Hello all.

My name is John and I live in St Louis, MO.  Was diagnosed systemic masto via BM biopsy mid february. Unlike many, the journey to dx for me was a short one.

My story goes like this:
I was an athletic, healthy, fit, active 47 yr old guy. Have always done something to stay in shape, martial arts, running, cycling. October 2010 while on a retreat I had a heart attack. No diabetes, no family history, no high cholesterol, not overweight, at the time I was cycling about 100 miles a week. No regular meds - only asthma rescue inhaler.  LAD was 90% blocked with other diffuse blockages in small vessels. Got 3 stents in the LAD. 10%damage or less to the heart. Started daily beta blockers, statin, plavix and niaspan. All was OK - had chest pains but all tests were negative. Did not even have much of a problem with flushing with niaspan, very common side effect.
September 29, 2011, I went from normal to unconscious (anaphylactic shock) in about 4 minutes, could not breathe and had a heart rate of about 200. Had another heart attack secondary to the anaphylaxis - lack of oxygenated blood to the heart. 911, EMT's, my doctor lives next door - and all thought it was a another cardiac episode only. Finally got to the ER, still no one put the pieces together. I have snapshot memories of 5 seconds every 5 or 10 minutes for the next 2 hours. One perplexing thing is I was never treated for anaphylaxis but came out of it anyway. Doc says that is unusual.
After another cardiac cath, cardio doc says heart is ok w/minor damage but no additional blockage and put together the pieces and says severe allergic rxn. I have never been allergic to anything but tetanus toxoid. So begins the battery of tests. Allergist calls and says your tryptase is elevated at 25. (yes - I know this is low in the masto world)- I am referring you to Washington University. Did another tryptase - 24, and scheduled biopsy. Biopsy results show that I meet the major and all minor WHO criteria for systemic masto. So we started the h1, h2, PPI, aspirin, and singulair.
Have had a 2 minor flushing episodes but benadryl cooled them off w/o the need to resort to the epipen.
Symptoms: No skin involvement, minor gut problems - mostly nausea, no enlarged spleen or liver. Occasional bad-assed fatigue, and extremely slow recovery from exercise.

Will close with a couple questions:
1) Any possibility of heart disease being related to the masto? I have found nothing significant in my research. You guys heard of link?

2) Is there any monitoring protocol for progression other than watching symptoms? regular tryptase tests?

Regards

John

Hi Rocky and welcome to the forum!  Sometimes a major health event will bring a mast cell disorder out of hiding.  Does anyone else in your family have anything like this?

Ana has pretty much said everything.  Was the tryptase mature tryptase or tryptase within an hour of anaphylaxis?  While 25 is not very high, it is a good marker for following the SM.  If it stays around 25 or increases very slowly and your CBCs are normal, not having many symptoms, then you probably are in the indolent category.  In that case, progression would be unlikely.  It's very rare anyway (under 5 % of patients progress).  Have carcinoid syndrome and pheochromocytoma been ruled out?  It's unlikely that you have either, but they should be tested, if they haven't.  Some doctors do other annual testing.  There are some posts here that talk about tests.  

You should have a bone density test, and I think that's every other year.  SM can cause bone loss, and you don't want to wait until you have a broken bone to find out.  Since it's not typically done on men, you might have to ask for it.  Bone loss from osteoporosis can be reversed with weight-bearing exercise and calcium supplements and, if needed, medicine.

The doctor might want you to also do a bone survey (a set of x-rays) to assess other aspects of possible bone changes.  My doctor also has ordered a chest and abdominal CT to look for organ enlargement, but you may have already had that.

Were you checked for the C-kit mutation?  That's potentially important if at some time your symptoms were worse, as it would dictate which kind of treatment would be helpful.

Do you mind sharing the name of the doctor in St. Louis (Wash. U.) who diagnosed your SM and what his or her specialty is?  There are others in that area who are looking for a doctor for testing and follow-up.  I grew up in the St. Louis area.  I like going back there.

It would be a good idea to look at the emergency protocols posted on this website and at www.tmsforacure.  Should you ever need another medical procedure, you ought to know about pre-medicating for it.  We are advised to take antihistamines and prednisone before any procedure to avoid any reactions/anaphylaxis during a surgery, etc.  This is very important in order to be safe.  It's always critical to talk personally to any anesthesiologist, too, before a procedure to ensure he/she knows what medicines should be avoided and which are less likely to cause problems.  There's a list in the emergency protocols that is a guide for your doctors.

Another list to look at is the one that lists many different meds and which are safer for mast cell disorder patients.  The ICUS website has a version of a low histamine diet that might be helpful in sorting out what triggers your symptoms.

Yes it sounds familiar Rocky. Though, my exercise tolerance plummeted to being barely able to walk for a period and now just getting back into slow walking and biking. I'm not sure which parts are mast cell, orthostatic tachycardia (POTS), adrenal or all of the above. I get a lot worse if I overheat and need to stay very cool during any exertion.

I also have the confounding variable of tapering off steroids.

Interesting that you are on Aspirin. Do you think it's helping? I've always wondered if it would help me but am apprehensive given that some have bad reactions to it. Do you think you are feeling any better on the med cocktail?

Yes fatigue may be multifactorial, but yes I have that ++ and it was one of my first symptoms. Need to sleep 10-12 hours a night right now.

That is so wonderful that you are back to work Peter!

Thanks for all the info and encouragement. I have been trying to listen to my body's response...but it usually has to scream at me for me to hear. But I think I am getting better. It is mostly adjusting to a "new normal" which is very difficult for an all or nothing type.
Ana - I dont know if the aspirin is helping or not but a whole aspirin is part of Dr. Wedner's standard drug regiment, IF it is tolerated. The purpose is for mast cell stabilization - but you probably already know that. Cardio doc was going to cut it back to 81mg daily; Wedner said stay at 325mg - baby aspirin works fine for platelets at the lower dose but the action of aspirin on mast cell membrane requires a full aspirin.

The only med that I am on that I know is working is prozac! The rest of them I am still trying to figure out if they make me feel better or worse. Time will tell.

Many thanks folk. I will be camping and fishing for the next 10 days so I will not be on here. This ought to be an interesting trip- my first post-dx.

John