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OBJECTIVES

“The most common presentation of a


Developmental Red Flags developmental disability
is failure to achieve age-appropriate
developmental milestones.”

How common are developmental problems?


Beth Ellen Davis, MD, MPH
Developmental Pediatrics How does one recognize developmental delay?
Director, UW LEND What are some developmental “red flags” not to miss
May 23, 2015 during a dental office visit?
Can one “anticipate” behavioral problems ?
How can one adapt the office visit for children with DD?

CSHCN: those who have or at risk for a chronic


physical, developmental, behavioral, or emotional condition
Disclosure and who also require health and related services of a type or amount
beyond that required by children generally
• I have nothing to disclose.

http://www.neserve.org/neserve/pdf/NES%20Publications/Shared%20Responsibilities
%20Toolkit/Screener_%20FACCT.pdf

Beth Ellen Davis MD MPH 1


Prevalence of DD
CSHCN Dx per 1000 age (mo) MD 1st
“GDD” 100 24 30%
Source: 2011-12 NSCH Source: 2009-10 NS-CSHCN ID (mild ID) 25 39 60%
ID (mod/severe ID) 5 12 90%
Learning disability 150 69 12%
ADHD 100 59 45%
CP 3 12-14 99%
ASD 8-12 40* 30%
Visual impairment .5 55 60%
Hearing Impairment 10-30 39* 40%
childhealthdata.org 2012 *changing

78.4% of CSHCN report


An infant brain
one of the following:
Health Issue % Health Issue % • The human brain at
Learning Disability 27 Speech problems 16 30 weeks weighs ½
ADHD 32 Tourette Syndrome .2
of term infant!
Depression 8.5 Asthma 30
Anxiety 13 Diabetes 1.4
Behavioral problems 14 Epilepsy 3 • 3rd trimester
Developmental Delay 15 Hearing impairment 4
40,000 new
Intellectual Disability 5 Vision Impairment 3
synapses/min
Autism Spectrum Disorder 8 Bone, joint muscle issues 8
Cerebral Palsy 1 Brain Injury 1

• At birth, 1 million
synapses/second
childhealthdata .org 2012

Beth Ellen Davis MD MPH 2


Babies are surviving the NICU Nature vs Nurture
(NICHD NRN 2003-2007)

Extremely preterm survival


100
90
80
70
60
50 Early experiences activate synapses,
40
30
strengthen existing pathways, create new
20 pathways.
10
0 Lack of experience increases synaptic pruning
22 23 24 25 26 27 28 Overall
and apoptosis (cell death)
Stoll BJ, Pediatrics 2010

Not all risk is biologic! Streams of Development


• Gross motor
• Fine motor
• Language
– Expressive
– Receptive
• Problem solving
• Social

Beth Ellen Davis MD MPH 3


DEFINITIONS Patterns in Development
MOTOR PS RL/EL SOCIAL
• Surveillance vs. Screening
ID V D D D
• Delay vs. Deviance
CP D V-N V-N D
VI D D N D
HI N N D V-N
LOOK FOR PATTERNS!
ASD N V-N D D

Developmental Quotient Ages for early diagnosis

DQ = Developmental Age/ Chronologic age X 100 0-12 mo. 1-2 yr. 2-3 yr. 3-4 yr.

ID, ID, mod ID, mild ID, mild


DQ <70 Delay mod/sev.
DQ 70-85 Monitor VI/HI HI HI
DQ >85 Typical range CP CP CP mild
*Perform for each stream of development Autism Autism/LD LD

Beth Ellen Davis MD MPH 4


RED FLAG Muscular Dystrophy
• 3/10,000 boys
Any time there is a history or exam • Absent dystrophin gene,
consistent with Xp21.2
• 30% no family history
LOSS of SKILLS (regression)
• CPK is > 20 x normal, even
at birth
THINK……metabolic, genetics w/u, • DNA blood test can make
neuroimaging, seizures, hydrocephalus, diagnosis in majority of
cases.
toxin exposure, autism

RED FLAG MOTOR DELAY: RED FLAG

• Any boy not walking


by 15 months should
get a CPK to r/o MD

• The most common


cause of delayed GM
is global delay.

Beth Ellen Davis MD MPH 5


Motor Delay + Identify early!
• 15% of two year olds do not have 50 single
words and/or two word combinations.
• Hypotonia vs. weakness • 5-10% of all children have developmental
language disorder ( at age 3-4 years)
• Delayed language may be a marker of other
• www.childmuscleweakness.org
developmental disorders
• Fasiculations
• Language is the BEST predictor of later cognitive
• High arched palate WEAK toddlers almost always
have hypotonia function
• Drooling but usually HYPOTONIA exists • Early intervention yields best outcomes
without weakness

Language Delay: Definitions Normal Language Development


• Language- a system of verbal, written, or • Expressive: two phases
gestured symbols used to communicate
information or feelings. Social smile 5 wk 1st word 11 mo
– Components: phonology, morphology, syntax, Coos 6-8 wk Immature jargon 12 mo
semantics, pragmatics Laughs 3-4 mo 4-6 words 15 mo
Raspberry 4-5 mo 2 words 21 mo
• Speech- the physical production of spoken Squeals 5 mo Pronouns indiscrim 2 y
language. Babbles 6 mo Tells stories 4 years,
– Components: articulation, phonology, voice quality, UNTIL 6-8 mo! 100% intelligible
pitch, loudness, resonance, fluency, rate, rhythm

Beth Ellen Davis MD MPH 6


Normal Language Development RED FLAG
• Receptive
• All children with language delay should be
Infant
Toddler
referred for hearing assessment.
Alerts to voice 1 mo
Regards speaker 3 mo
1 step command, • Infants who are deaf may have normal pre-
without gesture 14 mo
Listen then vocalizes 5 mo linguistic expressive language until 6-9
1 body part 15 mo
Enjoys gesture games 9 mo
Fetches on command 16 mo
Understands “no” 9 mo
Points to picture 18 mo
months of age.
Orients to name 8-10 mo
6 body parts 20 mo • 6-15% of kids who have PHL missed
Command,
2 step command 24 mo
with gesture 12mo
identification at newborn screening.

Language delay Language Delay: HI

https://www.youtube.com/watch?v=TD5E88fFnxE&feature=pla
• Developmental Language Disorder 5-10% yer_detailpage

• ID 3%

• Hearing Impairment .5-1%

• Autism .6-1% People with HI can often “HEAR” something but


cannot understand or comprehend what is
being said

Beth Ellen Davis MD MPH 7


Social Delay: Typical
Language Delay: RED FLAG
joint attention
A BIFID UVULA is
evidence of a
submucous cleft in
the palate and
warrants evaluation
if associated with
recurrent OM,
speech delay, or VPI.
CONSIDER VCFS
(del 22q.11)

Social Delay: Atypical


Language Delay: RED FLAG
joint attention

• Normal Pattern is for RL>EL.


• Expressive language that significantly exceeds
receptive language is deviant.
THINK :
1. AUTISM, with echolalia
2. Syndromes with “cocktail personalities”
3. Parent mis-interpretation

Beth Ellen Davis MD MPH 8


Joint Attention Skills* Problem solving
TYPICAL ASD

8-10 months Gaze monitoring No eye contact


• Problem solving milestones are evidence of
10-12 months Following a point Does not respond to
request “oh look!”
cognitive abilities, or intelligence, without
PIP Develops advanced
the use of language.
12-14 months
self help skills
…Patterns…..
14-16 months PDP * Consistently absent
• Typical PS=RL>EL..COMMON, often resolves
14-18 months Show and tell Often brings to
parent to obtain
• Typical PS>RL>EL…less common, often LD
repeated action
• Low PS, Low RL, Low EL = ID
*Joint attention deficits appear to be specific to ASD
and reliably differentiate children with ASD from
other developmental disabilities.

Social Delay: RED FLAGS Typical development


• Blocks
– Regards 3 mo
• Lack of response to name – Attains 5-6 mo
• Lack of eye gaze and monitoring – Takes 2nd 6-8 mo
• Lack of gestures (waving, pointing, head – Releases into cup 12 mo
nodding) – Takes a 3rd 12-14 mo
– Builds a tower of 2 13-15 months
• Lack of requesting items or attention
– Builds a tower of 4 18 months
• Lack of bringing and showing – Builds a tower of 6 24 months
– Train 26-30 months
https://www.m-chat.org/mchat.php
Free, online MCHAT-R screener with scoring

Beth Ellen Davis MD MPH 9


Intellectual Disabilities
RED FLAG ( outdated term: Mental retardation)
• Early Handedness • 2-3% of population
• Male 1.6 : Female 1
Children with handedness • 85% of ID is MILD category
before age 15 months usually
have an abnormally weak
upper extremity on the other Most common genetic cause: Down Syndrome
side. Most common inherited cause: Fragile X
Most common preventable cause:Fetal alcohol

“Global developmental delay” Levels of ID


• A significant delay in 2 or more streams • Mild (Intermittent Support) IQ~ 55-69
– Vast majority 85%
• NOT a diagnosis – More common in boys

• NOT regression or loss of skills • Moderate (Limited Support) IQ ~ 40-54


• Severe (Extensive Support) IQ~ 25-39
• Can be used for services in health care setting – Rare .5%
(ICD 9 315.9, ICD-10 F88) early intervention and – Ratio of boys to girls is equal
– Think about Rett Syndrome in girls
for Public Schools. Can’t be used for services
• Profound (Pervasive Support) IQ < 24
after age 6-9 years.

Beth Ellen Davis MD MPH 10


DSM-5
ID: The Search
Big improvement for ID
EX: DSM-5 (Social Domain)- severity descriptors The more severe the ID,
Immature in social interactions
Communication and language are more concrete
the more likely to find etiology.
May have difficulties regulating emotion, behavior
Difficulties are noticed and generally accommodated for by peers in social situations
At risk of being manipulated by others (gullible)
Moderate
• CMA (40% + in SEVERE, 10% for all )
Marked differences from peers in social, communicative behavior across development
• DNA for Fragile X (2-6%)
Spoken language typically a primary tool for social communication, but is less complex than peers
Social judgment and decision-making abilities are limited, caretakers must assist with life decisions
• ± Neuro-imaging (MRI study of choice)
Social and communicative support is needed in work settings
– IQ <50, micro/macrocephaly, abnormal neuro exam,
Severe
seizures, loss of milestones
Generally use nonverbal communication in social interactions; language, if used, involves names and
simple phrases • ± Metabolic Studies (if regression, family history)
May respond to/ understand simple social cues but in general lack understanding of social context
Relationships involve family, caretakers and other long term ties and are more typical of attachment
relations rather than of reciprocal friendships

ID: Adult outcomes


Intellectual Disability: Known causes
 Prenatal (60-75%)  Postnatal (1-10%)
– CNS malformation
– CNS infection
– Chromosomal abnormality
– Stroke/Hemorrhage
– Syndrome
– Genetic – Trauma/Abuse
– Toxins (FASD) – Hypoxia
– Infection – Degenerative
– Neurocutaneous syndrome – Epileptic encephalopathy
– Malnutrition – Metabolic
 Perinatal (10%) – Complications of
prematurity
– Hypoxia
– Neonatal seizures

Beth Ellen Davis MD MPH 11


Predictive value of a good exam:
Red Flag
Anticipating behavioral problems
The presence of three or more minor • Due to language delays
– Lack of understanding
anomalies is highly predictive of a major – Lack of ability to say “stop” or “wait a minute”
malformation (19.6%) • Due to social delays/deficits
– Inability to understand white coat, masks, strange equipment
Examples: bossing, absent hair whorl, anteverted nostrils, epicanthal folds,
preauricular tags, pits, abnormal pinna of ears, bifid uvula, extra nipples, – Stranger
single umbilical artery, umbilical hernia, dimple over sacrum, single – Routine disrupted
palmar creases, syndactyly, overlapping toes, recessed toes….. – Invasion of space, especially around face
• Due to sensory issues (hyper/hypo)
– Lights, reclining chairs
– Noise (drills, suction)
– Tastes and smells, oral aversion
– Gloved touch, paper gowns

Adapting the office setting for


Exam: Head Circumference children with DDs
 Rule of 3’s and 9’s • 4 year, old Josh, is healthy and experiences
– Birth: 35 cm ASD. He is able to speak in 3 word sentences.
– 3 mo: 40 cm
He ‘head bangs’ when frustrated, and has
sensory seeking behaviors according to his
– 9 mo: 45 cm
school OT. He calms with video games. His
– 3 yrs: 50 cm mother is calling to ask how best to prepare
– 9 yrs: 55 cm him for his first dental visit. . .

Beth Ellen Davis MD MPH 12


Preparing the Parent Communication
• Call to discuss visit early
• Pre-visit tour and/or picture story • Visual aids
• Use social stories/ story books – Help children express the need to take a break
• Practice knee-knee, “open” during visit
• Bring comforter, distracter, spoon – can be a single card or a board with several visual
• Does child have light and noise symbols
sensitivities?
– Sunglasses for bright lights – can reduce anxiety and negative behavior.
– Earphones with +/- music for sounds
– DVD options
• Benefits from weighted blanket?
• Minimize triggers: timing, fatigue, hunger
Stop !!

www.cshcn.org

Preparing for Success Oral aversion


• Schedule visit when the office is the quietest
• Minimize waiting time (allow car wait with cell phone) • Can be due to hyper or hypo sensitivity
• Let the parent be the child’s advocate
– Ask parent for stressors and motivators • Pre-visit home practice “open” and experience
– Let parent end the visit if its too much for child with appliance in mouth
• Sensory integration approach • If child is
– Weighted x-ray blanket, dimmed lights, fewer visual stimuli
– < 3 years, ask if Early Intervention is involved.
• Suggest dental team adaptations
– Upright dental chair If children become anxious when reclining – > 3, ask if Individual Education Plan includes oral
– Eliminate or reduce noises, smells, and sensations that trigger problem hygiene goals.
behaviors • Ask about Occupational Therapy support for
– Avoid problem textures – paper gowns, etc.
• Anticipate and avoid escape
sensory and adaptive intervention
• Book a double time slot • If setting is ASD, ask about ABA goal for oral
sensitivities.
http://www.autismspeaks.org/community/family_services/dental.php

Beth Ellen Davis MD MPH 13


“FTT” Resources
• Developmental Milestones 1: Motor, Gerber R Jason et al, Peds in Review
2010. Vol 31( 7) : 267. – a review article
• Developmental Milestones 2: Cognitive, Wilks T et al, Peds in Review
• Non oral feeders or liquid only oral feeders • 2010. Vol 31 (9) : 364. – a review article
often “graze” on liquids all day. • Developmental Milestones 3: Social-Emotional, Gerber R Jason et al, Peds in
Review 2011. Vol 32 (12): 533. – a review article
• Speech and Language Delay in Children, McLaughlin MR, Amer Fam Phys,
2011. Vol 83 (10)1183. – a review article
• Interdiscplinary team needed (MD, DMD/DDS, • Managing Feeding Problems and Feeding Disorders, James Phalen, Peds in
Review 2013. Vol 34 (12) 549.
SLP, OT, Nutrition, Behavioral support) • Oral Health Care for Children with Developmental Disabilities. Norwood KW,
Slayton RL. Pediatrics. 2013. 131(3):614-619.
• Grazing is a “deviant” oral feeding pattern. http://pediatrics.aappublications.org/content/131/3/614.full.pdf+html
• ‘Oral Health for Children and Adolescents with Special Health Care Needs –
• Goal is to have “batch” feeding, rinse, allow to Challenges and Opportunities (2nd Ed) [from National Maternal Child Oral
Health Resource Center] www.mchoralhealth.org
get hungry, and feed again for the same • To provide parents:
– Dental Toolkit on AutismSpeaks, www.autismspeaks.com
intake. – Challenging Behaviors Toolkit on AutismSpeaks, www.autismspeaks.com - these
materials are evidenced based interventions for all CSHCN!
– GREAT Handouts on www.cshcn.org, search “dental”

Special thoughts about


Children with DDs
• Carries and/or infections
– may present only as a change in behavior
– may go unnoticed because of child’s high pain threshold.
• Food over-selectivity usually more behavioral or
sensory than an indication of dental pathology
• GER may cause increased tooth erosion.
• Bruxism is common concern
• Food Pouching prevention
“Find the ability in disability”
The Center for Children
with Special Needs
www.cshcn.org

Beth Ellen Davis MD MPH 14

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