kitt

Congrats Kelly on getting this diagnosis nailed down

I guess many of us are in the process of doing so. Can you add the list of 40 symptoms that he asked you about?

It was me who posted the Grubb video talking with the young girl about hyperpots mca. I also added a link to a paper he wrote that has a list of symptoms for mca involvement with pots.

I took this list to my cardiologist, and he jumped on it. Flushing was listed as a symptom that is in 100% of patients with mca.

From memory I was surprised to read that only 38% of mca pots patients had high BP.

Dr. Grubb does understand mca with pots. I think the only obvious thing he differs on (from what I can read) is how prevalent it is. If you're looking for the video he did on hyperpots mca, it's on another thread, (think it's called Dr. Grubb and hyperpots mca) If you're looking for the list of symptoms he has written, (it's somewhat hidden in his article, as you have to double click on a box to see it), I can find the paper. But it too is on the same thread as the video.

Congrats again! Huge step forward in getting your treatment streamlined.

Anna,

Are you familiar with the work of Dr. Rowe at Johns Hopkins? He has devoted a great deal of study to children with ED/pots/ CFS.

On the day I was dx with POTS by an immunologist who specializes in CFS, I asked her why Johns Hopkins was 'so focused on children with CFS and Ehler-donlos?"

Her short answer in laymans terms "floppy joints can/does suggest a floppy vascular system'.

With 3 children with EDS, think you'd be interested in researching the work of Dr. Rowe and his colleagues. Would be interested in hearing what you think. (Like many here, I have EDS as well.)

This should probably be on a separate thread, but wanted you to see this. Somebody can move it it they feel it needs to be elsewhere. Just wanted to make just Anna sees it.

He has a webinar on utube

Thanks all for the great feedback.

People have made good observations on this thread.

Arizona girl, Interesting because during the short periods when I took BB's my bp was especially low in the middle of the night. 70's over 50's. Can no longer take BB's due to the cardiologist believing I have hyper pots with MCA. Flushing, vomiting...

Alex, I agree that breathing techniques are incredibly helpful for both pots and many other issues. I learned years ago 'how' to meditate and 'do' visualization and it's very helpful. You tube has videos with simple techniques. Deepok Chopra has a free newsletter with good techniques, and many places offer CD’s for sale. I find the simplest meditations (which include breathing techniques) work best for me. I can simply turn it 'on' in my mind and it becomes an automatic soothing technique for relaxing and falling back asleep. (Expect lately with the NE surges in the middle of the night.)

Flushing though is different animal. If my recollection is correct Dr. Grubb's list of symptoms for hyper pots mca, flushing is the only symptom that is in 100% of people with hyper mca pots. (sign of mca)

Thank you Alex for the kind thoughts. Hope you're doing well?

Seattle rain...Your comment makes sense that NE surges happen in the middle of the night, and anti-depressents can help. Am so sensitive to drugs that I avoid taking any more than absolutely necessarey. Tend to be overwhelmed by side affects. But would certainly take them for this if I could tolerate them. Is anyone taking SSRI’s to help with NE surges? Which ones have you found to be helpful?

Angela, I'm with you on the breathing. This does not feel like a panic attack to me though. I just suddenly and abruptly wake up about every 2-3 hours during the night, and think 'really? this %&!* again? and again and again. I do take a benzo, (klonopin) before sleep, and have for many years for CFS, and it's been incredibly helpful for CFS issues. Its a well documented treatment for neuro immune issues, but I have no knowledge of how it's used for pots.

E Soskis, you gave an interesting description. You said your skin feels so hot it burns. I've tried to describe it and it feels hot and cold at the same time. Like fire and ice… that’s the best description I can give.

Lisa, thank you for telling me I'm not alone. Like you I'm not taking meds either. (Yet.) I see my cardiologist next week. Have you talked with Dr. Thompson about meds for hyperpots? (I didn't know he was practicing.) As for my experience since first posting, this waking with the sweating clamminess seems to 'cycle' but the wide awake wakening is every single night. Becoming tedious. Do other people with hyper pots have any ideas for this? Do you take H1 and H2 blockers before sleeping?

I would just like to sleep at night and have some energy during the day. The not sleeping is wearing me down.

Chairi malformation is referenced several times in the new Mayo pots paper several times. (Online here on a separate thread.)

Some dancing around it, but certainly not avoiding it. Very interesting as this has shown up for years as an aspect of chronic fatigue syndrome in published papers. Not conclusive, but mentioned much the same way it's mentioned here.

This new Mayo papers makes a direct link between Pots and CFS (which is an amazing leap, and far overdue on their part) but finally.

Here's one comment in the Mayo paper about Chiari malformaion.

Quoting "Orthostatic intolerance has also been reported in patients with type 1 Chiari malformation. In some cases associated with syringomyelia. However, a direct between incidental type 1 Chiari malformation and orthostatic intolerance has not been convincingly demonstrated"

Note the use of the words 'incidental'. As I have stated before, in my opinion Mayo researches tend to regard important findings as incidental.

There are other references to Chiari in the Mayo paper, but none as significant as the one I mentioned...(at least not one I've found. It's a tedious paper though.)

Chiari has come up in multiple papers on CFS, and now even Mayo is making very defined statements about the relationship between CFS and pots. As I said a huge leap on their part. How long before they acknowledge Chiari malformation as something other than an 'incidental finding'?

Think Chiari is becoming (continues to become) an interesting and ongoing aspect of pots research. If it was not an aspect they're considering, why would they continually include mentions of it in research papers?

.

.

.

.

.

Arizona girl,

He's done a TTT, a 2 week holter monitoring, echo, stress echo, prescribed a bb, (with horrid results) and he now believes it's hyper pots with mca. He's been clear that he believes it's hyper-pots with mca, due to my response to bb's and the constant flushing.

My flushing started after all the testing he first did, and almost immediately after starting the bb he prescribed, (probably because bb's cause mast cells to degranulate?)

Think he's ruling out the pheo, which he should do, just as he first ruled out IST, before he confirmed the pots dx I'd already gotten from an immunologist. (IST is another form of dysautonomia, called Inappropriate sinus tachycardia), and apparently it's not uncommon for people with one to be misdiagnosed with the other.

He's been very through. Think the reason all catecholamines weren't measured has more to do with his nurse than him.

Again, I just feel very fortunate to have found this cardiologist. His credentials are wild...What he's done, and where he's done it. Amazing and impressive.

He really does understand dysautonomia, even one as rare as hyper pots with mca. (His nurse though is not used to seeing much dysautonomia though.)

Like lots of us, I've experienced some horrible doctors with other 'orphan' diseases I have.

That's why I said, I actually would prefer a pheo...Like you say, it's a curable condition. Wouldn't we all rather have that?

How do you manage your pots? I've learned so much from this forum! Thank you for that. I'm very appreciative.

Arizona girl, I mentioned the supine portions of the test at the hospital, but I assure you, THAT was not going to happen. There was a bed in the room, but only one room and other patients waiting.

I absolutely understand the purpose of supine to standing...So they can see the differential, but the most important part to the cardiologist at this point is to see how high the NE goes.

If anyone has a link to the correct procedure, please paste it. I'll print it out and give it to the cardiologist.

I'm almost 100% certain the cardiologist I see knows this, but he didn't write the order. He gave the order to his nurse and she had it typed up.

At first tryptase was not on the typed order. I had to track the nurse down and mention that the cardiologist had said he would order that as well, and the nurse added it as an addendum.

I'm just glad I got the test standing. It's a nightmare to think of going in there and having the blood drawn sitting and being told, 'your NE was normal'

Btw, when I called the cardiologist's nurse yesterday she said 'Why does it need to be done standing?' I reminded her that doctors had been measuring my heart rate and BP for years sitting, and that's why I'd never been diagnosed with pots. I reminded her that having those measurements done sitting is the 'correct protocol.' That was a lightbulb moment for her.

Bear in mind my cardiologist did not list all catecholamines. He also added metanephrine to check for a pheo, and also added cortisol, and tryptase.

As a thought..I actually wish this was a pheo. Unlike pots it could be removed.

Sue, Unbelievable! I read the same article in the Sunday NYT's!

I even saved the article. I'm reading and reading, and it's all about M.S., (I'm not convinced it actually helped many M.S. patients, as most doctors also believe), but I do agree that if someone with M.S. wants to go through this invasive procedure, Godspeed.

Like you, I almost fell over when I read, "this is just treating autonomic dysfunction."

I wondered though if it really would help autonomic dysfunction. Surely the doctors at Vanderbilt and Dr. Grubb would know if it would and would be doing this procedure on many many patients if they believed it could help?

Amazing that we both read this same article, and picked up on it. It's a fairly long article.

Heat intolerance is also a huge factor in CFS and other illnesses, not just MS.

This procedure and M.S. reminds me of a 'phase' when people with CFS were going through very invasive procedures because they thought CFS was caused by Chiari malformation.

Notice how many symptoms of Chiari malformation also are also symtoms of dysautonomia and CFS!

http://www.ninds.nih.gov/disorders/chiari/detail_chiari.htm

Yesterday, I called the cardiologist's office and told the nurse I was going to have my blood drawn at the hospital tomorrow, (this morning), and mentioned there was nothing on the order about having the blood drawn while standing. She said, "The doctor is in surgery today. The hospital should have a procedure code, but if you have a problem, call me back tomorrow, and I'll speak to the doctor."

Went to the hospital this a.m., fasting, and signed in etc...

First thing that happened, I was told to sit in the chair to have my blood drawn.

I explained I was supposed to be standing for 15-20 minutes before the blood draw. She said, "It doesn't say that on the order."

Explained that it was for the NE part of the test.

She huffed and puffed, and finally said, "Okay, go walk around the hospital for 15 minutes and come back." I did.

When I came back, the first thing the next person said was, "okay, sit in the chair and put your arm here"...I explained I needed to have the blood drawn standing.

They did it correctly, but this is exactly why it's important for everyone to educate themselves on something as simple as having blood drawn.

Even after calling the hospital etc...I still was still told to sit down. (Twice.) Amazing. Be proactive!

.

I'm dealing with this as well.

Not just cognitive function but mispronunciation of words.

Several times I've said referring to the show 'Project Runway', I've called it Pwoject Wonway'

Sounds like Gilda Radner doing Babwa Wawa.

Have a neighbor who is a neurologist, and once during a casual conversation I couldn't think of the word I was was looking for, and she watched me try and said, 'Dysphasia'. She couldn't help but be a doctor and diagnose me. Guess she'd heard me do it enough.

But find turning my R's into W's really strange.

.

I don't know if this helps, but I once read that being 'quickly satiated' while eating can be a symptom of POTS.

This 'hit' me, because I never eat more than half of anything served to me. I get full quickly on a small amount of food and always have.

Even a whole sandwich is too much. I eat half, and I'm done.

Feel for people who struggle with their weight. Think there's much more to it than calories in and calories expended...I suspect it's far more complicated. They're finding all kinds of hormones in the stomach that have a huge impact on when and why we feel 'full'.

,

This is a topic I haven't even wanted to broach, but it's good to read other people talking about it.

For the last year people say, 'I can't hear you...speak up' and I feel like I'm speaking normally.

I've had recurring dreams for months and months where I'm trying to speak and I can't get the words out. Desperate, on the phone, and no one can hear me dreams.

So I feel like I'm speaking loud enough, but am very often told, "what?"

I have pain in my chest sometimes, and try to breath deeply and meditate. I think meditating and learning to breath while doing it is very helpful.

Meditation videos on the internet help and D. Chopra has a free newsletter, and often has good (and short) instructional videos on easy short mediations and breathing techniques.

Hope this helps somebody. It's helped me.

.

Sorry for your nightmare doctor. There are lots of them out there. I agree, the important thing is not to go back.

I live in a big city and couldn't find a doctor that knew about POTS. (I had to go out of town to get a diagnosis.) I made calls, talked to some M.D.'s who are friends, and finally spent about 15 hours searching on the internet. No joke. All in an effort to find a doctor here in my town.

Finally, I found a 'dead' forum where people had posted about various forms of dysautonomia. The posts were still online, but you couldn't contact the writers of the posts, and you could not reply to a thread or post a new one.

On the site a woman with IST, inappropriate sinus tachycardia, a form of dysautomnomia mentioned she had found a cardiologist in my town to treat her. She mentioned him by name. It took a long time to get an appointment with him, (his credentials are amazing), but it was worth it. He's also a board certified internist, so he gets the big picture.

He tried a new BB, and when my BP dropped as low as it on one I tried previously, he began to suspect hyper pots with MCA. (I have flushing and vomiting.)

I feel very fortunate to have found him. Sometimes the harder I try, the more fortunate I get.

Don't give up. There may well be somebody near you that will help. Keep trying and good luck.

I suppose its lowering the NE levels which isnt really the problem if the idea of the transporter cells for NE are whats lacking . would this be correct? or am i way off.

Awwww, diamond, you made me have a light bulb moment ---what if because there are so many pathways and hormones worked on with Tramadol ---with me ----maybe it causes the NET to function properly?????? Since it is a NE reuptake - it would make it circulate higher and hold on longer before elimination ----but, since there could be a faulty connection somewhere else in the line and this works on serotonin, dopamine, NMDA, and opiate receptors - maybe the tweaking of one of the other things - pushes the NE transporter to function properly on me. OKay, now I'm way out on a limb!!!! But, what if that's what's happening with me??????

To answer your question ----whose to say that lowering NE wouldn't be another alternative way to treat high NE levels? If, it's lower and there is less to process then the transporter doesn't have to work so hard to pump it out. Think of a garden hose attached to a pump ---if the garden hose is small and the hose is turned full blast - it has a lot of volume to disperse, high pressure load --- but only so much can go through at a time. If the pressure is turned down and the volume amount is lower then the hose still delivers the same amount - but, it doesn't have the pressure behind it to try to deliver more - faster - causing a build up on the back side of the line. I hope that makes sense to people ---ha!ha! (I've learned quite a bit about this sort of thing over the years. , my husband's in the pump biz. )

Issie

Issie,

This is so interesting about the tramadol. I was taking it for another mast cell disease which causes pain, and was happy when I didn't need it as much. Now I'm realizing I had 'more energy' when I took it.

Right now I wake up all through the night with adrenaline surges, as well as bone pain. The adrenaline surges at night are bad, and it's hard to get back to sleep without taking a muscle relaxer.

I can't sleep on tramadol. I'm one of those who has the 'opposite reaction' to some drugs. For instance a sleeping pill will keep me wide awake.

Any more ah-ha moments about tramadol?

Am having a horrible time with total exhaustion and can't keep up with the doctors appointments.

Thanks all..

Hi, going to chime in! I have hyper pots diagnosed by Dr. Grubb. He suggested my cardio "she get supine and upright serume catecholamines done which consist of norepinephrine, epinephrine and dopamine levels". Mine rose from the 400s to 1000's. Which supported his diagnoses of hyper pots due to small fiber autonomic neuropathy.

If you look up labs and catecholamines, it will tell you how to prepare for the test, includes avoiding some foods and medications. I had mine done at a sonora quest lab and they did have a room I could lay down in. It is important to be very quiet and still during the first part and try to be mellow in the hours before getting to the lab.

Also Dr. Grubb has several articles about symptoms of hyper pots that have been posted here, or you can google his name, hyperandregenic symptoms and the article should pop up. He recently has also found a mast cell reaction cause as well.

Mainly though my symptoms were are significant rise in bp along with heart rate. You can feel jittery, break out in a cold sweat on your brow, feel fatigued on standing, to name a few.

There's no question about the correct procedure for doing this test. I agree with all that have had it done properly.

I've called Quest labs many times, and get a recording, "we can't take your call. Leave a message.' I did. They never called back.

I called the local hospital, and the person that answered the phone explained that Yes!, this is a complicated test. We have a procedure code we follow. Yes, if you come we will follow that code, and btw, you can sign a 'paper' and get the test results directly. I'm in.

So thank you! for chiming in. My concern was that I DID NOT WANT to contact my cardiologist and saying, "this is not correct, do it my way." Don't want that, because I'm dealing with a brilliant doctor so want to cooperate and not be problematic unless it's called for.

Thank you. I'm going to go to the hospital within the week, and will report back. (Have researched and found a mast cell disorder specialist in town...Pays to live in a big city.)

Thanks to ALL who have helped and put their two cents in! This has been my go to 'place'...Kudos to all!

They base your DX for HyperPOTS on what your standing NE levels are - my test at Mayo's range was anything above 650 was high - I was over 800. They also want to see if your NE levels are high while sitting - if so they would need to check you for a pheo.

I don't know what you mean about testing mast cells sitting. Mast cells are very hard to detect. They will check some of the chemicals that the mast cells put out when they degranulate. This can be done within one hour of an episode and then you are supposed to start a urine collection to see if the chemicals are in your urine. The only other ways are a bone marrow biopsy and now Dr. Afrin is doing biopsy's from tissue in the esophagus. I'm HyperPOTS and have MCAS and EDS.

Issie

Issie, thank you for your answer. All of these responses have been incredibly helpful.

I was/am having brainfog and mentioned having the mast cell testing done in a supine position because I think I misinterpreted Kellysavedbygrace's answer.

She wrote:

Last mo however, during a lab draw for mast cell testing I had a NE of 650+ taken in an almost supine position. I say this to point out that these levels can fluctuate a lot - thus the need often for multiple tests bc you have to catch the effect of the mast cells degranulating/ or NE levels rising. This is why the best place to test for hyper pots is a serum draw on tilt bc it is so controlled.

So my mistake.

I'm guessing that the test for mast cells everyone is referring to is a tryptase test? My instructions were to have the blood drawn during the time when I'm have an allergic response or episode.

I don't know about everybody else, but when my eyes are itching and tearing and I'm rubbing them, the last thing I'm thinking is, 'Oh, now would be a good time to drive to the lab and have blood drawn.'

So I'll call the hospital lab AND Quest tomorrow and ask them both how they test for norepinephrine and blood cortisol. Standing, sitting, supine, etc...

I've Googled 'serum catecholamine test' and it includes dopamine and there are differences in how the blood is drawn! So I guess the order needs to be specific! My point is illustrated on this site. http://labtestsonline.org/understanding/analytes/catecholamine/tab/sample

There is another thread on dinet about 'what else can go wrong with my body' or something to that affect. I'm not entertaining responding but all I can say is...'Oh, many, many, many things.'

Pots, and now adrenergic pots and most probably mca adrenergic pots as well as mastocytosis are just newcomers to an

ever-growing group of immune, neurologic, endocrine, bone, and pelvic diseases by body is juggling.

So I'm trying to keep up with not just the doctors appointments but my own understanding of what I'm being told.

As everyone here who has helped (Tremendously!) knows.. being an informed, educated patient takes a lot of time and energy, so thank you for your patience and sharing.

All of the lingo and specifics are familiar to most here. It's all new to me, and I'm trying to make sure the tests are done correctly. Don't want to waste time, energy, and money seeing specialists by being a 'sheep' and just going through the motions.

As I said, if it weren't for the help of all of you, I would have gone to Quest with this lab order and had my NE levels done (along with cortisol and tryptase) while sitting, and then gone to see the cardiologist at the end of the month for nothing...

So I thank each and every person who has/is helping me on this journey. I hope I can return the favor.

Hyperpots, thanks, I learned in my research since posting about dopamine being one of the catecholemines. I also learned that metanephrine is done to rule out pheochromocytoma. So you answered a question it took about an hour on Google for me to figure out.

That's the beauty of this forum. An amazing amount of good information.

Kelly, I see your point as to why having the blood draw done on a TT is optimal, but think that will happen only at Mayo and other big clinics where they have a lab in house.

Am thinking that instead of going to Quest, I should call the hospital, and see if they can do the test in the supine position and then standing.

My question to all of you. Why is it necessary to have the test done both ways..To see the differential? I just want the dx to be corrected from Pots to Hyper, so wouldn't a standing epinephrine test be enough?

Am curious why the mast cell test are always done in supine position? That's interesting. Have read that it the results vary a lot.

To my knowledge Quest lab doesn't even have any rooms for patients to lie down in.

Am going to call the cardiologist's nurse, but don't want to 'correct' the cardiologist other than to mention the standing part of the test, but if someone explains the 'need' to do it both supine and standing, I'll suggest both.

The cardiologist I'm seeing has treated IST (dysautonomia involving the sinus), but I'm sure he's never treated a case of hyper pots before, but he 'gets it,' Just want to be respectful about 'suggesting' how he order tests. I've heard so many horror stories about cardiologists telling patients with disautonomia, 'your heart is fine!'

I know everyone here has hyper pots, but am wondering how many have hyper mca pots?

Thanks again. Honestly, If not for this forum I would have gone to Quest, and they would have drawn the blood with me sitting in a chair, and I'd be back at square one.

This is an amazing resource.

So I have hyperadrenergic pots. I have been getting a lot of my salt intake by putting it in water. Today my dad called and said that drinking salt water is bad for you. He said that it can be very hardon your kidneys. He said it would be better to salt your food? Just wondering how everyone does it?

Amelia, I buy organic sea salt by the bag. This is the brand I get, and you can read about the huge difference in sea salt and salt that is mined, (regular table salt) http://www.redmondnatural.com/

I bought a bag of empty soft gel capsules from the health food store, and made my own sea salt capsules.

Also find that to use 'Electromix'...At health food stores, Whole Foods, but cheaper to order online.

I don't care for Gatoraide and but a packet of Electromix in a small amount of cold water. It helps.

Good luck!

(I'm in the process of being tested for MCA hyperadrenergic Pots since I started flushing and vomiting.)