This is a new initiative for FIAR that will provide background and information on this rare but debilitating and potentially lethal condition.
What is it?
Lymphedema is the swelling of the arms or legs due to a back up of lymph fluid. This swelling can also occur in the face, chest, abdomen, back and genitals. The lymphatic system is part of the circulatory system and consists of and intricate network of lymph vessels and nodes. These organs transport a fluid made up of water and protein called lymph. The lymph fluid bathes the tissues of the body, and then it is pumped back into the lymphatic system, where the vessels carry the water back to the heart and blood and the wastes products are processed in the lymph nodes.
The lymphatic system is a system “that collects tissue fluid and returns it to the blood” Taber’s Cyclopedic Medical Dictionary, Edition 18, 1997. It is an intricate circulatory system made up of lymph vessels, lymph nodes and lymph ducts. Lymph nodes can be found in clusters in the neck, armpits, groin and knees. They are soft bean-shaped organs that can become enlarged when bacteria exists in the system. Other organs included in the lymph system are the tonsils, adenoids, spleen, thymus gland and bone marrow. Just as blood circulates within the blood vessels, lymph fluid which is clear to white, circulates through the lymph vessels. Lymph fluid is made up of chyle (fluid from the intestines after digestion), red blood cells and white blood cells (lymphocytes) which attack bacteria.
The lymphatic system protects and maintains the fluid environment of the body by creating, filtering and transporting the lymph. It carries processed fats and proteins from the intestines and enzymes, hormones and other substances into the blood stream. It returns 60% of the fluid that “leaks” out of the blood capillaries into the interstitial space between cells during normal metabolism. Lymph nodes, along with the thymus gland and bone marrow produce lymphocytes, which are critical to the immune system. They also filter out microorganisms, foreign matter, and toxins.
Lymphedema is “the abnormal accumulation of lymph in the interstitial spaces” Taber’s Cyclopedic Medical Dictionary, Edition 18, 1997. This disease occurs when there is damage or dysfunction of the lymphatic system which causes fluid to collect in the soft tissues and under the skin. Two types of lymphedema exist: primary lymphedema can be hereditary as studies show a break in two genes, the FOXC2 and VEGFC.
Lymphedema can be present at birth or appear early in life (Milroy’s Disease), occur during puberty (praecox) or in middle-age (tarda). It can also remain inactive (latent). Lymphedema present at birth is a result of a malformed lymphatic system, infection in utero or injury during birth. Secondary lymphedema is caused by damage or injury to the lymphatic system, due to infection from insect bites (lymphatic filariasis), serious wounds and surgery, biopsies or removal of nodes, and radiation for cancer. Lymphatic Filariasis is the leading type of lympedema worldwide, affecting an estimated 120 million people.
A person can be born with a compromised lymph system and lymphedema (primary) or develop this disease later in life (secondary) due to injury/trauma, surgery, chemotherapy and radiation treatments. It is an endemic condition located in tropical regions of the world. These areas include South East Asia, India, Africa and areas of South America.
Genital lymphedema is the swelling of the mons pubis and labia in women and the scrotum in men, due to the accumulation of lymph fluid in these areas.
A primary function of the lymphatic system is to defend your body against bacteria and foreign substances. The skin and mucous membranes impede bacteria, viruses and fungus from entering the body, making it very important that the skin and membranes remain healthy and intact. This is the first line of defense. Once an infectious organism penetrates these barriers, damaged cells send chemical signals that increase blood flow to an injured area causing inflammation. The heat destroys the germs, brings nutrient rich blood into the area along with lymph fluid, which contains white blood cells called monocytes that clean up dead germs, dead cells and waste. In an uncompromised, strong immune system, this inflammatory response is strong enough to stop further spread of bacteria, virus and fungus. If the inflammatory response does not resolve the invaders, the body’s complement system and immune response take over.
The complement system is made up of proteins produced by the liver that bind to bacteria and open its pores, allowing salts and water to enter the cell and causes it to swell and burst. This system kills microbes, assists the inflammatory response and the immune response as well. The immune response produces specific antibodies to a specific invader and contains a memory factor which allows the antibodies to act faster of this invader appears again. The antibodies are made up of proteins or a combination of proteins and polysaccharides that attach themselves to the antigen (intruder) and kill or inactivate them. Then different types of white blood cells move into action to rid the body of intruder.
Staging and Complications
In addition to classifying lymphedema as genetic or traumatic, there are stages of severity as well. Stage I is where the limb is of normal size when waking up in the morning and becomes swollen as the day wears on. Elevation reduces the swelling. Generally this stage is considered reversible. Stage II is where the limb is no longer of normal size even upon waking. Tissue quality becomes spongy and skin becomes thick and hard making it difficult to make an indentation with a finger. Stage III is where the limb becomes very large and swollen; the skin becomes bumpy, hard and thick. The potential for infection exists at every stage: the accumulation of high protein fluid is a perfect environment for bacteria to grow.
Lymphedema can affect one or both arms or legs, the face, abdomen, neck, eyelids and the genital area. In the past, lymphedema of the leg was considered a hereditary condition (primary) and lympedema of the arm the result of removal of lymph nodes for biopsies or cancer. This is not true. Primary and secondary lymphedema can transpire in the arms and legs. “Ten percent of cases of lymphedema of the leg can lead to genital lymphedema and some have genital lymphedema alone” . Genital lymphedema is the swelling of the scrotum and/or penis in men and the labia and mons pubis in the women. Some cases of genital lymphedema are acute and reversible, but most cases become chronic and irreversible. Primary genital lymphedema is rare.
Complications in lymphedema are many. Studies show that the areas of swelling are immune deficient and are susceptible to infection and inflammation of the connective tissues, skin and subcutaneous tissues and lymph vessels; fungus; sepsis and gangrene.
What are the treatments?
Treatment for lymphedema is multi-faceted. First, edematous areas are treated with a therapeutic massage called Manual Lymphatic Drainage (MLD) which assists the lymph vessels and nodes to pick up, remove and return lymph fluid back into the circulatory system. A therapist begins the treatment by using a light, feathery touch on the torso and neck to activate the lymph nodes, spleen and thymus. Next, a gentle, rhythmic pumping motion is applied to the affected limb, beginning where the limb meets the torso and gradually reaching the end of the limb, always focusing the movements toward the heart. Working in this manner allows the limb to empty from the top down and encourages the lymph to re-enter the circulatory system. Once the entire limb has been worked on, the therapist returns to the torso and applies the finishing compression and feather-like strokes. MLD relieves the pressure in the limbs the excess fluid creates, reduces pain and softens the fibrotic areas in the limb which are prone to infection. After the MLD treatment, the limb/area is moisturized and the areas are bandaged with ACE-like bandages to support the skin and tissues and stop the fluid from returning to the area. The affected limb should remain either bandaged or covered with a support garment until the next treatment. Skin care is essential in caring for lymphedematous areas. It is important to keep the skin clean, soft and unbroken so that further breakdown does not occur from the toxic lymph fluid. Antibiotic and antifungal creams are used to keep the skin free from infection, and a special ph balanced lotion is used to moisturize the limbs. Exercise plays significant role in maintaining a person with lymphedema healthy. Exercise activates the natural pumps in the muscles helping to further reduce the accumulation of lymph fluid in the tissues and returns the fluid to the circulatory system, thus, promoting a healthy circulatory and lymphatic system.
Treatment for lymphedema consists of manual lymph drainage, the use of compression bandages and garments, skin care, exercise and in severe cases, surgery. Manual lymph drainage (MLD) is a gentle massage of the limbs and torso that assists the lymphatic system to move the fluid. Compression bandages and garments are applied after MLD treatments to keep the fluid moving in the areas it would accumulate. Skin care is utterly important when lympedema occurs. The skin needs to be kept clean, dry and checked for cuts, bruises, bug bites or anything that might cause inflammation or infection. During exercise, the muscles act as pumps and move fluid out of swollen areas. Surgery becomes necessary especially when the genitals are involved. Surgery removes excess skin pockets which form due to the accumulation of fluid in the genitals.
Draining wounds allows an entry area for infection as well as breaking down of the skin surrounding the wound. Swelling causes the nerves to become compressed and the skin to harden and stretch causing pain and loss of movement and function. Morbid obesity is common because of the inability to move. Blood clots can arise due to increased pressure in the limbs and fluid can build up in the lungs and abdominal cavity. Then there is the possibility of cancer of the soft tissues and/or lymph organs and amputation if a limb is becomes so diseased the tissue dies. The emotional ramifications for people with lymphedema include depression, low self image and humiliation because of disfigurement, feeling helpless, hopeless or useless from possible job loss caused by the loss of mobility and function. The genitals can become edematous resulting in sexual dysfunction in both men and women.
How does it affect people with HIV?
Lymphedema affects people with HIV/AIDS mostly when they undergo radiation therapy for the treatment of cancer and AIDS- related diseases such as Kaposi Sarcoma. As a result of radiation therapy, healthy lymph vessels and nodes can become damaged and full of scar tissue which in turn obstructs the normal flow of the lymph fluid. This is especially dangerous for people HIV/AIDS because of the risk of opportunistic infections of the skin and organs.
Lymphedema often affects people with cancer. Cancer is what occurs when the cells in the body become abnormal and begin to divide without control. Cancer cells can invade surrounding tissues and spread to other parts of the body through the bloodstream and lymph system. One type of cancer that afflicts people with lymphedema is Kaposi’s Sarcoma (see N.S. McNutt, V. Fleisher and M.A. Conant, American Journal of Pathology, 1983 April). Kaposi’s Sarcoma (KS) is different from other cancers because it can appear in several parts of the body at the same time. Common places for KS to develop are on the skin or internal organs, specifically the lymph nodes, lungs and digestive system. KS tends to affect people with weak immune systems such as those with HIV/AIDS. Although KS has become less widespread thanks to the advances made in the treatment of HIV/AIDS, the risk increases as the HIV infection develops and weakens the immune system. KS cells block the lymph nodes and disturb the normal circulation of lymph fluid. Lymphedema can be an uncomfortable and distressing symptom of KS and allows the opportunity for infection which is a disastrous situation for those with HIV/AIDS.
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