Advanced Fetal Care Center

In mid-2002, with a beautiful and energetic six-month-old Emma bouncing in her lap, it was hard for Michelle to remember feeling devastated.

Naming the problem

The Keenes' story began when Michelle was 21 weeks pregnant. An ultrasound at nearby Brockton (MA) Hospital indicated a defect, so Michelle's obstetrician immediately referred her to the high-risk obstetrics unit at Beth Israel Deaconess Medical Center in Boston. More tests revealed that the fetus had a congenital diaphragmatic hernia (CDH).

With the anomaly named, the Keenes learned that a CDH is a hole in the diaphragm, the breathing muscle that separates the chest cavity and the abdominal cavity. The size of the hole varies by individual - some are so large that it appears as if the diaphragm is missing completely. A CDH is most common on the left side of the body. When a CDH is present, the contents of the abdomen, including the stomach, intestines, liver and spleen may move through the hole into the chest. This abnormal movement prevents normal development of lung on the side affected, and may impact the lung on the other side. With underdeveloped lungs, the newborn with CDH is unable to breathe adequately.

The Keene baby had a moderately sized CDH and although most of the contents of the abdomen had moved, less than half the liver had passed through the hole. In addition, a heart test called an echocardiogram showed that the heart had shifted to an abnormal position, but the chambers and vessels were still growing. Magnetic resonance imaging (MRI) tests also showed some lung development on the left side.

"In a few days, we went from being about 70 percent negative to being about 70 percent positive," says Michelle. With growing hope, the Keenes needed a plan. Within a day, at the urging of Bill's aunt, a staff member at Children's Hospital Boston, the Keenes had an appointment at the hospital's Advanced Fetal Care Center.

Throughout the day, the Keenes listened to the input of Luanne Nemes, RN, MS, PNP, AFCC nurse practitioner and first contact for families, Russell Jennings, M.D., surgeon and AFCC Director, and AFCC pediatric radiologists Carol Barnewolt, M.D., and Judy Estroff, M.D., Co-directors of Fetal Imaging. When all the information was considered, the Keenes decided to follow the advice of the AFCC staff and deliver the baby via EXIT to ECMO.

The non-traditional birth was scheduled for December 18, 2001. With hospital administration and state regulatory agreement, Michelle was officially admitted to Beth Israel Deaconess Medical Center (BIDMC), but then came to Children's Hospital Boston to deliver.

The specialized series of procedures used to address the Keene baby's CDH is called EXIT to ECMO. With ex utero intrapartum treatment (EXIT), the fetus was partially delivered through C-section, but the umbilical cord was left attached. AFCC physicians then connected the newborn to extra corporeal membranous oxygenation (ECMO), a heart and lung bypass system that circulates oxygenated blood throughout the body. Once ECMO was functioning, the umbilical cord was cut.

As Michelle was transported back to BIDMC to recover, baby Emma was on her way to surgery, with Bill nearby. It was difficult for Michelle not to see her baby immediately. "She was born on Tuesday. I saw pictures on Wednesday. Then on Thursday, I saw her. It was amazing."

During the time immediately after birth, surgeons stabilized Emma and performed surgery to repair the CDH. Bill stayed with his daughter as she recovered on ECMO. The Keenes tried to prepare themselves for the experience of seeing their baby connected to the intimidating machine, so they had visited another child on ECMO at Children's before Thanksgiving. The Keenes also were happy that the AFCC staff worked hard to keep parents informed and prepared, so they weren't completely shocked by her critical condition.

"Nothing could have totally prepared us for seeing Emma in the condition she was," says Bill, "but Dr Jennings and Luanne definitely did not leave out any details in explaining to us the procedure, potential curveballs and the most likely path to recovery. We had numerous meetings leading up to her birth with them and they couldn't have been any nicer."

"It was especially hard for Bill the first few days," says Michelle. "Emma was so swollen and there was a lot of equipment. Her incision had to be left partially open to allow room for the organs, which were swollen." Emma remained on ECMO for four days, but that was only the beginning of what turned out to be a 1 1/2-month stay at Children's Hospital.

The first two weeks after the initial surgery, Dr. Jennings did two more surgical procedures to close the CDH incision. Surgery number three was needed to repair a pyloric stenosis, a narrowing of the part of the stomach that leads into the small intestines. During this procedure, gallstones, most likely the result of IV feeding, were discovered, so Dr. Jennings removed the gall bladder. Finally, Emma needed one more surgery to correct a common condition called gastroesophageal reflux, an irritation caused by the splashing or "reflux" of acidic stomach contents up into the esophagus.

Grandparents and other family members helped Michelle and Bill as they juggled time with Emma and work. Michelle missed only a handful of workdays during Emma's stay, striving to save her time off for when Emma came home. The Keenes say they were thankful for Emma's grandparents who would relieve them and put Emma to bed in the hospital. But they point to another important team. "For those few months, the Children's Hospital team was our family."

"Our family and friends were tremendous throughout the whole hospital stay. I don't know what we would have done without their love and support," observes Bill. "As far as the staff at Children's Hospital, I cannot say enough about how wonderful they all were. They really did become our second family. I actually miss seeing them. We went there every day for four months and it became our second home."

Moving in and out of the intensive care unit, the Keenes tried to take it all in stride. "I teased Dr. Jennings that he would have to send us home because Emma didn't have any more organs for him to remove or touch," chuckles Michelle.

After a consult with an AFCC-affiliated neurologist about possible future neurologic concerns about the effect of ECMO and other surgeries, the Keenes were released from the hospital on April 15, 2002. They planned a follow-up meeting with an AFCC-affiliated geneticist to better understand their families' medical history and how it can affect their health.

In June 2002, Emma was eating solid food and the Keenes were looking forward to final removal of her feeding tube by her first birthday. Michelle describes her daughter as happy and strong-willed. "At first, we asked 'why us?'" says Michelle. "But seeing her taking the strides she did during those difficult months - it made us keep going, too."