Behavioral Insightsand 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

Chromosome

deletion

17p11.2

“q” arm

“p” arm

17

POLYEMBOLOKOILAMANIA Orifice stuffing

ONYCHOTILLOMANIA Nail

yanking

?

Smith-Magenis Syndrome

SELF-INJURIOUS BEHAVIORS

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 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

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

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

Visual schedules

Visual timers

www.timetracker.orgwww.timetimer.org

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

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

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

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)