title: Headache and migraine in children with sickle cell disease are associated with lower hemoglobin and higher pain event rates but not silent cerebral infarction.
abstract: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors: Department of Pediatrics and Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, TX.
authors: Michael M
authors: MM
authors: Dowling
authors: Department of Neurology, Division of Pediatric and Developmental Neurology, St Louis Children's Hospital and Washington University School of Medicine, St Louis, MO.
authors: Michael J
authors: MJ
authors: Noetzel
authors: Rodeghier Consultants, Chicago, IL.
authors: Mark J
authors: MJ
authors: Rodeghier
authors: Division of Hematology, Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
authors: Charles T
authors: CT
authors: Quinn
authors: National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.
authors: Deborah G
authors: DG
authors: Hirtz
authors: Department of Neurology and Pediatrics, The Children's Hospital of Philadelphia and Perelman School of Medicine of the University of Pennsylvania, Philadelphia, PA.
authors: Rebecca N
authors: RN
authors: Ichord
authors: Department of Neurology and Pediatrics, The Children's Hospital of Philadelphia and Perelman School of Medicine of the University of Pennsylvania, Philadelphia, PA.
authors: Janet L
authors: JL
authors: Kwiatkowski
authors: Division of Child Neurology, Ohio State College of Medicine, Columbus, OH.
authors: E Steven
authors: ES
authors: Roach
authors: Neurosciences Unit, Institute of Child Health, University College London, London, United Kingdom.
authors: Fenella J
authors: FJ
authors: Kirkham
authors: Division of Pediatric Hematology, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD.
authors: James F
authors: JF
authors: Casella
authors: Department of Pediatrics Vanderbilt-Meharry-Matthew Walker Center of Excellence in Sickle Cell Disease, Vanderbilt University School of Medicine, Nashville, TN. Electronic address: m.debaun@vanderbilt.edu.
authors: Michael R
authors: MR
authors: DeBaun
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications* / Q000628:therapy; D015415:Biomarkers / Q000097:blood; D001803:Blood Transfusion; D002544:Cerebral Infarction / Q000175:diagnosis / Q000209:etiology* / Q000517:prevention & control; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D006261:Headache / Q000209:etiology*; D006454:Hemoglobins / Q000378:metabolism*; D006801:Humans; D016015:Logistic Models; D008279:Magnetic Resonance Imaging; D008297:Male; D008881:Migraine Disorders / Q000209:etiology*; D015999:Multivariate Analysis; D010146:Pain / Q000209:etiology; D012008:Recurrence; D012307:Risk Factors; D012720:Severity of Illness Index
chemical_list: D015415:Biomarkers; D006454:Hemoglobins