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Idiopathic pulmonary fibrosis (IPF) is a chronic condition, which usually gets worse over time. Currently there is no cure for IPF. Management is based on:
A multidisciplinary team will be in charge of your treatment.
The treatment you receive will depend on the severity of your illness, how quickly it is advancing, and your own personal preferences.
A multidisciplinary team will be in charge of your treatment. This will include respiratory doctors, nurses, radiologists and physiotherapists. You will need regular checkups and tests to help monitor your condition.
As well as any treatment you may receive there is a lot you can do to keep yourself as healthy, active and as positive as possible.
If you get breathless just doing normal everyday activities or while you are resting, your doctor may recommend that you are assessed for:
Your cough can have a large impact on daily life. Its effects include tiredness, shortness of breath, sleep disturbance, anxiety, dizziness, vomiting, incontinence and social exclusion. However, there are things that can be done to minimise your cough and the effect it has on you.
Many people with IPF experience tiredness to some degree. Sometimes it can feel overwhelming and leave you with little energy for everyday activities. Tiredness can be associated with low oxygen levels in your blood, and your doctor may refer you for an oxygen assessment to see whether you might benefit from oxygen therapy.
There is not yet a cure for IPF, but there are now medicines that can slow the advance of the disease. Two medicines, pirfenidone and nintedanib, have recently been approved for use in Scotland for people with mild to moderate IPF. However, they are not suitable for everyone and the benefits of treatment need to be balanced against the possible side effects.
A lung transplant may be an option for those who are physically healthy enough to undergo the operation. There is some evidence that a lung transplant may improve survival and quality of life in people with IPF.
However, a lung transplant is not suitable for everyone with IPF and there are many factors that need to be considered before this can be recommended.
On top of your usual symptoms, you may experience a flare-up (or exacerbation) of your IPF. It is important that you receive treatment as soon as possible to reduce further damage to your lungs.
Contact your specialist team urgently if you notice an increase in:
The quicker a flare-up is managed the better the outcome.