CENTRAL VENOUS ACCESS DEVICES IN CHILDREN WITH HEMOPHILIA - AN UPDATE

The most frequent indication for placement of a central venous access device in hemophiliacs is in very young boys (ages 1-2 years) with sev ere hemophilia who are started on a program of long-term factor prophy laxis designed to eliminate target joint bleeding and the development of chronic musculoskeletal disease. Although expensive, this strategy is extremely successful. It involves intravenous infusion of 25-40 fac tor units per kg on alternate days (minimum 3 times a week) for boys w ith severe hemophilia A, and twice a week for boys with severe hemophi lia B. To facilitate this prophylaxis regimen some hemophilia clinics routinely recommend placement of a central venous access device; other s, more concerned about associated complications such as sepsis, stres s the importance of using peripheral veins wherever possible, with cen tral access devices reserved for occasional, selected cases only A dec ision to use such a device should only be made after discussion of the risks/benefits with parents (or guardians) and with patients if of an appropriate age. If such a system is to be used, we recommend that a totally implantable device (Port-A-Cath) be placed because of the lowe r risk of infection, and because totally implantable devices allow chi ldren to take part in activities such as swimming. Important complicat ions include catheter-related sepsis, which may occur in 25% or more o f devices over time and, much less frequently, catheter-related deep v ein thrombosis.