SMASAC Short Life Working Group on Lymphoedema - Lymphoedema Care in Scotland, Achieving Equity and Quality

Section 2: Diagnosis and Management

2.1 Recognition of at-risk populations

Early identification and initiation of treatment is vital in lymphoedema to prevent complications and give the best possible outcomes for the patient. Early intervention has been shown to minimize the severity of lymphoedema, reduce the frequency of complications and lessen the need for complex treatment (Norman et al, 2009; Lund et al, 2011). Yet a study in Scotland highlighted that patients with lymphoedema found that their symptoms had not always been taken seriously or dealt with sensitively by health care professionals and that many experienced long delays in diagnosis and accessing appropriate treatment (Sneddon et al, 2008). A later study highlighted a perceived need across all professional groups for education in relation to recognition of early lymphoedema, selection of appropriate treatment and awareness of available referral pathways (Davies et al, 2012).

It should be recognised that a wide range of clinicians come into contact regularly with and manage issues related to lymphoedema. Therefore, clinicians managing conditions that either cause or co-exist with lymphoedema also require generalist skills to reduce the risk of lymphoedema, prevent lymphoedema from getting worse, and treat complications such as infections (cellulitis) early and vigorously.

It is possible to identify certain groups of individuals in the population who are more likely to develop lymphoedema. In particular, those with a family history of lymphoedema and those who have been treated for cancers in which the treatment has compromised regional lymph nodes are known to be at risk. It is also known that the incidence of lymphoedema increases with age (Moffatt et al, 2003; Moffatt et al, 2012; Sneddon et al, 2008) and obesity (Soran et al, 2011). The elderly population has an increased risk of lymphoedema, largely as a consequence of co-morbidities and mobility limitations. Additionally, increased risk is associated with the following conditions:

• Cancer: particularly of breast, reproductive organs, bladder, head and neck, melanoma
• Inflammatory joint disease
• Extensive burns, scarring or trauma involving lymph nodes
• Venous problems and poor venous return that overload the lymphatic system, e.g. venous hypertension, chronic oedema
• Chronic skin problems, e.g. psoriasis, eczema
• Family history of lymphoedema or 'heavy legs', which may reflect undiagnosed lymphoedema
• Severe mobility problems
• Recurrent cellulitis (Keeley, 2008).

In those 'at risk' or those with lymphoedema, the following factors may further increase the risk of developing or exacerbating lymphoedema:

• In cancer patients: lymph node excision; radiation to lymph node field; delayed wound healing
• In those treated for breast cancer: history of axillary web syndrome (cording); seroma; exercise above shoulder height within seven days of breast surgery; weight gain following treatment
• Other co-morbidities, e.g. diabetes, multiple sclerosis

• Immobility
• Recurrent cellulitis
• Trauma to the skin
• Wound healing problems
• Insect bites
• Sunburn
• Fungal infections, especially between toes
• Poor hygiene.

Venesection is frequently listed as a risk factor, as is the taking of blood pressure, in the arm of a person treated for breast cancer, on the affected side. Although anecdotal evidence exists, there is currently no research evidence to support this. However, to err on the side of caution, patients and health professionals are encouraged to use the arm on the unaffected side where possible.

Given the trend towards an increasingly elderly and obese population, it is important that health and social care professionals are aware of lymphoedema, who is at risk and what preventative strategies can be employed. Lack of awareness in health care professionals may result in missed opportunities to promote self care and risk reduction to minimise the severity and impact of the condition.

2.2 Interventions for minimising risk

Targeted screening, monitoring and prevention programmes in cancer-related lymphoedemas

There is emerging evidence that targeted screening, monitoring and preventative strategies have the potential to reduce the risk of developing chronic lymphoedema (Stout Gergich et al, 2008). Small uncontrolled studies of prevention programmes involving patients with breast, gynaecological and urological cancers have supported these findings (Lund et al, 2011; Mew and Kirwan, 2010; Box et al, 2002; Hayes et al, 2008).

General health promotion advice

Maintaining a healthy diet and weight, being physically active, not smoking, and reducing alcohol intake will help to minimise the risk of lymphoedema.

Specific preventative strategies

Key strategies for prevention are maintaining the integrity of the skin, preventing injury or infection and enhancing lymphatic system functioning through physical activity and breathing exercises. Prevention and early, vigorous treatment of cellulitis, to which individuals with lymphatic insufficiency are vulnerable, is important, as described in the British Lymphology Society/Lymphoedema Support Network Consensus Guidelines (2010), available from: http://www.thebls.co.uk/patients/files/consensus_on_cellulitis_aug_10.pdf

Genetic counselling for inherited lymphoedema

A proportion of the small number of people with primary lymphoedema will have a genetic predisposition. There is scope for reducing future incidence through genetic counselling.

2.3 Diagnosis

The diagnosis of lymphoedema is based upon clinical history, physical examination and confirmation of the diagnosis by specific tests. Lymphoedema can often be diagnosed by its characteristic clinical presentation, yet, in some cases, ancillary tests might be necessary to establish the diagnosis, particularly in the early stages of the disease and in oedemas of mixed aetiology. Identification of other causes of oedema may not preclude a co-existing lymphoedema. People with a degree of lymphoedema often have a number of co-morbidities which may make its identification challenging. Appendix 3 provides examples of such cases, with details of how diagnosis was determined and suggested management options.

The typical clinical history of primary lymphoedema includes:

• Possible family history of lymphoedema or 'thick legs'
• Swelling of part of body from birth or in early years
• More common in females, often arising around menarche, pregnancy or menopause
• No identifiable cause of swelling, other conditions having been excluded
• Acute cellulitis - may be a consequence of previously undiagnosed lymphoedema or it may precipitate lymphoedema in someone with latent lymphoedema.

The typical clinical history of secondary lymphoedema includes:

• History of condition known to compromise lymphatic system
• May be triggered by:
  • Uncharacteristic physical activity
  • Immobility
  • Minor or major trauma to 'at risk' part of body
  • Recurrent infections
  • Acute or chronic inflammatory process.

Early signs and symptoms may include:

• Swelling, usually of a limb, but not always
• Swelling is soft and oedematous initially
• Feelings of tightness, discomfort, tension, heaviness, tightness of jewellery (in limb).

Later signs and symptoms may include:

• Swelling that does not resolve overnight or on elevation
• Dry, scaly skin, skin becoming thickened and horny over time (hyperkeratosis), with possible lymph blisters or papillomatosis
• Thickening of tissues, becoming firmer over time with evidence of fibrotic changes
• Deepened skin folds and distortion of limb shape, squaring of toes
• Stemmer's Sign: inability to pick up a fold of skin at the base of the second digit in an affected limb
• Weeping from skin, 'wet legs' (lymphorrhoea).

Investigations

In suspected primary lymphoedema, specialist investigations may be required to confirm diagnosis, differentiate it from lipoedema and determine which parts of the lymphatic system are improperly developed. Such investigations may include lymphscintography, magnetic resonance imaging, ultrasonography or CT.

In suspected secondary lymphoedema differential diagnosis may be aided by:

• Examination of the cardiovascular system to exclude heart failure. Other causes of leg swelling include low protein states caused by a variety of conditions including liver disease, nephrotic syndrome and severe malnutrition. Simple blood tests will measure liver and renal function and serum proteins.
• Drug therapies, for example some of the calcium channel blockers, Steroids, Taxanes may cause localised leg and especially ankle swelling due to local capillary dilatation; thus, a careful drug history must be obtained.
• Individuals who are significantly obese (BMI >35) may have swollen legs due to dependent oedema.
• Recurrent disease for example cancer and lymphadenopathy.

2.4 Treatment

The aims of treatment for lymphoedema are to:

• Maximise the efficiency of the functioning lymphatics to enable
  • Reduction of swelling and maintenance of reduction
  • Improved condition of skin and subcutaneous tissue and limb shape
• Prevent or reduce exacerbations and complications such as cellulitis
• Maximise the individual's ability to self-manage the condition once a treatment regime with which the patient is comfortable and competent is in place and the condition is stable
• Improve quality of life
• For secondary lymphoedema, identify causes of reversible lymphoedema (for example, heart failure, obesity and immobility) and actively manage the underlying condition.
• Management strategies for all patients will involve a lifelong daily regime of care, including:
  • Skin care to preserve integrity and prevent trauma or infection
  • Physical activity/movement/positioning to maximise lymphatic flow and venous return
  • Compression therapy, the nature of which is determined by severity and other patient related factors
  • Potentially, lymphatic drainage and other specialist interventions determined by severity and other patient-related factors such as manual lymphatic drainage
  • Supported self care
  • Decongestives being recommended for regular use.

Self management

Self management is the development of skills a person uses to manage their medical condition. These skills can enable people to come to terms with lymphoedema, stay well-informed, and become confident and capable of undertaking some, if not all, of their treatment. Self management is not a replacement for formal health care but allows people to take as much control as they feel able to, supported by health and social care professionals. It replaces the paternalistic approaches of the past and provides an element of control to the individual.

The ability to self manage differs from one person to another. Therefore, self management should be individual to each person's ability. It must be recognised that the ability to self manage can fluctuate due to changes in circumstance and/or deterioration of the condition and therefore reliance on professional support may change over time.

In October 2012, the Macmillan Lymphoedema Project for Scotland produced "Top Tips for the Self Management of Lymphoedema - A Guide" with input from people with lymphoedema. The resource, which is aimed at people living with lymphoedema, their carers, and care professionals, is available via the Macmillan Cancer Support http://www.macmillan.org.uk/Home.aspx and NHS Inform websites http://www.nhsinform.co.uk/. The guide covers the recognition of signs and early symptoms of lymphoedema, understanding feelings, skin care, avoiding infection, staying active, breathing, lymphatic drainage, limb positioning and movement, diet and lifestyle.

The main burden of care falls on primary care to support the patients to manage the condition and its complications, and to signpost patients to relevant materials. More information is available at Appendix 5.

Additional sources of information and guidance on self management for both patients and professionals can be found in Appendix 6.

Psychosocial support

Psychosocial support is an important element of the holistic treatment of lymphoedema: it has the potential to have considerable influence on outcome by enhancing concordance, encouraging self management and maximising quality of life. Intervention involves planning and implementing psychosocial care strategies that help patients and their family/carers to take a positive role in the management of their lymphoedema and to achieve as good a quality of life as possible.

If psychosocial problems are not resolved within three months, the patient should be referred for specialist psychology assessment. The algorithm below (Figure 1) outlines the process.

Figure 1. Algorithm for psychosocial support, assessment and referral. (Lymphoedema Framework. Best Practice for the Management of Lymphoedema. International consensus. London: MEP Ltd, 2006.)

Surgery

Surgical treatment of lymphoedema can be divided into three main categories:

• Surgical reduction
• Procedures that bypass lymphatic obstructions
• Liposuction.

Patients for surgery need to be selected carefully and counselled to ensure realistic expectations of likely outcome. Maintenance of any improvement gained requires lengthy post-surgical compression therapy.

Potential indications for surgery in lymphoedema include:

• Severe deformity or marked disability due to swelling
• Removal of redundant tissue after successful conservative therapy
• Proximal lymphatic obstruction with patent distal lymphatics
• Lymphocutaneous fistulae and megalymphatics
• Eyelid and external genital lymphoedema
• Lack of response to compression therapy
• Recurrent cellulitis/erysipelas
• Intractable pain
• Lymphangiosarcoma.

Palliative care

Palliative care is focused on providing patients with relief from symptoms such as pain, swelling, and psychological distress, rather than treatment of the underlying condition and its causes. The aim is to improve quality of life for both patients and family/carers.

The needs of patients with lymphoedema who are otherwise ill with advanced disease and who require palliative care are often complex. Patients who develop oedema at this stage in life may or may not have lymphatic failure but symptoms may develop quickly and cause considerable distress. Principles and elements of lymphoedema treatment may be very helpful in minimising distress and further complications. A lymphoedema practitioner or palliative care practitioner with knowledge and skills in relation to lymphoedema may make a useful contribution to the multidisciplinary team. The potential benefits of any treatment strategy must be balanced with the potential burden on the patient and be determined by patient-driven goals, e.g. maintaining mobility. It is essential for those involved to have an understanding of the pathophysiology and possible contributory factors to oedema and where possible to address any treatable causes. Regular assessment is also important as the situation may change rapidly.

Sensitive communication on the prognosis is essential in determining a flexible, agreed plan of care reflecting achievable goals. Lymphorrhoea is a common, distressing complication and should be treated as any other skin wound with appropriate dressings and light compression applied with bandaging and reapplied as necessary if there is leakage. Specialist compression garments may be helpful if the skin is intact. All compression requires modification and so should be under the guidance of a practitioner with adequate training in the assessment and fitting of these for lymphoedema.

Further information can be found in the International Lymphoedema Framework publication 'The Management of Lymphoedema in Advanced Cancer and Oedema at the End of Life' (2010).

Recommendations

2	Health Boards should designate Leads for lymphoedema services who should be tasked with developing local pathways for the referral and management of lymphoedema based on existing evidence and guidance, to include management and prevention of cellulitis in people with established lymphoedema, and provision of advice on self management.
3	A SIGN guideline should be developed for the diagnosis, assessment and management of primary and secondary lymphoedema. SIGN should consider including lymphoedema in the remit when updating relevant cancer guidelines.
4	Healthcare Improvement Scotland should consider developing QPIs for the management of lymphoedema. Potential indicators could include: referral to lymphoedema specialist services/clinics by board area, including type of lymphoedema, referral source, and age ranges to determine equity across the lifespan proportions of medical, nursing and AHP staff by board area who have had additional lymphoedema training numbers of diagnoses in primary and secondary care
5	Scottish Cancer Taskforce should also consider lymphoedema outcomes when proposing updates for relevant sets of cancer QPIs.