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Pediatric Case of Sickle Cell Disease

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Physician's Field Guide to Neuropsychology

Abstract

Sickle Cell: Children with sickle cell disease (SCD) often have cognitive, behavioral, and mood-related difficulties, in part due to the effect of their medical condition on the central nervous system. They are at high risk for neurological injury and insult, with as many as one-third having neurological complications. This chapter details a pediatric case of SCD, assessed through repeated neuropsychological evaluations. It highlights the unique challenges of assessing a “moving target”—the interaction between a protean chronic illness and the developing brain.

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References

  1. Kral MC, Brown RT, Hynd GW. Neuropsychological aspects of pediatric sickle cell disease. Neuropsychol Rev. 2001;11(4):179–96.

    Article  CAS  Google Scholar 

  2. Dowling MM, Quinn CT, Plumb P, et al. Acute silent cerebral ischemia and infarction during acute anemia in children with and without sickle cell disease. Blood. 2012;120(19):3891–7.

    Article  CAS  Google Scholar 

  3. Quinn CT, Dowling MM. Cerebral tissue hemoglobin saturation in children with sickle cell disease. Pediatr Blood Cancer. 2012;59(5):881–7.

    Article  Google Scholar 

  4. Schatz J, Puffer ES. Neuropsychological aspects of sickle cell disease. In: Brown RT, editor. Comprehensive handbook of childhood cancer and sickle cell disease: a biopsychosocial approach. New York: Oxford University Press; 2006. p. 449–70.

    Google Scholar 

  5. King AA, Strouse JJ, Rodeghier MJ, et al. Parent education and biologic factors influence on cognition in sickle cell anemia. Am J Hematol. 2014;89(2):162–7.

    Article  Google Scholar 

  6. Ohene-Frempong K. Stroke in sickle cell disease: demographic, clinical, and therapeutic considerations. Semin Hematol. 1991;28:213–9.

    CAS  PubMed  Google Scholar 

  7. Solomou E, Kraniotis P, Kourakli A, Petsas T. Extent of silent cerebral infarcts in adult sickle-cell disease patients on magnetic resonance imaging: Is there a correlation with the clinical severity of disease? Hematol Rep. 2013;5(1):8–12.

    Article  Google Scholar 

  8. Brown RT, Buchanan I, Doepke K, Eckman JR, Baldwin K, Goonan B. Cognitive and academic functioning in children with sickle-cell disease. J Clin Child Psychol. 1993;22:207–18.

    Article  Google Scholar 

  9. White DA, Moinuddin A, McKinstry RC, Noetzel M, Armstrong M, DeBaun M. Cognitive screening for silent cerebral infarction in children with sickle cell disease. J Pediatr Hematol Oncol. 2006;28(3):166–9.

    Article  Google Scholar 

  10. Schoenberg MR, Scott JG. The neuropsychology referral and answers to referral questions. In: Schoenberg MR, Scott JG, editors. The Little Black book of neuropsychology. New York: Springer Press; 2011. p. 1–37.

    Chapter  Google Scholar 

  11. Schatz J, McClellan CB. Sickle cell disease as a neurodevelopmental disorder. Ment Retard Dev Disabil Res Rev. 2006;12:200–7.

    Article  Google Scholar 

  12. Individuals With Disabilities Education Act (IDEA). Vol 20 U.S.C. § 1400. 2004.

    Google Scholar 

  13. Ohene-Frempong K, Weiner SJ, Sleeper LA, Miller ST, Embury S, Moohr JW. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91(1):288–94.

    CAS  PubMed  Google Scholar 

  14. Schatz J, Finke RL, Kellett JM, JH K. Cognitive functioning in children with sickle cell disease: a meta-analysis. J Pediatr Psychol. 2002;27(8):739–48.

    Article  Google Scholar 

  15. Bernaudin F, Verlhac S, Fréard F, et al. Multicenter prospective study of children with sickle cell disease: Radiographic and psychometric correlation. J Child Neurol. 2000;15:333–43.

    Article  CAS  Google Scholar 

  16. Wasserman AL, Wilimas JA, Fairclough DL, Mulhern RK. W W. Subtle neuropsychological deficits in children with sickle cell disease. Am J Pediatr Hematol Oncol. 1991;13:14–20.

    Article  CAS  Google Scholar 

  17. Steen RG, Miles MA, Helton KJ, et al. Cognitive impairment in children with hemoglobin SS sickle cell disease: relationship to MR imaging findings and hematocrit. Am J Radiol. 2003;24:382–9.

    Google Scholar 

  18. Thompson RJ, Armstrong FD, Link CL, Pegelow CH, Moser F, Wang WC. A prospective study of the relationship over time of behavior problems, intellectual functioning, and family functioning in children with sickle cell disease: a report from the cooperative study of sickle cell disease. J Pediatr Psychol. 2003;28:59–65.

    Article  Google Scholar 

  19. DeBaun MR, Schatz J, Siegel MJ, et al. Cognitive screening examinations for silent cerebral infarcts in sickle cell disease. Neurology. 1998;50:1678–82.

    Article  CAS  Google Scholar 

  20. Wang W, Enos L, Gallagher D, et al. Neuropsychologic performance in school-aged children with sickle cell disease: a report from the Cooperative Study of Sickle Cell Disease. J Pediatr. 2001;139:391–7.

    Article  CAS  Google Scholar 

  21. Berkelhammer LD, Williamson AL, Sanford SD, et al. Neurocognitive sequelae of pediatric sickle cell disease: a review of the literature. Child Neuropsychol. 2007;13:120–31.

    Article  Google Scholar 

  22. Wills KE, Nelson SC, Hennessy J, et al. Transition planning for youth with sickle cell disease: embedding neuropsychological assessment into comprehensive care. Pediatrics. 2010;126:S151–9.

    Article  Google Scholar 

  23. Jordan L, Swerdlow P, Coates TD. Systematic review of transition from adolescent to adult care in patients with sickle cell disease. J Pediatr Hematol Oncol. 2013;35:165–9.

    Article  Google Scholar 

  24. Epping AS, Myrvik MP, Newby RF, Panepinto JA, Brandow AM, Scott JP. Academic attainment findings in children with sickle cell disease. J Sch Health. 2013;83(8):548–53.

    Article  Google Scholar 

  25. King AA, Rodeghier MG, Panepinto JA, et al. Silent cerebral infarction, income, and grade retention among students with sickle cell anemia. Am J Hematol. 2014;89(10):E188–92.

    Article  Google Scholar 

  26. Kolva E, Rosenfeld B, Brescia R, Comfort C. Assessing decision-making capacity at end of life. Pediatrics. 2014;36:392–7.

    Google Scholar 

  27. Crosby LE, Quinn CT, KA K. A biopsychosocial model for the management of patients with sickle-cell disease transitioning to adult medical care. Adv Ther. 2015;32(4):293–305.

    Article  CAS  Google Scholar 

  28. Quinn CT, Roger ZR, McCavit TL, Buchanan GR. Improved survival of children and adolescents with sickle cell disease. Blood. 2010;115:3447–52.

    Article  CAS  Google Scholar 

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Correspondence to Melissa Gerstle or Dean W. Beebe .

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Gerstle, M., Baum, K., Quinn, C.T., Beebe, D.W. (2019). Pediatric Case of Sickle Cell Disease. In: Sanders, K. (eds) Physician's Field Guide to Neuropsychology. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-8722-1_5

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  • DOI: https://doi.org/10.1007/978-1-4939-8722-1_5

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4939-8720-7

  • Online ISBN: 978-1-4939-8722-1

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