Hemophilia belongs to a family of inherited lifelong bleeding conditions that prevent blood from clotting properly. Patients with these disorders bleed for longer than normal, either as a result of injury or spontaneously without an external cause.
The severity of bleeding depends on the amount of clotting factor that is missing or not functioning properly, which in hemophilia A and B - the most common types of hemophilia - is the coagulation factors VIII and IX, respectively.
In addition to external bleeding, patients more commonly have internal bleeding around the joints and muscles, which can be extremely painful and cause permanent disability. Bleeding into major organs such as the brain is especially difficult to manage and can be fatal.
Here are 2 hemophilia educational videos by CDC: Playing it Safe With Hemophilia: Friends with hemophilia talk about playing sports growing up and the importance of making smart decisions.
Starting the Conversation: Hemophilia. How to talk to your friends about hemophilia. A group of friends ask their friend Billy questions about his hemophilia:
Hemophilia care has undergone substantial improvements during the past 40 - 50 years. Early clotting factor concentrates were not sufficiently refined to enable self-administered treatment at home until the 1970s.
Long-term substitution therapy (prophylaxis) of the missing clotting factor is the recommended treatment in severe hemophilia. The major side-effect of treatment, development of inhibitors to the infused concentrate, is the main threat to the health of patients.
Mnemonic: Differential Diagnosis of Bleeding Disorders: F-CAP
Fibrinolysis - tPA
Coagulopathy - hemophilia, vWD
Angiopathy - conditions affecting blood vessels, e.g. Osler-Weber-Rendu syndrome
Platelets - thrombocytopenia or thrombocytopathia
Initial diagnostic tests = 3P:
PT - INR
Making haemophilia a global priority - The Lancet, 2012.
Modern haemophilia care - The Lancet, 2012.