The Impact of Poverty and Sickle Cell Disease on the Education of Children

Allison King, MD, MPH

Program in Occupational Therapy

Pediatric Hematology and Oncology

June 11, 2008

Population

• Most common genetic disease identified in the newborn period– 1 of 2,200 American infants– 1 of 400 African-American infants

• 75,000 people with SCD in the US• > 400 patients with SCD @ SLCH• 80% of SCD patients are on Medicaid

ickle Cells

Fragile

Short Survival (15-20 days vs. 120 days)

Rigid and inflexible

Chronic hemolysis - increased bilirubin

Can become stuck and form plugs in small blood vessels

S

Complications of SCD

• Chronic anemia

• Pain episodes (lasting for hours, days, to weeks)

• Life threatening lung disease

• Increased risk for infections

• Stroke (overt and silent)

Educational Attainment in Children with SCD

1. Description – What is the prevalence?

2. Mechanism – Why is this happening?

3. Intervention – How can we improve?

Cognitive Test Scores in Children with SCD with Overt strokes, Silent

Cerebral Infarcts and Normal MRIs

OvertStroke

SilentStrokes

Patient withNormal MRI

WISC-R FSIQ 70.8 82.8 90.0

WJ-RReading 75.3 81.8 91.2

WJ-R Math 65.7 83.6 93.1

Pediatrics. 1996;97: 864-870

Gradient in Global IQ

Overt Stroke < Silent Strokes < No Silent Stroke

Cerebral Infarcts

• Frontal lobe lesions• Attention (directing and maintaining)• Memory

• Diffuse lesions (frontal and parietal)• Visual-spatial• Attention• Memory

Memory and attention deficits associated with poorer reading, writing and math

Neurocognitive Evaluation of 19 Children with Silent Cerebral Infarcts

Domain Abnormal

Attention/Executive 55%

Visual Spatial/Visual Motor 30%

Language 20%

Memory 10%

Deficit in any domain 80%

Schatz J, Brown RT, Pascual JM, Hsu L, DeBaun MR. Neurology 2001;56:1109-1111

Academics of Children with SCD and Cerebral Infarcts

• Study of children with silent infarcts• 14/19 (74%) retained a grade or required

special educational services• 7/19 (37%) with poor academic achievement

in either reading or math • > 1.5 SD below mean

Schatz J, Brown RT, Pascual JM, Hsu L, DeBaun MR. Neurology 2001;56:1109-1111

Cognitive Deficits Exist Even Without Infarcts (n = 45)

Schatz J, Brown RT, Pascual JM, Hsu L, DeBaun MR. Neurology 2001;56:1109-1111

Domain Abnormal

Attention/Executive 13%

Visual Spatial/Visual Motor 33%

Language 23%

Memory 10%

Deficit in any domain 36%

Students With Academic Problems

• 74% (14 of 19) students with silent strokes had a history of being retained or required special services due to academic difficulties

• 27% (12 of 45) students without silent strokes had academic difficulties

• 6% (1 of 18) siblings without sickle cell disease had academic difficulties

Our Experience at SLCH

• Students with SCD and infarcts (N = 23)– 20% graduated from

high school in four years

• Average FS IQ – 75 (past failure) vs. 93

(no failure), p = .002

King, et al. J of School Health, 2006

Characteristic N = 23

Ages in years

5 - 9 30.4%

10 – 14 30.4%

15 and older 39.2%

Gender

Male 47.8%

Failed a grade in past

43.5%

Hypotheses

Targeted memory strategy rehabilitation will have a greater improvement in

1) memory skills and

2) academic achievement

of children with SCD, cerebral infarcts, and memory deficits than in the same group of children who receive general tutoring

Demographics

• 5 male, 6 female• Mean age: 11.6 yrs (range 8 – 16)• Mean FSIQ: 90 (range 57 – 116)• 7 overt, 4 silent• 9 completed the program

Two year feasibility program with goal of 10 students

Children's Memory Scale

0

5

10

15

20

25

Numbers Forward Numbers Backward

Sta

nd

ard

Sco

re P

oin

ts

Memory Strategies

Improvement by both groups in working memory as measured by Numbers Backward exam, p=0.04.King et al, Neurology, 2007

Tutoring

Wechsler Individual Achievement Tests - II

-2

-1

0

1

2

3

4

5

6

7

8

Reading Math Spelling

Sta

nd

ard

Sco

re P

oin

ts

Improvement in reading achievement in both groups, p=0.046.King et al, Neurology 2007.

Memory Strategies

Tutoring

Factors Associated With Cognition and Academic Achievement

Mother’s highest level of education Marital status of primary caregiver Family’s annual incomeNumber of people living in the homeGender

CognitionAcademic

achievementCerebral infarctHematocrit

Without SCD

With SCD

SES Affects Cognition

• Cross-sectional study

• Children with SCD (N = 50)

• Cognitive testing completed

• Annual income recorded

• Education levels of parents obtained

Background:SES Affects Cognition

Schatz et al, J Dev Behav Pediatr 2004

Hypothesis

• SES status has a greater impact on academic attainment than the presence of cerebral infarcts

Results:Low SES and Gender Are

Associated With Grade Retention• Cross-sectional study at SLCH

– N = 112 students with SCD

• 29% had failed a grade in past• Risk of grade failure

– Medicaid insurance (80% of cohort)• OR 14.4 (95% CI 1.9, 111.1)

– Boys • OR 2.6 (95% CI 1.1, 6.2)

– Infarcts were not statistically significant

Multi-Center Evaluation24 sites

• Clinical trial to determine the efficacy of blood transfusion therapy as a treatment for preventing silent infarcts in children with SCA

• Will screen ~1800 children with SS or S-thal0 for silent infarcts

• Will randomize ~200 children for transfusion or observation

Hypothesis• In students with sickle cell anemia, a

lower SES will result in a lower educational attainment than students with a higher SES

Demographics

• n = 447 students• Average grade in school = 3rd

• Range of grades = K through 8th

• 34% have infarcts– n = 410 students with MRI

• Mean household income = $41,600• Mean # of individuals in home = 4.5

Preliminary Results

Risk Factors for Grade Failure OR (95% CI)

Medicaid 3.2 (1.6, 6.2)

Head of Household (< high school graduate)

2.5 (1.4, 4,4)

Male gender 2.0 (1.2, 3.5)

Marital status of Primary Caregiver (non-married)

1.5 (0.9, 2.6)

Infarct 1.5 (0.8, 2.5)

Grade Failure Associations by Annual Income

$ Per Person in Home OR (95% CI)

< 5k 4.8 (2.1, 11.0)

5k - 10k 3.2 (1.5, 7.6)

> 10k 1.0

What Issues Can We Tackle?

• Cognitive deficits?– Targeted rehabilitation in progress

• What is associated with living in poverty?– Styles of parenting– Behavior

• Can interventions be developed to alter these things?

• Prospective cohort– SS or S-thal0

– Students 6-16 years of age– MRI without infarct in past 24 months

• Mothers willing to participate

Cognition in Children with Sickle Cell Anemia COCCA

Hypotheses

• Among children with SCA without cerebral infarcts, SES is a major determinant in academic achievement

• Maternal depression is associated with child cognitive impairments in children with SCA without cerebral infarcts

Measures

• Cognitive testing– Study entry– 1.5 years later

• Hematocrit• Academic records

• SES– Caregiver’s education

level– Caregiver marital

status– Annual income– Persons in home– Health insurance

(Medicaid, private, none)

Measures

• Center for Epidemiologic Studies – Depression Scale (CES-D)

• CES-D administered to mothers of children in cognitive testing

• Other maternal functioning screens– Rosenberg Self-Esteem Scale– Mirowsky and Ross Control Scale – Harter Competency Scale– Other measures of chaos, parenting,

neighborhood/community

Preliminary Data(N = 49)

• 26% of students have failed a grade– 28% have IEP– 7% have 504 Plan

• 21% of mothers screen depressive

Preliminary Associations

• Maternal depressive symptoms– Lower level of maternal education– Government assistance– Child depressive symptoms– Less supportive parenting

Preliminary AssociationsParenting Style vs. Child Behavior

• Maternal Involvement/Emotional Support– Increased child adaptive skills– Increased child leadership– Less child externalizing– Less child aggression

Preliminary AssociationsParenting vs. Child Cognition

• Harsh parenting– Lower scores on executive function

• Consistent home routine– Higher scores on executive function

• Ongoing conflict between parent and child – Lower scores on executive function

Future Directions• Parent As Teachers is a viable intervention for

this high risk population

• Randomized Trial– Children with limited financial resource will be

benefit from PARENTS AS TEACHERS program PLUS preschool program

– Versus children with limited resources who only receive preschool program

• Both groups receive preschool program for at least two years

Zigler , et al. J of Primary Prevention, 2007, 29,103-120

Summary of Overall Strategy to Improve Educational Attainment

Observation: Children with SCD fail grades often

What factors are associated with failure?Infarcts – cognitive deficitsCognitive deficits without infarctsLow SES - Medicaid

What is it about Medicaid?IncomeEducation of caregiverPsychological state of caregiver

How can we intervene?Memory remediationMultidisciplinary team support

Identify other risk factors that may improve educational

attainment

Acknowledgements

• King/DeBaun Lab• Sickle Cell Disease Team

– Kim Ferguson MSW, Teri Lindsey, PNP, Cindy Terrill CRA

• SLCH Sickle Cell Stroke Research Team– Michael Noetzel- Neurology– Bob McKinstry-Neuroradiology– Desiree White- Neuropsychology

• Gene Brody – U of GA Psychology• American Heart Association• Doris Duke Charitable Foundation• NHLBI• NINDS – SITT Executive Council and Sites