Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease that involves the buildup of scar tissue deep inside the lungs, between the air sacs. This damaged lung tissue becomes stiff and thick, making it difficult for your lungs to work efficiently. The resulting difficulty in breathing leads to lower levels of oxygen in the bloodstream.
What to Expect
When faced with a new diagnosis, it’s natural to have lots of questions. You’re probably wondering what you can expect in terms of your prognosis and life expectancy.
Because IPF is a chronic, progressive disease, you’ll have it for the rest of your life. Even so, the prognosis for people with IPF can vary greatly. While some people may become sick very quickly, others may progress more slowly over the course of several years. In general, there are several symptoms that are common among people with IPF.
Shortness of Breath
With IPF, your lungs aren’t working as they should, and your body responds to the lack of oxygen in your bloodstream by making you feel like you need to breathe more. This triggers a shortness of breath, especially during periods of increased activity. As time goes on, you’ll probably start to feel this same breathlessness even during periods of rest.
Low levels of oxygen in the blood can tire you out, leaving you feeling exhausted and generally unwell. This feeling of tiredness may be worsened if you seek to avoid physical activity because you don’t want to feel short of breath.
A dry, hacking cough is a frequent complaint among IPF patients. You may experience “coughing fits,” where you can’t control your cough for several minutes. This can be very tiring, and can make you feel like you cannot get a breath at all. You may be more prone to coughing fits when:
- you’re exercising or performing any sort of activity that leaves you short of breath
- you’re feeling emotional, laughing, crying, or speaking
- you’re in environments with higher temperature or humidity
- you’re in close proximity or come into contact with pollutants or other triggers like dust, smoke, or strong odors
Pulmonary hypertension is high blood pressure in the lungs. This can happen because of the reduced oxygen level in your blood. Because hypertension makes your heart work harder than normal, it can lead to heart failure if oxygen levels don’t improve.
It can be hard to eat well with IPF. Chewing and swallowing food can make breathing more difficult, and eating complete meals can make your stomach feel uncomfortably full and increase the workload of your lungs. Because of this, it’s important to eat nutrient-dense food rather than junk food. You may find it helpful to eat smaller amounts of food more frequently rather than three, larger meals as well.
As your condition advances, you’ll be at increased risk for life-threatening complications, including:
- heart attack and stroke
- pulmonary embolism (blood clots in the lungs)
- respiratory failure
- heart failure
- serious infections in the lungs
Life expectancy can also vary in patients with IPF. Your own life expectancy is likely to be influenced by the progression of your disease and the intensity of your symptoms. In general, the life expectancy with IPF is about three to five years. You may be able to increase that time, and improve the quality of your life, by talking to your doctor about ways to manage your symptoms and your disease progression.
There’s no cure for IPF, but research through the National Heart, Lung, and Blood Institute is working to raise awareness of the disease, raise money for research, and conduct clinical trials to search for a life-saving cure.