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Thank you for helping my friend, Kristina Brightbill

I am deeply grateful for all the support this community has shown for Kristina, Lucas and the rest of the Brightbill/Daly family.

I am happy to report that Kristina is a semifinalist to win the wheelchair van! In three days, Kristina got almost 4000 votes. Rare disease community for the win!

I would also to thank the many people who offered financial or logistical support, software for speech rendered by eye movement, food for Lucas, prayers, good vibes and support. I am humbled to be part of this mast cell world, even when we all drive each other crazy.

I will keep you all posted on how this contest turns out.

Hope! We has it!

Kristina is a semifinalist!
Kristina is a semifinalist!

Please help my friend, Kristina Brightbill

Last October was a nightmarishly dark time. My friend Seth had a catastrophic metabolic crisis a few days before his third birthday so severe that I honestly cannot believed that he survived. He was in the hospital in California trying to sort out effective and tolerated IV nutrition when he crashed. Seth is a mast cell kid with no safe foods. None.

As impossible as it sounds, Seth was not the only little boy at that hospital in California with no safe foods. There was another little boy there named Lucas, who also has mast cell disease. These little boys and their parents became very close friends, bonding over the absurdities and terror of having young children who can’t eat. Lucas’ mom, Kristina, was also a mast cell patient. When Seth was crashing, Kristina called me and we worried together.

At the time that Seth was having his metabolic crisis, Lucas was a year old and exclusively breastfed due to his frightening and prolific food reactions. Kristina had removed almost all foods from her diet in order to provide nutrition that could Lucas could tolerate. Eventually, she was only able to eat quinoa, organic cantaloupe and one brand of safe water. She herself was predictably experiencing physical issues from such a restricted diet. Her breast milk was Lucas’ only safe food and she sacrificed her health for his.

The week after we worried together on the phone about Seth, Kristina was preparing to take Lucas home to Florida after months admitted in California. On a Friday afternoon, she had anaphylaxis, and then she had a stroke. No one realized what was happening until it was too late. In a matter of hours, Kristina became a prisoner in her own body. The stroke was in her brainstem and she has locked-in syndrome.  She is 26 years old.

Kristina is completely aware and able to understand everything happening around her but cannot move or speak. She is able to communicate by blinking while someone points to letters and spelling words in this way. She continues to have ongoing health issues secondary to the stroke and mast cell disease. Her needs are very, very complex.

After months of inpatient care and stroke rehab, Kristina will be going home to be with her devoted husband, their son and her family and friends. Her home has required significant remodeling and she needs round the clock care to stay safe. Kristina also needs a vehicle that can transport her in her chair in order for her to be able to safely go home. As you can imagine, meeting all of these needs is a massively expensive and time consuming undertaking.

Parents of mast cell children often say that they would give anything to help their kids, that they would gladly lay down their lives to stop their children from suffering. Kristina literally gave everything she had to make her body a vessel capable of producing a food that her son could eat.

If you have ever been helped by this blog, or me, or anything I have written, please help my friend, Kristina.

Kristina’s family just found out about this contest to win a van that can transport her in her wheelchair. Please vote for her. Voting closes in two days but it is still worth a shot.  If the link doesn’t work, please cut and paste the following address into your browser:

http://www.mobilityawarenessmonth.com/entrant/kristina-brightbill-sarasota-fl/

If you are able and would like to donate there is an ongoing fundraiser for Kristina and Lucas, who will both need expensive lifelong care.  Please cut and paste the following address into your browser:

https://www.youcaring.com/kristina-brightbill-jedidiah-brightbill-lucas-brightbill-379577

Many thanks. Please keep this family in your thoughts/prayers/good intentions. There is always hope.

Coexistence

The list of offensive things people say to patients with chronic illness is seemingly endless. Even after all this time, it still amazes me what people feel entitled to say about my health. It also still amazes me how much it can hurt.

My diseases permeate and affect every single part of my body and my life. That’s not because I’m “obsessed” or “caught up in it” or “looking for an excuse.” It’s because my body makes too many defective mast cells, defective collagen, antibodies to my thyroid, nuclei of my cells and joints, not enough cortisol, and a cardiovascular system that is pathologically incapable of maintaining stable blood pressure and heart rate. I work hard to live around the hindrance of multiple rare diseases, and I do, to a certain extent. But that’s not the same as “moving on” or “getting past it.” It’s like eventually learning to live with a roommate that leaves their hair in the drain, is always late with rent and sometimes poisons your food.

I am actively engaged in a power struggle with my body pretty much constantly. I take medication about every thirty minutes while I’m awake and continuously overnight. I plan every part of my day, including when I have to go to the bathroom. I identify triggers I may encounter and try to minimize exposure.

One of the most important ways I reduce exposure to triggers is by communicating them to the people around me. In situations where people know me and/or I am reliant upon them to care for me, I try to educate as much as possible. Otherwise, I just give my elevator speech: “I have a rare blood disorder that causes me to have severe allergic reactions to things I’m actually not allergic to. I have hundreds of these false allergies.” Sometimes I do it to avoid confusion, sometimes because I don’t feel like talking about my health and sometimes because I know that what I say won’t change their behavior.

I rarely eat in restaurants and when I do, it is because I have talked to the chef (usually ahead of time) and identified a safe meal for me. Even when I have done this, there can be misunderstandings that require my food to be sent back and made again. Even outside of restaurants, I prefer not to eat food that wasn’t prepared by me or someone I trust. There is too much risk.

People often don’t understand how easy it is to unintentionally contaminate my food. They sometimes think the amount of an ingredient is too small to elicit a reaction and therefore not worth the trouble to make it without. And then there is the biggest risk of all: the people who want to “test” me and see if I’m “really allergic.” They think it’s too much of a strain to just accept that if I say I need something prepared a certain way that it is necessary.

Or worse, they believe we can be sickened in the way we say, but feel absolved of social responsibility.  That society should not have to change to protect the few and neither should they.

When I am trying to explain the sort of things people like me experience regularly, I ask them to first think about pregnancy. Pregnant women observe a number of restrictions to keep themselves and their babies safe, such as avoidance of certain foods and medications, regular medical care, and avoidance of particular physical activities.

You know what I almost never hear people say to pregnant women?

“Why do you keep talking about being pregnant? Can’t you focus on something else?”

“It’s not healthy to talk about being pregnant all the time.”

“It’s probably not that dangerous, you should just try it.”

“I have never heard of that, I know lots of pregnant women and they say doing [triggering thing] is fine.”

“You’re not pregnant, you don’t even look pregnant.”

“You should give up your seat to that person, you don’t look like you need it to me.”

“Have you ever tried just not being pregnant?”

“Pregnancy is a mindset.”

“If I [make it without triggering ingredient], I’m going to have to cook your meal separately. Is it really worth it?”

“It’s only a little alcohol.”

“Why do you go to the doctor all the time?”

“Why would you go to the hospital for a tiny thing like that?”

“Well, it’s obviously your fault that you’re having pregnancy complications because you did [unrelated thing].”

“Your pregnancy is too much of a pain in the ass of us.”

“You can’t expect us to change things just to make it safe for you.”

“You would probably feel a lot better if you would just lose some weight.”

“You don’t need to listen what your doctor says, they are shills for Big Pharma. My cousin took [current popular panacea] and they did way better on it.”

So why is it okay to say things like this to people like me who are sick? If you replace “pregnant/pregnancy” with “chronically ill/chronic illness,” every single one of these things has been said to me many times over.

I’m a pretty brassy woman. It is not easy to embarrass me or make me uncomfortable. Except in this way. Requiring the assistance of people I don’t know well and don’t trust is inherently disarming and leaves me vulnerable. When I have to tell someone that I can’t eat that/go there/commit time to something worthwhile, I have to steel myself. I need to be safe but I also need to feel like I’m not a burden and an outsider.

I don’t ask most people to help keep me safe from every trigger I have, I just ask them to keep me safe from the worst ones. Once you start listing things, people either assume you are being dramatic or lying; or they realize you aren’t, but just don’t care. They might care that you’re sick but not enough to alter their routine in small ways.

We are not inventing our triggers to make your life more difficult. We are not pretending to be sick to get “special treatment” and are not just being “picky.” We are not sick because we don’t want to get better. We are struggling to make it through every day without triggering a reaction that could hospitalize or kill us.

Last night, my mastsister Addie was exposed to something that many people refuse to believe can actually trigger anaphylaxis.  She was not even in the same room as the trigger.  She need two doses of epinephrine, multiple doses of IV Benadryl, IV SoluMedrol and fluids.  She was sick for hours and is recovering today.

Addie asks people to help keep her safe

Please take our triggers seriously.  Please take our disease seriously.  People die from anaphylaxis every year.  In some cases, it could have been avoided if their allergies were taken seriously.

We are not telling you that the world needs to accommodate us in every way. We are asking you to help us live in it.

The Devil’s Arithmetic

When I was in grad school, I took immunology. I still have my textbook and refer to it sometimes, my crowded notes in the margins. The chapter on allergy and anaphylaxis is highlighted in green, somehow aggressively bright after eleven years.

It’s kind of amusing to recall this time in my life, before every mast cell activation pathway had been hammered into my brain. There’s also some black humor in reading about how IgE activation is the allergy pathway. You know, THE allergy pathway. This book doesn’t cover any other pathways. As if you cannot possibly be allergic to something without IgE.

That’s the problem, of course. This is what most healthcare providers or science majors learn in school. They learn about allergy and anaphylaxis, but they learn about the textbook description which invariably refers to IgE mediated food anaphylaxis. They learn about peanut allergy.

I don’t have a peanut allergy. I literally don’t have a single food allergy that displays the hallmark swelling/closing airway that people expect. But I have major food allergies, some bad enough to require epinephrine, IV Benadryl, Pepcid, Solu Medrol, Zofran and IV fluids.

The problem is not just that I’m allergic to some foods. It’s that I’m not always allergic to the same foods as I was the day before. Or the same medications. Or the same environmental exposures. My reactions on a given day are the cumulative product of the amount of irritation my mast cells have experienced in the previous day or two. There is always a running tally in my mind.

There are a lot of analogies and models used to describe mast cell attacks both to patients and to people who don’t have them. I have always thought of it as a bank. You make deposits and you make withdrawals. Like this:

For the sake of simplicity, let’s assume you have $100 in a bank account. Any activity that can cause mast cell activation has to be paid for. The cost is proportionate to the amount of activation. Getting a splinter: $2. Being hot: $10. Being in direct sunlight: $10. Standing up for 20 minutes while being hot in direct sunlight: $35. Cardiovascular exercise: $40. Arguing with your spouse: $60. Moderate pain experienced in your day to day life: $50. A painful medical procedure: $70. Mild cold: $40.

Some things are too costly to ever attempt.  Undercooked egg whites: $9000.  Massive bleach exposure: $7500.

You can make deposits into the bank with medications and physical changes. Getting enough sleep: $30. Wearing loose, comfortable clothes: $15. Doing orthostatic manuevers before standing up: $10. Taking baseline mast cell medications on your normal schedule: $50. Eating food that is warm but not hot: $15. Monitoring your exercise and stopping for breaks: $15. Wearing a cooling vest on a hot day: $20. Oral Benadryl: $25. IV Benadryl: $50. Steroids: $50.

So you have this running tally in your head all day long. When you start getting close to $100, you get stressed. You know you can’t afford to spend more than $100. Things that you could have done four hours ago safely are no longer safe. Things you could eat on a day spent relaxing at home inside with comfortable ambient temperature cannot be eaten if your apartment is too hot or if you are in a lot of pain.

You are constantly trying to avoid running out of dollars before you can get home and go to bed. Part of this is because you don’t want to trigger a physical reaction. Part of it is that this phenomenon – allergies as a function of circulating histamine/mast cell activation rather than IgE – is hard to explain briefly to people who don’t have this disease. So people will see you on a super crappy day only being able to eat one thing at a party and then four months later, when your body is much less inflamed, will see you eat three things at a party. And then it’s a thing, because these people invariably think that you are faking/being overdramatic as if somehow it is worth the effort to “pretend to have allergies.” WHO FUCKING DOES THAT?

Cost for being around someone who gives you shit for not always having the same restrictions: $75.

So everyday, you get $100. Except this is the US and our banks hate us so we have overdraft. This means that you can spend more money than you have but then they charge a steep fee and so the next day, you don’t have $100. You have maybe $30 dollars. After overspending, it can take a few days to get back to baseline.

Sometimes it’s worth it. Sometimes you can sort of game your body into getting more than $100 out of a day. This is the purpose of premedication for procedures and surgery. This is the purpose of good sleep hygiene, eating safe foods, not getting stressed, taking medications appropriately and on a schedule. You can bank a little. Not as much as you can overdraft, but you can get ahead a little bit.

Today, I went to the supermarket to grab some things for lunch at work. They didn’t have organic apples that looked in decent shape. They had non-organic apples and my safe peanut butter/honey and my safe pretzel chips. I had to run through my entire day to determine how much physical activity and stress was likely to be in the rest of my day to figure out what I could (probably) safely eat for lunch.

It’s like this all day, every day. This math wouldn’t be hard except that it’s constant and unavoidable and controls my life.

I kind of can’t believe I have to write this post

Hi, everyone –

I found myself in a really strange situation this week and feel like I need to say something.

I write this blog both as therapy for myself and in an effort to help patients with mast cell disease and other conditions.  The details that I omit are sometimes left out for privacy and are sometimes left out for safety.

Some of the drugs mast cell patients take to manage their disease can be abused, including IV Benadryl.  While I realize that the majority of people who read this blog and take IV Benadryl use this medication responsibility according to their doctor’s instructions, the fact is that this is the internet and I don’t really “know” most of my readers.

I do not and will not discuss things like exactly how I get various medications that can be abused because that could endanger me.  If I tell you that a line of questioning makes me uncomfortable and you continue to press me, I will never interact with you again.  Period.

I have made a choice to share my life but that choice does not extend to minutiae regarding my treatments that could affect my personal safety.

Thanks,

Lisa

Derivative

I am not easily intimidated. I have been sick a long time. I am used to being around hospitals, doctors and sick people. I am used to reading lab work and pathology reports. I have seen a lot of people pull out of medical crises. I have seen my intestine attached to the outside of my body, emptied colostomy bags, packed my own incisions, accessed my own port. It takes a lot to scare me.

Waking up with a fever of 103.2 two days after dental work scared the shit out of me. It’s kind of funny in hindsight in a morbid way: infectious diseases microbiologist develops tests for bloodstream infections, gets bloodstream infection. But it wasn’t funny then. I am very even when I speak to providers who don’t know me because my life could literally depend on it. I was even that day, but it took a lot of effort.

I was discharged after a few days of antibiotics and continued them at home for another week. I called out of work, a rare instance of sick time rather than working from home, because I was so exhausted and winded that it was difficult to do anything. In 2014, when the shit really hit the fan, standing up was enough to make me sweat, my heart race and blood pressure drop. It felt like that again. Like anything but being in bed was too physically demanding and being awake was too mentally demanding.

In the days after discharge, I lay in bed thinking about deconditioning and POTS and anaphylaxis and what if I had to start all over again? There isn’t a word for what I was experiencing. If we had a word for the crescendo to blind panic, the choking and the blood pounding before you scream, that might be it. What if I got these nine months of improvement and this was it?

The first few days back at work were very hard, my blood pressure was low and my mouth still hurt a lot. I had appointments last week at the hospital and one of my doctors is pretty convinced that I had a true bloodstream infection that just didn’t culture because of the antibiotics. I slept most of this weekend. But things are coming back together.

This past year, it was easy to settle back into a routine, to prioritize certain things over the things I had always dreamt of pursuing. It feels foolish now to have done that. I cannot take for granted that the way I have felt is the way I will continue to feel. If I hadn’t been on antibiotics since the dental procedure, this story could have ended very differently, with my port being pulled and time in the ICU to treat a bloodstream infection and anaphylaxis and months of recovery.

Life is short. All important things are derivative of this. Every lesson is secretly the same.

The unseen hand

I have spent a lot of the last several weeks trying to navigate the dark waters of mid-level care for a rare disease patient.  It is one of the trickier aspects of life with a disease like mine.  I need care and need providers to be cautious but I also need them to not be scared of my disease or they could refuse care.  When I’m shocking or having a serious medical event, it’s not really an option to refuse care.  But when something is serious but not life threatening, I have to tread carefully.  It requires just the right amount of education given confidently.  The tiniest twinge of voice, a catching of my breath while I’m talking about my disease, and they could easily walk away.

I got my tooth removed on Tuesday at a dental office in the same hospital where I receive all of my care.  The dental team was very good and took my special requirements in stride.  But I realized pretty quickly that this extraction was going to be harder than I had expected.  All told, it took three people two hours of actively trying to extract the tooth to achieve success.  EDS patients are often insensitive to anesthesia and they stopped several times to renumb everything.  There was some bleeding and a bit of bruising but nothing that felt inappropriate given the nature of the procedure.  I went home with an order for antibiotics and ketorolac.

My mouth hurt a lot Tuesday night and Wednesday but the pain didn’t feel disproportionate to the violence of the extraction.  I slept a lot and iced my jaw.  I could eat gingerly and talk.  Overall, I felt okay.  Not great, but okay.  I ironed work clothes on Wednesday night and got everything together to return to work on Thursday morning.

I woke up Thursday not because I had to work, but because of how badly my face hurt.  I had medicated before bed and it should not have worn off by that point.  I knew right away that something was wrong.  I flipped on the bathroom light to reveal flushed cheeks and glassy eyes, my hair matted to my forehead with sweat.  I put a thermometer into my mouth and started putting things into a backpack as the numbers climbed precipitously.  I had a fever of 103.2.  In less than an hour, I was in the emergency department of my regular hospital.

Dental procedures somehow manage to masquerade as routine despite the fact that they take a lot of skill to perform safely and that some of them are inherently risky.  Though the guidelines have been revised in recent years, many patients are still recommended to premedicate with antibiotics prior to dental work.  The reason is that it is very easy to transfer bacteria from the mouth to the bloodstream, even when all appropriate safety measures are followed.  If you have an artificial heart valve, or certain other conditions, there is an increased risk that these bacteria will become a true bloodstream infection, which is very, very serious.

The emergency department took me in right away.  I am always nervous in emergency departments because I have had a lot of trouble in the past, but everyone was great yesterday.  They knew about SM, they knew general guidelines and gave me no shit about needing hydromorphone for pain relief.  They were fine with following my home IV orders and using my port.  They worked together with my mast cell specialist, primary immunologist, dentist and PCP to make sure everyone was on the same page.

The big concern was that I had a bloodstream infection, or at least a bad dental infection, and that it would colonize my port.  My mouth looks fine and my white count is normal (which in itself is unusual, as it has been in the 16-20K range for years).  The port looks fine, isn’t red, tender or swollen, and works fine, which is great news.  All of this is good news except we have no idea why I have a fever of 103.

My doctors are generally fine with me handling things on my own at home, so I didn’t protest when they all said I should be admitted.  There is concern that because of some of my medications (like prednisone and Enbrel) that my bloodwork won’t show that I’m fighting an infection until it is severe.  So they admitted me to a surgical floor where I have been before and I’m just hanging out for a few days until we are convinced my blood cultures are truly negative.  The care has been excellent and there has been no bullshit about how I dose my meds, how often I use IV meds, or doing things the way I want them (dilute and slowly, as a rule).

I am on a stronger antibiotic as a precautionary measure, but I expect to have negative blood cultures and to go home in the next day or two.  The most plausible cause of this sudden high fever is that transient bacteremia, in which some bacteria from my mouth entered my bloodstream during the extraction.  My body then generated an immune response and was able to keep these few bacteria from becoming millions of bacteria in my bloodstream.  It’s basically a best case scenario.

I used to develop diagnostics for bloodstream infections.  I develop molecular diagnostics and have for several years, but my training is in microbiology and my focus was infectious diseases.   When I realized this tooth needed to come out, my biggest concern was that the tooth would get infected and I would get a bloodstream infection or that bacteria would be transferred during the extraction and I would get a bloodstream infection.  Most of the time, the most serious risk associated with a procedure I need is that I will have anaphylaxis.  This was different, and the way it played out proved that I was right to worry.

In the weeks leading up to getting this tooth removed, I talked to people in several dental/oral surgery offices, including the one where I was treated regularly for over fifteen years.  Some of the realized that I was not trying to be ridiculous and that there were rock solid reasons for me to want certain materials to be used, certain meds to be avoided and so on.  But, as there usually are, there were also several people who just thought I was being particular and difficult.  When people think this about me, they usually believe I have an anxiety disorder and that my need to control things is an extension of that.

And when they say to me, “You can’t control everything” with a snotty lilt on the last syllable, it’s not a bitchy imparting of a common adage.  It’s a warning.  For me, not being able to control everything could be catastrophic.  In some instances, it could be fatal.

The need to understand the entire procedure and know everything is not something I relish.  It is exhausting and scary.  Even when everything is done right – like my extraction on Tuesday – there are still risks.  There is still an unseen hand that can push us over the edge for no reason at all.

I have a short speech that I give to every single provider I meet who has not met me before.  It starts with, “Before we get started, I have a mast cell disease.  Have you ever treated anyone with that before?” I hit all the high notes briefly and tell them I will be using my Epipen first and then asking for help if I anaphylax, not the other way around.

Tonight I gave my speech to a new nurse.  We chatted about mast cell disease for a while.  “Does it bother you?  Having to teach everyone about this?”

It doesn’t, but if it did, it wouldn’t matter.  It’s my only chance to stay ahead of the unseen hand.

Fragility

I have a bad tooth. It needs to come out. The original plan was to have it removed in an OR so I can get twilight sedation but my insurance doesn’t want to pay for it and I’m left with having to cobble a plan together myself. I called a number of oral surgeons and no one wants to give me anesthesia outside of an OR. So any kind of general anesthesia means OR, which means a several thousand dollar bill from my insurance. No dice.

I have had dental work with local anesthetic and it’s not ideal but it’s okay. I premed heavily and then it takes a day or two to squelch reactions. It’s not super comfortable but it’s not life threatening and fortunately my laundry list of past procedures means that I have got pain management down to a science. I called my doctor and he agreed that using local sedation is fine if I premedicate. He is very good at giving advice for procedures and talking to providers that aren’t familiar with me. Great. All systems are go.

I have been a patient at my current dental office for about half of my life. I call them and they schedule me to have my tooth removed. Around this time, my dental pain went from sucky and uncomfortable to my entire face and all my teeth hurt and the pain is making me nauseous. Then the long suffering secretary at the dental office calls me to tell me that the dentist won’t remove my tooth with local anesthesia. They also won’t fill the cavity to make it more comfortable until I can figure out how to get this removed.

I talked to the dentist at length and will spare you the gory details of our exchange. I now had to find someone who didn’t know me who would agree to remove this tooth with local anesthesia quickly because the pain was awful. My entire face hurts and I’m reacting and it’s painful to talk, eat and be alive, and I’m terrified it will get infected.

The dental office at my hospital eventually agreed to do it with a local on a day when my specialist will be on campus in case anything goes wrong. In two weeks. A filling would be the same wait. So I’m getting it removed in a week and a half and while I am medicating to deal with the pain, it still hurts. It hurts a lot. I have had bowel obstructions and several surgeries and a million painful tests and good grief does this tooth hurt a lot.

I am so much better than I was a year ago. I can eat solids and exercise and travel and I’m not constantly riding the line that demarcating when I need epinephrine. I have made so much progress. But damn if it doesn’t feel like I am one bad day from losing all these gains. One bad tooth, one obstruction, one flu, one slip on an icy sidewalk. It wouldn’t take much to be right back where I was. Almost nothing.

When I have described my body as strong, it has never felt like the right word. Enduring, maybe. Durable. Not strong. Things that are strong and robust can withstand damage and still work fine.

But some things are not meant to be strong. It is not a defect, but an intricacy. A byproduct of artisanal process of craftwork. Not a mistake.

All beautiful things are fragile in some way. Marble cracks, pictures fade, buildings burn, people change. Beauty is a moment, the coalescing of so many things to form this fleeting arrangement. It is the impermanence that makes things beautiful.

My body has survived impossible things. It has recovered. But it isn’t strong, even if I want it to be, and saying that it is because of one good year feels like a lie.

Ill fit

I haven’t been posting as much of my personal writing because I am working through a lot of things.  It is hard to think about and hard to write about.  2015 was an incredible and powerful year for me, in both good and bad ways.  It seems impossible that all of the events of 2015 are bound together by time.  It was exhilarating and triumphant and horrifying and so, so costly.

I am very good at minimizing and compartmentalizing, especially when it comes to my own health.  My health care is like business for me.  The actual process of managing my physical health is stressful and difficult but it has never been the hardest part of this experience.  That hardest part is all the things I feel like I lost. No amount of struggle can force those into discrete pieces to be boxed up and pushed aside.

The loss of those things hurts more now that I am more stable and things are less emergent. I am no longer living in one continuous crisis. It has given me some distance to reflect on my life and my health and all these plans I used to have.  I used to write about them every night before I went to bed, quick notes on moving toward a goal or long essays on all the things I wanted to do.  Then I went to sleep one night and woke up the next day and none of those things ever happened and I stopped trying to make them.

I think a lot about the life I used to have.  But for the years in between, it is, in many ways, not terribly different from the life I have now.  Every day, it feels more and more like I was never the person who wrote those journal entries.  I remember her, but that’s not the same as being her.  I don’t even know when she left.  A new season, then two, and suddenly it has been seven years since that girl even existed.

I’m trying to pick up these pieces she left and recraft these dreams, to remember the way they made me feel.  I am trying to fit into the space I occupied before I got sick and I just don’t anymore. It’s like forcing something into a place it doesn’t belong, hitting it hard with the flat of your hand until it splinters and your hand hurts.  Anything can fit if you hit it hard enough, but it will never be whole again.

February 29 was Rare Disease Day.  I wanted to write something positive because I’m a very positive person and because I am hopeful and I want people to be hopeful, too. But the truth is that every sick person has been traumatized by their disease and there will always be days or hours or moments when they feel that keenly.  We can overcome and live good lives but this history follows closely and it takes very little to run your mind over it.  Sometimes it is hard to get out from under that.

I thought all day about a story that could make people understand what it means to have a rare disease, to see what I see, but I don’t think that story exists.   There is no rare disease story, just like there is no systemic mastocytosis story, or Ehlers Danlos story.

There is only my story. So that’s the one I’m telling.

Degranulation station

Most of my teeth erupted later than they were supposed to, some by years. My last molars came in when I was 25. I had my wisdom teeth and three molars removed around that time. We left the one that wasn’t fully erupted. When it finally emerged, it had a cavity. My first cavity, in a place where I couldn’t brush.

In the last few years, I had more cavities, all in that same tooth. One of them was a pretty straightforward filling. The other was at the gumline and caused by acid rot from vomiting. My dentist patched it up but the placement is awkward and over time, it has fallen out and gotten bigger.

I throw up pretty regularly and take massive antihistamine doses that dry out my mouth. So it was not terribly surprising when last week, my tooth started hurting a lot. I walked down to my dentist’s office and verified that I do in fact have a huge cavity in this same tooth. Given the damage, I would need a crown to fix it, and that requires lots of strange materials in my mouth. In order to have it done by dentists who know me and my disease, it would take multiple procedures as well, and I have to premedicate heavily for each procedure.

“Or we could just pull it,” she said. That was the winner. Take it away.

I react really badly to pain and as long as my pain is managed, I have no problem with procedures. But this requires some frontloading with IV meds and the ability to give more if necessary. My dentist can’t do any anesthesia but local in her office. I called oral surgeons who extracted my teeth before I was diagnosed. They wouldn’t work on me in the office because I’m a high anesthesia risk. (Which, in fairness, I am.)

So I visited the oral surgery office at the Brigham which inexplicably does not do IV sedation unless you are in an OR, and everyone agrees I need twilight sedation so that means I need an OR. First available is in late April. I called all the people and did all the things. Unless I end up in the hospital as a result of this tooth, they can’t move it up. I’m trying to come up with a workable solution, which I expect will be something like temporary filling to get me to the April date. Just ridiculously irritating.

I expect things like having pieces of my GI tract removed or devices implanted in my body to be complicated. It is the complexity of things that should be easy that is difficult. It should not be this hard to get a simple extraction.

I have been having this issue with my vision recently where later in the day, my vision feels darker, but when I turn on more lights, it hurts my eyes. I made an appointment to see an eye doctor at the same practice as my PCP. This guy took one look at me and my port and said I needed to see a specialist to have my eyes dilated because he wasn’t comfortable dilating them. I have my eyes dilated every two years. It’s never a problem. So I had to make another appointment for next month and now wait for that. Stupid.

I am fortunate that I am not particularly sensitive to chemicals, especially given my line of work. But I am very sensitive to certain cleaners. I’m so sensitive to some that everyone who works with me knows about it. I’m so sensitive that when I started telling this story at work today, I said, “You know how I react to bleach?” and my coworkers nodded knowingly, “Yes.”

Anyway, I came home on Tuesday and opened the door to my hallway and in thirty seconds was on my hands and knees on the floor, coughing and spitting out mucous. My landlord had cleaned the hallway with something that triggered a reaction. My respiratory tract was on fire and producing mucous like some gross sci-fi monster and this nasty wet cough and a headache that felt like it was trying to scalp me and I have lived here for three years are you fucking kidding me right now?

My dog got very upset and ran around the apartment barking authoritatively at lights while I lay on the floor coughing and feeling sorry for myself. It took two days to get rid of the smell using an aggressive amount of door opening, haphazardly arranged fans and kitty litter that my dog is scared of.

Some readers sent me messages to make sure I was okay since I just dipped out with no explanation. I’m right here, at Degranulation Station.

*makes choo-choo train noise*