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.

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.

Housekeeping: Revising posts and MCAS month

In February of this year, I decided to post a fact about mast cell disease every day for Rare Diseases Month.  I honestly had no idea how much attention that would generate.  People asked if I had a blog, so I decided to start one.  I started posting on April 26 of this year.  Since then, this blog has had over 21,000 page views, about 1,000 a week, and has been read in over 50 countries.  I am really humbled that so many people find this helpful. 

When I started the blog, I transferred over all the posts I had done for Rare Diseases Month. These posts are very short, and comprise the posts on this blog dated before April 26.  I have noticed recently that some of those are being read frequently now, so I am going to go back and revise them to reflect the style of my later posts.  Once I do, these posts will be republished.  These include the Mixed Organic Brain post, symptoms posts, and a bunch of others.

Most of the posts I have done have been on mastocytosis, which is more well studied and documented.  But I don’t want the MCAS and MMAS kids to feel left out, which is why for the month of October, I will be doing daily posts about mast cell activation syndrome, with additional posts on monoclonal mast cell activation syndrome.  

As always, please feel free to email me at Lisa.Klimas@gmail.com if you have questions you would prefer to ask privately. Thanks so much for reading.  

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.

Gastrointestinal manifestations of SM: Part 2

In SM, the small intestine is sometimes normal when biopsied.  When comparing data across many studies, it is believed that at least 30% SM patients have small bowel structural abnormalities.
In one study, small nodules in the mucosa could be observed in the small intestine in 73% of patients.  It is thought that the small nodules (1 mm) represent focal edema (localized swelling) in superficial mucosa and intestinal villi, while the larger nodules are focal edema in the lamina propria. These nodules do not represent mast cell aggregates.  On endoscopy, biopsy of these lesions showed no aggregates.   
In 23% of patients, lesions show an indistinct jejunal mucosa pattern, probably from excessive secretions.  13% show a malabsorption pattern with flocculation and segmentation; irritability of the muscularis and circular muscle layer is likely responsible for jejunization of ileum in 18% of patients. 
In one study, 57% of patients had small intestinal mucosal thickening, nodularity and/or polypoid lesions.  In a second study, 29% had small bowel abnormalities, including 14% with jejunal or ileal nodules and 14% malabsorption pattern. 
However, a whole host of abnormalities are sometimes seen: thickened jejunal folds with edema; dilated small bowel; blunted villi; partial villous atrophy or edema; complete villous atrophy; infiltration by eosinophils and/or mast cells; spru like mucousal changes responding to gluten free diet; malabsorption with tetany; osteomalacia; vitamin A deficiency; mesenteric thickening or infiltration; bulls eye lesions.
A key aspect of small intestine disease in SM patients is malabsorption.  Previously thought to be rare, multiple studies have now shown that malabsorption is more common in SM and is due to small intestine defects.  Approximately 5-25% of SM have malabsorption, which is generally mild.  One study of SM patients found that 31% had impaired absorptive function.  This was determined by 72-hour fecal fat studies, D-xylose tolerance testing and Schilling test.  Pancreatic function is normal in all SM patients evaluated in these studies. 
An older study found that 23/34 patients studied had elevated fecal fat excretion.  In most, steatorrhea (excess fat in stool) was mild, but it can be severe.  In one study, four patients with steatorrhea all had abnormal findings on biopsy, including villi changes, increased inflammation in the mucosa, increased plasma cells and eosinophils, and sometimes increased mast cells.
Due to the excess excretion of fat in SM patients, they may have malabsorption of fat soluble vitamins such as D or calcium, causing tetany (involuntary muscle contraction) or osteomalacia (softening of the bones.)  Malabsorption of vitamin A can cause night blindness due to rod cone deficiency in retina.
Rarely, celiac disease is reported with SM.  In order to determine which is present, intestinal mucosa must be examined carefully by microscope.  In SM, patients may have patchy lesions with partial villous atrophy.  Enterocytes are normal, which is not seen in celiac.  Sparseness and destruction of crypts in seen in lamina propria, as well as lesions from mast cell infiltration along with neutrophils and eosinophils.  Villous atrophy secondary to crypt atrophy is sometimes seen.  But in SM, there is no crypt hyperplasia.
There have been a few reports of SM patients with selective deficiency of IgA in duodenal fluid only.
For many years, colon involvement was not considered to be an inherent part of SM. More recently, it has been found that up to 20% of SM patients have colon abnormalities.  Diverticulitis occurs in as many as 19% of patients.  Less distension of rectum is necessary to induce pain or urgency in SM patients.  They are also more likely to have overactive rectal contractility and decreased rectal compliance, making complete defecation more difficult.

13% of patients were found to have nodules in the colon mucosa.  Lesions seen by barium examination are thought to be due to edema and not mast cell infiltration, though mast cell infiltration of the colon has been reported.  Mastocytic enterocolitis has been described.  (I’m doing a separate post on this.)

Abnormalities seen include edema with or without granularity, edema with urticarial lesions, purple pigmented lesions.  Diffuse intestinal telangiectasia is sometimes present.  Biopsies of polypoid lesions show extensive infiltration by histiocyte like cells.  In some patients, colon or rectal mucosa showed mixed infiltrates of mast cells and eosinophils, increased mast cells in perivascular spaces, lamina propria, submucosa or muscularis mucosa. 
Diarrhea is a common complaint of SM patients.  There are several possible causes.  Fat absorption can cause diarrhea, but this is unlikely in SM.  It has been shown in these patients that diarrhea can occur with or without fat malabsorption, indicating that the two processes do not stem from a single origin.  Mast cell patients with diarrhea generally do not have malabsorption.  GI transit time in SM diarrhea patients may be normal or even slow, contributing further to the lack of the clarity.
Specific GI regulatory molecules directly causing diarrhea in SM have not been identified, although mediator release can certainly cause this symptom by various pathways.  PGD2 has been suggested repeatedly as a cause.  PGD2 can be 100X normal in SM patients.  In patients with very high prostaglandin levels, use of aspirin decreased diarrhea. 
The treatment for mast cell diarrhea includes the usual suspects, like H1 and H2 antagonists and cromolyn.  Tixocortol was also found to be helpful in decreasing abdominal pain and stool frequency.  Patients who improved with tixocortol also showed improvement on biopsy, decreased fecal fat excretion and increased absorption.

References:
Jensen RT. Gastrointestinal abnormalities and involvement in systemic mastocytosis. Hematol Oncol Clin North Am. 2000;14:579–623.
Bedeir A, et al.  Systemic mastocytosis mimicking inflammatory bowel disease: A case report and discussion of gastrointestinal pathology in systemic mastocytosis.  Am J Surg Pathol.  2006 Nov;30(11): 1478-82.
Lee, Jason K, et al.  Gastrointestinal manifestations of systemic mastocytosis.  World J Gastroenterol. 14(45): 7005-7008.

Gastrointestinal manifestations of SM: Part 1

Gastrointestinal symptoms are among the most common in SM, with up to 80% of patients experiencing them regularly.  When averaging figures from many studies, about 51% of SM patients have abdominal pain, 43% have diarrhea, and 28% have nausea and vomiting.  11% of SM patients have GI bleeding, usually in the upper tract.  Other GI problems common in SM include steatorrhea (excess fat in the stool), malabsorption, swollen liver, swollen spleen, free fluid in the abdomen and portal hypertension.  GI distress in SM can be severe and often mimics Irritable Bowel Disease or Zollinger-Ellison Syndrome.
Abdominal pain in SM generally has two types.  The first is epigastric dyspeptic pain, found in the upper abdomen, and is associated with ulcer disease and oversecretion of stomach acid.  Despite early reports that peptic ulcer disease is rare in SM patients, more recent studies have repeatedly disproven this idea.  On average, about 23% of SM patients have peptic ulcer disease.  Ulcers in SM patients with dyspeptic pain are often found on endoscopy.  In one study, 19% had a duodenal ulcer, while 25% had severe duodenitis.
The other type of GI pain is characterized by lower abdominal cramping.  Generally, one type is more prominent in a patient than the other, and they rarely co-occur with equal intensity.
85-100% of SM patients demonstrate increased histamine production.  Histamine is known to stimulate acid secretion, so SM patients are generally expectly to produce too much acid in the stomach.  However, studies have shown a variety of conflicting results.  Some patients produce too much acid, some too little, and some in the normal range.  For those who overproduce acid, the levels can be extremely high, comparable to levels seen in Zollinger-Ellison Syndrome. 
Occasionally, achlorhydria, the absence of gastric acid, has been found in SM patients.  This is thought to be due to atrophic gastritis (chronic inflammation of the stomach mucosa) that leads to impaired signaling from the local cells; however, this is unproven. 
Multiple studies have attempted to link serum histamine levels with normal basal acid secretion.  In one study, all patients had high serum histamine, but 56% had normal basal acid secretion.  This finding can be attributed to several things, including measured histamine not being fully biologically active; circulating histamine level being less important to acid secretion rate than the level of histamine in the local mucosa.  High histamine has been found in the gastric mucosa of several SM patients with dyspeptic pain. 
Furthermore, the authors elaborated that the histamine levels might not have been high enough to stimulate acid production; that the H2 receptors on acid producing (parietal) cells may have become desensitized to such high histamine levels; or that parietal cells were unable to respond to the histamine signaling, for some other reason.  Of these possible explanations, desensitization is supported by previous research, though not in SM patients.
In a study of 21 patients, 30% of them showed abnormalities on upper GI barium studies.  19% had gastric nodules and 11% had gastritis or peptic disease.  Biopsies of gastric mucosa show increased histamine and increased inflammatory cell infiltration with increased mast cells.  GI symptoms did not correlate with mast cell counts.
Common endoscopic findings in SM patients with dyspeptic pain include: acid hypersecretion; peptic ulcer disease; thickened gastric or duodenal folds; nodular mucosal lesions; occasional altered motility; occasional urticarial lesions; and increased infiltration by inflammatory cells with or without increased mast cells.
Studies have shown that approximately 28% of SM patients have esophageal abnormalities.  These include esophagitis, reflux, varices (abnormally enlarged veins that may bleed) or motor uncoordination.  Difficulty swallowing was common in these patients.  When assessed, these patients showed that the lower esophageal sphincter did not close with enough pressure. 
Esophageal motor function was assessed in 16 patients by manometry.  In 15/16 patients, the esophageal body contractions were normal.  In 62% of these patients, the lower esophageal sphincter function was abnormal.  75% of patients had reflux symptoms.  2/16 did not relax the esophageal sphinter during swallowing.
Esophageal varices have been reported in several SM patients.  The current rate of occurrence is listed as 2.5%, but this is likely an underestimation.
References:
Jensen RT. Gastrointestinal abnormalities and involvement in systemic mastocytosis. Hematol Oncol Clin North Am. 2000;14:579–623.
Bedeir A, et al.  Systemic mastocytosis mimicking inflammatory bowel disease: A case report and discussion of gastrointestinal pathology in systemic mastocytosis.  Am J Surg Pathol.  2006 Nov;30(11): 1478-82.
Lee, Jason K, et al.  Gastrointestinal manifestations of systemic mastocytosis.  World J Gastroenterol. 14(45): 7005-7008.

MCAD, MCAS and the hierarchy of mast cell disease classifications

I have seen several posts recently expressing confusion about various mast cell diagnoses so I figured I would put up a post to clear things up.
Mast cell activation disorder (MCAD) is a catch-all term for mast cell disease (MCD.)  MCAD and MCD can be used interchangeably.  So if you have any mast cell disease, you have MCAD.  If you have SM, you have MCAD, because SM is a type of MCAD.  If you have UP, you have MCAD, and so on.  MCAD is an umbrella term.  It is non-specific.  It is similar to being told that you have heart disease when you have mitral valve prolapse.  It is true, but it is not precise enough to give all information needed to treat effectively.
Mast cell activation syndrome (MCAS) is the diagnosis you get if you do not meet the criteria for any of the defined mast cell diseases, but have mast cell mediator related symptoms.  You cannot have MCAS and another mast cell disease because, by its definition, MCAS is ONLY diagnosed if you do NOT meet the criteria for any other mast cell disease.  You cannot have UP and MCAS.  You cannot have SM and MCAS.  I think some people think that MCAS means you have mediator related symptoms.  This is not the case.  You can have mediator related symptoms with pretty much any mast cell disease. 
A paper was published a few years ago by a doctor who considers mast cell activation symptoms to be due exclusively to proliferation (like in SM.)  He wrote a paper that says that MCAS is found in people with SM.  This paper sort of confused the issue for a lot of people.  However, the mast cell community (including researchers and prominent doctors) do not consider this to be the case.  They agree that you cannot have SM and MCAS.
Also confusing is the fact that mast cell activation (MCA) is NOT the same as MCAS.  MCA just means that your mast cells are activated, which occurs in any mast cell disease.  MCA is not a diagnosis, it is a symptom.  So you can have MCA in SM.  But you still can’t have MCAS in SM.
So if you have SM and have lots of mediator related symptoms, you have SM.  If you want to speak broadly, you have SM.
If you test negative for SM and have no CM, but have mast cell symptoms and elevated mast cell markers, you have MCAS. 
If you have UP and then later develop SM, you have SM with skin involvement, or SM with UP. 
If you have UP or TMEP and have lots of mediator related systemic symptoms, you do NOT have UP and MCAS.  You have UP.  UP and TMEP (forms of CM) can cause systemic symptoms.  But you cannot have MCAS because you can only have MCAS if you do not meet the criteria for another mast cell disease.
Let’s review.
If you have UP: you have UP, you have CM, you have MCAD.
If you have TMEP: you have TMEP, you have CM, you have MCAD.
If you have SM: you have SM, you have MCAD.
If you have SM with UP: you have SM with skin involvement, you have UP, you have MCAD.
If you have SM with TMEP: you have SM with skin involvement, you have TMEP, you have MCAD.
If you have SM-AHNMD: you have SM-AHNMD, you have MCAD.
If you have ASM: you have ASM, you have MCAD.
If you have MCL: you have MCL, you have MCAD.

If you have MCAS: you have MCAD.

Reference:
Molderings GJ, Brettner S, Homann J, Afrin LB. Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options. J. Hematol. Oncol.2011; 4:10-17.