Amy Dixon was 22 when her eyes started playing tricks on her.
Back then, in 1998, she was working her way through college as a waitress and wine specialist. Dixon had always been athletic, a swimmer and equestrian in high school and college. She couldn't recall ever having major health problems. So when she began to experience flashes of light, strobing and incidents of blindness that lasted at least 10 seconds, she chalked it up to stress and exhaustion.
It turned out to be much more serious. After seeing a handful of specialists in the Northeast, Dixon’s condition was diagnosed as uveitis, an autoimmune condition of the eye that causes inflammation and destroys eye tissue.
While the type and severity of uveitis can vary, Dixon’s type, posterior uveitis, affects the retina (the light-sensitive tissue at the back of the eye) and the choroid (a layer of tissue that supplies oxygen and nutrients to the inner part of the eye). Before long, Dixon had lost 70 percent of her peripheral vision.
“Chronic inflammation in the choroid or retina damages these structures permanently,” says Ninani Kombo, MD, a uveitis specialist in the Department of Ophthalmology & Visual Science at Yale Medicine, who treats Dixon.
Oral steroids, the initial treatment for the condition, had unpleasant side effects, including a more than 60-pound weight gain, but they stopped the inflammation. Dixons’ vision was limited, but she could drive, ride horses and work. The one adjustment she made was leaving her restaurant job to work in wine retail. That way, she would not be at risk of bumping into people in a crowded restaurant and would not have to drive home late at night.
No good options
Dixon’s eye troubles flared up again in 2008, and this time the steroids were not as effective. “I lost my remaining peripheral vision in a few short weeks,” she says. Soon she stopped driving, started using a cane and adopted a yellow Labrador retriever guide dog named Guiding Eyes Elvis.
After seeing several specialists, Dixon started on a course of immunomodulatory steroid-sparing therapy—oral chemotherapy drugs meant to suppress her immune system, which would mitigate her uveitis symptoms without steroids’ sometimes harsh effects. (The dosage is far different from what a cancer patient might receive, says Dr. Kombo.)
But the fallout from this treatment was even worse. Vertigo, thyroid and adrenal gland problems and increased hair growth were a few of the side effects. “We kept trying all these different meds, and I didn’t tolerate any of them,” she says.
Dixon struggled to choose from among a few bad options. “I could go on a stronger chemo drug that would render me unable to work and be very sick,” she says, “or I could let the disease take my sight.”
One doctor told her about another option—Retisert, a grain-of-rice-size implant that time-releases steroids inside the eye for up to 30 months—and referred her to Yale Medicine. Dixon immediately made an appointment at the Yale Eye Center.
A scary train ride
Dixon’s Retisert surgery, performed by John Huang, MD, then a Yale Medicine physician (now in private practice in Connecticut), was successful, and she had three years without side effects. The steroids eliminated the inflammation, and Dixon adapted to life with limited vision.
She remained well enough to have another round of implants a few years later. But this time, a month after surgery, Dixon developed pressure and pain in her right eye. “I thought it was a migraine,” she says. That night Amy and Elvis were on a train, and she began to sense that “the rest of the world around me was black.” To test her vision, she looked at a poster on the train. “I went to close my left eye, and the poster was missing,” she says. “It was like I was 100 percent blind.”
Panicked, Dixon called her team at Yale Medicine, who urged her to come in immediately. She and Elvis got off at the next station and caught a train to New Haven. Once she arrived at Yale New Haven Hospital, Dixon received drops that reduced the pressure, and she went home for the night. But when she returned to Yale Eye Center the next day, her pressure was back up. The cause was glaucoma, a common side effect of Retisert.
The implications were so severe that a few days later, Dr. Huang surgically implanted an Ahmed glaucoma valve, a device that helps to relieve eye pressure. It was the first of several surgeries that Dixon would receive at Yale Medicine. Nearly six months after she received the Ahmed valve in her right eye, Dixon underwent a trabeculectomy, a procedure that created a small passageway so fluid in her left eye could drain.
“We were fighting really hard,” Dixon says.
There have been setbacks, and Dixon’s vision has deteriorated further. The hole in her left eye causes hypotony, or low eye pressure, which distorts her vision. Still, Dixon says her eyesight is “pretty good.”
Back in action
In 2013, about three years after she had stopped chemotherapy, Dixon got into a swimming pool for the first time in more than a decade. “I was tired of being overweight,” she says. “Everything hurt.” A friend had suggested that the enclosed space of an indoor swimming pool would be a safe way for someone with limited sight to exercise. “From there, I started hopping on a stationary bike,” Dixon says. “And then I started jogging, holding on to the treadmill and tying myself to the emergency brake.”
She shared her progress on social media, and one follower suggested that she try a triathlon, which combines swimming, biking and running. Intrigued, Dixon signed up to train with a local paralympic team.
“I love triathlon as a blind athlete because I compete with a guide, a sighted partner who acts as my eyes out on the course,” she says. “It’s like having a built-in coach and cheerleader beside you, as well as being your eyes.”
The support Dixon receives on the training course, she says, was similar to that she receives from Yale Medicine—in particular from the nursing staff, whom she describes as her “saving grace.”
“I was there once or twice a week. Now I go once a month,” she says. “I have become friends with all the nurses and general staff.” She appreciates that her doctors take time to explain things thoroughly. “I never leave with a question,” she says.
Dixon’s go-to doctor is Dr. Kombo, who began working as a uveitis specialist at Yale Medicine about a year ago. “She’s been a godsend,” Dixon says. “She’s very conservative in her approach—since I’ve had so many surgeries, she’s reluctant to go in there—but keeps a close eye on the uveitis.”
Dixon loves that all her Yale Medicine physicians studied under the renowned uveitis doctor Charles Steven Foster, MD, the founder of the Ocular Immunology and Uveitis Foundation. Recently, when Dixon had a disconcerting bleed, Dr. Kombo referred Dixon to Dr. Foster in Boston. He prescribed a new eye drop that brought her symptoms under control. “There’s no ego at Yale,” Dixon says. “It’s all about what is best for the patient.”
For now, that means trying a variety of strategies to treat the glaucoma—an ongoing risk, even while the uveitis is under control. The challenge with her condition, says Dixon, is that the uveitis makes the glaucoma worse, while the glaucoma makes the uveitis harder to treat.
“The scar tissue keeps coming back, which causes the valve to fail,” she says. Dixon recently had another surgery to clean out the scar tissue. Then she had another surgery because her eye pressure was too low, which is worse than too high. “The pressure was escaping too quickly,” she says. “The retina can fall off.”
‘It will be a battle’
The prognosis for someone such as Dixon is not good. “It will be a battle until I lose my vision,” she says. Still, she has cause for optimism. “I am the first patient with this combination of diseases to have good central vision in the middle still remaining after so many years of the disease,” she says. “So it could be a year, 10 years, who knows?”
“The cool thing is that technology is catching up to the disease,” she says. “Retisert didn’t even exist 10 years ago. Drugs are getting better. Surgeries are improving patient outcomes. I’m hoping someone finds a great way to manage uveitic glaucoma so I can see for years to come.”
She says she has a “dream team” at Yale Medicine.
“I could not have planned it better. Yale Medicine gave me a quality of life I never imagined,” she says. “My team at Yale and I are winning the fight together against my disease, despite every odd against us.”
As a member of the USA Paratriathlon National Team, Dixon has raced all over the world. Among the highlights: In 2015 she competed in Rotterdam, the Netherlands. Despite breaking her wrist on a training run, she placed fifth in the competition. She is now ranked No. 2 in the world in her sport.
Meanwhile, Dixon works as a patient advocate, helping to connect uveitis patients across the country with life-changing therapies through Glaucoma Eyes International, an organization she helped found.
“I saw a lot of patients losing their vision very quickly for a very treatable disease,” she says. “I want more patients to have access to the great treatment that I’ve got.”