Lymphedema involves blockage of the lymph vessels, with a resulting accumulation of lymphatic fluid in the interstitial tissues of the body. The lymphatic system consists of lymph vessels and lymph nodes throughout the body. The lymph vessels collect lymphatic fluid, which consists of protein, water, fats, and waste from cells. The lymph vessels transport the fluid to the lymph nodes, where waste materials and foreign materials are filtered out from the fluid. The fluid is then returned to the blood. When the vessels are damaged or missing, the lymph fluid cannot move freely throughout the system but accumulates. This accumulation of fluid results in abnormal swelling of the arm(s) or leg(s), and occasionally swelling in other parts of the body.
Lymphedema is a very serious condition. There is no cure for lymphedema and once it develops, it can be a long-term, uncomfortable, and sometimes painful condition requiring daily treatment. When lymphedema is not treated, the protein-rich fluid continues to accumulate, leading to even more swelling and hardening (referred to as fibrosis) of the tissues. This fluid is a good culture medium for bacteria, thus resulting in reoccurring infections when there are injuries to the skin, decrease or loss of functioning of the affected limbs, and skin breakdown. Infections, referred to lymphangitis, can affect the connective tissue under the skin. Repeated infections may result in scarring, which in turn makes the tissue susceptible to more swelling and infection. Over time, these infections result in tissue hardening (i.e., fibrosis), which is a characteristic of advanced chronic lymphedema. In very severe cases, untreated lymphedema may even result in a rare form of lymphatic cancer called lymphangiosarcoma.
Lymphedema affects approximately 100 million people worldwide, including at least 3 million people in the United States.
Symptoms of lymphedema include:
- swelling of an affected limb, which may develop gradually or suddenly
- tightness of the skin and a feeling of heaviness in the affected area
- discomfort or a feeling of “pins and needles” in the affected area
- pitting edema, which can be identified by observing a temporary indentation in the swollen area when pressure is placed on the affected area
- aching in the adjacent shoulder or hip due to the increasing weight of the swelling limb
- tight fitting of a ring, wristwatch, or bracelet, without a gain in weight.
In 90% of the cases, lymphedema is diagnosed through observations, measurements, and symptoms. The remaining 10% require the use of more complex diagnostic tests such as lymphoscintigraphy. Lymphoscintigraphy is a technique in which a radioactive substance that concentrates in the lymphatic vessels is injected into the affected tissue and is mapped using a gamma camera, which images the location of the radioactive tracer.Magnetic resonance imaging (MRI), computed tomography (CT) scanning, and duplex ultrasound are imaging techniques that are also sometimes used as diagnostic tools for lymphedema.
There are three stages of lymphedema:
- Stage 1 (spontaneously reversible)—tissue is still at the pitting stage and soft to the touch. Upon waking in the morning, the limbs or affected areas are of normal or almost normal size
- Stage 2 (spontaneously irreversible)—tissue is non-pitting and no longer soft to the touch, fibrosis begins to form, and the limbs increase in size
- Stage 3 (lymphostatic elephantiasis)—swelling is irreversible and the affected areas are very swollen. The skin hardens and begins to break down, fibrosis is more extensive, and patients may need surgery to remove some of the swollen tissues.
Primary lymphedema is an inherited condition, where the patient is born without lymph vessels and nodes. The swelling associated with primary lymphedema usually occurs during adolescence and affects the foot or calf. A rare form of primary lymphedema, called Milroy’s Disease, occurs inpregnancy. However, secondary lymphedema, or acquired lymphedema, develops as a result of an injury to the lymph system. Specific causes include surgical treatments for certain types of cancers, especially those cancers that currently require the removal of lymph nodes.
Radiation treatment for cancer or for some AIDs-related diseases such as Kaposi-Sarcoma may also result in lymphedema, as radiation may damage or destroy lymph nodes or cause the formation of scar tissue that can interrupt the normal flow of the lymphatic fluid. Specific cancers and their treatment that may result in lymphedema include malignant melanoma, breast (in both women and men), gynecological, head and neck, prostate, testicular, bladder, and colon cancer. Other causes of lymphedema include trauma to the lymphatic system from burns, liposuction, tattooing, injuries, surgery, radiation, obesity, heart or circulatory disease, and multiple sclerosis. Lymphedema in people at risk may not develop the condition immediately, but develop the condition weeks, months, or even years later.
Aircraft travel has been linked to the development of lymphedema in patients after cancer surgery, possible due to the decreased cabin pressure.
In Western countries, one of the most common causes of lymphedema is mastectomy with axillary dissection (removal of the breast and underarm lymph tissue for treatment of breast cancer), which may result in lymphedema of the breast, underarm, or arm on the side of the surgery in 10-20% of patients. This occurs because the lymphatic drainage of the arm passes through the axilla (armpit), and tissue in the axilla is removed during the mastectomy. To reduce the risk of developing lymphedema after breast cancer treatment, there is an alternative treatment that avoids axillary lymph node dissection.
Sentinel lymph node biopsy is a new diagnostic procedure used to determine whether the breast cancer has spread (metastasized) to axillary lymph nodes. A sentinel lymph node biopsy requires the removal of only one to three lymph nodes for close review by a pathologist. If the sentinel nodes do not contain tumor (cancer) cells, this may eliminate the need to remove additional lymph nodes in the axillary area. Early research on this technique indicates that sentinel lymph node biopsy may be associated with less pain and fewer complications than standard axillary dissection. Because the procedure is so new, long term data are not yet available. However, there is still a risk for developing lymphedema because of follow-up radiation treatments or chemotherapy, which may also damage the lymph nodes.
Persons who have developed lymphedema after cancer treatment should be checked for a possible reoccurrence of cancer if they experience a sudden increase of swelling, for the tumor growth may be responsible for blocking lymphatic flow.
Lymphedema is a chronic condition that cannot be cured, but it can be improved with treatment. There are several major components of a lymphedema treatment program, which should be administered by the health care provider in cooperation with a physical therapist trained in lymphedema treatment. Complete Decongestive Therapy (CDT; also referred to as Complex Decongestive Therapy (CDT) or as Complete Decongestive Physiotherapy (CDP)) combines manual lymph drainage (MLD) with compression techniques and with patient education on self-care needs. The goals of the treatment program are to:
- remove the stagnant lymph fluids out of the tissues
- reduce and help control swelling
- soften fibrotic tissue
- improve the overall health of the patient.
However, some lymphedema specialists feel that lymphedema patients with metastatic cancer should not be treated with CDT, to prevent the spreading of the cancer.
MLD was developed in 1932 in Denmark by a doctor and his wife. It was widely used in Europe and now is accepted as a therapy for lymphedema patients in the United States. In MLD a series of rhythmic, light strokes are made in a specific sequence along the lymphatic vessels and the adjoining tissues. These movements remove the lymph fluids from the tissues and return them to the circulatory system, thus reducing swelling in the affected area.
Compression techniques include the use of compression garments, compression aids, and compression bandages. These techniques encourage natural drainage and prevent swelling by supporting tissues in a way that aids in drainage. Compression garments are knit, stretch sleeves or stockings. Compression aids are custom-fitted sleeves, stockings, or pads made of fabric-covered foam. Bandages are an effective and flexible means of compression. They work when the patient is active or is resting and can easily be adjusted to fit changing limb sizes. However, the bandage should be a special type of short-stretch bandage and not the long-stretch bandage that is commonly known as Ace bandages. Only persons who are trained in lymphedema therapy should tape or wrap swollen areas.
Self-care techniques are practiced by the patient or his or her caregiver at home, between visits to the therapist. Self-care techniques include self-massage, skin care to maintain healthy tissue, nutritious diet, and exercise to increase lymph flow, increase mobility, and to improve the patient’s general health.
Exposure to extreme heat has the potential to increase lymphedema swelling, so an affected person or a person at risk of developing lymphedema should avoid hot tubs, saunas, and steam rooms.
To keep the affected extremities as healthy as possible, a person with lymphedema should keep the swollen areas clean and avoid heavy lifting and pulling as well as avoid any type of trauma, such as cuts, bruises, sunburn or other burns, injections, sports injuries, insect bites, or cat scratches. Some doctors and lymphedema therapists recommend that a person with lymphedema use a preventative course of antibiotics when having dental treatment, that is, starting antibiotics several days before the appointment and continuing several days afterwards. A person at risk of developing lymphedema (for example, a woman who has been treated for breast cancer) should also observe the same type of precautions to prevent the development of the condition.
If infections occur, then all treatments for lymphedema should be discontinued while the infection is present, and the infection treated with antibiotics.
Surgery is sometimes used to remove excess tissue (“debulking”) if the swollen limb becomes so large and heavy as to interfere with movement.
Exercise is important for a person with lymphedema, but only in moderation. If the extremity starts to ache, the person should lie down and elevate the swollen limb. Recommended exercises include walking, swimming, light aerobics, bike riding, and yoga.
Persons with lymphedema should wear a lymphedema alert bracelet or necklace for safety during a medical emergency, explaining the risk of infections. They may also benefit from counseling and membership in support groups to deal with the psychological impact of the disease. Sometimes patients with lymphedema will be denied insurance coverage for treatment; as a result patient advocacy groups in 2005 are attempting to get a law passed through the U.S. Congress guaranteeing insurance coverage for lymphedema.
Alternative and complementary therapies
The use of clinical aromatherapy in conjunction with CDT may improve the quality of life for persons with lymphedema. Clinical aromatherapy involves the use of essential oils to improve the functioning of the immune system, for the immune system is closely associated with the lymphatic system. Also a massage oil comprised of a blend of frankincense, grapefruit, hyssop, and lavender, may be used to soften scarred and fibrotic tissues.
Radiation treatments can cause skin contractures, which can be helped by massage with a blend of cajeput, frankincense, hyssop, lavender, sage, and tea tree. Radiation can also have adverse effects on the bowel, resulting in poor bowel functioning, scarring, and activity restrictions. Massaging the abdomen with a blend of grapefruit, fennel, helichrysum, lavender, myrrh, and sage may improve intestinal functions. When compression techniques are used, the underlying skin can be treated with a blend of bay laurel, chamomile, geranium, helchrysum, lavender, patchouli, and vetiver in a combination of castor oil, safflower oil, and grapeseed oil as carrier oils. Good skin care is important in preventing infections.
Body oils that contain cajeput, cypress, lavender, marjoram, and rosewood can be applied after bathing to keep the skin moist and healthy. Finger nail beds can be a portal of entry for infections, so can be kept moist with an essential oil blend of chamomile, geranium, lavender, lemon, sage, tea tree, and ylang ylang.
Axillary nodes — Lymph nodes found in the armpit that drain the lymph channels from the breast.
Clinical aromatherapy — Aromatherapy is the therapeutic use of plant-derived, aromatic essential oils to promote physical and psychological well-being. It is sometimes used in combination with massage and other therapeutic techniques as part of a holistic treatment approach.
Debulking — General term used for surgeries in which subcutaneous tissue is removed from lymphodemous limb.
Fibrosis — Formation of fibrous tissue as a reaction or as a repair process; may occur due to treatment and/or disease. in lymphedema condition known as hardening of the limb with resulting restriction of circulatory flow, increased infection, and weeping sores.
Fibrotic — Pertaining to or characterized by fibrosis. In dermatological description, “fibrotic” would be used to describe leathery, bound-down, or thickened, scarred skin.
Interstitial fluid — The fluid between cells in tissues. Referred to as the liquid subtance of the body.
Interstitial space — The fluid filled areas that surround the cells of a given tissue; also known as tissue space.
Long-stretch bandages — Specialized bandages, similar to an Ace bandage, that have 100 to 200% stretch.
Low-stretch bandage — Specialized bandages, with 30 to 90% stretch, that are used to obtain the correct compression during the treatment of lymphedema; also known as short-stretch bandages.
Lymph — The almost colourless fluid that bathes body tissues and is found in the lymphatic vessels that drain the tissues of the fluid that filters across the blood vessel walls from blood. Lymph carries antibodies and lymphocytes (white blood cells that help fight infection) that have entered the lymph nodes from the blood.
Lymph nodes — Small bean-shaped organs of the immune system, distributed widely throughout the body and linked by lymphatic vessels. Lymph nodes are garrisons of B, T, and other immune cells.
Lymph System — When sickness or infection invades the body, the immune system is the first line of defense. A big part of that defense is the lymph system. Lymph is carried through the body by lymph vessels that have valves and muscles to help move the fluid. Along the route are lymph nodes that serve as filters for harmful substances. This network of vessels and nodes together is called the lymph system.
Lymphatic fluid — The clear fluid found outside the cells which bathes the tissues. It is collected, filtered, and transported by the lymphatic system from around the tissues to the blood circulatory system. Fluid that collects as a result of lymphedema.
Nail beds — The underlying connective tissue that nourishes the finger and toenails.
Pitting edema — When a swollen area is pressed, the pressure leaves an indentation (pit) that takes time to fill back in.
Sentinel node biopsy — A newer procedure performed in order to determine whether breast cancer has spread to auxiliary (underarm) lymph nodes. A blue radioactive tracer and/or blue dye is injected into the area of the breast tumor. The lymphatic vessels carry the dye or radioactive material, to a “sentinel node”. This sentinel node is thought to be the first lymph node receiving fluid from the tumor and the one most likely to contain cancer cells if the cancer has spread. Only if the sentinel node contains cancer cells are more lymph nodes removed.
Skin contracture — A permanent tightening of the skin that prevents normal movement of the associated body part and that can cause permanent deformity. A contracture develops when the normally elastic connective tissues are replaced by inelastic fibrous tissue. This makes the affected area resistant to stretching and prevents normal movement.