Pirfenidone is one of the only drugs that has been approved in several countries (Japan, India, Canada, etc.) as a viable treatment option for IPF. In clinical trials it showed only an 8% success at slowing down the progress of the disease. However, secondary effects that were observed was a reduction in the probability of an acute exacerbation.

IPF patients often suffer episodes called acute exacerbations. During these periods, they suddenly get really bad, have trouble with oxygen saturation levels and sometimes need hospitalization. Pirfenidone was shown to reduce the incidence of such episodes.

In the case of my dad, his liver readings were slightly elevated in late February due to all the drugs he was taking. So, on our visit to the doctor on Feb 22nd, the doctor took him off Pirfenidone. At this point, my dad had been taking it for about 9 months. (since May 2012)

Shortly after the Pirfenidone removal, he went into an acuate exacerbation and eventually passed away on 5th March. It may be that he was already having an acute exacerbation since his condition had become much worse since December 2012. Another suspect in the exacerbation is the stenting procedure he underwent. We were looking into the possibility of a lung transplant but to have that, he would need to either get stented or have bypass surgery. Since stenting was a safer option, he got a stent placed on January 7th 2013. However, after this, his ability to do any physical activity greatly reduced. He had trouble keeping his O2 levels up while walking around and eventually had to use oxygen 24x7 (prior to this, he only took oxygen at night while sleeping - he could go about his day without supplemental O2).

I searched high and low on the internet to see if there was any correlation between a stenting procedure and an acute exacerbation but couldn't find anything. Unfortunately, not much is known about what can cause an acute exacerbation of IPF. There are some cases where a patient underwent kidney surgery and shortly thereafter had an IPF acute exacerbation. Pretty much anything can trigger it.

Pirfenidone is still not available in the US as the FDA hasn't approved it yet. The drug is pretty cheap in India and I would suggest that anyone who wants it should try and get it imported. It's produced in India by a fairly reputable company called Cipla. It would be much cheaper than buying it in Canada, etc.

Do not take Azathioprine

Another doctor we visited prescribed a combination of Azathioprine and Prednisone. This is a very dangerous combination and my personal opinion is that the doctor was not aware of the latest studies on IPF. There was a study in 2011 on this combination and it was found to be so dangerous that the study was stopped midway. If your doctor prescribes this combination - PLEASE TAKE ANOTHER OPINION. My dad took this combination for 2-3 days but when I found this study he stopped taking it and switched back to his older medication. Nothing was helping much, really.

Remedies for the coughing with IPF

The coughing with later stage IPF gets pretty bad. We couldn't really find many ways to control this. Prednisolone did control it for a little while but it would eventually return as the body adjusts to the dosage. Besides, steroids aren't really the best for you and your other systems. The only thing that would help the coughing is possibly humidifying the air and taking NAC (an antioxidant) but the benefits were small.

Some people find nebulizers helpful, you have to basically try a number of things and see what works for you.

A tough choice ?

Taking medication for IPF is a tough choice. The drugs don't really work. Pirfenidone is possibly the only medicine that is worth a shot and it is fairly safe. Other than that, a combination of steroids is possibly a bad idea.

We ended up experimenting with various steroid combinations (with the guidance of doctors of course) only to control my dad's cough. However, nothing seemed to be work and everything just seemed to make something else worse.

This unfortunately is the choice you are faced with. Take drugs to control the cough and make things worse ? Or take nothing and be bothered by this annoying cough ?

Every case is different, but please be careful with drugs. Again, there are no medicines that truly work for IPF so far and doctors are only too happy to prescribe 20 pills a day since drug companies take them on cruises if they meet prescription targets.

This does happen in India as well, so please be very careful when dealing with doctors. Make sure you go over every drug in the prescription and ask "What is this one for ?". It's best to be careful. We had situations when the doctor would say: "This one is to speed your heart up" followed by "This one is to slow your heart down". What ?!

Another example was that they prescribed blood pressure medication for my dad when he had no blood pressure problem. Please be careful when it comes to doctors and drugs. Good luck!