WHAT IS LYMPHEDEMA?

Lymphedema is an accumulation of lymphatic fluid in the interstitial tissue that

causes swelling, most often in the arm(s) and/or leg(s), and occasionally in other

parts of the body. Lymphedema can develop when lymphatic vessels are

missing or impaired (primary), or when lymph vessels are damaged or lymph

nodes removed (secondary).

When the impairment becomes so great that the lymphatic fluid exceeds the

lymphatic transport capacity, an abnormal amount of protein-rich fluid collects

in the tissues of the affected area. Left untreated, this stagnant, protein-rich fluid

not only causes tissue channels to increase in size and number, but also reduces

oxygen availability in the transport system, interferes with wound healing, and

provides a culture medium for bacteria that can result in lymphangitis

(infection).

Lymphedema should not be confused with edema resulting from venous

insufficiency, which is not lymph-edema. However, untreated venous

insufficiency can progress into a combined venous/lymphatic disorder which is

treated in the same way as lymphedema.

What Causes Lymphedema?

Primary lymphedema, which can affect from one to as many as four limbs

and/or other parts of the body, can be present at birth, develop at the onset of

puberty (praecox) or in adulthood (tarda), all from unknown causes, or

associated with vascular anomolies such as hemangioma, lymphangioma, Port

Wine Stain, Klippel Trenaury.

Secondary lymphedema, or acquired lymphedema, can develop as a result of

surgery, radiation, infection or trauma. Specific surgeries, such as surgery for

melanoma or breast, gynecological, head and neck, prostate or testicular,

bladder or colon cancer, all of which currently require removal of lymph nodes,

put patients at risk of developing secondary lymphedema. If lymph nodes are

removed, there is always a risk of developing lymphedema.

Secondary lymphedema can develop immediately post-operatively, or weeks,

months, even years later. It can also develop when chemotherapy is unwisely

administered to the already affected area (the side on which the surgery was

performed) or after repeated aspirations of a seroma (a pocket of fluid which

occurs commonly post-operatively) in the axilla, around the breast incision, or

groin area. This often causes infection and, subsequently, lymphedema.

Aircraft flight has also been linked to the onset of lymphedema in patients post-

cancer surgery (likely due to the decreased cabin pressure). For more

information, see the NLN Position Paper on Air Travel (pdf format, 231kb).

Another cause of lower extremity lymphedema is that resulting from the use of

Tamoxifen. This medication can cause blood clots and subsequent DVT (deep

venous thrombosis).

Radiation therapy, used in the treatment of various cancers and some AIDS-

related diseases (such as Kaposi-Sarcoma), can damage otherwise healthy

lymph nodes and vessels, causing scar tissue to form which interrupts the

normal flow of the lymphatic fluid. Radiation can also cause skin dermatitis or a

burn similar to sunburn. It is important to closely monitor the radiated area for

any skin changes, such as increased temperature, discoloration (erythema) or

blistering which can lead into the development of lymphedema. Be sure to keep

the area soft with lotion recommended by your radiation oncologist.

Lymphedema can develop secondary to lymphangitis (an infection) which

interrupts normal lymphatic pathway function. A severe traumatic injury in

which the lymphatic system is interrupted and/or damaged in any way may also

trigger the onset of lymphedema. Although extremely rare in developed

countries, there is a form of lymphedema called Filariasis which affects as many

as 200 million people worldwide (primarily in the endemic areas of southeast

Asia, India and Africa). When the filarial larvae from a mosquito bite enters the

lymphatic system, these larvae mature into adult worms in the peripheral

lymphatic channels, causing severe lymphedema in the arms, legs and genitalia

(also known as Elephantiasis).

Symptoms of Lymphedema

Lymphedema can develop in any part of the body or limb(s). Signs or

symptoms of lymphedema to watch out for include: a full sensation in the

limb(s), skin feeling tight, decreased flexibility in the hand, wrist or ankle,

difficulty fitting into clothing in one specific area, or ring/wristwatch/bracelet

tightness. If you notice persistent swelling, it is very important that you seek

immediate medical advice (and get at least one second opinion) as early

diagnosis and treatment improves both the prognosis and the condition.

Lymphedema develops in a number of stages, from mild to severe,referred to as

Stage 1, 2 and 3:

Stage 1 (spontaneously reversible):

Tissue is still at the "pitting" stage, which means that when pressed by

fingertips, the area indents and holds the indentation. Usually, upon waking in

the morning, the limb(s) or affected area is normal or almost normal size.

Stage 2 (spontaneously irreversible):

The tissue now has a spongy consistency and is "non-pitting," meaning that

when pressed by fingertips, the tissue bounces back without any indentation

forming). Fibrosis found in Stage 2 lymphedema marks the beginning of the

hardening of the limbs and increasing size.

Stage 3 (lymphostatic elephantiasis):

At this stage the swelling is irreversible and usually the limb(s) is/are very large.

The tissue is hard (fibrotic) and unresponsive; some patients consider

undergoing reconstructive surgery called "debulking" at this stage.

When lymphedema remains untreated, protein-rich fluid continues to

accumulate, leading to an increase of swelling and a hardening or fibrosis of the

tissue. In this state, the swollen limb(s) becomes a perfect culture medium for

bacteria and subsequent recurrent lymphangitis (infections). Moreover,

untreated lymphedema can lead into a decrease or loss of functioning of the

limb(s), skin breakdown, chronic infections and, sometimes, irreversible

complications. In the most severe cases, untreated lymphedema can develop

into a rare form of lymphatic cancer called Lymphangiosarcoma (most often in

secondary lymphedema).

Lymphangitis (Infection)

Signs and symptoms of lymphangitis (infection) may include some or all of the

following: rash, red blotchy skin, itching of the affected area, discoloration,

increase of swelling and/or temperature of the skin, heavy sensation in the limb

(more so than usual), pain, and in many cases a sudden onset of high fever and

chills.

Treatment for infections: immediately discontinue ALL current lymphedema

treatment modalities (including manual lymphatic drainage, bandaging, pumps,

wearing of compression garments) and contact your physician as soon as

possible. The antibiotics of choice for these types of lymphatic infections are

those in the penicillin family (note: people who develop side effects, such as

yeast infections or gastric upset can take Bicillin injections for two weeks), if no

allergies are present (for more information about Bicillin, request article reprint

"Efficacy of Benzathine Penicillin Administration," $1.75, available through

NLN). NOTE: Always carry antibiotics or a prescription with you when you

travel.

Treatments for Lymphedema

Planning the treatment program depends on the cause of the lymphedema. For

example: If the initial signs and symptoms of swelling are caused by infection

(redness, rash, heat, blister or pain may indicate an infection), antibiotics will

first need to be prescribed. Treating an infection often reduces some of the

swelling and discoloration.

If the lymphedema is not caused by infection: Depending on the severity of the

lymphedema, the recommended treatment plan should be determined using an

approach based on the Complex Decongestive Therapy (CDT) methods which

consist of: a) manual lymphatic drainage; b) bandaging; c) proper skin care &

diet; d) compression garments (sleeves, stockings, devices such as Reid Sleeve,

CircAid, Tribute, as well as other alternative approaches); e) remedial exercises;

f) self-manual lymphatic drainage & bandaging, if instruction is available; g)

continue to follow prophylactic methods at all times.

Contraindications

1. Post-cancer surgery lymphedema patients who experience a sudden

marked increase of swelling should immediately cease treatment and be

checked by their physician for possible recurrent tumor or disease. Tumor

growth can block the lymphatic flow causing a worsening of the

condition. Although not yet proven in a controlled clinical study, many

lymphedema specialists believe that patients with recurrent or metastatic

disease should not undergo Complete Decongestive Therapy (CDT) in

order not to promote the spreading of the cancer. Be sure to discuss this

treatment with your doctor.

2. Patients with a sudden onset of lymphangitis (infection) should

immediately discontinue treatment (see page 4) until the infection is

cleared. Patients with histories of vascular disease and who are taking

anticoagulants, should have a Doppler and ultra-sound to rule out deep-

venous thrombosis before being treated. During treatment, these patients

should be followed closely and regular laboratory tests should be

performed (prothrombentime).

3. Patients who have congestive heart failure must be monitored closely to

avoid moving too much fluid too quickly, for which the heart may not be

able to compensate.

4. If pain is present, discontinue all treatment until the pain subsides or the

underlying cause has been determined.