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Lisa Klimas

I'm a 35 year old microbiologist and molecular biologist with systemic mastocytosis, Ehlers Danlos Syndrome, Postural Orthostatic Tachycardia Syndrome, Adrenal Insufficiency, and an assortment of other chronic health issues. My life is pretty much a blast.

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. 


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.

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. 

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.


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. 

Effect of anemia on mast cells

A paper released in September 2012 addressed the effect of iron availability on mast cell degranulation.
Inside the bodies of mice, it was observed that mice with decreased iron stores had more severe inflammatory reactions.  Importantly, iron supplementation decreased the severity of the inflammation, particularly in the context of allergic asthma.  Increased iron inhibited the production of inflammatory molecules in pulmonary tissues, including various interleukins and interferons. 
Outside of the body, mast cells were incubated with and without iron for 30 minutes.  IgE was then added to activate the mast cells.  The mast cells that were incubated with iron degranulated 30% less than those without iron present.  Spontaneous degranulation, without IgE crosslinking, was not affected.  The presence of iron also dramatically affected the production of inflammatory molecules by mast cells.  Production of TNF, MCP-1 and IL-6 decreased by 94%, 29% and 27%, respectively.  MCP-1 attracts macrophages. 
Iron supplementation decreased the severity of allergic asthma, and decreased mast cell degranulation by IgE crosslinking 30%, as well as decreasing production of inflammatory molecules by mast cells.

Hale LP, Kant EP, Greer PK, Foster WM (2012) Iron Supplementation Decreases Severity of Allergic Inflammation in Murine Lung. PLoS ONE 7(9): e45667. doi:10.1371/journal.pone.0045667

Anemia of chronic inflammation

Anemia is the condition of having either too few red blood cells or too little hemoglobin.  Hemoglobin is a protein found in red blood cells that transports oxygen.  Hemoglobin is one of the hemeproteins, meaning that it has an iron atom in the middle of a structural ring.  The iron allows hemoglobin to transport oxygen from the lungs to the capillaries, small blood vessels.  Thus, anemia can result in less than enough oxygen in the organs.  Iron status (how much iron a person has available for use) affects how well the body can oxygenate the tissues and generate energy.

Anemia is fairly common.  It is generally caused by blood loss, destroying too many blood cells (hemolysis) or not diminished hematopoiesis (the process of making red blood cells.)  Typical symptoms include weakness, fatigue, palness and shortness of breath.  More serious cases can cause heart palpitations, chest pain, fast heart rate and even heart failure.    There are many types of anemia.
One type of anemia is anemia of chronic disease, also called anemia of inflammatory response.  This type of anemia is seen in chronic illness.  In recent years, we have learned that this is most likely caused by overactivity of hepcidin, a hormone.  Hepcidin is the chief controller of iron levels in the body.  It can slow the body taking up iron from the diet and prevent iron from being released from its stores.
Overloading with iron will activate the body to make hepcidin.  This will result in a decrease in available iron, an increase of iron inside the cells that store it, and decreased absorption of iron in the gut.  Iron stores are composed mostly of cells in the reticuloendothelial system (RES), an older name for the mononuclear phagocyte system (MPS.)  These are cells that “eat” disease causing organisms, damaged cells or cellular debris, like macrophages.  Some of the cellular debris is pieces left over from broken down red blood cells, including heme.  Your body stores excess iron inside these cells to save for a time when it is needed. 
Your hepcidin level is regulated in response to many things, including anemia and inflammation.  Acute hemolysis, or destruction of red blood cells, from repeated blood draws decreased the amount of hepcidin your body made, even if the level was very high before.  This means that having blood drawn frequently signals to the body that it needs to keep its iron in its stores and shouldn’t take up any more from your diet.   
Acute inflammation decreases hepcidin, making iron more available.  But chronic inflammation increases hepcidin over 6X, making iron much less available to your body.  When your body is inflamed, its cells produce inflammatory molecules, like cytokines.  Some of these molecules, like IL-6, tell your liver to make more hepcidin.  If your body frequently sends out inflammatory signals, it can actually make it so that your cells are less able to release their iron.  It can also make your bone marrow less able to make red blood cells. 
When your body releases inflammatory cytokines, your body thinks it is fighting an infection.  These cytokines tell your body to make white blood cells, which your body thinks it needs to fight the infection.  White and red blood cells are made from the same stem cells in the bone marrow.  If the body is making more white cells, it is inherently making less red blood cells.  In this way, chronic inflammation increases the level of hepcidin, so the body keeps iron in its stores and stops absorbing additional iron, while also stimulating white blood cell production and decreasing red blood cell production. 
There are other ways in which decreased iron affects red blood cells, including interfering with the release of erythropoietin from the kidney.  This is the molecule that tells your bone marrow to make red blood cells.  When iron is deficient, the survival of red blood cells is also shorter.
So regardless of dietary iron intake, many people with chronic inflammation are functionally anemic.

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.


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.