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Lymphedema is a high protein edema which accumulates in the tissues of the body and often leads to a massive, unsightly limb.  Globally, there are an estimated 140 million cases of lymphedema (Casley-Smith & Casley-Smith, 1992), a condition prevalent among the growing number of cancer survivors.  In the breast cancer population, the incidence of lymphedema has been reported as ranging from 5% to 52% following dissection of axillary lymph nodes (Casley-Smith & Casley-Smith, 1991; Love, 1990).  The risk of swelling increases if the patient receives radiation treatment, and onset of lymphedema can occur several years after the completion of cancer treatments.

Other causes of lymphedema include traumatic injury to or removal of lymph nodes or vessels.  The incidence of congenital (also known as primary) lymphedema is unknown, although it is 10 times more likely to occur in women than men, 10 times more likely in legs than arms and may not appear until adolescence or later.

Pain, paresthesias, weakness, decreased range of motion and loss of functional abilities can all result from untreated chronic lymphedema.  The medical complications of lymphedema include secondary bacterial and fungal infections, recurrent cellulitis, and decreased immune system function.  Wound healing capabilities can be so severely hampered that something as minor as a paper cut can lead to a severe infection and increase in swelling (Casley-Smith, Morgan & Piller, 1993).

UNDERSTANDING LYMPHEDEMA

The Lymphatic System
The role of the lymphatic system is to remove impurities from the body’s tissues.  The system is composed of numerous vessels which carry waste products, proteins and excess water, or lymph fluid, from the tissues of the body proximally towards the trunk.  All lymph fluid also passes through lymph nodes located throughout the body, such as in the armpit, groin, neck, abdomen, elbow and knee (see Figure 1).  From the nodes the fluid is carried into the venous system where it is eventually removed from the body.

The lymphatic vessels are arranged in five main quadrants in our bodies; one for the head and neck and four others dividing the trunk and limbs.  Each quadrant of the trunk consists of one extremity and the adjacent quarter of the trunk.  Lymph fluid is carried through vessels just beneath the skin in one direction towards the major lymph nodes which are located at the points where the limb meets the trunk at the groin and axilla (see Figure 2).
 

What is Lymphedema?
Lymphedema is a type of swelling where an excess of protein rich fluid accumulates in the tissues of the body.  It is associated with some kind of malfunction of the lymphatic system.  Swelling occurs when the amount of lymph fluid in the tissues (the “lymph load”) is greater than the capacity of the system.
Following severe trauma to lymph nodes/vessels or surgical removal of lymph nodes, the capacity of the system will be decreased.  In the case of lymphadenectomy, the onset of lymphedema may occur weeks, months or even years following the surgery. The precipitating factor can be any event which increases the “lymph load” such as infection/inflammation, muscle strain, sunburn, constriction or overheating of the involved quadrant.  The increased lymph fluid can cause an overload resulting in fluid backup and tissue edema.

Lymphedema can also occur in conjunction with other diseases.  Chronic venous diseases almost always involve the lymphatic vessels in the long term, making the venous disease worse.  The lymph vessels work hard in an attempt to compensate for venous malfunction.  If untreated, this chronic stress causes breakdown of vessel walls, overdilation and valve malfunction  resulting in  eventual lymphatic system dysfunction.

Whatever the onset, lymphedema may progress quickly or slowly during the initial stages.  In many cases, the swelling may initially resolve spontaneously or with elevation.  In most cases, lymphedema progresses to stage II which is not spontaneously reversible.  If treatment is not provided, the edema will continue to progress.  Long term deficits include severe pain, connective tissue fibrosis and irreversible tissue damage, muscle atrophy, decreased functional mobility and chronic acute infections.

LYMPHEDEMA MANAGEMENT OPTIONS

Lymph Drainage Massage
Lymph Drainage Massage (LDM) is a specialized gentle massage of the connective tissue rather than the muscles.  LDM stimulates the flow of stagnant fluid through the vessels to the venous system where  it is eliminated (Carriere, 1988).  LDM also opens collateral lymph channels to adjacent quadrants of the body which are healthier and better able to drain the excess lymph fluid. 

 For example, a left postmastectomy lymphedema patient has had lymph nodes removed from her left armpit.  This group of nodes is normally responsible for draining lymph fluid from the tissues of the left upper quadrant which includes the left breast, chest, upper back and arm.  In the presence of lymphedema it is desirable to reroute the direction of the lymph flow away from the left towards the unaffected right armpit.  This is accomplished by LDM techniques across the chest and the upper back from the left to the right armpit.

Other specific massage techniques are used to soften hardened or fibrosed areas.  Alternate routes of massage are taken around scars and adhesions.

Medical Compression Bandaging
Medical Compression Bandaging is a multi-layered bandaging technique which has proven to be very effective at reducing limb size (Casley-Smith, 1992).  The purpose of compression is twofold.  First, with compression, extracellular tissue pressures are increased which improves fluid uptake in the vessels.  Second, the bandages assist with lymph flow by facilitating the "muscle pump" (see Figure 3).  As muscles contract during normal movement they compress the lymph vessels and stimulate flow of the fluid.  Since the initial lymphatic vessels are just below the skin, healthy skin tissue provides a good counterpressure for the muscles to pump the fluid effectively.  In the case of lymphedema, the tissue pressure is low because the tissues are overstretched.  When a limb is tightly bandaged, the tissue pressure is increased, enhancing the effects of the "muscle pump”.

Bandages work well with lymphatic drainage massage; in combination, the two techniques soften the fluid and stimulate lymphatic circulation.

Compression Garments 
Limb size reduction may be achieved through a brief intensive course in bandaging, massage, and/or pumping.  To maintain the reduced limb size for long term, compression garments are usually necessary.  Patients should be fitted with garments once their limb size has stabilized following other decongestive techniques (Casley-Smith, 1992).

 A compression garment provides an important "supportive" function for the tissues.  In an edematous limb, the intercellular spaces are over stretched and the elastic component of the tissue is damaged.  Even after using a technique which empties fluid out of the limb the tissue spaces remain overstretched for several months and will continue to attract protein rich fluid.  Compression closes these spaces and prevents refilling of the limb (Casley-Smith, Morgan & Piller, 1993).

Garments vary significantly in fabric, style, color, compression, and price.  The most comfortable fabrics are knitted "in the round" rather than woven materials which are cut and sewn.  Several companies offer a range of "off-the-shelf" garments.  Compared with custom made garments , the "off-the-shelf" garments are much less expensive and are speedily delivered.  Once garments are delivered, patients should be instructed in proper donning techniques, wearing schedules and care of the garment.  Use of talc powder, rubber gloves, and metal "butlers" can improve the ease of donning.  Body adhesive and shoulder or hip straps are available to help prevent garments from sliding down. 

GARMENTS ARE NOT DESIGNED TO REDUCE LIMB SIZE, therefore, without decongestive techniques some limbs may be impossible to fit properly.

Compression Pumps
Many clinics utilize compression pumps to increase the uptake of fluid from the tissues into lymphatic vessels and pump fluid out of the limb.  Significant progress has been made since the original single cell pumps.  Multicell sequential gradient pumps are more effective because they are designed to segmentally move fluid from the distal to the proximal portion of the limb. Pumps are a convenient alternative compression treatment for patients who live in remote communities, have flaccid extremities, or are unable to self bandage (e.g. arthritis, obesity).

If pumps are used it is important to be aware of certain precautions/disadvantages.  Too much pressure will collapse the lymphatic channels and prevent fluid uptake.  The maximum recommended pressure is usually 40mm Hg, and some patients tolerate much less.

Another potential problem area is that the fluid is "dumped" into the proximal region of the limb.  For example, when a left upper extremity is pumped, the fluid is moved to the left shoulder.  This is a problem because fluid remains in the involved left upper quadrant, and continues to depend upon the ineffective left armpit nodes for drainage. Pumping is not recommended unless the patient is instructed in LDM techniques across the trunk from the left upper shoulder region to the healthy right armpit where fluid can be adequately drained (Casley-Smith, Morgan & Piller, 1993).

Exercise
As described earlier, the pumping action of muscle contraction helps move fluid along lymphatic channels.  Patients should be encouraged to use their affected limb as normally as possible and to get regular exercise.  Exercise with light to moderate weights is safe-but patients must be aware that a muscle strain may likely cause an increase in swelling.  Obviously, it is important to warm-up, cool-down and progress intensity gradually to reduce the risk of muscle strain. If a patient owns a compression garment it should almost always be worn during exercise.

Elevation
In the early or "spontaneously reversible" stages of lymphedema, swelling may be easily reduced with elevation.  During stage II lymphedema, if untreated, the limb usually refills as the day goes on.  It is naturally helpful to elevate the limb while at rest and to pace activities.  However, patients must understand that elevation should not replace activity.  Immobility will lead to muscle wasting away and decreased efficiency of the muscle pump, thereby worsening the condition.  It may be helpful to take rest periods with elevation intermittently to break up long periods of repetitive activity involving the edematous extremity.

Surgery
At the present time, surgical intervention is only minimally successful for reduction of lymphedema.  Trials continue throughout Europe and North America with lympho-venous and lympho-lymphatic anastomoses (Casley-Smith, Morgan & Piller, 1993).

Medications
Benzopyrones (also know as Lodema or coumarin) can reduce the volume of high protein fluid.  Coumarin, not to be confused with coumadin (a blood thinner), effectively reduces edema by increasing breakdown of protein (Foldi, Foldi, & Clodius, 1991).  It is available in powder, cream or can be taken orally.  Once the excess protein is removed, the fluid is not retained in the tissues.  Patients should be aware that it is slow acting and takes about 4-6 months to begin working. Unfortunately, this medication is not widely available or known about in the United States.  Coumarin is best used as a complement to LDM and compression therapy (CasteySmith, Morgan & Piller, 1993).  Homeopathic remedies such as bioflavinoids, rutin and pycnogenols are also being used.

New Products: CIRCAID/REID SLEEVE/LEGASSIST
 Several new products have recently become available for lymphedema management.  I would consider most of them still in a “trial “phase, as research studies have not been comprehensive.  All three of the products above are compressive devices designed to provide gradient compression.  They are non-elastic and utilize a series of velcro straps to tighten.  Durability, cost, styles and guarantees vary between products.

 Some of these products are advertised as an “easy” treatment for lymphedema.  However, one must realize the importance of a good knowledge of the lymphatic system and assuring a gradient compression.  Although ease of donning is the major advantage manufacturers claim, it is common to have to customize the product to properly fit the abnormally shaped limb.  If the patient or therapist do not carefully evaluate the fit a tourniquet effect can worsen the edema where it crosses narrow diameter joints or fissures.

Therapists treating lymphedema usually employ a combination of techniques.  Complex Decongestive Physical Therapy (CDPT or CPT) is a widely known treatment program in Australia and Germany and has made its way to a handful of clinics in the United States.  It is an intensive course of daily treatments of LDM, medical compression bandaging and exercise as well as essential skin care education (see Lymphedema Prevention). From 30%-100% reduction of lymphedema volume can be achieved in a brief intensive course of CDPT; 2-4 weeks for upper extremities and 3-4 weeks for lower extremities .  Following the intensive course the patient assumes the responsibility for their long term management, with intermittent therapy available as needed.  Whatever techniques you utilize, your best results will come from an integrated program where consideration is made for each patient's unique needs and personal goals.
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LYMPHEDEMA PREVENTION

Practicing good skin care and preventing infections of the involved limb will help you avoid episodes of swelling.  In addition to avoiding infections, you also need to be aware that muscle strain, sunburn, overheating , constriction and air travel can worsen swelling.

Following the guidelines below can help you avoid pain, swelling and complications.
 

• Daily skin check of the limb at risk will increase your awareness of cuts, burns, insect bites and signs of infection.  Treat these preventatively with antibiotic ointment and a band-aid.  The signs of infection are redness, warmth and tenderness.  Call your physician immediately for oral antibiotics if these signs are present.

• Avoid infection by keeping your limb clean and protecting hands by wearing gloves for gardening, cleaning and washing dishes.  Use an electric razor to remove hair, a thimble for sewing, and offer the other arm for injections, blood pressures and tests.

• Avoid muscle strain by using your limb normally but not excessively.  Be sure to pace heavy or strenuous tasks.  Ask for assistance with heavy lifting.  The use of a compressive sleeve during exercise may be necessary to control swelling during exercise.

• Avoid sunburn by using a high SPF sunscreen on the affected quarter of the body.

• Avoid overheating by being aware that the use of hot tubs, saunas and steam baths will most likely increase your swelling.  Stay well hydrated and avoid exercising during the hottest part of the day.

• Avoid constriction of your limb from clothing (esp. undergarments),  jewelry or heavy purses.

Carriere B. Edema: its development and treatment using lymph drainage massage.  Clin.  Management 8(5):19.21, 1988.

Casley-Smith JR.  Modem treatment of lymphoedema.  Mod Med Australia 35 (5):70-83, May, 1992.

Casley-Smith JR, Casley-Smith  JR. Modern treatment of lymphoedema 1. Complex physical therapy: the first 200 Australian limbs. Australas J Dermatol 33:61-68, 1992.

Casley-Smith JR, Casley-Smith JR.  Lymphoederna: a guide for therapists and patients. 2nd ed.  Australia: The Lymphoedema Association, 1991.

Casley-Smith JR, Morgan RG, Piller NB.  Treatment of lymphedema of the arms and legs with 5,6 benzo-a-pyrone.  NEJM.  Oct. 14, 1993:1158-1163.