Lymphedema is more than mechanical insufficiency due to reduced transport capacity. Lymphedema is an inflammatory edema associated with an increase in the adipose content of the subcutaneous layer and interstitial fibrosis.
Complete decongestive therapy is an accepted and effective combination of techniques that decongests the soft tissue swelling associated with lymphedema.
Multimodal treatment approaches, including manual lymphatic therapy, compression bandaging, exercise, intermittent pneumatic compression, as well as complementary and alternative therapies, are available to clinicians for the treatment of lymphedema.
Cancer survivors who have had treatment affecting the lymph drainage system have a lifelong risk of developing lymphedema.
Lymphedema management includes both risk-reduction education and lymphedema treatment.
While some risks for lymphedema development are generally recognized, there is no consensus on other risk factors and further research is necessary.
Risk-reduction education, individualized to each patient, is a necessary component of cancer survivorship to maximize self-management and adherence to treatment.
Early lymphedema detection and treatment improve outcome.
One of the most studied groups is patients with breast cancer-related lymphedema, but more rigorous research with prospective studies and larger randomized controlled trials should be conducted in this and other populations affected by lymphedema.
The Management of Lymphedema
Lymphedema management includes risk-reduction education and lymphedema treatment. Although cancer treatment-related lymphedema is the most recognized cause of nonfilarial lymphedema in developed countries, most of the management strategies described are also valid for individuals with primary lymphedema and associated disorders, such as phlebolymphedema and lipedema.
Educating At-Risk Individuals
Cancer survivors are a growing cohort—15.5 million Americans, as of January 2016. Survivorship presents a unique set of complex needs, including risk for developing lymphedema. While some survivors develop lymphedema as a late effect of treatment, limited empirical evidence prevents clinicians from predicting likeliness of onset in individuals at risk. Epidemiological reports of breast cancer-related lymphedema (BCRL) and cancer-related lower limb lymphedema vary among researchers, with higher rates noted when researchers combine patient self-report or utilize multiple strategies for measurement and lower rates when the sample is studied for less than 2 years. A systematic review suggests a 21% incidence of BCRL worldwide. Reporting of lymphedema following treatment for cancers other than breast varies from 4–75% for head and neck to 20–31% for gynecological and genitourinary. Factors that may increase risk for lymphedema following cancer treatment are identified in multiple studies, but data are inconclusive for absolute risk ( Fig. 10.1 ).
In contrast to the evidence for cancer-related secondary lymphedema, primary lymphedema remains an even further underdiagnosed, understudied, and undertreated disorder. Compelling work in genetics may one day improve predictability for lymphedema as a consequence of cancer treatment. While definitive criteria for lymphedema risk are lacking, medical professionals must educate patients at risk to allow timely access to care.
Early proponents of risk-reduction education urged health professionals to teach activity restrictions to limit potential triggers for lymphedema, basing recommendations on expert opinion and anecdotal evidence. Some past recommendations lack scientific rigor required for widespread adoption. Today, many advocate for teaching strategies to enable patients to make behavioral changes that minimize lymphatic burden. Some call for more rigorous science before broadly prescribing risk-reduction practices, especially in those with lesser risk, such as breast cancer survivors who have undergone sentinel lymph node biopsy.
Clinicians acknowledge the need for clinical trials to strengthen advice but assert that sound understanding of lymphatic physiology and pathophysiology of lymphedema provides the basis of current risk-reduction recommendations. Patients search for health information independently and may find inaccurate information. Ultimately, patients are better served in receiving direction from healthcare providers.
Knowledge Leads to Early Intervention and Best Practice
Contemporary lymphedema literature emphasizes the need for self-monitoring, early diagnosis, and access to specialized treatment. Educators teaching risk-reduction strategies have multiple aims:
Teach patients to understand personal risks and symptoms.
Make an appropriate response should onset occur.
Minimize stress on the lymph drainage system during typical activities of daily living (ADLs).
Resume ADLs mindfully and with confidence.
Engage in health-promoting activities ( Fig. 10.2 ).
Educational components of lymphedema risk-reduction include explanation of normal lymphatic anatomy and physiology, pathophysiology of lymphedema, signs and symptoms of lymphedema, signs and symptoms of infection, the need for physician diagnosis, and patient-specific personal risk factors ( Fig. 10.3 ). Initiation of services geared toward the prevention of the late effects of breast cancer treatments varies among practitioners in the multidisciplinary team of cancer care. Patients would benefit from improved consistency across disciplines and greater third-party reimbursement for preventative care.
Risk Reduction Is Important for Health-Related Quality of Life and Health-Promoting Behaviors
Research demonstrates that health-related quality of life (HR-QOL) is reduced in breast cancer survivors with lymphedema compared with breast cancer survivors who do not have lymphedema. Some studies find that complete decongestive therapy (CDT) improves HR-QOL and lack of knowledge around lymphedema leads to reduced HR-QOL. A milder stage of lymphedema leads to greater ease of treatment and long-term symptom management. For patients with and at risk for lymphedema, engagement in health-promoting activities can support improved HR-QOL. A growing body of evidence demonstrates exercise improves HR-QOL and may have a protective effect countering the development of BCRL.
However, fear may drive patient decision-making and lead to avoidance behaviors, such as limiting use of the affected arm during routine ADLs or avoiding physical exercise, possibly resulting in deconditioning and weight gain. Patients may implement inappropriate symptom management or ignore symptoms. If healthcare providers do not teach risk reduction, the patient will lack the ability to make informed decisions about lifestyle choices. Deconditioned muscle groups are more readily strained with the addition of exercise or strenuous ADLs, and research suggests that a sedentary lifestyle can contribute to lymphedema risk. The consequences of soft tissue strain may trigger lymphedema, further reinforcing avoidance behaviors and reducing quality of life ( Fig. 10.4 ).
Teaching the Layperson
Concepts of lymphatic anatomy, physiology, lymphatic load, and lymphatic transport capacity are complex subjects for the layperson to digest. Effective teaching requires lessons tailored to allow each individual to analyze personal risk for lymphedema, self-assess routine ADLs, review the activities for impact on the lymph system, and apply appropriate risk-reduction behaviors ( Box 10.1 ).
Skin Care—Avoid trauma/injury to reduce infection risk
Keep extremity clean and dry.
Apply moisturizer daily to prevent chapping/chafing of skin.
Attention to nail care; do not cut cuticles.
Protect exposed skin with sunscreen and insect repellent.
Use care with razors to avoid nicks and skin irritation.
If possible, avoid punctures such as injections and blood draws.
Wear gloves while doing activities that may cause skin injury (e.g., washing dishes, gardening, working with tools, using chemicals such as detergent).
If scratches/punctures to skin occur, wash with soap and water, apply antibiotics, and observe for signs of infection (i.e., redness).
If a rash, itching, redness, pain, increased skin temperature, increased swelling, fever or flu-like symptoms occur, contact your physician immediately for early treatment of possible infection.
Gradually build up the duration and intensity of any activity or exercise. Review the Exercise Position Paper.
Take frequent rest periods during activity to allow for limb recovery.
Monitor the extremity during and after activity for any change in size, shape, tissue, texture, soreness, heaviness, or firmness.
Maintain optimal weight. Obesity is known to be a major lymphedema risk factor.
Avoid Limb Constriction
If possible, avoid having blood pressure taken on the at-risk extremity, especially repetitive pumping.
Wear nonconstrictive jewelry and clothing.
Avoid carrying a heavy bag or purse over the at-risk or lymphedematous arm or shoulder.
These should be well-fitting.
Support the at-risk limb with a compression garment for strenuous activity (i.e., weight lifting, prolonged standing, and running) except in patients with open wounds or with poor circulation in the at-risk limb.
Patients with lymphedema should consider wearing a well-fitting compression garment for air travel. The NLN cannot specifically recommend compression garments for prophylaxis in at-risk patients.
Extremes of Temperature
Individuals should use common sense and proceed cautiously when using heat therapy. Observe if there is swelling in the at-risk limb or increased swelling in the lymphedematous limb, and cease use of heat such as a hot tub or sauna.
Avoid exposure to extreme cold, which can be associated with rebound swelling, or chapping of skin.
Avoid prolonged (greater than 15 minutes) exposure to heat, particularly hot tubs and saunas.
Additional Practices Specific to Lower Extremity Lymphedema
Avoid prolonged standing, sitting, or crossing legs to reduce stagnation of fluid in the dependent extremity.
Wear proper, well-fitting footwear and hosiery.
Support the at-risk limb with a compression garment for strenuous activity except in patients with open wounds or with poor circulation in the at-risk limb.
Following activity analysis, problem-solving, and behavioral changes, one may be able to minimize lymphatic burden that could contribute to the development of lymphedema. In our clinical practice, we have found effectiveness in teaching the following concepts in a community-based cancer survivorship seminar: synopsis of lymphatic system function, simplification of microcirculation concepts, instruction in the body’s lymphatic load, and lymph transport capacity of an intact system contrasted with a system having impairment. We encourage discussion around current risk-reduction practices, exercise options, and compression garment recommendations.
Lymphedema Risk Reduction as a Self-Management Behavior
Researchers studying health promotion with chronic disease equate successful self-management with improved self-efficacy, personal problem-solving, decision-making, and action-planning. Training in self-management is not curative but provides patients with tools necessary for maximal ADL function. Effective self-management for those with or at risk for lymphedema requires learning the signs and symptoms of lymphedema, triggers for symptom exacerbation, and steps to ameliorate swelling ( Fig. 10.5 ).
Preliminary data suggest that self-management reduces healthcare costs. Training modules may be delivered in a variety of formats, including online instruction or live individual or group settings ( Fig. 10.6 ).
Activity Modifications and Best Practice
While lymphedema is not inevitable, one should avoid unnecessary stress on a system with compromised lymph drainage. Avoiding physiological burdens that increase load on the system is wise. Modifications are recommended around the following activities: skin care, limb constriction, body temperature, and healthy lifestyle.
National organizations provide accessible patient information for lymphedema risk reduction ( Box 10.2 ). Coupled with individualized instruction around personal needs, these resources allow training in risk reduction. The International Society of Lymphology cautions against prescribing lists of all-inclusive “Dos and Don’ts” to avoid eliciting unnecessary anxiety in vulnerable populations when science is inconclusive regarding which survivors will one day develop lymphedema.
NLN—National Lymphedema Network
ILF— International Lymphoedema Framework
ALFP— American Lymphedema Framework Project
NCI-NIH—National Cancer Institute at the National Institutes for Health
ACS—American Cancer Society
ONS—Oncology Nursing Society
LERN—Lymphatic Education and Research Network
Susan G. Komen—Susan G. Komen Foundation
NCCN—National Comprehensive Cancer Network
Collaborative care models empower patients by disseminating knowledge, then developing health partnerships supporting self-management. The prospective surveillance model for rehabilitation following breast cancer treatment similarly advocates for health partnerships to allow early access to specialized care, rehabilitation, and health-promotion education. Improving survivors’ sense of control over their lives and supporting healthy behaviors are the obligation of healthcare professionals during the survivorship phase of care.
Self-reported symptoms are shown to be a predictor of lymphedema onset. Patient awareness allows early access to diagnosis, referral for treatment, and participation in normal ADLs. Healthy lifestyle, including normal body mass index and regular exercise, may provide protective effects for the limb at risk for lymphedema. Clinicians should collaborate with patients to improve delivery of knowledge around stratified lymphedema risk, personalized management, and treatment.
CDT is a multimodality approach to reducing and controlling lymphedema. CDT can also be effective for treating phlebolymphedema and lipedema. CDT involves patient education, specialized manual therapy, the application of multilayered short-stretch bandages, skin care, and specialized exercises. Therapists must have specialized CDT training.
CDT is divided into Phase I, treatment, and Phase II, maintenance ( Box 10.3 ). Phase I flows into Phase II. Ideally, Phase I therapy is performed daily until maximal volume reduction is achieved and the skin texture improves.
Phase I: Decongestion
Meticulous skin and nail care
Manual lymphatic drainage
24-hour compression bandaging with non-elastic bandages
Compression garment after the limb reduction stabilizes
Phase II: Maintenance
Compression bandages or a non-elastic appliance at night in cases of moderate to severe lymphedema
Lymphedema exercises in garment or bandages
Continued meticulous skin and nail care
Active engagement on the part of the patient and understanding the rationale for CDT improve adherence to the treatment plan. Patients should also understand that lymphedema is an inflammatory edema that results in interstitial fibrosis (subcutaneous scar tissue) and increased subcutaneous lipid due to lipogenesis. Thus, early detection and treatment are essential to optimal outcomes.
CDT treatment should be individualized to enhance effectiveness and contain costs. Patients with mild lymphedema may not need compression bandaging. Patients with mild nonprogressive lymphedema may be satisfied with no manual treatment or bandaging and may prefer to simply wear a compression garment. Other patients, due to concern about developing progressive lymphedema, will prefer CDT and will wear compression garments every day.
An ADL assessment is integral to lymphedema management. Therapists can identify and address limited joint range of movement, provide mobility training, and improve or provide adaptations to other impaired self-care skills. Psychosocial counseling may be necessary to help patients, significant others, and caregivers overcome emotional and social barriers to treatment and self-care management.
It is important to maintain healthy skin and reduce the risk of bacterial and fungal infections. This can be accomplished with meticulous hygiene, moisturizing the limb and trunk with emollients, and regular inspection of the skin and adjacent trunk for skin irritation or breakdown. Emollients such as bath oils and soap substitutes moisturize and soothe dry, irritated skin. Redundant skin folds and pendulous breasts can be associated with intertriginous skin irritation and tinea or candida infections. Absorptive pads are used to keep the skin folds separated. Nystatin cream, miconazole 2% cream or lotion, or clotrimazole 1% cream can be used to treat the tinea or cutaneous candida infections.
Compression garments and bandages may irritate the skin directly or may cause chafing and increasing moisture. Skin irritation could be a sign of an ill-fitting garment. Rarely, patients develop contact dermatitis owing to the dyes or latex in some fabrics. Latex-free bandages and garments are available.
Elevation decreases swelling by reducing the venous pressure and is more effective for venous or mixed edemas than for lymphedema. While there is no objective evidence that elevation improves the rate of lymphatic filtration, gravity helps the fluid in the distal limb drain into areas with more effective lymph drainage, such as the trunk. Elevation may reduce swelling associated with stage I lymphedema as a staging criteria. As a practical matter, elevation is generally used at night with the arm or leg positioned at or above heart level on pillows or on a foam wedge for all stages of lymphedema.
Manual Lymphatic Drainage
Manual lymphatic drainage (MLD) moves lymph fluid and excess interstitial protein through an increase in interstitial tissue pressure. Treatment duration and frequency depend on the severity and stage of the lymphedema. Phase I intensive treatment lays the groundwork for a successful Phase II self-management program.
The goal of MLD is to increase the transport capacity of the lymph collectors, thus increasing protein absorption. The sequence and direction of the strokes stimulate lymphatic flow and drainage from congested areas. MLD is a gentle pressure technique used in stage I lymphedema because the superficial lymphatics are fragile. The gentle pushing and stretching strokes stimulate the lymphangions and promote propulsion of fluid in the desired direction. Greater mobilization pressure is used on fibrotic tissue, avoiding skin redness and pain. MLD is effective only if there are functioning lymphatic vessels. MLD usually begins by stimulating lymph drainage within the normal, contralateral quadrant of the trunk to suction fluid across watershed pathways. The congested trunk is then massaged to move fluid along skin and subcutaneous collaterals. The lymph is then cleared in a proximal to distal gradient.
Generally speaking, MLD should not be used as a sole modality of treatment. MLD is contraindicated in the presence of cellulitis, radiation dermatitis, venous thrombosis, and directly over cancerous tissue. MLD is not believed to cause metastases in cases of lymphedema appearing after cancer therapy or in the case of malignant lymphedema.
Multilayered Low-Stretch Bandaging
Multilayered low-stretch bandaging (MLLB) is an integral part of CDT. Compression bandages maintain the therapeutic results of manual lymphatic decongestive therapy.
Bandages exert both a resting pressure when the limb is relaxed and a working pressure generated by contracting muscles pushing the skin against the resisting bandages. The highest resting pressure is achieved with long-stretch bandages. Working pressure is highest with rigid low-stretch bandages, while resting pressure is lowest ( Fig. 10.7 ).