Lymphoedema creates distressing, debilitating and uncomfortable swelling. Lymphoedema most commonly affects a limb but the face, neck, abdomen, genitals and internal organs can be involved. It is still not a well-documented condition, but early diagnosis and treatment are essential for the optimum management of the disorder.
Symptoms, Types and Causes
Lymphoedema is a serious disorder for which there is no cure and which, without the appropriate treatment can gradually deteriorate. A worsening of the condition is not limited to just the increase in size of the limb but also to the insidious changes in tissue structure, e.g. elephantiasis, and susceptibility to frequent acute inflammatory episodes (infections) with the resultant long-term dependency on antibiotics.
The physical and psychological impact on the patient with a swollen limb is immense. Extreme discomfort, heaviness in the affected part and problems of movement can be experienced. Over time, there may be a gradual thickening of the skin at the affected site and spontaneous lymphorrhea can occur. Repeated acute inflammatory episodes sometimes resulting in hospitalisation are not unknown. The quality of life is greatly diminished. Clothes and shoes that fit are difficult to find. The person has to adapt to an altered and more limited daily lifestyle. There is reduced working capability, intolerance to heat (climatic or in a sauna or baths for example) and minimal participation in social activities.
Primary lymphoedema - is due to a fault within the lymph conducting pathways, i.e. no identifiable cause. It is usually determined from birth and may be due to malformation of the lymphatic system. Swelling develops progressively at different stages in life and presents usually in the lower limbs. It is normally indolent but associated discomfort and debility are very common. Congenital lymphoedema can also be associated with numerous developmental abnormalities. There are several different types of primary lymphoedema:
Nonne-Milroy disease - congenital familial lymphoedema presenting at birth or soon thereafter. Any part of the body may be affected.
Lymphoedema Praecox - onset is usually during puberty and represents some 80% of all primary lymphoedemas, usually affecting the lower limbs.
Lymphoedema Tardum - onset is after 35 years of age.
Secondary lymphoedema - is when the cause of lymphatic system failure is known, e.g. surgery, radiotherapy, infection, trauma such as severe injury, burns or perhaps thrombosis. The progression of lymphoedema can be classified in three stages:
the reversible stage when swelling is soft and will pit easily with light finger pressure. The lymphoedema will disappear with bed rest.
the spontaneous irreversible stage when the swelling hardens, pitting is not so obvious and there is the slow formation of fibrous tissues (fibrosis) where protein-rich fluid has collected.
the elephantiasis stage when there is extreme swelling and thickening of the skin.
Drug therapy is of limited value but diuretics are still recommended to many patients. These, in fact, have little or no effect on lymphoedema and can cause long-term problems. Pressure pumps are contra-indicated except under strict medical control. Surgery, such as 'debulking' or 'reduction' is a procedure of last resort. A person with lymphoedema, or at risk from lymphoedema following surgery, should never allow blood to be drawn, blood pressure to be taken or injections given in the affected limb or body part as these procedures may well manifest or exacerbate the condition.
Lymphoedema cannot be cured but it can be controlled with early diagnosis, correct and early treatment and ongoing management. Decongestive Lymphatic Therapy (DLT) otherwise known as Complex Decongestive Therapy (CDT) has been the recommended treatment for lymphoedema for many years. Initially, an intensive course of treatment can last from ten days to six weeks. Following a course of treatment, compression garments will usually have to be worn indefinitely to maintain the volume reduction and tissue improvement achieved. It is also necessary to ensure that the body weight remains, or is brought within, normal limits. After six months to a year, a repeated course of intensive treatment may be necessary. In the meantime, daily exercises and, in an ideal world, regular manual lymphatic drainage therapy should be recommended.
Patient compliance has to be 100% in following the medical recommendations on a daily basis between treatments and often the patient will have to reconsider their work and social lifestyles in order to comply. The overall benefit is that the lymphoedematous region is returned to as near normal as possible. The patient can enjoy an improvement in their quality of life and there are fewer, if any, episodes of cellulitis or other life-threatening infections.
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