IPF
TREATMENT
OPTIONS

Treatments for idiopathic pulmonary fibrosis (IPF)

Idiopathic pulmonary fibrosis (IPF) is a chronic condition that scars the lungs, and makes breathing difficult. It is a progressive disease, which means it gets worse over time. Once your lungs are scarred by IPF, this can’t be reversed. There are options that can help manage its impact. Discuss your treatment options together with your doctor and decide on an approach which is right for you.

To get help talking to your doctor about IPF and your options view the discussion guide.

Pulmonary rehabilitation

Pulmonary rehabilitation is a programme of exercise, training and education designed to maximise the use of your lungs. Over time, the exercises and techniques will help you manage the day-to-day symptoms of IPF and stay as active as possible.

Lung transplant

A lung transplant is the only cure for IPF at the moment, but only a few people are eligible for a transplant. There are risks involved, and importantly, you will need to be matched with a suitable donor, which can take a long time. Your doctor will be able to help explain how the donor process works.

IPF medicines

There are medicines which have been developed over the past few years to treat IPF. While it is not completely understood how these medications work, studies show they can help reduce inflammation and fibrosis, which in turn may slow down the progress of the disease but are not a cure. Slowing the disease progression offers people with IPF the opportunity to continue doing the things that matter the most to them. With these medicines, side effects can include nausea, diarrhea, vomiting, decreases in weight and/or appetite and abnormal blood tests, but your doctor can usually help you to manage these.

Health Canada has authorized for sale two medications for the treatment of IPF:


ESBRIET® (pirfenidone)

What the medication is used for:
ESBRIET is used for the treatment of idiopathic pulmonary fibrosis (IPF) in adults.

What it does:
How ESBRIET works is not yet fully understood. It may reduce inflammation and fibrosis in your lungs. It may slow down worsening of your IPF.

ESBRIET should only be prescribed and monitored by physicians with the appropriate training and experience in the diagnosis and treatment of IPF.

Side effects may include:
• Increased Sensitivity to Sunlight: skin reactions after going out in the sun or using sunlamps, sunburn
• Skin problems such as rash, itchy skin, skin redness, dry skin
• Tiredness, feeling weak or feeling low in energy
• Indigestion, heartburn, acid reflux, loss of appetite, anorexia, changes in taste, bloating, abdominal pain and discomfort
• Infections of the throat or the airways going into the lungs and/or sinusitis, influenza and/or common cold
• Difficulty sleeping, feeling sleepy
• Headache
• Muscle pain, aching joints/joint pains
• Weight loss
• ESBRIET may cause liver problems and other abnormal blood test results. Your doctor will decide when to perform blood tests and will interpret the results

OFEV™ (nintedanib)

What the medication is used for:
Use OFEV to treat Idiopathic Pulmonary Fibrosis (IPF) in adults.

What it does:
OFEV works to reduce the fibrosis in your lungs.

OFEV should only be prescribed and monitored by physicians with the appropriate training and experience in the diagnosis and treatment of IPF.

Side effects may include:
• Diarrhea may lead to a loss of fluid and important electroylytes in your body
• Constipation
• Gas
•Heartburn
• OFEV can cause abnormal blood test results. Your doctor will decide when to perform blood tests and will interpret the results

Therapies for IPF symptoms

Your doctor may offer you other therapies to help with the symptoms of IPF, such as breathlessness, but they do not manage how the condition progresses. Oxygen therapy can help with the feeling of breathlessness as you get more oxygen to your body. However, this is often from a tank and so can limit how easy it is to move around. Tell your doctor about other symptoms you are experiencing.