Thursday, February 18, 1999
The Cruelest Disease:
destroyed the dreams of a Utah family
||By Norma Wagner
Robert Bishop has learned there are only two ways to look at Huntington's disease—before and after.
Before his wife began exhibiting symptoms of the neurological illness in 1990 at age 27, she had been a patient mother and an efficient homemaker. Amy Bishop was spontaneous, taking the children to Salt Lake City's Hansen Planetarium or Hogle Zoo to break up the day. She made the quilts on their beds and taught them to read. She expertly juggled her duties as wife, mother, cook, maid, financial manager and family photographer.
But over the next five years, her personality began to change as the differences between before and after grew more extreme. "One minute it was her. The next minute it wasn't," said Robert Bishop, 39, of Orem.
As the disease progressed, she became obsessed with making lists, assigning chores and doling out discipline. Over time, her angry outbursts went from emotionally frightening to physically abusive. Her judgment also became impaired. Unbeknownst to Robert, Amy spent $200,000 in savings and debt over a two-to-three-year period. His only clue to where some of the money had gone was the designer children's clothes he later found stuffed in dozens of bags in the basement, where they were stored during different seasons or as hand-me-downs.
Huntington's disease is more than a progressive neurological disorder for which there is no cure. It is a genetic parasite that feeds on the mind and body of its human host, destroying entire families.
And it doesn't stop there. The children of a parent afflicted with HD have a 50 percent chance of inheriting it.
The two oldest Bishop children, Rebecca, 14, and Craig, 11, are aware of the risk. The couple's three younger children—Trevor, 8; Andrew, 5; and Hannah, 3—are simply trying to understand why their mother isn't their mother anymore.
"If I were to design a disease that would provide maximum suffering, it would be genetic instead of viral—you could pass it on to your children. It would begin in midlife after you had achieved posterity instead of striking the very young or very old. And it would affect the mind as well as the body," Robert Bishop said.
"Huntington's disease is that. It's all of those things."
After his wife was diagnosed with HD in 1996, Robert sat down with his five children. "I talked at length about why I fell in love with their mother, how she acted before her illness, what a great mother she had been," he said. "My eldest daughter began to cry and said 'I don't remember that about Mom.'
"When I got married, all I wanted to do was be with my wife, have a family, grow old and enjoy our children. That's all I wanted. That's all she wanted," Robert said, tears filling his eyes. "I still see her the way she was. But in thinking about our future, I think about our afterlife because this life just isn't going to be there for us anymore."
A few years after the diagnosis, Bishop sold his computer-software company and invested the funds so he could provide for his children while caring for them at home.
He created a nonprofit company called CureHD Foundation (the Web site is www.curehd.org) and plans to devote his time to raising money for research into the disease, which he hopes will result in a cure.
"Amy's grandmother had Huntington's. Her [Amy's] grandparents had two children and one of them, Amy's father, got the disease. Amy's parents have six children and three inherited the HD gene," Robert said.
"Unless a cure is found, two or three of my five children will also die from the same disease that has taken their mother from our home."
Because HD is so devastating, many families affected by it don't talk about it. As a result, there is little public awareness about the illness, which affects 40,000 Americans, said Debra Lovecky, spokeswoman for The Huntington's Disease Society of America.
The society funnels about $1.5 million annually into research.
That's a small amount compared with the $36.5 million spent on research for cystic fibrosis, another fatal genetic disease that affects about 30,000 Americans.
Because symptoms begin early in life, cystic fibrosis is viewed as a childhood disease. The median age of survival is 31, though some patients die much younger. "From a fund-raising standpoint, children just have more of an appeal," said Christina Pietrasanta, spokeswoman for the Cystic Fibrosis Foundation in Maryland.
Amy Bishop and her five siblings were tested for Huntington's disease after their father, John Askew, was diagnosed in March 1995 at age 52. Her 29-year-old sister, Julie, has developed physical symptoms of the disease, and another younger sibling has tested positive for the HD gene but is not symptomatic, said their mother, Lael Askew. Julie and her father are in separate nursing homes.
Because of Amy's uncontrollable behavior, Robert said he eventually had to remove her from their home to protect the children. His parents cared for her during 1997. Last year, she moved in with her 56-year-old mother, who is now Amy's guardian.
"We didn't have six children knowing John had the disease," Askew said. "And Amy was five months pregnant with her last child when her father was diagnosed."
Amy is being treated for depression and delusions, but her condition continues to deteriorate. "She's aware of what's going on, but she won't talk," Askew said. "She's stoic and pretty much lives her life in the corner of my dark blue sofa." Amy's children visit once a week, and that is often the only time she shows any emotion—sometimes smiling or asking a question.
"It's tough because the younger kids don't understand why their mother usually won't return their hugs or kisses or why she won't speak," Robert said. "Andrew, our 5-year-old, will grab her face and say, 'Mom, talk.' And the younger children have had crying fits because they incorrectly feel their mother doesn't love them, that she's rejecting them.
"It's been really tough on the kids."
Amy's psychiatrist, Michael Smith, remembers the first time he met her. "She was an attractive young woman who had a kind of sophisticated air about her," he said. "There's been pretty dramatic deterioration since then."
After the family inheritance of the disease became apparent, Robert was forced to face two realities: One was the discrimination against families associated with HD; the other was the bizarre way in which the illness had affected his wife.
He could not find a U.S. laboratory that would test Amy anonymously for the illness after her father's diagnosis. He had just bought a separate health-insurance policy for her and feared a positive test result would jeopardize the new coverage. A Canadian lab finally agreed to test her under an assumed name.
As the disease progressed, the cost of Amy's prescriptions and inpatient psychiatric care hit $125,000 and her carrier balked at paying all but one-fifth of the amount. The company argued that Amy had reached her lifetime limit for psychiatric care, $25,000, and that the symptoms she was being treated for were more cognitive than physical. Robert argued her underlying condition was biological and threatened to sue. The company eventually agreed to cover the entire amount, but assured him it would not do the same in the future. The carrier suggested he enroll Amy in the Medicaid program, which he and Amy's mother did.
Robert also discovered that Amy's diagnosis meant he could not get life-insurance coverage for his children because of the likelihood at least some of them carry the HD gene.
It was only after Amy was removed from the home that Robert realized how much the disease had ravaged her mind. Through acquaintances, friends and household paperwork, he learned:
For nearly a year, Robert avoided subpoena servers from the Utah Attorney General's Office, which was trying to recoup his wife's unpaid medical bills at University Hospital. He said he was forced to refinance his house to consolidate the debt under a lower interest rate, and even made one mortgage payment with the $2,500 he earned by selling the designer children's clothing at a yard sale.
If a large computer firm had not bought his software company when it did, Robert said Amy's medical bills and the debt would have financially ruined the family.
"The odds of our having survived financially are astronomical," he said. "That's why nobody knows about this disease. Nobody talks about it because nobody survives it. They go through divorce, bankruptcy and lifelong misery."
Working out of an office in his home, Robert has assumed new roles. He is now both parents to his children and a fund-raiser in the fight against HD.
"I told my kids I was going to devote my life to raising funds to find a cure and that by the time they were old enough to get it, it wouldn't be an issue anymore," he said. "I told them they just had to trust me."