Children who are survivors of a life-threatening congenital defect called congenital diaphragmatic hernia (CDH) after being treated at UCSF's Fetal Treatment Center were reunited last Friday.
Seventeen former patients and their families attended the reunion, held in the CDH clinic at the Parnassus campus. That was a good turnout, since the Fetal Treatment Center sees only about 60 such patients each year.
"All the doctors and his primary care team of nurses were amazing," said Michele Maxwell of Riverside, whose son Mason was treated for CDH through the Fetal Treatment Center in 2000. "We have bonded with a lot of people, so coming up is like coming back to family."
CDH occurs when the diaphragm - the wide, flat muscle that separates the abdomen from the chest - doesn't form properly, leaving an opening through which the contents of the abdomen can migrate. CDH occurs in an estimated one in 2,500 live births and accounts for about 8 percent of all congenital anomalies.
With the contents of the abdomen spilling into the chest cavity, the intestines, liver, spleen and stomach can crowd the lungs and inhibit their growth and development. The condition threatens the chances of survival before and after birth. All babies require surgery to correct CDH and outcomes vary, depending upon the severity of the defect.
Maxwell said she felt as though the "wind had been knocked out me" when Mason was diagnosed with CDH at 25 weeks gestation. Although she was being cared for by an obstetrician in Southern California, when doctors there learned of Mason's condition, her family was immediately referred to the UCSF Fetal Treatment Center.
"San Francisco was the place to go," said Maxwell, who had been told by two prior physicians that her son had only a 50 percent chance of survival. The message at UCSF was a little different, she said.
"'This is really, really bad, but we have options and we are here for you,'" she recalls being told.
Mason was born at 37 weeks gestation - three weeks shy of a full-term pregnancy - and, at just 6 days old, required surgery led by Fetal Treatment Center founder and Director Michael Harrison, MD. The now 6-year-old boy has required subsequent surgeries to repair the hernia and insert a feeding tube, Maxwell said.
Like many CDH patients, he also has experienced residual effects of his prematurity and the CDH. He has developmental delays and hearing loss that requires the use of hearing aids in both ears. (It is not known why children with CDH suffer hearing loss.)
UCSF's Fetal Treatment Center is one of the leading centers in the nation for treatment of CDH, according to Barbara Bratton, RN, nurse manager for the CDH clinic. But the clinic's multidisciplinary approach to treatment is practiced at perhaps only three centers in the Americas, including one on the East Coast and one in Toronto, Canada, Bratton said.
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Michele Maxwell and her son Mason pose with his aunt, Marisa Rondeau, at a reunion last Friday at UCSF. |
"Unfortunately, some of these children have very little interest in eating by mouth. Some have impaired hearing and delayed speech. They can suffer from delayed development, breathing problems and digestive problems. However, the most worrisome issue is that the hernia can recur, so we schedule regular X-rays and offer anticipatory guidance about symptoms," Bratton said.
But at the UCSF CDH clinic, patients may see a host of specialists in one place at one time to treat their child's multiple health care needs, easing the burden on parents who would otherwise have to make multiple, separate clinic appointments to ensure their child received adequate care, she said.
Patients at last Friday's clinic might have been seen by a pediatric nutritionist to address ongoing feeding issues that arise from CDH, as well as by a neonatologist, pediatric surgeon, pediatric gastroenterologist, feeding therapists and developmental therapists, among others, Bratton said.
Paul Berghoff and his son Andrew wanted to return to the Fetal Treatment Center for the reunion, even though the journey was a long one - in more ways than one, he said.
"It was very hard," Berghoff, who lives in Chicago, said of learning of his son's CDH.
Andrew was born at full term in 1999, and one week later, received surgery led by Harrison to correct his CDH. He was born at the end of September and was hospitalized until the day before Thanksgiving. Ironically, Andrew Berghoff and Mason Maxwell have the same birthday, but were born a year apart. The two families have remained in contact with each other.
Berghoff said the experience "scared me to death." But since then, Andrew's condition has continued to improve. He has had feeding problems, and until recently was fed through a tube in his stomach.
Because of his attendance at a "refeeding" clinic in Baltimore on Saturday, Andrew Berghoff was able to eat yogurt with a spoon while his father looked on. It was something that most parents take for granted, but a small miracle for the 7-year-old Berghoff.
His father had nothing but praise for the Fetal Treatment Center Team. "The caring is so great," Berghoff said. "Jody Farrell [of the Fetal Treatment Center] is such a calming, sweet influence. No one could be more patient and sincere.
"It was easy to know that we wanted to come here for our care," he said.
Related Links:
The Fetal Treatment Center at UCSF
Children's Hospital Welcomes Babies, Families to Reunion
UCSF Today, October 24, 2006
