Philadelphia Inquirer - Children's Hospital of Philadelphia - 4

www.philly.com THE PHILADELPHIA INQUIRER Sunday, September 30, 2007 Joe Kadi, who spent countless hours by daughter Nadia’s bedside at home and in her hospital room at 8 South, leans over to give her a kiss. lungs. That accounted for her troubled breathing. The tests were ruling out problems: No heart ailment. Clear lungs. No evidence of serious infection. Then, in the small ER treatment room with barely enough space for the Kadis to stand on one side of Nadia’s gurney, Allison looked over at the female resident checking her baby. Was the young doctor crying? Oh, God! “What’s wrong?” Allison demanded. “Why are you crying?” The resident denied she was upset. But when Allison pressed her, the resident reluctantly looked up at the young mother. Her liver is failing, she said. Allison began to cry. She left the room to tell her parents. Before long, they were all in tears. Within minutes, the story changed yet again. A more senior doctor appeared to talk to the family. Nadia wasn’t going to die, the doctor said, not that day. But she was seriously ill with advanced liver disease. Still, she was at the best possible place for a child in her condition. Like the sickest 15 percent of the children who come to CHOP’s ER, she would be admitted. Nadia was going to 8 South. At 9:47 that night, more than 41/2 hours after getting to the ER, the Kadis arrived on the liver ward for the first time. Their small room was quickly crowded. In addition to Nadia’s parents and grandparents were a bedside nurse, the attending physician, a fellow, a resident, a medical student, and Noelle Bates, the nurse practitioner who helped coordinate the care of patients on the ward. While Nadia’s parents and grandparents focused on the doctors, trying to understand what was happening to their baby, Bates picked Nadia up to ease her breathing. Nadia stopped crying and fell asleep in her arms. Bates, 30, wouldn’t forget that moment — how Nadia, whose stomach was too distended to be held like a healthy baby, felt in her arms. The baby girl, with her curly brown hair and big brown eyes, stole her heart, and would soon do the same to the other staffers on South 8. A problem missed Elizabeth Rand, a senior liver specialist, often hears the unusual stories through the hospital grapevine. Residents and fellows want her to weigh in on the complicated cases. That’s how she heard about Nadia. Clearly, the child should have been referred to CHOP months earlier. The staff was sympathetic toward the young parents, who seemed like nice people who had tried to do the right thing. Rand, 44, knew most doctors never saw a case like her’s. It was unusual. Rand had trained at the University of Chicago and the Johns Hopkins School of Medicine. A native of New York, she had come to CHOP to do research, but the pull of real patients was a strong one. In 1998, she became a full-time clinician. Nadia’s story made her angry and frustrated. But in the end, she just felt sorry for the parents, and sorrier still that the opportunity to treat the child sooner — when the likelihood of a good outcome was significantly better — had been lost. She and the rest of Nadia’s team of doctors suspected biliary atresia, a disorder that afflicts one in 10,000 children. The condition destroys the bile ducts, which causes liver damage. Without treatment Nadia would likely die by her second birthday and certainly by age 3. Biliary atresia is typically diagnosed within a month or two of birth. Nadia was unusual; she got to CHOP very late. Nadia’s liver already showed signs of cirrhosis. Rand couldn’t predict whether the delay would change Nadia’s prognosis, but studies showed that the earlier it was found, the better the kids fared. Even then, half of all the kids with biliary atresia require liver transplants by their second birthdays. It was hard to predict what would be involved in saving Nadia. Rand knew medicine was imprecise, that survival often boiled down to a person’s luck as much as their biology. But the good news, for Allison and Joe, was that Nadia had arrived at one of the nation’s few centers that specialize in the rare illness. In the last decade, CHOP had treated 64 children with the disorder, including the granddaughter of Fred Biesecker, whose firm makes bottles for the pharmaceutical industry. In 2001, Biesecker and his family made a substantial 10-year donation, founding a center dedicated to the research and treatment of biliary atresia. Today, CHOP’s Fred and Suzanne Biesecker Liver Center attracts biliary atresia patients from across the nation. In that way, Nadia was lucky.

Philadelphia Inquirer - Children's Hospital of Philadelphia

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