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What would you do if you learned your unborn daughter had a serious – but treatable – birth defect? It happened to Diana Fernandez Rodriguez of Hazleton, who last winter was told her soon-to-be-born daughter, Isabella, had a rare condition called gastroschisis.
"I had already lost a child before," Rodriguez says. "This seemed to be something very dangerous."
To ease her worry, she trusted a team of specialists. They educated her about gastroschisis – a condition that causes a baby’s intestines to stick outside the body through a hole near the belly button – and kept her updated at every step.
Locating the problem
Gastroschisis is typically caused by a disruption in blood supply to a baby's abdominal wall as it’s forming, allowing the baby's intestines to go outside of the abdominal wall. It's rare but occurs mostly with young mothers (Rodriguez is 19). While it's most often not found until the second trimester, Rodriguez's obstetrician/gynecologist, Vadim Loshakov, MD, found it during her first trimester.
"Our sonographers get the credit for locating it on the scan," says Loshakov, who worked with Rodriguez through Lehigh Valley Hospital (LVH)–Hazleton's Healthy Beginnings Plus, a state-funded prenatal program that ensures mothers get quality care. "I've seen gastroschisis before, so I got maternal fetal medicine (high-risk pregnancy) specialists at LVH–Cedar Crest involved right away."
Rodriguez and her partner, Juan Fernandez, began making trips to Allentown to meet with Kara Coassolo, MD, with LVPG Maternal Fetal Medicine, in order to monitor fetal development and watch for other potential abnormalities.
Taking immediate action
On Jan. 21, 2016, Rodriguez drove to Allentown for a routine 34th-week checkup. Yet it was far from routine. "The baby's heart rate was higher than normal" Coassolo says. Quickly, physicians moved Rodriguez to LVH–Cedar Crest. "We feared induced labor may not be tolerated safely," Coassolo says, "so we made preparations for an emergency cesarean section."
It all happened in a flash for the panic-stricken Rodriguez and Fernandez, both of whom speak primarily Spanish. They relied on Carina Sanchez, a trained medical interpreter at the hospital. "Carina was wonderful with the parents during this several-hour process," Coassolo says.
Repairing the defect
Coassolo and her team brought Isabella Fernandez Rodriguez into the world that afternoon in the hospital's labor and delivery unit. A surgery team from LVPG Pediatric Surgery Specialties and LVPG Neonatology met Isabella there, and once she was stabilized, transferred her to the neonatal intensive care unit (NICU) to begin repairing the defect.
"We essentially placed the intestines into a pouch we call a silo to prevent water and heat loss, and gently pushed them back into the abdominal cavity over the course of two days," says pediatric surgeon Marybeth Browne, MD. "If all goes well as it did with Isabella – meaning there are no issues such as intestinal atresias or blockages caused by the defect – recovery becomes dependent on how long it takes for the baby to obtain good bowel function."
The procedure only took about 15 minutes, then the pouch is gently squeezed to assist gravity in moving the intestines into the body. "Our role is to make sure there is no additional abdominal pressure occurring, which might affect breathing or cause some other issue," Browne says.
Isabella spent less than six weeks in the NICU before going home on March 1, a short time considering the circumstances but still interminable for the family. Sanchez made sure to visit regularly.
"I wanted to make sure everything was going well for Diana and Juan during Isabella's time here,” she says. "I'm so glad the baby is doing great. It was a great team effort all around for a happy ending."
Mom and baby have a summer checkup scheduled at LVPG Maternal Fetal Medicine, and it may the last one needed. "We'll never forget what everyone at LVHN did for us," Rodriguez says. "I think LVHN is the best there is."
–Ted Williams