Lymphedema is a condition in which excessive fluid and protein accumulate in the extravascular and interstitial space. This occurs when the lymphatic system cannot either accept or transport lymph (the colorless fluid that bathes the cells of the body, carrying away byproducts of metabolism and helping to fight infection) into the circulatory system (Browse, Burnand, & Mortimer, 2003; Rockson, 2001). Primary lymphedema due to genetic and familial abnormalities in the lymphatic structure or function may occur in 1 of every 10,000 individuals (Townsend, Beauchamp, Evers et al., 2001). Incidence of secondary lymphedema varies depending on the cause. For example, 15% to 20% of breast cancer survivors in the United States may develop lymphedema, and approximately 90 million individuals worldwide may have secondary lymphedema caused by filarial (parasitic) infections (Petrek, Pressman, & Smith, 2000; Townsend et al., 2001). Although the occurrence of lymphedema in palliative care settings is unknown, one lymphedema clinic reported that approximately 30% of their clients were advanced cancer patients with lower limb swelling (Logan, 1995), and lymphedema is cited in the literature as a distressful symptom experienced by patients in palliative care settings (Winn & Dentino, 2004).

Lymphedema is a chronic medical condition requiring careful management. Because lymphedema can develop at any time throughout life (Browse et al., 2003; Foldi, Foldi, & Kubik, 2003), the clinician may have to address both recent-onset (acute) and long-existing (chronic) lymphedema in patients receiving palliative care.

In healthy individuals, blood capillaries and lymphatic structures support fluid exchange at the blood capillary–interstitial–lymphatic interface. Capillary pressure, negative interstitial pressure, and interstitial fluid colloid osmotic pressure collectively exert approximately 41 mm Hg outward pressure, and plasma colloid osmotic pressure exerts 28 mm Hg inward pressure, resulting in a net filtration pressure of 13 mm Hg from the arterial side of capillaries into the interstitial space (Guyton & Hall, 2000). Approximately 90% of this fluid reenters the blood circulatory system through venous ends of capillaries, and the remaining 10% enters lymphatic collectors. Fluid return to the venous end of the capillary is facilitated by the net venous reabsorption pressure of 7 mm Hg created by the imbalance between capillary, negative interstitial, and interstitial fluid colloid pressure of 21 mm Hg outward pressure and the inward plasma colloid osmotic pressure of 28 mm Hg (Guyton & Hall, 2000). Fluid and the larger protein molecules enter the lymphatic system through small one-way valves and are moved by contraction of lymphangions (segments of vessels), contraction of surrounding muscles, and contractile filaments in the endothelial cells through the lymphatic vessels into the blood circulatory system (Ridner, 2002).

When the lymphatic system can no longer transport the normal fluid and protein load or when there is reduced lymphatic transport capacity coupled with increased lymph, lymphedema develops (Browse et al., 2003; Foldi et al., 2003). Lymphedema can arise from either primary (idiopathic) or secondary (acquired) conditions. Primary lymphedema occurs in the presence of malformation of lymph vessels and/or lymph nodes and is associated with many medical conditions (Table 30-1). Primary lymphedema is classified based on timing of first noted swelling (Foldi et al., 2003):

Secondary lymphedema, lymphedema with a known cause, is the most common lymphedema in developed countries (see Table 30-1). It can occur immediately after the known insult to the lymphatic system or have a latency stage and appear many years later (Foldi et al., 2003). Any patient who has had a lymph node dissection or a tumor that impedes lymphatic circulation is at risk for developing lymphedema.

Both primary and secondary lymphedema may appear first as acute and then chronic disease (lasting longer than 6 months) that can progress over time through three stages of severity. Physical presentation of swelling is the same regardless of cause. Initially, in grade I, the limb will swell and pit with pressure, and elevation will relieve the swelling. In grade II, the limb will become firmer, not pit with pressure, and skin changes, hair loss, and alteration in nails may be noted. In grade III, elephantiasis results with very thick skin and large skin folds (Pain & Purushotham, 2000).

Lymphedema assessment may entail evaluation of new-onset acute swelling or preexisting lymphedema. In both cases, it is imperative to assess immediately for infection and, if present, initiate treatment (Feldman, 2005; Olszewski, 2005; Weissleder & Schuchhardt, 2001) (Table 30-2). Awakening with a hot, painful, swollen limb may be the first sign or symptom of lymphedema onset. Likewise, individuals with chronic lymphedema can rapidly develop cellulitis or lymphangitis. Infections can quickly escalate into emergency situations such as life-threatening septicemia. When assessing infection, look for redness, spreading either locally or in a distinct red line. Touch the area to determine if it is warm and/or painful. Observe for oozing or drainage in the area. Check the patient’s temperature. Palpate for enlarged nodes. Many patients report feeling flu-like symptoms both before and after immediate signs of infection, so inquire about aching, fatigue, nausea, vomiting, weakness, dizziness, chills, or sweating. Any of these signs or symptoms requires immediate antibiotic treatment, and, in the case of a patient near end-of-life, hospitalization may be considered.

When an infection is present and appropriately treated and if no signs or symptoms of infection are noted, both subjective and objective manifestations of lymphedema and associated psychological sequelae should be assessed.

When conducting a history and physical examination of a patient with lymphedema, it is important to keep in mind possible differential (or comorbid) diagnoses, such as myxedema, lipidema, deep vein thrombosis, cancer recurrence, chronic venous insufficiency, cellulitis, or other infections (Rockson, 2001).

History should include the following:

Physical examination should include the following:

Lymphedema onset is often multifactorial and challenging to diagnose in palliative care settings (Cheville, 2002). For this reason, it is helpful to distinguish between the progression of established lymphedema due to diminished efficacy of treatment and edema related to direct tumor spread and/or other systemic factors (Cheville, 2002