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 2 • 10:00 AM

SWAT Pranks and SWAT Mistakes

The proliferation of risky police raids over the decades.


September 2 • 9:12 AM

Conference Call: The Graphic Novel


September 2 • 8:00 AM

Why We’re Not Holding State Legislators Accountable

The way we vote means that the political fortunes of state legislators hinge on events outside of their state and their control.


September 2 • 7:00 AM

When Men Who Abstain From Premarital Sex Get Married

Young men who take abstinence pledges have trouble adjusting to sexual norms when they become husbands.


September 2 • 6:00 AM

The Rise of Biblical Counseling

For millions of Christians, biblical counselors have replaced psychologists. Some think it’s time to reverse course.


September 2 • 5:12 AM

No Innovation Without Migration

People bring their ideas with them when they move from place to place.


September 2 • 4:00 AM

Why Middle School Doesn’t Have to Suck

Some people suspect the troubles of middle school are a matter of age. Middle schoolers, they think, are simply too moody, pimply, and cliquish to be easily educable. But these five studies might convince you otherwise.


September 2 • 3:13 AM

Coming Soon: When Robots Lie


September 2 • 2:00 AM

Introducing the New Issue of ‘Pacific Standard’

The science of self-control, the rise of biblical counseling, why middle school doesn’t have to suck, and more in our September/October 2014 print issue.


September 1 • 1:00 PM

Television and Overeating: What We Watch Matters

New research finds fast-moving programming leads to mindless overeating.



September 1 • 6:00 AM

Why Someone Named Monty Iceman Sold Doogie Howser’s Estate

How unusual names, under certain circumstances, can lead to success.



August 29 • 4:00 PM

The Hidden Costs of Tobacco Debt

Even when taxpayers aren’t explicitly on the hook, tobacco bonds can cost states and local governments money. Here’s how.


August 29 • 2:00 PM

Why Don’t Men and Women Wear the Same Gender-Neutral Bathing Suits?

They used to in the 1920s.


August 29 • 11:48 AM

Your Brain Decides Whether to Trust Someone in Milliseconds

We can determine trustworthiness even when we’re only subliminally aware of the other person.


August 29 • 10:00 AM

True Darwinism Is All About Chance

Though the rich sometimes forget, Darwin knew that nature frequently rolls the dice.


August 29 • 8:00 AM

Why Our Molecular Make-Up Can’t Explain Who We Are

Our genes only tell a portion of the story.


August 29 • 6:00 AM

Strange Situations: Attachment Theory and Sexual Assault on College Campuses

When college women leave home, does attachment behavior make them more vulnerable to campus rape?


August 29 • 4:00 AM

Forgive Your Philandering Partner—and Pay the Price

New research finds people who forgive an unfaithful romantic partner are considered weaker and less competent than those who ended the relationship.


August 28 • 4:00 PM

Some Natural-Looking Zoo Exhibits May Be Even Worse Than the Old Concrete Ones

They’re often designed for you, the paying visitor, and not the animals who have to inhabit them.


August 28 • 2:00 PM

What I Learned From Debating Science With Trolls

“Don’t feed the trolls” is sound advice, but occasionally ignoring it can lead to rewards.


August 28 • 12:00 PM

The Ice Bucket Challenge’s Meme Money

The ALS Association has raised nearly $100 million over the past month, 50 times what it raised in the same period last year. How will that money be spent, and how can non-profit executives make a windfall last?


August 28 • 11:56 AM

Outlawing Water Conflict: California Legislators Confront Risky Groundwater Loophole

California, where ambitious agriculture sucks up 80 percent of the state’s developed water, is no stranger to water wrangles. Now one of the worst droughts in state history is pushing legislators to reckon with its unwieldy water laws, especially one major oversight: California has been the only Western state without groundwater regulation—but now that looks set to change.


August 28 • 11:38 AM

Young, Undocumented, and Invisible

While young migrant workers struggle under poor working conditions, U.S. policy has done little to help.


Follow us


Subscribe Now

When Men Who Abstain From Premarital Sex Get Married

Young men who take abstinence pledges have trouble adjusting to sexual norms when they become husbands.

Your Brain Decides Whether to Trust Someone in Milliseconds

We can determine trustworthiness even when we’re only subliminally aware of the other person.

Young, Undocumented, and Invisible

While young migrant workers struggle under poor working conditions, U.S. policy has done little to help.

Education, Interrupted

When it comes to educational access, young Syrian refugees are becoming a “lost generation.”

No, Smartphone-Loss Anxiety Disorder Isn’t Real

But people are anxious about losing their phones, even if they don’t do much to protect them.

The Big One

One third of the United States federal budget for fighting wildfires goes toward one percent of such fires. September/October 2014 big-one-fires-final

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