ORIGINAL ARTICLE Treatment The relative burden of haemophilia A and the impact of target joint development on health-related quality of life: results from the ADVATE Post-Authorization Safety Surveillance (PASS) study R. KLAMROTH,* H. POLLMANN,  C. HERMANS, à A. FARADJI,§ A. S. YARLAS, – J. D EPSTEIN** and B. M EWENSTEIN** *Vivantes – Klinikum in Friedrichhain, Klinik fuer Innere Medizin, Haemophiliezentrum, Berlin, Germany;  Institut fu ¨r Thrombophilie und Ha ¨ mostaseologie, Ambulanzzentrum an der Raphaelsklinik, Mu ¨ nster, Germany; àClinique Universitaires Saint-Luc, Haemostasis Department, Bruxelles, Belgium; §Haemophilia Regional Centre, Ho ˆ pital de Hautepierre, Strasbourg, France; –QualityMetric, Lincoln, RI, USA; and **Baxter BioScience, Westlake Village, CA, USA Summary. Studies with haemophilia A (HA) patients have shown burden in health-related quality of life (HRQOL) when compared with general population norms. In the current study, HA patients’ SF-36v2 health survey scores were compared with general population norms and to patients with other chronic conditions. The impact of target joints (TJs) on HRQOL was also examined. The sample was a subset of HA patients enrolled in the Post-Authorization Safety Surveillance (PASS) programme: a prospective open- label study in which ADVATE [Antihaemophilic Factor (Recombinant), Plasma/Albumin-Free Method] was prescribed. A total of 205 patients who were ‡18 years old and had SF-36v2 baseline scores were selected for this study. To measure the burden of HA on HRQOL, manova analyses compared these SF-36v2 scores to age- and gender-matched general population US and EU norms and to patients from other chronic condition groups. manova and correlational analyses examined the relations among TJ, age and SF-36v2 scores. Comparisons with general population norms confirm that HA negatively impacts physical, but not mental, HRQOL. Comparison with other chronic conditions shows the physical burden of HA is greater than for chronic back pain but similar to diabetes and rheuma- toid arthritis, while the mental burden of HA is less than for all three patient groups. The presence of TJs was negatively associated with physical HRQOL, although this association was much larger for older patients (45+ years) than for younger ones. Physical, but not mental, HRQOL is diminished in HA patients. Target joints are associated with lower physical HRQOL, although this effect is moderated by age. Keywords: burden analysis, haemophilia A, health related quality of life, target joint Introduction Approximately one in 10 000 people are born with haemophilia A (HA), making it an extremely rare disease (WFH 2005 guidelines [1]). Advances in treat- ment, from the development of factor VIII (FVIII) concentrates to the introduction of plasma/albumin-free recombinant FVIII products that eliminate the risk of pathogen transmission, have allowed average life expectancy to increase from 7.8 years in 1939 to over 70 years in 2001 [2–4]. With mortality risks in HA significantly reduced, issues regarding patient quality of life can now be pursued. Several published studies have focused on the impact HA has on patient health-related quality of life (HRQOL) [5–11]. These studies have generally at- tempted to demonstrate the burden in HRQOL because of HA by comparing scores on generic HRQOL instruments between haemophilia patients and norms or control samples matched by age, gender and nation- ality. Generally, these studies have indicated that the Correspondence: Robert Klamroth, Vivantes – Klinikum in Friedrichhain Klinik fuer Innere Medizin Haemophiliezentrum, Landsberger Allee 49, Berlin 10249, Germany. Tel.: +4930 130 23 1623; fax: +4930 130 23 1313; e-mail: robert.klamroth@vivantes.de Accepted for revision 3 September 2010 Haemophilia (2011), 17, 412–421 DOI: 10.1111/j.1365-2516.2010.02435.x 412 Ó 2011 Blackwell Publishing Ltd