Vision Access Volume 16, Number 2

FCCLV Awards Scholarship

Kerri Salter resides in the big little town of Loxahatchee, Florida. She is the 2009 Florida Council of Citizens with Low Vision scholarship winner. Email comments to Barbara Grill, President FCCLV, grillbh@comcast.net.

Kerri Salter is your average teenager. She has great friends and family, and she likes school. The only thing that is anywhere from average is the fact that she is legally blind. She has been dealing with this disadvantage all her life and has learned to overcome many challenges that she has faced.

At 18 years old, Kerri has experienced events in her life that others her age can't say they have. She had to hear the news that she can't drive because of her vision, while her friends were off getting their driving permits and licenses.

Kerri is a very determined young adult, as she gets ready for college in the fall. "I am ready to face the world and show that I can succeed with a disability."

With her passion for writing, Kerri was accepted into Lynn University in Boca Raton, Florida. She just graduated from Seminole Ridge Community High School on May 20th and already knows what she wants to do with her life. For years it has been her dream to become a journalist and one day become famous for her articles. Her overall goal is to work in New York. After 4 years of working on her high school's newspaper staff she has the confidence to go for her dream.

Kerri's hope of being a journalist has inspired her friends to realize that just because you have a disability doesn't mean you can't succeed at life. Live for your dreams, live for the future, and live for you.

Metropolitan Council of Low Vision Individuals, MCLVI
"Looking Close, Looking Good"

Just prior to opening day at the brand new Yankee Stadium, a final "outreach session" was held by the ball club's management with representatives of a variety of disabilities groups. People at this meeting discussed further the facility's physical features and attendance policies. Board members Artie Elefant and Todd Walerstein, and chapter member Joel Ziev, were active participants. The following week, chapter president Ken Stewart and board member Rick Morin, joined Matt Sapolin, commission-er of the NYC Mayor's Office for People with Disabilities, in a follow-up meeting at the new stadium with the team's attorneys.

Chapter members continue to be active participants in the monthly meetings of the Manhattan Borough President's Disabilities Task Force. At the April meeting, MCLVI led the task force adopting a resolution urging accountability from the city's Department of Transportation on the installation of accessible pedestrian signals at important intersections around the city.

National Capitol Citizens with Low Vision, NCCLV

On April 23rd, NCCLV members and friends had the pleasure of hearing from Dr. Stan Tempchin. He is the new Low Vision Director at Medical Faculty Associates at George Washington University in Washington, DC. He showed us some of the new hand-held assistive technolo-gies. Dr. Tempchin reminded us of the importance of wearing sunglasses and eating right to reduce the chances of getting cataracts and macular degenera-tion. We welcome him to our community! As usual, a highlight for many of us was meeting for dinner afterwards to catch up with friends.

For further informa-tion about our group, contact Barbara Milleville at ncclv@yahoo.com or 703-645-8716

An Invitation to Talk about Low Vision with Dr. Bill
By Bernice Kandarian

Recollections of First CCLVI Newsletter Editor By George Covington

Farewell to Cassette Tapes

Science and Health

Stem Cells and the Treatment of Retinal Degeneration: Hopes and Reality By Dr. Valeria Canto-Soler, PhD

Editor's Note: Dr. Canto-Soler is Assistant Professor of Ophthalmology at Johns Hopkins University

Last November I had the great pleasure of participating in the Mid Atlantic Convention of the American Council of the Blind. In that opportunity, I was kindly invited to share with the attendees the latest scientific advances towards the development of therapies to treat retinal degeneration. It was one of the most exciting experiences I have had so far, not only because the session developed into a very interactive conversation where I could answer many important questions raised by the participants, but even more because of all I learnt from their own personal experiences. This article is a result of that very enriching interaction and it hopes to bring answers to all those that could not be present at that meeting.

Before describing possible therapeutic approaches to treat retinal degeneration it is important to understand that retinal degenerative diseases are a very diverse group. This diversity results from the fact that: i) retinal dystrophies are caused by abnormalities in genes; ii) there are many genes that can cause retinal degeneration; for example more than 60 genes have already been identified as causative of Retinitis Pigmentosa; iii) in many cases more than one gene is affected in a particular patient, and even more, different patients with similar clinical manifestations may have different combinations of affected genes; iv) although all degenerative diseases lead to the death of photoreceptors, the "light-sensitive" cells of the retina, the cell type in the retina that is directly affected by the abnormal gene(s) varies among different forms of retinal dystrophies; v) some retinal dystrophies start very early in life (some even at the time of birth) and some appear latter in life; vi) some progress very rapidly and some have a much lower rate of progression; and vii) some of them share some clinical manifestations but at the same time they differ in others. This diversity clearly indicates that there may not be just one treatment or methodology that can be applied to all types of retinal dystrophies, rather every form of retinal dystrophy may require a different treatment.

What are the possible therapeutic approaches to treat retinal dystrophies?

From what we have said so far it is clear that the approach will mainly depend on the type of dystrophy and the stage of progression of the disease in each particular case. For example, in a patient in which the disease is still at an early stage, when there are still enough photoreceptor cells as to be able to maintain visual function, the best approach would be to help the remaining cells to survive. This could be done by, for example, replacing or correcting the abnormal gene that is causing the disease through "gene therapy", thus allowing the retinal cells to function normally and prevent further photoreceptor loss. An alternative approach to directly modifying the cells by gene therapy would be to modify the environment in which the cells live. This could be done for example by providing them with special molecules, let's call them "survival factors", that would help them to survive even though the genetic abnormality has not been corrected. A few clinical trials to test safety and effectiveness of these types of treatments are currently underway, and although it is still too early to tell, the results so far are very encouraging.

We face however a very different scenario in the case of patients in which the disease is more advanced. In situations like this, where most of the photoreceptors have already been lost, the type of approaches mentioned before would be of no use because even if the remaining photoreceptors survive, they would not be sufficient to maintain visual function. Thus, what is needed in these cases is a way to replace the lost cells with something that can "take over" their function. Here is where stem cells really come into play. The hope is that, at some point in the future, we could use stem cells to generate new photoreceptors to replace those that have been lost as a consequence of the disease and take over the job of capturing and processing visual information.

What are stem cells?

Our body is composed of millions of different cell types: neurons, cardiac cells, skin cells, retinal cells, etc. These cells have acquired, over time, a very specific and exquisite shape as well as molecular composition (the genes and proteins that they express) that allow them to function in a very concrete manner. Cells with these characteristics are defined as differentiated cells. This process of differentiation has occurred over time, following sequential steps during embryonic development, and in some cases it continued even after birth. In contrast, stem cells are cells that have not gone through this process of differentiation. Stem cells are cells that are in an undifferentiated state, that can divide and produce more stem cells, and that under appropriate conditions, can stop dividing and give rise to many (or in some cases all) of the specialized differentiated cell types present in the adult body. How many types of stem cells are there?

In general terms stem cells can be classified as "embryonic" and "adult" stem cells and both share the three characteristics mentioned above: undifferentiated, self-renewing and capable of differentiating into different cell types. However, when considering the potential of stem cells and their possible clinical applications it is very important to consider not only the similarities but also the differences between them. These differences and similarities can be summarized as follows:

Embryonic stem cells are the first embryonic cells that appear during development and they originate all the tissues and organs of the embryo. Adult stem cells are found in adult tissues like for example bone marrow, brain and cornea and their function is to maintain and repair the tissue in which they are found.
Embryonic stem cells have the potential to originate all the different cell types found in the body while adult stem cells are thought to be capable of originating only some cell types, usually the cells corresponding to the tissue where they reside (for example stem cells from the bone marrow may originate only blood cells and stem cells from the brain may originate only the cell types found in the brain).
Embryonic stem cells proliferate and grow very aggressively and when transplanted they usually develop into "teratomas" (tumors formed by a mixture of different tissues such us bone, teeth, cartilage, etc). Adult stem cells proliferate in a more controlled manner and they do not form "teratomas".
When transplanted, embryonic stem cells are likely to induce immunogenic rejection. In the case of adult stem cells, since they can be obtained from the same patient to be treated, they are free of potential immunogenic complications.
No successful therapies in humans using embryonic stem cells have been developed yet; the first clinical trial using embryonic stem cells to treat spinal cord injury was approved last January, but it will take a few years before we know if it succeeds. Adult stem cells have already been used to treat patients with several diseases including leukemia, corneal diseases, heart failure, diabetes, traumatic brain injury, Parkinson and even the replacement of windpipe.

There is still one additional, very important, consideration to keep in mind. The fact that embryonic stem cells are only found in the embryo and for a relatively short period of time, necessarily creates an ethical problem: to be able to obtain and use human embryonic stem cells for clinical therapies we need to destroy human embryos. The question that necessary follows is: Is the human embryo a human person? We could argue that a human embryo should not be considered a human person, but the truth is that science so far has not been able to demonstrate that the human embryo is not a person. On the contrary, there is much more evidence to support that a human embryo is a human person than to sustain that it is not. But even if we decide that it is still not clear, that we can not certainly know whether an embryo is a human person, the next thing we need to ask ourselves is: Is it "licit" or "reasonable" to run the risk of "killing" human embryos to be able to use their cells for research or clinical treatments? It is not my intention to give an answer to these questions here, but as a biomedical scientist I am convinced that these are not trivial questions and that all of us, as responsible members and "makers" of the society in which we live, need to carefully consider them.

Where do we stand with stem cells in the vision field?

Diseases of the cornea are an example in which adult stem cell transplantation has been very successful. In the periphery of the cornea, the transparent tissue that covers the front of the eye, there is a population of stem cells called limbal stem cells. These cells have the function of generating new corneal cells to repopulate the cornea and maintain it healthy. For several years already these cells have been used to treat conditions in which loss of vision is caused by injury or abnormalities of the cornea. During limbal stem cell transplantation, stem cells are obtained from a donor, in most cases the healthy eye of the patient to be treated, and transplanted to the affected eye. Transplanted stem cells grow and differentiate into the corneal cell type, repopulating the cornea. Key elements for the success of this approach are the fact that limbal stem cells are relatively accessible for their isolation and that the surface of the cornea is a very homogenous tissue, composed of a single cell type, with a very simple organization, and thus repopulation of the tissue does not require very sophisticated cell differentiation programs.

But the situation in the retina is very different. The retina is composed of seven major different cell types. These cell types are organized in layers, every layer containing very specific cell types. To make it even more complicated, all these different cells need to establish very specific and refined connections between them, and ultimately with the right structures in the brain, in order to function and produce a visual image. This means that for stem cells to be able to restore vision upon transplantation, they need first to be integrated in the right layer of the retina; second, to differentiate into the right cell type acquiring the right morphology and functional machinery; and third to establish the right connections with the other cells in the retina. Every one of these steps occurs during normal development and is regulated by exquisitely tuned cell and molecular mechanisms. To be able to use stem cells to repopulate a degenerating retina we need to instruct stem cells to perfectly recapitulate what normally happens during development. Unfortunately, we still don't know enough about these mechanisms as to be able to properly instruct stem cells to follow them, and even though experiments in animal models are promising, complete differentiation and anatomical and functional integration of stem cells in the host retina has not been achieved yet.

The discovery of adult retinal stem cells in humans a few years ago produced a great deal of excite-ment, mainly because it is reasonable to think that it may be easier to instruct these cells to differentiate into retinal cells than in the case of embryonic stem cells. Adult retinal stem cells reside in the ciliary body, a structure located at the periphery of the retina, in very close contact and continuity with it. Adult retinal stem cells can be isolated and grown in culture and the hope is that investigators will, at some point, find ways to induce them to differentiate and repopulate the retina replacing the cells that have been lost during retinal degeneration. Part of the excitement associated with the discovery of these cells is the thought that they could provide a source of stem cells derived from the same patient to be treated and thus avoid immunological complications. However, there is still no surgical procedure that would allow obtention of these cells without causing undesired damage to other eye structures or tissues. The only possible source of retinal stem cells at this point is from post-mortem donors, which still presents the limitation of potential immunological rejection. In addition, there are other problems that need to be overcome before we can use adult retinal stem cells in the clinic: i) the number of stem cells present in the adult retina is very low and it is very difficult to isolate them; ii) isolation and culture of these cells is not trivial and many investigators have failed in their attempts to do it; and iii) we still don't know how to induce them to differentiate and become the type of cell that needs to be replaced in the retina.

Are the limitations of embryonic and adult stem cells ever going to be overcome?

After all we have said, this is probably the most logical question. Fortunately, the answer is "most probably yes, and we are getting closer and closer..."

This optimistic answer is based on the fact that science is developing very fast and things that were thought impossible a few years ago are a reality today. One example of how science in the stem cell field is incredibly advancing, is the development of what is called "induced pluripotent stem cells" or simply iPS cells. iPS cells are fully differentiated cells from the adult body that have been reprogrammed to go back in development and become stem cells again. The development of this technology makes it possible today to obtain skin cells from a person through a standard skin biopsy, a very simple and well established procedure with no risk for the patient, and culture these cells in the laboratory under specific conditions that make them become stem cells. This discovery is of incredible significance for the potential development of feasible stem cell-based therapies, because it overcomes some of the most important limitations associated with embryonic and adult stem cells. There is no destruction of human life or any possible physical risk for the donor. They offer an unlimited source of stem cells and they have the potential to differentiate as any of the cell types found in our body. But probably one of the most important advantages is that they open the door for what is called "autologous cell-based treatments": treatments in which the transplanted cells come from the same patient to be treated. It is very exciting to think that, hopefully in a future not so far, we will be able to generate an unlimited source of patient-specific stem cells that could in turn be transplanted back into the patient. This technology would even allow treating the cells by gene therapy to correct gene abnormalities before transplantation. At this point many laboratories in the world, including our own, are working on the development of techniques that can efficiently induce iPS cells to differentiate as retinal cells, and specifically as photoreceptors. The results are very promising and the field is advancing at a quick pace.

Concluding remarks:

This is in brief and simple words the state-of-the-art regarding the potential of stem cells for the treatment of retinal degeneration. We haven't found a cure for these diseases yet, but we are certainly much closer. It wouldn't be appropriate to say that we will find ways to cure them in just a few more years, but we are surely on the right track... it is just a matter of time.

Dr. Bill Answers Questions about Low Vision

Editor's Note: This is a summary of CCLVI's first teleconference with host Dr. Bill Takeshita, Director of Optometric Services at the Center for the Partially Sighted in Los Angeles, CA. Dr. Bill answered questions from participants about their eye conditions. Dr. Bill stated that his answers and comments are in no way a substitution for a complete examin-ation by an eye doctor and are not intended to diagnose or make any specific recommendations for the treatment of any eye disease.

Here is a question to start: Where can I go to receive vision therapy to treat strabismus, amblyopia, and nystagmus in New York? The State University of New York has an excellent optometry program that is known for their excellent in vision therapy. It is located on 42nd Street in Manhattan and they have one of the best residency programs in vision therapy in the country. Vision therapy can treat crossed or turned eyes (strabismus), amblyopia (blurred sight without any sign of eye disease), and it can reduce nystagmus, the uncontrollable shaking of the eyes. Vision therapy is very controversial and it is important to understand that vision therapy treats vision conditions and it does not cure autism, attention deficit disorder, or dyslexia. However, many people with these disorders also have a vision problem and when their vision problems are corrected, their symptoms often improve.

How does one know if he or she has glaucoma?

Glaucoma can only be diagnosed by having a complete eye examination. Often, glaucoma has no symptoms of blurred sight, eye pain, or reduced peripheral vision in the early stages of the disease. The earlier the detection, the better the prognosis. A complete eye exam including peripheral field tests, eye pressure test, and a dilated retinal examination should be done. Eye drops and medications to treat glaucoma can have side effects but they are absolutely necessary to prevent blindness.

Is there a difference in the pressure of the eyes of women and men? Can the pressure of the eye fluctuate during the day?

Intraocular eye pressure is often higher in women than men and pressure may fluctuate during the day. The pressure of the eye is often higher in the morning and this is why doctors often want to measure the pressure at different times of the day. The important thing to remember about glaucoma is that each person has an eye pressure that is best suited for his or her eyes. Just because a friend has a pressure that is different than yours does not mean that it is better. Everyone over 40 years of age should have a glaucoma test.

What is Gyrate Atrophy?

Gyrate atrophy is a genetic abnormality that causes the abnormal production of an enzyme that affects the retina and can affect other parts of the body. Since gyrate atrophy affects peripheral vision first, field testing is very important to monitor the progression of the disease.

I have inverse retinitis pigmentosa and I have problems adapting to different lighting conditions. Do you have recommendations on special sunglasses that are not too dark?

Inverse RP: The cells in the retina need to re-generate a certain chemical in order to adjust to different lighting conditions. Some grey and black sunglasses are too dark. A polarized lens called the Melanin lens may be something your doctor can show you. It has helped many of my patients with this condition.

Can stem cells cure my blindness?

There was a report that stem cell injections in China have successfully restored vision. These reports must be carefully reviewed by researchers and larger studies must be performed for accuracy. A stem cell has the potential of becoming a retinal cell or other cell of the eye. However, the process is extremely complicated and numerous studies are being performed presently throughout the world. It is recommended that one proceed with extreme caution before participating in such a program. Discuss the treatment options with your specialist.

I have high myopia and macular degeneration and would like a suggestion on lighting to help me read.

If you have AMD and high myopia, lighting is important. Track lighting and low voltage halogen Solux MR-16 lights with 2 lights angled to prevent shadows can be helpful.

I had cataract surgery with implants in both eyes. Do I need to wear sunglasses?

Yes. It is important to protect the retina from the ultra-violet light and the short wave-lengths of light by wearing sunglasses. The normal lens of the eye that was removed during cataract surgery normally filters these harmful rays. When it is removed during the surgery, the eye does not have the same level of protection. You may ask your doctor if he or she implanted lenses into your eyes that filter the ultraviolet radiation. A brown or amber lens can be very protective if you have retinal disease.

Is it important for people with low vision to continue to have peripheral vision testing even if they already have glaucoma or retinitis pigmentosa?

Yes. These tests inform the doctor about the speed of the progression of your vision loss. In some cases, the doctor may prescribe alternative medications to slow down the progression of the disease.

Can stem cells repair the damage done to my optic nerve?

This is not known at this time. There have been some isolated reports that stem cells can treat optic nerve hypoplasia. However, it is important to understand that a stem cell must be able to turn into an optic nerve neuron and this is very difficult. The optic nerve contains millions of fibers and each fiber must connect to the precise region of the brain. Even if there were only 400 fibers to connect precisely, the improper connection of the nerve would not allow vision. Similar to the way that your video tape player or DVD player will not work when you connect one of the three wires improperly. Thus, vision is very complicated because it involves millions of nerves that must be connected precisely.

Sunglasses for People with Low Vision

Editor's Note: This is a summary of a CCLVI sponsored teleconference presentation by Dr. Bill Takeshita, Director of Optometric Services at the Center for the Partially Sighted, Los Angeles CA.

There are many types of sunglasses. Filters or sunglasses are important for people with low vision.

Visible light from either the sun or from light bulbs looks white. If this light is too bright, it causes discomfort or even pain to the eyes. Invisible light can even be dangerous and harmful to eyes. Ultraviolet light causes sunburn and bleaches clothing. It is a powerful light. Ultraviolet light can damage the retina. It is correlated with macular degeneration and other diseases. It predisposed people to cataracts. Therefore it is important to wear sunglasses to protect your eyes.

To be effective, sunglasses do not have to be dark. Recommendations regarding what color to choose will be determined by their effect on clarity, contrast, color vision as these factors relate to particular diagnoses or eye conditions.

When cataracts are removed, the natural protection against ultraviolet light is lost. People who have had cataracts removed require glasses to filter the ultra-violet radiation. Fortunately, there are now intraocular lens implants that are inserted into the eye after a cataract ahs been removed. Newer implants have UV protection and are made by Alcon.

If your eyes are bothered by overall brightness, you can reduce the brightness by wearing grey lenses. These do not distort color vision. Painters often use grey lenses.

Color vision varies with eye conditions. People with diabetic retinopathy find it hard to see blue. Blue ink looks black. Yellow sunglasses help people with diabetic retinopathy to distinguish colors.

People with macular degeneration have reduced contrast vision. Seeing steps and curbs can be a challenge. Because they enhance contrast, brown or amber lenses are useful here.

Most people need more than one pair of sunglasses. Early morning or late afternoon light is not as bright as afternoon light. So amber or light brown glasses are good when sunlight is not at its brightest. Grey polarized lenses which reduce reflected glare are helpful in the afternoon when light is very bright. If frames have side shields, they prevent light from coming through the corners.

Cocoon lenses fit on top of existing glasses

People with diabetic retinopathy have problems distinguishing color, have reduced contrast and they are bothered by bright light and glare. For indoors, yellow tinted lenses filter blue fluorescent light. Amber lenses help with problems caused by shadows. Grey lenses decrease the effects of bright light.

People with RP, Lebers, and Achromatopsia might be helped with Corning CP5-550 lenses. These are red colored lenses. People with these conditions can't tolerate direct sunlight. And if glasses are too dark, they can't see at all. Glasses with red lenses increase brightness and decrease glare.

Some shields fit over regular glasses. Those made by NoIR come in a variety of colors and intensities. But they do not come in prescription lenses.

Clip-ons often scratch regular lenses

UV shields fit over glasses and come in popular colors. Solar shields come in different colors--yellow to help with fluorescent lights, orange for cloudy days, grey or plum for bright light.

Slip in filters fit behind existing glasses

Transitions lenses are clear indoors and turn grey or brown outdoors. But they do not become dark in cars and they are expensive. Transitions lenses can be customized, for example, yellow lenses for indoor use and brown for outside use.

On rainy days distortions occur from reflected glare. Light often scatters causing glare. Colored glasses filter out short waves that scatter the most. Glare comes from light reflected off water, windows and cars. Yellow or amber polarized glasses decrease reflected glare. Such glasses can be yellow, pink, light blue or green. For greater contrast in colors use yellow, orange, or brown lenses.

When cataracts are removed, the natural protection against ultraviolet light is lost. People who have had cataracts removed require glasses to filter the ultra-violet radiation. Fortunately, there are now intraocular lens implants that are inserted into the eye after a cataract has been removed. Newer implants have UV protection and are made by Alcon.

Is there a correlation between light exposure and depression? Does wearing colored filters contribute to feeling depressed? Sunglasses have not been associated with depression and wearing sunglasses will not cause depression. People who do not receive as much light exposure have a higher prevalence of depression, a disorder labeled seasonal affective depression. This condition is treated by increasing the exposure to light. Generally, it is the shorter wavelengths of light that seem to be more beneficial but short wavelengths of light can be dangerous for the retina. If you are going to use a light box for seasonal affective depression, do not look at the light and wear glasses that filter the short wavelengths of light if you have a retinal problem.

If you ski or are at the beach, wear sunscreen and sunglasses

Alargen contact lenses, which are plastic, come in UVC, UVB, UVA; some filter out A, some filter out ABC. Most quality lenses cost about $30.

CRT monitors emit more radiation than LED monitors which use less electricity. Eyes do not become as dry with LED monitors. Blinking your eyes corrects dryness.

With regard to blind spots, the brain fills in what the eye does not see.

Research in Low Vision By Dr. Bill Takeshita

Editor's Note: This is a summary of our third teleconference with Dr. Bill Takeshita, Director of Optometric Services at the Center for the Partially Sighted, Los Angeles, CA.

To appreciate the complexity of this topic, let's consider the structure of the eye and how the eye works. The eye is the receiver of light. The retina is at the back of the eye. Behind the retina is the retinal pigment ephethelium, RPE. This RPE is like dry wall that covers the blood vessels that nourish the eye and take away waste products. Blood vessels are like pipes covered by dry wall--the RPE. The wallpaper is the retina.

Now let's consider how the eye functions. Light falls on the retina which is composed of millions of cells. Inside each cell is a pigment that converts light to electrical signals. These signals are sent through the optic nerve to the brain for processing.

What goes wrong to cause damage to the retina?

The eye is very complicated and therefore there can be many causes of vision loss. First, the retinal cells may die because of genetic mutations that result in the abnormal production of proteins in the cells. Inflammation, oxidation and free radicals from metabolic processes can damage the cells. Light exposure, and toxins can all damage the cells of the retina. Another cause of cell death in the retina occurs when the RPE cells become damaged and the RPE is no longer able to remove waste and provide nutrients to the retina. Damage to the RPE also removes the physical barrier between the blood vessels and the retina. For people with wet macular degeneration and diabetic retinopathy, blood vessels form under the RPE and leak blood into the retina, causing damage to the retina.

What have recent studies about smoking and nutrition shown concerning eye health?

It has been shown that smoking causes oxidated and inflammatory stress to the eye. People who smoke are 40 times more likely to develop macular degeneration.

AREDS, Age Related Eye Disease Study, showed that vitamins and antioxidants keep cells healthy. They keep free radicals from damaging the eye. Beta carotene, vitamin A, C, E, and zinc are helpful. People with moderate to severe macular degeneration who took PreserVision(r), a product offered by Bausch and Lomb, were 25 percent less likely to experience progressive vision loss. The formulation for smokers contains no beta carotene.

Studies have shown that certain foods influence vision health. Spinach, fish oil (omega 3) from tuna, mackerel, sardines and other dark fish are helpful. Nuts like walnuts, almonds, and flax seeds are beneficial. It has also been found that statins and baby aspirin decrease the progression of macular degeneration. In the AREDSII study Lutein is being studied to determine if it is more effective than beta carotene in slowing the progression of macular degeneration. Lutein is found in dark green vegetables.

What about genes?

Each cell has genes. Genes are made up of DNA which control the formation of proteins; genes can also inhibit the synthesis of proteins. When there is a genetic mutation, the wrong protein is produced and this affects the function of the cells. Leber's Congenital Amarosis and RP are examples of diseases where the cells do not function normally because of genetic mutations. Scientists discovered a breed of dogs whose rod and cone cells did not function normally. A healthy gene in a virus was injected into the eyes of these dogs and they regained vision. In humans clinical trials are being conducted. Clinical trials begin with Phase 1 trials which determine if the treatment is safe. Phase 2 trials determine the efficacy of the treatment. Phase 3 trials reproduce Phase 2 trials on a larger population. To qualify as a participant in gene therapy research, you have to have an eye condition for which a gene has been identified. It is known that many genes cause RP. To determine if you have one of the genes that have been identified, see your doctor, who will take a blood sample to determine if you have an identified gene.

What types of medications are available for treating eye diseases?

In wet macular degeneration and diabetic retinopathy, abnormal blood vessels grow under the RPE. Scientists have addressed how to stop these blood vessels from forming. VEGF (vascular endothelial growth factors) floating in blood vessels weaken these vessels and cause them to leak and bleed. Scientists have found ways to prevent blood vessels from leaking. Laser treatment stops the leakage but results in a burn spot or scar on the retina. Photodynamic therapy using Visudyne stops the leaking but new blood vessels spring up. Avastin, a drug used in the treatment of cancer, has been found to stop the formation of new blood vessels. Another drug, Lucentis, gets into the blood vessels of the retina improving vision and holding it steady. The problem with these treatments is that they involve injections into the eye; and this is scary to patients and risks infection and retinal detachment. Injections are given every 3 weeks. A strategy to decrease the number of required injections is to use VEGF-Trap which attacks and smothers VEGF molecules; not as many injections are required. Studies are also being performed to investigate the benefits of using radiation along with Avastin and Lucentis to reduce the need for injections to 18 months intervals.

Are there medications that can preserve my present level of vision?

There are ongoing clinical trials that are studying the effects of compounds that may keep the cells of the retina healthy. Ciliary Neurotrophic Factor (CNTF) is a chemical that appears to protect the health of the cells for people with retinitis pigmentosa and dry macular degeneration. In this study, they inject a small pellet in the eye and the CNTF is constantly delivered to the retina. There are also other studies that are looking at drugs such as Alphagan and whether it protects the cells of the retina.

What studies are being performed on dry macular degeneration?

Recent discoveries have found that dry macular degeneration has a genetic component and gene therapy may be helpful in the future. For many people with dry macular degeneration, there is too much of a protein complex called Membrane Attack Complex which attacks the cells of the retina. Gene therapy may be able to alter the production of this compound and protect the cells of the retina. Other studies are looking at ways to reduce the formation of a substance called drusen under the RPE. Drusen is a fatty substance found in the eyes of dry AMD and the use of medications such as Fenretinide and Copaxson may reduce the formation of drusen.

How can stem cells help the blind?

Stem cells are specialized cells that can develop into any type of cells, including a heart cell, liver cell, brain cell, or retinal cells. Stem cells can be cultured from a fetus but the use of fetal cells is controversial. Another way to obtain stem cells is through the bone marrow of adults. Numerous studies are being performed to culture stem cells and to attempt to produce retinal tissue, optic nerve tissue, and corneal tissue. In animals, stem cells have been injected into the retinas and the stem cells have developed into retinal cells. Another study has shown that the stem cells inserted into the retinas do in fact send electrical signals; stems cells have developed into cornea tissue in humans and have allowed the possibility of corneal transplants in humans. Stem cell research is very exciting and is being performed throughout the world. People should be cautious about going to foreign countries for stem cell treatments because it is not known whether the long term results of these treatments are safe.

To find out about clinical trials in which you may wish to participate: www.clinicaltrials.gov

To download lots of podcasts including seminars: www.airsla.org
To read about research projects on specific retinal diseases: www.blindness.org

To contact Dr. Bill Takeshita directly: Phone: 818 705-5954
Email: bill@drbillfoundation.org

People

Meet Connie Case
By Joyce Kleiber

My name is Connie Case. I work at Fabric Cut. We are wholesalers of fine fabrics. This family owned business is housed in five buildings and we have many sales reps throughout the country.

I have a long history in working with fabrics. I used my mother's sewing machine to sew clothes for my dolls. I began working for J.C. Penny sewing draperies and eventually was promoted to head my department. With Pennys I moved to Florida and then to Texas. And when their sewing operation was being eliminated, I applied for work with similar companies. Fabric Cut in Tulsa Oklahoma hired me.

Then eight years ago when I was 52, I couldn't read anymore! I worried that my job as manager of the contract department was in jeopardy. I thought there was something wrong with my brain. I couldn't function at work. I had headaches at the end of the day as the result of straining to do my job. Print looked grey, as if it was fading into the paper. Because I feared the loss of my job, I was afraid to say anything about my problem.

After visiting doctors I was diagnosed with cone/rod dystrophy, an eye disease that results in the loss of contrast. I was referred to a low vision doctor who had a closed circuit TV (CCTV) in his office. I found a low vision store and tried many things they offered. They showed me Zoomtext which helps me use my computer.

I went to my human resources department. When they learned what I needed in order to do my job, they bought me a CCTV and Zoomtext. I learned to use these tools by myself. I searched the internet and found CCLVI, an organization that connected me with others who were dealing with vision loss. I also found that the Southwest Retinal Institute in Dallas Texas is doing research on cone/rod dystrophy. I have been on their waiting list for 4 years. I want to get the treatment they offer to stop the gene that causes cone/rod dystrophy from progressing, thereby stopping further vision loss.

People at work have been very helpful. Now they even forget that I don't see well. They hand me something to read, and I tell them 'I'll be right back.' I go to the CCTV in my office to read the material they gave me. Other people don't hand me a printed page, they read it to me. I've found that my memory has improved. I supervise 13 employees who take orders from and offer services to customers. I do have long term disability benefits, but I don't want to stay at home. The people with whom I work are like a family, and I would miss these relationships.

At home, guests notice that my chairs get closer and closer to my TV. Soon I'll be able to turn the TV on with my toes. I'm grateful for recorded books. I listen to books when I clean my house and when I walk my dog, a white Maltese named Punky. If the book is really good, we take a very long walk. At the YMCA I use the elliptical machines and I lift weights. This gives me a chance for physical activity that does not depend on my vision.

I get frustrated when I have to fix things with small parts. I tried to put a little shelf together. The shelf was black and it had little black screws. This was so difficult, I waited for someone else to assemble my shelf. I gave away my sewing machine. When the needle breaks, it's too hard to replace the needle. It's not worth it.

For shopping I use a hand magnifier to read prices and labels.

I have lost more vision. I keep a notebook of resources that I may need if I lose even more vision. But I feel blessed that I have found ways to keep on functioning. Even if I lose vision, I will not lose my determination. I plan to work and live independently as long as I can.

Advocacy

Health Care Reform Proposals and Vision Loss, By Frank Welte

Editor's Note: Frank Welte is Director of Advocacy and Governmental Affairs for the California Council of the Blind. He addressed his remarks in a letter to the U.S. Senate Finance Committee on Health Care Reform. All of us can write, email or phone our senators and representatives regarding these important matters.

I am writing in response to your call for public comment on proposals under consideration by the United States Senate to reform our nation's health care system. I wish to draw your attention to health care disparities that currently exist with respect to Americans who are blind or visually impaired. Several issues are particularly important to us.

First, as you strive to address disparities in the American health care system, it is important to address the health care needs of blind and visually impaired Americans separately from the needs of others with disabilities. There is a temptation by policymakers to think of people with disabilities as a single, homogeneous population, and to craft a one size fits all solution to their health care needs. That approach won't work. Health care needs are specific to particular types of disabilities. The following paragraphs address specific concerns of people who are blind and visually impaired.

Second, blind and visually impaired Americans need to be able to independently use medical devices to manage their own health. Such devices include, but are not limited to thermo-meters, blood pressure monitors, blood sugar measure-ment equipment, etc. Cost containment is a primary goal of the health care reform movement. Any meaningful national health care cost containment strategy is bound to include programs to empower individuals to maintain their own health and to independently manage their illnesses as much as possible. In order to do so, people will use various medical devices to monitor their health, administer medica-tions, and perform other therapeutic activities.

In most cases, medical devices required for use by consumers are designed in such a way that people with vision impairments cannot operate their equipment independently, even though the modifications that would allow them to do so, such as displays showing enlarged characters or enabling spoken output, are readily achievable. In those few cases where accessible devices are available on the market, they are often expensive, and they are generally not covered by health insurance. This failure to make accessible medical equipment available to people with visual impairments at reasonable prices may seem cheaper in the short run, but in the long run it will result in an increase in healthcare costs because people with vision loss will either have to rely on extra home health care services to compensate for their lack of accessible equipment that would allow them to manage their medical conditions indepen-dently, or they will fail to properly manage their illnesses. So they will end up in expensive hospitals, suffering with more severe conditions.

Third, our health care system must provide the means for people who are blind and visually impaired to identify and properly administer their medication. It is well known that in this country many people receive the wrong medication or they take their medicine improperly. This places the health of individuals at risk. The resulting increase in illness represents a signify-cant cost to our health care system. This risk is magnified for people who cannot see well enough to easily distinguish their medications visually and to easily read the printed instructions and warnings provided with their medications. Some health care providers have addressed this problem by supplying medication to visually impaired patients at no extra charge and upon request in containers that patients can distinguish without sight and with large print or audible instructions. These practices should be followed throughout the entire health care system.

Fourth, in order to be restored to full health, people who lose their vision need more than surgery and drugs. They need to receive rehabili-tation services from professionals specifically trained to provide those particular services. These services include training in the skills that blind people use to travel independently in their communities, known as orientation and mobility skills, training in methods of communicating and sharing information in a non-visual way through the use of braille and through the use of speech-enabled computers and other speech-enabled devices, and teaching non-visual techniques for personal care and housekeeping.

Unfortunately, our current health care system doesn't adequately address this need. If a person loses his or her sight, Medicare, Medicaid and private insurance policies may pay for eye surgery, and they might help pay for some medications; but they will fail to pay for the services of orientation and mobility instructors or for the services of blindness rehabili-tation teachers.

By contrast, if someone breaks his or her hip, our health care system will not only help pay for the hip replacement surgery and for the medications that are required with respect to that surgery, but it will also provide for the necessary physical therapy that the patient will need after the surgery. There's no excuse for failing to address blindness in the same comprehensive way. This makes no sense. Do blind people have any less need to be taught how to walk than do people recovering from strokes? Do people who have lost the use of their eyes have less of a right to be retrained in personal care tasks than do people who have lost the use of a hand?

Fifth, there is a severe shortage of professionals trained in blindness rehabilitation, and our health care system perpetuates this shortage by its failure to compensate the important work of these professionals in the same way that it compensates physical therapists and occupational therapists, even though physical therapists and occupational therapists are not trained to provide blindness rehabilitation services. There are over 100,000 occupational therapists in the United States, but there are less than 3,000 professionals trained to provide blindness rehabili-tation services nationwide. Medicare and Medicaid reimburse physical therapists and occupational therapists for their services, but those programs don't reimburse orientation and mobility specialists, blindness rehabilitation teachers or low vision therapists. How can we hope to ease our nationwide shortage of qualified blindness rehabili-tation professionals if our health care system fails to even recognize their existence and to compensate them at the same levels that other rehabilitation professionals are compensated?

Finally, our health care system must address the need of people who are blind or visually impaired for prosthetic devices and durable medical equipment. A system that fails to pay for prosthetic eyes, magnifiers, or the other equipment that people who are blind and visual impaired need to compensate for their sight loss is as absurd as a system that would send amputees home after treatment without their prosthetic limbs or a hospital that would send a person home after lung surgery without providing for his or her oxygen equipment.

Issues in Determining Legal Blindness Status-Some New SSA Guidelines
By Randy Jose, OD, FAAO,

Editor's Note: Dr. Randy Jose is Diplomate Emeritus in Low Vision and Professor Emeritus, University of Houston.

Determining if a patient is legally blind has always been a difficult problem for clinicians working with patients who are visually impaired. For years, our standard working definition of legal statute has been that a patient must have 20/200 or less corrected acuity in the better eye or have visual fields reduced to no more than ten degrees radius in any meridian. This seems pretty straight forward. But as many vision rehabilitation clinicians have discovered, there is a lot of flexibility in making these measurements. There are also many problems with trying to determine patient need with a single number off of an acuity chart. The biggest failure of the traditional system of determining who will benefit from additional services or financial support is that the impact of the vision loss on the individual's ability to perform vocational, educational or daily living tasks is not considered. It is not the impairment that needs to be considered when determining services or developing an education or rehabilitation plan, rather, it is the handicapping impact of the vision loss for the individual due to the impairment. So why doesn't the government simply measure the handicap? Again, this sounds simple. But human personality and behavior is far too complicated to establish any reasonable consistent standards that would reflect the visual handicap and prevent significant abuse. After all, we are talking about humans and money!!!

In dealing with legal blindness, things have gotten better in the last forty years. It used to be almost a death sentence. Patients went to their primary care doctors because they could not see well, usually at near distance. They went in for an examin-ation-refraction to get a new pair of bifocals and came out with a diagnosis of Senile Macular Degeneration and the pronouncement that there was nothing more that could be done to improve their vision. It was devastating; they not only have this degeneration thing in their eye (which many thought was cancer and they might die and that is why the doctor did not spend any more time explaining what this condition was) but to add insult to injury, they were told they were SENILE!!! And then there was this comment dropped about them being legally blind. Talk about a bad day at the office!!!

The additional problem is that most patients did not know the difference between legal and illegal blindness!! So of course, they thought or felt as though they were blind. I have had people come to my clinic after such a pronouncement and actually act blind due to this diagnosis, even though they were able to read the larger numbers on my chart. This is another problem with measuring visual impairment. There are a myriad of charts available to clinicians to measure acuity and they do not always end up with the same number. Lighting in the room, anxiety in testing, previous lighting prior to the exam, pupil size, language skills and a dozen other parameters in the room set up can significantly change the outcome of the acuity measurement. None of these measurements are wrong; they reflect the acuity as measured under those conditions. This is kind of like blood pressure. I never seem to get a decent pressure in the doctor's office but am very normal when taken in other settings. There are standard conditions for testing but they are not usually followed in most clinical settings. Thus when you get differing acuity measurements in different doctors' offices, do not worry. These variances in acuity measurements are not wrong; they just reflect the acuity at that time of day, in that place.

The bottom line is that the traditional system would often allow those who were not legally blind into the system and others who needed services were denied. There was no malice in this situation as the inconsistencies were recognized but no one had any answers as to how to better define impairment and handicap so those who needed services could benefit. It is important that this issue be addressed as the Federal Guidelines for Visual Disability are followed by just about all insurance and service organizations and have an impact on society, individuals and government budgets.

A study was commissioned to look at better ways to measure visual impairment as it pertains to the individual's eligibility as a legally blind person for services and financial support. The committee expanded its study to look at several aspects of visual impairment besides the traditional acuity and visual field. They even looked at trying to develop some scientific measurements of visual handicaps or functional losses related to the inability to perform specific tasks. While this was a new frontier in looking at legal blindness, the committee too came up with a mountain of barriers trying to develop measurements that are fair and consistent between individual responses. Studies of visual behavior, secondary to vision loss, have far too many variables and do not allow for good control studies. However, the committee did develop a new approach to using differing aspects of visual impairment that can be measured in the clinical setting with consistency and better reflect on how an individual will/should respond to vision loss. The final report provided clinicians with some guidelines and formulas to incorporate acuity, contrast sensitivity and visual field loss as measured with our newer automated visual field testing equipment. These guidelines are published in the following book authored by the National Research Council and the Committee on Disability Determination for Individuals with Visual Impairments. This book is titled Visual Impairments - Determining Eligibility for Social Security Benefits, 2002 and published by National Academy Press, Washington, DC.

The main value of this book is the review of the study with the background on the rationales for making changes to the guidelines for the determination of legal blindness and of course the new formulas. However, I personally found it to be one of the best written books on visual impairment that I have read in my 40+ years of working in the field of low vision and vision rehabilitation. I do believe all eye care doctors in the vision rehabilitation field and many patients would benefit from reading the book. I am sure that after reading several of the chapters, people who have vision loss would have a much better understanding of their own visual impairment. Understanding what is going on with your own vision is a major step forward in being able to manage your own visual impairment and start you own rehabilitation compensations or performance modifications. The chapters on visual handicaps or performance of activities would be most helpful for most to read, including the patient, caretaker or eye care provider.

The new guidelines will not have any significant impact on changes in who is legally blind and who it not. I did address the committee as a reviewer about the issue of the possibility of people who are now legally blind and receiving services, losing these services when re-evaluated under the new guidelines. I did not get a specific answer but my understanding (and it is logical) is that many individuals have a permanent visual impairment and often do not need annual reevaluations. Secondly, the old traditional guidelines still exist. As far as I know at this point in time, the new guidelines are not statutes or have legal legislative status but are measurements that are accepted as comparable to one who has an acuity of 20/200 with the best corrected eye or a visual field of twenty degrees diameter or less. More importantly, the new guidelines make it easier to combine the visual field and acuity loss in making a more comprehensive assessment of the visual impairment. One can add the deficits of field and acuity loss in the traditional system but it gets too time consuming and complicated for most clinical assessments. The new system uses the visual fields normally run on patients and charts which are commonly available and not expensive if the clinic/doctor does not have them in the office. Again the LogMar charts are commonly used in most eye care offices. These charts show much greater consistency between measurements made from one office to another and also from one clinic visit to another.

The new guidelines try to address the quality of visual performance by adding Contrast Sensitivity test results as part of the legal blindness impairment. Contrast sensitivity is a measurement of the amount of acuity or detail an individual loses when the contrast between the detail they are trying to see and the background is reduced. Most of us with 20/20 can walk into a bright room and within seconds can adjust and maintain good detail vision. We can walk into a dark room and at first lose detail but then adapt to a much better level of detail vision. A visually impaired person may have a modest acuity or loss of detail vision when under ideal clinic testing conditions. However, a slight change in dimming of lights will result in a severe change in vision loss and a much greater inability to see things under the slight variation in lighting or contrast of objects being viewed. Basically, this person does not live in the ideal conditions of the clinic and enjoy the optimum acuity measured in this ideal setting. Contrast Sensitivity tests allow for a better assessment of what this individual's acuity loss is under daily living conditions. In my experience working with legal blindness issues over the last couple of years I have not seen any abuse of this system and have not had any patients or consumers of the Division of Blind Services be denied care due to these new guidelines. In fact, I do believe that many individuals who are not justifiably getting the care and support they need and have visual impairments that are consistent with the same handicapping affects of being legally blind with 20/200 acuity or having 20 degree visual fields. This new system will better recognize these individuals and allow them to participate in needed service programs. This additional assistance will allow the individual to maintain a sense of "normality and independence" within the confines of their lifestyle. I feel this is a very fair system and certainly a much better way to reflect with clinical measurements those who will truly benefit from and deserve the extra assistance that comes with being legally blind. These new guidelines will be an asset to all those needing and providing vision rehabilitation services.

Assistive Technology

Assistive Technology Visual Aides in the Work Place
By Marcel Hogervorst

Improved Access to Digital Download Books

On April 30, the National Library for the Blind and Physically Handicapped (NLS), a division of the Library of Congress, entered the first phase of its transition to a digital playback system, as well as releasing an upgraded version of its pilot download website. Beginning with the testing of 5,000 digital players by NLS patrons in their homes, NLS is preparing to launch full-scale production and distribution of 26,000 machines per month this year, ensuring continued access to reading materials for more than 500,000 registered users.

New to the NLS program is a state of the art online component, called the Braille and Audio Reading Download (BARD) site. This new site for patrons of NLS who are blind and physically handicapped now provides better than ever access to digital audio books and magazines. The BARD digital books and magazines are in the ANSI/NISO Z39.86 2002 format, the specification for navigable digital talking books. Readers may press buttons on the NLS digital talking-book player and compatible players to jump from chapter to chapter, section to section or page to page. This ability to quickly navigate the book structure, combined with the high-quality human recording, make the new digital NLS download books one of the most significant advancements in the provision of talking books for people who are blind and who have low vision.

Gilles Pepin, CEO of HumanWare says, "With BARD, NLS brings its library into the home of its patrons. Not only do NLS patrons now have access to thousands of digital books but they can find their book of interest and download it in minutes indepen-dently and from the comfort of their home computer."

HumanWare manufactures the popular Victor Reader Stream, a DAISY NISO compatible digital talking-book player designed in collaboration with the National Federation of the Blind (NFB), which several thousand NLS patrons already use to play the new digital NISO books. The NLS digital books on the BARD site are protected by encryption and compatible digital book players must be authorized by NLS for eligible patrons to play the books.

The ability to press buttons on the Victor Reader Stream to easily jump between chapters, pages, or magazine articles, like the NLS digital talking-book player, provides a rich audio book reading experience never before experienced by audiocassette readers. For reference books such as cookery books the user can even enter a page number from the table of contents on the Stream telephone-style number pad and the playback will move immediately to that page. With the NLS player, Victor Reader Stream, and the NLS BARD books, users now have immediate access to thousands of books and can navigate them just like they would navigate the equivalent print book's structure.

The new BARD download audio book and magazine service is available free of charge to eligible individuals in the U.S. and to citizens living abroad who are blind or who are physically handicapped. Eligible individuals must have access to a computer and high-speed Internet connection. And they must have a free NLS digital talking-book player or own an authorized compatible digital talking-book player such as HumanWare's Victor Reader Stream. To learn more about the new BARD service visit https://nlsbard.loc.gov/ or www.humanware.com

Vision Access

Contents

Happy 30th Anniversary, CCLVI!