All My Heads and All My Tails

Beware the Ides of Gene.When her sibling was diagnosed with Huntington's disease, my wife morphed into the Roman Emperor Julius Caesar, who had met his end by failing to heed the prophecy of a soothsayer. This soothsayer had divined his murder by reading the entrails of a sacrificed animal, and when Caesar ran into the haruspex on his way to the senate, he mocked her. “You realize the Ides have come?” he said. To which she replied, “You realize they have not yet gone?”On her way to the rest of our lives together, my wife, too, mocked a soothsayer, this one a genetic counselor. In the face of what appeared to be a commonsensical proposition—find out if you have this inherited disorder—she was uncharacteristically curt and dismissive. Under no circumstances would she get tested. She'd seen what a diagnosis without treatments can do. You walk in as a person, and you walk out as an object of pity. My wife wouldn't let the question of whether she had Huntington's touch her. It was like poison ivy or stinging nettle to the soul.“I'm living the way I want to live,” she told the counselor. “You mean with no regard for the future?” I interjected, my nerves and irritation surfacing. Caesar had at least listened to the haruspex's warning.I'd been reading Roman history and wanted my wife to get tested. I'm the sort of guy who has to know something if it can be known. Back then, I viewed tomorrow nervously, even suspiciously, like a soldier on night watch, and one day the threat of threats bore down upon us. Even if you can't stop what is coming, you can prepare for its arrival, I believed. You can be ready. She, on the other hand, was chill, as people like to say, exceedingly so. If a hurricane was about to make landfall, she'd propose a walk.She lived in the now and, ever more punishingly, in the no of not getting tested.We'd always been opposites in temperament, but we'd made it work. I used my energy to get a million things done; she used her calm to coax a brutalized six-year-old boy with autism, whom we'd adopted from foster care, to life and love. Never before had I been cut off from a major decision in our marriage. Never before had I felt as if she were toying with my anxiety.A positive result was better than not knowing, I told myself. Not knowing ate the present like some sort of frantic Pac-Man. If she lived in the now, I lived in the quivering when.In my mind, this beautiful, compassionate woman, to whom I'd been married for 30 years, became a dictator in Roman fashion. Surely, it was hubris to ignore the gods—in this case, genetic technology. What horror would befall us? Americans likely know of Huntington's disease through the folksinger Woodie Guthrie. An incurable, neurodegenerative disorder affecting a person's movement, thought, and emotions, it's akin, say the experts, to having Parkinson's, ALS, and Alzheimer's at the same time. Involuntary, dance-like twitching characterizes the condition—hence, the early name for it: chorea.Because Huntington's is an autosomal dominant disorder, every child of an affected parent has a 50 percent chance of getting it. Typically, the disease manifests itself in your forties or fifties, but it depends on the number of cytosine–adenine–guanine (CAG) repetitions in one gene on chromosome 4. In general, the greater the number, the younger you are when the phantom (as I started calling it) appears.“Genetics is a science full of gods,” writes Valeria Luiselli. Because it depends on a notion of determinism and because Huntington's seems about as grimly deterministic as a disorder can be, testing can't help but resemble a terrifying augury. Matt Ridley captures something of the brutal fatalism when he exclaims, “No prophet in the Old Testament . . . ever pretended to tell people exactly when their lives would fall apart, let alone got it right.”From the moment it was understood to be a hereditary phenomenon, Huntington's became entangled with suicide—to the extent that the latter also seemed predestined. Listen to the doctor after whom the disease is named, George Huntington, describe the “refuge” of self-harm in notes for a lecture to the New York Neurological Society in 1909: Then came the hanging of D.H. in his blacksmith shop. He was a victim of incipient chorea, knew it, had been waiting for it, the “Sanctus Invictus,” and well knowing the character of the foe he must meet, the so dreaded, the long expected, the conquering, he cut short the taper and his life went out. Other victims had sought the same refuge again and again by drowning.Now, as a legal precaution, genetic counselors make you sign a waiver before the augury can begin.The vast majority of people know that the disease may be coming for them because they've seen a parent struggle with it. As in that Heinz Ketchup commercial, where Carly Simon sings the word “anticipation” in elongated fashion, the wait defines the experience. In genetics, “anticipation” refers specifically to the process by which a disorder shows up earlier and is more severe in subsequent generations. You get your ketchup sooner, you might say, but it's no picnic.In the case of my wife's family, Huntington's knocked on the door without any warning. You can be on the threshold of having the disease, they learned, and, when you pass on your genes, your kids, as if pushed, might step inside. Everyone has the aforementioned CAG trinucleotide. Six to 26 repetitions are considered normal; 27 to 35 repetitions do not lead to clinical manifestation, although they confer an increased risk of expansion due to greater instability in the copying. Most of the de novo mutations come from this “reservoir” of instability, as one team of researchers describes it. While expansion is typically modest, large jumps have been recorded. (No genome is without palace intrigue.) Thirty-six to 39 repetitions sometimes lead to disease expression, and anything over 39 always does. Was it better to be surprised by Huntington's? To be enjoying our middle years without an inkling of the danger? Not for this planner. I had put my son through college; I had renovated my house; I had socked away money for retirement. Yet I had no idea whether my wife had a devastating disease, and if she did, how long we had together before her health declined. I tried everything to make testing happen, beginning with jokes to relieve the tension: “What kind of dinosaur is best at seeing the future? A tarot-dactyl.” Not even a groan from my beloved.I pointed to our son, who has high support needs. “How will I care for him alone? How will I care for him while caring for you? We need to put measures in place.” “You'll do just fine,” she said. “I have faith in you.”I spoke of bucket lists. “Which five things do you want to do? Do you want to make love at the top of Mount Everest? We'll have to get accustomed to frostbite, but let's go! Just get tested.”I wheedled; I cajoled, as only a scared, little man could. “Do it for me,” I begged—even though we were talking about her body. Whenever I used the possessive pronoun “our,” as in the phrase “our future,” I sensed a crack in the wing of my argument. Our future meant respecting her wishes.I understood this. I really did. But when it came to fear born of uncertainty, I felt like Dionysius II, tyrant of Syracuse. (My reading sometimes made me a ruler, though one excessively paranoid.) Dionysius insisted on trading places with the courtier Damocles to show what fear does to a king. A throne is a throat, a slit waiting. He must kill those who would kill him first. He must chase his dark imaginings.Genes can be regicides, too, I believed. I wanted to move through the halls of my wife's body, stabbing the protein conspirators. The future is now! Who needs CRISPR when you have a crown? Ridiculous, I know. My wife might be spared, and even if she wasn't, neither warrior nor sword awaited, only modest symptom management.“Tails she has it,” I whispered to myself. “Heads she doesn't.” I had taken to tossing a quarter in private. George Washington's noggin seemed as big as my own when it tumbled to the ground. At one point, I scored seven Georges in a row and illogically felt better.“You have an anxiety disorder,” my wife said more than once, the last time adding pointedly, “Maybe you need to be assessed?” Her jab, its irony, hit home. Long before the threat of Huntington's emerged, I'd written about the history of assessing disabled people. It's a barbaric story of exclusion, sterilization, and murder. The Nazi T4 Program, that warm-up to the Holocaust, explicitly mentioned Huntington's as a form of “life unworthy of life,” and it deployed assessors to send people with the disease to their deaths. In the United States we sent them to institutions, often very grim ones, and told their children not to have children. We still do this.Assessment has been an integral part of producing disability as a hopeless spectacle. Barriers to education, employment, housing, and social interaction seem to be rooted in a perfectly scientific appraisal of physiological distinctiveness. Pity, like a cat, chases its own tail.In Old French the word “assess” means “to sit by.” Oh, the friends the disabled have had!My son, in a piece of writing from the sixth grade, looked back on his abandonment and nearly three years in foster care. “No one was assessing me as sweet,” he wrote. Because he was nonspeaking, had a significant vision problem, and could dissociate on a dime, doctors had labeled him “profoundly mentally retarded.” We included him in a regular school, taught him how to read and to communicate on a computer, and scrambled the very future for someone with his disability and life trajectory. When he went to Oberlin College, my wife moved to the town to coordinate his support services—he wanted to live in the dorms.How could I, of all people, have pushed testing?I'm embarrassed to admit this, but Huntington's seemed, once I was facing it, beyond the pale. Distinctly awful. Scary in its foretold annihilation. How could I spin the disorder as a form of neurodiversity? None of my strengths as the father of a child with autism—rethinking what the experts said, fighting for inclusion, insisting on respect—appeared remotely relevant. Wild with worry and feeling out of control, I reached, as in a shipwreck, for the flotsam of a test.A paradox, to be sure. Because your genetic material is sent to two different labs, you can rely on the results, you can grab hold of them in the waters of panic, even as they might take everything from you. Either you're granted a reprieve, or you play your respective part in a tragedy.It didn't occur to me that the test ought to come with a philosophy. It should have the second the genetic counselor opened her mouth. She had as much perspective on a diminished horizon as the sun at dawn, and she was just as light-besotted. “It will be good to know,” she said.Have you ever been held by a fact? Kissed by it? The protocol seemed like hounds in the forest: everything smelled of blood. The need to know whether my wife had the disease couldn't be extricated from eugenicist impulses, especially when testing produced only stigma and despair. In “The Dingdingdong Manifesto,” Alice Rivières says she “wanted something better than a yes/no answer to Huntington's.” In both cases, how we think of the disorder remains the same: The “haves” run screaming to the hospital; the “have nots” jump for joy.Genes may dictate what happens to a body, but they shouldn't dictate the fullness of a person or the kind of life they live no matter the disease or condition. “To devise . . . the predictive test for Huntington's . . . and then to allow it to become the withering process I underwent is . . . the sign of a very grave failure of medicine,” writes Rivères. “Going through with the test means witnessing the radical and immediate transformation of your inner truth, that constantly quivering kaleidoscope, into the simple truth of a medical definition.”A definition, by definition, denudes. It's like a tree in autumn, stripping for the nurse, or the doctor's white coat the season after, falling on everything. Tragedy isn't a plan. Nor is it a philosophy. It leaves no room to be determined in other ways.When my wife and I adopted our son, people assumed it was the last act of a desperate couple. “Did you try in vitro?” someone asked. “So, you couldn't get a healthy white kid?” another person inquired. (She had no idea what a racist she was.) Years later, when people heard about the threat of Huntington's, they said, “How lucky that you didn't have your own children.” A friend, whom I think of as very smart, even joked, “You must be clairvoyant.”If I could leap and choose autism, I could leap and choose Huntington's when the time came.Refusing the test was an act of rebellion, a kind of subversive shrug. It let some of the air out of the disease. “We're all going to die of something,” my wife said. In Rainer Maria Rilke's novel The Notebooks of Malte Laurids Brigge, a character exclaims, “You die as best you can. You die the death that belongs to the illness you have.” But maybe you die another death as well, a death that belongs to the way you have loved.Watching my son converse with my wife's sibling, I understood what survival means. Their laughter fell like coins of another realm. I felt considerable shame. My wife might have said of me, who had embraced testing, what Julius Caesar says, in Shakespeare's play, of his friend who was among the assassins: “You, too, Brutus?” The betrayal isn't in our genes or bodies; it's in our response to them.Like Odysseus, I had lashed myself to the double helix and listened to the experts’ two-note dirge: “Get tested.” Then I came home from knowledge. To say that my wife was right takes no effort at all; she's been right about so many things. My little pinkie, broken, could lift the weight of such an admission.Early in our marriage she discovered that I apologized readily, ardently—so much so that she had to invent a game called “Too Soon.” A spouse needs time to soak in salty indignation. Emotions evolve—they're like caterpillars, which must inch along the branch before they can fly.“Too soon, too soon,” the chrysalis shouted, and I slunk away, having knocked on the door of our bedroom. (“I'm reading” is code for growing wings.)Hurry up, love, I wrote on a card. I'll meet you at the summit of Sagarmatha. It's what the people of Nepal call Everest, and in Nepali it means “forehead of the sky.”