Early Intervention Midterm

Early Intervention

term used to describe the services & supports that are available to infants & young children w/ developmental delays & disabilities and their families

oral cavity

maxilla (upper jaw)
mandible (lower jaw/chin)
upper lips
lower lips
buccals
floor of mouth
tongue
teeth
hard palate
soft palate

pharynx

responsible for swallowing food and directing airflow toward the larynx & trachea
- directs food bolus --\> esophagus

nasopharynx

the entrance to the eustachian tube & extends from soft palate

oropharynx

posterior portion of the tongue, the epiglottis, & the soft palate

hypopharynx

includes the structures of the pharynx from epiglottis & UES at the top of the esophagus

pharyngeal-esophageal juncture (PE)

mandible, thyroid cartilage, & hyoid bone

pyriform sinuses

larynx

mostly cartilaginous

VFs, false VFs, & aryepiglottic folds

hyoid bone is attached

esophagus

thin, hollow tube

combo of smooth and striated muscles

UES (cricopharyngeal sphincter)

food passes through by peristalsis

Stages of Eating

1-2: Oral Prep & Oral
3: Pharyngeal
4: Esophageal

Oral Prep phase

biting, chewing, preparing, and organizing the bolus

seeing the food, smelling the food, & opening the mouth for the food to enter

Oral phase

actively propels organized & prepared bolus posteriorly to back of mouth for swallow

Pharyngeal phase

nasal & laryngeal structures valve opening so food does not enter the airway

bolus moves through via changes in pressure & with peristaltic action of muscles

Esophageal Phase

bolus to esophagus to stomach

UES relaxes, hyoid elevates

LES relaxes & opens to allow food to enter the stomach

upon entering stomach, LES closes immediately

CN I

olfactory nerve

sense of smell

CN V

trigeminal nerve

chewing, movements of the lower jaw, palatal elevators

responsible for rooting, sucking, initiation of the swallow

CN VII

facial nerve

provides info to anterior parts of tongue

sweet, salty, sour tastes

facial expressions

fibers to salivary glands

CN IX

glossopharyngeal

provides info to back of tongue

bitter tastes

swallowing muscles & fibers to salivary glands

CN X

vagus

motor & sensory connections of pharynx, larynx, esophagus, & the heart

contributions to respiration, blood pressure & heart rate

CN XII

hypoglossal

responsible for assisting in the contraction of the tongue

bolus prep, sucking, & swallowing

0 months aka "newborn"

- lungs filled w/ fluid
- first breath sounds like a gasp
- increased blood pressure
- fluid drains/absorbed from respiratory system
- oxygen --\> bloodstream; carbon dioxide out

0 months motor patterns

- flexed position
- more flexed in prone; less flexed in supine
- movements are random: progression, symmetrical, startle, isolated, asymmetrical tonic neck reflex
- facial movements: grimaces, eyes opening & closing, smiles, sucking, tongue movements

progression movements

rhythmic alternating flexion movements of the limbs; head doesn't move

symmetrical movements

total body movements; slower than progressive

startle movements

quick, brief of the head, trunk, limbs into flexion, hands closed

asymmetrical tonic neck reflex (ATNR)

baby turns head to one side, arm & leg of other side flex

isolated movements

random & spontaneous movements of wrists & fingers; extension and abduction of toes

0 months oral-motor patterns

- very small oral cavity
- very big tongue
- face provides stability for oral mechanism
- orbicularis oris
- buccals needed for sucking and suckling
- muscles provide stability for further development of jaw & tongue movements

orbicularis oris

sphincter-like muscle of lips

protrude, round, & close lips

pharynx & upper airway

suspend from base of skull

suprahyoid muscles

assist in elevating the hyoid bone & larynx

including muscles that assist w/ mandibular depression & are part of extrinsic tongue muscles & stylohyoid

infrahyoid muscles

assist in depressing hyoid & larynx

attach hyoid to sternum, clavicles, scapulae, ribs, & thyroid

newborn hyoid bone

- directly attached to the mandible, tongue, larynx, shoulder girdle/scapulae, ribcage
- directly contributes to oral, pharyngeal, and laryngeal areas
- alignment of head, neck, shoulder girdle, rib cage
- actively participates in respiration
- maintains pharyngeal airway
- moves w/ tongue and larynx for swallowing

newborn tongue

impacts position and alignment of the larynx

newborn diaphragm

- positioned higher in thoracic cavity at rest
- more efficiency
- primary muscle of respiration
- consists of muscles & tendons, surrounded by tissue
- forms floor of thoracic cavity & separates thorax from abdominal cavities
- flattens when contracted on inspiration, abdominal wall pushed out & lower ribs pulled up (rib flaring)

newborn ribcage

- ribs & sternum primarily cartilaginous
- more flexible
- less susceptible to musculature forces and activity around them

newborn rooting response

when side of cheeks and or lip are touched, baby's head turns towards that side

- tongue cups/grooves around the nipple and moves up and down with the jaw

newborn suckling

tongue of newborn moves rhythmically with jaw in anterior-posterior direction/pattern

sound & phonation of newborn

- crying and vowel-like sounds upon exhalation
- sounds produced with general body movements
- short duration sounds like clicking noises
- newborn does not drool

newborn vision

- sensitive to light
- eye movements are random and disorganized
- visually fixates using one eye at a time
- sees moving object better than stationary
- looks at feeder while feeding

1-2 Months

- random movements are more coordinated
- head and neck elongate downward
- physiological flexion begins to diminish
- lifts head to shoulder level
- movements & gravity expand the chest
- starts grasping (reflexive and involuntary)

1-2 Months oral development

- same as newborn
- rooting response
- sucking, swallowing, breathing coordination
- more head movements, greater head control, dissociated movements of head & shoulders

1-2 months ribcage development

- similar to newborn
- better mobility b/w ribs
- physiological flexion lost by 2 months
- head lifts more actively
- prone and supine movements more asymmetrical

1-2 months phonation

- ribcage still rounded and high
- belly-breathing continues
- rib flaring evident
- cries are longer and vary in pitch
- nasal voiced sounds
- cries sound different depending on state

1-2 months vision

- fixate briefly on an object
- black & white patterns
- both eyes used simultaneously
- tracking abilities are better

3-5 months

- improved head control
- gaining postural stability
- prone is a functional position
- drooling around 3 m/o
- reciprocal leg kicking
- neck, trunk & shoulder girdle are stretched & elongated
- reach & grab objects
- bring hands to mouth

3-5 months oral development

4-5 months
- face elongates
- tongue grows in length
- mandible body enlarges
- pharynx elongates
- hyoid bone starts to descend

3-5 months sucking

- suckling more mature
- lips hold nipple more
- tongue cups nipple more
- liquid loss is typical
- neurological maturation
- rooting response diminishing by 4 months
- phasic bite on spoon

puree

- first solid food
- introduced when baby can sit upright supported (modified "ring" sit) and head control

3-5 months sounds & phonation

- babbling
- more vowel sounds with varying inflection and changes in pitch
- consonant sounds emerge

3-5 months vision

- track vertically & diagonally
- visually cross midline
- uses both eyes

6 months

- anti-gravity movements
- trunk elongation continues
- quadruped
- rolls between supine, prone, sidelaying
- sit in supported highchair
- sit independently with external support
- 1st set of teeth
- puts everything in mouth

6 months oral-motor development

- gag response diminishing
- improved sucking, greater coordination, and longer sequences of sucking-swallowing-breathing
- doesn't lose liquid while sucking
- introduced to cup
- sucking & suckling to move food
- drools often
- new food textures: mashed
- jaw moves up, down, and laterally

6 months old sounds & phonation

- vocalize during movements
- babbles longer & repetitive sounds
- vocalize pleasure sounds w/ rising intonation
- vocalize displeasure sounds like grunting

6 months old vision

visual control fully developed

eyes independent of head

7-9 months

- mouth used to explore and learn
- vision and hand-object manipulation
- ribcage descending
- may begin to crawl
- improved postural support & control
- sit and play with both hands
- transfer object between hands

7-9 months oral-motor and feeding

- sitting in highchair
- upper lip more active to remove food from spoon
- new textures: pureed and mashed
- sucking and suckling
- up/down rotary movements
- diagonal-rotary jaw pattenr
- bilateral lingual lateralization
- sucking is dominant pattern

7-9 months cup drinking

- uncoordinated suckle pattern
- learning to control liquid intra-orally
- introduce sippy cups

7-9 months old vision

- see in color and further away
- track a faster moving object/person

7-9 months old sounds & phonation

- more mature respiratory pattern
- diaphragm can expand more on exhalation
- sounds independent of movement
- longer sequences of consonant-vowel sound combinations (reduplicated babbling): labials and alveolars

10-12 months

- pulls to stand
- pincer grasp & pick up smaller items
- protect selves when they fall
- more time in upright position
- "trial and error" and "problem solving"

10-12 months oral-motor/feeding

- feeds self more
- biting more foods
- drinking liquids via cup
- swiping food from mouth is well organized & smooth
- cheeks and lips more active w/ chew
- food repertoire expanding: chopped, diced

10-12 months sounds

- variegated babbling
- resonation of mouth changes
- more vowel sounds
- babbling --\> "First words"

crawling

- sets stage for supporting structures of spine
- cross-crawling strengthens communication between both hemispheres of the brain
- enhances visual system (proprioception)
- balance & coordination fine tuned

nutrition

- greatest environmental influence
- iron, iodine, & DHA
- early shortages: reduce cell production
- later shortages: affects cell size & complexity

cleft palate/lip

- separation of parts of the mouth that are typically fused together in fetal development
- lips form between 4-7 weeks
- palate forms between 5-9 weeks
- laryngeal, esophageal, benign/aesthetic dimpling

cleft lip

- separation of one or both sides of the upper lip & alveolar ridge & upper gum
- can be incomplete
- can be complete (separation of alveolar ridge, lip, and extends up through nasal cavity)
- one side: unilateral
- both sides: bilateral
- submucous: surface of palate has a tissue covering but underneath there's a cleft
- more present in males
- unilateral more common
- genetic & environmental factors

cleft lip/palate feeding

variable & dependent on: baby itself, location & severity of cleft, ability to transition to alternate types of feeding

caregiver's ability to accept baby's "difference" & learn & apply new techniques

cleft lip/palate breast-feeding

hold baby with cleft-lip side against the breast

often does not work

cleft lip/palate bottle feeding

- same w/ small cleft may be able to suck from a traditional nipple w/ adjusting position & lip support
- most will need a specialty feeder: Haberman, Mead Johnson, Dr. Brown's Medical Zero-Resistance

The Haberman

expels milk into mouth from the nipple reservoir

- one-way valve presents backflow
- adjusts flow rate
- when baby stops, flow stops

Mead-Johnson

- longer nipple than standard
- cross-cut nipple
- caregiver can squeeze bottle
- disposable

Dr. Brown's Medical Zero-Resistance

- feeding valve is inserted
- more "infant-driven"
- readily available

cleft palate/lip treatment

nasoalveolar molding device (NAM)

- palatal
- individualized
- "plastic" retainer
- reduces cleft 2-3 mms every 1-2 weeks
- wear for approx. 4-5 months
- depends on location, severity, etc.

cleft palate/lip surgery

- timing is variable, depends on location, severity of cleft
- cleft lip repaired first (3-4 months old)
- severe clefts --\> \>1 surgery
- initial repair of cleft palate at 9-12 months old

Down Syndrome (Trisomy 21)

- congenital
- extra copy of chromosome 21

Down Syndrome physical characteristics

- flattened face
- almond eyes shaped up
- short neck
- small ears
- tongue protrusion
- small hands & feet
- poor muscle tone
- shorter height

Down Syndrome treatments

- speech therapy, physical therapy, occupational therapy
- medical treatments of heart defects
- prescription glasses
- hearing assistive devices

Pierre-Robin Syndrome

- congenital
- abnormally small lower chin (micrognathia) w/ receding chin
- displaced tongue (glossoptosis)
- cleft soft palate
- high arched palate
- lifelong

Pierre-Robin Syndrome diagnosis

- physical appearance at birth
- exact cause is unknown
- multi-system syndrome
- arrested development of lower jaw
- maternal viruses & folic acid deficiencies
- genetic counseling recommended

Pierre-Robin Syndrome characteristics & treatments

- feeding and respiratory difficulties
- intubation
- tongue-lip adhesion
- nasal prong
- tracheostomy
- jaw may catch-up in growth during first 2-4 years
- surgical repair of cleft palate

mandibular (jaw) distraction surgery

- done at about 6 weeks old
- over two weeks, the mandible is gradually lengthened and moved forward using two internal devices
- after 3 months, distractor is removed
- patients breathe more easily during distraction period

DiGeorge Syndrome (22q11.2 Deletion Syndrome)

- disorder caused when a small part of chromosome 22 is missing
- deletion --\> poor development of several body systems
- common medical problems: heart defects, poor immune system function, cleft palate, low levels of calcium in blood, delayed development with behavioral & emotional problems

DiGeorge Syndrome symptoms

- vary in type & severity
- heart murmur and blueish skin
- frequent infections
- facial features: underdeveloped chin, low-set ears, wide-set eyes, narrow groove in upper lip, cleft palate, delayed growth
- difficulty feeding
- breathing problems
- poor muscle tone
- learning disabilities
- behavioral problems
- delayed speech development

DiGeorge Syndrome Treatments

- ST, PT, OT
- procedures: heart procedures, cleft palate repair, genetics consultations
- ENT
- Infectious Disease Specialist
- Endocrinologist
- Immunologist

ATR-X Syndrome (Alpha Thalassemia X-linked Intellectual Disability Syndrome)

- serious delays in speech & walking
- severe hypotonia
- unique facial features: widely-spaced eyes, small nose w/ upturned nostrils, low-set ears, upper lip shaped like an upside down "V", lower lip sticks out)
- abnormal genitals
- small head size
- shorter height
- blood disorder: alpha thalassemia
- heart defects
- affects males primarily
- ranges from moderate to severe
- very rare

Menke's Syndrome

Males
Early birth
Not meeting milestones (2 months benchmark)
Kinky hair
Evidence of positive family history
Seizures & low muscle tone that cannot be explained

negatively affects: cell protection, cell function, formation of connective tissue, formation of skin & hair color, production of copper

copper

important functions:
- production of red blood cells
- regulation of heart rate and blood pressure
- absorption of iron
- prevention of prostatis
- development and maintenance of bone, connective tissue, & organs
- activation of immune system

videofluoroscopic swallow study (VFSS)

- aka modified barium swallow study
- provides a direct, dynamic view of oral, pharyngeal, and upper esophageal function
- helps determine if aspiration occurred (presence, timing, & amount)

indicated when:
- need to observe swallowing stages
- diagnosed/suspected presence of abnormalities in the anatomy of nasal, oral, pharyngeal, or upper esophageal structures
- aversion to endoscope

FEES

- assessment of the anatomy
- direct observation of swallowing function & control of the movement of secretions, liquids & food
- pooling in puneiform sinuses and/or valleculae

FEES pros

- can be repeated frequently
- no need for contrast material
- portable

FEES cons

- requires more cooperation from child
- tube placement into nares and nasopharynx is invasive

Cervical Ausculation (CA)

- stethoscope amplifies sounds during swallowing & breathing
- placed over/near thyroid cartilage to hear "click" of larynx elevating
- suck-swallow-breathe coordination can be heard during feeding
- portable

CA disadvantages

- SLP must be highly trained to hear swallow
- infant & toddler swallows are different than adults
- cannot tell if there is silent aspiration

penetration

when food/liquid goes into trachea and stays above VFs

aspiration

- when food/liquid goes into trachea and goes below VFs

- typically an individual coughs, sputters, has a wet vocal quality unless silent aspiration occurs

silent aspiration

food/liquid goes into trachea below VFs but the person does not cough/sputter

only detected on MBSS

aspiration pneumonia

- type of lung infection due to relatively large amount of material from stomach/mouth entering the lungs
- signs/symptoms: fever & cough, lung abscess, acute respiratory distress syndrome, effusion
- risk factors: decreased levels of consciousness, problems with swallowing, tube feeding, poor oral health
- diagnosis: presenting history, chest x-ray, sputum

hospital based: in-patient/out-patient

- only with dr. orders
- speech/swallowing "rehab" dept

outpatient

- will vary widely
- could be a NICU graduate who needs clinical work before EI
- baby/child who did not qualify for EI but needs services
- baby/child referred by physician

What do you do in a hospital-based setting?

- Why are they being referred?
- Read dr. orders for diagnosis/presenting problems
- R/O (rule out) or state "swallow eval"
- Review chart
- Is the baby connected to anything?
- oral-facial assessment non-nutritive NNS first
- rooting response?
- symmetrical features?
- feed baby in appropriate position
- visual & auditory observations
- MBSS indicated?
- schedule another B/S eval
- recs for NICU tea