I’m sure I’m not the only one who wonders if my disease is going to cause my untimely demise. I already know the risk of fatal anaphylaxis from things like wasp or bee stings and I carry epipens just in case but I’ve felt like I was going to die several times over the years just from degranulation alone. A better understanding of mast cell disease has helped me to understand why I felt that way.
The truth is, I actually may have been close to death those times!
I found a really great pdf that I’m adding to my “MCAS Forms You Need” list, entitled: Thousand Faces of Mastocytosis: Mistaken Medical Diagnoses, Patient Suffering & Gender Implications
In it they have the following to say about mast cell disease and the risk of fatal toxic shock as a result of mast cell degranulation:
In very severe cases of degranulation, the chemical soup generated by degranulating mast cells may lead to flushing of the body and the face, swelling of the eyes, nose and throat (angioedema), choking responses in the throat and loss of consciousness (anaphylaxis).
Moreover, because erupting (degranulating) mast cells dump high levels of histamines, prostaglandins, heparin, neutral proteases, acid hydrolases, chemokines, cytokines, etc. into the interstitial areas between cells, the body also experiences a form of toxic shock (Hermine et al., 2008). In some cases, the toxic shock is fatal.
As if the ones listed aren’t bad enough, in that “etc.” after cytokines there can be dozens of other mediators flooding our bodies all at once!! This is another reason why it’s soooo important to avoid triggers (and subsequent degranulation) as much as possible. It’s not just because it can ruin our day or our week or even because it can cause long term damage to our organs and systems — it can be fatal.
There’s really no cure or effective treatment for that yet (being dead I mean) so prevention is the best policy here.
Good luck and remember, we’re in this mast cell craziness together!