Fronto-temporal dementia is hereditary but normally strikes between the ages of 45 and 65
AS SHE is talking, Sophie Gilbert spots her older sister Becky Barletta returning from a walk in the Suffolk countryside with her carer.
Becky, 33, is wearing her blonde hair loose with sunglasses and a cap on.
When she enters her kitchen Sophie, 31, asks breezily: “Did you have a nice walk?” Becky looks towards her but does not respond and her face remains expressionless.
“To anyone meeting her for the first time she would look like a normal person and she can definitely hear me,” Sophie tells me quietly.
“But the real Becks is no longer there. The sparkle has completely gone. We all love her but she doesn’t look like our Becky any more.
“She was absolutely stunning and vibrant and took great care of her appearance but we are now living with the shell of our lovely girl.”
Two years ago this week Sophie was diagnosed with a rare form of dementia which runs in her family.
Fronto-temporal dementia is hereditary but normally strikes between the ages of 45 and 65.
Becky, a professional ski instructor who used to work at Richard Branson’s chalet in the upmarket Swiss resort of Verbier, was just 31 when she received her diagnosis – just 10 months after getting married to her devoted husband Luca, an Italian and a fellow ski instructor.
She is believed to be one of the youngest patients ever struck down by the disease. “Every night I hope she is happy in herself but you just can’t tell,” Sophie explains.
“There is no anguish, no pain, but neither is there any happiness or joy. There is nothing.”
During the past 24 months, Becky’s deterioration has been merciless as the parts of her brain concerned with emotion and behaviour have been slowly destroyed.
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My goal in speaking out to raise money is that my children don’t have to worry about a hereditary disease that ends lives without dignity
“She might copy a word or scream out occasionally but she doesn’t talk any more. It’s not that she can’t speak but that she doesn’t and that has happened in the past six months.
“Eventually you lose everything. The only unknowns are in what order and at what speed,” explains Sophie, who will officially open the Ipswich Memory Walk this Sunday, raising money for the Alzheimer’s Society.
Last year the family helped raise more than £10,000 for the charity from their own Memory Walk.
GROWING up in Suffolk, Sophie and Becky were always close but it was the elder sister who seemed to outshine the younger.
“I was in awe of her growing up,” admits Sophie.
“When my friends would come to play they would play with her. She was always thin while I was a bit dumpy and she was always the beautiful one. She was also the one who put 100 per cent of effort into things, and she was more sporty.
“She was the last person on earth you would have imagined this would happen to. She was a photographer, she loved to travel, she loved music and bands, and she was a ski instructor. She had hundreds of friends around the world. She was hugely popular.”
But it was shortly before her wedding in October 2015 that Sophie and her parents, Caroline, 62, and Allan, 69, began to notice subtle changes in Becky’s behaviour.
Her reactions became “slightly one-dimensional” and she appeared to be losing her empathy.
When Caroline and Sophie arrived to help with her wedding flowers, Becky wasn’t there but neither was she concerned by her oversight.
She also stopped asking after her baby nephew, Sophie’s son Alfie, now three.
“We hoped it was a bit of a breakdown caused by the stress of the wedding or perhaps an eating disorder. But I can see now we were making excuses.”
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What the family were trying to deny was any suggestion that their “charismatic blonde bombshell” might be succumbing to a disease that had already robbed Caroline of her brother James at the age of 59 and her niece – Sophie and Becky’s cousin – in her 40s.
The first clear signpost came when Becky returned to Verbier after her marriage and was suspended.
“She was always wonderful with the children and returning clients each year asked for Becky but she stopped watching the children as closely as she should. She was also sharing more personal details with clients than she should have.”
Allan and Caroline flew to Verbier.
“Mum took one look at her and arranged for her to be seen by the same consultant who had treated her brother.”
When the shattering diagnosis came Becky was protected from its full force by the illness itself.
“She just said, ‘Oh, I don’t want that.’
And this was the only time she showed any insight at all.
Mum started crying and Becky asked why.
She could recognise obvious emotions but she could not relate to them.
“After she was diagnosed we stopped fighting it. If she would spit on the floor or eat from someone else’s plate we stopped trying to reason with her because we knew it was outside her control.”
Within months of her diagnosis Becky lost control of her behaviour.
She would blow endless raspberries or ask strangers in the supermarket if they could make funny noises.
She also repeated the same stories.
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Sophie says: “One story was about when she was my bridesmaid. She would say, ‘I had to drink lots and do the limbo and my dress came down and showed my boobies.’ It was true but it was always the same story repeated word for word.
“At the time it was driving us crazy but now she has stopped speaking I would love her to tell her little stories again. You want to hold on to every moment you have of her.”
TODAY the family, whose bakery business Dorringtons will celebrate its centenary next year, remain committed to caring for Becky.
She lives with her parents in the farmhouse next door to Sophie’s converted barn near Bury St Edmunds.
Becky’s husband Luca visits often from Europe – where in winter he works as a ski instructor and in summer runs a hostel.
“He is here as much as he can to see Becks. It is not a conventional marriage but he loves her and in that respect it’s ‘in sickness and in health’,” says Sophie.
“I’ve lost a sister but they were supposed to begin their life together. It’s horrible that all that has been taken away from them.”
The gene carried by the family has not been identified and Sophie knows that she, Alfie, or his one-year-old sister Millie are at risk.
Typically each of the children or siblings of someone with a genetic mutation that can cause the disease has a 50 per cent chance of carrying it themselves although that does not mean they will develop symptoms.
“My goal in speaking out to raise money is that my children don’t have to worry about a hereditary disease that ends lives without dignity.
“What also really upsets me is that they won’t remember their Aunty Becky when she was well. I had always imagined them growing up with their cousins and having holidays together on the Suffolk coast but it’s awful that it’s never going to happen.”
To help them understand who their aunt really was Sophie has filled her house with framed photographs of Becky.
And she tells the children stories about her.
“At night, after I have read to Alfie, he asks me to make a story up. So I will tell him how I want it to be with him and us and Aunty Becky and Uncle Luca all going on holiday together. Or I will tell him the story of Aunty Becky and the Magic Skis. “Later, lying in bed, I try my best to remain happy about all that we have. And I take comfort from Luca’s words. He always says that it is better to live 30 years as a lion than 60 years as a mouse.”
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● Alzheimer’s Society is calling on Daily Express readers to unite against dementia and help fund vital research for a cure by signing up for a local Memory Walk at memorywalk.org.uk.
Forty flagship walks are taking place in September and October.
“Dementia is now the UK’s biggest killer with someone developing it every three minutes,” says Alzheimer’s Society chief Jeremy Hughes.
“Every pound raised through Memory Walk will help provide vital information and support, improve care, fund research and create lasting change for people affected by the disease.”