Learn about living with Usher syndrome from people with Usher

Bella's 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 we 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 her father 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. We 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.

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