Secondary Lymphedema in the cancer patient
MedSurg Nursing, Oct, 2006
by Jane E. Lacovara, Linda H. Yoder
October is Breast Cancer Awareness Month, and nurses should engage in all the prevention, treatment, and survivorship education and related activities that take place during the month. Nurses play a vital role when interfacing with breast cancer survivors. Medical-surgical nurses may interact with these individuals when they need care for non-oncology problems. It is important to describe a serious problem encountered by many breast cancer survivors--lymphedema.
Lymphedema is a quality-of-life, non-fatal condition that receives less funding for research than other pathologies, yet it affects a large number of people. Actual numbers are hard to assess with ranges of 15%-25%(35-40% according to more recent studies) in the breast cancer population alone. This does not include patients with melanoma or other surgical patients who are at risk for developing lymphedema. Perhaps 2 million cancer survivors or up to 400,000 patients cope with lymphedema daily (Petrek, Pressman, & Smith, 2000). The pathophysiology, etiology, stages, treatment, prevention, and nursing implications of lymphedema will be discussed in this column.
"Lymphedema is the accumulation of protein-rich interstitial fluid within the skin and subcutaneous tissue that causes chronic inflammation and reactive fibrosis of the affected tissues" (Wyatt & Pribaz, 2004, p. 817). Normally, lymph fluid is drained from the cells in the body and carried in the lymphatic system. Lymph is cleared through a network of thin-walled lymphatics, which cross the axillary nodal basin and ultimately empty into the venous system. The lymphatic system is part of the immune system, which carries lymphocytes throughout the body to respond to antigens and communicate responses to other parts of the body. The lymphatic system also carries excess fluid back to the venous bloodstream through a series of ducts or tubules, and empties excess fluid near the subclavian vein. Lymphedema occurs when the lymphatic ducts become blocked and a buildup of lymph fluid occurs in the fatty tissues just under the skin, resulting in swelling (Lymphedema, 2004). The ability of the medical-surgical nurse to recognize lymphedema and facilitate early treatment of this common side effect of breast cancer therapy will improve patient outcomes and quality of life (Sammarco, 2004).
Pathophysiology of Lymphedema
The lymph fluid passes through the lymph nodes, which are found in the articulations of the large joints, the mesentery system, and the neck. A healthy adult has approximately 600-700 lymph nodes. The nodes act as filters to remove waste and help to regulate protein content in the lymph fluid.
The lymph fluid is carried through long tubules called lymph ducts, which eventually empty the fluid into deeper and larger vessels called trunks. All the lymph trunks empty into either the thoracic duct or the right lymphatic duct. The thoracic duct drains the left arm, both legs, and three-fourths of the trunk of the body. The right lymphatic trunk drains the right arm and the remaining one-fourth of the trunk. The location of the surgery, tumor, or radiation therapy will predict whether or not the patient develops secondary lymphedema and how severe the lymphedema will be (Penzer, 2003).
Lymphedema results from either a mechanical or dynamic insufficiency in the lymphatic system or a combination of these conditions. In a dynamic insufficiency, the lymph flow actually exceeds the transport capability of the intact lymphatic system. Usually this type of lymphedema has a genetic etiology. This dynamic insufficiency causes primary lymphedema. In a mechanical insufficiency, an injury to the lymphatic system impairs the lymph flow due to paralysis, blockage, or inadequacy of the lymphatic system after surgery, radiation therapy, infection, or tumors. This mechanical insufficiency causes secondary lymphedema, which is the most common type of lymphedema in the United States. Approximately 15%-20% of breast cancer patients develop lymphedema up to 20 years following treatment. With 2 million breast cancer survivors, this number translates into approximately 400,000 patients coping with lymphedema on a daily basis (Petrek et al., 2000).
Lymphedema is categorized as either primary or secondary based on the etiology. In primary lymphedema, some type of developmental abnormality exists in the lymphatic system. Congenital lymphedema accounts for 10%25% of all primary lymphedema cases, with females affected twice as often as males (Revis, 2005). Primary lymphedema may occur at any time in life; however, primary lymphedema is thought to be a genetically determined disease and may present at birth or shortly thereafter (Milroy disease), at puberty (referred to as lymphedema praecox), or after age 35 (lymphedema tarda) (Wyatt & Pribaz, 2004). Primary lymphedema usually affects the lower extremities (International Society of Lymphology, 2003).
Secondary lymphedema is a result of some sort of an insult to the lymphatic system caused by an obstruction or a disruption that prevents the tubules from draining the fluid from the surrounding tissues. It may occur from infection caused by streptococci or filariasis (caused by a parasite), surgery, radiation therapy, or tumors. Lymphedema caused by infection must be treated with an antibiotic or antiparasitic agent (Petropoulos, 2005; Stedman, 2004). Secondary lymphedema may develop after surgery for malignant solid tumors, such as in breast cancer or lymphoma. It also may develop after an axillary node dissection, breast surgery, or after radiation treatments. Because surgery or radiation treatments are the most common cause of lymphedema in the United States, this type of lymphedema is the primary focus of this column. Although lymphedema may have different etiologies, the treatment for all types is the same.
Stages of Lymphedema: Stages I, II, and III
Stage I lymphedema is considered reversible. The lymphedema is pitting and the tissues are soft; the patient may or may not have an increased arm girth or feeling of upper-extremity heaviness. Elevating the affected area may reverse the edema. In Stage II, the lymphedema progresses and may become nonpitting and fibrotic. Stage II lymphedema is considered irreversible and elevation does not reduce swelling. In Stage Ill lymphedema, the tissues become hard and may have cartilage formation developing in the area. The swelling is severe and may form deep crevices. Stage III lymphedema is considered rare in patients with breast cancer (Harris, Hugi, Olivetto, & Levine, 2001).
In 2004, the Oncology Nursing Society (ONS) described a cluster of symptoms that is unique to breast cancer survivors with lymphedema. The cluster of symptoms includes an altered sensation in the limb, fatigue, psychological distress, and decreased physical activity (Muscari-Lin & Ridner, 2004). Other symptoms of lymphedema include:
* Painless swelling of the arms or legs, which may get worse during the day and better at night.
* Warmth or achiness in the extremity.
* A feeling of tightness, heaviness, tingling, numbness, or weakness in the affected extremity.
* Redness of the affected extremity.
* Bracelets, rings, or shoes may become tight.
Changes of sensation in an arm may be an early indication of lymphedema and should be thoroughly investigated (Amer, Radina, Porock, & Culbertson, 2003).
Other causes of lymphedema include (a) metastasis or development of obstructive tumors, such as tumors of the axilla or brachial plexus; (b) lymphangiosarcoma; (c) infection; or (d) axillary vein thrombosis. Lymphoscintigraphy may be used to rule out a systemic cause of the lymphedema and may be used to predict who will develop lymphedema. Lymphoscintigraphy is 100% sensitive and specific for diagnosing lymphedema; however, it is expensive and may not be available at all facilities where medical surgical-nurses practice (Ferri, 2004).
A simple assessment tool for the medical-surgical nurse is preoperative and post-surgical measurements of both arms. The circumference of the arms should be measured at several anatomical positions: (a) finger joints, (b) wrist, (c) 10 cm distal to the lateral epicondyles, and (d) 15 cm proximal to the lateral epicondyles. A difference of greater than 2 cm between the arms at any one of the four measurement points, as well as presence of the ONS cluster of symptoms described previously, may alert the nurse to lymphedema (Brown, 2004). If lymphedema is accompanied by redness and swelling, and an elevated WBC (>12,000/ml) or an elevated temperature (>38[degrees] C or >100.4[degrees] F), the cause may be cellulitis or a local infection rather than lymphedema, and must be treated with antibiotic therapy. Streptococcus Group A usually causes cellulitis, but staphylococcus aureus also may cause it. Treatment would consist of penicillin, dicloxacillin, amoxicillin, ticarcillin, methicillin, or vancomycin for a highly resistant bacteria strain (Gilbert, Moellering, Eliopoulos, & Sande, 2005). Cellulitis may occur concurrently in lymphedema because the pooling of protein-rich lymph fluid makes it easier for the patient to develop an infection. Before treating lymphedema, possible tumor involvement of the axilla or brachial plexus, cellulitis, or even deep vein thrombosis must be ruled out. All these conditions may cause edema of the extremities, which may be misdiagnosed as lymphedema.
Treatment of Lymphedema
The literature is limited in regard to scientifically proven lymphedema treatments because of the lack of prospective, randomized clinical trials that accurately control all variables and measure the amount of lymphedema present. Little rigorous science supports recommendations; however, several professional organizations have guidelines or recommendations (Lacovara, 2006). Compression garments (elastic sleeves) that deliver 20-60 mm Hg of pressure are used for treating lymphedema. A prospective study by Collins, Mortimer, D'Ettore, Ahern, and Moscovic (1995) demonstrated significant decreases in lymphedema in 27 women using the garments. CT exams were used to compare unaffected arms with lymphedema arms using compression for 1, 3, and 12 weeks of treatment (Collins, Mortimer, D'Ettore, Ahern, & Moscovic, 1995).
High-quality compression garments are custom made and should be fitted by properly trained personnel. The practice guidelines of the Canadian Medical Association (CMA) encourage the long-term, consistent use of compression garments when lymphedema occurs. The garments last for approximately 4-6 months depending on how often they are worn and how well they maintain their elasticity. Recommendations about how long to wear the fitted sleeves vary from only during working hours to 24 hours a day. The CMA also provides practical advice regarding skin care, exercise, and weight control (Harris et al., 2001).
Massage and physical therapies have been used with variable effectiveness in treating lymphedema. Complex decongestive physiotherapy includes manual lymph drainage, bandaging garments such as elastic wraps, and exercises. Complex decongestive physiotherapy requires further evaluation in randomized trials (Harris et al., 2001). This therapy usually is performed by physical therapists who specialize in lymphedema treatment, and some rehabilitation centers offer this specialty treatment (Foldi, 1994). The Foldi two-phase approach of intensive skin care, exercise, massage, and compression bandaging has been used widely in the United Kingdom (UK) as a gold standard (Badger, Peacock, & Mortimer, 2000).
Currently, the position paper of the International Society of Lymphology recommends complex physiotherapy by a physician, nurse, or physical therapist who has been professionally trained. It should be noted that compression bandaging, if done incorrectly, may be harmful or useless. Complex physiotherapy treatment consists of two phases. The first phase involves preventive skin care, light manual massage, range-of-motion exercises, and compression with multilayer wrapping. The second phase, which must be initiated promptly after phase one, includes use of a compression sleeve, remedial exercise, and repeated light massage as needed (International Society of Lymphology, 2003). At the Breast Cancer Clinic of the Arizona Cancer Center, patients are advised to wear the compression sleeve prophylactically when flying because pressure changes may trigger lymphedema (NLN Advisory Committee, 2004).
Pneumatic compression devices have been used for treating lymphedema, but insufficient evidence exists to support or reject this treatment. In addition, these devices are expensive and are contraindicated in cases of cellulitis or deep vein thrombosis. These devices are not recommended by any of the professional organizations cited in this column.
Prevention: Sentinel Node Biopsy
The advent of sentinel node biopsy (SNB) should lead to decreased incidence of lymphedema. This newer procedure has changed the way axillary lymph nodes have been evaluated for the last 100 years. The procedure used in the past, known as complete axillary node dissection, removed a large number of lymph nodes and caused about 20% of patients to develop lymphedema. In sentinel node biopsy, a blue dye is injected in the area of the breast tumor and the dye is traced to the first few axillary nodes that drain the breast area. This "blue" node is then checked for malignancy and if it is cancer free it is not removed. Even if the sentinel node is removed along with other "blue" nodes, in many cases fewer nodes are removed than in cases that require complete axillary node dissection (McCarthy, 2003).
Sentinel lymph node removal is better than complete axillary lymph node dissection with regard to lymphedema, range of motion, quality of life, and sensory loss for clinically node-negative patients. More than 5,200 patients participated in the Axillary Lymphatic Mapping Against Nodal Axillary Clearance randomized multi-center trial (ALMANAC) in the United Kingdom. Results were presented at the San Antonio Breast Conference in 2005 (Fleissig et al., 2006). This study provided the first long-term quality-of-life results from a randomized trial comparing SNB with standard treatment. Results demonstrated that SNB is associated with less arm morbidity and better quality of life than standard axillary treatment. Consequently, every oncology surgeon in the United Kingdom is currently being trained in SNB. It is currently the gold standard in the United States and should be offered to all patients at the time of their surgery. Once the surgery has been performed, the lymphatic pathways may be disrupted and so SNB cannot be performed later or after the surgery.
Prevention and Ongoing Care: Nursing Implications
Because breast cancer survivors remain at life-long risk of developing lymphedema, prevention of lymphedema is a very important role for the medical-surgical nurse. Clearly, the prevention of lymphedema is much more effective than treating the problem after it occurs. The National Lymphedema Network (NLN) published a position statement that lists risk-reduction practices, such as proper skin care (avoiding injury, reducing infection risks), appropriate activity levels, avoidance of constrictive clothing, use of compression garments, avoidance of temperature extremes, and other suggestions (NLN Advisory Committee, 2005). This handout for breast cancer patients and others at risk for lymphedema may be accessed online (http://www.lymphnet.org
). The handout may be used by health care personnel in its entirety for educational purposes only. Recommendations in the NLN handout help nurses with ongoing prevention and assessment of problems such as cellulitis to keep patients functioning optimally.
Amer, J., Radina, M., Porock, D., & Culbertson, S. (2003). Predicting breast cancer-related lymphedema using self-reported symptoms. Nursing Research, 52, 370-379.
Badger, C., Peacock, J., & Mortimer, P. (2000). Multilayer bandaging followed by compression hosiery was more effective than hosiery alone in reducing lymphedema of the limb. Cancer, 15, 2832-2837.
Brown, J. (2004). A clinically useful method for evaluating lymphedema. Clinical Journal of Oncology Nursing, 8, 3538.
Collins C.D., Mortimer P.S., D'Ettore, H., Ahern, R., & Moscovic, E. (1995). Computed tomography in the assessment of response to limb compression in unilateral lymphedema. Clinical Radiology, 50(8), 541-544.
Fleissig, A., Fallowfield, L, Langridge, C., Johnson, L., Newcombe, R., Dixon, J., et al. (2006). Post-operative arm morbidity and quality of life. Results of the ALMANAC randomized trial comparing sentinel node biopsy with standard axillary treatment in the management of patients with early breast cancer. Breast Cancer Research and Treatment, 95, 279-293.
Ferri, F. (2004). Ferri's best test: A practical guide to clinical laboratory and diagnostic imaging. St. Louis, MO: Elsevier.
Foldi, M. (1994). Treatment of lymphedema. Lymphology, 27, 1-5.
Gilbert, D., Moellering, R., Eliopoulos, G., & Sande, M. (2005). The Sanford guide to antimicrobial therapy. Hyde Park, VT: Antimicrobial Therapy, Inc.
Harris, S., Hugi, M., Olivetto, I., & Levine, M. (2001). Clinical practice guidelines for the care and treatment of breast cancer: 11. Lymphedema. Canadian Medical Association Journal, 64, 191199.
International Society of Lymphology. (2003). Diagnosis and treatment of peripheral lymphedema: Consensus document of the International Society of Lymphology. Lymphology, 36, 84-91.
Lacovara, J. (2006, January). Preventing secondary lymphedema in the cancer patient. Paper presented at the 37th Annual Focus on Women's Cancers, Tucson, AZ.
Lymphedema. (2004). Patient education handout. RNConsult.com. Retrieved December 21, 2004, from http:// home.mdconsult.com
McCarthy, A. (2003). Now what? Understanding a breast cancer diagnosis. Cure, 2,10-18.
Muscari-Lin, E., & Ridner, S. (2004). Lymphedema: Responding to our patient's needs. Presented at the 29th Annual Oncology Nursing Society Congress, Anaheim, CA.
National Lymphoma Network (NLN) Advisory Committee. (2004). Position statement of the National Lymphedema Network. Topic: Air travel. Retrieved September 18, 2006, from http://www.lymphnet.org/position.html
Penzer, R. (2003). Lymphoedema. Nursing Standard, 17, 45-49.
Petrek, J., Pressman, P., & Smith, R. (2000). Lymphedema: Current issues in research and management. Cancer, 50(5), 292-307.
Petropoulos, P. (2005). Lymphedema. In F. Ferri (Ed.), Clinical advisor. (pp. 491493). Philadelphia: Elsevier/Mosby.
Revis, D. (2005). Lymphedema. Retrieved January 19, 2005, from http://www
Sammarco, A. (2004). Enhancing the quality of life of survivors of breast cancer. Annals of Long Term Care, 12, 40-45.
Stedman, L. (2004). Stedman's medical dictionary. Baltimore, MD: Lippincott, Williams & Wilkins.
Wyatt, L., & Pribaz, J. (2004). Lymphedema. In M. Abeloff, J. Armitage, J. Niederhuber, M. Kastan, & W.G. McKenna (Eds.), Clinical oncology (3rd ed) (pp. 817-831). Philadelphia: Elsevier/Churchill Livingstone.
Jane E. Lacovara, MSN, RN-BC, CMSRN, CNS, is a Clinical Nurse Specialist, Surgery, University Medical Center, Tucson, AZ, and MEDSURG Nursing Editorial Board Member.
Linda H. Yoder, PhD, MBA, RN, AOCN[R], FAAN, is Program Director, Evidence-Based Practice, Outcomes & Research, Adventist HealthCare, Rockville, MD.
Note: This column is made possible through an educational grant from C-Change, a Washington, DC, based organization comprising the nation's key cancer leaders from government, business, and nonprofit sectors. These cancer leaders share the vision of a future where cancer is prevented, detected early, and cured or is managed successfully as a chronic illness. The mission of C-Change is to leverage the combined expertise and resources of its members to eliminate cancer as a (major) public health problem at the earliest possible time. C-Change is both a forum and a catalyst for identifying issues and major challenges facing the cancer community and for initiating collaborative actions to complement the efforts of individual C-Change members. Medical-Surgical nurses are invited to learn more about this important organization by visiting www.ndoc.org
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Arm and Leg Swelling After Cancer
With the advent of better and more effective cancer treatments, the survival rate for all cancers has risen dramatically. With this progress, a new and often misunderstood and misdiagnosed complication has arisen.
Many cancer survivors , having overcome cancer, find themselves with sudden and often unexplained swelling, usually of the arms or of the legs.
This swelling occurs because of one of several factors.
First, the swelling begins after lymph nodes have been removed for cancer biopsies.
Second, the swelling may start as a result of radiation damage to either the lymph nodes and/or the lymph system.
Due to either the removal of lymph nodes or damage to the lymph system, your body is no longer able to rid itself of excess fluids. The fluids collect in the limbs effected and swelling beings.
This swelling is called lymphedema. The swelling that occurs is permanent, and while it is not curable it is treatable.
Permanent Leg or Arm Swelling
****In the situation of any permanent leg swelling whether the cause is known or unknown, the diagnoses of lymphedema must be considered****
There are several groups of people who experience leg or arm swelling from known causes, but it doesn't go away or unknown causes where the swelling can actually get worse as time goes by.
This group includes those who have had the injuries, infections, insect bites, trauma to the leg, surgeries or reaction to a medication. When this swelling does not go away, and becomes permanent it is called secondary lymphedema.
Another extremely large group that experiences permanent leg or arm swelling are cancer patients, people who are morbidly obese, or those with the condition called lepedema. What causes the swelling to remain permanent is that the lymph system has been so damaged that it can no longer operate normally in removing the body's waste fluid.
In cancer patients this is the result of either removal of the lymph nodes for cancer biopsy, radiation damage to the lymph system, or damage from tumor/cancer surgeries.
This is also referred to as secondary lymphedema
Group three consists of people who have leg or arm swelling from seemingly unknown reasons. There may be no injury, no cancer, no trauma, but for some reason the leg simply is swollen all the time.
The swelling may start at birth, it may begin at puberty, or may begin in the 3rd, 4th or even 5th decade of life or sometimes later.
This type of leg or arm swelling is called primary lymphedema
. It can be caused by a genetic defect, malformation or damage to the lymph system while in the womb or at birth or be part of another birth condition that also effects the lymph system.
This is an extremely serious medical condition that must be diagnosed early, and treated quickly so as to avoid painful, debilitating and even life threatening complications. Treatment should NOT include the use of diuretics.
What is Lymphedema?
Lymphedema is defined simply as an accumulation of excessive protein rich fluid in the tissues of the leg. The accumulation of fluid causes the permanent swelling caused by a defective lymph system.
A conservative estimate is that there may be 1-2 million people in the United States with some form of primary lymphedema and two to three million with secondary lymphedema.
What are the symptoms of Lymphedema?
If you are an at risk person for arm lymphedema there are early warning signs you should be aware of. If you experience any or several of these symptoms, you should immediately make your physician aware of them.
1.) Unexplained aching, hurting or pain in the arm
2.) Experiencing "fleeting lymphedema." This is where the limb may swell, even slightly, then return to normal. This may be a precursor to full blown arm lymphedema.
3.) Localized swelling of any area. Sometimes lymphedema may start as swelling in one area, for example the hand, or between the elbow and hand. This is an indication of early lymphatic malfunction.
4.) Any arm inflammation, redness or infection.
5.) You may experience a feeling of tightness, heaviness or weakness of the arm.
How is Lymphedema Treated?
The preferred treatment today is decongestive therapy. The forms of therapy are complete decongestive therapy (CDT) or manual decongestive therapy (MDT), there are variances, but most involve these two type of treatment.
It is a form of massage therapy where the leg is very gently massaged to actually move the fluid out of the leg and into an area where the lymph system still functions normally.
With these massage treatments, swelling is reduced and then the patient is fitted with a pre-measured custom pressure garment to keep the swelling down and/or is taught to use compression wraps to maintain the leg size.
What are some of the complications of lymphedema?
1. Infections such as cellulitis, lymphangitis, erysipelas. This is due not only to the large accumulation of fluid, but it is well documented that lymphodemous limbs are localized immuno-deficient.
2. Draining wounds that leak lymphorrea which is very caustic to surrounding skin tissue and acts as a port of entry for infections.
3. Increased pain as a result of the compression of nerves usually caused by the development of fibrosis and increased build up of fluids.
4. Loss of Function due to the swelling and limb changes.
5. Depression - Psychological coping as a result of the disfigurement and debilitating effect of lymphedema.
6. Deep venous thrombosis again as a result of the pressure of the swelling and fibrosis against the vascular system. Also, can happen as a result of cellulitis, lymphangitis and infections.
7. Sepsis, Gangrene are possibilities as a result of the infections.
8. Possible amputation of the limb.
9. Pleural effusions may result if the lymphatics in the abdomen or chest are to overwhelmed to clear the lung cavity of fluids.
10. Skin complications such as splitting, plaques, susceptibility to fungus and bacterial infections.
11. Chronic localized inflammations.
Can lymphedema be cured?
No, at the present time there is no cure for lymphedema. But it can be treated and managed and most of the complications can be avoided. Life with lymphedema can still be active and full, with proper treatment, patient education, and patient life style adaptation.
For extensive information on lymphedema, please visit our home page:
(c) Copyright 2005 by Pat O'Connor and Lymphedema People. Use of this information for educational purpose is encouraged and permitted. It must be available free and without charge and not used for financial renumeration or gain. Please include an acknowledgement to the author and a link to Lymphedema People.