my feelings

Event zero


When I was 13 years old, my friends and I worked in an attraction haunted house.  I spent a lot of time running through the dark, foggy halls of an abandoned war fort, in costume, scaring people.  Eventually I slipped and hurt myself.  It felt like I had twisted something out of place in my right leg.  I didn’t know it then, but this was the beginning of an era that would consume a significant portion of my life.
I saw my PCP, then an orthopedist.  They both assumed the ileotibial band that sat atop my hip was weakened and that this had been injured in the fall.  I received some steroid and anesthetic injections into the muscle and was sent on my way. 
Over the following months, it became harder for me to walk.  I felt pins and needles in my legs, and the muscles felt tired and weak.  My doctors were unimpressed.  They gave me crutches and told me there was nothing seriously wrong.  This continued for six months until I sat down in school one day and was unable to stand up.  I could no longer feel my legs.
I was seen at the local, very famous pediatric facility.  No one knew what was wrong with me.  They didn’t order any tests.  The doctor pulled my mother aside and told her that they were holding a bed for me in the psychiatric unit.  Because I had conversion disorder, and was faking. 
I spent the next few days taking very high dose anti-inflammatories and laying around at home, unable to go anywhere.  After five days of this, I woke up in the middle of the night and got up to go to the bathroom.  I was halfway down the stairs when I realized I was walking.  The following Tuesday morning, I had an appointment with another orthopedist, who ordered an MRI. 
In my fall the previous October, I had fractured my L3 and L4 vertebrae.  The bones had crunched against each other and the resulting fragments had attracted fluid, which pressed on my spinal cord, functionally paralyzing me.  The high dose anti-inflammatories had alleviated some of the pressure of this fluid, restoring conduction and my ability to feel and use my legs. 
All of this makes perfect, medical sense when you have the whole story.  The fact that there is an explanation should make me feel validated.  It doesn’t.  No matter how hard I reassure myself that I will never again be a 14 year old with temporary paralysis, I cannot remember these things without fresh fear that I will be committed and shame that the doctors thought I was lying. 
This was my event zero.  Every person with a long term relationship with the medical establishment has one.  It is the first time that a medical professional wronged you in a serious way.  For me, it was the first time I was accused of lying about my health.  I will never forget it. 
Most people with rare diseases are accused of lying for years before they are diagnosed.  It is easier to accuse someone of faking than to acknowledge that you don’t know what is wrong with them.  I was accused of looking for attention, trying to make myself more interesting, both by medical professionals and people in my life.  I was accused of literally making myself sick, poisoning myself to skew my blood work.  I have been called a compulsive liar and a drug addict.  I have left appointments countless times crying and without the help I needed.  I have been literally shamed by doctors for faking while sick with a rare disease.
You don’t live with that for years and emerge without scars.  Every time I have an appointment, I go in with a secret. 
My secret is that I’m not a liar but I think that you think I am. 
My secret is that I will never not think that you think I am lying, because your profession has taught me that I am not worthy of your trust. 
My secret is that even if I trust you rationally, I still think you might tell me I am faking. 
My secret is that because of this, even though I have dozens of tests confirming my diagnosis, I sometimes wonder if it is all in my head.  But if I say this out loud, other people will believe it, too.
When you live in a chronically ill body, there is no mistaking that you are sick.  It is as plain to you as the color of your eyes.  Let’s say you have blue eyes.  Let’s say we live in a world where in order to get a driver’s license, you need someone to confirm the color of your eyes.  Let’s say you ask every doctor you see for several years what color your eyes are.  Some tell you brown, some tell you green, some say they might be blue in a certain light.  Some say you have no eyes at all.  Some say your eyes aren’t worthy of a color. Some say they don’t have time to tell you what color your eyes are.  But you know your eyes are blue, all the time, every day.  You know because you see them.  You know because they are your eyes.
But it doesn’t matter, because what you see doesn’t matter.  It only matters if they see it, too. 
This is called gaslighting, when people deny obvious things.  It makes you feel crazy. 
Our secret is that those of us with rare diseases are repeatedly traumatized in our pursuit of answers.  It is a trauma that is never treated and never acknowledged. 
Our secret is that many of us have PTSD from invasive procedures, from emergencies, from near death experiences.
Our secret is that we have been mentally abused, but everyone believes the perpetrators instead of us.
Our secret is that even if we know we aren’t, even if we have definitive proof of our illnesses, in the very bottom part of our souls, we feel like liars. 
You can never unfeel these things.  You can live around them, but they are always there.  They will always be part of us and how we see ourselves.  We can only ever be so empowered in getting treatment for our diseases.  We can only hide these fears so well.
There is a psychological burden that comes with being chronically ill.  It can be as crippling as our physical symptoms, and this part of it is created by the medical establishment. 



Cowardice


I had a whole life before I got sick.  I had jobs and relationships and friends.  And a temper and a bad attitude and a desire to always be right.  I was a complete person, with personality traits like everyone else. 
This hasn’t changed since I got sick, but I find that people tend to focus less on this.  They see me through the lens of my disease and they attribute all of my actions to the sickness.  It has surprising consequences.  The one that is most obvious is how strongly people in my life react when they perceive that I have been treated unfairly.  It feels more wrong to them that these things happened to me than if they happened to them.  The amount of anger they emote is sometime disproportionate to the offense, and even when they realize this, they cannot always control their response.  Feelings are complicated in that way. 
I find that they get the angriest about people who end their relationships with me.  In some ways, they feel the loss of this support as keenly as I do.  They love me.  They want to limit my suffering.
It is hard to be friends with someone who is chronically ill.  It can be time consuming and tiring.  It takes a lot of physical and emotional energy to support someone like me.  I’m sure it’s not always pleasant to drive me around after you’ve been working all day.  It’s also hard when you’re not able to get the favor paid back in kind – I am certainly in no position to come vacuum your floor, at least not anytime soon.  But more than these mundane things, being around the chronically ill reinforces our own fears about mortality, about the frailty of the human body.  It reminds us that we could all get sick and lose the things and people we care about.
We, the chronically ill, are afraid to lose the people we love because, statistically, we will.  Our partners will leave us, we will lose close friendships.  The stress of these events makes us sicker, but it is the emptiness, the guilt that we feel that is harder to cope with.  It’s hard to convince yourself that you’re not ruining everyone’s life when people you love and trust tell you that you are. 
I think a lot lately about the people who have cut ties with me.  It is sort of comforting to think that maybe it was because of my personality, because we just grew apart, but when I really dissect these situations, that is never what happened.   These people didn’t want to deal with me being sick.  Some started behaving callously, ignoring me when I was upset; others stopped returning my calls; others just outright got mad at me.  It’s easier to be mad at someone than stay with them while they deteriorate.  This isn’t for the faint of heart.
There is this recent concept in the American consciousness that you should never have to do anything that you find upsetting, that feeling anything bad is not your responsibility.  This is bullshit.  Sometimes you have to do things that are hard emotionally because they are the right thing to do.  You can’t just abandon people because it makes your life easier.  I’m not advocating total sacrifice here – self care is important.  But there is a balance to be struck in these situations, and I know there is because many in my life have done so.  The difference between them and those who leave is how important I am to them.  If I’m not worth the pain, it’s an easy choice.
You do not have to continue to be my friend because I am sick.  You do not have to continue to be my friend at all, if you don’t like me.  Someone said to me not so long ago, “I’m not a bad person because I can’t deal with your sickness.”  It doesn’t make you a bad person. 
But it does make you a bad friend, and I’m tired of pretending that’s not the case.  Be honest with yourself about your motivations.  We know when it’s about us as people and when it’s about our disease.  If you don’t want to be my friend anymore because I made out with your boyfriend and get embarrassingly drunk at your parties, that’s fine.  If you don’t want to be my friend anymore because I’m sick, that’s wrong.  This disease picked me.  It’s not a behavior or life choice that I made.  It is something that happened to me that I can’t control. 
It’s not fair to lose the people you love because you got sick.  And I’m tired of letting it happen gracefully.  The truth is that if you end your relationship with someone because of their disease, you are a coward.
I suppose things work out the way they’re supposed to, though.  Goddess knows I don’t have room for cowards in my life right now.


Purging


I was in love when I was diagnosed with systemic mast cell disease.  I was in a very stable, long term relationship with someone who rubbed my back when I threw up and commiserated with me when I slept for days.  I knew the statistics about chronic illness ending relationships but I never worried about it.  Everyone thinks their love is exceptional, and I was no different.  It didn’t even occur to me that I might end up alone.
I did.  It fell apart fast last spring, when I needed a lot of help.  It was shocking and painful.  I hadn’t expected it; I wasn’t ready for it.  I had a brand new colostomy and a broken relationship that never recovered.
This last year was the hardest of my life.  I tried hard to salvage, to find the love we had once had, but it was obvious that I was the only one who wanted that.  My disease was too much for him.  And this whole life that I had had, with its hopes and dreams for the future, eroded before my eyes until there was nothing left but old cards and pictures stored in a box.  I was alone. 
We forget sometimes that having a partner doesn’t always mean sharing the burden.  It means having help, whether or not you use it.  But you need to be able to take care of yourself.  It’s harder that way, and lonelier, but in the end, it is your own responsibility to stay alive.  There is a grim satisfaction in the fact that I am still alive, in no small part to my own force of will. 
I will never be as naïve as I was before.  I will never again think I’m exceptional in this way.  This stuff is hard.  Not everyone is cut out for it.  If I had a choice to deal with it, if the roles were reversed, knowing that I would have stayed is small consolation.  It feels like losing.  It feels like mast cell disease robbed me of something that I loved so much that I have to remind myself that the disease didn’t really do the robbing. 
This year has forced me to be tougher, work harder, be smarter about my disease and my life.  Yes, I have a lot of help.  I have an amazing support system of friends and family.  But in the middle of the night, it’s me looking for the occlusion in my IV line, taking my blood pressure, popping emergency meds and taking out my Epipens.  I am the one fighting for the things I need, organizing everything and ensuring that my needs get met.  And that’s okay.
I am the one who takes care of me.  And it’s harder, and more painful, but I never let myself down. 
I am my own last line of defense. 

The things I took for granted


I spent most of this week in the hospital.  I had anaphylaxis Sunday night at home, without an obvious trigger.  I used epi, called 911 and was transported to my hospital by ambulance.  I spent a few hours in the emergency department, received substantial doses of IV antihistamines and steroids, and was admitted.  I anaphylaxed the following day, and the day after that, despite employing serious measures to avoid it. 


This hospitalization was different.  I have reached a point in my disease where I am sick enough and complicated enough that doctors, even those who don’t know me, acknowledge that I am the authority on my health.  They no longer pretend to know more about my disease than I do; they allow me to determine what tests I need, and which are unnecessary.  They ask me questions like, “Is this a bowel obstruction?” and know that I will know the answer.  They monitor me very, very closely, but know that fretting over my wildly erratic blood pressure and pulse is likely fruitless.  They are sympathetic, but know there’s not much to be done that I’m not already doing. 
I both love and hate being inpatient at the hospital.  I hate that I am not home, that I am tethered to things even more than I am in my regular life, but I love that if something goes wrong, someone will notice.  I rest well at the hospital, in spite of the general noisiness and frequent intrusions for vital signs, exams, tests.  I am not worried about anaphylaxing in my sleep.  I am not afraid I won’t wake up.


It used to be that I had to defend the truth of my illness.  People didn’t believe me, or if they did, often didn’t believe the severity.  I looked grossly normal.  My life looked grossly normal.  What I suffered, I did alone; people knew I worked and lived a relatively active life, but never saw the nights I spent throwing up or sleeping through entire days.  It may have frustrated me, but it was also a gift.  If I was able to hide my disease, whether I wanted to or not, it meant that I was healthy enough to function.  I didn’t appreciate what this meant until now.


I am no longer mistaken for being healthy.  I never will be again.  My skin is permanently flushed, with only the intensity varying; I am swollen and squishy from steroids and edema; my abdomen is distended from poor GI motility; my PICC line winds along my arm, taped and waiting.  I take massive doses of medications and no one even bats an eye; being administered 350 mg of IV Benadryl over the course of a day (approximately equivalent to 1400 mg by mouth) isn’t even noteworthy.  If I am admitted to the hospital, my doctors come to see me themselves rather than send emissaries. 


I slept for 16 hours last night.  I didn’t wake to eat, drink or go to the bathroom.  My sister came in to check on me, unplugged and took my laptop, and generally was noisy for a few minutes at my bedside.  I didn’t even notice. More than any other thing it has taken from me, I feel the loss of time.  My days are devoured by this disease.  Everything feels urgent, because I never know when I will be able to do it again.  On the days when I wake early and can use my entire day, it feels amazing.  But there aren’t many of those any more.  The need to sleep is too overpowering. 


I require a lot of help to facilitate my life.  I used to be able to power through, even if it was hard.  That’s impossible now.  I used to be naïve about this.  I used to think that if I wanted it enough, I would be able to do something.  That’s untrue.  My will power is formidable, but it cannot enable me physically. I wish I had known that before.  I wish someone had told me that being able to clean my house, work every day, cook dinner, meant that I still had some health.  I wouldn’t have believed people who told me I would struggle so hard to independent at the age of 30.  I wish I had appreciated these things more. 


I used to participate in the 3-day for the Cure.  I walked 60 miles in three days a grand total of five times in four different cities.  I did all the training, even though I was sore and tired.  I walked in thundering rain and 100° heat, the watchful sun hovering overhead. 


I used to rock climb. I wasn’t very good at it, but I did it anyway.  I used to do yoga five days a week, exercise every night.  I had to medicate and be very careful, but I could do it. 


I used to eat whatever I wanted.  If it upset my stomach, I could still make that choice without fear of hospitalization.  I didn’t need to plan everything that went into my mouth.  I didn’t have to worry about how my body would process it and eliminate it. 


I used to be able to stay up late and still wake up for work.  I might not feel great, but I could do it.  Now I worry I won’t wake up in time, no matter how much I sleep.  I have to build redundancies into my system to achieve basic, everyday things.


I used to be able to clean my apartment.  I used to be able to go food shopping.  Now I need people to help me do either. 


I took for granted that I would always be able to do the things I loved, if I wanted them badly enough.  I took for granted that I would always be able to live the life I wanted, if I worked hard enough.  I took for granted that being smart would keep me alive. 


I used to think that I would “get better,” that I would eventually get to a place where I could function normally, even if it required medical intervention.  That’s not going to happen.  This is the best I can hope for.  I will never get better than I am now. 


I am generally a positive person.  I am not giving up.  I will be raising hell for years, if only because I have the support of friends and family.  I will be flying to Seattle this summer, even if I need to ship my medical supplies ahead of me and carry IV push meds for in-transit emergencies.  I am very grateful for opportunities like that.  But it doesn’t change how sick I am. 


I think that people need to know this.  That the sick people in their life can’t just “try harder” or “suck it up.”  That you can steel yourself mentally but eventually your body will fail you, maybe forever.  That you can’t blame yourself when you at long last need help to do everything.  That it doesn’t make you less than anyone else.  That all you can do is try to live within the confines of what your health allows, and milk that for all its worth.  That your body is broken and that’s not your fault.


That we’re all doing the best we can.  And that’s all anyone can do, really. 

On change and fighting the good fight

When I was in college, one of my friends had an eye-opening experience when she went home on winter break. 

“My parents look old,” she admitted, visibly distressed by this realization.  “Like a lot older than last summer.  I never noticed them aging before.” 
We don’t notice how dramatically things change when it’s gradual.  Her parents had never aged to her eyes because she saw them every day.  It’s the same way with chronic illness, at least for me.
In the beginning, it was just being tired, sinus congestion, allergy-type symptoms.  I slept a lot, felt sick a lot, but it didn’t really affect my life.  Then it was pills.  And more pills.  Specialists.  Endless tests, the wet heat of IV contrast dye, hundreds of tubes sloshing full of my blood.  IV push meds in emergency rooms, the burn of subcutaneous injections, the saline taste of IV infusions.  The false hope of an incorrect diagnosis.  The slinking fear that you won’t be believed when you have to explain one more time that you don’t have what you thought you had.  GI bleeds, organ damage, short term disability.  Hospitalizations, surgeries, central lines, hep locking, sterile dressings.  Anaphylaxis, more anaphylaxis.  Circular bruises on my thighs from autoinjectors.  Snuggling up with two liters of Lactated Ringer’s at bedtime.  Anyone can see that the life I have now in no way resembles the life I had when I started this. 
I always knew it was serious, even before I knew what it was.  I never underestimated this disease.  But I barely noticed how much harder it was for me to live a functional life, how many medicines I needed, how many surgical interventions were necessary, how my bathroom was stocked with first aid supplies, colostomy supplies, PICC line supplies.  I lost my frame of reference and this all feels normal.  Sometimes it feels like my life was always like this. 
I am never more aware of how sick I am than when I am in the hospital.  When I was scheduling my PICC line, the PA called me to go over my emergency protocol for shock, vascillating on whether it might be safer to place the line in the emergency room.  When the visiting nurse came to change my dressing, she was visibly frightened to touch my skin.  When I had major surgery last year, it took weeks of coordinating with several doctors to agree on how I should prep for surgery, what medications I needed for anesthesia, what the plan was for my recovery.  It is a reminder that even among medical professionals, my disease is intimidating and unpredictable.  We can only ever be so safe.
My disease has reached a point where I am so reactive that any procedure requires planning and premedication.  This is both a good and a bad thing.  It’s a bummer because I’m sicker; it’s a good thing because I get taken very seriously.  Even someone who knows nothing about mast cell disease can see that I am sick.  This is actually helpful.  It’s hard to argue the severity of disease when someone has a central line, an ostomy, and many surgical scars. 
This hospitalization has been much easier than the rest.  Every person I have come in contact with during this stay knows at least a little about mast cell disease.  The fact that they all remembered was that mast cell patients could progress very quickly to a dangerous situation.  Most of them knew some anecdotal things, like Darier’s sign and the flushing reaction.  I have gotten everything I needed in a reasonable amount of time.  They have clearly thought a lot about how my disease affects my other systems and test results.  They are aware that a nebulous explanation of why I have been so sick recently isn’t enough for someone like me.
I have had no problems except for one incident in which the floor resident insisted that I didn’t need epinephrine while I was anaphylaxing.  It was upsetting, but was straightened out.  It is frustrating and scary when things like this happen because no matter how prepared I am, I am still often at the mercy of authority figures.  And if those authority figures aren’t very, very well versed in mast cell disease, they can make decisions that can harm me.  We are our own best advocates but that matters very little if our providers don’t know our disease. 

It is getting better, if my current hospital stay is any indication.  The word is getting out.  More people have at least heard of mast cell disease, know that it can cause sudden, severe anaphylaxis, know that we react to things that most people never do and in ways that might seem strange.  No one has made me feel crazy, accused me of being drug seeking or treated me disrespectfully.  If they wanted to do something that wasn’t appropriate for mast cell patients, they called to confirm before proceeding. 

I know it doesn’t always seem like it, but every time we present for medical care, we are fighting the good fight. 

Keep fighting.

Dreams

I always wanted to travel.  From a very early age, I wanted to go everywhere, see everything, converse in other languages.  I spent hours in the library reading about faraway places, whispering phonetically written phrases in strange languages.  I never doubted I would make it everywhere I wanted to go.

I have done a fair amount of travelling and had many adventures.  I have turned a corner to see a brown bear eating cabbage in the moat of a castle, gotten lost hiking in the Norwegian wilderness and climbed ancient bell towers in Estonia.  My first overseas trip was almost ten years ago.  In the years that followed, I did a lot more travelling.  But all of these voyages were but practice for my ultimate goal – to travel around the world.
I get sentimental thinking about the trip around the world.  One day in 2007, I realized with that enough planning and saving, I could travel the world for a year.  For the next two years, I squirreled away money, took Russian classes, read reviews on hostels, memorized train schedules, priced airfares and planned and replanned the route over and over again.  I planned to leave in January 2011.
This trip was going to be my masterpiece.  I was going to start in Ecuador, then on to Peru to see Cusco, Nazca and Machu Picchu; fly out of Santiago, Chile, to Easter Island, where I would breathe in the ancient magic of the moa; from Easter Island to New Zealand by way of Tahiti; three weeks in NZ, from Te Reinga to skydiving in Christchurch; five weeks in Australia, travelling by train to see the Great Barrie Reef, Melbourne, Tasmania, Uluru, and Darwin; then north to tour southeast Asia, starting in Singapore, moving through Malaysia, Thailand, Cambodia and Vietnam; three weeks in Nepal hiking the Himalayas; Turkey, starting in Istanbul; on to Israel and Egypt to see the remnants of ancient civilizations; climbing Mt. Kilimanjaro in Tanzania; Namibia to see the Skeleton Coast; Botswana to see the Okavango Delta; and finishing in South Africa.
Of course, we all know that I didn’t travel around the world because I got sick.  In 2009, I lost a significant amount of hearing, and I spent a lot of the money I had saved for the trip on trying not to go deaf.  And from then I just got sicker and sicker, and I needed all my money for health costs, and I wasn’t physically able to travel like that anymore.  It was never going to happen.  It became a symbol of all the things I had lost to my failing health.
I used to have dreams often that I was going on an exciting trip and was either late for my flight or couldn’t find my passport.  Now I have dreams that I leave all my medication at home.   My body dictates my life, even in my sleep. 
I spend a lot of time thinking about the things that I want to do, the big things.  And no matter how many times I remind myself that I can’t travel around the world, I just want to do it anyway.  My spirit is stronger than my body.  It has hope that my logical mind suppressed years ago.  Our dreams sustain us, even if they are unlikely.  Sometimes especially if they are unlikely. 
I don’t know if I’ll ever take a trip like the one I painstakingly planned.  There are many reasons why it is improbable.  But I have to hope that I can.  I can live in a world where I have mast cell disease as long as I don’t have to live in a world where I could never travel around it. 
Life is nothing without hope.

More the same than different

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.  

On emergencies and making a scene

I am a pretty brassy person.  (I’m sure this surprises people who don’t know me in real life, as I am so shy on the internet.)  I am not easily embarrassed and never have been.  I have always been klutzy and loud, and I figured out early on that it was easier to just not be embarrassed by that.  Self acceptance.  I has it.


However, there is one thing that I get very embarrassed about, and I’m sure I’m not alone.  That, my friends, is making a scene, particularly if that scene is health related. 
Let me give you an example.  A few weeks ago, I was sitting at my mother’s house after eating Eggs Benedict talking about an upcoming family event.  I wasn’t feeling great but I had received some bad news that morning so I figured it was from stress.  Suddenly my stomach started hurting badly.  It hurt as badly as a bowel obstruction, but I knew it came on too quickly to be one.  At that point, I realized I was starting to anaphylax.
But instead of giving myself epinephrine, I decided to see if it would just go away.  (I’m actually laughing out loud as I type this because of the sheer stupidity.)  Shockingly, it did not just go away.  After several minutes of wailing like a wounded animal from the abdominal pain, I got that killer “Irish girl spent all day on the beach with no sunblock” full body flush.  You know the one.  The one that says I’m anaphylaxing. 
At this point, I should have given myself epinephrine.  (Please note: I am not afraid of epinephrine.  It resolves my symptoms quickly and I know that nothing bad will come from using it.)  But I didn’t want to use my Epipen because I didn’t want to cause a scene.  This is so stupid.  I know this is stupid.  I know everyone reading this is shaking their head because it’s stupid.  But it’s true. 
So my mother and sister noticed me changing colors like a decoder ring and asked if they should call 911 and give me epi.  I said no.  Instead, I pounded liquid Benadryl.  I actually can’t drink things quickly in regular life (I was an embarrassment in college – there was no chugging of any type of alcoholic drink as I was just physically incapable), but when I’m taking Benadryl to try and avoid using epi, I am a champion. 
As I was open-throating Benadryl straight from the bottle, my blood pressure dropped precipitously and my field of vision got fuzzy like a dream sequence from 90’s television.  Then my heart did that really entertaining thing where it skips beats.  At that point, my sister called 911.
Now, let’s recap.  I understand my disease – check.  I know that I should use epi sooner rather than later when having anaphylaxis – check.  I am a medical scientist and understand on a molecular level that epi will not hurt me  – check.  I know that sometimes I have to go to the hospital because I have a life threatening disease – check.
So why didn’t I just epi and call 911?  Because I didn’t want to make a scene.
Literally, as soon as my sister called 911, I was embarrassed.  It’s stupid.  I know it’s stupid, but it’s true.  I know they didn’t care that I interrupted their day because I obviously needed medical attention, but it didn’t matter.  It’s not logical.  I don’t like needing emergency care, especially if I am with other people. 
The ambulance showed up and then like half the neighborhood suddenly needed to walk their dogs by my mother’s house at exactly the same time.  You know how sirens make dogs have to pee, right?  Right.  So now there are people outside with binoculars and I’m in (unattractive, ill fitting) pajamas at 11:30 while I explain my disease to the first responders who have never heard of it.  It shouldn’t be embarrassing, and I wouldn’t be embarrassed to be present if it happened to any of you, but it embarrasses me. 
Side note: I think I would actually be more likely to seek emergency care if I could be sure no one would find out.  This has nothing to do with the behaviors of the people in my life.  They are all wonderfully supportive.  I know.  I’m weird. 
I find needing emergency treatment of pretty much any kind embarrassing.  Emergency room, epipen, whatever.  Needing this type of medical attention inherently puts me out of control.   There’s always the chance that I’m going to end up admitted to the hospital and lose days of my life.  I know it’s sometimes necessary, but that doesn’t change how I feel.  I’m usually not interacting with providers who know me, so I can’t predict what will happen.  I don’t like that.  And I don’t like it when people see that, either.
I don’t like making people worry about me.  Until fairly recently, I kind of coasted with regards to public perception of my illness.  By this I mean that people knew I was sick, but didn’t know the severity, and I knew they thought it was less serious and I let them.  It was easier for me, and I don’t regret making that choice.  But it did mean that when I told people, a lot of them were shocked.  Like, really shocked.
I knew that once I told people, they would be upset because they care about me and they worry about me and want me to be fine. Embarrassing!  I don’t want them to worry, and I feel like any time I have to make a scene and call a lot of attention to my failing health, it just sort of reinforces the gravity of the whole situation. 

And then there’s this other part, where I worry that I’m making a scene unnecessarily.  Like I worry that I’ll call the ambulance and they’ll arrive and be like, “Oh, you’re fine!  Why did you call us?” even though I AM ANAPHYLAXING AND THERE IS A CHANCE I COULD DIE FROM LOW BLOOD PRESSURE.  Like I said, it’s not logical.

If any of you mast cell people told me you waited forever to give yourself epi, I would be all over you.  I would send you pretty infographics about how you should use epi early and quote statistics about how often people accidentally stick themselves with epipens and live to tell about it.  Because you should use epi early.  It will help you.  I know the party line AND I AGREE WITH IT but this fear of embarrassment thing is really strong.  And I am sure I’m not the only one who feels like this.

This may surprise you, but I’m actually much less afraid of medical professionals telling me I wasted their time than I am of people in my life telling me that.  I can handle medical professionals.  This is not my first rodeo.  But feeling like I disappointed the people around me sort of mentally reinforces that my disease is “wrong” and therefore I am “wrong.”   (Again – this feeling was not provoked by the actions of the people around me.)
So I’m trying really hard to get over this because if I’m being honest, I can’t really afford to be cavalier with this anymore.  I am anaphylaxing a lot more than I used to and my body  is tolerating it a lot more poorly than it has in the past. 
So, there you go.  I wrote an essay and called a lot of attention to my fear of having a lot of attention called to me when I need attention.

If I’m going to the ER, I better need it

A friend expressed concern this week that I do not always go to the emergency room when I should. It’s a fair concern, and I’ve been thinking about it a lot.

Seeking emergency assistance presupposes that the benefit outweighs the risk. For people like me, this is not always true. People with mast cell disease often have a very complicated relationship with their medical providers, including their favorite local ER. Let me give you an example of how it plays out when I go to the ER.

On the way to the ER, I page the person covering service for my immunologist and GI mast cell specialist. At least one of these doctors will call ahead to the ER to tell them I have systemic mast cell disease and must be treated immediately. They will give orders for immediate IV medications to stop anaphylaxis. I also have a signed letter from my doctor listing emergency treatment protocols, in the event that I can’t get in touch with one of my doctors.

When I arrive, I say the following: “Hi, my name is ( ) and I have systemic mast cell disease. I am in anaphylaxis and need IV meds immediately to prevent going into shock. Dr. (whoever) called about me.” The receptionist may or may not tell me to fill out a form and take a seat. I may or may not have to argue with them. My mother may or may not have to argue with them. Most of the time, I am not looking great by this time, so either way, I am in a bed in the ER pretty quickly.

A nurse comes and puts in my IV. With my terrible IV access, this often takes upwards of twenty minutes. Remember – EVERY time you break the skin of a mast cell patient, you risk anaphylaxis. If I am anaphylaxing, this makes me worse. Getting an IV is dicey for me.

During this time, I speak to three or four other people. They might be nurses, physician’s assistants, interns or residents. An attending physician usually comes over. I tell every single person that I need specific IV meds immediately to prevent shock. I then have to explain this, telling each of them the exact same long, complicated story. They generally react badly, like I am telling them how to do their job. Most of these people have never seen anyone with my disease. Statistically, they may never see anyone with my disease again. I do not have time to wait for them to figure out the exact remedy for someone like me. People like me are directed by our physicians to be forceful and repetitive when presenting to an ER to avoid situations where we wait hours for meds.

If there are any students anywhere on the ward, they will ask if they can observe my (rare and exciting) existence. I always say yes, because medical professionals recognizing mast cell disease can only ever help me.

After half an hour in the ER, I am likely just getting my IV meds. They give them IV push, not infusion, because that is faster and at this point, faster is better. However, giving some of these meds IV push can cause wild variations in blood pressure for mast cell patients. I may or may not have problems related to stabilizing my BP.

Frequently, the reason I end up in the ER is because I had a bowel obstruction that hurt so badly that it triggered anaphylaxis. If this is the case, I am also told to request pain medication to suppress the pain to suppress the mast cell reflex. Most pain medications are not safe for mast cell patients. I am limited to tramadol, fentanyl and hydromorphone.

Telling anyone in an ER that you can take hydromorphone but not hydrocodone or oxycodone is guaranteed to get you an eyeroll and an assessment as a junkie. The fact that this can be easily verified through my medical records (or google) does not help my cause. So I just have to accept the fact that I have been branded as a drug addict. From that point on, I am treated as a junkie for the duration of my stay. People go from being curious and helpful to full of disdain pretty much immediately.

Last summer, the ER wanted to order a CT scan to make sure my bowel obstruction wasn’t turning into something worse. They wanted me to get a pregnancy test and wait for the result before getting the test. I refused because I had been in my OBGYN’s office twice that week and knew that I could not be pregnant. I didn’t want to wait hours longer for a test that I was already going to wait hours for. The physician’s assistant turned to the resident and told him that “she just wants dilaudid, she could care less if there’s a baby in there.” Unbeknowst to me, they called my PCP to let him know that I was “drug seeking.” This sort of things happens pretty much every time I go the ER. It is not unusual for people with mast cell disease.

Please note that this happened at the prominent hospital where I am seen several times a month, a hospital that has a department that treats my disease, and where all of my pertinent medical records reside.

Eventually, the reaction ends and I either go home or am admitted. Funny: as soon as one of my doctors, or their representative, shows up and states that I am being admitted because I have systemic mast cell disease, no one is calling me a drug addict anymore. But they’re not apologizing either. Once I am admitted, I generally have far fewer issues.

The emotional stress of going to the ER has to outweigh the physical stress of my current reaction for me to go. I wish it weren’t that way, but it is. If I cannot breathe, am bleeding freely, or have dangerously low BP, I go to the ER. But otherwise, I’m probably going to wait it out at home.

Experiences like this are the reason why rare disease awareness is so important.

At Least I Don’t Have Cancer


It happened again today.

I was engaged in casual small talk with someone when the subject of my illness came up. I showed my PICC line by way of explanation.

“You have cancer?” he asked, voice low.

“I have systemic mast cell disease,” I answered. I knew where this was going.

“Oh. Well, at least you don’t have cancer.” His whole body language changed. It wasn’t cancer, so I wasn’t worthy of his sympathy. Not that I want it, really; but I’m worthy of it, I think.

At least I don’t have cancer.

In the US, people tend to categorize illness into three groups: cancer; physical illness that is not cancer; and mental illness. The amount of respect and understanding you are afforded descends in that order. To most people in the US, having a verifiable physical illness gives me more credibility than someone with mental illness (although it shouldn’t), but less than someone who has cancer. Even if that cancer has been treated successfully and is in remission.

My disease is sometimes considered a cancer, but sometimes isn’t. It depends on the organization. Some hospitals treat it as cancer; some treat it as an immunologic disease; some treat it as a hematologic disease. It’s so rare no one can agree on what’s best.

My disease is classified as a myeloproliferative neoplasm. This is a fancy way of saying that my body makes too many cells in the bone marrow. Know what else are neoplasms? Cancers.

My disease is usually propagated by a mutation in an oncogene. Onco- is a prefix meaning “cancer.” Know what other diseases are caused by a mutation in this same oncogene? Cancers.

If you have involved systemic mast cell disease like I do, and you present to most hospitals, do you know what department you will be seen in? Oncology.

If you have smoldering or aggressive systemic mastocytosis, systemic mastocytosis with associated clonal non-mast cell lineage hematologic disorder, or mast cell leukemia, do you know what kind of therapies you will receive? Therapies designed to treat cancers. This is mostly chemotherapy in doses that are roughly equivalent to those used for – that’s right – cancers.

Cancer is a broad term. It refers to over 200 diseases that involve unregulated cell growth. Hey, know what other diseases involve unregulated cell growth? I bet you do.

There is massive variation in morbidity and mortality among those 200 diseases. The term cancer encompasses diseases like pancreatic cancer, for which 5-year survival is 6.7%, even when identified in early stages, as well as thyroid cancer, for which 5-year survival is 97.8%. When looking at all types of cancers, 66.1% of patients are alive after 5 years. In early stages, some cancers require minimal or no treatment at all. Some cancers have become essentially chronic diseases through innovations in drug therapies. Some offer the promise of remission for the majority of patients. And some are fatal, always.

There is this truth that doesn’t get discussed openly a lot in the mast cell community: that some of us will die from it. While the majority (SM and CM) won’t, life expectancy for SM-ANHMD averages 85 months (see paper here: http://www.ncbi.nlm.nih.gov/pubmed/24465546); for ASM, 41 months; for mast cell leukemia, 6 months. Many of us suffer progressive organ damage and repeat anaphylaxis, and eventually it kills us.

If mast cell disease was classified as cancer, insurance would be more likely to cover my therapies, including reconstructive surgeries, if needed; employers would be less likely to terminate me for illness-related absences; I wouldn’t be limited to a handful of treatment facilities; I would be more likely to have my pain controlled; I would be less likely to be referred to a psychiatrist when my symptoms couldn’t be managed; and I would have been diagnosed faster. Much faster.

There are obviously other aspects to living (or surviving) cancer aside from survival. There are financial issues due to loss of work and the general astronomically high expense of being sick. I know about that, as I have spent more than twice the cost of my education on my healthcare, all while having decent, employer-paid insurance. That’s just what I have paid out, not what I accumulated in debt. One difference here is that there are a significant number of charitable organizations dedicated to helping cancer patients pay for their treatment, or to assisting with incidental things, like transportation, cooking, etc. There are no organizations like this for me in my state. There is exactly one that I know of.

Cancer patients and survivors may also have to contend with body image issues due to disfiguring surgeries and tissue/organ removal. I have multiple scars from biopsies, tumor removal, organ removal, and am the proud owner of a colostomy. I would contend that gives me an idea of what it’s like to be disfigured.

For many cancer patients, there is the chance to become a “survivor.” There is a chance that their disease can go into remission. That will never happen for people with any type of mast cell disease. There is no cure for our diseases. For those that need chemo, it is essentially a lifelong process of on for a year, off for a year, or something to that effect. I personally know someone who has been on chemo for nine years.

The cancer “equivalent” of advanced mast cell disease, which I estimate to be Stage III chronic myelogenous leukemia, provides a 95% chance of 5-year survival. That would be an improvement for many with mast cell disease.

I want to be very clear about something: I do not have a problem with cancer patients or survivors. I feel a lot of empathy for them, especially as we have a lot of shared experiences. We go the same places, see the same doctors, take the same medicine, have the same procedures. I know what it is like to have a doctor tell you how long you are likely to survive. I know what it is like to take dozens of pills a day, to spend 20+ hours a week at the hospital, to be so nauseous that you can’t sleep and so tired that your vision is blurry. I know what it is like to wonder if you will be able to do all the things you want to do before you die.

I have a problem with the idea in American society that physical illness is either cancer or not cancer, and that “not cancer” is inherently less severe. It particularly bothers me because advanced mast cell disease is so similar to blood cancers. But that’s not really the point. Is advanced multiple sclerosis less severe than prostate cancer, for which 5-year survival is 98.9%? Is HIV? Diabetes? The fact of the matter is that some people with HIV take their medications and exercise certain precautions, but otherwise live a pretty normal life. Someone with diabetes, a very well-known and manageable disease, gets a difficult to treat infection and they die. You can’t say cancer/not cancer because disease progression is inherently individual. Two people with the same disease can have completely different experiences.

The point is that disease is disease. It all sucks. None of us deserve any of it. We should all be afforded the same compassion and understanding. I love my life, I really do, but this shit is hard. It is really, really fucking hard.

But, hey! At least I don’t have cancer.