Doctors Fight for Twins’ Independence
Maria de Jesus and Maria Teresa Quiej-Alvarez have shared a bed for the last 11 months, but they’ve never gazed into each other’s dark brown eyes.
The conjoined twins from a Guatemalan village were born with skulls fused at the top, their faces tilted in opposite directions beneath the thick black hair they share. On Monday, a team of physicians at UCLA’s Mattel Children’s Hospital is scheduled to begin the first attempt to change that, with a rare series of procedures to separate the infants and grant them independence.
Experts say that such cranial separations have been performed only five times worldwide in the past 10 years, but not all those twins survived long past the surgery. Because the two girls share only a small amount of brain tissue and both their hearts are working well, doctors at UCLA think their chances for full and unimpaired lives are good.
Their mother, Alba Leticia Quiej-Alvarez, 22, said she is not following the medical details closely. She just wants the girls, her only babies, to have normal childhoods in their hometown in southwest Guatemala, where she is a housewife and her husband, Wenceslao, 20, is a farm worker.
“But I don’t dream about it. I don’t want to have any dreams, because if you think and think, it doesn’t change things,” said Quiej-Alvarez, who speaks Spanish and cannot read or write. She was interviewed at UCLA, where she and the twins have shared a room since they were flown in from Guatemala by an international charity last month. The hospital and doctors are donating services whose cost is estimated at $1.5 million.
Craniopagus conjoined twins, or those joined at the head, have the rarest of an already rare condition. Conjoined twins occur once in every 200,000 live births, and only 2% of those twins are craniopagus, according to a 1990 study published in the journal Gynecology and Obstetrics. While the girls could remain healthy if they were unseparated, they would require immense amounts of care, said Dr. Jorge Lazareff, director of UCLA’s department of pediatric neurosurgery, who is coordinating their treatment.
“They certainly will have a very miserable life if they continue [conjoined]. They are as aware as anyone else of their limitations,” he said. “They will not die, although they will not be very happy.”
The girls were brought to UCLA by Healing the Children, a Spokane, Wash., group that matches volunteer medical personnel with children from developing nations who suffer from serious, yet curable, conditions.
The hospital, which has never separated conjoined twins before, has scheduled a preliminary procedure on the girls’ scalp for Monday. The actual separation, expected to take 10 hours, is planned for mid-July.
Both the twins are fully developed for 11 months, although Maria de Jesus is slightly smaller than her sister.
The ways in which they compensate for their limitation are astounding. Their faces contort while they try to roll over in opposite directions until one, usually Maria Teresa, wins out. Because their connection limits their shoulder movements, they often use their feet to grab things.
The sisters are intelligent, doctors say. They vocalize and even say a few words: mama, dada and agua. But their consciousness of each other remains a mystery to outsiders. Even when the girls look into mirrors, they can’t see each other’s faces, which are angled 120 degrees apart.
They have distinct personalities and desires. “Both are very well developed. Both are very independent human beings,” Lazareff said. “That doubles the responsibility.”
One will cry while the other is calm, and one will sleep while the other is awake. Their mother said Maria Teresa is the playful one and Maria de Jesus is shyer, quietly observing.
Every aspect of caring for the girls--diapering, cuddling, feeding--requires two people. Otherwise, when they cry, “I can only hold one and the other has to wait by herself,” Quiej-Alvarez said. During bathing, one girl has to be held upside-down while the other is in the tub.
Their hearts are the same size, which indicates that neither pumps blood for both of them. Tests have showed that the twins share less than 10% of their brain tissue. As a result, they have an equally good chance of not only surviving surgery, Lazareff said, but of achieving full mobility and intellectual growth.
Other tests, however, confirmed Lazareff’s biggest worry. Each sister has a major vein in the front of her head that drains into the back of the other’s head. During the separation surgery, doctors will block off these veins and hope that other blood vessels can pick up the slack. If things don’t go well with that procedure, the girls might suffer strokes and brain damage, or die. There are other complications. Once they are separated, the rectangular areas that have connected the sisters will lack skin and will have little dura mater, the spongy tissue found under the skull that protects the brain from damage and injury. Artificial material will be inserted to replace the dura mater during the main surgery in July. But without enough skin to close the wound, Maria Teresa and Maria de Jesus could be vulnerable to serious infection.
So, Lazareff enlisted the help of Dr. Henry Kawamoto Jr., a specialist in craniofacial reconstructive surgery. On Monday, Kawamoto is scheduled to implant a deflated balloon, like the kind used in breast reconstruction, just under the skin on each side of the twins’ shared skull. During the next three weeks, doctors will inject the balloons with saline to stretch the skin, the way the skin of a woman’s stomach stretches during pregnancy, Kawamoto said. The skin flaps will also grow hair, although slightly thinner than before.
After the balloons are put in, the twins’ heads will swell at the fused area, making them resemble extras on the set of a sci-fi movie, but Kawamoto said it will not be painful. He worries, however, that the twins could develop bed sores from resting on the balloons. A UCLA occupational therapist is designing a doughnut-shaped pillow to ease the pressure.
The physicians predict that skull tissue will grow to close the gap, but they will consider grafts of bone tissue later if necessary.
Next month, the main separation surgery will require four surgical teams--two reconstructive, two neurosurgical--to work on the children. “It has to be choreographed,” Kawamoto said.
For the twins’ mother, the weeks of waiting for the surgery have been the most time she’s spent with her daughters.
No one had expected twins, much less conjoined ones. Quiej-Alvarez said she was in labor for eight days at home before a midwife recommended that she go to a nearby clinic. There, she delivered the twins, who weighed 4.4 pounds together, by Cesarean section.
She and the babies were sent to a hospital far from home. After recovering, Quiej-Alvarez returned to her village and the babies remained in the neonatal intensive care unit. Until recently, they were restricted to formula because they could not sit up to swallow safely. (At UCLA, nurses prop the babies up to feed them strained foods.)
In Guatemala, Quiej-Alvarez and her husband made regular pilgrimages to visit the girls. They would ride a bus for six hours and walk an additional hour and a half to the hospital.
They were allowed to enter the twins’ room twice a day for 15 minutes at a time. Afterward, they would go home, because they had no place to stay.
The founder of Healing the Children, Cris Embleton, said transporting children to the United States for treatment usually is not as efficient as taking care to them in their own countries. But the separation and recovery of the twins require the kind of expertise available only at an institution like UCLA. For this case, the group needed to find “doctors with extremely high skills,” she said.
Lazareff has made several trips to Romania to treat children suffering from conditions such as brain tumors or spina bifida. After he heard about las dos Marias, as he calls them, his calls prompted UCLA’s Mattel Children’s Hospital to donate its services.
It’s not easy to plan a trip for such unusual travelers. The twins have individual passports, and in the passport photos it’s impossible to tell that they are conjoined. Naomi Bronstein, co-founder of Healing the Children, purchased Velcro tape to hold the children down during takeoff and landing of their charter flight to Burbank Airport. It wasn’t needed, however. Two seats were reclined to form a safe bed for the twins.
An ambulance brought the twins and their mother to UCLA. Nurses, rarely confronted with infants who need a space four feet long, quickly taped together two open-sided cribs.
Now that she lives at UCLA with them, the mother has a firm relationship with her daughters.
“If they hear me leave, they cry and cry so I have to sneak out,” she said. “And when I come in they scream, and they both want me to hold them.”
It hasn’t been easy. Every day, a steady stream of new faces--doctors, nurses and reporters--comes to the babies’ room. Still, she is lonely without her husband, who stayed home to work, and is tired of questions about her pregnancy and her personal life.
The twins will stay at UCLA through their recovery, which could take several months.
No matter what happens, Dr. Michael Karpf, vice provost of UCLA Hospital Systems and director of UCLA Medical Center, said he knew he had made the right decision in approving the surgery.
“They’re two distinct personalities,” he said. “They deserve a try at being themselves.”
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Times staff writer Elena Gaona contributed to this report.
(To offset the cost of the twins’ care, checks payable to the UCLA Foundation may be mailed to UCLA Medical Sciences Development, 10945 Le Conte Ave., Suite 3132, Los Angeles, CA 90095. Donations for the twins may also be made through Healing the Children at Box 221478, Newhall, CA 91322.)
