Doctors discovered that the daughter Debbie was carrying had a right-sided congenital diaphragmatic hernia (CDH).
The baby’s diaphragm had failed to close properly, leaving a large hole inside her body that allowed her organs to migrate from her abdomen to her upper chest, severely restricting the growth of her lungs. The birth defect occurs in one of approximately 2,500 live births, and can be extremely complex to treat, requiring surgical repair soon after birth and, often, a long course of intensive care. Depending on the severity of the defect, CDH can be fatal.
“It was like boom … you got sucker punched,” recalls Debbie. “Those first 48 hours I was stunned. I think I was in shock.”
Debbie’s doctor immediately referred her to The Children’s Hospital of Philadelphia’s Center for Fetal Diagnosis and Treatment for extensive testing. She was admitted to a nearby hospital and put on bed rest for the next five weeks, with the hopes that her baby would continue to grow and be strong enough to undergo lifesaving surgery when she was born.
(Today, mothers like Debbie have an even better option of being admitted directly to the Center’s Garbose Family Special Delivery Unit — the world’s first birth facility created specifically for pregnancies complicated by birth defects.)
A roller coaster ride
On March 31, 2008, Peyton Laricks was delivered by C-section. A multidisciplinary team was standing by and immediately put her on a ventilator to support her underdeveloped lungs. At just 4 days old, Peyton underwent surgery to repair her CDH right in CHOP’s Newborn/Infant Intensive Care Unit (N/IICU), avoiding the risks of transport to an operating room. The surgical team moved her organs back in place and closed the hole in her diaphragm with a soft tissue patch, creating space in her tiny chest for her lungs to grow.
The surgery went as planned, but by the next day, Peyton took a turn for the worse. Babies with CDH are often described as among the most fragile in the N/IICU. In Peyton’s case, her weakened lungs were not functioning well enough to keep her blood oxygenated, so she was placed on ECMO, a life-support machine that does the work of the heart and lungs.
After nearly two weeks, doctors decided Peyton’s lungs were strong enough to do their job, and the baby girl was taken off life support. Another month later, she was weaned off ventilator support. After spending the first 88 days of her life in the N/IICU, Peyton was finally ready to go home to Sea Isle City, N.J.
Little Peyton came home with a lot of equipment — a nasal feeding tube, oxygen and about nine different medications. Debbie and Dustin learned to care for their baby at home with the help of CHOP’s N/IICU nurses and the Family Learning Center.
At home, Peyton made progress but also encountered setbacks. Her pulmonary hypertension (a condition that commonly accompanies CDH and causes high blood pressure in the arteries of the lungs) was under control, but she wasn’t growing because of gastroesophageal reflux disease (GERD), also common in patients with CDH. Peyton returned to CHOP to undergo a Nissen fundoplication surgery to alleviate the reflux. Doctors also replaced a feeding tube in her nose with one in her stomach.
She was in and out of the Hospital in less than a week, and hasn’t required any more surgical treatments since. The week before her third birthday, Peyton’s feeding tube was removed. Now, after a year of eating on her own, “she’s the best eater out of my three kids,” says her mom.
Peyton may be small, but she’s a force to be reckoned with. Today, she continues to beat the odds as a sweet, energetic, 4-year-old with a vivid imagination who follows her big brother Dusty around and dotes on her baby sister, Quinn.
Peyton returns to CHOP regularly for checkups through the Pulmonary Hypoplasia Program, where a multidisciplinary team keeps a close eye on the condition of the patch as well as Peyton’s heart and lung function and overall growth. Last year she started preschool part time, and she’ll be going five days a week this coming fall. “She’s just like any other 4-year-old,” says Debbie.
“Kids are so resilient,” adds Debbie. “The doctors always said she’s stubborn, doing things on her time frame, and she’s still like that. You just can’t push her. That stubbornness is what got her through those 88 days and the fight of her life. She’s a survivor.”
Peyton and her family were fortunate to have access to a hospital with extensive experience in managing every aspect of CDH successfully — from the Special Delivery Unit, to the pediatric surgical and N/IICU teams, to the comprehensive follow-up CHOP provides for CDH patients as they grow. CHOP is also spearheading research into improved treatments for CDH, including surgical intervention before birth.
From the day they left the Hospital, Debbie and Dustin vowed to pay it forward. They hope that sharing Peyton’s story will bring comfort and hope to other parents faced with a CDH diagnosis. “She was given one of the worst prognoses, and I want people to know that it’s not a death sentence,” Debbie says.
They haven’t stopped there. For the past four years, the Laricks family has been raising awareness and money to continue CDH research through Peyton’s Promise. So far they have has raised more than $100,000, funding that has directly supported CDH-focused research being conducted by Marcus Davey, PhD, at CHOP’s Center for Fetal Research.
“That’s why we started this,” explains Debbie. “The more we know, the more that can get done. Knowledge is power.”
And that means more kids like Peyton overcoming the odds.
Originally posted: September 2012