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THE NEW YORK TIMES
2 Babies, 1 Heart And 1 Chance At Survival
by DENISE GRADY
August 10, 2000
Surviving twin, 14-month-old Darielis Milagro lives with her parents in Paterson, N.J.
When Sandra and Ramón Soto, a couple in their 20's from Puerto Rico, called Children's Hospital in Boston last year, it was to seek help for a desperate accident of nature. Mrs. Soto, a special-education teacher, was pregnant with twins. But the two tiny girls were fused at the chest and abdomen, locked in the classic embrace of Siamese twins. And only one had a heart.
Doctors in Puerto Rico and even the Sotos' own families had urged Mrs. Soto to end the pregnancy, but the couple, deeply religious and eager to have children, rejected that advice.
"We decided to fight for our babies," Mr. Soto said.
It was the beginning of a medical odyssey, part adventure and part ordeal, for the determined couple and teams of doctors who helped them, from two Boston hospitals and half a dozen medical specialties.
It soon became clear that one of the infants was doomed and that it would take complex surgery -- meticulously planned and perfectly performed immediately after the babies' birth -- to save the other. Over the next few months, the doctors planned for an operation that would require eight hours.
But the plan nearly became useless when Mrs. Soto suddenly developed life-threatening high blood pressure, requiring an emergency Caesarean section, which left the pediatric team only 90 minutes to assemble.
In the end, the gamble worked. Today the surviving twin is a healthy 14-month old, with huge brown eyes and an impish grin, living with her parents in Paterson, N.J. Her name is Darielis Milagro -- Milagro for "miracle."
Her birth and treatment cost more than $500,000, partly paid by Medicaid programs in Massachusetts and New Jersey and the rest absorbed by the hospitals.
The operation, performed in May of 1999, was not disclosed publicly at the time but is being described today in The New England Journal of Medicine by Dr. Steven Fishman and a team of eight pediatric heart specialists, obstetricians and radiologists from Children's Hospital and Brigham and Women's Hospital, where the babies were delivered.
Mr. and Mrs. Soto decided to talk publicly about their experiences because, Mr. Soto said, "We want other parents with this problem to try to save their kids." In an interview this week at the couple's apartment, Mr. Soto spoke with a reporter in both English and Spanish and translated for his wife when necessary.
Soon after learning that their daughters were conjoined and sharing one heart, the Sotos decided to seek help outside Puerto Rico, because they became convinced that doctors there had nothing to offer but abortion. Mr. Soto, who had seen a television program about Children's Hospital, called the hospital from the couple's home in Manatí, near San Juan.
A Spanish-speaking translator at the hospital referred them to Dr. Fishman, a pediatric surgeon, who said he would try to help them.
On the Sotos' first visit to Boston, a fetal echocardiogram disclosed an abnormality that had never before been reported in conjoined twins: a circulatory condition in which the twin with the heart pumped blood to the other through the umbilical cord. The condition meant that cutting the cord, normally a happy event in the delivery room, would kill both babies.
"One would die, and that would result in the death of the other, since they're essentially a single organism," said Dr. Mary van der Velde, director of fetal echocardiography at Children's Hospital, who diagnosed the circulatory condition.
Doctors realized that the only hope would be surgery to separate the twins as soon as they were born. But they knew that at best they would be able to save only the baby with the heart.
"It would be either zero or one," said Dr. Fishman. "There was no way that they could stay attached and live, or be separated and both live. And we knew that we would have very little time to separate them once the cord was cut."
Conjoined twins are rare, occurring in 1 in 30,000 to 1 in 100,000 births. Many have serious defects in addition to being conjoined; some die as fetuses, some at birth and in some cases parents choose abortion.
Conjoined twins with a single heart have been born before, but often the hearts have been abnormal, and few babies have survived. There have been no reported cases of conjoined twins with the Sotos' circulatory pattern, Dr. Fishman said, though cases may have occurred but gone undiagnosed, with the fetuses dying at birth or in the womb, or being aborted. So the operation separating the Soto twins was a first.
Most conjoined twins are separated weeks or months after they are born, to give them a chance to grow and become strong enough to survive surgery and to let doctors study their anatomy and plan the operation.
In the Sotos' case, doctors began planning while the twins were still in the womb. To find out what internal organs were present and whether any were joined, two radiologists, Dr. Clare Tempany and Dr. Lennox Hoyte, performed magnetic resonance imaging, or M.R.I. examinations, and used software to assemble the images into a three-dimensional model showing the babies' organs.
The software, normally used to study brain tumors in adults, had never been applied to the tiny structures inside fetuses before, and it took 70 to 80 hours of work to interpret the images and create the models, Dr. Tempany estimated.
Eventually, they determined that the vital organs were normal except for the livers, which were fused.
Surgeons would be able to divide them.
Additional studies also confirmed that not even a heart transplant would save the second twin, because she was missing major blood vessels.
"There was nothing to hook a heart into," Dr. Fishman said, adding that in any case, newborn donor hearts are virtually impossible to find.
Given that the second twin could not survive, Dr. Fishman planned to make his incisions as much as possible to her side of the bridge linking her to her sister, to spare the twin who would live and also to provide the ribs, skin and tissue needed to cover the large opening that would be left in her chest and abdomen.
Mr. Soto said that although the couple had been told that only one baby would survive, reality did not sink in until Dr. Fishman gathered them and other family members together in a conference room at the hospital and drew a detailed diagram of the proposed surgery on a blackboard. He finished by marking a large X over the outline of the twin who would die.
"We cried," Mr. Soto said. "I guess until then we thought God was going to put a little heart in there."
Only when he saw the family in tears, Dr. Fishman said later, did he feel sure that they understood that only one of their daughters could survive.
The couple chose names for both babies and made plans to bury one, who would be called Sandra Ivellise, in a family plot in Puerto Rico.
But the doctors and the family worried that both babies might die before they were born. Their circulatory condition, called a TRAP sequence, for "twin-reversed arterial perfusion," sometimes occurs in twins who are not conjoined but in which only one has a heart, with the blood circulating to the second through the umbilical cord.
In those cases, the twin with the heart, referred to as the "pump twin," can die from heart failure induced by the strain of pumping blood to its sibling. When the TRAP condition is diagnosed in non-conjoined twins, doctors can operate on the fetuses to cut off the blood supply to the second twin, which is usually not fully developed anyway. The second twin dies, but the twin with the heart is saved.
But that procedure cannot be done if the twins are conjoined, as the Soto babies were, because the death of one will kill the other as well.
As Mrs. Soto's pregnancy progressed, fluid began to accumulate in one of the twins' chests, a sign of heart failure. Doctors feared the babies would be lost, but somehow the condition resolved. A Caesarean delivery and immediate surgery for the babies were planned for the first week of June.
But at 7 a.m. on May 30, Mrs. Soto's obstetricians called Dr. Fishman with alarming news. She had developed a disorder called preeclampsia, and her blood pressure was so high she was at risk of having a stroke. She needed a Caesarean immediately, the obstetrician said.
"If you do that, the babies will die," Dr. Fishman said. He pleaded for time to assemble his operating-room team, about 20 people. The obstetricians gave him 90 minutes.
The team gathered, and Dr. Fishman stood by in the delivery room at Brigham and Women's Hospital, waiting to rush the babies through the corridors to Children's Hospital, which is next door.
But he was unprepared for the emotional impact of seeing two live babies, and knowing that soon there would be only one, he said, describing his feelings this way, "As much as we spent months planning for this, and it seemed ethically and emotionally simple, nevertheless when I saw them both initially pink and both crying and moving their arms and having the same size bodies, it was heart wrenching."
Mr. Soto saw his daughters in the delivery room, but, he said, he was afraid to touch them. Mrs. Soto, fearing that neither baby would live, said she could not bear to look at them.
As the doctors had predicted, Sandra, who lacked a heart, began to fail almost immediately when the cord was cut.
"We began to see things change before our eyes," said Dr. Errol Norwitz, part of the obstetrical team. "She became cool, and very pale." Her blood pressure was too low to measure, she lacked a pulse and she was not getting enough oxygen. It was urgent that she be separated from her sister.
The babies were given anesthesia, put on ventilators and whisked off to the operating room. The surgery went smoothly. The most wrenching moment occurred about two-thirds of the way through the procedure, Dr. Fishman said, when the twins were physically separated and it was time to take Sandra's body away.
Darielis spent six months in the hospital and required more operations to repair a heart defect and an intestinal obstruction. She also needed physical therapy and a brace to help straighten her spine, which was curved backward from her cramped position in the womb.
And for her first year she had a bulge in her chest the size of an adult's fist, created by a rib cage that Dr. Fishman had constructed from Sandra's tissue and bone to protect Darielis's heart, which at first did not fit into her chest. But recent surgery has smoothed the bulge, and she looks completely normal, with surprisingly faint scarring and a remarkably sunny disposition.
For now, the Sotos are not sure how long they will remain in the United States. They are living near Mr. Soto's mother and brother in Paterson.
Mrs. Soto is expecting another baby, a girl, in November.
But the family has not forgotten the daughter they lost. They hope to visit Sandra's grave in Puerto Rico soon.
Asked what they would eventually tell Darielis about her beginnings, Mr. and Mrs. Soto answered simultaneously, "La verdad." The truth.