Clara’s Type 4 Cleft Journey
Georgia
By Josh and Tori Swartz
When Tori was 16 weeks pregnant, Clara was diagnosed with congenital diaphragmatic hernia (CDH). Over the next few months, we decided to relocate to St. Petersburg, FL, to deliver and have her treated at Johns Hopkins All Children’s Hospital by Dr. Kays, one of the top experts in the world on this condition. At 32 weeks, we drove down in a rush because Tori was in early labor. Clara decided to hang out for a week before making her debut on May 24, 2024, one day shy of 34 weeks. She weighed 5 pounds 6 ounces, which really worked in her favor as every ounce makes a difference at this age.
Within two hours of her birth, we were told that she also had a type 4 laryngeal cleft, which essentially means her esophagus and airway were one connected tube rather than their own separate tubes side by side. Combined with the CDH, Clara’s chances of survival were extremely low.
Over the next few days, we learned that the doctor who delivered the news to us, Dr. Smithers, not only works very closely with Dr. Kays and CDH babies, but he is also one of the top pediatric airway/esophagus experts in the world. Both surgeons agreed to try everything they could to help Clara, and we truly believe that if we were at any other hospital, Clara would not be alive today.
Dr. Smithers in particular seems like the absolute best and most specifically skilled doctor in the world to be treating Clara’s specific conditions. We later learned that Dr. Smithers and a handful of others came from Boston Children’s Hospital, where they were integral in developing a world-renowned pediatric airway program. Dr. Smithers convinced Dr. Jennings, a pioneer in the pediatric airway surgery arena, into joining the team in Florida. This gave us the utmost confidence that we were in a place to give Clara the very best chance at survival, even if it was thought to be slim.
At 4 days old, Clara had her CDH repair. We learned it was more severe than we initially believed, but it was still very much in the manageable/repairable scope of Dr. Kays’ abilities. Three days later, due to her complicated airway and the effect on her lungs, Clara had to be put on a heart/lung bypass treatment called ECMO, and this significantly bumped up the timeline for her major airway repair.
At 11 days old, Clara underwent her second major repair. Dr. Smithers and Dr. Jennings worked together to separate her airway from her esophagus and repair them. The surgeons also separated the upper and lower sections of her esophagus and brought the top section to her shoulder to turn it into a spit fistula. This procedure helped Clara avoid reflux and aspiration through her residual laryngeal cleft. A G-tube was placed so she could be fed directly into her stomach. Clara’s laryngeal cleft is now between a type 1 and type 2.
We knew we needed a miracle for Clara to survive and recover from this major surgery, and the Lord truly delivered. It went amazingly well, and Clara continued to make steady progress in the weeks that followed. On June 15, she came off of ECMO.
On July 13, her breathing tube was removed and she went on CPAP support. She did well, except when she cried, her airway would close up and she couldn’t breathe. This is due to tracheomalacia (a floppy airway that wasn’t rigid enough to stay fully open), and it was determined that she needed a tracheopexy surgical procedure to stent the airway open by stitching the back of the trachea to her spine.
Clara had been slowly struggling more when doctors decided to reintubate her for the last few days before this next surgery. The additional breathing support allowed her to have a few days to rest.
On August 26, she had the tracheopexy. During this procedure doctors also removed an esophagus stump (the upper part of her lower esophagus that was still connected to her stomach), along with an esophageal lung (a piece of non-functioning lung tissue that was connected to it). Both had been causing Clara issues.
We were hopeful that the tracheopexy surgery would be enough to get Clara breathing just on CPAP support. After three different extubation attempts along with steroids/treatments to open up her upper airway more, she still wasn’t able to tolerate being extubated.
While the tracheopexy helped to stent open her main airway, the smaller airway branches (bronchioles) still collapse when she gets upset. The only remedy for that is time, and hopefully as she grows those smaller airways will firm up. It was determined that Clara needed a tracheostomy so she could work towards going home. Hopefully, she will only need this for a couple of years.
On September 23, Clara had a successful tracheostomy. We spent the next six weeks giving her time to strengthen to a point in which we could finally leave the hospital. We were able to discharge on November 8 to a wonderful parade from the staff and other families on the unit. Just two days later, Clara was back in the ICU as she had developed rhinovirus. We spent another two weeks in the hospital before discharging for a second and final time. We stuck around St. Petersburg for another three weeks before finally returning to Atlanta just before Christmas.
We are so incredibly proud of how strong Clara has been through all of this. She still has a long road ahead of her with future surgeries, but the long-term outlook is very positive. The medical team is hopeful that with a few more surgeries, along with time and growth, she just might be able to attend kindergarten one day with nothing other than a few scars to help tell her story.