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.
In general, the life expectancy with IPF is about three years. 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 outlook and life expectancy.
With IPF, your lungs aren’t working as they should, and your body responds to the lack of oxygen in your bloodstream by causing you to breathe more. This triggers shortness ofx 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.
A dry, hacking cough is one of the most frequent symptoms among those with IPF, affecting nearly 80 percent of individuals. You may experience “coughing fits,” where you can’t control your cough for several minutes. This can be very tiring and may make you feel like you can’t 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
Low levels of oxygen in the blood can tire you out, leaving you feeling exhausted and generally unwell. This feeling of tiredness may worsen if you avoid physical activity because you don’t want to feel short of breath.
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. Weight loss can also occur because your body spends a lot of calories working to breathe.
Because of this, it’s important to eat nutrient-dense food rather than junk food. You may also find it helpful to eat smaller amounts of food more frequently rather than three larger meals each day.
Pulmonary hypertension is high blood pressure in the lungs. This can happen because of the reduced oxygen level in your blood. This type of high blood pressure makes the right side of your heart work harder than normal, so it can lead to right-sided heart failure and enlargement if oxygen levels don’t improve.
As the disease 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
- lung cancer
Life expectancy can vary in people with IPF. Your own life expectancy is likely to be influenced by your age, the progression of the disease, and the intensity of your symptoms. You may be able to increase the three-year estimation, 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 increase awareness of the disease, raise money for research, and conduct clinical trials to search for a life-saving cure.
Newer anti-scarring medications, such as pirfenidone (Esbriet) and nintedanib (OFEV), have been shown to slow the progression of the disease in many people. These medications haven’t improved life expectancy, however. Reseachers continue to look for combinations of medications that may further improve outcomes.
Because IPF is a chronic, progressive disease, you’ll have it for the rest of your life. Even so, the outlook for people with IPF can vary greatly. While some may become sick very quickly, others may progress more slowlyå over the course of several years.
In general, getting support from a variety of services including palliative care and social work is important. Pulmonary rehabilitation may improve your quality of life by helping you manage your breathing, diet, and activity.