Therapeutic use of arteriovenous fistula in children with haemophilia

Abstract

Haemophilia is an X-linked inherited bleeding disorder, which only affects males. Contemporary treatment of children with haemophilia requires adequate venous access for the administration of prophylactic therapy which commences as a relatively early age. In the majority of children this treatment is administered at home, usually by the parents and then, once competent, by the boys themselves. As venous access in young children is fraught with difficulty, central venous access devices have become the mainstay of haemophilia care. However, these devices come with their own difficulties, with infection and thrombosis causing significant side-effects in some children. Small but substantial cohorts of boys with haemophilia develop antibodies (or inhibitors) to coagulation factors, rendering them both more likely to experience bleeding and more complex to treat. These boys are, for reasons not yet fully understood, more likely to experience central line infections, which also affect overall treatment outcomes. Arteriovenous fistulae were used in four such boys in an attempt to eliminate central line infection and to enable continued treatment administration. The experience and outcomes of these four boys is discussed along with considerations and implications for nurses caring for them.