Swedish national registry for bleeding disorders
Bleeding disorders are diseases that gives an increased tendency for bleeding. These diseases are usually hereditary and the most common bleeding disorders are hemophilia A, B and von Willebrand’s disease.
Hemophilia A and B are relatively rare inherited bleeding disorders that causes problems with the blood’s ability to clot. In the severe forms of hemophilia, there is no or very low levels of the missing clotting factor.
Hemophilia A and B affect almost exclusively men, while women are carriers. In hemophilia A there is decreased level of clotting factor VIII and in Hemophilia B factor IX. Hemophilia A is more common than Hemophilia B.
In von Willebrand’s disease, men and women are at equal risk of having the disease. von Willebrand’s disease means lack of or defective von Willebrand factor.
These bleeding disorders occurs in three different degrees of severity: severe, moderate, or mild form. In its severe form, the disease is usually detected during the first two years of life and give rise to prophylactic therapy with so-called coagulation or clotting factor concentrate, drugs which are administered as injections often several times every week.
The purpose of the national registry is to get a good follow-up of this limited group of patients who receive a costly treatment often life-long and to improve quality of care. The most important measures when evaluating the treatment is the occurrence of bleeds per year, joint injuries, which is the late effect of previous joint bleeding and which is measured functionally with so-called joint scores, which is a physical examination performed by a physiotherapist.