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

MAAynsley Brian, Brittany Garay, Caroline Johnson, Melissa Gutierrez, and Sarah Williams

History • 8 year old, 1st 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

Normal Developmental Milestones

Developmental Milestones (Pierre Robin with DiGeorge Syndrome)

• 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

Speech and Language Milestones

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 1st 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

Speech and Language Milestones (cont.)

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

-Hearing-

Children with Pierre Robin are prone to build up of fluid behind the ear-drum (secondary to cleft palate)

PE Tubes may be necessaryHearing Tests are important to monitor for any

hearing loss that may occur which may affect the child’s speech and language development

How an open palate affects ability to feed:• 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

Specific difficulties created by a bilateral, complete cleft of the secondary palate:

• 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

Specific difficulties created by a bilateral, complete cleft of the secondary palate: Cont’d

• Cause excessive expenditure of energy • Results in inadequate weight gain and nutrition

• Causes stress for the infant and caregiver• affecting the bonding experience

Specific feeding difficulties created by Pierre Robin Sequelae:

• 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

Feeding modifications made to help the infant with Pierre Robin sequence to feed:

• 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

Feeding methods, modifications, and facilitation techniques specific to a complete bilateral cleft of the secondary palate:

• 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

Feeding methods, modifications, and facilitation techniques specific to a complete bilateral cleft of the secondary palate: Cont’d

• 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


• 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


• 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


• 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


• 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

Specific feeding considerations and techniques• 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


• 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


• 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)


• 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


• 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


• 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


• 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

Documents

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