To most people it seemed the sad but certain end to the saga of the Lakeberg Siamese twins. Born joined at the chest with a fused liver and shared heart, they were separated last August at seven weeks of age in a controversial procedure that sacrificed one sister, Amy, so that the other, Angela, might live. The chance of success -- widely reported to be just 1% -- and the projected $1 million bill for the infants' care ignited a national debate over the limits of medical intervention. Now the Lakeberg girls lay reunited in death. A tragedy, surely, but not a surprise.
And yet to the people who knew best -- her family, doctors and nurses -- Angela's death on June 9 was sudden and unanticipated. In fact, says Dr. Russell Raphaely, director of critical care at Philadelphia's Children's Hospital, until her final respiratory illness, he would have estimated "a better than 95% chance" that she would leave the hospital a healthy child. Says Angela's mother Joey, 25: "We thought she would be home this summer." Far from the tortured existence that many predicted, Angela's brief life was largely free of suffering. Repairs to her heart had rendered it fully functional. Her chest was somewhat misshapen but healing well. Angela did not spend her days entangled in tubes and wires. She needed no sedatives or painkillers or emergency trips to the operating room.
Ensconced in the hospital's cardiothoracic intensive-care unit, the infant recovered quickly from the 5 1/2-hour operation that separated her from her twin. One week later doctors removed the breathing tube that connected her to a respirator. But since her lungs were still weak from surgery and congenital problems, they placed her in a negative-pressure ventilator. The cylindrical device works like an iron lung, enclosing the body from the neck down in a vacuum, so that air flows through the nose and mouth and into the lungs without the effort of inhalation. Over the next months, Angela's caretakers began the process of weaning her from the machine. But in the meantime she was fed her baby formula through a thin nasogastric tube so as not to interfere with her breathing.
By late fall she was able to leave the ventilator for short periods to be held and rocked by nurses. Therapists manipulated her mouth to try to teach her the motions of sucking and eating, and eventually began feeding her tiny ! amounts of pureed food. They played grabbing and reaching games to help her learn to sit up. In January doctors discovered an obstruction in a pulmonary artery and inserted a small tube to widen the passage.
Joey Lakeberg made half a dozen trips to Philadelphia to see her red-haired, blue-eyed daughter, but financial constraints and marital problems kept her in Indiana much of the time. On her most recent four-day visit in April, Joey spent time holding Angela, blowing her kisses and trying to teach her to wink. Angela's father Kenny saw his child only twice.
A ne'er-do-well who outraged relatives and the public by using money donated for the twins' care on fancy restaurants, a car and cocaine binges, Kenny has spent much of the past year in jail. He was in a drug-rehabilitation center when Angela died; hours after her death he was arraigned on auto-theft charges.
In the parents' absence, volunteers and hospital staff provided nurturing. "People brought her presents and visited with her Christmas Day," notes surgeon in chief Dr. James O'Neill, who separated the twins. Nurses bought her clothes, which they laundered themselves. They read books to her and stroked her cheeks, and Angela returned the affection. "She would blow kisses," says nurse Maryann Izzi, who has two new dresses at home intended as gifts for Angela's first birthday. "If you walked up and said her name or if you were someone she recognized, she would have a smile or a laugh. And if you mimicked giving a kiss, she would try to pucker up."
Angela delighted in baths when she was out of the ventilator and could splash freely. "We'd play the sound track to Grease at bath time," Izzi recalls. "She was smiling and laughing, and we'd dry her off and hold her a little while, just to get some human contact." She adored watching videos, especially Beauty and the Beast and Aladdin. "She wasn't really into Barney," observes Izzi. A lollipop was a special treat: "She would stick her tongue out, and we would rub it against her tongue."
Angela learned a clever trick to get attention. She knew that if she pulled the cardiac sensors off her chest, alarms would ring and nurses would come running. By April, Angela was playing outside her ventilator for "sprints" of two to four hours several times a day, and once for as long as eight hours. Though she remained a little under the average weight for her age, so steady and secure was her progress that the medical team cut back its daily * discussion of her case to a weekly assessment and predicted that she would be going home by early fall. In May, though, things suddenly changed. Angela caught a simple cold, the kind that she had overcome before. But then her temperature soared. On Wednesday two weeks ago, she began having trouble breathing. Doctors suspected she was developing pneumonia.
It turned out to be a more complex problem. A blocked vessel was keeping blood from flowing from the heart to the lungs, and the fluid was backing up into the heart. Angela lost consciousness. Doctors managed to bring her back two or three times, but at 1 a.m., in the room where she had lived for 10 months and had just cut her first tooth, she died.
Angela's death renewed the debate over whether her doctors had made the right choice in attempting such heroic surgery in the first place. Doctors at Chicago's Loyola University Medical Center, where the twins were born, had advised against any intervention. But Philadelphia's O'Neill insists that the child had a reasonable chance of recovery. "We never believed that it was a 1% chance. If we thought that it was not a reconstructible heart except for a snowball's chance in hell, we would have advised against it. We take long odds every day, but not crazy odds." Others agree. "The tragic end result does not mean that the right decision was not made from the beginning," says Mark Siegler, director of the MacLean Center for Clinical Medical Ethics at the University of Chicago. "The baby's clinical course supports the original judgment that this was a case they could deal with."
Dr. Jonathan Muraskas, the Loyola neonatologist who had advised the parents to "let nature take its course," admits that he devised the 1% figure only to put the situation in simple terms and "to try to make the family agree with us." Muraskas, who served as the girls' godfather, says Kenny Lakeberg's drug problems and the nature of the family entered into his thinking: "You have to ask yourself if chain-smoking parents in a trailer park is the most conducive environment for a sick child." O'Neill feels strongly that such considerations have no bearing on the decision to offer treatment; he also disputes the notion that the cost of Angela's care was out of line. Dr. Alan Fleischman, professor of pediatrics at New York City's Albert Einstein College of Medicine, agrees. "Interventions of this kind for newborns with congenital anomalies are far from rare in the U.S.," he says. "Expenditures of $1 million are hardly rare."
Maybe so, but it still begs the question of whether society can afford such astronomical bills for single patients. The Philadelphia team has no doubts that it was worth it. The lessons learned will help other babies survive. And Angela lived long enough to learn to laugh. "I'd do it all over again," says Joey Lakeberg. "There is no price on life."