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March 2016


I am not easily intimidated. I have been sick a long time. I am used to being around hospitals, doctors and sick people. I am used to reading lab work and pathology reports. I have seen a lot of people pull out of medical crises. I have seen my intestine attached to the outside of my body, emptied colostomy bags, packed my own incisions, accessed my own port. It takes a lot to scare me.

Waking up with a fever of 103.2 two days after dental work scared the shit out of me. It’s kind of funny in hindsight in a morbid way: infectious diseases microbiologist develops tests for bloodstream infections, gets bloodstream infection. But it wasn’t funny then. I am very even when I speak to providers who don’t know me because my life could literally depend on it. I was even that day, but it took a lot of effort.

I was discharged after a few days of antibiotics and continued them at home for another week. I called out of work, a rare instance of sick time rather than working from home, because I was so exhausted and winded that it was difficult to do anything. In 2014, when the shit really hit the fan, standing up was enough to make me sweat, my heart race and blood pressure drop. It felt like that again. Like anything but being in bed was too physically demanding and being awake was too mentally demanding.

In the days after discharge, I lay in bed thinking about deconditioning and POTS and anaphylaxis and what if I had to start all over again? There isn’t a word for what I was experiencing. If we had a word for the crescendo to blind panic, the choking and the blood pounding before you scream, that might be it. What if I got these nine months of improvement and this was it?

The first few days back at work were very hard, my blood pressure was low and my mouth still hurt a lot. I had appointments last week at the hospital and one of my doctors is pretty convinced that I had a true bloodstream infection that just didn’t culture because of the antibiotics. I slept most of this weekend. But things are coming back together.

This past year, it was easy to settle back into a routine, to prioritize certain things over the things I had always dreamt of pursuing. It feels foolish now to have done that. I cannot take for granted that the way I have felt is the way I will continue to feel. If I hadn’t been on antibiotics since the dental procedure, this story could have ended very differently, with my port being pulled and time in the ICU to treat a bloodstream infection and anaphylaxis and months of recovery.

Life is short. All important things are derivative of this. Every lesson is secretly the same.

Explain the tests: Complete blood cell count (CBC) – White blood cell count (Part five)

White blood cells, also called leukocytes, are key functionaries of the immune system.  There are several types of white blood cells and each is specialized for certain types of immune response.

White blood cell levels are useful for pointing to many conditions.  They can be high or low for many reasons.  They are commonly used to determine whether or not a patient has an infection.  A “left shift” in the white count indicates presence of high numbers of immature white cells, often called bands.  A left shift can occur for a number of reasons.  It is a natural response to infection as the body tries to make enough white cells to fight the infection.  A “right shift” refers to the absence or low level of bands, new white cells.  This indicates suppression of bone marrow.

Normal range for white blood cell count:

  • 0-11.0 x 109 cells/L

Types of white blood cells can be quantified as either a percentage of total white cells or as an absolute count.  Normal white cell count varies with age, especially neutrophils, lymphocytes and monocytes.

Normal range for neutrophil count:

  • 8-7.7 x 109 cells/L
  • 35-80% of total white cells

Normal range for eosinophil count:

  • 0-0.8 x 109 cells/L
  • 0-4% of total white cells

Normal range for lymphocyte count:

  • 8-4.8 x 109 cells/L
  • 18-44% of total white cells

Normal range for monocyte count:

  • 2-0.9 x 109 cells/L
  • 7-12.5% of total white cells

Normal range for basophil count:

  • 0-0.1 x 109 cells/L
  • 0-1.2% of total white cells

Explain the tests: Complete blood cell count (CBC) – High white blood cell count (Part seven)

High white blood cell count is called leukocytosis. High white blood cell count is often due to a healthy process, such as immune defense or inflammatory response after an injury.

Reasons for leukocytosis:

  • Infection response, especially bacterial infection
  • Inflammation
  • Physical stress and tissue death
  • Allergic disease
  • Proliferative diseases of white blood cells, such as leukemias

Conditions that cause tissue death and elevate white blood cells:

  • Physical trauma
  • Surgery
  • Ischemia
  • Heart attack
  • Burns

Allergic conditions that elevate white blood cells:

  • Allergic reaction, acute or chronic
  • Anaphylaxis
  • Asthma
  • Atopic disease

Inflammatory conditions that cause elevation of white blood cells:

  • Autoimmune diseases such as rheumatoid arthritis
  • Inflammatory bowel diseases

Medications that trigger excessive production of white blood cells:

  • Corticosteroids
  • Beta agonists


Explain the tests: Complete blood cell count (CBC) – Low white blood cell count (Part six)

Low white blood cell count is called leukopenia. Due to mast cell involvement in many bodily processes, leukopenia can occur for many reasons.

Reasons for leukopenia:

  • Bone marrow suppression
  • Disorder of white cell production or white cell precursors
  • Proliferative disease of other cell types in the bone marrow
  • Mechanical destruction of white blood cells, as in splenomegaly (swollen spleen)

Some conditions that interfere with making enough white blood cells:

  • Certain infections, such as tuberculosis, malaria, dengue fever, Lyme disease and viral infections
  • Sepsis
  • Nutritional deficiency, such as low copper or zinc
  • Nutritional toxicity of certain minerals, such as arsenic

Some proliferative diseases that interfere with making white blood cells:

  • Hodgkin’s lymphoma
  • Myelofibrosis

Conditions that affect white cell precursors:

  • Aplastic anemia
  • Myelodysplastic syndrome
  • Damage to precursors by radiation exposure or chemotherapy

Conditions that cause damage to white cells:

  • Splenomegaly, swollen spleen
  • Lupus


Medications that interfere with making enough white blood cells:

  • Immunosuppressants, like mycophenolate, cyclosporine and TNF blockers
  • Interferon preparations, like Betaseron
  • Other medications like clozapine, bupropion, minocycline, lamotrigine and valproic acid
  • Chemotherapy
  • Radiation

The unseen hand

I have spent a lot of the last several weeks trying to navigate the dark waters of mid-level care for a rare disease patient.  It is one of the trickier aspects of life with a disease like mine.  I need care and need providers to be cautious but I also need them to not be scared of my disease or they could refuse care.  When I’m shocking or having a serious medical event, it’s not really an option to refuse care.  But when something is serious but not life threatening, I have to tread carefully.  It requires just the right amount of education given confidently.  The tiniest twinge of voice, a catching of my breath while I’m talking about my disease, and they could easily walk away.

I got my tooth removed on Tuesday at a dental office in the same hospital where I receive all of my care.  The dental team was very good and took my special requirements in stride.  But I realized pretty quickly that this extraction was going to be harder than I had expected.  All told, it took three people two hours of actively trying to extract the tooth to achieve success.  EDS patients are often insensitive to anesthesia and they stopped several times to renumb everything.  There was some bleeding and a bit of bruising but nothing that felt inappropriate given the nature of the procedure.  I went home with an order for antibiotics and ketorolac.

My mouth hurt a lot Tuesday night and Wednesday but the pain didn’t feel disproportionate to the violence of the extraction.  I slept a lot and iced my jaw.  I could eat gingerly and talk.  Overall, I felt okay.  Not great, but okay.  I ironed work clothes on Wednesday night and got everything together to return to work on Thursday morning.

I woke up Thursday not because I had to work, but because of how badly my face hurt.  I had medicated before bed and it should not have worn off by that point.  I knew right away that something was wrong.  I flipped on the bathroom light to reveal flushed cheeks and glassy eyes, my hair matted to my forehead with sweat.  I put a thermometer into my mouth and started putting things into a backpack as the numbers climbed precipitously.  I had a fever of 103.2.  In less than an hour, I was in the emergency department of my regular hospital.

Dental procedures somehow manage to masquerade as routine despite the fact that they take a lot of skill to perform safely and that some of them are inherently risky.  Though the guidelines have been revised in recent years, many patients are still recommended to premedicate with antibiotics prior to dental work.  The reason is that it is very easy to transfer bacteria from the mouth to the bloodstream, even when all appropriate safety measures are followed.  If you have an artificial heart valve, or certain other conditions, there is an increased risk that these bacteria will become a true bloodstream infection, which is very, very serious.

The emergency department took me in right away.  I am always nervous in emergency departments because I have had a lot of trouble in the past, but everyone was great yesterday.  They knew about SM, they knew general guidelines and gave me no shit about needing hydromorphone for pain relief.  They were fine with following my home IV orders and using my port.  They worked together with my mast cell specialist, primary immunologist, dentist and PCP to make sure everyone was on the same page.

The big concern was that I had a bloodstream infection, or at least a bad dental infection, and that it would colonize my port.  My mouth looks fine and my white count is normal (which in itself is unusual, as it has been in the 16-20K range for years).  The port looks fine, isn’t red, tender or swollen, and works fine, which is great news.  All of this is good news except we have no idea why I have a fever of 103.

My doctors are generally fine with me handling things on my own at home, so I didn’t protest when they all said I should be admitted.  There is concern that because of some of my medications (like prednisone and Enbrel) that my bloodwork won’t show that I’m fighting an infection until it is severe.  So they admitted me to a surgical floor where I have been before and I’m just hanging out for a few days until we are convinced my blood cultures are truly negative.  The care has been excellent and there has been no bullshit about how I dose my meds, how often I use IV meds, or doing things the way I want them (dilute and slowly, as a rule).

I am on a stronger antibiotic as a precautionary measure, but I expect to have negative blood cultures and to go home in the next day or two.  The most plausible cause of this sudden high fever is that transient bacteremia, in which some bacteria from my mouth entered my bloodstream during the extraction.  My body then generated an immune response and was able to keep these few bacteria from becoming millions of bacteria in my bloodstream.  It’s basically a best case scenario.

I used to develop diagnostics for bloodstream infections.  I develop molecular diagnostics and have for several years, but my training is in microbiology and my focus was infectious diseases.   When I realized this tooth needed to come out, my biggest concern was that the tooth would get infected and I would get a bloodstream infection or that bacteria would be transferred during the extraction and I would get a bloodstream infection.  Most of the time, the most serious risk associated with a procedure I need is that I will have anaphylaxis.  This was different, and the way it played out proved that I was right to worry.

In the weeks leading up to getting this tooth removed, I talked to people in several dental/oral surgery offices, including the one where I was treated regularly for over fifteen years.  Some of the realized that I was not trying to be ridiculous and that there were rock solid reasons for me to want certain materials to be used, certain meds to be avoided and so on.  But, as there usually are, there were also several people who just thought I was being particular and difficult.  When people think this about me, they usually believe I have an anxiety disorder and that my need to control things is an extension of that.

And when they say to me, “You can’t control everything” with a snotty lilt on the last syllable, it’s not a bitchy imparting of a common adage.  It’s a warning.  For me, not being able to control everything could be catastrophic.  In some instances, it could be fatal.

The need to understand the entire procedure and know everything is not something I relish.  It is exhausting and scary.  Even when everything is done right – like my extraction on Tuesday – there are still risks.  There is still an unseen hand that can push us over the edge for no reason at all.

I have a short speech that I give to every single provider I meet who has not met me before.  It starts with, “Before we get started, I have a mast cell disease.  Have you ever treated anyone with that before?” I hit all the high notes briefly and tell them I will be using my Epipen first and then asking for help if I anaphylax, not the other way around.

Tonight I gave my speech to a new nurse.  We chatted about mast cell disease for a while.  “Does it bother you?  Having to teach everyone about this?”

It doesn’t, but if it did, it wouldn’t matter.  It’s my only chance to stay ahead of the unseen hand.

Death of the danger tooth

I finally got my tooth extracted yesterday. The root was really big so it took a couple of hours and a lot of force to get it out. My jaw is really sore but all in all, everything went fine.

I am giving myself a few more days to heal before I start going through my inbox/comments. I appreciate your patience.

The Danger Tooth


The Sex Series – Part Nine: Female pelvic floor dysfunction (2 of 2)

Muscular dysfunction in the pelvic floor starts when something happens that causes an injury or large scale inflammation to the pelvic floor.  This causes a large scale release of calcium, which causes the muscle to become too tight (hypertrophic).  As a result of this tightness, metabolism in the tissues increases and substances like histamine, serotonin and prostaglandins are released.  These mediators trigger neurologic pain perception.   The pain causes tightness, which causes more pain, and the cycle continues.

Hypertrophic muscles become musculodystrophic as fibrosis occurs.  The muscle becomes atrophied and is replaced by less extensible connective tissue.  As a result, the muscles aren’t as flexible as they should be. This also means that they cannot relax normally.  This activates trigger points in the pelvic floor and increases tone and spasm in pelvic structures, including the bladder, uterus, and rectum.

Treatment for pelvic floor dysfunction of women is very well described in literature.  It relies largely upon patient education and compliance with various exercises to retrain the muscles to relax completely at will.  Trigger-point pressure, both internal and external, can be applied by the patient or partner to help the muscles relax.  Vaginal or anal dilators, vaginal cones and bladder training can also be effective. Physical therapy including myofascial release and biofeedback are also important to treatment.

While initial treatment of PFD can be complex and time-consuming, the results are very good.  One study followed a cohort for ten years. 71% of women in this cohort reported major reduction or elimination of pain level following physical therapy and exercises done at home. After ten years, 89% of women reported major reduction or elimination of pain.  Many patients continued their home exercises during that time.



Bortolami A, et al. Relationship between female pelvic floor dysfunction and sexual function: an observational study. J Sex Med 2015; 12: 1233-1241.

Hartmann D, Sarton J. Chronic pelvic floor dysfunction. Best Practice & Research Clinical Obstetrics and Gynaecology 2014, 28: 977-990.

Espuña-Pons M, et al. Pelvic floor symptoms and severity of pelvic organ prolapse in women seeking care for pelvic floor problems. European Journal of Obstetrics and Gynecology and Reproductive Biology 2014, 177: 141-145.

Ramalindam K, Monga A. Obesity and pelvic floor dysfunction. Best Practice and Research Clinical Obstetrics and Gynaecology 2015, 29: 541-547.

Graziottin A, et al. Mast cells in chronic inflammation, pelvic pain and depression in women. Gynecol Endocrinol 2014; 30 (7): 472-477.

Ahangari A. Prevalence of chronic pelvic pain among women: an updated review. Pain Physician 2014; 17: e141-147.

The Sex Series – Part Eight: Female pelvic floor dysfunction (1 of 2)

Chronic pelvic pain (CPP) in women is staggeringly common, with incidence ranging from 5.7-26.6%, depending on the study. CPP is marked by intermittent or constant pain in the lower abdomen or pelvis, lasts at least six months, and is not associated directly with menstruation, pregnancy or intercourse. Mast cells are known to be involved in the inflammatory processes of these conditions and are therefore linked to CPP.

It can be caused a wide variety of conditions that affect organs or structures in the pelvis, including endometriosis, inflammatory bowel diseases affecting the lower tract, interstitial cystitis, ovarian cysts and hypermobility type Ehlers Danlos Syndrome (HEDS).  Over half of women with CPP report chronic bladder pain, for which interstitial cystitis is a common cause.  Interstitial cystitis is widely accepted to be a mast cell mediated disease.

Despite the frequency of CPP, many exploratory surgeries to identify the cause find nothing (28-55%). Chronic pain from these conditions alters the way the sensory nerves in the pelvic cavity send signals to the spinal cord.  This in turn disrupts interpretation of pain and sensation by the nerves, creating more visceral pelvic pain.

Pelvic floor dysfunction (PFD) affects about 26% of women with CPP.  This dysfunction can cause embarrassing and disabling symptoms, including urinary and fecal incontinence. Pelvic organ prolapse occurs when organs such as the bladder move out of the correct position and impinge on other structures, such as the vagina. Pelvic organ prolapse can be called by pelvic floor dysfunction and it can cause pelvic floor dysfunction.

Sexual dysfunction affects 15-65% of PFD patients. PDF interferes with correct function of a number of muscles, including the levator ani, which hold urogenital structures in place and allow them stretch and contract during penetration and orgasm.  Patients with pelvic organ prolapse often feel a bulge pushing against the vaginal wall that interferes with vaginal penetration.  Vulvodynia, vestibulodynia, vaginismus and painful intercourse are commonly seen in PFD.

In PDF patients, muscles in the pelvic floor can be hypotonic (not tight enough), hypertonic (too tight), or have normal tone. Hypotonic dysfunction is more likely to cause incontinence, bladder symptoms and pelvic organ prolapse.  Hypertonic dysfunction is associated much more with pain and sexual dysfunction. Reduction of the high tone is necessary to reduce pain.


Bortolami A, et al. Relationship between female pelvic floor dysfunction and sexual function: an observational study. J Sex Med 2015; 12: 1233-1241.

Hartmann D, Sarton J. Chronic pelvic floor dysfunction. Best Practice & Research Clinical Obstetrics and Gynaecology 2014, 28: 977-990.

Espuña-Pons M, et al. Pelvic floor symptoms and severity of pelvic organ prolapse in women seeking care for pelvic floor problems. European Journal of Obstetrics and Gynecology and Reproductive Biology 2014, 177: 141-145.

Ramalindam K, Monga A. Obesity and pelvic floor dysfunction. Best Practice and Research Clinical Obstetrics and Gynaecology 2015, 29: 541-547.

Graziottin A, et al. Mast cells in chronic inflammation, pelvic pain and depression in women. Gynecol Endocrinol 2014; 30 (7): 472-477.

Ahangari A. Prevalence of chronic pelvic pain among women: an updated review. Pain Physician 2014; 17: e141-147.


I have a bad tooth. It needs to come out. The original plan was to have it removed in an OR so I can get twilight sedation but my insurance doesn’t want to pay for it and I’m left with having to cobble a plan together myself. I called a number of oral surgeons and no one wants to give me anesthesia outside of an OR. So any kind of general anesthesia means OR, which means a several thousand dollar bill from my insurance. No dice.

I have had dental work with local anesthetic and it’s not ideal but it’s okay. I premed heavily and then it takes a day or two to squelch reactions. It’s not super comfortable but it’s not life threatening and fortunately my laundry list of past procedures means that I have got pain management down to a science. I called my doctor and he agreed that using local sedation is fine if I premedicate. He is very good at giving advice for procedures and talking to providers that aren’t familiar with me. Great. All systems are go.

I have been a patient at my current dental office for about half of my life. I call them and they schedule me to have my tooth removed. Around this time, my dental pain went from sucky and uncomfortable to my entire face and all my teeth hurt and the pain is making me nauseous. Then the long suffering secretary at the dental office calls me to tell me that the dentist won’t remove my tooth with local anesthesia. They also won’t fill the cavity to make it more comfortable until I can figure out how to get this removed.

I talked to the dentist at length and will spare you the gory details of our exchange. I now had to find someone who didn’t know me who would agree to remove this tooth with local anesthesia quickly because the pain was awful. My entire face hurts and I’m reacting and it’s painful to talk, eat and be alive, and I’m terrified it will get infected.

The dental office at my hospital eventually agreed to do it with a local on a day when my specialist will be on campus in case anything goes wrong. In two weeks. A filling would be the same wait. So I’m getting it removed in a week and a half and while I am medicating to deal with the pain, it still hurts. It hurts a lot. I have had bowel obstructions and several surgeries and a million painful tests and good grief does this tooth hurt a lot.

I am so much better than I was a year ago. I can eat solids and exercise and travel and I’m not constantly riding the line that demarcating when I need epinephrine. I have made so much progress. But damn if it doesn’t feel like I am one bad day from losing all these gains. One bad tooth, one obstruction, one flu, one slip on an icy sidewalk. It wouldn’t take much to be right back where I was. Almost nothing.

When I have described my body as strong, it has never felt like the right word. Enduring, maybe. Durable. Not strong. Things that are strong and robust can withstand damage and still work fine.

But some things are not meant to be strong. It is not a defect, but an intricacy. A byproduct of artisanal process of craftwork. Not a mistake.

All beautiful things are fragile in some way. Marble cracks, pictures fade, buildings burn, people change. Beauty is a moment, the coalescing of so many things to form this fleeting arrangement. It is the impermanence that makes things beautiful.

My body has survived impossible things. It has recovered. But it isn’t strong, even if I want it to be, and saying that it is because of one good year feels like a lie.

Ill fit

I haven’t been posting as much of my personal writing because I am working through a lot of things.  It is hard to think about and hard to write about.  2015 was an incredible and powerful year for me, in both good and bad ways.  It seems impossible that all of the events of 2015 are bound together by time.  It was exhilarating and triumphant and horrifying and so, so costly.

I am very good at minimizing and compartmentalizing, especially when it comes to my own health.  My health care is like business for me.  The actual process of managing my physical health is stressful and difficult but it has never been the hardest part of this experience.  That hardest part is all the things I feel like I lost. No amount of struggle can force those into discrete pieces to be boxed up and pushed aside.

The loss of those things hurts more now that I am more stable and things are less emergent. I am no longer living in one continuous crisis. It has given me some distance to reflect on my life and my health and all these plans I used to have.  I used to write about them every night before I went to bed, quick notes on moving toward a goal or long essays on all the things I wanted to do.  Then I went to sleep one night and woke up the next day and none of those things ever happened and I stopped trying to make them.

I think a lot about the life I used to have.  But for the years in between, it is, in many ways, not terribly different from the life I have now.  Every day, it feels more and more like I was never the person who wrote those journal entries.  I remember her, but that’s not the same as being her.  I don’t even know when she left.  A new season, then two, and suddenly it has been seven years since that girl even existed.

I’m trying to pick up these pieces she left and recraft these dreams, to remember the way they made me feel.  I am trying to fit into the space I occupied before I got sick and I just don’t anymore. It’s like forcing something into a place it doesn’t belong, hitting it hard with the flat of your hand until it splinters and your hand hurts.  Anything can fit if you hit it hard enough, but it will never be whole again.

February 29 was Rare Disease Day.  I wanted to write something positive because I’m a very positive person and because I am hopeful and I want people to be hopeful, too. But the truth is that every sick person has been traumatized by their disease and there will always be days or hours or moments when they feel that keenly.  We can overcome and live good lives but this history follows closely and it takes very little to run your mind over it.  Sometimes it is hard to get out from under that.

I thought all day about a story that could make people understand what it means to have a rare disease, to see what I see, but I don’t think that story exists.   There is no rare disease story, just like there is no systemic mastocytosis story, or Ehlers Danlos story.

There is only my story. So that’s the one I’m telling.