Lymphoedema
Introduction
Lymphoedema is a chronic (long-term) condition that causes swelling in the body's tissue. This can lead to pain and a loss of mobility.
Lymphoedema usually affects the arms or legs, although in some cases there may be swelling in the:
Lymphoedema is caused by damage or disruption to the lymphatic system (see below).
One function of the lymphatic system is to drain excess fluid from tissues. If the lymphatic system is disrupted or damaged, it can lose this ability and the excess fluid will cause the tissue to swell.
Types of lymphoedema
There are two main types of lymphoedema:
- Primary lymphoedema develops at birth or shortly after puberty and is caused by faulty genes.
- Secondary lymphoedema is caused by damage to the lymphatic system as a result of an infection, injury, trauma or cancer.
Secondary lymphoedema often develops as a side effect of cancer treatment. Surgery is often necessary to remove lymph glands to prevent a cancer from spreading. This can damage the lymphatic system.
Radiotherapy, where controlled doses of high-energy radiation are used to destroy cancer cells, can also damage the lymphatic system.
How common is lymphoedema?
It is estimated that 1 in every 10,000 people are affected by primary lymphoedema.
Secondary lymphoedema is a relatively common condition, affecting an estimated 100,000 people in the UK.
Secondary lymphoedema occurs more frequently in women, possibly because it can sometimes be a side effect of breast cancer treatment.
Cancer Research UK estimates than one in five women may have lymphoedema in their arm after they have had radiotherapy or lymph nodes removed to treat breast cancer.
Outlook
There is no cure for lymphoedema, but it is possible to control the symptoms using a combination of different techniques, such as massage and compression garments. See Lymphoedema - treatment for more information.
People with lymphoedema are more vulnerable to infection. This is because infection-fighting white blood cells, called lymphocytes, which travel in the lymphatic system, are prevented from reaching the part of the body where they are needed.
A bacterial infection of the skin called cellulitis is one of the most commonly reported infections in people with lymphoedema.
The lymphatic system
As well as a circulatory system that transports blood from the heart to the rest of the body, your body has a second circulatory system known as the lymphatic system.
The lymphatic system is made up of a network of vessels (channels) and glands called lymph nodes, which are distributed throughout the body. Lymph nodes are small, oval glands that remove unwanted bacteria and particles from the body. They are part of the immune system (the body’s natural defence system).
The lymphatic system has two important functions:
- It helps to fight infection. The lymphatic system contains a fluid called lymph, which is full of infection-fighting cells known as lymphocytes.
- It drains excess fluid from tissue. As the blood circulates through your tissue, it leaves behind waste products such as fluids and proteins. This material and fluid is removed from the tissues by the lymphatic system, which filters out any bacteria or viruses and drains the remaining lymph back into your blood.
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Symptoms
The symptoms of lymphoedema include:
- swelling in part or all of a limb - this can lead to difficulties in wearing jewellery/watches or fitting into clothes or shoes
- the affected limb can feel heavy and aching
- the affected limb loses some of its mobility
- pain in the affected limb
- painful joints, such as the elbow or knee, caused by swelling in the limb
- the affected limb develops repeated skin infections
- the skin on the affected limb becomes hard and tight, and blisters or wart-like growths can develop on the surface
The start of symptoms
In around 1 in 10 people, primary lymphoedema can cause symptoms that are present from birth. Most people with primary lymphoedema will experience symptoms after puberty.
Secondary lymphoedema, which is caused by an illness or injury, can appear at any time. For example, if you have had breast cancer that has been treated with radiotherapy, you may need to take precautions against lymphoedema for the rest of your life. See Lymphoedema - prevention for information and advice about avoiding lymphoedema.
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Causes
Primary and secondary lymphoedema have different causes.
Primary lymphoedema
Primary lymphoedema is caused by alterations (mutations) in some of the genes that are responsible for the development of the lymphatic system. The mutated genes interfere with the development of the lymphatic system, meaning it loses its ability to drain fluid properly.
Due to the genetic nature of primary lymphoedema, the condition usually runs in families. However, not everyone born to a parent with primary lymphoedema will develop the condition.
Primary lymphoedema is more common in females.
Secondary lymphoedema
Secondary lymphoedema can have a number of different causes. The most common causes are summarised below.
Surgical treatment of cancer
Cancer uses the lymphatic system to spread through the body, part of the treatment for cancer sometimes involves a surgeon to remove cancerous lymph nodes. The surgeon will try to ensure that the rest of your lymphatic system can still operate normally, although this is not always possible.
Cancers that have a significant risk of lymphoedema arising as a complication of treatment include:
Radiotherapy
Radiotherapy uses controlled doses of high-energy radiation to destroy cancerous tissue. However, it can also damage healthy tissue. If it is necessary to use radiotherapy to destroy cancerous cells that are present in your lymph nodes or vessels, there is a risk that the nodes and vessels will become permanently damaged.
See the Health topic about Radiotherapy for more information about this type of treatment.
Infections
In some cases, an infection can cause lymphoedema.
The bacterial skin infection cellulitis, is not only a complication of lymphoedema, it can also be its primary cause. In a severe cellulitis infection, the tissue around lymph nodes or vessels can be damaged, leading to scarring.
Another infectious cause of lymphoedema is a parasite infection called filariasis. This tends only to be a risk in parts of the developing world, such as parts of India, but is not a risk in the UK.
Inflammation
Conditions that cause tissue to become inflamed (red and swollen) can also permanently damage the lymphatic system. Some health conditions that can cause lymphoedema include:
- rheumatoid arthritis, which causes pain and swelling in the joints
- eczema, which causes the skin to become itchy, reddened, dry and cracked.
Venous diseases
Venous diseases, which affect the flow of blood in the veins, can lead to lymphoedema in some people. The abnormal or damaged veins can result in excess blood or fluid building up in tissues, can cause tissue damage. This can affect the drainage of the lymphatic system.
Some venous diseases that can lead to lymphoedema in some people include:
- deep vein thrombosis (DVT), a blood clot in one of the deep veins in the body
- varicose veins (swollen and enlarged veins), where poor drainage of blood in the veins causes higher vein pressure and more fluid passes into the tissues
Trauma and injury
A general injury or trauma, such as a burn or extensive scarring, can cause lymphoedema in a small minority of cases.
Reduced movement
The movement of the surrounding muscles when you exercise or move helps lymph drainage. Therefore, reduced movement can lead to lymphoedema because the fluid in the lymphatic system does not get moved on, causing swelling.
People who are paralysed (unable to move) may be at risk of developing lymphoedema as a result of being unable to move. Someone who is chair-bound for a long period of time due to an illness may also be at risk of lymphoedema in their legs because they are not moving.
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Diagnosis
If it is felt that you are at risk of developing lymphoedema because you have undergone cancer treatments, you may be offered an assessment for lymphoedema as part of your after care treatment plan. Otherwise, you should contact your GP if you experience symptoms of swelling.
In some areas, there are specialist lymphoedema treatment centres. Your GP may refer you to one of these for further assessment.
In many cases, it is possible to make a diagnosis of lymphoedema by:
- asking you about your symptoms and medical history
- measuring the distance around the affected limb to see if it is enlarged
Other tests may be recommended in order to assess the extent and severity of your symptoms. These tests are described below.
Tape measure
A tape measure is used at 4cm (1.6in) intervals up the leg to measure the limb circumference and then calculate limb volume.
Water displacement method
The water displacement method is based on the principle that you can calculate the volume of an object by measuring how much water it displaces (the amount of water that is pushed out of the way when an object is placed in water).
You will be asked to place the affected limb in a tank of water and the amount of water that is displaced will then be measured. This measurement can be used to calculate the volume of your limb.
Perometery
A perometery is a technique that uses infra-red light to measure the volume in an affected limb. This process can accurately calculate how swollen your affected limb is.
Bioimpedance test
During a bioimpedance test, electrodes (small metallic discs) are placed on different parts of your body. The electrodes release a small and painless electric charge. The charge is then measured using a hand-held device. Changes in the strength of the current can indicate the presence of fluid within your tissue.
Imaging tests
Imaging tests may also be used to help with the diagnosis. These include:
- A lymphoscintigraph, where a radioactive dye is injected into your lymphatic system. The dye can then be tracked using a special scanner. The scanner can then show how the dye moves through your lymphatic system and check for any blockages.
- magnetic resonance imaging (MRI) scan, which uses a strong magnetic field and radio waves to produce detailed images of the inside of your body
- an ultrasound scan, which uses high-frequency sound waves to create an image of the inside of your body
- a computerised (axial) tomography (CT or CAT) scan, which uses X-rays and a computer to create detailed images of the veins or lymph nodes
These scans can be used to build up a clearer picture of the affected tissue. They are also useful in ruling out another condition known as lipoedema, in which the limbs swell due to abnormal fat deposits.
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Treatment
The recommended treatment for lymphoedema is a treatment plan known as Complex Decongestive Therapy (CDT). It is also known as Decongestive Lymphatic Therapy (DLT).
CDT is not a cure for lymphoedema, but it can help control the symptoms of swelling and pain. Although CDT takes time and effort, it can be used to effectively control your lymphoedema.
Complex Decongestive Therapy (CDT)
CDT is made up of four components of treatment:
- Manual lymphatic drainage (MLD) - a specialised massage technique designed to stimulate the flow of fluid and reduce swelling.
- Multilayer lymphoedema bandaging (MLLB) - uses bandages and compression garments to move the fluid out of the affected limb.
- Remedial exercises - are designed to activate the muscle in the limb to improve lymph drainage.
- Skin care - is required to prevent infection.
Each of these treatments is described in more detail below.
CDT begins with an intensive phase of therapy, during which you may receive daily treatment for up to six weeks. This is followed by the second phase known as the maintenance phase. During the maintenance phase, you will be encouraged to take over your own care using simple lymph drainage techniques (MLD administered by either yourself or a carer) and exercises while wearing compression garments.
You will then have regular six-monthly review meetings to check how your treatment is progressing.
Manual lymphatic drainage (MLD)
During manual lymphatic drainage (MLD), the lymphoedema therapist will use special massage techniques to move fluid from the swollen areas into working lymph nodes so it can be drained.
It is important that you receive MLD from a trained lymphoedema therapist, and not a regular masseur. This is because the technique requires a detailed knowledge of the lymphatic system to perform it correctly.
Your lymphoedema therapist will also teach you a range of massage techniques that you can use during the maintenance phase.
MLD can make some existing health conditions worse. For example, it may not be suitable if you currently have cancer or if you have a history of:
- heart disease, where your heart's blood supply is blocked or interrupted by a build-up of fatty substances in the blood vessels around your heart
- deep vein thrombosis (DVT), a blood clot in one of the deep veins in the body
Multilayer lymphoedema bandaging (MLLB)
Unlike the blood circulation system, there is no central pump, such as the heart, to move fluid around the lymphatic system. Instead, the lymphatic system makes use of the massaging effect of the muscles surrounding the lymph vessels and nodes to move the fluid.
So the goal of multilayer lymphoedema bandaging (MLLB) is to support the muscles during exercise and encourage them to move fluid out of the affected limb. MLLB is often used after a session of MLD to prevent fluid accumulating again in the limb.
You will also be taught how to correctly apply your own bandages and compression garments so you can continue to use MLLB during the maintenance period.
Remedial exercises
Remedial exercises are designed to strengthen the muscles involved in lymph drainage. You will be given your own personalised exercise plan that will be tailored for your requirements and ability.
Skin care
Taking very good care of your skin is important as it will reduce the risks of you developing a skin infection, such as cellulitis. You should be given a daily routine to follow, where you are required to meticulously clean your skin and check for any cuts, abrasions or signs of infection. For more advice on skin care see the prevention section.
Surgery
In some cases, a surgical procedure using liposuction (where a tube is used to suck fat out of tissue) can be used treat lymphoedema.
Liposuction is used to remove excess fat from the affected limb which can then help reduce the swelling. Once the surgery is complete, you will have to wear a compression garment on the affected limb for at least one year.
The National Institute for Health and Clinical Excellence (NICE) has announced that liposuction for chronic (long-term) lymphoedema is acceptable in terms of clinical safety. However, NICE states that there is currently no evidence about its long-term effectiveness or whether there are risks of long-term complications.
Access to liposuction for lymphoedema may be limited depending on your local NHS trust.
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Complications
Cellulitis is the most common complication of lymphoedema, but the condition can also cause psychological issues. These complications are explained below.
Cellulitis
Many people with lymphoedema experience repeated episodes of cellulitis. Cellulitis is a bacterial infection of the deep layer of skin (dermis) and the layer of fat and soft tissues (the subcutaneous tissues) that lie underneath the skin.
Symptoms of cellulitis include:
- redness and a feeling of heat in the skin
- pain
- a general feeling of being unwell
Cellulitis can usually be successfully treated with antibiotics (medicines to treat bacterial infections). See the Health topic about Cellulitis for more information about the condition.
Psychological affects
Living with a chronic (long-term) condition that affects the way you look can cause a great deal of distress. Lymphoedema can cause increased distress because many people will develop the condition after treatment for cancer.
If you find that you have been feeling particularly down for the last few months and you no longer take pleasure in the things you usually enjoy, you might be experiencing a period of depression. If this is the case, talk to your GP or members of your lymphoedema treatment team as there are a range of successful treatments for depression.
See the Health topic about Depression for more information and advice.
Talking to other people who also have lymphoedema can be reassuring and decrease feelings of isolation, stress and anxiety. The Lymphoedema Support Network offers information and advice and can put you in touch with a support group in your area.
The Complex Decongestive Therapy plan has proved successful in the treatment of lymphoedema, and if you persevere with treatment, your symptoms should become less noticeable and troublesome.
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Prevention
If you have received treatment for cancer, the following steps could help prevent lymphoedema. If you already have lymphoedema, this advice may stop it getting worse.
Skin care
The part of your body that is affected by lymphoedema will be more vulnerable to infection as it will not be receiving a regular supply of infection-fighting cells (lymphocytes). So any cuts in your skin can allow bacteria to enter your body and may quickly develop into an infection.
You can avoid infections and help prevent lymphoedema by:
- not having injections or blood pressure readings in the affected area
- treating cuts and scratches immediately with an antiseptic cream so that they do not become infected
- using insect repellents to prevent insect bites
- moisturising the skin daily to keep it supple (your GP may prescribe a suitable cream for this)
- avoiding very hot baths and showers (the heat from saunas, stream rooms and sun beds may also increase the swelling)
- using a high factor protection (SPF) sun cream to prevent sunburn
- wearing gloves for gardening and household tasks to avoid cuts
- cutting your nails with nail clippers and using hand cream regularly
- using anti-fungal powder to prevent athlete's foot, (a condition that makes the skin red, flaky and itchy)
- seeing a chiropodist for foot and nail care (inform them if you have lymphoedema)
- wearing shoes that fit properly and provide support at the top of your feet
- using an electric razor if you need to shave to reduce the risk of cutting yourself
- not wearing tight-fitting clothes or jewellery
Diet and lifestyle
Following a healthy diet is also important in controlling lymphoedema. Being overweight puts more strain on areas of the body that are already swollen. This can make it more difficult to fit compression garments over the affected limbs.
People who have a body mass index (BMI) of 30 or more are classed as obese and may be at increased risk of developing lymphoedema. Use the Healthy weight calculator to work out your BMI. See the Health topic about Obesity for more information about this condition.
Some people find that alcohol and heat cause an increase in swelling, while some have reported that air travel makes their symptoms worse.
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