Yesterday was a quintessential New England fall day. It was warm and sunny with a little bite to the wind coming off the ocean. Astoria and I walked four miles. I got a bunch of normal people errands accomplished. I drove to go get coffee. It was really lovely.
It was especially lovely because September is tricky for me. The change of seasons hits my mast cells pretty hard. The falling leaves often have mold on them and mold is a huge trigger for me. I always contend with a pretty significant flare in September but it usually wraps up in a week or two. Last year, I deteriorated rapidly in September, ultimately needing months of medical leave. September is making me apprehensive this year.
On top of the physical issues, my last few Septembers have been pretty wrought with emotional stress. I will always associate this time of year with the fever pitch terror of someone you love almost dying and the horror of what happened to Kristina. I imagine I will have difficult Septembers for the rest of my life.
It’s the middle of the night here and I’m awake because I’m in a lot of pain. My abdomen is really swollen and painful. I’m not sure what happened but it’s the same thing that required hospitalization in June. I was sitting at my parents’ house, talking to my dad, and suddenly I was having killer abdominal pain. Like just this side of screaming pain. I took pain meds and extra steroids and mast cell meds but they didn’t do much. So I’m sitting here with a heating pad on my abdomen, waiting.
I am trying to remind myself that eventually it will stop and then I’ll just be in normal pain. That I’ve been in pain a long time but there are days when it is better. But every new pain is an echo of the first one, a lesser shadow that follows closely, a half step behind me. And if it’s all just one unending pain, a red path burned through my life, it doesn’t really ever stop. It might always be like this. I’m hopeful that it won’t but when you are in a lot of pain, it consumes everything. It consumes your happiness and optimism. It consumes your future.
I’m trying to pack my September full of fun plans to get me through. I’m going to a concert this Saturday with my Portland Soul Sisters, Alli and Alyson. I am going apple picking with my nephew. I am going apple picking again with my nieces the weekend after. My nephew’s birthday party is coming up.
Once I get through September, it will be October and I love October. I love Halloween. I watch a different scary movie every night and decorate my house and get a costume together for trick or treating with the kids. Halloween is always the gift I get for surviving September.
I pulled out all my Halloween decorations and decorated my apartment three weeks early. Even if I am in pain forever, I will still get Halloween. So it’s Halloween in my home.
The university I went to had a nuclear reactor. A real one, underground. The radiation source was an unstable isotope of cobalt that glowed an eerie and otherworldly blue at the bottom of a huge pool.
I was a student there a few years before I found out. I walked over it on almost a daily basis. I had no reason to know it was there until I did and so I didn’t. Naturally, as soon as I found out about the (really woefully and shockingly insecure) nuclear source under my campus, I became very paranoid that it would meltdown or be hijacked by terrorists. I wonder how many hours I have spent worrying about this.
I found out today that a friend of mine has cancer. She told me about her recent appointments and test results. We talked about the grieving process when you receive seriously health news. She hadn’t been having symptoms and the diagnosis took her completely by surprise.
We make a lot of the connection between our minds and bodies. We feel that this linkage is not only real but deeply spiritual, that it is the basis for our awareness of our very selves. We expect there to be signs when something is wrong with our bodies. We feel that we will intuitively known something is off when our bodies harbor such significant health issues. When we don’t, the betrayal is even more searing. We feel that we have failed ourselves in some way and that we have no one to blame but ourselves.
It is harder to reconcile your reality with your experience when the physical state of your body is at odds with your experience of living in it. It makes you wonder what would have happened if you just didn’t know. How many things do we notice only because we know this thing? How many things are significant only because we know this secret hidden inside ourselves? When would you have started to feel sick? Would you ever have noticed otherwise?
I am a scientist and the currency of science is inherently facts and truth. I have always felt it is better to know the facts both in my work and in my health. But it does make me wonder how much time I have lost to perseverating about unlikely worst case scenarios because I demand that knowledge. How much time I have spent being afraid of these things that never came to pass and probably never will? How long have I looked at this shimmering blue and convinced myself of a danger that was never real?
The term organ damage is tricky because people use it to mean a lot of things while providers and researchers often use it to mean one very specific thing. For providers and researchers, the term “organ damage” usually means a change in the organ that affects its structure, like it becomes misshapen or deformed in some way. Structural changes like this are often irreversible. This damage to the organ’s shape and structure usually affects how the organ works, called organ function.
When patients and laypeople talk about organ damage, they usually mean a change in the way the organ functions, even if the structure is not changed at all. This is different in a very important way: changes in an organ that do not affect its permanent structure can sometimes be reversible.
Both cutaneous and systemic mastocytosis cause organ damage in a way that damages the organ’s structure. When too many mast cells burrow into the tissue of an organ, it has to push other things out of the way. When you have mastocytosis, the mast cells like to stick together and form a big clump in the tissue. This punches holes in the tissue, affecting the organ’s structure and shape. This is called dense infiltration. It is one of the criteria for systemic mastocytosis and also happens in cutaneous mastocytosis.
In patients with mastocytosis, those mast cells clumping together cause a lot of the organ damage. This means that people who have the most mast cells usually have the worst organ damage. Patients with malignant forms of mast cell disease, like mast cell leukemia or aggressive systemic mastocytosis, often have organs that are riddled with TONS of mast cells.
Mast cells don’t live in the blood so when your body makes way too many mast cells, those mast cells will dive into whatever organ they can to get out of the bloodstream. This causes damage to the structure that you can see with scans or in biopsies. People with mast cell leukemia and aggressive systemic mastocytosis suffer so much damage to the shape and function of their organs that the organs can totally stop working, called organ failure.
One of the key differences researchers and providers see between mastocytosis and mast cell activation syndrome is that mast cells don’t cause THIS TYPE of structural damage in mast cell activation syndrome patients.
We know this because in biopsies, they do not have mast cells clumped together to punch holes in the tissue. Sometimes they have lots of mast cells, but it is much less damaging to the tissue if they aren’t clumped together. Think of it like poking something with finger versus punching with your fist.
In MCAS, mast cells do not cause structural damage to organs IN THIS WAY. However, many people with MCAS do have structural damage to their organs. Many of them also have organs that do not function correctly even if the organs look normal.
Even if you don’t have mast cells punching holes in all your organs, they can still do a lot of damage. This is because mast cells cause lots of inflammation, which can stress out your organs. Over time, your organs can be damaged by the mast cells releasing too many mediators. While this is not always dangerous, it is certainly painful and frustrating.
Many MCAS and mastocytosis patients have a lot of damage to their GI tracts from years of vomiting, obstructions, diarrhea or constipation. Hives and mastocytosis spots can damage your skin, causing discoloration, scarring or sensitivity. Muscles can become weaker over time because of mast cell inflammation. Swelling can stretch out your skin and connective tissues. Nerves can be damaged significantly, affecting organ function. Bones can become brittle and break, or can become too dense because the body is making new bone when it shouldn’t.
All of these effects on organ function can be caused by mast cells. Major changes in organ function can also cause secondary conditions to arise.
Mast cell patients are also at an increased risk for anaphylaxis which can cause changes in organ function or organ damage.
Patients who have trouble breathing or low blood pressure may not be getting enough oxygen to their whole body. That can cause lasting damage if it goes on long enough.
For more detailed reading, please visit the following posts:
72. How does mast cell disease affect your dental health?
Mast cells are found naturally throughout your body. One of their most important functions is to fight off parasites and infections in your GI tract, starting in the mouth. Everyone has mast cells in their mouth, although most people don’t have a lot of them. They release mediators there like they do everywhere else. For mast cell patients, releasing too many mediators can be a source of symptoms. It causes the oral symptoms many of us experience, including swelling of the lips, mouth and tongue. It can also cause excessive salivation or dryness depending upon the patient.
Your teeth and mouth can be damaged by things that are very acidic. Frequent vomiting as a result of mast cell disease (or anything) can really damage your teeth. It erodes the protective coating over your teeth. It is very hard to effectively wash all the acid out of your mouth after vomiting as it can collect at or below the gumline. This is the reason I personally have had some dental issues in the last few years. Even though I was very diligent about brushing after vomiting, I couldn’t brush beneath the gums to prevent formation of cavities.
My dentist recently recommended that I neutralize the acid in my mouth before brushing instead of brushing immediately after vomiting. Brushing your teeth with acid in your mouth spreads it around your teeth and causes little craters to form on your teeth. My dentist recommended I rinse my mouth out with water and baking soda to neutralize the acid before brushing after I vomit. I also use a prescription toothpaste to help keep my teeth strong. (Always consult your own care team about specific steps you can take before changing your care plan.)
Redness and burning in the mouth can be the result of mast cell activation. For mast cell patients, this can be worsened by exposure to triggers, especially triggers you ingest.
Gum health can be tricky for mast cell patients. For those of us with connective tissue diseases like Ehlers Danlos, we are always at a disadvantage. My old dentist used to constantly give me crap about not flossing even I flossed regularly. This was years before I knew I had EDS and that patients with EDS often have bleeding gums regardless of flossing. Bleeding of any kind activates mast cells, so if you bleed when you brush your teeth, that can be a trigger.
Having swollen or bleeding gums makes it easier for you to get infections in your mouth. Even more seriously, it makes it much easier for infectious organisms to be transferred from your mouth into your bloodstream, where they can cause an infection. This is exactly what happened to me in March 2016 when I had the Danger Tooth pulled. This is a concern for anyone but especially people who have central lines. When you have a central line, bugs that end up in your bloodstream can stick onto your central line and grow more quickly. As this line ends just above your heart, line infections can be very serious, even if they started in the mouth and not the line itself. Sometime dentists treat patients with antibiotics before dental care to avoid this, but it is very patient specific.
Dental cleanings use lots of materials or meds that can trigger mast cell degranulation. A lot of them have extra junk in them, like dyes or flavors. Vibration and scraping during the cleaning can be triggering. Many mouth washes are off limits for us, especially those with dyes and alcohols. And of course, dental work can be painful or cause bleeding which is problematic for us. Anxiety is also common.
I personally do okay with the plain grey pomice scrub for cleanings. Mast cell patients should premedicate before any procedures, including detail appointments. See the link below for the premedication recommendations for mast cell patients.
Dental procedures or surgeries have the same problems as cleanings but to a stronger degree. Installing permanent or semipermanent hardware into the mouth carries the risk of later reacting to it. Braces, retainers and splints can be super tricky for us. The decision to put in a crown or something similar should involve the mast cell specialist on your care team. I personally have opted to have a tooth pulled rather than run the risk of later reactions to the crown.
Numbing medications can be mast cell triggers, like some of the –caine anesthetics. Sometimes dentists will use a preparation of anesthetic that also has a little epinephrine in it to help control the bleeding. While I personally do not have problems with this preparation, a lot of mast cell patients do because it contains a preservative.
For more detailed reading, please visit the following posts:
71. What other diseases “look like” mast cell disease?
Mast cell diseases have many symptoms that are also commonly found in other disorders. This is one of the reasons why it is difficult to diagnose correctly. The following conditions have symptoms that can look like mast cell disease.
Neuroendocrine cells are specialized cells that help to pass signals from the nervous system to nearby cells, causing those cells to release hormones. There are many types of neuroendocrine tumors. Some conditions that look like mast cell disease are caused by these tumors. Symptoms from them are caused by the response of too much hormone.
• Carcinoid syndrome is the result of a rare cancerous growth called carcinoid tumor. This tumor releases too much serotonin into the body. This can cause flushing, nausea, vomiting, diarrhea, difficulty breathing, and cardiovascular abnormalities such as abnormal heart rhythm. Mast cells also release serotonin but they release much less than carcinoid tumors.
• VIPoma means vasoactive intestinal peptide –oma. When a word has –oma at the end, it means that it is a tumor. A VIPoma is a tumor that starts in the pancreas. It releases a chemical called vasoactive intestinal peptide. VIPoma can cause flushing, low blood pressure, and severe diarrhea leading to dehydration. A VIPoma can also abnormalities in the composition of the blood. Many patients have low potassium, high calcium, and high blood sugar.
• Pheochromocytomas start as cells in the adrenal glands. They release excessive norepinephrine and epinephrine. They can cause headaches, heart palpitations, anxiety, and blood pressure abnormalities, among other things.
• Zollinger-Ellison syndrome is a condition in which tumors release too much of a hormone called gastrin into the GI tract. This causes the stomach to make too much acid, damaging the stomach and affecting absorption.
Some blood cancers can cause mast cells to become overly activated. They may also cause an increase in tryptase, an important marker in diagnosing systemic mastocytosis.
Some other cancerous tumors like medullary thyroid carcinoma can cause mast cell type symptoms including flushing, diarrhea, and itching.
Most diseases with any allergic component can look like mast cell disease.
• Eosinophilic gastrointestinal disease occurs when certain white blood cells called eosinophils become too reactive, causing inflammation to many triggers. Furthermore, people are more frequently being diagnosed with both EGID and mast cell disease.
• Celiac disease is an autoimmune disease in which gluten causes an inflammatory reaction inside the body. The damage to the GI tract can be significant. Malabsorption is not unusual. Children with celiac disease may grow poorly. Bloating, diarrhea, ulceration, and abdominal pain are commonly reported.
• FPIES (food protein induced enterocolitis syndrome) can cause episodes of vomiting, acidosis, low blood pressure and shock as a result of ingesting a food trigger.
• Traditional (IgE) allergies can also look just like mast cell disease. They are usually distinguished by the fact that mast cell patients may react to a trigger whether or not their body specifically recognizes it as an allergen (does not make an IgE molecule to the trigger). Confusingly, it is possible to have both traditional IgE allergies and mast cell disease.
Postural orthostatic tachycardia syndrome (POTS) is commonly found in patients with mast cell disease. However, POTS itself can have similar symptoms to mast cell disease. Palpitations, blood pressure abnormalities, sweating, anxiety, nausea, and headaches are some symptoms both POTS and mast cell disease have. There are also other forms of dysautonomia which mimic the presentation of mast cell disease.
Achlorhydria is a condition in which the stomach does not produce enough acid to break down food properly. This can cause a lot of GI pain, malabsorption, anemia, and weight loss.
Hereditary angioedema and acquired angioedema are conditions that cause a person to swell, often severely. Swelling may affect the airway and can be fatal if the airway is not protected. Swelling within the abdomen can cause significant pain and GI symptoms like nausea and vomiting.
Gastroparesis is paralysis of the stomach. People with GP often experience serious GI pain, vomiting, nausea, diarrhea or constipation, bloating and swelling.
Inflammatory bowel diseases and irritable bowel syndrome can all cause GI symptoms identical to what mast cell patients experience.
This list is not exhaustive. There are many other diseases that can look similar to mast cell disease. These are the ones I have come across most commonly.
For more detailed reading, please visit the following posts:
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.
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.
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.
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.
