The dying season

Things die in the fall. It is both my favorite and the worst thing about this time. The scientist in me knows that these little deaths nourish the environment so that one day all of this can be born in me. The mast cell part of me is reactive. We don’t call it Shocktober for nothing. And the human part of me is sad. It’s hard to find the same beauty in stark branches and grey skies and I know that is coming.

We are living through a season in the mast cell community, a different kind of dying season. On a weekly basis for the last two months, I have logged onto social media to discover that yet another mast cell patient has died. There are varying causes of death, including complications of anaphylaxis, organ failure, complications of treatment, and suicide. And it has now been a year since my friend, Ginger, died suddenly.

I have gotten messages from several newer patients asking if this frequency of death is common for mast cell patients. It’s not. They are understandably alarmed at the number of people in our community who have died recently. I am alarmed. Watching your friends die never gets easier. There is no amount of expectation that can blunt the pain. There is no way to prepare.

Spending time in this space feels dangerous. It is not safe to linger here.

I have never stopped being affected by the deaths of mast cell patients I only knew casually. Even if you weren’t close to someone who has died, even if the only link you have is that you both have a common rare disease, you still feel it. You are bonded to the people who understand your suffering. We are part of the same whole.

I have seen a few people express concern that over time, these people will be forgotten. They will not. I remember the name of every single mast cell patient that has died since I joined the community several years ago. I remember the shock I suffered when I learned about each of them. So will all of you.

These people are gone now. There will be no vibrant resurgence in the coming spring. But they were people with lives that touched others and living in the love those people carry is a kind of rebirth. Those people will remember the ways they are changed by having known this person. They will remember favorite things and inside jokes. They will remember goals and aspirations and hopes and faith. They will remember better days.

I want you to remember that the people who are gone can never be completely lost to us. That the things they breathed into the air linger still. That when you breathe, you are breathing them in. This world has been marked by their presence, both physically and emotionally. It has literally been changed in a way that is individual to each person. And because of that, they will never be forgotten. How could they be, when they helped to build this world?

There is a poem about death that has always resonated with me. My instructions for my funeral, hopefully many years from now, include this poem being read.

Do not stand at my grave and weep.
I am not there. I do not sleep.
I am a thousand winds that blow.
I am the diamond glints on snow.
I am the sunlight on ripened grain.
I am the gentle autumn rain.
When you awaken in the morning’s hush,
I am the swift uplifting rush
Of quiet birds in circled flight.
I am the soft stars that shine at night.
Do not stand at my grave and cry;
I am not there. I did not die.
-Mary Elizabeth Frye

Be kind to yourselves. Take care. This season will end.

Somebodies

I’ve been trying to get this post out for a few days. I feel like it’s not finished and sharp in the wrong places but I feel like this needs to be said so I’m saying it now.

When I was first diagnosed with mast cell disease, I was pretty relieved. I had been sick a long time and was so tired of being abused by doctors and called a liar. I could have been diagnosed with anything. I could have been diagnosed with weekly limb falling off disease where every week one of my limbs fell off until I had no limbs left. I needed something to hold onto and a diagnosis had that. (I am grateful to announce that I do not have limb falling off disease.)

I do, however, have mast cell disease. It was a few months before it occurred to me that having mast cell disease might be scary. There wasn’t a lot of information available on it and I didn’t have great journal access then, so I wasn’t able to validate those fears. But I was still afraid. Just a little, at first. And then another several months past and I started having major organ involvement. And I started being afraid for real. This time, my fears were validated.

One of the more common questions I get is whether or not people can die from mast cell disease. I get it a lot from people who are newly diagnosed but I get it from people who have been diagnosed a while. I realized recently that people who have been diagnosed a while only ever ask me this question in private message or email. I’ve been thinking about why that is.

The answer is simple: people are afraid to ask if they can die from mast cell disease in a public forum because, overwhelmingly, the responses are not kind. I am guilty of this, too. Those of us who have been in this community a long time have learned to stratify mast cell patients by level of hematologic malignancy – that is, to separate mast cell patients into those who have malignant forms of mast cell disease (aggressive systemic mastocytosis, mast cell leukemia, and mast cell sarcoma) and those who don’t. Because typically the people who lose their lives to mast cell disease are those with those malignant forms, and those who don’t have them don’t die from mast cell disease. The medical institution views malignant mast cell disease as dangerous and the other forms as not dangerous. Specifically, the establishment touts to everyone who will listen that you don’t die from mast cell disease if it’s not malignant.

But the truth is that’s not really the case, if you think about disease and what it does to a person and all the ways it kills them. It’s true that a person with ASM is not likely to die in the same way as an MCAS patient. A patient with ASM will die from mast cell disease if the thousands of extra mast cells burrow into their organ tissue and destroy that tissue so much that the organ stops working. That’s not what happens in a patient with MCAS. But a patient with MCAS can die in other ways. They can die from anaphylaxis and complications of huge steroid doses and side effects from chemo and sepsis and not being able to afford their health care costs and not having insurance and not being able to face one more minute of the humiliation and desperation that is begging for care from people who don’t want to provide it. All of those things can kill a person, too.

Defining death from mast cell disease along by delineating along the lines of organ failure is disrespectful, unfair and missing the point. All of us who have spent years living inside the data of this disease have done it, including me, and we should be sorry. I am. It has never been my intention to characterize MCAS as less serious than other forms of mast cell disease but I think I did anyway, and whether or not I wanted to do it doesn’t change that. I am sorry for doing this. It is not okay. I am committed to doing better in the future.

Fall is a difficult time for mast cell patients. It’s a lot of change at once. It’s new routines and major environmental upheaval. It’s triggers on crack. Season changes are always hard for us but autumn is harder, I’m not sure exactly why. But in the same way that I associate September with ports, I associate fall with mast cell patients crashing and dying. This year has been no different.

In the last several weeks, we have lost a number of mast cell patients across a variety of diagnoses, to the tune of six in six weeks. It’s painful to even type that. One of them was my friend, an SM patient who died of complications of anaphylaxis. Another was a touchstone in the MCAS community, a young woman who did a great deal to comfort others, and who undoubtedly died of complications of MCAS. Still another died of suicide. These last few weeks have been so, so crushing.

There is a very, VERY good chance that you will live a full life with a normal lifespan as a mast cell patient. But it’s not enough to say that people don’t die from non-malignant forms of mast cell disease because “almost nobody does.” Those “almost nobodies” are somebodies, and they are people with lives and dreams and futures they don’t get to experience. They do not deserve to be lost in the data, digits rounded down to zero.

We owe it to them to remember that they were real and that they were here and that they mattered.

I owe it to them. So let’s do that.

Additional posts on prognosis and disease progression:

Progression of mast cell diseases (Part One)

Progression of mast cell diseases (Part Two)

Progression of mast cell diseases (Part Three)

Progression of mast cell diseases (Part Four)

Progression of mast cell diseases (Part Five)

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 15

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 48

On prognosis and dying from mast cell disease

I am not there. I do not sleep.

 

 

 

Love and rainbows

A friend of mine died yesterday. We never met in person, and shortly after we began talking, she found out she was dying. We met through the mast cell community, but she was living with something far more insidious – a rapidly progressing, heritable form of ALS. She died a little over a year after being diagnosed. She is survived by her husband and young son, and many close friends and relatives.

I found out that she was dying yesterday through Facebook. She posted herself that it would be her last day, as she had chosen to invoke Oregon’s Right to Die. I scrolled through all the supportive posts on her page, all the pictures of her with her close friends, recipes she had left for her son.

I learned a lot from her about grace and how to die a good death. Her openness about her illness, and about the ways her body has failed her personally and those in her life in a larger sense, has been a constant source of solidarity for me. She truly embodied the fact that you can love your life and be truly alive even while your body is becoming incapable of sustaining life. She really taught me that life has very little to do with the things you can do and more to do with feeding the relationships you have. I am grateful to have known her.

Every day, I see articles and posts advocating for people to “turn off the screens” and “connect for real”. If it’s not in front of you, if you can’t touch it, it’s not as worthy of your attention, it seems these people think. If you have a conversation via text message, it doesn’t mean as much as one across a table. One of these posts was in my Newsfeed right below Sherrie’s post that was leaving this world today. I shook my head.

These people who write these things don’t know what it means to have a rare disease. They don’t know the loneliness you feel every day surrounded by people who don’t know what it’s like to live in a body that can go into shock without any provocation. They don’t know the overwhelming sense of belonging you feel when you find someone online like you. They don’t know that having someone say, “I understand, I’m the same way,” in a group online can sustain you and validate you just as much as any in person interaction ever will. It is connecting for real, and it’s not less because it happens online.

Sherrie believed that when she died, she would go back up the rainbow she slid down when she was born. She surrounded herself with rainbows, and it became a metaphor for her larger experience. Some weeks ago, after a few difficult days, she wrote the sentence, “The rainbow is calling me.” I shivered when I read it. I can’t believe that the courage and surrender embodied in these few words are less important because I didn’t hear them in person. That’s not how the world works.

What we are doing here, in this community, matters. Supporting each other and understanding each other matters.

When the harder days come, I will remember Sherrie and that life has very little to do with our bodies. It has to do with love and rainbows.

The Cult of Optimism; or, Openheart

Optimism when chronically ill is like a cult. You just show up one day and decide to be optimistic. Because you are optimistic, everyone around you is also optimistic, and for a while, that makes it seem like things will be fine. Optimism is a reflex when presenting with a protracted, terrible situation. It is a defense mechanism, a sort of emotional shock that allows us to move forward.

But optimism is also a distraction, a slight of hand. It draws your attention away from the seedy underbelly of this type of thinking. When we are alone, we sit with the nameless fears we don’t share lest we shatter this illusion of positivity. You’re not supposed to talk about the bad things that could happen when you’re part of this cult.

A lot of us are worse off than we tell people, sometimes even people close to us. There are words we can’t give shape to. A sentence we type and delete.

Over.

And over.

When things started getting bad, I decided that I couldn’t control what happened, but I could control people’s expectations. I think I will be fine, but that doesn’t mean I will be. The wrong med during surgery, undercooked egg whites, a bad car accident that triggers anaphylaxis. The night is dark and full of terrors, and all that.

Believing I will survive won’t make me live longer. I can’t control that. But I can control whether or not people are surprised if something happens to me, and I don’t ever want them to be surprised. I don’t ever want anybody to say that they didn’t know how bad it was. It’s painful for me to lay it all out for them, to say the words, to share the risks. But not doing this feels like treason.

We live in a world of secondhand information, where people so often don’t remember how they know things. It makes so much of medicine and disease impersonal, removed. When someone wonders about what it’s like to live with chronic disease, I don’t want them to read emotionless facts and statistics. I want them to read this and feel my heart bleeding across the screen.

I want them to know that we’re optimistic while being scared, that being optimistic makes it easier to be alive with a disease like mine. I want them to know that optimism is a sort of bet, borrowing against a future we know might not exist. I want them to know that optimism doesn’t save lives.

A couple of days ago, a friend of a friend died as a result of chronic illness. He had many heart surgeries throughout this life, so many that he was known as Openheart Dave.   He was in this cult of optimism, too.

 

 

I am not there. I do not sleep.

There is this party line that people don’t die from mast cell disease.  I’ve sifted through a lot of data in search of statistics on this topic.  I haven’t found a lot that I find reliable.  Some of the numbers are derived from one or two studies.  Some of the data is really outdated.  If it existed before tyrosine kinase inhibitors, I don’t think it’s particularly useful.

“You are a resource for a lot of people,” someone recently told me.  “You write about dying and it scares them.  You can’t be scaring them.” 
I appreciate what she’s saying.  But mast cell disease is already scary, and people are already scared. 
I have a great friend with ASM.  He has been through several types of chemo and is coming to the end of his options.  Last week, he was told that he was dying.  It was a reality we both recognized months ago. 
I’m sick of hearing how rare ASM is in response to questions of life expectancy.  People die from ASM, and these people don’t matter less because they are the exception.  These are people, with lives and families and jobs.  Their stories matter.  They do not deserve less attention because their prognosis scares you. 
You learn a lot about people from their secrets.  The things we are afraid to speak lest we give life to them say so much about us.  Over and over, from the deepest recesses of their minds, people bring forth to me this same fear: that they will die from mast cell disease.  I feel like the collective consciousness of this community sometimes.  I keep everyone’s secrets, and so I keep this one, too. 
This one is easy, because I have the same secret.
Most people with mast cell disease will not die from it.  But some of them will.  Some will die from aggressive disease and some will die from organ damage and some will die from anaphylaxis. 

When they die, there is this immediate need to identify the ways in which they are different from us.  We cloak ourselves in these disparities, armor against the same fate.  Grief ripples through the community, a few days of remembrance and then they are gone.  These people exist only in our memories, where they dwell forever as incarnations of a struggle we hope we won’t lose. 
Then time goes on and someone says, “Death from mast cell disease is very rare,” and everyone agrees, but all I can think about is those people inhabiting those memories and that maybe with a few changes I could be them. 
Nobody lives forever.
I have never thought of Death as a person.  Death is a place, one we all must visit.  Like a strange customs office, or the shaded part of a woodland path that children invent ghost stories about.  Not bad in itself, just bad because it is unfamiliar.  Bad because those places are inevitably lonely, and we feel lonely when we think about them. 
Losing someone hurts.  Knowing you will lose someone – that hurts differently.
When I was in middle school, my class went to a cemetery to take gravestone rubbings.  I don’t remember where it was, but I remember the rubbing I took.  It was very tall.  It marked the grave of a child and had angels at the top corners.  It had the most beautiful poem on it. 

Do not stand at my grave and weep.
I am not there.  I do not sleep.
I am a thousand winds that blow.
I am the diamond glints on snow.
I am the sunlight on ripened grain.
I am the gentle autumn rain.
When you awaken in the morning’s hush
I am the swift uplifting rush
Of quiet birds in circled flight.
I am the soft stars that shine at night.
Do not stand at my grave and cry;
I am not there.  I did not die.
When I think about my friend telling me he is dying, I whisper to myself, “I am not there.  I did not die.”
Most people don’t die from mast cell disease. 

I’ll see you there

My Nana, my mother’s grandmother, died when I was five years old.  I remember my mother telling me that she had died.  I didn’t understand what that meant.  She told me that when the people we love die, their spirits stay close to us to protect us.  They become guardian angels.  I am grateful to my parents for many things, but one of the things that I most appreciate is the way they treated death.  It was never frightening or scary to me.  It was just a milestone, like marriage or retirement.  It was something everyone did. 

I liked this idea of their spirits being close.  I used to talk to Nana as if she was in the room with me.  I said good night to her when I went to bed.  I started first grade a few weeks after her wake.  I was very cool with the idea of her still being around in spirit, so I would draw pictures of myself doing regular things, except Nana was also there in her coffin, just keeping an eye on things.  It made me feel safe.
My first grade teacher was very creeped out about this.  She called me up to her desk and judged me with her wide eyes and unnecessarily slow speech.  I thought she was a fool.  Obviously her mother had not told her about how their spirits stay close to watch over us. 
My teacher told my principal, who called my mother in for a meeting.  They told her that they were worried about me because I thought Nana was watching me all the time.  My mother explained our family’s beliefs on that.  The teacher and principal were very concerned that I didn’t understand the difference between life and death, even though I had seen my Nana in her coffin.  They reiterated this concern repeatedly. 
“Look, my daughter realizes that my grandmother is not rolling around behind her in her coffin,” my mother told them.  She knew I understood what had happened.  Now she was the one judging.  I don’t know how the rest of the conversation went, but I’m sure it ended with my mother using some curt language and strategic eyebrow raising before leaving purposefully.  I get my righteous indignation from her.
My great-uncle and grandfather died a few years later.  I was fine with death by that point, but my sister was small and had been an infant when Nana died.  My mother read a book to her about a leaf named Freddie who learns about death when the leaves fall off the trees.  It was the first time a book made me cry.  When the winter came, Freddie fell off his branch and onto the ground with all his friends.  It seemed so peaceful.  It seemed quiet and like a relief. 
I told my sister about how the spirits stayed close.  We lay in bed together and talked to them.  We missed these people we loved but we didn’t think of death as something bad.   It wasn’t punitive.  It was just another part of life.  You left here and you went somewhere else.  And then your life continued, in this new place. 
My great-aunt was a therapist.  For my tenth birthday, she gave me a book called “Remember the Secret,” by Elisabeth Kubler-Ross.  It was about a girl who is friends with a boy who falls ill and dies.  But when he dies, they go dancing in the stars together and the little boy is not sick anymore.  It is not a sad book, not really.  I was so happy for the boy that he wasn’t suffering anymore.
I have seen a lot of death in my life.  I have literally watched someone take their last breath. I remember every person I have lost and I never fell out of the habit of talking to them.  I know that they are all close to me.  
I have knelt down and paid my final respects to people who died of old age after long happy lives, to those who died young of illness, to addicts who overdosed, to some who died by their own hand, to one whose heart suddenly stopped at the age of 13.  Some of these losses are harder than others.  Some of these losses are defining, in a before/after kind of way.  We had one of those in my family this weekend.   We lost someone young and my feelings are complicated and messy and it feels like my soul is an exposed nerve ending.  It feels like we will feel this loss forever.
I don’t know where you go when you die, and I won’t until it is my turn.  But I believe that we go somewhere, and that when you die, you are reunited with the people you love and you can all be together again forever. 
Every time I kneel down in front of a casket, I say the same thing: I don’t know where you’re going, but wherever it is, I will see you there. 
I’ll see you there.

On prognosis and dying from mast cell disease


There isn’t a lot of data on death from mast cell disease.  Not real data, with statistics and numbers.  People with SM and MCAS are frequently reassured that they will live a normal life span.  People with SM-AHNMD are quoted an average survival of about 8.5 years; ASM, 3.5 years; MCL, under a year. 
Of those groups, only the survival time for mast cell leukemia is convincing to me.  This is because mast cell leukemia has a pretty homogenous presentation, meaning that it affects most people in the same way.  When a disease is as rare as MCL, it is important that you remove as many variables as possible in order for the data to be sound.  And that’s the problem with the rest of the survival data, to my eyes – there’s just too much variability.  Throw in a patient population as small as ours and you’ve got a lot of uncertainty.
The effects of mast cell disease are highly individualized.  There are several B and C findings, meaning that combinations of symptoms and manifestations are very variable.  The SM-AHNMD group is a good example of this.  This category lumps together many different combinations of diseases, not to mention the stages of those diseases.  Someone with ASM-AML is going to have a very different prognosis than someone with SM-CEL.  Simply averaging the lifespans of these people and quoting this as a life expectancy does the mast cell community a disservice.  It is important to remember this when you are typing “mast cell disease death” in the middle of the night. 
Even though we know that most people with SM die from something else, or that for many people, it is a very manageable disease, there is always the possibility that it will be different for you.  It’s hard not to imagine that you will be in the unlucky percentage of people that have progressive disease, that develop ASM, that have leukemic transformation.  Admonishing people who bring up this concern as “negative” or “paranoid” doesn’t make it less terrifying.  It just makes people more afraid to talk about the fact that sometimes people die from mast cell disease and often they aren’t sure how best to minimize their chances of becoming one of them.
Due to the differences in presentation, it has been difficult to identify markers that definitively indicate prognosis.  A lot of effort was put into looking at various CKIT mutations, not just D816V, to see if this could be predictive.  There has not been statistically significant data that this is the case.
The closest things we have to prognostic markers don’t get a lot of play in the general mast cell consciousness.  We talk a lot about CKIT because it affects treatment, and symptoms because it affects diagnosis.  But beyond the initial workup, we don’t often hear much about the CD2 and CD25 markers.  However, a paper published in 2009, established a link between “immunophenotype,” in this case which markers the cells present, and prognosis. 
This study looked at bone marrow samples from 123 patients with different types of SM, including MCL.  Importantly, they also had a large control group of people who did not have SM.  A solid control group is key to determining that a finding is real.  They defined the patients as either good-prognosis (SM, well differentiated SM, and cMAD, clonal mast cell activation disorder (what we now call monoclonal mast cell activation syndrome, MMAS)), or poor-prognosis (ASM and MCL.) 
They determined that for patients whose mast cells expressed BOTH CD25 and CD2 (ISM/MMAS) or NEITHER CD25 and CD2 (WDSM), prognosis was good.  However, mixed expression (typically CD25+ and CD2-) indicated a poorer prognosis.  They compared it to current markers, like the D816V mutation and serum tryptase, as well as clinical findings, like swollen spleen, swollen liver, skin lesions and white blood cell count.  The expression of markers was found to be a sounder method for estimating life expectancy than any of these.
It’s okay to be scared.  We all know people who have died from mast cell disease.  It is scary to think that we could be next.  It is scary to live under the looming threat of anaphylaxis.  But the good news is that science is trying to catch up.  More people are being diagnosed with mast cell disease, and science is getting better at identifying the ways that we are alike and different.  There is every reason to think we will have comforting data in the future.  We just have to get there. 


Reference:
Teodosio, Cristina, et al.  2009.  Mast cells from different molecular and prognostic subtypes of systemic mastocytosis display distinct immunophenotypes.  Journal of Allergy and Clinical Immunology, 125: (3), 719-726.