brenda finucane, ms, cgc executive director, genetic services
DESCRIPTION
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. Chromosome deletion 17p11.2. “q” arm. - PowerPoint PPT PresentationTRANSCRIPT
Behavioral Insightsand Practical Strategies for
Working with Children who have Smith-Magenis Syndrome
Brenda Finucane, MS, CGC
Executive Director, Genetic Services
www.elwyngenetics.org
SELF-INJURIOUS BEHAVIORS
Hand biting Head banging Picking at finger / toenails Skin picking Inserting objects into
nose, ears, etc.
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 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
Smith-Magenis Syndrome
Smith-Magenis Syndrome
Parents and Researchers Interested in Smith-Magenis Syndrome
(PRISMS)
www.prisms.org
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
CLASSROOM SIZE AND SETTING
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!
STUDENT / STAFF MATCH
Individualized schedule
Individualized behavior chart
Visual reminders
Planned breaks
Opportunities to request breaks
Variety of positive reinforcers
Preferential seating
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 SUCCESSFUL
• 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
CLASSROOM STRATEGIES WHICH
ARE OFTEN UNSUCCESSFUL
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
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
Genetically-driven Behavior
ANTECEDENTS BEHAVIOR CONSEQUENCEWhat happened before? What happened? What happened afterwards?
THE ABCs OF BEHAVIOR
Teacher asked studentwith 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.
ANTECEDENTS BEHAVIOR CONSEQUENCEWhat happened before? What happened? What happened afterwards?
THE ABCs OF BEHAVIOR
Traditional Emphasis
Emphasis Needed for Genetically-Driven Behaviors
COMMON TRIGGERS
• Fine motor tasks
• Waiting
• Rushing
• Transitions
• Seeing people out of context
• Lack of clear expectations
• Competition for attention
• Highly-charged emotional atmosphere
relatively high cognitive and social abilities
versus
very young emotional
development
SMITH-MAGENIS SYNDROME
“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
• 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
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
Developmental Asynchrony Study
• 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
IMPLICATIONS FOR EDUCATION
• 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
KEYS TO SUCCESS
EARLY CHILDHOOD EDUCATION APPROACH
+AGE / IQ-APPROPRIATE GOALS
AND ACTIVITIES
=SUCCESS FOR OLDER CHILDREN,
ADOLESCENTS, AND ADULTS!
• 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
• 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
EARLY CHILDHOOD APPROACH GROWN UP!
• 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
OBSERVATIONS