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.

Want to know about gene therapy? Our playlist features four videos, one each from four renowned researchers, including Dr. Eric Pierce, chairman of FFB’s Scientific Advisory Board, who does a great job summing up the vital role research plays in finding treatments and cures:

We also offer playlists covering stem cell therapy and gene discovery. And you won’t want to miss the video on why making donations to FFB’s research is so important at this crucial time:

To view all of the Foundation’s playlists, click here.

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.

Living in the Philippines at the time, we drove to Clark Air Force Base, where the doctor gave the tentative diagnosis of retinitis pigmentosa. I then went back to the United States with Dad – a retired New York City police detective working in security abroad – and the doctors gave the no-doubts confirmation of Usher syndrome. My parents were devastated.

I don’t know if it was denial as a form of coping, but I was determined to live my life to the fullest. With support from my parents and close friends, I learned to constantly adapt to different countries, cultures, religions, schools and languages. It made me resilient and tenacious.

I returned with my family to the States in the early 1970s, when the RP Foundation was just getting started. My parents became strong advocates for what is now the Foundation Fighting Blindness while searching the world for a cure. Meanwhile, I entered my third high school, where the students thought I was aloof because I couldn’t see or hear many of their greetings.

In college, I earned a joint bachelor’s degree in international relations and economics, and then began working for the federal government. My parents encouraged me to do so, as this was before enactment of the Americans with Disabilities Act (ADA) and they worried about job security. But what I struggled with most was the uncertainty of when I would lose my vision. During meetings, my mind wandering, I’d ask myself questions like, “Do I renew the newspaper subscription for a year or six months?”

Then I lost both of my parents at a relatively young age. Shortly before my dad died, I promised him I would be OK. I’ve tried to live up to that promise ever since.

In my late thirties, I made one of the hardest, and best, decisions of my life: I got a guide dog. His name was Beau, and he renewed my sense of independence. No longer did I bump into people or objects. We even made history together when, in 1997, we were denied access to the Senate floor. My boss, Senator Ron Wyden, claimed this was a violation of the intent of the ADA. A public outcry and national headlines ensued, and, thanks to a rule change, Beau and I soon found ourselves on the Senate floor.

In my forties, I married a wonderful man who encouraged me to go back to school. A couple years later, I exited Harvard University with a master’s degree in public administration, another dream come true. But then I rapidly lost the remainder of my vision. During a tour of Glacier National Park, I realized I could no longer see and found myself crying, grieving for my loss. Work became a struggle, and I suffered from anxiety and depression.

But, eventually, that passed, as I tallied the things I can be thankful for – a loving husband, great friends, a trustworthy guide dog and good health. Another is my ongoing involvement with FFB. Now on its board of directors, I see the inroads being made into research – gene therapy, retinal chips, stem cell technology and more.

I believe, however, that without additional federal funding for Usher syndrome and other retinal diseases, finding cures will be delayed. I encourage everyone to contact their representatives in Congress and let them know that federal funding for medical research should be a priority.

And if you have a retinal disease, empower yourself. Take advantage of tools such as a cane, a guide dog and the latest assistive technologies. Stay ahead of the continuous cycle of lost vision by getting orientation and mobility training, even before you need it. And whenever you find yourself thinking “I cannot do this,” do it.

The challenges never end. Last year, I had the opportunity to travel to Dubai on business, which, because of the long flight, I had to do without my current dog guide, Finnegan. As fearful as I was, I knew that if I didn’t go, I’d close the door on a great experience – my first visit to the Middle East. So with a cane, and relying on strangers, I successfully navigated the trip, from beginning to end.

Pictured, above: the author, Moira Shea, with her guide dog, Finnegan.

Moira M. Shea recently retired from the U.S. Department of Health and Human Services, where she was a senior policy analyst in the office of the Assistant Secretary for Preparedness and Response. Previously, she held a number of government posts, including Congressional aid and as an economist specializing in international trade and technology development. She’s been involved with the Foundation Fighting Blindness since 1980, and is currently a member of its board of directors.

What’s signficant about many emerging neuroprotective therapies for the retina is that they have the potential to treat a wide range of diseases, regardless of the genetic mutation causing vision loss. With about 200 genes linked to retinal diseases, this is a huge plus. They might also be used in conjunction with a genetic therapy to enhance its vision-saving effects.

Though neuroprotection usually isn’t addressing the root cause of the disease, it may be nearly as beneficial as a permanent fix, if useful vision can be saved for a person’s lifetime.

Neuroprotective treatments are being developed in many forms — proteins, drugs (small molecules) and nutrients. And there are several ways to get a neuroprotective therapy to the retina, including eye drops and oral medications.

Imagine having a therapy factory in the retina — in the forms of transplanted cells or genes — that provides continual release of neuroprotective proteins. Well, researchers are working on those, too.

Here are three examples of emerging neuroprotective therapies for the retina:

Protecting Mitochondria — MitoChem Therapeutics, a Foundation-funded  start-up company, has identified two compounds, which show potential for saving vision for people affected by several retinal diseases, including retinitis pigmentosa (RP), cone-rod dystrophy, Bardet-Biedl syndrome, Usher syndrome, Stargardt disease and age-related macular degeneration (AMD).

The compounds work by protecting mitochondria, the power supplies for all cells in the body, including those in the retina. Investigators have refined the compounds to the point where they save virtually all of the photoreceptors in a mouse model of retinal degeneration. They’ve also demonstrated that eye drops can effectively deliver large amounts of one compound to retinas in eyes comparable in size to those in humans. Eye drops are beneficial, because they minimize potential systemic side effects.

Docosahexaenoic Acid (DHA) — DHA is an important structural component of cells in the brain and the retina. It appears to have many neuroprotective properties and is prescribed for a variety of conditions, including heart disease. Perhaps most relevant to retinal degenerative diseases, DHA can reduce the destructive effects of inflammation and oxidative stress. It also helps maintain the structure and metabolism of photoreceptors.

The Retina Foundation of the Southwest is completing a Foundation-funded clinical study of DHA for males with X-linked RP. Previous clinical studies conducted by Dr. Eliot Berson show that vitamin A combined with DHA can slow vision loss in people with RP. In 2014, the National Eye Institute will complete a clinical study of DHA and other nutrients for people at risk of advanced AMD. DHA can be obtained by eating salmon, tuna and other coldwater fish, or by taking fish-oil or algae supplements.

Rod-Derived Cone Viability Factor (RdCVF) — Drs. José Sahel and Thierry Léveillard, of the Institut de la Vision in Paris, received the Foundation’s Trustee Award for their discovery of Rod-derived Cone Viability Factor (RdCVF), a protein that preserves and rescues cones, the cells in the retina that provide central and color vision. They are now developing a gene therapy that provides sustained delivery of RdCVF after a single treatment. The protein could be beneficial to people affected by a broad range of retinal diseases, including several forms of retinitis pigmentosa. With Foundation funding, the researchers are working to move their RdCVF gene therapy into a clinical trial.

There are several other emerging neuroprotective therapies in the Foundation’s research portfolio. Stay tuned to Eye on the Cure and the Foundation’s website for updates on these emerging cross-cutting treatments.

Pictured, above: Some neuroprotective therapies may be delivered by eye drops. (Photo courtesy of the National Eye Institute.)