Key facts
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IPF is a rare, progressive disease that causes a build-up of scarring in the lungs
resulting in shortness of breath - IPF shares symptoms with many other conditions, meaning diagnosis can take a while
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Antifibrotic treatments help to reduce further scarring, which may slow down the
worsening of IPF
IPF stands for Idiopathic Pulmonary Fibrosis, a rare condition that affects about 1 in 3,800 Canadians. In IPF, the exact cause of this scarring is unknown, which means the pulmonary fibrosis is idiopathic. However, certain factors, such as cigarette smoking, some viral infections, genetics and GERD (gastro-oesophageal reflux disease) may increase the risk of IPF.
In someone with IPF, the lungs become scarred, thickened and stiff. This makes it harder for oxygen to enter the blood and results in shortness of breath.
Did you know IPF is idiopathic?
“Idios” means one’s own and “pathos” means suffering or disease. Together this means that the disease occurs from within the body and is not connected to any outside cause. This is usually the diagnosis when doctors rule out all possible causes.
IPF progression
IPF is a progressive disease, which means that it gets worse over time. However, there is no way of predicting how fast this will happen. The rate at which IPF will progress can also change over time. Some patients may notice few changes in their symptoms, while other patients experience a faster worsening of their symptoms. It is not known why some people’s disease progresses faster than others.
Click here for a more detailed description of this graph.
IPF progression over time
A line graph showing four ways that IPF can progress over time, from onset of disease, to onset of symptoms, to diagnosis, to death.
Line A shows rapid disease progression (from onset of disease to death within 1 year).
Line B shows periods of relative stability punctuated by acute worsening or exacerbations (from onset of disease to death within 3 years). Line B has a star icon at the points of acute worsening or exacerbations, at just over 1 year and at about 2.5 years.
Line C shows gradual disease progression (from onset of disease to death within 6 years).
Line D shows stable disease progression (from onset of disease to diagnosis in 6 years).
Some research suggests that untreated people with IPF live for an average of 3 to 5 years after diagnosis. However, these estimates were based on information provided before any treatments for IPF were available.
Although treatments cannot reverse the scarring on the lungs, they can reduce further scarring, which may slow down the worsening of the disease.
“Pulmonary fibrosis or IPF can be a difficult condition to diagnose because the symptoms can apply to other conditions. And you hear people complaining about how they've been misdiagnosed by their GPs, et cetera. So there seems to be a requirement for a little bit more education...to be more aware of pulmonary fibrosis. When I was diagnosed, when my friend was diagnosed, I didn't know what pulmonary fibrosis was.” – Paul, ON
(@ 2 years post diagnosis)*
Symptoms of IPF
Common signs and symptoms of IPF:
Shortness of breath, especially with mild physical activity
A dry, hacking cough that doesn't get better
“Velcro-like" crackles heard when the doctor listens to the lungs with a stethoscope
“Velcro-like" crackles heard when the doctor listens to the lungs with a stethoscope
In addition to the symptoms above, make sure to monitor your IPF symptoms for acute exacerbations. An acute exacerbation is a sudden and rapid worsening of symptoms. During the early stages of IPF, you may only notice small changes in your breathing. But as the disease progresses, there is a bigger risk of complications or an acute exacerbation occurring.
If you have a high temperature, you may think you have a chest infection or are experiencing a flare-up of your condition, but it's important you seek medical advice anyway. Acute exacerbations can be life threatening, and for this reason, it is vital that you work with your healthcare team to do everything you can to look after your lungs.
Important
If your symptoms worsen suddenly, keep calm and alert someone nearby so they can contact your healthcare team. If your symptoms are severe, you or someone close to you should contact the emergency services.
Your doctor, supported by a wider healthcare team of experienced specialists, will use your medical history, a physical examination and a combination of different tests to find out how your disease is progressing. Your doctor will then tailor the best treatment plan for you.
Unlike many other chronic diseases, there are only proposed stages of IPF, such as “mild,” “moderate,” “severe,” or “early” and “advanced” stages. Everyone experiences it differently, so it’s best to speak to your doctor who can advise you based on your individual situation. Make sure to ask as many questions as you need to in order to get a full understanding of your condition and situation.
“At first my attitude was I'm not going to deal with that right now so I'll just put it up in a box up on the shelf, but eventually you have to take the box out of the shelf and open the box...[I] read a little more on the actual disease and the variances of what was out there. What is life expectancy? What is this? How much? That's when I was like, okay, now to take it down off the shelf and look at what's in the box because I have to be able to deal with this one way or another. The sooner I deal with it, the easier it will be for my daughter to deal with it because she's going to have to deal with my end result, so the better I can do while I'm here, the better for her.” – Jenny, ON
(@ 1 year post-diagnosis)*
* Name has been changed for privacy.
