FAQ's about Lymphoedema
WHAT IS LYMPHOEDEMA?
Lymphoedema is the accumulation of excessive amounts of protein-rich fluid resulting in swelling of one or more regions of the body.
This is due to a mechanical failure of the lymphatic system and occurs when the demand for lymphatic drainage exceeds the capacity of the lymphatic circulation. The condition usually affects the limb(s) although it may also involve the trunk, breast, head and neck or genital area.
The lymphatic system is a network of vessels and nodes throughout the body that transports fluid (lymph) from the body tissues back to the bloodstream. The functions of the lymphatic system are to maintain the volume and protein concentration of the extracellular fluid in the body and to assist the immune system in destroying pathogens and removing waste products from the tissues.
WHAT CAUSES LYMPHOEDEMA?
Lymphoedema may arise because the lymphatic vessels or nodes have been damaged or were not formed correctly.
Secondary lymphoedema is the most common type developing following damage to the lymphatic system. The damage may occur as a result of some cancer treatments including the removal of lymph nodes, following radiotherapy to lymph node groups or with the progression of malignant disease. The onset of lymphoedema may be at any time. It may occur within months of the damage or it may appear years later.
Secondary lymphoedema may also arise without a cancer diagnosis when one or more of the following conditions occur:
Trauma and tissue damage
Immobility and dependency
Factious – self harm
Infection such as cellulitis
Primary Lymphoedema in comparison to Secondary Lymphoedema is the result of a congenital condition that affects how the lymph vessels where formed. This may result in hypoplasia of lymphatic vessels (a reduced number of lymphatic vessels), hyperplasia of lymphatic vessels (vessels that are too large to be functional) or aplasia (absence) of some part of the lymphatic system. This form may be presents at birth (congenital), develop at the onset of puberty (praecox), or not become apparent for many years into adulthood (tarda). It may be associated with other congenital abnormalities/syndromes.
Primary and secondary lymphoedema can occur together.
WHAT IS THE INCIDENCE OF LYMPHOEDEMA?
The National Breast and Ovarian Cancer Centre (NBOCC) review of research evidence on Secondary Lymphoedema states “conservative estimates suggest that 20% of breast, genitourinary, gynaecological, or melanoma survivors will experience secondary lymphoedema.
More specifically the incidence of secondary lymphoedema associated with vulval cancer is estimated at 36-47%, breast cancer 20%, cervical cancer 24% and melanoma 9-29%.
The incidence of lymphoedema following sentinel lymph node biopsy (SLNB) is reported to range from 4-8%. (4)
At birth, about one person in every 6000 will develop Primary Lymphoedema.
WHAT ARE THE RISK FACTORS FOR DEVELOPING LYMPHOEDEMA?
The consensus document suggests that patients at risk of lymphoedema will be encountered in a wide variety of health care settings, primary, secondary and tertiary. Key risk factors identified by NBOCC for secondary lymphoedema include, the extent of surgery, lymph node dissection and radiation treatment. Other factors involve trauma, infection, increased body mass index (BMI) and immobility. Any major damage to the lymphatic system causes a life long risk of lymphoedema
WHAT ARE THE EARLY WARNING SIGNS OF LYMPHOEDEMA?
Subjectively these may include transient swelling of a limb or other region of the body. Other symptoms may include aching, heaviness, stiffness, limitation of movement, tightness or temperature changes. Clothing, jewellery or shoes may feel tighter. Lymphoedema is not usually a painful condition but some people report pain and tension in an affected limb or body part.
Clients report that the swelling associated with lymphoedema is often aggravated by heat, at the end of the day, with overuse, with sustained positions and prolonged inactivity. They report that gentle exercise, elevation, massage and compression can ease their symptoms.
Many conditions may cause these symptoms to occur and any of the changes described will need to be assessed by a doctor in order for an accurate diagnosis to be achieved.
HOW IS LYMPHOEDEMA DIAGNOSED?
An accurate diagnosis is essential for appropriate therapy. This is determined from the clinical history and physical examination. Co-morbid and confounding conditions of morbid obesity, lipoedema, cardiac disease, renal disease, metabolic disorders, infection, and venous insufficiency will require thorough medical evaluation.
Lymphoedema usually has a gradual onset. However when lymphoedema has an acute onset appropriate tests to exclude, deep venous thrombosis (DVT), recurrence of cancer and infection may be necessary. Sometimes lymphoscintigraphy will be offered to confirm a clinical diagnosis of Primary Lymphoedema.
Whether primary or secondary, lymphoedema develops in stages, from mild to severe. Methods of staging are numerous and inconsistent. They ranged from three to as many as eight stages. In Australasia, the most commonly used stage scale is that adopted by The International Society of Lymphology (ISL) (3), which identifies the following stages:
Stage 0 A latent or subclinical state where swelling is not evident despite impaired lymph transport.
Stage I This represents early onset of the condition where there is an accumulation of tissue fluid with higher protein content, which subsides with limb elevation. The oedema may be pitting at this stage.
Stage II Limb elevation alone rarely reduces swelling and pitting is manifest. In later Stage II the limb may or may not pit as fat and fibrosis supervenes.
Stage III The tissue is hard (fibrotic) and pitting is absent. Skin changes such as thickening, hyperpigmentation, increased skin folds, fat deposits and warty overgrowth develop.
Stage III encompasses lymphostatic elephantiasis. At this stage, the swelling is spontaneously irreversible and usually the limb(s) is very large.
WHAT ARE THE AIMS OF TREATMENT FOR LYMPHOEDEMA?
The aims of lymphoedema treatment are to prevent progression of the condition, to reduce odema and then maintain the improvement. This includes the alleviation of associated symptoms, prevention of infection and improvement of function and quality of life. The NBOCC literature review states “research suggests that without treatment secondary lymphoedema may worsen”
Lymphoedema cannot be cured but it can be improved and managed with appropriate intervention. The stage, location and severity of the lymphoedema together with the individual circumstances of the client will influence the most appropriate intervention. Early intervention is recommended.
WHAT TREATMENT IS AVAILABLE FOR LYMPHOEDEMA?
Best practice management has a holistic multidisciplinary approach and is provided by qualified lymphoedema therapists. The treatment method is conservative and in Australasia is termed Complex Lymphoedema Therapy (CLT). Older alternative terminologies are Complex Physical Therapy (CPT) or Complex Decongestive therapy (CDT).
CLT consists of the following components
Manual Lymphatic Drainage (MLD)
Treatment is individualized for each client and not all components of treatment may be necessary in all cases. Those with mild lymphoedema may only require education, exercise and skin care whereas for more severe cases treatment may be longer and require two phases. Phase I is an intensive treatment provided by a trained lymphoedema therapist followed by Phase II, which is self management that the client continues at home. With improved health education people are reporting early limb changes promptly and this is contributing to a reduction in the need for intensive treatments
Phase I – Intensive treatment consists of daily
Skin care to optimise the condition of the skin, reverse skin changes and treat infections.
Manual Lymphatic Drainage (MLD) to enhance lymph flow. This precedes bandaging and directs lymph fluid to functioning lymphatic territories and helps to establish collateral drainage pathways.
Application of multi-layer compression bandaging.
Exercises to increase lymphatic and venous flow.
A compression garment is prescribed, following a successful reduction. This is essential to preserve the reduction achieved by treatment and to help prevent progression of the lymphoedema.
Education is incorporated to ensure that the client understands their condition and all parts of the intensive treatment as well as the importance of phase II.
Phase II - Home maintenance programme
Clients are educated by their therapist in the need for self-management to maintain the results achieved in Phase I. Without the active involvement of the client, the successful outcome of treatment may not be sustained.
The routine adopted may vary from person to person but will usually include daily skin care and prevention of infection, regular self massage, exercises and the wearing of the prescribed compression garment(s). Clients will be encouraged to practice self monitoring and adopt a healthy lifestyle that includes attention to diet, fitness and weight management.
At times this treatment may be offered in a modified form to accommodate particular individual needs.
WHY IS COMPRESSION IMPORTANT?
The use of compression garments is one of the cornerstones of lymphoedema treatment. They enable medically appropriate pressure to be applied to the swollen region. This means that the pooling of fluid can be reduced, the limb size and shape preserved and the lymphatic circulation supported and improved. Compression garments provide graduated compression and are available in a variety of styles, sizes, colours and grades of compression (class 1-IV). Severe swelling usually requires stronger support than mild swelling.
Lymphoedema therapists are skilled at garment prescription and can advise on garment style and whether a pre-sized (Ready to Wear) garment or a custom made garment is required. Garment fabric may be constructed as a circular knit or a flat knit design. Most commonly garments are worn during the day and removed at night. Compression garments need to be replaced regularly in order to provide optimal support. Some people with mild lymphoedema may not require compression garments.
*All information on this page is from the ALA Website : https://www.lymphoedema.org.au/about-lymphoedema/lymphoedema-management/