Losing your self
"This narrative is us," neurologist Oliver Sacks wrote. Who you are right now is the sum total of what you've done and thought: your childhood and school days, your career, your marriage, your children, your friends, your likes and dislikes, your skills, what you're hopeless at, your moral code.
So imagine if that narrative starts to unravel, if black holes appear in your happiest memories, your most intimate thoughts, your accumulated knowledge, even your most basic skills, so that you no longer know how the bread in your hand relates to the shiny appliance with the slots in front of you. And you can't quite remember your daughter's name, though it's on the tip of your tongue. And imagine how traumatic this process of unravelling would be if you're in the prime of life, have just met the love of your life and are paying off a mortgage.
Garry Lovell, 50, knew in his late 30s that he had the gene that could lead to early-onset Alzheimer's. His mother had got it at 51, and he nursed her until her death 10 years later. Tormented by not knowing his likely fate, he sought testing for the gene in 2001, very early in his relationship with his partner Mandy. "I said to her, 'Look, it's a horrible thing and it's okay if you don't want to go out with me any more,' " he says. "But she said, 'I'm never going to do that.' "
That must be true love. "It is," he replies seriously. "We love each other a lot."
In 2010, changes in Garry's short-term memory put the couple on alert. Just before Christmas, his annual test confirmed he had the disease. The gene had been expressed: he was 46, his wife was 39. A partner in a Melbourne landscaping business that planted indigenous trees, Garry soon had to quit his job.
While he misses his job, he still enjoys gardening. He's a champion dishwasher stacker, Mandy says, though he has trouble remembering where things go afterwards. He still helps in the kitchen - chopping vegetables, for example - and safety is not an issue. But the logistics of cooking, such as juggling timing, are too hard. "We rely on our memories so much, we don't even realise it," says Mandy, who has scaled back her private psychology practice to support her husband.
"We have our dark times about this," she continues, "where we notice the reality, when you notice something new that he can't do. That's the bit that scares me and I'm sure it scares Garry."
Alzheimer's is the most common form of dementia. While it is generally thought of as an old person's disease, 10 per cent of sufferers get it while still young (by medical criteria, that means under age 65). There are several forms of early-onset dementia - Alzheimer's is just one - and while most sufferers are in their 40s, 50s or early 60s, dementia can strike as early as the 20s or 30s, often due to head injury or AIDS, or as a side-effect of acute disease (one recent case was a teenager who got it as a dreadful aftermath of encephalitis).
Noel Hackett was diagnosed with Alzheimer's six years ago, at the age of 59, after a year of small but mounting bafflements. He was working in a government counselling service for the long-term unemployed, half of whom were homeless. He knew something was seriously wrong when he couldn't get his head around a new computer system. Small failures of memory had caught him out before, but this was like a brick wall. Sometimes his younger clients, weaned on screen-based technology, would help him out, cover for him, while they were in his office.
"I lost my sense of purpose and my sense of being capable," he says, "I used to go to meetings and I'd be thinking, 'I hope no one asks me a question about that.' "
Body blow … Noel Hackett says his diagnosis felt like a “whack to the back of the head”. Photo: Tim Bauer
He worried for a while, talked it over with his wife, thought it might be stress and reduced his working hours. On his first Friday off, in October 2007, he went to see his doctor, an old friend. Hackett was one of the fortunate ones: some people with younger-onset dementia struggle for years to find out what's wrong. Hackett's doctor was onto it straight away and sent him for a battery of tests, including those designed to preclude other possibilities that can cause dementia-like symptoms, such as vitamin B deficiency or a brain tumour. His doctor referred him to a neurologist, warning that it might be Alzheimer's, but it took another year before that was confirmed. Hackett's wife, Jenny Fitzpatrick, says: "It was a very long year, 2008."
The final diagnosis felt like a "whack to the back of the head", Hackett says. "I could see a very dark, long road."
His concentration as he talks is palpable, as if he's feeling his way from sentence to sentence, like walking in the dark, avoiding a steep drop. "I've got some timber, and it's a really soft wood. I haven't really got into it, but I'm not putting myself down for not getting into it. I just haven't got around it or something. I can see it there, I can almost reach it ..." More than once in our conversation, he turns to his wife and asks her, "What am I saying?"
Hackett and Fitzpatrick, a teacher who stopped work to care for her husband, live in a suburban flat in Sydney. They are fun to be with, all gentle banter and laughter. Conversation is halting, however, and Hackett often trails off mid-sentence. He likes to laugh so much it is difficult to know when he is parodying himself and when he has actually lost his train of thought. But away from the presence of outsiders, when they stop putting their best face on, there have been terrible moments of sadness and grief. "One moment it's a joke, the next moment it's awful," Hackett says. "I could be down after you leave." There can be tears and a lot of anxiety, Fitzpatrick admits.
Earlier in his life, Hackett was a priest. Now his faith comes and goes. Sometimes, when he feels low, life just seems "bloody crappy", he says. Other times, like when he sits on his balcony on a balmy day and listens to the birds sing, the world expands. "I think the big picture is immeasurable," he says, adding that he doesn't try to conjure God. "And I don't chase grace," he continues. "I don't chase God to give me another year. I don't think like that at all."
Adrienne Withall, co-leader of Inspired, a research collaboration between the University of NSW, the University of Sydney and several major hospitals, says that behavioural problems are more common in younger-onset Alzheimer's sufferers. Parents can appear apathetic to their children, as though they don't love them. "People think if a person's apathetic and just sitting in a chair, it's not too much of a problem. But it is for children who don't understand why their parent suddenly doesn't seem to care about them. And the other parent often has to work two jobs to keep up the family finances and they become a bit more absent, too."
Withall mentions an Australian woman in her 30s who was diagnosed with Alzheimer's, a single mother with two young sons, both special-needs children. She is racing against the clock to raise her children as well as she can and ensure that they are provided for before the illness claims her. At least she was given time to prepare. Diagnosis for younger-onset patients can take years. When a 45-year-old comes in complaining of memory loss and strange behaviour, Alzheimer's is the last thing most GPs think of. Work or marital stress, depression or menopause are what immediately come to mind, and antidepressants or hormone replacement therapy prescribed.
Diana Sinclair was in her early 40s when she started to show symptoms. At first they were little things. She would forget to close drawers. She would start a study course and not finish it. She was an intelligent and organised woman, according to her husband Ron, and her self-esteem began to dive when she realised she was failing to do things that should have been easy. It put pressure on their marriage and on her relationship with their son and daughter, who were in their early teens. "None of us could really understand and she was in total denial," Ron Sinclair says now. "That was her defence."
In the genes ... despite a family history of Alzheimer's, it took a decade for Diana Sinclair's condition to be diagnosed. Photo: Courtesy of Ron Sinclair
That was in the late '80s. It took a decade for her condition to be diagnosed, after an endless run-around with psychologists, psychiatrists, neurologists and other experts. She was kept on antidepressants for years and prescribed behaviour therapy. Ron remembers one psychiatrist particularly bitterly. "He ended up giving up on her after about three years. He said to me one day [in front of Diana], 'Your wife is the most devious patient I've ever had to deal with, and I suggest you take her to someone else.' "
They'd raised the possibility of dementia with several specialists. The family history was there: her mother and an aunt had died of Alzheimer's. Ron, a biologist, later traced it back through death certificates to the first of her forebears to arrive in Australia from England in the 1860s. It is, Ron says, a rare genetic mutation that causes 3 per cent of cases of the younger-onset disease.
Diana's condition worsened quickly after her diagnosis, though she and her husband still had lovely times together. Sometimes they went to see a film twice because Diana enjoyed it, then forgot it, so Ron could give it to her again, brand new. A turning point came when her driver's licence was taken away. "She fell apart on that very day," he says. "From that day on, she needed 24-hour care. It was a loss of identity, a loss of independence: all the things that she feared came at once."
Although her mind was disintegrating, Diana remained physically strong for years. She died in 2006, after a long descent into a living nightmare. She became violent with frustration and was medicated to complete passivity. Eventually, she was placed in a locked facility. The staff there, most of them not trained in dementia or palliative care, did not have the time or interest to help her eat. For five years, Ron swung by the facility every night to feed her morsel by morsel. He wonders now if he did the right thing, keeping up her strength that way.
Diana's death, Ron admits, came as a relief for everyone - not least, he surmises, for her. In a heart-breaking denouement, Ron is now looking after his mother, who also has Alzheimer's.
The Sinclairs came up against a catch-22 of younger-onset dementia. The best specialist knowledge is to be found in aged-care facilities, which is the last place a fit 50-year-old wants to be. Not that younger people are often allowed in: most dementia care programs are available only to people over 65.
Another difficulty arises when sufferers have to face the disease alone. Diagnosis for these sufferers is usually reached much later, because there's no one around to notice the accumulation of memory slips and unusual behaviours. A delayed diagnosis means a delay in getting onto medication and accessing services. Without someone to look after them, those living alone need to go into care much earlier: to help them take their medication, to provide healthy food, to soothe the emotional roller-coaster Alzheimer's often triggers.
"Another thing that concerns us greatly is money management," says John Watkins, chief executive of Alzheimer's Australia. "Someone with dementia is open to financial abuse for a whole range of reasons."
Going early into care affects the trajectory of the disease. "It's sad," Watkins says. "We know that once you go into residential care, the decline is rapid. The longer someone lives at home, which is where they want to live and where they should live, the better.'' It is a combination of morale and the change of habits. "Having day-to-day patterns that are structured is really important for someone with dementia. The worst place for someone with dementia is an acute-care hospital, where the routine is so foreign to them."
Alzheimer's disease was first identified in 1906, when a German neurosurgeon, Alois Alzheimer, dissected the brain of a dead dementia patient and described the build up of amyloid proteins into plaques and the growth of neurofibrillary tangles. Since then our understanding has come a long way. Scientists can plot brain-cell death, brain lesions and atrophy, and know that they lead to memory loss, disorientation and hallucinations, and that eventually the brain will forget to direct basic bodily functions, such as chewing, breathing and expelling waste.
We once thought what we called senility was just a stage of being; now we know that Alzheimer's is a terminal illness. We can see the affected areas on MRIs. We can test for a faulty gene in the cases that are genetic, but we still don't know exactly why it happens or how to prevent or cure it. Younger-onset dementia is the more inexorable condition: usually genetic and so both heritable and transmissible. Late-onset is a yet to be properly defined combination of genes, environment and general health.
That dementia comes in more than 100 forms, each with its own causes, presents problems for both diagnosis and research. At the top of the list are: Alzheimer's disease, which accounts for more than 50 per cent of cases; vascular dementia, which relates to general vascular health; fronto-temporal lobar degeneration (most commonly seen in young-onset dementia), which causes behavioural problems including disinhibition; dementia caused by head injuries, including sporting injuries (being punch-drunk, for instance); and alcohol-related dementia. Withall shudders when she thinks of young people's lifestyles choices. "I look at some of the drugs around at the moment, like ice, and I think it's going to be really terrifying. Someone in the Inspired study is working with drug and alcohol services to see who this population is and how we are going to manage them later on."
Keeping the mind active seems to delay onset, which is why those hoping to age gracefully are busy solving crosswords and sudoku and learning new languages. "We now know you can live with a degree of brain atrophy or tissue loss or amyloid load, and it's variable as to how it affects people," says David Ames, a Melbourne University professor who specialises in Alzheimer's. "So you see people who have got significant brain atrophy on a scan, who are still performing quite well. And you see, particularly in young-onset cases, people who don't look as though they've got much brain atrophy at all and yet they've got cognitive difficulties."
More highly educated people seem to deteriorate more slowly and have more brain damage by the time they notice a decline in their faculties. A New York professor of clinical neuropsychology, Yaakov Stern, developed the "cognitive reserve" hypothesis. "If you've had the opportunity to be well-educated, you have more connections to damage, and you can cope with more injury to your brain before it becomes apparent," says Ames. In other words, the more cells and connections your brain has made, the more it can afford to lose.
Researchers have isolated various gene abnormalities that can cause Alzheimer's, but they don't yet know what to do with the information. "I've been saying for 20 years there will be a cure in five, and we still don't have it," says Henry Brodaty, professor of ageing and mental health at the University of NSW. Billions of dollars are being pumped into clinical, epidemiological and drug research because the eventual pay-off will be astronomical. "It's huge money," Brodaty says, estimating that a cure could be worth $20 billion a year.
Even if it doesn't strike us personally, Alzheimer's will cast its shadow on many of us.
"I've come to the realisation that when we talk about dementia to other groups, we're actually talking about ourselves," says John Watkins of Alzheimer's Australia. "Because if you're a woman, and you live to 95, one in two women will have dementia. So it's something that's going to impact many of us."
Garry Lovell is making the best of what life has dealt him. He says he still enjoys his friends' company, though conversation in large groups is impossible. He plays a bit of golf and has taken up tennis. "I'm just trying to live it up and not worry about what might happen," Garry says. "If you kept thinking about it and think, 'Why me?', you're going to miss out on the next two years."
This article originally appeared in Good Weekend.