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'A god among men': Why a family with Huntington's disease is staying hopeful

A family ravaged by the Huntington’s gene is determined to help the Centre for Brain Research find a brighter future for others.

Huntington’s Disease has ravaged Mary’s family – the progressive neurodegenerative disease has claimed the lives of her father-in-law, a brother-in-law and, two years ago, her husband of 40 years. Both her sons inherited the gene from their father, and they, too, will be affected by Huntington’s sometime in the future.

There is no cure and only limited treatments for the symptoms of the disease, which include uncontrolled movement of the limbs, head and face, difficulty with speech and swallowing and behavioural and cognitive decline. If a parent has the Huntington’s gene, children have a 50/50 chance of inheriting it.

Mary’s family unwittingly connected with what would become the Centre for Brain Research well before its formal launch in 2009. Her father-in-law, Ken, had deteriorated in the years before his death, in 1990. Mary says they had no idea what was wrong with him, but he’d become difficult and unpredictable to be around.

A letter in 1991, from a brain researcher at the University of Auckland, revealed the condition that had affected Ken’s personality and that he died of – and that hangs over current and future generations of the family.

“We don’t even know how we ended up donating Ken’s brain, but I got a letter from Richard Faull and [his colleague] Maurice Curtis giving us the findings of the pathology report. That was our first contact with Richard.”

In 2017, Mary’s husband, Peter, a former secondary-school teacher, died of Huntington’s, aged 76. In hindsight, she sees that her husband’s symptoms had started 20 years before; in his final decade, his behavioural decline led to the couple separating. Despite that, Mary oversaw her husband’s care.

In a bitter twist, the younger of their two sons, Christopher, now aged 40, tested positive for Huntington’s the week his father died. He is now symptomatic, adding to the challenges of living with type-1 diabetes.

The couple’s older son, Anthony, married and living in Spain, also has the gene and, through the centre, is taking part in a global drug trial aimed at halting the progress of the disease.

Mary’s story is terribly sad but neither she nor her sons indulge in self-pity. Peter’s brain is now in the Hugh Green Biobank and Mary, Christopher, and Anthony will all, when the time comes, donate their brains for research.

“It was because of a Huntington’s brain that Richard Faull’s team established that there are stem cells in the human brain. If they hadn’t had that brain, which showed new cells being grown, they wouldn’t have known that. So, why wouldn’t you donate a brain?

“I’ll be dead; it’s not going to affect me or our family. It was, as I like to say, a total no-brainer.”

Mary, whose professional life included working in academic administration, is now dedicating herself to publicising Huntington’s and removing the stigma from it, so that people with the disease are better understood by family and friends. She’s moved from Hamilton back to the small town where she and Peter spent most of their 40 years together.

“The marvellous thing about going back there is that it reminds me that we were very happy there. We had a good life together. Because of the Huntington’s, he turned against me. But now, 20 years of ghastly memories have been wiped out and the good ones reinstated.

“There are people who were taught by Peter and they remind me he was a fabulous teacher and they thought he was a really good guy.”

Peter died calmly and comfortably and Mary is thankful for that. “He didn’t know us but he knew the kids were there. And it was really important for the boys to see him die as peacefully and well as he did.”

Mary, aged 70, who suffered a stroke last year, could be forgiven for being bitter or resentful about the cards dealt to her family, but she’s not.

“I’m not Pollyanna, but I look at the world reasonably positively and I hope I have helped my kids with that, too. My eldest, Anthony, told me the other day that he thought he was one of the luckiest people in the world. I said, “What makes you say that?”, and he said, “Well, if I ignore the brain thing and the fact that half of my family is dead and the other half is dying, I’m having such a good life that I really am one of the luckiest people in the world.” That’s quite a legacy, isn’t it?”

Mary says she is relieved that the Centre for Brain Research’s future has been secured with the huge donation from the Hugh Green Foundation.

“Richard Faull is, to me, a god among men. He and the centre team – their philosophy is about people and families such as ours. They respond to you, they’re immediately accessible, and that is not the norm for those sorts of institutions. It’s just wonderful for me and my family and others like us. Their support is outstanding.”

This article was first published in the November 30, 2019 issue of the New Zealand Listener.