WHAT is lymphedema?

• Lymphedema is the buildup of fluid in parts of your body because of a problem with your lymphatic system. Your lymphatic system is designed to reabsorb, filter fluid from tissues and direct it back to the blood circulation. If the lymphatic system is damaged or blocked, fluid will build up and cause swelling (Figure 1). The most common parts of the body affected by lymphedema are the arms, legs, head and neck, and genitals (Figures 2 and 3). Unless something is done to help your lymphatic system work as it should, this swelling continues to worsen, resulting in a cascade of inflammation, swelling, and ultimately scarring (fibrosis).
• What is the lymphatic system? The lymphatic system is a network of tiny vessels that acts as a sponge that removes fluid from tissues. Sources of fluids are swelling from trauma, infection, natural leaking from arteries or veins. The lymphatic system collects fluid, filters it and directs it back to the circulation. If the "sponge" is not working appropriately, the "unpure" fluid builds up, weeps through the skin, and can become a source of infection. Just imagine swimming in a pool with a broken filter!
• Lymphedema generally cannot be prevented by vaccines or cured by medication, nor does it just disappear. Diuretics (water pills) are not effective in removing lymphedema swelling. The goal of lymphedema treatment is to control the swelling caused by lymphedema and keep other problems from developing or getting worse. Following your clinicians' instructions is one of the most important factors to control the swelling. 

Fig.1 Normal leg and leg with lymphedema (fluid build up). Lymphedema may develop when the local lymphatic system is damaged or blocked

Fig. 2. Pictures show different severity levels of lymphedema of the arms. Severity level increases from left to rightFig. 3. Pictures show different severity levels of lymphedema of the legs. Severity level increases from left to right

WHAT causes lymphedema?

• Lymphedema is caused by a problem/ disruption of the lymphatic system that a person may be born with, called primary lymphedema. But more commonly it is a result of injury to the lymphatic system (due to surgery, radiation, cancer, infection or trauma), called secondary lymphedema. The most common cause of lymphedema in the United States is secondary lymphedema.

WHAT increases the risk of having lymphedema?

• Some risk factors for lymphedema of the arms are:
• Sedentary lifestyle leading to body mass index (BMI) in the overweight or obese category [2][3][4]
• Receiving surgery or radiation affecting the lymphatic system in the axilla or arms during treatment of cancer [2][3][4]
• Developing swelling or skin infection in the arm after cancer surgery [2][5][6]

• Some risk factors for lymphedema of the legs are:
• Sedentary lifestyle leading to body mass index (BMI) in the overweight or obese category [2][3][7][4]
• Surgery (e.g. knee replacement, hip replacement), accident, or radiation therapy affecting lymph nodes of the legs [2][3][7][4]
• Repeated infection (e.g. cellulitis)
• Having veins in the leg that do not work well and result in swelling of the legs (i.e., chronic venous hypertension) [1][8]
  
• Other diseases such as diabetes, heart and kidney disease [4]

WHAT are the symptoms of lymphedema?

• The symptoms of lymphedema are not always the same in every person, but they can include [9]:
• Swelling in your arms/legs, head and neck, or genitals. If your arms/legs are affected, one of your limbs can be bigger than the other one. Clothing and jewelry will start to feel tighter.
• Heavy feeling in your arms/legs, head and neck, or genitals.

HOW is lymphedema diagnosed?

• Your doctor can find out if you have lymphedema by talking to you, doing an exam, and measuring the size of your swollen body part. It is important to make sure there are no other causes of swelling, such as infections or blood clots. If it is not clear if the swelling is due to lymphedema, other tests may be ordered by your provider.

WHAT happens if lymphedema is left untreated?

• If lymphedema is not treated, symptoms such as swelling and heaviness can get worse and lead to complications. The most common complications are skin infections and skin breakdown (non-healing ulcer) causing fluid leakage.[1] Chronic lymphedema left untreated results in severe scarring and fibrosis of the involved extremities.

HOW can lymphedema be managed?

The goal of lymphedema treatment is to control the swelling caused by lymphedema and keep other problems from developing or getting worse.

• Different types of interventions are indicated according to the severity of the lymphedema. All lymphedema cases are first treated with physical therapy and conservative methods, such as:
• Compression of the swollen area with special lymphedema bandages or garment (Figure 4). These put a controlled amount of pressure on the swollen area to prevent fluid from building up, and need to be applied by a wound care/lymphedema specialist.
• Skin and nail care
• A type of massage that helps the lymphatic system work better called manual lymphatic drainage (MLD)
• Exercise prescribed by the lymphedema specialist
• Weight loss

• After the initial swelling has decreased, it is important to continue to use compression garments or bandages to prevent fluid from building up again.
• If lymphedema can not be controlled with the measures above, surgery might be an option for a few select patients. 
• As for managing complications of lymphedema: 
• For Infections: skin infections should be treated as soon as they appear with antibiotics, pain medication, and other treatments as indicated by your doctor. Compression bandages to decrease edema should be maintained during and after treatment.
  
• For skin breakdown (ulcer): your clinician may give you instructions on how to care for the ulcer at home. Wound care includes cleansing of the ulcer, removal of dead skin and tissue, control of fluid leakage, protection of the skin around the ulcer. See more details on wound care in topic "When is my wound going to heal?"

HOW can you prevent lymphedema from getting worse?

To prevent lymphedema from getting worse, follow instructions for self-care [10]:

• Careful skin and nail care helps prevent infection:
• Keep skin creases and nails clean, use neutral soap.
• Use moisturizing skin creams to prevent dry or cracking skin. 
• Avoid injury to the skin, such as cuts, bites, scrapes or sunburn. If injury occurs wash the skin well and monitor for swelling
• Protect the affected arm/leg with clothes and gloves when working outdoors
• Avoid blocking the flow of fluids through the body:
• Avoid tight clothing or jewelry
• Avoid positions or movements that might cut off blood supply to your arm/leg (carrying heavy bags, sitting cross legged)
• Avoid blood pressure measurements in the affected arm/leg
• Keep blood from pooling in the affected limb
• Keep limb with lymphedema raised higher than the heart when possible
• Do not apply heat to the affected limb
• Maintain a healthy BMI and adopt carefully controlled exercises, as taught by lymphedema specialists.

WHEN to call your healthcare provider

• Call your healthcare provider if you have an increase in swelling, redness or pain, if your skin changes, breaks or starts leaking fluid, if you have a fever or any other new symptoms.

Frequently Asked Questions (FAQ)

• Q: As a cancer survivor, what is my risk of getting lymphedema?
• A: For patients with breast cancer, the risk of getting lymphedema varies from 2-50% depending on the surgical procedure used to treat the cancer.[11][12][13] For patients with malignant melanoma, the risk is about 5-30%.[14] For patients with cancer of the female reproductive tract, the risk is about 5-50%.[14] For patients with cancer of the male reproductive organs or the bladder, the risk is about 5-20%.[15]
• Q: Does radiation affect my risk of getting lymphedema?
• A: Yes, radiation is known to directly damage your lymphatic network, which can cause lymphedema. However, this risk varies from person to person and it is difficult to accurately predict.
• Q: Can I exercise if I have lymphedema?
• A: Yes, exercise is in fact part of the treatment of lymphedema. It is important however to follow only the exercises taught by your lymphedema specialist. These exercises are designed to avoid excessive strain to your arms/legs during exercise and to avoid positions or movements that might cut off blood flow to your arms/legs.
• Q: Will this ever go away?
• A: Unfortunately, lymphedema is a disease that will not get better on its own. It may get worse over time and cannot be cured by medication. However, physical therapy lowers the chance of lymphedema getting worse, improves symptoms and maximizes the return of your body’s normal function. This is especially true if lymphedema is caught early and if you, the patient, are actively involved in your treatment plan over time.

Insurance information

• Caring for lymphedema requires regular visits with your primary care provider and with a physical or occupational therapist (PT/OT) who specializes in lymphedema.
• Here is a list of things you should talk about with your insurance company [16]:
• Coverage for primary care provider visits
• Coverage for visits with a physical therapist/ occupational therapist
• Out of pocket co-pays or caps to outpatient services
• If an insurance plan requires you to go to a specific facility, make sure in-network therapists are trained in lymphedema. If necessary, ask your primary care provider to help you by making an out-of-network referral

For more information

• For more information about lymphedema, contact your healthcare team or cancer treatment center.
• Online resources include:
• National Cancer Institute information for patients
• National Comprehensive Cancer Network information for patients
• Lymphedema support groups, via LymphNotes.com