Behavioral Insights
and Practical Strategies for
Working with Children who have
Smith-Magenis Syndrome
Brenda Finucane, MS, CGC
Executive Director, Genetic Services
www.elwyngenetics.org
Smith-Magenis Syndrome
Smith-Magenis Syndrome
“p” arm
17
“q” arm
Smith-Magenis Syndrome
ONYCHOTILLOMANIA
Nail yanking
POLYEMBOLOKOILAMANIA
Orifice stuffing
Smith-Magenis Syndrome
SELF-INJURIOUS BEHAVIORS
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


Hand biting
Head banging
Picking at finger / toenails
Skin picking
Inserting objects into
nose, ears, etc.
Smith-Magenis Syndrome
Smith-Magenis Syndrome
SLEEP DISTURBANCE
Frequent awakenings
at night
 Early wake-up
 “Sleep attacks” during
the day
 Inversion of melatonin
cycle

Smith-Magenis Syndrome
CHALLENGING BEHAVIORS



Attention-seeking: Crave
one-to-one interactions
with adults
Often in competition with
peers or siblings for staff
or parent attention
Perseveration - repeatedly
asking the same question
Smith-Magenis Syndrome
CHALLENGING BEHAVIORS





Poor impulse control
Aggressive hugging of
others
Prolonged tantrums,
outbursts
Difficulty adjusting to
changes in routine
Poor sense of time - can’t
be rushed!
Smith-Magenis Syndrome
POSITIVE ASPECTS


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Engaging, endearing, and
full of personality!
Appreciative of attention
Eager to please
Sense of humor
Communicative
Smith-Magenis Syndrome
POSITIVE ASPECTS




Responsive to structure
and routine
Motivated by a variety of
reinforcers, activities
Causes of aggression,
outbursts often identifiable
Tantrums, aggression can
often be redirected if
caught early
Spasmodic Upper
Squeezing Tic Thing,
With Facial Grimacing
(self-hugging)
AUTO AMPLEXATION
Smith-Magenis Syndrome
Parents and Researchers Interested in
Smith-Magenis Syndrome
(PRISMS)
www.prisms.org
CLASSROOM SIZE AND SETTING
Small class size
Calm structured classroom
Importance of staff, classroom
atmosphere, structure, and
curriculum
Many planned (not spontaneous)
activities as in preschool /
primary grades
CLASSROOM SIZE AND SETTING
Natural breaks in schedule
Class composition vs. staff
match
Good communication among
staff
Staff training / consistency of
approach
STUDENT / STAFF MATCH
Staff need to:
• be emotionally neutral
• avoid power struggles
• be comfortable with close proximity
• be versatile but not volatile
• be creative
• think on their feet
• have a good sense of humor!
CLASSROOM STRATEGIES WHICH
ARE OFTEN SUCCESSFUL
Individualized schedule
Individualized behavior chart
Visual reminders
Planned breaks
Opportunities to request breaks
Variety of positive reinforcers
Preferential seating
Visual schedules
Visual timers
www.timetimer.org
www.timetracker.org
CLASSROOM STRATEGIES WHICH
ARE OFTEN SUCCESSFUL
Redirection and distraction
Sensory input
Transition warnings
Handshakes, not hugs
Humor
CLASSROOM STRATEGIES WHICH
ARE OFTEN SUCCESSFUL
Whole language / sight word
approach to reading
High interest materials
Adult-like activities
Audio-visual materials
Alternatives to fine motor tasks
“Prevention versus intervention”
behavioral approach
CLASSROOM STRATEGIES WHICH
ARE OFTEN UNSUCCESSFUL
• Time-out in classroom when student is already
engaged in a behavioral outburst
• Teacher or aide getting visibly upset or raising voice
• Ignoring the student rather than distracting,
redirecting, or engaging
• Counseling, coaxing, touching the student during an
outburst
• Physical restraint during an outburst, except when
necessary to avoid injury to self or others
BEHAVIOR SUPPORT
•
Prevention versus intervention!
•
Behavior support strategies need to focus
on the antecedents
•
Know the person, recognize early signals
•
Need for alternative or replacement
behaviors
•
Respect for the power of genetically-driven
behaviors
Genetically-driven Behavior

Has roots in physiological impulses, drives

Requires great effort on the part of the
person to suppress, control the impulse

Environment is often key in motivating the
person to work toward suppressing,
replacing the impulse

For some genetic syndromes, what starts
out as an involuntary, genetically-driven
impulse becomes a learned, manipulated
behavior through the response it generates
in the environment
THE ABCs OF BEHAVIOR
ANTECEDENTS
BEHAVIOR
CONSEQUENCE
What happened before?
What happened?
What happened afterwards?
Teacher asked
student
with SMS to
complete a
handwritten
worksheet
Child with SMS
refused
OR destroyed
materials
OR smacked self
on face
Removed from
room, hours of
tantrumming,
injury to self and
others, attention
from peers and
adults.
THE ABCs OF BEHAVIOR
ANTECEDENTS
BEHAVIOR
CONSEQUENCE
What happened before?
What happened?
What happened afterwards?
Traditional Emphasis
Emphasis Needed
for Genetically-Driven Behaviors
BEHAVIOR CHANGE
isn’t just about the
person with the
syndrome!
COMMON TRIGGERS
• Fine motor tasks
• Waiting
• Rushing
• Transitions
• Seeing people out of context
• Lack of clear expectations
• Competition for attention
• Highly-charged emotional atmosphere
SMITH-MAGENIS
SYNDROME
relatively high
cognitive and
social abilities
versus
very young
emotional
development
“EMOTIONAL TODDLER” IN SMS
•
Emotionally volatile
•
Low frustration tolerance
•
Prone to tantrums / outbursts
•
Attention-seeking
•
Distractible
•
Excitable
•
Reactive
•
Multisensory learners
“EMOTIONAL TODDLER” IN SMS
•
Inconsistent (“Yes / no” game)
•
Upset by seeing people out of context
•
Live in the moment
•
Possessive attachments to caregivers
•
Difficulty awaiting turn (me first!)
•
Adult vs. peer-oriented
•
Relentless question-asking
•
Need ongoing reassurance
DEVELOPMENTAL ASYNCHRONY
•
•
•
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disparity between intellectual and
socio-emotional development
described in highly gifted children;
not well-researched in people with
intellectual disabilities
parallel phenomenon observed in
people with Smith-Magenis syndrome
significant contributor to maladaptive
behaviors in SMS
Developmental Asynchrony Study
Research goal:
Develop a test battery to detect and measure developmental asynchrony
Measures:
Kaufman Adolescent & Adult Intelligence Test (KAIT):
• Crystallized intelligence: acquisition of facts and problem-solving
ability using formal learning and experiences
• Fluid measures adaptability and flexibility when faced with new
problems
BERS-2 (Behavioral & Emotional Rating Scale):
measures personal strengths of children
Reiss Profile: evaluates personality and motivation
Carey Temperament Scales: assess temperament, unique strengths, and
needs of children at different ages
IMPLICATIONS FOR EDUCATION
•
•
•
•
relatively good fit between intellectual
and emotional development in early
childhood (preschool, K-2)
increasing disparity in later childhood
through adulthood
emotional development grows at much
slower pace
by 3rd grade, increasing need to adapt
education practices to meet both
types of development
KEYS TO SUCCESS
•
•
•
communication, staff training about
developmental asynchrony
acknowledging developmental asynchrony
does NOT mean treating older individual
with SMS like a young child
individualized education / vocational /
behavior plan should incorporate
relevant approaches in early childhood
education, even in older children and
adults
EARLY CHILDHOOD EDUCATION
APPROACH
+
AGE / IQ-APPROPRIATE GOALS
AND ACTIVITIES
=
SUCCESS FOR OLDER CHILDREN,
ADOLESCENTS, AND ADULTS!
EARLY CHILDHOOD APPROACH
•
•
•
•
•
•
use of visual cues and schedules
“smorgasbord” of varied, high interest activities of
relatively short (~20 minutes) duration
mix of academic, functional, and recreational
activities presented in a multi-sensory way
emphasis on concrete, hands-on learning
well-defined areas for different activities (cooking
center, quiet area, free play area, etc.)
individual attention; staff attuned to children’s
emotions
EARLY CHILDHOOD
APPROACH GROWN UP!
•
•
•
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use of day planner with post-its; computer-based
schedule; email / phone reminders
“smorgasbord” of school, work, volunteer, and
recreational activities of relatively short duration
emphasis on hands-on, functional aspects of
curriculum / work schedule
vary work / school activities throughout the day
vary work / school environment throughout the day
one-to-one support as needed
OBSERVATIONS
•
•
•
•
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Developmental asynchrony appears to be common in
children and adults with SMS and significantly
contributes to maladaptive behavior
Emotional development progresses with age, but at a
much slower rate than intellectual development in SMS
The bigger the disparity between intellectual and
emotional development, the greater the potential for
maladaptive behavior
Long periods of destabilized behavior further delay
emotional growth
Long periods of success, behavioral stability enhance
emotional growth
OBSERVATIONS
•
•
•
Professionals working with adolescents and adults
generally not trained in early childhood special
education approaches
Emphasis on “normalization” philosophy in adult
services ignores impact of unique SMS developmental
profile on functioning and quality of life
Individualized approach that combines appropriate
early childhood practices with age / IQ appropriate
goals often promotes success
ACKNOWLEDGMENTS
•
Many thanks to our SMS families!!!
•
Martha WS Rogers Trust, Philadelphia, PA
•
PRISMS
•
The Elwyn Genetics team:
•
Barbara Haas-Givler, MEd, BCBA
•
Elliott W Simon, PhD
•
Mary Delany, MS, CGC
•
Heather Jones (Kutztown University)