Menus Subscribe Search

Life in the Data

(ILLUSTRATION: DANIEL STOLLE)

(ILLUSTRATION: DANIEL STOLLE)

Fifty-Fifty: Whether to Test for Huntington’s Disease

• January 16, 2013 • 9:40 AM

(ILLUSTRATION: DANIEL STOLLE)

“I had no idea which of my parents carried the gene,” writer Mona Gable recounts. But the death of her brother led her to find out if she carried the marker.

Listen to Life in the Data, Episode 1, featuring Mona Gable:

UNTIL HE DIED in 2010, my brother was often mistaken for being drunk. There were the frequent car accidents, the flashes of anger, the cloudy thinking, and especially Jim’s wild body movements and slurred speech. People would stare after him as he lurched and wove down the sidewalk. We thought his illness was the result of a snowboarding head injury. When I’d press Jim about his health, he would deflect my questions. “I’m better,” he insisted.

Then Jim was dying. As I sat in the pale winter light of a hospital room in Colorado with my sister-in-law, she confessed that for years she’d suspected he suffered from a deadly hereditary disease. I forced the truth from his doctor: my brother had Huntington’s disease. I didn’t know exactly what Huntington’s was, but I knew it was an irrevocable death sentence.

There was no time to focus on what the word hereditary might mean for the rest of my family. I had to call Jim’s close friends. I had to rearrange my work schedule. My brother had no health insurance, so we needed to figure out how we were going to cover the bills.

In the hospital, Jim never complained. Mostly, he slept, while I walked the corridors, snapping photos of the snow-glazed Rockies and the big Western sky. Caring for Jim helped stave off my own terror.

It wasn’t until after his memorial service that I focused on what his diagnosis meant for me. One night I sat down at my computer and did a Google search. Huntington’s is all about the numbers; I learned I had a 50 percent chance of getting the disease. There is no cure. Then I saw the unthinkable: not only might I carry the lethal HD gene, but my children might too.

Huntington’s typically appears in people in their 30s and 40s, but has been seen in patients as young as 2, and as old as 90. Everyone has an HD gene, but those with the disease also carry a defective copy, the impact of which is measured with something called a CAG score. A score of 34 or lower means symptoms won’t be triggered. A score between 35 and 39 means they might. A score of 40 or higher guarantees you will get Huntington’s.

My choice was excruciating: Should I get tested? The disease can’t be treated, so what good would it do? But what about my children? At 18 and 20, they certainly had the right to know if they were confronting a fatal illness.

Nancy Wexler, a neuropsychologist at Columbia University, learned decades ago that she was at risk for Huntington’s. That inspired her to lead the ultimately successful search to identify the HD gene. But the 67-year-old scientist has never been tested herself. “I know that with me,” she said in 2009, “if I were to go to bed every night thinking I’m going to die of Huntington’s … why should I bother getting up?”

Afraid of losing their jobs and health insurance, of being abandoned by loved ones, people with Huntington’s often conceal the disease, as a 2012 study from the University of British Columbia in Vancouver confirms. That fear can lead to parents who don’t get tested unknowingly passing the disease to their children. I had no idea which of my parents had carried the gene; they’d both died years earlier, neither from Huntington’s.

Had my brother know? My sister-in-law insisted that he hadn’t. If he had, though, his fear and loneliness must have been terrible.

On Christmas Eve in 2010, two months after Jim’s death, I sat in a room at UCLA Medical Center with my husband, talking with a genetics counselor. I had decided to take the test, because of my children.

The numbers went my way. I cried with joy. But as grateful as I was, I could not stop thinking about Jim, and the cruel and capricious nature of genetics. He had drawn the wrong number. And it had sentenced him to an awful fate. ★

This story originally appeared in Pacific Standard magazine under the headline, “Fifty-Fifty.”

Mona Gable
Mona Gable is a writer and journalist whose articles on politics, medicine, travel, and parenting have appeared in the Los Angeles Times, Health, Los Angeles Magazine, Budget & Travel, and Salon, among other publications. She is a contributor to the 2008 anthology, The Maternal Is Political: Women Writers at the Intersection of Motherhood and Social Change and her essays have been published in two bestselling anthologies, Mothers Who Think: Tales of Real-Life Parenthood, and A Cup of Comfort for Mothers and Daughters.

A weekly roundup of the best of Pacific Standard and PSmag.com, delivered straight to your inbox.

Recent Posts

September 19 • 4:00 PM

In Your Own Words: What It’s Like to Get Sued Over Past Debts

Some describe their surprise when they were sued after falling behind on medical and credit card bills.



September 19 • 1:26 PM

For Charitable Products, Sex Doesn’t Sell

Sexy women may turn heads, but for pro-social and charitable products, they won’t change minds.


September 19 • 12:00 PM

Carbon Taxes Really Do Work

A new study shows that taxing carbon dioxide emissions could actually work to reduce greenhouse gases without any negative effects on employment and revenues.


September 19 • 10:00 AM

Why the Poor Remain Poor

A follow-up to “How Being Poor Makes You Poor.”


September 19 • 9:03 AM

Why Science Won’t Defeat Ebola

While science will certainly help, winning the battle against Ebola is a social challenge.


September 19 • 8:00 AM

Burrito Treason in the Lone Star State

Did Meatless Mondays bring down Texas Agriculture Commissioner Todd Staples?


September 19 • 7:31 AM

Savor Good Times, Get Through the Bad Ones—With Categories

Ticking off a category of things to do can feel like progress or a fun time coming to an end.


September 19 • 6:00 AM

The Most Untouchable Man in Sports

How the head of the governing body for the world’s most popular sport freely wields his wildly incompetent power.


September 19 • 4:00 AM

The Danger of Dining With an Overweight Companion

There’s a good chance you’ll eat more unhealthy food.



September 18 • 4:00 PM

Racial Disparity in Imprisonment Inspires White People to Be Even More Tough on Crime

White Americans are more comfortable with punitive and harsh policing and sentencing when they imagine that the people being policed and put in prison are black.



September 18 • 2:00 PM

The Wages of Millions Are Being Seized to Pay Past Debts

A new study provides the first-ever tally of how many employees lose up to a quarter of their paychecks over debts like unpaid credit card or medical bills and student loans.


September 18 • 12:00 PM

When Counterfeit and Contaminated Drugs Are Deadly

The cost and the crackdown, worldwide.


September 18 • 10:00 AM

How Do You Make a Living, Molly Crabapple?

Noah Davis talks to Molly Crapabble about Michelangelo, the Medicis, and the tension between making art and making money.


September 18 • 9:00 AM

Um, Why Are These Professors Creeping on My Facebook Page?

The ethics of student-teacher “intimacy”—on campus and on social media.


September 18 • 8:00 AM

Welcome to the Economy Economy

With the recent introduction of Apple Pay, the Silicon Valley giant is promising to remake how we interact with money. Could iCoin be next?



September 18 • 6:09 AM

How to Build a Better Election

Elimination-style voting is harder to fiddle with than majority rule.


September 18 • 6:00 AM

Homeless on Purpose

The latest entry in a series of interviews about subculture in America.


September 18 • 4:00 AM

Why Original Artworks Move Us More Than Reproductions

Researchers present evidence that hand-created artworks convey an almost magical sense of the artist’s essence.


September 17 • 4:00 PM

Why Gun Control Groups Have Moved Away From an Assault Weapons Ban

A decade after the ban expired, gun control groups say that focusing on other policies will save more American lives.


September 17 • 2:00 PM

Can You Make Two People Like Each Other Just By Telling Them That They Should?

OKCupid manipulates user data in an attempt to find out.


September 17 • 12:00 PM

Understanding ISIL Messaging Through Behavioral Science

By generating propaganda that taps into individuals’ emotional and cognitive states, ISIL is better able motivate people to join their jihad.


Follow us


For Charitable Products, Sex Doesn’t Sell

Sexy women may turn heads, but for pro-social and charitable products, they won't change minds.

Carbon Taxes Really Do Work

A new study shows that taxing carbon dioxide emissions could actually work to reduce greenhouse gases without any negative effects on employment and revenues.

Savor Good Times, Get Through the Bad Ones—With Categories

Ticking off a category of things to do can feel like progress or a fun time coming to an end.

How to Build a Better Election

Elimination-style voting is harder to fiddle with than majority rule.

Do Conspiracy Theorists Feed on Unsuspecting Internet Trolls?

Not literally, but debunkers and satirists do fuel conspiracy theorists' appetites.

The Big One

One in three drivers in Brooklyn's Park Slope—at certain times of day—is just looking for parking. The same goes for drivers in Manhattan's SoHo. September/October 2014 new-big-one-3

Copyright © 2014 by Pacific Standard and The Miller-McCune Center for Research, Media, and Public Policy. All Rights Reserved.