Lymphoedema

Do you have a persistent, asymmetric swelling in one or more of your limbs? Is your affected limb painless, but you feel tightness in it? Are other family members also experiencing swelling of the limbs or did you undergo a cancer treatment or surgery where lymph nodes had to be extracted? If so, your lymphatic system may be compromised and you may have developed lymphoedema. Read here why compression is essential in treatment of lymphoedema.

What is lymphoedema and what are its symptoms?

Lymphoedema is a condition of localised fluid retention and tissue swelling caused by a compromised lymphatic system. This results in swelling in one or more limbs. However, lymphoedema can also occur in other body parts, e.g. neck, genital region, inguinal region, face, etc.

Approximately 1/3 to 1/4 of lymphoedema cases have a primary (e.g. genetic) cause that led to a compromised lymph system. Women are 6 to 10 times more likely to develop primary lymphoedema than men.

Secondary lymphoedema occurs if a secondary reason is responsible for the damage that affected the lymphatic system (e.g. cancer, cancer treatment, surgery, infections, etc.)

Here’s what you should know about lymphoedema, its treatment, and why compression therapy is essential.

How do I recognise lymphoedema?

Swelling (called oedema or congestion) in one or more limbs (often asymmetric)
If lower extremity is involved, the swelling is present on the back of the foot
Painless
Feeling of tightness
Restriction in range of motion, depending on lymphoedema stage
Thick or hardened skin, depending on lymphoedema stage
Recurring skin infections
Stemmer’s sign positive, depending on lymphoedema stage: This means that a fold of skin at the base of the second toe, the base of the middle finger, or another body part with swelling cannot be pinched and lifted.

The lymphatic system

The lymphatic system is a large network of lymphatic vessels and lymph nodes that plays an important role in transporting lymph fluid, immune function, fluid homeostasis, blood cleaning, and blood filtering.

The lymphatic system is our “waste disposal and recycling” unit. It transports waste products and toxins out of the body. Body waste products include proteins, metabolic breakdown products, inflammatory products, and fat from the abdominal cavity.

The lymphatic vessels, which are spread throughout the body like a net, carry a clear fluid called lymph towards the thoracic duct, which drains into the blood circulation at the venous-lymphatic junctions in the neck.

How the lymphatic system works

Lymph is formed from the fluid that filters out of the blood circulation into the interstitium and taken up by the blind-ended lymph-sinuses. The lymph consists of interstitial fluid, proteins (smaller than albumin), fibrinogen, and other coagulation factors, small molecules and ions of the serum and interstitium, leucocytes, immunoglobulins, fat in form of chylomicrons, cellular debris, waste products, and bacteria.

The lymphatic system is responsible for the majority of fluid uptake from the interstitial spaces. This collection of fluid is carried out by the initial lymph-sinuses, that are blind-ended epithelial lined vessels with fenestrated openings that allow fluids and particles as large as cells or proteins smaller than albumin to enter.

The fluids are then sucked and pressed into lymph pre-collectors. The lymph collectors and continually larger lymph vessels have a lymph valve system and lymphangiones (e.g. enlarged units with smooth muscle cells between two lymph vessels).

Through active contraction of the lymphangiones and restricted back flow of lymph due to the lymph vales, the lymph is transported in proximal direction through the lymph vessels and through lymph nodes.

Hundreds of lymph nodes are located within the human body (e.g. around the neck, intestinal tract or in the axilla or groin area) and are responsible for the removement of debris, regulation of the protein content of the lymph, the immune response, recirculation of lymphocytes, and re-absorption of water (approximately 5 to 8 litres per day).

Every day the lymphatic system takes up approximately 10 litres of interstitial fluid.

Intact lymphatic drainage

If the lymphatic drainage is intact, the initial lymph vessels, vessels with blind ends and windowed openings, transport the lymph fluid from the interstitial spaces.

Interrupted lymphatic drainage

Lymphedema can develop if the lymphatic drainage is interrupted, impaired, or if secretion of fluid into the interstitial tissue exceeds the uptake capacity of the lymph system.

What causes lymphoedema?

Lymphoedema is classified in two subtypes: primary lymphoedema and secondary (or acquired) lymphoedema.

Hereditary: primary lymphoedema is thought to be the result of a congenital abnormality of the lymph conducting system.
Acquired: secondary lymphoedema results from damage to the lymphatic system (lymphatic vessels and/or lymph nodes) or from functional deficiency. Infections from insect bites, serious wounds, or burns can cause lymphoedema when they damage or destroy the lymphatic system. Severe obesity, any type of surgery, serious injury, or radiation for cancer treatment (or the tumor itself) can cause the onset of the disease.

The following factors can be causes of or lead to a progression or aggravation of existing lymphoedema:

Surgery (lymph node removal, e.g. cancer treatment)
Trauma that might induce damage to the lymphatic system
Radiation and chemotherapy as cancer treatment
Serious infections of the skin or lymphatic channels (lymphangitis, cellulitis, erysipelas, microscopic parasite filarial larvae)
Obesity
Chronic venous insufficiency

Lymphoedema has three stages

Depending on disease progression, lymphoedema can be divided into three stages.

In stage 0 (or Ia), swelling is not yet evident despite impaired lymph transport, subtle alterations in tissue fluid/composition, and changes in subjective symptoms. It may be months or years before overt oedema occurs.
In stage I, the accumulation of fluid subsides when you elevate the affected limb. When pressed by the fingertips, the affected area indents and reverses with elevation (“pitting”).
Stage II signifies that limb elevation alone rarely reduces the tissue swelling. Pitting is present. Later in Stage II, the limb may not pit as excess subcutaneous fat and fibrosis develop.
Stage III encompasses lymphostatic elephantiasis where pitting can be absent and trophic skin changes, such as acanthosis, alterations in skin character and thickness, further deposition of fat and fibrosis, and overgrowths have developed.

How can lymphedema be treated?

There are different possibilities to treat lymphedema. The “gold standard” and key treatment is the complex physical decongestive therapy (CPD).

Complex physical decongestive therapy (CPD) is based on two main pillars:

manual lymphatic drainage (MLD)
medical compression

These two pillars are complemented by skin care, dietary changes, and exercise.

The treatment goal in the first phase of CPD is to reduce the volume as much as possible (reduction phase). Typically, medical compression in this phase consists of multi-layered bandage wrapping applied by a therapist or other medical professional that needs to to be replaced on a daily basis. The first phase of CPD lasts between 2 to 6 weeks, depending on the extent and stage of the lymphoedema. Skin care is also very important.

In the second phase of CPD – called the maintenance phase – the goal is to conserve and optimise the volume reduction achieved in the first phase. Medical compression with flat-knit compression wear, skin care, exercise, and repeated MLD as needed, are the main elements of treatment.

Compression is key

There are several products that are used for lymphoedema compression therapy.

Inelastic multilayer bandages are typically used in the first phase of CPD, with the main goal to reduce limb volume as much as possible. They provide very stiff compression and treat even misshapen limbs.
Flat-knit compression hosiery is mainly used for the long-term management of lymphoedema in the maintenance phase. This stiffer compression option is best used in stabilized limb volume conditions. Flat-knit compression hosiery also treats misshapen limbs.
Wraps with hook-and-loop shutter or bra hooks can be used in the first and second phases of CPD therapy. Wraps are easy to don and doff. They are easily adjustable to vary the level of compression to adapt to limb volume changes.
Circular-knit compression hosiery is only used in the very early stages of lymphoedema where the volume of the extremity has not yet reached extreme sizes.

Why is skincare important with lymphoedema?

If the lymph flow is chronically disturbed, the protein-rich liquid accumulates in the soft tissue and the swollen body parts become inflamed. Also, the function of the immune system is reduced since the lymph system is impaired. Even a small injury to your arm or leg can be an entry point for infection (such as cellulitis or erysipelas) or severe inflammation. Thus, it is important you maintain the integrity of your skin and carefully manage skin problems. This way, you minimize the risk of infections.

Skin care principles include:

Washing daily, using pH neutral soap, natural soap, or a soap substitute
If skin folds are present, ensuring they stay clean and dry
Monitor the affected and unaffected skin for cuts, abrasions, and insect bites
Applying emollients
Using only low pH lotions
Avoiding scented products

Exercise helps with lymphoedema

Basic motions of the extremities (muscle pumping exercises), especially combined with external limb compression, performed on a daily basis (walking, yoga, bicycling, climbing stairs) are useful.

Health tips for those with lymphoedema

Wear compression garments as prescribed or recommended.
Protect your affected arm or leg. Avoid injury to your affected limb; use high factor sunscreen and insect repellent as cuts, scrapes, and burns can invite infection.
If possible, avoid medical procedures, such as giving blood and vaccinations, on your affected limb.
Exercise, stretching, and a balanced diet are encouraged. But avoid strenuous activity until you've recovered from potential surgery or radiation.
Avoid heat and extreme cold on your affected arm or leg. Don't apply ice or heat, such as with a cooling or heating pads.
Prop up your arm or leg. Whenever possible, elevate your affected limb above the level of your heart. This allows gravity to assist your body in draining lymph.
Avoid anything that could constrict your arm or leg, such as tight-fitting clothing or jewellery and, in the case of your arm, blood pressure cuffs. Ask that your blood pressure be measured in your other arm. While your compression wear is designed to assist your body in draining excess fluid, too-tight clothes, which bind in the wrong places, may actually impede drainage.
Keep your arm or leg clean and your skin moisturized. Make skin and nail care high priorities. Inspect the skin on your arm or leg daily, watching for changes or breaks in your skin that could lead to infection. Always wear shoes to protect your feet from harm.
Follow a balanced diet and maintain optimal body weight. Controlling your weight can help to alleviate your symptoms.
Keep hydrated. Drinking plenty of water helps you stay hydrated and facilitates proper kidney function which is required to remove excess fluids, proteins, and other substances.
Avoid excessive dietary salt intake to help prevent fluid retention.

Summary

Persistent, asymmetric, and painless swelling in one or several of your limbs with a feeling of tightness could be a sign of a compromised lymphatic system. If other family members are also affected by swelling of the limbs or if you had a past cancer treatment or surgery where lymph nodes had to be extracted, it is recommended that you contact your doctor to clarify if you suffer from lymphoedema.

Basic medical knowledge on lymphoedema

Lymphoedema is a condition of localised fluid retention and tissue swelling caused by a compromised lymphatic system.
Protein-rich fluid can accumulate in the soft tissue, leading to swelling in one or more limbs or other body parts.
A compromised lymph system can be congenital or acquired.
With lymphoedema, we distinguish four stages (0–3), the first of which is usually symptomless.
The gold standard for lymphoedema treatment involves a multicomponent approach, including physical and psychosocial interventions and is called complex physical decongestive therapy.
Depending on the severity and stage of the disease, other treatment options, like surgery or excisional procedures, might be applied.