Old Before My Time Part 6

Chapter 17.

Hayley

Sibling Rivalry

I WAS NOT PLEASED when my brother Louis was born. I had to share my bedroom with him and he would wake me up in the middle of the night crying. When my sister Ruby was born I was happy. I wanted a sister to play with and I knew when she was old enough we could share our dolls and clothes. When I was little I had loads of Bratz dolls and I would let Ruby play with them and I would do her make-up. Now I am too old to play with Bratz and Ruby doesn't let me do her make-up any more. I asked her why but she just says she doesn't want to. I think I may have done it wrong once, but I don't really know.

Me and Ruby have a weird relationship. Sometimes we hate each other and other times we are best friends. It depends what mood Ruby is in. If she's in a bad mood we hate each other. We argue a lot. But at the end of an argument Ruby says, 'Are we still best friends, Hayley?' I say, 'Yes we're still best friends, Ruby,' until the next time when we argue again. When my best friend Erin comes round to hang out with me, Ruby decides she wants me to do her make-up and I say no, go away because I am busy. Recently Ruby has started saying, 'You don't love me Hayley,' when I tell her to go away. I say, 'Don't use that excuse.' But I still feel bad when she comes into my room crying and hugs me. I think she might get jealous of my friends.

Sometimes Ruby can be really annoying. Like once we were out in the garden playing with our tea set and I went to pour some juice in Ruby's cup but she wanted to pour the juice. I told her to say please. She didn't say please so I wouldn't let her have the juice. She ran in the house to tell Mum. That was really annoying. I hate snitches and I hate it when Ruby is a snitch. Sometimes I wish Mum would have another baby sister but one that would let me put make-up on her and won't snitch. Louis annoys me when he won't let me watch my programmes on TV or he won't let me play on his Xbox.

I feel like Ruby and Louis' big sister even though Louis is taller than me and Ruby is almost as tall as me. I ask them about their day at school and help them out with their homework. When I am watching TV in my room Ruby usually comes in and asks if I will help her. Sometimes I do, but it depends what mood I'm in and what I'm watching on TV. When I say no she always cries.

At night before we go to sleep me and Louis talk about random stuff. I tell him when I have aches in my body and he says, 'I'm really glad I don't have progeria.' Other times Louis worries that he is the smallest in his cla.s.s. I told him, 'Don't worry, Louis, I am the littlest in the whole school, just deal with it.' Louis has a girlfriend now and it's really sweet. When I ask him about his girlfriend he goes all shy. I don't have a boyfriend, I'm not interested in boys they smell and they are annoying and they expect you to do the washing up. Louis is a typical boy, he just sits around watching TV. Daddy is not annoying and he smells nice but he doesn't do the washing up. Mum says I have a boyfriend called Harry, but he is not my boyfriend. He's just a friend. He is eleven years old and lives in Yorkshire with his mum Sharron. He has progeria as well but his is a different sort of progeria. He ages five times faster. I met Harry when Mum took me to London on the TV programme This Morning and he was really nice and funny. We talk on Facebook all the time. Mum says he is a 'real gentleman'.

I think Ruby sometimes get jealous of me. One day when someone was filming me for TV, she wanted to get in front of the camera and the people with the camera asked her to play in the other room. She said, 'Why do they always want to film Hayley and not me?' Mum told her it's because I have progeria. If Ruby gets jealous, she goes off to play with her friends and forgets about it.

I can understand why she feels that way, if it was reversed I would be asking exactly the same thing and be acting the same. Louis doesn't act like Ruby, but he doesn't like getting his picture taken so every time the cameras come he hides in his room and plays on his Xbox.

Sometimes I feel jealous of Ruby. I'm not sure why. She has really nice clothes and I used to be able to steal them from her wardrobe. But now she is getting taller than me. Every day she says, 'Hayley let's see how tall we are?' She makes a really big deal that she's taller than me. So I usually stand on my tiptoes, when we measure so I look taller. I don't feel jealous of Ruby and Louis' health. If I didn't have progeria, I would not get to do cool stuff and meet really cool people.

Chapter 18.

Kerry

The Second Worst Day of my Life

HAYLEY'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 ma.s.sive heart attack and had pa.s.sed 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 pa.s.sing 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, "h.e.l.lo 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 att.i.tude 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: hobn.o.bbing 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 G.o.d, 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, 'h.e.l.lo 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, 'h.e.l.lo, 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

FOR CHRISTMAS 2005 MARK 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 pa.s.sed more children died and it became more urgent that a cure could be found in time for Hayley. Our once naive 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 Inst.i.tute 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.