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.

But before I report on that promising human study, I’d like to venture back in time and tell you how FFB funding of key research efforts made MERTK gene therapy possible. It’s also a story about the ubiquitous Royal College of Surgeons (RCS) rat — a rodent with a retinal degeneration that was, and continues to be, important to the study of retinal diseases.

In 1999, a Foundation-funded research team, which included Drs. Matt LaVail and Doug Vollrath, discovered that mutations in the gene MERTK caused retinal degeneration in the RCS rat. Subsequent Foundation-funded research revealed that MERTK mutations caused retinitis pigmentosa in humans. Dr. Vollrath, among other scientists, determined that MERTK played a critical role in the maintenance of photoreceptors, the cells that make vision possible.

Nearly 15 years later — with the disease culprit identified and extensive knowledge of how it causes vision loss made possible through additional FFB-funded research — a gene therapy for MERTK-linked retinitis pigmentosa is being evaluated in people. Six patients have been treated thus far, and they are doing relatively well.

While it is too early to make a definitive judgment about the treatment’s safety or efficacy, two patients have demonstrated some vision improvement, which is reason for cautious optimism.  While restoring vision would be great, even halting vision loss would be a good outcome. Time will ultimately reveal the treatment’s true potential.

While perusing the MERTK poster, I had the privilege of meeting Dr. Nicola Ghazi, an investigator on the clinical trial. I also got a chance to catch up with Dr. Vollrath. But most exciting for me was to see the two investigators meet for the first time. They were truly delighted to acknowledge one another for their contributions in advancing the MERTK research.

Well, I am off to more meetings, posters and presentations. At ARVO, you never know who you’ll run into and the great advances they’re making toward life-changing treatments and cures.

Pictured, above: Drs. Nicola Ghazi (left) and Doug Vollrath. 

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.

Years later — despite efforts to cure my retinal disease, first as a graduate student, then as a postdoctoral researcher focused on ocular pathology — I came to accept the truth that those words carried. I was slowly being forced to adapt to a world not designed for me, or for any visually impaired person. But the progressive nature of my disease, retinitis pigmentosa, meant that time was on my side. I had time to change how I do things, how I communicate, how I think.

In life, I want the same things most people want: a successful career doing something I enjoy, plenty of time to spend with my family, the chance to pursue personal goals and an opportunity to put a dent in the world. Despite what I once feared, losing vision has not only allowed me to live the life I truly want to live; it has also taught me to do so.

After completing my undergraduate studies in 2005, I announced to my parents that I was setting off on a four-month backpacking trip across Europe. When they emphasized the importance of getting a job, I pulled out my trump card — my failing eyesight — and explained that I could not wait to see the world until I was 65, or even 35, because I would not be able to see much of anything at that point.

So off I went, with their eventual blessing and encouragement. I later penned and published Backpack, a book detailing my adventures, realizing that writing a book — a life goal of mine — would be easier to do while I still had some useful vision.

The same sense of do-it-now urgency prompted me to start my first business — Journal Prep, an academic publication support company — in 2010. But from the very beginning, I found it nearly impossible to work in the business, especially in the role of editor. My disability forced me to spend more time working on the business, hiring other — sighted — people to do the things I could not, and enlisting the help of my brother, Kyle, to coordinate daily activities. This was unfamiliar territory for me, yet it also offered a valuable lesson in leadership and served as the driving force behind the business’ rapid growth.

In my other job, as an ocular pathology researcher, my colleagues and superiors do not expect me to do any of the hands-on experimentation. Rather, my role centers on project conceptualization. I get paid to learn and think about ways to cure blindness, and I cannot imagine a more fulfilling job.

My dependence on hearing rather than seeing has freed me from the 9-to-5-at-a-desk workday. With the help of my assistant, I can handle emails via Skype while taking a walk. I frequently set up phone calls with potential business partners and clients rather than engaging in multi-part email exchanges.

My assistant tracks, reads and records research papers in my field, uploading the MP3 files to a shared Google folder on a weekly basis. I listen to these articles on my iPod while commuting to and from work. This allows me to keep up-to-date on the latest scientific research – including projects funded and supported by FFB – and the most thought-provoking business ideas.

The benefits of impaired vision have extended well beyond the boundaries of work. I now read for pleasure more than I ever have by listening to audiobooks while at the gym. I play with my kids without being distracted by alerts on my smartphone, which I recently downgraded to a “regular” phone. And I habitually embrace challenges put forth by friends and family — running a half-marathon, learning to cook and writing this blog post, for example — in part because I choose to be the one who defines my limitations.

My vision has never been worse and my quality of life never better. While I firmly believe that vision research has hit a tipping point and that blindness will soon become something of the past, I am thankful for the paradigm shift that I have personally experienced as a consequence of my own affliction with a blinding eye disease.

Pictured, above: Shawn Maloney

Market approval of the Argus II is especially poignant because FFB funded an early stage of its development, and we’ve closely followed and reported on its development ever since. For these reasons, Foundation staff and board members were quoted, last week, in articles on the device in The New York Times and The Washington Post. Our offices were also paid a visit by Al Jazeera TV, which aired this piece over the weekend.

But the buzz extends even farther, with articles and posts showing up in numerous blogs and publications, including USA Today, Forbes, Popular Science, Bloomberg, The Wall Street Journal and the Los Angeles Times.

We congratulate Second Sight and are honored to have played a role in the Argus II’s development. And, as always, we look forward to reporting more good news about treatments and technological advances for restoring vision for people with  retinal diseases in the future.

Pictured, above: the Argus II implanted on the retina