Long-Term Venous Access Catheters and Infections in Patients with Severe Hemophilia.
Authors: Harrison Janet A. , Powell Jerry S. 
Published: April 2020

Treatment of hemophilia A or B with factor concentrates requires uncomplicated venous access, and frequently this access uses venous access catheters, which have been associated with increased frequency of thrombophlebitis and catheter related infections. Reports have estimated the incidence of infection as 8.3% to 55%. A prospective multi-center study was undertaken to determine the rates of these complications associated with venous access catheters in patients with severe hemophilia. Inclusion criteria were: severe hemophilia, age 6 months to 70 years, and non-emergent need for a long-term venous access catheter. Excluded were catheters inserted for less than 28 days or in a femoral site, and other medical conditions compromising expected survival. 53 subjects (48 hemophilia A, 5 hemophilia B) were enrolled by nine hemophilia treatment centers, with 3 subjects receiving repeat catheter insertions, for a total of 56 catheter experiences. Factor replacement was given to all subjects during catheter placement. Families were trained in catheter use according to individual center practices, and each subject was followed at least monthly in the clinic or by telephone. 54 catheters were placed by a physician in an operating room under sterile conditions; antibiotics were used for catheter placement for 18 subjects. The ages at placement ranged from 10 months to 59 years (mean 8.9 years). Most of the Catheter types were Port-a-Cath (n = 43), 6 Broviac, 4 Hickman, and 3 PICC. The reasons for the catheters varied: primary prophylaxis, 10, secondary prophylaxis, 23, immune tolerance, 17, and for treatment after surgery or trauma, 6. Five subjects were positive for hepatitis C, 2 subjects for hepatitis B, and one for HIV. Of the 53 subjects, 10 have had catheter related infections. The infections were detected 19 to 762 days after catheter insertion (mean 272.7 days). Two subjects had recurrent infections, 4 and 3 respectively. The ages of the subjects at the time of infection ranged from 1.8 to 13.9 years old (mean = 5 years). The peripheral blood cultures and the catheter tip (if removed) cultures revealed a variety of different bacteria and one fungus (candida albicans). Perhaps surprisingly, staphylococcus epidermidis was found in only 2 subjects, and no case of s. aureus was detected. Klebsiella was cultured from 3 subjects who experienced infections. No subjects died during the study. Eight catheters were removed for reasons determined by the responsible clinician: 6 for infection, 1 by accident, 1 was no longer needed. Nine of the 10 subjects with infections received antibiotics. Of the 6 Broviacs, 2 subjects experienced infections (33%). Of the 43 Port-a-Caths, 8 subjects experienced infections (18.6%). The 2 subjects with repeated infections both had Port-a-Caths, and the first infections occurred at 354 and 120 days, respectively. Of the 17 subjects on immune tolerance, 2 subjects experienced infections (not significantly different from the larger cohort). No subjects experienced thrombophlebitis or other catheter failure. In summary, the results of this study demonstrate that long-term venous access catheter use in patients with severe hemophilia: 1) is associated with significant risk of catheter related infections, particularly in children, 2) that these infections can be managed clinically in most cases, with appropriate clinical monitoring, and, 3) the risk of thrombophlebitis is quite low. These data should assist to evaluate the risks and benefits of the clinical decision to use venous access catheters in patients with severe hemophilia.