Lymphedema: Unlocking the Doors to Successful Treatment

" . . . women with breast cancer are warriors, also. I have been to war and still am . . . I refuse to be reduced in my own eyes or in the eyes of others from warrior to mere victim, simply because it might render me a fraction more acceptable . . . to those who believe that if you cover up a problem, it ceases to exist."

Audre Lorde

The Cancer Journals

Author: Linda T. Miller, BA, BS, PT
Linda T. Miller is Clinical Director, Breast Cancer Physical Therapy Center, Philadelphia, PA
Reprinted on OncoLink with permission of the publisher
Innovations in Oncology Nursing Vol 10, No. 3, pp. 53,58-62, 1994
Copyright, 1994, Meniscus Health Care Communications

Upper-extremity lymphedema is a potentially serious consequence of breast cancer surgery and often results in a painful, debilitated, and swollen limb. This stagnation of high-protein fluid in the body tissue can result from the surgical procedure itself and can be exacerbated by the radiation therapy that follows many breast-conserving techniques. Despite its long history in the literature, lymphedema remains an enigma that often leaves the medical community and those who suffer from the condition frustrated and searching for answers.

Part of the frustration stems from a lack of consistency in the literature in defining, measuring, and clinically identifying lymphedema. Its reported incidence following breast cancer surgery varies greatly. Earlier literature, based largely on the prevalence of edema following the Halsted radical mastectomy, reported incidence as high as 62.5%.[1,2] More recent studies of patients who had less extensive surgery, however, have reported incidence as low as 2%.[3,4] Although the shift from the Halsted procedure to more conservative surgical approaches has led to an overall decline in the incidence of lymphedema, women who undergo breast-conserving techniques (i.e., lumpectomy or partial mastectomy) are still at risk for lymphedema because the procedure involves axillary dissection.

The dissection disrupts normal lymphatic drainage pathways from the entire upper quarter, disturbing the flow of lymphatic fluid until new lymphatic pathways can be regenerated. Because radiation therapy, a documented precipitant of lymphedema owing to its effect on the regeneration of new pathways, is often an adjunct to breast-conserving procedures, women who have this surgery may be at even greater risk.[5-7] Unfortunately, because of the widespread misconception that lymphedema occurs only with more extensive surgery, many women undergoing other procedures are not informed of the risk of lymphedema or taught proper post surgical arm care and skin precautions.[8]

With 182,000 newly diagnosed cases of breast cancer in 1993, even a conservative estimate of a 10% incidence of lymphedema would leave over 18,000 women with the condition in that year alone. Such numbers, multiplied year after year, give us a large population of women with a complication that remains largely shrouded in secrecy and silence and carries its own documented psychological morbidity.[10,11]

Physical and Psychological Changes

For many women, lymphedema is a continual reminder of the battle they have waged against breast cancer and can impede illness adjustment, making it prolonged and more difficult Tobin et al found that women which often lead to a decreased interest in family, social activities, and occupational goals.[10] Conversely, they found that women who did not experience arm edema following breast cancer surgery were much more likely to adjust to the illness and move forward with their lives.

Negative self-esteem and body image are also problems associated with upper-extremity lymphedema.[12] Many report a decreased interest in personal appearance and a diminished sexual drive. Women with lymphedema overwhelmingly complain of difficulty in dressing and finding appropriate clothing that fits them and hides the condition, to avoid repeated questions and explanations. The onset of the condition, often sudden and without warning, coupled with the woman's inability to stop the arm from swelling, causes a sense of helplessness and loss of control and often precipitates fear and self-blame. This can become exacerbated by frustration with the perceived apathy of the medical community and the difficulty women with lympedema have in finding information on the condition and its treatment.

Often, it is not apathy but lack of knowledge among health care professionals that leaves many women with lymphedema untreated. Frequently, arm edema is not identified as a problem, or early symptoms are misdiagnosed. Even when correctly diagnosed, lymphedema is often passed off as an unfortunate but untreatable consequence of breast cancer surgery. For years, a lack of understanding of the anatomy and physiology of the lymphatic system led to unsuccessful treatment protocols that supported this misconception. The following provides an overview of the physiology of lymphedema and a discussion of treatment options.

Physiology of Lymphedema

The lymphatic system has two primary immunologic functions:

activating the inflammatory response
infection control.

However, equally important is its symbiosis with the hematologic vasculature in the regulation of tissue-fluid balance. This delicate balance is achieved through the unidirectional transportation of proteins from the tissue to the blood system. In conjunction with the vessel networks, the lymphatic system maintains the equilibrium between tissue-fluid filtration and resorption.[7,13].

Protein molecules carry oxygen and nutrients to the cells of the tissues and then remove metabolic wastes from them [14]. Many protein molecules, too large to be carried in the venous system, are returned to the blood system via the lymphatics. Thus, lymphatic fluid is high in protein but also carries fat, broken down cells, and other macromolecules. Normal protein circulation requires an adequately functioning lymphatic vessel system; without it, the interstitial tissue can become congested with lymphatic fluid. Lymphedema occurs when there is a diminished capacity for the lymphatic system to transport lymphatic fluid from the tissue. Because lymph contains the protein fibrinogen, coagulation and fibrosis can occur with long-standing lymphatic fluid transport deficiency, leading to the firmness and pitting seen in many cases of postoperative edema. [14,15]

. Three major factors affect lymphatic flow:

First, larger lymphatic vessels have their own anatomic mechanism of rhythmic contraction, similar to a heartbeat. The reflex action of this contraction can be facilitated by a stretch simulation.
Second, pressure on the lymphatic vessels exerted by external forces, such as pulsating arteries or muscle contraction.
Finally, the changes in intrathoracic pressure that occur with breathing increase lymphatic flow.[13,14]

Treatment techniques that are grounded in these normal physiologic principles and consider the pathophysiology of lympedema can successfully reduce and maintain the appropriate limb size.

When diagnosed early, lymphedema usually responds more readily to treatment. Many women, noticing swelling for the first time, become frustrated when their physician say that the arm is "not so bad" or "it could be worse." The notion that one arm should be twice the size of the other before it deserves attention is not only unfair but potentially dangerous. Stagnant edema, rich in protein, serves as an excellent breeding ground for bacteria. This predisposes the limb to low-grade inflammation or infection, and women with even mild lymphedema can develop cellulitis. The ensuing inflammatory response increases fluid movement from the capillaries to the surrounding tissue and can congest the lymphatic capillaries, making fluid transport even more difficult and worsening edema. [7, 15].

Treatment Options

Determining the appropriate treatment for lymphedema depends greatly on the size of the limb and the nature or firmness of the edema. Currently, the common belief is that multimodality treatment, rather than single-line therapy, most effectively manages lymphedema. However, acceptance of this concept by the medical community has been delayed, and women able to obtain treatment often receive what has for years proved unsuccessful. Not only has the medical community been slow in accepting new treatment ideas, but only a limited number of resource facilities around the country offer a comprehensive treatment program. Successful lymphedema management takes an enormous amount of time and commitment from the patient and treating clinician. However, when the commitment to a comprehensive approach has been made, the results have been encouraging.

The most successful lymphedema treatments consider both the normal physiology of lymphatic flow and the pathophysiology of lymphedema. Although the techniques and modalities that are the standard of care today do have a physiologic basis, in many circumstances their application must be reviewed for overall merit.

Elevation

Often, the first suggestion made to a woman who develops lymphedema is to elevate the extremity, making use of gravity to assist proximal lymphatic flow. Although this may be somewhat effective with mild edema, it most often provides only a temporary solution. For larger extremities, elevation has even less impact. In fact, elevation alone has not been shown to be an effective treatment of lymphedema.[16]

Performing exercises (e.g., ball squeezing) with the arm overhead has often been prescribed. Again, this leads to little, if any, sustained reduction in overall limb size. Finally, the practicality of prolonged arm elevation must also be questioned. Perhaps the most beneficial effect of periods of elevation is to provide the woman with comfort and a decreased feeling of "heaviness" of the limb. However, to be effective in reducing edema long term, elevation must be combined with other forms of lymphedema management.

FIGURE 1: Exercise for the Treatment of Lymphedema

Exercise

Exercise has generally been accepted in the treatment of lymphedema (Fig 1). However, the specific type of exercise recommended can vary greatly. There does not appear to be any scientific literature comparing different exercise protocols of varying intensity and duration. Despite the lack of specific research, the rationale for some form of exercise as a tool in the treatment of lymphedema can be substantiated by lymphatic physiology. Lymphatic fluid moves as the body moves; muscle contractions serve as a pumping force, which can facilitate lymphatic flow.[14]

Many women report sudden onset of lymphedema with overuse or overstress of the limb. These stresses can vary in intensity, and activities that were routine before surgery often precipitate the condition. Reconditioning the arm through a controlled exercise program may be one way to help prevent the lymphedema that can occur with sudden, unexpected stress. Prescribed activities can range from simple flexibility exercises to gentle strengthening exercises using free weights. The woman should be encouraged to use her arm as a barometer of tolerance to the activity. If exercises are performed without an increase in the edema for several sessions, the exercise can progress. If, at any time, the arm responds adversely (i.e., increased edema or pain), the intensity of the exercise should be decreased.

An aerobic exercise program (which may include walking or biking) may also be a beneficial addition to the lymphedema treatment program. A drop in intrathoracic pressure occurs with abdominal breathing and creates a suction force that can facilitate lymphatic flow [14]

Compression Garments

A compression sleeve or bandage, although often unpopular with patients, is extremely important in providing external support to the skin and can be an effective way to reduce and control the edema (Fig 2). The gradient compression forces inherent in most garments and created by bandaging push the lymphatic fluid into the lymph vessels from the interstitium and help move the fluid proximally. Furthermore, wearing a compression garment gives resistance to the contracting muscles beneath, which increases the interstitial pressure and thus the lymphatic flow.[17] Therefore, wearing an appropriate compression garment does have its place. It is especially important during the treatment phase to maximize reduction and prevent the return of fluid between treatments. For continued progress and control, the garment should be worn as long as the edema persists. A compression sleeve or bandage should be worn during times of increased activity, as during an exercise program. To be maximally effective, the garment should be fitted properly and replaced when well worn.

Manual Lymphatic Drainage

Manual lymphatic drainage is a special massage technique (Fig 3) that uses the superficial lymphatic vessels of the skin to transport lymphatic fluid from an area of congestion to an area of functioning lymphatics. The massage is performed in the direction of normal lymphatic flow by using light and sequential strokes.

The addition of manual lymphatic drainage to a treatment program is considered essential if long-term results are desired. [15,17] The technique increases the transport capacity and the amount of collaterals adjacent to the congested area, leading to increased lymphatic drainage from the areas of damage. Therefore, repeated manual lymphatic drainage can actually lead to anatomic changes that improve lymphatic flow, reduce the size of the extremity, and, when combined with a properly fitting compression garment, help maintain the limb's size.

FIGURE 2: Compression Treatment for Lymphedema

Compression Pumps

Vasopneumatic pumps have long been used in the treatment of lymphedema (Fig 4). The pumps vary greatly in design and function. Protocols for using this equipment also vary widely, with times ranging from 20 min/d to 8 hr/d and pressures ranging from 30 to 120 mm Hg.[18,19] There does not appear to be a consensus in the literature on which pumps are most effective, at what pressure, and for what duration. This, coupled with the general lack of knowledge about lymphedema in the medical community, has led to wide misuse of the equipment. Lack of direction and proper instruction in using the pumps has left many women frustrated with an arm that shows little progress, despite hours of commitment to pumping.

FIGURE 3: Massage for Lymphedema FIGURE 4: Compression Pump for Lymphedema Treatment

Discussion at the XIV International Congress of Lymphology in Washington, DC, in 1993 led most clinicians to agree that pumping alone is not an effective treatment for lymphedema, and that to be truly beneficial, it must be combined with other modalities. Specifically, the quadrant of the trunk adjacent to the affected limb should be cleared by manual lymphatic drainage in conjunction with pumping to prevent chest wall edema and pooling fluid.

Although pumps do have their place in lymphedema management, the equipment should never be prescribed indiscriminately, and the patient should be closely monitored by trained clinician throughout its use. Too often, patients are provided with equipment without adequate follow-up that monitors its use and effectiveness. Many women, frustrated by lack of progress and tired of the strict compliance required, abandon the pump after only a short time. This poor medical management has led to the distribution of thousands of pumps with marginal patient response or satisfaction and hundreds of thousands of dollars wasted on unused equipment.

Benzopyrones

Recently, much attention has been given to the pharmacologic treatment of lymphedema through the use of benzopyrones (coumarin). While this drug is not yet approved by the FDA, interest in it has been described, and clinical trials are soon expected in the United States.[20]

Coumarin reduces lymphedema by stimulating the body's own macrophages, thereby increasing protein degradation in fluid.[20,21 By decreasing the excess protein, fluid is no longer retained, and the edema decreases. Although the drug is slow-acting, it has been shown to decrease lymphedema and the chronic inflammation that accompanies it over a 6-month period.[20] If eventually approved by the FDA, coumarin could be one more tool in the management of lymphedema.

Conclusions

It is clear that successful management of lymphedema depends on the proper blend of treatment modalities. The combination that works best will be different for each woman. Finding facilities that offer all, if not most, of these techniques can be a challenge. Unless a clinic specializes in lymphedema management, the latest information is often not readily available, hence older and unsuccessful treatments may be used. This situation can be frustrating for women who have researched their condition and are searching for the more advanced, comprehensive approaches. Perhaps the greatest challenge is convincing the medical community that lymphedema is no longer an acceptable consequence of breast cancer surgery, and that a comprehensive and effective management program should be available to every woman who develops the condition.

Nurses and physical therapists play a major role in helping women locate resources, use equipment properly, and discuss physical and psychosocial issues. They also ensure that comprehensive and effective management programs are in place and utilized to their fullest potential.

References

1. Lobb AW. Harkms HN "Post-mastectomy swelling of the arm with note on the effect of segmental resection of the axillary vein at the time of radical mastectomy." West J. Surg 1949;57:550-557.

2. Britton RC, Nelson, PA, "Causes and treatment of post mastectomy lymphedema of the arm." JAMA 1962;180:95-102

3. Khoury CG., Josli CA., Brennan TG. "Risk of arm lymphoedema following conservative treatment of breast cancer." Proceedings of the Seventh Annual Meeting of Therapeutic Radiology and Oncology The Netherlands, 1988:39.

4. Brisman B. Ljungdald I. "Post-operative lymphoedema after treatment of breast cancer." Acta Chem Scand. 1983;149:687-689.

5. Swedhorg I. Wallgren, A. "The effect of pre- and post-mastectomy radiotherapy on the degree of edema, shoulder-joint mobility, and gripping force." Cancer 1981;47: 877-881.

6. Nikkanen TAV, Vanharanta H, Helenius-Rewianeit H. et al. "Swellings of the upper extremity, function and muscle strength of shoulder joint following mastectomy combined with radiotherapy." Ann Clin Res. 1978;10:273-279.

7. Jeffs E. "The effect of acute inflammatory episodes (cellulitis) on the treatment of lymphoedema." J. Tissue Viabil. 1993;3(2):51-55.

8. Cawley M, Kostic J, Cappello C. "Informational and psychosocial needs of women choosing conservative surgery/primary radiation for early stage breast cancer." Cancer Nurs. 1990:13(2):90-94.

9. Cancer Facts & Figures -- 1993 Atlanta, Ga: American Cancer Society; 1993.

10. Tobin M, Lacey H, Meyer L, et al. "The psychological morbidity of breast cancer-related arm swelling." Cancer 1993;72:3248-3252.

11. Dennis B. "Acquired lymphedema: a chart review of nine women's responses to intervention." Am J Occup Ther 1993;47(10):891-?898.

12. Passik S, Newman, M, Brennan, M, "Psychological aspects of lymphedema after breast cancer treatment" Natl Lymphedema Net News 1992;4(4):4.

13. Wittlinger, H. Textbook of Dr. Vodder's Manual Lymphatic Drainage, I Heidelberg, Germany: Karl R. Hang Publishers: 1982:64.

14. Wittlinger, H. Textbook of Dr. Vodder's Manual Lymphatic Drainage, II Heidelberg, Germany: Karl R. Hang Publishers: 1989:72.

15. Casley-Smith JR. "Modern Treatment of lymphoedema" Mod Med Aust. 1992;35(5):70-83.

16. Swedborg J. "Elevation for reduction of post-mastectomy lymphoedema" Scand J. Rehab Med. 1993;25:79-82.

17. Foldi E., Foldi M., Weissleder, H. et al. "Conservative treatment of lymphoedema of the limbs." Angiol. J. Vasc. Dis. 1985;3171-180.

18. Pappas C., O'Donnell T. "Long-term results of compression treatment for lymphoedema" J. Vasc Surg 1992;16(4):555-563.

19. Raines, J. O'Donnell, T., Kalisher, L. et al. "Selection of patients with lymphedema for compression therapy. Am J. Surg 1977:133:430-437.

20. Caley-Smith JR, Morgan RG, Piller, NB et al. "Treatment of lympedema of he arms and legs with 5,6-benzo-a-pyrone." N Engl J. Med 1993;329:1158-1163.

21. Casley-Smith JR, Caley-Smith JR. "The pathophysiology of lymphoedema and the action of benzopyrones in reducing it." Lymphology 1988;21:190-194.