Eight

I went to the New England Aquarium yesterday afternoon with my two nieces, Miranda and Amelia. Miranda is 13. Amelia will be 9 next month. On our way to the Aquarium, Amelia asked who the people coming to Boston to protest were. I told her that some of the people who announced they were coming were verifiably white supremacists. I gave her examples of what certain groups believed about other people in society. I told her that many more people believe that you should treat everyone the same regardless of race, religion, gender or sexual orientation.

I stopped short at the end of that sentence in a way that surprised me. My body literally would not push out the next few words. What I was about to say was that you should treat everyone the same whether or not they have disabilities and differences. But Amelia already knows that. She was sitting next to her sister, and her sister has physical and intellectual disabilities.

In the US, disabled persons are considered a protected class. This basically means that it’s harder to discriminate against someone based upon their disabilities. In reality, it’s very hard to enforce. It can be very difficult to prove that you were discriminated against directly because of your disabilities.

Disabled Americans have won important battles in the last few decades. We saw the passage of the Americans with Disabilities Act. We have access to Family Medical Leave Act if your employer meets certain requirements. We have legal rights to some accommodations at school or work. Our situation has improved without a doubt.

But disabled people are still trying to navigate a society that views them largely as a nuisance at best and a freeloader at worst. There is still open scorn for people who aren’t able bodied. It is politically incorrect maybe, but only just. You are constantly accused of wasting society’s resources. You are irritating. Annoying. If you don’t tell people about your illness, you’re hiding things. If you tell people about your illness, you’re always talking about your disease. If you post about your disease, you are looking for attention. If you don’t post about it, people message you privately for savory details. You can’t win. You seek validation and acceptance with every interaction and you seldom find it.

I couldn’t get the words out yesterday because Amelia is going to know soon anyway. The days when she is not regularly confronted by the marginalization of disabled people are rapidly coming to an end. But she has still has some days and I couldn’t take them from her.

If you live in the world, you may have heard that there was a political rally slated to happen in Boston today. Some high profile racist groups had announced their intentions to attend. But so did tens of thousands of Bostonians. I wanted to go so badly. But I can’t. I can’t walk into a charged situation where I could be robbed of my immediate access to lifesaving medication or emergency care. I can’t risk getting maced or hit with tear gas.

Because I can’t, people often feel that I don’t care enough about standing up for my beliefs and values. And they often feel like it’s okay to say that, too. Because there aren’t really any consequences except my hurt feelings. Society just expects you to fit into this role and if you can’t fulfill those expectations, you are difficult or whiny or weak.

Today was a beautiful day in Boston. I spent it with my mom, my sisters, my niece and Kristin’s mother in law to be, Ellen. Strong, intelligent, hardworking women all of them. I thought about a day in the future when Miranda might help Amelia into a wedding dress and when Amelia would be a champion for her sister.

Those days are coming. But today, she gets to be eight.

 

Waves

I used to think a lot about death. I imagine I still do think about it more than most people. It is something that both comforts and terrifies me. On the hardest days, it seems like a gift. On those days, I just want to lay back and close my eyes and sigh my last breath and be transported to oblivion. A reprieve. Nothing. But on every other day, I just want to get survive until tomorrow.

I sometimes find myself passing these thoughts over each other until they have lost any sharpness and danger. What if this kills me? And hiding just under my breath, in that space where my mind hides its deepest unspoken mysteries: what if it doesn’t? What if I am never saved from this?

The fall of 2013 was a big turning point in my health. It was the first time that I was so sick that I thought I would die. Not when I got my colostomy. Not when I was having severe mast cell attacks and anaphylaxis that debilitated me for weeks before I was diagnosed. Not when I had a GI bleed that lasted for months. In the fall of 2013, for the very first time, my pain was my worst symptom, worse than the exhaustion. And when I was in bed and in so much bone pain that it hurt to stand, so much that I couldn’t go to work, I thought that this must be what it feels like to be dying.

2014 was mostly a blur punctuated with hospital stays and epipens. So, so sick. Vomiting blood and bowel obstructions and anaphylaxis. I got a PICC line and then a port. I started using IV fluids and meds. I drafted my first will and advanced directive in 2014 at the ripe age of 30. I organized my whole life around my disease. I literally wrote letters to friends and family in case I died. It was bad.

But then something happened that I did not expect: I got better. I was still pretty sick but I no longer felt like I was constantly flirting with death. I was able to travel a little with heavy support from my friends. In 2015, I had GI surgery and worked very hard on reconditioning myself. And I got even better. Like, a lot better. That lasted for over a year until last fall. And then this past spring, I recovered. I would say I am in probably the best health state than I have been since 2013. These things come in waves for me. Feeling better doesn’t always last but feeling like I’m dying doesn’t always last either.

Today, I was able to meet a mast cell friend who was in town for an appointment. Her illness and diagnosis are much more recent. Like most mast cell patients shortly after diagnosis, she is still in a really difficult spot. I told her about my own ups and downs with mast cell disease. I told her that she wouldn’t always feel this way. And I’m sure she won’t.

I wrote this post to say something that I don’t think gets said often enough in this community: That it won’t always be like this. You won’t always be like you are right now. There will be improvements and there will be setbacks. But whatever reality you are living right now will assuredly be replaced by something new. And this means that every day, you have a reason to be hopeful. Just get through the day. If you can get through it, tomorrow could be the day that you could get better.

Don’t give up. Stand up and fight.

It gets better.

Stolen summers

I didn’t always hate the summer. When you’re a kid, summer represents freedom and sleeping late and vacations and swimming. You wait for the heat to come, for the sun to stay high in the sky longer, for the stickiness of sweat from playing and the grime of dirt stuck to your skin. You wait for the clanging of the final school bell and the shouts of students as they rush out of school and into the summer. You wait for it and wait for it and when it arrives, you celebrate it.

I didn’t hate the summer until I was an adult. I was afraid of fish so ocean swimming was fraught with danger. We didn’t have a pool. We camped a lot when I was growing up but stopped doing that when I was a teenager. I worked in school age childcare summer camps so it was exhausting, the only kind of exhaustion I have encountered that has ever rivaled the day to day tired of mast cell disease. I hated sand. I hated sunblock. And that was even before summer started making me sick.

Heat is probably my absolute worst trigger. My body does not do heat well. I turn bright red almost instantaneously. I feel faint. I get nauseous. Any sunburn blisters. I have scars from them. My diagnosis with mast cell disease legitimized my aversion to summer. I could hide in the darkness of my apartment in peace. There were few things that could convince me to go out in the summer heat.

The women in my family go away for a girls’ weekend in Ogunquit every summer with some of our close friends. I was in pretty rough shape the first time we went. I was super sick all the time and so unhappy with the 30 lbs steroids had tacked onto my body. I had a PICC line and couldn’t go swimming so I just hid inside with the air conditioning until evening arrived.

The following year was different. I had had GI surgery a couple of months before and had started a reconditioning program that was working for me. I had lost a lot of steroid weight and was much less reactive. I also had a port by then so I could go swimming. The Atlantic coastal waters in Maine are so cold that it made my arms and legs numb as a kid. But it was so hot that weekend that the water just felt refreshing. I stayed in the ocean for hours. For the first time in a decade, I remembered why I had once liked the beach.

I have been more stable since this past spring. This summer has been very different. I have been more able to travel. I have been to Mexico, Ogunquit and Florida in the past ten weeks. Summer parties and cookouts are less complicated because I can eat many common foods again. I can have ice cream at night. I can take long walks to the beach with Astoria. I can do fun summer things with my nieces and nephew and friends. I don’t always feel wonderful and sunlight and heat are still tiring but this is a wholly different experience. I am not afraid to leave my house. I am not afraid to eat.

We are now just a few weeks from the end of the summer. I love fall. It has always been my season. I love Halloween and spooky things and scary movies. I love the cooler weather. Every August feels like an obstacle to getting to autumn.

It doesn’t feel like that this year. It feels like something amazing is slowly winding down. I’m not ready for the wind to be cold again. I’m not ready for a dark sky watching me when I walk after dinner. For the first time in my adult life, I don’t want summer to end.

I wonder how many summers I could have loved if I hadn’t been so sick. Would I have loved the sun? The beach? The heat? Was this stolen from me, like so many other things?

I am trying to commit every moment of this summer to memory. Because it will be gone soon. And I will miss it.

The West Coast Florida Tour and an update on Kristina Brightbill

I realize how lucky I am to be able to travel at all but that doesn’t mean it is easy. Travel always takes a lot out of me. I start premedicating days in advance. I count and organize meds and supplies to pack and repack. I try to prepare myself for the emotional and logistical stress of air travel with luggage full of IV bags, line supplies, and dozens of bottles of pills and vials of IM and IV meds.Even when everything goes smoothly, I need a ton of meds, sleep, fluids, and easily digestible food to recover and get closer to my baseline.

The fact that I travel as often as I do is largely a testament to my friends. I am lucky to have such incredible people in my world that all the strain and stress of traveling to visit them is worth it. Being with people like that has a way of pushing away all the stressful things about my life. I also come home feeling peaceful and more like myself.

I met my friend Pat a few years ago when she came to Boston for MCAS treatment. She lives in Hong Kong for most of the year and was my coadventurer on my trip to the mainland China and the Great Wall. She prosecuted murder trials in Toronto for years and has an endless reservoir of fascinating stories. Her husband is wonderful and the most organized prepared person I have ever met. Their daughter is currently in university and is funny and bright. She actually did some behind the scenes organization of the blog this past spring. This family is very special to me.

On Thursday, they kindly drove me two hours away to see my friend, Kristina, and her family in Sarasota. I met Kristina a few years ago when her son had no safe foods and was reliant upon her breast milk produced by Kristina while she was surviving on a two food diet.

In October 2015, Kristina had a catastrophic stroke in her brain stem. I have written about this in great detail and am not going to rehash it but you can read about her here, here, and here.

Kristina has locked in syndrome as a result of her stroke. Her mind is completely intact but she was completely paralyzed and unable to speak. People ask about her a lot and Kristina said it was okay to give everyone an update.

The scariest part of Kristina’s stroke was the amount of things her family was told she would never do. They said she would never be able to communicate. They said she would never breathe without a ventilator. They said she would never eat. They said she would never regain any movement. They said she would never recover any of the function she lost.

Kristina started recovering some motion in her head, neck, and face when she went home in June 2016. She could communicate by spelling words by blinking when someone recited the alphabet to her. It was a very slow process and very taxing for Kristina and her family.

Kristina worked in physical therapy before the stroke. When she went home, she had an amazing support system of friends who were physical therapists, occupational therapists, and speech/swallow specialists. These incredible people donated their time to give her many hours of therapy not covered by her insurance. These people, along with Kristina and her relentless family, have helped her to regain an astounding amount of function.

Kristina can move her arms and legs, although some of the movements are very small. She can control some fingers and her left thumb. She has much better facials and eye control. She is able to use a Tobii system to communicate now. She types out the words and the system says it aloud. It also allows her to text, make phone calls, and use social media. This incredible technology has given her a voice after almost a year and a half of not having one.

Kristina is getting stronger by the day. While I was there, she did edge of bed exercises where she would balance herself with only support at the very bottom of her back. Her core is now strong enough to support her upright. She can turn her head while sitting up also. She sat up for about twenty minutes without needing a break!

Kristina had a tracheostomy to help her breathe since the stroke. She now breathes normally and medication changes have allowed her to have the trach removed. She also recently had a picc line removed because she no longer needed it. She can chew and swallow and eats a fair amount of purees. She still gets a lot of nutrition through feeds in her G tube but is working towards eating mostly by mouth.

Her overall health profile is hugely improved. She is pretty stable these days. Her stamina is much better. She is able to go out for appointments and errands with a specially equipped wheelchair van. She recently visited an organic farm to discuss growing safe foods for her son who has MCAS.

Last year, I visited Kristina on August 10. She was still Kristina but she was in a bad place emotionally. I firmly believed that if she could get to a better place with her communication that she would regain a huge amount of emotional health.

I visited Kristina this year on August 10 and am thrilled to report that she is in a much happier place. She made the decision several months ago to move in with her parents to make it easier to ensure that she was always getting the very best care.

Her son is with her five days a week and he loves his Mama. She talks to him with her Tobii software and he loves it. He pushes her wheelchair around and likes to eat and watch tv sitting in her lap or in between her feet on the footrest of her wheelchair. His MCAS is also improved although there have recently been some problems with his diet. I am confident that with some effort that we can get him back to a good place.

I stayed for a while and Kristina and I gossiped and shared some funny stories. It was lovely and such a treat to see her feeling much more like herself. Her mind is so clear and her energy is so good and against all odds, her body is continuing to recover. There is literally no medical precedent for this. Kristina is the only person with locked in syndrome known to have regained function after almost a year of no gains and she pretty much did by sheer force of will.

When someone’s body does things I don’t understand, I assume that there is a scientific explanation and I just don’t know what it is. But I have never felt that way about Kristina. Her astounding recovery feels supernatural. It feels like an actual miracle.

I was sad to leave Kristina but was excited that she felt up to meeting my friends who have heard so much about her. She met Pat and her daughter when they dropped me off. Later, she met my friend, Nicole, when she picked me up that night.

I was sad to leave Kristina but was excited that she felt up to meeting my friends who have heard so much about her. She met Pat and her daughter when they dropped me off. Later, she met my friend, Nicole, when she picked me up that night.

Nicole and I met years ago in a mast cell FB group. She was the first person I knew other than myself who also had a port that she accessed and used herself to administer routine and rescue meds. She is half my adventuring partner and half my adopted little sister. I’m currently at Nicole’s horse farm outside of Ocala. This place is so beautiful that I can almost feel it nourishing my soul.

Tonight, I went to a dinner party her parents were hosting and met a bunch of great people. A couple attending did not speak English and I was glad for the opportunity to connect with them in Spanish. It was just an all around pleasant and fun night.

I’m going home tomorrow night. I never feel like I have spent enough time with my friends but it is good motivation to come back soon. I expect I will be back next summer. In the meantime, I am so very grateful to be living this life with these amazing people, and for the refreshed mind and spirit they give me.

Many thanks to all the people who keep Kristina in their thoughts and prayers, it means a lot to her. Kristina’s story is so powerful and a lot of people have connected with it, including lots of people who don’t really know her. A community of caring people can be so encouraging and uplifting.

Alright, time to wrap this up. It’s late and I have an appointment in a morning to ride a horse named Porkchop.

August 10 is Kristina Brightbill day!

What we are

I’m on vacation right now. I flew to Florida last Friday. I have been staying with my friend, Pat, in Naples since Friday. On Thursday, she will bring me to visit my friend, Kristina, who lives a couple of hours away. Thursday night, my friend, Nicole, will pick me up from Kristina’s and bring me back to her horse farm to stay for a few days. I met them all online in a time when my life was a constant struggle to live with mastocytosis. I have since had adventures with each of them. All of us have mast cell disease.

I have recently regained a lot more control over my disease and my life. I started Xolair in March. Two days after receiving a Xolair injection into each arm, I could eat solid food again. I have steadily acquired more foods, including things I thought I would never be able to eat again. Cherry pie, my all time favorite food, and one of the first things I lost. Girl Scout cookies. Tacos. I am relearning not just what I can eat, but how to eat. I am re establishing a relationship with food. I am finding a new path in which food is not a dangerous necessity.

I have difficulty moderating myself with foods I have regained. My stomach is still tiny. My GI motility is still garbage. My stomach is still largely paralyzed. I have to remember that platefuls of food will still sit in my stomach for hours, whether or not I have a mast cell reaction. I can still make myself by eating too much too fast.

It is the same with activity. I can be outside in the heat a lot longer. I can sit in the sunlight. I can push myself physically without it ending in disaster. If I go too far, I pay for it. I still need to sleep. I still need to adhere to a rigid med schedule. I still need to manage my stress level vigorously. But my body will bend now where it would previously have broken.

Today, while my friend and her husband were out at an appointment, I took some meds, put down a yoga mat, and started a documentary on my iPad. I found a vinyasa yoga sequence I wanted to do. It was 92 degrees out and very humid, the sun blazing overhead. I stopped every five minutes or so to drink some water, wipe myself down, and rest. I didn’t mind going slowly and stopping when my body needed it.

The heat started to overtake me. I sat down and assessed my body to see if I could continue. I just wanted to see if I could do it, because I genuinely thought that I could. This was not a stubborn line in the sand. I believed I could do it safely. But I did not want to push myself too far. And I was very hot.

As I was coming to terms with needing to end my practice early, it started raining. There’s rain and then there’s southern Florida summertime rain. The kind of rain that falls so heavily that you almost can’t see it. I walked out from under the roof of the lanai and into the falling torrents. I closed my eyes and and let the water overtake me.

Water is a purveyor of emotion and memory. I was transported to a million other moments when my body was strong. When I walked my first Breast Cancer 3-day through similar heat in the first week of August 2007. When I climbed mountains in Norway. When I camped underneath the Golden Gate Bridge and walked across it on a misty San Francisco morning. This strength has always been there, even if it has been buried my disease.

The nature of this disease is that there is no real nature. It changes constantly. You can never really adapt because you can’t even comprehend what changed. You just learn to control the spin amidst an unpredictable world. Sometimes not even that.

This is the first time in a long time that my recent stability has not given me anxiety. In the past, it has been hard for me to be present. I worried a lot about how long this reprieve would last. It was excruciating to think that I could accept this good fortune only to have it torn away without warning. I felt so exposed. Vulnerable. I didn’t want to risk another heartbreak.

The last few years have been painful on every level. Even so, it is silly to think the adage in the fable of my life could be that bad things happen anymore than it could be that good things happen. They are two sides of the same coin. These two faces are matched. You cannot have gain without loss. Getting knocked down is no more important to my story than is the getting back up.

I am also surrounded every day by other people who have triumphed against this disease. Pat has made some strides this year in identifying important pieces of her health puzzle. Nicole was recently admitted for a serious line infection but she is home now and in one piece. Almost two years after a catastrophic stroke that left her trapped inside her body, Kristina has just started working on standing. This disease has threatened to drown us but we surfaced anyway.

As the rain washed over me today, I remembered that strength is not something we have. It is something we are. And just like that, I wasn’t hot anymore.

 

Catharsis

Hello, MastAttackers and Other Good People of the Internet,

A warning that I am about to be super sappy and emotional.

As MastAttack has grown into a sort of rare disease cultural touchstone, my life has become progressively more complex and more stressful. My role as a community resource affects every part of my life, and not always in a good way. In particular, the past year has been difficult for me, for a lot of reasons. There are days when I wake up and want to blow it all up and dye my hair brown and return to a life of anonymity.

But I never do and that’s exclusively because of you guys. It is my privilege to belong to this MastAttack community with all of you. On the hardest days, you really keep me going. You believe in me and that is so, so powerful.

Revealing the plan for MastAttack U to all of you has been cathartic. I am not a fan of secrets and keeping this a secret for so long really disconnected me from the community. On a more selfish level, I was also worried that you guys wouldn’t like it. The positive response to the announcement for next year’s courses has honestly been humbling and overwhelming. It has been my dream for a long time. I don’t have words to describe the feeling I get when I think about being able to teach this course to all of you.

MastAttack may be my idea and my project but it doesn’t really belong to me. It belongs to all of us in this community. It is not something that I am doing. It is something that we are doing. Together, we have an opportunity to develop hundreds of capable advocates for mast cell disease. We could change the way mast cell disease is treated and managed. We could directly impact our own care and the care of other patients by understanding our disease and how to teach others about it. The next generation of mast cell patients could be born into a world where there are hundreds of patients who have educated hundreds of physicians.

In order to achieve this, I have to believe that this will work, and I do. I believe in all of you the way that you believe in me. There will always be hard days and we will learn as we go along what works and what doesn’t. But we are a team. We can do this. I know we can.

So thanks for believing in me and in MastAttack and for being my people. It is pretty much the only thing holding me together sometimes. Sincerely.

I am leaving tomorrow evening to visit with some friends and take a much needed vacation through August 14. After 5pm tomorrow, I may pop in and out a bit online but will mostly be unavailable. I have set up some auto posts on the blog to continue the MastAttack 107.

Any and all questions/patient/meeting requests/masto related communication will be returned after August 14. In the event of an emergency, please contact one of the MastAttack admins for the Facebook group. They can get in touch with me.

Thanks for everything. Hope you guys all have a super week!

Xoxo,
Lisa

In which I share the MastAttack end game and I hope you guys like it

Dear MastAttackers,

I have some news to share. I hadn’t planned to announce it until September but it has been a rough few days as a community and I thought sharing something positive might be helpful.

A couple of years ago, I made a list of the goals I wanted to achieve through MastAttack. The most ambitious of those goals was to teach five other people everything I know about mast cell disease. The idea was that I would mentor and teach these people with the understanding that they would become responsible for teaching a certain number of people everything they had learned from me. In this way, my knowledge would be redundant and information much more readily accessible for patients and providers. The point was to nurture and protect an open flow of knowledge that increases as more people learn about these diseases in depth.

So I had this idea and I spent several months turning it over and over in my mind. I knew it would take up a lot of my time and mental capacity. To be worth the huge time investment, it had to be effective. I had to figure out exactly what I knew that and how to teach that to other people, especially people who do not have a strong science background. I needed people to already have some basic understanding of mast cell disease in order to be able to teach the more technical points. I also had to figure out how to do it without it stressing me out and jeopardizing my health.

Along those lines, I have been working on a secret project for over a year. I organized all the information that I felt a person had to understand to learn everything I knew. I divided it into sections and developed a syllabus for three university style courses on mast cell disease and mast cell biology.

The first of these courses will start in the spring of 2018. It will teach intermediate topics beyond the basics of diagnosis/treatment/activation biology. It will run for 8 weeks. I will post a 20-25 minute video lecture on YouTube on a specified topic. The following week, we will meet online to discuss specific questions or issues associated with the topic. These discussions will happen in the MastAttack Facebook group, to which I will be returning later this year. I will also provide some handout style materials. I’m sure the first run of this course will have some growing pains and we can see what works and what doesn’t for subsequent teaching activities.

I also wrote an introductory text specifically to give a knowledge base for students to build upon in this course. You are all currently reading that introductory text. It is the MastAttack 107 series.

The most amazing part of MastAttack is seeing people who knew nothing about mast cell disease grow in their knowledge of these complicated concepts. For people who have a very solid understanding of mast cell disease and want to learn much more detailed, technical concepts, I plan to offer a Master class. I do not yet know the format this will take. I will let you all know when I’ve developed the idea further.

I strongly believe that people should not have to pay for access to information that allows them to understand their health. For this reason, this course will be free. Lectures and materials will be publicly available. Given how much time and effort it has taken to put this all together, I am open to the idea of compensation if people want to and are able to. However, this will not affect your access to this course or the information taught in it, or your personal standing with me. No one will have to pay. I mean it.

The last few days have been hard for a lot of people. The reason for this is largely because we rely on specific people to educate others on our behalves. The solution to this specific issue is to learn how to educate others ourselves. This will never replace the need for the support of well informed providers and researchers, but it will help people to regain some agency in their own care.

The best possible situation for our community is a legion of well informed patients that can advocate for themselves and help others. The best possible scenario is for me to be obsolete. This is the MastAttack end game, and has been from the beginning.

So now you all know my big secret. I’m really excited to do this. I hope you guys are, too.

Never give up hope. Stand up and fight.

Xoxo,
Lisa Klimas
Author and founder of MastAttack

Non existent

One of the more arcane pieces of Lisa Klimas trivia is that I lost the majority of my hearing in 2009. I grew up hearing to hearing parents and am culturally hearing. I knew some sign language but it was pretty minimal. While I was losing my hearing, I focused all my available energy on learning ASL. I practiced for hours every day. I fingerspelled all day long and sang along to songs in ASL when I was driving or in the shower. My boyfriend at the time learned to sign as well and we signed at home.

After eight years of being Deaf, I straddle the worlds of the hearing and Deaf cultures. ASL has become a part of me. I no longer have to consciously translate from English. I can think in ASL. Sometimes the words that my brain generates in response to something are signs instead of English words. When this happens, it is often a word or concept that has no direct translation in English. Some ideas are just more natively ASL.

My post yesterday was written in response to a recent video that really downplays mast cell activation syndrome. I felt like the wind had been knocked out of me when I watched it. I had an immediate visceral reaction. My inner voice was signing to me. A flat palm brought up to the mouth and blowing gently across it. There are a lot of translations for this sign, none of which perfectly capture the meaning. It is close to nothing left or non-existent. Like you had something in your hand but now it is gone.

I thought a lot today about why exactly this interview upset me so much and what my motivations were for speaking about it publicly. I think it’s because as a community we are so small and hidden. We really can’t afford bad press or harmful misinformation. Most people will never know about mast cell disease. Many will never even hear those words at all. Our stories will never reach everyone.

But we try anyway. We try even when we know that our words will be swallowed by the cacophony of billions of voices talking over us. We shout as loud as we can that this is our story. That we exist. That we are falling through the holes in a health care system that isn’t prepared for us. That this is real and that we are scared and that we need help.

We are a tiny community, a fraction of a fraction of a percent of the people living in this world. A fraction of a fraction whose faces and voices and stories are at great risk every day of never been known. A fraction of a fraction that lives with the knowledge that at any time, this disease could take everything. That any story they tell might be their last. That they might leave this world and become a story.

That there will be nothing left of them. That they will cease to exist.

 

Raising hell

I started this blog in 2014 with the expectation that only a few people would read it. There were some educational posts but they were pretty cursory. They were very directed towards patients without a lot of science background. I sometimes included references. I didn’t fret over the wording or the way I presented facts because controversy seemed unlikely to strike. I was basically talking to myself in those early months.

Everyone who reads MastAttack knows where this ends up: that a lot of people started reading it. People came to me in droves for answers to their questions. Caregivers came for advice. Health care providers and researchers began to view me as an opinion leader and MastAttack as a reliable source of information about mast cell disease. I started working with patients and medical teams to advocate for appropriate management, diagnosis and treatment. I wrote and rewrote posts and read and reread literature and data about mast cell disease to guarantee its accuracy. I became an authority on mast cell disease and sort of authority figure in the mast cell community.

I spend a massive amount of time researching mast cell disease. I write and rewrite posts constantly. I read and reread literature and data. The pressure to be right is huge, and it should be. MastAttack is a readily available point of contact for any person trying to learn about mast cell biology or mast cell disease. Failing to accurately vet something could affect people’s lives.

Ultimately, I have to be right, every time, because when I speak, I am speaking for every mast cell patient. I take that responsibility very, very seriously. It is more than just understanding the science. I have to know the story I am trying to tell about these diseases that can direct patients and providers toward effective care. I have to convey the human impact of these diseases. As a representative of the mast cell community, I have to be on message.

I have philosophical and academic differences with mast cell researchers, providers, and patients, especially about some of the more nuanced science. Despite this fact, overwhelmingly, this community is united in telling one story with one message.

Mast cell disease is cruel and debilitating. It steals things from you. It steals your life. It is scary and graphic and gross and endless. It is handfuls of pills and organ damage and surgeries and central lines. It is so, so serious. It is epipens and swelling and wheezing and puke. It is danger. It is the exhaustion of arguing with insurance companies for hours at a time. It is the metal taste of fear when we wonder if our kids will get it. It is the quiet terror that this disease will get worse. It is the unutterable thought that maybe one day it will kill us.

It is also the strength of a patient’s bonds with their family. It is the discovery that you can keep going long after you are certain that you can’t. It is iron will. It is the exhilaration of achievement in spite of the damage your body sustains on a daily basis. It is love and joy and tears and uncertainty. Because this is real life and these are real people and real diseases. That is the message.

Misrepresenting that message in private or in public does more than a disservice to mast cell patients. It can be harmful. It can be dangerous. And since rare diseases are by their very nature scarce, media around them is also limited. A person without these diseases might only ever read one article about mast cell disease or see one interview. We do not have infinite opportunities to educate people about these diseases. We are not living in forever. When a person speaks to the public on behalf of the mast cell community, what they say MUST be correct. It MUST tell the real story. It MUST represent the experience of living with this disease accurately. It MUST be on message.

For patients who are wondering why I’m waxing philosophical at 3am about the onus upon subject matter experts in this community, there was a very recent televised interview about mast cell activation syndrome (MCAS). I’m sincerely trying not to be unkind but I was genuinely bewildered by the amount of inaccuracy jammed into a four minute video. I’m not going to link to it because frankly, I don’t want to generate additional traffic to the video. It was insulting and irresponsible at best.

Mast cell activation syndrome (MCAS) is a serious, chronic health condition that affects all organ systems. It increases the risk of anaphylaxis, which can be fatal. Complications of MCAS can include organ damage. It is often disabling. It is often found alongside other conditions such as Ehlers Danlos Syndrome and POTS. It is not well known among medical providers. It is difficult to test for. Treatment revolves around blocking mast cell mediators with things like antihistamines and mast cell stabilizers. Most people are not able to manage their diseases simply by avoiding triggers. Most people need medication, and often, a lot of it. Most people have symptoms every day and sometimes experience bad mast cell attacks and anaphylaxis. MCAS is in no way a mild or minor health issue. It should not be minimized and neither should the experiences of the people living with it.

In the same way that experts are responsible for sharing accurate information, patients also have a duty. This is your community. You have a voice and a duty to use it. If you are not being represented accurately, raise hell. If your disease is being minimized, raise hell. If the science is not being explained correctly, raise hell. When misinformation threatens your safety, You. Raise. Hell. Yes, you.

Use your voice. Tell your story.

Stand up and fight. And raise hell.

 

For people looking for reliable information to provide to lay people or providers about mast cell disease, please visit the following posts:

The Provider Primers Series
The MastAttack 107
The Mast Cell Disease Fact Sheet

Extraordinary

I forget sometimes that this life is extraordinary. Being sick just becomes incorporated into your life. It is impossible to survive if you are upset about it every day. It just becomes part of your routine and you learn to live with it.

I had a new IV line placed last week. My port has been accessed continuously in the same spot for three years. My skin is indurated and paper thin over the access site. I accidentally tore the needle out last month and that further irritated the skin. Since I was likely weeks away from being able to literally see the port through the hole in my chest, we opted to place a temporary IV line for me to use so I could deaccess the port to heal the skin. They put in a midline last week and deaccessed my port.

I had a PICC line for a while before I had my port. The PA who placed it was pretty terrified of my mast cells. She had been warned by the infusion nurses at the hospital. The placement itself was uneventful but I will never forget having to reassure her. It was the first time I saw a provider scared of my disease. The following day, a home IV nurse came to change the dressing and check the site. She was also scared. She asked me to hold my epipens while she changed it in case of anaphylaxis. I reassured her, too.

While I am grateful to have IV access because it keeps me out of the hospital, I had forgotten what a royal pain the ass it is to have a line in your arm. The port is easier is so many ways. I can access it and deaccess it at will. I can change the dressing myself. I can get it wet. I don’t have to deal with my pump constantly squawking that the line is occluded because I bent my arm. Blood doesn’t back up in the port line. I don’t have to constantly lock the line with heparin. I forgot the way IV Benadryl burns when it’s pushed into a smaller blood vessel. The midline is temporary but obnoxious after years of having a port.

Having the midline has brought back a lot of memories for me from around the time I got the PICC placed. One of the strategies social workers recommend for adapting to a medical device or deformity or disease is to give it a name. I named my PICC because I had to convince myself that I could learn to live with it. I named my ostomy, too. I don’t bother naming things anymore. Because it has become routine.

Sunday night, I ended up in the ER after sudden onset severe GI pain. This pain is high in the tract and much more severe than what I have experienced before, both in intensity and in duration. I went to the hospital because the pain was so bad that I honestly thought I had ruptured something. It was the kind of pain that makes you think you are dying. I was literally screaming in pain.

I spent the next day in the hospital where my screaming pain was interrupted only by intense vomiting from the pain meds. We have no idea what is causing the pain. I am not convinced that it is mast cell related. I came home last night because the hospital couldn’t do anything for me that I couldn’t do at home. The nausea and pain were still there. So I left with no answers and a lot of pain.

One of my nurses yesterday was really horrified when I told him all the things I do on a daily basis to manage my disease. He in particular was horrified that I needed so much medication and was still left with debilitating symptoms. It is only in seeing this awe reflected in the eyes of people who see so much suffering that I remember how sick I am.

Today was the longest day of the year. In many pagan traditions, the summer solstice is the day when the land of the living and the land of the dead overlap. It is a day for seeing ghosts of those who have gone before us and specters of who we used to be. A day when the past whispers to you as you walk past.

I have spent all day reading through my journals from when I had my PICC line placed. I have thought about all the ways my life has changed. In many ways it has gotten better. But it definitely changed me. There is a before and after in my identity as a chronically ill person. That timeline splits along the line extending from that date.

What’s funny is that while so many things have gotten worse in that time, a lot of things have gotten better. I am much happier. I am much less scared. I am much more independent. I am much more in control of my disease and my life.

I no longer have to convince myself everyday that I can make it through the day with a central line that everyone can see. Because it is just part of my life and it’s no longer extraordinary.