Idiopathic Pulmonary Fibrosis
Treatment for Idiopathic Pulmonary Fibrosis
There is no cure for IPF and there are currently no procedures or medications that can remove the scarring from the lungs. Treatments are used to slow progression of the lung scarring, and may not necessarily lessen the symptoms of cough and breathlessness. Pirfenidone (Esbriet) and nintedanib have both been shown to slow the progression of IPF; however, some patients cannot take these medications due to their side effects. These medications are not approved for use in other interstitial lung diseases.
Some additional medications can be used to improve the symptoms of IPF, such as shortness of breath and cough. This includes treatment of gastroesophageal reflux with anti-acid therapy and treatment of shortness of breath with opioids (a class of medications that includes morphine). Other treatments include supplemental oxygen, pulmonary rehabilitation (exercise training, education and support for people with chronic lung disease), and lung transplant.
Understand your IPF
It is important to work with your family doctor or respirologist (lung specialist) to find the best treatment for you. Not all medications will work for everyone. Your doctor knows your medical history and will be able to determine the best treatment for you.
The following can help you prepare for your appointment with your doctor:
- Prepare a written list of the questions you want to ask. Put the most important questions at the top of your list.
- Take notes as the doctor is answering your questions. If you don't understand the doctor's answers, ask for more details.
- Show the doctor a written list of all your symptoms. Be honest.
- Bring along all of your medications (including herbal remedies).
- If you're not sure you're taking your medications correctly, ask your doctor to review it with you.
- Bring a friend or relative to your appointment to help you remember details and take notes for you.
- Ask your doctor about programs in your community that help people with IPF and other lung diseases. Ask about joining a pulmonary rehabilitation program.
- Ask your doctor about a local support group for IPF. Obtaining psychosocial support, education and learning coping skills through patient support groups can be very valuable for the patients and their caregivers.
- Discuss the course of your disease with your doctor at follow-up visits and ask your doctor to help you develop goals for your care.
Your health-care team can help you learn more about IPF and how to cope with it. Local home-care services can be very important in providing you with essential services in your home by liaising with you doctors when the need arises. People on your IPF health-care team may include:
- doctor(s), including your family doctor and a respirologist (lung specialist)
- certified respiratory educator, a health professional with special training in lung disease
- respiratory therapist, nurse, or cardiopulmonary physiotherapist who leads your pulmonary rehabilitation program
- certified respiratory educator from the Lung Association's free helpline (call 1-866-717-2673)
- occupational therapists through your doctor’s clinic, pulmonary rehabilitation program or Homecare who can provide you with suggestions on how to live better with breathlessness.
IPF is an irreversible, progressive disease. So it is important to learn good coping skills and educate yourself about the disease as much as possible.
- Quitting smoking is the most important thing you can do to help slow down the progression of any lung disease.
- Get a flu shot each year.
- Ask your doctor about getting a pneumonia shot.
- Keep away from people with chest infections and colds.
- Wash your hands properly and often.
- Stay as fit as you can.
The scarring in the 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 supplemental oxygen (home oxygen). This helps to reduce breathlessness and can help you be more active. Some people with IPF may need oxygen therapy all the time, while others may only need it during exercise or sleep.
If your doctor has prescribed supplemental oxygen, it is important to use it as prescribed. Many patients fear that they will become “addicted” to supplemental oxygen. Supplemental oxygen is not addictive and your body needs a proper amount to maintain normal body functions. People who take supplemental oxygen must continue taking their other medications.
Not everyone who has IPF needs to be on oxygen. Generally, oxygen therapy can help only people with very low blood-oxygen levels (hypoxemia). To find out if you need supplemental oxygen, speak to your doctor or certified respiratory educator.
There are no medications that cure IPF or decrease the amount of scarring in the lungs. Some medications help slow the progression of pulmonary fibrosis.
It is very difficult to find medications that will work for everyone with IPF. Medications for IPF have side effects in some patients, so it is important to talk to your doctor about the possible risks.
As with any medication for any condition, you should talk to your doctor about different treatment options and ask about medication side effects.
Pirfenidone is an anti-scarring (anti-fibrotic) medication that slows the progression of IPF. Some patients taking pirfenidone have side effects, most commonly stomach upset and skin rash, particularly with exposure to sun. Pirfenidone has been approved by Health Canada for the treatment of mild to moderate IPF.
Nintedanib is an anti-scarring (anti-fibrotic) medication that slows progression of IPF. Some patients taking nintedanib have side effects, most commonly including diarrhea.
Corticosteroid pills (for example, Prednisone©) can reduce inflammation in your lungs by suppressing your immune system. Corticosteroids are only used in patients with IPF who have an acute exacerbation of their lung fibrosis, and can be harmful in patients with IPF that have scarring that is stable or slowly worsening.
N-Acetylcysteine (oral or aerosolized)
N-acetylcysteine (NAC, mucomyst) is an antioxidant that has frequently been used in patients with IPF. A large clinical trial published in May 2014 showed that NAC does not slow progression of IPF.
Other medications have been studied and previously used in IPF, including azathioprine, cyclophosphamide, and others. These medications are not recommended in IPF as they have no proven benefit in IPF and are associated with significant side effects.
Pulmonary rehabilitation is an exercise and educational program designed just for patients with lung disease. Pulmonary rehabilitation can help in teaching patients with IPF how to breathe more efficiently and to perform their activities of daily living with less breathlessness.
Most pulmonary rehabilitation programs are designed for people with COPD (emphysema and chronic bronchitis), although the majority of pulmonary rehabilitation programs in Canada accept and welcome patients with IPF.
Read more about pulmonary rehabilitation and find a program near you.
Lung transplant is not right for everyone, but it may be a treatment option for some people. It is important to work with your doctor to see if lung transplant is an option for you.
If a person qualifies for a lung transplant, they are put on a waitlist for organ donation. The wait can be long and the surgery can be risky. People who get transplants must take several anti-rejection medications (immune-suppressants) for the rest of their lives.