Neuropsychiatric Impairment among Patients with Sickle Cell Anemia

Sickle cell anemia (SCA) is an inherited disorder of hemoglobin. Each year over 150,000 children in Africa are born with SCA. Cognitive impairment is a common complication of SCA. This case-control study evaluated cognitive function in 41 adolescents and adults with SCA and an equal number of healthy demographically matched controls using the community screening interview for dementia (CSID), Trail Making Test A (TMTA), Saint Louis University Mental Status examination (SLUMS), and Mini Mental State Examination (MMSE). Mood (anxiety and depression) was assessed using the Hospital Anxiety and Depression Scale (HADS). The controls had better total scores on all screening instruments; however, the difference between their performance and that of the SCA adults was not statistically significant; SLUM (p = 0.179), TMTA (p = 0.359), MMSE (p = 0.241), and CSID (0.494). On specific task, the controls performed significantly better based on SLUM (naming), p = 0.016; SLUM (repetition), p = 0.015; SLUM (recall), p = 0.003; and CSID (language expression), p = 0.001. The systolic blood pressure (SPB) was inversely correlated with the MMSE scores (p = 0.009). In addition, there was direct linear correlation between the creatinine levels and the MMSE scores (p = 0.009). The proportion of SCA patients compared with the controls that had abnormal mood were anxiety (7.3% vs. 4.9%), borderline anxiety (17.1% vs. 4.9%), depression (2.4% vs. 2.4%), and borderline depression (14.6% vs. 2.4%). SCA was associated with an increased prevalence of cognitive impairment in adults when compared to controls. SCA is associated with a higher proportion of mood abnormalities.