Idiopathic pulmonary fibrosis (IPF) is a lung disease that results from the formation of scar tissue deep inside the lungs. The scarring gets progressively worse. This makes it more difficult to breathe and keep adequate levels of oxygen in the bloodstream.
Ongoing low oxygen levels cause a variety of complications throughout the body. The main symptom is shortness of breath, which can lead to fatigue and other problems.
IPF is a progressive disease. This means symptoms worsen over time. There is no cure, and scarring can’t be reversed or removed. Getting treatment early to manage symptoms and slow disease progression is the best way to maintain quality of life.
There is no cure for IPF. However, there are numerous treatments. Most of these aim to support a healthy lifestyle and manage symptoms.
Lifestyle changes and other nonmedical treatments can help you stay healthier and improve your quality of your life. Here are some recommendations.
Lose weight or maintain a healthy weight
Talk to your doctor about healthy ways to reduce or manage your weight. Being overweight will only add to your breathing difficulties. Losing weight and maintaining a healthy weight can also improve your quality of life.
Smoking cigarettes is one of the worst things you can do to your lungs. Now, more than ever, it’s critical to stop this habit from causing even more damage.
Get annual vaccinations
Talk to your doctor about yearly flu and updated pneumonia and whooping cough (pertussis) vaccines. These can help protect your lungs from infection and further damage.
Monitor your oxygen levels
Use an at-home pulse oximeter to monitor your oxygen saturation. Often the goal is to have oxygen levels at or above 90 percent.
Participate in pulmonary rehabilitation
Pulmonary rehabilitation is a multifaceted program that has become a staple of IPF treatment. It aims to improve everyday life for people with IPF as well as to reduce shortness of breath both at rest and with exercise.
Key features include:
- breathing and conditioning exercises
- stress and anxiety management
- emotional support
- nutritional counseling
- patient education
Medical treatment options include drugs that work against possible causes of IPF. These drugs can include suppressants and antioxidants, along with:
- corticosteroids, like prednisone, which act to reduce inflammation and suppress the immune reaction
- proton pump inhibitors, which block the stomach from producing acid (inhalation of excess stomach acid is linked and may contribute to IPF)
- antioxidants, like N-acetylcysteine, which may help protect lung tissue from oxidative damage
- antifibrotic drugs, like pirfenidone and nintedanib, which can slow the progression of lung tissue damage
- immune suppressants, such as mycophenolate and azathioprine, which can treat autoimmune disorders and help prevent the rejection of a transplanted lung
Your doctor may also recommend other treatment options. Oxygen treatment might help you breathe easier, especially during exercise and other activities.
Additional oxygen can reduce problems related to low levels of oxygen in the blood. It can also help prevent right-sided heart failure.
You may even be a candidate for a lung transplant. Lung transplants were once reserved for younger recipients. But now they’re commonly offered to people over age 65 who are otherwise healthy.
There are several new potential treatments for IPF under investigation. You have the option of applying to a variety of clinical trials that are looking to find new ways to prevent, diagnose, and treat a wide range of lung diseases, including IPF.
You can find clinical trials at CenterWatch, which tracks major research on searchable topics. The National Heart, Lung, and Blood Institute provides information about how clinical trials work, risks and benefits, and more.
There are also support systems. These can make a big difference in your quality of life and outlook regarding living with IPF. The Pulmonary Fibrosis Foundation has a searchable database of local support groups along with several online communities.
These resources are invaluable as you come to terms with your diagnosis and the changes it can bring to your life.
While there is no cure for IPF, there are treatment options available that can help you manage your symptoms and improve your quality of life. These include drugs, medical interventions, and lifestyle changes.
In addition, there are support systems that can provide you with information on living with IPF.