Health: Dementia

Published on Sunday 28 February 2010 10:32

A house in Glasgow, an ordinary house, any house. A daughter is visiting her mother, Maggie, for the weekend. It is Friday night and she has put her to bed, just like her mother once put her. Maggie is in her 80s now, a tiny little lump in the bed, small face engulfed in pillow, her body rigid and unable to move unaided because of her condition.

There is a guard against her bed to prevent her falling, or worse, trying to get out alone when she gets confused and forgets she can't. She has music playing softly to soothe her to sleep, like a child's lullaby light. The Three Priests are her favourite right now. Ave Maria, an echo of a faith she once followed but barely remembers. Beside the bed, an array of photographs. Maggie as a young woman: beautiful, shapely, almost saucy the way she looks at the camera. Invincible. There is a black and white picture of her late husband as a young man, like a portrait of a film star, above her bed. Little pictures of the different stages of their life together clutter the bedside table.

There are lots of houses in Britain like that house in Glasgow. Maggie suffers from dementia and there are 700,000 people like her in Britain. We tend to think of dementia as one disease but in fact, there are many different types and causes. Alzheimer's is the most well known and the most common, accounting for over 50 per cent of cases. But Maggie has Dementia with Lewy Bodies (DLB), a condition few have heard of despite it being the second most common form of degenerative brain disease in the world. It accounts for one in seven of dementia cases and is closely linked to Parkinson's disease. Both are caused by Lewy bodies, small deposits of protein in the brain which cause brain cell abnormalities. Lewy bodies interfere with the normal activity of brain cells and cause a gradual loss of control of bodily functions. Walking, talking and eating become difficult and people with DLB are often incontinent. They sleep a great deal during the day but have disturbed nights, and while they typically have fewer memory problems than Alzheimer's patients, sequencing thoughts and planning ahead can be difficult. One of the definitive characteristics of the disease is experiencing hallucinations. A person who doesn't exist sitting in the room. Non existent children playing in the garden beyond the window. A phantom dog who must be walked. But sufferers' abilities vary from day to day and even from one minute to the next and these fluctuating levels of consciousness and awareness sometimes cause carers to mistakenly assume they are "putting it on" or "being difficult". It is a bit like a faulty electrical connection; there can be a surge of normality one minute as the wires connect, then sudden failure the next as they lose contact.

"This fluctuation is probably a bit like living in a fog the whole time," explains DLB expert Ian McKeith, Professor of Old Age Psychiatry at Newcastle University and President of the Edinburgh-based Lewy Body Society, the only dedicated DLB charity in Europe. McKeith believes it important to recognise DLB as a distinct disease with its own care and management needs. "People with DLB have extra movement disability so you find they are more functionally disabled than people with Alzheimer's and their quality of life is substantially worse. It costs twice as much to care for people with DLB."

Dementia poses an enormous challenge to health and social care budgets for the future. People are living longer and dementia is essentially a disease of old age. By 2030, it is estimated, there will be twice as many sufferers, yet dementia has never been given the same priority as, for example, cancer or heart disease. Now, the government has initiated a dementia strategy for England and Wales with similar planning taking place in Scotland. For Ian McKeith, there is hope, particularly for DLB patients. "My view is that DLB will turn out to be one of the most treatable forms of dementia. In Alzheimer's you get demented because cells die, irreversibly die, and there's not much you can do about that. "In DLB, you probably get the dementia because cells aren't working properly which is why it fluctuates. There's more potential to improve and treat."

The UK has been a world leader in this field and McKeith is about to embark on a collaboration with German scientist, Walter Schulz-Schaeffer. "I think he is very close to discovering what is causing the cells not to work," says McKeith. "We are at last developing a really useful understanding that may help us to design a treatment. It's like any field of complicated science: things will take months and years but I think DLB has the potential for relatively quick success compared to other dementias." That's crucial as developing countries gradually begin to match our life expectancy. "It really falls to us to find solutions before the world's population becomes so enormous," he says.

In that house in Glasgow, Ave Maria is still playing softly. Maggie's daughter Ray sits in the semi-darkness watching her mother's face. They are real people, Maggie and Ray, though in a way it doesn't matter the details of their names or where that house is. They could be any of us. The rising statistics mean Maggie could be your mother or mine. Or actually, you or me. Maggie has had Parkinson's for many years, and her condition has developed into DLB, as it will for around 60 per cent of Parkinson's sufferers in the final stages.

Ray feels guilty. She doesn't live with Maggie like others in her family. Ray just visits; they do the heavy duty, day to day caring. Maggie's eyes open very suddenly, looking at Ray with confusion, an unspoken question. Her face has the mask like quality of Parkinson's. Her eyes used to be so warm and alive but sometimes Ray feels when her mum looks at her, it's as if she's looking at someone she knows she loved a long time ago. But that blank expression is misleading. "I'm just sitting while you fall asleep Mum," Ray explains. Maggie's eyes peep out from the sheet. "It's nice," she says.

OVER IN Edinburgh, Ashley Bayston is watching the final cuts of new video pods for the Lewy Body Society she founded in 2006 after her mother was diagnosed with the illness. One features the charity's patron, actress June Brown, who plays EastEnders' Dot Cotton and whose husband Robert Arnold died of the disease. Robert used to see figures behind the windows of the shed in the garden. When he watched the Bafta awards on TV he got confused, thinking he and June were there. "I think Judy saw us," he said as he watched Dame Judy Dench. "I never thought I would go like this," he once told her in a moment of lucidity. "I wish I were dead." It is imperative, says Brown, that more research is done.

Bayston and the Lewy Body Society fund research by supporting a PhD student in Newcastle who may also become involved in Ian McKeith's new collaboration. A vibrant, outgoing American, Bayston was brought up in America but has lived in Britain since the 70s. She trained to be a lawyer when she was in her 40s, becoming a senior counsel and magistrate in the south of England but her mother's DLB changed her life. "When she was diagnosed at the end of 2005, I wanted to help whatever charity there was and I found there wasn't one. That's why I started this." When her marriage collapsed and her mother died within the space of a few months, she moved to Edinburgh but was determined the charity would continue. "This whole charity is driven out of frustration because I couldn't do anything for her. This I will do."

Ashley's mother was Princess Millicent Medvied, a Russian title she inherited from her own mother who fled the revolution. When she was a child, her mother had a dress that Ashley always loved, a navy blue shirtwaister with white spots and white collar and cuffs. Her French lady dress, Ashley says. The logo of the LB society, a dotted ribbon in the shape of an L, is inspired by that dress. The spots represent Lewy bodies but the logo is a tribute to a woman Ashley describes as vivacious, intelligent and funny. "She was a fabulous artist. She could paint, she could sculpt, she was tremendously talented. Everyone who met her loved her. She was charming, beautiful and kind. She worried too much – but she was an exceptional person."

Millicent was 85 when she died. But in her late 50s, long before she became ill, she lost her sense of smell. The family put it down to a bad case of flu. When she developed mobility problems, the cause was thought to be orthopaedic because she had wrecked her knees when she was younger. But then the hallucinations began, the bizarre behaviour. "She saw and heard things we didn't understand." Experts often advise carers not to challenge hallucinations too forcefully, but simply to offer reassurance.

"The hallucinations were horrifying," says Ashley. "I don't know what she was seeing but whatever it was, it was nasty. You hear of people having nice hallucinations, children and bunny rabbits and things. There's a professor in Manchester who talks about a patient he once had who saw the Halle orchestra at the bottom of the bed. I wish that had happened to my mother. That would have been so nice. He said one time he went in and the woman said you'll have to wait until the end of the movement!" Ashley laughs. She has an irrepressible humour, is deeply funny when she switches to describing her disastrous attempts at internet dating, the weird presents that potential suitors brought: omega 6 fish oils or her favourite, a DVD about a boxer – in Thai. Her ability to embrace tragedy and humour as all part of life extends to her mother's condition. Once, she recalls, her mother had been sitting in what Ashley calls "a Lewy state" neither awake nor asleep. Then Arnold Schwarzenegger came on TV and suddenly her mother said dryly, "And he calls himself a politician!"

But when her mother was frightened, it was deeply distressing to watch. Sometimes she would become paranoid and think her husband, Herbert, and Ashley were plotting against her. Other times she would plead with them for help. "She would say please, please help me and we would say of course, what can we do? But she couldn't say. When I was at university I saw people take LSD. I never had any desire to take it myself because it was so scary to watch. That's what it reminded me of, someone having a bad trip."

Her parents were together for 65 years, marrying in secret in wartime because Herbert was an aviation cadet and not allowed to marry. They retired to Florida but would spend the summer months with Ashley in England. Herbert worked in insurance and was a successful, dynamic kind of man, used to making things work. He did everything doctors suggested but he couldn't make his wife better and that devastated him. She was a tiny woman, an American size two, who had never eaten much but as she became more ill she had to have a diet of pured food. Herbert looked after her at home, and Ashley flew over every two months, but eventually Millicent was bedridden and only a team of nurses enabled her to remain at home. "You need help at some point. Nobody can do it on their own. They were fantastic and looked after her like she was their own mother."

Illness is always hard. But there is a terrible poignancy about one that seems to rob the spirit of a person and leave you with their shell, an imposter who isn't quite who they seem to be. "You're losing someone while they're still there," explains Ashley. "You're grieving while they're still with you." Ashley felt her mother survived longer because of the love she was given. The weekend she died, Herbert called to say she had stopped eating. Ashley flew home. "She died 40 minutes after I got there. She waited, I'm sure she did. I said, 'I'm here'. She was pretty much comatose but I don't think it was coincidence she died 40 minutes later."

This year, on the anniversary of her mother's death, Ashley returned to America to be with her father and to deal with her mother's things. Her mother liked clothes, was always very stylish. Ashley found a coat Millicent had bought from a designer in Rome. She is having a dress made to go with it and will wear it to her daughter's wedding. She was fine sorting out the dresses, the trousers, the blouses. It was when she got to drawer after drawer of nightgowns that she finally broke. It was all her mother wore for the last few years of her life. But now Ashley prefers to take her mother out of that period of illness in her mind and place her where she belongs. "A young, vibrant, gorgeous lady who, up until her 50s, had her hair down beyond her waist which she had in a long plait, piled up. That's how I think of her."

SATURDAY NIGHT now in the weekend visit to that house in Glasgow. A family dinner. Brothers, sisters, bottles and candles. Maggie sits in her wheelchair. She hasn't said anything for hours. Ray wonders how much she takes in of the conversation round her. Someone get drinks, says a voice from the kitchen. Who wants this? Who wants that? A chaotic kind of conversation. Maggie used to be a teacher, was forever saying, "Hands up…" Her voice is tiny now, has no power, but she cuts through the disorganisation. "Hands up for gin and tonic," she says, and the first hand in the air is her own.

Edinburgh nurse Christine Thompson remembers taking her father to the beach once when he wanted to take the dog. But there was no dog. She ended up putting the imaginary animal in the car to please him. "When I got to the beach I went to get the dog out of the back and he was saying, "Which dog?" I was just thinking, oh my word…!"

Originally from Newcastle, Christine is warm, friendly and upbeat, and as a nurse is used to talking about difficult subjects. But eventually her composure cracks because this is her dad and recently his condition has been deteriorating. That's hard for any family to watch. Modern families are often more scattered than they once were but Christine visits every couple of weeks and her brother lives close to her parents. The burden of care, though, is still with her mum Pat, who is now in her 70s. Given the mobility problems, it has become impossible to look after Brian at home and for safety reasons, he has had to go into residential care. At the beginning of his illness he hated even going to day centres. Now he seems reasonably content to be in care. Pat visits every day. "It's awful for her," says Christine. "She misses him dreadfully. It's my dad and I'm devastated but this is her husband, her life partner."

Brian watched his brother, who was 13 years older, die from DLB. At 59, Brian himself was diagnosed with Parkinson's disease, an obvious genetic link, you would think. The brothers' mother died in her 70s with no obvious signs of dementia but their father died young, in his 50s, so no one knows if he would have developed either disease.

Some people – like Ashley Bayston's mother – can be misdiagnosed with Parkinson's when they actually have DLB, but some people do develop both. "It's the same biological process," explains Ian McKeith. "If you get this disease in your 50s or 60s, the odds are it will present itself as Parkinson's disease. If you live with your Parkinson's for an average of ten years, which people do now because we treat it well, then the disease spreads to more of your brain and you get dementia on top. On the other hand, if you get this disease when you are in your 70s or 80s, for some reason it starts in a different part of your brain and therefore you present with dementia rather than Parkinson's."

Treatment for DLB is usually either Parkinson's drugs or Alzheimer's drugs. (Neuroleptics, controversial sedatives sometimes given in care homes to subdue patients, are extremely dangerous for DLB patients. "They have a mortality rate two or three times the norm and really shouldn't be given," says McKeith.) Parkinson's drugs can improve mobility but tend to exacerbate hallucinations. But Alzheimer's drugs can be very effective and the speed with which the condition progresses varies enormously in individuals. Peter Ashley, for example, a sufferer used in the Lewy Body Society's new videos, has been diagnosed for nine years but can still walk and talk lucidly. A keen musician, he has, though, lost the ability to play the piano. He reads the music in his head still but his hands no longer know where to go on the keys.

Brian Thompson was a spirited man in his earlier years, says his daughter, humorous and sarcastic and great fun. He was an electrical engineer and when he became ill would still take things apart. He was given Meccano to keep him from more dangerous activities. When he was diagnosed with Parkinson's, he didn't want support groups. He went to the library for books and quietly came to terms with it in his own way. He was quite artistic and he and Pat used to go to art classes. He stopped when he asked the family which paintings they thought their mum had done and they knew immediately.

His family and friends have closed round him, visiting him constantly. Christine doesn't feel she has lost him. "I suppose physically he's a different person but when you are able to get him in a wheelchair and spend an afternoon with him, he's very much with you. But sometimes you can sit with him for a few hours and he may not really wake or be very responsive." He might not talk much but he still recognises them. His eyes light up when he sees his own. He does, though, get confused. "My grandma died when I was six and a few years back he said, 'When's my mum coming in?' It was as if he'd really gone back in his earlier life. He doesn't do that now but we always make sure there is a picture of his mum around. If it does mean something to dad, at least it's there."

When Christine is there, or her brother, her dad can come home for the day. It's lovely having him back where he belongs. "It's like… he's home. We've got him." Her voice breaks and she can only manage one more word. "Nice."

SUNDAY AFTERNOON in the garden of the house in Glasgow. Baking hot, bees droning. Maggie likes the sun still, though she can't smell the flowers. Sometimes, it seems to Ray that her mother has become a shadow person, a black and white outline instead of a full colour drawing. She used to be so lively and funny, incorrigible. Always illogical, always right even when she was wrong. Always.

The weekend visit is nearly over. "I have to go home today," Ray tells her mother. "Back up north." Maggie looks at her with vague regret. "Are there lions up north?" she asks. Ray wants to laugh and cry simultaneously. "No," she tells her mother, holding her eyes. "Only deer. No lions." Maggie looks back at her. "Yes there are," she says. There's just enough defiance to make Ray smile. Maggie's still there. DLB patients always are. They're just a little harder to find than they once were. r

For more information, visit www.lewybody.org

This article was originally published in Scotland on Sunday on 28 February 2010