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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 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 patients 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).

 

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.

 

(c) 2005 Saskia R.J. Thiadens, R.N. All rights reserved.

Revised © 2005 National Lymphedema Network.  Permission to print and duplicate this page in its entirety for educational purposes only.


COMPRESSION GARMENTS

**

Knit Compression Garments

 

Compression garments are specialized knit two-way elastic stretch sleeves or stockings that can be worn under clothing throughout the day, including while exercising.

 

Compression garments are not worn while sleeping because they provide too much pressure when the body is inactive while at rest. Also, if the garment moves out of place during sleep, it can cause constriction that damages the circulation.

 

Compression garments do not provide protection against sunburn and, unless an outer layer of clothing is worn, a high SPF sunscreen should be applied under the compression garment.

 

Compression Sleeves

 

When the arm is affected, a compression sleeve that covers the entire arm from the wrist to the shoulder is worn. Wearing a compression sleeve just to the elbow is not recommended.

 

Sleeves are available in several designs to hold them in place. Some have a silicone band around the upper edge to prevent sliding. When keeping the sleeve up in place, so that it doesn’t constantly slide down is a problem, the solution may be a style that covers the shoulder and has a strap that goes across the chest. Although a sleeve that stops at the wrist is convenient, it does not provide compression for the hand. For many patients particularly before the swelling is well controlled, compression on the hand is also essential.

 

Compression gloves provide partial finger coverage while leaving the finger tips exposed.

 

Gauntlets are a specialized compression garment for the hands that does not cover the fingers. Compression gloves and gauntlets are separate from the compression sleeve and this makes it easier to place and remove both garments. for hand washing and similar tasks.

Compression Stockings

Compression stockings, also known as compression hose, are available in a wide range of styles, sizes, and compression strengths. When compression stockings are worn to treat lymphedema, they usually extend to the hip. When both legs are involved, a panty-hose style may be worn. If the patient has a health problem, such as a heart condition, the compression garment usually extends only to the knee.

 

Fitting Compression Garments

 

A properly fitted compression garment has the appropriate compression level, stays comfortably in place, and fits smoothly without wrinkles or bulges that can damage the tissues. Compression garments are widely available in stores and from many online sources; however, in the treatment of lymphedema a garment that does not fit properly, or is of the wrong compression, can do more harm than good! For this reason it is important that your lymphedema therapist, or an appropriately trained fitter, must take the necessary measurements and make the correct recommendations.

 

  • Compression garments are custom measured for size so that the garment stays comfortably in place and fits smoothly without wrinkles or bulges that can damage the tissues.

 

  • Measurements for these garments should be taken by a trained fitter and the fit should be re-evaluated every six months. Garments that no longer fit properly because of changes in the size of the limb, or due to wear of the garment, should be replaced immediately.

 

  • Compression garments are prescribed to provide the correct amount of pressure to enhance the control of swelling. Too little compression is ineffective and too much compression can damage the tissues.

 

  • If the garment has too little compression, it is ineffective. If it has too much compression, it can damage the tissues.

 

  • Compression garments with the correct amount of pressure enhance the comfort and the control of swelling.

 

  • Some insurance companies will cover the cost of these garments, most will not.

 

Compression Levels

 

  • Garments with compression levels below 20 mm/hg (millimeters of mercury) are not suitable for the management of lymphedema; however, stockings of these compressions are commonly used as support hose.

 

  • Garments with compression levels of 20-30 mm/hg and 30-40 mm/hg are commonly recommend for lymphedema patients.

 

  • Patients with lower extremity involvement usually require a compression level III garment (40 to 50 mm/hg)

 

  • A level II arm sleeve (30 to 40 mm/hg) is used for daily activities.

 

 

  • A patient with upper extremity lymphedema may require a compression level III (40-50 mm/hg) arm sleeve if he or she is involved in a high-intensity and repetitive activity such as playing golf.
  •  

Compression for Truncal Lymphedema

 

Thoracic Compression Garments. These garments have many features that are helpful in controlling truncal lymphedema and post-surgical swelling. These features of these garments include:

 

  • A pocket to hold prosthesis.

 

Wide padded straps to prevent excess pressure on the shoulders.

 

  • Special underarm gussets for added support and coverage in the tender armpit area.

 

  • Adjustable closures in the front and back.

 

Putting on Compression Garments

 

At first putting on a compression garment seems difficult; however with practice, the process goes smoothly. In addition to the suggestions provided here, most garment manufacturers provide helpful guidelines and some sell donning aids to make this process easier.

 

  • A compression garment is easier to put on early in the day before swelling occurs.

 

  • A thin layer of cornstarch or powder over your carefully dried skin will help the garment slide more easily.

 

  • Applying a low pH moisturizer to the affected limb is an important part of skin care; however, freshly applied moisturizer makes putting the garment on more difficult. Instead of using moisturizer in the morning just before putting on the garment, apply it in the evening after the garment has been removed.

 

  • When donning or removing a knit garment, do not wear rings or jewelry that can snag and damage your garment.

 

 

  • Wearing rubber or vinyl gloves while putting on the garment provides a better grip on the fabric and prevents fingernails from damaging the fabric.

 

  • When the garment is in place it should fit smoothly without wrinkles or bunched areas that can irritate the tissues.

 

Care of Compression Garments

 

Ideally each patient should have two compression garments: one to wear and one to be drying. These garments should be hand-laundered following the manufacturer’s instructions. The specialized liquid soaps sold by the garment manufacturers are designed to thoroughly clean the garment and to enhance the durability of the garment’s elasticity. The garment should be placed flat to dry.

 

References

Living Well with Lymphedema by A. Ehrlich, A, Vinjé-Harrewijn, PT, CLT-LANA and E. McMahon. PhD, Lymph Notes. 2005, pages 34-37.

Lymphedema Management: The Comprehensive Guide for Practitioners, 2nd ed. by J. Zuther. Thieme, 2009. pages 124-130.

NLN Air Travel Position Paper issued 2009. (http://www.lymphnet.org/pdfDocs/nlnairtravel.pdf)

Lymphedema Caregiver’s Guide by M.K. Kearse, PT, CLT-LANA, E. McMahon PhD, and A. Ehrlich, MA. Lymph Notes 2009.

Compressure Comfort Bra (http://www.bellisse.com).

© LymphNotes.com 2009. This information does not replace the advice of a qualified health care professional.

(c) 2005 Saskia R.J. Thiadens, R.N. All rights reserved.

Revised © 2005 National Lymphedema Network.  Permission to print and duplicate this page in its entirety for educational purposes only.