Treatments

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Pulmonary fibrosis can be managed using many different treatment strategies, as well as by making some lifestyle changes

An operation to replace a patient’s diseased lungs with lungs from a donor
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The worsening of a disease/condition over time

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A formal documentation when someone else is given the responsibility to manage your affairs and make certain decisions on your behalf

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Refers to the lungs

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A group of tests used to check how well the lungs take in and release air and how well they supply oxygen to the rest of the body14

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A type of high blood pressure that affects the blood vessels to the lungs and the right side of the heart15

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Therapy that provides relief from symptoms to help patients live more comfortably with their disease13

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Administration of oxygen as a medical intervention11

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A small plastic tube or prongs that fit in the nostrils to deliver supplementary oxygen11

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Listening to and playing music as a therapy which aims to ease the symptoms of those living with IPF

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A sleep disorder characterised by breathing that repeatedly stops and starts during sleep12

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A specialist who helps someone recover or live with their symptoms more easily

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A machine that removes other gases from the air to provide oxygen for oxygen therapy11

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An education and exercise program designed to improve the quality of life for people with lung conditions16

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A physician specialised in lung problems (also known as a respirologist)

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A product that gives protection against a specific infection

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A test used to monitor oxygen levels in a patient’s blood, usually with a non-invasive sensor11

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A breathing technique to help control breathlessness and reduce anxiety17

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A disease that affects only a small percentage of the population

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Something that is associated with an increased risk of disease or infection

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A technique that helps to reduce stress and anxiety by helping to understand and manage your emotions

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Therapies used alongside conventional treatments that help treat symptoms and may improve overall physical and mental wellbeing

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A medical study that investigates how safe and effective a new therapy or technique is for treating a certain disease

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A disease or condition that occurs at the same time as another disease or condition

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A device to deliver compressed air to improve sleep in people with obstructive sleep apnoea6

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A disease where a waxy substance (plaque) builds up inside the coronary arteries, which supply oxygen-rich blood to the heart muscle7

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A type of chronic disease that typically worsens over time and is characterized by long-term breathing problems and poor airflow. Chronic bronchitis and emphysema are older terms used for COPD5

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Refers to the heart, and blood vessels

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A type of medication that aims to slow the scarring and stiffening of lungs to slow disease progression2

View in glossary

Tiny air sacs in the lungs where the exchange of oxygen and carbon dioxide to and from the blood takes place

View in glossary

A test that shows how well the lungs are working by measuring how much oxygen and carbon dioxide is in the blood. This test requires that a small volume of blood be drawn from the patient3

View in glossary

Techniques that involve breathing in a certain way to control breathlessness and strengthen your lungs

View in glossary

A procedure in which a bronchoscope (a medical instrument like a tube) is passed through the mouth or nose into the lung and fluid is squirted into a small part of the lung and then collected for examination4

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A lung condition where the air sacs within the lungs (alveoli) become damaged5

View in glossary

Extreme weariness resulting from exertion or illness

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Of unknown cause

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Rapid and uncontrolled breathing

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A disease where there is progressive scarring or thickening of the lungs without a known cause4

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The surgical removal of cells or tissue samples from the lung for examination by a pathologist10

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The lung tissue becomes thickened and stiff

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A test that uses a type of X-ray that produces multiple, detailed images of areas inside the body4

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A burning sensation in the chest, which can spread to the throat, along with a sour taste in the mouth

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Where inflamed tissue is replaced with scar tissue, making it thicken and become stiffer4

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A symptom where the ends of the fingers become wider and rounder8

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A digestive disease where stomach acid moves up out of the stomach and irritates the lining of the food pipe (oesophagus)9

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A physician specializing in the management of diseases of the digestive system

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An event characterized by sudden, severe worsening of symptoms or an increase in disease severity1

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While there is no cure or way to reverse pulmonary fibrosis, various therapies may help to alleviate your symptoms.1,2  Treatment strategies can include options such as medications to fight inflammation in some specific disease, as well as pulmonary rehabilitation, oxygen therapy and lung transplantation.2,3

The main goal of your treatment will usually be to try to control your symptoms. Your treatment team will be able to work with you to develop a treatment plan that suits your needs.

An operation to replace a patient’s diseased lungs with lungs from a donor
View in glossary

The worsening of a disease/condition over time

View in glossary

A formal documentation when someone else is given the responsibility to manage your affairs and make certain decisions on your behalf

View in glossary

Refers to the lungs

View in glossary

A group of tests used to check how well the lungs take in and release air and how well they supply oxygen to the rest of the body14

View in glossary

A type of high blood pressure that affects the blood vessels to the lungs and the right side of the heart15

View in glossary

Therapy that provides relief from symptoms to help patients live more comfortably with their disease13

View in glossary

Administration of oxygen as a medical intervention11

View in glossary

A small plastic tube or prongs that fit in the nostrils to deliver supplementary oxygen11

View in glossary

Listening to and playing music as a therapy which aims to ease the symptoms of those living with IPF

View in glossary

A sleep disorder characterised by breathing that repeatedly stops and starts during sleep12

View in glossary

A specialist who helps someone recover or live with their symptoms more easily

View in glossary

A machine that removes other gases from the air to provide oxygen for oxygen therapy11

View in glossary

An education and exercise program designed to improve the quality of life for people with lung conditions16

View in glossary

A physician specialised in lung problems (also known as a respirologist)

View in glossary

A product that gives protection against a specific infection

View in glossary

A test used to monitor oxygen levels in a patient’s blood, usually with a non-invasive sensor11

View in glossary

A breathing technique to help control breathlessness and reduce anxiety17

View in glossary

A disease that affects only a small percentage of the population

View in glossary

Something that is associated with an increased risk of disease or infection

View in glossary

A technique that helps to reduce stress and anxiety by helping to understand and manage your emotions

View in glossary

Therapies used alongside conventional treatments that help treat symptoms and may improve overall physical and mental wellbeing

View in glossary

A medical study that investigates how safe and effective a new therapy or technique is for treating a certain disease

View in glossary

A disease or condition that occurs at the same time as another disease or condition

View in glossary

A device to deliver compressed air to improve sleep in people with obstructive sleep apnoea6

View in glossary

A disease where a waxy substance (plaque) builds up inside the coronary arteries, which supply oxygen-rich blood to the heart muscle7

View in glossary

A type of chronic disease that typically worsens over time and is characterized by long-term breathing problems and poor airflow. Chronic bronchitis and emphysema are older terms used for COPD5

View in glossary

Refers to the heart, and blood vessels

View in glossary

A type of medication that aims to slow the scarring and stiffening of lungs to slow disease progression2

View in glossary

Tiny air sacs in the lungs where the exchange of oxygen and carbon dioxide to and from the blood takes place

View in glossary

A test that shows how well the lungs are working by measuring how much oxygen and carbon dioxide is in the blood. This test requires that a small volume of blood be drawn from the patient3

View in glossary

Techniques that involve breathing in a certain way to control breathlessness and strengthen your lungs

View in glossary

A procedure in which a bronchoscope (a medical instrument like a tube) is passed through the mouth or nose into the lung and fluid is squirted into a small part of the lung and then collected for examination4

View in glossary

A lung condition where the air sacs within the lungs (alveoli) become damaged5

View in glossary

Extreme weariness resulting from exertion or illness

View in glossary

Of unknown cause

View in glossary

Rapid and uncontrolled breathing

View in glossary

A disease where there is progressive scarring or thickening of the lungs without a known cause4

View in glossary

The surgical removal of cells or tissue samples from the lung for examination by a pathologist10

View in glossary

The lung tissue becomes thickened and stiff

View in glossary

A test that uses a type of X-ray that produces multiple, detailed images of areas inside the body4

View in glossary

A burning sensation in the chest, which can spread to the throat, along with a sour taste in the mouth

View in glossary

Where inflamed tissue is replaced with scar tissue, making it thicken and become stiffer4

View in glossary

A symptom where the ends of the fingers become wider and rounder8

View in glossary

A digestive disease where stomach acid moves up out of the stomach and irritates the lining of the food pipe (oesophagus)9

View in glossary

A physician specializing in the management of diseases of the digestive system

View in glossary

An event characterized by sudden, severe worsening of symptoms or an increase in disease severity1

View in glossary

It is important to remember that treatment decisions for pulmonary fibrosis are personal for each patient. The treatment decision may be affected by factors such as: 2-4

  • Other conditions you may have
  • Other medications you may be taking
  • Treatment side effects
  • If there is a risk of acute exacerbations (a sudden and rapid worsening of your condition)
  • Your age and physical condition
  • Your personal preference for treatment options

It's important that you talk to your treatment team about which therapy options may be right for you.

Show references Hide references
  1. 1.
    1. Lipsi R, Mazzola D, Caminati A, et al. Severe idiopathic pulmonary fibrosis: A clinical approach. Eur J Intern Med. 2018;50:20–27.
  2. 2.
    1. Holtze C, Flaherty K, Kreuter M, et al. Healthcare utilisation and costs in the diagnosis and treatment of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180078.
  3. 3.
    1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2011;183(6):788–824.
  4. 4.

    Richeldi L, Varone F, Bergna M, et al. Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence. Eur Respir Rev. 2018;27(150):180074.

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It is important to stay positive, to find the doctor that you trust in, to have a good support from the family, stay active, have a healthy food intake, and so overall it is important to create a positive atmosphere
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