Message From The Editor:  A Vision For The Future

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One day in the hopefully not too distant future...

   "Keith Shuler!"
   The nurse held the door open as Keith tossed the magazine he was reading onto a table and walked towards her.  "Good morning," he said.
   She gave him a half-smile, half-nod, half-grunt and lead him down a hallway decorated with holly and Christmas lights and into an exam room.  She asked him to step on the scale and dutifully noted his weight along with  his blood pressure, pulse, and temperature, all without saying a word.  "Now," she said, looking up at last. "How old are you?"
   "I'm thirty-two."
   "And what brings you here today?"
   "I've been getting headaches when I play basketball," Keith replied.
   "How would you describe the headaches?"
   "I get an intense pain back here," Keith pointed to the back of his head.  "It's like there's a fist inside my skull  squeezing my brain.  Then, if I lie down for a few minutes it just goes away.  At first I didn't think it was any big deal, but I now I'm not sure.  I think they might be getting worse."
   "And how long have you been having these headaches?"
   "About two to three months.  They just started getting really bad the last couple of weeks.  I had one two days ago that didn't go away for almost half an hour.  That's when I called."
   "Any other problems...colds, congestion, fever...anything like that?"
   "No, not really."
   "OK.  Are you on any medications?"
   "No, I don't like taking medicines."
   The nurse finished writing, gathered her paperwork and started to leave, "Dr. Hennigan will be with you shortly."
    A few moments after the nurse left, Keith started to fidget on the exam table and got up to look around the room.  There were the usual posters of body parts and explanations of diseases.  There was a small desk with a stool and another chair beside it.  Keith decided to sit in the chair.  He didn't feel sick and sitting on the exam table made him uncomfortable.
    There was a sharp knock on the door and Dr. Hennigan came into the room.  A compact man in his late forties, his energy immediately filled the room.  He shook Keith's hand, sat on the stool next to Keith, and said, "Good morning Mr. Shuler.  Good to see you again.  What seems to be the problem?"
    Keith repeated his story of the headaches adding that he knew it was probably nothing, but they were getting very painful.
   "No, you were right to come in, " Dr. Hennigan said.  "Headaches are often a sign that something might be wrong.  Have you noticed any other activities that give you headaches.  Do you jog?  When you sneeze?  When you cough?"
   "Now that you mention it, I had a cold a couple of weeks ago, and when I coughed it would kind of hurt in the same spot.  I haven't really jogged lately.  Oh, I almost forgot, sometimes if I bend over for more than a few seconds I'll start to feel the pressure building."
   "What about your balance, any problems there?  Do you find yourself unsteady, or falling down?"
   "Maybe a little.  Sometimes I walk into doorways.  I never paid it much attention."
   "OK, let's have a look at you."  Dr. Hennigan asked Keith to sit on the exam table and proceeded to look into his ears, throat, and up his nose.  Next he had Keith squeeze his fingers and checked the strength in Keith's arms and legs.  He got out a reflex hammer and spent some time tapping Keith in various spots.  Finally, he spent quite a bit of time looking into Keith's eyes.
   When he was finished, Dr. Hennigan smiled at Keith, "Well, there are a couple of possibilities.  Before we go into details, the best thing to do with headaches like this is to get an MRI of the area to see what that shows us.  We'll also take some blood to see if that's normal.  I really don't think its anything to worry about.  Let's see what the tests say.  OK?  They can set up your MRI visit out front."
   Keith thanked the doctor, set up a time for his MRI, and left wondering if there was more to the headaches than he had suspected.
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   Three days after his doctor's visit, Keith arrived at the MRI center fifteen minutes before his appointment.  After filling out the requisite paperwork, a technician escorted him to a second waiting room and reviewed his medical history and asked a thousand questions about metal implants.  Keith changed into a gown and was led into the actual MRI room.
   The room was cold and stark, dominated by the big MRI machine.  The technician, a young guy with a pony tail, situated him on his back and placed a Hannibal Lechter looking device on his head.
   "Now," the technician said, "we'll slide you into the machine.  I'll be able to talk to you and you'll be able to talk to me.  If you start freaking out, just squeeze this.  How long you're in there will depend on what we see, but the whole test should take less than fifteen minutes.  You ready?"
   Keith gave him a thumbs up and the tech pushed a button and guided Keith into the machine.  It was bright inside and crowded, but not claustrophobic.
   "Can you hear me?"  The tech's voice was coming through a microphone.  Keith said he could.
   "OK.  The first scan will only take a minute and then once the computer looks at that, we'll know what else we need to do.  Try not to move."
   The machine banged, popped, and whirred for about a minute and then stopped.  A short time after that, Keith heard the tech's voice again.  "We're going to go ahead and do some more tests.  Nothing to worry about, we just want to check some things out.  You'll hear a series of noises for about 5 minutes."
   Keith tried to stay calm and lie as still as possible, but inside he knew something was wrong.  They would only do more tests if they found something bad.  He wondered if it was a tumor.
   "One more test Keith, you're doing great.  This time, when I say go, I want you to cough as hard as you can without moving too much at least five times."
   Keith complied and then the test was over.  The technician said he'd hear from his doctor in a couple of days.
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   Dr. Hennigan called Keith the next night.  "The MRI did find something, but it's not that serious and it's very treatable.  It's called a Chiari malformation.  I'd like you to see a neurosurgeon.  His name is Dr. Bellow.  He's very good, he actually operated on my back."
   "A neurosurgeon?" Keith asked, his voice cracking a little with nerves.
   "Please trust me, it's not very bad.  Dr. Bellow will explain everything to you.  Believe me, in the scale of things that can be wrong, this one is barely on the charts.  But it does involve the brain, so the neurosurgeons are the ones who handle it.  I think you already got all the tests you need, we'll forward all your information and my people will set up the appointment for you.  I'll check in with you after you speak to Dr. Bellow."
   Dr. Hennigan was very reassuring, but when Keith hung up the phone, a kernel of doubt remained.  He wondered what a Chiari malformation was and decided to do some research on his own.
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   Keith had found a lot of good information about Chiari on the web, and he was feeling pretty good about things as he waited for Dr. Bellow in the exam room.  Everything he read said the condition, if caught fairly early, was treatable with no lasting effects.  It was the treatment he was still a little worried about, it sounded like brain surgery was the main option.
   Dr. Bellow broke his reverie with a brief knock and a quick entrance.  Keith placed him in his fifties with a Caribbean winter vacation type tan and a confidence that immediately put Keith at ease.  He introduced himself and asked Keith some questions about his headaches.  He then proceeded to repeat the strength tests and reflex check that Dr. Hennigan had performed in his office.
   When he was done he sat down and looked Keith in the eye, "I know Dr. Hennigan informed you that you have a Chiari malformation.  Do you know what that is?"
   "Yes, I've done some research."
   "Good, we'll skip the basics.  Basically, you have a mild case that is starting to become symptomatic.  Because of the headaches, and the risk of letting it go, I recommend we go ahead and treat it.  The MRI you had done created a mathematical model of the anatomy of your skull and spinal area and also how the fluid in the area moves from your brain into your spine and back.  From this information, we can model how you will respond to different treatments - meaning surgeries - so we know pretty much exactly what we have to do to correct the situation.  When you coughed in the MRI, it showed us how your brain/spinal system responds to abrupt pressure changes.  Are you with me so far?"
   "I think so.  Like I said, I did a bunch of reading.  Can we talk about the actual surgery?"
   "Sure.  In your case, it will not take much to correct the situation.  We will drill a small hole in your skull right around where you get the headaches.  We will then expose the brain with a very small incision.  We insert a small device that can shrink the cerebellum - specifically the tonsils that are hanging down and crowding the spinal area.  There's an MRI machine built into the OR which will help guide us in how much to remove.  We will also inject a drug which will make your dura - the covering of your brain - more flexible.  For reasons we don't quite understand yet, the dura in some people gets too stiff as they reach your age and that causes a problem.  Keeping the dura flexible lets us make a minimal structural change and not worry about problems down the road.  In addition, your genetic screen shows that you are susceptible to developing a syrinx in the spinal cord if the problem were left untreated.  We will inject another substance which should help your body resist that just in case.  The whole surgery will take about 90 minutes.  You'll spend one night in the hospital.  Rest a week or two at home just to make sure everything is ok.  Then it's back to normal.  What do you think?"
   "Wow.  You make it sound so straightforward.  What's the success rate?"
   "Greater than 95%.  For you, since there aren't any other related problems, the chances of success are even higher.  I do about 5-10 of these a year and in the last 5 years, I only had one patient that we had to go back in for.  And she did fine after the second surgery."
   "How do you define success?" Keith asked.
   "Complete recovery.  No symptoms.  No restriction on activities.  Like there was never anything wrong."
   Keith let out a big sigh of relief, "Can anything go wrong during surgery?"
   "There are always risks, but the complication rate is less than 1%, and serious complications are very rare.  The biggest risk is probably if you have a bad reaction to the anesthesia."  Dr. Bellow glanced at his watch, "So what do you think, should we do it?"
   "Yeah, let's do it."
   "Good.  My people will set it up."  Dr. Bellow started to leave and then turned back, "You know you're lucky.  When I started my career, things were much different for people with Chiari.  We were barely scratching the surface of understanding it.  People went years before being diagnosed - some were even told they were crazy.  Most primary care docs didn't know anything about it, and even some neurosurgeons.  About 50% of the time, a cyst would develop in the spine, very painful, and would result in permanent nerve damage.  And the surgery... it was black magic.  Every surgeon did it different.  It was very traumatic and failed as much as 30% of the time.  We'd take out a big chunk of your skull, some of your spine, cut the dura wide open.  Anyway, you're lucky."
   Keith didn't know what to say.
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   A few weeks later Keith was at home watching TV when Dr. Hennigan called.
   "How are you feeling Keith?"
   "I feel great.  I'm going back to work tomorrow. I shot some baskets today no problem.  No headaches, nothing.  It's like there was never anything wrong.  I guess I feel like I dodged a bullet."
    "Good.  Barring any bad luck, I guess I wont' see you for awhile then, right?"  Dr. Hennigan asked.
    "I don't think you will be seeing me.  I feel lucky."
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Obviously, this is a fictional story...but it doesn't have to be.

- Rick Labuda

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