Haemophilia A - Therapy

Therapy

Most severe hemophilia patients require regular supplementation with intravenous recombinant or plasma concentrate Factor VIII. The prophylactic treatment regime is highly variable and individually determined. Apart from "routine" supplementation, extra factor concentrate is given around surgical procedures and after trauma. In children, an easily accessible intravenous port (e.g. Port-a-Cath) may have to be inserted to minimise frequent traumatic intravenous cannulation. These devices have made prophylaxis in hemophilia much easier for families because the problems of "finding a vein" for infusion two to three times a week are eliminated. However, there are risks involved with their use, the most worrisome being that of infection. Studies differ but some show an infection rate as high as 50%. These infections can usually be treated with intravenous antibiotics but sometimes the device must be removed. Also, there are other studies that show a risk of clots forming at the tip of the catheter. Still, many families choose to use the device because of the benefits.

Some patients with severe hemophilia and most with moderate and mild hemophilia treat only as needed without a regular prophylactic schedule. Due to risk of permanent disability, prophylactic treatment is always indicated if a "target joint" (a joint that has repeated bleeding episodes) is identified.

Mild hemophiliacs often manage their condition with desmopressin, which releases stored factor VIII from blood vessel walls.