Headache and migraine in children with sickle cell disease are associated with lower hemoglobin and higher pain event rates but not silent cerebral infarction

Michael M. Dowling, Michael J. Noetzel, Mark J. Rodeghier, Charles T. Quinn, Deborah G. Hirtz, Rebecca N. Ichord, Janet L. Kwiatkowski, E. Steven Roach, Fenella J. Kirkham, James F. Casella, Michael R. Debaun

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20 Scopus citations


Objective To identify risk factors for headache and migraine in children with sickle cell disease and test the hypothesis that either or both are independently associated with silent cerebral infarcts. Study design In this cross-sectional study, we evaluated the health history, laboratory values, and brain magnetic resonance imaging findings of participants with sickle cell disease (hemoglobinSS or hemoglobinSβ°-thalassemia) with no history of overt stroke or seizures. Participants characterized headache severity and quality. Migraine was defined by International Headache Society criteria modified for increased sensitivity in children. Neuroradiology and neurology committees adjudicated the presence of silent cerebral infarction by review of magnetic resonance imaging and standardized examination by pediatric neurologists. Results The cohort included 872 children (51.1% males), ranging in age from 5 to 15 years (mean age, 9.1 years). Of these children, 317 (36.4%) reported recurrent headaches, and 132 (15.1%) reported migraines. In multivariable logistic regression analyses, both were associated with lower steady-state hemoglobin (P =.01 for headaches; P <.01 for migraines) and higher pain rate (P <.01 for headaches; P <.01 for migraines), defined as the number of admissions requiring opioids in the previous 3 years. The presence of silent cerebral infarction was not associated with recurrent headaches or migraines. Only 1.9% (6 of 317) of children with recurrent headaches received medication for headache prophylaxis. Conclusion Recurrent headaches and migraines are common and undertreated in children with sickle cell disease. Low hemoglobin levels and high pain rates are associated with recurrent headaches and migraines; whereas, silent cerebral infarction is not.

Original languageEnglish (US)
Pages (from-to)1175-1180.e4
JournalJournal of Pediatrics
Issue number5
StatePublished - Jan 1 2014



  • ACS
  • Acute chest syndrome
  • CBF
  • Cerebral blood flow
  • Hemoglobin
  • Hgb
  • MRI
  • Magnetic resonance imaging
  • SCD
  • SIT
  • Sickle cell disease
  • Silent Cerebral Infarct Transfusion
  • TCD
  • Transcranial Doppler ultrasound
  • VIF
  • Variance inflation factor

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Dowling, M. M., Noetzel, M. J., Rodeghier, M. J., Quinn, C. T., Hirtz, D. G., Ichord, R. N., Kwiatkowski, J. L., Roach, E. S., Kirkham, F. J., Casella, J. F., & Debaun, M. R. (2014). Headache and migraine in children with sickle cell disease are associated with lower hemoglobin and higher pain event rates but not silent cerebral infarction. Journal of Pediatrics, 164(5), 1175-1180.e4. https://doi.org/10.1016/j.jpeds.2014.01.001