Management of totally implantable vascular access devices in patients with cystic fibrosis

Cystic fibrosis (CF) is a genetic disease associated with recurrent lung infections, that represent a major cause of mortality and morbidity. Cystic fibrosis requires frequent antibiotic treatments, sometimes by mouth or via aerosol but often via the intravenous route. Totally implanted venous access devices (ports) allow an easy and safe vascular access for unlimited periods of time, and they can be used in CF to administer antibiotics and other i.v. infusions; if compared to external central venous catheters, ports are better tolerated, since they permit almost unlimited physical activity and do not interfere with patient's self-image. Though ports require a minimal level of care, they may be sometimes associated with relevant complications, which can be insertion-related (pneumothorax, arterial puncture, local hematoma), or management-related (infection, occlusion of the lumen, venous thrombosis). This article summarizes some recommendations on the management of ports in CF, considering the existing literature. Still, some issues remain unsolved and will need further research and studies.