Seeing the Power in Blindness

As a physician, when I care for patients I often wonder what thoughts and feelings I’m not hearing from them. What inner dialogue is running, what worries do they have, not only about their symptoms but also about the present and the future, about how their illness will unfold and change their lives?

Andrew Leland, a writer with declining vision, offers an eloquent inner dialogue of stark honesty in The Country of the Blind. Leland has a condition called retinitis pigmentosa (RP), which causes degeneration of the retina. The disorder primarily affects its photoreceptors: the rods that mediate vision at low light and the cones that are active at higher light levels, mediating color vision and conferring spatial perception. An underlayer of pigmented cells in the retina supports the rods and cones, and as they die, the pigmented cells become exposed. A doctor looking with an ophthalmoscope into the eye of a patient with RP sees deposits of black pigment—often in what is termed a “bone-spicule pattern” (“spicule” from Latin for an ear of grain).

The genetics of RP are complex. It may be inherited in several ways—as a dominant, recessive, or X-linked trait—or it may occur sporadically. More than three thousand genetic mutations in approximately seventy different genes have been identified as causing RP. The worldwide prevalence of the condition is estimated at one in four to five thousand. About 80,000 Americans, out of a population of 335 million, have RP. Some develop visual loss in childhood, others only in adulthood. As the rods and cones degenerate, patients suffer from night blindness (nyctalopia), reduced peripheral vision, and diminished visual acuity. Other symptoms include photopsias (sensations of sparkling lights) and headaches. Central vision declines more slowly than peripheral vision. Most patients meet the criteria for legal blindness by their fifties.

Leland opens his narrative by noting that there is no cure for RP as the reader accompanies him into his ophthalmologist’s office. He was diagnosed over twenty years ago and visits his eye specialist every other year, undergoing a full day of tests that document the progression of his malady. On a recent visit, the specialist

showed me an illustration of how much vision I had left. It reminded me of ice cubes melting in hot water: two small, wobbly ovals in the center, and two skinny shapes floating along the sides. The wobbly ovals represented the central vision I still had, and the strips were my peripheral vision. I had about 6 percent of what a fully sighted person sees. My doctor frowned graciously as she gestured at the skinny french fry shapes. “When those go,” she said in her medical deadpan, neither cheerful nor grim, “your mobility will become more limited. Those two strips of residual peripheral vision are what you’re using to get around.”

While physicians invoke the clinical term “tunnel vision,” Leland explains:

What I see doesn’t look like a tunnel; the walls of the enclosure aren’t visible…. I bump into furniture in my house that hasn’t moved in years. I’ll put a cup down for a moment and it disappears. I’ll painstakingly rake the wobbly ovals and slender french fries of my residual vision across the table’s surface again and again, and when I finally find the cup, it’s standing blamelessly in what even a few weeks ago I would have described as “plain sight.” It’s still in plain sight—it’s just that my sight is growing less and less plain.

Since vision loss due to RP is gradual, Leland lives in a liminal space. While his degenerating retina is not painful, the uncertainty is:

The most painful part so far has been the not-knowing. I live much of my life these days in a speculative mode…. As I cook dinner, or walk my son, Oscar, home from school, or find my way from the airport to a train station in an unfamiliar city, I ask myself: What will this be like when I can’t see? I perceive everything with this paradoxical double vision: through sighted eyes, and through blind ones. While most futures are difficult to see, shrouded as they are beneath the fog of contingency, mine is doubly difficult to visualize. The crystal ball remains clouded over.

His book is in large part a journey of discovery to reduce this gnawing uncertainty:

The blinder I get, the more curiosity I feel about the world of blindness and what possibilities might exist there. So I went out in search of that world, to find a more accurate image of what might be waiting for me.

An often unspoken fear among patients is of becoming infantilized, unable to perform chores or attend to bodily functions. Leland finds questions about the daily functioning of the blind insulting:

How do you eat? Who dresses you? Can you sign your own checks? Questions like these, which suggest that the world of blindness is an infant’s world, where a blind person can’t even put on a shirt or bring a fork to his mouth without sighted assistance, are infuriating.

Why such a visceral reaction?

They exacerbate the painful difference that the experience of disability carries—no one else is being asked how she accomplishes the most basic tasks of daily living as she waits in line for her breakfast burrito.

But Leland, who “still feels like a tourist in the country of the blind,” nonetheless finds such insulting questions urgent. He ponders how he will travel independently, or write, or work: How, as a blind father, will he experience his son Oscar’s maturing “from little kid to teenager”?

Leland is also infuriated by comic stereotypes of the blind like Mr. Magoo, a “bumbling, senile cartoon character unaware of his own imminent physical peril.” In counterpoint, Leland appreciates absurdist jokes, like “A blind man goes into a department store and starts swinging his guide dog by the tail over his head. ‘Can I help you?’ the clerk asks. ‘No, thanks,’ the man replies. ‘I’m just looking around.’”

His inner dialogue swings between extremes, comparing blindness to

a bickering, annoying family and a loving, supportive one; an interesting hobby, whose fellow hobbyists can inspire and delight as well as irritate and depress me; an identity that I embrace and revile, that defines me and has nothing to do with who I really am.

This tension is examined dispassionately: he muses that there is “beauty and power” in blindness in addition to

its almost definitional sense of loss and exclusion. How can a thing that causes so much alienation also be a source of growth and joy? How can something that estranges us from so much of the world also bring us closer to it?

At times Leland invokes blindness to ask fundamental questions about cognition:

How does anyone know the world? Does vision deserve the privileged place it holds at the top of the hierarchy of the senses? How much of perception happens in the eyes, and how much takes place in the mind, regardless of which senses supply its stimulus?

While we often imagine blindness to be a totality—“Modern dictionaries still subscribe to this sense: blindness is the antonym of vision, and connotes a destitution of sight”—Leland explains that blindness is not typically “a black veil draped over the world.” While some 15 percent of blind people fully lack light perception, “most see something…a blurry view of their periphery, with nothing in the middle, or the inverse—the world seen through a buttonhole.” Others perceive a “dim haze” or “a shower of excruciatingly bright needles.” Even lacking any light perception, the brain “can still produce washes of brilliant color and shape.”

The onset of visual loss began during Leland’s youth in New Mexico. He had difficulty climbing darkened hillsides with friends at night, at times walking face-first into trees. At the cinema, he waited to leave his seat until the lights were turned on for fear of crashing into people. He mentioned this to his mother, who dismissed it: “Everyone has ‘night blindness,’ she said—it’s dark at night!” But he diagnosed himself by looking up “night blindness” using “pre-Google search engines.” The diagnosis ultimately was confirmed at the eye clinic at UCLA using several procedures, including an ERG,

which involves numbing your eyes and then attaching electrodes to your eyeballs to measure the amount of electricity your retinas are putting out in response to light. (It’s like testing the charge on a battery, but the battery is part of your face.)

Leland’s inner dialogue at times turns harshly confessional: “Skepticism, pity, revulsion, curiosity. I know I’ve looked at blind people this way too.” But he finds he’s changing this view, and feels “a stronger desire for solidarity, for community—to meet other people who’d had this experience of the many ways that the sighted world expresses fear, discomfort, or condescension around blindness.”

Beyond the strictly personal, Leland highlights the socioeconomics of visual impairment: “The most salient factor determining the quality of a blind person’s life may not be what culture or historical period they live in, but the economic and familial situation they’re born into.” Many blind people “live at the margins of society, shunned by their families, living lives of unremitting poverty, while blind contemporaries live lives of relative ease.” While there are historical examples of worldly success—John of Luxembourg, the fourteenth-century king of Bohemia who ruled without vision; Nicholas Saunderson, the Lucasian Chair in Mathematics at the University of Cambridge a decade after Isaac Newton—they represent a small fraction of the blind.

Leland acknowledges being economically fortunate as the grandson of Neil Simon, the writer of plays and films, who bequeathed him “a financial cushion.” This cushion is not available to the majority of blind Americans. The average US unemployment rate is around 5 percent; it is fourteen times higher for the blind at about 70 percent, Leland notes.

Encountering these statistics changed the stakes of what it meant for me to become a member of a blind community. I’d initially approached it as a sort of cultural or philosophical question: What is the phenomenology of blindness?… But as I considered the lives of most blind people, the question of what blindness is became inescapably political. Every state in the US has its own commission for the blind, staffed by vocational rehabilitation counselors trained to help blind people find employment, with government dollars earmarked for essential tools and services—canes, screen reading software, magnifiers, training. But clearly this system—overwhelmingly run by sighted administrators and teachers—was failing blind people. State support, supplemented by relief from private charitable institutions, has proven itself insufficient in the face of that astonishingly durable 70 percent unemployment figure.

The history of blindness is marked by humiliation and exploitation. The first state-run hospice for the blind was founded in Paris around 1260 and named the Quinze-Vingts, denoting its three hundred blind residents. They wore yellow fleur-de-lis badges to signify the king’s protection. While some had jobs as bell ringers or managers of a tavern within the hospice, the majority were beggars who went out into Paris accompanied by a sighted guide.

The Quinze-Vingts cast the blind person as a professional mendicant, sparking resentment. This resentment crystallized into a cruel “contest” in 1425. Four blind men were dressed in battle armor and, carrying a banner with the image of a pig, marched with an actual pig to a park. “The blind men were given batons and told that whoever killed the pig could keep it,” resulting in “a violent, inverted game of blindman’s buff.” Believing they hit the pig, they actually struck each other. Such degrading “contests” were regular entertainment for sighted Parisians. Louis IX, who founded the Quinze-Vingts, forced French Jews to wear yellow badges; it was one of his successors who required the blind residents of the Quinze-Vingts to wear them as well. Leland cites a scholar who wrote that Jews and blind people were similarly castigated as being greedy, lazy, and prone to criminality. Like with other victims of bigotry, they were “at least partly blamed for having chosen their marginalization, Jews by eschewing conversion and blind people through sinfulness or lack of faith.”

Both Jewishness and blindness, Leland writes, morphed into

biological facts that one could hardly choose to escape. The Nazis made Jews wear yellow badges too, and in the first half of the 1930s, Nazi civil servants sterilized at least three hundred thousand people, focusing in particular on children and anyone with hereditary diseases, including inherited causes of blindness.

This historical association leads Leland to ponder the nature of his identity:

I’m a Jew because of who my parents are, but also because of something more ineffable, that feels far more cultural than it does biological. I’m drawn to other blind people through a similar sense of shared experience, even as, frequently, I realize that this affinity is superficial, and misguided. At times I’m sure that I have—or want—nothing to do with those groups, even though I am, inescapably, a member of both. The self-hating Jew is a well-worn trope. How about the self-hating blind person?

In the historical annals of blindness, there is both oppression, like the pig contest, and liberation, epitomized by the development of braille. In the early nineteenth century, Charles Barbier, a former army officer under Louis XVI, began his quest for a universal language. He raised the question of how the blind could read. His novel writing system became known as écriture nocturne—“night writing,” based on a code of raised dots embossed on a page using an awl. After Barbier’s innovation came that of Louis Braille, who was blind from the age of three as a result of an accident with a pruning knife. At twelve, Leland writes, he used a

similar tool to tweak and refine Barbier’s system, adapting it to the needs of blind readers and writers. He cut the number of dots in a single cell in half…a braille cell is composed of a two-by-three grid of just six possible raised dots, numbered from the upper left (1) to the lower right (6). Which of these six dots are raised determines their meaning: dot 1 on its own is the letter a; a cell with only dots 1 and 2 raised is b; dots 1 and 4 is c; and so on. Braille’s six-dot cell could be felt in its entirety in a single pass even by a child’s small fingers.

With this invention, “the fingers had found their native language.” Interestingly, when a blind person reads braille, neuroscientists have discovered that the area of the brain mediating vision is activated in much the same way as that of a sighted reader.

Leland works hard to learn braille and is rewarded in an unexpected way: “Reading on the braille display was a palliative against my anxiety about going blind. The more facility I gained with it, the more I could imagine a rich life for myself as a blind reader.” To more closely experience his future blindness, he enters a center in Littleton, Colorado, where residents wear sleep shades for seven or eight hours a day, for an average stay of nine months. Managed by blind people, they learn not only how to read braille but to cook, clean, navigate streets, “even use power saws in on-site woodshops—all using nonvisual techniques.” Leland felt “relief and liberation” at the center: he was “no longer the outlier.”

Medicine is developing gene therapies for inherited diseases. These treatments block deleterious mutations or substitute for them. Leland describes the use of CRISPR in creating gene therapies as “a pair of molecular scissors…that finds the misspelled gene, cuts it open, and actually edits the typos in the genetic sequence directly,” like a copyeditor who corrects a grammatical error in a manuscript. He hopes for “a targeted, personalized treatment that would replace my misspelled gene with a corrected version.” The FDA approved a gene therapy in 2018 for a different RP mutation. According to Leland,

That therapy, Luxturna, wouldn’t do anything for me, but for those whose RP is caused by a mutation on the RPE65 gene, the effect is transformative. Researchers described children putting away their canes, reading print with comfort, seeing starlight for the first time.

Leland’s RP is the result of the MAK-1 mutation. Even without a targeted therapy, he finds hope in the uncertain outcome of his aberrant DNA:

The MAK-1 mutation…was only identified in 2010, and there have only been a handful of small studies on patients who carry it. One of these studies, my doctor told me, observed twenty-four people with the mutation and five of them maintained a small amount of central vision into their seventies. Their seventies!

As a writer, Leland seeks inspiration and kinship among authors who also suffered loss of sight, like Homer, Borges, and Milton. He quotes Milton’s rebuttal to a critic who invoked the poet’s blindness as a metaphor for faulty reasoning:

I would, sir, prefer my blindness to yours; yours is a cloud spread over the mind, which darkens both the light of reason and of conscience; mine keeps from my view only the coloured surfaces of things, while it leaves me at liberty to contemplate the beauty and stability of virtue and of truth.

James Joyce, whose vision was impaired, required an operation to rescue one eye from going blind. Facing this uncertainty, he nonetheless kept working on Finnegans Wake, writing to a friend, “What the eyes bring is nothing. I have a hundred worlds to create, I am losing only one of them.” Leland embraces Joyce’s words:

This comment, for all its bravado, still captures, I think, the reality of vision loss: one is indeed losing an entire world, a planet’s worth of images, all those dioramas plunged into darkness. And yet the worlds that persist after blindness—in the remaining senses, in the imagination, and in the depth of feeling that has nothing to do with visuality—far exceed what’s lost.