“Crossing The Line From Visual To Nonvisual Skills”

An Interview with Maurice Peret
(Blind Industries and Services of Maryland)
Dan Roberts, Moderator
International Low Vision Support Group
February 10, 2011
Dan: Welcome to our webcast, Maurice. Please tell us how you became blind and how that led you into your work as rehabilitation trainer for Blind Industries and Services of Maryland.
Maurice: How I came to work at Blind Industries is a bit circuitous, but I went blind in infancy and grew up doing most of the normal things that children do: not not really identifying myself as blind, per se, but just that I was different. So I grew up as, I guess, what I thought to be partially sighted at the time. Then secondary glaucoma in my teenage years caused a very gradual deterioration of my perception. So I’ve been totally blind for over 20 years.
Dan: Long enough to become an expert at it. And that’s why we have you here today. We with macular degeneration are all pretty much new at this, unless we are in our 80s or 90s and acquired this disease as long ago as you did. And so we have a problem with having had vision for most of our life, and then having had to make the transition, or what we call crossing the line, from visual to nonvisual. So I think we’re going to get into that a lot today.
Before we do, however, you have earned the unique honor of being called ”the other blind guy who climbed Mount Everest”. Would you tell us a bit about that fascinating experience?
Maurice: Actually, I was listening very carefully to your introduction about how I was selected to participate in the Mount Everest base camp team, because I was waiting to hear an answer. I was hoping we would get into that, because I was wondering how I had become selected to be part of it.
I just know that I was on vacation in northern Pennsylvania back in December 2000, and I checked my home answering machine. There was this message from Dr. Marc Maurer, president of the National Federation of the Blind, and my first response was that he doesn’t just call me for no reason. I thought maybe I was supposed to be somewhere, and maybe I was in trouble for something. I called him back, and he asked me if I remembered this guy named Erik Weihennmayer, and I remembered hearing of him at a former national convention. He was talking about climbing mountains and climbing ice faces, and I thought this guy was just nuts. That was my impression, and now all of a sudden I am being asked if I wanted to become part of the base team to handle communications to help get the message of the NFB out into the world about the first blind man to climb Mount Everest.
I was broadsided, and my first question was, “You’re kidding, right?” And if anyone has anyone has ever heard a speech or talked to Dr. Maurer, he’s got a great sense of humor, but not quite that flippant. He definitely wasn’t flippant, and he wanted an answer within 48 hours. The bottom line for me was, if I give up this chance, someone else will have it, and I’ll probably spend the rest of my life wondering what would it have been like. So I jumped at it, and I mostly don’t regret that decision. It was quite a fascinating trip and experience.
Dan: What would you say is the key ingredient to reaching that level of achievement and similar levels of accomplishment that you have achieved, and which other people with virtually no vision are able to achieve?
Maurice: You know, I’m trying to think what that ingredient is. If it’s a characteristic or a personal attribute. Or if it’s the right words or the right technology. And I’m left at somewhat of a loss. I guess in my case it was that I was hungry for that opportunity to have an impact in some way and have experiences that often times people who are blind simply don’t have, or are not offered. Or, as we are losing vision, at whatever rate, we tend to limit our experiences and slow down or stop doing having experiences.
I was at a point in my life where I saw lots of opportunities go by while I sat waiting, and here was a great opportunity to go somewhere I never thought of going. So I was hungry for it, and perhaps a little insanity doesn’t hurt, but I just went for the opportunity. I think it’s having the guts or not having the reason to not accept a challenge like that when it’s presented.
Dan: Do you think youth had a lot to do with that?
Maurice: You know Dan, the easy answer would be to say yes, it has a lot to do with youth. But my experience tells me that I am surrounded by people with whom I work who have me by quite a few years and are embarking on careers and new skills. So certainly youth is part of it, but I think there are studies that show the brain’s capacity for learning new skills even after 50 years old, so the capacity to do that is there.
The willingness is what we’re talking about. Being daring and getting out of our comfort zone. My experience tells me that there are people doing this all the time: people who I am inspired by. I might add on that at the Mount Everest expedition, there were four or five world records broken. One was Sherman Bull, who was 64 years old at the time. He broke the record as the oldest man to summit Mount Everest.
Dan: Okay, I’m glad you didn’t give us the easy answer. It isn’t easy, is it, being blind or losing vision? But we can do something about it, and that’s what we’re talking about today. You are committed to the belief that alternative techniques can help a blind or visually impaired person to match the abilities of his sighted counterparts. Do you think that is possible in every situation?
Maurice: Before I had embarked upon by journey into this whole field of blindness, I’m not sure how I would have answered that. Probably “no”. What I’ve learned in my development in the field, professionally and personally, and what I’ve seen time and time again is that yes, it is possible. It is possible in every situation despite acuity, despite the different situations, and even additional challenges. I’ve just seen too many wonderful stories, miraculous stories, of people who have regained their lives and done things I never thought they ever would have thought of before the onset of vision loss. So I absolutely believe that it is not only possible, but that it’s within sight to even expand those numbers of people who would take on challenges like that.
Dan: What are the most important skills for a newly impaired or blind person to acquire?
Maurice: You know, there are very basic core skills of blindness that should be achieved, and it would be hard to single out what they would be. Whether it would be information, communication, Braille or large print, or travel skills. But since you’re asking about the single most important, I believe it has to do with one’s mindset or attitude. I like to call it one’s philosophy, and I realize that people cringe sometimes at that description, but it is a way that we need to begin to think of the world and to think of ourselves, and to think of our disability, if you will, differently than it has been ingrained in us to think.
So the most important ingredient is to have that attitude change, and the only way I know to achieve that is to be surrounded by people who reflect or model those attitudes. People doing things that I sort of dismissed for myself, because, after all, I was blind. And so, it’s being part of a community and seeing oneself as something more than the disability would have us believe about ourselves, which tends to be less.
Dan: We did find in a recent study that we were doing that up to 99% of skills of daily living skills that we have as a visual person can be replaced, supplemented, or substituted for with nonvisual skills. Do you agree with that figure, or am I just being optimistic?
Maurice: I think you’re being optimistic, but I think it’s entirely realistic. Generally, I will sit down with a new student with whom I’m working, or a newly blind person, and play this little game. I once did this with a group of first graders. I said, “Try to think of what blind people can’t do,” and one smart aleck raised his hand and said, “See.”
I couldn’t argue with that, but by and large, people will come up with all kinds of careers and duties and tasks for which there is always a way. if I don’t know how to do a thing nonvisually or by using alternative techniques, I know somebody who has. Until recently, the one thing we had to put on that list is driving, and it’s made quite a lot of press. But that technology is being put into place experimentally at this point that answers the question definitively that even driving is not off limits. It can be done in an alternative fashion. It is all about how we can rewire our brains to think. We are wired for vision, there’s no doubt about that, but can we rewire or rethink how we do things? Absolutely.
Dan: A common low vision rehabilitation model recommends a training period during which all skills are introduced, after which more intensive practice and specific skills is offered as needed. How effective has that approach been in your field of work?
Maurice: You know, that’s a much bandied about discussion, and I don’t know there is sufficient scientific data that can say for a fact that one or another approach is statistically more effective. I think that that’s one of the weaknesses of the blindness and low vision field, is that we need to compile that kind of research.
But I’m familiar with a number of those models. I’ve worked in environments that offer the approach that you described. You’ll forgive me for using shorthand, because there are pitfalls to that, but what you described I have often described as the cafeteria approach. Here are the skills that are available, and you can make up your mind, so when you think you might need Braille, come back in a few years. Or cane travel, where you don’t travel that much now, and people take you to the grocery store. Maybe in a few years if your vision gets worse, you might want to come back and we’ll teach you how to use the cane.
The problem to me with that approach is I believe in a process by which people should try to get the fullest benefit of what a training program can offer in a comprehensive way. So that they can be equipped to discern what skills are affective under what circumstances, depending upon their visual acuity or their level of activity. Many of the skills we are talking about take time to learn. I don’t recommend waiting until you lose enough vision where you would be thinking about using a cane or learning Braille, because that would be a lot of time wasted, and it takes a lot of time to learn and master these skills. So the short answer is, when people are presented with a cafeteria approach to rehabilitation, they may not know the full benefit of what those skills can offer them. They may not feel that they are up to the challenge to learn those skills, so it would take some time to be among confident instructors and other peers who have mastered those techniques, to begin to have the confidence and to begin to feel like those skills can belong to them, that independence can really belong to them.
So just taking a look at “I want a little bit of this and a little bit of that later,” what you can have is people returning to training over and over again. I’ve seen that, and it’s a little bit sad, in that sometimes I think they could’ve gotten a full meal all at once in a one-stop, comprehensive, few-months-out-of-their-life period of time.
Dan: How do you explain how would you explain the resistance that you run into with people who say they don’t need rehabilitation
Maurice: It’s a very interesting challenge, Dan. People, you find that no matter what age, when they take up things they love, that they’re passionate about, it may be music, it may be cooking, it may be knitting, or it might be listening to books on tape, name it, they’ll take it on with a gusto. And if it involves learning a new technology to do it, it’s interesting that they’ll find a way to do it.
The reistance to learning these new skills is tied very much to confidence and to one’s self perception as going through this experience of losing vision, however gradually. So what we’re really dealing with is a person facing a challenge and thinking, “Am I up to it? Can I really do these things?”
And tied to this, also, is the very ubiquitous social stigma of blindness. Call it whatever you want. I use the term blind very generically in order to demystify or try to bring it into common parlance so that it’s not a dirty word. It’s that social stigma. That’s the resistance. No one wants to be perceived as less or somewhat less intelligent, or slower, or that you need to raise your voice when talking with someone with low vision, because of all these misperceptions and public misunderstandings about blindness.
When we’re losing vision, we’re part of the public. Those are the things that we think about, so being blind is something we want to hide and resist. So learning new skills like putting a cane in your hand or using adaptive techniques, oh my gosh, that kind of makes me one of those, and I don’t want to give in to that demon. But if we can get past it and help support one another in a positive atmosphere, again with lots of other blind role models with various visual acuities, whether it’s macular degeneration or retinitis pigmentosa, or whatever it is, but seeing what other people are doing and wanting to be part of that is part of the process of getting over that resistance. It can happen. It’s just a matter of time and how one can process that and get over the sometimes very paralyzing fear and grief and all those things that are associated with newly acquired vision loss.
Dan: How important is patient education and self-determination in the success of a person’s low vision rehabilitation?
Maurice: That’s an excellent question, Dan, because it’s critical. Information is key to everything. We want to be, in our society, educated consumers, right? Going back to the previous question about traditional low vision rehabilitation models or the model to which I subscribe, I have an opinion about that. Others have opinions about traditional low vision rehabilitation, but what really matters is the person who’s going to be a consumer of that training.
And so I recommend that people don’t just come by and see us and see what we do, but that they go next door, or to the next state, or across the country and see what other people are doing so that they can make an informed decision. It’s critical to educate ourselves as much as possible about rehabilitation paradigms and models, as well as the research behind our condition, so that we can understand better about what we are seeing or not seeing. It’s critical and should always be encouraged.
Dan: One of the issues we brought up earlier was that some people wait too long. They come in for rehabilitation or they are expected to come in when they have a problem. You called it the cafeteria approach. Unfortunately, that isn’t always a practical situation. Another problem we have with obtaining knowledge and training in rehabilitation is the factor of distance and time. Is there a low vision rehabilitation clinic or agency within a reasonable amount of distance or time from our homes? And how motivated are we to make that effort, especially in the beginning when we are not that familiar with it? Which brings me to our Self Help Guide to Low Vision Skills. Would you like to say a few words about this approach?
Maurice: Dan, I did read the guide, and we’ve talked about it over the phone. One has to appreciate the obvious love and effort you’ve put into this document. And if we can make this available to others, they may want to add to it as well, It’s a very useful tool. Somebody can sit at home and read this on their computer or have someone read this to them, whatever, and go down the list, and it might really enlighten them to seeing the many activities that they are not doing anymore that they could be doing. It would inspire people to look into ways that it could be done. I do think it’s a very useful tool.
One of the things I’m reminded of is that I used to run a program that the NFB launched involving over 300 newspapers being made available for free to anyone who wants access to them. That’s an example of the kind of resources that you include in that guide, which is exciting. It’s a dynamic list. It’s always changing, and hopefully, it will give people some ideas.
And when you talk about social activities like dancing or going out to dinner, people don’t do these things anymore. They won’t go to restaurants for Valentine’s Day anymore, because it’s dark. And they don’t want to be embarrassed, because they can’t see their food. So I just want people, whatever it takes for them to bring this to light, to make the phone calls and visit an agency or group or low vision clinic that can help show people the possibilities. Contact us to see how you can do this or do that, and it’s a wonderful discussion to have.
Keep up the great work with it, Dan. I’ll continue to follow it. I hope other people on this forum will add things that they have learned can be done, and it could be a very lively and dynamic piece of work.
Dan: I appreciate your positive thoughts on that, and I hope you not only follow it, but that you help lead the way with this guide and become a major part of it, because it is a living document that’s going to continue to change. It’s totally free to use, and every other organization can take it and expand upon it. That’s what it’s out there for.
Maurice: I really do want to thank you very sincerely, as well as the people who’ll be listening in on this now and later on, for welcoming me so warmly to what I feel is another branch on our family tree. I do feel like it’s a community where we should be open and discuss anything that comes to mind. It’s stimulating to me, it’s exciting to me, and the message, I hope, is that the people who are beginning to see changes in their vision will pursue all the research they can. But know that life doesn’t need to end or even diminish. There is no substitute for 20/20 vision, but there are ways you may not expect that might be better. Keep that in mind, consider it, and let’s talk more about it. I do thank you, and I’ll be looking for news on the website and our further correspondence.

Summary of Research and Developments in Macular Degeneration: 2006-2007

by Dan Roberts
This is a summary of all major research and developments pertinent to macular degeneration that occurred between mid-May 2006 and mid-May 2007. It is organized by month of occurence under the subsets “Pharmacology,” “Surgery,” “Nutrition” and/or “Miscellaneous.” The final section, “The Future,” summarizes work in progress that may lead to promising developments in the coming year.
An audio/visual presentation of this summary is available on the International MD Support Group website. In most cases, full articles are available from the MD Support Library. Copies are permitted as long as proper credit is given.
May 2006
Pharmacology
Triple therapy found to be effective for wet AMD
Triple therapy with a combination of intravitreal Kenalog, photodynamic therapy (PDT), and intravitreal Macugen (pegaptanib sodium) has been found to be safe and effective for treatment of wet AMD. Improvements in visual acuity and in macular thickness in sixteen patients were reported at the ARVO meetings by JM Colina-Luquez MD, ophthalmologist, New England Retina Associates, Hamden, Connecticut
7.9% of those who had had prior therapy had an improvement in visual acuity of 3 lines or more, compared to 33% of those with newly diagnosed disease. This is an improvement in acuity from 20/200 to 20/50.
Macugen may present risk of increased IOP
Optometrist Allison Toler, OD (East Florida Eye Institute, Stuart, Florida) and ophthalmologist Ronald Frenkel, MD, Bascom Palmer Eye Institute, University of Miami, Miami, Florida) reported to the ARVO meeting that treatment with pegaptanib sodium (Macugen) may run the risk of significantly increased intraocular pressure (IOP). In a study of eight patients, mean pre-injection IOP was 12.9 mm Hg and mean post-injection IOP was 39.4 mm Hg. The IOP returned to normal in most cases within 30 minutes of the injection, and eventuially all patients showed normal IOP levels.
The researchers then compared Macugen injection to Avastin (bevacizumab) injection. The spikes in IOP were similar, but IOP decreased faster in patients who underwent Avastin treatment. As a result of these findings, physicians were cautioned to closely monitor IOP levels in glaucoma patients who are also being treated with Macugen.
Intravitreal injections affect the fellow eye
S.D. Martin et al (Department of Ophthalmology and Visual Sciences, University of Louisville School of Medicine, Kentucky Lions Eye Center, Louisville, KY) reported to the ARVO meeting that intravitreal injections of anti-VEGF drugs (i.e. Kenalog, Macugen and Avastin) also have an effect in the fellow eye. This is possibly the result of systemic absorption. Conclusions were drawn from analysis of pre- and post-injection ocular coherence tomography (OCT) graphs of 29 patients over a six-week period. Intravitreal use of these drugs, therefore, should be done with caution, since such absorption might also affect other systemic functions of the body. The researchers suggested that lower dosages of Macugen may be wise.
Surgery
No association between cataracts and macular degeneration
Susan Bressler, MD (Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland) reported to ARVO that analyses of data from the Age Related Eye Disease Study (AREDS) has shown “no clear evidence of an association between cataract surgery and neovascular age-related macular degeneration,” and that “most patients undergoing cataract surgery can probably be reassured that surgery will not markedly increase their risk for progression to neovascular age-related macular degeneration.” The conclusion was drawn from checking outcomes after 1,704 cataract surgeries and 543 neovascular ARMD events after baseline among 8,152 eyes with a median follow-up of nine years.
Nutrition
New Lutein and Zeaxanthin Findings
The Melbourne Collaborative Cohort Study presented evidence to ARVO that neither lutein nor zeaxanthin are protective against the progression of AMD.
The same study of over 41,000 subjects showed an association between high intake of unsaturated fats and development of AMD, and that olive oil intake may be beneficial.
At the same meeting, a second multicenter study of 300 subjects showed that daily supplementation with 18 mg lutein and 2.4 mg zeaxanthin over six months resulted in a slight increase in macular
pigment density.
Miscellaneous
Blue light risk
Research from the University of Chicago has confirmed that the blue light wavelength peaking at 440 nm causes retinal damage through photochemical change and apoptotic cell death. The study authors repeated the recommendation stressed by MD Support at the 2005 ARVO meeting that blue blocking lenses be worn to protect the retina from direct exposure to strong blue light (i.e. sunlight, either natural or artificial).
June 2006
Pharmacology
VEGF Trap
Regeneron Pharmaceuticals announced positive results at the sixth week of its Phase I dose-escalation study of intravitreal anti-angiogenic drug VEGF Trap. 21 patients received a single injection of one of six doses, from 0.05mg to 4mg, and were monitored for 12 weeks.
July 2006
Pharmacology
Avastin Demonstrating Safety and Efficacy
In a paper presented at the 2006 meeting of the Association for Research in Vision and Ophthalmology researchers suggested that off-label Avastin has demonstrated safety and efficacy after over 5,000 injections. In July 2006, Lebanese researchers published positive results from a small study of 17 patients. Thirteen eyes (76%) showed total resolution of subretinal fluid, and the median acuity improved from 20/200 to 20/50. No systemic or ocular side effects were reported, but further controlled studies are needed.
Lucentis Approved for Wet AMD
On June 30, the Food and Drug Administration (FDA) approved Lucentis (ranibizumab injection) for the treatment of patients with wet age-related macular degeneration (AMD). Lucentis is the first treatment which, when dosed monthly, can maintain the vision of more than 90 percent of patients with this type of AMD.
“This approval is of great importance for the 155,000 Americans who are diagnosed each year with AMD, a common cause of severe and irreversible vision loss in older adults,” said Dr. Andrew von Eschenbach, Acting Commissioner of Food and Drugs. “At a time when our elderly population is rapidly increasing, this product preserves quality of life for those affected by this disease, helping them to regain the ability to participate in everyday activities such as reading and driving.”
The per vial price of Lucentis is $1950. The average patient will receive between 5 and 7 treatments in the first year of therapy ($9,750 and $13,650 a year), which is covered by Medicare and secondary insurance. Most patients will have a co-pay of $50 or less per treatment. For patients who are uninsured, Genentech has established financial assistance through its Access to Care Foundation. Eligible patients who cannot afford their co-pays may be able to receive co-pay assistance through one of the independent public charities to which Genentech provides funding. For more information, call 1-866-LUCENTIS (1-866-582-3684).
Research
Godfather gene discovered
News has come out of Sydney, Australia about a so-called “Godfather Gene” called C-jun that, if switched off, may be another way of stopping inflammation and neovascularization. Researchers based at the Centre for Vascular Research, University of NSW, have developed a drug that may be able to act as that switch.
The drug, called Dz13, targets the gene and destroys it, thus cutting off the blood supply that not only harms the retina, but also promotes cancerous tumors and inflammation. Scientists are, therefore, hoping that Dz13 will prove to be effective against several diseases, including wet macular degeneration, cancer, heart disease and arthritis.
Human trials were to begin in early 2007, so it may be several years before definite conclusions can be drawn.
August 2006
Pharmacology
New eyedrop treatment being tested for wet AMD
On August 22, 2006, Athenagen, Inc. announced that it had begun human testing of its topical (eye drop) therapy for wet AMD. Phase I trials of ATG003, a formulation of mecamylamine, are expected to generate data by the end of the year, and the company plans to move directly into a larger safety and efficacy study in early 2007.
Research
Stem-cell procedure promising
University of Washington scientists reported on August 14, 2006 that they have successfully used stem cells to treat diseased tissue in mouse retinas.
Tom Reh, UW professor of biological structure and leader of the research, said that if such research continues to be successful, the first human tests of the technique could begin in about two years.
The UW team used a mix of “growth factors,” natural proteins that encourage cell growth to coax the embryonic cells into becoming retinal cells. When the scientists mixed the new cells with damaged mouse retina, the cells replaced key cells essential to vision. Reh said his team now have begun injecting the new cells into the eyes of retina-damaged mice to see if there is actual vision improvement. “If things,” he said, “continue to look [good] over the next six months and other research moves ahead, we should be in a position to use this for eye diseases.”
Miscellaneous
Age-related Macular Degeneration Does Not Cause Blindness
A poll sponsored by MD Support shows that a strong majority of people affected by AMD do not think of themselves as blind, and they do not want the term to be used to describe their visual impairment.
The results of a recent MD Support opinion survey show that 93% of people with AMD are averse to the use of the word “blind” in connection with their condition. 91% of them do not consider themselves to be blind, 93% know they will not go blind from AMD and 93% think the word by itself should not be used in connection with AMD. These are convincing statistics that are now available for the first time to eye care professionals, patient advocacy organizations and public service agencies. Hopefully, the message is clear and will be heeded.
September 2006
Pharmacology
Research
Stem cell experiments slow vision loss in rats
On September 21, 2006, Raymond D. Lund, (University of Utah’s John A. Moran Eye Center in Salt Lake City) and Robert Lanza (Advanced Cell Technology Inc. in Worcester, Mass.) reported that cells grown from human embryonic stem cells slowed vision loss when injected into the eyes of rats with a disease similar to macular degeneration.
According to a recent press release (“Stem Cell Experiments Slow Vision Loss in Rats” by Rick Weiss, Washington Post, Thursday, September 21, 2006; Page A12) the researchers achieved the transformation in all 18 stem cell lines they worked with, proving that their approach can consistently produce the crucial pigment cells. Then they injected the cells, about 20,000 per eye, into the retinas of 14 rats with a genetic disease similar to macular degeneration. Eight control rats received eye injections without any cells.
Forty days after treatment, the team measured retinal electrical activity in response to flashes of light, and it found that the treated rats were twice as responsive as the untreated ones, which by then were going blind. A separate test — which tracks eye and head movements in response to a moving display, a measure of an animal’s ability to discern fine details — showed that the treated rats had twice the visual acuity of the untreated rats nearly three months after treatment.
Microscopic examination of the retinas at autopsy showed that the treated eyes had healthy photoreceptor layers five to seven cells thick, while the untreated eyes had an average thickness of just one cell. (Healthy rats have layers 10 to 12 cells thick.) None of the cells divided abnormally or grew into tumors, the team reported in the September issue of the journal Cloning and Stem Cells.
Miscellaneous
Medicare Starts Paying for Visual Rehabilitation Services
People who qualify for Medicare and whose eyesight has significantly declined due to macular degeneration, diabetes or certain other conditions can now get up to nine hours of vision rehabilitation through a new Medicare-sponsored demonstration project.
The five-year project is available in six areas of the United States: Atlanta, New York City, New Hampshire, Kansas, North Carolina and the state of Washington. It will allow the Centers for Medicare and Medicaid Services (CMS) to study the demand for – and effectiveness of – services aimed at helping visually impaired individuals make the most of their remaining vision.
After the project concludes, administrators will decide whether to extend reimbursement for those services to all Medicare recipients across the country, Greene said. “We think there’s a strong possibility that they’ll do that.”
In the six areas chosen, Medicare will pay for therapy services provided in clinics or at home by certified vision rehabilitation therapists, orientation and mobility specialists, low vision therapists and occupational therapists, but not the low vision aids themselves.
October 2006
Research
New Molecule Discovered
Case Western Reserve University and the Cleveland Clinic Cole Eye Institute have discovered a molecule that triggers neovascularization in the retina. Called Carboxyethylpyrroles (CEPs), it attaches to proteins in the retina, causing blood vessel growth that leads to sight loss in wet AMD. The next step is to identify the receptors that are activated by the CEPs and then develop drugs that can block the formation of the toxic molecule.
Miscellaneous
Leaders in the Low Vision Industry Reach Consensus on Important Patient Issues
MD Support took part in a roundtable discussion hosted by the National Association of Visually Handicapped in Washington, D.C. It proved to be informative and worthwhile, as the participants left with a better understanding of the concerns of patients and a consensus of opinions on potential improvements in care and management. Leaders from twelve advocacy, health and research organizations were present, in addition to representatives from Novartis Ophthalmics (sponsors) and the National Eye Institute. Three main topics were discussed in the day-long session:
1. Identifying and appreciating patient expectations and experiences with AMD diagnosis and treatment in order to help health care personnel provide individualized, efficient and holistic treatment.
2. The role of support services in helping AMD patients continue to perform daily tasks and maintain a positive attitude.
3. The language used to discuss the effects of AMD on vision and the effects of using terms such as “visually impaired,” “legally blind” and “blind.”
Unanimous consensus was reached on three principal issues:
1. Patients must be guided to information and emotional support immediately upon diagnosis. This would be best accomplished through support services at the clinic, direction to low vision counseling and/or guidance to reliable community and Internet resources.
2. Areas of concern that need to be addressed by support services are activities in daily living (including transportation), low vision evaluation, counseling, orientation and mobility, employment, and library resources.
3. To help lessen the often profound emotional response to a diagnosis of AMD, sensitivity is needed in the language used to describe the condition. In particular, the word “blind” should not be used without qualification to describe AMD to patients.
A Safer Way to Treat SAD(ness)
Research has shown that melatonin is a necessary antioxidant for physical and psychological health. The high point of the melatonin cycle, however, lasts 9-10 hours, and the average person does not have (or allow) that much time to sleep. Blue light exposure during the day may make a person less drowsy and depressed by suppressing melatonin levels in the blood, but the body still needs up to 10 hours of high melatonin levels for peak health.
Compounding the dilemma, blue light exposure appears to lead to macular degeneration in some people, due to interruption of the photoreceptor cells’ metabolic visual cycle (see www. mdsupport. org/library/hazard.html). This makes blue light therapy risky, especially for people with compromised retinas.
The obvious answer is to get more sleep; but for those who cannot do so, one option to potentially harmful blue light therapy has been proposed by Richard L. Hansler, Ph.D. (Director, Lighting Innovations Institute, John Carroll University. Executive Director, Light and Health Foundation). Dr. Hansler’s well-thought-out suggestion is to allow full completion of the melatonin cycle by avoiding blue light for 9-10 hours, beginning before sleep and continuing until morning. One easy way to accomplish this would be to simply wear your blue-blocking glasses for awhile before going to bed. Another is to switch to lamps that filter blue wavelengths.
November 2006
Pharmacology
New Anti-angiogenic Drugs Enter Trials
Two new drugs are now in trials to test their safety and efficacy in treatment of wet AMD. On October 31, MacuSight, Inc. announced the start of a Phase I study of sirolimus (rapamycin). On November 1, 2006, TargeGen announce the initiation of Phase I trials involving the prodrug, TG100801.
Sirolimus, which is injected into the eye, differs from other anti-angiogenic drugs in that it is a highly-potent, broad-acting compound that may be useful in treating a wide range of ocular diseases and conditions. TG100801 is administered as an eye drop, which makes it attractive to patients who are now having to undergo injections that are uncomfortable and carry certain risks.
Research
New Gene Discovery Provides Potential Marker for AMD
The protein Complement Factor H (CFH) has previously been found to play a role in the development of drusen leading to AMD. CFH has implicated inflammation as part of the AMD pathogenesis, and now, discovery of a new gene may complete the picture. Researchers have recently found that a single mutation in the gene HTRA1 on chromosome 10q26 is a major genetic risk factor for wet AMD.
Studies that genotyped 581 people with AMD and 309 without AMD have provided a strong predictor for individuals who have family histories of the disease. A blood test to identify the mutant HTRA1 gene can now identify those who are up to seven times more likely to develop AMD than those with a normal gene. Not only will this allow such individuals to take preventative steps to lower their risk, but identification of the gene as a drug target can bring about new treatments for AMD and other retinal diseases brought on by drusen formation.
Drusen Lasering Ineffective
Drusen are thought to be fatty waste products from the photoreceptor cells. They often appear on the macula (the center of the retina) in the early stages of macular degeneration, and they can cause gradual loss of central vision.
In 1999, ophthalmologists took an interest in using the laser to destroy drusen, based upon the theory that ridding the retina of these deposits may slow the development of MD, or even stop the progression from the “dry” form to the “wet” form. Two studies, however, have recently shown drusen lasering to be ineffective, and even potentially harmful, as a treatment for macular degeneration. The studies were called Complications of AMD Prevention Trial (CAPT) and Prophylactic Treatment of AMD (PTAMD).
In 2006, PTAMD researchers reported that laser treatment “to an eye with multiple large drusen in a patient whose fellow eye has already suffered a neovascular event places the treated eye at higher risk of developing choroidal neovascularization.” They concluded by advising against using prophylactic subthreshold diode laser treatment in these eyes.
Then, on November 1, 2006, the National Eye Institute announced that their CAPT studies have been small, and the results inconsistent. No difference in vision or in progression to advanced AMD between treated and untreated eyes were observed, so doctors are advised to reconsider drusen lasering as a treatment for AMD.
Miscellaneous
Exercise May Protect Against Wet AMD
A study at the University of Wisconsin has shown that regular exercise may help to prevent the wet form of age-related macular degeneration (AMD). As reported in the November 2006 issue of British Journal of Ophthalmology, the study monitored almost 4,000 people between the ages of 43 and 86 over a period of 15 years. Researchers discovered that those who engaged in regular physical activity at least three times a week were 70% less likely to develop neovascularization. One explanation put forth is that– like coronary disease–inflammation, high cholesterol and high blood pressure are suspected to contribute to the development of wet AMD, and exercise helps to reduce those three conditions. The study showed that exercise had no effect on the incidence of dry AMD.
December 2006
Pharmacology
Avastin still showing success
Three abstract sessions at the AAO meeting in December revealed that intravitreal treatment with bevacisumab (Avastin) for wet AMD and pathologic myopia is showing no systemic adverse events and that the off-label drug is showing success in inhibiting neovascularization and improving visual acuity. The general conclusion is that re-treatment is needed at 2-3 month intervals. The study titles were:
“Safety and effectiveness of intravitreal bevacizumab for subfoveal choroidal neovascularization in pathologic myopia” (Hernandez-Rojas, ML, et al, abstract #673).
“Intravitreal bevacizumab (Avastin) in the treatment of neovascular age-related macular degeneration” (Avery, RL, et al, abstract #673).
“Intravitreal bevacizumab (Avastin) for recurrent choroidal neovascularization” (Frederico Graue-Wiechers, et al, abstract #722).
Updates on anti-angiogenic drug studies
At the same meeting, reports were presented on recent research in the areas of antiangiogenic drug therapy for wet AMD, including Genentech’s MARINA and PIER studies of ranibizimab (Lucentis) and Alcon’s progress on the anecortave acetate (Retaane 15 mg) trials. Nothing particularly new was presented, but some interesting findings were discussed:
Elias Reichel, M.D. reported that in the MARINA trials, there was improvement at all visual acuity levels, but there was not a big difference if the patient’s baseline vision was either low or excellent. This “floor-to-ceiling” effect is not unusual with this kind of study, but it is useful information for patient communicating with patients about expectations.
Nancy Holekamp, M.D., discussed key anatomic endpoints in the MARINA trials, in particular, the total lesion area over time (no growth of the lesion area was noted in the treated patients), the mean area of leakage (the amount of decrease was statistically significant) and the mean foveal retinal thickness (treated eyes showed a significant thinning of the retina). The bottom line is that all anatomic outcomes from the trial favored Lucentis, and the treatment is so effective over a long period of time without any signs of toxicity, there is no indication that anything in lieu of Lucentis should be used to treat wet AMD.
Peter Kaiser, M.D., reported on subgroup analysis of Genentech’s PIER study. The primary endpoint was met, showing a difference of 16 letters visual acuity between the treated group and the sham group. Further, the dosing regimen was effective, but the visual acuity benefit was not as robust when injections went from monthly to quarterly. The persistence of monthly injections may depend upon who has dry lesions and who has wet lesions at the 5th month. The dry lesion group did better than the wet lesion group with quarterly dosing. This data is pointing toward better prediction of dosage outcomes for individual patients.
Jason Slakter, M.D., reported that Alcon’s anecortave acetate has shown weak anti-angiogenic activity, but the method of treatment (injection outside of the eyeball) gives it the chance to have an effect over a longer period of time. This, he said, justifies continuing the study in the face of the impressive Lucentis results.
Melissa Brown, M.D., M.N., M.B.A. (Center for Value-Based Medicine) addressed the question, “On what basis can doctors compare pharmacologic treatments for wet AMD?” She suggested value-based analysis, looking at both quality of life and cost, and starting with an examination of the evidence. In a comparison of several current interventions, she noted that Lucentis shows the highest percentage in quality of life, but highest in cost. Still, Lucentis–at approximately $50,000 per quality of life year–is within commonly accepted parameters in the field of medicine.
John Thompson, M.D., reported no significant difference in three dose levels of Acuity Pharmaceutical’s bevasiranib, an anti-VEGF small interfering RNA drug with the ability to turn off genes that cause wet AMD. Results indicate that treatment with direct VEGF inhibitors may be necessary initially before treatment with bevasirinab. Hopefully, a Phase III study will show effectiveness as long term treatment.
January 2007
Research
RHEO Therapy Back in Trials
It was recently announced here that OccuLogix’s multicenter MIRA-1 study failed to prove effectiveness of its RHEO System, a blood filtration device being used in Canada for treating dry AMD. Now the company has announced that it has obtained clearance from the FDA to commence a new phase III study called RHEO-AMD. If successful, the results are expected to support OccuLogix’s application to the FDA for approval to market its RHEO System in the United States.
Another source of stem cells
On January 7, 2007, researchers at the Institute for Regenerative Medicine at Wake Forest University School of Medicine discovered another potential source of embryonic stem cells in the amniotic fluid that protects babies in the womb. These cells appear to be almost as malleable as those in the embryo itself, and the advantage would be that harvesting them would not harm the embryo. Several more years of study are needed to assess their application in humans.
Lucentis Approved in Europe
Novartis AG announced on January 24, 2007 that Lucentis (ranibizumab) has received European Union approval for patients with wet age-related macular degeneration. According to a press release issued by the company, the EU commission decision applies to all 27 member states as well as Iceland and Norway. Lucentis will be launched in Europe beginning this year. It has already won approval in the United States, Switzerland and India.
February 2007
Pharmacology
Lucentis and Risk of Stroke
The media has recently reported on a letter sent by Genentech to retina specialists highlighting rates of stroke observed in their Phase IIIb SAILOR study of Lucentis.
Genentech has found in their planned 6-month interim analysis that 1.2 percent of patients treated with a high dose of Lucentis in a clinical trial suffered a stroke, compared with only 0.3 percent of those treated with a low dose. The difference was statistically significant.
MD Support encourages patients to not overreact to this news. The letter was simply a proactive attempt on behalf of Genentech to provide additional information to treating physicians. The observed rates of stroke in the SAILOR trials are within those observed in the MARINA and ANCHOR pivotal studies that lead to the FDA approval of Lucentis and that were reported in October in the New England Journal of Medicine. The FDA still stands with its approval based upon the trial results, which are clearly reported in the Lucentis package insert. (None of the Genentech studies, by the way, excluded patients with cardiovascular conditions.)
Avastin Benefits Patients With CNV From Myopic Degeneration
On February 15, 2007, Elias Reichel, M.D. reported to Hawaiian Eye/Retina 2007 that Avastin has shown good results in a small retrospective case series for treatment of choroidal neovascularization (CNV) from myopic degeneration. 15 eyes studied showed a mean improvement in acuity of 3 lines, central foveal thickness decreased an average of 93 µm (micrometers) and there were no complications.
CA4P for Myopic Degeneration Successfully Completes Phase II
Oxigene, Inc. has reported positive results from its safety and efficacy phase trials of CA4P in the treatment of myopic macular degeneration. Safety results were favorable and in line with expectations, with no drug related serious adverse events being reported.
The company also announced that it has completed a pre-IND meeting with the FDA regarding two topical ophthalmic formulations (eye drops and ocular mini-tabs) for CA4P in the treatment of age-related macular degeneration and intends to proceed with further development of topical formulations.
New anti-angiogenic eyedrops
On February 26, 2007, Othera Pharmaceuticals presented new preclinical data demonstrating the safety and effectiveness of OT-551, an antiangiogenic drug in eyedrop form. Results from the Phase I trials demonstrated that when the compound is added to either Lucentis or Avastin treatment there is a synergistic effect versus either treatment alone. Phase II trials are expected to begin in the second quarter of this year.
Another company, Athenagen, Inc., has announced successful completion of Phase I of a trial testing its topical (eye drop) drug ATG3 for wet AMD. On January 31, 2007, the company announced successful completion of Phase I, and that “the eye drop therapy showed excellent ocular tolerability. There were no study medication-related systemic side effects, consistent with the very low levels of the compound found in the blood following eye drop application.” Athenagen’s Phase II clinical trial is expected to begin in the first quarter of 2007.
Lucentis shown to be better than PDT
On February 15, 2007, Peter Kaiser, M.D. reported two-year results of the Phase 3 ANCHOR trial comparing Lucentis with photodynamic therapy (PDT) for wet AMD. The study showed that Lucentis helps maintain vision, with few adverse effects, significantly better than PDT. For details, see www.lucentis.com.
March 2007
Pharmacology
Positive Results From Combined Radiation/Avastin Treatment for Wet AMD
NeoVista, Inc. reports positive results from NVI-111 Study (concomitant radiation/Anti-VEGF) for the treatment of wet AMD. Using the Epi-Rad 90(TM) Ophthalmic System, manufactured by NeoVista, treatment involved intravitreal 24 Gy Strontium-90 beta radiation plus initial treatment with Avastin. At six months, 45% of the patients treated per protocol achieved >=3 lines of visual acuity gain. This compares favorably to the results obtained in Genentech’s MARINA Trial, which utilized 0.5 mg of Lucentis alone. In addition, all patients treated in this study achieved a mean change in visual acuity of 13.4 letters at 6 months, which compares favorably to the reported 6.5 letter gain at month six of the MARINA Trial.
NeoVista will soon begin its CABERNET trial incorporating the concomitant use of Lucentis and Epi-Rad 90 therapy. For more information, visit the company website at www.neovistainc.com.
DHA supplement trial
Retina South Africa E-News announced a Phase ll trial in the U.S. to test the effect of the fish oil supplement Docosahexaenoic Acid (DHA) on X-linked retinitis pigmentosa (RP). Some researchers believe that DHA supplementation may also slow the progress of the dominant form of RP and age related macular degeneration. Other research, however, has shown that oxidized forms of DHA are found in drusen. No patients should self-medicate without first consulting an eye care practitioner.
Zinc May Harm the Retina: New Finding
BBC News announced on March 18 that researchers at London’s Institute of Ophthalmology think the mineral zinc may play a role in the development of AMD. The study, published in Experimental Eye Research, found that drusen containing high levels of zinc may contribute to progression of the disease. When asked for her opinion on this new development, nutritionist Ellen Troyer, CEO of BioSyntrx, Inc., said:
“I’m carefully watching these studies. There is another study published in the Journal of Urology that links the ARED study participants to a much higher incidence of kidney problems. We [at BioSyntrx] repeatedly have said in every talk we give (as has every biochemist I know) that 80 mg of zinc is way too much for daily consumption, and that has been our position since the ARED study was published. This does not mean, however, that we should not supplement with reasonable amounts of zinc, because it is vital to good health. And it is beyond appalling that they continue to use zinc oxide when there are much safer forms of zinc.”
We will continue to stay in touch with Ms Troyer for updates. Meanwhile, anyone taking the AREDS formula containing high amounts of zinc may want to consider switching to a multi-supplement with less.
Miscellaneous
Visually Impaired, Not Blind
The word “blind” is becoming increasingly less associated with age-related macular degeneration (AMD). MD Support reports that the majority of press releases and media broadcasts are now using the more correct terms “low vision,” “vision loss” or “visual impairment.”
Dan Roberts, director of MD Support, spent five months tracking virtually every public article and news broadcast about macular degeneration appearing on the Internet. As of November 10, 2006, he had found that 58% were still using the word “blind” as a description of AMD. As of March 10, 2007, he reports that the ratio had dropped to 42%, and the trend is continuing downward.
April 2007
Nutrition
Beta-carotene May be Ineffective in Fighting Macular Degeneration
Vitamin A in the form of beta-carotene has been found to be ineffective in slowing the progression of maculopathies such as age-related macular degeneration (AMD). This conclusion was derived from a follow-up study derived from the Physicians’ Health Study 1, which found neither benefit nor harm from 12 years of supplementation with beta carotene on cancer or cardiovascular disease. The follow-up study found that beta-carotene also had no significant effect on development of AMD, advanced maculopathy or maculopathy with or without vision loss.
The landmark age-related eye disease study (AREDS) found that a high-dosage formula of antioxidants and zinc, which included beta-carotene, was effective in slowing the progression of AMD. Beta-carotene, however, was not studied separately. The supplement might still be indirectly effective when taken in combination with other antioxidants, but that has yet to be researched.
As always, MD Support recommends that patients consult with both their general physicians and eye care specialists before making any significant changes in dietary supplements.
May 2007
Pharmacology
Lucentis and Avastin comparable as first-line treatments
N.J. Sund, M.M. et al reported to the ARVO meeting that further evidence of the safety and efficacy of intravitreal Avastin and Lucentis as first line monotherapy in the treatment of neovascular AMD. In their study, both drugs showed comparable results as first line treatments in terms of visual acuity outcomes in the short term. (abstract title: Efficacy of Intravitreal Bevacizumab (AvastinTM) vs. Ranibizumab (LucentisTM) as First Line Monotherapy for the Treatment of Neovascular Age-Related Macular Degeneration?)
Surgery
Cataract surgery and AMD
R.C. Milton, et al reported to the ARVO meeting that their large clinic-based longitudinal cohort study showed no clear evidence of an association between cataract surgery and the geographic atrophy form of advanced AMD. Patients undergoing cataract surgery can probably be reassured that the surgery is unlikely to increase their risk.
(Abstract title: The Effect of Cataract Surgery on the Development of Geographic Atrophy)
Research
Blue light and macular degeneration
A.R. Wielgus, et al reported to the ARVO meeting that blue light exposure promotes the oxidation of A2E and iso-A2E in rodent eyes. A2E is a blue light absorbing retinal chromophore that increases with age. The researchers noted that, as A2E oxides are toxic to retinal tissue, this may partially explain blue light induced retinal injury. This study is another in a growing compendium of research pointing to a connection between blue light and macular degeneration. (abstract title: Blue Light Induces A2E Oxidation in Rat’s Eyes)
Ultraviolet radiation and macular degeneration
A.J. Harring, et al reported to the ARVO meeting that subjects who were exposed to higher levels of ultraviolet (UV) radiation for the majority of their lifetimes were less likely to develop neovascular (wet) AMD than subjects who were exposed to lower UV levels. These results should not be confused with studies that have shown a connection between UV exposure and the increased risk of corneal disease and retinal cell damage.
(Abstract title: UVR Exposure and Risk of Neovascular Age-Related Macular Degeneration)
Drusen reduction
T.I. Metelitsina, et al reported to the ARVO meeting that inreased blood circulation may result in the resolution of drusen. This conclusion was drawn from observing eyes that show more drusen reduction following laser treatment and noting that those eyes have larger increases in choroidal circulatory parameters. The 23 subjects were drawn from the Complication of Age-Related Macular Degeneration Prevention trial (CAPT). (Abstract title: Laser Induced Resolution of Drusen Is Associated With Increases in Choroidal Blood Flow)
Effects of cardiovascular disease and hypertension on AMD
U.Chakravarthy, et al reported to the ARVO meeting that cardiovascular disease is strongly implicated as an etiological factor in the development of choroidal neovascularisation in a proportion of older adults. The researchers emphasize the importance of control of blood pressure and cholesterol, avoidance of smoking and maintenance of a normal body weight. This is further evidence linking cardiovascular disease and high blood pressure with wet macular degeneration.
(Abstract title: Cardiovascular Disease and Hypertension Are Strong Risk Factors for Choroidal Neovascularisation)
Nutrition
Dietary antioxidants ineffective in preventing AMD
E.W. Chong, et al reported to the ARVO meeting that there is insufficient evidence from the published literature to support the role of dietary antioxidants, including the use of dietary antioxidant supplements, for the primary prevention of early AMD. This conclusion was based upon a systematic review and meta-analysis of 9 prospective cohort studies and 3 randomized clinical trials of dietary antioxidants and dietary antioxidant supplements.
Pooled results from prospective cohort studies suggested that vitamin A, vitamin C, zinc, lutein, zeaxanthin, beta-carotene, cryptoxanthin and lycopene have little or no effect in the primary prevention of early AMD, and Vitamin E had a modest protective association for early AMD. The three randomized clinical trials did not show antioxidant supplements to be protective in the primary prevention of early AMD.
This study shows that antioxidants and zinc will not prevent the onset of AMD. It does not dispute the original ARED study, which showed that antioxidants and zinc can moderately lower the risk of developing the wet form of the disease in patients with advanced AMD.
(Abstract title: Dietary Antioxidants and Primary Prevention of Age-Related Macular Degeneration:A Systematic Review and Meta-Analysis)
NSAIDs and progression to advanced AMD
H.Sen, et al reported to the ARVO meeting that there was no significant association between regular systemic use of anti-inflammatory drugs (aspirin or NSAIDs) and the likelihood of developing advanced AMD (defined as the development of either neovascular AMD or central geographic atrophy). This conclusion was drawn from data collected over a median of 11 years from the original AREDS group.
(Abstract title: Systemic NSAIDs and Age-Related Macular Degeneration: The Age-Related Eye Disease Study (AREDS)
Statins may not prevent CNV
C.A. McCannel1, et al reported to the ARVO meeting that there is no consensus in the literature on whether or not statins offer protection against progression to the advanced stage of AMD (defined as the development of either choroidal neovascularization (CNV) or central geographic atrophy (CGA) in patients with early AMD. Data gathered from subjects in the Complications of Age-Related Macular Degeneration Prevention Trial (CAPT) do not support the concept of a strong protective effect of statins against the development of CNV. CAPT statin users did have a lower risk of GA, but not to a statistically significant degree.
(Abstract title: Statin Use and the Incidence of Choroidal Neovascularization and Geographic Atrophy in the Complications of Age-Related Macular Degeneration Prevention Trial (CAPT)
Phototrop continues to show effectiveness
J.Feher, et al reported to the ARVO meeting that “prospective case studies on the long term effects of Phototrop treatment (see www.mdsupport.org/library/phototrop.html) confirmed the previous clinical trial’s data on an initial improvement followed by stabilization of visual functions in early AMD. Surprisingly, fundus alterations may further improve after one year of treatment, as shown by three different objective methods. All of these findings give further support to the efficacy of the long term use of Phototrop in early AMD patients, as early AMD may continue to improve even after 12 months of use.”
(Abstract title: Long Term Improvement of Early AMD Treated With Phototrop: Prospective Case Studies)
Miscellaneous
Statins, smoking and wet AMD
A scientific abstract presentation at the 2005 AAO meeting, “Reduced Risk of Progression to Exudative ARMD with Statin Use” (Gregory R Nettune, MPH, et al) presented research finding that “the risk of exudative AMD was increased by smoking and reduced by use of statins. Further, the duration of statin use up to four years was associated with an increasing degree of protection.” The study also found that current smoking or smoking within the past 20 years led to a 6-fold increase in risk of conversion from dry to wet AMD. No difference was found in subjects who had not smoked in 20 years.
As reported at the 2006 ARVO meeting, research from Duke and Vanderbilt University Medical Centers (published in the online edition of the American Journal of Human Genetics, March 6, 2006) has discovered that a version of the LOC387715 gene significantly increases the risk of developing ARMD in tobacco smokers. This is an unusual instance wherein genetics and environment can combine to create a disease risk. The other example related to ARMD is the CFH gene and how its effects are related to the body’s immune system.
A more recent study (Johanna M Seddon, et al, “Association of CFH Y402H and LOC387715 A69S With Progression of Age-Related Macular Degeneration,” JAMA, April 2007) showed that common variants of CFH and LOC387115 increased the risk of progression to advanced AMD. The variants, called Single Nucleotide Polymorphisms (SNP), independently increased the risk of progression from early or intermediate stages to advanced stages of AMD by 2.6 times (CHF) and 4.1 times (LOC387715). The presence of both risk genotypes, when combined with smoking and body mass index of 25+, increased the AMD progression risk 19-fold.
Low vision devices and reading speed
N.Nguyen, et al reported to the ARVO meeting that their study confirms the importance and efficiency of visual rehabilitation with low vision aids. The researchers evaluated magnification requirement and reading ability before and after administration of appropriate low-vision aids to 484 patients with different stages of AMD.
Mean reading speed [words per minute] was 20±33 before and 72±35 after administration of low vision aids. With appropriate low-vision aid, patients increased their reading speed an average of 53±19 wpm. Patients with a lower magnification requirement (less than 10X) showed a higher increase of reading speed (59±31 wpm) than patients with high magnification requirement (40±18 wpm).
The researchers concluded that “all patients benefited greatly from the rehabilitation measures in optimizing reading ability and therefore qualify of life. In face of the increasing number of elderly patients with AMD, rehabilitation should start as early as possible.”
(Abstract title: Reading Ability Before and After Administration of Low-Vision Aids in Patients With Age-Related Macular Degeneration)
The Future
Encapsulated cell technology
Phase 2 of the encapsulated cell technology (ECT) trial to deliver growth factors to the retina is ongoing. Phase 1 trials (see www.mdsupport.org/library/summary2006.html) showed an unexpected improvement in vision. This 2 year trial is being funded by the National Eye Institute in hopes that slow delivery of the growth factors will preserve photoreceptor function and serve as a drug delivery system for other conditions, as well. For more information, see www.mdsupport.org/library/ect.html.
AREDS 2
A second age-related eye disease study (AREDS 2) is looking at changes to the original formula. Potential changes include lowering the amount of zinc, eliminating beta-carotene, and including the carotenoids lutein, zeaxanthin, and DHA/Omega 3 (fish oil). The study, which will collect data from about 4000 subjects over the next five years, is being sponsored by the National Eye Institute.
AREDS2 is now recruiting participants with bilateral large drusen or advanced in one eye. All participants will be followed annually with a minimum follow-up of 5 years. For more information, see http://www.areds2.org.
Implantable miniature telescope
VisionCare’s Phase clinical trials of the IMT have been successfully completed, and a 2-year follow-up has revealed no serious safety issues. The company now looks forward to completing the regulatory review process on the way to marketing the IMT for public use by the end of this year.
New Drug In Trials for Dry AMD
Fenretinide (ST-602), a drug that has been used to treat certain cancers, rheumatoid arthritis, acne, and psoriasis, has been found to also slow the production and accumulation of a toxin that leads to vision loss in macular degeneration patients. The toxin, called A2E, is a byproduct of vitamin A, the formation of which encourages production of waste deposits called lipofuscin. These deposits accumulate in the retinal pigment epithelium (RPE), interfering with the RPE’s ability to nourish the photoreceptors.
Early research showed that A2E accumulation was decreased by 60% in mouse models after 28 days of treatment with fenretinide. Sirion Therapeutics, Inc. is now running FDA-approved multi-center studies using up to 225 AMD patients as subjects. If the drug proves effective with dry AMD patients, it may then be used off-label for treatment of Stargardt’s disease, a juvenile form of MD.

Age-related Macular Degeneration Does Not Cause Blindness

A revealing opinion poll sponsored by MD Support shows that a strong majority of people affected by AMD do not think of themselves as blind, and they do not want the term to be used to describe their visual impairment.

The pronouncement is well known: “age-related macular degeneration (AMD) is the leading cause of blindness among senior citizens.” Along the same line, some organizations declare that “macular degeneration causes blindness,” or that donating to their cause will help them to put an end to macular degeneration’s “blinding impact.”

Macular Degeneration Support (MD Support), one of the world’s leading patient advocacy organizations, strongly emphasizes that people can see with AMD. They are not, therefore, going to go blind, and telling them so can be seriously hazardous to their psychological health, quality of life and motivation to seek treatment. In a study of 51 newly-diagnosed AMD patients researchers found that 33% met criteria for a depressive disorder at 6-month follow-up.1 Another study compared AMD patients who had unilateral central vision loss with those who had bilateral legal blindness. The unilateral group showed greater emotional distress despite comparable levels of disability, leading investigators to speculate that expectation of future worsening vision can cause severe emotional anguish and fear.2

The purpose of the MD Support survey was to gather opinions from AMD patients about the indiscriminate use of the word “blind” as a description of their condition. The survey was taken between June 1 and June 19, 2006, and the results have been distributed to patients, advocacy organizations and eye care professionals worldwide.

Survey population

253 people responded to the opinion survey. 87 were members of the AMD Internet community, 7 were members of a live support group and 159 were residents from a total of 12 retirement centers across the United States. Each respondent reported having one of the following AMD conditions:

  • Dry, unilateral (1%)
  • Dry, bilateral (31%)
  • Wet, unilateral (11%)
  • Wet, bilateral (31%)
  • Wet in one eye, dry in the other (26%)

The respondents represented a diversity of years with the disease:

  • Under 1 year (15%)
  • 1-4 years (37%)
  • 5-10 years (39%)
  • Over 10 years (9%)

Survey Design

The survey presented five statements, requiring the respondent to indicate either agreement or disagreement. An interactive checklist-type form was accessible during the first half of June 2006 on the MD Support web site.3 The statements were also read aloud to retirement center groups during an Internet conference meeting of MD Support’s National Low Vision Support Group on June 8, 2006. This venue required response by a show of hands.

The statements were as follows.
A. The term “blind” means no functional (useable) eyesight in either the peripheral or central fields.
B. AMD affects the macula, or central vision, only.
C. AMD does not, by itself, cause blindness.
D. As a person with AMD, I DO NOT consider myself to be blind in either or both of my eyes due to AMD alone.
E. The terms “blind” and “blindness” should NOT be used without qualification to describe a person with AMD.
Space was provided on the online form for comments by the respondents. Comments from the retirement center participants were passed along by the group facilitators.

Results and observations

The following percentages of respondents agreed with the five statements.
Percent of Agreement Per Statement

Statement
% in Agreement
A 91%
B 95%
C 93%
D 91%
E 93%

Observation 1: As expected, not all respondents agreed with the established definition of blindness (A).
Possible rationales:

  1. Differences in interpretation of the definition.
  2. The perceived need to use the word for dramatic effect, whether or not it is used accurately.

Basis for rationales from respondents’ comments:

  • “I do feel blind when I can’t see without aids.” (Rationale #1)
  • “The word “blind” has many degrees. I am not totally blind, but I am partially blind and am considered legally blind.” (Rationale #1)
  • “’Blind’ is the only word the population acknowledges.” (Rationale #2)
  • “If ‘blind’ or ‘blindness’ is not used an an outcome of AMD, some people will not understand the total effect AMD will have on their sight.” (Rationale #2)

For a discussion of the established definition of “blindness,” see “What is the established definition of blindness?” below.
Observation 2: A small percentage of respondents did not agree with the established definition of AMD.
Possible rationales:

  1. Liberties taken with the established definition of AMD.
  2. Misunderstanding of the physiology of the disease.

Basis for rationales from respondents’ comments:

  • “Losing one’s central vision changes the quality of life. Central blindness is blindness, no matter how you try to phrase it.” (Rationale #1)
  • “Macular degeneration doesn’t just destroy [macular] cone cells. It destroys [peripheral] rod cells, too.” (Rationale #2)
  • For a discussion of the established definition of AMD, see “What is the established definition of age-related macular degeneration (AMD)?” below.

Observation 3: Exactly half of those who disagreed that AMD, by itself, does not cause blindness (C) also disagreed with the definition of blindness (A). 71% of those respondents think of themselves as blind, even though they retain useable peripheral vision.

Possible rationales:

  1. Liberties taken, or confusion about, the established definition of “blindness.”
  2. Misunderstanding of the physiology of the disease.

Observation 4: Statements D and E are personal opinions which can be strongly held and which are the main focus of this survey. Of the group who consider themselves to be blind (D), only 79% agreed with the established definition of “blindness,” but 92% agreed with the established definition of AMD. 81% were aware that AMD affects only the macula.
Of the group who disagreed that the word “blind” should be avoided in connection with AMD (E), 38% agreed with the definition of “blindness,” while 85% agreed with the definition of AMD. Only 46% were aware that AMD affects just the macula.

Possible rationales:

  1. Liberties taken, or confusion about, the established definition of “blindness.”
  2. Misunderstanding of the physiology of the disease.

Observation 5: Comparing responses by number of years of experience with AMD yielded some interesting variations, as shown by this table.

Percent of Agreement by Experience Group

Experience Group
A B C D E
1. Under 1 year 91% 100% 73% 82% 82%
2. 1-4 years 84% 92% 88% 84% 84%
3. 5-10 years 89% 100% 89% 96% 93%
4. Over 10 years 86% 86% 71% 57% 71%

The group reporting 5-10 years with the disease (Group 3) was significantly more likely (96%) than the other groups to not think of themselves as blind (D) and more in disagreement (93%) with the other groups about using “blind” in connection with AMD (E).
Possible rationales:

  1. Realization over time that AMD will not rob one of total sight.
  2. Eventual attenuation of the original dread that a diagnosis can cause.

Observation 6: Group 4 (over 10 years experience) showed a significantly lower percentage of agreement with nearly all of the statements (Statement A being the exception) than the other three groups.

Possible rationales:

  1. Frustration with the length of time with the disease.
  2. Frustration with aging in general.
  3. Blindness from a secondary undiagnosed condition.

Discussion

The results of the MD Support opinion survey show that 93% of people with AMD are averse to the use of the word “blind” in connection with their condition. 91% of them do not consider themselves to be blind, 93% know they will not go blind from AMD and 93% think the word by itself should not be used in connection with AMD. These are convincing statistics that are now available for the first time to eye care professionals, patient advocacy organizations and public service agencies. Hopefully, the message is clear and will be heeded.

More important, however, is that people with AMD can use these findings to defend themselves against those who tell them they will go blind. To dispense that kind of false information is irresponsible. It can have grave emotional consequences that can lead to serious depression and even thoughts of suicide.4 It also blurs the line between people who are visually impaired and people who are truly blind, a distinction which all governmental agencies, experienced AMD patients and especially blind people recognize.

Here are comments from selected respondents who have spoken well for the majority opinion.

1. “I resent the media and advertising agencies using this term for the sole purpose of shocking the consumer to pay attention to their causes.”

2. “I prefer ‘visually impaired’ over ‘blind.’ The word ‘blind’ to most of us would mean black blind–seeing nothing at all. This could be very frightening to someone who is first diagnosed.”

3. “I definitely do not consider myself blind. The director of my local support society feels that I should refer to myself as blind. I prefer my own description, ‘vision impaired.’ I disagree thoroughly and completely with the society’s stance.”

4. “When I went to renew my driver’s license, the people at the local MVD wrote ‘blind’ in the space for my left eye. I was insulted, because it was not totally accurate.”

5. “If people are told I’m blind, they don’t understand when they see me navigate the room fairly effortlessly, even though I can’t read, drive or play golf. I think the term ‘blind’ confuses people.”

6. “When first diagnosed, the only word I heard was ‘blind.’ I have since learned that I am going to be visually impaired–never blind. I went through much pain and despair because of incorrect or poor terminology. That was so unnecessary.“

7. “Blindness is a powerful word. For the newly diagnosed it can be like a bomb going off with disastrous emotional results.”

8. “Should we not also think about those who are really blind? I am not denying that AMD presents a good deal of problems. I am not denying that some of us really suffer. But, does that give us the right to claim fellowship with those who are really blind?”

9. “I still have good usable vision in both eyes, even though I have wet AMD and significant geographic atrophy in both eyes. When I read articles or comments that say I will go blind from MD, it frightens me and my family.”

10. “Although I agree to all these things at this time, it is the opposite of the way I felt for two years, which caused severe panic attacks. Even the RN told me I could go blind.”

11. “I consider myself visually handicapped, but certainly not blind. I resent reading publications that say AMD is the leading cause of blindness. This is not true, and it is time that we take a stand.”

Even though more than 90% of the AMD community agrees with these people, we must not ignore the few who do not. Obviously, several needs must be met in order to address the welfare of everyone living with this disease.

  • Complete and accurate patient education about personal pathology and prognosis.
  • Public accounts and media presentations that are accurate, truthful and noninflammatory.
  • An uninterrupted continuum of care from diagnosis to successful self-management, with the goals being greater confidence, continued independence and a higher quality of life.

This ends the report on the opinion survey. Following is discussion and clarification of the established definitions of blindness and AMD.

What is the established definition of blindness?

Six reliable online sources were consulted:

American Heritage Dictionary Online
dictionary.com
encarta.msn.com
www.medterms.com
wordnet.princeton.edu
www.websters-online-dictionary.org

These sources confirm that “blind” means “without sight,” “unable to see,” “sightless,” “unseeing,” “without useful sight” and “unsighted.” It should be noted that www.medterms.com varies from the theme by adding that blindness is also “without part or all of the sense of sight.” This is contradictory (or unclear at the least), as it immediately follows that resource’s definition, “unable to see.”

Mixed in with these definitions, one will sometimes find the statutory definition of blindness (commonly called “legal” blindness), which was established to determine who may take advantage of governmental assistance and benefits. The definitions of legal blindness by these sources are variations of the American Heritage Dictionary version: “Having a maximal visual acuity of the better eye, after correction by refractive lenses, of one-tenth normal vision or less (20/200 or less on the Snellen test). The Social Security disability program (among other ruling agencies around the world) adds that a person is considered visually disabled when he has “a limitation in the field of vision of your better eye, so that:

(a) [he has] a contraction of peripheral visual fields to 10 degrees from the point of fixation, or

(b) the widest diameter of [his] visual field subtends an angle no greater than 20 degrees.”

The Royal National Institute for the Blind (RNIB) says that “Interestingly, for statutory purposes, the definition of blindness is occupational rather than medical.” 5 For that reason, statutory (legal) blindness is not part of the issue presented here.

Section 64 of the National Assistance Act 1948 (UK) defines blindness as “unable to perform any work for which eyesight is essential.” This is in line with the medical definitions quoted above, but arguments to the contrary are often set forth. Dr. Kenneth Jernigan spoke for the opposing viewpoint in Who Is Blind?6 when he wrote “blindness can best be defined not physically or medically but functionally or sociologically.” A sociologic definition of blindness, or one that is based upon an individual’s own perception of functionality, may be a convenience, but it would be insufficiently arbitrary. A person who has been recently diagnosed with AMD does not want to hear “blind” used indiscriminately. He wants a clear appraisal of his prognosis, and that appraisal, by definition and by virtue of its unnecessarily incendiary impact on his emotions, should not include the word “blind” without qualification. The people of the AMD community have spoken clearly in favor of this standpoint, and they will either speak out against or ignore any attempts to use the word indiscriminately.

What is the established definition of age-related macular degeneration (AMD)?

AMD is a progressive disease of the retina wherein the light-sensing “cone” cells in the central area of vision (the macula) stop working and eventually die. The disease is thought to be caused by a combination of genetic and environmental factors, and it is most common in people who are age sixty and over.

At its worst, macular degeneration will damage only central vision, which arises from the macular area, comprising less than 5% of the total retina, but responsible for about 35% of the visual field. This means that an affected person will find it difficult or impossible to read, drive, or recognize faces. The peripheral vision, however, is left untouched. Many affected people move about with no assistance at all and, with the help of both visual and non-visual devices, many lead independent, productive lives.

This description of AMD is from the MD Support web site.7 It is typical of the descriptions found everywhere, most notably the emphasis on the fact that the peripheral field is spared by the disease and that blindness is not a result. Here are examples from four other respectable sources.

1. “Macular degeneration alone does not result in total blindness. Most people continue to have some useful vision and are able to take care of themselves.” (Kellogg Eye Center, University of Michigan)8

2. “This disorder results in the loss of central vision only — peripheral fields are always maintained. Although loss of ability to read and drive may be caused by macular degeneration, the disease does not lead to complete blindness.” (U.S. National Library of Medicine & the National Institutes of Health) 9

3. “Patients can be told that although central visual loss is common, peripheral visual loss is rare.” (American Academy of Ophthalmology)10

4. “ARMD never causes total blindness. Persons with ARMD, even its most severe form, have normal ‘peripheral’ or side vision. If you know of someone who has ARMD and has lost peripheral vision, this is not because of ARMD but because of another eye condition.” (University of Alabama Department of Ophthalmology)11

As the MD Support survey showed, most people who have had AMD for several years know that, unless a secondary condition affects their peripheral field, they will maintain a fair amount of independence and unassisted mobility. They may have defects in their visual fields (blind spots, distortion or scarring) that prohibit seeing with that part of their retinas, but personal experience and the facts of medical science assure them that AMD will not blind them. They are, instead, affected by “visual impairment,” also called “partial sight” or “low vision.” Their condition may even be described as “central blindness” or even “legal blindness,” which are two examples of how the word “blind” can be suitably qualified.

Most agencies officially recognize the difference between a blind person and a visually impaired person. The United Kingdom’s Disability Rights Commission Act 1999, for example, differentiates between “blindness” and “partially sighted.” The Blind Citizens of Australia differentiates between “blind” and “visually impaired.” These distinctions, like the definitions of “blind” and AMD, are well-known in the eye care field and by those who provide information and support for patients. The only reason, therefore, that someone in such a position would purposefully interchange the terms would be for dramatic effect. As one organizational representative put it, “A patient will get over the shock of being told she will go blind once she learns the truth. It won’t hurt her physically, and it’s a way of getting her through our door.”

If the word “blind” needs to be used to inspire a greater fear of AMD, then the disease must not be frightening enough in itself. That is good news for those who have it, and it happens to be true. AMD can be initially devastating, but the patient can ultimately learn that it is not something to fear. It is neither painful nor life threatening, and it can be well-managed with proper education and treatment.

Conclusion

AMD patients must continue to arm themselves with the facts in order to not be misinformed and misled. They must maintain a healthy skepticism by not accepting what they read and hear without examining the evidence and weighing the author’s motivation. Equally important, they must assist in the proper education of others. In this case, that means resisting use of the clinically inaccurate term “blind” when describing AMD. “Visually impaired” may not be as dramatic, and it may require definition for those who are new to the term, but it is at least accurate. It is most certainly easier for the newly-diagnosed to live with.

The AMD community must continue to encourage contributions to research and the necessity of eye exams for everyone. It must, however, find ways to emphasize those points without resorting to fear tactics and unsupported health claims. AMD is not the leading cause of blindness in senior citizens. It is, however, the leading cause of vision loss. That is more accurate and more acceptable to the patient population. No one knows their visual capabilities better than the people who have lived for awhile with AMD. They have now had their say, and it might be to everyone else’s benefit if they are heard.

References:
1 Alliance for Aging Research. Independence for older Americans: An Investment in Our Nation’s Future. (Accessed Nov. 7, 2006 at www.agingresearch.org/brochures/independence/welcome.html.)
2 Rovner BW, Casten RJ, Tasman WS. Effect of depression on vision function in age-related macular degeneration. (Arch Ophthalmol. 2002;120:1041-1044.)
3 www.mdsupport.org
4 Brody BL, Gamst AC, William RA, et al. Depression, visual axis, comorbidity and disability associated with age related macular degeneration. (Ophthalmology 2001; 108 : 1893-1900)
5 “What’s In a Name?” (Royal National Institute for the Blind: www.rnib.org.uk/xpedio/groups/public/documents/ Visugate/public_terminog.hcsp)
6 “Who Is Blind?”, Kenneth Jernigan” (www.haverford.edu/ods/who_is_blind.html)
7 “What Is Macular Degeneration?”, Dan Roberts (www.mdsupport.org/library/md_description.html)
8 www.kellogg.umich.edu
9 www.nlm.nih.gov
10 www.aao.org/aao/education/library/ppp/upload/Age-Related-Macular-Degeneration.pdf
11 www.eyes.uab.edu