1. 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

2. Neurodevelopmental Disorders (# Children 0-20 Years, POP 80 Million)

3. Neurologic and Genetic Disorders (# Children 0-20 Years, POP 80 Million)

5. ICF Model and Cerebral Palsy Health Condition Cerebral Palsy Body Function & Structure Skeletal alignment Range of motion Muscle performance Fitness Activity Mobility Object manipulation Communication Self-care Playing Participation Family Friendships Day Care Child Care Relatives Environmental Factors Accessibility Accommodation Physical support Emotional support Personal Factors Age Gender Interests Self-regulation

7. Parental Concerns About Function 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?

8. 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

12. 1983-86 RCT + Open Infasurf R 24-28 weeks n=194 50 Betamethasone 55 Neither Kwong, et al. Pediatrics 1985; 76: 585-92 and 1986;78: 767-74 89 Surfactant

13. Neurodevelopmental Impairments Neurodevelopmental morbidity among survivors (N=149). CP = cerebral palsy; MR = mental retardation

14. Functional Status at Kindergarten Entry Msall et at. J Perinatology 1994; XIV: 41-47

15. Functional Outcomes and Neurodevelopmental Disability Msall et at. J Perinatology 1994; XIV: 41-47

16. Predictors of Functional Disability Parenchymal Brain Injury (IVH 3-4, PVL) Retinopathy of Prematurity Sepsis

17. Kindergarten Readiness Status Requirements for special education resources among survivors of extreme prematurity (N=149) 41% 50% 9%

18. Predictors of Special Education Resources at Kindergarten Entry RR95% CI Poverty7.32.5 - 21.4 Minority status 2.51.2 – 5.3 Male gender2.41.1 – 5.0 Msall et al. AJDC 1992; 146:1371-1375

20. 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 Spectrum of Gross Motor Function in ELBW Children with Cerebral Palsy@18Months

21. 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 Background and Subjects

22. NICHD Network Sites Case Western Wayne State University of Miami U of New Mexico University of Cincinnati Indiana University Harvard University University of Alabama Emory University Univ. of Texas, Dallas Emory University U Tennessee, Memphis Yale University Stanford University Brown University Research Triangle NC Vohr, et al., 2005

23. GMFCS @ 18 months 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

24. Cerebral Palsy Status at 18 months 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% Cerebral Palsy in 15.2% Diplegia in 39% Quad/Tri in 33% Hemi/Mono in 18% EPS-Hypotonic in 10%

25. Risk Factors for CP 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) Vohr, et al., 2005

26. Neurologic Status and GMC Vohr, et al., 2005 Neurologic Status and GMC

27. Vohr, et al., 2005

28. Multiple Disabilities (CP+Blind/DD/HI) Normal/Abnormal other 0% Monoplegia 64% Hemiplegia 55% Diplegia 83% Triplegia 88% Quadriplegia 100% EPS Hypotonic 89%

29. Neurologic Status & MDI (%) Vohr, et al., 2000

31. Walking at 18 Months 99.7% of normals 85.9% of other abn neuro 73% of di, hemi, monoplegia 18% of tri, quadriplegia Vohr, et al., 2005

33. 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

34. Purpose To describe medical, developmental, functional and social factors associated with health related quality of life (HRQOL) in children with cerebral palsy

35. 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

36. Measuring Quality of Live in Neurodevelopmental Disabilities Medical Management, Developmental Interventions, Family Supports Family Values, Beliefs, Judgment Health, Growth, Neurological Integrity Developmental Skills Functional Skills Behavioral Competencies Social Interactions and Community Participation Educational and Vocational Successes -Adapted from Spilker

37. 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

38. 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

39. 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, Self- care, Pain, Fertility

40. 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 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 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

41. Feeling Thermometer Most Desirable =100 Least Desirable =0 Self RatingCynthya Adrian Freida

42. Pilot Cohort (n=26) 89% Caucasian 46% Male 50% Diplegia 31% Hemiplegia 19% Quadriplegia Mean age=7.9 years

43. Health Status (CHQ) and Functional Status (WeeFIM), HUI-2 and Severity of Cerebral Palsy CHQ = Child Health QuestionnaireDi/Hemi = Diplegia/Hemiplegia WFQ = WeeFIM QuotientQuad = Quadriplegia HUI-2=Health Utilities Index-2

44. Family Supports, HUI-2 and Severity of Cerebral Palsy FSS = Family Support ScaleDi/Hemi = Diplegia/Hemiplegia SFS = Support Function ScaleQuad = Quadriplegia HUI-2= Health Utilities Index-2

45. HUI-2, Friendships and Community Participation (FARS) HUI-2 - Feeling Thermometer (self) *P<.05 * FARS = Functional Academic Resource Scale N-13N-11

46. Functional Status, Friendships, and Community Participation WeeFIM Total Quotient *P<.001 * FARS = Functional Academic Resource Scale N-13N-11

47. 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

48. 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

49. The Myths of Dyslexia 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

50. Clues to Dyslexia in School-Age Children History –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 –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

51. 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 –Childhood history of reading and spelling difficulties –Accurate but not automatic reading –Slow performance on timed reading tests –Penalized by multiple-choice tests

52. 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.

53. 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’

54. 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

55. 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

56. 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

57. 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)

58. 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

59. Results at End of Grade 2 Phonological Awareness (N=33) Phonics (N=36) Resource (N=37) Control (N=32) Word Attack 99.486.7 81.6 Word Ident. 98.292.19286.3 Passage Comp. 91.787.486.485.2

60. Results at End of Grade 2

61. 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

62. Conclusion Common sense holism Be careful about grade repetition Time on task matters Promote prevention, resiliency, and community participation

63. 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 –Peer tutoring –Cooperative learning –Graphic organizers –Study guides –Organizational routines –Learning strategies instruction Lerner & Yasutaki, pg 476.

64. 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

65. WWW Resources IDEA Rehab Act 504 Chad.org Allkindsofminds.org AACPDM.org Nichy.org Dbppeds.org Pediatrics.org Eparent.com Pathways.org

66. 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.

67. 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

68. 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.

70. 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.