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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.
Reference:
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.

Beautiful things I can’t have

I’m a Buffy fan girl.  I’m sure this surprises no one.  Strong characters, the supernatural and witty banter is basically a recipe to get me as a loyal fan.  I know all the words to the sing along episode and have been compared to Willow more times than I can count. 

There’s an episode in the fifth season where Xander and Anya go to look at an apartment.  She is upset because she doesn’t think they’ll get it.  Xander asks her what’s wrong and she says this:
“What’s going on with me is that my arm hurts… and I’m tired… and I don’t really feel like taking a tour of beautiful things I can’t have.” 
That’s how I feel right now.  I don’t really feel like looking at beautiful things I can’t have.
I have always identified as a traveler.  As a kid, I would take out language books from the library with the clear intention of learning the language for when I went there.  When I was 19, it occurred to me that if I saved up my money, I could go wherever I wanted.  I got some books and planned a trip and went to Europe. 
In 2007, when I was starting to get sick, I went backpacking in Scandinavia.  I went hiking and got really lost in this beautiful national park in Norway.  I got drunk on a ferry sailing between Estonia and Finland.  I felt tired and at the end, I felt sick, but I was mostly able to do everything I wanted to do.
In 2009, I bought tickets to Ireland and went for a quick four day trip with my boyfriend at the time.  I was exhausted and sick, but I was still able to go.  I needed a lot of sleep but otherwise it was fine.
I took a bunch of other trips.  In 2012, I went to Seattle and we all know how that went.  I came home to lots of serious news and slowly I stopped thinking of myself as a traveler because it hurt a lot.  I stopped reading my dozens of travel books and drawing maps and studying languages.  Doing those things felt like sticking my hand into an open wound.  It felt like trying to push through the looking glass only to find I was still in this strange facsimile of my life where I was sick and couldn’t do this thing that defined me.
In the last couple of weeks, I started feeling like maybe this was going to be possible again.  Maybe I could travel, if I had someone with me to help me.  Maybe I could plan trips again and look forward to them.  Maybe I could go to all these faraway places and feel this stillness in my soul that I only find when I’m away from home. 
I was supposed to go to Asia in the fall.  I was originally supposed to go in the spring but I was too sick.  So we decided to go in the fall.  We were going to fly through Dubai to Bangkok, spend a few days in Bangkok, take a private tour to Angkor Wat in Cambodia, go to an elephant preserve, spend a few days in Dubai on the way back.  I have been planning this for months, both the fun part and the not insignificant logistical shuffle of travelling out of the country with a ton of medication, medical supplies and large bags of IV solutions.  I was planning to book our airfare tomorrow.
But a few days ago I found that one of my best friends was stealing from me, and she was supposed to go to Asia with me.  She was supposed to help me to live this dream that has buried for years under the rubble of mast cell disease. 
I’m more upset about Asia than I am about the money.  Because I found out a couple of weeks ago that I could still travel, but only if I’m with someone who loves me and understands my disease and what to do in an emergency.  I cannot travel alone.  I wish I could, but I can’t deny that it’s too dangerous for me. 
The fallout of this woman stealing from me has been significant.  I have needed epinephrine and lots of IV meds and steroids, and even now as I type this, my skin is burning and my heart is racing and my insides are doing that cringe and shudder that means that with just a little push, I can be in trouble again.
When you have mast cell disease and people mistreat you, your choices are to move on quickly so as not to anaphylax or to get mad and risk ending up in the hospital.
It’s not as much of a choice as you might think.
If I fall down and scrape my leg, it is an emergency.
If I forget to take a single dose of any of my many medications, it is an emergency.
If I forget to put on the AC and fall asleep, it is an emergency.
If I get too mad, it is an emergency.
Around 4 this afternoon, I locked a syringe of 50 mg IV Benadryl into my PICC line and angrily uncapped my Epipen.  “I really hate that I’m so sick that I have to use epinephrine at home on a regular basis.  That makes me really mad,” I told my mother as I brought the tip down against the outside of my thigh.
There seems to be some confusion over whether or not what my “friend” did was wrong because she “tried to pay it.”  (I know, don’t get me started.)  So let me make it really clear.
I don’t care about the money.  If she had asked, I would have given it to her.  This is not about the money.
This is about everything else she took from me.
She took my right to feel like I can trust people to take care of me.
She took my right to feel like I can accept help without getting screwed.
She took a lot of my energy over the course of our friendship.  A lot. 
She took my safety because this was such a violation that I can’t help but feel angry and it is making living in this body even more dangerous than usual.
She took my ability to go on this trip.  She took my ability to feel like I can get this part of myself back. 
So disappointing.  I have never been more disappointed in a person in my entire life. 
And for the sake of being thorough, I want to deal with this: at least one person thinks that it’s okay that they knew about it and didn’t tell me because she wasn’t “being malicious.” (I don’t even know what that means.)
What she did is criminal.  Stealing is a crime.  Stealing from a very sick person is a more serious crime.  This is not the personal opinion of Lisa Klimas.  This is the opinion of the criminal code of the State of Massachusetts.  If you know someone is preying upon an ill person, and you do nothing, you are just as guilty.  You are not a “good person” who is getting “blamed by association.”  You are a loser.    
My friends are closing ranks around me.  I have received over a dozen offers to do my food shopping, all with the added promise that they will not steal from me.  They are making me laugh.  (Quote of the day:  “’How does one pay a credit card without the bill?’ ‘Oh, I just write credit card on cash and throw it out the window, does that not work?’”) They are reassuring me that they will not betray me.  They are reassuring me that they will take care of me and that they love me.   They are letting me know that they will support me however this plays out.
They are really angry that this happened to me.  Honestly, I am a little surprised at how angry they are.  But I get it.
I’m tired of looking at beautiful things that I can’t have. 
And they’re tired of watching me. 
So disappointing.

On being vulnerable and trusting the wrong people

I like routines.  On Sunday nights, I take a shower, wash my hair, take down my ostomy, organize my pills for the week, pack my stuff for work, organize my dressing change supplies, put clean sheets on my bed and hook up my IV fluids.  I do it even if I know I won’t sleep because it relaxes me.

Last night, I was lying in bed infusing and cruising around the internet because I couldn’t sleep.  I needed to pay some bills so I pulled them up on my phone and started making payments.  When I got to my credit card, I was confused because the card was over the limit and there was no reason it should have been.
I pulled up recent transactions and there were several in the last few months.  I immediately got a sinking feeling in my stomach.  This spring when I started having a really rough go of life, one of my best friends offered to do my food shopping for me.  I gave her my credit card to pay for the groceries and haven’t seen the card since. 
I sent her a text asking if she had used my card to buy things and waited to hear that she had lost the card or something.  Nope, she admitted to using my card on multiple occasions without my permission.  She told me that she had sent in payments, none of which have been credited to my account.  So in the last four months, she has, on multiple occasions, used my credit card without my permission and tried to send in payments without my knowledge so that I wouldn’t find out. 
This is a person who has taken me to the hospital for appointments and taken care of me after surgery.  This is a person I chose to help me at a time when I was feeling unbelievably fragile and vulnerable.  I have known this person for almost twenty years.  And she stole from me. 
If she had some type of emergency and used my card, and had let me know about it, I wouldn’t have cared.  If she had asked me to borrow money, I would have given it to her, no questions asked.  Instead she has been stealing from me for months.  It’s really making me wonder about a lot of other things.
I can only assume that she was counting on my illness to not get caught.  She knew I was exhausted and distracted because of how sick I have been recently.  She knew that I trusted her and would keep sending in my regular payment without paying attention to my statement because I believed she was trustworthy.  She was right.  That’s exactly what I did.  If not for the fact that I couldn’t sleep last night, this might have gone on indefinitely. 
I was planning to go to Asia with her this fall, and felt very secure in that plan because of how much I trusted her.  I was really looking forward to going.  Now instead of booking our tickets for that trip (which I was planning to do on Wednesday!), I have spent the day on the phone with my credit card company and in a really uncomfortable state of stage 2 anaphylaxis.  I have needed a whole mess of extra meds today and it is just barely containing the reaction caused by all of this.  I’m accepting bets on the over/under for how much extra medication it takes in the next few days to get me stable. 
I have been very open about needing a lot of help to get through my days.  The people I have asked to help me are my very closest friends and family.  These are people I am not embarrassed to puke in front of.  These are people who I trust to help me into the shower.  These are people who have seen my stoma.  These are people who will rub my back when I am screaming in pain from a bowel obstruction.  These are people who make jokes with me about my illness and generally make me feel less alone. 
Finding out your friend is screwing you is bad enough, but sick people are especially at risk because we often have no choice but to trust people to help us.  And since I’d rather not need someone to help me do basic things, it is really humiliating and horrifying to discover that my trust was misplaced.  I have stood by this person through seriously hard times and supported them when no one else did.  I just really can’t believe it. 
I generally try to wrap up my posts elegantly, but I just can’t right now because seriously what the ever loving fuck. 

Mast cell disease and the spleen

Spleen is known mostly for having a stupid name, but it actually does a lot of really important things.  It is located in the upper left quadrant of the abdomen.  It is essentially a filter.  It removes old or damaged red blood cells.  It retains a supply of blood that can be used to regulate blood pressure in case of hemorrhage.  It also recycles and conserves iron by metabolizing hemoglobin removed from red blood cells filtered out.  The spleen also produces antibodies and filters out bacteria and blood cells that have been attacked by antibodies and marked for removal.  In this way, it is effectively a very large lymph node. 

Another very important function of the spleen is creation of blood cells.  In cases where the bone marrow is producing fewer blood cells than normal (like myelofibrosis), the spleen can actually compensate to produce red and white blood cells.  About ¼ of the body’s white cells are stored in the spleen at any time. 
Swelling of the spleen is called splenomegaly.  If a swollen spleen can be felt manually, it is at least twice its normal size.  The spleen usually swells when it is working harder than normal.  This can happen for several reasons. 
Swelling of the spleen often has no symptoms.  It can cause abdominal, chest or back pain and the feeling of having a “small stomach,” being able to eat less than normal in one sitting.  Anemia may also be present.  The most common reason is developed countries is mononucleosis, followed by infiltration by benign or neoplastic cells, or portal hypertension.  Bacterial infections like syphilis can also cause a swollen spleen.  In people whose bone marrow is not producing enough cells, the spleen can become swollen by the effort of producing extra cells. 
It is should be stated that swelling of the spleen is pretty common in blood disorders.  It is sometimes found in systemic mastocytosis.  As the spleen gets bigger, it grows beyond the borders of the protective rib cage and can be more easily damaged.  Swollen spleens present an increased risk of rupture. 
In SM, splenomegaly (swollen spleen) is NOT a C finding indicating aggressive disease.  It is a B finding.  This is because mast cells often infiltrate the spleen, causing swelling, but not necessarily impairing function.  Hypersplenism with splenomegaly is a C finding.  Hypersplenism is when the spleen is working too hard. 
Hypersplenism is indicated by swelling of the spleen, reduction in circulating red blood cells, platelets or granulocytes (neutrophils, eosinophils, basophils or mast cells), increased proliferation by the bone marrow to compensate for the decrease in circulating cells, and probable resolution of these symptoms by removal of the spleen.    Hypersplenism can cause additional complications, including portal hypertension and ascites.
Portal hypertension is increased pressure in the vein that connects the GI tract and spleen to the liver.  Ascites is free fluid in the abdomen.  Both portal hypertension and ascites are also mastocytosis C findings.  Removal of the spleen can decrease the patient’s risk of portal hypertension and ascites, thus improving prognosis.  Additionally, in the context of mast cell disease, an infiltrated organ is inflamed, which is in turn activating to mast cells.  Removal of a source of inflammation can cause relief of systemic activation.
People living without a spleen are more susceptible to certain types of infections, including H. influenzae, S. pneumoniae, and Meningococcus spp.  They should receive vaccinations for these organisms and also regular flu vaccinations.  In some cases, long term prophylactic antibiotics are needed.  Often, patients without a spleen develop a stable high white blood cell or platelet count as their numbers are not regulated by the spleen. 

Your top five problems

I had a conversation with one of my friends many years ago about kids worrying about things.  I don’t know why I remember this so vividly, and I don’t remember why we were talking about it.  I remember him saying, “I think no matter who you are in life, you have your top five problems.  And you worry the same way about those problems, no matter what they are.  So if a kid is worried about a spelling test and you’re worried about losing your house, the amount you each worry is the same.”  I started assuming that everyone was worrying about something and even if it seemed insignificant to me, it was important to them.  It has made it much easier for me to be kind to people who frustrate me.

I remember this conversation when I observe interactions in the mast cell community, which largely happen online.  Communities like this have some weird dynamics.  Sociologically, it’s interesting; as someone occupying one of these microcosms, it can be annoying.   Venting about your symptoms can create a weird situation.  Some people will relate to you and will say so.  Some people will relate to you, but feel guilty saying they relate because they feel like it will offend those with more severe disease.   Some people will think you’re whining and don’t have it that bad and should get over it. 
It is okay to vent.  You do not need to feel guilty if your symptoms are not as bad as someone else’s.  You do not need to feel guilty if you have support.  You do not need to feel guilty if you have good insurance.  You having these things does not change the realities for others who have worse symptoms or circumstances or whatever.  The strength of our community is that we understand each other and support each other.  In exchange for getting mast cell disease, you also get to complain about it sometimes to people who get it.
When I sleep, I move through my dreams in a whole, disease-free body.  I don’t have a colostomy or a central line or surgical scars.  I can hear and I never puke.  When I wake, I am often surprised to be in this body, this damaged and painful vessel.  I am forced to acknowledge and accept that this is real.  On some days, that’s really, really hard for me.  I wake up to pain and vomiting and I pull my blanket up over my face to be alone with my tears for a little while.
So when people say things like, “You need to get over it,” all I can think is: I’ll never get over this.  I live my life, and I like it, but I will never get over what this disease has done to me and my life.  I will never get over who I could have been if not for mast cell disease. 
Because I like the person I am, but the person I was trying to become – I liked her, too.  She’s who I think about when I’m under my covers on those mornings when I don’t want to believe that this is still real.  I think about all the extraordinary things she could have done, and how the only extraordinary thing I’ll do that day will be to get out of bed when I don’t think I can. 
You can’t get over something that is still happening to you.  You can only learn to live with it.