Understanding PF

Treatment & Care

Unfortunately there is currently no cure for pulmonary fibrosis and there is limited treatment available. Using an oxygen tank can help manage the strain of Pulmonary Fibrosis, however this varies from case to case and you should consult your doctor to learn more.

Recent research data also suggests a potential role for several therapies for people with IPF. Please see below for more information.

  • There is new hope for IPF patients with the Health Canada approval of OFEV™ (nintedanib) for IPF. OFEV™ was approved on June 24, 2015 and we’ll have more information about availability soon.
    With the approval of OFEV™, patients who are diagnosed with IPF now have a choice of therapy. Working with their doctor, patients with IPF can determine which treatment option is right for them.
    The Canadian Pulmonary Fibrosis Foundation will be working to ensure affordable access to this treatment in all the provinces.
    If you have any questions about this treatment and its availability please contact the Boehringer Ingelheim Customer Care Team at 1-800-263-2425.
  • Manitoba announced, in this bulletin (page 3), that they will be reimbursing Ofev as of January 25th, 2017 under Exceptional Drug Status with the following criteria outlined here. A list of other provinces that fund Ofev and the criteria, visit here (under Provinces Provide Choice for IPF Patients).
  • HeadStart™ Patient Assistance Program
    Download information on OFEV here: Product Monograph (PDF Version ~ 175K)
    Download the UHN Patient Education handout – OFEV

  • Pirfenidone – Esbriet™ (pirfenidone) is an anti-fibrotic/anti-inflammatory agent approved by Health Canada for the treatment of mild to moderate idiopathic pulmonary fibrosis in adults.
  • Inspiration™ Patient Assistance Program
    Download information on Esbriet here: Product Monograph (PDF Version ~ 175K)
    Download the UHN Patient Education handout – Esbriet
  • N-Acetylcysteine (NAC) – One research study found that adding N-acetylcysteine (NAC) to traditional prednisone-containing therapies may be helpful, but it is not clear how helpful this drug is when used on its own. N-acetylcysteine (NAC) is available over the counter at many pharmacies and nutritional stores and has very few side effects.
  • Prednisone plus Azathioprine – Combining two medications, such as Prednisone and a second drug such as Azathioprine (or Mycophenolate and Cyclophosphamide), has been used for the treatment of IPF.

Your doctor will talk to you about which, if any, of these treatments may be right for you.

The Canadian Pulmonary Fibrosis Foundation is supporting the University Health Network’s Interstitial Lung Diseases Clinic at Toronto General Hospital with a research grant for their Idiopathic Pulmonary Fibrosis and Gastroesophageal Reflux Disease research project. Read more…
Lung Transplant
Currently a lung transplant is the only effective treatment for Pulmonary Fibrosis. The Multi Organ Transplant Program at Toronto General Hospital can be consulted regarding lung transplants.

At some stage you may be considered as a candidate for a lung transplant. This is currently the only intervention known to prolong life in IPF patients. The first successful single lung transplant was done at Toronto General Hospital in 1983. The first double lung transplant was done at the same hospital in 1986. Toronto is one of the most experienced centres for lung transplant in North America, and provides care that is on par with the best programs in the world.

If you are referred there for a transplant, you can be sure of first-class treatment. Forty five percent of lung transplants in Canada go to patients with pulmonary fibrosis. The criteria for receiving a lung transplant are constantly changing. It used to be that people over the age of 55 were considered too old, now people as old as 74 are receiving new lungs and the operation success rate is very high. New anti -rejection drugs are becoming available that significantly reduce the risk of rejection.

Although there are risks with a lung transplant, the quality of life after a transplant can be vastly improved.

There are of course risks, but excellent researchers around the world are addressing these risks constantly.

You should know that in Canada, apart from very limited availability of donor lungs, the only criteria for receiving a lung transplant are:

• Your blood type must match with the donor lungs
• The lung size must be right for you – you can’t put 7 litre lungs in 4 litre space, or vice versa
• Your general health, with the exception of IPF, and whether you can handle an 8 hour operation

With the right patient at the right time, a lung transplant, while being a very risky and major operation, can indeed be a miracle, extending the patient’s life expectancy and improving their quality of life.

Please visit the University Health Network to find out more about the internationally renowned Multi-Organ Transplant Program. View a list of Transplant Programs in Canada.

Organ Donation
If you would like to become an organ donor, please visit the Organ Donation page.