Central venous access devices for Hemophilia

Overview
People with hemophilia who have developed inhibitors may need frequent or urgent doses of intravenous medications, especially while undergoing immune tolerance therapy. Some people choose to have a central venous access device (CVAD) placed to allow for quick dosing at home and reduced needle sticks in peripheral veins.

What does it involve?
There are several different types of CVADs. Each type of CVAD must be placed and eventually removed surgically.

Ports are a popular choice. A port consists of a small disk with a silicone bubble on top. The port is usually placed in the chest. Injections are given into the silicone bubble, and the medication travels into the blood vessels via plastic tubing called a septum that is surgically introduced into a vein. Ports can last for many years. It is safe to bathe and swim with a port.

An arterio-venous fistula (AVF) is a surgically-created pathway between an artery and a vein. The fistula is usually placed in an arm or a leg, where it is visible. Once the fistula heals, it provides a new peripheral pathway for infusion. AVFs can last for many years. It is safe to bathe and swim with an AVF.

A peripherally inserted central catheter (PICC line) is a temporary solution. PICC lines are flexible tubes inserted into the upper arm. Medication is infused through the part of the tube that hangs out of the arm. Inside the body, the tube is inserted into a large vein in the chest. It may be difficult to bathe with a PICC line, and it is not safe to swim.

Tunneled Central Venous Access (Hickman or Broviac lines) are long silicone tubes placed into the chest by a surgeon. Medication is infused into the part of the tube that hangs out of the chest. Inside the chest, the tube interfaces with a large vein. It may be difficult to bathe a child with a Hickman or Broviac line.

Constraints
CVAD placement and removal requires minor surgery with associated risks for pain, bleeding, and infection.

CVADs have been associated with serious lung injuries including pneumothorax (collapsed lung) and hemothorax (blood trapped in the chest cavity).

CVADs can become infected.

Some types of CVADs are prone to thrombosis (clotting) and stenosis (narrowing).