Key facts
- Antifibrotics are important treatments that help in disease management
- There are two antifibrotic treatments currently available in Canada
- You may be given more than one medication to treat pulmonary fibrosis and any other comorbid conditions
- Being prescribed a new medication may affect other treatments you are taking. This is because some medications interact with each other
In addition to pulmonary rehabilitation, oxygen therapy and lung transplantation, there are medications available in Canada.
The medications belong to a type of medication class known as antifibrotics. There are currently two antifibrotic medicines available — nintedanib and pirfenidone. They are likely to form a key part of your treatment plan. Speak to your doctor to find out what antifibrotic treatments are most suitable for you.
Nintedanib is available in Canada to:
- treat idiopathic pulmonary fibrosis (IPF)
- slow the rate of decline in lung function in patients with systemic scleroderma associated interstitial lung disease (SSc-ILD), also known as scleroderma lung disease
- treat other chronic fibrosing ILDs where pulmonary fibrosis (lung scarring) continues to worsen (progress), also known as progressive fibrosing ILD
Pirfenidone is available in Canada for the treatment of:
- idiopathic pulmonary fibrosis (IPF)
ESBRIET® (pirfenidone)
What the medication is used for:
ESBRIET is used for the treatment of 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 progression 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.
Severe drug interactions can occur if ESBRIET is taken in combination with some other medications. In particular, do not take ESBRIET with fluvoxamine.If you currently take fluvoxamine, a different medication should be prescribed for you before you begin taking ESBRIET.
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:
OFEV is used to:
- treat idiopathic pulmonary fibrosis (IPF)
- slow the rate of decline in lung function in patients with systemic scleroderma associated interstitial lung disease (SSc-ILD), also known as scleroderma lung disease
- treat other chronic fibrosing ILDs where pulmonary fibrosis (lung scarring) continues to worsen (progress), also known as progressive fibrosing ILD
What it does:
OFEV blocks some of the activity of tyrosine kinases (a type of cell proteins) that are involved in the development of scar tissue in the lungs. This helps reduce the decline in your lung function by slowing down the worsening of the pulmonary fibrosis (scarring in the lungs).
OFEV should only be prescribed and monitored by physicians with the appropriate training and experience in the diagnosis and treatment of the conditions for which OFEV is indicated.
Side effects may include:
- Diarrhea, which may lead to a loss of fluid and important electrolytes in your body
- Nausea/vomiting
- Stomach pain
- Areas of hair loss
- Bleeding
- Constipation
- Dizziness
- Decreased appetite
- Gas
- Headache
- Heartburn
- Muscle pain, aching joints/joint pains
- Weight loss
People with IPF often have other co-existing conditions that can have a big impact on their quality of life. These other diseases are known as comorbidities, or comorbid conditions.
Some comorbid conditions may impact the progression of IPF and can affect your overall health and sense of well-being.
Some of the common comorbid conditions that patients with IPF may have include:
- Gastro-esophageal reflux disease (GERD), or acid reflux disease
- Other lung conditions (chronic obstructive pulmonary disease [COPD], emphysema, lung cancer)
- Heart and vascular conditions such as pulmonary hypertension (high blood pressure in blood vessels that supply the lungs) or heart disease
- Depression
- Obstructive sleep apnea (repeated pauses in breathing during sleep)
“We spent about 90 minutes with [the respirologist] and she gave us the news in regard to the diagnosis…both my GP and the respirologist were aware of the fact that my friend had passed away with IPF that year. So, we were in shock in regard to the diagnosis with my friend dying so quickly. Of course, you get obviously a little despondent…The respirologist is excellent, she explained that what happened to my friend was very rare…So I was a 'mush brain' as they call it. You can’t process things, you think the negative, you think you’re going to pass away within months…Then we started on the medication and my mindset changed. I did a 360, I had a chat with myself. I don’t mope because of my condition. I don’t feel sorry for myself. I don’t dwell on what I have.”– Paul, ON
(@ 2 years post-diagnosis)*
* Name has been changed for privacy.
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