‘I Have A Rare Syndrome That's Making Me Go Blind, So I'm Making The Most Of My Vision—Even In Quarantine'
My younger brother, Tyler, and I were both born with a moderately severe hearing loss. Without hearing aids, I can only hear high-intensity sounds like lawn mowers and car horns, and I can’t make out conversation-level sounds unless people are super close to me.
Fortunately, after my diagnosis when I was young, I was fitted with hearing aids, which gave me close to normal hearing. My quality of life significantly improved, but living with a hearing impairment still came with plenty of challenges. My hearing aids would short out if they got even a little bit wet (even from sweat), and it was hard to accurately listen and stay engaged in group conversations.
Despite these obstacles, I always took great comfort in knowing my hearing aids would be enough to guide me through life. And I always had my vision, I thought at the time. That all changed during the spring of my sophomore year of high school, in 2010.
I started to notice that the nighttime seemed darker than usual.
At first, I didn’t think much of it, but I still remember the moment I realized something was seriously wrong. It was around 9:30 p.m. on a school night, and I was out with the girls on my tennis team. Carrying out a local tradition, we were sneaking across my hometown to the houses of some players on the boys’ tennis team in order to toilet paper their yards in celebration of the next day’s match.
One of the last houses we headed to was in a more rural part of town. We were crouched down in the bushes outside the house, about to throw some paper, when we suddenly heard the front door open. Immediately, my teammates got up and started sprinting. I turned to follow, but amidst the pine trees and bushes, I could barely see what was in front of me. Thankfully, one of my teammates ran back to grab me, and I simply pretended I had stopped to tie my shoe.
That was the first time I realized that my eyes didn't seem to adjust well to the darkness of the night. After that, I started to become more aware, noticing that my eyes didn't even adjust to the diminished light as the sun began to set. The breaking point for me, finally, was when it occurred to me that I hadn’t seen the stars in a really long time. The next clear night, I went outside and sure enough, I could no longer see the stars at all.
As most people do when something’s wrong with their health, I Googled every terrifying explanation for why I couldn’t see at night. After several different eye doctor appointments, my parents eventually brought me to Massachusetts Eye & Ear in Boston to get checked out.
At 17 years old, I was officially diagnosed with Usher syndrome.
Usher syndrome is a rare genetic condition that causes combined and progressive deafness and blindness. And shortly after my diagnosis, my 15-year-old brother Tyler received the same one via genetic testing, but he had yet to experience any symptoms of vision loss.
Our diagnosis came as a complete shock to my family. We’d never heard of Usher syndrome. Around the world, only about three to 10 in 100,000 people have this disease, according to the National Organization for Rare Disorders (NORD).
Usher syndrome is rooted in a genetic mutation that causes retinitis pigmentosa (RP), a disease that progressively destroys the cells in your eye’s retina, and interferes with the development of crucial sound receptor cells in your inner ear. There are three clinical types of Usher syndrome, which are defined by the severity of the symptoms as well as the age in which the person starts experiencing hearing and vision loss. Since my brother and I have Usher syndrome type 2, the doctors don’t know if we will go completely deaf, but it is certain that we will continue to lose our vision until there is nothing left.
I was devastated upon receiving my diagnosis, but I also felt a strange sense of relief.
Even though I had just found out I would eventually go blind and potentially deaf, I still had time to do something about it, time to live my life as fully as I could during the years that I still had my daytime vision.
Today, eight years after my diagnosis, my hearing loss remains moderately severe. Unlike with Usher syndrome types 1 and 3, which always lead to profound or total deafness, the doctors still don’t know if or when my hearing could start to worsen with type 2. It’s a little unnerving, to say the least.
My night vision, on the other hand, has significantly deteriorated. I can no longer drive at night, so every day I look up the sunset time in order to schedule my activities accordingly. In the last eight years, I’ve had to rely heavily on friends, family, and, if I have spare money, ride-sharing apps. In the evening, I’m always fumbling around my apartment in order to locate things like my phone, the light switch, or the doorway.
During the day, I also can’t see in any dimly lit environments, like restaurants with low lighting, many bars (clubs are an automatic no for me now), movie theaters, and even airplanes when the shades are lowered.
The vision loss component of Usher syndrome begins with night blindness, followed by a narrowing of the visual field into tunnel vision. The final stage is total blindness. These days, I have a few blind spots during the day, most noticeably in my peripheries. This means my visual field is narrowing and moving in the direction of tunnel vision.
As my vision continues to deteriorate, I understand that the challenges I face on a daily basis will only increase. Not only am I continuing to learn how to deal with my disabilities, as they impact all areas of my life, but I'm also figuring out how to mitigate the emotional weight they hold. It’s been particularly difficult not knowing how quickly my vision will disappear.
Doctors can’t tell me exactly when I won't be able to see at all, but the general guidance from the medical community is that those with my type of Usher syndrome will have very little vision left in their 30s and 40s. However, I know several members of the Usher syndrome community that fall outside of this parameter. Some lose their vision sooner, while others get a few extra years. Regardless, I will likely lose most of my vision over the next five, 10, maybe 15 years. A silver lining: My vision won’t be completely gone in a matter of months or anything.
Knowing there is presently no cure for Usher syndrome is another emotional burden. I’ve had to learn how to tell both old and new friends what was happening to me. But to this day, many people in my life still don’t know. I haven't mastered how to start that difficult conversation.
Up until recently, I was traveling the world as much as I could to create visual memories before I run out of time.
When I’m not working as a marketing manager and pursuing my MBA at Vanderbilt University, I spend at least 30 minutes every day, if not more, researching all of the places I want to go and planning overly detailed itineraries.
In early 2016, I went to Iceland on a mission to see the northern lights. I spent four hours searching for the lights, unsure if my vision had deteriorated so much that it was too late for me to see them. I broke down crying when I finally saw the lights dancing across the sky just outside of Reykjavik.
The next November, I ventured off to Asia for the first time to experience one of my other most incredible visual memories to date: the annual festival of Yi Peng. In Chiang Mai, the largest city in northern Thailand, I watched as thousands upon thousands of lanterns were simultaneously released into the night sky in order to let go of misfortunes and make wishes for the new year. Witnessing this was indescribably beautiful and emotional as I released my greatest wish that one day a cure would be found for Usher syndrome.
From trekking across Europe to checking Bali, Indonesia, off of my bucket list, I’m grateful for the motivation Usher syndrome has given me to live my life in the moment. I recently started a travel blog, Wanderlight Moments, to share my experiences and hopefully inspire others to also travel the world, despite life’s unexpected challenges. Whether it’s Usher syndrome or something entirely different, life is full of struggles that we all face every single day. We are not alone in our adversity.
Of course, the current global health crisis has changed a lot of my plans, and I'm accepting that.
At first, I didn’t hide from the news, despite the extra anxiety it provoked. I saw the footage of what was going on in the frontlines at the hospitals. I saw the first responders, grocery store employees, gas station attendants, construction personnel, delivery drivers, and businesses struggling to stay safe, help one another, and survive.
I’m one of the millions of people staying at home to do their part. I see the economic devastation produced by this virus. Putting food on the table and paying this month’s rent is a luxury. Emotionally, I’m having a hard time holding up these days, and I'd be lying if I said otherwise. But my inability to travel for a year of my life in which I still have my eyesight is a pain I just have to get over. In the midst of everything happening, sometimes I tell myself: My individual situation doesn’t matter.
All of that being said, one of my mentors has also reminded me that it is okay to give myself permission to grieve my own loss in the midst of novel coronavirus. I’ve lost something that was such a core part of who I am and the primary way that I cope with this terrible disease I have. Valuable time in which I still have my vision will be spent in self-quarantine, and I have to come to terms with that.
Embracing the grief process has also allowed me to find strength in developing a new approach for 2020 and onward. Even though I cannot physically travel, I am now spending my extra free time planning future trips, possibly for 2021 and 2022. I’m finding joy envisioning myself in bucket-list destinations, like the Galapagos and New Zealand, as I draft detailed itineraries that, even if not followed exactly, will save me significant time later on. I also started digging into my old trip archives in order to document my previous travels on my blog and better preserve those visual memories, too.
Right now, I'm focusing on the gift I have in being able to still visually witness the simpler things in life.
For so long I was focused on creating visual memories around the world that I neglected the value in forming visual memories at home. Recently, my siblings and I moved back in with my parents, so I am cherishing every moment I have to spend time with loved ones and see their faces. Deep down, I’m beginning to fear the day in which I will no longer see the faces of those I love.
When I look back on 2020, I don’t want it to be a year just spent in quarantine. I don’t want it to be a year in which I wasted precious time with my vision. No matter where I am in the world, I will make the most of the eyesight I have left. I know that one day I will get to travel again. In the meantime, I will create new visual memories right here at home, with family.
As I look into the future and picture my life with Usher syndrome, there are so many unknowns. I know it won’t always be easy, but I am determined to never let this disease hold me back from traveling and living my life. Above all else, I am deeply committed to raising awareness of Usher syndrome. I hope one day we will find a cure.
If you’d like to learn more about Usher syndrome, treatments, and research, please visit the Usher Syndrome Society website.
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