When I was living with Idiopathic Pulmonary Fibrosis (IPF), there was no effective treatment for the disease. As a matter of fact, one of the routinely prescribed drug cocktails turned out to do much more harm than good. In October of 2014, two drugs were approved by the FDA for the treatment of IPF. Pirfenidone and Nintedanib were shown to slow the rate of progression of IPF in patients who were at a mild to moderate stage of the disease. By the time these drugs were available to the public, I was passed the mild/moderate stage and could not get on the medicines.
These are not cures, and they do not work for everyone. They slow the progression of the fibrosis for many patients. That is pretty awesome in a disease where a patient can often expect a 3 to 5 year life expectancy after diagnosis. These drugs offer hope, and trust me, hope is a precious commodity when you have an expiration date.
Some very good news for patients who did not see posative results with Pirfenidone has just been published in Thorax (one of the world's leading respiratory medicine journals). As with a lot of these studies, the title is quite long. "Effect of continued treatment with pirfenidone following clinically meaningful declines in forced vital capacity: analysis of data from three phase 3 trials in patients with idiopathic pulmonary fibrosis".
So what did they find that is so awesome? From the paper:
In patients with idiopathic pulmonary fibrosis who experienced a ≥10% absolute decline in FVC during the first 6 months of treatment, continued treatment with pirfenidone reduced the risk of a second ≥10% decline in FVC or death compared with placebo.
Our findings provide the first available evidence to suggest that continued treatment with pirfenidone may confer a benefit to patients with idiopathic pulmonary fibrosis who exhibit evidence of meaningful disease progression during treatment.Not a cure by any means, but a life extender for many. Slowing the progression increases the odds of living until they do find a cure, or more likely, a patient is matched up with a good set of lungs and successfully transplanted.
We do need a cure, and there are promising trials and studies going on right now. Pirfenidone and Nintedanib are an important step in the right direction, I am excited to see what's next.