Idiopathic pulmonary fibrosis (IPF) is a lung disease that results from the formation of scar tissue deep inside the lungs. This scarring gets progressively worse, making it more difficult to breathe and to keep adequate levels of oxygen in the bloodstream. The main symptom is shortness of breath, which can also lead to fatigue and other problems.

Why Is Early Treatment Important?

IPF is a progressive disease, which means that symptoms worsen over time. It cannot be cured, and the scarring that exists cannot be reversed or removed. Early treatment aimed at managing symptoms and slowing disease progression is the best way to maintain quality of life.

What Types of Treatments Are Available?

Although no cure has yet been discovered for IPF, there are numerous treatment options available. Most of these are aimed at supporting a healthy lifestyle and managing symptoms.

Nonmedical Interventions

Lifestyle changes and other nonmedical treatments can help you stay healthier and improve the quality of your life with IPF. Here are some general 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, and could keep you off the transplant list if you need a lung transplant.

Stop Smoking

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 inflicting even more damage.

Get Annual Vaccinations

Talk to your doctor about yearly flu and pneumonia vaccines. These can help protect your lungs from infection and further damage.

Monitor Your Oxygen Levels

Use an at-home pulse oximeter and try to maintain an oxygen saturation 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 patients and reduce shortness of breath at rest and during exercise. Key features include:

  • breathing and conditioning exercises
  • stress and anxiety management
  • emotional support
  • nutritional counseling
  • patient education

Medical Interventions

Medical treatment options include drugs, such as suppressants and antioxidants, that work against possible causes of IPF, including:

  • corticosteroids like prednisone, which act to reduce inflammation and suppress the immune reaction
  • proton pump inhibitors, which block the creation of acid in the stomach (the inhalation of excess stomach acid is thought to contribute to IPF)
  • antioxidants like N-acetylcysteine, which may help protect lung tissue from oxidative damage
  • anti-fibrotic drugs like pirfenidone and nintedanib, which can slow the progression of lung tissue damage
  • immune suppressants, such as mycophenolate mofetil and azathioprine, which can treat autoimmune disorders and help prevent the rejection of a lung transplant

Your doctor may recommend other treatment options too. An oxygen tank 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 and help prevent right-sided heart failure.

You may even be a candidate for a lung transplant. Though once reserved for younger recipients, lung transplants are now commonly offered to patients over 65 who are otherwise healthy.

Experimental Treatments

There are several new potential treatments for IPF under investigation. Headed up by the National Heart, Lung, and Blood Institute, the Idiopathic Pulmonary Fibrosis Network (IPFNet) is a group of premier medical institutions working together to find a cure.  

Support Groups

Not to be overlooked, a support system can make a big difference in your quality of life, and your outlook on living with IPF. The Pulmonary Fibrosis Foundation has a searchable database of local support groups, in addition to a number of online communities. These types of resources are invaluable as you come to terms with your diagnosis and the changes it can bring to your life.