I have Reynaud's.   This goes along with the autoimmune form of masto.  I won't say that all of us have it, but Raynaud's has a known autoimmune cause and this is something that my doctors began seeing when my masto came out of hiding.  It wasn't present before hand, only after it went active.


As to the positive response to the antihistamine, this is part of the MCAS diagnostic criteria, yes.  And you were very smart to figure out taking some without having to go into a full blown attack before doing so.  That positive response confirms that what you were feeling was indeed connected to histamine release.

As to that pool, I have found that I can't tolerate cool water anymore.  When I went to the beach a few years back it was in the 90s and as much as the water was delicious, it was just cool enough to begin triggering me and I had to come out of the water because my stomach began churning and I could tell I was going to have an attack.  I have only been able to venture into swimming pools and they had to be almost bath water temperature - very discouraging for an ex-fish!      So, I've learned that the temperature of water is very important.  The issue is that of maintaining your core temperature stable.  Extremes of heat or cold will cause major issues and when it comes to being in a pool or a large quantity of water, you must be careful for you don't realize how being emersed in a lot of water can effect your core temperature really quickly.  Then getting your core temperature back to it's normal is tricky because for some of us, our masto had our temperatures on the low side.  Mine runs about 96 degrees!  The other day I woke up FREEZING and took my temperature and it was 95!!!!!!!!!   I've asked my doctors about this and even masto doctors and nobody seems to really understand why or how this happens.  This is why I say that being in a pool or a larger body of water is very tricky for us for if you're already runnging a below normal temperature as a normal temperature, then by reducing your core temperature even further, you could be causing serious harm to yourself because our bodies are supposed to function properly with 98.6 degrees and when it gets too low, even on a normal basis, things aren't going to function properly and it could get you into trouble.

So, be careful.  Living with a MC disorder means that your body is going to do some weird things and some of those things people are not going to understand!



Lisa

Guantai, a MC disorder breaks the rules of medicine.  It´s like a teenager who will defy the rules just to defy without rhyme or reason.   For example, there is one rule of anaphylaxis - it ALWAYS happens with HYPOTENSION.   With masto, this is not so and there are enough of us who have pressure which will go as high as 200/110 during anaphylaxis.   I´ve had my pressure go to 220/110 due to contrast injection!!   And when we talk about anaphylaxis, when you ask a doctor what he looks for with anaphylaxis he will almost always say breathing obstruction due to the throat closing up.   If you ask for a poll here on the site how many of us have this issue, the overwhelming number will be that it does NOT occurr.  

Why?!

This is because with a MC disorder you have the MC degranulating in a very different manner than it would if this were an IgE mediated allergy.   The mast cell has recently been discovered to have a SELECTIVE RELEASE of it´s granules.  This is because cells have been found to be able to "speak" with other cells.  When you have an IgE mediated allergy to something like peanuts you have an enzyme which has created antibodies to that protein and the mast cell has receptors that identify those antibodies and enemies and it will then release it´s mediators en masse and thus create an explosive like reaction - the more times the bigger the explosion.  This causes the entire body to react, with edema, (which is the cause for the throat closing and swelling of the interior tissues) brochospasms, (which make the breathing difficult), nausea, vomiting, diarrhea, hypotension, etc.   It´s a MASSIVE reaction and why it is so deadly.  

However, with a mast cell disorder, our mast cells are not functioning properly or we have too many or BOTH.  This means that they are not being triggered to release en masse like an IgE allergic reaction, but that depending upon what triggered it, depending upon whatever is involved with the reaction, the MC, in its MALFUNCTION will release, sometimes en masse and sometimes selectively, its mediators.  Well, this is going to give you various kinds of reactions.   And as recent research has been showing, it also may depend upon the genetic defect you have on those defective mast cells which has you doing certain reactions versus others.  

For example, I do not have any angioedema EVER. But I go through tons of syncope.  This may be due to my form of the MC disorder.  And I also get hypertensive in my reactions and only hypotensive when the MC mediator release is less acute.  This may be my own genetic version - the researchers still have a great deal to figure out to understand all of these things.

So, when one of us goes into the hospital with vomitting, hypertension and flushing and dyspnea, they will not recognize this as anaphylaxis.   This happened with me about 3 months ago and it was due to the heat.  They thought it was gastroenteritis.  It was only when I went back into the ER about 3 weeks later after reacting to silicone that I was able to talk with the doctor about that prior reaction and told him that my doctors had come to the conclusion that it was not food which put me into the ER but anaphylaxis showing itself with the vomiting, something that I very rarely go through.  

This is why masto breaks rules and why our doctors have a terrible time trying to identify it all!!


So, why would this be any easier for US if our doctors themselves can´t figure it out?  

I have found, Guantai, that it is through speaking with other patients that some of the riddles of this disease begin making sense for in finding others who have similar reactions and situations that I begin finding understanding.  With that understanding I begin to learn how to keep myself from reacting and this gives me more security in dealing with my illness and thus relieves a great deal of pressure and anxiety and with this life begins to return to something more NORMAL - PEACEFUL!!!

As to our core temperature, I find that this is what makes the most sense to me.  I live in Brazil and we get some pretty heafty summer heat here and it´s for months.  Now in August and early September, it´s supposed to be "winter" technically speaking, however we get a high pressure system over us which blocks all cold fronts and since we rarely go below an average of 75 degrees in the winter, this means that as the duration of the high pressure system endure, the higher the temps and we´ve seen it stay about 90 degress on average through this 6 week period with temps up to 104 as well.  Then when Spring comes, the temps drop back down into the low 80s and they slowly climb back up to the 90s in December through April with temps in the low 100s in Jan and February.  I´m happiest in May, June and July for these are when our temps are best in the upper 70s most of the time.  

In the 5 years I´ve been living with an active mast cell disorder, I have found that I can endure the heat as long as my trunk is kept cool and as long as I am breathing cool air.  If the air is hot to breath, this will trigger me.  I can have a wet t-shirt on or be in a bathing suit and in the shade, but if the air is hot, then just breathing in this hot air will begin the triggering process.  That is because in breathing in either very hot or very cold air you are directly taking those temperatures into your lungs and this directly affects your blood and that inner core temperatue.   Granted, it does take more than this, but this is why many of us prefer to have our bedrooms cool and have blankets on us than having the room warmer and going without blankets.  This is also why when we get into a tight spot with the heat more often than not just having the AC blowing in our faces and breathing that cool air in will do us more good than getting out of the sun with a breeze blowing.  The cold air going directly into our lungs helps more to bringing down that core temperature than anything else.  

Yet this is also why when you go into a pool, even though it may be very hot outside, if that water is too cool it you will begin shivvering quickly, this is because your extremities are carrying the cooled blood back into your warm trunk, but if the trunk is emerged in the water then it too is cooling down quickly and your body is beginning to react to it.  

This is why for surgeries there are protocols written by Nancy Gould that the doctors need to put you on a warm table and have warm IV fluids being pumped into you.  Those cold fluids cause the MCs to react and by not being on a warm table, especially since it is metal, your trunk is also being exposed to cooler temps and your core temperature is being reduced as well.

Now, if this has anything to do with having a low body temperature, I have no idea!   All I know is that I feel the cold more quickly than ever before in my life and that I feel the heat more too!!!  That´s my life with masto!


Lisa

I wish there were even ONE article on this autoimmune form, but sadly there is not.  It is amongst us patients that we know and share what our markers are.  Dr. Castells is the only masto authority who openly recognizes this but she's not really made any open statements however, Dr. Akin did present this to the TMS conference in 2009.  I do not believe it has been officially recognized with the WHO Consensus document which came out recently on MCAS.    However, if you ask your doctor to speak with Dr. Castells about this she will give him that information, I'm sure.

You see, there are no studies nor even one case report which has been written!  I'm working with my doctors here in Brazil and we're trying to draw a profile and this is because all of my 3 children have this as well.  The more my doctors and I can find out, the better it is for my kids.  

I've got a raised ANA of 1:320, Hashimoto's Thyroiditis and a positive ASST skin test.  That skin test is where your own serum is injected back under your skin and compared to the injection of pure histamine and saline.  I gave a good reaction to both the histamine AND my own serum and left that doctor totally FLABBERGASTED!!

Two of my kids have raised ANAs of 1:320 and one has Hashimoto's.  The other has autoimmune markers and behavior with the rheumatoid area - his connective tissues.  One of my kids is in remission and although the ANA and Thyroid was normal, that child had a very high level of autoantibodies to thyroid only a couple of years ago and so my doctor thinks that this child is in remission and that later on in life the masto will show up.  However, this child ate some shrimp and sushi the other day and came home with hives and edema on their face and I knew exactly what the cause was!!! MASTO.    
One of my children has eosinophila in their intestines and we found a focal of MC aggregates there too. That one has a tryptase of 2.6ng whereas mine is 4.6ng and the other who flushes most and has gone through 3 cases of anaphylaxis has a tryptase higher than mine at 5.5ng.   The one who is in remission has no autoimmune markers other than the Hashimoto's in remission and has a tryptase of 1.0ng.

So, this is the profile of my family.  

In speaking with others, there are other markers, but generally we have found that when an autoimmune disease has been investigated and ruled out then those vague autoimmune markers belong to masto.   In speaking with some authorities, they have agreed with this and my doctor has finally agreed with me that these autoimmune markers, which she could not understand before, are without a doubt important and do belong to our form of masto.  

So, in your telling this to your doctor he may ask - does this rule out Systemic mastocytosis?  NO!!!   For now, nobody has given any importance to these autoimmune markers for either you were SM or you were nothing!  Once the authorities began giving validity to Dr. Castells and Dr. Escribano's and Dr. Moldering's convictions that there was MORE out there, then they began accepting MCAS, but to include autoimmune into this is up in the air.    I know that these doctors  all firmly believing in this but how it fits in is the question.  Without it being defined and a study looking into this as to what the profile of this patient is, it's hard to say what makes up the autoimmune profile.  For some, they are considered non-clonal MCAS, whereas in the case of my children and I we are proving to be MMAS, or a middle of the road with one foot in the SM camp and the other foot in the MCAS camp.  They suspect that this patient is a pre-SM patient for some of these patients move into full blown SM.  But it's very HARD to give any definites for now - only TIME will tell us.


Now, what about YOU???   GO TO CASTELLS WITHOUT A DOUBT!!   However, your doctor should speak with her first and do whatever testing that may remain to be done or talk over with her whether it should be done in Boston.  They may want a BMB to rule out SM and your tryptase should definitely be done for that's the best indicator for SM.  He should also consider what medications to adjust with her in order to help giain you more stability.  

But all in all it sounds like you are in GREAT hands and that your doctor will help you loads!!!  He should also be aware of the hypertensive variant of masto and you need to have him write out some instructions for the ER in case of an emergency so that you have these with you otherwise, you can go through the same difficulties with upcoming situations.

Now as to those reactions and those situations you went through, here on the board we have some protocols IN ENGLISH for pre-medications.  You must NEVER, EVER face contrast again without being PRE-MEDICATED!!   That syncope, I know ALL about that and am working with doctors who are going to try to figure this out for it seems to be much MORE than the doctors claim it to be.  I've been in this reaction for up to 6 hours due to contrast!!  Ugh!  The pre-medication may or may not help you with that reaction, it's not helped me.  For that you must have an IV infusion of epinephrine for it's a longterm degranulation reaction and only epinephrine and IV antihistamines deal with it.  

However, ALL of those reactions you underwent were due to either surgery or contrast and that's because they cause ACUTE AND MASSIVE MC degranulation!   With the proper pre-medication and then adjusting the masto meds to COMPENSATE for the acute reaction following those procedures you can come out of either situation as well as any normal person!  You don't have to suffer with all the extra reacting as long as you have been medicated PROPERLY AND ACCORDINGLY!!!!  I PROMISE  YOU!!

Look, Guantai, I went through open heart surgery 2 years ago and was kept safe and ALIVE due to those protocols!   Just copy them for your doctors, or get the PDF file and keep a copy in your car and leave them with the hospital and you will be SAFE for any medical procedure you need to face!  Just go to the link and READ them carefully, they'll even teach you how your body will and can react and to what.

http://mastcelldisorders.wallack.us/yabb/YaBB.pl?num=1293571751/4#4


Lisa

This Topic was moved here from Symptoms by DeborahW, Founder.