Monday, February 11, 2013

Not Your Average Ordinary: My Story.... for those who have no idea...

What is normal? Whose definition serves as a standard? Because my life has been far from what most would consider “normal,” while at the same time following exactly what others would consider “normal.”
            For starters, I had an encephalocele at birth: a small sac of fluid protruding from my skull, containing mostly fluid and a negligible amount of tissue. The protrusion happened to be situated for easy removal, and the doctors duly (because the existence of an encephalocele belies circulation and drainage problems of the cerebral fluid) installed a cerebral shunt and warned my mother (whose only experience with mental disabilities came in the form of her sister with Down’s Syndrome, and horror stories from her days as a speech pathologist) that her daughter may have developmental problems, that the presence of the encephalocele may tell of deeper, more serious and less obvious complications, that there is a chance her daughter may always need help feeding and bathing herself, and that she might never walk or talk. 
This is an attempted view of my scar from the encephalocele. Apologies that you can't see it very well, but it's now a tiny lump of bald scar tissue at the back of my head.
            Twenty years later, they still don’t know much about my condition; very few babies are born with it, and half of those who do never achieve an IQ above 83. (I looked it up, and apparently an adult with an IQ of less than 85 has just enough mental capacity for basic gardening and menial housekeeping…)
            My poor mother was on edge for the next few months. Each little sound or quirk I displayed that was the least bit unlike her three other daughters, she immediately questioned. However, I continued to develop normally. (Of course I did… how many of you mothers of multiple children understand that not one infant acts exactly like their older siblings? My mom’s problem was not knowing what a seizure looked like, poor thing!)
            As I grew, I guess the one thing that wasn’t “normal” (or at least wasn’t like my sisters) was the fact that I was very introverted, and very much into reading. My oldest sister Dena was the only one who read a lot and wrote some; Paula and Jamie just went along with her, and did what she told them. It was probably Dena who most inspired my imagination with her own. She was the one making movies all the time, the only one besides me who actually wrote a book. My proficiency with words did not run common through my family. Even before I could read (and though I did not learn to read until I was five), I distinctly remember flipping through one of Dena’s books and making up stories about the illustrations, even though I didn’t have the foggiest what the actual book was about! Years later, I returned to that same book, and had the most intense moment of deja vu ever, as I remembered the stories I had made up when I was four, which were different than the story I was actually reading then.
            I loved reading books. But I never knew why. I would far rather read a book than do anything else. When I started taking literature classes in middle/high school, all of a sudden I knew why I loved some stories and not others. Where for some people those classes would open the world of literature, they did different for me. The world of literature was already wide open; what those classes did was open for me the world of literary psychoanalysis. As if I didn’t already have enough floating through my head, what with my imagination and all, I now had the ability to mentally dissect any work, its characters, conflicts, flaws, and successes. I knew all the terminology, and I reveled in it. I loved stories for their plot elements and their denouements. I liked certain stories in a genre for their plot developments and the language chosen by the author; I disliked others for their misuse of such things. More than anything else in the world, I wanted to become a writer. What those classes also did was to help me become a better writer, too. Suddenly, it wasn’t just about my characters going where I chose, and doing what I wished I could do; suddenly, they had a purpose, which was more than I could say for myself in a lot of areas at the time.
            All this time, I lived with a tube in my head that stretched down past my collarbone and supposedly to my abdominal cavity. I could feel it on my skull, but really had no idea what it looked like. The only scan of my insides I’d ever had was when I was 2 days old. My second x-ray ever was when we began going to a new chiropractor, when I was about 16 or so. It was the first time I had ever seen my shunt, snaking its stark-white way parallel to my spine on the negative. I remember thinking it was a growth behind my ear, one that made it difficult to wear headbands. I knew I didn’t have one on my left side. I knew that when I had a headache, I could massage the “growth” and it would go away.
            When I was nine, I had a mild febrile seizure. I remember everything about that day; I remember exactly what I was wearing: my favorite outfit, because it was purple and had cats and roses on it. (Purple and cats were two favorites of mine, and what girl doesn’t like roses?) It must have been near my birthday, likely around Thanksgiving or so. I was wearing the necklace I had gotten for my eighth birthday, the one that looked like a gold cross set in a gold heart rimmed with faux pearls. I was watching my younger sister Leanne and “oldest” (but still younger) brother Joe play the Polly Pocket board game (which incidentally disappeared shortly after this...). My grandparents were visiting, but leaving then. I remember hearing my Mom call me to say goodbye to them. I remember falling into a stare that I couldn’t break. I remember falling over on the couch---
Then nothing. The next thing I remember is laying on our kitchen counter, while a navy-shirted EMT stood over me, asking for my name. I had a kitchen towel (slightly damp) under my head. I could move, but I couldn’t talk. He was so close I could feel his breath in my face. I blacked out again…
The next thing I remember is sitting on a bed in a hospital room, wearing a hospital gown, while a nurse handed me a medicine cup of orange-flavored “children’s” liquid aspirin. I’ve hated that stuff ever since. Strangely enough, I prefer cherry-flavored. I don’t mind cough syrup. But the orange-flavored stuff tastes like someone fed an orange to a cat, and the cat vomited, and they caught 1-2 teaspoons in the medicine cup.

My sisters tell me I rode an ambulance to the hospital. Of all the times to be unconscious, that had to be it. During my first and only ambulance ride. I am still disappointed.

For a while, we were careful, worried that it might happen again. I continued on with a normal life, still devouring books and such voraciously, unaware that anything was out of the ordinary.
When I was seventeen, I became the first person in my family to be away from home for more than a month. I attended the Excellence in Character, Education and Leadership (EXCEL) Program in Dallas, TX, which lasted eight weeks. I had never been somewhere without my family. Every chance I got, I called them, and even if I left a message, it was at least 2 minutes long every time. I had an amazing time, but I missed my family very much.
It was probably this experience that helped me decide that I wouldn’t be traveling anywhere to attend college; I began looking at online colleges. A program called CollegePlus! seemed to fit the bill nicely: an accelerated program that involved student responsibility and accountability, while at the same time the flexibility to take as short or long a time as I needed between tests. All I had to do was periodically drive out to a designated college to take a credit-by-exam-type test, and in this way, I earned 105 credits in 2 ½ years—the equivalent of taking 3 college courses at the same time for the duration of that time period. In fact, perhaps it was more like 4 courses at a time, because those 2 ½ years also included family vacations, a few trips to Mexico, and an intense experience that threatened to change my life forever.

The year was 2008; I was eighteen, just a month before my 19th birthday. We were hosting another family at our house, so my sisters and I all moved out to our trailer so they could use our room. Sleeping out there on little more than a narrow foam pad, I noticed after a few days that every time I lay in a certain position—normally my most comfortable position—an inexplicable pressure seemed to build in my head, rendering it very uncomfortable. I ignored it, simply avoiding that particular position. I attributed the pressure to the fact that this wasn’t a very good mattress I slept on, and reasoned that it would all go away once I returned to my normal bed. I had to remind myself of this more often, as after a few days, the discomfort increased so that there was not really a comfortable position to lie in at all.
Finally, our guests left, and I gratefully sank into my own bed. To my chagrin, the pain did not subside; it increased. The pain grew so bad, that in spite of taking ibuprofen before bed, I could not lie flat; I had to sleep propped up. Then the pain increased so that as I sat in front of the computer to study or even reading a textbook, I could not focus. It literally felt like a grown man sitting on my head. Not even walking around, closing my eyes, or any other method I tried to relieve the pressure worked. Finally, it got so bad that I threw up from the pain in my head. My mom promptly took me to see the doctor. (Which tells you how bad and mysterious the pain was; the last time I had been to the doctor was probably not since just after my seizure) The physician recommended that we see a neurologist, and the quickest way to do that was through the emergency room. We went right away.
In the ER, they gave me a room, had me change into a hospital gown (being 18, I was still in the “children’s” category; the gown they gave me just barely reached my knees), took an x-ray of my shunt, (the technicians were both guys—super awkward!) and—glory be to God!—hooked me up to an IV painkiller that completely broke my headache almost instantly. After this, we finally got to meet with the neurologist, whom we’ll call Dr. M. He did a few neurological tests on me (you know: touch your finger to your nose, walk on your heels, walk in a straight line, etc.), and—being now pain free for the first time in at least two weeks—I of course performed each test perfectly. He then proceeds to explain about what he saw on the x-ray. He informed us that in fact, my shunt was broken in 2 pieces right around the neck area. He said it had probably happened during a growth spurt some time ago. He asked me if I was in any pain, and I of course said no, right now, with the painkiller still in my system, was not experiencing any pain. From this, Dr. M concluded that he thought I might be “shunt independent”—that my brain had outgrown its need for the shunt. He gave me a promising diagnosis, told me that the pain probably would go away after a while, and sent me on my merry way. The only problem was, I still wondered, “If I am ‘shunt independent’—then why on earth did it hurt so bad all of a sudden?” Dr. M never managed to answer that question.
The painkiller at least allowed me to sleep soundly that night, and when the pain began to return the next day, I could keep it at bay with regular, maximum dosing of both ibuprofen and acetaminophen, (even Excedrin, if I really couldn’t make it 2 hours between doses!), and I tried to resume my studies. It was now only a few weeks before my birthday. In addition, we had a mission trip to Mexico scheduled in mid-October as well. My dad began to question whether this mysterious pain that kept me taking pills at all hours and sleeping propped up would prevent me from going with them. It would be the first one I’d missed (besides the one I missed while attending EXCEL). What were we going to do about this? Local neurosurgeons like Dr. M obviously didn’t really have an answer for us. Where could we turn?

I must pause here and introduce my brother, briefly. He was the first boy after five girls. More importantly, he was born after me, and since my condition, the doctors said, increased Mom’s chances of having another baby with similar defects, they watched the ultrasounds carefully. The next child after me was a girl and she was a healthy baby, but the next, a boy—the first boy—they noticed something odd.
“We can only see three chambers of the heart,” the nurses repeated to my mom. Turns out, they couldn’t see the fourth chamber because it didn’t exist. My brother was born missing his left ventricle. This meant that the oxygenated blood that was supposed to be spread throughout the rest of his body was backwashing in his heart, mixing with the de-oxygenated blood, and causing the overall quality of his blood to be much poorer than a healthy person’s blood. Medicine has progressed to the point where doctors, when they catch such a condition early enough, they can take measures to save the baby’s life, but a lot of time, it goes unnoticed. The baby is born and looks healthy for the first few hours, the parents take it home, and within the first month, it turns blue from lack of oxygen and dies. In my brother’s case, however, they were watching for encephalitis, and they caught the heart defect. So, essentially, my defect saved his life. By the time he was seven, he had undergone seven open-heart surgeries. He’s got the wickedest scars of anyone I know. And about this time he began having regular annual checkups with the doctors at Seattle Children’s Hospital.

One of these checkups occurred shortly after I began having the headaches, around the end of September. Also this same day was a party I wanted to go to, an engagement party for one of my older sisters (and the first one to be engaged). I knew I would far rather go to the party than to Seattle, but my parents had received the opportunity to meet with the head of Pediatric Neurosurgery there at Children’s, and requested that I come. By that time, constant dosing on ibuprofen and over-the-counter pain meds kept the pain down to a dull ache, but the pressure was still such that I literally could not see straight, and every time I tried, my head would hurt worse. I did not protest much.
We met with the head neurosurgeon, a Dr. A. It was the most insightful consultation we had experienced yet. As it turned out, my condition happened to be his specialized field of study. I was not just another chapter from his textbook that he never expected to see in real life. I was a person who had a condition—albeit a rare one—who needed his help and his knowledge. He gave us three assignments to do in the following weeks: an MRI with contrast dye (to see where and how the fluid was flowing in my brain), a lumbar puncture (to check the pressure of the cerebral fluid in my spinal cord), and—strangest of all—he wanted us to see an ophthalmologist to check, he said, for something called papilledema.
I had just had my regular eye checkup in August. I had perfect vision; I had never had anything but perfect vision all my life. Every single one of my siblings has had minor variations between near- and farsightedness, but not me. So why would we need to check for papilledema, and what was it, anyway?
We worked with the local neurosurgery clinic (Dr. M’s office) to get the MRI. This meant more hospital gowns. (I hate hospital gowns now.) We went in to see our regular eye doctor. She was a little surprised to see us, more so when we tried to explain why. She sat me down in the chair, dilated my pupils, took one look in my eye, and said, “I’m going to get another pair of eyes for a second opinion.”
When your regular physician, who has basically watched you grow up, is the one to seek out a second opinion, you can pretty much bet that it is something serious. And for me, it was.
Turns out, she and the second doctor both saw beginning, early stages of papilledema in my eye. And what is papilledema? “Swelling of the tissue around the optic disc, which puts pressure on the nerves and affects the sight.” And how serious is it? The ophthalmologist put it bluntly, “If this issue is not resolved, Leslie will go blind.
I was shell-shocked; I went from perfect vision to going blind in only a month? What in the world was happening inside my head?
The next week, we went in for the lumbar puncture. They got my back numb (Note: isn’t it the height of irony that Novocain, the painkiller, stings like peroxide going in? Of course, it was effective, but—still!), and inserted the needle, testing the initial pressure of the fluid in my spinal cord.
In a normal spinal cord, the typical amount of pressure is usually somewhere around the low 20’s, like 22-23 or so, with 25 being too high to be usual. The pressure in my spinal cord? A whopping 53. I told the doctors, “Gee, no wonder my head was hurting!” I was walking around with more than twice the amount of pressure a normal person should have around the brain! They drew off enough fluid to bring the pressure down to 15, and I could literally feel the pressure leaving my head as they did so. It was such a relief!
We met with Dr. M again after the puncture. Obviously, he had by now rescinded his previous prognosis of “shunt independence.” Now we were talking surgical procedures. For the first time, he showed us the MRI scans of my brain. The four ventricles of fluid in my brain stood out like white lakes amidst the grey matter. (For reference, the ventricles of the brain are as follows: there are two ventricles in the main lobes of the brain, a third right in the middle, around the cerebellum, and situated back and below that is the fourth, which connects to the spinal cord.) Dr. M pointed to the narrow passageway (“aqueduct”) between the third and fourth ventricles, informing us that to him, the knowledgeable one, it looked a bit too narrow (“stenosed.”) I listened to him throwing around these very “textbookish” terms, and thought, “Why does he use these words? Does he expect that I will know what he is talking about?”
He informed us that, in light of the results of the lumbar puncture, he was willing to allow that, yes, we had a problem. He said we had two options, in light of the fact that my shunt was broken, he didn’t expect that it was working properly, and we needed to relieve the pressure on my brain somehow. The first option was installing a new shunt. He told us that the general failure rate for cerebral shunts was somewhere around 25%. He also said that if we chose this option, he would not feel comfortable removing the old shunt, as not only was it broken, but parts of it had calcified (fused) to my collarbone, and he did not want to risk taking it out and leaving shards of plastic in my body. They would just line the new shunt up next to it, if we chose that option, he said.
Option number two would be an endoscopy in my brain, to create a new drainage channel that would bypass the normal channels entirely. Dr. M said that maybe the “aqueduct” between my third and fourth ventricles was small from the beginning, hence the encephalocele, and that the endoscopy would create a new channel leading right from my third ventricle to the spinal cord. When my dad asked about the risks for this, Dr. M said that such a procedure ran very close to my hypothalamus—the part of the brain largely responsible for many things, including short-term memory. I thought this sounded more scary than a new shunt (even though the 25% failure rate was a wide margin!), especially since, being in college, I knew I would need my short-term memory to study for tests and such! Dr. M gave us 2 weeks to think about it. This was on a Friday, exactly one week before our trip to Mexico.
My parents brought up the trip to Mexico, and Dr. M immediately shook his head. “Oh no,” he said, “no matter what procedure we do, she is definitely not going anywhere for at least a month!” He sent us home with many worries and questions, not the least of which was voiced by my dad as we got into the car, “If the trouble is with the ventricles up in Leslie’s brain,” he queried, “Why was the pressure so high, down in the lumbar region of her spine?” Dr. M hadn’t really explained that.
When we got home that afternoon, Mom e-mailed Dr. A in Seattle and told him about it. I think she probably figured he’d be able to glance at it later that weekend, but within the hour, she receives his reply.
“The national failure rate for cerebral shunt revisions is between 8 and 11%,” he informs us, “Here at Seattle Children’s Hospital, I supervised over 200 revisions in the last year, and the failure rate was less than 5%.”
Sort of makes one wonder where Dr. M got his “25%” figure, doesn’t it? But wait, Dr. A continued,
“I am in the OR this weekend, and I would be willing to let Leslie be my first case of the day on Monday morning.

Great. So now, instead of two weeks to prepare for a surgery, I have two days. And this is no “go into a section of the body where there is lots of space and take out something small, like an appendix or something” type of surgery, nor is it the type where there are no major organs, and the doctor can just immobilize the area in a cast or something to speed recovery.
This is my brain we’re talking about, people. This is a “open the skull, take a shunt tube out of the brain, feed a new tube into the brain, down through the neck, past the ribs, and coil it in the abdominal cavity” type of operation.
Two days. That’s all I had to get physically, mentally, and emotionally prepared for this. Plus, I was still battling headaches and nausea even after my lumbar puncture, both because my body was no doubt trying to replace the fluid the doctors had removed, and because it was probably doing so at an abnormally fast rate. (In fact, even though they put me on medication that supposedly slowed the production of cerebrospinal fluid, by the time of the surgery, my pressure levels were already back in the thirties)
We went to church that Sunday morning, I lasted about an hour before I had to leave and lay down in the car because of nausea and exhaustion, we came home, my mom and I packed for a few-day trip, and we drove up to Seattle that afternoon for the pre-op stuff. (Meeting the OR team, taking a few vials of blood (which meant another hospital gown! I was getting pretty tired of those things!) Luckily, we got to stay at my mom’s brother’s house, because he lived only five minutes from the hospital.
6 AM Monday morning, we went into the hospital for my operation. The anesthesiologist “just happened” to be a Brit, which I regard as entirely God’s doing, because till now only He knew that I regard the British accent to be the single coolest accent on the planet. (Until you try to say something like, “I’m artistic”; go ahead, try saying it out loud, and you’ll realize what I mean) I also received immense comfort seeing the door of the OR plastered with a large sign proclaiming “LATEX ALLERGY.” So at Children’s Hospital, apparently they have an entire OR of non-latex equipment, meant especially for those kids with latex allergies. One less thing for me to worry about. (To all you medical professionals who are thinking, "Of course they would!" This is a legitimate fear for me, since it was my own negligence in reminding a new hygienist at the dentist office about my allergy that resulted in near-asphyxiation; since then I've been paranoid of rubber gloves)

Quite frankly, the morning of the operation, I had just one condition I was concerned about the most. You recall that the first time I went to Seattle to meet Dr. A, that my sister was having her engagement party. She was getting married in January, less than three months away. Therefore, when the surgeon asked me, “Is there anything you’re worried about?” I immediately looked up at him and asked,
“Just how much hair are you going to take off?”
I had voiced this to my dad earlier; he smiled and told me, “Don’t worry, Leslie; I’ll buy you a nice wig to wear for the wedding.” So comforting, my dad.
The surgeon smiled and promised he would take of as little hair as he needed to; he reassured me that he definitely would not need to shave the whole side of my head to perform the operation. The anesthesiologist came forward and hooked me up to a nitrous oxide mask and tried to have his assistant put an IV needle for more painkiller into my hand. She missed the vein, so he had to do it himself. Meanwhile, he’s chatting his is pleasant, British way all about the history of nitrous oxide. I take one deep breath, and feel the uncontrollable giggling welling up inside me. One more breath, and I black out completely.
            At about 1 PM, I come to in the Post-op Recovery area. My mouth is very dry, and I have new scars with stiff, black stitches in two places on my abdomen and along the side of my skull. I still have most of my hair. It’s all pulled off to the left side of my head. Only about an inch-wide strip on the right side of my head is bald.
^^Top view. The curve of this scar is visible when I part my hair on the right.
This is where the valve from the old shunt (the "pump") was removed.

            They wheel me into a room of my own, where my mom is waiting. She’s on the phone a lot, talking to people and telling them how my surgery went. I am hooked up to an IV morphine drip, but I’m not sure if it’s doing all that much. I don’t hurt, but probably after the first hour, it wasn’t because of the morphine. The most that stuff ever did, I think, was make me feel nauseous at the sight or taste of food. It’s weird having stitches covered by large pieces of gauze all over my body. I got visits from my mom’s sister-in-law and my dad’s sisters, who also lived in Seattle. Some friends of ours who also live in Seattle paid me a visit. I got lots of presents. As the day wears on, I feel better and stronger.
            By evening, my biggest frustration is the respiration monitor I have that measures the time between breaths. The morphine drip, for its part, slows down my respiration, so I can relax for several moments between breaths. The only trouble is, when I relax like this, the respiration monitor sets off an alarm, thinking I’ve asphyxiated or something, and it goes off till a nurse comes in to turn it off. Between that and nurses coming in every four hours to take my vitals, and every six to give me Tylenol and Oxycodone to take, I didn’t get much sleep that night.
            Tuesday morning, I feel great. They take me off a constant morphine drip, and give me a button that, if I am in pain, dispenses a predetermined amount of morphine. I never pressed that button once, the reason being that I was very hungry by now, and as long as the morphine was in my system, I could not eat. Mom and I watch a lot of TV; laughing makes my stitches hurt. That morning I receive a present from the hospital gift shop: a gigantic stuffed cat the size and shape of a body pillow. Clutching that against my chest as I laugh makes it easier on the stitches. By the afternoon, I am able to walk around, and I feel completely normal. Dr. A pays us a visit.
            He was very happy at how well I’ve recovered. He said if I felt well enough in a few hours, he’d let us leave the hospital, though for safety’s sake we should probably spend one more night in Seattle, just in case something goes wrong. Then Dr. A speaks the unimaginable.
            He turns to my mom and says, “So, you mentioned that you are going to Mexico sometime soon; is that this week?”
            My mom, fully aware of what Dr. M had told us, says yes, there is a trip planned, but of course, she and I are not going.
            Dr. A: “Why not?”
            Till now I had all but given up on my chance of going to Mexico. I was ready for a lonely birthday at home with just my mom. Now, Dr. A has brought it up himself, and he didn’t have a problem with us going! He said, “If Leslie feels well enough, I don’t think it would be a problem. The stitches are secure, I don’t think they’ll fall out. Let me send you with a CD of your MRIs, just in case you run into an emergency down there.” He then shows us the post-operation MRI: where once I had large white lakes in my brain, now there are tiny white pinstripes. I thought, So THAT’s what my brain is SUPPOSED to look like! Shunt independent, my eye!
            Just to test, I tried flipping my hair over the bald spot on my right side. With my part on the left, you couldn’t even see the stitches! I had four scars from the operation. Between them, there were almost one hundred stitches, I think.
This picture was taken right after the previous two. I promise!
            Discharged from the hospital on Tuesday afternoon. Left Seattle on Wednesday afternoon. Packed all day on Thursday. Left with my family to Mexico on Friday. Celebrated my birthday by arriving at our host family’s house by Saturday night. What a crazy week!
So, I ask you: what is normal? Would you consider me normal? Is it because you think I look like you? Is it because I conform to your expectations? Looks can be deceiving. I am just as far from normal as the next person. Or perhaps you are the one who is not normal, having never experienced such “coincidental miracles” as I have.