syncope (passing out) may also occur.

The symptoms of PSVT usually occur while the heart is beating quickly but the fatigue can persist after the heartbeat returns to normal. By its nature, PSVT is intermittent and its onset cannot be predicted. As well, once the heart starts to beat quickly, there is no way of predicting if and when it will return to normal rhythm on its own. Some patients are unaware that their heart is beating quickly and PSVT is found only when the patient presents for care and is found to have a rapid heartbeat.

If you do not have symptoms or any other heart condition, PSVT may not need treatment.

If you have an episode of PSVT, there are techniques you can try on your own to interrupt the fast heartbeat.

One is called the Valsalva maneuver. To do this, you hold your breath and strain, as if you were trying to have a bowel movement.

Another technique you can try is to cough while sitting with your upper body bent forward.

Some people find that splashing ice water on the face is helpful.

[gjensen]

Peace, no one has mentioned that to me. That is very similar to what I experience. Sometimes I know it is coming. Other times I do not. Mine never stays either. And it tends to fluctuate. I think the highest HR and longest lasting might have hung around at 200 for a little while. I have peaked at around 250 twice. Like you, as it settles it tends to fluctuate. Up and down.

I can't get some low readings in the middle of these spells. I can feel the up and down.

I suspect one of those spells might come soon. I feel awful with the numb cheeks etc. Dizzy. I do not know how to explain it. I just get a weird sensation with the ears ringing louder etc.

I wonder what the trigger is, and how it is associated with these sensations . . .

I also end up shaking. During, but most often as it settles back down. As if I was cold. Sometimes I will feel cold, other times I will not.

Well, you put a name on it for me. I will look that up. Thank you very much.

[gjensen]

Thanks, I hope so. Like you, I can't imagine living the rest of my life this way. In fact, with my congenital heart defects, I'm pretty sure this would eventually kill me if it didn't eventually heal - just too much constant stress on the heart.

Are they considering putting you on beta blockers since you have elevated heart rates sitting?

No one has gave me an explanation for anything yet. When I first had the high heart rates at rest, they alarmed me to. Suddenly my one safe place was not safe.

The first one I had a weird sensation spread over me, and then BOOM. I checked my heart rate and it was 160 and my blood pressure was climbing. Went to the ER and all of that. It did not look like it was going to stop. It did eventually settle down.

It happened the other day, and part of what motivated me to write this post. I was laying down feeling awful like I described. Then that sensation came over me, everything went dark, I quit breathing, had the swallowing sensation. My heart rate went up real high then.

I do not get concerned about the heart rate and standing. I did at first, but it is tolerable. I will push it until it gets too bad. My head symptoms is what limits me. When I feel my cheeks going numb, I get off of my feet.

The spells at rest bother me. A weird numbing doom feeling takes over. Sometimes I can tell that one might hit. Other times it is with no warning. My breathing stops and everything. Everything goes dark. Just momentarily, but the heart races for some time afterwards. Afterwards I always go through this chattering shaking spell as if I was cold. I have had doctors tell me it was anxiety, LOL.

No. I do not think that I could get used to these spells. I do not like knowing another one will come sooner or later. I always wonder what it is and if it will settled own. It is a roller coaster afterwards with peaks and valleys. It always hits suddenly though. Very weird sensations accompany this.

I think that I would have rather heard this was normal for this LOL.

Under my finger nails stays purple pink now. My blood vessels enlarge in my legs more than they used to if I stand for a bit. That is all I have seen concerning these things. My lips become discolored and my face pale if I stand to long, or when I am having a flare like related to this post. My wife gets concerned about that.

Dave, you will get better. You have the right mindset. If I had to bet on it, I would say that you will continue to improve. I look forward to hearing progress.

Hate to admit it, but I do not think than anyone is considering much of anything. That is how I feel right now.

I did try Bystolic. I stopped. My blood pressure tended too low, and mostly it aggravated my breathing problems. After all day of difficulty breathing a few different days . . . I said that was enough.

[dmjl27]

I'm having very similar symptoms right now. I have severe pure autonomic failure. I believe the HYPOPERFUSION (not enough blood to the brain) is the cause of MANY of my new and old symptoms. It's getting much worse. For some odd reason, it's worse right now than it has ever been. It's very scary!!!!! It hurts more than most of my other problems, and let's be honest...it scares you because there is always that stroke risk in severe cases. However, there are a few things u can do to help.

1) elevate your legs but do not lay flat. (kind of like in a recliner position). Lying flat can actually make things worse!

2) No matter how bad you feel, get up and walk around at least once an hour. I'm bed bound all but going to the bathroom or getting water. Every 50 minutes, I make myself get up and walk to the kitchen. It's very important.

3) It's proven that lying in the bed or couch will actually make hypoperfusion WORSE. I know, makes no sense to me either, but it does. Keep that in mind.

4) Watch your arms. Blood can pool in the arms, so try to keep them on your stomach or chest

5) wear compression stockings even in the bed

It's very normal for the heart rate to go up very high in result of not enough blood to the brain. It's trying to compensate.

Research hypoperfusion online for more infomation on what is actually happening inside the body. I cannot get rid of these symptoms completely, but I can keep the blood flowing so I am staying safe.

IN my experience, if you have deprived your brain of blood, these symptoms can linger for WEEKS after an acute incident. I'm extra sensitive for 2-3 weeks after an incident. You have to curb your routine during the next few weeks. My worst is the dizziness that lingers. My doctor gave me something to help with that debilitating symptom..and it lowered my heart rate, too!

[gjensen]

Thank you very much Melanie.

What would you think about mine being instigated by me being upright too long, or when I am most symptomatic, by just sitting up?

I had a bit of a break yesterday, but I paid for it last night and this morning.

This is my most limiting symptom. I do not get worked up over the heart rate anymore. Like you, I am figuring that many of the most troubling symptoms is a result of hypo perfusion. That is if what I am experiencing is in fact that.

Have you had any tests that showed this, or is your doctor going by your symptoms?

If I could learn to make improvements on this topic, I will be much better off. Thank you for the tips.

[IceLizard]

I am very sorry that you are having to go through all of this. I understand because I was nearly as bad 9 years ago. I was bedridden: any attempt to stand or sometimes even sit caused me to have a jump in heart rate followed by collapse then shaking or convulsions. I felt bad even when lying down and felt like I was slipping away. I am 5'6'', but I lost weight until I was 108 pounds. I had acid reflux and no appetite. Eating made all of the symptoms worse. I lost muscle until my knees were the largest part of my legs.

When in this dire situation I was lucky enough to get in touch with the good people of the National Dysautonomia Research Foundation. They convinced me that to be able to get out of bed I must exercise. So, I started doing muscle strengthening exercises in bed. Then I progressed to getting up and doing 1 squat before lying back down. I would do 1 squat every hour or so (or more frequently if I could) throughout the day. That added up. Eventually I was able to get up and walk around the room. I was really excited when I could make it to the bathroom! Then I tried walking down the hall. I also had to force myself to eat more, yogurt helped. Cymbalta also helped my autonomic functioning.

Now I am doing much better, but I still struggle every day. At least I can usually manage to get out of bed and do about 50-70% of what a normal person could do in a day.

I hope you find a way to better times.