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By y Jennifer L. Brown, MA, OTR/L
Lymphedema can be defined simply as “an abnormal accumulation of lymph fluid in the tissues of an extremity or other body part.”1 Lymph fluid collects in the tissues when the lymph system becomes impaired. This fluid is rich in protein and encourages fibrosis and infection if left untreated. Primary lymphedema is hereditary or congenital where developmental dysplasias are present in the lymph vessels.2,3 Secondary lymphedema develops when there has been trauma to the lymph system, such as surgery to remove lymph nodes, radiation therapy, tumor, infection, and filariasis. Roughly one in 6,000 people in the United States experiences hereditary primary lymphedema.2 The little data regarding the incidence of secondary lymphedema pertains only to breast cancer patients having had axillary lymph node dissections. Estimates range from 6% to 50% depending on the extent of the lymph node dissection, the type of surgery, and the extent and location of radiation therapy.2-4 An estimated 211,300 women will develop breast cancer in 2003,5 resulting in a growing awareness of lymphedema and the tremendous benefits of early diagnosis and intervention. Lymphedema, if left untreated, can result in disfigurement, disruption in the ability to perform activities of daily living (ADL), cellulitis, skin changes, and wounds. Other conditions such as arthritis or carpal tunnel syndrome (CTS) can be exacerbated by lymphedema. Lymphedema patients have also been shown to experience increased psychosocial problems.6 Although the risk is very low, severe uncontrolled lymphedema can lead to the development of Stewart-Treves syndrome, or the highly malignant lymphangiosarcoma.7,8 PATIENT EVALUATION Once a patient has been diagnosed with lymphedema, he or she should be referred to an OT, PT, nurse, or physician who has completed specialized training in lymphedema treatment. The size of the limb, or extent of the lymphedema, can be assessed using a volumeter or water displacement technique, although it is more commonly assessed by taking circumferential measurements. These measurements are usually compared to the same measurements on the unaffected limb. In situations where it is difficult to take measurements or use water displacement, such as lymphedema of the head and neck, photographs can be useful. Significant swelling can impede joint mobility and therefore range of motion (ROM) should be assessed. Many lymphedema patients have had radiation therapy or surgery that has already resulted in limited ROM. Lymphedema can lead to a worsening of the problem as patients tend to favor the limb due to discomfort, heaviness, or even fear of increasing the swelling. An assessment of the patient’s functional status or activities of daily living (ADLs) is also very important. If the patient has upper extremity lymphedema, she may have difficulty fitting into her clothing, reaching for things, or fastening a bra. If the patient has lower extremity lymphedema, can she wear shoes? Can he bend down to wash his feet? Can he be on his feet long enough to complete necessary tasks such as grocery shopping? The patient’s skin condition should be addressed. Does the area feel fibrotic? Are there any wounds or scratches that need to be addressed? In very severe cases, the skin may be taut and shiny, and the lymphedema may “weep” through the skin. It is important to assess the color of the skin. Generally, a limb with lymphedema should be normal in color; however, there may be redness, which could indicate infection or lymphangitis, an inflammation of the lymph vessels. If the limb is bluish in color, this could indicate a vascular insufficiency or deep vein thrombosis (DVT). If an infection or DVT is suspected, the patient should be immediately referred to the physician for further evaluation. Although lymphedema by itself is not usually painful, pain should be assessed. Frequently, patients complain of a generalized discomfort of the affected area. They may also experience an increase of symptoms from other problems affecting the limb. Usually, these complaints are diminished or eliminated with successful treatment. Finally, the patient’s ability to tolerate treatment needs to be assessed. Thorough management of lymphedema can be cumbersome and difficult. Some patients may not be able to commit the time required. Others may not have help at home or the ability to learn the techniques. Some may be ill and unable to tolerate some or all of the treatment options. TREATMENT METHODS While lymphedema cannot be cured, it can be treated very effectively, even for those patients receiving palliative care for end stages of cancer. Today, the accepted treatment for lymphedema includes several elements. These are manual lymph drainage (MLD), compression bandaging, exercise, use of compression garments and devices, education, and occasionally pneumatic sequential compression pumps. Combinations of these treatment modalities are often referred to as Combined or Complete Decongestive Therapy (CDT), Complex Physical Therapy (CPT), or Complex Lymphedema Therapy (CLT). Reduction of fluid, relief of discomfort, restoration of function, decreased incidence of infection, and the ability to manage the lymphedema either independently or with the assistance of a caregiver are the goals of treatment. These are achieved by using a combination of these techniques and training the patient and caregiver. MLD is a superficial massage that has been shown to facilitate resorption of the large protein molecules found in lymph fluid,9 and increase the frequency of lymph vessel contraction thus increasing lymph circulation.1 The patient or caregiver should be instructed in the technique so they may continue at home. Compression bandaging is a key component of treatment. Several layers of bandages are applied over foam or padding, increasing pressure in the tissue and interstitial space. The increased pressure provides several benefits including supporting tissues that have lost elasticity and preventing further loss of elasticity, softening of fibrotic tissue, enhancing protein resorption, and preventing further accumulation of lymph fluid. It also allows lymph vessels to absorb and transport more fluid by increasing the effectiveness of the muscle pump. It is imperative that low stretch bandages are used as they increase the working pressure, or pressure when the muscles are contracted. Long stretch bandages increase the resting pressure, or pressure when the limb is at rest. This can lead to a constriction of the limb and worsening of the lymphedema. Once the bandages have been applied, the patient can perform exercises to facilitate muscle pump function in the limb. A variety of exercises can be used, but they should address all the muscle groups in the affected limb. Aerobic activity such as walking is also encouraged as long as the patient has compression on the swollen limb. When the lymphedema has been reduced to an acceptable level, the patient is fitted with a compression garment. While compression bandaging’s goal is to reduce the lymphedema, the goal of the compression garment is to prevent further accumulation. Compression garments are available in custom-made and standard sizes. A good fit is crucial for the garment to be effective, prevent a worsening of the lymphedema, and for comfort to ensure that the garment is worn. Garments also come in a variety of compression classes that indicate the amount of pressure. Generally, a compression class 2 (30-40 mmHg) is used for upper extremity lymphedema and compression class 3 (40-50 mmHg) for lower extremity lymphedema. However, this can vary depending on the patient’s tolerance and ability to apply the garment. There is also a variety of other compression devices available for patients who may not be able to or may choose not to use compression bandages. These devices also come in custom-made or standard sizes. They are made of foam enclosed in moisture wicking material. They tend to be bulky, but are generally more comfortable and easier to don than compression bandages. These devices are excellent for areas that are difficult to bandage. Education is crucial for the success of treatment. Because lymphedema patients are at increased risk of infection, education in skin care techniques must be provided. This includes keeping the limb clean and moisturized and avoiding injury or strain, sunburn, and needle sticks. It is also suggested that blood pressure be taken on a nonaffected limb. Education regarding the chronic and fluctuating nature of lymphedema is important so that the patient can have realistic expectations. Patients often need guidance in resuming activities or altering activities to accommodate the recommended precautions. Finally, the patient and caregiver can be trained in self manual lymph drainage and compression bandaging so they may continue once formal treatment is completed. Pneumatic compression pumps have been used to treat lymphedema as well. There is controversy as to their effectiveness10 and whether they should be included as part of a lymphedema treatment plan. Some suggest that they may even be dangerous in that they can push fluid into unaffected areas. They may also encourage an increase in fibrotic tissue as a result of draining water but not protein from the limb.2 Many lymphedema practitioners avoid using the pump altogether. There are several contraindications for lymphedema treatment. Patients with congestive heart failure may not be able to compensate for the increased fluid load in the bloodstream that results from treatment. Patients with acute untreated infections or DVTs should not be treated as well. If these conditions have been or are being treated, and the patient’s physician has approved lymphedema treatment, treatment may proceed cautiously. If pain is present that is not promptly relieved by compression, treatment should be adjusted to relieve the pain, or terminated until the pain resolves. Some believe the presence of malignant tumor is an absolute contraindication, with the concern being that stimulating the lymph system will encourage metastasis.8 Others believe that avoiding lymphedema treatment will not prevent metastasis and that the comfort of the patient is more important. However, at this point there are no data to support either position. When planning a treatment program, it is important to consider several factors. The first is severity. A severe case may require weeks to months of daily treatment while a very mild case may require just a few days. Does the patient have scheduling, transportation, or other obligations that might interfere with treatment? Is the patient undergoing chemotherapy or radiation, or does she have other health problems? Are there cognitive or emotional problems that interfere with the patient’s ability to learn to manage the lymphedema? The patient’s financial status also needs to be regarded. Medicare covers the treatment, but does not cover supplies or compression garments. Other third-party payors may cover only a limited number of treatments or a portion of the supplies and garment costs. Can the patient afford these items? Finally, the patient’s tolerance for treatment should be considered. While it is important to keep lymphedema under control, it is also important to consider whether the treatment causes the patient more distress than the lymphedema itself. CONCLUSION Lymphedema is a serious condition that requires specialized treatment. All patients undergoing lymph node dissections should be provided with education regarding signs and symptoms of lymphedema so they may develop healthy practices to reduce their risk, and to ensure that they seek appropriate treatment at the onset of the condition. It is also imperative that therapists treating lymphedema have specialized training. Inappropriate treatment can, at best, have little effect and, at worst, create additional problems. Lymphedema awareness has increased in recent years due in part to the work of the National Lymphedema Network and also due to an increasing availability of appropriate treatment. However, there is still a need for continued research, and education of health care providers and patients regarding the seriousness of the condition and the availability of treatment. Jennifer L. Brown, MA, OTR/L, is an occupational therapist and manager of rehabilitation services at City of Hope National Medical Center in Duarte, Calif, and has managed the lymphedema program for 6 years. REFERENCES
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