Old Before My Time

by Hayley Okines

  Throughout the day I counted the seconds between the contractions as they got shorter. I ran myself a hot bath to ease the discomfort. At 7 p.m. – just as we were sitting down to a takeaway kebab – the contractions changed.

  ‘I think it’s time for hospital.’ Mark helped me into the back of his red Ford Sierra and drove me five miles to the maternity unit at Hastings Conquest Hospital.

  I will never forget the moment – 2.50 p.m. on Wednesday December 3 – when Rita the midwife laid my baby on my tummy. Just days before I had gone into labour I had a dream about our baby. I imagined I was being handed a bundle of blankets and all I could see was one big blue eye, nothing else. Now as I looked down, my head still drifting away on the large doses of gas and air, I saw just one eye, bright blue and sparkling like an aquamarine gem, looking up at me. It lit up the room. She reached out to touch the air with her perfect little fingers and I immediately felt relief. The pain was over. Our little girl was healthy.

  I looked at Mark, who had stayed by my side throughout the entire delivery and said, ‘Don’t it look like she’s got highlights?’ Her head was plastered with a thick mop of brown hair.

  ‘Hello, Hayley,’ Mark whispered. We had already decided we would name her after the comet Hale Bopp, which had been streaking across the sky on the night that she was conceived.

  She was absolutely beautiful. Weighing in at 6lb 6oz, she was perfect. As we cooed over her, a smile broke out across her elfin face. It was the first of many.

  ‘She’s gonna be a cheeky one,’ Mark warned, ‘she’s got your wicked glint in her eye.’

  I would have punched him playfully but I didn’t have the energy. After a 40-hour labour I was knackered but I was also the happiest woman in the world.

  Chapter 2

  Kerry

  Am I Being Paranoid?

  I WAS ON A permanent high for weeks after Hayley was born. Mark and I moved into a new phase in our relationship, taking on the role of new parents. Charlotte and Stacey were thrilled to have a little half-sister to care for and would help me to change her nappy and nurse her to sleep.

  Some new mums always complain of being tired, but not me. I seemed to have more energy than I had ever had before and I had lots of support from my family, who were equally besotted with our new addition to the family.

  Late one night, just as we were settling down for bed, there was a knock on the front door. We were not expecting visitors so late in the night. When I opened the door, my mum was standing there. ‘I just had to see her again,’ she said, having made a 90-minute trip from her home in Kent. Little Hayley had cast a magical spell over us all.

  Hayley was what the midwife called a ‘grazer’, which meant she would eat little and often. As I was breastfeeding it meant I was getting up every two or three hours during the night to feed her. But I didn’t mind. As the weeks passed, I started to worry that Hayley was not gaining weight. She could still fit into the little makeshift gown that she had been dressed in when she was born because she was too small to wear the hospital-issue baby-grow.

  Every week I would take her to the baby clinic and every week there was little change. When the health visitor lifted her on to the scales she looked like a plucked chicken with her bony legs dangling from her chubby little body, but the scales hardly moved no matter how much she ate. Being a first-time mother I had bought all of the ‘new mum’ books I could, and I followed the baby growth chart religiously. By my reckoning Hayley should have been gaining a pound or two every session, but she was only registering an ounce or two. Instead of a healthy arc, Hayley’s growth seemed to be flat-lining. I started to dread our appointments at the clinic. I was feeling paranoid. What if the nurses thought I was neglecting our child?

  ‘Maybe I’m feeding her wrong,’ I moaned to my mum, who assured me that I was doing everything by the book. Soon I switched to feeding her baby formula milk, after my health visitor suggested my own milk might not be rich enough. But even that did little to tip the scales.

  One morning as I was changing her nappy I noticed she had two little lumps either side of her belly button. ‘It looks like she’s got a hernia or something,’ my mum suggested. I made an appointment with my local GP, but he dismissed it as ‘nothing to worry about’. I got the feeling that they thought I was being an over-protective first-time mother, that it seemed like I wasn’t satisfied with a healthy, happy baby and I was looking for trouble. But I couldn’t ignore the fact that Hayley was very small for her age.

  By the time she was six months old, Mark and I had convinced ourselves that something was seriously wrong.

  She was still wearing newborn baby nappies and clothes for a 0–3-month-old. But the doctors still didn’t share our worries. I was beginning to think perhaps I was being paranoid. By her first birthday the doctors started to show concern too. Hayley was still pitifully underweight. The lumps next to her belly button were still visible and the skin around her tummy was tight and thin, almost as if there was cling-film wrapped around her. Call it a mother’s intuition, but I knew there was something not quite right. Eventually my GP agreed to refer us to a consultant at London’s Guy’s Hospital.

  Over the following months Hayley had test after test. Every couple of weeks Mark and I would take Hayley on the 150-mile round trip from our home in Bexhill to the centre of London for our appointments. At first Hayley thought it was a big adventure, she would sit on the train with her colouring and counting books. Over the months she became a human pin-cushion as samples of her blood were sucked out of her and sent off for analysis. They even tested her chromosomes, but they could not find anything to cause concern. At one stage they thought she might be suffering from cystic fibrosis. At that point an alarm bell rang in my head as I remembered a boy who lived in our street and suffered from the condition. We were told he would be lucky to see his 14th birthday as there was no cure for cystic fibrosis. I always remembered how he struggled to breathe and was continually coughing up thick green mucus from his lungs. Being a school-kid at the time I recall I found this particularly disgusting. The doctors told us that cystic fibrosis was not uncommon in children and on average five babies a week were born with it. It was terrifying to think that Hayley could be suffering from a life-threatening illness. I thought she might never live to reach her twenties and would spend most of her young life struggling for breath, and having to go through a daily regime of physiotherapy to clear away the mucus that would clog up her lungs.

  When the results for the cystic fibrosis test came back negative I thought, Phew! At least we don’t have to deal with that. With this ruled out, Mark and I had a new glimmer of hope. We were referred to specialists at Great Ormond Street Hospital. At the time it did not register just how serious the situation could be. Over the years I had watched enough documentaries following the work of the Hospital for Sick Children, its young patients and its groundbreaking research into serious and unusual illnesses to know that it was one of the best children’s hospitals in the world. Mark and I hoped its specialist doctors would be able to give us the answers we needed and treat whatever was stunting Hayley’s growth so we could get on with our lives.

  At Great Ormond Street we were referred to the department headed by the late Professor Robin Winter, a clinical geneticist whose field of expertise was dysmorphology (using a patient’s outward appearance to diagnose rare and unusual genetic syndromes). His colleague Dr Sheila Mohammed, an Asian lady with an air of calm about her, arranged a skin and hair biopsy. Taking a sample of hair was easy, but the tests required that they cut a piece of skin from Hayley’s left arm. She was given an injection to numb her arm. I think this was probably the start of her phobia about needles. What followed next was too awful to watch. Hayley sat on my lap as Dr Mohammed brought her scalpel up close to Hayley’s arm to shave off a sample of skin. Hayley screamed and squirmed trying to get away from the scalpel. I was powerless. I knew it was something that had to be done but I couldn’t stand to watch. Eventually I had to sta
nd outside the doctor’s room as three nurses held her still so Dr Mohammed could take a sample.

  ‘We think Hayley may have a rare genetic condition called Hutchison-Gilford Progeria,’ Dr Mohammed said at our next consultation when the results of the skin biopsy came back. ‘Her skin has an alarming lack of elasticity for a child so young.’ Writing the word progeria on a page torn out of her notepad, she told us to go home and do some research.

  ‘We can’t be certain at this stage. We are still waiting for some of the tests from her skin biopsy. But we could be looking at a rare ageing disorder.’ As we left the consulting room, knowing it would be two weeks before our next meeting, Dr Mohammed warned us, ‘Be very careful what you read about progeria. Not everything on the internet is factual.’

  On the car journey home, Mark and I looked at each other baffled.

  ‘Pro-jee-ree-ya?’ I said, carefully spelling out every syllable. ‘I love watching medical documentaries on the telly, and I’ve never heard of that one before.’

  ‘It sounds like something from a science fiction film,’ he suggested. All the way home we wondered what this ageing disorder could mean. What did it matter if Hayley’s skin wasn’t as springy as other toddlers? I pinched the back of my hand and watched my own skin snap back into place. I reached out to touch my baby girl, who was strapped into her car seat watching the world fly by through the car window. Now the thought had been put in my mind, her skin did seem different.

  But did it really matter? At almost two years old Hayley was already the centre of our world. She was smaller than other toddlers in play group but what she lacked in size she made up for in intelligence. She was advanced for her age. She had taken her first steps at 10 months old. By the time she was 18 months you could hold conversations with her. She knew the colours of the rainbow and could count to 10. She definitely seemed much wiser and more inquisitive than other 20-month-old toddlers.

  When we got home we shot around to see our friend Andy who was the only person we knew who owned a home computer. We tapped the word progeria into the Ask Jeeves search engine, taking care to spell it as the doctor had written it down. Then we waited as the results started to appear on our screens. One by one photos of children who looked like little old people, flashed up on the monitor. There was one little girl with arms so thin, you could see her bony elbows through her skin. Her face was ancient and shrivelled. She looked like she was a hundred years old. She was just 12. And she had passed away.

  On another page there was an image of a little boy with a large bulbous bald head, laced with blue veins, and eyes as wide as a bush baby.

  We learnt that Hutchinson-Gilford Progeria Syndrome is a very rare disorder characterized by children ageing eight times faster than normal. The word progeria comes from the Greek, meaning prematurely old. Children with the condition experience growth delays in the first year of their life resulting in short stature and low weight. That could explain why Hayley was still low on her growth chart. The website described how children with progeria suffer from deterioration of the layer of fat beneath their skin. Characteristics of the syndrome were abnormally small faces, underdeveloped jaws, unusually prominent eyes, and sometimes small ‘beak-like’ noses.

  I stared at Hayley, who was asleep in her Moses basket, looking for these abnormalities that made the children look so strange and space-like. But she didn’t look like them. Yes she had big eyes, but so did I when I was her age. The website said that in the first year or two of their life Progeria children’s scalp hair, eyebrows, and eyelashes may become sparse, and veins of the scalp may become unusually prominent. Hayley’s hair was thick, tied up in her pretty pink headband, she looked like she had a fine head of highlights.

  As we read on we were horrified to discover that not only did these children look like old people, but they suffered the same ageing illnesses too: arthritis and joint stiffness, bones that broke easily and would not heal properly, ageing skin, tooth problems, and heart problems.

  But the words that really hit us hard were: Children with progeria die of heart disease or strokes at an average age of thirteen years.

  ‘The doctors must be wrong. Hayley doesn’t look like those children. Turn it off,’ I screamed reaching for the switch to power off the computer. I didn’t want to know any more. The thought that our beautiful daughter would not live long enough to become a troublesome teenager was more than I could take. We thought that this was every parent’s worst nightmare – knowing that our dear daughter would not make it to adulthood. I would never worry whether her first boyfriend would be good enough for her. Mark would never walk her down the aisle. My ambition had been to have a family of my own, but I wanted one for keeps. I couldn’t face the thought that Hayley might be snatched away from us as suddenly as she had come into our lives.

  Chapter 3

  Kerry

  Progeria Confirmed

  SEPTEMBER 30 1999 IS the day when I lost all hope that we would ever be a happy family again, when the ‘maybe nots’ and ‘everything will be all rights’ turned into ‘sorrys’. For Mark and I the two weeks waiting for our next appointment with Dr Mohammed at Great Ormond Street Hospital moved in slow motion. It felt like we were waiting for a death sentence to be passed on our little Hayley. We were stuck in no man’s land, scared at what our next visit to Great Ormond Street might bring, but daring to hope that some of the most senior specialists in the world might be wrong.

  ‘It will be all right,’ my mum said optimistically. ‘Don’t worry it won’t be that,’ Mark’s mum reassured us. These sentiments were echoed by all our friends and family as the day of our appointment drew nearer.

  ‘We’ve got more chance of winning the lottery than of Hayley having progeria,’ Mark mused when he found a statistic on the internet that said it affected one in eight million children. I knew what I would rather. No contest.

  The morning of the consultation, Hayley was her usual chirpy self. It was a crisp autumn morning as we left the house for our train journey to London. I had wrapped Hayley in her favourite pink leggings and padded pink anorak with fur around the hood. She looked like a cute little piece of candy floss and I wanted to eat her. While we sat in the waiting room, Hayley lay on the floor drawing.

  ‘Look, Mummy, a ’pider,’ she said handing me a red scribble on a scrap of paper. She had not yet grasped the word spider.

  ‘That’s a nice ’pider,’ I replied as Dr Mohammed called our names.

  The wait was over.

  After that everything became a blur. I barely remember walking into the doctor’s room, or registering the apologetic look on her face as she tried to explain how Hayley was ‘a very special little girl. One in eight million.’ It was confirmed she had Hutchinson-Gilford Progeria Syndrome, a genetic condition so rare that there were only 40 other known cases around the world and only one other child in the UK. It meant her body would age eight times faster than that of a normal human.

  ‘What’s the prognosis?’ I asked. To this day I still do not know why I asked that question. I didn’t even realise I knew such long words and I was certainly not prepared for the reply.

  ‘The life expectancy is 13.’

  At that point I collapsed on the floor, like someone had just pulled the carpet from under my feet. That was one piece of information from the internet that was correct.

  On our way back to the train station in the back of a London cab, Mark and I sat in stunned silence. It was all too much to take in. There was so much we wanted to know, but couldn’t find the words to ask. Dr Mohammed accompanied us on the taxi ride and as we parted company she gave us her home telephone number and told us to call any time.

  ‘I want to see my mum,’ was all I could say to Mark.

  Suddenly I felt like I was three years old again and I wanted my mummy to make things better. Hayley sensed there was something wrong. She sat completely still on my lap throughout the entire journey, rubbing my face and kissing my cheek. It was all I could do to choke back t
he tears, struggling to make sense of an illness that was so rare only one other child in the country was known to have it. To make matters worse there were no other parents to talk to about it, no support groups or other sufferers for Hayley to meet. We felt completely alone on the start of a journey that would have no happy ending.

  When we reached my parents’ house, Dad was in the garden and Mum was in the kitchen. I was unable to speak. I just cried. There was nothing to say. Hayley ran out on to the lawn to see her ‘Pops’ while my mother put the kettle on. As we sat at the breakfast bar, the silence broken only by my sobs, I looked out of the window and saw Dad standing by the fishpond. He was holding Hayley’s hand and pointing down to the fishies in the water to disguise the tears that were rolling down his cheek.

  They say that before you can pick yourself up, you have to hit rock bottom. For Mark and I, the months that followed saw us freefalling into the darkest of places. Most nights, once Charlotte and Stacey were fast asleep and Hayley was tucked up in her cot at the foot of our bed, Mark and I would lie awake till 3 a.m. asking, ‘Why Us? Why are we being punished?’ We had been honest and open with Charlotte and Stacey, who were now thirteen and eight – old enough to be told the truth. But we tried our best to hide the full force of our grief from them. It was as if Hayley was living under the threat of execution. We knew it would happen, we just didn’t know when. It could be tomorrow, it could be next week or it could be 10 years away, and that was the hardest thing to deal with. We had been hoping to be told Hayley had a rare condition and given a prescription for tablets to make it all better. But there was no cure and at that point it seemed there was no hope.

  It put huge stress on my relationship with Mark – we were so distressed that we took it out on each other. At night when the girls were tucked up in bed we would fight over anything and everything. Who had drunk the last of the milk? Why had Mark left his jacket on the sofa? Why hadn’t I washed the dishes? They were all trivial things that seemed so irrelevant in the bigger scheme of things. Sometimes Mark would come home late and I would yell at him, ‘Where have you been?’