PURPOSE: To evaluate the incidence of implantable venous access device infection in patients with sickle cell disease. MATERIALS AND METHODS: The authors performed a retrospective search of their hospital's information system from January 1, 1996 to December 31, 2001 to identify hospital admissions with ICD-9 codes related to sickle cell anemia. This search yielded 2,703 admissions in 293 patients. A search of the radiology information system identified 23 of these patients who had placement of an implantable venous access device. Excluding two patients who were lost to follow-up, the population of this study included eight men and 13 women aged 23 to 62 years old (mean, 37 years). A total of 30 implantable venous access devices (25 venous ports, five tunneled catheters) were placed by interventional radiologists. Cases of device infection were identified based on clinical data, microbiology, reports of device removal, and clinical follow-up. Infections were defined according to the Centers for Disease Control criteria for catheter-related bloodstream infection. The incidence of infection, organism, and time from device placement to infection was determined. RESULTS: In 21 patients with 30 devices, 18 device infections (60%) occurred in 12 patients (57%) involving 15 venous ports and three tunneled catheters. There were a total of 12,389 days of catheter use and a rate of 1.5 infections per 1,000 catheter days. Infections occurred from 16 to 1,542 days (mean, 349 days) after device placement. Blood, wound, and catheter tip cultures yielded solitary organisms in 13 cases and mixed organisms in four cases. Staphylococcus aureus was the most common pathogen (59%). One patient was considered infected based on clinical signs and purulent discharge from the port site, despite negative cultures after partial antibiotic treatment. One patient died of sepsis resulting from an infected port. CONCLUSION: This study shows a high incidence of infection associated with placement of implantable venous access devices in patients with sickle cell disease. Therefore, the authors avoid placing these devices in this patient population.