Lymphedema

Lymphedema is a chronic, debilitating condition in which the flow of the fluid called lymph is disrupted, resulting in its accumulation in the tissues which causes swelling (edema). It usually affects any one of the limbs but, in some cases, both the arms or both the legs may swell.

Lymphedema is most commonly caused due to damage or complete removal of a lymph node as a part of cancer treatment. It can be classified as primary or secondary lymphedema.

Primary lymphedema is present from birth whereas, secondary lymphedema develops as a result of damage or dysfunction of the lymphatic system. While there is no cure for lymphedema, compression treatments, and physical therapy may help reduce the swelling and discomfort.

At early stages of lymphedema, symptoms like feeling of heaviness, tingling, tightness, warmth, or shooting pains in the affected extremity can occur. These symptoms can be present even before any noticeable swelling in the affected area.

Other signs and symptoms of lymphedema include:

• A decreased ability to see or feel the veins or tendons in the extremities
• Swelling of a part or all of the arm or leg, including fingers or toes
• Restricted range of motion
• Aching or discomfort
• Hardening and thickening of the skin (fibrosis)
• A feeling tightness or heaviness
• Redness of the skin
• Asymmetrical appearance of the extremities
• Tightness or reduced flexibility in the joints
• Slight puffiness in the skin

Causes of lymphedema vary depending on its type.

The lymphatic system is crucial for keeping the body healthy. It circulates protein-rich lymph fluid throughout the body for collecting bacteria, viruses and waste products.

The lymph fluid carries these harmful substances by the lymph vessels to the lymph nodes for filtering them out by the lymphocytes into the blood and ultimately to flush them out of the body. Lymphocytes are the infection-fighting cells that are abundant in the lymph nodes.

Lymphedema occurs when the lymph vessels are unable to adequately drain lymph fluid, usually from an arm or a leg.

Lymphedema can be either primary or secondary.

When it occurs on its own, it is termed as primary lymphedema whereas, when it occurs as a result of another disease or condition, it is known as secondary lymphedema. Primary lymphedema is present from birth whereas, secondary lymphedema develops as a result of damage or dysfunction of the lymphatic system and is comparatively more common.

Causes of Secondary lymphedema:

Any condition or procedure that damages lymph nodes or lymph vessels can cause lymphedema but, the most common causes include:

• Surgery - Complete removal or an injury to the lymph nodes or lymph vessels during a surgery (as in the case of breast cancer surgery) may hinder the flow of lymph and can result in lymphedema.
• Radiation - Radiation can cause scarring and inflammation in the lymph nodes or lymph vessels. It usually occurs during radiation treatment for cancer.
• Cancer - When cancerous cells grow in such a number near lymph nodes or vessels that they can block the passage of the lymph fluid, they can cause lymphedema.
• Infection - Spread of a parasitic infection or inflammation to the lymph nodes or lymph vessels can also hinder proper lymph drainage thus resulting in lymphedema. Infection-related lymphedema is more common in tropical and subtropical regions and is more likely to occur in developing countries.
• Medication - Certain medicines like tamoxifen (Nolvadex) can also cause lymphedema.

Causes of Primary lymphedema

As mentioned earlier, primary lymphedema is present from birth but develops very rarely. It is caused due to abnormalities during the development of lymph vessels.

Associated causes of primary lymphedema include:

• Milroy's disease (congenital lymphedema) - This is a genetic condition which results due a mutation in a gene. The genetic mutation leads to the development of abnormal and small lymphatic vessels or no development of the lymphatic vessels.
• Meige's disease (lymphedema praecox) - This type of lymphedema usually develops around puberty or during pregnancy, though it can occur later, until age 35. In addition to legs, it may affect other parts of the body like arms, face, and larynx. It is the most commonly occurring type of primary lymphedema.
• Late-onset lymphedema (lymphedema tarda) - This type of primary lymphedema occurs rarely and usually begins after age 35. It usually affects legs, but may affect other body parts as well.

While diagnosing lymphedema, the doctor will ask the patient about his condition in detail. It is recommended that the patient should make a note of every detail of his condition prior meeting the doctor. 

A person should consult a doctor as soon as he/she notices any abnormality in the body.

Some of the important information which the doctor may ask, includes:

• Your symptoms, including any that, may seem unrelated to the reason for which you scheduled the appointment.
• Key personal information, including major illnesses, cancer treatments or recent life changes.
• Medications, vitamins and supplements you take.
• When did the swelling begin?
• Has the swelling been continuous or occasional?
• Does anything seem to make the swelling better?
• Does anything seem to worsen your symptoms?

Based on the information provided by the patient, the doctor will analyse if the patient is at a risk of developing lymphedema. For instance, if the patient recently had cancer surgery involving lymph nodes, he/she is at a high risk of developing lymphedema and the doctor may diagnose lymphedema based only on the signs and symptoms.

But if the cause of lymphedema is not as obvious, the doctor may ask for imaging tests to get a look at the lymphatic system. These tests may include:

• MRI scan: Magnetic Resonance Imaging (MRI) is a diagnostic test which uses a magnetic field and radio waves to produce 3-D, high-resolution images of the organs and structures inside the body.
• CT scan: Computed tomography scan is an imaging technique which makes the use of computer-processed combinations of many X-ray images taken from different angles to produce detailed cross-sectional images of the scanned part of the body. CT scans can reveal blockages in the lymphatic system.
• Doppler ultrasound: Doppler ultrasound is a diagnostic test that uses high-frequency sound waves (ultrasound) to measure the amount of blood flow through the arteries and veins, usually those that supply blood to the arms and legs.
• Radionuclide imaging: Radionuclide imaging of the lymphatic system is also known as lymphoscintigraphy. The patient is injected with a radioactive dye and then scanned by a machine to produce images of the dye moving through the lymph vessels and highlighting blockages.

Although there is no permanent cure for lymphedema, the treatment focuses on reducing the swelling and controlling the pain.

The treatment approach will involve:

• Exercise: Regular exercise which involves light contraction of the arm or leg muscles to help stimulate lymph to flow, could be prescribed by the doctor or physical therapist. A certified lymphedema therapist can teach effective exercises that can help in coping with the disease.
• Bandage: Wrapping your arm or leg with a bandage is also helpful in lymphedema management. The bandage should be tightest around the fingers or toes and loosen as it moves up to the affected limb. A lymphedema therapist can show how to wrap the limb, correctly.

• Manual lymph drainage: A special massage technique called manual lymph drainage may encourage the flow of lymph fluid out of the arm or the leg. But it is highly recommended that it should be done by a specialist who is trained in the technique and should only be done with a doctor's prescription.

  This massage therapy is not recommended if the patient has a skin infection, active cancer, blood clots, and congestive heart failure. Massage on the body parts that have received radiation therapy should be avoided.

• Pneumatic compression: In this technique, sleeves or stockings connected to a pump are worn over the affected limb. The pump provides sequential compression from the end of the extremity toward the body. These may be used in the clinic or at home and are useful in preventing long-term scarring. This technique cannot be used for all individuals, for instance, patients with congestive heart failure, deep venous thrombosis, or certain infections should avoid this therapy.
• Compression garments: Long sleeves or stockings which are specially made to compress the arm or leg to encourage the flow of the lymph fluid out of the affected limb can also be used. Wearing a compression garment of a correct fit while exercising the affected limb is more beneficial.
• Complete decongestive therapy (CDT): This approach associates combining therapies with lifestyle changes. CDT is not recommended for those people who have high blood pressure, diabetes, paralysis, heart failure, blood clots or acute infections.
• Surgery: In severe cases of lymphedema, the doctor may consider surgery to remove excess tissue from the affected arm or leg to reduce swelling.

It is not possible to completely prevent lymphedema.

If a person is at a risk of developing lymphedema, following steps may help him/her in reducing the probability of lymphedema incidence:

• Adopt a healthy lifestyle and take proper care of your body, especially limbs.
• Eat a healthy diet.
• Exercise regularly.
• Maintain hygiene.
• Maintain a healthy weight.
• Prevent skin infections; if you have an infection, get proper treatment immediately.
• Do not allow blood to be drawn from the arm which is at a risk of lymphedema.
• Do not allow vaccines or other shots to be given in the arm which is at a risk of lymphedema.
• Do not allow medical personnel to put a blood pressure cuff around the arm which is at a risk of lymphedema.
• If you are undergoing cancer treatment or any other surgery which involves damage to the lymph nodes or lymph vessels, consult your doctor and take necessary measures.

Some measures which could help you in coping with lymphedema include:

• Consult a doctor as soon as any of the above-mentioned symptoms appear. Do not ignore even any slight increase of swelling in the arm, hand, fingers, neck or chest wall.
• Protect your arm or leg from sunburn. Using a sunscreen lotion of appropriate SP is an effective protection.
• Wear gloves when gardening or doing housework.
• 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.
• If possible, avoid medical procedures, such as blood draws and vaccinations, in your affected limb.
• Exercise Regularly. Stretching is helpful but avoid strenuous activity until you have fully recovered from surgery or radiation treatment for cancer.
• Do not apply ice or heat to your affected limb. Protect your affected limb from extreme cold or heat.
• Avoid tight clothing.
• Avoid anything that could constrict your arm or leg, such as tight fitting clothing and blood pressure cuffs.
• Clean your skin daily, looking over every inch of your affected limb for signs of trouble, such as cracks and cuts.

There are several risks and complications associated with lymphedema.

Factors that may increase the risk of developing lymphedema include:

• Cancer
• Cancer treatment including surgery and radiation therapy
• Excess weight or obesity
• Rheumatoid or psoriatic arthritis
• Pre-mature aging 

Lymphedema can lead to serious complications as described below:

• Infections: Possible infections that can result from lymphedema include cellulitis (a serious bacterial infection of the skin) and lymphangitis (an infection of the lymph vessels). The smallest injury to the arm or leg can become an entry point for infection.
• Lymphangiosarcoma: This rare form of soft tissue cancer can result from the most severe cases of untreated lymphedema.