Idiopathic pulmonary fibrosis (IPF) is a lung condition that scars your lungs and makes it difficult for you to breathe. It’s the most common type of pulmonary fibrosis.
What does IPF stand for?
• idiopathic = there is no known cause
• pulmonary = it affects your lungs
• fibrosis = the name of the scar tissue in your lungs
IPF is a progressive condition. This means it gets gradually worse over time. The scar tissue in the lungs cannot be repaired by the body or any drugs, and there is currently no treatment that stops or reverses the scarring.
Current treatment focuses on slowing the rate of scarring in the lungs and controlling symptoms. Some people respond well to treatment and find their symptoms remain the same for many years. For others, the symptoms get worse more quickly.
It’s difficult to predict how quickly IPF will develop because it varies a lot from person to person. There are no clear stages of IPF, though some people may talk about the mild, moderate, or severe disease. Everyone is different – talk to your specialist doctor about your own situation.
How does IPF affect breathing?
Scar tissue causes the lungs to become stiffer and to lose their elasticity. This stops them from working properly: they’re less able to inflate and transfer oxygen from the air you breathe into your body.
Each time you breathe in, you take in air through your nose and mouth, down through your throat, and into your windpipe (trachea). Your windpipe splits into two main tubes, the right and left bronchi, which supply your lungs with air.
The main bronchi divide into gradually smaller airways called bronchioles. Bronchioles have many small air sacs (alveoli) at their ends. Inside the air sacs, oxygen moves across paper-thin walls to tiny blood vessels and into your blood. The air sacs also exchange waste gas (carbon dioxide) from your blood ready for you to breathe it out.
If you have IPF, scarring affects the air sacs, eventually limiting the amount of oxygen that gets into the blood. With less oxygen in the blood, you can get breathless from everyday activities like walking.
What causes IPF?
Idiopathic means the cause is not known, but researchers now believe that the body creates fibrosis (scarring) in response to an injury to the lung.
The initial injury to the lungs might be from:
- acid reflux from the stomach
- viruses – in some studies, IPF has been linked to certain viruses, including herpes, hepatitis C and the Epstein Barr virus, which causes glandular fever
- environmental factors – breathing in certain kinds of dust from wood, metal, textiles or stone, or from cattle or farming
- cigarette smoke exposure.
It is not known if these factors directly cause IPF.
Some people may also get IPF if it’s in their family history. But this link is only found in a minority of cases. According to a 2021 survey by the British Thoracic Society, 5.7% of patients were found to have a known relative with IPF.
Who gets IPF?
IPF is more common in people over the age of 40. It is also more common in men than it is in women.
Symptoms of IPF
The first symptom you might notice is getting out of breath when you’re doing everyday activities like walking up the stairs.
The main symptoms of IPF are:
- a cough that doesn’t go away
- feeling tired all the time
- clubbing of the fingertips or toenails.
If you have IPF you may feel short of breath a lot, and not just when you’re moving about. If you feel breathless you should visit your GP.
A cough that doesn’t go away (usually a dry cough ) and feeling tired are other symptoms of IPF.
You may also notice clubbing of your fingertips or toenails. This means the shape of your fingers or toes might appear like a drumstick, with the tips being larger around the nails. The base of the nails may also feel spongy. Having this doesn’t mean you have IPF as it can occur in other conditions too. But check with your GP if you notice it developing.
How is IPF diagnosed?
A team of several experts (called a multi-disciplinary team), often at specialist hospitals, will collect information about you before making a diagnosis. This is because there are many types of pulmonary fibrosis.
IPF is one of many different types of pulmonary fibrosis. This means diagnosing IPF will involve a lot of tests to rule out the other types of pulmonary fibrosis. Your doctor is likely to listen to your chest and ask about your family, medication, work, and medical history. You may also have blood tests to rule out other causes of your symptoms.
If you have IPF, doctors listening to your chest can often hear crackles in your lungs that sound like Velcro opening.
You may need tests such as:
- a CT scan to produce a detailed image of your lungs
- breathing tests, such as spirometry, to measure how well your lungs are working
- a bronchoscopy to see inside the lung and get samples of the lung tissue – this is done using a small tube with a camera
- a lung biopsy, where tissue or cells are taken from the lung for testing.
IPF sometimes shows up as a honeycomb pattern on the lungs in a CT scan. The image shows lots of empty pockets or bubbles appearing where more solid-looking tissue would normally appear.
Being diagnosed with IPF might feel overwhelming at first. It can help to talk to your family and friends about it. You may want to read our information about looking after your mental health too.
Treating and managing IPF
Treatment for IPF aims to slow the rate of scarring in the lung and treat the symptoms.
Medication to slow the scarring
The importance of self-care
You should be treated at a specialist clinic, or by a team that comes to your local hospital. You should have regular appointments every 3-6 months.
If the IPF gets worse in spite of treatment, a few people might have the option of having a lung transplant. Your specialist team should discuss lung transplant with you within six months of your diagnosis if it’s suitable.
Treating the symptoms rather than the cause of a disease is called best supportive care. Your doctors may discuss pulmonary rehabilitation to help with your symptoms of breathlessness and oxygen therapy to help maintain oxygen levels to other organs.
Your doctors may also discuss palliative care – this is a plan to help you through any stage of a life-limiting illness and focuses on improving symptoms of the condition.
Your doctor will look for and treat problems that could be making your coughing worse, such as heartburn (acid reflux) or a blocked nose. Your doctor may also be able to refer you to a physiotherapist who can suggest ways to manage your coughs, such as sipping water or swallowing.
Severe coughing can often be suppressed with low doses of liquid morphine. It is usually given by syringe into the mouth and works quite quickly. There are potential side effects of morphine, so your doctor will discuss these with you before you decide if you want to take it.
If you find it difficult to cough up mucus, you may be prescribed medicine such as to help loosen and thin mucus in the lungs. Some people find it helps their cough, but others may experience side effects such as stomach discomfort, trapped wind, or feeling sick.
To help you cope when you get out of breath, pulmonary rehabilitation is an important treatment and you’ll learn breathing techniques so that you feel more in control.
IPF can cause the level of oxygen in your blood to fall. This can make you feel more breathless. If this happens, you may be prescribed home oxygen treatment to allow you to be more active.
For more severe symptoms of breathlessness, which can sometimes make you very anxious, your doctor might recommend low doses of a short-acting medication such as lorazepam which can help take away sensations of severe breathlessness and panic. These tablets dissolve under your tongue.
Monitor the effectiveness of your therapy
Tell your doctor if you have symptoms of gastro-oesophageal reflux, such as heartburn, indigestion, or a sour taste at the back of your mouth. There’s evidence this may make IPF worse and make a cough worse. You can have treatments that reduce the amount of acid your stomach makes, such as omeprazole. Liquid antacids such as Gaviscon can also be helpful in preventing reflux at night.
Sometimes acid reflux is caused by a Hiatus Hernia, and this can be more common in those with IPF.
You might feel anxious or even depressed when living with a long-term, progressive condition. Talking about your concerns with your medical team, friends and family can be helpful. But some people may also need medication or other therapies to help improve their mood. Don’t be afraid to ask if you or your family member needs help to improve their mental well-being.
It’s important to look after yourself to stay as well as possible if you have IPF.
If you smoke, the best thing you can do for your health is to quit.
Exercise as often as you can to stay fit. This can also help to improve your mood.
Eat a healthy, well-balanced diet.
Try to get enough sleep
Try to keep away from people who are ill with coughs or colds.
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