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DeborahW, Founder
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Reply from Lisathuler
Hi Kim!!!
Állow me to give you a suggestion: Please ask for your doctors cell phone numbers in case of an emergency with Brie. You are right about your concerns for any doctor who doesn't know Brie could easily suspect this with you. Having your doctors' cell phone in an emergency will assure that this accusation never occurs because then all you have to do is call your doctor and hand the phone over to the attending and he will be given the low down on Brie and perhaps even better intervention becuase of it. Brie's situation is such that you need that extra support.
Now, let me explain a bit about anaphylaxis and masto. '
Kim, we masto patients do not present with the same symptoms that an IgE mediated allergy patient goes through. This is due to the mechanisms being different. An IgE mediated allergy is one where the receptors on the mast cells are primed to react to that specific allergen and it will cause the MCs to open en masse, which is why you get this FULL BLOWN EXPLOSION in the patients. This en masse degranulation happens throughout the entire body which is why patients will have ALL of the systems involved! This explains why there is so much swelling of the tissues both on the exterior of the body as well as interior. Depending upon the genetic makeup of each person you will have certain reactions but this is what will cause the intestines to let loose for they are injecting high quantities of liquid from the walls of the intestines into the feces and they totally let loose like a dam which broke! This same releasing of liquid and edema happens within the esophagus and lungs and mouth and throat. But at the same time, this escape of liquids ends up depriving the blood of the necessary amounts of volume and thus the blood pressure lowers! So this is why you will see skin involvement, respiratory involvement, blood pressure involvement and gastric involvement! The system is totall overloaded with extreme reacting!!! And this is what doctors are trained to recognize as anaphylaxis for this is indeed what the majority of people out there go through when they go into anaphylaxis.
But we masto patients are different and this is due to the mechanisms involved. It's still mast cells being triggered, but it's not always an overall reaction. We tend to react where our weakest spot is, and depending upon where that is, that is what is going to be the more consistant reacting going on, but how that is chosen by the body is something the researchers just don't know.
For example: Two years ago following Christmas and New Years, due to how late I was staying up at night due to the holiday parties with family, I ended up going through 3 seperate crises with only two major symptoms: hypotension and explosive diarrhea! It was horrible and my intestines not only let loose, but they kept at it and the intervals went from once every 45 minutes to reducing to every 3 minutes and the pain was intense because there was nothing more to pass except clear liquid and my doctor said that it was getting to the point that I was going through micro hemorrhaging! I was desperate for the first crisis hit me on New Years day and I didn't know what to do! I was thinking ER, but I already knew from experience that they didn't know what to do either! At that point I was still such a rookie at this that it hadn't occurred to me in the beginning that this was anaphylaxis. I thought it was something I ate! but by the time I was 3 minutes apart from racing to the bathroom the lightbulb went off and said, THIS IS ANAPHYLAXIS!! So I took Ranitidine and BINGO!! EVERYTHING MAGICALLY STOPPED DEAD IN ITS TRACKS AND REVERTED! The hypotension improved and the diarrhea totally stopped!!
When I spoke with my masto specialist she said, Lisa, That was Anaphylaxis! That was the Masto!!! When it happened twice more within the next few weeks I knew what to do! It's never happened since!!! Why it attacked only my intestines, I don't know, but that was the major weak spot for those 3 times. Was it anaphylaxis MOST DEFINITELY and I had the involvement of the cardiovascular system as well as the skin for by the last point of it I was doing some flushing.
Kim, according to the AAAI, there must be 2 systems involved to be considered anaphylaxis. But unfortunately most doctors are not trained to think of a combination of the two and they are instead trained only to recognize only the classic IgE explosion as anaphylaxis. Yet according to the AAAI, the first major symptom is that of skin involvement, be it flushing or urticaria, or whatever, it doesn't matter, but skin involvement is the first major symptom. The next symptoms can by any one of the other systems be it diarrhea, or dyspnea, or throat closing, or hypotension.. it doesn't specify exactly what! This is a document stating openly that it's AT LEAST 2 systems involved, one being cutaneous and the others being whatever. But most doctors are not trained this way and so they don't recognize it!
My own doctors have been stumped time after time Kim, and during the time when we didn't know I had masto, I ended up in a crisis for 2 days. I was playing Brazilian Dodgeball with the children of my school (it was a great game!) and when the game was over I was shaking horribly and I could barely speak in Portuguese and any question asked to me was horribly difficult to answer and even in English it was a challenge - I could not think straight! My husband took me home and I crashed out all afternoon but when I woke up I was so weak and exhausted but I had dinner to prepare and so I pushed myself to try to make dinner, but I no sooner got preparing dinner then I went into a crisis - strong dyspnea, intense weakness, abdominal pain e flushing and I couldn't do a thing! I called my husband and he rushed me to the ER! They didn't know what to do with me and I was kept in observation for 3 hours until the crisis resolved itself. I was there with an IV only. They took my BP and it was 150/110! I would be doubled up with the pain and dyspnea but then the dyspnea would subside and I'd go into the faint. This carried on for those 3 hours and finally resolved itself. The doctors wanted to give me a seditive, but I refused it for I knew that this was not the problem!!! Unfortunately the ER in my area loves to give sedatives! The following morning I was to go see my doctor and take with me results to the 5-HIAA urine serotonin test for carcinoid syndrome. We picked up my test results and I went into his waiting room there at the maternity hospital. While sitting there waiting to see my doctor I went back into the crisis - it was biphasic anaphylaxis and since only 12 hours had passed from that of the crisis the night before, I was still reacting! I had not been put on any antihistamines yet for we didn't know that this was masto, only suspected it by this time. Well, my gyno was desperate for he put me into an empty office and had me under observation while he had another emergency with a pregnant patient come in and he was by chance the only attending gyno at the hospital that morning - the others weren't scheduled to come in until noon and it was 9AM when this was going on! So my gyno, (who is the doctor who raised the flag of suspicion for disease) kept me in the other office and would come into see me between that of attending to the emergency case as well as his other patients and it was one of those days which he won't forget due to that situation of mine. Again my pressure was high, I was flushing, intense dyspnea and abdominal pain and then fainting in between the dyspnea. He kept me with him for 2 hours and finally had my husband take me to the ER of the main hospital along with a written report of my symptoms. The ER doctor saw me and the first thing he said after reading the report was "You don't look so red to me"! Well, they kept me at the ER, but didn't give me a thing other than an IV and I was in this state for another 2 hours until once again it resolved on its own.
Nobody recognized it Kim and to today, it still is hard for them to believe that this is anaphylaxis! Last year my angiologist put me through a CT with contrast. She's seen me react and she knows what the disease is and we know how to protect me against the contrast, but you see, my disease is autoimmune in nature and the suspicion is that I also have IgG involvement and that I've become IgG allergic to the contrast so my reactions keep getting stronger. Well, due to the challenges that my doctors were having recognizing the syncope, they thought that I was sleeping since every time I had done a procedure I was sedated or under anesthesia. This is importan that in my case that this be used for I am the uncommon patient who has Stress Anaphylaxis. Not many of us do this, but I'm one who does which means that anything which will stress my system will trigger anaphylaxis. I've reacted to the testing for a glucose tolerance test - it put a strain on my system even though my test came back negative. I react to the BP meds when they pull my pressure down too quickly - the reaction doesn't happen immediately when the medicine gets into my blood stream, but only moments later after my pressure begins to fall. If you inject medication into my vein too quickly, this will cause me to react and if I take laxatives, when the medication begins to take affect, this has also triggered me. So, those patients who are this sensitive, the only way that they can be dealt with safely for invasive procedures is by using sedation or anesthesia, which is what my doctors were doing. But, they kept saying that my prolonged syncope wasn't syncope but that it was sleep due to the medications they were using. So for that CT last year I refused the sedation but the contrast ended up putting me into anaphyalxis immediately as it was injected! I had intense flushing, intense dyspnea, but no throat closing, just pressure upon my chest, and hypertension. Yet my doctors didn't give me epinephrine for they are trained that never use epinephrine with a hypertensive patient! They're right, but not in this case for the hypertension is caused by the mediator release, but because I had an aortic aneurysm I'm sure this was also a deciding factor. Anyway the dyspnea resolved itself and they had me on oxygen until it did. But the flushing continued for HOURS afterwards and the pressure was brought down by diuretics because they knew already that using the meds to pull my pressure down quickly would trigger another attack and they couldn't risk that. But the thing is, that when I went into the syncope for 6 hours this time, my doctor was able to openly state, this IS SYNCOPE due to intense VASOPLEGIA and yes, Kim, my pressure was 180/110 while in syncope and while in vasoplegia!!! THIS IS MASTO!
Anyway, later on, when I spoke with my doctor about this situation, she ended up saying that she didn't feel that this was anaphylaxis!!!! Every single one of her colleagues disaggreed as well as I! She said that this was a "side effect" of the contrast even though she's seen me react before! But she just couldn't believe it could be anaphylaxis because it resolved without epinephrine! I was so loaded up with the pre-medications of prednisone and antihistamines that there was no way it wasn't going to resolve one way or another, but because she had me only on oxygen, this shook her up and made her doubt her own eyes and experience with me!!! Yet, there it was, skin involvement, cardiovascular involvement, respiratory involvement! 3 systems!
So, you see, Kim, masto reactions and crises can be intense or mild, but the fact that you have mediator release behind all of these situations it is STILL ANAPHYLAXIS!!! And therefore there is the POTENTIAL for a very serious situation in that it can snowball and end up getting out of control!! It is very important that you do not underestimate these reactions of Brie. You must take her BP when she begins into a crisis situation. If her BP drops a lot, then you need to hit in with an Epi-pen and get her to a hospital. Sometimes, Kim, the reaction will show itself without skin involvement and this is because the reaction can be so quick that the skin will be bypassed and instead you can go into a severe cardiovascular reaction! These are what I am now doing in that I'm bypassing the flush and dyspnea but instead I find myself going straight into the prolonged syncope! These are severe cardiovascular reactions and this is why in my case I am suspected of having this MMAS form of masto - these reactions are more typical to this form of the disease.
One of the things the research is beginning to show Kim, is that depending upon the form of the masto, you have certain reactions which are more typical to that form. The recent report by Dr. Escribano which differentiates between MMAS, SM and nonclonal MCAD is that the MMAS patient doesn't have urticaria or angioedema but has syncope and cardiovascular reactions as well as a low tryptase. Well, there are many non-clonal MCAD patients who do have angioedema and urticaria as major issues! I don't. Depending on the form that Brie has, apparently Kim, the symptoms she presents are different. This is only NOW being discovered.
Well,l hope that this long explanation helps you understand more. I'm going to send you some files on anaphylaxis that I got from the NIH. If anybody else wants them please let me know and I'll be glad to send it to you as well. If I don't already have your email, then PM it to me!
Lisa
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