Central venous access devices for paediatric patients with haemophilia: a single‐institution experience

Abstract

Use of a central venous access device (CVAD) can facilitate early introduction of home-based infusion of factor concentrate for long-term prophylaxis or immune tolerance therapy in children with bleeding disorders. The aim was to review outcomes associated with use of CVAD. Retrospective review of paediatric patients with bleeding disorders was observed at the Mayo Clinic Comprehensive Hemophilia Center. Thirty-seven CVAD were placed in 18 patients (haemophilia A [n = 15], type 3 von Willebrand disease [n = 2] and haemophilia B [n = 1]). Follow-up was for 45 952 CVAD days, and median time that CVAD remained in place was 1361 days per device. Factor VIII (FVIII) inhibitors were present in 4 of the 15 patients. Ten CVAD-related infections occurred (median, 672 days; range, 72-1941 days), of which six were in one patient with FVIII inhibitors. Overall infection rate was 0.22 (95% confidence interval [CI], 0.10-0.40) per 1000 CVAD days, with 0.11 infections in patients without FVIII inhibitors compared with a pooled incidence of 0.66 (95% CI, 0.44-0.97) reported in the literature. Indications for removal of 27 CVAD were blockage, change to peripheral venous access, catheter displacement, infection, leak in the port septum, short catheter and skin erosion. No clinically apparent thrombosis or sequelae of thrombosis were observed. Infection is the most common complication associated with CVAD use and is increased in patients who have inhibitors. The low rate of clinically apparent thrombosis reflects our practice of not screening for thrombosis. The low infection rate reflects our practice of using and reinforcing the aseptic technique.

Complications associated with central venous access device in children with haemophilia: a nationwide multicentre study in Finland

“…Non‐infectious complications were the most common reason for CVAD removal. Incidence of mechanical complications is similar to previously reported , but the malfunction rate seems to be higher than that previously reported . When comparing to the findings of a meta‐analysis , CVADs remained in situ for twice as long; the median life span was 1159 days (3.2 years) in our cohort compared to 578 days (95% CI 456–733 days) in the meta‐analysis.…”

The impact of clinical practice on the outcome of central venous access devices in children with haemophilia

“…The infection rate in this large cohort was comparable or somewhat higher than those from previously published data . This may be because only ports that had been removed were included and those that were still in situ without complications were excluded thus giving a selection bias towards increased infections.…”

Parents of children with haemophilia at an early age: assessment of perceived stress and family functioning

“…In our study, we observed the appearance of inhibitors only affects the effort to cope with the stress on the emotional distress (10.75%). In the same line, other authors , noted how arthropathy and inhibitors may cause fear, isolation, depression or aggression in the parents.…”