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August 2017


Ports were developed expressly for the administration of chemotherapy to cancer patients. They addressed a couple of specific issues in cancer care.

Chemo can be very damaging to blood vessels. Many drugs are caustic and can cause sclerosis of the blood vessels and damage to soft tissues around the vessels. Port lines terminate in very large blood vessels close to the heart so the risk of damage was much lower.

Chemo patients are also at a significantly increased risk for infection. Ports are implanted between the skin and the muscle so they are entirely within the borders of the body. In order to use the port, you push a very large needle through the skin and into the port. This is called accessing the port. When you are done using the port, the needle is removed and the port no longer has contact with the outside environment. They were designed to be accessed occasionally for treatment and to be deaccessed as soon as that treatment was done.

Like so many other things in medicine, ports were not designed with mast cell patients in mind. I am part of a cohort of patients that currently have ports for chronic, continuous use. My port is accessed all the time. There is a needle going through my skin and into my port all the time. The needle is changed once a week and I take the needle out for a few hours sometimes if I am going swimming or taking a bath. Otherwise, the needle is never out.

My port is a bit unusual in that it is very difficult to get the needle into the port without going straight in through one spot. Most patients who have accessed ports move the needle to a different spot when they change it weekly. This allows the skin to heal a bit between accesses. This has never worked for me. For this reason, over the last three years, I have developed a literal hole in my chest over my port where the needle is placed.

Last spring, I accidentally tore the needle out of the port. It made a hole that was already pretty big much bigger. I started deaccessing for a few hours a couple of times a week to try and help the skin to heal. I slathered cromolyn cream all over it and hoped for the best.

In June, I had a temporary IV line called a midline placed in my arm so that I could stop using the port for a few weeks and give my skin a rest. Unfortunately, that didn’t go well. Aside from all the pain in the ass things about having a line in your arm (which I had forgotten), using IV Benadryl through the short line caused phlebitis, a form of vasculitis that affected the vein where the line terminated. I was diluting it to a ridiculous extent (1:50) and it was STILL causing really severe pain both while I was pushing it and for hours afterward. We pulled the midline after a week.

The result of always accessing in the same spot is not just a hole but a hole surrounded by thick scar tissue. My nurse was able to get it accessed at different angles by pushing through the scar tissue but it was shockingly painful. The port was getting inflamed and it was getting to a point where the inflammation looked just like a pocket infection. It wasn’t infected but the fact that I could no longer tell the difference was alarming.

The pain surprised me. I can tolerate a lot of pain. This is unbelievable. The hole is now large enough that my body recognizes it as a wound. I have been really tired and achy lately and I think it’s because my body is expending energy trying to close this hole.

I did not expect the emotional effects of this situation. Memories from 2014 keep cropping up. My life is unrecognizable compared to the life I had when it was placed. I knew it was necessary but I was really nervous. I didn’t like having a PICC line but I was finally used to and troubleshoot it without panicking. I was worried that the port would get infected or I wouldn’t be able to access it myself. It was the coda to a year of abysmal health.

It’s funny to reminisce about how anxious I was about getting it placed because my port is my lifeline now. It gives me confidence. It makes me feel secure. I have travelled to the other side of the world with this port. It has allowed me to work full time. It has saved me countless ambulance rides and admissions. It is part of me now, a part that has given me back a lot of my life. A part I don’t want to use.

Because we are concerned that the hole makes me much more susceptible to infection, we decided to just cut our losses and pull the port and place a new one on the other side of my chest. I will be getting this done in the next few weeks. I think things will stabilize without the inflammation.

For me, Septembers will always be tied to pumpkin flavors, the smell of falling leaves, harvest moons, and ports.

Mastos abroad: The China Chronicles (Part 1)

It surprises me how often people think I am unflappable. A lot of times people asking questions say things like, “I know this is stupid,” or “I did a really stupid thing” or “You are going to think I am an idiot.” We all do dumb things sometimes. We all get confused.

I do not think you’re stupid and I do not think you’re an idiot. In that spirit, I would like to share some stories to ensure you that sometimes I am not just flappable but next level flappable. I flap. A lot.

People who have been following along for a while may remember that I went to China in 2015. Seeing the Great Wall has always been a dream of mine. I am fortunate to have a dear friend who lives in Hong Kong for half of the year. She has MCAS and we met because she came to Boston for treatment. She invited me to Hong Kong and suggested we take a tour to mainland China. After spending a few days in Hong Kong, we flew to Beijing on November 10.

There really was no way to prepare myself adequately for mainland China. Hong Kong struck me as a European city with Chinese sensibilities. Mainland China could be compared to nothing. It was like an all you can eat buffet that somehow also had a scarcity of food. I’m amazed I didn’t see anyone get run over while I was there since the locals just hurled themselves haphazardly into traffic. The pollution was unlike anything I have seen before and the entire city smelled like coal.

There was no social understanding that people should be orderly and wait in line or take turns. Louise, our tour guide, warned me, “Do not be a gentle lady.” I am not a gentle lady so I was tossing old ladies out of the way in no time. I will remember this for the rest of my life because it was the only time I have ever been called gentle and it will probably never happen again.

I had wanted to travel to Asia for years before I actually got there. The main reason I hadn’t gone was because my health is unpredictable and I was afraid to receive medical care there. I was also worried that they would confiscate critical meds or supplies. I don’t speak any Asian languages and I was concerned that I wouldn’t be able to communicate effectively in a health care setting.

Because of how nervous I was about needing medical attention in Beijing, I basically traveled there with nothing but meds and supplies for both routine use and emergencies. I flew with a set of three nesting luggage pieces, my backpack with infusion meds and pump, and another bag. The only things I brought that weren’t meds or supplies were three changes of clothes, sneakers, spare glasses, my journal, an electrical converter, a plug adapter, and an iPad. I bought everything else there or got it from Pat.

The other thing that had held me back from travel to Asia was food. I couldn’t eat soy, seafood, vinegar, or fish sauce. It is almost impossible to find food in China that does not contain any of those. I was fine in Hong Kong because I was staying at Pat’s house and we had ready access to fresh groceries and a full kitchen. I didn’t have those things in mainland China and didn’t expect to have them.

The staff at our hotel (the Peninsula) were unbelievably helpful and accommodating of our food restrictions. They came to our room to get a list of what we could safely eat and what we could not. They made us boxed lunches to bring with us on the tour. They made breakfast items that weren’t on the menu because I couldn’t tolerate anything else. Our dinners were carefully made without contamination. We didn’t need to get food anywhere else. Truly, it was an amazing experience. They could not have been more accommodating.

I sometimes print images of places I want to go and paste them into my journal as a reminder to live my life with purpose. The Forbidden City was one of those places. We visited it the day after we arrived. It was a palace for the Emperor that was more likely a small city. The day after we arrived in Beijing, Pat and I passed under a spectacularly large picture of Mao Tsetong and entered this magical place. It was stunning and amazing and better than I had imagined.

The following day was a day I had waited decades for. I was finally going to see the Great Wall of China. Our tour guide recommended that we visit the Wall at a less visited site called Mutianyu to hopefully avoid big crowds. As we drove out of Beijing proper, Louise told us things about the neighborhoods we passed and stories about Chinese history. We chatted about the effects of Communism on her generation versus her parents’ and grandparents’. She was intelligent and spoke really impeccable English.

I lost most of my hearing years ago. I can’t hear high pitched sounds really at all. As we were driving out, Pat turned to me and asked, “Can you hear that?” I couldn’t. I asked what it sounded like and she said she wasn’t sure. She kept hearing it. I kept not hearing it because Deaf. She started looking around in the car. “It’s like a chirping,” Pat explained. Finally, she asked Louise if the chirping was the ring tone on her phone.

We were pulling into a parking spot when Louise answered that she thought it was the driver’s. She asked him in Mandarin if it was his phone. After he parked the car, he reached into his jacket pocket.

I wasn’t looking at them when he pulled it out. I turned back to find him holding out a small cage with an enormous cricket in it. This was the biggest cricket I have ever seen. Easily the size of my palm and black. It was so large that you could see all the features of its creepy little face including its eyes looking back and forth.

If I had been aware that I was going to encounter a cricket the size of Rhode Island on this trip, things might have gone better. But I wasn’t so they didn’t. I’m not sure exactly what happened but I screamed and starting flapping my hands hysterically while making a noise that was sort of like turkeys gobbling.

“Is that the international sign for “get that thing the hell away from me?” Pat asked. It was in fact the international sign for get that thing the hell away from me.

I felt it best not to make eye contact with the cricket as it sized me up. The driver explained that this cricket was a fighting cricket and that people would have cricket fights and bet on the outcome. He kept it in his pocket in case he came upon an impromptu cricket fight. You know. Just to be prepared in case that happened.

Pat asked if this cricket was a champion. Louise translated his response.

“He wants you to know that yes, this cricket is the champion, and also, that if this cricket bites you, you will bleed.” And you know? I believe that. It had to be carried around IN A CAGE. This thing was clearly a menace.


This is a different cricket our driver brought the next day. Sadly, this cricket is not a champion.



The Great Wall was a good distraction from the fact that this cricket was sharing the van with us. It was so foggy. Like walking into a cloud. It seemed to swallow the wall and me with it in the cold damp. After we took the cable car down from the Wall, Pat and I went into a shop looking for something to warm us up. There was a little coffee shop in the visitor’s center. I ordered a small coffee to have something hot. Pat did the same. We sat at a little table outside drinking our coffee while we waited for our driver to pick us up.

For the record, this cup of coffee was the ONLY thing I consumed in Beijing that was not made by the Peninsula. Somehow, despite my excessive fretting over food and drink contamination in China, I decided that this shady little café was definitely capable of making me coffee safely and that it would be fine. I have no excuse. I just flung caution into the polluted Chinese wind.


The Wall disappearing into the fog



As I sat there, sipping my coffee and staring into this grey abyss, I thought to myself, “This is a lot sweeter than I thought it would be.” This didn’t worry me at all. I have no defense. I continued to drink it. I drank the whole thing in about five minutes and got into the van to go the Ming Tombs, the next stop on our tour.

As you might be expecting, that van ride turned into a circus real fast. About fifteen minutes after drinking the coffee, literally within seconds of each other, Pat and I both started having symptoms. It quickly became apparent that this coffee wanted out of our bodies. For me, the coffee felt that vomiting would be the path of least resistance. For Pat, it charged through her GI tract all the way down.

The road we were driving along was punctuated by people sitting on the side of the road and burning hell money, a ritual that they believe will provide their deceased loved one money to spend in heaven. Literally every 100 feet or so had what looked like a small garbage fire being tended to by locals wearing masks. When we finally got to a stretch of road that wasn’t hosting small fires of fake currency, I told Louise that we needed to pull over. I threw up on the side of the road. I threw up enough that poor Louise got out of the van to pat my back while I puked everywhere. Making memories.

I felt better after I threw up but Pat was still having some problems. Our tour guide assured us we were only a short distance from the next stop. After fifteen very long minutes, we jumped out of the van and ran to the bathroom. We told the driver to park as close to the bathroom as possible in case we needed to get out of there quickly. Our tour guide ran with us while the driver went to find a parking spot.

Once Pat was safely in a stall, I walked out of the bathroom to see if the driver had found us. As I was looking for him, the gates flew open on the far side of the parking lot as a van came screeching through the fence. The van pulled up close to the bathroom and slammed on the brakes. The driver jumped out and opened the van door so his passengers could get in quickly. It was our driver.

The good news is that Pat did not shit her pants. The bad news is we were trapped in that bathroom for a while. There were a couple of chairs near the bathroom entrance that we commandeered for a while while we waited to see if this was going to pass or not. Long suffering and committed Louise knelt down on the bathroom floor holding a map and calmly telling us the history of the site while we waited to see our intestines would continue wrenching. We learned all about the Cultural Revolution and how adherents to communist philosophies had permanently damaged ancient monuments and works of art. On the bathroom floor. At a very busy tourist attraction.

After about half an hour, we told Louise that we were ready to brave the world outside of the bathroom. She was very pleased by this turn of events. She led us into the main courtyard and told us about the people entombed there. There was a door where it is traditional for people to walk through, then to walk backwards and hop over the threshold backwards so as not to be trapped in the land of the dead. I was feeling pretty cocky since I hadn’t puked in 40 minutes so I went for it. (I did not get trapped in the land of the dead, if you’re wondering. I think.)

I looked at Pat to ask her if she was feeling up to it. Her response was, “If I don’t keep both feet on the ground, something is coming out of one end or the other and I’m not sure which one.” She’s a lot more practical than me. We carried on with the tour.

Pat and I had some other adventures in China that I’ll get around to writing about someday. I’m sure we’ll have more if I ever get to Hong Kong again.

Anyway, see my point? I’m totally flappable. Your questions are not stupid. You are not stupid. It’s all good.

More posts about my trip to China here:

Great Wall

Just before waking

River stones


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.



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 MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 56

70. What is premedication and when should I do it?

Premedication is taking extra medication in advance of doing something that you expect to trigger your mast cells. The current premedication protocol for mast cell patients is as follows:
Prednisone 50mg orally (20mg for children under 12): 24 hours and 1-2 hours before procedure
• Diphenhydramine 25-50mg orally (12.5 mg for children under 12) OR hydroxyzine 25mg orally, 1 hour before procedure
• Ranitidine 150mg orally (20mg for children under 12) 1 hour before procedure
• Montelukast 10mg orally (5mg for children under 5) 1 hour prior to procedure

This protocol was developed for the Mastocytosis Society by Dr. Mariana Castells and the original can be found here.

This premedication protocol uses medications to interfere with the molecules mast cells release as well as medication to decrease the amount of molecules mast cells make and release. Diphenhydramine (called Benadryl in the US) stops histamine from getting to the H1 histamine receptors on the outsides of many cells. Ranitidine stops histamine from getting to the H2 histamine receptors on the outsides of many cells. In these ways, these medications can help to stop symptoms from histamine released by mast cells.
In a similar way, montelukast stops leukotrienes from getting to receptors on cells. This helps to curb some of the symptoms that occur when leukotrienes are released by mast cells.

Prednisone is a glucocorticoid, commonly called referred to as a “steroid.” This medication suppresses the production and release of inflammatory molecules by mast cells and other immune cells. Importantly, this medication can take hours to achieve maximum effect. This is why the first dose is the day before the event for which you are premedicating. By being dosed again a couple of hours before the event, it can also provide some additional protection for delayed reactions.

It is important to know that this premedication protocol may need to be changed to achieve the most effective protocol for individual patients. These recommendations are general and are not based upon study or clinical trial data.

This procedure is intended to be used for all major and minor medical procedures, including imaging tests like x-rays and MRIs, whether or not they use contrast. However, many patients find some benefit in premedicating for other types of events as well, such as flying, childbirth, and days of planned elevated physical or emotional stress. Patients should discuss what sorts of events are appropriate to premedicate for with a knowledgable provider.

For more detailed reading, please visit the following post:
Premedication and surgical concerns in mast cell patients

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!

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 55

69. What routine monitoring should mast cell patients receive?

There are not yet routine testing recommendations for MCAS patients, but there are some for mastocytosis patients. Many doctors use the mastocytosis recommendations to monitor their MCAS patients in the absence of specific MCAS guidelines.

Mastocytosis patients should monitor tryptase level annually. In mastocytosis patients, tryptase level is often a good marker for how many mast cells are in the body (although this is not always true.) If a patient’s tryptase is increasing over time, the provider will need to check other things to see if their disease is moving to a more serious disease category.

DEXA scans measure bone density. Osteoporosis is a common complication of systemic mastocytosis. Patients should receive regular osteoporosis screening, even if they are young.

Mastocytosis patients usually receive routine bloodwork annually that includes a complete blood count (CBC), which counts the amount of blood cells a person has; and a metabolic panel, which looks at how well the liver and kidneys are working.

Repeat biopsies are usually only done if the result will change treatment in some way. Most patients with systemic mastocytosis are diagnosed based upon bone marrow biopsies. These don’t usually need to be repeated unless tryptase level increases sharply or there are unusual results in routine blood count testing. Increasing tryptase can indicate that the body is making more mast cells much faster, which is sometimes linked to a more serious disease category. Unusual blood cell counts can indicate not just too many abnormal mast cells, but also other bone marrow conditions sometimes seen in mast cell patients, like myelofibrosis and essential thrombocythemia.

Patients with cutaneous mastocytosis are diagnosed by skin biopsy. There is not usually a need to repeat a skin biopsy for patients with CM.

Patients with systemic mastocytosis are usually diagnosed by bone marrow biopsy but can also be diagnosed as a result of a positive biopsy in any organ that is not the skin. A person can be diagnosed with SM via a GI biopsy.

GI biopsies are a little different than bone marrow biopsies in that there are sometimes reasons to repeat them. GI biopsies may be repeated to see if the general inflammation in the GI tract is improved or worsened. The provider may also be interested in whether or not the amount of mast cells in the GI tract has decreased. The result of GI biopsies often change treatment options so it is not unusual to repeat them. However, unlike bone marrow biopsies, repeated GI biopsies do not tell the provider if the mastocytosis is moving toward a more serious disease category or not.

MCAS patients are diagnosed based upon positive tests for molecules that indicate mast cells are overly active, like n-methylhistamine, and D2- or 9a,11b-F2 prostaglandins. Once the patient is diagnosed, there’s not a clear rationale for repeating these tests, although some providers do for their own information. Some providers like to check prostaglandin levels to see if treatment to stop mast cells from making prostaglandins (like use of aspirin or other NSAIDs) is helping.

However, it is important to understand that the level of mast cell mediators is not associated with symptoms. A person who has a normal level of 9a,11b-F2 prostaglandin may have the same symptoms as a person above the normal level, who may have the same symptoms as a person who has three times the normal level. For this reason, many providers consider these mediator tests to be less about the numerical value of the test and more about whether it’s normal or high, period.

For more detailed reading, please visit the following post:
The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 5
The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 6
The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 7
The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 8
The Provider Primer Series: Diagnostic criteria of systemic mastocytosis and all sub variants
The Provider Primer Series: Diagnosis and natural history of systemic mastocytosis (ISM, SSM, ASM)
The Provider Primer Series: Mediator testing
The Provider Primer Series: Mast cell activation syndrome (MCAS)

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 54

68. How does mast cell disease affect pregnancy?

One of the things mast cells normally do in the body is regulate the female reproductive cycle. Mast cells in the endometrium, the uterine lining that is shed during menstruation, become activated and release mediators in the days before and during menstruation. Many of the symptoms of premenstrual syndrome (PMS) occur because of mast cell degranulation. These symptoms include things like cramps and bloating.

Because mast cells are involved in controlling the reproductive cycle, they are responsive to the effects of hormones like estrogen and progesterone. In particular, estrogen can directly cause mast cell degranulation.

In some allergic conditions like asthma, patients often have flares right before or during their menstrual period. This is often the case with mast cell patients as well. The change in hormones, the built in mast cell activation, and the bleeding, can all cause mast cell symptoms.

A study on the effects of the pregnancy on mastocytosis found that there was a lot of variability in what patients experienced. 33% of women had symptom improvement during pregnancy. In these women, their symptoms mostly improved beginning in the first trimester and continued throughout their pregnancy. 45% of patients had no change in symptoms during pregnancy. The remainder had worsened symptoms.

Mastocytosis did not seem to affect the outcome of pregnancy compared to the normal population. Premedication was recommended at the start of labor. Many women safely received anesthesia. In women who reacted, 2/3 had not premedicated. Induction of labor with medication was well tolerated. Both vaginal delivery and Caesarean section was performed safely on women with mastocytosis. The frequency of Caesarean section, miscarriage, prematurity and low birth weight were similar to the general population.

In some instances, severe allergic reactions and anaphylaxis can induce early labor, so patients should be aware of this risk.  Histamine can trigger uterine contractions.

An important thing to consider is that mast cell patients may have to change or stop some of their medications while pregnancy to avoid effects upon the fetus. In particular, the use of epinephrine is discouraged in pregnancy because it causes uterine contractions. Mast cell patients should have an alternative plan for anaphylaxis that excludes epinephrine where possible. Any mast cell patient who is pregnant or considering becoming pregnant should have detailed discussions with their providers about it.

For more detailed reading, please visit the following posts:
Pregnancy in mastocytosis
Effects of estrogen and progesterone and the role of mast cells in pregnancy

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.