Idiopathic pulmonary fibrosis, or IPF, is a progressive hardening of the lung. It is distinguished from other forms of pulmonary fibrosis in a few regards. It alone carries the name “idiopathic.” This means they aren’t sure what causes it, or, in other words, that they are ignorant. I know of no doctor whom spent the front third of their life and hundreds of thousands in tuition to be called ignorant. This is why they use the term “idiopathic.” It is Greek for “ignorant but educated.”

This would be similar to the American term “a genuine asshole.” While it concedes that the individual in question is an asshole, it gives us the assurance that at least they are sincere about it.

IPF also differs from the other forms in that it has very limited treatment options.  Historically there has been supplemental oxygen when needed and a double lung transplant when oxygen alone no longer cuts it. As of today there are two experimental drugs about to be brought before the FDA in an effort to slow the progression.

In a few cases IPF seems to have a genetic component. You use to be able to be part of a research study which would disclose whether or not you carried the gene or genes which were suspected. They don’t do this anymore. Some who carried the gene or genes never developed the illness. Some who didn’t, did. You can still be part of the research study, but you do it blind, and this hardly seems to be objectionable for a test that has trouble seeing anyway.

But what does it tell us anywyay?  Would it be a terrible surprise to learn that all of us, with our self-destructive tendencies, are carrying around self-destructive genes?

My father was diagnosed with IPF well over a year ago. Some patients don’t make it 6 months after a diagnosis, at which time they presumably find the answers to all the questions we spend our lives wondering at. Some make it many years. My father is doing well, has taken it in stride, remains active doing what he wants to do, needs no oxygen, and only battles a persistent cough. Every few months he goes in for a checkup, and I and my sister, Katie, accompanied him today.

Despite all kinds of variations, when you boil it down there really seems to be only two ways to deal with an illness: either you keep it to yourself or you talk about it. If we take the former, the risk seems to be that it will distance us from those we are close to. If we take the latter, the risk seems to be that it will let others up close and personal. Near as I can tell, one risk is as considerable as the other.

For his part, Dad chose the second and talks about things freely. I tend to be caught between the two.  I find humor works in a unique way to let us do both.  After a two hour car ride I was beginning to wonder if my humor hadn’t left.

Just then a doctor came through the door my father had disappeared behind an hour before. He passed my sister and me, and approached a burly fellow, who had been in there so long he had fallen asleep in the corner.

The two were about the same age, but had little in common otherwise. The doctor was slender built and looked younger than he was. The man across from him, just waking up, had the brown ears and red nose of one who labored while the doctor carried in his sandals the tanned feet of a man accustomed to a different lifestyle. The doctor spoke in a business-like, matter of fact way, and the laborer met him in kind.

“Well I wanted to let you know that it was in a really tight spot. It was kind of tucked around behind like we suspected, and there were a lot of blood vessels and other things surrounding it.”

“That’s how they said it looked on the scan.”

“Yes, and the scan was accurate. I couldn’t get very close to it. At one point I kind of jabbed a needle at it with the scope, and I stuck a brush out and tried to rub up against it a time or two. I’m not very optimistic that I got much of anything. I’m afraid with where it’s at surgery won’t be an option for us. She did very well though.”

“Oh, I knew she would. She’s so strong. She greets everything with a smile and never gets discouraged.”

“Yes, she’s a trooper. You can come back in five minutes or so. I’ll culture what I got, and we should know something by tomorrow. I’ll call you. Now when you go back there I want to warn you. We had a water pipe break somewhere up on the sixth floor when we were in the middle of everything. So it is going to look like complete chaos with buckets everywhere.”

The burly man laughed a big laugh and said, “I don’t suppose I’ll even notice. I want to thank you, Doc, for everything you’ve done.”

“Yes, well that’s the trouble. I was hoping to have done more. Still, we are pretty sure we know what we are dealing with. This was just to confirm it. Dot our “i’s” and cross our “t’s”, you know? If we came up empty today, we still have a plan in place as to how to proceed.”

“Do you agree about the difference in the high and low rates?”

“Yes. A high rate is the only rate that offers a hope for a cure.”

“How long will it take her to recover from today?”

“She should be back to her old self in no time.”

“Is a bike ride this weekend out of the question?”

“A bicycle?”

“Do I look like a man that rides a bicycle to you, Doc? The bike I ride is a big old Harley Davidson.”

Now the doctor laughed. “I think a ride on the back of a motorcycle would be just fine. Big plans?”

“Oh, kind of a bucket list thing, you know?”

“Well, I think that’s wonderful. If you don’t have any more questions, you can come back in a few more minutes.”

And so two men whom seemed to have nothing in common, were united by the one thing that unites us all, and found that more than enough to share a mutual respect over. I respected it too.  I had gotten up close at a distance.

How many times that conversation took place today I don’t know. Thousands, perhaps. I suppose in hearing one I received an education. At the same time, I hardly know any more about why it happens than I did before. It’s idiopathic, I would guess, and I’m being genuine.