Behavioral Insights and Practical Strategies for Working with Children who have Smith-Magenis Syndrome

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Behavioral Insightsand Practical Strategies for
Working with Children who have Smith-Magenis
Syndrome

• Brenda Finucane, MS, CGC
• Executive Director, Genetic Services

www.elwyngenetics.org
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Smith-Magenis Syndrome
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Smith-Magenis Syndrome
p arm
Chromosome deletion 17p11.2
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q arm
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Smith-Magenis Syndrome
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ONYCHOTILLOMANIA
Nail yanking
POLYEMBOLOKOILAMANIA
Orifice stuffing
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Smith-Magenis Syndrome

• SELF-INJURIOUS BEHAVIORS
• Hand biting
• Head banging
• Picking at finger / toenails
• Skin picking
• Inserting objects into nose, ears, etc.

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Smith-Magenis Syndrome
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Smith-Magenis Syndrome

• SLEEP DISTURBANCE
• Frequent awakenings at night
• Early wake-up
• Sleep attacks during the day
• Inversion of melatonin cycle

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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

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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 - cant be rushed!

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Smith-Magenis Syndrome

• POSITIVE ASPECTS
• Engaging, endearing, and full of personality!
• Appreciative of attention
• Eager to please
• Sense of humor
• Communicative

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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

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Spasmodic Upper Squeezing Tic Thing, With Facial
Grimacing (self-hugging)
AUTO AMPLEXATION
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Smith-Magenis Syndrome

• Parents and Researchers Interested in
  Smith-Magenis Syndrome
• (PRISMS)
• www.prisms.org

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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
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CLASSROOM SIZE AND SETTING
Natural breaks in schedule Class composition vs.
staff match Good communication among staff Staff
training / consistency of approach
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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!

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CLASSROOM STRATEGIES WHICH ARE OFTEN SUCCESSFUL
Individualized schedule Individualized behavior
chart Visual reminders Planned breaks Opportunitie
s to request breaks Variety of positive
reinforcers Preferential seating
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Visual schedules
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Visual timers
www.timetimer.org
www.timetracker.org
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CLASSROOM STRATEGIES WHICH ARE OFTEN SUCCESSFUL
Redirection and distraction Sensory
input Transition warnings Handshakes, not
hugs Humor
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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
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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

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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

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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

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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.
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THE ABCs OF BEHAVIOR
ANTECEDENTS BEHAVIOR
CONSEQUENCE What happened before?
What happened? What
happened afterwards?
Traditional Emphasis
Emphasis Needed for Genetically-Driven Behaviors
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• BEHAVIOR CHANGE
• isnt just about the person with the syndrome!

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COMMON TRIGGERS

• Fine motor tasks
• Waiting
• Rushing
• Transitions
• Seeing people out of context
• Lack of clear expectations
• Competition for attention
• Highly-charged emotional atmosphere

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SMITH-MAGENIS SYNDROME
relatively high cognitive and social abilities
versus very young emotional development
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EMOTIONAL TODDLER IN SMS

• Emotionally volatile
• Low frustration tolerance
• Prone to tantrums / outbursts
• Attention-seeking
• Distractible
• Excitable
• Reactive
• Multisensory learners

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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

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DEVELOPMENTAL ASYNCHRONY

• 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

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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

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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

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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

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EARLY CHILDHOOD EDUCATION APPROACH AGE /
IQ-APPROPRIATE GOALS AND ACTIVITIES SUCCESS
FOR OLDER CHILDREN, ADOLESCENTS, AND ADULTS!
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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
  childrens emotions

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EARLY CHILDHOOD APPROACH GROWN UP!

• 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

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OBSERVATIONS

• 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

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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

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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)