The fear of the known

We all live with a sense of the unknown. But Mandy Lee knows it intimately.

It's unknown whether Lee will someday start to act erratically, or lose physical and mental function.

It's unknown whether she should have a baby and risk passing on a cruel fate.

Lee's family has a history of Huntington's disease (HD), an inherited genetic disorder that leads to the slow and irreversible loss of mental and physical capacity. Some 30,000 Americans are currently diagnosed with HD, and an additional 150,000 may develop it.

"This is a family disease and it impacts every aspect of the family," said Lee, 27, who lives in Olympia.

On Saturday, the Northwest Chapter of the Huntington's Disease Society of America will hold a symposium at the University of Washington Center of Human Development and Disability. The free event, which runs from 9 a.m. to 4 p.m., will help build community and offer workshops on the latest medical interventions, care options and research updates.

Just this month, researchers reported that the compound histone deacetylase showed promise in reversing Huntington's disease in mice.

In the meantime, HD looms over generations of families like a vengeful matriarch, affecting relationships, finances and futures.

"Huntington's disease has orchestrated my life," Lee said. Her father was diagnosed when she was a senior in high school. She watched as he lost his memory, movement, his ability to swallow.

His decline and death "changed the very essence of what we knew," Lee said.

It also forged her career path. After seeing how HD affected her father, Lee decided to become a vocational rehabilitation counselor.

For years, Lee has put off being tested. Now married, she wants to have a child.

It may be time to make the unknown, known.

"A lot of people don't want to be tested, because you can never undo what you know," Lee said.

"You can never go back."

That personal debate plays out in the office of Donna Ross, a clinical social worker at the UW's HD Center for Excellence.

Ross knows generations of families who have been affected by HD — and understands the angst about being tested.

Those who are tested usually do so before getting married, having children or investing time and money in something like medical school.

"It is so daunting to get this information," Ross said. "And when you have it, what do you do with it?

"People are growing up their whole lives waiting for the other shoe to drop."

Those who are tested are urged to seek counseling, no matter the diagnosis.

Some of those tested positive for HD may veer toward suicide; those spared may suffer from survivor's guilt, or the knowledge that they are going to be the one to care for parents or siblings with the disease.

"It's going to alter their lives, either way," Ross said.

Lee prefers a positive spin: "I see HD as a constant reminder that you should live every day in the present."

Nicole Brodeur's column appears Tuesday and Friday. Reach her at 206-464-2334 or nbrodeur@seattletimes.com.

Keep at it, Dr. Wexler.