No, using the manual syringe/vial method is NOT the same as using an epinephrine autoinjector

I have a lot of inflammatory things to say about the current economic situation for patients who need epinephrine autoinjectors but for now, I’m going to stick to dispelling the most damaging myth I have seen spreading like wildfire.

No, manually drawing epinephrine from a vial or ampule into a syringe and then administering is NOT the same as using an autoinjector.

The American Academy of Allergy, Asthma & Immunology is a professional organization that regularly publishes updated practice parameters on the treatment of anaphylaxis. In their 2015 Anaphylaxis Practice Parameter Update, the AAAAI recommended that providers “prescribe two doses of auto-injectable epinephrine for patients who have experienced an anaphylactic reaction and for those at risk for severe anaphylaxis.”

This publication also addresses commonly disputed situations relating to anaphylaxis such as when and how to administer epinephrine to patients in special populations, like children under the weight range for Epipen Jr (33 pounds). The recommendation in the practice parameter was to use an Epipen Jr rather prescribe a syringe and vial of epinephrine for the parents to administer a smaller dose. The justification for this recommendation given in the 2015 Anaphylaxis Practice Parameter Update is that “…underdosing might not effectively treat anaphylaxis, giving a dose that is slightly above the ideal dose appears to be a better option than giving a dose that is below the recommended dose.”

A 2001 study by Simons et al. assessed how long it takes parents to manually draw up epinephrine from a vial. They also determined how long this took for resident physicians, general duty nurses and ER nurses. The results are in table 1 below.

Table 1: Mean time to draw up dose of epinephrine, range of time and variation in drug concentration
Group Mean time to draw up dose Range of time Variation in epinephrine content within group
Parents 142 ± 13 seconds 83-248 seconds Forty-fold variation
Resident physicians 52 ± 3 seconds 30-83 seconds 7 to 8-fold variation
General duty nurses 40 ± 2 seconds 26-71 seconds 2-fold variation
ER nurses 29 ± 0.09 seconds 27-33 seconds No variation

 

As you can see, the fastest parent drew it up in 83 seconds (about a minute and a half) while the slowest drew it up in 248 seconds (over four minutes). There was a 40-fold variation in the amount of epinephrine drawn up so both underdosing (which will not stop anaphylaxis) and overdosing (which can cause severe CV effects) would have occurred. Please note that this study does not assess how much longer it took to inject the medication for obvious reasons. It was also done in a controlled environment that assuredly was much less stressful and chaotic than one that would accompany a real life anaphylactic emergency.

Say it takes 15 seconds to find the vial and needle and open them and another 15 seconds to inject it. In a best case scenario, in which the parent is aware of anaphylaxis at the exact moment it begins, is in no way flustered, confused, or scared, and is in a situation where they can immediately respond (by which I mean, they are not driving, they are not swimming, they are not watching their child from 100 feet away), the fastest a parent would be able to draw it up and administer the med would be about two and a half minutes. Two and a half minutes with no oxygen if the child’s airway closes right away. If you are an anaphylaxis patient and are trying to do this for yourself, you would be expected to be able to function without oxygen for two and a half minutes.

About 30% of anaphylaxis patients require a redose of epinephrine to control anaphylaxis. So if you or your child is one of those people, you then have to do all of this again. If the first dose doesn’t do much, it could be another two and a half minutes.

The numbers were better for resident physicians and general duty nurses but you are still looking at 2-2.5 minutes for the slower members of these groups. There was a 7 to 8-fold difference in amount of medication drawn by resident physicians. ER nurses reliably drew up the dose in about 30 seconds and assuming that it takes 30 seconds to get the med and needle, and to inject it, they could draw and deliver in about a minute. That is the best you can hope for.

I realize that many mast cell parents and patients draw from vials/push meds/inject meds frequently and they may be practiced enough to draw the dose reliably and administer correctly. If we assume the most competent among them to be as competent as the ER nurses, it takes about a minute to administer the med. I just timed myself and it took eight seconds for me to get the epipens out of my purse, open the case, and remove the blue cap. (I used a trainer). It took less than one second to bring the injector to my leg. The entirety of the dose of epinephrine is delivered from an epipen in three seconds.  So 12 seconds for use of an epipen vs 60 seconds for the fastest, most reliable manual draw and inject.

There are currently two other autoinjectors available in the US: Adrenaclick and epinephrine autoinjector, a generic version. Both of those autoinjectors differ from epipens in two important ways: they are syringe based, whereas epipens are cartridge based; and their needles are shorter compared to epipens. In one specific comparison study by Ram et al., Epipens were found to more reliably deliver the most epinephrine (74.3% of intended dose) compared to syringe autoinjectors like Adrenaclick and epinephrine autoinjector (25.7% of intended dose). Auvi-Q was pulled off the market for similar delivery issues. (Author’s note: please note that this study did not include the specific generic epinephrine autoinjector, but its delivery mechanism is comparable to Adrenaclick).

A 2015 study reported by Umasunthar and colleagues compared how often mothers of food allergic children were able to correctly administer Anapen (a syringe based autoinjector not available in the US) vs Epipen in a simulation. Six weeks after they were shown how to use it, 42% correctly administered Anapen and 43% correctly administered Epipen. This means that over half of participants could not correctly administer epinephrine even with an autoinjector.

Alternative autoinjectors are not comparable to Epipens and neither is the manual syringe/vial method.  Delay in administration of epinephrine is a strong risk factor for poor outcome, including fatal anaphylaxis. Additionally, delay in administration of epinephrine or inadequate initial dosing is a risk factor for biphasic anaphylaxis.

Stay safe out there.

References:

Lieberman P, et al. Anaphylaxis – a practice parameter update 2015. Ann Allergy Asthma Immunol 2015: 115, 341-384.

Lieberman P. Biphasic anaphylactic reactions. Ann Allergy Asthma Immunol 2005: 95, 217-228.

Ram FSF, et al. Epinephrine self-administration in anaphylactic emergencies: Comparison of commonly available autoinjectors. Journal of Asthma and Allergy Educators 2012: 3(4), 178-181.

Simons FER, et al. Epinephrine for the out-of-hospital (first-aid) treatment of anaphylaxis in infants: Is the ampule/syringe/needle method practical? Journal of Allergy and Clinical Immunology 2001: 108(6), 1040-1044.

Umasunthar T, et al. Patients’ ability to treat anaphylaxis using adrenaline autoinjectors: a randomized controlled trials. Allergy 2015: 70(7), 855-863.

Vigil

I like to be alone. It took me a long time to feel that way. When I was in grad school, I lived in a cheap apartment on the border of a neighborhood that was mostly known for gang wars and being the setting for a documentary called (I swear this is real) “Getting high on crack street.” Once while I was having a party, an episode of COPS happened on the sidewalk I traversed every day to leave my apartment. COPS was filming in Lowell and they literally caught some guy and arrested him on my sidewalk. So basically what I’m saying is my apartment was charming. And cheap. But still charming.

Not that long after the COPS incident, my roommate moved out abruptly. Shocking, I know. I was 23 and generally a person who worried a lot about money at that point because we didn’t have money growing up, I paid my way through school myself, and grad students are stereotyped as living off ramen noodles for a reason. Lowell had the cheapest housing in the state at that point, due in large part to its impressive record of violent crime and also its propensity to flood like it was the Merrimack River’s job.

Living in Lowell, you learned to view the Merrimack as an adversary. A worthy opponent. When a river can rise enough to cover four blocks beyond its banks in a foot of water, you respect that river. My little cousin wanted to see how bad the flooding was one time and we walked a few blocks down closer to the university. We identified partially submerged landmarks and then two white plastic chairs just floated by. You know, just on their way to meet up with their chair friends and all the other debris beside a shuttered ice cream place where the flood deposited all the detritus.

Anyway, my roommate moved out abruptly and I was stressed about money because I was always stressed out about about money. I didn’t sleep for two days and on the third night, I ordered the best, greasiest fake Chinese food and ate it while sitting on my living room floor, watching some crappy horror movie. I slept that night, and the following nights, and eventually decided I didn’t want to deal with roommate stress. I decided to live alone.

I worked two full time jobs while in grad school and was also in grad school so I didn’t have a lot of free time. This was helpful in the beginning. I was so tired when I got home that I usually just went to bed. I put Lord of the Rings in my Playstation 2, turned the volume down on my cathode ray tv, and fell asleep. I wasn’t really experiencing being alone. It wasn’t until later when I had a more normally structured work schedule that I had to learn how to keep myself company.

The funny thing is that once I learned how to be alone, I loved it. It alleviated so much stress. When I went to Scandinavia in 2007, I came home, walked up to my apartment, took off all my clothes in the living room and just left them in a pile while I made coffee. Living alone was fantastic. I haven’t lived with anyone who wasn’t my partner in several years and I prefer it that way. I like having the choice to not talk to anyone and to think about things and make decisions on my own.

Being confident in your ability to take care of yourself and support yourself is very empowering and liberating. But it can be isolating, too. In the years since I lived in my charming apartment in Lowell, I have become progressively sicker. It was pretty obvious to my partner at the time but not to most people, even people I was close to. I looked healthy and still worked and fundraised and volunteered and lived a fairly normal life. It became easy to not let on how unwell I was and most people didn’t know until I crashed super hard and needed major surgery and all that.

In the last few weeks, I have finally gotten my lower GI pain mostly under control. My baseline pain is lower but I am still having colitis attacks about twice a week for 2-3 hours. I don’t know what is triggering the colitis so I can’t stop it. The good news is that most of the time, my pain level is lower. The bad news is that when it’s not controlled, it is unbelievably painful. Like can’t stand up straight, think my intestines might somehow drill through my abdominal wall and enter the outside world Alien-style, painful. It is awful and unpredictable and really frustrating.

This morning after I got off the train near work, I started having bad lower GI pains. I got to work and was stuck there until the attack subsided. I took all relevant meds but these episodes seem to be self limiting and resistant to symptom interventions. I sat at my desk with my legs up and distracted myself from the pain with work.

A coworker and I were chatting and I told her about my abdominal pain which was presently terrible. I don’t know what caused this realization but I realized while we were talking that she had no idea I was in pain before I told her. That really jarred me. I have been in pain for so long that my body has adapted to a neutral affect in response. People not only can’t tell that I’m sick but they also can’t tell when I am in serious pain.

I felt suddenly this swell of loneliness. It was momentary but overwhelming. I made a choice to share my life openly. People read about my pain but something is always lost when you distill emotions and moments into words. Over time, I have developed a complicated and difficult but enriching life and this world exists solely within the confines of my body. My friends and family can visit this place but they can’t stay.

I am alone in this strange place. I know how to be alone. But being alone in this body and this pain and this struggle is a different kind of alone, a solitary vigil in an invisible space.

 

Second life

I feel very much that I have had two lives. My first life stretched from childhood to my late 20s when I became seriously ill. I lived a lot in that first life, even for a few lifetimes. I grew up in Boston and went camping in New Hampshire most weekends in warmer months. I was a good student and read a lot and journaled compulsively. I traveled, went to college and grad school, worked several jobs. I fell in love and had my heart broken and broke some hearts. I had a lot of fun in my first life, a fact for which I am very grateful.

My second life is the one I often write about here. It’s the one that started when I became too sick to work and could no longer function. It’s the life I’m still living right now. A life that started with me being so sick that I just wished it would be over, that I would just close my eyes and transcend to an oblivion where I wasn’t puking and in pain all the time. A life that saw me through those times and carried me here. A life that brought me to Hong Kong and Beijing, to a challenging job, to bright red hair.

And to Water Country.

Water Country is a New England institution, complete with an iconic and maddeningly catchy jingle that everyone from New England reading this just sang in their head. It’s a water park with slides and a wave pool and a lazy river. I went as a kid for several summers. My mom and her friends would pack coolers and beach bags and all the kids and mothers would pile into my aunt’s unbelievably large and hideous brown van. The kids mostly sat on the floor in the way back like you would do in a station wagon. No seatbelts. Very early 90’s vibe. I think I was 12 the last year we went.

I think 2013 will go down resoundingly as the worst year of my life. I was super sick, had a resection and colostomy, broke up very shortly after my ostomy placement, moved, and generally had an awful time. I had worked so hard to shape this second life into a place where I could feel happy and fulfilled. And then it was just gone. I called my best friend in the middle of the night after we broke up, sobbing. “I had this whole life,” I said in between the deep whistling hiccups of genuine despair. “I know,” Alli said. “You lost a lot.” And she was right.

Late in the summer of 2013, the two of us decided to go to Water Country. I don’t even remember the conversation that led to the decision. In the last week of August, when the NH kids were back in school, we drove up to Water Country. It was warm but not terribly hot. There was almost no one in the park. We ran up three flights of wet wooden stairs, went down the slide, and then ran back up to do it again because there was no line. It was fantastic.

We made the poor decision to try a slide that you went down on your stomach on a mat. Nothing bad happened, it was just so ridiculously difficult. I had a colostomy bag and abdominal pain and she had ulcerative colitis and a knee that had been operated on and still didn’t work well. We were on the mats on our stomachs and just could not push ourselves down the slide. It took several minutes and was not even that good of a ride. Water slide fail for two. Then we went on a bunch of other slides and drove home with the smell of chlorine and conditioned air in the car.

We go to Water Country every year. Water Country is a place that I solidly place in my second life. This was a tradition I started in my second life and that means that there was no comparing it to before I got sick. Even when things go well and I don’t react, there is an inherent discomfort in doing things in this life that I did in my old one. A lot of the time, there is an inherent discomfort in relationships with people who knew us before. It is a reminder of the differences and the space between who I used to be and who I’m not now. It isn’t something that makes me angry anymore but it is something that I don’t think will ever stop. There are some things that you hide deep in your heart you just never get over and when you are in these situations, you feel a twinge when they are hidden inside.

I don’t feel that twinge on Water Country day. It’s like a holiday for me, a testament to the fact that this second life is so much harder and scarier than my first one but that it’s still a good life. It’s hard and messy but that doesn’t mean I’m unhappy. I can be sick and happy at the same time. Every year, that day proves it.

I wish that I had never gotten sick and that my existence would never have split along this fault line, opened a chasm between who I used to be and who I am now. But it did. And most of the time, this second life isn’t really so bad.

Lucky day

The nights are getting cooler and the wind is no longer warm. Darkness descends earlier. Fall is close. I walk a lot but especially at night when it is just a little cool. I listen to music and look at the moon with a dog in tow. Life seems less antagonistic when you look at the ocean.

Last week on just such a night, I took Astoria for a walk to the water. It wasn’t late but the town was quiet. About a mile from home, as we walked down the block to the ocean, a man ran past us and almost bumped me. I turned to steady myself just in time to get coated in pesticide that was being sprayed from the back of a pickup truck.

I just stood there for several seconds because I couldn’t believe what had just happened. I was stunned. Astoria has saddlebags with her treats, water, bowl and bags on one side, and my epipen, IV meds, and emergency protocol in the other. My immediate reaction was to take benadryl but I was afraid I would contaminate the line with pesticides if I used it. We were close enough to my apartment that hurrying home would get me there faster than calling someone to pick me up and bring me to my place. I took the epipen out of the case and walked home as fast as I could with it in my hand.

I was home in under ten minutes. I took off all my clothes and threw them into the washer with Astoria’s collar, harness, leash and pack. I rinsed myself off so I could use IV meds and got benadryl and pepcid onboard. I took 50mg oral prednisone and gave Astoria a bath.

Astoria can be a lot of work sometimes but one thing that is never a problem anymore is bathing her. She loves water. She loves the hose. If I am trying to spray something with the hose, she would prefer what I’m spraying to be her face and will jump back and forth to body block whatever I am aiming at. If I wash dishes, she stands close to the sink so I can spray her with the little nozzle. She knows all about the bathtub and that the water lives there. When I get home from work and open the bathroom door, she ferries her toys from the bedroom to the bathroom one at a time, piling them on the bath mat. When she has all the toys she wants, she picks up her favorite dinosaur and gets in the tub. Just hanging out in the tub so she doesn’t miss bath time. If she is dirty (or not), she will actually get into the bathtub and look at me plaintively until I wash her. She is a ridiculous creature.

Well, it was her lucky day because after getting covered in pesticides, we had a long bath. She was pumped. I shampooed her all over and rinsed her really thoroughly while she repeatedly slapped me in the face with her tail. She shook off all over the bathroom because why not at this point and let me aggressively towel dry her, which meant she was at most 70% dry.

Since I was now covered in water, fur, dog shampoo and probably pesticides that I just washed off her, I took another shower. Fifteen minutes later, after I wiped down every last surface in the bathroom because they were somehow all coated in fur, Astoria started coughing. So she got another bath. More shampoo. More water. More shaking. More towel drying. More cleaning every single thing in my bathroom. She is having her best day ever.

As I’m changing over the laundry and loading a truly impressive amount of used towels into the washer, she is running in and out of the bathroom with her tail going so fast that she is knocking things off the coffee table. LOOK MOM THIS IS MY DINO. More wagging. More wiggling. She is at a cool 97 on the excitement scale if 100 is the point at which you actually detonate from euphoria. LET’S DO THE THING WHERE I WANT YOU TO PLAY TUG WITH A STUFFED ANIMAL THAT ISN’T BIG ENOUGH TO GET A GOOD GRIP ON. LET’S DO IT.

But I still need to shower myself again so I put some peanut butter in her kong for her to play with and I climb back into the shower. I am listening to music and trying to calm down as I scrub myself again. In the next room, Astoria finishes up with her kong and comes looking for me and then lets herself right into my shower. She is now 100% wet instead the 30% I was settling for earlier. She is slapping bottles of shampoo and body wash off the shelves with her tail. I have two towels left and it takes 1 ½ to dry her off. At this point, the dog has been washed and buffed dry three times in two hours. Her coat is so shiny, I can’t look directly at her without sunglasses. I’m bright red and developing my own cough so I take IV steroids and just plan to never sleep again.

My town was spraying for mosquitos that night, which is how all this happened. I have a landline for calls like the one I expect for spraying. I didn’t get a call. I usually do and realize that this was just an unfortunate glitch. I don’t think anyone is to blame for this situation. I didn’t make any mistakes and this still happened. You can plan and prepare but there is always an unknown element. The world is not entirely predictable and that means that there is always the chance that some unlikely thing will happen. I had my meds, I had epi if I needed it. I was responsible. I understood that I can’t always control my disease or my environment so I had to be ready to respond. A large part of learning to function with mast cell disease is understanding that you will never be able to completely control your exposure to triggers.

A completely different situation that also happened last week confronted me with a separate but related reality: that people often think that people with disabilities are just not doing it right. By which I mean being disabled and living with a disability. It is omnipresent and so frustrating and degrading. When we say that someone gave us trouble for parking in a disabled spot for which we have a placard, they say that if they were disabled, they wouldn’t park there because it’s not fair for people who have worse mobility.When we say that we are scared about not being able to get medication for chronic pain, they say that if they were disabled, they would meditate and rub themselves in [insert herbal remedy] and push through the pain by virtue of their willpower. When we say that we are worried about how changes in healthcare policy will affect disability payments, they say that if they were disabled, they would never allow themselves to need disability payments because they would continue to work fulltime. When we say that we can’t pay for our meds and copays, they say that if they were disabled, they would manage their money better. When we say that our insurance won’t pay for a medication that we absolutely need and should have, they say that if they were disabled, they would never let that happen to them, that they would just be so forceful and smart that no insurance would be able to deny them anything. (I just made a Michael Phelps face as I typed that last sentence. This point is a bit more sore than I thought.)

I realize that this response derives mostly from two things: ignorance, and to a larger extent, fear. It is terrifying to confront the fact that we live in a society where people can just refuse to provide you with things you need to function or even to stay alive. It is scary to think that you can become so sick overnight that your old life will never be manageable again and that you will need so much help to do anything. It is scary to think that providers and government agencies and pharmacies can refuse to alleviate your serious pain. It is much easier to think that disabled people are just doing it wrong than it is to realize how vulnerable we all are in so many ways. Few things are scarier than impotence. Maybe nothing is.

Hope you’re having a great summer.

 

Astoria has a bath

 

 

Astoria has a life jacket

 

 

Astoria has a bathtub

Unshakeable: An update on Kristina

On Father’s Day in 2008, my father had a massive heart attack. My mother, sister and I were passengers in the car he was driving to a restaurant for dinner when he suffered a full cardiac arrest. I know all of the facts about what happened because I have recited them so many times, but I don’t truly “remember” most of what happened. I remember performing CPR in the middle of the street and worrying that I was not being forceful enough with compressions immediately before I cracked his sternum. I also remember calling my uncle from the ambulance and thinking at the hospital that this was one of those moments that would divide my life into periods of before and after.

There have been a few other moments and I mostly recognize them when they are happening. I did not realize that the week spanning the end of September and beginning of October in 2015 would be one of those. I spend a lot of time reassuring people about their health, including myself, to the point that it is almost a reflex. I assume things will be fine until I am shown incontrovertible evidence that they will not. I trick my body into thinking it’s not scared by donning the physicality of confidence and busy my mind with other things.

I have a practiced eye and I should have been able to see the impending storm last year. On the other side of the horizon, a darkness gathered, spinning and spinning into a fury. But I didn’t. None of us did. I think about it every single day.

Last week, a friend commented that Naples is not too far from where Kristina lives. It’s lucky that she did because I had no idea. Kristina is a mast cell patient and mom of a mast cell baby. She is my friend and part of a group that included me and six other masto moms or patients who shared everything. Kristina suffered a brainstem stroke last October and suffers from locked in syndrome. She is aware and understands everything happening but is unable to control her body or speak.

I visited Kristina yesterday at home with her family. She is still Kristina. She laughed at my sarcasm and bad jokes. I asked her if I could give an update for everyone thinking of her and praying for her. She said I could.

The last ten months have been very challenging for her and her family because she lost all functionality but very limited eye movement in the stroke. Additionally, she is a mast cell patient, which complicates her management and treatment. Her team is aggressively working to teach her brain to make new pathways to control movement, especially in her face, arms and fingers. She is working on sitting upright and spent some time in a wheelchair while I was there.

She has regained some movement in her face and neck. She can turn her head from side to side. She can control her eye movement better, including blinking. She can move some other muscles in her face, including above her eyes and near her mouth. Kristina communicates currently by eye movement and answers yes/no questions or spells words by blinking when the alphabet is recited to her.

Kristina is able to breathe on her own with a tracheostomy, which allows extra assistance to help clear secretions and cough if needed. Her speech therapist tried a new valve on her trach while I was there and for the first time in ten months, I heard Kristina’s voice.

It is not in my nature to be hopeless, even when things are grim. In 2008, my father was in a coma after his heart attack long enough that we were approached about giving him a trach to keep him ventilated long term. We were given a target date for this procedure since he was not expected to wake from the coma before then. Instead, the vent was removed and he woke up on my parents’ 25th wedding anniversary. It has been eight years since that day and I am still grateful for that every day. The fact that my father survived has given me the unshakeable understanding that sometimes, even when it seems hopeless, and even when we can’t explain it, people get better.

Kristina is a tenacious, intelligent and eminently capable person. Her family is amazing. They are so dedicated to her recovery. Getting to hug Kristina, and her mom, husband and son, was easily the highlight of my year. I don’t know what will happen but I am hopeful. I told her that we love her and that so many people think about her and ask about her often. I told her that I was glad to see how well she was adapting to being home and that she was making gains and that I had an unshakeable belief that she would get better, too.

Thank you for keeping Kristina and her family in your thoughts and prayers.

An uncommon gift

Rare disease patients often have little in common but their disease.
We speak different languages.
We are of different races.
We live in different countries.
We have different families.
We have different ages
And politics
And religions.
We laugh differently
And love differently
And cry differently
But when you are in the moment, it all feels the same.
And because we are so different
We might never have been friends otherwise
But that doesn’t matter
Because we are.
And sometimes our differences make our friendships messy.
And sometimes our closeness despite our differences feels like cheating
Like getting a gift in celebration of something
We shouldn’t be celebrating.

Today I visited a dear friend
Who was a gift from this disease
And I sat with her
And rubbed her hands
And told funny stories
And reminisced
About the hard days
Before the hardest days came.
And thought to myself
That I will never be able to put this feeling into words.
To put this connection
Into words.
To describe
How one day
A long time ago
This woman told me about her son
And that she was scared
And when she did
The fear in my heart saw hers
But it saw the courage too
And hearts are always more courageous
When they don’t beat alone.
I will never be able to put into words
What happened inside me
When she lost her voice
And things seemed hopeless
And she was scared
And I was scared
But my heart mustered some courage
So that her heart would see it.
And we don’t know what will happen
But the only way to overcome these hardest days
Is by opening our eyes
Even when we don’t want to
And laughing
Even when it makes it hard to breathe
And remembering
Even when it twists your insides
To know
That none of us saw this coming
And that we are scared
That we won’t overcome this.

But we are part of this world
A world that gave us
Sunshine
And warm breezes
And the night sky.
That gave us
Swimming
And family
And friends
To remind us
That there is good here.
That gave us
Terror and pain
So that we would understand joy
Desperation
So that would know peace.
This disease
So that we could find each other.
These hardest days
So that we can overcome
And so that nothing
Will ever
Seem hard
Again.

Indiscretion

I am not terribly discreet about my health and the accompanying body differences. It is not in my nature. When I was growing up, I would get dressed for school in the living room in front of a window that provided an unobstructed view for anyone passing by. It took me a year to put up blinds in my current apartment because I just couldn’t be bothered. The idea that someone could see me naked is really not something that I particularly care about.

This lack of concern has extended to my day to day management of my health. This means that my health issues are pretty visible, even to people who don’t know me. My port is accessed all the time and I don’t usually wear clothes that hide it. When I had a PICC, I never wore long sleeves for the express purpose of covering the line. While many people didn’t know what I had under my clothes, they could certainly see that there was a bulge on my abdomen where the ostomy bag was. I take medication in front of other people all the time, including IV meds, sometimes in public. I sometimes need to infuse fluids during the day and so then I am walking around with a backpack holding a pump and an IV bag trailing a line connected to my port.

Also, my hair is bright red and I have distinctive glasses and dress like a 60’s housewife. Blending into a crowd is not really something that is going to happen for me.

I made a decision a long time ago to be as transparent about my health as possible in all areas of my life. During the years before diagnosis, and for about a year after, my friends and family understood in a general way that my health was not great. I was fairly functional and at that point didn’t require a lot of help to manage my day to day life.

In the winter of 2013, that changed. I crashed so hard that in the span of a few weeks I ended up unable to stand for very long, or exercise, or eat most foods, or clean my apartment, or run errands. After a couple of episodes in which I got out of bed and literally fell on my face from low blood pressure, it became obvious that I could not manage my life on my own. I was mostly unable to leave my apartment.

So I told everyone. I told my family and friends and work and pretty much everyone else I interacted with. By doing so, I was able to create an insular environment that was largely safe for me. Venturing outside of that bubble was inherently dangerous for me.

In the last couple of years, I have gradually gained more stability and more independence. I travel a lot now. In the last two years, I have been to Seattle, Colorado, Florida, California, Maine, Hong Kong, Beijing, Colorado, Maine again and Florida again. I’m currently in Naples, Florida.

Travel was a big part of my life and identity in my adult life. When I got sick, it was easily the thing I missed the most. It wasn’t even really that I wanted to go somewhere as much as I wanted to be able to go somewhere if I decided. The anxiety associated with being unable to just book a trip and go created a weird sensation of claustrophobia. I was trapped. And by traveling now, I am no longer trapped, but I am less safe.

I have written a lot about the difficulties encountered while I travel. I actually didn’t even realize how often I have written about this until yesterday when I was reading through old posts on the blog and on Facebook. I pretty much always run into trouble getting my boarding pass, despite many attempts to mitigate this ongoing problem. I sometimes have trouble even getting an airline to let me onboard the plane because some employee made a decision that I don’t look healthy enough to fly. Onboard, I often encounter trouble because I have to keep a bag with meds and an infusion pump with me at all times because the IV bag and pump are attached to my body.

And then of course there are the people who just don’t like that I’m sick. You know the people. The ones that don’t actually know you, but who make snide comments or stare.

I flew to Florida on Saturday. For the duration of that flight, I was connected to an infusion pump. The woman next to me was staring and rolling her eyes pointedly which seems awfully stupid since she could just ask me what it was if she wanted to. Two hours into the flight, I diluted a med using a needle. She finally said, “You know, it makes me uncomfortable that you’re doing that.”

I responded, “You’re going to be a lot more uncomfortable if I start going into shock,” and then pushed the med and reconnected my fluids.

Any time I venture outside of this bubble I built for myself, I accept the risk associated with a naïve and sometimes hostile environment. I find it incredible that in 2016 these issues are still present. I am clearly dedicated to educating people about mast cell disease and seize the opportunity to do so when it presents itself. But situations like this have really driven home for me that often people have already decided how they feel about what I am doing or what I look like before we even interact. In some instances, teaching them about my disease won’t help. And sometimes, it makes things worse.

There is also this sticking point: that despite the fact that I don’t try to hide my health issues, I still have a right to privacy. I don’t mind discussing my port or why I’m bright red if people ask, but I have a choice. There should not be an expectation that I disclose my private health information just because I don’t look like everyone else.

Our stories as patients are our greatest weapons against ignorance and fear about our diseases. They are our greatest assets in making the world safer for us and people like us.

But they are still your stories. And telling them is a choice.