Consensus recommendations for use of central venous access devices in haemophilia

Abstract

Venous access is essential for delivery of haemophilia factor concentrate. Wherever possible, peripheral veins remain the route of choice, and the use of central venous access devices (CVADs) should be limited to cases of clear need in patients with caregivers able to exercise diligence in CVAD care and should continue no longer than necessary. CVADs are of recognized value for repeated administration of coagulation factors in haemophilia, particularly for prophylaxis and immune tolerance therapy and in young children. Evidence to guide best practices has been fragmentary, and standardized methods for CVAD usage have yet to be established. We have developed management recommendations based upon available published evidence as well as extensive clinical experience. These recommendations address patient and CVAD selection; CVAD placement, care and removal; caregiver/patient guidance; and complications, including infection and thrombosis. In the absence of inhibitors, ports are recommended, primarily because of fewer associated infections than with external catheters. For patients with inhibitors, ports also appear to be associated with fewer infections. Infection is the most frequent complication, and recommendations to prevent and treat infections are supported by extensive clinical data and experience. Strict adherence to handwashing and aseptic technique are essential elements of catheter care. Evidence-based data regarding the detection and treatment of CVAD-related thrombotic complications are limited. Caregiver education is an integral part of CVAD use and the procedural practices of users should be regularly re-assessed. These recommendations provide a basis for sound current CVAD practice and are expected to undergo further refinements as new evidence is compiled and clinical experience is gained.

The principal results of the International Immune Tolerance Study: a randomized dose comparison

“…9,11,12 Potential treatment-related ITI outcome variables include bleeding, central venous catheter device (CVAD) infection, FVIII type, and dosing regimen. Although CVAD infections have been reported repeatedly to adversely affect ITI outcome, [13][14][15][16][17][18] they did not predict ITI outcome in the NAITR. 9 The impact of bleeding has never been examined.…”

Haemophilia in the first years of life

“…Patients with inhibitors have more infections probably because of minimal bleeding around the port after use. Table 1 shows other potential factors that may influence the rate of infection [49,57]. The most important are probably related to aseptic measures, quality of education and the compliance of the users, whether these are parents or health-care professionals.…”

How I use bypassing therapy for prophylaxis in patients with hemophilia A and inhibitors

“…Peripheral venipuncture is preferred for the administration of replacement clotting factor. 65 However, a CVAD (either a tunneled, fully implantable, subcutaneous port or a tunneled, external catheter) may be needed as a bridge to venipuncture in infants and children, whose small peripheral veins are difficult to access, and occasionally in older children or adults requiring very frequent (ie, daily) peripheral venous access to administer clotting factor. CVADs make regular infusion of clotting factor feasible for some patients but are not without risks.…”