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What Is Lymphedema?

Lymphedema is a swelling of a body part, most often the extremities. It may also occur in the face, the trunk, the abdomen or the genital area. Lymphedema is the result of an accumulation of protein-rich fluid in the superficial tissues, which can have significant pathological and clinical consequences for the patient if left untreated. Once present, this chronic and progressive condition will not disappear again.

Causes of Lymphedema

Lymphedema is classified as either primary or secondary.

Primary lymphedema is caused by congenital malformations of the lymphatic system and may be present at birth or develop later in life, often in puberty or during pregnancy. Primary forms usually affect the lower extremities but may also be present in upper extremities.

Secondary lymphedema is more common and often the result of surgery or radiation therapy for cancer. Surgical procedures in combination with the removal of lymph nodes, such as mastectomies or lumpectomies with the removal and/or irradiation of axillary lymph nodes, are a very common reason for the onset of secondary lymphedema in the United States. Other causes include trauma or infection of the lymphatic system. Severe venous insufficiencies may also contribute to the onset of lymphedema (phlebolymphostatic edema)

Primary and secondary lymphedema may affect the upper or lower extremity. In general it can be said that the legs are more often involved in primary lymphedema whereas secondary forms are more commonly found in the upper extremities.

Symptoms of Lymphedema

Early stages of lymphedema (stage 1) may be temporarily reduced by simple elevation of the limb. Without proper treatment, however, the protein-rich swelling causes a progressive hardening of the affected tissues; this condition is known as lymphostatic fibrosis and is present in stage Il lymphedema. Other complications such as fungal infections, additional hardening, and very often an extreme increase in volume of the swollen extremity are typical for stage IlI lymphedema. Primary and secondary lymphedema usually affect one extremity only; if both extremities, for example, both legs, are involved, the swelling appears asymmetrically.

Treatment Methods

Medication: Diuretics are often prescribed in order to control lymphedema but are proven to have very poor long-term results in the treatment of this condition. Diuretics decrease the water content of the swelling while the protein molecules remain in the tissues. As soon as the diuretic loses its effectiveness, these proteins continue to draw water to the edematous area.

Surgery: Several surgical procedures for lymphedema are described. It is safe to say that not a single one of these surgeries performed during the past century showed consistent results.

Pneumatic Compression Pumps: This mechanical device works with sleeves containing compressed air, which are applied to the patient's swollen extremity. Inappropriate use of these devices can cause serious complications in lymphedema patients. In some cases pumps may be applied under the supervision of specially trained therapists and in combination with other treatment modalities (see below).

Complete Decongestive Therapy (CDT): Since there is no cure for lymphedema, the goal of the therapy is to reduce the swelling and to maintain the reduction. For the majority of the patients this can be achieved by the skillful application of this therapy, which is safe, reliable, and noninvasive. CDT shows good long-term results in both primary and secondary lymphedema; it consists of two phases and the following combined modalities:

Manual Lynph Drainage (MLD): this gentle manual treatment technique increases the activity of certain lymph vessels and manually moves interstitial fluid. Applied correctly, a series of MLD treatments decreases the volume of the affected extremity to a normal or near normal size and is applied daily in the first phase of the therapy.

Compression Therapy: the elastic fibers in the skin are damaged in lymphedema. In order to prevent reaccumulation of fluid it is necessary to apply sufficient compression to the affected extremity. 

Compression therapy also improves the function of the muscle pumps, helps to reduce fibrotic tissue, and promotes venous and lymphatic return. In the first phase of CDT, Compression therapy is achieved with the application of special short-stretch bandages. These bandage materials are used between MLD treatments and prevent the reaccumulation of Iymph fluid, which has been moved out of the extremity during the MLD session. Once the extremity is decongested, the patient wears compression garments during the day. In some cases it may be necessary for the patient to additionally wear bandages at night. In order to achieve best results, specially trained personnel should take measurements for these elastic support garments; incorrectly fitted sleeves or stockings will have negative effects. The type of garments (round or flat-knit style) and the compression class depend on many factors such as the patient's age and the severity of the swelling, For upper extremity lymphedema, compression classes I (20-30 mmHg)or II (30-40 mmHg): for lymphedema of the lower extremities, compression classes II, IlI (40-50 mmHg) or IV(> 50 mmHg) are suitable. In some cases it may be necessary to apply compression class III to an upper extremity or an even greater compression than class IV to a lower extremity lymphedema. This can be achieved by wearing two stockings on top of each other, or by the application of bandages on top of a stocking To have the maximum effect, garments must be worn every day and replaced after 6 months.

Exercises: a customized exercise program is designed by the therapist for each patient. These decongestive exercises aid the effects of the joint and muscle pumps and should be performed by the patient wearing the compression bandage or garment. Vigorous movements or exercises causing pain must be avoided Exercises should be performed slowly and with both the affected and nonaffected extremity.

Skin care: the skin in lymphedema is very susceptible to infections and usually dry. A low-pH lotion, free of alcohol and fragrances, should be used to maintain the moisture of the skin and to avoid infections. You should consult your physician if there are any fungal infections present in your affected extremity.

Do's and Don'ts

Avoid any injuries to the skin  which could lead to a worsening of your lymphedema. Listed below are just a few general guidelines: be careful working in the garden, playing with your pets or doing housework. Avoid the use of scissors to cut your nails and don't cut your cuticles. Injuries, even small ones, may cause infections.

Avoid mosquito bites - wear insect repellents when outdoors. A single mosquito bite can cause an infection.

Use caution when exercising - avoid movements that overstrain; discuss proper exercises and activities with your therapist.

Avoid heat - very hot showers, hot packs on your extremity, sunbathing, and the use of saunas could have a negative effect on your lymphedema.

Avoid extreme changes in temperature (hot/cold), massages ("Swedish") on your affected extremity or any cosmetics that irritate the skin.

Inform all health care personnel that you have lymphedema - injections or acupuncture in your affected extremity should be avoided. Blood pressure should be taken on the extremity free of lymphedema.

Nutrition is important - there is no special diet for lymphedema. Today most nutritionists recommend a low-salt, low-fat diet. Obesity may have a negative effect on your swelling.

Travel - avoid mosquito-infested areas; when traveling by airplane apply an additional bandage on top of your garment.

Clothing - clothing that is too tight may restrict the proper flow of lymph. Avoid tight bras, panties or socks and make sure your jewelry fits loosely.

See your doctor - if you have any signs of an infection (fever, chills, red and hot skin), fungal infections or if you notice any other unusual changes that may be related to your lymphedema.

General tips - always wear your compression garments during the day and if necessary your bandages at night; elevate your extremity as often as possible during the daytime; perform your exercises daily and always consult your doctor or therapist should you have any questions about your lymphedema. 

Please visit the National Lymphedema Network and its complete information for those at risk of lymphedema at: https://lymphnet.org/position-papers

And here is another page of FAQ's regarding lymphedema: https://store.acols.com/FAQ.php