Skip to content

May 2017

Other than flawed

I first fell in love with my body when I was 16. That was also the summer when I first fell in love with a boy; I don’t think they are unrelated. He loved me, too, and we spent most of the summer in his Jeep, driving around New England, camping in the mountains, hiking, climbing up fire towers. I felt strong, strong enough to hike for hours, to swim across a lake in the state park. And he liked my body, too, and I don’t think that’s unrelated either.

It’s funny how much perception affects memory. In my memory, I was slender with a flat stomach. This was my memory because that’s how I believed I looked and that flavors recall. But I have a picture in my bedroom of me that summer and I’m not particularly slender and my stomach wasn’t flat. I have a double chin in pictures from that summer. The difference was that I had not yet begun to care much about my appearance. I knew that I was strong and that was enough.

I never had a summer again as good as that one. I’ve had a lot of great times and had lots of adventures but there is really no substitute for self confidence and your first great reciprocated love. In the way that they always do, things changed. My body changed. And my perception of it changed, too.

When you are sick, your body is out of your control in so many ways. How it functions. What you use its energy for. The way it limits your life and controls your future. How strong it is. What it looks like. What I found is that as I got older, the way my body looked controlled my perception of it, and not the other way around.

Knowing that it is out of your control doesn’t matter much when you have gained 40 lbs of steroid weight or lost 15 lbs from vomiting up every single bite of solid food for months. I tried so hard to exercise even when I knew I wouldn’t be able to because I wanted so badly for my body to return to a shape I recognized and liked. Seeing my moon face in the bathroom mirror every morning was just one more reminder of how much my life was out of my control.

I have been practicing yoga since that summer when I was 16. In the last few years, I have gotten pretty at it, even when I’m feeling sick. 4-5 times a week, I put on a documentary and do 45-60 minutes of yoga. I have tried to use it in the past to control my weight but never had much luck with it. I can’t do intense enough yoga for that to be realistic. But it’s relaxing, stabilizes my joints, and helps keep my arthritis and muscular pain in check.

I do elaborate things to my hair in the morning before going to work. Since I do it myself, I hold up a mirror while standing in front of the bathroom mirror to see how it looks before leaving my apartment. This week, while I was doing this, I realized that I have muscle definition in my back and shoulders. I was shocked. Then I looked at my body in my full length mirror and realized that I have definition in other places, too. When I bend over, you can see my spine.

I gained back most of the weight I lost over the months when I couldn’t eat, healthy weight that I needed. I lost a few more pounds but not because I was so sick. After all this time, I am in the normal range of my BMI. My body is strong. Not all the time and not every day. But it is strong now even if it is sick.

17 years later, I am falling in love with my body again. It finally feels like something other than a flawed vessel holding me back.


The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 25

I answered the 107 questions I have been asked most in the last four years. No jargon. No terminology. Just answers.


33. What is the difference between primary and secondary disorders? How do you know if your disorder is primary or secondary?
• This is a case where we badly need better vocabulary for describing a phenomenon than these two words. The reason for this is because primary and secondary have an inherent numerical association – primary means first, secondary means second. We think that it means primary disorders happen first and secondary happen after. This isn’t always the case.
• A primary disorder does not mean the first disorder to be found. It also does not necessarily mean the disorder that causes other disorders. In this context, primary means only that the disorder arose irrespective of any other health issues, that it was always going to happen anyway.
• So let’s say you have a primary disorder, systemic mastocytosis. Now let’s say that you have another health issue. Whether you had cancer, a bad sunburn, or a broken toe, systemic mastocytosis was always going to happen to you. Or if you lived a charmed life and never had another health issue at all, even a runny nose. Systemic mastocytosis was always going to happen to you.
• We need to make a very, very important distinction here. Somewhere inside your cells was a seed that would later grow into systemic mastocytosis. However, sometimes that seed grows much faster because something else happens to you. People sometimes say “I got SM after I got pregnant”, or “I got SM after a bad car accident”. This isn’t really accurate. You were going to get SM at some point anyway. These are things that really activate mast cells so sometimes events like that can trigger mast cells so that you see the disease then for the first time.
• Clonal disorders are disorders where your body makes too many cells that don’t work correctly. Clonal disorders are almost always considered primary diseases. Clonal mast cell diseases are monoclonal mast cell activation syndrome, mastocytoma, mast cell sarcoma, and all forms of systemic mastocytosis and cutaneous mastocytosis. In all of these conditions, the body makes too many mast cells that do not function correctly.
• Now remember – primary does not mean first. It also does not meet only. You can have multiple primary diseases. You can have systemic mastocytosis and a genetic immunodeficiency. Both of those are primary. If you never had SM, you would still have the genetic immunodeficiency. If you never had the genetic immunodeficiency, you would still have SM. You can have multiple primary disorders.
Secondary disorders are disorders that do depend upon another health condition. Many disorders are secondary.
• For example, steroid induced diabetes is a classic example of a secondary disorder. If you take high dose steroids for too long, you develop diabetes as a result. But if you can get off the steroids, the diabetes resolves. You only had the diabetes because of the steroids. The diabetes was not always going to happen to you. It happened because of the steroids.
• When mast cell patients ask about primary and secondary diseases, they are almost always asking about mast cell activation syndrome. Mast cell activation syndrome is overwhelmingly considered to be a secondary disease. Many patients with MCAS have other diseases and MCAS is thought to be a reaction of the body to the stress and damage of the other diseases. Autoimmune diseases, connective tissue diseases, and even bad IgE allergies are commonly cited as the primary diseases that MCAS is secondary to.
• In a conversation with a world known authority on mast cell diseases, I was told that about 95% of MCAS is thought to be secondary. I also think that MCAS is almost always secondary, though I haven’t seen data to really feel solidly convinced of that.
The terms “primary MCAS” and “monoclonal mast cell activation syndrome” are usually used interchangeably. This is because monoclonal MCAS is clonal (too many broken cells) and clonal diseases are primary diseases.
• However, I am not personally convinced that there is no other primary form of MCAS. This is the same caveat as the previous point – there’s just not enough data. I do think that most MCAS patients have secondary disease. But those are just thoughts without evidence.
In idiopathic disorders, you don’t know why they happen. The same expert told me that idiopathic MCAS occurred in about 5% of cases. In these instances, the patients did not have any other diagnoses that were thought to cause secondary MCAS (and sometimes no other diagnoses at all).
• Let’s review.
• Primary disorders were going to happen to you at some point no matter what.
• Secondary disorders happen when your body reacts to something else happening in your body.
• Idiopathic disorders have no obvious cause.

This is an extremely confusing topic. Please ask questions in the comments if you have them.

For more detailed reading, please visit these posts:
The Provider Primer Series: Mast cell activation syndrome (MCAS)
The Provider Primer Series: Cutaneous Mastocytosis/ Mastocytosis in the Skin
The Provider Primer Series: Diagnosis and natural history of systemic mastocytosis (ISM, SSM, ASM)
The Provider Primer Series: Diagnosis and natural history of systemic mastocytosis (SM-AHD, MCL, MCS)

The absence

My port is currently deaccessed. It has been accessed in the same place, 24 hours a day, 7 days a week, for three years. Except when the needle is changed weekly, or it is briefly deaccessed for another reason, like to go swimming, it is accessed all the time. I feel much safer with it accessed as it gives me ready IV access in case of bad reactions or anaphylaxis. As much as I do not like needing a port, I am very happy to have it. It makes me feel much safer and more secure.

Last weekend, I accidentally tore the needle out of the port. I already have a permanent hole in my skin from being constantly accessed and it made the hole bigger. I was able to get it accessed again safely but something will have to be done about my access site. I may need to get a temporary line placed so that I can deaccess my port for a few weeks and give my skin a chance to heal.

For now, I’m deaccessing for three hours three nights a week and slathering my site with MML (magic masto lotion – recipe at the bottom) to soothe the skin and the site. Meanwhile, I just finished doing yoga on my living room floor, and am sitting here, thinking about my port, the absence of the needle, and the other absences my disease has rendered me.


I didn’t immediately realize what was happening when I started losing my hearing. I imagine it is that way for many people. It was springtime and I was training to once again walk 60 miles in 3 days for a breast cancer fundraising event. I walked a lot, 8-10 miles at a whack, people watching and getting lost in my head, music loud in my ears. When I noticed that the music wasn’t as loud in my left ear, I assumed my headphones were broken. I bought new ones and shortly realized that I had the same problem. Because I am not overly bright, I bought new headphones AGAIN, only to discover that I was being targeted by a complex conspiracy to deprive my left ear of sound.

It was still a few days before I realized that all of these headphones worked fine and that it was my left ear that wasn’t. I could hear in my left ear but it was dampened. I was also beginning to have balance issues. I will never forget the moment when I understood that I couldn’t hear well on my left side and that there was no obvious explanation for it. I somehow just knew that this would not be something that could be readily fixed. I felt this wave of panic, electric and silent, hidden under the beat of my quickening pulse. Always there, waiting without a sound.

I was pretty terrified while I was losing my hearing. Over several months, I lost all the hearing in my left ear and most of the hearing in my right. I listened to music compulsively, constantly. I noticed the blank moments where there used to be notes. I noticed the empty spaces in words. It grew. This hollowness grew and swallowed all these pieces of the world where there used to be sound.

You learn to live around absence. It is an instinct to adapt to your environment. And even though my environment didn’t change, it changed for me. It was both terrifying and fascinating. The way I interacted with the world was fundamentally changed. I was present in a world full of absence.

As strange as it sounds, when I lost my hearing, I wasn’t terribly “sick”. Like I lost my hearing but my overall, day to day functionality was still very good. It was over the next couple of years that things took a serious turn. And you get sick, like properly sick, you lost things. It’s not always a lot at once but it is steady and unending. You lost friends. Opportunities. Money. Jobs. Dignity. A million little things and a lot of big ones.

One of the big losses for many of us with mast cell disease is food. I didn’t start really losing foods in a significant way until late 2013/early 2014. Things picked up speed and soon I was down to very few foods that I could keep down that wouldn’t trigger mast cell reactions or anaphylaxis. I have regained and lost a lot of foods in the last few years. This past winter was the worst patch in a while. I was mostly limited to liquids, and very few liquids, at that. I was still eating plain potato chips because without any other solids, I would wake up because of the hunger pains. I lost a ton of weight, a lot of muscle, and a whole lot of hope.

I started Xolair injections in late February. My expectations were pretty tempered but it was worth a shot. Within a week, I could keep down some solids. My stomach has become a lot of smaller and I still can’t eat a lot of food at once but I can eat again. I can eat things I haven’t eaten safely in years. I still react to certain foods but I don’t seem to react anymore to the process of eating.

I am very aware that I could lose solids again at any time. For now, I’m just trying to be present.
*Magic masto lotion (MML): a cream applied to the skin for hives, itchiness, eczema, really any type of mast cell skin irritation. It is made by mixing liquid cromolyn with whatever your safe cream or lotion is. Some of us have prescription liquid cromolyn ampules while other people making it using over the counter Nasal Crom. I put about 3 ml of cromolyn liquid in a plastic bag, squirt some cream in there, close the bag, mix it up, and then slather it on wherever I want. Always speak with your health provider before adjusting your treatment plan.

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Disease, Part 23

I answered the 107 questions I have been asked most in the last four years. No jargon. No terminology. Just answers.

  1. Is mast cell activation the same as mast cell activation syndrome?
  • No.
  • This is the single most important clarification I make as an educator. It is crucial to understand that they aren’t the same thing, especially if you research mast cell activation syndrome online.
  • Mast cell activation is a normal and healthy process. Mast cell activation mostly means that they are ready to release chemicals in response to signals from inside the mast cell or from other cells. It is one of the major ways mast cells carry out their normal functions, like fighting infections, healing the body post trauma, and regulating the menstrual cycle.
  • Many things activate mast cells to tell mast cells to act in their normal functions. Bacteria, viruses, fungi, cancer cells, diarrhea, pain, surgery, physical or emotional stress, and many other things all activate mast cells normally. It is not surprising that these things activate mast cells because they should activate them.
  • Sometimes mast cells overreact to signals to activate, like in allergies and anaphylaxis.
  • The reason mast cell activation is a problem in mast cell disease is because mast cells respond way too strongly to activation signals. They release too many chemicals too often.
  • The other reason mast cell activation is also a problem in mast cell disease is because they become too easily activated.
  • Think of mast cells like houses. Like any house, they have doors. In healthy people, you need a lot of people knocking on the doors and windows at the same time to get the mast cell to open the doors and release chemicals. In mast cell patients, one person can knock a few times and all the doors open and release chemicals at once.

For more information, please visit this post:

The Provider Primer Series: Introduction to Mast Cells

The Cost of Being Sick

I am fortunate to have comprehensive health insurance. The table below is what it would cost me to stay alive and functioning without insurance.

This is the cash price of my medical expenses for a year. A good year. Figures are rounded but based upon explanation of benefits (EoBs) for past procedures, medications, etc.

I have excluded the costs of living in a safe apartment, full of safe furniture, with utilities turned on, to be able to care for myself. I have also excluded the costs of skilled nursing that I do for myself (which saves about $250 a DAY).

I have chosen to ignore the fact that the stress of not having insurance would significantly trigger my disease, resulting in higher expenses. I have chosen to ignore this because it would also likely kill me so my expenses would drop off pretty quickly.

If I lost my insurance, used every bit of money, and sold everything I own, I could pay for less than a month of care.

I’m sure I’m forgetting some stuff. I’ll add them in when I think of them.

Item Cost Frequency Total per year
Standard meds (cetirizine, ranitidine, cromolyn, ketorolac, ketotifen, levothyroxine, magnesium, calcium, vitamin D, turmeric, prednisone, trimethobenzamide, ondansetron, metoclopramide, montelukast, gabapentin, fluticasone, glycerin, lidocaine jelly, and pain meds) $1,527 Monthly $18,324
IV meds (IV Benadryl, IV Pepcid, IV Solu-Medrol, 5% Dextrose in Lactated Ringer’s ) $1,600 Monthly $19,200
Specialty injectable meds (Enbrel, Xolair) $6,709 Monthly $80,508
Compounded medications $110 Monthly $1,320
As needed meds $85 Monthly $1,020
Epipens $650 (2 pack) Three times a year $1,950
Office appointments (routine) $980 (4/month) Monthly $11,760
Office appointments (specialty, procedure) $1,260 (2/month) Monthly $15,120
Surgical procedures (scopes, etc) $3,000 Twice a year $6,000
Lab work $1,000 (4/month) Monthly $12,000
Skilled nursing care $1,000 (4/month) Monthly $12,000
Central line supplies $800 (4/month) Monthly $9,600
Other medical supplies $150 Monthly $1,800
Imaging studies $1,800 Four times a year $7,200
ER Visits $4,400 Twice a year $8,800
Hospitalization $6,850 (per night) 7 nights a year $47,950
Major surgery $29,000 Once every other year $14,500
Ambulance $650 Twice a year $1,300
Dentist $325 Twice a year $650
Safe foods $450 Monthly $5,400
Transportation (Uber, taxis, gas) $300 Monthly $3,600
Phone (to schedule appointments, receive results, communicate about medical issues) $80 Monthly $960
Total     $280,962


The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Disease, Part 22

I answered the 107 questions I have been asked most in the last four years. No jargon. No terminology. Just answers.

  1. Does mast cell disease cause cognitive issues?
  • Yes.
  • The most common cognitive issue reported by mastocytosis patients is “brain fog”, a sort of difficulty in thinking and reacting normally.
  • Inability to focus, pay attention, find words, and keep things in short term memory are frequently reported by mast cell patients. Attentive deficit disorders are sometimes seen.
  • Aside from the effects of mast cell disease on your body, they also affect the lives of patients dramatically. 42% of mastocytosis patients in one study reported a high stress level. I would be willing to bet that across the entire population of mast cell patients, the number of people that feel a lot of stress is a lot higher than 42%. Many patients feel hopeless, guilty, or like a burden. While this is distinct from depression, a neurologic disorder, these feelings can make it hard for patients to focus or pay attention.
  • Mast cell disease can lower serotonin. Even where this is not the case, mast cells can greatly impact the way serotonin works in the body. Serotonin in a chemical that nerves and other cells use to talk to each other. It is also important in cognition. While this isn’t totally understood yet, it appears that increasing serotonin levels can improve memory and decrease impairment. It can also improve ability to learn things. Not enough serotonin was associated with memory and learning difficulties.
  • When mast cells are activated, your body thinks there is an emergency or an infection. It can activate a stress response. One of the things your body does during this response is release cortisol. Cortisol can further activate mast cells. It is also released by mast cells. Over time, more cortisol than normal can really fatigue the body. Long term stress response is associated with a lot of cognitive issues, including brain fog.
  • Mast cell disease is very disruptive to your sleep cycle. Personally, this is one of the hardest parts of the disease for me. Your body naturally starts releasing more histamine around 10pm, every night, for everyone. Mast cell patients often have worsened symptoms starting around then and continuing overnight.
  • Another mast cell mediator, prostaglandin D2 (PGD2), is the strongest known inducer of sleep in the body. Mast cell patients may have this in excess, making them even more tired.
  • Despite the common idea that histamine makes you drowsy, it actually keeps you awake. Many mast cell patients have insomnia because of the histamine release overnight. This translates to being exhausted during the day when histamine levels drop. Lack of sleep is a well documented cause of cognitive dysfunction.
  • Many mast cell patients have POTS or another form of dysautonomia. These conditions can prevent getting enough blood and oxygen to the brain.

For more information, please visit these posts:

Neuropsychiatric features of mast cell disease: Part 1 of 2

Neuropsychiatric features of mast cell disease: Part 2 of 2

MCAS: Neurologic and psychiatric symptoms

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 21

I answered the 107 questions I have been asked most in the last four years. No jargon. No terminology. Just answers.

30. Why does my skin get red and itchy?
• Flushing is one of the hallmark signs of mast cell disease. It is sometimes the symptom that drives providers to look at mast cell disease as a potential diagnosis.
• Mast cells make and release many chemicals. These chemicals are commonly called mediators because they mediate many reactions in the body that affect the body in many different ways.
• Some mast cell mediators make blood vessels relax. The vessels get a little wider. When the vessels get bigger, the ones under the skin get closer to the skin. Because those vessels show red from the blood in them, the blood is closer to the skin so the skin looks red.
• Flushing is often asymmetrical. There isn’t a hard and fast reason for why this happens but is likely caused by local mast cell mediator release. Essentially, if the mast cells on the right side of your face get irritated, the right side is more likely to flush than the left side.
Flushing is mostly mediated by prostaglandin D2. Aspirin is often prescribed for mast cell patients that tolerate it because aspirin blocks cells from making prostaglandins. This is because aspirin interferes with the molecule that manufactures them. Many other substances can also interfere with this, including other NSAIDs. Another class of drug, 5-lipoxygenase inhibitors, can also stop production of prostaglandins in a different way.
• To a lesser extent, histamine contributes flushing and antihistamines sometimes help.
What exactly causes itching is still not entirely clear. There are special little places in your body called itch receptors. When they notice something itchy, it’s their job to raise the alarm. We think that mast cells carry the message from those places to the nervous system that then spread the itch signal. It’s like carrying the flame of one candle from the itch receptor to the nervous system, which sets the forest on fire.
• Hydroxyzine and other antihistamines are often used for itching. Corticosteroids like prednisone, either oral or topical, may help. Also, medications that interfere with prostaglandin production, like an NSAID or a 5-lipoxygenase inhibitor, sometimes help.

For more detailed reading, please visit these posts:
The Provider Primer Series: Management of mast cell mediator symptoms and release
Prostaglandins and leukotrienes
Mast cell mediators: Prostaglandin D2 (PGD2)

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 20

I answered the 107 questions I have been asked most in the last four years. No jargon. No terminology. Just answers.

29. Why do I swell up when I have a reaction? Where does the fluid come from and where does it go?
• Your body feeds its cells by keeping blood circulating. The blood passes by cells. The cells pull nutrients, oxygen, and other things they want out of the blood. In return, the cells release their wastes into the bloodstream to carry them away to a place where they can be broken down.
• Mast cells make and release many chemicals. These chemicals are commonly called mediators because they mediate many reactions in the body that affect the body in many different ways.
• Mast cell mediators do many things. They can make blood vessels get looser or tighter to help control heart rate and blood pressure. Their ability to make blood vessels relax is the important point when considering swelling. When mast cells release certain mediators, the vessels relax and get a little wider.
• Vessels are made of a complicated network of cells and fibers. It’s like an afghan blanket: some parts of mostly solid and in other places, there are holes you can put your fingers through. When the vessels relax, those holes get larger so it’s easier for things to fall through the holes. In this case, what falls through the hole is fluid from the blood.
• Keep in mind that blood is a mixture of many things. For right now, let’s divide it into cells and everything else. Everything else is a liquid with some stuff dissolved in it.
• When the blood vessels relax, that liquid from the blood trickles out of the blood vessel and goes into the tissue. In some cases, if the blood vessels relax enough, cells actually fall out of the bloodstream and end up in tissue, too.
• The problem is that once you fall out of the bloodstream, you can’t just turn around and go right back in. That’s why swelling takes longer to subside than other symptoms, even with appropriate treatment.
• Everyone is familiar with the bloodstream. Less familiar is the lymphatic circulatory system. Lymphatic circulation is how your body moves things that fall out of the blood back to the bloodstream. This process is slower than processes that can release other symptoms and can sometimes take days.
• When you swell up, fluid falls out of your bloodstream and gets stuck in your tissues. The cells nearby will absorb some of the fluid and take up molecules they can use. However, if there is a lot of extra fluid there, the cells nearby cannot take up all of the fluid. Gradually, your lymphatic system sucks up that fluid and brings it back toward the heart so it can get back into the bloodstream.
• When you get hives (urticaria), it happens because fluid falls out of the bloodstream in a layer of tissue in the skin called the upper dermis.
• When you get angioedema, it happens because fluid falls out of the bloodstream in lower portions of tissue in the skin called the dermis, subcutaneous tissue, mucosa, and submucosa.
• The fact that the process for hives and angioedema is so similar and really distinguished only by which tissue layer they affect is the reason urticaria and angioedema so commonly occur together and are discussed together.

For more detailed reading, please visit the following posts:
Chronic urticaria and angioedema: Part 1
Chronic urticaria and angioedema: Part 2
Chronic urticaria and angioedema: Part 3
Chronic urticaria and angioedema: Part 4
Chronic urticaria and angioedema: Part 5
The Provider Primer Series: Management of mast cell mediator symptoms and release