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In utero surgery gives struggling twins a better shot at mutual survival

WHEATON FRANCISCAN HEALTHCARE

In April, 2010, Chrissy Pircio — 21 weeks pregnant with twins — found herself lying flat on her back in a rented RV as her husband and brother sped down the highway on a 10-hour trip, from her home near Pittsburgh to Milwaukee in a desperate bid to save her babies' lives.

Eight years earlier, in August, 2002, Katie Fast of San Francisco found herself in much the same situation — 19 weeks pregnant with twins and making hurried preparations with her husband to fly to Milwaukee for the same lifesaving operation.

Both Pircio and Fast were carrying identical twins, and both sets of twins were suffering from a potentially fatal disease of the placenta. After long-distance consultations, both expectant mothers were en route to Wheaton Franciscan Healthcare-St. Joseph, where Dr. Julian E. De Lia, director of the International Institute for the Treatment of Twin-to-Twin Transfusion Syndrome (TTTS), was set to perform endoscopic laser surgery that could dramatically improve their odds of delivering healthy babies.

"The most vulnerable of families come to our door with the most vulnerable of babies, often after a recommendation to terminate the pregnancy," says De Lia. "Our paradigm for TTTS includes both placental and maternal factors; we provide a comprehensive treatment plan for patients, which can reverse the perinatal mortality and morbidity for these children."

Survival in the balance
In TTTS, twins (or other multiples) share a single placenta that contains blood vessels that connect the fetal circulations. These interconnections may cause one baby, called the recipient, to receive too much blood, overloading the cardiovascular system and potentially causing heart failure. Meanwhile, the other baby, called the donor, may die from loss of blood. Two maternal abnormalities — significant protein-calorie malnutrition and anemia, and cervical insufficiency, or shortening — can contribute to TTTS morbidity and mortality as well.

Though TTTS strikes only 10 percent of identical twins, presenting at midpregnancy, the death rate without treatment may be as high as 80 percent to 100 percent. Fatalities may occur in utero, from prematurity or years later from the effects of TTTS. Survivors can suffer from many serious problems, including cerebral palsy. In the U.S., TTTS affects 5,000 babies (2,500 pregnancies) each year; worldwide, the disease strikes 200,000 twins annually.

De Lia first encountered TTTS as an obstetrics/gynecology resident in Newark, N.J., in the 1970s. "Ultrasound was in its infancy then, and resolution was so poor that you couldn't make the diagnosis," he says. "Most babies died, and more than half the survivors had neurologic deficits."

Pioneering surgery
In 1983, De Lia was working at the University of Utah Health Sciences Center in Salt Lake City when he diagnosed TTTS in a patient at 22 weeks gestation, through much improved ultrasound technique. By then, it had been documented that heart medicine given to a mother could pass through the placenta to a fetus to correct heart failure. Working closely with a pharmacologist at the university, De Lia treated his patient with digoxin, which improved the babies' situation. The twins were born at 33 weeks and survived.

"Looking at this placenta, I suspected that by using a laser in a small scope and inserting it into the uterus, I could find and destroy the problematic blood vessel connections responsible for the syndrome," he recalls.

De Lia devised an instrument — a laser married to an endoscope — and began experiments on sheep and monkeys to prove he could cauterize blood vessels without harming mother or fetus. Within five years, he had pioneered the surgical technique and began operating on obstetrics patients suffering from TTTS.

The operation has dramatically altered the odds for twins with TTTS. In 90 percent of cases in which the operation is performed, at least one twin survives; in two-thirds, both twins live. Less than

4 percent of survivors have any disabilities or birth defects. Healthy infant survival is also maximized in TTTS patients by treating co-occurring cases of cervical insufficiency with cerclage, or cervical stitching, and malnutrition with aggressive nutritional therapy and bed rest.

Compassion on-call
Those kinds of odds are what bring families like the Pircios and the Fasts to Wheaton Franciscan Healthcare-St. Joseph from all over the country. A number of health care centers in the U.S. now offer similar fetoscopic laser occlusion surgeries; and De Lia continues to consult with physicians and families nationwide — as well as in 58 countries around the globe.

In fact, De Lia's far-reaching reputation is what caused him to relocate his practice from the University of Utah to the Midwest in the 1990s. In 2000, he launched the International Institute at Wheaton Franciscan Healthcare-St. Joseph — the highest birth volume hospital in Wisconsin.

"One of the hallmarks of TTTS is that the mother develops a very large, rapidly growing uterus due to excess amniotic fluid, making travel risky," he explains. "I needed a central location so that couples could reach me easily by car or plane. We have access to three airports with lots of direct flights — two in Chicago and one in Milwaukee."

Yet patients say they seek out De Lia for more than his geographic accessibility or even his expertise at TTTS surgery. His personal attention and compassion at a time of desperation are legendary.

Patron saints
"I knew I was having twins at five and a half weeks. At 20 weeks, I was looking forward to my ultrasound because I would learn the gender of the babies," recalls Pircio. "Instead the twins were diagnosed with TTTS, and I was rushed to a hospital for amnioreduction the next day."

Frantically searching the Internet for information, Pircio's husband, Francesco, came across an article that mentioned De Lia's pioneering surgery. The couple immediately called De Lia's office — despite the fact that it was a Saturday night.

"Unbelievably, Dr. De Lia called us at 7 a.m. Sunday morning and talked to us for 20 minutes about the situation. He wasn't sure it called for surgery yet, and asked me to go back for a checkup the next day and then call him again," says Pircio. "That Monday, I learned that the problem had progressed and the twins were now in kidney failure and heart distress. We made immediate plans to drive to Milwaukee."

Pircio says De Lia kept in touch by cell phone during the long trip, even suggesting an alternate route to avoid rush-hour traffic on the way to the hospital. She arrived at 10 p.m., bedded down for the night and met De Lia the next morning.

"He showed me videos of other surgeries so I would know what to expect," says Pircio. "Then he held my hand as I was prepped for surgery, and told me he kept prayer cards for St. Gerard and St. Jude, the patron saints of pregnancy and hopeless causes, in the operating room.

"The next thing I knew, I was in the recovery room," she recalls.

After five days in the hospital with clinicians monitoring the babies' conditions, the Pircios returned to Pittsburgh. There Chrissy Pircio remained on bed rest, drinking Ensure shakes several times a day and checking in with De Lia on a weekly basis. Despite a preterm labor scare, her twin boys, Alessandro (Alex) and Giovanni (Jojo) were born July 28, 2010 - at 36 weeks gestation and completely healthy.

Saving lives
Katie Fast had an equally happy outcome to her pregnancy: the birth of healthy twin daughters, Sara and Sophie, at 37 weeks gestation.

"I owe my beautiful family completely to Dr. De Lia," Fast says of her now-8-year-old daughters.

When Fast learned her babies had TTTS during a routine ultrasound at 17 weeks of pregnancy, she followed her own doctor's recommendation and called De Lia. He performed the cauterizing placental procedure about two weeks later, telling Fast and her husband, Spence, that on a scale of one to 10, the results were a 12.

"He gave us so much confidence," Fast says. Back in San Francisco, Fast's labor and delivery were blessedly routine.

"We are still in contact with Dr. De Lia. He visits us when he comes to California, and we see him when we are in Milwaukee visiting family there," says Fast.

As for De Lia, he says his work fulfills not only the ministry of the Wheaton Franciscan system - "to provide exceptional and compassionate health care services that promote the dignity and well-being of the people we serve" - but also his own personal ambition.

"When I was a student, I thought about becoming a neurosurgeon and saving lives," he says. "Now I know that every time I perform this endoscopic procedure, which lasts 10 to 20 minutes, I have a chance to save two babies who will be born without neurologic deficits and can live an average of 75 years each."