Fellow blogger and artist
Glendon Mellow recently encouraged me to write about Cystic Fibrosis, and a few months ago,
Amanda was also wondering about it. I've never really felt motivated to write about the disease, but it seems like a good way to educate people about it. If you want to know the technical jargon and exactly how CF works,
Wikipedia's got you covered. For my part, though, I thought I'd write about
living with CF, because that's generally what people are curious about.
Well, it's not painful. The biggest hassle is that I take a ton of medications in the morning. Most are pills, but two are nebulized aerosolized meds which take 15 minutes (Pulmizyme) and one takes 30 minutes (Tobramicine). Then you have to boil the nebulizers and shake' em dry and maybe get new filter pads for the latter, and...I know I probably shouldn't be complaining, but it really does take forever. Then I use a chest therapy device called a
Flutter. There's a steel ball in there, and the idea is to blow into the mouthpiece forcefully and evenly, thus causing the ball to rattle and moving sputum in my lungs further up until I can cough crap out.
The whole point of my routine is to kill or weaken the bacterium that live in my lungs, loosen the gunk that's built up in there, and cough it out. Right now, my gunk probably doesn't look a whole lot different than yours, except maybe it's thicker. When I'm actually sick, my gunk is green. Sometimes I'll get old green "pellets" out. These are pieces of sputum shaped like a BB and have been lodged in an airway for probably months. Feels good to get
those out, although it's rare. There are certainly a lot of CF patients a lot worse off than me. I don't need oxygen (yet), and I haven't had an IV in five years or so. My weight is up (180 lbs) which indicates good health in CF.
I need to eat a lot. One of the problems I face is a pancrease that has essentially atrophied. I have to take artificial pancreatic enzymes with every meal, or else I absorb nothing, get horrible stomach cramps, and diarrhea. However, the same things happens if I take
too many enzymes. I have to make a guess as to how caloric or fatty something is before I know how many caps to take. Oddly, the number of caps required to eat, say, a pizza, has gone down as I've gotten older. I used to take eight, but now I take six. Because my body doesn't process calories and fat very well, I generally eat about three times as much as a normal person. That means I'm wolfing down nuts, popcorn, cheese, milk, etc. with wild abandon and I barely gain any weight. Most people see this as a blessing, but it can be a curse, too! If I'm actively trying to
gain weight, I have to eat past the point of comfort to make any progress. And if I'm ever sick, my appetite virtually disappears, but I have to force myself to eat to keep my weight up.
Doctors appointments are never enjoyable. My doc is a worrywart, and he orders plenty of hospital tests every time I get a check-up. Chest X-ray, sputum culture, bloodwork, etc. That stuff takes
hours. Not necessarily because the procedures themselves are complex, but because
I hate hospitals, especially the one I go to. Dropping off a time-sensitive sputum sample? Well, just sit and wait for God knows how long so you can get preregistered. What? You were here just last month? Too bad! We forgot who you are!
It's retarded.
When I DO have to get an IV, it's not so bad because it's always a take-home, but the darn tubes catch on
everything on the planet. They're living organisms, and will purposefully reach out and get tangled on crap I'm not even
near. The tube has a tendancy to get clotted up with medication or regrowing vein tissue (yuck), and the little computer sensor that I carry around starts beeping, and sometimes this happens at like 3 o'clock in the morning and...yeah. Even getting the IV put in isn't that bad, but dealing with it is a chore.
Really, that's what CF is, to me: more of an inconvenience than anything else. I have to drag my nebulizers and air compressor around anytime I fly anywhere, and those electricity-demanding machines prevent me from going on extended camping trips or digs at the Colville. But, you know, small price to pay, I guess. The only times I really feel
sick are when a big piece of sputum has successfully moved into the main branches of my lungs. That is, of course, a good thing, but it takes a
lot of huffing and coughing and Fluttering to get that sucker out from there, and it's exhausting. It's not like I can just let the stupid gunk sit there, either, because once it hits the major airways, I can't take as deep a breath anymore.
I have to thank my lovely wife, of course, for basically keeping me alive. When I met her (back in college), I was in pretty bad shape, but she kept (and keeps) me regimented, which has made all the difference in the world. She helps me make good food choices and tells me to go lay down when I obviously need to (but stubbornly won't). She's an angel, and I can't thank her enough.
Honestly, my back gives me more trouble than the CF does. I should re-write about that sometime, because that particular post was deleted when my old blog came down. My back, in fact, has been killing me for the last three days! Hell, where's my vicoden?
Well, it's not painful. The biggest hassle is that I take a ton of medications in the morning. Most are pills, but two are nebulized aerosolized meds which take 15 minutes (Pulmizyme) and one takes 30 minutes (Tobramicine). Then you have to boil the nebulizers and shake' em dry and maybe get new filter pads for the latter, and...I know I probably shouldn't be complaining, but it really does take forever. Then I use a chest therapy device called a Flutter. There's a steel ball in there, and the idea is to blow into the mouthpiece forcefully and evenly, thus causing the ball to rattle and moving sputum in my lungs further up until I can cough crap out.
The whole point of my routine is to kill or weaken the bacterium that live in my lungs, loosen the gunk that's built up in there, and cough it out. Right now, my gunk probably doesn't look a whole lot different than yours, except maybe it's thicker. When I'm actually sick, my gunk is green. Sometimes I'll get old green "pellets" out. These are pieces of sputum shaped like a BB and have been lodged in an airway for probably months. Feels good to get those out, although it's rare. There are certainly a lot of CF patients a lot worse off than me. I don't need oxygen (yet), and I haven't had an IV in five years or so. My weight is up (180 lbs) which indicates good health in CF.
I need to eat a lot. One of the problems I face is a pancrease that has essentially atrophied. I have to take artificial pancreatic enzymes with every meal, or else I absorb nothing, get horrible stomach cramps, and diarrhea. However, the same things happens if I take too many enzymes. I have to make a guess as to how caloric or fatty something is before I know how many caps to take. Oddly, the number of caps required to eat, say, a pizza, has gone down as I've gotten older. I used to take eight, but now I take six. Because my body doesn't process calories and fat very well, I generally eat about three times as much as a normal person. That means I'm wolfing down nuts, popcorn, cheese, milk, etc. with wild abandon and I barely gain any weight. Most people see this as a blessing, but it can be a curse, too! If I'm actively trying to gain weight, I have to eat past the point of comfort to make any progress. And if I'm ever sick, my appetite virtually disappears, but I have to force myself to eat to keep my weight up.
Doctors appointments are never enjoyable. My doc is a worrywart, and he orders plenty of hospital tests every time I get a check-up. Chest X-ray, sputum culture, bloodwork, etc. That stuff takes hours. Not necessarily because the procedures themselves are complex, but because I hate hospitals, especially the one I go to. Dropping off a time-sensitive sputum sample? Well, just sit and wait for God knows how long so you can get preregistered. What? You were here just last month? Too bad! We forgot who you are!
It's retarded.
When I DO have to get an IV, it's not so bad because it's always a take-home, but the darn tubes catch on everything on the planet. They're living organisms, and will purposefully reach out and get tangled on crap I'm not even near. The tube has a tendancy to get clotted up with medication or regrowing vein tissue (yuck), and the little computer sensor that I carry around starts beeping, and sometimes this happens at like 3 o'clock in the morning and...yeah. Even getting the IV put in isn't that bad, but dealing with it is a chore.
Really, that's what CF is, to me: more of an inconvenience than anything else. I have to drag my nebulizers and air compressor around anytime I fly anywhere, and those electricity-demanding machines prevent me from going on extended camping trips or digs at the Colville. But, you know, small price to pay, I guess. The only times I really feel sick are when a big piece of sputum has successfully moved into the main branches of my lungs. That is, of course, a good thing, but it takes a lot of huffing and coughing and Fluttering to get that sucker out from there, and it's exhausting. It's not like I can just let the stupid gunk sit there, either, because once it hits the major airways, I can't take as deep a breath anymore.
I have to thank my lovely wife, of course, for basically keeping me alive. When I met her (back in college), I was in pretty bad shape, but she kept (and keeps) me regimented, which has made all the difference in the world. She helps me make good food choices and tells me to go lay down when I obviously need to (but stubbornly won't). She's an angel, and I can't thank her enough.
Honestly, my back gives me more trouble than the CF does. I should re-write about that sometime, because that particular post was deleted when my old blog came down. My back, in fact, has been killing me for the last three days! Hell, where's my vicoden?