Chronic brain damage in sickle cell disease and its relation with quality of life.
OBJECTIVE
Sickle cell anaemia causes progressive organ damage. The objective is to describe school performance of patients with sickle cell anaemia and their clinical parameters and quality of life that may have an influence. The hypothesis is that if school alterations occur without other objective data, additional factors must be present besides the disease itself.


METHODS
Transversal study performed in November 2015 considering analytical variables, complications and neuroradiological images of children with sickle cell anaemia, and family survey on school performance and quality of life.


RESULTS
Median age was 6.8 years and 78% were diagnosed at birth. Sixty patients were included. School performance was altered in 51% of cases and was related to nocturnal hypoxemia. Acute stroke incidence was 6.7%. Transcranial ultrasound was abnormal in 4% of cases and magnetic resonance imaging in 16% of cases. Quality of life showed pathological findings in all areas and the low values increased proportionally in older ages. The stroke affected the physical and social sphere, and lung disease affected the physical and emotional spheres.


CONCLUSIONS
Poor school performance affects half of the patients and it is related to nocturnal hypoxemia, although other socio-cultural factors may have an influence. Quality of life is affected in most of these cases independently of academic results. The absence of alterations in neuroimaging or the apparent lack of severe clinical parameters do not mean that quality of life and schooling are normal.
Hematología Pediátrica, Hospital General Universitario Gregorio Marañón, Facultad de Medicina, Universidad Complutense de Madrid, Madrid, España. Electronic address: elena.cela@salud.madrid.org.
Elena
E
Cela
Estudiante de pregrado de Medicina, Hospital General Universitario Gregorio Marañón, Facultad de Medicina, Universidad Complutense de Madrid, Madrid, España.
Ana G
AG
Vélez
Servicio de Radiodiagnóstico Pediátrico, Hospital General Universitario Gregorio Marañón, Facultad de Medicina, Universidad Complutense de Madrid, Madrid, España.
Alejandra
A
Aguado
Instituto de Investigación Sanitaria Gregorio Marañón, Hospital General Universitario Gregorio Marañón, Facultad de Medicina, Universidad Complutense de Madrid, Madrid, España.
Gabriela
G
Medín
Instituto de Investigación Sanitaria Gregorio Marañón, Hospital General Universitario Gregorio Marañón, Facultad de Medicina, Universidad Complutense de Madrid, Madrid, España.
José M
JM
Bellón
Hematología Pediátrica, Hospital General Universitario Gregorio Marañón, Facultad de Medicina, Universidad Complutense de Madrid, Madrid, España.
Cristina
C
Beléndez
D000073978:Academic Performance / Q000523:psychology / Q000706:statistics & numerical data; D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000523:psychology; D001925:Brain Damage, Chronic / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000523:psychology; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D006305:Health Status Indicators; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D059906:Neuroimaging; D011788:Quality of Life / Q000523:psychology; D020521:Stroke / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000523:psychology; D014463:Ultrasonography
Anemia de células falciformes; Calidad de vida; Neurocognitive disorders; Neuroimagen; Neuroimaging; Quality of life; Sickle cell anaemia; Trastornos neurocognitivos