In all types of mast cell disease potentially fatal anaphylaxis can occur, and there are many triggers which can cause it which is why anyone with mast cell disorders of all kinds should probably already have two emergency auto-injectors, extra medicines on hand, a Medic Alert bracelet and also these handy MCAD forms all filled out and up to date and within reach at all times in case of an emergency.
When you have mast cell disease you have to be proactive and take precautions even if you’ve never experienced anaphylaxis because we are at a much higher risk than normal people. Here’s what the experts are saying:
Based on the dramatic increase in incidence of anaphylaxis in patients with mastocytosis, recent studies analyzed the presence of clonal mast cell markers, including D816V c-kit mutation, in patients with recurrent IgE- and non-IgE-mediated anaphylaxis. These studies demonstrated the presence of an aberrant mast cell clone in a significant proportion of patients with unexplained anaphylaxis, or anaphylaxis due to hymenoptera venom. Clonal mast cell disease should be suspected in particular in patients presenting with profound cardiovascular manifestations such as hypotension and syncope in the absence of urticaria.
I have MCAS and I experienced anaphylaxis symptoms after being stung by a wasp. By some miracle I was never stung in my entire life and then suddenly I was stung three separate times over a period of a few months just a couple of years ago. Each time my reaction was worse than the last and it was scary, to be honest.
After I got my diagnosis of MCAS I wanted to know more about the wasp sting reactions and whether or not there was anything I could do about reducing my risk of anaphylaxis. I found the last part of this incredibly interesting:
Again, from PubMed:
Patients with primary MCAS and HV anaphylaxis are predominantly males and do not have skin lesions in the majority of cases, and anaphylaxis is characterized by hypotension and syncope in the absence of urticaria and angioedema. A normal value of tryptase (≤11.4 ng/ml) in these patients does not exclude a diagnosis of mastocytosis. Patients with primary MCAS and HV anaphylaxis have to undergo lifelong venom immunotherapy, in order to prevent further potentially fatal severe reactions.
Here’s the thing: once you begin venom immunotherapy you have to be on it for life or your risk of death from a wasp or bee sting (or hobo spider?!) goes up immensely. You have to be on them, on schedule, regularly, for life. I find it hard to do anything on schedule with this disease!
Still, we do, or did, have a lot of hobo spiders here. We’ve taken care of them coming in the house to terrorize us for the most part but they are still around. I don’t know if they can trigger anaphylaxis, but I assume they can, and I know the bees and wasps who love my beautiful yard can probably kill me, based on my previous reactions.
Spring is around the corner and I am an avid gardener (or try to be!) so I’ll be putting my life on the line out there in pursuit of more flowers. If venom immunotherapy is something I should be pursuing more seriously then it’s time to get together with my doctor and make a decision, I guess.
For now I do have my auto-injectors at hand, as well as my forms and handy dandy MCAD wallet card all ready. I wear a Medic Alert bracelet and I do my best to protect myself from insect stings as best I can. I try not to live in fear of “what if” and rather make sure I’m as prepared as possible so if an emergency does arise, I know just what to do in order to save my own life.