The role of the audiologist on the cleft palate-craniofacial team is to diagnose, monitor, and (re)habilitate hearing loss, as well as to make educational recommendations/modifications and medical referrals as needed. The audiologist works closely with the otolaryngologist (ENT) because of the high incidence of middle ear infections in children with cleft palate. Hearing testing begins at birth (OAE/ABR testing) with frequent re-testing/monitoring thereafter. Behavioral testing and middle ear assessment (tympanometry) are performed at least twice a year for most children, more often if needed. Early diagnosis and treatment of hearing loss is crucial for optimal speech/language, social and educational development.
As you welcome your newborn baby, feeding is one of the first needs parents will have to meet. Infants born with a cleft lip and palate need time like any other baby to learn to feed. Depending on the type of cleft your baby has, there may be less sucking strength with feedings. Several bottles and nipples are available to help your baby feed and grow.
Feeding a Baby with a Cleft Lip Only
These infants usually feed without difficulty by breast or bottle. A lactation consultant can help you find a breastfeeding position that works for both you and baby. It is important that you have regular visits with your baby's pediatrician to check your baby's weight and growth. As long as your baby is gaining weight when you go to the pediatrician, there is no need to stop breastfeeding or use special bottles. If your baby does need to have some bottle feedings along with breastfeeds, then you may pump your breast milk and give it to the baby in a bottle.
Feeding a Baby with a Cleft Palate (With or Without a Cleft Lip)
Babies with cleft palates usually need special bottles to feed. For mothers wanting to breastfeed, giving your expressed (pumped) milk in a bottle will give your baby all of the benefits from breast milk. Most babies with a cleft palate are not able to exclusively breastfeed due to less sucking strength from their cleft palate. These babies often do best and have better weight gain with mostly bottle feedings. Some attachment at the breast is possible if the baby is not having difficulty gaining weight. Your baby's pediatrician, the lactation consultant, and the speech therapist can help you know which feeding methods and bottles will work for your baby.
Giving Your Baby Breast Milk
Your breast milk has antibodies to help prevent infections in the baby. After delivery of the baby in the hospital, a mother may start using an electric breast pump. Double pumping of the breasts is recommended 8 times a day, or every 3 hours for 10 to 15 minutes at each pumping session. The lactation consultant can help adjust the pumping schedule to meet both the mother's and infant's needs. Any amounts of breast milk you are able to feed your baby will benefit them. Before a mother leaves the hospital, arrangements may be made to rent a hospital-grade electric breast pump. A social worker and lactation consultant can help with the rental and possible insurance coverage.
Bottles and Nipples
There are several bottles and nipples recommended for babies with clefts. The milk for your baby will flow easier with the following bottles and nipples:
Mead Johnson Cleft Palate Nurser
This is a soft plastic bottle that needs to be gently squeezed as the baby sucks with pauses as the baby swallows. The nipple has a cross cut for an easier milk flow. Practice squeezing and releasing the bottle with your breast milk or formula before you feed the baby for the first time. Remember to pause to let the baby swallow.
Ross Orthodontic Nipple
This nipple has a wider base and sometimes a fast flow cross cut. Some babies will do better with this nipple. It may be placed on the Mead Johnson Cleft Nurser squeeze bottle.
Medela Haberman Feeder
This bottle has a soft nipple with a slit. The bottle does not have to be squeezed. The nipple has 3 raised line markings on it that control how fast the milk will flow. The longest line will have the easiest flow. Gentle pressure may also be placed on the nipple if needed to promote better flow and feeding. The lactation consultant and speech therapist can help you learn how to use this bottle.
Suggestions for Feeding Your Baby
- Feed the baby in an upright position.
- Burp the baby frequently.
- Babies do better if the feeding is able to be done in 30 minutes. If the feedings start to take longer than 40 minutes you may need to change to a different nipple or bottle. Call the cleft palate team coordinator.
- If your baby is having difficulty gaining weight, the pediatrician may add a powder to your pumped breast milk or formula to add extra calories to help your baby grow. This may only be done by the baby's pediatrician.
- See Feeding Your Baby with a Cleft Lip and/or Palate.
Genetic counselors are health professionals with specialized graduate degrees and experience in the areas of medical genetics and counseling. Most enter the field from a variety of disciplines, including biology, genetics, nursing, psychology, public health, and social work.
Genetic counselors work as members of a healthcare team, providing information and support to families who have members with birth defects or genetic disorders and to families who may be at risk for a variety of inherited conditions. They identify families at risk, investigate the problem present in the family, interpret information about the disorder, analyze inheritance patterns and risks of recurrence and review available options with the family.
Genetic counselors also provide supportive counseling to families, serve as patient advocates, and refer individuals and families to community or state support services. They serve as educators and resource people for other healthcare professionals and for the general public. Some counselors also work in administrative capacities. Many engage in research activities related to the field of medical genetics and genetic counseling.
Genetic counselors often work within sub-specialties, such as prenatal services, general pediatrics, hereditary cancer clinics, inherited metabolic disease clinics, cystic fibrosis, craniofacial disorders, hemoglobinopathy screening programs, neurocutaneous disorders, and many others.
Among the services provided by genetic counselors are the following:
Prenatal Genetic Evaluations for
- Family history concerns
- Ultrasound abnormalities
- Abnormal test results during pregnancy
Neonatal Genetic Evaluations for
- Isolated birth defects
- Multiple congenital anomalies
- Failure to thrive
Pediatric Genetic Evaluations for
- Any of the above reasons
- Developmental delay or mental retardation
Adult Genetic Evaluations for
- Any of the above reasons
- Genetic disease carrier screening
- Presymptomatic testing
Components of a Genetic Evaluation
- Comprehensive family history
- Ultrasound examinations
- Prenatal laboratory testing
- Medical and developmental history
- Dysmorphology (birth defects and minor abnormalities and normal variation)
- Prenatal diagnosis and other diagnostic or investigative testing when appropriate
- Significance of findings and appropriate associations
- Diagnosis/differential diagnosis
- Impact on family members
- Family/individual regarding diagnosis and recurrence risks
- Prognostic information
- Management needs/follow-up
- Testing for other concerned family members
- Refer to other specialties
- Family/individual decisions
- Crisis management
- Provide anticipatory guidance
- Provide community/national support information and resources
Oral and Maxillofacial Surgeons
Patients with cleft lip and palate deformities require a team of specialists to properly follow and manage the surgical and non-surgical care that many of these patients need. The oral and maxillofacial surgeon is an integral part of this team, and is responsible for reconstructing bony deformities of the jaw that often present in these patients.
Following the repair of the lip and palate at an early stage of growth, the oral surgeon must address the bony defect of the jaw at the cleft site and close the communication between the mouth and the nose. The timing of this treatment is usually governed by the development of the maxillary canine tooth. More specifically, the bone graft should be placed at the cleft site about 6-8 months prior to the eruption of the canine tooth (age 7 through 9). This schedule will ensure that there will be adequate bony support for this important tooth.
Some patients with cleft lip and palate deformities will also develop malocclusions and jaw size discrepancies (under bite, over bite) due to a restriction in facial growth. These types of dentofacial deformities are usually treated by conventional orthognathic surgery (jaw surgery) by the oral and maxillofacial surgeon during the teenage years of development.
Similar to other specialists associated with the cleft palate team-craniofacial at Stony Brook, the oral and maxillofacial surgeons work closely with other team members to assure that patients receive the best care possible at all times.
Orthodontists are involved with the study and guidance of the growth and development of the face, and the arrangement of teeth of the child with a cleft or craniofacial anomaly from birth to maturity. Their role includes diagnosis of changing facial shape and function due to treatment and growth. They provide orthodontic and orthopedic treatment and general expertise for consultation with all of the other members of the cleft palate-craniofacial team. Because of the long-term treatment required for the majority of patients, different phases of active treatment, interspersed with periods of retention or no treatment, will be necessary.
The otolaryngologist (ear, nose and throat specialist) plays an important role in the child born with a cleft palate. The reason is that the child will have a difficult time clearing fluid out of the ears, especially after an ear infection. This happens because the muscle of the palate that helps to open the eustachian tube does not work well to equalize pressure and therefore cannot clear the fluid as easily. The only way to clear the fluid is to artificially place a ventilation tube in the ear. Therefore, almost all children with a cleft palate will require a set of ear tubes. Removing the fluid is very important for improvement in hearing (because the presence of fluid can cause a hearing loss) and also for speech and language development. The child's hearing is tested prior to, and after the procedure.
The otolaryngologist places the ear tubes under general anesthesia. After surgery, the child will have only mild discomfort from placement of the ear tubes. This can be relieved with some Tylenol. He/she can be taken home the same day if only the ear tubes are being placed. If the surgery is being done at the same time as the repair of the cleft palate, then the child will be admitted to the hospital. The child will usually need antibiotic ear drops after surgery for at least three days. The child will need to see the ear nose and throat specialist a few weeks after the surgery to check the ear tubes and the hearing. These ear tubes and the child's hearing will also need to be followed periodically throughout childhood.
The ear tubes may “fall out” on their own. When they do, if the body again cannot clear the fluid on its own, then another set of ear tubes would become necessary. This is still true even after the plastic surgeon repairs the cleft palate. This is due to the fact that the muscle that opens the eustachian tube is still not able to function normally because of the expected scar tissue that forms with healing.
Some children may require several sets of ear tubes in their childhood. It may take a child with a cleft palate longer to “outgrow” the fluid clearance problem than a child without a cleft, so the child may need to be followed until the teenage years. While the ear tubes are in place, it is critical to keep all water out of the ears. The reason for this is that water contains bacteria that can then set up an infection in the middle ear, and the child will have pus draining from the ear, as well as ear pain and fever. (Just like an ear infection prior to placement of the ear tubes, but instead the pus can now come out.) Therefore, it is very important to use one of the three following methods to keep water out of the child's ears:
- Cotton with Vaseline (Vaseline is a water repellant)
- Ear plugs made of silicone (Drug store)
- Custom made ear molds (Doctor's office)
If the child swims or puts his/her head underwater, then a waterproof headband should be worn for double protection. It also helps keep the plug in place.
Once again, the role of the ear, nose and throat specialist cannot be overemphasized. You will see this doctor at your initial cleft palate team visit. If you have any questions, please have them ready to be asked at that time. If your child is having hearing problems, you may see the ear, nose and throat specialist in the office before the first team visit.
The coordination of care for the child with a cleft palate is very important because there are many specialists who need to see and care for the child. The team members will do everything possible to schedule necessary surgeries together. However, if any particular surgery becomes necessary sooner, this can also be done if it is beneficial for the child.
By request of the plastic surgeon, the pediatric dentist evaluates and provides appliance therapy to enhance feeding for infants with cleft palate and those infants whose alveolar clefts require reduction in preparation for primary lip surgery by the plastic surgeon. The pediatric dentist counsels families in the appropriate home care for their infant or child to prevent dental caries, which is common for these infants because of feeding concerns. The pediatric dentist provides oral care for those children who suffer tooth decay or need interceptive orthodontic care for bite problems in the primary dentition.
The plastic surgeon works closely with all team members. The plastic surgeon’s involvement starts at the time the diagnosis of a cleft lip/palate has been made or is suspected. This may be before birth, if the diagnosis is made on ultrasound, or more often at birth. The plastic surgeon assesses the child at birth and a treatment plan is formulated for repair of the cleft lip/nose deformity and/or cleft palate.
The baby is evaluated with the pediatric dentist at birth and a decision is made as to whether the baby would benefit from placement of a dental appliance to help align the maxillary arches, reduce the cleft size, and improve on nasal contour. If so, the pediatric dentist makes the appliance and adjusts it often over the ensuing months to align the arches and facilitate the repair of the cleft lip/nose deformity. The cleft lip/nose is repaired between three and six months. The infant is admitted overnight and sent home one to two days later once he/she is feeding well.
The cleft palate is repaired when the infant is between 9 and 16 months of age. The palate is ideally repaired at this age prior to the child beginning to speak. This facilitates the development of normal speech. The child will subsequently require speech therapy to help achieve optimal speech. In general, at the time of the cleft palate repair, ear tubes are placed by the otolaryngologist in order to prevent ear infections and improve hearing which will help in the development of normal speech. The surgery is performed in the hospital, and the child is usually in the hospital for two to three days.
If the child is felt to have persistent speech problems (hypernasal speech) by age 4 to 6 by the speech therapist, then a videonasoendoscopic evaluation is performed. The videonasoendoscopy is performed in the office with the speech pathologist and otolaryngologist and plastic surgeon. Depending on the findings, another intraoral surgery known as a pharyngeal flap may be needed to help improve speech. This is usually performed between ages 4 and 6.
At age 5 to 6 a touch-up procedure may be necessary on the repaired cleft lip and nose to improve on the cosmesis (looks). This is usually performed as an outpatient procedure.
At age 16 to 18 the adolescent is evaluated and, if necessary, a rhinoplasty (reshaping of the nose) and/or scar revisions on the repaired cleft lip and nose is performed.
The social worker provides clinical and concrete services to patients and their families. The social worker functions as a counselor, advocate, and coordinator of appropriate community services. The social worker provides psycho-social assessments and intervention for patients and families where appropriate, when problems impact on treatment, or when the health or welfare of the child is jeopardized. The social worker provides resource education and advocacy as well as counseling, and works collaboratively with the interdisciplinary treatment team.
The speech-language pathologist plays an active role in the management of children with craniofacial anomalies including cleft palate. Evaluation and treatment of feeding, speech, language, and resonance disorders are imperative to optimize each child's oral feeding and communicative potential.
A newborn with a cleft lip/palate may have some difficulty with breast/bottle feeding. The speech pathologist, in conjunction with the lactation specialist, provides adaptive feeding techniques and guidance for positioning and feeding modifications.
With the exception of children with cleft lip only, speech-language evaluations are provided at our clinic approximately 3 months post-repair of the soft palate to determine baseline functioning and again at 2½ years of age to assess improvement in articulation and language skills. Additional re-assessments by community and/or school-based speech pathologists occurs annually thereafter. Frequent contact is made with treating speech pathologists to jointly develop individualized treatment plans.
At approximately 4 years of age, a speech resonance Evaluation is conducted to assess nasal airflow for children with previous concerns of “nasal sounding” speech. Perceived hypernasality, hyponasality, mixed nasality, and nasal turbulence are assessed during specific speech tasks. Objective testing is then performed using the floxite mirror, See-Scape, and Nasometer (a device which measures oral air pressure and nasal air pressure separately and calculates a ratio between the two).
For children presenting with hypernasality on a speech resonance evaluation, a videonasoendoscopy is recommended. This test is performed in conjunction with a plastic surgeon and otolaryngologist. Findings on a videonasoendoscopy will determine the course of intervention regarding continued speech therapy, the need for a prosthesis, and/or surgical management. Re-assessment of speech resonance is then performed within 12 months to determine the outcome of the treatment plan.
The coordinator of the Cleft Palate-Craniofacial Center is responsible for the coordination of care for the patient in collaboration with other members of the cleft palate team. The coordinator provides support for patient/families through referrals and obtaining necessary information from specialists. The coordinator will also assist families with insurance referrals, billing issues, and letters to insurance companies. The coordinator is the central person to contact with any questions or concerns regarding the diagnosis and care of their child in order to be directed to the proper specialty for appointment.