Summer 1997
Born with multiple birth defects, including spina bifida, Linsey's life has always been challenging. Before birth, her biological mother made arrangements for Linsey to be adopted. The adoptive parents reneged, however, when they learned of the child's numerous problems, so the state took custody. Alone and isolated in a hospital neonatal intensive care unit, Linsey fought for life.
Enter Sue and Walter Ponfick, both in their mid-50s, who have been foster parents to about 30 children. They saw an article in the San Antonio Express-News about Linsey and spoke with their caseworker about her. "When Walter and I met her she was 2 weeks old and paralyzed from the lower rib cage down. She had an open and protruding spine and a shunt on the right side of her brain," said Mrs. Ponfick. "We were told that this child had only one brain hemisphere and that she wouldn't live past six months. We were also told not to take her home—and I said we would make that decision.
"So we went home, talked with our family (children and grandchildren) and did a lot of praying. We saw, even at that age, a smile on her face. At 4 weeks she put her hands together— I thought people with one brain hemisphere couldn't do that. So, we just decided that this child was responding and decided to take her home at 5 weeks," she said.
When Linsey was 4 months old Mrs. Ponfick insisted on another CT scan of Linsey's brain. The baby was demonstrating increasing control over body movements and responding well to sounds, light and other stimuli. "The scan showed normal configuration of the brain— she had both hemispheres," said Mrs. Ponfick. "We all learn in this medical field that statistics don't always prove out and that things aren't always what they seem to be."
At 6 months, after successfully battling meningitis and recovering from bladder surgery, Linsey's life was further complicated by the discovery of scoliosis—spine curvature. "One day, when I was giving her a bath, I noticed her spine was curving," explained Mrs. Ponfick. "I took her to the orthopaedist who had taken care of our other foster children, and he said, 'I think you need to see Dr. Campbell.'"
Since Linsey's right ribs were normal and left ribs were fused—stacked on top of one another—the right side of her chest was growing, but the left was not. Her spine was growing, too, and being forced to curve. As a result, her heart and left lung were trapped between the ribs and spine. Over time, with Linsey's chest area prohibiting enough room for heart and left lung growth, she would either suffocate from inadequate air supply, suffer heart complications or both.
Following extensive examinations, Robert M. Campbell Jr., MD, associate professor of orthopaedics, and Melvin D. Smith, MD, clinical professor of surgery, at the Health Science Center and their surgical team members agreed Linsey would be a good candidate for a titanium rib implant. First the surgeons would separate Linsey's ribs and then implant the titanium device in a vertical position, attaching it to an upper and lower rib. The prosthesis would maintain the space, between the ribs, that had been acquired through surgical separation. As a result, the chest would be expanded and could accommodate normal left lung and heart development; the expansion would indirectly correct the scoliosis. (See x-ray.)
"We were delighted to hear the news, but I was scared to death," said Mrs. Ponfick. "I knew it was a tough surgery and that anything could happen."
At the time of Linsey's evaluation, Drs. Campbell and Smith were the only surgeons in the world who performed the life-extending surgery, so Linsey was placed on a waiting list for a little more than a year. "During the wait, a lot of things were going through my mind," said Mrs. Ponfick. "My mind said yes, but my heart said no. I could see where people would not want to put their child through it."
The day of the surgery finally arrived in August 1994. At the end of the successful six-hour procedure at Santa Rosa Children's Hospital, Linsey had a new prosthesis and the ability to inhale a little deeper. She went home in two weeks.
"When I meet parents of my patients I tell them that the first surgery is the start of a ten-year romance;" said Dr. Campbell, "we have to follow these young kids until they finish growth and routinely expand the prosthesis. To ratchet up the rib we make a 1-inch incision, unlock the prosthesis, expand it as much as necessary, in 1/2 centimeter increments, and then add a new lock. We have to do it about every four to six months—it's an outpatient procedure," explained the device's inventor.
Now 4 years old, Linsey has normal clinical lung capacity and a strong heart. She knows numbers, letters, colors and even sign language. Because of numerous intubations and resulting scar tissue, she had a tracheostomy before the age of 2 and could not speak. Recently, however, the tracheal tube was capped off to determine if surgical removal of it would be a possibility. Linsey quickly learned that she could then make audible sounds. "It wasn't long before she raised her hand, pointed her finger towards the door and said her first word, 'outside!'" said Mrs. Ponfick. "There was no 'mama' or 'daddy,' just 'outside!'
"She loves being outdoors. We go outside, we bounce on the trampoline, we swing, we ride horses—we lead as normal a life as we can, because I think we have to look at the positives instead of the negatives," said Mrs. Ponfick. "When the trach comes out we're going to the swimming pool."
Although Linsey will never walk, because of the spina bifida, she skillfully navigates her wheelchair and enjoys physical therapy. "We take her to physical therapy mainly for her self-esteem—to get her up and get weight on her bones and legs," said Mrs. Ponfick. "She's aware of her legs—she pushes them around with her hands, and we count her toes and tell her, 'These feet don't work, but they're beautiful feet and they're here for a reason.' "We want Linsey to like herself and use her disability as an asset to help others, not to feel sorry for herself.
"You live from day to day with Linsey. Each day is a gift. She's in school right now, Comal Elementary, and doing marvelously. She's a very bright girl. I think Linsey knows about herself, and I'm proud she has those cognitive skills. She's able to take life as it comes," added Mrs. Ponfick.
The Ponficks have permanent management conservatorship of Linsey. "We gave her our name, and we have conservatorship for her lifetime. Our daughter will care for Linsey when we're not able to—it's all in our legal papers," explained Mrs. Ponfick. The couple elected not to adopt Linsey, since the state's obligation to pay medical expenses for foster children would have been nullified.
As Drs. Campbell and Smith continue to treat other children with problems similar to Linsey's, the Ponficks provide both a support group and counseling for the children's parents. "We see people from all over the world who visit with Dr. Campbell and Dr. Smith—in fact some of them have stayed with us in our home," said Mrs. Ponfick. "It's marvelous to be able to provide someone with a support network—I've seen it work. We're able to say to them, 'Hey, it's a slow process, but look, we have proof right here that miracles happen.'"
About 45 patients have had titanium rib implant surgery performed by Drs. Campbell and Smith. The invention was created ten years ago when one of Dr. Smith's patients was born with the ribs absent on his right side. Dr. Smith asked for Dr. Campbell's assistance in building an artificial chest wall that would provide support for the baby's right lung. Dr. ampbell, who attended Vanderbilt School of Engineering before deciding on a medical career, fashioned a prosthesis with pins used to set bone fractures. It would work for the short term. But Dr. Campbell knew he must immediately begin blueprints for a long-term prosthetic implant—a growing baby needed an expandable device.
"We needed something simple to be implanted and something that could be easily expanded with minimal surgery. And even after I finished the design, I had to knock on a lot of doors to find a company willing to manufacture the titanium rib. It was difficult finding a manufacturer that would make a custom, one-time implant with no precedent," said Dr. Campbell. A California firm agreed to manufacture the prosthesis. The child who received the first titanium rib implant is now 10 years old and looking forward to playing on a baseball team this season.
"After that surgery, we got referrals for all sorts of rib problems," stated Dr. Campbell. "And at that time there wasn't a single, logical approach to treat all of them." The titanium rib surgical team coined a term for the problems suffered by the referred kids: thoracic insufficiency syndrome (TIS). Simply stated, TIS is the inability of the thorax (the part of the body between the neck and abdomen) to support normal respiration or lung growth.
TIS patients have one or a combination of absent ribs, fused ribs or severe scoliosis with small chest syndrome. Dr. Campbell's surgical team has developed three basic procedures over the last nine years to treat each of these problems. Two other Health Science Center physicians are on the team: Humberto Hidalgo, MD, associate professor of pediatrics, and Thomas C. Mayes, MD, associate professor of pediatrics and chief of the critical care division of the department of pediatrics. Ricardo F. Pinero, MD, was associated with this group during its early years. This year teams of surgeons in other United States cities, trained by Drs. Campbell and Smith, will begin performing titanium rib implants under a multi-center study, to include Boston Children's Hospital and Children's Hospital of Pittsburgh.
"While likely less than 1 percent of the population is affected by TIS, the problem still needs to be addressed," Dr. Campbell said. "Without surgery, these kids could become dependent on respirators. Right now, surgery seems to be the only way to treat them."
