Vestibular Research as a "lab-rat" for NF2

Total darkness

Chair moves left

Was that right or left

I think that went right

oh, definitely left

Lights come on-

then off, again.

Chair moved down

and down, again.

I know I can't say down-

too many times-

I'd go through the floor.

I think that was up.

Was that up or down

It is confusing

hard with no balance

Tilt to the right

Right, I think

Feeling weary now

Chair tilts to the left

Lights come back on.

I hear “start” and beep

I hear “respond” and beep

I have to decide

which button to press

I press the right

then press the left

Repeat hundreds of times

It was mentally exhausting... when I got back, I think I slept for 9 or 10 hours!

---------------------------------------------------------------------------------------------------------

 explanation...

I had an interesting experience on Thursday, I had two appointments and pre-op in Boston for my NF2 , but during the short time breaks before, after, and between each of those appointments and then some more on Friday, I participated in about eight hours of a vestibular research project at Massachusetts Eye and Ear Institute, that I had been asked to take part in. (They paid be a little compensation to do it, which was a nice bonus.) It was especially interesting because they had never done the test with a person with bilateral loss of balance nerves, nor a deaf person, for that matter. I had to sit in a strange looking big robot chair that moves on some tracks, in complete darkness, and then press either of two buttons to indicate which direction I perceived the chair motion having had made. My feet were on a platform below and moved in the same motion as the chair. It could move side to side, or up and down, or tilt right or left. Sometimes I could tell my feeling the direction my body moved, but other times I just had to press a button and guess! This task would be difficult for any person, but I think they were particularlly interested in working with me because they know I have no intact balance nerves, however do these things that I'm “not supposed to be able to do,” such as marathons, triathlons, etc.

My Mid-Life Curveball

I’m a 50 year old woman with Neurofibromatosis Type 2 (NF2
for short) but I didn’t know it until the winter of 2001.

Rewind to the summer of 1973. I was young,a mere twelve years old. I was lucky too, living out a childhood that was full of love, fun and unencumbered by any needs or wants. I spent every summer at the Jersey shore where my grandparents had a house on the bay. We belonged to the local yacht club were I learned to swim and to sail. I had a circle of many friends and we spent our summer days at the beach, swimming in the bay and riding around in our motorboats, sailboats and jet skis that we were so privileged to have. We also wrestled around a lot and on a clear early summer day we were across the street from a friend’s house playing on grass that was like a cushion, someone grabbed my upper left arm, hard, and I howled in pain. It was at that point that the twelve year old started to grow-up. Running home to my mother for comfort, I looked at my arm and could see that it was visibly larger than my right arm and had funny looking bumps just under the skin running all the way down my arm to my wrist. Why hadn’t I noticed this before?

I was scared. I vaguely remember thinking “CANCER”! After much reassurance from Mom, Dad and my Grandfather I was begrudgingly convinced it wasn’t cancer but worried that it was likely something just as bad or worse.

My privilege kicked in again. Because my Grandfather was a doctor he had “connections” at the Hospital of the University of Pennsylvania. He got me an appointment to see a world-class neurologist. I felt I was in good hands or at least that’s what my parents and grandparents kept telling me. I worried. Boy, did I worry. I’ve always been a world-class worrier but I was having visions of having my entire arm amputated. After a series of painful appointments, painful biopsies and painful EMGs (electromyographs) that lasted for over two years, a diagnosis was forthcoming. When I heard my world-class doctor say “a very mild case of neurofibromatosis type 1” I looked at my parents, they looked at me and after a collective sigh we asked “What’s that”? The doctor was generous with his time and explained to us that neurofibromatosis type 1 is an incurable disease of the peripheral nervous system characterized by café au lait spots on the skin, and the growth of tumors along nerves in the body. He said I had an atypical case but that the symptoms of this disease vary widely among affected people. He also said that he saw no reason that I couldn’t continue to grow up and have normal teen years and an adult life. I felt an enormous weight lift – I was going to be ok! My
parents and I were so relieved.

Even though I continued to have to cope with the pain in my arm, I never let my NF1 get in my way. I continued to spend summers at the shore, continued to live my privileged life. I began racing sailboats at our local yacht club and at other clubs in the area. And I was good. I was winning! I sailed competitively for about 6 years until I was 17 or 18 and won a lot of races and received a good share of the trophies too. I went deep sea fishing with my father and reeled in a lot of fish. A sport I continue to enjoy to this day. I learned to waterski and snow ski. I learned to surf too, but not well! I was even a bit of a dare devil! I excelled at school too. My father was a History Professor at a local college that I enrolled in after graduating from high school with honors in 1978. I graduated from our local college and went on to study Biology at Trenton State College. I had a high bar to meet because my father had graduated number 1 from his college. I was driven to match his achievement. All the while I barely gave my NF a second thought. As a science major I took it upon myself to learn more about my NF1 but never let it hold me back. I graduated number 1 with a 4.0 grade point average and received my Bachelor of Science in Biology. Take that, DAD, I thought!

After working at several low-paying jobs in labs in academia, I began working for Bristol Myers Squibb where the pay was considerably higher! Several years later I had the opportunity to work for a small startup company on Long Island New York doing cancer research. I would have to move out of my parents’ house, out of New Jersey and move, alone, to Long Island. I didn’t really give it a second thought. I moved, never even considering my NF1.

Fast Forward to the fall of 2002. I was 41, married to the love of my life, Henry, still working at the startup company on Long Island and living in New York City! We were child-free, a decision we made together since there was a 50% chance I could pass the gene and therefore the disease onto each of my children. Thankfully I had the good luck to be married to the only man I know that didn’t really want children. Neither one of us wanted to take the chance because even though I had a mild case of NF1, my children could end up with much worse.
So, I loved my husband, my job and living in NYC. I also still spent some time (weekends, vacations) at our house at the shore that was now my owned by my parents. Henry and I took advantage of living in NYC. We went to plays, to concerts, to marches, to dinners, to friends. I thought about my NF1 over the years but never saw a doctor for it. I remember having some fleeting thoughts like “Thank goodness I have a mild case of NF1 and not the other more serious type called NF2.” At least I thought NF2 wasmore serious. My work life was going well too. We had discovered a compound that had a good chance of becoming a real anti-cancer drug and I had been in on it. I was in the group that discovered it and had done much testing on it along with colleagues. I had visions of our group on the cover of Time Magazine! It was named OSI774. And sure enough, through a great deal of work our compound passed clinical trials and was approved by the FDA with an indication in lung cancer. It was named erlotinib and marketed as Tarceva. Whata thrill it had been! My privilege had kicked in again. In fact what a privilege it had been to have worked on it! But my privilege was soon to take a serious dive.

I wasn’t feeling all that well. In fact I had this ringing in my ears that wouldn’t go away, my balance was off as if I were dizzy and the pain in my arm had gotten considerably worse. Motrin wasn’t killing the pain anymore. I had a sneaking suspicion that my NF1 was acting up so I made an appointment to see a neurologist. She examined me and ordered an MRI which I’d never had before. I was shaken. But I made an appointment to have an MRI and went. Essentially you have to lie very still in this coffin-like tube, the room is cold and dimly lighted and you only have a gown on. The platform moves back until your upper body in inside the very large circular tube. When the technician starts to image, very loud, extremely loud magnets sound like they are going around your head inside the tube. If you have pain it is very hard to endure lying completely still for about 40 minutes! Well, I made it through the test and a few days later the neurologist called and told me the image of my brain showed some tumors and she wanted me to see a specialist. Tumors? I was really scared.

My husband and I went to see the specialist. She got right down to business practically blurting out, “There’s been a mistake, you don’t have NF1 you have NF2.” So now I thought my privilege and luck had finally run out. My mid-life curveball.

I was devastated. NF2 is progressive (as is NF1) but it means brain surgeries and more pain and balance/falling problems. I would go deaf, my facial nerve might be cut during a surgery permanently deforming myface. Paralysis, amputations - it was all too much for me. My head was swirling that I literally almost fell off the examine table. And then came the tears. I was inconsolable. All the assumptions I had made about my illness had been WRONG. For over 30 years I had learned to cope with NF1. Now I would have to start coping with a not so mild case of NF2.

Fast forward to present day. I am a 50 year old woman with NF2,not NF1. Since my re-diagnosis I have had a rocky road. I had to replace one set of coping skills with an entirely different one. And my disease seemed to be progressing all too fast. I have already had one brain surgery with more likely. I’ve had to cope with a major jump in pain. My backand legs hurt as well as my arm. I have scoliosis. But I found a dear pain management doctor who got me on the correct combination of medications that made coping with the pain manageable. I have learned that with my many doctorsthat I am my best advocate – ask and I receive! There was a considerable amount of guilt too. My father was sure he’d passed this onto me but there is no familial evidence that he did. Up to 50% of new NF2 cases are due to a spontaneous mutation in the NF2 gene and not genetically passed. I had to go on permanent disability so I retired from my dearly loved job. Much of our self-esteem comes from what we do and I had lost mine. I had to find something to get my mojo back. It took years but I have found several projects to work on as a volunteer which has made life a whole lot better. And there was depression too. But I found an absolutely wonderful therapist whom I still see. She has taught me so much. Thanks, Sharon.

So life with NF2 when you get right down too it is not unlike life with NF1. It’s mostly just a different set of coping skills and support groups. Thank goodness for Facebook, Inspire and the NF2Crew on Yahoo Groups. I’ve met a wonderful circle of new friends. Some of my new friends have NF2 and some don’t but they all understand NF2 issues and challenges. Thanks to Jaime C., Lori, Marie, Barbara and Megann! They are a wonderful support system.

I’ve enrolled in a clinical study at the NIH about the natural progression and history of NF2. Lately, I am helping a new friend with her website which is an NF2 support and information website. We are working on a list of drugs that are either in NF2 clinical trials or candidates for them. She sent me the list and there in black on white I met on old friend. The compound that I had worked on more than 10 years ago and watched get FDA approval, OSI774, erlotinib or Tarceva was on the list that might be a good candidate for NF2 trials. And THAT has given me the greatest sense of privilege and accomplishment I’ve ever known!

The Meanies Social Group and Elliefest 2011

They went the distance for this little girl.

The town let them plug-in and use town

facilities for cooking etc.

The Electric company brought a bucket truck to help

hang her banner.

I made some great friends that day,

The Meanies became my family,

the prayer warriors did too.

The creator of Elliefest seems to be missing from this picture. I will

have to replace it with another.

Calling... (Contributed with permission by Jess S.- Julia's daughter)

It starts as a whisper.
Many won’t hear it, but those that do...
Will start searching for it.
Some will look high.
Some will look low.
Some will look in.
Some will look out.
Some will look over.
Some will look under.
Some will look in the dark.
Some will look in the light.
There will be some that take to the skies.
And some who will take to the seas.
And deep into the forest.
Those that find it...
Will do many things.
Be they big or small.
Great or Grand.
They will change the world.
Because they found their calling.
So if you hear a whisper.
Go catch it.

Silenced Marathon

(WRITTEN IN 2008)


For eighteen years in the hearing world in which I lived I thought “deaf” was the worst insult anyone could fling at me. When a doctor told me in the fall of my senior year of high school that I would become deaf at some point in my life, I responded, “Yeah, maybe when I’m 80!” I never dreamed that six weeks later, I would lose the hearing that I had taken for granted all my life.

**** Prettiest Smile ****

It is a hot summer day in 1996; I am nine years old. (Yawn!) … “Anne… Sweetie… Wake up! It is Saturday. Will you help me make the pancakes? Shhhh… Mommy and Ken [my little brother] are still asleep,” I hear Dad say. I am hungry, so I bound out of my cozy old double sized bed that lies in the center of my big bedroom, with light green painted walls surrounding me. I dash downstairs, as if I were running from something. I get out Aunt Jemima Buttermilk pancake mix, 2% milk, 2 eggs, Canola oil, syrup, a bag of Nestle-House chocolate chips, and a brown-speckled ripe banana. As I mix the ingredients in a large bowl, and enjoy the feeling of squishing the unpeeled yellow banana running between my tiny fingers, Dad tells me that he got more Olympics tickets for my family to see some games in the next few weeks. “Maybe I can be in the Olympics someday”, I think to myself. I watch Dad flipping the oozy slabs of light brown batter into the air, and then like magic each one lands perfectly back on the hot griddle. I walk outside to feed our old yellow Labrador retriever, Destin. I feel the heat of another summer day in Atlanta as the sun bakes against my youthful pale skin; it is a perfect day to go to the neighborhood pool.
(Squeak) I swing the kitchen door open. I see that Mommy is awake now, and the smell of scrumptious pancakes fills the air. She is taller than me, beautiful like a Dalmatian, dark brown hair, smells of flowers, Pantene shampoo, and Lever 2000 soap. She is wearing her long pink terry bathrobe, as I run up to hug her. She whispers into my ear “I love you.”
Mom, Dad, Ken, and I sit at the circular kitchen table; I happily gobble down my pile of pancakes. Mom comments to Dad; both look at me and ask why I am making goofy faces. I tell her that I’m not making silly faces or playing childish games. Mom walks me to the bathroom; she tells me to look into the wide mirror, which covers half of the wall. I obey. I look into the mirror, but I walk away as I deny seeing anything. It is the summer after second grade. One side of my face has become paralyzed, overnight.
Within ten days my parents had me on a plane to California for surgery to remove a tumor on the auditory nerve of my right ear. I was already functionally deaf in this ear since I was seven years old; that was also when I had learned that I have Neurofibromatosis Type 2 (NF2), a rare genetic disorder affecting 1 in 25,000 birth, which commonly causes benign tumors on the auditory nerves and spinal cord. While I was very scared, I knew the surgery had a great outcome, because I woke up grinning from ear to ear, something that I couldn’t do before going into surgery. With the medications and lack of activity, I gained a lot of weight that summer. When I returned to school to start third grade, I had a horrible time. I was living in a nightmare, a nightmare that I couldn’t wake up from. At night I cried myself to sleep. There was a mean girl and her friends on the playground who harassed me every day and called me “pregnant.” My friends from Girl Scouts didn’t know what to do at that young age, and stopped sticking up for me. I wanted to hit her, but I knew that was not right; I ran away and cried.
**** Cookies + Brain Surgery = Babies ****
I am chubby. I am ugly. Half of my head is shaved. I wear a bandana, trying to hide, to hide from the world. I wish I can disappear. I am only nine years old, and those mean girls call me pregnant. I am a child. I am not pregnant! Now, my friends have left me; they walk away. I run. I run far, but then I stop. I am breathing harder than I ever have. My tiny legs do not take me as far as my aching heart and trampled mind have the desire to go. I feel like a baby bunny in a field of big bulls. The bulls are chasing me. I will die, not because I am small, but because they are huge, mean, and hungry. Surely I will die. I will wait until I get home before I cry. I am a big girl. I am strong. I have dreams of flying away, like Peter Pan, I want to fly away.
The surgery and its effects caused me to have a horrible time fitting in at school. I went through a lot of changes in my schooling. Situations similar to what happened in third grade repeated through elementary school and middle school. When it was time to go to high school, I decided to go to a large public high school with magnet programs in arts and sciences. I was a chubby little girl, but when I got to high school, I went out for cross-country, lost every ounce of “baby fat,” and through pure determination and diligence transformed myself into a dedicated athlete, lettered in three varsity sports and ultimately became a marathon runner.

**** Running ****

Why do I run? When I started running, I could barely run a lap on the track. I struggled to run a 5k. I pushed to run a 10k. I went for a run, continued for nine miles, so I decided to run a half marathon. I didn’t think I would finish, but I never gave up and each time that I ran farther, it made me hungry for more. I fell in love with running and I had to prove to myself that I could go farther. Some friends and even my mom were worried about me finishing a marathon, so I had to prove something to them.
When I got to high school, I became friends with Rachel in my English class who was deaf. She had a cochlear implant, spoke well, and did not use sign language. Most of the students knew this, but did not view her differently. She knew that I was deaf in one ear, so we had something in common.

**** “You Will Be Deaf!” ****

Nine years have passed; it is fall 2005. “I’m a senior! Class of ’06!” I’ve been waiting for this all of my life! I’m excited that I will go to college next year. Finally I will get to fly away. I am sad because this is my last season running on the high school cross-country team.
It is October 20, 2005. I stand in a long line at Hartsfield-Jackson Atlanta International Airport, and soon we are soaring to the sky. I am flying with my parents because I have doctor appointments in Boston, even though I would much rather be in Senior English, AP-Art, and even my awful Algebra III classes today. Boston is chilly with strong winds. We get a taxi from the airport and find where to go. At Harvard Medical Center, upstairs and down the hall, a large sign reads: “Massachusetts General Hospital - Neurofibromatosis - NF2 Clinic”. I check in at the desk and then sit down to wait. There are a few other chairs and an old lady knitting a blue blanket. I feel a little queasy. “Ann-ee Shi-t-gii-ley” (stupid people always say my name wrong), the nurse bellows to me from the door. She leads me to a room around the corner and tells me to wait. The doctor comes in. After looking over my files and MRI scans, she looks straight at me and says: “You might, well probably will, become deaf at some point in your life.” I am shocked. I can understand her perfectly. I respond, “Yeah, maybe when I’m 80!”
The doctor’s old golden retriever, a retired rescue dog, is in the office. Like a little child, I walk over and pet Madison’s soft coat, as if she can make my awful reality disappear. I am feeling sick, confused, mad, and betrayed. I have been punched in the guts. I think the doctor is crazy. Somehow, I have the knowledge to realize that even a friendly dog, cookies, or ice cream can’t ever take away the truth. My parents and I catch another taxi to Logan International Airport, and board a plane back to Atlanta. I am sitting in my seat on the plane and the engine is loud. I am really upset, and I don’t want to make a scene in front of all those strangers, so I write notes back and forth to my dad, who is sitting across the aisle from me, on my right. My mom’s tired head leans onto his left shoulder as she drifts off to sleep. I remember the conversation:
Me: I hate you! … My life sucks! … That doctor is crazy and wrong! … How would you feel if your doctor told you that? I’m not going to be deaf!
Dad: I’m sorry you feel that way. I understand that you are upset. I am too. I think that you will be okay. They could fix it with stem-cell research, if that happens in ten years.
Me: I hate you! … You knew this would happen! Why did you never tell me? You knew I’d become deaf! She said I’m going to be deaf! What the fuck? When?
Dad: Watch your language, kiddo. I knew that it was a possibility, but we didn’t talk about it because we didn’t want it to hold you back from accomplishing your dreams. I don’t know if that will happen or when. Maybe it will be years from now, after college, when you have a family, or when you are an old lady. You might want to go to the bathroom to wipe your tears and try to calm down.
Me: What if you were deaf? I think you would lose your job! How would you feel?
Dad: I would be very sad. …
Me: I still hate you! …
I was angry at the doctor, my family, even at God. I didn’t know what to do, so I left my seat to go sit in the back. In the plane’s tiny bathroom, I cried my eyes out, like streams go into rivers, forming the flow the creation of ponds and oceans. I vomited into the airplane toilet. My heart hurt deep inside.

**** Tears. Blood. Tumors. Senior Year. ****

I go back to school on Monday, still thinking about what the doctor told me. I try to keep my thoughts and emotions hidden as deep as I can. I usually wait until I get home from school to cry because I don’t want my friends to see. I have a hard time focusing on my classes.
Today is a mild day in late October. I can’t hold it back at my cross-country meet. I have just run as hard as I can in the Boling Park Invitational, but I have fallen twice and finish three minutes slower than last year. I am upset, not about the race, but about my life. I gulp down a little paper cup of Lemon-Lime Gatorade. I do not know that I have four stress fractures on both legs, but I feel the agony and anguishing pain of the grinding bone, along with strained muscles. I feel a pat on the shoulder; “good run,” my coach says. Blood streams from my knee down to my muddy sock, my face is red as a tomato with a small tear that rolls down my cheek. I limp across the damp lush green grass, a little into the edge of the dusky woods, just yards from my team, find a stump, sit down and cry. My shins hurt and my back aches, as if a thousand needles have stabbed me. I use ibuprofen because it helps a little with pain, but I am hurt; my heart is bleeding. I feel as though nobody can possibly understand what I am going through. Do people even know about NF2? Do they know about benign tumors on the spinal cord and auditory nerves? Do they know that I am hurt?
Two months later, the doctors gave me a steroid medication in hopes of saving my hearing. I returned to Boston on the second day of December. I had surgery two days later to remove the three tumors on my spinal cord that had been altering my running gait, causing a lot of physical and emotional pain during the last cross-country season. Thank God, that was successful and I could still run! As soon as I got discharged to leave, rather than going home, I was on a plane to California for another surgery. Two days later, the surgeon operated to decompress the auditory neuroma tumor of the eighth cranial nerve on the left side, which was caused by my rare genetic disorder. That surgery did not help. On Christmas Eve I had another one. I spent Christmas in a hospital room in Los Angeles where I could see the famous Hollywood sign from my window. My dad gave me a small white iPod wrapped in shiny gift-wrap; it was quickly exchanged for a portable DVD player and two movies: Forrest Gump and Four Minutes.
The New Year came, 2006. I was in a complete state of denial, because I was not ready to accept such a sudden and drastic change to my life. I had viewed myself as hearing, all of my life. I didn’t see that there could be anything in between. Just hearing and deaf. I could still hear a lot of sounds at that point, so I knew that meant I was not deaf. I figured I was hearing, even though I could not understand speech. I probably had tinnitus, which is a roaring sound in the ears. Suddenly, all communication had to be written. It was a strange transitional time for me. The only required course remaining for high school graduation was a quarter economics class, which I took with the help of a captionist who sat next to me with a laptop computer. I didn’t know anything about deaf people, culture, or sign language. So I began taking an American Sign Language class at a community college while I was still in high school, but I really was not emotionally ready for that. I went to deaf gatherings and introduced myself at a meeting of the Association of Late Deafened Adults at the Jimmy Carter Presidential Center. I felt like God was punishing me.

**** “Can You Read My Lips?” ****

Reality begins to sink in. I spend a lot more time alone now. I don’t know how to communicate with people that I love, so I pretty much take our dog Daisy running, hang out with the dog, and even talk to the dog. There are no communication problems between me and my dog; she loves me and I love her. If people do try to talk to me, it goes something like this:
Person 1: eifef ndjf ferf jkf geraf rfer pust dosesee fssdu sfed fhc? (cannot understand)
Me: Hi. How are you? Wait, what? (Tries to read lips, but the speech is too fast.)
Person 1: nefirbind. Arrr yru detvf orr sobethieg?
Me: Um…
Person 1: (turns away to talk to another person, and then looks back at me, laughing…)
Person 2: (slowly shouting) CCAANNN YOOUUU (points at me) RRREEEAADD MMMYY LLLIIIPPPSS??? (Person points at mouth, laughing, and looking at the other person.)
Me: *** I don’t know what to do, so I leave. ***

Don't laugh at me
Don't call me names
Don't get your pleasure from my pain
In God's eyes we're all the same
Someday we'll all have perfect wings
Don't laugh at me

- Mark Wills

**** Maryville College ****

It is the middle of August and I’m packing my things to leave home and go to college. I have been accepted at my first choice, Maryville College, a small Presbyterian college located in the foothills of the Smoky Mountains in eastern Tennessee. A month ago, I got a letter in the mail: “Congratulations! You have been selected for the Church and Leadership Scholarship!” Mom jumped up and down, she was so excited. The school offers sign language classes, so I will take that. Before I lost my hearing, my parents thought Maryville would be a good choice for me since they knew there was a chance I would become deaf at some point in my life, but hoped medicine would advance before that happened. They never imagined I’d be deaf before I finished high school. They thought I could go there and learn sign language while in a normal college setting, just in case I ever did become deaf. I haven’t even started college yet and my ability to understand speech is already lost. I do not understand why God chooses this fate for me, but I pray for a miracle that He can make my hearing come back. At least, I’ll get to run cross-country again! I will enjoy my time at Maryville, although communications will often be bitterly frustrating. I see friends laugh, but what is so funny? What? “Never mind”, they say.
They are my friends, but they don’t know that they hurt me. “Never mind”, “It doesn’t matter.”, “I’ll tell you later,” is so frustrating. I want to know! They talk too fast for me to understand anything they say, but just a few months ago, I could understand that fine. I’m having dangerous thoughts, but I don’t have the courage to attempt anything serious. What is happening to me? Who am I? What have I become?
Maryville would have been an ideal small liberal arts college for me if I were still hearing. I took the first class in ASL, but no one wanted to practice out of class, so I didn’t progress very fast. I made a lot of friends even though communication was difficult, volunteered at a small church, and ran on the NCAA-Division III Women’s Cross Country Team.

**** “Suck It Up!” ****

By a year later, 2007, I realized that I really am deaf. On January 29^th, I received a letter via snail mail, informing me that I needed to have surgery again because that same tumor had grown more. I knew that when I woke up, I would hear no sounds, but I didn’t know what total silence would be like. At the same time that I had that tumor removed, I also got an Auditory Brainstem Implant (ABI). It is similar in concept to a cochlear implant, but works differently. It has fewer sounds, and is made for people with detached auditory nerves. I was scared but I sucked it up, just like my cross-country coach always had told me to do, when I ran hill repeats or speed intervals. No pain, no gain. “Ya aint gonna get nowhere in life, if ya don’t learn to suck it up.” Complain about heat, cold, injury, or fatigue and coach yelled “suck it up!” My doctors said that it had to be done, or I could die from the tumor’s pressure onto my brainstem, so I bit my tongue and sucked it up.
It was a tough decision but I decided not to return to Maryville. I felt like my deafness and the people around me formed a tall brick wall and I was a little bird with clipped wings. There is a world of opportunities on the other side of the wall, where I was before my wings were clipped and the wall was built. I wanted to move on in life not dwell on my loss. I researched and found out about RIT/NTID and the large deaf population in Rochester. I talked to a few students that I found through friends in a Facebook group, and they were helpful. Having lived all my life in the deep South, wearing flip flops to school most of the winter and seldom seeing a snowflake, I imagined freezing to death in New York. I talked to my parents about it, and we all came to visit during my spring break. I saw hands flying everywhere and only understood a little of it, but for some reason it seemed right. I met up with some friends I had met though a Facebook group, more cool people, and visited the deaf Bible study which was amazing.

**** 26.2 Miles ****

After visiting RIT, I returned to Atlanta to run the ING Georgia Marathon, my first full marathon, with the Children’s Tumor Foundation NF Marathon Team. Five girls from my Bible study group at Maryville drove down from Tennessee to cheer me along the route and joined my family for lunch afterward. A photo shows my fans all in bright yellow t-shirts and mom waving her big South Carolina flag; I stand smiling, sweaty, tired, wearing my big marathon medal around my neck. I felt great accomplishment and happy that I finished. I felt sore, tired, and wondered how I would make it up four flights of stairs to my writing class the next morning. I returned to Maryville for just a couple of days after the marathon to pack up my stuff and move out of the dormitory before flying across the country.

**** Eating Jell-o In Silence ****

Today is April 2, 2007. I am on a plane flying to Los Angeles, California. My parents, Ken, and his girlfriend Nicole are with me. Tonight we will go to Santa Monica Pier, hang out, go run and get dinner. Tomorrow morning, I will check in to House Ear Institute / St. Vincent’s Hospital, because I have to get a lot of tests, EKG, blood work, even another damn hearing test, and have surgery the day after that. I wish I could fly away.
Two days later, the day I dread is here. I glance at my Timex watch; it reads “5:02AM. – APR. 4.” I ran my first marathon just ten days ago, but now I am told I will not be allowed to run until the middle of May. I’m thirsty, but I am not allowed to eat or drink anything now. I’m scared, but I act mature and courageous.
“It is another surgery, just another damn tumor,” I think to myself. I am rushed early in the morning, as I hear the sounds around me. The sounds sink in. I remember how things sounded before last year, and I know how they sound now. My parents and I go sit in the waiting room. A nurse walks up to me and directs me to a desk. She holds a stack of papers, and then points to where I need to sign. I sign my full name on the line below the long paper, which I don’t bother to read. I already know that it is about the surgery and death warnings. She straps a plastic bracelet with my name and numbers printed on it, while she continues to talk to my parents. I sit, fumble, and observe my surroundings. There are chairs, people, and that strange scent of blood, cleaning spray, and bactericide soaps. Dad writes notes to me; he tells me to not be scared. Soon I am lying on a stretcher, wearing another hospital gown and the weird no-slip socks. I am still scared as I grasp my mom’s hand for comfort; I try to remain calm. She lets go, after the surgeon shaves half of my hair off. He gives me a shot, and I fall asleep.
Many hours later, I wake up. I am groggy with drugs and wired to machines. My head hurts; a white gauzy bandage that looks like a large turban is wrapped really tight around my head. I hear sounds, but I soon realize those sounds are not real. The sound is silence; now I know what silence sounds like. Silence sounds like static, but sounds can be felt by vibrations. I have been told that silence is golden, calm, peaceful, a sanctuary, holy. Wrong! Silence is dark, empty, mute, speechless, scary, sulky, and black like death.
Tomorrow I will move from ICU to my own room and eat real hospital food, not just urine-colored lemon-flavored sugar-free Jell-O. I hate Jell-O! I continue to improve. I am mad when I see the signs outside my door; one says “FALL PRECAUTIONS” and the other “HEARING IMPAIRED.” I get a huge pile of get well cards! I am so drugged that I manage to start speed-walking laps around the nurse’s station two days before the rehab person brings a walker to my door, which I refuse to use. I am a marathon runner, so I will not touch that walker; I am deaf, but I am strong. I am independent, always independent, just like the “Miss Independent” award I got when I was in pre-school.
Two weeks later, I was back at home in Atlanta. I slept a lot, ate a lot, felt sad, and wondered, “Why am I deaf?” Sometimes I slept all day, and then went downstairs, where I slept more, watched television, text messaged, and daydreamed – all day. As soon as I was allowed to drive again, I went to visit my friends at Maryville College. They ran over and hugged me so tight, it was like they wanted to break my ribs! I was very happy to see them. Some asked me silly questions like, “Can you hear?” I wanted to fly away.
I became eager to run, but I was told to wait. I took the dog for walks, and by the next week, the dog walks had greatly increased in length. I was surrounded by sounds, but all I could hear was silence. When I saw people talk, there was only the sound of silence. This was my life, a silenced marathon.
It was the beginning of May 2007, and my long walks became walking, then jogging. It did not hurt; it felt good, so I kept going. Within a few days, I got in trouble, because I was not allowed to be running yet. My dad looked angry as he quickly scribbled notes to me.
Dad: Were you running?
Me: (shakes head, no)
Dad: Jogging? Why is your shirt wet?
Me: It is hot outside.
Dad: If you run, before your doctor says you can, you could displace the electrodes in your implant. You MUST let it heal! If you ruin it, they cannot fix it! You will NEVER hear anything! You may have already caused a LOT of damage! Do you understand?
I was angry. I probably slammed the door, a silent slam to me. I went to the bathroom. I cried, silently. I knew I jogged a little but I didn’t know it could harm the ABI. I wondered why he had not told me that. Inside of me, I was scared. I was told I had ruined my expensive implant. I waited two weeks before I was allowed to run; I waited two months before I knew if jogging a few miles had ruined my ABI. I wanted to fly away.

**** New Sounds ****

Two months have trickled by. Today is June 6^th, the day after my 20^th birthday. I flew back to California yesterday, and now I am sitting in a waiting room with my mom. We are eager to see if and how my implant will work. Little butterflies flutter in my stomach. I am nervous, scared, maybe excited. The audiologist finds me and leads me to a small room with three chairs and an old PC computer. He shaves a one inch circle on my head, just behind the ear, and attaches a small sticker that has a metal disc in it. I get hooked up. How strange to have a plastic thing hooked over my ear and a small disc on my head with another wire coming down from that to the processor. I sit and wait as the audiologist presses buttons; suddenly I hear “BEEP!” I have never been so eager in all of my life to listen for a little “beep.” Whoa, that is loud! Loud, quiet, high pitch, low pitch, all of the sounds are different, all ten of them. I hear my audiologist and my mom speak, but I cannot comprehend the sounds.
Audiologist: 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, baseball, hotdog, airplane.
Mom: Hi, sweetie. Can you hear me?
Me: Um, you sound like a…. a…. a…. dying cow and a sick frog!
Mom laughs; she looks a bit horrified, but the audiologist explains that it will take time for me to get used to it. I immediately think that I will not use it, because I cannot comprehend any of these sounds.
It has been a week since I got my implant turned on. I will now go to be a counselor at my church’s summer camp for high school students. This is my sixth year of camp, second year being a counselor. I fear communication problems, but I know I will have fun.
From my camp journal:
June 16, 2007
Dear God,
Sometimes I want to think that I am strong, but really I know that you are strong and I am weak. Please Lord; help me to hear your voice calling to me, and to recognize it, even though communication with others can be difficult. I got this new ABI turned on just a little over a week ago, because now I am deaf. Maybe I need your help with accepting that reality? Or have I already? I am not sure. Anyways, when I turn on my ABI, I can hear sounds, and maybe I will be able to understand more than in the silence. Perhaps I can better hear your words without it? I want to hear my friends, too. If many are talking, the sounds are much more confusing. Where should I go in life? Please listen and call to me; help me to hear you! Allow me courage to fly.
- Me
My prayer partner, Juliana, and other awesome friends patiently repeated things, wrote notes, and learned to fingerspell, which meant a lot to me. They went that extra mile to write notes like they would for classes, for a counselor meeting, evening speaker’s story, or which song we were on. They wanted to make sure I didn’t miss anything; they helped a lot. I decided to give the ABI a chance, even though some people seemed to think it was magic hearing plastic.

Dance as though no one is watching you.

Love as though you have never been hurt.

Sing as though no one can hear you.

Live as though heaven is on earth.

- Souza

**** Epilogue****

**** Acceptance And Flying At RIT / NTID ****

I decided to transfer to RIT. Since coming here in the fall, I have made a lot of friends, improved my signing, as well as lip-reading / listening with ABI, and learned more than I had ever imagined. My time here thus far has helped me to change for the better and has helped me begin to accept myself for who I am. I still run, perhaps I run to escape the world or for the thrill. If you wonder what I am running from, it’s nothing, really. Now, I am not stuck in a shell. I believe in myself. I believe I can really fly...

I believe I can fly

I believe I can touch the sky
I think about it every night and day
Spread my wings and fly away
I believe I can soar
I see me running through that open door
I believe I can fly
I believe I can fly
I believe I can fly

- R. Kelly

NF: A Life Sentence

Pet therapy 2 days after my NF surgery

Hi everybody! My name is Jen and I have NF1. My mom and brother also have NF1.  I never thought anything of it when I was told I had NF.  Other than 'spots' and outer tumors, etc, I thought that was all NF was about. I never had pain or complications, etc. Then in 8th grade (around 1996) I came down with migraines as well as  the most terrible facial pain. I can't describe it. The pain would then extend to my neck. I woke up one morning with my neck all twisted, and I had to stay home from school for a few days. The facial pain got worse so I thought it was my wisdom teeth. Had an x-ray and from there he saw something strange (and it had nothing to do with my wisdom teeth) so I had 2 more x-rays, a CT scan and an MRI I guess to really make sure what they saw. The revelation: a tumor in my skull, and an optical glioma. Had a follow up MRI a year later, and nothing changed, so I didn't see anymore doctors for at least 10 years (when I moved to Boston and learned about the NF clinic at MGH). I actually didn't know the extent to my facial tumor until I moved to Boston. Imagine a flat jellyfish and that's what the tumor in my skull looks like. It wraps around my sinus cavity, my nose bone, my upper jaw (I have a lump in my jaw that dentists keep thinking it's some gum disorder), around my temple (part of my skill is actually lifted at my temple) and somewhat behind my ear. so that explained the facial pain and frequent migraines! When I was first given the MRI, my doctors were not very clear about where the tumor was, how big it was, etc. So I am REALLY glad I am in Boston, and that my doctor is cool enough to show me pictures. Dr. Scott Plotkin is a good guy. :)

Thanks to NF I am short. I also have mild scoliosis and thanks to my optical glioma, I am partially color blind in my right eye. I wear glasses. When I was in school, of course, people made fun of my height and often made fun of my spots. One day people in my drama class passed a note around with a picture of a face and neck with spots everywhere (though I don't have spots on my face). I knew it was about me. I wonder what kind of pleasure that gave them to make fun of my cafe au lait spots.... silly.

Anyway, my face doesn't hurt as much anymore, and I rarely get migraines, but in addition to my facial pain, I developed awful lower abdomen pains for years that was worse than my facial pain. When the pain got worse, I thought it was a gynecological issue. Had ultrasounds, etc, nothing. Then I thought it was a  gastrointestinal issue. So I had an upper GI, endoscopy, colonoscopy and guess what? They randomly found cancer in my stomach- totally unrelated to my pain in my abdomen! This was in 2009. I am still dealing with repercussions from THAT surgery (mainly esophageal problems).

My NF survival wound. You can sort of see my scar
from my cancer surgery, which extends from my belly button
to my sternum. They recut a good portion of my old scar!

 During the summer of 2010, my abdominal pain became excruciating. I had my gastroenterologist run many more MRIs, etc, and turns out I had an NF tumor where I had pain. I didn't find out about this until February 2011. I then couldn't get a hold of my NF doctor until this past JULY to talk to him about dealing with it. Had a biopsy to make sure it wasn't a sarcoma (needle biopsies are terrible by the way! I felt EVERYTHING). The only thing that could be done for me (since pain meds did nothing even oxycodone for a <100 lb person did nothing!) was surgery. So on Aug 23 I had my first NF surgery.  Of course now I have some sensory nerve damage (that's slowly going away). I take 2400 mg of gabapentin a day and that helps tons! I recommend that for any tingling types of pain you might have! And visit a pain clinic. I see a pain psychologist and she is AWESOME. My primary care doctor is also awesome, gotta add that in there too :)

I still have pains in my left and right side. Before my surgery, my surgeon told me that my right side pain was just referred pain, and that it would go away after my surgery. Well, it hasn't. It took 7 years for doctors to finally find the answer for my left side pain, let's see how many years they will take to find something on my right side.

I additionally worry about new tumors growing. I never knew tumors can just randomly GROW with NF, but they do. That scares me. What if more grow back? What if I become in pain again? I seriously could not function from 2010-now. I don't know how I survived my first semester of grad school but I did. What keeps me going is that I know there are people who go through worse.

I call NF a life sentence because those with it have to ENDURE IT. They have to worry about tumors growing randomly, tumors pressing on nerves, surgeries, pain meds, etc. It is baffling to me how NF is so misunderstood, yet it is one of the most common genetic disorders. I am not downplaying other genetic disorders, but NF deserves more attention. Some people have no problems with their condition, but many DO, and when people DO, their problems are awful. And there's usually nothing that can be done about it.  Some people go deaf, some people become paralyzed, some people have disfigured faces, some people can't function because they are in so much pain (back pain, stomach pain, leg pain, etc). I really love how there have been more walks, more races and more fundraisers about NF.

This is Bob :) I have named
all of my tumors

There are several NF non-profit centers around the country that help put on races, etc, so you should check it out. Here is the Facebook link for NF Northeast. This is for Michigan This is for Arizona This is for California, and this is for Louisiana. ... that's about all I can find on Facebook!

I have a personal blog which chronicles my personal battles (and all the drama that occurs between me and doctors). Feel free to follow me and if you have a blog let me know so I can follow you!

Thank you for reading my story.

This Girl...

This Girl...
So there I was, at the end of my second quarter of college, about to go on spring break with Campus Crusade for Christ (Now known as Cru) Spring quarter registration had come and gone, and I had signed up for a class in American Sign Language, (ASL) because of the large deaf population on campus.

I go to meet up with the group, to leave for the airport, and I see everyone else has two carry-on bags, while I only have one. It had been a bit since I flew. So I asked the first person I saw who seemed outgoing, if we could have two, and she said yes, then proceeded to show me how to properly compress them by jumping on them. I went and grabbed another bag.
By the time I got back, we were just leaving for the airport. I wanted to thank this girl for telling me that, but it seemed like she didn't hear me. One of the other students going on the trip got her attention, and I noticed a small flesh-coloured piece of plastic behind one of her ears. This was odd, because she spoke well for someone wearing an implant. I didn't think much of it at the time.
We got to the airport early, and went through security without any problems. As we were supposed to, we had arrived early, so someone broke out a deck of cards and we started to play. That's when I noticed that this girl really was hard of hearing. Except, apparently, to me. She seemed to understand me just fine, while she'd struggle with other people.

We played cards for the hour or so before our flight, then boarded the plane, and due to our flight schedule, I didn't see this girl again until we landed in Florida. We were waiting for some checked baggage at the carousel, and this girl saw the sign that said "Don't climb on the carousel. " She promptly went and climbed on and pretended to be getting eaten by the baggage gate. She hopped off just before the baggage started to come out, and then we went to our separate cars for the three-hour journey to the hotel.
It was close to 1 or 2am by the time we got there, so we all checked in and went right to bed. The next morning, the whole group met up to go to the morning session, and this girl was there. I started talking to the interpreters, one of whom was my roommate for the week, and told them that I was going to be starting an ASL course in the spring. This girl was following the conversation and immediately offered to help me learn sign, something she had only done recently. I accepted.
As the week progressed, I started hanging out with this girl more and more. I found out that she was late-deafened at the age of 19 (Which I thought was weird because she couldn't be more than 18.) due to some genetic disorder called "neu-ro-fi-bro-ma-to-sis, typetwo." (Which I would learn more about later) This kinda struck me because I had only just celebrated my 19th birthday, and tried to think of how my life would be different without sound. I found out later that week that she was actually 21.

Thursday night, there was a speaker who gave a really good message on being a warrior for God. He talked about how we had to completely surrender everything to Him in order for us to fulfill our calling. I did, and that included my then-girlfriend, and my major in college. After the session, I was looking around at all the audio and video stuff that was going on, and thought, "Boy, I think I could do something with this if I switch to telecommunications engineering technology. I don't really like all the coding I have to do for computer anyway."

We came back after a week in Panama City Beach, and I had really formed a relationship with this girl. While there were two interpreters on the trip, there were also several deaf students who used ASL as their primary communication. This girl wanted to use voice, and practice hearing with her "super-ear." She could also understand my voice very well, for reasons I didn't know at that point. So instead of an interpreter, I spent the whole week with her repeating things that needed to be said or just talking, while she'd teach me signs and fingerspelling.
I also found out in those first few weeks that this girl had a weird obsession with this thing called "running." I had participated in indoor track in high school, but I was a shot-putter. My running was minimal. In soccer, I played goalie. In lacrosse, I sat the bench. My sport was swimming, and I had lost it my senior year of high school to chlorine-induced asthma. I thought maybe someday I'd do a triathlon, because I could still open-water swim just fine, and I rode my bike everywhere. It was just that third part, that "running" that confused me.
During the spring quarter, this girl took me to No Voice Zone, where I continued to learn and practice ASL. Through that, I found out about a weekend event between RIT and Galladuet, and a party that was hosted at the end of it. I went, and made some chainmail bracelets, because I was bored. I met this one guy there who was quite interested in the chainmail, and bought a few of the ones I had.

Shortly after, spring quarter ended, and the summer started. This girl drove all the way to Colorado by herself, and posted lots of pictures on facebook, of which I was very jealous. During the summer break, I ended up breaking up with my then-girlfriend, because I knew I had to, since God had told me to 3 months prior. A month and a half later, she was nearly killed in a car accident and through that we became close friends and remain so to this day.
While that was happening, this girl that I had met on spring break was still in Colorado, and I was working at a camp in Rome, NY, as a lifeguard. The rest of the summer flew by, and before I knew it I was back in school in September. When I got back, this girl invited me to go to running club with her. What's running club? I mean, do people actually enjoy running together? Apparently they did, and soon I was racing nearly every weekend at some 5k or other race. My first race was a XC fundraising run at a local high school. I won my age group by default, I think. This girl, and another one of our friends from running club, also did.

As fall quarter progressed, I started learning more about this "NF2" and all the problems it had caused for this girl, and I found out about this endurance-racing team that supports research that she was on. I learned about what the tumors can do to someone, I learned a little bit about what it's like to live with, and I learned that no matter what life throws at you, it's always your choice to stay beaten down, or get back up.

One day, I encountered the guy who had been so interested in the chainmail while at a deaf event with this girl. The next day, this girl and I were part of a 6-pack of Coor's Light that ran a 5k for Halloween. After I got back that night, I got a text from the guy I had met saying he thought it was so cute to see me with my girlfriend. I thought it was funny, since we weren't "officially" "dating" at that point, so I texted this girl. Shortly after I sent that text, I had a relationship request on facebook, which I guess technically makes it "official."

Throughout fall and into winter, I found out that this girl really loved to do marathons and distance running. Again, I found it weird, since I equate distance running with pain and suffering. (Still do, actually.) But before I knew it, I was running races during the winter. And the races were getting longer, first 4 miles, then 5, and then somehow on my 20th birthday, this girl suckered me into running 8 miles.

It was now spring break of my second year at school. This time, I went to visit her parents down in Atlanta, and ended up doing a 17-mile trail run all over a mountain on that break. Again, I'm not sure how that happened. All I know is I sprained my ankle.

We were only able to stay half of break week in Atlanta, so we came back to NY to visit my parents. And got into a minor car accident on the way home. That's a great way to have your girlfriend meet your parents: "Hi mom and dad, this girl is coming to visit and we got into an accident." No injuries, though. Just a fair bit of damage to my van. Not three days later, we decided to go for a snowshoe run at a local park and found ourselves at the open event for snowshoe nationals. We ran it, and rather enjoyed it, since it was a completely different experience to winter road racing.
Spring quarter came and went, a few more races here and there, I almost got pulled into running a half marathon on Long Island, but I managed to avoid that somehow. This girl and I decided to work at the same camp that summer, I would teach hockey, she would teach arts and crafts.
But a few weeks before we were to start that job, this girl decided to fall out of her bed and wind up in the hospital for 4 days during finals week. I was there as much as I could be, and I made her food and made sure she felt OK. It was at this point that her mother said to me "You're acting like a married couple right now." And I realized that was true, and I started praying about that idea.

After the bed stunt, we went to work at the camp. That had it's ups and downs, but for the most part, we enjoyed it. We had the same days and evenings off, and spent most of our free time together. After that summer, I decided to try doing an olympic distance triathlon on my mountain bike, since I was now able to run 10k at once. I chose an inaugural event at the same lake where I had worked the previous summer, while this girl was in Colorado.
I immediately got hooked on triathlons, for some reason that still escapes me. I then found out about a half-ironman in Syracuse that went, almost literally, right by my house. I said I wanted to do it next year. This girl said she'd do it too, even though she had no balance nerves, had a large tumor in her leg, wasn't supposed to open-water swim, and wasn't supposed to be able to ride a bike. I said "fine, whatever," half thinking that she wouldn't do it.
During that winter, I kinda fell out of the running that I had been involved in the previous year. I had started curling more actively, and most of my tournaments conflicted with winter races. This girl kept doing subzero winter races, despite growing up in Atlanta, Georgia. This spring break, she went back home, and I visited my grandmother in Florida.
Spring of my third year. (2011) I got a co-op back home for spring and summer, while this girl, who changed her major from interior design to multidisciplinary studies, took a few classes. During the co-op, one night while I was out of town in a motel, I called this girl's dad and asked for his blessing, as I was planning on proposing to her relatively soon.
Every weekend for two quarters, either she would come and visit me in Syracuse, or I would drive to Rochester to visit her. One of these weekends, I drove to Rochester and we did a trail marathon, even though I had told her I'd never do one. Another weekend, I drove to Rochester and we were going to do our first triathlon together. However, the swim got canceled, so it was a duathlon. Her real first triathlon would be the next weekend.
July 31st, 2011. It was 5 am, when we woke up to go to the same triathlon I had done a year before. It was at a lake where I had spent part of my summer every year for my whole life. It was the lake where I worked as a lifeguard for 6 years. It was the lake where I had done my first triathlon in 2010. It was the lake where she would do her first triathlon in 2011. My little sister came to watch and take pictures of us "racing."
I finished the tri in two hours, 38 minutes. My sister had been holding a small wooden box the whole time. In it was a ring. A ring meant for this girl. 26 minutes after I finished, I saw her running toward the finish line. I stood in the finish area with my hands behind my back. When she crossed the line, I gave her a hug, and said: "I have something to ask you..." To which she replied "You're supposed to be on one knee!" So I dropped to one knee, pulled out the ring box, and asked her if she'd marry me.
She said yes.
And then this girl told me that I was supposed to put the ring on her finger. I did. A few minutes later, the race director announced our finish-line engagement. We made (almost) all the compulsory phone calls, to share with our family, and then posted it on facebook.
Since it was the end of the summer, I was done with my co-op, she was almost done with her classes, I went for a little road trip to New Hampshire to visit some friends and family while she went back home to show her new hardware to her family. During my visit to NH, I did my first half-ironman triathlon, and enjoyed it immensely, other than the fact that I felt sick for the whole run.
A month later, this girl and I did our first race as an engaged couple. It was a half ironman. In Syracuse. My second, her first. We both finished, with both our families cheering us on. It was a wonderful feeling.

This girl is Anne Shigley. No, you can't have her, no matter how awesome you think she is after reading this. Anne's going to be mine (and God's) until one of us dies. How did I get so lucky? Anne, I love you. I never would have been able to race an Ironman without you nagging me to run, I never would have done a trail marathon, or a triathlon. We've had our ups and downs, but I'm always amazed by your drive and motivation, and that's one of the reasons I love you. I never would have become as proficient at ASL as I am if you hadn't shown me NVZ. And I may very well have ended up just another computer-game playing, single, nerdy, RIT student if it wasn't for you. NF sucks. It's cost you a lot that you valued. But if it wasn't for NF, I wouldn't have met you. I would have a different outlook on life.  And I love you just the way you are.  You're beautiful, loving, and compassionate, if a bit stubborn at times. (Although the tumors can go take a hike, I don't like them.) How does a southern-runner girl get engaged to an "eskimo-hockey-playing-canadian-boy?" I'll never know, but what I do know is that it's a match made in heaven. <3

Why you should NOT take up running....

My roommate read this and told me I needed to be careful because this might scare some folks, so here is your warning: 
IF YOU ARE A "NEWBIE" RUNNER...
DO NOT READ THIS!!!! 








I got the basic idea to write this when I was out for an 18-mile solo training run, on Monday afternoon. I jotted notes on paper and kept having trouble with formatting it, so its not perfect, but here it is...

     Shin splints
     Operations
     Muddy, very
     Energy draining
     Tripping and falling
      Illiptical Band Syndrome
     Metatarsals inflammation
   dEhydration
     Stress fractures

     Reptured bloody heels
     Undies chafing
   aNkle sprains
   teNdinitis of Achilles
     Intervals
  deNtal repairs
     Giant blisters

     Snot "rockets"
heaT poisoning
  fatIgue
     Nails on toes turned black
     Knee soreness
     Side stitches


    Sometimes running is no fun! 
   ...but then I remember why I do what I do! 
(other stuff from my lists that didn't fit well in there: calf strains, fevers, tumors, stomach aches, barfing, hallucinations, business in woods, frozen sweat... By the way, I carefully ONLY selected things that I have experienced since I began running, 9 years ago!)

Happy running! :)

My Experience with NF2

My name is Bonnie, I'm 24 years old, and I was diagnosed with NF2 when I was twelve.

I was in the midst of puberty, that awful, confusing period where things just start happening to your body and you have no idea how to stop it or control it. Puberty hit me pretty hard. My hair was frizzy and I needed braces and I just had that feeling and look of awkwardness. While the other girls in my grade all seemed to be perfect, with shiny hair and straight smiles, I was the freak with Sideshow Bob hair.

As if puberty isn't bad enough, not long after I got my first period, I started experiencing discomfort, twinges mostly, in my right thigh. We were on vacation at the time and I mostly ignored them, thinking I had pulled a muscle in gym. Eventually, though, the twinges became less annoying and more debilitating and I told my parents. I was taken to a doctor, where he diagnosed me with scoliosis and prescribed an MRI.

I was terrified. I was twelve years old and my mom patiently explained to me that I would be going in a tube for an hour that was loud and it seemed like the end of the world. To me, my first MRI experience felt like being trapped in a coffin--a white, incredibly LOUD, coffin. The only upside was at least I got to listen to music.

A few weeks later, the doctor called my parents with a diagnosis. I had neurofibromatosis type 2, or NF2, as us cool kids call it. I didn't understand the connotations at first. My parents sat me down and explained but all I heard was a lot of medical jargon that made no sense to my addled preteen mind. I just wanted to watch Buffy the Vampire Slayer, thank you.

It wasn't until a year later that the reality of the situation hit me. I was 13, we were in the pediatrician's office for a checkup, and my dad mentioned I had tumors. In my mind, tumors equaled certain death. I cried all night until I was reassured that they were benign and weren't terminal and as long as they were watched closely, I would be fine.

I had my first NF2-related surgery that year. Previously, my only two experiences with the hospital was when I was 3 and had to get my appendix out and when I was 9 and 30 lb weights fell on my fingers, requiring surgery. So this was a pretty big deal for me. I had to have spinal surgery, as one of the fourteen tumors there was pressing on my nerve, causing pain. I was in the 7th grade and I had to be homeschooled for 3 months. At first, this seemed, well, awesome. I wouldn't have to wake up at 6 am anymore! No more immature classmates throwing stuff at me and making fun of me for being awkward. Success!

After awhile, though, this situation became quite lonely. I was in the hospital for a week recuperating from surgery. My roommate was an 8 year old boy who had just had brain surgery. It was terrifying for me. My mom stayed with me the entire time, sleeping in a hard-backed nylon chair. I was 13 and the idea of staying in the big, bad hospital alone was unfathomable. This was Columbia Presbyterian, a hospital that would come to mean hell in my eyes.

As the years went on, I needed to have a few more surgeries, but the NF was mostly an annoyance. At 14, I had my first brain surgery, which took the hearing from my right ear. THe doctors said it would come back eventually, which obviously never transpired. I would have to miss a lot of classes because I wasn't feeling well, or I had a doctor's appointment in the City, but other than that, life was relatively normal.

It wasn't until after I graduated that things took a turn for the worse. I had spinal surgery right after graduation and, a month later, I came down with meningitis as a direct result, an infection. That took the hearing in my other ear. Suddenly I was 18 and deaf. I couldn't hear my friends when they would call on the phone to check up on me. I couldn't hear the doctors. I couldn't hear music. My entire life was derailed.

While I was at the hospital, there was a lot of screw-ups. For 4 days, the doctors had no idea what was wrong with me so I remained in the ER all that time. When they finally figured it out, I was, among other things, forced to wake up at 2am for a CT scan that, as it turns out, I didn;'t need; the results weren't given to us until n oon the following day, after my dad complained countless times. I had a pick line inserted (like an IV inside the body) and I was told I would be under anasthesia and not to eat all day. I didn't, and no anasthesia. My back kept opening up , leaking spinal fluid, because they told me nhot to lay on anything hard then promptly put me on a cold, HARD, metal examining table.

My parents wanted to sue for malpractice. Our lawyer said we didn't have a case so we just switched hospitals. I now go to NYU Medical Center. My doctor is John Roland and he's been caring for me for the past six years.

I have an ABI, but it doesn't work; it abruptly quit on me 3 weeks after I got it. For 5 years, I pretty much did nothing but laze around the house, crying because a lot of my friends had abandoned me, and I felt very alone. Then came RIT.

I was 23 and a Freshman at RIT/NTID, the National Instutite for the Deaf. Suddenly, I had so many experiences that were lacking in my life before. I made friends. I fell in love (with an awful excuse for a human being but that is neither here nor there). But still, the NF haunted me. In 2008, I had major brain surgery to remove a tumor and, as the brain controls everything, for months I couldn't even walk myself. I had to use a walker, a wheelchair. My hand shook terribly. Now I have mostly recovered but I still have issues with balance. I trip over air, and not in that adorably klutzy Twilightish way either. I can't walk in a straight line, making me appear intoxicated all the time. the vision in my right eye is blurry and the eye is very sensitive. Just the slightest irritation will turn it beat red. It...well, it sucks.

My biggest fear is that I won't find someone to share my life with because of my condition. My last boyfriend said he understood then did everything wrong and couldn't cope. I'm 24 and most of my friends are either engaged, married, and/or parents, and I don't want to miss out on all of those wonderful things because of the NF. Most people have large goals--to be CEO of a company or have riches and fame. All I want...is to be happy.

Thank you...

Thank you to the father who took me to that first cross country practice at 6:30am and who sat in his car, watching me struggle to run half a lap on the track.

Thank you to the mother who has always been my #1 cheerleader, but I know she had some doubt and fear about me finishing my first marathon.

Thank you to the student who once told me that he didn't think I would still be able to ride a bike, due to my loss of balance. That week I bought a cheap new bike.

Thank you to the NTID faculty person who I understood to have had told me that she thought I was too smart for those classes.

Thank you to all of the coaches I've had through six years of cross country and three years of track, but I thank one most of all, my first running coach.

Thank you to the endurance team that has supported me through everything, even though we are too spread apart to actually train together.

Thank you to the doctor who took some junk out of my back, sewed me up, asked how I was feeling and laughed when I said okay and then puked on his shoes.

Thank you to the friend who I met through facebook.com, before I came to RIT, and helped me realize who I am, even if she was blunt or harsh, I love you.

Thank you to the running club that patiently waits for every single person to finish every group training run and every race.

Thank you to the wonderful family who has opened their home to me and a group of amazing young ladies, numerous times, even though I'm always causing trouble.

Thank you to the NF doctor at Harvard who told me that I really should not swim at all, especially in open waters, due to my loss of balance. With caution, I ignored.

Thank you to my long time best friend, who has always been there for me, through the thick and the thin.

Thank you to the teacher who when I brought in a 23-page memoir draft for a 15-page assignment, told me I ought to write book, but I basically need to start over.

Thank you to the random people who have asked me how far I've ever run and when they get an answer, their jaws hit the floor and they called me insane.

Thank you to the doctors who have dealt with me, cut open my head - on numerous occasions, and gave me my ABI, so I can "hear" again.

Thank you to the wonder boy, my fiance, who always makes me feel like the most beautiful woman and loves me no matter what.

Thank you to everyone else who has made any impact on my life.

Thank you to God for making my life wonderful and continuing to do so!

My World Without Sound

I live my life in a kind of isolation, a nothingness. I see things
that aren't meant to be seen. My fifth sense has been snatched
away from me, without warning, without preamble. Growing up
all my life with sound, children laughing, birds singing, waves crashing
along the shoreline, only for it all to be taken away. I am lost,
I grieve as if I have witnessed someone close to me slowly perish. For years, I
had no control, over myself, over my life, over anything. I was simply forced
to watch while everything I knew, everything I'd once taken for granted, was taken away.
It is my world without sound. I "hear" things that aren't really there, memories,
recollections of how it should be, but isn't. As I drive in a car, I see a
neighborhood boy bouncing a basketball. I hear the thump-thump as the
ball smacks against the concrete, but it is not really there. When the dog barks,
I "hear" it, but it is not really there. When a child laughs, so innocent, I "hear" it,
but it is not really there. My world without sound. I would do anything, give anything
to hear the waves crash along the shore again.

I Would Still Run

If I found out tomorrow that my son was misdiagnosed--that there was another explanation-- I would still run. I know because of the people I have met. I see the look in the eyes of mothers whose children suffer from NF, and I think, "I must look like that to other people." Until there is an answer to all the questions. I will run.

For my daughter, Shannon...

For my daughter Shannon, who was diagnosed with NF2 in 2002.

I remember leaving home the day you were born. I thought, things will never be the same, and they weren’t.

What love, hope, inspiration and happiness you have brought into my life. I would walk into your nursery and you were always happy, smiling and reaching up to grab life with all the joys it can bring. That love of life and your ability to see good in all things around you has helped you and especially me deal with NF2.

Even in the face of adversity and illness you move forward with courage. You always find the positive side to the ravages NF2 causes to your body.  You have taught me so much and give me the strength to face what lies ahead. 

When NF2 took your hearing and you could no longer sing, you didn’t give up. You used your eyes to photograph all of the beauty you used to sing about.

When NF2 took your sight, you didn’t think of it as an ending but a beginning to teaching and helping others by showing them that no matter what their differences, or handicaps they are to be valued and can be productive members of society. 

Neither of us knows what lies ahead but together we can get through anything NF2 throws our way.  We will always move forward, always look up and embrace what NF2 can never take from us: OUR LOVE.

If I could take your pain away and have the NF2 instead of you I would.  I am always here for you. You are my hero.