Pediatric Case of Sickle Cell Disease

  • Melissa GerstleEmail author
  • Katherine Baum
  • Charles T. Quinn
  • Dean W. BeebeEmail author


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.


Sickle cell disease Pediatric Cognitive Neuropsychology Medical/neurological assessment 


  1. 1.
    Kral MC, Brown RT, Hynd GW. Neuropsychological aspects of pediatric sickle cell disease. Neuropsychol Rev. 2001;11(4):179–96.CrossRefGoogle Scholar
  2. 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.CrossRefGoogle Scholar
  3. 3.
    Quinn CT, Dowling MM. Cerebral tissue hemoglobin saturation in children with sickle cell disease. Pediatr Blood Cancer. 2012;59(5):881–7.CrossRefGoogle Scholar
  4. 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. 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.CrossRefGoogle Scholar
  6. 6.
    Ohene-Frempong K. Stroke in sickle cell disease: demographic, clinical, and therapeutic considerations. Semin Hematol. 1991;28:213–9.PubMedGoogle Scholar
  7. 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.CrossRefGoogle Scholar
  8. 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.CrossRefGoogle Scholar
  9. 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.CrossRefGoogle Scholar
  10. 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.CrossRefGoogle Scholar
  11. 11.
    Schatz J, McClellan CB. Sickle cell disease as a neurodevelopmental disorder. Ment Retard Dev Disabil Res Rev. 2006;12:200–7.CrossRefGoogle Scholar
  12. 12.
    Individuals With Disabilities Education Act (IDEA). Vol 20 U.S.C. § 1400. 2004.Google Scholar
  13. 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.PubMedGoogle Scholar
  14. 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.CrossRefGoogle Scholar
  15. 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.CrossRefGoogle Scholar
  16. 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.CrossRefGoogle Scholar
  17. 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. 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.CrossRefGoogle Scholar
  19. 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.CrossRefGoogle Scholar
  20. 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.CrossRefGoogle Scholar
  21. 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.CrossRefGoogle Scholar
  22. 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.CrossRefGoogle Scholar
  23. 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.CrossRefGoogle Scholar
  24. 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.CrossRefGoogle Scholar
  25. 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.CrossRefGoogle Scholar
  26. 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. 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.CrossRefGoogle Scholar
  28. 28.
    Quinn CT, Roger ZR, McCavit TL, Buchanan GR. Improved survival of children and adolescents with sickle cell disease. Blood. 2010;115:3447–52.CrossRefGoogle Scholar

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© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children’s Hospital Medical Center, Department of PediatricsUniversity of Cincinnati College of MedicineCincinnatiUSA
  2. 2.Division of Hematology, Cincinnati Children’s Hospital Medical Center, Department of PediatricsUniversity of Cincinnati College of MedicineCincinnatiUSA

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