More babies born with cleft palates
Kathy Manwaring knew there was something wrong with her baby immediately after giving birth to Timothy, 18 years ago.
“The doctor said, ‘Oh, no,’ ” Manwaring recalled. “Then they brought him in and the other mother with a normal, I mean typical, baby was looking.”
So the hospital moved Manwaring, of Clarkston, Wash., into a private room where she spent the next five days struggling to feed Timothy, who was born with a cleft lip and palate, one of the most common birth defects. Depending on the severity of the cleft palate, a baby may have difficulty feeding. Later in life, the child may develop ear or dental problems as well as delays in speech development.
In Spokane County, the incidence of oral-facial cleft has been unusually high in the four years ending in 2004, the last years for which data is available, according to Laurie Vessey, nurse coordinator for the Spokane Regional Health District’s maxillofacial program for the past 14 years. Although there has been no scientific study done, the best information available shows that the rate of oral-facial cleft in Spokane County in recent years has been about twice the national average of one in 700 to 750 births, she said.
Data from 2005 will be available in July, Vessey said, but it appears as if it will be a high year as well. Vessey believes the incidence could be even higher because neonatal units do not track cleft palate in infants who do not survive to leave the hospital.
“The neonatals do not call me,” she said. “The baby died and they move on.”
A cleft lip is the separation of the two sides of the lip resulting in an opening between the upper lip and the nose. A cleft palate is an opening between the roof of the mouth and the nasal cavity in which the two sides of the palate did not fuse. The lip and palate develop separately in the first several weeks after conception, so a child could have a cleft lip, a cleft palate or both.
Such oral-facial clefts also are associated with more than 400 genetic syndromes, and up to 13 percent of babies born with a cleft may have other birth defects, according to the March of Dimes. Babies born with cleft lip and palate likely will have at least two surgeries before their first birthday, one to repair the lip and one to repair the palate. They are often just the first of numerous childhood medical procedures.
Parker Button, 11, has had more than 20 procedures since he was 10 or 12 months old, said his mother, Angel Button. The Clayton, Wash., mother and son attended an evaluation on Friday by the cleft palate review board, a team of health and social services providers that gathers every month on the Washington State University Riverpoint Campus to look at children and determine what can be done to improve their speech, their looks, their lives.
The program, which is nearly 40 years old, is funded by the state Department of Health.
Parker has been attending these sessions since he was a newborn. Thanks to ultrasound, his mother knew even before he was born that her son had a cleft. She said knowing ahead of time gave her time to prepare for the ordeal to come.
“When you are a parent going through all this … you are in survival mode,” she said.
As an infant, Parker wore a device to bring his palate together so that surgery could be done. She said she had to tighten the screws that dug into her baby’s mouth.
She recalled the torture of his tears, but also the joy in his little smile.
When the device was being installed at the doctor’s office, Parker momentarily stopped breathing during the procedure.
“Now it is only done in hospitals,” Button said.
The list of Parker’s procedures is long. The device was installed in May 1995. In September 1995, lip adhesion was performed and tubes were installed to relieve pressure on Parker’s eardrums. His palate was repaired in 1996. There was a “lip-nose revision” in 1997.
In 1999, Dr. Elizabeth Peterson, a review board member, performed plastic surgery on Parker’s nose and straightened his septum. The boy began orthodontic treatment in February of 2003, and in 2004 Dr. Mark Paxton, another review board member, performed a maxillofacial bone graft. Last year, new tubes were installed to relieve pressure in his ears.
“Surgeries are the worst,” Parker said.
On Friday, Paxton and orthodontist Steve Gregg, who work in tandem at the monthly cleft palate clinics, recommended a new bone graft as soon as possible and a titanium dental implant.
Families of children with cleft palates have difficult choices to make about how much medical and dental care is enough and how much they can afford. Many children have deficiencies in the forward development of their upper jaws, which often must be corrected with surgery. There are also the issues of missing, extra or malformed teeth and room for teeth to emerge.
“The orthodontist and oral surgeon work together to achieve facial balance,” Gregg said. “It’s a long road.”
Surgery for these children may end at 20 or 21 years old, he explained, but orthodontic maintenance is forever.
The families of children with clefts come to the clinic for the information necessary to make such choices. They go from room to room, meeting with the experts who will gather later in the day to discuss each patient’s needs. Besides Peterson, Paxton and Gregg, there is social worker, a genetic counselor, an ear, nose and throat specialist, an audiologist and two speech pathologists.
Most children with clefts have articulation errors, said Sandy Bassett, a speech pathologist and associate WSU professor. They make substitutions or omit sounds. The children are more prone to ear infections, so they are not hearing as well and their language skills are delayed. Almost all undergo six months to a year of speech therapy before surgery is recommended to improve speech.
Nasal endoscopy can determine whether the palate is short or does not move well. These conditions can result in “hypernasality” in which sounds come from the nose rather than the mouth.
“We want to make sure speech does not draw attention to itself, so that you are listening to what they say, not how they say it,” Bassett said.
In another room, Dr. Michael Olds attempted to perform an endoscopy on a 7-year-old who would have none of it. Though these children have put up with a lot in their young lives, everyone has a breaking point.
“How many doctors have poked him over how many years?” Olds asked after letting the boy forgo the procedure this time.
Children with clefts are resilient but some, especially adolescents, have unrealistic expectations about how they will look after a surgery, said social worker Audrey Burr. This can cause depression or even suicide. Some have other medical or behavioral problems, developmental disabilities or family problems.
“Parents will often treat their kids with disabilities differently and they grow up expecting to be treated specially,” Burr said. “Often schools and employers will not and the child has to come to terms with that.”
The family of a child with birth defects wants to know why, said genetic counselor Lael Hinds.
“For a couple thinking about other children, you can help them make informed decisions about reproductive help,” Hinds said.
If either parent has a cleft, the chance of having a child with a cleft is between 4 and 6 percent, according to the March of Dimes.
Clefts are more common in some races, such as Asian, and less common in others, such as blacks.
Though the causes of isolated cleft lip and palate are not well understood, experts believe it can result from genetic and environmental factors, or both, according to the March of Dimes. Infections, maternal illnesses, and drug, tobacco or alcohol use may all be factors, as well as vitamin B or folic acid deficiency.
As if it were not difficult enough growing up with a cleft, these children must also overcome social obstacles.
Timothy Manwaring’s mother said her son often found friends who were older and more accepting. She is proud of her son, who has become an Eagle Scout despite having to endure 25 medical procedures, including maxillofacial and plastic surgery.
“It was hard at times,” Timothy Manwaring said. “There are some who make fun of you. A lot of people ask, ‘What happened to you?’ It’s something you learn to deal with.”