Last Friday, my sister Liz and I went to the Day of Science conference by the Foundation Fighting Blindness (FFB) in San Diego. We were in town anyway for our father’s birthday the day before, so this event was a welcome opportunity to find out the latest research in retinal degenerative diseases. We both have Usher syndrome, a genetic condition that marries deafness to retinitis pigmentosa.

The Foundation Fighting Blindness is a research-focused philanthropic organization that my family has been very involved with for many years. They’ve raised millions of dollars (between 14 and 19 million dollars in 2007!), almost all of which goes right to researchers and clinics across the country and the world. This investment in retinal degenerative diseases (retinitis pigmentosa, Usher syndrome, macular degeneration, and glaucoma among others) is paying off–there are a few treatments in FDA-approved Phase I and II trials, and a dozen more treatment strategies are poised to make the leap from animal models into human trials. Other researchers are close to unlocking the secret of protein structures and processes within the photoreceptor cells in the retina–the very cells that make us see–and whose slow deaths lead to progressive blindness in millions of Americans.

These are 21st century, Age of Biology cutting-edge stuff: gene therapy, stem cell research, nanotechnology. Tiny little capsules inside the eyeball delivering life-saving proteins to photoreceptor cells. Subretinal injections of embryonic stem cells into the retinal pigment epithelium layer. Flooding the photoreceptor layer with sixty trillion “fixed” DNA so these photoreceptors adopt the correct genes for normal function. By the end of the day, Liz and I had learned enough about retinal function, amino acids, and protein types to walk away with master’s degrees in biology.

There are sight-saving technologies being tested on humans right now. Real treatments and cures are not far off. Barring worldwide collapse of civilization due to either climate change or peak oil (or both), I know that, because of these treatments, I will not go completely blind, and even perhaps my full sight will be restored one day. It’s exciting news, and it makes me happy.

About 20,000 people in the United States have Usher syndrome. It is the leading cause of deaf-blindness–3% to 6% of all deaf people have Usher syndrome, and probably around 90% of all Deafblind people have Usher. There are large networks out there supporting Deaf-Blind people. These include organizations such as American Association of the Deaf-Blind and the Helen Keller National Center, social services programs like Lighthouse International, hearing and deaf interpreters trained in tactile sign, or the Deaf-Blind mecca, Seattle, Washington.

In a world where there are sight-restoring treatments for people with Usher, what happens to these networks and organizations? It’s an interesting scenario to consider: the loss of jobs, knowledge, and community as human ingenuity declares victory over a genetic disease.

Unlike the majority of Deaf people who refuse any treatments for their deafness, I have a hunch that most people with Usher are definitely going to take advantage of sight-saving and sight-restoring treatments as soon as they become available. Hell, I would.

Here, some of you will start making comparisons to the Deaf conundrum. Would Deaf people want to be cured? Absolutely not. So shouldn’t it be the same for Deaf-Blind people?

Not really. I’m not admitting that I’m less of a person because I have Usher. But at the same time, being Deaf means you have access to a wonderful, beautiful community with an incredibly fun language. Becoming Deaf-Blind means you lose access to much of that. Remember, most Deaf-Blind people–and everybody with Usher–were Deaf first for decades.

Being Deaf-Blind is an endless cycle of loss. There’s the loss of feeling safe on your own two feet at night, the loss of independence on the highway as you give up your driver’s license, the loss of friends who are unable to adapt to your new visual limitations, the loss of spontaneity for ordinary errands as support service providers (SSPs) must be requested in advance, the loss of confidence in your future because you see less and less as time goes on. And throughout it all, you struggle to maintain an outward veneer of strength and bravery–”I can overcome this! I still can do whatever I want except hear and see!”–while this process tears your insides into pieces every day.

Not to say it’s all bad. There’s a lot of fun and freedom in having Usher, too, and while I haven’t had much experience with the Deaf-Blind community, others say it’s amazing in there with all the inside jokes, close tactile contact, and finding joy in simple daily victories.

But would most of these people in the Deaf-Blind community give it all up so they could see again? I think so. And I think that’s okay. And I don’t think it means that most Deaf people would give up deafness for hearingness.

Let’s consider another aspect: what happens to those who remain Deaf-Blind despite new treatments? Many Deaf-Blind people have lost their vision due to trauma or other sight-stealing conditions, so Usher treatments will be useless for them. And then there are those who will refuse any treatment, choosing to accept wholly their Deafblindness (much like late-deafened adults who choose to forego the cochlear implant and embrace their new Deaf identity). And what about those who have less access to treatments due to social injustice?

I’m reminded of something Michael Chorost wrote in Rebuilt, which he paraphrased in his speech to Gallaudet University:

You might think, for example, that African-Americans, being 12% of the U.S. population, would also be 12% of the [cochlear] implanted population. In fact, they’re only 4% of the implanted population. And that’s despite the fact that minorities have a higher incidence of disabilities than the Caucasian population. These numbers suggest that most white and wealthy kids will get implants and live entirely in the hearing world, while many nonwhite and poor kids will become signing deaf. The conclusion I draw in my book is that not only is the signing deaf community likely to become smaller, it will become more multiracial, and unfortunately, more economically disadvantaged.

The same could be predicted for the Deaf-Blind community. With vastly fewer Deaf-Blind people to serve, how will Deaf-Blind organizations continue to serve the remnants of the Deaf-Blind community? How can we make sure those people are not left behind even as their numbers shrink to statistical insignificance?

And probably most relevant to you, the reader: is the coming demise of the Deaf-Blind community a harbinger for the coming onslaught of stem cell and genetic treatments for the larger Deaf community?

After all, a lot of what was discussed at FFB’s Day of Science was research into the structure and function of cilia cells such as photoreceptors in the retina, and stereocilia, also known as cochlear hair cells. By looking at cilia from other parts of the body such as the kidney or the ear, researchers hope to discover more about photoreceptors. In fact, one specific study centered on the use of modified adeno-associated virus (AAV) as a vector to deliver proteins or modified DNA to the retina. That same study looked at the feasibility of using AAV for stereocilia. Guess what? This same approach to treating retinal degenerative diseases is probably going to work for treating genetic hearing loss.

More than ever, hearing and vision are linked. After all, the man who invented the Clarion cochlear implant is working on developing retinal chip implants! It’s rather ironic that finding a cure for the blindness part of Usher syndrome will probably be immediately followed with a cure for hearing loss. The Deaf-Blind community is going to change forever within our lifetimes, but not without witnessing transformational changes in the Deaf community as well.