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Management
Prevention
Treatment
Compression garments
Pharmacologic therapy
Dietary management
Pain management
Complications
Psychosocial Considerations
Prevention
It is important to identify patients at risk for lymphedema early and to begin
preventive monitoring and instruction for self-care. Inadequate nutritional
status, obesity, immobility, and other medical conditions may increase the risk
of developing lymphedema. The following parameters may facilitate early
detection of the condition:
- The ratio of actual to ideal weight.
- Extremity
measurements.
- Ability to perform activities
of daily living (at each physician examination).
- History of contributing factors (e.g., edema).
- Previous radiation
therapy or surgery.
- Concurrent medical illnesses (e.g., diabetes,
hypertension, kidney or cardiac disease, or phlebitis).
Patients should also be assessed for knowledge of their disease and the
potential for developing lymphedema. Deficient lymphatic drainage due to node
dissection and/or radiation therapy predisposes the affected limb to serious
infection. Even minor infection of the limb may lead to significant
lymphedema.
Patients should understand the potential for developing lymphedema and should be
instructed on limb and skin care following surgery or radiation therapy.
(See the list of Considerations for Teaching Patients Prevention and Control of Lymphedema, below.) There is no empirical evidence for these or similar recommendations,
though giving advice to avoid injury and infection in the affected limb seems
intuitive. Lymphedema may occur as many as 30 or more years postsurgery.
Breast cancer patients who comply with instruction on skin care and exercises
following mastectomy show a significantly lower incidence of lymphedema.[1]
Lymphatic drainage is improved by tissue compression from muscular contractions
during exercise. In exercise, muscles squeeze the soft tissue causing lymph to
travel proximally to the vascular system.[2] Therefore, exercise is important
in the prevention of lymphedema. Breast cancer patients should be instructed
on hand and arm exercises following mastectomy. Patients who undergo operative
procedures affecting pelvic lymph node drainage should be instructed in how to
perform appropriate leg and ankle exercises. The physician should determine
how soon the exercise is initiated following surgery. Physiatrists or therapy
professionals should be consulted for a tailored program of exercises for each
patient.
One study of breast cancer survivors who performed twice-a-week weight training over a 6-month period did not note an increase or exacerbation of symptoms related to lymphedema.[3]
Because the recovery rate is increased when lymphedema is detected early,[2]
patients should be taught to recognize the early signs of edema and to report
any of the following symptoms to their doctor: feelings of tightness in the
extremity; shoes that don’t fit; decreased strength; pain, aching, or
heaviness; redness, swelling, or signs of infection. Rings may become tight as
well, but patients are discouraged from wearing them on the side of risk.
Considerations for Teaching Patients Prevention and Control of Lymphedema
- Keep arm or leg elevated above the level of the heart, when possible.
Avoid rapid circular movements that cause centrifugal pooling of fluid in
distal parts of the limb.
- Clean and lubricate the skin of the extremity daily.
- Avoid injury and infection of the affected limb:
- Upper extremities:
- Use an electric razor for shaving.
- Wear gardening and cooking gloves; thimbles for sewing.
- Maintain good nail care; do not cut cuticles.
- Lower extremities:
- Wear foot coverings outdoors.
- Keep feet clean and dry; wear cotton socks.
- Cut toenails straight across; see a podiatrist as needed to prevent
ingrown nails and infections.
- Either upper or lower extremities:
- Suntan gradually; use sunscreen.
- Clean breaks in skin with soap and water, then use antibacterial
ointment.
- Use gauze wrapping instead of tape, but avoid a tourniquet effect.
- Consult physician about rashes.
- Avoid invasive venipuncture, including finger sticks and intravenous
fluid administration, in affected extremity.
- Avoid extreme hot or cold (e.g., heating pads or ice packs) .
- Avoid prolonged and strenuous work with the affected extremity.
- Avoid constrictive pressure on the arm or leg:
- Do not cross legs while sitting.
- Wear loose jewelry and clothes with no constricting bands.
- Carry handbag on opposite arm.
- Do not use blood pressure cuffs on the vulnerable limb.
- Do not use elastic bandages and stockings with constrictive bands.
- Do not sit in one position without change for longer than 30 minutes.
- Watch for signs of infection (e.g., redness, pain, heat, swelling, fever).
Call your physician immediately if signs or symptoms occur.
- Practice prescribed exercises, as instructed.
- Keep regular follow-up appointments with your physician.
- Closely observe all areas of the limb daily for signs of problems:
- Measure the circumference of the arm or leg at intervals suggested by
your physician/therapist at two consistent levels on the limb and report
any sudden increase in size to your physician.
- Sensation may be diminished. Use the unaffected extremity to test
temperatures (e.g., for bath water, cooking).
Treatment
There are two broad categories of conservative management or treatment of
lymphedema: mechanical and pharmacologic. Mechanical modalities include
elevation of the affected limb; manual lymphatic drainage (a form of massage
that mobilizes edema fluid from distal to proximal areas and from areas of
stasis to areas of healthy lymphatics); use of multilayered compression
bandages and custom-fitted pressure-graded garments; and meticulous skin
hygiene to prevent infection. The use of multilayered bandaging for 18 days before the use of hosiery doubled the reduction in limb volume over the next 6 months.[4] A number of these modalities have been combined
in a strategy known as complex physical therapy (or complex decongestive
therapy), which consists of manual lymphedema treatment, compression wrapping,
individualized exercises, and skin care, followed by a maintenance
program.[5-7] Complex physical therapy has been recommended by consensus
panels and is an effective approach for lymphedema that is unresponsive to
standard elastic compression therapy. It must be performed by a properly
trained therapist.
Surgical interventions are not recommended because they are not generally successful
in curing lymphedema. Several techniques that have been tried include staged
excision of the skin and subcutaneous tissue with or without skin grafting and
the Thompson dermal flap, which combines excision of edematous tissue with
burying a shaved dermal flap to establish continuity between the superficial
and deep lymphatic vessels. These methods have minimal success and high
complication rates of skin necrosis, infection, and sensory difficulties.[8]
The oncology patient is usually not a suitable candidate for these techniques.
Compression garments
Compression garments should always cover the entire area of edema. For
example, a stocking that reaches only to the knee tends to become tight and
occludes lymphatic and venous return if there is significant edema in the
thigh. Extremity pumps that use intermittent sequential pneumatic compression
may also be helpful in the management of the edematous limb, though many feel
such pumps are ineffective and potentially counterproductive. The cuff is
alternately inflated and deflated according to a controlled time cycle. This
action increases fluid flow in the veins and lymphatic vessels and prevents the
accumulation of residual fluid in the limb. Compression pumps should be used
only under the supervision of a trained health care professional. High
external pressure can damage superficial lymphatic vessels. Furthermore, when
compression pumps and other techniques are used, caution should be exercised if there
is a potential for residual tumor, which some theorize may be mobilized into
venous or lymphatic channels.
Pharmacologic therapy
Pharmacologic therapy uses antibiotics to treat and prevent bacterial
cellulitis and lymphangitis. Other drugs that have been used include
diuretics, anticoagulants, pantothenic acid, pyridoxine, and hyaluronidase.
These drugs have no proven therapeutic value and may cause adverse
reactions.[9]
It is important to determine the specific etiology of the swelling and to treat
it appropriately. Infection is a frequent sequela of edema and causes
increased capillary permeability, which increases protein deposition in the
tissues. If an infection is diagnosed, appropriate antibiotics should be given
that are effective against gram-positive cocci and, less frequently, fungal
infections. Laboratory data (e.g., complete blood cell count [CBC]) should be evaluated. Because massage
and techniques to encourage drainage would be contraindicated if venous
thrombosis is present, diagnostic tests may be indicated to distinguish
vascular blockage from deep vein thrombosis. If thrombosis is found,
anticoagulation therapy should be given.
Coumarin (Chemical Abstracts Service registry number 91-64-5; NSC 8774;
systematic name 2H-1-Benzopyran-2-one, also referred to as
5,6-benzo-[a]-pyrone), is a compound that has been studied for the management
of high-protein lymphedemas such as those associated with local and regional
treatments for neoplastic diseases.[10,11]
In the United States, dietary supplements such as coumarin are regulated as food, not drugs.
Premarket approval by the U.S. Food and Drug Administration (FDA) are not required
unless specific disease prevention or treatment claims are made. Because
dietary supplements are not required to be reviewed for manufacturing
consistency and no specific standards for dose or purity exist, there may be
considerable variation from lot to lot for all products marketed as dietary
supplements.
Coumarin was formerly used in the United States as a fixative and flavoring
agent in foods and as a pharmaceutical excipient. In response to
investigations by coumarin manufacturers that demonstrated the compound caused
liver toxicity in animals when used in amounts comparable to or greater than
that appearing in human foods, it was reclassified by the FDA in 1954 as a food adulterant. Since that time, its
addition to human foods has been prohibited, and importation of
coumarin-containing foodstuffs from outside the United States is not permitted.
Coumarin is marketed for medical use in several European countries, but its
therapeutic use has not been approved in the United States or Canada.
Adverse effects commonly associated with coumarin include mild nausea and
diarrhea.[10] Liver toxicity has been reported in as many as 6% of treated
patients.[12-15] Patients typically present with increased serum
concentrations of hepatic transaminases, with or without coincidentally
increased serum bilirubin.[16,17] Aberrant laboratory values generally resolve
within a few weeks after coumarin treatment is discontinued; however, liver
pathology may be progressive and fulminant despite withdrawal of the
compound.[18] Long-term toxicity data are sparse for patients who have
received continuous treatment for up to 2 years. The clinical toxicity of
longer durations of coumarin treatment has not been investigated. Animal
toxicology studies have shown that the incidence of coumarin-induced
hepatotoxicity is highly variable between species.[19] Reports of hepatic
toxicity in humans have led to coumarin’s removal from the market in some
European countries as well as in Australia.
In one study, coumarin administered as tablets for oral use at a daily dose of
400 mg was shown to partially reverse edema fluid accumulation, to reduce the
size of swollen extremities, and to decrease the discomfort associated with
lymphedema.[10] However, a double-blind placebo-controlled crossover study of 140
women with lymphedema of the arm following treatment for breast cancer demonstrated that coumarin was not more effective than placebo in the
treatment of lymphedema. This study also found a higher incidence (6%) of
coumarin-associated hepatic toxicity and concluded that coumarin was not a safe
or effective treatment for lymphedema.[11,15]
Diuretics encourage vascular fluid depletion, but they do nothing for excess
protein deposits and could hasten connective tissue fibrosis.[20] Therefore,
diuretics should be used with caution and only for the treatment of excess
vascular fluid due to other causes.
Dietary management
The nutritional status of the patient should be evaluated and supportive
measures instituted as required. Hypoalbuminemia encourages fluid to pass into
interstitial tissues with excess protein and higher colloid osmotic pressure.
The serum albumin level should be kept above 2.5 g/dL. Patient weight
should be monitored, and patients should be encouraged to eat protein-rich
foods and supplements.
Pain management
Patients with lymphedema may experience pain as a result of pressure on nerve
endings or as a result of atrophy or muscle contractures during movement.[1]
Following assessment, pain may be managed with nonopioid analgesics,
relaxation techniques, mild-to-strong opioid analgesics, adjuvant drugs (e.g.,
amitriptyline), and/or transcutaneous electrical nerve stimulation (TENS). The
most successful treatment, however, is reduction of the lymphedema.
Complications
Edematous tissues are less well nourished and more prone to necrosis during
immobility. Therefore, patients with lymphedema should be monitored for areas
of skin breakdown, especially over bony prominences.
Excess pressure on inguinal or pelvic lymphatics may indicate pelvic metastasis
with subsequent interference of bladder emptying. Pressure, in conjunction
with opioids, may cause problems with bowel elimination. Patient
bladder and bowel status should be monitored for signs of urinary retention or
constipation.
Psychosocial Considerations
There are multiple psychosocial and adjustment issues faced by cancer patients
and survivors with lymphedema. Because lymphedema is disfiguring and sometimes
painful and disabling, it can create problems in many aspects of functioning,
e.g., psychologic, physical, and sexual. Historically, however,
inadequate attention was paid to the psychosocial impact of lymphedema. Several
articles have noted that women who develop lymphedema following treatment for
breast cancer encounter more difficulties in each of these aspects of functioning than women
who do not develop the condition after such treatment.[21-23] Additionally,
because the treatments for upper extremity lymphedema can be uncomfortable,
arduous, and time-consuming, the presence of psychologic difficulties can
significantly interfere with treatment efforts. Upper extremity pain in women
following breast cancer can have a highly complex differential diagnosis. One
study has highlighted the deleterious impact of pain on quality of life and
coping in patients with upper extremity lymphedema.[24] Using the European Organisation for Research and Treatment of Cancer (EORTC) Quality of Life Questionnaire C30 (QLQ-C30), a study of 744 breast cancer patients found that those with lymphedema had impaired quality of life.[25]
Another study highlighted the factors associated with psychologic distress
within a group of patients who developed upper extremity lymphedema after
breast cancer treatment. Risk factors for poor adjustment to the condition
include poor social support, use of an avoidant and reclusive style of coping
(some women avoid social situations in which their lymphedema causes a
constant reminder of their cancer experience), and the presence of pain of any
intensity.[21] Group and individual counseling that provides specific
information about preventive measures, the role of diet and exercise, advice
for selecting comfortable and flattering clothing, and emotional support can be
helpful to women coping with lymphedema. (Refer to the PDQ summaries on Normal Adjustment and the Adjustment Disorders and Sexuality and Reproductive Issues for more information.)
References
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Getz DH: The primary, secondary, and tertiary nursing interventions of lymphedema. Cancer Nurs 8 (3): 177-84, 1985.
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Markowski J, Wilcox JP, Helm PA: Lymphedema incidence after specific postmastectomy therapy. Arch Phys Med Rehabil 62 (9): 449-52, 1981.
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Ahmed RL, Thomas W, Yee D, et al.: Randomized controlled trial of weight training and lymphedema in breast cancer survivors. J Clin Oncol 24 (18): 2765-72, 2006.
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Badger CM, Peacock JL, Mortimer PS: A randomized, controlled, parallel-group clinical trial comparing multilayer bandaging followed by hosiery versus hosiery alone in the treatment of patients with lymphedema of the limb. Cancer 88 (12): 2832-7, 2000.
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Casley-Smith JR, Casley-Smith JR: Modern treatment of lymphoedema. I. Complex physical therapy: the first 200 Australian limbs. Australas J Dermatol 33 (2): 61-8, 1992.
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Boris M, Weindorf S, Lasinkski S: Persistence of lymphedema reduction after noninvasive complex lymphedema therapy. Oncology (Huntingt) 11 (1): 99-109; discussion 110, 113-4, 1997.
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Daane S, Poltoratszy P, Rockwell WB: Postmastectomy lymphedema management: evolution of the complex decongestive therapy technique. Ann Plast Surg 40 (2): 128-34, 1998.
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Savage RC: The surgical management of lymphedema. Surg Gynecol Obstet 160 (3): 283-90, 1985.
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Brennan MJ: Lymphedema following the surgical treatment of breast cancer: a review of pathophysiology and treatment. J Pain Symptom Manage 7 (2): 110-6, 1992.
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Casley-Smith JR, Morgan RG, Piller NB: Treatment of lymphedema of the arms and legs with 5,6-benzo-[alpha]-pyrone. N Engl J Med 329 (16): 1158-63, 1993.
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Loprinzi CL, Kugler JW, Sloan JA, et al.: Lack of effect of coumarin in women with lymphedema after treatment for breast cancer. N Engl J Med 340 (5): 346-50, 1999.
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Casley-Smith JR, Casley-Smith JR: Frequency of coumarin hepatotoxicity. Med J Aust 162 (7): 391, 1995.
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Bassett ML, Dahlstrom JE: Liver failure while taking coumarin. Med J Aust 163 (2): 106, 1995.
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Fentem JH, Fry JR: Species differences in the metabolism and hepatotoxicity of coumarin. Comp Biochem Physiol C 104 (1): 1-8, 1993.
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Földi E, Földi M, Weissleder H: Conservative treatment of lymphoedema of the limbs. Angiology 36 (3): 171-80, 1985.
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Passik SD, Newman ML, Brennan M, et al.: Predictors of psychological distress, sexual dysfunction and physical functioning among women with upper extremity lymphedema related to breast cancer. Psychooncology
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Maunsell E, Brisson J, Deschênes L: Arm problems and psychological distress after surgery for breast cancer. Can J Surg 36 (4): 315-20, 1993.
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