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By KRISTA CONGER Go stand next to your daughter. She's dying," the doctors told Micaela Verdusco as her 16-month-old daughter Destiny lay in a San Jose emergency room. Micaela began to cry and pray. But she did one other thing too: she asked the doctors to call Lucile Packard Children's Hospital where Carol Conrad, MD, had been treating Destiny for a chronic lung condition during the previous year."Before I knew it, I felt someone push me out of the way," says Verdusco. "It was a Packard ICU nurse." The transport team from Packard's pediatric intensive care unit managed to stabilize Destiny, who had a severe infection, and transport her back to Packard for further treatment. Conrad is one of several physicians at the Pulmonary Disease and Cystic Fibrosis Center which specializes in treating infants and children like Destiny. Many of the patients suffer from asthma, chronic infections, congenital disorders of the lungs or airways and other problems that prevent them from breathing normally.  Destiny during a checkup with Conrad last week Affected children may have problems sleeping, growing and developing normally. New techniques to diagnose lung problems in very young and premature infants help the Packard physicians begin treatment early and avoid long-term problems. Advanced imaging capabilities also allow physicians to view 3-D pictures of the airways. "We are one of fewer than a dozen centers in the country that can do these very specific types of pulmonary testing on infants," says Richard Moss, MD, professor of pediatrics and director of the Center. But even with the most advanced equipment, diagnosing and treating Destiny's coughing, wheezing and frequent bouts of pneumonia was tricky. "At first her symptoms seemed to indicate severe asthma," says Conrad. "But when the standard treatments didn't work, we had to start thinking of other things." After she ruled out an immune deficiency or cystic fibrosis, Conrad, an assistant professor of pediatrics, cast her net more widely. "We ran dozens of tests, all of which were negative. And yet Destiny's condition was clearly progressing," says Conrad. "Some patients panic you because you can see them slipping away despite your best efforts. Destiny was like that." "Destiny was very, very sick," agrees Moss. "Her problem often can not be picked up in time to prevent a fatal outcome. But Carol kept trying." Eventually a lung biopsy confirmed that Destiny was suffering from low-grade inflammation in her lungs and helped Conrad find a name, if not a culprit, for her condition – idiopathic pulmonary fibrosis. "We don't know why, but her immune cells are invading her lung and chewing it up," says Conrad. Although immune suppression with high doses of inhaled steroids is the usual approach, the treatment itself can have nasty side effects, such as the severe streptococcal infection that landed Destiny in the San Jose emergency room in September of 2000. Eventually Conrad hit on the idea of administering the steroids intravenously in once-a-month pulses. The pulses give Destiny's immune system time to recover before the next dose, but are strong enough to protect her lungs from attack. Within two months her symptoms began to recede, and Destiny is now a happy, active 2 year old. "Destiny was always tired," says Verdusco. "She could only play for a few minutes before she had to take a nap. Now I can't stop her from running around." "It's so great to have such a positive effect on a patient," says Conrad. "Although she's being treated for a chronic condition, she's going to grow, get old, and do all the things she's supposed to do." Although Destiny's story is compelling, she represents only one patient out of hundreds treated at the center each year. In addition to chronic pulmonary diseases, the center treats about 200 cystic fibrosis patients each year. The center's cystic fibrosis program is the busiest in California, and it has been designated as one of only eight cystic fibrosis clinical research centers in the nation. New techniques allow Packard physicians to diagnose previously undetected borderline cases and ongoing research focuses on finding new treatments for the disease. An integrated team of physicians, nurse coordinators, nutritionists and respiratory therapists help manage the life-long treatment required for cystic fibrosis patients. "The cystic fibrosis program integrates pediatric and adult medicine," says Moss. Noreen Henig, MD, assistant professor of medicine, coordinates the management of the adult patients, which includes specific family and career counseling to help with issues such as reproductive decision making and management of secondary diseases such as diabetes. But the organizational details and advanced research and technology available at the Center don't matter to Destiny, who, by the way, loves broccoli, the Grinch and hospital cafeteria food. She and her family focus on something less tangible but even more important. "As soon as we got here, the nurses and the doctors cared about Destiny," says Verdusco. "I won't take Destiny to any other hospital now. I bring her straight here." This is the first in a series of articles profiling Lucile Packard Children's Hospital's Centers of Excellence program.
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 Lucile Packard Children's Hospital |
Stanford Report, February 20, 2002
