Mundane dangers

I recently went out to a restaurant with my family.  I loaded up on meds before we went.  As we were sitting at the table, a thunderstorm rolled in.  I watched with dismay as the street before me became a water way.  I wondered how I was going to get to the car without getting my PICC dressing soaked, causing it to come away from my line and exposing me to a central line infection.  I ended up asking the waitress for a trash bag and wrapped my arm in it.  I ran to the car and was terrified until I got home and could pat it dry.

Today I found out that the IV medication I paid $200 to overnight to Seattle was not delivered and is not refrigerated, meaning that it will almost certainly be useless.  My other option is to pack all of these supplies in my luggage, but it would be too heavy for me to carry.  Not having IV fluids guarantees a bowel obstruction so going without is not an option.  I have talked to eight different people about this and needed medication when I started reacting from getting upset. 
I hate how much I need everything to work perfectly to manage my disease.  It is by the far the most difficult part for me.  I hate that I need people to be completely accommodating.  I hate that I also need nature to be forgiving.   I hate that my life has no flexibility and when I protect my routine, people think I am unyielding for the sake of it.  I can’t control these things, but they can control me.
Day to day life with mast cell disease requires elaborate scheming to achieve even basic things.  Routines are crucial to not needing epinephrine.  Making plans ahead of time keeps us safe, but only if nothing changes.  When things do change and we get upset and people act like we’re crazy, it further reinforces how frustrating this disease is. 
I don’t want to be this way.
I don’t want to be the type of person who cries when something goes wrong.
I don’t want to need other people to keep me safe. 
I don’t want to be demanding and unreasonable.
Some days, I am just so tired of this world, with all its mundane dangers.

World without end

I have absolutely no idea what I did on July 8, 2011.  I’m sure I did mundane things and stayed up late.  I didn’t write in my journal.  It’s funny that I don’t remember this day, one of the fault lines along which my life split. 

At the same time that I was ambling through a normal day, my cousin was climbing over his futon to hang himself outside of his bedroom window.   Almost an hour later, first responders took him to a hospital.  He was resuscitated after such a long time that meaningful recovery was impossible.  
My cousin and I grew up together, less than two years apart, but drifted apart once we hit high school.  There was never any animosity, we just didn’t really see each other much anymore.  We still knew each other in the way you only can if your mothers are close – we heard about each other, all the highs and lows, but had little actual contact.  It had been years since we had seen each other, lots of hauntingly near misses.  I regret deeply every one of them.
My mother called me early on the morning of the 9thand told me what had happened, that he was in the ICU.  I called around to mutual childhood friends until I found someone to grieve with.  There wasn’t much to say.  Together we went up to the hospital that night. 
There were a lot of people in the waiting room for my cousin.  He had a lot of family on both sides, a lot of friends.  We ran interference with the staff while his mother smoked in the courtyard.  At his mother’s behest, I grabbed his chart off the wall by the nurse’s station and flipped through it.  I explained the medical terms, a hollowness expanding inside of me.  And then, when there was nothing else to do, I went into his room to visit with him.
He looked fine.  The fact that he looked fine made it much harder.  He literally looked like he was going to open his eyes and start talking.  There was a bandage covering the thin red line encircling his throat.  His neck wasn’t broken.  The rope had compressed the vagus nerves, cutting off communication from his brainstem to his vital organs.  It had probably happened in less than a second.  He had probably been dead in just a few minutes. 
I talked to him for a while.  I reminisced for a while, shared funny memories with the otherwise empty room.   I thought about how long it had been since we were children.  I thought about how long it had been since he was truly happy. 
My cousin lived for 13 days in the ICU.  It was awful.  We all knew he would never wake up, but it felt like not hoping was a betrayal.  So we carried this sharp and dangerous hope inside of us, and it tore everything around it.  I wish that he had died on July 8, that I had never had to see his body shudder with artificial ventilation.  I wish that I could remember him as he was, all of it, until that day.  I don’t want this ugliness to be part of his story.
Late on the night of the 20th, my friend called to tell me that my aunt was ready to let him go.  I went to the hospital.  I opened his eyes to see them one more time.  I checked his non-existent reflexes out of habit.  I told him that I would see him again, somewhere in that world without end.  I told him that I forgave him and I went to go get drunk with his brother. 
On the morning of July 21st, I opened my eyes into a world in which my cousin was no longer alive.  My sister and our friend, my cousin’s best childhood friend, drove 80 miles to the campground we all had gone to every weekend and all summer long for most of our formative years.  We drove slowly down the dirt roads, the air thick with nostalgia.  We swam in the river and lay on the beach, smaller than I remember. 

Our friend climbed high into the rafters of one of the sitting areas and wrote my cousin’s name in thick bold lettering.  He wrote dates below it, just numbers and punctuation, and the time they spanned felt short.  It all felt very wrong.  Watching him write, I knew that we were too old to be here, in this place where we had grown up. 
But in that moment, my memory stretched back 15 years, to the summer of 1996.  I could see all us running over the packed dirt and concrete, doing cartwheels over picnic tables, laughing.  We were all young and plotting and exploring.  He was having fun.  He was happy and safe.
I like to think that heaven is a lot of things, the best moments of your life played out over and over again.  I like to think that everyone who was present in those moments is present for real in heaven, because it’s the culmination of all things in which all boundaries are lost.
In one part of heaven, I will see this: a big group of kids at a secluded beach at the bottom of a steep dirt hill, jumping off a dock and swimming in the river.  This is the story I want – the one where he never left that summer.  He is still 14 and he never finds drugs or depression.  And in this story, I remember clearly that we were all happy once.  For a brief moment in time, we were all happy together.  That is what I want to remember.

As good as it gets

Yesterday, I had a very normal day.  I worked from home and stayed inside, out of the heat.  I ate low histamine food and avoided triggers all day long.  Around 8 last night I started reacting anyway and needed all the meds.  Stability is like a mirage; it looks close, but I can never quite reach it.

There are a lot of people for whom mast cell disease is mostly a nuisance.  My disease was never like that, but I sort of behaved like it was.  I knew I would pay for it if I stayed out late or exercised too much but for a while, I could make myself do it anyway.    I wanted to believe I could have any life I wanted.  I wanted to believe that my body could take as much as my mind could.
But my disease isn’t just a nuisance and eventually it caught up to me, all at once.  I don’t know about you, but mast cell disease runs my life right now.  It will be at least a voting member for as long as I live.  Mast cell disease isn’t something I gave into it.  It is something that happened to me.  To imply that by living within the lines it drew means I have less willpower or ambition is offensive.  To say that I am weak because I have to avoid triggers as much as possible is just naïve. 
And when people say, “I don’t let my disease control me,” all I can think is, Well, there’s still time for that, isn’t there. 
I used to be so independent.  I used to do everything myself.  Now I have to have people drive me around most of the time, I have to get help to carry things, my friends come over and do chores for me, I can’t travel alone.  Maybe my apartment is messier than I would like, and I always have unfolded laundry piled in my basket chair, but I can work full time, and see my friends and family, and be safe. 
I still have dreams.  I still want to do things I know I’ll never be able to do.  Every once in a while, I’ll be talking to someone about these things and get really excited.  I think to myself, if I could just hold onto this feeling, I could do this.  But I never can.  This loss of possibility is the hardest for me.
For some of us, this is as good as it’s ever going to get.

Seattle, redux

I was diagnosed with mast cell disease in January, 2012.  At the time I was diagnosed, I was sleeping through entire days, so brain fogged that I frequently forgot basic facts, and spent most of the time I wasn’t asleep in the bathroom.  I was a hot mess.  My mast cell specialist ordered the relevant tests but was sure this was some kind of mast cell disease, so he put me on medication immediately. 

About two weeks after starting medication, I woke up one morning and felt better.  Not 100% better, but better than I had felt in months.  A few weeks after that, I went back to work.  I was trying to learn the boundaries of my disease but in those first few months, I honestly thought that I might get my life back.  I still needed a lot of rest but I felt stronger, happier and much more functional.  I call this my “honeymoon period.” 
During this time, one of my dear friends came home from Seattle to have a wedding shower with her Boston family and friends.  While making duct tape flowers, I decided I was going to go to Seattle for the wedding.  I had been feeling pretty good for a few months and figured there was no reason not to.  Several of our friends were going so I figured if I got into trouble, there would be people to help me. 
So I bought airfare to Seattle and arranged to fly out and share a hotel room with one of my friends.  I requested the time off from work and didn’t really think very much about it.
The flight out there was fine.  We got picked up from the airport and found a bar that served us grilled cheese sandwiches and tomato soup on a very wet night.  We went back to the hotel and crashed.  So far so good.
In the morning, we wanted a big breakfast.  We walked around Seattle and eventually ended up at an awesome place called Glo’s.  I had Eggs Benedict and a lot of coffee.  I then discovered that I could no longer go to the bathroom. 
I didn’t know exactly what was happening but I knew that it was different than anything I had experienced before.  I have had GI problems my whole life.  Something was wrong.  We went back to the hotel and things got worse into the next day.  I called my doctor and he agreed that if I felt it was safe, that avoiding going to a hospital in Seattle was the best idea.  I employed some extraordinary measures, put my friends to work and pushed through the pain to make it to the wedding.  I’m glad I did, but it was not the trip I wanted to have.  It proved definitively that I was sick, that I was always going to be sick, and if I didn’t remember that, I was going to end up in a lot of trouble.
That trip to Seattle is the very first time I gambled that I was healthy enough to do something and lost.  It was also the point at which I realized my mast cell disease was seriously damaging my body.  The dehydration caused by the flight had turned an annoying but manageable situation into a nightmare.  It would have happened eventually, I’m sure, but the fact that I wasn’t near my doctors and home when it happened made it worse.  In the few months after I got back, I had scopes and tests and met with a surgeon.  My systemic symptoms were being triggered by the pain and poor lower GI function.  Less than a year later, I had surgery to place a colostomy. 
Next week, I’m going back to Seattle.  It feels a lot like I have a score to settle.  There’s going to be a 40’s lounge night and a trip to Portland and cute dresses and maybe a death tour.    There’s also going to be low histamine food and some flushing and probably vomiting and possibly some anaphylaxis.  And maybe I’m going back with a central line, a colostomy, lots of IV meds, several bags of Lactated Ringer’s, ten Epipens, a backpack full of oral meds, healthcare proxy paperwork, sterile dressing change supplies and a best friend who can push my meds if I get into trouble, but I’m going back.  Stand up and fight.
I’m coming for you, Seattle!  This is going to kick ass.

Knowing it when you see it

I grew up in Massachusetts, a proud liberal.  I believe strongly that society should be as inclusive as possible.  We have a long way to go in combatting racism, sexism and other forms of discrimination.  I think the difference between equality and fairness is sometimes not obvious, and this can cause some confusion.  I try to be as sensitive as possible to all groups of people. 

One of the things I have come to understand about being chronically ill is that we are misunderstood in a way that is permanent.  It is not like being misunderstood for your beliefs or ideals.  There is no way to make anyone who doesn’t live this life understand me.  They can sympathize, but they can never really know.  I try to educate wherever possible, but that’s not the same thing.  I accommodate the world to my needs as much as possible and do pretty well with that.
People with mast cell disease have so many bizarre restrictions.  There’s a reason people sometimes don’t believe us when we tell them.  If you can separate yourself from your own reality, it’s easy to see how absurd some of our claims are.  They are true, but that makes them no less unusual.  In this way, we are very much a minority.  So when people make jokes about seemingly innocuous things, they are sometimes assuming that they are in no way offensive. 
I find that one area that causes trouble over and over again is comedy.  For people who live in fringe communities, like rare disease or genderqueer or whatever, it is sometimes hard to know what exactly is offensive.  This sounds counterintuitive, because I think that most people think that they’ll know it when they see it.  But sometimes you don’t. 
I think part of the outrage we feel when people make jokes about things we can’t do is that it makes us feel misunderstood.  But the fact is that most people are never going to understand us.  And so instead of being able to definitively say, that is offensive and that’s not, we are left with this infinite space populated by our myriad feelings of hurt.  Then the day changes and maybe we feel differently.  It is a moving target.
I am not easily offended and haven’t been for years.  When people make jokes about things are harmless to most people but dangerous for me, I do not get offended.  I usually comment after, “Unless you have mast cell disease.”  Sometimes it spawns a conversation and sometimes not.  I try to consider the intention of the person telling the joke.    
But if you are offended by things like this, that’s okay.  It is okay to feel however you feel.  We are all at different places in this journey.  We don’t move through certain feelings and eventually all end in a place of acceptance.  It is more like floating in the ocean; we live in the ebbs and flows, dynamic. 
There was a Supreme Court case in 1964, Jacobellis v. Ohio.  It involved whether or not the state banning the showing of a French film with considerable nudity and sex was a violation of the first amendment.  Of specific importance was whether the film was considered pornography.  While I don’t remember the details of the case, I do remember a famous opinion handed down by one of the Justices.  “I shall not today attempt further to define the kinds of material I understand to be embraced within that shorthand description (of pornography)… But I know it when I see it.” 
It’s not always that easy.

Diagnosis of mast cell diseases

There seems to be a lot of confusion regarding diagnosis of mast cell diseases, so I figured I’d do a review.

Cutaneous mastocytosis (CM) is diagnosed by skin biopsy.  Urticaria pigmentosa (UP), also called maculopapullar cutaneous mastocytosis (MPCM), diffuse cutaneous mastocytosis (DCM) and telangiectasia macularis eruptive perstans (TMEP) are types of cutaneous mastocytosis.  They each present with a rash and may have accompanying systemic symptoms. 
Mastocytoma of the skin is also diagnosed by skin biopsy.
Systemic mastocytosis (SM) has the following diagnostic criteria:
Major:
1.       Multifocal, dense infiltrates of mast cells (15 or more in an aggregate) detected in sections of bone marrow and/or extracutaneous organ. 
Minor:
1.       In biopsy sections, more than 25% of mast cells in infiltrated area are spindle-shaped or have atypical morphology; or, of all mast cells in bone marrow aspirate smears, more than 25% are immature of atypical. 
2.       Detection of Kit mutation at codon 816 in bone marrow, blood or other extracutaneous organ.
3.       Mast cells in bone marrow, blood or other extracutaneous organ that co-express CD117 with CD2 and/or CD25.
4.       Serum total tryptase persistently >20 ng/mL (if there is not a clonal myeloid disorder.)
SM is diagnosed if a patient has either one major and one minor criteria, or three minor criteria.  So let’s look at how this plays out.
A patient with mast cell symptoms gets a bone marrow biopsy.  It shows more than 25% abnormal mast cells in the section.  They are CKIT negative, have a serum tryptase of 2, and do not express CD2/CD25.  They are diagnosed with SM.
A patient has a biopsy that does not show dense infiltrates.  All their mast cells are shaped normally.  In blood tests, their mast cells are found to express CD2.  They are CKIT+, also from blood.  Their serum tryptase is 28.  They are diagnosed with SM.
A patient has a biopsy that shows dense infiltrates, but they have less than 25% abnormal mast cells and their mast cells do not express CD2/CD25.  They are CKIT- and have a serum tryptase of 18.  They are not diagnosed with SM.
A few things to keep in mind:
Most people with SM are diagnosed by bone marrow biopsy, but a biopsy from any non-skin organ showing mast cell infiltration as described above can be used.  This means if you have a positive lung biopsy, liver biopsy, whatever, you may not necessarily need a bone marrow biopsy. 
It can take up to six bone marrow biopsies to diagnose SM in a patient who has had it the entire time.  This is because there is no way to know where the mast cells will cluster.  A negative bone marrow biopsy does not necessarily mean that you do not have SM.  Hence the minor criteria.
The CKIT test looks for a specific mutation, the D816V mutation.  There are other mutations found in codon 816.  You may have a mutation but test CKIT- because you do not have the D816V mutation.  Also, the blood test for CKIT is not always reliable.  The test way to test this is from a bone marrow sample.  You could test CKIT- in blood and then test CKIT+ in bone marrow.
The serum tryptase criterion refers to persistent baseline level tryptase, not reaction level tryptase. 
So let’s say you have a negative bone marrow biopsy and a blood test that shows you are CKIT+ and have mast cells expressing CD2/CD25.  What do you have?  You have monoclonal mast cell activation syndrome (MMAS.)  MMAS is diagnosed in patients who have one or two of the minor criteria for systemic mastocytosis.
Let’s say you have a negative bone marrow biopsy and blood work that shows normal mast cells and tryptase below 20, but you have systemic symptoms.  What do you have?  You probably have MCAS (mast cell activation syndrome.)  There are some other tests for that.  24 hour urine tests are usually done to measure the levels of histamine metabolites and prostaglandin D2 metabolites.
The following are the diagnostic criteria for MCAS:
1.       Episodic symptoms consistent with mast cell mediator release affecting two or more organ systems: skin (urticarial, angioedema, flushing); GI (nausea, vomiting, diarrhea, cramping); cardiovascular (fainting or near fainting due to low blood pressure, rapid heartbeat); respiratory (wheezing); naso-ocular (itching, nasal stuffiness, red eyes.)
2.       A decrease in frequency or severity; or resolution of symptoms with antihistamines, leukotriene inhibitors or mast cell stabilizers.
3.       Evidence of elevation of urinary or serum marker of mast cell activation: Documentation of elevation of marker during a symptomatic period on at least two occasions, or if baseline tryptase is persistently above 15 ng.  This includes urinary histamine and prostaglandin D2.
4.       Clonal and secondary disorders of mast cell activation ruled out.
MCAS is a diagnosis of exclusion.  It is the diagnosis you receive if you have mast cell symptoms that are ameliorated with mast cell medications if you do not meet the criteria for any other mast cell disease.
Back to SM.  Let’s say you’re positive for SM.  Now what?
They will determine if you have other important markers of disease severity.  These are called B and C findings.  They are as follows:
B findings:
1.       Increased mast cell burden (>30% mast cell aggregates on bone marrow biopsy and/or serum tryptase >200 ng/ml).
2.       Hypercellular marrow, signs of overproduction or abnormal development of blood cells, normal or slightly abnormal blood counts that are not abnormal enough to be considered an associated hematologic disorder.
3.       Swelling of the liver that can be felt manually, no free fluid or signs of dysfunction, persistently swollen glands, swelling of the spleen that can be felt manually without signs of dysfunction.
If you have two or more B findings, you have SSM (smoldering systemic mastocytosis.) 
C findings:
1.       Unusual blood counts (low ANC, low Hb, low platelets)
2.       Swelling of the liver that can be felt manually, with impaired liver function, free fluid and/or portal hypertension.
3.       Large osteolytic lesions and/or pathological fractures.
4.       Swelling of the spleen with impaired function.
5.       Malabsorption with weight loss and/or low albumin.
If you have one or more C finding, you have ASM (aggressive systemic mastocytosis.)
How are these B and C findings identified?  Bone marrow biopsy, blood tests and imaging (ultrasounds, MRI, etc.) 
If you have SM and one B finding, or no B findings, you have indolent systemic mastocytosis (ISM.) 
If your bone marrow biopsy shows significant overproduction or abnormal development of a cell type that is not a mast cell, you may be diagnosed with SM-AHNMD (systemic mastocytosis with associated hematologic non-mast cell lineage disease.)  People with this type of SM also have another blood disorder, such as chronic myelogenous leukemia, myelodysplasia, etc.  In these patients, serum tryptase is not reliable to assess mast cell burden.  
Mast cell leukemia (MCL) is extremely rare.  It is diagnosed by >20% mast cells on the bone marrow aspirate smear.   
Mast cell sarcoma is a very aggressive form of sarcoma.  It is diagnosed by biopsy of the tumor.  People with these tumors quickly developed mast cell leukemia.  There have only been three cases reported in literature.  To be clear, this is NOT the same as mastocytoma.  Mastocytomas are benign.
I think I got everything.  Any questions?  Ask in the comments.

The right way to be sick

“You can’t be on here with that,” a woman told me earlier this week. I was riding the red line train to Hindi class. She waved pointedly at my PICC line. I smiled at her.

Sometimes they’re less outright ridiculous. “Is that on your list of things you can eat?” someone recently asked at a party. “Definitely not,” I answered, laughing. “Thank goddess for Benadryl.”

Then there are the comments about the big decisions, that are harder to find funny.

“I think you should just go on disability.”

“I don’t think you should live alone.”

“It’s not safe for you to travel.”

“You shouldn’t go out alone.”

And so on, and so on.

Or how about these:

“I didn’t think someone so sick could do that.”

“If you’re too sick to go into work, you’re too sick to go to the store.”

“If you’re healthy enough to take the train, you’re healthy enough to do this.”

Everybody has an opinion.

Society in general has expectations of the sick. They expect you to get better or to die. This has been demonstrated in sociology studies. Obviously, a great number of us live in the grey area between getting better and dying. We’re not going anywhere and the general public doesn’t know what to do with us.

There is also an expectation that we will try to get better. Of course, how the public thinks that should look and how it actually looks are rarely the same thing. If we don’t fulfill their criteria for trying to get better, we are told not to complain when we don’t feel well. There is seemingly no end to the commentary.

Living with mast cell disease takes a lot of work. I take handfuls of pills all day long, eat low histamine, try to stay as cool as possible and still react every day. I react to things that are unavoidable, like sunlight and humidity and stress. I have made the choice to lead as normal a life as possible for as long as I can, even though walking outside literally makes me sick. This doesn’t make better than people who choose to isolate to avoid reactions. It just makes me a sick girl who made a different choice.

Sometimes I do things that I know will make me sick. These include things like exercising, eating a piece of cake, sitting in the sun. I am an adult. I am an autonomous human being who can make her own choices. The reaction I get sometimes is absurd. “I can’t believe you think it’s okay to do that, when everyone worries about you all the time,” I was told not too long ago. “I can’t believe you think you get to judge me for eating a meatball,” I replied with mock horror. I mean, come on.

I spend my days calculating risk. A lot of things I enjoy have been sacrified on the altar of mast cell disease. A lot. Sometimes I really want a chocolate frappe or to go to a party in the summer heat. I don’t need to be reminded that I can anaphylax at any time. I am aware of this fact.

So if you’re wondering what an appropriate way is to make these types of comments, there isn’t one. If you ever feel tempted to second guess how someone manages their body and their illness, don’t. Even if you feel you’re doing it out of concern. It’s not helpful. Trust me. For some reason, people seem to think that my illness gives them the right to judge my actions, even if they wouldn’t do it to a healthy person. The odds are pretty good that you don’t walk up to people eating dessert in a restaurant and dazzle them with your knowledge of obesity related health issues. And if you do, stop. Just stop.

When this really bothers me is when it happens among people who sick. Every sick person thinks they have the best way to be sick. I see this all the time. “Why aren’t you on this?” “I have that and I still work.” “Why don’t you wear a mask?” Everyone is different. And even more, everyone’s life is different. I don’t know if I would stay home if I didn’t have to pay my bills, but I do, so it doesn’t matter. We all do the best we can in the confines of what we can do. Cheating on our diet or staying out late doesn’t make us bad people. It makes us normal for a fleeting moment.

Tonight I walked down the street in 85 degree heat to get a chocolate frappe. It was great. I needed Benadryl a half an hour later. I’m still pretty flushed and I don’t care. If I were to give anyone advice on the best way to be sick, it would be to do it in whatever way makes you feel the most like yourself. Do it in whatever way allows you to do the things that make you happy.

Turns out, there’s no right way to be sick.