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Lisa Klimas

I'm a 35 year old microbiologist and molecular biologist with systemic mastocytosis, Ehlers Danlos Syndrome, Postural Orthostatic Tachycardia Syndrome, Adrenal Insufficiency, and an assortment of other chronic health issues. My life is pretty much a blast.

Metabolic issues associated with MCAS

MCAS patients often have a whole host of metabolic irregularities.  Abnormal levels of electrolytes are very common, as are mild increases in liver function tests, including aspartate transaminase, alanine transaminase and alkaline phosphatase.  Magnesium levels low enough to cause symptoms is not common, although the reason for this is not known.
Vitamin D deficiency is often present in MCAS.  In one study looking at children with asthma, low vitamin D was correlated with decreased lung function and exercise sensitivity.  In MCAS patients, there is no obvious relation to osteoporosis.  Many people receive vitamin D supplements to correct low levels, but it is not clear if there is any benefit to this.

Hypothyroidism (including Hashimoto’s thyroiditis) and elevated levels of TSH are often seen in MCAS patients.  Previous studies have linked hypothyroidism to increased mast cells in bone marrow.  In mice, TSH has shown to increase both the mast cell population in the thyroid and to trigger degranulation.  Hyperthyroidism is sometimes seen in MCAS patients, but much less frequently.  Antithyroid antibodies (TPO) are often high, sometimes extremely high, and sometimes without obvious clinical thyroid disease.

Hyperferritinemia is not unusual in mast cell disease, including MCAS.  18% of ISM patients have high serum levels of ferritin.  It is often misinterpreted as hemochromatosis, even in the absence of the HFE mutation.  MCAS patients with a history of red cell transfusion are often told they have hemosiderosis, even when serum ferritin is much higher than to be expected from hemosiderosis.  High ferritin in MCAS patients is probably secondary to systemic inflammation.  The widely variable nature of the ferritin levels is indicative of inflammation.
MCAS is also associated with obesity and diabetes mellitus (types I and II), all of which are systemic inflammatory conditions.  MCAS patients often have lipid abnormalities.  Hypertriglyceridemia is the most common presentation, but there are many variations.  Lipid issues that have been resistant to treatment with statins are often reversed quickly when MCAS patients are effectively managing their mast cell issues. 
MCAS is also heavily associated with metabolic syndrome.  (There will be a full post on this tomorrow.)

References:
Afrin, Larry B.  Presentation, diagnosis and management of mast cell activation syndrome.  2013.  Mast cells.
A Melander, C Owman, F Sundler.  TSH-induced appearance and stimulation of amine-containing mast cells in the mouse thyroid.  Endocrinology, 89 (1971), pp. 528–533

Siebler T, Robson H, Bromley M, Stevens DA, Shalet SM, Williams GR.  Thyroid status affects number and localization of thyroid hormone receptor expressing mast cells in bone marrow.  Bone. 2002 Jan;30(1):259-66.

Chinellato I, Piazza M, Sandri M, Peroni DG, Cardinale F, Piacentini GL, Boner AL.  Serum vitamin D levels and exercise-induced bronchoconstriction in children with asthma.  Eur Respir J. 2011 Jun;37(6):1366-70. 

Zhang J, Shi GP. Mast cells and metabolic syndrome. Biochim. Biophys. Acta 2012 Jan, 822(1):14-20.

Genetics of MCAS: mutations and methylation

Despite having largely the same symptom profile as SM patients, people with MCAS often lack the signature D816V mutation, considered by many to be a marker of clonality and thus proliferation.  Despite the fact that their mast cells may look normal under a microscope, there is now a growing body of evidence indicating that mast cells in MCAS patients behave aberrantly due to mutations aside from D816V. 
In three studies that look at KIT mutations in MCAS patients, they were found in 26.5%, 44% and 65% of patients, respectively.  Even the average of these three values represents a significant number of people with MCAS who have KIT mutations.  Of note, these mutations are mostly outside of exon 17, where the D816V mutation is found.  In one patient, a mutation was found in the NLRP3 gene, associated with the inflammatory response. 
There are a number of other mutations in genes with various functions commonly found in patients with SM.  These include genes that make proteins to regulate other genes and genes that affect how we make proteins (epigenetic regulatory proteins, splicing machinery and transcription factors.)  To date, there have been no studies looking at whether MCAS patients have similar mutations.  However, there are clear hints that they do. 
The mutations previously mentioned were all somatic and not germline.  This means that the mutations arose after early embryonic development and thus were not heritable.  This fact indicates clearly that there are other germline mutations not yet identified that may induce the subsequent mutations.  This has been bolstered by a 2013 paper that found familial clustering in MCAS patients.   
An important finding is that nearly all mutations found in MCAS patients are heterozygous, meaning only one of the two copies was mutated.  This implies that homozygous mutations, in which both copies are mutated, could cause the cell to die.  Alternatively, the various mutations may work together to make the mutations work as strongly as if there were two mutated copies. 
At CpG sites, the cell can add a special marker to the cytosine called a methyl group.  This is called methylation.  If the cytosine in a gene is methylated, it turns the gene off.  When a gene is turned off, your body will not use that gene or make a protein from it.  This is one type of gene regulation (epigenetics.) 
Looking at the methylation status in the genomic DNA of white blood cells from MCAS patients shows aberrant methylation patterns.  The genes incorrectly methylated included some involved in DNA/RNA repair, DNA/RNA processing, cell death, cell activity and communication with other cells.  195 individual CpG sites have been identified as candidates as markers for MCAS.  Importantly, there is a correlation between the age of symptom onset and the year of birth, which indicates a sort of anticipation of developing MCAS.  This means that gene regulation by methylation could affect acquisition of later, non-heritable mutations like the ones seen in KIT.
A gene is made up of introns and exons.  When an RNA code is made from a gene to tell the cell how to make a protein, the cell cuts out some pieces of that RNA.  These pieces are called introns.  The remaining pieces, which are connected back together, are called exons.  The way the cell cuts the RNA and reconnects the pieces is called splicing.   The mutations in KIT seen in MCAS patients almost all involve intron and exon junctions, where they meet.  There are also some differences in the way splicing occurs in MCAS patients. 

Reference:
Molderings, Gerhard J.  The genetic basis of mast cell activation disease – looking through a glass darkly.  2014.  Critical Reviews in Oncology/Hematology.

Constitutional symptoms of MCAS


Constitutional symptoms are any symptoms that affect the function of several systems at once.  They are often nonspecific and can be attributed to many causes, complicating diagnosis. For many people with MCAS, the constitutional symptoms present first and with the greatest frequency.

Fatigue and malaise (the feeling of being “out of it”) are the most common symptoms reported in MCAS patients.  While many patients with these symptoms remain functional, for some, it can be truly, severely disabling, with some patients sleeping for the majority of the day.  Dr. Afrin has referred to stories of “patients in their twenties acting like they are in their eighties.”  Chronic fatigue syndrome, in which patients have severe fatigue unrelated to exertion, not relieved by rest and unrelated to other medical conditions, has been tentatively linked to mast cell activation by Dr. Theoharides. 

I see a lot of discussion about whether or not fevers are part of MCAS.  It depends which researcher is reporting information.  Castells feels strongly that fevers are not part of MCAS symptomology, while Afrin and Molderings feel that they are.  They report that intermittent elevated temperatures are not unusual.  These temperatures are low-grade temperatures, with frank fever being quite rare.  MCAS patients often report constantly feeling cold, though chills and shaking is less common.

I know that one of the ways I can tell my mast cell disease is ramping up is by severe night sweats.  This is apparently common in most presentations of mast cell disease.  As such, many MCAS patients have severe, unprovoked sweating, often overnight, sometimes not.  Some patients report a circadian rhythm.  Furthermore, this sweating is often accompanied by swollen or tender lymph nodes.  When these two symptoms are taken together, usually infection or lymphoma is suspected.  Once these are ruled out, patients are often left with no relief for this frustrating symptom.

Some patients report lack of desire to eat.  Some report quickly feeling full (early satiety.)  In some of these patients, the root cause is a swollen spleen.  A minority of MCAS patients lose weight due to their disease.

Weight gain in MCAS patients is far more common than weight loss.  It often begins suddenly and progresses rapidly, in the absence of dietary or activity changes.  This is partially due to the fluid dynamics of edema due to mast cell activation.  Less often, weight gain is from ascites (free fluid in the abdomen) or serositis, inflammation of the serous tissues, including the pleura (tissue lining the lungs), pericardium (the compartment containing the heart and origination of the large vessels connecting to the heart) and the peritoneum (tissue lining the abdomen.) 

However, the gain in adipose (fat) tissue seems to be responsible for most of the persistent weight gain.  Some patients gain more than 50kg in a year despite significant caloric restriction and frequent exercise.  Many people (and their providers) often attribute their worsening symptoms to the gained weight.  Some people undergo bariatric surgery and despite initial losses, regain the weight, with no improvement of other symptoms.

Pruritis (itching) is very common in MCAS.  Its presentation is varied; episodic or constant; local or diffuse; migratory or not; tolerable or disabling.   

The hallmark of MCAS is that patients invariably present with a collection of “sensitivities.”  These include severe or bizarre reactions to virtually anything, including drugs, food and environemental triggers.  Environmental triggers can be due to the presence of common allergens, physical (such as heat), electrical (such as generation of electrical charge when brushing hair) and even osmotic.  Exposure to harmless microorganisms can cause severe reactions.  Summers are often difficult for MCAS patients due to heat and increased UV exposure, while spring and fall are difficult due to pervasive pollen.  Triggers can cause reactions when the patient touches, inhales or ingests them. 

Though less of a problem than heat, exposure to cold can trigger a hyperadrenergic response that will fuel mast cell activation.  Care must be taken to avoid temperature extremes on either end of the spectrum.

Drug sensitivities are often found to be due to an inactive ingredient in the formulation.  Compounding is an important tool for MCAS patients.  Lactose monohydrate and potato are common fillers for MCAS patients.  Reconstitution at time of use with water is also not uncommon.

 

Reference:

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

Worth

I saw a new doctor last week to address some hormone issues.  She had never heard of mast cell disease.  I gave her the run down succinctly: “The hallmark of my disease is anaphylaxis in the absence of IgE stimulation.  We can anaphylax at any time for any reason.  Some things make reactions more likely and we avoid those things when possible.”
Immediately upon hearing these words, she became uncomfortable.  She took some history and asked me what medications I took.  She looked over my test results and told me I should have IM steroids available in case of emergency.  When I told her that injections increase my risk of anaphylaxis and that I use IV meds to avoid that, she literally threw up her hands.  “Well, do you want the shot or not?” she asked crossly.  I told her I didn’t want it. 
She informed me that I needed several blood tests, which needed to be drawn first thing in the morning.  No one at the lab will draw out of my port and getting blood drawn results in several sticks and generally cause a reaction.  I asked her what I should do and she said, “Well, we’ll figure that out later.”  Which means that she doesn’t care enough to help me figure it out.
She had me get up on the exam table.  She listened to my heart.  “When you touch my skin, it may start to welt, that’s normal,” I told her.  “Oh, I’m not going to touch you,” she said dismissively.  I closed my eyes and felt my hands curling into fists.  She put her stethoscope away and ended the appointment.
I left her office upset.  I have been around the block with arrogant doctors.  They don’t really stress me out much anymore.  This was different.  This was not an instance of a doctor thinking they knew better than me how to manage my disease.  This was an instance of a doctor deciding that treating me effectively was more trouble than I was worth.  She decided that I wasn’t worth an examination.  She decided it didn’t matter if I got those tests because she didn’t care anyway.  
I live with this disease every day.  Most of the time, being sick isn’t the hard part.  Waking up every day and trying to believe that I am worth the trouble – that’s the hard part. 

Birthday wish


I turned 30 on my last birthday.  It was very cold outside, but it was dry.  I stood around a fire with my friends that night and looked for the moon.  It was barely alive, just a white sliver hiding behind the clouds.  It matched my mood.  The knowledge that it would grow to be heavy with light in the sky was little consolation.

The day before my birthday party, I cut my vacation short to see a rheumatologist.  She was sympathetic and wise, but largely didn’t know what to do with me.  She sat with me for two hours while we talked in comfortable armchairs.  It didn’t help much in the way of the treatment, but sometimes unburdening myself is treatment enough. 

I watched as my sister lit the candles on my birthday cake.  I wasn’t in much of a wishing mood.  I leaned forward and for the first time in several years, I did not wish to get healthy in the coming year.  Instead, I wished for a world without mast cell disease, and I meant it. 

I think about mast cell disease all day long.  I dream about mast cell disease.  When people ask how I’m doing, I am completely honest.  I read mast cell papers, write mast cell articles, answer mast cell questions.  I tell stories about this mast cell life I live because I think if I try hard enough, I can make people understand what it means to live like this. 

But all these words can’t make them feel the way my pain throbs under my ribs.  They can’t make them literally hear connective tissue tearing when I reach too far to tip something closer with my fingertips.  They can’t make them feel the silent terror that swells anew every time I put something into my mouth. 

I tell people about my life in graphic detail because I think if I try hard enough, I can make them care about mast cell disease.  And maybe if enough people care, someone will do something about it. 

Someone was telling me about their recovery from surgery today.  I listened while standing on one leg, a habit I developed when I broke two bones in my lower back as a teenager.  “Oh, that happens because of mast cells,” I interjected.  “Mast cells are involved in tissue remodeling.” 

I find myself saying things like this a lot lately.  “That’s because of mast cells.”  Scab itchy?  That’s mast cells.  Flushed after running?  Mast cells.  PMS?  Mast cells.  Mast cells are everywhere, doing everything, all the time. 

What I find very strange is the fact that this ubiquity of mast cells is recently making me feel more connected to people.  Every time I tell someone that their mast cells have done something to them, I feel like I have forged a tiny bit further in this daily struggle to make people care.

Living with mast cell disease seriously sucks, but I can’t deny that it forces me to live my values.  Last year when I wished for a world without mast cell disease, I knew that it would take brutal, public honesty about my life and my disease.  I knew that it would take constant studying, constantly talking about my personal health, and constantly being willing to educate people. 

In the last month, I have been asked to talk about life with mast cell disease to key opinion leaders, researchers and doctors in the mast cell community.  I have been approached by a start-up looking to lay the groundwork for mast cell research.  I have received an overwhelmingly enthusiastic response to an upcoming department presentation I am giving on mast cell disease. 

I know that going to a presentation won’t make them understand what it feels like to live in my body.  But I don’t think that’s necessary for progress to be made.  Maybe all we need is for them to say, “I bet it sucks to live in a body like that.”  Maybe that’s enough. 

This is what change looks like.  This is what it looks like when we’re a little closer to a world without mast cell disease.

Hope

Yesterday I got myself really stressed out and had full blown anaphylaxis.  I used my epipen and pushed meds and sulked on my couch.  It was a sulking kind of day. 
Today I woke up with sore, weak muscles.  My hands shook as I made coffee in the dark, my eyes too sensitive for light.  My skin burned.  I burnt my tongue on my coffee, swallowing a handful of pills.  I rolled my eyes when I confirmed that I still have a lower GI bleed.  I hooked up another bag of IV fluids and went back to bed.  I thought about some recent events and how low they had me feeling.  I thought about the danger to a community that doesn’t get all the information they need.
But tonight I got hundreds of messages from people supporting me and thanking me for all I do.  It was really, really humbling and amazing. 
There needs to be a safe place to vent about your experiences and receive support from people who understand you.
There need to be multiple reliable sources of information, with cited sources, readily accessible by people who need it to make decisions.
There needs to be security in the community that you are among honest people who do not seek to manipulate others. 
There needs to be certainty that when you entrust someone with your personal health information that they will not betray that confidence.
There needs to be an understanding that people who do what I do exist to serve our community, not the other way around. 
There needs to be democracy.  There needs to be a way for people to disagree without retribution.
There needs to be hope.  People deserve to know that it’s not hopeless. 
I have mentioned before that a lot of people tell me their secrets.  They’re not always health related; I seem to be the recipient of quite a bit of gossip these days as well.  I told a mast cell friend of mine recently that I didn’t know why people told me these things. 
She responded, “It’s because you’re our leader,” and I shivered.
I don’t think of myself as being a leader.  I think of myself as being a sick woman who is trying to help other people be less sick and more heard.  I am just another mast cell patient.  I am just like all of you. 
But if you think of me that way, then I want you to know this: 
It bothers me to see so many scared people when we live in an age of so much hope for these diseases. 
This is not rhetoric.  We are seeing real results with new medications and are diagnosing people earlier.  Just twenty years ago, mastocytosis was usually found on autopsy.  How amazing that now we can be treated and live with this, even when it doesn’t feel like a blessing.
I believe we won’t always be sick.  I believe that if just twenty years ago, very few could live with mastocytosis, that in another twenty, we’ll all take a pill every day and be free.  I really, really believe this. 
None of the nonsense that happened this week matters.  If I can give people hope, it is worth it. 
Hope is what’s important.  I have it.  So should you.

Effects of estrogen and progesterone and the role of mast cells in pregnancy

The term estrogen generally refers to estrogen estradiol (E2.)  This steroid hormone is induced when gonadotropin releasing hormone (GnRH) is released in the hypothalamus and acts on the pituitary gland.  This in term releases follicle stimulating hormone (FSH) which acts on the follicle, resulting in the release of estrogen.  Secretion of GnRH is stimulated by a protein called kisspeptin. High levels of estrogen or progesterone inhibit the secretion of kisspeptin.  Hormone levels are regulated in this way. 

Estrogen is mostly produced by the ovaries and placenta, but is made in smaller amounts by the liver, adrenal glands, breasts and fat cells.  E2 promotes secondary female sex characteristics, increases metabolism, increases fat stores, stimulates endometrial and uterine growth, promotes vaginal lubrication, thickens the vaginal wall, maintains integrity of blood vessels and skin, reduces bone resorption and increases bone formation.  It also promotes effective coagulation by increasing platelet adhesion.  E2 increases HDL cholesterol and triglycerides while decreasing LDL and fat deposition.  It balances salt and water retention, increases cortisol levels, reduces bowel motility, and increases the amount of cholesterol found in bile.  It also promotes wound healing and has anti-inflammatory properties.

With progesterone, E2 promotes and maintains the uterine lining, as well as increasing the amount of oxytocin released during pregnancy.  Estrogen surge induces the secretion of luteinizing hormone, triggering ovulation. 
Progesterone (P4) also regulates salt and water balance, prepares the uterus for implantation, affects vaginal tissue and cervical mucus to prevent sperm from entering the uterus during pregnancy, suppresses menstruation, decreases maternal immune response to pregnancy, decreases contractility of uterine smooth muscle and inhibits lactation during pregnancy.  With prolactin, progesterone prepares breast tissue for milk production after childbirth.  Drop in progesterone levels during pregnancy is thought to be a key step in induction of labor.  Progesterone also has a variety of other regulatory effects, though the exact nature of these functions is not entirely clear.
Progesterone receptors on cells can be increased by the action of estrogen.  Furthermore, the activity of progesterone is amplified by the presence of estrogen.
The importance of mast cells in reproductive biology has been known for over sixty years.  Mast cells express receptors for both estrogen and progesterone.  These hormones together attract mast cells from the peripheral tissues to the uterus.  Furthermore, they induce the maturation of mast cells and directly cause degranulation in a dose dependent manner.  Together, they induce more degranulation than individually.
During pregnancy, embryo-derived histamine releasing factor induces secretion of histamine by uterine mast cells.  Histamine is also secreted by endothelial and decidual cells.  Mast cells have a protective role in ensuring successful embryo implantation.  Mast cells also positively influence the growth of blood vessels and participate in tissue remodeling so that the pregnancy can be sustained through placental growth and adequate blood supply. Degranulation increases uterine contractility through histamine and serotonin action.  Allergic activation causes significant contractions. 
In placentas from intrauterine growth retardation, mast cell concentrations are significantly decreased.  When mast cell numbers are diminished, the cells formed following implantation are at different stages, and are smaller and delayed.  Pregnancies with this feature generally do not survive.
In some cases, severe allergic reactions are thought to be responsible for preterm labor.  Additionally, degranulation in pre-eclampsic patients caused increased vascular resistance, likely from vasoconstriction by histamine.  Asthmatic pregnant women are known to be at a higher risk of pre-eclampsia.  People with other mast cell diseases should likewise by monitored for this condition.
Estrogen and progesterone levels can be correlated to symptoms in asthma.  Postmenopausal women taking hormone replacement therapy have a higher risk of new onset asthma.  30-40% women have asthma with more symptoms during the premenstrual period when estrogen and progesterone concentrations are dynamic.  Many women with mast cell disease likewise report more degranulation when menstruating.  Mast cell density in non-uterine tissues is much higher in pregnant woman, likely due to the higher hormone concentrations. 
A paper released in 2013 referenced a 2001 study by Metcalfe and Akin that found that women with SM were more likely to have preterm labor and delivery.  However, a 2011 study in Spain found that only 3/45 (6.7%) women delivered prematurely.  The rate of preterm birth in the general Spanish population is 7.4%.  It is unclear whether this change was due to increasing understanding of SM and more effective treatment, or due to the changes in diagnostic criteria between these studies.
The presence of mast cells is crucial for healthy pregnancy.  However, excessive activation can cause contractions and increased symptoms for pre-eclampsia patients.  The most recent study demonstrates that overwhelmingly, women with SM deliver healthy babies at the appropriate time.

 

References:
Woidacki, K., Jensen, F., Metz, Zenclussen, A. (2013). Mast cells as novel mediators of reproductive processes. Front. Immunol. 10.
Woidacki, K., Popovic, M., Metz, M., Schumacher, A., Linzke, N., Teles, A., et al. (2013). Mast cells rescue implantation defects caused by c-kit deficiency. Cell Death Dis.4, e462.
Metcalfe, D. D., and Akin, C. (2001). Mastocytosis: molecular mechanisms and clinical disease heterogeneity. Leuk. Res. 25, 577–582.
Jensen F, Woudwyk M, Teles A, Woidacki K, Taran F, Costa S et al. (2010). Estradiol and progesterone regulate the migration of mast cells from the periphery to the uterus and induce their maturation and degranulation. PLoS One 2010; 5: e14409.
Matito, A., et al.  (2011.) Clinical impact of pregnancy in mastocytosis: A study of the Spanish network on mastocytosis (REMA) in 45 cases.  Int Arch Allergy Immunol; 156: 104-111.

Just a horse

This past spring, I started developing a rash on my back.  It was macular and itchy and swelled and turned red when I touched it. 
“It looks like urticaria pigmentosa except it’s not dark,” one doctor told me.  It’s important to note here that my rash pigmentation has been unusual for my entire life.  I have, on several occasions, had rashes misdiagnosed because they were “too pink” or “too faint.”  I don’t know the reason for this, but it happens.  “I’m sure it’s some kind of mast cell issue, your skin is very reactive,” he followed up.
“That is definitely urticaria pigmentosa,” another doctor told me.  He touched the spots and they puffed up and got itchy.  “See, it has a positive Darier’s sign.”  My skin will urticate will very little provocation so I was not convinced.  But I figured I was probably bound to have UP eventually, so I wasn’t very concerned.
“It is probably a mast cell rash, but you should get it biopsied just in case,” a third doctor told me.  By this point, the rash was all over my back and shoulders.  It was itchy, but not all the time.  I scheduled an appointment with a dermatologist. 
I saw the dermatologist on Thursday.  She took one look at it and said, “Oh, that’s not cutaneous mastocytosis.  That’s a harmless fungal rash.  It’s more common in people who are immunosuppressed.  I’ll give you a cream.”
We had a good laugh over the fact that when you have a rare disease, everyone assumes it is the cause of all your symptoms.  She told me a funny story about a patient with several rare diseases who had a “mysterious rash” that the residents couldn’t identify.  It was tinea versicolor, a very common fungal rash.  The residents had assumed it was something exotic and had not considered more mundane options.
Then there was a small fire in the building while I was dressed only in a gown, educating the visiting PCP about systemic mast cell symptoms from skin reactions.  I threw my clothes on and ran outside as the fire department arrived.  Always lively. 
Mast cell disease is hard to manage in part because it can cause so many problems.  But just because it can cause all of them doesn’t mean it does. 
Mast cell patients are zebras, often many times over.  But even zebras mingle with horses once in a while.

Inconquerable

I scanned Astoria’s medical records today to send them to my vet.  As I was removing the papers from the scanner, I caught the name of her previous owner.  For some reason I can’t really explain, I decided to look this woman up. 
I saw pictures of Astoria as a puppy, with captions that clarified some of her history.  She was indeed rescued from a Southern shelter as a very young puppy.  Story lived with this woman until the spring of this year, when she felt she could no longer keep the dog and planned to give her to a shelter.  Astoria’s trainer offered to keep her until a suitable owner was found.  I brought her home last month.  I am in her fourth home in two years.
Every time I go out, Story thinks I am never coming back.  She hangs her head and follows me around when she can tell I am leaving.  After I’m gone, she pulls down the covers on my bed and lay in my spot.  If I’m home and there is a closed door between us, she paws at the door and whines. She wants to be close to me all the time.  Every time I come home, her body vibrates with the intensity of her relief.  It is strange for me to think that she is still wondering why this other woman never came back. 
She is triggered by odd things and it frustrates me that there is this history I don’t know.  She lives around these limitations, but she is still scared.  She does not believe that I will return until I do.  I think she will eventually trust that I am her person forever and I will always come back.  I think someday her past will not hamper her spirit.
My health has been bad for so long that it started to feel like my body didn’t have any good days left in it.  I woke up this morning typically nauseous and sore.  I took a handful of pills with my coffee before disconnecting my overnight infusion from my port.  I knocked all my makeup off its shelf and was gearing up to fight through another difficult day.
As I was walking out the door, I realized I wasn’t nauseous anymore.  It was a cool and breezy morning and it smelled like frost.  I went to work and had a really pleasant and productive morning.  I had forgotten my lunch so I took a chance on a salad from a restaurant down the street.  I ate it at my desk in case I needed IV Benadryl.  But I had no reaction.  I felt totally fine.
I worked a full day and got a lot done.  I was able to share with my coworkers the success of a child on midostaurin, a Novartis clinical trial drug for Aggressive Systemic Mastocytosis.  They were so excited to hear the personal impact of one of our drugs.  After work, I got a Pumpkin Spice Latte at Starbucks and took the train home.  My train was delayed for twenty minutes in a train tunnel for some reason and I didn’t even care.  I didn’t have any bone pain or joint pain.  I had no headache.  I wasn’t short of breath.  I wasn’t flushing.  I wasn’t bowel obstructed.  I wasn’t nauseous and I hadn’t thrown up all day.  I was still a little sore from the port being placed but it wasn’t bad.  I walked between stations rather than switch lines, drinking coffee as I navigated the sunny Boston streets.  It has been a long time since I walked around my city without having to sit down every few blocks.
When I came home, I found that Story had unrolled my yoga mat and was literally doing upward facing dog on it, so I thought, why don’t I do some yoga?  So I unrolled my other mat (because Story was still laying on the other) and did a good 40 minutes of yoga for the first time in a long while.  I brought her out to the yard to play with Harry while my friend came over to give me a massage.  And of course, that was super pleasant and I felt very relaxed when she was done.
I took Harry for his usual ten minute walk around the school yard and as the first stars were coming out, I walked down to the beach with Astoria.  For once, she wasn’t pulling me all over the place and was happily killing a stick as we walked.  We walked the length of the beach in the chilly night air and when we got home three miles later, I felt tired but otherwise fine.  I drank my can of Coke while reading a paper on mast cell biology.  I took a hot shower, which has recently become a lot more pleasant due to no longer having to cover the PICC line.  When I face into the water, I just hold a face cloth over the accessed port.  When I’m done, I just wipe it off with a towel.  It is seriously the greatest.
I took my night pills and opened Netflix on my computer after crawling into bed.  As I looked through new arrivals, I saw a documentary I have been wanting to see.  I put it on and pulled out my other laptop to write this as I snuggle under my heated blanket.
It is now after midnight, so I’m no longer worried that I will jinx it by saying: I had a perfect day.  I somehow, after all this time, and all this pain, had a day where mast cell disease did not affect my life at all.  I had a day with a really great Cobb Salad and yoga and a walk down the beach and a massage and getting to remind my very hard working colleagues on behalf of the mast cell community that what they do can save lives.  I am optimist because I don’t think there’s any other way worth being, but I have to admit that I was doubting whether or not I would see a day like this again.  Sometimes you get what you need. 
Someday Astoria will have a perfect day, too.   Someone will bounce a ball off the ground for her for hours so she can catch it and then a dog will play chase with her somewhere where she can roll around in the dirt.  She will get to pull all the stuffing out of a stuffed crocodile.  Then she will eat several cookies, and when I go to work, she will know that I will come home. 
We both live with these damaged spirits, but I’m starting to believe that maybe they don’t have to be damaged forever.  Because when I have a day like this, I realize it’s still there, as full and as vibrant as it ever was, and I know at once that it is inconquerable. 

Fat

Once I hit puberty, I was a fat girl.  I wasn’t morbidly obese, but I was overweight and it was obvious on my short frame.  This was not something I hated about myself, and it wasn’t until college that I felt uncomfortable with my body, but it informed my later years.  The experience of being overweight (and therefore mocked/ridiculed/generally viewed as “unfortunate” or “unseemly” or “lazy”) has affected my ongoing relationship with myself.
In 2007, I lost 40 lbs by training for the Breast Cancer 3-Day.  I also lived alone and worked a lot, on my feet.  I was committed to training, but also still had the privilege of a largely functioning body.  I was tired and had some joint issues, but it was more occasionally annoying than anything else.  Most importantly, I had time and stamina.  I could walk 10 miles a day, in the sun, in the heat, without any fallout. 
My weight fluctuated a little bit for the next few years, until in 2009, when I lost my hearing.  My neurotologist wrote out a long, high dose steroid taper and within a month, I had gained over 20 lbs.  In 2012, after a serious effort (working out 6-8 hours a week for several months), I lost 10 of those pounds. 
This was the point at which I realized that there was some fuckery afoot with my weight.  Like no matter what I ate, or how much I exercised, my body would not lose any more weight, and especially not around my swollen midsection.  A few months later, I had my ostomy surgery and in the weeks after that, I lost 10 more pounds.  The swelling and squishiness was gone.  The proof was in the pudding.  My mast cell disease and its subsequent inflammation were keeping me swollen, and squishy, and fat. 
Fast forward several months and a prescription for high dose steroids was being slid across the desk to me.  “I don’t want to do this again,” I started, but I knew I basically had no other play.  So I took them.  And two months later, I had gained thirty pounds. 
I am still on steroids; very low dose, but still on them.  As I have stepped down the steroids, I have lost some weight, but I am still 20 lbs over where I was.  I walk a lot (10-15 miles a week), and do yoga as I’m able, but my body has taken a serious beating this year.  I got a PICC line placed in March, which meant no weight bearing with that arm, and that eliminated most strenuous forms of exercise I can safely do.  I can’t do cardio.  I couldn’t swim with the PICC.  Now I have a port, and I can’t do any exercise for at least five days.  I’m forever being told not to exert myself while also being reminded that being overweight causes me a lot of problems. 
I have almost no control over the way my body looks.  I don’t mind having a colostomy and a port, I really don’t.  But I do mind that being overweight means that people judge me for being “lazy” or “unhealthy” or “making bad choices.”  I don’t know why anyone would ever comment on a person’s diet or general fitness, but it happens to me, so I’m sure it happens to you.  People are always like, “Oh, anyone can do [insert name of cliché fitness trend],” or “Your problem is that you drink soda,” or whatever. 
Are you kidding me, people?
Are you fucking kidding me?
I think my problem is that I have a rare, severe, life threatening disease that is destroying my body.  I think that’s my problem.
I cannot eat your stupid diet food because it’s full of artificial sweeteners and garbage.
I cannot do cardio because it will cause me anaphylax. 
I cannot do most other types of exercise because my body fucking sucks and has failed me repeatedly.  And the fact that it is fat is the least of the ways it has failed me. 
I throw up a lot of what I eat.
I drink one can of Coke a day.  I will probably do this every day until I die.  And you know what?  That’s 140 calories my body needs, because while you’re thinking about how much less I should be eating, I am not getting the amount of calories or vitamins or minerals that my body needs.  And frankly, for all the shit I have to put up with on a daily basis, a can of Coke is the least of what I deserve.
I don’t like being inactive.  I don’t like lying in bed and needing to sit frequently.  I don’t like feeling weak. 
There are some days when I look in the mirror and think that can’t be me.  I am so tired of living in this shell that doesn’t even look like me.