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Aim: Quality of life (QOL) in haemophilia involves both physical and psychological issues. This study was performed to determine parents' perceptions of the QOL of their sons with haemophilia and compare this to their children's own assessment.Methods: In this study, we used the Haemo-QoL questionnaire for haemophilia, to compare the responses of 22 parents to the responses of their children within the age groups 4-7, 8-12 and 13-16 and also for the severity groups mild versus moderate-severe.Results: In the 4-7 age group, the children considered their QOL to be less than that estimated by their parents for a number of areas, while in the 8-12 age group the parents considered the QOL for their children to be lower than that estimated by the children themselves. In the 13-16 age group, there was almost universal agreement on QOL between the children and their parents apart from the subscale of school and sport (P = 0.04). Within the severity groupings, there was much more concordance between the parents and children. The only significant difference between parents and children was for physical health in the mild group (P = 0.03). In the moderate-severe groups, no results were significant, however, five of the subscales showed borderline significant (P < 0.1) results.Conclusions: Parental perceptions of QOL of their sons changes with the age of the child and the severity of the haemophilia. These perceptions need to be taken into account when developing an overall care strategy involving both the child and parent. [ABSTRACT FROM AUTHOR]

Aims Fully implantable central venous access devices ( CVADs) can offer long-term reliable venous access to facilitate regular factor replacement therapy in haemophilia. However, CVAD-related infection remains a major deterrent to the optimal use of CVAD in this population. This report represents the first review of CVAD use in haemophilia in Australia and aims to examine the rate of complications including CVAD-related infections. Methods A retrospective review of medical records was conducted of all haemophilic patients with fully implantable CVADs at the Royal Children's Hospital ( RCH), Melbourne, between 1 June 1992 and 30 June 2009. CVAD-related bloodstream infection was defined based on the guidelines from the Centre of Disease Control and Victoria National Nosocomial Infection Surveillance. To further enhance identification of CVAD-related infection in this study, a third criterion of 'suspected infection' was added by the authors. Results Eighty-one CVADs in 56 patients were managed at the RCH during this time period resulting in a combined study period of 94 756 CVAD days. Median age at first CVAD insertion = 2.16 years (range 0.66 to 13.98 years). CVADs were inserted predominantly due to difficult venous access and prophylaxis initiation (70.4%). Median life-span of a CVAD was 1227 days, equivalent to 3.36 years ( n = 50; range 0.22 to 9.44 years). Fifty-seven CVAD-related infections occurred in 37 CVADs (46.3%) in 29 patients (51.8%). Overall incidence of confirmed CVAD-related bloodstream infection = 0.42 per 1000 CVAD days (95% confidence interval ( CI): 0.31 to 0.58 per 1000 CVAD days) and indicate better performance compared with the published benchmark of 0.66 per 1000 CVAD days (0.44 to 0.97 per 1000 CVAD days). The incidence of both confirmed (criteria 1, 2) and suspected (criterion 3) CVAD-related infection is 0.60 per 1000 CVAD days (95% CI: 0.46 to 0.78), which is comparable to the international benchmark. The majority of CVAD-related infections (73.7%) were successfully treated with intravenous antimicrobials without necessitating CVAD removal. Klebsiella pneumoniae was the most common organism found in positive blood cultures. Conclusion CVAD-related infection in this Australian population was comparable to rates described in the medical literature. Ongoing surveillance for infection rates is important to provide an up-to-date assessment of risks associated with CVAD use in this population. [ABSTRACT FROM AUTHOR]

The article highlights a research study on a benchmarking initiative to help manage hemophilia in children in Australia. Topics noted include the role of congenital deficiency of factor VIII and factor IX in the occurrence of hemophilia A and hemophilia B, involvement of site visits to pediatric hemophilia treatment centers, and importance of physiotherapy and social work in hemophilia treatment.