SALT LAKE CITY — Utah firefighter Richard Rich and the doctor who treats him still aren't sure why he woke up 10 years ago with blurred vision in his left eye. It was, he says, "like looking through wax paper," and neither eyedrops nor the passage of time made his vision better.
When he sought medical care, Rich was diagnosed with keratoconus, a degenerative eye disease that could be genetic, or could be caused by something as simple as rubbing your eyes too much in childhood.
In people who have keratoconus, the outer layer of the eye, which is normally rounded, grows thin and bulges into a cone, distorting vision and causing extreme sensitivity to bright light. The condition affects about one in every 2,000 Americans, most often teenagers or young adults, according to the National Eye Institute.
For Rich, the diagnosis was not only a threat to his vision, but his career. Like all first responders, firefighters have to pass physical tests every year to remain on the job, and at his last physical, the doctor expressed concern about Rich's ability to continue to work if his vision continued to deteriorate.
Now, however, Rich is hoping that a procedure approved last year by the Food and Drug Administration has solved the problem. The treatment he had six months ago is called corneal cross-linking, and it involves the application of liquid vitamin B2 to the eye’s cornea, which is then exposed to ultraviolet light.
It’s the same treatment that late U.S. Olympic bobsledder Steven Holcomb, who grew up in Park City, had in 2008 and credits with saving his career.
When Holcomb had the procedure, corneal cross-linking was used “off-label” — meaning that the Food and Drug Administration had not approved its use in the treatment of keratoconus. The FDA’s approval in 2016 has expanded the number of doctors who offer it, and the number of patients willing to try it.
From concept to primary treatment
Since introduced in the 1990s, corneal cross-linking has grown from “an interesting concept to a primary treatment" for keratoconus, according to Dr. J. Bradley Randleman, a professor and director of the Section of Cornea, External Disease and Refractive Surgery at Emory Eye Institute, whose study on the procedure was published in the journal Survey of Ophthalmology.
Before its use became widespread, people with keratoconus were treated with a hard, uncomfortable contact lens (which feels like "putting a cereal bowl in your eye," Rich says), corneal implants or, in severe cases, a corneal transplant from an organ donor.
Corneal cross-linking is simpler and safer. A solution containing riboflavin — also known as Vitamin B2 — is applied to the cornea and soaks in for about 20 minutes, then the eye is exposed to ultraviolet light for about 10 minutes.
That may sound like a recipe for melanoma of the eye, but the riboflavin blocks the light from penetrating too deeply, said Dr. Darcy Wolsey at the Eye Institute of Utah, who has treated about 100 patients with corneal cross-linking.
The vitamin solution, combined with the light, enables the cornea to form new bonds (or cross links) in its collagen, and in some cases, causes the cornea to take more of its natural shape. The amount of improvement differs between patients, but in most cases the progression of disease slows or stops, and in some people, vision improves.
Wolsey, who has been performing the procedure for about five years, said doctors expect corneal cross-linking to be of most benefit to teenagers, since it can slow or halt the progression soon after diagnosis. Teens as young as 14 have been treated, she said.
For now, the treatment is only covered by a few large insurance companies; it takes a few years after FDA approval before coverage becomes common. Out-of-pocket, it usually costs between $2,500 and $3,000, she said.
Hands off your eyes
Six months after his treatment, Rich, who is 47 and has been a Salt Lake City firefighter for 23 years, has already seen a slight improvement in his left eye and is hopeful that keratoconus in his right eye, which was just beginning to show signs of the disease, has been stopped. Should it progress, his prospects of continuing to work grow bleaker.
“The human body is a great work of art; it does adapt very well,” he said. “At this point, I’ve been dealing with this long enough that I’ve learned to adapt, but if I get something in my right eye, I’m definitely at a disadvantage. I’m much more careful about the safety of my eyes than I was in my youth.”
As for the hypothesis that keratoconus occurs when the cornea is damaged by vigorous eye rubbing, several studies have shown an association, but there's no proof.
“I personally think you need some genetic factor there already and rubbing can make it worse,” Wolsey said.
Regardless, the cornea is the gate-keeper of the eye, blocking dirt, germs and debris that can hurt it, and helping us to focus. Keep eye rubbing to a minimum, and be aware that the cornea can be burned, just like your skin. A corneal flash burn, also known as ultraviolet keratitis, can result from sunlamps or the reflection of sunlight off water or snow, particularly at a high elevation.
If you absolutely have to rub your eyes, Dr. Mark Mifflin, a Salt Lake City ophthalmologist, said on the University of Utah's Health Science Radio that you should apply no more pressure than if you were gently washing your face or drying it with a towel.
"Anything more than that is probably unhealthy for the eye," Mifflin said.
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