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 anomalies 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 patient’s post-cancer surgery (likely due to the decreased cabin pressure).

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).

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).

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.

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:

• Manual lymphatic drainage
• Bandaging
• Proper skin care & diet
• Compression garments (sleeves, stockings, devices such as Reid Sleeve, CircAid leggings, Legacy Sleeve, as well as other alternative approaches)
• Remedial exercises
• Self-manual lymphatic drainage & bandaging, if instruction is available
• Continue to follow prophylactic methods at all times.

1. Absolutely do not ignore any slight increase of swelling in the arm, hand, fingers, or chest wall (consult with your doctor immediately).

2. Never allow an injection or a blood drawing in the affected arm(s). Wear a LYMPHEDEMA ALERT bracelet.

3. Have blood pressure checked on the unaffected arm, or on the leg (thigh), if bilateral lymphedema/at-risk arms.

4. Keep the edemic or at-risk arm(s) spotlessly clean. Use lotion (Eucerin, Lymphoderm, Curel, whatever works best for you) after bathing. When drying it, be gentle, but thorough. Make sure it is dry in any creases and between the fingers.

5. Avoid vigorous, repetitive movements against resistance with the affected arm (scrubbing, pushing, pulling).

6. Avoid heavy lifting with the affected arm. Never carry heavy handbags or bags with over-the-shoulder straps on your affected side.

7. Do not wear tight jewelry or elastic bands around affected fingers or arm(s).

8. Avoid extreme temperature changes when bathing or washing dishes, and it is recommended that saunas and hot tubs be avoided (at least keep arm out of the hot tub). Protect the arm from the sun at all times.

9. Try to avoid any type of trauma (bruising, cuts, sunburn or other burns, sports injuries, insect bites, cat scratches) to the arm(s). (Watch for subsequent signs of infection.)

10. Wear gloves while doing housework, gardening or any type of work that could result in even a minor injury.

11. When manicuring your nails, avoid cutting your cuticles (inform your manicurist).

12. Exercise is important, but consult with your therapist. Do not overtire an arm at risk: if it starts to ache, lie down and elevate it. Recommended exercises: walking, swimming, light aerobics, bike riding, and specially designed ballet or yoga. (Do not lift more than 15 lbs.)

13. When traveling by air, patients with lymphedema (or who are at risk) must wear a well-fitted compression sleeve. Additional bandages may be required on a long flight. Increase fluid intake while in the air.

14. Patients with large breasts should wear light breast prostheses (heavy prostheses may put too much pressure on the lymph nodes above the collar bone). Soft padded shoulder straps may have to be worn. Wear a well-fitted bra: not too tight, ideally with no underwire.

15. Use an electric razor to remove hair from axilla. Maintain electric razor properly, replacing heads as needed.

16. Patients with lymphedema should wear a well-fitted compression sleeve during all waking hours. At least every 4-6 months, see your therapist for follow-up. If the sleeve is too loose, most likely the arm circumference has reduced or the sleeve is worn.

17. Warning: If you notice a rash, itching, redness, pain, increase of temperature or fever, see your physician immediately. An inflammation (or infection) in the affected arm could be the beginning or worsening of lymphedema.

18. Maintain your ideal weight through a well-balanced, low sodium, high-fiber diet. Avoid smoking and alcohol. Lymphedema is a high protein edema, but eating too little protein will not reduce the protein element in the lymph fluid; rather, this may weaken the connective tissue and worsen the condition. The diet should contain easily digested protein (chicken, fish, tofu).

1. Absolutely do not ignore any slight increase of swelling in the toes, foot, ankle, leg, abdomen, genitals (consult with your doctor immediately).

2. Never allow an injection or a blood drawing in the affected leg(s). Wear a LYMPHEDEMA ALERT Necklace.

3. Keep the edemic or at-risk leg spotlessly clean. Use lotion (Eucerin, Lymphoderm, Curel, whatever works best for you) after bathing. When drying it, be gentle, but thorough. Make sure it is dry in any creases and between the toes.

4. Avoid vigorous, repetitive movements against resistance with the affected legs.

5. Do not wear socks, stockings or undergarments with tight elastic bands.

6. Avoid extreme temperature changes when bathing or sunbathing (no saunas or hot tubs). Keep the leg(s) protected from the sun.

7. Try to avoid any type of trauma, such as bruising, cuts, sunburn or other burns, sports injuries, insect bites, cat scratches. (Watch for subsequent signs of infection.)

8. When manicuring your toenails, avoid cutting your cuticles (inform your pedicurist).

9. Exercise is important, but consult with your therapist. Do not overtire a leg at risk; if it starts to ache, lie down and elevate it. Recommended exercises: walking, swimming, light aerobics, bike riding, and yoga.

10. When traveling by air, patients with lymphedema and those at-risk should wear a well-fitted compression stocking. For those with lymphedema, additional bandages may be required to maintain compression on a long flight. Increase fluid intake while in the air.

11. Use an electric razor to remove hair from legs. Maintain electric razor, properly replacing heads as needed.

12. Patients who have lymphedema should wear a well-fitted compression stocking during all waking hours. At least every 4-6 months, see your therapist for follow-up. If the stocking is too loose, most likely the leg circumference has reduced or the stocking is worn.

13. Warning: If you notice a rash, itching, redness, pain, increase of temperature or fever, see your physician immediately. An inflammation or infection in the affected leg could be the beginning or a worsening of lymphedema.

14. Maintain your ideal weight through a well-balanced, low sodium, high-fiber diet. Avoid smoking and alcohol. Lymphedema is a high protein edema, but eating too little protein will not reduce the protein element in the lymph fluid; rather, this may weaken the connective tissue and worsen the condition. The diet should contain easily digested protein such as chicken, fish or tofu.

15. Always wear closed shoes (high tops or well-fitted boots are highly recommended). No sandals, slippers or going barefoot. Dry feet carefully after swimming.

16. See a podiatrist once a year as prophylaxis (to check for and treat fungi, ingrown toenails, calluses, pressure areas, athlete’s foot).

17. Wear clean socks & hosiery at all times.

18. Use talcum powder on feet, especially if you perspire a great deal; talcum will make it easier to pull on compression stockings. Be sure to wear rubber gloves, as well, when pulling on stockings. Powder behind the knee often helps, preventing rubbing and irritation.

• Avoid sunburn
• Avoid insect bites (especially spider bites)
• Don't overdo sports that you are not used to. Be wary of the more strenuous excursions that may cause trauma (or bumps and stress) to limbs
• Beware of fungal infection (tinnea)
• Wear buckle-up plastic sandals if you have lymphedema of leg/s, if on the beach or paddling. If on a coral beach or snorkeling near coral, be extra careful. Coral infection can cause lymphedema in people with normal limbs
• Use a good skin moisturizer
• If it is hot, realize that you can cool your limb with your compression garment on just by wetting it! Put your limb under a tap or shower. Evaporation will then cause cooling
• Be careful shaving, pushing back cuticles etc.- the general "Do's and Don'ts" (see 18 Steps to Prevention)