Tiny liver transplant patient goes home

Monday, February 22, 1999

Tiny liver transplant patient goes home

Determined parents, dedicated hospital staff and luck made 3-month-old Sydni a survivor

Judy Quinlan cuddles her daughter Syndi in her room at Children's Hospital Medical Center.
(Glenn Hartong photo)
| ZOOM |

More than 200 patients have received liver transplants at Children's Hospital Medical Center since 1986, but only three of those children were smaller than Sydni Quinlan.

After surviving at least two near-death crises and undergoing intensive care for a good chunk of her first three months of life, Sydni went home to Batavia this monthcured of a rare genetic condition that doctors almost overlooked.

Sydni owes her life to determined parents, a small army of experts at Children's Hospital, and a large dose of luck.

“We almost lost her twice,” said Sydni's mother, Judy Quinlan. “I'm here to tell you that when it comes to taking care of kids, all hospitals are not the same.”

To Mrs. Quinlan and her husband, Rick, the ordeal offers a fresh reminder about the importance of an age-old medical precept: getting a second opinion.

Sydni was born Oct. 28, the apparently healthy third child of the Quinlans. Two days later, the couple took the baby home from the hospital.

“She was cold. She wasn't breathing right. Her arms, her face, her legs were ashen white,” Mrs. Quinlan said. “We called our pediatrician, who told us to take her to the emergency room, right away.”

Mrs. Quinlan declined to say which hospital they went to first. “It's probably a very good hospital. I don't want to hurt its reputation,” she said.

But from the start, Mrs. Quinlan said, the emergency staff struggled with Sydni's case. She said it was a Saturday night and no one in the emergency room felt comfortable enough to draw blood from an infant, so a labor-and-delivery staff member was called to assist.

“Then, the local ER didn't find anything wrong enough with Sydni to keep her, so they sent us home,” Mrs. Quinlan said. “They told us repeatedly that whatever she had wasn't life-threatening ... that we should call the pediatrician again on Monday, and if we were still concerned we should go to an urgent care center.”

“They thought we were new parents who were overly concerned,” Mrs. Quinlan said. “So we left.”

That was about 3 a.m. By 6 a.m. Sydni was getting worse. “She was breathing like a little goat, just bleating,” Mrs. Quinlan said.

The couple tried calling the urgent care center, only to find out the office doesn't open until 8:30 a.m. So they called their pediatrician again. This time, the doctor told them to go straight to Children's Hospital.

“When we got there, the doctors practically ran her to the trauma room,” Mrs. Quinlan recalled. “She stopped breathing a few minutes after that.”

Sydni had lapsed into what appeared to be septic shock, possibly from a bacterial infection that had run out-of-control. The emergency staff at Children's revived Sydni, stabilizing her enough to move her to neonatal intensive care, where she was placed on a respirator.

That was just the beginning. Soon, her lungs began to hemorrhage. Later, her liver began to fail. She would go through six dialysis treatments to cleanse her blood.

Fighting the shock symptoms made it hard for doctors to spot Sydni's disease. The picture came into focus on the third day in intensive care, when a blood test revealed a sharp spike in Sydni's ammonia levels, which can cause brain damage.

The test helped doctors determine that Sydni had citrullinemia, a genetic condition that occurs once in every 50,000 to 100,000 births. Patients with this condition have a liver that cannot process ammonia, a waste product created as the body breaks down proteins from food.

Sydni's condition is so rare that doctors at Children's Hospital wouldn't expect it to be detected at any community hospital emergency department.

It can take two or three days after birth for the ammonia to build up to health-damaging levels, said Dr. Nancy Leslie, a geneticist at Children's Hospital who worked on Sydni's case.

“For some of these kids, it's like falling off a cliff,” Dr. Leslie said. “They can look sort of OK for a while, then just hours later they look awful.”

DONATIONS

Medical bills for Syndi Quinlan reached $86,000 before she received her transplant, which is expected to cost more than $300,000. Medications could cost $20,000 a year.
Her parents' medical insurance will cover most of Syndi's medical bills. Even so, a family friend has established a fund to defray uncovered costs. Send donations to:
“Cents for Baby Sydni Fund”
P.O. Box 453
Batavia, Ohio 45103.

Once diagnosed, Sydni's parents faced two choices.

They could skip surgery and put Sydni on a diet that sharply limits protein intake to keep ammonia levels low. Then doctors would use various medications and treatments to cleanse the blood during flare-ups. The risk to this approach is that every serious or long-lasting spike in ammonia levels would further damage her brain, and possibly kill her.

Or they could try for a liver transplant. A successful transplant does cure the genetic condition. But the risks include waiting for a donor, surviving the operation, then spending a lifetime on immune-suppressing, anti-rejection medications that carry risks of triggering infections, cancer and organ damage.

The prospect of a real cure led the Quinlans to choose the transplant. For the next several weeks, Children's Hospital nutrition experts custom designed a diet to make Sydni gain weight as fast as possible while controlling ammonia levels in her blood. The bigger she was, the better her chances of surviving transplant surgery.

Some patients can spend years on the waiting list for a liver transplant. The disparities in waiting times are so sharp from region to region that transplant centers, patients groups and regulators spent much of last year fighting over ways to reform the system.

Sydni waited just four days. She went on the list Jan. 15 and was in the operating room on Jan. 19.

Her case had a high priority because of the risk of further brain damage, said Dr. John Bucuvalis, the liver disease expert who put her on the waiting list.

Even so, it was sheer coincidence that a 22-pound child died in Galveston, Texas and was close enough in size to Sydni to make the transplant possible.

Transplant surgeons Maria Alonso and Frederick Ryckman used the left lobe of the donated liver to fit inside Sydni's 8-pound body. The smallest baby to get a liver transplant at Children's Hospital weighed 6.5 pounds.

“Every time I look at Sydni, I think about that other child,” Mrs. Quinlan said.

Despite the rare circumstances in Sydni's case, doctors and Mrs. Quinlan said they drew two lessons from the ordeal.

First, parents should trust their gut feelings about their children. Parents know — often better than doctors — when something is wrong with their child.

“If you are not comfortable with a doctor's opinion, get a second opinion. Don't wait,” Mrs. Quinlan said. “Our waiting may have caused even more damage.”

Second, that Greater Cincinnati is lucky to have Children's Hospital. Mrs. Quinlan marveled at the sheer number of health professionals involved in Sydni's care: trauma specialists, liver experts, transplant surgeons, geneticists, nutritionists, entire teams of nurses and technical staff.

Dr. Deborah Borchers, Sydni's pediatrician, said she often recommends that parents with sick children under 2 years old go to Children's Hospital, even if other hospitals may be closer.

“Without slamming any other hospital, my opinion is that Children's Hospital is one of the top five or 10 pediatric hospitals in the country,” Dr. Borchers said.



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