Researchers recently found that the Montreal Cognitive Assessment (MoCA) score is a promising option for screening cognitive impairment in adults with sickle cell disease (SCD) and published their findings in the British Journal of Haematology.

Early et al enrolled 49 adults with SCD who received care at the Johns Hopkins Sickle Cell Center for Adults in Baltimore, Maryland. The purposes of the investigation were to detect whether MoCA scores correlate with literacy and health literacy levels in adults with SCD; to identify clinical factors linked with poor performance on the MoCA, literacy, and health literacy assessments; and to characterize the patients with high educational attainment who obtain abnormal cognitive testing scores.

Median MoCA scores were correlated with the validated Wide Range Achievement Test 4 and the Test of Functional Health Literacy in Adults. Both are validated tests of health literacy that have clinically meaningful cognitive domains modifiable by rehabilitation and mitigated by changes in clinical practice.

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The team also carried out a retrospective review of the participants’ electronic medical records including acute chest syndrome (ACS). Prior history of ACS has not been previously explored in relation to cognitive performance, but these results suggest it might indicate increased disease severity.

“In our sample, there was no significant association between history of ACS and cognitive performance. However, more than 60% of participants had a history of ACS; operationalizing ACS differently, such as identifying those with ACS in the past 2 years, might have more predictive power,” the authors said.

Additionally, patients with SCD with high educational attainment were found to be more vulnerable to deleterious cognitive disease processes. “In our sample, 18 participants with post-secondary education, including 9 college graduates, had abnormal scores on at least 1 cognitive test,” the authors said.

Adults with SCD experience hemolysis, inflammation, and vascular occlusion, which can cause end-organ injury. Simultaneously, the brain can also suffer from anemia, cerebrovascular accidents, vaso-occlusive events, decreased cerebrovascular reserve, and arteriopathies. Affected individuals may have clinical or subclinical cognitive damage, negatively affecting educational attainment, employment status, quality of life, and health behaviors. Rehabilitation and prevention of further damage rely on the early identification of cognitive impairment.


Early M, Linton E, Bosch A, et al. The Montreal cognitive assessment as a cognitive screening tool in sickle cell disease: associations with clinically significant cognitive domains. Br J Haematol. Published online April 6, 2022. doi:10.1111/bjh.18188