The inherited curse of Huntington’s and new hope for a cure
As with the case in the High Court, doctors hid a Huntington’s diagnosis from Deborah Goodman’s family, she tells Rosa Silverman
Deborah Goodman was seven when her father’s personality changed. “He became clumsy, forgetful, argumentative,” she says, “but he kept saying there was nothing wrong.” In fact, there was something wrong that would profoundly affect the lives of his entire family. Ken Goodman, then a 33-year-old engineer living in Hornchurch, East London, had inherited Huntington’s disease.
As his decline continued, and it being the early Seventies, he quickly ended up in a mental hospital. At which point, something occurred to Deborah’s mother, Heather.
“My mum said, ‘Oh! His dad was in a mental hospital.’ So they got [my late grandfather’s] medical records and they said, ‘Huntington’s disease – family not to be told.’ We think it was my Nan who said not to tell anyone. . If my parents had known, they wouldn’t ouldn’t have had children.”
By the he time Deborah’s parents s learnt that the devastating ating degenerative brain disease isease ran in Ken’s family, amily, they had five children. ldren. With a 50 per cent chance of any child of fa a parent with the illness falling victim to it themselves, elves, the future ure looked bleak.
Their r story has echoes of a case now being heard at the High Court in London. A woman who wasn’t told her father had Huntington’s is suing three NHS trusts who she says had a duty of care to inform her. Had she known at the time of her pregnancy, she says, she would have had an abortion. In the event, the claimant – known as ABC – only found out when her daughter, now nine, was four months old.
The details of the case are heartwrenching: Huntington’s was suspected after the claimant’s father shot her mother dead and was detained under the Mental Health Act. He then tested positive for the disease – caused by a mutation of the HTT gene, which produces a toxic protein that slowly damages the brain. The mutated gene is dominant, so only one of your HTT copies (we all have two) need be faulty to cause Huntington’s.
Those who live with it, or have watched a loved one suffer, have h described it as like having motor neurone disease, Parkinson’s, Parkin dementia and depression depressio all at once. The symptoms symp – difficulty concentrating, concentratin memory lapses, lap stumbling, clumsiness clumsines and jerking – usually start between b 30 and 50, 5 and life expectancy expe after afte that is about ab 15 years. y Th There is no cur cure. Not for nothing was it recently called “one of the cruelest, coldest killers on Earth”.
ABC’s case raises difficult ethical questions. “The ruling may have a far-reaching impact on doctor-patient relationships and information that can be shared,” says a spokesman for the Huntington’s Disease Association.
Deborah, now 57 and living in Orpington, Kent, feels strongly that doctors should have told her father, who died of a heart attack a decade after his diagnosis.
“I was petrified,” she says. “Not only for me, but for my siblings. Which of us were going to get it? I suffered with depression. I would break down at work. It was like a big black cloud.”
She decided two things early on: she wouldn’t be tested to learn her fate, and she would never have children.
“I knew that if I had the gene it would be a death sentence; I wouldn’t want to live with that. And I didn’t want a child of mine to suffer the way I suffered: to see their mother struggling and to think that they might have it. The test didn’t come out until I was 30, by which time I’d already made that choice.”
Deborah has not developed the disease, but two of her brothers did, in their thirties. Philip died in his late forties, while Nigel’s case was more complicated: “He always said if he got Huntington’s he wouldn’t want to live with it, and I don’t blame him,” she says. “He was trying to starve himself to death and drink to stay unconscious. He’d had enough but didn’t want to die alone, so he asked my mum.”
In 2000, Heather, then 63, was conditionally discharged for a year after she admitted aiding and abetting Nigel’s suicide on his 42nd birthday. She had cuddled him as he died.
“It has been horrendous and that’s why I think people should be told [if the disease is in their family],” says Deborah. “But there’s divided opinion and I can understand why.”
Indeed, Maureen Waddell, whose husband Mark Lawson died of Huntington’s and passed it on to their daughter Emily, believes responsibility lies with the parents, and not the NHS.
Mark’s family kept from him the fact that his father died of the disease. “His grandfather and father had it,” says Maureen, 47, of Bathgate, West Lothian. “I don’t know why they didn’t tell him.”
She and Mark learnt the truth after doing their own research, and Mark was tested in 1997. When he learnt he had the gene he decided never to start a family, but two months later Maureen got pregnant by accident.
Mark died in 2007, at 34, after Maureen had cared for him for nine years. Then, when Emily turned 14, Maureen started to spot symptoms in her, too. “It was subtle things: her
schoolwork suffered, she became less sociable, clumsy, had mood swings.” She was tested at 18, but Maureen already knew the outcome.
Emily is 21 now, and according to her mother, who quit her job as a nursing assistant to look after her, “has an amazing life” despite the disease. “She is living it in a shorter period but more fully than most people will ever do,” says Maureen.
Deborah, too, is embracing life. At 37, she started to see the possibility of a future, quit her corporate job in telecoms and retrained as a holistic therapist. “I’ve been on a bit of a journey ever since,” she says. “I’m so lucky to have my life.”