Update May, 2011: My pulmonologists now thinks I have the NSIP (Non-Specific Interstitial Pneumonitis) version of Pulmonary Fibrosis, which is less aggressive than standard IPF. That’s a good thing, probably why I’m still alive past my five-year sell-by date. On the other hand, he thinks I have the more aggressive fibrotic version of NSIP rather than the cellular-version, so that’s bad. My oxygen needs have stayed stable for a year, so that’s good.
Original Post with O2 update at the end:
Well, maybe (I'm not dying) right away, so my liberal friends shouldn't get their hopes up. And, no, not over the latest bad news out of Washington.
Sure, I realize that every adult is technically “dying,” having started the long deterioration process that leads to dusty death. I mean I have a diagnosis of a terminal illness. Actually, I had the first diagnosis in June of 2006, about six months after the symptoms started. (Sorry I didn’t tell you before, but it slipped my mind.)
It started with a cold and a cough that I couldn’t shake, on December 31, 2005. I’ve pretty much had that cough every day since. It didn’t worry me too much at first, as I have a history of lingering coughs, going back at least to collage. Finally, I ended up at the pulmonologist. He did a CT scan, a pulmonary function test and diagnosed “early interstitial fibrosis.”
That got my attention, as my mother died of pulmonary fibrosis at age 69, in 1995, less than six months after her diagnosis. (They don’t know what causes PF, but heredity is considered a strong factor, and probably there is a gene. My exposure to secondary smoke over the years may be a factor as well.) He said he didn’t think I was at high risk of developing full blown PF, but not at average risk, either. He suggested some therapies they hoped would slow the advance of the disease, with which I was compliant. So I lived with the (productive, which is atypical for IPF) cough and went on with life.
In October of 09 I went to Colorado Springs on business, and the altitude knocked me for down and sat on me. Even after returning home, I started to be short of breath after exertion. If I carry stuff up the stairs, or walk too far or fast, I pant like I’m back on the parade deck at Parris Island after a long run. A bronchoscopy in January confirmed that I have Idiopathic Pulmonary Fibrosis.
All you really need to know is that there is no cure for IPF, and it’s eventually fatal, except for a small number of people who are lucky enough to get and survive lung transplants. (I’ve met a couple, which is encouraging.) About 85% survive the transplant operation, and the 5-year survival rate is between 70% and 50%, my doc tells me. The catch is that you have to be a lot sicker than me to get on the transplant list. And if you are too sick, you don’t survive the process. IPF can apparently stabilize for years, then take a sudden drop. It makes life interesting.
So, most people who have IPF eventually die from it. The question is when “eventually” is. About 50% of those with IPF are dead at my point. But I’ve talked to or know of people who are still trucking along at seven or ten years, and met one who was diagnosed in 1982, been on oxygen since 1999, but is still getting around. The idea is to stretch it out so that something else gets you.
You probably haven’t heard of IPF (there are a lot of variations. A lung biopsy might narrow mine down further—but that carries its own risks.), so you are thinking it’s pretty rare, even though Robert Goulet, Evil Knievel and Liam Clancy died from it. And WD-40 doesn’t cure it—John Barry died from it too.
Actually, the estimates are that 40,000 to 45,000 Americans die every year of PF, and that anywhere from 200,000 to 500,000 people are living with it nationwide. It is under diagnosed and the death rate is also hard to pin down. For example, if I exert myself too much and my “sats” (oxygen saturation levels) drop below 90 too often or too far, it can damage my heart, and I could die of congestive heart failure. (I carry a meter to check my pulse and my sats.) So my death might be classified as “heart disease,” though it would be the PF that caused it. (When I was single, I was hoping cause of death would be, “Shot be a jealous husband at age 100,” but Bonnie has kind of ruled that out. And wheezing and coughing are hardly going to get you one of Tiger’s girls.)
This death rate is believed higher than the Breast Cancer death rate. Breast Cancer now is estimated to claim slightly under 40,000 lives a year. The reason you don’t know about IPF is that you don’t turn on your TV and hear about pink ribbons, postage stamps or the latest high hype “Race for the Cure” for IPF, because it hits both men and women. So it gets only a tiny fraction of the research funds that Breast Cancer gets. Breast Cancer is, of course, an elite, political disease. It gets six to ten times the research dollars per death as even lung cancer.
What is IPF? Basically, the sacs in your lungs that transfer oxygen to your blood become scar tissue and cease to function. If you listen to me breath with a stethoscope, you hear a “cracking” sound—the scar tissue trying to flex. When you can no longer get enough oxygen, you smother. Oops--too blunt. I mean you “expire from respiratory failure.”
Note that I’m not a doctor and don’t play one on TV, so you should double check any facts I give you here.
I’m pretty lucky among those who have IPF. I’m past the 50% survival rate line, still able to work full time, and not yet on oxygen. I have the type of job that I can do even when I do have to be on oxygen, and the surgeons I work for are classy folks and very supportive. My wife Bonnie is also a tower of strength and help.
There are a lot of folks ten or twenty years younger than me with IPF, who are in worse shape. And, having won the lottery of birth and been born in the United States, I’ve benefited from the political and economic freedoms we were bequeathed by those who went before us. I’ve already lived longer and better than 98% of the people who have ever lived. Not that I’m giving up—I’m shooting for 99%. But when I do croak (I hate “Pass away.”), I won’t really have a complaint coming.
I’m in very good spirits, not depressed, and very much enjoying life and my job. I’m still writing, still learning; recently read Thomas Sowell’s Applied Economics and as I write am re-reading Hogan’s Kicking the Sacred Cow about controversies in science, both of which I highly recommend.
I do laugh when fans of my blog write and say I should run for president. Along with no money, base or name recognition, I hardly have the ability to campaign and give speeches. I also laugh when critics write and say I’m an old fart and should die and let the younger generation take over. Not just yet, thank you. I hope I have several years of productive work in me. I don’t know, of course, there are those “sudden declines.” But none of us is promised tomorrow. And I volunteered for Vietnam when I was twenty, had an easy tour and was lucky enough to come home in one piece, so the past 44 years have been gravy. (No, I don’t think my IPF was caused by Agent Orange—or George Bush.)
I didn’t write this because I’m looking for sympathy, so spare me. As my old Gunny used to say, “If you want sympathy, Hall, you can find it in the dictionary between shit and syphilis!” And I have a Marine bumper sticker that says, “Don’t you quit on my, Maggot!” to motivate me just like my DIs did. So I’m doing fine.
I wrote this post because I’ve become active in IPF support groups and the Pulmonary Fibrosis Foundation to educate myself. Their biggest hurdle to fighting the disease is that few people know about it, while everyone knows about Breast Cancer and AIDS, which get the lion’s share of research dollars.
I can’t educate the whole country. But I felt I should do my part—and now you know about IPF. Thanks for reading. If you want to know more, there are some websites linked below. Semper Fi. ~Bob
Well, time to go on Oxygen
I gather I’ll have a concentrator in my home and tanks. I told the pulmo tech I preferred liquid, but don’t know if that translated. Also found out that I can rent a portable concentrator from them for $75 a week to take when I fly or on vacation. I understand it only goes to 3 LPM, but think that will do for short periods if I don’t overdue it.
I had a sleep study May 25, 2010, then was called back Friday, June 4 for one with a CPAP, as my O2 saturation levels were dropping into the high 70s late in the night. They didn’t get the results to the Pulmo doc in time for my appointment on 6/8, but I’m sure a CPAP is in my near future, not sure with or without O2.
The pulmo doc ordered a 6-minute walking test. The tech quickly stopped me to add O2, then to increase it twice. The verdict is I need 4 LPM of O2 when walking or in other exertion, but am okay without when sitting. They did a flight simulator test and said I need 1 LPM when I fly.
The pulmo doc reviewed the CT scan they did. He said it shows some increase in the fibrosis, but not a lot, however, the O2 sats and the shortness of Breathe indicate it has gotten worse. He said five different Thoracic Radiologists had reviewed my CT scans. The first three thought UIP-usual interstitial pneumonia. The last two think NSIP-non-specific interstitial pneumonitis, which would be good as he says UIP is more aggressive. He is “cautiously optimistic” that it’s NSIP. I would need a lung biopsy to be sure, but I’m not up to the time out of work and the risks. I hear it’s a tough go. Given that there are no good treatments either way, I’m going to sit tight and use the O2 and probably the CPAP.
Using the O2. hate the tether, but it does make me feel and sleep better.
Here's a good clip on IPF:
The Most Deadly Disease You've Probably Never Heard Of