Sisters battle incurable, progressive brain disorder
Sisters Tracey Meloche, Tammy Lenz and LeeAnn Harball share the same piercing eyes and infectious laugh.
They also share a degenerative, incurable disease that will cut all three of their lives short if progress isn’t made soon on a cure. The siblings were diagnosed one after the other in 2015 with Huntington’s disease, a hereditary, progressive brain disorder that causes uncontrolled movements, emotional problems and loss of thinking ability through an ongoing breakdown of nerve cells in the brain.
Lenz, 51, was the first to find out what was causing her symptoms that dated back seven or eight years. “They started out being the smallest of symptoms — of not being able to follow a recipe,” Lenz said. Eventually she experienced bouts of uncontrolled anger and an inability to form the correct words for objects. A tic developed below her right eye.
“I knew in my brain that something was wrong, that something wasn’t connecting,” she said. So the search began to find out what was wrong.
Genetic testing gave her the answer.
“When I shared with my (sisters) that I had (Huntington’s disease) then they both immediately knew that they had it because you have the symptoms,” Lenz said.
“You don’t have to really be tested to know.” Huntington’s disease (HD) is caused by an inherited defect in a single gene. Each child of a parent with Huntington’s has a 50 per cent chance of testing positive for the disease.
The sisters said their father, who is estranged, is the carrier but because he has not been part of their lives they didn’t know he had the disease.
They do suspect their grandfather had Huntington’s because of symptoms he displayed, although he was never diagnosed. Males and females are at equal risk to inherit Huntington’s. Symptoms usually appear between the ages of 35 and 55 but can begin before 20 ( juvenile HD) or after 60 (late-onset HD).
Life expectancy is generally about 20 years following onset of visible symptoms. Meloche, 52, was officially diagnosed next, followed by Harball, who is the youngest sister at 47. “When Tammy told us her results, I knew instantly that I was going to test positive as well,” said Meloche, who started noticing symptoms in 2011. “Things started changing, the way I was performing my job, and I also had a lot of
ELI & THE STRAW MAN CONCERT TO BENEFIT HD
Saturday, May 12 Doors: open at 7 p.m., show at 8 p.m.
Royal Canadian Legion Branch 594, 5030 Howard Ave. Tickets: $20. Silent auction and a 50/50 draw
The band Eli & The Straw Man hails from the Niagara region. Band member Tamara Maiuri’s family is affected by Huntington’s disease. Visit: www.hscevents.ca/ Windsoreli
anger issues.
“I would sit at my desk and think about sending an email and think, ‘Is this the right way to word it,’ and I would delete it all, start again and next thing you know, that took me 20 minutes.”
All three have had to stop working because of the progression of their symptoms. The sisters agree coming up with a diagnosis can be challenging because other illnesses such as Alzheimer’s, Parkinson’s and ALS (Lou Gehrig ’s disease), as well as menopause, can have similar symptoms.
Research is being conducted through drug trials to try to “shut off ” the Huntington gene and stop it from making its destructive protein. That would not reverse the disease’s progress but could stop it. The sisters are hoping to get into the next trial, which is expected to start next year.
“Once they stop the progression, then let’s say, God forbid, one of our kids test positive, then they can get that so they shut the gene off (right away),” Meloche said. Harball has five children, one of whom she said tested negative. The other four have yet to be tested. Meloche has two sons — one has tested negative and the other has chosen to not be tested. And Lenz’s two children have not been tested. “I think it’s been really hard. I think my first worry is about passing it to our children,” Meloche said. “And I miss working.” She said the sisters have good days and bad days but events like the upcoming May 12 benefit concert, a fundraiser for The Huntington Society of Canada, help them get through the tough times. The society delivers individual and group counselling services and funds medical research into the disease.
One in every 7,000 Canadians has HD. Symptoms vary from person to person and at different stages of the disease but can include weight loss, involuntary movements, difficulty walking, talking and swallowing, difficulty with focus, planning, recall of information and making decisions, depression, apathy, irritability, anxiety and obsessive behaviour.