Old Before My Time

by Hayley Okines

  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 cocktail 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 Institute 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 wh
eelchairs 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

  Hayley

  Just Call Me Robo-Chick

  EVERYONE TELLS ME I’M brave. And when I go to hospital I try to be brave. But when I’m not in hospital I’m a big wimp. I’m even scared of spiders and the dark.

  When Mum told me about the new drug trial, that had to be injected, I wasn’t very keen on the idea. I don’t like having needles but I thought, if it will make me better, then I have to do it I suppose. I asked if they had tablets I could take instead of having an injection but they didn’t. The injection was a bit scary, I was worried in case anything went wrong. First I had to have a little needle stuck in my arm and taped over, then they put another needle with the drugs into it. The needle hurt when it went in the first time and I felt a bit sick. I had a tube coming out of my arm which went to a machine with numbers on it. I couldn’t really feel the bisphosphonate going into my body. It was going so slow, it made a snail look like a cheetah. I had to lie there and do nothing for ages. Then I had to take some medicine because they said I might have headaches, or achey bones and stuff, but all the time I have been taking the drugs nothing has been wrong. I take them to make sure. If they say it will make me better I might as well take it, that’s what I think.

  When I dislocated my hip the first time, Mum said I was really brave because it hurt so much. It hurt even more when I did it the second time but I wasn’t so scared because I knew what to expect. It happened again when I was in the kitchen with Mum. I asked if I could jump on a washing machine tablet to burst it. I stomped on it and it didn’t pop so I started jumping on it with both of my feet and I twisted wrong and my hip popped out again. My mum called an ambulance. I just thought, here we go again, while mum was crying. I was in so much pain it was horrible.

  The third time it happened I was having a sleep-over with Erin and my cousin Sophie. Me and Sophie were lying on the bed and Erin was on mattress on the floor. I was doing Sophie’s make-up but I couldn’t see very well because it was a bit dark. So I went to open the curtains and I twisted on the duvet and I felt my leg pop out. We had to cancel the sleep-over and I had to go to hospital again, which was bad.

  After that the doctors said I should wear a brace. I said, ‘No way!’ It was a big ugly metal thing that they said I would have to wear for six months. It looked really uncomfortable and I didn’t want to have it. Mum said that maybe there was an operation I could have but I would have to stay in intensive care for a week and stay in hospital for a while. Then I would have to stay in bed, but I didn’t want to do that either. Anyway the doctors put my leg back in and I went home.

  I was happy when Mum said I didn’t have to go to school because of my hips, so I could do my work on the computer instead. I had to do maths, English, science and citizenship and it was much better than having to go to school. After school Erin would come round to my house and we’d catch up on all the gossip so I didn’t feel like I was missing anything. Whenever Erin comes to my house she likes watching the Disney Channel because she says her mum can’t afford it at their house. We watch Hannah Montana and sometimes we watch the Music Channel but not much. Other kids in school like the Music Channel but I think music videos are boring – unless they are by Justin Bieber. I like cartoons better, they make me laugh.

  The last time I dislocated my hip happened I was walking up stairs with my dad. I was three steps from the top when I caught my toe and twisted funny. I didn’t even fall because Dad caught me but I felt both my left hip and my right one pop out. I could tell that Dad was scared because he had never seen me do it before. He tried to lift me up but he couldn’t hold me under my bum like he usually did because it hurt too much. So I had to put my arms around his shoulders and hold on tight and he held my body and carried me into the bedroom. I said to him ‘I think I’ve done the other one.’ I started to cry because I was so uncomfortable. When I hurt my left hip I could lie on my right one so I could ease the pressure. But I couldn’t lie on either side because it hurt so much. So I had to lie on my back. I was crying. Louis and Ruby were crying too because they were scared for me. They were both really helpful. Ruby watched out for the ambulance and Louis was rubbing my head and my hand while we waited for the ambulance.

  When we got to the hospital I had gas and air again. I love that stuff. It doesn’t smell or taste of anything but when you breathe in it makes you feel like you’re away with the fairies. I took three puffs and I started giggling. Just before I went under the nurse was holding my hand. Dad took Ruby home to fetch my pyjamas and Mum and Louis stayed by my bed. Louis was so sweet as I was going down to the operating theatre he was holding my hand and rubbing my head and it made me cry.

  After I came around from the operation Louis was really scared for me. I said, ‘Don’t worry, it won’t happen again.’

  That’s when the doctors said I had to wear the dreaded leg brace. It’s horrible. It has a big thick metal belt that goes around my waist and metal rods that are strapped around my legs. Mum calls me ‘Robo-Chick’ because I look like a robot when I am wearing it. The only good thing I can say about it is that it is purple and that’s my favourite colour. The first time I only wore it for four hours and then I took it off and said, ‘I can’t wear that.’ It was too uncomfortable. I am supposed to we
ar it for six months, 24/7. The only time I take it off is when I have a bath or shower and I’m not even supposed to do that. It’s really annoying. I thought it might be a bit comfortable because it had purple padding around it, but it’s not. When I sit in the car I can’t even lean back because it sticks in me and hurts. When I am lying in bed it feels horrible and I can’t move. If I want to go to the toilet in the night I have to wake Louis up and ask him to take it off for me, because I’m not supposed to. In the beginning I used to wrap my cardigan around it to hide it when I went out because it looked so bad. I’m stuck with it until my hips get better. I suppose it’s better than being in a wheelchair or intensive care or something. I’m getting used to it now, I don’t care so much. I’ve found that I can tuck my iPhone into it so it has its uses after all. But I am counting down the days until the time I can take it off for good and my legs will be better.

  Chapter 28

  Kerry

  Pressures of Progeria

  THEY SAY THAT DIVORCE, debt and moving house are the most stressful things in life, but I bet the people who said it have never tried to organise a holiday for progeria families. When the Sunshine Foundation charity, which had organised the American reunions for the previous 27 years, announced it had to cancel the 2010 reunion due to the worldwide recession, the drop in the number of charity donations and the spiralling costs of making it happen, Mark and I decided to step in.

  ‘The reunions have been a lifeline for so many families like us. It would be such a shame for the children if they stop for ever,’ Mark said when we first heard the news. So in a moment of what I can only now call utter madness, I said, ‘Let’s organise our own.’ For years we had said how great it would be to organise a reunion in England, now we were putting our talk into action.