Recent research data also suggests a potential role for several therapies for people with IPF. Please see below for more information.
- 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.
- 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.
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.
Idiopathic Pulmonary Fibrosis: Management & Treatment
This video is a recording of an interview with Dr. Gerard Cox shown to high school students from Hamilton, Ontario at a dissemination event at McMaster University on December 2nd, 2013.