It is what it is...

Several folks have asked how I'm doing and I realized  that it's been awhile since I've sent out any updates about my health. (For those who don't know what I'm talking about, click Here .)

A couple of phrases that I learned while working up in North Dakota and Western Minnesota the past several years come to mind: “Keep on keeping on” and “It is what it is.” And that pretty much speaks to how I'm doing. I'm keeping on and it turns out that Pulmonary Fibrosis is what it is.

We've settled into an uneasy accommodation with this disease. For example, I take a “small” oxygen tank with me if I think I'm going to be walking any distance. I also do a much better job of planning ahead so that I not going to be caught breathless or decide that I can't do something because I don't have oxygen tanks with me.

So far I'm not needing to use oxygen when I'm at rest except on very unusual days. However it's difficult to know when a day is going to be unusual or not. That offends my need for  predictability but it is what it is.

I often find that I grow both physically and mentally fatigued in the late afternoon and more irritable. I'll be more forgetful and have a more difficult time finding the right word to say or write. Generally that's accompanied by an overall sense of weariness as well as a feeling of heaviness in my arms.

The antidote is taking a 30-45 minute “refresher nap.” I don't like to take naps because it tends to make a regular bedtime more difficult to achieve. But slowly I'm getting the hang of it.

All of these symptoms are very similar to what I experienced when Barb and I took the kids out west and visited Pikes Peak. The lack of oxygen at the top of the ascent caused exactly those same symptoms. So I've decided to rule out mountain climbing as a hobby for the future. Sorry, Mount Everest.

Shortly after the diagnosis, I started taking a medication called Esbriet. While others have reported physical side effects, I haven't experienced any significant ones. There is also a higher incidence of liver damage with Esbriet but my monthly lab results have been unremarkable.

Esbriet doesn't cure the disease but slowed down the progression of fibrosis for many (but not all) patients in clinical trials. In September I'll retake the pulmonary function test and we'll have a better idea of whether (or how quickly) the fibrosis is progressing.

Esbriet is a very expensive drug. The annual cost is slightly more than $100,000 and my copay would be about $7000. Fortunately there's an organization which provides grants to help cover the copays.

I was extremely happy to get the grant because they're very difficult to obtain. Applicants need to be singularly attractive and highly intellectual. Well, that's my story and I'm sticking to it.

So, we keep plugging ahead although my mantra when walking is now “slow and steady” and I often find myself filling the role of Tail-end Charlie when walking with Barb. That's a reversal so I think her legs have grown longer.

I'll write more in 6-8 weeks or if things change substantially.