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September 2014

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.
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.)
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.


I didn’t sleep well last night, and when I opened my door to go to work, it was really hot out.  Over 90 degrees, sunny, stiflingly humid.  The very short walk from the car to my office left me flushed and sweaty.  I was feeling usual level nauseous and my skin was burny.  My PICC line insertion site is sore.  It was shaping up to be a rough day in the neighborhood. 

I had a meeting this morning and then another one late in the afternoon.  I needed to take Benadryl before the first one was over.  But I was tired of being that sick girl who had to reschedule a meeting again, so I sucked it up and stuck it out.  It wasn’t super comfortable, but some days my stubborn streak is just wide enough to make it work.

I worked in the office from 9-345.  I got a lot of things done.  When it was time to leave, the sky glowered down at me, heavy and ready to thunderstorm.  I walked to the train and by the time I got to my final stop, there was a downpour of biblical proportions happening.  I was wearing a sleeveless dress and somehow had to keep my PICC line dry.

I walked around the train station while I formulated a plan.  I eventually shortened the strap on my brief case all the way and then closed my arm into the briefcase.  I ran through puddles in high heels like a boss and I’m sure I looked ridiculous but my PICC line stayed dry. 

The dogs played for a while in the yard after I got home and for no particular reason, I felt victorious.  I worked almost an entire work day in the office.  I got myself home without paying for a cab in ridiculous heat.  Physically, the heat was killing me, but I made it home in one piece and in no apparent danger. 

On days like today, I think maybe there’s no limit to what I can do.  I think maybe even though it felt like it, there were never any limits at all.

Intersecting realities

It has been a long time since I had a good summer.   Several years, at least.  The last few years, I have said, “This summer has to be good, to make up for last year.”  They never were, though. 
Part of it is the heat.  I like the idea of summer, but the reality is that the soaring temperatures put me into a constant state of reactivity.  Part of it is history.  My cousin hanged himself in July of 2011.  It was a month I will never forget.  Whenever the air gets sticky, I think of him in his hospital bed, and me waiting for him to die.  In order to overcome the general physical difficulties and emotional entanglements of those sweltering weeks, the fun I have can’t just be good, it has to be fantastic.  So the bar set for summer fun is quite high. 
I was in the hospital when the weather started to turn warm this year, and I wanted to be happy that summer was coming, but instead I steeled myself for months of regret.  I always want so badly to enjoy it, but I inevitably find myself wishing it were over.
This summer has had a very surreal quality to it.  My health has been quite terrible.  I have been nauseous and bloated and bleeding and exhausted.  I have needed a lot of rescue meds, a lot of Epipens, a lot of IV fluids and push meds.  My aunt died very young after a brief, severe illness.  Someone very close to me stole from me.  My lows have been low this summer, to be sure. 
But I went back to Seattle this summer, and that trip was the truest expression of magic I have ever encountered.  We really caught lightning in a bottle for those five days.  The entire experience was otherworldly, in a way that I can’t properly articulate.  It was a beacon of love that I badly needed. 
And my cousin got married this summer, and being around people who love each other so much is inherently healing.  I will never forget how happy I was that day.  I am so grateful to have been a part of it, and as hard as it was on my body, it did wonders for my soul.
This past Wednesday, I went to a water park with two of my oldest, dearest friends, who are brother and sister.  We have known each other since September of 1988, when I met him at Catholic school kindergarten.  It was very, very hot and humid last Wednesday.  The combination of heat and sunlight is potent.   I took a lot of premeds and still felt sick for a good part of the day.  I was worried about my PICC line getting wet, under its Drypro cover. 
Late in the afternoon, we waded into the wave pool and for the first time in a year, I swam underwater.  Slipping under the surface washed all of the worry and fear away.  It was rejuvenating and wonderful.  I treaded water as the waves came and surrendered as they crashed around me.
When summer ends, I usually find myself mourning.   I would focus on the things I couldn’t do or the bad things that happened, all the ways summer had wronged me.  It is so easy to tally all the ways my body and the world failed me.  I would mourn the fact that it wasn’t better, and that once it ends, there is no chance that it will improve.  Those summers are permanently marred in my memory. 
This summer was different.  I found my soul again.  It feels very much like I pushed my hand through the veil that separates the worlds and found that sometimes I am living in all of them at once.  It feels like this reality briefly intersected with realities where I am strong and healthy, and I unknowingly walked through all the places where they met.  This living in several worlds at once is confusing and painful, but it is also empowering and truly magical.  I think it’s the only way to be. 
This year I’m not sad because my summer was miserable.  This year I’m sad because this summer was heartbreakingly beautiful, and like everything else in life, it ended much too soon.