Lymphedema is the buildup of lymph (a fluid that helps fight Infection and disease) in the fatty tissues just under the skin. Lymphedema is a common complication of cancer and cancer treatment and can result in long-term physical, psychological and social issues for patients.
The lymphatic system consists of a network of specialized lymphatic vessels and various tissues and organs throughout the body that contain lymphocytes (White Blood Cells) and other cells that help the body fight infection and disease. The lymphatic vessels are similar to veins but have thinner walls. Some of these vessels are very close to the skin surface and can be found near veins; others are just under the skin and in the deeper fatty tissues near the muscles and can be found near arteries. Muscles and valves within the walls of the lymphatic vessels near the skin surface help pick up fluid and proteins from tissues throughout the body and move the lymph in one direction, toward the heart. Lymph is slowly moved through larger and larger lymphatic vessels and passes through small bean-shaped structures called lymph nodes. Lymph nodes filter substances that can be harmful to the body and contain lymphocytes and other cells that activate the immune system to fight disease. Eventually, lymph flows into one of two large ducts in the neck region. The right lymphatic duct collects lymph from the right arm and the right side of the head and chest, emptying it into the large vein under the right collarbone. The left lymphatic duct or thoracic duct collects lymph from both legs, the left arm, and the left side of the head and chest, emptying it into the large vein under the left collarbone.
The lymphatic system collects excess fluid and proteins from the body tissues and carries them back to the bloodstream. Proteins and substances too big to move through the walls of veins can be picked up by the lymphatic vessels because they have thinner walls. Edema may occur when there is an increase in the amount of fluid, proteins, and other substances in the body tissues because of problems in the blood capillaries and veins or a blockage in the lymphatic system.
Lymphedema may be either primary or secondary.
Primary lymphedema is a rare inherited condition in which lymph nodes and lymph vessels are absent or abnormal.
Secondary lymphedema can be caused by a blockage or cut in the lymphatic system. Blockages may be caused by infection, cancer, or scar tissue from radiation therapy or surgical removal of lymph nodes. Lymphedema that is secondary to cancer is not usually life-threatening. This summary discusses secondary lymphedema.
Acute versus gradual onset lymphedema
There are four types of acute lymphedema.
The first type of acute lymphedema is mild and lasts only a short time, occurring a few days after surgery to remove the lymph nodes or after injury to the lymphatic vessels or veins just under the collarbone. The affected area may be warm and slightly red, but is usually not painful and gets better within a week.
The second type of acute lymphedema occurs 6 to 8 weeks after surgery or during a course of radiation therapy. This type may be caused by inflammation of either lymphatic vessels or veins. The affected area is tender, warm or hot, and red and is treated by taking anti-inflammatory drugs.
The third type of acute lymphedema occurs after an insect bite, minor injury, or burn that causes an infection of the skin and the lymphatic vessels near the skin surface. It may occur on an arm or leg that is chronically swollen. The affected area is red, very tender, and hot and is treated by taking antibiotics. Mild redness may continue after the infection.
The fourth and most common type of acute lymphedema develops very slowly and may become noticeable 18 to 24 months after surgery or not until many years after cancer treatment. The patient may experience discomfort of the skin; aching in the neck, shoulders, spine or hips caused by stretching of the soft tissues or overuse of muscles; or posture changes caused by increased weight of the arm or leg.
Temporary versus chronic lymphedema
Temporary lymphedema is a condition that lasts less than 6 months. The skin indents when pressed and stays indented, but there is no hardening of the skin. A patient may be more likely to develop lymphedema if he or she has one of the following:
- ·Surgical drains that leak protein into the surgical site.
- Temporary loss of lymphatic function.
- Blockage of a vein by a blood clot or inflammation.
Chronic (long-term) lymphedema is the most difficult of all types of edema to treat. The damaged lymphatic system of the affected area is not able to keep up with the increased need for fluid drainage from the body tissues. This may be caused by one of the following:
- Recurrence or spread of a tumor to the lymph nodes.
- Infection of and/or injury to the lymphatic vessels.
- Periods of not being able to move the limbs.
- Radiation therapy or surgery.
- Inability to control early signs of lymphedema.
- Blockage of a vein by a blood clot.
A patient who is in the early stages of developing lymphedema will have swelling that indents with pressure and stays indented but remains soft. Continued problems with the lymphatic system cause the lymphatic vessels to expand, allowing lymph to flow back into the body tissues and make the condition worse. Pain, heat, redness, and swelling result as the body tries to get rid of the extra fluid.
Patients with chronic lymphedema are at increased risk of infection. No effective treatment is yet available for patients who have advanced chronic lymphedema. Once the body tissues have been repeatedly stretched, lymphedema may recur more easily.
Factors that can lead to the development of lymphedema include radiation therapy to an area where the lymph nodes were surgically removed, a larger number of lymph nodes removed in surgery, and being older. The part of the body treated may affect where lymphedema occurs. Risk factors for lymphedema include the following:
- ·Radiation therapy to the neck regions.
- Scar tissue in the lymphatic ducts or veins, caused by surgery or radiation therapy.
- Cancer that has spread to the lymph nodes in the neck.
- Having an inadequate diet or being overweight. These conditions may delay recovery and increase the risk for lymphedema.
Specific criteria for diagnosing lymphedema do not yet exist. To evaluate a patient for lymphedema, a medical history and physical examination of the patient should be completed. The medical history should include any past surgeries, problems after surgery, and the time between surgery and the onset of symptoms of edema. Any changes in the edema should be determined, as should any history of injury or infection. Knowing which medications a patient is taking is also important for diagnosis.
Patients at risk for lymphedema should be identified early, monitored, and taught self-care. A patient may be more likely to develop lymphedema if he or she eats an inadequate diet, is overweight, is inactive, or has other medical problems. To detect the condition early, the following should be examined:
- Comparison of actual weight to ideal weight.
- Protein levels in the blood.
- Ability to perform activities of daily living.
- History of edema, previous radiation therapy, or surgery.
Other medical illnesses such as diabetes, high blood pressure, kidney disease, heart disease, or phlebitis (inflammation of the veins). It is important that the patient know about his or her disease and the risk of developing lymphedema. Poor drainage of the lymphatic system due to surgical removal of the lymph nodes or radiation therapy may make the affected area more susceptible to serious infection. Even a small infection may lead to serious lymphedema. Patients should be taught about skin care after surgery and/or radiation. Lymphedema can occur 30 or more years after surgery.
Lymphatic drainage is improved during exercise; therefore, exercise is important in preventing lymphedema. The doctor decides how soon after surgery the patient should start exercising. Physiatrists (doctors who specialize in physical medicine and rehabilitation) or physical therapists should develop an individualized exercise program for the patient.
Better recovery occurs when lymphedema is discovered early, so patients should be taught to recognize the early signs of edema and to tell the doctor about any of the following symptoms:
- Redness, swelling, or signs of infection.
Considerations for Teaching Patients Prevention and Control of Lymphedema
- Watch for signs of infection, such as redness, pain, heat, swelling, and fever.
- Call the doctor immediately if any of these signs appear.
- Do prescribed exercises regularly as instructed by the doctor or therapist.
- Keep regular follow-up appointments with the doctor.
Lymphedema is treated by physical methods and with medication. Physical methods include manual lymphatic drainage (MLD - a specialized form of very light massage that helps to move fluid from the end of the limb toward the trunk of the body) and cleaning the skin carefully to prevent infection. Lymphedema may be treated by combining several of these physical methods. This is known as complex physical therapy (or complex decongestive therapy). which consists of manual lymphedema treatment, individualized exercises, and skin care followed by a maintenance program. Complex physical therapy must be performed by a professional trained in the techniques.
Surgery for treating lymphedema usually results in complications and is seldom recommended for cancer patients.
Antibiotics may be used to treat and prevent infections. Other types of drugs such as diuretics or anticoagulants (blood thinners) are generally not helpful, and may make the problem worse.
Finding the exact cause of the swelling and treating it correctly is important. Edema often leads to infection, which then increases fluid and protein deposits in the tissues. If an infection is diagnosed, appropriate antibiotics should be given. Blood clots should be ruled out because massage and other therapy techniques to encourage drainage may cause the clots to move through the bloodstream and cause more serious heart or lung problems. If blood clots are found, they should be treated with anticoagulants.
Coumarin is a dietary supplement that has been studied as a treatment for lymphedema. In the United States, dietary supplements are regulated as foods, not drugs. Supplements are not required to be approved by the Food & Drug Administration (FDA) before being put on the market. Because there are no standards for manufacturing consistency, dose, or purity, one lot of dietary supplements may differ considerably from the next. Coumarin was once used in some foods and medications in the United States. It was found to cause liver damage, and its use in foods and medications in the United States has been banned since the 1950s. Coumarin is available in several countries, but has not been approved for use in the United States or Canada.
The nutritional status of the patient should be evaluated and appropriate dietary recommendations should be made. Blood protein levels and weight should be monitored regularly, and patients should be encouraged to eat protein-rich foods.
Patients with lymphedema may experience pain caused by the swelling and pressure on nerves; loss of muscle tissue and function; or scar tissue causing shortening of muscles and less movement at joints. Pain may be treated with medications, relaxation techniques; however, the most successful treatment is to decrease the lymphedema.
Edema can make tissues less able to take in nutrients. Therefore, patients with lymphedema should be monitored for areas of skin breakdown, especially over areas with very little tissue between the skin and bone.
Patients with lymphedema who are also taking opioids may develop bowel problems. Bowel and bladder status should be monitored regularly for any signs of urine retention or constipation
The added stresses associated with lymphedema may interfere with treatment that is often painful, difficult, and time-consuming.
Coping is also difficult for patients with painful lymphedema. Patients with lymphedema may be helped by group and individual counseling that provides information about ways to prevent lymphedema, the role of diet and exercise, and emotional support.
In addition to the complications associated with chronic lymphedema noted in previous sections, a rare but fatal complication of lymphedema is lymphangiosarcoma, a tumor of the lymphatic vessels. After a patient develops lymphangiosarcoma, the average survival time is a little more than 1 year.
The cause of lymphangiosarcoma is not known. Death usually results from metastasis to the lungs.