Hydroxyurea use is associated with executive functioning and nonverbal
skills in young children with sickle cell disease (SCD)
Cognitive impairment in children with sickle cell disease (SCD) is
thought to reflect the complex pathophysiology of the disease.
Hydroxyurea (HU) is used in children with SCD to increase fetal
hemoglobin (HF), contributing to a decrease in physical symptoms and to
potential protection against cerebral microvasculopathy. There has been
minimal investigation into the association between HU use and cognition
in this population. This study examined the relationship between HU
status and cognition in young children with SCD. Neuropsychological data
were collected in a prospective study from 38 children with SCD HbSS or
HbS/0 thalassaemia ages 4 to 11 years with no history of overt stroke
or chronic transfusion. Controlling for hemoglobin, children on HU
performed significantly better than children who were not on HU on
measures of attention/executive functioning and nonverbal skills.
Performance on verbal measures was similar between groups. Though not
statistically significant, duration of HU use was positively associated
with better performance in all domains. These results provide
preliminary evidence that treatment with HU may not only reduce physical
symptoms, but may also be protective against cognitive dysfunction in
young children with SCD, particularly in regard to attention/executive
functioning and nonverbal skills. Replication with larger samples and
longitudinal studies are warranted.