OBJECTIVES: Recurrent bleeding among severe hemophilia A children can lead to disability and lower health-related quality of life (HRQOL). Little is known about how many bleeds can be endured before patients report an impact on health outcomes. The objective was to assess health outcomes of pediatric patients reporting a zero annual bleed rate (ABR) to identify the impact of higher ABRs on outcomes. METHODS: This cross-sectional survey of parents of severe hemophilia A patients aged 2-17 was conducted in Argentina, Chile, Colombia, Mexico and Panama.  Eligible, consenting patients completed a questionnaire: from October-November 2009 (Argentina), June-August 2011 (Chile, Colombia, Mexico) and September-October 2012 (Panama).  HRQOL was measured by the Pediatric Quality of Life Inventory (PedsQL).  ABR, target joints and school days missed were also assessed. RESULTS: 211 parents of severe hemophilia A children completed the survey. Compared to patients with a 0 ABR who reported a mean PedsQL Total score of 76.6, patients with ABR categories of: 3-4, 5-10, 11-20, 21-30, 31-50, 51 or more showed significantly worse mean PedsQL Total scores of: 66.4, 63.5, 67.4, 62.5, 62.4 and 59.9 respectively (all p<0.05).  Similarly, compared to patients with 0 ABR who reported  a mean number of target joints of 0.69, patients with higher ABR categories showed significantly higher mean target joints:  1.49, 2.57, 3.42, 3.47, 3.74, 4.67 (all p<.05).  Differences in missed days from school showed the same significant trend when comparing 0 ABR to ABR categories of 3-4 and beyond. There were no significant differences between patients with zero compared to 1-2 ABR on these health outcomes.   CONCLUSIONS: This analysis suggests that even 3-4 bleeds/year may have a negative impact on a patient’s joint health, missed school days and HRQOL. Efforts to maintain a 0 ABR among pediatric patients with severe hemophilia A may help ensure optimal outcomes.