Living with Retinal Degeneration and Legal Blindness

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Living with Retinal Degeneration and Legal Blindness:
How those with retinal eye disease cope with the challenges of everyday life By Ann Chiappetta

Legal Blindness is defined by the Social Security Act of 1935 as: visual acuity for distance of 20/200 of less in the better eye after correction or visual acuity of more than 20/200 if the widest diameter of visual field subtends an angle no greater than 20 degrees (1).

Over 85,000 Americans lose their sight each year, 15,000 from eye injury and the remaining 70,000 from retinal eye diseases. The majority of legally blind people are over the age of 55. In the U.S. 19,000 people become blind due to eye injury, and nearly a million have some kind of impairment resulting from a previous eye injury.

No worldwide estimates are available on the incidences of blindness internationally, but by examining these instances individually by country, an approximation can be given as to how many people are affected by retinal disorders or eye injuries worldwide. It is estimated there are over one million people around the world each year lose their sight. In Nigeria, for example, 25 percent of those accidentally blinded are school children. In a developing country like Nigeria, a minor injury to the eye can ulcerate and cause serious visual loss because there may not be proper medical care available (2).

The leading cause of Legal Blindness in the United States and other first world countries is from retinal degeneration. There are approximately a dozen related eye diseases that fit into this category including macular degeneration (m.d.), diabetic retinopathy, and retinitis pigmentosa (r.p.), to name a few. These and other related diseases and syndromes affect the retina, the key structure of the eye. If the retina fails, the eye is blind. The retina sends visual information through the optic nerve to the brain. It is made up of nerve tissue, about as thick as a piece of blotting paper, tearing as easily as paper when it is wet. Under a microscope, there are ten layers of tissue. The outermost layer is called the pigment cell layer. The other nine layers are neural tissue. The layer closest to the pigment cell layer houses the cones and rods, or light sensitive cells that take in and transmit information through the optic nerve to the brain. The retina is transparent and benefits from both its own blood-vessels and the choriod blood supply surrounding it. If the retina is injured or diseased, visual loss will follow (3).

For the purpose of this paper, I will focus on r.p. and how it affects the individual. R.p. is progressive and has no known cure. It is a genetic retinal disease that affects the cones and rods in the retina, causing the retina to give the wrong signals to the brain resulting in loss of periphery and light sensitivity, night blindness, and in most cases, total visual loss. Nationwide, over 100,000 people have lost their sight from r.p., myself included.

15 Participants all in stages of moderate to severe visual loss answered the following questionnaire posted electronically via email at this email address: rplist@maielstron.stjohns.edu
Survey on Legal Blindness
Your age: Ages ranged from 30-70.
Age you became legally blind:
This indicates how long the affected person has known about their retinal condition. This time ranged from less than one year to fifteen years.

What eye malady (s ) do you suffer from?
All participants suffer from r.p.

What is your visual acuity( ex. 20/400 )?
Most participants had fair to good central vision, but their visual field suffered greatly, the average periphery is less than 20 degrees total, confirming Llgal blindness.

Do you use a cane?:
Half of the participants used a cane, all of those using their cane relied on it at night more than during the day, indicating night blindness as the most predominant condition of r.p. besides loss of periphery.

What other visual aids do you use around the home/office? List anything from sewing needles to computer equipment.

Visual aids ranged from magnifiers (the most common), to complex reading aids, like a Xerox document enlarger. Two of the participants own guide dog though most of the participants are not considered affected enough to be considered a candidate for guide dog training.

Other common tools are Closed Circuit TV (CCTV) , personal computer with at least a 17" monitor and customized software for the visually impaired. Kitchen devices include a battery operated pouring "buzzer" to prevent spills. Tactile paint can be applied to knobs of appliances to mark buttons and oven dials.

How has your visual loss affected your life? What was the hardest part of adjusting to your loss? The easiest?

For this question, half agreed there was nothing easy about losing their sight, The other half, those that have lived with their loss five years or longer, wrote, because of their blindness, they have learned to take nothing for granted and to appreciate all they do have instead of all they have lost.

The most difficult aspect of their adjustment was to give up driving, a major source of independence. Loss of independence was the most relevant concern of all the participants, followed by the fear that they will lose all their sight.

Have you found any positive things about living with low vision?
The positive: learning how to ask for help, how to travel without a car, caring and living with a guide dog, and living a more sedate lifestyle. One participant mentioned that because of her vision loss, she has grown more independent and self-reliant (4). Problems Associated With Legal Blindnes I am a wife, mother, writer, and student. I am also legally blind. Can I see? Yes, but not very well. I don't drive. I use a cane at night and in unfamiliar places. How do I cope? What do I see (or don't see)? How does it make me feel?

The clinical definition of low vision is called "subnormal vision", or, the relationship of a person's acuity to their visual function and how it has affected their life. As an example of this statement, I am in the severe stages of visual loss. My visual function is restricted so that what little sight I do have is very limited and often unreliable. I am color blind, night blind and sun-sensitive. My visual field, (side-to-side, up-and-down) is less than 20 degrees using both eyes. All these are symptoms related to r.p.

To experience restricted visual field, also known as peripheral loss, take an empty paper towel tube cup your hands around your eyes and look through it. That is about how much I am able to see around me. That is just one aspect of my visual problems.

Most people admit they really don't know someone has severe visual problems unless the person in question is either using a white cane or a guide dog. For this reason, adjusting to a gradual decrease in vision can be more emotionally traumatic than losing sight all at once. Those that do not totally rely on a cane or a guide dog but still cannot see well enough to drive, read books, street signs or see a computer screen without difficulty may in fact have the greater challenge. Those who are born blind or loses their sight early in life learn permanent adjustment regarding their handicap. Those who hadtheir sight taken from them gradually face unique transitional changes that affect them emotionally and physically. They are neither blind or sighted, what affected people call "visual limbo." We are always adjusting and that is the most emotionally challenging phases of having r.p. and being legally blind.

Most people with low vision suffer from depression. Many also suffer from stress and anxiety disorders due to a loss of independence or confidence. Some that are diagnosed later in life, between the ages of 30-50, divorce because of the stress or issues attached to the diagnosis. The last five years for me have been the most upsetting because constantly walk around with the fear that one day I may wake up and find myself with less sight than I went to bed with. This is the number two concern of the r.p. survey participants. The number one concern is giving up driving. This is so traumatic, some say they've never overcome the loss of independence (4 ).

Every day obstacles include finding another means of transportation. This is the most common cause of emotional stress of those affected. Once their last means of independence is gone, the life-long challenge of finding transportation begins.

Some other physical challenges, referred to in professional circles as mobility issues, include training to use a number of visual aids, like the white cane, the primary mobility tool for the blind. For instance, for my mobility training I learned how use my cane to safely traverse steps, intersections, and public places. I was also given other adaptive visual aids for reading, writing, cooking, cleaning, sewing and matching my clothes.

For someone like me, even writing this paper has its own challenges. For instance, I need the proper computer equipment and a way to see the texts for the paper. My computer has been customized for this purpose. A large monitor and software assist me. I also have a text enlarger to help me read books, notes, and charts.

All in all, this handicap has affected me in both positive and negative ways, often leaving me with a less than kind outlook on life. But like many others with the same physical limitation, I pull out of this dismal attitude and fight back, dispelling most of the obstacles that come with losing my sight.

Bibliography
1. Ludman, Mark, Encyclopedia of Genetic Disorders , Facts on File, Inc. 1991.
2. Scarangella, Cheryll, The History of Retinitis Pigmentosa, 1991.
3. Encarta Encyclopedia, "Blindness" Microsoft Corp. 1993-1998.
4. Chiappetta, Ann Survey on Legal Blindness, 1999 RP List, St. John's University List Server, maelstrom@stjohns.edu.com