Clara’s CDH Story
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 while I was in early labor, and then Clara decided to hang out for a week before making her debut on May 24th. She was a day shy of 34 weeks, but she weighed 5lbs 6oz, which really worked in her favor, as every ounce makes a difference at this age.
Within 2 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. Especially combined with the CDH, her chances of survival were extremely low, but they would try whatever they could to help her. 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 with the CDH babies, but he is 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.
On May 28th, at 4 days old, Clara had her CDH repair surgery. We learned it was more severe than we 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 ECMO (a heart/lung bypass treatment), and this significantly bumped up the timeline for her major airway repair. On June 4th, 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), separate the upper and lower sections of her esophagus, and brought the top section to her shoulder to turn it into a spit fistula (to avoid reflux/aspiration through the residual cleft), and placed a G-tube so she could be fed directly into her stomach. 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 15th, she came off of ECMO, and on July 13th, her breathing tube was removed, and she went on CPAP support. She did well with this, except when she was upset, her airway would close up as she cried, and she couldn’t breathe. This is due to tracheomalacia (a floppy airway that wasn’t rigid enough to stay open entirely), and it was determined that she needed tracheopexy surgery to stent the airway open by stitching the back of the trachea to her spine. She had been slowly struggling more until August 21st, when they decided to reintubate her for the last few days before this next surgery, and she was able to have a few days to rest on more breathing support. On August 26th, she had her tracheopexy, during which they also removed her esophagus stump (the upper part of her lower esophagus that was still connected to her stomach and was causing issues), along with an esophageal lung (a piece of non-functioning lung tissue that was connected to it and) that was causing the issues.
We were hopeful that the pexy surgery would be enough to get Clara breathing just on CPAP support, but after three different extubation attempts and steroids/treatments to open up her upper airway more, she still wasn’t able to tolerate being extubated. While the pexy helped to stent open her main airway, the smaller branches (bronchiole) still collapse when she gets upset, and the only remedy for that is time so she can grow and those can firm up. It was determined that she needed a tracheostomy so she could work towards going home, and hopefully, she will only need this for a couple of years.
On September 23rd, she had a successful tracheostomy, and then we spent the next 6 weeks giving her time to strengthen to a point at which we could finally leave the hospital. We were able to discharge on November 8th to a wonderful parade from the staff and other families on the unit, but we found ourselves back in the ICU just 2 days later after she developed Rhinovirus. We spent another 2 weeks in the unit before discharging for a second and final time. We stuck around for St. Pete for another 3 weeks before finally returning to Atlanta just before Christmas.
We are so incredibly proud of how strong Clara has been through all of this and how amazing the care we have received has been. She still has a long road ahead of her with future surgeries, but the long-term outlook is very positive. The team is hopeful that with a few more surgeries and 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.
