Blakely’s CDH Story
Blakely’s story began at 20 weeks of gestation when she was diagnosed with a right-sided Congenital Diaphragmatic Hernia (CDH). Through a fetal MRI, her doctors were able to see her liver was up and partially fused to her right lung and a Hepatopulmonary Fusion. They continued to explain that a CDH with an HPF was extremely rare, with only a few reported cases and of those few, the mortality rate was high. Our CDH surgery team at Dell Children’s Medical Center in Austin, Texas, had never operated on an HPF. They prepared us for her arrival, for the possibility of ECMO, and for surgery within her first few days.
Blakely arrived via scheduled induction in the Special Delivery Unit at DCMC, was intubated at birth, and immediately taken to the NICU. They discovered she had several heart defects, one being Scimitar Syndrome, a rare variant of PAPVR, where the right pulmonary veins abnormally drain into the IVC. Blakely was also suffering from severe pulmonary hypertension and went into a crisis at just a week old. She was in no state for an operation, and they feared that if they did operate, things would not end well. They tried placing her on an oscillator, but when that didn’t work, they took my husband and I into a conference room and prepared us for the worst. They told us she was very, very sick and got us a room at the hospital for the night so we would be there with her.
Day by day, Blakely slowly started getting better. She was on a minimal stipulation plan, where doctors and nurses couldn’t even talk in her room unless it was a whisper. Blakely eventually made a full recovery from her PPHN crisis, however, she will have pulmonary hypertension for the rest of her life. She was slowly taking off respiratory support, moving from intubation to a high flow, then eventually to room air.
She was discharged after 66 days with no operation to date. Blakely is now home and is four months old, with frequent weekly checkups. She will eventually have her operation, however, since she is stable now, her team wants her to grow as big and strong as possible before surgery. She is facing some other battles, such as feeding difficulties and losing her hearing due to Cytomegalovirus (CMV), but considering it all, she is doing amazing. Her neonatologists call her the miracle baby! If you saw her X-ray, you wouldn’t believe she is home and thriving! We keep a close eye on her, and her journey is far from over, but we are so thankful for her team and God’s grace for taking this rare case with a high mortality rate to a baby girl thriving at home! No one ever thought this was how her story would go, but we are so grateful.
