Central venous access devices in patients with hemophilia

Abstract

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.

Assessment of Treatment Practice Patterns for Severe Hemophilia A: A Global Nurse Perspective

“…Peripheral venipuncture remains the first choice for venous access, but CVADs are often necessary to facilitate prophylaxis in young children [23, 24]. While there are few case reports of symptomatic thromboembolic events associated with CVADs [25], infection is a major problem [26, 27].…”

Side‐effects and venous access issues with immune tolerance therapy

“…Each type of CVAD has its own advantages and disadvantages but in general, ports are significantly less likely to become infected [9]. Other key advantages of ports over external CVADs in patients with haemophilia is that they require less day‐to‐day care [10], are less subject to tampering by young children [11], and do not require the use of any special protective dressings for bathing or swimming.…”

Management of Bleeding Disorders in Children

“…23 Reported rates of catheter-related infection in hemophilia vary widely; a recent metaanalysis calculated a pooled incidence of infection of 0.66 per 1000 central venous access device (CVAD) days (CI: 0.44-0.97 per 1000 CVAD days). 24 This analysis also showed the overall risk of infection with CVAD was 40.0%. The infection risk in inhibitor patients is higher still perhaps due to the increased number of times ports are accessed during immune tolerance induction.…”