PXE and Intermittent Claudication

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By Mary Krieger, R.N.

One of the major systems in the body affected by PXE is the cardiovascular system-- the heart and blood vessels. Arteries carry oxygen rich blood from the left side of the heart throughout the entire body. Normally, arteries are strong and flexible, and the inner walls are smooth allowing blood to flow freely to muscles, skin, tissues, and organs. As a person ages, the walls of the arteries may become clogged with clumps of fat and other materials, called plaques. The arteries become hardened and narrow, and this slows down the flow of blood. This condition is known as atherosclerosis, and individuals with PXE may develop clogged, hardened arteries in their late 20's or early 30's.

While atherosclerosis can occur in arteries anywhere in the body, it most commonly affects the peripheral arteries (arteries to the arms and legs) in PXE. The pulses in the wrists and feet may be weak or absent. When the flow of blood to leg muscles is decreased, it can cause pain, cramping, or heaviness in the legs which is called intermittent claudication. The word claudicatio in Latin literally means "to limp." The leg discomfort is "intermittent" because it begins with exercise (like fast walking or climbing stairs) and subsides when exercise stops. The reason for this is that the muscles in the legs need more oxygen when they are working. When blood flow is decreased, the oxygen supply to the muscles is also decreased. So if the demand for oxygen is greater than the supply, pain or cramping in the muscle occurs.

If the narrowing of the arteries is severe enough, leg pain may also occur while at rest. The toes may appear pale or bluish, and they may be cool to the touch. In severe cases, sores or ulcers can develop on the lower legs, ankles, or feet.

How is intermittent claudication diagnosed?

To diagnose the cause of leg pain or cramping, the physician may perform a few simple tests:
1) Ask about diet, smoking habits, and medical conditions that occur in the family
2) Check the pulses in the arms, groin, knees, and feet
3) Check the arms and legs for coolness of the skin, hair loss, or non-healing sores
4) A simple test called the Ankle-Brachial Index can be performed which measures the blood pressure in the ankle and arm and compares the two.
5) Treadmill walking at a slowly increasing speed to determine when the leg pain first occurs
6) Other tests such as an ultrasound may also be used.

What can be done about intermittent claudication?

1) Eat a balanced, low fat diet with plenty of fruits and vegetables to prevent any further buildup of fatty deposits in the blood vessels
2) Keep your blood cholesterol level within the normal range
3) Do NOT smoke
4) If you have high blood pressure or diabetes, see your physician regularly to treat these conditions.
5) A regular, supervised walking program, under the direction of your physician, is one of the MOST effective treatments to reduce the symptoms of claudication, and to increase the distance and time that you are able to walk comfortably.
6) Certain medications can increase blood flow in the legs and decrease leg pain caused by poor circulation. If you have PXE, be sure that your treating physician is aware of this condition.

Tell me more about the exercise program that helps with claudication...

These are some of the general guidelines for exercise rehabilitation programs for persons with claudication. You should consult your personal physician before starting any exercise program.

1) Warm up for 5-10 minutes prior to exercise and cool down for 5-10 minutes afterwards
2) Walking on a treadmill or around a track is considered to be the most effective for claudication.
3) Walk at a speed that produces mild claudication symptoms within 3-5 minutes. When the symptoms become moderately severe, rest for a short time, either sitting or standing, until the symptoms subside
4) Continue the walk-rest-walk pattern throughout the session. The first session will optimally include 35 minutes of intermittent walking. Then the amount of walking is increased by 5 minutes each session until 50 minutes of intermittent walking is achieved.
5) This exercise should be done 3 to 5 times per week.

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Sources:
Walk this way: Managing the pain of intermittent claudication. Mayo Foundation for Medical Education and Research http://www.mayoclinic.com/

Stewart KJ, Hiatt WR, Regensteiner JG, Hirsch AT. Exercise training for claudication. N Engl J Med. 2002 Dec 12;347(24):1941-51)

For reliable information on atherosclerosis, peripheral arterial disease, and other medical conditions, visit the MedlinePlus web site: http://medlineplus.gov/