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
Kral MC, Brown RT, Hynd GW. Neuropsychological aspects of pediatric sickle cell disease. Neuropsychol Rev. 2001;11(4):179–96.
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.
Quinn CT, Dowling MM. Cerebral tissue hemoglobin saturation in children with sickle cell disease. Pediatr Blood Cancer. 2012;59(5):881–7.
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.
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.
Ohene-Frempong K. Stroke in sickle cell disease: demographic, clinical, and therapeutic considerations. Semin Hematol. 1991;28:213–9.
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.
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.
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.
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.
Schatz J, McClellan CB. Sickle cell disease as a neurodevelopmental disorder. Ment Retard Dev Disabil Res Rev. 2006;12:200–7.
Individuals With Disabilities Education Act (IDEA). Vol 20 U.S.C. § 1400. 2004.
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.
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.
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.
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.
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.
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.
DeBaun MR, Schatz J, Siegel MJ, et al. Cognitive screening examinations for silent cerebral infarcts in sickle cell disease. Neurology. 1998;50:1678–82.
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.
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.
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.
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.
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.
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.
Kolva E, Rosenfeld B, Brescia R, Comfort C. Assessing decision-making capacity at end of life. Pediatrics. 2014;36:392–7.
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.
Quinn CT, Roger ZR, McCavit TL, Buchanan GR. Improved survival of children and adolescents with sickle cell disease. Blood. 2010;115:3447–52.
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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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