Orkambi—the new Cystic Fibrosis drug from Vertex Pharmaceuticals—just received FDA approval. I was hoping that it would, because it’s a fine drug. That’s my review of it: it’s fine. It does some things very well—my lung function is currently hovering around 77%, which matches my adulthood high from 2009—while other things—my digestion, diabetes and sinuses—haven’t responded as positively. If you’d like, I wrote a whole bunch of words about my experience here, here and here. Most of that still applies, though I’ve since switched digestive enzymes and added a long acting insulin to my routine, both of which have been positive changes. With my new enzymes, I’m able to get away with around 240,000 units of lipase per meal, which is down from the all-time high I hit during the study—300,000, a number that’s probably unsafe–but still above my pre-study number of 200,000 units. Continue reading
May is Cystic Fibrosis Awareness Month. I don’t actually say that in the following video, because I don’t want to constrain Cystic Fibrosis Awareness to just one month; every month should be Cystic Fibrosis awareness month.
In any case, I feel like the angry swearing thing has taken me as far as it’s going to go, so here’s a positive video about how I’m not letting Cystic Fibrosis stop me from living life to the fullest.
The first inkling of an idea is intoxicating, filled with the promise that this is going to be the idea that changes everything. At that moment, the goal is right in reach and every door is open; this could lead anywhere. But the cold light of day moves the goal line somewhere over the horizon and forces you to pick a door. That door leads to more doors and you don’t always get to go back the way you came. Doors turn into walls and possibilities die. That’s why so many projects remain unfinished—it’s much easier to deal with what they should be rather than what they could be. The ones that do get finished are often hated for what they’re not, until a little distance proves them to be “fine.” Continue reading
You should support your local library. Speaking as someone with a pathological need to consume facts, stories and even how-to guides for things I’m never going to do (there is no reason for me to have read a book on French cooking when my main culinary influence is Chef Boyardee), the library is a great alternative to spending a week’s pay on Garfield comics.
I did not always feel that way about the library though. Continue reading
I live my life like a Plinko chip: I rarely end up where I meant to be and there’s usually not any money when I get there. However, if you can correctly guess the price of a Mr. Clean Magic Eraser, you can throw me from the top of the stairs. Continue reading
Over the weekend, I’m going to be migrating this site from it’s current WordPress.com location to dedicated web hosting. Service interruption should be minimal, but depending on when you show up, things might look weird. You’ve been warned.
You’ll know the move is complete because this post will be replaced by one about Plinko,
I feel left out when people talk about what they’d do if they won the lottery, because I know I’m never going to win (it’s an unavoidable consequence of never playing). This is the part where I point out the odds of winning the lottery and how even if you do win it will ruin your life, but you know all that, so I’ll let you fill in the details. Instead, I’ve taken a moment to catalog exactly how it would ruin my life and, by extension, the lives of many around me. Continue reading
When I feel like disappearing up my own ass, I think about the things I care about. I don’t mean family and friends–that’s boring. Saying you care about your family and the people around you is like saying your car is really good because it goes forward. It’s as close to saying nothing as you can get without using silence. Continue reading
I’m a giant fucking production. At minimum, it takes me 8 pills and one injection to get ready to eat, which means it costs about $70 for me to even think about having dinner. (luckily I have insurance right now, so I’m not paying that directly). If I ever had to pay $70 for a meal, I’d crap myself in protest. Even if I decided not to eat today, it still costs roughly $60 for me to wake up. Sure, I could wake up and not do my pulmozyme, but it’s not worth it. Continue reading
Here’s my main CF related problem right now: I’m sick enough to not want to go to work, but not sick enough to justify it to myself. I don’t earn a lot of sick time, so I don’t want to waste it on lungs that feel like they’re coated in a layer of dried pancake batter (wet cement or GTFO). I could get a leave that would enable me to call out using vacation time, but I burn a lot of that on doctor’s appointments anyway—I have three within the next month. I could probably work out a deal where I call out unpaid, but I have $700 worth of medical bills on my desk from those doctor’s appointments I used my vacation time on. There’s nothing Earth shattering happening right now, just a bunch of shit I don’t feel like dealing with. Maybe it’d be easier if I could breathe. Continue reading