Old Before My Time Part 8

The obvious choice of big school was Bexhill High School, where most of the children from her cla.s.s would be going. However, Mark and I worried that Hayley might find it a bit of a culture shock. She was moving from a small cla.s.s of 20 children in her primary school to a school of 1500 pupils. With so many older children from a wider area, I worried that Hayley might become more of a target for bullies. Up until that point she had lived her life relatively protected by her cla.s.s teachers and her close friends. Mark and I started looking at other schools and considering whether our finances could stretch to the thousands of pounds it would cost to send her to a private girls' school, where we imagined she would have more protection from playground bullies. Hayley was determined she wanted to follow her friends to Bexhill High School, so we dropped the costly idea of private education. She didn't seem to be worried about being in a school with bigger children whom she didn't know.

In the final days of her primary school, Hayley's teacher took her and her best friends Erin and Lydia for a tour of Bexhill High. When Hayley came home she was buzzing. 'Teacher says I can dissect a frog at Bexhill High,' she announced. It made me laugh knowing how much she disliked having needles yet she was not at all squeamish about cutting up a dead frog. She told me how during the tour of the school the headmistress had showed the girls around the biology laboratory where her friends dared her to stroke a snake. They were also given the opportunity to ask their new teachers about anything that might be worrying them.

'Erin told the teacher she had eczema and asked whether it would matter that she had to wear her jumper when it was warm. And the teacher told her that her parents could sign a form so that they would know and it would be all right,' Hayley said. 'Then Lydia asked if she would get bullied because she was short.'

'What did the teacher say?' I fished, hoping to get an insight into Hayley's own bullying fears.

'She said bullying wasn't an issue at Bexhill High.' I had already spoken with Hayley's teachers and been told there was a strong anti-bullying policy in place and they came down hard on any pupils they identified as bullies.

Unlike the start of primary school, shopping for uniform wasn't an issue. The school was very helpful in getting a navy skirt and jumper made in Hayley's size and a shorter clip-on school tie which was easier for her to wear. They even arranged a special bandana with the school badge on the front, which she wore for a few weeks before deciding she stood out too much and reverted to her own navy bandana.

The morning of her first day at Bexhill High arrived and, once again, I had more b.u.t.terflies than Hayley. She packed her pencil case, notebook, geometry set and her dinner money into her new backpack and waited for the school taxi.

As we walked up to the school gates she dropped my hand and started edging ahead. She looked so tiny and fragile compared to all of the other children. I was suddenly overwhelmed by my maternal instinct to wrap her up in cotton wool.

'Do you want me to carry your back pack?' I asked.

'Mum, I can do it,' she said.

'But there are other mothers doing it,' I protested.

'Mum, I can do it,' she said again and marched off to join her best friends, Erin and Lydia, in the playground, leaving me out on the pavement with the other mums.

At the end of the day I was back at the gates waiting to meet her. The school had allowed her to leave earlier than the other children to avoid getting caught in the mad scrum for the doors when the bell rang at home-time. It was the one concession where Hayley didn't mind being treated differently. Elsewhere in the school she wanted to be the same as the other children. Originally, the school had allocated her a lower desk and special chairs, but she hated being singled out for special treatment. When they moved to a brand new school building which had been built with lower desks in all of the cla.s.srooms she was much happier. Each cla.s.sroom pod had a selection of different types of chairs including six lower padded stools which she was able to sit on with the other pupils. She would not use anything that singled her out as being different from the other kids.

Throughout her high school life, Mark and I have always insisted that Hayley shouldn't be given any special treatment and this proved to be the case one day when I had a call, to say that Hayley was being kept behind after cla.s.s for being naughty. It turned out that she had drawn on the shirt of one of the boys in her cla.s.s and was rightly being punished for it. The boy's mother called me afterwards to apologise saying that she had asked the school not to reprimand Hayley. But I say, 'You do the crime, you do the time,' and Hayley had to learn that her progeria didn't protect her from punishment. It was her first, and I hope last, taste of detention.

In general, the teachers at the school have been very supportive of Hayley and her progeria throughout her school life. They have held special events and cake bakes to raise awareness and money for Hayley's charity. While Hayley is in school she has a teaching a.s.sistant shadowing her at a safe distance during break times to make sure she doesn't get knocked over. And whenever she is not feeling well the school will call me and let me know. I always tell them I would rather they call for nothing than risk leaving it. My biggest fear is that when her time comes I am not going to be there, so I don't mind being called for a false alarm.

Just before 4 p.m. on February 17 2011 the alarm bells rang in my head when I got a phone call from Hayley's school. I was in the house on my own as Mark had just taken the car to pick Ruby and Louis up from school. 'Nothing to worry about,' the voice on the other end said. My heart thumped. 'Hayley has fallen over and we think she's hurt her leg.'

'Has she bruised it?' I asked. This was a regular problem with Hayley's fragile skin. She was always covered in bruises from b.u.mping her head on the fridge door or brushing her arm against her bedroom door and quite often the bruises looked worse than they actually were.

'We don't know,' the school replied.

'Is she crying?' I asked. Again, they didn't know as the caller wasn't in the same room.

'Wait there, we'll be up straight away.' I put down the phone and called Mark. Five minutes later we were ushered into the school gym where Hayley was lying on a big blue crash mat. She looked a bit washed-out but she was trying to put on a brave face and was laughing and giggling while sitting on the mat surrounded by her friends.

'What have you done?' I asked.

'I've fallen over, but I don't want an ambulance,' she insisted.

I offered to carry her to the car but as I tried to pick her up, she yelled out in pain. I couldn't move her, and that's when it hit me that this was more serious than another b.u.mp. The teachers cleared the area and called an ambulance which was able to park right outside the entrance to avoid any on-lookers. It was hard to be standing by and not able to do anything as she screamed when the ambulance man lifted her onto the stretcher. That's when I noticed that one leg was three inches shorter than the other and panicked that she had done some serious damage.

At the hospital, the doctors X-rayed Hayley's leg and discovered she had dislocated her hip. She was taken down to the operating theatre and her hip was put back into place. It was an anxious time for Mark and me as it was the first time she had ever had a general anaesthetic. It was crucial that the doctors got the dose right. The dose for an average 13-year-old would be too much for her, but doctors were afraid that a dose that was equivalent to her weight might not be enough to knock her out. While we waited Hayley became more and more anxious. 'What if I don't wake up, Mummy?' she cried.

'Of course you will. And when you come round we'll get you the iPad you've always wanted,' I joked, trying to keep her mind off what lay ahead. When the mask was put over her face, she froze. She counted backwards from 10, and soon she was out. As they wheeled her into the theatre I stood in the doorway, not wanting to close the door on her, until a nurse came and led me away.

Chapter 25.

Hayley

Why I Hate School

IT WAS WEIRD STARTING my new school because everyone seemed really big and I was really tiny.

In the primary school there were kids who were littler than me but at Bexhill High I was, and still am, the smallest. At first I was afraid that someone might knock me over.

When I had to choose my big school Mum said 'Wouldn't you like to go to an all-girls school?' I told her I wanted to be with my friends. I asked Erin and Lydia what school they were going to go to. When they said they were going to Bexhill High I told Mum that that was where I wanted to go. I told her, 'I want to be with my friends, thank you very much.'

Our teacher at the primary school took us to Bexhill High to have a look and ask questions. They showed us the biology cla.s.s. Dad said to me that you have to cut up frogs in big school. I was too shy to ask the teacher but Lydia said, 'Hayley wants to know if she can dissect a frog?' The teacher said we would. I thought that big school would be cool.

On my first day at big school all the year sevens had to go into the hall for a.s.sembly. There were so many other kids there I was scared I might get lost or lose my friends. I sat down near the front of the hall with my friends. Then we had to stand up when the headmaster came in.

'Good morning year seven,' he said.

'Good morning, sir,' we said. He started talking, telling us something about beginning a journey that helps to prepare us to make our choices for the future.

Then he said there was to be absolutely no violence in school, like fighting and pushing and shoving to get to the front of queues.

'That is totally unacceptable,' he said and asked us if we understood. We all nodded. I felt happier. I was scared that I might get knocked over.

In my cla.s.s's form room I had a special chair and desk which was higher than the others. I had two other chairs that I was supposed to carry around to lessons because I was too little to sit on the normal chairs. But I didn't like them. In biology my special desk was near the front next to the teacher, but I didn't like sitting there and being treated differently. So I asked the teacher if I could move and she let me move further back to sit next to Erin.

In school they gave me a special chair to reach the desks but my mum said I didn't have to use it if I didn't want to. In cla.s.s we have blue and yellow chairs which are really hard and we have cushion chairs called pouffes. I like to sit on the pouffes.

The playground in my school is ginormous, not like my old school. Everywhere is really busy and I am afraid I will get knocked over. When I have to walk past boys playing football, Erin and Lydia walk in front of me to protect me from getting knocked over by the ball. During the break times I like to go to the library where it's quiet and safe. I don't like going outside as there's nothing to do except stand around, and it's noisy with everyone shouting. No one shouts in the library. It's not allowed.

I am lucky that I have amazing friends. I have become more self-conscious as I get older but I don't think anyone would say anything bad behind my back. Obviously people who don't know me probably would and some people have, but I ignore it.

I know I have loads of friends at school who will stick up for me. If someone is staring at me they say, 'Stop staring! Leave her alone.' They are not afraid to stick up for me even if the kid doing the staring is bigger than them. Erin will stick up for me even if the other kids are much bigger than her. She is fearless. I am really lucky to have good friends like them. Sometimes when we are in the playground my friends get down on their knees to talk to me, but this annoys me. I say, 'You can stand up, you know. I can hear you.'

I like doing cooking in school. I made cheese straws, cheesecake and flapjacks like Nanna makes. I cooked risotto as well. I had never had it before and now it's one of my favourite meals. It's easy to make, but it takes for ever. Mum and I make it with carrots, onions, garlic peppers and stock. I would eat it every day if I could. Mum says I don't eat a lot but that's because I always feel full. I think it must be because of my progeria because Erin can eat loads more than me.

Once I had detention because I drew on a boy called Kyle's shirt because he was annoying me. The teacher caught me and I had to stay in cla.s.s late and Mum had to pick me up after the other kids had all gone home. It was annoying because Kyle's mum wasn't mad about it. But Mum said it was right that I should get detention because I am no different to the other kids and that's what I get for being naughty. The next day Kyle said 'What kind of smiley face was that?' He wasn't bothered I had drawn on him.

Now I am 13, I hate school. The work is pointless. I like reading books at home, but not in school because they have rubbish books at school. When we have to go and choose a book to read I always get there last and all they have left is a dictionary. I have to read a dictionary for half an hour which is boring. So when we are supposed to be reading, Erin and I talk behind our books.

I hate my teachers too. They split me and Erin up for no reason. We were just sitting there talking and the teacher made Erin sit on another table. The best thing about school is the lunch time and home time.

I was pleased when I first dislocated my hip last February because it meant I didn't have to go to school. Mum arranged for me to have lessons on the computer at home.

It all happened when I was in the PE hall watching my friend trampolining. While she was waiting to go on I was sitting on a bench, and I stood up to get my bag from the other side of the room and I just sort of tripped over the bench and my left hip popped out. It really hurt, but I didn't cry. At first I was going, 'Owww,' and shouting. Miss Baker, the teacher, was really nice to me. She told two of my friends Fred and Charlie to get a crash mat. Everyone was gathering around, so Miss Baker told them all to go away. There was just me, Fred and Charlie. The teachers at the school asked me if I wanted an ambulance and I said no because I didn't know I had dislocated my hip. Then they said, 'Let's call your mum.' While we were waiting for Mum the teachers tried to get me to stand up and that's when it really hurt and I said, 'Ow!'

Mum, Dad, Louis and Ruby rushed in. By this time it was nearly home-time so Fred and Charlie had gone home. When Mum tried to pick me up she couldn't do it because it really hurt. She said, 'Call an ambulance.' I was just thinking, here we go, because I didn't think there was much wrong with my leg. I just thought I had fallen and badly bruised it.

When we got to hospital I had an X-ray and they said it was dislocated. I was thinking, what are they going to do with me? When they said they were going to put me under general anaesthetic I was really scared. I thought, what if I don't wake up? They put the mask on and told me to count down from 10 to 1. They said, 'You probably won't get to 7.' I was holding my mum's hand and started counting. I said 'I love you, Mum,' and then I was gone. I remember it was 12.20 a.m. when I went into the operation and 1.20 a.m. when I woke up. I know that because there was a clock on the wall. At first I felt sick and I wanted to gag where there had been a tube stuffed down my throat. I was really tired as well. Then I saw my mum and said, 'Am I going to get an iPad?' That's all I cared about because when we were waiting in the hospital Mum said she would buy me one. The next day I was allowed to go home with my mum.

I didn't have to go to school for a couple of weeks. But the next day my friend Erin came round after school and said there were some kids who said that I had died. That was really annoying. I thought that it was so typical of the kids in school. Why don't they just get their facts right? Just because I fell and had to have an ambulance doesn't mean I've died. I told Erin to tell them I had dislocated my hip and I was fine, thank you very much.

I can't wait to leave school. I know what I want to be. I want to be a DJ or a film maker like James and Nicki who have made programmes about me. Sometimes when they are filming me they leave their camera in our house and when they are not looking I pretend to be a film maker. When I am a famous director I want to make chick flicks like my favourite movie Confessions of a Teenage Drama Queen, which is about a girl who moves away from New York and has to make new friends in a different place, but she gets into lots of trouble.

I am always listening to Radio 1 so I think it would be cool to get a job as a DJ. I could play lots of different kinds of music and talk in between tracks. We have a radio station in school and they let me go on and talk about the fund-raising we were doing for the Progeria Reunion. I got to play a record for my mum so I chose Next to You by Justin Bieber and Chris Brown because that was one of her favourites. I think I would make a good DJ. Maybe one day I will write to Fearne Cotton who is my favourite DJ on Radio 1 and ask her if she needs some help.

If I can't be a film maker or a DJ I want to be a beautician. This year I had to choose my options and I have chosen hair and beauty. Me and Erin want to run our own beauty salon. Erin says we should call it Magic Fingers but I don't like that name, I think Stars is better. Sometimes we dream about what our salon will be like. It will have a red carpet and a mud bath in the middle and it will be somewhere that isn't Bexhill. I can't wait until I am old enough to leave school.

Chapter 26.

Kerry

Tough Choices

BY SPRING 2009 AS the first drug trials in Boston were coming to an end we received news from the Progeria Research Foundation of another trial which doctors hoped would have even better results by using a combination of different drugs. The research team had identified two extra drugs that combined with the FTI drug Hayley was currently taking could prove even more effective. The additional drugs were statins, usually given to patients with high cholesterol, and a bisphosphonate drug used to help osteoporosis sufferers.

There was just one fly in the ointment. At that point we had not had any official results from the first trial and once again we were in a dilemma as to whether or not to expose Hayley to another c.o.c.ktail of drugs. Our gut feeling was that it was working. Before she started taking the FTIs she was growing an average of 2cm every six months, but her growth rate had almost doubled since the trial started. Dr Whincup also seemed to be pleased with her progress during our monthly check-ups at the Conquest Hospital.

In Boston the research team had started conducting a month-long mini-trial of the triple drug on five children aged between two and three to check if they were able to tolerate the potential side-effects. When the results of these trials proved satisfactory they would move on to a larger group, giving us the opportunity to include Hayley.

I read out the detail of my email from Dr Gordon at the foundation to Mark. 'It says that bisphosphonates are used to help elderly people with their bones. The drug is administered intravenously and its common side-effects are fatigue, anaemia, muscle aches, fever and swelling in the legs.'

'Do we really want to put her through another round of needles and possible side-effects for something that will make her grow just a little bit?' Mark asked. I shared his concern. If we wanted to take part this drug wasn't simply a question of popping a pill, it would mean Hayley would have to be hooked up to a drip for 30 minutes at a time. She hated needles and I wasn't sure we could force her to have more injections. Once again we were torn. Mark and I wanted to give Hayley every possible chance of prolonging her life, but not at any cost.

To help us learn more we visited a progeria expert at Brunel University in the UK. Dr Ian Kill and his team of scientists were studying the effects of ageing in progeria children and normal adults. In his laboratory Dr Kill was working on fruit flies, which apparently have a similar genetic make-up to humans, to find ways of slowing down this ageing. Through a microscope we were shown the flies they had injected with progeria cells. They were not buzzing around like normal flies, but they were crawling around half dead. Dr Kill then sat us down and explained how the FTI drugs, which Hayley had been taking for two years, were able to block the pathway of the progeria cells in the body. But he told us that sometimes this happened late in the process and that's why his team of scientists were trying to find ways of starting the blocking earlier.

They had discovered that the pathway of ageing was similar to the one used in the production of cholesterol. He showed Mark and I a diagram with arrows pointing to lots of long names. It meant nothing to us, but the long and short of what he was telling us was that statins, which are taken by people with high cholesterol, could be used to treat progeria. By a fortunate coincidence, Hayley was already taking statins, prescribed by Dr Whincup. He had first prescribed them because both my mum and I suffered from high blood pressure. Armed with this new information, Mark and I decided it was worth a try.

Before leaving for Boston that August for the final session of the original FTI trials and the start of the triple drug trial we had another consultation with Dr Whincup for the results of Hayley's ultra-sound kidney scan. One of the known side-effects of taking bisphosphonate was that it could cause calcium to build up in the body. Previously there had been some concern that Hayley might be showing a build-up of calcium on her kidneys and the Boston doctors needed proof that Hayley's kidneys were fit and functioning well. The results showed no deterioration in her kidneys, liver or heart. 'No lumps, no nasty things. You're good to go,' Dr Whincup said to Hayley as he gave his blessing for the new round of drugs.

During our visit to Boston, Hayley and her Belgian friend Michiel were presented with trophies for completing the first-ever progeria clinical drug trial. They were among 45 children from 24 different countries who would be taking part in the new study. The group also included some progeria children who had been too young to take part in the original trial and others who were just starting the single FTI drug trial. The Boston Children's Hospital and its partner the Dana-Farber Cancer Inst.i.tute had received a $3.1 million grant to fund the cost of the trials. By signing up we were committing to another two years of trips to Boston.

After the usual rounds of blood tests and tissue samples, Hayley was hooked up to the machine which delivered the bisphosphonate into her blood. She had to lie still on a bed while the drug was pumped into her blood stream through a canula in the back of her hand. It was a boring process and one that would have to be repeated every six months but Hayley was already beginning to treat the Boston Children's Hospital as her second home and was happy to lie there playing on her Nintendo DS game while the drugs did their work. When her 30 minutes were up everyone gave her the thumbs-up which seemed to be a good sign. Sometimes that was the only way we could tell if things were going well, we watched the doctor's expressions. If they smiled we knew they were happy and we could take some comfort from them too. The doctors explained that if the three drugs worked effectively then the progerin would become 'paralyzed' and Hayley's progeria could be improved. The scientists working for the Progeria Research Foundation say they are learning a lot about ageing from Hayley and the other progeria children, particularly when it comes to heart disease. Children with progeria don't smoke and don't have high cholesterol so scientists are able to study the effects progerin has on heart disease.

'Our initial reasons for coming to Boston were purely selfish to give Hayley a chance of longer life,' I reflected to Mark on our journey home. 'Yet it's mind-blowing to think that these drugs we are giving her could help millions of older people around the world who suffer from heart disease. Not only are we saving Hayley's life, Hayley could be saving the lives of others.'

For the next two years we continued visiting Boston for the triple drug trial treatment.

Unlike the first trial there were no bad side-effects and we felt that the trips were worth it. Hayley continued to grow and her check-ups with Dr Whincup continued to bear positive results. A CT scan of her heart during this time showed none of the deterioration in her arteries which he had expected. In real terms Hayley's body was then equivalent to people in their nineties. We knew that the biggest killer of progeria children was heart disease so confirmation that her heart was still strong was good news. But the greatest boost to our confidence came when Hayley visited another specialist team who had not seen her for more than a year. They were surprised by how healthy she looked. They said her they could see an improvement in her face, her cheeks were fuller and her skin was healthier. Being with her every day we noticed changes for the better but often wondered if it was more wishful thinking on our part; to have our thoughts endorsed by a professional was a real boost to our optimism.

My other great fear for Hayley as she got older was mobility. Over the years at the Progeria Reunions we had met several children who were handicapped by their prematurely ageing bodies. Like some elderly people they had to rely on wheelchairs to get around because they were too frail to walk on their own. After Hayley's accident in the school gym I began to worry that her bones were beginning to let her down. Hayley was so full of life I knew it would kill her to lose the use of her legs.

A few weeks after her accident in school we had a second scare when she slipped on a washing powder capsule in the kitchen and dislocated her left hip again. It was such a silly, typically childish accident. She was trying to burst the plastic washing powder bubble with her foot and slipped. Seeing her in such agony upset me. I worried that this was the start of a major problem. I called the ambulance and again she was rushed into the Conquest Hospital for an operation to put the leg back in its socket.

We found ourselves back in the accident and emergency department a few weeks later again. It was beginning to feel like groundhog day. There was obviously a weakness in her hip by now as she had just turned around on her bed and her leg had popped out of its socket again.

'We think Hayley would benefit from a leg brace. She will need to wear it 24 hours a day for six months to give her muscles and ligaments the chance to repair themselves,' the doctor advised me.

'Wouldn't a hip replacement be better?' I asked, knowing that there was no way on earth I could persuade Hayley to wear the uncomfortable and ugly leg brace for half a year. But the doctor explained that there was no guarantee a hip replacement operation would work in Hayley's case.

Not happy with the suggestions we sought a second opinion with a specialist in London, who did a CAT scan of Hayley's hips. The scan showed that whereas most normal people have C-shaped hip sockets which securely cup the ball joint on the top of the thigh, Hayley's was so worn down it was virtually flat. So even if they did operate to replace the hip joint there was no guarantee the artificial socket would be secure. The conclusion was that the brace was the only alternative.

Hayley was unhappy with the idea and I had my work cut out convincing her otherwise. Then a few weeks later she dislocated both her left and right hips while she was walking up a flight of stairs and the decision was made for us and the hospital ordered Hayley a leg brace. When it arrived she wasn't at all happy and I can't say that I blamed her. It was such an ugly, uncomfortable contraption, it swallowed her up.

During her periods of convalescence I was worried that she would be falling behind with her school work. But Hayley's teachers were great and quickly made arrangements for her to take her lessons online. Every day we sat down together and Hayley would learn maths, English or science and when she was done she would sit quietly in her bedroom and read. It was the ideal solution. Hayley was happier not having to go into school and I felt safer knowing that she was back under my protection.

Chapter 27.