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Wednesday 27th September 5pm
June Lloyd Room, Ground Floor, UCL GOS Institute of Child Health, 30 Guildford Street, London WC1N 1EH
Title: Cognition in sickle cell disease: through the ages
Speaker: Professor Fenella Kirkham, UCL GOS Institute of Child Health
Abstract: The Sickle cell disease (SCD) is now the most common inherited condition in the United Kingdom. Overt stroke occurs in 10% of untreated children by the age of 20 years and covert (silent) cerebral infarction detected on MRI also accumulates with age. Compared with controls, meta-analysis has shown reduction in verbal and performance intelligence quotient in children with SCD even if there is no infarction. There may be deterioration in cognitive performance over time but the underlying pathophysiology is poorly understood and includes socio-economic and geographical as well as genetic and medical factors.
Processing speed is a particularly vulnerable domain, with deficits potentially mediating difficulties across other domains. Our data suggests that processing speed is lower in older patients with SCD. The integrity of normal-appearing white matter, using quantitative microstructure parameters from multi-shell diffusion MRI, is related to processing speed.
Poor sleep is common in SCD and the prevalence of sleep-disordered breathing, including snoring, arousals, obstructive sleep apnoea (OSA) and nocturnal desaturation, is higher in SCD than in the general population. Data from the Sickle in Preschool Study (SIPS) and Sleep Asthma cohort (SAC) studies suggest that these disorders impact on cognition, particularly processing speed and other aspects of executive function.
The key question is whether any of the cognitive difficulties are preventable or reversible and whether cognitive endpoints are useful endpoints in randomised controlled trials of treatment. A pilot trial of auto-adjusting continuous positive airways pressure suggested benefit in terms of improved cancellation. We are currently funded for the NIHR funded Prevention of Morbidity in Sickle Cell Anaemia Phase II RCT of auto-adjusting CPAP (POMS2B) and the Action Medical Research funded trial of Montelukast (SMILES), both with processing speed endpoints.
All welcome to attend
Contact: cdcn@ucl.ac.uk