Pulmonary fibrosis (idiopathic)

Treating idiopathic pulmonary fibrosis

Syndication_logo

There is currently no cure for idiopathic pulmonary fibrosis. The main aim of treatment is to relieve symptoms of the disease as much as possible and slow down its progression.

Medication

Medication can help reduce symptoms in some people, but not everyone. Some people, particularly elderly people whose symptoms are mild or not getting worse, may be advised not to take any medication. This is because possible side effects could be worse than the progression of the disease.

Steroid tablets

A low dose of a corticosteroid called prednisone may be prescribed. If there is no improvement after a few months, the treatment may be stopped.

Taking steroid medication, especially for a prolonged period of time, can cause side effects including:

  • weight gain
  • osteoporosis (brittle bones, which especially affects women after the menopause)
  • cataracts (cloudy patches in the lens in the eye)
  • glaucoma (an eye disease that impairs vision)
  • stomach problems

It can also trigger diabetes and raised blood pressure. For more information, see Side effects of corticosteroids.

Your doctor or specialist should always weigh up potential benefits of steroid treatment against the risk of side effects.

Other medications

A number of other medications have been recently used to see if they could be effective in treating IPF. The most promising are described below.

N-acetylcysteine

N-acetylcysteine is what is known as an antioxidant. Antioxidants can have a limited effect in protecting cells against damage that can occur with aging.

There is evidence that combining N-acetylcysteine with a corticosteroid can slow the progression of IPF in the short-term. Its long-term effectiveness is uncertain.

N-acetylcysteine is available from many health shops on an over-the-counter basis. Check with the doctor in charge of your care before taking this medication as it may not be safe or suitable for everyone with IPF.

Sildenafil

Sildenafil is a medication that can widen the blood vessels inside the lungs which should help improve blood flow while reducing blood pressure (useful if you also have pulmonary hypertension),

Research has found sildenafil can lead to a modest improvement in breathlessness which can improve a person’s range of daily activities. This in turn can lead to an improvement in quality of life.

Sildenafil may not be suitable for you if you:

  • are also taking medicines or drugs that contain nitrates – organic nitrates are often used to treat angina and amyl nitrate is a recreational drug commonly known as "poppers”
  • have a severe problem with your liver 
  • have recently had a heart attack or stroke
  • have severe low blood pressure
  • have certain eye conditions

Common side effects of sildenafil include:

Pirfenidone

Pirfenidone is an antifibrotic medication that may be used for treating adults with mild to moderate IPF.

More studies are needed on its long-term effects, but current research suggests pirfenidone is able to slow the progression of IPF.

Side effects of pirfenidone may include:

  • nausea
  • vomiting
  • diarrhoea
  • abdominal pain
  • tiredness (fatigue)
  • photosensitivity rash (on exposure to sunshine)

Pirfenidone is currently only prescribed on a named patient basis by specialist centres.

Oxygen support

When idiopathic pulmonary fibrosis is severe, levels of oxygen in your blood fall and you feel more breathless. In this case your specialist may prescribe you oxygen, which can help with your breathing and enable you to be more active.

You will need to take oxygen through nasal tubes or a mask from a portable machine called an oxygen concentrator. This device provides air with a much higher oxygen level than the air you breathe in naturally. The tubes from the machine are long, so you will be able to move around your home while connected.

You can also use an oxygen inhaler when you are out and about.

Pulmonary rehabilitation

Pulmonary rehabilitation is a multidisciplinary treatment (involving many different types of healthcare professionals) used for many long-term lung conditions. Courses aim to help a patient come to terms with their condition, learn the best ways to deal with it and improve their ability to function on a daily basis without extreme breathlessness.

Courses in pulmonary rehabilitation are usually held locally and may include:

  • education about pulmonary fibrosis
  • physical exercise
  • breathing exercises
  • advice on nutrition
  • psychological support
  • a social support network

For more information, watch the above video in which a respiratory nurse specialist and patients explain pulmonary rehabilitation.

Lung transplant

If your condition continues to get worse despite other forms of treatment, your consultant may recommend a lung transplant. The decision to have a transplant will be based on:

  • how bad your condition is
  • how quickly your condition is getting worse
  • your age and general health
  • how much your condition is likely to improve after a transplant
  • whether a donor lung is available

Few people with idiopathic pulmonary fibrosis are suitable candidates for a lung transplant.

Self help

As well as medical treatments above, there are measures you can take to stay as healthy as possible:

  • stop smoking
  • exercise regularly and stay as fit as you can
  • eat a good, balanced diet
  • take the flu jab and the pneumococcal vaccination (influenza and pneumococcal bacterial infections can become severe if you have a lung disease)
  • try to keep away from people with chest infections and colds