By Everyday Heroes Kids Community Manager, Pamela Aasen, as published in Exeptional Parent Magazine, May 2023
When we receive a diagnosis that our child is not the healthy baby we were expecting, the future can look bleak and the unknown can feel scary. Of course, we need to grieve for the future we had envisioned for our child, but once acceptance settles in, it is time to make a new plan and put the pieces in place to give our children the tools to be successful.
FINDING THE PIECES
Regardless of the diagnosis, we seek out the therapies and strategies that are going to help our children and start putting the pieces together to find what makes sense for an individual’s needs and interests. Everyone has their own puzzle and the pieces and sizes are different. For my sons, Ethan (21) and Gavin (18), who have Usher syndrome type 1b, there were times we had to work on finding the bigger pieces so eventually we could move on to the smaller pieces and then put it all together. They were born with profound sensorineural hearing loss with no residual hearing, have retinitis pigmentosa (causing tunnel vision and eventual blindness), and vestibular dysfunction. None of this was familiar to my husband and I and in the early days it seemed that understanding how to put this puzzle together was impossible. We had to look at it in parts so it was manageable and not overwhelming.
THE PATH TO LISTENING AND SPOKEN LANGUAGE
When Ethan was diagnosed with a profound sensorineural hearing loss at ten months old, we were presented with communication options and after thoroughly researching those choices, we decided on cochlear implants to give him the opportunity to be a part of the hearing world. This decision was not made lightly as we had never heard of cochlear implants 20 years ago and it required surgery. Since the technology would give him access to listening and spoken language and success was related to the age of implantation, we felt we needed to make that decision for him because he was too young to do it himself. When Gavin was identified during the newborn hearing screening and diagnosed soon after, our decision was the same for him. Making the decision was just the beginning of this journey. There was still a lot of work to be done to ensure that both of our children were successful cochlear implant users.
After Ethan and Gavin’s cochlear implants were turned on, attending Auditory Verbal Therapy appointments was crucial to helping them make sense out of what they were hearing; having the implant did not mean that they were automatically going to be able to ‘hear’ and understand. They had to work to build new pathways in their brains that would help them understand these sounds. The boys’ speech language therapist, Karen MacIver-Lux in Toronto, made it clear that it was our efforts, both during therapy and afterward, that would determine how well Ethan and Gavin would communicate and use listening and spoken language. She gave us the tools, in a play-based setting, to support our children and coached us on how to make everyday activities into learning opportunities for language. Once Auditory Verbal Therapy stopped, they had Teachers of the Deaf and Individual Education Plans (IEP’s) until they graduated from high school to continue to provide the support that was crucial to their success as their needs changed.
I first had to support the learning of different sounds, however, once they started to develop language, I began with language experience stories about every activity and event. Digital cameras were just coming out so I made stories with the pictures so we could talk about what we did. Of course, we read a lot of books and my husband took on the bedtime story each night. Every toy we bought had a purpose. We chose toys that would elicit language, like the Fisher Price Little People Neighborhood sets…I think I played more with toys during that time than I did when I was a child. As they got older, in elementary school, their Teacher of the Deaf (TOD) would talk to them about current events if she had time left after reviewing the lessons of the day. They really enjoyed this, so I put up a map so we could talk about what was going on in the world and where the countries were located. They loved airplanes, space, and rockets so I found models of the planets and bought toy airplanes and spaceships; all regular toys but for us, they had a purpose in helping us increase their vocabulary through our discussions. Their grandparents, Aunts, and Uncles also participated in the efforts to build their language skills.
I didn’t need to buy specific therapy toys, or games, they had the same toys as their friends. It was the need for increased interaction with a focus on language development that made it different. Ethan and Gavin just needed a little more emphasis to help them with listening and spoken language. Vocabulary and colloquial expressions needed to be taught because they do not naturally absorb that information the way hearing children do. With this in mind we started dinner conversations when they were in kindergarten using prompts from the Scholastic Family Dinner Box of Questions. Once we exhausted those questions we moved to brain teasers, bananagrams, puzzle games, and trivia until we eventually found the Professor Noggin Card Games. We had trivia games for Space, Canada, Canadian geography, Countries of the World, Famous Inventions, and more. When we moved to New Jersey, we bought the States, the History of the United States, and the Civil War. That a family tradition developed from this was an unexpected bonus. Our family dinners are fewer but when we do eat together, Professor Noggin often joins us.
VISION FOR THE FUTURE
With the later diagnosis of Usher syndrome when Ethan and Gavin were 7 and 5 years old, we welcomed a Teacher of the Blind or Visually Impaired (TVI) and an Orientation and Mobility Instructor to their team of specialists. We knew that there were accommodations they needed for their low vision, night blindness, and difficulty with contrast vision and we wondered when to teach them the special skills and tools they needed to navigate their surroundings as their vision loss progressed. As with everything, we navigated the situation the best way we could, with the information we had and looked to them to help us figure out what we thought they could handle. We started by letting them know that their eyes, like their ears, needed help as well and a teacher would be working with them to help them just like their TOD. Turns out they knew exactly what they needed; they came home excited to tell us that they had pointed out where yellow tape would help them and in what parts of the school they had difficulty seeing because of bad lighting. They worked with a TVI throughout their school years to ensure that their needs were being met in terms of font size, use of contrast, books on tape, and other accommodations that were appropriate for their visual needs.
Through elementary and middle school they had cane training and in high school they decided to learn the basics for braille. For the time being they prefer using sighted guides and rely on friends and family to assist them while their white canes hang on a stick by the door. For our part, we have concentrated on providing them with opportunities to travel and have as many visual experiences as possible. They have participated in events with the Visual Experience Foundation, including a Bus Tour of New York City and their Surfing for Vision event on the Jersey Shore, where they were encouraged to save their memories through a visual ‘Burn It’ experience.
During the summer of 2019 I wrote a blog for the Usher Syndrome Coalition about our approach to our children’s lack of balance (see resources). I was at the 2019 Adaptive Sports Junior Nationals watching my children compete in swimming, track, and field events; Ethan was 17 and Gavin was 15 at the time. For me, I couldn’t believe that these were the infants who couldn’t hold up their heads, the babies who couldn’t sit on their own and the toddlers who couldn’t walk…not to mention the challenge of their combined hearing and vision loss. They would go on to compete and have success in para swimming events across the country, even the National Paralympic Championships. Despite their challenges, they were ATHLETES competing on a national level.
Unfortunately, the pandemic swiftly put an end to their paralympic goals and dreams for many different reasons. There was certainly the disappointment that accompanies such abrupt ends, but I reminded myself that those were the unexpected bonuses that came as a result of the years of therapy and training. It started with occupational therapy when they were babies to strengthen their motor skills and deal with their sensory integration issues. Then physical therapy when they were toddlers to work on their vestibular dysfunction. It took a few years and different physical therapists, but eventually we found a neuro Physiotherapist (PT), Sharon Shew, at Mobile Health in Milton, Ontario, Canada who explained that we needed to build their strength and help them develop a strong core to compensate for their missing senses.
A program was designed for them that combined traditional physical therapy and personal training. They had a plan for home using weights, a stability ball and a bosu ball. They also met with their PT at her office for specific exercises and assessment of the program and on a track so they could start running. At first, they couldn’t run in a straight line, but they persisted and eventually entered races, starting with Kid’s 2K’s and moving on to 5K races. As they got older, they joined the gym and worked with personal trainers. Sports were a huge part of their lives. They tried and loved them all…even if they weren’t very good at some of them. They never gave up and worked hard on getting better both by themselves, with their coaches, and with their personal trainers. Gymnastics and Tae Kwan Do were activities they enjoyed, they played golf, tennis, baseball, basketball, and volleyball at different times with baseball lasting from elementary until high school. They have skied since they were toddlers. In high school they joined the ski club, swam varsity, and joined the cross-country team. Now that they are in college, they enjoy playing club sports. Ethan and Gavin have always enjoyed their involvement in mainstream sports with their peers but there are and have been many challenges with that. It was joining a paralympic sports club that really gave them the opportunity to continue to participate in sports on an equal footing.
Swimming was the game changer for Ethan and Gavin because they had always loved the water. As time went on swimming was the sport that really transformed their bodies, they became stronger, and as they got stronger, they moved their bodies with confidence. They even learned to ride a bike…they had been trying to master riding a bicycle for years! The key for success in therapy for their vestibular dysfunction was not constant therapy appointments but finding the activities they enjoyed that incorporated the skills they needed to develop. Swimming might not be that activity for everyone, maybe it’s horseback riding, martial arts or another activity that requires core strength. My husband and I never forced activities on them, we gave them the opportunity to explore and find out what they wanted to pursue. One thing that the pandemic gave them was time to practice biking at the golf club next door. And more importantly for me, it gave us our very first family bike ride when they were 18 and 16 years old.
Addressing social emotional and even nutritional needs was just as significant as attending the therapy sessions for Ethan and Gavin’s vision, hearing, and balance. Our strategy of turning appointments into an adventure and a visit, as I have addressed in a previous article on the parent professional partnership (see resources), was important because we did not want them to feel like their lives were just about appointments. We were thinking about their mental health from an early age; addressing that need also included counselling for us as their parents and ‘check ins’ for them to talk about their feelings with a professional. As they got older, mental health checks continued and scheduled more regularly as needed, particularly recently in dealing with the aftereffects of the pandemic. As their parents, we can only hope they will choose to continue to take care of their mental health as they move into adulthood. We have helped them move toward that by finding mental health professionals who have experience working with individuals with hearing and/or vision loss. They even have a friend/mentor, Rebecca Alexander, who is an author, extreme athlete, and a psychotherapist who they can talk to about their diagnosis.
I believe having role models, mentors, and peers is another aspect that contributes to positive mental health (see resources for previous article). Ethan and Gavin have attended many events and activities over the years that gave them the opportunity to be around other children and adults like them. Attending the Ava’s Voice USHthis Camp, an overnight camp for youth with Usher syndrome, first as campers and now as counsellors/mentors, has addressed their need to connect with their peers in a way nothing else can compare. It gives them the opportunity, for a short time, not to have to explain themselves. Presently, they are also both attending the Rochester Institute of Technology (RIT) which houses the National Technical Institute for the Deaf. RIT has more than 1000 Deaf and Hard of Hearing students on campus and provides them with high quality communication access and support services.
Sharing their story has also has positively impacted their social emotional well-being. When Ethan and Gavin showed an early interest in giving speeches, I built on that through opportunities to speak at fundraisers, teacher trainings, and university classes. Having the confidence to speak in front of groups to raise awareness about their challenges helps them face life unapologetically and builds their self-esteem. I felt strongly they needed to understand and embrace what was/is different about them and go out into the world believing they are fine just the way they are without shame or the reluctance to speak up. The strategy has more than paid off. As current Advocacy Ambassadors for Ava’s Voice (with Ava) they continue to make the choice to speak to families and share their story. As a result of everything they have done they wereasked to share their advocacy journey with the Amplify Advocacy Series by the SPAN Parent Advocacy Network, have shared what life is like for them in the Sense Stories series by the Usher Syndrome Society, and along with both being Cochlear Scholarship recipients, they were named National Inspirers for Cochlear’s Achieve Anything Program (see resources).
Of course, none of this means there are not bumps along the way. How could there not be…they both went through middle and high school, experienced bullying, lived through a pandemic, lost the ability to drive, and innumerable other things that affect mental health. My husband and I have tried to emphasize to Ethan and Gavin that there is no right or wrong path, just their path. All we can do is give them the tools along the way through the different types of therapy, programs, and activities. This has also included dealing with sensory integration issues through sensory diets, massages, and learning coping strategies. Though there is no cure for their eye condition, we have encouraged making healthy food choices through nutritionist visits and nutrition education about ‘eye foods.’ We have embraced alternative medicine through a naturopath and recent visits to an acupuncturist. We also showed them by example that an active life, through a combination of sports, outdoor activities, and travel are good habits to develop throughout life for both physical and mental health.
A BEAUTIFUL MOSSAIC
Like most people, we are Facebook family. We share the successes of our children because we want family and friends to know Ethan and Gavin are doing well despite the challenge of their diagnosis. We are more private about the struggles or the dark times when the reality seeps in that life is hard for them every day despite all we have done to help them. It has been important for us to talk about this openly and with trained professionals because having Usher syndrome doesn’t mean that life can’t be good. The important lesson we have tried to teach them is to be open, seek out assistance when they need it to adjust to change, and most importantly to believe that they are worthy. Now, as I see my sons move on to early adulthood, I realize it wasn’t really a puzzle, because there was no one right way to make the pieces fit and there were pieces that were very different for both along the way. When I look at my sons, I see two beautiful mosaics made up of irregular pieces; some are colorful, some shine brightly, some are dark and grey, some were placed many years ago and some more recent, some were put in place by us, their parents, many were placed by the family, friends, and professional that surrounded them, and most were placed by each of them as they made choices throughout their lives. As their parents we did not aways make the right choices, react appropriately, or say what we should have but we did our best and are proud of the young adults they are now.
Finding Balance Blog
Mobile Health Network
Professor Noggin’s Card Games
Visual Experience Foundation
Amplify Advocacy Series by the SPAN Parent Advocacy Network
Advocating as a Family in the Community by Pamela Aasen
Mentors, Role Models, and Peers by Pamela Aasen
The Parent and Professional Partnership
Ava’s Voice USHthis Camp
Usher Syndrome Society Sense Stories
Everyday Heroes Kids
Usher Syndrome Ireland
Usher Syndrome Coalition