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CHS Research Grants for 2018

VWF in megakaryocytes and the role of platelet-VWF in VWD


Dr. Walter Kahr
Hospital for Sick Children – Toronto
Second year funding

People with severe von Willebrand disease (VWD) have bleeding problems caused by a lack of von Willebrand factor (VWF), typically missing in their plasma and platelets. We have found, however, that patients with a particular mutation have some VWF in their platelets. They have less severe bleeding problems than most severe VWD patients, possibly owing to this platelet VWF.

Our study addresses two key knowledge gaps: 1) How is VWF packaged by megakaryocytes into platelets? 2) What is the role of platelet VWF in reducing bleeding symptoms in some severe VWD patients? We will address these questions using methods we have successfully employed in my laboratory involving platelet development and function, including high resolution microscopy and genetic manipulation of megakaryocytes.


Evaluation of the burden of bone fracture in patients with hemophilia A: A population-based study


Dr. Alfonso Iorio
McMaster University – Hamilton Ontario
One year funding

Co-investigators:
Dr. Chatree Chai-Adisaksopha, McMaster University
Dr. Michelle Sholzberg, University of Toronto
Dr. Adrienne Lee, University of Calgary

There are increasing numbers of patients with hemophilia (PWH) developed age-related co-morbidities, for instance, cardiovascular disease, diabetes or cancer. Age-related bone disease, such as, low bone mineral density and osteoporosis are the emerging concern in PWH. However, the data on the incidence of bone fracture in PWH are limited. The aims of this study are to determine the incidence of bone fracture in PWH compared with general population and to explore risk factors of bone fracture. Better understanding of the burden of fracture in PWH will enable physicians, hemophilia nurses, physiotherapists and stakeholders to allocate resources in hemophilia care. In addition, this information will enable physicians to identify patients at greater risk of fracture.


The Hemostatic Stress Response: Do Differences Explain Phenotypic Variability in VWD?


Dr. Paula James
Queen’s University – Kingston, Ontario
First year funding

Von Willebrand Disease (VWD) is the most common inherited bleeding disorder known in humans, and affects ~35,000 Canadians. It causes excessive bleeding from the skin and mucous membranes and can be highly variable in its clinical presentation. Some affected patients experience little in the way of bleeding symptoms, but some suffer from prolonged and excessive bleeding with menstruation, and following dental procedures, childbirth or surgery. In the worst cases, blood transfusions or surgical procedures such as hysterectomy are required to treat patients. At present, factors that lead to more severe bleeding are not completely understood, and new knowledge in this field could help us provide more effective treatment for patients. In this study, we will specifically test the hypothesis that Type 1 VWD patients who have more severe bleeding have a lesser capacity to respond to stresses on their blood coagulation system. We will include stressors such as exercise or treatment with a medication called desmopressin, and will also perform experiments on endothelial cells cultured from patients and healthy controls.    


A scanning electron microscope study of clot ultrastructure of coagulated factor VIII deficient-plasma in the presence of anticoagulants


Lab work studentship

Bushra Sajjad
National University of Ireland, Galway
Under the supervision of Dr. Anthony Chan and 
Jorell Gantioqui, McMaster University – Hamilton, Ontario

Hemophilia patients suffering from serious bleeding problems may also develop blood clot complications due to replacement in the blood of the absent clotting protein. Although blood thinner is traditionally used to treat blood clotting, this exacerbates the risk of bleeding in patients suffering from hemophilia as they are inherently more susceptible to bleeding. As a result, the management and treatment of this situation poses a challenge for clinicians. Therefore, we propose a study investigating the changes of blood clot structure through the use of electron microscopy. Information from this study will be used to provide important insight regarding blood clots in hemophilia patients treated by blood thinners. Results of this study will analyze the clotting process from another standpoint which can aid researchers in understanding the effects of blood thinner in hemophilia patients who are already deficient in a clotting protein.