Mcad/mastocytosis Attack Happening Now And Have Question

[Clairefmartin]

Hi all,

My throat is currently "tight" feeling as I just had what I think was another MCAD/Masto attack. I'm currently being tested for both, don't know what i have , but the attack I just had scared the heck out of me.

I think it was set off by touching an air filter - things like that and insulation have always irritated my skin, but nothing like this! I have only eaten the same old things I always eat today that don't bother me.

I wheeled back to my room after touching th filter (have infant I care for in bed), and my ear went nuts itching and going numb, half my face then followed suite, and then my tongue started itching and my throat started feeling swollen. Of course I panicked, I am alone with a baby with no local family. All my friends and my husband work. My husband has to be pulled from his classroom by an administrator if I call in a panick, so I try to wait things out before calling - he has missed a lot of work because of me. I called my home health PT, she said call the allergist - so I spoke with a PA who wanted me to go to the ER. She said with mast cell issues, the anaphylaxis can last a long time. I take klonopin, so I don't take benadryl (have to care for baby AWAKE) and antihystamines make me feel tachy and anxious, so I dont even have any here. So she said go to the ER so they can monitor my throat, and that they were just starting to get my bloodwork back, and that my general IgE levels were high, so they will give me an epipen. No other specifics at this point, I just had the bloodwork done. As you can tell, I am still at home trying to wait it out and not in the ER. Its been about an hour, and my throat is still tight, I am burping as well which is weird (I took Zantac earlier) and that started when my throat started closing, and I do have a hiatal hernia which I think can make your throat feel tight as well. I took more Klonopin to calm down, this episode really scared me.

Anyways, the weird thing was that I usually react on my right side of my face, but this all happened on the left - wondering if I touched my skin on that side after touching the air filter. Does anyone have any thoughts on this?

Also - I read a post where poeple were talking about your tryptase levels and getting a diagnosis- my allergist said they have to be above 20 for mastocytosis, wasn't sure what the criteria is for MCAD. I remember a few of you debating the tryptase thing as well, was wondering if there is any literature on it - I have only found the one article on the link between hyperpots and mcad.

Thanks! If it gets worse I'm going to the ER. Hopefully it won't.

[anaphylaxing]

Sorry to hear! I would go to the ER if you don't have any antihistamines on hand! Do you have an epipen?

HAve you had a baseline tryptase? Always worth drawing it during an acute reaction. Yes there is debate about what a totally normal tryptase is. You are right about the level for mastocytosis, but some say about 0.99 is indicative of MCAS; depends who you ask.

I definitely get reactions like you are describing and it seems no two are the same. They are SO scare especially when the throat swells. I take 25mg of atarax for episode and lie down and try to relax. But not to be alone IF YOU CAN and if you haven't experienced a lot, going to hosp is reasonable.

I'm also on a schwack of other meds below. I react to all smells chemical or not these days, many foods, heat, and stress.

Hang in there and keep us posted!

[MomtoGiuliana]

I hope you go to the ER if that is what you dr says to do. Do you have any neighbor who could take you?

[Clairefmartin]

Nope, I gutted it out. Lasted over 2 hours...was very scary. I'm wondering if mast cell issues are progressive???

[Serbo]

I think julie gave good advice previosuly when she said when she had a severe reaction she used to go to ER rooms and just wait in the waiting room. If it got worse she checked in, if not she left. i'd always err on the side of caution with any unusual reaction.

[HopeSprings]

Oh, how scary! and with a baby to care for, my goodness. Hope you're ok now and get answers soon.

[juliegee]

(((((Claire)))))

I have tried to respond several times and my very long post keeps disappearing ; I'm afraid that you will end up with the Cliff Notes's version. I know that you already KNOW this, but it certainly bears repeating. You MUST figure out a rescue med that you can safely use. If benadryl makes you feel badly, try some other H-1's, like zyrtec, allegra, or claritin. (I think CLARITIN is the one most easily tolerated by very reactive folks.)

Aaron/Serbo makes a great point about heading to the ER and not checking in when you feel so badly. Just hang out (with your rescue meds and epi-pen) and watch TV. IF you needed to use them, you would be very close to help, should you need it.

Your high IgE is indicative of traditional allergies. Did you recently have those tested? Do you know what you are allergic to? It could also be an indication of how reactive you are...to your traditional allergy(s.) Could you still have MCAS/MCAD? Yes. For instance, if you KNOW that you are allergic to cats (and cats only!) and you have a reaction and were NOT exposed to a cat- THAT could be an indication of overly reactive mast cells or MCAS/MCAD.

Re, serum trptase & mast cell disorders... Your level must consistently be elevated to meet the criteria for mastocutosis. Here is the World Health Organization's (WHO) criteria for mastocytosis: http://www.medicalcriteria.com/criteria/hem_mastocytosis_class.htm

As far as the level needed for a MCAS/MCAD DX; if only it was that easy !!! The WHO is about to come out with that criteria. Based upon the best evidence I can find (recent medical articles posted by the authors of the WHO criteria), this is what it will most likely be:

1. Episodic symptoms consistent with mast cell mediator release and affecting 2 or more organ systems, for example

a. Skin: urticaria, angioedema, flushig

b. Gastrointestinal: nausea, vomiting, diarrhea, abdominal cramping

c. Cardiovascular: hypotensive fainting or near fainting, tachycardia (rapid heart rate)

d. Respiratory: Wheezine

e. Naso-ocular: redness and swelling of the conjunctiva, itching, nasal stuffiness

2. A decrease in the freuency or severity or resolution of symptoms with antimediator therapy (H1 and H2 antihistamines, antileukotriene medications (Singulair, Zyflo), or mast cell stabilizers (cromolyn, ketotifen)

3. Evidence of an increase in a urinary or blood serum marker of mast cell activation: increase of tryptase level above baseline on 2 occasions or only once if the baseline level of tryptase is 15ng/ml or higher. This is the preferred method of indicating mast cell activation. Less specific markers are 24-hour urine with elevated level of histamine metabolite or prostaglandin metabolite, PGF2.

Ana mentions a serum tryptase level above .99 ng perhaps being indicative of MCAS/MCAD; I'm afraid that is unsubstantiated at this time. A knowledgeable source (that we both know) repeatedly makes this claim. This is the research supposedly backs up that claim:

http://circres.ahajournals.org/content/108/11/1316.full I just don't see it here... There IS an article by Dr. Lawrence Shultz that seems to somewhat back that up, BUT he refers to a very specific type of serum tryptase, called "Mature Tryptase." (He has apparently patented that test.) Any MATURE serum tryptase above .99ng is supposedly indicative of MCAS/MCAD according to Dr. Schwartz : http://www.aebm.org/jornadas/alergia/7.-%20Immunol%20Allergy%20Clin%20NA%202006.pdf

You can see that this is very complex and still evolving. I wish it were easier to understand and know that you are seeking help now. Definitely get your epi-pen, follow up with your doc, identify your allergies, avoid them( DUH!), and continue to seek an MCAS/MCAD DX if you think you meet the criteria after your testing is complete.

Feel better & keep us posted on what you learn.

Hugs-

Julie

[anaphylaxing]

I totally agree with the just going to ER and not checking in thing! If I get worried, I drive to the ER parkade and monitor how my rescue meds are working +/- take more if I have to. I bring a BP cuff and monitor my BP. I have a rule that if my BP goes down, or my throat swelling/trouble breathing keeps getting worse, I have to check in. Even after checking in they might stick you in the waiting room and maybe you'll get better and just say you can go! But you need to make sure you have some meds and some plans for help!

SO glad it resolved! Hang in there!

[Clairefmartin]

Thank you all! Julie - I really appreciate the info! I am in a weird situation in that I am home bound, and fairly bed bound, without local family, and all of my local friends work. I can't drive to the ER, I actaully have not gone anywhere except to doctors and the hospital (spent 9 months in patient last year) in over a year and have to go by medical transport because of the stairs into my building. I have to call 911 if anything happnes during the day, and try and find someone that can leave work and come take care of the baby. Its very frustrating!!! I worry that I am over reacting, and dont want to bother my husband or anyone else if it turns out to be nothing.

I am calling my allrgist tomorrow to get an epi-pen. I have been having all these random reations to things I am not allergic too, and have severe GI issues, big time dermatographism, random rashes and hives, and am having my tryptase tested next week. I'm peeing in a "bucket" right now for the 24 hour test, which I guess is good since I had an episode today. I'm pretty sure something mast related is going on, but I do have traditional allergies as well (cats, dust mites, pollen, etc...). I just found this article http://www.jhoonline.../content/4/1/10 and sent it on to my allergist, he has been wonderful with reading what I give him and did his fellowship at Mayo so knows about dysautonomia.

Anyways, I am going to talk to them about what to stock up on, I take Zantac, and I guess I'll get Zertec start? I have not taken any decongestiants or cold related meds besides benadryl because I react so badly, but you are right, I need something in case of an emergency and to take regularly. Oh, and my neoroendocrine doc is testing my for it as well after he saw my skin reactions and I have been taking pictures of these weird flushing things and rashes.

Thank you all again, this is very scary and frustrating!

[juliegee]

YES- Great article by Dr. Afrin, my personal favorite. i wish HE were making up the diagnostic criteria for MCAS

Wow, Claire- I get a better idea of your situation and see how frightening that would be. Out of curiosity, WHY were you hospitalized for so long? I was also repeatedly hospitalized throughout my life for so many weird random things (all MCA related- I learn in retrospect), but I have been remarkably stable since getting on my MCAS regimen. I bet you will be too

Because you are so isolated at the moment, It is really important for you to try new meds when you are with someone, like your hubby. I might try the claritin first- as that might have the least side effects associated with it. But finding the right H-1, when you are very reactive, is just a matter of trial and error...

YES, you need an epi-pen, but rescue meds are even more important. All of us "masties", have a go-to med that we quickly down BEFORE using the epi-pen. That really is the last resort... Should you need to use it, DON'T hesitate. I've only done it once & ALL of my symptoms resolved within seconds- with NO negative side effects. Good stuff when you need it. If you are alone, call 911 first tell them what you are going to do. Have a squad on the way. Chances are, they will find you well- but i just want you to be safe.

Enough scary talk Think happy thoughts and play with your adorable little girl and get down to your thug music. I know better days are ahead.

Hugs-

Julie

[POTSMama]

Claire,

I just caught your post and am SO sorry you're having to deal with this!!! Big cyber hugs!!! I wish I lived nearby and could come help you, darn it. I have this great fantasy of taking off in a motor home, traveling across the U.S. (or overseas in some cases), and visiting my cyber POTS/MCAD friends who have helped me so much by giving me support/encouragement and advice these many weeks. But since I can't do that, I can offer online sympathy as I really know how it feels to have these episodes, and it IS scary.

It would be so good if you just had people you could call to talk you through an episode... I know that helps me. I have done the sit in the ER thing, and I've called my husband or brother or another friend I trust and had them talk me through it because it is frightening to be alone (and it must be even more frightening with a baby to care for). I hope you can find more support as it sounds like you are very isolated, and with this to deal with, that's tough.

I agree with what the others have said - - you need a plan in case you have one like that again. When I feel it starting, I've found now that if I quickly use dye-free liquid Benadryl, after about 20 minutes it will largely stop (but I keep an Epi-pen nearby in case, although I would only use it last resort). About antihistamines - - if you haven't tried any in awhile it might be worth revisiting it (with someone there with you the first time you try a new med),because before I had my first episode of this a couple months ago, Benadryl would wipe me out and make me incredibly sleepy, but now it really doesn't - - my husband is surprised how many antihistamines I down without any sedative effect now (Atarax, Benadryl, Zantac, Allegra)... must be something related to the MCAD...in fact,I get more overstimulated on these meds than tired. Allegra is the one I've added most recently, and even though I react to a lot of meds too, I didn't react to that one and it seems to be helping (especially for the smell sensitivity I was developing), but I am still not stable so continue to try meds.

I listen to Julie and Ana and hold onto the hope that this will eventually settle once I'm on the right meds long enough. So hang onto that thought, Claire, and let us know how you are doing!

[anaphylaxing]

Claire, I'm so sorry to hear that you have so much to deal with. I agree that talking to someone or having someone around would be great IF you can.

Great that you are getting an Epipen and going to try to start on some H1 and H2 blockers. Zantac/Ranitidine and Zyrtec/Reactine/Cetirizine would be a good place to start. Lots of people say it takes awhile to sort out which ones work for them---I'm not there yet! I also take mast cell stabilizers ketotifen and cromolyn. I also take Singulair (antileukotriene) and use Atarax (potent H1 blocker) for attacks/episodes

Keep us posted! We're here for you!

[Clairefmartin]

Thank you all! My parents are actually moving down here from DC and my Dad will work remotely in April to help out and be nearby, so that shoudl be a big big help. I called my allergist this morning, they are calling in an epipen and will give me recommendations for ans H1 blocker. I also called DR. Vinik - my new POTS neuroendocrine doc who i LOVE, and asked for advise as well, since they are testing me for mast cell issues too. They want to do a pH test of my stomach relating to mast cell overactivity (I think).

We also just hired (courtesy of my generous family, cause we are broke!) a family friend/college student to be here 3 days a week for a few hours, so I can take a break and go to Dr apts. That is a huge relief, after my Mom left when the baby was 6 weeks old I have been on my own - home and bed bound raising her, it has been the most challenging thing yet (besides surviving the pregnancy and the PEs). I think I am not prgressng in reconditioning because I spend all my (limited) energy on the baby care, and God knows what I'm going to do once she's mobile.

And Julie - I was in the hospital for almost my entire pregnancy, my POTS went nuts and I was falling left and right, HR and BP went crazy, had vasovagal fainting for the first time, O2 sats and HR dropping before I fainted, and the only way I would stay hydrated was by IV. Then they put a pick line in, didn't flush it with heparin while I was bed bound, pregnant, AND have the heterozygous factor 5 Leden gene, and I had double digit DVTs, a 2 cm clot in my right atrium, and over a dozen bilateral PEs that almost killed me. It was no fun. Then physical therapy wouldnt work with me because of my HR and BP not being stable, and me being pregnant and on blood thinners - fully thinned - was a real fall risk liability. So they let me rot in bed in a hospital, and I am now trying to recondition with the help of a very nice home PT that knows nothing about POTS.

Anyways, thats why I blog so much now, it was quite a journey and my care was terrible to say the least, and I think I have PTSD from it This mast cell stuff is all new since leaving the hospital - I have always had the dermatographism and really really sensitive skin and allergies, but the random food reactions and increase in my skin's reactions are recent.

Thank you all again for all the input!!! I'll let you know what the docs say. I go on Monday for my first (in almost a decade of POTS) autonomic workup and a huge blood work panel for all kinds of stuff, including more mast cell testing, autoimmune testing, and neuroendocrine testing.

[MomtoGiuliana]

So glad you are getting the help you need.

[juliegee]

Wow, that's the first time that I heard your whole story, Claire. Unbelievable, I am so sorry. I pray that you get hooked up with a knowledgeable doc to oversee your re-conditioning. I am convinced that will be the key to your re-gaining your health. Definitely get your epi-pen and work to figure out a rescue H-1....and ultimately a MCA regimen that stabilizes you on a day-to-day basis.

I went from bed bound to running 5 miles at a shot (on a good day ) You can too. It really starts with one step, then two, then three, etc. and working your way towards health. I am most definitely still quite ill, but am fairly well stabilized with my meds, AND so much improved from where I was.

So happy help is coming & that you have such a terrific support system. You also have us all cheering for you.

Hugs-

Julie

[POTSMama]

Julie, your story gives me hope, and hopefully will give Claire that too! It is too soon to know, but I have felt better and with less bad episodes randomly or to smells this week than I have in a month, and so that makes me hopeful that my meds are starting to work. Now if I could just eat more without episodes - - but I'll take one thing at a time!

Claire, I'm relieved to know that help is on it's way for you! I am also trying to get more conditioned (up and about more and doing leg lifts for strength, etc), as I holed up on the couch the first few weeks of this because it hit me so hard and I just didn't feel I could do anything). But I can't imagine how hard it is after 9 months bedridden. I am just slowly getting out and about more, walking more, etc, but I'm sure it will take time. Hang in there and keep posting and let us know how you're doing! What a beautiful baby you have (in your picture)!

[juliegee]

Carol-

I've been following you too and am soooooo encouraged that you are tolerating the allegra. Have you been able to back down on the atarax at all? No worries if you haven't; you KNOW it's all about homeostasis- tiny baby steps- you'll get there. You are moving in the right direction

Yay!!!

Julie

[Clairefmartin]

Thanks Julie and Carol and Anaphylaxing (and everyone!). I spoke with the allergists PA, he will be back in the office on MOnday and they are most likely going to call in the epi-pen then. She had reserached POTS and read what seems like a good starting point - and that was what I have read on here - Zyrtec! I was so happy to hear she read up on everything, what a chnge to deal with concerned docs that want to help. I am officially done with peeing in the bucket, we'll see how the results come back.

I did get some interesting IgE results though, was wondering what you guys thought:

-total level was 127, she said normal was below 114

-barely positive for shrimp (knew that, make me itch, haven't eaten in years)

-positive for cats, REALLY allergic to dust mites, and positive for pecan trees.

-negative for birch tree, which shocked me, because I react to a lot of the foods that are typical to oral allergy syndrome, like carrots and other random stuff like melons

-Neg for all nuts! Have broken out in hives from eating almonds, itched from peanuts, flushing for everything....really weird that my IgE's are neg.

If I understand correctly, that may point to mast cells? Who knows. Anyways - my body is whacky as always. Thanks again for all the encouraging words - I need them I try to keep my sense of humor during all this (Drop it like its POTS, gangsta style), but its tough! Oh, and Julie, I don't really listen to much rap - only older stuff, but I taught high school so I was down with the homies

[juliegee]

Oh come on, Claire- I wanted to think you really had an inner thug-thing going on

Yep, you are right about the likelihood of MCA for the things you react to & test negative to. Some of us, like me, react negatively to everything, but will have have anaphylaxis, nonetheless. We are a bit easier to sort out. But you do demonstrate that same phenomenon.

For instance, I test negative to pistachios- both in skin and RAST testing. But, I ate no food one day (just busy) until I got to car pick-up line & I ate a whole bag of pistachios, waiting for my son- by the time I got home, I was covered in full body hives and had almost no BP. It was VERY clear what I had reacted to- yet tests NEVER bear that out....hence the MCAS. So I can relate to your frustration with testing.

Your doc sounds great. I am so happy that she & her office have been as helpful as they have been- a willingness to learn, understand, interact with top docs, and treat you appropriately & compassionately. YAY!

Julie

[Clairefmartin]

I will work on my inner thug just for you Julie. Not sure what that entails, since I dont walk much I can't have my pants hanging off my butt very easily

"POTS on the ground, POTS on the ground, looking like a fool with your POTS on the ground" (another song spoof -- I feel lyrics coming!)

[POTSMama]

Carol-

I've been following you too and am soooooo encouraged that you are tolerating the allegra. Have you been able to back down on the atarax at all? No worries if you haven't; you KNOW it's all about homeostasis- tiny baby steps- you'll get there. You are moving in the right direction

Yay!!!

Julie

Hi again Julie, I just saw your question here. Still baby steps, but I'm up to a full Allegra a day, and also tolerated 1/2 of a Singulair today (I plan to work up to a whole one slowly). Also, yes, I've cut the Atarax back from that insane 8/day originally prescribed by my first allergist, to 5 or 6/day now. I seem to be doing about the same on the lower amount, and maybe even a bit less brain fogged, so my goal is to cut back to 4/day if I continue to tolerate the Allegra and Singulair. And I also still take the 2 Zantac/day.

My sensitivity to smells really does seem less bothersome, which is huge for getting me out of the house and to my office more hours, and I'm having fewer and less intense episodes and the body numbness seems to have abated. Unfortunately, what has not changed is that I still can only eat a few foods - - my food reactions as I try new foods are sometimes mild (just throat irritation for a few hours) and sometimes the whole "BP drop/tachycardia/shaky/pale/scalp rash/numb lips/throat-closing" thing. So for the latter I'm still taking liquid Benadryl probably too often, but so far it's always stopped the episode within 20-60 minutes, and that's a comfort (thinking I won't have to use an Epi-pen after all).

My GP just called me and said he's been trying to get hold of my new allergist/immunologist by phone to ask her about putting me on cromolyn sodium/Gastrocom or ketotifen (if we can get either at reasonable cost or insurance-covered), but she's apparently out of town so it will have to wait until she's back. He is worried about my continuing weight loss. I sure hope I can get onto one or the other mast cell stabilizer, and that it then helps me be able to eat reasonably again. Meanwhile, I'm just feeling grateful that I am functional more of the time now than I was 2 weeks ago! Thanks for your support, Julie!

Claire, sorry, didn't mean to hijack your topic! Hope you are feeling better today!

[anaphylaxing]

Claire, just checked out your blog; it's great! You have a wonderful positive attitude! I hope you feel a lot better soon.

Julie-- I am VERY inspired by your running comment. WAY TO GO!

Carol---exciting on the Allegra and Singulair! I haven't asked you, did your POTS flare with this ?MCAS flare? I am so glad you are back to work a bit. If I can get off steroids, I might try Allegra for the scent issues.

Yes, Claire, too many threads in a thread, sorry!

[POTSMama]

Ana, yes my hyper POTS flared with the MCAD symptoms (BP/pulse all over the board), but then when I got on the MCAD meds (H1 and H2 antihistamines) the POTS symptoms subsided substantially (far more stable BP/pulse unless I'm having a food or med reaction) which does seem to indicate the POTS is a part of the MCAD doesn't it?

[Frugalmama]

Ana, yes my hyper POTS flared with the MCAD symptoms (BP/pulse all over the board), but then when I got on the MCAD meds (H1 and H2 antihistamines) the POTS symptoms subsided substantially (far more stable BP/pulse unless I'm having a food or med reaction) which does seem to indicate the POTS is a part of the MCAD doesn't it?

Can I ask a strange question? . I've read that MCAS can be exacerbated by exercise and standing - is that correct? If so, that would totally explain the POTS symptoms, wouldn't it?

Also, there's a new info document from a seminar last summer that was just released by the Canadian Masto Society, and in it there is mention that folks tend to just get worse as they get older unless treatment is given. Did you all find a progression in symtoms as well, or did you just head directly into the anaphylaxis? (sorry for hijacking, I just thought this is all related

[anaphylaxing]

Frugalmama

Yes excercise/strain can be a MCAS trigger for many

I had some weird hot flushes and tingling for a few weeks which might have been pots/mcas but nothing debilitating until the anaphylaxis and all that followed

what happened with you?