Port for hemophilia treatment

Parents’ Guide to CVADs

What you need to know about ports and other infusion devices for children with bleeding disorders
Author: Bridget Murray Law

For many parents of children who are newly diagnosed with hemophilia, those first few months of treatment can seem like a blur of bleeds, bruising and relentless needle pricks.

That’s why many parents make the decision to have their child use a central venous access device (CVAD) to make infusions easier. Doctors surgically implant these devices under the skin to allow frequent, sometimes daily, injections.

Types of CVADs

These are the most commonly used types of CVADs:

Port (internal CVAD)

A port is a small device that is surgically implanted under the skin, usually in the upper chest above the heart. The Port-A-Cath is a common version of this device. To infuse, a person places a special needle into a container, or reservoir, connected to a catheter inserted into a blood vessel near the heart. This popular CVAD can be used for several years and requires no dressings or external lines. Infections can occur, however, so the infusion site must be kept completely sterile.

Nontunneled (external) central venous catheter

Doctors insert this type of catheter into a vein near the heart, usually through the chest, for short-term use. Factor product is injected into a cap on the end of the tube, typically located on the arm or chest. Dressings over the cap are necessary to prevent the external site from becoming infected. They must be changed often to keep the infusion area sterile. The device can only be used for several weeks or months.

Tunneled central venous catheter

These catheters, which include the brand-name Hickman and Broviac catheters, are surgically implanted under the skin and inserted into a vein in the neck or chest. Infusions are done through the external end of the catheter. The catheter can stay implanted for a year or more.

Peripherally inserted central catheter (PICC)

A PICC is inserted into a vein in the arm and connected to a large blood vessel in the chest. Infusions are done through the external end of the catheter. The devices are cost-effective and easy to install, but they can only be used for several weeks or months.

Know the Pros and Cons

The biggest advantage of CVADs is that they help take the guesswork out of locating hard-to-find veins. They also enable more frequent infusions and more independence from medical staff, alleviating some trips to the hospital.

But there can also be CVAD complications, too. The most immediate risk from ports is excessive bleeding when they are implanted surgically. But the more common complications are blood infection and clotting.

Since a port is foreign material implanted in the body and accessed frequently from the outside, it can easily get infected. When this happens, bacteria grow rapidly in the bloodstream, causing dangerous contamination and fever.

Children need to be closely monitored if they have a fever, especially if it’s not explained by another childhood illness. If a blood culture comes up positive, they should be placed on IV antibiotics immediately to treat the infection.

Being vigilant about sterilizing the port site helps cut the risk, so nurses thoroughly train parents on how to cleanse and flush the port.

A hematoma, or bruise, can occur on the surface (or septum) of the port device. It is caused by leakage of blood from the port to underneath the skin when the needle is removed from the port. If this occurs, the port should not be used, since the accumulated blood is an excellent growth media for bacteria, and may lead to an infection. A hematoma is more likely with frequent, such as daily, access, or in individuals with inhibitors. However, this can easily be prevented by applying direct pressure over the puncture site once the needle is removed. Pressure should be applied for 10 minutes.

Clotting is another common problem with access devices. Up to half of patients with ports develop blood clots in the vein accessed by the port’s main line. Some patients develop clots in the device itself. Flushing the port with a blood-thinning medication can help prevent clots and possible infection. Physicians may also need to use blood thinners to treat clots. In some cases, the only way to break up the clot is to remove the port.

Other problems can develop when a port’s line, or catheter, separates from its reservoir. As a result, factor can leak into tissue surrounding the port. The port’s line can also get twisted, in which case the entire device must be reinstalled.

Making the Decision

When it’s time to decide if your child should have a CVAD, consult with your child’s doctor and consider the following questions:

Is the hemophilia severe? Is there an inhibitor?

Children with mild or moderate hemophilia generally do not require the multiple infusions that warrant a CVAD, the nurses say. Most CVAD candidates have severe hemophilia. However, a person who develops an inhibitor will likely need a CVAD regardless of severity because of the need for immune tolerance therapy. Even some adults with inhibitors opt for CVADs.

How close is the hospital?

Healthcare access is part of the decision-making equation. If, for example, a child needs to infuse only twice weekly and the hospital is 5 miles away, nurses can easily travel to your home to help you infuse. However, if you’re hundreds of miles from a healthcare facility or hemophilia treatment center and your child needs daily infusions, a CVAD might be a better option.