She was 22 when she found out — a time when you’re figuring out the routes on life’s road map. She went from feeling like the world was full of options, to suddenly feeling hopeless and powerless.
“When you are told you are going blind, all those things you thought you were going to be come into question,” said Megan Kennedy, now 30. “Am I going to be able to do what I wanted to do?”
What made the diagnosis even scarier was that Kennedy was born profoundly deaf. As her vision shrank, she feared her happiness would surely wane along with it.
Kennedy, of St. Louis, has Usher syndrome, which causes varying degrees of deafness and blindness. She was born with severe hearing loss but didn’t know she had the genetic syndrome until she later started losing her sight.
Since childhood, she had wanted to be an architect. She loved how a building could make her feel. Reeling from her diagnosis, she left the architecture program at the University of Kansas and returned home. She enrolled in the program at Washington University, not yet ready to give up on her dream.
“This was the one thing I had to hold on to,” Kennedy said.
Completing the degree was a struggle. Her eyesight got worse. Assignments took her twice as long. She was surrounded by successful classmates. She thought she wasn’t talented enough, that she didn’t work hard enough.
With little information about Usher syndrome and no others to talk to about what it was like living with the diagnosis, she felt isolated.
But on days when she felt most crushed, she would get a call, a note or a visit from family, friends and even acquaintances. That support lifted her.
In 2008, Kennedy and her mom, Terry Cacciatore, were inspired to start a foundation – now called the Megan Foundation – to help others with the syndrome. They held a fancy fundraiser the following Valentine’s Day.
More than 500 people attended. Kennedy was amazed when she saw the long line of people waiting to get in, half of whom she didn’t even know. Maybe, she thought, there was something to this.
This Valentine’s Day will be the Megan Foundation’s fifth annual fundraiser, bringing in a total of more than $100,000.
Kennedy has lost her peripheral vision and sees just 10 percent of what’s in front of her, but she sees life more clearly than ever.
Kennedy took a test that estimates when she will lose her sight completely. The prediction: age 36.
She has an 80 percent hearing loss. That level has stayed about the same most of her life, but Kennedy has learned of people with Usher syndrome going deaf in their 40s or 50s. She uses hearing aids in both ears and struggles in noisy environments and deciphering high-pitched sounds.
In college, after nearly getting into an accident while driving at night, she realized something was wrong. She had never heard of Usher syndrome when she was diagnosed.
More than 400,000 people in the world have Usher syndrome, including 50,000 in the U.S. It is a complex genetic disorder, involving multiple mutations that result in varying degrees and rates of hearing and vision loss.
The vision loss is due to the degeneration of the retina, where cells convert light into electrical impulses that transfer messages to the brain. Hearing loss is similarly caused by damage to nerve cells found in the cochlea, the transmitting structure in the inner ear.
Dr. Rajendra Apte, a retina expert at Washington University School of Medicine who cares for Kennedy, said that while Usher syndrome has no treatment, medications to slow the progression of the disease could be ready for testing in five years. A bit further off, he said, are gene and stem therapies that could regenerate eye cells. Scientists are also working to create a retinal implant, which would work similarly to a cochlear implant for the deaf.
Research has improved in large part thanks to groups like the Megan Foundation and the Usher Syndrome Coalition, which also started in 2008, Apte said. More people are now learning how devastating Usher syndrome can be for families.
Kennedy had a hard time imagining what it would be like to lose her sight. The fear would stop her in her tracks as she went through her day, watching her niece laugh or sunlight filter through a window. She wondered how she could find purpose, spend time with friends or feel close to those she loved.
TEXTURE OF VELVET
Because the disease slowly progresses, dread can be just as difficult to deal with as the worsening physical limitations, Apte said. “It’s like having an 800-pound gorilla on your head, where you’re thinking the whole time, ‘Where am I going to be 10 years from now?’”
Kennedy’s mom recalls the heart-wrenching weeks after the diagnosis, when her daughter was trying to finish her semester at KU before returning home. All Kennedy wanted to do was sleep. Depression and exhaustion were gripping her, so Cacciatore visited often.
“I remember one instance when I busted the door open, pulled the covers back and put her feet on the floor,” Cacciatore said. “I said, ‘You are not going to let this destroy you.’”
Coming home was the cure. Kennedy saw how her family and friends believed in her. She realized they would love her no matter what and would always be there. It’s an appreciation she never would have had otherwise.
“Mom, would I ever change this?” Cacciatore said her daughter would wonder. “As horrible as it was, we found so much love from people. Even in my own brothers and sisters, I saw something more deeply in them that I wouldn’t have ever noticed in my busy life.”
Kennedy’s fear is subsiding. She doesn’t want to forget what things look like, she says, so she studies hard things like the texture of velvet, the color red and the house she grew up in.
“I can be in the moment and be really happy where I’m at,” Kennedy said. “There are beautiful things that come out of that – just learning how great it is right now.”
Her guide dog, Sunshine, alerts her to obstacles and finds the doors in a room. She enjoys going to the Missouri Botanical Garden on sunny days and visiting friends in quiet coffee shops.
People tell her she’s an inspiration, but she finds inspiration in others. People accept her, so she has become more accepting. Everyone struggles, she’s learned, and connection and empathy are what give us strength.
Decisions, she said, have been stripped down to what’s important. In May, she eloped with Ryan Kennedy, 34, after just a few months of dating. In September, she quit her job at a marketing company where she wasn’t happy. A couple of weeks ago, she and her husband moved into an apartment building on the Hill that her parents rehabbed and she helped design.
She wants to focus on the foundation — maybe start youth camps, scholarships or travel expeditions to see things while patients can. She could devote more time to her service with the national Usher Syndrome Coalition and local Diversity Awareness Partnership. Maybe she’ll refurbish old buildings.
“My story is changing,” Megan Kennedy said. “I’m not exactly sure what it is, but I’ll find an answer to what my life is supposed to be.”