We Orioles fans appreciate that Adam isn’t marveling at how well his eyes are tracking the ball during its quick, 300-foot journey. That, of course, might distract him from catching it.

But for many researchers fighting blindness, understanding the complex process of vision, and how the retina makes it possible, is their game.

Take, for example, Erika Ellis, a medical student at the University of California, San Diego, and Howard Hughes Medical Institute research fellow, who is receiving a one-year career development award from FFB to study retinal ganglion cells. Erika will be researching how these cells refine and package visual information and send it through the optic nerve to the brain, where the final images are created and interpreted.

While the process of seeing begins when photoreceptors convert light into electrical signals, it’s up to many other types of downstream retinal cells — including ganglion, amacrine and bipolar cells — to contextualize and enhance the signals so we can perceive motion, contrast, edges and boundaries and other visual details.

Researchers like Erika are particularly interested in how ganglion cells map to different regions of the brain. There are approximately one million axons — fibers in the optic nerve — connecting the retina’s ganglion cells to the brain, so the task is daunting. But documenting the brain-retina relationship will enable experts to better understand how they work together and how well emerging retinal treatments are restoring vision.

Ganglion cells are also an attractive target for vision-restoring treatments, because they survive long after photoreceptors degenerate from diseases like retinitis pigmentosa and macular degeneration. Emerging optogenetic therapies are designed to empower ganglion cells to respond to light, so they can function somewhat like photoreceptors and restore vision. While their research is at an early stage, it holds promise for people who have lost their photoreceptors to the most advanced retinal conditions.

If you are interested in learning more about ganglion cells, optogenetics and the Foundation’s diverse research portfolio, there’s still time to register for our VISIONS 2013 conference, taking place in Baltimore June 27-30. You’ll also get the opportunity to meet nearly 50  of the Foundation’s research all–stars.

And if you happen to be a baseball fan, the Yankees are also in town that weekend, playing at Camden Yards, right down the road from the conference hotel. Come root for Oriole standouts like Adam, Chris Davis, Manny Machado, Matt Wieters and Nick Markakis. The Yankees have some players as well, but I can’t recall who they are.

Pictured, top, Baltimore Orioles centerfielder, and Golden Glove recipient, Adam Jones; and, above, HHMI-FFB Medical Fellow Erika Ellis.

Retinal diseases may be incurable, at the moment, but, with the help of specialized training and technology, they are not unmanageable. In fact, a savvy patient working with a conscientious eye doctor can adjust relatively well to the various stages of vision loss — via, for instance, mobility or assistive-technology training. And in the case of wet age-related macular degeneration, or AMD, there are treatments that slow vision loss.

That makes the recent announcement that the FDA approved use of an iPhone test enabling those with AMD to monitor their eyesight at home a heartening one. Called myVisionTrack, the test was designed at the FFB-funded Retina Foundation of the Southwest by Dr. Yi-Zhong Wang. He co-founded the company Vital Art and Science, which developed the test and tried it out it in clinical trials last year.

The test displays three circles on the screen of an iPhone 4S, one of which is shaped differently from the other two. The user taps the odd circle, which calls up three more, only the difference is more subtle this time. This process continues until the user hits the wrong circle. The results are then stored and compared to previous tests’ results, which lets both patient and doctor — who receives the results remotely — know whether the former’s vision has gotten worse and may demand treatment.

Although this isn’t the first time the FDA has approved a smartphone-related monitoring system, it’s not a common occurrence, especially in the retina field. For various technical reasons, the FDA approved a test designed for use with the iPhone 4S only, but Vital Art and Science is working to make it compatible with other devices. The whole package is also prescription-only. There’s a long way to go on this, but the company believes that, once the test can be used on other phones, an app will qualify for over-the-counter status.

We at FFB are hopeful that myVisionTrack proves to be a useful tool for AMD patients. Early detection is essential for properly managing AMD. Technology that empowers someone who’s been diagnosed to monitor his or her own vision will help that person do all he or she needs to do, with help from a doctor, to live a healthy, fulfilling life.

The CNTF caused the cones to deconstruct, regenerate and come back even stronger, so that they were able to transiently provide day vision. Specifically, the cones grew new and more robust outer segments, the antennae-like projections that process light to make vision possible.

The Pennsylvania team came up with this innovative approach because it was having problems with a gene therapy it had developed for achromatopsia caused by mutations in the CNGB3 gene. The therapy worked well in younger dogs, but not in canines older than one year. However, the injection of CNTF prior to the gene therapy proved to be an effective solution to the problem.

You may be asking: Will this restorative approach work for rods, the cells that provide night and peripheral vision, or other diseases, such as retinitis pigmentosa (RP)? Can photoreceptor deconstruction and regeneration be a new cross-cutting therapeutic approach?

While these are distinct possibilities, much more research is needed to determine CNTF’s potential. Deconstructing a photoreceptor to resurrect it is still scientifically bold. Before we move this type of therapy into humans, we want to have a reasonable level of confidence that photoreceptors will be regenerated by the process. It is also important to demonstrate that cone restoration can last longer than it currently is.

Those of you who have been following Foundation-funded research may already be familiar with CNTF. It’s the therapeutic protein delivered by Neurotech’s encapsulated cell technology (ECT), a tiny implantable device the size of a pencil head. But the amount of protein diffused by the ECT is a relatively low dose that is thought to be only protective; it isn’t enough for cone destruction and regeneration.

Results from clinical trials of ECT suggested that it was slowing vision loss for people with dry age-related macular degeneration. It also appeared to have a beneficial effect on retinal health for people with inherited retinal diseases such as RP and Usher syndrome.

Human studies of ECT continue. The Foundation is funding an imaging study of ECT at the University of California, San Francisco, to better understand its true potential for saving cones and vision. The National Eye Institute is also conducting an ECT clinical trial, interestingly enough, for people with achromatopsia, but as a protective therapy.

As I’ve said in previous blog posts, science is always full of surprises, and CNTF is a prime example. Its potential applications — in both low and high doses — are very intriguing.

One final observation: Whoever came up with the adage, “You can’t teach an old dog new tricks,” never met a researcher funded by the Foundation.

Pictured, above: an image of a retina, courtesy of Dr. Nicolás Cuenca, University of Alicante.

I’ll always remember my first Foundation Fighting Blindness conference, when I heard Dr. Dean Bok, of the University of California, Los Angeles, discuss the retina’s design and how it worked. I was new to the field and just learning. Dr. Bok opened his presentation by saying that, as a student, he was “seduced” by the beauty of retinal science. By the end of his impassioned talk, I, too, was hooked.

And, now, I’m always excited to speak and write about this magical piece of tissue. Even though the best retinal researchers still don’t understand the science completely, I think everyone can appreciate the basics, including how to keep their retinas as healthy as possible.

How the Retina Works

So what does the retina do? In simple terms, it converts light into electrical signals that are sent through the optic nerve to the brain, where they’re interpreted as vision. Put another way, the retina is like film in a camera — for those of us old enough to remember when film had to be loaded into a camera.

The photoreceptors — a.k.a., rods and cones — are the elongated retinal cells (like antennae) that transform light into electricity through a complex biochemical process fueled by vitamin A. Rods provide night and peripheral vision. Cones, which are concentrated in the macula (central retina), enable people to perceive details, colors and objects. Approximately 125 million photoreceptors are packed into each human retina.

While the retina is small — it’s a circular tissue just 35 millimeters in diameter (a little bigger than a quarter) and half a millimeter thick — it’s a real workhorse. In fact, the retina processes more oxygen for its size than any other tissue or organ in the body, including the heart and lungs. Not only does it provide vision during waking hours, the tips of photoreceptors are shed, disposed and regenerated during sleep. For the retina, there’s always work to be done.

Inherited retinal diseases often originate in photoreceptors, but there are a number of other cell types in the retina that can be affected. These other cells may provide nutrition, waste disposal and image-processing.

Keeping the Retina Healthy

Whether or not you have a retinal disease, there are things you can do to keep the retina healthy and functioning optimally. As we always say at the Foundation, what’s good for your heart is good for your eyes.

First and foremost, don’t smoke. It is the most significant modifiable risk factor for age-related macular degeneration, and research has shown that just one cigarette’s worth of nicotine reduces retinal sensitivity.

Also, eat lots of colorful vegetables and fruits, which are rich in antioxidants, such as lutein and zeaxanthin. In fact, these antioxidants are present in the retina, especially in the macula. They help protect the retina from light damage and daily wear-and-tear.

Docosahexaenoic acid (DHA), a healthy fat, is another important nutrient that protects retinal cells and keeps them functioning well. DHA is abundant in coldwater fish, such as tuna, salmon, herring and sardines. It is also available in fish oil or vegetarian supplements.

Protecting your eyes from bright sunlight is also important. Always wear sunglasses that screen out UV rays and a wide-brimmed hat when in the sunshine.

And, finally — again, whether or not you have a retinal disease — see an eye doctor immediately if you experience sudden changes in vision. The sooner you get help, the better chance the problem can be resolved and vision saved or restored.

Conditions like wet age-related macular degeneration and retinal detachments can be treated.  And while a doctor can’t yet treat an inherited retinal disease, there are related complications, such as cystoids macular edema (swelling), which may be resolved with a medication.

For More Information

To learn more about the retina and emerging treatments, visit the Foundation’s website and return regularly to Eye on the Cure. Both sites are chock-full of articles and posts on the latest advancements. Also, feel free to ask questions in the comments section of each blog post or send them to info@fightblindness.org. It’s our pleasure to keep you informed.

Pictured, above: An image of a mouse retina captured by Dr. Luca Della Santina, University of Washington.

But you know what? I can no longer shop for groceries.

Before I explain why, I’d like to mention a book I wrote and published two years ago. It’s titled Eye Envy: Perspectives Into Vision Loss. And while it tells my story, it also features the stories of more than a dozen other people, of various ages and walks of life, affected by retinal diseases. They’re among the hundreds I’ve met in my travels worldwide, as a triathlete, coach, speaker and fundraiser for the Foundation Fighting Blindness. In fact, the proceeds from the book—which you can order, in print, on my website, or as an e-book—go to FFB.

I got involved with the Foundation almost 10 years ago, after I’d been diagnosed with cone-rod dystrophy at the age of 35. Before that, I knew that I had vision problems, but it wasn’t until I hooked up with Dr. Gerald Fishman, a Foundation-funded researcher and clinician, that I discovered the cause. Since then, I’ve dedicated not only my book sales to FFB, but I’ve co-hosted fundraising events in both Florida and Chicago with my parents, and I co-founded the Race to Cure Blindness program, through which athletes leverage their competitions to fundraise for FFB.

I wrote Eye Envy for a couple of reasons. In every way possible, I want to help FFB raise the considerable amounts of money necessary to fund the lab, pre-clinical and clinical-trial work aimed at providing people like myself with treatments and cures. My younger brother, Russell, has also been diagnosed with cone-rod dystrophy. And our paternal grandfather, once a larger-than-life Chicago attorney who loved driving his Cadillacs, playing golf and watching the Cubs and Bears — we watched him deflate in his later years, as the effects of age-related macular degeneration robbed him of his sight. He died just two months before I ran my first race for the Foundation, in 2005.

Now, I’m not married, and don’t have children, but I have five nieces and nephews. And these diseases I’m talking about — they’re hereditary. So, yes, in many ways I’m doing this for myself and my family.

But I also wrote Eye Envy because, over the past decade, I’ve met truly inspirational people who, despite their conditions, live fulfilling lives. And, like me, they once experienced the loneliness and despair that accompany a retinal-disease diagnosis. More than anything, you need to know you’re not alone, and my book is a means of offering the support so many of us need. And for the sighted community, it hopefully provides both inspiration and a glimpse of the low-vision world.

So, back to the groceries. When I wrote Eye Envy, I had some trouble with daily tasks, but not much. A few years earlier, in fact, I was able to drive. My vision, however, has deteriorated significantly in the last year alone. Even in optimal conditions, my vision is twice the legally blind limit.

So shopping in a grocery store — trying to get around others, seeing signs, reading labels — is no longer possible. An Ironman competition is easier for me to navigate than a Whole Foods. And because I live by myself, I have an assistant do it.

Food shopping is just one of many tasks that have become difficult for me. So I’m more determined than ever to help fund treatments and cures. But I also consider myself fortunate. Without this condition, I would not have met many of the people I have and made the same kinds of deep, meaningful connections.

I’m also confident enough in FFB’s abilities that I can imagine the following happening in my lifetime – an eye doctor, after delivering a diagnosis to a patient, saying, “OK, this is what you have. And this is what we’re going to be able to do for you.”