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Saving A Skater's Vision

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BOSTON, Mass. ( Ivanhoe Newswire) - While the summer Olympics start this month, the athlete you're about to meet is already training for the 2018 winter games. But an eye disease threatens to keep him from competing.

"I really just like the feeling of flying and spinning and jumping in the air and rotating as fast as you can," Adrian Huertas, told Ivanhoe. "I just think it's an amazing feeling and it is so much fun."

Figure skater Adrian Huertas has his sights set on the 2018 Olympics.

"I would really like to be part of the U.S. team," Adrian said.

But a problem with his vision could ice those dreams.

"About three years ago, I told my mom my eye was blurry and I just thought it was blurry in one eye, but it turned out to be more than that," Adrian said.

Adrian's eye doctor diagnosed him with Keratoconus, a common eye disease affecting one in two-thousand people.

"In Keratoconus the cornea becomes misshapen so that the light rays aren't focused," Kathryn Colby, M.D, PhD, a cornea surgeon at Mass. Eye and Ear and an associate professor of ophthalmology at Harvard Medical School, said.

Sometimes it can lead to blindness. The only option used to be a corneal transplant, but now as part of a clinical trial at Massachusetts Eye & Ear, Adrian tries a new treatment to save his sight called collagen cross linking.

"What this does is strengthen the structural proteins of the cornea," Dr. Colby said.

Doctors first soak the eye with the b-vitamin riboflavin. Then they apply UV light. The combination forms the cross links or bonds that strengthen the collagen molecules and stabilize the cornea.

"In Europe it's standard of care for Keratoconus," Dr. Colby said.

In a three year trial in Italy, the procedure improved vision in almost 70 percent of patients. Those are promising stats for Adrian as he eyes Olympic glory.

"Hopefully I can make it and be good enough to do it," Adrian said.

Collagen cross-linking still needs FDA approval in the states. Doctors aren't sure when that will happen. Keratoconus currently has no approved treatments in the U.S. and accounts for 15 percent of the country's corneal transplants.

RESEARCH SUMMARY

BACKGROUND: Keratoconus, also known as KC is a non-inflammatory eye condition in which the normally round dome-shaped corneas become progressively thin, causing a cone-like bulge to develop. This causes distortion and reduced vision. It has been estimated to occur in 1 out of every 2,000 people. Keratoconus is generally first diagnosed in young people at puberty or in their late teens. (Source: http://www.nkcf.org)

WHAT CAUSES KC: The exact cause of keratoconus is unknown. There are many theories based on research and its association with other conditions. However, no one theory explains it all. It is believed that genetics, the environment and the endocrine system all play a role in keratoconus.

SYMPTOMS: In its earliest stages, keratoconus causes slight blurring and distortion of vision and increased sensitivity to light. These symptoms usually first appear in the late teens and early twenties. Keratoconus may progress for 10-20 years and then slow or stabilize. Each eye may be affected differently. Most people who develop Keratoconus start out nearsighted. The nearsightedness tends to become worse over time. (Source: http://www.nkcf.org)

It can usually be diagnosed with slit-lamp examination of the cornea. The most accurate test is called corneal topography, which creates a map of the curve of the cornea. When keratoconus is advanced, the cornea will be thinner at the point of the cone. This can be measured with a painless test called pachymetry. (Source: http://www.ncbi.nlm.nih.gov)

TREATMENT: In the early stages, eyeglasses or soft contact lenses may be used to correct the mild nearsightedness and astigmatism caused in the early stages of keratoconus. As the disorder progresses and the cornea continues to thin and change shape, rigid gas permeable (RGP) contact lenses are generally prescribed to correct vision more adequately. The contact lenses must be carefully fitted, and frequent checkups and lens changes may be needed to achieve and maintain good vision. (Source: http://www.nkcf.org)

NEW TECHNOLOGY: Because numerous visits to the doctor to constantly have contacts and glasses prescriptions modified can be exhausting and not to mention frustrating, a new surgery is undergoing clinical trials at Massachusetts Eye & Ear. It's called Corneal Collagen Crosslinking (CXL). CXL works by increasing collagen crosslinks which are the natural "anchors" within the cornea. These anchors are responsible for preventing the cornea from bulging out and becoming steep and irregular. During the corneal crosslinking treatment, custom-made riboflavin drops saturate the cornea, which is then activated by ultraviolet light. This process increases the amount of collagen cross-linking in the cornea and strengthens it.(Source: http://www.nkcf.org)

Collagen crosslinking is not a cure for keratoconus. The aim of this treatment is to halt progression of keratoconus, and thereby prevent further deterioration in vision and the need for corneal transplantation. Glasses or contact lenses will still be needed following the cross-linking treatment, although a change in the prescription may be required, but it is hoped that it could limit further deterioration of vision.(Source: http://www.nkcf.org)

While crosslinking is used in virtually every country around the world, it is still in the FDA approval process in the United States. (Source: http://www.allaboutvision.com)

INTERVIEW

Kathryn Colby, MD, PhD, Cornea Surgeon, Mass. Eye and Ear, Associate Professor of Ophthalmology at Harvard Medical School, talks about a new treatment that could save people's sight.

What is keratoconus?

Dr. Colby: Keratoconus is a shape problem with the cornea, which is the clear front window of the eye. What the cornea does is focus light rays so we can have clear vision. In keratoconus, the cornea becomes misshapen so that the light rays aren't focused;people with keratoconus have blurry vision. It's a very common disease, about one in two thousand Americans have some form of keratoconus.

Are there different types of keratoconus?

Dr. Colby: There are different severities of keratoconus. It's all basically the same condition but some people might have just mild keratoconus where they can see pretty well with glasses and better with contact lenses. Then some people have more advanced disease where they need corneal surgery or other treatments in order to see.

So right now there's no cure for it?

Dr. Colby: There is no cure, no.

But there is a clinical trial going on?

Dr. Colby: Yes. We have a treatment called collagen cross linking. What this does is strengthen the structural proteins of the cornea. It's been done in Europe for about the last fifteen years. It is not FDA-approved in the United States yet, so we can only offer it to patients as part of a clinical study.

It's showing fantastic results right?

Dr. Colby: In Europe it's standard of care for people with keratoconus.

Does it halt the progression?

Dr. Colby: It halts the progression of the keratoconus in most cases. The earlier you get someone to get it done, the less they would have already progressed and so you could halt them at that point. In Europe they're doing all kinds of novel things that we can't even think of doing here in the US. One example is fixing the cornea with the laser and then cross linking it to make it more stable. Hopefully in the future we'll be able to do things like that but for right now, at least as part of a trial, we can offer the collagen cross linking and prevent people from getting worse.

How does it work?

Dr. Colby: Believe it or not, the exact mechanism is still somewhat of a mystery. We soak the cornea with riboflavin and then we apply UV light. The combination of the riboflavin and the UV light forms the cross links or the bonds between the collagen molecules, the structural proteins of the cornea, to prevent the bowing forward of the cornea from worsening.

It helps to strengthen that collagen?

Dr. Colby: Exactly. The analogy I like to use with patients is if you've ever seen a skyscraper going up, they put the steel beams and then they put the cross beams between the steel beams. The cross-links basically act to strengthen the structural proteins of the cornea.

It sounds like a pretty simple treatment too?

Dr. Colby: Like anything in ophthalmology you do have to have a certain skill level in order to be able to do it and prevent problems. But it really is a very straight forward treatment for the amount of benefit that is possible.

What are typical results and once they have the treatment what can they expect?

Dr. Colby: What we like to tell patients is that the goal is to prevent progression of the disease. For someone who already has keratoconus and who receives the cross-linking treatment, five years later their keratoconus should not be any worse. In the results from Europe, sometimes people actually do get a little bit better vision but that's not how we like to portray it to the people who are undergoing the trial.

This has to be very gratifying work for you correct?

Dr. Colby: It is and I don't know if you're aware but the hospital and I and one of my partners who is also participating in the trial are doing this procedure for free. It's not FDA approved so we don't really feel like it's appropriate for us to be charging patients (nor does the FDA). We are committed to being able to offer this to our patients, especially those in their teenage years, which is the group that tends to progress more rapidly. We're all committed to being able to do it as part of our trial.

Is this open nationwide?

Dr. Colby: It's a multi-center trial and other groups are participating in the trial. I would say most of them are actually charging patients to be involved in this study but again we feel like it's not an approved treatment so we really want to offer it but not have the patients have to pay for it.

Can you talk about Adrian?

Dr. Colby: I've known Adrian for a few years now and he and his mother are both very astute observers of his condition, so he had noticed things. We put him in a contact lens a few years ago, which is the first line treatment for this condition. He does okay with the contact lens but there was evidence that the keratoconus in one of his eyes was getting worse. Especially given his hope to someday skate in the Olympics, it's a nice opportunity to be able to offer him the ability to stabilize his vision where it is so it doesn't get any worse.

What would be the state of their care here now if you do have keratoconus and you're starting to go down that line?

Dr. Colby: We don't really have any treatment. We encourage patients to not rub the eye because we do know that rubbing the eyes can make the weakening of the cornea frequently. There are a number of different types of contact lenses that we can use for people with keratoconus and the vast majority do fine with a contact lens of one sort or another. I don't know if you wear contact lenses but they can be a nuisance and they are difficult to deal with. Then there are a few other types of procedures that are done. One is putting rings within the cornea. We generally haven't had dramatic improvement in patients after doing that treatment. And then finally the last treatment is replacement of the cornea in one way or another.

What would be the pitfalls of that?

Dr. Colby: The only way that we replace the cornea now is from a donor cornea from another person whose died and graciously donated their eyes to the organ bank. It's a replacement of a person's cornea with a donor cornea. They do very well but it is somebody else's cornea in your own eye and you have to use eye drops to prevent rejection of the transplant. If we can avoid having to do those transplants that would be even better.

FOR MORE INFORMATION, PLEASE CONTACT:

Mary Leach
Media Relations
Harvard Medical School
Mary_Leach@meei.harvard.edu

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