Abstract:
Port-a-cath is a widely used device in patients with long-term venous access demand such as frequent or continuous
administration of medications such as Enzyme Replacement Therapy (ERT), chemotherapy delivery,
blood transfusions, blood products, and fluids. Patients with Lysosomal Storage Diseases (LSDs) often require
recurrent courses of ERT. We reviewed our experience of using port-a-caths in patients with LSDs with the focus
on challenges and complications associated with these catheters. Among 245 adult patients who were treated
with ERT, twenty patients (8.2%) had a port-a-cath inserted due to poor venous access. Six patients were using
their first port whereas five other patients had their port-a-caths replaced at least once. The remaining six
patients had inactive port-a-caths. The majority of patients with active port-a-caths never missed more than one
consecutive infusion, although one patient missed 2 consecutive infusions whilst on holiday. We identified
significant gaps in patients' and their families' understanding of the management of port-a-caths and risks associated
with them. It resulted in producing a leaflet and designing an educational program for our LSD patients.
