Are you drinking enough fluids?  I can get tachycardia if I'm even a little dehydrated.

It was the ascending aorta.  It's hereditary.  And boy is that a long story!!  To make it short however, my doctors had no ideia of how to judge whether the masto had a role.  Recent research from Harvard proves that MCs do have a role and that the mediators are strongly involved, yes.  So, knowing the size of my aneurysm was near operating size, and hearing my angiologist say she thought she'd never need to operate, I realized that I could no longer keep my head in the sand and I needed the help of higher authorities to judge it properly.

Thankfully I found someone, Dr. Craig Basson who was then at Cornell.  He's an authority on hereditary aortic aneurysms and he about jumped out of my computer into my bedroom saying YES I'll be glad to help!  He's also done research on the involvement of MCs in aortic aneurysms so he was perhaps the only doctor in the world who had the capacity to judge my situation.  Then after he indicated me for surgery, he found a cardiologist in Sao Paulo who could help me.  I also gained the help of a Brazilian cardiovascular surgeon, Dr. Pauo Evora, who is an authority in vasoplegia, and he knew all the very best surgeons here and he guided me through the surgeon jungle.  "It so happened" that the cardiologist that Dr. Basson indicated worked on the surgical team of the surgeon that Dr. Evora indicated.  With this cardiologist's support, his ability to understand the role of the MCs with my aneurysm helped the surgeon and the rest of his team understand the need for surgery and also the role of the masto.  When it came time for surgery, Dr. Evora helped the surgeon comprehend my situation even better and the anesthesiologist had also done some serious study and adapted her procedures to fit my case and so on on June 9th last year they successfully got me through what is perhaps the most dangerous surgical procedure a masto patient can face!


Now, as to the aneurysm itself, I'm still working with Dr. Basson.  He's returned to Harvard from Cornell and his colleagues are the very researchers of the MCs in the aneurysms.  He's also a geneticist and is working with Dr. Christine Seidman of the Seidman Lab at Harvard.  Within a year after I began working with Dr. Basson, my family began looking at their own aortas and we found that ALL of my siblings and 1st cousins have one.  My aunt also died from hers shortly after I began working with Dr. Basson.  So Dr. Seidman decided to take over Dr. Basson's NIH study and transfer it from Cornell to Harvard and modify it to study my family in particular due to this very unusual activity within my family.  It's 2 generations of almost 100% activity, and this breaks the rules of genetics.  Well, it was only after we found that my eldest son has mastocytosis that they began to suspect that the genetic defect of masto is behind my family's aortic aneurysms! They suspect that although all of us may not show masto 100%, that the genetic defect is indeed there and that it is showing itself in the form of the ascending aortic aneurysm!  They've chosen to study us due to this suspicion.

So, how's that for a mind blower????!!!!  

Yet, thankfully, I've found the needed support in time for my family, for something like this requires the help of high level doctors on a long term basis and God led me to the very doctors who could give this to me!!

This is why I've learned so much in so short of time - I've been doing a crash course in med school for the past 4 1/2 years!  All of my doctors have been doing as much as they could with the few resources they have at hand in order to help me.  Thankfully they are open minded doctors who recognized that I could have an important role in my own illness and treatment and they accepted my input and allowed me free reign while they gave all the support they could!  It put the ball always in my court, mind you, and has kept me under a lot of pressure, but, it also allowed me to find the answers I needed to save my life!  They've been more than happy to take the back seat and to learn right along side of me!

So, this is how I faced it!

Long story, and I left a TON of things out!!  But the short of it is, I'M ALIVE AND DOING VERY WELLL!!!!

We masto patients CAN and DO find our answers and find a means of living well with this disease as long as we respect it and work with it.

Hugs!

Lisa

Lisa

thanks so much.  Yes most of me is fairly convinced that I at least have MCAD. The question of how long this flare will last, I do hope it will settle.  I will get the cardiac investigations and presuming that all checks out will definitely try an increase in antihistamines. If I get to my coping limit of itching etc, then I might also try it sooner. The problem is with this steroid taper I'm trying not to change too many things at once so I can see what the effects of the taper are since I had adrenal insufficiency symptoms before. I wish there were magic answers or that it would magically stop but I am THANKFUL that I am breathing on my own, not in the hospital, and able to do little things each day right now.

Lisa

If you have time can you tell me more about how your mast cell disorder initially manifested after the surgery, what medications you went on and the timing of things? What caused you to go into anaphylaxis at home? You said you reacted for 6 months?

Thanks so much! I still have to email you!

Wow, you've opened up a real can of worms!!  It's a long story so I'll try to cut it short a bit!

Prior to my hysterectomy, nobody knew I was sick.  Yet, in retrospect, I can see signs now that before were so indescript that I didn't realize I was sick.  The year prior to my hysterectomy I had vague fatigue.  I thought it was because I'd returned to college and at 45 years of age, you expect to get tired, so although my gyno did some blood testing, everything was normal.  But I have this vague achiness when I'd breath on certain days and I felt just really, really tired, but I kept pushing.  I also had 5 or 6 cases of giant plaques of Hives that didn't resolve with normal doses of antihistamines.  I had to use my husbands prescription of hixizine to resolve them.  Yet they were idiopathic and I couldn't figure out what the trigger was and since I'd never had this happen ever before as an adult, I didn't think too much of it.  I'd have these as a child, but never anything other than one day there, the next gone.  So, I figured it was something similar, but since I couldn't find a trigger, I knew that the dermatologist wouldn't be able to do much either.  The next thing was some abdominal pain, but I figured that this was due to an epigastric hernia that I'd developed with my last pregnancy.  These were all clues and my doctors know this now.  

What led me to surgery was that my 2 fibroid uterine tumors which had been very quiet and stable for years suddenly kicked into overgrowth.  They went from 2.5cm to 5cm within 6 months!  It's suspected in my case that the masto had something to do with the rapid growth.  My original date for surgery was cancelled because I had a sudden case of phlebitis.  I got booted out of surgery when my doctor saw my leg - in the 2 days prior to surgery since he had seen me, it had changed overnight!  The anxiety over it caused my cervix to hemorrhage, which this was found later with a biopsy and proper staining of these tissuesa to have a MC hyperplasia.  According to Dr. Castells, this is indeed something which mastocytosis can do - it's not often seen, however, and rarely caught because it comes and then goes without rhyme nor reason.  And when I look back to situations 10 years prior to this surgery, I can see situations where what I thought was food poisoning was instead full blown anaphylaxis!  Always cheese was involved and I was the only one to get sick even though everybody else ate the very same food!  And the bouts of diarrhea I would have which I always blamed upon food, I can now pinpoint at to signs of masto!!  Retrospect is always 20/20!


With the trauma of my surgery, my masto came out of hiding.  According to Dr. Cem Akin, this is not unusual with masto.  Either it was the medications used with the surgery or it was the physical trauma of the surgery itself which caused the masto to come out.  It came out ANGRY for 2 days following surgery, while I was stil in the hospital I had an allergic reaction to IV pain meds!  I've never reacted to any kind of medication before in my life, and since I've had 3 c-sections, I thought this was very, very strange!  

The rest went well and I was released 4 days following my c-section.  However, during that week prior to my 1st revision with my doctor I began having trouble sleeping.  I'm not a back sleeper and I was pretty uncomfortable and began losing sleep.  With this I began feeling like I was going to faint as I was just drifting off to sleep!  So, this frightened me and I began to fight sleep, which only made everything worse!  After 2 days of getting almost no sleep I called my doctor for I began to have episodes of dyspnea.  This was 2 days prior to my appointment with him, which would be 5 days following my release, 9 days following surgery.  He gave me some medication to relax me, which didn't help a bit and after yet another night of almost no sleep, I went into his office, sat down and proceeded to go into a crisis of dyspnea!  I was so weak from surgery that I barely tottered into his office and when he saw how I was, hospitalized me on the spot!  He ran tests looking at the level of post-op anemia (normal for that stage) and infection (nothing) and then a pulmonary embolism. The only clue beside the dyspnea was hypertension, otherwise there was nothing else that they could see was wrong with me so I was sent down to the main hospital to do an angiogram.  My angiologist was called in for she had been the doctor to treat the phlebitis.  During the exam I began fainting all through it for they asked me to hold my breath and this made me horribly faint and I no longer had the strength to fight the faint and I was GONE!  When my angiologist saw this, she called the ICU chief in and she accompanied me through the angiogram, trying to call me back to full conciousness.  Then when they saw the size of the subcutaneous hematoma which I had at the site of my incision the two of them immediately put me into the ICU!  There was no pulmonary embolism found and I was kept under observation for 2 days then released to a normal room.  They called in another clinical doctor to see what was going on and he couldn't figure it out either.  My case left them all scratching their heads!  I was sent home, only to have this very same situation happen two more times in the 3 weeks following this incident.  My gyno had to call in colleagues to see if they could figure things out and both times he fought with them trying to convince them that nothing was wrong with my head, for they said to him "HYSTERECTOMY!"  and he said, "NO WAY! I know women and this one is not having any pyschological issues due to her surgery!  Something else is wrong!"

From that last hospitalization my doctor kept sending from one colleague to another trying to find answers.  As the weeks turned into months, my symptoms became more and more established.  The stronger I got from recuperating from my surgery, the more evident the symptoms got.  In the beginning there was no flush - I was so very weak and anemic from my surgery, that all color was gone from me.  Now, in looking back, both I and my doctor know that it was by God's grace I didn't react during surgery, however, it was really by God's grace!  The subcutaneous hemorrage was very large and we know that this was due to the heparin release from the MCs, just like the hemorrhage from my cervix just prior to surgery was due to this as well.  This contributed to my being so weak because of the loss of blood.  Yet, because my masto was only coming out of hiding, there were symptoms which took a bit longer to show themselves like the chronic diarrhea and abdominal pain, the reactions to foods like chocolate and tomatoes and cheeses, etc.  

After going to doctor after doctor, we found a few more clues.  An upper GI revealed gastritis!  We figured the stint in the ICU was the cause for this, but it took a good 3 months for the gastritis to show.  I also had a PHmeter done and it showed that I had reflux, but only during the day!  when I had the Manometer done, I not only had episodes of dyspnea, but I also fainted during the exam, freaking out the doctor and me!!  Now that I know how I react, the small tube used to do this exam had triggered a small episode of anaphylaxis!  I am extremely sensitive to invasive procedures and I go into anaphylaxis with them, but at that time, we didn't have a single clue!  We were thinking of physical causes to the problems, not disease!  We also found 3 small gallstones and my doctors pounced on this saying, THIS IS THE PROBLEM and I kept saying NO!! for it was completely assymptomatic!!  So we kept searching!   Meanwhile, my gyno kept sending me to his colleagues, all men!  They all said EMOTIONAL/PSYCOLOGICAL and when his last hope, a gastrologist/surgeon saw me and openly declared that I was " in mourning for my uterus"  he about had a cow!  He decided to take my case back in hand!  Fortunately, by this time I was getting so very reactive that it was easy to trigger me!  Going to all these men doctors also made me anxious for I now was pyschologically traumatized by their accusations and I was so upset with doctors in general that just going in to consult was so momentous that I would end up triggering in their offices right in front of them!  Even with my gyno!  And sure enough, I went in to see him and triggered right there with a bigger reaction.  He saw me turn bright read and then purple due to the fact that the dyspnea had become so strong and clamped down on my thorax and would not let me open up my chest to breath properly!!  I also had one hand turn blue and the other dead white, another proof of autoimmune activity - Raynauld's!

With this he took my various symptoms and researched it.  He raised the suspicion of Carcinoid syndrome and masto and asked for the 5-HIAA test for methylserotonins.   The day before my exam was ready, I had been playing a game of Brazilian Dodgeball during the recess of the children at my school.  It was a great game and after 4 months of a tough recovery, it was the first time I felt well enough to play with the children.  Never having heard of excercise anaphylaxis before I had no idea that this would trigger me!  I didn't go into anaphylaxis right away.  I first had incredible brain fog, weakness, abdominal pain and diarrhea. My husband took me home and put me to bed, so exhausted that I was!  I was extremely cold and slept heavily for 4 hours!  When I woke up I was horribly weak and tired but I had to make dinner, and the effort to stand and make dinner was enough to tip the scales and I went into anaphylaxis!  The dyspnea would NOT stop!  After about 20 minutes of this I told my husband to take me to the ER!  They didn't know what to do because my pressure was high and they couldn't recognize what was wrong so I stayed 3 hours on an IV without relieve going from intense dyspnea to syncope, back and forth until finally the reaction worked itself out.  Yet the next morning I had an appointment with my doctor but I never made it into his office cause the stress of getting dressed and to his office was too much and I went into a crisis in his waiting room.  He put me on an available table in another office and he spent 2 hours running back and forth from his office to where I was to check on me and most of the time - he caught me going from syncope to dyspnea and back and forth again!  After 2 hours of this he sent me to the ER at the main hospital (he's the director of the maternity hospital).  I didn't even get to show him my test results which were negative anyway.  My friend who took me said he was literally pulling his hair out when he saw the results and said to himself "I've sent her to the best clinicians I know but they don't take her case seriously!  I can't get any help here!"  At the ER, he sent a note detailing my symptoms and while talking with the doctor he looked at me and said "You don't look very red to me!"  and then put me into a bed, with another IV, did  a few tests.  He offered Buscopan for the abdominal pain, but they only thing they wanted to give me was a heavy sedative which I refused.  This crisis lasted 4 hours!  

This situation freaked me and my husband out as well as my gynecologist!  Yet, it served to reinforce his theories that there was diesease behind this!  So I went to a hematologist, whose very first question was "So, do you feel mutilated?"  Yet when he examined me and pressed upon my abdomen, he triggered the dyspnea and flushing!  He didn't give much value to the carcinoid theory but ordered a CT with contrast - a barrium swallow.  Since my prior angiogram 5 months earlier didn't cause any reaction whatsoever, I didn't have any suspicion of being allergic to the contrast, so without any pre-medication I took the barrium.  I had within 20 minutes explosive diarrhea and flushing!  The diarrhea lasted a week but thankfully the reaction was no worse.  No tumor found!!

This doctor couldn't figure it out and he sent me back to my doctor!  We got so frustrated with this that my husband and I figured that since the carcinoid tumor was brought up as suspect and it was the more dangerous of the two diseases, that I'd go see an oncologist.  He confirmed my symptoms and suspected carcinoid but he openly said that he couldn't hunt it down!  He said the reaction to the contrast was definitely due to the disease and because of this he wanted me to see a specialist.  So, with this statement we tried a 2nd oncologist.  He said the very same thing and said that it was too dangerous for me to try to work with him due to my reaction to the contrast and he said I needed to seek out a specialist either in Rio or Sao Paulo, but he didn´t have anybody he could send me to.  

So, after googling some case histories of Brazilian patients with carcinoids I wrote to one doctor who told me of an excellent doctor in Rio and he made the contacts for me and within 3 days I was in my now oncologist´s office!!  He is who prescribed Allegra to me as an SOS medication.  It took 7 full months of going through absolute torture before I finally was in the hands of someone who KNEW what they were looking for and gave me something to help.  However, I didn´t begin taking the Allegra on a daily basis until another 4 or so months had passed.  My oncologist didn´t  prescribe it for daily usage, only for emergency use.  Yet my husband saw that it got me out of my crises which were becoming more frequent and it was he who suggest I use it daily.  So, to finally get on daily antihistamines it took 11 months, but that was only 1 pill of allegra 180mg a day!  I stayed on that dose until I finally found my wonderful dermatologist a year after this, 20 months following my surgery!!

The wear and tear of the anaphylactic episodes with hypertension that would go on for an hour or more during those first 6 months took their toll upon my aortic aneurysm! The very first CT showed it was 4.6cm, but that 2nd one showed it was 5.0cm after only 6 months!   However, although the first angiogram is what showed the aneurysm, nobody noticed it for they were too distracted by the large subcutaneous bleeding they had seen from the plastic done with my hysterectomy.  It took another 11 months to find the aneurysm - not a single doctor had noticed it, or they thought I already knew and didn´t mention it to me.  Yet when I returned to my angiologist to see if we could find explanations for the syncope, which had become serious, she took one look and did this !  So, she asked for another angiogram to take a look at my aneurysm and although I pre-medicated this time with common allergy protocols, yet I still had a severe reaction!  (we've since learned that I apparently have some kind of IgG reaction happening here for every single time I have contrast, my reaction is stronger and more severe even with the REMA protocols!  Again, another first situation for masto patients!)   My pressure shot up to 220/110 and I lost full consciousness immediately.  After they brought me back and she and the anesthesiologist went their separate ways, I ended up going into syncope again for 3 full hours this time - NOBODY could get me out of it!  And it freaked EVERYBODY out for nobody had seen anything like this before!!  They tried to get my angiologist back, but they could't reach her and when I sent her an email the following day telling her about what I'd gone through, she put me on prednisone right away due to may ongoing reactions.  I was a total wreck!!  Yet, this situation ended up being a watershed situation for with this my oncologist released me and sent me on to the highest authorities in carcinoid and they ruled out the carcinoid!  However, they gave me a "diagnosis"  of Hysteria because they sent me to a neurologist when I described how my syncope was - it's a semi-conscious state where I'm partially aware of things around me depending upon the depth of the reaction.  This has been studied but only once and it's been found to have intense vasoplegia as it's cause.  It's a very, very, very rare masto reaction and only masto patients undergo this particular form of syncope.  It's not epilepsy - I've already investigated this with Dr. Castell's insistance - the highest authority in epilepsy in Brazil took my case and fully ruled it out and declared it to be syncope and agreed with the literature.   Yet, at the time of this "diagnosis"  I was devastated for although it ruled out the carcinoid, it left me totally abandoned and completely alone not knowing where to go nor what to do!!

Yet when I got home and patched up my wounds I began working with women doctors for they don't consider a hysterectomy as tragic a situation in a woman's life as the men doctors do and they were incensed over the horrendous treatment I'd incurred and they are who began looking in the right areas.  The first, my nephrologist/generalized clinician said, "Let's look in autoimmune, all your testing has only been limited to cancer!"  This is when my ANA came up positive!  I cried from sheer relief for it confirmed what my gyno and I had been saying all along - something was definitely WRONG!  From that point on, I began seriously studying masto and asking thousands of questions and reading as much literature as I could - case histories, scientific articles, etc!!  I've made myself a total pest with the experts for I quickly recognized that I had a case that was highly unusual!  Every finding we got, I googled it in connection with mastocytosis and the more I found, the more rare my case showed itself to be!

Yet, this hard work and study has definitely paid off by saving my life!!  Without it we would never have known even half of what we now know about my case - I would never have gotten the diagnosis, nor understood the syncope and the danger it puts me into, I'd not have known that the masto could be involved with my aneurysm and I would never have guessed my angiologist couldn't take me into surgery or that if she did that I would never have survived it being done here in my small town!  My insistance has without doubt reaped incredible fruit and with every victory, I've regained my confidence in doctors and also gained newfound confidence and self assurance in my own abilities!  With all of this, the fears of the anaphylaxis have disappeared as well as fears for the future and what it holds!  I've faced the most dangerous surgery a masto patient can face, I've faced death, and gained a victory over it!  It has totally changed my outlook on the future and I no longer fear it!  Instead, be what may happen to me, I can accept it regardless, for I'm not emprisoned by my fears of the present!


So, I'm sorry for such a looooong post.  Yet it's such a complicated story and I honestly left out a great deal!!!!   So, I do appologize for how big this is!!  I've hesitated to write my story for people here due to how complicated and big it is!!  Instead, I prefer to relate only some of what I've been through as according to what each patient might be facing.  My reactions are not typical masto patient reactions - most patients don't have such severe reactions.  This is because I'm suspected to have a very rare form of masto, MMAS, which is only recently discovered.  It is known for very severe cardiovascular reactions and my doctors say I fit the profile to a T.  However, it's a very hard form to diagnose since this patient is known to have normal or low tryptase levels.  When our levels are as low and normal as mine are, the amount of MCs found in the bone marrow are very, very few and a special flow cytometry process to purify the bone marrow must be done in order to find the few MCs and then test them.  So, unless my trytase rises and until it does, we must be content to leave it as a very strong suspicion.  It could open up some treatment options if we new, but for now it's fine because the medications I'm on are giving me improvement and really, that's all that really matters!

So, I hope that my story doesn't freak you or anybody else out.  I hope it helps you gain some insight for your own case.  If you want, you may copy this for your doctors if you think it will help them.

As to your doctors and THAT NURSE, she should be FIRED!  My sister is a doctor and she told me that it is NORMAL for patients in pain to be anxious!  Yet, our doctors who see us reacting or flushing and see that we are anxious jump to all the wrong conclusions and it only goes to prove how LITTLE they understand the mast cell and the body and how the body responds to anxiety!  This overemphasis upon stress and anxiety is forcing doctors world wide to miss the boat!!  Yes, anxiety will indeed trigger reactions, IT'S SUPPOSED TO!  Dr. Castells told me once that MOST DOCTORS do not understand the mast cell.  I FULLY AGREE!!  If they were to study the lowly mast cell and gain understanding as to what factors make it trigger, then they would know that anxiety is a major trigger, but that doesn't mean it's a psychological problem!  Patients have a normal amount of anxiety and it should not be looked upon as a symptom unless there are other, accompanying psychological symptoms to CONFIRM that the anxiety is out of balance and thus a factor in the life of the patient.  But due to this blatent ignorance as to how the MC functions, they can't undestand the flushing or other symptoms that will come up with us masto patients!!  Granted, flushing may be seen in a normally anxious patient, but NOT reactions to contrast!  That was pure STUPIDITY and IGNORANCE and I too had that comment done when my pressure shot up to 220/110 with that 2nd angiogram!  It was the anesthesiologist who declared that my reaction was not due to a hematological cause!  (read psychological between the lines!)  Yet, what our doctors don't realize is that due to their own psychological pressure to have answers for their patients and their reluctance to show before the patient that they don't have all the answers, they jump too quickly upon accusations of emotional or psychological imbalances!  They end up causing more emotional scars this way of an already traumatized patient!!!   Although it was hard to hear a doctor say, "I don't know, I can't help you any more"  and then send me packing, it was far more beneficial to me than attacking me saying I needed psychological help because I was "too anxious"!   Honestly, if they had gone through what I'd experienced, they'd be traumatized too and I KNEW it!   And knowing this is what totally undermined all of my trust in doctors!  I at first suspected it was only Brazilian doctors, but after speaking with other patients around the world, I'm convined that this is an international attitude which goes beyond borders!  It's pandemic!

All I can say, is, without a single doubt, YOU DO NOT HAVE PSYCHOLOGICAL/EMOCIONAL PROBLEMS!!!   THIS IS MASTO!   This is what masto patients undergo!!!   I'm very sorry you were so badly treated, for you are 100% correct, we often are much safer trying to deal with our reactions at home than that of going to the hospital!  It's a dangerous disease to have to deal with doctors who don't know what they are dealing with and my advice to you, which I also received from a doctor who saved my life - do NOT do any kind of invasive procedure without first being pre-medicated.  Do NOT do any kind of surgery until you have a diagnosis!  If you must, then presume it is masto and pre-medicate accordingly!   There are no patients who are more allergic than masto patients!   So, in premedicating with the REMA protocols, you can indeed face surgery and your doctors will make sure you are the safest patient in the entire hospital!!!!  


Well ENOUGH!!!!   I've spoken enough for 10 people!  Again, sorry for the size of this!

Lisa

Just an update, I didn't tolerate dropping the steroid level. I had my cortisol level checked and it was one tenth of the lower limit of normal suggestive of severe adrenal insufficiency. So I have to go back up on the steroids. There's a chance they might wake up eventually, but there's a chance I'll need steroids for life.

My reactions have been fairly controlled. Other than, I had a holter monitor and my skin is still red from where the leads were stuck on  and I got blisters on my lips today when I ate supper.

Really hoping better days are ahead.

AHA !
Once again, Lisa's right (Lisa- You SURE you're not a Dr.?
I too got bumps all over my lips shortly after I'd taken echinacea for a bad cold. Also, inside of my mouth felt like ALL of it was burned for over a week, from holding Listerene in it (for a bad crown job). Molting is the perfect word to describe what happens to my son when he used any of the toothpastes w/whiteners. Jeez... are we a bunch of misfits or what?
Make that "mastofits"
lori