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Spring 2019

Spring/Summer 2010 Table of Contents
Versión Español de este artículo (Spanish Version)


By Amelia Westerfield
Reprinted with permission from Building Effective Programs (Winter/Spring, 2010), the Oregon Deafblind Project quarterly newsletter. To learn more about the Oregon Deafblind Project, visit them at .
Abstract: A young shares her life experiences and her approach to life with Usher syndrome. Keywords: Family Wisdom, deafblind, Usher Syndrome, transition, active living
Editor’s Note: We appreciate Amelia allowing us to reprint her wonderful article, as her positive approach and inspiring tips for living are beneficial for everyone.


Usher Syndrome is a genetic disorder that is characterized by congenital hearing loss and progressive vision loss caused by retinitis pigmentosa (RP). There are three subtypes of Usher syndrome: I, II, III. Usher syndrome type I patients have profound congenital sensorineural hearing loss, vestibular dysfunction, and progressive retinal degeneration, with impaired night vision and decreased visual fields. Usher type I is typically diagnosed within the first few years of life by early onset retinitis pigmentosa. Usher syndrome type II, the most common form of Usher Syndrome, is characterized by moderate to severe sensorineural hearing loss that is stable in most cases, normal vestibular function, and later onset retinitis pigmentosa during the second decade of life. Usher syndrome type III is the least common type, characterized by moderate sensorineural hearing loss with progression to acquired deafness, progressive and variable vestibular dysfunction, and later onset retinitis pigmentosa.

ONE DEAFBLIND WOMAN'S APPROACH TO LIFE

Three months before the end of my senior year of high school in Oregon, I was diagnosed with Usher syndrome II. On that particular Saturday afternoon, my parents called my brother and me into the living room and broke the news. I do not remember what was said, but I do remember lying in bed that night believing that my life was over and that all of my plans and dreams were unattainable. Little did I know that 11 years later I would be where I am today—living out my dreams in Seattle, creating more plans for the future, and knowing that despite my vision and hearing loss, anything is possible.
As I sit here at my computer and reflect on the impact that Usher syndrome has had, I realize that I am living the life that I always wanted to live. I am surrounded by my loving family and friends; I am married to my best friend; I recently got my graduate degree as a Master in social work and have begun my professional career as a MSW. I am training for my first marathon and am planning a trip to Russia next summer. At the same time, this does not mean that living with Usher syndrome is always a piece of cake. I still have moments when I want to curl up into a ball of self-pity or times when I am overcome by negative thoughts about what the future might bring. Despite these feelings, however, I accept who I am—deafblindness and all. We all have challenges in life that must be faced, and mine just happens to be Usher syndrome. Here are things that I have found helpful in my approach to life with Usher syndrome.

DEAFBLINDNESS IS NOT AN EXCUSE

I have never used my deafblindness as an excuse to stop working toward my goals or as a reason to lower my expectations. After I was diagnosed with Usher syndrome, my parents never once changed their expectations of me. It was never a question whether I was going to attend college, develop friendships, travel the world, start a career, fall in love, and continue to seek out new adventures while doing the things I love to do. It was my parents’ confident, consistent, overarching belief in me that taught me that despite my disability anything is possible. Over time, I have learned that I really don’t know what I’m capable of until I try, and that if I don’t try, I’ll never know. Once I truly began to adopt this philosophy, I found that things always have a way of working out for the best.

ADAPT, ADAPT, ADAPT, AND THEN, CONTINUE TO ADAPT SOME MORE

The hardest thing for me about living with Usher syndrome is that my vision loss is progressive. Although the deterioration has been slow, it seems like as soon as I become comfortable with the way things are, my vision decreases some more, forcing me to start all over again. I feel like I constantly have to adapt and adjust the way I do things. As a result, I have learned the importance of being open to change and developing new strategies to overcome the challenges brought on by my progressive vision and hearing loss. For example, during undergraduate school, I fell in love with running. After several blows to my forehead from lowhanging branches, I started wearing a hat with a visor when I was outside to help protect my head. Ten years later, I am still running and I am now training for my first marathon. In addition to wearing a hat, I now run in the evenings after work, using my husband’s arm as a guide. I am also slowly learning to enjoy running on a treadmill. In spite of the inexorable progression of my vision loss, I know that running will always be an important part of my life. It may just require a few adaptations as time goes on.

TAKE ADVANTAGE OF SERVICES AND PROGRAMS AVAILABLE TO PEOPLE WITH DISABILITIES

Another thing that I have found helpful in my approach to living with Usher syndrome is learning how to swallow my pride and accept help. Growing up with a moderate to severe hearing loss, my parents were constantly advocating for me at school and setting up special support in the classroom, which I found extremely embarrassing at the time. It was a constant fight in my house to get me to attend my IEP (Individualized Education Program) meetings. It was not until after I was diagnosed with Usher syndrome and began experiencing significant vision loss in addition to my hearing loss that I finally realized that to excel in school and life in general, I needed to accept help. After graduating from high school, I headed off to Colorado to attend college just as I originally planned to do pre-Usher diagnosis. At the time, I remember wondering if moving halfway across the country away from my support system was the best idea, but it turned out to be exactly what I needed. Being in a new environment without my parents advocating and arranging support behind the scenes, I quickly realized that no one was going to help me, unless I took the initiative. I did not know what kind of help was available until I asked, so I decided to inform myself. I was amazed to learn about all the different levels of services that are available to students with disabilities. In addition to the services provided through my school, I also received an amazing amount of support from the Oregon Commission for the Blind.

STRIVE FOR INDEPENDENCE

I believe that continuing to strive for independence is extremely important in maintaining my overall sense of wellbeing and self-confidence. As a deafblind individual, I have found that fitting into a society that revolves around being able to hear and see can be challenging. It would be extremely easy to sit back and rely on others to help navigate the world. I am married to a wonderful man who would not think twice about chauffeuring me to and from work and doing all the grocery shopping. I am not saying that I never ask for a ride or let my husband lend a hand; I am saying that for me, it is important to maintain my independence in certain areas of my life. For example, I have strategically picked where I live so that I am within easy walking distance of multiple bus routes, grocery stores, parks to walk our dog, and a gym. Being able to ride the bus to work and go shopping by myself provides me with daily reminders that, despite being deafblind, I am a capable, independent woman.

HOPE

Another thing that I have found helpful in my approach to life with Usher syndrome, is maintaining a sense of hope about what the future in science might bring for individuals with deafblindness. Although Usher syndrome is incurable at this time, hoping for a cure helps me cope. With the help of my father, I stay current with the latest research on deafblindness. Researchers have succeeded in reversing one form of deafblindness in mice, and others have given sight back to blind dogs. Reading about new scientific advancements inspires me to be excited and hopeful about the future. Sure, it still may be relatively far off, but there are scientific advancements being made all the time, especially now since the Obama administration is once again supporting scientific and medical research. I definitely feel better when I think about the possibility that there will be some sort of biological or technological procedure by the time I am 40 or 50 (just a guess) that would at least prevent me from going completely blind, if not restore my vision to the normal (or even better than normal!) standard. Regardless of what happens in the future, it can’t hurt to hope.

ACCEPTANCE

Coming to terms with being born with Usher syndrome has been a long process for me. I realize that having Usher syndrome is something that I cannot change, so I try not to let myself worry about it or feel sorry for myself, because ultimately that will be unproductive and not change anything. Whenever I bump into someone or stumble off of a curb, I try to push away the negative thoughts and keep going because I know I could not have avoided it and that it is not my fault. Now that I have adopted this mindset, I spend less time dwelling on the negative aspects of my disability and more time being thankful for what I have and hopeful for the future. And, as my brother says, hearing and seeing are overrated anyway!