I think a lot about mast cell disease. I’m sure this doesn’t surprise anyone. I’m a scientist; I like to collect the data and identify the patterns, see if I can place myself into one. Thinking about things a lot is how I process my world. If I can learn everything about it, I can never be unprepared. It’s not completely true, but it’s soothing. It has kept me alive this long.
The term “mast cell disease” encompasses several diagnoses. At present, it includes solitary mastocytoma, systemic mastocytosis (indolent, smoldering), systemic mastocytosis with associated clonal hematologic non-mast cell lineage disease, aggressive systemic mastocytosis, mast cell leukemia, cutaneous mastocytosis, mast cell activation syndrome, and all of the various disease subcategories, like TMEP and so on. That is quite a spectrum. Then you have to consider that within each of those diagnoses is a continuum of symptoms, treatments, survival, people. Every case is individual in a way that you don’t always see with other diseases.
I actually wonder a lot the extent to which these diseases are related to each other, and if they are all related more to each other than other diseases. Like if mast cell leukemia is more of a mast cell disease or a leukemia, that sort of thing. If SM-AHNMD should really be an umbrella term when the particular blood disorder affects prognosis so much. These things remind me of a phenomenon found in nature, in which animals look alike but are not related. An example of this is the now extinct Tasmanian Tiger (thylacine), which looks similar to a dog or wolf, but is in fact more closely related to kangaroos. Do we all just look like each other, because we haven’t yet found anything that makes us look more like something else?
It’s easier to see how we’re different. And our differences are what we tend to focus on. We tend to place ourselves into a hierarchy of severity, and where we place ourselves is indicative of our relationship to our disease.
If you put yourself at the bottom of this order, the least severe, it is often because you are afraid. Some people are afraid to admit that they are sick; they prefer to think of everyone else as much sicker. Some people put themselves toward the top, the most severe; they think their symptoms are worse than everyone else’s, that they will die soon. Maybe they will. To be sure, there are some conditions with much worse prognosis than others (MCL and ASM being the most severe). But I don’t notice this behavior much with people having those diagnoses.
I notice this behavior mostly with people who have the same prognosis: a relatively normal life expectancy. People with indolent systemic mastocytosis and mast cell activation syndrome are expected to live long lives. This might seem impossible to some of you, but it’s true. While it’s reported as being a little shorter in some studies, it’s largely normal.
There is a certain fluidity to these diagnoses. I know a few people who were originally diagnosed with MCAS, only to later be diagnosed as SM. There are certainly people who used to be SM and became SSM or ASM. People with SM tend to think their disease is worse; people with MCAS tend to think theirs is. They’re all right. Whatever disease you have is the worst, because it affects you and your life. Perception is reality. If you perceive the world through the lens of ISM, then you tend see all the ways it affects you. Or SSM, or CM, or whatever. And you see that as being more terrible than anyone else’s experience.
This behavior is divisive. I don’t know a single person involved in the mast cell disease community who hasn’t experienced this. It is the conversations that go like this:
A: “Does anyone get a rash after they shower? I get a rash and it’s freaking me out.”
B: “Yes, I get it when the water is too hot.”
C: “Too hot? TOO HOT?! I have to shower in water with ice cubes, and haven’t touched warm water in thirty years.”
We have ALL had conversations like this. Let’s look at what happened.
The person asking the question was concerned about something happening in her life. The second person answered the question neutrally. The third person was likely frustrated about their disease in that moment, or frustrated that other people with similar conditions have comforts that they don’t. It is okay to be frustrated. But when we have conversations like this, it confuses the issue and damages the sense of community.
Let’s say the first person was newly diagnosed. This person is probably scared and looking for some guidance. They have found a community of people like them and are hoping for some help. After two responses to their question, they see that people one-up each other and also might think they can never use hot water again. I know it sounds dramatic, but it happens. I promise you, it happens.
I saw a comment not long ago about how MCAS is often considered less serious than SM. I agree that this is the general thought, and I think it’s inaccurate for a few reasons. The first is because MCAS generally accompanies another condition. So while the MCAS may be less serious than another person’s SM, the overall health of the person with MCAS may be worse. There is also the unignorable fact that people with MCAS don’t get taken as seriously as those with SM. As I mentioned before, all of these diseases are just so variable.
One thing that varies a lot from person to person is how you define “serious.” Do you mean life limiting? Likely to kill you? Or likely to prevent you from living the kind of life you want? Is it better to live until 40 and have a good life, or live until 80 and spend 25% of your life in the hospital? The answers to these questions are not uniform, and for that reason, there can never be a “worst” mast cell disease. There can only be the disease with the highest mortality, with the least mortality, with the least frequent occurrence of anaphylaxis. Because the kind of disease you have is the worst to you. And that’s okay.
We also tend to use various facts as markers for disease severity, like frequency of anaphylaxis, ability to work, or need for chemo. If you look at me based exclusively on whether or not I still work, and how many hours I work, you would assume my disease was mild. You would be wrong. Many people consider SM to be more severe because chemo is sometimes prescribed for it, but then there is the fact that chemo drugs are now being prescribed, with good results, for people with non-proliferative mast cell disease, like MCAS.
The fact of the matter is that in the end, we are more alike than we are not. We mostly have the same symptoms, in varying combinations; we mostly take the same drugs, avoid the same things, carry Epipens. There is a lot of power in this fact. There is power in our fellowship, in the fact that we can work together to support each other and force the world to recognize mast cell disease.
Let’s say that there are about 200,000 people in the US with mast cell disease. (I am estimating here.) The overwhelming majority of this number will live long lives. Say that’s 90%. So there’s 10% with life-limiting disease, which equals 20,000.
There are about 560,000 licensed and practicing physicians (MDs and Dos) in the US. If those with life-limiting disease needed to educate all of those doctors, each person would need to talk to 28 doctors. But if everyone with mast cell disease contributed to this effort, each person would only need to educate 3 doctors. That sounds possible, doesn’t it? Could you educate three doctors? I bet we all have more than three doctors.
We are more powerful as a community. Our collective knowledge is massive. And we are educating people. Doctors are learning about us; sometimes by force. It’s an arduous task and impossible to do it alone.
Maybe we do just look alike. But we are more alike than different. And it might be frustrating sometimes, and we might all get irritated with each other, but for right now, we’re in this together, and that’s really all that matters.