SMILES

Study Of Montelukast In Children With Sickle Cell Disease

Young children with sickle cell anaemia have cognitive difficulties, specifically involving slowed processing speed. This appears to be related to the vulnerability of the deep white matter of the brain to injury related to low oxygen saturation.

Oxygen saturation often falls during sleep, especially if there is airway obstruction related to the size of the adenoids in the nose as well as the tonsils, clinically detected as snoring. Montelukast, a drug widely used in asthma in young children, appears to reduce the size of the adenoids in children who do not have sickle cell anaemia but we do not know whether that improves processing speed.

Our project involves a randomised controlled trial of Montelukast in children with sickle cell anaemia. The primary endpoints are measures of processing speed (NIH toolbox and Cancellation). Secondary endpoints include brain magnetic resonance imaging of the adenoids and the brain, as well as questionnaires on sleep and pain.