The deposit of cholesterol and inflammatory cells within arteries is termed atherosclerosis and is the leading cause of death in Canada. The endothelium is a protective surface on the arteries that if disturbed can increase the risk of developing atherosclerosis and its complications including heart attacks and strokes. Early studies suggest that patients with the bleeding disorder, hemophilia, have a reduced likelihood of dying from heart attacks and strokes. However, other studies suggest the opposite and further research is therefore warranted to provide definitive answers and to identify the mechanisms.

To solve these puzzles, Dr. Shannon Jackson, a member of the CBR and a Hematologist at St. Paul’s Hospital, along with her co-investigators, Drs. Vickars, Anderson, Chan and Poon, will lead a study supported by Pfizer Canada entitled “Heart disease in hemophilia: A prospective study evaluating endothelial function, fibrinolysis and the development of atherosclerotic vascular disease in the aging hemophilia population”.

Dr. Jackson and her colleagues have gained expertise in studying endothelial function in hemophilia with a specialized ultrasound. Abnormal function of the endothelium is felt to represent early disease of the arteries which may develop into atherosclerosis and cause heart attack or stroke. From preliminary studies initiated in Calgary, Dr. Jackson learned that the endothelium of large arteries in patients with hemophilia is not different from those of normal individuals. However, endothelial function of the smaller arteries of patients with hemophilia is notably impaired. This is a surprising finding which needs confirmation with larger numbers of patients with hemophilia. These Pfizer-supported studies, to be performed at UBC, will hopefully provide new insights into the mechanisms underlying the vascular disease associated with heart attacks and strokes that affect the aging person with hemophilia.

We congratulate Dr. Jackson and her collaborators on obtaining this highly competitive award and wish her rapid progress in this important field of investigation.