An Affliction Of The Cornea Gets A Closer Look Keratoconus, which afflicts an estimated one in 2,000 teens and young adults, causes the surface of the eye to gradually thin and bulge outward into a cone shape. The result: badly blurred vision. Now, surgeons are refining treatments like the corneal transplant and exploring newer, less-invasive ways to treat the disease.
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An Affliction Of The Cornea Gets A Closer Look

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An Affliction Of The Cornea Gets A Closer Look

An Affliction Of The Cornea Gets A Closer Look

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On a Monday morning, it's MORNING EDITION from NPR News. I'm Steve Inskeep. Renee Montagne is getting some well deserved time off. And Mary Louise Kelly is with us for the next three weeks.

Mary Louise, welcome back.


Thank you. Delighted to be here, keeping Renee's seat warm for her.

All right. We're going to do Your Health today. And we're going to be talking about two types of surgery�- breast implants and corneal transplants - and how patients fare after receiving those operations.

First, the debilitating eye disease that strikes young people and it affects about one in 2,000 Americans.�Teenagers and young adults with advanced cases of the disorder can face a life of near blindness.�But there are effective treatments, including a corneal transplant, as NPR's Patti Neighmond reports.

PATTI NEIGHMOND: Kaley Jones remembers the day vividly.�She was in her high school history class, just 17.��

Ms. KALEY JONES: On Friday afternoon in the class I had no problem reading the board. Everything was sharp and clear. I could see all my classmates. And come Monday morning, I went to go sit down in my class, I looked at the board and I couldn't see anything.

NEIGHMOND: Everything was blurry.�She could make out shapes, but not faces.

Ms. JONES: It was kind of like looking through plastic wrap. I could see color but no real detail.

NEIGHMOND: Kaley's doctor diagnosed a disease she'd never heard of -keratoconus. The cornea - the outer window of the eye -�becomes thin and changes shape. Instead of rounded, it becomes cone shaped.�It turned out Kaley had a particularly advanced case.�One of her corneas had thinned so much it ruptured, triggering her sudden blurred vision.��

When corneas are only a little misshapen,�glasses can help.�But many patients, like Kaley, need a hard contact lens to literally try to push the corneas back into place.��

Ms. JONES: We went through probably 20, 30, 40 pairs of contacts. I mean, just trial after trial, but because my corneas were so cone shaped it was kind of like trying to put a plate on a point, you know, on a peak. So they just kind of wobbled and they didn't fit.

NEIGHMOND: And that's what happens to one in five patients with keratoconus.�Dr. Tony Aldave is a corneal transplant surgeon�at UCLA's Jules Stein Eye Institute.

Dr. TONY ALDAVE (Surgeon): In Kaley's case, one eye was distorted enough that she needed to have a corneal transplant to restore clarity. In the other eye, she developed this condition where the inner layer of the cornea breaks. That typically results in corneal scarring, so now you have distortion of the cornea and scarring.

NEIGHMOND: Dr. Aldave performed Kaley's transplant just seven months after her diagnosis.�

Ms. JONES: Immediately, the day after, I could see shapes, I could see detail, faces. It was insane. I mean it was just incredible.

NEIGHMOND: She could do things she hadn't been able to do for months:�drive, read, see a billboard or a white board. And follow her passion - photography. Eventually, she got a transplant in her other eye too.� �

The new corneas don't guarantee�20/20 vision. But today, with the help of contacts, Kaley has nearly that.�There is one hitch - the average transplant lasts only 20 to 30 years and Kaley is just 24 years old.�Dr. Aldave.�

Dr. ALDAVE: In Kaley's case, my hope is that she can get at least 30 years out of each of these corneas, because they were very good donor tissue and she's had an uncomplicated course after surgery. And with newer techniques of corneal transplant surgery, if her cornea does need to be replaced in 30 years, I may be able to replace just the inside layer of the cornea, and again, allowing her to have a quicker vision recovery. And that'll hopefully give her another 30 years after that.

NEIGHMOND: And there aren't many risks, he says, beyond those of any surgery. The body is much less likely to reject a donor cornea than it is other organs like, say a kidney or a liver. And its success rate is high.�Dr. Aldave says�95 percent of patients see dramatic improvement. Still, it's always best to avoid surgery if you can.��

Dr. Yaron Rabinowitz directs Eye Research at Cedars Sinai Medical Center.�And he's looking into a procedure that could eliminate the need for transplant, if the disorder's caught early. It combines the vitamin riboflavin with UV light to work a little magic on the stuff cornea's are made of.�

Dr. YARON RABINOWITZ (Cedars Sinai Medical Center): The interaction between the UV light and the vitamin creates a reaction which causes the collagen fibers in the cornea to stick to each other, and in the process, it stiffens the cornea.

NEIGHMOND: And stops the disease.�So, how do you know if you have keratoconus? If you've been finding you need to switch the prescription of your glasses or contacts every few months because your eyes are changing so fast, then you should see a doctor. You may have to have the shape of your eyes examined.

Patti Neighmond, NPR News.

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