Idiopathic Pulmonary Fibrosis, or IPF, is a rare and serious condition that affects the delicate tissues of the lungs. Normally lung tissue is soft and flexible, making it easy to breathe, but IPF causes lung tissue to stiffen and become scarred. Once this happens, the tissue can’t recover.
What causes IPF?
There is no known cause. Several factors are seen as potential triggers, including viral infections, gastroesophageal reflux (‘heartburn”), exposure to occupational dusts and chemical fumes, and smoking. Occasionally more than one family member develops IPF, suggesting genes may play a part.
Most people develop IPF when they are over 60, and it is extremely rare in people under 50. Men are affected more commonly than women.
What are the symptoms?
IPF symptoms include breathlessness, a chronic dry or hacking cough, discomfort in the chest, finger “clubbing” (a change in the shape of the fingernails), fatigue, loss of appetite and weight loss. Because these symptoms are not specific to IPF, it can be difficult to diagnose. Symptoms often only become evident when the disease has already caused substantial lung damage.
How many people have IPF?
There is no clear data on this for New Zealand, but a reasonable estimate is around 5 cases per 10,000 people – meaning most GPs may encounter a new case about once per decade of full-time work.
What’s the outlook for people with IPF?
Some people with IPF stay stable for many years, while others deteriorate quickly. Unfortunately, IPF is a terminal disease with an average life expectancy of about 3-5 years following an early diagnosis.
What happens as IPF progresses?
People can experience more mucus secretions, interrupted sleep, and increased breathlessness. It is important for people to discuss a plan with a doctor or respiratory specialist to get clear guidelines on when and how to seek help.
Having support from a team of health professionals, such as physiotherapists, occupational therapists, social workers, dieticians and clinical psychologists can help make things easier.
What treatments are available?
Treatment for IPF is a complex area, and it’s important that people get the right advice from their healthcare team. While there is no cure, there are two drugs licensed for use in New Zealand (pirfenidone and nintedanib) that may slow the progress of IPF.
Lung transplantation can be an effective treatment in the right circumstances, but it is a major undertaking and only works for certain people. Transplants become much more difficult in patients over 60, and it is highly unlikely that a patient over 65 would be offered transplantation for IPF, as it is unlikely to succeed.
Are there ways to manage your IPF symptoms?
There are several ways that IPF sufferers can maintain or improve their quality of life. These include stopping smoking, gentle exercise, good diet, and getting plenty of rest.
Want to know more?
For more information and resources about IPF, visit asthmafoundation.org.nz/your-health/otherrespiratory-conditions/about-ipf
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