For those of us on the front lines of fighting blindness — whether we’re raising funds, conducting  research or stand to personally benefit from the results — the device’s approval is one of the most exciting milestones in vision restoration ever achieved.

The Argus II provides only rudimentary vision; users have recognized doorways, sidewalks, flatware on a table and lights on a city skyline. But the device is enabling people who were completely blind to see. That translates into improved mobility, independence and quality of life

The Argus II’s ability to restore some basic vision is remarkable, but people want to see much more of the world — faces of loved ones, text on a computer screen, paintings in museums and so on. A device that provides the ability to perceive more detail will not only help those without any eyesight; it will also benefit many people with varying degrees of vision loss.

Developing such a device is the next challenge for companies like Second Sight. While the Argus II is equipped with a 60-electrode, or –pixel, chip implanted in the retina, the California-based company plans to develop a 256-electrode prosthesis. But it is still several years away from making it available.

There are several other companies in the artificial retina arena, with Retina Implant AG, of Germany, being the furthest along. Its device, the Alpha IMS, is a 1,500-pixel chip and, unlike the Argus II, does not require an external video camera. Approximately 36 people have used the Alpha IMS in clinical trials. Some have recognized objects, such as doorknobs and telephones. One person saw the movements of a wild goose. Another read store signs.

The company’s clinical trial is underway in Germany, Hong Kong and the United Kingdom, and it is planning to extend the study to Wills Eye Institute in Philadelphia. Retina Implant AG is working to overcome some technical and surgical challenges, and has not indicated when they expect to apply for regulatory approval in the United States or Europe.

A research group from Stanford University is developing a 5,000-pixel system known as the Photovoltaic Retinal Prosthesis, which it believes will provide more detailed vision than the alternatives. The group is hoping to launch a clinical trial within two years. With this device, images are captured by a tiny video camera mounted on a pair of glasses and converted to near-infrared (NIR) light. The NIR beam is routed to multiple one-millimeter chips implanted underneath the recipient’s retina and converted to electrical signals that are sent back to the brain through the optic nerve.

Other groups developing artificial retinas include: Bionic Vision Australia, Nano Retina in Israel, the Boston Retina Implant Project and several organizations in Japan. This is an active and innovative field, though developing this cutting-edge technology is no small feat. Stay tuned — I’ll report on the progress of these efforts as it is made.

Editor’s note: The photos above, which appeared in the spring 2013 issue of In Focus, the Foundation’s newsletter, were incorrectly described as being Second Sight’s Argus II device. They are Retina Implant AG’s Alpha IMS.

Photos provided by Retina Implant AG

One reason that retinal degenerations are so devastating is that, once photoreceptors are lost, they don’t grow back. As you may know, the Foundation is funding a number of promising projects to replace lost retinal cells with those derived from a number of sources, including a patient’s own skin or blood.

But a potentially more elegant approach to overcoming retinal degenerative diseases would be to stimulate the patient’s retina to regenerate its own photoreceptors. In some animals — namely, fish and amphibians — the retina can regenerate. Unfortunately, such is not the case in people — at least not yet.

But there’s hope for retinal regeneration for humans, thanks to Foundation-funded researcher Dr. Thomas Reh, who has reported progress on this treatment approach at a couple of ARVO sessions. Dr. Reh is investigating how to derive new photoreceptors from retinal cells called Muller glia. In the developed retina, Muller glia provide architectural support and a number of protective and waste-disposal functions.

But Dr. Reh has shown that Muller glia can be reprogrammed in a dish to become neurons — cells that are closely related to photoreceptors. Much more work remains, but if he can derive photoreceptors from Muller glia in a mammal such as a mouse, it would be a major step toward making retinal regeneration possible in humans.

A key benefit of a regenerative treatment is that researchers don’t have to worry about getting transplanted cells to functionally integrate with the patient’s exiting retina. That’s a big hurdle at the moment. Regeneration would also eliminate the concern of potential immune reactions that might come from newly introduced photoreceptors.

At this stage of the game, retinal regeneration is definitely an audacious goal; unlike stem cell therapies now in clinical trials, regeneration is several years away from becoming a reality. But we have to keep thinking big. Just 15 years ago, we had no idea that we could even grow new retinal tissue from stem cells. But we have come a long way since then.

Finally, please keep in mind that the ARVO meeting is just a snapshot of where the research is today. We are making new advancements all the time, and our progress is accelerating like never before. Is it fast enough? Absolutely not. But we have the audacity to keep urgently driving the research until everyone can see.

Pictured, above: Dr. Thomas Reh 

Thank you for reading Dr. Rose’s daily ARVO updates.  Since this is the first time the Foundation has provided daily updates in this format, we would like to get some feedback from you.  Please take a moment to fill out a short, two-question survey.  Thank you for reading and for your support of the Foundation.

One of the optogenetics posters at this year’s Association for Research in Vision and Ophthalmology (ARVO) conference features a canine study conducted by a Foundation-funded collaboration. I had the pleasure of getting a presentation of the poster from Keirnan Willett, a young member of that team from the University of Pennsylvania. The group also includes Drs. Jose Sahel, of the Institut de la Vision, and Botond Roska, of the Friedrich Miescher Institute.

Keirnan explained that the treatment is a gene therapy designed to reactivate dormant cones by making them light-sensitive. The treatment leads to the sustained production of halorhodopsin, a light-sensitive protein. The research is still at an early stage; three dogs with retinal diseases — two with retinitis pigmentosa and one with cone-rod dystrophy — have been treated thus far.

Keirnan said there were no signs of inflammation or infection, which is a critical outcome at this juncture.  There was a suggestion that the dogs could navigate a maze better after treatment, but more dogs need to studied, and additional measures of vision improvement need to be taken.

Among the other interesting projects on optogenetics reviewed at ARVO was LambdaVision’s subretinal chip, which provides sustained release of light-sensitive proteins, and a drug therapy from the Foundation-funded Kramer Lab, at the University of California, Berkeley, which restores light sensitivity to a degenerating retina. These, too, are in early lab studies, but are promising projects.

We have not made the white cane obsolete just yet, but thanks to therapies like optogenetics, we are making good progress toward that goal.

Pictured, above: Keirnan Willett.

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. 

For more than a year, the NEI has focused significant resources on establishing what it calls an “audacious goal” to fundamentally change the game in vision research and eye care. With so many great breakthroughs occurring in a variety of eye research areas, the NEI recognized it was the ideal time to consolidate energies of the vision science community and present a challenging goal that NEI Director Dr. Paul Sieving said is “big, important and inspiring.” The development of the initiative included review of more than 500 suggested research goals, which were read by more than 80 experts.

I am delighted to report that the NEI’s unveiling of its audacious goal at the annual meeting of the Association for Research in Vision and Ophthalmology is an enormous boost and affirmation for those of us on the front lines of fighting retinal degenerative diseases. The goal, “to regenerate the neurons and neural connections in the eye and visual system,” is exactly what people with retinal diseases need to save and restore their vision. And most of the cutting-edge technologies that will get us to the goal — including gene therapies and stem cells — are exactly what the Foundation Fighting Blindness has taken a leadership role in supporting for the last four decades.

When you look back at the biggest breakthroughs made in retinal research — the identification of the first retinitis pigmentosa gene, the proof that gene therapy can restore vision in humans, and making new photoreceptors from stem cells — most were made by visionary researchers funded by the Foundation. We empowered those scientists to take the bold risks that held the potential to save and restore vision. Many times, those risks led to big advances and dramatically changed the retinal research landscape.

It took audacity for Gordon Gund and the late Ben Berman to establish the Foundation in 1971. But they knew no one would do it for them. Everyone who has joined the Foundation’s family since — researchers, donors and volunteers alike — is audacious in their own way. Whether you are forming a VisionWalk team, organizing a chapter meeting or donating money, you are stepping out of your comfort zone to make a difference. You know the work isn’t always easy, but you also know that no one will do it for you.

We at the Foundation applaud the NEI for its audacious goal and look forward to hearing more about the details to achieve it. The initiative holds great potential for helping people with retinal degenerations. The Foundation stands ready to help the vision community achieve this goal by continuing to drive the cutting-edge research that will deliver the preventions, treatments and cures available to the millions who need it.

In the meantime, don’t forget to be audacious. After all, you are part of the Foundation Fighting Blindness.

Pictured Above: Paul A. Sieving, M.D., Ph.D., Director of the National Eye Institute