FOX19 Change the Outcome Series

Congenital Diaphragmatic Hernia: Surgery Saves Abram's Life

"He definitely is a miracle."

Abram Friend was born with a rare condition called congenital diaphragmatic hernia (CDH), where his insides were protruding into the left side of his chest cavity. He had surgery to create a diaphragm from stomach muscle when he was only 1 week old. Today, Abram is on the road to a healthy, normal life.

Abram Friend is well known in the Regional Center for Newborn Intensive Care (RCNIC) unit at Cincinnati Children's Hospital Medical Center. He began seeing doctors here 10 weeks before he was born.

Abram's mom, Tiffany, was doing all the things first-time mothers-to-be do, enjoying baby showers and preparing for her precious arrival. But 24 weeks into her pregnancy, Tiffany had an ultrasound scan that failed to show her baby's heart. Tiffany and Mark Friend's joyful anticipation suddenly turned into fear that their baby boy may not survive.

A Life-Threatening Diagnosis

Abram was diagnosed in-utero with a condition called CDH, congenital diaphragmatic hernia. "Basically he was born with his insides herniated into the left side of his chest cavity and he had to have surgery to survive, to breathe," said Tiffany.

A specialist near their home in Troy, Ohio, referred the Friends here to Cincinnati Children's -- home to both the Fetal Care Center of Cincinnati and a level three RCNIC unit, providing the highest level of care in our region for critically ill newborns. "Several people suggested Cincinnati Children's and we met with Dr. Crombleholme of the Fetal Care Center at Cincinnati Children's and his team," said Tiffany.

The team includes Timothy Crombleholme, MD, director of the Fetal Care Center of Cincinnati and the Center for Molecular Fetal Therapy; Jon Fridriksson, MD, medical director for the RCNIC; and Beth Haberman, MD, associate director for High-Risk Infant Follow-up Clinic.

"CDH is a fairly rare disorder. It happens in about 1 in every 2,500 births. An average-sized center across the country only takes care of 8 or 10 of these kids a year," said Dr. Haberman. "We developed this team in 2002. We do a multidisciplinary approach by having both medical and surgical specialties, nursing, therapists, pharmacy. Everybody has equal input into how we make decisions for those kids. The numbers show that we have significantly improved outcomes."

"Prior to 2002, our outcome for isolated CDH was about 50 percent. Since then our numbers are 85-90 percent for isolated CDH in terms of survival," said Dr. Haberman.

The team prepared Tiffany and Mark for what would happen moments after their son was born. "They showed us around the RCNIC. We met several RCNIC nurses during our tour. They prepared us. 'This is where your baby will be,'" said Tiffany. "They walked us through each process and through the testing we were able to see how severe his condition was, and Dr. Crombleholme was very thorough with us on everything."

"Part of the whole concept of fetal therapy is the idea that this is prenatal pediatrics. You shift the time frame when a baby becomes a patient from the delivery room to when you can make a diagnosis with an ultrasound," said Dr. Crombleholme.

An Early Arrival

Abram Friend was born at University Hospital, a few weeks premature, on November 18, 2005. He weighed 6 pounds and was 19-1/4 inches long. He was rushed to Cincinnati Children's before his parents could even hold him. "After his delivery there was a whole team that was there in delivery room and took him immediately," said Mark, Abram's father. "I was able to go with them in the ambulance to Children's, straight to Children's. They worked on him to stabilize him because he did have severe breathing difficulty."

"When these babies are born often they have insufficient lungs to support them and even with every ventilatory management strategy we have, some of these babies do not survive," said Dr. Crombleholme. "In Abram's case, there was herniation of small and large bowel and liver into his chest, which was shifting the heart into the right chest. So the heart was plastered into the right chest wall. The intestines and liver compress the lung and prevent it from developing normally."

"It was very traumatic to see all that reality of it. And I waited in the waiting room for six hours before I was able to see him while she (Tiffany) was in the other hospital," Mark said.

Abram had his first surgery at Cincinnati Children's on November 25, 2005. He was 1 week old. The goal was to use stomach muscle to create a diaphragm. "There's a critical amount of decision making that goes into the timing of surgery for CDH," said Dr. Crombleholme. "If you operate too soon you can make the baby worse."

"The operation that is done is a large incision is made in the baby's abdomen. In Abram's case we had a large hernia so there was a lot of small bowel, large bowel, stomach, spleen and liver up in the chest so all of that has to be brought down. And then you have a defect there because the diaphragm didn't develop normally. All you have is a tiny little rim," said Dr. Crombleholme. "If you view the defect as a circle, just a tiny portion of it has any muscle residual of what would have been the diaphragm. So the options are to use a prosthetic patch or use Abram's own tissue. The abdominal wall has three layers to it. We take the innermost layer, make an incision lower down in the abdomen, split off that innermost layer and it comes down like a garage door closing. We sew that to the chest wall and it recreates the diaphragm."

Abram came through surgery, but he was being monitored so closely that he was covered with tubes and wires, which meant his parents didn't get to hold him until December 5 -- nearly three weeks after he was born. Abram and his parents remained in Cincinnati Children's through Christmas awaiting his second surgery.

"About two-thirds of babies with diaphragmatic hernia have gastroesophogeal reflux disease (GERD). This is, just as we experience heartburn, when gastric content refluxes up into the esophagus. In Abram's case, try as we may, we couldn't feed him without significant reflux and we knew he was going to get into trouble," said Dr. Crombleholme. "And so when we had exhausted all the medical therapies, we counseled Mr. and Mrs. Friend that the best option was to do a Nissen fundoplication, which wraps the fundus of the stomach around the esophagus so you have a one-way valve. Food and formula can go into the stomach but gastric content can't come back out. So it protects the baby's esophagus, the baby's airway, and allows them to grow and thrive."

Ten days after his second surgery, January 26, 2006, it was finally time for Abram Friend to head home. His proud papa posted the following entry on their Cincinnati Children's CarePage, a free, private and personalized web page that allows family and friends to stay in touch before, during and after hospitalization:

Tiffany and I want to thank everyone for your thoughts and prayers. You never know how many good friends and family you have until you're in a crisis. Please understand that Abram will have to be isolated from many visitors because of susceptibility to colds, flu or RS virus. Any one of these could put him back in the hospital. He will be going home with a feeding tube until he's able to eat on his own. This will ensure that he gains weight. Thank you all very much and we will be home shortly.

A Frightening Setback

But that long-awaited, happy trip down this road to their home in Troy was short lived. Less than a week after Abram finally arrived home, Tiffany and Mark faced their scariest moments yet in their son's short life.

"We were on our way to the pediatrician and were a few houses down the road. Abram turned blue and I told Mark to pull over. As soon as I took him out of his car seat, blood came out of his nose," Tiffany said. Mark and Tiffany called 911.

"Mark did CPR on him and saved his life until the ambulance came. They transported him to the local hospital. They Care-Flighted him back down to Cincinnati Children's," Tiffany said. "There was no one as familiar with Abram as Dr. Crombleholme and no one else was going to see him again and do his surgery."

On January 31, at 11-1/2 weeks old, Abram would undergo his third surgery. "We had known Abram had something called a broncho-pulmonary sequestration, which is an abnormality of the lower left lobe, where the lung tissue had not developed normally," Dr. Crombleholme. "And our plan had been all along to remove this but we wanted to wait until he got older and bigger and stronger and there was minimal risk to waiting that interval – or so we thought. And so Abram had his third surgery to go in his left chest and remove this hybrid Congenital Cystic Adenomatoid Malformation (CCAM) lesion."

Changing the Outcome for Abram

Abram made his second trip home 10 days after his third surgery. "We think Abram will be a healthy boy, we don't have any doubts about that," said Tiffany and Mark. "We've been very blessed. We've met wonderful doctors and we can't say enough about them. We consider them our friends. They're very honest with us and we were very fortunate to be where we were. We may not have had this result without the doctors and nurses and caretakers and everyone at the hospital."

One of those nurses was Jessica Raglin who's been with Children's for 2-1/2 years. "I think the hospital in general and the RCNIC very specifically really work on being very family-centered to make sure there are people who can support the family in every way," said Jessica.

"What I think really sets us apart is the teamwork involved between multiple specialties. We talk about Abram. He had the involvement of genetics, internal fetal medicine, cardiology, neonatology, pediatric surgery, physical therapy, occupational therapy, operating room nurses, labor and delivery nurses, RCNIC nurses, pediatric radiology and the list goes on and on and on," said Dr. Crombleholme.

"He's going to know all about his wonderful doctors and all the people who helped get him on the road to a normal life," said Tiffany and Mark. "They definitely changed the outcome for Abram."

"There's a reason people work in a center like this and it is for these kids. The idea that by working collaboratively we can actually have an impact on how these kids will do not just today and tomorrow but for the rest of their lives and that's a rare privilege," said Dr. Crombleholme.