Rare genetic disease is mysterious killer

Cure remains elusive for Huntington’s

Donna Meigs, who was diagnosed with Huntington’s disease last year, relaxes Wednesday in her Fayetteville home with her dachshunds, Alley (left) and Lydia. Meigs is among those promoting a new Huntington’s support group in Fayetteville.

Donna Meigs, who was diagnosed with Huntington’s disease last year, relaxes Wednesday in her Fayetteville home with her dachshunds, Alley (left) and Lydia. Meigs is among those promoting a new Huntington’s support group in Fayetteville.

Monday, June 25, 2012

— Sometimes it’s easier for retiree Donna Meigs to explain the physical symptoms of her Huntington’s disease to people by dodging the issue.

She tells them she has Parkinson’s disease, and they nod in understanding as actor Michael J. Fox comes to mind.

“Until somebody rich and famous gets this, you’ll always have to explain what Huntington’s disease is,”Meigs said Wednesday as she relaxed in her Fayetteville home, two dogs in her lap.

There’s no cure for the hereditary, degenerative brain disorder, which is fatal within 10-25 years of onset, nor are there treatments to slow its progression. Symptoms include involuntary movements and physical and cognitive declines.

“It’s like a cross between Parkinson’s and Alzheimer’s,” the former schoolteacher and school administrator said. “It feels like your body has betrayed you.

“Sometimes I will stumble and it looks like I’m either incredibly clumsy or drunk,” said Meigs, 58. “And doing a little staggering from time to time.”

The child of someone with Huntington’s disease has a 50-50 chance of inheriting the gene that causes it, according to the Huntington’s Disease Society of America. All carriers of the gene will develop the disease.

In 1993, a genetic test be-came available that can accurately determine whether a person carries the gene. But without a cure, some children of people with Huntington’s elect not to take the test. Others want to know because of the chance of passing it on to offspring.

Meigs’ mother, Olga Meigs, learned she had Huntington’s in the late 1990s. She died in 2003 at age 82.

“Until she was diagnosed, we didn’t even know that it ran in the family,” Donna Meigs said.

In 2011, Olga’s sister who lived in Gentry also died from Huntington’s, her niece said.

Donna Meigs, who has no children, took the test in July 2011 and learned she had the Huntington’s gene. For years, she chose not to take the test, but when symptoms that mimicked arthritis and carpal tunnel syndrome emerged, she wanted to know.

One in 10,000 Americans has Huntington’s, according to the national organization. An estimated quarter-million either have the disease or have a parent who has been diagnosed.

NEW SUPPORT GROUP

Meigs recently began attending a new group, the Huntington’s Disease Support Group of Northwest Arkansas.

“Donna actually was able to give them some advice,” said the support group’s founder, Sarah Green, a genetic counselor with the northwest satellite campus of the University of Arkansas for Medical Sciences who works with Huntington’s patients on her own time.

Meigs had experience helping people apply for benefits and knew of a resource for a blind Huntington’s patient who wanted to continue enjoying books, Green said.

The new group has just a handful of patients, family members and caregivers, but Green and Meigs hope that will change once word of its existence spreads.

The meetings began March 15, with subsequent meetings on April 5 and May 29. The next meeting will be from 6-7:30 p.m. Tuesday at theFayetteville Public Library’s Henry Boardroom.

Green doesn’t have a personal connection to Huntington’s. A series of chance occurrences led to her involvement.

She first attended a Huntington’s support group while doing an internship in Boise, Idaho, said Green, a 2010 graduate of UAMS with a master’s degree in genetic counseling.

Her UAMS instructor knew Green had earned her undergraduate degree at the University of Arkansas at Fayetteville and might want to return there.

“She wanted me to move back to Fayetteville and start a Huntington’s support group,” Green said.

Green moved back to take the genetic counselor job. One day when she was taking the family history of one of her pediatric clients, she learned the child’s father had Huntington’s.

“He’d been tested in seventh grade. He told me he was positive,” she said. “There was no support for him. Just his family members - and most of them had died from it.”

Green knew then she hadto carry out her instructor’s wishes.

She began researching what services were available in Arkansas. At first, the nearest Huntington’s support group she could find was in Austin, Texas, so she traveled there to talk to its leaders.

Soon after, she discovered there was an active support group in Fort Smith, so she made contact with its members as well.

The group meets the third Thursday of each month at Fort Smith Public Library, said Sandee Farley, who handles the Fort Smith Huntington’s Disease Support Group’s emails and announcements.

‘HOPED FOR THE BEST’

Farley’s group is already publicizing its 11th annual Walk of Hope, a fundraiser for Huntington’s set for Sept. 22 at Creekmore Park.

“The support group has probably been around as long as the walk, about 12 years,” said Farley, who joined about nine years ago as a family member. “It started with a group of about three people.”

“I think they raised $200 the first year they had a littlewalk,” she said, and probably eight or 10 people turned out. “Last year, we had over 200 walkers and raised $15,000.”

Farley’s husband, Mark Farley, was diagnosed with Huntington’s in 2000. His father died from it in 1984.

Mark Farley opted not to be tested for many years.

“We planned for the worst, and hoped for the best,” Sandee Farley said. “We took out insurance. ... But I guess you just have to reach a point where you’re emotionally ready. That’s a big decision.”

In talking to others who went through what her husband did, she found similar stories: “Some people just say, ‘No, I’m not ready for that news yet.’”

According to the Huntington’s society, other factors that can influence a person’s decision on testing include discrimination from employers and insurance carriers and its own guidelines on pre- and post-counseling requirements before a non-symptomatic patient can be tested.

Green and Meigs said those who may have the gene are urged or required to get counseling to make sure they can handle any bad news, as there is a suicide risk in some patients.

Dr. Kent Westbrook is a surgical oncologist in Little Rock, but after his father and other kin got Huntington’s, he became involved at the national level with a disease completely unrelated to his cancer specialty.

He chaired the Huntington’s society for a number of years, he said.

Westbrook wishes doctors knew more about Huntington’s, but that’s the good and the bad that goes along with such a rare disease.

“Most doctors probably don’t see a single case in their life,” Westbrook said. “Even an active neurologist doesn’t see a lot of cases.”

Meigs recently joined a clinical trial run by Massachusetts General Hospital, East. Its principal investigator at its Fayetteville site is Dr. Alan Diamond, a Fayetteville neurologist.

She’s taking a medication called Coenzyme Q10 - or its placebo.

“Their hopes are that it will slow the progression of the symptoms,” Meigs said.

Northwest Arkansas, Pages 7 on 06/25/2012