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Research Projects Funded in 2011

von Willebrand Disease plasma and platelets: Functional characterization of quantitative and qualitative von Willebrand factor mutations


Dr. Paula James
Medicine & Pathology and Molecular Medicine
Queens University - Kingston, Ontario
First year funding

Co-investigators: Dr Walter Kahr; Dr Sue Robinson


In this study, we propose to perform a detailed analysis of two unique Canadian families; one with individuals with both Type 1 and Type 3 von Willebrand disease (VWD) and one with Type 2B VWD.  VWD is the most common, inherited bleeding disorder known in humans and causes excessive bleeding from skin and mucous membranes and in its most severe form, Type 3 VWD, bleeding into muscles and joints as well.  We believe that only through detailed study will we improve our understanding of this disease, an understanding that we hope will ultimately lead us to better treatments for individuals affected with VWD.  We have identified two interesting VWF mutations in these families and will investigate the significance of those mutations in our laboratories.  Investigators from three Canadian centers will be involved: Dr. Paula James from Queen's University, Dr. Walter Kahr from The Hospital for Sick Children and Dr. Sue Robinson from QEII Health Sciences Center.  We believe the comprehensive set of experiments included in this proposal will allow for the synergistic interaction of the three Canadian centers, improving our understanding of  VWD.


Bone mineral density in Canadian children with severe hemophilia A or B: A multi-centre, cross-sectional, observational study.


Cecily Bos, PT 
Hemophilia Department
Hamilton Health Sciences - Hamilton, Ontario

and

Dr Anthony K. Chan
Hemophilia (pediatric)
McMaster University - Hamilton, Ontario

First year funding

Co-investigators: Dr Colin Webber; Dr Kathryn Webert

Recent research has shown that bone mineral density, a measure of bone strength, may be lower among people with hemophilia than among those without this disease. The majority of this research has been done in countries where the treatment for hemophilia differs from the standard of care in Canada. This study is being done to find out whether Canadian children and youth with severe hemophilia A and B have lower BMD than their health peers.

People with hemophilia do not have sufficient amounts of certain blood components (called coagulation factors) to help the blood clot. Current treatment strategies offer a greatly improved quality of life and the expectation of a lifestyle similar to the rest of the population. These strategies can vary, but the standard of care in Canada for patients with severe hemophilia A and B is to regularly infuse the missing factor to prevent bleeding.

In the first study of its kind in Canada, researchers will explore any association between BMD and factor replacement therapy, joint health, bleeding history, and physical activity levels by evaluating bone scans of children and youth with severe hemophilia A and B across the country.



Development and evaluation of an innovative Web-based Educational Program to promote self-management for teens with hemophilia


Dr. Jennifer Stinson
The Hospital for Sick Children - Toronto, Ontario
First year funding

Co-investigators: Dr. V. Blanchette; Dr. V. Breakey; Mr. Danial Ignas

In recent years, the importance of smooth transition of care from pediatric to adult care has received increasing attention. Pediatric hematologists who care for teens with childhood-onset hematological conditions, such as hemophilia, face challenges in supporting transition to ensure that these young people are educated and able to manage their medical conditions as adults. This program of research aims to develop an innovative web-based self-management and transition care program to meet the needs of young people with hemophilia. The proposed research includes a usability study to test the user-friendlessness of the website and a pilot study to assess the feasbility of the web-based educational intervention for adolescents with hemophilia. Data from the pilot study will guide the development of a randomized controlled trial that will assess the effectiveness of the intervention on important clinical health outcomes. The knowledge gained through this work will be transferable across chronic conditions affecting adolescents and will be useful for developing similar programs to improve transition of care. For youth with hemophilia, it will provide ongoing education and support through transition of care and beyond.