The Dunning Story

Bella was born profoundly deaf, which was an extremely difficult experience. We were fortunate to have access to some of the best and most caring professionals in the world in the Boston area.  Bella began signing at 8 months of age, earlier than most children speak, and after receiving cochlear implants, slowly transitioned to spoken English.  She was soon in a mainstream school maintaining grades on a par with her peers and a reputation as one of the more popular kids in class.

But my wife and I sensed that something else was wrong. Bella had severe balance problems. Even at seven years of age she still couldn’t negotiate a balance beam while her four year old brother could do so with ease. She tripped over things all the time, obvious things, like our poor yellow labrador retriever. One weekend we went camping and Bella was terrified to walk to the outhouse in the dark, even with the aid of a flashlight. She clung to me like plastic wrap as we wound our way down the path.

A few months later it all made sense. Bella had Usher syndrome. Usher was the cause of her deafness and her balance problems. It was also threatening to take her vision. At eight years old, Bella was effectively night blind, requiring 100x more light to see an object than the rest of her family. My wife and I were devastated.

But the truth is that it has only gotten better from there. Bella continues to thrive in school. She rides horses and dances and next year she plans on joining the school chorus. Not bad for a deaf kid with poor balance. Two years since diagnosis her vision has shown no further deterioration, though we fully expect that it will change for the worse over time.

Bella needs supports to succeed. The school provides an aid in the classroom. The aid is there mainly for Bella but helps all the kids in class. Bella uses an FM to assist her in the classroom. Without it, she struggles to hear. She has physical therapy a couple of times a week to improve her strength and offset her balance issues. Her vision is not yet a problem in class, but the school is making arrangements to help her mobility, such as adding bright tape to the edges of stairs to help them stand out. We encourage Bella to carry a small flashlight with her and she has a good friend in class that walks with her during firedrills since in a real fire her lack of night vision might be problematic. In the evenings we often have to hold Bella’s hand in parking lots or when we first enter the house to guide her past obstacles.

This might sound overwhelming and complicated, but it was all done incrementally. We adjusted as problems arose, adding a new procedure or intervention when it became necessary, one at a time. For Bella, it’s just her way of life. She finds none of it odd and few of the other children in school do either.  In fact, Usher syndrome has made her more of a celebrity than an oddity. Perhaps it is just her personality, but Bella seems to see her physical problems as a challenge rather than an inconvenience. Arriving earlier, asking more questions, and working harder is simply the way she lives. She knows nothing else. In short, Bella leads a happy life with few limitations at the moment, so that’s where we focus. Tomorrow is always uncertain, especially with Usher syndrome, but for today life is good.

The McKittrick Story

When Conner was 10 months old we found out he was profoundly deaf. At 13 months of age, he received a cochlear implant and because of this, he was able to attend a mainstream school and lives a life with little impairment. His speech and language is still delayed but within normal limits. As his eyesight degenerates, he has trouble with dark areas, but his sense of humor and supportive family help him to overcome the challenges he meets each day. Now, with the birth of our fourth son Dalton who was born with profound deafness, we are undergoing the process for cochlear implants once again. We work harder than ever to support research to find a cure for our sons and all of those with hearing and vision related disorders.

The Rush Story

Hello. It was nice coming upon your web site. I myself have Ushers and so does my sister. I was born with hearing loss. I remember being fitted with hearing aids. I used to say to myself that I am blessed to have my eyes. Little did I know why I was going to Children’s Hospital of Boston. I never could understand why they were looking at my eyes also. I would say, “I am hear for my ears.” (lol.) By age 6, I started having trouble seeing at nighttime. By the time I was in my late 20’s to early 30’s, I started to lose some of my field vision. I could no longer play sports. I couldn’t see up close. I was using my central vision and peripheral. I had to move my eyes back and forth, sort of like scanning, and then used my thought process to fill in the missing blind spots. Of course, this is very stressful and takes a lot of energy from me. I am constantly bumping into co workers all the time. They now stop walking completely when I am approaching nearby. They get my attention by saying “Hi Mark” so I am able to spot their location and slowly maneuver around them.

I am 49 years young and I have been waiting all my life to be helped or cured. I tell people that I am not handicapped, I am challenged. My beautiful wife and I just had a baby girl 13 months ago. I WANT TO SEE and HEAR HER GROW UP. I hope my story helps others realize how hard it is for people like us with Usher syndrome and that my message reaches others who can help our cause. I don’t want to give up hope.

Sincerely,
Mark Rush