title: Economic costs associated with acute attacks and long-term management of hereditary angioedema.
abstract: BACKGROUND
  Hereditary angioedema (HAE) is a rare autosomal dominant disorder characterized by recurrent acute attacks of swelling of the larynx, abdomen, and periphery.
  
  
  OBJECTIVE
  To assess the economic burden associated with acute attacks and long-term management of HAE.
  
  
  METHODS
  Burden was assessed via a Web-based survey of HAE patients (> or = 18 years old) that solicited information on attack characterization, short-term treatment, long-term disease management, impact on work, and patient costs. A standardized instrument, the Work Productivity and Activity Impairment questionnaire, was included to assess impact on work productivity. Standard medical costs and US average wage costs were assigned to survey items to assess direct medical and indirect costs, respectively.
  
  
  RESULTS
  Total annual per-patient costs are estimated at $42,000 for the average HAE patient, with costs totaling $14,000 for patients with mild attacks, $27,000 for patients with moderate attacks, and $96,000 for patients with severe attacks. Hospital costs account for 67% of direct medical costs. Respondents reported high rates of missed work, lost productivity, and lost income, contributing to indirect costs totaling $16,000 annually for the average patient. Almost all costs increase with disease severity, although the distribution varies with severity: indirect costs account for 75% of costs for patients with mild attacks, whereas emergency department and hospital costs account for 68% of costs for patients with severe attacks.
  
  
  CONCLUSIONS
  HAE results in considerable economic burden to patients, payers, and society in terms of direct medical and indirect costs and compounds the substantial humanistic burdens, which will be reported separately.
authors.affiliation: Massachusetts General Hospital Institute of Health Professions, Boston, Massachusetts 02129, USA. dwilson@mghihp.edu
authors.forename: David A
authors.initials: DA
authors.lastname: Wilson
authors.forename: Konrad
authors.initials: K
authors.lastname: Bork
authors.forename: Elizabeth P
authors.initials: EP
authors.lastname: Shea
authors.forename: Anne M
authors.initials: AM
authors.lastname: Rentz
authors.forename: Marc B
authors.initials: MB
authors.lastname: Blaustein
authors.forename: William E
authors.initials: WE
authors.lastname: Pullman
mesh_terms: D000208:Acute Disease / Q000191:economics / Q000628:therapy; D000328:Adult; D054179:Angioedemas, Hereditary / Q000175:diagnosis / Q000191:economics* / Q000628:therapy; D017281:Cost of Illness; D016527:Drug Costs / Q000706:statistics & numerical data; D004632:Emergency Medical Services / Q000191:economics / Q000706:statistics & numerical data; D005260:Female; D017048:Health Care Costs / Q000706:statistics & numerical data*; D019538:Health Care Surveys; D017721:Hospital Costs / Q000706:statistics & numerical data; D006760:Hospitalization / Q000191:economics / Q000706:statistics & numerical data; D006801:Humans; D008297:Male; D011795:Surveys and Questionnaires