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Babies With Birth Defects Spend First Holiday Season With Families

Ruzanna Harutyunyan's picture

Just 6 months after the Garbose Family Special Delivery Unit opened, more than 75 babies have been born and are sharing their first holiday season with their joyful families. Families from 13 states and at least two other countries have traveled to Children's Hospital for expert prenatal care, delivery and postnatal care for their babies in a place where family comes first.

The Garbose Family Special Delivery Unit (SDU) at The Children's Hospital of Philadelphia is the world's first comprehensive obstetrical unit within a pediatric hospital for mothers carrying a fetus with a known birth defect. It is an innovative new labor and delivery unit for expectant mothers whose babies require highly specialized, sophisticated neonatal, surgical or cardiac care at birth. Unlike centers where mothers give birth and recover in one hospital while their critically ill newborns are transferred to a specialized pediatric facility, the SDU allows the mother and baby to stay in the same hospital with a multidisciplinary team of experts caring for both the mother and the baby.

The uniqueness of the SDU goes far beyond the geography of having the mother and baby together in a pediatric hospital. The unit staff strives to optimize and individualize the birth experience for mothers and normalize their delivery experience as much as possible during a difficult and technically challenging time for the baby. After the birth, the SDU team immediately fosters bringing the family together, encouraging parents to be with their new babies as soon as possible and will even provide some of mom's postpartum care in the intensive care units where these babies are being cared for. This helps to make an overwhelming time a little bit easier because familes aren't divided.

The SDU teams also works to help families achieve the best experience even at a time of uncertainty. Families start from the baby's birth, not from the time of discharge, and are supported by other familes on the unit, because everyone is experiencing a similar type of life changing event.

"This state-of-the-art, one-of-a-kind Special Delivery Unit provides the most personalized, comprehensive care for these families before, during and after birth," said N. Scott Adzick, M.D., surgeon-in-chief at The Children's Hospital of Philadelphia and medical director of the Center for Fetal Diagnosis and Treatment (CFDT). "Babies are delivered and treated immediately in the Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit (N/IICU) or the Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit (CICU) while the mother recovers in the same hospital."

Caoimhghin Williams, Albany, N.Y., first baby delivered in the Special Delivery Unit

The first baby born in the Special Delivery Unit, Caoimhghin Williams, arrived on Tuesday, June 10 at 9:43 a.m. During their 18-week ultrasound, Courtney and Martin Williams of Albany, N.Y. learned that their fourth son had spina bifida.

Courtney's younger sister had been born with spina bifida and she was surprised but not completely shocked with the diagnosis. Their obstetrician referred the Williamses to the Center for Fetal Diagnosis and Treatment at Children's Hospital. After a one day evaluation process with the CFDT team, they knew for certain that the baby had spina bifida and that they would need to deliver their baby at CHOP.

Courtney and Martin welcomed Caoimhghin into the world and were able to spend time with him before he went into surgery - which was necessary to close the opening on his spine. They were by his side immediately after the procedure. Courtney and Martin were thrilled to be able to go down the hall to the Newborn/Infant Intensive Care Unit to see their son the same night that he was born. The SDU team focused on keeping the family together as much as possible.

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Courtney's mother tells a truly compelling story about how Courtney's experience delivering Caoimhghin in the Special Delivery Unit is light years away from the care she received when she delivered a baby with spina bifida more than 20 years ago. The grandmother poignantly notes that she remained in her community hospital, unable to visit the hospital where her baby daughter was receiving care which was hours away from home, until after her discharge from the hospital five days later. She needed to search to find a neurosurgeon for her daughter's surgical repair and just wanted to feel like a normal mom. The SDU is exactly the experience she hoped that Courtney would have--the ability to be with Caoimhghin immediately, to know his neurosurgeon and to feel like her delivery experience was normal.

Noah Sweitzer, Liberal Kansas (now of Lock Haven, Pa.)

Tricia and Todd Sweitzer of Liberal, Kansas, found out in March 2008 they were expecting their first baby and that their due date was November 28. After learning the exciting news, the couple decided to move to Kansas from New Orleans to be closer to Tricia's family. Tricia got a teaching job and everything was working out well for the couple. On July 10 when their doctor looked at the much anticipated sonogram, he saw and knew immediately that the large tumor growing from the baby's backside was a sacrococcygeal teratoma (SCT). SCT is the most common tumor of the newborn and occurs in 1 out of every 35,000 to 40,000 live births. Most SCTs diagnosed in neonates are not likely to be malignant and the prognosis tends to be good after surgery, but very large tumors diagnosed before birth have a much poorer prognosis.

Their doctor referred the Sweitzers to a specialist in Wichita, Kan., who saw them the following week. The specialist recommended the couple see the experts at the Center for Fetal Diagnosis and Treatment at Children's Hospital. The Sweitzer's traveled to Philadelphia later that week. After a full day of tests, the Sweitzer's made a quick trip back to Kansas to pack and moved closer to Philadelphia so they could have ongoing care and deliver their son at Children's Hospital. They saw the team once a week for ongoing monitoring routinely for four weeks and the parents and team watched the SCT continue to grow.

During an appointment in late August, at the end of the sixth month of pregnancy, the team noticed that the baby's tumor was growing very rapidly and the baby was developing signs of heart failure due to the rich blood supply to the SCT. Tricia had developed a massive increase in amniotic fluid around the baby and removal of some of the fluid revealed that the huge tumor had ruptured. The baby needed to be delivered immediately.

Tricia was admitted to the Special Delivery Unit and the baby, Noah, was delivered in one of two state-of-the-art operating rooms by Ex Utero Intrapartum Therapy (EXIT). The EXIT procedure is similar to a cesarean section, except that the mother has general anesthesia to keep the uterus from contracting, that the placenta stays attached and the surgeons have time to operate on the baby. The baby was delivered from an opening made into the uterus through an abdominal incision; then, with the umbilical cord still attached and the baby still hooked up to the mom via the placenta, surgeons removed the huge tumor and stapled the wound closed. Afterward, the umbilical cord was cut and Noah was transferred to the N/IICU. At birth, Noah weighed 2 pounds, 11 ounces; the tumor weighed a few ounces less at 2 pounds, 6 ounces.

The experts at CHOP have managed more than 70 SCT cases, including five cases resected before birth, but this is the first time ever that a baby with an SCT underwent successful resection during an EXIT procedure.

Because Noah was so premature and unstable at birth and because Tricia had been under general anesthesia, she wanted to be able to see Noah as soon as possible. Just hours after her surgery, nurses from the SDU were able to reunite the Sweitzer family in the N/IICU, the night Noah was born. Mom, Dad and baby were able spend crucial time together that first night and begin bonding as a family immediately. Todd didn't have to make a choice between staying with his wife after surgery or visiting with his new son. Tricia says that it was difficult enough being away from her baby and she couldn't imagine if he wasn't just down the hall. This is the norm for SDU families, the care of the mom is tailored to the needs of the baby, so familes can be familes immediately and mom's care may happen on the SDU or in the N/IICU or CICU if necessary to achieve this goal.

An MRI in early October showed that in addition to the known residual tumor in Noah's pelvis, that the tumor had also grown into spinal canal. Dr. Leslie Sutton, chief of neurosurgery at Children's Hospital removed the tumor in Noah's spinal canal and a week later Dr. Adzick removed the rest of the pelvic tumor.

Noah has an excellent prognosis and should be a normal, healthy boy with little chance of a tumor recurrence. But the team will keep monitoring him to be sure that the tumor does not return or become malignant, which is very important and one of the reasons that early detection is essential. Noah is doing well and with his family in their new home in Lock Haven, Pa. The Sweitzers say "we are so lucky, we would never want to be anywhere else but here".

Patients are first introduced to the CFDT program during their outpatient evaluation. The outpatient evaluation and prenatal services are also contained within the Special Delivery Unit at CHOP and staffed by a dedicated team of obstetricians, maternal fetal medicine specialists, radiologists, sonographers, echocardiogram technicians, nurse midwives and nurses. When a family comes to CHOP for an initial evaluation by the Center for Fetal Diagnosis and Treatment, they have series of tests in a single day - a high-resolution ultrasound, an ultrafast fetal MRI and an echocardiogram. After the images for the different testing modalities are evaluated, the team sits down with the family to discuss the diagnosis and treatment options and a care plan. It is at this time that families may be enrolled into the SDU program if their baby will require immediate critical care upon birth.