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Lisa Klimas

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

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.

PICC Lines: Dressing change and blood draw

PICC Line Sterile Dressing Change Procedure:

1.       Place arm on drape provided in dressing change kit.
2.       Open StatLock and Biopatch packages into the dressing change kit box.
3.       With gloves on, remove the occlusive dressing by pulling up the edges and then pulling the dressing toward the midline.
4.       With gloves on, pull up edges of StatLock.  Unclip the locks on the StatLock.  With one hand, lift the PICC line up, while pulling the StatLock off the skin with the other hand.
5.       With gloves on, remove Biopatch from around the PICC line at the insertion point.
6.       With gloves on, use alcohol (if appropriate) to clean any remaining adhesive from the area.
7.       Put on sterile gloves provided with dressing change kit.
8.       Disinfect the area around the PICC line (either with chlorhexidine or betadine.)  Allow to dry.  Do not touch the area while drying.
9.       Connect wings of PICC line to StatLock and apply StatLock to the skin.  Apply Skinprep to the the skin prior to pressing the StatLock to the skin (if appropriate.)
10.   Apply Biopatch to the PICC line near the insertion site.
11.   Apply occlusive dressing.  (I use IV3000.)
12.   Use adhesive strips from the StatLock package to secure the PICC line where it exits from the occlusive dressing.
 
PICC Line Blood Draw Procedure:
1.       Flush line with 10ml sterile saline.
2.       With the same syringe still attached, gently draw back to pull 5ml of blood into the syringe.
3.       Disconnect syringe containing blood.
4.       Attach needleholder/Vacutainer.  DO NOT STERILIZE PICC LINE CLAVE IMMEDIATELY BEFORE THIS STEP.
5.       Insert appropriate tube into needle holder and collect appropriate amount of blood.
6.       Remove tube.
7.       Disconnect needleholder/Vacutainer.
8.       Flush line with 10ml sterile saline.
9.       Flush line with 10ml sterile saline again.
10.   Lock line with 5ml heparin.

Becoming reality

I scheduled my colostomy surgery about six weeks before I had it.  That afforded me a comfortable window of time to overthink it and work myself up.  I am a logical person.  I am a scientist.  I understand the risks and rewards of procedures and meds and so on.  But I am also human.  While I knew it was the right decision, when I was alone, I often thought about all the ways it could go wrong.
One of my friends asked me if I was ready for surgery a couple of weeks before I went in for it.  “I wish I could just do it right now,” I answered.  “Once it’s my reality, it’ll be fine.  This thinking about it all the time is exhausting.”  I think that sums it up well.  I just need these things to happen because once they become my reality, I just deal with it and move on.
Part of why mast cell disease is scary is because so many things can go wrong.  That doesn’t mean they ever will, but even if you feel confident you can manage your symptoms, you can’t help but think about all the horrors lurking in the dark places of the world.  But it’s not productive or comfortable to live your days living afraid of all the terrors that might befall you.  When living with mast cell disease is your reality, you just do what you have to do to get through your day.  It’s okay to worry as long as it doesn’t keep you from living.
I got a port placed today.  I have known this was coming for some time and I know plenty of people who have them.  It was not something I was logically worried about. 
But last night, the mental gymnastics started and suddenly I was worried about IV contrast accidentally being used and my friends and family reading my journals after I died from the reaction.  There was no reason to think this would happen.  It was pure ridiculousness.  But that doesn’t mean it’s not scary. 
I told a friend about it.  “Oh, I thought I was the only one who did that!” she said.  No, you’re not.  We all do it, whether or not we admit it. 
This morning I arrived at my hospital at 6:30am to have my port placed.  I met with the PA doing the procedure at 7.  He had read my entire history (“Which is really long and interesting,” he noted) and did some research on masto.  He went through the entire procedure, what materials would be used, what meds would be pushed, and made necessary changes.  (No Tegaderm, no chlorhexidine, absolutely not under any circumstances IV contrast.)  He asked what I wanted for premeds and ordered them for one hour before the procedure exactly as I requested, right down to the diluted Benadryl pushed over 10 minutes followed by a slow flush.  The nurses and technologist were excellent and the procedure went very well.  I am very sore and tired, but I have an accessed port and no PICC line and no reaction to speak off.  That’s what I call success.
Whenever my body changes in a noticeable way, I show it to my animals and let them investigate it.  Tonight I sat on the floor and Story came over and sniffed at my port and mouthed at it a little.  “It’s okay,” I told her.  “My body used to look different but now it looks like this and it’s okay.”
And you know what?  It really is. This reality is not so bad at all.
 
 
 

Last days here

I have a lot of flaws, but one thing I am is fair.  I have always seen the world the same way: balanced in all things, if you wait long enough.  It seems to me that life is just a series of interconnected decisions, a closed system; that if you had a lot of bad, you would have a lot of good to balance it out, to zero the sum.  Part of the difficulty of being sick is that, in the back of my mind, I am waiting for it to balance and it never seems to.  In the quiet moments, it leaves me disappointed and confused.
Life gets a lot less confusing when you realize that even if it balances, it is not fair, and that sometimes things happen without a reason.  It is much less confusing when you accept that sometimes, no matter how hard you fight, life breaks you in a way that can’t be fixed.
I have been trying for a while to remember a particular day: the last day when I was healthy.  It’s hard because every time I think I have identified the window in which it would have taken place, I am reminded of some previous strange illness or reaction that looks decidedly like masto.  I remember my back injury when I was 13 and the bizarre subsequent neuro issues.  I remember breaking out in hives from eating salsa and thinking for years that I was allergic to tabasco, chili and cayenne.  I remember sudden, severe abdominal pain as a child and burning lungs.  The truth is that I lived my last day as a healthy person so many years ago that the memory is lost, and I never even knew it. 
My disease has changed this past year.  It used to be that I would have sick days and then they would pass and I would feel better, normal.  Now I have bad days and normal days, except now on my normal days I am nauseous and flushed and in pain.  Like so many things about my life, it is hard to isolate exactly when it became this way, constant and more pervasive.
I cherish these normal days, so wonderful compared to the bad ones.  In the dark of night, I fear they will end forever someday.  What will I do, when they are all gone? 
I worry that maybe this feeling of transience I experience now is a sign of this happening.  I am afraid that maybe I’m living the last days of this stage of my life, and when it is gone, I will miss it.

Neurologic symptoms of mast cell disease

Mast cells are known to closely associate with nerve endings and to be important in neurotransmission.  This can translate into a variety of neurologic symptoms.
In 2011, a retrospective study on the neurologic symptoms of mast cell patients (171 SM patients, 52 CM patients, all adult) was published.  The following is a summary of the results.
Syncope (fainting) is a well-defined complication of mastocytosis, reported here in 14.3% of patients .  In these patients, evaluation revealed that the likelihood of epileptic involvement was likely low.  About 2/3 of patients who had fainting episodes also had loose stool, cramping, nausea, sweating and flushing accompanying the episode.  Prostaglandin D2 and histamine are known to cause low blood pressure and fainting in addition to GI symptoms.  Aspirin is thought to protect against acute vascular collapse and fainting, and sees use in tolerant patients for these purposes.   
16.6% of mastocytosis patients complained of back pain.  In all but one patient, the cause was determined to be multifocal compression fractures throughout the spine, including thoracic region.  Vertebroplasty, a procedure in which special bone cement is applied to the fractured vertebrae, has been suggested for symptom relief of these patients.  One patient was found to have back pain due to dense mast cell infiltration of the vertebrae.  In this patient, radiation therapy provided symptom relief.
35% of patients reported headaches.  Several of these patients met the criteria for migraines.  Mast cells have been implicated repeatedly in migraine pathology, and mastocytosis patients are more likely to suffer from them than the general population.  In response to mast cell degranulation, reactive changes have been noted in trigeminal nerve, the structure responsible for sensation in the face and activities like chewing.  Trigeminal neuralgia has been noted in some patients with mast cell disease.
This paper was also the first to find a link between mastocytosis and multiple sclerosis.  Two adults with ISM developed relapsing remitting MS, and a patient with isolated UP developed primary progressive MS.  Mast cells are known to associate with MS lesions, and mast cell activation can be detected in cerebrospinal fluid of MS patients.  This study found an MS frequency of 1.3% among mastocytosis patients, compared to 0.1% in the general population.
Lastly, an association has been found between overall mast cell burden and susceptibility to experimental autoimmune encephalitis (EAE.)
Reference:
Smith, Jonathan H, Butterfield, Joseph H, Pardanini, Animesh, DeLuca, Gabriele, Cutrer, F Michael.  Neurologic symptoms and diagnosis in adults with mast cell disease.  Clinical Neurology and Neurosurgery 113 (2011) 570-574.

A long, long way

This weekend, one of my oldest friends got married.  The wedding was about three hours away.  My mother rented a Mustang convertible, I packed all my various medical supplies and a couple of dresses and we drove up on Friday night.
“Can you ride with the top down?”  she asked, excited.  I thought about it.  I thought the sun might make me feel gross, and it was very hot out.  But it isn’t every day that you get to drive around in a convertible so I figured I’d give it a shot.  I put on sunscreen and sunglasses and away we went.  It was very Thelma and Louise.
I felt gross on Saturday morning, but I didn’t really care.  I loaded up on meds and tried to keep the nausea at bay.  I put on my dress and braided my hair.  I crossed my fingers that I got through the day without vomiting indiscreetly or needing epinephrine.  And I did.
The bride and her family have been close to me and mine for over twenty years.  She and I grew up together.  She and her mother both told me how glad they were that I made it.  They knew how sick I had been, and that making the trip was hard on my body.   So often people don’t understand how hard things are for us that when people do understand, it means a lot. 
It was this month last year that I started having serious bone pain.  It was this month last year that one of my doctors told me that he thought my CT scan had been misread, that my spleen looked swollen to him.  It was this month last year that I lost the semblance of health I had been holding onto. 
It was this month this year that I drove to my friend’s wedding three hours away in a convertible on a hot summer day.  It was this month this year that I went to the wedding and didn’t need epi or IV meds while I was there.  It was this month this year that I reflected on how far I had come while driving home from the White Mountains.  There has been a lot of struggle, but there have also been a lot of good days, and some really great ones that I’ll never forget. 
One of the things about being sick that healthy people don’t experience is how satisfied and accomplished you feel when you are able to do something important in spite of your illness.  I was exhausted and in a lot of pain when I got back to the hotel last night.  But I was also very happy that I had been able to be present for such an important day.  I was proud of myself and my body for pulling it together.
You don’t get a choice in being sick, but you do get a choice in where you place your energy.  A lot of the time, it goes to mundane things, cooking, shopping, laundry.  But every once in a while, I save up and blow it all on something big.  It will take a few days to get back to my baseline, and I took a lot of extra medication, but some things are worth it. 
I can live with this if I can still do the things that are worth it.  Having mast cell disease doesn’t matter when you get to be with someone you love while they have the happiest day of their life.  And when I look back, I can’t deny that I have come a long, long way this year.

Mast cell disease and chronic constipation

Okay, folks.  I like to maintain the illusion that I am at least kind of classy, but I’m about to do something that will erase that perception forever.  It’s time to talk about poop.
One of the more embarrassing things about mast cell disease (and one of the things that doesn’t get talked about a lot) is the fact that it involves a lot of poop.  You spend a lot of time in the bathroom, and can never be far from one.  It is so disheartening and humiliating.
The very first time I had a full blown mast cell attack was in September 2010.  I had drunk a lot of alcohol, which was unusual for me, and had a very stressful week, which was not.  A few hours after I got home, I got nauseous and could not stop getting sick.  I also got diarrhea and spent the entire night in the bathroom. It was awful.  Imagine my horror when it happened again a few weeks later, and with increasing frequency until I was diagnosed with mast cell disease. 
In the few months after I was diagnosed, I started on several mast cell medications.  My mast cell attacks largely subsided, and diarrhea was no longer an issue.  Hooray!  There was much gladness throughout the land.  Then I went to Seattle and found I was unable to go to the bathroom.  I have written about this at length before, so I won’t rehash the details.  I went on to develop true bowel obstructions, as well as some pseudoobstructions, severe abdominal pain, bleeding, and literal inability to poop.  I got a colostomy in April 2013 and you could not pay me to reconnect my colon to my rectum.  It improves my life that much.
Diarrhea is more associated with mast cell disease because it is associated with anaphylaxis.  Even when not anaphylaxing, people often have loose stools and increased frequency.  However, a French study of mastocytosis patients found that only 12% had four or more stools per day.  Doctors are realizing that while diarrhea is more common during episodes or anaphylaxis, most patients find themselves chronically constipated or pseudoobstructed.  In that same French study, 57% of SM patients reported having at least two bowel pseudoobstructions a year.  Pseudoobstructions are when your body behaves like it has a bowel obstruction, but it does not.  It is often associated with long term constipation and GI dysmotility.
I pretty much consider myself the poster child for mast cell derived constipation, so I am going to tell you everything I tried and share all of my tips with you.  Please keep in mind that at the end, I was functionally unable to defecate, so I am relating the effect of my methods to their likely effect on you. 
When I started having major difficulties, I would make myself smoothies twice a day.  These smoothies had strawberries, pineapple, banana, yogurt and orange juice.  This made the stool softer.  I realize these are high histamine, which for some can contribute to diarrhea, and thus explains why it moved more quickly through my GI tract.  I tried adding this stuff called Green Super Food, which did not seem to make a difference and also smelled weird.  Later, I added two scoops of Metamucil.  I didn’t see any benefit, but it didn’t cause a reaction.  It was recommended by mast cell GI specialist. 
I took 300mg of docusate twice daily.  This is several times the recommended dose.  It made the stool softer and it moved through my GI tract more quickly.
I tried senna, which I later discovered can increase serotonin and exacerbate mast cell symptoms.  It did help move things along, but it made me very nauseous.
Glycerin suppositories did nothing for me.  Literally went in and stayed there.  I once punched a package of glycerin suppositories.  It was not my proudest moment.
Bisacodyl suppositories helped, but did not result in complete evacuation.  My rectum is damaged, so inserting suppositories was painful and caused bleeding.
Magnesium citrate worked if I also used a saline enema, but I had to drink two bottles of magnesium citrate.  It usually made me throw up and flush.  It was not a great option, but in an emergency, it was successful. 
Castor oil did nothing except make me grumpy because of how gross it tasted.
Saline enemas were my mainstay.  I used two every three days.  At the end, I sometimes needed to manually disimpact, which is as gross and humiliating as it sounds.  Frankly, I am only admitting it because I’m sure someone else has needed to do it and I want them to know that they are not alone and they are still awesome. 
I tried miralax because my very well intentioned PCP felt I should try everything before I had my colostomy, which I appreciate.  I took half a bottle and three days later still had to use enemas.  It didn’t hurt me or cause a reaction, it just did nothing.
About three weeks before my surgery, I met with a man who did bowel retraining at another hospital in Boston.  This therapy was originally developed for kids with Hirschsprung’s disease.  The idea is that the colon and rectum can be trained to pass stool at the same time every day.  This is a good option for people with long term constipation and can be done at home.  It works by picking a time every day (it is important that it be done at the SAME TIME each day), inserting a glycerin suppository and trying to defecate.  For most people, glycerin suppositories will stimulate contraction of the relevant muscles so you should not be straining.  DO NOT STRAIN.  I’ll get to that in a minute.  Eventually, your body becomes trained to defecate at the same time each day without the glycerin suppository.  For several reasons, I did not do bowel retraining, but it does work for some people. 
A lot of people who are chronically constipated have pelvic floor dysfunction.  This means that your muscles are not working correctly and so they are holding in the stool when you strain.  Often, you can retrain your muscles with biofeedback. This can be done by a physical therapist trained in pelvic floor PT.  
I strongly advise anyone with chronic constipation to get an anorectal manometry test.  This gives a lot of information about how your body feels relevant sensations and how it works when defecating.  I had this done twice before my surgery at two different hospitals.  In both instances, I was found to have substantial nerve damage and so my body did not feel the need to go the bathroom until about five times the normal amount of stool was present.  The stool sitting in the colon caused it to further dehydrate, making the problem worse.  It was the perfect storm of unmovable shit.  The man who did bowel retraining told me that he had never met anyone whose body seemed so determined to prevent them from defecating.  But he said it like it was an honor, which made the whole thing even stranger.
I find bowel transit time tests to be very helpful and easy.  You swallow a pill that contains a bunch of tiny corkscrews.  You get xrays taken at specific intervals (I think it was 1 day after, 3 days after and 7 days after) and the corkscrews show where they were pushed into the tissue.  If they were pushed into the tissue, it means the stool sat in that place for a long time.  It allows you to see which part of the GI tract is not working.  I also recommend a colonoscopy with biopsies and staining for mast cells. 
I have had many other GI tests, as I have had GI problems since well before I knew I had systemic disease.  I have had multiple endoscopies, esophageal motility testing, pH probe testing (this was before they developed the pill you can swallow that records the pH data), and an MR defecography.  I do think that there is a subset of people for whom this last one is a useful test, but if your doctor is ordering it, I encourage you to ask how the data they get will change your treatment plan.  It is literally being forced to defecate into a diaper in an MRI machine in front of a bunch of strangers when you know you can’t.  It was awful.  Again, I’m only admitting this because someone out there has had this test and is mortified and thinks they are alone and they are not. 
Straining causes as much damage as constipation.  It causes long term nerve damage, hemorrhoids, bleeding and fissures.  You should not be straining.  If you need to in order to defecate, something is wrong.
Though I was passing stool regularly and was no longer generally constipated after damaged portion of my colon was removed, I still got bowel obstructions.  Obstructions are unbelievably painful.  They hurt worse than getting my colostomy.  My bowel is herniated in a few places and it twists on itself so the stool can’t get through.  My colon also swells seriously when I have a mast cell reaction or anaphylaxis, which makes it harder to pass stool.
If this happens to you, and it is a new phenomenon, you should go to the hospital.  Bowel obstructions can cause bowel rupture and are serious.  If this happens to you regularly, and your doctor is okay with you managing at home, my recommendations are: hot liquids (tea works best, though not all mast cell patients can drink it); lots of water (IV fluids if possible); heat packing the abdomen; taking a hot bath; abdominal massage, especially if you can find the obstruction (it is hard when you massage the abdomen); moving, like walking or yoga.  All of these things also work for pseudoobstructions.  I know that heat is bad for some of us, but I find that in this situation, the benefit outweighs the risk of reaction for me.  Especially because the pain causes me to react anyway, so I’m generally already taking extra meds by that point.
Because bowel obstructions are so painful, we are often given opiates to manage the pain, which further decreases GI motility and reinforce the issue.  Most of the meds we take for mast cell disease also cause decreased GI motility, so we have to be careful with taking anything more that has that side effect.  I have found only two medical therapies to be helpful in managing recurrent obstructions: steroids and IV fluids.  Since starting IV fluids three times a week, I have had some bowel episodes but they were minor compared to the years of constant nonsense I had put up with.
The low residue diet is designed to be easy to pass through the colon.  However, it is not very masto friendly.  It is also called the “junk food diet.”  I will sometimes do it for a few days if I am very sore.  When I first started eating low histamine, I was very sore because my GI tract was working so much harder to digest everything. 
Sometimes there is no choice but to remove the damaged portion of bowel and place a permanent ostomy.  If you are considering this option, I am very happy to talk to you about it.  It is not the end of the world.  I am so glad I got mine.
I know this is a lot of information, but the bottom line is that a lot of people with mast cell disease can’t poop. It is painful and humiliating and I want them to know they are not alone.

PICC Lines: Nomenclature, fluid infusions and IV Benadryl

My PICC line set up and what the various pieces do:

 
How to hook up a fluid infusion: 
 
How to dilute and administer IV Benadryl. 
 
 
IV Benadryl should always be diluted as it can be damaging to the veins and can cause spasms of the airway if pushed too quickly.  It should always be pushed slowly. I generally dilute 1ml (50mg) of Benadryl with 9ml of saline for a total volume of 10ml, which I then push over about 15 minutes.  The flush after the Benadryl should also be slow.  I push that over about 5ml. 
I made a video of me drawing blood for labs through the line and then deleted it by accident because I am a fool.  Bah!  So I’ll make another one next week, as I don’t have any spare tubes and Vacutainers.  I am putting together a video on dressing changes as well.