Old Before My Time (7 page)

Chapter 6
Kerry
Starting School

H
AYLEY'S FIRST DAY AT
Sidley Primary School was something I had never dared to dream of. When we were first told that Hayley's progeria was terminal, I had rather naively assumed that Hayley would not live long enough to reach school age, yet there I was on September 4 2001 ironing her tiny uniform and making sandwiches for her big day.

As I laid out her freshly pressed blue and white checked pinafore dress and waited for her to wake up, I suddenly felt a lump in my throat. I was scared for her. For the first four years of her life Mark and I had been by her side every second of every waking hour. We made her feel like the most special person in the world because she was, in our world. We had held her hand and told her what a brave girls she was being when she cried as doctors dug needles in her looking for blood. We had lifted her up to reach the handle of the back door when she wanted to go outside to play. We had shielded her from the stares of the ignorant and the inquisitive. Now we had to hand her over to the school, and although I was sure that the head and the other teachers would do their best to protect her from bullying and educate the other children, I was still nervous about handing over responsibility.

Three months before the start of term we had attended an open day where Hayley met her new teacher Mrs Haines and some of the other children who would be in her class for the new term. It had all been a big adventure as she played shop with the other children and painted pictures. I remember how at the end of the day she had run to meet Mark and me waving a paper plate covered with glue and strands of black wool and crayoned eyes and mouth. ‘This is Stacey,' she announced handing her artwork to Mark. ‘She's been ever so good,' Mrs Haines reassured us.

In the months that had passed between the open day and the start of new term Hayley had already aged the equivalent of two years but she hadn't grown a centimetre. Shopping for her school uniform was a challenge. How many two-year-olds need navy school cardigans? Most of the uniforms Mark and I could find started at age 4–5 and they were swimming on her. The best we could find was a white polo shirt for a four-year-old. When we tried it on her, it reached down to her knees.

‘I can tuck it in, Mummy,' she said stretching out her arms as the short sleeves reached past her tiny little elbows. She looked like she was playing dress up in her big sister Stacey's clothes. Finding a dress to fit was just as difficult. Again we had to buy the smallest we could find and even though sewing was not my strong point I turned up a large hem so the skirt wouldn't drag on the floor. Buying shoes to fit was equally challenging. Up until now she had trainers and little sandals as her feet were narrower than most children's. Finding black patent leather shoes to meet the school regulations was hard work.

‘You look like a moon man,' Mark joked with her as she walked around the shop in boots that were way too big. When the shop assistant measured her feet, they were size 3 with a narrow e fitting. Eventually we found a pair of tiny black leather shoes with a T-bar which she declared were ‘a gorgeous fit' as she stomped around the shop like a supermodel showing off her new footwear. ‘Gorgeous! Gorgeous! Gorgeous!' she announced to the amusement of the shop assistant and other customers.

To prepare Hayley for school we had to explain to her why the progeria made her look different to other kids.

‘When I get older and lose my progeria, will I have hair like Stacey?' she asked. I cuddled her and said, ‘I hope so,' and crossed my fingers tightly as I said it. How could I tell her the truth that she will never grow old or grow hair unless doctors come up with a miracle drug?

The morning of her first day at school, Hayley was full of beans. She skipped into the kitchen in her fluffy pink dressing gown. I made her favourite breakfast: scrambled egg. Then she washed and brushed her teeth and I helped her get dressed in her new uniform. As I buttoned up her blouse, hitched up her skirt and helped her buckle her shoes, I felt so proud. For the finishing touch, I tied a navy blue bandana to match her cardigan around her head. She had lost all her hair when she was three and it had never really bothered her. But starting school with other, ordinary kids had made her more self-conscious and she refused to go outside without covering her bald head. She had started wearing pretty pink bonnets and baseball caps when we went out. But for school I had bought her a selection of her trademark bandanas.

In the kitchen her Powder Puff Girls lunch box was waiting, packed with a chocolate spread sandwich, a packet of crisps and her favourite Pink Panther wafer. I hoped it would be enough to maintain her energy levels throughout the day. With Louis in the pushchair, Mark and I walked her to school, followed by a crew of TV cameramen who were still making the Channel 5 documentary. Being so young Hayley thought it was perfectly normal to have an entourage following you around.

At the school door Mrs Haines met us and we waved goodbye. I tried hard not to cry. There were other first-time mums in the yard, nervous for their children's big day, but I had more reason to be afraid. Hayley already had the body of a 32-year-old. One little bump and she could break her arm.

At regular intervals throughout her primary school life Mark and I met with Hayley's teacher for progress reports and to share any worries. We were concerned that she might be falling behind in her school work due to the number of days off for hospital appointments. We also wanted to ensure that she wasn't getting bullied. She was such a cheerful child, I thought I would be able to spot the signs if she was being called names. Her personality and childhood innocence had protected her so far but her insistence on covering her head in public made me think another child had said something to her.

Mrs Haines told us that she was not lagging behind in her school work. ‘She does have more time off than most of the other children but she works hard to catch up. And she is popular with the other children because of her sunny nature: she brings out the best in the others.'

‘Has she been bullied because of her bald head?' I asked. ‘Having no hair has never seemed to bother her until she started school. Now she refuses to go out without her bandana? Have any of the other children said anything to her?'

Mrs Haines told us there had been a ‘conversation' with some of the other children who asked her why Hayley doesn't have hair. They wanted to know if she will ever grow hair. She then explained how the class had been given a lesson about differences, where she explained how we are all different: some of us have brown hair, some of us have blue eyes, some of us are tall, Hayley has no hair and we have no choice over these matters.

I was satisfied that the school would do everything they could to protect Hayley from cruel name-calling. And her academic progress was good. I just wished that there was something that could be done help her progeria. It frustrated me that no matter how hard we tried to make Hayley's life better and spread the word about her condition, there was nothing we could do to further medical advances.

‘There ain't no cure for old age,' Mark would say in his philosophical way. And he was right up to a point. He said that it would be a waste of money to spend millions of pounds in research for a condition that affects so few people when there are millions of people dying of cancer or AIDS every year. But that was no comfort to me. Some days when Hayley complained of joint pains we would be annoyed and ask ourselves, ‘Why more can't be done to research this terrible illness?'

While Hayley was learning her ABC and numbers at school, Mark and I were learning more about the progerin protein which causes progeria. We heard about the Geno Project, where scientists were trying to find the bad gene that causes progeria. They had already made progress by cloning the progeria gene which they had in a database alongside all the other genes that make up human beings, but they hadn't yet worked out how to find it. They reckoned it was a lot like hunting a serial killer. They knew the killer was out there but they didn't know what it looked like. We lived in hope that with humans living longer and more people reaching old age, these scientists would find a cure while Hayley was still at primary school.

Chapter 7
Hayley
No Hair, no Nits

T
HE 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 class. ‘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 class 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 class 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, ‘Hello, 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, ‘Hello, 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

W
ITH EACH PASSING 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 reassured 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 attitude 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 blasé, 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 passed a secret on to Jane, it was safe. And Jane was able to give us the reassurance 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 assess 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
documentary in the UK, a TV company from Japan came to England to spend a couple of weeks following our family around for a Japanese documentary 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 Japanese cases and thought that if Hayley's programme could help one Japanese 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 Japan once again. But as the day of the Japanese 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 passing 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 Japanese 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 Japanese 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.