Sadly, there are no medications that can cure IPF or undo its damage to the lung. But there is good news. Two medications can slow the speed of scarring in the lungs for people with IPF. And there are some things you can do to help stay healthy.
Anti-Scarring (anti-fibrotic) medicines
Pirfenidone and nintedanib are the only two anti-scarring medications approved by Health Canada to treat IPF. Both slow the disease. Both have side effects.
- Some people who take pirfenidone have upset stomach. Some have skin rash, especially if they are in the sun.
- Some people who take nintedanib have diarrhea. Some have upset stomach.
You will want to work with your health care team to decide which one is best for you. The decision will be based on your needs, your lung health and other medications you might be taking.
There are other medicines that you might hear about for IPF. Some are used during a flare up of symptoms. Others have little benefit so are rarely if ever used.
- Corticosteroid pills (for example, Prednisone©) work by calming your immune system. This can lessen the swelling in your lungs during a flare-up of IPF.
- You may be given it during a flare-up of your symptoms.
- Corticosteroids can be harmful in patients with IPF that have scarring that is permanent or slowly worsening. They have many side effects when taken for long periods of time.
- N-Acetylcysteine pills or inhalation mist (for example, NAC, Mucomyst©) is an antioxidant. It was used in patients with IPF.
- A large clinical trial published in May 2014 showed that NAC does not slow the worsening of IPF. Side effects of these medicines include nausea and vomiting.
- It is not recommended to treat IPF.
Ask your healthcare team if you have questions about any medications you are taking or are curious about. They can help you make the best decisions for your health.
Antioxidant: something that prevents or slows cell damage caused by “free radicals”, or unstable atoms. The body makes free radicals to help fight infection or other causes of damage.
Pulmonary rehabilitation is an exercise and education program designed for people with lung disease. A pulmonary rehabilitation program can help you:
- Breathe in a way to help you manage your day-to-day activities.
- Exercise so you have more energy to do the things important to you.
- Learn how to cope with IPF from other people with lung disease.
Pulmonary rehabilitation programs in Canada are usually meant for people with COPD. Most can take patients with IPF but there might be a wait list.
Speak with your healthcare team about pulmonary rehabilitation.
The scarring in your lungs can prevent oxygen from being absorbed into your body. Low oxygen levels can make you feel short of breath and tired. If your blood oxygen levels are too low, your doctor may prescribe home oxygen therapy. Oxygen therapy can help you be less breathless so you can:
- Feel less tired.
- Do more things important to you.
- Sleep better.
There is testing needed to find out if oxygen therapy is needed for you. This might mean a special blood test or a walking test with a probe that measures your oxygen levels while you walk. Some people with IPF need oxygen all the time. Others only need it for exercise or sleeping.
Oxygen is in the air we breathe. You need a certain amount in your blood to be healthy. Some people might worry they will become addicted to their oxygen therapy. But this cannot happen. Like other medicines, it helps your body work best, and you feel better.
Oxygen therapy is not for everyone with IPF. But for those people who have very low blood oxygen levels, it can make a big difference in their lives.
Lung transplant is not right for everyone, but it may be an option for some people. It is important to work with your health care team to see if lung transplant is an option for you.
There is a wait list for people who qualify for a lung transplant. The wait can be long, and the surgery can be risky. People who get transplants must take several anti-rejection medicines for the rest of their lives.
Lung transplants are only done is specialized hospitals. For a list of hospitals in your province click here.
Anti-rejection medicines: medicines that help keep your immune system from seeing the new lungs as a ‘foreign agent’. If people with transplants do not take these medicines, their body will attack the new lungs. This is called rejection.
Reviewed by the Canadian Thoracic Society's Pulmonary Fibrosis Clinical Assembly