COLLEGE OF HEALTH & HUMAN SCIENCES DEPARTMENT OF HUMAN DEVELOPMENT AND FAMILY STUDIES STRENGTHENING GOAL-DIRECTED BEHAVIOUR FOR ADULTS WITH DS IN POST-SECONDARY SETTINGS DEBORAH J FIDLER, PHD COLORADO STATE UNIVERSITY
COLLEGE OF HEALTH & HUMAN SCIENCES DEPARTMENT OF HUMAN DEVELOPMENT AND FAMILY STUDIES
STRENGTHENING GOAL-DIRECTED
BEHAVIOUR FOR ADULTS WITH DS
IN POST-SECONDARY SETTINGS
DEBORAH J FIDLER, PHD
COLORADO STATE UNIVERSITY
FUTURE DIRECTIONS FOR CHHS:
BEHAVIORAL PHENOTYPES IN NEUROGENETIC SYNDROMES
BEHAVIORAL PHENOTYPES IN NEUROGENETIC SYNDROMES
Behavioral OutcomesBehavioral OutcomesBehavioral OutcomesBehavioral Outcomes(Cognitive, Linguistic, Social, Emotional,
Psychopathology, Motor)
Down syndrome
BEHAVIORAL PHENOTYPES
Syndrome Etiology Phenotype
Down syndrome Trisomy 21 Moderate ID, social strength, language deficits,
visual >verbal processing
Williams syndrome 7q deletion Moderate ID, outgoing, sociable, fears, anxiety,
verbal strengths, poor visual spatial
Prader-Willi syndrome 15q deletion
(paternal)
Mild ID, hyperphagia, hoarding, compulsive
behavior, tantrums, skin picking,
simultaneous>sequential processing
Smith-Magenis
syndrome
17p deletion Moderate-severe ID, self-injury, hyperactivity,
stereotypies, sleep disturbances
5p- syndrome deletion 5p Severe ID, atypical cry, hyperactivity, stereotypies,
self-injury
DOWN SYNDROME BEHAVIORAL PHENOTYPE
• Expressive Language
• Verbal Processing
• Motor Development
• SelfSelfSelfSelf----regulationregulationregulationregulation
– Goal-Directed Behavior
– Executive Function
Relative challenges:
Relative strengths:
• Receptive Language
• Visual processing
• Social Relatedness*
OVERVIEW OF DD LAB PROJECTS
COLLEGE OF HEALTH & HUMAN SCIENCES DEPARTMENT OF HUMAN DEVELOPMENT AND FAMILY STUDIES
EXECUTIVE FUNCTION IN
DOWN SYNDROME
FUNDED BY U.S. DEPARTMENT OF EDUCATION,
NATIONAL INSTITUTE FOR DISABILITY AND
REHABILITATION RESEARCH (H133G100197)
FUNDED BY U.S. DEPARTMENT OF EDUCATION,
INSTITUTE OF EDUCATIONAL SCIENCE, SPECIAL
EDUCATION RESEARCH GRANTS (R324A110136)
EXECUTIVE FUNCTION
Cognitive Cognitive Cognitive Cognitive
Processes Processes Processes Processes
integral to integral to integral to integral to
adaptive and adaptive and adaptive and adaptive and
goal directed goal directed goal directed goal directed
behaviorbehaviorbehaviorbehavior Working Memory, Inhibitory Control,
Cognitive Flexibility (Shifting), and
Planning
EXECUTIVE FUNCTION: COMPONENT PROCESSES
Working MemoryWorking MemoryWorking MemoryWorking Memory
Inhibitory ControlInhibitory ControlInhibitory ControlInhibitory Control
Cognitive FlexibilityCognitive FlexibilityCognitive FlexibilityCognitive Flexibility
PlanningPlanningPlanningPlanning
Holding and manipulating
information while completing a
task
Control of pre-potent
responses
Ability to modify thinking
and/or strategies across
contexts
Means-end behavior reliant on
some or all of the other
constructs
Will, Fidler, Daunhauer, & Gerlach-McDonald (2017)
Ex: MultiEx: MultiEx: MultiEx: Multi----step step step step
Instructions Instructions Instructions Instructions
Ex: Raising Ex: Raising Ex: Raising Ex: Raising
HandHandHandHand
Ex: Changing Ex: Changing Ex: Changing Ex: Changing
behavior based behavior based behavior based behavior based
on settingon settingon settingon setting
Ex: Ex: Ex: Ex: morning morning morning morning
routines, routes routines, routes routines, routes routines, routes
to workto workto workto work
EXECUTIVE FUNCTION
Executive Executive Executive Executive
Function Function Function Function
Adaptive Adaptive Adaptive Adaptive
BehaviorBehaviorBehaviorBehavior
Social SkillsSocial SkillsSocial SkillsSocial Skills
Academic Academic Academic Academic
AchievementAchievementAchievementAchievement
Academic Academic Academic Academic
ReadinessReadinessReadinessReadiness
Health Health Health Health
OutcomesOutcomesOutcomesOutcomes
(Blair & Razza, 2007; Bornstein et al., 2013; Diamond et al., 2007; McClelland et al., 2000; 2007; Riggs,
2012)
EF MEASUREMENT
Proxy Report
Laboratory
Assessment
PARENT BRIEF-P REPORTS:
% CLINICALLY ELEVATED [T SCORE > 65]Perc
enta
ge
BRIEF-P Domains
Total Indexes Scales
BRIEF-P: ↑ scores, ↑ EF symptoms
* * *
*
EF RESEARCH I: LABORATORY BASED MEASURES
WORKING MEMORY/INHIBITION
----Form of Simon Says
-Child must remember
rule to respond correctly
-Language check
-4 practice trials
-10 experimental trials
-Coding : Total correct
responses
-Kappa M=.89; .62-1.0
Pony & GatorPony & GatorPony & GatorPony & Gator
INHIBITORY CONTROL
-Snack placed under
cup
-Retrieve snack when
bell rung
-4 trials:
-5-, 10-, 15-, 20-s
-Coding : Total
disinhibited behaviors
-Kappa M=.78; .61-.94
Snack DelaySnack DelaySnack DelaySnack Delay
COGNITIVE FLEXIBILITY/SHIFTING
----Cards sorted on what shape Cards sorted on what shape Cards sorted on what shape Cards sorted on what shape
dimensiondimensiondimensiondimension
----1 practice1 practice1 practice1 practice
----6 trials first rule6 trials first rule6 trials first rule6 trials first rule
----Rule is changedRule is changedRule is changedRule is changed
----6 trials post6 trials post6 trials post6 trials post----switchswitchswitchswitch
----12 trials border version12 trials border version12 trials border version12 trials border version
----Coding : Total correct Coding : Total correct Coding : Total correct Coding : Total correct
responsesresponsesresponsesresponses
----Kappa Kappa Kappa Kappa MMMM=.85; .64=.85; .64=.85; .64=.85; .64----1.01.01.01.0
DCCSDCCSDCCSDCCS
PLANNING
-Children are told Here
are some toys for you
-Play with unusual
group of objects
-2 minutes
-Coding : Total new
objects explored; total
functional acts
-Kappa M=.77; .63-1.0
GenerativityGenerativityGenerativityGenerativity
METHODS
N = 42N = 42N = 42N = 42NVMA=49.98 NVMA=49.98 NVMA=49.98 NVMA=49.98 mosmosmosmos
CA=91.07 CA=91.07 CA=91.07 CA=91.07 mosmosmosmos
N = 38N = 38N = 38N = 38NVMA= 50 NVMA= 50 NVMA= 50 NVMA= 50 mosmosmosmos
CA= 40.52 CA= 40.52 CA= 40.52 CA= 40.52 mosmosmosmos
DSDSDSDS TDTDTDTD
Daunhauer, Gerlach-McDonald, Will & Fidler (2017)
LABORATORY MEASURES OF EF
DSDSDSDS
n= 42n= 42n= 42n= 42
TDTDTDTD
n= 38n= 38n= 38n= 38
M (SD) M (SD)
Raw Score Z-Score Raw Score Z-Score p
Working Working Working Working
memorymemorymemorymemory 5.81(2.17) -.29(.83) 7.16(2.39) .23(.92) .05
InhibitionInhibitionInhibitionInhibition .76(1.26) .14(1.10) .23(.77) -.33(.68) .131
Shifting Shifting Shifting Shifting .495(.44) -.06(1.0) .68(.42) .35(.97) .163
Planning Planning Planning Planning 5.10(2.41) -.18(.78) 7.0(3.0) .43(.97) .01
OVERVIEW OF DD LAB PROJECTS
How does the emerging EF profile in DS impact
adaptation and achievement in home and
community settings?
What are the implications for intervention?
EXECUTIVE FUNCTION
EF and EF and EF and EF and
Functional Functional Functional Functional
PerformancePerformancePerformancePerformance
Daunhauer, Gerlach-McDonald, Will
& Fidler (2017)
EF AND FUNCTIONAL PERFORMANCE
Functional Performance:
The self-care, mobility, and social function that one needs in order to be effective in their daily environment
METHODS
DS DS DS DS = = = = 30303030NVMANVMANVMANVMA= 49.98 = 49.98 = 49.98 = 49.98 mosmosmosmos
CACACACA= 91.07 = 91.07 = 91.07 = 91.07 mosmosmosmos
ParticipantsParticipantsParticipantsParticipants
Daunhauer, Gerlach-McDonald, Will & Fidler (2017)
LeiterLeiterLeiterLeiter----RRRR
PEDI PEDI PEDI PEDI ((((The Pediatric Evaluation of The Pediatric Evaluation of The Pediatric Evaluation of The Pediatric Evaluation of
Disability Disability Disability Disability Inventory (PEDI; Haley, Inventory (PEDI; Haley, Inventory (PEDI; Haley, Inventory (PEDI; Haley,
et et et et al., al., al., al., 1992)1992)1992)1992)
EF BatteryEF BatteryEF BatteryEF Battery
BRIEFBRIEFBRIEFBRIEF----PPPP
MeasuresMeasuresMeasuresMeasures
FINDINGS: EF AND FUNCTIONAL PERFORMANCE
Self-Care (PEDI)
Planning Planning Planning Planning ((((Generativity)Generativity)Generativity)Generativity)
Working Working Working Working MemoryMemoryMemoryMemory(BRIEF(BRIEF(BRIEF(BRIEF----P)P)P)P)
F (3, 27) = 4.41; p = .012; R2 = .25
Daunhauer, Gerlach-McDonald, Will & Fidler (2017)
EXECUTIVE FUNCTION
EF and EF and EF and EF and
School Function School Function School Function School Function
Daunhauer, Fidler, & Will (2014)
WHAT IS SCHOOL FUNCTION?
A student’s ability to perform
important functional
activities that support or
enable participation in the
academic and related social
aspects of an educational
program (Coster et al., 1998, p. 2)
SCHOOL FUNCTION
Coster et al., 1998
using school-related
materials appropriately
requesting assistance when needed the ability to move around the school
environment independently
ability to manage
self-care and
personal needs
SCHOOL FUNCTION
School function and academic
aspects of schooling are
different
School function skills enable
students to engage in
academic activities without
assistance or
accommodations
Which factors are important for
competent school function in DS?
SCHOOL FUNCTION
To identify school function profile in
primary school-aged students with Down
syndrome
Predictors of School Function Outcomes:
• Language
• IQ
• Executive Function
FUNDED BY U.S.
DEPARTMENT OF
EDUCATION, NATIONAL
INSTITUTE FOR DISABILITY
AND REHABILITATION
RESEARCH (H133G100197)
FUNDED BY U.S.
DEPARTMENT OF
EDUCATION, INSTITUTE OF
EDUCATIONAL SCIENCE,
SPECIAL EDUCATION
RESEARCH GRANTS
(R324A110136) DAUNHAUER, FIDLER, & WILL (2014)
METHODS
DS DS DS DS = = = = 29292929CA=94.35 (months)
NVMA=47.88 (months)
20 male
90% Caucasian
ParticipantsParticipantsParticipantsParticipants
School Function Assessment
(SFA)
Leiter-R
Oral and Written Language
Scales (OWLS)
Teacher report BRIEF-P
MeasuresMeasuresMeasuresMeasures
DAUNHAUER, FIDLER, & WILL (2014)
SCHOOL FUNCTION: RESULTS
bbbb SE(b)SE(b)SE(b)SE(b) BBBB pppp
Intercept 134.34 20.32 <.001
MA -.30 .33 -.18 .385
Lang. -.04 .23 -.03 .867
EF ----.86.86.86.86 .19.19.19.19 ----.74.74.74.74 <<<< ....001001001001
Cognitive-Behavioral Tasks
emerged as most
problematic area for
students with DS:
– Compliance
– Task
Behavior/Completion
– Safety
– Social Conventions
– Positive Interaction
Profile
Predictors of School Function
DAUNHAUER, FIDLER, & WILL (2014)
Adjusted R2 = .55; F = (3, 17), p < .001
IN OTHER WORDS…
Executive function skills (especially
planning and working memory) are critical
for both school function and functioning in
daily life
EXECUTIVE FUNCTION
EF and Adult EF and Adult EF and Adult EF and Adult
EmploymentEmploymentEmploymentEmployment
Gerlach-McDonald, Fidler, Telapatra
& Riley (in press)
METHODS
EF AND EMPLOYMENT IN ADULTS WITH DS
----Vineland Adaptive Vineland Adaptive Vineland Adaptive Vineland Adaptive
Behavior ScalesBehavior ScalesBehavior ScalesBehavior Scales----IIIIIIII
----BriefBriefBriefBrief----Adult versionAdult versionAdult versionAdult version
----Employment Employment Employment Employment
informationinformationinformationinformation
MeasuresMeasuresMeasuresMeasures
Gerlach-McDonald, Fidler, Telapatra & Riley (in press)
- 31 Young Adults 31 Young Adults 31 Young Adults 31 Young Adults
with DSwith DSwith DSwith DS- 55%male
- 84% Caucasian
- M FSIQ = 47
- M CA=25.9 yrs
ParticipantsParticipantsParticipantsParticipants
ASSOCIATIONS WITH EMPLOYMENT STATUS (POINT BISERIAL
CORRELATIONS)
Domain NameDomain NameDomain NameDomain Name Employment StatusEmployment StatusEmployment StatusEmployment Status
Communication (VABSCommunication (VABSCommunication (VABSCommunication (VABS----2)2)2)2) .223
Daily Living Skills(VABSDaily Living Skills(VABSDaily Living Skills(VABSDaily Living Skills(VABS----2)2)2)2) .545*
Socialization (VABSSocialization (VABSSocialization (VABSSocialization (VABS----2)2)2)2) .281
Working Memory (BRIEFWorking Memory (BRIEFWorking Memory (BRIEFWorking Memory (BRIEF----A)A)A)A) -.575*
Shift (BRIEFShift (BRIEFShift (BRIEFShift (BRIEF----A)A)A)A) -.489*
Plan/Organize (BRIEFPlan/Organize (BRIEFPlan/Organize (BRIEFPlan/Organize (BRIEF----A)A)A)A) -.369
Task Monitor (BRIEFTask Monitor (BRIEFTask Monitor (BRIEFTask Monitor (BRIEF----A)A)A)A) -.403
Full Scale IQ (SBFull Scale IQ (SBFull Scale IQ (SBFull Scale IQ (SB----5)5)5)5) .283
PREDICTING EMPLOYMENT STATUS
Currently
employedWorking Working Working Working MemoryMemoryMemoryMemory
VABSVABSVABSVABS----Daily Living Daily Living Daily Living Daily Living
SkillsSkillsSkillsSkills
OVERVIEW OF PRESENTATION
SYNDROME-INFORMED INTERVENTION
PLANNING
�Behavioral phenotype research may
make it possible to ‘‘know where to
look’’ for potential vulnerabilities
�Parents/practitioners use information
regarding phenotypic predispositions to
monitor potential areas of vulnerability
SYNDROME-INFORMED INTERVENTION
PLANNING (FIDLER, PHILOFSKY & HEPBURN, 2007)
If interventions are natural and without harm,
then potential ‘‘over-employment’’ is of low risk,
while the gains may be of high benefit
SMALL GROUP DISCUSSION QUESTIONS
• What are some common ways that we use executive function skills
during adult life?
– Working memory
– Inhibitory Control
– Shifting
– Planning
• How might you work to practice these skills in naturalistic ways?
Identify one idea for each construct.
OVERARCHING STUDY GOALS
• ADULT ED SERIES ADULT ED SERIES ADULT ED SERIES ADULT ED SERIES funded by the Global Down
Syndrome Foundation
• Identify new ways to continue to support
adaptation/EF growth and development
throughout the lifespan
• Enhance adult education experiences by
shaping curriculum and instruction to meet
needs of individuals with DS
STUDY MODULES
• Dance and Physical Activity
• Nutrition and Healthy choices
• Social Communication
• Personal Safety
PROJECT RATIONALE
• All activities aimed to enhance adaptation and goal-
directed behavior in adults with DS to support growth
and well-being
• Each activity was an opportunity to practice more
basic aspects of goal-directed behavior and cognitive
underpinnings
– Specific opportunities were given to practice aspects
of EF
PHENOTYPE INFORMED EF TARGETS
• Working memory: “updating” necessary for following
recipes, remembering steps to a dance
• Inhibitory control: group participation, resisting the
urge to quit during more challenging exercise, waiting
for recipe information before adding ingredients
• Shifting: natural transitions during each part of a
class session (group discussions/warm
up/exercise/group discussions)
• Planning: organizing steps of activities; integration of
activities at home with homework
KEY FEATURE OF PROJECT
Phenotype informed modifications
• Visual supports for instructions and planning
• Activities embedded in socially rewarding contexts
• Supports to enhance/accommodate for working
memory needs
• Minimizing expressive language demands
• Token economy
WELLNESS I: PHYSICAL ACTIVITY
• Week 1 Defining Exercise
• Week 2 Stretching
• Week 3 Exercises Targeting Core
• Week 4 Exercises Targeting Upper Body
• Week 5 Nutrition During Exercise
• Week 6 Exercises Targeting Lower Body
• Week 7 Group and Team Exercise
• Week 8 Benefits of Exercise
• Week 9 Selecting and Planning for Exercises
• Week 10 Review
SAMPLE LESSON
• Opening
– Social greeting and Warm Up song and Dance
• Group Discussion
– Review of material from previous sessions by asking students questions regarding content (e. g. “Who can tell me some of the steps to exercise?”)
• Warm up and Stretching
– Warm up song video and Personal Trainer lead stretching session
• Lecture
– Introduction to new content (e .g. What positive changes happen when you exercise?”) and visual power point lecture slides to accompany material
• Exercise
– Personal Trainer demonstrates various circuit stations and divides group into smaller groups (2-3 individuals per 1 course assistant)
– Cool Down
• Dance and Stretching
COOKING AND NUTRITION MODULE TOPICS
• Food groups,
• Food for fuel,
• Portion sizes,
• Nutrition label,
• Types of cooking,
• Building a pantry,
• Food allergies,
• A field trip to the grocery store,
• Planning a dinner party,
• Having a dinner Party
PARTICIPATION RATE AT EACH SESSION BY MODULE
0%
10%
20%
30%
40%
50%
60%
70%
80%
90%
100%
Physical Activity Nutrition Safety Communication
Perc
enta
ge o
f Adults P
art
icip
ating in L
essons a
nd A
ctivi
ties
Module
Participation by Module
SOCIAL ENGAGEMENT
69%
75%
83%
76%
0%
10%
20%
30%
40%
50%
60%
70%
80%
90%
Module 1 Module 2 Module 3 Module 4
Perc
ent Engaged
Module
Engagement with Peers & Course Materials by Module
Trendline
PRELIMINARY FINDINGS
VABS DomainVABS DomainVABS DomainVABS Domain BaselineBaselineBaselineBaseline
M(SD)M(SD)M(SD)M(SD)
PostPostPostPost----InterventionInterventionInterventionIntervention
M(SD)M(SD)M(SD)M(SD)
Receptive vReceptive vReceptive vReceptive v----ScaleScaleScaleScale 9.38(3.49) 10.0(4.16)
Expressive vExpressive vExpressive vExpressive v----ScaleScaleScaleScale 7.23(3.66) 7.45(3.92)
Written vWritten vWritten vWritten v----ScaleScaleScaleScale 6.97(2.42) 7.55(2.48)
Communication SSCommunication SSCommunication SSCommunication SS 44.7(22.2)44.7(22.2)44.7(22.2)44.7(22.2) 48.9(19.5)48.9(19.5)48.9(19.5)48.9(19.5)
Personal vPersonal vPersonal vPersonal v----ScaleScaleScaleScale 7.55(3.23) 8.36(2.66)
Domestic v scoreDomestic v scoreDomestic v scoreDomestic v score 8.32(2.86) 9.10(2.90)
Community v scoreCommunity v scoreCommunity v scoreCommunity v score 6.26(2.57) 6.41(3.05)
Daily Living Skills SSDaily Living Skills SSDaily Living Skills SSDaily Living Skills SS 55.5(14.8)55.5(14.8)55.5(14.8)55.5(14.8) 57.8(14.7)57.8(14.7)57.8(14.7)57.8(14.7)
Interpersonal Interpersonal Interpersonal Interpersonal
RelationshipsRelationshipsRelationshipsRelationships
9.03(2.50) 8.77(2.94)
Play and LeisurePlay and LeisurePlay and LeisurePlay and Leisure 9.45(2.42) 9.64(2.08)
CopingCopingCopingCoping 11.2(1.94) 11.9(2.59)
Socialization SSSocialization SSSocialization SSSocialization SS 64.0(13.7) 65.0(15.2)
Adaptive Behavior Adaptive Behavior Adaptive Behavior Adaptive Behavior
Composite SSComposite SSComposite SSComposite SS
52.9(15.4)52.9(15.4)52.9(15.4)52.9(15.4) 55.3(15.3)55.3(15.3)55.3(15.3)55.3(15.3)
First round of piloting complete, working to
identify additional sources of funding for scaling
up and additional curriculum development
BROADER EF SKILL DEVELOPMENT
• Divide tasks into smaller parts, then plan
order of actions
• Embed EF practice in fun, pleasurable
activities
– Games, sports, mindfulness activities
BROADER EF SKILL DEVELOPMENT
• Card games, board games
– working memory: Concentration, Go Fish
– processing speed: Speed
– Strategizing: Sorry!, Battleship, Mancala, Checkers
• Physical Activities/games
– Monitoring, rule following, decision making, self-control :
Four Square, Tetherball
– Organized sports—attention skills
– Mindfulness activities (yoga, Tae Kwon Do)
BROADER EF SKILL DEVELOPMENT
• Strategy games
– Attention, working memory, cognitive flexibility:
Puzzles, brain teasers, matching games
– Working memory, flexible thinking: Guessing
games
• Music, singing, dance
– Attention, self-monitoring--Playing an instrument
GENERAL IMPLICATIONS
• Bridging behavioral phenotype science and
intervention practice will strengthen education and
intervention for individuals with Down syndrome
across the lifespan
• We can continue to support growth and
development of goal-directedness skills into
adulthood through engaging and phenotype-
informed educational opportunities
ACKNOWLEDGEMENTS
� Lisa Daunhauer, ScD
� Karen Riley, PhD
� Brianne Gerlach-McDonald, PhD
� Susan Hepburn, PhD
� Amy Needham, PhD
� Sally Rogers, PhD
� Laura Hahn, PhD
� Elizabeth Will, PhD
� Nancy Raitano Lee, PhD
� Audrey Blakely-Smith, PhD
� Amy Philofsky, PhD
� Cordelia Robinson, RN, PhD
� David Most, PhD
DD LAB @ CSU
� Emily Schworer, MS
� Mackenzie Howshar, BS
• Emily Legatski
• Bailey Geisen
• Hayley Lewis
• Anna Pritchard
• Lauren Carlson
• Mariah Hancock
• Kathryn Gerken
• Emily Pennington
• Aimee Wakeman
With gratitude to the families, children, and adults
who participated in this research
COLLEGE OF HEALTH & HUMAN SCIENCES DEPARTMENT OF HUMAN DEVELOPMENT AND FAMILY STUDIES
QUESTIONS?