"Most of the time, you feel helpless," says Ray Eby, whose wife Donna and daughter Elizabeth have Huntington's disease. Ray and Donna often sit quietly together in the chapel at the Masonic Health Care Center.
Though her speech is limited, Donna usually manages to get her point across.
Donna and Elizabeth have Huntington's disease, an inherited neurological disorder with devastating physical and psychological symptoms that escalate in the years between diagnosis and death.
Ray spoons raspberry sherbet into Elizabeth's mouth, which she clumsily attempts to open in time for the spoon's arrival. Feeding his 46-year-old daughter is something else Ray never expected to do.
Donna spends time in the sunroom, where watching the tropical fish seems to calm her.
Ray and Donna spend hours watching movies, like "Amadeus" and "Jesus Christ Superstar," on the large flat-screen TV in her room. Even on her worst days, Ray says, Donna is always kind.
Ray Eby expected to while away his retirement years touring Civil War battlefields and fly-fishing at his cabin along Cedar Run.
He always pictured Donna, his wife of 46 years, on the battlefield, by the creek, beside him.
Instead Ray finds himself in a noisy dining room crowded with a maze of walkers and wheelchairs, and people in failing health.
Donna's thin face brightens at the sight of her husband, who has arrived for the first of his faithful daily visits.
But despite Donna's best efforts to greet Ray, she manages only a low moan. Her arms and legs jerk violently, no longer under her control.
Across the room, the Ebys' daughter, Elizabeth Leaman, drinks chocolate milk from a sippy cup. A few stray drops dribble down her oversized bib.
Ray spoons raspberry sherbet into Elizabeth's mouth, which she clumsily attempts to open in time for the spoon's arrival.
Feeding his 46-year-old daughter is something else Ray never expected to do.
Donna and Elizabeth have Huntington's disease, an inherited neurological disorder with devastating physical and psychological symptoms that escalate in the years between diagnosis and death.
Huntington's most often strikes at midlife — in many cases, without warning. Most people with the disease die by age 60, usually from complications like infections, choking or falls.
Huntington's has shattered the lives, homes and plans of three generations of the Eby family, leaving behind a nearly unrecognizable new reality.
"Most of the time, you feel helpless," admits Ray, a 70-year-old retired mechanical engineer.
Donna, 66, can't walk or use her hands. She manages only pureed foods, when she can be convinced to eat at all.
Elizabeth's once-simmering anger — a symptom of the disease — has died down. But she struggles to form sentences longer than two or three words, and sees her teenage sons for just four hours a week.
"Beth's 46," Ray says. "She lost her home. She lost everything."
Ray visits Donna for up to eight hours a day. Cocooned in her little room at Masonic Village, Elizabethtown, they watch endless movie musicals.
Some days, Donna isn't up to visiting, and doesn't hesitate to tell him.
Ray is unfazed. He just comes back later.
He knows that time is running out. Donna has nearly choked to death several times in recent weeks.
A Thief in the Night
Donna first noticed problems with her memory and coordination in the early 1980s.
Worsening symptoms eventually affected her job as a supervisor and quality-control manager for Dutchmaid, an Ephrata garment company.
Donna complained that her body felt "off," like the right and left sides didn't match. Her speech grew slurred, and she had trouble walking.
Multiple doctors' visits and tests revealed no definitive answers.
Ray realized the severity of the problem when Donna brought work home and asked him to help finish it.
Donna's deteriorating condition eventually forced her to quit her job. The Huntington's diagnosis finally came around 1990.
Ray struggled to accept what it meant for their future.
"I hope they come up with a cure for this hateful disease, and things can go back to the way it used to be," he says.
When Donna and Ray met on a school bus in 1959, they never imagined their lives would unfold this way.
Donna was a senior at Garden Spot High School. Ray, 21, was the bus driver.
After their first date to a big-band concert, the couple courted for three years. They eloped on Aug. 4, 1962, and settled in New Holland.
Donna joked about their four-year age difference, and how someday, when they got much older, she'd take care of Ray.
Family Mystery
Donna's mother and grandfather both died of unknown causes at age 50.
"We thought it was cancer," Ray says.
Donna's mother, he says, acted strangely in the years before her death.
It didn't seem significant at the time.
Huntington's typically strikes between ages 30 and 50, often leaving a trail of broken families, job loss and financial turmoil in its wake.
"It's a much crueler disease because of the stage of life it hits people," says Dr. Adam Rosenblatt, clinical director of the Baltimore Huntington's Disease Center at Johns Hopkins School of Medicine.
An estimated 30,000 Americans are currently living with Huntington's disease. An additional 220,000 are at risk for inheriting the abnormal gene that causes it.
The age of onset and speed of progression vary, depending on the nature of the genetic mutation.
For people with no known family history, the diagnosis can be especially shocking.
"Most of them are very normal people before (onset)," says Dr. Xuemei Huang, a neurologist at Penn State Milton S. Hershey Medical Center. "They fall in love and get married. They have children.
"They never know they have the disease."
Huntington's patients display a wide range of symptoms, including speech and motor problems; jerky, involuntary movements (chorea); depression; personality changes; explosive anger; and loss of inhibitions.
Death usually comes 15 to 20 years after symptoms first appear.
Huntington's impact on families is especially cruel.
"It's a very sad situation," Huang says. "They bury their spouse.
"They also bury their children."
In Her Prime
Someone whose parent has the Huntington's gene stands a 50-50 chance of inheriting it.
Donna and a sister have the disease, Ray says. Two other sisters do not.
The Ebys hoped their only child would be spared.
Lively Elizabeth was a cheerleader at Garden Spot, where she also played flute in the band.
"She never caused us any kind of trouble whatsoever," Ray says.
"That really is true."
The problems came later, as Elizabeth approached age 40, the married mother of two young sons.
She had built a career selling Mary Kay cosmetics, hoping to earn the storied pink Cadillac.
For Elizabeth, Huntington's announced itself in signs of depression.
"It's a gradual change," says her former husband, Steve Leaman, of Manheim.
"It's like watching your hair grow."
In fact, Leaman says, friends noticed subtle differences — nervous fidgeting, a slower reaction time, an odd throat-clicking sound — before he did.
Elizabeth deteriorated much faster than her mother. She lashed out at everyone, especially her family.
Leaman says the marriage was troubled long before Elizabeth's diagnosis. Her intense anger only fueled the fire.
"I knew things weren't going to get any better," he says.
Leaman says some people, including fellow church members, judged him harshly for ending the marriage. But he says divorce was the best path for everyone in the family.
The Decision
In 1998, Ray took early retirement from the former Sperry New Holland.
He took over the household chores and learned to cook. To his surprise, he found that he liked it.
But by 2005, visiting nurses noticed Ray's rundown state and urged him to find residential care for Donna.
Ray's search turned up only a few nearby facilities willing to take Huntington's patients.
With Donna's blessing, he chose Masonic Village, with its stately stone buildings and lush green lawns. (Ray, like his father, is a Mason.)
In fall 2005, Donna moved to the village's Masonic Health Care Center. One month later, Elizabeth joined her on the fourth floor.
The cost of Donna's care is staggering, Ray says.
He was forced to spend down his and Donna's considerable savings before government help kicked in.
For several months, Ray dutifully made the daily 34-mile drive from New Holland, where he lived alone in the sprawling modern Colonial he designed in 1977.
Eventually Ray made the difficult decision to sell the family home and move to an apartment at Masonic Village.
A New Reality
Two or three times a day, Ray trudges up the small hill from his apartment to the health-care center.
Warm and outwardly cheerful, Ray is rarely sick, unless you count his macular degeneration and occasional bouts with the flu.
Though he works to keep smiling, Ray admits that visiting his wife and daughter is the most difficult job he's ever done.
He tries to arrive during meals. He doesn't like to see Donna go to bed without eating.
"Sometimes she doesn't feel like eating," he says. "I'll show up, and for some reason, she'll eat."
Before Donna's condition deteriorated, she and Ray bought Civil War books and began making plans to tour battlefields.
Now their traveling is confined to walks around the health-care center and occasionally outside.
Ray pushes Donna's chair into the sunroom, where watching the tropical fish seems to calm her. Sometimes they sit together quietly in the chapel.
The Ebys also spend hours watching movies, like "Amadeus" and "Jesus Christ Superstar," on the large flat-screen TV in Donna's room.
"Wasn't she a doll?" Ray asks, picking up her high-school portrait. "You can see why she caught my eye."
Now Donna is tiny — Ray estimates she weighs 96 pounds — her bones prominent. But to Ray, this isn't cause for alarm. She has always been small.
Though her speech is limited, Donna usually manages to get her point across. She recently called Ray a "bad boy" when he was late for supper.
The Ebys have also developed a shorthand: One blink means no. Two is yes.
Even on her worst days, Ray says, Donna is never angry.
She is always kind.
Testing and Treatment
Ray is a passionate advocate of Huntington's research, even if a breakthrough comes too late for his own wife and daughter.
Donna and Elizabeth are registered with a research bank at Johns Hopkins, one of the nation's largest and most well-respected Huntington's centers.
Both Hopkins and Hershey offer genetic testing for families affected by Huntington's.
Most providers mandate counseling throughout the testing process, which can be a minefield of stress, anxiety and even guilt.
There is no medical reason to have the test, Huang says. Current treatment can't delay onset in people who test positive for the Huntington's gene.
"We offer it to people who want to know," she says. "It's a very personal decision."
At 16 and 17, Elizabeth's sons are too young to be tested. Leaman, who has remarried, says the boys are aware of the odds and how Huntington's could thwart future plans to have their own families.
"When they're old enough (18), it's up to them (whether to have testing)," he says. "They see their mom and see ... where they can be."
(The boys' names are not used here, to protect their privacy.)
Researchers haven't yet discovered a cure or treatment to slow Huntington's assault.
But a multifaceted approach, from medications to speech and occupational therapy, can help manage symptoms. Support for the family is also crucial.
Despite ongoing worldwide research, Rosenblatt says he thinks the medical community has oversold the idea of an imminent breakthrough.
"I've learned to say ... the breakthrough could come tomorrow, or it could come in 20 years," he says.
"Let's talk about what we can do for you today."
Signs of Hope — and More Trouble
Elizabeth takes tetrabenazine, a drug newly FDA-approved for treatment of Huntington's.
Ray is encouraged by its calming effects. Elizabeth's arms and legs are under better control. She smiles often.
She used to look blankly at Ray. Now they can have simple conversations, though her phrases longer than three words are often difficult to understand.
Elizabeth spends most of her time in her room, surrounded by dried flowers, a childhood doll and the Mary Kay products she once sold but now has little use for.
She plays solitaire on the computer and sometimes goes for walks by herself.
Elizabeth's sons — both honors students with creative tendencies — visit most Sunday afternoons.
"I miss them," she says, speaking slowly and with great effort.
Twenty-five years after her onset of symptoms, Donna has to some extent beaten the odds when it comes to living with Huntington's.
But doctors now fear the breast cancer she battled years ago is back.
Donna's weakened state makes it unlikely that she could survive aggressive cancer treatment, Ray says.
So for now, they wait.
The Dawn of a New Life
Ray recognizes the need to forge a life away from the two women who mean most to him — and to prepare himself for the day when both are gone.
He runs a Thursday morning bridge group. Someday soon, he hopes to have time to set up his N-gauge train layout.
For stress relief, Ray calls his brother. Sometimes he sneaks off to his cabin in Lycoming County for a day or two.
He doesn't have much time to cry.
Though Ray is a man of strong Christian faith, he can't help but ask why Huntington's has unraveled his family.
If a miracle happens, he's ready.
"I have a nice big apartment ..." Ray says. "If Donna does get cured, I have room for her."
There's room for Elizabeth, too.
HUNTINGTON'S RESOURCES
Dr. George Huntington first described his namesake disease in 1872. Its most famous victim was probably singer-songwriter Woody Guthrie, who died in 1967 at age 55.
But Huntington's affects thousands of average, everyday families.
Here are some regional resources:
• Baltimore Huntington's Disease Center, Johns Hopkins School of Medicine, (410) 955-2398 or www.hopkinsmedicine.org/bhd...
Services include assessment, treatment, psychiatric care, social-work services, genetic testing, support groups and opportunities to participate in research studies.
Current Huntington's studies include clinical trials of medications and observational studies of people with the disease.
A federally funded grant allows the center to offer most services free of charge.
• Penn State Milton S. Hershey Medical Center, 531-4191 or www.pennstatehershey.org/ne...
Monthly Huntington's disease clinic provides a full range of multidisciplinary care services for evaluation and treatment.
• Huntington's Disease Society of America, 505 Eighth Avenue, Suite 902, New York, NY 10018; (212) 242-1968 or (800) 345-HDSA (4372); www.hdsa.org
Education, resources and support
Hershey support group meets 10 a.m. to noon the third Saturday of every month. Call (877) 384-3721.
CONTACT THE NEW ERA: mschweigert@LNPnews.com or 291-8757
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