Sydney Moss arrived early. “We didn’t even have diapers,” says her mother, Lindsay Moss.
The newborn presented another surprise for her family and physicians in Alexandria, Va. Soon after her birth, a local hospital admission for treatment of her respiratory illness RSV called attention to her jaundice and elevated liver enzyme tests. Several diagnostic tests were done, including a liver biopsy, which confirmed the suspected cause: biliary atresia. A rare obstruction and inflammation of the bile ducts, biliary atresia causes bile to pool in the liver, damaging and scarring it. The only “cure” for this disease lies in a transplant. If caught early, within the first few weeks of birth, an operation called the Kasai procedure, in which a loop of intestine is used to help drain bile from the liver, can help prevent further damage to the liver and delay or, in some cases, prevent the need for a transplant.
The family had already researched the Web and made telephone calls to find the best place to have the surgery. “We were given the option of Georgetown, but had discovered already that Johns Hopkins was the place you want to take your child,” says Moss.
Three days later, in January of 2008, chief of pediatric surgery at Hopkins Children’s Paul Colombani, an expert in the Kasai procedure, performed the surgery.
“The staff was amazing,” says Moss, “the care terrific.”
Sydney came home a week later. Today the family participates in the NIH-funded BARC (Biliary Atresia Research Consortium) study at Hopkins Children’s, led by the director of its Pediatric Liver Center, Kathy Schwarz, a specialist in caring for children with biliary atresia. “It was the best decision we ever made,” says Moss. “We’re helping others, too.”
Feeling helpless, still, against a chronic threatening disease about which so little still is known, including its cause, Moss and her husband, Stephen, decided to help fund Hopkins research that Schwarz believes may finally explain it.
“We’ve known a lot about the disease’s devastating inflammatory process, but not what causes it,” says Schwarz. ”This may be about to change.”
Led by pediatric gastroenterologist Maria Grazia Clemente and sponsored by the Zachary Meehan Biliary Atresia Research Foundation and Pediatric Liver Center Gift Fund, Hopkins Children’s researchers discovered, in 2008, that two rotaviruses (SA11 and RRV), known to cause the disease in neonatal mice, also grow in human bile duct cells and normal liver cells. Schwarz’s group is now testing serum samples from babies with biliary atresia to see if they’ve the antibodies to this rotavirus.
Avid joggers, the Mosses decided to host a charity foot race to help fund this research. They hired Pacers Running Store to manage the event. Bremmer and Goris Communications in Alexandria, Va., designed, pro-bono, a logo and Web site. Friends, local businesses and Stephen’s employer, defense contractor Argon ST, signed on as early sponsors.
On Nov. 8, 2008, more than 300 runners took part in the first annual Biliary Atresia 5K Run/1K Walk for the new Sydney Moss Fund for biliary atresia research at Hopkins Children’s. Among them were Schwarz, Clemente and BARC research coordinators Robert Jurao and Aparna Roy. To date, more than $8,000 has been contributed to the fund.
For more information on the Sydney Moss Fund and other funding opportunities at Hopkins Children's, contact Linda Smeyne, 410-516-4545.