Tuesday, August 19, 2014

A Stranger on this Planet

I was born with Klippel-Feil syndrome (KFS).  Named after two doctors who first discovered it in France in the early 1900s, the main characteristic is fusion of the neck before birth.  I was also born with a cleft palate and hearing defect.  The doctors didn’t want my parents to see me.  Instead, they wanted to stow me away in an institution.  They informed my parents that I would die or become a “vegetable.”  My parents ignored the so-called professionals’ dire news and took me home after a month in an incubator.

Because I was so small when I was born, the surgeon chose to wait until I gained some weight to operate on my cleft palate.  I couldn’t suck, so my mom had to use a syringe-like instrument to pour milk down my throat.  After six months, I had the surgery.  I had a second operation in my mouth when I was eight years old to correct my speech.  Until then, no one but my sister could understand me.  She was my interpreter.  Unfortunately, this second surgery caused me to develop sleep apnea, of which I didn’t know I had until my mid-30s.  I also couldn’t breathe through my nose or blow it.  I used a mini turkey baster-like device to suck out the nasal discharge.  When I became an adult I was finally able to blow my nose, although I still can’t breathe through it.

In my elementary years I developed kyphosis, like a hunchback, so I wore a brace that was a metal cage for my torso.  The metal put a hole in every one of my shirts.  I wore that thing for five years, twenty-three out of twenty-four hours a day.  It didn’t hurt me, but it was a lazy excuse for bullies to tease me.  I paid them back by telling them to put their finger under my chin.  Then I squished it between my chin and neck brace.  Ha-ha!  Sweet revenge.

In my teens I developed scoliosis, so I wore a muscle stimulator at night, every night, for two years.  Rubber pads were put on the shoulder blade that stuck out because of the crooked spine pushing against it.  The pads connected to an electric box that would give a quick “shock” every minute to press this shoulder blade against the spine, to keep the curve from getting worse.  If the pads weren’t put on right, it felt like a bee sting.

I also developed an underbite over the years, so much so that I couldn’t chew meat or hard foods.  I had jaw surgery as a teen, awake since I couldn’t be intubated.  The surgery lasted four hours.  I remember the saw that cut the bone, which vibrated my head so much that I feared I would pass out, if I didn’t focus on one spot on the ceiling. The saw sounded like a drill, and for years after that I felt nauseous and dizzy every time I heard one. I had nightmares years after of losing my teeth, or  my jaw falling apart.

Finally, my hearing loss.  I was born with some defect in my right ear.  As time went on, I had recurrent ear infections so I had to have ventilation tubes put in my ears.  I still do, as my Eustachian tubes, the connection between the ear and the throat, are unable to properly drain.  No one knew about my loss until I was three, my being able to adapt so well.  In high school I acquired hearing aids.  Finally I could hear a cat’s purr or a frog’s croak. 

Since KFS was so rare, I felt like an alien from another planet that got lost on my journey and found myself in the wrong place.  Seven years ago I started an online support group for others like me.  We have more than a hundred members, about ten regular contributors to the discussion.  Now there are other KFS groups online for other languages.  The worldwide web helped us come together in a way never thought possible before, and for that I no longer feel alone or a stranger on this planet.


Julie Johnson said...

God Bless you Amy! You are are a beautiful lady :) I feel blessed to know you. Keep writing my friend!

amyjean1010 said...

Thank you! So good to hear from you!