American Action Fund for Blind Children and Adults
Future Reflections Summer 2024 NEW DIRECTIONS
To See and Not to See: Helping Students with Cortical Visual Impairment Access the Resources They Need
by Stephanie Duesing
From the Editor: The parent of an adult son with cortical visual impairment, Stephanie Duesing is a powerful advocate for the CVI community. Eyeless Mind, the story of her son's journey with CVI, is available on Amazon and on Bookshare.org.
My son Sebastian has cerebral visual impairment, or CVI, as does our teenaged friend Mae [see the article "Living and Growing with Cortical Visual Impairment" elsewhere in this issue). The NFB has helped many people with CVI over the years. I am writing both to thank you in the blind community and to ask for your help.
A Rising Storm
CVI is the leading cause of visual impairment in the developed world, but according to the website www.cviscotland.org, most people have never heard of this condition. As you read this article, you may be learning about CVI for the first time, though CVI is more prevalent than ocular forms of blindness.
According to the Perkins School for the Blind, research shows that one in thirty students in the typical regular education classroom is likely to have symptoms of CVI. According to this finding, CVI is more prevalent than autism. Yet fewer than twenty percent of people with CVI have been diagnosed. More than 80 percent of the people who live with this frequently life-altering disability never receive any blindness-related services or any training in the alternative skills of blindness. Research suggests that more than 150,000 people in the US alone live with undiagnosed CVI. You can read more about the prevalence data at https://www.perkins.org/our-work/cvi/by-the-numbers/.
We desperately need a new, updated definition of legal blindness. The definition should not be based simply on visual acuity. It should be based on a person's need for blindness skills such as Braille and orientation and mobility (O&M). Some 150,000 CVIers in the United States are living without the blindness services they need, and often they are severely traumatized as a result.
Sebastian fully supports my advocacy efforts, and I share this article with his full permission. I am a former music teacher, and I made a major medical discovery in the field of visual neuroplasticity. I am an author, speaker, and international advocate for all people who have CVI.
To See or Not to See
Sebastian has a unique form of visual processing. To date he is the only person in the world known to use verbal mediation to process his vision. He sees with words, much as a bat sees with sound. According to Dr. Lotfi Merabet, the director of the Laboratory for Visual Neuroplasticity at Schepens Eye Research Institute, associate scientist at Massachusetts Eye and Ear, and associate professor of ophthalmology at Harvard Medical School, Sebastian is the most studied individual with CVI in the world.
Sebastian is the only person in the world known to be able to choose to see or not see with his eyes wide open. When Sebastian is not using his verbal mediation to see, he has no conscious perception of sight. He spends most of his waking hours with his very limited vision "turned off." In other words, he is fully blind most of the time.
In my book, Eyeless Mind, I explain how we discovered Sebastian's CVI and how we figured out that he was using verbal mediation to see. We also have shared our story in radio interviews and through podcasts, which you can explore at https://stephanieduesing.com/in-the-media.
What Is CVI Anyway?
Many people reading this article may wonder what CVI is and how it differs from ocular forms of blindness. CVI is a brain-based form of visual impairment/blindness that is caused by damage to the brain's visual pathways. In ocular forms of blindness, damage to the eye and/or the optic nerve impair or prevent light from being transformed into an electrical signal and passed to the brain to form images. People who have CVI (and who don't have co-occurring ocular visual impairments) can see light. However, they struggle to, or simply can't, process those signals into understandable visual images.
CVI may be accompanied by ocular vision impairments. However, it is common for people who have CVI to have normal acuity, as my son does. My blind son passed every vision test, every year, because our optometric exams are decades out of date. There is so much more to vision than acuity, contrast, and color perception!
Research has shown that all conscious perception of sight happens in the brain. Scientists have found that it takes about a tenth of a second after light hits the retina for the signal to reach the back of the brain, when humans begin to experience conscious vision. In other words, the eyes see nothing on their own. The phrase, "We see with our brains, not with our eyes" isn't merely a slogan. It's an actual fact. Our eyes really are just light collectors and signal senders. Seeing is such a complex neurological process that more than forty percent of our brain is involved in visual processing.
CVI is caused by damage to or malformations of the brain. Different areas of the visual processing centers of the brain control different aspects of vision. For example, the right fusiform gyrus is above and behind your right ear. The right fusiform gyrus is associated with facial recognition. People with damage to that area of the brain are likely to have prosopagnosia, or face blindness, the inability to recognize faces. Other areas of the brain are involved in recognizing places, objects, biological forms, words, letters, numbers, simple shapes, movement, color, the ability to map complex scenes, and much more. Each of these areas is connected to other areas of the visual processing parts of the brain. Thus, damage to one area inevitably results in problems with other aspects of vision, because communication breakdowns occur when brain damage is present. Each part of the network is connected to other parts of the visual system.
When a person experiences brain damage and develops CVI, where the damage occurs in the brain is a fairly random process. For that reason, every person who has CVI has a unique experience of their functional vision. Some parts of vision in undamaged visual processing centers of the brain may work quite well, while other parts of vision may be difficult or impossible to use. For example, although my son has severe CVI and is almost completely blind, he can see words, letters, numbers, and simple shapes as a typically sighted person can. The parts of his brain that see and recognize words, letters, numbers, and simple shapes are undamaged.
Sebastian has always appeared to be typically sighted, and he has always excelled intellectually, physically, and artistically. Yet Sebastian has no ability to recognize faces, places, objects, or biological forms by sight. Visually he can't recognize his own hands, face, or body, or anybody else's. However, Sebastian can read and do math visually. His ability to see symbols such as letters and numbers and his ability to form visual memories of them allowed Sebastian to excel academically.
A Tiny Window
Sebastian has another very common symptom of CVI called simultanagnosia. Simultanagnosia is the inability to see more than one or two things, or parts of things, at the same time. For Sebastian, having simultanagnosia means he has a tiny patch of visual acuity in the center of his visual field that is a couple of millimeters wide. When he's reading, he usually can see two or three letters at a time if the letters are in ten-point font, which is quite small. For Sebastian, and for many other CVIers I know, magnifiers commonly used for people with ocular visual impairments don't help. In fact, they may even make seeing more difficult. Sebastian needs to have things shrunk down to a tiny size so he can fit what he's looking at into his miniscule patch of acuity.
As a result of all these problems with his vision, Sebastian can see almost nothing as a typically sighted person can. He sees like someone in the middle of the ocean in a dense, colorful fog. Everywhere he looks, he sees light, movement, and vague, colorful, unrecognizably blurry shapes. Sebastian's vision is mostly a useless blur of visual "noise," like static on a radio.
Static noise is irritating. Many CVIers describe the constant barrage of unusable visual "static noise" they experience as relentless, painful, exhausting, and overwhelming. They can't turn it off, except by closing their eyes, and they very often wish that they could turn it off permanently. The constant flow of useless visual nonsense coming in through their eyes overwhelms their senses, and it is often exhausting.
In the midst of all this visual "noise," the only things Sebastian sees like a typically sighted person are occasional words or numbers floating by. The words might say, "Walmart," or "Dunkin’ Donuts" as we drive down a road, but Sebastian can't see the sign that the words are written on. He can't see the store, or the parking lot, or the cars around us, or the pedestrians crossing the street, or the grass and trees, or the sky. He simply sees the words floating in space. He is severely visually impaired when his vision is at its best, and blind most of his waking hours because his conscious vision is "turned off."
Visual Fatigue
Finally, one CVI symptom that I have left for last is called visual fatigue, or visual tiring. I left visual fatigue for last because I think it is so important. Visual fatigue is a very common, very well-documented symptom of CVI. It can be an extremely dangerous symptom because it causes CVIers to go suddenly, uncontrollably, blind at unexpected moments. They lose all conscious perception of sight at random times—from being overheated, sick, over-tired, stressed, or overwhelmed. This random loss of conscious vision can and does occur at any time or in any place. Very often it occurs in the most stressful situations—out in a crowd, on a busy street, or at a congested shopping center.
Visual fatigue is caused by fluctuations in blood pressure. I have fainted twice in my life. Both times when that happened, my vision suddenly blacked out, and then I lost consciousness, in that order. People who have CVI have less neurological visual processing power than neurotypical people do, so their vision is more sensitive to fluctuations in blood pressure. When their blood pressure drops, they lose conscious perception of sight far more quickly and frequently than others do. It can take hours or days for them to recover.
Visual fatigue is an extremely debilitating and common symptom of CVI. For people who have no blindness skills, it can be dangerous, even life-threatening, to randomly, uncontrollably go blind while out in public. According to every CVIer I know, visual fatigue is just miserable, even at home in bed.
The Cloak of Invisibility
One of the most important things I want everyone in the NFB to know about CVI is that it can be an entirely invisible disability. Academically, socially, or physically, there is no way to tell that Sebastian is almost completely blind. If any of your typically sighted friends or family members met Sebastian, they would not know that he has any disabilities at all. No doctor, teacher, friend, family member, acquaintance, or stranger on the street ever suggested to us that Sebastian might have a disability of any kind, let alone that he was almost completely blind.
We discovered Sebastian's severe CVI entirely by accident when he was fifteen. I felt terrible that I had missed something so critically important for so long. My child had struggled for years, and I had been unable to help him. It was the most horrible day of my life!
A Mission to Raise Awareness
I spend a lot of time raising awareness about CVI. I am a frequent podcast guest. People listening to our story often think that Sebastian is somehow remarkable because no one knew he was blind until he was fifteen.
Unfortunately, what happened to us happens all the time. Sebastian represents the typical presentation of CVI. With one in thirty students in a regular education classroom having symptoms of CVI, we know that the vast majority of CVIers appear to be typically sighted, just as Sebastian does. Furthermore, there is absolutely no way to tell how severe someone's neurological visual impairment is from outward appearances alone.
We found it extremely painful and difficult to bring our discovery of Sebastian's use of verbal processing to the attention of the low-vision profession. I fought hard to get scientific evidence of Sebastian's verbal vision to the professional community. I knew our discovery had huge implications for understanding the "why" behind many learning disabilities. If it was possible for my blind son to read and do math visually, to be blind but not to look blind, I knew that the opposite must also be possible. I knew there must also be people who, like Sebastian, look typically sighted, but who are totally unable to see or visually recognize words, letters, numbers, and simple shapes.
What if some people who are labeled as having learning disabilities aren't learning disabled at all, I wondered. What if those people have CVI and are blind or visually impaired? What if those people have letter and/or number agnosias? Suppose they can't form a visual memory of symbols when they try to read or do math? What if those people could learn just fine if they had access to tactile learning tools such as Braille? I found that it is common for people who have CVI to be misdiagnosed as having autism, intellectual disabilities, or emotional and behavioral disorders.
Not long after we went public with our story, I met Katie Lane-Karnas and her brilliant child Mae. Mae has CVI and is blind. Like Sebastian, Mae passed for typically sighted. Not one person suspected that Mae is blind. Like us, Mae's family had little support from their school district.
Sebastian has graduated from college and has started his career, using only his vision to read, but he has often expressed regret at not having had the opportunity to learn Braille. Though Sebastian can read visually and has outstanding reading comprehension, the process of using his vision exacerbates his visual fatigue and contributes significantly to his visual exhaustion. Access to tactile learning in childhood would significantly have improved Sebastian's quality of life.
Through my work as a CVI advocate, I know many people with CVI who went undiagnosed and/or misdiagnosed for years, as my son and Mae did. All of them suffered trauma from being blind in a world that refused to recognize their blindness or do anything to help them. My son, along with all of my friends who have CVI, experienced trauma because nobody knew they were blind until they were teens or young adults. They did not receive the training in blindness skills that they needed as young children.
It is my position that all blind and visually impaired people have a right to access to tactile educational and habilitative methods and strategies, whether their visual impairment is ocular or neurological. Braille, access technologies, occupational therapy, and O&M with a white cane should be available to all blind and visually impaired people who need it, regardless of the cause of their vision problems.
Visual fatigue is a debilitating symptom of CVI that is easily reduced or relieved by using tactile methods for educational access. Tactile learning needs to be considered when making decisions regarding which educational methods are most beneficial to people who have CVI, even for those who, like Sebastian, can read print visually. Many CVIers find that they learn best using both visual and tactile methods. They can switch back and forth as they need to during the day.
I am deeply grateful to the members of the National Federation of the Blind for your support of families of blind children, including those with CVI. Thank you from the bottom of my heart! We decided as a family to come forward so that what happened to my son would never happen to another child.
I never dreamed that I would spend the second half of my life begging the world to give blind people access to blindness services, no matter the cause of their blindness. Sebastian and I were labeled crazy by the medical establishment for seeking the most basic help. Imagine the outcry if access to medical diagnosis, treatment, and educational and habilitative services were denied to people who have cerebral palsy, migraine, or epilepsy, simply because their conditions are neurological. CVI is not different from other neurological conditions, and the people who live with it have a human right to the blindness services they require to participate fully in life. Please help me to end the madness! We have more than 150,000 CVIers to find, and more are being born every day.
For Further Information
Here are a couple of blogs from dear friends of mine whose CVI also went undiagnosed for years. The first blog is by my very dear friend Dagbjört Andrésdóttir, who didn't know she was blind until she was twenty-six years old. She is a bel canto-trained lyric soprano from Iceland who, like my son, can see and read words despite being legally blind. However, she can't read music because she can't see the staff. She sings the most challenging vocal repertoire entirely by ear.
https://dagbjortandresd.wixsite.com/livingwithcvi
The second blog is from another very dear friend of mine, who prefers to remain anonymous. They are a highly respected low-vision professional. Like Dagbjört, their CVI wasn't recognized until they were an adult. This blog is a must-read for everyone in the low-vision community who cares about people who have CVI.
https://thecviperspective.wordpress.com/home/
This video from the Perkins School for the Blind features Jack Brossert. Jack's CVI was not diagnosed until he was a teen. In this video, he discusses how blindness skills training and his guide dog have helped him develop the skills he needed to go to college and study science.
https://www.perkins.org/resource/my-experience-navigating-the-transition-from-high-school-to-college-as-a-student-with-cvi/