Haemophilia in the Netherlands-6
S.C. Gouw (project coordinator), S. Hassan (PhD Student), E.C. van Balen (PhD Student)
Prof. dr F.R. Rosendaal, Prof. dr J.G. van der Bom
HiN-6 study group, all Dutch haemophilia treatment centers
Haemophilia is a hereditary disorder of blood clotting with a prevalence of 10 per 100 000 inhabitants. Patients with hemophilia have a lack of clotting factor VIII (haemophilia A) or IX (haemophilia B). The residual amount of clotting factor determines the severity of the disease. Severe haemophilia (<1%) is characterized by spontaneous bleeding in muscles and joints. In mild (>5-40 %) and moderate haemophilia (1-<5%) bleeding occurs after trauma, surgery or dental procedures.
In spite of the small patient population hemophilia is a well-described hereditary disease. In the past 45 years five Haemophilia in the Netherlands surveys have been performed that have contributed significantly to the knowledge about haemophilia. The last national survey was performed 15 years ago.
HiN-6 project aims to describe the current health status of the Dutch haemophilia population, especially focussing on ageing patients, HIV/HCV patients and inhibitor patients. In addition, quality of life may be reduced in some persons with haemophilia (PWH) despite similar levels of physical health. Further, differences in clinical phenotypes have been described in PWH with comparable coagulation factor activities. Lastly, inhibitor formation is still an important complication in haemophilia treatment and a better understanding is needed about the mechanisms that lead to inhibitor formation.
The main aim of the HiN-6 study is to
- describe the health status of the Dutch haemophilia population, with special focus on viral infections, inhibitor development and age-related co-morbidities
- attempt to gain insight in health-related quality of life of PWH
- evaluate the quality of care
- explain the variability in clinical phenotype among PWH
- gain insight into the mechanisms underlying the humoral and cellular immune response to FVIII.
In this study, all 1600 male Dutch patients with a clinical diagnosis of severe, moderate, or mild haemophilia A or B will be included. We will collect data through questionnaires filled in by each participant and by blood sampling.