Faces of Usher Syndrome
“You are a beautiful, strong, smart  individual.  You were that before your diagnosis, and nothing has changed that . What has changed is the way you will live your life, overcoming this obstacle that is Usher syndrome.” UW Retina Specialist


Usher syndrome is the leading cause of deaf-blindness in the United States.  You can read more about it in the About Usher Syndrome  link. 

You probably wouldn't recognize Usher syndrome in someone, if you didn't know they had it.  They look just like anyone else.  Your sister.  Your brother.  Your best friend.  Your son.  Your daughter.  You wouldn't know that they were struggling to see all of their surroundings, or to hear all of the conversation around them.  But they are. 

Someone with Usher syndrome recently said
that if they had lost an arm or a leg, people would be more kind, more understanding, because it would be obvious that there was something wrong.  That sometimes people laugh when they trip, or walk into something, calling them drunk, or a clutz.  That people get offended when they don't hear them say "hello," or see them walk up, calling them rude, or stuck-up. That sometimes their friends forget that they cannot see in dim light, and leave them alone in a crowd in a dimly lit room, or to walk in the dark alone at night. 

Part of the mission of  Vision for a Cure is to raise awareness about Usher syndrome.  We think getting up close and personal with four people with Usher syndrome is the perfect way to do that.  Here are but four of the estimated fifty thousand faces of Usher syndrome in the United States. 

 


Jodi ...  


I was diagnosed with Ushers syndrome in the Spring of 2009.  I was born with hearing loss, so I have had hearing aids my whole life.  It wasn’t until my mid 20’s that I noticed something wasn’t quite right with my vision.  I always had a very hard time in surroundings where the lighting was low/dark, and I now know why my clumsiness is excused.  I was always missing a step or running into low objects.  At the time I thought I was just clumsy, but now I know I really just didn’t see what was in front of me. 

My vision is best described as a donut … I see what’s in front of me.  The ‘hole’ - the circle - is best described as to where my ‘blind spots’ are.  Beyond the circle, I can tell something is there if it moves, but I would have to turn my head to actually see it. 

While I live a somewhat normal life, this disease affects my daily life.  I no longer drive.  I had to switch jobs because my job involved driving all day.  While the change was scary for me because I took a pay cut, I also know that I made the best decision for myself, and for my career.  Luckily, I am very blessed that I love my new job and the company I work for has been very supportive and helpful in understanding my needs/conditions.

When I was first diagnosed the best feeling I can describe was pure frustration. I didn’t understand why, at 29 years of age, I was just being told that I had this condition.  I had the same audiologist since I was 2 years old, how could he NOT put two and two together.  Needless to say, I switched audiologists after that.  

My frustrations then turned to depression, and I found myself wondering what my adult life was going to be like.  I was single at the time, and now terrified that if I met prince charming he was never going to want to take on my challenges.  Who would want to be with someone who is losing their vision, and could someday be blind?  He will be forced to ‘take care of me,’ and who knows how my mental state will be if/when that day comes.  While I did a great job of hiding this, inside I was in a really dark place. 

I sought out counseling and learned to find the positives.  I need to live my life
NOW, and try not to worry about what the future holds, because we cannot predict the future. 

We really have NO IDEA how bad my vision will get … my progression is slow right now, so I only notice minimally.  This is normal for me, because I have adapted to my surroundings over the years.  This is my NORMAL.  I take advantage of the smallest opportunities now because I don’t want to look back and say I wish I had done this, I wish I had done that.  I want to LIVE.  I hope/want to have a family someday, and I want to see my children have children.  I want to travel, I want to
SEE everything.  I cannot imagine NOT SEEING.  We need to find a CURE!

Thankfully, I am very blessed to have an amazing support system.  Without this I don’t know how I would feel today.  I have an amazing husband who is so caring and understanding.  He understands my needs, and is by my side, helping me before I even know I need help sometimes. I found my prince charming and I am so fortunate, because the future really doesn’t seem as scary with him by my side.  I have really found the light in my life since meeting him, and found myself letting go of the daily worries I once had.  My family and friends have been my rock, and while there are times I have to remind friends I can’t see or something, they all do a great job at making me feel 'normal.'

I truly am blessed and thankful for what I do have. 

I recently viewed a video that another Usher Syndrome person posted online. Her name is Molly and she is 19 years old. She puts it very bluntly in that you don’t have to look disabled to be disabled.

While I may have my daily struggles, there are people every day that will never know what mine are. Showing compassion and being sensitive to others’ needs is something all of us can do. It’s really sometimes very easy and, for someone who is disabled, makes all the difference in the world with how they are left feeling.

Ushers Sucks. What helps me get through it is knowing that we are making money for research …. knowing that we are making a difference …. knowing that five years ago none of you knew what Usher Syndrome was either, and now, together, we are raising awareness along with funds.

All of us can make a difference.  Let compassion be your first answer when you witness a moment in which someone may be struggling.

For me, life goes on. I choose to not let this take my spirit anymore. I want to live my life and enjoy what I do have. It’s important to focus on the positive and to surround myself with people and things that make me happy. I choose to expand my compassion to others, and I hope that hearing my story will help you all to do the same.
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Ryan ...


 Usher Syndrome isn't easy to write about.  I've tried numerous approaches and different angles.  But the most effect means is just being plain honest.  Usher Syndrome is a terrifying genetic disease to have.  I have no time frame of when degeneration will end, no cleat path to try and find a way to attack the disease, no clear future other than 'maybe' and 'possibly,' - nothing.


Every day I am lucky to have a tremendous support system in my wife that keeps me sane, keeps me going and keeps me from stepping back.  Thanks to her, I only move forward.  She drives me to work every single morning and picks me up every single night.  I have not driven a car more than one time in the last 3 years, and that was to take my wife to the ER at 3am.  My wife is the reason I am able to cope when I run into something, someone.  She tells me everything is ok when my frustration builds up and my eyes are showing the pain that I feel inside because it is just so, so hard to express what it’s like on a daily basis.  I would not be who I am today, where I am today, if it wasn’t for the support and love of my wife.

Then there are my kids, Lincoln and Finley.  How do I describe what it’s like being a parent and having this disease? I bump into them all the time, even when I’m trying really hard to make sure I’m paying attention to where I’m walking.  I sometimes knock them down or into something.  Sometimes they cry, sometimes they think it’s a game, sometimes they’re just confused.  Every time, my heart breaks.  It’s very hard for me knowing that because I sometimes can’t see them because of the complexity of my vision degeneration I am the reason my kids may get hurt.  I make sure they’re ok, or with their mom, and I have to go be somewhere by myself to try and keep myself from crying.  It’s incredibly frustrating, but sometimes, kids just know Daddy does things a little differently.  I’ve never sat down and talked to either of them about what is happening to my vision and hearing, it’s not something they need to fully understand yet.  Right now, they just know that sometimes I need help finding the ball we’re playing with, or finding something on the ground.  My kids are my heart and soul, and I don’t know what will change when they know the full extent of this disease.  I try not to think about it myself, and what I may miss out on in their future, it’s too much.  For now, I cherish every day, every minute with my kids.

Not everyone that has Usher Syndrome has this kind of support, not everyone has a person, a place, an outlet to go to.  In some cases, they are already blind, and can only hear because of a cochlear implant, or they are three years old and trying to start their lives in a way I didn’t know about until I was 28.  There are babies, kids, teenagers, twenty somethings, and Grandparents, that are all impacted by Usher Syndrome.  They all need our support, our love, and in the end, the vital funding that may one day help them, and future generations.  Usher is genetic, while my kids don’t have it, they are carriers of Usher Syndrome.  It’s a great burden to know that someone down the line in future generations of my family will have this disease because of me.  This is about more than raising money for research.  This is about helping to show the world that Usher syndrome is something that needs to be dealt with and giving people a ray of light in the darkness.

Thank you all for your support, your love, and for helping start the discussion of Usher Syndrome, its impact, and how we can work together on finding some research that may one day make life just a little bit easier for those of us with this disease.
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Leslie ... 


I am the youngest of four children.  I am the only one in my family with Usher Syndrome. I was diagnosed with a moderately severe hearing loss at the age of two, after my grandma was shaking a toy near me, and I didn't turn my head to see it.  


I was fitted for hearing aids, and received early intervention. I was able to be successful all through grade school, with amazing teachers, and hard work.  I never let my hearing loss bother me, nor did I really ever feel judged for having a hearing loss.  I went to a great school in a small community.  I spent my years playing sports and even made the volleyball team at a small college.  

It wasn't until after my second year of college, when I went to live with my brother and his wife to work for the summer, that I was diagnosed with RP (retinitis pigmentosa), then Usher syndrome.  I was 20 years old. Some things that had happened to me before the diagnosis made sense - like why I was tripping in a movie theatre, or slowly maneuvering through the woods and needing a friend to help, while everyone else was running.  


I was luckily still able to drive a few more years, which got me through college.  The reality of this eye disease didn’t completely hit me until just before my twenty-fifth birthday, when I went to renew my license, and failed the vision test.  The lady made me feel so miserable because I couldn't see the stupid dots.  I left crying hysterically.  I wasn't expecting to lose my driver's license so soon. I was only 24 years old. I may have been able to drive a little longer, but night time was already terrifying, and I did not want to be the cause of an accident.  

My husband and I have three beautiful girls who are the light of my life.  Yes, it has been a struggle for us, but we make do with a simple life.  Simple things others may take for granted are difficult - like grocery shopping, taking the kids places, getting to work, anywhere you would want to go. We have to brainstorm the best way to make it not stressful for us to go places.  It gets frustrating at times!  

I recently had a visual field test done, and my vision has gotten worse more quickly than I expected again.  Each year I face more challenges, and have to get used to the idea of people helping me --- I've always wanted it the other way around.  

One activity that keeps me going in the right direction is running. I am so thankful I have an outlet to clear the mind.  I also focus on what it is that I want my children to see when they watch me; A strong mom that continues to work hard.  

I have to live for today, because, as my vision worsens, thinking of the future brings me sadness, which is why we need a 
CURE!  A cure might not be in my life time, unless it is my miracle, but I think about the future of other children with Usher syndrome, and we need to fight for them!
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Andi... 
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July of 2012 was when I was officially diagnosed with Usher Syndrome Type II. What a crazy year. Lots of emotions. Feeling uncertain, happiness, grieving, blessed, fear, comfort, sadness, loved, etc.  There were many times where I felt guilty for having some sad days when I knew friends were going through cancer treatments and others who have it far worse than I do.  Around this time when I was feeling that, I had received an email from a fellow USII person whom I met though a blog last year and in her email she randomly shared this with me....

"It's such a roller coaster with this diagnosis. You feel so sad that this is happening to you, yet you feel so grateful that you've had the gift of being able to see in the first place. And you think of others who go through far worse things than you do, like cancer, but it still doesn't make the sadness of your situation go away, and it's perfectly ok to have that feeling, and I encourage you to really feel that for yourself. If you deny any sympathy for yourself, then you can't get started on a road towards encouragement and excitement and happiness."

Oh HELLO GOD! Yeah, that was HIM speaking through her to ME. I hadn't shared to anyone what I was going through because I was feeling so guilty. It was amazing to hear those sweet words. I then shared a bit to my mom and she gave me some wise words when I told her I felt guilty and selfish.... 

"You're not being selfish being sad...it's called grieving. Grieving of something that's being taken away from you"

All that to say, I have felt much happier now that I have allowed myself to grieve over the loss of my vision and my future vision. I have to remind myself that every day is new, take advantage of every opportunity of TODAY and not think about WHEN, or IF I will go completely blind. I continue to pray every night before bed that God will heal me when it is HIS time. 

You can read Andi's blogs at Notions 'N Dreams

 

 

  

 The mission of Vision for a Cure is to raise awareness and compassion about Usher syndrome, as well as provide funding to those that are dedicated to research to find a cure.
email: 
Info@VisionForACure.com  phone: 425-350-0121 mail:  PO Box 9  Bickleton WA 99322 
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