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Cystic fibrosis (CT) is a genetically inherited, multisystem condition, resulting from abnormal salt and water transport across epithelial surfaces. Advances in medical treatments have resulted in vastly improved care of CF patients. This has led to the increased survival of CF sufferers from early childhood in the 1940s to nearly 40 years of age in 2012 (1). These therapies include nebulized and inhaled mucolytics, and bronchodilators, as well as prophylactic antibiotics and physiotherapy. Most CF patients require some form of long-term venous access for ease of administration of repeated courses of intravenous antibiotics. Some of our patients are fitted with a totally implantable venous access device (TIVAD) referred to as a Port-A-Cath. Over time, these indwelling devices present their own complications. We report a series of three patients with potentially life-threatening thrombotic and thromboembolic phenomena arising from their indwelling central venous catheters (CVCs).

Increased vigilance needed for the detection of thrombotic complications of central venous access in adolescent cystic fibrosis patients