Old Before My Time Part 3

Chapter 7.

Hayley

No Hair, no Nits

THE DAY I STARTED school I don't really remember very much about it but I had a film camera following me. Mum said they were making a programme about me for television. I thought that all children went on television when they started school, until Mum told me that it was because I had progeria. When I asked what progeria was, she said it meant that I was very special because only one in eight million children had progeria and that's why I was little and didn't have much hair. I remember thinking that if I was special I must be a princess or something.

When I first started school I didn't mind that I didn't have much hair because I knew I had progeria. One day the man with the camera who was making the programme about me asked if I minded having no hair. I said, 'It doesn't worry me.' Then I told him a funny story. I said 'One day someone said to me if you had a wish what would you wish for. I said nits. Because other people have hair they can get nits and I don't.' The man with the camera thought that was funny. When I look at the old videos of me on TV, it makes me laugh because I was so little.

One day in school I remember my teacher Mrs Haines told us about being different. She called John and Samantha out to stand in front of the cla.s.s. 'What colour eyes does John have?' she asked us.

We all said 'blue' because they were the same colour as mine.

'And what colour eyes does Samantha have?'

'Brown,' we said.

Then she asked if they could change the colour of their eyes. I thought it would be cool if you could change the colour of your eyes. I would have pink eyes because pink is my favourite colour. But Mrs Haines said we can't change the colour of our eyes. 'That's just the way we are. They were born with blue and brown eyes,' she said. Then she called Kyle out to the front of the cla.s.s and asked us what colour hair he had and if he could change it. I thought that might be a trick question because you can change hair colour. When me and Mum were out shopping once we saw one of her friends and her hair was red. Mum said she had dyed her hair. I didn't know hair could die and thought maybe that's what happened to mine.

Kyle's hair was almost white, but Mrs Haines says it's called blond. 'Kyle has blond hair. That's who he is.' Then she called me out in front of the cla.s.s and asked the other kids, 'Does Hayley have hair?' The other kids said no. 'Can she do anything to change it?' Mrs Haines asked.

'No, Miss,' they said.

'Repeat after me, we are who we are,' she said. 'We are who we are,' we said.

Even though my teacher said we can't do anything to change who we are, sometimes I still felt different having no hair. Mum bought me some hats and a bandana and now I wouldn't go out without wearing one. I don't mind going around the house with a bare head but I always wear a bandana outside. That's my trademark now. I have lots of different coloured ones but my favourite is pink. I don't like people staring at my head in school or when we go shopping. When I was little Mum told me I could stick my tongue out at people when they stared at me. I don't have to do it so much now because people don't stare so much now. They come and say, 'h.e.l.lo, Hayley,' because they have seen me on television and in the newspapers. It is almost like I am famous or something.

The day I first started school I met my best friend Erin who started at the same time as me. She is exactly one month older than me. Her birthday is November 3 and mine is December 3.

I remember Erin because she was sitting in the corner on her own playing a Thomas the Tank Engine game on the computer. I noticed her because she always wore a jumper, even when it was hot. She was scratching herself all the time and the other kids called her 'spotty' and 'beetroot' because she had red skin that was always itching. I didn't care and went over and said, 'h.e.l.lo, my name is Hayley. Why are you scratching?' She said she had something called severe eczema that made her skin get all red and itchy so she never took her jumper off, even in the summer.

'I have got progeria and I can't run or play IT,' I said. I wanted Erin to be my friend because she was different like me, but she was bigger. When I played hide and seek in school I couldn't catch the other kids because they ran too fast, but Erin didn't run away. She has always stood by me because that's what best friends do.

Chapter 8.

Kerry

Growing Up

WITH EACH Pa.s.sING YEAR the progeria began to leave its mark on Hayley. Throughout her primary school years her body took on the features of old age, she lost more and more fat and her frame grew thinner. Mark would call her a 'bag of bones'. The most obvious sign of change was the loss of her hair. While we were on holiday in Disney in 1999, her last strands of hair fell out. She only had one tuft left on the back of her head but she begged me to have it braided in a pretty pink hair wrap. When the wrap came out several weeks later, it took her final strands of hair with it. At the time this was more upsetting for me than Hayley because it was an obvious sign of her deterioration and I knew that without a miracle it wouldn't grow back. I still have it today, tucked away in a special box along with her first hospital gown and the nametag she was given when she was born. They are the bittersweet memories we keep.

Emotionally she started to grow up too. Like many other children of her age she started to develop a mind of her own and became more stubborn. If she didn't want to do anything, no amount of persuasion could get her to change her mind. This sometimes proved difficult when we had to visit the doctors and bribery would often come into play a new toy or sweets usually did the trick.

Fortunately she didn't need too much persuasion when it came to our six-monthly check-ups with her cardiologist Dr Graeme Whincup at the Conquest Hospital, Hastings. Heart disease is the most common killer in progeria children so it has always been important to check for thickening of the arteries or any other signs or irregularities that could indicate her heart is growing old. At first I think she had a little bit of a crush on Dr Whincup because he was young, blond and handsome. She was fascinated by the large collection of coloured bow ties he used to wear and she was always made to feel special because he treated her like a best friend rather than just another patient. In order to check the condition of her heart Dr Whincup takes regular echocardiograms which use ultrasound waves to show the outline of Hayley's heart on a monitor. That way he can see if the valves and chambers are all in order and pumping blood around the body as they should. Another regular test is the electrocardiogram (ECG). To do this he used sticky patches to attach electrodes to Hayley's arms, legs and chest which gave a reading of her heartbeats and would show any signs of enlargement. He also monitors her blood and checks her cholesterol levels. The cardio visits have always been and an anxious time for us as we never know when an abnormality will appear inside Hayley's body. It was, and still is, a relief when the tests come back as normal and we are rea.s.sured that Hayley's vital organ is not deteriorating.

Late one night, when Hayley was around six years old, I woke up in a panic. I heard Hayley cry out, 'Mummy, I've got a pain in my chest.' I rushed into her bedroom where she was sitting up in bed, looking grey. I felt her forehead it was clammy. 'It feels like someone is sitting on my chest,' she whimpered. My immediate thought was, are we losing her? With no time to waste to call an ambulance, I bundled her into the car and shot to the nearest Accident and Emergency unit, jumping red lights en route. Eight minutes later Hayley was rushed into the cardio unit and hooked up to an ECG machine. I spent the rest of the night beside her bed, watching the monitor for any changes, willing her to be all right. When she woke up the following morning, she seemed much better, the colour was back in her cheeks and the doctors were happy that her heartbeat was back to normal. We were told she had suffered a supraventricular tachycardia or SVT for short. Doctors explained to us that it was nothing to worry about and was usually caused by intense excitement, stress of fright. We had been to a bonfire party the night before it happened and it was possible that the excitement of the fireworks had set her heart racing. We were just relieved it had not been more serious.

Around this time in her life our family was introduced to a new type of care for Hayley. Jane Streeter was a palliative care nurse who worked for our local hospice for terminally ill children, Demelza James House. When it was first suggested that she could help us, Mark and I were sceptical.

'We don't need a care worker. We know how to look after our own child and we can answer any questions she might have,' Mark said. I agreed with him up to a point. It seemed to be a slight on our ability as parents to have outside intervention. But, once we were introduced to Jane, it became apparent that she was going to be a valuable friend to us all. Jane was an experienced bereavement counsellor and play worker all rolled into one and was able to talk to Hayley about her child-like concerns in a way that Mark and I couldn't. By combining play and conversation, Jane quickly built up a trust with Hayley. She became another of Hayley's friends, who would take her out to the park or swimming pool and stay indoors and cook and colour with her. Their time together was private and special and as Hayley became more inquisitive it became obvious that Jane's intervention was essential when coping with difficult inquisitions.

Death was the big question. From the day of Hayley's diagnosis it had hung over us like a giant boulder which could drop anytime without warning, causing major damage. As parents of a progeria child thoughts of death were always in the back of our minds. Especially when we attended the Progeria Reunions and learnt that several children, who had seemed perfectly healthy the previous year, had died in the previous 12 months. Initially Hayley was too young to register the terminal nature of her illness and that of children like her, but we knew that one day the subject would naturally arise and we would need to arm ourselves with an answer that wouldn't frighten her. Mark's att.i.tude was 'there's no point in worrying about it, it will just make us older and greyer than we already are. Just take it as it happens.' But I couldn't be so blase, I couldn't risk saying the wrong thing and causing emotional damage to our happy little girl.

'Does Hayley know her life expectancy?' Jane once asked me when we were alone. 'No, she doesn't need to know. The average age for a child with progeria is 13, but some die as young as six,' I confided in Jane. 'One girl died last year and she was 22. So it's hard to tell. At the moment she hasn't mentioned it but we know as she gets older there will be more questions. Sometimes it's hard being her parents. We want to protect her but we also want to be honest with her.'

'You need to be careful of what you need and what Hayley needs,' Jane replied. 'For a child her perception of life is very innocent. Hayley will ask awkward questions when you least expect it. You need to prepare yourself. You will be rushing to get ready for school one morning and she might ask the question you're dreading: "Mummy am I going to die?" Answer her and say, "We need to talk about it later, that's a big subject." But be honest we are all going to die sometime. Trust me, I am used to dealing with children who have terminal illnesses and that's the best way to answer.'

Watching Hayley and Jane together gave me confidence. They had a special friendship, as well as a patient's confidentiality. Hayley knew if she pa.s.sed a secret on to Jane, it was safe. And Jane was able to give us the rea.s.surance we needed about Hayley's state of mind at a time when I was still too emotional to cope alone. 'She wants to protect you and is careful what she says in front of you because she doesn't want to upset you,' Jane explained.

As the progeria progressed we noticed changes in Hayley. Her arthritis worsened to the point where we saw she was unable to straighten her fingers or bend down to put her shoes on. Like an old person, she needed special adaptations around the house to help her lead an independent life.

Jane would sometimes take her out for the day to give Mark and me the chance to meet with an occupational therapist in order to a.s.sess Hayley's ability to do daily tasks and to recommend adaptations to make her life easier. There was a high ledge leading from the patio door down on to the garden, which was too difficult for Hayley's little legs to climb over. A step and handrail were fitted so that Mark and I no longer had to carry her out into the garden. As she started learning to wash herself and brush her teeth, we had special lever taps fitted in the bathroom so that her frail hands could turn them on. Another of the special aids she needed was a cushion for her chair at the dinner table. We had noticed she was developing a red bed-sore-type mark on the top of her thigh from sitting in one position for a long time. It was nothing for her to sit at the dinner table for an hour at a time at meal times and sores were developing. The therapist suggested a special cushion, such as they give old people, might help, but that was easier said than done. Her stubbornness turned this into another battle of wills and again I was grateful to Jane who managed to convince her it would be a good thing for her.

Her stubborn streak was not always easy to deal with and could reveal itself at the most inappropriate times. Following the broadcast of Hayley's first Extraordinary People doc.u.mentary in the UK, a TV company from j.a.pan came to England to spend a couple of weeks following our family around for a j.a.panese doc.u.mentary called Precious Life. At the time we were happy to take part and spread the understanding of progeria across Asia. We were not aware of any j.a.panese cases and thought that if Hayley's programme could help one j.a.panese family identify the condition in their own child, just like it had with the Vandeweert family in Belgium, it would be worthwhile. Two years later the company wanted to come back and follow Hayley's progress. At the time I asked Hayley if she was happy to do another programme and she was keen on being big in j.a.pan once again. But as the day of the j.a.panese crew's second visit drew nearer, Hayley started to get sulky. I couldn't work out what was upsetting her. When I tried asking her what was wrong, all I got was 'nothing'. With a little help from Jane I managed to get to the bottom of the problem. I felt like I was pa.s.sing the buck once again, but I knew Jane would be able to root out the problem if there was one. Hayley admitted to Jane she was not looking forward to the j.a.panese TV company coming back.

'They are bossy and I get tired,' she told Jane. I was shocked and a little upset to hear this, it seemed like I was forcing her to do something that was obviously upsetting her. 'But she seemed quite keen when we asked her,' I said in my defence. Jane thought Hayley had been telling us what she thought we wanted to hear rather than what she really felt. I was devastated that something which seemed so straightforward had caused her this anguish and didn't want to make her do anything that she was not happy with. On the other hand the j.a.panese crew had already booked their flights and scheduled the film. With this in mind I called them and came to a compromise filming will be less obtrusive than last time and their filming sessions will be shorter. Everyone seemed happy, the film was made and there were no more sulks from Hayley.

Chapter 9.

Hayley

Doctors and Days Out

LIVING WITH PROGERIA IS hard. People treat you like you are a baby. I want to shout at them, 'I'm not a baby.' They say I can't do stuff like run around properly and Mum worries that if I run I will fall over and hurt myself. It's really annoying.

The worst thing about having progeria is all the blood tests and needles and stuff. Ever since I was little I have had to see lots of different doctors. My favourite is Dr Whincup. He is really nice. Whenever I see him he gives me a big hug. He's not like a doctor although he talks like one and says really long words. But he explains the long words to me and I like that. Because he's more of a heart doctor, he doesn't do things that hurt me like taking blood.

Dr Whincup does something called ECGs, which is when I have jelly put on my chest so that he can watch my heart on TV. Before I have the ECG I have to lie on the bed in Dr Whincup's room. He always says to me, 'I bet you can't leap up onto the bed.' And I always tell him, 'I bet I can.' I have to run and jump as high as I can because the bed is very high. But I never quite make it and Mum or Dad have to help me up. Once I'm on the bed Dr Whincup's nurse helps me undo my blouse and puts the jelly on my chest, then she puts wires on my chest. The jelly is cold and slimy. Then Dr Whincup points to my heart on the TV and so I can see it beating.

When I first saw my heart on TV it looked really weird. It wasn't like the TV at home; it was black and white and fuzzy. Dr Whincup always lets me look at the pictures. He says things like, 'Your numbers are good and your pictures are good,' which makes Mum and Dad happy. And he always winks at me. I used to think he was called Dr Whincup because he always winks. When it's time to go home Dr Whincup always kneels on the floor and shakes my hand and says, 'See you in six months.' I hold on to his hand and shake and shake and shake. I keep shaking his hand and won't let go. It's our little game. He always laughs and says, 'You can let go now.' It's fun. Sometimes I think if I didn't have progeria I would never get to meet cool people like Dr Whincup.

Whenever I had to see a doctor I would have the day off school. Some days I was allowed to stay home from school because my friend Jane was coming to see me. Jane was a grown-up but she was like a best friend, like Erin and Maddie. When she came to see me we would do lots of fun things like cooking and making things. Sometimes she would teach me how to cook. I remember one time we made a pizza for Mum and Dad's tea. She asked me what topping I wanted to put on it. I said ham and mushroom because I knew Dad liked mushrooms and Mum liked ham. Then Jane took me to the shop to buy all the things we need to make it. I got to push the trolley. 'Don't crash,' Jane said. But I did. I pushed the trolley into the shelf with tomatoes and it went bang. Then I got stuck and Jane had to help me move it back out. I found the mushrooms but I couldn't reach the scale to weigh them, so Jane had to lift me up. When we got back home Mum and Dad were in the living room talking to a woman who was writing things down on paper. So Jane took me into the kitchen and we made our pizza. It was weird because quite often when Jane came to see me there would be strange people with Mum and Dad. I never knew why. When the pizza was ready Jane helped me to put it on a plate and I carried it into the living room for Dad. The writing lady had gone. Dad said it was the best pizza he ever tasted and I felt so proud.

Another time Jane took me swimming. On the way to the pool I was sitting in the back of Jane's car in my special car seat. Jane said to me, 'Is your leg hurting? Maybe you need a softer seat?' I didn't know how she knew my leg was sore because I hadn't told her. I thought that Mum must've told her because when was bathing me I cried when she was washing my legs and she noticed it was red. Mum said I had a bed sore, which I thought was odd because I was in the bath not bed.

'I don't care if my leg is sore,' I told Jane.

'But I care,' said Jane. 'I don't want you to get sores. It might stop you going swimming and doing other things that you enjoy.' I thought about it. I liked swimming. Dad would take me every week for therapy and I didn't want to stop going just because I had a sore.

'OK I'll have a cushion,' I said. Soon after that I got a new cushion to sit on when I was having my food at the table. It wasn't very comfy, but at least it stopped the sores and I didn't have to stop swimming and good stuff.

I used to tell Jane my secrets and things that I didn't like to worry Mum about. She once asked me if I had friends in school. I told her I was very popular and had loads of good friends. But I said I didn't like it if everyone is like, 'h.e.l.lo Hayley.' 'It's hard work being a star,' I told her. Jane said it's OK to be sad sometimes. I said 'Everybody has to be sad sometimes.' Jane was pleased when I said that. But I wasn't sad I was just saying it.

My favourite toy when I was younger was my Wendy house. My Nanna and Pops made it for me as a surprise. Mum and Dad took me away and when we came back there was a house at the bottom of the garden. It was just like a real house but it was made of wood. It had pink curtains on the windows, pink fluffy cushions on the chairs and a sign saying Hayley's House. Mum always used to be afraid that I would fall and hurt myself but she liked me playing in my Wendy house because she knew I was safe. I used to pretend it was my princess castle. Whenever my friend Maddie, who also had progeria, came to play with me we would go in my castle and pretend to be princesses. I would be Princess Hayley and she would be Princess Maddie. Now I am older I don't play in my house any more because it's for babies.

Chapter 10.

Kerry

Grandparents Equals Spoilt Rotten

THERE IS ALWAYS A special bond between grandparents and their first grandchild, but when that child has a terminal illness the connection is stronger. From the moment Hayley entered the world my mum was beside me in the delivery room with her camera, taking photos of little Hayley from all angles. I remember one of the nurses turned to her and said, 'You would think she was a princess.' To which my mum replied, 'She is a princess to me.' And that has pretty much summed up Hayley's place in her affections from day one.

Throughout my first months as a new mum, Mum was by my side giving me her support and first-hand advice. And when I was at my most suicidal after the progeria diagnosis, it was Mum who watched over me and gave me the verbal shake I needed when she told me, 'Don't be selfish, think of Hayley.'

Hayley was not just her granddaughter, she was almost like her fourth child. For the first years of her life I had spent many weekends with Mum and Dad. Technically speaking Derek was my stepdad as Mum had married him when I was four and my sister Janie was three. But we had never had any contact with our biological father and Derek immediately became the father figure that was missing in our family. When Mark and I were having trouble with our relationship, I would run home. And even when things were good between us Hayley still spent every weekend at her grandparents.

Nanna and Pops were the most important people in Hayley's young life and she was the centre of their world.

It became our weekly ritual. On a Friday afternoon Hayley would wait for Pops' van to pull up so that her weekend adventure could start. Dad works as a building foreman and as soon as she saw his flatbed truck driving down around the corner she would grab her bag and stand at the door waiting. A weekend at Nanna and Pops' house usually consisted of baking cakes with Nanna or gardening with Pops. Mum worked as a school cook and was an excellent baker, so at the end of the weekend Hayley would come home with flapjacks, cookies, gingerbread men or chocolate cakes. Other times she would go shopping for clothes, knowing Nanna would indulge her and buy her anything she wanted. I remember on one occasion Hayley was so excited when Nanna bought her a bright pink strapless dress with a tutu-style skirt.

'It's hideous. I tried to persuade her not to buy it,' Mum apologised to me. 'I told her it was too big, but she said I'll grow into it. It's so big, she looks like a meringue on a lollipop stick.' Even against her better judgement, Nanna could never say no to Hayley.

In the garden Hayley would help Pops to plant bulbs or feed the koi carp in the fish pond. Like her grandparents, Hayley loved all flowers as long as they were pink. But they never grew quickly enough for her and she would always check for growth on a daily basis, only to be disappointed.

Nanna was like a second mum to Hayley. She was just as protective as me and equally sensitive to the comments and stares of strangers. Whenever they took Hayley out on day trips to the beach near their home strangers would stop and say, 'Isn't she lovely.' Mum would always agree with grandparental pride. But by the time Hayley reached the age of four and the baldness and thin, frail features of progeria became more obvious, Nanna became more sensitive. 'It makes me so angry, when people stare at her,' she would say to me. 'It happens all the time and I want to shout at them.' On one occasion a middle-aged couple stopped them as they were walking along the seafront at Dymchurch, and said, 'I hope you don't mind us asking, what is wrong with your little girl?' Mum explained all about progeria and how Hayley was ageing before her eyes. It was an education for both sides. Mum accepted that Hayley did look different and she could never stop people looking and the couple, who were brave enough to ask, had a better understanding of Hayley's condition. In public Mum managed to put on a brave face, but in their private moments, she worried about Hayley's future. This was particularly difficult when it came to bath time. Hayley was a real water baby and loved having a bath every night before bedtime. She would stay in as long as she could, asking for more warm water when it started to cool. For seven years Mum had worked at a care home bathing frail and elderly people every day so she was used to seeing the paper-thin, easily-bruised skin and over-sized arthritic joints on 90-year-olds. But it was hard to accept on her young granddaughter.

As soon as she was old enough Nanna and Pops converted their spare bedroom, which had been my old bedroom, into Hayley's room. They painted all the walls pink, hung pink curtains at the windows and a Piglet duvet cover on the bed. All her toys were stacked neatly on shelves and in baskets. Yet she never slept there. She would always insist on sleeping between them in their bed. Eventually they had to give in and put a mattress on the floor next to their bed at night. These bedtime intrusions turned out to be quite useful, while snuggling together in bed watching TV, Hayley would often let Nanna in on her secrets.

On one occasion, shortly after she had started school, I was getting worried that she was not eating her packed lunch. Her lunchbox was always empty when she came home, but her weight was still low and I was concerned that she was not getting the nutrition she needed. When she was at home I could see what exactly she was leaving on her plate, but in school it was difficult to monitor. One night while they were tucked up in bed, Nanna dropped the subject of school and food into their bedtime chat. 'Are you eating your packed lunch?' she asked, slyly. Hayley whispered back, 'Sometimes I give it away, but you mustn't tell Mummy, she thinks I eat it all.' With this information I was able to speak to Hayley's teachers and get them to be extra vigilant at lunch times.

Nanna also came to the rescue on the rare occasion when bullying threatened to become an issue. 'Some girls in school keep calling me names, Nanna,' she confessed.

'Take no notice of them they are just jealous,' Nanna replied.

'What do you mean jealous?'

'Well, you have so many friends and you are so pretty that they are jealous they are not like you.' With this idea in her head, Hayley was happy and bullying never really became an issue in her life.

When the all-important question of death reared its head, it was Nanna who saved the day. 'She's asking me what happens when I die. What do I tell her?' I said to Mum when we were alone. With hindsight it was the typical inquisitive question that five-year-olds have. Both Ruby and Louis would later reach the same stage in their development, when the question of life and what next is important. But with Hayley it was a more sensitive subject.