Possible presenting signs and symptoms:

• Early stage:
  • Mild and/or intermittent swelling
  • Swelling affects one or more: arm, part of arm, hand, breast, chest wall, back and/or shoulder
• Later stage:
  • Persistent and/or worsening swelling
  • Shape changes
  • Tendency to cellulitis
  • Skin and tissue changes may include:
    • fibrosis (firm, sometimes non-pitting)
    • fragile skin that breaks easily
    • deposition of fat tissue.

Additional information
Stages of lymphoedema defined by the International Society of Lymphology (2013):

Possible causes of upper limb lymphoedema

Cancer-related:

• Primary presentation of a cancer (e.g. breast, sarcoma)
• Recurrence of previously treated cancer
• Side effect of cancer surgery and/or irradiation involving lymph nodes and/or local lymphatics (e.g. breast cancer, malignant melanoma), particularly if there has been a wound infection, or repeated surgical incisions
• Advanced, obstructive, metastatic cancer

Non-cancer-related:

• Primary lymphoedema: may be present at birth or develop at any age, with possible family history and/or genetic element (e.g. Milroy’s disease)
• Non-cancer surgery or traumatic injury such burn, orthopaedic problem, or renal shunt

• Cellulitis: may be a cause or symptom of lymphoedema
• Previous deep vein thrombosis (post-thrombotic syndrome)
• Dependent arm/hand (Motor Neurone Disease, Multiple Sclerosis).

Breast oedema: additional information
Patients who have undergone surgery and/or radiotherapy to the breast are also at risk of breast oedema. Risk is increased in women with larger pendulous breasts. It may or may not occur with limb swelling.

Possible worsening or co-morbid factors for upper limb swelling

• Wound infection
• Body Mass Index increase
• Recurrent/metastatic cancer
• Recurrent cellulitis or inflammatory skin conditions such as psoriasis
• Deep vein thrombosis/venous flow abnormalities
• Brachial plexus neuropathy/pain
• Organ failure (liver/renal/cardiac)
• Medications such as calcium-channel blocking agents, corticosteroids
• Protein-losing disease/hypoalbuminaemia due to any cause
• Poor mobility or function
• Person unable to tolerate/apply compression garments

Investigations to be considered in Primary Care:

• Baseline bloods:
  • Full blood count
  • Urea and electrolytes
  • Liver function tests (LFTs)
  • C-Reactive protein and/or plasma viscosity: to exclude inflammatory conditions that may exacerbate swelling
  • Thyroid function tests (TFTs): to identify thyroid dysfunction that may influence weight and compromise lymphoedema treatment outcome
  • Blood glucose: to screen for diabetes (patients with lymphoedema already have an increased risk of cellulitis)
  • Serum protein: to exclude hypoalbumaemia affecting tissue fluid homeostasis
• Physical examination: to identify signs and symptoms of cardiac failure, chronic liver disease, renal disease and chronic inflammation or infection
• ECG, chest X-ray, abdominal or renal ultrasound scan as guided by above findings
• Urine dip for protein and blood: to exclude protein-losing kidney disease.
• Mycology nail clippings or skin scrapings: to identify chronic infections of skin/nails

Exclude: RED FLAG

• Deep vein thrombosis: refer to DVT algorithm
• Recurrent cancer: refer to cancer services
• Musculo-skeletal injury.

Consider referral for secondary care-based investigations or treatment:

• Orthopaedic, rheumatology or physiotherapy referral: to manage musculo-skeletal problems that affect mobility and exacerbate swelling
• Dermatology referral: to manage skin or nail conditions that may be exacerbating chronic skin/tissue problems
• Vascular referral: to exclude treatable vascular disease.

Tertiary referral for specialist lymphatic investigations and support:

A child or young person with a new or suspected diagnosis of primary lymphoedema may require lymphoscintigraphy and genetic counselling in a specialist centre:

NHS Glasgow Lymphoedema Service
70 Commercial Road
Glasgow G5 0QZ
Tel: 0141 330 6318

Initial self-care support prior to referral to lymphoedema practitioner

• Assess extent of swelling, and impact on daily life
• Commence emollients (refer to Highland Formulary):

• use a bland emollient such as Diprobase® cream (500mg pump dispenser) or Epaderm® ointment
• consider Oilatum® as bath additive or Dermol® 200 shower emollient
• if risk of minor skin infections, consider Emulsiderm® as bath additive

• Manage cellulitis/recurrent cellulitis: refer to cellulitis algorithm
• Avoid routine diuretic use:

• Diuretics do not improve lymph drainage and are not indicated for managing lymphoedema. They may reduce swelling and soften the tissues in the short term, but lead to tissue fibrosis. However, spironolactone may be indicated for someone with advanced cancer and intractable swelling.

Advice may be obtained from: nhsh.highland-lymphoedema@nhs.scot And the Lymphoedema Advice Line: 07870 483860

People with moderate/severe/uncontrolled or complicated lymphoedema may require treatment with a Lymphoedema Practitioner with specialist skills

This may include individuals with:

• Oedema that affects hand, fingers, back, and/or chest wall
• Breast oedema with/without limb swelling
• Problematic skin/tissue changes - fibrosis, scars, history of cellulitis
• A poorly shaped limb
• Complications such as poor mobility and function, advanced cancer, fungating tumour, venous outflow obstruction, neuropathic changes.

Management may include:

• A comprehensive lymphoedema assessment and treatment plan
• Compression bandaging using a short-stretch bandaging system, with or without manual lymph drainage (MLD) massage
• Manual Lymphatic Drainage (MLD) massage: a gentle skin massage that enhances the work of the lymphatic system, redirecting fluid towards healthy lymphatics, without increasing capillary filtration. It is useful for individuals with breast or trunk swelling. Patients can be taught self-MLD and breathing techniques
• Measurement and fitting of standard and custom-made compression garments (European compression class)
• Kinesio-taping: a specialist hypo-allergenic tape that enhances lymph drainage; particularly useful for trunk and breast oedema
• Specific exercise and lifestyle advice
• Support to patient and family.

People with mild/controlled lymphoedema

Individuals may be managed and/or followed up in primary care by a suitably trained practitioner who may be a lymphoedema practitioner, link practitioner or professional who has undergone a lymphoedema training programme.

This may include individuals with:

• Stable lymphoedema: the person may have had previous specialist treatment
• Mild oedema affecting arm only, where skin is soft and in good condition
• Oedema of the breast where the person is self-caring
• A limb that fits a standard ‘off-the-shelf’ compression garment that requires simple measurements
• No complications such as brachial plexus neuropathy or recurrent cellulitis.

Management may include:

• Fitting of compression garments: patients may require a regular supply (every 3-6 month) of repeat garments, with appropriate follow up care
• Support with self-care including advice on skin care, exercise, self-massage and weight management
• Referral to a self-management course, exercise group or support group
• Support to patient and family.

You have been given this leaflet because you have symptoms of lymphoedema.

What is lymphoedema?

Lymphoedema is a type of swelling. It develops when fluid does not drain easily from areas of the body through the lymphatic system. The lymphatic system normally helps to ensure a healthy balance of fluid in the body. It also deals with infections. Lymphoedema has various causes, and can be a long-term effect of cancer treatment.

How does lymphoedema affect people?

Swelling may be mild, and vary throughout the day. In some people, lymphoedema becomes more of a problem. It can affect the whole arm, hand, breast and/or torso. It may lead to skin changes, sensations of heaviness, and a risk of infection (cellulitis).

What can be done to improve lymphoedema?

Lymphoedema can be a distressing problem, but with the correct advice and treatment, it can be well controlled. Treatments for arm lymphoedema usually include use of a compression garment to reduce the swelling. Your doctor may refer you for assessment and treatment with a lymphoedema practitioner.

What can I do to manage my lymphoedema?

You can take an active role by following these lymphoedema self-management tips:

Skin care:

• Wash and moisturise your skin daily to keep your skin healthy and supple
• Protect your skin from damage that may lead to more swelling: use insect repellent to avoid insect bites; wear gloves when gardening; protect from sunburn; avoid having your blood pressure taken or injections on the arm
• Use antiseptic cream or spray on any breaks in your skin on the affected area
• If you are unwell, and your arm is red, sore, or more swollen, see your doctor or call NHS 24 immediately as you may have an infection (cellulitis)

Movement and exercise:

• Keep moving your arm as normally as possible, but avoid repetitive movements, and lifting heavy weights; gradually build up the strength in your arm
• Wear a compression garment, particularly while exercising; take it back for more advice if it does not fit.

There may be a lymphoedema group or lymphoedema professional in your area. Ask for more information through your GP surgery or hospital team.