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August 2014

Okay or better

A couple of years ago, I found out a piece of really disturbing information that I felt I should share with a good friend of mine.  I called him and told him I needed to talk to him.  He met up with me right away.  I told him and afterward, he said, “All of this is weird, but I’m not gonna lie, I thought you were gonna tell me you were dying.”  We laughed about it and I reassured him I wasn’t dying. 

One of the hardest parts of being sick is that the people who love you worry about you all the time.  My relatives call me to check up on me a lot.  My parents walk down to my apartment if they haven’t heard from me all day.  My friends ask if they can help with me anything because they feel like they should be doing something.
I feel really guilty about this.  I know that my disease doesn’t happen in a vacuum, that everyone close to me is affected.  I wish that they weren’t, but I understand.  When they are struggling, I worry about them, too.
Part of why I feel guilty that they worry is because I feel it’s unnecessary.  I am well equipped to live with mast cell disease.  I have excellent medical care from experts in my disease, I understand my disease, I have a strong support system that allows me to safely live independently and work full time.  I have a lot of things working for me that many people don’t.
But even more than these things, I just know that I’ll be fine.  I don’t know how I know, but I have the most distinct feeling that it’s all going to work out.  On a cosmic level.  I feel like all this pain is temporary.    This feeling is not based on science or test results.  It is deeper than that, more primal.  It is true.  I know it is true. 
I know I’m sick, I know anaphylaxis is dangerous, and so on.  I know all these things the way I know anything else scientifically – because I can review data and draw that conclusion.  But this feeling is stronger than that.  I think it’s the reason I am generally happy – because it seems silly to worry about this when it will all be okay.  It’s how I know things won’t be hard forever. 
So all you people who love me – try not to worry so much.  If you need to worry, I will tell you.  My life is difficult right now but it’s all going to sort itself out.  I believe it.  So should you.
“I’ll be okay.  Okay, or better.  It’s like my guarantee.”  -Buffy Summers, Buffy the Vampire Slayer

Rolling the hard six

The day before I went to Seattle, I saw my mast cell GI specialist.  I was drinking coffee when I arrived.  No matter what I put into my mouth, I get nauseous.  It’s not as severe with liquids, but it still happens.  I throw up a lot, and I know that it is due to my mast cell disease because I discovered that if I have taken IV Benadryl in the hour before eating that I don’t get nauseous.  But I can’t take IV Benadryl before every meal.  It’s not sustainable and I need to reserve this option as a rescue med.   

I am very transparent with my doctors.  I call, text and email them often.  My mast cell GI specialist is aware that I vomit so often that my throat bleeds.  I asked him at what point I need to be concerned about the blood.
“If you throw up enough blood to fill up a cup like that, you need to be concerned,” he answered casually as he made notes.  I have become someone who throws up blood often enough that it is not concerning. 
The lower GI fallout from eating is becoming more and more significant as well.  I have always had heavy GI involvement and my digestive systems sustains more and more damage with each passing day.  It was only a matter of time for me.
I keep eating, even though I know it will make me sick.  I don’t want to end up on TPN but I realize it is becoming a likelier option.  I’m just stubborn in this way.  I want to eat, even though it’s obvious that my body can’t. 
I am probably consuming less than 1000 calories a day most days, and of those, I am often throwing some of it back up.  I can generally keep down liquids, so I rely on those calories.  I spent part of my evening looking up elemental formulas online.  I do not want to be living this life sometimes.  I do not want to be a 30 year old woman who drinks formula.
I saw my immunologist a couple of weeks ago and laid it all on the table.  “It’s one thing to be a 30 year old woman who sleeps through days and shits into a bag.  It’s another thing to be a 30 year old woman who sleeps through days and shits into a bag and also can’t eat anything without throwing up.  That’s where the line is.  That’s my line, right there.”  I pointed to my proverbial line, stretching out between us. 
“Yea, I think it’s time to do something drastic,” he said, and I agreed.  We had a lengthy conversation about the molecular mechanisms, benefits and risks of various meds, monoclonal antibodies and chemo drugs.  We agreed to respectively reach out to the experts we had available to us and formulate a plan.  We have not yet reconvened, but the plan will assuredly involve some type of medication with very serious risks.  It will probably involve more lower GI surgery. 
Of all the qualities that have prepared me to live with rare diseases, this is the one I find most valuable – the ability to not blink.  I make the hard decisions with little hesitation and I don’t regret them.  If I were given a choice between living ten years and spending five in bed in massive pain, or living seven and living my life until the end, I would take the seven.   Without blinking.
I don’t know if being aggressive with healthcare is the better way, but I know that for me, it has always been the only way.  Some things are worth fighting for.  Even if you know you will lose, sometimes it’s worth it to fight.  I have to know that I tried everything. 
I have to live in this body every day.  I have to know that I did everything I could to salvage a life. 
We all have to make choices we can live with. 
So I make these choices.  And I don’t blink.

Losing time

Stories about time travel have always fascinated me.  I read my first one in grade school and was both intrigued and horrified by the implications.  You could go back and fix mistakes, but sometimes those mistakes shape who you are.  Everything you do matters.  Change one thing and you change everything.

I have always tried to do more than I was able to in any window of time.  When I was in college, I worked full time, did lab work for my senior thesis and took a full course load.  When I realized that I didn’t have enough time to do all of this, I stopped sleeping on Tuesdays and Thursdays.  I wasn’t satisfied with the amount I could do in the time I was given, so I stole some extra time.

When I interview for jobs, people are always confused.  “The dates on your resume are wrong,” is one I get a lot.  They’re not.  I really did work two full time jobs in grad school at the beginning of my illness.  I just needed to do all these things at once so I sacrificed sleep and time off.  It has taken its toll, but I don’t regret the decisions I made.  I had to get through school and I had to support myself.  Necessity is a powerful motivator.

In the weeks before I was diagnosed, I would sleep through entire days.  I would not wake to eat, drink, pee, or take medication.  I sometimes could not be woken, and if I was, I was very disoriented and confused.  My disease had strained my body to the point that it needed huge amounts of sleep to function.  I would sleep for 22 hours, then be awake for 36-48 hours and do it all over again. 
Since about April, I have had this overpowering need to sleep all the time.  It had gone away for a while, but this was an obvious side effect of the steroids and you can’t take steroids forever.  I have to be woken up every day in order to take my morning medications or I will wake up and anaphylax.  When I go to sleep, I never know when I will wake up.  I have numerous alarms, including a deaf alarm that shakes the bed, but when I am in these “mast cell comas,” it makes no difference.  I need a person to come in and wake me up.
Last week was quite a week for me.  I knew there would be fallout, and I was not wrong.  I have been awake for less than 36 hours in the last five days.  In the hours I am awake, I am uncomfortable and flushing and having GI issues.   I know it will equilibrate once I get through this.  We are changing some meds to try and address this issue.  But the feeling of losing is palpable. 
When you are young, you have this feeling that you can always do something later.  You procrastinate and reschedule things and there is no danger that you won’t eventually get to these things.  But then one day you wake up after 17 hours of sleep and you have all these things to do and you have to do them all right now because once you fall asleep, they might not get done.   I know that this is because of what happened last week.  I know my body is trying to recover from the stress.  But I can’t help but feel like I’m losing time, and that feeling is overwhelming and sad. 
There’s no time anywhere for me to steal.  I read this story by Harlan Ellison in high school about a society in which people only get an allotted amount of time for their entire lives.  This feels like that.  It feels like I manipulated the time continuum earlier in my life and because I did, I have to live with less now. 
Every time I wake up, I hear this buzzing in my head and I think, that’s what time sounds like and it’s catching up to me.

Pregnancy in mastocytosis

Mast cells are involved in regulating the female reproductive cycle.  There are direct correlations between the contraction of uterine wall and serum histamine during pregnancy, as well as correlations to other mast cell mediators.  Mast cell activation and degranulation in the endometrium occurs immediately before and during menses.  Many allergic conditions, including asthma and angioedema, are exascerbated during menses, which is thought to be due to mast cell activation.

Throughout pregnancy, sex hormone levels change.  Sex hormones, such as estrogen, can directly influence mast cell activation and degranulation.  Mastocytosis patients often discontinue antihistamine and antimediator medications during pregnancy due to safety concerns.  In 2011, a study was published examining the effect of pregnancy on mastocytosis patients and of mastocytosis on pregnancy and delivery.
During pregnancy, 45% had itching; 40% had flushing; 24% had GI symptoms; and 9% had anaphylaxis.  22% of patients reported worsening symptoms throughout the pregnancy.  2% felt their symptoms were more frequent, while 18% developed new symptoms, and 2% had both new and more frequent symptoms.  New symptoms generally appeared in the first trimester, occasionally in the third.  Worsening of symptoms occurred in 3/6 women with CM and 7/35 with ISM with skin involvement.  One woman developed skin lesions during the third trimester and was diagnosed with ISM via bone marrow biopsy after delivery.
33% of women reported their symptoms had improved during pregnancy.  15% had complete resolution of symptoms, 15% had at least one symptom disappear and 3% had at least one symptom disappear but others worsen.  All resolutions occurred during the first trimester and lasted throughout the pregnancy with the exception of one patient.  In patients who had idiopathic anaphylaxis before pregnancy, 50% of them had no anaphylaxis while pregnant.  In those women who did have anaphylaxis during pregnancy, it was resolved without the use of epinephrine and did not cause early labor or complications.
Complete resolution of symptoms occurred in a patient with well differentiated SM (WDSM), ½ patients with ISM and no skin involvement, 9% of patients with ISM with skin involvement and 17% of patients with CM.  Partial resolution occurred in ½ patients with ISM and no skin involvement, 11% of patients with ISM with skin involvement and 17% of patients with CM.  In 6% of patients with ISM with skin involvement, at least one symptom disappeared while others worsened. 
45% had no change in symptoms during pregnancy (19/35 ISM with skin involvement patients, 1/6 CM patients.)  One patient experienced significant improvement of skin lesions in the first trimester. 
For women who worsened during pregnancy, mast cell symptoms continued to be worse after delivery for 50% of them.  Symptom resolution observed during pregnancy continued after delivery for 4/7 cases that had complete resolution and 3/6 cases that had partial resolution.  Complete resolution of symptoms occurred in two patients that had partial resolution during pregnancy.  There were five cases of worsening skin lesions after delivery. 
78% of infants were delivered vaginally and 22% by Caesarean.  Nine deliveries were induced with oxytocin (8/9) or dinoprostone (1).  In 38% of cases, the patient took mast cell pre-meds at the onset of labor.  Anesthesia was used in 82% of cases, including epidural (32 cases), local (3) and general (2.)  11% of patients had mast cell attacks during or immediately after labor.  Anesthesia and medications used for labor seemed to be safe.  Only three women had mast cell reactions to epidural; of these, two had not take pre-meds.  Premedication at the initiation of labor is recommended.
3 out of 45 newborns were born premature and 4 out of 45 had low birth weights.  One had Down Syndrome; one had respiratory distress; one had jaundice; and one had heart rhythm abnormalities before birth.  There was no correlation between mother’s symptoms and outcome.  There was no correlation between anaphylaxis and outcome.  None of the children had skin lesions at birth, but one developed CM at the age of 5. 
The frequency of spontaneous pregnancy loss during first trimester (10-15), birth by Caesarean section (25%), prematurity (7.6%) and low birth weight (3-5.8%) were comparable to the rates in the general population.  Nonaggressive forms of mastocytosis did not appear to impact pregnancy outcome.
Matito, A., et al.  Clinical Impact of Pregnancy in Mastocytosis: A Study of the Spanish Network on Mastocytosis (REMA) in 45 Cases.  Int Arch Allergy Immunol 2011;156:104-111.

Keep going

In February of 2007, I went to a party my friend was having.  While there, I started talking to a woman I didn’t know.  She told me about having walked the Breast Cancer 3-Day, in which you fundraise to support breast cancer research, prevention and treatment, and then walk 60 miles over 3 days. I told her I could never do anything like that and ate some cheese and crackers.

Later on, I woke up in the middle of the night.  While walking to the bathroom with my eyes closed, I realized that my ass was jiggling.  I am laughing as I write this because it really did unfold like a movie shot – I realized my ass was so big that it had its own center of gravity and I immediately froze and opened my eyes, completely awake.  I decided right then to do the Breast Cancer 3-Day, not because I am a good person who wanted to fundraise (although I later became passionate about this), but because I wanted to have an ass that didn’t shock me into wakefulness. 
I did the training and lost a lot of weight.  Like 40 lbs or something.  I remember closing my medicine cabinet and looking into the mirror and realizing I didn’t have a double chin anymore.  I got smaller and healthier and most of all, I felt stronger and more able.  I got in shape.  I walked 10-12 miles at a time in the sun and heat.  I did everything I was supposed to.
The weekend of the Boston 3-Day was blistering, and so hot and humid that they almost called it off.  I have since walked four other 3-Days, some in other cities, one other in Boston, and it was by the far the worst weather I encountered (including the massive freak rain storm in San Diego.)  I put on sunblock and stretched and got walking.  At the end of the first day, I couldn’t straighten out one of my legs and I was sore everywhere and caked with dirt.  I didn’t think I would be able to walk the next day.
But the next morning, I woke up and my leg straightened.  I was sore but I could walk.  So I did.  I walked slowly.  I was one of the first to leave camp and the last to get back to camp that day.  On the third day, the shortest and easiest, with milder weather, my feet were such a mess that I literally wrapped them in ace bandages and walked the last few miles in flip flops.  They hurt so bad.  But it seemed such a waste to not finishing after walking 55 miles in 3 days.  So I did it.  I did it really slowly, and it hurt, but I did it.
It is funny to think that this event, that I undertook to achieve a smaller ass, changed me as a person. My mind wants to smooth over the suffering and forget how hard it was, give the memory a glossy touch.  But I never let it.  My strength is in how far I have come.  My strength is in still going even when it seems hopeless.
In December of 2009, when I was struggling to understand speech, one of my best friends grabbed my face and said very loudly, “I will learn ASL because I can’t not talk to you.”  And so I kept going.
In the winter of 2011, when I was numb from the pain and stress and exhaustion, my doctor said, “I want you to see one more doctor, and I think he will know what’s wrong with you.”  And so I kept going.
When I had a lengthy discussion with my doctor a couple of weeks ago about the virtues of chemo for the treatment of systemic mast cell disease, one of my best friends said, “Tell me when you’ll be at the hospital and I’ll come see you at the infusion center.”  And so I kept going.
I have posted about my friend with ASM whose doctors told him he was out of treatment options.  His doctors had applied for compassionate use of a clinical trial drug for him and it had been rejected, twice.   I told him, “Your doctors are doing something wrong, you are the exact type of person who should be on this drug.”  He told me it was over and he wasn’t going to get the drug.  I told him not to stop.  I told him to keep bothering them.  I told him that I believed this could be fixed and that it would get sorted out. 
Last week, he found out that the company approved him for compassionate use of this drug and he will start treatment next week.  And so he can keep going. 
I had no way of knowing that he would get the drug.  I just thought there was a chance and sometimes that’s all you have to hold onto.  Sometimes you just have to find anything that might work and bang on every door until someone gives you a chance to prove that it won’t.  And maybe it won’t work.  But at least he’ll get to find out. 
My mind recoils when I think back to the months before I was diagnosed.  I would never go back to that, and that means that I must have made progress.  It was hard then.  Every day was so hard.  But I kept going, and I came all this way.
I don’t know how this is going to play out.  I don’t know where I’ll end up. 
But I know it’s not here.
So I keep going.

Unqualified success

Everyone knows about my very stressful week that resulted in huge doses of extra meds, including steroids, and multiple doses of epi to keep me safe.  So by the time Thursday rolled around, I was seriously hurting, and not in a metaphorical way.  I was literally hurting and exhausted and my skin hurt and my GI tract was bleeding and being generally obnoxious. I was hoping that I would make it through this wedding but I was really not sure it was going to happen.

Thursday night was the rehearsal dinner.  I put on my best rockabilly dress and infused on the way over to the church.  I stood outside the stone church and enjoyed the cool weather and soft breeze.  We went into the church and practiced walking down the aisle.  (I consider myself an expert walker.)  I took steroids and went to the dinner.  I got home at a reasonable time but the steroids guaranteed I couldn’t sleep.  I fell asleep sometime around 4:30.


We left at 8am to drive over to the hotel to get ready.  I took a ton of extra meds and hooked up a fluid infusion to try and stay hydrated.  The stylists made my hair very beautiful while discussing my obvious health issues in French.  (Surprise!  My spoken French is bad, my understood French is pretty good.)  And then suddenly it was time to put on my dress and go to the church.  I was mostly functioning on adrenalin at this point (natural, not Epipen) and very grateful for that. 
We went to the church, walked down the aisle and my cousin married his wonderful, kind bride, who is one of my favorite people.  It went off without a hitch and was amazing.  We took pictures and climbed into the limo to head to the reception, where we took more pictures.  Then we had a huge party that I will remember forever as one of the best parties I have ever been to.
The wedding was at 2, the reception started at 5 and by 7, I felt like I was going to pass out.  My body was starting to seriously lose it.  I was having low level anaphylaxis and feeling generally miserable.  I really wanted to go home and go to bed.
But I wanted to be at my cousin’s wedding more so I rallied and danced and sang loudly and then went out to the firepits and toasted marshmallows and made smores.  (Side note: everyone thinks they can dance at weddings until they go to a dance where many of the guests are professional dancers.)  By the time it was time to leave, I had to be helped into the car and my friends had to literally help me out of my dress at home.  I was in that much pain.


I write a lot about how frustrating my disease is and how people often insult me or make my life difficult with their actions.  This is not one of those stories.  I am regularly overwhelmed by the kindness I am shown by people, even people who don’t know me well.  That has never been more obvious than yesterday, when I needed all the help and got it.
These kindnesses include things like: my cousin’s sweet friend doing my make up when I was running short on time from screwing around with my PICC line; the groomsmen helping me in and out of the limo; everyone making sure I never had anything heavy to carry; everyone asking “Can I help you with anything?” pretty much anytime they saw me; the groomsman who escorted me down the aisle being careful with my PICC line; my best friend telling me during dinner that he was ready to take me home as soon as I wanted to go; people getting me drinks so I didn’t have to stand up unnecessarily; the bride running over to me because she heard I wasn’t feeling well and might go home; and lots of other very small things that mean so much to me.
This week has been a disaster on several fronts.  But yesterday was an unqualified success, and it was only possible because of the help of many people.  People say to me sometimes that they wish they could do more to help me, and I appreciate that.  But these little things – the carrying of my meds or patience in giving me five extra minutes to get ready – these are just as important.  I am blessed to have people in my life who do these things for me. I am also blessed to discover over and over again that most people are generally decent and willing to help.  And when I’m around people like that, it becomes a little bit easier to have mast cell disease and I feel a little less like a burden. 
Congrats, Matt and Jacqui!  Thanks for the memories, it was amazing.


Histamine effects on neurotransmitters (serotonin, dopamine and norepinephrine)

Some of the most important actions of histamine involve regulation of neurotransmitters.  Release of acetylcholine, norepinephrine and serotonin are all controlled in part by histamine levels.  Injection of histamine into the hypothalamus increased metabolism of norepinephrine and serotonin, while dopamine metabolism increased in some places and not in others.  Medications that block the H1 receptor increase dopamine release.  Histamine stimulates prolactin release via the H2 receptor, which in turn inhibits dopamine production.  Histamine can locally increase the concentration of norepinephrine.

Serotonin is a neurotransmitter.  This means that cells nerve cells use this to communicate.  Most of the serotonin in the body is found in the GI tract, where it controls the way the intestine moves food through it.  However, one study indicated that as much as 40% of serotonin in the human body could originate in mast cells.  Serotonin is metabolized to 5-HIAA, which can be tested for as a sign of mast cell activation.
Serotonin released in the GI tract eventually enters the blood stream. On its way to the blood stream, it is taken up by platelets and later used in clotting.   Serotonin is released when eating, which decreases dopamine release and decreases appetite.  If the food consumed is irritating to the GI tract, more serotonin is secreted to move it through the gut faster.  In these situations, the serotonin cannot be fully taken up by platelets and enters the blood stream as free serotonin.  When this happens, it stimulates vomiting.  Some foods contain serotonin, but it does not cross the blood brain barrier and thus does not affect brain chemistry. 
Mast cells contain dopamine, a hormone and neurotransmitter.  This chemical is most often associated with reward seeking behavior, including addiction behaviors.  It also has other important roles, including motor functions.  Mast cell activation causes depletion of dopamine as frequent degranulation causes a decrease in dopamine production by these cells.   Dopamine can be converted to norepinephrine.
In blood vessels, dopamine inhibits norepinephrine release and acts as vasodilator.  Dopamine also increases sodium excretion and urine output, reduces insulin production, reduces GI motility, protects intestinal mucosa and reduces activity of lymphocytes.  It is responsible for cognitive alertness.  If you consider that high histamine levels can decrease dopamine levels, this means that in a mast cell patient, low dopamine levels might cause decreased urine output, increased GI motility and overactivation of white blood cells.  Additionally, low dopamine can translate into higher than normal norepinephrine levels, which could be the link between mast cell disease and POTS.  Brain fog and decreased alertness are effects of low dopamine.
Defective transmission of dopamine is also found in painful conditions like fibromyalgia and restless legs syndrome, associated with mast cell disease.  Activation of D2 dopamine receptors causes nausea and vomiting.  Metoclopramide is a D2 inhibitor and achieves its anti-nausea effects through this mechanism. (Note: metoclopramide can inhibit histamine metabolism and for this reason is not recommended for mast cell patients.)  Some dopaminergic drugs like clozapine, bromocriptine and haloperidol inhibit mast cell degranulation.
Norepinephrine is responsible for concentration and vigilance.  It also increases vascular tone by action on alpha adrenergic receptors.  Norepinephrine is important in the fight or flight response, directly increasing heart rate, triggering release of glucose, increasing blood flow to skeletal muscle and increasing brain oxygen supply.  Interestingly, fasting increases norepinephrine for days.  Glucose intake, but not carbohydrate or protein intake, also increases norepinephrine.  Increased histamine can cause increases in norepinephrine production and secretion.