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

August 20 • 4:00 AM

Of Course I Behaved Like a Jerk, I Was Just Watching ‘Jersey Shore’

Researchers find watching certain types of reality TV can make viewers more aggressive.


August 20 • 2:00 AM

Concluding Remarks About Housing Affordability and Supply Restricitions

Demand, not supply, plays the dominant role in explaining the housing affordability crisis. The wages are just too damn low.


August 19 • 4:00 PM

Can Lawmakers Only Make Laws That Corporations Allow?

There’s a telling detail in a recent story about efforts to close loopholes in corporate tax laws.




August 19 • 12:00 PM

How ‘Contagion’ Became Contagious

Do ideas and emotions really spread like a virus?


August 19 • 10:00 AM

Child Refugees: The New Barbarians

The disturbing rhetoric around the recent rise in child refugees into the United States from Central America may be shaping popular opinion on upcoming immigration reform.


August 19 • 8:00 AM

Making Police Departments More Diverse Isn’t Enough

Local police departments should reflect the communities they serve, but fixing that alone won’t curb unnecessary violence.


August 19 • 7:15 AM

Common Knowledge Makes Us More Cooperative

People are more inclined to take mutually beneficial risks if they know what others know.


August 19 • 6:00 AM

Seeking a Healthy Public School Lunch? Good Luck

Mystery meat will always win.


August 19 • 4:00 AM

The Positive Effects of Sports-Themed Video Games

New research finds sports-themed video games actually encourage some kids to get onto the field.


August 19 • 1:00 AM

DIY Diagnosis: How an Extreme Athlete Uncovered Her Genetic Flaw

When Kim Goodsell discovered that she had two extremely rare genetic diseases, she taught herself genetics to help find out why.



August 18 • 3:30 PM

Mister Rogers’ Heart-Healthy Neighborhood

Researchers find living in a friendly, cohesive neighborhood lowers seniors’ chances of having a heart attack.


August 18 • 2:00 PM

Wealth or Good Parenting?

Framing the privileges of the rich.


August 18 • 12:00 PM

How Much Did the Stigma of Mental Illness Harm Robin Williams?

Addiction treatment routinely fails people with mental illnesses, while mental health care often ignores addiction. And everywhere, stigma is rife. Can a tragic death prompt a more intelligent approach?


August 18 • 10:00 AM

Punished for Being Poor: The Problem With Using Big Data in the Justice System

Correctional departments use data-driven analyses because they’re easier and cheaper than individual assessments. But at what cost?


August 18 • 8:00 AM

What Americans Can Learn From a Vial of Tibetan Spit

Living high in the mountains for thousands of years, Tibetans have developed distinct biological traits that could benefit all of us, but translating medical science across cultures is always a tricky business.


August 18 • 6:00 AM

The Problems With William Deresiewicz’s New Manifesto

Excellent Sheep: a facile approach to an urgent critique.


August 18 • 4:00 AM

Ferguson Is a Serious Outlier

One black city council member is not nearly enough. In a study of city councils, only one place in America had a greater representational disparity than Ferguson, Missouri.


August 16 • 4:00 AM

Six Days in Ferguson: Voices From the Protests

A day-by-day chronology of what happened in Ferguson, drawn from the best reporting by journalists and witnesses on the ground.


August 15 • 4:00 PM

Skirting Ochobo: Big Business Finds a Way Around Local Customs

The “liberation wrapper,” which was designed to shield mouths from public view while eating, has helped a Japanese chain increase sales by over 200 percent.


August 15 • 2:00 PM

How Wall Street Tobacco Deals Left States With Billions in Toxic Debt

Politicians wanted upfront cash from a legal victory over Big Tobacco, and bankers happily obliged. The price? A handful of states promised to repay $64 billion on just $3 billion advanced.


August 15 • 12:00 PM

How the Sexes Evolved

The distinction between males and females is one of the oldest facts of biology—but how did it come to affect our social identity?



Follow us


Common Knowledge Makes Us More Cooperative

People are more inclined to take mutually beneficial risks if they know what others know.

How a Shift in Human Head Shape Changed Everything

When did homo sapiens become a more sophisticated species? Not until our skulls underwent "feminization."

Journalists Can Get PTSD Without Leaving Their Desks

Dealing with violent content takes a heavy toll on some reporters.

Do Ticking Clocks Make Women More Anxious to Have Children?

Yes, but apparently only women who grew up poor.

Facebook App Shoppers Do What Their Friends Do

People on Facebook are more influenced by their immediate community than by popular opinion.

The Big One

One in two full-time American fast-food workers' families are enrolled in public assistance programs, at a cost of $7 billion per year. July/August 2014 fast-food-big-one
Subscribe Now

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