Idiopathic pulmonary fibrosis (IPF)
Idiopathic pulmonary fibrosis (IPF) causes progressive scarring of the lungs, making breathing increasingly difficult. Symptoms, diagnosis and treatment are explained here.
What is idiopathic pulmonary fibrosis (IPF)?
Idiopathic Pulmonary Fibrosis, or IPF, is a serious lung disease which affects the delicate tissues around the alveoli (air sacs) within the lungs.
Normally the lung tissue is soft and flexible which means it is easy to breathe, but in someone with IPF the lung tissue becomes stiff, thickened and scarred, making it difficult to breathe. The process of the scarring of the lungs is called fibrosis, and once it has occurred the tissue does not recover. The scar tissue also reduces the delivery of oxygen into the blood, where it is needed to be transported to the rest of the body.
- Idiopathic means a condition without any known cause
- Pulmonary relates to the lungs or respiratory system
- Fibrosis is a thickening and scaring of tissues
There is no known cause for IPF, however we do know:
- it is not an infection, nor is it infectious - you cannot pass it on to other people
- it is not a form of cancer
- it is not a form of cystic fibrosis
Who gets IPF and what are the symptoms?
Most people develop their IPF symptoms after the age of 60 years, and the disease is uncommon in people below the age of 50 years. Men are affected more commonly than women.
The most common symptoms are:
- Breathlessness, especially with activity, such as walking up hills or stairs
- A chronic, dry or hacking cough
- Finger “clubbing”, which is a change in the shape of the fingernails
- Fatigue and weakness
- Loss of appetite and/or rapid weight loss
The above symptoms are not specific to IPF, meaning that there are other conditions that can have the same symptoms. This means that IPF can be difficult to diagnose in the early stages of the disease. The symptoms often only become more evident when the disease has already caused some damage to the lungs.
What causes IPF?
Although the cause of IPF is not known, certain risk factors could be related to the condition, such as smoking or exposure to a variety of occupational dusts.
A small number of cases are linked to genetic factors; this is usually associated with a family history of having IPF.
How is IPF diagnosed?
As IPF is relatively uncommon (a reasonable estimate would be around 5 cases per 10,000 individuals), and as the symptoms are non-specific, it can take many months for the diagnosis to be made.
A diagnosis is usually made by an expert respiratory specialist, after a patient is referred by their general practitioner (GP), because of unexplained symptoms or an abnormal chest X-ray.
Most people will have the following investigations:
- History and physical examination: A respiratory specialist will assess a patient’s symptoms, rate of change, as well as take a detailed medical history to identify known causes of pulmonary fibrosis.
- Chest X-ray: This may show signs of scarring, even early on, so some people will be diagnosed by an abnormal chest X-ray before they develop symptoms.
- Lung function tests: These are breathing tests to show how well your lungs are working. They are used to monitor both how severe the disease is and its progression.
- Pulse oximeter: This test indicates the amount of oxygen carried in the blood.
- Blood tests: These are usually done to exclude other causes of lung disease.
- CT scan: This is a special type of X-ray scan that produces 3-dimensional, high-resolution pictures of the inside of the lungs.
In very rare circumstances, some people may require a:
- Bronchoscopy: This involves passing a small flexible telescope down into the lungs to collect samples.
- Surgical lung biopsy: This is done under general anesthesia to make a firm diagnosis of IPF.
How is IPF treated and managed?
There is currently no cure for IPF, but there are treatments which may slow down the progression of the disease, help with symptoms, and maintain or improve a patient’s quality of life.
Medication
Medication
In New Zealand there are two anti-fibrotic medicines: pirfenidone and nintedanib. These help to reduce the risk of exacerbation (acute flare-up) in patients, as well as help to reduce the rate at which scarring worsens, and therefore disease progression. These medicines are subsidised by Pharmac for patients who meet certain eligibility criteria, and must be prescribed by a respiratory specialist.
Physical activity
Physical activity
Through regular physical activity and being active, our muscles become stronger and more resistant to fatigue. With practice and training you can learn to perform tasks more efficiently, which means you use less oxygen carrying them out. A formal pulmonary rehabilitation programme may be preferred for some patients because it allows for observation during exercise and can be tailored to a patient’s specific needs.
Nutrition
Nutrition
It is important to eat a balanced and nutritious diet, and to monitor changes in weight. While specific research on nutrition in pulmonary fibrosis is limited, the same principles for other lung conditions, like COPD (chronic obstructive pulmonary disease), are applied. Patients who are overweight or underweight may be referred to a dietitian for specialist advice.
Emotional support and counselling
Emotional support and counselling
For many patients and their families, a diagnosis of IPF can be difficult to come to terms with, and anxiety and depression are commonly experienced. Talk to your GP if you are feeling anxious or depressed. It may help to see a clinical psychologist for counselling and support.
Oxygen therapy
Oxygen therapy
A specialist doctor may prescribe oxygen to patients with low blood oxygen levels, so the body can met its oxygen needs, especially during physical activity.
General measures
General measures
It is important that you do not smoke and that you avoid second-hand smoking.
Ensure your annual flu vaccination is up to date. Talk to your specialist and healthcare team about which vaccinations they recommend to support your immune system.
Clinical trials
Clinical trials
Your specialist may refer you to enrol in a clinical trial, if appropriate.
Lung transplant
Lung transplant
Lung transplantation may be suitable for younger (< 65 years) and fitter patients with IPF, in the right circumstances. Very few patients are actually suitable and will benefit. Having a lung transplant is a serious undertaking – it is a major operation and there are risks during and after transplantation. Post-operatively, anti-rejection drugs need to be started and continued for the rest of a patient’s life.
Community health team
Community health team
Having support from a team of different health professionals can help to make things easier. Talk to your GP for referral to the following health professionals to help: physiotherapists, occupational therapists, dietitians, social worker, clinical psychologists.
What is the life expectancy for someone with IPF?
IPF is a serious, long-term lung condition that tends to get worse over time.
The speed at which the condition progresses is variable, so each person’s individual experience and prognosis after receiving a diagnosis of IPF is different.
Some stay stable for many years, while others may worsen over months. But it affects everyone differently, so it's hard to predict exactly what your journey will look like.
The following can influence how the condition progresses and a patient’s prognosis:
- How early the condition is diagnosed
- Whether antifibrotic treatment is used
- Age and overall health of the patient
Some people with IPF may experience an acute exacerbation which is a sudden worsening of their symptoms, usually over one month of less. The cause is not always known.
Recovery after an exacerbation can take many months and often people do not return to the same level of health they had before.
Estimating life expectancy for an individual when first diagnosed is difficult and usually it becomes more clear after a period of monitoring.