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SALT LAKE CITY — A defect that literally unzips the diaphragm in newborn children is a scary diagnosis for many parents.
CDH occurs in approx. 1 in every 2,500 births (1,600 cases in the U.S. each year). -Cherubs
Congenital diaphragmatic hernia, or CDH, is more common than cystic fibrosis, muscular dystrophy or spina bifida — yet few people are even aware of it.
“When you look at it you think this is a really scary situation, so as a parent it was really hard to hear the diagnosis,” said Jamee Hirschi, whose son was born with CDH.
Hirschi's son James was one of the lucky ones. Following surgery at Primary Children’s Medical Center, he survived.
For some unknown reason during development in the womb, the left or right diaphragm fails to close. The colon and liver, sometimes even the kidneys and bladder, migrate into the infant’s chest cavity. The lungs are squeezed and crushed.
James Strickler knows well what happens because one of his twin daughters, Allisyn Grace, died from CDH shortly after birth.
“It’s just a random thing that happens while their diaphragm is forming," he said. "A couple of cells that were supposed to stick together, don’t. Because of that, the next two don’t stick and the next two and next two so that eventually the diaphragm just unzips.”
In Allisyn’s case, one lung was only 10 percent the size of what it should have been at birth — the other only 30 percent.
Though the Children’s Hospital of Philadelphia specializes in CDH treatment and has boosted the survival rate to 75 percent, physicians were unable to save Allisyn’s life. Survival often depends on how early or late the defect appears while the child is growing in the womb. By the time Allisyn was born, the lungs were critically damaged and underdeveloped.
“At that point, my daughter’s stomach was visible in her chest, so she had an early herniation,” said Allisyn's mother, Kathleen Strickler.
While CDH snuffed out Allisyn’s life, her twin sister, Kathryn Julene, was born a healthy, normal baby.
“Losing one of my children was the worst nightmare I could imagine in this life," James Strickler said. "I actually had to go through that. It’s the worst thing in the world. But having a healthy baby to bring home was my solace.”
“Even though we did lose Allisyn,” Kathleen Strickler said, “we did walk out of the hospital with a baby in our arms. There are many parents who have their children in intensive care units and who go home empty handed.”
At a fundraiser at the Texas Roadhouse in Logan, CDH families recently gathered as part of a state designated awareness day. Brian and Jamee Hirschi said the small incision on James’ abdomen is the only sign now that he had surgery to patch the hole in his diaphragm.
According to his mother, “It’s amazing. He’s so healthy. He’s just a big butterball. He’s tall and he’s fat and chubby — and he’s got lungs.”
Like other parents, the Hirschis had never heard of CDH. When Brian broke the news to his wife, recovering at the time from a cesarean-section, “that was one of the worst moments of my life when I had to go into the room and tell my wife we don’t know what is happening to our baby. He might not make it. Though it was very difficult to break the news, luckily, in our case, it turned out.”
Life or death, CDH families all experience the same scary news when it happens.
The Hirschi and Strickler families, each with different outcomes, now share a common bond. Both are crusading to make more people aware of what CDH is all about. The fundraiser at the Texas Roadhouse was only the beginning.
Roughly 50% of babies born with CDH do not survive. Most of those who survive endure long hospital stays, feeding issues, asthma and other problems. -Cherubs
James Strickler said if funding can support more research to detect CDH early in the pregnancy, the day will come when no parent will have to lose a child to this defect. So far, experience has shown that correcting the defect has been most successful after the child is delivered and before the lungs have been damage beyond recovery.
According to Jamee Hirschi, the bond is like an instant love. “My heart breaks to know that we have our child living while they don’t. It’s hard, but it helps to know that we both have gone through the same thing and have the same goal — to increase awareness.”
A temporary fund has been set up to support more research. Those wanting to donate for CDH research can go to www.allisyngrace.org. Funds can be donated through the site until April 15.