Dr. Man-Chiu Poon MD, FRCPC
Emeritus Professor & Clinical Professor of Medicine – Paediatrics & Oncology, Cumming School of Medicine, University of Calgary
Hemophilia is a hereditary disorder affecting approximately 3,000 Canadians of all ethnic origins in which blood doesn’t clot normally due to a lack of the protein Factor VIII or Factor IX. Individuals living with hemophilia don’t bleed more profusely or more quickly than other people; they bleed for a longer time.
Hemophilia is divided into type A (Factor VIII deficient) and type B (Factor IX deficient). Approximately 80% of cases in Canada are type A. Classified as severe, moderate, or mild, the most severe forms of hemophilia affect males almost exclusively. The greatest danger for those living with hemophilia is internal bleeding, which occurs in tissues, muscles, and joints — especially knees, ankles, and elbows.
“If not treated appropriately, these hemorrhages cause much pain and often lead to arthritis-related disabilities and problems with the muscle-skeletal system,” explains Dr. Man-Chiu Poon, of the Southern Alberta Rare Blood and Bleeding Disorders Comprehensive Care Program at the University of Calgary and an internationally recognized researcher in the area of hemophilia.
Dr. Poon has experienced the evolution of care for people with hemophilia over the past 45 years and witnessed the continual improvement of their quality of life and life expectancy. They now have many management options and he believes their future is bright with curative treatment being on the horizon.
Leading a full, active life with hemophilia
With new treatments becoming available frequently, most people with hemophilia can lead full, active lives. The basic treatment to stop or prevent bleeding related to hemophilia A and B is factor replacement therapy — the injection of Factor VIII and IX concentrates into the bloodstream. An innovative factor VIII replacement therapy approved by Health Canada in July leverages an extended half-life to help Canadians living with hemophilia A better manage bleeding episodes. There are also extended half-life products for those with hemophilia B.
“Whereas we previously would have had to provide many patients with treatments several times a week (some even daily), with the longer half-life we’re able to reduce the number of treatments,” explains Dr. Poon.
In Canada, medical services for the treatment of hemophilia and related conditions are provided at a comprehensive care clinic.