Advances in Understanding Children with Cerebral
Palsy: Risk , Resiliency, Learning, and Independence
Michael E. Msall MD
Partnering for Progress
Reaching For the Stars
Rehabilitation Institute of Chicago
Saturday August 11, 2007
Neurodevelopmental Disorders
(# Children 0-20 Years, POP 80 Million)
Condition
Rate Per 1,000 # Children
Severe Communicative
5.0
400,000
Disorders
Cognitive-Adaptive DD
5.0
400,000
(IQ <50-55)
Cerebral Palsy
Autism
Hearing Loss (>50db)
Visual Loss (<20/200)
Technology Dependent
2.5
2.0
2.0
0.5
1.0
200,000
160,000
160,000
40,000
80,000
Neurologic and Genetic Disorders
(# Children 0-20 Years, POP 80 Million)
Condition
Down Syndrome
Fragile X Syndrome
Rate Per 1,000 # Children
1.2
96,000
1.0
80,000
Fetal Alcohol Syndrome
1.0
80,000
Epilepsy
5.0
400,000
Childhood Stroke
0.5
40,000
Muscular Dystrophy
0.5
20,000
Congenital Heart Disease.
7.0
560,000
Any Congenital Disorder
30.0
2,400,000
ICF Model and Cerebral Palsy
Health Condition
Cerebral Palsy
Body Function &
Structure
Skeletal alignment
Range of motion
Muscle performance
Fitness
Environmental
Factors
Accessibility
Accommodation
Physical support
Emotional support
Activity
Participation
Mobility
Object manipulation
Communication
Self-care
Playing
Family
Friendships
Day Care
Child Care
Relatives
Personal Factors
Age
Gender
Interests
Self-regulation
Parental Concerns About Function
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Will my child be healthy?
Will my child walk?
Will my child talk?
Will my child learn self care?
Will my child learn at school?
Activities of Daily Living
Basic functions which occur daily
• Self-Care (Feed, dress, groom, bathe)
• Maintaining Continency
• Moving, Changing Positions
• Communication
• Learning
• Problem Solving/Social Interaction
• They have been used developmentally,
educationally and in rehabilitation
Total WeeFIM
10%, Mean, 90% Profiles
WeeFIM Scores
130
90%
120
Mean
110
10%
100
90
80
70
60
50
40
30
2
3
4
5
Age (years)
6
7
1983-86 RCT + Open
R
Infasurf
24-28 weeks
n=194
50
Betamethasone
55
Neither
89
Surfactant
Kwong, et al. Pediatrics 1985; 76: 585-92 and 1986;78: 767-74
Neurodevelopmental Impairments
90%
80%
70%
60%
50%
40%
30%
20%
10%
0%
79%
10%
None
CP
10%
MR
1%
Blind
0%
Deaf
Neurodevelopmental morbidity among survivors (N=149). CP
= cerebral palsy; MR = mental retardation
Functional Status at Kindergarten Entry
Walks 150 Feet
97.3%
Talks in Sentences
96.6%
Toilets Self
95.9%
Self-Care/ADL
95.9%
Msall et at. J Perinatology 1994; XIV: 41-47
Functional Outcomes and
Neurodevelopmental Disability
Walks 150 Feet
87%
Talks in Sentences
84%
Toilets Self
81%
Self-Care/ADL
81%
Understands Request
94%
Msall et at. J Perinatology 1994; XIV: 41-47
Predictors of Functional Disability
• Parenchymal Brain Injury (IVH 3-4, PVL)
• Retinopathy of Prematurity
• Sepsis
Kindergarten Readiness Status
9%
41%
50%
None/At Risk
Some
Intensive
Requirements for special education resources among survivors
of extreme prematurity (N=149)
Predictors of Special Education
Resources at Kindergarten Entry
RR
95% CI
Poverty
7.3
2.5 - 21.4
Minority status
2.5
1.2 – 5.3
Male gender
2.4
1.1 – 5.0
Msall et al. AJDC 1992; 146:1371-1375
Spectrum of Gross Motor Function in ELBW
Children with Cerebral Palsy@18Months
Vohr BR, Msall ME, Wilson D, et al.
Pediatrics 2005;116:123-129
Background and Subjects
• ELBW infants are at high risk of cerebral
palsy and neurodevelopmental sequelae.
• Children with cerebral palsy vary in their
gross motor performance
• 1860 ELBW infants born between 8/95 and
2/98 evaluated at 18 + 4 months CA.
Vohr, et al., 2005
NICHD Network Sites
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Case Western
Wayne State
University of Miami
U of New Mexico
University of Cincinnati
Indiana University
Harvard University
University of Alabama
Vohr, et al., 2005
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Emory University
Univ. of Texas, Dallas
Emory University
U Tennessee, Memphis
Yale University
Stanford University
Brown University
Research Triangle NC
GMFCS @ 18 months
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Level 0: Walks 10 steps independently
Level 1: Crawls, Pulls to stand, cruises
Level 2: Sits tripod, creeps
Level 3: Sits with support
Level 4: Rolls, good head control
Level 5: No head control
Cerebral Palsy Status at 18 months
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Cerebral Palsy in 15.2%
Abnormal Neuro in 7.5%
Diplegia in 39%
Quad/Tri in 33%
Hemi/Mono in 18%
EPS-Hypotonic in 10%
Risk Factors for CP
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Vohr, et al., 2005
Lower GA (p<.0001)
IVH Grade 3-4 ((p<.001)
PVL (p<.001)
NEC (p<.005)
CLD (p<.001)
Postnatal steroids (p<.001)
Outborn (p<.01)
Neurologic Status and GMC
GMC
Tri/Quad Di, hemi,
mono
1%
11%
Normal
Abn
Abn
Other
Other
44%
44%
Normal
Normal
I
17%
62%
42%
42%
4%
4%
II
16%
16%
6%
6%
0.3
0.3
Vohr, et al., 2005
96%
96%
Neurologic Status and GMC
GMC
III
Tri/Quad Di, hemi, Abn
mono
Other
15%
5%
3%
Normal
0.1%
IV
24%
3%
4%
0
V
27%
3%
2%
0
Vohr, et al., 2005
Multiple Disabilities
(CP+Blind/DD/HI)
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Normal/Abnormal other 0%
Monoplegia 64%
Hemiplegia 55%
Diplegia 83%
Triplegia 88%
Quadriplegia 100%
EPS Hypotonic 89%
Neurologic Status & MDI (%)
MDI
85-115
Tri/Quad Di, hemi,
mono
8
20
Abn
Other
19
Normal
47
70-84
5
17
28
31
50-69
20
4
32
18
<50
68
28
1
3
<50
Vohr, et al., 2000
Walking at 18 Months
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99.7% of normals
85.9% of other abn neuro
73% of di, hemi, monoplegia
18% of tri, quadriplegia
Vohr, et al., 2005
Health Status, Functional
Limitations, Family Supports and
Health Related Quality of Life in
Children with Cerebral Palsy
Giles Robinson, Michael E. Msall,
Michelle R. Tremont, Mary Fournier,
and Maura Taylor
Brown University and University of Chicago
SDBP Chicago, IL March 17, 2006
Purpose
• To describe medical, developmental,
functional and social factors associated with
health related quality of life (HRQOL) in
children with cerebral palsy
Hypotheses
• Higher levels of functional independence
and family supports would be related to
higher HRQOL ratings
• Children with more severe cerebral palsy
would have lower ratings of health status,
functional status, and HRQOL
Measuring Quality of Live in
Neurodevelopmental Disabilities
Medical Management, Developmental
Interventions, Family Supports
Family Values, Beliefs, Judgment
Health, Growth,
Neurological
Integrity
Social Interactions and
Community
Participation
Developmental Skills
Functional Skills
Behavioral Competencies
-Adapted from Spilker
Educational and
Vocational Successes
Developmental Status and
Support Status Assessments
• Pediatric Functional Independence Measure
(WeeFIM)TM self-care, mobility, communication
• Functional Academic Resource Scale (FARS)
• Family Support Scale
• Support Function Scale
• Health Utility Index - 2:
– Sensory, Mobility, Emotion, Cognitive, Self-care, Pain
• Feeling Thermometer
Functional Academic
Resource Scale
Describe your child’s abilities in the following areas:
Unsupervised play
Making friends with other children
Practical judgment
Following directions and completing chores
Running during a game
Participating in scouts, church, 4-H, band
(1=significantly behind, 3=about same, 5=considerably ahead)
*The higher the score, the greater the friendships, social
maturity, and community participation
Quality of Life Rating
• Using hypothetical cases of varying degrees
of health status, subject rates the cases and
then self on a Feeling Thermometer
• Self rating then becomes the HUI-2 Quality
of Life Rating
• Attributes
– Sensory, Mobility, Emotion, Cognitive, Selfcare, Pain, Fertility
Hypothetical HUI-2 Cases
• Adrian
– Can see, hear, and
talk normally
– Can walk, bend,
lift, jump, and run
normally
– Happy and not
worried most of
the time
– Learns and does
schoolwork
normally without
special help
– Can eat, bathe,
dress, and is
continent
– Free of pain
• Cynthya
– Wears glasses but
can hear and speak
normally
– Walks, bends, and
jumps with
limitations, more
slowly than others
– Sometimes angry
and fearful
– Learns schoolwork
very slowly and
needs special help
– Eats, bathes,
dresses and uses
the toilet
independently with
difficulty
– Free of pain
• Freida
– Blind, deaf,
unable to talk
– No purposeful
movement of
arms or legs
– Frequently
hospitalized to
control anger and
irritability
– Unable to learn
and remember
– Completely
dependent with
all self-care tasks
– Frequent pain
relieved by
prescription
narcotics
Feeling Thermometer
Most
Desirable
=100
Adrian
Self Rating
Cynthya
Freida
Least
Desirable
=0
Pilot Cohort
(n=26)
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89% Caucasian
46% Male
50% Diplegia
31% Hemiplegia
19% Quadriplegia
Mean age=7.9 years
Health Status (CHQ) and Functional Status
(WeeFIM), HUI-2 and Severity of Cerebral Palsy
Mean
Mean
Mean HUI-2
CHQ (SD) WFQ (SD)
(SD)
Di/Hemi
(N=21)
79 (13)
79% (19)
84.8
(11.5)
Quad
(N=5)
66 (12)
40% (24)
60.6
(33.7)
.049
.001
.185
p
CHQ = Child Health Questionnaire
WFQ = WeeFIM Quotient
HUI-2=Health Utilities Index-2
Di/Hemi = Diplegia/Hemiplegia
Quad = Quadriplegia
Family Supports, HUI-2 and Severity of
Cerebral Palsy
Mean FSS Mean SFS
(SD)
(SD)
Mean HUI-2
(SD)
Di/Hemi
(N=21)
71.5
(15.7)
48.6
(8.0)
84.8
(11.5)
Quad
(N=5)
61.1
(1.1)
36.4
(4.1)
60.6
(33.7)
p
.007
.003
.185
FSS = Family Support Scale
SFS = Support Function Scale
HUI-2= Health Utilities Index-2
Di/Hemi = Diplegia/Hemiplegia
Quad = Quadriplegia
HUI-2 - Feeling Thermometer (self)
HUI-2, Friendships and
Community Participation (FARS)
100
90
80
70
60
50
40
30
20
10
0
*
HUI-2
FARS <14
N-13
FARS = Functional Academic Resource Scale
FARS >14
N-11
*P<.05
WeeFIM Total Quotient
Functional Status, Friendships,
and Community Participation
100
90
80
70
60
50
40
30
20
10
0
*
WeeFIM Q
FARS <14
N-13
FARS >14
N-11
FARS = Functional Academic Resource Scale
*P<.001
Results
• Children with higher self reported quality of life (HUI-2) had
significantly more friendships, community participation, and social
maturity scores (FARS)
• Children with higher reported quality of life had significantly more
family supports
• Children with higher functional status scores (WeeFIM) reported
more friendships and social competencies and higher quality of life
(HUI-2) ratings
• Children with more severe cerebral palsy have lower health and
functional status rating
• No significant difference in HRQOL were found between children
with diplegia/hemiplegia versus quadriplegia in this pilot study
Conclusion
• Functional status and community
participation are key components of quality
of life in children with cerebral palsy
• Explicit measurement strategies for a larger
sample is required
• Future research measures should include
child’s perception of quality of life
The Myths of Dyslexia
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Mirror writing is a symptom of dyslexia
Eye training is a treatment for dyslexia
More boys than girls are dyslexic
Dyslexia can be outgrown
Smart people cannot be dyslexic
Shaywitz, Scientific American, Nov. 1996
Clues to Dyslexia in School-Age Children
• History
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Delayed language
Problems with the sounds of words
Expressive language difficulties
Difficulty naming
Difficulty learning to associate sounds with letters
History of reading and spelling difficulties in parents and siblings
• Reading
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Difficulty decoding single words
Particular difficulty reading nonsense or unfamiliar words
Inaccurate and labored oral reading
Slow reading
Comprehension often superior to isolated decoding skills
Clues to Dyslexia in School-Age Children
• Language
– Relatively poor performance on tests of word retrieval
– Poor performance on tests of phonologic awareness
– Relatively superior performance on tests of word recognition
• Clues most specific to young children at risk for dyslexia
– Difficulty with tests assessing knowledge of the names of
letters, the ability to associate sounds with letters, and
phonologic awareness
• Clues most specific to bright young adults with dyslexia
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Childhood history of reading and spelling difficulties
Accurate but not automatic reading
Slow performance on timed reading tests
Penalized by multiple-choice tests
Lessons from Research
• RD is a language based disorder
• Phonological processing is the key core
developmental process
• Reading single words from lists or text is
the critical unit of analysis
Lyon GR, Chahabra. MRDD Research Rev 1996;2:2-9.
Phonological Awareness
• The phoneme is the smallest unit of functional
sounds
• It can be measured in rhyming tasks*, rapid
naming, digit and word span, memory tasks
*the ability of children “to hear” the ‘at’ sound in ‘hat’
and ‘cat’
Significance of Phonology
Awareness Difficulties
• Phonological awareness difficulties co-occur with
reading disorders and impede the acquisition of
reading skills
• Limitations in phonological awareness lead to
slow, labored, and inaccurate decoding of words
• Inaccurate decoding leads to poor reading
comprehension
• The key to reading intervention is not context or
memory, but skills in decoding single words
Intervention
• Phonological deficits can be identified in
kindergarten
• Reading is the product of decoding and
comprehension
• The fundamental problem is the accurate
and fluent identification of words
Gough 1996
Preventive Intervention in
Reading Disorders
(N=180)
• Children selected by phonological
awareness scores in kindergarten
• Scores predict bottom 10% of children by
second grade
• Children had VIQ >75
• Random assignment to 4 curricula
Intervention
• Phonological awareness at oral/motor level
plus synthetic phonics (PA+)
• Implicit phonological awareness training
plus phonics instruction of reading and
spelling (Phonics)
• Regular classroom support groups receiving
individual instruction to support classroom
reading (Resource)
• No treatment control group (Control)
Intervention (cont.)
• Each instructional group received 20
minutes (4 times) per week one to one
supplemental instruction in reading
• Over 2.5 years, 88 hours of supplemental
instruction occurred
Results at End of Grade 2
Phonological Phonics Resource Control
Awareness
(N=36) (N=37) (N=32)
(N=33)
Word
Attack
99.4
86.7
86.7
81.6
Word
Ident.
98.2
92.1
92
86.3
Passage
Comp.
91.7
87.4
86.4
85.2
Results at End of Grade 2
45%
40%
35%
30%
25%
20%
15%
Grade Retention
10%
5%
0%
PA
Phonics
Resource
Control
Reading Disability and ADHD
• 40% co-morbidity in referred samples
• 15% co-morbidity in non-referred samples
• The impact of ADHD on higher cognitive
function is variable with primary impact on
rote verbal learning and memory
• ADHD exacerbates the cognitive morbidity
of RD
Conclusion
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Common sense holism
Be careful about grade repetition
Time on task matters
Promote prevention, resiliency, and
community participation
Section 504 of Rehabilitation Act
and IDEA 1997 (PL 105-17)
• School must provide reasonable accommodations
and necessary related services and special
education supports. Helpful tools include
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Peer tutoring
Cooperative learning
Graphic organizers
Study guides
Organizational routines
Learning strategies instruction
Lerner & Yasutaki, pg 476.
Principals of Accommodation
• Recognize individual differences in pacing
and cognitive style and promote full
inclusion
• ADHD is not an excuse for threatening,
noxious, or counterproductive behavior in
school or employment
Hinchlaw in Accardo & Whitman, 2000. Pg XV
WWW Resources
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IDEA
Rehab Act 504
Chad.org
Allkindsofminds.org
AACPDM.org
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Nichy.org
Dbppeds.org
Pediatrics.org
Eparent.com
Pathways.org
What We Should Do
Connect the dots: examine the relationship among risk, structural
difference, motor control, and developmental functioning
Evaluate interventions: systematic assessments coupled with safe imaging
and quality habilitative and family supports.
Use the best science to understand potential pathways that would decrease
the functional severity of CP.
Understand the pathways of risk and protection among specific cohorts of
children with one of the CP Syndromes.
Key Points
• All children with cerebral palsy are children
first, not a disease
• All children with cerebral palsy learn
• Most important preschool skills are curiosity,
communication, hand skills and self-mobility
• Adults with cerebral palsy rank communication,
self-care, and education as the most important
areas for long term success
Translational Research
Opportunities
Critically important to understand biological markers that increase
communicative, behavioral and learning impairments in vulnerable populations
Develop networks and mechanisms for tracking community outcomes for
children receiving neonatal, cardiac, oncological, neurological and genetic
interventions with respect to pathways of risk and resiliency
Optimizing outcomes among children with brain injury whether developmental
or acquired requires quality family, neurodevelopmental and educational
supports that promote functioning and social participation.
Current studies are underway examining transition to adulthood for teenagers
with chronic illness and disability in order to better understand health,
educational, vocational, and independent living outcomes.
Acknowledgments
Supported by 1U01HD37614 entitled “NICHD Family
and Child Well Being Network: Child Disability”.
Irving Harris, Herb Abelson, Paula Jaudes, and Nancy
Schwartz provided support of a shared vision and
commitment to vulnerable populations.
Larry Gray, Peter Smith, Tom Blondis, Shelly Field,
Melissa Gray, Rupa Nimmagada, Dilek Bishku, Jill
Glick, Todd Schuble, Cybele Raver, and Diana Ryan
provided critical feedback.