Neurocognitive functioning in symptomatic adults with sickle cell disease: A description and comparison with unaffected siblings.
Staci Martin, Marie Claire Roderick, Cristina Abel and 5 others
PMID 30924397WHAT IT FOUND
Symptomatic adults with sickle cell disease showed slower processing speed than their healthy siblings.
While formal cognitive tests were normal, patients reported more executive dysfunction. These self-reported difficulties were linked to pain and depressive symptoms, not just the disease itself.
Key findings
01Patients scored significantly lower on the Processing Speed Index compared to their sibling donors.
02Patients reported significantly more executive dysfunction on self-rating questionnaires than their siblings.
03Differences in self-reported executive functioning between patients and siblings were no longer significant after controlling for depressive symptoms.
STILL TO COME
How it was doneWhat they found
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What it does not show
Small sample size (30 pairs) limited the ability to detect differences. Participants were recruited from a specialized NIH transplant protocol, likely representing a higher-functioning subset of the SCD population. Most patients were not working full-time, making it difficult to assess the real-world occupational impact of the cognitive findings. The study was cross-sectional, so it cannot determine if cognitive deficits caused depression/pain or vice versa.
Declared interests
The authors declare no conflicts of interest.
The easy way to misread this
Do not assume that objective cognitive test scores capture the functional difficulties these patients experience. Patients performed normally on tests but reported significant executive dysfunction, which was largely explained by depressive symptoms and pain rather than direct neurological damage from SCD.