LOS ANGELES (January 30, 2000) - "Goodness, gracious, sakes alive!" They were unlikely first words for a toddler, but then, the very fact that Wayne Abney was talking at all was a miracle. Come to that, the fact that he was even alive, was a miracle. Wayne, now 19, was born with an extremely severe case of a rare congenital disorder that made it impossible for him to breathe unassisted while sleeping, and conventional wisdom of the day said that in cases as severe as Wayne's, it was best to let nature take its course - to not intervene - and "not intervening" would have meant immediate death.
Fortunately, neither Wayne's neonatologist, Augusto Sola, M.D., nor his parents subscribed to "conventional wisdom," and after seven months in a New Orleans hospital Neonatal Intensive Care Unit, Wayne became the world's first baby to go home on a respirator. He went on to become one of the first respirator-dependent youngsters -- if not the first -- to be mainstreamed in the public school system from kindergarten through grade 12. Last June he graduated from high school with a grade point average of 3.7 (out of a possible 4), and he hopes to become a radio disk jockey. Today, he is the longest-living patient to be on a respirator since birth and to live at home.
According to Dr. Sola, who last October received the American Academy of Pediatrics' 1999 E.H. Christopherson Award on International Child Health for his long-standing efforts to improve the lives of newborns and infants throughout the world, Wayne's disorder is rare, but an increasing number of cases are being diagnosed.
"Congenital central alveolar hypo-ventilation affects the portion of the brain that controls involuntary breathing," said Dr. Sola, who is director of Cedars-Sinai’s highly respected division of Neonatology. "It can appear in a wide spectrum of intensities, ranging from extremely mild - as in some types of sleep disorders where breathing is temporarily interrupted -- to extremely severe, where breathing completely stops. Wayne's case is at the 'extremely severe' end of the spectrum."
According to Wayne's mom, Judy, everything seemed fine as she went through labor and delivery on July 2, 1980. True, the baby was not expected for another four to five weeks, but there didn't seem to be cause for concern. However, after Wayne was born, it quickly became apparent that something was wrong. "He didn't cry," said Judy. "They took him to a table and began working on him, and Gary and I (her husband) didn't know what was going on, but he wasn't making a sound."
As it turned out, Dr. Sola was in the hospital that day seeing another case, and he was called emergently to the delivery room. He immediately intubated the infant to get air flowing into the baby's lungs. "What I remember most about that day," said Judy, "was that as busy as he was working on Wayne, Dr. Sola looked over at Gary and me and invited Gary to join him over by Wayne so we could understand what was going on. He showed Gary that the baby really was crying - we just couldn't hear him because of the intubation. I felt so much better when I heard that."
Later that day, Dr. Sola took Wayne by ambulance to another New Orleans hospital - one with more specialized capabilities for seriously ill infants. For several months, no one knew why Wayne couldn't breathe. "He was doing well in every other way," said Judy. "He was a happy, responsive baby; he smiled and laughed, but he wouldn't breathe without the respirator."
Because of the ventilator tubing, Wayne could not swallow or eat, so another tube was required for feeding, but Wayne's doctors continued to be puzzled by his inability to breathe. Baffled, Dr. Sola researched possible diagnoses extensively - checking with colleagues around the country to see if anyone had treated a baby with similar symptoms.
When Wayne was about four months old, a diagnosis was finally reached -- congenital central alveolar hypo-ventilation - or "Ondine's Curse," as it was sometimes informally known. There was - and is - no known cure, and it is not a disorder that can be outgrown nor one that improves over time. Wayne would need a respirator for as long as he lived, and up until then, no babies had ever been released from a hospital to live at home on a respirator. The future for the Abneys - and for Wayne -- looked uncertain at best.
Then came the turning point. One day when Judy came to the hospital for her daily visit, Wayne's isolette was empty. Puzzled, she looked around to see if one of the nurses in the NICU was holding him. She didn't see him anywhere. So she started asking. "Oh, Wayne is with Dr. Sola," one of the nurses told her. Walking down the hall to Dr. Sola's office, Judy stopped short when she reached his door. There, sitting at the desk doing paperwork was Dr. Sola. With one hand he was operating a manual resuscitation bag so that Wayne could breathe away from the respirator, while with the other hand he dealt with the papers on his desk.
He had just wanted to spend some time interacting with the baby and to give Wayne an opportunity to get out of the NICU for a little while. "When I saw Dr. Sola there doing his work and "bagging" Wayne at the same time, something in me clicked," said Judy. "I thought to myself, 'I can do this. I'm going to take Wayne home. This can work.'"
Terrified at the thought of having a baby at home who couldn't breathe, Judy nevertheless approached Dr. Sola with the idea. He agreed that she and Gary could provide the care Wayne needed, and immediately began organizing pulmonary and caregiver training for them. After two months of training, they were ready for a "test run." A private room was set up for them in the hospital, and Judy and Wayne "lived" in that room 24 hours a day, seven days a week for two weeks. For one of those weeks, Gary, who worked on off-shore oil rigs every other week, lived there with them. The room was equipped with the same medical equipment that Wayne would have at home. Plus it had an emergency button that they could push to summon a nurse if a crisis arose.
"We wanted to be sure that we had faced every eventuality and that we would know how to cope with every situation," said Judy. "We had some crises, but we managed to figure them all out, and we never once had to push the panic button. We just put into practice what we had learned."
At the end of the two weeks, when he was seven months old, Wayne came home. Friends and family members initially questioned Judy's and Gary's decision, wondering aloud what kind of quality of life the baby would have. Judy knew, though, that the key to giving Wayne as normal as life as possible, was to help create understanding and help him build positive loving relationships.
"As soon as he came home from the hospital, when people came to visit, I would place him in their arms," she said. "He was so responsive and so happy, and they could see that for themselves when he smiled up at them. Instead of being intimidated and fearful of a disease they didn't understand, they were bonding with an outgoing baby, and they grew to understand why he needed the various equipment and what it meant when an alarm buzzer went off."
Judy adopted the same philosophy when Wayne started school. "I made arrangements to visit each classroom one-on-one with Wayne as the 'show-and-tell.' I explained to the children what the purpose of the ventilator was, why Wayne needed it, and what it meant when they heard certain sounds. Wayne joined in and did some the explaining, himself. Kids are so much more accepting. They were great, and they weren't intimidated by it at all."
Gary agrees, pointing out that adults are generally more reserved in their approach than are children. "Sometimes when we go places, the little kids will come running up to Wayne - they just seem to be drawn to him - and the parents will scold them, telling them not to touch him as they might hurt him, or not to stare at him. We encourage them to approach him and touch him. That way he and we can explain to them and build understanding. He loves interacting with them, and they love him."
When Wayne was four years old, he became a big brother - an event that was thrilling for him as he now had someone to look after and "show the ropes." More than that, though, said Judy, Mark's arrival was the perfect "gift" for Wayne - incentivizing him to ever greater developmental accomplishments. For example, it was after Baby Mark arrived that Wayne learned to walk, talk and even developed the necessary muscles for swallowing and eating without the feeding tube. "As Mark began to be mobile and crawl around, Wayne would watch out for him," said Judy, "keeping an eye on his baby brother to be sure he was safe."
Over the years, Wayne faced a multitude of additional medical challenges - open heart surgery, multiple stomach surgeries, and a congenital bone defect that makes his bones very brittle. He once fell and simultaneously broke both legs, resulting in the need for a wheelchair. Through it all, though, he maintains his zest for life, and his parents have helped him operate on the premise that life is for living. "When he was little, we had to figure out ways to enable him to do everyday activities that many people just take for granted," said Judy.
For example, when they took little Wayne to the park, he obviously couldn't take the ventilator down the slide. So Judy would go to the top of the slide with him, help him use a manual resuscitation bag to hyperventilate, send him whizzing down the slide, then simultaneously toss the bag down to Gary who would catch him at the bottom. Gary would then use the bag to manually resuscitate Wayne at the bottom of the slide, and they would start the process all over again - much to Wayne's delight!
They family has even figured out ways to take Wayne on the thrilling theme park rides at Walt Disney World, Six Flags and more. "Disney is especially accommodating to people with special needs," said Judy. On some of the rides, we could actually strap in the whole wheelchair so he could use his ventilator throughout the ride. On other rides, we used the manual resuscitation bags, with either Gary or I riding right beside him to keep the air flowing into his lungs."
Other family trips have included ones to Atlanta, South Carolina, Miami, and currently, to Los Angeles. "This is our longest trip," said Gary. "But it all comes down to planning ahead."
Wayne, Judy, Gary and Mark, are also active in the Louisiana Chapter of a support organization for technology-dependent children known as Sick Kids Need Involved People (S.K.I.P.). President of S.K.I.P. for the past 13 years, Judy organizes an annual four-day camp for kids and their families. "What makes this camp different from other camps is that if a kid qualifies for it, his or her family automatically qualifies, too," said Judy. "This has to involve the whole family."
For the next two months, they plan to be in the Los Angeles area to give Mark an opportunity to pursue his dream of becoming an actor. "He's auditioning with agencies and for pilots," said Wayne proudly.
"I've always hoped to have a career in the entertainment industry," Mark added. "I've done community theater for eight years and dance for 12. I'm excited about the opportunity to be here in Los Angeles and see what happens."
As for Wayne, he too has his sights set on an entertainment industry career. Like his little brother, he has also participated in school drama clubs and community theater - sometimes even building stage ramps and lifts to accommodate his wheel chair when necessary. Recently, he had an opportunity to attend the Jay Leno Show - an experience he describes as "great! I even had my picture taken with him," Wayne exclaimed. Ultimately, he hopes to combine his love for performing with his love of music by becoming a disk jockey. "I love music," he says enthusiastically. "Rock and rap, especially, but I'm not too much into classical - unless it's classical rock!"
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