The worsening of a disease/condition over time
A formal documentation when someone else is given the responsibility to manage your affairs and make certain decisions on your behalf
Refers to the lungs
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
A type of high blood pressure that affects the blood vessels to the lungs and the right side of the heart15
Therapy that provides relief from symptoms to help patients live more comfortably with their disease13
Administration of oxygen as a medical intervention11
A small plastic tube or prongs that fit in the nostrils to deliver supplementary oxygen11
Listening to and playing music as a therapy which aims to ease the symptoms of those living with IPF
A sleep disorder characterised by breathing that repeatedly stops and starts during sleep12
A specialist who helps someone recover or live with their symptoms more easily
A machine that removes other gases from the air to provide oxygen for oxygen therapy11
An education and exercise program designed to improve the quality of life for people with lung conditions16
A physician specialised in lung problems (also known as a respirologist)
A product that gives protection against a specific infection
A test used to monitor oxygen levels in a patient’s blood, usually with a non-invasive sensor11
A breathing technique to help control breathlessness and reduce anxiety17
A disease that affects only a small percentage of the population
Something that is associated with an increased risk of disease or infection
A technique that helps to reduce stress and anxiety by helping to understand and manage your emotions
Therapies used alongside conventional treatments that help treat symptoms and may improve overall physical and mental wellbeing
A medical study that investigates how safe and effective a new therapy or technique is for treating a certain disease
A disease or condition that occurs at the same time as another disease or condition
A device to deliver compressed air to improve sleep in people with obstructive sleep apnoea6
A disease where a waxy substance (plaque) builds up inside the coronary arteries, which supply oxygen-rich blood to the heart muscle7
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
Refers to the heart, and blood vessels
A type of medication that aims to slow the scarring and stiffening of lungs to slow disease progression2
Tiny air sacs in the lungs where the exchange of oxygen and carbon dioxide to and from the blood takes place
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
Techniques that involve breathing in a certain way to control breathlessness and strengthen your lungs
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
A lung condition where the air sacs within the lungs (alveoli) become damaged5
Extreme weariness resulting from exertion or illness
Of unknown cause
Rapid and uncontrolled breathing
A disease where there is progressive scarring or thickening of the lungs without a known cause4
The surgical removal of cells or tissue samples from the lung for examination by a pathologist10
The lung tissue becomes thickened and stiff
A test that uses a type of X-ray that produces multiple, detailed images of areas inside the body4
A burning sensation in the chest, which can spread to the throat, along with a sour taste in the mouth
Where inflamed tissue is replaced with scar tissue, making it thicken and become stiffer4
A symptom where the ends of the fingers become wider and rounder8
A digestive disease where stomach acid moves up out of the stomach and irritates the lining of the food pipe (oesophagus)9
A physician specializing in the management of diseases of the digestive system
An event characterized by sudden, severe worsening of symptoms or an increase in disease severity1
IPF stands for idiopathic pulmonary fibrosis, a rare condition that affects 10-60 people in 100,000 each year.1 Fibrosis is the medical term for a build-up of scar tissue. In someone with IPF, the lungs become scarred, thickened and stiff. This makes it harder for oxygen to enter the blood and results in shortness of breath.1,2
In IPF, the exact cause of this lung scarring is unknown which means the pulmonary fibrosis is idiopathic.3 However, certain factors, such as cigarette smoking, some viral infections, genetics and GERD (gastro-oesophageal reflux disease) may increase the risk of IPF.4,5
Symptoms of IPF
There are a variety of symptoms you may experience with IPF and some of these will worsen as your disease progresses. Keep in mind that there are lifestyle changes and different treatments that can help you manage them. Symptoms can also vary from one person to another, so your symptoms may not be the same as somebody else who has IPF. 3,6-9
Most people don’t notice the changes to their lungs at first and may think they’re unfit or tired. This can be true but it’s important to be aware of your body’s capabilities and notice any changes.
Common signs and symptoms of IPF: 3
Cough is the most common symptom of IPF and may start long before the disease is diagnosed. It most commonly is a dry cough but occasionally affected people can bring up small amounts of sputum (phlegm). Shortness of breath, especially with physical activity develops as the disease gets worse.
The worsening of a disease/condition over time
A formal documentation when someone else is given the responsibility to manage your affairs and make certain decisions on your behalf
Refers to the lungs
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
A type of high blood pressure that affects the blood vessels to the lungs and the right side of the heart15
Therapy that provides relief from symptoms to help patients live more comfortably with their disease13
Administration of oxygen as a medical intervention11
A small plastic tube or prongs that fit in the nostrils to deliver supplementary oxygen11
Listening to and playing music as a therapy which aims to ease the symptoms of those living with IPF
A sleep disorder characterised by breathing that repeatedly stops and starts during sleep12
A specialist who helps someone recover or live with their symptoms more easily
A machine that removes other gases from the air to provide oxygen for oxygen therapy11
An education and exercise program designed to improve the quality of life for people with lung conditions16
A physician specialised in lung problems (also known as a respirologist)
A product that gives protection against a specific infection
A test used to monitor oxygen levels in a patient’s blood, usually with a non-invasive sensor11
A breathing technique to help control breathlessness and reduce anxiety17
A disease that affects only a small percentage of the population
Something that is associated with an increased risk of disease or infection
A technique that helps to reduce stress and anxiety by helping to understand and manage your emotions
Therapies used alongside conventional treatments that help treat symptoms and may improve overall physical and mental wellbeing
A medical study that investigates how safe and effective a new therapy or technique is for treating a certain disease
A disease or condition that occurs at the same time as another disease or condition
A device to deliver compressed air to improve sleep in people with obstructive sleep apnoea6
A disease where a waxy substance (plaque) builds up inside the coronary arteries, which supply oxygen-rich blood to the heart muscle7
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
Refers to the heart, and blood vessels
A type of medication that aims to slow the scarring and stiffening of lungs to slow disease progression2
Tiny air sacs in the lungs where the exchange of oxygen and carbon dioxide to and from the blood takes place
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
Techniques that involve breathing in a certain way to control breathlessness and strengthen your lungs
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
A lung condition where the air sacs within the lungs (alveoli) become damaged5
Extreme weariness resulting from exertion or illness
Of unknown cause
Rapid and uncontrolled breathing
A disease where there is progressive scarring or thickening of the lungs without a known cause4
The surgical removal of cells or tissue samples from the lung for examination by a pathologist10
The lung tissue becomes thickened and stiff
A test that uses a type of X-ray that produces multiple, detailed images of areas inside the body4
A burning sensation in the chest, which can spread to the throat, along with a sour taste in the mouth
Where inflamed tissue is replaced with scar tissue, making it thicken and become stiffer4
A symptom where the ends of the fingers become wider and rounder8
A digestive disease where stomach acid moves up out of the stomach and irritates the lining of the food pipe (oesophagus)9
A physician specializing in the management of diseases of the digestive system
An event characterized by sudden, severe worsening of symptoms or an increase in disease severity1
Keep a diary of your symptoms to track how you feel over time. Also, make a note of any questions and be sure to share your symptoms with your doctor
Receiving a diagnosis
IPF shares symptoms with many other diseases, meaning diagnosis can take a long time
Diagnosing IPF can take a long time (up to two years or more), which can be very frustrating for patients.3 Many people will have been given a lot of different tests before being diagnosed with IPF. However, doctors are always looking for ways to shorten the time to diagnosis.
When looking for the correct diagnosis of IPF, your treatment team will need to rule out all other diseases. They will check for different symptoms, ask about your medical history and perform some tests to reach a final diagnosis. This is why you may have seen many different types of doctors and specialists before being diagnosed with IPF. 3,10
How is IPF diagnosed?
When diagnosing IPF, your treatment team will look at many areas of your health. For example, they will ask you about your work history, family medical history, any potential drug and medication use and whether you have ever smoked.
A physical examination is also used to give the doctor a better understanding of your lung health. Your doctor, or you, might notice finger clubbing (widening and rounding of the tips of fingers or toes with a spongy sensation).
"Velcro-like" crackles will typically be heard when the doctor listens to the lungs with a stethoscope. However, other tests, particularly breathing tests and CT scans, will be needed to make a clear diagnosis.3,10
Treatments for IPF
Antifibrotic medicines are one of the most recent treatment options available for IPF that have been shown to improve patients’ outcomes. 11
Antifibrotics are medicines which aim to slow disease progression by slowing down the process that causes scarring in the lungs. They can be effective, so they will likely form a key part of your treatment plan. Speak to your doctor to find out if they are a suitable treatment option for you. 11
In many countries, there are two antifibrotic medicines available to treat IPF—nintedanib and pirfenidone. 11
Your doctor may sometimes prescribe treatments other than antifibrotics. These may aim to improve your symptoms and your general wellbeing and might include pulmonary rehabilitation or oxygen therapy. The treatment team will always consider your individual situation and work with you to decide on the treatment plan best suited to you.3,11
The worsening of a disease/condition over time
A formal documentation when someone else is given the responsibility to manage your affairs and make certain decisions on your behalf
Refers to the lungs
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
A type of high blood pressure that affects the blood vessels to the lungs and the right side of the heart15
Therapy that provides relief from symptoms to help patients live more comfortably with their disease13
Administration of oxygen as a medical intervention11
A small plastic tube or prongs that fit in the nostrils to deliver supplementary oxygen11
Listening to and playing music as a therapy which aims to ease the symptoms of those living with IPF
A sleep disorder characterised by breathing that repeatedly stops and starts during sleep12
A specialist who helps someone recover or live with their symptoms more easily
A machine that removes other gases from the air to provide oxygen for oxygen therapy11
An education and exercise program designed to improve the quality of life for people with lung conditions16
A physician specialised in lung problems (also known as a respirologist)
A product that gives protection against a specific infection
A test used to monitor oxygen levels in a patient’s blood, usually with a non-invasive sensor11
A breathing technique to help control breathlessness and reduce anxiety17
A disease that affects only a small percentage of the population
Something that is associated with an increased risk of disease or infection
A technique that helps to reduce stress and anxiety by helping to understand and manage your emotions
Therapies used alongside conventional treatments that help treat symptoms and may improve overall physical and mental wellbeing
A medical study that investigates how safe and effective a new therapy or technique is for treating a certain disease
A disease or condition that occurs at the same time as another disease or condition
A device to deliver compressed air to improve sleep in people with obstructive sleep apnoea6
A disease where a waxy substance (plaque) builds up inside the coronary arteries, which supply oxygen-rich blood to the heart muscle7
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
Refers to the heart, and blood vessels
A type of medication that aims to slow the scarring and stiffening of lungs to slow disease progression2
Tiny air sacs in the lungs where the exchange of oxygen and carbon dioxide to and from the blood takes place
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
Techniques that involve breathing in a certain way to control breathlessness and strengthen your lungs
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
A lung condition where the air sacs within the lungs (alveoli) become damaged5
Extreme weariness resulting from exertion or illness
Of unknown cause
Rapid and uncontrolled breathing
A disease where there is progressive scarring or thickening of the lungs without a known cause4
The surgical removal of cells or tissue samples from the lung for examination by a pathologist10
The lung tissue becomes thickened and stiff
A test that uses a type of X-ray that produces multiple, detailed images of areas inside the body4
A burning sensation in the chest, which can spread to the throat, along with a sour taste in the mouth
Where inflamed tissue is replaced with scar tissue, making it thicken and become stiffer4
A symptom where the ends of the fingers become wider and rounder8
A digestive disease where stomach acid moves up out of the stomach and irritates the lining of the food pipe (oesophagus)9
A physician specializing in the management of diseases of the digestive system
An event characterized by sudden, severe worsening of symptoms or an increase in disease severity1
Speak to your doctor about the treatment options available and discuss those best suited to you.
It’s 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
IPF progression
IPF is a progressive disease, which means that it gets worse over time. However, there is no way of predicting how fast this will happen. The rate at which IPF will progress can also change over time. Some patients may notice few changes in their symptoms, while other patients experience a faster worsening of their symptoms. It is not known why some people’s disease progresses faster than others.3
Some research suggests that half of people with IPF live for about 3 to 5 years after diagnosis. However, these estimates were based on information provided before new treatments for IPF were available.1,3
With new antifibrotic treatments for IPF, people with IPF can live longer. Although the new treatments cannot reverse the scarring on the lungs, they may slow the progression of the disease.5,12,13
Monitoring the progression of IPF
Your doctor, supported by a wider treatment team of experienced specialists, will use your medical history, a physical examination and a combination of different tests, you'll be familiar with from diagnosis, working closely with you, to find out how your disease is progressing. Your doctor will then tailor the best treatment plan for you.3,14
Unlike many other chronic diseases, there are no clear-cut "stages" of IPF. Everyone experiences it differently so it’s best to speak to your doctor who can advise you based on your individual situation.14
Acute exacerbations
In the early stages of IPF, you may only notice small changes in your breathing. As the disease progresses, there is a bigger risk of complications or an acute exacerbation occurring. An acute exacerbation is a sudden and rapid worsening of symptoms.1,15
If you have a high temperature you may think you have a chest infection or are experiencing a flare-up of your condition but it's important you seek medical advice anyway. Acute exacerbations can be life-threatening and for this reason, it is vital that you work with your treatment team to do everything you can to look after your lungs.15
The worsening of a disease/condition over time
A formal documentation when someone else is given the responsibility to manage your affairs and make certain decisions on your behalf
Refers to the lungs
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
A type of high blood pressure that affects the blood vessels to the lungs and the right side of the heart15
Therapy that provides relief from symptoms to help patients live more comfortably with their disease13
Administration of oxygen as a medical intervention11
A small plastic tube or prongs that fit in the nostrils to deliver supplementary oxygen11
Listening to and playing music as a therapy which aims to ease the symptoms of those living with IPF
A sleep disorder characterised by breathing that repeatedly stops and starts during sleep12
A specialist who helps someone recover or live with their symptoms more easily
A machine that removes other gases from the air to provide oxygen for oxygen therapy11
An education and exercise program designed to improve the quality of life for people with lung conditions16
A physician specialised in lung problems (also known as a respirologist)
A product that gives protection against a specific infection
A test used to monitor oxygen levels in a patient’s blood, usually with a non-invasive sensor11
A breathing technique to help control breathlessness and reduce anxiety17
A disease that affects only a small percentage of the population
Something that is associated with an increased risk of disease or infection
A technique that helps to reduce stress and anxiety by helping to understand and manage your emotions
Therapies used alongside conventional treatments that help treat symptoms and may improve overall physical and mental wellbeing
A medical study that investigates how safe and effective a new therapy or technique is for treating a certain disease
A disease or condition that occurs at the same time as another disease or condition
A device to deliver compressed air to improve sleep in people with obstructive sleep apnoea6
A disease where a waxy substance (plaque) builds up inside the coronary arteries, which supply oxygen-rich blood to the heart muscle7
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
Refers to the heart, and blood vessels
A type of medication that aims to slow the scarring and stiffening of lungs to slow disease progression2
Tiny air sacs in the lungs where the exchange of oxygen and carbon dioxide to and from the blood takes place
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
Techniques that involve breathing in a certain way to control breathlessness and strengthen your lungs
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
A lung condition where the air sacs within the lungs (alveoli) become damaged5
Extreme weariness resulting from exertion or illness
Of unknown cause
Rapid and uncontrolled breathing
A disease where there is progressive scarring or thickening of the lungs without a known cause4
The surgical removal of cells or tissue samples from the lung for examination by a pathologist10
The lung tissue becomes thickened and stiff
A test that uses a type of X-ray that produces multiple, detailed images of areas inside the body4
A burning sensation in the chest, which can spread to the throat, along with a sour taste in the mouth
Where inflamed tissue is replaced with scar tissue, making it thicken and become stiffer4
A symptom where the ends of the fingers become wider and rounder8
A digestive disease where stomach acid moves up out of the stomach and irritates the lining of the food pipe (oesophagus)9
A physician specializing in the management of diseases of the digestive system
An event characterized by sudden, severe worsening of symptoms or an increase in disease severity1
If your symptoms worsen suddenly, keep calm and alert someone nearby so they can contact your treatment team. If your symptoms are severe, you or someone close to you should contact the emergency services
Mental wellbeing
Many people with IPF can find it hard to manage their thoughts or mental wellbeing. Thoughts can involve feeling down or worried and struggling to cope with the diagnosis. Looking after your mental health is just as important as looking after your physical health. Speak to your treatment team if you feel like you need help managing your mental health.
Comorbidities, or co-existing conditions
You may have other ongoing health conditions that should be managed alongside your IPF
Many people with IPF also have other health conditions which are often called comorbidities, or co-existing conditions. IPF may or may not influence these but managing them can be just as important as managing IPF itself.
To manage co-existing conditions people receive medications and care in addition to their treatment for IPF. Some of the most common co-existing conditions people with IPF may have include: 16,17
- Chronic obstructive pulmonary disease (COPD): A lung disease that causes the flow of air into and out of the lungs to be restricted. This makes it harder to breathe
- Gastroesophageal reflux disease (GERD): A stomach condition that causes stomach acid move up into the food pipe (oesophagus), leading to heartburn
- Obstructive sleep apnea: A condition where breathing stops and starts during sleep
- Pulmonary hypertension: High blood pressure in the blood vessels that supply blood to the lungs
- Heart conditions such as coronary heart disease, where there is a build-up of plaque in the blood vessels in the heart
- Other conditions: Diabetes, underactive thyroid and hiatal hernia
It is important to remember that every person with IPF is different and may experience some, or none, of these co-existing conditions. Always be aware of any changes in your condition or symptoms and discuss them with your treatment team. They will be able to guide you on your condition and treatments.
I think many of the things one does to stay healthy with IPF are the same things that one does to stay healthy in general
Coping with a serious illness
Am I alone in how I feel about my diagnosis?
Although IPF is rare, you are not alone. There are other patients with IPF, like you, all over the world, who are working through the emotional and mental wellbeing challenges of living with IPF. In fact, anywhere from 10-60 people in every 100,000 people are diagnosed with IPF every year in Europe and North America.1,18
The worsening of a disease/condition over time
A formal documentation when someone else is given the responsibility to manage your affairs and make certain decisions on your behalf
Refers to the lungs
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
A type of high blood pressure that affects the blood vessels to the lungs and the right side of the heart15
Therapy that provides relief from symptoms to help patients live more comfortably with their disease13
Administration of oxygen as a medical intervention11
A small plastic tube or prongs that fit in the nostrils to deliver supplementary oxygen11
Listening to and playing music as a therapy which aims to ease the symptoms of those living with IPF
A sleep disorder characterised by breathing that repeatedly stops and starts during sleep12
A specialist who helps someone recover or live with their symptoms more easily
A machine that removes other gases from the air to provide oxygen for oxygen therapy11
An education and exercise program designed to improve the quality of life for people with lung conditions16
A physician specialised in lung problems (also known as a respirologist)
A product that gives protection against a specific infection
A test used to monitor oxygen levels in a patient’s blood, usually with a non-invasive sensor11
A breathing technique to help control breathlessness and reduce anxiety17
A disease that affects only a small percentage of the population
Something that is associated with an increased risk of disease or infection
A technique that helps to reduce stress and anxiety by helping to understand and manage your emotions
Therapies used alongside conventional treatments that help treat symptoms and may improve overall physical and mental wellbeing
A medical study that investigates how safe and effective a new therapy or technique is for treating a certain disease
A disease or condition that occurs at the same time as another disease or condition
A device to deliver compressed air to improve sleep in people with obstructive sleep apnoea6
A disease where a waxy substance (plaque) builds up inside the coronary arteries, which supply oxygen-rich blood to the heart muscle7
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
Refers to the heart, and blood vessels
A type of medication that aims to slow the scarring and stiffening of lungs to slow disease progression2
Tiny air sacs in the lungs where the exchange of oxygen and carbon dioxide to and from the blood takes place
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
Techniques that involve breathing in a certain way to control breathlessness and strengthen your lungs
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
A lung condition where the air sacs within the lungs (alveoli) become damaged5
Extreme weariness resulting from exertion or illness
Of unknown cause
Rapid and uncontrolled breathing
A disease where there is progressive scarring or thickening of the lungs without a known cause4
The surgical removal of cells or tissue samples from the lung for examination by a pathologist10
The lung tissue becomes thickened and stiff
A test that uses a type of X-ray that produces multiple, detailed images of areas inside the body4
A burning sensation in the chest, which can spread to the throat, along with a sour taste in the mouth
Where inflamed tissue is replaced with scar tissue, making it thicken and become stiffer4
A symptom where the ends of the fingers become wider and rounder8
A digestive disease where stomach acid moves up out of the stomach and irritates the lining of the food pipe (oesophagus)9
A physician specializing in the management of diseases of the digestive system
An event characterized by sudden, severe worsening of symptoms or an increase in disease severity1
Whether you have just been diagnosed with IPF or have been living with it for a longer period, it is important that you have a clear plan in place for the future of your treatment and overall wellbeing.
The worsening of a disease/condition over time
A formal documentation when someone else is given the responsibility to manage your affairs and make certain decisions on your behalf
Refers to the lungs
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
A type of high blood pressure that affects the blood vessels to the lungs and the right side of the heart15
Therapy that provides relief from symptoms to help patients live more comfortably with their disease13
Administration of oxygen as a medical intervention11
A small plastic tube or prongs that fit in the nostrils to deliver supplementary oxygen11
Listening to and playing music as a therapy which aims to ease the symptoms of those living with IPF
A sleep disorder characterised by breathing that repeatedly stops and starts during sleep12
A specialist who helps someone recover or live with their symptoms more easily
A machine that removes other gases from the air to provide oxygen for oxygen therapy11
An education and exercise program designed to improve the quality of life for people with lung conditions16
A physician specialised in lung problems (also known as a respirologist)
A product that gives protection against a specific infection
A test used to monitor oxygen levels in a patient’s blood, usually with a non-invasive sensor11
A breathing technique to help control breathlessness and reduce anxiety17
A disease that affects only a small percentage of the population
Something that is associated with an increased risk of disease or infection
A technique that helps to reduce stress and anxiety by helping to understand and manage your emotions
Therapies used alongside conventional treatments that help treat symptoms and may improve overall physical and mental wellbeing
A medical study that investigates how safe and effective a new therapy or technique is for treating a certain disease
A disease or condition that occurs at the same time as another disease or condition
A device to deliver compressed air to improve sleep in people with obstructive sleep apnoea6
A disease where a waxy substance (plaque) builds up inside the coronary arteries, which supply oxygen-rich blood to the heart muscle7
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
Refers to the heart, and blood vessels
A type of medication that aims to slow the scarring and stiffening of lungs to slow disease progression2
Tiny air sacs in the lungs where the exchange of oxygen and carbon dioxide to and from the blood takes place
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
Techniques that involve breathing in a certain way to control breathlessness and strengthen your lungs
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
A lung condition where the air sacs within the lungs (alveoli) become damaged5
Extreme weariness resulting from exertion or illness
Of unknown cause
Rapid and uncontrolled breathing
A disease where there is progressive scarring or thickening of the lungs without a known cause4
The surgical removal of cells or tissue samples from the lung for examination by a pathologist10
The lung tissue becomes thickened and stiff
A test that uses a type of X-ray that produces multiple, detailed images of areas inside the body4
A burning sensation in the chest, which can spread to the throat, along with a sour taste in the mouth
Where inflamed tissue is replaced with scar tissue, making it thicken and become stiffer4
A symptom where the ends of the fingers become wider and rounder8
A digestive disease where stomach acid moves up out of the stomach and irritates the lining of the food pipe (oesophagus)9
A physician specializing in the management of diseases of the digestive system
An event characterized by sudden, severe worsening of symptoms or an increase in disease severity1
Talking about and making preparations for all of these things can sometimes be stressful and tiring. Don’t feel that you have to do it all at once. Care planning is an ongoing activity and sometimes takes time.
The worsening of a disease/condition over time
A formal documentation when someone else is given the responsibility to manage your affairs and make certain decisions on your behalf
Refers to the lungs
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
A type of high blood pressure that affects the blood vessels to the lungs and the right side of the heart15
Therapy that provides relief from symptoms to help patients live more comfortably with their disease13
Administration of oxygen as a medical intervention11
A small plastic tube or prongs that fit in the nostrils to deliver supplementary oxygen11
Listening to and playing music as a therapy which aims to ease the symptoms of those living with IPF
A sleep disorder characterised by breathing that repeatedly stops and starts during sleep12
A specialist who helps someone recover or live with their symptoms more easily
A machine that removes other gases from the air to provide oxygen for oxygen therapy11
An education and exercise program designed to improve the quality of life for people with lung conditions16
A physician specialised in lung problems (also known as a respirologist)
A product that gives protection against a specific infection
A test used to monitor oxygen levels in a patient’s blood, usually with a non-invasive sensor11
A breathing technique to help control breathlessness and reduce anxiety17
A disease that affects only a small percentage of the population
Something that is associated with an increased risk of disease or infection
A technique that helps to reduce stress and anxiety by helping to understand and manage your emotions
Therapies used alongside conventional treatments that help treat symptoms and may improve overall physical and mental wellbeing
A medical study that investigates how safe and effective a new therapy or technique is for treating a certain disease
A disease or condition that occurs at the same time as another disease or condition
A device to deliver compressed air to improve sleep in people with obstructive sleep apnoea6
A disease where a waxy substance (plaque) builds up inside the coronary arteries, which supply oxygen-rich blood to the heart muscle7
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
Refers to the heart, and blood vessels
A type of medication that aims to slow the scarring and stiffening of lungs to slow disease progression2
Tiny air sacs in the lungs where the exchange of oxygen and carbon dioxide to and from the blood takes place
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
Techniques that involve breathing in a certain way to control breathlessness and strengthen your lungs
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
A lung condition where the air sacs within the lungs (alveoli) become damaged5
Extreme weariness resulting from exertion or illness
Of unknown cause
Rapid and uncontrolled breathing
A disease where there is progressive scarring or thickening of the lungs without a known cause4
The surgical removal of cells or tissue samples from the lung for examination by a pathologist10
The lung tissue becomes thickened and stiff
A test that uses a type of X-ray that produces multiple, detailed images of areas inside the body4
A burning sensation in the chest, which can spread to the throat, along with a sour taste in the mouth
Where inflamed tissue is replaced with scar tissue, making it thicken and become stiffer4
A symptom where the ends of the fingers become wider and rounder8
A digestive disease where stomach acid moves up out of the stomach and irritates the lining of the food pipe (oesophagus)9
A physician specializing in the management of diseases of the digestive system
An event characterized by sudden, severe worsening of symptoms or an increase in disease severity1
Some days will be tougher than others but remember that the good days will help you cope with the bad ones. Always try and find the time to make memories with your loved ones as these will keep you going and help your mental wellbeing
Key Takeaways
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IPF is a rare, progressive disease that causes a build-up of scarring in the lungs resulting in shortness of breath
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IPF shares symptoms with many other diseases, meaning diagnosis can take a while
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Antifibrotic treatments aim to slow disease progression by slowing down the process that causes scarring in the lungs
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If you're caring for a loved one with IPF, remember that there are a lot of supports and resources out there for you and that you are not alone.
Life is a roller coaster with IPF enjoy the highs and hold out your hand for the lows
Show references
Hide references
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1.
Martinez F, Collard H, Pardo A, et al. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers. 2017; 3:17074.
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2.
Upagupta C, Shimbori C, Alsilmi R, Kolb M. Matrix abnormalities in pulmonary fibrosis. Eur Respir Rev. 2018;27(148):180033.
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3.
Meltzer E, Noble P. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis. 2008;3(1).
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4.
Fernandez I, Eickelberg O. New cellular and molecular mechanisms of lung injury and fibrosis in idiopathic pulmonary fibrosis. The Lancet. 2012;380(9842):680-688.
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5.
Woodcock H, Maher T. The treatment of idiopathic pulmonary fibrosis. F1000Prime Rep. 2014;6.
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6.
Vainshelboim B, Oliveira J, Izhakian S, et al. Lifestyle Behaviors and Clinical Outcomes in Idiopathic Pulmonary Fibrosis. Respiration. 2017;95(1):27-34.
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7.
Senanayake S, Harrison K, Lewis M, et al. Patients' experiences of coping with Idiopathic Pulmonary Fibrosis and their recommendations for its clinical management. PLoS One. 2018;13(5): e0197660.
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8.
Robalo-Cordeiro C, Campos P, Carvalho L, et al. Idiopathic pulmonary fibrosis in the era of antifibrotic therapy: Searching for new opportunities grounded in evidence. Rev Port Pneumol. 2017;23(5):287–293.
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9.
van Manen MJ, Geelhoed JJ, Tak NC, Wijsenbeek MS. Optimizing quality of life in patients with idiopathic pulmonary fibrosis. Ther Adv Respir Dis. 2017;11(3):157–169.
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10.
Wells AU, et al. Interstitial lung disease guideline. Thorax. 2008; 63: v1-v58.
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11.
Raghu G, et al. Nintedanib and Pirfenidone. New Antifibrotic Treatments Indicated for Idiopathic Pulmonary Fibrosis Offer Hopes and Raises Questions. Am J Respir Crit Care Med. 2015; 191:252-4.
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12.
Fisher M. et al. Predicting life expectancy for pirfenidone in idiopathic pulmonary fibrosis. J Manag Care Spec Pharm. 2017; 23: S17-S24.
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Fleetwood K, et al. Systematic review and network meta-analysis of idiopathic pulmonary fibrosis treatments. J Manag Care Spec Pharm. 2017; 23: S5-S16.
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14.
Raghu G, et al. An official ATS/ERS/JRS/ALAT statement: Idiopathic pulmonary fibrosis: Evidence-based Guidelines for Diagnosis and Management. Am J Respir Crit Care Med. 2011; 183: 788–824.
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Kim DS. Acute exacerbations in patients with idiopathic pulmonary fibrosis. Respir Res. 2013; 14:86.
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Raghu G, et al. Comorbidities in idiopathic pulmonary fibrosis patients: a systemic literature review. Interstitial and orphan lung diseases. Eur Respir J. 2015; 46: 1113-1130.
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Oldham JM, Collard HR. Comorbid Conditions in idiopathic Pulmonary Fibrosis: Recognition and Management. Front Med (Lausanne). 2017; 4:123.
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18.
Data on file. Boehringer Ingelheim. Worldwide prevalence, 2016.
Also in this section
Conditions with pulmonary fibrosis
Pulmonary fibrosis can take many years to be detected and multiple tests can be required to rule out other conditions
Co-existing conditions
You may have other ongoing health conditions, but these can be managed alongside your condition with pulmonary fibrosis.
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What is pulmonary fibrosis?
Taking care of yourself
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