The sound of her daughters arguing is music to Kimberly Derque’s ears. “I almost lost Kate and Abbi to a fatal genetic disease that was destroying their kidneys and liver,” recalls the Crystal City mom. But the girls’ lives were saved by Saint Louis University Liver Center physicians and surgeons.
“Both Kate and Abbi have an inherited condition called autosomal recessive polycystic kidney disease (ARPKD), accompanied by congenital hepatic fibrosis, a liver disease,” says Dr. Ajay Jain of SLULC, medical director of the pediatric liver transplant program at SLU and SSM Cardinal Glennon Children’s Medical Center. “It causes cysts in the kidneys and scar tissue in the liver. The scarring impedes blood flow through the liver and results in portal hypertension, which causes abdominal swelling and a high risk of infection, gastrointestinal bleeding and the buildup of toxic chemicals in the blood.”
Abbi, 15, was diagnosed at 5 months; Kate, 10, was diagnosed in utero. “Abbi had significant liver fibrosis and scarring, suffered from frequent hemorrhaging, and once required 64 units of blood and a liver stent,” Derque recalls. “By age 7, Kate’s kidney function had decreased so much that she needed dialysis four hours a day, three days a week.” Both girls endured multiple hospitalizations and procedures, including endoscopic surgery to relieve their hypertension. Abbi missed her entire eighth-grade year and has been homebound this year since Christmas; Kate always missed school on dialysis days, Derque adds.
“Many patients with this condition don’t survive beyond the second decade of life, so kidney and liver transplants were essential for both girls,” Jain says. The procedures were a collaboration between SLU faculty and Cardinal Glennon’s pediatric transplant team. Kate’s 10-hour double transplant occurred last year; older sister Abbi’s procedure was last March. “Their prognosis is good—with medication and monitoring, they’ll lead normal lives,” Jain says.
Staffed by top physicians and researchers, SLU Liver Center is one of the nation’s leading research facilities. It focuses on understanding liver disease, developing new technology and treatments, and applying the results to patient care. Since 2003, Friends of SLULC has raised more than $2.8 million to support the center’s work. A major fundraiser hosted by Friends, the annual Diamonds Gala, takes place Nov. 15 at The Coronado Ballroom. The theme is Carnival @ The Coronado. Major sponsors include Saint Louis University Hospital, Special Design Healthcare, Salix Pharmaceuticals, Mid-America Transplant Services and Quest Diagnostics.
“Luckily, the girls’ disease was caught early,” Derque says. “This has been an amazing journey for us, especially for Kate and Abbi’s brother, Johnathan, 13, their strongest supporter. They have stood strong for each other through times no child should have to face. We’re grateful to the heroic donor families who gave our girls new life, and for the care and expertise shown by Dr. Jain and the teams at SLU Liver Center and Cardinal Glennon. We look forward to starting a new school year in the fall—complete with fully functioning parts!”
By Tony Di Martino