Lymphoedema is an abnormal, long-term swelling of part/s of the body caused by a collection of lymphatic fluid (including water and proteins) in the tissues below the skin and occurs when there is a dysfunction in the lymphatic system.

Lymphoedema is most commonly seen in one or both arms or legs, but may present in the face, body or genitals. It is a persistent swelling that may range from a mild increase in size of the limb/area to a grossly swollen limb with skin changes that affect the quality of the skin. Severe cases of lymphoedema can be debilitating and impair quality of life. Lymphoedema is sometimes, but not always painful.

Gold Standard of treatment is Complete Decongestive Therapy – CDT. This includes (but is not limited to) Manual Lymphatic Drainage Massage, multi-layer compression bandaging, skin care, exercise program, other modalities such as low-level laser, pneumatic compression devices, oscillation, elastic taping, etc. Once swelling is reduced then one MUST be fitted with a good quality compression garment to maintain the reduction of oedema achieved in therapy.

ANATOMY OF THE LYMPHATIC SYSTEM

The lymphatic drainage system is a network of lymphatic vessels and lymph nodes (‘glands’) which drains clear, colourless lymph fluid (similar to the fluid in a blister) from your tissues. These lymph vessels run alongside the blood circulatory system.

The lymphatic vessels transport fluid to lymph nodes. After being filtered through one or more groups of lymph nodes, the fluid finally drains into a large vein close to the heart. The blood pumped from your heart to your tissues via your arteries, contains nutrients, oxygen, proteins, water and many other products needed to maintain the health of the different tissues in your body.

Your body tissues use what it requires, and the excess, along with any waste products is drained away from your tissue by two different systems. The first in the venous system, which is part of your circulatory system, and the second is the lymphatic system. The circulatory system-the arteries and veins, continuously circulates blood through each part of the body while the lymphatic systems main function is to drain and transport waste products and excess fluid away from each part, along its own system of vessels, before eventually joining the blood (circulatory) system close to the heart at the thoracic duct.

There are different parts to the lymphatic system. The superficial part of the lymph system drains and transports lymphatic fluid from the skin and subcutaneous areas which is where most lymphoedema occurs.

The lymphatic system is a network of lymph nodes, lymph ducts, and lymph vessels that is responsible for drainage and transport of lymph fluid from body tissues back into the bloodstream. Through this path of drainage, the fluid is filtered and processed before being returned back to the bloodstream/circulatory system. The lymphatic system – lymph organs include lymph nodes (lymph glands) that play a vital role in immune response.

The Function of Lymphatic System

The primary function of the lymphatic system is that it is a transport system to drain lymphatic fluid, which is a clear, colourless fluid (containing water, proteins, white blood cells, waste and other waste materials), from your tissues. This fluid is then filtered through your lymph nodes. The filtered lymph fluid then drains back into the large veins and is pumped back to the heart, where it is eventually removed from the body as urine through the kidneys.

There are 600 to 800 lymph nodes in the human body and occur in groups that drain particular areas of the body. These groups of lymph nodes provide the lymphatic system with an extra form of filtration and a protective function as they are one of the lymphatic organs responsible for the production of anti-bodies (lymphocytes) which have a vital role in fighting infection.

How is the Lymphatic Fluid Drained?

The fluid is drained along the system by three main mechanisms which operate synergistically. Each has an important role:

  • The Respiratory Pump via Diaphragmatic Breathing
  • The Muscle Pump via Exercise
  • The Lymphatic system via peristalsis in the digestive system/intestines

STAGES OF LYMPHOEDEMA:

Lymphoedema can gradually progress through the following stages:

Stage 0 (Latent stage)

  • Person is at high risk due to a known dysfunction in the lymphatic system
  • No swelling
  • “Normal” tissue consistency

Stage I (reversible)

  • Soft
  • Putty-like
  • Pitting (when you press your finger into the swelling, an indent is left when you remove the pressure)
  • Mild Swelling is markedly or completely reduced by elevating the affected area

Stage 2 (spontaneously irreversible)

  • May be firm and rubbery (indicating varying degrees of fibrosis)
  • No pitting or mild pitting when pressure is applied
  • Swelling not reduced by elevation
  • May be accompanied by frequent infections

Stage 3 (lymphostatic elephantiasis):

  • Extreme increase in volume and size
  • Significant changes to the texture of the skin
  • Skin changes- papillomas, hyperkeratosis and skinfolds
  • No reduction in swelling with elevation

Types of Lymphoedema:

Lymphoedema is classified into different types based on the origin or cause of the lymphoedema. Primary or Secondary Lymphoedema.

Primary Lymphoedema

Conatal/ Congenital Lymphoedema

  • Presents in very young children at birth to 2 years
  • Accounts for 5-10% of all primary lymphoedemas
  • Normally affects the legs, but arms, face and genitalia may also be affected
  • Affects females more than males

Lymphoedema Praecox

  • Develops after puberty, often within the 2nd and 3rd decades of life, before 35 years of age
  • Accounts for 80% of all primary lymphoedemas
  • Affects mainly females

Lymphoedema Tarda

Starts after the age of 35 years
Accounts for 10% of all primary lymphoedemas
Affects mainly females
This is as a result of a developmental deficiency in the anatomy of the lymphatic system i.e. the lymphatic vessel or lymph nodes have not developed completely before birth. This may be due to a familial predisposition or a congenital cause. There are different types:

  • Aplasia (absence of lymphatic vessels)
  • Hypoplasia (Decreased number or unusually small lymphatics)
  • Hyperplasia (Increased in size and number, but with valves that malfunction)
  • Obstruction of lymphatic vessels

Subdivisions of Hereditary Lymphoedema

  • congenital hereditary lymphoedema
  • hereditary lymphoedema, type I
  • lymphoedema-distichiasis
  • lymphoedema praecox
  • lymphoedema tarda
  • Milroy disease
  • Nonne-Milroy disease
  • Turner Syndrome, Klinefelter Syndrome, Noonan Syndrome, yellow nail syndrome or Kinmonth Syndrome (fibrosis of the lymph nodes), Klippel-Trenaunay-Weber Syndrome

Primary lymphoedema mostly affects the lower limb (unilateral or bilateral) but can affect the arms and other areas. In the leg, the swelling usually starts in the foot and gradually moves up the limb. There may or may not have been a trigger event such as minor trauma, infections or immobility. Often the swelling is mild at first but progresses without treatment.

Hereditary lymphoedema is a genetic developmental disorder affecting the lymphatic system. It is characterized by swelling (edema) of certain parts of the body. The lymphatic system is a circulatory network of vessels, ducts, and nodes that filter and distribute certain protein-rich fluid (lymph) and blood cells throughout the body. In hereditary lymphedema, lymphatic fluid collects in the subcutaneous tissues under the epidermis due to obstruction, malformation, or underdevelopment (hypoplasia) of various lymphatic vessels. There are three forms of hereditary lymphedema: congenital hereditary lymphedema or Milroy disease; lymphoedema praecox or Meige disease; and lymphoedema tarda.

Symptoms include swelling (lymphoedema) and thickening and hardening of the skin in affected areas. In most cases, hereditary lymphoedema is inherited as an autosomal dominant trait.

Lipoedema is a symmetrical accumulation of abnormal collection of subcutaneous fat, most often in the legs. Lipoedema occurs almost exclusively in females 85% and 15 % male. Tenderness and bruising is common. Typically, the feet are not swollen and no thickening of the skin of the toes or dorsum of the feet (no Stemmer’s sign). Lipoedema is frequently misdiagnosed as lymphoedema.

Signs & Symptoms

The main symptom associated with hereditary lymphoedema is swelling (oedema) or puffiness in different parts of the body because of the accumulation of protein-rich fluid (lymph) in the soft layers of tissue under the epidermis (lymphoedema). Swelling frequently occurs in one or both legs, but may also be present in the trunk, face, genitalia and arms. When lymphoedema develops in the legs, swelling is usually most noticeable in the foot and ankle but may also be present in the calf and thigh. In some cases, swelling may cause tightness, discomfort and unusual tingling sensations or paraesthesia’s in the affected areas. The affected area heals poorly even after minor trauma (e.g., cut or insect bite). The skin of the affected area may become abnormally dry, thickened or scaly skin (hyperkeratosis) resulting in a “woody” hard texture.

Hereditary lymphoedema type IA (Milroy’s disease) is characterized by swelling (oedema) that is present at or shortly after birth (congenital). In rare cases, oedema may develop later in life. The legs are most often affected. The extent and location of oedema varies greatly from case to case even among individuals in the same family. In some cases, the genitals may also be affected. Additional complications sometimes associated with hereditary lymphoedema type I include up-slanted toenails, small warty growths on the affected areas called papillomatosis, abnormally large or prominent leg veins, and, in males, urethral abnormalities and the development of a fluid-filled sac along the spermatic cord of the scrotum (hydrocele).

Hereditary lymphoedema type II (Meige disease, lymphoedema praecox) develops around puberty or shortly thereafter in most individuals. This is the most common type of primary lymphoedema. In addition to lymphoedema of the legs, other areas of the body such as the arms, face and larynx may be affected. Some individuals may develop yellow nails.

Lymphoedema tarda is defined as primary lymphoedema occurring after the age of 35. The legs are most often affected, but the arms and other areas may be affected as well.

Hereditary lymphoedema may progress and, in some cases, may improve over time. Obesity makes management of lymphoedema more difficult. Affected individuals with lymphoedema are at risk for developing infections including bacterial infection of the skin and underlying tissue (cellulitis) or infection of the lymphatic vessels (lymphangitis). These infections are characterized by areas of warm, painful and reddened skin. Red skin “streaks” may also develop in the infected area. Increased edema is common. A general feeling of ill health (malaise), fever, chills, and/or headaches may also occur. If left untreated, cellulitis can lead to septicaemia, skin abscesses, areas of ulceration, and/or tissue damage (necrosis). Cellulitis is more common in males than females. Athlete’s foot (Tinea pedis) can cause cracks in the interdigital skin, bacterial invasion and cellulitis.

In rare cases of persistent lymphoedema, additional complications may develop including fluid (e.g., chyle) accumulation body cavities such as the thorax (chylothorax) and abdomen (chylous ascites). Chyle is a fat-laden cloudy fluid that is absorbed during digestion by the lymphatic vessels located around the intestine. Chyle normally flows through lymphatic vessels into the upper chest (thoracic duct) and is then deposited into veins, where it mixes with blood. In some people with hereditary lymphoedema, the lymphatic vessels may rupture or become blocked (obstructed), causing chyle to accumulate in the chest cavity (chylothorax). Addition of a mild diuretic has been reported to be a valuable adjunct to dietary control.

Affected individuals may also be at a greater risk than the general population for developing a malignancy at the affected site. These malignancies include angiosarcoma. Angiosarcomas are cancerous tumours that develop from blood or lymphatic vessels. They may occur in any area of the body. A specific type of angiosarcoma is known as lymphangiosarcoma, or Stewart-Treves syndrome. This cancerous tumour may rarely develop in longstanding cases of primary or secondary lymphedema. Angiosarcoma occurs in the lymphedematous extremity but can spread to the adjacent trunk and lungs.

Causes

Many researchers believe that hereditary lymphoedema may result from changes or mutations in one of the different disease genes (genetic heterogeneity). Most cases of hereditary lymphoedema type IA and type II are inherited as autosomal dominant traits. Genetic diseases are determined by the combination of genes for a particular trait that are on the chromosomes received from the father and the mother. Dominant genetic disorders occur when only a single copy of an abnormal gene is necessary for the appearance of the disease. The abnormal gene can be inherited from either parent or can be the result of a new mutation (gene change) in the affected individual. The risk of passing the abnormal gene from affected parent to offspring is 50 percent for each pregnancy regardless of the sex of the resulting child.

Affected Populations

Hereditary lymphoedema affects females more often than males. The estimated prevalence of these disorders is 1 in 6,000 individuals within the general population. Hereditary lymphoedema type II (Meige syndrome) is the most common form accounting for approximately 80 percent of cases. The prevalence of hereditary lymphoedema type I (Milroy disease) is unknown.

Hereditary lymphoedema may be associated with several genetic multi-system disorders including Noonan syndrome, Klippel-Trénaunay-Weber syndrome, lymphoedema-hypoparathyroid syndrome, and Turner syndrome.

Researchers have determined that hereditary lymphoedema type II (Meige syndrome) and three other disorders occur due to different mutations of the same gene (FOXC2). These disorders include yellow nail syndrome, distichiasis-lymphoedema syndrome, and lymphoedema-ptosis syndrome. The exact relationship between these disorders is unknown.

Yellow nail syndrome is a rare disorder characterized by yellow, thickened, and curved nails with almost complete stoppage of nail growth. Loss of the strip of hardened skin at the base and sides of a fingernail (cuticles) may also occur. Separation of the nails from the nail bed (onycholysis) may cause the nails to fall out. Yellow nail syndrome is usually associated with the presence of fluid in the lungs (plural effusion) and swelling of the arms and legs (lymphoedema). Other respiratory problems may occur such as chronic inflammation of the bronchi and bronchioles, chronic bronchitis, and/or ongoing inflammation of the membranes that line the sinus cavities (sinusitis). Lymphoedema usually occurs around puberty. Yellow nail syndrome occurs because of mutations to the FOXC2 gene and is inherited as an autosomal dominant trait.

Distichiasis-lymphoedema syndrome is a rare autosomal dominant multi-system disorder characterized by swelling due to fluid accumulation that usually occurs around puberty and the development of extra eyelashes along the posterior border of the lid margins (distichiasis). Distichiasis may range from a few extra lashes to a full set of extra eyelashes. Swelling most often affects the legs. Additional anomalies associated with this disorder include cleft palate, droopy eyelids (ptosis), abnormalities of the curved transparent outer layer of fibrous tissue covering the eyeball (cornea), cysts on the spinal cord, an abnormal sensitivity to light (photophobia), and cardiac (heart) defects. Distichiasis-lymphoedema syndrome is caused by mutations of the FOXC2 gene and is inherited as an autosomal dominant trait.

Lymphoedema-ptosis syndrome is an extremely rare genetic disorder characterized by swelling because of fluid accumulation and droopy eyelids (ptosis). Swelling most often affects the legs. Lymphoedema usually occurs at or shortly after puberty. Lymphoedema-ptosis syndrome occurs because of mutations of the FOXC2 gene and is inherited as an autosomal dominant trait.

Diagnosis

The diagnosis of hereditary lymphoedema may be confirmed by a thorough clinical evaluation and a variety of specialized imaging tests including lymphoscintigraphy, ultrasound, and magnetic resonance imaging (MRI). During lymphoscintigraphy, a radioactively labelled colloid substance is injected intradermally into either the hands or feet. The time required for the tracer to be transported from the point of injection to the regional lymph nodes is recorded. In congenital lymphoedema, the tracer may move sluggishly or not move from the site of injection. During an ultrasound, reflected sound waves create an image of the developing fetus. An ultrasound is used to rule out other conditions. A Doppler ultrasound can evaluate venous conditions such as varicose veins and venous blood clots. An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues. An MRI is used to detect findings characteristic of hereditary lymphoedema including swelling (oedema), a mass surrounded by a sac containing lymph fluid (lymphocele), and the formation of fibrous tissue (fibrosis).

Related Disorders

Symptoms of the following disorders can be similar to those of hereditary lymphoedema. Comparisons may be useful for a differential diagnosis.

Secondary Lymphoedema

Secondary lymphoedema is an acquired disorder that results from obstruction or damage to the lymphatic system. Affected individuals experience swelling due to the accumulation of lymph in the affected area. Secondary lymphoedema is an acquired condition resulting in damage to or obstruction of previously normal lymphatic vessels and channels. There are many causes and the most common causes are:

  • Surgery (e.g. the removal of lymph nodes due to cancer-patients are at lifelong risk)
  • Any surgery resulting in damage to lymph vessels and scar tissue formation (from Orthopaedic, Vascular, Plastic Surgery procedures)
  • Radiation of lymph nodes
  • Injury/Trauma
  • Infection (cellulitis)
  • Cancerous tumour infiltration
  • Chronic Venous Insufficiency
  • Chronic Wounds
  • Filariasis (parasitic invasion of lymph nodes and vessels following a mosquito bite carrying the filariasis worm. This is not common in South Africa, but a person may have contracted it while travelling in areas where the mosquito/parasite is found

The most common cause and highest incidence of secondary lymphoedema worldwide occurs in tropical and subtropical regions and is filarial infection in which lymphatic obstruction is caused by a parasitic disease known as lymphatic filariasis. Lymphatic filariasis is caused by three different species of worms known as Brugia malayi, Brugia timori and Wuchereria bancrofti. These worms cause damage and inflammation to the lymphatic system. The worms are introduced into the human body through the bite of infected mosquitoes. Secondary lymphoedema is treated by addressing the underlying condition. For example, in lymphatic filariasis, certain drugs are used to kill filarial larvae and worms.

Diagnosing Lymphoedema

As with all medical conditions, correct diagnosis makes correct treatment possible. It is very important to consult with a doctor if swelling develops suddenly. In some cases, the diagnosis and cause are very clear, and no special tests required. However, sometimes special testing is needed. The following are a few of the tests available.

Lymphoscintigraphy

A radioactive tracer is injected in the web spaces of the toes or fingers and the movement of this radioactive tracer within the lymphatic system is monitored. The flow and emptying of the glands is timed and compared to the unaffected side for comparison.

MRI

A technique mainly to assess the presence of tumours, but also used to assess vascular infiltration into an area. It is very expensive and rarely used for lymphatic mapping.

Venous Doppler:

There are many reasons that swelling can occur. One very serious and potentially life-threatening reason is due to a Deep Vein Thrombosis (DVT). The Doppler Ultrasound is one of the tests used to diagnose a DVT. It is also used to see if one of the causes of the swelling is a vascular problem (ie. the vein or circulation).

ICG Lymphography:

This is new technique, soon to be available in South Africa. A fluorescent tracer is injected in to the limb. A near infra-red camera is used to visualise the tracer. The lymphatic vessels and function can be mapped in real time, as well as the location of obstruction or dysfunction and its severity. It is a very detailed and accurate assessment.

TREATMENT

There are a number of possible treatments for lymphoedema, many of which can be used together. International best practice guidelines recommend complete decongestive therapy (CDT) to manage and treat lymphoedema. Lymphoedema is a chronic condition (i.e. it cannot be fully cured) and is progressive (i.e. it worsens) if not treated.

Only basic information on treatment is provided here – please consult your doctor and then a lymphoedema specialist (a specially trained professional nurse, occupational therapist or physiotherapist, etc. for additional information FIND A THERAPIST at www.lymphafrica.com

Complete Decongestive Therapy

A trained lymphoedema therapist will do a detailed Evaluation and assessment and together with the patient will plan the best effective and most realistic treatment programme. CDT helps the impaired or damaged lymphatic system to function better and reduces the collection of lymph fluid in the swollen area. This type of treatment does not ‘cure’ the problem and it needs to be maintained regularly. Treatment therefore involves an intensive phase, a maintenance phase as well as an important home management programme.

CDT includes gentle massaging known as manual lymph drainage (MLD), use of a multi-layered compression bandages on the swollen limb, compression garments, exercise (with your therapist and at home), and nail & skin care.

Manual Lymph Drainage (also referred to as MLD) is a very light, slow and rhythmic skin movement technique where the skin is stretched in a semi-circular movement. The pressure used on the skin is no more than the weight of touching a new born babies head. Gentle as it is, this technique effectively stimulates the re-absorption and flow of lymph fluid in the vessels and nodes of the lymphatic system. This light movement accelerates the re-absorption of fluid and wastes present in the bodies tissues. Swelling may be due to injury, surgery, radiation, etc. The lymph system filters the waste products in the fluid and returns it to the venous bloodstream, enabling the body to excrete the waste and excess fluid. The direction and order of the MLD technique is as important as the gentle strokes. First, the areas of the body where nodes are concentrated (neck, axilla, or groin) are stimulated in order to prepare the area to receive the built-up fluid. The therapist begins closest to the nodes, moving fluid toward them with slow and rhythmic strokes. The treatment continues with the therapist’s hands moving farther away from the cleared node area. The lymph moves through the lymph vessels by way of one-way valves called lymph angions. Angion means heart – so this is really the pump that pushes the lymph fluid. Each lymphangion has an internal stretch sensor. The walls of the lymphangion stretch when they fill up with lymph fluid, and then the stretch sensor tells the muscle to contract. This spiralling muscle contracts, squeezing the lymph into the next chamber. This swells the next lymphangion, which then contracts, pushing the fluid down the line. At the same time the lymphangion is pushing the lymph forward, it also is creating a vacuum behind it. It is partly because of this vacuum effect that the lymph gets pulled into the initial lymphatics. Once the lymphangions begin contracting, they cause a chain reaction, or a wave of contractions that start to push and pull the lymph fluid through the body. In this way, stimulating lymph flow in one area can increase lymph flow in another. Important factors that assist the movement of lymph fluid are skeletal muscle contractions, diaphragmatic breathing, and peristalsis, which includes good digestion and movement of bowels and not being constipated.

MLD is effective because this light technique is able to activate the stretch response, which causes the anchoring filaments to increase and stretch the diameter of the lymph vessels and therefore, increase the pulsation rate of the lymphangions increasing lymph flow through the vessels. By performing MLD correctly, and on a daily basis, one can stimulate the opening of the initial lymphatics and increase the volume of lymph flow by as much as 20 times the normal flow of filtration. If a therapist pushes too hard on the skin, they can cause the collapse of the initial lymphatics, diminishing the lymph flow by the excessive pressure can break the anchoring filaments that hold the initial lymphatics in place and this can damage the overlapping inlet-valves which are only one cell thick. This is the reason that deep tissue massage is contraindicated in cases of oedema/lymphoedema. When one has several MLD treatments, the consistent stimulation of lymph vessels opens up additional or new pathways and this is called anastomosis.

MLD affects the parasympathetic nervous system, which produces a calming, relaxing and healing effect on the body and mind. Many patients fall asleep during treatment, and report sleeping extremely well after treatment. MLD treatment may last from 45-90 minutes depending on the problem area. Some find it hard to believe that MLD, with its gentle strokes of massage can have any beneficial effect at all. All patients are pleasantly surprised at the quick response rate of their body to this gentle treatment and moving of the fluid trapped inside them. MLD works and is very effective!

PREPARING FOR MLD TREATMENT: On days of MLD treatment, avoid using lotions or other skin lubricants after bath or shower as movement and stretching of the skin is important to lymph flow. Wear comfortable, loose-fitting clothing that is easy to get on and off. Your therapist will ensure you are comfortable, as relaxation improves the effects of MLD. Dimming of lights is standard procedure, and some therapists add music to enhance the healing effects and encourage rest and peaceful quiet. For most MLD treatments you will need to remove everything but your underwear. Your therapist will provide a pillow, comfortable support for your knees, and soft sheets, blanket or towels to drape your body. Throughout the massage you will remain modestly draped, with only the areas being worked on exposed at any one time. When stagnant lymph fluid is successfully moved back into circulation in your body, the result is that more fluid is available to be processed by your kidneys, so you may experience an urgent need to urinate following the MLD session. You’ll want to be sure to use the bathroom before your treatment, and again before heading home.

COMPRESSION BANDAGING

This involves the use of multi-layered lymphoedema bandaging (MLLB). Lymphoedema bandaging or wrapping is a multi-layered system of various sizes of bandages. One starts with a cotton stockinette, followed by finger or toe bandages. Cotton padding is then applied using uniform or graded spacing and tension to create compression around the swollen limb. Foam is often used in the layering process to reduce fibrosis and increase comfort. Then short stretch, non-elastic pure cotton bandages ranging from 6cm, 8cm, 10cm to 12 cm width wide bandages are used. Self-bandaging or bandaging by a caregiver is very important if there is limited access to a skilled therapist. Good self-bandaging may also be part of the home management programme for the long-term management of the persons’ lymphoedema once the intense phase of treatment is complete. The purpose of bandaging following MLD is to reduce the return of lymphatic fluid to the affected area and to enhance lymphatic flow.

Exercise

Exercise with muscle contraction and deep diaphragmatic breathing increases lymphatic flow and is most effective in conjunction with short stretch bandaging or compression garments. Exercise programmes differing in frequency and intensity, are important for people with lymphoedema and for those at risk. A specific exercise program for enhancing lymphatic drainage is tailor made for each persons’ needs and considering any other medical history that the person may have. Exercise can be categorised as lymphoedema exercise (non-resistive, active movement of the swollen limb, particularly related to how the lymph drains from the area), stretching (reducing tightness/scarring), resistive exercise (progressive weight resistance – this type poses the greatest risk) and aerobic conditioning (improving cardiovascular fitness). The following are important tips to remember when doing the programme given by your therapist.

  • Perform exercises slowly, smoothly and gentle manner, it should not be painful
  • Wear bandages/compression garments while exercising this will enhance pumping action of the lymphatic vessels
  • Start with exercises of the trunk to help with lymph drainage centrally, followed by exercises of the swollen limb/s: first use the proximal muscles (i.e. the muscles closest to the body e.g. shoulder/upper arm; hip/thigh) then the distal muscles of the limb last (i.e. muscles furthest from the body e.g. forearm and fingers; calf, ankle and toes).
  • Do exercises in the order advised by your therapist
    Include deep diaphragmatic breathing – this causes a pressure change which acts like a vacuum inside the ribcage, helping to drain lymphatic vessels towards the trunk
  • Get into a routine, set an appointment time in the day that fits in with your lifestyle
  • A person’s exercise programme is tailored to the individual and will depend on their age, occupation, lifestyle, level of fitness and current health

It is important to know that exercise alone will not reduce the swelling effectively and that any programme should be carefully explained and tailored to your needs. Exercise is only one part of a complete treatment programme. If any exercise seems to cause you any problems or seems to result in further swelling, STOP, and seek professional advice.

Skin & Nail Care

Maintenance of skin integrity and careful management of skin problems is important to minimise the risk of infection. People with lymphoedema are at a higher risk of infection in the swollen limb than elsewhere in their body. Infection can significantly worsen lymphoedema. The principles of skin care for the affected area include:

  • Wash daily with a pH-neutral soap, natural soap or a soap substitute-fragrance free.
  • Dry your skin thoroughly, ensuring that skin folds (if present) or areas between your fingers or toes, are clean and dry.
  • Monitor your skin for cuts, abrasions or insect bites. Ensure that you carefully inspect areas where you experience numbness or loss of sensation as you may not feel the cut or bite. Treat any cuts or abrasions. If there are any signs of infection, seek medical advice.
  • Apply fragrance free moisturising cream. Emollients or moisturizers help keep the skin healthy by reducing water loss from the epidermis – the outer layer of skin. Emollients keep the skin moist and supple by providing a protective film. For people with extremely dry skin, such as those with lymphoedema, emollients are an essential part of their daily skin care.
  • Avoidance of scented products, particularly in hot climates. Vegetable-based products are preferable to those containing petrolatum or mineral oils.

Self-management

Self-management by the person suffering with lymphoedema is a vital part of the treatment and long-term management of lymphoedema. How well the lymphoedema is controlled and reduced is highly dependent on the person’s commitment to all the different aspects of treatment and home management, as well as their resources and support system. Self-management by the individual/caregiver involves education and training on massaging (Simple Lymph Drainage), bandaging, exercising and skin/nail care. As lymphoedema is a chronic condition (i.e. it is not cured), those affected have to master life-long management. Consistency is essential during self-management and returning to your therapist is every six months for follow-up or sooner if there is an acute problem.

Compression garments

Once adequate reduction of the swollen limb is achieved, compression garments are applied to maintain the reduction. Garments can be custom-made or ready to use (off the shelf), depending on the shape and size of the limb, or if there are any special considerations. People living with lymphoedema wear the garments during the day and alternate these with bandaging at night during the maintenance phase of treatment. Compression garments are essential for long-term improvement and allow the person to do all their normal activities while still controlling their lymphoedema. They can also be used to prevent the progression of lymphoedema. Sometimes a person at risk of developing lymphoedema (and who does not already have lymphoedema) will wear a sleeve, glove or stocking for certain activities only e.g. long-haul flights or intensive exercise.

Garments can be categorised according to fabric construction (i.e. circular or flat knit) and the pressures exerted (Classes I – IV). High quality Lymphoedema compression garments are currently not manufactured in South Africa. The compression classes for the different garments vary according to the country in which they are made. Assessment and measurement of garments require that the therapist and/or the orthopaedic supplier has a good understanding of the garment specifications and the patient needs. It is important to note that these are special compression garments manufactured for people with oedema, lymphoedema and/or lipoedema are not the same as garments that you may purchase form your local pharmacy such as Clicks or DisChem. If you have any questions about the correct compression garment for you, please ask your lymphoedema therapist for advice.

Medication

Few pharmacologic therapies have been found to be effective in the treatment of lymphoedema. However, chronic changes caused by the lymphoedema (For example in conditions such as elephantiasis nostra verrucosa (ENV), includes lymphatic filariasis, chromoblastomycosis, lipedema, lipodermatosclerosis, papillomatosis cutis carcinoides, popular mucinosis and pretibial myxedema) can be treated with oral and topical treatment. Recurrent cellulitis (skin infection) should be treated with appropriate antibiotics as prescribed by your doctor.

Always consult your doctor when using prescribed medication. It is important to understand what the purpose of that prescribed medication is as it may be used to treat other medical conditions that you may have. For example, ‘water tablets’ may be used to treat a cardiovascular condition rather than to reduce your swelling. The use of traditional or alternative medications must also be discussed with your doctor.

Surgery

Advances have been made in recent years in the surgical treatment of lymphoedema. Surgery is available in South Africa. Surgical procedures are an option for certain patients only and depend on many different criteria. Most require an attempt at conservative treatment first, and some still require some treatment after surgery. These are specialised surgical techniques performed by highly trained in this particular type of microsurgery.

Surgical treatment is reserved for patients who do not improve with conservative measures or for cases in which the extremity is so large that it impairs daily activities and prevents successful conservative management. It is rarely indicated as the primary treatment modality. ‘Removal of excess skin’ may be necessary after significant decongestion following CDT.

Procedures such as the Charles procedure to ‘debulk’ the limb results in the removal of normal tissue (such as muscle, fat and possibly intact lymphatic vessels) and CDT thereafter becomes challenging. Please consult with a lymphoedema therapist/specialist before embarking on any surgery.

Compression or Pneumatic Pumps

Pneumatic compression pumps are a long-term maintenance and home treatment option for patients with lymphedema and should not be used in isolation but rather as a conjunction to CDT. There are a variety of pumps available with varying design and complexity.

A lymphedema pump is an intermittent pneumatic compression device that consists of an inflatable garment that wraps around the arm or leg and an electrical pneumatic pump that fills the garment with compressed air. The garment is intermittently inflated and deflated with cycle times and pressures that vary between devices. This helps squeeze the lymph fluid through any lymph channels that are present. When the device deflates, blood and lymph fluid circulates more effectively through the area. After many repeated cycles, this device may reduce swelling from lymphoedema.

This device should only be used under the recommendation and supervision of trained/qualified staff and is not appropriate for every patient. It is also used in conjunction with the other aspects of CDT, especially compression.

Please visit the list of Find a Distributor on the following page. We stand behind each Distributor and re-seller of JUZO Compression Garments. We require ongoing product training and review with each individual/company listed. Lymph Africa is not responsible for quality of service or rates charged from each Distributor. Distributors, Therapists and fitters vary in experience, expertise and skill and you are encouraged to inquire about training, background, fees for service and to verify coverage with your medical aid prior to treatment or ordering products. As when making any medical decision, research your options, ask questions, then decide on the best course of treatment for you.

The “Find a Distributor List” only includes Lymph Africa re-sellers. For a full list of certified therapists please visit www.lymphafrica.com FIND A THERAPIST page. This list does not represent all Lymphoedema clinicians currently in practice in South Africa only those that have gone through the ILWTI Lymphoedema Certification course with Lymphatic Education Africa.