Cleft Lip and Cleft Palate

1. Cleft Lip and Cleft Palate Pam Kraus, Janelle O’Connell, Jessica Daiker Amy Barron, Laura Underwood, Jaci Gauna

2. Overview: • A cleft lip or palate is an oral-facial birth defects in which the tissues of the mouth or lip don't form properly during fetal development. • In the United States, clefts occur in 1 in 700 to 1,000 births, making it one of the most common major birth defects. • A cleft lip may be just a small notch in the lip. It may also be a complete split in the lip that goes all the way to the base of the nose. • A cleft palate can be on one or both sides of the roof of the mouth. It may go the full length of the palate. • The incidence of cleft palate alone is one in 500 live births • Considering the cleft deformities of all races grouped together, 50% are cleft lip and palate, 30-35% are palate only, and 15-20% are cleft lip only.

3. The cleft palate was not recognized as a congenital disorder until 1556. • For centuries, perforations of the palate were considered to be secondary to syphilis. • The first successful closure of a soft palate defect was reported in 1764 by LeMonnier, a French dentist. • The first closure of the hard palate was performed in 1834. • Clefts occur more often in kids of Asian, Latino, or Native American descent. • More boys than girls have a cleft lip, while more girls have cleft palate without a cleft lip. • A child may have one or more of these conditions at birth.

4. Problems that may be present because of a cleft lip or palate are: • Failure to gain weight • Feeding problems • Flow of milk through nasal passages during feeding • Misaligned teeth • Poor growth • Recurrent ear infections • Speech difficulties

6. Pathophysiology • Cleft lip: • Caused by incomplete fusion of the nasomedial or intermaxillary process during the 2nd month of embryonic development • Cleft causes structures of mouth and face to develop without the normal restraints of encircling lip muscles • May affect external nose, nasal cartilages, nasal septum, and alveolar processes also • Usually just beneath the center of one nostril • Can occur bilaterally, symmetrically or asymmetrically • More complete the cleft lip, the greater the chance that teeth in the line of the cleft will be missing or malformed • Complete cleft= entire thickness of the lip • Incomplete cleft= only a portion of the lip is involved

7. Pathophysiology • Cleft Palate: • Often associated with cleft lip, but may occur without it • Fissure may affect only the uvula and soft palate (secondary palate, formed ~ 9 weeks), or may extend forward to the nostril and involve the hard palate and the maxillary alveolar ridge (primary palate, formed ~ 4-5 weeks ) • Complete= involves the primary and secondary palate • Incomplete= involves the secondary palate only • Unilateral= on one side the palatal process of the maxilla is fused with the nasal septum • Bilateral= not attached to the nasal septum, and the septum is visible through the cleft • Cleft occupies the midline posteriorly and can go as far forward as the alveolar process. Clefts involving the palate only are usually midline • Some cases, the vomer and nasal septum are partly or completely undeveloped • When these facial bones are involved, the nasal cavity and the oral cavity are open to each other

8. Cultural Beliefs Regarding Cleft Lip and Cleft Palate • South Africa: • 2006 study: participants included 35 cleft patients and their families • Beliefs about the cause: • God • Witchcraft • Ancestors punishing the mother • Fate • Genetics and family history of the condition • 2007 study: looked at the beliefs and practices of religious healers • Most common belief for both Muslim and Hindu healers= it was God’s will or because of an eclipse • Both also identified evil spirits, witchcraft and genetics as causes • Hindu group identified karma as being a possible cause • Both groups reported that their patients came to traditional healers because they were looking for comfort, or to be relieved of guilt because of their culture and what their ancestors did • Beliefs of African Americans in US are similar (punishment for something the mother has done, evil spirits, or displeasing a god)

9. Cultural Beliefs Regarding Cleft Lip and Cleft Palate • Nigeria • 2007 study regarding the beliefs of mothers in Nigeria who had a child with a cleft lip or palate • Identified as Hausu/Fulani (Muslim)= believed cleft was an act of God • Other beliefs included the mother going outside during an eclipse while pregnant or laughing at someone with a cleft lip •  Because of their belief that the cleft is an act of God, may feel less shame related to the condition and also less likely to have it repaired • Identified as Yoruba (Christian)= believed cleft was due to evil spirits or punishment from ancestors • Other beliefs included curses, not having enough food during pregnancy or hereditary/environment factors

10. Cultural Beliefs Regarding Cleft Lip and Cleft Palate • Asia: • 1990 paper discussing the beliefs of people of Asian/Pacific Island descent living in the United States: • Some view a cleft as a gift from God, while others view it as a curse • Many beliefs exist about the role different foods play in causing or healing physical problems • Ex/ eating hare while pregnant could cause a cleft lip • Members of some religions, such as Buddhism, may view a cleft as part of a person's karma and not treat it at all • Beliefs regarding the cause include spirits, negative forces, punishment for the actions of ancestors and eating certain foods • Depending on the belief regarding the cause of the cleft, patient may feel shame

11. Cultural Beliefs Regarding Cleft Lip and Cleft Palate • Latino • Article focused on Latino people (from Mexico, Central and South America, and the Caribbean) who are living in the United States • Eiology of a cleft lip or palate may be accounted for by folklore or mythology • Some beliefs include that cleft is due to natural events (eclipse), actions of the parent (drinking alcohol or having a STD), or emotional factors (stress during pregnancy) • Witchcraft or evil spirits also believed to be a cause • Health care underutilized due to lack of insurance and/or fear of not being a legal citizen of the US

12. Impact on Neonatal Nursing • Nurses need to remember: • to be sensitive when dealing with the family of a baby with cleft lip and palate • Try to remember this may be quite a shock and they may be grieving the “healthy & normal” baby they wanted • Encourage parents to do cares for the baby as you normally would • Sometimes listening is the best way to show empathy, just let them know you’re there and you’re someone they can confide in

13. Impact on Neonatal Nursing • Beyond the cosmetic abnormality, there are other possible complications that may be associated with cleft lip and cleft palate, including the following: • feeding difficultiesFeeding difficulties occur more with cleft palate abnormalities. The infant may be unable to suck properly because the roof of the mouth is not formed completely. • ear infections and hearing lossEar infections are often due to a dysfunction of the tube that connects the middle ear and the throat. Recurrent infections can then lead to hearing loss. • speech and language delayDue to the opening of the roof of the mouth and the lip, muscle function may be decreased, which can lead to a delay in speech or abnormal speech. Referral to a speech therapist should be discussed with your child's physician. • dental problemsAs a result of the abnormalities, teeth may not erupt normally and orthodontic treatment is usually required.

14. Infant with a Cleft Lip and Palate using a Pigeon system bottle, which encourages “active” feeding. • The baby still has to suck, but not as hard and there is a one way valve that doesn’t allow milk to go back into the bottle after it has been sucked through. • This helps the child later on with feeding after surgery has been complete since the baby has already taken an active roll in eating. • Corpak’s are also utilized to provide the baby nutrition without burning so many calories. • Most NICU protocols will not allow the nurse to try to feed for more than 20 minutes. • The remainder of the bottle is then corpaked. • Breastfeeding can still be accomplished but it is recommended to consult a lactation educator before attempting.

15. Impact on Neonatal Nursing • There may be many people involved in management of a cleft abnormality, because the skills of many different areas are needed to help with the problems that can occur with cleft abnormalities. The following are some of the members of the team: • Plastic/craniofacial surgeon- a surgeon with specialized training in the diagnosis and treatment of skeletal abnormalities of the skull, facial bones, and soft tissue; will work closely with the orthodontists and other specialists to coordinate a surgical plan. • Pediatrician- a physician who will follow the child as he/she grows and help coordinate the multiple specialists involved. • Orthodontist- a dentist who evaluates the position and alignment of the child's teeth and coordinates a treatment plan with the surgeon and other specialists. • Pediatric Dentist- a dentist who evaluates and cares for the child's teeth. • Speech and language specialist- a professional who will perform a comprehensive speech evaluation to assess communicative abilities and who will closely monitor the child throughout all developmental stages.

16. Otolaryngologist (Ear Nose Throat specialist) - a physician who will assist in the evaluation and management of ear infections and hearing loss that may be side effects of the child's cleft abnormality. • Audiologist (hearing specialist)- a professional who will assist in the evaluation and management of hearing difficulties the child may have. • Genetic counselor- a professional who reviews the medical and family history, as well as examines the child to help in diagnosis. A genetic counselor also counsels the family regarding risk for recurrence in future pregnancies. • Nurse team coordinator- a registered nurse who combines experience in pediatric nursing with specialization in the care of the child and acts as liaison between the family and the cleft team. • Social Worker- a professional who provides guidance and counseling for the child and family in dealing with the social and emotional aspects of a cleft abnormality and assists the family with community resources and referrals (i.e., support groups).

17. Signs and Symptoms • Split in lip or palate • Symptoms related to location of cleft: • Hard & soft palate: Facial defect • Soft palate: leaking of ingested liquids from the nose • Hard palate: weak, dysfunctional suck, trouble swallowing, gagging, choking • Submucous Cleft- only in muscles of soft palate and hidden by lining of mouth • May not be diagnosed until later in life • Indicated by: trouble feeding, nasal drainage, excessive gas, nasal sounding voice, increased nasal discharge

18. Risk Factors • Family History: Cleft lip more likely to be inherited than cleft palate • Race: More common in Native American, Hispanic & Asian patients • Sex: Males 2x as likely to have cleft lip; Females 2x as likely to have cleft palate • Environmental factors: exposure of fetus to alcohol, cigarette smoke, or drugs • Maternal Nutrition Deficiencies:especially lack of folate • Encourage use of prenatal vitamins.

19. Diagnosis • Physical examination at birth • Ultrasound: not always visible • Genetic testing for parents to determine risk of having additional children with cleft lip/palate

20. Treatment and Interventions • Treatment of the child with CL is surgical and usually involves no long-term interventions other than possible scar revision • However, management of CP involves the care of a multidisciplinary health care team to provide optimum results • includes pediatrics, plastic surgery, orthodontics, otolaryngology, speech pathology, audiology, nursing and social work • Management of both is directed towards closure of the cleft, prevention of complications and facilitation of normal growth and development of the child.

21. Treatment and Interventions • Surgery: • Can make a big difference to the child's ability to grow and develop normally • The baby will usually have an operation to close his or her cleft lip before the age of three months old. • An operation to repair a cleft palate usually happens a bit later, but usually before the age of one year. • Under general anesthesia, the surgeon will trim the tissues and sew the lip together. The stitches will be very small so that the scar is as small as possible. Most of the stitches will absorb into the tissue as the scar heals, so they will not have to be removed later. • Speech and Hearing • A tympanostomy tube is often inserted into the eardrum to aerate the middle ear • Speech problems are usually treated by a speech-language pathologist • Encourage the child's early attempts to make sounds, even before the cleft is repaired

22. Treatment and Interventions • Prevention • If you have had a child with a cleft lip or palate, the chance of any more children you have being affected may be slightly increased • Genetic counseling or testing may provide answers • Daily folic acid supplements in the first month before conception and in the first two months of pregnancy • Avoid alcohol and drug consumption during pregnancy • Nutrition • Assess the infant's nutritional intake, adequate amount of nutrients is necessary to maintain growth • Feeding is best accomplished in an upright position • Special nipples or other feeding devices may be needed • Breastfeeding is usually a viable option and sometimes more successful than bottle-feeding • Avoid the use of suction or objects in the mouth such as thermometers, spoons or straws • Coping • Address parent's emotional needs, answer and acknowledge questions and concerns • Provide support to the child and the family members • Promote attachment/bonding • Emphasize positive aspects of baby's appearance • Express optimism

23. Implications for Nursing Practice • The exact cause of cleft lip and palate is unknown but it is believed to be a combination of genetic (inherited) and environmental factors. • Certain Drugs, illnesses, and the use of alcohol or tobacco while the woman is pregnant are thought to be contributing factors. • restrict alcohol and drug consumption while pregnant

24. Implications for Nursing Practice • FEEDING • Cleft lip= makes it more difficult for an infant to suck on a nipple • use special nipples to allow the baby to latch properly (either pump or use formula) • Cleft Palate= may cause formula or breast milk to be accidently taken up into the nasal cavity • don’t feed baby without palatal obturator (prosthetic palate) • feed in an upright position to keep milk from coming out of the nose • Babies are more susceptible to altered nutrition (inadequate) related to difficulty eating due to physical defect • SPEECH • Muscle function is decreased and cleft lip or palate makes it more difficult for the child to form words properly • need to see a speech therapist at 18 – 24 months

25. Mead Johnson/Enfamil Cleft Feeder Special Needs Feeder / Haberman Feeder Pigeon Feeder Dr. Brown’s Natural Flowto relieve gas

26. References: • Author Unknown. (n.d.) Lucile Packard Children’s Hospital at Stanford. Retrieved January 28, 2010, from http://www.lpch.org/diseaseHealthInfo/healthLibrary/craniofacial/cleft.html • Author Unknown (2009, June). Cleft Lip and Palate. Retrieved January 30, 2010, from http://hcd2.bupa.co.uk/fact_sheets/html/cleft_lip.html • Bartoshesky, L.E. (2010). Kids Health: Cleft Lip and Palate. Retrieved January 28, 2010, from http://kidshealth.org/parent/medical/ears/cleft_lip_palate.html# • Cute, S. (2009, July). Cultural Considerations in Cleft Lip and Palate: Improving Relationships and Health Care Delivery Between Patients and Professionals. Knol, 25. Retrieved February 1, 2010, from http://knol.google.com/k/cultural-considerations-in-cleft-lip- and-palate# • Hockenberry, M. J. & Wilson, D. (2007). Wong's Nursing Care of Infants and Children, 8:458- 465. St Louis: Elsevier. • Huether, S.E. & McCane, K.L. (2008). Understanding Pathophysiology, 4:985-986. St. Louis, MO: Mosby, Inc. • Khwab, E.K. (2009, August). After birth cleft lip and palate NAM. Retrieved January 28, 2010, from http://ekkhwab.com/2009/08/11/feeding-bottles-for-clefts/ • Rush, M. (2008, January).Children Born with Cleft Lip or Cleft Palate. Retrieved January 28, 2010, from http://hubpages.com/hub/Children-born-with-Cleft-Lip-or-Cleft-Palate • Stewart, M.G. (1991, June). Introduction to Cleft Lip and Palate. Retrieved February 1, 2010, from http://www.bcm.edu/oto/grand/6191.html • Tolarova, M.M. (2009, March). Cleft Lip and Palate. Retrieved January 23, 2010, from http://emedicine.medscape.com/article/995535-overview • Wiki (2009, September). Palatal Obturator. Retrieved January 28, 2010, from http://en.wikipedia.org/wiki/Palatal_obturator