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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.

Myers-Briggs Testing; or, the day I told my coworkers I was sick

Last fall, I took a Myers-Briggs workshop for work.  This event determined our Myers-Briggs Type ahead of time and then used several exercises to explain how we perceive the world and make decisions, compared with how our coworkers did so.  It classifies you are being one or the other in each of four pairs of preferences: extraversion vs introversion; sensing vs intuition; thinking vs feeling; judging vs perception. 

On the morning of the training, the woman leading the course handed out thick reports to each of us.  It revealed our results in explicit detail, and was unbelievably accurate.  My result was one of the most uncommon types, ENFJ.  I am extraverted, intuitive, feeling and judging. 
The ENFJ person is sometimes called “The Giver” in Myers-Briggs literature.  They address things primarily by how they feel about them.  More than any other type, they have excellent interpersonal skills, usually knowing the right thing to say in most situations.  They are empathetic, understanding and caring.  They are very honest and forthright.  They are especially good at developing talents in others, and do well in positions where they can lead and inspire.  Their main interests are giving love, support, and encouraging others.  They like to make things happen for people.  They are extremely organized, planning out everything in their lives well ahead of time.  They have a strong need for close relationships and will expend significant energy maintaining them.  They are very loyal and trustworthy. 
Because they are so good at handling people, ENFJs can be good manipulators, able to elicit desired reactions with ease.  Their motives are not usually selfish, but they can easily get under the skin of other people.  ENFJs tend to judge themselves harshly and to have deep, upsetting thoughts when alone, and to place others’ needs above their own.  ENFJs have strong values and opinions, but are likely to let go of a strongly-held view if doing so would serve another person’s needs.  ENFJs are not good at impersonal reasoning. 

We broke up into groups of similar attribute (all extroverts, all intuitives, etc.) to demonstrate the differences in how we address situations.  When we broke up in groups of thinking vs feeling, there were only two feeling people out of twenty.  We were given a scenario in which we had to cut some people from a planned trip, and asked to explain how we would determine who would be cut.  The thinking group had a very clear, logical decision tree.  We, the feeling people, also had a decision tree, but it was clearly based on helping people the most.  The thinking group wanted to send the most qualified people, who would best represent the company. We wanted to send the people who had never been, who would appreciate it the most, who might never get to go again. 

The reaction we got to this was largely incredulous.  Some people obviously felt this was a poor use of theoretical company money.  This exercise was very upsetting to me.  It upset me because my life often forces me to appeal heavily to this empathetic side of people.  It upset me because so often in previous situations, I had been refused opportunities because I was sick, and it didn’t make sense to give responsibilities to someone who was sick.  It upset me to realize that so few people thought that it was worth it to give a chance to someone because it would mean a lot to them. 
This specific exercise was the subject of a lengthy discussion at the end of the class.  One of my coworkers, a man I like and respect, was very curious as to why we felt the way we did.  We went around the table discussing our feelings, me feeling unsettled the entire time.  When it was my turn, I weighed my options and decided it was time to lay it all out on the table.  There’s never a good time to “come out” as a sick kid, and it seemed like it was time. 
“I have a rare, life-threatening blood disorder,” I said quietly.  A couple of the people present knew, but I had started having new, more severe symptoms.  I wouldn’t be able to hide it much longer.  I felt the flush rising on my chest but there was no going back.  “I know I don’t look like it, but I’m very sick.” I tried to choose my words carefully, both for clarity and for maximum effect.  “I think that it is a privilege to give people opportunities based upon the fact that it will mean a lot to them.  It costs you nothing to be nice to people.  Good will means a lot to people who are struggling.  And maybe for you, this is just a job.  But for me, it’s probably the last job I’ll ever have.  So I’ll remember kindnesses like that for the rest of my life.”
The instructor had mentioned earlier that people rarely change their Myers-Briggs type without a major life trauma.  This wasn’t my first Myers-Briggs test.  I had previously been ENFP, more spontaneous and adventure-seeking.  Every chronically ill person reading this knows that those behaviors become memories once you are diagnosed.
“I used to be an ENFP person, but then I got sick.  And now I have a plan and several back up plans for everything.  Someone mentioned earlier that it’s impractical to be a judging person, because you can’t plan everything.  I am prepared for everything, because I have to be.  I have to plan everything because it is the only way to keep myself safe.  If you don’t plan everything, probably nothing terrible will happen.  If I don’t plan everything, it could kill me.”   The room was silent.  Some of my coworkers looked stricken.  Some were crying.  “So when we talk about this stuff and how we make decisions, remember that these things aren’t abstract.  Every decision you make has the power to affect someone positively or negatively.  It’s your choice.  I just think it’s the better choice to always be positive, if you can be.”
This was the very first time I had decided to use the power of my story to positively impact the way someone viewed the world.  It was both empowering and terrifying.  Several of my coworkers came up to me afterward to hug me or tell me they were sorry I was sick.  A lot of them asked about my disease and I told them.  They cared about me and accepted me. 

Being around someone who is chronically ill often brings out the best, or the worst, in people.  It is rarely neutral.  One of the privileges I have in life is to work with people who have shown me an incredible amount of compassion and understanding.  “The other day I sent my friend flowers for no reason,” one of my friends told me a few weeks after the training.  “I thought about your ENFJ speech.” 

It made me smile.  They matter so much, these little things.

Broken heart

Emotional stress is my biggest trigger.  Not emergencies, mind you – I am good at reacting, administering, directing in an emergency.  I mean the type of emotional stress that can only come when someone who loves you wrongs you.  I can deal with people I care about minimally treating me poorly without risking anaphylaxis.  But when it’s someone very close to me, someone whom I love deeply, it is very dangerous to my health.

I got a colostomy in April of 2013.  My doctor expected that to resolve the majority of my systemic symptoms, as the long term bleeding and physical stress that necessitated the surgery were also triggering my mast cells.  His aftercare instructions were very clear: don’t lift anything heavy, eat a low residue diet, do not go anywhere strange, stay out of pain and do not get upset.  If I could do all these things, he expected my mast cell symptoms to subside considerably. 
I had five weeks of no mast cell activation.  I lost my swollen, pregnant midsection.  The act of sitting, walking, existing, was no longer inherently painful.  I had energy.  I was getting better. 
Six weeks after the surgery, my ex-boyfriend left me abruptly almost four years into our relationship.  We tried to work it out.  I think it might have been alright, but then we found out we had to move.  
We had moved into this apartment a year and a half earlier.  I was clear when we looked at the apartment that I didn’t want to move until I bought a house.  Moving is dangerous for me.  New apartments, with the possibility of hidden mold and environmental triggers, are dangerous for me.  I have true IgE allergies to several environmental allergens and exposure to any one of them can cause anaphylaxis. 
Our landlord knew this and reassured us repeatedly that we didn’t need a long term lease because he would never make us leave.  He lied.  When he told us we had to leave, I completely broke down.  “This could take years off my life,” I sobbed, dignity lost.  “I feel bad about that,” he muttered.  We were excellent tenants, he agreed, he just wanted his daughter to live there instead.  He didn’t care.  They never do.
The next day, I got a bowel obstruction.  The mast cell symptoms returned in force, all at once.  “I told you not to get upset,” my doctor said, as if I could possibly control any of this.  But I understood his point.  When you have mast cell disease, the ones you love can truly be your undoing. 

I ended my relationship for good in February.  I would rather be alone than be with someone who handles my illness so poorly, though this is no comfort when there is no one moving against me at night.  I wanted to stay, but I could no longer ignore the fact that the stress of trying to fix this had taken a very serious physical toll on me.  It wasn’t worth my life.  And my heart was already broken, anyway.

I have been through a lot, all things considered.  It has made me stronger, all of it, even the things I wish hadn’t happened.  Every time I am grateful for my steel will, for my ability to make hard decisions without hesitation, for my ability to not get upset every time my body fails me in a new way, I have to admit that it was the hard times that made me this way.  I can’t imagine surviving any other way.  But the wounds – those are real, too.
There are nights when I can’t sleep and start sobbing uncontrollably.  I press my hands against my chest, against the hardness of my sorrow.  I run my fingers along the edge and it is shaped like a broken heart.  It feels like being half awake and reaching for him, before I know where I am and that he will never be there again.  There is no physical pain on earth that can compare to the memory of a love that is no longer real.
My mother used to have a seashell in her bathroom.  When I was little, she would hold it up to my ear and I could hear the ocean.  I feel sorry for that shell now.  It remembers the sound of the waves but can never go back.  I know what that’s like.
They say you can’t die of a broken heart.  Maybe they’re right.  But years from now, when they open me up to see what happened, they’ll find glittering shards in my chest and know that they cut me every time I breathed.

On blind faith and believing things will get better

The very first person to recognize that I had a systemic disease was the doctor who performed my sinus surgery in 2008.  The previous June, I had gone on a three-week backpacking trip through Scandinavia and contracted a sinus infection I couldn’t shake.  In November, after several rounds of different antibiotics, I was referred to an ear, nose and throat specialist.  He took one look at my massively deviated septum (a casualty of my wild child teen years) and scheduled surgery to clean out my sinuses. 

He also advised that I contact an immunologist as soon as possible, as I was a 23-year-old in seemingly good health, yet had been unable to clear an infection for six months.  I set up an appointment and became an active player in a game I hadn’t yet realized I was playing.  The hunt for mast cell disease was on.
This doctor’s greatest asset was the longevity of his career and, owing to this, the fact that he had seen thousands of patients over the years.  “You remind me of this woman who used to be a patient of mine,” he told me during a follow-up appointment as he looked through the scope snaking into my sinuses.  “She looked so healthy, like you.  But something was wrong with her.”  He remembered the specifics of her case very clearly.  She had Hashimoto’s thyroiditis, like me.  She had hearing loss.  She had also required sinus surgery.  “She knew there was something affecting her whole body, but I didn’t know what it was.”  He had thought at the time that it might be an endocrine issue. 
This woman took on a mythical status in my imagination.  I hadn’t been convinced that what I had wasn’t just a collection of mild, treatable ailments.  But once I heard about this woman, I knew.  I knew that this wasn’t all a coincidence. 
I haven’t seen that doctor in years, but when I was diagnosed with mast cell disease, I called his office and left a message for him.  He called me back a few days later.  He remembered me and my collection of symptoms.  He was fascinated to learn that I had mast cell disease.  I asked about the woman, in case she had never received a diagnosis.  “Oh, she died some years ago,” he told me casually.  I was too afraid to ask how she died.  I don’t have any reason to think that it was from mast cell disease, or that she had even had it in the first place.  But the news that she had died, this woman I had never met, hit me strangely. 
There are so many tiny things that affect you when you have a disease like mine.  You feel an immediate kinship to anyone like you.  The emotion you feel can sometimes be disproportionate to the actual intensity of the relationships.  You take things personally.  You are acutely aware that you are different from everyone else.  You can’t freak out about the big things, so you freak out about the little ones instead.  You get very good at cataloguing the suffering.
But there is this other about being sick.  You see good in everything because some days you need all those little beacons of light to scatter the darkness.  Hope begins to feel like a religion.  Making plans, thinking things will improve, believing that you can still have the life you want – these things feel like acts of faith.  Every step that moves you forward is a prayer to the universe to let you have one more.  The act of staying alive feels sacred.  You have to believe it will get better.  There is no other way.
Many languages do not differentiate color in the same way that English does.  Several of them use the same word to refer to both blue and green.  I can only think of hope now as being brilliant against this dark horizon.  That’s how I feel when I plan my trip around the world, like I’m executing a divine will.   I don’t have any words for that, but when I close my eyes, the light it shines inside me is blinding.   

The doctor-patient relationship and the danger of self-advocating

I had an appointment with one of my immunologists yesterday.  I like this doctor.  We have an excellent relationship.  He has been with me through a lot.  When I met him several years ago, I didn’t know what I had but I knew my diagnosis was wrong.  He didn’t know what I had, but he believed I had something and was willing to try some unusual choices.  Some of them did nothing.  Some of them worked.  The ones that worked gave us information that ultimately led to me being diagnosed with mast cell disease.

At the same as I was being seen by this doctor, I was also being seen by a doctor, a rheumatologist.  This rheumatologist thought I had rheumatoid arthritis and did not think I had anything else wrong with me.  This rheumatologist went so far as to state that all my (anaphylaxis) attacks were due to anxiety.  He thought I was a hypochondriac.  He wasn’t alone.  I was seeing several specialists at the time, and all of them thought I was generating abnormal lab results by being upset about life.  All of them except this particular immunologist that I saw yesterday.
About two months before I was diagnosed with mast cell disease, I stopped believing that the disease was damaging my body worse than the many medications I was taking without a solid diagnosis.  I was tired.  I was mentally unable to fight anymore.  It is so hard to keep believing something is true when so many people who should be able to find it insist that there is nothing there.  I decided to discontinue all of my many medications in a last ditch effort to identify what I had.  Predictably, I anaphylaxed and went to the hospital to be monitored and tested for a few days.  But no one recognized it was anaphylaxis, so they didn’t find anything.  I gave up and went home.  I told my immunologist that I was done and I meant it.
Two weeks later, the week of Christmas, he called me.  He told me that there was one more person he wanted me to see.  I told him I didn’t want to see anyone else.  That I meant it when I said I was done.  He told me that he thought this person would know what I had, and that if nothing came of this one appointment, he would leave me alone.  And because he had believed me for so long when no one else did, I agreed to go. 
That appointment happened on a Tuesday four weeks later.  It is the appointment where I was diagnosed with mast cell disease.  I never saw that rheumatologist, or any of those other specialists, again. 

I was very lucky.  I was lucky that I didn’t die from anaphylaxis prior to diagnosis.  I was lucky that my family and friends (most of them) continued to believe me when it was unclear if I was right.  I was lucky that I had even one doctor who believed me.

This is not always true for people without diagnosis.  This is not always true for people with diagnosis.  There is a very real tendency by medical professionals to ignore or downplay things they do not understand.  There is a desire to always be the most informed person in the room.
I interact a lot with providers who don’t know very much about mast cell disease.  I explain a lot of things to them.  Some people are grateful for the knowledge.  My stoma nurse recently said to me, “I love you because I know you’ll tell me exactly what you need.”  Sometimes they appreciate my directness.  Sometimes they refuse simple requests for no reason other than the fact that I had the audacity to know about it when they did not.  No one likes to be told what to do, but for people like me, there isn’t always another option.  I can’t assume that they will eventually arrive at the appropriate conclusion when they don’t understand my disease.
Once they get mad, they are not interested in providing me the best care anymore.  They think I’m a know-it-all and they tolerate me at best.  “Smart people like to feel smart,” the floor intern told me during my recent admission, as explanation for why the resident was frustrated when I understood my symptoms better than she did.  “I’m smart, too,” I reminded her. 
I am not a medical doctor.  I was too sick to go to medical school.  Before I knew I had mast cell disease, I spent years researching various diseases that I thought I might have.   I have worked in medical research for thirteen years, worked in pharmacy for ten years, and have both undergraduate and graduate degrees in the biological sciences.  Additionally, I have direct access to mast cell fluent people through my job.  These experiences have taught me a lot about medicine, physiology and pharmacology.  If I sat the med school admission exam again, I would do well.
When I was diagnosed, I spent months doing nothing but learning mast cell biology.  I got books, read papers, emailed researchers, called doctors (some of whom even called me back.)  Even still, I read papers about mast cell disease and related topics on a daily basis.  I do not know everything, but when it comes to mast cells, I know this shit cold.  But that only helps me if my doctors see me as participating in my care and not making outrageous demands or insulting their intelligence.
Yesterday, my immunologist and I discussed whether or not I needed to go to the hospital after self-administering epinephrine.   I am anaphylaxing a lot these days.  I regularly lose four days of my week to intense pain and borderline anaphylaxis, in that “safe to not use epi if I drink Benadryl constantly, but very uncomfortable” stage.  He agreed that when that happens, I can give myself epinephrine and IV Benadryl at home and not go to the hospital if things stay stable.  This is a huge relief.  He knows that if I feel unsafe, I will go to the hospital.   He knows this because after years of seeing me, he understands that I can be trusted to make decisions like this for myself. 
Many people don’t have doctors like that.  It makes me so mad to see people talking about how their (non-mast cell aware) doctors won’t increase their antihistamines, prescribe mast stabilizers, learn about their disease.  It makes me so mad when my friends are told by specialists that they “don’t know about their disease,” as if they shouldn’t be expected to educate themselves beforehand.  It makes me so mad when doctors ascribe any problem you have to mast cell disease when we so frequently have comorbidities.  It makes me so mad that we have to argue for the things that keep us safe when getting tests, procedures or surgeries.  It endangers us and forces us to understand our disease in order to prevent injury, something that isn’t always possible. 
There is no remedy for this beyond education and a new paradigm in medicine that involves active patient participation.  I worry about my friends who don’t have a good relationship with their doctors.  It makes me extra grateful for people like my immunologist. 
Last night as I was drawing up my IV Benadryl, I literally thanked him out loud.  I am one of the lucky ones, and I know it.

Mast cells, eosinophils and the perfect storm of inflammation

Mast cells and eosinophils have a lot of common functions.  In allergic and inflammatory states, these cells come into physical contact with each other, as well as communicate using chemical signals called cytokines and chemokines.  Mast cells and eosinophils are often found together in affected tissues in disorders like allergic rhinitis, atopic dermatitis, and asthma.  Mast cells initiate the allergic inflammatory response once activated.  This signals for eosinophils to come to the tissue.  Increased numbers of mast cells and eosinophils are found in diseases like eosinophilic esophagitis, chronic gastritis, GI neoplasms, parasitic infections and IBD.  Both mast cells and eosinophils respond to eotaxins, molecules that draw eosinophils to the inflamed area.  So one signal causes both cell types to go to the affected tissue. 

Mast cells and eosinophils interact a lot by using chemicals.  Mast cell released heparin stabilizes eotaxins.  Mast cells produce IL-3 and IL-5, which lengthen the lives of eosinophils in tissue.  Mast cell mediator chymase suppresses eosinophil death and causes eosinophils to release several chemicals.   Tryptase can limit eosinophil activation.  In turn, eosinophils produce stem cell factor (SCF), which attract mast cells and protects them from cell death.  Both cell types express some common receptors, like Siglec-8, which induces eosinophil death and inhibits IgE-mediated mast cell activation.  Interactions between these cells increase activation and proliferation. 
Patients with SM may have another blood disorder, including CEL or hypereosinophilic syndrome (HES.)  SM-HES and SM-CEL with the D816V CKIT mutation has been found, and the mutation is present in both the mast cells and the eosinophils.  However, it is likely that the FIP1L1-PDGFRA fusion gene (an aberrant tyrosine kinase) is the cause of the coexistent eosinophilic and abnormal mast cell proliferations.  The FIP1L1-PDGFRA fusion has been found in several cell types, including neutrophils, monocytes and mast cells.  This finding is consistent with a mutational origin in a blood stem cell that makes mutated mast cells and overproduces eosinophils.  When these cells are not neoplastic, they are derived from separate stem cell lineages.
Shortly after the discovery of this fusion gene, there was significant debate over whether FIP1L1-PDGFRA+ disease was an eosinophilic neoplasm with increased mast cells or systemic mastocytosis with eosinophilia.  Patients with FIP1L1-PDGFRA+ eosinophilia have a lot of symptoms in common with SM: swollen spleen, hypercellular bone marrow, high numbers of abnormally shaped bone marrow cells, marrow fibrosis and elevated serum tryptase.  However, these bone marrows show less dense clusters of mast cells.  In some cases, mast cells were spindled and expressed CD2 or CD25.  Still, the WHO considers it a distinct entity and not a subset of SM.
In CKIT+ patients, GI symptoms, UP, thrombocytosis, serum tryptase value, and dense mast cell clusters aggregates in bone marrow are significantly increased.  Cardiac and pulmonary symptoms, eosinophilia, eosinophil to tryptase ratio, elevated serum B12 and male sex were higher in FIP1L1-PDGFRA+ group.
Eosinophilia in SM patients has no effect on prognosis.  Eosinophilia in MDS patients predicted significantly reduced survival.  In T lymphoblastic leukemia, eosinophilia was unfavorable for survival.  Density and activation of tissue eosinophils is related to disease progression in several neoplasms.  Mast cells and eosinophils are found in increased numbers in neoplastic disorders like Hodgkin lymphoma. 
Presence of FIP1L1-PGDFRA indicates treatment with imatinib (Gleevec), regardless of organ dysfunction.  It can show remission within 4 weeks, even at low doses.  Some patients with CKIT+ SM with HES or CEL have rapid and complete normalization of severe eosinophilia with midostaurin treatment. 

Gotlib, Jason, Akin, Cem.  2012.  Mast cells and eosinophils in mastocytosis, chronic eosinophilic leukemia, and non-clonal disorders.  Semin Hematol 49:128-137. 

On losing my hearing

I have this philosophy about my illness, that I’m not my illness and I’m not my body.  It keeps me sane a lot of the time.  It is easier to see it as something separate from me, an antagonist.  It is easier to not feel complicit in all this.

I started feeling that way when I lost my hearing.  I lost it in 2009, and never got it back.  I have no hearing in my left ear, some in my right ear.  The hearing in my right ear fluctuates.  I stopped seeking technological interventions when I was accused in 2011 of Munchausen’s by a specialist and referred to a psychiatrist.  The psychiatrist agreed I did not have Munchausen’s.  I never tried to get a hearing aid again. 
A few short weeks after the appointment with the psychiatrist, I was diagnosed with mast cell disease.  “We’re not sure why, but a lot of people with this disease have trouble with their hearing,” the doctor agreed kindly.  I cried when he told me.  It felt so final.  A tiny part of me had always hoped that once I was diagnosed, the treatment would give me back my hearing.  But it didn’t, and it won’t.  I am Deaf.  I will be Deaf for the rest of my life.
It is impossible to describe how it felt to lose my hearing.  It was like slowly bleeding with no way to stop it.  There was panic and anxiety.  I couldn’t focus on anything else.  And then eventually, it stopped.  I was damaged, and I moved on. 
The thing about your hearing is that you use it for everything.  I had never even noticed.  Very early on in my Deaf life, I looked both ways and stepped into the street.  A friend pulled me out of the way just before a car hit me.  I had seen the car, but because I couldn’t hear it, my brain told me it wasn’t moving.  It was a jarring realization that I used my hearing to keep me safe, and now it was gone.  The whole world felt different.  It felt alien.
Losing my hearing represents the first time in my life that I couldn’t make my body work through force of will.  In 2009, I was having joint pain, tiredness, fevers, rashes.  I had a few inaccurate diagnoses.  In spite of that, I could still make my body do whatever I wanted, even it hurt.  I could overcome the pain.  I could not will myself to hear. 
It was also the first time I had to demand accommodations.  I had to tell people to look at me when they spoke.  I had to get an earpiece to talk on the phone.  I had to request interpreters for medical appointments.  It was my introduction to self-advocating, and that has served me better in my adult life than any other quality.  I am not afraid to fight. 
I have adapted over the years to the point that I barely notice my Deafness.  I can hear on the phone if it is quiet; I sign well enough to use a video phone.  I watch the tv closed captioned, use a vibrating alarm clock and a lamp turns on in my living room when you ring my doorbell.  Learning to function as a late-deafened adult was hard but not impossible. 
In many ways, my hearing loss is hard to talk about.  It is still a wound, one that comes raw with too much touching.  The entire experience affected me and changed me in ways I could never have expected.  It was a loss I felt more acutely than anything else that has been taken from me.  It was the point of no return, after which I would never recover the health I had previously had.  My life is divided into two epochs: before and after I lost my hearing. 
But I owe a lot to my hearing loss, I think.  It forced me to learn another language, to become a part of a culture I had known nothing about.  I have made friends I would never have made otherwise.  I found out who in my life really cared about me.  It made me think differently about my health.  It made me realize that deafness was not a disability, but an attribute, a facet of who I am.  It made me realize that I could still have the same life I had before, if I wanted to work for it.
I recently went on high dose steroids to treat my mast cell disease.  It was a treatment I had been given for my hearing loss, one that worked, but was discontinued due to terrible side effects.  I woke up in the middle of the night a few months ago, and I heard a strange noise.  It was sort of a soft ticking, a sound I didn’t recognize.  I turned on the light and tried to locate the source.  I eventually realized it was the fan.  It had been so long since I had heard one that I had forgotten what it sounded like.  It was like being visited by an old friend.  My dosage decreased and this brief glimpse of my old life vanished.  The blades spun silently now. 
My life isn’t better or worse for being able to hear a fan.  It is a reminder of both my damaged body and the ways I have learned to live with it. 

In case of emergency

If you have mast cell disease, then your life has the possibility of being turned inside out in a matter of seconds.  The last time I anaphylaxed, I was sitting on my couch at home after a very pleasant day.  I had to call 911, use epi, and spend five days in the hospital because I kept anaphylaxing.  So with basically five minutes of notice, I had to leave my house for a week.

I am a strong advocate of having a “go bag” like pregnant women pack close to their due date.  That way you can grab it and take it with you.  If you’re not home, you can have someone go get it for you and it’s already packed.  That way you can be sure you have all the important things you need.

So what should your “go bag” have in it? This is what mine has:

Toiletries.  A lot of us are reactive so we need specific products.  I pack a toothbrush, toothpaste, face wash, body wash, deoderant, shampoo, lotion, Rocket Shower, hair brush, elastics, pins, tweezers, nail clippers, razor and razor blades, my spare glasses and my mouthguard. Rocket Shower is a spray that you spray onto your skin and then wipe off with a towel if you’re not able to shower.  It’s moisturizing and it makes you feel clean.

Clothes.  I always feel better in actual clothes.  I pack a couple of changes of pajama type clothes, like pajama pants, tank tops and a hoodie for when it gets chilly at night.  Tank tops are good because they give easy access for EKG/heart monitor leads as well as central/IV lines.  Make sure your sweater/hoodie is loose so it doesn’t compress the lines or the infusion pump will think it’s occluded and beep incessantly.  I find that zip up sweatshirts are better than pull overs because you get less things tangled. 

Medication.  I know this seems antithetical, but hospitals don’t always have the meds you take regularly.  This is particularly important for mast cell kids like me because we don’t always tolerate inactive ingredients in medications, so switching brands can be dangerous for us.  If you have over-the-counter meds that you take regularly, or meds that you know the hospital is unlikely to have, bring them with you.  Also, things like nasal sprays and inhalers are good to bring with you.  I bring ranitidine (my hospital stocks famotidine), fluticasone nasal spray and ketotifen. 

Chargers.  Because it’s 2014 and we can’t live without our phones.

Specialized medical supplies.  I have a colostomy, and products for ostomies are not really interchangeable.  I also have a PICC line.  Pack at least a week’s worth of supplies.  I pack them in a little black pack, which also has antiseptic wipes and adhesive remover wipes that are safe for me.  If the hospital has supplies that you can use, great.  If not, they’re going to use whatever they have, and that might not be safe for you.

Safe snacks.  If you get there at night, you’re not going to get food.  If you eat low histamine or have a lot of food triggers, you may not have a lot of options. Put a few nonperishable things in the bag.  (I have saltines and peanut butter.)

Head phones.  So you can watch movies or listen to your music on your phone or laptop.

Journal/notebook.  I write every day.  It’s also good to have on hand so you can take notes while talking to your doctors, or make notes about things you want to ask.

Book.  Hospitals are boring.  Bring some entertainment.

Paperwork.  This is important.  You should have a “greatest hits” sheet on you all the time.  Mine has my diagnoses and relevant procedures, indicates high risk for anaphylaxis, states emergency protocol for anaphylaxis/trauma, indicates central line, has an UP TO DATE medication list including IV meds, and has a list of emergency contacts as well as physicians to be contacted in case of emergency, with phone numbers.  I also always have the Mastocytosis Society Emergency Protocol on me.  At home, I keep copies of these two things on my fridge as well.

Epipens.  Bring your epipens.  Yes, they will almost certainly administer it to you at the hospital, but if you’re somewhere unfamiliar, with non-mast cell aware providers, it’s best to be safe.

Dog.  Bring your dog to keep you company.  This is Harry P.  I bring him in my backpack. 

And bring your purse/wallet so you have your ID, insurance cards and money.  I also bring my laptop and cord so I can work. 

Other things: 

You should have a health care proxy, and you should know where it is.  If you are regularly seen at a hospital, it should be on file there.  Your proxy should have a copy. 

If your condition is advanced, consider an advanced healthcare directive. 

Contact your local EMS provider and ask if you can stop by and teach them about mast cell disease.  My local ambulance company and fire department both have copies of my emergency protocol, so when they responded a couple of weeks ago, they knew exactly what to do.  Saves time and makes you feel safe.

You should have a medic alert bracelet.  If you anaphylax suddenly, you might not have time to tell someone what’s wrong. 

If you have involved responsibilities, like a special diet for a pet, write them down and file them somewhere. 

Write out a bill-pay schedule with typical amounts and note how they are paid (automatic withdrawal, check, etc.)  In the short term, this is not a big deal.  If you’re in a coma for a month, it’s crucial to not waking up to a disaster. 

If your health is deteriorating and you are single (like me), I strongly advocate having either a joint account with someone you trust, or having your account in trust for someone.  This allows them access to your account if anything happens to you.  This is for the same reason as the bill-pay schedule: it allows you to not emerge from a health crisis and transition to a huge mess. 

Have important papers organized and filed.  Make sure you tell people close to you where these things are.  This should include copies of your health care proxy, advanced directive, life insurance, disability policies, insurance paperwork, things like that.

I know this stuff is not fun to think about, but if you ever need it, you’ll be glad you did it, and if you don’t need it, it will make you feel better to be prepared. 

Mast cell disease in families

Three types of MCAD are currently known: systemic mastocytosis (SM); mast cell activation syndrome (MCAS); and mast cell leukemia (MCL).  SM and MCL are thought to be rare, while MCAS is now believed to be much more common, and possibly even the underlying cause of various clinical presentations (such as IBS and fibromyalgia.)  Very little is known about the heritability of these conditions , but many patients report that they have family members with similar symptoms. 

A study examining the familiality of MCAD found that 74% of patients interviewed had at least one first degree relative (parents, siblings, children) with systemic MCAD, regardless of MCAD subtype or gender.  The prevalence of systemic MCAD among first-degree relatives was 46%, while the prevalence in the control group is about 17%.  The prevalence of MCAD among first-degree relatives of patients with MCAS was 60%; with SM was 44%. 

MCAD subtype and severity of symptoms varied between family members.  Variable genetic alterations in CKIT were detected.  Activating CKIT mutations were found in 65% of patients, compared to 15% of the control group. The genetic mutations detected in the three families included mutations at position 816 of CKIT (D816G, D816V, S1A).  This finding is remarkable in that it disproves the longstanding belief that the somatic nature of KIT and related exon 17 mutations means that it cannot be inherited.  It also supports the belief that other mutations in genes that regulate mast cells could be contributing to these diseases.  Multiple mutations were sometimes found in the same patient, including those found in other genes (JAK2, TET2, DNMT3A, ASLX1, CBL, U2AF1, SRSF2, MS4A2). 

There was also no obvious relation between the CKIT mutations and clinical severity of MCAD.  Although familial occurrence due to shared environmental factors cannot be ruled out, it is likely that there is a significant genetic contribution to this phenomenon.  More females than males were affected.  The prevalence of MCAS was expected to be at least within the single-figure percentage range in the population (1-9%.) 

Systemic MCAD family histories include more systemic MCAD cases than would be expected when compared to the prevalence in the general population. This study advocates that the different subtypes of MCAD (MCAS and SM) should be more accurately regarded as varying types of the same disease rather than distinct diseases of mast cell dysfunction.


Molderings GJ, Haenisch B, Bogdanow M, Fimmers R, No¨ then MM (2013) Familial Occurrence of Systemic Mast Cell Activation Disease. PLoS ONE 8(9):e76241. doi:10.1371/journal.pone.0076241

Not a war

People use battle terms when they talk about disease.  “Keep fighting!” they say.  They talk about health in terms of “winning” and “losing.”  They call my life a “battle.”  War makes it easier to be merciless.  You want to win at all costs.  War turns the enemy into a faceless horde, and the consequences of your actions aren’t important.   You can scorch the earth if you know that eventually this will end and life will spring forth from the ground again.  You can imagine a future in which the scars have been consumed by nature, this history swallowed whole and invisible to a new generation.

In the beginning, I thought like that, too.  My disease was my enemy.  My medications were weapons.  In learning more about my illness, I was arming myself for the future.  Sometimes I was advancing; others, retreating.  Every day I made tactical decisions in my fight to overcome these obstacles.  I was a soldier, drafted into a conflict I didn’t want to fight, but doing it nonetheless.
But this isn’t a war.  This is my body.  It tries to kill me, but it also allows me to walk at night, balance in Crow pose, drink coffee.  It is sick, and it is damaged, but it is the only body I’ll ever have.  There is no known instance of a person with no mast cells surviving.  They heal my wounds, thin my blood and protect me from infection.  It pains me to admit it, but I need my mast cells. 
It’s harder to envision this as a war when you know you will never win.  I will never be able to kill off all my mast cells; I have to live with this faceless horde.  I can only appease them and mitigate my damage.  When you think about this every day, it begins to change you.  You might still see your body as an adversary, but you grudgingly find ways to work together.  And not often, but sometimes, you find that by doing this there are things you can do that couldn’t before.  In some ways, you can learn to play your disease to get everything out of your body that you possibly can.
My disease has caused me to accept my body.  It is scarred and misshapen and swollen, but it is still here.  Looking in the mirror is like visiting a monument to the resistance.  We’re outmatched, but we try anyway.  Sometimes it is better to work with your enemy than to fight them on principle.  There is a kind of quiet honor in that. 
Some days it is still a battle.  But most of the time, it is just my life, living in this body, trying to work with it.

The memory of pain

“It’s so hard to forget pain, but it’s even harder to remember sweetness.  We have no scar to show for happiness.  We learn so little from peace.”  -Chuck Palahniuk, Diary
I’m a medical scientist.  At a conference a few years ago, one of the doctors was recounting a phenomenon we are all familiar with – the patient who swears that their current illness is the worst they have ever had.  It’s not, though.  “They just forget how bad it is,” he surmised.  They are lucky, these normal people.  They get sick a few times a year, so infrequently that the light of their health overwhelms those dark spots. 
I’m not like that, and I don’t think I ever have been.  I’m very grateful for my good days, but when I look back over the landscape for the last few years, that’s not what stands out.  I remember the happiness and enjoyment of those days, but not the physical feeling.  It’s hard to commit the sensation of “less” or “better” to memory; it is merely a fact I can regurgitate when my doctors ask me.  It evokes nothing in me physically. 
I remember pain more than anything else.  I feel like this says something about me as a person, but it’s true.  I spend a lot of time with my pain, after all; it changes and evolves, but never really leaves.  It started in my hands and feet, arthritis that I feel as soon as I open my eyes.  Then my other joints, stiff and sore with motion, throbbing when still.  My lower back, that feels like a seam along which my body will break when I bend.  My lower abdomen, my entire GI tract that burns and twists.  The throbbing in the long bone of my thigh, the twisting in my chest.  The bright red sunburn of anaphylaxis all over my skin.  I have become skilled at cataloguing it, at knowing what is normal and what is new. 
Whether I like it or not, I have learned a lot from my pain.  It has forced me to prioritize my life, to actively pursue the things I want and to eschew what I don’t.  It has forced me to really want things, or to forget about them.  My pain makes me tired and irritable; it guarantees I only spend time with those people who are deeply important to me.  I never do anything just for the sake of doing it.  And in many ways, that is a blessing.
It used to bother me when people talked about being grateful for their illnesses.  I’m not grateful for my illness.  I would rather not have it.  But I like my life, and I like who I am, and my disease is part of the shaping forces responsible.  I am more empathetic now, more organized.  I expect less of people and am let down less.  I deal with disappointment better.  I accept that I cannot do everything I want to.  I suppose I’m grateful for those things, even if I would rather have come to these realizations by another route. 
Sometimes I’ll have a couple good days in a row and I think to myself, maybe this is when it gets better.  Maybe this is when things start steadily improving.  Maybe this is when my pain subsides and I get back the life I had where I could stay out late and drink alcohol and run a 5K and do yoga every day. 
Realistically, that’s never going to happen.  I will never be healthier than I am now; there is too much damage.  But every once in a while it feels like a possibility, and it doesn’t erase the memory of the pain, but it does soften it, just a little.