Old Before My Time (5 page)

Chapter 4
Kerry
Learning to Live with Progeria

T
HE TURNING POINT IN
my acceptance of Hayley's progeria came from conversations with other families who had already been through diagnosis and were not only coping, but living full and happy lives.

For the first few months after my breakdown I had been in complete denial, foolishly thinking that if I didn't acknowledge the progeria it would go away. Meanwhile in the background Mum and Mark were finding out more about the disease, driven on by Mark's mantra ‘knowledge is power'. The world was still a big place in those days and with the few progeria families spread around the world, information for newly diagnosed children and their parents was like searching for that elusive needle in a haystack. Back then Google was still only a small private American company set up by two Stanford University students rather than a globally-recognised verb for finding information online. When we Asked Jeeves about progeria only 2,200 web pages appeared and many of those lacked hard information and facts. Whereas now you can sift through around two million pages on the condition and Hayley has more than 94,000 online references in addition to her dedicated progeria website. Even the UK's top doctors knew very little about the condition back then. Professor Winter at Great Ormond Street Hospital had only ever known of one other case in his career, so we were very much in the dark and finding help was mainly through word of mouth.

My mother was determined to track someone down who could help lift my depression. She spent hours on the phone talking to families and friends.

‘I've just been speaking to a woman called Doreen in Northampton whose adopted son, Danny, had progeria,' she said after putting the phone down one day.

‘Had,' I said, picking up on the past tense.

‘Yes. He had classic progeria and he was 22 when he died in 1996. He's the longest survivor we know about, so there is some hope out there. Doreen helps families with children with progeria like you and she's expecting a call from you.'

My initial reaction was ‘I can't' and I threw the piece of paper with Doreen's phone number in the bin. I didn't think anyone could help Hayley and couldn't see the point of wasting my breath or the cost of a telephone call.

On my doctor's advice Mark and I started seeing a counsellor at a local hospital to help us talk through our worries.

‘What are your worst fears, Kerry?' my counsellor asked.

‘No mother should bury her own daughter,' I whispered. Although physically and emotionally draining, the sessions allowed me to share my concerns. Voicing my inner demons gave me the strength to accept Hayley's progeria and move forward for everyone. When I finally felt strong enough to pick up the phone to call Doreen it was like a weight had been lifted off my shoulders.

Mum had been right all along. Just talking to someone who had been at the point Mark and I were at helped. She understood my feelings and could relate to us. Speaking to her, I felt her positivity rub off on me. Even though she had lost her son, she talked about the happy times they had together and the joy of having a special child with progeria. She also told me about something called the Sunshine Progeria Reunion where all the known progeria children in the world and their families would meet in the United States for an annual get-together. She had taken her son for many years during his life and had continued to attend as a volunteer after his death. She also gave me a telephone number for a couple of doctors in America who had set up a new organisation called the Progeria Research Foundation to help families like Mark and me with information about the disease. Doreen's parting words were, ‘Don't waste your time worrying about what might be. Make every minute count for Hayley.'

As part of our fact-finding mission we spoke to scientists to get a clearer understanding of what had gone wrong in Hayley's genes and attempted to find out whether it was genetic or just bad luck. We spoke to Dr Richard Faragher, a Research Scientist at the University of Brighton. He told us that progeria was a premature ageing disease, which mimics or caricatures many aspects of the normal ageing process.

‘What you are seeing in all probability is the result of a gene that has just gone bad by accident in one or other of the parents. Any patient that has progeria is not very likely to live to produce children so the gene is unlikely to be passed down.'

I also called Dr Leslie Gordon, a very helpful mum in America. Leslie and her husband Scott Berns, both paediatricians, had a three-year-old son Sam who was diagnosed with Hutchinson-Gilford Progeria when he was 22 months old. They were a year ahead of us in their diagnosis but Leslie told me how difficult it had been even for a pair of doctors to find information. They had just started an organisation called the Progeria Research Foundation to help other families.

‘We found there wasn't much available for families like us and there was no way to definitively test for the disease, no funding for progeria research, and no organisation advocating for children with progeria, so we are going to change that with our foundation. We are on a mission to find the cause in order to work on a treatment and cure,' she promised.

‘We use the words ‘premature ageing' in quotes because even though there are some aspects of ageing progeria children do have like wrinkled skin, hair loss and heart problems, their brains remain sharp and they are bright, sweet little children,' Dr Gordon said. Like us, the Gordons were on a sharp learning curve and determined to spread their knowledge with others.

Meanwhile life for Hayley went on. We made three-monthly visits to our nearest hospital the – Conquest Hospital, Hastings – for check-ups with consultant paediatrician Dr Lorna Bray. Although there was no medicine Hayley could take to cure the progeria, there was a cocktail of preventative pills and remedies to help her quality of life. One of the main concerns was that children with progeria usually died of heart attacks or strokes, just like old people. As a result our bathroom cabinet started to resemble that of a typical octogenarian. Her daily routine involved a concoction of pills, which we would joke made her rattle. Hayley has always been good at taking tablets, which was fortunate as she needed four doses of vitamin E with her meals to help her heart, cod liver oil for her joints and fluoride for her teeth. We learnt that most progeria children had problems with their teeth due to having underdeveloped facial bones and lower jaws, which meant that their teeth were slow to break through their gums and when they eventually did they were small and irregularly formed. Tooth decay was also a common problem so fluoride would help by strengthening her tooth enamel and making it more resistant to decay. We gave Hayley half of a 1.1mg tablet every day.

Narrowed coronary arteries was another common problem with progeria children and clinical trials had proved that a small daily dose of aspirin, enough to prevent dangerous blood clotting, reduced the risk of strokes and heart attacks. We started out by giving Hayley half of a 75mg tablet every day, but later reduced it to a quarter as we found her skin was bruising easily, one of the common side-effects. Hayley's eating problems which had started in her first months of life continued as she grew older, again we discovered that this was not uncommon in children with progeria, who had tiny stomachs. Throughout her life Hayley has never really enjoyed eating as she complains she gets full quickly. During her early years before bedtime we would give her a bottle of build-up milk called Nutrini which provided all of the nutrients essential for well-being and health. We later discovered something called Pro-Cal, which we would sprinkle into her morning cup of tea to add extra energy, protein and minerals to her diet.

Hayley's favourite item on Dr Bray's prescription was hydrotherapy. She didn't see it as medical – it was a chance to put on her pink water wings and matching pink costume and go swimming. From an early age Mark took her to our local hospital for 30-minute sessions in the hydrotherapy pool. It helped Hayley relax and relieved any pain in her elderly joints. The best thing about hydrotherapy was that she didn't ever see it as another ‘treatment', it was just a fun day out with her daddy splashing around in water, yet she would be getting much-needed exercise to stop her arthritic joints from deteriorating. Hayley was a real water baby, she loved the bath and would have been happy to have daily hydrotherapy if she could.

At home Hayley behaved perfectly normally, she had the innocence of a toddler and was totally unaware that she was different to the majority of kids. Hayley loved skipping along the seafront and throwing pebbles into the sea. It was odd to think that although she was young, Hayley was already facing the same health issues as many of the elderly folk who nodded politely as we passed them on the prom.

Whenever we took her out in her pushchair, Hayley's big blue smiling eyes and frail features always attracted the attention of strangers who would just stare awkwardly. To counteract this I taught her a trick to deflect the unwanted attention of anyone who stared at her for too long. I wouldn't have minded if these people had spoken to us, asked me why she looked different, I could have educated them with the little knowledge I had about progeria. But to stare was just plain rude.

‘Can I do it now, Mum?' Hayley would whisper to me.

‘Go on then,' I would reply and she would stick out her tongue causing the ‘starer' to quickly look away and move on. It was naughty, I know, but it gave both Hayley and me a wicked sense of satisfaction to watch their embarrassment.

Two months after her diagnosis, Hayley turned two. She had grown very little and was still wearing clothes made to fit a three- to six-month-old baby but Mark and I wanted to make it special and showered her in presents. Like every other toddler at the time she was obsessed by the Teletubbies. It was a daily ritual for her to sit glued to the television as the brightly coloured, podgy characters with TVs in their tummies ran around in Tellytubbyland and said ‘Eh-oh'. For her birthday we splashed out and bought her the set of Teletubby toys. There was Tinky Winky the purple one, Dipsy was green, Po was red. But her favourite was Laa-Laa, the yellow one. She carried it around everywhere with her. They were only 10 inches high but at that point the toys were almost as big as Hayley and she would wrestle with them. To transport them around we bought her a pram, shaped like a lady bug, which she could just about manage to hold on to.

From my first conversation with Doreen there had been one thing that stuck in my mind: ‘make every minute count'. There was no doubt in my or Mark's minds that Hayley was our special girl, but I wanted to make her life even more extraordinary. ‘I want to take Hayley to Disney World,' I said to Mum one morning in January as we were making breakfast.

‘Where are you going to find the money to do that?' she challenged. She was right. I had given up my job at the prep school to care for Hayley after she was born, Mark was earning barely more than minimum wage on his delivery van rounds. We had no savings, our old Ford Sierra needed hundreds of pounds to be spent on fixing the rust to get it through its MOT. We had not been able to afford a family holiday the summer after Hayley was born and here I was thinking of jetting off to Florida on a trip that would cost at least £5,000. That kind of money could buy us a nearly new car, one that would fly through its MOT. What was I thinking?

‘We could set up a fund, do events, raise money. We could tell the local paper and they can run a story on Hayley. I've read stories about other kids who are sent on dream holidays, why not Hayley?' I explained to Mum, who looked at me as if I had lost leave of any tiny bit of sense I had.

‘But what if you can't raise the money? What will you do then? You can't disappoint Hayley, and the girls.'

‘Then I'll get a bank loan.' For the first time in months I felt positive. Raising money for Hayley gave me a focus. I was on a mission.

I waited until office hours and rang the news desk at the
Bexhill-on-Sea Observer
. I told them about Hayley and her one in eight million disease and how her family and friends were raising money to send her to Disney World. They were astounded. That afternoon they sent a reporter and photographer to our house in Bexhill and interviewed Mark and me and took pictures of Hayley.

On Friday January 7 2000, Hayley's story made front page news. ‘Why friends want to give little Hayley a Disney treat', the headline read. Below it was a photo of Mark and me holding Hayley between us. The story related how Hayley was one of only three children in the UK with the rare genetic condition progeria which made her age eight times faster than normal children. It explained how her life expectancy was 13 and how Mark and I had set up a trust fund and friends and family were working to raise money to take her to Disney World. The photograph said more than any words could. Hayley looked her cutest, grinning for the camera.

Very soon after Hayley's diagnosis, Mark and I agreed that we wouldn't shield her from the public gaze. Until that day when the doctor handed us the slip of paper we had never heard of progeria and neither had any of our family and friends. With so few cases in the world, it was safe to say there was a lot of public ignorance about it. Mark and I made a conscious decision to tell the world – and that meant appearing in the media.

When the local paper hit the newsstands it opened the floodgates for what has become a lifelong media circus. Hayley's progeria story was picked up by one of the UK's leading broadsheet newspapers: the
Daily Telegraph
. As soon as the story ran in the
Telegraph
the frenzy started; my phone was red hot. I had calls from news agencies, women's magazines, television companies, radio stations, all wanting exclusive access to Hayley's amazing story. I called Mark at work panicking about what I should do and, even in the few minutes I was on the phone, six journalists had left messages for me to call them back. While I was on the phone being interviewed by one magazine, there was a knock on the door. I opened it to be handed a telegram. It was from Richard and Judy, who at the time were the brightest stars in daytime TV. Their mid-morning programme was required watching for mums like me. They wanted to whisk us up to London to appear on their show that week. We said yes and within 48 hours we were sitting being interviewed live by the most famous presenters on British TV. It was more than we had hoped for and we took the opportunity to spread the word about progeria. As we sat on the sofa with Richard and Judy one viewer called in to donate a computer having heard us talk about how difficult it was to find out information about progeria. It was incredible, in those days the average home computer cost more than £1,000 and there was a complete stranger offering to give us one.