Josh and Tori didn’t hesitate when she felt signs of early labor 33 weeks into her pregnancy. 

They jumped in the car and started a 500-mile drive from Marietta, Georgia, to St. Petersburg, Florida. As they whizzed down Interstate 75 — keeping in touch with medical teams on both ends of the trip — they knew where their baby needed to be born: 

Johns Hopkins All Children’s Hospital. 

“When we told our medical team in Atlanta we were going to drive to St. Pete, they were not very comfortable with our decision at first,” Josh says. “But they understood why we needed to go right away, and they checked on us while we were on the road while the team in St. Pete was also monitoring our travel. The hospital even had a room ready and waiting for us to deliver the baby when we arrived, if needed. But that’s not how it played out. Our trip turned out to be uneventful. We checked into the hospital immediately on arrival to St. Pete but were discharged when the labor did not progress.” 

Josh and Tori were intent on delivering their baby at Johns Hopkins All Children’s because they knew it had expertise in congenital diaphragmatic hernia (CDH). Little did they know they would need to tap into even more of the hospital’s specialized care. 

A CDH Diagnosis 

Josh and Tori had two children at home and had lost another at birth. During Tori’s most recent pregnancy, doctors detected abnormalities through a routine ultrasound. Additional tests confirmed the baby had CDH, a hole in the diaphragm that allows organs such as the intestines, stomach and liver to move into an infant’s chest cavity, threatening lung development.

Although there are hospitals in the Atlanta area that treat CDH, Josh and Tori’s doctor told them that those hospitals don’t have as much experience or the volume of cases as the program at Johns Hopkins All Children’s, which has the only full-time unit devoted to CDH in the country. The program’s positive outcomes exceed 90%, well above national benchmarks. 

“We learned that the survival success of CDH patients at Johns Hopkins All Children’s is one of the best in the country,” Josh says. “It gave us a lot of confidence and led us to call and talk with the program coordinators.” 

“After the call, we decided to have an in-person consultation with Dr. David Kays, the medical director of the Center for Congenital Diaphragmatic Hernia at Johns Hopkins All Children’s,” Tori says. “That meeting helped us make the decision that we wanted the baby to be born and treated in St. Pete. There are other CDH programs closer to Atlanta where we would not have to uproot our family, but after the meeting, we knew this is where we needed to be.” 

An Additional Challenge 

Josh and Tori had plans to relocate to St. Petersburg at 34 weeks of pregnancy, but when Tori suspected an early labor, they rapidly devised a new plan. 

After they left, friends in Marietta packed key belongings and brought their 5-year-old son and 3-year-old daughter to St. Petersburg. When the early labor signs subsided, they settled in to wait for the baby. 

Another ultrasound indicated the baby may have had an airway issue unrelated to the CDH, the first indication of a complication other than CDH. 

Here Comes Clara 

The baby, named Clara, arrived on May 24, 2024. 

After a quick photo in the delivery room, Clara was whisked away to the Johns Hopkins All Children’s CDH unit, just a short elevator ride away.

“We would rather be holding her instead of seeing the medical team wheel her out the door,” Tori says 

Almost immediately, doctors realized Clara’s airway hadn’t developed properly. The medical team was having trouble intubating her to help her breathe.

Jason Smithers, M.D., is director of the Esophageal and Airway Treatment program at Johns Hopkins All Children’s. He and his team specialize in innovative care of throat and windpipe conditions. 

“Dr. Smithers seemed like he was a tailor-made doctor for the additional medical issues our baby faced,” Tori says.

Smithers explained that Clara had type IV laryngeal cleft. It’s very rare and the most severe type of a congenital defect that extends from below the vocal cords into the trachea (windpipe), sometimes reaching into the chest. It causes severe breathing difficulties and aspiration issues when eating or drinking due to food entering the lungs. 

A patient with severe CDH can have a high risk of death, and a type 4 cleft with no other problems carries its own high risk. Clara had both. 

Smithers noted that in five years of working with Kays, this was only the second time he had seen the combination of these medical conditions.

Tough Decisions 

The medical team was clear with Josh and Tori: Clara’s condition was complex, and her survival was in jeopardy. 

“The doctors told us they could attempt to save her life,” Josh says. “However, there was high likelihood that she would not survive even with the surgeries. The alternative was to do nothing and enjoy the time we have holding her until she passed.” 
 
“Dr. Smithers told us he would work with Dr. Kays to do everything he could to help Clara survive, as long as Josh and I were onboard,” Tori says. “This is where our faith came in. We said, ‘God is in control here.’ He’s the one who calls the shots. We could ask him for miracles. There have been a lot of scares during this pregnancy, and he has protected Clara. He brought us this far and can bring her through this. We were hopeful, but we didn’t know what the future would hold or how much time we were going to have with her.” 

First Things First 

When Clara was 4 days old, Kays and Smithers performed surgery to repair the hole in her diaphragm. “The defect was a large one with the liver up in the chest,” Kays says. “Because of the additional issue of an unstable airway, we ended up using ECMO (extracorporeal membrane oxygenation, a life support device that temporarily replaces the function of a patient's heart and lungs) to stabilize her. 

“This is almost certainly a lethal combination,” Kays continues of Clara’s multiple anomalies. “CDH is severe enough without having airways that are going in different directions. Instead of one breathing tube, we had to use two. One going to one lung and another to the other one.” 

“We felt the expertise of Dr. Kays and Dr. Smithers in the same unit at the same time was unique and allowed Clara to have a chance at survival,” Josh says. 

More Challenges 

After some recovery time but while Clara was still on ECMO, it was time for Smithers to address the airway issue. In many cases, Smithers would wait until Clara was about 3 months old before performing her airway procedure. But because of her condition, she needed the airway repair at just 11 days old.

During the 12-hour plus surgery, Smithers called on Russell Jennings, M.D., a pioneer of pediatric surgical procedures for malformed esophageal and tracheal conditions, to assist him. 

“We learned that Dr. Jennings was a mentor to Dr. Smithers earlier in his career,” Josh says. “It was comforting to know that two of the best airway surgeons in the world are at the same hospital working together on our daughter. There is no road map for the medical challenges Clara has faced.” 

Jennings says he looked through medical records and could not find a record of any other child surviving this combination of defects. He says he doesn’t believe any other hospital would even attempt to repair these two defects together. 

“You couldn’t have two more wonderful and dedicated, calm and positive parents like Josh and Tori,” Kays says. “They are amazing and have faced all the bumps in the road with a calm confidence.” 

The Future 

At 5 months old, Clara had already been through four operations and numerous procedures. She still has a way to go, Smithers notes. 

“She suffers from bronchomalacia, which causes wheezing and trouble breathing,” he says. “The condition frequently resolves on its own as babies mature. She will need to remain on a trach and a ventilator for a while.

“More airway surgeries will be needed when she gets older. She needs to be able to swallow without aspirating, which will involve additional surgeries. Right now, we are at a point where she is breathing pretty well. 

“I think overall she has done very well,” Smithers continues. “Our goal is to have her off the trach and vent by the time she is ready for kindergarten, so she can function as a pretty normal child. We all have a positive outlook for her.”