They are sisters, but one wouldn't know it. As Julie moves down the hall in her wheelchair, passing a constantly pacing Amy, there's no eye contact or sense of recognition.
Amy Bishop, at 36, has beautiful, dark brown eyes that seem alternately cunning and vacant. Her hair is thick and slightly wavy, but gray is overtaking the dark brown strands. She doesn't curl it now and sometimes forgets to comb it. She's gaining weight and no longer fusses over her clothes, choosing instead to wear a pair of baggy jeans and a slightly rumpled shirt. She seems much older than her years, angry and somehow depleted.
Julie Askew, 29, smiles more often, a perpetual lift at the corner of her mouth. Her eyes, too, are deep brown. But they seem to smile. She's rail thin, wasted first by a car wreck when she was 20 and more recently by the disease that is eating away her mind and body.
Amy can speak but seldom does. Julie loves to talk but is hard to understand, as if the consonants that frame the vowels have disappeared.
They are both sisters and strangers, watching their young lives wind down inside nursing homes, held prisoner by a disease that will eventually rob them of their ability to think, to communicate, to move. It will weaken them. When they are most vulnerable and frail, an opportunistic illness like pneumonia will kill them.Then, the disease will go after Amy's children.
Huntington's is a hideous disease. Formerly called Huntington's chorea (from the Greek word for "dance" for the jerky motions it can cause), it is marked by progressive degeneration from inside the brain. Certain brain cells waste away. Symptom severity depends on how many cells are lost.
Physicians dropped the word chorea because it implies that the dancelike movements are an integral part of the disease. Many people, however, don't have that symptom. The diagnosis was being missed.
At its worst, the disease has been compared to having Alzheimer's, multiple sclerosis and Parkinson's diseases simultaneously.
Most cruel of all, doctors say that people with Huntington's retain their memories of who and what they were before. But it destroys the ability to reason, to communicate, to focus.
Researchers are convinced that if they could find an effective treatment or cure for Huntington's, it would help them understand a host of other illnesses, from depression and schizophrenia to Alzheimer's, Lou Gehrig's disease, Parkinson's disease and cancer.
But there is no effective treatment.
Dr. George Huntington documented the symptoms in 1872 but noted that similar descriptions could be traced back to the Middle Ages. It's unlikely he expected the disease he described as "tragic" to forever bear his name.
Huntington, who was a family practitioner like his father before him, based the conclusions he reached about what he called "hereditary chorea" on long observation of a family living near him on Long Island.
In a paper he presented to the Meigs and Mason Academy of Medicine at Middleport, Ohio, and published in The Medical and Surgical Reporter that year, he concluded that it passed from one generation to the next, wreaking havoc in lives. He also noted that it appeared in adulthood, created a "tendency to insanity" and if it bypassed someone, it didn't show up again in their direct descendants.In the 127 years since, neither time nor technology has challenged those conclusions.
Lael Kunz and John Askew had never heard of Huntington's disease when they fell in love their senior year at Jordan High School.
Life was an adventure ride, and they were anxious to climb aboard. They married on Aug. 25, 1961, just two months after they graduated.
It wasn't easy. John wanted to go to school, but money had to come first. He sat out what would have been his first year of college and they both worked so they could afford a tiny apartment on E Street in Salt Lake City.
Family was a priority, and they started theirs almost immediately. Scott was born in 1962, Amy a year later and Charlie in 1965. Lael stayed home with the kids and John enrolled in finance courses at the University of Utah. He'd go to school, work 50 hours a week, then play with the babies, help feed them and tuck them in before he ever cracked a book.
After Charlie was born, Lael went back to work, and John joined one of his professors in the U.'s Office of Long Range Planning.
Family life and plans for their future consumed them. John was a young man with big dreams. He wanted to take what he'd learned about finance and try it out on Wall Street.
He also had the happy capacity to turn those dreams into action. After he graduated, the Askew family, five members strong, loaded everything they owned into an old station wagon and a U-Haul. They were heading for New York.
The car was so decrepit that Lael's dad followed them up Parley's Canyon to see if they could clear the first hill. They didn't. The car boiled over. "We had $300 in our pockets and big plans," Lael says now, smiling. No overheated car was going to stop them.
John had a scholarship to New York University to get his master's of business administration degree. They stayed with his aunt on Long Island until they could find an apartment in Queens. John got a job with a stock brokerage.
Weekends revolved around church. John was a counselor in his LDS ward; Lael played piano in the Relief Society, led the singing in Primary and taught in the Young Women's organization, besides doing visiting teaching.
Work and school went well. Before long, they could buy a home their first in New Jersey. And the babies kept coming: Julie in 1969, then Jane in 1971.
When a man who had been John's mentor moved to a prestigious company, John followed. Soon he was director of operations. One of the partners became president of the stock exchange. John became vice president of operations. He was the youngest member of the Wall Street Club, a renowned financial group.
He had arrived.
Still, he found corporate America stifling.
He decided to become a lawyer. The family returned to Utah and he enrolled at the University of Utah. History would remember his class because it included serial killer Ted Bundy.
Most families find out that the future can't be scripted for good or bad. Over the decades life would deliver many surprises to this family. But the first a pleasant one came at the end of John's first semester. It was Frank Zarb, John's mentor, calling from the White House.
It hadn't been announced yet, but Zarb had been appointed energy czar and offered John the deputy post.
They were headed for Washington, D.C.
For the Askews, the two years when he was deputy director of the Federal Energy Administration were glorious.
John met regularly with President Ford. He traveled around the country, making presentations on behalf of the administration. He planned meetings at Camp David and rubbed shoulders with powerful people.
Lael and John danced at the White House. They dined on the presidential yacht, the Sequoia, as it cruised up and down the Potomac.
When America turned 200 in 1976, the Askews were on the front row for the birthday party.
Lael's worries were small, her sources of joy many.
"Sometimes I thought maybe we would move back to Utah and he would run for governor or Congress or something," Lael says. "I don't know that he ever thought about that, but I could envision it.
They were careful not to give their children a sense of privilege or entitlement. They shopped at discount outlets. Lael sewed clothes for the family. They were "regular folks" who worked hard and happened to be living an extraordinarily exciting life.
In their free time, they'd ramble, taking car trips to Nowhere in Particular. Exploring was John's passion. Asked where he was going, John would reply, "It doesn't matter."
That phrase would resonate three decades later.
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It didn't take a man of John's impeccable credentials long to find a job after he lost his presidential appointment with Ford's defeat to Jimmy Carter in 1976. He was snapped up by a company specializing in direct marketing insurance. The Askews headed for a nice area of Los Angeles and John became a corporate vice president.
Danny, their baby, was born in California in 1978.
Still, John was drawn to his college-days dream. He wanted to be a stock broker again.
So the young, rambunctious Askew clan returned to Utah in 1981. They built a big house at the mouth of a canyon. They bought a boat and nice cars. They surrounded themselves with creature comforts, high hopes and the camaraderie of an extended family and friends.
They didn't know that just 15 years later, John Askew wouldn't be able to keep a job. He would forget that sleeves go on arms and pants go on legs.They didn't know the future was to be feared.
Dr. John Roberts, a neurologist who specializes in movement disorders including Huntington's, notes that it takes a different form with almost everyone. But there are three common traits:
There's a movement disorder of some kind, though not necessarily chorea. It could be ataxia, which is difficulty walking. Occasionally a patient looks as if he has Parkinson's disease. Instead of being dancelike, the movements are slow and rigid.
Dementia, too, is a common thread. Subcortical reasoning and judgment decline. Concentration disappears.
And there's a psychiatric component that "goes with but is separate from the dementia. Most often, that's depression. But it can be psychosis, with delusions and paranoia. Some people have hallucinations or mania, obsessive compulsive disorder. There's a huge range of psychological symptoms that someone with Huntington's disease can have."
American folk singer and composer Woody Guthrie is perhaps the most famous person to have Huntington's disease. He died Oct. 3, 1967, 13 years after he was diagnosed with it. That diagnosis was hard-won. For years he had been branded a hopeless alcoholic and shuffled in and out of hospitals and mental institutions.
Coincidentally, Guthrie's story is almost identical to that of John Askew's mother, Idelle. She, too, was labeled a hopeless alcoholic and institutionalized. Her husband divorced her and moved away with his two grade-school-age sons, John and Bob. Eventually, they learned that she had died in that institution.
Her sons have never been sure if she was ever properly diagnosed. Their father had never talked about the illness, if he knew of it. They seldom spoke of their mother after the divorce.
The disease is an "autosomal dominant genetic disease." That means it's inherited directly from one parent. Gender doesn't seem to make any difference. One or two of 20,000 people have the disease probably close to 40,000 in the United States. And estimates say another 160,000 Americans have tested positive for the gene and are presymptomatic or haven't been tested but are at risk.
A child has a 50-50 chance of inheriting the gene from a parent. Each child's risk is completely independent of siblings' risks, so it's not supposed to mean that half the children will inherit the disease. But that happens in many families.
As a genetic counselor explained it to Amy's husband, Robert Bishop, in their family with five children, there's a 1-in-32 chance that no child will get it. And the same chance that all the children will inherit the gene. But there's a 90 percent chance that two or three of the children will carry the Huntington's gene.
Neurologists call it a cruel disease, because it usually doesn't show up until someone is 35 to 40 after they've already had their children. Occasionally it appears in young children or very old adults.Not having children seems to be the only effective prevention. But most families have children by the time they know they have the disease. And it begins to claim another generation.
John's problems were subtle at first. And they were perhaps overlooked for a while because Julie had been leading the family on a wild dance. She'd gone from merely stubborn to outright rebellious, staying out late, getting into trouble, going her own way.
That came crashing down, literally, in 1989, when she was in a serious car accident. She would never again be independent.
But it soon became impossible to ignore what was going on with John. He got in a lot of fender benders. He couldn't seem to focus. His mind "wandered," and he hit an ambulance once.
The day he crashed into a telephone pole while trying to park the car out front, he and Lael decided he probably shouldn't drive. Later, he would come to resent that decision and Lael.
He'd been having trouble at work. He couldn't concentrate. Tasks went undone. Eventually, he was fired.
In the past, companies were lined up to woo him away from whoever employed him. Suddenly, he couldn't keep a job. He had three employers in three years, including a job The Church of Jesus Christ of Latter-day Saints gave him in "humanitarian services."
Lael remembers it as humanitarian in more ways than one. It gave John a place to go, a bit of focus. It got him out of the house and into a world where he could use some of the magnificent contacts he'd made over the years.
She was worrying herself sick over bills. Still, he was on top of the world. No matter what went wrong, his wife said, he was "convinced he could do anything." He was always one step away from making a comeback.
He invested in trips to explore his career options. He cashed his checks from the humanitarian services office to buy a mountain bike or some other desired toy. Early on, the bike provided a physical outlet for him, and he rode it everywhere.
He seemed to have no concept of their new financial reality. But Lael knew. At one point, she was working three jobs to make ends meet, rushing home in between to check on John.
They moved to a smaller house. They sold the boat and a car to pay the bills. But they told themselves it was all right. They didn't need that much space. Most of the kids were grown and married. They didn't need the toys.
The other kids had moved out by this time. But Danny, the baby, was growing up in the middle of this. He doesn't remember the glory years when his dad was an important man in Washington. His memories of his dad are more basic.
While he never met the father who worked long hours and was a powerful policymaker in the nation's capital, he's the only one with a child's memories of a father who was always available to him. Who stayed home and played with him. Every day, into his high school years.Danny got the scaled-down life. But perhaps he got to know John Askew better than the other children. There's no question that he had the best view of the devastation that was beginning to take place.
As John's world and therefore his wife's fell apart, the family assumed he was having some kind of nervous breakdown. But that didn't help them figure out what to do. They needed professional help.
Lael started keeping a list of the things that worried her:
John couldn't concentrate.
He couldn't understand "Hi, how was your day?" when the TV was on. He'd have to turn it off and ask, "What?"
He couldn't turn off the light and walk across the bedroom to the bed if he was trying to talk.
And if you gave him instructions to do three sequential things "Pour me a glass of water, turn out the light and bring it down here" he couldn't do it.
He walked leaning to one side. (Guthrie's wife said that Woody walked like he was carrying a guitar on his shoulder.)
And he was getting so thin that Lael was becoming frightened.
She asked the children to add to the list.
Jane, a psychology student at the time, had just finished reading a chapter in a textbook that talked about Huntington's. "Mom, ask the doctor if there's any chance it's that," she said.Lael didn't know what it was, but she wrote it at the bottom of her list: Huntington's?
The doctor said no. John's euphoria pointed more to bipolar disorder (formerly known as manic depression), he said. But he decided to be on the safe side and send them to a neurologist.
Lael will never forget that doctor's appointment. John was taking a simple function test. Touch your nose with your fingertip. Walk a straight line.
Like a drunk pulled over on a busy Saturday night, he was failing it.
She was almost embarrassed to ask about Huntington's, because the other doctor had pooh-poohed it. She had to screw up her nerve.
"Is there any chance it's Huntington's?"
The doctor gave her a brief but curious look. "It almost certainly is," he said.
The Askews still had no idea what Huntington's actually was. But like so many others who have taken a string of disturbing symptoms and tried to find a diagnosis, they were relieved. They had been given a reason with a name for John's increasingly erratic behavior. It wasn't his fault. And he wasn't mentally ill. It was a physical disease that could be pointed to and blamed and treated. Or so they thought.
They didn't know that Huntington's is incurable. That it kills but only after a long time of eating away at a person. That treatment helps symptoms but the disease progresses unchecked.
And though they were told that their children could have inherited the mutant gene, they had no idea what had been unleashed into the family.
Dr. John Roberts, the neurologist who now treats John, remembers thinking once that John Askew, a man who loved his children more than his own life, had planted land mines in his family.They were about to explode.
John and Lael called a family meeting with Amy, her husband Robert and Danny. They were waiting for genetic confirmation but felt they had some answers to John's problems. And it was clear that the children could be affected.
"They told us that John might have a disease called Huntington's and that there was a 50 percent chance the children could have inherited it," Robert Bishop, Amy's husband, remembers.
It was February 1995, and by this time Scott Askew was an attorney in Phoenix, Amy was expecting her fifth child, Charlie was working for Sprint in Kansas City and Jane, the psychologist, was beginning to raise a family in Florida. Danny was the only one living at home.
Julie's life was more complicated. She was still recovering from the brain damage she suffered in the car wreck five years before. They all recognized that she would never be the same.When her parents told her about Huntington's, she said, "I hope I don't have it. I have enough problems already."
Gene testing for Huntington's has only been possible since 1993, though a less-accurate method was developed in 1983, using a genetic marker that had been located near the fourth chromosome. That process involved tracing the marker using DNA samples from not only the subject but close relatives. It was a long and complicated process that was only 95 percent accurate.
A neuropsychologist named Nancy Wexler had used her private fortune to fund the research that found the marker. Her mother had the disease. At that point, only time would tell her whether she would have it, too.
They located the Huntington's mutation in 1993 when researchers figured out the critical area is in a set of three DNA bases or "tri-nucleotides," C, A and G. The number of times those bases are repeated is associated with the chance of developing Huntington's disease.
Everybody has repeats, but the normal range is from 7 to 27. Someone with 27-35 repeats doesn't have Huntington's symptoms but may pass it on to a child because repeats in this range are unstable. At 36-39 repeats, some people will develop Huntington's and some won't. Again, their children are at risk.
Huntington's symptoms are very likely to appear at 40 or more CAG repeats, if a person lives long enough.
Researchers believe the severity of symptoms and age of onset are linked to the number of repeats. But what form the disease will take and what symptoms will appear simply can't be predicted. Roberts points out that's he's seen people with very mild symptoms of the disease, even late in life.
The testing itself is a multistage affair. There's genetic counseling, a neurological exam and some psychological assessment before any blood is even drawn. The assessment will determine whether someone is even considered stable enough emotionally to get the results of such a test.
It takes three to four weeks to get the results, which are supposed to be delivered in person, with a psychiatrist or psychologist and genetic counselor both present. Follow-up counseling is suggested, no matter what the outcome. And if the patient doesn't seem stable, he or she won't be given the results.The patient can also change his or her mind at any time. Until the results are given, it's never too late to back out.
Lael Askew learned one truth in 1995. People are anxious to have genetic testing only so they will know they don't have a disease. And they don't talk much about the testing, even to their siblings or their mother.
A genetic counselor explained it this way to the Boston Globe two years ago: Before testing is done, brothers and sisters are all in the same boat together. Once the testing's completed, some are standing safe on the island and others are swimming with sharks.
In April, the results of John's test came back. He definitely had Huntington's disease. That meant all six of their children were at risk.
Individuals have to decide for themselves whether they want to get genetic testing. Since the advent of testing for the disease, experts have expressed some surprise that not as many people get the test as they had expected.
Perhaps pre-knowledge of the relentless disease is just too much to bear, they posit. Or equally possible, the experts add, people may be reluctant to get the testing done because of insurance concerns.
In a recent study of people at risk for a genetic disease, 63 percent said they wouldn't have genetic testing if the results would be available to insurance companies. The fear is very real.
"Insurance issues potentially pose a real deterrent," said Dr. Jeffrey Botkin, a Salt Lake pediatrician and bioethics expert. "If a company knows what it's doing, it could figure it out. But it's not clear to me to what extent they're sophisticated enough to get family histories."
There's enough evidence of insurance discrimination that the issue has been addressed by a Task Force on Genetic Information and Insurance, created by the Working Group on the Ethical, Legal and Social Implications of Human Genome Research.
Among other things, the group noted, "One of the ironies of the current crisis in health-care coverage is that developing more accurate biomedical data could make things worse rather than better for those most in need.
"Knowledge useful in predicting the individual's likelihood of developing a particular disease opens the door to both the welcome preventive strategies and the unwelcome possibility of genetic discrimination."
It said information about past, present or future health status, including genetic information, shouldn't be used to deny health-care coverage or service and asked insurance companies to consider a moratorium on use of genetic tests in underwriting.
Several states have taken a proactive stance against genetic discrimination, including California, Maryland, New Hampshire, Ohio, Wisconsin and Minnesota, barring discrimination by insurers against people who have a genetic predisposition to a disease. Others are considering it. Utah's Legislature has twice declined to pass a proposed genetic privacy act.
The good news for people with a positive genetic test, according to Robert Perry of the Perry Group is most insurance policies don't ask about genetic testing. And if they don't ask, you don't have to tell them.
But that only deals with genetic discrimination in cases of a future illness. That's very different than having a diagnosis of an active disease.
So people who have Huntington's in their families exchange information on insurance. There are numerous Web pages explaining how important it is to have insurance before you get a diagnosis of Huntington's or similarly devastating and expensive diseases.
And personal horror stories abound. With a disease like Huntington's, where children aren't tested and no one knows until they are adults who will get the disease, some companies deny all at-risk children coverage. Others don't.
In a recent survey, 22 percent of the people with a known genetic condition in the family said they had been denied health insurance coverage because of their genetic status.
A woman with Huntington's in her family suggests that people who are at risk can get insurance, as long as they don't tell about their at-risk status. That may mean not even discussing it with a family physician, whose medical records may be requested by insurance companies, she said.
The important thing to remember, she said, is that you don't "have" Huntington's until symptoms appear, even if you have the genetic mutation. Only then must you list it on an insurance form.
But she cautions people to be careful about the "cause of death" question insurance companies ask: Are your parents and sometimes siblings living or dead and what did they die of?
It's always possible to get life insurance, Perry said. But the cost of the premiums could be significant.
You can get health insurance, too. If you can afford it.And there's another problem with Huntington's, even with good insurance. Most health plans carry a limit on or exclude mental-health coverage. Since much of the treatment offered for Huntington's is psychiatric in nature, policies often try to exclude it.
It was against this backdrop of insurance concerns and outright fear that John and Lael Askew's children wrestled with the question of finding out, individually, whether they carried the Huntington's gene.
The decision on whether to be tested was very private and very painful, Scott Askew remembers. He swayed back and forth. Divorced, with a young daughter, he didn't see how knowing would help him. And he was approaching the prime age for showing signs of Huntington's.
But he desperately wanted to know that his daughter wasn't going to be affected by the disease.
Still, he wavered and put off making a decision.
Robert Bishop, Amy's husband, was particularly concerned about the insurance ramifications for his children. He had become convinced that his wife did, indeed, carry the gene.
It seemed to him that the witty, clever woman he'd married was being replaced by someone who was increasingly anxious and depressed. She said odd things.
For example, she had come to believe that the spacing between children was somehow wrong with her latest pregnancy. Her first four children were each three years apart. This baby was only two years apart. She was convinced the baby would be doomed to failure.
He searched for a place that would allow anonymous testing, while also scrambling to make sure that everyone in his family had adequate health and life insurance, should his fears be realized. A positive test, he reasoned, would make it hard to get insurance for Amy's children whether they should in later years prove to carry the Huntington's mutation or not.
He found he couldn't get additional health insurance for Amy until the baby, Hannah, was 30 days old. And he wasn't about to get a diagnosis until he had the insurance.
The pressure on Amy was tremendous.
Eventually, he arranged for Scott and Amy to be tested anonymously in Canada. Scott remembers that Amy cried all the way to Vancouver. She was falling apart. And Scott was anxious himself about the testing, which they were doing without telling the rest of the family.
Although they were tested together, they couldn't get their results together. Each needed a separate support person, regardless of the outcome, they were told.
They returned home Amy to Orem and Scott to Phoenix to sweat it out. It was just before Christmas 1995.
Some of the others were getting tested at about the same time, but it's hard to pinpoint exactly when. They simply didn't talk about it. The exception was Charlie, who called his mother from the clinic when he got his results. He and his wife Traci would be driving home from Wichita to Kansas City. If something happened to them en route, they wanted to be sure that their children would have the knowledge they needed to face their future.
Jane went with her husband Gary to Tampa, two hours' drive from home, to get the testing.
John's brother, Bob, also flew to Wichita for testing. But he and his wife, Judy, had determined they wouldn't even tell their children about their "Uncle Skip" as they called John, until they knew what they were facing.
"I just wanted to know all the answers to their questions before they asked me," he says now. "We'd even been hiding Lael's letters from them because we didn't want to upset them. I know they thought it was strange that suddenly she stopped writing to us or we stopped enjoying the letters as a family." After he tested negative for the gene, he sat down with his children to discuss the disease.
Lael went with Julie. By that time, she was sure that Julie had the defective gene. "Her physical condition had become such that everyone was pretty convinced that she had it. Getting the test was pretty anti-climactic" by that time. She'd been recovering from the head injury, then she started deteriorating. It looked like she'd been given a double whammy a debilitating accident and a killer disease.
Danny, who had just graduated from Brighton High Shool, where he was a popular kid and a cheerleader, had decided to have the genetic test. The only question was when. First, because there's no treatment or cure, genetic testing isn't available for underage children unless there's a compelling medical reason. He couldn't even consider it before he turned 18. Later, when he got his LDS mission call to Chile, his mother hoped he'd put off testing until he got back. Instead, he was tested in 1997 between the time he got the mission call and when he was supposed to enter the Missionary Training Center.
"I was sure I didn't have the disease," he says. "I knew it could happen, but it wouldn't. I just wanted them to tell me I was all right, so I could go on with my life."
Lael went with him. She, too, was sure that her baby would be OK.
She figured if three of her children had the illness, it would be Julie, who already showed symptoms, Amy, who was acting very different and had become stick-thin like her dad, and Charlie, who was also very thin.
All she could do was wait. Not only for the children to be tested but for them to decide if they wanted to tell her the results.
Two years would pass between the time the first genetic test was done and the last one. And it would be weeks before most of the children would share the results. Those who had the disease needed time to digest the information. Those who didn't weren't sure how to tell those who did.
Scott had already decided that, no matter what the outcome, their world would soon divide into two groups: Those who were healthy and would have to help and those who were sick and would desperately need help.
Jane was the first to call. Then the others started trickling in.Finally, Lael knew. And she was horrified.
When the Bishop kids visit their mother, they never know what to expect.
Will she touch them, look at them, talk to them? Or will she sit silent, rubbing her fingers together incessantly, eyes downcast and out of focus as they chronicle for her the intricacies of their young lives?
One day, she wishes the middle child, Trevor, a happy 9th birthday. Another day, she asks Rebecca, almost 15 and the oldest, when she got braces.
Most of the time, they dance around her, reaching mother-hungry arms for a hug. And they come up with nothing but air.
The children visit her most every Sunday, loading up the car after church to make the drive from Orem to the Salt Lake City nursing home where Amy has lived for nearly three months. She has become a management problem; she's very aggressive. Because of that, for the past six months she's been in and out of the hospital and moved from the nursing home where she and Julie lived doors apart. Now she lives down the hall from her father, so the children usually stop in to see Grandpa John, too.
Sometimes he seems to know who they are. Other times, he seems indifferent.
The older children, Rebecca, Craig and Trevor, look forward to seeing her, though they often come away disappointed and sad. They have some very good memories of their mother though too few for Trevor. "If I was older, I would get to keep more memories," he says.
Andrew, 6, and Hannah, 4, don't really remember Amy as a mother. They were too little. During visits, they're apt to play with each other. Sometimes Hannah amuses herself by dancing for the elderly residents at the nursing home.
"It's hard and easy to go see her," Rebecca says. "She was in the hospital more than a month, and I started looking forward to seeing her. But it's hard to see her, too. She's not what she was."
Robert Bishop makes it a point to go through old photos and talk to his children about their mother and why he fell in love with her. He tells funny stories of things they did. He emphasizes how much she loves her children.
For the most part, the children don't remember that portion of their lives.
While John seemed to fall apart a little at a time over the course of many years, there was really nothing subtle about Amy's descent into what seems to be a personal hell.Looking back, he sees traces of the disease even before John was diagnosed, Robert Bishop said. By the time Amy went with Scott to Vancouver, she was in a downward spiral from which she would never recover.
To understand what the Bishop children have lost, you have to know the Amy who was.
For one thing, she was stunning. And she was very, very witty.
Robert Bishop, by his own admission, can bluster and brag sometimes. When he did, his wife, he said, could "pin me to the wall in no time flat and still make me feel loved."
Most of all, Amy was caring and absolutely dedicated to her husband and babies.
"She was really smart," Rebecca says. "She always helped us with our homework. We'd get home from school and we'd do that first. It was the most important thing."
She also played hard with the kids. On more than one occasion, she kept Rebecca and Craig out of school to go on an educational field trip. They went to the zoo and the planetarium. She helped her children choose topics for reports. That's how Rebecca became something of an expert on polar bears when she was in the second grade.
Amy and Robert's wedding photo, taken May 12, 1982, shows a young, exuberant couple, surrounded by her family. John Askew stands in the back, his face so young and unmarked that he could pass for Amy's not-much-older brother, instead of her father. She looks like a princess. Danny is tiny, about 4 years old and sporting a tuxedo and bow tie. He's leaning casually on his mom's lap. Julie and Jane are flower girls, carrying bright bouquets, while Charlie stands tall and curly haired beside Robert. Scott was serving a mission at the time.
They are the all-American family, obviously delighted by this happy occasion.
Amy had always been a good student. She could get a B without cracking open a book. She was frustrated that her friends sometimes had to study to do that well; she wanted them to have more time to play.
She was also a clothes horse. Her mother says that Amy's room could be a disaster, but her closets were always neatly arranged: pants together, then skirts, then dresses, then shirts. And arranged by color within those categories. She never went anywhere unless she looked just right.
She and Robert met at Brigham Young University. After a couple of dates, they stopped dating other people.
After they were married, he started a computer software company. When the babies came, she stayed home to raise the family and keep the house. The two jobs got her full attention, even devotion.
But things started to change even before John was diagnosed with Huntington's.
Robert Bishop was working long hours by this time, so he admits ruefully that he didn't really know what was happening when he was away. Later he would learn that Amy had been changing, becoming more demanding of the children, especially Rebecca and Craig.
Rebecca remembers having to get up at 5 a.m. to do laundry, hauling heavy baskets of clothes to the closets upstairs. She was about 10 at the time. Sometimes, when the clothes were all clean, Amy would take clean clothes out of the closet and make Rebecca wash them again.
If she asked her mother if she could go out and play, Amy would tell her yes, as soon as the chores are finished. Then she would pile them on until at best there were only a few minutes before it would be too late to play outside.
He didn't know the extent of the problem, but Robert knew that something was wrong. Amy sometimes thought that he was seeing someone else. Sometimes she thought that he was abusing the children. She had become "difficult," in her mother's words; "paranoid," is how Robert put it.
Lael and Robert don't agree on some points: For example, she believes he pushed for the genetic testing for Amy. He says they researched it and discussed pros and cons, but Amy made her own decision.
On this point, Robert Bishop and Lael Askew are solid: Each recognizes that the other loves Amy very much. And that, though they handled differently the challenges they faced when they unknowingly married someone with a Huntington's mutation, they each did the best they could.
By the time Amy was tested, it seemed possible that she actually had the illness. She was erratic, angry and even abusive with Robert and the children. It had been almost a year since she'd said a simple "I love you."
When the test results came back, Robert and Amy Bishop took a cruise. During the moonlit evenings, on deck or in exotic ports, they'd talk. "We talked about getting hired help for her, so that she could just take it easy and love the children and be the queen of the house," Robert says.She was afraid she'd hurt the children. She knew she wasn't holding up well under the strain of having five children. "She made me promise to protect the children from her and from the disease. 'Promise.' 'Promise.'"
Things began to move too fast. In what seemed like a cruel repeat of what happened to Lael and John, Amy and Robert's financial ship was going down.
Amy was spending money like it was water, even writing checks on closed accounts. Then she hid the debt from Robert thousands upon thousands of dollars.
She was also taking her anger and frustration out on the children particularly her oldest son, Craig. One day she screamed in his face, "I hate you! I hate you!"
Another day, she bit Rebecca on the forehead.
Robert knew it was the disease talking. But how do you explain that to children? Especially children who had known a mother who adored them.
He had to protect the kids, who couldn't even begin to understand what was happening to their lives.
He asked the state for help and was told he could put the children in foster care for a while. He didn't want to do that. He had Amy hospitalized a couple of times. As a father, he could remove the children from the home. But he couldn't remove her unless she was declared incompetent and he became her guardian.
Then he called his mother for help.
Carolyn and Joseph Bishop were missionaries in Guatemala at the time. He stayed there to continue the mission, she came home to help her son.
They got a nanny, who stayed for three months. Carolyn helped find another, who lasted one day. Eventually, they got things squared away, or so they thought. Robert's mother went back to Guatemala.
The spiral continued.
Finally, Robert had Amy ruled incompetent. He became her guardian. When his folks came back from their mission in 1996, the family moved Amy in with them.
Carolyn couldn't believe how much Amy had changed. She stopped talking most of the time. She stopped getting up in the morning. She had delusions. Sometimes she'd sit out on the porch even in the bitter cold and wait for hours for her "true love" to pick her up.
Mostly, she'd sit silently and click the TV remote, watching the images fly past her almost-unseeing eyes. She was obviously depressed, even distraught. She was miserable.
One day she mixed baking soda and applesauce together and "ate a ton of it." Her stomach bloated and she was desperately ill. They believed she was trying, in a most ineffectual way, to put an end to her misery. She told Rebecca she'd like to join the military so she could be shot and killed. She even talked to a recruiter.
After about nine months with Joseph and Carolyn Bishop, Amy went to live with her mother.
Again, she mixed the baking soda and applesauce together. Again, she became violently ill.
On Friday nights, Lael would take her to Orem for dinner with the family, then take one or two of the children back to Lael's house for the weekend. It was clear she was sick, but she seemed to do better when she had one or two of the children. She was still interacting at that point.
After awhile, she'd deteriorated to a point where Robert questioned the value of those visits. And he was frustrated because, with his work schedule, weekends were the only time he had to spend with the children.
The overnight visits with Amy tapered to every other week, to Lael's dismay.
And Robert was still struggling under a mountain of debt. "We were, without question, going down the toilet," he said. Medical bills were mounting. He didn't see how he could pay that or the debt Amy had accumulated.
Even those with good health insurance say a disease like Huntington's can run through the lifetime benefits in a hurry. Deductibles alone can break a family.
Dr. John Roberts, Amy's neurologist, shakes his head sadly when asked about how families cope. "You would be surprised how many families divorce in order to stay alive," he said.
The disease puts a lot of pressure on personal relationships. It can be devastating financially.
"It's very common for a couple to divorce" so that the ill spouse can qualify for Medicaid, a federal-state matching program that provides medical care to families if they meet very stringent guidelines.
And this is where Lael and Robert again made different choices. Lael stayed married to John. Robert filed for divorce from Amy, although he is still very involved in her life.
"I had to think about my children," he says. "I could have built a bomb shelter and surrounded us with scripture. But we had to face reality. I had to do something. The insurance company had abandoned us. We were drowning in debt. And as a family, she couldn't qualify for Medicaid."
The decision made Amy's family sad. "In fairness, though, by the time I was faced with that choice and made a different one," says Lael today, "I had nothing left to protect. We'd already lost everything. Perhaps I would have chosen differently had I been in his place."
Because of the divorce, Robert Bishop turned over his guardianship rights to his mother-in-law. He no longer officially has say over what becomes of Amy.
He has started a foundation, called the CureHD Foundation, which attempts to educate people about the disease and raise money to support the research of Nancy Wexler's Hereditary Disease Foundation and the Huntington Disease Society of America. The two groups have prominent scientists who are doing most of the research into the disease.
He's created a Web site, http://www.curehd.org, and spends a large portion of his time disseminating information about Huntington's, keeping abreast of the latest research, trying to think of ways to get more research done.
He is convinced that if he could go on the Oprah Winfrey show, with its audience numbering in the millions, he could tell his story and people would care. Something would happen then, he thinks. Right now, most people have never heard of Huntington's. They don't know how devastating it is for families.
And if a cure isn't found, Robert Bishop believes he will watch some of his children suffer the same fate as their grandfather and their aunt and their mother.
He'd rather die himself than see that.So Robert and Lael made many different choices. And they are too close to see it, but Robert is perhaps the only person in her world who really understands the depths of Lael's nightmare. And only she can understand the fear and the rage he feels for his children's future.
Perhaps some odd cosmic equation explains Julie Askew's cheery disposition.
She is, without question, the most physically devastated of the Askews at this point. She uses a wheelchair all the time. John can walk, albeit jerkily. Amy is still very mobile, though her physical decline seems to be accelerating.
Without a doubt, Julie knows what she has given up to injury and disease. She told her mother once that she knows she will never marry or have children, something for which she longs. She joked that she might adopt, so they could have babies and she could become a grandmother one day.
But every Saturday, she has someone at the nursing home dial Lael's house so that Julie can sing, "Saturday is a special day, it's the day we get ready for Sunday."
Her life has been completing circles for a very long time.
Genetic testing not only showed that Julie had inherited the disease; her CAG repeats were so numerous that the neurologist believes now that she had the rare juvenile form of the disease. It would go a long way toward explaining what for more than a decade was a mystery to Lael Askew: Why is Julie like this?
When she looks back, Lael describes her middle daughter as a "needy child. She needed to be entertained constantly. Jane, just less than two years younger, was her main source of entertainment, and she about wore Jane out."
Jane liked to play by herself and was happiest when Julie was somewhere else so she could.
All three of the Askew girls were singers, the love of which they inherited from their father, John, who always had a beautiful singing voice. The younger pair were in singing groups as they were growing up.
Lael believes now that symptoms of the disease appeared when Julie was a teenager, even before her father was becoming ill. Her parents thought she had low self-esteem, so they enrolled her in classes for voice training, modeling anything to make her feel better about herself.
They encouraged her as she joined the English hand-bell choir at Brighton High and as she sang in choirs and was in school plays. Nothing seemed to raise her self-esteem. She was running with a wild crowd. She had trouble making and keeping friends. Her grades bordered on being disastrous, though her mother was convinced she was as smart as the other children, if she'd just apply herself. Only in music classes did she pull down good grades.
She'd stay out all night, argue with her parents, drive the whole family nuts. "I did not like her at all back then," says big brother Scott. "Now it's a pleasure to be with her."
She had a tender side, even then. She loved animals, especially cats, but she was allergic to them. She still brought them home. Years later, Lael Askew learned that a couple of times Julie went to the pet store, bought the animal and then asked some guy to give it to her as it if were her birthday present so she'd be allowed to keep it. And she always was, though the cats had to stay outside because of her allergies.
Lael and John were worried sick that they'd one day get a call that something terrible had happened to Julie.
The call came in 1989. The 20-year-old and her boyfriend had hit a horse; she'd smashed into the windshield. The physical damage was unbelievable.
She would recover, over time and only to a degree. On her best days, Danny Askew says, "she could go around the corner to the grocery store and get a frozen yogurt and go back home. But that was about all."
Then the physical deterioration of Huntington's began to add to the toll.
And the worse Julie got, the more she seemed to glow almost as if she'd been imparted with some special grace.
She never misses church, never snaps at people, never fails to say "thanks" and "please." John is clearly frustrated and Amy is increasingly hostile and aggressive. But it is poor, devastated Julie who has become the calm in the center of the storm."I feel sorry for you, Mama," she said recently, holding out her twisted arms and hands for a hug. "This isn't fair."
You can get some idea of the relentless pace of Huntington's disease by reading Lael Askew's letters to her brother-in-law Bob and his wife:
In November 1995, she wrote, John went to Phoenix to get together with business associates from his successful days to try to interest them in a financial deal.
When Lael told him to be careful, he hung up on her. She had become the enemy, his prison guard. She's not fun anymore, he complained to their friends.
"He really hates me," she wrote. "Anything I say sounds negative to him. I am purposely trying not to discourage him, but it doesn't matter what I say or how I say it, he ends up hanging up on me. When he originally left, I only hoped he would be gone long enough for me to get some rest and feel relaxed. Now I am worried that he will be gone too long, use his money all up and who knows what he'll do if he is desperate."
In August 1996: John is anxious and agitated. For one week, he sleeps all the time and lacks the energy to walk to the corner and get on a bus. The next week, he can't be left alone. Lael gives notice at her second job but still must finish out the week. When they look at an adult day-care center, John says he'd kill himself first. Instead, he calls friends and acquaintances until he finds someone who will let him stay with them during the day.
The doctor arranges to hospitalize him for a few days to adjust his medications. When he goes back home, he sleeps most of the time. Lael worries that he's too drugged, but she's relieved to know where he is.
He still talks about taking exotic trips. Lael just lets him dream.
By now, she writes, Amy is falling apart.
"Robert is really concerned about her behavior with the kids. In fact, she will be hospitalized before the day is out, either with or without her cooperation. It is so sad. It gives me a new outlook on how John and Bob's mother must have been with them. Amy loves those children dearly, but at the same time she is so mean and talks so mean to them. I just don't know where it all will end. In fact, there is no end in sight. It's pretty discouraging and hopeless and will be perpetuated with yet another generation."I still remember when life was normal but have to think harder now about that than I used to."
A few days later, on their 35th anniversary, John ordained his baby, Danny, as an elder in the LDS Church. He needed help with the wording, but as the family gathered near Scott and his daughter from Phoenix, Amy and her family from Orem, Lael's mother from Payson Lael felt a spirit move through the room and around the circle. She was comforted. In spite of everything, she felt, God held them in his hands.
Later that night, John fell twice. His speech was becoming more slurred. When she wrote to Bob and Judy, she told them she thought they were "just starting the bad part" of the disease.
By December 1996, Julie was beginning to fall a lot. But unlike Amy and John, she was content to sit. Her Huntington's "movement" showed up as flailing arms. John, on the other hand, appeared restless. He'd sit, then stand, then sit again.
Lael says a conversation with Amy, at that point, went something like this: "Amy asked me the other day if a color was her best friend. Then she asked why she was so unlovable ... . She wanted to know from me what my hopes and dreams were for her as she was growing up. I didn't have a good answer, so she asked what my mom's were for me. I didn't know, but I asked her what hers were for Rebecca. She didn't answer.
"She asked me to name three things that she would be worst at if she had to earn a living for herself. I told her that was negative, but rather she should think of three things she would be good at. She persisted, so I came up with three things: rocket scientist, landscaper and I can't remember the third.
"Then I told her I thought she would be a good dietitian, fashion merchandiser or writer. The whole conversation took a half-hour or so, most of which was silence waiting for her to answer a question or make a comment."
Scott calls those days Amy's "bump on a log" days. When he first moved back from Phoenix, he'd go to visit and find her sitting on the couch, expressionless, flipping the remote control. He'd speak to her and she'd ignore him. So he'd sneak behind her, put his hands on her shoulders and bounce her up and down, chanting, "Bump on a log," because that's what she was.
He could always, back then, coax a smile from his little sister. Now he never sees her smile.
In March 1997, Lael made a decision. She was working full time to pay bills but had to leave frequently to deal with John's crises at home. And to cap it all, she had a broken ankle. She was relying on neighbors for help she didn't think they should be asked to give. And she was so tired she could hardly stand it.
She decided John's disease had moved beyond her capability.That's when John entered a nursing home.
Someone meeting John for the first time today would note first that he's handsome and still looks far too young for his 56 years. Next, they'd note that John doesn't quite focus. He tends to ask what day it is over and over. And he becomes fully animated and engaged only when the conversation turns to children or food his favorite thing, since his disease burns up to 6,000 calories a day and leaves him ravenous. He loves both.
At a restaurant, he asks several times if he can have pie but can't remember which kind is his favorite. Lael gently offers him advice: "You want pumpkin? You always love that."
A friend's 2-year-old is sitting in a high chair at the end of the table, coloring. He smiles at her, pats her hand and says, "You're a little honey, aren't you?" Then he retreats into his own world again, while the adults talk around him.
Asked if he remembers someone or what he's been doing or any number of other questions, John replies with an old favorite phrase: "It doesn't matter." But the meaning has changed.He's not a happy man. He's not an unhappy man. He simply is.
Lael will never forget her relief when Scott, Jane and Charlie told her they didn't carry the genetic mutation for Huntington's. Or her grief and rage that her baby, Danny, did. She wasn't surprised that Amy and Julie had the mutation.
"For the couple of years between John's diagnosis and Danny's testing, I'd been known to say I can handle John, Julie and even Amy, but if it turns out that Danny has it, I think my heart will break in two."
Now, Danny occupies the strangest place in this family's tale. He is well but knows he will be sick. The question is when and to what degree. Nobody can foretell that.
At 21, he's a handsome young man with a powerful build and the clean-cut look of a returned missionary, which he is. He got back from Chile in May.
His two years in the mission field gave him time to think about his future and to make some decisions. He got a chance to decide what was important and what wasn't. And it gave him time to read, over and over, a talk given by the late LDS Church President Spencer W. Kimball, entitled "Tragedy and Destiny."
Danny says President Kimball was talking about death and disease. And he said God made people sick for a reason. If he healed everyone, the prophet reasoned, he could mess up God's plan.
"Faith plays a huge part for me. My two years on the mission helped me a lot. My basic belief is this world is short. We'll all be resurrected. We'll all be perfect. And we'll all be together.
"This is just the way it is now."
It is, perhaps, not surprising that Danny takes a comforting long view of life. Because he was growing up when his father was falling apart and his mother had to work extra hours to meet expenses, he "largely raised himself, and did a great job," his mother says.
Lael did what she could to maintain some stability in a very unstable time. As they down-sized their houses, they always left Danny in the same school, so it was like they hadn't moved. He was able to participate in sports and get good grades and enjoy his school experience because he wasn't always uprooted.
And he was always a rock when it came to supporting his family, Scott says.
"It never occurred to me that Danny could get it. I was sure he wouldn't. When he called me, I cried and cried and cried. He was comforting me, telling me it would be OK."
Danny and his mother had already done their crying when they told other family members. He was getting ready to go to the Missionary Training Center. And he decided he just couldn't do it. Then he changed his mind.
"It seemed to me that a mission was a very good place for me to be for the next two years.
"This disease has really opened my eyes," he says today. "I remember my dad starting to get sick. I was 10 or 11, and financially it went downhill fast. I thought that happened to everybody. I would see some of my friends and think, 'Man, that's going to suck when it happens to them.' "
That it doesn't happen to everyone is driven home to him a lot. He played gene roulette unwillingly and seems to have lost. But he's determined not to let it ruin his life, as it has so many others who forget to live in the present while they're busy dreading the future.
"HD will and won't affect my future," he says. "I had to decide, if I have Huntington's, do I want to get married. I know that I need to be married and have a family. Because I have this doesn't necessarily mean I won't do those things. But I will do them differently."
He has a gift that John, Amy and Julie were denied, he says. Time to plan. And to save. "My dad's disease hurt my mom financially. I can do some planning for it. So it will affect me, but some of it will be in a good way."
He knows that the only way to eradicate Huntington's at this point is to choose not to have biological children. And he will grapple with that. In the meantime, he watches with interest scientific advances.
It is possible now to do gene testing on a fertilized egg to see if it contains the mutation, according to Dr. Jay Jacobson, a bioethicist at the University and LDS hospitals. Eggs could be fertilized and tested, and one that was mutation-free could be implanted using in vitro fertilization. The technology exists, although no one seems sure if it has been used with Huntington's disease yet. It's very possible that sperm could be tested prior to fertilization, researchers say.
Danny's not sure how he feels about either of those. But he knows he doesn't want to pass on the disease to another generation. And it comforts him to know that his options for treatment and hope seem to be growing every day.
The disease and the future are not something he readily discusses. His friends know, "I'm pretty sure," but they don't dwell on it. "We're too busy water skiing and working and getting ready for school in the fall."
His own plans are not solid yet. He'll stay close to home to help his family care for John and Julie and Amy. He'll probably attend Salt Lake Community College. He works days as a landscaper, then goes out with a gang of friends.
"I don't really know what's in my head or heart," he confesses. "As real as it is, for me it still seems fake. It's prepared me and matured me a lot.
"I don't think I'm in denial. But I'm healthy. So it's hard to relate to it.
"It's in my family, and I'm dealing with it that way but as if I didn't have the gene. And whether I have the mutation or not, either way, I have to be to work at 7:30 a.m."
It bothers him that what has happened to his dad and to his sisters could happen yet again to Rebecca or Craig or Trevor or Andrew or Hannah. At the same time, when people say they have to find a cure for the kids, it strikes him that he and Julie and Amy were the kids their folks worried about. And on back through the generations.
He wants to see a cure. But he also desperately wants to see effective treatments for those who already have the disease."Please. We can't just forget about Dad, Amy and Julie."
Research breakthroughs have come in dribs and drabs for those who'd like to cure Huntington's. The discovery of the marker in late 1983 was the first really big one. Then, a few years later, location of the actual gene and the advent of genetic testing.
This year has been a good one for Huntington's research.
Recently, the most exciting discovery was that it may be possible to repair the brain. For centuries, the brain was thought to be unchangeable and unrepairable. That notion's being tested and turned upside down.
Some studies involve the use of brain cell transplants to send stem cells in to interrogate and repair cells that have been damaged. It's too early to tell if it will have a dramatic impact for people with Huntington's. But scientists believe the stem cells have an extraordinary, most likely chemical, way of communicating. It boils down to this: When they get to a damaged cell, they ask it if it's OK. And they make healthy copies to repair those that aren't.
Other scientists have discovered that an enzyme, transglutaminase, usually involved with skin formation and healing, appears to be especially active in the brains of people with Huntington's disease. The discovery was made by studying brains during autopsy. They theorize it could be the "smoking gun" behind formation of the disease and therefore the key to the cure.
That discovery was made by a team of U.S. and Israeli scientists. It backs up a theory put forth in 1993 by a Harvard professor, Howard Green, that the enzyme might act as a catalyst for gathering together a protein called huntingtin. Huntingtin is found in clumps in the brain cells of people with Huntington's disease. It's not found elsewhere in their bodies.
A pharmaceutical company, Regeneron Pharmaceuticals, and Medtronic Inc. have also entered into a joint development agreement to collaborate on research and development of a family of treatments for central nervous system diseases. They'll use the pharmaceutical company's experimental treatments and the Medtronic delivery systems. They're especially hopeful that an implantable pump will be able to infuse a medication into the brains of people with Huntington's disease.
The May 20 issue of Nature said scientists have learned that the enzyme caspase-1, present in the brains of both humans and mice, could be important to development of Huntington's disease. By inhibiting the enzyme in the brains of mice that have a syndrome with characteristics of Huntington's, they were able to delay the disease's progression. Caspase-1 is believed to help trigger the death of brain cells. When the enzyme was inhibited, the mouse's life was extended by 20 percent compared to those without the inhibition. And the disease was significantly delayed.
They're hoping that further tests will show it works in human brains, too. But they're nowhere near ready to go for human trials yet. And treatment based on the research, even if it's successful, is years away.
That's all wonderful news, says Robert Bishop. Things are beginning finally to happen. But the budget for Huntington's research is miniscule compared to other diseases. It has long been, and remains, at the bottom of the priority list. The fact that it's gaining any attention thrills him.
But it has come, without question, too late to help Amy or Julie or John.Now the Askews and the Bishops can only hope it's not too late to change Danny's future. And that of his nieces and nephews.
It's bedlam at the Bishop house in Orem. Andrew, 5, answers the door with a sleepy smile and invites visitors in without waiting for an introduction. He's wearing pajamas with fire engines and Dalmatians on them. Grandma Carolyn, their paternal grandmother, is dishing up cereal for the two youngest kids, who keep up a constant stream of chatter. Their father, Robert, is attending the national Huntington's convention.
They like new people, particularly females. They long for a mother who is home doing mommy things, comforting them when they scrape their knees, tucking them in at night. That has become Robert's job.
If she has seen you a couple of times and you're a woman, Hannah may ask you if you'd like to be her mother. It's not personal. It's just a deep-seated longing. She relates more to the nanny they had for a while than she does to Amy, as far as mothering goes.
Rebecca's in the living room, sketching. She's become quite the artist and quite the nurturing force to this band of small people. She helps with the kids, showing a maturity and sense of responsibility well beyond her 15 years.
Craig's apt to be messing with the computer. He loves computer games and videos and "guy stuff." And where Craig is, Trevor won't be far away.
Ask Trevor what he likes and he says, proudly, "All the things that Craig likes. I want to grow up to be like my brother. I like to draw the things he draws."
The older children, if asked, will tell you about their mother and grandfather.
"She doesn't talk," Craig says. "We're used to it, so it doesn't seem like such a big deal now."
What they don't talk about is the fear they feel. They are just beginning to understand that they may one day have the same disease that has taken her from them.
The other day, Trevor told his father that he's angry. He misses his mom. And he's convinced that he will probably become sick, too.
When Robert asks them what they're thinking or if they have any questions, they usually say no. Instead, they introduce the topic a little at a time, in digestible spurts, and not very often.
Mostly, they're busy being children.
Rebecca stretches out on the couch to talk. Trevor walks over and sits on her back. "Should I tickle you hard?" she asks, laughing.
Hannah and Andrew are busy stacking Legos. They're very organized. She has the red and green stack, he has the yellow and blue. When they hold them up together, hers is longer, so she shortens it. Now they're just alike.
The two are, in fact, incredibly compatible.
After a while, they decide to play their favorite game. Andrew runs out of the room and Hannah sprawls on the carpet, an arm outstretched.
"Father," she gasps hoarsely. "Please help me."
Instantly, Andrew is at her side. "Don't worry, Mother," he says earnestly. "I'll save you!"
Around the house, their brothers and sister stop what they're doing to listen and smile.
The older siblings call the game "Father-Mother." Between Lego-building adventures, biking and general kids play, Andrew and Hannah play "Father-Mother" a couple of times a day.
The game has a simple premise and a million variations. Either "Father" or "Mother" is in grave danger. The other one will, somehow, ingeniously and at great personal risk manage a daring rescue. They'll hug and laugh and thank each other, then run off to play with something else.
The older kids wish it was that simple. That fathers and mothers could always rescue each other.
Rebecca halfway believes it. She's convinced that her dad will be able to raise enough money for Huntington's research that they'll find a cure. She's not sure it will be in time to help her mother. But she's pretty sure he'll be able to protect her and her little brothers and sister from the disease.She just wishes he'd hurry.
Sources of information about Huntington's disease
There are many sources for information about Huntington's disease.
On the Web, three sites will provide phone numbers and links, as well as their own wealth of information:
Huntington's Disease Society of America, www.hdsa.org.
Hereditary Disease Foundation: www.hdfoundation.org, call 1-310-458-4183. CureHD Foundation: www.curehd.org, phone 1-801-227-0737.
Project editors: Wendy Ogata and Ray Boren.