Using a doll helps Kim Uhrich, a social worker, talk with Andrew
Lyle about his treatment. Photo by Will Owens.
After three decades, research at the UNC-CH craniofacial center begins and ends at the same place: with the patient. More than 6,000 people, most of them children, have come to the center for treatment of craniofacial anomalies.
Like many patients, four-year-old Andrew Lyle came to the center for correction of his cleft palate, a gap in the roof of the mouth that opens into the nasal airways. One out of every 700 babies in the U.S. is born with a cleft palate, a cleft lip, or both.
Using a doll helps Kim Uhrich, a social worker, talk with Andrew
Lyle about his treatment. Photo by Will Owens.
Andrew was first seen by members of the craniofacial team when he was an infant. During the diagnostic consultation, specialists spent one-and-a-half days assessing his physical, neurological, and social development, and he has returned several times since then for treatment. Surgeons repaired his cleft when he was two years old, and physicians have prescribed glasses for him. He also had temporary tubes in his ears to clear up recurrent infections, a problem more common in children with cleft palates.
Recently, Andrew returned to the center for a day-long follow-up evaluation. An oral surgeon and a plastic surgeon checked his healed palate. Two speech pathologists assessed his language skills, looking for the anomalous speech patterns which cleft palate patients sometimes develop. An otolaryngologist and an audiologist assessed his hearing, and an ophthalmologist tested his vision. A pediatric dentist and an orthodontist checked his teeth, and a social worker talked with his mother about preschool, about his social development, and about other health-care matters. Finally, two clinical researchers introduced him to the equipment they will use during future appointments.
Before Andrew's next visit, the team will meet to review his history, which is scrupulously documented in a six-pound medical chart. The members will discuss the most recent set of evaluations and decide whether to recommend any additional treatment.
Patients like Andrew have received coordinated, comprehensive care at the craniofacial center since it was established in 1963 by Donald Warren, the center's director and Kenan professor of dental ecology and otolaryngology, and by Erle Peacock, a plastic surgeon no longer at UNC-CH. Though originally created to treat cleft lip and palate patients, the program has expanded to include other craniofacial anomalies. "Our program… used to be mostly cleft lip and palate because those babies survived and the others didn't," says Warren. "The needs of the patients now are much more complex than they used to be."
Six programs in the state treat patients with cleft lip and palate, but only four of those treat the full spectrum of craniofacial patients. The program at UNC-CH is the largest, with a core group of providers and researchers from 16 different specialties.
Team members rely on research, as well as clinical expertise, to address the patients' needs and desires. "Being able to knowledgeably discuss the impact and implications of an anomaly or a treatment, using data, not anecdotes, provides a foundation for us to work with families," says Ronald Strauss, professor of dental ecology and social medicine. "It also helps us to provide better treatment. We ask questions we might not have asked otherwise, and we can answer more questions for the patients and their families."
"We do this work to serve the people of the state," says Warren, "But it's also a learning opportunity for us."
For 33 years, with continuous support from the National Institutes of Health (NIH), Warren and some of his colleagues have been exploring fundamental questions about speech and breathing. Because clefts, and the reduced or obstructed nasal passages that often accompany them, direct more air through the nose, people with clefts speak and breathe differently from people without clefts. They may learn to compensate for the unusual airflow, which makes their speech less nasal-and also less intelligible.
"Why do they develop compensatory strategies? That's what we wanted to know," Warren says. "It's unusual because these strategies undermine, rather than enhance, speech."
Speech is regulated by the tautness, thinness, and position of the vocal cords, and by respiratory effort. Manipulation of the vocal cords and other respiratory-tract structures controls pressure in the lungs. Robert Mayo, assistant professor of speech and hearing; David Zajac, a speech scientist; and Warren tested the speech system's response to congenital deficits and experimental perturbations by conducting pressure-flow assessments, measurements of the air flow through the nose and mouth. They learned that people with clefts develop compensatory strategies, sacrificing speech quality, to maintain the pressure in their lungs.
Years ago, Warren and some collaborators designed and built the first pressure-flow apparatus, which is now being produced by Microtronics, a company in Chapel Hill. The craniofacial team uses the apparatus as a diagnostic, as well as a research, tool. A more recent project, headed by Ryuta Kataoka, a surgeon and visiting researcher from Japan, uses acoustic measurements in lieu of the aerodynamic pressure-flow assessments. The goal is the same: to identify respiratory tract anomalies with a non-invasive tool.
Breathing, as well as speech, is affected by craniofacial disorders characterized by reduced or obstructed nasal airways. "If you are sleeping and someone closes your nose slowly, by pinching the nostrils, your mouth will open just enough to compensate," Warren explains. "Most people are nasal breathers, but most people with a cleft become nose and mouth breathers. What type of nose do you need to be a nasal breather?"
To answer questions about speech and breathing in cleft lip and palate patients, Warren and his colleagues have been collecting data on people without clefts. One longitudinal study, started in 1986, has been following the development of speech and breathing in a group of 115 non-cleft children in Finland. Another study looks at an older population, people aged 60 and above, also in Finland. The observations from these studies will serve as a reference for the data collected from people with clefts.
The relationship between breathing and sense of smell is being explored by Martin Kendal-Reed, an experimental psychologist from England. Using a device called an olfactometer, Kendal-Reed simultaneously introduces a precisely controlled amount of chemical stimulus and documents the physiological responses in the nose. He is collecting data from normal and anosmic (no sense of smell) subjects to study how anomalies, both congenital and acquired through accident, disease, or surgery, affect the sense of smell. The results indicate that two sensory systems are involved in smelling: a sense of "touch" and a sense of smell. People who lack the sense of smell retain the sense of "touch," characterized by a tingling sensation when a large amount of stimulus is used. By clarifying the fundamental processes involved in smell sensations, Kendal-Reed hopes to identify ways to help anosmic patients.
Gerald Sloan is aiding patients by evaluating two surgical techniques for repairing velopharyngeal incompetency, which can interfere severely with speech. He heads a five-year, multi-center study, funded by NIH, to compare the pharyngeal flap technique, which improves speech but disrupts nasal drainage and causes sleep apnea in some patients, to pharyngoplasty, which is not as obstructive but may not improve speech as much. Sloan and his colleagues are evaluating patients using medical exams, x-rays, sleep studies, and speech and hearing tests before and after surgery to determine which treatment, if either, is preferable.
Other work on the impact of craniofacial anomalies and their repair is being done by Timothy Turvey, chair of oral and maxillofacial surgery, in conjunction with the Hospital for Rehabilitation in Bauru, Brazil. Because it is the largest craniofacial center in the world, the specialists can collect information rapidly about the effects of surgical treatment on speech, occlusion, and breathing.
At UNC-CH, Malcolm Johnston, professor of cell biology and anatomy and of dental research, and Kirt Simmons, assistant professor of orthodontics, have been studying very unusual sets of identical twins-one twin has a cleft and one does not-to understand the cleft's impact on growth and appearance. "If there's a cleft in the palate, you can expect the patients to look different because they don't grow normally," Simmons explains. "We're trying to see the effect of both the cleft and of the surgeries to correct it." One study involves more than 30 sets of twins, with the twin who never had a cleft serving as a reference for the twin who does, or did. In a more detailed study, following three sets of these twins from birth to 13 years, Simmons and Johnston observed that the twin with a cleft had a shorter lower jaw from birth and that the differences in jaw size decreased as the children grew. In a third study, Johnston has been comparing non-cleft twins to people with no family history of clefts, in an effort to identify factors that increase a person's susceptibility to cleft palate.
Instead of the scientific questions surrounding craniofacial anomalies, the social and psychological needs of the patients motivate some team members' research. "Twenty-odd years of surgery, speech therapy, genetics, hearing problems, and the rest-it adds up," Strauss says. "Our patients are a reminder of the complex of social, psychological, and medical issues and of the ways they overlap."
One of the confounding factors of particular concern to Strauss is the economic impact associated with the birth of a child with a craniofacial anomaly. "The birth of a child like this is a personal and family-and also an economic-crisis," he says. "The third or fourth question the parents ask after the child's birth is, 'How am I going to pay for this?'"
Strauss and his colleagues also have dealt with how craniofacial patients view themselves, how other people view them before and after surgery, and how the patients interact with other people. Some of this research, such as a recent study in which parents described how physicians told them about their children's craniofacial anomalies, led the researchers to suggest guidelines for encounters between the patients and the specialists. Most of the time, Strauss says, the goal is not to develop new techniques for dealing with situations but to recognize the needs and the concerns of the craniofacial patients. "The papers lead to increased awareness by the family, the patient, and the team," he says, "And that's what makes the difference."
Last modified: 5/20/96