by: AmyLu Weaver, M.D.

Vascular access may be difficult to obtain in Ehlers-Danlos syndrome (EDS) patients becomes of blood vessel wall fragility. Frequently, EDS patients have reported difficulty in starting peripheral IV's and obtaining blood samples because of venous collapse and perforation. Also, there has been an increased rate of intravenous infiltration and necrosis of tissue with IV therapy. Many EDS patients have reported the need for frequent and repeated cannulation of the subclavian and jugular veins. (There are also known as central lines.) Central line placement carries the risk of pneumothorax and bleeding complications with each line placement.

Vascular access ports are an alternative to central line placement for those patients requiring frequent intravenous access. Port placement carries the same risks as central line placement, i.e., bleeding, pneumothorax or collapse of the lung, thrombosis, embolism, and air embolism. However, the ports are placed into the subclavian vein under sterile operating room technique and fluroscopic guidance with direct visualization. (Central lines are usually placed via blind stick - Seldinger approach).

After the catheter placement via fluoroscopy, the port is surgically fixed under the clavical, with the skin layer being sutured and dressings applied. The port can be accessed with a special needle (such as a Huber needle) and used for bolos or continuous infusion. The scar that forms is similar to a pacemaker implant scar.

After the initial healing phase, patients may resume normal activity between use, i.e., exercise, swimming, showering and bathing. If the port is not used for intravenous infusion or blood collection, it must be irrigated approximately every month with a heparing solution to keep the catheter from clotting off. As long as the catheter is patent and not clotted off, the port may be left in place and used for vascular access over a number of months of years repeatedly. The patient may still receive magnetic resonance imaging studies with most brands, and they infrequently set off metal detectors at airports. The ports can be surgically removed under local anesthesia if they are no longer needed.

Ports are not for everybody, but they may save you from frequent sticks, bruising and hematomas in patients requiring frequent intravenous therapy.