Day 243. Detour into Litrachoor.

I know this blog is about pain and disease and how I SUPPOSEDLY have only 255 days left to live, but assuming that is true, literature truly may be the most relevant topic.  What better way to spend my time than reading?
If you knew you had less than a year left to live, what books would you choose to read?

My friend Scott sent me a surprise present this weekend, a great anthology, Best American Non-required Reading 2011, edited by Dave Eggers, whose book A Heartbreaking Work of Staggering Genius I fell deeply in love with five or so years ago when I was on a memoir craze and teaching a memoir course.

I have been familiar with the Best American Anthology Series: Fiction, Poetry and Essays, since the 1980s, when my sweet stepmother, Marilyn was sharp enough to notice I had fallen in love with writing and began giving them to me for Christmas.  If you don’t read much else, reading these books is a wonderful way to find out what is good about writing, as well as what has been happening in the recent world.

I wasn’t familiar with this very post-post modern genre, the Non-required Reading Anthology.  The pieces I’ve read so far include the Best American Fax, one from Don De Lillo (to an author in an interview, because he doesn’t do email, so this is his representative communication; he discusses topics such as Religion, Paranoia and Discontent, The Freedom to Write), also Best American Memos in the Wikileaks Revelations, and Best American New Band Names (including Dale Earnhart, Jr. Jr., Guantanamo Baywatch, Organ Freeman, etc.)…and so on.

Each of the entries lights up my brain with a snowstorm of ideas.  I am reminded of how little of what I read truly does that.  Guillermo del Toro writes the Introduction to the volume.  It’s hard to use the verb write for what he does.  I really don’t know a word that would credit the kind of writing del Toro does, so gorgeous, so smart, yet not so difficult that it would turn people off.  He starts, “‘ONE OF MY TEACHERS LIED TO ME at an early age. I didn’t know it back then, of course, but she lied nevertheless. I was in third grade in a private Jesuit school and my teacher explained the role books played in our lives: ‘They contain all the answers,’ she said. And I believed her.”  Talk about your great first sentences!  I’m hooked.

Then he goes on to analyze the role books have played in his life, more as blissful providers of mystery than of simplistic boxes of answers.  He tells of the “joyous days” when he read a book a day (yes! I remember those too!).  Telling how books speak to him, he explains, every time he starts a new project, directs a new film, or writes one, he rearranges the books on his shelves.  This is no small project.  Long ago, he moved from one house to another across the street: He made a library of what was left in the shelves in his seven-room home full of books he had saved since he was a boy.  He needed another to fill. Rapture! So as he rearranges the books on the shelves, they speak to him, he reads through them, looks at passages and images, and he gets ideas for the project.  It writes itself.

“Books,” says del Toro, “are objects of great power and reservoirs of great magic.”  Those such as Bleak House or El Aleph, says del Toro, “are grimoires,” or books of magic, “and every time one of these books is opened, a tacit ritual takes place. The book reads you back, it scrutinizes and probes the limits of your language, the cadence and music in your soul.”  Thus, the book finds its perfect reader in you.  The magic takes place and curiosity becomes the goal, not the fire to quench.  That’s why his teacher was a liar when she said that books have all the answers. We don’t love them because we’re looking in them for answers! Au contraire, indeed, mes freres. We love our books because they have mysteries.

My goodness.  I fully intended to launch into a debate here on the merits of Dave Eggers (of A Heartbreaking Work of Staggering Genius fame…but also of What is the What? Fame).  Much could be said there, but after my exultation of del Toro, I hardly feel like saying more than read the former, skip the latter.

Definitely get The Best American Nonrequired Reading 2011.  It’ll take your mind off of all that other stuff in your head.

del Toro, Guillermo. “Introduction.”  Eggers, Dave, Ed. (2011-10-04). The Best American Nonrequired Reading 2011: The Best American Series. Houghton Mifflin Harcourt. Kindle Edition.

Now here’s a question for all of us, readers.  If we were to make just such a post-post modern anthology of non-standard entries, what would you nominate to be included?

I know I would suggest two bloggers’ work:

Water Literacy +

Confessions of an Analytiholic

How ’bout you?

Day 246. Weighing the Pain Scale.

One of my favorite bloggers, an amazing college student, writes Simple and Well, a blog about her experience with Hypermobility Ehlers Danlos Syndrome.  It differs from the type I have (Vascular); each of the types shares the main symptoms with the others, the identifiable ones being hypermobility, skin fragility, joint pain, etc.  My symptoms of hypermobility appear on a small scale (causing joint pain), while the activities of daily living cause this blogger’s joints to sublux, or to come out of their sockets.  Rolling over in bed, for example, may cause her hip to come completely out of its socket.  Her shoulder, she writes, has the tendency to “pop,” as do her ankles.

So what does she do?  She tapes up her loose joints for support and goes swimming to get a good workout.  That is one tough young woman.  She is my current hero.

Mosby Pain Scale

Last week, she wrote her own version of a pain scale.  If you haven’t spent much time in the hospital of late, you may not be familiar with the Mosby Pain Rating Scale that incorporates the McCaffrey 1-10 scale numerical reference with words along with the Wong faces scale (image right). I’m particularly fond of the faces of misery that accompany the numerical scores for those of us for whom numbers are particularly meaningless.

The pain scale is a good idea as an attempt to quantify and measure pain empirically, when viewed from the perspective of the nurse, doctor, or pharmacist.  One must measure the pain to treat it. However, from the perspective of a patient, those faces of misery seem laughable. How can they possibly measure pain, an entity that is unquantifiable and incomparable from person to person.

I’ve often thought about this when I have confronted those infernal frowning faces in the hospital or the doctors’ office. How does mundanity of my daily #4, “Hurts a little more,” or on the McCaffery Scale “Moderate,” have any mathematical, or for that matter existential association with the things-are-getting-worse-ness of a #6, “Hurts even more” or on McCaffery still “Moderate.” In fact, I’m still “Moderate” until I am a #7 on McCaffery, which is a long time before a medication decision might be made.  But by the time I am a #7, it may be too late to take a moderate dose of medicine because it is important to treat pain before it becomes too serious; otherwise, it is very difficult to manage.

The rating system becomes more complex because sometimes my leg (where I had an aneurysm rupture) may feel like that face above the #6 “Hurts Even More,” but the rest of my body is pretty okay.  I can manage having that pain better than some other days.  Thus, I can manage taking less pain medication than I would take on a day when I was feeling a #6 AND every joint in my body hurt on top of it.  I wish there was a #6+ or a #6J-with feeling.  That, to me, would have some real empirical value.

Still, I was pretty sure learned from my venture into pain research that I am a big, fat baby. If I were to follow my favorite blogger’s pain scale, I believed that the numerical scores I would assign myself daily would be dramatically lower.  I quite frankly assign pain scores the way America assigns grades on English papers: in a ridiculously inflated fashion.

I have spent the last week or so wondering what that inflation says about me. Do I misunderstand the pain scale? Am I malingering for attention? Love from doctors?  More pain pills? I worry about these things constantly.  I am a baby, no question.  If I just powered through this, I would have less pain.

So then I had a epidural injection on Friday. I absolutely detest getting them, as I guess anyone would hate having a needle poked into a vertebra and into the nerve.  So the doctor uses an X-ray machine to find the nerve precisely; then he injects me with enough local anesthetic so that I don’t jump off the table.  Then, he stimulates that nerve to make sure he has the one that is causing the leg pain—so I feel not exactly pain, but just sensation all the way down my leg and even in the foot where it hurts.  Then, he injects tons of anesthetic, followed by cortisone (which will hopefully block the pain for a few months, until he does it again).

The payoff for the misery of having the injection is standing up and walking off of the operating room table:  about an hour of pain-free walking around.  It hurts like hell in my lower lumbar, where he poked me, but I don’t care.  My leg doesn’t hurt for the first time I can remember.

My doctor’s office building is in Bethesda, kind of a tony address; right across the hall from his office is the snack bar, where one can find the expected coffee and candy bars.  However, the reason we drive 45 minutes to Bethesda instead of seeing the doctor five minutes away in his D.C. office is that the Bethesda snack bar also sells Swarovski crystal barettes and hair things, as well as designer knockoff purses (not that I would ever buy one of those).  So I have about a half hour to walk around this store without having to figure out a way to stand on one leg, or lift it up bent and rest it on the other.  The important thing is, I never even think of my leg this whole time, not how my shoe feels, or whether my sock is cutting into me, how long I have until the pain pill, not one thing. When this happened, I realized that I am not exaggerating when I say that the pain never goes below a #4, even with three narcotics.  When I had a complete nerve block with anesthesia, on an otherwise pretty good day, well now, those pain faces smiled for quite some time.

“Loosey Goosey,” the blogger of Simple and Well, tells me that someone has gone to some trouble to write a better, more sensible comparative pain scale.  This one has some good points; however, I think it is skewed to the lower side.  According to this scale, #5 is pretty intolerable (like trying to walk on a sprained ankle). Uuummm…okay.    Call me inflated all day long, but that just has a lot more seven-ness than five-ness, for me, folks.  Some of the distinctions made to explain the pain, though, are so clear and so apt.  For example at #6, it becomes difficult to hold a job.  Yes!  So clearly it does.

Without a doubt, if the aim is to standardize the description or definition of pain, then more work remains to be done on standardizing the pain scale.  Being able to describe or define your pain may seem esoteric to you, but if you’re in the hospital, in pain, you may find yourself at the mercy of healthcare givers who are suspicious of your motives.  One thing to keep in mind (I learned this thanks to Cathy, who sent me a very interesting journal article): beginning in March 2012, hospitals will receive large amounts of Medicare and Medicaid funding based on the patient evaluation scores they get on pain control.  That means that the government is requiring hospitals to do a good job at controlling your pain in the hospital.  I’m not even on Medicare, and I’ve been surveyed many times already.  So, whether you use the happy and sad faces, or whatever numerical score you devise for yourself, keep in mind, rating your pain may be vital to you in the future.

Day 249. The Heartbreak of Presbylarnyx.

A few years ago back around 2003, when in an iPod listening frenzy we got rid of our cassette tapes [and our stereos] Margie and I had a listening party. One I played was my college radio show. My radio handle had been Anne Archy—I stole that from a sign in NYC and thought it was super cool, but was so not down with what it really meant.

In fact, I remember a drunken college argument with Greg Hansen in which neither of us could let the issue of anarchy go. He argued that a world without government would be superior to the present one.“Yes, hun,” I disagreed (we called each other hun), “but who would take care of the roads?”I thought myself so superior for taking up something so mundane, but, perhaps I was missing the point? Greg gave up in frustration.

But I digress. So, I’m playing this tape of my radio show, and the break between the songs comes up, and on comes my voice: “That was UB40’s ‘Red, Red Wine’ going out to Megan Hudson….”

Margie is stunned.“That’s you?”

My voice had already changed a great deal by then.Granted, we all sound a bit different after twenty years, to be sure, but this difference was between, say, Debra Messing and Brenda Vaccaro. Either voice would be a vast improvement on mine, granted, but my point is the vast difference in range.

My voice felt gravelly almost all the time, as though I had swallowed a handful of asphalt.And if I didn’t bring my customary cup of tea, speaking to my students for three hours could be pure hell. It felt like a perpetual frog in the throat. I assumed the cause was post nasal drip, from allergies and chronic sinusitis. When I resolved those problems to some extent, my allergist said that sometimes the medications for asthma could cause one’s voice to be hoarse.

Indeed, I learned the hard way one day way back in 1999 giving a lecture to the whole Physical Therapy Assistants Program, that asthma could interfere with one’s ability to complete a full sentence (I still get that some days).The first day it happened, though, my lips turned blue, and I had to go in for an emergency breathing treatment.

These days I’m such an old pro at not breathing, I have a nebulizer under my bed so that I can use it without fully waking up if things get really awful (though I proudly will say I haven’t used the thing since early 2011, long enough ago that I should probably refill the meds in case of a midnight emergency because they may be expired; having them is a helluva lot better than awakening an allergist and then having to go to extract my wheezing self on a midnight jaunt to Walgreens. Ugh.).

Anyway (how I do digress), once I realized it wasn’t my sinuses or my asthma, my allergist (the amazing Henry Fishman, a doctor I can recommend as one of the good guys in the business, incredibly smart, and very kind as well).He sent me to a voice specialist, after ruling out any of the major issues. Maybe this guy can help by doing some voice work with you, he told me.

By this time, ten years had passed since my original voice problem: it was summer 2009. So the ENT, “Dr. Quacker,” a voice specialist I went to at a major University Hospital named after a dead president, was a disappointment to say the least.

My first appointment was very exciting. Apart from the singularly unpleasant experience of the scope shoved up my nose and down my throat, it was pretty cool to see my vocal cords projected on a 48” screen so close I could touch it. He asked me to phonate various sounds: /ee/, /ay/, /i/, etc. The evaluation continued at great length with a speech therapist who I never saw again (nor was speech therapy ever suggested or discussed with me).

Dr. Quacker finally went over the results of my exam, saying that my vocal cords do not meet, which causes a gravelly, breathless sound, and that the fix for that would be to inject them with fat (unfortunately, not four or five pounds from my gut). As a member of PeTA, I don’t want to think about the lab rodent who sacrificed his or her life for my vocal vanity. I agreed to do something flatly against my beliefs hoping fiercely to be normal.

I should say this was only eight months after my aneurysm, so I was prepared to do anything to return to normal. I still thought I could do some procedure, somehow and be normal again. Sometimes I still do. I still think that this is all a joke and all this foolishness will stop and there will be a surprise party for me one day and I will get to go back to work and everyone will have a big laugh about what a nutty rib-tickler all of this foolishness has been.

So when I wrote a check for $800 for the part of the procedure that wasn’t covered by insurance (namely, the fat from the poor creature—a considerable sum for me) and the result was that my voice sounded WORSE, I was livid.

It goes without saying that I returned to Dr. Quacker to let him know how upset I was. It was one of those days when I could do nothing but be polite, and in my most humiliating 1950s June Cleaver way, say, “Well, Dr. Quacker, I’m not very happy with these results, I can tell you. And I’d like to have some restitution, or else”

This is one of many times in my life when I have cried tears of rage…on the way out the door…because was I born a sorry bird from the suburbs with no street savvy, no moxie to stand up for herself and tell someone to go straight to hell. This is where it would have been immeasurably helpful to break nasty and say, “Look fatface. You fix this or I’ll….._________” You fill in the blank there. Any number of threats or pseudo-threats would have sufficed. Instead, I had to bite the insides of my cheeks so that the humiliation didn’t extend beyond his having to extend the tissue box to me.

His response was predictable: “Oh, I guess I should have told you. I mean, what you have is a Connective Tissue Disorder and the Vocal Cords are Connective Tissue. So, you know, the fat might not work on them at all. There’s no way of knowing what might happen with your disease. You understand. It’s the fault of the genetic disorder.

Um. I should have told you?

You understand? I really love that one. No, I don’t understand, lunkhead, or I wouldn’t be sitting here boohooing in your chair.

“I’d like to have a refund,” I blurted out, like some sort of a sick cartoon character at negotiation remediation school.

“Oh no.” He actually laughed. “Refunds are impossible! We have to pay for the fat. It comes from a lab.”

So I had no idea what to do, other than to review him honestly (read: scathingly) on the web—and right here on this blog—I mean, it wouldn’t take a detective to determine his identity, based on the information I have given.

So that was the end of voice work for me forever, until I had to visit an endocrinologist for my Adrenal Insufficiency. (My endocrinologist is another fantastic doctor, Jason Wexler at Washington Hospital Center. He gives the finest thyroid palpation on the planet; I give him my highest recommendation. He sorted out my complex case with thyroid and adrenal gland issues, which was tall order. Endocrinology is such a difficult specialty, and Dr. Wexler is outstanding). Dr. Wexler strongly suggested that I give the voice work another try. He knows of another ENT who has a good track record with helping people with unique and challenging voice problems. Because he is such a good doctor, I thought I should follow his advice.

What was interesting about Dr. Nazaneen Grant at Georgetown University was that she performed the same exam with the scope down the nose and saw the same problem with the vocal cords not closing as the previous doctor had seen almost a decade  before. However, her conclusion was entirely different.

She prescribed a short course of speech therapy, shrugging her shoulders about why anyone would ever have wanted to do fat injections. Possibly he might have thought that fat would have made the vocal cords connect, she surmised, but she explained why (without voice therapy) that would never have worked.

“Presbylarnyx is my diagnosis,” she explained, which is understandable, as Greek roots in words make sense. Here, the Greek word presbys (πρέσβυς), refers to “old man,” and larynx (voice) is clear all by itself. Thus, she called me an old-voicer. Thanks ever so, young whippersnapper! Nice as she was, I felt like telling her to go straight to hell. But seriously, the age that “presby” refers to might be premature aging thanks to my Connective Tissue Disorder.

Lucky me! That is what makes me sound like crap, along with Breath Control issues (which make me run out of steam in the middle of a sentence, and sometimes in the middle of a word—particularly when I am excited, walking, running, or tired). This is complicated by what seem to be my Chiari Malformation issues that I am just learning about. Whatever the cause, speech therapy is already helping.

That is good news for me, but maybe not so good for my neighbors. Speech exercises didn’t seem to be bringing about any improvement for the first two weeks, not until we started working on volume. These are exercises that really force the vocal cords together.

For example, I say /ee/ /ee/ /ee/ (loudly) then breathe, ten times. The phonation has to be done at between 80 and 90 decibels—that’s quite loud. To the neighbors, it must sound like I have captured a baby seal and am keeping her as a pet in my living room.

The next exercise is even funnier. It reminds me unfailingly of my friend E-beth, who has always adopted a joking voice at about 100 decibels; this volume would thrill my sweet speech clinician on Tuesday mornings, but it is not an inside voice. Still, just for giggles in a restaurant or something, E-beth’ll just shout out, “It’s fat free half-and-half” or something. It’s funny, but you have to be there, I guess, to appreciate it.

So as I do my exercises, I am channeling E-beth. My second exercise, at 80-90 dbs, is to read a list of words:“Good Morning! Thank you! Happy Birthday! How are you! Ouch! No, thank you! Not today! Yes, Please! Let’s Go! Help!” and so on. Most of the phrases are positive, but others are not. I envision that the neighbors hear me shouting “Ouch!” and “Help!” and worry that I have fallen, or worse. Or maybe they think E-beth is visiting, and we’re playing with the baby seal in the living room.

The good news is that, while I don’t have back my singing voice, I do notice a change, my speech therapist gave me a great response today–and even my physical therapist noticed the difference.

So, here’s one cool exercise to help bring vocal cords together, in case your voice is froggy (clearing the throat is a less than ideal method).Take a swallow of straight bourbon (or whatever it is you’re drinking) and immediately out of that swallow, make the /ee/ sound and hold it as long as you can.The sound should be a kind of gulp-eeeeeee.That forces you to start phonating with your vocal cords together. I tried it the other day on the phone when I had such a gruff voice that I couldn’t get rid of it. My voice came back well as a result of the exercise—I was so surprised that it actually worked on the spot! So give the process a whirl. Who knows? It may work for you too.

Now we all know I will likely never get my radio show voice back, but then again I was kidding myself and everyone else about the whole Anne Archy thing, too. Not only that, but who could hear me anyway to do a comparison now that nobody has cassette players anymore? That sad fact didn’t stop me from keeping my radio show on cassette, along with all the mix tapes my brother made, and the mix tape Scott made me the summer we were 17, and the mixes Tim Jones made me, too. Don’t ask me why. That’s just how I am.

Day 261. Touching the Ceiling

Sunrise at Pollution Pond

Just got back from art class tonight.  My painting looked so much better in class–but don’t blame me for the colors:  she only let us use two!  Think of it as sunrise at pollution pond.

Blame it on my mood.  I’m pure nerves about the costs of my healthcare today.  When will I touch the ceiling….and crash to the floor?

One reason I love about writing creative non-fiction, such as the work in my book and the writing in this blog, is that it involves a certain amount of research.

So a research question is embedded in the topic I pose myself today: What is the ceiling on your health insurance, Heidi?

                 I find myself stuck on this one.

Yet, it isn’t as though my poor little hypermobile fingers won’t type the question at Google‘s receptive, blinking cursor, or that I can’t compose the precise search term.  Rather, it is that I can do it spot on, and Google will respond in 0.040126678 seconds.

 I don’t want to know the answer.

While I talk a good game at being poor at mathematics, I could in seconds have a running ticker in the lower left-hand corner of my “screen,” that is to say my personal visual field.  Then, every minute, waking and non-, for the rest of my existence, a dwindling balance would be ticking away there, like the doomsday clock does in Times Square.

Oh, I have a days-left clock going already (thanks to Dr. Dimwit of the “You have 261 days left to live fame”), which I try to grey out for my own sanity.  But the fact is that I write a $512 monthly health insurance premium check, and, again, math disability be damned, the figure turns red as I write it.

How can this possibly be, it wants to know, that you can be paying this amount, exorbitant to be sure, and it’s not enough, until we realize that you’ve had three MRIs and two cat scans in the past four months. Let us devise a broad estimate that each of those studies costs $1500 (without taking into account the cost of the radiologists’ time to read the exam and report on it).  So that’s $7500.  Add to that a $12,000 hospital bill for December (again, without any of the doctors’ bills). Not to mention that is one of only four hospitalizations in 2011.

The good news is that while I am not a cheap date, 2011 was not a bad year as they have gone for hospitals. Compared with 2008, for example, when I spent some six weeks in the hospital, 2011 was a banner year. In 2008, my hospital bills totaled more than $50K.  Are you keeping track?

I’m not. I have already purged these numbers from my poor, diseased mind.  Good Lord!  There is only so much a chick can tolerate, and when you supposedly have 261 days left on the planet that type of data is disease.

This reminds me, actually, of the era when I first had a credit card, back in the early 1990s.  If you don’t know me, then let me apprise you that I just paid the last one off last year.  That’s twenty years of paying off balances. It took that long to rebound from my original accounting method, which involved my putting a figurative hand over one eye daily or so whilst making large purposes and shouting loudly “Oh, ah just can’t possibly have reached mah $25,000 limit yet!”  My goodness, I was expert at that shout (and loud could be so relative, particularly when I was unaware they raised the credit limit a few times).  But I perfected it at a time when my therapy was conducted via the retail channel.

It wasn’t my fault.

So I can see myself with these medical bills too–oh, the fault is on the insurance company.  So what if it’s $50K? Or $100K?  Who cares?  I don’t care what the limit is because I have an expiration date–so I can leverage my limit against the insurance limit, see?

I realize how dumb I sound here. Irony. Measured Irony.

My only hope, actually, may be the threatened “death committees,” which reportedly will vote (supposedly frequently against) benefits to those who have situations like aneurysms and genetic diseases.  They will make the decision for me.  I won’t have to worry about whether I can pay for the insurance.  I simply won’t be able to afford for my treatment out of pocket, and out-of-pocket may become my only option.

I guess that it’s sort of like pollution, though.  I sort of have to look at it like the repulsive person my age that I’ve become:  What does it matter if I’ll be dead by the time it  becomes unavoidable?

Life is a picnic, at least for now.  I think I’m going to eat desert first from now on.

Day 284. It’s Just a Cervical Collar

Dr. Francomano said that.  She said, so you have this Chiari Malformation.  There are various treatment options, but one of them is wearing

Looks comfy to me!

a cervical collar, which can really help with all the symptoms you’re having.  “It’s just a cervical collar,” she said.  She really said that.  So I pictured the kind a person who has whiplash wears, maybe. It even looks comfortable with all that padding and is only $5.95 at  Now that’s affordable fun!

I read a really cool medical literary journal this weekend called Ars Medica; in one graphic story, a social worker counsels her patient to expect the very worst outcome in health situations so that you can be pleasantly surprised when something good (or even marginally better) happens. I probably should have considered this excellent advice before I made an assumption about the cervical collar. The real version is below.

Fear not:  You will never see me wearing the Aspen Vista device.

That is, you won’t see me wearing it, unless you visit me at home, and it turns out to be fantastic.

Curative or torture device?

The Aspen Vista Torture Device–eh, Collar should manage a cavalcade of symptoms.  Here is an oversimplified description (forgive me if you are an expert or sufferer and I have not done it justice):

Chiari Malformation is mainly a structural defect in the cerebellum, the part of the brain that controls balance.  This defect can result in headaches, fatigue, and muscle weakness in the head and face.  From all that can come difficulty swallowing and voice hoarseness.  Further are Dysautonomia, that is, exercise intolerance, intolerance to temperature extremes (heat for me), and the related Postural Orthopedic Tachycardia Syndrome(POTS) (a sort of trouble one can have with the rapid contrasts between sitting and standing).  This, in other words, is a veritable feces storm of fun.

In my experience, my Chiari Malformation symptoms started, of course, with the headaches. The headaches serve as the umbilicus of my ever-cycling Diaspora of symptoms.  But, though I was not aware of any of these maladies other than the migraine, Chiari Malformation truly began to distinguish itself from migraine and Ehlers Danlos Syndrome when I was finishing graduate school and still teaching.  I would take a shower, getting ready for work, and (not understanding my heat intolerance) as soon as I was done putting the lotion on my legs—or sooner—I felt faint and would have to lie down in bed with a fan blowing on me, while the room spun around.  Frequently, by the time I cooled down, my head started to pound like holy hell was going on inside it.  Sometimes it took an hour or more for me for me to be able to stand.

Ah, hindsight.  The more I look back, the more I realize how unnecessary all that stressing out was. I’m so sorry if I was ever short with my coworkers because I had “so much to do.”  Who really cared?  My Type A personality would rile me to an insane froth.  I’d be shouting at myself, “Get up you lazy sloth!” Very helpful. Yet, my mind could not shake the truth that in one hour 27 freshman comp students would be dozing in their seats, and while they were not particularly interested in my class activities, they would raise holy Hell if I didn’t show.

It is true that intense pressure applied without release can cause a certain implosion, but I try not to spend too much time worrying that I caused my own aneurysm with stressing out over the way these terrible symptoms were causing me to miss work or work poorly. I realize now that I went about twenty years without taking a deep breath. I’m just learning a week at a time not to bash Heidi.

Are you stressing out?  What are you stressing out about?

If you’re stressing out and yelling at yourself….why?

If you knew you had only 284 days left, how would you spend the morning with your coworkers and clients/students?

Please, do go to the movies instead of stressing.  In fact, go see Albert NobbsIt’s fantastic.

Day 286. You Can’t Study For a Genetic Test

So, I’ve been talking about my examination by Dr. Clair Francomano at Greater Baltimore Medical Center.

But. so. what were the outcomes?

Ehlers Danlos Syndrome (EDS) Testing (if you’re thinking, wait, wasn’t that her firm diagnosis already? Not genetically:  Dr. Francomano’s first recommendation is genetic testing for Hereditary Connective Tissue Disease (HCDT–these diseases include EDS, which is what we assume I have).  Thus they are going to analyze the COL3A1 gene for abnormalities.  That involved sending 2 vials of my blood to the University of Washington Collagen Diagnostic Laboratory. Academic Laboratories don’t work at the speed of your local medical lab that turns over your blood chemistry panel overnight (or in an hour in an emergency).  My results will take eight weeks or more (my academic friends will appreciate this breakneck speed).  Results are expected to show that I have EDS (and which variety, particularly; we assume the Vascular Type (VEDS) because of my aneurysms); or results may show that I don’t have the disease but instead have Fibromuscular Dysplasia (FMD), which is another disease that causes one to develop aneurysms — or, as the doctor suspects, both. By the way, if you’re interested, here is an abstract of a paper I just found that Dr. Francomano co-wrote, theorizing a new EDS version that includes both VEDS and FMD. Oh joy. Alphabet soup.

Having read that monstrously long more than thirty-page questionnaire I completed, and scoured my medical records, the doctor and her assistant saw that a 2010 body CT Angiogram had shown some “dilation of the aortic root,” not a good sign in a person with my aneurysm issues.  None of my fairly well renowned doctors had followed up on those findings. Dr. Francomano did, though.  There was something comforting, oddly, in her suggesting (prescribing) an echocardiogram.  And suggesting is what she does.  So kindly.  I couldn’t, wouldn’t, possibly say no.  She wants the echocardiogram to rule out [further] aortic root dilation, mitral valve prolapse, or any other structural heart abnormalities.  I think I’ll be fine.  But at this point, what else can I say?  That I am terrified? (I have an appointment on Friday Feb. 3.).

So of course I am to continue to have my regular CT Angiogram screenings at Johns Hopkins University at least annually, or twice annually if there are changes.  (Those are the body scans of which I have spoken to my friends.  In about 120 seconds, the CT scan machine scans my body and makes a perfect 3-D model of my body’s arteries below the neck.  It is incredibly cool.

However, Dr. Francomano notes that none of the previous scans have included the head and neck, and she asks that future screenings correct this.  I feel vindicated!  I have asked this question repeatedly at my screenings….”uh, how do we know that I am not getting a brain aneurysm?”  The answer has been a furtive, “Oh, you’re not a brain aneurysm type.”  And the Carotid Artery (in the neck) is one that sometimes explodes without warning.  It’s critical to monitor for any signs of difficulty, right?  God, I can’t tell you enough how much Dr. Francomano just makes sense(Incidentally, the answer is that it takes so much time and that it may take two appointments and that much more radioactive dye exposure.  Sigh.  Is that a good reason?  Why they can’t just do it all in 240 seconds is beyond me.  The geniuses at Johns Hopkins are sorting that out now — all because Dr. Francomano says so.  She rules.)

Can you imagine how having worries like that all the time weighs on a girl?  You leave the doctor’s office after the CT Angiogram thinking, wow, I’m not an expert in this at all, but they seem to have missed the boat when they didn’t scan my head and neck.  What if I have a brain aneurysm?  What if my carotid artery ruptures (you die within minutes)?  My body has the potential to form an aneurysm anytime, any place, anywhere.   When I asked about why they didn’t scan my head and neck, the doctors waved me away.  I used to cry on the way home from Johns Hopkins visits in Baltimore (trying not to let my parents see), not tears of self-pity, but sheer frustration.  There was nothing I could do.  That sums up my last three and a half years.

So you may understand why right this minute I want to nominate Dr. Clair Francomano for President.  (Except, I take it back because then she couldn’t be my doctor!)

You may also understand why there was no need for Dr. Francomano to add anxiety to the list of diagnoses.  It comes with the territory.

Two more surprising diagnoses came up.  When the doctor entered the room and I greeted her, she said, “Oh, you have a hoarse voice.  Do you have a cold?”  I confessed that I did not.  At one time, I thought I had a sort of pleasing voice (I thought).  I liked to sing. I just have gotten progressively more hoarse over the years; I used to think it was from my sinus problems, but then it seemed a bigger issue, and I had even bigger concerns to worry about, so I rather let the voice problem slip out of my focus.  I did see an ENT at GWU who my allergist recommended.  He promised to fix me, injecting fat into the vocal cords at my great expense.  When my voice got worse, he about-faced: “Oh, yes, I forgot to say that was one of the possibilities.”  My failing voice was another one of those “soft” signs that whatever was wrong with me (I didn’t know what it was) was getting much worse, systemically.  So I just forgot about it.  Denial is a wonderful thing.

Chiari Malformation - a fault at the base of the brain

Dr. Francomano nodded. “Okay.  We’ll take that up later.”  She asked me if I had ever heard of Chiari Malformation.  Indeed I had:  it had been one of the diseases I studied closely during one of my sorties into the medical journals on late, sleepless nights of pain.  Chiari Malformation causes terrible headaches. I wondered if I had it, but the literature said it was so rare and typically found in infants with spina bifida, so I assumed that #1, I probably didn’t, and #2 I would be considered a hypochondriac for ever asking.  However, Dr. Francomano said, “For a number of reasons, I think you do have Chiari Malformation, with or without cervical instability, which is an extremely rare disease, except among HCTD patients–for you it’s quite common.”  Swell.  You can read about the specifics, but it causes a host of neurological problems, including headaches WITH INTENSE PRESSURE BEHIND THE EYES.  Oh gentle Jesus, can I tell you about those, people.  Also, hoarse voice!, dysautonomia (a disorder that has a number of symptoms of its own, including exercise intolerance–I mean that it makes you completely sick afterwards and often the day or two afterwards–something I have had severely for the last several years), intolerance for heat and cold, sleep disturbances, brain fog, and memory issues.  Well, that pretty much describes my last five to ten years.

The interesting thing is that there is a simple, albeit temporary fix:  wear a cervical collar (of a specific kind).  How easy.  Dr. Francomano has practical solutions for things that I love.  In addition, she says, there’s a neurosurgical fix.  I think I may leave that alone.  But the cervical collar sounds workable. My friend John suggests that I try the big gold ones that African Princesses wear, which sounds even more workable, especially if I could have a silver one and a copper one.

So another one she brings up is Occult Tethered Cord Syndrome. I’ll let you read about it, but this disorder is associated with Chiari Malformation: if the Universe graces you with one, you’re likely to win the lottery on the other, too.  Still, for the record I would like to state I show just the very most basic symptoms of the syndrome, gratefully, and none of the ones that involve the word “incontinence.” The syndrome, however, is progressive, meaning that it gets worse over time. Yuck.  Well, so the test, she offers, to see whether indeed I do have it, involves going to Greenbelt, Maryland, having a urinary catheter inserted, and then having it reached around all up in there (and that isn’t even the treatment).  I don’t know which of those options is worst (possibly having to go to Greenbelt…). I just said no-kay!

Not surprisingly, Dr. Francomano was fine with my refusal.  She explained, “Here’s our philosophy: we offer you six or seven options, each of which has the potential make your life 10% better.  Even if you try four of them and they work, that 40% would make your life substantially better.”  I thought that was pretty profound! So even if I skip that bladder business, I’m still headed in the right direction for some improvement.

There are a couple of things the doctors can treat with meds.  She diagnosed my Mast Cell Activation Disorder, which is a situation I already was about 50% aware of; that’s when you become horribly allergic to everything.  That has been painfully clear to me for some time, since I have hives all the time, allergies, my diet is so limited, etc.  Well, Dr. Francomano actually has a drug that I might be able to try, Gastrocrom (in liquid form), which if it works, might mean I could add back some foods.  Wow.  Like, what if I could march right down to the Cold Stone Creamery and order a Sweet Cream with Reese’s Cups?  NIRVANA.

However, toward the end of the four hours, I was overcome with the sheer number of congenital abnormalities I have.  I had to sit back in my chair and take a deep breath so the panic attack that was rising up and into my chest could evaporate and I would not humiliate myself.  Then, I thought I might cry.  She had just finished listing so many illnesses, malformations, rare diseases—deformations of my body, essentially, that I just sort of didn’t know how or what I did to get a body that is so diseased, so very different from everyone.

In fact, the whole next day, my eyes were the deepest red, as though I were going to start sobbing any moment.  I didn’t want to cry exactly.  I just felt deeply wounded.  God forbid my heart should pound, though, right? Don’t want to stress my aortic root!  Don’t want that BP up…there’s a delicate balance on those aneurysms!  (Just a little gallows humor, folks!)  When, back in that moment, I told Dr. Francomano how I felt (well, sort of in sum)  she took my hand, looked in my gray eyes, and said, “I’m sorry.”  Just very simply and honestly.  It moves me even now.  I’ve never had a doctor be so attentive and so honest.

So get this. On my way out the door, she gave me a ten-page report explaining my diagnosis and treatment plan. It also explains some basic information about hereditary connective tissue diseases, including the connections between some of these diseases and the many related nutty disorders I also seem to have. When has that ever happened to any of you?  You got the doctor’s report on the way out the door?

Well, I needed it because, as you have probably gathered, the sheer breadth and depth of what she told me and my mom was so vast that it would have been impossible for anyone to remember.

I was a little nervous about discussing my medical history in such detail on the internet, under my REAL NAME as I wrote this.  But then I thought about how much I have suffered with nobody believing me about my symptoms, the illnesses I thought I had.  So I just said, the hell with it.  This is information that belongs out there.  I don’t care who knows it.  And besides, I only have 286 days left to live, right?

And…but…so…as for my panic about being a mass of congenital abnormalities?  I have to comfort myself with the thought that I’m the same mess I was yesterday and five years ago.  It’s nothing new, Heidi. She’s just giving a name to all this angst that has been lonely torment for so long.  Now we all can know about it.  So hooray, I…think.  What do you think?