UI NICU team rushes to treat infant born with rare condition

Four weeks and a day before she was set to deliver her first baby, Hannah Clark’s water broke.

The early days of her pregnancy were more stressful than most: severe morning sickness put her in the hospital three times and she lost 16 pounds. The last two trimesters of her pregnancy, however, seemed to go fairly well and there were no signs of anything unexpected.

Then, in a middle-of-the-night bathroom trip, her water broke.

“I was more confused than anything, I didn’t know what was going on,” she said.

She and husband, Josh, went to Trinity Regional Health System in Moline, where they live. They arrived at about 11:30 p.m. and by 1:45 a.m. on February 21, 2011, Hannah was ready to push. A short time later, baby Jamison was born.

An x-ray of Jamison’s chest shortly after birth showed their son had esophageal atresia and tracheoesophageal fistula, conditions that kept him from being able to eat. Rather than extending from his mouth to his stomach, the upper end of Jamison’s esophagus ended in a pouch-like sac just below his throat. The lower end of the esophagus had a misplaced, open connection to Jamison’s trachea, which if left open could allow stomach fluids to enter the boy’s lungs.

Trinity wasn’t equipped to perform the specialized pediatric surgery that would connect the esophagus and close the opening to the trachea, so Hannah and Josh had to decide where to send their infant son. They chose University of Iowa Stead Family Children’s Hospital.

In a very short time, Jamison was in an ambulance with the UI Stead Family Children’s Hospital neonatal transport team. Hannah, however, had to stay behind; she wasn’t able to leave Trinity until at least 8 hours had passed since giving birth.

Once Hannah and Josh arrived, Jamison was already settled into a room and hooked up to intravenous lines and pumps that provided nutrition and machines that monitored his vital signs and oxygen levels while he waited for surgery the next day.

At just one day old, pediatric surgeon Graeme Pitcher, MB, BCh, FCS(SA), performed Jamison’s first surgery to repair the fistula. When Jamison was 14 days old, he had a second surgery to dilate his esophagus.

Hannah and Josh spent seven weeks with Jamison at UI Stead Family Children’s Hospital before he was released April 13, 2011.

The condition facing Jamison when he was born was very rare, according to Jonathan Klein, MD, medical director of the Neonatal Intensive Care Unit (NICU) at UI Stead Family Children’s Hospital.

“It happens in one out of every 2- to 3,000 births,” Klein said. “At that rate, you’d expect to see only 10 to 15 babies born with this condition in all of Iowa.”

Despite the rarity of the condition, doctors and surgeons were ready and available to take corrective action. That’s what comes with Iowa's Level IV NICU. That designation – the highest level available – means the unit is fully staffed and equipped to care for premature babies born as early as 22 weeks gestation and as small as 12 ounces as well as term infants who are critically ill or born with birth defects. The NICU team – neonatologists, surgeons, specially-trained nurses, neonatal nurse practitioners, nutritionists, respiratory and rehabilitation therapists, pharmacists, imaging specialists, social workers, anesthesia specialists, and others are on-site around the clock.

“It’s important to have all of these teams here and working together, as well as having access to all of the surgical subspecialists,” Klein said.

“It was heartbreaking,” Hannah said. “It was one of those moments you won’t ever forget, seeing him connected to all those machines.”

In the initial surgery, surgeons spent two to three hours operating on Jamison, who weighed 5 pounds, 14.5 ounces at birth the day before. They were able to close the connection between the trachea and the esophagus and open and create a connection between the upper and lower sections.

Hannah and Josh stayed at the nearby Ronald McDonald House during the seven weeks Jamison was at UI Stead Family Children’s Hospital, sitting with their son throughout the day at the hospital. Hannah said they felt they were part of a larger family while they were in the NICU.

“With everything that was going on with Jamison they all kept reassuring us, saying that eventually everything would be OK, we just needed to give him time,” she said. “We weren’t just patients, we were someone they cared about with a baby they were all genuinely hoping would get better.”

Hannah said Jamison is well on his way to recovery. He was discharged from the hospital on April 13 and is developing a regular feeding schedule. He will continue to have follow-up visits with surgeons, geneticists and pediatricians to ensure his continued progress.

The Clarks won’t have to travel far for Jamison’s follow-up care. UI Stead Family Children’s Hospital offers regular pediatric surgical consultations and follow-up care in the Quad Cities, as well, expanding the reach of the only group of surgeons in eastern Iowa and western Illinois specifically trained and certified in the surgical and medical treatment of children.

Now, Hannah said, life is slowly beginning to seem “normal.”

“Right now we’re just following Jamison’s lead, letting him let us know when he needs to eat and when we can sleep,” Hannah said.