CE Home > Occupational Therapy > OT11 An Occupational Understanding of Lymphedema

Joe was born with a facial birthmark that didn’t bother him until, in 1978, his bottom lip began to grow larger. This impeded his ability to have full lip closure and normal lower dentition. In 1989, Joe had his lip medically shaved in the hopes that he could fully close his mouth and to improve his appearance. However, since the surgery, his lip has grown three times larger. Now, at age 45, his right face, neck, lip, ear and scalp are affected with large nodules, papillomas and crevices. His right ear opening is closed, causing muffled hearing, and he has 25% lip closure, causing poor oral motor control and drooling. Joe works as a game warden, which requires him to investigate suspicious activities associated with wildlife. Even though he wears a uniform when making house calls and serving warrants, his appearance often undermines his position and even scares people.

Joe’s friends introduce him to Cindy, an occupational therapist who is certified in lymphedema. Cindy explains her work with clients not as a cure but as a way to enable an individual to affect his own cure. Joe was particularly intrigued by her discussion of the evidence supporting appropriate exercise as part of intervention with lymphedema.¹ Joe told Cindy about his experiences, opening up to her as she gently encouraged him to talk about his lymphatic symptoms. He felt comfortable with her, which surprised him, and he wondered how a person could create such a sense of warmth while delivering to-the-point medical information.² Joe and his wife Lucy went to his physician the next day and requested a referral for occupational therapy.

At his first appointment, Cindy said she felt that the birthmark has affected the superficial lymph plexus of Joe’s lymphatic system. Cindy developed an occupational profile and collaborated with Joe to develop goals, following the Occupational Therapy Practice Framework,³ that support health and participation in life through engagement in occupation. Her treatment recommendations targeted decreasing the size of Joe’s lymphatic features to increase social acceptability with work clients and new friends, ease donning and doffing of overhead shirts, and improve independence with personal grooming and hygiene. Further, she focused on health management, specifically helping Joe enact a plan to establish habits to follow a healthy diet and exercise routine, helping him to understand how weight instability can exacerbate the symptoms of lymphedema.⁴

Lymphedema

Lymphedema is a chronic condition that affects a person physically, mentally and spiritually. Medically, it is defined as an abnormal accumulation of protein and water within the subcutaneous and interstitial tissues. This condition is further delineated by two categories. Primary lymphedema is caused by congenital malformation or lack of lymphatic anatomy and can manifest at any time. Secondary lymphedema results secondary to trauma, including self-injury, surgery, removal of lymph nodes, immobility, fluctuating weight and fluid, changes in atmospheric and capillary pressure, and medication.⁵ When particles cannot move across a membrane from a high to low concentration, interstitial fluid can pool, and the capacity of the lymphatic drainage system to transport and remove the fluid through normal mechanisms is impaired. As lymphedema progresses, the body’s ability to efficiently remove waste products and direct nutrients to where they are most needed is compromised.^6-9 Temperature, concentration, molecule size, surface area and diffusion distance increase the body’s ability to transport lymph, and this diffusion becomes a core consideration in treatment.

Typically, our bodies use osmotic pressure to move water from the interstitial space toward the cells and blood capillaries. Osmosis is the diffusion of a fluid across a semipermeable membrane, a membrane that is permeable to the solvent but not the solute. For example, proteins are too large for reabsorption through blood capillaries, so the lymphatic system will return the interstitial fluid from the lower body through the inguinal duct, which joins with the fluid from the upper body and together dumps into the thoracic duct. Lymph is returned to the venous circulatory system through the left subclavian vein, returned to the circulatory system, and recycled to the tissues.⁶ When this biological system breaks down, a person suffers from lymphatic obstruction and fluid is retained in the tissue. Swelling occurs — thus the name, lymphedema. Functionally, this swelling presents with pain, heaviness, tingling and aching, limiting range of motion and the client’s engagement in all occupations. Dressing becomes a problem, and driving, work, and leisure pursuits are all impacted. Patients often withdraw from others because they’re self-conscious about their appearance and restricted by the pain and fatigue secondary to the primary condition.

A Review of the Lymphatic System

The lymphatic system develops in utero in the fifth gestational week. This is an open circuit system that includes lymph nodes, lymph fluid, leukocytes, blood plasma, lymph collectors and angions.⁶ The lymphatic system can be described as the trash collector and garbage disposal of the body and is the first line of defense for infection and toxin removal. Eventually and simplistically, foreign bodies and large cell particles are collected, powered by water, and then distributed through the body, kidneys and bladder, exiting the body through urination.

The circulatory system is a closed network of the heart, blood and blood plasma. The circulatory system supplies oxygen, nutrients, and white and red blood cells to the body. The lymphatic and circulatory systems make exchanges in two places: at the blood capillary network where filtration and reabsorption take place and at the venous angles of the heart.³ Capillary exchange involves both circulation and lymphatics and allows for diffusion, osmosis, filtration and reabsorption.^6-9

A complex lymphatic network exists throughout our body, and its components include lymph fluid, lymph loads and lymph vessels. Typically, lymph fluid (a transparent semi-fluid or milky substance) contains protein, water, cells, particles and fatty acids; it assists in cell nutrition, immune defense and blood coagulation, and it helps transport fats, minerals, hormones and waste products.⁶

There are six types of lymphatic vessels. Starting in the superficial layer of skin are the initial lymph vessels, or lymph capillaries, that combine to form a plexus. When you touch your skin, the lymph capillaries are stimulated. Lymph capillaries are large, permeable, and irregularly shaped. They lack valves, have open and closed junctions, and have anchoring filaments. The lymph plexus has collectors, valves and lymph angions that work similarly to veins to collect and direct lymph load into and out of lymph nodes.

When this system doesn’t function properly, there is a backup in the system, just like a hose with a kink in it that will swell and perhaps leak. Limbs can become elephantine, and movement is slow, heavy and awkward. Clients often feel like they are a burden on their families and can feel overwhelmed by the relentless self-care — donning compression sleeves or stockings, meticulously caring for their skin — that drains their limited energy reserves. Rest and sleep cycles can become interrupted. Clients say they feel as if their bodies have betrayed them, and they can even lose the hope that their bodies will ever function normally again. There can be a downward spiral: limited physical activity, which results in fatigue, which, in turn, limits activity.⁴

Lymphedema is often seen as a secondary condition post-breast cancer treatment.¹ Since lymphatic trunks are families of lymph nodes and collectors, innervated by the sympathetic nervous system, surgery can compromise the system. The nine lymphatic trunks overlap in some of their draining, so if injury occurs to one, another trunk can still drain portions of the region. In the mammary gland, for example, 75% drains into the same quadrant’s axillary nodes while the remaining 25% drains into the parasternal lymph nodes located on the opposite quadrant. However, if the lymph ducts are removed, as often happens during a mastectomy, the redundancy in the system is removed as well. The lymphatic trunks that are supposed to dump the lymph load into the venous angles of the heart can no longer function. The arm swells with lymph, and the debilitating cycle begins.

Functionally the lymphatic system is divided into territories, quadrants, watersheds and anastomoses.⁶ Lymphatic territories drain specific areas of the body and transport lymphatic loads to regional lymph nodes, creating four quadrants, not including the head and neck. Each upper and lower extremity is a quadrant. The head and neck region, located above the upper horizontal watershed, drains at tributaries that conclude in the venous angles. Boundaries of territories/quadrants are distinguished by watersheds.6 There are three watersheds: sagittal (anterior and posterior), horizontal (anterior and posterior), and upper horizontal (anterior and posterior). Passageways between the lymphatic territories/quadrants exist at specific places on the watersheds, and are called anastomoses. These boundaries become important during manual lymphatic drainage, a treatment that uses a gentle form of skin stretching or massaging the affected area, working to milk the lymph out of the interstitial area and into drainage vessels.

When the system is working normally, or when manual manipulation is applied, five anastomoses allow an affected lymphedematic territory to be drained by neighboring territories. The anastomoses are named by the direction and the bridge they create between territories.

• The anterior axillo-axillary anastomosis (AAA) occurs on the sagittal watershed above the nipple line; fluid can be moved from left to right or right to left.
• The posterior axillo-axillary anastomosis (PAA) occurs on the posterior sagittal watershed between the upper medial margins of the scapulae; fluid can be moved right to left or left to right.
• There are two inguinal-axillary anastomoses (IA) or axillary-inguinal anastomoses (AI) existing at the horizontal watershed, below the caudal limit of the ribs and lateral to the navel between each upper and lower territory/quadrant.
• The anterior inter-inguinal anastomosis (AII) bridges the anterior right and left lower territories/quadrants of the sagittal watershed.
• The posterior inter-inguinal anastomosis (PII) bridges the right and left lower quadrants on the sacral, posterior, lower portion of the sagittal watershed.

Watersheds and anastomoses are important in the treatment of lymphedema.^6-9

Lymphedema is associated with psychological sequelae, including frustration, distress, anxiety and depression, and it often results in diminished function, an erosion of social support, pain and major disability. Lymphedema can reduce movement in the affected body part and cause skin breakdown or weeping. Patients can experience emotional distress, the loss of valued occupations, poor quality of health and, ultimately, a lower quality of life.¹ As the affected limbs become engorged with fluid, movement is restricted, and patients often report intense dysfunction in all areas of occupation. Depending on the severity, clients can present with severe limitations in function.

Once lymphedema occurs,6 it is labeled according to stage and grade. There are four levels to staging: 0, 1, 2 and 3. Stage 0 is broken down into two types.

• In stage 0: latency, trauma affects the lymphatic system, but there is no observable edema. The transport capacity controls edema by working at a reduced rate.
• In stage 0: lymphagiopathy, the lymphatic system’s transport capacity has been affected congenitally but continues to control edema. In both types of stage 0, the individual is at risk to develop lymphedema once the transport capacity cannot manage the lymphatic load.^6-9
• In stage 1 (reversible stage), the skin is soft without much change. It may indent with pressure, and elevation usually reduces swelling. Stage 1 requires investigating the patient’s past medical history and understanding the insufficiency that causes the swelling.
• Stage 2 (spontaneously irreversible) is characterized by development of fibrotic tissue, making pitting difficult; a positive Stemmer sign; and the possibility of frequent infections. Complete decongestive therapy (CDT) is imperative.
• Stage 3 (lymphostatic elephantiasis) is noted by extreme tissue and girth changes and a positive Stemmer sign. The patient has skin alterations, infections of the nails and skin, and ulcerations. Multiple episodes of continuous CDT and patient compliance are necessary.

Lymphedema is also graded by comparing limb volumes. After taking limb measurements, a minimal grade is assigned with less than 20% difference in volume, less than 4 cm. A moderate grade has a 20% to 40% difference, or 4 cm to 6 cm. A maximum, or severe, grade has greater than 40% difference, equal to or greater than 6 cm.^5,7 Lymphedema presents in any body part and is usually unilateral and asymmetrical. Common patient complaints are impaired sensation; taut skin; impaired flexibility; difficulty with donning, doffing and wearing clothing; and tightness along sock edges, bra lines, or when wearing jewelry. The skin may present with growths, skin breakdown, or papillomas. All of these factors should be carefully reviewed and documented, along with a list of medications and a thorough medical history of surgeries, comorbidities, removal of lymph nodes, occurrences of cancer, and radiation and chemotherapy treatments.

Lymphatic insufficiencies occur when the transport capacity is defeated. Dynamic insufficiency develops into edema because the lymph load is greater than the transport capacity and the passive and active protective measures are burned out. With dynamic insufficiency, elevation, compression and exercises are required to remove the abnormally high levels of lymphatic load. Mechanical insufficiency develops into lymphedema because the lymphatic system is unable to manage a normal amount of lymphatic load or respond to an increase in the lymphatic load of water and protein.^6-9 Factors leading to this mechanical insufficiency are trauma, surgery, radiation, inflammation, lymphatic system alterations, and fibrosis, which cause a stagnant lymph load and increased diffusion distance. Complete decongestive therapy is warranted with mechanical insufficiencies. The third type of insufficiency is a combination of dynamic and mechanical. Combined insufficiency occurs when the transport capacity of the lymphatic system is reduced and the volume of the lymphatic loads is simultaneously elevated.⁶ Combined insufficiency may develop into severe tissue damage (necrosis) and chronic inflammation. Complete decongestive therapy is to be used with caution.⁹

Treatment Options

Joe’s treatment addressed areas of occupation and client factors. Treatment of lymphedema varies according to the person, but it is likely that all areas of occupation (ADLs, rest and sleep, education, work, play, leisure and social participation) will be impacted. Occupational therapy will look at all areas:^3,8,9

Client factors include a client’s values and beliefs, spirit, body function and body structure.³ Joe’s neck and head are the affected areas of his body. As they had enlarged to the point that getting dressed or even finding clothing that fit had become virtually impossible, completed decongestive therapy was performed to restore normalization to that body structure and increase function in that area of the body. The outcome goal of donning and doffing a shirt was a basic ADL function. Moreover, he had begun to gain weight, withdraw from his friends and family, and display symptoms of depression, all common with lymphedema. Cindy helped Joe realize that there were interrelated symptoms, helped him identify the area of leisure he valued, and allowed him to move from lymphedema to wellness, change his emotional state and engage in his own treatment.^1,8

Complete decongestive therapy is a non-invasive adjunct approach to treat lymphedema and related conditions and has been used since the 1970s in Europe.⁶ In the 1980s, the technique was introduced in the U.S. Compression is necessary to assist in reduction of lymphedema after manual lymph drainage is completed. Decongestive exercises are gentle active, range of motion exercises to assist in lymph fluid movement using existing muscle pumps.

Overall nutrition helps to decrease inflammation and promote systemic health.4 Health management and maintenance are defined by the Occupational Therapy Practice Framework as “developing, managing, and maintaining routines for health and wellness promotion, such as physical fitness, nutrition, decreasing health risk behaviors.”³ Joe had always loved the outdoors and being active and fit. However, his avoidance of activity and withdrawal from social opportunities had begun to wear away his level of fitness. Reversing that trend by eating healthy meals and beginning a daily walking regimen improved Joe’s spirit, raised his energy level and lessened his symptoms. He and his wife have always enjoyed cooking together, trying new recipes and inventing new ways to use the fresh vegetables from his garden. He has returned to this leisure activity and reports a deeper relationship with wife as they engage, together, in a mutually satisfying occupation. His recovery is an example of the circle of healing concept, which has been demonstrated to produce strong positive effects for individuals with lymphedema.1 When Cindy met with Joe for his last session, she gave him a plaque that displayed Mary Reilly’s immortal quote that “man, through the use of his hands as they are energized by mind and will, can influence the state of his own health,” to remind him of the importance of living his life to the fullest through engaging in occupation.

Using Complete Decongestive Therapy

Performance patterns in the Occupational Therapy Practice Framework refer to habits, routines, roles and rituals. Clients with lymphedema must follow a complex and sometimes challenging routine of self-care, monitoring their skin for breakdown or cuts, applying compression garments or bandages, and following good patterns of work simplification and energy conservation. These routines can become tiresome and fatiguing, so clients need to understand the benefits of following the routine as well as the consequences of poor compliance. Complete decongestive therapy promotes drainage and restores function.

The anatomical anastomoses, watersheds and territories help guide practitioners in successful complete decongestive therapy, specifically in the manual lymph drainage component. Genna’s medical history includes a right-side mastectomy, one of the most common causes of lymphedema. She talks about feeling like a burden and that she should be able to get the job done and not be weak. She has become disenchanted with the medical community.⁸ The right and left territories/quadrants are not available because of past trauma, so the manual lymph drainage protocol must be modified. If lymphedema is observed in both upper extremities, the short stretch compression bandaging would include both arms. As an occupational therapist, what areas might you address with your treatment plan? You may want to start with an occupational profile — to learn what areas of occupation are impacted for Genna.  You may assume that dressing, grooming and hygiene, and other ADLs are included. You may then want to consider client factors, especially values and beliefs about disability. Body structure and function may become your primary area of focus for Genna.^1,3,6

Conclusion

OTs are skilled in accommodating areas of occupation — feeding, dressing, functional mobility, personal hygiene and grooming — to enhance the lives of those with lymphedema; practitioners can become knowledgeable in the application of complete decongestive therapy to further enhance a client’s occupations.^3,5 Our profession is effective in establishing goals related to individuals with lymphedema through enhancing occupational performance and social participation.3,5 Joe continues to show improvements in his ability to don overhead shirts, eat food without drooling, and engage in meaningful occupations he thought were lost to him forever.  His spirit has improved, and he has developed new habits and routines that are supportive of his continued healthy outcomes. Through this progress, he is participating in social events more readily, and he says he is happier. Last week, he and Lucy celebrated their 15th wedding anniversary by having a photo taken of them together — the first one Joe has allowed since 1978.

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