title: Central venous access devices in children with hemophilia: an update.
abstract: The most frequent indication for placement of a central venous access device in hemophiliacs is in very young boys (ages 1-2 years) with severe hemophilia who are started on a program of long-term factor prophylaxis 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 factor units per kg on alternate days (minimum 3 times a week) for boys with severe hemophilia A, and twice a week for boys with severe hemophilia B. To facilitate this prophylaxis regimen some hemophilia clinics routinely recommend placement of a central venous access device; others, more concerned about associated complications such as sepsis, stress the importance of using peripheral veins wherever possible, with central access devices reserved for occasional, selected cases only. A decision 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 lower risk of infection, and because totally implantable devices allow children to take part in activities such as swimming. Important complications include catheter-related sepsis, which may occur in 25% or more of devices over time and, much less frequently, catheter-related deep vein thrombosis.
authors.affiliation: Department of Paediatrics, The Hospital for Sick Children and the University of Toronto, Ontario, Canada.
authors.forename: V S
authors.initials: VS
authors.lastname: Blanchette
authors.forename: A
authors.initials: A
authors.lastname: Al-Musa
authors.forename: A M
authors.initials: AM
authors.lastname: Stain
authors.forename: J
authors.initials: J
authors.lastname: Ingram
authors.forename: R M
authors.initials: RM
authors.lastname: Fille
mesh_terms: D001424:Bacterial Infections / Q000209:etiology; D002405:Catheterization, Central Venous / Q000009:adverse effects; D002675:Child, Preschool; D005164:Factor IX / Q000008:administration & dosage* / Q000627:therapeutic use; D005169:Factor VIII / Q000008:administration & dosage* / Q000627:therapeutic use; D006395:Hemarthrosis / Q000517:prevention & control; D006467:Hemophilia A / Q000150:complications* / Q000188:drug therapy*; D006801:Humans; D007223:Infant; D008297:Male; D009140:Musculoskeletal Diseases / Q000517:prevention & control
chemical_list: D005169:Factor VIII; D005164:Factor IX