I am not a physician, and so don't take anything in this post as medical advice.  I am a person who has had symptoms of Hyperadenergic Postural Tachycardia Syndrome for as long as I can remember ( H POTS). What defines H POTS is the high levels of Norepinephrine (NE). Other forms of POTS do not have this finding. I also have symptoms of Mast Cell Disease and have elevated Tryptase. I have been taking medication (Clonidine) for H POTS for many years, and medicating for Mast Cell instability for just over two years. The paper connecting H POTS and Mast Cell Activation can be found here:  http://hyper.ahajournals.org/content/45/3/385.full

I am writing this post because... well, because there wasn't one that I could see...because we (those of us with H POTS) can present differently...and because we may have different test results. The whys of this is not yet understood as  far as I can tell. I keep watching, listening, and reading for information on this, but so far have come up empty handed. Instead of the low Blood Pressure most medical professionals are familiar with, we may well have high Blood Pressure. Instead of diarrhea, we may have constipation, and on and on.

Someone suggested that the high NE may be due to us having high Histamine levels, as Ne helps control Histamine. (If you read this, and recognize yourself, I am most definitely NOT picking on you. Your Posts have helped me a great deal over the past two years.) I understand this reasoning, but I do not have high Histamine levels.  

Another person wrote to me about False Negative Test Results. I do understand about both False Negative and False Positive results, and hope others do as well. What convinced me my Histamine Test results were accurate, however, is my own reaction to Antihistamines. I was not able to take the Zertec and Zantac combo that many recommend, although I did take Claritin and Pepcid for some time before recently stopping both. I do, however, continue with Clonidine, Cromlyn Sodium, Hydroxyzine (for sleep only), and have recently started Aspirin at a higher than usual dose.

My point here, is that we may well have different symptoms, and we need to medicate our own symptoms. This difference is not unusual if you think about it. Systemic Mastocytosis will cause different symptoms than  TMEP for example.

Know yourself. Keep a log, if that helps. List symptoms, when you start a certain med, and how you react. If something doesn't help or makes you feel worse, stop taking it! And look for something else that may help. Do realize it may take awhile to realize some results from some meds, though.  

I hope this post helps someone.

Gail

This Topic was moved here from Specific Mast Cell Conditions and Those that Mimic Them by DeborahW, Founder.

Gail,

  That's an interesting article about H. POTS.  I have a few additions and thoughts about your posts.

  Levels of mediators, including histamine, can bounce around a lot for a variety of reasons.  If a person isn't producing too much or one's mast cells aren't releasing too much histamine, e.g., that person could be ingesting it from foods that are high in histamine.  Almost any food can contain a high level of histamine, as it's a product of deterioration, although certain foods more commonly contain large amounts, even when fresh.  Other foods cause direct histamine release, so that would also temporarily raise a histamine level and potentially cause symptoms.  

  If histamine isn't the cause of symptoms, then it's possible there isn't a MC disorder involved in the illness, or the person might be super-sensitive to small amounts, as others have said.  Even if histamine isn't an issue, there are a number of vasodilators that can produce flushing and other symptoms similar to a MCA disorder, as well as other diseases.

I would not go off my H1 antihistamines, as I believe they help me feel better, even though my histamine level has been low, and I have been diagnosed with SM.  Some antihistamines, such as desloratidine (Clarinex), also have a stabilizing effect on mast cells, so help reduce  degranulation and release of a number of mediators, as stated in this abstract.  Desloratidine specifically stabilizes mast cells in the skin:

[url]http://www.ncbi.nlm.nih.gov/pubmed/19826448/url]

Regarding diagnosis of Pheochromocytoma, this article seems to be more detailed and more current (2012) than the article you cited.  If it doesn't answer your specific question, re-post what it is you want to know.

http://ssl.texmed.org/virtual/cme/PDF/12DIA_30.pdf

It's not, though, written specifically for a differential diagnosis of H. POTS or MCAD.  Still it's always surprising how testing changes in such a short time!  This article includes a list of meds that should be discontinued for a period of time before testing, including clonidine.

To be clear, pheochromocytoma is one of several diseases that need to be ruled out before a MCAD is ruled in.  Symptoms such as flushing, tachycardia, and others are found in several diseases, as well as in reactions to high histamine foods.  In some diseases, antihistamines are helpful, so testing must be used to differentiate among the possibilities.

Gail, thanks for putting in the original references. That's helpful when people are trying to put questions into perspective.  Often we have to go back to the research someone is reading to figure out a question or answer.

While we all have somewhat different symptoms, and those don't necessarily reflect the seriousness of the illness, my opinion is that medicating should be done in conjunction with a mast cell expert.  They are knowledgeable about testing and possible differential diagnoses.  They have a great deal more experience in prescribing and adjusting meds to fit the individual's symptoms and type of MC disorder.  They also are aware of interactions of meds, co-existing conditions that can interfere with treatment, and other facts that will help to keep patients safer and more comfortable.  For example, using epinephrine to control a carcinoid syndrome crisis can kill the patient, yet it can be life-saving for a MC patient.

Finally, it's not uncommon for MC patients to have variable BP depending on symptoms.  Mine is routinely low (90-100/60).  Yet, on at least 2 occasions, during anaphylactic episodes, my BP went very high.  This presents a dilemma for ED doctors when we come in with high BP, no breathing difficulty, and ask for epinephrine.  That's one reason why guidelines for treatment and a letter from a doctor can be helpful.  Epi is what brought my BP down and stopped the anaphylaxis for me very quickly.  Before I was diagnosed, I would wake up every morning with 80/50 BP.  A combination of meds, hydration, etc. helped raise my a.m. BP.

If you haven't already done so, I would recommend seeing a mast cell disorder specialist for a full work-up and diagnosis.

Some of my test results:

Norepinephrine: normal range 0-600..........1001 pg/ml

N-Methylhistamine: normal range 30-200.....100 mcg/g Cr

Creatinine Concentration:  25 mg/dl

Tryptase: normal range 1.0-11.5 ..... 35.0 and 32.9 mcg/L

Gail

Added: The two Tryptase are essentially baselines as I was not having any major symptoms at the time of either test.

I started this thread, in part, because although folks seem to get "We all present differently", they still seem to have questions about whether or not I have a Mast Cell disorder. I also hope what I have posted will clarify why I think as I do and help others with H POTS.

If POTS is caused by Mast Cell activation, how can the high NE be explained? There may be an answer somewhere, but I haven't seen it. And yes, I do have questions about Histamine. In light of my test results, my levels are a bit of a puzzle. I really do like puzzles, but not of this sort!

I don't really have any direct questions; just wanted to share some research and thinking.

Deborah, thanks for the platform.

Gail

Gail, nice post!   I think you have a lot of really valid things to say.  

If you don't mind I'd like to add a bit to it.  


When we talk about hypertension we are talking about various things which can be sending it up - cardiac issues, renal issues, vascular problems, diabetes, and a great many other things.   There are tumors, as you mentioned as well as other things which can push the pressure up.  I think that after the average exam by a good cardio looking at the usual stuff and ruling it out, then that´s when you begin looking at the unusual stuff and this is where POTs and MC Activation comes into play.  

The connection has been made between Hyperadrenergic POTs and MCA, so I don´t think it takes much to take it one step further that masto and HyperPOTs is proven for MC activation is masto.   You see, the problem that the doctors were facing when this article was published is that they were force to question what the disease pathology was behind it and that´s because the WHO refused to recognize anything but the criteria for SM as masto!   All of this has now totally changed and when there is any kind of proven MC mediators which are known to come from the MCs there is no way to consider anything else.  

But the problem we are now facing is that MCA is a diagnosis of exclusion and that´s because according to research it may be that the patient is not far enough along in their disease state - it´s not progressed enough, or it may depend upon the form the patient is showing, or other issues.    The researchers still don´t understand why some of us will show elevated histamines, others elevated prostaglandins and others elevated tryptase and yet others none of these and yet other MC mediators like heparin.   There is so much more they don´t know now than they thought they didn´t know before!!!   There´s so many pieces to these puzzles that it´s driving them mad!   So this is why some of these articles on POTs don´t make the full connection, because they were published prior to 2010 when they could be allowed to make the connection with masto!!

There is a really terrific article published recently by Drs. Horny, Solter and Valant that helps to differentiate between it all!   Evaluation of mast cell activation syndromes: impact of pathology and immunohistology.
Horny HP, Sotlar K, Valent P.   -   http://www.ncbi.nlm.nih.gov/pubmed/22555026


Another problem we face is that there haven´t been any real studies on the hypertensive masto patients.   I´ve hunted high and low to find some kind of information which shows a study and have come up empty handed.  There are a couple of articles which will cite case histories, but the only article I´ve found which specifically mentions this phenomenon is by Drs. Roberts and Oates back in the early 90s.  This is where they mention that there can be a hypertensive variant and they hypothesize as to what could be behind it.
Biochemical diagnosis of systemic mast cell disorders.
Roberts LJ 2nd, Oates JA.  -   http://www.ncbi.nlm.nih.gov/pubmed/16799604


I have really, really, really had to deal with this issue with my doctors since none of them knew this existed in masto.   My masto specialist has had to learn with me and this is what made me press to find research to help teach them what happens here.   I´ve spoken with several high authorities in masto regarding this and have gotten some excellent replies as to what can happen and yes, for some there may be a hyperadrenergic response going on due to the chemical chain reaction which all of the MC mediator release creates.  There can also be a metabolite of prostaglandins which is produced and this too can push the pressure up.   But again, getting down to tracing what is going on is the issue.  

I´m finally going to get my pots investigated!!  It´s a round about means of finding out what is going on with the syncope.  I´ve got some neurologists in Rio who are taking an interest in my case and they are going to start with the area of dysautonomy and then we´ll progress from there.  I´m sure they are going to see the contradictions that my masto is causing, but having said that, we´ve ruled out EVERYTHING POSSIBLE and these things we know beyond a doubt are connected to my masto.

So, in saying this, having ruled everything out, if masto remains then those findings that you get are thrown at masto.   This is how the research is going today and the researchers are having to update their thoughts now that the new MCAS diagnosis is out.   All of the older articles which tried to find answers but could not blame masto due to the extremely restrictive SM diagnosis.  The old research needs to be updated and balanced in light of the new MCAS diagnosis and this will help the doctors find the connections of things like IC and POTs and other issues with mast cell disorders.


Lisa

Lisa:
I´ve spoken with several high authorities in masto regarding this and have gotten some excellent replies as to what can happen and yes, for some there may be a hyperadrenergic response going on due to the chemical chain reaction which all of the MC mediator release creates.

Lisa, I would really like to see something published on this, and until that happens, I am holding out. I have read and read, and can't put NE into the equation. Why would my NE be perfectly normal, then way overshoot when I stand? Something is just not adding up.

I do understand that we may release different mediators, but it seems that, in general, people do not believe Histamine would be on that selective release list.

Glad you are being evaluated for POTS. Make sure they test correctly for NE!

I would absolutely agree that research, papers and general knowledge is lacking. Wish they would all hurry up!

Wait a minute! we are getting "cause" and "associated" mixed up. You probably know I have not accepted that Mast Cells have caused my POTS, but I do know that they are associated. Couldn't seem to be any other way at this point. But as far as I am concerned, the puzzle is missing some pieces.  

Point taken, but yes, my Dr knew about testing for other things. Glad he did, because at that point, I didn't! Something else to think about here... I am not a youngster, and I have had symptoms for as long as I can remember. Either of the other two ... Carcinoid or NET... would have probably done me in by now.

Thanks for the encouragement. I need it sometimes.

Gail