Lymphoedema

Lymphoedema is a condition of localized fluid retention and tissue swelling caused by a compromised lymphatic system. Treatment includes medical compression, among others.

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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 and what other treatment options exist.

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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 liters per day).

Every day the lymphatic system takes up approximately 10 liters 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 is lymphoedema and what are its symptoms?

Lympheodema is a condition of localized 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.

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

How do I recognize lymphoedema? 

  • Swelling (called edema 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.

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. It is estimated that primary lymphoedema represents 5 to 10% of lymphedema cases (Cooper, 2017). 
  • 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. Secondary lymphedema is thought to represent 90 to 95% of lymphoedema cases (Cooper, 2017).

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 edema 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 manifest. 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. 

Lymphoedema stages 0, I, II, III leg

Lymphoedema stages 0, I, II, III arm

How can lymphoedema be treated?

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

Complex 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 exercises.

Manual lymphatic drainage uses a specific amount of pressure (less than 9 ounces per square inch or about 4 kPa) and rhythmic circular movements to stimulate lymph flow.

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 and 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 optimize the volume reduction that was 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 extremely-shapedlimbs.
  • 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 extremely-shaped 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.
  • Intermittent pneumatic compression (IPC) is a mechanical method that mimics MLD by massaging the fluid out of the limb. It should be only used in conjunction with MLD to favor truncal drainage. IPC is applied with stationary devices which hinder movement during treatment.

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 and supplemented with daily activity movement (walking, yoga, bicycling, climbing stairs) are useful.

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  • 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 blood draws 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 jewelry 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 proper hydration. 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.

Other treatment options for lymphoedema

Surgery – can help to improve lymphatic flow

  • Microsurgical procedures: Lymphatic vessels are connected to venules to enhance lymphatic drainage.
  • Vascularized lymph node transfer: Lymph nodes with surrounding tissue are transferred from a part of the body to a damaged site. With the help of microsurgery, the nodes are connected to blood vessels; lymph vessels connect themselves to the implant.
  • Bypass: Lymph vessels from damaged areas are connected to areas that are still functioning

Liposuction 

Excessive volume, mainly fat and protein, is removed with a liposuction-like procedure.

Surgical resection

Removal of excess skin and subcutaneous tissue of the lymphoedematous limb (in extreme cases).

Kinesiotapes

For stimulation of lymph flow in patients with breast-cancer related lymphoedema.

Nutrition

  • Helps to reduce weight
  • Helps to reduce inflammatory processes
  • Special diet plans available
  • Supplements like chitosan, grapeseed oil, and selenium are supposed to have a positive impact on the disease

Further reading