Diagnostics, Day Surgery and Flash Fires 06.27.09
It wasn't cancer. At least it isn't yet. The cysts on my vocal chords were discovered by the Otolaryngologist as he checked for obvious physical abnormalities possibly contributing to the bogus diagnosis of sleep apnea (Sleep Study). He removed them about a year ago now and in the follow-up was surprised to see they were back. He was convinced they were caused by acid reflux. Sure enough, Google confirms granulomas in the larynx are normally the result of acid reflux. His initial treatment is "the purple pill" twice a day. When they had no effect, he went in, snipped out the little buggers and sent them to pathology. Granulomas - benign, he said. Cool, I croaked. When they came back he packed me off to see his partner Otolaryngologist (OH-TOE-LAR-IN-GOLLA-JIST) as she specializes in the larynx. They were in the larynx the first time, I am too cowed to say. This guy is a Otolaryngologist rock star with an entourage, a website, and big flat screen TVs in every room scrolling credentials and confidence 24/7.
Sleep Study 05.02.08
I'm sitting in the exam chair for the usual eternity when the querulous nurse returns with an instrument dangling a two foot tube from one end. Slapping it down on the ancient video monitor she smiles knowingly. It will soon be up my nose and down my throat. What I used to call an ENT (Ear Nose and Throat professionals are now known by the utterly unpronounceable honorific Otolaryngologists) arrives with an entourage. Two guys in suits, a young woman in a doctor coat and the intern who "worked me up" earlier. Doctors don't actually talk to patients directly any more as they don't have the time. An intern collects all the necessary data, presents to the doctor and the doctor swishes in to confirm the interns findings. One can only hope the diagnostic aspect of the doctor-patient relationship is properly preserved by having the trainee collect all the relevant data. Much like having the SWAT team break down your door because your neighbor's kid in the local Criminal Justice magnet school assessed your hobby modeling clay looks a little too much like plastique. But I digress. The Oh Toe Larn Gaul A Just explains the guys in suits are determining whether they want to specialize in Oh Toe Larn Golly Gee. We all watch as the tube is snaked up my nose and down my throat. I don't like the looks of this little cyst, he says. I'm not saying it's a tumor (OMG IT'S CANCER!) but we should take a look in four months and see what it looks like (FOUR MONTHS?!? IT COULD BE THE SIZE OF A HUMMER IN FOUR MONTHS!!!). Meanwhile, let's get a sleep study on you to see how you sleep. OK, doctor, thanks.
I arrived on time for my sleep study with a New Yorker, The Stone Gods by Jeanette Winterson and a fully charged IPOD with a recently revised set of playlists. I might as well have brought Proust. All these will end up on the night table, untouched. By eight PM I had seventeen electrodes affixed to my scalp, face, chest, and ankles, a belt around my chest, another around my stomach, two sets of nasal breathing tubes hooked over my ears and a harness around my neck. Each of the electrodes were plugged into the harness and their multi-colored wires ran over, under, sideways and down my t-shirt, boxers and socks. All this applied by two chatty technicians while I watched a video on the magical effect of the CPAP breathing system designed to alleviate my sleep apnea and give me the best rest of my life. The video is made by the folks at Respironics and sure enough, they've been in the CPAP business since a rested Rip woke up. The many satisfied users all speak earnestly of the need to give it a chance to work. Compliance seems an issue. But I'm not there yet, I'm getting copious quantities of paste and tape applied in such a way that I couldn't unplug a wire if I tried. Leaving the prep room I walked the short distance to the sleep wing. On the way I encountered a small child walking hand in hand with an adult. Unaware I looked like a suicide bomber held together by great patches of white tape I smiled at the little boy. His eyes went to saucers, his mouth opened in silent scream and the grip on his keepers hand switched to death mode.
I tried reading but the harness weighed fifteen pounds and kept knocking the book from my hands. I was provided with a phone for outgoing calls only and told to switch off my cell so I wouldn't be disturbed. Fifteen minutes later the outgoing calls only phone rings. I answer. No one there. Five minutes later it rings again and this time the caller seeks Aubrey. Not here I say. They don't seem to believe me so I explain they've called a room at the hospital. Oh my god and then dial tone. I unplug the phone. I lay down. The tech returns and plugs the console into the bed side, flips on the infra-red light and turns off the fluorescents. Two minutes later the speaker phone next to the headboard cracks to life with, we need to test the wiring Mr. Stiles. He's back in the room in seconds as it appears I have managed to disconnect an EKG lead. More paste, more tape. Off with the lights and the bedside speaker returns with flex this foot, that foot, hold your breath, breath through your nose, mouth, look left, right, up down, cough (there is a microphone taped to my neck I learn) and, good night see you in the morning. This is an obvious lie as he will be watching me all night on the video camera. Supposedly.
Seven hours later I am disconnected and spend thirty minutes in the shower trying to wash the paste out of my hair and beard.
I'm thinking the sleep study will confirm I don't sleep well.
Not that it matters, the tumor in my throat will probably kill me before the results are back.
Less than an hour after the nurse called to report the findings I got a call from a toll-free "sleep service" company to schedule my appointment for the follow-up study "your doctor recommended." Most interesting.
Imagine you go to the doctor complaining about a runny nose and sneezing. You go to a ENT for an exam but first you watch a video about antihistamines and the "miracle" they offer. The video is made by Pfizer, a pharmaceutical company. You then see the ENT and he says it looks like an allergy. By the time you get home there is a voice mail on your machine from Pfizer offering a 14 day free trial.
I'm sure there is such a thing as sleep apnea and I'm equally sure people have benefited from CPAP machines. It is more than a little disconcerting that the people who make the devices have inserted their infomercials into the medical process and are sufficiently organized and connected to the medical establishment that they underwrite a service to "follow-up" and make sure I get scheduled for my introduction to the CPAP device.
Diagnostics and Me 04.19.08
The last couple of times I've tried to schedule an annual physical with my gatekeeper I've mucked it up by mentioning some extraneous issue. This time I referred to a mid day energy collapse. I have yet again been sentenced to wait for the call from the folks at the sleep clinic. They want me to spend the night at the hospital wired up to monitors so they can measure my sleep. Or they don't. They never call.
I recently finished a most interesting book by Jerome Kroopman titled, "How Doctors Think." The author was on rounds getting frustrated over his charges failure to think outside the box when presenting.
For those of you who only watch Desperate Housewives, interns are led from room to room in teaching hospitals and "present" each patient to their resident or attending physician. I never can keep the difference between resident and attending straight. Resident means they have graduated from internship, I think, and attending means they have graduated from resident and actually have patients for whom they are responsible. But no matter.
Kroopman's residents struck him as automatons. Their methodology for diagnosing disease appeared to involve no imagination, no "thinking outside the box." Kroopman, a physician with a lot of time on his hands apparently, began questioning how his charges were being taught to think about the art of diagnosis. He discovered a centuries old philosophical school had recently returned to favor and was being used to instruct med students in how to think. The school is known by the name of its founder, Thomas Bayes, an eighteenth century minister and mathematician. Known now as Bayesian analysis it predicts the likelihood of a subsequent event by factoring in previous events. The explosion in computing capability has validated some of its more arcane precepts ushering it back into favor and medical schools. Bayesian reasoning can be summed up in the adage, "if you hear hoof beats, think horses not zebras." Every other time you've heard hooves it's been horses so the probability that the hooves you hear are again horses is great enough for you to safely conclude horses will come round the corner. The problem Kroopman sees with this methodology is that unlike insurance actuarial tables where predictability can protect profits, predictability based on past experience in diagnostic medicine can kill people. Exploring our common thinking errors in light of this new med school teaching tool, Kroopman decries the dangers inherent in Bayesian reasoning. Our predisposition to cling to our first impression, our investment in a decision made, our tendency to rely on technical aids all make Bayesian reasoning dangerous in difficult diagnostic scenarios. Bringing in statistical studies belying the "accuracy" of x-rays, CAT scans and the like, Kroopman makes a stronger case for medicine as art rather than science. The number of times x-rays are read differently by different specialists, in some cases by the same specialist at different times, is more than just statistically significant, it's frightening.
The upshot of all this is how truly difficult it is to perform the diagnostic function well. Add in pressure to maximize patients seen and it isn't surprising that doctors interrupt patients describing their ailments after eighteen seconds on average. After eighteen seconds! About as long as it took to read the last five sentences.
Armed with this new arsenal of facts about diagnostic difficulty I anxiously await the doctor's arrival. But I forgot. Here, the intern will "work up" the case and present their findings to the doctor out of earshot. The last time my intern was working on his endocrinology specialty. He decided that I was pre-diabetic and ordered a round of glucose tolerance tests and delivered a stern warning about weight and desserts. I don't know what this intern's specialty was but I was hoping it wasn't psychiatry. I waited forty minutes in the exam room. When the intern did arrive he asked if I minded if he typed while we talked. Everything is on computer now but it might as well be in Mayan hieroglyphics for all the good it does. They don't have and won't take the time to read through the history. When I finished describing the failed knee surgery, the historically high triglyceride count, the recent steroidal spinal injections, my difficulty breathing without daily antihistamines and insomnia he summarizes, "so your chief complaint is exhaustion?"
"Sure, I guess so."
I failed to remind him I was here for an annual physical exam. You know, bloodwork, chest x-ray, whatever. So I ended up with a referral to the sleep clinic.
They'll call you tomorrow to set up an appointment.
More from The Healers and Medicine's Front Lines 11.14.01
Several weeks ago, I made an appointment with the gatekeeper for my semi-annual physical exam. Gatekeepers are also called Primary Care Physicians, or PCP for short. Something else goes by the acronym PCP, something more or less equivalently hazardous.
She's a nice lady but she doesn't know me from Adam. She reads the chart just before she walks in the exam room, so she can call me by name, I guess, because she never seems to know why I'm there. I told the girl when I made the appointment that I thought it was near time for a regular physical exam but I wasn't sure so I asked her to look it up. "Oh your chart is gone, Mr. Stiles."
"Gone," I asked? "You mean it's at your other facility."
"No sir, it was one of the eighteen thousand files lost in the flood last summer."
The Texas Medical Center not only kept its files in the basement, they also kept the emergency generators there. You know, the emergency generators that keep power supplied during power outages caused by storms or floods. They were six feet under when the power went out. Even the most modern diesel powered emergency power supply can't get started while submerged. Recognizing the error of their ways, the Medical Center recently held a press conference to announce their latest innovation. Big steel doors to keep the flood waters out of the basements. Now this is Houston, mind you. We average fifty eight inches a year in rainfall. The climate is in the same category as the Amazon rain forest save for ten or so days every year when the temp gets below freezing. Now I would think you would want to move the emergency generators out of the basement.
But I'm not an engineer so what could I possibly know that they don't?
At any rate, I made the appointment. I only waited an hour and a half past my appointment time before being called. When my gatekeeper comes in, she asks why I'm here to see her. "Annual physical," I told her.
Last year when I asked about the new test for prostate cancer she spat out what had to be a practiced speech. Her eyelids fluttered and her eyes rolled upwards as if she were reading something written inside her eyelids too high to quite make out. The upshot was that the test hadn't yet demonstrated its effectiveness, and even if it did reveal a high likelihood of early stage prostate cancer, I would still have to take more tests to determine if it was cancer or not and even then have to make the decision to respond surgically with its well publicized side effect of erectile dysfunction (a phrase way too clinical to adequately describe the condition). I took the eyelid fluttering to be a "tell" that she was not being forthright. My guess is the HMO carrier had probably come down hard in their opposition to a test that would lead to more tests. A week or so later, NPR did a story on the remarkable effectiveness of the PSA test in diagnosing prostate cancer's likelihood.
But that was last year and I'm not a doctor so what could I possible know that they don't?
She listened to my chest, checked to see if my pupils dilated and constricted in uniform manner, and felt around on various lymph nodes. She appeared to be done and was making notes in my new file so I brought up the back pain. I had previously resolved to bring it up. It's been with me most of my adult life but it got much worse recently so I figured it was time to seek medical advice. She thought for a moment and said, "I guess we better do a CT scan and some x-rays. I'm going to prescribe a muscle relaxant and pain medication for your back." She wrote the prescriptions, handed them to me and started to walk out. "Shouldn't I have some blood work done as part of my physical exam," I asked. "Oh yes," she says and writes another prescription for blood work. "Thanks Doctor."
I didn't bring up the prostate issue or suggest a urinalysis or any other diagnostic tools that might help to keep me alive beyond my life expectancy as I didn't want to be branded a hypochondriac. I've always believed they write code words in our files to indicate less formal medical conditions like "sarcastic" or "psycho" or "thinks he's soo funny." My previous history was lost in the flood so here was my chance to look like a model patient and I wasn't going to waste it on silly cancer or kidney problem diagnostics. I was going in for a CT scan and that was good enough for this year.
At check-out (sounds like the grocery except there's never more than one checker) I'm told I can't make the CT scan appointment because the referral hasn't been approved by the insurance carrier (HMO). Two days later I get a call from the scanners that I am cleared to come in. I make the appointment for the following day but not before asking them about the approval process. "What approval process," she asks.
"The one that kept me from coming to you two days ago when my PCP referred me," I explain.
"There's no approval required if your PCP tells you to get it you just bring the paper down here and we do it." Now I'll know what to say at check-out next time. Meanwhile, I'm off to get a CT scan.
The CT (Computerized Tomography) scan is like an MRI (Magnetic Resonance Imaging), only cheaper. It's primary diagnostic function is more skeletal than soft tissue related. The MRI does better at distinguishing soft tissue problems. The CT scan is essentially a computer assisted x-ray. The end product looks like a simple x-ray with collating inserts and diagrams that allow the viewer to determine precisely which "slice" of the scanned body part is being observed. An MRI uses magnets to excite the hydrogen atoms present throughout the body. The radiation they emit as they return to their pre-excited state is captured on photographic plates. Since we are two-thirds water and each molecule of water is two parts hydrogen and one part oxygen, there are a lot of hydrogen atoms to excite and emit, especially in the softer (higher water content) tissues.
I called my gatekeeper several days later to ask about the results. Of course, I didn't actually speak with her. Her nurse told me the CT scan indicated disk problems and I would need to see a back specialist. Back specialist means surgeon these days so I begin the wait for the referral from the PCP's office. Another week goes by and no referral. I call the HMO itself to find out what the hold-up is. They approved the referral a week ago.
Note that seeing my PCP and having her decide to refer me is not sufficient. The HMO itself has to pass on her referral to a specialist. Even the gatekeepers have gatekeepers in the HMO universe. I call the PCP. "You have had a referral for a week, may I have it?"
I call the lab and ask them to send me the photos.
"We'll mail them to you."
Anyway, two days later, no photos. I call the lab. "What's happened?" I ask.
The day comes for my appointment with the neuro-surgeon. I walk into a waiting room with one other occupant. She's filling out the same paperwork I'll soon be filling out. She's a stunning beauty, I wonder if she's maybe a film star, she looks familiar. She goes in first. An hour and fifteen minutes later, an hour after my scheduled time, she departs. I spent the last forty minutes listening to her and the surgeon going on about traffic, the weather, working out, blah-blah-blah. I wonder if I'll get the same expansive treatment when it's my turn. Not being a beautiful woman, I somehow doubt it.
But I'm not a physician, so what do I know about such matters?
I'm ushered into the doctors office and we talk for a half hour. He asks me about previous surgeries and I tell him about suffering a knife wound. In the process of telling the story I explain that the culprits were conversing in Spanish with my friend before things got out of hand and since I don't speak Spanish I don't know what was said but things got pretty heated. He asks me about the back pain. "OK, go down the hall into room one, undress except for your underclothes and I'll be right in as soon as I look at your x-rays and CT scan." I roll out some clean paper on the exam table, wadding up the used paper and stuffing it into the trash. I stand shivering for another twenty minutes. I can hear him on the phone in the next room. He makes two and takes one call. I guess he's a multi-tasker and is scanning my photos as he reschedules his weekend barbecue. I can also hear the office manager trying to find a patient. She calls the patients house. "I'll call Robert at work and if I can't get him there, tell him we're looking for him. His test results are in. Ha-ha-ha-ha-ha." She gets him at his office. "Your test results are in and the fusion didn't fuse, ha-ha-ha-ha-ha. We don't know why, that's what you and the doctor are going to talk about and plan what to do next. Ha-ha-ha-ha."
Later, in the exam room, he says, "Hispanics are violent people. They especially like the knife." That he is a racist is bad enough. That he would make the assumption that having been attacked by a member of an ethnic group I would automatically be receptive to racist comments about that ethnic group is even worse. Then he says I need an MRI because he can't see anything in the CT scan. Now two other doctors did see something, but the surgeon can't. Maybe it's a vision thing.
At check-out, the syrupy sweet office manager notices that my referral is missing.
"You're an HMO, where is your referral?"
Hmm, an inconsiderate racist surgeon with bad vision who teaches his staff that I'm the enemy, do I want this guy cutting on me? What if he determines I have been infused previously with non-Ayran blood? Would he be less careful? What if he finds out I believe HMOs are the only way quality medical care can be made available to people of moderate means?
I think I need another doctor.
But what do I know, I'm just a patient.
Hippocrates Turns In The Grave 09.01.01
My first recollection of the doctor's office was at seven years of age. My parents were concerned that I was too thin. This was back in the days when fried pork chops and mashed potatoes were considered healthy. The potatoes came from some sort of dried flakes mixed with boiling water. The lumps we found in them were clumps of undissolved flakes. If mom felt energetic, there were no lumps and the potatoes had a consistency of vanilla pudding that had been stretched from four to six servings with a little extra whole milk. She would ladle the potatoes onto our plates and make an indentation in the top to hold the margarine or if Dad cooked, the gravy. Margarine because butter was too expensive, not because of the fat. The gravy was flour, margarine, milk and salt, mixed in the grease from the pork chops and poured over the chops and potatoes. Somehow, in spite of my dietary regimen, I was too thin. Dr. Punyell's office sat on the corner across from the high school. The waiting room had plenty of light and I remember it as a friendly place. The good doctor's prescription was to give me a small glass of red wine about an hour before dinner to "build my appetite." I remember the glass; it held about four ounces of Mogen David. At my current weight (I am no longer thin) it was the equivalent of an ice tea tumbler of wine. I don't know how many years I was on this dietary supplement, but I can tell you I did finally manage to stop drinking nine years ago. To be fair, I don't think drinking wine as a child caused me to have a problem with alcohol but it didn't help any. I didn't gain any weight, by the way, until I took a summer job at the local ice cream parlor years later.
In my freshman year in high school, I contracted Bell's palsy. One side of my face was paralyzed. I looked like the early film version of The Phantom of the Opera. The doctor announced that it would either go away after six weeks or be permanent and there was nothing he could do. It went away after six weeks.
The last time I saw Dr. Punyell he gave me a stern lecture about financial responsibilities. Dad died owing the good doctor several hundreds of dollars. In Dr. Punyell's view, the responsibility for that bill passed to me and my little sister and he was intent that we should pay. I was sixteen and my little sister was fourteen. We never saw him again.
I had all four wisdom teeth out in a single procedure back in 1969. My dentist was a classmate's dad, Puddinhead Wilson. "Easier to do all four at once," he told my mom as if the truth of his statement were self-evident. One of them shattered under the pliers. Removing the remains required nearly an hour of slicing and scraping. Three days later the wound closed and I could wake up without seeing blood on the pillowcase.
My brother suffered some sort of emotional collapse while attending Vanderbilt University in the late sixties. Given the times, everyone assumed drugs were somehow involved, but there was no talk, that I heard, of detox or rehab. He was incarcerated at the local psych hospital for a few weeks. I remember visiting him a couple of times and I remember Thorazine being discussed as treatment. He was pretty much a zombie when I saw him, with spittle dried up in the corners of his mouth. I don't guess I'll ever know if his state was Thorazine induced or from the depression they said he suffered. He wasn't responding, in any event, they said, so they began electric shock treatments. That's when they send an electric current through your temples to help clear the brain of unpleasant memories. The shock is strong enough that the patient has to be strapped to a gurney and a fat wad of tape on the end of a tongue depressor stuffed into their mouth to prevent the biting or swallowing of the tongue. His doctor at the time, Dr. Shreve always wore a hounds tooth sport coat over blue or brown slacks. He was a huge man with what we would now describe as a profound eating disorder. He cured my brother with electric shock. Jerry had some holes in his memory afterwards that never filled in. He would ask us about times of his life and we would do our best to fill him in. He didn't have that much time left, though. He hung himself a couple of years later when a later shrink broke off their affair.
A few months ago I noticed an enormous swelling below the gum line under one of my pre-molars. I begged an appointment from Dr. Gladwell's office manager and went in immediately. "Oh my," the technician said as she buckled on the blood bib. Dr. Gladwell took a look and said, "Oh my, we'll need to get you to a endodontist." My insurance plan recognizes only two endodontists within sixty miles of my home. About five million people live here, but Cigna is only able to find two endodontists willing to sign on their program. Wonder why that is. About twenty minutes into the procedure I feel little jolts of pain as he jams the wire into the cavity to clean out the diseased tissue. He's shot two full syringes worth of novocaine into my jaw so I'm thinking it can't get any worse than this. Error. The next pain is that blinding, drop whatever you're holding, gasp for air kind of pain. I reflexively push back and into the chair. Dr. Gladwell says, "that area is numb, you can't be experiencing any pain." I disagree. "Well, I don't see how," he says and shoots another load of novocaine into my jaw. "I have patients waiting," he says in his lilting accent as if to encourage me to stop slowing down the process.
Six months prior to this, on a routine visit to Dr. Gladwell's office, I complained that a tooth in my upper jaw hurt. Fifteen minutes and two x-rays later I was told that tooth already had a root canal and the pain I was feeling could be cured with this little fifteen dollar bottle of senso-rinse. The tooth will be pulled next month and a two thousand dollar bridge will replace it.
That these beacons of healing were wrong is not what irks me so. It's their insouciance. When's the last time a member of the medical profession looked you in the eye and asked how you were doing? And listened to the answer? Generally, they walk in reading your chart to see who you are and why you're in their office. Does your doctor know what you do for a living? Does your dentist know if you have a family?
They complain about HMO's and the interference with the patient-doctor relationship. What relationship, I ask? I submit their problem with HMO's is more about income controls. I knew three people in pre-med in school. Each of them declared their interest in medicine was driven my money. None of them were particularly interested in healing. They were all headed for specialties. The only one I kept track of is an emergency room physician who shuttles between here and a two day a week stint in an emergency room in Las Vegas. Lots of money, no relationships. Sounds like he was just way ahead of the curve.
BugsAn ancient native-American view of the world, Gaia, posits that everything is a part of a larger life form. The rivers, rocks, wind, and grass are just as "alive" as the buffalo and the Blackfeet. The concept of a "mother Earth" springs from this belief. All life survives by striking a balance between itself and its environment. Humanity hammers away at that delicate balance by virtue of our self-proclaimed God-given right to dominion. We "master" the environment and dominate other life forms. Hence we struggle more than, say, the bumblebee. Other, much smaller life forms struggle as well. Their struggle is, while dramatically less visible, nonetheless complex.
Microbes, bacteria, and viruses don't live on their own. Their environment usually consists of another living organism. Human cells often provide a home for these micro parasites. Their replication can wreak havoc on our bodies or it can pass unnoticed, depending upon how well the organism has struck a balance with its host. If the havoc is serious enough, death can result. If that death is too quick, before the organism has had a chance to find a new host, the micro parasite dies with its host.
We can be made to suffer and die horribly by something so literally beneath our notice that it remained invisible to us for our first 100,000 years. Once these pesky little creatures became known to us, though, all our ingenuity and creative thought was brought to bear on them. Penicillin, pasteurization, sewer systems, DDT, have all played their part in helping us fend off the nastier varieties. Malaria, arguably the most persistent and pernicious of these "bugs" was on the brink of annihilation when Rachel Carson published her seminal environmental work, Silent Spring. DDT was subsequently banned. A thin layer would be sprayed on the walls of homes in an infected area. Mosquitoes retire to a wall or ceiling after feeding to digest their food (our blood). Landing on a microscopic layer of DDT, they dropped like mosquitoes. That same microscopic layer, though, seemed to hang around for eternity. In any event, while we have eradicated some and held others in check (smallpox and tuberculosis as bad examples), still others remain in the background (or the uninhabited jungles of Africa and the Amazon), while new microparasites are discovered every month. Microparasites are living organisms that find a food source in a "host." The impact on the host can be terminal or it can be unnoticed. Some merely hitch a ride until the next host can be found. The varieties number in the billions.
William McNeill's book, Plagues and Peoples, takes a most interesting look at the relationship between microparasites and hosts. McNeill looks at the role microparasites played and play in humanity's story.
Anthropologists place clothing and agricultural development at the forefront of our movement from hunter-gatherers to the dominant species we have become. Mankind's movement out of sub-Saharan Africa into the Indus, Tigris-Euphrates, and Yellow River basins was certainly facilitated by the development of clothing and our mastery of tools sophisticated enough to allow us to construct shelter. What we left behind, though, is far more important than what we used to escape. What we left behind was the planet's best incubator of microparasites. The steamy tropical jungles of sub-Saharan Africa house a far wider variety of life than any other locale on Earth. Included in that variety are microparasites. While we remained in the tropical clime that birthed us, we were constantly attacked by microparasites of every imaginable form and variety.
Some forty to one hundred thousand years ago, mankind moved out of the tropical forest and began hunting large game in the plains and savannas of non-equatorial Africa. Our expansion across the globe was remarkably fast (for any species, much less one with poor eyesight, no claws, and long infancy) and was made possible by our escaping the perennial diseases and infections of the tropics.
Our foray into the world of agriculture presaged another huge increase in population. The increase in agricultural production was through our mastery of the art of irrigation. Irrigation meant standing water (particularly along the Nile, Indus and Euphrates valleys) and standing water means safe haven for microparasites. Microparasites can live outside their host only for brief periods, made longer by warm wet climates (like equatorial Africa) or large pools of standing water. Standing water also means breeding grounds for parasite vehicles like the mosquito. Further, agricultural production meant grain storage. Grain storage means rats. As cities developed (ancient Sumer for example) alongside these new agricultural centers, population density grew to sufficient levels to allow host to host transmission of microparasites.
One of the adaptations microparasitic organisms make is to become endemic in the population. This is difficult if the impact on the adult population is dramatic. If the "die-off" is great enough, the population thins to the point that it becomes a non-viable host. The initial introduction of a disease can have terminal consequences for the civilization (look to Cortez and the native-American population). If the introduction is to a population group of greater than half a million and the disease is not universally fatal, a significant enough number of the population groups children develop the disease and recover. Once recovered, sufficient immunity exists to prevent recurrence (at least in its most virulent form). Over many generations, a large percentage of the adult population become immune. No such immunity exists for children, however, so the disease becomes a disease of childhood. Fatality rates can remain high in the child population and not threaten the balance established between microparasite and host. Children are more easily replaced and their death carries less impact on the larger social structure. This a stasis that develops over many generations.
Escaping the tropics and its cornucopia of microparasites allowed mankind's numbers to dramatically increase. One of the "balances" had been upset. The numbers of humans increased but remained scattered in hunting groups. As we began to settle into cities with the advent of agriculture, our numbers increased while we simultaneously created better living conditions for microparasites. It isn't as if the non-tropical climates were free of microparasites or disease. Equatorial Africa, however, was so rife with microparasites and consequent disease that mankind's numbers were held in check. The malarial plasmodium (malaria) is a prime example of a parasite in balance with its host. Malaria is generally a recurring and lifelong illness that comes and goes in bouts of fever and listlessness. Should significant numbers of a population become infected, the impact on general and reproductive activities can restrict overall social development. The incidence of malaria in equatorial Africa is sufficiently high to have significant social consequence.
As population centers began to develop, microparasites began to develop as well. The impact on ancient civilizations can only be inferred. One of the confounding characteristics of microparasitic disease is that symptoms change over time. As microparasites struggle to achieve their balance with their hosts, they adapt. As they change, the impact on the host changes. Further, ancient descriptions of disease often fail to identify the telling characteristics. For example, the coughing up of blood was described in an ancient Roman text as the salient feature of a particularly nasty pestilence. The formation of small pustules on the skin was mentioned in passing. The small pustules are probably the mark of smallpox but we shall never know. Just as the animals of the Galapagos Islands often resemble no other creatures, microparasites, isolated by distance begin to develop independently. As population centers developed around flooding river valleys and trade and transportation between these centers was still millennia away, separate and independent microparasite and disease pools developed. When a population center is introduced to a new form of microparasite, the impact can be devastating. Cortez's invasion of the New World and the subsequent die-off of the native populations is the most oft-cited example of such an event. In ancient times, such events occurred and were likely relegated to the displeasure of the Gods. The Indus River civilization that evolved into modern India may provide a more interesting and profound example of the impact of previously unknown microparasites' introduction into a new "host" pool. The Indus civilization spread over a geographically disparate landscape. To the north and west of the sub-continent, urban centers developed. South and east of these urban centers lived "the forest peoples" living in small self-contained communities. The urban centers developed as a result of their location north of the more microparasitic laden forests of the interior. As the urban centers began their inevitable expansion is search of land and food sources to accommodate their population growth, they encountered the "forest-people" and their army of microparasites. A biological standoff ensued. Instead of subsuming these peoples into their larger ranks, the urban north had to find a method of co-existence that would not spell death by disease. The result, a caste system that endured to modern time. Should contact occur between castes, an elaborate ritual of purification must be performed.
In more recent times, and closer to our Western home, the cradle of democracy, Athens, fell in the Peloponnesian War to the Spartan League. The Peloponnesian War began in 431 BC An epidemic of cataclysmic proportions struck the Athenian people and its army over the two years 430-429 BC. One fourth of the Athenian army died. The population of Athens itself was stricken and demoralized. Would they have defeated the Spartans had their army not been reduced by twenty-five percent? The result of Athens defeat is, of course, ultimately unknowable. Would Rome have developed as it did if the Athenian (or larger Greek) empire had flourished?
Two dramatic epidemics occurred between AD 165 and 180 and between AD 251 and 266 that may represent the movement of smallpox and measles into the Mediterranean world. The magnitude of these two epidemics can never be measured with certainty but as much as a quarter of the Roman population may have perished. Five thousand a day died in Rome itself during the second of these two pestilences. Rome was, at the time, at the height of its empire. Roman armies were garrisoned throughout the empire and the cost of their maintenance fell on Roman taxpayers. Should one quarter of the tax base disappear, the impact on the ability of the government to maintain its empire would be dramatic. As the Roman government became less able to foot the bill for its soldiers, Armies disbanded or severed their allegiance to Rome and hired themselves out to those more able to support them. The borders of the Empire became permeable and the results well documented.
The ten thousand years leading up to the 14th century saw our evolution and egress from the parasite rich environment of central Africa to the comparatively disease free environs of less tropical climes. From a relatively sparsely populated species of hunter-gatherers, we began a rapid population expansion through our mastery of the finer points of agricultural development. Confronted with more food than could be consumed by day's end (hunting game for survival or looking for edible fruit tends to occupy most available hours) we were able to focus our energies on more productive (or reproductive) efforts. Commensurate with this newfound agricultural expertise, though was a sudden increase in parasite visitation. Much of our early agricultural effort involved flooding irrigation as many of the Old World crops (rice) dictated inundation. The resulting pools of standing water combined with a more densely packed population that the parasite community had seen hitherto together with large stockpiles of grain products created an ideal environment for parasites and their transport vehicles (rats, fleas, etc.). Mankind again began to be racked by the onset of epidemic infection. Having escaped the tropical parasite paradise, we created another home for parasites in our agricultural combines. Many centuries later, diseases like smallpox, mumps and measles had become endemic and not epidemic. Those who survived repeated periodic outbreaks became immune. Those without immunity were those born after the last outbreak, the children. Measles, smallpox and the like became, in those locales where population was dense enough and conditions right to hasten regular outbreaks of disease, diseases of childhood.
Global population soared as that elusive balance between host and parasite appeared to have been finally achieved. Population in Japan, for example, grew from less than four million to more than ten between the 4th and 10th centuries. Great Britain's inhabitants saw a similar increase in numbers from one to nearly four million.
Thousands of miles to the southeast, another population was enjoying an increase in numbers. In the narrow chambers of an underground city at the foothills of the Himalayan mountains, thousands of rodents lived in relative harmony with a bacterium called Pasteurella pestis - the plague. Like another community of burrowing rodents in Central Africa, they shared their ancestral homes with a parasite that would soon turn life on earth to Hell on earth for its human population.
The plague had made two previous appearances. The eastern Mediterranean world of the sixth century was visited by the Plague of Justinian, almost assuredly a bubonic plague. Less certain, though, is a dim reference in seventh century China. Population densities of the time were insufficient to support host to host transmittal of the disease and the outbreaks, though severe and nearly entirely fatal, faded into obscurity.
Pasteurella pestis has infected burrowing rodents in the Himalayan foothills and Central Africa as far back into history as we can determine. Some of these burrowing rodents belong to the "black rat" species of northern India. Living as they do in the shadow of the Himalayan mountains, they are excellent climbers. Some climbed aboard a ship bound for the Mediterranean. Sometime in the sixth century AD, the first flea leapt from its dying host onto a dockworker near the headquarters of the Eastern Empire, Constantinople. Ten thousand a day died at the height of the ensuing epidemic. The plague had arrived. Within fifty years, Moslem armies out of Arabia would overrun the remnants of the once great Roman civilization. The West was now fully in the throes of the Dark Ages.
The Mongolian empire of the twelfth through fourteenth centuries introduced the first regular trade routes to Eurasia. These trade routes were maintained by supplying outposts with copious amounts of water and grain to sustain the traders on their continent spanning travels. Hitching a ride on fleas on the backs of rats in saddle-bags filled with grain picked up at the last outpost, Pasteurella pestis founded a third colony of burrowing rodents in the steppes of Eurasia. This colony stretched from Manchuria to the Ukraine. One day a few wondering black rats paid a visit and left with a newfound friend. The black rats, though, were not as comfortable with this bacterium as were the native rodents of the underground and began to die off too quickly to support that delicate balance between host and parasite. As they succumbed to a ravaging infection, the fleas sought out a new host. An obliging soldier in the Mongol army at Caffa lent his blood to the flea's cause. The year was 1346. The Mongolian army soon withdrew as its ranks were decimated by a terrifying illness that killed in less than twenty-four hours. Lymph nodes became garishly swollen and the surrounding skin turned black from subcutaneous bleeding. The Mongolian army did not withdraw in time to spare the Caffan population, though. Caffa was a seaport and more than one ship set sail carrying diseased sailors, infected rats and their fleas. The plague comes in two varieties, bubonic and pneumonic. Pneumonic is spread through the air when an infected person coughs or sneezes. Tiny droplets are inhaled directly into the victim's lungs and infection commences immediately. A modern day outbreak of pneumonic plague occurred in Manchuria in 1921. Fatality rates were one hundred percent of those infected. The plague of 1346-1350 was largely pneumonic. One of every three people alive in Europe at the time died. This was only the first outbreak. The plague recurred regularly for the next three hundred years.
The Great Fire of London in 1666 marked the end of the plague in northern and Western Europe. Thatched roofs provided fuel for the fire as they had provided quarters for the plague-infected rats or urban London. The roofs were banned and the rat population dropped precipitously. Simultaneously, a new breed of rat had begun to make its home in London, pushing the older black rats to the fringe. Extraneous events brought the curtain down on Europe's Black Plague. The eastern Mediterranean and Russia continued to suffer into the eighteenth century, until the introduction of antibiotics finally brought the disease under control.
The economic, social, and spiritual toll was, and is, unimaginable. The spiritual optimism of the tenth through thirteenth centuries that gave rise to many of the great cathedrals of Europe gave way to a grim, even bizarre sense of doom and despair. Three centuries of endless rounds of sudden horrible death produced such groups as the Flagellants who beat themselves, and others, senseless in an effort to propitiate God's wrath. So many educated people died that Latin, the language of the learned, disappeared. The Roman Catholic Church was shaken and soon split asunder partly as a result of a rise in anti-clericalism among the people. Easy to understand when the church stood helpless in the face of a grim indiscriminate killer. The Mongol empire faded into the steppes from which it sprang.
On the other side of the globe, an Amerindian civilization flourished. The empires of the Aztec and Incan peoples, along with countless tribes and societies up and down the Americas, numbered more than one hundred million. In the ensuing hundred years, those numbers would be reduced to less than ten million. Smallpox, measles, typhus, and influenza laid waste to the indigenous peoples of America. The population was in an almost pristine state by comparison to its more scarred and disease hardened European counterparts. Diseases that had shrunk into the background as serious but not catastrophic in the European people were unknown in the Americas.
Several factors contributed to this disease free condition. The America's two most important food crops were grown without the use of flooding irrigation. A smaller population spread over a larger area prevented the development of endemic disease that might have carried sufficient antibodies to protect the Amerindians. Animal herds were less dense and further from their human counterparts, reducing the frequency of species hopping parasitic infection. The full range of two millennia of European disease history was unleashed on the Amerindian population in less than a century. The die-offs were catastrophic beyond anything Eurasia had seen. Nine out of ten perished. Pathetically, the indigenous peoples interpreted this result as a sign of their God's desertion. Clearly, the European Gods were more potent. Those that didn't succumb to disease surrendered to despair. Accounts of abandoned newborns and adult suicides abound in the literature of the time. The Old World's disease pool was now shared by the few remaining indigenous New World citizens. The historical ravagers of the human population have settled into long term stasis as childhood diseases or are controlled through antibiotics or, as is the case with smallpox, inoculated into non-existence.
Cholera and influenza have been the 20th century's plagues. The twenty-first century will undoubtedly carry its own new microparasitic plagues. Mad cow disease and HIV may be the precursors of even more horrific threats to our position as this planet's dominant species.
In unraveling the mystery of DNA and its role in cell reproduction, Francis Crick and James Watson in 1952 virtually created the modern science of molecular biology. Every living organism carries within its cellular structure a genetic map in the form of a double helix or ladder of four base compounds. The various combinations of these bases along the helix form the unique basis or genetic code for every living organism. This ladder of base compounds divides and recombines forming replicas of itself. Cell division, growth, results with each cell carrying an exact duplicate of the original genetic code within. Specialized proteins pry the DNA ladder apart and create a single strand replica of a portion of the DNA molecule. This single strand "messenger" is RNA (ribonucleic acid). Another element within the cell nucleus, ribosome, reads the RNA code and uses it as a template to assemble various amino acids into a complex molecular structure called a protein. Proteins make up more than half the human body's dry weight. Muscles, nerves, the liver, even antibodies in the blood are proteins. They assume their roles as directed by RNA, which receives its message from the DNA molecule. This interaction between DNA, RNA and the resulting protein synthesis comprise the foundation of modern molecular biology. In a nutshell, this is the central dogma of molecular biology, DNA makes RNA and RNA makes protein. No living organism can grow or replicate without the essential ingredients of DNA and RNA. Or so we thought. Francis Crick cited the causative agent of the disease Scrapie as a possible candidate for overturning the central dogma of molecular biology.
Scrapie is a disease of sheep first recorded in the eighteenth century in central Europe. The first recorded incidence of Scrapie in England occurred in 1730. Scrapie is a neurological disease afflicting about one percent of the sheep population. Sufferers itch so fiercely that they will scrape their wool and even flesh from their bodies in an effort to achieve relief. Later stages of the disease involve a loss of balance, tremors, blindness, and death. Scrapie appeared in the US in 1947 in Michigan.
That same year brought the first signs of the "shaking" disease to a small indigenous population in New Guinea. The Fore, a cannibalistic aboriginal people, began to suffer from what they called Kuru, after their word for shaking. The disease began as a loss of balance followed by tremors and a general loss of muscle control. Sufferers wasted away as they lost the ability to swallow. Death soon followed. Oddly, only the women and children of the Fore suffered from Kuru. The symptoms resembled Parkinson's or multiple sclerosis, but, unlike those neurological diseases, this malady appeared to be infectious. The Fore people were in the grips of an epidemic of Kuru in the 1950's. Their unusual situation garnered the attention of several first-rate medical and anthropological personnel.
Attempts to identify the causative agent of Kuru were unsuccessful. As the first autopsies were performed on the Fore victims, the pathologists recognized symptoms similar to a rare neurological disorder called Creutzfeldt-Jakob (CJD) disease. The similarity was in the condition of the brain at autopsy. The brains of both CJD and Kuru sufferers were riddled with holes. The brain matter had taken on the appearance of a sponge.
An American research scientist on loan to the British medical community to assist in their search for the cause of Scrapie, visited a London exhibit in 1959 that described an unusual disease among a New Guinea people called the Fore. When he looked at photographs of brain sections of victims of the Fore disease, Kuru, he recognized the telltale damage. The brain of the Kuru victims resembled the brains of sheep that died from Scrapie.
In order to maximize the milk production of dairy cattle, large quantities of protein supplements are administered daily. One of the most concentrated and cost effective sources of protein is found in offal, the remains of butchered animals. Rendering plants, through cooking, grinding, and dissolving animal remains, produce a number of products from tallow to protein supplements. The rendering process leaves nothing to waste as bones and viscera are reduced to powder and liquid and reconstituted for a variety of purposes. Although the primary source product is the remains of butchered cattle, other sources are roadkill, euthanized pets, and downer sheep and cattle. Downer animals are animals that have become lame or ill. Although health regulations prevented these animals from being processed directly into the food chain, nothing prohibited the rendering plants from taking these animals in and producing concentrated protein supplements for dairy cattle. Sheep infected with Scrapie provided a plentiful source for the rendering plants in the UK.
In April of 1985 a veterinarian was summoned by a dairy farmer in Kent England to examine a cow that had suddenly become uncoordinated and uncharacteristically aggressive. Scrapie had "jumped species." Along with the women and children of the Fore people of New Guinea, CJD sufferers and European sheep, now British cattle were falling victim to this strange new disease. As yet, the cause was unidentified. Over the next ten years, 150,000 cattle would fall to bovine spongiform encephalopathy (BSE). Millions of cattle would be destroyed only partially in an effort to contain the epidemic. The primary motive was to "restore the public's confidence in British beef."
In 1993, two British dairy farmers died of what was then diagnosed as CJD. When a fifteen-year-old British schoolgirl contracted CDJ, a disease almost entirely confined to the fifty-plus age group and even then occurred with a frequency of one in a million, the British government was confronted with the awful reality that BSE, like it's predecessor, Scrapie, had "jumped species."
Fifteen years previous, in an American laboratory near Ft. Detrick, Maryland, a variety of similar diseases were being transferred from species to species. These diseases would become identified as transmissible spongiform encephalopathy (TSE). Among the diseases being studied were Kuru, CJD, Scrapie, and transmissible mink encephalopathy. BSE would be added to the list in the 1980's. Utilizing the then new technology of scanning electron microscopy, a young researcher in New York identified what she called "sticks" present in every instance of Scrapie that fell under her powerful microscope. The causative agent for TSE had been observed for the first time, although she didn't know it at the time. The young researcher, Patricia Merz, was home washing dishes when she made the connection between her "sticks" and TSE. She threw up. She called her "sticks" Scrapie associated fibrils (SAF). Meanwhile, a ruthless and determined researcher was approaching the causative agent from a different direction. When Kuru infected materials were exposed to powerful radiation and high temperatures sufficient to destroy any nucleic acids (DNA and RNA) the samples were unaffected. When protein interrupting measures were used, the Kuru samples became harmless. The causative agent, whatever it might be, appeared to contain no nucleic acids. It was pure protein. This was the element that undermined the basic dogma of molecular biology that Crick had inadvertently predicted might someday be found. Here was a material that apparently reproduced without benefit of DNA or RNA. This was protein that made protein. Not only had a new disease agent been identified but an entirely new life form had appeared on the horizon.
Proteins, as they are formed in the cell nucleus from a complex chain of amino acids, at the direction of RNA, fold in such a manner as to create various proteins. When, however, that folding process spontaneously goes awry, a rogue protein is produced. This rogue protein carries with it the ability to break and refold proteins with which it comes in contact. In this manner, large clumps of insoluble, useless proteins are formed at the heart of cells. Eventually, the cell dies and is cleared away by normal body processes. As the replication of rogue protein continues over the course of the disease, the brain begins to take on the appearance of a sponge. This rogue protein is called a prion. The prion is virtually indestructible, it can be frozen, boiled, soaked in formaldehyde, irradiated, and washed in bleach without effect. Certain chemical solvents destroy it. Solvents that used to be common in the rendering process. When they were abandoned in the early 1980's, Scrapie survived to be passed into the cattle populations in England through protein supplements. Those "infected" cattle passed their prions into the human population as beef products. Described as vCJD (variant Creutzfeldt-Jakob disease) this version of TSE continues to kill small numbers of British subjects. Since the incubation period for vCJD is between twenty and thirty years (versus fifty years for spontaneously occurring CJD), the number of British citizens succumbing to this fatal disease should climb over the next few years. A spontaneously occurring prion (like the one thought to be the cause of CJD) is thought to have first made its way into the food chain of the Fore people through their cannibalistic rituals. Since only women and children participated, only women and children were struck by Kuru. Since they abandoned cannibalism in the early 1960's they are now nearly free of their version of TSE.
The origin of prions remains a mystery. Whether they are a regularly occurring mutation or the product of contact with another pre-existing prion is, as well, the subject of speculation. Some of the fields most respected authorities liken prions to Kurt Vonnegut's fictional Ice-9 molecule from his novel Cats Cradle. Crystals form to an atomic pattern. Using an original template as its guide, the process of crystallization proceeds unabated when conditions (e.g. below 32 degrees F water) permit. Without a pattern to mimic, however, elements that would normally crystallize enter a state of "super saturation." Once the pattern is introduced, crystallization rapidly commences. That pattern is called the nucleating agent. The rogue protein, or prion, may serve as such a nucleating agent, supplanting the normal protein schemata with its own.
Where the incidence of prion "infection" is a naturally occurring one, as would appear in CJD, the incidence of infection is fairly low. Where we have helped the process by concentrating the prion source through consuming flesh or flesh by-products, the incidence of "infection" (infection appears in quotes because this is not an infection in the traditional sense as no immune response occurs) grows dramatically. Whether through the cannibalism of the Fore or the hamburger from the fast-food restaurant, the exposure to prions occurs whenever we consume flesh. The milk from prion carrying cattle contains prions. The droppings of prion "infected" sheep (Scrapie) contain prions. Most governments have banned the use of mammalian products in the feed for cattle and sheep. Pigs and chickens, however, continue to be fed rendered food products. No pigs or chickens have been diagnosed as prion carriers. The life cycle of pigs and chickens is too short to allow manifestation of TSE symptoms. That only means they haven't seen the full TSE cycle, it does not mean they are free of prions.
Rose foods made from bone meal now carry a warning not to open indoors.
The fine dust could be inhaled.
Update Christmas eve 2003 - the US government announces a cow from a herd in Washington State has tested positive for BSE. Mexico, Japan, and Chile have banned the import of US beef, effective immediately.