Mast Cell Activation

[EarthMother]

As I read more from POTS people with Mast Cell Activation Syndrome and recently read the Vanderbilt study on Hyperadrenic POTS and MCA ... I realized I have some (not all) of the symptoms of MCA and was wondering how to procede with my doctor about testing.

So I had a few questions for those here who are in the know

1. What type of Doctor can diagnose Mast Cell Activation? (i.e. Can my primary phycian request and interrpret the tests?)

2. Have the treatments for MCA, like anti-histamines helped your POTS or just the MCA flare ups?

3. What were your MCA SYMPTOMS? (e.g. I get acte episodes of facial heat, burning etc. but not always do I turn red. What else should I look for to warrant going down this road for testing.)

Anything else?

Thanks so much for thinking about this with me!

[juliegee]

Hey EM-

1. I wish I could say that any allergist could DX you, but that's simply not the case. You need to find a masto specialist.

2. The treatment for MCA has definately improved my POTS/NCS. In an allergic reaction, your blood vessels leak out causing low BP, high HR- especially with postural changes.

3. The thing that caused me to realize my symptoms were mast cell activated were hives. I had the flushing you describe AND periodic episodes of systemic hives. (Not all MCA patients have dramatic skin involvement.) That was just the tipping point for me. I also had very low BP, very high HR, trouble swallowing, chest tightness, severe acid reflux, bone pain, "D", cognitive impairment, etc. I rarely have all of these symptoms at the same time, rather- all of them intermittently over a period of time. I had several episodes that many here call "potsy" episodes where I used my epi-pen and ALL symptoms subsided.

I had tons of skin and RAST testing. I was officially allergic to nothing, yet I continued to have dramatic allergy-type symptoms. (some MCA patients also have real allergies.) That coupled with the fact that I responded so well to MCA treatment: ranitidine, zyrtec, singulair, atarax, etc. led me to seek out an official DX. Also, all of my symptoms return with a vengeance if I skip my meds.

Most important for me is the idea that I can't get "too"- too happy, too sad, too stressed out, too energetic, too tired, too hungry, etc. That will set off an episode. Strong chemically smells will also tip me off. Lifestyle changes are as important as a good med regimen.

I joined a few Masto listserves to chat with others, compare symptoms, read reasearch, find doctors, etc. That was most helpful in figuring this out.

I wish you all the best. Please keep asking questions!

Julie

[firewatcher]

EM,

A serum tryptase level can be done to check for Mastocytosis, and should be done anyway if you think you might have MCAD. Dr. Biaggioni at Vandy recommended that I have urine methylhistamine check done after an episode of flushing. Apparently there are certain POTS markers (BP responses) that are exclusive for MCAD and hyperadrenergic POTS:

Orthostatic hypertension has also been associated with a number of conditions involving some form of autonomic dysfunction. In a recent study of patients with POTS in the context of disordered mast-cell activation, 38% (3/8) were shown to have orthostatic hypertension.19 Interestingly, in this group of patients, the orthostatic hypertension manifested as either a persistent hypertensive response to assumption of an upright posture or as a hypertensive crisis, with BP as high as 240/140, with upright posture. This has not been observed in patients who have POTS but do not have mast-cell activation disorder. Patients with baroreflex failure may also occasionally experience episodes of orthostatic hypertension.26, 27, 28, 29 These episodes are especially likely to occur in patients presenting with acute baroreflex failure. In the initial days and weeks after onset such patients have orthostatic hypertension immediately on standing, though it usually declines somewhat over the following few minutes. It is not a universal manifestation of baroreflex failure, which is more typically characterized by labile blood pressure and heart rate which track together. In a final phase of baroreflex failure, usually months to years after onset, orthostatic hypotension is more typically the dominant hemodynamic expression of the disorder. In the rare syndrome of norepinephrine transporter deficiency, an increase in blood pressure with upright posture can also be seen.30

Orthostatic hypertension: when pressor reflexes overcompensate

Joshua Fessel and David Robertson*

http://www.nature.com/ncpneph/journal/v2/n...cpneph0228.html

This is the only reference that I can find of Orthostatic Hypertension being exclusive as an indicator of MCAD, but Dr. Biaggioni seemed fairly certain. My allergist is looking into this for me, since I have huge reactions to allergy shots and skin tests. He is the head of his practice group and I think he sees me as a challenge, the average allergist will look at you like you are nuts. I have or have had flushing, hives, itching, throat tightening, mucusy diarrhea, wheezing and strong reactions to smells and all the stuff I'm allergic to.

I feel a lot better on antihistamines, and they do not sedate me like most people. One thing to remember is that all antihistamines, except Allegra, WILL WORK ON THE CNS!!!! That is how they do their job, they block histamine at the nerve, so start very carefully.

Good luck!

Jennifer

[juliegee]

Very interesting research citations, Jennifer. Thanks for sharing this! I know the doc at Vanderbilt have done the most work connecting MCA & autonomic issues. Based on that, it seems like all hypoadrenogenic potsies should look into MCA. Interestingly, I also experience little or no sedation with antihistamines...even atarx. It actually makes me a little hyper.

Your comment re. antihistamines working on the CNS is interesting. I think zyrtec, like allegra, is also an exception. Mast cells are connective tissue cells that work in the CNS so the relationship between mast cells and the autonomic nervous system is quite intertwined. There is so much here to be explored.

Julie

[EarthMother]

Thank you so much for the info.

Noob questions ... can I have the blood tryptase levels checked by my primary physician, i.e. will he know what I am asking for? And can he also write the order for the urine methylhistamine catch? I'm concerned I will be unable to find a "knowledgeable" in my local area and I'm simply not well enough to travel to another county.

[Tammy]

I've never been checked for Mast Cell, however I have the Hyperadrenegic version of POTS. but as stated "Most important for me is the idea that I can't get "too"- too happy, too sad, too stressed out, too energetic, too tired, too hungry, etc. That will set off an episode. Strong chemically smells will also tip me off. " I have this exact problem! Now I'm wondering if I too should be checked on this? However, I do not have any skin issues, hives, skin reddening, etc. So I'm not sure what to think? Interesting though!

[juliegee]

Yes, my allergist still has me carry orders for serum tryptase/blood and methylhistamine and prostaglandin-D2/urine in my wallet so that I can have them checked after an episode. Your PCP can easily order these tests. They are best done immediately after (w/i an hour or so of) an episode of extreme symptoms/flushing/anaphylaxis. The problem I have is that I'm pretty wiped out after an episode. I usually fall asleep. I couldn't imagine driving into the lab to get tested when I feel that bad...

If you can get these tests done and they show any high levels; you probably should look further into MCA.

Julie

[firewatcher]

Earth Mother,

You can get your serum tryptase levels done at any time. Your methylhistamine catch needs to be done after an "episode." Have your doc get you a urine bucket and keep it at home with you. After the next "episode" urinate and flush it, then catch the next four hours of urine. That is what needs to be tested. Have your doc right the order so it can go to the lab immediately with the catch. THis is how Vandy explained it to me and it is in my written directions for aftercare.

Not all hyperadrenergic POTS patients have MCAD, but all MCAD POTS patients have the hyperadrenergic form of POTS; that was my understanding.

See, clear as mud!

[MightyMouse]

E.M. Thanks for asking--I was wondering too. Personally, I have pretty much every symptom on the list...and for the past several years I've been on a constant regimen of zyrtec, benedryl, singulair and nexium...and when with hives, also add zantac. If I were to get tested, would i need to go off of the histamine-active meds?

Nina

[mkoven]

Initially all my ans symptoms seemed allergy-related. I would get lightheaded, rashy and itchy all at once. My summer of the allergic meltdown, 2006, I seemed to react to EVERYTHING. I test negative for mastocytosis, but I suppose mcad is a possibility. I take allegra and singulair all the time, and benadryl as needed. I do have allergies, with a documented IGE response, though. But my ans stuff and allergies feel linked.

[iheartcats]

I'm very curious about this, too - I have horrid allergies and even get hives taking a shower. Just with water! I'm sensitive to foods, have trouble swallowing sometimes, and have eczema.

I am trying to figure out how to approach this with my PCP - the tests sound so complicated, but she's good with helping me figure things out.

I have an appointment with an allergist for a full test and I have to go off my Allegra for 3 days - is this likely to make my POTS horrible? Could this be a 'bad' thing to do (I need to find out if I'm allergic to nuts/wheat/etc)?

Can I bring up MCAD with the allergist? Will they have an idea of what it is?

Thanks for your help - I think we all have a lot of questions.

[mkoven]

I have to say that I saw one of the top allergists in the country in Chicago and MCAD was not a diagnosis on the table. She was not about to link ans and my weird allergic reactions. When I later was in the hospital for my catheterization, I had a consult with one of her fellows to go over how I should premedicate, given my allergy to contrast agents. He said it was indeed confusing that my ans symptoms sound anaphylactic--but he didn't link them. About the hives in the shower-- I also have dermatographism--an entitiy allergists recognized. Pressure, or a light scratch will give me welts--so I produce hives and histamines without an external allergen. That seems like some sort of ans-allergy link? This seems "cutting edge"--this connection. I have read somewhere on this site that ans docs recommend keeping allergies under control, as they worsen pots. And allergic reactions involve vasodilation.

I also have both vasomotor rhinitis (autonomic) and allergic rhinitis--gotta be a connection in there somewhere!

At this point, aggressively treating my allergies does not eliminate my ans issues. But leaving them untreated would probably worsen. And I go through periods where I'm randomly itchy with no clear external precipitant.

[firewatcher]

I was not on an antihistamine at the time of my testing, so I don't know if you need to come off for the tryptase check. For the methylhistamine urine check, it should not be necessary because it will be after an "episode."

As for this being "cutting edge," sorry, they've know about it for YEARS! They just don't understand histamine that well and it's role in the brain and nervous system. With anaphylaxis, histamine is release throughout the entire body and it is a super-potent vasodilator and causes a sudden, extreme drop in blood pressure (gee, that sounds familiar ) Histamine also acts as a neurotransmitter and that is the part nobody completely understands.

(look it up on Wikipedia: histamine neurotransmitter---http://en.wikipedia.org/wiki/Histamine)

With my "crash" last year, one of my docs thought of anaphylaxis since high, constant doses of benadryl managed many of my problems. I thought so too and began looking into histamine. I found that the sites that were the richest in histamine were the ones I'd had the most problems with (gastrointestinal tract, gallbladder, breasts, lungs...) Histamine (in the brain) also acts to regulate the sleep/wake cycle, that's why MOST people get sleepy on antihistamines. There is an alternative medicine site (orthomolecular nutrition) that classifies people and certain mental disorders by histamine reaction (histadelia/histapenia) and it touches on Ramakentesh's theory of methylation.

It is all just one big knot and the doctors are attempting to tease out a few strands and unravel the whole mess and this is as far as they've gotten so far.

This is the article that I gave my allergist, it should be good for any doctor who knows your condition:

http://hyper.ahajournals.org/cgi/content/abstract/45/3/385

I don't think that every hyperadrenergic POTS patient will have MCAD, but that doesn't mean that it's "working the right way." It is something to be aware of and have looked into, perhaps adding an antihistamine would help gain more control over all this.(?)

[EarthMother]

I also looked in to histamines a while back ... read bits and pieces of a book by Pfeiffer on Nutrition and Mental Illness (an orthomolecular approach to balancing body chemistry). I didn't get much out of the book because it seemed to be all based on Schizophrenics ... but the link between illness and histamines was on every page! I also had come across an article about mast cell and multiple chemical sensitivity that talks about how histamines impact the amygdala (fight/flight portion of our brain) and explained how/why there is an emotional component to the sensory inputs.

So if I understand the two tests ... one would expect a negative result on the serum tryptase level which would rule out what we might consider a more serious condition of Mastocytosis. But perhaps a positive urine methylhistamine taken after a flare which would lend itself to believe a POTS and MCAD combination. Is that correct?

I plan to call my PCP next week to set up an appointment and I want to make sure I know what it is that I am going to discuss with him.

Thanks for much for all the advice and input on this thread. Its a big help.

[firewatcher]

I also looked in to histamines a while back ... read bits and pieces of a book by Pfeiffer on Nutrition and Mental Illness (an orthomolecular approach to balancing body chemistry). I didn't get much out of the book because it seemed to be all based on Schizophrenics ... but the link between illness and histamines was on every page! I also had come across an article about mast cell and multiple chemical sensitivity that talks about how histamines impact the amygdala (fight/flight portion of our brain) and explained how/why there is an emotional component to the sensory inputs.

So if I understand the two tests ... one would expect a negative result on the serum tryptase level which would rule out what we might consider a more serious condition of Mastocytosis. But perhaps a positive urine methylhistamine taken after a flare which would lend itself to believe a POTS and MCAD combination. Is that correct?

I plan to call my PCP next week to set up an appointment and I want to make sure I know what it is that I am going to discuss with him.

Thanks for much for all the advice and input on this thread. Its a big help.

Yes, the serum tryptase should be low, if it is high, it will be Mastocytosis (which would be even rarer than POTS!) If the methylhistamine levels are high, it means that you had a histamine dump and would indicate MCAD (Julie/Mack's Mom, is this right?) I have not had the methylhistamine done yet, so I'm not sure. What I got off the Mastocytosis site is that MCAD in POTS is not too many mast cells, just an inappropriate overreaction to a stimulus: exercise, emotion, posture change...

Good luck and take that article with you! Also, make sure the lab tech knows the proper procedure for processing the lab test (make them look it up!) I had a whole 24 hour urine catch wasted from improper lab handling!

[iheartcats]

To verify, as I am calling my doctor, too...what is the exact test I ask for (the 24-hour urine dump)? Is that the main test to do first? Any blood tests?

[firewatcher]

To verify, as I am calling my doctor, too...what is the exact test I ask for (the 24-hour urine dump)? Is that the main test to do first? Any blood tests?

One is a serum tryptase (for mastocytosis)

One is a four hour urine collection for methylhistamines (must be after an "episode" or it will be normal!) for MCAD

[flop]

Just a quick note on dermographism that was mentioned earlier in the thread. It directly translates to mean skin writing. Basically if you scratch the skin (eg with a match stick / lid off a biro) the skin flares up in itchy red weals that don't rapidly fade (it is normal to get a red mark, it is not normal for the mark to be swollen / raised up and last 10 mins or more). This weird skin problem happens because the pressure to the skin causes histamine release in susceptible people. When I am not on antihistamines I can write my name on my inner arm with my finger nail and it will be bright red for ages afterwards (I used to do this as a kid to show off - no idea why as it itches!!).

I remember reading something by Dr Grubb that said that impeccable allergy control was a vital part of treating dysautonomia.

Sedating vs non-sedating antihistamines, in general older antihistamines cause drowsiness but the more modern ones don't cross the blood-brain barrier so don't cause the same side-effects (this has nothing to do with some people not being drowsy on older meds, I can't explain that).

Sorry that is all a bit jumbled, just a few different thoughts that I cam up with whilst reading the whole thread.

Flop

PS - just found a good picture of dermographism on wikipedia

[EarthMother]

I can write my name on my inner arm with my finger nail and it will be bright red for ages afterwards (I used to do this as a kid to show off - no idea why as it itches!!).

That's so strange! I use to do the same thing as a kid. Hadn't thought about that in decades. I thought that was normal for everyone to get raised bumps from scratches.

I've always had what I considered over-reactions from minor scratches. Goodness if the cat presses too hard on my lap as she is jumping off I get welts for hours.

[iheartcats]

To verify, as I am calling my doctor, too...what is the exact test I ask for (the 24-hour urine dump)? Is that the main test to do first? Any blood tests?

One is a serum tryptase (for mastocytosis)

One is a four hour urine collection for methylhistamines (must be after an "episode" or it will be normal!) for MCAD

Thank you - very helpful!

[iheartcats]

Just a quick note on dermographism that was mentioned earlier in the thread. It directly translates to mean skin writing. Basically if you scratch the skin (eg with a match stick / lid off a biro) the skin flares up in itchy red weals that don't rapidly fade (it is normal to get a red mark, it is not normal for the mark to be swollen / raised up and last 10 mins or more). This weird skin problem happens because the pressure to the skin causes histamine release in susceptible people. When I am not on antihistamines I can write my name on my inner arm with my finger nail and it will be bright red for ages afterwards (I used to do this as a kid to show off - no idea why as it itches!!).

I remember reading something by Dr Grubb that said that impeccable allergy control was a vital part of treating dysautonomia.

Sedating vs non-sedating antihistamines, in general older antihistamines cause drowsiness but the more modern ones don't cross the blood-brain barrier so don't cause the same side-effects (this has nothing to do with some people not being drowsy on older meds, I can't explain that).

Sorry that is all a bit jumbled, just a few different thoughts that I cam up with whilst reading the whole thread.

Flop

PS - just found a good picture of dermographism on wikipedia

I always get hives after shower/heat...I've looked this up and it's somewhat 'normal' in the grand scheme of things (some people have this reaction, not much you can do, etc).

I also always turn bright red after scratching/touching (I am fair skinned)...but I don't think I welt like that. I just 'scratched' my arm to see...it's rather red, but I don't see welts. I do hive easily, though.

I've always had horrid allergies and it may just be horrid allergies rather than MCAD. But I'd like to at least 'rule it out.'

Thanks for all this help.

[juliegee]

I'm no expert on MCAD/mastocytosis, but have learned a fair amount through my research. I had a few comments that might be helpful. Mastocytosis occurs when you have too many mast cells. MCAD occurs when you have a normal number of mast cells, but they are too reactive. The symptoms and treatment for both diseases is the same:

Serum tryptase taken anytime will positively DX mastocytosis. A high reading is an indicator that you may have mastocytosis and should see a masto expert immediately. It is also helpful to have your serum tryptase checked within an hour or two of an anaphylactic/flushing episode. A high level at that time will verify anaphylaxis.

Nina, no you don't have to go off of antihistamines when you get your methylhistamines checked via 24 (not 4) hour urine catch. Antihistamines supress your reaction to histamine, but not the histamine itself.

Cat Lady, Yes, by all means bring up MCAD with your allergist. You may get a blank stare. All allergists know about mastocytosis, but, as Jennifer said on some level they know about MCAD, but most were not trained to recognize it and may not acknowledge it's existence. The diagnostic criteria is in it's infancy and there are currently no agreed upon laboratory markers to DX. Serum tryptase, methylhistamine, and prostaglandin D-2 is what my masto specialist checked. When our Linda Joy was recently DXed, she didn't get any new lab results, she just took her previous test results'history to a masto specialist who "gets" this.

Dermatagraphism is another symptom of MCA.

Yes. yes, yes to everything Jennifer/Firewatcher was saying about mast cells being neurally transmitted. The relationship between mast cells and the central/autonomic nervous system is inextricable. Good doctors like Dr. Grubb and those at Vanderbilt are trying to untangle the relationship.

I was lucky enough to have a local allergist who formerly worked for Mayo/MN under Dr. Joseph Butterfield. That faction doesn't formerly acknowledge MCAD, but despite that, my allergist has treated me for years w/o a formal DX. He seemed almost relieved (if not downright giddy) with Dr. Castells formal MCAD DX from Brigham and Women's.

Please look at the Mastocytosis Society Website for more information. They can also link you to masto listserves where I learned alot: www.tmsforacure.org

Keep researching & asking questions. No one cares more about your health and getting you better than YOU do.

Julie

[carinara]

I'am just wondering if the MCA can change from time to time. Whenever i get in a real bad POTS hole that lasts for months and my body gets totally out of control, i do experience some symptoms that are listed under MCAD like shortness of breath, different smells send me into attacks, have to urinate up to 6 times an hour, my face feels swollen and hot and so on.... In during them times i do experience my worst attacks and my body seems to either be in a state of a attack or needs to sleep to wake up to another attack. After a while though, my body seems to get more in balance again and all the before mentioned symptoms disapear. Just to return a few years later again. That hapened already 4 times in the past 10 years. Iam now wondering if it's possible to get MCAD just sometimes and not all the time. Because most of the time i seem to manage my PoTS symptoms but like i said something strange happens after a while and my body goes crazy and i cant do anything about it. After reading this post, i wrote an email to my POTS Doctor yesterday. He said that he will discuss it with some expert but he thought it would be a good idea to get some blood if ever i return in such a bad health stadium and then go from there. What do you think? is this possible?

carinara

[juliegee]

Yes, Carinara, I think it would be a good idea to do testing when you are doing very badly. That's when MCA will show up in laboratory markers. My MCA has developed over a period of years, starting in 2003. I would go on "heavy duty" MCA meds for long periods, then gradually taper off of them- only to have another awful attack, etc. I am now on permanent maintenance meds and am doing great. I haven't been hospitalized for almost a year. (Knock on wood!)

Julie

[carinara]

Julie, thanks a lot. Thats very interesting. I will definately get checked if such a bad episode appears again. At least i know now, that there might be something else involved and that there is a way to check it out. When these episodes happen i feel so helpless. Now, i feel that there might be something to help me manage these times better by looking into MCAD.

I havent found any articles about in in german yet. Can anybody tell me if MCAD is a serious condition? Can it seriously harm us?

carinara