Paisley’s Story - Congenital Diaphragmatic Hernia (CDH)
My daughter Paisley was born on July 18, 2020, at Monroe Carroll Jr. Children’s Hospital. At 20 weeks gestation, she was diagnosed with Congenital Diaphragmatic Hernia (Left-Sided). I ended up delivering at 37+1. After laboring for 26 hours and pushing for 3, Paisley’s heart rate started to fluctuate, so I had to get an emergency C-section. When she was born, she was purple and limp, with no detectable heart rate and not breathing. After rushing her to the NICU and resuscitating her, she was placed on the ventilator and lots of medicines to keep her stable. Paisley was not a candidate for ECMO due to a small brain bleed; had they given her the blood thinners required for ECMO, the brain bleed would have possibly killed her. Her neonatologist was at her bedside for hours trying to keep her stable, and that she did! We quickly discovered that her hernia wasn’t the only battle we were facing. Paisley also had very severe pulmonary hypertension, so severe there were times that her care team feared that she wouldn’t pull through. She was on nitric gas, sildenafil, and bosentan to control her PH. She had her repair surgery at four days old, and it went surprisingly well! The hole in her diaphragm was about the size of a golf ball, we were told. Her liver, stomach, and intestines had all made their way into her chest, causing her left lung not to grow properly, and her heart to be pushed to the right side of her body. After her repair was completed, our primary focus was getting her PH under control and getting her off so much respiratory support.
The team had started talking with dad and myself about taking the steps to get a remodulin pump covered by insurance because Paisleys PH was still very high. We did a heart cath a few days later, where they started to wean Paisley off of her nitric gas. She did well with this change and was able to come off it a few days later! After being off the gas for a day or so, they pulled her ventilator and placed her on vapotherm. While on vapotherm, Paisley struggled horribly with the weaning off her medications. It was the worst part for us to watch. When she was finally ready to come off vapotherm a few weeks later, she did not require regular oxygen. She has not needed respiratory support since then!! Due to all of the negative things Paisley had to encounter, she now has an oral aversion. We had to have another surgery to have a G-tube placed before returning home after 96 days in the NICU. Before coming home, Paisley successfully went off all of her PH meds, which was a HUGE blessing. We came home with a feeding tube and two medications. Paisley is doing very well for the most part. She struggles with weight gain due to throwing up a lot and some pretty rough acid reflux. Her dad was finally able to return to work this past Monday, but my job let me go for being out of town with her for so long. Although I need to be with her, it’s very difficult for us financially, and we are facing some struggles. God has been so good to us throughout this journey, and we could not ask for a better turn out for our baby girl!