In 2018, more than 3.1 million women with a history of breast cancer and those currently being treated for breast cancer will be living in the United States.1 Five-year overall survival is 90%,2 and 6% to 70% of patients will be diagnosed with breast cancer–related lymphedema (BCRL).3 Despite a growing body of knowledge about BCRL, we do not yet have a comprehensive document for patients and caregivers to inform them about (1) early lymphedema signs and symptoms, (2) when to seek treatment from a certified lymphedema therapist (CLT) or physician, and (3) basic lymphedema self-care techniques.
BCRL is a progressive swelling due to accumulation of protein and fluid in the interstitial space that may occur after removal of, or damage to, lymph nodes or lymphatic vessels as is common during cancer treatment.4 If the amount of fluid that needs to be evacuated from an area is greater than the ability of the lymphatic system to carry that fluid, then swelling (ie, lymphedema) may occur.
The most widely accepted standard for lymphedema staging is from the International Society for Lymphology.5 Four stages of lymphedema exist: 0 to 3. Stage 0, or latency, refers to the increased risk of swelling with no visible edema present, although lymphatic system impairment exists, that is, from surgical removal of lymph nodes, scarring, and/or damage from radiation treatment. Stage 1 lymphedema is reversible. Stage 2 is partially or wholly irreversible with treatment. Stage 3 is elephantiasis, which involves an obstruction of the lymphatic system, most often by parasitic disease and rarely if it all secondary to breast cancer treatment complications.
The incidence of breast cancer–related arm, breast, and trunk lymphedema varies widely. Statistics differ secondary to the use of multiple diagnostic criteria for lymphedema, assorted objective measurement tools, diverse medical treatments, genetics, the presence of postoperative infection, body weight, and other unknown factors.6–8
Levenhagen et al3 cited lymphedema incidence as between 6% and 70%. Shaitelman et al9 performed a comprehensive literature review and cited a pooled incidence of arm lymphedema following sentinel lymph node biopsy to be 6.3% (range, 0%-23%) versus axillary lymph node dissection (ALND) at 22.3% (range, 11%-57%). Armer and Stewart6 reported a 5-year incidence of lymphedema to range from 43% to 94%.
Breast and trunk lymphedema is not as widely recognized, but these sites are also at risk for lymphedema as they drain to the affected axillary lymph node group. Degnim et al8 found breast lymphedema was present in 49% of women after axillary lymph node removal versus 0% in women without axillary lymph node removal.
Lymphedema can have a large effect on physical, psychological, emotional, and even financial well-being and thus needs to be included as integral part of the patient's oncology treatment plan.10–14 Lymphedema may negatively affect quality of life; it can increase the risk of poor self-esteem, unhealthy body image, depression, physical discomfort, isolation, and anxiety.12–14 Shoulder impairment, decreased strength, and functional disability have also been linked to lymphedema.10,15–18 Because of the large numbers of women with BCRL and the significant effect on function and quality of life, identification of risk factors and the early recognition of lymphedema symptoms are imperative.
Risk management can be defined as “identifying what could go wrong, evaluating which risks should be dealt with, and implementing strategies to deal with those risks.”19
We can apply risk management principles to the medical management of lymphedema by teaching patients and caregivers advantageous or disadvantageous behaviors that can either decrease or increase their risk of lymphedema, respectively. After this identification of threat, these behavioral changes may positively impact their risk of disease progression. The Lymphedema Action Plan (LeAP) was developed to assist with this process (Figure 1).
LeAP DEVELOPMENT RATIONALE
Lymphedema is a devastating disease that can be an unfortunate side effect of cancer treatment. As little as 10 years ago, standard of care was to wait for swelling to develop prior to the initiation of treatment. Today, however, we know that early education and proactive treatment can reduce lymphedema risk and progression in some cases. Many studies confirm this proactive approach.
Lu et al20 found that early physical therapy combined with lymphedema education significantly reduced the incidence of secondary lymphedema by 65% versus groups that did not receive lymphedema education or physical therapy. In a group of 120 women undergoing ALND, Torres Lacomba et al21 demonstrated that the incidence of lymphedema was 25% in the group that received lymphedema education alone versus 7% for those who received physical therapy plus lymphedema education. Fu et al22 reported that women who received lymphedema education practiced risk reduction behaviors and reported fewer symptoms of lymphedema than those who did not receive lymphedema education.
Unfortunately, proactive education is underutilized. Patients undergoing breast cancer treatment often report that they do not receive enough education regarding lymphedema risk and management.23 Choi et al24 found that only 25% of patients with breast cancer were educated about lymphedema risk prior to surgery, only 17% realized that lymphedema is not completely curable, and 20% believed that lymphedema does not require treatment.
Best practice by all involved medical professionals should include early referral to a CLT after an ALND as well as after a sentinel lymph node biopsy if the patient is in a high-risk category (ie, obese, treatment including chest wall radiation, patients with postoperative infections, and/or patients who complain of early signs or symptoms of lymphedema).
The Lymphedema Action Plan, or LeAP, is a simple, easily read, 1-page handout that was developed in action plan format to help patients and caregivers manage lymphedema risk and treatment most effectively. The idea was adapted from the Asthma and Allergy Foundation of America's Asthma Action Plan.25 The Asthma Action Plan provides guidance to maximize asthma control. The desired outcome of the LeAP is similar: to reduce lymphedema progression by affecting those risk factors that are under the patient's control and by encouraging and guiding early intervention, recognizing that in some cases lymphedema progression may be unavoidable.
The LeAP was designed to empower those at risk for BCRL by educating patients and caregivers about (1) recognized risk factors and proactive behavior modification, (2) early signs and symptoms of lymphedema, (3) actions that can be taken to control an exacerbation, and (4) when to seek a medical provider with the ultimate goal of reducing progression of the lymphedema process.
The LeAP is divided into 3 sections: green light, yellow light, and red light.
Green Light Section
The “green light” section of the LeAP was developed to summarize lymphedema risk factors that are under patient control, together with suggested behavior modification to mitigate those risks, as described by the National Lymphedema Network Medical Advisory Committee26 and clinical experience. Shahpar et al7 found that interventions that promoted a healthy body mass index (BMI), self-care, and patient education may reduce lymphedema incidence and improve quality of life. The ultimate goals of proactive lymphedema therapy are to empower the patient with recommended risk reduction behaviors and to keep patients at stage 0 lymphedema if at all possible.
Suggested behavior modifications include tips for healthy skin care, activity and body weight recommendations, avoidance of tight jewelry/clothing, and precautions with hot tubs/saunas, as well as a reminder to use compression if the patient already has documented clinical lymphedema.
These guidelines should become lifelong behavioral adaptations. Although 89% of lymphedema may occur in the first 3 years after surgery,27 patients who have had lymph nodes removed or damaged are at risk of lymphedema for the rest of their lives.
Many studies have been published in an attempt to better understand what places patients at a higher risk of lymphedema following breast cancer treatment. Lymphedema risks can be categorized as unavoidable/treatment-related, and avoidable/non–treatment-related.
Risk factors that are related to breast cancer treatment and thus may be unavoidable and not under direct patient control include ALND, advanced stage of carcinoma, early postoperative swelling, number of positive lymph nodes, chemotherapy, taxane chemotherapy, radiation, nodal radiation, older age, duration of axillary drainage, and body weight at the time of diagnosis.7,20,28–34,36
Risk factors under patient control include BMI,7,28–31,35 infection, injury, or trauma to the affected region,37,38 and overuse of the limb.37,39
Body Mass Index. The association of preoperative BMI of more than 30 kg/m2 (obesity) with secondary lymphedema is strongly supported in the literature.7,31,33,35 Pretreatment BMI of more than 30 kg/m2 versus pretreatment BMI of less than 25 kg/m2 carried a hazard ratio of 1.59 in a cohort of 666 women treated surgically for stage 1-IIIa breast cancer.35
Clinicians should include weight intake in their assessment and follow those whose BMI is more than 30 kg/m2 closely as they are at an increased risk for lymphedema. Referral to a lymphedema therapist, an exercise physiologist, and/or a dietician may be indicated. It is interesting to note that research has not yet demonstrated whether weight loss during or following breast cancer diagnosis reduces lymphedema risk; this question will hopefully be answered in the future.
Infection. Vigilant healthy skin care recommendations are discussed with the goal of reducing infection risk. Infection prevention is essential to reduce the risk of lymphedema, as postoperative infection increases the risk of lymphedema by 3.8 times.37 Bacteria can enter the body through breaks in the skin such as cuts, cracks, or other trauma. Patients need to be educated about simple ways to reduce the risk of skin trauma such as wearing gloves when gardening, using insect repellent, and caring for dry skin in the winter.26
Prompt attention to cuts with the use of soap and water and/or the use of topical antibiotic and band aids may reduce infection risk. In the past, patients have been instructed to avoid blood draws on the affected side; this is now controversial, as the blood draw site should be thoroughly swabbed with disinfectant and covered with a band aid. Regardless, it may be wise to use the unaffected arm for a blood draw if possible to lower the risk of infection, no matter how slight the advantage.
Overuse of the Limb/Activity Precautions. Activity or exercise involving the affected limb increases blood flow, which imparts an increased strain on the lymphatic and venous systems to export this increased load out of the limb to prevent swelling.4 In years past, patients were advised not to lift more than 5 pounds in an effort to reduce this potential overload. Unfortunately, this did not allow a return to prior level of function, as most activities of daily living (laundry, groceries, children, weight lifting/physical exercise) involve lifting more than 5 pounds.
However, we now understand that progressive weight training is not harmful, and is in fact beneficial, for women at risk for lymphedema as well as for those with lymphedema.40–43 In the PAL Study, or Physical Activity and Lymphedema Study, women with lymphedema who perform supervised resistance training programs had fewer arm and hand swelling symptoms, had 50% fewer lymphedema exacerbations, had increased muscle strength, had less decline in muscle mass during treatment, and did not have any greater incidence of lymphedema than women who do not participate in the exercise program.
It is important to understand that women in the PAL Study followed a gradual exercise progression, were frequently evaluated for swelling exacerbations, and were treated with compression garments if needed.40,41,43 Similarly, gradual return to daily activities such as house cleaning, carrying groceries, lifting at work, etc, is important. Slow progression of activities should occur with surveillance of the arm/hand/breast at risk to ensure that swelling does not occur as exercise and/or activity demands increase.
Yellow Light Section
The yellow light section of the LeAP describes early signs and symptoms of lymphedema and directs the patient to self-care activities such as massage, compression, and exercise (components of complete decongestive therapy, or CDT), as well as a call to their lymphedema therapist, in an effort to initiate treatment early for maximal effect.
Early symptoms of lymphedema include fullness, heaviness, achiness, fatigue, numbness, and/or tingling in the affected area(s). In one study, numbness, swelling, tightness, and tenderness of the limb at risk were the most commonly reported symptoms of early-onset lymphedema.44 Rings, watches, or arm sleeves may feel tighter or bra straps may be more evident as deep skin indentations on the affected side. It may be more difficult to pinch and roll the skin at the base of the second digit of the hand on the edematous versus the nonedematous limb, also known as Stemmer's sign. Veins or tendons may be less visible on the dorsum of the hand, at the ventral aspect of the wrist, or at the antecubital space. The palpated area may feel soft and edematous.
Stout et al45 found that the use of a compression sleeve soon after the onset of swelling (ie, at stage 0-1 lymphedema) may improve overall outcome. Women with breast cancer were evaluated preoperatively for baseline measurements, then postoperatively every 3 months for 1 year, and then again at 18 months. If there was a greater than 3% increase in edema volume in the postoperative limb versus preoperative measurements, they were instructed to wear a compression garment for 4 weeks. This resulted in a significant decrease in arm volume, thus potentially reducing disease progression.
Red Light Section
The red light section of the LeAP teaches the patient to identify common signs and symptoms of infection (redness, heat, acute edema) in the affected area. It then clearly directs the patient to seek immediate physician-based medical attention if those signs and symptoms are present. Without proper education and prompt to action, patients may not realize the potential ramifications of infection in terms of progressive illness as well as the increased lymphedema risk secondary to that infection.
Inflammation and infection are often cited together in the literature as recognized risk factors for lymphedema. Mak et al37 found a 3.8 times greater incidence of lymphedema in patients with infection/inflammation compared with patients without infection/inflammation in the affected breast, chest, or arm. This may be due to the inability of the impaired lymphatic system to remove extra fluid from the affected area, which may result in swelling.7,31,37,46
The LeAP was designed to provide patients and caregivers with a tool that summarizes lymphedema risk reduction techniques, teaches the patient how to identify early signs and symptoms of lymphedema to promote early referral to physical therapy to optimize lymphedema prognoses, guides patients to self-care techniques in case of lymphedema exacerbation, and promotes immediate presentation to a doctor in the case of infection. Despite a growing body of risk reduction educational tools,26 this is the first tool designed to educate patients about early signs and symptoms of lymphedema and further guide them to symptom management and professional treatment by a CLT or physician as appropriate.
The LeAP was designed to be an adjunct tool to raise lymphedema awareness and stimulate discussion. It has been used with our BCRL patients along with other lymphedema educational tools during their initial 1 to 2 visits in the physical therapy/lymphedema clinic, as well as during preoperative visits if the patient is in the high-risk category (ie, obese, radiation treatment, ALND, postoperative infection, or presenting with early signs of swelling). We have also used it to facilitate discussion in patients both preoperatively and postoperatively, as lymphedema is a real and fearful concern of many patients.
The LeAP may also be used as an informational handout by nurses, physicians, or other medical personnel who have the opportunity to interact with the patient during consultation and/or treatment. Unfortunately, lymphedema therapists are not yet a standard part of the primary oncology health care team. Thus, by empowering other caregivers to provide this information, we can stimulate discussion and direct patients to a lymphedema therapist if questions or concerns arise.
Knowledge is power. By teaching patients to address modifiable risk factors, we may be able to reduce the overall incidence of lymphedema by transitioning from a reactive approach to a proactive approach.
The LeAP is a risk management tool designed to build a bridge between professional lymphedema knowledge and patient-directed care. It can be used clinically to promote better lymphedema outcomes as well as to maximize lymphedema control as part of a comprehensive treatment plan.
At this time, there have not been any trials to test the validity of this tool, but the author's clinical experience has been that patients find it easy to understand and use. It has prompted patients to present to physical therapy during acute lymphedema exacerbations, many of which were resolved with manual lymphatic drainage, compression, and exercise. It has also influenced the decision of some patients to present to the emergency department or their physician upon onset of infection in the affected extremity, which some patients stated that they may otherwise have ignored or delayed.
Of utmost importance, the LeAP can be used to improve lymphedema awareness and outcomes in patients with breast cancer, with the goal of being an integral part of a proactive, multidisciplinary breast cancer treatment plan.
The author thanks Linda Koehler, PT, PhD, at The University of Minnesota and Rochelle Chiffelle, DNP, RN, FNP-C, at the Cancer Transplant Institute at Scottsdale Healthcare for their encouragement and assistance with manuscript revision. The author also thanks Therese Owen, PTA, for her aid and clinical expertise.
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