April 2015

We are Boston

It’s cold in Boston today, and damp and grey; the sort of unredeemable weather Boston is known for. I read the forecast from my iPhone as I drank my coffee this morning. I knew I should dress warmly, but it felt wrong to wear fleece tights and boots in April. I pulled on my blue tights with stars and crescent moons, slipped my feet into warm weather flats that don’t support my floppy ankles. Dressing for warm weather is a sort of hopefulness we Bostonians force upon ourselves – even if it doesn’t come to pass, at least we know we tried.

It was sleeting when I got off the train at Longwood, pebbles of ice bouncing off the pavement, cast aside by passing cars. My legs and feet were cold; I rubbed my fingers together to warm the tips. I put my head down and walked fast to the closest hospital entrance, running through a held-open door under the carved words, “The Peter Bent Brigham Hospital”.

People come from thousands of miles away to be seen here, at my local hospital, Brigham and Women’s. I was born in this hospital. Out of towners call it “Brigham’s”, but anyone from Boston knows that Brigham’s is an ice cream store. No, this is “The Brigham,” because it used to be two separate hospitals, the Peter Bent Brigham Hospital, and the Boston Lying In Hospital, where the discipline of obstetrics was first practiced as a medical science. The Lying In Hospital was a women’s hospital, and when the two merged, it became Brigham and Women’s.

I know lots of these arcane trivialities because I have lived in Boston all my life. I am a Bostonian; I am from Boston. This is my city, and it is part of me.

I rubbed my calves together while waiting for the E line train, my music in my headphones loud enough for a Deaf girl to hear. As I stood up to board the approaching train, I realized it was snowing. I climbed aboard and paid the fare. I folded myself into the smallest shape I could make and sat in the first empty seat, the one closest to the driver. I pulled out my phone and opened the browser. I navigated to CNN, read the banner in bright red.

“They found him guilty,” I said to an E line train car full of strangers. Everything stopped talking and looked at me; they knew who I meant. It is April 8th and snowing in Boston, and Dzhokhar Tsarnaev was found guilty of all thirty charges in relation to the Boston Marathon Bombing.

The day of the 2013 Boston Marathon was warm and sunny. I was babysitting my friend’s daughter that day and I had walked her over to my mother’s house to run around in the yard. I carried the baby into my mother’s living room, where the tv showed live coverage of the finish line. As I sat the baby on the sofa, the bombs exploded.

In the beginning, there was some speculation that this had been a gas explosion. I walked the baby home to make her dinner, watching the muted news from another room as I fed her chicken nuggets. There were early reports of explosions at the JFK Library, which later turned out to be an unrelated electrical fire. I texted, emailed and called everyone I thought might be near Boylston St.

Within a couple of hours, it was obvious that this was a terrorist attack. I packed a bag full of the baby’s things and my father drove over to get us. It didn’t seem like the time to be alone with a toddler.

The news coverage was harrowing. It was uncensored and so graphic and so bloody. The insides of ruined legs, shorn blood vessels, shrapnel. And people who ran to help, regular people picking up the injured and running with them to safety, regular people pinching the ends of veins and holding wounds closed with their bare hands. Not just regular people. Bostonians.

I didn’t think it would be worse the following day, but that was before I knew my mother would call me in the afternoon. “They are showing the names of the people who were killed, and the girl… who died… it’s Krystle Campbell,” she said. I was holding the baby, same baby, on my hip. I packed her things up again and we went back to my parents’ house to think about Krystle Campbell and how she had been murdered by a bomb in our city.

One of my father’s best friends when he was growing up was Billy Campbell. They both grew up and had families; my father had two daughters; Billy had a son, also named Billy, and a daughter, named Krystle. Krystle was my friend when I was a little girl, when we were both little girls together. We spent most warm weekends together for several years when we were kids, swimming in the lake, riding bikes on dirt roads in the woods, sitting by campfires on summer nights. We went to each other’s birthday parties and watched fireworks together on the Fourth of July.

We got older and started doing different things and lost touch. Suddenly it was April 16, 2013, more than half a lifetime later, and Krystle’s face was on the news and on the internet and all I could think about was that she used to be really proud of the streamers she had on the handlebars of her bike. And that that same little girl with the streamers lived for another twenty years until one day when she stood at the finish line of the Boston Marathon and someone killed her and two other people.

Two nights later, the entire city watched the press conference in which they released the pictures of the Tsarnaev brothers. Hours later, an MIT police officer was shot and killed. I knew right away it was connected. I don’t know why I assumed that, but I went to sleep that Thursday night feeling like I might wake up to a very tense situation. I was not wrong.

By Friday morning, residents were being asked to shelter in place and to stay off Boston streets to assist the police in apprehending Dzhokhar Tsarnaev. News crews broadcast images of Faneuil Hall, the Boston Common, Kenmore Square, the Esplanade – all still, completely vacant.

That night, Dzhokhar Tsarnaev was apprehended, found hiding in a boat in a backyard in Watertown, a mile from my grandmother’s house. And I was happy. I was really happy because two days later I went to Krystle’s wake and hugged her parents and her brother and thought about her bike streamers and I was really happy that they had caught him before her family had to say their final goodbye. I was happy that he wouldn’t be able to blow up anything else in my city and that those four people were the only ones he would be able to kill.

If I could point to any one quality that defines Boston and its people more than anything else, it would be the ability to keep going when bad things happen. We are not people who give up. We are not people who let things get to us. We are ordinary people who run towards the sound of explosions in case people need help. We are police officers who chase terrorists through city streets while they throw homemade bombs at us. We are citizens who shovel off the Boston Marathon finish line in the middle of a recordbreaking snowfall in memory of those who died there. We are runners who minutes after finishing the marathon run to Mass General and the Brigham to donate blood to explosion victims. We are first responders who ensure that every person who made it into an ambulance survived their grievous injuries. We are medical professionals in world class hospitals who saved the lives of 264 people in a matter of hours. We are Boston.

The day of the Boston Marathon the year after the bombing, the spectator turnout was great. I never doubted it for a second. Because this is Boston and that’s what we do. Because fuck terrorism. Because fuck Dzhokhar Tsarnaev.

Today is April 8th, and it’s snowing in Boston.  Dzhokhar Tsarnaev was convicted of murdering Krystle Campbell, Martin Richard, Lu Lingzi, and Officer Sean Collier, and injuring 264 other people.

 

 

Love and rainbows

A friend of mine died yesterday. We never met in person, and shortly after we began talking, she found out she was dying. We met through the mast cell community, but she was living with something far more insidious – a rapidly progressing, heritable form of ALS. She died a little over a year after being diagnosed. She is survived by her husband and young son, and many close friends and relatives.

I found out that she was dying yesterday through Facebook. She posted herself that it would be her last day, as she had chosen to invoke Oregon’s Right to Die. I scrolled through all the supportive posts on her page, all the pictures of her with her close friends, recipes she had left for her son.

I learned a lot from her about grace and how to die a good death. Her openness about her illness, and about the ways her body has failed her personally and those in her life in a larger sense, has been a constant source of solidarity for me. She truly embodied the fact that you can love your life and be truly alive even while your body is becoming incapable of sustaining life. She really taught me that life has very little to do with the things you can do and more to do with feeding the relationships you have. I am grateful to have known her.

Every day, I see articles and posts advocating for people to “turn off the screens” and “connect for real”. If it’s not in front of you, if you can’t touch it, it’s not as worthy of your attention, it seems these people think. If you have a conversation via text message, it doesn’t mean as much as one across a table. One of these posts was in my Newsfeed right below Sherrie’s post that was leaving this world today. I shook my head.

These people who write these things don’t know what it means to have a rare disease. They don’t know the loneliness you feel every day surrounded by people who don’t know what it’s like to live in a body that can go into shock without any provocation. They don’t know the overwhelming sense of belonging you feel when you find someone online like you. They don’t know that having someone say, “I understand, I’m the same way,” in a group online can sustain you and validate you just as much as any in person interaction ever will. It is connecting for real, and it’s not less because it happens online.

Sherrie believed that when she died, she would go back up the rainbow she slid down when she was born. She surrounded herself with rainbows, and it became a metaphor for her larger experience. Some weeks ago, after a few difficult days, she wrote the sentence, “The rainbow is calling me.” I shivered when I read it. I can’t believe that the courage and surrender embodied in these few words are less important because I didn’t hear them in person. That’s not how the world works.

What we are doing here, in this community, matters. Supporting each other and understanding each other matters.

When the harder days come, I will remember Sherrie and that life has very little to do with our bodies. It has to do with love and rainbows.

A comprehensive list of antihistamines: H1 receptor (part 2)

Chloropyramine is a first generation H1 antagonist. It is used for allergic eye and nasal symptoms, bronchial asthma, Quincke’s edema, and allergic reactions to food, medications and insect bites. It is available in several European countries as an oral or IV/IM preparation for emergent situations. It has the typical anticholinergic side effects seen in older H1 antihistamines. It is available under several names, including Allergosan, Suprastin, Supralgon and Avapena, in several countries, including Georgia, Hungary, Lithuania, Latvia, Russia, Croatia, Serbia, Bosnia and Herzegovina, Bulgaria and Mexico.

Chlorpheniramine is a first generation H1 alkylamine antihistamine. It is less sedating than other first generation antihistamines. It is sometimes used off label as an antidepressant and anti-anxiety medication as it has serotonin-norepinephrine reuptake inhibiting properties. It is available alone and in various combinations in the US, Canada, throughout Europe and Asia.

Chlorphenoxamine, more commonly known as Systral, is a medication with structural similarities to diphenhydramine. It is mostly used as an anti-Parkinsonian drug and is available in Latin American and Caribbean countries, as well as some European countries and Thailand.

Cinnarizine is an H1 antagonist that functions as both an antihistamine and also as a calcium channel blocker. It is a piperazine derivative. It improves blood flow to the brain and is used for cerebral apoplexy, cerebral symptoms following trauma and cerebral arteriosclerosis. It is also used for nausea and vomiting due to several causes. It is also antiserotinergic and antidopaminergic. Due to its action of dopamine receptors, it can cause parkinsonism if used frequently. It is not available in the US or Canada, but is available under a number of names in many countries, including Brazil, Peru, Philippines, Malaysia, China, Bangladesh, India, and Israel, among several others.

Clemastine is an H1 anthistamine. Though it can be sedating, it has fewer side effects than several other H1 medications. It is also a functional inhibitor of acid sphingomyelinase. It is available in many countries without prescription under brand names such as Tavist, Tavegil or Agasten. It is particularly effective for itching.

Cyclizine is an H1 antihistamine mostly used for nausea, vomiting and dizziness from motion sickness or medications, such as anesthesia. It is also used to potentiate the effects of opiates and opioids. It is a piperazine derivative and is available in the US and UK as IM/IV and oral formulations.

Cyproheptadine is a first generation H1 antihistamine. It is also antiserotonergic and for this reason is used to manage serotonin syndrome. It has a variety of unusual uses, including as a local anesthetic, to treat nightmares in children and due to PTSD, for hyperhidrosis, and to prevent migraine.

Dexbrompheniramine is a widely available medication with structural similarities to chlorpheniramine. It is used to manage general allergic symptoms. It is available in the US and Canada as Drixoral.

Dexchlorpheniramine is the one form of the drug chlorpheniramine. It is available in the US and Canada as Polaramine.

 

Initial diagnosis and treatment of mast cell activation disease: General notes for guidance

Mast cell disease is becoming more well known among both the public and medical providers, but there is still a lot of confusion regarding exactly what it is, how to diagnose and how to treat.

There are several tests that should be used when working up a patient for mast cell disease. Tryptase is the most well known of these tests, due to over 85% of patients with systemic mastocytosis (SM), a form of mast cell disease, having elevated tryptase. However, tryptase can be normal in mast cell patients, or may only be elevated during times of severe symptoms or anaphylaxis. While an elevated baseline tryptase can be used as confirmation for a mast cell disease (in the absence of frank hematologic disease), a normal tryptase test should not be used to discard the possibility of mast cell disease.

24-hour urine tests for mast cell mediators are most likely to capture evidence of mast cell activation when executed correctly. These tests measure n-methylhistamine, a metabolite of histamine, and prostaglandins D2 and F2a, which are all released by mast cells. Urine collected for this test should be kept refrigerated or on ice during collection and transport to the lab. I STRONGLY recommend communicating with the lab prior to beginning to this test to be sure that they understand the temperature requirements. The molecules being tested are not stable at room temperature and inappropriate storage can result in a negative test result in a positive patient. (For details on this topic and specific recommendations for testing, please refer to Afrin 2013).

Some providers also find utility in the measurement of other less specific mediators. Please refer to my previous post on this topic: https://www.mastattack.org/2014/10/mast-cell-mediators-recommended-testing-for-mcas-diagnosis/

Due to the well established time sensitive nature of these tests (Afrin 2013), a patient who presents a “mast cell clinical picture” and responds to typical mast cell medications may in fact have mast cell disease in the presence of negative tests.

Depending on the clinical picture, a provider may feel it necessary to order a bone marrow biopsy, skin biopsy or biopsy of another organ to determine if mast cell infiltrates are present. This is not always immediately done in the presence of positive tryptase, n-methylhistamine, D2 prostaglandin or F2a prostaglandin test and will not always affect treatment. It is common knowledge among mast cell fluent providers that a negative biopsy does not exclude mast cell disease, but it is instead used to rule in the presence of specific proliferative entities like systemic mastocytosis (Picard 2013, Molderings 2011). Furthermore, a single biopsy may fail to capture a positive specimen in a known-positive patient (Butterfield 2004).

For more specific details regarding differentiation among the diagnostic categories of mast cell disease, please refer to my previous post on this topic: https://www.mastattack.org/2014/07/diagnosis-of-mast-cell-diseases/

There are a number of well known, well tolerated medications that can be used to manage mast cell disease. First line medications include antihistamines, leukotriene inhibitors, and mast cell stabilizers (Cardet 2013, Picard 2013, Molderings 2011, Afrin 2013).

Histamine is released by activated mast cells in large quantities. Histamine acts on the body by interacting with four different types of receptors, called H1, H2, H3 and H4. Medications that block the H1 and H2 receptors are available in plentiful supply in many countries. Once diagnosed, mast cell patients generally begin daily treatment with both H1 and H2 antihistamines. Longer acting, non-sedating H1 blockers like cetirizine are typically used to provide a baseline H1 coverage. H2 coverage is achieved with medications like Zantac or Pepcid. Dosage can be increased as needed to provide effective symptom relief, and these medications are often taken in moderate to high doses by mast cell patients. It is not uncommon to take multiple drugs together to block one type of histamine receptor, but this should be managed by a provider.

Leukotrienes are also released by activated mast cells. Singulair is an example of a leukotriene inhibitor that is a common add-on for mast cell patients. This medication is not a replacement for antihistamines.

Mast cell stabilizers achieve effects by making mast cells less likely to release chemicals. Cromolyn is typically the first line mast cell stabilizer in the US. This medication can take several weeks to demonstrate its full effect, so patients and providers should be aware of this fact. Another mast cell stabilizer, ketotifen, is also available in the US through compounding pharmacies. Ketotifen is also an H1 antihistamine.

Medications should ideally be added one at a time to allow easy identification of a bad actor in the event of a med reaction. As a result, tweaking a patient’s medication regimen takes time and patience. If a patient reacts to a medication, care should be taken to determine if the medication is truly the issue or if it is an inactive ingredient in the preparation (lactose, etc).

Mast cell disease can result in a highly variable clinical picture and mast cell patients are often only diagnosed following years of investigation for other possible causes of their symptoms. For this reason, many mast cell patients have acquired a long list of diagnoses prior to a mast cell diagnosis. In some cases, these diagnoses may be accurate and co-existing. All existing prior diagnoses should be considered for their accuracy in light of a mast cell diagnosis.

Additionally, there are a number of conditions which are frequently comorbid with mast cell disease, including Ehlers Danlos syndrome, postural orthostatic tachycardia syndrome (POTS), a variety of autoimmune diseases and several digestive conditions.  Patients should be evaluated according to their clinical picture and laboratory findings.

 

References:

Afrin, Lawrence B. Presentation, Diagnosis and Management of Mast Cell Activation Syndrome. 2013. Mast Cells.

Juan-Carlos Cardet, Maria C. Castells, and Matthew J. Hamilton. Immunology and Clinical Manifestations of Non-Clonal Mast Cell Activation Syndrome. Curr Allergy Asthma Rep. Feb 2013; 13(1): 10–18.

Matthieu Picard, Pedro Giavina-Bianchi, Veronica Mezzano, Mariana Castells. Expanding Spectrum of Mast Cell Activation Disorders: Monoclonal and Idiopathic Mast Cell Activation Syndromes. Clinical Therapeutics, Volume 35, Issue 5, May 2013, Pages 548–562.

Gerhard J Molderings, Stefan Brettner, Jürgen Homann, Lawrence B Afrin. Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options. Journal of Hematology & Oncology 2011, 4:10.

I still live here

I flew home from Florida today. I am feeling pretty beat up. I had an amazing time and regret nothing, but I need to recover in a serious way. I need rest and IV fluids and soft, safe foods. Probably for several days.

My immediate reaction to this thought to be repulsed. I don’t like the idea of spending days in bed, even if I need it. I make so many judgments about my body and what I think it should do. I sleep too much. I don’t sleep enough. I need to exercise more. I need to do yoga every day. Every day, all day, I judge my body and its abilities, a ceaseless undercurrent to my more complicated feelings about being sick.

Why do I do that? Why do I compare every day to the day before? Why does every aspect of my life have to be measured against its previous self?

I judge my body so harshly sometimes. It is too weak. It is too fat. It is incapable of adapting to change. But sometimes I am struck by how utterly amazing my body is in the larger context of my life. It may react while flying through the air at hundreds of miles an hour but it let me ride roller coasters a few days ago. And really, that is amazing.

It is astounding to think how much I could achieve if I just stopped comparing my body to what I think it should be. Because the fact is that every time my body overcomes a reaction or a trigger, it is the result of the convergence of thousands of complex reactions executed in the name of self preservation. It is a miracle it can still recover after all this time.

I am alive. I live in this body. It might fight me, but I still live here. For the first time in a long time, it doesn’t feel like my body is filled up with nausea and bleeding and pain. It feels like it’s filled up with me.