Lymphoedema

Appropriate assessment

Causes

Explanation to patient and family

Consider disease specific treatment

Non-pharmacological management

Four main principles of treatment

Specific problems

Pharmacological management

Conclusion

Involve the interdisciplinary team

Consider referral to specialist care

In palliative care, lymphoedema is usually due to blockage of lymphatic vessels and glands in cases of malignancy or fibrosis as a result of previous radiotherapy or surgery. There is often a latent period that averages 18-24 months from lymphatic damage to manifestation of oedema. Lymphoedema differs from other forms of chronic oedema in that the swelling is not due to fluid accumulation alone. 

Appropriate assessment 

• Chronic condition – usually affecting limbs (can affect trunk, head, genitalia) 
• Symptoms – discomfort, heaviness, tightness, bursting feeling, pain 
• Signs – generally a combination of non-pitting and pitting oedema

Oedema that does not reduce significantly after overnight elevation is likely to be lymphatic in origin. 

• Progressive changes – grossly swollen, coarseness, folding and distortion of skin, elephantiasis (which differs from all other forms of oedema) 
• Stemmer’s sign: thickened skin folds of the toes prevent pinching of the skin, particularly at the base of the second toe. 
• Risk factors – 
  • Obesity, 
  • Lack of physical exercise. 
• Exclude other causes of oedema in a unilateral swollen limb: 
  • e.g. Venous outflow obstruction in breast cancer: dilated collateral veins around shoulder and chest wall, skin that appears mottled or cyanosed. 
  • Lack of movement in limb which limits the activation of the muscle pump, which in turn helps with lymph and venous drainage. 
  • Neurological deficit causing reduced mobility. Progressive neurological signs may be the first clue to recurrence of tumour by infiltration or compression. 
  • Hypo-albuminaemia 
  • DVT 

Causes  

Primary lymphoedema – congenital 

Secondary lymphoedema (damage to lymphatic system) 

• Cancer 
• Treatment of cancer (surgery and radiotherapy) 
• Trauma 
• Infection – post cellulitis, filariasis in tropical countries 
• Chronic venous disease 
  • Deep vein thrombosis (DVT) 
  • Venous insufficiency 

Explanation to patient and family 

The aim of lymphoedema treatments is to encourage lymph transport by means of physical therapies, to prevent complications, to achieve maximum improvement and long-term control. Success requires full patient cooperation and the ultimate aim is to make the patient feel more comfortable. 

Consider disease specific treatment 

• Decrease tumour bulk if possible, by radio- or chemotherapy 
• Treat cellulitis promptly 

Non Pharmacological management 

Regular reassessment is needed to adjust treatment as the condition progresses. 

Four main principles of treatment

SKIN CARE 

1. Scrupulous skin care to prevent dryness, cracking and infection. 
2. Avoid trauma, exposure to heat, venepuncture, acupuncture or vaccinations in the affected limb to minimise risk of infection. 
3. Avoid insect bites 

EXTERNAL SUPPORT/COMPRESSION 

1. Compression bandaging: when the aim is to re-shape the limb and decrease the swelling: multi-layer lymphoedema bandaging (MLLB) – this is used daily in the intensive phase of treatment to increase the lymph drainage when the limb is exercised. It’s left in place and re-applied every 24 hrs. (First a tubular stocking is applied as protection against chafing, next a layer of padding to protect the joint flexures and to even out distortion in the shape of the limb, finally high-compression bandages are applied to give graduating pressure) 
2. External support: when reduction of limb size is not anticipated or necessarily desirable: These garments are used during the maintenance phase to prevent increased swelling. They require specialist measurement v fitting and should be replaced on a regular basis. They provide graduated external pressure and are not the same as anti-embolism stockings which do not offer graduated pressure. 
3. Compression pumps have been used but may cause problems with skin integrity and misdirect oedema into unwanted areas. 

MASSAGE 

1. The aim is to move lymph from a congested area towards an area where the lymphatics are functioning normally. 
2. Manual lymphatic drainage (MLD) is the use of specific massage techniques by a qualified therapist. 
3. Simple lymphatic drainage: modified form of MLD; should only be enough to move the skin and tissues beneath the hand; it should not cause the skin to redden; involves stroking the surface of the body more than a massage; the skin should be free of oils, creams or powder when massaging; can be performed by the patient or carer in the maintenance phase of treatment 

MOVEMENT 

1. Exercises in the form of gentle movements to improve or simply preserve movement in the swollen limb and prevent joint stiffness. 
2. Exercise programme may be provided. 
3. There is no need to elevate the limb beyond the horizontal. 
4. Avoid sitting or standing for prolonged periods. 
5. Movement is better than elevation 

Specific problems 

LYMPHORRHOEA 

Clean the limb, apply thick sterile pad to the leakage area, bandage in usual way, maintain pressure for 24 to 48 hours 

TUMOUR IN THE ABDOMEN OR PELVIS 

• If chemo- or radiotherapy have not been able to decrease the size of a pelvic tumour, which may cause obstruction to venous or lymph flow, high dose steroids may bring some relief by reducing the peritumour oedema of a tumour. If used in large doses over a long period, steroids may cause more fluid retention and lead to muscle wasting. 

Pharmacological management 

1. Cellulitis (streptococcal infections are the most common) should be treated promptly: Penicillin V 250 mg qid or erythromycin 250 mg gid or amoxicyllin 250- 500 mg tds. 
2. Patients who have had 2 or more episodes of cellulitis should have prophylactic antibiotics. Penicillin V 250 mg bd or erythromycin 250 mg bd. 
3. Diuretics are of very limited use in treating lymphoedema. The long term use of diuretics is contra-indicated. They may be useful in palliative care where oedema may be very tense and prognosis is short. 
4. Benzopyrone group of drugs which may promote breakdown of fibrous tissue have been used in some centres to treat lympoedema, but concerns regarding hepato-toxicity remain and they are not indicated for routine use. 

Conclusion 

Conservative physical treatments are the mainstay of therapy and need to be initiated early and perhaps even prophylactically. 

Involve the Interdisciplinary team 

Once lymphoedema is established, it is incurable. Therefore the whole team is involved when patients suffer from lymphoedema for support and motivation in empowering the patient and family to help prevent complications. Some patients are unable to assume their duties because the lymphoedematous limb has restricted movement, is heavy and at risk for developing infection, especially when in frequent contact with water or chemicals. The psychological condition of the patient should be evaluated. Studies have shown that emotional distress, psychosocial maladjustment and psychological morbidity are all significantly higher in women with lympoedema. They experience more anxiety, depression, sexual dysfunction, disturbance of body image and social avoidance than those who do not develop lymphoedema. 

Consider referral to specialist care 

There may be a lymphoedema clinic/service available.