To manage your IPF, both drug treatments and other treatments are recommended. You should also have regular check-ups and medical examinations.

As soon as you are diagnosed with IPF, you should be offered psychological support. You should also get in touch with patient groups, which can support you at all stages of your disease.

Figure 10: IPF management (adapted from: ¹)

Figure 10: IPF management (adapted from: ¹)

Pharmacological treatments

There are currently no curative treatments for people living with IPF. However, two drugs which have been approved by the European Medicines Agency are able to slow down the disease progression. ²⁶ ²⁷ One of these drugs contains the active substance (ingredient) nintedanib and the other contains the active substance pirfenidone.

You can find more information about these two drugs at the following links:

Both of the approved drugs are antifibrotic drugs. They help to prevent tissue scaring and have been shown to reduce the loss of lung function by about 50 % in 1 year ²⁶ ²⁷ ²⁸. (This was measured by spirometry – a method of measuring breathing capacity.)

Recent IPF guidelines do not favour one drug over another; they provide a conditional recommendation for the use of either nintedanib or pirfenidone in the treatment of IPF ²⁸. This means that your doctor will evaluate which drug to prescribe on a patient-by-patient basis.

You should always consult your doctor to discuss treatment options, as every patient is different and no two treatment plans are alike. Furthermore, each European country has their own national official guidelines on IPF and its treatment. Payment and reimbursement criteria for these drugs may also differ between countries. A final decision on your treatment should be taken together with your doctor as many factors need to be taken into consideration.

If you have any further questions about possible pharmaceutical treatments and/ or if you notice and want to report any side effects, talk to your doctor or pharmacist.

 

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Clinical trials

A clinical trial is a study which tests the effectiveness and safety of a new therapy on humans. There have been several clinical trials into treatments designed to treat IPF – you should check with your doctor if there are any ongoing trials you may be able to take part in. You can also find wider information on on-going clinical trials using ClinicalTrials.gov (worldwide) or the EU Clinical Trials Register.

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Non-pharmacological treatments

While a lung transplant is the only effective cure for someone with IPF, there are several non-pharmacological treatments which can help improve your quality of life. ²⁹ They and other treatments can help lessen the burden on you, your family and your carers

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Pulmonary rehabilitation

Pulmonary rehabilitation includes different activities designed to relieve the symptoms of IPF and improve your overall quality of life. This can include:

  • exercise training;
  • nutritional counselling; or
  • learning breathing strategies.

Pulmonary rehabilitation can be particularly effective in:

  • alleviating symptoms;
  • increasing how much you can exercise (which in turn can reduce your tendency to psychosocial issues such as anxiety or depression); and
  • reducing how long you may need to spend in hospital. ³⁰
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Oxygen therapy

Since IPF makes the oxygen in your lungs less able to travel into the bloodstream and around your body, you may suffer from abnormally-low levels of oxygen in the blood in your arteries. This is called hypoxemia. You may have hypoxemia while you are:

  • resting;
  • sleeping (nocturnal hypoxemia); or
  • exercising.

To deal with the effects of hypoxemia, you may be given extra oxygen to improve some symptoms of IPF and your overall quality of life.³¹ Supplemental oxygen can help to reduce shortness of breath and improve your ability to perform everyday tasks. At first, you may only need extra oxygen during exercise and while sleeping, but as the disease progresses you may need oxygen all the time to keep the oxygen levels in your blood at a healthy level. There are several different ways you can take in extra oxygen (oxygen therapy), through:

  • a concentrator;
  • face mask;
  • oxygen cylinders (compressed gas); and
  • liquid oxygen
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Psychological support

Psychological support is a very important part of living with IPF. You can discuss this with your doctor and they may be able to offer it to you as soon as you are diagnosed with IPF. This will help you accept the disease, relieve physical and emotional suffering and improve your quality of life.

Psychological support can help you accept the treatment and the drug’s side effects as well. In an advanced stage, psychological support can help you in end-of-life planning and choosing hospice care or the comfort of your home.

It is important to involve your friends and family members in the psychological support programme as they can help you manage your IPF.

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Palliative and end-of-life care

Learning about IPF and its treatment can improve your quality of life. Since IPF is a progressive disease with no known cure, you can often feel better if you can discuss end-of-life issues with knowledgeable professionals. When possible, your close family members should be included in these discussions.

Palliative care can be used for symptom control and is part of a holistic approach to managing IPF.³² It aims to provide you with relief from both the physical pain, and the broader stresses and problems, associated with your condition. Palliative care includes things like:

  • advance care planning; and
  • patient and carer education.³³

Palliative care may also include physical, mental, social, and spiritual activities – depending on your needs and preferences. The goal is to improve your quality of life, and that of your family or carers.

No matter what stage of disease progression you are at, palliative care should be an integral part of your overall care. It is essential for IPF patients nearing the end of their life.²⁶ You should speak with your doctors to discuss how palliative care may help you and your family.

 

Learning to manage breathlessness

Palliative care can also help you manage episodes of breathlessness. It is very important that you learn how to minimise, cope and manage breathlessness. Learning to manage and live with breathlessness can make a big difference to your quality of life. Your emotions can affect your breathing and being anxious about your breathlessness can make your breathing even worse. Relaxation can help to reduce breathlessness and it is useful for relieving anxiety, so try to find what relaxes you.

Managing your cough

Palliative care can involve treatments to help reduce the symptoms of cough as well. Being able to manage your cough plays an important role in managing IPF because the cough can make you feel isolated and embarrassed unless you can control it. This can make it difficult to interact and communicate with other people. In fact, managing your cough is a significant factor in maintaining your overall quality of life. 

 
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Lung transplantation

Your doctor may refer you to a lung transplant centre, where you will be evaluated as a candidate for transplant of one or both of your damaged lungs from a donor. This is the only surgical intervention procedure which can:

  • reverse the progression of IPF;
  • improve your quality of life; and
  • improve your life expectancy.³⁴

Some 30% of lung transplants worldwide are performed on IPF patients. ³⁵

Unfortunately, only 5% of all IPF patients qualify for a lung transplant, since the criteria for selection as a viable transplant candidate are quite strict. These consider a range of factors such as a patient’s;

  • age;
  • general health;
  • likely outcome following the procedure;
  • severity of IPF; and
  • progression of IPF.

The criteria are established by local or national health authorities and you should speak to your doctor about whether or not you may qualify. ²⁶

If you do qualify, it is important to remember that there are risks associated with the procedure, including serious complications, infection, or rejection of the new lung or lungs.

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