I always feel like I want to preface posts that sort of originated on one of the masto lists with the apology that I'm not trying to pick on people or their perspective, so much as I am taking this over here so as to not specifically engage them in the conversation.
More than a few people with masto have set up google alerts so that they get an email whenever mastocytosis makes the news.
This "story" (actually an obituary) popped up in the past week:
Long story short, it's the obituary of a gentleman who died at the age of 83 due to mastocytosis. It's an obituary, it's written for or by the family to announce his death. This lead to discussion on one of the masto lists I'm on about how frustrating it is that the "article" didn't get more in depth regarding how the mastocytosis killed him...sort of ignoring the point that it was an obituary and not an investigative report on mastocytosis. Now, we don't know how this gentleman died, and we never will. All we do know from reading this "article" is that he lived a full and varied life and died 9 years over the average life expectancy for males in America while having mastocytosis. We don't know how masto killed him, we don't know how long he'd been diagnosed for, but to me, I don't want to know. He lived a long life, longer than average, and I guess to me that is comforting. If this were the obituary of a 24 year old, I'd be more concerned and probably want more answers. but in the end, the point of an obituary is to dwell on someone's life, not their death.
I understand fully that there is a great likelihood that this disease may kill me, just as much as I understand that I may die of heart disease (it runs in my family) or cancer (as I am a smoker - though I'm working on quitting, I swear) or I may die from being hit by a car or something else. Point being, I'm going to die at some point. We all are. The most important thing that we can do as people (masto patients or not) is to learn the adaptive behaviors necessary to live as long (and fruitfully) as possible.
Case in point, I really like tattoos. There's no real information out there on whether or not it is safe for masto patients to get tattoos. The one I have, I got years before my diagnosis. As much as I'd love to add to my collection of body art, I am unsure of the risk. If the risk is death, obviously the risks outweigh the benefits 100fold. So I will not get any tattoos until that risk has been assessed (and not by me personally, but by physicians, hopefully at the TMS conference).
I also know, from anecdotal evidence and empirical evidence that certain things carry a great risk for masto patients (sulfa drugs, opiates, etc) so I avoid them. I don't need to read every story of every person who died to be stalwart in my avoidance of these drugs. When I have to schedule a medical test I run it by either the list or my doctors to see if anyone has experience. If I'm going to attempt to do something new (using a vibration plate for exercise, going hiking where I may get bitten by mosquitoes) I ask people if they have any experience.
I don't focus on the mortality stuff as much for a few reasons
1) This disease, in my experience, is incredibly subjective. I can do things some people cannot do, others can do things that I cannot do.
2) Chances are we may not know the full story. In the case of the man in the obituary, how long did he have masto? Did he know he had it when he died? Was he being treated? Was he being treated properly? Unless we call his grieving family, there is no way to know this stuff. I know one story of a masto patient who almost died during surgery because they suffered cardiac arrest due to the anesthesia. Scary right? It's scary until I include this piece of information: they were diagnosed -post- surgery -due- to their near fatal reaction to the anesthesia. They weren't following any surgical protocol, their anesthesiologist did not know they had a high risk patient on the table...all of these facts came to light after the procedure.
As a mastocytosis patient (and one who strives to be educated on their disease) I carry all my protocols with me (being Dr. Castell's emergency protocol and the TMS's anesthesia protocol) , carry epi pens, instruct everyone around me on how to administer epi, wear a medical alert bracelet, don't wear perfume, don't drink, don't typically eat out (food seems to be my biggest trigger) carry a pillbox with prednisone, atarax, benadryl, singular, zantac and ketotifen in it (it's a sexy pillbox) and just sort of do the best I can. I follow the low histamine diet because it works for me. I slowly add foods to my diet to see if I do or do not react to them.
I feel like it's a double edged sword. On one hand, knowledge is power. As a cohort of people with an orphan disease, much of the information we gather is going to be gleaned from our peers. There's not a lot of money going into research, there aren't alot of interested researchers. I totally get that. But I also feel like there is only so much information I want to fill my head with, especially when it becomes redundant (opiates are dangerous, anesthesia is dangerous) and incredibly depressing. As I said in my previous post, I don't want this disease, knowledge of this disease, to comprise most of my time. I want to learn to live with it, in spite of it.
I am not scared of dying any more than anyone else is. It's not a pleasant thought. But I feel like there is only so much I'm going to learn when researching the how/why of masto patient mortality.
The following comes from the TMS website, to me the bolded part is the most important part. The rest are just guidelines which are optimal to be followed. Obviously I can't be next to a hospital at all times, but I just take solace in the idea that epi buys me time I guess. Cause really, to me, what is the point of being alive if I'm miserable and constrained?
There is great variation from person to person in what is a trigger, and even within the same person. The triggers may change day-to-day - that is, heat may set off an attack on one day, but not on other days. The above list is not complete, but is meant to show the wide range of triggers that affect mast cells.
Some people with the indolent form of Mastocytosis, and/or people with Mast Cell Activation Disorder or Syndrome, have been told by some physicians that they can expect a nearly normal life expectancy, as long as they keep themselves as stable as possible by avoiding triggers and taking medications as prescribed. However, since it is not clear what is causing mast cell activation in patients with MCAD, this may not apply. Mast cell diseases are extremely unpredictable, and some people can very quickly develop acute symptoms that may require immediate medical attention. That is why it is advisable to stay within range of a medical facility, and to carry a written protocol from your mast cell disease specialist for emergency care.