Sickle Cell Disease and Developmental Conditions

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    Dominique Thomas

    Dr. Qixuan Chen

    Dr. Sara Lopez-Pintado

    Sickle Cell Disease and

    Developmental Conditions

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    Sickle Cell Disease

    Genetic disordercharacterized by

    defective hemoglobin

    in red blood cells

    Affects 1 in 350 Black

    births and 70,000

    Americans

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    Purpose

    To determine if SickleCell Disease

    significantly affects

    developmental

    conditions in AfricanAmerican children

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    Methods National Health Interview Surveys

    1997-2007

    Annual multistage probability sample survey of civilian

    non-institutionalized population of U.S.

    20,799 African American children aged 0

    17 years 2004 data was omitted from project for lack of RACE

    variable

    Coding 0 = Has never had disease/disability

    1 = Has had disease/disability

    Data was recoded into order to provide consistency in

    interpretation

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    Methods (cont.) Variables

    Sickle Cell Disease (SCD)Attention Deficit Disorder/Attention Deficit Hyperactivity

    Disorder (ADD) Learning Disability (LEARND)

    Intellectual Disability (AMR) Hearing Disability (CHEARST) Vision Disability (CVISION)

    Statistical Analysis

    SPSS Contingency Tables Chi-Square Tests Odds Ratio

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    Results

    AMR ADD LEARND CHEARST CVISION

    P(x = 1|SCD = 0) 0.0095 0.0559 0.0816 0.0275 0.0292

    P(x = 1|SCD = 1) 0.0251 0.0925 0.1553 0.06 0.04

    Odds Ratio (OR) 2.69 1.72 2.07 2.25 1.39

    Chi-Square Test p = 0.044F p = 0.037 p = 0.001 p = 0.005 p = 0.367

    95% CI (1.096, 6.617) (1.026, 2.891) (1.347, 3.182) (1.251, 4.068) (0.680, 2.824)

    FFishers Exact Test

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    Results (cont.)

    MALE AMR ADD LEARND CHEARST CVISION

    P(x = 1|SCD = 0) 0.012 0.082 0.109 0.031 0.027

    P(x = 1|SCD = 1) 0.027 0.125 0.218 0.073 0.064

    Odds Ratio (OR) 2.30 1.59 2.29 2.45 2.49

    Chi-Square Test p = 0.152F p = 0.130 p = 0.001 p = 0.022F p = 0.029F

    95% CI (0.719, 7.330) (0.867, 2.932) (1.373, 3.831) (1.184, 5.079) (1.147, 5.404)

    FFisher's Exact Test

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    Results (cont.)

    FEMALE AMR ADD LEARND CHEARST CVISION

    P(x = 1|SCD = 0) 0.007 0.029 0.054 0.024 0.032

    P(x = 1|SCD = 1) 0.022 0.052 0.081 0.044 0.011

    Odds Ratio (OR) 3.33 1.84 1.54 1.89 0.34

    Chi-Square Test p = 0.128F p = 0.288F p = 0.296F p = 0.173F p = 0.373F

    95% CI (0.804, 13.796) (0.666, 5.062) (0.666, 3.575) (0.689, 5.199) (0.047, 2.458)

    FFisher's Exact Test

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    Discussion

    Initial analyses provide sufficient evidence tosuggest that children with SCD are more likely to

    have intellectual disabilities, ADD/ADHD, learning

    disabilities or hearing problems than children

    without SCD

    However, adjusting for gender, there is sufficient

    evidence to conclude that there is a significant

    relationship only between SCD and learning,

    vision, and hearing disabilities in boys.

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    Limitations

    Parents completedthe survey

    Misdiagnosis

    Missing data

    Confounding variables

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    References

    Boulet, S. L., Yanni, E. A., Creary, M. S., Olney,R. S. (2010). Health status and healthcare use

    in a national sample of children with sickle cell

    disease. American Journal of Preventive

    Medicine, 38, S528

    S535.

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    Acknowledgements

    Biostatistics Department

    BEST Diversity Program

    Dr. Qixuan Chen and Dr. Sara Lopez-Pintado