Stroke burden and functional impacts in adults with sickle cell disease

Abstract

Stroke in sickle cell disease (SCD) has been well characterized in children, but data in adults remain insufficient, particularly regarding long-term functional consequences. The objective was to determine lifetime prevalence of symptomatic stroke, followed by characterization of stroke type, aetiology, treatments and functional status at last follow-up. We retrospectively reviewed adults (≥18 years) with any SCD phenotype followed at a tertiary centre from 2011 to 2023. Functional status was assessed using the Montreal Cognitive Assessment (MoCA) and Modified Rankin Scale (mRS). Among 454 adults with all major phenotypes of SCD, median age was 32 years [range 18-79] and 261 (57.5%) were women. At last follow-up, 21 individuals (4.6%) had a confirmed history of symptomatic stroke (median age at stroke onset of 42 years [range 4-68]), including 14 (3.1%) with cerebral infarction (median age 32 years [4-68]) and 7 (1.5%) with intracranial haemorrhage (median age 45 years [20-65]). Stroke was associated with marked long-term impairment, represented by lower MoCA (20.6 (±1.3) vs. 26.1 (±0.2), p < 0.001) and higher mRS (2 [1-3] vs. 0 [0-1], p < 0.001). These findings fill a critical evidence gap and underscore the urgency of targeted prevention and intervention strategies in this high-risk population.

Keywords: Sickle cell disease; cognition; functional status; neurovascular injury; stroke; stroke aetiologies.