Lymphoedema and lipoedema

What causes lymphoedema?

Your lymphatic system is like a miniature railway - a network of lymph ducts connected at 'stations' called lymph nodes or glands. It plays a major part in transporting white blood cells around your body to fight infection - the swollen glands you get in your neck when you have a sore throat are lymph nodes full of white blood cells. It also carries excess fluid away from your body tissues.

'Oedema' is the medical term for some kinds of swelling. Lymphoedema is swelling, often in your leg(s) or arm(s), caused by abnormal drainage in your lymphatic system. To begin with, the swelling may settle overnight but as time goes on, it becomes more constant, especially without treatment.

Primary lymphoedema

There are two main kinds of lymphoedema. 'Primary' lymphoedema isn't triggered by anything and is caused by faulty genes which mean your lymph system doesn't develop properly. It often runs in families.

Secondary lymphoedema

'Secondary' lymphoedema is much more common - it's caused by blockage of, or damage to, your lymph channels. Causes include:

• Removing lymph nodes during cancer surgery (particularly for melanoma and cancer of the breast, cervix, vulva, prostate and penis).

• Radiotherapy treatment for cancer.

• Infection - most commonly cellulitis, although a parasitic infection called filariasis is common in other countries.

• Past trauma causing damage to the lymphatic system.

• A deep vein thrombosis, in which a vein or veins carrying blood back to the heart (usually deep in the calf) become blocked off.

• Inflammation from other conditions such as severe eczema or rheumatoid arthritis.

• Being very overweight or immobile.

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What are the symptoms of lymphoedema?

Lymphoedema can give rise to:

• Uncomfortable swelling in part of all of the limb that's affected. To begin with the swelling may settle overnight and become more noticeable as the day wears on.

• Restriction of movement.

• A sensation of heaviness or aching of the limb.

• The skin (and jewellery or clothes you put on it) feeling tight.

• Leaking of clear fluid from the skin.

• Thickening of the skin.

But the main complication is skin infection called cellulitis. Red, hot, swollen, painful patches of skin are often accompanied by fever and feeling generally unwell. See your doctor for advice and assessment if this happens to you.

How is lymphoedema diagnosed?

If you have had surgery for cancer and have developed swelling in a nearby limb (for example, an arm following breast cancer surgery) your doctor may be able to make the diagnosis based on your symptoms and examining you. Your doctor will measure the circumference of both limbs to see if one is enlarged.

Sometimes you may need other tests. For instance, your doctor may arrange:

• Blood tests and a specialised ultrasound scan to exclude a deep vein thrombosis (a clot deep inside the limb).

• An MRI or CT scan to see if cancer is blocking the lymph nodes.

• A scan called a lymphoscintigram, which uses radioactive dye to check movement of lymph through your lymphatic system.

• Perometry - a method of measuring the volume of a limb using infrared light.

What are the treatments for lymphoedema?

Decongestive lymphatic therapy

A combination treatment called decongestive lymphatic therapy may be recommended. This is usually initiated and monitored by specialist clinics and includes:

• Regular exercises of the affected limb(s), which can improve lymphatic drainage.

• Compression bandages to help improve the drainage of lymphatic fluid, and cut the degree to which it recurs.

• Manual lymphatic drainage - a specialised form of massage, designed to stimulate lymphatic drainage.

• Skin care to reduce the risk of the skin infection called cellulitis.

Liposuction

The National Institute for Health and Care Excellence (NICE) has produced guidelines stating that there is strong enough evidence for liposuction to be recommended for some patients with chronic lymphoedema, if all standard treatments have not worked. It can only be recommended by a multidisciplinary team in specialist lymphoedema service centres. See the guidance in Further Reading and References below for more details.

Avoiding complications from lymphoedema

Taking special care of your skin is very important to reduce the risk of infection. If your feet are affected, regular visits to a podiatrist will help. If it's one arm, avoiding tight jewellery, blood pressure readings or injections on that side are all important.

As a general rule, tips to avoid infection if you have lymphoedema include:

• Keeping your weight down.

• Properly fitted shoes that don't rub if you have lymphoedema of the feet.

• Using insect repellents to avoid insect bites.

• Regular moisturising.

• Avoiding very hot baths (and sauna and steam baths); and

• Using an electric razor to avoid razor cuts.

Can you die from lymphoedema?

Lymphoedema itself is not fatal. Lymphoedema can however lead to skin infections (cellultitis). Rarely these infections can be serious and even fatal, but this is much less likely if they are treated early.

Lymphoedema might also be caused by conditions which can be fatal. For example, cancer might cause lymphoedema. If that cancer cannot be removed or otherwise treated, the cancer itself might eventually be fatal.

Can you fly with lymphoedema?

Lymphoedema does not stop you from flying. However, you will find that your lymphoedema will become worse during a flight. Longer flights are likely to cause more swelling than shorter flights. It is usually advisable to wear compression garments before, during and after the flight.

What is the outlook for lymphoedema?

In the early stages, it may be possible to reverse lymphoedema, especially if any blockage can be cleared. However, following cancer surgery this may not be possible. As time goes on, the tissues in the affected area become damaged, so lymphoedema cannot be cured.

However, treating lymphoedema with regular lymphatic drainage and compression, and taking steps to avoid complications, can often prevent it from worsening.

What is lipoedema?

Lipoedema is an abnormal build-up of fat cells, and it's almost exclusively a female condition. Unlike lymphoedema, it isn't caused by surgery or infection - it often runs in families and tends to develop when your hormone levels are fluctuating (puberty, pregnancy, menopause).

We tend to think of fat cells as being associated with being overweight. In fact, while lipoedema is made worse by carrying excess weight, many women with lipoedema are within the normal weight range.

Lipoedema symptoms

Lipoedema most commonly affects the whole of both legs and less often arms. It may look as if your legs, hips and buttocks are out of proportion with the rest of your body. It may start at around puberty, or possibly during pregnancy.

However, lipoedema does not affect feet or hands. This means that it may look as if there is a band or bracelet around your ankles or wrists.

As well as being swollen, your legs may feel achy, and you can develop small broken blood vessels or bruising. You may develop restriction of movement in your limbs and find it difficult to keep active.

In some cases, this can lead to low mood or even depression.

How is lipoedema diagnosed?

Your GP will ask you questions and examine you. In particular, they will be looking for:

• Disproportionately large legs compared to your feet or the rest of your body.

• Soft, cellulite-like fat on both legs (or arms).

• Tenderness over the skin.

• Easy bruising to the skin of the affected areas.

Because lipoedema often runs in families, it is important to explain to them which, if any, members of your family (almost always female) are affected.

How to diagnose lipoedema

Your doctor may want to refer you for the tests used to diagnose lymphoedema (above) to see whether your symptoms are caused by lipoedema. They may refer you to a specialist lymphoedema clinic. The staff here can help confirm the diagnosis. They can also help with treatment, both to improve your lipoedema and to prevent lymphoedema developing.

How to treat lipoedema

Unlike lymphoedema, losing weight makes little difference to lipoedema. Neither do 'water tablets' or keeping your legs raised - both of which can be very helpful in other forms of oedema.

There are two main groups of treatment for lipoedema, which can be helpful in lymphoedema too. The first is non-surgical. Compression garments don't affect the fatty tissue much but they can reduce swelling and discomfort. Low-impact exercise like swimming and massage can also help.

However, the only way to get rid of the fat cells that cause lipoedema is a form of liposuction. Unfortunately, you may need several operations and it's not usually available on the NHS. This is because there are potentially serious complications from the procedure, such as deep vein thrombosis and fat embolism (where fat travels in the bloodstream and lodges elsewhere in the body). There is also only limited evidence that this procedure is an effective treatment.

Can you prevent lipoedema?

Currently we do not understand what causes lipoedema. It might be related to the genetic make-up of your family, or to hormonal changes such as at puberty or the menopause. These are both factors which you cannot change so, at the moment, we do not know how to prevent lipoedema.

What is the outlook for lipoedema?

It is important to see your doctor if you think you might have lipoedema or lymphoedema. Without treatment, people with lipoedema can go on to develop lymphoedema. Lymphoedema and lipoedema can be distressing conditions, and being affected can affect your mental well-being. While neither condition can be cured, there is support out there that can make a big difference.