Learn about living with Usher syndrome from people with Usher

Rachel's Story

Nearly 18 years ago, I did not understand what sounds meant to me. Every morning for the first few weeks, when I was a rough little three-year old child, my mother had to sit on me in order to get the magnet on my head. I know that sounds cruel of my mother, but I am grateful to her for doing this because she has taught me that it is unimaginable to live in a silent world. Today, I understand that every morning, when I wake up and put my cochlear implants on my ears, I am welcomed to the broad spectrum of opportunities, which include being able to learn a second spoken language, French.

I was born profoundly deaf. Because the most powerful hearing aids did not help me and my parents did not want me to sign, they decided to opt for the cochlear implant. In 1989, I became one of the first 200 children in US to receive a cochlear implant as part of the FDA clinical trials. When my parents and I traveled to New York University, they hoped that I would at least hear traffic noise and other environmental sounds necessary for safety. However, our expectations with my cochlear implant were quickly surpassed. I attended Auditory-Verbal therapy to learn to hear and to speak, and within six months I made tremendous progress and was beginning both to understand what people were saying and to speak.

During my young childhood years, I continued to go to Auditory-Verbal therapy to improve my hearing and speaking skills and my language. I always had to leave school a half hour early to attend sessions once a week. This never bothered me as my parents taught me that attending therapy sessions was going to pay off in the future, and it did as my language is now caught up. I am one of those people who can easily fool others into thinking that I am a normal hearing person. Sometimes when I tell people that I am deaf, they are astounded and refuse to believe me.
In order for me to be part of the hearing world, my parents placed me in mainstream school settings so that not only could I learn to hear, speak, and communicate, but also to be an independent hearing person. Thus, I attended mainstream schools throughout my life where I excelled and received honors. I was also very involved in extracurricular activities throughout my school years, including student council, National Honor Society, Film Club, French Club, and I also had officer positions in some of these activities.

About four years ago, I received an implant for my other ear so that I could hear bilaterally. I am certainly hearing better with two implants, and everything sounds richer and fuller.

I also have a younger sister, Jessica, who was born profoundly deaf. She received her cochlear implant at NYU too so that she could be implanted at the age of 15 months. At that time, she was the youngest child in the country to be implanted. She also received a second implant when she was nine years old. She is now in middle school and is doing beautifully like me. We have a brother who is 17 and has normal hearing.

When I graduated from high school with honors including a Faculty Cup, an award selected by the entire high school faculty for having demonstrated the greatest academic, leadership, social, and personal development, I felt that this marked the end of my overcoming obstacles.
About a month before I started my first year at Savannah College of Art and Design, I was at the optometrist to have my eyeglasses prescription checked, and that one appointment turned my life around. The optometrist noticed that I was reading the first and last letters incorrectly, and this sign alerted her to check my eyes. She noticed spots in my eyes, which meant that there was a possibility that I had retinitis pigmentosa. Fortunately, my father, who is a surgeon at the hospital where my appointment was located, was able to immediately put me contact with an RP specialist. After hours of testing, including the visual field and the ERG and different specialists looking in my eyes, my diagnosis was confirmed. I was hit with a new obstacle. I felt that I had fallen down the ladder, and I had to climb back up to reach a star.

After a few days of tears and worrying, I eventually moved on by not thinking about my diagnosis of RP and thinking, “Today is the present. That is why it’s called a gift.” I just enjoy my life and all the usable vision that I have now.
My family and I certainly did go through some moments of ups and downs during the first year, especially when I was advised to transfer to a liberal arts school as my parents were concerned about my future. While I understand that my parents don’t want me to be disappointed about my future if there are no treatments by the time I need them, I want to enjoy what I love to do while I have good usable vision and just wait to deal with the obstacles when the day arrives. Also, because there are so many potential future treatments, if an effective treatment for RP arrives, I don’t want to feel that I have wasted my time worrying, and I don’t want to feel disappointed that I could have stayed with my desired path, which is to be a photojournalist.

I’m still currently at SCAD and am majoring in photography, a major that I absolutely love very much. I’m also a features editor for the school newspaper. Being involved with the newspaper has given me peace of a mind that I have a back-up plan to be a journalist if the RP treatments do not work out.

Speaking of my statement, “I felt that I had fallen down the ladder, and I have to climb back up to reach the star,” after a lot of thinking since my diagnosis, I realize that I actually did not fall down the ladder because I did overcome the deafness part. I was just simply given another ladder to climb up, and now, I’m just dealing with the blindness part. I have a website that speaks about my sister’s and my life with cochlear implants and has information about cochlear implants.

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