The Devastating Effects Of Huntington’s Disease
Once upon a time — at least, it seems like it was that long ago — Albert and Glenna Faye Rodman had 10 children, born over a span of 17 years in rural Arkansas.
And then came the baby of the family, born March 5, 1960: Johnny.
Half the Rodman children inherited Huntington’s disease. Half of those children’s children are in danger of inheriting the fatal disease, too.
In 2011, Johnny’s daughter, Candace, moved to Columbia for graduate school. She has spent the past four years as a long-distance daughter and a mourner. The Rodmans are her family, Huntington’s disease is their scourge, and this is her story.
Sing it with me, if it’s just for today
Maybe tomorrow the good Lord will take you away
On March 19, 2004, Candace Rodman waited.
Earlier that day, in biology class, the teacher had asked about the high schoolers’ weekend plans. “Going to see Aerosmith in Little Rock!” had been Candace’s eager reply, to the blank stares of her classmates. Aero who?
It didn’t matter. None of the people in the room was her daddy, the man who took her hunting and fishing and hiking and camping. None of them listened to old rock ’n’ roll with her. Presumably, none of them were in love with Steven Tyler. But Candace was, and after school, she bolted home in anticipation of the evening ahead.
Johnny Rodman, quiet and witty, got home from work around 5:30 p.m., as usual. Candace clutched the tickets and sat in her blue Volkswagen Beetle, Mr. Lagoon, honking the horn impatiently. The concert started at 8 p.m., and Alltel Arena was 29.2 miles away, and she would not miss a second of it.
After all, Steven Tyler was her idol. Well, one of them. The other was her dad.
When Johnny Rodman was 7, his father, Albert, died from Huntington’s disease at age 57. Glenna Faye would follow years later, in her old age, but not before watching the family go the way of a tree in a hurricane. Of her five children with Huntington’s disease, two died in single-car crashes, one died of unknown causes, and two died in the bitter end-stages of the disease. (Another Rodman sibling died of cancer.)
Huntington’s disease affects an estimated 1 in every 10,000 Americans, or about 32,000 people. The disease is passed down by either affected parent, although in rare cases it can spontaneously arise from genetic mutations. It is caused by an expanded version of the HD gene, which we all have. It seems that the extra genetic coding produces a mutant huntingtin protein, which ends up being toxic for brain neurons. Scientists are still trying to understand exactly how the disease works.
Patients can be any age, although most develop symptoms in middle age, suffer for 10 to 25 years, and die of a complication such as pneumonia. Juvenile Huntington’s disease affects younger people and has its own challenges.
The most visible characteristic of Huntington’s is an involuntary, jerky muscle movement called chorea, from the Greek and Latin words for “dance.” Back when Candace was in high school, it wasn’t clear whether Johnny had inherited Huntington’s. But in college, when Candace’s best friend, Anna Steinhaus, would come home for breaks, she noticed the slight changes that Johnny’s family silently observed: the sudden hike of one leg higher than the other as he walked, or the twitch of a hand when he raised a fork to his mouth. Johnny, stubborn, proud and hurting, started having difficulty writing checks to pay the bills. Candace took over that task for him.
The movement disorder is merely the most visible symptom of Huntington’s. Patients experience personality changes and mood swings that often create rifts in families. Depression is common for HD patients, who also suffer forgetfulness, slurred speech, difficulty swallowing, loss of coordination and more. Suicide rates are estimated at five to 10 times the rate of the typical population.
There is no cure.
The night of the Aerosmith concert in Little Rock, Candace and Johnny hiked to the top tier of Alltel. Rock band Cheap Trick opened. Candace and Johnny peered through camo hunting binoculars, and Candace insisted they stay by their seats, not even venturing out for drinks or food in case they missed Aerosmith’s grand entrance.
Steven Tyler arrived onstage.
“Daddy, it’s him! It’s Steven Tyler!” Candace screamed. “It’s really him in person!”
Johnny probably smiled and told her, with his typical Johnny-humor, that he sure hoped it was really Steven Tyler.
Candace was born in 1987, six years before researchers figured out which gene causes Huntington’s. She doesn’t blame her parents’ generation for spinning the roulette wheel and hoping their kids would be free of the disease. That generation couldn’t know who was sick until symptoms began to show. Earlier generations weren’t even sure what the disease was. Outsiders thought the Rodman family just had some crazy Rodman disease.
Now a genetic test is available to see how many CAG repeats, or extra coding on the HD gene, a person has. People who choose to get tested are often trying to make informed career and family decisions. Her generation, Candace says, has a responsibility to end the disease if medicine won’t. Not knowing her own status, she convinced herself as a teen that she didn’t want children.
“C’mon, take one for the world team,” she told herself. She tells others to do the same. “You don’t wanna burst someone’s dream, but life happens,” she says. “It’s hard.”
Sometimes older parents and grandparents who haven’t developed symptoms undergo testing to calm the fears of their children, because the disease doesn’t skip generations. Two of Candace’s aunts did this for their children.
Presymptomatic testing is a decision for only a minority of those at risk for Huntington’s. Testing is not recommended for everyone, especially children and people who are already at risk for suicide. The test itself can be expensive. And although the Genetic Information Nondiscrimination Act of 2008 prevents health insurers and employers from discriminating based on test results, the law’s protection does not apply to disability or life insurance.
In 2010, long after his symptoms started showing, Johnny decided to be tested. One of his two HD gene alleles had 17 CAG repeats. If Candace inherited that allele from him, she was safe. Johnny’s other allele had 44 CAG repeats. If Candace got that allele, she’d have an early death sentence, too. Her future was as predictable as the flip of a coin.
Johnny didn’t want to deal with the possible guilt of having passed on Huntington’s to daddy’s little girl. But she had to know. In 2008, without telling her parents, 20-year-old Candace decided to be tested.
Johnny and Huntington’s are tearful subjects for Ronna Rodman. She met Johnny when they were both getting Cokes out of the vending machine in her tiny town, and the night he proposed, he’d wondered aloud, “What if I have this disease?”
“What if I have any other disease?” she said. It didn’t change anything.
They married in 1984.
Soon after Candace was born, Ronna and Johnny went to the doctor. They’d already lost one baby to a miscarriage, and although they would never know whether that child had Huntington’s, they didn’t want to risk the disease for any more children.
Ronna says the doctor initially refused, saying Johnny was too young for a vasectomy. Then he heard Huntington’s ran in the family. The doctor scheduled the procedure for less than a week later.
In the days leading up to her 21st birthday in November 2008, Candace waited. She had received the required counseling through her university. She had her blood drawn at her family doctor’s office and sent it off to a testing center. Now all she had to do was wait. Right before Thanksgiving, the phone rang. It was time to know.
Candace was negative.
“It was one of the best feelings ever,” she says. “Ever ever.”
But as the euphoria wore off, guilt settled like dust.
“Oh, good for you, Candace, you don’t have to deal with this horrible disease that your entire family is going through,” she would tell herself. “You had a 50-50 shot and you beat the odds; you cheated death. But guess who didn’t cheat death? Your dad, the rest of your family.”
She told Johnny on one of his last birthdays.
Ronna loved Johnny, loves him still, but it turned out it she was wrong the night he asked her to marry him: Somehow, Huntington’s did change things. She says it’s hard to tell what was the disease and what was Johnny when he refused to talk to her for weeks at a time. For a year and a half, she says, she went to counseling, coming home and asking him why he hadn’t gone with her.
“I had nothing to say,” she remembers him replying. She felt shut out and low. After 25 years and 10 months of marriage, Ronna and Johnny divorced. Ronna would spend years questioning her decision, wondering if she had been selfish. Candace began holding a silent grudge.
Ronna still took Johnny to his monthly doctor appointments, worrying whether it was safe for him to drive. Candace, out of the house by then, spent every free weekend with her father, mowing the lawn or just hanging out with him. She tried to get him to sign up for Social Security and disability and quit his job as the dynamite foreman at a rock quarry. She feared leaving him alone with his hunting guns and the progressing disease.
“With all neurodegenerative diseases, the burden of care ends up being primarily on the family,” says Dr. Irving Asher, director of the Missouri Movement Disorders program at the University of Missouri Health System. Caregivers feel they can’t turn their backs or leave the house for fear of something happening. That becomes a problem, he says, as 24/7 care simply isn’t possible.
Candace’s family watched her try to shoulder the heavy responsibility of caring for her father. Her aunt, Denice Schuler, was one who encouraged her to move to Missouri for graduate school, where Candace could pursue her master’s degree in sports science and rehabilitation.
“You can’t stop your life, even if it’s for your father,” Denice says.
So Candace, stubborn like her dad, finally moved to Columbia. She got a job that fit her tough and outdoorsy personality; she works in property management, doing things like jacking up houses to repair basement walls. Once school started, she took off Fridays and Mondays so she could do homework and visit Arkansas. She talked with Johnny every Monday night.
On Monday, Oct. 28, 2013, Candace played volleyball with her friend Mandy Dixon at Columbia’s Activity & Recreation Center. As they walked to their cars after playing, Mandy noticed she had missed calls from Candace’s friend and boss, Kenny Terry. Candace looked at her own phone and saw 17 missed calls.
None were from her dad.
She called Ronna, who insisted the women drive home before she would tell Candace anything. Mandy followed Candace home and watched as her friend leaped from the car and up the stairs into the living room. Candace was frantic.
Johnny had been driving home from Calico, Ark., that evening, Ronna said. His car wrecked. He’s in Melbourne now, Ronna told Candace.
“When she said he’s in Melbourne now, I knew he wasn’t OK, because there’s no hospital in Melbourne,” Candace says. “There’s a funeral home in Melbourne.”
And just like that, the love of Candace’s life was gone.
Denice, Johnny’s older sister, was devastated. But, she told Candace, she was almost happy that Johnny didn’t have to go through the final stages of the disease she’d watched two other siblings suffer through to curtain call. “Oh, it’s awful at the very end,” she says. “It’s awful. You lose all your dignity, all your health, and all your mind.”
The comment was meant to be a comfort for Candace, but it was only a small one. She’d still give anything and everything to have her dad back.
In 1967, folk singer Woody Guthrie died from Huntington’s disease. His widow, Marjorie Guthrie, started the Huntington’s Disease Society of America, now a leading organization devoted to eradicating Huntington’s and helping families deal with the disease.
Outside of the Huntington’s community, though, the search for a cure still seems to be a low-visibility cause. Candace’s friend Mandy Dixon says it’s frustrating to see so little attention devoted to HDSA fundraisers such as the annual Team Hope Walk that Candace directs in Columbia. Last summer, Dixon watched the ALS Ice Bucket Challenge gain viral status. She wished their friends would give similar attention to Huntington’s.
In Columbia, the University of Missouri Health System’s genetic counselor Meghan Kraus works with Dr. Asher to provide support, information and resources for Huntington’s patients and caregivers. And there are Facebook groups where people can vent and ask for support and advice. Some of the posts are sad stories of relationships ruined by the mental deterioration that comes with Huntington’s. Others are photos of relatives coming home from care facilities for the holidays. Those visits are temporary.
“For Huntington’s patients, there is no hope,” Candace says. “There’s no light at the end of the tunnel. There’s no possibility you’re gonna get over it. There’s no thinking, ‘Oh, this medicine might work for me.’ Beause there is no medicine.”
But there is some hope for a cure in the future. In October 2014, scientists at Washington University in St. Louis announced they’ve found a way to reprogram skin cells into a specific type of neuron affected by Huntington’s. In mice brains, the reprogrammed cells even worked like native cells. The researchers are continuing their research with human cells.
But for Johnny, it’s too late.
For Candace, life without her dad is becoming a shadowy normal. In her bedroom, memories of Johnny linger in every direction: a bin of baseball caps on the floor, his old work shirts and flannel hanging in her closet.
Different personalities and different points of view conspire to weave a rocky dynamic between mother and daughter. To Candace, Johnny was her territory since the divorce, and she bristled when she perceived Ronna acting like a wife when her ex-husband died rather than the role of Candace’s mother. To Ronna, the day Johnny died is the day she lost her daughter.
Candace is getting married in June. Her fiancé, Jeff Kebert, has two young girls. Candace still doesn’t want to have biological children. On her refrigerator hangs a letter from Jeff’s now-5-year-old, the S’s adorably written backward in thick black marker: “YOU ARE THE BEST STEPMOM CANDACE.”
The marriage ceremony will be a small one, held at Glenna Faye Rodman’s farm in Calico Rock, Ark., where the four surviving Rodman siblings still gather every month or so. Candace feels bad about not inviting her friends, but she’s not going to change her mind and plan an extravaganza — not without Johnny.
“If he can’t be there,” she says, “then I don’t want a wedding.”
After the encore of the Aerosmith concert, after Steven Tyler blew a kiss to the crowd that Candace would claim for herself and Johnny would jokingly say was for him, a glittering rain of confetti filled the arena. Candace caught a shiny silver piece, shaped like Aerosmith wings. Johnny bought her a T-shirt that’s still her favorite shirt from her favorite person on her favorite night.
“Leaving was just the saddest thing ever,” Candace says.
But they had to leave. So they got in Candace’s car and drove home.