Lymphedema

Introduction

Lymphedema refers to swelling that occurs most often in your arms or legs. It may affect just one arm or leg, but sometimes lymphedema can involve both arms or both legs.

The swelling occurs when a blockage in your lymphatic system prevents the lymph fluid in your arm or leg from draining adequately. As the fluid accumulates, the swelling continues.

No cure for lymphedema exists. But lymphedema can be controlled. Controlling lymphedema involves diligent care of your affected limb.

Signs and symptoms

Lymphedema is a type of abnormal swelling of an arm or leg. Swelling ranges from mild, hardly noticeable changes in the size of your limb to extreme swelling that can make it impossible to use the affected arm or leg.

Lymphedema symptoms include:

• Swelling of part of your arm or your entire arm or leg, including your fingers or toes
• A feeling of heaviness or tightness in your arm or leg
• Restricted range of motion in your arm or leg
• Aching or discomfort in your arm or leg
• Recurring infections in your affected limb
• Hardening and thickening of the skin on your arm or leg

Your body's lymphatic system, part of which is seen here, is part of your immune system, which protects you against infection and disease. It includes your spleen, thymus, bone marrow, lymph nodes and lymph channels, as well as your tonsils and adenoids.

Lymphedema is swelling in an arm or leg. In rare circumstances it affects both arms or both legs.

Causes

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 these 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 from your 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).

Causes of primary lymphedema
Primary lymphedema is a rare, inherited condition caused by problems with the development of lymph vessels in your body. Primary lymphedema occurs most frequently in women and usually affects the legs, rather than the arms. Specific causes of primary lymphedema include:

• Milroy disease (congenital lymphedema). This is an inherited disorder that begins in infancy and causes a malformation of your lymph nodes, leading to lymphedema.
• Meige disease (lymphedema praecox). This hereditary disorder causes lymphedema in childhood or around puberty. It causes your lymph vessels to form without the valves that keep lymph fluid from flowing backwards, making it difficult for your body to properly drain the lymph fluid from your limbs.
• Late-onset lymphedema (lymphedema tarda). This occurs rarely and usually begins after age 35.

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

• Surgery can cause lymphedema to develop if your lymph nodes and lymph vessels are removed or severed. For instance, surgery for breast cancer may include the removal of one or more lymph nodes in your armpit to look for evidence that cancer has spread. If your remaining lymph nodes and lymph vessels can't compensate for those that have been removed, lymphedema may result in your arm.
• Radiation treatment for cancer can cause scarring and inflammation of your lymph nodes or lymph vessels, restricting flow of the lymph.
• Cancer cells can cause lymphedema if they block lymphatic vessels. For instance, a tumor growing near a lymph node or lymph vessel could become large enough to obstruct the flow of the lymph fluid.
• Infection can infiltrate your lymph vessels and lymph nodes, restricting the flow of lymph fluid and causing lymphedema. Parasites also can block lymph vessels. Infection-related lymphedema is most common in tropical and subtropical regions of the globe and is more likely to occur in undeveloped countries.
• Injury that damages your lymph nodes or lymph vessels also can cause lymphedema.

When to seek medical advice

Make an appointment with your doctor if you notice any persistent swelling in your arms, legs or lymph nodes.

Screening and diagnosis

Your doctor may try to rule out other causes of swelling in order to arrive at a diagnosis of lymphedema. Swelling can have many causes, including a blood clot or an infection that doesn't involve your lymph nodes.

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

If the cause of your lymphedema isn't as obvious, your doctor may order imaging tests to determine what's causing your signs and symptoms. To get a look at your lymphatic system, your doctor may use an imaging technique, such as:

• 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 areas where the lymph fluid is blocked.
• Magnetic resonance imaging (MRI). This scan gives your doctor a better look at the tissues in your arm or leg. He or she might be able to use an MRI to see characteristics of lymphedema.
• Computerized tomography (CT). A CT scan produces images of your arm or leg in cross sections.
• Doppler ultrasound. This variation of the conventional ultrasound assesses blood flow and pressure by bouncing high-frequency sound waves (ultrasound) off red blood cells.

Complications

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

• Infections. Lymphedema makes your affected arm or leg particularly vulnerable to infections, including cellulitis and lymphangitis. Any injury to your arm or leg provides an entry point for an infection.
• Elephantiasis. This condition occurs when your arm or leg becomes so hardened with thickened skin that you have difficulty moving it. Elephantiasis may make the skin on your arm or leg very weak, leading to chronic ulcers and repeated infections.
• Lymphangiosarcoma. This rare form of soft tissue cancer can result from the most severe cases of untreated lymphedema. Lymphangiosarcoma originates in the lymph nodes and lymph vessels.

Treatment

Lymphedema can't be cured. Treatment focuses on minimizing the swelling and controlling the pain. Lymphedema treatments include:

• Exercises. Light exercises that require you to move your affected arm or leg may encourage movement of the lymph fluid out of your limb. These exercises shouldn't be strenuous or make you tired. Instead, they should focus on gentle contraction of the muscles in your arm or leg. Exercises help pump the lymph fluid out of your affected limb. Your doctor or a physical therapist can teach you exercises that may help.
• Wrapping your arm or leg. Bandages wrapped around your entire limb encourage lymph fluid to flow back out of your affected limb and toward the trunk of your body. When bandaging your arm or leg, start by making the bandage tightest around your fingers and toes. Wrap the bandage more loosely as you move 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. Manual lymph drainage involves special hand strokes on your affected limb to gently move lymph fluid to healthy lymph nodes, where it can drain. Massage isn't for everyone. Avoid massage if you have a skin infection, active cancer, blood clots or congestive heart failure. Also avoid massage on areas of your body that have received radiation therapy.
• Pneumatic compression. If you receive pneumatic compression, you'll wear a sleeve over your affected arm or leg. The sleeve is connected to a pump that intermittently inflates the sleeve, putting pressure on your limb. The inflated sleeve gently moves lymph fluid away from your fingers or toes, reducing the swelling in your arm or leg.
• Compression garments. Compression garments include long sleeves or stockings made to compress your arm or leg to encourage the flow of the lymph fluid out of your affected limb. Once you've reduced swelling in your arm or leg through other measures, your doctor may suggest you wear compression garments to prevent your limb from swelling in the future. 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.

In cases of severe lymphedema, your doctor may consider surgery to remove excess tissue in your arm or leg. While this reduces severe swelling, surgery can't cure lymphedema.

Prevention

If you're at risk of developing secondary lymphedema, you can take measures to help prevent it. If you've had or are going to have cancer surgery, ask your doctor whether your particular procedure will involve your lymph nodes or lymph vessels. Ask if your radiation treatment will be aimed at any of your 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 any injury to your affected limb. Cuts, scrapes and burns can all invite infection, which can cause lymphedema. 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, avoid heavy activity with that limb. Early 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 heat, such as with a heating pad, to your affected limb.
• Elevate your arm or leg. When you get a chance, elevate your affected limb.
• 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 readings. Ask that your blood pressure be taken in your other arm.
• Keep your arm or leg clean. Make skin care and nail care high priorities. Inspect the skin on your arm or leg every day, keeping watch for changes or breaks in your skin that could lead to infection.

Coping skills

It can be frustrating to know that no cure exists for lymphedema. But if you find yourself getting down about the daily bandaging or constant need to protect your affected limb, know that 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 helps you better understand the signs and symptoms you experience. The more you know, the better you can 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 full of fruits and vegetables. Exercise daily, if you can. Reduce the stress in your life that you can control. Try to get enough sleep so that you wake up refreshed each morning. Taking care of your body gives you more energy, encourages healing and helps you control your lymphedema.
• 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. They can also put you in touch with other people with lymphedema with whom you can connect via e-mail or letter.

If you feel frustrated or overwhelmed by lymphedema, talk to your doctor or other health care provider about how you feel. He or she may be able to address your concerns.