Living every day with Huntington's
What is love when a person says they hate you? The answer is in Bridget Lyon and Jeff McDonald's New Zealand International Film Festival documentary on Huntington's disease.
Lyon has Huntington's disease, not yet affecting her. Barring a scientific breakthrough, she can't avoid its ugly progression, and it will kill her. Lyon's grandmother had it and her mother has it and others from the maternal line in her family have, too.
Lyon's grandmother, in the days of more ignorance of the disease, was labelled with a mental breakdown. Her mother, 72, was diagnosed with Huntington's in middle age and now, with its inevitable advance, barely knows the people who care for her. The involuntary physical movements that characterise the disease have become so violent she can't move and is in bed or a wheelchair, completely dependent on Lyon's father and outside help.
And here is Lyon, bright and beautiful and 42, but with Huntington's disease and the inevitable decline ready to begin altering her life at any moment.
Her reaction? Make a movie, directed by her husband, Jeff McDonald, so that this little- known disease is aired for understanding through a handful of sufferers and researchers and the couple's own story. It is also a message from Lyon's vibrant present, to herself and her two young children after Huntington's surfaces, if science fails to find a cure in the interim.
Lyon is a film editor and McDonald has also had a career in the film industry. Their documentary, Inheritance, is screening in the New Zealand International Film Festival.
"I thought we had the skills between us and the story to tell," says Lyon.
Most people who inherit Huntington's, she adds, don't get a chance to tell a story, "they don't get a chance to get their heads above water. If you're 20 or 25 when you have symptoms, by the time you're 45, and as your parents die, you're getting it.
"I always thought I would tell the story at some point."
Lyon's mother, Judy, a former schoolteacher, did get a chance to promote awareness of the disease. For many years, before the Huntington's disabled her, she was a prominent part of the Wellington Huntington's Disease Association. For Lyon, the film will extend her work.
"I felt, not an obligation, but a loyalty to her."
Huntington's is a hereditary neuro-degenerative disease with a 50 per cent chance of being passed on to the sufferer's child. There is no cure. It usually strikes in middle age, or even earlier if from the male side. It brings mental and emotional changes which can include aggression and depression, jerky, uncontrollable limb movements, muscle weakness and difficulty swallowing, speaking or moving, then death 10 or 20 years after diagnosis, usually by pneumonia or choking.
By that time, says McDonald, most people's partners, unlike Judy's devoted husband, have left them.
"It's hard," says Lyon, not to take it personally when she says 'I hate you'."
Judy's husband, says McDonald, is completely selfless.
Making the documentary was one of Lyon's reactions to the disease but she has struggled with her own prospects and her mother's frightening decline.
"It has," says Lyon, "been really shocking all along the way, as we watched her brain deteriorate. It challenged our sense of what love is when a person is saying they hate you.
"She hallucinated and had psychotic symptoms. Watching all that horrifies you, and the idea it will happen to you."
Lyon says she started to come to terms with her own prognosis by "letting go".
"As you go towards it you let go of your ego. If you're going to become an idiot you start by letting go of pride."
McDONALD says he is not scared by the future. "If you love somebody you just don't want it to happen. I don't live in a world where it's going to happen. I could wake up tomorrow and there could be a cure. I deal with it each day. No, it doesn't scare me.
"And, no, they're not galloping towards a cure. Not wanting to sound negative but the more they find out the more they find how much more complex it is to fix it."
Most research is done in the United States, he says. Professor Richard Faull at the University of Auckland "is doing his bit".
Lyon's grandmother was never diagnosed with the disease but Lyon believes her doctor was aware she had it "but didn't tell mum". Lyon's mother was in her 50s when the first subtle symptoms emerged and a genetic test led to her diagnosis.
Lyon met McDonald about 14 years ago, around the time her mother began having problems.
"I had an editing company in Sydney and Bridget came to work at my place," McDonald says.
"When we met, Bridget was quite distraught. Something was happening in her family - Huntington's. Almost at our first meeting she asked for a hug and she told me about her mother. When I met her mother, I was expecting worse than she was but you knew something was up with Judy in the early stages. Even five years into the relationship people would say she seemed fine, but she was not.
"It's a very interesting disease, not predictable as to how it's going to manifest.
"Doctors know cognitive symptoms happen 12 years prior to physical symptoms, from forgetfulness to personality changing and irrational behaviour. It's different for everyone. It starts in different parts of the brain. It's quite interesting and that's what makes it so hard to tackle. Not starting in one place makes it quite difficult."
The hug in Sydney led to love and an unplanned son, now 10. Their second son, now seven, was born using in vitro fertilisation and testing to determine he would not carry the gene.
"He's very proud he doesn't have it - 'everyone's got it but I don't have it'," Lyon says. Her first son will learn later, when he is old enough to make an informed decision, if he has the gene, avoiding early trauma.
"It has been a really big journey to live with it," says Lyon.
"The knowledge you're going to get the disease is quite hard, there's denial, and trying to laugh about it. It really does play with your outlook. Obviously everyone dies, but how, and approximately when. You live every day."
A lot of Huntington's disease, says McDonald, "happens behind closed doors".
"With the documentary we wanted people to understand the disease, so it's not scary, to try and take the fear out of it.," he says. "We all want to know what cancer is and what we can do. When it's Huntington's people say, 'What's that?' - and are less likely to be supportive. Tragedies are unfolding behind doors no-one knows about. People haven't shared stories because they're too busy falling apart."
The Inheritance screens at The New Zealand Film Archive on Friday, 6.30pm, Saturday, 4.15pm and Sunday 11am.
The Dominion Post