LLUMC Legacy: Daring to Care
PAUL GABRIEL BAILEY HOLC: "The Incredible Holc"
"It's the first time it's been done, and it's fair to say there was some electricity in the air," reported Elmar Sakala, M.D., director of the maternity unit at Loma Linda University Medical Center.
He was referring to one of the most incredible infant-heart-transplant cases, that of Paul Holc, a patient who was referred to Loma Linda from South Surrey, British Columbia, before birth.
Paul's surgery became an unprecedented milestone in transplantation history. Not only did he become the youngest person in the world to undergo heart transplantation, but also his donor would spark a debate "heard 'round the world."
Because of the age of his mother (35) and the heart disease of two close relatives, a series of three fetal echocardiograms was performed. This technique, a specialized form of diagnostic ultrasound, uses high-frequency sound waves to create pictures of the inside of the body. It is used for diagnostic studies on pregnant women to avoid the risks of using X rays. The last two "echos" showed that the baby had hypoplastic left-heart syndrome (HLHS), a lethal underdevelopment of the left side of the heart.
By this time, August, 1987, even though Loma Linda University Medical Center had gained an international reputation for performing successful heart transplants on newborns, some physicians still were wary. The diagnosis was explained to the parents, Gordon and Alice Holc. The prognosis, they were told, was hopeless. The Holcs asked about heart transplantation, but were at first discouraged by their physicians. Several days later, their doctors decided to reevaluate their position and, after further study, referred the Holcs to Loma Linda.
Echocardiography at Loma Linda confirmed that the baby had HLHS. The parents consulted with Loma Linda's multidisciplinary heart-transplant team, who presented various options to the family, including heart transplantation. After extensive discussion and consideration, including talks with another Canadian family whose daughter, Jessica, had received a new heart at Loma Linda, the parents opted for an attempt at heart transplantation.
The baby would be placed on Loma Linda's transplant list at 36 weeks gestation (36 weeks into the pregnancy) and registered with the United Network for Organ Sharing (UNOS), the North American organ-procurement agency. When a donor became available, a transplant would be performed following a cesarean-section delivery. Four days after the baby was listed with UNOS, a two-day-old donor, coincidentally from Canada, became available.
It should be emphasized that transplant centers, as a standard practice, do not identify donors. Donors and recipients and their families are almost always kept anonymous. If they want to communicate, they can do so through an intermediary. But in some cases, generally because of unusual circumstances, donor families have held press conferences in which they have told the circumstances of their gift.
In this case, the donor family later identified their child as a baby girl named Gabriel. She had been named after the Biblical angel, Gabriel. They reported that she also had been diagnosed before birth. Her condition also was not compatible with life. She was an anencephalic infant (missing all of the brain except for the rudimentary brain stem). The family reported that they had deliberately carried the pregnancy to full term in order to turn their personal tragedy into something good, by donating the organs of their first-born to try to save another baby. They were proud of their daughter and for what she was able to do after her death. She was a little heroine.
Baby Gabriel was born at Soldier's Memorial Hospital in Orillia, Ontario, Canada, on October 12, 1987. She weighed 5 pounds, 5 ounces, and was almost four weeks overdue. She was breathing on her own. Dr. Timothy Frewen, director of Canada's largest infant-transplant center, the Children's Hospital of Western Ontario, was notified that the baby's parents wished to donate her organs to save another child.
The next day, Baby Gabriel's breathing became shallow. She was given artificial breathing assistance and intravenous medication to help protect her organs.
Still attached to a respiratory, Baby Gabriel was flown to London, Ontario, Canada, by a five-member transport team. At six-hour intervals, the respirator was turned off to determine her ability to breathe on her own. The next day, three physicians, not associated with the transplant team, determined during three separate 10-minute tests, that she could not breathe on her own.
In addition to being checked for permanent cessation of breathing, Baby Gabriel was checked extensively for spontaneous eye movement, pupil response to light, and gag and sucking reflexes. Baby Gabriel was declared clinically dead and respirator support was reinstituted to protect her organs.
Once brain death was established, the procurement centers were notified. Michael Bloch, transplant coordinator for London's University Hospital, telephoned Loma Linda pediatric cardiac-transplant coordinator, Cheri Mathis, R.N., who in turn contacted Alice Holc, in British Columbia. A potential donor had been found, Mrs. Holc was told, and she should come to Loma Linda immediately.
The next day, on October 15, beginning at 4:30 a.m., Bloch, a pediatrician, a nurse, and a respiratory therapist flew Baby Gabriel from Toronto International Airport to Southern California aboard a Skycharter Learjet 25. A ventilator supplied mechanical breaths as she lay in a heated transport incubator.
Because foul weather delayed the flight, batteries in the donor's heating unit ran low and most of her oxygen supply was used up. The plane had to refuel at Stapleton International Airport in Denver, Colorado, where Bloch made an urgent call at 5:30 a.m. to Paul Taylor, transplant coordinator for the University of Colorado. Taylor contacted the Children's Transport Services of Children's Hospital in Denver to arrange for oxygen tanks to be delivered to the waiting plane. Meanwhile, Bloch heated some towels in the airport lounge microwave to keep the donor warm. Then the sleek white jet shrieked back into the sky over the Rocky Mountains with its precious little life-giver.
Meanwhile, at the other end of Canada, another drama was unfolding. Because Canada provides healthcare for Canadian citizens and because of some logistic problems in arranging for an air ambulance, a friend of Gordon and Alice Holc, out of desperation, went to the Canadian news media to try to pressure the Canadian government into cutting the red tape. The story spread to the Canadian wire services and from there to the American wire services. Seventeen news camera crews from Canada and all over the United States converged on Loma Linda.
The Holcs arrived at Ontario International Airport at 6 a.m., after a five-hour flight from Vancouver International Airport in British Columbia. It had been a dark and stormy night. A cesarean-section delivery and a heart transplant were tentatively scheduled. Fog diverted Baby Gabriel's plane from nearby Norton Air Force Base to Los Angeles International Airport almost four hours later. She arrived in Loma Linda by ground ambulance at 11:45 a.m.
A major complication developed that threatened to postpone the landmark surgery. Tests showed that Baby Gabriel was a questionable donor. Her brain death was verified by a team of pediatric neurologists, but her heart was not contracting normally. Dehydration, which could be treated easily, might be the cause. The transplant team decided to wait to determine if overnight therapy could improve the donor's condition. Tests the following morning determined that Baby Gabriel's heart was contracting well. Now she was an acceptable donor.
A cesarean-section delivery was scheduled for later that morning--October 16, 1987. This time, serious complications developed that further threatened the transplant. The first problem with Baby Gabriel was not life-threatening to the recipient baby. If Baby Gabriel had been determined to be an unsuitable donor, the unborn baby would not have been in jeopardy. He was still safe in his mother's womb. Another donor might become available.
However, now another life-threatening problem arose. Baby Paul was delivered by Dr. Sakala at 10:54 a.m. The baby, weighing 6 pounds, 6 and three quarter ounces, took one breath and started to die. His ductus arteriosis (a significant remnant of his fetal-blood-circulation system) closed immediately. Frequently the closing of this vessel takes time--hours, days, or even weeks, depending on its size--and is a natural process. But in Paul's case, the vessel closed--maybe after just one breath--and, because of his malformed heart, the baby deteriorated rapidly.
Severe abnormalities in blood gases required immediate mechanical ventilation with a respirator. The baby's lungs retained fluid. Circulation in his extremities was poor, especially in his left leg. Intermittent mottling was evident in his upper extremities. In a press conference following the transplant, his surgeon, Leonard Bailey, M.D., a 1969 graduate of the Loma Linda University School of Medicine, reported that the baby really looked terrible immediately after birth.
If Joyce Peabody, M.D., then chief of Loma Linda's Division of Neonatology, or another one of her neonatologists (physicians who specialize in the care of premature/sick babies), had not been standing by during the delivery, the baby would not have made it to transplant surgery. It was a team effort.
Paul was handed through a special window from a high-risk delivery room into the neonatal intensive-care unit. This facility was designed to permit premature/sick newborns to be placed in the care of specialists as soon as possible. Peabody immediately placed umbilical venous and arterial lines and started the baby on prostaglandin E1 (a drug that opens the ductus), cyclosporine, antibiotics, and steroids (drugs designed to prevent infection and organ rejection). The pallor and poor perfusion improved dramatically with the infusion of prostaglandin. Within an hour the baby had been stabilized enough to proceed.
Meanwhile, as soon as Alice arrived at her recovery room, she called Jason, her 5-year-old son, staying with his grandmother in South Surrey, and told him he had a brother. An echocardiogram confirmed that the newborn had HLHS. The clinical picture was consistent with the need for a heart transplant. Baby Paul was held for a few minutes by his parents.
Three hours after the infant was born, Dr. Bailey and his team began a heart transplant on the youngest person in the world to undergo an organ transplant of any kind. Baby Paul's temperature was lowered to 65 degrees. Sewing the one-ounce heart into the tiny chest took 47 minutes. His new heart began beating on its own at 3:35 p.m., during the warming procedure.
When Baby Paul was transported to his room after four and a half hours in the surgery suite, his vital signs were stable, and he had good urine output. His parents got a brief look at him as he was wheeled into his room.
At a 9:00 p.m. press conference, Bailey reported, "We have both mother and baby in pretty good shape tonight.... I'm an optimist, some of you know, but this baby does look good at this hour."
The next day, Baby Paul gradually woke up and began moving his arms and legs. Dr. Josiah Lowry of Soldier's Hospital reported that Baby Gabriel's parents were pleased about what was happening in Loma Linda. "They see it as the one positive thing in this," she said.
Robert Brehl, a reporter for the Toronto Star interviewed Gabriel's parents, identified later as Karen and Fred Schouten, and said they were "bursting with pride."
"Some people work their whole lives to do something memorable," said Karen. "Gabriel did it in two days. We're just praying the other baby will make it."
"I immediately fell in love with her the first time I touched her," said Fred. "I want the whole world to know about Gabriel. Our baby has done something phenomenal. This feeling of being so proud has taken away the grievance period." Tears flowed freely down his face.
"We don't want anyone to feel sorry or pity us," said Karen. "Sure we cry. We cry constantly. But it's a happy cry."
Baby Paul was hospitalized for 35 days and was discharged from the Medical Center in good condition. More than 100 people on the heart-transplant team had been involved in his care. The baby and his happy family returned to Canada eight-and-a-half months later. He returns to Loma Linda for periodic checkups.
Out of respect and honor to the donor and to the transplant surgeon, Alice and Gordon Holc gave their son two middle names. His complete name is Paul Gabriel Bailey Holc. He has been affectionately nicknamed "The Incredible Holc."
Paul Holc's story was dramatized in a two-hour, made-for-television movie, "Heart of a Child," broadcast nationwide by the NBC television network on May 9, 1994. The final scenes of the movie show Paul Holc meeting Karen Schouten in a hug, with two close-up "freeze frames" and accompanying captions. The caption for Paul says, "Paul Holc, who portrays himself in the film, is an active first grader who loves to play baseball."
This is followed by a close-up of the actress portraying Baby Gabriel's mother, with the caption saying, "Karen Schouten recently gave birth to a perfectly healthy baby boy, named Sam."