Luke’s Story
O Warrior Stories
Written by: Faith L’Esperance
Diagnosis (September 2016, Rochester NY)
Our “O” story begins like so many others: we first heard the word “omphalocele” during our anatomic ultrasound at 20 weeks. Multiple professionals filed into the room and spoke to us about the abdominal wall defect, the curve in his spine, and their inability to see the fourth chamber of our baby’s heart. They suspected a diagnosis of Trisomy 13 or 18, and advised that we get genetic testing done quickly because we only had a few weeks left to terminate the pregnancy if we so chose. We were devastated.
We were not given any information about the omphalocele itself, or pointed in a direction to find any. Weeks later, as we waited for bloodwork and appointments began to stack up, I discovered “MOO (the Mothers of Omphaloceles Facebook group)” that we received any hope at all about the diagnosis and our son’s future.
Preparation -
The remaining 19 weeks of my pregnancy were well monitored, and we gradually got more information about what we were dealing with. Luke’s omphalocele was “giant,” which is how it is classified if the liver is involved. The omphalocele also made it difficult to get a proper growth measurement on him. Further ultrasounds showed us that his spine appeared normal. Multiple fetal echocardiograms found a suspected ASD that was not problematic. We met with pediatric surgery, a palliative care team, and got our grand tour of the NICU. A plan began to form and we began to have a lot of confidence in the care our son would receive after birth.
My MFM team was efficient and kind in their care for me, but, due to lack of knowledge about omphalocele, they gave zero optimism for the outcome of the delivery. My mental health was very poor during this time. Those last few months of 2016 would likely have looked very different without the tireless love, support, prayer and encouragement from my husband, my co-workers, our families and our church.
Delivery -
Luke Joshua L’Esperance was born via scheduled c-section on January 30, 2017. It was a beautiful snowy morning! It’s hard to predict how an O baby’s lungs will do after birth, and how much support they will need. He came out screaming, showing us that he was breathing on his own. The NICU team whisked him away quickly. His omphalocele was not the largest the team had ever seen, but it was pretty large! The “O” was so fragile at birth that he was taken into surgery when he was a couple of hours old. We knew we had a long road ahead, but we were blessed with so much joy that day. I had dreaded his birthday, but it was such a gift from God! It would be 9 days until we would hold him, but it was ok. He was getting everything he needed. After his first surgery, Luke remained intubated. He was extubated four weeks later, but stayed on CPAP for a time. TPN was his main source of nutrition as his body stabilized. VERY slowly, we worked up to NG feeds.
NICU (Golisano Children’s Hospital, Rochester NY) -
We can’t say enough about the excellent facility, providers and caregivers we were blessed with during our NICU stay. Though our hopes for a quicker O repair quickly dwindled, we were blessed enough to have a private room during those long days. His nurses were some of the best people on Earth, and they helped us grow into confident advocates.
Luke had a wound vac for the first five to six weeks. This method seemed to work pretty well, but he developed a yeast infection and that plan was scrapped. We met with a wound specialist and developed a method for “dressing” the o as it gradually developed skin coverage. We tentatively planned for repair around two years. That time frame was very disappointing at the time, but it would not have been safe to attempt any kind of closure when he was that small. Luke needed time to heal and grow.
At eight weeks old, we learned that Luke had hydrocephalus. One week later, he had a VA shunt placed (a VP placement would have been preferable, but it was not possible due to the omphalocele and the plans to repair it). While this diagnosis was shocking to us, we believe in God’s perfect will for Luke’s life even though this was not something we would have chosen for him. It is important for other parents reading this to know that we are told this was unrelated to the other physical issues we were already dealing with. It’s just something that happened. Luke recovered very quickly from his first neurosurgery, and we started to look towards doing omphalocele wound care at home and learning to live as normally as possible with hydrocephalus. One last NICU setback: Luke developed a hernia less than two weeks before discharge. This is very common in O babies, and it was dealt with quickly. Finally, after 94 days and three surgeries, Luke went home on Star Wars Day!
Home adjustment/equipment
Medically, he had a lot going on when we got home but the Facebook group had definitely prepared us for that. He began to receive physical therapy and speech therapy for feeding shortly after we came home. His respiratory rate was pretty high, and he was still on a small amount of oxygen at time of discharge. He was not a g-tube candidate, so Luke was fully NG
fed for the first year.
. Growth and time were the biggest factors in becoming independent of extra O2. He came off it during the day around 7 months old, but needed it overnight until just under one year of age. There wasn’t much we could do other than keep him healthy and wait. The NG tube, however, took a lot more work on our part. As with many parents, The “Growing Independent Eaters”
Facebook group was a lifesaver! We did not end up officially working with them, but received so many tips and advice from their social media pages, groups and families who had already gone through it. It took a lot of work, patience, and prayer, but at 13 months old Luke went off the tube for good!
Luke’s suspected ASD (we were never entirely sure) closed on its own after the age of one. He has an interrupted IVC, but it has always been more of a “curiosity” than anything. We have had zero issues with the function of his heart.
Repair (Children’s Hospital of Philadelphia, September 2021) -
Luke’s omphalocele was not repaired until age 4. This was much longer than we ever expected, but (amazingly) we did not have any issues with it during that wait. We did daily “belly care” with aquaphor, and when he became mobile we began to support it with an ace bandage.. There are videos on MOO and YouTube that mirror how we ended up doing our own version of “wrapping” to contain. We believe wrapping the o to be instrumental in the success of his 2021 repair, despite being told it wouldn’t be effective. In 2019, he also became a big brother!
We love our team at Golisano Children’s Hospital. They saved Luke’s life, and gave us the tools we needed to become the best parents we could be to Luke. Ultimately, we chose to get a second opinion at CHOP in December of 2019. CHOP seems to be the “gold standard” for omphalocele repair, especially the larger ones where it becomes a bit more complicated. Following a confident evaluation from PEDS surgery and plastic surgery, we made the decision to proceed with tissue expanders. We were supposed to have them placed in March of 2020, but we all know what happened that month! We delayed our plans for a year during the pandemic.
In February of 2021, tissue expanders were finally placed and we began injections. We seemed to struggle a little more with expanders than other families, but it was absolutely the perfect method to achieve a safe repair. After seven months of injections and preparation, our biggest prayer was answered: Luke’s omphalocele was successfully and safely repaired. Exactly five years to the day after the omphalocele diagnosis, he was taking his first steps with a repaired belly (and a belly button)! He was inpatient for nine days as he healed and worked back up to eating enough.
Luke -
Luke is eight now, and has no memory of omphalocele life. He has been reading since age 4.5, and will start third grade soon! He loves animals, being outdoors, planes, road trips, and playing with friends. He has a great singing voice, and he is a great hiker! We deal with picky/anxious eating, and weight gain is hard, but Luke has overcome every obstacle with time. We can’t wait to see what God has planned for him!