Risks of long-term port use in enzyme replacement therapy for lysosomal storage disorders

Abstract

Totally implantable vascular access devices (TIVADs) are commonly used in conjunction with enzyme replacement therapy (ERT) for lysosomal storage disorders (LSDs). This case series describes potential complications associated with long-term TIVAD use, such as compromise of skin integrity, infection, or port failures. Best practices and skilled specialists are essential for minimizing complications from long-term TIVAD use for ERT. Keywords: Mucopolysaccharidosis, Enzyme replacement therapy, Totally implantable venous access device, Lysosomal storage disorder