Education on certain birth defects a long journey
Cleft lip, palate a process from birth to age 18
Preparation for caring for a child with a cleft palate or cleft lip begins before a baby is born, when ultrasound reveals there is a problem.
It is a journey that lasts from infancy to 18 years of age.
"I tell people that cleft is not life or death; however, it is a long journey, and that is what I try to prepare my parents for. I tell them they will be doing cleft care until their child reaches adulthood," says Alison Repass, MSN, CPNP, a pediatric nurse practitioner in the Center for Craniofacial Disorders at Children's Healthcare of Atlanta.
A cleft lip results when the upper jaw and nasal area fail to close, resulting in one or more clefts in the upper lip. A cleft palate is a birth defect in which there is a hole in the middle of the roof of the mouth. Frequently, these conditions occur together. Both problems are repaired by surgeries.
Repass has found when parents come to the center they have no idea of the timing of the surgeries or the expected number. A lot of times parents think only one surgery will be needed, but often more are required. Therefore, she goes over a cranial facial care map with families.
Once the baby is born, Repass accompanies the surgeon to the birth hospital to visit the family and assess the severity of the cleft. At that time, the baby also is evaluated for other cranial facial anomalies such as a small jaw.
Feeding issues are discussed, as well, as part of a feeding and nutritional evaluation. Babies have difficulty sucking, so breast feeding often is impossible and usually a special cleft bottle must be used. Repass shows parents the different bottles available. After parents choose one, staff make sure they are using the bottles correctly so the babies are meeting their caloric needs, says Repass.
Often staff at the birth hospital are not skilled in feeding infants with cleft and rely on the center to help train the mothers and fathers in how to feed with the cleft bottles. Repass says that the parent feeding the baby must know where to place the nipple of these special bottles and how to manipulate them with their hands.
"It is hands-on practice. The feeding clinic is done by an occupational therapist and a nutritionist," explains Repass.
Parents also are told about pre-surgical molding that is made shortly after the infant is born. It is like a retainer used by orthodontists, but in this case it is used to bring the lip or palate closer together so they can be repaired more easily.
"The molding often allows us to create a better cosmetic result, because the tissues have grown closer over the months," Repass says.
The first few months before the repair of the lip or palate, the orthodontic appliance is manipulating the tissues so as to reduce the gap that must be closed surgically. Then, typically, the care team at the Center for Craniofacial Disorders repairs the cleft lip at three to five months of age and the cleft palate at six to eight months of age.
Several team members required
In addition to meeting with the occupational therapist and nutritionist for a lesson on feeding, the family meets with the cranial facial surgeon and if the baby is determined a candidate for the pre-surgical molding device they see an orthodontist. Also at this time, families are encouraged to meet with the geneticist to determine if the baby has a functional cleft or if the cleft is associated with a syndrome, which might mean he or she has other health issues, such as cardiac or pulmonary problems.
Children who have a cleft palate are evaluated by a speech pathologist when they are 1 year old and seen at the center every six to 12 months. They have weekly therapy within their local community.
Also, children with cleft palates have frequent ear infections and may need an ear, nose, and throat specialist. They also will need a dentist and orthodontist who are familiar with cleft lip and palate.
If the child has a complete cleft lip and palate, parents are told right away that future surgeries will be necessary. A lip and nose revision might be needed, which is done right before a child enters kindergarten, and a bone graft will be done when the child is middle-school age.
During the teen-age years, rhinoplasty and some revisions to the mouth might be done. This work is done when skeletal growth is complete and is for better cosmetic results. By the time the teens are 18 years old, the problems have been taken care of; however, they may want more cosmetic dentistry or surgeries on their face, says Repass.
The first surgery to repair the cleft lip takes about an hour and a half and requires an overnight stay at the hospital. The baby wears arm restraints for about a week to prevent him or her from pulling out the stitches. Once the stitches are out, Repass gives the parents information on ways to help soften the scar.
"Parents want to hear what they can do to make their baby look more normal," says Repass.
Care instructions for surgery on the cleft palate include information on protecting the area that is healing. For example, parents are told not to give the baby any food that is sharp or to use straws because they could puncture the palate.
Repass lets parents know that future surgeries are common, as complications can occur. For example, the palate can open up again in certain places and more repair work must be done. In addition a surgical procedure to lengthen the palate may be required if the child continues to have speech problems.
When the children are middle-school age and teens, Repass works with them as well as the parents. She understands what it is like to go through the surgeries, as she was born with a cleft lip and palate.
"A lot of my education for these children is explaining how I think they may be feeling and the process of what I know professionally and what I know personally," says Repass.
Sometimes the conversation is about the child's feelings and their outlook on the upcoming surgery. The children often ask informal questions as well, such as how Repass reacted when other children made fun of her.
In the beginning there can be barriers to education. For example, the problem may not have been caught on ultrasound so the parents have not had prenatal education. However, most know about the birth defect before the baby is born, said Repass.
Often parents don't want to hear about the long repair process; they just want to know when the lip or the palate will be fixed. Repass says parents often are in denial about the longevity of the problem, so she tries not to overwhelm them and sticks with education on the infant's immediate needs.
Often small hospitals aren't familiar with the treatment process for cleft lip and palate and send the families to surgeons. Repass tries to outreach to local hospitals to provide staff education.
"I try to educate them on the technology of cleft today and how early we do repair," she explains.
For more information about education on cleft lip or palate, contact:
- Alison Repass, MSN, CPNP, pediatric nurse practitioner, Center for Cranofacial Disorders, Children's Healthcare of Atlanta. Phone: (404) 785-3674. E-mail: Alison.Repass@choa.org.