Central Venous Access Device-Related Infections in Hemophilia.

Central venous access devices (CVAD) are commonly used in young children with hemophilia who need frequent factor infusions, including prophylactic infusions given every other day to prevent joint disease and immune tolerance therapy given every day to neutralize inhibitors, both of which result in better health outcomes. CVAD use, however, may be complicated by infection or sepsis, catheter replacement, and rarely, subacute bacterial endocarditis (SBE) or death. Infection rates have varied widely among institutions, possibly in part because of variable diagnostic and reporting methods. In order to identify risk factors for CVAD-related infection in children with hemophilia, we distributed an electronic survey to 14 U.S. hemophilia treatment centers participating in the TMH network regarding children with hemophilia whose initial CVAD was placed between January 1, 2002 and December 31, 2004. Thirteen (94%) sites responded, providing data on 94 patients with CVAD. All but two (98%) were ports. CVADs were placed for prophylaxis in 66 (70%) patients and for immune tolerance therapy in 28 (30%) patients. Thirty-one (33%) patients developed CVAD-related infections, all in the first year following insertion. One subject developed SBE, and one died of CVAD-related sepsis. Infection was significantly more common in subjects receiving immune tolerance, 19 of 28 (68%), than on prophylaxis 12 of 66 (18%) (odds ratio = 9.5, p<0.001). The time from CVAD insertion to initial post-operative CVAD access did not influence the risk for infection (p = 0.13), but the duration of factor replacement following CVAD insertion did. Factor replacement for fewer than 7 days following insertion was associated with an increased incidence of CVAD-related infection (odds ratio = 2.2, p= 0.04). We conclude that individuals using CVAD for immune tolerance and/or receiving fewer than 7 days of factor replacement following CVAD placement are at greater risk for first CVAD-associated infection. The reasons for these findings are not known. However, the more frequent access of CVAD in patients on immune tolerance (daily vs. every other day) increases exposure to skin organisms, which could account for the higher rate of infection in these patients. Those receiving fewer than 7 days of factor replacement after CVAD placement may have insufficient wound healing or suffer local hematoma formation, which is a potential culture medium for local skin organisms. The high rate of CVAD infection in hemophilic children, >30% overall, constitutes a major public health problem for which prevention trials are in development.