LIFE WITH LUCY Throughout her third pregnancy Jan Betts had worried there was “something wrong” with her baby. As she’d told her husband Clive, ‘I don’t know what it is, but the baby isn’t moving, isn’t kicking, the way it’s supposed to.” . Clive, a no-nonsense software salesman, had brushed off his wife’s worries. “Don’t be daft ,Jan,” he told her. “The doctors said everything will be fine.” But she was still concerned. Call it mother’s intuition. On a bitter January morning Jan Betts lay on an operating table in a hospital in Newfolk, England, about to give birth by Caesarean section. Clive, wearing a surgical gown and mask stood by her side holding her hand so tight she thought he might sprain it. While this was Jan’s third child it was the first she had with Clive. . “Relax, Jan.”said one of the midwives as the doctor lifted her scalpel to slice through her lower abdomen, numbed by an epidural. Agreen surgical prevented Jan from watching the operation. “We are almost there. Relax.” The surgeon carefully cut into Jan’s uterus. As her baby was pulled from her womb, Jan heard a gasp. Then the room went eerily quiet. “What’s wrong?” asked Jan. “Is there a problem? What’s wrong with my baby?” There was no answer. Clive could not believe his eyes. His daughter was totally covered in rough, rigid, yellowish motteled and cracked 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 had to answer Jan. He took a deep breath. “There’s a problem, sweetheart. Ther’s something wrong with the baby.” The baby’s condition-she was named Lucy-was so rare it took doctors 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 plates that can cover the body like armour and cut off the circulation. “Lucy’s life expectancy, like majority of Harlequin babies ,was brief.
. Many died from infection or respiratory problems within days. Jan begged the doctors to let her hold the 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. Standing in front of Lucy’s incubator, Clive and Jan locked arms and watched their daughter, not much bigger than Clive’s outstretched hand crying in obvious pain. They knew now that the disease was incurable. With her bright red, constantly flaking skin, Lucy looked like a burns victim. If thick skin anywhere her body cracked it could lead to infection, sepsis and death. As a doctor told Jan, “Lucy’s skin could kill her.” Doctors devised a stategy to keep Lucy alive : an emollient rich bath twice a day that would soften her new , underlying skin and helped her “shed” her older skin. When she was out of the water she would be kept coated in a thick, waxy moisturizer to prevent cracking. It worked. After five days a doctor recommended putting salicylic acid into Lucy’s moisturizing cream; but this penetrated her skin and she developed aspirin poisoning. Her lungs nearly failed and she teetered near death. Thinking they were losing her , Clive and Jan had Lucy baptized. Somehow , Lucy survived. “A fighter’” Jan called her . “She’s tough.” As well as the constant threats to Lucy’s life , Clive and Jan faced another harrowing problem; Lucy’s appearance.As parents often do, they had immediately seen past their baby’s bizarre 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 playground? .