EE and/or Mast Cell Disorders in Adults

[spacecanada]

A question I forgot to ask my allergist, but hopefully some of you know: should I be modifying my diet before seeing the gastroenterologist (?) or not? Is this test like the Celiac one where you have to eat the offending foods for the test to yield proper results? I had or remove wheat because of hives, eczema, and throat swelling - which also made me very scared.

Also, does Epi help for throat swelling due to EE?

All questions I need to ask my doctor, for sure.  But in the meantime...

[StridAst]

When I went in for my EoE scope, I was told, just be sure to take the PPI (Prilosec) for 3 weeks straight.  No need to change my diet unless something actually caused food impaction.    When I went in, they asked about allergies to peanut and soy, and since I'm allergic to both, they gave me a medication that contained neither.   I remember nothing of the appointment after they gave me the sedative.  My throat was sore for a couple weeks after, and I had a liquid only diet for seversl days after since the biopsy spots need to not get food stuck inside them before they heal.

The actual scope was non traumatic.  Finding food I could safely eat after was far more of an inconvenience, but only an inconvenience.

[krasota]

I've never had to avoid food post-biopsy, FWIW.

[spacecanada]

Still waiting on an initial appointment date but I now know which endoscopy specialist it will be: he has exceptional ratings for both his doctor abilities and bedside manner and his office is super close to where I live - only a 6-minute drive or 15-minute bike ride. (Versus up to an hour drive to get downtown in rush hour.) That helps to ease a bit of my stress.

I found someone in my local allergy group on fb who has EE and has been a helpful resource.  Food still gets stuck all the time (even right now), but I haven't had a full esophagus closure (where I couldn't even swallow water) since I cut out wheat and sorghum. 

Ciel - any update on whether or not you have EE? I hope you were able to get some answers one way or another. 

[Ciel]

I haven't had any further testing at this point, other than IgE specific blood tests for foods I have reacted to in the past. All negative.

StridAst was telling me about Mast Cell disorders in another thread, and hopefully it is okay for me to bring it back over here. I was going to start a new thread, but this one already has valuable information about EE and Mast Cell disorders and all the things that seem to come tangled together so it makes more sense to keep it all in one place. I will edit the thread title to expand the description.

StridAst, would it be okay with you if we copy our mast cell related conversation over into this thread? I will wait for your approval before I do that but I think it would be useful to have here as well.

[Ciel]

Space, I think you said they ruled out EE for you? Or what happened with your testing?

[Ciel]

From Curious, how many people with a PA tolerate/trust peanut oil?

My problem is it's hard for me to pick apart my reactions with certainty, as I have more than just allergies.  I also apparently have a mast cell disorder, as well as eosinophilic esophagitis.  Both the MCAS and the EoE can cause reactions that you can't test for. (Non IgE mediated). MCAS can also cause reactions to things like sunlight, exercise, heat, emotional stress, etc. Also, MCAS, EoE, and food allergies all interact with each other. There isn't an obvious difference between an MCAS reaction and a food reaction, so it becomes much *much* harder to be 100% certain as to why I'm having severe GI distress, hives, a feeling of impending doom, difficulty swallowing, etc.   So I get stuck taking way too many pills, avoiding way too many foods, and much guessing as to what causes what.

I feel for you. I am still trying to figure out my own, somewhat similar issues. Same symptoms you are describing, reactions to foods, exercise, heat/cold, and maybe emotions (I haven't paid enough attention to be sure but I think it has happened -- If I get really upset and panicky I tend to flush and get hives on my face, but I'm not sure if that is a normal physiological response to anxiety or not).

I have not been diagnosed with MCAS of EoE though. And all IgE allergy testing keeps coming back negative. My allergist called me this morning to talk about food challenges, and she touched on the fact that some reactions don't seem to be IgE mediated but nobody knows what does trigger them, because the reactions are to all appearances anaphylactic. And epinephrine helps. I was diagnosed with cold urticaria as a teen, a non-IgE mediated anaphylactoid reaction to sudden drop in body temperatures. It seems to have become much less severe over the last 18 years though, now the worst I get is a few hives and my ears or hands sometimes swell a little. I have reacted significantly to intense exercise. Sometimes the severe GI distress seems to be obviously tied to a food and other times it seems to be random so I don't know if it is a reaction from residual contamination from a known allergy, a new food reaction, or anxiety, or what

I have not been diagnosed with MCAS of EoE though. And all IgE allergy testing keeps coming back negative. My allergist called me this morning to talk about food challenges, and she touched on the fact that some reactions don't seem to be IgE mediated but nobody knows what does trigger them, because the reactions are to all appearances anaphylactic. And epinephrine helps. I was diagnosed with cold urticaria as a teen, a non-IgE mediated anaphylactoid reaction to sudden drop in body temperatures. It seems to have become much less severe over the last 18 years though, now the worst I get is a few hives and my ears or hands sometimes swell a little. I have reacted significantly to intense exercise. Sometimes the severe GI distress seems to be obviously tied to a food and other times it seems to be random so I don't know if it is a reaction from residual contamination from a known allergy, a new food reaction, or anxiety, or what

Thanks!  I want to add that your story sounds *exactly* like a mast cell disorder.  Seriously, look into it.  There are two main types. MCAS (likely not rare, but no hard knowledge as to it's prevalence) and Mastocytosis (1 in 200,000 rare). Symptoms are mostly the same between the two.

Things to know:  MCAS was only accepted by the world health organization in 2007.  So most doctors are unaware it even exists.  Older diagnossis likely predate it's discovery.   Even now the places that know about it are the more well known university hospital's or research clinics.  Here in Utah there is just the U of U hospital's allergy department that knows anything about it.  Other good examples include the Mayo Clinic.  Reputable places that are more clued in on recent medical knowledge.   It's also a hard one to test for. (24 hour urine collection and testing seriously sucks.  So does needing tryptase taken while you are having a major flare and *might* make a 5 min drive without risking an accident.)

Hallmarks of mast cell disorders are along the lines of allergic reactions you test negative to, and or allergic reactions to things you can't be truly allergic to, like heat/cold, exercise,  chemical smells and many other things.  For me, yet another clue that a mast cell disorder was going on was dermatographia (Google it). 

https://www.novapublishers.com/catalog/product_info.php?products_id=42603

That's a peer reviewed paper written by one of the few experts on MCAS.  You might find it enlightening.  Look into it.   Also, anxiety can sometimes be an anaphylactic reaction.  That whole "feeling of doom" stuff.  To me, I get this at every major flare, I feel like I'm about to lose everything and everyone.  Like my whole world is going to collapse out from under me.  And yes, it can be both symptom AND trigger for someone with a mast cell disorder, which can really drag out the duration of a flare.  Kind of a feedback loop. (5 weeks to get feeling "normal" again is my worst record)

Oh wow, thank you StridAst. I need to look into this more. So much seems to be spot on after initial skimming.

I booked a tree nut challenge for September so I have until then to gather info to talk to the allergist about this.

Do you mind if I start a dedicated thread for this? I know we have chatted a bit in the thread about EE and a lot of what you said there also hit home. I would love to try to make a clearinghouse thread to explore how all of this stuff is related. I have many questions! I also think I need to summarize my own history so I can see it better and would be interested in comparing notes with you about your journey in all this (as much as you are comfortable sharing).

[StridAst]

Here are some things to aid your research.

http://www.mastattack.org/

This is a very reputable site, where the author lists her references for the statements she makes.  *Very* informative.  The author has systemic Mastocytosis, but this site is the best source for pretty much any mast cell disorder information, told from the perspective of someone with a severe case.

http://www.mastcelldisease.com/

This is a much less scientific website, written from the perspective of someone with a bad case of MCAS.  She's been bloging about her life for a while, and has more recently tried starting an informative website.  Worth reading as while it's not a scientific look, it's also much less sterile of a perspective.  The first website's author tries to maintain her scientific objectivity which comes across very different.  There are other sites too of course, but these are I'm good places to read up some.  I'm going to list some common words you will run across and what they mean:

Degranulation:. The event where the mast cells release their payloads of mediators in large numbers due to a trigger.  This causes a major flare of symptoms, possibly up to anaphylactoid reactions.  This can go on for hours or even weeks.

Triggers:. Self explanatory, these are whatever cause degranulation with any mast cell disorder.  These can be foods, illness, sunlight, temperature changes, exercise smells, any type of stress physical or emotional.  Even positive emotions have been known to cause problems for some few. Etc.  Any allergen will act as a trigger if allergies are present.  Triggers tend to be cumulative.  If say, onion causes you problems, and so does exercise and sunlight, then you might be able to eat a little onion, so long as you don't then go exercise in the sun.  Or you could be out in the sun for a while, so long as you don't exercise and eat onion.  But 2 of the three would cause a reaction.   This additive nature is why it is absolutely critical to keep a food journal that you also write down what else you do. I.e. activities.  It's the best way to figure out what is causing you problems.

Mast cells:. These are part of the normal allergic process, and the mast cell disease process..  They are the immune system's "big guns". Though perhaps carpet bombing is a better metaphor for what they do.  They have granules of "mediators" like histamine, heparin, cytokines, leukotrines, and so on.  The have, and can release dozens of these very potent mediators.  In IgE allergies, what went wrong is your body makes the IgE antibodies for the wrong things, which trigger mast cells, basophils, eosinophils, or leukocytes to release their mediators.  Of these cells the mast cells have the overwhelming majority of the mediators.  With mast cell disease, they release these mediators without the need for IgE antibodies. Instead they release when you encounter your triggers.

Mastocytosis: extremely rare disorder that is characterized by the body making too many mast cells.  Symptoms are pretty much identical to MCAS.  Much easier to diagnose, however it's insanely rare at 1 in 200,000.

Urticaria Pigmentosa:. The cutaneous (skin) based type of mastocytosis.  It's characterized by spots on the skin that turn into a hive if you rub them.  These hives form only over the spots. They can be large spots or small ones. Some people have thousands, some have only a single spot.  This is the most easily identifiable mast cell disorder.

Dermatographia:. This condition is where you can draw in you skin with a blunt instrument and create hives.  This, unlike Urticaria Pigmentosa isn't restricted to just over spots.  This is often a symptom of a mast cell disorder.  With MCAS patients, the hives are often less raised but quite red. (Though not always)

MCAS: the most recently discovered and least well understood mast cell disorder.  Mast Cell Activation Syndrome was accepted by the World Health Organization in 2007.  They have not, as yet, have an official diagnostic criteria.  This leads to challenges in getting diagnosed.  The most informed places are research hospitals and clinics.  Most doctors and even allergists, have not yet heard much about it.  It is commonly comorbid with other conditions like Ehlers-danlos Syndrome, POTS, eosinophilic disorders, and seems to be often a secondary condition as it's not often found alone. 

Treatment for mast cell disorders revolves around suppressing the symptoms.  Epinephrine for anaphylaxis, antihistamines (both H1 and H2 types), leukotrine inhibitors, mast cell stabilizers etc.  Many of us mast cell patients are on high doses of the antihistamines.  I'm personally on 4 Zyrtec per day. 2 pills in the morning, 2 at night.  (Along with all the others, not explaining my full regimen, just that we often need excessive amounts if antihistamines). For this reason, among nanyothers, it's ill advised to attempt to self medicate with Zantac and Zyrtec (H2 and h1 inhibitors respectively) as you might need high doses and need to know what's safe for your body weight.  Treatment also involves limiting as many of your triggers as possible.  The treatment won't stop degranulation, just makes it more tolerable and less severe. At least that has been my own experience.  It also minimizes the daily minor level symptoms.  I feel a lot better than I did last year, but I've still had 2 degranulation episodes this year. They were my most mild yet.

This post will be spell checked tomorrow. I typed it on my phone, and autocorrect probably garbled done of it.  Will likely add more info then too.

[Ciel]

Thanks for taking the time to post this StridAst. Very well done. I appreciate your insight!

I have a basic working understanding of some of this stuff but not enough to make things in my head any clearer than mud.

This sounds a lot like me in many ways.

I will come back with a better reply after I have had time to digest the info a little more.

[spacecanada]

Space, I think you said they ruled out EE for you? Or what happened with your testing?

My tests came back negative for EE.  My allergist initially wondered if it was a false negative because I avoided my trigger food (wheat) exceptionally well for the four months leading up to the test.  (I am someone used to total and complete avoidance, including trace amounts, after all.)  I was instructed to try wheat again at home and see if my symptoms returned, and they most definitely did, though not consistently.  My last two encounters with wheat gave me hives and other IgE-like symptoms, so I am really confused and scared now.  I have my annual allergist appointment in October; no sense booking an appointment before then.  I am not in a good place mentally to deal with it right now.  My own allergy mystery/saga continues...

[GoingNuts]

I'm glad you don't have EE, but sorry you don't have any more answers, SC.  So terribly frustrating. 

[Ciel]

I'm sorry Space.

[Ciel]

I had a gastroscope (gastroscopy?) last week. He took biopsies but I haven't heard anything back yet. I'm not holding my breath for any answers. He said that visually everything looked good...I guess that is good. One doctor thinks the pain might be esophageal spasms, it does feel like a milder version of the two I had a few years back, so maybe. I'm not sure how food would be a trigger for spasms like that though. I have very severe reflux, which makes more sense but they don't occur at the same time which I would expect. And they are different types of pain -- reflux is not the same as the squeezing pain that leaves me doubled over. I need to make a follow up appointment for next week with my GI.

[spacecanada]

I hope you can get some answers, Ciel. 

[StridAst]

Good luck, I hope you get answers!  My own endoscopy that I got the EoE diagnosis from looked mostly normal (some "washboard like contractions" was all he noticed.  Biopsies came back with counts per hpf in the 20s with anything over 15 eosinophils per hpf being a positive biopsy.

My previous symptom history includes a trip to the ER for chest pain, where they determined it "must just be heartburn." And I kept explaining "I've had heartburn.  This doesn't feel anything remotely like heartburn." Definitely was a waste of time and money.  Now I recognize it was most likely EoE related, but at the time I had no idea what was going on.