retinitis-pigmentosa

2

Researcher advances retinal implant that could restore sight for the blind

People who went blind as a result of certain diseases or injuries may have renewed hope of seeing again thanks to a retinal implant developed with the help of Florida International University’s W. Kinzy Jones, a professor and researcher in the College of Engineering and Computing.

A tiny video camera mounted on special glasses captures the scene in the patient’s environment, and a pocket controller relays the captured video signal to the implant. Inspired by cochlear implants that can restore hearing to some deaf people, the retinal implant works by electrically stimulating nerve cells that normally carry visual input from the retina to the brain, and bypassing the lost retinal cells.

The Boston Retinal Implant Project, a highly-specialized, academically-based team of 30 researchers including Jones, was responsible for bringing the implant to light. The group is comprised of biologists and engineers from Harvard, Cornell, Massachusetts Institute of Technology (MIT) and others who are developing new technologies for the blind.

“Jones’ work was one the most important technological developments needed to make the device possible,” said Douglas Shire, engineering manager for the Boston Retinal Implant Project. “As a result, users of the retinal implant will be able to adjust the implant according to their needs.”

Jones has been working for years to advance the airtight sealed titanium housing and feed-through component that transfers the signals from the implanted microchip to the electrodes. His improvements in the density of that feed-through will greatly improve the quality of the image the person wearing the device will see.

The retinal implant was designed for people who lost vision due to injury to the eyes; progressive vision loss caused by eye disorders (also known as retinitis pigmentosa); or age-related macular degeneration, when the center of the retina that is responsible for central vision deteriorates. According to the National Institutes of Health, age-related macular degeneration is a leading cause of vision loss in Americans 60 years old and older.

“The impact of this technology, which increases the available pixels that can be stimulated, will bring enhanced visual acuity to people with debilitating eye loss,” Jones said. “My mother had macular degeneration and I saw the quality of her life degrade as the disease progressed. Hopefully, when these devices are available for FDA approved use, total loss of eye sight from macular degeneration or retinitis pigmentosa will be a thing of the past within 10 to 15 years.”

Recently, a similar device that features 60 electrodes was approved for use in patients and has proven successful in allowing people who were blind to read words on a screen.

Shire explained that the device that the Boston Group is building with Jones’ help has more than 256 electrodes and therefore allows for images with a larger number of pixels, which is expected to give patients a meaningful visual experience.

An important message, please read.

Hi guys, Tiger here.

Now I know we’re primarily an icon blog but, considering the raise in wanting to bring more awareness to less-known diseases, I want to help with a disease that strikes very close to home. That disease is retinitis pigmentosa, otherwise known as RP. It’s a genetic hereditry eye disease caused by variations in about 150 genes. And it runs in my family. My grandma has it, and my dad has it. I’ll likely get it at some point in my life; it’ll be a huge surprise (and, to be honest, a relief) if I don’t get it - but I won’t know until I begin to develop it. It might be possible to find out with genetic testing, but this is costly and not guaranteed to be accurate because of the number of genes involved. And to be honest? I’d rather not know. I’d rather have the hope that I won’t get it than be told “Yeah, you’re gonna get it”.

This disease is fairly unknown, and definitely one of the less known eye conditions. Everyone’s heard of cataracts, most people know about macular degeneration and glaucoma. Not many know about RP. I’ve tried to do little bits here and there with raising awareness, but this is the start of what will hopefully be a full-blown campaign. RP affects approximately 1 in 3,000 to 4,000 people. My family are included in that chunk.

Do you want to know how unknown this disease is, though? Once, my mum donated into one of those little pots that charities have and the person at the table had one question for her: “Who do you know that has it?”.

Whilst some research is going on currently, particularly in the area of stem cells, but more research is going to be needed to develop any form of cure or preventative for this disease. 

It is more commonly associated with those aged 40+, it can affect people who are younger than this, at least for the early stages. Commonly the first sign is a loss of night vision. From there, it can develop in a number of ways, depending upon the precise gene mix causing the problem. The most common variant is that the eyesight goes into tunnel vision, where there is vision at the centre of the eye and disappears at the periphery - this is the version my grandma had (she doesn’t have any useful vision beyond yep that’s light and yep that’s dark). Other forms exist with different symptoms - for example, my dad’s is tunnelling in one eye but the other eye has patches of pigmentation over the retina rather than a defined degeneration. What this may develop into we don’t know. From the loss of night vision to the total loss of sight can be as little as about five years; more commonly, it is likely to be around twenty years and can be longer.

I can’t speak for everyone with the disease and everyone who knows someone with it, but I can speak for my family’s experiences. As I said before, my grandma went down the route of tunneling vision, but my father’s symptoms are somewhat different: currently his vision is “as though he’s looking up through a pool of water” (this is how he describes it).

I remember when we first really noticed the symptoms in Dad. I was around eight years old, and we were all on holiday in Greece. For one of the trips we went on, we had to go down the driveway to our hotel. This driveway wasn’t particularly lit, and Dad was stumbling around and struggling to see. I stayed close to Mum, recognising I needed to keep out of Dad’s way but not really understanding why. I was only a kid, after all. And I was scared. Scared for my Dad, and scared for what this could mean. As time went on, more things in my family developed (these are independent of RP and not really going to be expanded on), but it meant that any further deterioration, which has happened, needed to be monitored and dealt with carefully. When the disease was properly diagnosed (the first signs were spotted in a regular examination by an optometrist about two and half years ago, and was confirmed by electro-diagnostic techniques in early 2014) in this later deterioration, Dad stopped driving because he didn’t feel like he would be a safe driver with this diminishing sight. (It is worth noting here that many driving license authorities would require this diagnosis to be declared to them.)

This is the donate link if you wish to donate. Please, if you can afford it, donate. We might not be able to find a cure in my lifetime, but if we can start work on one and possibly even preventative measures for future generations, I will consider this campaign I’m trying to start a success. Hell, even making people more aware will make this campaign a success. I’m also hoping to start a challenge similar to the ALS ice bucket challenge at some point, so if you can’t donate - keep an eye out for that. And if you want to know more about my experiences with RP in my family, send me a message (preferably off anon) here.

Please help spread!

   This is a Facebook page that I’ve created to spread awareness of Retinitis Pigmentosa, a debilitating eye disease that concludes with complete blindness.  If you’re interested in helping support this cause and spreading awareness, please visit this site and hit the “Like” button.  It would really mean a lot to me because my girlfriend, who got me hooked on Tumblr, suffers from this disease.  Thank you for your time in reading this paragraph.

Noninvasive Brain Control

New light-sensitive protein enables simpler, more powerful optogenetics.

Optogenetics, a technology that allows scientists to control brain activity by shining light on neurons, relies on light-sensitive proteins that can suppress or stimulate electrical signals within cells. This technique requires a light source to be implanted in the brain, where it can reach the cells to be controlled.

Continue Reading

What It’s Like to See Again with an Artificial Retina

Elias Konstantopoulos gets spotty glimpses of the world each day for about four hours, or for however long he leaves his Argus II retina prosthesis turned on. The 74-year-old Maryland resident lost his sight from a progressive retinal disease over 30 years ago, but is able to perceive some things when he turns on the bionic vision system.

“I can see if you are in front of me, and if you try to go away,” he says. “Or, if I look at a big tree with the system on I can maybe see some darkness and if it’s bright outside and I move my head to the left or right I can see different shadows that tell me there is something there. There’s no way to tell what it is,” says Konstantopoulos.

A spectacle-mounted camera captures image data for Konstantopoulos; that data is then processed by a mini-computer carried on a strap and sent to a 60-pixel neuron-stimulating chip that was implanted in one of his retinas in 2009.

Nearly 70 people around the world have undergone the three-hour surgery for the retinal implant, which was developed by California’s Second Sight and approved for use in Europe in 2011 and in the U.S. earlier this year (see “Bionic Eye Implant Approved for U.S. Patients”). It is the first vision-restoring implant sold to patients.

Currently, the system is only approved for patients with retinitis pigmentosa, a degenerative eye condition that strikes around one in 5,000 people worldwide, but it’s possible the Argus II and other artificial retinas in development could work for those with age-related macular degeneration, which affects one in 2,000 people in developed countries. In these conditions, the photoreceptor cells of the eye (commonly called rods and cones) are lost, but the rest of the neuronal pathway that communicates visual information to the brain is often still viable. Artificial retinas depend on this remaining circuitry, so cannot work for all forms of blindness.

Read more

As I Go Blind

As I go blind, please feel free to ask me what I can see. It changes constantly, and you’re curiosity shows me you are not afraid.

As I go blind, also ask me what I can hear, feel, and smell. I am learning to experience the world in a new and different way that can be astounding.

As I go blind, be patient with me as it may take me longer to complete tasks. The world can be nice at a slower pace too.

As I go blind, don’t expect me to be the poster child for blindness or always upbeat. I am terrified sometimes.

As I go blind, let me make jokes about my blindness. It helps me to be less afraid.

As I go blind, if you don’t know how to help me, ask. I will show you how to do so.

As I go blind, if I say I don’t need help, don’t be offended. I am still an independent person. I know if I need help or not.

As I go blind, don’t treat me like your pet or someone you have to fix. I’m not broken, just different.

As I go blind, understand that I am going to become more acutely aware that ableism exists. I am truly sorry I failed to realize how much of an issue this was until it affected me. Don’t make the same mistake.

As I go blind, don’t pity me. I am still human. I am still alive. My life is just as much worth living as yours.

As I go blind, remember that my wants and wishes may differ from those of another person with vision difficulties. We all deserve to be respected as individuals.

As I go blind, remember that I am still myself and that is never going to change.

THE PERFECT TUNNEL VISION ANALOGY

How many of you have played Pokemon? More specifically, how many of you remember Rock Tunnel from Pokemon Fire Red/Leaf Green?

Here’s a screenshot: 


This may seem like a blatant example of tunnel vision. ‘Blah blah restricted vision, so what’. However, allow me to explain why I find this such a particularly good analogy: usually, you aren’t just looking at a screenshot, you’re actually playing it. And here are the problems you encounter in the game.

  • While you can see everything directly around you just fine, everything only has to be a little out of your vision for you to be totally lost. 
  • Finding items is a nightmare, because you’re looking through such a small area. You can be in the same area as it but if you don’t know exactly where it is, good luck to you.
  • The trainers can see you even when you can’t see them. Usually the first you know of trainers is when you get stopped because they’ve spotted you and they want to battle. This is a very big part of tunnel vision.

I mean… Maybe this all seems really blatant to you guys, but for me it was a big mental breakthrough for how to explain it. Because tunnel vision isn’t just a still picture - and in this moment in this game, you actually live it. It makes me want to hack a Pokemon game so all of it is like that, then make people play through it. Then I can point at it, and go ‘That’s what it’s like to have tunnel vision.’

Low Vision ≠ Low IQ

Outrageous claim: I may be going blind, but I’m not a moron.

I’m in the middle of a heated debate with the leaders of a support group for people with retinitis pigmentosa. I was diagnosed with RP at age 13 and have been riding the line of legal blindness for about ten years.

Quick background: RP is a degenerative eye disease that causes light and color receptors in the retina (rods and cones) to die. It starts at the outside of the retina, so it affects peripheral and low-light vision in early stages. The result of this is severe tunnel vision, which manifests (at least in my case) as clumsiness and/or rudeness. (I’ve unknowingly snubbed many a handshake in my time.) Later stages bring about waterlogging in the retina, which blurs central vision. Progression of both of these eventually leads to total blindness, though the rate of progression varies from person to person. There is no cure.

The group in question recently posted an article on their blog about how to sign up for Twitter, which was the catalyst for this debate. The article makes no mention of RP (i.e., how to use social media to connect with organizations involved with RP or other RP patients). It is literally a Twitter instruction manual. I have been known to be sensitive about certain topics, especially where RP is involved, but I found the inclusion of this article on the group’s blog condescending and offensive.

I recently heard a story on NPR about Katherine Bouton, who discussed the effects of spontaneous deafness in one ear. She authors a book called Shouting Won’t Help, and it really struck a chord with me. When someone has RP, it’s not always obvious, which has pros and cons. On one hand, an RP patient may be met with situations that make them appear clumsy, rude, drunk and/or stupid, such as these things that have happened to me:

  • Tripping over an unsupervised toddler at the movie theatre and dropping a jumbo tub of popcorn
  • Failing as a floor hockey goalie when the opposite goalie threw the ball across the gym in my direction and scored
  • Appearing to ignore a new acquaintance, who held his hand up to high-five me in a poorly lit establishment
  • Routinely knocking over full glasses of wine/pop/water at restaurants
  • In broad daylight, falling off the curb and onto the street while walking with a new romantic partner

The upside is that I have learned from these things and can take necessary precautions. Just as a person with hearing loss might face away from light sources so that they are better able to lip read, I can make sure I’m walking on the inside of the sidewalk and put my drink in the same place every time I sit down so that I know where it is. The fact that I can’t see well isn’t outwardly apparent to most people, so I get to choose who I tell and when. I’m not nearly as scared of losing my vision as I am about how I will be treated when it becomes obvious to the world that I am blind.

Maybe not all people suffering from vision loss feel this, but I want to maintain as much independence as possible for as long as possible. I might not be a good choice for the dodgeball team, but I can certainly figure out how to sign up for social networking sites myself. The fact that this is being featured on a blog for the RP community sends a message that members must not be smart enough to figure even the simplest things out for themselves. It assumes that RP patients are technology challenged and incapable of logical thinking. They might as well have posted an article about how to tie your shoe.

The author asserts that the article is relevant because another member asked about how to use Twitter, and if the information is useful for one, then others might find it useful as well. This may be true; however, I argue that, even if this is the case, there are FAR better resources available to help people use Twitter. It’s a support group, not a tech blog.

I was a member of an RP support group in London and only went to one event. I was 23 at the time and was the most junior attendee by at least 20 years. It was terrifying to meet a coffee shop full of people wearing sunglasses who had white canes and seeing eye dogs. I kept thinking, “This is my future. This is what’s going to happen to me.”

I was hoping that some people my age — I know they’re out there — would show up and be able to share the concerns that I had. When is the best time to tell new boyfriends that I have RP? Is it worth starting cane training now? When should I stop driving? Which bars and clubs have good lighting, so that I can enjoy nights out with my friends? This is what I’d like from a support group.

In my mind, an RP blog that explains how to enter your email address into a web form is akin to being in the coffee shop for that first and only support group meeting. I want to belong to a support group that I feel comfortable identifying with. The difference is that I simply wasn’t at a stage that allowed me to identify with the group in London.

One of the responsibilities of a support group is to accurately represent its members. I have a lot of trouble identifying with a group that presents me as an idiot.

Totally blind mice get sight back

Totally blind mice have had their sight restored by injections of light-sensing cells into the eye, UK researchers report. The team in Oxford said their studies closely resemble the treatments that would be needed in people with degenerative eye disease. Similar results have already been achieved with night-blind mice.

Experts said the field was advancing rapidly, but there were still questions about the quality of vision restored. Patients with retinitis pigmentosa gradually lose light-sensing cells from the retina and can become blind. The research team, at the University of Oxford, used mice with a complete lack of light-sensing photoreceptor cells in their retinas. The mice were unable to tell the difference between light and dark.

Reconstruction

They injected “precursor” cells which will develop into the building blocks of a retina once inside the eye. Two weeks after the injections a retina had formed, according to the findings presented in the Proceedings of the National Academy of Sciences journal. Prof Robert MacLaren said: “We have recreated the whole structure, basically it’s the first proof that you can take a completely blind mouse, put the cells in and reconstruct the entire light-sensitive layer.”

Previous studies have achieved similar results with mice that had a partially degenerated retina. Prof MacLaren said this was like “restoring a whole computer screen rather than repairing individual pixels”. The mice were tested to see if they fled being in a bright area, if their pupils constricted in response to light and had their brain scanned to see if visual information was being processed by the mind.

Vision

Prof Pete Coffee, from the Institute of Ophthalmology at University College London, said the findings were important as they looked at the “most clinically relevant and severe case” of blindness. “This is probably what you would need to do to restore sight in a patient that has lost their vision,” he said.

However, he said this and similar studies needed to show how good the recovered vision was as brain scans and tests of light sensitivity were not enough. He said: “Can they tell the difference between a nasty animal and something to eat?”

Prof Robin Ali published research in the journal Nature showing that transplanting cells could restore vision in night-blind mice and then showed the same technique worked in a range of mice with degenerated retinas. He said: “These papers demonstrate that it is possible to transplant photoreceptor cells into a range of mice even with a severe level of degeneration. “I think it’s great that another group is showing the utility of photoreceptor transplantation.”

Researchers are already trialling human embryonic stem cells, at Moorfields Eye Hospital, in patients with Stargardt’s disease. Early results suggest the technique is safe but reliable results will take several years.

Retinal chips or bionic eyes are also being trailed in patients with retinitis pigmentosa.

youtube

Blind man sees wife for the first time in a decade using bionic eye

URGENT MESSAGE: MILES FOR MEGAN

TUMBLR USERS! I have an urgent message to bring to you all!

As this is a community of lovely people that are always talking about how much they want to help others, I thought that this would be a good forum where I could promote a cause I have been promoting for a very long time - finding a cure for Usher Syndrome. 

I was born with Usher Syndrome Type 1B, and it has impacted every day of my life. I am profoundly deaf and going blind. I have Retinitis Pigmentosa, which makes my peripheral vision decrease and reduces my vision at night to nil. We have done much to raise money for this cause, walking almost every year in our local Vision Walk for the Foundation Fighting Blindness, amongst many other things. We recently partnered up with the Usher Syndrome Coalition in our most recent efforts.

To raise money for this cause, my father is embarking on a cross-country trek from Virginia to the West on his bicycle starting on the day after Earth Day. It is a great challenge, and our goal is to raise $4,000 - not very much, but we are setting the bar low! We hope to earn more, obviously. I was hoping that my lovely friends on the Tumblr community could help! Please consider donating here (http://milesformegan.causevox.com/) or just simply liking and sharing our Facebook page (https://www.facebook.com/milesformegan) and leaving a comment to support my dad as he embarks upon this trek across country!! For more information about Usher Syndrome, go here (http://www.ushersyndrome.net/), and to read up on the Usher Syndrome Coalition, go here (http://www.usher-syndrome.org/). If anything, I hope to raise awareness about this genetic disease that affects millions of people around the world. :D

THANK YOU SO MUCH!!!!! I LOVE YOU ALL!!!! <3

Bionic eye maker has vision of the future

Robert Greenberg got tired of hearing from senior engineers that it wasn’t possible to build his product idea: a bionic eye that gives sight to the blind.

"A lot of the folks straight out of school didn’t know any better, so I hired them instead," quipped Greenberg, chief executive of Second Sight Medical Products Inc., a Sylmar biotech company. "They didn’t know how hard it was going to be, that it was impossible. And so they tried."

Greenberg can laugh now that he once thought developing the device would take a year and $1 million. Some 20 years and $200 million later, the first bionic eye has helped more than 20 European patients regain some of their sight.

Called the Argus II Retinal Prosthesis System, the device recently was approved by the Food and Drug Administration. Second Sight, which has 100 employees, is allowed to sell the bionic eye system to patients in the U.S. with advanced retinitis pigmentosa, a degenerative eye disease that can cause blindness.

"We are a far cry from restoring 20/20 vision," said Brian V. Mech, Second Sight’s vice president of business development, who holds a doctorate in materials science and an MBA from the UCLA Anderson School of Management.

"We are taking blind people back up to low vision, and that is pretty significant."

Mech likes to show videos of once-sightless patients who, after receiving the retinal prosthesis, are able to follow a person walking down the street and discern a street curb without using their canes.

"Until our product, these patients had no other option to obtain the ability to see," Mech said of the $100,000 device, part of which rests on a pair of Oakley Inc. sunglass frames. The cost to European patients has been paid by insurance companies in most cases.

Palo Alto attorney Dean Lloyd, who lost his vision 17 years ago, got the bionic eye system as part of the U.S. testing process. It allows him to see “boundaries and borders, not images” but has had a profound effect on his life.

Lloyd cites an incident before he received the eye system that still rankles. In the middle of a courtroom trial, an opposing attorney said Lloyd didn’t stand a chance with his case because he couldn’t even keep his socks straight: Lloyd had mixed up his black, courtroom socks with his white athletic ones.

"What did I do after the surgical procedure that I hadn’t been able to do?" Lloyd said. "I went home and sorted all of my socks."

The story of how the bionic eye came to be made in Sylmar underscores the state’s long record of medical device advances and involves top university researchers who were brought to Southern California to work on the project.

Greenberg likened the degree of difficulty to “shrinking a television set to the size of a pea, then throwing it into the ocean and expecting it to work.”

For Greenberg, it began in the early 1990s when he was a doctoral candidate in the Department of Biomedical Engineering at Johns Hopkins University in Baltimore.

Some of the first work was being done there, testing patients who had lost their vision because of retinitis pigmentosa, to see if electrically stimulating their retinas would produce results. It did.

"Using one electrode, the patient saw one spot of light," Greenberg said. "Second electrode, and the patient was seeing two spots of light. During that experiment, I was hooked."

Greenberg said he thought: “This is just engineering. Put more spots and you could make more pixels, like lights on a scoreboard or pixels on your computer monitor. You could see images.”

There was a breakthrough of another sort a few years later, in Washington. There, Greenberg was working as a medical officer and a lead reviewer for the FDA’s Office of Device Evaluation when he met entrepreneur Alfred E. Mann.

Mann had already established himself as a medical device developer through Mannkind Corp. and several other Southern California companies. During the 1980s, the self-made billionaire founded Pacesetter Systems, which made cardiac pacemakers. From there, he moved on to insulin pumps and related equipment.

Another Mann-funded company, Advanced Bionics Corp., took on cochlear implants, which could restore hearing to the deaf. It was the electrode-based cochlear implant that formed the rough basis of Second Sight’s first bionic eye.

In 1998, Second Sight opened with the financial backing of Mann and Sam Williams, another successful entrepreneur whose company, Williams International, designed and built small, efficient turbofan jet engines.

"Sam Williams was blind from retinitis pigmentosa, the disease that we are treating," Mech said. "He had invested along with Al in Advanced Bionics, which restores hearing for deaf people, and they were already on the market in the ’90s. Sam said to Al, ‘Why can’t we do the same for blind people?’"

youtube

i decided to start posting more personal stuff on here, so to kick us off here’s a poem about my mom’s disability. x