Fronto­temporal Dementia: brave testimony of a loving son for a dear mother

Not Fade Away

She slipped away from us gradually, and yet suddenly.

One year she was herself: private, stoic, the proud mother of six and grandmother of legions.

The next she was someone quite different: confused, fearful, a little lost.

The fierce light in her eyes had dimmed to a flicker.

It was her language that went first.

Words began to come to her late, or not at all.

She mixed up people and places and pronouns.

The woman who never forgot a birthday suddenly couldn’t recall her favourite singer, Cliff Richard, or the neighbours of my childhood street.

Eventually she couldn’t differentiate between real life and what she would see on the TV.

I recall her crying just twice in my life, once when we were terrible tweens, and once in a church car park after breast cancer had taken her best friend on its second attempt.

Now she wept often.

Or she would go into confused rages.

My father the target and consoler.

At odd moments she would cast aside her careful English reserve, behaving – as she would have once put it – foolishly.

She was endlessly restless, clasping her purse, ready to go.

But to where?

Away from this new self, perhaps.

It took years to get a diagnosis and in the end it was determined to be fronto­temporal dementia, one of the less common types.

Labels don’t really help, though: every dementia is different, but the result is much the same – you lose your mind.

Finally, after years of looking after her around the clock at home, my father found a care home nearby.

Two days later, while getting up from her Sunday breakfast, something else broke in her brain.

She collapsed to the floor, never to get up.

The family was distraught; my father was devastated.

Ann Broatch, love of his life, wife and companion for the past 50 years, had gone for good at age 69.

A COMING CRISIS

My mother’s story is only one of countless tales of this savage disease. Perhaps 50,000 people in New Zealand have dementia. As our population ages and lives ever longer, the number of people with dementia is predicted to triple. By 2050 we will have maybe 150,000 sufferers out of a population of about 5.3 million on current projections.

We hear about dementia most often when famous people are diagnosed: Terry Pratchett, Ronald Reagan, Margaret Thatcher and actress Prunella Scales. But we almost all know someone’s relative, someone’s mother. No longer is the subject hidden under the rug of embarrassment.

Yet although the stigma is fast disappearing and the awareness of dementia risk increasing, there is still no quick cure. After decades of research and a flurry of news stories about imminent treatment breakthroughs, we are no closer to popping a pill to make dementia go away. The available drugs do work, sort of, but only temporarily. Then it’s downhill again.

But there is now a light at the end of this darkest tunnel.

Leading medical journal the Lancet recently published a comparison of two large studies of dementia numbers in the UK, 20 years apart.

The first, in 1994, found 650,000 people with the condition. With the 2011 repeat survey, using exactly the same methods as the first, researchers with the University of Cambridge project expected to find 900,000 with the disease. Yet it was 200,000 people short.

When age groups were more closely examined, it appeared people were developing dementia later in life.

The Lancet reported the findings as “unequivocally good news”. This research, as New Scientiststressed recently, suggests “people who are able to take control of their lives can reduce their individual risk of dementia”, perhaps through improved cardiovascular health, better diet and higher educational achievement. Another study published in the Lancet appears to support such suggestions.

This research, conducted at the University of Southern Denmark, reported on the health of two groups of Danish people in their mid-nineties, one group born in 1905 and the other in 1915.

Those born a decade later, in 1915, markedly outperformed the first group in a battery of cognitive tests. The 1915 group were not healthier, but they were “smarter”, reported the researchers. Their changed life circumstances had allowed them to build up the kind of cognitive reserve that helps the brain to keep functioning.

Stokes, who is global director of dementia care for healthcare and insurance company Bupa and a visiting professor at the University of Bradford, is wary of the numbers.

But he says the research finding could be the first evidence that for some people, lifestyle combined with “what is often referred to now as the healthy brain – not puzzles but a stimulating lifestyle, engagement, occupation, music” – might be delaying the disease.

Rising prosperity and more education appear to be playing a role too. “There is some research that does show that the longer you spend in education, the less likely it is that you’ll reveal dementia.”

Taking control of our diet, health and activity levels appears to be increasingly important. There are measures, Stokes suggests, that can be taken to prevent vascular dementia, “because if you reduce the risk of stroke, you will reduce the risk of vascular dementia.

It’s the cellular dementias – Alzheimer’s disease in particular – that you want to make some impression on. And there are the beginnings of a sense that if you make inroads into the conditions that logically would have been more associated with vascular dementia, you might have an impact on Alzheimer’s disease: obesity, hypertension, diabetes, depression and underactivity.”

Increasing awareness and fear of dementia may be the very things that motivate individuals to take control of their lifestyles. Surveys that ask baby boomers their biggest concerns for the future usually come up with some combination of maintaining financial independence, health – and mental acuity.

In 2013, a survey of 5000 Australians aged 32-66 commissioned by the country’s pharmaceutical industry body found that dementia was not far behind cancer and heart attack as a serious health concern. “The research shows that once you pass 55, dementia is the health condition you fear most,” says Stokes. “Partly the dread is pertaining to self-oblivion: that’s what makes it different.”

The most common diagnosis of dementia – perhaps 60% – is Alzheimer’s disease, in which the brain’s nerve cells die off surrounded by clumps and tangles of proteins. Memory and language loss (aphasia) is common, as are the appearance of depression, confusion and changes in behaviour.

The next most common variety is vascular dementia, in which the brain’s blood vessels deteriorate and minor strokes occur, leading to sometimes rapid loss of thinking skill. It accounts for perhaps 10-20% of dementia cases.

Frontotemporal dementia, which typically exhibits as aphasia, reduced emotional control and judgment, and sometimes movement problems, is diagnosed in about 10% of cases, but may be more common in people under 65 with dementia. Lewy body dementia, in which abnormal deposits damage the brain, accounts for perhaps 15% of cases.

Symptoms include loss of clarity in thinking and reasoning, hunched posture, psychosis and sometimes vivid hallucinations. Speculation is growing that the older you are, the more likely you are to suffer a combination of the different forms of the disease. You are extremely unlucky to be diagnosed with dementia under 60, says Stokes.

“Two-thirds of people with dementia are over 80.” The average age to be diagnosed with Alzheimer’s is in the late seventies, he says.

“Anyone who is diagnosed at age 70 has about four years of knowing there’s something wrong.” One complication is that the first signs are pretty much indistinguishable from everyday forgetting, and those years of uncertainty are hard for sufferers and their families.”

People typically live 11 years after diagnosis, says Stokes. But before diagnosis there are 15-20 years of pathology in the brain, he says. “By that time the pathology’s so widespread in the brain tissue, I’m not sure how humans can reverse it, and probably not even halt it.”

When Stokes speaks to professional and lay groups, telling them some people will be “dementing now”, few actually change their lifestyle, he says. “When you’re in your mid-forties, you’re not really thinking about dementias, and definitely people younger than that ignore lifestyle advice.”

However, in the same way that cancer often progresses more slowly in older people, later onset of dementia could slow its advance.

Stokes, who has worked in dementia research and care for a couple of decades, starting his professional life as a clinical psychologist, says the G8 got involved this year – the first time the globe’s most powerful nations have met on a health matter since HIV-Aids – because of the crippling costs associated with caring for so many sufferers. “But also because dementia cuts a swathe across society.

It doesn’t respect power or privilege or wealth.” Dementia requires more research, better diagnosis and support, because even with a drop in expected cases, the number of sufferers will continue to rise as we survive longer with the disease.

Every decade, we are living 2.5 years longer on average, says Stokes. “In 40 years, average life expectancy in high-income countries like New Zealand will be around 90 for a man and 92 for a woman.”

In a way, that makes it more of a women’s disease, says Dr Chris Perkins, who has been a psychiatrist for older people since 1992 and is the author of the local reference book Dementia: What You Need to Know. “Women outlive their spouses and are more likely to need care because they are on their own.

Women are the poorly paid formal care­givers and the unpaid family carers – wives, daughters, daughters-in-laws – though husbands often do a fine job if they are still around.”

DIAGNOSIS AND TREATMENT

The UK launched a national dementia strategy in 2009. It is one of 11 countries, including Australia and the US but not New Zealand, that has a national strategy. Its dementia diagnosis rates at the time were about 35-40% and are now 48%. Says Stokes: “One of the reasons you don’t have more being diagnosed early is because GPs say, ‘Well, what are they going to do?’ If there was a wonder drug out there, I am sure more would be put forward.”

Britain is further ahead in this area because of the national strategy and because its population is older, suggests Perkins, who works for The Selwyn Foundation, a charitable provider of elder care. But that’s still less than half of cases being diagnosed, she notes. It’s a problem that New Zealand’s diagnosis rates are only estimated, she says. “We can’t ever tell we’re doing a good job in prevention until we know what the rate is.”

And we have no idea of dementia rates in Maori. “They presumably get it earlier because they’ve got more risk factors.” Those factors include obesity, high blood pressure and poor vascular health.

There are 3240 people in residential dementia care beds and about 32,000 in government-funded aged residential care overall, says the office of Associate Health Minister Jo Goodhew. The rollout over the next year of the internationally developed interRAI clinical assessment tool in both home and residential care settings should help gather more accurate information.

A few years back, a story would appear in the media just about every month promising imminent treatments for dementia. An Australian Medicines Industry report says 98 clinical trials are under way globally “to better understand, treat and ultimately prevent dementia”.

Australian drug companies spend more than A$71 million a year on dementia research. Yet all the drugs so far have been “spectacular failures”, says Stokes. In March, for example, the media went wild when it was suggested a simple blood test might be able to predict Alzheimer’s. Within days, the test’s accuracy was seriously questioned, as was the media’s gullibility.

Look to the pharmaceutical companies, says Stokes. He started to get a sense around 2011 that they were starting to step back, saying they didn’t trust the basic science about dementia, and because so many phase 2 and 3 drug trials ended in failure.

The existing so-called anti-dementia drugs – acetylcholinesterase inhibitors such as donezepil, marketed as Aricept before its patent expired in 2010 – have been around since the late 90s, he says.

“The CEOs [of drug companies] knew they were going to go generic. You know that roughly three or four years into the drug they were saying to the scientists, ‘Come up with the next wave.’ And it didn’t happen. And given the potential, 115 million people close to having this condition in the next 30-odd years, the opportunities are immense. Yet there’s failure.”

Stokes says our knowledge of the mechanics of dementia is still very limited. “We don’t even know if there is anything called Alzheimer’s disease. It could be lots of diseases with the umbrella of Alzheimer’s disease. When you’re trying to put a drug in play, what actually are you asking that drug to do if you don’t understand the basic science?”

“I think of it now as brain failure,” says Perkins. “You get heart failure, for example, and there are all sorts of causes.” They include vascular problems, poisoning and infections. She believes researchers could make incremental progress against the disease, finding a cure for one form, maybe a genetically triggered young-onset form, then maybe another one. “Just like we can treat child leukaemia now, but we’re not very good with malignant melanoma.”

Anti-psychotic drugs are sometimes used to control the behaviours of people with dementia. My mother was on them for more than a year. They didn’t appear to ease her suffering, or my father’s lot, one iota. Stokes says they are a step back into the old disease model.

In Bupa, which has about 450 care homes around the world, he has seen the number of residents on them drop from about 35% to 20%, partly because doctors are less willing to prescribe, but also because staff are more confident about alternative ways of working. Anti-psychotic drugs also have potentially serious side effects. The UK Government demanded a two-thirds reduction in their use because of hundreds of “unnecessary” deaths each year, he says.

“It’s not pathology, it’s about people’s reactions to their lives. Nobody wanders because their brain isn’t working; they’re getting out of chairs because they don’t like where they are. For a whole host of reasons. So if you find out why that might be, you start to go into the world of solutions.”

A harried, exhausted partner or child trying to care at home for a wandering loved one with dementia, of course, may be willing to try anything.

The transformation in care in recent years has been from a disease model, in which everything that happens after the diagnosis was attributed to the diagnosis, to a person-centred therapeutic model, says Stokes.

“It was very nihilistic, very custodial in attitude. These are people who are trying to survive with an intellectual disability that progressively worsens – that’s what dementia is. And everybody’s resourced differently, everybody’s quality of life is different, the relationships they are engaged in are different.”

As Perkins says, all sorts of people get dementia and they all react differently. “There’s a saying: you know one person with dementia, you know one person with dementia.”

In the absence in this country of a national dementia strategy, which usually includes factors such as time frames and extra funding, district health boards are meant to be producing dementia care “pathways”, tracking headway in prevention, awareness-raising and diagnosis all the way through to palliative care. “We’re not sure how everybody’s doing.” DHBs have to report quarterly to the Government on what they’re doing, but this means 21 different reports, Perkins says.

She belongs to the National Dementia Co-operative, a group of mostly health professionals that recently decided to try to track the DHBs’ progress. If pathways are instituted, we are likely to see huge gaps in areas such as provision and training, she says. Some areas have specialised dementia at-home caregiver services, for instance, but others might have general carers who are not trained in dementia care.

Perkins is concerned about how sufferers not in care facilities and their families are coping. Rest homes are audited but we don’t know what’s going on in the community, she says.

Care workers are often not well paid, Stokes acknowledges. “It’s a very challenging area to work in. I’ve got immense regard for the nurses and the care workers. Unfortunately, because of the way it’s now defined as a social-care matter, this is a low-wage economy. We’ve placed the most vulnerable people into the hands of those who are working for wages that most people would say, ‘Forget it, I’m not going to do that.’”

Outside of care homes, the assumption in policy/funding set-ups seems to be that there will be a primary caregiver who is willing and able to do the job.

However, partly because of the initial effects of the dementia, the family relationship is not always strong and sometimes the “well” party would rather not be married to the person at all, much less care for them for years and years. Couples might want to have that conversation when concerns first arise because it’s extraordinarily hard to leave once you or your partner is diagnosed.

When the time comes, supporters need every option available. Relief services, such as personal care and housework assistance, are available, but getting the right help can be a trial, as can being assessed for the carer-support subsidy or finding a suitable care home nearby.

Stokes, not surprisingly, is a proponent of care homes. The current thinking of “living well with dementia” means people’s fundamental needs – healthcare, a safe living environment – are being met. But it also means living long with dementia as survival time increases. The result is total dependency, prior to that unpredictable high dependency, and the burden of care falling on family and society, he says.

“So that’s why even though people say care homes are archaic, let people live in their own homes – true, for as long as possible. For me, carers don’t exist in families. They are caring partners, caring children. To inflict 24/7 total dependency care needs on to a family beggars belief.”

Says Perkins: “I would love to see the Government energetically providing day programmes, as they do for early childhood education.”

A string of “eldergartens”? Regardless of how policy pans out over the coming decades, she says, “It would be great to see people with dementia just treated like people – both in the community and in care. Not people to be avoided, talked over or ignored or feared.”

And Perkins is not enthusiastic about prolonging the life of late-stage dementia people beyond what’s necessary.

For me, from the distance of another city and visiting not nearly enough, my mother’s death was not an absolute tragedy. Had she been an observer of her own plight, she would have hated having to be dependent on others to dress her, to feed her. Ann Broatch had disappeared years before, her personality a faint trace of what it had been. To a powerful degree, we are our imaginations, our memories.

And when that goes, we go.