Hyperadrenergic Pots And Dr. Grubb

[Kellysavedbygrace]

Kitt, sorry to hear youve been feeling so lousy. I also have low BP. On BBs I have had my lowest recorded BPs- although Im normally pretty low. I have discontinued use of BBs due to side effects/fatigue low HR/possible hair loss. I have had two tilts- 1st one stopped at 5 mins second stopped at 9 mins. Since antihistamines are avail over the counter it wouldn't hurt to try- but usually for us we need more than the recommended OTC dose so that's why it's import to get tested and work w a doc who can recommend right dosage. As far as MCA- I think you are right on- think it is a much bigger player in POTS than at first glance.

I see the new Dx as good news because it helps to explain the widespread multi system dysfunction And it is something that is currently being studied.

[NMPotsie]

I have severe chest/facial flushing, probable MCAD with elevated tryptase, methylhistamines, prostaglandins and IgE. Also dxd hyperpots with significantly elevated plasma cats. I am on the Mayo mast cell protocol AND beta blockers, which work well for me, so I guess I'm uber-weird.

Doc says the bbs are fine as long as I'm tolerating them. My hyper episodes were so severe, especially when I was ill, that propranolol is a med I won't give up unless I really, really must. I'm terrified of that.

I also have a completely wacky immune system that overresponds to friendly stuff and allows bad stuff to just waltz right in and make itself comfortable. I have an infection somewhere random about once a month and spike very high fevers that exacerbate my pots symptoms.

The more I read on here the more I think there is something else going on as I'm not fitting any "model."

[Lizzegrl]

I see Dr. Grubb and Dr. Karabin and was diagnosed HyperA Pots. I have the flushing quite frequently and have talked to them about it and several other symptoms. Ironically, before I was diagnosed with POTS I was already on a med regimen for allergies/asthma that included pretty much everything that is recommended for mast cell disorders.

[issie]

I had also been taking the H1 and H2's before and thought they helped a little - but, it didn't seem to give complete relief or as much help as I thought they should. (Supposedly, the full benefit of Gastrocrom won't be felt until 6 months.)

Issie

[kitt]

Kitt, sorry to hear youve been feeling so lousy. I also have low BP. On BBs I have had my lowest recorded BPs- although Im normally pretty low. I have discontinued use of BBs due to side effects/fatigue low HR/possible hair loss. I have had two tilts- 1st one stopped at 5 mins second stopped at 9 mins. Since antihistamines are avail over the counter it wouldn't hurt to try- but usually for us we need more than the recommended OTC dose so that's why it's import to get tested and work w a doc who can recommend right dosage. As far as MCA- I think you are right on- think it is a much bigger player in POTS than at first glance.

I see the new Dx as good news because it helps to explain the widespread multi system dysfunction And it is something that is currently being studied.

Thank you Kelly, Am seeing my cardiologist today because of the extreme low BP on BB's. Attenolol 80/40...Metoprlol...70's/50's. So no more BB's I'm thinking! How low did your BP go on BB's? Anyone else have this experience?

I'm confused because most of the POTS MCA cases I've studied have high BP as a cofactor. I have low BP, but have flushing, vomiting, and other mast cell diseases. My immunologist wrote me a script for a tryptase test to check for mastocytosis. Has anyone else had a tryptase blood test done? (I haven't done it yet...)

Thank you for anyone who might be able to help by sharing their experiences with POTS, and low BP on BB's as well as flushing and vomiting.

Btw, I found a stat sheet done by Dr. Grubb that listed symptoms, and the percentage of POTS patients he's seen (many many thousands), and under vomiting, he only listed 8%. I have that list bookmarked, but am rushing to put together questions for my visit with the cardiologist today, and will post it later.

Best to All...

[kitt]

I have severe chest/facial flushing, probable MCAD with elevated tryptase, methylhistamines, prostaglandins and IgE. Also dxd hyperpots with significantly elevated plasma cats. I am on the Mayo mast cell protocol AND beta blockers, which work well for me, so I guess I'm uber-weird.

Doc says the bbs are fine as long as I'm tolerating them. My hyper episodes were so severe, especially when I was ill, that propranolol is a med I won't give up unless I really, really must. I'm terrified of that.

I also have a completely wacky immune system that overresponds to friendly stuff and allows bad stuff to just waltz right in and make itself comfortable. I have an infection somewhere random about once a month and spike very high fevers that exacerbate my pots symptoms.

The more I read on here the more I think there is something else going on as I'm not fitting any "model."

Interesting Potsie, you have an interesting case. Hyper and able to take BB's. Did Mayo explain why? Can you tell me why you feel Propranolol is such a lifesaver for you? Why you wouldn't want to give it up...May I ask what dosage you take?

Do you take H1 and H2 blockers for the MCA aspect, or was it ever suggested?

I've been to Mayo In MN. and FL. many times, and it's hard to argue with them when they make a diagnosis. Just curious if they explained why this works for you with MCA POTS.

Glad you're getting some relief!

In addition to low BP, BB's make me even more EXHAUSTED. Has anyone else experienced this?

Thank you again Potsie for your feedback.

Be well..

[issie]

There are a few who do have low bp's with HyperPOTS. Most of us DO NOT tolerate BB's - they will cause a degranulation of mast cells making that aspect of our illness worse. That alone will cause a drop in bp's when the mast cells release and then the problems compound. How do you know the bb's are not affecting you --if you have mast cells - it releases histamines and other chemicals and that in itself can make you feel awful. Unless you are in a flare and catch it one hour after the start of the flare - many won't have high Trypase levels. It's really hard to catch - cause most of us don't feel like running down to the hospital to get a blood draw or have a 24 hour urine - when we are so sick - we can barely think or sit up.

The H1 and H2's are very important and which kind you use. I didn't get benefit until I switched to Allegra and Pepcid (although I can only use 1/2 of pepcid or it makes my stomach hurt). But, when the GastroCrom was added that seems to be making a difference. I'm also using the Nasal Crom a couple times a day and have just added the aspirin once a day. I'm also doing some diet changes and using some supplements.

As far as I know the things that supress the sympathetic system and are used with MCAS are clonidine and gafacine. I couldn't use the gafacine but, a very, very small amount of clonidine - helped with the surges. I had to come off it though - because I started having edema really bad and got tired of feeling tired all the time. I will reserve it for if those horrible surges start back. It leveled out my bp and hr. It was nice for awhile. With me, I have to cycle things.

Hope this helps a little.

Issie

[kitt]

There are a few who do have low bp's with HyperPOTS. Most of us DO NOT tolerate BB's - they will cause a degranulation of mast cells making that aspect of our illness worse. That alone will cause a drop in bp's when the mast cells release and then the problems compound. How do you know the bb's are not affecting you --if you have mast cells - it releases histamines and other chemicals and that in itself can make you feel awful. Unless you are in a flare and catch it one hour after the start of the flare - many won't have high Trypase levels. It's really hard to catch - cause most of us don't feel like running down to the hospital to get a blood draw or have a 24 hour urine - when we are so sick - we can barely think or sit up.

The H1 and H2's are very important and which kind you use. I didn't get benefit until I switched to Allegra and Pepcid (although I can only use 1/2 of pepcid or it makes my stomach hurt). But, when the GastroCrom was added that seems to be making a lot of difference. I'm also using the Nasal Crom a couple times a day and have just added the aspirin once a day. I'm also doing some diet changes and using some supplements. But, not until I added the GastroCrom did I notice the improvements.

As far as I know the things that supress the sympathetic system and are used with MCAS are clonidine and gafacine. I couldn't use the gafacine but, a very, very small amount of clonidine - helped with the surges. I had to come off it though - because I started having edema really bad and got tired of feeling tired all the time. I will reserve it for if those horrible surges start back. It leveled out my bp and hr. It was nice for awhile. With me, I have to cycle things.

Hope this helps a little.

Issie

Thank you Issie,

Great post! You gave a lot of good information. I'm going to be referring back to it soon I think.

I thought I would have to walk the cardiologist I'm seeing through this today, and it was amazing. He got it. I started by telling him about the flushing and the vomiting, the other mast cell diseases I have, and showed him the script for a tryptase test the immunologist had ordered which I hadn't done yet.

Just as you said Issie..Running down to the hospital for another blood test. I'm just trying to keep up with the M.D. appts I already have.

Anyway, he said "We need to do a epinephrine and metanephrine level test, a cortisol level, and a plasma histimine tryptsase test." ...And we may have to do a 24 hour urine test.

Was amazed and impressed. Don't think he's ever treated a case of MCA Pots before, but he sure knows what to do.

I found a form with percentages that Dr. Grubb published, and on it it had all the different symptoms of MCA hyper POTS. (I promise to find it and cut and paste..Too tired right now, but will do it.)

On the form with all the symptoms of MCA hyper POTS...Things like vomiting, headache, difficulty sleeping....It came to high BP (forget the medical term) and ONLY 38% of MCA hyper POTS patients have high BP! Thought that was odd. I checked off all the symptoms I had in a bright felt tip pen, and he locked on that and put it in my file.

So, you're right, low BP POTS patients can be in the group.

Strange...At his office today his nurse took my BP and said, "that can't be right"...Let me try the other arm. I laughed and said, 'right it down' It will be right. It was 145 over 70. Crazy...So guess mine swings like a monkey in a tree.

The GastroCrom is interesting. I haven't read that much about it being used.

Who did you find to diagnose you Issie, or did I miss that?

Be well...

[Kellysavedbygrace]

Issie, that is awesome news! So glad to hear you are having some improvement w the GastroCrom. Look forward to hearing your report at the 6mo mark.

Kitt,

Lowest BP I had recorded on BBs was 57/31 - but that was an unusual one. Had lots of BPs in the 80/50 range on BBs when I was symptomatic- especially following exercise. Still get a few of those now and then but it seems not as much. Averaged more like 95/65 on BBs now average 100/68- not that much difference. The Bpms have definitely been higher and when symptomatic I can get really high ones but the symptoms associated w high HR are much easier to deal w than the low BPs. With high HR I just go to bed and eventually I recover from symptoms- often a low BP means I'm already in bed and no amount of salt and Gatorade are helping. When really bad I need ltrs of IV saline to help me recover.

NMPotsie,

The thing with MCAD is that when the mast cells degranulate they can release 200+ different mediators (chemicals, neurotransmitters, etc) and one MCAD patient may have an entirely different presentation than another because the Mast cells are mutated- therefore the chemicals that are causing havoc for me may be different than the ones causing havoc for you. This would also explain why Issie has improvement after 2 wks on GastroCrom and ChristyDs son did not have the same experience at 2 weeks. Yet both have MCAD. That is why the trial and error of different meds, combo of meds, supplements is so key. This may also explain why some have really bad flushing and some do not. Anyway, all that to say, don't give up, you are not alone. I don't quite fit the profile of others either.

[Kellysavedbygrace]

Issie, that is awesome news! So glad to hear you are having some improvement w the GastroCrom. Look forward to hearing your report at the 6mo mark.

Kitt,

Lowest BP I had recorded on BBs was 57/31 - but that was an unusual one. Had lots of BPs in the 80/50 range on BBs when I was symptomatic- especially following exercise. Still get a few of those now and then but it seems not as much. Averaged more like 95/65 on BBs now average 100/68- not that much difference. The Bpms have definitely been higher and when symptomatic I can get really high ones but the symptoms associated w high HR are much easier to deal w than the low BPs. With high HR I just go to bed and eventually I recover from symptoms- often a low BP means I'm already in bed and no amount of salt and Gatorade are helping. When really bad I need ltrs of IV saline to help me recover.

NMPotsie,

The thing with MCAD is that when the mast cells degranulate they can release 200+ different mediators (chemicals, neurotransmitters, etc) and one MCAD patient may have an entirely different presentation than another because the Mast cells are mutated- therefore the chemicals that are causing havoc for me may be different than the ones causing havoc for you. This would also explain why Issie has improvement after 2 wks on GastroCrom and ChristyDs son did not have the same experience at 2 weeks. Yet both have MCAD. That is why the trial and error of different meds, combo of meds, supplements is so key. This may also explain why some have really bad flushing and some do not. Anyway, all that to say, don't give up, you are not alone. I don't quite fit the profile of others either.

[NMPotsie]

I wish I knew how to do the quote thing! I need dinet training. I will try to get it right.

Kitt: The beta blockers help to control the dumps of norepinephrine so I don't get surges. I would randomly get these horrible episodes in which my bp would go incredibly high (ER got 180/160 once), my heart rhythm would go completely wackadoo, and I would be sick like death for a week; so nauseous I couldn't stand up without vomiting, cold chills, sweats, and extreme dizziness. Since I've been on the propanolol I haven't had a single severe surge (worse bp was 150/110 but I wasn't incapacitated for a week after). These things were so terrifying that I cannot imagine having to endure them again. I take 10 mg TID. If I don't take it every 6 hrs. in the day my tachy is terrible.

I have been put on h1, h2 blockers, gastrocrom, aspirin, dymista and ketotofin eye drops to control the mcas (lots of mast cells in your eyelids I was told; didn't know that, and if you rub them they release loads of histamine). I haven't been able to start the gastro because I'm fighting a MRSA infection, but hope to at the end ot his week. Now that I've been on the blockers I've noticed a significant drop in my bp. It's running low, and the neuro said when I go back in December he will put me on midodrine to augment the bp. He wants me to stay on the bb.

They didn't really explain why it worked for me, just that my particular kind of hyper needs something to control the adrenaline issue, and clonidine gave me severe rebound hypertension.

They even added xanax at night because my sympathetic nervous system is so overactive that I lay and twitch and you can see the twitching of my muscles. I had to videotape it because it was so weird.

Kelly: thanks for that information. It helps to know I'm not the only one who seems to have an odd profile. It's bad enough to have pots, which no one has heard of; worse to hear you're not even a typical potsy. The variety of chemicals make sense for why I'm not responding exactly like everyone else. The important thing is I have more good days than bad now, and for that I am soooo gratefull.

[issie]

I got DX'd at Mayo in AZ about 5 years ago. A Cardio doc - caught my POTS with a TTT. Then a few more visits got the EDS dx and the MCAS probable DX. Now there are more things with autoimmune and other issues that we are working on at the moment. I'm a DX work in progress. LOL! At least still getting answers as to WHY. Now just need to know the solution.

Glad I could help earlier.

Issie

[kitt]

Thanks Potsie, Issie, and Kelly,

Want to respond to your posts later. They're excellent!

Issie, am not surprised that you were dx. by Mayo. When they do give a dx., they are almost always correct. When they don't give a dx., it's because they're not willing to put themselves on the line unless they are 100% certain. Have experienced both situations at Mayo in MN, and Fl.

Potsie, did you say where you were dx? Forgive me if I missed it.

Great posts. Definitely have questions for both of you. Today's another day though, and I have another M.D. appt. Seems to never end. One appt. begets another, and on it goes.

I reread my post, and am mad at myself for making it sound so 'shocking' that my cardiologist is so brilliant. Jeez, this is what he has dedicated his life to. I've just read so many, many stories from people who haven't been able to get help with POTS, and I am so grateful to be able to get help in my town, although I do live in a big city. (Was initially dx with POTs out of town, but am not getting it narrowed down to a subtype, and getting treatment that will help. BB's have been a nightmare.)

I'll post questions to you both about your posts later, but for the moment though I found the form I mentioned that Dr. Grubb included in one of his writings. I'd promised to paste it for you.

I found this so helpful! As mentioned before, I printed it out, and notated which symptoms I have, and gave it to my cardiologist.

TABLE 2. Clinical Manifestation of Patients With POTS and MCA Disorder

Orthostatic Symptoms—n (%) MCA+POTS MCA+OH n % n % Flushing 8 100 5 100 Palpitations 8 100 5 100 Lightheadedness 8 100 3 60 Chronic fatigue 7 88 3 60 Headache 5 63 2 40 Dizziness 5 63 4 80 Presyncope/syncope episodes 3 38 1 20 Shortness of breath 3 38 2 40 Confusion 3 38 Increase in blood pressure 3 38 Paresthesias 3 38 Gastrointestinal symptoms (nausea and vomiting) 3 38 4 80 Abdominal cramps and diarrhea 3 38 3 60 Blurred vision 2 25 Anxiety 2 25 1 20 Excessive diuresis 5 100 Orthostatic intolerance exacerbated by—n (%) After exercise 5 63 5 100 Prolonged standing 3 38 1 20 After meals 3 38 1 20 Premenstrual 2 25 1 20 Heat intolerance 2 25 5 100 Emotional stress 1 13 1 20 Sexual intercourse 1 13

[kitt]

Didn't paste. Here it is.

TABLE 2. Clinical Manifestation of Patients With POTS and MCA Disorder

Orthostatic Symptoms—n (%) MCA+POTS MCA+OH n % n % Flushing 8 100 5 100 Palpitations 8 100 5 100 Lightheadedness 8 100 3 60 Chronic fatigue 7 88 3 60 Headache 5 63 2 40 Dizziness 5 63 4 80 Presyncope/syncope episodes 3 38 1 20 Shortness of breath 3 38 2 40 Confusion 3 38 Increase in blood pressure 3 38 Paresthesias 3 38 Gastrointestinal symptoms (nausea and vomiting) 3 38 4 80 Abdominal cramps and diarrhea 3 38 3 60 Blurred vision 2 25 Anxiety 2 25 1 20 Excessive diuresis 5 100 Orthostatic intolerance exacerbated by—n (%) After exercise 5 63 5 100 Prolonged standing 3 38 1 20 After meals 3 38 1 20 Premenstrual 2 25 1 20 Heat intolerance 2 25 5 100 Emotional stress 1 13 1 20 Sexual intercourse 1 13

[kitt]

Sorry...it will NOT paste. Here's the link. http://hyper.ahajournals.org/content/45/3/385/T2.expansion.html

[issie]

Thanks for the listing.

Issie

[NMPotsie]

kitt I was dxd at Mayo Scottsdale after a harrowing 2 years of thinking I was dying at least once a month. Everytime I have gone I have come back feeling better and doing better. My doctor, Dr. Goodman, is like Superman. I would give him my firstborn if he asked. LOL

[adigitalashley57]

I'm an Mcad-er with spikes of high blood pressure. They still feel pretty awful and strangely i still feel like I'm going to pass out. I should say though every study I've read says beta blockers are awful for Mast Cell. But frankly I've read a lot of studies that didn't hold true so I would take this information with a grain of salt.

Some of my biggest symptoms are shortness of breath and chronic hives as well as heat intolerance and severe severe reactions when i haven't had anything to eat.

I tried Gastrocrom for a week and it messed with my sleep a ton and gave me awful palpitations. The thing is I think its one of those medicines that make it worse before it gets better so maybe i didn't give it enough time... I also ended up getting MRSA/staph infection after experimenting with it so maybe all those Mast Cells protecting my body are actually doing me some good even thought they are making me feel like crap!!!

-ashley

[issie]

Really, there are way too many studies done on MCAS and too many people that have it - to discount that it has been found that beta's degranulate the mast cells. I would really keep that in mind - if what you are doing isn't working to help control the MCAS reactions.

Yes, GastroCrom - you get worse before you get better. You have to go slow and low and gradually build up. I was told it would be a good 6 months before I'd be able to completely tell what it would do. I intend to stay with it that long. Although, I can tell some benefits. I also do sense my reactions more. I've written about that before. But, I think that helps to sort out triggers better.

Issie

[kitt]

I I also ended up getting MRSA/staph infection after experimenting with it so maybe all those Mast Cells protecting my body are actually doing me some good even thought they are making me feel like crap!!! -ashley

Ashley, you are the 2nd person on this thread that has mentioned having a MRSA infection. Why is this associated with MCA POTS?

Had never heard of anyone with POTS getting a MRSA infection, and now 2 people. How or why do you think you got it, and how does someone who gets infected with this strain of staph keep from infecting others?

How are you doing with your treatment for MRSA? Is it responding to anti-biotics?

[kitt]

I got DX'd at Mayo in AZ about 5 years ago. A Cardio doc - caught my POTS with a TTT. Then a few more visits got the EDS dx and the MCAS probable DX. Now there are more things with autoimmune and other issues that we are working on at the moment. I'm a DX work in progress. LOL! At least still getting answers as to WHY. Now just need to know the solution. Glad I could help earlier. Issie

Thank Issie for you post. Am curious though. Am wondering why MCAS dx is probable. Are you waiting to have more testing done the next time you're at Mayo? My experience with Mayo is that they do a lot! of blood work, and testing.

Just curious what Mayo's 'take' is on it.

Thanks!

[issie]

Dr. Goodman, is treating many of us with the MCAS protocol now - this just started last month. It is so hard to get "concrete" scientific proof of the problem and most of us don't feel like jumping into a car and driving to an ER to sit there to wait to have a blood draw - when we are having a "spell". So, we don't have the concrete proof. Although, I do have an ER visit that indicates an issue with MCAS and my heart - over night stay and lots of nitroglycerin. So, doc's can't say for sure you have something unless they have the testing to back it up. So, that's why probable. I have had genetic testing that also indicates issues with Mast cell problems. So, with me it is nearly a given. And I am responding to the meds for it. So, another positive sign of that being an issue.

I think Dr. G is thinking that us with HyperPOTS and many of us with EDS - there seems to be a trilogy with MCAS attached to it. So, he is giving us a trial with the treatment. He has pretty much tried everything else and most of us have not responded - so why not try something else. Right? Hopefully, this will be the solution.

Issie

[kitt]

Thank you Issie, that helps.

I am scheduled to have blood tests for hyperadrenergic POTS. The order calls for epinephrine, and metanephrine. The lab order does not say the tests needs to be done standing, but I know it does.

Before I call the cardiologist to change the order and add 'standing', can anyone tell me if they had these blood tests and how they were done.

I know Thankful said she (on another thread that she had them done both standing and supine. Not sure if I need to have it done both ways and if so, is in done on two different trips to the lab?

If anyone has had these tests done, I'd be very appreciative on feedback!

Thank you...

[HyperPOTS8]

The way they do it at Mayo Rochester is place an IV (since the stress of a venipuncture can be enough to raise your catecholamine levels. They then have you lay in a dark, quiet room for 30 mins, draw the supine values there (norepinephrine, epinephrine, and dopamine), then have you stand/walk around for ten minutes and draw them again (sitting).

[kitt]

HyperPOTS, Thank you for this answer! Not sure I can get that done in town, (I've been to Mayo in Rochester and they are very precise in every thing the do.) Were you given special instruction about when and what to eat/not eat the day and night before?

I didn't know testing dopamine was part of the testing for Hyperadrenergic POTS? Any idea why?

My cardiologist also didn't order norapinephrine...Only for epinephrine, and metanephrine.

He also ordered a blood cortisol level.

Thanks again!

I'll call his nurse tomorrow and ask her to rewrite the order.

I'd be interesting in hearing how other people have had this testing done?

Thanks!