Colorado heartbeat

I am alive. I haven’t been feeling well. The adrenal crisis really kicked my ass and, if I’m honest, scared the shit out of me. It has taken me longer to recover than I anticipated and have had to focus my meager available energy on work. I didn’t realize how long it had been since I posted. Sorry if I scared anyone.

In the midst of these busy and exhausting last few weeks, I decided to run away from my life for a long weekend in Colorado. Colorado is my happy place. It is so beautiful that words don’t seem worthy to describe it. It is quiet and clean. It represents every amazing thing the earth is capable of. I have visited twelve times and I am always just as awed as I was the first time.

Since 2007, a series of people in my life who don’t know each other have moved to Colorado. It started when my best friend at the time moved to Broomfield from New Hampshire. I visited her several times. Then my uncle and his wife moved to Denver and then Golden. In 2014, one of my best friends, Priscilla, discovered that her masto daughter, who had such frequent severe reactions that she literally could not go outside during the day, didn’t have reactions in Colorado. Pris went back to Texas, packed up her family’s life, and moved to Summit County. It has been three years and her daughter no longer has severe reactions.

Priscilla had not spent much time in Colorado before moving there so there are still a lot of places that I have been in Colorado that she hasn’t. Last time I visited, I took her to Garden of the Gods in Colorado Springs. This time, we went to the Stanley Hotel in Estes Park, high in the mountains above Boulder. The Stanley is beautiful and interesting in its own right but it is most famous because the Overlook Hotel in the Shining is based upon the Stanley Hotel.

I am a huge horror movie fan and like the connection to the Shining. But that’s not the reason I go to Estes Park again and again. I go because Estes Park is the edge of my personal universe. I feel very much that if I don’t watch my step there that I could just fall into oblivion. I don’t know what it is about Estes Park that affects me so much. I don’t know if it’s because it is nestled in tightly among the peaks of formidable mountains or the remoteness or the quiet. Something about standing on the porch of the Stanley Hotel and looking down at the sparse twinkling lights below makes me feel tiny against the awesomeness of the universe. The whistling of emptiness is palpable as it runs down these peaks and into the valleys below. It is both lonely and heartbreakingly beautiful at once, both somewhere I enjoying being and somewhere I fear being abandoned.

The last several weeks have been difficult for me. Fall is always hard and the adrenal crisis really exacerbated things. I have almost no energy and am so tired that it takes a huge amount of effort to make my brain work. I find myself needing more sleep and I have always needed a lot. I am having a lot of pain and am so sore that I haven’t done yoga in a couple of weeks. I am limiting my activity and keeping my stress level down. Fall and early winter are always hard for me and I know I will feel better in January. I’m biding my time and listening to my body to give it what it needs in the meantime.

If I’m honest, I was not in great shape for travelling when I went to Colorado. Traveling has been mentally taxing for a while because getting on a plane with luggage full of IV fluids and meds and syringes is not easy. This is different. I am not recovering well from the physical strain on top of feeling generally poorly when I left. Travel is hard on my body and I can’t deny that as time it goes, it is getting harder. Each trip takes longer to recover from. I spend most of my time in bed when I arrive.

I had to stop traveling for years because of my health. Every trip I take now feels like a gift. It is frightening to imagine that the physical strain of travel may eventually become insurmountable. I won’t lose travel. I can’t lose travel.

I don’t know long I will be able to travel. Hopefully I have years left. But just now it feels like my body might not allow that. It feels like I’m walking along the edge and if I fall, I will never get myself back.

Ruthless

I was discharged from the hospital last night. I was admitted on Monday after going to the ER. I wasn’t having anaphylaxis. I wasn’t sure exactly what was wrong. But I felt disgusting. I had a pounding headache and bad bone pain in both legs and my pelvic bones. I was exhausted. I was so tachycardic that it made me short of breath. My blood pressure was all over the place. I was miserable. Nothing I did was helping. So I went in.

I am adrenally insufficient. I have been for years, since daily high dose prednisone in 2013/2014. My body doesn’t make cortisol so I have to take daily steroids to compensate. When my body is under physical stress, I have to take more steroids to cover the additional need for stress hormones. If I don’t, it can cause a life threatening situation called an Addisonian crisis. I had a crisis in May 2014 that lasted several days. Since then, I am very careful to monitor my body for signs of low cortisol.

In case it’s not obvious where I’m going with this, I was having an Addisonian crisis. My cortisol level was almost undetectable. It didn’t feel like it has in the past and I have no idea what triggered it. I took some extra steroids and stayed a while to see how much it helped. I sat in my hospital bed all night, headphones in, doing work. Work is rapidly becoming the only thing in my life I can control.

I take a lot of medications. I take handfuls of pills every day. I use IV meds and IV fluids every day. I get weekly and monthly injections. The schedule I have to keep in order to accommodate taking all those meds is insane. I basically take medication every thirty minutes while I’m awake. I have to carefully time my meals and anything I drink. I can only exercise at specific times. I can only shower 1-2 hours after night time meds. I have to be very careful with things like public transportation and going to a crowded place in case I get stuck somewhere.

I professionally develop diagnostics to determine which patients will benefit best from clinical trial therapies. It is easier to develop diagnostics than to manage my health from day to day.

People often ask how I am able to travel and work full time. IV Benadryl is not the entire reason I can do those things but it is a huge part of it. I have been using IV Benadryl at home for almost four years. It has kept me out of the hospital on many occasions. It has prevented many reactions from turning into anaphylaxis. It gives me much more control in emergency situations and has kept me safe in many situations that could otherwise have been catastrophic.

Despite how much this drug has helped me, I literally get anxiety thinking about how much IV Benadryl has helped me. The reason for this is that it is a nightmare getting ahold of it. It is not expensive and it is not a controlled substance. Still, getting this is a weekly stressor. I get all my IV meds, infusions, and line care/nursing through a home infusion company. Despite the fact that they provide me with a supply of everything else I need several days before I need them, they will not do this with Benadryl. My doctor has asked. I have asked. My nursing team has asked. I have asked all the people who can be asked. The pharmacy will not do it. Instead, they insist that they deliver me meds on the day that I will be out of them, meaning that if that order does not arrive as expected, I could be out of medication.

I have been a patient there for four years. My nursing care and line care has been amazing. That’s why I have stayed there. I have had a continuously used central line for all that time. I have never had an infection. The reason I have never had an infection is because I adhere to really extraordinary guidelines pertaining to contamination. If I think there is a tiny chance that something is contaminated, I just throw it away and start over. If a needle slips, or I drop something, or accidentally touch a surface with the syringe, I throw it away. This means that I throw away a vial of Benadryl every few days. I have asked the pharmacy to provide me with a few extra days of meds every few months to cover for this situation. They won’t.

The worst part of this whole situation is that my meticulousness, which is regularly commended by my providers, means that I am already working with less medication than I should be. Compounded with this pharmacy’s actions, I have been left without medication on several occasions.

Someone new at this pharmacy recently decided that they would send me my meds a day early. This seemed like a great plan. I was totally onboard. But I was still nervous that it would get messed up. It also didn’t allow for short term changes or short notice deliveries. Earlier this week, when I was in the ER before being admitted, I had to use my own meds for more frequent dosing because they weren’t getting my meds on time. (This was acknowledged and approved by the ER team – I would never do this unless they were aware and onboard.)

The bottom line is that my pharmacy delivery is going to be a day late again after being assured again today that it would arrive today. Because I had to discard two vials over this past weekend for possible contamination and I needed to use extra on Monday (as directed), I am now out of this med. Again.

I had a legit breakdown tonight when I realized that I was going to be out of this med again. Screaming, hysterically crying, the whole thing. This has been such a struggle for so long over something that is really, really stupid to argue about. No one argues that I don’t need the med. I don’t take more than I am directed, ever. No one argues that I should discard anything that might be contaminated and start over. But no one in any position to authorize something as silly as giving me three or four extra vials a week will do so. So I will be up all night hoping I don’t end up having anaphylaxis and going to the hospital if I have serious symptoms that could potentially become anaphylaxis even if they didn’t start that way.

I am very, very tired of this life. I have lots of good days. But as time as gone on, the bad days have gotten much worse. There is no aspect of living with chronic, life threatening health problems that is not stress. I really want to just rip this port out and stop taking IV meds and stop working and stop fighting every single day. I just want to rest. I want it to be quiet. I don’t want to have to explain myself over and over and beg people who just don’t care to help me.

 

There is a ruthless truth to chronic illness, one that has taken me years to come to terms with. It is this: that fighting against my illness and the life it gave me is not a successful way to improve it. I cannot overcome this disease. I can only cooperate with it. I have to learn to live with it, have a relationship with it, greet it every morning and say goodnight when I close my eyes at night. It is a part of me that cannot be cut out or ignored. And to have a life with it, a good one, I have to want that life. I can’t fight for a life I don’t want. My insistence upon having a good life with this disease is not a choice. It is a survival mechanism. It an instinct.

I know that this will pass, but there are some days when I don’t want to do this.

I don’t want to fight anymore. I don’t want to be afraid.

And tonight, I don’t want this life.

Crazy

I took a long, hot shower tonight. Hot showers are my guilty pleasure. The heat and the standing in one place can trigger both mastocytosis and POTS symptoms. I can make myself do a lot of unpleasant things. I will not sacrifice this. I cannot make myself take a lukewarm shower. I just deal with the symptoms.

Tonight, I was in the shower, singing along to Operation Ivy, looking down at my bright red, splotchy legs and purple feet. The purple feet in the shower is a very classic symptom of POTS. It is a symptom I have had every single time I have showered for years. But tonight, it bothered me for some reason. I didn’t want any external confirmation that I was sick. It’s hard when you are literally living inside of an organic shell that reminds you at every impasse that it is deeply, fundamentally flawed. There are days when I don’t even want to look at myself.

I was accused of Munchausen’s Disease shortly before I received my diagnosis in 2012. The doctor told me that I needed to see a psychologist to be evaluated for Munchausen’s because there was nothing organically wrong with me. I called her bluff and went to the eval. An hour into the two hour appointment, the psychiatrist stopped making notes and ended the exam. “You don’t have Munchausen’s,” he said. “If you do, you’re not doing it right.” He wrote an exam note that he didn’t know what was wrong with me but that it wasn’t psychological. A few weeks later, I was diagnosed with mast cell disease.

The strangest thing about that situation is that a tiny part of me was hoping that this was all psychological. I hoped that I could receive therapy and feel my feelings and that it would make my health problems go away. It seemed easier than constantly fighting with doctors and getting an endless litany of tests that highlighted problems but never a cause. I was still undiagnosed at that point and I was tired. Very tired. And this little part of me was willing to accept that I was crazy if I could just feel better.

The result of repeatedly being accused of faking or lying or being crazy is that you start to wonder if you are. Even years later, you still feel the impact of those accusations. I was accused of inventing my disease more times than I care to remember. The result is ridiculous: II sometimes wonder if I’m really sick.

It doesn’t matter how much tangible physical evidence I have to prove this fact to myself. It’s like my mind just sort of breaks once in a while and stops accepting that I’m sick. It would be easier for me to accept that I might be crazy than that I will never get better and I will never be healthy again. The idea of forever with this body and these diseases is crushing.

I’m not crazy. This is real. I live under the burden of all the ways my body fails me. I will live with this burden every day for the rest of my life.

I am aching tonight in a way I haven’t in a while. I have bone pain in both legs and pelvic bones. All my long bones are throbbing. I have recently had blistering hives and diffuse bruising again. Last year, those were the first symptoms of vasculitis. In this moment, I don’t think I have vasculitis again, but there’s no way to know except to wait and see what else develops.

The bone pain is keeping me awake. So I’m just sitting here, wishing I were crazy.

Roller coaster

I got my first central line in February 2014. At that point I was at the hospital two or three times a week. Being able to manage my needs at home as much as possible was a welcome relief and so I welcomed the line.

I had a PICC line placed in my left arm about three inches above the elbow. The poor PA who placed it was terrified. She called me the day before to go over the procedure in pinched, staccato notes. She casually mentioned that she was considering doing the placement in the ER since they would have a crash cart nearby. The infusion nurses had regaled her with stories of my reactions and anaphylaxis history. I laughed and then felt bad about it. “Whatever you need to feel comfortable is fine,” I told her. It is the first time I remember a provider being scared of my disease.

The second time I remember a provider being scared of my disease was the following day when a home infusion nurse came to change my dressing. She was much more frightened than the PA had been. She made me hold my epipen while she did the dressing change. “You’re doing fine,” I reassured her, hoping that was true. Comforting trained professionals while they treat me is a special sort of pain, like pulling out a thorn. It shouldn’t have happened in the first place and half the pain is disbelief and feeling ridiculous.

That PICC saw a lot of action. But before I was a mast cell patient, I was an infectious diseases microbiologist, and I wanted that PICC line out. PICC lines are basically a straight shot to infections. I managed to keep my line sterile and my site immaculate and lobbied hard for a port to be placed. In September 2014, the PICC was removed from my arm and a port was placed in my chest.

I wanted a port for a few reasons, but the big reason was because I could manage it completely independently. I expend a lot of energy trying to stay infection free and a big part of that is staying out of the hospital. The PICC line allowed me to give myself meds and fluids but I needed someone else to change the dressing and it was harder to troubleshoot outside of a hospital. With a port, I could access and deaccess the line, change the dressing, and give meds on my own. That meant I only needed to have supplies and myself, and I could bring those just about anywhere. I could also shower more easily, take a real bath, and go swimming with the port. It was an all around win.

In the three years and three weeks that I had it, that port lived quite a life. I took it to my parents’ house and on long walks with my dogs. I took it to work on countless days. I took it to the beach and water parks. I took it to my pool on hot summer evenings. I took it to the hospital for appointments and surgeries and procedures. I took it to my niece’s First Communion. I took it wedding dress shopping with my sister. I took it on many long weekends in New Hampshire. I took it apple picking and trick or treating. I took it Christmas shopping. I took it to my girls’ weekend in Maine. I took it to the Garden of the Gods in Colorado Springs. I took it to California. I took it to Disney World. I took it all over Florida. I took it to the blue waters of Tulum and underground rivers and the Mayan Ruins at Coba. I took it on the Star Ferry and on long walks around Hong Kong. I took it to the Forbidden City and the Great Wall of China.

It wasn’t completely without issues. I had some trouble with my port and a line infection abroad is on my top ten list of things I never want to experience.  But the horrors my mind produced in response to my fear never materialized. I am fortunate to be able to say that my panic attacks have been the scariest things I have experienced with a central line.

My port was my security blanket. It still is. I could travel to all these places because I wasn’t terrified of anaphylaxis. I still took extreme measures to prevent anaphylaxis but I wasn’t paralyzed in fear by the thought of traveling. I had the meds and IV access and could keep myself stable. In an emergency, I could get myself to a decent hospital, and from there, I could get home.

I started having trouble with my port in May. My port had a small reservoir and was difficult to access unless you always put the needle through my skin in the same spot. After years of having a needle continuously penetrating the skin in the exact same spot, scar tissue formed around this spot and the site became indurated. Eventually, the skin at that site became ulcerated and millimeters thin. It was almost to the point that you could see the port through the hole. I developed a literal hole in my chest over my port.

I had a huge amount of anxiety over it. My port allowed me to control so many things about my life and I was really scared about losing that control. I spent a few months trying to get the hole to heal. It did not heal. It ulcerated and got larger.

I had the port replaced at the end of September. They were able to put the new port in the same pocket as the old one with the new line ending in the same blood vessel. They did a fantastic job on the ulcer repair and removed some additional dead tissue. All in all, it went as well possible.

It is staggering to think about how much has changed between the placement of my first central line and my most recent one. It never occurred to me that I would need a permanent central line. I figured I would have it to for a few months and I would get better and it would be removed. It obviously didn’t play out like that.

There have been some very low points. I decompensated a lot. I ended up needing more GI surgery. I transitioned to doing IV meds daily. I needed continuous IV fluids for a while. I’ve had a bunch of procedures, scopes, etc. I lost the ability to eat all solids. I needed several months of medical leave from work.

I’ve had plenty of high points, too. And in the past year, I’ve had a lot of them. Most things considered, I have steadily improved since the spring of this year. I am back to work. I am actively working on MastAttack again. I have been able to adjust my meds a lot as some symptoms have improved. I can eat again, and not just eat, but eat real food. It still takes a lot of work to keep this body functioning but it’s not as much of a constant struggle.

I no longer feel like I’m constantly flirting with anaphylaxis. I haven’t used an epipen in several months. It’s a reality for me that I will never be free of the risk of anaphylaxis. I still keep epipens out in every space in my home. I still carry around four epipens and IV rescue meds at all times. But I’m not waiting for it anymore. I’m not always afraid. That in itself has been unbelievably liberating.

I am working on decreasing my IV meds and IV fluids very, very slowly. My long term goal is to get to a place where I do IV fluids overnight three nights a week and only access as needed for IV rescue meds or procedures. It will likely take years to get to this point, even if I continue to improve. It has been hard because these things have kept me safe for a long time. I think I will never be free of them entirely and that’s okay.

But my relationship with my port is changing. I am very slowly trying to transition to a person who uses my line but who is not completely dependent upon it for safely. Part of this is training my body. Part of this is training my mind to stop catastrophizing and to let go of my anxiety. It’s not obvious yet which part which be the hardest.

I get Xolair every four weeks at my immunologist’s office at the hospital. Last week, his nurse and I were chatting about my recent port placement and decreasing my IV meds. I shared that the idea of not having a continuously accessed port and therefore ready IV access was scary. “Imagine that,” she said as she gave me my injections. “Imagine just accessing a few nights a week. Imagine if you could have it removed!”

I can’t though. I can’t imagine it. I think I will always have a port and will always need some IV support. But the idea that I could only need it to be accessed for three nights a week is mind blowing. A year ago I was passing out if I stood up, my stomach was newly paralyzed, and I couldn’t eat at all. Now I’m back to work full time, working on the courses for MastAttack U, planning international travel for next year and eating chicken pot pies for dinner. Bananas.

As I get more comfortable with this process, the fear is still fresh, but there is now an edge of exhilaration. Like an amazing ride from a high height. A long drop with a safe landing.

A roller coaster.

The dying season

Things die in the fall. It is both my favorite and the worst thing about this time. The scientist in me knows that these little deaths nourish the environment so that one day all of this can be born in me. The mast cell part of me is reactive. We don’t call it Shocktober for nothing. And the human part of me is sad. It’s hard to find the same beauty in stark branches and grey skies and I know that is coming.

We are living through a season in the mast cell community, a different kind of dying season. On a weekly basis for the last two months, I have logged onto social media to discover that yet another mast cell patient has died. There are varying causes of death, including complications of anaphylaxis, organ failure, complications of treatment, and suicide. And it has now been a year since my friend, Ginger, died suddenly.

I have gotten messages from several newer patients asking if this frequency of death is common for mast cell patients. It’s not. They are understandably alarmed at the number of people in our community who have died recently. I am alarmed. Watching your friends die never gets easier. There is no amount of expectation that can blunt the pain. There is no way to prepare.

Spending time in this space feels dangerous. It is not safe to linger here.

I have never stopped being affected by the deaths of mast cell patients I only knew casually. Even if you weren’t close to someone who has died, even if the only link you have is that you both have a common rare disease, you still feel it. You are bonded to the people who understand your suffering. We are part of the same whole.

I have seen a few people express concern that over time, these people will be forgotten. They will not. I remember the name of every single mast cell patient that has died since I joined the community several years ago. I remember the shock I suffered when I learned about each of them. So will all of you.

These people are gone now. There will be no vibrant resurgence in the coming spring. But they were people with lives that touched others and living in the love those people carry is a kind of rebirth. Those people will remember the ways they are changed by having known this person. They will remember favorite things and inside jokes. They will remember goals and aspirations and hopes and faith. They will remember better days.

I want you to remember that the people who are gone can never be completely lost to us. That the things they breathed into the air linger still. That when you breathe, you are breathing them in. This world has been marked by their presence, both physically and emotionally. It has literally been changed in a way that is individual to each person. And because of that, they will never be forgotten. How could they be, when they helped to build this world?

There is a poem about death that has always resonated with me. My instructions for my funeral, hopefully many years from now, include this poem being read.

Do not stand at my grave and weep.
I am not there. I do not sleep.
I am a thousand winds that blow.
I am the diamond glints on snow.
I am the sunlight on ripened grain.
I am the gentle autumn rain.
When you awaken in the morning’s hush,
I am the swift uplifting rush
Of quiet birds in circled flight.
I am the soft stars that shine at night.
Do not stand at my grave and cry;
I am not there. I did not die.
-Mary Elizabeth Frye

Be kind to yourselves. Take care. This season will end.

Past present

My grandmother was murdered when my father was a teenager. He spent the next several years bouncing from home to home, often staying with his friends. One of those friends became like a brother to him. Our families were very close. And when I growing up, my very best friend was that man’s daughter.

She is a year older than me and was a grade ahead in school. We lived within a mile of each other for our whole lives until she was in high school. We were together constantly, easily six or seven days a week. We had almost the same life. It is impossible to overstate how important she was to me. I loved her and wanted to be just like her.

She has struggled with addiction for more than half our lives now. Our paths diverged in high school and never crossed again. I have seen her a few times at family functions. She never looks like herself. I often see things that remind me of her or that she would like and wish I could text her to tell her. We have very different lives now. But I still miss the person she was.

I visited her today in the hospital. I had appointments in the same hospital where she is a patient. Seeing her today was a jarring experience. I wasn’t really sure what to expect. When I opened the door, she turned toward me. It was the first time in twenty years that she looked like herself. I wondered for the first time if we could ever be close again.

I stayed for a while and caught up with her before going to my appointment. My doctor and I compared my symptoms and talked about how generally improved I am. I enjoyed pretty decent health from June 2015-August 2016. “Your symptoms are more like they were after your surgery,” he commented, referring to my recovery post GI surgery in 2015. He’s right. They are.

I often wonder if the reason why I so often reach into my past to compare the present to is because I am expected to literally do this at every appointment. I’m always looking for changes since the last time I was seen. Or since the last time I felt pretty good. Or since I was diagnosed with mast cell disease. Sickness is assessed by the changes it brings about in your body, and you can only do this by dragging the past into the present.

The past can be the precarious face for the present to balance on. We can never go back. Any of us, for anything, for any reason. It is over. But sometimes it feels like I catch the past in my present. In the drudging of old wounds and deeds, pieces of our old selves and our old lives are conveyed to the now. We can fit these pieces in our growing lives. We can remake lost connections.

Our lives will never spin as freely with these old pieces, weighed down with history. But they can still be strong enough to hold you up.

Loud

I am a dramatic person by nature. This is directly at odds with the logical habits and orderly thinking of a scientist. As you might imagine, I am not infrequently conflicted on how to behave or how to react in many situations. I am also bossy. I like to be right. These aren’t good qualities but it’s who I am and I know it.

I’m also loud in just about every way. My hair is fire engine red. I have a violet streak behind my left ear. I wear large colorful glasses. I do unusual and interesting things to my hair and trap it with flower clips or jeweled hairpieces or brightly patterned bobby pins. I dress like a 50’s housewife. I am physically loud, owing largely to my hearing loss and my inability to modulate my own voice level, but I was loud before.

It is a different loudness I’m thinking about tonight. I’m thinking about the loudness of a voice when you scream for help. This is likely the only way in which I am not loud. I used to be. It was never helpful. I am self reliant in large part because I learned early that screaming for help because I was sick or something was wrong with my body was a fruitless endeavor. It never helped and I gained a reputation as being dramatic and attention seeking, a hypochondriac. I learned to swallow those sounds, the ones that signal that I am wounded, that tell the other creatures that I am prey. This unmet need formed the core of my self esteem as a teenager, or lack thereof.

I met up with an old friend this week, a guy I went to high school with. We were friends when I was starting to become aware that something was wrong with my body. I was always having weird health issues and had episodes of inexplicable severe abdominal pain at the most inopportune times. We chatted about my health, then and now.

“So you always had this and just found out about it as an adult?” He asked.

This is more or less what happened. I’m not convinced that everything I had as a teen was related to mast cell disease, but probably a lot of it was. He mentioned that at the time, he wasn’t sure what was going on, if I was sick or just dramatic. This didn’t upset me at all, and was not new information. We have discussed this in the past, some time ago.

“I actually wasn’t sure either,” I admitted. When so many people think you are just inventing these things, it’s hard not to become convinced yourself. Still, I haven’t often said this explicitly. It seems like a betrayal of my deepest self, the one that swallows those sounds, and feels unnecessarily loud.

It has been a long time since those days, when I was 16 and confused about what exactly was happening to me and how much my mental health played a role. But even now it is a reflex to think I am being too direct. I have to remind myself that telling the world what is happening to me is okay and that wanting empathy and help is okay. I’m gaining volume. But I don’t know that I’ll ever be loud.

Somebodies

I’ve been trying to get this post out for a few days. I feel like it’s not finished and sharp in the wrong places but I feel like this needs to be said so I’m saying it now.

When I was first diagnosed with mast cell disease, I was pretty relieved. I had been sick a long time and was so tired of being abused by doctors and called a liar. I could have been diagnosed with anything. I could have been diagnosed with weekly limb falling off disease where every week one of my limbs fell off until I had no limbs left. I needed something to hold onto and a diagnosis had that. (I am grateful to announce that I do not have limb falling off disease.)

I do, however, have mast cell disease. It was a few months before it occurred to me that having mast cell disease might be scary. There wasn’t a lot of information available on it and I didn’t have great journal access then, so I wasn’t able to validate those fears. But I was still afraid. Just a little, at first. And then another several months past and I started having major organ involvement. And I started being afraid for real. This time, my fears were validated.

One of the more common questions I get is whether or not people can die from mast cell disease. I get it a lot from people who are newly diagnosed but I get it from people who have been diagnosed a while. I realized recently that people who have been diagnosed a while only ever ask me this question in private message or email. I’ve been thinking about why that is.

The answer is simple: people are afraid to ask if they can die from mast cell disease in a public forum because, overwhelmingly, the responses are not kind. I am guilty of this, too. Those of us who have been in this community a long time have learned to stratify mast cell patients by level of hematologic malignancy – that is, to separate mast cell patients into those who have malignant forms of mast cell disease (aggressive systemic mastocytosis, mast cell leukemia, and mast cell sarcoma) and those who don’t. Because typically the people who lose their lives to mast cell disease are those with those malignant forms, and those who don’t have them don’t die from mast cell disease. The medical institution views malignant mast cell disease as dangerous and the other forms as not dangerous. Specifically, the establishment touts to everyone who will listen that you don’t die from mast cell disease if it’s not malignant.

But the truth is that’s not really the case, if you think about disease and what it does to a person and all the ways it kills them. It’s true that a person with ASM is not likely to die in the same way as an MCAS patient. A patient with ASM will die from mast cell disease if the thousands of extra mast cells burrow into their organ tissue and destroy that tissue so much that the organ stops working. That’s not what happens in a patient with MCAS. But a patient with MCAS can die in other ways. They can die from anaphylaxis and complications of huge steroid doses and side effects from chemo and sepsis and not being able to afford their health care costs and not having insurance and not being able to face one more minute of the humiliation and desperation that is begging for care from people who don’t want to provide it. All of those things can kill a person, too.

Defining death from mast cell disease along by delineating along the lines of organ failure is disrespectful, unfair and missing the point. All of us who have spent years living inside the data of this disease have done it, including me, and we should be sorry. I am. It has never been my intention to characterize MCAS as less serious than other forms of mast cell disease but I think I did anyway, and whether or not I wanted to do it doesn’t change that. I am sorry for doing this. It is not okay. I am committed to doing better in the future.

Fall is a difficult time for mast cell patients. It’s a lot of change at once. It’s new routines and major environmental upheaval. It’s triggers on crack. Season changes are always hard for us but autumn is harder, I’m not sure exactly why. But in the same way that I associate September with ports, I associate fall with mast cell patients crashing and dying. This year has been no different.

In the last several weeks, we have lost a number of mast cell patients across a variety of diagnoses, to the tune of six in six weeks. It’s painful to even type that. One of them was my friend, an SM patient who died of complications of anaphylaxis. Another was a touchstone in the MCAS community, a young woman who did a great deal to comfort others, and who undoubtedly died of complications of MCAS. Still another died of suicide. These last few weeks have been so, so crushing.

There is a very, VERY good chance that you will live a full life with a normal lifespan as a mast cell patient. But it’s not enough to say that people don’t die from non-malignant forms of mast cell disease because “almost nobody does.” Those “almost nobodies” are somebodies, and they are people with lives and dreams and futures they don’t get to experience. They do not deserve to be lost in the data, digits rounded down to zero.

We owe it to them to remember that they were real and that they were here and that they mattered.

I owe it to them. So let’s do that.

Additional posts on prognosis and disease progression:

Progression of mast cell diseases (Part One)

Progression of mast cell diseases (Part Two)

Progression of mast cell diseases (Part Three)

Progression of mast cell diseases (Part Four)

Progression of mast cell diseases (Part Five)

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 15

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 48

On prognosis and dying from mast cell disease

I am not there. I do not sleep.

 

 

 

Monster

I have a sunburn. Today, I embarked upon that most quintessential New England autumn venture: apple picking. It was supposed to be about 70 degrees with a breeze and some clouds. Instead it was almost 90 degrees with a little breeze and a sun beaming directly down upon us. I always wear sunblock on my face and cover a lot of my skin, even in the summer, so it wasn’t too bad.

The sunburn is on my chest and shoulders. It’s not serious. It won’t blister. It looks red and angry and kind of hurts. Not a lot of pain, but there. Persistent. Stinging.

I have been sick a long time. And that means have been in pain a long time, too. There are lots of different pains and I know them all. I know the loose instability of stretching a joint too far. I know the burning of flushing skin and the stinging that remains after the flush is gone. The abdominal neuropathy that spreads like lightning across my midsection, electric fractals emanating from a central point. The hard swelling of my colon when fluid is trapped in the tissue. The way it feels like broken glass when my GI tract reluctantly moves things through its lower portions. The white hot fire of something passing over an open wound. The acid throbbing of vasculitis. The beating against the back of my skull of a terrible headache. The pressure of food that will not move as my stomach swells around it.

I have needed pain medication of one kind of another for several years. I have lived a very privileged life and I have been very lucky to have a medical team that has been largely the same for several years. My doctors know me pretty well. And because they have seen me screaming in pain and seen the damage that caused the pain, they are willing to help me manage my pain. It is a never ending process of adjusting medications and behaviors and foods but I have options that many people do not. I am lucky. Very lucky.

Drug addiction is a hell I wish on no one and one that has affected me personally in many ways. Heroin abuse is often a complication of opiate abuse: it renders a similar high but is cheaper and easier to get (as I understand it – I do not have personal experience with heroin). Sometimes people who are prescribed opiates for legitimate reasons become addicted and are forced to buy pills illegally or resort to other products, like heroin. Often, people with addiction come to heroin or pills another way, without ever having had a medical reason for using them.

Last year, the CDC made broad recommendations regarding prescriptions for opiates, probably the most well known drug class of pain killers. I had anticipated their recommendations with dread and I was not disappointed. In brief, their recommendations were that every other avenue for pain management be thoroughly exhausted before use of opiates, which would almost certainly render pain relief. The quantities to be prescribed were small and the courses short, irrespective of whether or not this was appropriate to the pain condition or realistic in any way.

Patients would all be drug tested frequently so that we could prove over and over again that we are not drug addicts. Our medication would not be filled without us proving over and over again that we are not drug addicts.

And for those patients like me who have debilitating, chronic pain, there were provisions for trialing removal of pain meds so that they could determine whether or not we actually needed them and if it was possible to reduce the dose at all. Most alarmingly, in my state, they adopted guidelines that could people like me to be evaluated by a doctor who knows nothing about me or my rare disease in order to determine whether or not I really needed these drugs. I am not an addict. I take my medication as directly. I fully comply with any and all guidelines and am happy to pee in a cup every time they instruct me to. But I am still afraid. I am afraid that I will do everything right and still end up in severe pain with no drugs to manage it because government agencies are conflating the epidemic of drug addiction with the necessary pain management of chronic pain patients. And that fear is getting larger and larger as time passes.

This past week, a large pharmacy chain in the US announced that they would only dispense seven days of opiates for “new” patients. There is already a lot of debate about what exactly this means but I guarantee it means more trouble and stress and fear for people like me. I cannot imagine a system in which a pharmacist can know my personal health and pain situation better than my provider of many years. What if the pharmacy decides I’m a new patient and just won’t give me more than seven days? What happens then? There are no answers yet on what will happen, but I don’t need to specifics to know that the people who will be most affected are people like me.

There are many practical reasons why this particular practice is a terrible idea. For one, it requires patients to be seen again to get a new prescription after seven days. For a pain situation in which a patient might need pain meds for a month, this would translate to four office visits instead of one. That’s four copays. That’s four trips with associated expenses. And that’s four copays at the pharmacy. That’s four office visits that already overextended provider’s office now needs to find time for. And when they can’t find the time, those patients may be left without any pain medications until the next appointment.

But these are not the reasons that literally keep me up at night so that I am writing this post at 12:50am despite being absolutely exhausted. What keeps me up is that no matter what, no matter what the intentions are for all these restrictions, and who they are supposed to affect, me and people like me will suffer. We already know what it is like to be treated like a junkie. We already know that we have to convince every provider we interact with that we use pain medication responsibly as directed at appropriate doses for our pain condition. We already know that we’re never totally convinced that those providers believe that.

But most of all, we know pain. And we know that in the current climate of increasing restrictions on pain management, we will have more pain than we used to. Pain that could be treated effectively if there was not such a stigma upon using opiates for chronic pain when everything else has failed. Pain we remember and are afraid of.

Pain is such an abstract quality to those who don’t live with it. People who don’t have it often do not empathize with those of us who do. We are often painted as lazy or attention seeking. Some day, those people will know about pain, and they will feel guilty then. But it will be too late at that point. People like me can’t wait for that day.

Pain is a monster with many faces that haunts every moment of your life. It eats our sore muscles and swollen joints and ruined organs and twisted bones. It eats and eats and eats until there is nothing left that suggests a person lived in this vessel. That there was once a life here, and dreams, and aspirations. That beautiful things lived here before they were destroyed.

Pain is not something you can overcome as a society by pretending the people who have it will be able to figure out another way to manage it when you rob them of their best tools. Chronic pain kills people. We know this fact. It is not disputed. Pain causes inflammation that can cause strokes, heart attacks, cancer. It causes despair and loneliness. It causes suicide.

I am afraid that we will not be remembered as the generation that overcame opiates. I am afraid that we will be remembered as the generation that pain destroyed. If we are remembered at all.

 

 

The really important things require much more than that

I have two best friends named Allison and Alyson who are both very, very into music. With few exceptions, all of my musical influences arrived to my ears via one or both of them. Alyson lives in Seattle. Alli and I went to visit her there in 2014 when I had a PICC line and 40 lbs of steroid weight and a constant flirtation with anaphylaxis.

While we were visiting, the three of us went to Portland, Oregon for the weekend. It was a really amazing and cathartic experience for me. It feels like that was when I began to reclaim myself and my body and my life. It was an experience I will never forget.

Alyson has always been the type of person who follows bands around the country because she is much cooler than I am. In the last few years, she has been obsessed with Kasabian, a British rock band. She flew into Boston for less than 48 hours to see them in Boston two days after seeing them in New York and a few days before following them to Chicago.

I hadn’t really heard much Kasabian before but tonight I went to the Kasabian with Alli, Alyson and Alyson’s mom, Charlene. We were having some hairy health care moments in the line outside, waiting to get in, so I wasn’t really sure how things were going to go. But I decided that if we got into the venue that I was going to be a huge Kasabian fan for the duration of the show. It didn’t matter that I knew virtually nothing about them. I knew I loved the people I was with. I was happy to be well enough to even go to a concert. So I was a Kasabian fan. Sometimes it’s enough to just believe something, even when there’s no good or logical reason for it. Believing is enough.

We stood right up front and jumped up and down and screamed at the band. I really shouldn’t have sang along since I knew literally no words but that has never stopped me before. I jumped and bounced and screamed for two and a half hours. The energy in the crowd was amazing and the show was great.

I said to Alli tonight that I think that concert years are like dog years. I am therefore 132 concert years old. It sure as hell feels like it. I’m going to need a cane to walk tomorrow. No thigh blaster workouts necessary.

The Portland Sisterhood does Kasabian

I have a Wall of Hopeful Things in my home office space, where I do most of my MastAttack work. It is covered with things patients or parents have sent me. There is a trinket dish on my Wall of Hopeful Things that says, “It is not enough to put your heart and soul into something, the really important things require much more than that.” I think of this as the MastAttack motto. You can’t build something without leaving pieces of yourself among the blocks.

In the last few weeks, I have painstakingly mapped out the next year of my personal, professional (work) and professional (MastAttack) worlds. I have monster goals for MastAttack for the next year. I have started putting together the materials for the course videos for the spring. I have been networking a lot to work towards some other MastAttack goals. I have professional development and program goals at work. I have lots of personal things that I have forced myself to find time for. It is an intimidating amount of life I am trying to fit into these time constraints. But I decided that I could do it, and as soon as I did, I became capable of it. You have to believe it and hold your nerve. So here goes nothing.

Hope you all are having a super weekend. Be on the lookout for some MastAttack announcements this week.

It is not enough to put your heart and soul into something. The really important things require much more than that.