1. Aynsley Brian, Brittany Garay, Caroline Johnson, Melissa Gutierrez, and Sarah Williams

2. 8 year old, 1 st grade student in regular classroom DiGeorge syndrome and Pierre Robin Sequalae Tracheomalacia Tracheostomy tube secondary to tracheomalacia 2 decannulations unsuccessful due to cyanotic Complete bilateral cleft – repaired at 12 months Sucking and feeding problems Fed via Mickey tube until age 6 Expressive language: apraxia, severe nasality and used to use AAC device

4. MA Sat up at 6 months Walked at 12 months Toilet trained at 5 years with frequent accidents Those with PR/DiGeorge … Mild delay in infancy, usually unnoticed until later years Developmental delays, including delayed growth and speech development Learning disabilities Increased risk of developing mental illnesses such as schizophrenia, depression, anxiety, and bipolar disorder More likely to have ADHD and developmental conditions such as autism spectrum disorders that affect communication and social interaction

5. Normal Development Birth-6 mos Cooing, gooing Cries for different needs Reduplicative babble includes /p,b,m/ Chuckle, laughter, gurgling 7mos-1yr Variegated babble Uses words/sounds to gain attn. 1-2 words around 1 st Birthday 1-2 years Vocabulary increases Uses many different initial consonant sounds Combines 2 words 2-3 years Up to three word utterances Asks “why?” questions 3-4 years Combines multiple sentences Describes past events Answers simple “Wh” Questions Pronouns (I, you, me, we etc.) Plural marker ‘s’ 4-5 years Acquired most all speech sounds Knows letters/numbers Tells short stories and uses action words

6. MA (Pierre Robin) Expressive language disorder Apraxia of Speech Severe hypernasality Single word utterances Highly unintelligible Past use of AAC device to communicate/augment his speech. Current level of function: Use of full sentences Articulation skills not age appropriate

7. Children with Pierre Robin are prone to build up of fluid behind the ear-drum (secondary to cleft palate) PE Tubes may be necessary Hearing Tests are important to monitor for any hearing loss that may occur which may affect the child’s speech and language development

8. Effects the oral motor mechanics and the ability to generate negative intraoral pressure May cause difficulty with suck, swallow, breathe coordination. Inadequate airway protection during swallowing Negative effect on the parent-infant bonding process

9. Inability to generate negative pressure for suction Unable to find a hard palatal surface for compression of the nipple If placement not achieved, nipple may be pushed into the area of the cleft Nasal regurgitation Allows for air to continue to flow in through the nose and mouth during feeding resulting in excessive intake of air causing bloating and/or spitting up

10. Cause excessive expenditure of energy Results in inadequate weight gain and nutrition Causes stress for the infant and caregiver affecting the bonding experience

11. Micrognathia and retracted tongue position: Affects ability to compress the nipple Can disrupt coordination of the suck-swallow-breathe sequence Glossoptosis: Can cause chronic airway obstruction U-shaped cleft: Difficulty generating negative pressure

12. Side-lying positioning help position the tongue anteriorly and facilitate tongue movements with use of a modified bottle Standard, semi-reclined feeding position helps minimize the gravitational pull on the tongue

13. Breastfeeding: Challenging because infant is unable to generate negative pressure for suction and retracted position of tongue causing inadequate compression of the milk ducts. Good options: Utilizing a reservoir Utilizing a breast pump then feeding the infant

14. Modified Nipples Pliability- Must be pliable enough to release breast milk or formula, with limited compression and suction. Must be firm enough to provide appropriate proprioceptive input to stimulate sucking Good options: Nipples designed for premature infants Specialized Pigeon nipple Standard nipple softened through boiling

15. Modified Nipples (cont’d) Shape- Must facilitate adequate contact between the nipple and the tongue for compression Should enhance the oral-motor patterns desired during sucking Good option: An orthodontic nipple that has a broad, flat bulb-type end that flares to a large, wide base

16. Modified Nipples (cont’d) Length- Should be based on what is needed to provide adequate contact between the nipple and tongue Varies with regard to type of base and distance from the tip to the base, especially for those nipples that have tapered bases. Other factors to consider: strength of the infant’s suck, degree of lip closure around the nipple, and the control the feeder provides to maintain nipple position

17. Modified Nipples (cont’d) Hole type- May be standard or crosscut Good option: Crosscut, because it allows milk to flow only when the infant compresses the nipple Hole size- Large enough so that when the bottle is held upside down, the liquid drips out but does not run out rapidly Small enough as to not cause the infant to have difficulty with coordination of swallowing and breathing

18. Flexible bottles and assisted fluid delivery Pressure applied to squeeze bottle, plastic liner, or nipple reservoir must be in rhythm with the infant’s suck and swallow Inappropriate rate or continuous squeeze may result in increased swallow rate and decreased breathing time Good option: Mead Johnson Cleft Palate Nurser

19. Feeding and mandibular distraction Fixed position of the mandible after mandibular distraction causes infants to be unable to feed. NG tube used during this period Following the distraction, oral feedings resumed and improved Feeding after cleft lip and palate repair Postop feeding recommendations controversial and variable: Immediate unrestricted feeding a restricted approach to facilitate good healing recommend use of a spoon and discourage sucking recommend supplemental tube feedings for a period of 7-12 days

20. Positioning the infant Semi-upright position (at least 60 degrees) facilitates control of jaw, cheek, lip, and tongue movements Also allows gravity to assist with swallowing helps prevent nasal regurgitation Head supported in neutral A-P alignment with shoulders symmetric and forward, trunk in midline, hips flexed

21. Pacing intake Provide fluid in rhythm with infants suck and swallow Oral facilitation strategies Jaw and cheek support may be recommended Preventing excessive air intake Increase the frequency of burping (after every ounce)

22. Managing nasal regurgitation When it occurs, stop feeding to allow the infant time to clear the nasal passage Ensure the infant is in an upright position Consider a slower flow nipple Slow the presentation of fluid

23. Consistency of method Feed in the same position, with the same nipple and bottle and with the same technique during each feeding Use of feeding obturators Retained in the crevices of the cleft provides partial seal between the mouth and nasal cavity Differing views about them

24. Oral hygiene Cleanse the cleft and surrounding areas following feedings washcloth, gauze, or toothette and water or water+hydrogen peroxide Transitioning to a cup Most infants ready to transition by 8-9months of age Initially beneficial to use slightly thickened liquid flow Select a cup that does not promote continued sucking Best option: Small open cup without a spout, straw, or valve

25. Introduction of solid foods Introduced to the baby with an unrepaired cleft palate at the same time as with any infant Use appropriate positioning, small boluses, slow pace, and alternate food with liquid Transition to more textured foods introduced in the same sequence as for other children