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Sunday, March 22, 2009

Tough Question (part 1)

I dread the tough question. It's inevitable. People are
curious beings. They want to know and understand. So they
always ask it. "How did you become deaf-blind?"
I just pause and groan. Here we go again. It's not that
I'm offended. I don't mind people asking questions. It
helps them learn, which is always good. The problem is
that it's rather complex. there's no easy way to explain it
I sometimes envy people with Usher Syndrome. Usher Syndrome
is the leading cause of deaf-blindness in America. It is a
rare genetic disorder. People with Usher Syndrome are born
deaf or hard-of-hearing. They usually begin losing their
peripheral vision as young adults. The visual loss is
called Retinitis Pigmentosa (RP). The progression of loss
is very gradual. The world slowly closes in on them. some
people with RP become totally blind. Many retain so central
vision, but often have a visual field less than ten degrees.
They also suffer from night blindness and poor balance.
Everybody in the deaf-blind community knows what Usher
Syndrome is. For those who have never heard of it, it's
not too hard to explain. If I had this disease, I could
just answer the question by saying, "Usher Syndrome." Nice
and simple. But I don't have Usher Syndrome.
Okay, pull up a chair. Get comfortable. It's story time.
I'm afraid this one will be quite long. Try to stay awake.
There will be a quiz afterwards.
I was born with no disabilities at all. In fact, my family
had no hints of the troubles that lay ahead. I suppose it
began when I was seven. My brother Charles was twelve at
the time. Another student threw a pencil and it hit
charles in the eye. He had to go to the doctor and eye
specialist to treat the injury.
While examining his retinas, the doctor made a sad
discovery. Charles was diagnosed with RP. He already
tested for reduced field of vision. My parents were told
he would probably become blind by age 20. They were
Because RP is hereditary, my other brother and I were tested
too. Neither one of us had symptoms of RP. I wore
glasses, though. The doctor warned my parents to watch me
closely for signs of trouble.
Over the years, Charles tried desperately to prove that he
wasn't blind. He seemed to think if he could drive, play
soccer, and work as a life guard, there was nothing wrong
with him. Coping with his visual impairment would be a life
time struggle for Charles.
I was thirteen when it all started for me. My parents
noticed I wasn't responding when the spoke to me. The
doctors shrugged it off and said, "Oh, she's just being a
teenager." But it got worse.
Finally my parents took me to an audiologist. I was
diagnosed with a mild-moderate hearing loss. He assured my
parents that I wouldn't lose more hearing. I was fitted
with hearing aids. They were supposed to make everything
I continued to lose more and more hearing. The hearing aids
didn't work. They actually seemed to make the problem
worse. But there it was again. "She's just being a
teenager. she doesn't want to look different. She won't
try." How I hated those words!
I fought constantly with my parents about wearing the
hearing aids. I secluded myself in my bedroom. It was the
only place I could find peace.
By age sixteen, I was profoundly deaf. I had lost all my
friends. I had no boy friends. I couldn't hear on the
phone or understand movies and TV. I couldn't understand my
teachers, either. I never knew about home work assignments
and tests. My grades were lower every semester.
Everything was out of control. The specialists, who seemed
to know everything about everything, told my parents not to
allow me to learn sign language. It was the easy way out,
they insisted. If I would wear the hearing aids I would be
able to understand speech. Sign language would make all my
problems worse.
I was sent to the area's leading hearing center. They had
me attached to some machine that showed a picture of my
inner ear. I couldn't move because something was stuck in
my ear. That's when they told my parents I had Usher
Syndrome. My mother burst into tears right in front of me.
I felt helpless. Her reaction confirmed my own thoughts.
My life was over.
This was, in fact, the first in a long line of misdiagnosis.
They looked at my brother's RP and my deafness and concluded
I had Usher Syndrome.
I went to the hearing center every other month for
evaluation. Still the loss progressed. Finally, near the
end of my second year in high school, someone listened to
The audiologist had set me up in a booth for a speech
discrimination test. I was supposed to listen to a tape
recording and repeat what I heard. I waited a long time
but nothing happened.
She came back with a puzzled look on her face. Then she
said, "Let's try something a little different." She told me
to raise my hand if I heard the voice. "Don't worry about
what the person says. Just tell me if you can hear it."
Then she started the test.
I was so relieved. This was something I could do. I
thought I did it right. Actually, I failed miserably. But
that turned out to be a good thing.
We went into a meeting room with the audiologist, ear
doctor, and my parents. The audiologist announced, "she
can't hear. She really can't hear." I wanted to cry. I
had been telling them this for two years. Now they believed
The doctor decided I was a good candidate for a Cochlear
Implant. Most people know what that is now. But this was
back in 1990. It was still new and considered experimental
for children. Since I was only sixteen, I was considered a
I had to go through extensive testing before my surgery.
Because this was experimental, they needed all kinds of pre
and post-op data.
There was one serious problem. How to pay? Insurance
won't cover experimental treatment. My parents believed
that if this was meant to be, it would happen. They would
find a way.
The Vocational Rehabilitation counselor working with my
brother had an offer. Despite my diagnosis of Usher
Syndrome, we had not yet confirmed if I actually had RP.
She told them if I was tested for RP, her office would pay
for the Cochlear Implant.
My parents were horrified. With all I was facing
emotionally, they were not about to put me through the
trauma of testing my vision. They refused the offer. But
they were right about something - they did find a way. As
it turned out, my grandmother died one week before my
surgery. Her estate helped fund the medical bills.
The Cochlear Implant was a great success for me. It allowed
me to once again hear sounds and voices. By combining the
sounds I heard with visual cues of lip reading, I was able
to understand speech. Within weeks of being activated, I
was wearing my Cochlear Implant processor full time. So
much for "being a teenager." I never cared about being
different. It worked so I used it.
This was a roller coaster year for me. I was doing better at
school and made some new friends. The other students my
age were enjoying the new freedom of driving. I wanted that
too. But I was also scared. Before I could start driving,
I would have to face the big question. Did I have RP?
Charles did not do well with driving. He was in five
accidents in a year before he finally gave up his license.
He was lucky he never got hurt or hurt anyone else. It was
a scary time for him. My parents didn't want me to repeat
his mistakes. If I wanted to drive, I would have to have
my eyes tested.
At first, I wasn't ready. I wanted to drive, but not enough
to deal with this. As time went on, however, the urge grew.
Finally, I agreed. It was only a few months before my 17th
birthday when I was tested. My hopes were crushed in an
instant. I had RP too. I never got to drive a car.
The years passed. I finished high school and went on to
college. I considered myself hard-of-hearing and visually
impaired. Most people didn't even know I had a vision
problem. I functioned well enough that I never felt the
need to learn sign language or walk with a white cane. My
parents were so proud when I graduated Summa Cum Laude with
a triple Special Education major.
I moved out of state and got married. Around this time, my
entire family sent blood samples to Boys town Research for
an Usher Syndrome study. The results came back. Although I
was deaf and had RP, I did not have Usher Syndrome. My
diagnosis was changed to Retinitis Pigmentosa and hearing
loss due to unknown causes.
My husband and I did not undergo genetic counseling before
having our first and only child. We were not concerned.
The risks were very low. He would have to be a carrier to.
And even if they child did have RP, he would only have
visual problems. My hearing loss was not genetic. And, so,
JD was born. This stars a whole new chapter in my life.

(Coming soon - see part 2 for more.)

1 comment:

  1. This recent Dotbug Blog entry is a great example of what individuals who have disabilities are subjected each and every time professionals ask the necessary probing questions about etiology. It is hard to know what aspects of an individual’s life are significant enough for us to hear about when attempting to provide support and which aspects should remain private. My training has taught me to probe thoroughly so that something critical is not missed. Dotbug has revealed a couple of things to me, probing can be very informative for me, but painful for the consumer who has to repeatedly relive painful past experiences. And, even the most painful probing can result in important revelations that would otherwise remain clandestine if questions are not asked. Without any probing on my part this entry revealed to me the tenacity of Dotbug’s spirit, how brain plasticity can contribute to successful education and functional life outcomes and most importantly – learning to listen to the essential voices in our lives often has very little to do with our ability to hear with our ears.