Mast Cell Disorders Forum

Mast Cell Disorders Forum
http://mastcelldisorders.wallack.us/yabb/YaBB.pl Welcome! >> Introduce Yourself/Share Your Story >> New User with MMAS http://mastcelldisorders.wallack.us/yabb/YaBB.pl?num=1389366090 Message started by RobbyC on 01/10/14 at 03:01:30

Title: New User with MMAS
Post by RobbyC on 01/10/14 at 03:01:30

Hi All,
Thank you for reading my story. I am hoping to find others here with a confirmed diagnosis of MMAS to see how they manage their symptoms and in the end, anxiety related to having a fatal reaction.

I am 50 years old and in good health otherwise, though also have been diagnosed with hypothyroid (treated with levothyroxine). For most of my adult life I have had either sporadic anaphylaxis reactions, just hives, that I attributed to unconfirmed food allergies. I have also had a long history of contact dermatitis that would come and go. No big deal.

In 2009 I had an anaphylaxis reaction that started out as hives but then I had pounding in my arms and chest and some dizziness indicative of hypotension. I popped a bunch of Benadryl and Prednisone and was getting better by the time I got to the ER. Follow up consisted of a full battery of allergy testing all of which was negative. At the time, Dr Cem Akin was at Univ of Michigan and he convinced me to get a bone marrow biopsy that showed I had the c-kit mutation. I did not have CD2 or CD25 on my mast cells but I had a long, 20 years history of elevated serum tryptase. This prompted the diagnosis of Monoclonal Mast Cell Activation Syndrome.

I have kept a journal since to keep track of my reactions which until 2013 consisted only of period hives to varying extents with limited blood pressure problems that responded to benadryl. Then last year I had a rapid onset reaction that resulted in syncope within about 15 min. My wife hit me with two epi pens and I was conscious when the paramedics arrived and fine by the time I got to the hospital. I upped my emergency meds to include prednisone and ranitidine and had only one flushing and uticaria reaction since that I managed with the meds until last month when I had a reaction that knocked me out in less than ten minutes after popping all my meds and getting one epi in. My wife hit me with another and again I was fine by the time paramedics arrived.

I have no known triggers except possibly NSAIDS though my reactions happen many hours after taking these. My reactions are seasonal, I have never had a reaction from April through August, so I thought maybe it was a cold thing (I live in NY now but MI before) but my reaction last month happened while I was on vacation in Orlando.

Is there anyone here with confirmed MMAS?? Do you have similar symptoms?? Has it gotten worse over the years?? Obviously, my family is terrified and I wonder if people can die from this. I am going to try to get a consult with Dr Akin and my hematologist to see what they think but would appreciate opinions from the folks here.

Thanks,
Rob

Title: Re: New User with MMAS
Post by DeborahW, Founder on 01/10/14 at 14:44:53

Hello. I think they you aren't getting any responses because no one knows what MMAS is. I am actually not clear about it, but I know it is a form of Masto.

I am wondering why you wouldn't go to see Dr. Akin in Boston for additional help? you are already established as his patient from Michigan, so I would think that you could easily get in him to him. He has years more experience than Dr. Afrin, and is one of the leading masto specialists. So, I would suggest reconsidering and going to see him....

In regard to the fatality of Masto, it is not fatal. Those with the disease of Masto Leukemia, which is a different disease than Masto, do have to deal with Leukemia worries and those with ASM (Aggressive Systemic Masto) do seem to be much worse off than the regular SM people. So, I would think that since you don't have those, that you don't need to worry about your longevity.

I have IA - Idiopathic Anaphylaxis. While it will not affect my longevity, I do have to keep my eyes open regarding any actual event of going into anaphylaxis because anaphylaxis can be fatal, of course. So, I take the precautions of always having 2 epi pens with me at all times as well as my normal and emergency meds. It's not a big deal; it's just an awareness that I maintain.

Title: Re: New User with MMAS
Post by RobbyC on 01/14/14 at 02:54:18

Hi Deborah,

Thanks for the advice. There are some 2013 reviews on MMAS I could post links to if that is permitted (or upload pdf files). These reviews also address IA so may be of interest to others like yourself.

And I am going to seek advice from Dr Akin.

Rob

Title: Re: New User with MMAS
Post by DeborahW, Founder on 01/14/14 at 15:33:10

Definitely feel free to post links and info! :). Sometimes trying to post the actual document on the forum as an attachment is problematic as something here prevents it from showing up. I have never figured out why that happens!

Title: Re: New User with MMAS
Post by RobbyC on 01/14/14 at 15:45:57

Sounds good. Here is the best and most recent one from Dr. Castells at Brigham and Women's Hospital. Let me know if the link doesn't work and I can try uploading the pdf. There is a ton of useful info in here for anyone with a mast cell disorder.

http://www.clinicaltherapeutics.com/article/S0149-2918(13)00171-9/fulltext

Title: Re: New User with MMAS
Post by brigitte on 01/14/14 at 16:24:27

Thank you for the link! I've been looking for information on Xolair for MCAS and haven't found anything until now!
I am in the process of being qualified for Xolair (omalizumab). It doesn't list it as a drug for mixed brain syndrome (which would be nice :)) but who knows with these disorders. We're all so different, different drugs can help (or make worse) certain symptoms. I blew up like a blowfish with angioedema from ketotefin! Yikes! That was scary!
If anyone has any info or experience with Xolair, I would love to hear about it!

Title: Re: New User with MMAS
Post by sarahkay1111 on 02/06/14 at 07:44:47

Hi Rob,

I don't know exactly what I have, but I have the anaphylactic episodes. I think I'm at about 20 now in the last 3 years. I have been diagnosed with Mast Cell Disease and Eosinophilic Esophagitis, Raynaud's, and a heart arrhythmia. I get very high b/p and heart rate, throat swelling, faintness, and tremors with reactions. I take Benadryl and sometimes prednisone with reactions to help stop them. I know that once I have a big reaction, I have to be very careful for the next few weeks, or I get in BIG trouble again. I also have almost all of my reactions in November through March. I hope you are getting the help you need and feel good :) Sarah

Title: Re: New User with MMAS
Post by RobbyC on 02/28/14 at 03:57:14

Hi Sarah,

Sorry for the slow reply. Sounds very similar to my MCD. Have you ever had a bone marrow biopsy and had your mast cells tested for the c-kit mutation? Do you have chronically elevated tryptase??

Since my last reaction I now take Zyrtec, Zantac and Singulair every day a (knock on wood) haven't had a reaction.

Best Regards,

Rob