Oct 242011
 

This article was published when Hayley was eight, before she started the drug trails in the US. She will be 14 in December 2011 – you’ve come a long way Hayley!

Hayley Okines suffers from a rare disease called Hutchison Guildford Progeria which ages the body more than eight times faster than normal. When she was first diagnosed with Progeria, there was no hope, no cure. However, a treatment for Progeria is now being developed but, given that her body is equivalent to an old woman, can it offer hope for Hayley?

This year began with a tragic reminder of the urgent need for a Progeria treatment. Hayley and her family attended the funeral of her best friend who died of a heart attack. She too had Progeria and was only two years older than Hayley. Progeria accelerates the aging process, the average life-span is just thirteen years. At eleven, Hayley’s friend was into her nineties in progeria years when she died. Hayley is eight, equivalent to her seventies. Tragically, the death of her friend was just two months before a possible breakthrough in the treatment of the disease.

Hayley Okines

Hayley’s parents heard about the possible treatment at the annual progeria reunion in the United States. In Autumn 2006, the family are told that they can expect Hayley to be on the treatment by the end of the year. If true, there will be plenty to celebrate on Hayley’s ninth birthday in December.

Back in 2002, Hayley and her father were in Florida for the annual progeria reunion. Thirty two children, three-quarters of the world’s known progeria population, were there. In the middle of the reunion, one of the guests suddenly died. It was a sad reminder of the reality of the disease at what was supposed to be a happy event.

With children from all over the world in one place, a reunion is an invaluable opportunity to research the disease. In 2001, the Progeria Research Foundation began to take samples from children in order to build up a cell bank. This work led to the discovery of the treatment currently being developed.

The treatment offers hope that Hayley’s life could be longer than those of her friends but, until it is ready, medical care is in the hands of her local hospital in Hastings where her paediatrician has monitored the progression of the disease for the last seven years.

Mark Okines

On a recent visit to the USA for tests, the first sign of possible cardio vascular problems were detected. The blood flow in an artery in Hayley’s neck was found to be restricted, so she was given an MRI scan. Her mother explains “She was one of the first progeria children to complete an MRI scan and get proper results which confirmed that the blood wasn’t flowing and could cause a stroke. So, she has been put on synthetic statin which stops the arteries furring up”. Hayley has been taking the statins for the past two years. There has been no noticeable deterioration since.

Progeria is caused by a tiny mutation in a child’s DNA; a defective protein called Lamin A. This protein holds the nucleus of a cell together, but in progeria children that nucleus is unstable. This instability leads to the process of premature aging.

Having identified the defective protein, American researchers, testing the progeria cells they had collected, made an exciting discovery. A drug developed for cancer treatment restored the nucleus of the cells to a near-normal appearance. The drug is called a farnesyltransferase inhibitor or FTI.

The drug has not been trialled on children and Hayley is reluctant to be one of the first human guinea pigs but, with so few children available for trials and with their lives so short and in the interest of fairness for all, waiting for others to go first is not an option.

The Okines are playing host to another progeria family, Wim and Godelieve Vandeweert from Belgium, who first heard about the condition from a television documentary. The chance of having a child with progeria are one in eight million. The Vandeweerts have two progeria children; Michiel and Amber. Doctors believe that the Vanderweert’s situation is a remarkable chance occurrence. It is thought that both parents could be carrying defective cells, although these cells have not been identified.

The Okines finally get word that the new treatment will start in June. Hayley will undergo extensive testing in Boston before the drug is used, The drug trials are shrouded in secrecy. All the participants have signed a confidentiality agreement.

Old Before My Time is published in paperback on 24th November by Accent Press, priced £9.99.

Oct 232011
 

The dream of writing her own book, Old Before My Time: Hayley Okines’ life with progeria and seeing it printed has come true for Hayley Okines, the 13-year-old schoolgirl born with the rare genetic condition progeria.

hayley-421

Hayley, who has appeared on the Channel 5 programme Extraordinary People and numerous documentaries, visited CPI Mackays  in Chatham to see her autobiography on press. Accompanied by her mum, Kerry, Hayley helped on the binding line and was presented with her own personalised hi-vis vest by the team.

The visit was filmed by a crew from Rabbit Productions as part of a documentary about Hayley’s extraordinary life, to be screened on Channel 5.

Hayley’s body ages eight times faster than the average person. In medical terms, her body is that of a 100-year-old woman. Despite her condition, Hayley lives life to the full, travelling the world meeting her pop heroes Kylie, Girls Aloud and Justin Bieber. In her book, Old Before My Time, she reflects on her adventures as well as her hopes for the future and her pioneering drug trials in America.

Thanking everyone at CPI Mackays, Hayley said: ‘I’ve had a fantastic day – it was really strange seeing my face going round the factory on thousands of my books’.


Oct 162011
 

Hayley is 13 and loves shopping, Justin Bieber, and Twitter… and she has the body of a 105-year-old woman
By CLAIRE BATES

Like many young teenagers, Hayley Okines is obsessed with pop star Justin Bieber, messaging her friends on Twitter and going shopping. But there is one big difference. Hayley has progeria, which means her body ages eight times quicker than normal.
She suffers from arthritis, hip problems and baldness and dreams of having long lustrous locks that she can ‘flick in the wind.’
Her life story is the subject of a ongoing documentary series for Five. When she last featured in 2010 she was starting secondary school and taking part in a pioneering medical trial in America.

Radio star: Hayley with her best friend at school before a hip problem forces her to learn from home

Check-up: Hayley is no stranger to hospitals. Her heart is currently strong thanks to the multiple medicines that she takes
A year on and she is getting to grips with being a teenager – not an easy task at the best of times.
‘It’s annoying having an old woman’s body’, she admits early on.
The episode, which she is narrating herself for the first time, shows her planning her own autobiography and writing a speech to give to experts on her condition.
‘How do you spell progeria?’ she wonders aloud, revealing just how young she is, despite all she has gone through.
‘I really should know how to spell that,’ she adds with a sheepish grin to the camera.

More…
I’m only 26! Doctors baffled by condition that sees wife age 50 years in a matter of days
Her mother Kerry, who lives with Hayley and her two other healthy children in Bexhill, told Mail Online: ‘Hayley is a typical teenager, she’s more stroppy now and has a messy bedroom. She always likes to have the last word and thinks she knows everything like most teens!’
She admitted it was hard letting go of the reins as Hayley grows up.
‘It is difficult,’ she said.
‘She obviously wants to go out and I do let her go down to the local shops and go on sleepovers.
‘She also goes to the local shopping centre with her friends but I like to be somewhere in the centre at the same time and I make sure she has her mobile on her in case she needs me.’

Hayley wearing a headscarf: The teenager now regularly wears a brunette wig

Old before my time: Hayley and her mother Kerry watch her book hit the presses
Kerry has good reason to be worried. In April this year, Hayley was told by a doctor that her hip bones were in danger of dislocating and she now has to wear an uncomfortable brace nearly 24-hours a day.
‘I don’t like the brace as it’s a bit heavy and stops me moving around as much,’ she told Mail Online.
Her mother Kerry added: ‘She hasn’t been able to go back to school since April. We were hoping she would go back but she’s had more problems with her hips.
‘They have partially slipped out even despite the brace and the doctors are now looking at the possibility of surgery and speaking to experts in America.
‘Maybe she can go back to school next year…’ she trailed off hopefully.
Despite the latest setback Hayley continues to stay positive.
‘She does online schooling which she loves. Maths, English, Science and Art. She is also doing a health and beauty course and they are designing their own salon,’ Kerry said.
The internet has opened up a whole new world for Hayley, who loves the social-networking site Twitter. It was thanks to her Twitter followers that she achieved a lifelong dream of meeting the pop singer Justin Bieber.

Justin meets Hayley: The kind-hearted pop star gave Hayley front row tickets to see his show
‘Some girls started a campaign on Twitter to get Hayley to meet Justin,’ Kerry said.
‘Justin Bieber’s people heard about it and contacted us. We arranged to meet him at a London hotel last December as a surprise for Hayley’s birthday.
‘When she saw him she just screamed and attracting attention from everyone in the hotel!’
Hayley said: ‘I remember I came down the stairs and he was just standing there. I was so surprised I just screamed and ran up to him and put my arms around him.
‘He asked me what my favourite song was but I said I didn’t have one. Then he asked what I wanted for Christmas and I said him!’
Hayley’s younger brother Louis and sister Ruby consider her to be a ‘celebrity’ because she’s been on Blue Peter and met famous people.
‘They don’t realise the bad stuff that comes with it,’ Hayley says.
She is increasingly aware that she has a terminal illness and is shown laying flowers for a family friend with progeria who died at 14.
‘We don’t sit down and talk about it but she’s had friends who have passed away and she’s aware,’ Kerry said.
‘We’d rather get on with living.’
Hayley is being lined up to take part in another U.S drug trial for Rapamicin, otherwise known as the ‘forever young drug.’
‘We were due to start trials in November, now that’s been put back to March 2012,’ Kerry said.
‘I hope the new drug works out alright and Hayley stays healthy.’

Hayley is shown having trouble shopping for clothes. She says she is an expert at finding things in the children’s section
But while they are obviously frustrated about the speed of progeria research, they continue to focus on the future.
‘We organised a reunion last year in Ashford for children with progeria and will do the same this year,’ Kerry said.
‘It takes a lot of organising and fundraising but it’s such a fantastic event. We had 16 children and their families and this year we will have have 20.’
Hayley meanwhile is excited about having just got a dog.
‘She’s a shitzu called Angel and she’s so silly – she keeps running into walls,’ Hayley said.
‘And we’re getting a chihuahua for my 14th birthday.’
Hayley has also been fitted with a real-hair wig which she now wears most days.
‘She loves it and we’ve spent a fortune getting hair straighteners and curling tongs for her!’ Kerry said.
Meanwhile the ever enterprising Hayley has decided her next project will be launching her own perfume.
‘I saw that Justin Bieber had a perfume out and I thought ‘that’s something I’d like to do.’
‘I think I’d like it to smell like vanilla, although I haven’t decided on a name.’
As her mother says, Hayley is determined to just keep on living.