Hello,

As I have been writing, I had flare up of my mastocytosis symptoms.  My docs are now attempting to help me shut this off by trying some new medications.

I had an anapylactic event, and got the tryptase test results back.  It was three times higher than my normal baseline (it was 60, I'm usually around 20).

As part of the workup, they also want me to  take a trip back to hematology.  Just wondering if I will have to get another BMB to check my mast cell load.  I have not had one in seven years.  I have been very stable up to this point, tryptase always ranging fro 15-20.  This is my first major flare up since I was diagnosed.

Just wondering what experiences the rest of you have had with flares?  Have then generally been transitory, or did this usually happen when you had more mast cell growth going on?

Thanks!

Thanks Lisa.  MY, you are a prophet!  The doc actually just gave me Singulair yesterday to try.  Yes, it did help some.  I started with just half a pill at first, as I am so med sensitive.  I was awake all night, but felt fine for the most part with just one breaththrough event around 4am.

If I get any worse, or have more attacks, I am supposed to go the the ER.

My docs are having a pow-wow on Monday about me.  I am doing collections now for prostaglandin and N-methylhistamine which they are sending out for analysis.

So, trying to get to the bottom of this and shut it off.

Mom is doing a little better today, talking more normal.

Hi Jill,

Flares do come and go.  I had a biggie (over 2 years), and am just now feeling better.  My symptoms were the worst at onset, 18 years ago, and then not too bad most of the time until 3 years ago.  I believe most mast cell experts agree that symptoms and severity of disease are NOT correlated.

Having your mother being this sick and possibly declining, is a stressor of the highest order.  It's good to do the testing and make sure everything is stable since you're having increased symptoms.  The other part, increasing meds so you can get through this, is also very important.  Forgive me if I already suggested lorazepam (Ativan) as a possible aid for this stressful time, but I've found it very helpful.  I just lost my Dad, and don't know how I would've gotten through it without that.

Hope your tests show you're stable.  It's very possible that your baseline tryptase is 15-20, but spikes during an episode.  That doesn't mean your disease is progressing.  Even though 60 is a high tryptase, there are many SM people around with tryptase in the hundreds.

Your doctors sound pretty enlightened, and they are doing the right things!  Lucky you!

HOLY COW, JILLY!!!!


I am REALLY sorry to hear about this!!!   But I am glad to hear that they took the right step and hospitalized you to do a full workup and figure things out!!   I hope that the testing will come back showing that things are not as bad as you all are fearing it could be, but instead shows that things are really good instead!!  

I can't help but feel that it's all due to the stress you've been under and not that the disease has progressed.  Those triggers that you've been up against are really strong triggers and they can get you into that vicious cycle of triggering itself.   this is what I'm hoping is the reality and not a progression of the disease.  

Let us know how things go Jilly!   We're here praying for you!!

Ha! you and Josie should compare notes as to how the hospitals end up varying between the systems!!  

Not nice to have two of your girls in the hospital at once!!!   Really sorry to hear about it for the both of you!!   I'm glad Ramona's out!  We don't want an epidemic of masto patients flooding the hospitals now, do we?



Hugs!

So, now I shocked this morning again, but am being released from the hospital, because they really don't think it is life  threatening, and they really do need the hospital beds, etc.  Housed in a room where noise rules were never enforced, and nursing staff did not seemed too concerned about shocking, this may be a blessing for me to leave. No test results back yet.

So today, I will write something special for all my masto friends on what it REALLY feels like to have a disease, as opposed to just studying it.

See my entry:  Sorry for the Incovenience

Hi Ramona,

They did send me home with prednisone script.  I am supposed to begin tapering now.

My tryptase did some significant spikes, that is pretty interesting.  They did keep ordering the tryptase throughout the process, which goes a long way to illustrate just what goes on with this disease.

I run at a baseline of 20.  During the shocking, it went up to 49 (for those of you higher than that, you are my idols, as I can not imagine it coping with it higher than that!).  Then, after some meds, just three days later, now I am at 14!  I am almost at a normal level.  Never been that low before.  So we are all going "what the heck?".  So, it seems that I blow off with real style, and the switch flips, and it shuts off.  Seems like my changes are pretty rapid, hence the reason I feel so AWFUL when it happens.

So now I am trying to figure out what is medication side effect and what is masto.  They think my mediators are causing me neurological symptoms.

Now I am on

Gastrcrom 200 mg, 3 X daily
Prednisone, 20 mg, been on it a 10 days, now starting to taper down
Randine , 2X daily
Loartidine, 5 mg morning, 10 mg at night
Benedryl as needed (4-6 hrs) for shock symptoms

I totally could NOT tolerate other antihistamines, like Allegra and Zyrtec, made me feel absolutly horrible, even worse.  I tried Sinculair, same thing, made me feel tons worse, could not tolerate it.

The reaction seems to initiate at the gastrointestinal tract, can actually feel flushing from there that radiates outwared to my arms and legs, like a warm wave that moves fast when it hits.  Sometimes, if I hit it with GI meds first, it shuts it off before it gets bad.  I feel this warm rush, then feel like I have to go to the bathroom, a little nausea, and then this HORRID sensation of my skin burning, really strong, on the arms and legs.

I get it most at night, just out of waking from sleep.  This is very interesting to me as I used to work in sleep research.  It seems to be associated with my sleep cycle somehow, maybe some kind of cirdadian thing going on.

Anyway, the docs told me they actually thought I got my medicine better and faster to myself than waiting for the nurse to answer my call light, so they thought I'd be better at home.  Problem is, I live by myself, so it gets pretty scarey.  I have to figure out what to do about that.

I am having huge questions about giving the epipen.  So far, have not gotten airway closure,  I just go tachycardic, hyotensive, and pass out.  I was told different things:  (1) Give epipen if you are feeling faint (2) Only give epipen if you have airway closure.

So, what's the deal?  Now, since I am very medication sensitive, and tachycardic when it hits, epi might through somebody like me into arrythmia, on the other hand, if I pass out and my airway shuts, then how will I get a pen in me? I will have to get more guidance on this subtle, but very important point. 

Judging from my reactions thus far, things go up and down quickly.  For me, the whole thing is over in 15 -30 minutes.  Once I blow off, then I get this crazy muscle shaking that lasts 15 minutes or so, feel cold, and then its all done.

I am awaiting my bone marrow tests.  The group doing it is a really good hem/0nc group with new cutting edge testing at their disposal.  They want to pull out the mast cells, then do some molecular analysis to see what is genetically upregulated.  They want to know just what it is that my cells are putting out, and use that to help guide the meds.  I also got the urine collection during that time period.  So this is being examined at multiple levels.  They are trying to determine if this is just a flare up, or if I now have more mast cell proliferation, and if so, what % of the marrow?  I am also going to have upper and lower endoscopy to figure out if there is more extensive mast cell involvment in the gi, as symptoms seem to initiate there.  As of three years ago, I did not.  They want to know if that has changed.

So that's where it is at right now.  But all the meds and mast cell mediators are giving me a lot of awful symptoms and sensations.  Like today, I felt low blood pressure in my neck, ears ringing, etc.  Then, it sort of subsided.  Comes and goes.  So does the "burning skin" sensation.  I just totally hate how I feel right now, creeps me out.  Sometimes, I feel like I am about to drop dead.  It's always been like that with me though.  I don't get it often, but when I do, it's a major blow out.

I've already lost eight pounds in the last couple of weeks.  I am working hard on keeping my weight stable and hydrating.  Supplementing my bland diet with Pedialyte, lots of water, and Ensure.

OK 2 more suggestions... how about Prilosec for one month to stop the bile production and help calm your tummy?? Also when I react, I take one extra 300 mg zantac (They would give it to you IV in the ER anyway).. and it helps
Love you
Ramona

Hey Jilly, I'm sorry you're going through such a hard time right now!  Gosh I wish I had some way to help you!!!   I can understand why you were released, some of us really don't go through the really horrible, life threatening episodes and it's not like we're IgE mediated anaphylaxis.  We have the "POTENTIAL" of having bad reactions, but it's not always our situations.  Mine can get bad, but the majority of them are manageable with my antihistamines.  I'm forever going through syncope here at home and the only time I really need hospitalization is when I have some kind of medical procedure which is the trigger behind the reaction - that's when the potential for vascular collapse is worst.  

Yet, YES, you should be using your epi when your pressure drops with these situations.  Dr. Escribano's protocols also indicate this usage.  

As to the medications while in the hospital.  I had trouble with this and the nurses and other doctors didn't like that I had my own stash cause they didn't like that they couldn't control it!!  But I did have it and it was with my doctor's full knowledge and instructions to use it when i really had no other choice.   At first I didn't use it, just to see how things would be and they realized that it was true, they couldn't get it to me in time!   Then they didn't gripe with me so much when I had to use it and then inform them for they saw that it was lightening fast and that I'd have been in trouble had I waited upon their system!!!   Got to the point where the girls were saying, Lisa, where are your meds??!!!

We masto patients MUST INSIST on having access to our own meds while in hosptial because there is too much to make us nervous and anxious while hospitalized and this only makes us prone to triggering!!!!   I'm a firm believer of medicine to help make us sleep at night and also a QUIET atmosphere as well as meds at our bedside!!!   I wont'do it any other way again!!

I hope you're better!!!

Lisa