What's it like to start high school worried that you're going blind? That's what Jake Fernandez faced. He had a severe case of an eye disease called keratoconus, which was complicated by a chronic infection. Standard treatments like eye glasses or special contact lenses weren't working.
Luckily, Jake was eligible for a new type of corneal transplant at the Jules Stein Eye Institute, University of California, Los Angeles.
Listen to Jake describing his vision with keratoconus (MP3, 1.9MB).
About one in every 2,000 people worldwide has keratoconus, which is a disorder of the cornea – the clear, outer surface of the eye that focuses incoming light. As the disease gets worse, the cornea becomes much thinner than normal and may bulge outward in a cone shape, making it impossible to see clearly. Usually both eyes are affected, though one may be worse than the other. In some people keratoconus slows down or stabilizes after 10 to 20 years, but in others the disease would eventually cause blindness if left untreated.
Symptoms of keratoconus often begin in patients' teens to early 20s. Vision may become blurry or distorted, or the person may become extra- sensitive to bright light. Ophthalmologists (Eye M.D.s) can now diagnose the disease more accurately than ever by using topographic scanners that "map" the corneal surface.
UCLA's Dr. Sophie Deng is the ophthalmologist who performed Jake's corneal transplant surgeries.
Listen to Dr. Deng discuss Jake's condition, the transplant procedure and her prognosis (MP3, 1.2 MB)
A recent Australian study of teenage patients who had corneal transplants to treat keratoconus found that they were very likely to regain and sustain good vision: 90 percent of them still had functioning corneas at their 10-year follow-up. This success rate is much higher than what occurs in adults. And of course, gaining decades of good vision has a huge impact on teenagers' education and quality of life.
Listen to Jake planning for a future with good vision (MP3, 1.9MB).
Whether patients are young people or adults, corneal transplants are usually only done for keratoconus after all other options have failed. A corneal transplant requires ongoing, lifelong medical follow-up, as Dr. Deng explains.
Listen to Dr. Deng discuss lifelong follow-up care after corneal transplantation (MP3, 750KB).
Dr. Deng used a newer corneal transplant procedure known as "DALK" to treat Jake. The main difference between DALK and a standard corneal transplant is related to a tissue layer of the cornea, called the endothelium. In DALK, the patient's endothelium layer is left in place when the diseased cornea is removed, and the donor cornea transplanted into the patient's eye does not include the donor's endothelium. Retaining the patient's own endothelium significantly reduces the risk that the donor cornea will be rejected. Though it is more difficult to perform than the transplant procedure commonly used today, Dr. Deng and other leading corneal surgeons think DALK will become the new standard. DALK transplants are likely to last an additional 10 years — up to 30 years in total.