Providing of the long-term vascular access in hemophilia patients

Background. There are no clinical guidelines in Russia regarding the use of central venous access devices in hemophilia despite venous access being crucial for hemophilia treatment. Objective. To analyse different long-term vascular access options in hemophilia patients. Materials and methods. We reviewed 12 cases (11 hemophilia patients and 1 patient with von Willebrand disease) in which long-term vascular access was established. All patients were treated in the National Research Center for Hematology between 2014 and 2018. Results. In total, 17 long-term central venous devices (LTCVD) were implanted in 12 patients (11 peripherally inserted central catheters, PICCs, and 6 ports). The PICCs were implanted in 7 patients of whom 4 had FVIII inhibitors. Median PICC dwell time was 214 days (7 to 464 days); the incidence of catheter-associated bloodstream infections was 0.41 per 1000 PICC days. The ports were implanted in 6 patients (3 via internal jugular veins, 2 via subclavian veins, and 1 via femoral vein in a patient with stenosis and thrombosis of the superior vena cava system). The incidence of catheter-associated thrombosis in this group was 0.15 per 1000 port days, and the incidence of catheter-associated bloodstream infections was 0.15 per 1000 port days. Conclusion. In inhibitor hemophilia patients and hemophilia patients who need intravenous therapy other than with clotting factors, PICCs are a good choice. It is necessary to estimate the demand for LTCVDs in hemophilia patients in Russia and to develop national guidelines regarding their use.