Well, there's always a first and it HAD to be ME!  LOL.  

I had to do what is called a Renal Scintigraphy.  This is a nuclear exam that takes a look at the kidneys.  They use a type of contrast which does not cause reactions.  There are absolutely NO CASES reported for reacting to this solution.  I spoke with Dr. Luis Escribano and he said that it is not recognized to cause MC degranulation.  

Regardless, I REACTED!  

I had looked up on the internet and tried to find cases of masto patients regarding this contrast and I couldn't find a single case report.  I has been reported to be of use especially in regards to doing bone lesions studies with masto patients but there is not one report of a reaction.  I figured I was in the clear and didn't bother telling my doctors of when I'd do the exam nor even to pre-medicate.  THIS WAS MY MISTAKE!  

Immediately upon injection of the solution, my vein begin to sting very strongly!  As the solution went up through my arm I could feel the changes going on.  As it reached my shoulder I could tell something was wrong and felt the solution changing things as it went up into the right side of my face - this is why I began to flush.  As it also went down into my chest I began having dyspnea.  With both of these symptoms also came mental confusion and brain fog and terrible weakness.  I realized what was happening but had difficulty getting the words out in Portuguese to get my purse and my meds!  I couldn't connect the words!  The attendant finally handed it to me and I fished it out for myself.  He called the doctor and as I sat there trying to keep a hold of myself he took my wrist and took my pulse - no tachycardia, but this is normal for me.  The place on my wrist where he was feeling, left a spot of pressure urticaria - something which never happened before.  My other wrist, where the medication was injected was inflammed and still burning strongly.  

His first question was, Do you have Panic Syndrome?   I chuckled at that and told him NO, even while I was struggling to keep my control and deal with my symptoms.  I had wanted to lie down, but I knew that if I did, I'd pass out.  I didn't want that so I refused to lie down and kept talking with the doctor instead.  I knew that as long as I could keep fighting this mentally, I'd get past this once the antihistamine kicked in and sure enough this is what happened.  As we talked things began to calm down again.  He saw that I wasn't freaking or panicking so the theory of the panic syndrome was tossed out the window.  He was worried about my throat closing up but I told him that according to the research that Dr. Escribano had done, MMAS patients don't have angioedema as a symptom usually and I believe that this is why I never get wheezing or throat swelling with these reactions of mine.  From what I've been asking amongst the researchers, I believe that depending upon the genetic defect itself, the form of the mast cell disorder, this influences our symptoms and their types.  This are only MY conclusions, however.  

Anyway, things calmed down a good bit and I went into the waiting room for a bit before I went home.  The exam itself was only to be about 5 hours later and so I was free to leave, but I ended up having to take a 2nd allegra and continue waiting it out cause things wanted to get worse again.  I've never had to do this before.  After another 20 minutes, I was better and could leave.  I had to return to work and couldn't go home until later, but at work I was not doing well.  I had to finally step in with prednisone and yet another allegra about 3 hours later and then went home for about 2 hours before going back to the clinic.  This time my husband took me.  

I talked with the doctor and showed him my wrist.  It was bruised and the vein was enlarged!  He was impressed.  We spoke about when I have to do the 2nd round of these tests in 2 weeks and for that one I'm going to pre-medicate and we're going to see how I respond to it.  The next exam will be that of receiving the injection and then go right into 2 hours of scanning, so this may be trickier for me to have to deal with.

Yet while I was undergoing the 30 minutes of this scan, after the machine was turned on, something about this machine caused me to begin to feel weak and I fainted away.  When the scanning was done the technician had to shake my arm and yell into my ear to get me to respond and I was woozy and weak - something which doesn't happen all that often!  From there I went home and slept for a couple of hours and kept quiet the rest of the evening.  

I've been having to deal with this reaction since adding prednisone and doubling up my antihistamines.  My doctor told me to drink "an absurd amount of water"  Ha! my doctor is a kick!  She put me on Prednisone until yesterday and told me to have complete rest!  (right!  something I just can't do at this moment due to my school responsibilities!)  

So, I've tried to stay home and did on Sunday, but yesterday I had to come into work and today and the rest of this week I have no choice and must come into work - nobody can replace me right now.  But I'm not having an easy time.  I'm taking my antihistamines like Tic Tacs and yesterday was still taking the prednisone.  Today I haven't taken any but may have to for I was no sooner here in my school when a crisis hit me.  I usually will have only flushing, shakiness, weakness and dyspnea but this time I had all of this but I also got the anxiety where I can't sit still and diarrhea also hit me!  I was inches away from the full shebang!  I don't usually get this bad!

So, WHAT HAPPENED????    I've been speaking with some of the authorities about this as well as my own doctor.  This was NOT supposed to happen and I am this one patient who ALWAYS seems to be reacting to things that NOBODY reacts to!!   My form of masto is just so incredibly sensitive!   I have what Dr. Escribano calls, STRESS ANAPHYLAXIS.  This is recognized amongst some of us in that we are so very, very sensitive, that any kind of medical procedure is a risky situation.  This is why he has on his protocols that some procedures like a BMB must be done within the ICU or the operating arena where the crash cart material is right at hand.

When I spoke with my doctor she had some insight and we think we know what happened.  Dr. Escribano is 100% correct, this contrast is not known to degranulate mast cells and usually this is not a danger to masto patients.  HOWEVER, in MY CASE, I have something MORE going on with my system and my body seems to recognize ANY contrast almost as it would the CT contrast with Iodine.  CT contrast is indeed a KNOWN MC degranulator and all of us have the potential to react to it.  But my body seems to have developed an IgG true allergy to this contrast and my doctor feels that my body recognized this contrast in a very similar pathway as to the CT contrast and this is what caused the reaction!

She said that the bruising on my wrist is proof of the immediate heparin release and that this is why I was "feeling" it going all the way up the line!  This is what caused the burning!  If we had done some lab testing, my labs probably would have been totally off!   I'm going to ask for these tests the day of my upcoming 2nd exam to see what we can find!

I WILL PRE-MEDICATE!!!   I MUST!

I have asked the attending doctor to write up a report of this incident for my doctors and Dr. Escribano.  We are going to do a before and after kind of report so that he can report what happened without the pre-medications and then after the REMA protocols.  The solutions they use for this contrast are very similar and so it should be interesting.  

My husband will be in attendance and I will be totally tanked up with meds.  I should not react for these protocols have held me before.  However, with CT contrast they have not and this is what makes me nervous.  I have gotten so very allergic to CT contrast that Dr. Castells had to adjust them for me in order to go through a catheterization last year.  I've reacted to the CT contast 2 years ago even using these protocols, which is why she had to adjust them for me.  Yet that contrast is a KNOWN MC degranulator and although I did react to this contrast, the reaction was not so very severe.  I was able to deal with the symptoms at the time of the anaphylaxis and my allegra did hold me and bring things back to rights.  So, this is what makes me feel secure that I will indeed get through this fine.   However, if anything should happen, the doctor is aware and he will have my doctor's phone numbers and the hospital is but about 2 minutes away and we can get there faster that the ambulance can get to us!

So, this explains my absence here.  I've been a bit "under the weather" and also swamped with work.    I WILL BE FINE!!   And this is the beauty of having masto versus some other horrific disease!!   I know this disease is a tedious monster to have to live with, but it's still normally not this horrendous killer that cuts our lives in the bud!  The great majority of us just turn into these horribly allergic RED monsters!   I can live with this!


Lisa

Oh Mona, you are SUCH a DEAR and I love how caring you are of me and of others!!   You are truly a lovely woman!!


As to me acting up like you do... no, it wasn't THAT bad!  I've had much worse.   You're right on the nose about the heparin.  My vein is all bruised about it and when I think of the intense burning going on now I know that my doctor was 100% right that it was the heparin releasing and the blood seeping out of my vein!   A scary thought!

Yet, we don't think this reaction is going to be much worse because it isn't Iodine contraste.  CT contrast is iodine based and that is a KNOWN MC degranulating contrast.   Everybody KNOWS it will degranulate MCs bit time which is why none of us can underestimate our risks with it.  However, this contrast is like the MRI contrast in that it does not have any MC degranulating properties.  

What we believe happened here is that the same pathways which have made me this unique case with CT contrast is what is at work here.  So, it's not really the "contrast" which is the triggering factor, but the pathway that it's working on, which is what is suspected.  It's very confusing and difficult to understand and I myself am not sure I quite get it either.   However, before my masto came out of hiding, I went through 1 CT about 5 or 6 years prior and had no reaction to the contrast.  When my masto came out of hiding with my surgery in 2007, I had a CT with contrast 1 week following my surgery and didn't have a reaction either.  Yet, 6 months later, I did!  The reaction was mild - flushing and diarrhea, but it was a reaction nonetheless.  Another 6 months passed and I had to do another CT.  This time we premedicated with common antiallergy meds and I had a severe reaction - freaked everybody out!   My doctor could not believe the changes she saw with her own eyes that 11 months had brought!  She had been with me for the 1st one following my surgery and when she saw the size of my reaction, she was totally taken aback!

Well, after that scare, once we knew it was masto, I asked Dr. Escribano to give me a copy for his REMA protocols, which he graciously did.  I even translated them into Portuguese for him.  I've used them ever since!  when we used them for yet another CT with contrast, they held me perfectly - my pressure didn't spike and stayed normal the whole time - no flushing, no reacting!   However, a year later, when I had to face the CT again, this time they could not hold me and I reacted to the contrast.  

There is something more going on with me that nobody else seems to do, Mona!  Nancy apparently spoke with a couple of the immunologists about me and they said "IgG" to her.  Well, without an immunologist to take over here, we have NO CLUES as to what the mechanisms are nor the pathways.  It's not psychological, even though the brain is incredibly powerful and holds a lot of MC degranulating chemicals.  If it is an IgG mediated pathway, then it's not really just the iodized contrast, so your concerns are valid.   However, the real PUNCH here is the idodine and this is what makes my "allergy" to contrasts so dangerous.  Contrast is like a bomb exploding inside me.  However, you make it an idodized contraste and you are talking about nuclear warheads!!!   Do you see the difference?  

The contrast I had on Friday was really a pretty light punch - it took the wind out of me.  CT contrast could do real damage which is why I've chosen to refuse it unless it is absolutely necessary.  

I was holding my own on Friday, sweety and even the doctor saw that.  i don't think he realized what he was working with, but he was concerned and he advised me to pre-medicate for round two.  But, I'm going to talk with my doctors and we'll see if perhaps we don't get an ambulance to sit outside just in case.

Now, as to round two, I've not much choice.  This is a renal scan.  I've been doing some 24 hour creatinine clearance urine testing.  This is a more precise urine test for kidney damage.  I did two within 10 days of each other and the alterations were enough for my nephrologist to ask for this test and a doppler sonogram.  She feels that my surgery has done some damage to my kidneys.   I had some really serious edema in my feet and legs during the worst of our summer heat where my feet balooned up suddenly.  We've ruled everything out but my kidneys and this is where we are finding trouble.  

Due to the suspicions of the masto being part of the trouble for my kidney pain and cloudy urine, that the anaphyalxis mediators are causing trouble, and also due to the fact that I was on a heart/lung bypass machine for an hour, it's very possible that my kidneys have indeed "felt it".  We need to find answers.  

Mona, I don't want you to be frightened for me.  Your reactions of anaphylaxis freak me out, sister!  Your situations are 100 times worse than mine!  REALLY!!!  My situation last Friday wasn't so very dangerous and due to how quickly I can respond to the allegra, I'm in a very good situation!   Do you think you could possible get your symptoms to abate with 1 allegra 180mg?!   Well, I DO!!    My having to take a 2nd about 20 minutes later was very unusual and testifies that it was a stronger reaction yes.  But, I've never had to use an epi-pen and I very honestly don't think I'll need it this time either.   I WILL BE OKAY especially since I was NOT pre-medicated for Friday's exam.  For this 2nd round I'll be chock full of prednisone, allegra, ranitidine, singulair and polaramine!!  

So, I know well these protocols and I trust them with my life!   They held me through every single procedure I've gone through including my open heart surgery.  Granted, for that surgery they had to keep me pumped up with meds, but I still got through it!!   This will not be a problem!!!

So, be at peace!!!   I'll be FINE!   I PROMISE!!!

Is Heparin bad for people with mast cell issues?  This is what they put thru my port after I get my iv saline for my POTS.  

Lisa ~ I am thinking of you and hoping the 2nd round goes better.

Take care,
Kate

Lisa
"this contrast is like the MRI contrast in that it does not have any MC degranulating properties.  " I understand that but what Im fearing is that your mast cells will recognize something it didnt like and already reacted to FASTER the next time and with more acute reaction

You know I tried the NAC and I couldnt stay on it cause it made more lung secretions that then dried in place and I would cough for hours trying to clear my lungs.. but all is better now.

I would bet you that your IgG level is very low--mine is and I have the same heparin mast cell kidney reaction.  And youre right about the heparin--most of it should be avoided but the small amount they use as a flush in an IV line is ok to use (or the line tries to clot off).

love ya
me

I don't have near the knowledge that you, Lisa, or Ramona have on this topic but an ambulance on hand sounds good but a doctor on hand would sound even safer to me. Isn't today your test?

Kim

Doctor on hand will have!!!   And I wouldn't be surprised that BOTH doctors will attend!     So, I'm feeling secure about this exam.

I can reassure you, I would not do this exam were it not important.  My nephrologist and the researcher supporting her feel that my original kidney issues are due to the autoimmune element of my masto.  We've proven that my anaphylaxis is causing damage, thankfully slight.  There is what is called IgA nephropathy and this may be what it is, but until recently my doctors didn't feel that it was necessary to investigate because the damage is very slight.  Well, that is until my open heart surgery.  Being on the heart/lung machine is very hard on the entire body and my doctor suspects that this has caused my kidneys damage - perhaps it made the more vulnerable to the anaphyaxis, we don't know for we've not had to investigate this further.... until now.  Yet, to see the kidneys and their function either you do an MRI with contrast (which is very hard on the kidneys), or a CT with contrast, which we know about that contast, or you do the scintigraphy testing.  Since it's considered the best of the three....this is what my doctor chose.  

There was no way to know I'd react to it - it's a complete fluke!  And my doctors feel it was the autoimmune factor at play.  Yet, with me and this suspected form of masto I have, severe cardiovascular reactions and syncope are typical.  So, I got the K-mart Red light special!

During the weekend, in speaking with my doctors and some of the masto authorities, trying to gain understanding for my doctors, I did get an opinion from an authority which I found very intriguing and I asked him if I could share it here.  

Your reactions during the other exams are quite common among patients with mast cell activation disease and they are due to psychic stress caused by the anticipation of the examination. Many people underestimate the unconscious stress induced by those situations. They believe a situation like a technical exam would not bother them, but anyway there is an activation of the "stress system". However, this activation normally has no consequences in a healthy person (perhaps only transient diarrhea or dizziness) but it can trigger the release of mediators from mast cells. You probably know that there is a tight interaction between the stress system and the immune system. One stress hormone always released in such situations is corticotropin releasing hormone (CRH). Mast cells are endowed with CRH receptors and, in fact, CRH is on of the strongest activators of mast cells.
In order to prevent such reactions we always intravenously apply a premedication consisting of prednisone (40-80 mg), ranitidine (50 mg) and dimetindene (1 mg per 10 kg body weight) 30 minutes before the beginning of an exam. This premedication is given prior to every distressing examination, e.g. gastroscopy, coloscopy, bone marrow biopsy, etc.. Generally, an acute episode of MCAD can be prevented by this means.


In considering this reply, it helped to shed some light on a few situations I've encountered, but it also raised more questions with others.  It certainly explains why I flush so much in front of doctors!  They've created in me anxiety and boy do I show it when they've not yet won over my trust and confidence!  

Yet, this situation was most definitely not this, not even unconsiously.  The reacting had a starting point and physical proof of the bruising at the site as the reaction began.  I've had situations where I've been surprised or scared and those situations caused strong reactions and they always began in the abdominal/chest regions or with my head getting all foggy and then working it's way throughout my body.  

This was a reaction like drinking a cup of wine where you can feel the changes happening as the liquid does down your esophagus and then begins to spread out from there.  I once took some paragoric - an opiate known for stopping diarrhea.  I could feel the burning and changes going on as the liquid went down and it was only a few drops in half a cup of water and even then, I knew what the trigger was!  

Yet, this is not the first time this "stress anaphylaxis" has hit me without an emotional cause for it and my doctors have witness it.  I've reacted to the effects of medications, not when I've taken them but when they've gone into action.  Laxatives can do this to me hours later when they force the intestines to move.  Hypertensive medication that is meant to quickly reduce your pressure also do this to me.  I even needed Dr. Castells help with this for it really shocked my doctors!  I'd had a hypertensive crisis after standing on my feet for about 10 minutes waiting at my angiologist's office.  My pressure shot up to 230/110 and I was in bad shape!  She had me put captopril under my tongue and after it began to take affect, I went into anaphylaxis!  My doctor thought it was the captopril itself, but any medication like this put under the tongue is going to cause an immediate reaction because it goes directly into the blood stream.  Yet, this only made me react moments later and the reaction was strong!  We confirmed this when we switched to Adalat which is a hypertensive med that is a know MC stabilizer.  It too causes the very same reaction and it's even quicker into the blood stream since it's in liquid form!  The higher my pressure is, the stronger the anaphylaxis!  And this isn't emotional!

Yet, to even prove this I was once in syncope following sedation for a colonoscope and because the anesthesiologist was concerned as to how long it was taking me to wake up she decided to use an antedote to the anesthesia - the instant she injected it into the port, I went into anaphylaxis!!!  She had to give me IV antihistamines to counteract the reaction!  We still don't know if I reacted to the medication itself, or if it was the effect!  We suspect it was the effect since this medication isn't know to have any cases of anaphylaxis connected to it!  


Yet, this statement of Dr. Molderings was wonderfully illuminating for me for it did indeed give me understanding as to some of my mysterious reactions in that when I've not been able to find a trigger and I've known I was nervous, it explains why for me.

So, in weighing all of these factors, knowing my kidneys are at stake here, I've had to already weigh the various options and although the one option is no doing anything at all, I very honestly don't feel that I'm in such danger that not doing the exam is one of those options, ESPECIALLY when we're taking every precaution to keep me safe now that we know.

I WILL BE OKAY, I'm sure of it!

Lisa


by the way, Kim,  I don't know all that much!  I really don't!  I'm trying hard though and that's all any of us can do!

YIPPIE!! YAHOO!! Great news Lisa!