Peripheral vein access is often a problem in patients with sickle cell disease (SCD). Totally implantable venous access devices (TIVAD) have offered other groups of patients safe long-term venous access. We reviewed our own experience with the use of a Port-A-Cath device in five patients with SCD undergoing exchange transfusion programmes. All five lines required removal due to infection associated with SBE, septic arthritis, pulmonary embolus and axillary vein thrombosis. The organisms involved were Staphylococcus aureus (3), Staphylococcus epidermidis (1) and Streptococcus sp. (1). The median working life of the catheters was 240 days (range 61-428). The median length of time from presentation to the diagnosis of a line-associated infection was 29 days (range 1-58). The rate of complications (0.4 per 100 patient days) in this small group of patients contrasts with the lower rates in patients with HIV and malignancy (0-0.1 per 100 patient days). Our results suggest that patients with SCD suffer an unacceptable incidence of infective complications associated with the Port-A-Cath. Bone infection is more common where there is pre-existing infarcted tissue. While these systems provide a valuable tool, our experience has led us to discontinue the use of TIVADs in SCD.