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Lucy
A remarkable family turns the tables on a fearsome disease I B Y R O B E R T K I E N E R
Throug h out her third pregnancy Jan Be tts had w r r i e d o there was “something wrong” with her baby. As she’d told her husband Clive, “I don’t know what it is, but the baby just isn’t moving, isn’t kicking, the way it’s supposed to.” She was also ill more often than she had been with her previous daughters K ate and Emma. Clive, a no-nonsense software salesman, had brushed off his wife’s worries. “Don’t be daft, Jan,” he told her. “The docto rs said everything will be fine.” But she was still co n cerned. Call it mother’s intuition. On a bitter January morning Jan Betts lay on an operating table in Norfolk and Norwich University Hospital about to give birth by Ca e sa rean section. Cl ive, wearing a surgical gown and
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mask, stood at her side, holding her hand so tight she thought he might sprain it. While this was Jan’s third child it was the fi rst she had had with Clive. The chatter of nurses, midwives and the doctor filled the air. “Relax, Jan,” said one of the midwives as the doctor lifted her scalpel to slice t h ro ugh her lower abdomen, numbed by an epidural. A green surgical curtain prevented Jan from watching the operation. “We are almost there. Relax.” The surgeon carefully cut into Jan’s uterus. As her ba by was pulled fro m her womb, Jan heard a gasp. Then the room went eerily quiet. “What’s wrong?” asked Jan. “Is there a pro bl e m? What’s wrong with my ba by?” There was no answer. Clive could not believe his eyes. His d a u g h te was totally covered in rough, r rigid, yellowish mottled and cra c ke d skin the thickness of orange peel. Her eyes looked more like two small open red sores; her mouth, nose and ears seemed horribly malformed, her hands were tiny claw-like appendages. Clive was speechless, but knew he h ad to answer Jan. He took a bre at h . “There is a problem, sweetheart. There’s something wrong with the ba by.” The baby ’s condition—she was named Lucy—was so rare it took docto rs more than a day to identify it. It was Harlequin Ichthyosis (HI), a dangerous genetic flaw occurring in less than one in 250,000 births that causes the skin to grow 14 times faster than normal, forming thick diamond-shaped pl ates (hence “Harlequin”) that can cover the body like armour and cut off
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circulation. Lucy’s life expectancy, like the majority of Harlequin babies, was brief. Many died from infection or respiratory problems within days. Jan begged the doctors to let her hold her baby, but they could only allow her a moment to look. As she saw the misshapen child, swaddled in a blanket, Jan was overcome with emotion. I love you, she thought as her eyes filled with tears. We’ll always be here for you. Then Lucy was gone, rushed to the sa fe ty of an incubator.

and Clive, “Lucy’s skin could kill her.” Docto rs devised a strategy to keep Lucy alive: an emollient-rich bath twice a day that would soften her new, underlying skin and help her “shed” her older skin. When she was out of the wate r she would be kept coated in a thick, waxy moisturiser to prevent cracking. It wo rked. After five days a doctor recommended putting salicylic acid into Lucy’s moisturising cream; but this

with the two girls as she led them to the incubator that held Lucy. The girls s topped and stood staring through the plastic walls. Stretched out on a white bl a n ket was a tiny, raw-red, glistening fi gu re, attached to a network of wires and tubes. The girls watched as Lucy’s small chest rose and fell in tune with the re s p i rator. Jan held her bre ath as t h ey turned to look at her. “She’s beautiful!” they both said.

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ver the next few days it became clear that Lucy was a fighter. The doctors and n u rses, all on uncharted ground, gently peeled away the thick layer of skin that enveloped her. Incredibly, after it was removed her weight dropped from five pounds, three ounces to just three pounds. Standing in front of Lu c y ’s incubato r, Cl ive and Jan locked arms and watched their daughte r, not much bigger than Clive’s outstretched hand, crying in obvious pain. Th ey knew now that the disease was incurable. With her bright red, co n s ta n t ly flaking skin, Lucy looked like a burns victim. She had no eyelids, a frequent side effect of HI. Rapidly developing new skin was creating multiple threats to Lucy’s life. If the skin round her chest we re allowed to dry out it, it would shrink so much that she co u l d n’t breathe. If thick skin anywhere on her body cracked it could lead to infection, sepsis and death. As a doctor told Jan

If Lucy survived, would she be constantly stared at or cruelly teased in the playground?
penetrated her skin and she developed aspirin poisoning. Her lungs nearly failed and she te e te red near deat h . Thinking they we re losing her, Cl ive and Jan had Lucy baptised. Somehow, Lucy survived. “A fighter,” Jan called her. “She’s tough.” As well as the co n s tant thre ats to Lucy’s life, Clive and Jan faced another harrowing problem; Lucy’s appearance. As parents often do, they had immediate ly seen past their baby’s biza r re exterior to the normal child beneath. But how would others react? If Lucy survived, would she be constantly stared at or cruelly teased in the pl ayground? Jan and Cl ive were sure of one thing, though. Th ey would never hide their little fi g h ter away. The fi rst test of acce p tance came almost immediately. It was time to introduce Ja n’s two daughte rs, Kate, 11, and Emma, nine, to Lucy. Jan held hands By day six Lucy’s lips had beco m e less swollen and Jan could finally breastfeed her. The doctors told Jan and Clive not to get their hopes up. “Lucy’s not going any wh e re,” said Jan as she cradled her small, cream-covered daughter in her arms. “Except home with us.” But even as Jan spoke, she wondered how she would cope. There was only one other surviving HI baby in the entire country. She was in almost unknown territory as a mother. Just get on it with i t, she told herself as she cradled Lucy on her lap, while Clive drove them home f rom the hospital. They set about devising a daily ritual to keep Lucy alive. It was a gruelling ro utine that they ’d fo l l ow day in and day out as months passed, then years. ‘‘Time to get up, sw e e t i e”coos Jan as she turns on the light in Lucy’s small upstairs bedroom. It’s just after 4.30 in
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the morning. Lucy climbs out of bed and rubs the sleep from her eyes. As she does, bits of skin come off in her hands. Her entire body is covered with thick, flaking skin which has grow n overnight and is now shedding itself. Her sheets are full of skin that has peeled off as she slept. So is the floor, which Jan will later vacuum. D ow n s ta i rs in the bat h room Lu c y slips off her py jamas and climbs into the tub, which is filled with warm water and emulsifier. For nearly two hours she’ll stay in the bath to soften her skin.

a moisturiser. Lucy is creamed fo u r times a day. It is a daunting schedule, but it works. Lucy continues to thrive. Two years aft e r Lucy’s birth Jan and Cl ive had another child, Hannah, also born with HI. They now had double the work and worry. But they kept their promise of trying to bring up both girls normally. In public, people were curious but mostly considerate. I n ev i ta bly, there we re exce p t i o n s . O n ce, when Lucy was about two ye a rs old, Jan had her in a pushchair

she’d like for Christmas. “Mummy,” she said, “can I have new skin?” Jan wrapped her arms round her daughte r. “I’m so sorry,” she said. “That isn’t something I can get for you.” At times like this Jan would wonder: Is there a place in the world for Lucy with all her problems? Brought up to stand up for hers e l f, when Lucy was old enough to go to school she coped with the inevitable s ta res and occasional jeers from fe llow pupils. Once when she’d noticed people staring at her, she told Ja n : “They’re only looking, Mum. They just don’t know any better.” With the help of a care g iver who helped her change clothes at lunchtime and cream her skin several times a day, Lucy sailed through primary school.

If her skin is allowed to dry out, she can’t bend her fingers enough to grip a toothbrush
“Stand up, Lucy,” says Jan as she then begins the painful process of scrubbing her daughte r’s entire body with a flannel. As Jan reaches a particularly raw spot, Lucy cries out, “It hurts, Mummy!” Jan winces in sympathy, but both know that despite Lu c y ’s te a rs, she has to scrub off the skin to prevent cracking and infection. After half an hour of scrubbing, the bath-water surface is cove red with laye rs of skin. Lu c y ’s body now looks raw and sunburned. The second she gets out of the bath her skin starts drying out. Soon it looks as if she’s been cove red with cling film. If she isn’t cove red with cream i m m e d i ately her skin will dry out so quickly she won’t be able to bend her fingers enough to grip a toothbrush. Jan slathers her, head to toe, with Lipobase,
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at Debenhams in Norwich. A woman passed by, looked at Lucy and stopped dead in her tracks. She returned twice more to sta re. Jan lost her temper and shouted: “Well, how many times do you want to look at her!” In a Great Yarmouth shopping mall Jan heard a woman tell her friend, “Come here, you’ve got to see this!” “I wo u l d n’t do that if I we re you,” Jan told her. “That’s my daughter!” The woman apologised and hurried away. Although Lucy was cove red with cream during her every waking hour, she rarely co m plained. She had never known anything else. One day Jan noticed Lucy playing with her dolls. She had undressed them and was covering each with a smooth white cream. One December, Jan asked Lucy what

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ach of Lucy’s birthdays was ex t ra-special for Cl ive and Jan. Lucy was now one of the longest-living HI sufferers in the wo rld, so no one knew how long she’d continue to survive. As she ente red her teens, Lucy encountered another cruel challenge. Because HI caused Lucy’s eyes to dry out, her corneas were ge tting incre a singly scratched. By 12 she had lost all the sight in her left eye and had only ten per cent vision in her right. But Lucy was still a fighter. Instead of giving up activities such as cycling, she rode her bicycle even fa s te r, te r r i f ying her pa rents and friends. “Lucy knows life is for liv i n g ,” Jan said. “And that’s just what she does.”

Lucy made close friends at school, b ut never a boyfriend. After all these years she still had to get up at 4.30am for her painful ritual; soak in the bath, rem ove built-up skin, get creamed and get to school. Every day she missed part of some classes to change her clothes and re-cream herself. Because of its size, kids at her co mprehensive often ba re ly knew each other. Lucy was sometimes ta u n te d . “Hey, is that a sunburn or what?” “Get away, you’ve got cream all over!” The jibes hurt; but they also made her more determined to succeed. I ro n i c a l ly it was the low ex p e c ta, tions of some te a c h e rs that bothered her most. “You shouldn’t ta ke so many GCSEs,” said one. Another told her to forget about being a vet or even a beautician. “People would object to all that cream on you.” Another said she’d never find a job working with special-needs children, something Lucy longed to do. B ut adve rs i ty still brought out the fighter in Lucy, and Jan and Clive, who h ad always refused to spoil her, beamed with pride as they watched her struggling to re ad her textbooks with a powerful hand-held magnifier. In 2003 Jan waited nervously to hear Lu c y ’s exam results. “Hiya,” said Jan as Lucy came home after getting them. She was aching to ask Lucy how she’d done. Lucy, her expression grim, said nothing. Then she broke into a huge smile and announced: “I passed all 11 of my GCSEs!” By now Lucy was thinking of a career. More than anything she wanted to work with children who had special
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c h a l l e n ges, like herself or Hannah or m a ny disa bled friends she had mad e . To do that she’d have to cope with co l l e ge. Her te a c h e rs we re sce p t i c a l , b ut Lucy pl u n ged in. Last year she

Still smiling: Lucy with mum Jan and dad Clive at home in Great Yarmouth

co m pleted a two-year course at Great Yarmouth College in caring for specialneeds children. To day as she sits in a booth at her favourite restaurant, a Pizza Hut near her home in Gre at Yarmouth, Lu c y sounds like any other exc i ted 20-yearold, confessing how she rece n t ly spent the night clubbing with friends. “I didn’t get home until 4.45,” she smiles as she sips a soft drink. “Mum nearly killed me!” She wears a blue sweatshirt and tight
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jeans, and her light brown hair is pulled tightly back against her scalp. Her skin is pink, as if she’s been in the sun too long, but there is a surprisingly elegant, mask-like quality to her fa ce. Her high forehead is crisscrossed by wispy wrinkles and when she smiles, which she does a lot, her entire face lights up. The cream she has cove red herself with e a rlier has soa ke d through her clothes in places. “You get used to it,” she says. The Be tt s family washes so many clothes—the girls have to change outfits two to three times a day—that they have worn out 20 washing machines in 20 years. Lucy excuses herself. When she returns from the ladies room many of the diners sneak a look as she comes to the ta ble. There are tra ces of wh i te cream on her fa ce, neck, arms and hands. She pre tends not to notice the stares. “If people keep staring,” she says, “I usually sta re right back at them until they realise they ’ re being rude.” Her favourite T-shirt, one she ofte n we a rs when she goes shopping, has a special message for those who persist in gawking. It says, “Keep staring. I might do a trick.” Recently a man on the street came up to Lucy and told her he’d seen a TV documentary about her and Hannah. Then he slipped a £10 note into her

hand, telling her she was “an inspiration” to others. “I ran after him and tried to give it back. I wanted to tell him I’m just living my life.” As she finishes her p i zza, she adds: “I’m not brave. It’s my mum who’s brave. She’s the one who gave us life and saved our lives.”

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t is just past nine on a sunny Sat u rd ay morning and Co ntact, a ce n t re outside Gre at Ya r m o uth that provides re spite care for special needs child ren, is bustling. A dozen pre - te e n s , with medical and behav i o u ral pro blems ranging from epilepsy to multiple sclerosis to autism, crowd to gether exc i te d ly as care g ivers we l come them. Wheelchairs whirl across the centre’s linoleum floors; arts and cra fts supplies spill on to tables. Lucy, now a part-time pl ay worker, spots one of the children, a bl u e - eyed, 12-year-old autistic boy sitting at a table, his crayons and paper unused. She walks over and half kneels to seem less of a t h re at. He suddenly narrows his eyes and shouts at her, “You look funny. Don’t sit by me; you’re weird.” Lucy is ta ken aback and retreats. He yells even louder, “Go away!” Lu c y,

shoulders slightly hunched, walks away. Half an hour later as Lucy helps a disabled little boy in a wheelchair she feels a tug at her sleeve. It is the aut i stic boy. She smiles at him and the rad iant ex p re ssion seems to melt his anger. He is silent but Lucy knows he’s trying to make co n tact with her. Eve n t u a l ly he asks: “Well, wh at ’s wrong with you?” “I have a problem with my skin.” A flicker of recognition darts across his face. “Oh,” he says. Then he looks up at her and grins broadly, the fi rst time Lucy has seen him smile since he has been here. “Will you pl ay fo o t ball with me?” “Sure,” says Lucy and together they walk outside. Th rough the door comes the re gular thump of a football being kicked and peals of delighted laughter. Lucy is fighting on, living life as it comes. Scientists have finally identified the gene responsible for HI. Further research, for which more funding is needed, may lead to a new treatment for sufferers. For details contact the HI Trust at 020 7618 1720 or www.bartsandthelondon. nhs.uk/fundraising/harlequin_ ichthyosis_appeal.asp/

I T ’S G O O D TO B E B AD

Our sweet little powerboat was overshadowed by a sleek sailing cra ft moored next to us. Its name was Bad News and I asked the captain why. “I love to race my boat,” he said. “And everyone knows, bad news travels fast.”
LO R N A WAT S O N

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