CDH International’s featured patient of the week is 2 year old CDH Survivor Johanna “Jojo” Ferrage. Johanna is the daughter of Jordan and Andrea. They had this to share about their daughter’s journey with CDH:
“Johanna was diagnosed with LCDH on September 13th, 2018 at our anatomy scan. She had intestines and stomach up, but no liver. We sought treatment with Dr. Vincent Adolph at Ochsner Baptist. Dr. Adolph has treated CDH for 25 years. We debated JHAC and Texas Children’s, but we felt comfortable with Ochsner and Dr. Adolph. Plus, we are a busy family with a 3 year old at the time, a dental practice that I own, and my wife’s nurse practitioner doctoral program. An interstate commute or lengthy time away would be too difficult.”
Johanna was born on January 23, 2019 via C-section. Initially upon admission to the NICU, the doctors thought Jojo had a coarctation of the aorta as well as CDH, but fortunately she did not. The CDH was pressing on her major vessels that made it appear like a coarctation. Johanna was placed on ECMO at 6:54 pm on January 24. It was a rough night for us. Johanna was on ECMO for almost 11 days. At one point, we were afraid she would never come off ECMO. Her doctors said she was likely a large defect. The doctors were even considering doing her repair while on ECMO. They told us over that weekend that surgery was Tuesday, ECMO or not. We were so thrilled when she was decannulated the day before her repair.
Johanna got her repair at 13 days old. She was a patch repair due to the size of her defect. She was extubated at a month old. I got to hold her for the first time on Valentine’s day. Best Valentine’s ever by a mile. Jojo underwent a nissen and g-tube around 2 months old since she wasn’t tolerating her feeds well. Jojo was discharged from the NICU after 75 long days. To this day, she is a celebrity at Ochsner Baptist. The sickest kid in town at one point became the warrior baby praised by many especially her doting parents.
Happy ending, story over, right? Wrong.
Johanna started having bad desats around bowel movements and feedings after being home for one week. Her doctors suspected it was the redundancy or looseness of her patch, which would help her grow into it. After a few rough days and sleepless nights, Jojo was admitted to the Ochsner PICU and intubated on Easter Sunday. CT scan showed bowel in her chest, but on the right side. Dr. Adoph was unavailable so his partner, Dr. Rodney Steiner, was scheduled to do another repair. Dr. Steiner was no stranger to us; we got to know him well during his rounding on Jojo in the NICU.
Dr. Steiner could not go through the same incision in such a short time, so he and another pediatric surgeon, Dr. Roybal did bilateral thoracotomies to access her hernia. Dr. Steiner reinforced her first patch, and repaired a second hernia on the right side with sutures. Johanna was an undiagnosed bilateral CDH. The right side was her problem. Bilateral CDH is rare, but her doctors had never seen a bilateral or even heard of a kid presenting like Jojo.
After her discharge, we got in with a dietician to get her calories and feedings right. Jojo had lots of therapy and doctors appointments in the months after her discharge. She was on supplemental oxygen for a few months. Johanna caught up with milestones at 9 months old. She was an early discharge from her developmental pediatrician which is awesome. Jojo got her g tube removed at 18th months old. All of this is due to her mother. Johanna’s mom is a nurse and a former naval officer out of Annapolis. She busted her tail and rallied the troops to get Jojo on track. Johanna has the best mom in the world by far.
Today, Johanna is your typical terrible two. She climbs on everything! Johanna loves her big sister, watching Sesame Street, coloring, playing with play doh, dance parties with her big sister and dad, her two cats, and her dog. Her favorite foods are popsicles and pasta.
Jojo is a CDH warrior of the most legendary caliber.”
