Hemophilia is a hereditary disorder in which the major clinical manifestation is
bleeding into the joints, muscles, internal organs and the CNS, often without any obvious
trauma.
Bleeding can be fatal as in the case of CNS
hemorrhage, or severely debilitating following repeated
bleeding into joints that results in crippling
arthritis. Treatment for
hemophilia includes the
intravenous administration of
clotting factor concentrates to replace the missing or defective
protein. Venous access is therefore critical to the treatment of
hemophilia and the prevention of complications due to
bleeding. According to the US Centers for Disease Control and Prevention, approximately half of patients less than 16 years of age and one-third of all patients with
hemophilia receive regular prophylactic
injections of
clotting factor concentrates. Prophylaxis, or the regular scheduled administration of antihemophilic factor concentrate, is effective in preventing
bleeding. Among those patients with severe disease, in the absence of prophylaxis, approximately 13 bleeds, including nine joint
hemorrhages, occur annually. In contrast, when prophylaxis is administered, the annual number of total and joint bleeds decreases to five and three, respectively. One of the major barriers to the more wide-spread use of prophylaxis is venous access. While peripheral venipuncture remains the first choice for venous access, central venous access devices are frequently used to facilitate repeated and/or urgent administration of
clotting factor concentrates. The advantages of central venous access devices are well recognized in certain treatment regimens such as prophylaxis and immune tolerance
therapy, as well as certain patient groups such as young children in whom venipuncture is often difficult and traumatic, and adults with scarred veins. Central venous access devices also allow earlier commencement of both home treatment and prophylaxis. The goal of this review is to discuss the different types of central venous access devices and their role in the management of
hemophilia to provide practitioners that care for patients with
hemophilia with the necessary information to make sound therapeutic recommendations to their patients.