medicine's stepchild

Lymphedema has all but been ignored by U.S. physicians. But, fortunately, times are changing.

By George Gilman, MD

Called the stepchild of medicine, lymphedema has all but been ignored by U.S. physicians. The consequences of this ignorance are severe.

A story bears this out. One of my patients, a breast cancer survivor, was traveling in Munich, Germany, when her hand swelled. She had no idea what was wrong, so she went to a local doctor. "You have lymphedema," he said. "Here's a prescription for therapy." She went down the street to a therapist, who treated her three or four times, and the swelling went away.

About six weeks later, her hand swelled again. She went to her own doctor in the United States, who said, "There's nothing to be done for it." She started searching and found us.

This patient's lymphedema occurred because the axillary nodes were removed and the axilla was radiated, putting her at risk for lymphedema. About one-third of women who have this procedure eventually develop lymphedema. This usually occurs within one to five years after the treatment, but it can occur sooner or later than that.

The first indication is painless swelling that may start in the hand, but is more often in the soft tissues just above or below the elbow. This is the beginning of the first stage of lymphedema, when the swelling is caused by fluid accumulation. Characterized by reversibility, the swelling may go down with elevation, and pressure will make an imprint in the skin. This stage may last a few months to a few years.

In the second stage, part or all of the swelling is due to scarring in the tissues and build-up of abnormal cells. In this stage, the tissues become hard and don't fluctuate in size. These tissue changes are unique to lymphedema and are caused by the cellular reaction to the abnormally high amounts of serum protein in the tissues.

One of the most troubling complications of lymphedema is the tendency for recurrent infections. The tissues involved in lymphedema are immune-deficient. The increased concentration of protein in the tissues harms the cells of the immune system and makes an excellent medium for bacterial growth. The type of infection that's typical of lymphedema is cellulitis and may produce spreading infection that can cause severe illness within hours of its onset.

Patients with lymphedema should be aware of this since it is often puzzling to emergency room personnel when the patient has a red arm and a high fever. It helps if the patient can say, "I have lymphedema, this is cellulitis and I need antibiotics."

Arm lymphedema resulting from breast cancer and its treatment is the most prevalent form in the United States. Lower extremity lymphedema, which may arise from many causes, is more prevalent worldwide.

Reducing Limb Size

Although the first descriptions of the conservative method to treat lymphedema were published in 1890, we can trace its present form to the work of investigators in the 1960s and 1970s. This work laid the foundation for today's lymphedema treatment, a method that moves the edema fluid from the interstitial space back into the blood stream by way of the lymphatics. The method is termed Komplex Physicalische Enstauungstherapie (KPE) or complex physical decongestive therapy. KPE has effectively reduced the size of swollen limbs, reversing the tissue changes of lymphedema. It includes the following:

1. Manual lymph drainage (MLD). This is a specific patterned manual technique to stimulate the lymphatics and reduce the swelling. The lymphatic channels that are involved originate in the skin where most of the fluid forms and are carried in subcutaneous channels to the lymph nodes of the axillae and groins. The work consists mainly of light slow rhythmic stretching of the skin in circular movements at about 60 repetitions per minute. The movement of the skin accelerates the entrance of tissue fluid into the lymphatic channels, increasing the effectiveness of the pumping of the lymph trunks. The trunks carry the lymph through the filtering lymph nodes and eventually back into the bloodstream at the base of the neck. MLD is patterned to empty the most proximal lymphatics first and then progressively work outward. It's also patterned to guide the lymph from the blocked pathways into alternative routes, but it alone is not a treatment for lymphedema.

2. Compression by low-stretch material. This provides containment, which prevents enlargement of the arm and pressure. The lymphedema bandage must have strong containment and much weaker compression, described as high working pressure/low resting pressure. Ace bandages will not provide this; a special low stretch bandage is necessary. Elastic garments--sleeves and stockings--increase tissue pressure.

3. Exercise. When the limb is in compressive containment, exercise enhances the formation and movement of lymph. Because the lymphatic channels have valves, alternating compression and relaxation will pump the lymphatics just as it does the veins. The difference is the important veins lie inside the nonelastic fascia, so muscle contractions raise the pressure in the veins. But since the main lymphatics lie under the skin, they are not squeezed as much by the muscle contraction. Women can exercise, anything from calisthenics to sports to walking. Exercise without compression is controversial; some people use it as a treatment, and some advise against it because exercise produces increased fluid load. Lymphedema sometimes "blows up" after extra activity, especially prolonged unaccustomed activity.

4. Bandaging. Reducing the size of an edematous arm will involve bandaging, since this is the strongest form of compression. Because bandages are hard to put on and uncomfortable to wear, we use them only for a short time, usually during the intensive phase, which lasts about a month. The patient will typically have one or two MLD sessions daily and keep the bandages on for 23 hours a day. This will rapidly reduce the limb size at first, then more gradually. When the size of the arm no longer changes, the patient can wear a compression garment. Compression preserves the improvement following MLD. It increases tissue pressure, which reduces tissue fluid formation and increases reabsorption to maintain the reduction

During the maintenance phase, patients must wear the compression garment during the day and use a bandage at night. They should exercise while wearing the garment and perform self-massage. If women immediately discontinue wearing the compression garment, the arm will quickly resume its former size.

KPE can only be successful when patients meticulously follow the regimen. When they do, they may continue to see the limb reduce in size. Within six to 12 months, women can typically discontinue using nighttime compression. By then, the arm tissues will have remodeled to the new size. The tissue changes are slowly reversible, and the prolonged practice of self-massage may produce improved flow over the lymphatic byways that carry the lymph.

Because reducing the size of a large arm is so laborious, prolonged and labor-intensive, interest is now growing to start treatment early. At our clinic, patients participate in a five-day treatment program.

They receive a daily treatment of massage and bandaging and learn the anatomy and physiology of lymphedema, along with how the treatment works. They also learn self-massage, self-bandaging and return after two weeks to be measured for a compression garment. Knowledge, self-massage and bandaging give patients the tools to control lymphedema.

European Models

Although we've progressed in treating lymphedema, we lagged behind Europe for many years. More than a decade ago, U.S. medical schools didn't teach students about lymphedema. There was nothing in the CPT or ICD9 codes, and none of the scientific papers appeared in mainstream journals. Patients with lymphedema went home and lived with their swollen limbs.

This started to change about 10 years ago when Robert Lerner, a vascular surgeon, visited the Foldi Clinic in German and opened a lymphedema clinic in New Jersey using Foldi-trained therapists. (See sidebar.) He subsequently opened a school with the curriculum modeled on the German requirements, now the Academy of Lymphatic Studies.

Bolstered by the need to educate patients and doctors, Saskia Thiadens opened San Francisco's National Lymphedema Network shortly thereafter. The NLN serves as an information center for patients, therapists and physicians. The three bi-annual NLN-sponsored conferences have helped uncover the hidden epidemic.

But we still have a long way to go.*

For more information about lymphedema, contact the NLN at (800) 541-3259.

George Gilman, MD, retired from a 35-year career as an orthopedic surgeon in 1995. The former medical director of the Lymphedema Clinic of Seattle, he attended the first English-speaking course at the Foldi Clinic and is now the Foldi American representative in this country.