If you're diagnosed with idiopathic pulmonary fibrosis (IPF) your healthcare team will talk to you about treatment.
You can get treatment to:
- relieve symptoms as much as possible
- help you keep fit and control your breathing
- slow down how fast your condition progresses
Things you can do to help manage IPF
There are things you can do to stay healthy and relieve symptoms as much as possible if you have IPF.
Do
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get help to quit smoking if you smoke
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eat a healthy, balanced diet
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get the flu vaccine
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get the pneumococcal vaccine
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try to keep away from people with colds and chest infections
Pulmonary rehabilitation
Pulmonary rehabilitation is used for many long-term lung conditions.
It is a an exercise and education programme to help you:
- understand and manage your condition and symptoms
- increase your confidence and ability to cope with your symptoms
- improve your muscle strength and fitness
- improve your quality of life
Pulmonary rehabilitation involves:
- education about pulmonary fibrosis
- supervised physical activity
- breathing exercises
- advice on nutrition
- emotional support
- talking to other people who have the same condition
The courses are usually held in your local hospital, health centre or leisure centre.
Learning to control your breathing
Everyday activities can make you feel short of breath. For example, getting dressed.
Feeling short of breath may make you feel panicked or anxious. This can make your shortness of breath worse.
When you feel short of breath try to:
- stop speaking and moving
- give yourself time to recover your breath
- relax or distract yourself by focusing on a picture or a view from the window
Learning to control your breathing
Oxygen therapy
IPF can cause levels of oxygen in your blood to fall. This may make you feel more short of breath.
Some people need:
- long-term oxygen therapy - oxygen you take for at least 16 hours a day
- ambulatory oxygen therapy - oxygen you use when you walk or are active in other ways
Your specialist doctor will assess your oxygen needs and give you advice on how much oxygen to take. Use your oxygen the way your doctor tells you to.
There are different types of devices that can be used to give you oxygen. It is important to use oxygen therapy devices safely. Do not smoke, vape or be near an open flame with an oxygen therapy device.
Contact your healthcare team or oxygen supplier if you are struggling with your oxygen therapy.
Looking after your mental health
Looking after your mental health is important when living with IPF.
To mind your mental health, try to:
- have a daily routine
- talk to friends, family or someone you trust
- control your breathing - this can reduce stress and improve your mood
Talk to your doctor if you are finding it hard to cope with IPF.
Your healthcare team may recommend you:
- get talk therapy
- try mindfulness or relaxation tips
They can also refer you to mental health services that can help you cope better.
Looking after your mental health
Medicines
There are 2 medicines that can help slow down how quickly IPF progresses in some people. These medicines cannot treat symptoms, stop or reverse IPF.
They are:
- pirfenidone
- nintedanib
Pirfenidone is a tablet you take 3 times a day with food and water. Your dose is increased over 3 weeks.
Nintedanib is a 150mg or 100mg capsule that you take 2 times a day, 12 hours apart. You take the capsules whole (not chewed or crushed) with food and water.
Do not take pirfenidone or nintedanib with grapefruit juice.
Before starting pirfenidone or nintedanib, you will have blood tests to check your liver and kidney function and full blood count.
Side effects
Side effects of pirfenidone and nintedanib can include:
- feeling sick (nausea) or getting sick (vomiting)
- diarrhoea
- weight loss
- a skin rash
They can also cause serious liver damage. This risk is thought to be low but your care team will regularly check if it has affected your liver.
Urgent advice: Contact your doctor immediately if
you have any of the following symptoms of liver damage:
- yellowing of the eyes or skin (jaundice)
- loss of appetite
- itchy skin
- your pee (urine) turns a darker colour
- feeling tired
- pain in the upper right side of your tummy (abdomen)
Lung transplant
If your condition continues to get worse despite treatment, your doctor may recommend a lung transplant.
The decision to have a transplant is 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
- if a donor lung is available
A lung transplant can help people with IPF live longer. But it's a major surgery (operation) that puts a great strain on the body.
Not many people with IPF are suitable for a transplant and there are not many donor lungs.
Palliative care
Palliative care is a way to:
- understand how a condition affects your life
- plan support to improve your quality of life and help your family
Your healthcare team may use palliative care at any stage of a serious illness. It can be a part of your care plan along with other medical treatment.
You could ask your doctor about specialist palliative care if you:
- have shortness of breath that is getting worse and affects your daily life
- are admitted into hospital frequently
- have symptoms that are difficult to control, such as a cough or tiredness (fatigue)
- have feelings of anxiety, depression or being overwhelmed
- want to discuss your future care wishes and plans
Your healthcare team or palliative care team can support you to:
- plan for a time when you cannot make decisions about treatment
- talk to your family or other important people about your wishes
- record your wishes in a document
This is called advance healthcare planning.
You can get palliative care through:
- a hospital or hospice (in-patient care)
- your community
You do not have to be dying to get palliative care. But palliative care can include end-of-life care.
Outlook for IPF
Treatments can help slow down IPF. But it usually gets worse over time. It can be hard to know how quickly this happens. It is different for everyone.
Your healthcare team will check how your symptoms change over time.
Other health problems can sometimes develop.
For example:
- chest infections
- pulmonary hypertension - high blood pressure in the blood vessels that supply your lungs
- heart failure
You can ask your doctor or healthcare team for more information on the outlook of your condition.