Lymphedema

Your lymphatic system is crucial to keeping your body healthy. It circulates protein-rich lymph fluid throughout your body, collecting bacteria, viruses and waste products. Your lymphatic system carries this fluid and harmful substances through your lymph vessels, which lead to lymph nodes. The wastes are then filtered out by lymphocytes — infection-fighting cells that live in your lymph nodes — and ultimately flushed from your body.

Lymphedema occurs when your lymph vessels are unable to adequately drain lymph fluid, usually from an arm or leg. Lymphedema can be either primary or secondary. This means it can occur on its own (primary lymphedema), or it can be caused by another disease or condition (secondary lymphedema). Secondary lymphedema is far more common than primary lymphedema.

Causes of secondary lymphedema

Any condition or procedure that damages your lymph nodes or lymph vessels can cause lymphedema. Causes include:

• Surgery. Removal of or injury to lymph nodes and lymph vessels may result in lymphedema. For example, lymph nodes may be removed to check for spread of breast cancer, and lymph nodes may be injured in surgery that involves blood vessels in your limbs.
• Radiation treatment for cancer. Radiation can cause scarring and inflammation of your lymph nodes or lymph vessels.
• Cancer. If cancer cells block lymphatic vessels, lymphedema may result. For instance, a tumor growing near a lymph node or lymph vessel could enlarge enough to block the flow of the lymph fluid.
• Infection. An infection of the lymph nodes or parasites can restrict the flow of lymph fluid. Infection-related lymphedema is most common in tropical and subtropical regions and is more likely to occur in developing countries.

Causes of primary lymphedema

Primary lymphedema is a rare, inherited condition caused by problems with the development of lymph vessels in your body. Specific causes of primary lymphedema include:

• Milroy's disease (congenital lymphedema). This disorder begins in infancy and causes lymph nodes to form abnormally.
• Meige's disease (lymphedema praecox). This disorder often causes lymphedema around puberty or during pregnancy, though it can occur later, until age 35.
• Late-onset lymphedema (lymphedema tarda). This occurs rarely and usually begins after age 35.

Risk factors

Factors that may increase your risk of developing lymphedema after cancer, from cancer treatment or from other secondary causes include:

• Older age
• Excess weight or obesity
• Rheumatoid or psoriatic arthritis

Complications

Lymphedema in your arm or leg can lead to serious complications, such as:

• Infections. Possible infections that can result from lymphedema include a serious bacterial infection of the skin (cellulitis) and an infection of the lymph vessels (lymphangitis). The smallest injury to your arm or leg can be an entry point for infection.
• Lymphangiosarcoma. This rare form of soft tissue cancer can result from the most-severe cases of untreated lymphedema. Possible signs of lymphangiosarcoma include blue-red or purple marks on the skin.

Prevention

If you have had or you are going to have cancer surgery, ask your doctor whether your procedure will involve your lymph nodes or lymph vessels. Ask if your radiation treatment will be aimed at lymph nodes, so you'll be aware of the possible risks.

To reduce your risk of lymphedema, try to:

• Protect your arm or leg. Avoid injury to your affected limb. Cuts, scrapes and burns can invite infection. Protect yourself from sharp objects. For example, shave with an electric razor, wear gloves when you garden or cook, and use a thimble when you sew. If possible, avoid medical procedures, such as blood draws and vaccinations, in your affected limb.
• Rest your arm or leg while recovering. After cancer treatment, exercise and stretching are encouraged. But avoid strenuous activity until you've recovered from surgery or radiation.
• Avoid heat on your arm or leg. Don't apply ice or heat, such as with a heating pad, to your affected limb. Also, protect your affected limb from extreme cold.
• Elevate your arm or leg. Whenever possible, elevate your affected limb above the level of your heart.
• Avoid tight clothing. Avoid anything that could constrict your arm or leg, such as tightfitting clothing and, in the case of your arm, blood pressure cuffs. Ask that your blood pressure be taken in your other arm.
• Keep your arm or leg clean. Make skin and nail care high priorities. Inspect the skin on your arm or leg daily, watching for changes or breaks in your skin that could lead to infection. Don't go barefoot.

If you're at risk of lymphedema — for instance, if you've recently had cancer surgery involving your lymph nodes — your doctor may diagnose lymphedema based on your signs and symptoms.

If the cause of your lymphedema isn't as obvious, your doctor may order imaging tests to get a look at your lymph system. Tests may include:

• MRI scan. Using a magnetic field and radio waves, an MRI produces 3-D, high-resolution images.
• CT scan. This X-ray technique produces detailed, cross-sectional images of your body's structures. CT scans can reveal blockages in the lymphatic system.
• Doppler ultrasound. This variation of the conventional ultrasound looks at blood flow and pressure by bouncing high-frequency sound waves (ultrasound) off red blood cells. Ultrasound can help find obstructions.
• Radionuclide imaging of your lymphatic system (lymphoscintigraphy). During this test you're injected with a radioactive dye and then scanned by a machine. The resulting images show the dye moving through your lymph vessels, highlighting blockages.

There's no cure for lymphedema. Treatment focuses on reducing the swelling and controlling the pain. Lymphedema treatments include:

• Exercises. Light exercises in which you move your affected limb may encourage lymph fluid drainage and help prepare you for everyday tasks, such as carrying groceries. Exercises shouldn't be strenuous or tire you but should focus on gentle contraction of the muscles in your arm or leg. A certified lymphedema therapist can teach you exercises that may help.

• Wrapping your arm or leg. Bandaging your entire limb encourages lymph fluid to flow back toward the trunk of your body. The bandage should be tightest around your fingers or toes and loosen as it moves up your arm or leg. A lymphedema therapist can show you how to wrap your limb.

• Massage. A special massage technique called manual lymph drainage may encourage the flow of lymph fluid out of your arm or leg. And various massage treatments may benefit people with active cancer. Be sure to work with someone specially trained in these techniques.

  Massage isn't for everyone. Avoid massage if you have a skin infection, blood clots or active disease in the involved lymph drainage areas.

• Pneumatic compression. A sleeve worn over your affected arm or leg connects to a pump that intermittently inflates the sleeve, putting pressure on your limb and moving lymph fluid away from your fingers or toes.

• Compression garments. Long sleeves or stockings made to compress your arm or leg encourage the flow of the lymph fluid out of your affected limb. Wear a compression garment when exercising the affected limb.

  Obtain a correct fit for your compression garment by getting professional help. Ask your doctor where you can buy compression garments in your community. Some people will require custom-made compression garments.

  If you have difficulties putting on or taking off the compression garment, there are special techniques and aids to help with this; your lymphedema therapist can review options with you. In addition, if compression garments or compression wraps or both are not an option, sometimes a compression device with fabric fasteners can work for you.

• Complete decongestive therapy (CDT). This approach involves combining therapies with lifestyle changes. Generally, CDT isn't recommended for people who have high blood pressure, diabetes, paralysis, heart failure, blood clots or acute infections.

In cases of severe lymphedema, your doctor may consider surgery to remove excess tissue in your arm or leg to reduce swelling. There are also newer techniques for surgery that might be appropriate, such as lymphatic to venous anastomosis or lymph node transplants.

Coping and support

It can be frustrating to know there's no cure for lymphedema. However, you can control some aspects of lymphedema. To help you cope, try to:

• Find out all you can about lymphedema. Knowing what lymphedema is and what causes it can help you communicate with your doctor or physical therapist.
• Take care of your affected limb. Do your best to prevent complications in your arm or leg. Clean your skin daily, looking over every inch of your affected limb for signs of trouble, such as cracks and cuts. Apply lotion to prevent dry skin.
• Take care of your whole body. Eat a diet rich in fruits and vegetables. Exercise daily, if you can. Reduce stress. Try to get enough sleep. Taking care of your body gives you more energy and encourages healing.
• Get support from others with lymphedema. Whether you attend support group meetings in your community or participate in online message boards and chat rooms, it helps to talk to people who understand what you're going through. Contact the National Lymphedema Network to find support groups in your area. The organization can also put you in touch with other people with lymphedema.