my feelings

You build your life around your secrets. Around other things too, but never more than your secrets. They are always there, in every place, the silent beat at the end of your words, the air you take in when you gasp. They are a comfort in their familiarity, a bane in their power. When you go to bed, it is just you and them, whispering to each other in the dark.

I have plenty of secrets. Most of us do. Most of them don’t matter. They remain secret only because I have never had occasion or need to reveal them to another person. But those aren’t the secrets I am talking about.

My biggest secret is that I am hopeful but I am afraid all the time. I have been this way a long time and so I am skilled at working around. I can talk myself into most things, even if I’m scared. But the doubt and uncertainty can be overwhelming sometimes.

I have another secret, too. I am scared that I have enough health to follow my dreams but not enough to achieve them. I grapple with this every time I have some wins and gain any measure of control. My mind immediately starts planning, lays out these paths to things I want badly but that my body could never accomplish. It is painful and frightening.

I think I’m finally coming to the end of this rough season. I am eating some solids again. I am reacting less. My mind continues to make plans that I don’t know I can achieve.

We so often equate success with results. That’s why we feel like a failure when we can brings these things we want to fruition.

But success is more than that. You have to try. The result may be the same but your heart knows the difference.

I receive excellent health care. I have a large care team including an attentive PCP that is willing to take advice on managing mast cell disease; a mast cell GI specialist; an excellent colorectal surgery; a wonderful home care nurse; and a bunch of other people. I live in a city renowned for health care innovation. When I call 911, the ambulance brings me to my local hospital, a place that treats mast cell patients from around the world. I have access to medications I need. My providers are open to hearing my input because they acknowledge that I am an expert in these diseases. I have great insurance and can afford the costs of my healthcare. I can afford safe food. I have a safe place to live. I have local support from family and friends. I have a job that gives me the flexibility I need to balance my health and my work.

I am extraordinarily lucky in these respects. I am very, very cognizant of my privileges in receiving health care. Despite these privileges, I struggle every single day to facilitate my health care. I spend hours on the phone. I have an excel sheet that tells me when I need refills of my meds, when I have to schedule appointments, when I am due for various tests, and how much all of that will cost. It keeps track of prior authorization numbers, which pharmacy or office is responsible for prescribing/ordering and the name and direct line of my contact there. I have notes all over my house and reminders littered throughout my daily journals. But even when I do everything right, I sometimes have to exert a superhuman amount of effort to get something as straightforward as a refill for a harmless medication with no potential for abuse.

I have written before about how stressful this aspect of my life is. Nothing is ever easy. And everybody’s life is hard, whether they are sick or not. The difference is that rare disease patients have so many more high stakes things to do on a daily basis. It’s not an option for any of these moving pieces to fall off the board. Letting even one thing falter could be catastrophic. I am constantly worried that I am forgetting something. Not realizing that I’m out of one med for a few days can result in hospitalization. Accidentally putting two of the same pill into my morning slot in my pill box could sicken me for days.

There’s another facet of this experience, too: that most of the things we need require cooperation from someone else. An admin, a nurse, a pharmacy tech, a doctor, a case manager. These people all wield a disproportionate amount of power.

Part of learning to survive as a rare disease patient is learning how to finesse relationships and convey your needs without being overbearing. You have to learn how to converse with people without arguing, ever, because once you are arguing, you have already ceded control. It is not easy. No one likes to be told how to do their job, even if they are doing it wrong and you can improve their skills and your care. No one likes to be spoken sharply to, even if they deserve it. No one likes to feel like they are not good at their job, even if they’re not.

But the biggest danger is this: that even if you do every thing right, if you say and do all the right things, if they accept your demonstration that you need and are entitled to these things, sometimes they still won’t cooperate. Not necessarily because they don’t like you. Just because.

When you tell this to people living outside of the machinations of the healthcare establishment, they think you’re nuts. Why wouldn’t they help you, if you’ve given them no reason not to? But to say that these people would never deprive you without reason is to ignore a key feature of human nature: that sometimes, people just want to see what will happen. And in health care, when many people view patients as obstacles or enemies, they often find out what will happen: they will win a tiny victory, and we will suffer.

I saw over 100 doctors before I was diagnosed with mast cell disease. I was a long term patient for several years in a department that treats my disease before it was caught. In that time, I collected a number of diagnoses, some correct but not the major problem, and many others that were completely inaccurate. Those diagnoses follow you.

So even now, several years and lots of irrefutable document after correct diagnosis, providers who don’t know me see this past history. They see lots of doctors and lots of conditions and lots of meds. And if they’re so inclined, they see that I am a crazy lady who doctor shops and vies for medical attention. They often don’t see these data inside of the complete narrative – that I am a victim of this system, not a perpetrator of it. That I went broke treating all of these diseases I never had because doctors told me I did and I was so desperate to feel well. That my assertive instruction is not condescending but educational. That I want to feel as well as possible and that I can help them achieve that goal.

I have almost as many privileges as you could possibly have as a rare disease patient. These things still happen to me. Consider how often they happen to people in less ideal situations.

Remember when you interact with people like me that we are sick. We are exhausted and desperate and afraid to lose any more than we already have.

We are not trying to alienate you. We are not trying to do your job. We just want one thing to be easy, even once.

I can understand most things if you can drop it into a living system. If a body can do it, I can imagine it. But there is this thing about the inner dynamics of the human organism: that the more you study it, the less obvious causality is. There is no one way to arrive at an end point. There are dozens.

The body is clever. It is redundant. It learns. If it wants to, the body will find a way.

Mast cell disease is largely a consequence of this fact. Every patient has encountered this. If you treat a symptom with a med, it will crop back up a few months later. If you arrest mast cell production, your body finds a way to circumvent it. If you get stable at a certain dosage, you eventually need higher doses to achieve the same effect.

I think about the intricacies of my body and my diseases almost constantly. Every time my body does some new mysterious and irritating thing, I run through the various possible causes. I try to determine which pathways I have blocked and which can still be used to injure me. The body is cunning. It has many tools at its disposal that can be weaponized toward a singular goal.

But there is a flip side to this ingenuity: we are more than just our bodies.

I first met Yssabelle Eddlemon when she was airlifted to Boston from Oklahoma right before Christmas a few years ago. I had spoken with her mother both online and on the phone prior to meeting her. Yzzy and I had a lot in common. We both had major colon involvement, frequent anaphylaxis, persistent anemia, ports, an ever dwindling list of safe foods, and a short supply of treatment options not yet tried. But she also had mastocytosis in her skin, major liver involvement, and such severe airborne reactions that she mostly lived wearing a mask. And she was five years old.

I became friends with Yzzy’s mother in the way that you do when your lives are miserable in similar ways. I became more involved in Yzzy’s care in the way that you do when you don’t want a kid’s life to be miserable in the same way yours is. I spent a lot of time reviewing her labs and pathology reports and learning about her. She became one of my little people.

She was seen by a ton of doctors, all of whom agreed she was very sick but didn’t know what to do about it. Her implanted port remained used for months after it was placed because no one wanted to be responsible for it. She was in and out of the hospital with anaphylaxis that closed her throat in seconds on a weekly basis. It was a struggle to keep her alive.

Eventually, Yzzy was able to get into a pediatric mast cell specialist in California. Things changed a lot, in a good way. Her meds were revised significantly. IV meds were prescribed to help manage anaphylaxis. The difficult decision to completely remove oral nutrition paid off.

After a few months on TPN (nutrition given completely by IV), she stabilized a lot. A scope done before starting TPN showed that she had confluent sheeting of mast cells in her colon – literally wall to wall mast cells in her colon tissue, so many that they couldn’t be counted. After a year on TPN, her colon biopsy was normal. For the first time, Yzzy was stable enough to go to school for half days while her mom stayed close by. She made friends and loved school. Her quality of life improved dramatically.

Then something happened that I did not expect: she started having these bizarre episodes of crazy high fever and hemolysis. The first few times, we thought it might be her central line so it was treated as an infection. But it kept happening and it became pretty obvious that this was not an infection. There are so many ways for the body to arrive at a sudden fever. No one could figure out the cause, including me. There were too many possibilities and not enough evidence to justify any one of them.

Patients with central lines are advised to go to the emergency department if their fever is over 100.5F. Yzzy’s fevers were sometimes over 105F. She would be brought in only to be sent home in the morning with no treatment and no explanation. She was also deteriorating in other ways. She had to stop going to school. Bizarre symptoms and bloodwork abnormalities piled up.

All said, she was brought into the ED 22 times over the span of several months before anyone figured out what was going on. Last fall, Yzzy was diagnosed with another rare blood disorder: hemophagocytic lymphohistiocytosis. Her immune system was eating her blood cells.

Things happened fast after she was diagnosed. She had suffered significant damage because HLH had been untreated for so long. They initially tried biologics and high dose steroids but the episodes continued. Then they started chemo. It was around this time that we started to grasp the eventuality of the situation. HLH can be fatal. Treatment was slowing it down but it wasn’t stopping the attacks enough to protect her life. She was going to need a transplant.

The weeks after the decision to proceed with transplant were tense and grim. She was frail and the chemo was making it worse. She lost all her hair. She swelled badly from the chemo and steroids. She had a recurring upper GI bleed. She was admitted most of the time. Managing both her systemic mastocytosis and HLH was complicated. Coordinating care across specialities was difficult and frustrating.

Yzzy had a rare stroke of good luck then, one so good that I actually cried: the search for bone marrow donors turned up three possibilities, two of them a perfect match. The transplant was scheduled for just after Christmas. She was discharged so she could spend some time at home before being admitted for several weeks for the transplant.

In early January, Yzzy underwent a brutal course of induction chemo. She developed major clotting issues and severe anemia. A second central line had been placed and was constantly problematic. She was miserable. But she made it through. And on January 12, she had the transplant.

Bone marrow transplants are dicey for the simplest patients. Yzzy is not simple. The risk for serious complications and death were significant. But it was the only option to manage her aggressive HLH. There was also a silver lining, a big one. If the transplant worked, it could cure not just the HLH, but her mast cell disease.

It is impossible to overstate how much we expected a disaster. But there wasn’t one. The transplant went perfectly. In under a week, we started seeing signs that her the transplant was making blood cells for her. In under a month, the transplant had engrafted and replaced her old bone marrow. She stopped having mast cell reactions. She was weaned off her continuous benadryl drip. She started taking oral meds instead of IV. She started trialing things for oral feeds. Her TPN infusion time was decreased. For the first time in years, she was not attached to an IV line 24 hours a day. And her HLH was long gone.

Yzzy has never known a life when her body wasn’t trying to kill her. She’s not old enough. Her body has damaged her organs, caused seizures, and repeatedly sent her into shock. For seven years, Yzzy’s body found ways to work around every treatment, every medication, every change that was made to keep her safe.

But we are more than our bodies. When her team discusses her care plan with her parents outside the door to her hospital room, she plays video games. When she isn’t strong enough to walk around, her parents drive her around to catch pokemon. When she is puking constantly, she plans the menu for a day when she can eat. This is her life and she just lives around it.

Yesterday, fifty days after her transplant, Yzzy went home. She joined a Girl Scout troop and is aggressively selling cookies online. She is making plans for her birthday party in May. She is happy to be home and reunited with her little brother. She is having Nerf gun fights. She is strong enough to run around and can laugh without risking anaphylaxis. This is the dream.

Yzzy is more than her body. And her body was no match for her.

I am struggling a lot with grief lately. As it has become increasingly apparent that my life and my body will never be the way they were before, I have thought a lot about what that means. How they are different. If I can live with it. I think few things cause as much personal revelation as grief. Every fear is amplified. Every dream is farther away.

I had such a firm idea of who I wanted to be. I knew exactly. I wanted to be a doctor and travel around the world. I wanted to get married and have kids. I wanted to treat infectious diseases. I wanted to live in twelve countries for a month each to live abroad for a year. I wanted to buy a little house and paint it purple. I wanted to have a home where every shelf was low enough that I could reach it without standing on something. I wanted to be happy. I wanted a quiet little life with a rewarding career and children. I knew the life I wanted. I loved that little life.

You do not naturally love the things that populate the set of your life. You do not love sitting on the couch. You do not love drinking water. You do not love walking your dog. You do not like to breathe. You do not love every moment when your throat isn’t swelling. The moment you begin to love these things marks a fundamental change. It happens when you glimpse a life beyond the veil, a life where you can’t take out your own trash or drive or clean your apartment. Everything you do is imbued with a frantic appreciation. You come to love these things but you wish you didn’t have to.

I really loved my life. It was beautiful. It was warm and full of possibility. I wasn’t grateful for breathing or waking up to an alarm clock because I didn’t have to be. I could never have imagined how bad things would get and how hard it would be to become a different person with different goals and different dreams.

It has taken me years to build a new life. Nothing beautiful is easily repaired. There is beauty still but it is deeper and less obvious. It is not the excitement for the future. It is not the having of things and opportunities. It is the rare moments when you aren’t struggling. When things are wrong but not more than usual. When the pain is managed. It is the kind of beauty you can only find when everything around you is burning. Beauty is nothing. It is a feeling. It is silence and a heartbreaking void where you can rest for a little while.

I am trialing a new biologic tomorrow in the hope that it will help me to eat again. I am scared that it won’t work. I am scared because I don’t think I can live like this. I am tired.

There is still beauty in my life. It is just harder to find it when every minute is a struggle.

I don’t handle change well. I have never liked it. A few years ago, I bought a little piece of wall décor that says “Embrace change.” It hangs next to my bathroom sink. Whenever I stand in front of the sink, I catch myself shaking my head as I read it. I keep it up mostly for irony.

My body doesn’t handle change well either. Lately, it is doing things that it has never done for reasons I can’t determine. Last night, my face swelled as I was reading on my couch. I didn’t have any other symptoms and still don’t know why it happened. I sat up for hours, compulsively poking my face and trying to determine if other parts of my body were swelling, while trying not to panic and induce symptoms that could be confused for anaphylaxis.

I’m starting to realize that I’m not having a bad episode that will resolve and return me to my previous baseline. This is different. I am different. My body and my disease are evolving. Whatever baseline I arrive at will be different from before. I can’t wait this out because it’s not going to end. I will stabilize but I will not be the same. My body will not be the same and my life will not be the same.

So it’s time to stop waiting for this to end. I have to learn how to live my life like this. I have to learn how to fit all the things I love and that bring me joy into this smaller space. I have to find a way to get light in here again because this darkness is suffocating. It has swallowed so many things.

I have spent a lot of time staring into this darkness in the last few months. Some of it with an audience, rehashing it for paramedics and ER doctors, mulling it over with my care team. Most of it alone, in moments when I can’t breathe, suddenly struck by the fact that this disease will almost certainly kill me.

But there are bright spots, even if they seem farther away as the arc of my life grows longer. Good days and moments of joy and traveling. Touching the Great Wall of China. Eating Reuben sandwiches. Walking 60 miles in three days. Encouraging words. Hugs. Seattle. Roller coasters. Kindness. Alone these moments are just tiny flecks of light, but you can gather them. If you string them all together, you can make a tiny sun.

It was really warm in Boston this week, almost 70 degrees Thursday and Friday. I packed up my infusion pump and meds and supplies and walked to the beach with Astoria as I infused IV fluids. I sat in the shade at the beach wall as she ran around, flailing around in the sand and playing with her ball. We walked slowly. We didn’t stay very long. But we got there.

The sun only grows if you feed it. Otherwise, those moments are just holes in the dark.

This is my life now. I am learning how to enjoy it. I am choosing the sun.

I do yoga everyday. It is the form of exercise most suited to both my limitations and my needs. I suppose also well suited to my personality. On days when I feel strong, it lets me balance on my hands and fly through the air. It lets me draw my legs up from my core and see the world upside down. On days when I don’t feel strong, I lay my weight into various muscles, stretching them back to their right shape. It lets me think it’s exercise even when all I can do is sit on the mat and breathe. An achievement instead of survival.

On Thursday afternoon, while waiting to check in for my appointment with my mast cell GI specialist, I had anaphylaxis. I was standing and wasn’t immediately sure if it was anaphylaxis or POTS because they both start with a fuzzy lightheadedness. I sat down on the floor and within a few minutes, it became obvious that this was definitely anaphylaxis.

I told one of the admins that I was having anaphylaxis, took a few steps into the bathroom, lay down on the floor and used an epipen. They called a code team and very quickly there were a lot of people. I drew up and pushed some IV meds. I was already infusing IV fluids. They took me to the ER where I stayed for several hours to be sure I wouldn’t rebound or have a biphasic reaction.

The resident asked if I knew what the trigger was for the anaphylaxis. “I think standing up,” I said. It was funny in the way that terrifying things are sometimes funny.

My body is so different now. In every way, it seems. I buy new clothes online because everything I own is too loose or ill fitting. As I put them on, I find all these sharp places beneath my skin, angles where there used to be a softness. The layer of fat over my muscles has thinned considerably. It has the strange effect of making me look stronger and more toned when really I am just unhealthy.

I write constantly and so I always have a journal in reach. I was recently looking through previous entries for something. I was struck by how often I speak of my past self in the third person. I talk about myself like a character. Like I’m the narrator in a story that doesn’t involve me.

I say that I am a different person now because it’s easier to believe that the person I was then could never become the one I am now. It is easier to think that this person is entirely separate for a lot of reasons but mostly so that she could never decay like this. It is much harder to accept that I was that person that did all those things and that I am still that person and never will again.

The days are getting longer and warmer. My mind is working better. My body is well enough to be out of the hospital. I can’t keep down much elemental formula but can get down some nutritional drinks and plain potato chips. I am starting to make plans for later this year that I believe could be realistic.

Every day, I roll out my purple mat. Today I can only do a little and tomorrow I will only do a little but one day, I will be strong enough again to fly.

 

 

(PS: Hypermobility much?)

Long before science gave us the knowledge that the universe is populated by revolving celestial bodies, people attributed many things to the magic of the moon. A bright, shining globe traversing the sky and controlling the tides, it was blamed for all manners of catastrophe and natural disaster. The sky and all the things that live there are still frequently seen as symbols of a larger truth. My own writing is littered with examples of this.

Last night, there was a full moon, a lunar eclipse and the close passing of a comet. Even the eclipse felt unusual, a threat of darkness across a snow moon bright over Boston after a barely finished blizzard. It was a fitting end to an unusual week.

A friend who also has SM came to town for a family emergency. She had planned to stay over for one night but we had a blizzard so she stayed over for three. We hung out and played with the dog and watched documentaries. We chatted about some exploits we plan to undertake when we can collectively get our health issues to stabilize. We went to Disney World a few years ago and have always planned to travel abroad at some point.

I started trialing elemental formulas last week. So far, everything I have tasted has been vile. I can force myself to do a lot of unpleasant things but I cannot get much of it down. I am back to Orgain protein with almond milk and maple syrup. My doctor approved adding back in plain potato chips to help with getting in calories and fats so at least I don’t feel like I’m starving all the time.

Some things are improving. I’m not vomiting every day. My lower GI issues are largely the same but my bleed has slowed. I’m still tired but my stamina is increasing. I have had to step up pain management but no major reactions or anaphylaxis. I have appointments this week to determine what the next step is. I’m starting Xolair soon and am hopeful that will allow me to eat more normally.

I have spent a lot of time speculating about exactly why I got so sick so fast. Pesticides from getting spraying are a possibility. But there are lots of other possibilities, too. Stress. Overall damage to my GI tract. A sudden reaction to something I previously tolerated. A strange moon.

It is hard for me to accept that my disease doesn’t need a reason to worsen. I can do everything I am supposed to and still get sick.

I am allergic to alcohol. I can get away with small exposures, alcohol on my skin or my port line claves, the antiseptic coolness. Ingesting it is a different story. My last sip of alcohol was the day of my cousin’s wedding before the wedding party headed to the church. Champagne bubbled in pretty flutes that we raised to toast. I took a sip, the tiniest sip, with the bride and the other bridesmaids. As soon as the champagne hit my tongue, my entire mouth went numb. I sit it out and never drank again.

I was never a big drinker. Partly because I didn’t like the taste – I was well into adulthood before I discovered how to make drinks that I enjoyed. Partly because I was so prone to hangovers. Mostly, it was because my family has a long and sordid relationship with alcohol.

Alcohol has always been part of my life. It is the buzzing undertone to a million memories. It is the cousin that gets you in trouble, that tells lies about you that everyone believes. It looks like it belongs and by the time you realize it doesn’t, it is too late. It has hooks and claws in everyone, trapping them together and pulling them apart.

I was wary of alcohol more than any other drug because I had lived with the horrors of alcohol abuse. The screaming, fighting, slamming, breaking, volatile, relentless horror of alcohol abuse. The secrecy and the denial and the despair of alcohol abuse. It was in my home growing up. When I was a teenager, I dated a man 8 ½ years older than me who had a raging drinking problem. Friends, cousins, uncles. I knew all about the danger of being in the blast radius.

I tried to cultivate a casual drinking habit when I turned 21 but I was never very good at it. Being able to experiment and broaden my palate helped. I liked novelty beers, like Wachusett Blueberry Ale. I liked pumpkin flavored beers and hard ciders. A friend of mine was a big wine drinker so I learned about wine. I liked buttery white wines, rieslings and chardonnays. Red wine made me sick. Plum wine with Japanese food was a guilty pleasure, one of the few alcoholic beverages that I still miss. I could drink beer or wine but almost never enough to get drunk.

I liked drinks made with Kahlua, White Russians and such. Vodka only if it was mixed with something strong enough to cover the scent. Dark rum for Dark and Stormies. Amaretto and Bailey’s to mix with coffee. Mixed drinks went down much easier. I could have a couple of drinks socially but I just didn’t really want to most of the time. The few times I really indulged made me horribly sick. Puking for days, GI pain and headaches for days. I didn’t know then that I was reacting to the alcohol but it made it easier to completely stop drinking.

I am five years older than my sister but the age gap feels larger because we were further apart in school. She was always a drinker. I spent a fair amount of her teenage years tracking her down and picking her up when her friends called me. I would get calls in the middle of the night from her friends who recognized that she needed help but didn’t want to get her in trouble.

If my phone rings after midnight, I get palpitations. I spent so many nights afraid that she would drink herself to death. The fear that her drinking would kill her is still so fresh.

Last year, after several particularly nightmarish months, my sister got sober. She became heavily involved in the recovery community and went to AA meetings everyday. Her sponsor started working through the twelve steps with her almost immediately.

I met her sponsor a few months after my sister got sober. She came to my little apartment on a Wednesday, an hour or so before my nurse would arrive. We talked about the recovery process and ways for family to support alcoholics in recovery. She asked if I drank and I told her no, that I was allergic to alcohol.

“She’s allergic to alcohol, too,” she said, referring to my sister. “Both of your allergies can kill you.”

It is hard for many people to view addiction as a disease and not as a series of elected choices. But there is compelling support for the model of addiction as a neuropsychiatric disease. An imbalance of neurotransmitters, a nervous system that betrays you. One of the neurotransmitters thought to be involved in addiction pathology is histamine.

Mast cell patients are familiar with the psychiatric effects of mast cell reactions. We call it masto rage but it has a real name: mixed organic brain syndrome. This condition was first described in the mid-80’s in a patient who had a psychotic break secondary to systemic mastocytosis. When I react, I get anxious, paranoid and nasty. I perseverate. All of these symptoms have been attributed to histamine release.

Mast cells are involved in a lot of things. I don’t know that I think addiction is a mast cell driven disease but I think it’s an organic illness and not a choice. Our allergies to alcohol are different sides of the same coin. My allergy is treated with benadryl and epinephrine. Hers is treated with twelve steps and searing honesty.

Last Friday, I got to watch as my mother presented my sister with her one year chip at a meeting of her AA home group. My sister has a job she enjoys and just started night school for her MBA. She is happy.

I am allergic to alcohol. So is my sister.