Both of my children have Usher syndrome, the most common genetic cause of combined deafness and blindness. It is a rare genetic disorder that affects hearing, vision, and balance; three major senses in the body. My sons were born deaf, are progressively losing their vision, and have severe balance issues due to vestibular dysfunction.
They were usually categorized as deafblind for academic purposes, but that label doesn’t include the part of their diagnosis that could also affect their physical, social emotional, and cognitive development. The combination of their sensory impairments creates a complex disability with unique challenges. Support in school included Teachers of the Deaf and Hard of Hearing (TOD), Mobility Instructors, and Teachers of the Blind and Visually Impaired (TVI). However, we were mostly left on our own to deal with the balance issues and the fatigue of living with a multi-sensory loss that adds another layer of difficulty to their daily lives.
MORE THAN JUST BALANCE
Early on, before the Usher syndrome diagnosis, while we were preparing for cochlear implant surgeries and attending auditory verbal therapy, my husband and I knew that there was more than both of them being deaf. They could not hold up their heads, sit on their own, or walk steadily. We brought it up at appointments, and despite being unable to meet their gross motor developmental milestones, it was not significant enough for most doctors to be concerned. We were told they would catch up, and that the cause was lax ligaments and low muscle-tone. We were anxious to find the cause, but since we couldn’t wait for that to happen, we embarked on a journey of therapy and exploration, determined to help our children.
I had been a special education teacher for 15 years by that point, with experience in adaptive physical education. My master’s thesis addressed the effects of a motor development program on the self-esteem of physically awkward children in first grade. My twin sister’s thesis looked at academic performance in the same motor development program. My inclination while watching my children falling and struggling during gross motor activities was to work on a home motor development program, of which just being active was a huge part. When they were babies and toddlers, we lived in a condo building in Mississauga, Canada with a pool, a racket ball court, a backyard garden path, and a park nearby. We used them all. We went to the pool and park almost daily. I bought a tee-ball set and baseball gloves with the purpose of working on hand-eye coordination. A couple of times a week, we would go to the racket ball court to throw balls and try to catch them. We would go for hours, around and around the garden path at the back of the building, so they could practice walking with their Fisher-Price walkers. These are all things that kids do regularly, but we did them with more of a purpose toward increasing body awareness and coordination.
I was fortunate to have had a year of maternity leave after Gavin was born. I returned to work part-time, so I could focus on what I needed to do for the boys. We began occupational therapy for sensory-integration issues when they were babies, and added physical therapy when they were toddlers. We did not know the cause yet, but knew instinctively they needed to be stronger. After the diagnosis, we searched for a therapist who really understood what to do for a child without a functioning vestibular system. We went to a few who focused on balance activities each week, but that did not yield any results. They still struggled with standing on one foot or anything balance related. Our search continued until we finally found Sharon Shew, a neuro physical therapist at Mobile Health Network and part of the Neurosciences Division of the Canadian Physiotherapist Association. She was the first to explain to us that with a multi-sensory loss, there was missing information that the brain was going to need to fill in through other sensory inputs from the environment, before producing movement and actions. She described her role as providing appropriate guidance, training, and coaching, while challenging our children and giving them opportunities to develop strategies to compensate when activities required balance and coordination. She understood that kids who face these challenges might feel less sure of themselves when it comes to trying new things. She warned us that it could make them less interested in joining activities. She mentioned that they might seem shy or even embarrassed if they feel like they’re taking longer to learn things. She explained that if they were doing things clumsily, it could lead to avoiding situations or activities that they find difficult (Synapse: The Vestibular System and Balance Vol. XXXIII, No. 2 Fall/Winter 2013/14).
FROM THERAPY TO THE FIELD
Sharon didn’t set up traditional weekly therapy. She basically became their personal trainer. She was aware that financially it would be difficult to meet weekly, so after the first month of evaluations and getting to know each other, we met monthly. We all agreed that we did not want their lives to be about therapy, so joining recreational sports also became part of the plan. They had a program to do at home using weights, a stability ball and a bosu ball (a fitness training device that consists of an inflated rubber hemisphere attached to a rigid platform, that is often used for balance training). We also met on a track so they could learn how to run, a fundamental skill of many sports. She gave them the opportunity to explore their bodies and movement patterns at their own pace, and to develop the necessary skills and coordination for smooth movement. In the beginning, they couldn’t run in a straight line. But they persisted, and eventually we entered running races starting with Kid’s 2Ks and eventually moving on to 5K races. It became a fun family activity that unexpectedly would lead to running cross country in high school.
Ethan and Gavin embraced it all. Even though they were young, they welcomed the personalized training programs, home exercises, and track sessions to build strength and core stability to compensate for their missing vestibular senses. They had an early love of sports, with parents who continued to participate and coach team sports in adulthood. They tried various activities: gymnastics, Tae Kwon Do, basketball, volleyball, baseball, and soccer. Their dedication and hard work paid off, as they made travel baseball teams and tried out for high school sports teams. They have developed a lifelong love for skating and skiing recreationally, and even had the opportunity to attend an adaptive skiing clinic for visually impaired skiers. They have played golf and attended golf camps since they were young and were a part of a blind golf organization that provided clinics, lessons, and even equipment. They consistently worked hard on improving their skills and getting stronger at whatever activity they were doing, both by themselves and with their coaches or personal trainer. They were varsity swimmers and ran cross country throughout high school. Ethan even made his high school freshman baseball team, and Gavin the high School Freshman volleyball team. They literally wanted to try anything and everything.
SWIMMING INTO SUCCESS
Ethan and Gavin have always loved being in the water and being recreational swimmers in our backyard pool. Whenever I watched them, it seemed to be a very comfortable space. It occurred to me that it helped them understand where their bodies were in space; perhaps the only place where they truly felt in control of their bodies. Initially, swimming lessons were a challenge because they couldn’t wear their cochlear implants to hear the instructor in group lessons. We tried private lessons for a while, but they were frustrated with having to take their processors on and off. Then Cochlear developed the waterproof cases for their processors and it was a whole new ballgame. Our family’s subsequent move to New Jersey in 2014 gave them the opportunity to join a summer swim club, which became a pivotal moment in their lives. It was swimming that truly turned them into athletes.
Swimming transformed their bodies. They became stronger. As they got stronger, they learned to ride a bike. Their confidence grew, and their coordination improved. Next came the high school swim team and a more competitive club team. However, while they enjoyed swimming and competing with their typical peers, their fatigue level at the end of a school day was problematic. It was like they had already practiced for two hours and then had to have a two hour practice with their typical peers. As their vision deteriorated, they sought an environment where they could compete on equal footing. The Children’s Lightening Wheels Paralympic Club provided that in swimming and track and field events. Witnessing their success at the 2019 Adaptive Sports Junior Nationals and then the 2019 National Paralympic Swimming Championships filled us with an emotional mix of pride and disbelief. These were the same clumsy toddlers who now competed at a national level. The pandemic disrupted their Paralympic dreams, but not their spirit. On a side note, the pandemic gave them the opportunity to practice riding a bike in a safe space at a nearby golf course. That gave us our very first family bike ride. Once the pandemic ended, they decided they wanted to focus on enjoying their life as college students and on being social, instead of spending all their time in a pool. Their love of sports, going to the gym, and being active motivates them to find other activities they enjoy, and at the same time helps them maintain a strong core throughout their lives.
LIFE BALANCE
My sons, Ethan (22) and Gavin (19) are now young adults and both students at the Rochester Institute of Technology (RIT). We feel fortunate that they were both accepted and then chose to attend RIT, because it houses the National Technical Institute of the Deaf (NTID). RIT/NTID has specially trained staff, and in my opinion, is the most accessible college in the world for deaf and hard-of-hearing students. The Disability Services office has worked with them to determine the most appropriate accommodations for their needs in relation to deafblindness. We are certainly very happy with the accommodations provided, but there are still gaps in addressing the needs of students with a combined hearing and vision loss, and/or balance issues in relation to housing, transportation, and scheduling. When living with multi-sensory loss, fatigue becomes a significant hurdle affecting daily activities, impacting both memory and vision, as well as increasing fall risks. This reality is essential when defining "full-time" for individuals facing these challenges. In 2021 RIT was one of six universities named as a Microsoft Accessible University. In 2022, Gavin was one of eight freshmen selected to become the university’s first Microsoft Disability Leadership Scholars to advocate and educate about disabilities. We applaud RIT’s commitment to providing accessibility for all students during their university years. We hope RIT considers the significant impact of fatigue.
While the world may not be perfect, I have faith that Ethan and Gavin possess the confidence, knowledge, and resourcefulness to advocate for themselves. During high school, their team meetings often included me, but now they handle things largely on their own, seeking my support only when needed. They understand their bodies, know when they need to rest, and try to adjust their schedules accordingly: all while trying to work hard and enjoy all aspects of college life. Recognizing the benefits of living with trusted friends in an on-campus house, they persuasively conveyed how this arrangement would enhance their quality of life. They could be exempt from the 2-year requirement to live in a dorm for deaf and hard of hearing students. These friends understand their needs and offer crucial support, like transportation or guiding them at night. Ethan created a ’saddle up’ code word to cue his friends when he needs a shoulder for someone to guide him. One of their life hacks is to use the camera on the phone to magnify and adjust contrast and the brightness of their surroundings, making it easier to see details and navigate independently. They considered a guide dog but decided that the logistics were too complicated, while away at school. They use identifier canes when travelling, but despite my efforts at bribery, they do not use their white canes regularly. Ultimately, I respect their journey and trust their choices.
DID SOMEONE SAY HOCKEY?
Ethan and Gavin have joined multiple intra-mural sports at RIT and decided last fall to add hockey! We are a hockey loving family and have always enjoyed watching and going to games in person. For my sons, it has been a lifelong desire, despite their balance challenges on skates, to play in a league. They did have the opportunity to play when they were much younger in a club for new Canadians who wanted to learn about the sport. They whole-heartedly enjoyed their chance until we moved to New Jersey in 2014, where their focus became baseball and swimming. However, they never stopped wanting to play and have finally done it, once again proving limitations are meant to be pushed. Recently, my husband and I drove the 5½ hours to Rochester so we could watch them play. When asked why we would travel so far to watch intramural sports, my husband put it into context, going back to when we thought they wouldn’t be able to skate at all. He explained that now it’s about watching them defy limitations and chasing passions. I had a huge smile on my face the whole time I was watching them! I am certainly proud of all the successes they have had. I think I am even more proud that they found a way to play this sport that they love, proving that ’good enough’ can be pretty darn amazing.
As I watched my sons on the ice, their struggles mirrored the challenges they face in everyday life. Each fall, missed pass, missed puck, or wobble on the ice did not discourage them. Instead, I sensed renewed determination. With each stride they took, I could see how much they were enjoying being on the ice in a real hockey game with timers and referees. Even with limited vision, they moved with confidence, a testament to their inner strength, both physically and mentally, and how hard they have worked to be able to do this. Their cheers for their team and appreciation of the skills of others show the best side of sportsmanship, with only occasional thoughts leading to what might have been. Usually, it is simply appreciation to be able to do what they are doing. In that hockey arena, I saw not just a game, but a powerful metaphor for their journey: falling down, getting back up, and facing every obstacle with unwavering spirit. They may not always see the puck clearly, but it is clear that their story can offer a message of hope and encouragement for anyone facing challenges, demonstrating that with the right support and determination, anything is possible.
ABOUT THE AUTHOR:
Pamela Aasen is the parent of two adult sons with Usher syndrome. She was a Special Education Teacher for 25 years and most recently was the Early Hearing Detection and Intervention (EHDI) Mentoring and Family Engagement Project Director for the SPAN Parent Advocacy Network from 2017-2022. She is the Education Advisor for Ava’s Voice and a Volunteer Ambassador for the Usher Syndrome Coalition.
Read the article here.