The prevalence of frequent headache in children with sickle cell disease (SCD) compared to that of control subjects without known sickle cell trait, and the cause of the headaches were studied at the Children’s Hospital of Philadelphia, PA, and Duke University Medical Center. Patients and controls were ages 6 to 21 years. Thirty two per cent of 241 children with SCD and 27% of 141 black control subjects reported having headaches at least weekly, a prevalence not significantly different (P-0.27). Mean age of those with frequent headache was 14.2 +/- 4.3 years for SCD patients and 15.9 +/- 3.2 for controls (P=0.03). In children <13 years old, prevalence of frequent headache was 24% for SCD and 9.7% for controls (P=0.013). In both groups, headache was more common with increasing age (P<0.001). Prevalence was similar in various SCD genotypes (SSD-SS, SCD-SC, SCD-SB-thalassemia). Headaches meeting IHS criteria for migraine occurred in 22.1% of patients with SCD and in 21.1% controls. Children with frequent headache were more likely to report specific SCD-related factors such as frequent vaso-occlusive pain episodes (VOE) that occurred concurrently with headache: obstructive sleep apnea (OSA) that may cause headaches; and cerebral vessel stenosis and vasculopathy detected by MR angiography. Silent cerebral infarction or stroke was not associated with headache. Abnormal transcranial Doppler (TCD) together with abnormal MRA were associated with frequent headache. Screening with TCD may be helpful in the evaluation of frequent headache and the risk of stroke. 
COMMENT. The overall prevalence of headaches in children with SCD is not greater than that in the general population. Younger children with SCD have headaches more frequently than control subjects without sickle cell trait. SCD-specific factors such as vaso-occlusive episodes and cerebral vessel stenosis may contribute to the headaches.