Old Before My Time (12 page)

Chapter 18
Kerry
The Second Worst Day of my Life

H
AYLEY'S CLOSEST FRIEND
–
THE
one who understood her the most was Maddie. Maddie was three years older than Hayley but they had so much in common apart as well as their progeria. We first met Maddie and her family at one of the early Progeria Reunions in America and when we both returned to our homes in the UK, we stayed in touch and met up often so the girls could have sleepovers.

They would play together for hours, painting one another's finger nails and putting make-up on each other's faces.

One Sunday afternoon after Maddie had returned home from one of their sleepovers I had a frantic phone call telling me Maddie had been rushed to hospital. I arrived at the emergency unit to be told the news. Maddie had suffered a massive heart attack and had passed away. She was just eleven years old.

My heart was in pieces for Maddie's mum. There were no words I could say that would change anything. I tried to put myself in her place and imagined how I would be feeling. It was too horrible to think about. And worst of all I had to go home and break the news to Hayley. How could I tell her that her best friend, the one person in the world she was closest to and had most in common with, was no longer with us? When I had left our house earlier that afternoon Hayley had no idea Maddie had been taken ill.

As I turned the key in the front door lock, I could feel my heart throbbing in my mouth. I took a deep breath, trying to compose myself for the task ahead, and walked into the living room where Hayley was watching her favourite cartoon Spongebob Squarepants. Fighting back the tears, I switched off the TV and sat down beside Hayley and that's when the floodgates opened.

‘What's the matter, Mummy?' Hayley asked as she climbed onto my lap. Sensing that something bad had happened she started to rub my cheek, just like she had done the day five years ago when the doctor delivered the news about her progeria.

‘Mummy has been with Maddie because she wasn't feeling very well,' I said, choking back the tears.

‘We had to get an ambulance for her. Mummy went over to hospital with her. But she was too tired and she went to sleep and she's gone to Heaven.'

I didn't need to say any more. Hearing the word Heaven, Hayley burst into uncontrollable sobs. I wrapped my arms around her, drawing her tight to my chest. ‘It hurts in my tummy, Mummy. My heart really hurts,' she cried.

Apart from the day when we had confirmation of Hayley's progeria, Maddie's death was the second most upsetting time for our family. As the parent of a progeria child death is something that is always in the back of your mind. When we looked back over the old photographs taken at the Progeria Reunions Mark and I would add up the number of children no longer with us and count our blessings that we still had Hayley. But Maddie's passing was more devastating because the girls had been so close and spent so much time together.

The hardest decision was whether we should let Hayley go to the funeral. She was only eight and while we wanted to do what was best for her, we didn't want her to start worrying about death. But Hayley wanted to go. Not knowing how to deal with such a delicate issue, we deferred to Hayley's care worker Jane, who suggested it would be therapeutic for Hayley to attend.

‘Can I wear my purple dress? Because pink and purple were Maddie's favourite colours.' Hayley asked.

Hayley sat down at her computer and wrote a letter to her friend that she wanted to read to her at the funeral. On the day of the funeral Hayley coped better than we had expected. She stood up in front of a crowded church, where everyone wore pink and purple in honour of Maddie. When she read her letter to Maddie there wasn't a dry eye in the church. In the cemetery Mark and I stood beside the open grave holding Hayley's hand, she bent down and placed a card and her favourite photograph of the two of them on top of the coffin, as it was lowered into the ground.

In the weeks that followed Maddie's funeral Hayley would regularly talk about Maddie. She said she had seen her in her dreams. We would sit down together and draw her pictures and write letters. For a while she had an obsession about visiting Maddie's grave. I would take her to the grave yard and she would lay letters and little bracelets beside the headstone which was shaped like Piglet from the Winnie the Pooh books. She bought a wind chime to hang in the tree beside Maddie's grave and placed a tiny grey fairy on top of the ground above the grave to watch over her friend. Other days she would ask if she could visit Maddie's house and sit in her bedroom taking presents and pictures to place on her bed and cards to stick on the wall. Her letters to Maddie usually had a picture of Piglet on top.

One read: ‘Dear Maddie, I just want to say I miss you a lot and everyone says, “hello Maddie”. It's really annoying. Must go now. Love Hayley.'

During this distressing time, Hayley's counsellor Jane was invaluable. It was not always easy to have difficult discussions with Hayley as she tried to protect us from her true feelings and fears. But when she was painting and drawing or making things with Jane, she seemed to open up and talk about Maddie's death and her own life expectancy. Jane also introduced us to
Muddles, Puddles and Sunshine
, a book which helped young children cope with bereavement. It told the story of two characters called Bee and the Bear and there were puzzles and games to help Hayley to make sense of her feelings of loss. ‘She has a very positive attitude towards Maddie's death and is comfortable with it,' Jane relayed back to us a week or so after the funeral. ‘Clearly she has put Maddie in a beautiful place. When she thinks of her, she pictures her in heaven, she has hair and she is dressed up in beautiful clothes. Hayley feels she can go and visit her in her dreams. She can put herself in the dream with her and she's comfortable.'

Maddie's death was, without a doubt, the most traumatic things to happen to Hayley. For although her life had been one long succession of hospital appointments, poking, prodding and pills, it had been outweighed by the happy times: hobnobbing with the stars and visiting the kinds of exotic places most of her friends had never heard of. Yet no matter what she did, Maddie's name was always in her conversations. It was as if by talking about her, she was keeping her memory alive. One afternoon, while we were out shopping, Hayley came out with something that stopped me in my tracks. We had just reached the front door of Matalan, when Hayley said, ‘Mum, Maddie's just whispered in my ear. She was going “Hey, Hay, can I come shopping with you.”'

Oh my God, is she really hearing voices? I thought. I didn't want to discourage her, so I replied, ‘Of course, she can come along, Chick. And she can help you choose a necklace if you like.' Inside the shop, she chose two identical angel necklaces: one for her and one for Maddie which she placed in her bedroom.

The hardest part of Maddie's death was the knowledge that there was a treatment in sight. Two months earlier we had been at the Progeria Reunion in America when Dr Leslie Gordon from the Progeria Research Foundation told us of a new treatment they were working on. It broke my heart knowing that we were so close to the breakthrough we have been waiting for all our lives and Maddie never lived to get the chance to try it.

Chapter 19
Hayley
My First Funeral

I
HAD NEVER BEEN
to a funeral before until I went to Maddie's. Maddie was my best friend. She was nearly four years older than me but we were like sisters. Some people even thought we were really sisters. We used to give each other presents all of the time. Once she gave me a teddy bear holding a heart that said ‘Sisters are really angels in disguise'. We liked angels.

Maddie didn't really mind about her progeria, and neither did I when I was with her. When we were together we acted as if we didn't have it.

The last time I saw Maddie was the day before she died. We had a sleepover at my house and we argued over my Nintendo DS. She wanted to play on it and I didn't want her to because I wanted her to play with me. We had a bit of an argument.

The next morning, after Maddie had gone home, Mummy got called away. I didn't know where she had gone, but she rushed out suddenly. When I asked my dad he said it was ‘grown-up' stuff. That usually means that it's something boring, so I sat down and watched TV.

It was hours before Mum came home and when she did I noticed her eyes were red as if she had been crying. She turned off the TV while I was watching SpongeBob SquarePants. It was one I had seen before where SpongeBob can't stop laughing and Squidward gets really annoyed. Why are you turning the telly off? I thought, then Mum sat next to me and started crying. She said Maddie had gone to heaven. That made me so sad. I started crying too. I sat on Mummy's lap and we both cried and cried. I cried so much that my tummy started to hurt.

One day after Maddie had gone I heard Mummy and Daddy talking about going to Maddie's funeral.

‘What's a funeral?' I asked. They said it was when people go to a grave yard before they go up to heaven. I remembered I had been to the grave yard with Nanna and Pops so I said I wanted to go to the grave yard with Maddie, too.

Mummy wasn't very happy about it. She said it would be better if I stayed home with Nanna and Pops.

‘But I want to say sorry to Maddie,' I said. They said I could go as long as I wouldn't get upset.

The night before the funeral I sat in my bedroom and wrote a card to my friend. I traced a photo of Piglet because she liked Piglet.

For the funeral I wore my purple satin dress, Mummy wore a pink top, and Daddy wore a pink tie. That's what Maddie would have liked. She didn't like black. She wanted people to wear pink and purple. I knew this because we talked about it. Mum said I could put some make-up on to go and she let me choose what colour eye shadow I wanted to wear. I chose purple to match my dress. I wanted to wear Mummy's wedding veil, but she said that was a bit much.

At the church Maddie's mummy and sisters were crying all the time. I cried a little bit too. There was a man in black with a white collar around his neck, reading from a book, he said lots of nice things about Maddie and her progeria. Then I was allowed to say the speech I had written.

I said, ‘Maddie, I love you lots and always. I'm sorry we had a fight. Thank you for being my best friend and sister. Hope you had a safe journey. See you in Heaven.

P.S. Have fun.'

I felt sad and upset, but I didn't cry. Mummy and Daddy were crying and lots of Maddie's aunties and uncles and cousins were crying too.

When I went to bed that night I dreamt about Maddie. In my dream we didn't have progeria because we had taken a cure. We both had hair. My friend was tall with long, blonde hair. She looked like a normal eleven-year-old girl. In the dream I was out shopping. I went up to the counter and Maddie was an angel. She said to me, ‘Hello Hayley. See? I'm not really dead.'

I always talk about Maddie as I don't want her to ever be forgotten. In my bedroom I have a memory box where I keep all of the things I don't want to throw away like birthday cards, old photos and tickets from our trips to America. In my box I also keep a half eaten packet of crunched-up Frazzles that Maddie didn't finish on her last sleepover. Maddie's teeth were not very strong so she couldn't crunch crisps. Whenever we had crisps I would squash them into little bits for her so that she could eat them. She had kept half the packet for the next day, but she never had the chance to eat them.

I miss Maddie the most when I am sad or hurting. I think she was the only one who could understand what I am going through. Sometimes when I wish hard enough I can hear her. She says, ‘Hello, Hay,' that's what she used to call me. I am never sure whether I really hear her voice of if I am dreaming, but it sounds real to me. One day I hope I will see Maddie again in Heaven.

Chapter 20
Kerry
Hope for Hayley

F
OR
C
HRISTMAS
2005 M
ARK
and I took Hayley, Louis and baby Ruby on holiday to Disney World, Florida, to meet up with Dr Leslie Gordon and Scott Berns, founders of the Progeria Research Foundation, and their son, Sam. A year older than Hayley, Sam was a happy, all-American school boy who loved Lego and the Boy Scouts.

As we were standing beside the tea cup ride, watching Hayley, Louis and Sam spinning around and laughing, Leslie said the words we had been so desperate to hear,

‘I think we might be close to starting a drug trial.' Although it was unofficial and still under wraps it was the best Christmas present we could have wished for. I looked across to Hayley and Sam, who were grinning as the force of the spinning tea cup rocked them from side to side. Was it too much to dream that these children would live to an age where one day they would be seeking their thrills from white-knuckle roller-coasters instead of gentle merry-goround rides?

For the past four years Leslie and a team of scientists in America had been trying to find the cause of progeria in order to work on a cure. Every year at the Progeria Reunions, parents would get an update on the laboratory work and we would go home and cross our fingers, hoping that one day in Hayley's lifetime a drug would be found to counteract the devastating effects of progeria. By taking samples from children at the reunions, Leslie and The Progeria Research Foundation had built up a cell bank that held the progeria cells for scientists to discover things like the gene mutation which caused progeria. In 2003 they had come up with an exciting breakthrough discovery. They had found that the reason children like Hayley were ageing so quickly was all down to a tiny mutation in each child's DNA. Every human cell has a protein called Lamin A, which holds the nucleus of the cell together, but they found that progeria children had a defective mutation in their Lamin A called progerin. Progerin was a defective and unstable protein that caused children to age prematurely and only one in eight million children were affected by it..

‘We now know enough about progerin to find a potential treatment for it,' Leslie explained. ‘We have found a drug called farnesyltransferase inhibitor. We treated cells with it and also progeria mice with it and it looks promising in those experiments. Now we have to wait for approval from the American Federal Government's Drug Agency to start human clinical trials.'

I wanted to hug her. With every year that passed more children died and it became more urgent that a cure could be found in time for Hayley. Our once naïve notion that a pill could combat Hayley's condition now seemed to be more real. But the question remained – would it arrive in Hayley's life time? Our holiday ended on a high and we flew home to Britain to wait for the call that we hoped would change our lives for ever.

As part of the ongoing research, we were asked if Hayley would be willing to take part in a major study into progeria which was being carried out at the National Institute of Health in Maryland, near Washington DC.

Once the scientists working with the Progeria Research Foundation had discovered the progeria gene in 2002 they knew they would need to understand progeria at a clinical level if they were ever going to find a cure. In order to do this the PRF had teamed up with researchers at the NIH to launch the Natural History Study to get a better insight into the bodies of progeria children. A total of 15 children, including Hayley, were invited to take part in the tests. It meant two week-long trips to the US for a full body MOT. Everything was tested and checked; height, weight, heart, blood, bones and teeth. They even tested her tears for acidity using litmus paper. They also made her walk on a treadmill to test her heart rate under strain. During these tests Hayley became the first progeria child to complete an MRI scan to build up a picture of the inside of her body. Usually children of that age were unable to lie still for long enough to get a complete picture, but Hayley managed to spend 45 minutes in complete darkness to give doctors their first insight into the body of these special children. During these tests doctors noticed a constriction in one of the arteries in her neck which was feeding her brain. It was a worry as strokes were one of the common killers of progeria children, but we were able to monitor it and it righted itself in time.

After the Washington tests, we returned home and waited and waited. Winter turned to spring, and there was still no news. Summer came and we flew to America for another reunion, but there was still no start date. With every week that passed we became more frustrated. We were told that the treatment had been tested on mice and the early results were promising. The mice had put on weight and lived longer. I understood that the US Government's Drug Agency had to give its approval before they could be allowed to test the drug on children, but a clock in my head was ticking. Time was not on our side. Every month we waited was almost a year off Hayley's short life. The wheels of government might move slowly, but progeria doesn't.

In the meantime Mark registered a charity in Hayley's name to raise money to support children and their families living with progeria. With Mark as chairman, me as vice-chairman and a family friend as trustee, Hayley's Hope gathered the numerous offers of help with fundraising and donations from well-wishers into a central charity, which helped to pay for extra expenses for our trips to Boston, which weren't being covered by the Progeria Research Foundation. Through the charity we were also able to donate money to the foundation for their on-going research.

On April 28 2007, we finally had the official call we had been waiting for. Hayley had been accepted on to the drug trial. I remember the day well as our Belgian friends, the Vandeweerts, who had been frequent visitors to our home since our first meeting at the Progeria Reunion, were on the final day of a holiday with us in England. Hayley and Michiel were playing in the garden, I was in the kitchen cooking pasta for our dinner when the phone rang. It was Leslie.

‘Great news. We've had the go-ahead from the Drug Agency. The trials are starting next week,' she said. Sixteen months of anticipation were over, 28 children from 16 countries would soon be flown out to Boston Children's Hospital, two at a time, to be given what we hoped would be the wonder drug. They would start with some of the American children. Hayley was being paired up with her friend Michiel for her treatment, which would start on May 26. It meant that for the next two years we were committing ourselves to fly out to Boston every four months, for a total of seven visits.

Leslie explained that arrangements were being made for Hayley and Michiel to spend five days at the children's hospital. For the first four days she would have every test and scan available to ensure her ageing body was capable of taking the new drug. Then on the last day she would be given the pill, making history as the first child from the UK to test it. In the meantime we had already spoken to our own doctors in the UK, who consented to monitor Hayley on a weekly basis checking for growth and improvement and potential side-effects. Their health reports would then be fed back to the research team in Boston.

‘Are we doing the right thing, taking part in this trial?' I asked Mark. ‘This drug has never been tested on humans before. We don't know if it will work or if it will cause more damage.' We had been warned about the possible side-effects which ranged from mild nausea on one end of the scale to possible death at the other end.

‘Without it the prognosis is not good, so we have no choice, really,' Mark said.

He was right. Without the drug we were four years away from the dreaded life expectancy age of thirteen. For every year after that we would be living on borrowed time, worrying that at any moment Hayley could suddenly drop and pass away in our arms, just like her friend Maddie.

‘There are side-effects for everything. Even paracetamol can cause breathing difficulties in some extreme cases if you read the small print. The drug companies are just covering themselves. We have to look on the positive side. We can't let Hayley sense we are worried,' Mark said. I knew he was right, but it wasn't easy to follow his advice. My mum had also been putting a dampener on it ever since we first mentioned the possible trials a year earlier.

‘What if it goes wrong?' she asked.

‘I know it's a risk because the drugs have never been used on progeria children before,' I replied. ‘But we know what the best outcome could be – a longer and healthier life for Hayley. We just don't know what the side-effects will be, if any. She's getting older, Mum. It's a risk I'm willing to take. We have no choice.' I reassured her that we were in safe hands as the drug company was one of the biggest in the world. ‘They are not going to play God with our children,' I continued. But I understood Mum's concerns, I had already spent many nights lying awake worrying about the consequences if it went wrong.

In the days leading up to our departure for Boston, I began to sense that Hayley might be getting nervous. One night as I tucked her into bed and kissed her goodnight, she asked, ‘Can we delay the trial, Mummy?' Waiting was no longer an option, but how could I tell a nine-year-old child that her life is so fragile we may not have the opportunity next year?

Instead I replied, ‘We've got our plane tickets and hotel all booked, we can't change the dates now. And Michiel will be disappointed if you don't go, he's looking forward to seeing you again.' I was aware that Hayley was afraid of sharing worries with Mark and me. She worried that she might upset us. I was apprehensive too, but I had to put on an optimistic face for everyone's sake.

The night before our first trip to Boston we threw a ‘good luck' party with all of our family and Hayley's friends at a local pub, the White Rock Hotel. ‘Can I wear make-up, Mum?' Hayley asked as she picked out her outfit for the night – a white flowery dress and matching headscarf. I let her choose the colour of her eyeshadow. ‘Pink to match the flowers on my scarf,' she said. She was growing up into such a girlie girl, she had her own sense of style and fashion. At eleven some people might have said she was too young to be dressing up in make-up when she was pretty enough without it. But my attitude was always to let her be. She might never reach an age when she can go out for a night on the town independently, so I wanted her to enjoy her life.

A long, black stretch limo pulled up outside our house to chauffeur Hayley round the corner to her party where all of her aunties, uncles, cousins, friends from school and their parents were waiting. She danced the Locomotion and the Macarena, she ate sandwiches and crisps, and she spent the night telling everyone how excited she was to be going to America. When the party ended, Mark took over the DJ's microphone and thanked everyone for coming.

‘Keep your fingers crossed when we're out in America and we will do our best to come back with some good news.'

All our friends raised their crossed fingers in the air in unison and cheered. ‘Good luck, Hayley!' We didn't know what lay ahead. We had nothing to lose, but, in a way, we had everything to lose.