Lymphangioleiomyomatosis (LAM)

Lymphangioleiomyomatosis, usually called LAM, is a rare condition which damages the lungs. It almost always occurs in females.

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What is LAM?

Lymphangioleiomyomatosis, usually called LAM, is a rare, long-term condition that gets worse over time. LAM almost always occurs in females.

  • Rare – happens in around 3 – 8 females per million people
  • Long-term – a condition you’ll always have

LAM mainly affects the lungs, but may also affect the kidneys and lymphatic system. The lymphatic system is a network of tubes (vessels) and glands (nodes) in the body that help fight infection and remove excess fluid.

Lymphangio – related to the lymph and blood vessels

Leiomyomatosis – the abnormal growth and formation of LAM cells

Lymphangioleiomyomatosis is pronounced: lim-FAN-jee-oh-ly-oh-my-oh-maTOE-sis.

What causes LAM?

LAM is caused by changes in the tuberous sclerosis complex (TSC) genes. This causes the growth of abnormal cells, called LAM cells.

LAM cells grow throughout the lungs, causing holes or cysts, blocking the airways and preventing the lungs from getting oxygen to the rest of the body. Sometimes, LAM cells also cause problems in the lymphatic system and kidneys.

There are two ways you can get LAM:

  • on its own – this is called sporadic LAM. The cause of sporadic LAM is currently unclear. Sporadic LAM is not inherited, and it is not passed on to children.
  • with a rare genetic condition called tuberous sclerosis. Tuberous sclerosis is passed on through family genes. Many people who have tuberous sclerosis also get LAM. But most people with LAM do not have tuberous sclerosis.

As LAM is a condition that almost always occurs in females, it’s thought the female hormone oestrogen encourages LAM cells to grow, but it’s not a direct cause.

What are the symptoms of LAM?

If you have LAM, your symptoms may include:

  • breathlessness, which may get worse the longer you have the condition and may get worse at the same time as your period
  • feeling very tired
  • developing a collapsed lung (a pneumothorax)
  • bloated stomach and fluid around the lungs
  • kidney tumours, called angiomyolipomas - they are usually not harmful but can be painful and cause bleeding around the kidneys
  • coughing up blood.

How is LAM diagnosed?

Because LAM is rare, it may take some time to get a diagnosis. LAM is usually suggested when lung cysts are seen on a CT scan or in females with tuberous sclerosis. As there are other causes of lung cysts, you may also have blood tests or a lung biopsy.

If LAM is suspected, you should also have an Ultrasound or CT scan of the stomach.

How is LAM treated?

LAM progresses at different rates and not everyone will need treatment.

There is currently no cure, but you may be given rapamycin (also called sirolimus) if you have a more active case of LAM. Rapamycin is an immunosuppressant medicine, which means it helps to calm your body’s immune system. This medicine can slow disease progression if taken continuously.

If you are breathless, you may be prescribed inhalers and offered pulmonary rehabilitation. You may also be assessed for oxygen therapy if your blood oxygen levels are low. A lung transplant may be an option for those with very severe disease.

In the UK, the National Centre for LAM is at Nottingham University Hospital. If LAM is suspected, it’s likely that you’ll need to go there to be diagnosed and treated.

If you have a pneumothorax (collapsed lung) or large or growing angiomyolipomas (kidney tumours), you might need additional hospital treatment.

Living with LAM

If you have LAM, you should eat a regular healthy diet and keep active. Stay up-to-date with your regular vaccinations - flu, coronavirus, and pneumonia. And if you smoke, take a look at our information on stopping smoking to help you quit.

As pneumothorax is a possible complication of LAM, talk to your health care professional about developing an action plan of signs to look out for and what you should do.

It’s thought oestrogen products can make LAM worse. This includes contraceptive pills and hormone replacement therapy (HRT). If you have LAM and are pregnant, or are considering pregnancy, talk to your health care professional and make sure to speak to your specialist team. There’s a possibility that pregnancy can also make LAM worse.

Living with a long-term, progressive condition like LAM can be difficult. It’s important to talk to your health care professional if you feel like your symptoms are getting worse. If you’re struggling with breathlessness, you might be able to be referred to pulmonary rehabilitation.

You should talk to your health care professional if you feel like you’re struggling emotionally – support is there if you need it. You could also take a look through our information on looking after your mental health.

Useful resources:

  • National centre for LAM – the centre for LAM diagnosis in the UK, based in Nottingham
  • LAM Action – a charity for those with LAM, their families and health care professionals.
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