The Low Von Willebrand factor Ireland Cohort of Kids (LoVIC-K) study

Von Willebrand factor (VWF) is a large plasma protein essential for maintaining normal blood coagulation. Approximately 1% of the children and adults inherit low levels of his blood clotting protein, and consequently have a lifelong bleeding disorder known as von Willebrand disease (or VWD). Children with VWD often demonstrate easy bruising, nose bleeds and may have significant bleeding after surgical procedures or dental extractions. Although VWD is the commonest inherited bleeding disorder in children, the biological mechanisms that cause reduced VWF levels remains poorly understood. This is particularly true for children with mild to moderate reductions in VWF levels. The fact that the biological basis responsible for causing Low VWF levels is not understood, poses significant clinical challenges when it comes to the diagnosis and management of these children. Consequently, in order to answer these important clinical and scientific questions, we propose to establish the Low VWF Ireland Cohort of Kids (LoVIC-K) study. This study will include 125 Irish children with Low VWF levels who will be followed up in a longitudinal manner. Through a previous successful study of adult Irish patients with Low VWF levels, we have already established an international network of leading experts who will again be involved in the LoVIC-K paediatric study. In addition, we have optimised a series of new laboratory blood tests that can now also be applied in paediatric patients. In combination, the findings from the LoVIC adult and LoVIC-K studies will not only establish Ireland as an international leader in this field, but will be have direct relevance in terms of improving the clinical management of these children.

Award Date
30 June 2017
Award Value
€369,360
Principal Investigator
Professor James O'Donnell
Host Institution
Royal College of Surgeons in Ireland
Scheme
Investigator Led Projects