Performance of Children with Sickle Cell Disease

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Performance of Children with Sickle Cell Disease

• Paige C. Hays
• Washington University School of Medicine
• Program in Occupational Therapy
• April 6th, 2006

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Sickle Cell Disease (SCD)

• Inherited hematological disorder
• Red blood cells can develop a sickle shape and
  rigid cell membrane
• Sickle cell anemia is the most common and severe
  variant, occurring in 1/375 African Americans
• (Sickle Cell Disease Panel, 1993)

Image taken from the American Medical Association
website http//www.ama-cmeonline.com/pain_mgmt/m
odule03/04raci/10_01.htm
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Complications of SCD

• Two main causes of complications
• Shortened life span of red blood cell causing
  anemia
• Vaso-occlusions causing pain crises, acute chest
  syndrome, and tissue damage in major organs
• Cerebrovascular accidents (CVAs)
• (Beers et al., 2005 National Institutes of
  Health, Division of Heart, Lung, and Blood
  Institute, 2002 Ashley-Koch, Yang, Olney,
  2000)
• A study of 63 children with SCD found that 33 had
  evidence of a CVA on neuroimaging
• Location of lesions 90 occurred in the frontal
  lobes, also high rates for the temporal and
  parietal lobes
• (Brown, Davis, Lambert, Hsu, Hopkins, and Eckman,
  2000)

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Effects of SCD on Children

• Damage to brain tissue
• CVAs
• Subtle and progressive vessel disease
• Hypoxia from chronic anemia, sleep hypoxia, or
  poor pulmonary functioning
• Chronic condition with pain and fatigue
• High rates of absenteeism from school
• (Noll et al., 2001)

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School Performance

• Higher rates of grade repetition and service use
  for poor performance
• Lower performance on measure of academic
  achievement, such as reading and math assessment
  scores
• (Schatz, 2004 Schatz et al., 2001)
• Of 26 children, 17 to 19 years old with SCD, only
  15 were on target to graduate
• Of 39 children with strokes or 3
  hospitalizations in the last year, only 49 had
  been evaluated for an individualized education
  plan (IEP)
• (Herron, Bacak, King, DeBaun, 2003)

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Executive Functioning

• In occupational therapy, we have adopted the
  terms of awareness and executive functions to
  describe the main components of higher-level
  cognition
• (Katz and Hartmann-Maeir, 2005, p. 3-4)
• Executive functioning is a macroconstruct that
  includes phases of problem solving (problem
  representation, planning, execution, and
  evaluation) that captures the way in which
  subfunctions work together to accomplish a single
  higher-order function
• (Zelazo, Carter, Reznick, and Frye, 1997, p. 220)
• Measuring executive functioning
• Ecological validity of tests of executive
  functioning- do they predict real life
  performance?
• Traditional measure vs. performance-based measure
• (Chaytor, Schitter-Edgecombe, 2003 Burgess,
  Alderman, Evans, Emslie, Wilson, 1998 Wilson,
  1993)

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Aim and Hypothesis

• The aim of this study is to examine executive
  functioning performance of children 8 to 12 years
  of age with SCD.
• Hypothesis
• Children with SCD will show deficits on
  traditional and performance-based measures of
  executive functioning compared to controls.

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Participants

• Inclusion criteria African American, ages 8-12
  years
• Exclusion criteria aphasias, limited English
  language skills, disabilities that would
  significantly interfere with test performance
• For children with SCD child reporting pain at
  the time of testing
• For controls receiving special education
  services or have a diagnosis of a learning
  disability
• Recruitment Department of Hematology/ Oncology
  at St. Louis Childrens Hospital and the local
  community

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Measures

• Behavior Rating Inventory of Executive Function
  (BRIEF), parent and teacher forms
• (Gioia, Isquith, Guy, Kenworthy, 2000)
• Traditional neuropsychological tests of executive
  functioning
• Digit Span
• (Wechsler, 2003)
• Delis-Kaplan Executive Function System Sorting
  Test
• (Delis, Kaplan, Kramer, 2001)
• Delis-Kaplan Executive Function System
  Color-Word Interference Test
• (Delis, Kaplan, Kramer, 2001)
• Performance-based test of executive functioning
• Kiddy Kitchen Task Assessment (Kiddy KTA)
• (Baum Edwards, 1993)

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Discussion

• The BRIEF, both parent and teacher forms,
  indicates that children with SCD have more
  problems related to executive functioning in
  everyday life than controls.
• The traditional measures of executive functioning
  shows few differences between the groups.
• The performance-based measure of executive
  functioning, the Kiddy KTA, shows a trend of
  worse performance of children with SCD compared
  to controls.
• Secondary findings
• Children with SCD have significantly more
  literacy problems than controls.
• The Kiddy KTA correlated more highly with the
  BRIEF than the traditional measures of executive
  functioning, indicating greater ecological
  validity.

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Limitations

• Small sample size
• Comparison of subgroups, such as categories of
  CVAs and types of SCD
• Limited age range
• Convenient sample
• Possible researcher bias

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Acknowledgements

• Kristy Rocke
• Dr. Berg and Dr. Edwards
• Dr. DeBaun and the staff at the Hematology clinic
• General Clinical Research Center at St. Louis
  Childrens Hospital
• Adams School and nurse Shaw
• Dr. Hollingsworth
• Gina, Heidi, Natalie, and Ashley for all their
  assistance

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References

• Ashley-Koch, A. Yang, Q., Olney, R. S. (2000).
  Sickle hemoglobin (Hb S) allele and sickle cell
  disease A HuGE review. American Journal of
  Epidemiology, 151(9), 839-845.
• Baum C. Edwards, D. F. (1993). Cognitive
  performance in senile dementia of the Alzheimers
  type The kitchen task assessment. The American
  Journal of Occupational Therapy, 47(5), 431-436.
• Beers, M. H., Fletcher, A. J., Jones, T. V.,
  Porter, R., Berkwits, M., Kaplan, J. L. (Eds.)
  (2005). Sickle cell disease. In The Merck Manual
  of Medical Information- Second Home, Online
  Version (section blood disorders chapter anemia
  topic sickle cell disease). Retrieved June 12,
  2005 from http//www.merck.com/mmhe/sec14/ch172/ch
  172g.html
• Burgess, P. W., Alderman, N., Evans, J., Emslie,
  H., Wilson, B. A. (1998). The ecological
  validity of tests of executive function. Journal
  of International Neuropsychological Society, 4,
  547-558.
• Chaytor, N., Schitter-Edgecombe, M. (2003).
  The ecological validity of neuropsychological
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• Brown, R. T., Davis, P. C., Lambert, R., Hsu, L.,
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• Gioia, G. A., Isquith, P. K., Guy, S. C.,
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• Herron, S., Bacak, S. J., Allsion, K., Debaun,
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  resources required for high-risk students with
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• Hollingshead, A. B. (1975). Four factor index of
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• Katz, N. Hartmann-Maeir, A. (2005).
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References

• National Institutes of Health, Division of Heart,
  Lung, and Blood Institute (2002). The Management
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