by Lylas G. Mogk, M.D. & Marja Mogk
Presented by Dr. Mogk at the
Pfizer Ophthalmology Therapeutic Area Conference
March 25, 2004
I. Introduction
Every year Age-Related Macular Degeneration (ARMD) causes more than 200,000 Americans to lose their central vision, and many more are already living with vision loss. In fact, macular degeneration alone has turned the demographics of vision loss upside down in the last two decades.
For most of the twentieth century, the majority of Americans with visual impairments were children or working aged adults. Today, at the dawn of the twenty-first century, there are anywhere from two to five times as many people over 65 with visual impairments as all those under 65. In my home state of Michigan, for example, there are approximately 3,000 blind and visually impaired children, 16,000 blind and visually impaired young adults, and roughly 100,000 seniors with vision loss just from AMD. Short of a miracle, the number of seniors with vision loss is expected to escalate significantly as the baby boomers age. Given these demographics, I will focus here on the impact of vision loss among seniors, especially those with macular degeneration.
II. The Experience of Vision Loss: Visual Patterns and Function
Those of us with full sight generally think of vision simply in terms of acuity and describe it in terms of the familiar 20/20 scale. When we get old enough to need reading glasses, we keep that same understanding of vision, because we are focused on the clarity of the print we read, which we experience as largely a matter of acuity. But vision is not a one-dimensional experience. It is made up of many elements that come together to give us the experience that we call “full sight.” We rely on our contrast sensitivity to distinguish an object against a similar background. We rely on our eyes to process glare from sunlight or indoor lighting and to provide us with accurate depth perception. These elements of vision are often affected very early in the disease process of macular degeneration, when visual acuity may still be pretty good, and themselves cause enormous changes in the visual experience of people with AMD, and enormous changes in their lives and the lives of their families.
Contrast Sensitivity and Glare
Loss of contrast sensitivity makes even familiar human faces interchangeable with each other and obscures facial expressions, because faces are monochromatic, and it can make even large objects disappear against a similar background – a mahogany table on a dark carpet or even an elephant standing in front of a huge, grey rock may be imperceptible against its background. So when someone with AMD then can see very small high contrast objects — a dark leaf on a cement sidewalk or a piece of black yarn on a beige carpet — it gives rise to a sense that one’s vision is capricious and arbitrary. Loss of contrast sensitivity causes a need for a lot of light, but also brings tremendous sensitivity to glare, so those who need light to see may instead keep their drapes closed to block out the light, another seeming inconsistency.
Depth Perception
New loss of vision in one eye presents difficulty with depth perception, such that in spite of seeing an object you want to reach for, you may miscalculate its location by a fraction, and thus miss it altogether or knock it over.
Central and Peripheral Visual Fields
Varying patterns of vision loss also play a major role. With loss of central vision, people with macular degeneration may not see the biggest letter on the eye chart or recognize a face, but their full peripheral vision allows them to walk around quite easily, so they often appear to others to be unaffected by their vision loss.
Conversely, those with vision loss from glaucoma may be able to read a magazine with their central vision, but have tremendous difficulty with mobility, because of loss of peripheral vision. Sighted observers, including friends and families, often misinterpret these apparent inconsistencies and conclude that the senior with vision loss is exaggerating, just “seeing what she wants to see,” or worse yet, is cognitively impaired.
Patterns of Central Vision Loss
About 20% of people with dry AMD have a central blind spot or “scotoma” in the shape of a ring or a doughnut with a small island of good visual acuity in the center. This island of vision may be only large enough to see one letter at a time on the eye chart. People with a ring scotoma may perform well reading the chart one letter at a time, but have difficulty doing almost anything else, from reading to pouring to cutting to all kinds of daily activities. If this pattern is not detected, it can be a great source of confusion to the person who experiences it and to their families and even their physicians.
Charles Bonnet Syndrome
This confusion and misinterpretation can be amplified by another experience that is shared by about 20% of adults with vision loss: the phantom images of Charles Bonnet Syndrome. Charles Bonnet was an 18th century Swiss philosopher who described his father-in-law as an intelligent man with normal cognition but poor vision who saw repetitive images of people and animals superimposed on the real world, images that his father-in law knew were not there. Bonnet’s descriptions were later reviewed in the 1930’s and the phenomenon was named after him. These images are believed to be analogous to phantom sensations: pain or itching in an amputated finger, for example. Like phantom pain, and unlike hallucinations, the viewer is fully aware that the images are not really there, that it is their eyes playing tricks on them.
The people or animals that appear with Charles Bonnet Syndrome do not make contact with the viewer, although they may move; the experience can be somewhat like watching a silent movie. One woman reported seeing life-sized Elizabethan figures who sat around her dining room table as if they had come for dinner. Another reported seeing little girls in white dresses with pink sashes and pink bows in their hair laugh and play in the front Seniors will rarely report their experiences with Charles Bonnet Syndrome, even to their closest family members or friends, for fear of being suspected of Alzheimer’s or dementia or of being further set apart from their peers.
We asked some of our patients with Charles Bonnet Syndrome to draw what they see. These pictures look like children’s drawings because the patients are not artists and, of course, do not see well, but they indicate how vivid, prominent, and lifelike these images can appear. [Note: these images may be seen in Dr. and Marja Mogk’s book, Macular De generation: The Complete Guide to Saving and Maximizing Your Sight, which can be found in the MD Support Book Store.
III. The Experience of Vision Loss: Living in a Sighted Culture
To save someone’s sight is not only to spare them from having adjust to their changed visual experience, but to save their life as they know it because we live in a sighted culture. We convey vast amounts of information visually or in print. We have built our buildings and crosswalks, designed our appliances, and furnished our offices and homes with the assumption that ev eryone has full vision – regardless of small concessions like the Braille you may find in an elevator – and our love affair with automobiles has produced urban geographies in most parts of the country that require everyone to drive. Those of us who are sighted routinely assume full sight in others and practice a myriad of social behaviors that are sight-based, such as giving directions by pointing, responding to comments with facial expressions, or seeing certain body postures or pedestrian movement patterns as “normal” and others as not. If we had tried to build a society and a culture that would condition us to be totally dependent on our vision in every respect – physically, functionally, socially, and emotionally – we really could not have done a better job.
As a result, we are strikingly ill-adapted to vision loss. The loss of vision to almost any degree in a fully sighted adult compromises function more globally than any other single physical impairment. So it is not a surprise that sighted Americans acknowledge that a person who is blind can achieve many things that sighted people routinely do not, like climbing Mt. Everest, yet at the same time sighted people fear vision loss more than anything else except loss of mental capacity.
Social and Emotional Impact
To lose vision as an adult is therefore to experience one of your worst fears. But the shock of vision loss and the adjustment it requires is not just about a single individual’s adaptation to a physical impairment. It often affects one’s relationships – prompting changes in gender roles, duties, professions, living arrangements, travel, and communication with spouses or children. More subtly, losing vision means crossing a line such that your own experience of the world is now fundamentally different from your sighted neighbors, and yet you look no different, so this line is often unseen, unrecognized, or misinterpreted by others.
Losing vision also means risking and perhaps confronting the prejudices, assumptions, and expectations of sighted people, and we have many of them. Seniors who lose vision were, until that point, sighted people themselves, and often carry many of these prejudices, assumptions, and expectations with them as they experience visual impairment. As a result, they may avoid organizations associated with the blind, believe in the stigmatized status of old age or blindness, or seek to minimize their acknowledgement of vision loss, even if it means staying at home and avoiding social engagements. “Passing” as fully sighted is a common phenomena among seniors and it inevitably adds to the isolation and loneliness of vision loss.
But isolation or aloneness is often a hallmark of losing vision anyway. Unlike acute medical crises, like accidents, operations, or serious illnesses, vision loss from macular degeneration comes quietly, gradually, invisibly, without flowers or phone calls.There is little or no recognition from others that a major adjustment is underway and assistance is needed. Or, conversely, adult children, unfamiliar with vision loss and frightened by it, become overprotective and make decisions for their parents that are not always optimal – and that often reflect a sighted perspective on living. For all these reasons, vision loss is lonely and isolating. “Losing my vision,” said one patient, is as if someone I love died and nobody came to the funeral.”
When one spouse loses vision, especially among the age cohorts of seniors today, it may create upheaval in the household as traditional gender roles are no longer maintainable. When the husband loses vision, the wife must drive. When the wife loses vision, the husband must participate in managing the household. Both spouses are suddenly required to make many unforeseen adjustments in their communication styles and in their expectations for skill levels in each other.
Depression
This potent mix of social pressures, individual expectations, fears, functional difficulties, and relationship adjustments is a set-up for clinical depression. Indeed, vision loss in adults is associated with more depression than just about any other condition, including heart disease, lung disease, and even cancer.
Seniors today are the tough generation. They survived the Great Depression and two world wars. They grew up on a philosophy of independence and bootstrapping your way up in the world. And just as their vision loss is invisible, so is their depression. They don’t even believe in depression, so they are unlikely to report it. Instead they attempt to cope without expressing their feelings or seeking help. In a study completed at our center we found that of the 102 seniors who qualified as depressed on the Geriatric Depression Scale, fewer than 5% had been previously been identified as depressed by either their physicians or family members.
As research is now proving to us, depression with adult onset vision loss is not correlated to age, sex, marital status, living situation, or degree of vision loss itself. It is correlated almost exclusively with functional capacity. If you can still do the things you want to do, you are far less likely to become depressed. This makes intuitive sense if you keep in mind that blind people across the country lead full lives without depression. But what these results also tell us is that a little been of vision loss can impact function tremendously, so visual rehabilitation early on is crucial.
IV. Rehabilitation & Disability: Saving Lives in a Sighted Culture
With comprehensive visual rehabilitation, virtually everyone with macular degeneration can read at some level and maintain their participation in many of their home and community activities. Visual rehabilitation does not save eyes, as we all count on you to do here at Pfizer, but it saves lives. It makes possible the transition from being a sighted person in a sighted culture to living fully as a visually impaired person in that same culture.
Outcome studies are new in the field and the methodologies and tools for them are just being developed, so it is difficult to quantify the importance of visual rehabilitation. But studies so far have shown definite improvement in easily measurable parameters like reading speed and in patients’ assessment of their quality of life. We look forward to developing a robust literature that confirms these findings and more.
The anecdotal evidence, however, for the effectiveness of comprehensive visual rehabilitation is overwhelming. Our patients in Michigan have described our program as “a light in the darkness;” and they have described our occupational therapists as needing larger doorways so their angel wings will fit through; and my personal favorite, as “better than Prozac.”
As all of us continue our dedication to saving eyes and saving lives, both here at Pfizer, in our medical practice s, and in the field of visual rehabilitation, blind services, and advocacy, I am excited about the tremendous opportunities we have to make a difference in so many more lives if we continue to listen to one another across our disciplinary divides.
Those of us who are scientists, researchers, or physicians here today must become informed about visual rehabilitation so that we can take this awareness back to our family, friends, and communities – or, in the case of doctors, so that we can communicate with patients who experience vision loss effectively and refer them to visual rehabilitation programs. I am happy to say that the American Academy of Ophthalmology is stepping up to the plate on this one, and I am confident that, in the next several years, you will see a vast improvement in rates of ophthalmologists’ referrals for visual rehabilitation.
Those who have been blind for years or who have traditionally worked with blind or visually impaired children or younger adults can make a tremendous difference by increasing your outreach to seniors, by understanding their experience of loss, and by offering them membership in the larger community of those who are blind and visually impaired, with a new non-sightist way to see blindness and vision loss.
Those who are younger must become aware of our subtle assumptions about the experience of being older. One of the great surprises of aging is that you feel like exactly the same person you did at 12 or 25.We must come to consider loss of vision in later life as seriously as we would consider it if it occurred at a younger age.
Finally, those of us who are sighted have a responsibility to become aware of the ways in which we harbor assumptions or prejudices about blindness and visual impairment. We must work to change those attitudes in ourselves and others. I know that accepting blindness as a valid, vibrant way to live in the world seems counterintuitive when you spend your professional life fighting vision loss, but it’s not. We live in a sighted culture. So long as that is true, then having sight makes functional living easier and enjoying the pleasures that sight has to offer enriches life.
But while there are many ways to be fully human and to experience an enriching life – as sighted people, we will never experience the enrichments that blindness may itself have to offer. So long as we fear blindness and reject it, so long as we believe in some way that blindness is lesser than as a way of being, not simply a more difficult way, then we are part of the problem. We are part of what makes vision loss itself so difficult.
We can work to save sight and work to help those who lose vision to continue to thrive by expanding their skills and making the society more accessible, and at the same time we can celebrate the richness of being human in every form. If we do this, we will truly be part of the solution, on all fronts.