Haemophilia A and B are hereditary X-linked disorders due to deficiency (or absence) of coagulation factor VIII or IX, respectively. Bleeding risk is related to the severity of factor deficiency. Repeated joint bleeding can lead to a severe haemophilic arthropathy resulting in disabilities. Outcome measurements in persons with haemophilia (PWH) have been limited to laboratory evaluation (factor...

Over the past 20 years, there have been many advances in haemophilia treatment that have allowed patients to take greater control of their disease. However, the development of factor VIII (FVIII) inhibitors is the greatest complication of the disease and a challenge in the treatment of haemophilia making management of bleeding episodes difficult and surgical procedures very challenging. A meeti...

Abstract Background Increasing survival among people with haemophilia means that more individuals are at risk of developing age-related morbidity. Little is known about morbidity and health-related quality life (HRQoL) in different age groups within a single large population haemophilia. Aim This study aimed to explore the association between increasing comorbidity compare their HRQoL sample ge...

OBJECTIVES Target joints are a common complication of severe haemophilia. While factor replacement therapy constitutes the majority of costs in haemophilia, the relationship between target joints and non drug-related direct costs (NDDCs) has not been studied. METHODS Data on haemophilia patients without inhibitors was drawn from the 'Cost of Haemophilia across Europe - a Socioeconomic Survey'...

Quality of life (QoL) is a recent focus of research in haemophilia. It can be defined--in analogy to the World Health Organization (WHO) definition of health--as patient-perceived wellbeing and function in terms of physical, emotional, mental, social and behavioural life domains. The paper describes conceptual, methodological and practical foundations of QoL research in adults and children at a...

The Haemophilia Utilization Group Study (HUGS) was created 10 years ago to examine the annual utilization and cost of haemophilia-related healthcare services. Retrospective chart reviews for 336 patients with haemophilia A receiving treatment in one of five comprehensive haemophilia treatment centres (HTCs) during 1995 were completed through interview of the provider. This method provided adequ...

Carriers of haemophilia face difficult choices regarding prenatal diagnosis, but little is known about the determinants that influence their decisions. The aim of this study was to assess the incidence of prenatal diagnosis and potential determinants affecting the choice for prenatal diagnosis. A nationwide survey was performed among all women who underwent carriership testing for haemophilia i...

Disease-specific measures of quality of life (QoL) for children with haemophilia are now available for use in clinical studies [Haemophilia, 10, 2004, 9-16]. One of these measures, the Canadian Haemophilia Outcomes - Kids' Life Assessment Tool (CHO-KLAT), was developed in Canada with emphasis on the perspectives of children [Pediatr Blood Cancer, 47, 2006, 305-11; Haemophilia, 10, 2004, 34-43]....

OBJECTIVE To assess the cost effectiveness of primary prophylaxis with clotting factor instead of treatment following a bleed (on-demand) for individuals with severe haemophilia. DESIGN Different data sources on the clinical effects and costs of treatments were combined using a Markov model. SETTING English treatment centres. PERSPECTIVE UK societal. PARTICIPANTS Hypothetical cohorts of...

Adequate replacement therapy in haemophilia has been available for two decades. This has led to considerable improvements in the life expectancy and physical status of haemophilia patients. A study was conducted to investigate whether this has also led to improvements in quality of life. With this aim, information was obtained from 935 Dutch haemophiliacs by mailed questionnaires on relationshi...