Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease with no known cause and currently no cure. However, medications and other therapies can often help manage symptoms or slow the progression of the disease.
Each person’s experience with IPF is different. Sometimes the disease advances rapidly, despite treatment. For others, treatment can help individuals experience relatively stable periods between phases when symptoms grow worse.
IPF treatments include medications, nondrug therapies, lifestyle changes, and in the most serious cases, lung transplantation.
A variety of prescription medications are available to treat certain aspects of IPF. Some help reduce inflammation in your lungs, while others help slow the loss of lung function caused by IPF’s main symptom: the thickening of deep lung tissue.
The medications used to treat IPF include the following:
Prednisone (Rayos) is a type of corticosteroid. It helps reduce inflammation by mimicking the activity of cortisol, a natural hormone that controls inflammation when your body is under stress.
Azathioprine (Azasan, Imuran) is an immunosuppressant drug sometimes prescribed to treat autoimmune diseases, such as rheumatoid arthritis. It’s also given to patients who receive organ transplants to help prevent rejection of the new organs. This drug can sometimes cause serious side effects. It may be given to people with IPF in combination with prednisone for short-term use.
Nintedanib (OFEV) was approved by the U.S. Food and Drug Administration (FDA) in 2014 to treat IPF. The medication interferes with the scarring of lung tissue. One of the main characteristics of IPF is a decline in forced vital capacity, a term that describes how much air you can exhale from your lungs after taking a deep breath. Nintedanib may reduce that decline.
Like nintedanib, pirfenidone (Esbriet) was also approved in 2014 by the FDA to combat the decline in forced vital capacity.
Cyclophosphamide (Cytoxan) is a chemotherapy drug used to treat cancer. However, it’s been found to help treat IPF in some cases.
Mycophenolate mofetil (CellCept) is another drug meant to prevent organ rejection in transplant patients. It’s sometimes prescribed with corticosteroids.
All of these medications are powerful drugs that carry risks of potentially serious side effects. Talk with your doctor about the risks and benefits of any prescribed medication. And be sure to know what side effects are possible and how to recognize them quickly.
As your lung function decreases, you may benefit from therapies that complement your medications. These include:
This type of rehab is helpful for people with IPF, lung cancer, or chronic obstructive pulmonary disease (COPD). Pulmonary rehab includes education sessions, during which you’ll learn about IPF and how to manage it. You’ll get exercise training and nutrition counseling. Rehab will also teach you about energy conservation and breathing techniques to help you deal with reduced lung function.
Most pulmonary rehab programs include two to three sessions per week for 4 to 12 weeks. Each person has a program tailored to their condition, age, gender, overall health, and other factors.
Another important feature of pulmonary rehab is emotional support and counseling. Dealing with any type of chronic disease can be emotionally stressful. Pulmonary rehab includes therapy to help you deal with your fears and concerns about IPF or feelings of depression or anxiety. Some sessions are done in a group setting, so you can talk with people who are going through the same experiences. Coping skills others have learned may help you.
IPF limits the ability of your lungs to supply blood with oxygen. Without a steady supply of oxygen, your organs and muscles will suffer. Oxygen therapy can often help. Individuals on oxygen therapy inhale oxygen from a portable tank through a tube and prongs that fit into their nostrils.
You may need oxygen therapy only while you sleep or when you’re physically active. In more serious cases of IPF, you may need oxygen therapy 24 hours a day.
Lifestyle changes are also important when managing IPF. The most crucial is to quit smoking if you’re a smoker. If you’ve tried to quit unsuccessfully before, understand that most people try several times before making a permanent change. Talk with your doctor about medications, nicotine replacement products, and smoking cessation programs in your community.
Maintaining a healthy weight and exercising regularly are helpful in easing the burden on your lungs. Exercising safely and using breathing strategies learned in pulmonary rehabilitation are also important steps to keep taking after rehab ends.
Other important steps to discuss with your doctor include:
- getting an annual flu shot to help prevent an infection
- taking cough medicine as needed to prevent a cough and the stress coughing can cause to your lungs
- taking vitamin D and calcium supplements to combat bone loss that sometimes happen to people taking corticosteroids
- taking a medication, such as a proton pump inhibitor, to combat gastroesophageal reflux disease (GERD), which is common among people with IPF
Future of Treatment
Researchers are working to better understand IPF and what causes it. They are also trying to come up with new ways to treat it.
One promising treatment may come from blocking molecules involved with excessive collagen production. So far, it’s been used in lab mice.
Another exciting approach is stem cell therapy. Stem cells can turn into many different types of cells. Researchers are looking at ways to use a person’s own healthy stem cells to replace damaged lung tissue cells. This therapy isn’t available to the general public, but clinical trials are underway exploring the potential of stem cells as an IPF treatment.