title: Costs and utilization of hemophilia A and B patients with and without inhibitors.
abstract: OBJECTIVE
  To evaluate the health system costs among patients with hemophilia A and B with and without inhibitors over 5 years.
  
  
  METHODS
  This was a retrospective, observational study utilizing medical and pharmacy electronic medical records and administrative encounters/claims data tracking US patients between 2006-2011. Patients with diagnosis codes for hemophilia A and B were identified. Patients with inhibitors were characterized by utilization of bypassing agents activated prothrombin complex concentrate or factor VIIa on two or more distinct dates. Severity was classified as mild, moderate, or severe based on laboratory tests of clotting factor.
  
  
  RESULTS
  There were 160 hemophilia A patients and 54 hemophilia B patients identified. From this group, seven were designated as patients with inhibitors (five with hemophilia A and two with hemophilia B). Hemophilia A patients without inhibitors reported 65 (41.9%) as being severe, 19 (12.3%) as moderate, and 71 (45.8%) as mild. Hemophilia B patients without inhibitors reported nine (17.3%) had severe, 13 (25.0%) had moderate, and 30 (57.7%) had mild hemophilia. All patients with inhibitors had been hospitalized in the previous 5 years compared to 64 (41.3%) with hemophilia A without inhibitors and 22 (42.3%) with hemophilia B without inhibitors. The median aggregate cost per year (including factor and health resource use) was $325,780 for patients with inhibitors compared to $98,334 for hemophilia A patients without inhibitors and $23,265 for hemophilia B patients without inhibitors.
  
  
  CONCLUSIONS
  The results suggest that, while the frequency of inhibitors within the hemophilia cohort was low, there was a higher frequency of hospitalizations, and the associated median aggregate costs per year were 3-fold higher than those patients without inhibitors. In contrast, hemophilia B patients experience less severe disease and account for lower aggregate yearly costs compared to either patients with hemophilia A or patients with inhibitors.
authors.affiliation: Strategic Therapeutics, Tucson, AZ, USA, and College of Pharmacy, University of Arizona , Tucson, AZ , USA.
authors.forename: Edward P
authors.initials: EP
authors.lastname: Armstrong
authors.forename: Daniel C
authors.initials: DC
authors.lastname: Malone
authors.forename: Sangeeta
authors.initials: S
authors.lastname: Krishnan
authors.forename: Maj Jacob
authors.initials: MJ
authors.lastname: Wessler
mesh_terms: D000293:Adolescent; D000328:Adult; D001779:Blood Coagulation Factors / Q000191:economics* / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D015942:Factor VIIa / Q000191:economics* / Q000627:therapeutic use; D005102:Health Expenditures / Q000706:statistics & numerical data*; D006467:Hemophilia A / Q000188:drug therapy / Q000191:economics* / Q000276:immunology; D002836:Hemophilia B / Q000191:economics* / Q000276:immunology; D006760:Hospitalization / Q000191:economics / Q000706:statistics & numerical data; D006801:Humans; D007223:Infant; D007345:Insurance Claim Review; D007518:Isoantibodies / Q000276:immunology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012720:Severity of Illness Index; D015599:Trauma Severity Indices; D055815:Young Adult
chemical_list: D001779:Blood Coagulation Factors; D007518:Isoantibodies; C025667:prothrombin complex concentrates; D015942:Factor VIIa
keywords: Healthcare cost; Hemophilia; Hospitalization; Medication adherence