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Wound Series Part 4: Lymphedema and Chronic Wounds

2.00 Contact Hours
A score of 80% correct answers on a test is required to successfully complete any course and attain a certificate of completion.
Authors:    Linda J. Cowan (PHD, ARNP, FNP-BC, CWS) , Mary Nametka (MSN,RN,CWS,CWON,DAPWCA,FNP-BC)

Outcomes

This course prepares the healthcare to diagnose, treat, and refer patients with lymphedema and chronic wounds. It also addresses the differences between short stretch and long stretch bandages and why it is important to know'

Objectives

  1. Review etiological factors of lymphedema.

  2. Discuss diagnosis of lymphedema

  3. Introduce the CEAP-L classification system

  4. Review the treatment and management of lymphedema

  5. Describe differences between lymphedema and lipedema

  6. Identify where to refer patients for follow up or therapy

  7. Review the differences between short stretch and long stretch bandages and why it is important to know this in the treatment options for lymphedema

  8. Discuss the role of lymphedema in some chronic wounds

Please note, this module is not meant to be a “how to” text on how to do lymphedema therapy. Nor is the goal of this educational program to make the reader a lymphedema specialist or therapist, but rather to provide the reader with a basic overview of the problem of lymphedema and become familiar with diagnostic, differential and treatment options.

Lymphedema Overview

When interstitial body fluids (containing foreign debris, proteins, white blood cells and microorganisms) leave the interstitial spaces and enters the lymphatic system, it is referred to as lymphatic (or lymph) fluid. The lymphatic circulatory system is similar to the cardiovascular circulatory system, as far as having lymph vessels and channels and two types of capillaries. However, it does not have the benefit of cardiac muscle contractions to pump lymph fluid along and there is not a continuous string of vessels that connect to one another directly and so it can be described as a relatively passive system of fluid movement.

Lymph vessels are typically larger than capillaries but smaller than veins. Most lymph vessels also have valves to keep the flow of lymph fluid uni-directional – toward the heart and preventing back-flow or clotting. The lymph system also has hundreds of lymph nodes which serve as filters for the lymphatic fluid (Lawenda, et al., 2009, NLN white paper, 2011). There are two separate systems of lymph drainage within the body: those found within the subcutaneous tissues, which drain lymph fluid which has diffused through capillaries from the superficial tissues and skin of the body, and those that drain lymph fluid diffused from tissues within the deeper spaces and structures of the body such as the head, neck and thorax. The movement of fluid through the lymphatic drainage system occurs mainly by muscles contracting and increasing pressure against other structures, effectively “squeezing” the fluid from one place to another.

The deeper lymphatics drain the deep tissues such as muscle, and follow a pathway similar to the cardiovascular blood vessels. The superficial lymphatics are in the subcutaneous fatty layer of the body and drain the subcutaneous tissues and skin.  The deeper lymphatic system connects with the more superficial lymphatic system through perforating vessels which pass through the fascia (fibrous layer covering muscle).  However, these vessels do not connect directly, but rather have open spaces between them. The lymph fluid (containing proteins, cellular debris and fluids which have escaped from cells and tissues and semi-permeable blood vessels) passes from the intercellular spaces of tissues into the lymphatic vessels, where they are directed to lymph nodes – the filters of the body. The lymph vessels return the filtered lymph fluid to the venous system. The smallest of the lymph vessels which are blind at one end and collects lymph fluid in tissues and organs is also known as lymph capillaries, although they are not the same as those found in the venous-arterial circulatory system.

Until recently it was not thought that the lymphatic channels crossed the midline, yet new research reported by Fife et al. (SAWC oral presentation Fall, 2014), suggest this is not the case.  Fife et al. demonstrated with fluoroscopy and radio-opaque tracers that during lymph massage, Lymphatic fluid was seen to cross the midline of the body (head, neck, chest, trunk),

A severe medical problem may occur when lymph fluid does not drain through the lymphatic system adequately. This is called lymphedema and is more often unilateral in presentation. Lymphedema may be mild and hardly noticeable to the patient or clinician, or it may be severe and extremely debilitating (some lower extremities have been reported to get so large, they exceed the size of the rest of the body) and may become life threatening. Over time, if left untreated, lymphedema may result in further lymph vessel damage, chronic inflammation, and what is often referred to as fibrotic changes or hardening of the skin (NLN, 2011, Vojackova, Fialova & Hercogova, 2012).  It has been suggested that it is the prolonged sequestering of accumulated lymph fluid which leads to cross-linking of proteins and other components of the fluid leading to the fibrotic changes that if untreated, makes movement and reduction of the built-up fluid increasingly more difficult to achieve.

Etiological Factors of Lymphedema

Lymphedema is caused when there is an excessive build-up of interstitial fluid in any part of the body due to a pathological disruption in the lymphatic drainage system (Lu et al., 2009, NLN, 2011). This may occur when lymphatic vessels are deformed, injured, or chronically over-taxed. It may appear at any age and may be sudden (such as after surgery) or progressive and worsen over time (such as when due to filariasis) (NLN, 2011).  However, not all edema or swelling is due to lymphedema. In addition, lymphedema may occur concurrently with other forms of edema, such as uncontrolled venous insufficiency, lipedema, and cellulitis.

Lymphedema should not be confused with lipedema. Lipedema (may also be called lipoedema or lipodema) is really a fatty tissue disorder, caused by an abnormal deposition of adipose tissue with hypertophic or hyperplastic fat cells that trap fluid in tissue (Fife et al., 2010). This typically occurs symmetrically in the lower extremities above the ankle but may also occur in the upper portion of the upper extremities. One of the easiest ways to clinically differentiate Lipedema from Lymphedema is that fact that in Lipedema, the feet are spared and will appear quite normal in shape compared to the often puffy “box foot” of someone with lymphedema.

Lymphedema vs Lipedema

Source: Wikimedia Commons

Lipedema may be hereditary and occurs almost exclusively in women over the age of puberty, which has led to theories that estrogen/progesterone may play a role in the development of this problem. One German study suggested that 11% of the general female population have lipedema and up to 17% of women with lymphedema also have lipedema (Foldi, 2006; Herbst, 2014). Persons with lipedema will typically have minimal pitting edema and a negative Stemmer sign (screening test for lymphedema, see clinical pearl box below). In addition, persons with lipedema have pain or tenderness with pressure to the affected tissue and may experience easy bruising. They also may experience persistent enlargement despite limb elevation or weight loss. To complicate matters, persons with lipedema may experience secondary lymphedema due to damaged blood vessels and tissue (altering lymphatic flow) over time (Karen L. Herbst, PhD, MD website (2014)). Unfortunately, persons with lipedema and/or lymphedema are often under-diagnosed, misdiagnosed or just dismissed as being obese (Fife et al, 2010).

Clinical Pearl: Checking for Stemmer’s sign as a screening for lymphedema. It is diagnostic for lymphedema if you cannot pinch the skin on the anterior surface of the base of the second toe (or the base of the second finger on the dorsal aspect of the hand for the upper extremity). If you can gently pinch and left this skin, it is negative. If you cannot due to thickened skin, it is positive (NLN, 2011).

 Stemmer's Sign Test

There are two main types of lymphedema: primary and secondary lymphedema. Primary lymphedema is typically congenital or due to a genetic disorder of the lymphatic system and is more common in women. Secondary lymphedema is much more common than primary lymphedema and is an acquired impairment of the lymphatic system, typically due to infection, trauma or as a complication of medical treatment, especially surgery and radiation and may also result from damage to lymphatic vessels due to chronic venous insufficiency (Vojackova, Fialova & Hercogova, 2012; www.uptodate.com, 2014). Cancer involving the lymphoid tissue is known as lymphoma. Lymphoma will not be discussed in depth in this module.

Multiple types of cancer and cancer treatment is responsible for a large number of the estimated 3 to 5 million cases of lymphedema in the United States (Lawenda, et al., 2009). For instance, after breast cancer surgery and treatment, women have a 10-30% risk of developing lymphedema of the upper extremity on the side of surgery/radiation.  It has been reported that the risk of developing lymphedema after any type of cancer is 15.5%. This risk is a lifelong risk (NLN, 2011).

Lymphedema Classification System

A universal classification system does not currently exist. Most classifications systems recognize primary or secondary lymphedema as “classifications” of the disease. However, attempts have been made to refine lymphedema classification schema and staging over the past 30-40 years (Szuba & Rockson, 1998). Browe and Stewart did a nice job of describing the pathophysiology and classification of lymphedema in 1985 but as more advanced diagnostic tools evolve, so does our need for a consistent and less subjective lymphedema classification system. The most widely accepted staging system for lymphedema is a four staging system (Stage 0 through 3) proposed by the International Society of Lymphology (ISL). This staging system is traditionally only used for staging lymphedema of the limbs (arms and legs). Information for the following lymphedema staging table resource was provided by Bryant & Nix (2012, chapter 13) and the Lymphnotes website, online at: http://www.lymphnotes.com/article.php/id/474/ (2012) and is based on information contained in the June 2009 Consensus Document of the International Society of Lymphology.

Table 1: Common Lymphedema Staging
STAGE OAlso known as the latent or preclinical stage, this is a newer stage added to the classification.

At this stage the patient is at risk of developing lymphedema; however no swelling or other visible evidence of impaired lymph transport is present.

Stage 0 can be present for months, or years, before any more serious signs appear.
Studies indicate that through the use of bioimpedance it is possible to identify changes in the “at risk” limb before they become visible. When changes develop, if specialized treatment is started immediately, it may be possible to prevent the development of further stages of lymphedema. Bioimpedance is a non-invasive method of determining the composition of body tissues to evaluate the presence of body fluids such as lymphedema. Such measurements have been used to establish a baseline at the time of certain cancer treatments.
STAGE IStage I lymphedema is an early accumulation of fluid that is relatively high in protein content.

There is visible swelling with  protein-rich lymph, however, no palpable fibrosis.

This swelling can be temporarily reduced by elevation of the limb (reversible pitting edema that begins distally); however, the swelling soon returns when the limb is returned to a normal position.

Negative or borderline Stemmer sign.
The swollen tissues are soft and pitting edema is present.
Lymphedema treatment should be begun as soon as signs are early detected. Waiting for the swelling to increase, or for an infection to develop, only makes the condition more difficult to treat. Prompt treatment of this stage can often control the condition and may prevent it from becoming more severe.

STAGE II

Stage II lymphedema is an increase in the swelling and a change in the tissues. (Minimally pitting or non-pitting (brawny) edema.

Elevation of the limb will not reduce the swelling.

Positive Stemmer sign.

Hyperkeratosis (thickening of skin)

The tissues become increasing firm due to fibrosis. Fibrosis is the formation of fine scar-like structures within the tissues that cause them to harden.

Papillomatosis of skin (rough cobblestone appearance and texture).

Pressure against the limb produces only a slight indentation or in indentation at all.

The tissue changes at this stage increase the risks of even greater swelling, fibrosis, infections, and skin problems.
Stage II lymphedema can usually be improved with intense treatment.

STAGE III

Stage III lymphedema, which is also known as lymphostatic elephantiasis (massive enlargement and disfigurement of limb), is a condition in which the tissue becomes extremely swollen and thickened due to a blockage in the flow of lymph, chronic buildup of fluid in tissues and loss of elastic components of skin.

The tissues become increasingly fibrotic (hardened). Pressure does not produce any pitting.

Normal elasticity is lost and the skin hangs in folds.

The skin may change color.

Papillomas are small solid benign tumors that project above the surrounding tissue, may develop.

Hyperkeratosis is an increase in the thickness of the outer layer of the skin, can develop.

These changes in texture of skin are disfiguring and can limit mobility.

Infections become more common because of increased risks of breaks in the skin. These infections include fungal infections and open wounds that form within the folds of skin.

Skin ulceration.
With intense therapy, Stage III lymphedema can be improved and potentially be prevented from becoming worse; however, it is rarely reversed to an earlier stage.

Nevertheless, in a more recent consensus document, “Diagnosis and Treatment of Primary Lymphedema: Consensus Document of the International Union of Phlebology (IUP)-2013” experts admit that “most of the currently available staging systems reflect only tissue turgor and limb shape, etc. and neglect other critical clinical information (e.g. number of major joints with changes in tissue composition) as well as socioeconomic status to reflect quality-of-life (QoL) properly.”  It is apparent that more research is needed in this area.

Eberhardt & Raffetto, 2005 described a “CEAP” Classification of Chronic Venous Disease but it does not include lymphedema, even though they recognize that chronic venous insufficiency may lead to the development of lymphedema. (Lee et al., 2013). The acronym CEAP was developed to be a means of stratifying patients into categories based on the presentation of their venous disease and symptoms. “C” represented Clinical presentations, “E” represented Etiological considerations and diagnostic results, “A” represented Anatomical locations and manifestations, and “P” Pathophysiologic events and skin/tissue changes leading to skin ulceration or other complications. Initially, these classifications used a complex and detailed scoring system which many clinicians felt were too cumbersome, so a 6 stage classification was developed under the CEAP umbrella: CEAP 1 = Reticular and spider veins (cosmetic problem, no need to refer), CEAP 2 =Varicose veins only (recommend routine referral to vein specialist for duplex & photoplethysomography assessment), CEAP 3 = Varicose veins + leg swelling (recommend quick referral to vein specialist for duplex ultrasound & photoplethysomography assessment), CEAP 4 = Varicose veins + evidence of venous stasis skin changes (recommend quick referral to vein specialist for duplex ultrasound & photoplethysomography assessment), CEAP 5 = Varicose veins + history of healed venous stasis ulceration (recommend quick referral to vein specialist for duplex ultrasound & photoplethysomography assessment), CEAP 6 = Varicose veins and an open venous ulceration (recommend urgent referral to vein specialist for duplex ultrasound & photoplethysomography assessment and refer to wound care center or wound specialist for wound assessment and management) (McNeill & Rosenberg).

Gasbarro et al. (2009) adopted the acronym C.E.A.P. (used for venous disease) and added an “L” (CEAP-L) to be more specific to Lymphedema. Gasbarro et al. designed the CEAP-L “based on the most objective sign in these patients” (edema), and subdivided the edema into 5 different classifications or levels that are dependent on the clinical manifestations noted by the clinician. “The etiological aspect considers 2 types of alterations of the lymphatic system: congenital and acquired. The anatomic is aimed to locate the anatomical structures involved. Pathophysiological conditions are gathered into 5 groups: agenesia or hypoplasia, hyperplasia, reflux, overload, obstruction.” While all of these classification and staging systems are promising (and better than nothing), the most important factor in the treatment of lymphedema is to identify it in patients first and as early as possible. Winiwarter described the development of lymphedema in the scientific literature as early as 1892 (Wittlinger et al., 2011), yet Poage et al. (2008) suggest that only about 36% of individuals with lymphedema will actually seek treatment in the early stages, even after cancer surgery when they are told it may be a complication. Unfortunately, there is not a lot of information about how many cases of lymphedema are misdiagnosed, but clearly, earlier intervention and patient education/support is needed.

Diagnosing Lymphedema

As with any disease or condition, diagnosis begins with a thorough history and physical examination. If secondary lymphedema is suspected after surgical excision of lymph nodes, or following procedures such as revascularization or knee replacement surgery, imaging diagnostic tests may be minimized or deemed unnecessary. However, if evaluating a patient with unknown etiology of edema, suspicious for lymphedema, a simple diagnostic test using the Stemmer’s sign is recommended. See clinical pearl box above.  Early detection (sometimes even before physical signs or symptoms) may be accomplished using a bioimpedance spectroscopy (BIS), where an electrical current is passed through an extremity and impedance is measured. Reduced impedance is suggestive of lymphedema. BIS may also be used to monitor treatment effectiveness (Bryant & Nix, 2012, p 218).  Diagnostic tests may also include MRI, CT scan, and Doppler ultrasound, which may help to identify where there is a potential blockage of the lymphatic system (such as a tumor growth). Lymph fluid analysis is also sometimes conducted (protein content between 1.0 and 5.5 g/dl usually indicates lymphedema). Another diagnostic test (gold standard) provides actual visualization of lymphatic vessels by lymphoscintigraphy (a radionuclide imaging test showing the movement of a radioactive dye through the lymphatic system). Genetic testing may be recommended in patients with primary lymphedema.

There are basically 4 stages of lymphedema: a latency stage (a pre-stage or stage 0, where physical symptoms are mild and reversible), Stage 1, Stage 2, and Stage 3 (Poage, E. (2008), p. 952). [See Table 1 in previous section)]. Chronic lymphedema has no cure (Bryant & Nix, 2012).

Implications of Lymphedema: Chronic Wounds

Lymphedema creates a management challenge for the clinician trying to manage a patient with a chronic or complex wound. In a chronic, non-healing or slowly healing leg ulcer, lymphedema may often be mistaken for venous insufficiency, and indeed these two problems may occur concurrently. Likewise, cellulitis may be blamed for edema related to lymphedema. To complicate matters, cellulitis, lymphedema and venous insufficiency may also occur concurrently, making diagnosis of one or all three problems difficult. The important things to remember is to conduct a thorough assessment (physical examination and history) including palpation of the skin in the affected area and the contralateral side/extremity (look for Stemmer’s sign as a screening tool if applicable) (Bryant & Nix, 2012). Mentally list lymphedema in your differentials any time you have a non-healing wound with any edema and/or surrounding tissue changes present, and/or suspicious history (such as previous serious injury to nearby soft tissue or cancer surgery). One of the key goals in your treatment of the chronic wound complicated by lymphedema should include fluid management / promoting lymphatic flow in the affected extremity / area.

Treatment and Management of Lymphedema

Main tenants of lymphedema management must revolve around fluid management and may be grouped into 3 main approaches: Compression, Manual Lymph Therapy or Drainage (Massage), and Surgery (Vojackova et al, 2012; Lawenda, Mondry & Johnstone, 2009). Preoperative reduction of poorly controlled lymphedema is considered helpful in promoting successful healing of the incision. Reconstructive surgeries such as Lymphatic transplant, debulking or bypass procedures may be considered in extreme cases however healing is often difficult and does not eliminate the need for lifelong maintenance treatment and control with appropriate compression garments.

Compression

It is important to know the differences between short stretch and long stretch bandaging systems when considering treatment options for lymphedema. Short stretch bandaging systems provide a firm “wall” for muscles to push against during bodily movement – this compresses or “massages” the tissue where lymphedema is present and helps to move the lymph fluid and prevent the accumulation of lymph in the affected area(s). Ace bandages are long stretch bandages and not appropriate in most cases for lymphedema compression treatment.

Manual Lymphatic Therapy (MLT) or Manual Lymph Drainage (MLD)

Several schools and programs exist to teach this (such as the DR. Vodder School International). Developed in 1932 in France, MLD as we know it today is essentially a “light skin” technique (they don’t like to refer to it as “massage” because massage is typically a deeper tissue/muscle technique). This technique of MLD involved gently stretching and twisting the skin in a “light skin touch” in precise directions in order to stimulate the movement of lymph fluid out of the tissue and into the superficial lymph channels and back into the vascular circulation.

Skin care is a key element of successful lymphedema management. According to Nowicki & Siviour (2013, pp. 62-63), edematous stretching of the skin can lead to reduced flow of nutrients to the skin with associated dryness due to reduced oil secretion, leading to cracking and fungal or bacterial infection. Changes in the skin associated with chronic lymphedema are described as hyperkeratosis typified by a thick build-up of scale and plaques, lymphagioma appearing as blisters caused by dilated lymph vessels and papillomatosis which presents with a ‘cobblestone’ appearance (p. 62). Cleansing and moisturizing the skin daily is important as a means to promote moisture retention and prevent cracking. However, use of soap which is typically alkaline, is discouraged for the potential to disrupt the skin’s protective natural ‘acid mantle.’ Soap substitutes are preferred and may be combined with bath oils.  Atraumatic removal of hyperkeratotic build up should be considered to facilitate action of topical moisturizers. A relatively new method involves use of a microfiber pad (Debrisoft®) to effect mechanical debridement. Moisturizers are recommended to be placed after bathing and prior to application of compression wraps or after removal of knit compression garments.

Exercise

Exercise may also fall under MLT because exercise can help to move the lymphatic fluid through lymph channels by the action of muscle contractions acting as a “pump” especially if compression garments are worn.  There are also quilted garments specially designed to fit any body part, with integrated channels to facilitate lymph flow that can be worn during the night (e.g., Reid Sleeves ® and Jovi Paks ®), to help maintain control while standard compression garments are removed for sleep.  Adjunctive therapies such as Jovi paks and Reid sleeves or other types of padded compression modalities are good options for caregivers to know about as they can be sometimes better tolerated than wraps or knit garments; some patients will use these when the swelling has become too painful or becomes temporarily out of control. See more descriptions of potential compression garments available on: http://www.lymphnotes.com/article.php/id/232/.

When and Where to Refer Patients for Therapy and Other Resources

Any patient with suspected lymphedema, especially when the referring provider is not a specialist in lymphedema management, should be referred to a certified lymphedema therapist (CLT). These specialists are critical in providing a comprehensive lymphedema management plan which will likely include some form of manual lymphedema drainage therapy (also called decongestive therapy). In most cases, lymphedema is a life-long condition. In most management plans, manual lymphedema therapy is typically combined with compression garments and sometimes, at-home manual lymphedema therapy taught to a caregiver or achieved with the use of lymphedema pumps (which use segmented/sequential gentle pulsing massage to move lymph fluid toward the body’s central venous circulatory system).

Manual lymphatic drainage therapy is best accomplished by highly trained individuals and they can determine an appropriate treatment plan. Unfortunately, certified lymphedema therapists are not available in every part of the United States, but hopefully, this is changing. Physicians, nurses, nurse practitioners, physician assistants, physical therapists, physical therapist assistants, occupational therapists, occupational therapist assistants, athletic trainers, and massage therapists may receive specialized training to become a Certified Lymphedema Therapist (CLT). These are some valuable resources:

Lessons Learned:

Both of the authors have first-hand personal experience with lymphedema. What we have learned (even through writing this module) is that most clinicians, including wound care specialists are not quick to identify lymphedema, particularly if it is mild and the patient does not complain about it.  The best advice for persons who experience any type of edema or swelling or tissue changes in one particular part of the body is to educate yourself on the possibility of lymphedema playing a part in the etiology.

Questions to ask yourself if you suspect lymphedema is a problem for you or your loved one:

  • Do you have swollen lymph nodes in the area? Note: Lymphedema does not always present with swollen lymph nodes, but if they are chronically slightly enlarged, this is important to note. Chronically enlarged lymph nodes (with or without tenderness) should be reported to your health care provider for evaluation.
  • Are they tender? – tender swollen lymph nodes (especially acutely so) could be a sign of infection or other disease process – report these to your health care provider if they continue to be swollen and tender after 7 days (or if accompanied by fever > 100.4 F and/or other symptoms such as sore throat, unusual drainage or discharge from body, skin lesions or sores)
  • Do you have other edema or swelling in the same area/extremity?
  • Is the swelling pitting?
  • Have you ever experienced soft tissue injury or surgery above this area (more proximal to the center of the body)?

If you answered yes to any of these questions, try the Stemmer sign screening test (if a hand/arm or lower extremity is involved) and talk to your healthcare provider about your answers and ruling out lymphedema.

Case Scenario 1

Mr. J is a 47 y.o. 6' 4" 279lbs (BMI 34, overweight) Afro-American male. Past medical history includes history of hypertension, well controlled diabetes and chronic venous leg ulcers. He presents to your wound clinic with a recurrence of his Left medial ankle ulcer. You are the clinic wound care clinician and this is the first time you have seen this client. Mr. J. states he is not experiencing any pain, does not smoke, use alcohol or other unprescribed substances and complains that he is really tired of this ulcer reappearing every few months because 'I like to be really active, would like to exercise more but the drainage is smelly, I feel self-conscious with this bandage on it all the time, and I can't use the pool.  Mr. J. expresses his frustration over the fact, "I keep wearing those knee hi stockings every day but it just doesn't do much good."

After reviewing Mr. J.'s vital signs and observing all are well within recommended limits, you proceed to do a focused examination of his lower extremities. The medical record review shows a relatively normal ABI (ankle brachial index) of 0.8 done 9 months ago to assess his arterial perfusion. You remove his prescription compression stockings (30-40 mm hg, firm compression) which are not yet 6 months old but appear stretched as far as they will go; you note his dorsal and post tibial pulses are strong bilaterally (4+). There is a shallow 3.5cm diameter ulcer with irregular margins located over the medial ankle above the malleolus which is what you expected to see as it is consistent with a classic venous ulcer. But you also notice something else. The toes look like stacked inner tubes and when you attempt to pinch the skin at the base of the 2nd toe, you are unable to do so and note that this is a positive Stemmers sign suggestive of a lymphedema component that has previously not been identified in the patient's chart.

You continue your exam to gather more clinical clues. Even though Mr. J. has been wearing his stockings, the dorsum of the feet appears puffy, giving the contour of his foot a 'box-like' appearance- another indication of lymphedema. Although it is sometimes difficult to discern hemosiderin staining of the gaiter region in people with darker skin, you palpate this area and feel a woody non-compressible quality to the tissues that is also consistent with venous insufficiency. However, you continue palpating proximally and note that the calf areas are also firm or what is often described as 'fibrotic' and this clue is suggestive of lymphedema. The thigh and inguinal areas are spared.

You ask Mr. J. if he has ever heard the diagnosis of 'lymphedema' and he looks puzzled as says, 'No- never heard of that before!" You go on to explain that longstanding venous insufficiency can also be associated with secondary lymphedema which can make it more difficult to manage but once properly identified and treated, can put an end to the cycle of re-ulceration or at least minimize recurrence. You decide that this case could likely be managed in clinic without referral to a lymphedema specialist since there isn't one in the local area and many won't treat while there is still an active wound. Nevertheless, you consider this potential future referral to a lymphedema therapist as an option, should it become necessary. Mr. J looks both  relieved and hopeful saying he is eager to try the new plan of care. Over the next few weeks, multi-layer compression in the 40mmHg range is used on both legs although it is important to note, some insurance companies may not approve compression therapy on a non-ulcerated, contralateral extremity-so it's important to verify coverage limitations.  If this limitation is present, make sure that the diagnosis of lymphedema is mentioned in the justification and refer to any progress made by the Lymphedema Treatment Act submitted to congress in March 2015 (see http://lymphedematreatmentact.org/) to see if any states have adopted the stipulations mandating treatment for lymphedema including compression garments. Also consider referral to a lymphedema therapist as an alternate avenue to initiate earlier in the treatment plan.

You encourage Mr. J. to limit his salt intake, continue efforts with weight loss maintaining his protein intake, and continue his exercise program. You then proceed with evidence-based wound care for the open ulcer. Serial circumferential measurements of both lower extremities are taken at each visit along with measurements of the wound(s) and Mr. J. is delighted to see the measurements steadily decrease. Finally the ulcer is now closed and you start the next phase of combined lymphedema/venous insufficiency treatment: maintenance. Since Mr. J. initial therapy with 30-40mmHg standard compression stockings in the past, this time you have him measured for a custom knee high such as Elvarex (r), a special flat knit type of stocking that is heavier and is typically used for effective control of lymphedema.

You realize that if you discontinue compression therapy while the custom stockings are being made, that there is a good chance of the ulcer reappearing. Since there is no reimbursement for continued compression wrapping on intact legs, you place two layers of a tubular compression material (e.g., Tubigrip (r)) over each leg and instruct him to remove the second layer at night to avoid compromising the circulation and replace both layers on during the day. Mr. J. returns to clinic in about 3 weeks with his new stockings. His legs have increased in circumference as expected but thankfully the ulcer has not reopened and you start him on daily wear of his new stockings with a return visit in 2 weeks. Mr. J. says he is still noticing some difficulty with swelling even though control is better with the new stockings. It was decided to augment his lymphedema control with a sequential compression pump, since T.J. did not have access to lymphedema therapy in his local area and he said he couldn't afford to take that much time off from work anyway. Since Mr. J. was a Veteran, it was possible to obtain a basic 3 chamber pump through the Veterans Administration prosthetics department and he was instructed to remove his stockings and use the pump for 45 minutes - at first, once a day in the late morning, then replace the stockings and if he felt the swelling was becoming troublesome, he could use it again later in the afternoon or early evening. Because of the underlying venous disease, the pump was set at a higher level (40mmHg) than is typically used for uncomplicated lymphedema (around 30mmHg). Stockings were to be removed during the night, washed and hung to dry for wear the next day.

Epilogue: This regimen worked well for Mr. J. and he had no recurrence of ulceration for the next 2 years in which he was followed in clinic. If he had problems with swelling at night, the next step would be to consider a specially fitted knee high 'boot' with quilted channels to promote lymph drainage and help control nighttime swelling (such as Jovi-Pak (r)). Diagnosis and treatment of secondary lymphedema was effectively achieved in the clinic environment and resulted in helping Mr. J. resume his active lifestyle, avoid the cost and 'downtime' of recurrent ulcers and reduce costs to the healthcare system.

Case Scenario 2

Health care delivery in the United States is changing and the focus is becoming one of prevention and early detection of problems before they become more difficult and costly to treat. And so, in-home care assessments, once rarely done, are being evaluated as a means to improve patient outcomes and reduce hospitalizations. You’re one of a growing number of certified wound care professionals (in this case, a nurse practitioner) who see home-bound patients for a wound care consult. The referral is for you to see a 63 year old woman, Mrs. W., who lives with her husband and has not been to see her Primary Care Provider (PCP) in over a year and has a history of obesity and chronic swelling of both legs. The husband called the PCP’s office to ask for help with ‘constant drainage’ from her legs.

It’s a sunny fall afternoon and the air is brisk. You note there are people out walking their dogs, children playing and generally enjoying the nice weather before it starts to get cold. When you enter the single family home, it is quite dark, the drapes drawn and you are greeted by Mr. W, a slender man in his mid-60’s, appearing fatigued and worried. Before he takes you in to see his wife, he expresses his concern that he is at his wit’s end and does not know how he can continue to cope with his wife’s condition, explaining that she does not want to leave the house and the drainage ‘is everywhere, it does not ever stop.’

Already you have made a mental note that this situation will likely require further evaluation by a social worker to better assess the needs of the husband as well as the identified patient, Mrs. W. You enter the small living room and see a morbidly obese woman sitting on a love seat with her legs that have taken on the shape of small tree trunks, resting inside an inflatable ‘Kiddie’ pool. You can see the serous drainage slowing oozing from her legs and dripping into the pool which you recognize as lymphorrhea (leaking of lymph fluid onto the skin). You greet Mrs. W. who acknowledges your presence but does not offer more than a few words in response to your questions. She denies pain but endorses difficulty ambulating.

Mr. W. says he has given up on trying to bandage his wife’s legs: “gauze gets soaked as soon as I put it on and it’s expensive.” You complete a physical assessment, the blood pressure is elevated but otherwise she appears stable. You next examine the lower extremities and are relieved to not see any signs or symptoms of infection in the legs which can initially start off as a localized erythema and cellulitis, but can travel up in streaks along lymph channels otherwise known as ‘lymphangitis,’ which can signal an infection is becoming systemic. You note that both legs evidence the clinical signs and symptoms of lymphedema when usually it is a unilateral condition, acknowledging that in some instances such as with morbid obesity, there can be bilateral involvement.

In addition to the loss of anatomic landmarks and typical shape of an unaffected lower extremity, there are trophic changes to the skin of both legs sometimes described as in peau d’orange, with abnormal thickening or pachydermia and hyperkeratotic papillomas. The edema is too severe to be able to palpate dorsalis pedis or post-tibial pulses but you are able to detect biphasic waveforms by Doppler. You know that sometimes malignancies can be associated with lymphedema and chronic venous insufficiency so you look carefully for any atypical lesions, understanding that any area of concerned should be referred for possible biopsy and appropriate follow up before starting any MLD (manual lymph drainage) or compression therapy. There are folds of fibrotic tissue hanging over the malleolar areas so you look for evidence of pressure injury between the folds, possible folliculitis and fungal infections. You also try to check the inguinal areas for signs and symptoms of intertrigo (bacterial, fungal or viral rash that can occur between folds of skin), but find it is physically too difficult for the patient to reposition herself in order to permit visualization of these areas. The presence of a dermal infection would also be a contraindication to MLD and decongestive therapy until brought under control. These findings are consistent with stage III lymphedema with depressive symptoms.

As you conclude your assessment visit, you let the patient and her husband know that you will be discussing your findings with the PCP and they can discuss treatment options. In order to control the copious drainage and make it more possible for Mrs. W. to leave the house, you suggest use of high absorption dressings (such as Eclypse Super Absorbnet Secondary Dressings by Advancis Medical or even other super absorbent clean products for human use such as superabsorbent diapers, although some have also tried other super absorbent products such as SpillEx Super-absorbing Spill Remover Pad by Freudenberg) wrapped around the legs, taped only where the plastic overlaps, and secured with gauze roll bandage. The caution with these approaches is that trapping moisture against the lower extremity skin for extended periods of time can cause maceration and increase risk of fungal growth. These approaches are only temporary to help address patients decreased quality of life. Home health nursing will be recommended with a social services consult. The longer range plan would be to consider short term placement in a care facility where the patient could receive MLD & decongestive therapy or successive compression wrapping as well as address her mental health needs before transitioning her to maintenance garments upon discharge. You point out that even though these situations can seem impossible to improve, there are evidence based therapies that can bring even stage III lymphedema under control and foster a better quality of life.

References

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This course is applicable for the following professions:

Advanced Registered Nurse Practitioner (ARNP), Clinical Nurse Specialist (CNS), Licensed Practical Nurse (LPN), Licensed Vocational Nurses (LVN), Registered Nurse (RN)

Topics:

CPD: Practice Effectively, Wound care


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